USC Case # 16 Pediatric Febrile Rash Greg Vigesaa OMSIII Wythe County Community Hospital Preceptors: Albert Aymer, DO Belle Jones, MD Chief Complaint and HPI CC: Fever with a rash HPI: K.C. is a 10 year-old white female who presented to the ER on 3/5/06 with a one day history of fever, vomiting, diarrhea, joint pain, and painful rash. She also complained of intermittent abdominal pain over the past day and a frontal headache. HPI Cont. HPI: K.C. did eat hamburger at a fast food restaurant two days prior to the onset of her symptoms. She has not been hiking or done any foreign travel. She does have multiple animals at home including a ferret, finches, and a cockateil bird. Past Medical/Surgical History PMH: History of multiple urinary tract infections since she was three years old. She has never been hospitalized. PSH: None Allergies and Medications Allergies: penicillin and ciprofloxacin Medications: Urised- medication used as a bladder antispasmodic and antiseptic. It contains atropine, benzoic acid, hyoscyamine, methenamine, methylene blue, and phenyl salicylate. Family/Social History FH: Significant for kidney disease. Multiple family members have either a single kidney or a history of vesicoureteral reflux. SH: C.K. lives at home with her father, mother, 18 year-old brother, and 14 month old sister. Review of Systems General: +fever, +fatigue, no chills, change in weight, appetite. HEENT: +HA, no pharyngitis, rhinorrhea, tinnitus, epistaxis, hearing or visual changes. CV: No CP, palpitations, orthopnea, syncope. Resp: No SOB, dyspnea, cough, hemoptysis. GI: +intermittent abdominal pain, +vomiting, +diarrhea, no melena or hematochezia. GU: No dysuria, hematuria, increased frequency, hesitancy. Review of Systems MS: +arthralgias, no myalgias, weakness, swelling. Neuro: No change in sensation, strength, LOC, dizziness, seizures or parathesias. Skin: +painful rash, no puritis. Psych: No depression, anxiety. Physical Exam Vitals: T 100.9 P 136 BP 94/46 R 20 O2 100% General: overweight, alert, pink, NAD. HEENT: NC/AT, PERRLA, EOMI, TMs clear bilaterally, mucous membranes moist. Neck: supple with full range of motion. Lungs: CTAB. Heart: RRR without murmur, gallop, rub. Physical Exam Abdomen: soft, NT/ND, normoactive BS, no masses, oragnomegally, guarding, rebound or CVA tenderness. Extremities: no C/C/E, brick capillary refill. Skin: petechial, purpuric rash with some maculopapular areas throughout her body, sparing her face. Neruo: CN II-XII grossly intact, normal tone, moving all extremities well. Symmetrical sensation and strength. Negative Kernig and Brudzinski sign. Review of Kernig and Brudzinski Kernig- upon flexion of the thigh to 90 degrees, there will be resistance to extension at the knee if meningeal irritation is present. Brudzinski- involuntary flexion of the thighs and knees upon flexion of the neck when meningeal irritation is present. ER Management Seeing that her blood pressure was only 94/46 and pulse was 136, it was determined that K.C. needed immediate fluid resuscitation to increase her intravascular volume and cardiac output. She received a bolus of NS 20 ml/kg x 2. After the fluids her blood pressure increased to 116/68. STAT labs and blood cultures were obtained. Following labs K.C. received Rocephin 1 gram IV x 1. Consultation After interviewing and examining K.C., Dr. Turski consulted Dr. Belle Jones who was the pediatrician on call. She agreed with the initial assessment and management and agreed to admit her to the hospital. It was decided that a lumber puncture was not indicated do to lack of meningeal signs. Assessment and Plan 10 year-old female with petechial, purpuric rash, fever, gastroenteritis, arthralgias, leukocytosis, mild dehydration, metabolic acidosis. Admit to