Advanced liver disease in Russian children and adolescents with chronic hepatitis C.

Russia has one of the highest prevalences of paediatric chronic hepatitis C infection (CHC). Our aim was to provide a detailed characterization of children and adolescents with CHC including treatment outcomes. Thus, an observational study of children with CHC aged <18 years was conducted in three hepatology centres from November 2014 to May 2017. Of 301 children (52% male), 196 (65%) acquired HCV vertically, 70 (23%) had a history of blood transfusion or invasive procedures, 1 injecting drug use and 34 (11%) had no known risk factors. Median age at HCV diagnosis was 3.1 [interquartile range, IQR 1.1, 8.2] and 10.8 [7.4, 14.7] at last follow-up. The most common genotype was 1b (51%), followed by 3 (37%). Over a quarter of patients (84, 28%) had raised liver transaminases. Of 92 with liver biopsy, 38 (41%) had bridging fibrosis (median age 10.4 [7.1, 14.1]). Of 223 evaluated by transient elastography, 67 (30%) had liver stiffness ≥5.0 kPa. For each year, increase in age mean stiffness increased by 0.09 kPa (95% CI 0.05, 0.13, P < 0.001). There was significant correlation between liver stiffness and biopsy results (Tau-b = 0.29, P = 0.042). Of 205 treated with IFN-based regimens, 100 (49%) had SVR24. Most children (191, 93%) experienced adverse reactions, leading to treatment discontinuation in 6 (3%). In conclusion, a third of children acquired HCV via nonvertical routes and a substantial proportion of those with liver biopsy had advanced liver disease. Only half of children achieved SVR24 with IFN-based regimens highlighting the need for more effective and better-tolerated treatments with direct-acting antivirals. Further studies are warranted in Russia on causes and prevention of nonvertical transmission of HCV in children

Современные возможности эндохирургической биопсии у детей с хроническими болезнями печени

Chronic liver diseases in children are a group of pathological conditions which varies greatly in etiopathogenetic features heterogeneity and clinical picture, tends to progress if associated with cirrhosis and decompensate hepatic functions consequently following by orthotopic transplantation. Despite the introduction of many non-invasive methods of examination, biopsy continues to be a standard in the diagnosis of histopathological changes in liver tissue. The article reflects modern ideas about the efficacy and safety of various methods of liver biopsy in children. Comparative analysis results on the informative value of hepatobioptates obtained by various puncture techniques are provided.Хронические болезни печени у детей представляют обширную, неоднородную по этиопатогенетическим признакам и клинической картине группу патологических состояний, неуклонно прогрессирующих с развитием цирроза и декомпенсацией печеночных функций, что в конечном итоге может потребовать ортотопической трансплантации. Несмотря на внедрение множества неинвазивных методов обследования, биопсия остается ключевым методом диагностики гистопатологических изменений печени. В статье отражены современные представления об эффективности и безопасности различных методов биопсии печени у детей. Приведены данные сравнительного анализа информативности гепатобиоптатов, полученных посредством различных методик пункции (чрескожной, трансъюгулярной, лапароскопической).КОНФЛИКТ ИНТЕРЕСОВАвторы декларируют отсутствие явных и потенциальных конфликтов интересов, связанных с публикацией настоящей статьи

Bilateral Pale Cell Acanthoma of Mammary Glands in 13 Years Old Boy: Clinical Case

Background. Degos acanthoma is rare benign tumor. Typical signs of this disease are localization on the lower limbs, abdomen and anterior chest in middle-aged and elderly patients (50–70 years old). The only one clinical case of pale cell acanthoma with the atopic dermatitis, and eczema in the area of areoles in the 26 years old patient was described.Clinical Case Description. The boy had redness and swelling in the area of the right mammary gland at the age of 11 years. This mass lesion was regarded as the eczema of the areola region due to burdened allergic history (atopic dermatitis), thus, antihistamine therapy was prescribed. Similar lesion has appeared in the area of the left areola 3 months later, the same therapy was used. Saniopurulent discharge was occasionally noted from the lesions. The child was consulted by dermatologist, endocrinologist and surgeon at the place of residence, and diagnosed with chronic purulent mastitis. Conservative therapy had no positive dynamics. Bilateral changes in areoles were revealed during hospitalization at the age of 13 years. There were red lesions with non-homogenous surface, yellow discharge, hyperemia around the right areola. The lesion sizes were 2.03.0 cm on the left and 3.03.5 cm on the right side. Biopsy has revealed pale cell acanthoma. Conservative treatment with oral and systemic glucocorticosteroids was prescribed, positive dynamics was obtained for 3 days after the therapy initiation.Conclusion. The presence of eczema in the area of the nipple associated with sanioserous discharge requires the differential diagnosis with pale cell acanthoma. The conservative therapy (with glucocorticosteroids) efficacy for pale cell acanthoma is shown

Modern Possibilities of Endosurgical Biopsy in Children with Chronic Liver Diseases

Chronic liver diseases in children are a group of pathological conditions which varies greatly in etiopathogenetic features heterogeneity and clinical picture, tends to progress if associated with cirrhosis and decompensate hepatic functions consequently following by orthotopic transplantation. Despite the introduction of many non-invasive methods of examination, biopsy continues to be a standard in the diagnosis of histopathological changes in liver tissue. The article reflects modern ideas about the efficacy and safety of various methods of liver biopsy in children. Comparative analysis results on the informative value of hepatobioptates obtained by various puncture techniques are provided

Клинические рекомендации по ведению детей с дефицитом лизосомной кислой липазы

Lysosomal acid lipase deficiency is s a rare hereditary enzymopathy. The article presents epidemiological data and features of etiopathogenesis of two phenotypic forms of lysosomal acid lipase deficiency — Wolman disease and cholesterol ester storage disease. Special attention has been given to the key issues of differential diagnostic search, clinical guidelines based on the principles of evidence-based medicine have been given.Дефицит лизосомной кислой липазы — редкая наследственная ферментопатия. В статье представлены эпидемиологические данные и особенности этиопатогенеза двух фенотипических форм дефицита лизосомной кислой липазы — болезни Вольмана и болезни накопления эфиров холестерина. Подробно описаны клинические характеристики быстропрогрессирующей формы и медленно развивающейся болезни накопления эфиров холестерина. Особое внимание уделено ключевым вопросам дифференциально-диагностического поиска, приведены рекомендации по лечению, основанные на принципах доказательной медицины