Posterior urethral valve (PUV) obstruction is the most common cause of bladder outlet obstruction in boys. Currently, the diagnosis of PUV is commonly made prenatally. In our environment, however, prenatal diagnosis is rare and the diagnosis is usually made postnatally from the clinical and radiological features. This study therefore examines the clinical and radiological spectrum of boys with PUV in our environment. We examined the clinical presentations, ultrasonographic and the micturating cystourethrographic (MCUG) features of boys with PUV in our institution over a 22-month period from June 2006. There were 28 patients with PUV over this period. The age at presentation ranged from 11 days to 11 years (mean age = 2.7 years). Although prenatal ultrasound scan was done in 23 (82.1%) patients, no prenatal diagnosis was made in any of the patients. The diagnosis was made after infancy in 16 (57.1%) patients. Recurrent urinary tract infection (UTI) was the most common mode of presentation occurring in 14 (50.0%) patients with 7 (50.0%) of the patients with UTI presenting with septicaemia. Voiding dysfunctions, which occurred in all the patients, were the primary mode of presentation in 12 (42.9%) patients. Three (10.7%) patients presented with renal insufficiency, which was significantly associated with the age at presentation (P = 0.026). Ultrasound scan done in the postnatal period strongly suggested the diagnosis of posterior urethral valves in 22 (78.6%) patients in whom the posterior urethra was found to be dilated, associated with thick-walled urinary bladder and bilateral hydronephrosis. Trabeculations of the bladder was a constant feature on MCUG. Other features on MCUG included dilatation of the posterior urethra in 26 (92.8%), bladder diverticuli in 15 (53.6%) and unilateral and bilateral vesicoureteric reflux in 3 (10.7%) and 1 (3.6%) patient(s), respectively. The diagnosis of PUV obstruction which is often made late in our environment, is mainly by clinical, sonographic and MCUG features in the postnatal period. A majority of patients present late, with recurrent UTI.

Identifying AKI associated with DEG is difficult. Detailed drug history, increasing metabolic acidosis, and multiorgan deterioration despite peritoneal dialysis should arouse suspicion. Simple diagnostic tests need to be developed and facilities for hemodialysis of infants and financial support provided. Recurrences can be prevented by creating awareness, improving manufacturing practices, field-testing of drugs, and international monitoring of pharmaceuticals imported for manufacture.

Prune belly syndrome (PBS) is a rare congenital disorder affecting 2.5 to 3.8/100,000 live births worldwide. Our objective of this report is to describe clinical manifestation, laboratory, and radiological characteristics of PBS in our patients, to highlight the limitations to offering appropriate patient care due to parents demanding discharge against medical advice and the need to increase the awareness regarding this rare disease. We report three cases; all referred after birth with lax abdominal wall, congenital anomalies of kidney, and urinary tract. One of the patients had an absent right foot. They all had cryptorchidism, and in one, there was deranged renal function. The reported cases had both medical and radiological interventions to varying degrees. They all had an abdominal ultrasound which revealed varying degrees of hydronephrosis, hydroureters, and bladder changes. Voiding cystourethrogram showed vesicoureteric reflux in one of the reported cases. Urinary tract infections were appropriately treated with antibiotics based on sensitivity. PBS management in our setting remains a challenge because of strong cultural beliefs, and high rate of discharge against medical advice. Focus should be on parent education, early diagnosis, and multidisciplinary management approach.

Accumulation of ammonia in the blood may result in an acute life-threatening event in children. Patients typically present with lethargy, poor feeding, and hypotonia; if not managed efficiently, patients can develop seizures, coma, and eventually die. Management of hyperammonemia is difficult in children given the non-specific symptoms, age specific etiologies, and lack of consensus in the treatment plan. We systematically reviewed published literature to provide expert consensus panel recommendations for medical management and renal replacement therapy (RRT) in pediatric patients with hyperammonemia. Methods: PubMed/Medline, Embase and Cochrane database search was performed to include studies about hyperammonemia and RRT in children <18 years old. Two independent reviewers reviewed each title, abstract, and relevant full text articles. An expert panel of international pediatric nephrologists discussed medical management and RRT for hyperammonemia in children at a consensus conference to provide recommendations. Results: The initial search returned a total of 477 citations and 25 studies met the inclusion criteria. A total of 132 patients were included in these 25 studies and were treated with different dialysis modalities. Twenty-three heperammonemia patients were treated with peritoneal dialysis with 65% success rate, five were treated with intermittent hemodialysis with 100% success rate, 92 were treated with continuous renal replacement therapy (CRRT) with 60% success rate and three were treated by extracorporeal membrane oxygenation (ECMO) combined with CRRT and had 100% success rate. Conclusions: Expert panel recommendations were provided with regards to non-RRT (medical management), hemodialysis, peritoneal dialysis, CRRT, high dose CRRT, and hybrid therapy. CRRT was the first line dialysis modality of choice recommended for hyperammonemia management in children. Indications for RRT were variable among studies reviewed; RRT was recommended at ammonia blood level > 400mmol/L or in hemodynamically unstable children irrespective of blood ammonia level. More studies are needed to further strengthen these expert recommendations.