Peculiarities of pancreas exocrine and endocrine functions in children with Shwachman–Diamond syndrome

Ипатова, М. Г.; Shumilov, P.V.; Blokh, Sofya; Шагалова, Д Л; Mukhina, Yu.G.

doi:10.24110/0031-403x-2017-96-6-48-52

Cited by 2 publications

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“…Two patients had с.653G>A (p.Arg218Gln) variant and for one patient for every of the following variants: c.258+1G>A, с.107delT, с.356G>A, c.297_300delAAGA, с.338C>T. All children with SDS had growth delay, in 11 (39%) cases we revealed bone abnormalities. In blood samples of 24 (86%) children we revealed neutropenia and less frequently anemia andРезультаты обследования 14 пациентов с генетически подтвержденным СШД опубликованы ранее[15]. SBDS, а также прилегающие к ним интронные области.…”

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confidence: 85%

“…Для СШД характерна экзокринная недостаточность поджелудочной железы с рождения с последующим появлением клинических признаков дефицита жирорастворимых витаминов А, D, Е и К, белково-энергетической недостаточности разной степени тяжести [9,12]. У большинства пациентов определяется снижение уровня панкреатической амилазы и липазы в крови [9,12,15], у 50-75% -повышение активности трансаминаз печени [9,12]. В 88-100% случаев обнаруживаются гематологические нарушения, в частности нейтропения, которую можно диагностировать уже в неонатальном периоде [9,16,17].…”

Section: в помощь врачуunclassified

“…Выражаем признательность С. П. Блох и Д. Л. Ша га ловой, которые участвовали в ранней фазе исследо вания [15].…”

Section: выражение признательностиunclassified

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Genetic and Clinical Features of Shwachman-Diamond Syndrome in Russian Population: Prospective Study

Ипатова

Deordieva

Швец

et al. 2020

Vopr. sovr. pediatr.

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confidence: 85%

Section: в помощь врачуunclassified

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Genetic and Clinical Features of Shwachman-Diamond Syndrome in Russian Population: Prospective Study

Ипатова

Deordieva

Швец

et al. 2020

Vopr. sovr. pediatr.

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“…В послеоперационном периоде введение Базиликсумаба повторяли на вторые сутки в тех же дозах, Такролимус вводили через сутки после окончания операции. Судорог на введение Такролимуса не наблюдали [12]. В качестве глюкокортикоидного фона использовали Метилпреднизолон.…”

Section: материал и методыunclassified

Homeostasis of the early postoperative period in orthotopic liver transplantation in children

Shmakov,

Elizar’Eva,

Kolosov

et al. 2023

jour

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Purpose An observational study was undertaken to identify diff erences in children ' s homeostasis in the early postoperative period after orthotopic liver transplantation (OLT) depending on the age. Materials and methods.We have compared the paraclinical parameters of children in the early postoperative period after OLT in the early postoperative period (up to 4 days). Selected groups: group 1-18 patients aged 2 to 11 months; group 2-13 patients older than 1 year. Observational study. Statistical processing by nonparametric criteria.Results. The identifi ed intergroup diff erences in homeostasis in the immediate postoperative period after OLT were determined mainly by the usual nuances of age-related physiological diff erences. In the children of the fi rst year of life, the functions of the donor liver made it possible to level the preoperative defi ciency in the synthesis of antithrombin-III, but this group demonstrated manifestations of the refeeding syndrome and intestinal motor dysfunction, which led to a prolongation of observation in the intensive care unit. The results obtained are reproducible in the most surgical hospitals. Conclusion.In the children of the fi rst year of life after orthotopic liver transplantation, the digestive and motor functions of the digestive tract are slowly restored, which necessitates prolonged epidural blockade, careful resumption of nutritional support, and prolonged observation in the intensive care unit. In the children older than 1 year, there was noticed a tendency to leukopenia, which increases the probability of infectious complications. No dangerous manifestations of acute renal injury were noted.

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Peculiarities of pancreas exocrine and endocrine functions in children with Shwachman–Diamond syndrome

Cited by 2 publications

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Genetic and Clinical Features of Shwachman-Diamond Syndrome in Russian Population: Prospective Study

Genetic and Clinical Features of Shwachman-Diamond Syndrome in Russian Population: Prospective Study

Homeostasis of the early postoperative period in orthotopic liver transplantation in children

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