CLINICAL CASE SYNDROME CHEDIAK-HIGASHI AT 4 YEARS OLD KID
The article presents the clinical data to identify patients with a rare hereditary disease - Chediak-Higashi syndrome. Chediak-Higashi syndrome - is extremely rare hereditary disease with autosomal recessive mode of inheritance. Its prevalence of less than one case per million population. Clinical manifestations are due to violation of education in neutrophils due to a defect phagolysosomes Lyst-protein, which leads to incomplete phagocytosis. The disease manifests in early childhood with severe recurrent pyogenic infections that occur with high fever. It affects the skin, with the development of skin and subcutaneous tissue abscesses, respiratory system - bronchitis, pneumonia, lymphadenopathy. The most common infection is streptococcal and staphylococcal etiology. Patients SCHH higher risk of developing malignancies. Prognosis is poor. The disease usually leads to death before the age of 10 years.