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February 2015, at the age of 6 months was diagnosed with acute respiratory viral disease, accompanied by rise of temperature up to 38 °C. He was treated as outpatients, ampicillin was prescribed. For the first time mother noticed the swelling on the face, then, during breastfeeding atonic seizures with loss of consciousness occured. The child was hospitalized to the intensive care unit of the regional children's hospital in Aktau. The severity of the condition when taken to the hospital due to neurological symptoms. Biochemical analysis: hypocalcemia(1.57 mmol/l) and hypoproteinemia (31 g/l) was detected. Blood tests for CMV (ELISA, PCR) were negative. Ultrasound of abdominal organs: the presence of a small amount of perihepatic effusion, in and around the splenic departments, between loops of bowel. Computer tomography of the brain revealed retrocerebellar cyst. The disease was complicated by bronchopneumonia. The patient was prescribed antibacterial and symptomatic therapy with positive dynamics. With the improvement of the health status the child was discharged, however, the concentration of protein in the blood remained at a low level (up to 35 g/l). Diagnosis at discharge: pneumonia (severe), retrocerebellar cyst, seizures caused by hypocalcemia. Idiopathic nephrotic syndrome., 2 weeks after discharge, swelling in the face and extremities appeared again, because of what the child was sent to the Scientific Center of Pediatrics for further diagnosis. Health status at admission was of moderate severity. The skin was without rashes, skin turgor decreased, edema of the lower extremities. In the biochemical analysis of blood: protein concentration in serum - 28.0 g/l, albumin 24 g/l, CRP - 9.0 mg/l (followed by a significant increase to 292.4 mg/l within 2 days), hypocalcemia (1.99 mmol/l). Analysis on protein fractions showed decrease of albumin (50% of the reference), α1-globulins - within normal limits, α2 -globulins -was decreased by 50% from the norm, β1-globulins reduced by 28%, β2-globulins - a decline of 80 %, y- globulins reduced by 98% from normal content.signs: the temperature rose up to 38 0C, restlessness, single vomiting, bloating.study: mucosa of the duodenum and jejunum- moderate edematous, thick white coating was observed on the walls of the intestine. lymphangiectasia malabsorption hematoxylin eosinexamination of the jejunum biopsy: Examination of the biopsy of the jejunim mucosa revealed that brush border is visualized throughout. Villi of varying heights, the accumulation of net eosinophilic masses visualized, consolidate friable surface epithelium with the formation of cystic cavities, deforming the villi. Dilated lymphatic vessels were also revealed (Fig 1,2). Crypts are not changed, the number of goblet cells within norm. Panett cells had a moderate amount of azurophilic granules. Lymphoplasmocytic infiltration were revealed in stroma. Conclusion: intestinal lymphangiectasia. Chronic atrophic eyunit without exacerbation.results of immunological examination: the total number of leucocytes within norm, absolute lymphopenia. The ratio of cell subpopulations was severely disrupted due to the decrease of the absolute number of T-helper lymphocytes. The content of activated T- and B-lymphocytes increased. Severe hypogammaglobulinemia of all classes. Immunologist opinion: concomitant immunodeficiency manifested by infectious syndrome due to the underlying disease.doctors, taking into account the age of the infant patient, the presence of massive, symmetrical, peripheral edema, absence of factors that trigger the occurrence of secondary lymphangiectasia, as well as basing on data of histological examination, put the final diagnosis of primary intestinal lymphangiectasia syndrome (Waldman), secondary immunodeficiency.at the initial stage of therapy was as follows: transfusion of albumin to restore protein levels, antibacterial drugs to prevent infectious processes, Octagam to restore the concentration of antibodies, prednisone for anti-inflammatory, as well as drugs for the correction of electrolyte disturbances. Positive effect detected, the child's condition had remained moderate severity without deterioration. The concentration of serum protein after albumin transfusions increased up to 40-42 g/l for a short period of time. Because of the constant hypoproteinemia which was not corrected by albumin and ongoing corticosteroid therapy, decision to start sandostatin therapy in the dosage of 1 mg/kg was made.of the extremities and restlessness appeared 2 hours after injection, but these side effects were leveled in the next hour. Biochemical analysis showed the increase of the protein level up to 46 g/l. With the improvement of the health status child was transferred from intensive care to the Department of somatic pathology. Two days after therapy with sandostatin transfusions of albumin were cancelled. During treatment the protein level increased up to 53 g/l, swelling disappeared, but general sponginess of the tissue still remained. The patient was discharged with recommendations for the permament sandostatin therapy and compulsory monitoring of immunoglobulin level. :

- Intestinal lymphangiectasia should be suspected in every patient with obscure symptoms of hypoalbuminemia, especially on the background of steatorrhea and/or lymphocytopenia.

- Thus, this clinical case has the following interesting features:

. a rare occurrence pathology

. uncommon early onset

. the severity of developed immunodeficiency,

. manifested infectious syndrome that made difficult to diagnose the underlying disease

. rare use of sandostatin in children the first year of life Literature 1. Braamskamp MJ, Dolman KM, Tabbers MM. Clinical practice. Protein losing enteropathy in children. Eur J Pediatr 2010; 169: 1179-1185.

2. Zlatkina A.R. Chronic enteropathy: pathogenesis and treatment strategy// Quality of the life.