Mohamed M. Ismail Case scenario Modified by: Abdel Razak Ali Shadeed Investigations and treatment In the exam a Case scenario is offered and you mention Investigation or treatment Kindly hide the diagnosis and test yourself Nutritional disorders A mother brings her 12 months old boy because he is always crying. She observed that her child is wasted and looks markedly wasted. On examination, the child is crying most of the time he looks very thin and wasted. His weight is 6.5 kg His lips look pale with macerated mouth angles. Subcutaneous fat is lost over the abdomen, buttocks, with senile face. Chest examination reveals scattered 4 rales and crepitations all over the chest What is your diagnosis: Marasmus 3rd degree complicated with pneumonia? Mention possible investigations and treatment INV: For marasmus: 1. CBC: Anemia, leukocytosis (infection). 2. Other markers of infections: CRP, ESR, Stool analysis. 3. Serum protein: not markedly ↓↓ (DD: Kwashiorkor). 4. Electrolytes, blood glucose. 5. Investigations of non-nutritional marasmus. For pneumonia: 1. Chest X-ray: a. Confirm the diagnosis. b. Complications: effusion- empyema- pneumothorax- lung abscess. 2. Blood gas: in severe cases lowered O2 tension and raised CO2. 3. CBC, ESR, CRP: DD/ bacterial and viral causes. 4. Culture and sensitivity test: morning nasopharyngeal aspirate or sputum culture TTT: For marasmus: A) Hospital Management 1. Indications: • Moderate or severe Kwashiorkor. • Marasmic kwashiorkor. 1 Mohamed M. Ismail Case scenario Modified by: Abdel Razak Ali Shadeed • 3rd degree marasmus. • Complications. 2. Management plan (Stabilization and management of complications): • Infection (GE, pneumonia...): Proper antibiotics. • Shock: Shock the spy (Immediate IV fluid: Lactated ringer's 20 ml/Kg). • Dehydration: IV fluid therapy (Deficit therapy). • Electrolyte disturbances: should be corrected. • Anemia: Packed RBCs Hypoglycemia (IV glucose). • Hypothermia: adequate clothing or radiant warmer. B) Nutritional management (Home or hospital): Marasmus ↑↑ Calories 150 Kcal/Kg/day (to be reached gradually) According to the age: • Milk-fed: Milk (Lactose-free may be used initially), standard formula • Weaned infants: Balanced diet [CHO = 50%, Lipids = 35 %, Protein = 15%] Start with ≈ 75 Kcal/kg/day ↑↑ Amount and concentration according to the child tolerance (Rate of ↑↑ 5-10 Kcal/kg/day) Oral or Nasogastric tube (NGT) Vitamins: 1. Vitamin A: • <6 months: 50.000 IU/day • 6-12 months: 100.000 IU/day • 12 months: 200.000 IU/day 2. Vitamin B, C, D, E, Folic acid Minerals: 2 Mohamed M. Ismail Case scenario Modified by: Abdel Razak Ali Shadeed • Iron (4-6 mg/kg/day) and zinc For pneumonia: A. Hospital management (7-10 days): 1. Indications • Severe pneumonia (severe RD) or complicated pneumonia. • Small infants (Less than 6 months). 2. Supportive measures: Humidified oxygen - IV fluid (NPO) - suction - mechanical ventilation. 3. Specific treatment: Broad spectrum combined parenteral antibiotics to cover G+/(Ampicillin 50-100 mg/kg/day + gentamycin 4-6 mg/kg/day). Antibiotics may be changed according to results of culture and sensitivity and clinical response. 4. Treatment of complication: • Drainage of empyema. • Mechanical ventilation (in respiratory failure). B. Home management for most cases: 1. In Older children with mild pneumonia without distress. 2. Oral or better intramuscular antibiotics. 3. Amoxicillin 50 mg/kg/day or better broader-spectrum antibiotics such as amoxicillin-clavulanic acid for 7-10 days. A mother brings her 14-month-old boy because he is not well. She observed that her child has recently lost his appetite and became disinterested in playing. The mother says that she got another baby 3 weeks ago, and in the last 2 months, she stopped breastfeeding her older child and started to give him mashed potatoes cooked rice with some added sugar and boiled macaroni. On examination, the child looks distressed, coughing with cyanosis and grunting but he is not feverish. His weight is 8 Kg. His temp is 36.3 C and his respiratory rate is 60 breaths/m. He looks edematous with sparse light color. His lips are pale Diagnosis: Kwashiorkor complicated with pneumonia & respiratory failure Mention possible investigations and treatment 3 Mohamed M. Ismail Case scenario Modified by: Abdel Razak Ali Shadeed INV: For Kwashiorkor: 1. CBC: Anemia, leukocytosis (infection). 2. Serum albumin: ↓↓ (N= 3.5-5.5 g/dl). 3. Serum globulin: ↓↓ α and β-globulins (↑↑ γ-globulins due to infections). 4. Electrolytes: • Hyponatremia: it is dilutional (N:135 -145 mEq/L) Total sodium increased (aldosterone), but serum sodium decreased (water retention). • Hypokalemia and hypoglycemia. • Hypomagnesaemia. For pneumonia: 1. Chest X-ray: A. Confirm the diagnosis. B. Complications: effusion- empyema- pneumothorax- lung abscess. 2. Blood gas: in severe cases lowered O2 tension and raised CO2. 3. CBC, ESR, CRP: DD/ bacterial and viral causes. 4. Culture and sensitivity test: morning nasopharyngeal aspirate or sputum culture. TTT: For Kwashiorkor: A) Hospital Management 1. Indications: • Moderate or severe Kwashiorkor. • Marasmic kwashiorkor. • 3rd degree marasmus. • Complications. 2. Management plan (Stabilization and management of complications): • Infection (GE, pneumonia): Proper antibiotics. • Shock: Shock the spy (Immediate IV fluid: Lactated ringer's 20 ml/Kg). • Dehydration: IV fluid therapy (Deficit therapy). • Electrolyte disturbances: should be corrected. • Anemia: Packed RBCs Hypoglycemia (IV glucose). • Hypothermia: adequate clothing or radiant warmer. 4 Mohamed M. Ismail Case scenario Modified by: Abdel Razak Ali Shadeed B) Nutritional management (Home or hospital): Kwashiorkor (More difficult) ↑↑ Proteins 4-6 gm/Kg/day According to the age: • Milk-fed: Milk (Lactose-free may be used initially) then standard formula • Weaned infants: High-protein diet (Egg, meat, chicken, beans), Vegetables and fruits. Start with = 1 gm/kg/day ↑↑ Amount and concentration according to the child tolerance • Oral • NGT: if there is marked anorexia • TPN: may be required in severe cases Vitamins: 1. Vitamin A: • <6 months: 50.000 IU/day • 6-12 months: 100.000 IU/day • 12 months: 200.000 IU/day 2. Vitamin B, C, D, E, Folic acid Minerals: • Iron (4-6 mg/kg/day) and zinc For pneumonia: A. Hospital management (7-10 days): 1. Indications • Severe pneumonia (severe RD) or complicated pneumonia. • Small infants (Less than 6 months). 2. Supportive measures: Humidified oxygen - IV fluid (NPO) - suction - mechanical ventilation. 5 Mohamed M. Ismail Case scenario Modified by: Abdel Razak Ali Shadeed 3. Specific treatment: Broad spectrum combined parenteral antibiotics to cover G+/(Ampicillin 50-100 mg/kg/day + gentamycin 4-6 mg/kg/day). Antibiotics may be changed according to results of culture and sensitivity and clinical response. 4. Treatment of complication: • Drainage of empyema. • Mechanical ventilation (in respiratory failure). B. Home management for most cases: 1. In Older children with mild pneumonia without distress. 2. Oral or better intramuscular antibiotics. 3. Amoxicillin 50 mg/kg/day or better broader-spectrum antibiotics such as amoxicillin-clavulanic acid for 7-10 days. A 1.5-year-old boy presents with abnormal gait and poor weight gain. He was breast fed for the first year without any other supplementation. On examination, he is pale with prominent forehead and a marked abdominal distension. He has small chest swellings. His length is on the 25th centile and his weight on the 10th centile. The hand and foot show abnormal fixed position. And the child started to convulse What is your diagnosis? rickets complicated with hypocalcemic tetany & convulsion Mention possible investigations and treatment INV: A) Laboratory • Serum calcium: Usually normal but may be decreased in cases with: bone Ca depletion, parathyroid exhaustion or with the use of high dose of vitamin D Serum phosphorus: (N = 4.5-6.5 mg/dl) • Serum alkaline phosphatase: ↑↑ (Earliest manifestation) B) Imaging (Radiological improvement start to occur after 2 weeks of vitamin D therapy) Healing (2-3 weeks Healed Active of TTT) 6 Mohamed M. Ismail Case scenario Modified by: Abdel Razak Ali Shadeed Metaphysis Diaphysis Epiphysis Broadening, Cupping and Fraying ↓↓ Bone density Fractures (Greenstick) Double periosteal line ↑↑ Joint space Bone age (Carpal bones) Dense concave white line of calcification Dense straight white line of calcification Still there is manifestations of active rickets (but less severe) Improved bone density Deformities may persist TTT: Prevention: 1. Nutritional education: Value of breastfeeding, proper weaning.... • Diet rich in vitamin D: Oily fish (salmon, sardines), egg yolk, liver, butter, fortified milk • Avoid rachitogenic diet. 2. Vitamin D supplementation [40 IU = 1 µg] • Full-term: 400 IU/day (since birth) • Preterm: 800 IU/day (as early as the 1st month) 3. Sun exposure (UVR) 4. Regular assessment of nutritional state: Early manifestations of rickets Treatment: A) Vitamin D therapy: a. Oral therapy: • Dose: Vitamin D3: 3000-5000 IU/day • Duration: 2-4 weeks b. Parenteral therapy: • Dose: Vitamin D3: 600.000 IU (Shock therapy) • Duration: Single IM dose • Advantages o More rapid o No need for parents’ compliance o Diagnosis of non-vitamin D deficiency rickets • Disadvantages (Side effects) o Tetany o Hypervitaminosis D 7 Mohamed M. Ismail Case scenario Modified by: Abdel Razak Ali Shadeed B) Treatment of complications: a. Tetany: IV Ca gluconate 10% "1 ml/Kg" (Slowly while monitoring heart rate) b. Deformities and Fractures: Orthopedic care (After complete bone healing) c. Infections: proper antibiotics d. Iron deficiency anemia: Iron (6 mg/kg/day) Genetics A 3-days old female infant is referred from a community hospital for bilious vomiting and a heart murmur. The baby was born at 37 weeks gestation to 39-year-old women. On examination, he appears jaundiced and has a flat facial profile, short upward slanting, flat nasal bridge with epicanthal folds; a small mouth with protruding tongue and single palmer crease. A loud holosystolic murmur is heard over the chest. Generalized hypotonia is present What is your diagnosis? Down syndrome mostly non-disjunction with VSD. Mention possible investigations and treatment INV: i. For down syndrome: Prenatal: A- Triple test (15-16 weeks of gestation): ➢ Increase hCG ➢ Decrease α fetoprotein, and unconjugated estriol B- Fetal karyotyping: ➢ Chrionic villous sampling (9-12 weeks of gestation) ➢ Amniocentesis (14-16 weeks of gestation) C- Fetal US: ➢ Nuchal Translucency thickening due to delayed drainage of fluid from upper part of body. ➢ Short femur ➢ Cystic hygroma and duodenal stenosis. Postnatal: A) Laboratory 1. Karyotyping (for patient and his mother) to determine the genetic type of Down syndrome and risk of recurrence. 8 Mohamed M. Ismail Case scenario Modified by: Abdel Razak Ali Shadeed 2. Complete blood count, if leukemia is suspected. 3. Thyroid profile and regular blood glucose checking. B) Imaging 1. Plain X ray: • Chest for pneumonia. • Abdomen to exclude GIT anomalies (in neonates e.g., duodenal atresia). 2. Echocardiography: to exclude cardiac anomalies. 3. Abdominal ultrasonography: to exclude renal and gastrointestinal anomalies. C) Regular hearing and vision testing. ii. For VSD: 1. Chest X ray: • Heart: biventricular enlargement • Chest: Lung plethora 2. ECG: biventricular hypertrophy (mainly the left ventricle). 3. ECHO will assess. • Position and size of the defect and blood flow across. • Pulmonary pressure. • Cardiac dilation and efficacy of contractility. TTT: For down syndrome: rehabilitation and management of complications: 1. Diagnosis and management of complications and associated anomaly e.g., heart failure, chest infections and hearing aids if needed. 2. General measures: special schools for rehabilitation and education. 3. Specific measures: speech therapy and physiotherapy. For VSD: A) Medical: • Infective endocarditis (Prophylaxis and treatment). • Proper nutrition. • Management of HF. • Treatment of chest infections. 