Cyclosporine in the treatment of childhood idiopathic steroid resistant nephrotic syndrome: a single centre experience in Nigeria.

INTRODUCTION: Children with steroid resistant nephrotic syndrome usually require treatment with second-line agents and calcineurin inhibitors such as cyclosporine are now recommended as initial therapy. These agents only recently become available in our environment and their impact on care is unknown. We reviewed the short-term treatment outcomes of their use in comparison with previous outcomes. METHODS: Medical records of children managed for idiopathic steroid resistant nephrotic syndrome over a 5 year period were reviewed. Remission rates and improvement in renal function following use of various agents were compared. RESULTS: Of 103 children with idiopathic nephrotic syndrome, 25(24.3%) were steroid resistant, of whom 17 received additional medications. Full remission rate for cyclosporine was 70% (7/10). Remission rates prior to the availability of cyclosporine were 40% (2/5) for cyclophosphamide and 66% (2/3), (partial remission only) with enalapril, an angiotensin converting enzyme inhibitor used in combination with alternate day prednisolone. One child with cyclophosphamide resistance subsequently achieved remission with cyclosporine. Remission was not related to sex (p=0.96), age (p=0.54), serum albumin (p=0.37) or hypertension (p=0.43) but to serum cholesterol (p= 0.02). The estimated glomerular filteration rate (eGFR) among children treated with cyclosporine ranged from 30-167 ml/min/1.73m2 as follows: >90 (5); 60-89 (3); 30-59 (2) while the mean pre and post treatment eGFR in those with eGFR <90 were 60 and 104ml/min/1.73m2 respectively (p=0.03). Mortality rate was 10% (1/10) in children treated with cyclosporine compared with 28.6% (2/7) in those treated with other medications (p=0.54). CONCLUSION: Cyclosporine resulted in improved treatment outcomes in children with idiopathic steroid resistant nephrotic syndrome.

Impact of revascularization on hypertension in children with Takayasu's arteritis-induced renal artery stenosis: a 21-year review.

BACKGROUND: Surgery for reno-vascular hypertension (RVH) is complex, and the techniques utilized vary with anatomical presentations of the disease. The long-term outcome of revascularization on RVH in children with Takayasu's arteritis (TA)-induced renal artery stenosis (RAS) at our centre was reviewed. METHODS: This study was a 21-year retrospective review of pre- and post-intervention RVH in children with angiographically confirmed RAS. The outcome of hypertension was defined as follows: (1) cured (normotensive off anti-hypertensives), (2) improved (normotensive on same or reduced number of medications), or (3) failure (no cure or improvement in number of medications). RESULTS: The medical histories of 59 children (median age 9.98 years) were reviewed, of whom 20 (44 %) had revascularization procedures. All were hypertensive, with a mean systolic and diastolic blood pressure of 161.5 ± 36 and 106.5 ± 31 mmHg, respectively. RAS was present in 45 (76.3 %) children. Twenty-four revascularization procedures were performed in 20 children (44 %), of whom five had contralateral nephrectomies. Outcome was available for 17 patients at the 3- and 6-months follow-up, with cure, improvement and failure rates at 3 months of 2/17 (11.8 %), 7/17 (41.2 %) and 8/19 (47 %), respectively, and similar rates at 6 months. Associations between outcome and age (p = 0.51), sex (p = 0.32), number of pre-surgery anti-hypertensives (p = 0.18) and stenosis sites (p = 0.22) were not statistically significant. CONCLUSIONS: Revascularization was beneficial to the management of blood pressure control in about half of our RVH patients.

Clinical profile and hospital outcome of children with severe acute kidney injury in a developing country.

INTRODUCTION: In resource-constraint regions of the world, the spectrum of childhood diseases is changing, creating a need to clearly define the epidemiology of severe acute kidney injury (AKI). METHODS: Medical records of children aged between 1 month and 17 years with stage 3 AKI in a tertiary hospital were reviewed. RESULTS: Ninety-one children, comprising 63 (69.2%) males and 26 (28.6%) infants, were studied. Majority (75.8%) had stage 3 AKI at the point of hospitalization. Sepsis (41.8%), primary kidney diseases (PKD; 29.7%) and malaria (13.2%) were the most common causes of stage 3 AKI. Twenty-eight (30.8%) children died. Mortality was highest in those with sepsis, less than 5 years old and needing dialysis. CONCLUSION: Sepsis, PKD and malaria were the most common causes of severe AKI. A third of children with severe AKI died. Mortality was highest in those less than 5 years old, with sepsis and needing dialysis.

