M. Gorky Donetsk National Medical University Department No. 2 of Pediatrics Head of the Department Dr. Churilina A. V., Ph. D. Malabsorption syndromes in children icon

M. Gorky Donetsk National Medical University Department No. 2 of Pediatrics Head of the Department Dr. Churilina A. V., Ph. D. Malabsorption syndromes in children




НазваM. Gorky Donetsk National Medical University Department No. 2 of Pediatrics Head of the Department Dr. Churilina A. V., Ph. D. Malabsorption syndromes in children
Дата19.09.2012
Розмір445 b.
ТипДокументи


M.Gorky Donetsk National Medical University Department No. 2 of Pediatrics Head of the Department Dr. Churilina A.V., Ph.D. Malabsorption syndromes in children

  • Associated professor Masyuta D.I.


Gluten-Sensitive Enteropathy (Celiac Disease)

  • Gluten-sensitive enteropathy is a disorder in which small-bowel mucosal damage is the result of a permanent sensitivity to dietary gluten.

  • The disorder does not present until gluten products have been introduced into the diet.

  • Typically, the most common period of presentation is between 6 mo and 2 yr of age.



PATHOGENESIS

  • Three components interact in the pathogenesis:

    • toxicity of certain cereals,
    • genetic predisposition, and
    • environmental factors.
  • The disorder develops only after chronic dietary exposure to the protein gluten, which is found in wheat, rye, oats, and barley.

  • The activity of gluten resides in the gliadin fraction, which contains certain repetitious amino acid sequences (motifs) that lead to sensitization of lamina propria lymphocytes.



PATHOGENESIS

  • The evidence that there is a genetic predisposition is that

    • (1) up to 2–5% of first-degree relatives have symptomatic gluten-sensitive enteropathy,
    • (2) as many as 10% of first-degree relatives have asymptomatic damage to small-bowel mucosa consistent with this disorder, and
    • (3) there is an association of the disorder with certain human leukocyte antigen (HLA) types (B8, DR7, DR3, and DQw2).


PATHOGENESIS

  • Environmental factors must influence the expression of this genetic predisposition because

    • (1) there is a 30% rate of discordance in monozygotic twins,
    • (2) there is a 70% rate of discordance in HLA-identical siblings,
    • (3) the age of onset among siblings is variable, and
    • (4) the onset of symptoms can be precipitated by gastrointestinal surgery, pregnancy, antibiotic use, or a coincidental diarrheal illness.


PATHOGENESIS

  • The immunologic response to gluten results in

    • villus atrophy,
    • crypt hyperplasia, and
    • damage to the surface epithelium in the small bowel.






PATHOGENESIS

  • The injury is greatest in the proximal small bowel and extends distally for a variable distance. The latter observation is undoubtedly the explanation for the variable degree of symptoms and findings of malabsorption among individuals with gluten-sensitive enteropathy.

  • A decrease in absorptive and digestive capacity results from a decrease in small intestinal surface area and a relative increase in immature epithelial cells.

  • Pancreatic secretion is decreased as a result of lowered serum cholecystokinin and secretin levels.



^ CLINICAL MANIFESTATIONS

  • The mode of presentation is variable; the majority present with diarrhea.

  • Children can have failure to thrive or vomiting as the only manifestation.

  • Perhaps as many as 10% of children referred to endocrinologists for growth retardation without an endocrine or overt gastrointestinal disorder have gluten sensitivity.

  • Anorexia is common and may be the major cause of weight loss or lack of weight gain.



^ CLINICAL MANIFESTATIONS

  • Infants with gluten-sensitive enteropathy are often, but not always, clingy, irritable, unhappy children who are difficult to comfort.

  • In contrast to infants with cystic fibrosis, they are not interested in food, although this is not always the case.

  • Pallor and abdominal distention are common.

  • Large, bulky stools have been described in some children with this condition.

  • Digital clubbing can occur.









Celiac Disease (a 4-year-old child)



Celiac Disease (a 6-year-old child)



Celiac Disease (a 23-year-old man after treatment)







EVALUATION

  • Screening tests for malabsorption are not particularly helpful because they may be normal in a child with gluten-sensitive enteropathy.

  • Anemia and hypoproteinemia may be present.

  • The first serologic tests, including antigliadin antibodies, were not reliable enough.

  • However, the sensitivity and specificity of serum IgA-endomysial antibody testing have approached 100% (except in IgA-deficient patients).



EVALUATION

  • Histologic findings on small-bowel biopsy remain the gold standard for diagnosis and biopsy should be performed if one has a high suspicion of gluten-sensitive enteropathy or if serum endomysial antibody is found.

  • The strictest approach to diagnosis is to demonstrate that the biopsy returns to normal within 1–2 yr after starting a gluten-free diet and then to rechallenge with a gluten diet and repeat the biopsy.

  • This approach is now in evolution because it is possible to demonstrate antibody conversion while on a gluten-free diet and only an initial small-bowel biopsy may be necessary.



TREATMENT

  • Treatment requires a lifelong, strict gluten-free diet.

  • All wheat, rye, and barley products should be eliminated from the diet; many children tolerate oats.

  • Initially, vitamin and iron supplementation is advisable.

  • When the disorder presents with fulminant diarrhea, initial treatment with oral prednisone can be useful; this approach is rarely necessary.



