Hirschsprung's disease in Oman.

The incidence of Hirschsprung's disease (HD) was studied retrospectively in Oman using hospital-based data. In Oman there is a single pediatric surgery unit where a register has been kept from 1989 to 1994, and because all cases are referred to this unit, a national survey could be carried out. There were 85 children with HD born between 1989 and 1994, and during the period there were 261,000 livebirths among Omani nationals.

Empty sellae, impaired testosterone secretion, and defective hypothalamic-pituitary growth and gonadal axes in children with Bardet-Biedl syndrome.

We evaluated growth parameters and hypothalamic-pituitary-gonadal and growth functions in five children with Bardet-Biedl syndrome (BBS). Three of the five children had stature below the fifth percentile for age. Their growth hormone (GH) response to provocation was defective, and computed tomographic (CT) scanning revealed empty sellae in all of them.

Defective growth hormone secretion in children with pycnodysostosis and improved linear growth after growth hormone treatment.

Short stature is a characteristic feature of pycnodysostosis. We report defective growth hormone secretion in response to provocation and low insulin-like growth factor-I (IGF-I) concentration in five out of six patients with pycnodysostosis. Physiological replacement with growth hormone increased IGF-I concentration and improved linear growth in these children.

The frequency of posterior urethral valves in Oman.

Objective: To ascertain the frequency of posterior urethral valves (PUV) in Oman, using hospital records.

Materials And Methods: The resident population of Oman is 1.5 million and all complex paediatric surgical cases are referred to a single unit in the capital city of Muscat.

Growth hormone deficiency and empty sella in DIDMOAD syndrome: an endocrine study.

Two girls with DIDMOAD syndrome are presented. One also had severe megaloblastic-sideroblastic anaemia and the other several neurological manifestations. Both were short with defective growth hormone secretion.

Hereditary thrombophilia among 217 consecutive patients with thromboembolic disease in Jordan.

This is a four-year prospective study on patients admitted or referred with thromboembolic disease to Jordan University Hospital or to the Thrombosis/Haemostasis Laboratory at the University of Jordan. The aim of the study was to find the relative prevalence of hereditary thrombophilia. For the purpose of this work, hereditary thrombophilia was diagnosed in the absence of an acquired cause of thrombophilia in addition to two of the following: 1) positive family history of thrombophilia, 2) confirmed same deficiency in a closely related family member, 3) the deficient protein is constantly below 2 SD of the normal mean on repeated testing.

Survey of infection in babies at the Khoula Hospital, Oman.

A 12-month prospective study of infection was carried out in the special care baby unit (SCBU), Khoula Hospital, Muscat, Sultanate of Oman. During this period, 8720 babies were born in the hospital and 1265 were admitted to the SCBU. Altogether, 490 babies were of less than 36 weeks' gestation.

Islet transplantation to the renal subcapsular space in streptozotocin-diabetic rats: short-term effects on glucose-stimulated insulin secretion.

The immediate and short-term effects of transplantation of freshly isolated islets on glucose-stimulated insulin secretion were studied in streptozotocin-diabetic rats. At 2 days after the intravenous injection of streptozotocin (70 mg/kg), either 400 or 1000 freshly isolated, hand-picked islets were transplanted to the left renal subcapsular space. Intravenous glucose infusion (10 mg/min) performed 1, 3, 6, and 10 days after transplantation revealed that already at day 3, rats transplanted with 1000 islets had a significant plasma insulin response to glucose, though the response was lower than in healthy controls.

Neonatal limb gangrene and renal vein thrombosis. Case report with review of literature.

A 3-day-old male infant born to a non-diabetic mother was admitted to the University Hospital with gangrene of the right forearm and some gangrenous patches of the left forearm. This was associated with left renal vein thrombosis. Screening tests for coagulation disorders gave normal results.

Absorption of subcutaneously administered regular human and porcine insulin in different concentrations.

The influence of the insulin concentration on its absorption from the subcutaneous tissues was studied by direct measurement of the appearance of insulin in the circulation after its subcutaneous administration. 10 units of regular insulin of semisynthetic human and porcine origin in strength of 15 units/ml, 40 units/ml, and 100/units/ml were administered as bolus to 9 healthy male volunteers, using switched-off, manually handled insulin pumps. Insulinaemia rose significantly higher (p less than 0.