Diabetes Management in Chronic Kidney Disease: Synopsis of the KDIGO 2022 Clinical Practice Guideline Update.

DESCRIPTION: The KDIGO 2022 Clinical Practice Guideline for Diabetes Management in Chronic Kidney Disease is an update of the 2020 guideline from Kidney Disease: Improving Global Outcomes (KDIGO). METHODS: The KDIGO Work Group updated the guideline, which included reviewing and grading new evidence that was identified and summarized. As in the previous guideline, the Work Group used the GRADE (Grading of Recommendations Assessment, Development and Evaluation) approach to appraise evidence and rate the strength of recommendations and expert judgment to develop consensus practice points. New evidence led to updating of recommendations in the chapters Comprehensive Care in Patients With Diabetes and CKD (Chapter 1) and Glucose-Lowering Therapies in Patients With T2D and CKD (Chapter 4). New evidence did not change recommendations in the chapters Glycemic Monitoring and Targets in Patients With Diabetes and CKD (Chapter 2), Lifestyle Interventions in Patients With Diabetes and CKD (Chapter 3), and Approaches to Management of Patients With Diabetes and CKD (Chapter 5). RECOMMENDATIONS: The updated guideline includes 13 recommendations and 52 practice points for clinicians caring for patients with diabetes and chronic kidney disease (CKD). A focus on preserving kidney function and maintaining well-being is recommended using a layered approach to care, starting with a foundation of lifestyle interventions, self-management, and first-line pharmacotherapy (such as sodium-glucose cotransporter-2 inhibitors) demonstrated to improve clinical outcomes. To this are added additional drugs with heart and kidney protection, such as glucagon-like peptide-1 receptor agonists and nonsteroidal mineralocorticoid receptor antagonists, and interventions to control risk factors for CKD progression and cardiovascular events, such as blood pressure, glycemia, and lipids.

Executive summary of the KDIGO 2022 Clinical Practice Guideline for Diabetes Management in Chronic Kidney Disease: an update based on rapidly emerging new evidence.

The Kidney Disease: Improving Global Outcomes (KDIGO) 2022 Clinical Practice Guideline for Diabetes Management in Chronic Kidney Disease (CKD) represents a focused update of the KDIGO 2020 guideline on the topic. The guideline targets a broad audience of clinicians treating people with diabetes and CKD. Topic areas for which recommendations are updated based on new evidence include Chapter 1: Comprehensive care in patients with diabetes and CKD and Chapter 4: Glucose-lowering therapies in patients with type 2 diabetes (T2D) and CKD. The content of previous chapters on Glycemic monitoring and targets in patients with diabetes and CKD (Chapter 2), Lifestyle interventions in patients with diabetes and CKD (Chapter 3), and Approaches to management of patients with diabetes and CKD (Chapter 5) has been deemed current and was not changed. This guideline update was developed according to an explicit process of evidence review and appraisal. Treatment approaches and guideline recommendations are based on systematic reviews of relevant studies and appraisal of the quality of the evidence, and the strength of recommendations followed the "Grading of Recommendations Assessment, Development and Evaluation" (GRADE) approach. Limitations of the evidence are discussed, and areas for which additional research is needed are presented.

Sickle cell nephropathy: insights into the pediatric population.

The life expectancy of individuals with sickle cell disease has increased over the years, majorly due to an overall improvement in diagnosis and medical care. Nevertheless, this improved longevity has resulted in an increased prevalence of chronic complications such as sickle cell nephropathy (SCN), which poses a challenge to the medical care of the patient, shortening the lifespan of patients by 20-30 years. Clinical presentation of SCN is age-dependent, with kidney dysfunction slowly beginning to develop from childhood, progressing to chronic kidney disease and kidney failure during the third and fourth decades of life. This review explores the epidemiology, pathology, pathophysiology, clinical presentation, and management of SCN by focusing on the pediatric population. It also discusses the factors that can modify SCN susceptibility.

Diabetes Management in Chronic Kidney Disease: Synopsis of the 2020 KDIGO Clinical Practice Guideline.

