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Celiac Disease 









Gluten-induced Enteropathy

Is a mal-absorption disease of the proximal small intestine , that is characterized by abnormal mucosa with permanent intolerance to gluten .

Etiology :

1. unknown .
2. genetics .
3. toxic .
4. allergic .

Altered physiology :


Characteristics of the disease include :

- Impaired intestinal absorption .

- Histological abnormalities of small intestine .

- Clinical and histological improvement with gluten-free diet .

- Recurrence of clinical manifestations and histological changes after reintroduction of dietary gluten .

Histological changes :

- Loss of normal villous pattern .

- Obliteration of intervillous spaces .

- Loss of epithelial cell brush border .

These changes result in disaccharide deficiency , inability to absorb fates , fat-soluble vitamins , minerals and some proteins and carbohydrates .

Mal-absorption result from decreased area of absorption .

Clinical manifestations :

- Diagnoses is most commonly made at 6-24 months of age ( it can be made in adult .

- Chronic or recurrent diarrhea ( foul . bulky , and greasy stool ) .

- Anorexia , vomiting , abdominal distention , muscle wasting and hypotonia .

- Steatorrhea .

- Malnutrition .

- Weight loss and dehydration .

- Mood changes ( irritability and nervousness ) .

- Secondary deficiencies such as anemia , hypocalcaemia and hypoproteinemia .

Diagnostic evaluations :

- History and general status .

- Small bowel biopsy .

- D-xylose test ( less than 20-25mg/dl at 60 minute ) .

- Hb level .

- Stool test ( fecal fat excretion ).

- Sweet test ( to rule out cystic fibrosis ) .

- X-ray .

Treatment :

- Lifelong gluten free diet .

- Adequate caloric intake .

- Supplemental vitamins .

- Decreased fat intake .

Treatment of celiac crisis :-

1. Restore fluid and electrolyte balance .

2. Parenteral hyperalimination .

3. Steroids .






Hirschsprung's Disease ( Congenital A ganglionic Megecolon )







Is congenital anomaly that results in mechanical obstruction from inadequate motility of part of the intestine .

Etiology :

- Arrest in embryological development affecting the migration of parasympathetic nerves of the intestine ( prior to the 12th week of gestation ) .

- Unknown cause .

- May be familial .

Clinical manifestations : { vary depending on degree of involved bowel } .

- Appearing at birth or within first weeks of life .

- Failure to pass meconium within 24- 48 hours after birth .

- Vomiting ( bile-stained or fecal ) .

- Abdominal distention .

- Reluctance to ingest fluids .

- Overflow – type diarrhea .

Older children :

- History may reveal obstipation at birth .

- Abdominal distention .

- Constipation ( relieved temporary with enema ) .

- Ribbon like , foul - smelling stool .

- Inadequate weight again .

- Fecal masses may be palpable .


Complications :


- Prior to surgery : Enterocolitis ( major cause of death ) , water intoxication ( enemas ) , perforation .

- Post-op : Enterocolitis , leak of anastomosis , pelvic abscess .

- Colostomy : hemorrhage , shock , and infection .

- Diagnostic evaluations :

- Rectal examination : no fecal material .

- Barium enema : narrow intestine proximal to anus , dilated intestine proximal to narrow segment .

- Rectal biopsy : absence or reduced number of ganglion nerve cells .

Treatment :

- Initially : colostomy or ileostomy to decompress the intestine rest normal bowel .

- Definitive surgery : abdominal pull- through , at 9-12 months or 7-10 kg body wt. .






Measles and German Measles



Measles ( Rubeola )



Agent : rubeola measles virus .

Source : respiratory secretions, blood and urine of infected person .

Transmission : by direct contact with infected person .

Passive immunity : birth to 1 year of age if mother is immune prior to pregnancy .

Incubation period : 5-15 days .

Common age : school age in spring .

Period of communicability : 4 days before to 5 days after rash appear .

Clinical manifestations :

- Catarrhal stage : fever, malaise, cough, conjunctivitis, koplik's spots .

- Rash : Appear 2-4 days after onset of catarrhal stages begins in face and gradually spread downward .

Treatment :

- Vitamin A supplement .

- Bed rest .

- Antipyretics .

- Antibiotics to prevent secondary bacterial infections .

- Calamine lotion to minimize itching .


Rubella ( German measles )


Agent : rubella virus .

Source : respiratory secretions, blood and urine of infected person .

Transmission : by direct and indirect contact with infected person .

Incubation period : 14-21 days .

Period of communicability : 7 days before to 5 days after rashes appear .

Clinical manifestations :

- catarrhal stage : headache , anorexia , low grade fever , sore throat , conjunctivitis and malaise for 1-5 days and subside1 day after appearance of rashes .

- rash : firstly in face then down ward to neck , arms , trunk and legs and disappear the same way by the 2nd or 3rd day .

Treatment :


no treatment necessary other than antipyretics and analgesics .

complications : greatest danger is teratogenic effect on fetus ( cataract , CHD , and deafness )






Esophageal Atresia with Trachoesophageal Fistula







Is malformation caused by failure of the esophagus to develop a continues passage , the esophagus may or not form a connection with the trachea .

Altered physiology :

Failure of septal development between trachea and esophagus , during the 4th week of gestation .

Types :

1- Type (A) : 8% blind at each end of the esophagus , widely separated with connection to the trachea.

2- Type (B) : rare , proximal segment of esophagus opens into trachea , distal end is blind .

3- Type (C) : 80-90% , proximal esophagus segment terminates in a blind pouch , and the distal segment is connected to the trachea or primary bronchus by a short fistula at or near the bifurcation .

