Children with congenital hyperinsulinism now have the opportunity to have high-quality care in accordance with international guidelines in Russia. Before that such patients were referred to foreign clinics.
The main problem of congenital hyperinsulinism is the unregulated insulin production that leads to severe hypoglycemia and irreversible brain damage. In contrast to tumor hyperinsulinism (insulinoma), in the congenital disease the source of insulin secretion cannot be identified by conventional methods. The only way to find it is radioisotope technology of PET-CT using the radiopharmaceutical 18 Fluoro-L-DOPA. Judging by the type of its accumulation in the pancreatic tissue, it is possible to determine if it is a diffuse and a focal form of the disease, indicate the exact localization and size of the pathological tissue – all that is necessary for effective surgery.
After genetic study, the specialists of the Almazov Centre performed PET tomography and identified a focal form of the disease in a four-month-old child with a congenital hyperinsulinism. After its removal, the functional activity of the patient’s pancreatic gland is preserved.
The exact localization in this case turned out to be at the head of the pancreas, and an intraoperative biopsy allowed correct resection saving the pancreatic duct and performing the maximum possible organ-saving operation on the child. At present, the little boy feels well and will go home with his mother soon.
Planning and organization of all stages of medical care for children with congenital hyperinsulinism in accordance with international guidelines was performed by the experts of the Institute of Endocrinology of the Almazov Center and its subdivision, the Laboratory of Pediatric Endocrinology.
The full treatment cycle is realized thanks to the joint professional efforts of the multidisciplinary team of specialists:
• Institute of Endocrinology (Director Professor Elena Grineva; Head of the Pediatric Endocrinology Department Professor Irina Nikitina)
• Nuclear Medicine Group (Professor Daria Ryzhkova)
• Laboratory of Congenital and Hereditary Diseases Surgery (Professor Vladimir Bairov, Head of the Department of Children’s Surgery Dr. Anna Sukhotskaya)
• Laboratory of Pathomorphology (Professor Lyubov Mitrofanova)
• Institute of Molecular Biology and Genetics (Director Prof. Anna Kostareva).