9 Mohamed M. Ismail Case scenario Modified by: Abdel Razak Ali Shadeed B) Surgical closure (Surgery or catheter): Indications: a. Large defect with failure of medical treatment b. Infant 6-12 months old with large VSD and pulmonary hypertension • Surgery should be delayed in stable child with moderate VSD. "Spontaneous closure" • Surgery is contraindicated in patients with Eisenmenger syndrome • Heart-lung transplantation is the only surgical option for Eisenmenger syndrome • Pulmonary artery banding: to limit increased pulmonary blood flow may be useful in multiple muscular VSD A 13-year-old girl presents to your clinic for evaluation of short stature. The patient has not yet attained menarche and her mother reports no breast development. She has been well with no chronic medical problems. Her mother is 173 cm and had menarche at age of 12. Her father is 185 cm and started shaving at age of 15 years. On examination her height is 120 cm less than 5th centile she is pre pubertal, has a webbed neck and widely spaced nipples the carrying angle is increased What is your diagnosis: a case of turner syndrome? Mention possible investigations and treatment. INV: 1. Laboratory 1. Karyotyping: 45 XO 2. Hormonal study (gonadal failure); increased FSH and LH 3. Thyroid profile (more prone to hypothyroidism). 2. Imaging 1. X ray to determine bone age 2. Echocardiography: may be aortic coarctation 3. Abdominopelvic ultrasound: may be renal anomalies (horse shoe, ectopic kidney: 40% of cases), uterine anomalies, ovaries (streaks of connective tissues). TTT: 1. Recombinant Growth hormone to reach at least 5 feet height. 10 Mohamed M. Ismail Case scenario Modified by: Abdel Razak Ali Shadeed 2. Estrogens: To induce the development of 2ry sex characters (Start at 11-12 years). 3. Follow up thyroid function. 4. Management of any associated conditions e.g., aortic coarctation Cardiac disorders An 8 year old boy being followed up in the cardiology clinic has a 3 week history of low grade fever of unknown source, fatigue, weight loss, myalgia, and headache. On repeated examination, he is found to have developed a murmur, petechiae and mild splenomegaly. What is your diagnosis: Infective endocarditis Mention possible investigations and treatment INV: Laboratory: Blood culture (repeated 3 times after proper skin decontamination) CBC, ESR, CRP Imaging: CXR, ECG, Echo (vegetations), transesophageal echo (prosthetic valve) TTT: Prevention: ➢ Proper oral hygiene. ➢ IV or Oral Amoxicillin single dose 50mg/kg 1h before dental procedure, or 30 mins before GIT procedure. And 6 h after GIT procedure give amoxicillin half dose. Treatment: Prolonged parenteral therapy according to culture and sensetivety: ➢ Staph: Vancomycin ➢ Streptococcus viridans and enterococci: ampicillin for 4 weeks. ➢ HACEK: ampicillin + gentamycin for 4 weeks Surgical care indicated in: • • • • Fungal infection Worsening valve obstruction or regurge Progressive cardiac failure Periventricular abscess 11 Mohamed M. Ismail Case scenario Modified by: Abdel Razak Ali Shadeed A 10-year-old boy presents with fever and joint pains, initially the pain affected his right wrist, but now affects his left wrist and right ankle. He had tonsillitis 4 weeks previously treated with oral penicillin. On examination, his temp is 38.7 C respiratory rate 20/m, and heart rate 110/m. His left wrist and right ankle are tender What is your diagnosis: Rheumatic fever Mention possible investigations and treatment INV: 1. Acute phase reactants • CBC: Leukocytosis • Elevated ESR and CRP 2. Evidence of recent Streptococcal infection: • Recent scarlet fever. • Positive throat culture. • Rapid antigen test. • Antistreptococcal antibodies. • High titer of: Antistreptolysin O titer (ASOT). Antistreptokinase. Antihyaluronidase. Anti-DNase. 3. Cardiac assessment • CXR: Cardiomegaly • ECG. Tachycardia and prolonged PR interval • Echocardiography: assesses chamber enlargement, valve affection, and cardiac contractility and detects pericardial effusion. TTT: Prevention: 1. Primary prevention • Prevention of Streptococcal infection: good housing and adequate ventilation. 12 Mohamed M. Ismail Case scenario Modified by: Abdel Razak Ali Shadeed • Tonsillectomy for frequent recurrence. • Proper treatment of Streptococcal throat infection: IM Benzathine penicillin 1.200.000 IU once is the best treatment Alternatively oral Penicillin V 15 mg/kg/day or oral amoxicillin 50 mg/kg/day for 10 days Oral erythromycin (20 mg/Kg/day twice daily for 10 days): in patients allergic to penicillin. 2. Secondary prevention (Prevention of recurrence of rheumatic fever) Indicted in all patients with documented history of rheumatic fever or isolated chorea. Drugs used: a. Benzathine penicillin: • IM: every 2-3 weeks (sensitivity skin test...). • Dose: 1.200,000 IU for weight ≥ 20 Kg and 600.000 IU for weight less than 20 Kg. • Duration: 10 years after the last attack or till the age of 21 years whichever longer then reassess: o No RHD: stop prophylaxis. o RHD: continue till the of 40 years or longer. b. Oral penicillin 250 mg twice daily. c. Penicillin sensitive patients: erythromycin 250 mg twice daily. d. Oral sulphadiazine 0.5 gram once daily. Treatment of acute rheumatic fever A) Antibiotics: • IM Benzathine penicillin: 600.000-1.200.000 IU (Sensitivity skin test is essential). • Given to eradicate streptococci and serves as the 1st dose of penicillin prophylaxis. B) Supportive Management a. Diet: • Salt restriction in cases of heart failure. • Fluid restriction in cases of severe heart failure. b. Rest: For patients with arthritis, carditis or heart failure. 13 Mohamed M. Ismail Case scenario Modified by: Abdel Razak Ali Shadeed C) Specific Management a. Arthritis: • Salicylates 100 mg/Kg/day, 4 times/day for 2 weeks followed by 75 mg/Kg/day, 3 times/ day for 2-3 weeks b. Carditis: • Prednisone: 2 mg/Kg/day 4 times daily for 2 weeks with gradual tapering (Over 4 weeks). • Salicylates: 75 mg/Kg/day 3 times daily started with steroid tapering and continued for 6 weeks. c. Chorea: • Phenobarbitone: 15 mg/Kg/day. • Haloperidol: 0.5-2 mg/kg/day. • Carbamazepine or valproic acid can be considered in severe cases. D) Treatment of complications a. Heart failure: see later b. Rheumatic heart disease: • Medical: rheumatic activity and infective endocarditis • Surgical: Valve repair or replacement A 3 months old boy presents with poor feeding, excessive sweating during feeding, and poor growth. On examination his respiratory rate is 80/minute, and blood pressure is 90/65 mmHg in the upper and lower extremities and the heart rate is 180 per minute. The cardiac exam reveals a systolic thrill and a grade 4 pansystolic murmur at the left sternal border What is your diagnosis? VSD complicated with 1st degree heart failure. Mention possible investigations and treatment INV: 1. Chest X ray: • Heart: biventricular enlargement • Chest: Lung plethora 2. ECG: biventricular hypertrophy (mainly the left ventricle) and arrhythmia. 14 Mohamed M. Ismail Case scenario Modified by: Abdel Razak Ali Shadeed 3. ECHO will assess. • Position and size of the defect and blood flow across. • Pulmonary pressure. • Cardiac dilation and efficacy of contractility. 4. Laboratory: arterial blood gas, CBC, ESR, CRP, ASOT and cardiac markers (CK-MB) for heart failure. TTT: For VSD: A) Medical: • Infective endocarditis (Prophylaxis and treatment). • Proper nutrition. • Management of HF. • Treatment of chest infections. B) Surgical closure (Surgery or catheter): Indications: a. Large defect with failure of medical treatment b. Infant 6-12 months old with large VSD and pulmonary hypertension • Surgery should be delayed in stable child with moderate VSD. "Spontaneous closure" • Surgery is contraindicated in patients with Eisenmenger syndrome • Heart-lung transplantation is the only surgical option for Eisenmenger syndrome • Pulmonary artery banding: to limit increased pulmonary blood flow may be useful in multiple muscular VSD For 1st grade heart failure: 1. Supportive measures: • Rest in semi sitting position • Fluid restriction to 60-70% • If distressed, IV fluids are used initially. • Then, nasogastric tube feeding, oral when tolerated Salt restriction • Oxygen therapy: to reduce distress and correct hypoxia . 2. Digoxin therapy to improve myocardial contractility: • Digitalizing dose: IM or IV 0.05mg/kg (given in 3 doses over the first 24 hours) 15 Mohamed M. Ismail Case scenario Modified by: Abdel Razak Ali Shadeed • Maintenance dose: 0.01mg/kg/day in 2 divided doses; IM or IV, oral when tolerated. 3. Diuretic therapy: Furosemide (Lasix) to reduce preload: • IM or IV 1-2mg/kg/dose/ 12 hours. Shift to oral if tolerated • Monitor K and avoid hypokalemia 4. After load reducing agents • ACE inhibitors as Captopril 0.5-2 mg/Kg/day in 2-3 divided doses Treatment of pulmonary edema: grade II as grade I but: 1. Diuretics: higher doses and can use vasodilators as nitroglycerin infusion. 2. Inotropes: IV digoxin and can use others as dobutamine 3. Continuous positive airway pressure (CPAP) or mechanical ventilation according to severity. Treatment of cardiogenic shock: grade III: 1. Inotropic drugs (IV dopamine or dobutamine IV or both or IV milrinone) not digoxin as it is slowly acting and high risk of toxicity 2. Captopril: 0.5 to 6 mg/kg/day in 2 to 4 doses An infant 11 months old presenting with bluish discoloration noticed by the mother 3 months ago. There is history of feeding difficulties, exertional dyspnea and recurrent attacks of cyanotic spells where the baby becomes more cyanosed with marked tachypnea. She becomes more cyanosed following crying. Examination revealed central cyanosis, and ejection systolic murmur over the 2nd left Space/ left sternal border. What is your diagnosis? Fallot complicated with cyanotic spell. Mention possible investigations and treatment. INV: 1. CBC: ↑↑ Hb and ↑↑ hematocrit (microcytosis if there is iron deficiency). 2. Chest X ray: Coeur en Sabot (= Boot-shape). 3. ECG: Hypertrophy of the right atrium and right ventricle. 4. ECHO. 16 Mohamed M. Ismail Case scenario Modified by: Abdel Razak Ali Shadeed 5. Cardiac catheterization: if indicated to visualize the coronary and pulmonary arteries. TTT: Treatment of the fallot: A) Medical • Hypercyanotic spells: Positioning (knee-chest position/squatting) O₂ therapy IV fluid NaHCO3: to correct acidosis Sedation (SC Morphine) IV B-Adrenergic blockers (Propranolol): to relax the infundibulum IV alpha agonist (increase systemic resistance) • Iron • Infective endocarditis (Prophylaxis and Rx) • Partial exchange transfusion (using FFP or albumin), When? If hematocrit is > 6570% • Prostaglandin (PG1): in duct dependent pulmonary circulation (done in severe cases presenting in neonates) B) Surgical b. Palliative: Blalock-Taussig (anastomosis between Subclavian artery and the ipsilateral pulmonary artery). Can be considered as "artificial PDA" c. Total correction (at 6-9 months): Closure of VSD, infundibular resection and pulmonary valvotomy A 5 hours old male newborn on the postnatal ward is noticed to be blue around the lips and tongue. The baby was born by normal vaginal delivery and weighted 3.8 kg. The APGAR SCORES were 7 at 1 minute and 8 at 5 min. On examination the temp is 36.6 C, his lips tongue and extremities are cyanosed. He is crying normally. Heart rate is 160 /m femoral pulses are palpable and second heart sound is single, oxygen saturation is 70% in air and does not rise with oxygen mask. What is your diagnosis? Congenital cyanotic heart disease: TGA. Mention possible investigations and treatment. 17 Mohamed M. Ismail Case scenario Modified by: Abdel Razak Ali Shadeed INV: 1. CBC: ↑↑ Hb and ↑↑ hematocrit 2. CXR: • Heart: Egg-on-side, narrow pedicle (narrow upper mediastinum) • Chest: Lung plethora (↑↑ PVMs) 3. ECG: Hypertrophy of the RV 4. ECHO. TTT: 1. Prostaglandin: maintains the patency of ductus arteriosus (immediately after birth) 2. Catheter: balloon atrial septostomy: Rashkind procedure (urgent shunt) 3. Surgical repair: Within the first 2-3 weeks of life: Arterial switch Respiratory disorders A 5 months old boy developed a runny nose and a cough 2 days previously, but has become progressively and has now gone off his feeds. He has two older siblings who also have colds. On examination, he is febrile. 37.8, has clear nasal secretion and dry wheezy cough. His respiratory rate is 65 /m with intercostal and subcostal retraction. On auscultation, there are widespread fine crackles and expiratory wheezes. What is your diagnosis? Acute bronchiolitis Mention possible investigations and treatment. INV: 1. Chest x-ray: hyperinflation of the lungs with focal atelectasis. 2. Blood gas analysis: hypoxia- CO2 retention. 3. RSV antigen detection from nasopharyngeal secretions. TTT: 1. Infants with minimal or mild respiratory distress (at home): a) Close observation: Increasing distress is an indication for hospitalization. b) Careful feeding to avoid aspiration. c) Drugs as cough medicines and bronchodilators are generally not helpful. 