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Objective: To compare the efficacy of tacrolimus versus cyclosporine (Calcineurin Inhibitors) in the management of childhood steroid-resistant nephritic syndrome (SRNS)., Study Design: Quasi-experimental study., Place and Duration of Study: Department of Paediatric Nephrology at The Children's Hospital and Institute of Child Health, Lahore, from August 2014 to September 2015., Methodology: Patients of either gender aged 1 - 12 years, with the diagnosis of mesangioproliferative glomerulonephritis (MesangioPGN), focal segmental glomerulosclerosis (FSGS) or minimal-change disease (MCD) were included. Patients were assigned into two groups, one given tacrolimus in dose of 0.1 - 0.2 mg/kg/day in two divided doses, and other given cyclosporine in dose of 150 - 200 mg/m2/day in two divided doses along with oral steroids 30 mg/m2/day in divided doses, followed by alternate day with tapering dosage. Trough drug levels were done with dose adjustment accordingly. Patients were monitored and followed for the response to treatment and adverse effects of these two calcineurin inhibitors., Results: Atotal of 84 patients, 58% males and 42% females, were included in the study. The age ranged from 1.25 to 12 years. The most common histopathological diagnosis was MesangioPGN (69.04%), FSGS (21.4%), and MCD (9.52%). Complete response was seen in 80.95% and 97.6% patients treated with cyclosporine and tacrolimus, respectively. Partial response was in 19.05% patients treated with cyclosporine and 2.4% in patients with tacrolimus. The most common adverse effect with cyclosporine and tacrolimus was hypertrichosis in 80.95% and 2.38%, hypertension 16.66% and 11.9% respectively while gum hypertrophy with cyclosporine was seen in 26.19% patients., Conclusion: Tacrolimus was more efficacious than cyclosporine in achieving remission in childhood SRNS with insignificant adverse effects.

Gitelman's syndrome is a hereditary disorder occurring due to loss of functional mutations of the gene encoding the distal convoluted tubule sodium chloride cotransporter (NCCT) and is characterized by hypokalemic metabolic alkalosis, hypomagnesemia and hypocalciuria. This case reports an adolescent girl presenting with episodes of carpopedal spasms and difficulty in walking with laboratory tests suggestive of Gitelman's syndrome along with hypophosphatemia.

Galloway-Mowat syndrome is a rare multisystem genetic disorder with constellation of neurological, skeletal, growth, facial, gastrointestinal and renal abnormalities. This case report describes Galloway-Mowat syndrome in a young boy suffering from congenital microcephaly, developmental delay, seizures and various dysmorphic features in whom nephrotic syndrome became apparent at 5 years of age.

Objective: To determine the clinicopathological pattern of lupus nephritis in paediatric nephrology patients., Design: Case series study., Place and Duration of Study: The department of paediatric nephrology at the Children's Hospital and Institute of Child Health, Lahore, Pakistan, over a period of five years from January 2001 to December 2005., Patients and Methods: Twenty six patients upto the age 16 years of either gender, with a mean age of 12.4 +/- 1.90 years having primary SLE with renal involvement in the form of oedema, hypertension, haematuria and proteinuria were included. Twenty one were females. Percutaneous renal biopsy was performed. Histological lesion was classified according to WHO classification. Patients were treated with immunosuppressive therapy and their clinical course was followed for at least one year. The mean duration of follow up was 1.77 years., Results: Renal involvement was seen in 92.30% within 2 years of the onset of primary disease. Diffuse proliferative glomerulonephritis was the commonest histological lesion (n=14) followed by membranous nephropathy (n=6). The commonest clinical manifestation was oedema (80.76%) followed by hypertension (46.15%). Proteinuria was present in 100% of cases, haematuria in 38.46% and azotemia in 19.33% of patients. Nephrotic range proteinuria was more common in class III and IV, while azotemia was observed only in class IV. The disease was well controlled in 73.07% , relapse was seen in 3.8% of patients, 15.38% died of infections and uremic encephalopathy while 7.69% were lost to follow-up., Conclusion: Diffuse proliferative glomerulonephritis is the commonest histological lesion in our set-up. Renal involvement is mostly seen within first two years of the primary disease which can be controlled satisfactorily with immunosuppressive therapy.

We report a case of a rare inherited tubular disorder of linked transport of magnesium and calcium at the level of ascending limb of loop of Henle, characterized by hypomagnesemia, hypercalciuria and nephrocalcinosis, known as "Manz syndrome," who presented with polyuria, nystagmus and recurrent episodes of tetany with radiological evidence of rickets and nephrocalcinosis.

