Статья посвящена церебральной аллергии (ЦА), описанной в середине XX века. Отражена эволюция представлений о ЦА; представлены дефиниции, имеющие отношение к ЦА. Кратко рассмотрены механизмы ЦА; приводятся данные о пирролах и пиролурии. Описаны основные неврологические и психиатрические проявления ЦА и пиролурии, а также подходы к их лечению.Отмечено, что имеются все основания полагать, что механизмы возникновения ЦА не имеют существенных отличий от таковых при формировании других аллергических реакций (при пищевой аллергии), основанных на двух основных типах иммунного ответа (сопряженных с избыточной выработкой IgE или с тучными клетками). Симптомы ЦА многочисленны и многообразны. Они зависят от возраста пациентов и могут манифестировать в чрезвычайно широких пределах. Спектр проявлений ЦА варьирует от приступов потери сознания и/или головных болей в сочетании с крапивницей, зудом и нарушениями зрения или транзиторных нарушений речи (афазия и т. д.) до одностороннего онемения конечностей и/или языка, сонливости, ступора, бессонницы, неспособности к концентрации, раздражительности, возбудимости, депрессии, тревожности, головокружения, мышечных проявлений. Проявления ЦА обычно находят выражение в форме конкретных психических нарушений (шизофрения, депрессия, галлюцинации, бред, кататония, эпилепсия, аутизм и т. д.).Указывается, что стандартных методов терапии для ЦА и пиролурии не разработано. Существующие подходы частично являются эмпирическими, а частично теоретически обоснованными. Обычно детям с церебральными проявлениями аллергии назначается гипоаллергенная (элиминационная) диета различной степени интенсивности. При необходимости такая диета может достигать уровня олигоантигенной.

Innovative technologies in the reanimation and intensive therapy permitted to improve the survival of premature infants, including those with extremely low birth weight infants. There are considered various issues of practical medical care for very-low-birth weight infants in the first three years of life. The special attention is given to patients with bronchopulmonary dysplasia (BPD). There is briefly presented the own authors’ experience of the observation for premature infants in conditions of a multidisciplinary team care approach. There were described such important aspects of the mentioned category of patients as neurodietology/nutritional rehabilitation, compliance with aseptic environmental conditions, the correction of visual and hearing impairment, treatment of neurological deficit, especially neuropharmacology, treatment of paroxysmal disorders and epilepsy.

The authors consistently consider various aspects of iron metabolism in children and in pediatric patients with accompanying infectious process. Special attention is attributed to the problem of combined infections with anemia/iron-deficiency states, non-infectious factors of iron deficiency and anemia in childhood, as well as to novel approaches to diagnostics of iron metabolism disorders (including the studies of markers such as hepcidin, ferroportin, soluble transferrin receptor, hypoxia inducible factor, divalent metals transporter, etc). Foreign investigations incorporating novel iron metabolism markers in pediatric patients of various ages with infectious/inflammatory disorders are concisely described. The ambiguity and disputability of iron metabolism in pediatric patients against the background of infections and conditions, accompanied with inflammation are stressed.

CDG Ib type is a rare autosomal recessive disorder (OMIM 154550) manifesting as coagulopathy, hypoglycemia, enteropathy, liver damage, delayed physical development etc. Approximately 20 cases of CDG Ib are described world-wide, being one of two CDG types, responding to treatment (along with CDG IIc type). CDG Ib is caused by a mutation in mannose-6-phosphate isomerase. Then, mannose-6-phosphate for protein N-glycosylation can only be synthesised from outside sources mannose. Treatment with d-mannose is the only described therapy for CDG Ib. We have extablished the diagnosis in 2 patients. Patient 1 was a 9 months old girl with exudative enteropathy, hypoproteinemia, ascites, liver fibrosis, malabsorption, multiple deficiency syndrome, hypoglycemia, coagulopathy with low antithrombin III and protein C, and right atrium thrombosis. Patient 2 was a 10 months old boy with severe physical delay and liver damage. Coagulogram studies showed low antithrombin III/protein C levels. The diagnosis was proved by molecular genetics and biochemistry in both cases. Mutation in mannose-6-phospahte isomerase gene (Ar219Trp – R219W) and abnormally structured glycoproteins (transferrin, α1-antitrypsin, haptoglobin) were discovered in patient 1. Mutation c.1252T>C in homozygotic state was found in MPI gene of patient 2. Treatment with d-mannose (150 mg/kg) resulted in stool normalisation, albumin, glucose, antithrombin III and protein C levels’ increase, intoxication symptoms and clot’s disappearance in 2 weeks. Frequent episodes of acute respiratory infections with manifested CDG symptoms demanded the increase of d-mannose (up to 230 mg/kg). The therapy revealed no side effects. Normal glucose, albumin and antithrobmin III were the criteria for adequate dose selection. We conclude that CDG Ib should be considered in patients with unexplained chronic diarrhoea, hypoglycemia, liver pathology, thromboses/haemorrhages, allowing early diagnostics/effective management for this rare disorder.

