Lymphangiectasia: causes, symptoms, diagnosis, treatment

Lymphangiectasia can be local, affecting submucosal and serous membranes, combined with the expansion of lymphatic capillaries of other organs. Due to insufficient intake of amino acids, there is a violation of albumin synthesis, and then of gamma globulin in the liver. Owing to the loss of lymphocytes, lymphopenia develops.

Primary lymphangiectasis is inherited more often in the autosomal recessive type. The manifestation can occur at different ages, depending on how long the reserves of the synthetic function of the liver can compensate for the loss.

ICD-10 code

K55.8. Other vascular diseases of the intestine.

Symptoms

Ocular syndrome often prevails, diarrhea may be absent. Possible gipokaltsiemiya, provoking convulsions. Weakness, fatigue, loss of body weight are associated with hypokalemia. Only in some patients the disease is accompanied by persistent diarrhea, polyphecal, bloating, steatorrhea, ascites. Often there are unilateral lymphatic edema of the extremities, genital organs.

Diagnostics

The diagnosis is made with endoscopic examination (the mucosa of the small intestine is hypertrophied and resembles a boucle) with the taking of material for biopsy. Contrast lymphography can detect enlarged lymphatic capillaries. When X-ray examination of the abdominal cavity, sometimes there are signs of hypotension, fluid levels, thickening of the intestinal walls. In the coprogram, a positive reaction to plasma protein is positive.

Treatment

Treatment involves a diet with high protein content and restriction of fat. When the edematous syndrome is expressed, aldosterone antagonists (veroshpiron) are prescribed.

[1], [2], [3], [4]