Spectrum of CFTR gene mutations in patients with chronic pancreatitis in Russia

Razumovskiy

et al. 2022

jour

Chronic pancreatitis is one of the most pressing problems of pediatric gastroenterology and surgery of hepatopancreatobiliary organs. Diagnosis and treatment of this category of patients requires a comprehensive examination using modern highly sensitive research methods and the collegial participation of a surgeon, gastroenterologist and endocrinologist. Due to the fact that the algorithm for managing these patients is not regulated, patients often receive enzyme replacement therapy for a long time, with indications for surgical treatment. In addition, the non-speciﬁcity of complaints and clinical manifestations of chronic pancreatitis, the asymptomatic course and the initial detection of exo- and endocrine insufficiency lead to a later choice of the optimal treatment method and increase the risk of complications. The key to effective care for children with chronic pancreatitis is the staging and continuity in research and treatment. At the Center for the Treatment of Developmental Anomalies and Diseases of the Hepatopancreatobiliary System in Children on the basis of the Filatov Hospital, highly effective interventions are carried out for chronic pancreatitis in children, the purpose of which is to ensure an adequate outﬂow of pancreatic juice using longitudinal pancreaticojejunostomy, which, in addition to draining the pancreas, allows to achieve clinical remission and stop the progression pathological process, including diabetes mellitus. The article reﬂects the experience of diagnosis and treatment of chronic pancreatitis in children.

Section: заключениеunclassified

Modern aspects of diagnosis and treatment of chronic pancreatitis in children: experience of the center Center for the treatment of developmental anomalies and diseases of the hepatopancreatobiliary system in children

Razumovskiy

et al. 2022

jour

“…Техника операции стандартизирована и подразумевает мобилизацию и удаление головки поджелудочной железы и двенадцатиперстной кишки, холецистэктомию с резекцией общего желчного протока, с последующим реконструктивным этапом -наложение панкреатоеюноанастомоза, холедохоеюноанастомоза и гастроеюноанастомоза на петле кишки по Ру (рис. [4][5][6][7][8][9][10][11].…”

Section: материалы и методыunclassified

“…Учитывая тяжесть патологии и сложность проводимого оперативного вмешательства, пациенты этого профиля должны концентрироваться только в специализированных центрах, обладающих всем спектром диагностических и лечебных мероприятий, а также достаточным опытом применения данной технологии и владением микрохирургической техникой. Всем детям после ПДР рекомендовано находиться на длительном диспансерном учете у детского хирурга и гастроэнтеролога, проводить контрольные биохимический и клинический анализы крови, выполнять инструментальные исследования (контрольное МРТ, КТ и рентгенологическое исследование пассажа по ЖКТ с контрастом) [9].…”

Section: заключениеunclassified

Experience of performing pancreatoduodenal resection in children

Razumovsky

et al. 2022

jour

Introduction. The article is devoted to the experience of treating children with pancreatic tumors who underwent pancreatoduodenal resection (PDR) at the Department of Pediatric Surgery, Faculty of Pediatrics, N. I. Pirogov Russian National Research Medical University and at the Center for the Treatment of Developmental Anomalies and Diseases of the Hepatopancreatobiliary System in Children on the basis of the N. F. Filatov Children’s City Clinical Hospital. This type of operation was performed in children with tumors of the head of the pancreas, with the spread of fibroinflammatory changes to nearby organs in chronic pancreatitis and with complicated forms of chronic duodenal obstruction. Materials and methods. For the period from 2010 to 2021 9 PDEs were performed in patients aged 1 to 16 years. The results of treatment in this category of patients were assessed as satisfactory. It is noted that the postoperative period has a number of features: prolonged restoration of gastric motility and passage through the gastrointestinal tract (100% of patients), exacerbation of chronic pancreatitis (100% of patients). Against the background of conservative therapy in all children (100% of patients), the symptoms of pancreatitis were stopped in the period from 26 to 41 postoperative days, the evacuation function of the stomach was fully restored within 2-4 weeks after the operation. Discussion and conclusions. Among the postoperative complications, there were: stenosis of biliary and pancreatic-digestive anastomoses (22% and 11% of those studied, respectively), adhesive intestinal obstruction (11% of cases), intra-abdominal bleeding (11% of patients). One patient had a lethal outcome after repeated surgical interventions for pancreatic head limphangioma complicated by intra-abdominal bleeding and biliary peritonitis. The prognosis in this category of patients is favorable - in the follow-up after surgical treatment in all children (100% of patients), the passage through the gastrointestinal tract is not disturbed, the endo- and exocrine function of the pancreas is preserved (100% of patients), there were no indications for prescribing enzyme therapy and insulin preparations …

“…Н. Ф. Филатова. Кроме того в ряде случаев были исследованы генетические маркеры панкреатита (SPINK -serine protease inhibitor Kazal type 1, CFTR -сystic fibrosis transmembrane conductance regulator, PRSS1-cationic trypsinogen) и онкомаркёры (СА -Carbohydrate antigen и СЕА -Carcinoembryonic antigen) [6,7]. В ДГКБ им.…”

Section: материалы и методыunclassified

Experience in surgical treatment of diseases of the pancreas in children

Разумовский

et al. 2021

jour

The article is devoted to the experience of surgical treatment of pathology of the pancreas at the Center for the Treatment of Developmental Anomalies and Diseases of the Hepatopancreatobiliary System in Children on the basis of the Children’s City Clinical Hospital No. 13 named after NF Filatova: annular pancreas, acute and chronic pancreatitis, tumors and cysts of the pancreas. From 2016 to 2020, more than 267 reconstructive interventions on the pancreas were performed, including longitudinal and transverse pancreatojejunoanastamosis, pancreatoduodenal resection, cystoenteroanastamosis, sectoral and total resection of the pancreas. In the postoperative period, the most severe complication in this category of patients was arrosive bleeding, which is the main cause of all deaths after surgery.