Surgical Repair of Atrial Septal Defect in a Patient with 3-Methylcrotonyl-CoA Carboxylase Deficiency.

3-Methylcrotonyl-CoA carboxylase (3-MCC) deficiency is a rare autosomal recessive disease of leucine catabolism. 3-MCC deficiency may lead to metabolic decompensation under stress; however, outcomes of elective surgery requiring cardiopulmonary bypass (CPB) are unknown. We report a 4-year-old girl with asymptomatic 3-MCC deficiency and atrial septal defect (ASD) who's undergone surgical ASD repair under CPB.

Recurrent Acute Methemoglobinemia in an Infant With Persistent Gastroenteritis: A Case Report.

Acute toxic methemoglobinemia is a rare and fatal condition with increased levels of oxidized hemoglobin. The clinical presentation of methemoglobinemia varies primarily based on total methemoglobin levels in the blood. Patients sometimes have significant cardiopulmonary compromise, but the majority are asymptomatic, with only cyanosis as the most prevalent sign.