Childhood idiopathic steroid-resistant nephrotic syndrome at a Single Center in Khartoum.

Prevalence, clinicopathological features, and outcome of childhood idiopathic steroid-resistant nephrotic syndrome (ISRNS) vary in different countries. We report on these parameters in a single center in Khartoum. We retrospectively reviewed all the records of children with idiopathic nephrotic syndrome (INS) followed up in the pediatric renal unit, Soba Hospital, Khartoum between 2001 and 2012. ISRNS was defined as no remission within four weeks of daily prednisolone at a dose of 60 mg/m2. In 430 children with INS 130 (28%) had SRNS with a mean age of 7.7 ± 4.12 years. Males were 78 (60%). At presentation, hematuria was recorded in 57%, hypertension was recorded in 48%, and renal impairment in 15%. Histopathology showed focal segmental glomerulosclerosis in 40.8%, mesangioproliferative glomerulonephritis (22.3%), mesangiocapillary glomerulonephritis (16.9%), minimal change disease (MCD) (16.2%), and membranous glomerulonephritis (3.8%). Therapy included cyclosporine in 38.5%, additional therapy with cyclophosphamide, mycophenolate mofetil or tacrolimus in 20.8%, and steroids ± diuretics ± angiotensin converting enzyme (ACE) inhibitors in 40.7%. After 3.64 ± 2.84 years, 26.8% had complete remission (CR), 18.6% partial remission (PR), 26.8% were unremitting, 21.5% had chronic kidney disease (CKD), 1.6% died, and 4.6% were lost to follow-up. Non-MCD cases had significantly lower CR and higher CKD rates than MCD (P = 0.047 and 0.041, respectively). Cyclosporine ± additional therapy was significantly associated with higher rate of CR than steroids ± ACE inhibitors ± diuretics (P = 0.001), but the prevalence of CKD between the two groups was not significantly different (P = 0.604). Impaired renal function and hypertension at presentation were risk factors for CKD (P = 0.001 and 0.001, respectively). In Sudanese children with ISRNS, non-MCD lesions were the most common lesions. This pattern in addition to the lack of adequate therapy may explain the relatively lower CR and higher CKD rates. Impaired renal function and hypertension at presentation were risk factors for progression to CKD.

Prognosis of acute post-streptococcal glomerulonephritis in Sudanese children.

INTRODUCTION: Acute post streptococcal glomerulo-nephritis (APSGN) is a form of acute nephritic syndrome characterized by edema, hematuria, proteinuria, and hypertension. The immediate prognosis of acute post-streptococcal glomerulonephritis in children is usually excellent, however, the long-term prognosis has been a subject of debate. METHODS: This is a retrospective and prospective cohort study of Sudanese children with APSGN followed in a tertiary care hospital between 2006 and 2010. Patients who presented for follow-up 1-5 years after initial diagnosis were assessed for proteinuria, hematuria, urinary albumin to creatinine ratio (ACR), hypertension, and glomerular filtration rate (GFR). RESULTS: Data of 69 children (46 males; 66.7%) was analyzed. At presentation, 29% had severe acute disease requiring dialysis. On discharge, 60 children (87%) recovered their renal function, seven children (10.1%) showed no recovery and two children (2.9%) died. Forty out of 69 children presented to follow-up 1-5 years after initial diagnosis. Thirty-four of these children (85%) had normal blood pressure and GFR while six children (15%) progressed to chronic kidney disease (CKD); three of whom died. Among children with normal GFR, eight (23.5%) had microalbuminuria plus hematuria and four (11.7%) had hematuria. Persistence of proteinuria and/or hematuria was universal among the 14 children with normal GFR who continued follow-up more than three years after initial presentation. CONCLUSION: APSGN in this group of Sudanese children had a less favorable prognosis. This reflects the tertiary care set-up of this study. Persistence of hematuria and/or proteinuria was common on follow-up.

Primary vesicoureteral reflux in Sudanese children.