DESCRIPTION: The Kidney Disease: Improving Global Outcomes (KDIGO) organization developed a clinical practice guideline in 2020 for the management of patients with diabetes and chronic kidney disease (CKD). METHODS: The KDIGO Work Group (WG) was tasked with developing the guideline for diabetes management in CKD. It defined the scope of the guideline, gathered evidence, determined systematic review topics, and graded evidence that had been summarized by an evidence review team. The English-language literature searches, which were initially done through October 2018, were updated in February 2020. The WG used the GRADE (Grading of Recommendations Assessment, Development and Evaluation) approach to appraise evidence and rate the strength of the recommendations. Expert judgment was used to develop consensus practice points supplementary to the evidence-based graded recommendations. The guideline document underwent open public review. Comments from various stakeholders, subject matter experts, and industry and national organizations were considered before the document was finalized. RECOMMENDATIONS: The guideline includes 12 recommendations and 48 practice points for clinicians caring for patients with diabetes and CKD. This synopsis focuses on the key recommendations pertinent to the following issues: comprehensive care needs, glycemic monitoring and targets, lifestyle interventions, antihyperglycemic therapies, and educational and integrated care approaches.

Executive summary of the 2020 KDIGO Diabetes Management in CKD Guideline: evidence-based advances in monitoring and treatment.

THE KIDNEY DISEASE: Improving Global Outcomes (KDIGO) Clinical Practice Guideline for Diabetes Management in Chronic Kidney Disease represents the first KDIGO guideline on this subject. The guideline comes at a time when advances in diabetes technology and therapeutics offer new options to manage the large population of patients with diabetes and chronic kidney disease (CKD) at high risk of poor health outcomes. An enlarging base of high-quality evidence from randomized clinical trials is available to evaluate important new treatments offering organ protection, such as sodium-glucose cotransporter-2 inhibitors and glucagon-like peptide-1 receptor agonists. The goal of the new guideline is to provide evidence-based recommendations to optimize the clinical care of people with diabetes and CKD by integrating new options with existing management strategies. In addition, the guideline contains practice points to facilitate implementation when insufficient data are available to make well-justified recommendations or when additional guidance may be useful for clinical application. The guideline covers comprehensive care of patients with diabetes and CKD, glycemic monitoring and targets, lifestyle interventions, antihyperglycemic therapies, and self-management and health systems approaches to management of patients with diabetes and CKD.

Acute kidney injury in children in Nigeria.

AIM: To highlight the epidemiologic characteristics and therapeutic challenges of childhood acute kidney injury (AKI) in Nigeria. METHOD: A review of AKI publications on Nigerian children between January 1990 and December 2012 was carried out. RESULTS: Mean age at presentation varied between 3.1 ± 2.6 and 6.28 ± 4 (0.05 - 16) years. Male/female ratio ranged between 1.38 and 2.5 to 1. Hospital-acquired AKI (hAKI) and community-acquired AKI (cAKI) accounted for 17.1 - 27.2% and 72.8 - 82.9% of all AKI cases, respectively. 12 - 35 new AKI cases are seen per year. A single-center study puts cAKI and hAKI incidences at 9.8 per million children population (pmcp)/year (0.46%) and 3.7 pmcp/ year (0.17%), respectively; cAKI and hAKI prevalence rates were 49.2 pmcp (2.23%) and 18.3 pmcp (0.84%), respectively. Leading causes of AKI, accounting for 80.0% of all etiologies, were nephrotoxins (29.0%), infection (20.0%), intravascular volume depletion (17.9%), and glomerular disease (13.1%). Financial constraints, late presentation, presence of ≥ 2 comorbidities, need for dialysis, non-dialysis when indicated, severe hypertension, white cell count > 15 000/mm3, and platelet count < 100 000/mm3 are significant mortality risk factors in childhood AKI in our environment. Mean all-cause mortality rate from pooled data was 50.4 ± 25.2% (range: 28.4 - 86.5%). CONCLUSION: AKI incidence and its leading causes, in Nigerian children, can be significantly reduced if attention is paid to public health education, enforcement of environmental sanitation laws, and prompt utilization of healthcare services during sickness.

Childhood Nephrotic Syndrome in Africa: Epidemiology, Treatment Trends, and Outcomes.

