Results of the prospective study conducted to assess the perinatal risk factors contribution in the asthma phenotypes formation in childhood are presented. Of 712 children, which have been observed from the first wheezing onset in St Petersburg’s state ambulances, 238 children with bronchial asthma developed in later years of life were included in random sample. Bronchial asthma proceeded in the structure of atopic disease in 128 children (phenotype ABA), and as the part of the limited allergic lesion of respiratory tract - in 110 children (phenotype RBA). It was found that bronchial asthma in mother, especially mother’s asthma with the early age onset, pregnancy pathology and maternal Smoking during pregnancy are the most significant risk factors which contribute in formation of ABA asthma phenotype. Maternal Smoking during pregnancy increased the frequency of severe asthma with ABA phenotype in the offspring during next years of their life. Clinically, the ABA phenotype is characterized by more frequent debut at the age of 1 year and usually diagnosed before 7 years of age, the presence of food sensitization in 70-90 % of cases, and a high level of hyperimmunoglobulinemia E. Such triggers as the Cesarean delivery, absence of the breastfeeding and exposure to tobacco products in the first years of life are the most significant risk factors in formation of the RBA asthma phenotype. The absence of allergic diseases in both parents or allergic ллерголог in mothers, later age debut and diagnosis of asthma, extremely low frequency of food sensibilization (less than 15 %), high frequency of hyperresponsivity, and low frequency of hyperimmunoglobulinemia E, exceeds the norm in 2 times are the typical features of RBA asthma phenotype.

This disease is characterized by the onset of primary immunodeficiency, which expresses itself as autoimmune multisystem failure, often clinically manifests during the first year of life; there are only about 150 cases in the world described by now. IPEX syndrome is caused by FOXP3 gene defect, which is a transcription factor that affects the activity of regulatory T-cells responsible for the maintenance of aytotolerance. There are around 70 pathogenic mutations in this gene described so far. Most patients with IPEX-syndrome have a clinical manifestations of the disease in the early neonatal period or during the first 3-4 months of life. For this disease the following clinical triad of manifestations is typical: Autoimmune enteropathy (100%), diabetes mellitus (70%), skin lesions (65%), as in the syndrome structure includes severe developmental delay (50%), thyroid disease (30%), recurrent infections (20%), rarer autoimmune cytopenia (Coombs-positive hemolytic anemia), pneumonia, nephritis, hepatitis, artrit, myositis, alopecia. However, some cases of later manifestations were described (in patients of more than 1 year of age) when patients did not show all clinical and laboratory symptoms typical for severe forms of the disease. Due to the severity of the disease and the high mortality in this group of patients, it is very important to diagnose it early and start therapy timely. The article describes a clinical case of permanent neonatal diabetes mellitus in the structure of IPEX syndrome.