18 Mohamed M. Ismail Case scenario Modified by: Abdel Razak Ali Shadeed 2. Infants with moderate to severe respiratory distress (at hospital): a) Oxygen therapy to correct hypoxemia. b) I.V maintenance fluid therapy to prevent dehydration. c) Nebulized salbutamol may be used and some infants may benefit. d) Mechanical ventilation may be rarely used in those with severe respiratory failure not responding to the above measures e) Don’t use antivirals or corticosteroids. A 3-year-old boy was seen because of a cough and fever and was diagnosed as having a viral upper respiratory tract infection. On examination, he miserable, flushed, toxic and febrile 38.8 C. His pulse is 140 beats/m His respiratory rate is 48 breath/m with nasal flaring. There is dullness to percussion in the right lower zone post. With decreased breath sounds and bronchial breathing What is your diagnosis? Lobar pneumonia Mention possible investigations and treatment. INV: 1. Chest X-ray: a. Confirm the diagnosis. b. Complications: effusion- empyema- pneumothorax- lung abscess. 2. Blood gas: in severe cases lowered O2 tension and raised CO2. 3. CBC, ESR, CRP: DD/ bacterial and viral causes. 4. Culture and sensitivity test: morning nasopharyngeal aspirate or sputum culture. TTT: For pneumonia: A. Hospital management (7-10 days): 1. Indications • Severe pneumonia (severe RD) or complicated pneumonia. • Small infants (Less than 6 months). 2. Supportive measures: Humidified oxygen - IV fluid (NPO) - suction - mechanical ventilation. 19 Mohamed M. Ismail Case scenario Modified by: Abdel Razak Ali Shadeed 3. Specific treatment: Broad spectrum combined parenteral antibiotics to cover G+/(Ampicillin 50-100 mg/kg/day + gentamycin 4-6 mg/kg/day). Antibiotics may be changed according to results of culture and sensitivity and clinical response. 4. Treatment of complication: • Drainage of empyema. • Mechanical ventilation (in respiratory failure). B. Home management for most cases: 1. In Older children with mild pneumonia without distress. 2. Oral or better intramuscular antibiotics. 3. Amoxicillin 50 mg/kg/day or better broader-spectrum antibiotics such as amoxicillin-clavulanic acid for 7-10 days. A 3 years old girl presented 4 days previously with a cough and fever and was diagnosed with viral upper respiratory tract infection. On examination, she is febrile 38.8 with capillary refill of 2 seconds. Her pulse is 140/m, oxygen saturation is 85% in air and BP is 85/60. Her respiratory rate is 48/m with nasal flaring. There is dullness to percussion in the right lower zone post. With decreased breath sounds and bronchial breathing. Oxygen saturation is given, however respiratory distress worsens (RR more than 60/m, severe Intercostal recession. She becomes cyanosed and lethargic What is your diagnosis? Pneumonia with respiratory failure Mention possible investigations and treatment. INV: 1. Chest X-ray: a. Confirm the diagnosis. b. Complications: effusion- empyema- pneumothorax- lung abscess. 2. Blood gas: in severe cases lowered O2 tension and raised CO2. 3. CBC, ESR, CRP: DD/ bacterial and viral causes. 4. Culture and sensitivity test: morning nasopharyngeal aspirate or sputum culture. TTT: A. Hospital management (7-10 days): 1. Indications 20 Mohamed M. Ismail Case scenario Modified by: Abdel Razak Ali Shadeed • Severe pneumonia (severe RD) or complicated pneumonia. • Small infants (Less than 6 months). 2. Supportive measures: Humidified O2 - IV fluid - suction - mechanical ventilation. 3. Specific treatment: Broad spectrum combined parenteral antibiotics to cover G+/(Ampicillin 50-100 mg/kg/day + gentamycin 4-6 mg/kg/day). Antibiotics may be changed according to results of culture and sensitivity and clinical response. 4. Treatment of complication: • Drainage of empyema. • Mechanical ventilation (in respiratory failure). B. Home management for most cases: 1. In Older children with mild pneumonia without distress. 2. Oral or better intramuscular antibiotics. 3. Amoxicillin 50 mg/kg/day or better broader-spectrum antibiotics such as amoxicillin-clavulanic acid for 7-10 days. A 12-year-old boy is referred by his GP with a chronic nocturnal cough. He has been losing weight and has had a poor appetite 3 months ago. He lies with his mother and three younger siblings in a damp two bed room flat and his mother has also been coughing a lot over the last month with occasional blood-tinged sputum. They are uncertain which immunizations he has received. On examination, he is very thin and his weight is on the 3rd percentile. His heart rate is 80/m and his respiratory rate is 26 breaths /m. There is no wheeze but there are bronchial breath sounds in the right upper zone of his chest What is your diagnosis? TB pneumonia Mention possible investigations and treatment. INV: 1. CBC: Lymphocytosis. 2. ESR: Very high ESR usually above 100. 3. Tuberculin test: Mantoux test is the most important immunological diagnostic tool. 4. Isolation and culture of organism: • Set sputum or morning gastric aspirate • Direct smear with ZN stain • Culture on a Lowenstein Jensen medium (4 weeks) 21 Mohamed M. Ismail Case scenario Modified by: Abdel Razak Ali Shadeed • BACTEC culture (10 days only) 5. Biopsy of lymph nodes or pleura: For pathological study. 6. Radiological studies: Chest x-ray and chest computed tomography (CT scan). 7. Recent methods for diagnosis: Usage of ELISA and PCR (polymerase chain reaction). 8. QuantiFERON TB test: (good negative test) it depends on the release of IFN Y from the patient's T lymphocytes. TTT: Prevention: 1. General measures: • Good nutrition and pasteurization of milk. • Early diagnosis and treatment by mass radiography 2. BCG vaccine (live attenuated) 3. Chemoprophylaxis by isoniazid. Treatment: 1. Combination of anti-tuberculous Drugs. 1st 2 months (4 first line drugs) then the following 4 months continue on Rifampicin and isoniazid only. First line: • Isoniazid (15mg/kg/day) • Rifampicin (20mg/kg/day) • Ethambutol (20mg/kg/day) • Pyrazinamide (20-40mg/kg/day) Second line: • Ethionamide (20mg/kg/day) • Streptomycin (20-40mg/kg/day) 2. Corticosteroids is indicated in: • Allergy to anti tuberculous drugs • Spread: TB serositis or miliary TB • To avoid post operative fibrosis: after surgical removal of gland or TB cervical lymph node. A9 year old boy is seen in the emergency department. This is his third attendance with an acute wheeze in the 3 months. He has developed a cold and became acutely breathless. 22 Mohamed M. Ismail Case scenario Modified by: Abdel Razak Ali Shadeed On examination, he is quite but able to answer questions with short sentences. His chest is hyper inflated and he is using his accessory muscles of respiration. On auscultation there is equal but poor air entry with widespread expiratory wheeze. His pulse is 125 /m with good perfusion. What is your diagnosis? Acute severe Asthma Mention possible investigations and treatment. INV: • PEFM, spirometry: decreasing flow • Skin prick test and seum specific IgE. • CBC: peripheral blood eosinophilia. • Chest X-ray TTT: 1. Management of acute attack: Goal ( PEF 60-80 %) Fatal: ICU, Oxygen, SABA and prepare for intubation Severe: Oxygen, SABA, high dose corticosteroids, ipratropium bromide, IV Mg. Mild- Moderate: Oxygen, SABA, oral corticosteroids, ipratropium bromide 2. Controllers: Less than 5 years: Infrequent: --- Exacerbation ≥ 3 /year: low dose ICS Not controlled on low dose ICS: double dose ICS Not controlled on double dose ICS: add LTRA (montelukast), increase dose and frequency of corticosteroids 6 years and more: ˂ twice / month: --≥ twice / month: low dose ICS 23 Mohamed M. Ismail Case scenario Modified by: Abdel Razak Ali Shadeed Weekly: low dose ICS + LABA OR medium dose ICS Weekly + low lung function: medium dose ICS + LABA Severe: add anti IgE or anti IL5 3. Avoid triggering factor 4. Patient and family education: encourage vaginal delivery and breastfeeding, discourage the use of antibiotics in first year of life, avoid exposure to allergens and tobacco. Hepatology A 12-year-old boy is brought to the clinic by his parents He has been complaining of mild abdominal pain and his parents noticed that the sclera looked yellow. He has been scratching himself mildly. The parents report that he had flu like illness with fever nausea and poor appetite over the last 10 days. He has previously been healthy, but he went to a summer camp 5 weeks previously on examination he is jaundiced and appears uncomfortable but alert and fully oriented. His temp is 37.8 C and his liver is palpable 4 cm below the costal margin and mildly tender. What is your diagnosis? Acute hepatitis Mention possible investigations and treatment. INV: To diagnose liver cell injury 1. Bilirubin: direct or mixed hyperbilirubinemia. 2. AST (aspartate aminotransferase) and ALT (alanine aminotransferase) are elevated (10 folds). 3. Urine analysis: bilirubin is present. To diagnose acute hepatic failure 1. Raising Bilirubin level (above 10 mg/dl, level above 20 mg/dl may also occur. 2. Liver transaminases (ALT, AST) are increased (10-100 times normal). 3. Low serum albumin (below 3 gm/dl occurs later because of long half-life of albumin 4. INR (international normalized ratio) ≥2, uncorrectable with vitamin K or between 1.5 and 1.9 uncorrectable with vitamin K plus encephalopathy. 24 Mohamed M. Ismail Case scenario Modified by: Abdel Razak Ali Shadeed 5. High blood ammonia level 6. Metabolic Acidosis. 7. Hypoglycemia, Hypokalemia and Hyponatremia. To diagnose the causative virus (hepatitis markers) 1. Hepatitis A: • IgM antibodies to hepatitis A (anti HAV IgM): recent infection (acute disease). • IgG anti HAV antibodies persist after recovery (immunity). 2. Hepatitis B: • HBsAg (surface antigen) followed by anti HBc (core antibodies) IgM: indicate recent infection. • Anti-HBs (surface antibodies) appear after recovery and indicate immunity. • Persistence of HBsAg (surface antigen) and anti HBc IgG indicate chronicity. 3. Hepatitis C • Anti-HCV antibodies: these antibodies denote exposure to infection but do not mean recovery or development of immunity. • HCV-RNA antigen detected by PCR denotes viremia. • Viral load can be assessed by quantitative PCR for treatment purposes. 4. Hepatitis D and E • Anti-HDV and anti HEV antibodies (IgM). • Hepatitis D virus antigen. TTT: Treatment of any correctable condition: 1. Post viral: oral Antiviral drugs e.g., for hepatitis B. oral direct acting antivirals (DAA) for hepatitis C e.g., nucleotide/nucleoside analogue 2. Autoimmune hepatitis prednisolone and azathioprine. 3. Wilson disease: Penicillamine is a copper chelating agent (promotes urinary copper excretion) Liver supportive measures: 1. Nutritional support 25 Mohamed M. Ismail Case scenario Modified by: Abdel Razak Ali Shadeed • • Vitamin, minerals and carbohydrate rich diet. Fat in the form of medium chain triglycerides. 2. Ascites • Sodium and fluid restriction and diuretics. • Albumin infusions or Paracentesis for refractory ascites. 3. Encephalopathy • Treat precipitating factors e.g., GIT bleeding, sepsis • Oral neomycin • Protein restriction • Oral lactulose to ↓ ammonia reabsorption (it ↓ colonic pH and ↑ colonic transit) Liver transplantation in end stage liver disease: survival rate 70-90%. 4 weeks old full-term female presents to the office with increasing jaundice over the last week. Her parents' report that 2 weeks previously, she began to have yellow eyes and skin her stools is clay in color for the past 10 days along with dark urine. There were no complications noted at birth. On examination her weight and height are at the 50th percentile. Her skin is jaundiced with scleral icterus. Her liver is felt 4 cm below the costal margin firm in consistency and no splenomegaly is noted. What is your diagnosis? Neonatal cholestasis Mention possible investigations and treatment. INV: 1. Search for treatable cause: • Galactosemia: reducing substance in urine or Galactose 1phosphate in blood while the infant is receiving a lactose containing formula. Measuring the enzyme galactose-1-phosphate-uridyl transferase in red cells. • Septicemia and other bacterial infections: CBC, CRP, ESR, cultures. 2. Search for TORCH infections: • Total IgM antibody: level above 18-20mg/dl is highly suggestive. • Specific IgM antibodies of TORCH agents e.g. CMV IgM. Must be done early in life within 3 weeks of age. 26 Mohamed M. Ismail Case scenario Modified by: Abdel Razak Ali Shadeed 3. Search for metabolic conditions: • α-antitrypsin assay (α-antitrypsin deficiency): normal serum level 150- 250mg/dl • Tyrosinemia: → succinyl acetone in urine and aminogram. 4. Exclude choledochal cyst: • By abdominal ultrasonography (visualization of a cyst separate from the gall bladder related to biliary system). 