The Centre of Excellence for the Care of Children with Congenital Hyperinsulinism at the University Clinic of Almazov Centre continues to successfully treat young patients from other countries with this severe rare disease.
For example, a 1-year-5-month-old girl with congenital hyperinsulinism (CHI) from Kazakhstan who had severe life-threatening hypoglycemia was recently discharged.
Congenital hyperinsulinism is one of the most dangerous diseases, especially for young children. It is an inherited condition characterized by excessive insulin production. The disease has a negative impact on the central nervous system and the cognitive development of the brain. For quality care of this disease, it is crucial to determine the morphological form of CHI – diffuse, focal or atypical.
At the University Clinic of Almazov Centre, the child received a full diagnostic evaluation and effective treatment – the diagnosis was confirmed, 18F-DOPA PET-CT was performed to clarify the local lesion. A multifocal form of CHI with foci located in the head and body of the pancreas was diagnosed, followed by surgical treatment to remove the foci with Roux-en-y pancreato-jejunal anastomosis. After this complex procedure, the patient underwent medical rehabilitation at the Children's Rehabilitation Clinic of Almazov Centre.
At present, the specialists have confirmed the child's full recovery with restoration of carbohydrate metabolism. All medications have been stopped and the girl has been discharged home.
«It is worth noting that our Centre of Excellence for the Care of Children with Congenital Hyperinsulinism is not only the only one in Russia for the treatment of children with CHI, but has already gained an international reputation. So far, children from Kazakhstan, Belarus, Armenia and other countries have been treated. All treated patients have been discharged with recovery,» says Professor Irina Nikitina, Head of the Centre of Excellence for the Care of Children with Congenital Hyperinsulinism.