Posterior urethral valves in South African boys: Outcomes and challenges.

BACKGROUND: Posterior urethral valves (PUV) are a common cause of congenital obstructive nephropathy. The outcome of patients with PUV at Chris Hani Baragwanath Academic Hospital in Johannesburg, South Africa, has not been documented previously. OBJECTIVES: To describe the outcome of patients diagnosed with PUV over a 29-year period from January 1985 to December 2013, and to analyse risk factors for chronic kidney disease. METHODS: This was a retrospective record review of boys aged <14 years diagnosed with PUV at Chris Hani Baragwanath Academic Hospital. PUV was diagnosed by a voiding cystourethrogram (VCUG) and/or at cystoscopy. Valves were resected primarily or after vesicostomy. The glomerular filtration rate was calculated using the Schwartz formula, and stratified as normal or decreased for age at presentation and at the final visit. RESULTS: Records of 181 patients were analysed. The diagnosis was made during the first year of life in 139 patients (76.8%). Clinical presentation included urinary tract infection (UTI) in 109 patients (60.2%), palpable bladder in 98 (54.1%), palpable kidney in 85 (46.9%), and poor urinary stream in 78 (43.1%). An ultrasound scan was reported normal in 10.9%. Vesicostomy was performed in 80 patients (44.2%) and primary valve ablation in 101 (55.8%), with vesicostomy being more prevalent in the pre-2000 era. The median duration of follow-up was 21 months (interquartile range 5 - 79) and renal outcome at last visit was normal in 117 patients (64.6%). The presence of bladder diverticula was associated with a favourable renal outcome. Thirteen patients (7.2%) died, and 102 (56.3%) defaulted from follow-up. CONCLUSIONS: PUV frequently presents with UTI and palpable bladder and/or kidneys. Findings on ultrasound were normal in 10.9% of our patients with PUV. A VCUG is indicated in the presence of palpable kidneys or bladder even if the ultrasound scan is normal. Bladder diverticula as a pressure-release mechanism are renoprotective. Vesicostomy or primary valve ablation did not affect final renal outcome. Chronic kidney disease occurred in 34.8% of patients after surgical correction. Adherence to scheduled appointments is problematic in this population. Long-term follow-up is mandatory.

Demographics and presenting clinical features of childhood systemic lupus erythematosus.

OBJECTIVES: To review the presentation and characteristics of children with systemic lupus erythematosus (SLE). METHODS: The records of children with sufficient American College of Rheumatology (ACR) criteria for SLE treated by the renal units of the Johannesburg and Chris Hani Baragwanath hospitals, and the arthritis clinic of the Johannesburg Hospital between January 1974 and March 2000 were reviewed. The clinical presentation, age distribution and race were examined. RESULTS: A total of 36 children met the criteria. There were 26 girls and 10 boys, with a mean age of 11.5 and 10.2 years respectively. The male-to-female ratio was 1:2.6 overall, with a ratio of 1:1.2 under 10 years and 1:4 over 10 years. There were 15 white, 2 Indian and 5 coloured patients. The 14 black patients all presented after 1986. Rashes were found to be the commonest clinical feature present at the time of diagnosis, followed by polyarthritis and renal pathology. Constitutional symptoms were common, as were generalised lymphadenopathy and hepatosplenomegaly, while neurological, pulmonary and cardiac signs and symptoms were less common. Renal disease was present in 58% of patients on presentation. CONCLUSION: There is a diverse array of presenting features in childhood SLE. There has been increased recognition of the disease in young black South Africans since 1986.

Pulse cyclophosphamide for steroid-resistant focal segmental glomerulosclerosis.

We report the response of ten patients (6 male, 4 female) with steroid-resistant focal segmental glomerulosclerosis (FSGS) to treatment with intravenous pulse cyclophosphamide (IVCP) together with oral prednisone. All patients had been treated with 60 mg/m2 oral prednisone daily for 2 months upon initial presentation. IVCP was given monthly at a dose of 500 mg/m2 over 6 months. Oral prednisone was given concurrently at 60 mg/m2 daily for 2 months and then on alternate days for 4 months, followed by 30 mg/m2 on alternate days for 6 months. Prednisone was then tapered monthly by 10 mg and finally discontinued. Five patients failed to respond to steroids from the onset and were considered as primary steroid resistant. Two of these patients achieved sustained remission after IVCP, one patient showed a partial response, with loss of edema, normalization of serum albumin, and persistent proteinuria, while two patients showed no response to IVCP. The other five patients had achieved remission after 2 months of daily prednisone at 60 mg/m2 upon initial presentation, but had suffered from more than three relapses per year and had eventually become steroid resistant. They were considered secondary steroid resistant. All five patients achieved sustained remission after IVCP. None of our patients suffered from adverse effects of IVCP. We suggest IVCP as an adjunctive therapy for steroid-resistant FSGS, particularly for patients with secondary steroid resistance.