Nephrotic syndrome is a common childhood glomerular disease that is associated with massive proteinuria and edema. Children with nephrotic syndrome are at risk of chronic kidney disease, disease-related complications, and treatment-related complications. Patients with frequently relapsing disease or steroid toxicity may require newer immunosuppressive medications. However, access to these medications is limited in many African countries owing to prohibitive cost, the need for frequent therapeutic drug monitoring, and a lack of appropriate facilities. This narrative review examines the epidemiology of childhood nephrotic syndrome in Africa, including trends in treatment and patient outcomes. In most of North Africa, as well as among White and Indian populations in South Africa, the epidemiology and treatment of childhood nephrotic syndrome closely resembles that of European and North American populations. Historically, secondary causes of nephrotic syndrome (eg, quartan malaria nephropathy and hepatitis B-associated nephropathy) were predominant among Blacks in Africa. Over time, the proportion of secondary cases has decreased, along with rates of steroid resistance. However, focal segmental glomerulosclerosis increasingly has been reported among patients with steroid resistance. There is a need for consensus guidelines for the management of childhood nephrotic syndrome in Africa. Furthermore, establishing an African nephrotic syndrome registry could facilitate monitoring of disease and treatment trends, and provide opportunities for advocacy and research to improve patient outcomes.

Quartan malaria-associated childhood nephrotic syndrome: now a rare clinical entity in malaria endemic Nigeria.

BACKGROUND: The study determined (i) whether or not quartan malaria nephropathy (QMN) is still a major cause of childhood nephrotic syndrome (CNS) in Nigeria, (ii) secondary causes other than QMN and their associated glomerular pathology and (iii) renal and patient outcome. METHODS: The study was a prospective non-randomized study of consecutive cases of secondary CNS. Patients with idiopathic CNS were excluded. RESULTS: Twenty-four of 78 (30.8%) CNS cases were of secondary aetiology. Overall mean ages at onset of secondary CNS aetiology and CNS onset were 8.97 +/- 3.59 (1-15.3) and 9.95 +/- 3.15 (5-15.3) years, respectively. Male (14)/female (10) ratio was 1.4. Secondary causes comprised systemic lupus erythematosus (SLE, 37.5%), sickle cell anaemia (SCA, 16.7%), hepatitis B virus (HBV, 16.7%) infection, Churg-Strauss syndrome (12.6%), SLE/human immunodeficiency virus infection (4.2%), rhabdomyosarcoma (4.2%), bee stings (4.2%) and Addison's disease (4.2%). The overall cumulative complete remission (CR) rate was 88.0%. Remission was sustained in 11 of 16 (68.8%) CR patients, while one patient (6.25%) relapsed; the remaining four patients (24.95%) were yet to attain sustained remission. Median relapse-free period was 10.5 (0.75-25) months. Cumulative renal survival was 75.2% at 3 years. Three patients were lost to follow-up, while two died. Overall cumulative patient survival probability at 36 months was 90.8%. All patients were followed for a median period of 12.5 (0.11-36.0) months. CONCLUSION: Overall outcome of CNS has improved significantly compared to the 1960s and 1970s when the poor outcome of QMN was the predominant glomerular lesion in Nigeria. While quartan malaria-associated nephrotic syndrome has become a rare clinical entity, SLE, SCA and HBV infection have become the major secondary aetiologies of CNS in Nigeria.

Nephropathy, polyneuropathy, and gastroenteritis in a child with Churg-Strauss syndrome.

Churg-Strauss syndrome (CSS) is a serious but rare pauci-immune vasculitis of small- and medium-sized blood vessels. It is commonly seen in association with bronchial asthma and/or allergic disorders. The syndrome is characterized by the presence of asthma, hypereosinophilia, and vasculitis in any part of the body. Vasculitis is often associated with significant distortion of normal functions. A rather severe case of CSS in an 8-year-old Nigerian girl with asthma and allergic rhinoconjunctivitis is reported. She presented with multiple morbidities, namely, vasculitic polyneuropathy and also nephritic-nephrotic syndrome that eventuated in acute renal failure after an onset of vasculitic gastroenteritis. Routine screening of all asthmatic patients for CSS is advocated.

Childhood nephrotic syndrome in tropical Africa: then and now.

