Tubular Dysfunction and Ruptured Ureter in a Child with Menkes Syndrome.

Children with Menkes disease may develop various urological and renal problems that evolve as the disease progresses. A 4-year-old boy with Menkes disease had multiple bladder diverticula and a history of recurrent urinary tract infection caused by urea-splitting organisms. The child developed urosepsis and right pyelonephritis.

Life long follow up and management strategies of patients living with native livers after Kasai portoenterostomy.

We present a 37 years' experience in the management of biliary atresia (BA) and discuss long-term complications after Kasai portoenterostomy (KPE). A retrospective territory-wide study from 1980 to 2017 on 231 patients with open KPE from three tertiary paediatric surgical centres was performed. Outcome parameters were clearance of jaundice (COJ), native liver survival (NLS) and long-term complications.

D-dimer level correlation with treatment response in children with venous malformations.

Aim: Localized intravascular coagulopathy is present in children with venous malformations (VMs) as evidenced by elevated D-dimer levels. Few studies have looked into the changes in D-dimer after sclerotherapy and its correlation with treatment outcome and complications. Our study aims to investigate changes in D-dimer in children with VMs undergoing alcohol sclerotherapy.

Are all patients with short segment Hirschsprung's disease equal? A retrospective multicenter study.

Background/purpose: Short segment Hirschsprung's disease (HSCR) carries a better prognosis than long segment disease, but the definition of short is controversial. The objective of this study is to determine anatomically the extent of disease involvement that would be associated with a better functional outcome.

Methods: This is a retrospective multicenter (n = 3) study with patients (≥ 3 years) who had transanal pullthrough operation done for aganglionosis limited to the recto-sigmoid colon were reviewed.

An unusual cause of intestinal obstruction in an adolescent: a case report and management review.

A 15-year-old boy presented with intestinal obstruction two weeks following a blunt abdominal trauma. He had progressive bilious vomiting without abdominal distension or peritonitis. The contrast computed tomography (CT) scan of the abdomen provided the definitive diagnosis: there was an obstructing duodenal hematoma, which might have been slowly progressing or have arisen from secondary hemorrhage after the initial injury.