5. Differentiation between neonatal hepatitis and extrahepatic biliary atresia: • Persistent clay-colored stools, inspected by physician, suggestive of biliary atresia • Abdominal ultrasound: non visualization of gall bladder in fasting infant suggestive of biliary atresia Liver biopsy: the most important • in neonatal hepatitis: giant cell transformation and degeneration of hepatocyte. • In atresia: expansion of portal areas with fibrosis and bile duct proliferation. HIDA scan: Hepatobiliary iminodiacetic acid (radioisotopic scan): appearance of the dye in the intestine excludes biliary atresia. TTT: A. Extra hepatic biliary atresia: early diagnosis is very important: 1. Correctable lesion (rare): direct drainage. 2. Non correctable lesion: Kasai operation, it must be done before 60 days to obtain best results. 3. Liver transplantation for end stage liver disease. 4. Biliary atresia is the commonest indication for liver transplantation in infancy. B. Treatment of correctable conditions: 1. Antibiotics for septicemia or urinary tract infections. 2. Elimination of lactose from diet in Galactosemia. 3. Surgical treatment of choledochal cyst. C. Liver supportive measures: 1. Replacement therapy: • Fat soluble vitamins, medium chain triglycerides, Pre-digested formula 27 Mohamed M. Ismail Case scenario Modified by: Abdel Razak Ali Shadeed 2. Symptomatic: • Pruritis: bile acid binders as cholestyramine • Varices: injection sclerotherapy or band ligation • Hepatic encephalopathy: 10% glucose infusion, enema and oral neomycin D. Liver transplantation: most common indication in infancy, especially in EHBA. A 2-year-old girl was brought to the emergency department with sudden severe hematemesis. History revealed umbilical sepsis during neonatal period. The child appears pale and physical examination reveals a firm spleen palpable 7 cm below costal margin. She does not exhibit hepatomegaly ascites or lymphadenopathy Laboratory findings show a hemoglobin level of 6 gm/dl. What is your diagnosis? Portal hypertension - pre hepatic type Mention possible investigations and treatment. INV: 1. Upper GIT endoscopy: detect esophageal varices 2. Abdominal ultrasonography and Doppler: • Direction of flow within the portal system. • Patency of the portal vein and. • Presence of portosystemic collaterals. 3. CT angiography and MR venography (demonstrate vessel patency). 4. Liver function test in other types. 5. Investigation for the cause: • Hepatitis markers, autoimmune screening, sweet chloride test, liver biopsy. TTT: Management of variceal hemorrhage: 1. Emergency therapy for bleeding varices: • Hospitalization: Anti-shock measures: blood transfusion, intravenous fluids. • Correction of coagulopathy: vitamin k, fresh plasma, platelets transfusion. • Nasogastric tube placement. • H2 receptor blocker (ranitidine) IV to decrease risk of bleeding from gastric erosion. • Third generation cephalosporins. 28 Mohamed M. Ismail Case scenario Modified by: Abdel Razak Ali Shadeed • Vasopressin infusion if bleeding persists. 2. Emergency endoscopy (if hemodynamically stable): and either injection sclerotherapy or band ligation. 3. Emergency shunt: • Trans jugular intrahepatic Porto-systemic shunt (TIPSS) • Surgical Porto-systemic shunts Prevention of bleeding from varices: 1. Prevention of the first attack of bleeding: • Avoid aspirin and non-steroidal anti-inflammatory drugs. • β adrenergic blockers (Propranolol) to lower the pressure in portal area. • Prophylactic sclerotherapy or band ligation. 2. Prevention of re-bleeding; in addition to the above, the following may be needed: • Beta adrenergic blockers (propranolol) Endoscopic sclerotherapy or band ligation • Surgical porto-systemic shunt • Liver transplantation GIT disorders 1 year old girl has had diarrhea and non-bilious vomiting for 2 days. In the fast 8 hours. He vomited 5 times and passed 6 liquid stools. The infant passed a very little amount of urine during the last day. On examination, HR is 135/m, respiratory rate is 45/m, his temp is 37.7 and his capillary refill time is less than 2 seconds. His anterior fontanelle is depressed; eyes are sunken, skin pinch returns back slowly. What is your diagnosis? Gastroenteritis with dehydration. Mention possible investigations and treatment. INV: For Gastroenteritis: A. To identify the causative organism: 1. Stool analysis: Parasites (E. histolytica & G. lamblia), WBCs (Bacterial etiology) 2. Stool culture & sensitivity: Bacteria & viruses 3. CBC, CRP, ESR, Blood culture: help in diagnosis of bacterial infections: B. To detect complications: 29 Mohamed M. Ismail Case scenario Modified by: Abdel Razak Ali Shadeed 1. KFTs (Urea & Creatinine): Renal failure. 2. Electrolytes: Na, Ca, K. 3. Blood gases: Metabolic acidosis. 4. Prothrombin time, platelet count, fibrin degradation products: DIC. TTT: A) Home management: 1- Oral rehydration solution (glucose facilitated sodium absorption) Dose: Mild (50-80 ml/kg), moderate (80 ml/kg) Route: 1 spoon every 2 mins or nasogastric tube 2- Feeding 3- Symptomatic Treatment: Antipyretics and anti-emetics. 4- Specific Treatment: Metronidazole ( parasites), Tetracycline (cholera), Ampicillin (shigella or salmonella). However antibiotics may interfere with normal flora so increasing pathogens. 5- If persistent diarrhea: give lactose free formulas, soy protein based formula, and vitamin A B) Hospital management: IV fluid therapy for isonatemic dehydration Shock therapy: 10 ml normal saline over 20-30 mins. May be repeated by max. 30 ml. Deficit therapy: mild (50ml/kg) moderate (80ml/kg) severe (100ml/kg) of glucose: saline ratio 1:1 over 6-8h. Add K when patient passes urine and serum K is not elevated. Maintenance therapy: 1st 10 kg (100ml/kg) 2nd 10 kg (50ml/kg) the (20ml/kg) of glucose 5% to saline ratio 4;1 over 16-18 h • If dehydration is still present: give maintenance +deficit. • If dehydration has been corrected switch to oral intake. IV fluid therapy for hypernatremic dehydration Shock therapy: same. Deficit therapy: glucose: saline ratio 2:1 over 6-8h. Add K 30 Mohamed M. Ismail Case scenario Modified by: Abdel Razak Ali Shadeed Maintenance therapy: same. • maintenance and deficit should be added and given over the period of correction (1.25 times maintenance rate) • correction should be slowly to avoid brain edema. Na must not decrease more than 12 mEq/L per 24h. • Resistant cases may need dialysis IV fluid therapy for Hyponatremic dehydration Shock therapy: same Deficit therapy: glaucose to saline ratio is 1:1 A 9 months old male infant is brought to the emergency department by his mother with a 4-days history of vomiting and diarrhea. On examination, respiratory rate is 45/m unlabored. Proximal pulses are poor, distal pulses are absent and extremities are cool. Capillary refill is 8 sec. HR is 185 /m and BP is 85/40. The infant is extremely lethargic and responds to pain only, with a minimal grimace. What is your diagnosis? Gastroenteritis + Hypovolemic shock. Mention possible investigations and treatment. INV: For Gastroenteritis: A. To identify the causative organism: 1. Stool analysis: Parasites (E. histolytica & G. lamblia), WBCs (Bacterial etiology) 2. Stool culture & sensitivity: Bacteria & viruses 3. CBC, CRP, ESR, Blood culture: help in diagnosis of bacterial infections: B. To detect complications: 1. KFTs (Urea & Creatinine): Renal failure. 2. Electrolytes: Na, Ca, K. 3. Blood gases: Metabolic acidosis. 4. Prothrombin time, platelet count, fibrin degradation products: DIC. 31 Mohamed M. Ismail Case scenario Modified by: Abdel Razak Ali Shadeed TTT: Treatment of dehydration: A) Home management: 6- Oral rehydration solution (glucose facilitated sodium absorption) Dose: Mild (50-80 ml/kg), moderate (80 ml/kg) Route: 1 spoon every 2 mins or nasogastric tube 7- Feeding 8- Symptomatic Treatment: Antipyretics and anti-emetics. 9- Specific Treatment: Metronidazole ( parasites), Tetracycline (cholera), Ampicillin (shigella or salmonella). However antibiotics may interfere with normal flora so increasing pathogens. 10- If persistent diarrhea: give lactose free formulas, soy protein based formula, and vitamin A B) Hospital management: IV fluid therapy for isonatemic dehydration Shock therapy: 10 ml normal saline over 20-30 mins. May be repeated by max. 30 ml. Deficit therapy: mild (50ml/kg) moderate (80ml/kg) severe (100ml/kg) of glucose: saline ratio 1:1 over 6-8h. Add K when patient passes urine and serum K is not elevated. Maintenance therapy: 1st 10 kg (100ml/kg) 2nd 10 kg (50ml/kg) the (20ml/kg) of glucose 5% to saline ratio 4;1 over 16-18 h • If dehydration is still present: give maintenance +deficit. • If dehydration has been corrected switch to oral intake. IV fluid therapy for hypernatremic dehydration Shock therapy: same. Deficit therapy: glucose: saline ratio 2:1 over 6-8h. Add K Maintenance therapy: same. • maintenance and deficit should be added and given over the period of correction (1.25 times maintenance rate) • correction should be slowly to avoid brain edema. Na must not decrease more than 12 mEq/L per 24h. 32 Mohamed M. Ismail Case scenario Modified by: Abdel Razak Ali Shadeed • Resistant cases may need dialysis IV fluid therapy for Hyponatremic dehydration Shock therapy: same Deficit therapy: glaucose to saline ratio is 1:1 Treatment of shock: Monitoring: Clinical (HR, RR, BP, Temperature), CO, SVR, Laboratory (blood gases, electrolytes, blood sugar and cultures), Imaging (echo, x-ray). Cardiovascular support: Oxygen therapy Preload augmentation ( crystalloid) Inotropes(dopamine, dobutamine or adrenaline) Antiarrhythmic drugs Vasopressors (noradrenaline) Afterload reduction ( sodium nitroprusside) Specific treatment of the cause: Antibiotics for sepsis Urgent surgery for bleeding Chest tube for pneumothorax Pericardiocentesis in cardiac tamonade A 7 weeks old infant presents to the emergency department with 1 week history of nonbilious vomiting. His mother describes the vomit as very forceful. He has a good appetite but has lost 300 gm since he was last weighed a week earlier. He has mild constipation. On examination he is mildly dehydrated, his pulse is 170 beats/m, blood pressure 82/43 mm Hg and peripheral capillary refill 2 seconds. An olive like mass is felt in the right upper quadrant of the abdomen. What is your diagnosis? Congenital hypertrophic pyloric stenosis. 33 Mohamed M. Ismail Case scenario Modified by: Abdel Razak Ali Shadeed Mention possible investigations and treatment. INV: Laboratory: 1. Hypochloremic metabolic alkalosis. 2. Hypokalemia. Imaging: 1. Abdominal US: confirm the diagnosis. 2. Barium meal: only in doubtful cases (Narrow pyloric canal “string sign”. TTT: 1. Correction of fluid & electrolyte disturbance. 2. Pyloromyotomy. A 7 months old infant was referred with a 2-days history of diarrhea with blood and mucous in the stools. His mother states that he has periods of inconsolable crying which are getting worse and more frequent. There is no history of contact with gastroenteritis, of travel, or of bleeding disorders. On examination he has a temp of 37.9 his pulse rate 186/m, blood pressure is 80/44 and capillary refill is 3 seconds. He is difficult to examine due to frequent crying. But when examined during. a period of quiet, a sausage-like mass is felt on the right side of the abdomen. What is your diagnosis? Intussusception. Mention possible investigations and treatment. INV: 1. Abdominal US. 2. Plain X ray abdomen: distended loops, absence of gas in distant colon. 3. Barium enema: Claw sign of coiled spring sign. TTT: Depends of the time of presentation: 1. Air enema: pneumatic reduction. 2. Resection-anastomosis: in neglected cases. 34 Mohamed M. Ismail Case scenario Modified by: Abdel Razak Ali Shadeed Endocrinal disorders The mother of a 2-week-old infant reports that since birth her infant sleeps most of the baby; she has to awaken her every 4 hours to feed, and she will take only an ounce of formula at a time. She also is concerned that the infant has persistently hard pellet like stools On examination, you find an infant with normal weight and length, but with an enlarged head. The heart rate is 75 beats/m and the temp is 36 C the child is still jaundiced. You note large anterior and posterior fontanelle, a distended abdomen and an umbilical hernia What is your diagnosis? Congenital hypothyroidism. Mention possible investigations and treatment. INV: Laboratory: Thyroid profile: T3, T4 and T.S.H. 1. Low T4 (normal range 5-12 microgram/dl). 