This descriptive and comparative review examines the changing epidemiology, treatment, renal and patient outcome of childhood nephrotic syndrome (NS) in tropical Africa (TpAfr). In the 1960s to 1980s, corticosteroid-resistant non-minimal change disease (nMCD) including quartan malaria nephropathy (QMN) was the dominant renal histopathology type. The overall incidence of NS was 0.35-1.34% of hospital admissions. Median age at onset of NS ranged between 4.0 and 12.0 years while the mean (SD) age range was 5.8 (3.8) to 10.3 (4.8) years across studies. The male: female ratio was 1.6:1.0. The overall mean (SD) incidence of idiopathic minimal change disease (MCD) [21.6 (18.6%)] compared with idiopathic nMCD [59.1 (25.7%)] demonstrates significant dominance of the latter (p = 0.0001). Post-1989, the following mean (SD) incidences of histopathological types were: MCD 20.4 (17.7%), focal segmental glomerulosclerosis 39.0 (26.3%), membranoproliferative glomerulonephritis 25.4 (16.8%), proliferative glomerulonephritis 16.7 (27.0%) and membranous nephropathy 7.4 (4.5%). While the mean (SD) proportion of steroid resistance (SR) [73.5 (19.2%)] was significantly greater than the mean complete remission (CR) [26.5 (19.2%)] during 1960-1989 (p=0.005), mean (SD) SR [27.4 (25.3%)] was significantly lower than mean (SD) CR [66.1 (28.0%)] post-1989 (p < 0.001). Unlike QMN, hepatitis B virus, HIV infection, sickle cell disease and systemic lupus erythematosus are now increasingly being associated with NS in TpAfr. Mean (SD) renal survival post-1989 was 58.3 (37.0%) while all-cause mortality was 9.8%. Children with NS now survive better than before, reflecting improved access to healthcare and transition to a clinical pattern favouring idiopathic NS and increased sensitivity to corticosteroids.

Outcomes in adults and children with end-stage kidney disease requiring dialysis in sub-Saharan Africa: a systematic review.

BACKGROUND: The burden of end-stage kidney disease (ESKD) in sub-Saharan Africa is unknown but is probably high. Access to dialysis for ESKD is limited by insufficient infrastructure and catastrophic out-of-pocket costs. Most patients remain undiagnosed, untreated, and die. We did a systematic literature review to assess outcomes of patients who reach dialysis and the quality of dialysis received. METHODS: We searched PubMed, African Journals Online, WHO Global Health Library, and Web of Science for articles in English or French from sub-Saharan Africa reporting dialysis outcomes in patients with ESKD published between Jan 1, 1990, and Dec 22, 2015. No studies were excluded to best represent the current situation in sub-Saharan Africa. Outcomes of interest included access to dialysis, mortality, duration of dialysis, and markers of dialysis quality in patients with ESKD. Data were analysed descriptively and reported using narrative synthesis. FINDINGS: Studies were all of medium to low quality. We identified 4339 studies, 68 of which met inclusion criteria, comprising 24 456 adults and 809 children. In the pooled analysis, 390 (96%) of 406 adults and 133 (95%) of 140 children who could not access dialysis died or were presumed to have died. Among those dialysed, 2747 (88%) of 3122 adults in incident ESKD cohorts, 496 (16%) of 3197 adults in prevalent ESKD cohorts, and 107 (36%) of 294 children with ESKD died or were presumed to have died. 2508 (84%) of 2990 adults in incident ESKD cohorts discontinued dialysis compared with 64 (5%) of 1364 adults in prevalent ESKD cohorts. 41 (1%) of 4483 adults in incident ESKD cohorts, 2280 (19%) of 12 125 adults in prevalent ESKD cohorts, and 71 (19%) of 381 children with ESKD received transplants. 16 studies reported on management of anaemia, 17 on dialysis frequency, eight on dialysis accuracy, and 22 on vascular access for dialysis INTERPRETATION: Most patients with ESKD starting dialysis in sub-Saharan Africa discontinue treatment and die. Further work is needed to develop equitable and sustainable strategies to manage individuals with ESKD in sub-Saharan Africa. FUNDING: None.

Burden of Hypertension and Abnormal Glomerular Permeability in Hypertensive School Children.

