PRIMARY IMMUNODEFICIENCY IN A CHILD WITH HETEROTAXY

Heterotaxy is a complicated symptom complex in which the location of the main internal organs differs from their normal and complete mirror reflection. Ivemark syndrome - a combination of spleen agenesis with congenital heart disease and abnormalities in the location of the abdominal organs. The exact reason is unknown. Mostly sporadic cases. Genetic and infectious-toxic factors that damage the differentiation of embryonic tissues between the 31st and 36th days of embryogenesis are discussed. The article presents a clinical observation of a patient with Ivemark syndrome, illustrating the difficulties of diagnosing congenital malformations. The peculiarity of this clinical case was determined by the combination of the syndrome with primary immunodeficiency and vascular malformation. Ivemark syndrome is a rare disease, therefore, the awareness of primary care physicians in relation to this pathology is low. Early diagnosis of primary immunodeficiency before a child is at risk of infection is extremely important. There is demonstrated an example of pharmacological correction, which allows prolonging the patient's life.