2. High TSH (normal: 0.5 - 4 micro unit/L). Imaging: 1. Plain X ray: delayed bone age. 2. Thyroid scanning (radioactive iodine) essential to differentiate between aplasia, ectopic dysplasia, and malfunction of the thyroid gland) 3. Thyroid Ultrasonography Neonatal thyroid screening 1. Timing: between 3 and 7th day of life. 2. Technique: a blood drop is obtained by heel prick on filter paper and analyzed for TSH. 3. Interpretation: if TSH level > 20 mU/L, an immediate blood sample is withdrawn and analyzed. If data are confirmed treatment is immediately initiated. TTT: Lifelong therapy with L-thyroxin to maintain normal growth, T3 and T4 1. Onset: before 2 weeks to 3 weeks of age. 2. Dose: • Neonates: 10-15 microgram/kg/day. • Children: 100 microgram/m²/day. 3. Monitoring: 35 Mohamed M. Ismail Case scenario Modified by: Abdel Razak Ali Shadeed • Clinical assessment Height (every 3 months) and Bone age (every year), Mental development, I.Q, and Sexual. • Laboratory assessment: good response is associated with: T4 on high normal, TSH on low normal. An 11-year-old girl is admitted with drowsiness and difficulty breathing. She was well until 3 weeks previously, when she began feeling tired, thirsty and losing weight. On examination she responds only to pain. Her breath has an abnormal smell, is deep and rapid 25/m, her hear6 rate is 100/m and her temp is 36.8 C. She has cool peripheries and capillary refill time of 5 seconds What is your diagnosis? Diabetic ketosis. Mention possible investigations and treatment. INV: 1. Blood glucose: hyperglycemia (blood sugar above 200 mg/dl) 2. Blood gas analysis: metabolic acidosis (pH<7.3, bicarbonate <15 mEq/L) 3. Urine analysis: glucosuria and ketonuria 4. Urea, creatinine and electrolytes (especially potassium) 5. Evidence of a precipitating cause e.g., infection (blood picture, blood and urine cultures. TTT: 1- ABC 2- Fluid therapy ( if glucose < 300 then add glucose) 3- Insulin therapy (0.1unit/kg slow infusion) to avoid brain edema. No boluses 4- Potassium 5- Treat Complications: Shock, brain edema, pulmonary edema, and arrythmia. 6- Monitoring 7- Don’t use Bicarbonate to avoid the risk of alkalosis Blood disorders A 5-year-old girl is referred to the pediatric clinic because of pallor and progressive abdominal distention with a history of repeated blood transfusion since the age of 6 months. On examination she appears pale and she has yellow sclera. Her heart rate is 105/m. Her height and weight are below the 5th centile. She has a grade 2/6 ejection 36 Mohamed M. Ismail Case scenario Modified by: Abdel Razak Ali Shadeed systolic murmur at the left sternal edge she has HSM, BUT NO PURPURA OR GENERALIZED LYMPHADENOPATHY What is your diagnosis? Thalassemia major ( Cooley’s anemia) Mention possible investigations and treatment. INV: • CBC: low Hb, microcytosis, anisocytosis ( size), target cells, poikilocytosis (shape). • Hemoglobin electrophoresis or High-Performance Liquid Chromatography (HPLC): In the affected child: Hb F is markedly elevated (10-90%) with reduced Hb A. Parents: increased of Hb A2 > 3.5% (normal: 3%). TTT: • Supportive: Ca, vit D, folic acid (1mg/day), Hepatitis B vaccine, avoid iron • Exchange transfusion (10-15ml/kg/month) • Iron chelating agents: after 10 times of blood transfusion. • splenectomy after age of 4: vaccination of pneumococci, meningococci, and H.influenza before splenectomy. And prophylactic penicillin after splenectomy. • BM transplantation: better before age of 3. • Hydroxyurea: stimulate Hb F production • Treatment of complication: insulin therapy, Growth hormone, cholecystectomy. • Gene therapy A 2-year-old previously well boy is brought to the office by his aunt. She reports that he developed pallor and red urine and jaundice over the past few days. He has not been exposed to a jaundiced person, but he is talking Sulphamethoxazole- trimethoprim for otitis media His aunt seems to recall his 4-year-old brother having had similar problem after a viral illness, which also caused a short-lived period of anemia and jaundice What is your diagnosis? Acute hemolytic anemia: G6PD. Mention possible investigations and treatment. INV: During the attack: • CBC: Anemia (normocytic normochromic) • Chemistry: indirect hyperbilirubinemia -hemoglobinemia - hemoglobinuria 37 Mohamed M. Ismail Case scenario Modified by: Abdel Razak Ali Shadeed • Blood film shows Heinz bodies and reticulocytosis Between episodes: normal. Estimation of enzyme activity after at least 3 weeks of hemolytic attack, because immediately after hemolysis, bone marrow release reticulocytes with normal enzyme level giving misleading normal result. TTT: • Urgent packed red cell transfusion (10 ml/kg) is lifesaving in very severe hemolysis • Prevention of subsequent attacks. An 18-month-old boy presents with a chief complaint of pallor. He is a picky eater taking small amounts of meat and some vegetables; he survives mainly on bottles of milk. On examination. He looks pale but not icteric. He is an active toddler. His heart rate is 100/m and he has grade 2/6 ejection murmur at the left sternal edge. What is your diagnosis? Iron deficiency anemia. Mention possible investigations and treatment. INV: Blood picture: • Low Hb. • Microcytic hypochromic anemia • Color index = Hb% divided by (RBC X2). It will be below 1. • Reticulocytic count is normal. It shows mild increase with therapy. Blood chemistry: • Low serum iron < 50mcg % (normal: 90-150 µg/dl). • Low serum ferritin <10 ng (normal: 30-150 ng/ml). • Increased iron binding capacity (normal: 250-350 µg/dl). Detect the cause: • Adequate clinical history to discover dietary problems. • Focused and systemic clinical examination to rule out other causes of anemia. • Stool analysis: to detect Ankylostoma - blood in stool – bilharziasis. 38 Mohamed M. Ismail Case scenario Modified by: Abdel Razak Ali Shadeed • Endoscopy might be indicated: to exclude peptic ulcer or chronic H-Pylori infection. TTT: Prevention: 1. Adequate supply of iron to pregnant female. 2. Making powdered formula well-fortified with iron. 3. Prophylactic iron therapy to premature. 4. Proper weaning by supplying iron containing foods. 5. Avoid cow milk introduction in the first year of life. 6. Provision of appropriate amount of iron rich food for infants and children according to their age and economic resources. 7. Treatment of cause. Treatment of: A. Treatment of the cause: (Schistosomiasis: Praziquantel) (Ankylostoma: Albendazole). B. Iron therapy: a. Oral iron therapy: • Ferrous sulfate or gluconate 3-6 mg/kg elemental iron in 3 divided doses/day • Iron supplementation should be continued until the Hb is normal and then for a minimum of a further 3 months to replenish the iron stores. b. Parenteral iron preparations: • Indications: poor compliance or malabsorption. • Oral therapy is otherwise as fast, as effective, much less expensive and less toxic. • Parenteral iron sucrose and ferric gluconate complex have a lower risk of serious reactions than iron dextran. C. Diet: Rich in iron (Meat five, green vegetables) and vitamin C. Successful iron therapy: • BY DAY 1: Reduced irritability, improved appetite. • By day 2; Erythroid hyperplasia in Bone marrow, show erythroid hyperplasia. • By day 3: Reticulocytosis peaking at 5-7 days (good therapeutic test). • By 1 month: elevated hemoglobin 0.4-1gm/dl. • 4-6 weeks: Increased Stores. 39 Mohamed M. Ismail Case scenario Modified by: Abdel Razak Ali Shadeed A 2 years old child develops bruising and generalized petechie two weeks after a viral syndrome. No HSM or lymph node enlargement is noted. The examination is otherwise unremarkable Laboratory testing shows the patent to have a normal Hb, Hematocrit and white cell count and differential. The platelet count is 15,000. What is your diagnosis? Purpura (ITP). Mention possible investigations and treatment. INV: CBC: • Thrombocytopenia, usually < 20,000/mm³ (Normal: 150,000 - 400.000mm³) • May be low Hb due to blood loss. • Normal WBCs count with relative lymphocytosis. Bone marrow examination: • Megakaryocytes are normal or increased in number with defective budding. Anti-platelet antibodies: are found in 60% of cases. TTT: 1. In mild cases (Cutaneous hemorrhage only): • conservative management and follow up (platelet count >10,000) • Avoid trauma and salicylate, NSAID. • Advice the parents to attend to clinical care immediately if the child has active bleeding other than cutaneous hemorrhage like gum bleeding or epistaxis. 2. In moderate cases (Persistent muco-cutaneous hemorrhage): • Prednisone: Dose 2 mg/kg/d for 2 weeks followed by gradual withdrawal. Action: It inhibits antibody synthesis & phagocytic activity. • Intravenous immunoglobulin (IVIG): dose 0.8-1 gm/kg/day, Duration for 2 days. Action It causes rapid rise of platelet count (block the phagocytic activity) 3. In severe cases: (severe muco-cutaneous hemorrhage or intra-cranial hemorrhage) • I.V. methyl prednisolone 20 mg/kg/day for 5 days • Platelet transfusion after starting steroid +/- Fresh whole blood if needed. • IVIG. • Emergency splenectomy: final solution. 40 Mohamed M. Ismail Case scenario Modified by: Abdel Razak Ali Shadeed 4. In chronic cases: (>12 months): • Careful evaluation for associated disorders (E.g., SLE: frequent screening of autoantibodies). • Prednisone & IVIG. • OR Splenectomy (75% curative). • OR Immunosuppressive therapy (e.g., Rituximab- azathioprine- Cyclosporine). A 4-year-old girl is referred to the pediatric unit with pallor and non-blanching skin rash. She had a cold and sore throat 2 weeks previously. She is otherwise very well. On examination she has no dysmorphic features. Her height and weight are on the 25th centiles. There is no jaundice and she is afebrile. She is pale and clinically anemic There are petechiae mainly on her limbs. No hepatosplenomegaly no enlarged L.N. What is your diagnosis? Purpura most propaply ITP. Mention possible investigations and treatment. INV: CBC: • Thrombocytopenia, usually < 20,000/mm³ (Normal: 150,000 - 400.000mm³) • May be low Hb due to blood loss. • Normal WBCs count with relative lymphocytosis. Bone marrow examination: • Megakaryocytes are normal or increased in number with defective budding. Anti-platelet antibodies: are found in 60% of cases. TTT: 5. In mild cases (Cutaneous hemorrhage only): • conservative management and follow up (platelet count >10,000) • Avoid trauma and salicylate, NSAID. • Advice the parents to attend to clinical care immediately if the child has active bleeding other than cutaneous hemorrhage like gum bleeding or epistaxis. 6. In moderate cases (Persistent muco-cutaneous hemorrhage): • Prednisone: Dose 2 mg/kg/d for 2 weeks followed by gradual withdrawal. Action: It inhibits antibody synthesis & phagocytic activity. 41 Mohamed M. Ismail Case scenario Modified by: Abdel Razak Ali Shadeed • Intravenous immunoglobulin (IVIG): dose 0.8-1 gm/kg/day, Duration for 2 days. Action It causes rapid rise of platelet count (block the phagocytic activity) 7. In severe cases: (severe muco-cutaneous hemorrhage or intra-cranial hemorrhage) • I.V. methyl prednisolone 20 mg/kg/day for 5 days • Platelet transfusion after starting steroid +/- Fresh whole blood if needed. • IVIG. • Emergency splenectomy: final solution. 8. In chronic cases: (>12 months): • Careful evaluation for associated disorders (E.g., SLE: frequent screening of autoantibodies). • Prednisone & IVIG. • OR Splenectomy (75% curative). OR Immunosuppressive therapy (e.g., Rituximab- azathioprine- Cyclosporine). A 5 years old girl was noticed to be pale and lacking energy. She was complaining that her legs were aching. She had an ear infection with a high temperature that was slow to respond to antibiotics. On examination she is quite and pale. Her temp is 37.8C. She has a generalized lymphadenopathy. She is pale but not jaundiced. She has bruises on her shins, left and right upper arm. The liver is 4 c below the right costal margin and her spleen is palpable 2 cm below the left costal margin. What is your diagnosis? Leukemia. Mention possible investigations and treatment. INV: 1. CBC: anemia -thrombocytopenia - WBC (increased, decreased or normal) 2. Peripheral blood film may show blast cells 3. Bone marrow examination: is essential to confirm the diagnosis (blast cells) and to identify immunological and cytogenetic characteristics which give useful prognostic information. 4. Lumbar puncture to identify disease of CSF 5. Chest X-ray to identify mediastinal mass characteristic of t-cell disease. TTT: 42 Mohamed M. Ismail Case scenario Modified by: Abdel Razak Ali Shadeed Treatment of Acute Lymphocytic Leukemia (ALL): 1. Supportive treatment: • Blood & platelet transfusion • Treating the infection • Hydration 2. Chemotherapy (combination): • Induction of remission: asparagenase -vincristine -prednisone – cytarabine. • Intrathecal: methotrexate, hydrocortisone- cytarabine. • Systemic continuation therapy: 2-3 years- (6 mercaptopurine – methotrexate). 