BACKGROUND: Childhood hypertension has been associated with target-organ damage in young adults. It is often asymptomatic in both children and adolescents; when persistent, and long-standing, it could be a significant risk factor for kidney damage and increased glomerular permeability. OBJECTIVES: Burden of hypertension and its impact on glomerular permeability were prospectively determined in randomly recruited primary school children. PATIENTS AND METHODS: Blood pressure (BP) measurement was performed by the auscultation method, and abnormal glomerular permeability was assessed by dipstick testing of urine for persistent proteinuria and/or hematuria for ≥ three months in hypertensive children. RESULTS: Of 1,335 pupils aged 10.0 ± 2.4 (6.0 - 14.0) years, 33 (2.5%) were hypertensive. Overall mean systolic/diastolic BP was 125.6 ± 6.5/81.7 ± 3.3 (range: 114.0 - 140.0/80.0 - 90.0) mmHg. Nine (27.3%) had combined systolic and diastolic hypertension, 126.7 ± 5.7/80.0 - 80.0 ± 0.0 (120.0 - 130.0/80.0 - 80.0) mmHg. Isolated systolic hypertension, 125.4 ± 6.7 (114.0 - 140.0) mmHg, was present in 14 (42.4%), whereas 10 (30.3%) had isolated diastolic hypertension, 82.0 ± 3.5 (80.0 - 90.0) mmHg. Mean systolic and diastolic BP were 131.0 ± 3.3 (130.0 - 140.0) mmHg and 86.5 ± 4.43 (80.0 - 90.0) mmHg, respectively. According to the dipstick test, none of the hypertensive pupils showed urinalysis evidence of proteinuria and/or hematuria after three months of testing. CONCLUSIONS: Although the burden of hypertension was 2.5%, the dipstick method did not detect any hypertension-related abnormal glomerular permeability in the school children.

Outcomes of acute kidney injury in children and adults in sub-Saharan Africa: a systematic review.

BACKGROUND: Access to diagnosis and dialysis for acute kidney injury can be life-saving, but can be prohibitively expensive in low-income settings. The burden of acute kidney injury in sub-Saharan Africa is presumably high but remains unknown. We did a systematic review to assess outcomes of acute kidney injury in sub-Saharan Africa and identify barriers to care. METHODS: We searched PubMed, African Journals Online, WHO Global Health Library, and Web of Science for articles published between Jan 1, 1990, and Nov 30, 2014. We scored studies, and all were of medium-to-low quality. We made a pragmatic decision to include all studies to best reflect reality, and did a descriptive analysis of extracted data. This study is registered with PROSPERO, number CRD42015015690. FINDINGS: We identified 3881 records, of which 41 met inclusion criteria, including 1403 adult patients and 1937 paediatric patients. Acute kidney injury in sub-Saharan Africa is severe, with 1042 (66%) of 1572 children and 178 (70%) 253 of adults needing dialysis in studies reporting dialysis need. Only 666 (64%) of 1042 children (across 11 studies) and 58 (33%) of 178 adults (across four studies) received dialysis when needed. Overall mortality was 34% in children and 32% in adults, but rose to 73% in children and 86% in adults when dialysis was needed but not received. Major barriers to access to care were out-of-pocket costs, erratic hospital resources, late presentation, and female sex. INTERPRETATION: Patients in these studies are those with resources to access care. In view of overall study quality, data interpretation should be cautious, but high mortality and poor access to dialysis are concerning. The global scarcity of resources among patients and health centres highlights the need for a health-system-wide approach to prevention and management of acute kidney injury in sub-Saharan Africa. FUNDING: None.

Epidemiology, pathophysiology, clinical characteristics and management of childhood cardiorenal syndrome.

Cardiorenal syndrome (CRS) is a new term recently introduced to describe the acute or chronic comorbid state of the heart and kidney that has been long known and frequently managed in very sick individuals. The tight and delicate coordination of physiological functions among organ systems in the human body makes dysfunction in one to lead to malfunction of one or more other organ systems. CRS is a universal very common morbidity in the critically ill, with a high mortality rate that has received very little research attention in children. Simultaneous management of heart and renal failures in CRS is quite challenging; the therapeutic choice made for one organ must not jeopardize the other. This paper reviews the epidemiology, pathophysiology, clinical characteristics and management of acute and chronic CRS in children.

Aminophylline improves urine flow rates but not survival in childhood oliguric/anuric acute kidney injury.

