‫‪Celiac disease‬‬
‫أ‪.‬عبد المطلب عيد الكحلوت ‪/‬ماجستير تمريض األطفال‬
 Definition :it is a disease of small intestines
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characterized by permanent inability to tolerate
dietary gluten ,which present wheat.
Etiology :mainly unknown .
Genetic may due to autosomal recessive but uncertain.
Familial .
Environmental presence of allergies but unclear.
Current theory may be due to :autoimmune .
Deficient enzymes digest gluten.
Altered epithelial cells surface .
‫ماجستير تمريض األطفال‬/ ‫عبد المطلب عيد الكحلوت‬.‫أ‬
Altered physiology
 Impaired intestinal absorption :due to mucosal damage
result in :
 Disaccharide deficiency impaired CHO absorption
and digestion.
 Depression in peptidase activity impaired protein and
fat digestion and absorption.
 Impaired other enzymes activity and decrease area of
absorption of small intestine.
‫ماجستير تمريض األطفال‬/ ‫عبد المطلب عيد الكحلوت‬.‫أ‬
 Histological abnormalities of small intestine (duodenum
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and jejunum):
Irregular of epithelial cells .
Loss of normal villous pattern .
Obliteration of intervillous spaces which are infiltrated
with plasma cells and eosinophils.
Loss of epithelial cells brush border.
Clinical and histological improvement with wheat ,rye
,barley ,and oat free diet.
Clinical and histological recurrence of manifestation after
reintroduction of dietary gluten.
‫ماجستير تمريض األطفال‬/ ‫عبد المطلب عيد الكحلوت‬.‫أ‬
Clinical manifestation
 Most common present between 6-24 months of age.
 Most typical present between 9-18 months of age.
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Chronic ,severe and recurrent diarrhea (pale ,soft
,bulky ,having offensive odor ,greasy due to
steatorrhea.
Vomiting ,anorexia abdominal distension .
FTT ,muscle wasting (obvious in buttocks and
proximal parts of extremities ).
Hypotonia :mood changes ill humor ,irritability
,shyness.
Mild clubbing
of fingers.
‫ماجستير تمريض األطفال‬/ ‫عبد المطلب عيد الكحلوت‬.‫أ‬
 6-secondary manifestation :
 Anemia ,vit deficiency sore mouth and smooth
tongue.
 Edema due to hypoprotinemia .
 hypocalcemia ,hypo prothrombinemia due to vitamin
K mal absorption .
 7-progressive malnutrition.
‫ماجستير تمريض األطفال‬/ ‫عبد المطلب عيد الكحلوت‬.‫أ‬
Celiac crisis
Occur in very young child and toddler :
1. Profound anorexia.
2. Severe vomiting and diarrhea.
3. Weight loss.
4. Marked dehydration and acidosis .
5. Immobility .
6. Abdominal distention (severe) fluid rattles is
present ,abdominal flattens with passage of large
liquid stool ,shock like state.
7. Profound depression.
‫ماجستير تمريض األطفال‬/ ‫عبد المطلب عيد الكحلوت‬.‫أ‬
Diagnostic evaluation
 Small bowel biopsy most diagnostic severe damage or flat villous
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lesion with lymphocytes.
D –xylose absorption :normal after one hour 25-100% absorbed
less than 20-25 mg/dl has a disease.
RBC hypochromic and level of iron and folic acid decrease, Hb
also.
Increase level of IGA in acute stage.
Determine fat absorption .
Prothrombin time (before biopsy ) .
Skeletal X ray shows retarded bone age and demineralization .
Low glucose tolerance .
Sweat test and pancreatic function studies to rule out cystic
fibrosis.
‫ماجستير تمريض األطفال‬/ ‫عبد المطلب عيد الكحلوت‬.‫أ‬
Treatment
 Life long (gliadin) gluten free diet include: barley
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,oats wheat and rye’’. a wheat like cereal plant that
tolerates poor soils and low temperatures’’.
Sign of improvement should be seen 1-4 weeks after
proper diet is initiated.
Adequate caloric intake .
Minerals and vitamins supplement.
Iron and folic acid .
‫ماجستير تمريض األطفال‬/ ‫عبد المطلب عيد الكحلوت‬.‫أ‬
 Treatment of celiac crisis :
 I V .line for fluid and electrolyte replacement and keep
patient NPO.