Vesicoureteral reflux (VUR) is a common congenital renal tract anomaly in children. Reports from Sudan are scanty. We report the characteristics, presentation and outcome of primary VUR in a tertiary care hospital. The records of 30 patients (16 males; 53%) followed-up between January 2004 and December 2010 were reviewed. The mean age at the time of diagnosis was 4 ± 3.9 years and 47% were <2 years of age. Renal ultrasound scan (USS) failed to predict VUR in 17% of the patients. On voiding cysturethrogram (VCUG), VUR was bilateral in 57% and severe grade in 64%. Grades were not significantly associated with age, gender or site of VUR. Initial dimercaptosuccinic acid radionuclide scan showed renal damage in 61.5% of the patients. Renal damage was significantly associated with female gender and severe VUR, but not with age of onset or history of urinary tract infection (UTI). Thirteen patients (43.3%) presented with acute UTI, eight (26.6%) with non-specific urinary tract symptoms and nine (30%) with persistently elevated serum creatinine. Urine cultures were positive in 73% of patients, and E. coli was the most common pathogen. Renal impairment at presentation was significantly associated with bilateral severe VUR and history of UTI but not age or gender. After a mean follow-up period of 1.78 years (6 months to 5 years), 70% of patients remained with normal renal function and 30% progressed to chronic kidney disease; two of them died. In conclusion, our data is different from many studies. Features of primary VUR in Sudanese children are late age of onset, equal gender affection and predominance of severe grade. Presentation is associated with a high rate of UTI, renal damage and advanced renal impairment. Measures to improve early detection and treatment of VUR may reduce the risk of kidney damage.

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.

Filicide and suicide in a family by paraphenylene diamine poisoning: a mother who committed suicide and poisoned her four children of which one died.

Paraphenylene diamine (PPD) hair dye poisoning is a common health problem in the Middle East and Indian subcontinent. It is the most common cause of suicide intent especially among females. We hereby present a 27-year-old female who presented with a clinical feature of PPD poisoning due to a suicidal attempt, and she died soon after arrival. She had survived a previous suicide attempt with PPD 4 years before. This time she also intentionally tried to kill her four children using PPD. One child died, one recovered after dialysis for acute kidney injury and the other two survived without any further intervention. This case illustrates that PPD poisoning may be used in filicide and suicide intent and that all patients who committed suicide should be reviewed and assessed by clinical psychiatrist.

Pattern and outcome of renal diseases in hospitalized children in Khartoum State, Sudan.

In developing countries, renal diseases in children constitute important causes of morbidity and mortality. In Sudan, data about patterns and outcome of these disorders is generally scanty. We conducted this study to provide basic renal data that may be utilized by researchers and health planners in a resource poor setting. A retrospective record review of all pediatric patients, followed in four teaching hospitals in Khartoum State over a five-year period (January 2000-June 2004), was achieved. In 150 hospitalized children a total of 200 renal diagnoses were recorded. Urinary tract infection (UTI), occurring with other underlying renal morbidities or isolated, was the commonest renal diagnosis (20%). The second common renal disorders were nephrotic syndrome (NS) and urolithiasis/stones accounting for 16% and 15.5% of cases, respectively. Acute glomerulonephritis (AGN) and congenital anomalies were relatively less common (12% and 10.5%, respectively). Other less frequently detected diseases were acute renal failure (ARF) in 6%, chronic renal failure (CRF) in 4%, hereditary nephropathies in 3.5% and renal tumors in 2.5%. There was a significant correlation between the pattern of renal diseases and age of patients (P =0.001) but not their gender or social class (P = 0.211 and 0.34, respectively). On follow up, 99 out of 150 patients (66%) recovered their normal renal function, 6/150 (4%) remained with persistent proteinuria, 30/150 (20%) progressed to CRF, 10/150 (6.7%) died, and 5/150 (3.3%) were referred to radiotherapy department for further management. Our data reflects geographical variations of patterns of renal diseases in Sudanese children as in other countries. Many of these diseases are preventable or potentially curable. Therefore, improvement of pediatric renal services and training of health workers would help in early detection and treatment of these conditions leading to reduction in their morbidity and mortality.

Paraphenylene diamine hair dye poisoning in an adolescent.