hospital. CBC with diff., PT/PTT/FDP, BMP, UA/UC, blood cultures, stool cultures, rapid strep (TC), influenza A and B. Rocephin 50 mg/Kg q24h pending BC/UC. Bolus of NS at 20 ml/kg x 2 in ER. D5 1/2 NS IV with 20 mEq of KCL at 100ml/h. NPO. Differential Diagnosis Rocky Mountain Spotted Fever Ehrlichiosis Hemolytic-Uremic Syndrome Toxic Shock Syndrome Henoch-Schonlein Purpura Serum Sickness Meningococcemia Meningitis Coxsackie Virus (hand-foot-and-mouth) Working Through the Differential RMSF is caused by Rickettsia rickettsii and can cause fever, cough, headache, macular and petechial rash. It is transmitted by the ixodid ticks in the Atlantic states mainly from May to September. A history of a tick bite can be elicited in about 70% of patients. K.C. has no history of cough, playing outside, or being bitten by a tick. Being that it is March it is unlikely that she has RMSF. Ehrlichiosis is caused by Ehrlichia species and causes fever, chills, headache, malaise, macular or petechial rash involving the trunk and extremities. It can also cause abdominal pain, vomiting, and diarrhea, DIC. It is an obligate intracellular bacteria that invades lymphocytes and neutrophils. It is also transferred by ticks, sometimes carried on canines. We are unsure if she has contact with doges. This scenario matches the signs and symptoms of K.C.; therefore, we cannot rule this out without further investigation. Working Through the Differential HUS is most commonly caused by Escherichia coli 0157. It is causes fever, petechial/purpuric rash, micoangiopathic hemolytic anemia, thrombocytopenia, diarrhea with bloody stool, and abdominal pain. It is also one of the main causes of acute renal failure in children. E. coli is transmitted through contaminated food such as beef that is undercooked. She does not complain of hematochezia or melena, but the fact that she ate a hamburger at a fast food restaurant raises the suspicion that she may have HUS. TSS is caused by the exotoxin of Staphylococcus aureus it causes fever, vomiting, diarrhea, hypotension, and a skin rash. It occurs most commonly in menstruating women who use tampons. The fact the K.C. has hypotension and other symptoms support the diagnosis. But fact that she has not started menses and dose not uses tampons argues against TSS being the cause of her illness. We can put this farther down on the list of differential diagnosis. Working Through the Differential HSP is an acute or chronic vasculitis that affects small blood vessels of the skin, joints, GI tract, and kidneys. The disease causes a purpuric rash of the extensor surfaces feet, legs, arms which is often preceded by an acute respiratory illness. Patients also often complain of fever, arthralgias, abdominal pain, and edema of the hands and feet. Being that K.C. has no history of previous episodes, URI, or edema of the hands or feet we can put this lower in the differential diagnosis. We cannot; however, rule this out without further workup. Serum sickness is a type III immune reaction which causes antibody-antigen immune complexes to deposit in various tissues. These complexes initiate inflammation by causing complement activation and recruitment of phagocytic cells. The signs and symptoms include fever, arthralgias, skin rash. It is usually preceded by administration of vaccinations, new medications to which the patient develops an allergy to. K.C. has not received and new vaccinations or medications so this can be put lower in the differential diagnosis. Working Through the Differential Meningococcemia and meningitis are caused by the bacteria Neisseria meningitidis. It can cause fever, vomiting, headache, nuchal rigidity, arthralgias, myalgias, petechial or purpuric rash. In meningitis the CNS is involved and may cause