 Steroid .
 Parenteral hyper alimentation with amino acid
,medium chained triglyceride ,and glucose for short
period (TPN).
 Initial oral feeding may need to be disaccharide or
completely sugar free.
‫ماجستير تمريض األطفال‬/ ‫عبد المطلب عيد الكحلوت‬.‫أ‬
 Complications of celiac disease :
 Lymphoma (most serous complication of small
infection).
‫ماجستير تمريض األطفال‬/ ‫عبد المطلب عيد الكحلوت‬.‫أ‬
‫أ‪.‬عبد المطلب عيد الكحلوت ‪/‬ماجستير تمريض األطفال‬
‫‪Pyloric stenosis‬‬
‫أ‪.‬عبد المطلب عيد الكحلوت ‪/‬ماجستير تمريض األطفال‬
‫أ‪.‬عبد المطلب عيد الكحلوت ‪/‬ماجستير تمريض األطفال‬
‫أ‪.‬عبد المطلب عيد الكحلوت ‪/‬ماجستير تمريض األطفال‬
Pyloric
 Definition: congenital progressive hypertrophic of pyloric
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muscle cause partial or total obstruction stomach outlet
(pyloric sphincter) .
Etiology :
1. mainly unknown .
2. may be due to immature pyloric ganglion cells –
environmentally ,partial genetic basis.
Incidence :
1. 1:800 in white ,1:2000 in black.
2. 80% in male infant and more at first one .
3. 4:1 ratio predominant with males .
‫ماجستير تمريض األطفال‬/ ‫عبد المطلب عيد الكحلوت‬.‫أ‬
Altered physiology :
 increase in size of circular musculature ( hypertrophy
& hyperplasia of it) size and shape of an olive .
 this cause narrowing of pyloric lumen this cause
stomach dilation.
 Gastric emptying is delayed ,vomiting after feeding
and obstruction occur.
 Gastritis and bleeding may occur .
‫ماجستير تمريض األطفال‬/ ‫عبد المطلب عيد الكحلوت‬.‫أ‬
 Clinical manifestation :
 Onset of it 3 weeks -2 months of age.
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vomiting may be gradual or sudden ,forceful or
sudden : firstly not projectile vomiting then become
projectile and bile stained.
Constipation ,weight loss , failure to gain weight.
Visible gastric peristaltic waves left to right.
‫ماجستير تمريض األطفال‬/ ‫عبد المطلب عيد الكحلوت‬.‫أ‬
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Excessive hunger after vomiting .
Dehydration ,electrolyte disturbance with alkalosis.
Decrease UOP ,palpable pyloric mass in upper right
quadrant ,to the right of umbilicus best felt during
feeding or immediate after vomiting ,mobile non
tender hard like an olive.
‫ماجستير تمريض األطفال‬/ ‫عبد المطلب عيد الكحلوت‬.‫أ‬
 Diagnostic evaluation :
1. Metabolic alkalosis due to loss of HCL acid and K from
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vomiting decrease serum Na,K,rise Ph and co2.
urinalysis :alkaline and concentrated.
Increase HCT and Hb due to hemoconcentration.
Flat film of abdomen shows dilated ,air filled stomach ,non
dilated pyloric canal .
Barium meal :
Narrowing pyloric canal .
Delayed gastric emptying .
Enlarged stomach and increase peristaltic waves.
Gas distal to stomach.
Umbrella shaped duodenal cap.
‫ماجستير تمريض األطفال‬/ ‫عبد المطلب عيد الكحلوت‬.‫أ‬
‫أ‪.‬عبد المطلب عيد الكحلوت ‪/‬ماجستير تمريض األطفال‬
 Treatment :
 Rehydration and correct electrolyte ,and acid base.
 Surgical :pyloromyotomy (Fredet –Ramastedet) where
the surgeon cuts through the muscle fibers of enlarged
pyloric muscle in order to widen the opening into the
intestine.
 Hypertrophy of pyloric muscle regress to normal size
about 12 weeks post operative.
‫ماجستير تمريض األطفال‬/ ‫عبد المطلب عيد الكحلوت‬.‫أ‬
‫‪Cleft lip and cleft palate‬‬
‫أ‪.‬عبد المطلب عيد الكحلوت ‪/‬ماجستير تمريض األطفال‬
‫أ‪.‬عبد المطلب عيد الكحلوت ‪/‬ماجستير تمريض األطفال‬
‫أ‪.‬عبد المطلب عيد الكحلوت ‪/‬ماجستير تمريض األطفال‬
 Definition : mal formation result when fusion
involving the first brachial arch fails to take place
during embryonic development.
