Cholelithiasis in children with hereditary spherocytosis

Abstract

Hereditary spherocytosis (Minkowski-Shoffar disease) - protein-dependent mebranopathy, leading to the destruction of red blood cells, is one of the causes of gallstone disease. The purpose of this work: to acquaint doctors of various specialties with a possible complication of hereditary spherocytosis in the form of cholelithiasis. The article briefly presents etiopathogenesis, clinical features, diagnosis and treatment of this disease. Two clinical cases of cholelithiasis with hereditary hemolytic microspherocytic anemia of Minkowski-Shoffar are described. Results. In the cases presented, there was a severe course of hereditary spherocytosis with hemolytic crises. A detailed picture of suprahepatic jaundice due to microspherocytosis, a decrease in the osmotic resistance of red blood cells, was accompanied by anemia, an increased content of reticulocytes, an increase in the level of indirect bilirubin. The formation of large amounts of thick bile created the conditions for the formation of stones in the gallbladder. Patients had surgical treatment of gallstone disease. Conclusion: pediatricians, family doctors, hematologists need to remember about the possible complication of hereditary spherocytosis in the form of gallstone disease for forehanded diagnosis and treatment.