3. Bone marrow transplantation in relapsing cases. A 7-year-old girl presents with a 3 days history of rash and ankle swelling and painless frank hematuria. She had a cold 4 weeks previously, but has otherwise been healthy She received no medications and is fully immunized. On examination, she has palpabl non blanching purple spots 1-4 mm in diameter especially over the shins and buttock. Her left ankle is swollen, worm and tender with restricted movement. What is your diagnosis? Henoch Schönlein purpura. Mention possible investigations and treatment. INV: On clinical basis (no specific lab finding). • Laboratory: 1. Complete blood picture: normal platelet count. 2. ESR, CRP: elevated (inflammation). 3. Urine analysis (screen for hematuria). 4. Stool analysis (screen for occult blood in stool). 5. Blood Urea, creatinine and C3: screen for nephritis. 6. Ig A level (maybe elevated). • Imaging: 1. Abdominal X ray and ultrasound (gut perforation or intussusception). 2. MRI and MRV with CNS manifestations for diagnosis of cerebral vasculitis. TTT: 1. Supportive treatment with maintenance of good hydration and electrolyte balance. 43 Mohamed M. Ismail Case scenario Modified by: Abdel Razak Ali Shadeed 2. Analgesics 3. Prednisolone (GIT disease and hemorrhage) 4. IV methyl prednisolone and cyclophosphamide (nephritis) 5. Control of hypertension if necessary A 3-year-old boy fell while playing in the garden; he developed a very painful swelling of the right knee. He was born at 37 weeks gestation and experienced a prolonged bleeding after circumcision that necessitated an urgent blood transfusion. A family history of bleeding tendency is reported What is your diagnosis? Hemophilia Mention possible investigations and treatment. INV: 1. prolonged PTT. 2. Specific factor VIII assay (reduced below normal) Normal > 60% & Carrier 3060% (female). • Mild hemophilia 5-30%: (bleeding with trauma or surgery). • Moderate hemophilia 1-5%: (bleeding with minor trauma). • Severe hemophilia < 1%: (spontaneous joint bleeding). TTT: Prevention: Avoid trauma-aspirin - non-steroidal anti-inflammatory- make sure the child is vaccinated against Hepatitis B virus - Physiotherapy prevents joint contracture. Treatment: 1. Cold compresses minimize bleeding in mild cases 2. Replacement: • I.V. infusion of cryoprecipitate (Plasma concentrate of factor VIII) (Dose: 25-50 Unit/kg every 12 hours). • I.V. infusion of Purified plasma derived factor VIII concentrate • IV infusion of Recombinant factor 8. • Prophylactic F VIII in severe hemophilia (2 times per week) 3. Desmopressin: it increases endogenous release of FVIII (ineffective in hemophilia B). 4. Physiotherapy 44 Mohamed M. Ismail Case scenario Modified by: Abdel Razak Ali Shadeed Nephrology A 4-years-old boy presents with a 2 weeks history of general malaise and puffiness of the face. There is nothing of note in the history. Examination reveals generalized puffiness with pitting edema of the lower limbs. There is mid abdominal distention, but no tenderness or organomegaly. his scrotum appears swollen. His B.P is 90/65 mmHg What is your diagnosis? Nephrotic syndrome, Minimal lesion type. Mention possible investigations and treatment. INV: A) Laboratory: a. Urine: • Urinalysis: Proteinuria (3+ or 4+). • Urine protein/Creatinine ratio > 2. • 24-hour urine proteins: > 40 mg/m²/hr. (needs timed urine collection which is difficult). ➢ Proteinuria is selective (mainly albumin, no high molecular weight proteins). b. Blood: • Serum albumin < 2.5 g/dL. • Serum cholesterol > 200 mg/dL. • Kidney functions: Normal. • Complement (C3 & C4): Normal (No consumption). B) Imaging: Renal U/S C) Invasive: Renal biopsy (not routine); indicated in: a. Pre-treatment (when Minimal change nephrotic is unlikely): • Age at onset < 1 yr. or > 10 yrs. • Gross hematuria • Persistent hypertension • Renal impairment • Hypocomplementemia (↓↓ C3 and/or C4) • Family history of NS 45 Mohamed M. Ismail Case scenario Modified by: Abdel Razak Ali Shadeed b. Steroid resistant NS (SRNS) = Failure to achieve remission after 4-6 weeks of steroid therapy TTT: Induction of remission: prednisolone 2mg/kg/day divided on 3 doses after meals for 4 weeks. • If steroid responsive: alternate day therapy with gradual tapering over 3 months. • If steroid resistant: Do renal biopsy, Add ACEI, Add immune suppressive drugs: Cyclosporine: Hypertension+ nephrotoxic Tacrolimus: DM+ nephrotoxic Treatment of relapses: shorter induction and longer maintainance Treatment of frequently relapsing: Cyclophosphamide: leucopenia Mycophenolate mofetil: BM suppression Treatment of edema: salt and fluid restriction+ frusemide Treatment of infection: Antibiotics A 5-year-old boy presents with a 3-days history of smoky colored urine. This is not associated with dysuria, although his urine output is diminished. He had tonsillitis 4 weeks previously. There is no family history of note. On examination, he is paraxial and well. Respiratory rate is 14 /m and pulse is 90/m with a blood pressure of 110/85 mmHg. Abdomen is non-tender with no masses What is your diagnosis? Acute glomerulonephritis. Mention possible investigations and treatment. INV: 1. Laboratory: A. Urine (Urinalysis): • Color: Brown, tea or cola-like or smoky. • Proteinuria (mild to moderate). B. Blood: • KFT (Urea & creatinine): may be impaired. • Evidence of recent Streptococcal infection (↑ ASOT, throat or skin contact). 46 Mohamed M. Ismail Case scenario Modified by: Abdel Razak Ali Shadeed • • ↓↓ in C3 (returns to normal within 8 weeks). Normal C4. 2. Imaging: Renal U/S. 3. Invasive: Renal biopsy is rarely indicated: A. Severe renal impairment (Rapidly progressive GN = RPGN). B. Persistent hematuria or proteinuria > 6-12 months. C. Normal C3. D. Persistent hypocomplementemia > 3 months. TTT: 1. General measures: • Salt restriction, Fluid balance. • Rest: During the oliguric phase 2. Supportive treatment: a. Edema: Salt restriction, fluid balance, diuretics (Furosemide 1-2 mg/Kg/day) b. Hypertension: • Salt restriction, fluid balance, diuretics (Furosemide 1-2 mg/Kg/day) • Ca channel blockers (Amlodipine 0.6 mg/kg/day) 3. Treatment of complications: a. Renal failure: Fluid balance, diuretics, dialysis (in severe cases) b. Heart failure: • Preload reduction: Diuretics • Afterload reduction: ACE inhibitors • Inotropes: Dopamine (digitalis should be avoided) c. Hypertensive encephalopathy: Antihypertensive (IV Hydralazine or diazoxide) A 5-year-old girl has been passing urine frequently for the last 2 days and complaining of pain when doing so. She is febrile and had an episode of shivering. SHE HAS ALSO complained of pain in her lower back and has vomited three times today. On examination her temp is 39.1, her abdomen feels soft and is not distended, but there is significant discomfort when palpating right loin. What is your diagnosis? Acute pyelonephritis. 47 Mohamed M. Ismail Case scenario Modified by: Abdel Razak Ali Shadeed Mention possible investigations and treatment. INV: 1. Initial investigations: A. Urine analysis: • Pyuria (Pus cells> 5/HPF) is suggestive of UTI. • WBC casts is suggestive of pyelonephritis. • Not reliable (false positive & false negative results). False Positive (Pyuria without UTI) False Negative (UTI without pyuria) Fever Antibiotics therapy Dehydration TB Nephritic syndrome (APSGN) Closed infection (Obstructive uropathy) B. Urine Culture: • It is essential for diagnosis and treatment. • Colony count > 100,000 single pathogen is diagnostic. • Any bacterial growth in a suprapubic aspirate or catheter sample is diagnostic. • The presence of more than one organism suggests contamination. C. Others (in pyelonephritis): • CBC: leukocytosis & neutrophilia. • ESR, CRP & blood culture. 2. Imaging studies in children with febrile UTI: A. Ultrasonography. Indications: • First episode of febrile UTI (pyelonephritis). • Frequently occurring lower UTIs (cystitis). Value: • Diagnosis of obstructive uropathy (hydroureter and hydronephrosis). • Detection of renal scarring. B. Renal scan (DMSA) • Detection of renal scarring. • Estimation of renal function (total & split function). 48 Mohamed M. Ismail Case scenario Modified by: Abdel Razak Ali Shadeed C. Voiding cystourethrogram (VCUG) Indications: • Febrile UTI with abnormal renal US. • Febrile UTI with abnormal renal scan (DMSA). Value: • Diagnosis of VUR. • Diagnosis of PUV & neurogenic UB. TTT: • Treatment should be started without delay then modified according to the culture result. • Hospital admission may be indicated if there is severe vomiting, infants < 3 months of age or with poor oral intake 1. Antibiotic Therapy a. Drugs: • Ceftriaxone Or • Cefotaxime Or • Gentamicin + Ampicillin or Oral 3rd gen. cephalosporin (e.g., Cefixime) b. Route: Oral or parenteral c. Duration: 7-14 days. 2. Other Lines (for recurrent UTI) • Adequate fluid intake. • Frequent voiding, ensuring complete bladder emptying, Avoid/treat constipation. • Antibiotic suppressive therapy (Co-trimoxazole: 1/3 therapeutic dose single daily). • Lactobacillus acidophilus (probiotic): Replaces pathogenic bacteria (prevention of UTI). • Cranberry juice: prevents bacterial adhesion. An 8-year-old boy wets the bed most nights, does not walk up when it happens and there is a large pool of urine. He has no previous medical problems and no recent illness. On examination, his height is 140 cm) 75th centile and his weight is 35 kg (75th centile). His 49 Mohamed M. Ismail Case scenario Modified by: Abdel Razak Ali Shadeed blood pressure is 112/70 mmHg. Cardiovascular, respiratory and abdominal examinations are unremarkable. What is your diagnosis? Nocturnal enuresis. Mention possible investigations and treatment. INV: • • • • • • Head and neck x-ray lateral view (adenoid hypertrophy) Blood glucose (DM) Check for UTI: urinalysis, urine culture, CBC, ESR, CRP Check for renal failure: Serum albumin, creatinine, urea Check for sickle cell anemia: CBC, blood film, Hb electrophoresis Psychological assessment TTT: 1. Simple Measures: (Children > 4 years): a. punishment should be avoided. b.↓↓ Fluid intake in the evening b. Urination before sleep c. Waking the child up few hours after sleep to void d. Rewarding for dry nights. 2. Drug therapy (Children > 6 years): a. Anticholinergic (↑↑ Bladder capacity): Oxybutynin: may be needed in nonmonosymptomatic nocturnal enuresis b. Desmopressin (synthetic vasopressin analogue): Single night dose c. Enuresis alarms (conditioning therapy): Auditory or vibratory alarm is attached to a wetness sensor in the underwear (wake the child up for urination) Neurology A 13-month-old male was born at term by difficult vaginal delivery and birth weight was 4200 gm. At 6 months of age, his head control was poor. He is not able to sit or stand. His height and weight are both between the 25-50th percentiles. Some primitive reflexes such as the asymmetric tonic neck reflex persist and he has increased muscle tone especially in his legs. What is your diagnosis? Spastic cerebral palsy. 50 Mohamed M. Ismail Case scenario Modified by: Abdel Razak Ali Shadeed Mention possible investigations and treatment. INV: For Cerebral palsy: 1. CT brain MRI brain • May determine the location and extent of structural lesion. • May show associated malformations or brain atrophy. 2. EEG. 3. TORCH screening for mother and infant to define the cause. 4. Auditory assessment and VEP:(visual evoked potential). For Mental retardation: 1. Karyotyping if a chromosomal abnormality is suspected or if the child is dysmorphic 2. Metabolic screening e.g., blood lactate -plasma amino acids - ammonia 3. Urine: amino acids - organic acids 4. Thyroid function tests. 5. Skeletal Survey. TTT: By rehabilitation: 1. Physiotherapy. 2. Positioning and splints to prevent contracture. 3. Associated complications and neurological problems as a. Epilepsy: antiepileptic drugs b. Orthopedic management: Tendon releases in contracture c. Treatment of chest infection d. Special feeding program A 15 months old boy presents with seizures associated with fever. He has been in good 4.4 health except for a high fever that developed today to about 38.8 C. He has slight cough and mild nasal congestion Just prior to the seizures he was playing with some toys. Past medical history is unremarkable. 104 Examination shows congested throat mucosa, congested ear drums and normal neurological examination What is your diagnosis? Febrile seizures. Mention possible investigations and treatment. INV: 51 Mohamed M. Ismail Case scenario Modified by: Abdel Razak Ali Shadeed Evaluation: 1. Convulsions occur at the onset of rise of body temperature. 2. Evident extracranial Infection: Usually URTI or gastroenteritis 3. Exclude features of CNS Infections 4. Exclude other causes of seizures (trauma - toxins) 5. If we cannot exclude CNS infection: CSF examination is a must. TTT: 1. ABC 2. Lower the temperature: tape water and antipyretics 3. Antiepileptics. 