High Steroid Sensitivity among Children with Nephrotic Syndrome in Southwestern Nigeria.

Recent reports from both Caucasian and black populations suggest changes in steroid responsiveness of childhood nephrotic syndrome. This study was therefore undertaken to determine the features and steroid sensitivity pattern of a cohort of black children with nephrotic syndrome. Records of children managed for nephrotic syndrome from January 2008 to April 2013 were reviewed. Details including age, response to treatment, and renal histology were analysed. There were 108 children (median age: 5.9 years, peak: 1-2 years), 90.2% of whom had idiopathic nephrotic syndrome. Steroid sensitivity was 82.8% among children with idiopathic nephrotic syndrome but 75.9% overall. Median time to remission was 7 days. Median age was significantly lower in steroid sensitive compared with resistant patients. The predominant histologic finding in resistant cases was focal segmental glomerulosclerosis (53.3%). No cases of quartan malaria nephropathy or hepatitis B virus nephropathy were diagnosed. Overall mortality was 6.5%. In conclusion, unusually high steroid sensitivity is reported among a cohort of black children. This is likely attributable to the lower age structure of our cohort as well as possible changing epidemiology of some other childhood diseases. Surveillance of the epidemiology of childhood nephrotic syndrome and corresponding modifications in practice are therefore recommended.

Peritoneal dialysis for children with acute kidney injury in Lagos, Nigeria: experience with adaptations.

UNLABELLED: ♦ INTRODUCTION: International professional groups are supporting the training of physicians in developing countries in nephrology, including peritoneal dialysis (PD). This report documents the impact of such efforts in the provision of manual PD for children with acute kidney injury (AKI) in a public hospital in Lagos, Nigeria. ♦ METHODS: Medical records of all children with AKI managed with PD between July 2010 and March 2013 were reviewed. ♦ RESULTS: Seventeen children with a mean (SEM) age of 3.8 (0.8) years received PD for 0 - 18 days; about half were infants. PD was provided manually, frequently with intercostal drains as catheters. Blockage of catheters and peritonitis occurred in 4 (23.5%) and 2 (11.8%) children, respectively. Eight (47.1%) of the 17 children had resolution of AKI and were discharged from hospital. In 4 (57.1%) of the 7 children who died, PD was performed for ≤ 2 days. ♦ CONCLUSION: PD for childhood AKI is feasible in resource-constrained regions with fairly good outcome. Blockage of catheter was the most common complication encountered.

Paediatric acute kidney injury in a tertiary hospital in Nigeria: prevalence, causes and mortality rate.

BACKGROUND: The modest decline in child mortality in Africa raises the question whether the pattern of diseases associated with acute kidney injury (AKI) in children in Nigeria has changed. METHODS: A database of children, aged between one month and 16 years, with AKI (using modified pediatric RIFLE criteria) was reviewed. The cause of AKI was defined as the major underlying disease. The clinical and laboratory features of children with AKI who survived were compared to those who died. RESULTS: Of the 4 015 children admitted into Lagos University Teaching Hospital between July 2010 and July 2012, 70 episodes of AKI were recorded equalling 17.4 cases per 1000 children. The median age of the children with AKI was 4.8 (range 0.1-14.4) years and 68.6% were males. Acute kidney injury was present in 58 (82.9%) children at admission with 70% in 'failure' category. Primary kidney disease (38.6%), sepsis (25.7%) and malaria (11.4%) were the commonest causes. The primary kidney diseases were acute glomerulonephritis (11) and nephrotic syndrome (8). Nineteen (28.4%) children with AKI died. Need for dialysis [odds ratio: 10.04 (2.94-34.33)], white cell >15 000/mm(3) [odds ratio: 5.72 (1.65-19.89)] and platelet <100 000/mm(3) [odds ratio: 9.56 (2.63-34.77)] were associated with death. CONCLUSION: Acute kidney injury is common in children admitted to hospitals. The common causes remain primary kidney diseases, sepsis and malaria but the contribution of sepsis is rising while malaria and gastroenteritis are declining. Acute kidney injury-related mortality remains high.