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- W2911238519 abstract "Pancreatic agenesis is a rare condition that occurs when the pancreas fails to develop before birth. The severity depends on how much functional pancreatic tissue is present. Children with this pathology experience neonatal diabetes due to lack of insulin. Pancreatic agenesis may occur in association with a number of other birth defects. Martinez-Frias syndrome is an extremely rare, autosomal recessive disorder, characterized by the combination of multiple malformations: duodenal atresia, biliary atresia, hypoplastic pancreas, intrauterine growth restriction. There are only 6 cases presented so far in the literature. We present the case of a child diagnosed at birth with complex cardiac malformations (interrupted aortic arch, VSD, pulmonary artery hypoplasia, tricuspid valve dysplasia), agenesis of the pancreas, gallbladder agenesis and hypospadias. The child underwent cardiac surgery when he was 2 days old and, three days afterwards, he presented with ketoacidosis. Pancreatic insufficiency was controlled initially with continuous intravenous insulin therapy and subsequently with subcutaneous insulin, associated with pancreatic enzymes, maintaining a HbA1c value between 6% and 7%, with favorable outcome, which allowed the child a normal physical and neuropsychological development until the age of 2. As for the cardiac disease, he developed severe heart failure with cardiac fibroelastosis demonstrated during the surgery, which subsequently led to death." @default.
- W2911238519 created "2019-02-21" @default.
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- W2911238519 date "2018-01-01" @default.
- W2911238519 modified "2023-10-17" @default.
- W2911238519 title "Particular case of diabetes mellitus by pancreatic agenesis in a plurimalformative syndrome" @default.
- W2911238519 doi "https://doi.org/10.26416/pedi.52.4.2018.2157" @default.
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