Mukta Mantan

Treatment with tacrolimus and prednisolone is preferable to intravenous cyclophosphamide as the initial therapy for children with steroid-resistant nephrotic syndrome

There are limited data on the relative efficacy and safety of calcineurin inhibitors and alkylating agents for idiopathic steroid-resistant nephrotic syndrome in children. To clarify this, we compared tacrolimus and intravenous cyclophosphamide therapy in a multicenter, randomized, controlled trial of 131 consecutive pediatric patients with minimal change disease, focal segmental glomerulosclerosis, or mesangioproliferative glomerulonephritis, stratified for initial or late steroid resistance. Patients were randomized to receive tacrolimus for 12 months or 6-monthly infusions of intravenous cyclophosphamide with both arms receiving equal amounts of alternate-day prednisolone. The primary outcome of complete or partial remission at 6 months, based on spot urine protein to creatinine ratios, was significantly higher in children receiving tacrolimus compared to cyclophosphamide (hazard ratio 2.64). Complete remission was significantly higher with tacrolimus (52.4%) than with cyclophosphamide (14.8%). The secondary outcome of sustained remission or steroid-sensitive relapse of nephrotic syndrome at 12 months was significantly higher with tacrolimus than cyclophosphamide. Treatment withdrawal was higher with cyclophosphamide, chiefly due to systemic infections. Compared to cyclophosphamide, 3 patients required treatment with tacrolimus to achieve 1 additional remission. Thus, tacrolimus and prednisolone are effective, safe, and preferable to cyclophosphamide as the initial therapy for patients with steroid-resistant nephrotic syndrome.

Benefit of gluten‐free diet in idiopathic pulmonary hemosiderosis in association with celiac disease

Lane–Hamilton syndrome refers to the uncommon co‐occurrence of idiopathic pulmonary hemosiderosis and celiac disease (CD). Three children aged between 7 and 14 years with IPH were detected to have co‐existing non‐diarrheal CD. Institution of gluten‐free diet in each of the three children resulted in amelioration of the pulmonary symptoms along with improvement of anthropometric parameters and hemoglobin over a short‐term follow‐up period of 8–17 months. Inhaled/oral steroids and immunosuppressants could be weaned off after dietary exclusion therapy in each of the three children. Gluten free diet should be instituted in all patients diagnosed with Lane–Hamilton syndrome. It ameliorates both the pulmonary as well as the intestinal symptoms although the precise mechanism of the pulmonary response is as yet unclear. Pediatr Pulmonol. 2011; 46:302–305.

Management of urinary tract infections.

Urinary tract infection (UTI) is common in childhood. It may result in long-term complications due to renal scaring. Younger children are at higher risk of renal scarring. The diagnosis of UTI is based on urine culture. The bacterial count for diagnosis of UTI depends on the method of urine collection. Urinalysis is useful for making a presumptive diagnosis of UTI and allows initiation of empirical treatment in high-risk patients, after urine culture has been obtained. The treatment of UTI is guided by the severity of illness and age of the patient. Following a UTI, investigation should be performed to identify an underlying urinary tract anomaly. Recurrence of UTI occurs in 30–50% children. Important predisposing factors include VUR, urinary tract obstruction, voiding dysfunction and constipation. Vesicoureteric reflux (VUR) is seen in 30–50% children with UTI. The cornerstone of management of VUR is long-term antibiotic prophylaxis, which has been found to be as effective as surgical reimplantation.

Efficacy of intravenous pulse cyclophosphamide treatment versus combination of intravenous dexamethasone and oral cyclophosphamide treatment in steroid-resistant nephrotic syndrome

We compared, in a randomized controlled trial, the efficacy of a regimen based on intravenous (i.v.) cyclophosphamide therapy with a combination of i.v. dexamethasone and oral cyclophosphamide therapy in inducing remission in patients with steroid-resistant nephrotic syndrome (SRNS). During April 2001 to December 2003, 52 consecutive patients with idiopathic SRNS, normal renal function and renal histology findings showing minimal change disease, focal segmental glomerulosclerosis or mesangioproliferative glomerulonephritis were enrolled into the study. Patients in group I received i.v. injection of cyclophosphamide once a month for 6 months and prednisolone on alternate days. Those in group II received i.v. treatment with dexamethasone (initially on alternate days, later fortnightly and monthly; total 14 doses), oral cyclophosphamide therapy (for 3 months) and prednisolone on alternate days. Data from 49 patients (26 in group I, 23 in group II) were analyzed; their clinical and biochemical features were similar at inclusion. Following treatment, complete remission was seen in 53.8% and 47.8% patients in groups I and II, respectively (P = 0.6). Long-term follow up showed favorable outcome in 14 (53.8%) patients in group I, and 9 (39.1%) in group II. Chief adverse effects, including cushingoid features and serious infections, were similar in both groups. Patients receiving i.v. dexamethasone therapy commonly showed hypertension and hypokalemia, while vomiting and reversible alopecia occurred in those receiving i.v. treatment with cyclophosphamide. In patients with SRNS, the efficacy of treatment intravenously with cyclophosphamide and orally with prednisolone was similar to the combination of dexamethasone intravenously, orally administered cyclophosphamide and prednisolone.