confusion, stupor, or coma. A patient with meningitis will often have a positive Kernig or Brudzinski sign. K.C. does not have nuchal rigidity, confusion, or a positive Kernig or Brudzinski sign, this argues against meningitis. But her symptoms are consistent with meningococcemia. Because this is a potentially fatal disease we will put this possible diagnosis at the top of the differential diagnosis and possibly begin empiric antibiotics. Hand-foot-and-mouth disease is caused by the Coxsackievirus A16. It often causes a fever, vesicular rash that affects the oral mucosa, hands, and feet. K.C. has no oral lesions and her rash is not vesicular, but petechial and purpuric. Hand-foot-and-mouth disease is self-limiting and the treatment is symptomatic; therefore, we will put this at the bottom of the differential diagnosis. Labs/Workup CBC w/differential PT/PTT/FDP BMP UA/UC Blood cultures Stool cultures Rapid strep Influenza swab Labs/Workup CBC w/diff: BMP: WBC- 18,000 Na- 134 Hgb- 13.4 K- 3.2 Hct- 38 Cl- 103 Plts- 291,000 CO2- 18 Neut%- 93.4 BUN- 16 Lymp%- 5.0 Cr- 0.8 Bands- 6 Glu- 120 Neut- 80 Ca- 8.4 Lymp- 11 AG-16 Toxic granulations: moderate UA: Color- yellow App- clear WBC- 1-5 RBC- 1-5 Sp. Gravity- 1.030 Mucous- large Bacteria- many Squamous- many Protein- 30 Labs/Workup PT: 13.3 9.4-11.0 PTT: 35.2 24.3-30.2 FDP: 5 <5 Rapid strep- Negative. Influenza- Negative. Labs/Cultures UC: >100,000 col/ml gram- rods (E.coli). TC: Normal flora. SC: Normal flora. BC: Gram- cocci predominately in pairs. Probable Neisseria meningitidis. Sent to state lab for serotyping. Final ID: Neisseria meningitidis serogroup Y. Diagnosis Septic meningococcemia Urinary tract infection Hospital Course Throughout her admission K.C. continued to receive Rocephin 1 g IV q24h and D5 ½ NS with 20 mEq of KCL at 100 ml/h. Routine labs included: CBC with differential and BMP. Her CBC, BMP, and vital signs improved throughout her nine day admission and were normal on the day of her discharge. Hospital Course Blood Pressure Temperature 102 Systolic 130 98 110 90 Diastolic Temp F 100 96 94 Admission Day3 Day5 Day7 70 50 D/C Admission Day5 Day7 D/C Resp and O2 Sat Heart Rate 140 02 sat 110 120 100 Resp Pulse Day3 80 60 Admission Day3 Day5 Day7 D/C 90 70 50 30 10 Admission Day3 Day5 Day7 D/C Labs on 3/10/06 CBC w/diff: WBC- 6.2 Hgb- 11.6 Hct- 32.9 Plts- 261,000 Neut %- 56.1 Lymph %- 32.5 BMP: UA: (3/7/06) Na- 136 K- 3.7 Cl- 105 CO2- 24 BUN- 4 Cr- 0.5 Glu- 98 Ca- 8.8 AG-11 Color- yellow App- clear WBC- none RBC- 5-10 Sp. Gravity- 1.020 Mucous- none Bacteria- none Squamous- few Protein- negative Discharge Instructions K.C. was discharged on 3/13/06, nine days after admission in good condition. She was to follow up with Dr. Belle Jones two days after discharge at Wythe Bland Pediatrics. Prophylactic antibiotics were given to her family and healthcare providers who were in close contact with her. Etiology Neisseria meningitidis. Gram-negative diplococcus. Polysaccharide capsule with lipid A lipooligosaccharide (endotoxin). 13 serogroups- A, B, C, Y, W-135 cause majority of clinical disease. Grows on Thayer-Martin chocolate agar. Ferments glucose and maltose. Epidemiology Causes outbreaks of bacterial meningitis, acute and chronic meningococcemica. 