4. Treat the cause: Antibiotics. 5. Long term anti-convulsant is indicated if: • Persistent EEG abnormality. • Atypical febrile convulsions. • Interval less than 3 months between attacks. A 13-month-old girl presents to the emergency department with a 2-days history of lowgrade fever runny nose and increasing fussiness. SHE ALSO DEVELOPED A PETECHIAL RASH AND VOMITING. On examination, her temp is 39.6 C, heart rate is 170/m. Respiratory rate is 50/m and blood pressure is 80/50. Her extremities are mottled and her capillary refill is 4 seconds. While 4 - 4 trying to obtain an IV access, the infant becomes more lethargic and her blood pressure drops further What is your diagnosis? Meningococcal septicemia. Mention possible investigations and treatment. INV: CSF examination: 1. Differentiating bacterial meningitis from tuberculous and viral meningitis (table). 2. Culture and sensitivity tests are essential: negative with (pervious intake of antibiotics or in aseptic meningitis e.g., Viral). 3. Detection of antigens (PCR) and antibodies (ELIZA) of viral infection if viral meningitis is suspected. 4. Ziehl-Nielsen stain of CSF if TB meningitis is suspected (may show acid-fast bacilli). Normal Bacterial Tuberculous Viral Aspect Clear Turbid Opalescent Usually, Clear 52 Mohamed M. Ismail Case scenario Modified by: Abdel Razak Ali Shadeed Protein: mg/dl 20-40 ↑ marked ↑ Normal or mild ↑ 40-80 Glucose: 60% of blood ↓ very low ↓ Normal mg/dl glucose Lymphocytes ↑↑↑ Polymorph. ↑ lymphocytes 250- ↑ lymphocytes Cells/mm³ 0-5 10-100,000 500 15-2000 Other investigations: 1. CBC: marked leukocytosis with bandemia 2. Blood culture 3. Kidney functions test and electrolytes 4. Coagulation screen if DIC is suspected 5. If TB is suspected: chest X ray, tuberculin rest. 6. CT with contrast to detect meningeal enhancement 7. MRI brain for better visualization of cerebral infarcts. TTT: Prevention: • Vaccination: ➢ 1st year: HIB vaccine+ pneumococci ➢ “-3 years: meningococci • Chemoprophylaxis by rifampicin Treatment: 1. Supportive treatment: a. I.V fluid if meningitis is complicated by shock (otherwise fluid is restricted minimize cerebral edema: only 75% of maintenance is given). b. Anticonvulsants c. Assisted ventilation if respiratory failure occurs.co d. d. Subdural taps to evacuate extensive subdural effusions. 2. Specific treatment: a. Antibiotics: IV for at least 10- 14 days (in neonates 3 weeks). • Neonates and infants below 2 months: 53 Mohamed M. Ismail Case scenario Modified by: Abdel Razak Ali Shadeed Third generation cephalosporins e.g., Cefotaxime 200 mg kg/day plus ampicillin 100mg/kg/day. • Infants and children above 2 months: Third generation cephalosporin e.g., Cefotaxime 200mg/kg/day plus vancomycin b. Dexamethasone: in H influenza infection to decrease incidence of gliosis and hearing loss 3. Follow up to detect late complications e.g., Epilepsy and mental retardation by periodic monitoring of neurological and developmental status for (at least 2 years). A 9-year-old boy presents in a confused state. He developed a fever 2 day previously. And E4 had been complaining of headache, fever and photophobia. He had vomited once. Previous history was unremarkable on examination, his temp was 38 C and he has mild neck stiffness and photophobia. Heart rate is 82 beats/m and respiratory rate is 16 breaths/m. there are no other focal signs of infection. What is your diagnosis? Meningococcal septicemia. Mention possible investigations and treatment. INV: CSF examination: 5. Differentiating bacterial meningitis from tuberculous and viral meningitis (table). 6. Culture and sensitivity tests are essential: negative with (pervious intake of antibiotics or in aseptic meningitis e.g., Viral). 7. Detection of antigens (PCR) and antibodies (ELIZA) of viral infection if viral meningitis is suspected. 8. Ziehl-Nielsen stain of CSF if TB meningitis is suspected (may show acid-fast bacilli). Normal Bacterial Tuberculous Viral Aspect Clear Turbid Opalescent Usually, Clear Protein: Normal or mild 20-40 ↑ marked ↑ mg/dl ↑ 40-80 Glucose: 60% of blood ↓ very low ↓ Normal mg/dl glucose 54 Mohamed M. Ismail Case scenario Modified by: Abdel Razak Ali Shadeed Lymphocytes ↑↑↑ Polymorph. ↑ lymphocytes 250- ↑ lymphocytes 0-5 10-100,000 500 15-2000 Other investigations: 7. CBC: marked leukocytosis with bandemia 8. Blood culture 9. Kidney functions test and electrolytes 10. Coagulation screen if DIC is suspected 11. If TB is suspected: chest X ray, tuberculin rest. 12. CT with contrast to detect meningeal enhancement Cells/mm³ 7. MRI brain for better visualization of cerebral infarcts. TTT: Prevention: • Vaccination: ➢ 1st year: HIB vaccine+ pneumococci ➢ “-3 years: meningococci • Chemoprophylaxis by rifampicin Treatment: 1- Supportive treatment: a. I.V fluid if meningitis is complicated by shock (otherwise fluid is restricted minimize cerebral edema: only 75% of maintenance is given). b. Anticonvulsants c. Assisted ventilation if respiratory failure occurs.co d. d. Subdural taps to evacuate extensive subdural effusions. 2- Specific treatment: a. Antibiotics: IV for at least 10- 14 days (in neonates 3 weeks). i. Neonates and infants below 2 months: Third generation cephalosporins e.g., Cefotaxime 200 mg kg/day plus ampicillin 100mg/kg/day. ii. Infants and children above 2 months: Third generation cephalosporin e.g., Cefotaxime 200mg/kg/day plus vancomycin b. Dexamethasone: in H influenza infection to decrease incidence of gliosis and hearing loss 55 Mohamed M. Ismail Case scenario Modified by: Abdel Razak Ali Shadeed 3- Follow up to detect late complications e.g., Epilepsy and mental retardation by periodic monitoring of neurological and developmental status for (at least 2 years). A 4-year-old boy's parents complain that their child has difficulty walking. The child rolled, sat and first stood at normal ages and first walked at 13 months of age. Over the past several months however, the family has noticed an increase inward curvature of the lower spine as he walks and that his gait has become more waddling. On examination, the child is oriented and interactive with the physician with an average body built. Enlargement of his calves has been noticed What is your diagnosis? Duchenne Dystrophy. Mention possible investigations and treatment. INV: 1. Serum CPK (creatine kinase). Marked elevation even at birth (can be used as screening test). 2. Electromyography. 3. DNA studies: deletion on Dystrophin gene. 4. Muscle biopsy: diagnostic (absent Dystrophin). TTT: 1. Nutritional support and physiotherapy. 2. Treatment of chest infections and respiratory aids as CPAP. 3. Gene therapy is now available. 4. Night splints to prevent contractures 5. Corticosteroids: to preserve mobility and prevent scoliosis (its action may be inhibition of muscle proteolysis, anti-inflammatory effect.). Rheumatology For the past 7 weeks, a 3-years-old girl was increasingly reluctant to get up in the morning and feel stiff all over. As the day goes by, her stiffness improves unless she has been sitting for a while. On examination both knees and her right ankle are mildly swollen and feel warm to touch, but there is no overlying skin changes. There is pain and decreased range of movement. There are similar findings in the proximal interphalangeal joint of the left index finger. 56 Mohamed M. Ismail Case scenario Modified by: Abdel Razak Ali Shadeed What is your diagnosis? JIA. Mention possible investigations and treatment. INV: CBC: anemia, leukocytosis, thrombocytosis Elevated ESR, CRP, Ferritin Rheumatoid factor: determine severity X-ray: joint deformaties TTT: 1- NSAID: diclofenac 2- Disease modifying anti-rheumatic drugs: Methotrexate 3- Biological agents: anti tumer necrosis factor-α, anti IL6, anti IL1 4- Steroids: side effects→ cushing syndrome, growth failure, osteopenia. 5- Physiotherapy 6- Family and psychological support 7- Periodic slit lamp examination: anterior uveitis. Infections A 2-years-old boy is referred with a history of fever cough, runny nose and sticky eyes for 6 days. On examination, his temp is 38.5 C. he is miserable and lethargic. He has a wide spread maculopapular erythematosus rash, which is coalescing over the face, neck and trunk. There is no respiratory distress but he is coughing. His nose is streaming with catarrh and he has exudative conjunctivitis. He had not received all his childhood immunizations due to parental concerns regarding vaccine safety What is your diagnosis? Measles. Mention possible investigations and treatment. INV: CBC: leukocytosis Normal ESR and CRP ELISA, PCR TTT: 57 Mohamed M. Ismail Case scenario Modified by: Abdel Razak Ali Shadeed Prophylaxis: 1. Active immunization: MMR vaccine (1st dose: 1 year , 2nd dose: 18 months) 2. Passive immunization with gamma globulin (0.25 ml/kg I.M) 2-5 days after exposure. Treatment: 1. Symptomatic: antipyretics - decongestants. 2. In immunocompromised patients, the antiviral drug ribavirin maybe used. 3. Antibiotics for complications as otitis media and pneumonia. 4. Large doses of gamma globulin in encephalitis to reduce complications. 5. Oral vitamin A (400,000 IU) modulates the immune response (reduces morbidity). A 9 months old boy is brought by his parents because of fever for 5 days He has been slightly irritable and feeling less than usual. His temperature measure at home has been up to 40 C He looks quite well but his temp remains raised and no focus can be found. He is admitted to the ward overnight for observation and his temp subsides. On the morning ward round, he is found to have an erythematosus macular rash on his trunk. What is your diagnosis? Roseola infantum. Mention possible investigations and treatment. INV: CBC: mild leukocytocis Normal ESR and CRP ELISA, Viral culture TTT: 1. Antipyretics: for the high fever. 2. Sedatives: in infants susceptible to febrile convulsions. A 7-years-old boy presents with fever, sore throat and a fine maculopapular rash for 2 days. On examination, he has enlarged erythematosus tonsils with exudates, enlarged tender cervical lymph nodes and a strawberry tongue. A diffuse blanching rash with a rough texture to touch is noted. What is your diagnosis? Scarlet fever. Mention possible investigations and treatment. 58 Mohamed M. Ismail Case scenario Modified by: Abdel Razak Ali Shadeed INV: Diagnosis is mainly clinical 1. Throat culture: group a beta hemolytic streptococcus. 2. Serologic tests: A significant rise of Antistreptolysin O titer (ASO titer). 3. CBC: Leukocytosis (PNL 10,000-20,000/mm³) and anemia 4. Elevated ESR and CRP TTT: Prevention of droplet infection. Treatment: 1. Antibiotic therapy: • Oral penicillin V: 400. 000 IU/dose 4 times/day /10 days. • Procaine penicillin: injection for 10 days. • Erythromycin (50 mg/kg/day) in penicillin sensitive patient for 10 days. 2. Antipyretics for the high fever. A 4-years-old boy is brought by his parents to the pediatric clinic with mild fever and a few small blisters on the shoulder and chest. The parents state that fever was noted for 1 day, and then the skin lesions appeared. These lesions began as tiny papules which progressed rapidly to vesicles. On examination, he has a widespread skin rash at different stages, the rash is more concentrated on the trunk than the limbs. The parents report that that have been several other children with similar lesions at his school. What is your diagnosis? Chickenpox. Mention possible investigations and treatment. INV: CBC: mild leukocytocis Normal ESR and CRP ELISA, Viral culture TTT: 1. Antipruritic agents (local and systemic) 2. Antipyretics. • Aspirin should not be used as it increases the risk of Reye syndrome (acute encephalitis with fatty infiltration of the liver) 59 Mohamed M. Ismail Case scenario Modified by: Abdel Razak Ali Shadeed 3. Antibiotics are indicated only if secondary bacterial infections Occur. 4. Antiviral drugs: Acyclovir I.V in (immunocompromised - encephalitis - pneumonia) A 10-years-old patient calls his parents from summer camp to state that he has had fever, muscular pain (especially in the back), headache and malaise. He describes the area from the back of his mandible towards the mastoid space as being full and tender and that his ear lobe on the affected side appears to be sticking upward ad outwards. Drinking sour liquids causes much pain. When his father calls your office, you remind him that he had refused immunization for his child due to concerns regarding vaccine safety. What is your diagnosis? Mumps Mention possible investigations and treatment. INV: CBC: mild leukocytocis Normal ESR and CRP ELISA, Viral culture TTT: Prophylaxis: 1. Passive: mumps gamma globulins early in the incubation period. 