Lymphangiectasia: causes, symptoms, diagnosis, treatment

Lymphangiectasia may be local, affecting the submucosa and serous membrane, combined with the expansion of lymphatic capillaries of other organs. Due to insufficient supply of amino acids, there is a violation of the synthesis of albumin, and then gamma globulins in the liver. Due to the loss of lymphocytes, lymphopenia develops.

Primary lymphangiectasia is inherited most often in an autosomal recessive manner. Manifestation can occur at different ages depending on how long the liver's synthetic function reserves can compensate for losses.

ICD-10 code

K55.8. Other vascular diseases of the intestine.

Symptoms

Often, edematous syndrome dominates, diarrhea may be absent. Hypocalcemia is possible, provoking convulsions. Weakness, fatigue, weight loss are associated with hypokalemia. Only in some patients is the disease accompanied by persistent diarrhea, polyfecalia, abdominal distension, steatorrhea, ascites. Unilateral lymphatic edema of the extremities and genitals is common.

Diagnostics

The diagnosis is made by endoscopic examination (the mucous membrane of the small intestine is hypertrophied and resembles a boucle) with the collection of material for biopsy. Contrast lymphography allows to detect dilated lymphatic capillaries. X-ray examination of the abdominal cavity sometimes reveals signs of hypotension, fluid levels, thickening of the intestinal walls. The coprogram has a positive reaction to plasma protein.

Treatment

Treatment involves a diet with increased protein content and limited fats. In case of severe edema syndrome, aldosterone antagonists (veroshpiron) are prescribed.

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