A 14-year-old female was referred to the pediatric nephrology unit with a short history of progressive angio-oedema of the face requiring tracheostomy. She ingested an unusual substance which caused swollen tongue, cervical oedema and dark coloured brown urine. These clinical features with the laboratory tests confirmed the diagnosis of paraphenylene diamine (PPD) poisoning. Psychological assessment confirmed that the child had severe depression. The suicide attempt was just immediately after failure in the final qualifying examination to secondary school. She did not have any family support but she used to be punished in different ways. In Sudan PPD in its pure form is available in the local market and until recently there was no restrictions for its use or trade. Adolescents are well aware of its toxic effects and it is commonly used to attempt suicide. Legislation and laws should prevent its use in different hair dye formulations and look for another substitute. Clinical management is hampered by the lack of a rapid diagnostic test; no other treatment other than supportive measures can be offered.

Pattern of glomerular diseases in Sudanese children: a clinico-pathological study.

Glomerular diseases are a common cause of chronic kidney disease (CKD) in many countries. The pattern of glomerular diseases has been reported in adult Sudanese patients but there has been no previous study on Sudanese children. The aim of this study is to describe the pattern of glomerular diseases in Sudanese children from a clinico-pathological perspective. We retrospectively reviewed the clinical records of 321 children seen with nephritis/nephrosis at the Pediatric Nephrology Unit, Soba University Hospital and Dr. Salma Dialysis and Kidney Transplantation Centre, Khartoum, Sudan during the period from 2002 to 2007. Biopsies were studied with light microscopy and immuno-histochemistry with electron microscopy performed abroad in selected patients (predominantly Alport's). The mean age of the 321 study children was 8.71 years (range 2 months-16 yrs) of whom, 188 were males (60.2%). The most common presentation was with the nephrotic syndrome, seen in 202 patients (62.9%). The most common glomerular disease encountered was minimal change disease, seen in 96 children (29.9%), followed by post-infectious GN in 78 (24.3%) and focal and segmental glomerulosclerosis, seen in 44 patients (13.7%). Membranoproliferative GN (MPGN) was seen in 43 patients (13.4%) while mesangioproliferative GN was seen in 24 (7.5%). Systemic lupus erythematosus (SLE) was the most common secondary glomerular disease accounting for 16 patients (4.9%). HBsAg was positive in 10 patients and the most common associated lesion was MPGN (60%). Histopathology enabled us to change the therapy in 55.3% of the patients. Our study suggests that the pattern of GN in our cohort of patients is comparable with reports from other parts of the world with a high prevalence of post-infectious GN. Renal biopsies have an important part in planning therapy and management. Also, the importance of establishing a Sudanese renal registry including pediatric patients is stressed.

Chronic renal failure in Sudanese children: aetiology and outcomes.

The provision of tertiary paediatric nephrology facilities has led to increasing referrals of children with chronic renal failure (CRF). We report the aetiology and outcomes over 5 years, during which period the improving socio-economic situation has allowed increasing provision of dialysis and transplantation. The records of 205 children (124 male; 60.5%) who were referred to a tertiary paediatric nephrology unit in Khartoum between 2001 and 2006 with a glomerular filtration rate of less than 50 ml/min per 1.73 m(2) body surface area were reviewed. The mean age at the time of diagnosis with CRF was 9.8 years (range 3 months-17 years). The aetiology was chronic glomerulonephritis in 52 (25.4%), congenital urological malformations in 36 (17.5%), urolithiasis in 19 (9.3%), hereditary nephropathy in 14 (6.8%), multisystem diseases in 4 (2%), and cause unknown in 80 (39.1%). Of the 205 children, 136 (63%) had reached end-stage renal failure, with chronic haemodialysis being undertaken in 48 (35.3%), intermittent peritoneal dialysis in 43 (31.6%), continuous ambulatory peritoneal dialysis in 17 (14.7%), and no treatment in 25 (18.4%). At the end of the study period 53 of the 205 (25.9%) remained on dialysis, 51 (25%) were on conservative treatment, 8 (3.9%) had received transplants, 48 (23.4%) had died, and 45 (22%) had been lost to follow-up. The results illustrate the geographical variations in CRF aetiology in different countries, which may be influenced by current patterns of referral as well as environmental and society factors. The large number with an unknown aetiology for their CRF requires further prospective investigation. We hope the current high mortality rate will improve with earlier referral, improved nutrition, family support, and better access to treatment modalities, especially the provision of kidney transplantation from living related donors.