Pentalogy of Cantrell: case report and review of the literature.

Pentalogy of Cantrell is a syndrome that consists of five anomalies: a midline, upper abdominal wall abnormality; lower sternal defect; anterior diaphragmatic defect; diaphragmatic pericardial defect, and congenital abnormalities of the heart. The pathogenesis of this condition is not fully known yet, associations are common with this condition and treatment is extremely challenging. Prognosis and outcome depends on the type of the pentalogy of Cantrell and associated cardiac anomalies. It can be diagnosed reliably by antenatal ultrasound during the first trimester. We describe a Sudanese baby of undetermined sex who had the typical features of pentalogy of Cantrell and died shortly after birth.

Outcome of acute kidney injury in Sudanese children - an experience from a sub-Saharan African unit.

UNLABELLED: ♦ BACKGROUND: Acute Kidney Injury (AKI) is an important cause of morbidity and mortality in developing countries. Although continuous renal replacement therapy is gaining more popularity worldwide, peritoneal dialysis (PD) in children remains an appropriate therapy for AKI in children for all age groups including neonates. ♦ METHODOLOGY: We retrospectively reviewed all children who have been admitted with AKI at the pediatric nephrology unit, Soba University Hospital, Khartoum, during the period from January 2005 to December 2011. ♦ RESULTS: Over 7 years we recorded 659 children of whom 362 (54.9%) were male. The spectrum of age was variable with the majority being neonates, 178 (27.1%). The average patient admission rate was 94 patients per year, with an estimated incidence of 9.8 patients/million population/year. Common causes of AKI were sepsis 202 (30.8%), acute glomerulonephritis 75 (11.5%) and obstructive uropathy due to stones 56 (8.5%). The most common dialysis modality used was PD, 343 (52.4%), and peritonitis was reported in 53 (15.4%) patients. Recovery from AKI was achieved in 450 (68.9%) children, 37 (5.7%) went into chronic kidney disease (CKD), 33 (5.1%) referred to the pediatric surgery and 194 (29.7%) died. ♦ CONCLUSION: In the setting of developing countries where AKI is a common cause of morbidity and mortality, reasonably equipped renal units with adequately trained medical staff may save many lives. International funding programs for communicable diseases and charity organizations should include AKI management in their programs. Acute PD remains the treatment modality of choice for AKI in developing countries.

Morbidity and mortality amongst infants of diabetic mothers admitted into Soba university hospital, Khartoum, Sudan.

The prevalence of diabetes during pregnancy is increasing and this is associated with an increased risk of complications in both mother and fetus. The aim of this research is to study the neonatal complications of maternal diabetes. This was a prospective observational study that was conducted in Soba university hospital between September 2010 and March 2011. All infants born to diabetic mothers during the study period were admitted to the neonatal care unit for evaluation. Data on sex, gestational age, and birth weight, mode of delivery, complications, investigations, birth injury, and length of hospital stay were recorded. Maternal data were retrieved from records. Data was analyzed using Minitab 15. A total of 50 infants of diabetic mothers (IDMs) were included in the study. Thirty infants (60%) were females and 20 (40%) were males. Forty two (84%) of the neonates were born by caesarian section, only 7(14%) were born by spontaneous vaginal delivery. Birth injury was observed in 4% of them. The mean gestational age was 37.2±2.051 weeks. The median birth weight was 3.5 kg. 14 (28%) of the babies were macrosomic, and 17 (34%) were large for gestational age (LGA). Congenital anomalies were found in 3 (6%), hypoglycemia in 6 (12%), hyperbillirubinaemia in 10 (20%), hypocalcaemia and hypomagnesaemia each occurred in 2%, transient tachypnea of the newborn occurred in 5 (10%) of the neonates and respiratory distress syndrome in 2%. Cardiomyopathy occurred in 2% and mortality was 4%. We concluded that macrosomia, LGA, and hyperbillirubinaemia were the commonest complications in IDMs, maternal glycaemic control was found to have a significant effect on a number of outcomes.

Successful separation of craniopagus conjoined twins.

Craniopagus conjoined twins represent a rare phenom- enon of congenital malformation/ dysmorphism. The clinical pathology of this complex entity is reviewed and placed in perspective. Confusion surrounds the severity of craniopagus conjoined twins especially in relation to the difficulty of separation and subsequent outcome. Successful separation of craniopagus twins remains a rarity, however modern neurosurgical tech- niques have created opportunities for successful sepa- ration and brought hope for a normal survival of these children who in the past were often left as historical footnotes or put on display as oddities of nature. We report on a craniopagus conjoined twins from Sudan who had been successfully separated.