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Адрес для корреспонденции: Морозов Сергей Леонидович-к.м.н., научный сотрудник отдела наследственных и приобретенных болезней почек НИКИ педиатрии им. академика Ю.Е. Вельтищева Длин Владимир Викторович-д.м.н., проф., зав. отделом наследственных и приобретенных болезней почек НИКИ педиатрии им. академика Ю.Е. Вельтищева Сухоруков Владимир Сергеевич-д.м.н., проф., зав. лабораторией общей патологи НИКИ педиатрии им. академика Ю.Е. Вельтищева orcid.org/0000-0001-8927-3176 Воронкова Анастасия Сергеевна-научн. сотр. лаборатории общей патологи НИКИ педиатрии им. академика Ю.Е. Вельтищева 125412 Москва, ул. Талдомская, д. 2.

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Accumulating data suggest that the brain undergoes various changes during aging. Among them are loss of both white and gray matter, neurons and synapses degeneration, as well as oxidative, inflammatory, and biochemical changes. The above-mentioned age-related features are closely related to autophagy and mitochondria. Therefore, we aimed to reveal the most peculiar morphological features of brain nervous tissue and to characterize the expression of autophagy and mitochondrial immunohistochemical biomarkers in neurons of different human brain zones during aging. Counting the number of neurons as well as Microtubule-associated proteins 1A/1B light chain 3B (LC3B), Heat shock protein 70 (HSP70), Lysosome-associated membrane protein type 2A (LAMP2A), Alpha subunit of ATP synthase (ATP5A), and Parkinson disease protein 7 (DJ1) immunohistochemical staining were performed on FFPE samples of human prefrontal cortex, corpus striatum, and hippocampus obtained from autopsy. Statistical analysis revealed a loss of neurons in the studied elderly group in comparison to the young group. When the expression of macroautophagy (LC3B), chaperon-mediated autophagy (HSP70, LAMP2A), and mitochondrial respiratory chain complex V (ATP5A) markers for the young and elderly groups were compared, the latter was found to have a significantly higher rate of optical density, whilst there was no significance in DJ1 expression. These findings, while preliminary, suggest that both autophagy and mitochondria are involved in neuronal maintenance during aging and could indicate their potential role in adaptive mechanisms that occur in aging.

Nephropathy is a common associated pathology with hereditary connective tissue dysplasiaPurpose. To determine clinical and laboratory signs of renal pathology in the conditions of persisting hypoxic syndrome and anatomic abnormalities of the urinary system with hereditary connective tissue dysplasia syndromes in children Characteristics of children and research methods. We examined 36 children with Ehlers–Danlos syndrome and 10 children with Marfan syndrome to reveal signs of metabolic disorders in the blood and urine.Results. All children revealed abnormalities of the urinary system. In addition, children with Ehlers–Danlos syndrome demonstrated an increase in certain signs of dysmetabolic nephropathy when growing older: an increased content of parathyroid hormone in the blood, which inactivation and elimination is normally provided by the kidneys. Hypermicroproteinuria with a high content of microelements in proteins, increased excretion of medium molecules, lipid hydroperoxides, glycosaminoglycans, a decrease in antioxidant defense and crystal formation inhibitors are the characteristic signs of dysmetabolic nephropathy.Conclusion. Children with hereditary connective tissue dysplasia syndromes have a risk of developing nephropathy with signs characteristic of dysmetabolic nephropathy, requiring dynamic monitoring by a nephrologist.

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