Gallium scintigraphy in the diagnosis and total lymphoid irradiation of Takayasu's arteritis.

Takayasu's arteritis (TA) in children causes appreciable morbidity and mortality, predominantly as a result of the complication of renovascular hypertension (RVH). Ten children with TA, complicated by RVH, were treated at our centre over the past decade. An initial raised erythrocyte sedimentation rate (ESR) and a purified protein derivative greater than 15 mm were present in every case. More recently, gallium scintigraphy has been used to demonstrate sites of active inflammation in affected vessels (3/4 patients) which became negative after total lymphoid irradiation (TLI). The latter was used in the last 6 children, and appeared to be effective in controlling disease activity as evinced in the normalisation of their ESRs and negative findings on gallium scintigraphy (in all 3 patients with prior active inflammation). Because of vascular damage caused by the vasculitic process, surgical intervention is often required to improve organ perfusion, particularly of the kidney/s. Renal autografting (or allografting) seems preferable (6/11 kidneys functional) to renal bypass grafting (5/5 kidneys clotted). Patient survival improved when TLI was used in addition to standard surgical and medical therapy; this included steroids and antituberculous therapy with TLI, and steroids and cyclophosphamide in the two relapses. Five of 6 patients treated with TLI were alive after 32-54 months' follow-up, while 4 patients who received standard medical and surgical therapy but not TLI all died within 18 months of diagnosis. Gallium scintigraphy is a helpful diagnostic tool in assessing vasculitic activity in TA; TLI is an important mode of immunosuppression, but still needs to be compared with cyclophosphamide as the major immunosuppressive.

Evaluation of urinary tract infection in malnourished black children.

Urinary tract infection (UTI) is a well recognized complication in malnourished children. The need to investigate these patients for underlying renal pathology has not been clearly defined. Seventy-five children with malnutrition were evaluated for UTI by culture of urine obtained suprapubically prior to antibiotic therapy. All patients with UTI were investigated with renal ultrasonography, intravenous pyelography (IVP) and voiding cystourethrography (VCU). Haemoglobin, white cell count, serum urea, creatinine and electrolytes were determined in all the children. The mean age of the children was 15.5 months (range 3-60 months). UTI was diagnosed in 26 (34.7%), of whom 21 (81%) were boys. The overall prevalence of UTI in those with kwashiorkor/marasmic kwashiorkor was 42%. Escherichia coli was the organism most commonly cultured (84.6%). Renal sonography, IVP and VCU were normal in all infected cases and vesicoureteric reflux was not detected in any. This study confirms the high prevalence of UTI in malnourished children. As no anatomical abnormalities were demonstrated in the patients with UTI, imaging of the renal tract other than real sonography does not appear to be indicated in the malnourished child in a first episode of UTI with normal renal function.

Management of severe hypertension in childhood Takayasu's arteritis.

Six children presented with severe hypertension caused by Takayasu's arteritis (TA), of whom four had bilateral renal artery narrowing and two coarctation syndrome. Two presented with hypertensive encephalopathy and four with congestive cardiac failure. All had a strongly positive skin reactions to purified protein derivative of mycobacterium tuberculosis. Bilateral renal arterial bypass grafts performed in two children resulted in prolonged normalization of their blood pressures, but the grafts clotted 12-18 months later. Primary renal autotransplantation was unsuccessful in two children, one with bilateral renal arterial narrowing and iliac vessel involvement and one with a long coarctation. Secondary renal autotransplantation was successful in a third child with localized aortitis. A successful aortic patch graft was performed in one child with coarctation of the aorta. Angiotensin-converting-enzyme inhibitors should be used with caution in treating the hypertension caused by TA, since bilateral renal arterial narrowing is common and their administration may result in renal insufficiency. The long-term prognosis is guarded in severely hypertensive children with extensive vascular disease due to TA.

Fulminant hepatitis in children: report of 12 cases.

Twelve cases of childhood fulminant hepatitis seen over a 4-year period are described. Six had hepatitis A, five hepatitis B and one non-A non-B hepatitis. Encephalopathy, the cardinal feature of fulminant hepatitis, was usually evident within 2 weeks of onset of illness, and the median duration of illness in fatal cases was 19 days. Deep jaundice, prolongation of the prothrombin time and raised serum ammonia were invariable. Eight children died and the four survivors were critically ill before recovering. Acute viral hepatitis is generally a benign illness in childhood. Although infrequently recorded, fulminant hepatitis may, however, ensue and is associated with a high mortality.