Background: There are few data on co-occurring with bronchopulmonary dysplasia diseases but there is no single point of view on their mutual effect.Objective: Our aim was to learn the structure and frequency of extrapulmonary disease, concomitant of bronchopulmonary dysplasia, in children aged up to 3 years.Methods. A retrospective analysis of histories of 93 children with bronchopulmonary dysplasia with an analysis of the consequences of perinatal pathology structure was carried out.Results. On average, each patient with bronchopulmonary dysplasia accounted for 5 comorbidities. The most common (89; 96%) were perinatal lesions of the nervous system and their consequences. In children with bronchopulmonary dysplasia at the age of 3 years there was a relatively low incidence of hydrocephalus and, on the contrary, high — of infantile cerebral palsy. Violations of the organs of vision were found in 58 (62%) children, malnutrition and other violations of physical development — in 58 (62%) and 27 (29%), respectively, and the cardiovascular system pathology — in 59 (63%).Conclusion. The most commonly, extrapulmonary pathology, co-occuring with bronchopulmonary dysplasia, includes neurological deficit with psychomotor retardation, violations of organs of vision, pathology of the cardiovascular system, malnutrition/delay in physical development.

Fabry disease is a serious degenerative hereditary disorder, which is referred to as a lysosomal storage disease and is a form of sphingolipidosis. Fabry disease often starts in childhood and adolescence, although the complete clinical manifestation occurs in adulthood. Early diagnostic is often difficult due to polymorphic clinical picture, untypical initial symptoms and doctors’ low level of awareness. Fabry disease patients should undergo a special kind of pathogenetic enzyme replacement therapy. Timely diagnosis and prompt treatment can prolong life expectancy and improve life quality.

Fabry disease is a serious degenerative hereditary disorder, which is referred to as a lysosomal storage disease and is a form of sphingolipidosis. Fabry disease often starts in childhood and adolescence, although the complete clinical manifestation occurs in adulthood. Early diagnostic is often difficult due to polymorphic clinical picture, untypical initial symptoms and doctors’ low level of awareness. Fabry disease patients should undergo a special kind of pathogenetic enzyme replacement therapy. Timely diagnosis and prompt treatment can prolong life expectancy and improve life quality.

Aim: to evaluate the impact of subcutaneous interferon β-1a on matrix metalloproteinases 3, 8, 9 (MMP), cytokines (tumor necrosis factor α — TNF α, and transforming growth factor β1 — TGF β1) levels in serum of children with relapsing-remitting multiple sclerosis. Patients and methods: the results of treatment of 32 patients with relapsing-remitting multiple sclerosis aged 12–18 years (22 girls and 10 boys) were analyzed. Treatment efficacy was assessed by the number of relapsing-remitting multiple sclerosis exacerbations during 12 months, MRI data, MMP and cytokines dynamics in serum. Results: statistical evidence acquired for MMPs, their tissue inhibitor and cytokines levels decrease (p < 0,005) in patients receiving therapy with subcutaneous INF β-1a. Conclusions: subcutaneous interferon β-1а is highly efficient in treatment of relapsing-remitting multiple sclerosis in children and adolescents.

Neurologic and somatoneurologic aspects of lactose intolerance are considered in the article. Authors stress the role of adequate diet therapy in this form of food intolerance.

Neurologic and somatoneurologic aspects of lactose intolerance are considered in the article. Authors stress the role of adequate diet therapy in this form of food intolerance.

It is known that the interferon beta therapy is more effective for multiple sclerosis. The literature review focuses on the problem of neutralizing antibodies (NAbs) in interferon beta therapy of multiple sclerosis for children and adolescents. The authors provide domestic and foreign data concerning therapy of multiple sclerosis, NAbs negative effects on bioactivity and bioavailability of diseasemodifying treatment, methods of NAbs detection and their impact on multiple sclerosis clinical course, and approaches to management of NAbs high titers. Key words: multiple sclerosis, disease-modifying treatment, interferon beta, interferon beta-1a, interferon beta-1b, neutralizingantibodies, children. (Pediatric Pharmacology. — 2011; 8 (5): 61–64.)

Authors consider the possibilities of using phytomedicine Persen in child neurology and in various fields of pediatrics. Publications from medical periodic press dating from 2000-s and associated with problems of Persen application are cited.

Authors consider the possibilities of using phytomedicine Persen in child neurology and in various fields of pediatrics. Publications from medical periodic press dating from 2000-s and associated with problems of Persen application are cited.Key words: psychosomatic disorders, anxiety, depression, sleep disorders, Persen, infants, children, adolescents.

It is known that the interferon beta therapy is more effective for multiple sclerosis. The literature review focuses on the problem of neutralizing antibodies (NAbs) in interferon beta therapy of multiple sclerosis for children and adolescents. The authors provide domestic and foreign data concerning therapy of multiple sclerosis, NAbs negative effects on bioactivity and bioavailability of diseasemodifying treatment, methods of NAbs detection and their impact on multiple sclerosis clinical course, and approaches to management of NAbs high titers. Key words: multiple sclerosis, disease-modifying treatment, interferon beta, interferon beta-1a, interferon beta-1b, neutralizingantibodies, children. ( Pediatric Pharmacology. — 2011; 8 (5): 61–64.)