Introduction. Congenital hyperinsulinism (CHI) is an inherited disease characterized by severe persistent hypoglycemia in the neonatal period due to insulin hypersecretion and rapid development of neurologic and systemic disorders. There are two main CHI forms: a diffuse one when hyper functioning of beta-cells affect the whole pancreas, and a focal one, characterized by an area of intact parenchyma. Currently, surgery is an acknowledged approach in focal forms, while in diffuse CHI forms surgical treatment is prescribed only in severe pharmacoresistant cases.Material and methods. A total of 11 patents with CHI, aged 1.5 - 26 months, had laparoscopic pancreatic resections at the department of pediatric surgery of Russian Medical Academy of Continuous Professional Education. All patients were examined , treated and prepared for surgery at the Endocrinology Research Centre ( Moscow). The focal CHI form was diagnosed in 3 children; the diffuse one - in 8 patients. In the focal CHI form, a focal resection was done: laparoscopic corpocaudal resection of the pancreas – 2 patients; resection of the pancreatic head with Roux–en-Y pancreaticojejunostomosis - 1 child. All children with diffused disease had laparoscopic near-total (98%) pancreatectomy.Results. In all cases, there were no intraoperative complications. Stenosis of the distal common bile duct developed in one child after the resection of pancreatic head which required a laparoscopic cholecystoduodenoanastomosis. There were no other complications. Two children with focal CHI were reported to recover completely; one patient suffered of hypoglycemia attacks not related to the increased insulin production. In diffuse cases, a stable euglycemia was achieved in 2 patients; 3 children had recurrent hypoglycemia attacks which required Somatostatin analogue therapy. One patient developed diabetes mellitus. In this case, follow-up period lasted for 2 months which is too short to assess the endocrinological outcome.Conclusion. The article presents the first Russian experience of laparoscopic pancreas resection in children with CHI. The laparoscopic approachcan be successfully applied in the surgical treatment of children with CHI.
The paper describes the characteristics of the natural features of the northwestern part of the Southern Urals, near the transition zone of the advanced ridges of the Urals to the band of the Urals. Administratively, the territory covers the vicinity of the village of Lakly in the Salavatsky district of the Republic of Bashkortostan and is part of the UNESCO Yangan-Tau Geopark. The description of unique geological and geomorphological objects is given. The necessity of properly informing the population about the importance of natural objects, the need for careful attitude to them and environmental education, as well as the involvement of this territory in the network of tourist excursion routes, the directions of its development are given.
Introduction. The small size of the bladder plate is a poor prognostic sign for successful treatment of exstrophy. Primary closure in newborns and infants with microcystisis often accompanied by complications and relapses; there for, the approach to the treatment of this group of patients requires a more detailed analysis. The aim of the study. Identify advantages and disadvantages of delayed primary closure surgery in patients with bladder exstrophy and microcystis. Materials and methods. During the period from 1994 to 2020, 265 children with bladder exstrophy were subjected to surgery, 123 patients were newborns. There were 37 children with microcystis (bladders plate <3 cm). In 30 children, primary closure was performed at the newborn age. Seven patients with microcystis were subjected to everyday manual and mechanical stretching of the bladder and injections of botulinotoxin type A into the bladder plate (2-3 times) for 1-2 years. In 5 children the size of the bladder plate was increased from 3 cm to 6 cm, they were underwenteded delayed primary closure at the age of 1-2 years, supplemented by ureteroneoimplantation, bladder neck reconstruction, with bilateral osteotomy. Results. Complications of primary closure in patients operated on during the neonatal period (30) such as relapse of the exstrophywere observed in 10 patients (33.3%) and partial wound dehiscence in 3 children (10%). In patients with microcystis that underwent delayed primary closure after stretching the bladder, there was no relapse of exstrophy, and growth of the bladder was observed. Conclusions. In our opinion, delayed primary closure of the bladder in children with microcystis after mechanical stretching of the bladder plate in combination with injections of botulinum toxin type A into the detrusor can improve the results of correction of exstrophy.
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