INTRODUCTION: Acute kidney injury (AKI) morbidity and mortality rates remain high. Variable AKI outcomes have been reported in association with aminophylline treatment. This study evaluated AKI outcome in a group of Nigerian children treated with aminophylline. METHODS: This is a retrospective study of AKI in children treated with (N=9) and without (N=8) aminophylline. Studied outcome indices comprised urine flow rate (UFR), duration of oliguria/anuria, progression through AKI stages, number of patients requiring dialysis and mortality. RESULTS: Mean ages for the control and aminophylline arms were 4.6±2.7 and 4.9±2.1 years (P=0.7), respectively. All patients progressed to stage-3 AKI. Baseline median UFRs in the aminophylline and control arms were similar (0.13 Vs 0.04 ml/kg/hour respectively, P=0.5). The median UFR was significantly higher on day-5 (0.8 Vs 0.1; P=0.03), day-6 (1.0 Vs 0.2; P=0.02), and day-7 (1.2 Vs 0.2; P=0.03) in the aminophylline than the control arm, respectively. Short duration of oliguria/anuria (≤ 6 days) was more frequently observed in aminophylline- treated patients compared to controls (77.8% Vs 25.0%; odds ratio 0.09; 95% CI: 0.01-0.89; P=0.04). Only the aminophylline group maintained steady serum creatinine levels. Four out of five patients in the control group were dialyzed compared to only one out of eight patients in the aminophylline group (odds ratio 0.16; 95% CI: 0.04-0.71; P=0.03). Mortality rates were similar in aminophylline- treated and control patients (33% Vs 25%; hazard ratio 0.8; 95% CI: 0.1-5.5; P=0.8). CONCLUSION: Aminophylline therapy was beneficial for patients with AKI in terms of improved UFR and reduced need for dialysis, but failed to impact positively on survival.

Acute childhood cardiorenal syndrome and impact of cardiovascular morbidity on survival.

Cardiorenal syndrome (CRS) clinical types, prevalence, aetiology, and acute cardiovascular morbidity impact on the outcome of acute kidney function perturbation were determined. Forty-seven of 101 (46.53%) patients with perturbed kidney function had CRS. Types 3 and 5 CRS were found in 10 and 37 patients, respectively. Type 3 CRS was due to acute glomerulonephritis (AGN; n = 7), captopril (n = 1), frusemide (n = 1), and hypovolaemia (n = 1). Malaria-associated haemoglobinuria (n = 20), septicaemia (n = 11), lupus nephritis (n = 3), tumour lysis syndrome (n = 2), and acute lymphoblastic leukaemia (n = 1) caused Type 5 CRS. The cumulative mortality in hypertensive CRS was similar to nonhypertensive CRS (51.4% versus 40.9%; P = .119). Mortality in CRS and non-CRS was similar (45.7% versus 24.5%; P = .053). Type 5 survived better than type 3 CRS (66.7% versus 12.5%; P = .001). Risk factors for mortality were Type 3 CRS (P = .001), AGN-associated CRS (P = .023), dialysis requiring CRS (P = .008), and heart failure due to causes other than anaemia (P = .003). All-cause-mortality was 34.2%. Preventive measures aimed at the preventable CRS aetiologies might be critical to reducing its prevalence.

Childhood idiopathic steroid resistant nephrotic syndrome in Southwestern Nigeria.

Clinical charts of 23 Nigerian children diagnosed with idiopathic steroid resistant nephrotic syndrome (iSRNS) between January 2001 and December 2007 were retrospectively reviewed to determine their clinicopathologic characteristics and outcome. iSRNS (54.8%) was primary in 19 patients (83%) and secondary in four (17%). The mean age at diagnosis was 8.3 ± 3.5 years (2.1-13 years). Histopathology revealed membranoproliferative glomerulonephritis (MPGN) in 43.5%, focal and segmental glomerulosclerosis (FSGS) in 39.1% and mesangial proliferative glomerulonephritis in 8.7% of the patients while minimal change disease (MCD) and membranous nephropathy accounted for 4.35% each. Routine treatment protocol comprised pulse intravenous (i.v.) cylophosphamide infusion and i.v. dexamethasone lisinopril or spironolactone. Cumulative Complete Remission (CR) rate was 57.12%. The overall median time to CR from start of steroid sparing agents in 12/21 treated patients was 4.5 weeks. CR was better achieved in MPGN than FSGS (P = 0.0186). Five patients had eight relapses with the overall median relapse-free duration being four months. Cumulative renal survival at 36 months was 41.8%. The median follow-up duration was eight months. Our study revealed that there was a high prevalence of iSRNS and preponderance of non-MCD lesions, with MPGN and FSGS being the major morphologic lesions. The outcome with steroid and cyclophosphamide-based treatment for iSRNS was further enhanced with addition of either lisinopril or spironolactone.

