Repair of proximal hypospadias with single-stage (Duckett's method) or Bracka two-stage: a retrospective comparative cohort study.

Background: Surgical correction of proximal hypospadias is challenging. The complication rate of proximal hypospadias is still high, and the debate over its optimal treatment is ongoing. Duckett's method is a classic non-staging operation, and two-stage Bracka repair is an attractive alternative procedure.

Case report: maple syrup urine disease with a novel DBT gene mutation.

Background: Maple syrup urine disease (MSUD) is a potentially life-threatening metabolic disorder caused by decreased activity of the branched-chain α-ketoacid dehydrogenase (BCKD) complex. Mutations in four genes (BCKDHA, BCKDHB, DLD and DBT) are associated with MSUD. Here, the presenting symptoms and clinical course of a case of MSUD with a novel DBT gene mutation are described.