2. Active: MMR vaccine. Treatment: Symptomatic: analgesics and antipyretics Treatment of complications: 1. In orchitis: the testis should be supported with ice bags. 2. In encephalitis: control of convulsion and increased intracranial tens Neonatal disorders A term female infant was born to a 28-years-old mother by CS after an obstructed labor. Apgar scores were 3 and 4 at 1 and 5 minutes, respectively. She required vigorous 60 Mohamed M. Ismail Case scenario Modified by: Abdel Razak Ali Shadeed resuscitation at birth and was admitted to the NICU under observation. She developed generalized tonic clonic seizures 10 hours after delivery. On examination her tern was 36.8 C heart rate was 140/m and respiratory rate 60/m she is no respiratory distress but she is sleepy. Head shoes mild molding. There is generalized hypotonia. Blood sugar and calcium were normal. What is your diagnosis? Hypoxic ischemic encephalopathy. Mention possible investigations and treatment. INV: 1. Prenatal: Fetal biophysical profile - Doppler U/S (assess the cord blood flow). 2. Perinatal: blood gas analysis (acidosis) and saturation (hypoxia). 3. Postnatal: Laboratory: a. Monitoring of ABG - saturation - blood glucose- serum electrolytes temperature - serum calcium. b. Renal function -bleeding profile. c. Liver function tests. Imaging: a. Cranial ultrasound. b. Electroencephalogram (EEG, cerebral function monitor) to detect: abnormal background activity, early encephalopathy or seizures. c. MRI: at 7-10 ± 14 days: bilateral abnormalities in basal ganglia and thalamus. d. Echo (cardiac dysfunction and persistent pulmonary hypertension may occur). TTT: Prevention: Good perinatal care and decision when to do cesarean section. Treatment: 1. Proper stabilization to minimize neuronal damage 2. Treatment of seizures by anticonvulsants: discuss 3. Treatment of hypotension by volume and inotropic support 4. Treatment of hypoglycemia (glucose 10%) and hypocalcemia (Ca gluconate IV) 5. Therapeutic hypothermia (cooling to a rectal temperature of 33-34 C°) for 72 hours 61 Mohamed M. Ismail Case scenario Modified by: Abdel Razak Ali Shadeed Total body cooling and head cooling have shown to be effective in reducing brain damage if started within 6 hours of birth A 23-years-old lady in her first pregnancy is admitted to hospital at 29 weeks gestation because she has had spontaneous rupture of membranes. Forty-eight hours later she goes into spontaneous labor and a baby boy is delivered weighing 900 gm Two hours after delivery. He develops marked subcostal and sternal recession with audible shunting What is your diagnosis? Respiratory distress syndrome. Mention possible investigations and treatment. INV: 1. Chest x-ray: • Diffuse reticulogranular pattern • Air bronchogram (air in the major bronchi appears in contrast with the white background of collapsed alveoli). • Complete opacification of both lung fields (white lungs) in severe conditions. 2. Blood gases hypoxia, hypercapnia and acidosis. 3. Electrolytes, calcium and glucose. 4. CBC, ESR, CRP 62 Mohamed M. Ismail Case scenario Modified by: Abdel Razak Ali Shadeed 1. Basic life support measures: a. Thermoregulation. b. Correction of acidosis, fluid and electrolyte balance. 2. Prophylactic antibiotics until cultures appear (as RDS is clinically indistinguishable from early-onset severe group B streptococcal disease). 3. Monitoring: a. HR, RR, BP and temperature, blood gases, electrolytes, Ca, albumin, glucose b. Oxygen saturation by transcutaneous O2 pulse oximeter, Hemoglobin 4. Correction of hypoxemia a. In the delivery room: prophylactic Nasal continuous positive air way pressure (CPAP) b. NICU management: Nasal CPAP or Intermittent positive pressure ventilation (IPPV) or high frequency ventilation in cases of risk of air leak. 5. Surfactant replacement therapy: A term 4200 gm female infant was delivered by CS because cephalopelvic disproportion. The amniotic fluid was clear, and the infant cried almost immediately after birth. Within the first 15 minutes of life, however the infant's respiratory rate increased to 70 breath/minute, and she began to have mild chest retractions and intermittent grunting respirations. The infant was transferred to neonatal unit. On admission, chest examination showed bilateral fair air entry What is your diagnosis? Transient tachypnea of the newborn. Mention possible investigations and treatment. INV: X ray: a. Prominent pulmonary perihilar markings (dilated lung lymphatics). b. Fluid in the interlobar fissures and small pleural effusion may be seen. 63 Mohamed M. Ismail Case scenario Modified by: Abdel Razak Ali Shadeed 1. Basic life supports and Monitoring. 2. Supplemental oxygen to maintain adequate arterial oxygen saturation. 3. IV fluids, then tube feeding until respiratory rate has decreased enough to allow oral feeding. A 4-day-old full-term infant is referred to the pediatric outpatient clinic by his GP because of jaundice. This is the first baby of a 22-year-old mother His mother states that the jaundice started within the first 24 hours of life, His birth weight was 3.7 kg on Examination the sclera is yellow and the jaundice is extending to the palm and soles. There are no other signs What is your diagnosis? Neonatal jaundice. Mention possible investigations and treatment. INV: Serum bilirubin (total and direct) to determine type of hyperbilirubinemia. In cholestasis conjugated bilirubin is more than 20% of the total: A) Conjugated hyperbilirubinemia: Cholestasis: see hepatology B) Unconjugated hyperbilirubinemia: 1. Blood picture: • HB % and reticulocytosis hemolysis. • WBC count and I/T ratio→ septicemia. 2. To exclude hemolytic disease: • Blood grouping (ABO and Rh) for baby and mother. • Coombs’ test (to detect maternal antibodies that coat the baby's RBCs). 3. To exclude hemolytic anemia: • Enzyme assay: G6PD deficiency. • RBCs morphology and osmotic fragility test: spherocytosis. 4. If septicemia is suspected: CRP - ESR - cultures. 5. Thyroid profile: if not done in neonatal screening program. 64 Mohamed M. Ismail Case scenario Modified by: Abdel Razak Ali Shadeed 1- Monitoring (nomogram): Total serum bilirubin or transcutaneous bilirubin. 2- Phototherapy: Indications: ➢ Bilirubin >15mg/dl in full term neonates ➢ Prophylactic for very low birth weight infants ➢ Before, after, and in between exchange transfusion Technique: ➢ Distance: 45cm ➢ Maximum amount of skin exposed ➢ Cover eye and genitalia Side effects: ➢ Hyperthermia ➢ Loose stool ➢ Dehydration ➢ Damage retina ➢ Erythema, Rash ➢ Defective maternal-infant interaction Contraindications: Cholestasis→ bronze baby syndrome 3- Exchange transfusion Indications: ➢ Bilirubin >25mg/dl despite phototherapy ➢ Hemolytic disease Technique: ➢ Umbilicus venous catheter ➢ Amount: 2 x baby blood volume ➢ Type: O negative or infant’s blood group ➢ Blood should be fresh, irradiated and screened ➢ Alternative withdrawing of 5-10ml of baby’s blood and replacing them with donor blood. Complications: Related to blood: Incompatibility, diseases, hyperkalemia, hypocalcemia Related to catheterization: Infection, Thrombosis, embolism, vasospasm Related to technique: Volume overload, HF 65 Mohamed M. Ismail Case scenario Modified by: Abdel Razak Ali Shadeed 4- Pharmacological: ➢ IVIG reduces need for exchange in isoimmune hemolytic disease. ➢ Phenobarbitone in Crigler Najjar type 2 only: induces glucuronyl transferase A Newborn infant delivered at 34 weeks gestation by vaginal delivery after 96 hours of ruptured membranes. His birth weight was 2 kg. A breast feed was attempted, but was unable to suckle and swallow properly. At the age of 6 hours, he developed frequent apneic episodes. On examination, the baby appears lethargic and hypotonic. His temperature is 35.8 C What is your diagnosis? Neonatal septicemia. Mention possible investigations and treatment. INV: 1. CBC with differential: • WBCs <5,000 cells/mm³ and ANC (Absolute Neutrophilic Count) <1,000 are most predictive of infection • WBCs >20,000 and Immature/Total neutrophils (I/T ratio) >0.3 are suggestive of infection 2. CRP: serial measures 3. Cultures: Blood culture and other cultures (urine culture obtained by catheterization or suprapubic aspiration, endotracheal tube and central line cultures) 4. Others: • Hyperglycemia (because of sympathetic), metabolic acidosis may be present • Thrombocytopenia, PT, PTT, INR in severely ill neonates • Lumber puncture for suspected meningitis: CSF analysis & CSF culture • Chest X ray and arterial blood gas for suspected pneumonia TTT: 1. Antibiotics: • Type: Empirically (first line): Ampicillin + Aminoglycoside (as gentamycin), then according to results of the culture. • Duration: 14 days in proven sepsis, 3 weeks in meningitis and 6 weeks in septic arthritis. 66 Mohamed M. Ismail Case scenario Modified by: Abdel Razak Ali Shadeed 2. Supportive: • Pnemonia: mechanical ventilation • Hypotension: Volume and pressor • Seizures: Anticonvulsants Measures to prevent /minimize infections in the community, nurseries and NICUS: I. Caregiver: Hand washing by care givers: the most important. Proper hygiene of the caregiver. II. Baby: 1. Exclusive breast feeding (especially colostrum). 2. Keep cord dry and uncovered (no powder or antibiotic ointment application). 3. No unnecessary intervention- strict aseptic technique during procedures 4. Proper management of IV lines. III. Unit: 1. Cots and incubators should be well spaced. 2. Disinfection of equipment in general and humidifier in particular. 3. Proper nurse/patients ratio. 67 Mohamed M. Ismail Case scenario Modified by: Abdel Razak Ali Shadeed Emergency A 2-year-old girl had a running nose for 2 days, a cough for 1 day and developed noisy breathing 3 hours ago. Her father had a cold the previous week. On examination, her temp is 38; there is a loudly noisy breathing, mainly on inspiration and a barking cough. Her respiratory rate is 40/m with suprasternal and substernal recessions. On auscultation, there are no cracks or wheeze. What is your diagnosis? Croup (Acute laryngitis: stridor grade 3) Mention possible investigations and treatment. INV: Emergency so should start treatment immediately. However we can do CBC, ESR, CRP, Viral culture, PCR. TTT: Home management: stridor grade 1. 1. Warm steam inhalation relieves minor obstruction. 2. Oral steroids or nebulized steroids 3. Close observation: worsening → hospital management. Hospital management: Indications: 1. Grade II. 2. Suspected bacterial disease. 3. Worsening of home treatment. 4. Grade III or IV (IV need immediate intubation). Treatment plane: 1. Oxygen therapy (mask, head box) follow up by pulse oximetry. 2. Warm steam inhalation: ultrasonic nebulizer. 3. Parenteral steroids to decrease laryngeal edema. 4. Antibiotics in suspected bacterial disease. 5. Close observation: worsening endotracheal intubation. Treatment of acute epiglottitis: ICU: 1. Endotracheal intubations (very skilled personnel). 68 Mohamed M. Ismail Case scenario Modified by: Abdel Razak Ali Shadeed 2. Parenteral antibiotics: cephalosporin. 3. With proper treatment most children recover within 2-3 days. 4. Prophylaxis with rifampicin for close contacts. N.B. Examination of the pharynx or larynx in children with epiglottitis may precipitate complete airway obstruction and it should be avoided until airways are secured Allergic disorders A 12 years old boy is brought to the emergency department after being stung by a bee. He initially complained of localized pain and swelling. Fifteen minutes later, he began to complain of shortness of breath and wheezed. He felt weak and dizzy. On examination, he is drowsy and pale, and in mild respiratory distress, he has generalized urticaria. His lung examination shows mild wheezing with minimal retractions What is your diagnosis? Insect bite hypersensitivity – anaphylaxis Mention possible investigations and treatment. INV: Emergency so should start treatment immediately. However we can CBC: eosinophilia, IgE TTT: Management: 1. ABCS 2. Drugs: a. Epinephrine: 0.01 ml/kg (IM) is the most important b. Antihistaminic: Histamine 1 receptor antagonist (diphenhydramine) orally c. Anti-inflammatory: Corticosteroids: I.V. hydrocortisone. d. Nebulized salbutamol for bronchospasm 3. Treatment of shock will also require a. Trendelenburg position b. 20 ml/kg ringer or saline 69 Mohamed M. Ismail Case scenario Modified by: Abdel Razak Ali Shadeed Acute bronchiolitis 70 Mohamed M. Ismail Case scenario Modified by: Abdel Razak Ali Shadeed Acute Bronchitis 71 Mohamed M. Ismail Case scenario Modified by: Abdel Razak Ali Shadeed Acute brochiolitis Acute hepatitis 72