Metabolic evaluation for pediatric renal stones: a stitch in time

Objectives: To evaluate the pediatric patients with renal calculi in terms of bio-metabolic profile

Study Design: Cross sectional study

Place and Duration of Study: Department of pediatric nephrology the Children's Hospital and the Institute of Child Health Lahore over a period of 10 months from Nov 2016 to Sep 2017

Material and Methods: A total 85 patients with urolithiasis up to the age of 14 years were enrolled for study. Structured history and tailored investigations were collected from all the patients. Results of the physical examination, blood chemistry, and urinary excretion of metabolites [urinary calcium, citrate, magnesium and oxalate] were recorded

Results: Out of 85 patients; 65 percent were males and 35 percent were females [2:1]; mean age at presentation was 8.15 +/- 5.04 years. Hypertension was documented in 57 percent patients. Mean level of urea and creatinine was 73.02 +/- 59mg/dl and 4.435 +/- 4.024mg/dl respectively. Vitamin D level was 37 +/- 15.6ng/ml while serum PTH level was 51.2941 +/- 26.067pg/l. Serum calcium and phosphorus was 8.54 +/- 1.18 and 5.0224 +/- 0.885 respectively. Among all patients, 95 percent were found to have metabolic abnormalities. The most common was hypercalciuria [54 percent] followed by hyperoxaluria in 28 percent patients. Hypocitraturia was seen in 21 percent patients. Distal renal tubular acidosis was found in 6 percent children. Only 5 percent children were having low magnesium level in their urine

Conclusion: Majority of the children with stone disease had underlying metabolic risk factor and in our setting, hypercalciuria is the most common one

Comparison of efficacy of tacrolimus versus cyclosporine in childhood steroid-resistant nephrotic syndrome

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: A total 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 Mesangio PGN [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

rare case of Gitelman's syndrome with hypophosphatemia

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

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 nephritic syndrome became apparent at 5 years of age

Lupus nephritis in children

To determine the clinicopathological pattern of lupus nephritis in paediatric nephrology patients. Case series 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. 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. 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. 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

Levamisole in steroid dependent and frequently relapsing nephrotic syndrome

To determine the efficacy of levamisole in steroid dependent [S.D] and frequently relapsing [F.R] nephrotic, from syndrome [N.S]. Quasi-experimental study. Department of Nephrology at The Children's Hospital, Lahore, over a period of 5 years from January 2000 to December 2004. 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 2.5mg/kg, if either the dosage of steroids to maintain remission was >1mg/kg/every other day [EOD], or Z with CARON 0.5mg/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 2.5-5mg every four weeks to less than 0.5mg/kg/EOD. The patients were monitored for maintenance of remission and side effects of drug. 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 [0.1-0.3mg/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. 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

Familial hypomagnesemia hypercalciuria [manz syndrome]

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

Efficacy of steroids, Cyclosporin and cyclophos-phamide in steroid resistant idiopathic nephrotic syndrome

To determine the efficacy of three different treatment protocols in steroid resistant idiopathic nephrotic syndrome, [SRINS]. Interventional study. Department of Nephrology at The Children's Hospital, Lahore, over a period of 3 years from January 2000 to December 2002. 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. 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]. Cyclosporine proved to be a better option for MCD and Mes. PGN, while MPP showed limited response in patients with FSGS

Nephronophthisis: a variant

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