Clinicopathology of childhood-onset renal systemic lupus erythematosus.

AIMS: To determine the clinicolaboratory renal manifestations; glomerular, extra-glomerular histopathologic lesions; renal tubular dysfunction (RTD) frequency and outcome of a short-term renal follow up in Nigerian children with systemic lupus erythematosus (SLE). METHODS: A non-randomized prospective study of consecutive cases of childhood-onset SLE with nephropathy was conducted. Baseline/follow-up clinicolaboratory data were collected. Each patient was followed up for 12 months. RESULTS: Seven of the 11 children studied were girls. The median age at diagnosis was 11.0 years. Median diagnosis time interval (1.9 years) and median time of renal disease onset (1.0 year) were similar. Hypertension, nephrotic syndrome and acute renal failure (ARF) occurred in 45.5%, 54.5% and 63.7% of the patients, respectively. The glomerular lesions were non-proliferative lupus nephritis (LN) in 9.0% (class II LN); focal (class III LN) and diffuse (class IV LN) proliferative LN (PLN) in 27.0% and 64.0%, respectively. Tubulointerstitial nephritis (TIN, 91.0%) and RTD (64.0%) were common. ARF (P = 0.033) and RTD (P = 0.015) were significantly associated with severe TIN. Complete renal remission rate at end-point was 71.4%. Relapse and renal survival rates were 14.3% and 86.0%, respectively. RTD was persistent in 43.0%. CONCLUSION: Renal function disorders, diffuse PLN and extra-glomerular lesions were frequent. Significant association of ARF and RTD with severe TIN in this series suggests the need for early renal tubular function (RTF) assessment in our SLE patients. Deranged RTF may be marker of severe TIN in SLE warranting early confirmatory renal biopsy and aggressive interventional treatment.

Reversible renal failure in hypertensive idiopathic nephrotics treated with captopril.

Angiotensin converting enzyme inhibitor (ACEI)- induced acute renal failure (ARF) is not as commonly reported in children as in adults. We report two cases of idiopathic nephrotic syndrome that developed ARF following captopril (an ACEI) treatment for prednisolone-induced hypertension. The two cases further alert us to the potential risk of ACEI-induced ARF in any nephrotic child on ACEI treatment. Low or high dose ACEIs should be given with extreme caution in active nephrotics in view of their relative hypovolemic state that may provoke ARF. The nephrotic children, who must be treated with ACEIs with or without diuretics, should be closely monitored for the development of ARF during the use of ACEIs.

Acute renal failure in African children with Burkitt's lymphoma: a comparison of two treatment regimens.

BACKGROUND: The outcome for patients presenting with acute renal failure and Burkitt lymphoma (BLARF) without dialysis is poor. This was a retrospective non-randomized comparative study designed to determine the outcome of two different treatment protocols. METHODS: One group of patients (TPA) received oral allopurinol, intravenous (IV) cyclophosphamide, vincristine, methotrexate, furosemide, 8.4% sodium bicarbonate, and intrathecal (IT) methotrexate; the other (TPB) alternate day IV infusion of low dose cyclosphosphamide (125 mg/m(2) x 4 doses), IT methotrexate (Days 1 and 5) and aggressive pre-emptive anti-tumor lysis syndrome therapy including oral allopurinol and calcium lactate, IV calcium gluconate, salbutamol, insulin and infusions of furosemide, sodium bicarbonate and glucose. RESULTS: Nine of 16 received TPA, 7 received TPB. Post chemotherapy anemia was more severe with TPA (P < 0.05). TPB patients received significantly more chemotherapy than those in TPA (P = 0.04). All 16 had tumor lysis syndrome (TLS). Six of nine patients with TPA died from this (three from other causes), two deaths in TPB were due to causes other than tumor lysis. Other evaluated outcome indices were similar in both groups. CONCLUSION: Slow IV infusion of low dose cyclophosphamide given on alternate days in addition to pre-emptive anti-TLS measures (TPB) were associated with better outcome in BLARF patients compared to a high dose multiple chemotherapy regimen (TPA).