TREATMENT

  • Although the parents of a child with gluten-sensitive enteropathy usually become very knowledgable about diet, initially they need the help of an experienced dietitian.

  • National celiac support groups provide much specific information about the gluten content of foods and medications.

  • Processed foods must be considered carefully because it is common that they contain some gluten.

  • Gluten-free foods are commercially available.



PROGNOSIS

  • The clinical response to a gluten-free diet of a child with celiac disease is gratifying.

  • Improvement of mood and appetite is followed by lessening of diarrhea.

  • In most cases changes occur within 1 wk of starting therapy, but the response may occasionally be delayed.

  • Older patients and very ill patients tend to respond slowly, but once in remission the celiac child should be treated as a well child.





PROGNOSIS

  • Subtle manifestations of growth failure or delayed sexual maturation may take place when receiving a gluten-containing diet.

  • Appropriately diagnosed gluten-sensitive enteropathy is a lifelong condition requiring lifelong treatment.

  • No complications from long-term gluten-free diet treatment are recognized.



Coeliac disease:

  • is a gluten-sensitive enteropathy

  • classical presentation is at 8-24 months with abnormal stools, failure to thrive, abdominal distension and wasted buttocks, and irritability

  • other modes of presentation - short stature, anaemia, screening, e.g. children with diabetes mellitus

  • diagnosis - positive serology (tissue transglutaminase and anti-endomysial antibodies), flat mucosa on jejunal biopsy and resolution of symptoms and catch-up growth upon gluten withdrawal

  • treatment - gluten-free diet for life.



Intestinal Lymphangiectasia

  • This group of disorders is characterized by dilatation of intestinal lymphatic vessels and leakage of lymph into the intestinal lumen and, at times, the peritoneal cavity.



Causes Of Intestinal Lymfangiectasia

  • Intestinal lymphangiectasia can be

    • primary or
    • can result from
      • abdominal or thoracic surgical damage to lymphatic vessels,
      • chronic right-sided heart failure,
      • constrictive pericarditis,
      • retroperitoneal tumor, or
      • malrotation with lymphatic obstruction.


Causes Of Intestinal Lymfangiectasia

  • Primary intestinal lymphangiectasia is

    • the result of a congenital abnormality of lymphatic drainage from the intestine and
    • may be associated with abnormalities in lymphatic drainage from other regions of the body.
  • Turner and Noonan syndromes have been associated with intestinal lymphangiectasia.



PATHOGENESIS

  • Because absorbed fat is normally transferred from the intestine via the lymphatic vessels, children with this disorder have steatorrhea with protein-losing enteropathy and may have lymphocyte depletion.



^ CLINICAL MANIFESTATIONS

  • Manifestations may include any combination of

    • hypoalbuminemia,
    • hypogammaglobulinemia,
    • edema,
    • lymphocytopenia,
    • fat malabsorption, and
    • chylous ascites.


Diagnosis

  • The diagnosis is suggested by the typical findings described previously in association with an elevated fecal alpha1-antitrypsin level consistent with protein-losing enteropathy.

  • The characteristic radiologic findings of uniform, symmetric thickening of mucosal folds throughout the small intestine are usually, although not always, present on small-bowel contrast radiographs.



Diagnosis

  • The diagnosis is confirmed by the presence of collections of abnormal dilated lacteals with distortion of villi on peroral small-bowel biopsy.

  • The disorder may be seen only in the submucosa, requiring surgical biopsy of the intestine.



Lactose Intolerance

  • Lactose intolerance is the development of clinical symptoms resulting from lactase deficiency following ingestion of lactose in water in a standard dose.





^ CAUSES OF LACTOSE INTOLERANCE

  • Primary lactose intolerance –

    • An autosomal recessive condition.
    • Results from absence of hydrolytic activity for lactose or absence of intestinal capacity to transport glucose and galactose.
    • Child becomes symptomatic as soon as breast or cow’s milk is started.
    • Responds to elimination of lactose from diet.


^ CAUSES OF LACTOSE INTOLERANCE

  • Secondary lactose intolerance – results from decreased intestinal activity secondary to damage to intestinal mucosa from some primary pathology

    • Acute gastroenteritis
    • PEM
    • Worm infestations
    • Malabsorption syndromes
    • Animal milk allergy
    • Drugs like neomycin, antimetabolites, etc.


^ CONSEQUENCES OF LACTOSE INTOLERANCE.

  • Osmotic diarrhea

  • Metabolic acidosis

  • Bacterial proliferation

  • Caloric loss

  • Pneumatosis intestinalis



^ CLINICAL FEATURES

  • Diarrhea – Watery, frothy, greenish-yellow, sour smelling stools

  • Perinal excoriation

  • Failure to thrive

  • Abdominal distension

  • Borborygmi

  • Flatulence



INVESTIGATIONS

  • Stool pH and reducing substances –

    • Acidic pH < 5.5
    • Reducing substances > 0.5 %
  • Stool chromatography

  • Breath hydrogen test

  • Lactose tolerance test

  • Intestinal enzyme activity by biopsy



TREATMENT

  • Primary lactose intolerance –

    • Elimination of lactose from diet
    • Life long treatment


TREATMENT

  • Secondary lactose intolerance

    • Treatment of primary cause
    • Lactose free diet if
      • Persistent diarrhea
      • Weight loss
      • Reducing substances > 1 %


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