Disease course in steroid sensitive nephrotic syndrome

ObjectiveTo review the disease course in patients with steroid sensitive nephrotic syndrome (SSNS) and the factors that determine outcomeDesignRetrospective, analyticalSettingPediatric Nephrology Clinic at referral center in North IndiaParticipants/patientsAll patients with SSNS evaluated between 1990 and 2005InterventionNoneMain outcome measuresDisease course, in patients with at least 1-yr follow up, was categorized as none or infrequent relapses (IFR), frequent relapses or steroid dependence (FR), and late resistance. Details on complications and therapy with alternative agents were recorded.ResultsRecords of 2603 patients (74.8% boys) were reviewed. The mean age at onset of illness and at evaluation was 49.7±34.6 and 67.5±37.9 months respectively. The disease course at 1-yr (n=1071) was categorized as IFR in 37.4%, FR in 56.8% and late resistance in 5.9%. During follow up, 224 patients had 249 episodes of serious infections. Alternative medications for frequent relapses (n=501; 46.8%) were chiefly cyclophosphamide and levamisole. Compared to IFR, patients with FR were younger (54.9±36.0 vs. 43.3±31.4 months), fewer had received adequate (≥8 weeks) initial treatment (86.8% vs. 81.7%) and had shorter initial remission (7.5±8.6 vs. 3.1±4.8 months) (all P<0.001). At follow up of 56.0±42.6 months, 77.3% patients were in remission or had IFR, and 17.3% had FR.ConclusionsA high proportion of patients with SSNS show frequent relapses, risk factors for which were an early age at onset, inadequate initial therapy and an early relapse.

Megacystis, microcolon, intestinal hypoperistalsis syndrome and bilateral streak gonads.

Megacystis, microcolon, intestinal hypoperistalsis is an uncommon condition presenting in neonatal age with features of intestinal obstruction and bladder evacuation abnormalities. We present here an infant girl with the diagnosis consistent with this entity.

Acute renal failure in children in a tertiary care center

In this retrospective study, records of all patients aged one month to 12 years who presented with acute renal failure (ARF) between May 2005 and August 2010 were retrieved. Clinical details, biochemistry, need for renal replacement therapy (RRT), cause of ARF and outcome at discharge were recorded. During this period, 230 children presented with ARF; their median age at presentation was 30 months (range: five-144 months); 120 (52.2%) were males. The causes of ARF were acute tubular necrosis (ATN) in 121 (52.6%), glomerular disorders in 5.7%, structural anomalies of the urinary tract in 9.6% and hemolytic uremic syndrome in 27 (11.7%). The mean duration of hospital stay was 17.8 ± 7.6 days. RRT was required for 54 patients (23.6%); peritoneal dialysis in 49 and hemodialysis in five patients. Complete recovery was noted in 99 study patients (43.2%) and sequelae remained in 84 patients (36.7%). Forty-six patients (20.1%) with ARF died. ATN secondary to septicemia was the most common cause of ARF in our study.

Post-infectious glomerulonephritis following infective endocarditis: Amenable to immunosuppression.

Glomerulonephritis develops in about 20% patients with infective endocarditis (IE), but is mostly asymptomatic. Heavy proteinuria or derangement of kidney functions is uncommon. We report here a child with IE and proliferative glomerulonephritis who manifested as significant proteinuria that recovered on treatment with immunosupressants.

Acute Lymphoblastic Leukemia: An Unusual Cause of Nephromegaly in Infancy

We present here an infant who had acute lymphoblastic leukemia with massively enlarged kidneys due to leukemic infiltrates. A 7-month-old girl child presented with complaints of low grade fever and coryza for 6 days. The mother noticed angular deviation of mouth and tongue to right side for the past 4 days and decreased movements of right upper limb for same duration. On examination, the child had pallor and few petechie over the trunk. The vitals were HR 122/min, pulses well palpable, RR 48/min; blood pressure was 134/94 mm Hg. Her anthropometry was: weight 6.6 Kg (10th centile), length 65 cm (25th centile), and head circumference was 41.5cm (25th centile). Central nervous system examination revealed normal sensorium. There was left facial lower motor neuron palsy, right hypoglossal palsy and right sided hemiparesis (upper motor neuron type). There were no meningeal or cerebellar signs. She had a soft hepatomegaly (2.5 cm below costal margin) and splenomegaly (6 cm below costal margins). Bilateral kidneys were palpable and occupied most of the abdomen, their surface was smooth.

Acute kidney injury in a child: A case of Munchausen syndrome by proxy.

Renal and urologic problems in pediatric condition falsification (PCF) or Munchausen by proxy (MSP) can result in serious diagnostic dilemma. Symptoms of hematuria, pyuria and recurrent urinary tract infections have occasionally been described. However, MSP presenting as azotemia has not been previously reported. We describe the case of an unfortunate boy who had to undergo unnecessary hemodialysis for persistent hyperkalemia and azotemia before a final diagnosis of the falsification of investigations by the parents was made.