5-10% of the population are nasopharyngeal carriers, higher in daycare centers. Incidence: 0.8-1.3 / 100,000 persons Mortality: 8-13% 50% of cases occur in children < 2yo Risk factors: day care centers, military recruit camps, college freshman living in dorms (2-8 fold increased risk), viral illness, smoking, chromic disease, low socioeconomic status, complement deficiency (c5-c9) Pathogenesis Spread through respiratory droplets. Incubation: 1-10 days. Adheres to non-ciliated epithelial cells via a pili (capsule prevents phagocytosis). Induces host to rearrange microvilli production which causes endocytosis. Traverses the cell in membrane bound vacuoles, possesses porins that assist in escaping complement (c3b, c4b). Pathogenesis Enters the circulation: If abs present- blocks dissemination via complementmediated bacterial lysis. If abs not present- meningococcemia with potential to cause meningitis. Lipid A of the LOS activates inflammatory cytokines TNF-a, IL-1, IL-6, IL-8, also the intrinsic and extrinsic coagulation cascade. The amount of inflammatory response is directly proportional to the concentration of lipid A in the circulation. The inflammatory response can lead to progressive capillary leakage and DIC leading to multi-organ system failure, septic shock, and circulatory collapse. Pathogenesis Pathogenesis Clinical Manifestations Fever Pharyngitis Headache Nausea and vomiting Myalgias Arthralgias Altered mental status (stupor) Nuchal rigidity Petechial or purpuric rash Petechial/Purpuric Rash Purpuric Rash/Necrosis Diagnosis Isolation of N. meningitidis from a sterile body fluid (Blood cultures, CSF, synovial or pleural fluid). Culture and gram stain of petechial or purpuric scrapings. Latex agglutination test of CSF. PCR used in the United Kingdom. Gram-negative Diplococcus Complications Waterhouse-Friderichsen Syndrome. Gangrene Endocarditis, myocarditis, pericarditis. Renal infarcts. Avascular necrosis of epiphyseal plates. Waterhouse-Friderichsen Syndrome Bilateral adrenal hemorrhagic necrosis. Caused by DIC- adrenal glands become “sacs of clotted blood”. Decreased adrenocortical steroids (aldosterone and cortisol) leads to hyponatremia and hyperkalemia. Tx the underlying cause with antibiotics, may require supplemental corticosteriods. Waterhouse-Friderichsen Syndrome Hemorrhagic necrosis of adrenal gland. Loss of normal architecture: – Fasiculata – Glomerulosa – Reticularis Treatment Neisseria Meningitidis is very sensitive to antibiotics. Penicillin G 250,000 U/kg/d IV x 5-7 d. Cefotaxime 200 mg/kg/d IV x 5-7 d. Ceftriaxone 100 mg/kg/d IV x 5-7 d. Isolation (droplet precautions) for 24 hrs after initiation of antibiotics. Immunization There is a quadrivalent meningococcal vaccine against serogroups A, C, Y, and W-135. It is available to patients older than two years of age. Routine immunization is not recommended because the infection rate in the general population is low. However, immunization is recommended for persons in high-risk groups with risk factors. Prophylaxis for Contacts Ciprofloxacin 500 mg PO, single dose if >18 years old. Ceftriaxone 250 mg IM, single dose if >12 years old. Ceftriaxone 125 mg IM, single dose if <12 years old. Osteopathic Considerations Very little literature about OMM and sepsis. OMM seems contraindicated in a child with septic meningiococcemia. Would not want to facilitate further dissemination of the bacteria. Also given the potential for DIC, manipulation could cause hemorrhage because of decreased platelets and clotting factors. Osteopathic principles apply to all patients: – – – – Body is a unit. Body has self-regulating mechanisms. Structure and function are reciprocally interrelated. Rational treatment is based on these principles. References Behrman, Kliegman, Jensen. Nelson’s Textbook of Pediatrics. 17th ed. Pgs. 896-899. 2004. Saunders. Cohen & Powderly: Infectious Diseases. 2nd ed. Pgs. 2173-2187. 2004. Mosby. Long. Principles and Practice of Pediatric Infectious Diseases. 2nd ed. Pgs 748-756. 2003. Churchill Livingstone. Kumar, Abbas, Fausto. Robbins and Cotran Pathologic Basis of Disease. 7th ed. Pgs. 377-378, 1214-1215. 2005. Saunders. The Red Book Report on the Committee of Infectious Disease. American Academy of Pediatrics. Pgs. 430-436.