4- Type (D) : rare , both upper and lower esophagus segment connected to the trachea .

5- Type (E) : 5% , fistula without atresia .

- Child is unable to swallow effectively .

- Saliva or formula accumulate in upper esophagus pouch and is aspirated .

- Reguratiation of gastric acid.

- Abdominal distention may lead to respiratory distress.

Clinical manifestation :

- Appear soon after birth , excessive amount of secretions , constant drooling , intermittent cyanosis , abdominal distention , choking fluids retune through nose and mouth through feeding process.

- Inability to pass catheter through nose or mouth.

Diagnostic evaluation :

- Recognize infants risk " premature + polyhdramnios ".

- Observe symptoms.

- Inability to pass 10 -12 stiff catheter.

- X-ray (chest and abdomen ) gas in stomach , tip of the catheter in blind pouch.

Complications and associated problems :

- pneumonitis ( salivary or gastric acid reflex ) , congenital heart disease , imperforated anus , prematurity.

Treatment :

- Propping infant at 30-degree angle to prevent reflux of gastric content.

- Suctioning.

- Supportive I.V fluids , antibiotic , respiratory support.

- Surgery.






Acute Rheumatic Fever






Is a systemic disease characterized by inflammatory lesions of connective and endothelial tissue :

Etiology :

- The exact pathogens are unknown.

- It is thought to be an autoimmune response to group a beta hemolytic streptococci.

- Most first attacks of rheumatic fever preceded by streptococcal infection of the throat or upper respiratory tract at an interval of several days to several weeks.

Altered physiology :

- The unique pathologic lesion of rheumatic fever is the Aschoff body.

- The basic changes consist of exudative and proliferative inflammatory reactions in the mesenchymal supporting tissues of the heart, joints blood vessels and subcutaneous tissue.

- The inflammatory process involves all layers of the heart.

- The inflammatory may involve the heart valves, most frequent the mitral and/or the aortic valves.

Clinical manifestations :

- The diagnosis is based on a combination of manifestations of the disease.

- The presence of 2 major criteria or 1 major and 2 minor criteria, plus evidence of preceding streptococcal infection are required to establish the diagnosis.

Major manifestations :

1- Carditis: significant murmurs, signs of pericarditis, cardiomegaly or congestive heart failure.

2- Polyarthritis: the affected joints are swollen, tender and red migratory, the large joints are affected.

3- Subcutaneous nodules :
- Firm, painless bodies seen or felt over the extensor surface of certain joints, particularly the elbow, knee and wrists.

- Disappear mostly after 4 months.

- Presence of nodules is an indicator that the heart is involved.

4- Erythema marginatum :
Circinate or annular rash occur on the arms, trunk and legs { never on the face}. evanescent, pink rash, have pale centers and round or wavy margins.

5- Chorea : Purposeless involuntary movement often associated with muscle weakness, incoordination of voluntary movement and emotional instability.

Minor manifestations :

1- Fever.

2- Arthralgia.

3- Prolonged P-R interval in the ECG.

4- Increased E.S.R, leukocytosis, positive C-reactive protein.

5- A history of streptococcal infection, scarlet fever, previous history of rheumatic fever.

Treatment :

1- Bed rest for 2 weeks then gradual ambulation.

2- Cases without cardiac involvement, aspirin only 100 mg/kg/day in 4 hours divided doses . until E.S.R. is normal for 2 weeks.

3- With cardiac involvement aspirin and prednisone.

4- Prevention of rheumatic fever through control of streptococcal infection: procaine penicillin G 1.200.000 units once a month.




Thalassemia major " Cooley's anaemia "





- Beta-thalassemia refers to inherit the group of blood disorders characterized by a reduction or absence of the beta chain of globulin in haemoglobin syntheses.

- Haemoglobin b-thalassemia is the most severe of the b-thalassemia syntheses and is known as thalassemia major or cooleys anaemia.

Etiology :

- Genetically determined inherited disease.

- The autosomal recessive pattern of inheritance.

Incidence :

- Most prevalent in the Mediterranean, Middle East, India, Pakistan, Southeast, Asia and Africa.

Prognosis :

- No known cure.

- often fatal in late childhood or early adolescent.

Preventive measures :

- Parents of a child with thalassemia should be tested for the trait and referred for genetic counselling.

- Prenatal diagnosis terminates pregnancy .

Clinical manifestations :

- Insidious onset at the end of the 1st year of the life.

- Pallor, poor feeding, protuberant abdomen due to hepatosplenomegaly, headache, bone pain, exercise intolerance and easy fatigability and growth retardation.

Complications :

1. Splenomegaly and hepatomegaly due to extramedullary hemopoiesis and rapid destruction of RBCs. coagulation abnormalities.
2. Growth and endocrine complications :
- Growth retardation.
- Diabetes in older children due to iron deposition in the pancreas.
- Adrenal and pituitary dysfunction. 
3. skeletal complication :
- Maxillary hypertrophy. 
- Broad ribs. 
- Skeletal osteoporosis. 
4. Cardiac complication : 
- Arrhythmia, pericarditis, congestive heart failure " the usual cause of death of thalassemics ".
5. Skin :
- Brownish discolouration due to iron deposition. 
- Jaundice.

Treatment :

- Frequent and regular blood transfusion to maintain haemoglobin level above 9-10 gm/ dl.

- Packed RBCs usually used.

- Iron chelating agent to reduce the toxic side effects of excess iron " hemosiderosis ".

- E.g. deferoxamine: desferal.

- Folic acid.

- Splenectomy in huge hypersplenism.

- Bone marrow transplantation.