 Etiology :
1. Unknown –failure of embryonic development.
2. Hereditary factor.
3. May be related mutant gene , chromosomal
abnormalities ,teratogenes.
‫ماجستير تمريض األطفال‬/ ‫عبد المطلب عيد الكحلوت‬.‫أ‬
 Associated problem :
 Pierre Robin syndrome “cleft palate ,glossoptosis
,micrognathia.
Glossoptosis :tongue falls back to pharynx.
Micrognathia :underdeveloped mandible.
Intellectual deficit.
‫ماجستير تمريض األطفال‬/ ‫عبد المطلب عيد الكحلوت‬.‫أ‬
‫‪micrognathia‬‬
‫أ‪.‬عبد المطلب عيد الكحلوت ‪/‬ماجستير تمريض األطفال‬
 Long term complication :
 Speech impairments .
 Improper tooth placement.
 Recurrent Otitis media.
 hearing impairment.
 Faulty socially adjustment related to poor self concept
and abnormal speech.
‫ماجستير تمريض األطفال‬/ ‫عبد المطلب عيد الكحلوت‬.‫أ‬
 Treatment :
 The main goals of treatment are:
 Closure of clefts (cheiloplasty for cleft lip and repair for
cleft palate)
 Prevention of complication .
 Facilitation of normal growth and development of the
child.
‫ماجستير تمريض األطفال‬/ ‫عبد المطلب عيد الكحلوت‬.‫أ‬
Post operative care
 good post operative care and observe complication:
 Cleft lip
1. Elbow restraints are the most effective way to prevent
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hands from reaching the lip.
Special type adhesive placed from check to other to
prevent tension on suture.
Minimize crying to decrease tension on suture .
Position patient supine or sideling to keep him from
rubbing his lip on sheet.
Position seat may be useful for change position.
‫ماجستير تمريض األطفال‬/ ‫عبد المطلب عيد الكحلوت‬.‫أ‬
 Cleft palate:
Avoid use of straw ,eating utensils and fingers ,tongue
depressor small spoon and suction catheter.
2. Prevent crying ,blowing ,sucking ,talking and laughing to
prevent suture stress.
3. Note respiratory effort.
4. If palate (alone)put infant on prone position.
5. Croup tent with mist decrease occurrence of respiratory
problem and provide moisture to mucus membrane
which may become dry due to mouth breathing.
1.
‫ماجستير تمريض األطفال‬/ ‫عبد المطلب عيد الكحلوت‬.‫أ‬
 Keep suture line clean to decrease infection and eliminate
crust formation which enlarge the resulting scar:
1. Suture of cleft removed 3-14 days after surgery.
2. Irrigate and clean suture line by saline or water ,hydrogen
peroxide .
 Gently irrigate the palate with bulb syringe ,in cleft lip
gently wipe with wet cotton tipped applicator.
 Use antibiotic ointment or petroleum after drying .
 May be left open to air.
 In case of palate : keep mouth moist to promote healing
and provide comfort ,rinse mouth after feeding have the
child sitting position with his head forward.
‫ماجستير تمريض األطفال‬/ ‫عبد المطلب عيد الكحلوت‬.‫أ‬
 Maintain adequate nutrition and fluid intake for wt, gain
and dehydration prevention.
1- For cleft lip:
for several days post op ,feeding will have to be accomplished
without tension on suture line :
• Dropper or syringe with rubber tip inserted from side of
mouth.
• Side of spoon (never put spoon into mouth).
• NGT usually last treatment of choice.
• Advance slowly to nipple feeding as indicated by surgeon
‫ماجستير تمريض األطفال‬/ ‫عبد المطلب عيد الكحلوت‬.‫أ‬
 2- for cleft palate :
• Diet progress from clear liquid to full liquid to soft
food(soft food for one month post operative ).
• Check weight periodically .
• Feed the child in the manner used preoperative (cup
,side of spoon ,or rubber tipped syringe )never use
straw nipple or plain syringe.
‫ماجستير تمريض األطفال‬/ ‫عبد المطلب عيد الكحلوت‬.‫أ‬