Waardenburg-Shah syndrome (WS type IV): a rare case from Pakistan.

Waardenburg-Shah syndrome is a rare autosomal recessive [AR] inherited disorder characterized by the presence of Hirschsprung's disease with a high likelihood of aganglionic megacolon, due to which the mortality is high. The management of the condition involves surgical intervention for the removal of the aganglionic segment of the colon. Here, we report a neonate that presented with a white forelock, white eyelashes, iris hypopigmentation, and sensorineural deafness associated with bilious vomiting, refusal to feed, and failure to pass meconium indicating intestinal obstruction.

Fibro Dysplasia Ossificans Progressiva.

We present a case of 7 years old boy with a very rare debilitating autosomal dominant disorder characterized by heterotopic ossification. Fibro dysplasia ossificans progressiva affects 1 in 2 million individuals with only 2 previous cases reported from this region. The disease manifests as multiple foci of bone formation in muscles, fasciae, tendons and ligaments often triggered by trauma.

Successful Repair of a Pseudoaneurysm of Renal Allograft Artery.

Aneurysm of renal artery is a rare vascular complication after renal transplantation. A 60 years man showed an aneurysm of the renal artery in the transplanted kidney was an incidental finding on routine abdominal ultrasound. The function of the allograft was stable.

Erythromycin establishes early oral feeding in neonates operated for congenital intestinal atresias.

Purpose: The recovery of gastrointestinal function following surgery for congenital intestinal atresias can be prolonged and may increase morbidity and hospital stay. This study was conducted to investigate the prokinetic effect of erythromycin in neonates undergoing surgery for small bowel atresias.

Methods: A randomized-controlled trial was conducted at the Departments of Paediatrics and Paediatric Surgery, Military Hospital, Rawalpindi, Pakistan, from January to December 2007 to study the prokinetic effect of erythromycin (3 mg/kg per dose 4 times daily).

An unusual cause of haemoperitoneum in a child.

We present a case of haemoperitoneum in a child, who presented with signs of acute abdomen with subacute intestinal obstruction. Abdominal paracentesis aspirated fresh blood. Ultrasound and CT scan abdomen showed loculated haemoperitoneum.

A survey of infection control practices of consultant anaesthesiologists in teaching hospitals of pakistan.

Objective: To determine the infection control practices of Anaesthesiologist in the teaching hospitals of Pakistan.

Study Design: Cross-sectional survey.

Place And Duration Of Study: The survey was conducted from January 2002 to December 2002 in Combined Military Hospital Quetta.

Wilms' tumour: a comparison of surgical aspects in patients with or without pre-operative chemotherapy.

Objective: To compare the technical aspects of Wilms' tumour (WT) surgery in patients with and without pre-operative chemotherapy.

Design: Quasi-experimental.

Place And Duration Of Study: Military Hospital (MH) and Combined Military Hospital (CMH), Rawalpindi, from January 1999 to December 2004.

Bicycle and motorcycle spoke injuries in children as passengers.

Objective: To determine the spectrum and severity of injuries seen in children passengers, who get their feet entangled in the spokes of bicycle and motorcycle wheels, and to study the mechanism of this injury to find a pattern.

Design: Descriptive and prospective study.

Place And Duration Of Study: Combined Military Hospital, Quetta, between July 2001 and June 2003.

Planned external fixation to locked intramedullary nailing conversion for open fractures of shaft of femur and tibia.

Objective: To determine the outcome of initial external fixation and then conversion to intramedullary nailing in patients having open fracture of shaft of femur or tibia.

Design: Descriptive study.

Place And Duration Of Study: Combined Military Hospital (CMH), Quetta, from July 2002 to July 2004.

Persistent hyperinsulinemic hypoglycemia of infancy - nesidioblastosis.

Persistent hyperinsulinemic hypoglycemia of infancy (PHHI) or nesidioblastosis is a rare condition presenting with severe hypoglycemia. Prompt diagnosis and early pancreatectomy can save many of them, in spite of the magnitude of surgery. We present two cases in which near total pancreatectomy was performed with favourable outcome.

Triage and management of mass casualties in a train accident.

Objective: The purpose of this study was to assess the practicality and usefulness of different scoring systems of triage process in mass casualty management.

Design: Descriptive/observational.

Place And Duration Of Study: An experience of management of train accident in Balochistan in the year 2003.