Objective: To determine the efficacy of levamisole in steroid dependent (S.D) and frequently relapsing (F.R) nephrotic, from syndrome (N.S)., Design: Quasi-experimental study., Place and Duration of Study: Department of Nephrology at The Children s Hospital, Lahore, over a period of 5 years from January 2000 to December 2004., Material and Methods: S.D.N.S and F.R.N.S patients between the ages of 1-15 years, were given levamisole on alternate day in a dose of 2.5 mg/kg, if either the dosage of steroids to maintain remission was >1 mg/kg/every other day (EOD), or 0.5 mg/kg/EOD with signs of steroid toxicity. The agent was continued for a period of one year and the steroids were gradually tapered off by 2.5-5 mg every four weeks to less than 0.5 mg/kg/EOD. The patients were monitored for maintenance of remission and side effects of drug., Results: Seventy patients with a mean age of 5.50+/-2.97 years , with male to female ratio of 4:1 were studied. Nineteen (27.14%) patients did not relapse on therapy, while it was ineffective in 11(15.7%). Rest of 40 (57.14%) patients, though, relapsed during therapy, their duration of remission was prolonged from six months to one year, and dose of corticosteroids could be significantly reduced (0.1-0.3 mg/kg/EOD). It was also observed that levamisole is more effective in older children (>5 years versus <5 years) [P-value 0.03]. The only side effects were transient rash and occasional vomiting., Conclusion: Levamisole is a safe and effective steroid sparing drug, in steroid dependent and frequently relapsing nephrotic syndrome, for the prolongation of remission, especially in older children.

The case report describes a young boy with renal, retinal, hepatic and cerebellar involvement in a rare syndrome. He had polyuria, deranged renal functions and cystic lesions in kidneys, which led to the diagnosis of nephronophthisis (NPH). Extra-renal involvement with night blindness, truncal ataxia, mental retardation and hepatosplenomegaly. Thus, every patient with NPH should be carefully examined for extra-renal involvement.

Objective: To determine the efficacy of three different treatment protocols in steroid resistant idiopathic nephrotic syndrome, (SRINS)., Design: Interventional study., Place and Duration of Study: Department of Nephrology at The Children's Hospital, Lahore, over a period of 3 years from January 2000 to December 2002., Patients and Methods: Nephrotic children who did not respond to four weeks of steroid therapy (60mg/M2) followed by three pulses of methyl prednisolone (1Gm/1.73M2) over a period of one week were labeled as steroid resistant. Those with histopathological lesions of minimal change disease (MCD), focal segmental glomerulosclerosis (FSGS) and Mes.PGN were divided into 3 groups. Group-I was treated with cyclophosphamide (CPM) and oral steroids, group-II with cyclosporine and oral steroids and group-III with pulse methyl prednisolone (MPP) and oral steroid + CPM. The response to treatment and course of disease were observed in each group., Results: Twenty patients with mean age of 4.4 years were enrolled. On the whole 10 (50%) had complete remission. In group-I, 5 (50%), in group-II, 3 (75%) and in group-III, 2 (33.3%) had complete remission. Depending upon histological lesion 100% (n=2) with MCD, 50% (n=6) with Mes.PGN and 25% (n=1) with FSGS achieved complete remission. Cyclosporine and CPM induced remission in 100% of patients with MCD, while in Mes.PGN response rate in group-I, II, and III was 100% (n=1), 50%(n=1), and 44.4%(n=4) respectively. In patients with FSGS, MPP was the only drug used with limited response of 25% (n=1)., Conclusion: Cyclosporine proved to be a better option for MCD and Mes.PGN, while MPP showed limited response in patients with FSGS.

Objective: To assess acute complications of haemodialysis in patients with end-stage renal disease (ESRD)., Design: Observational study., Place and Duration of Study: The Nephrology Department of The Children's Hospital and Institute of Child Health, Lahore over a period of 2 years (January 2001-December 2002)., Patients and Methods: The children in age group of 6-14 years, suffering from end-stage renal disease (ESRD), were provided renal replacement therapy in the form of haemodialysis mostly twice a week and were observed for the complications of the procedure., Results: A total 519 haemodialysis were performed on 25 patients. The mean age was 11.06 years and weight was 26.8 kg. The clinical problems observed were hypertension in 40.46%, hypotension 3.4%, chest pain 4.04%, headache 13.87%, vomiting 1.73%, gastrointestinal bleeding 1.92%, muscle cramps 8.6% and convulsions 0.57% of sessions. Hypokalemia was main electrolyte disturbance seen during 8%, while fever with rigors and chills in 4.02% of sessions. Minor bleeding from subclavian catheter was seen during 2.89% of sessions while thrombosis, infection and accidental dislodgment in 0.77%, 12.50% and 0.57% of sessions respectively. Technical problems like clotting in dialyzer during 0.95% and failure of ultra-violet control module was seen in 3.42% of sessions., Conclusion: Haemodialysis, a life saving treatment modality, is not without risk of complications. Hypertension, headache, muscle cramps and catheter infections were the most commonly encountered complications in this series.