Nutcracker Syndrome with Hypertension as an Unusual Initial Presentation.

Hypertension has been rarely reported in patients with the nutcracker phenomenon (NCP)/syndrome. We describe a case of a young adolescent female patient where a computed tomography angiography (CTA) provided evidence of left renal vein dilatation, probably due to its compression through the angle between the aorta and the superior mesenteric artery, during the evaluation of secondary hypertension. Blood levels of renin and aldosterone were within normal limits. Ultrasonography of the kidney showed minimal scarring on the left side. As there were no other signs of secondary hypertension, we proceeded with a CTA, which revealed findings compatible with the so-called NCP/syndrome.

Management of steroid resistant nephrotic syndrome.

JUSTIFICATION: There is a lack of evidence based guidelines for management of children with steroid resistant nephrotic syndrome (SRNS). PROCESS: Experts of the Indian Society of Pediatric Nephrology were involved in a two-stage process, the Delphi method followed by a structured face to face meeting, to formulate guidelines, based on current practices and available evidence, on management of these children. Agreement of at least 80% participants formed an opinion. OBJECTIVES: To develop specific, realistic, evidence based criteria for management of children with idiopathic SRNS. RECOMMENDATIONS: The Expert Group emphasized that while all patients with SRNS should initially be referred to a pediatric nephrologist for evaluation, the subsequent care might be collaborative involving the primary pediatrician and the nephrologist. Following the diagnosis of SRNS (lack of remission despite treatment with prednisolone at 2 mg/kg/day for 4 weeks), all patients (with initial or late resistance) should undergo a renal biopsy, before instituting specific treatment. Patients with idiopathic SRNS secondary to minimal change disease or focal segmental glomerulosclerosis should receive similar therapy. Effective regimens include treatment with calcineurin inhibitors (tacrolimus, cyclosporine), intra-venous cyclophosphamide or a combination of pulse corticosteroids with oral cyclophosphamide, and tapering doses of alternate day corticosteroids. Supportive management comprises of, when indicated, therapy with angiotensin converting enzyme inhibitors and statins. It is expected that these guidelines shall enable standardization of care for patients with SRNS in the country.

Treatment of neonatal candidiasis with liposomal amphotericin B(L-AMP-LRC-1): phase II study.

A liposomal amphotericin B preparation (L-AMP-LRC-1) has been developed and tested successfully in adults by us. This preparation was administered to 23 neonates with candidiasis in an open phase II study. All the 14 assessable patients responded completely to the L-AMP-LRC-1 therapy given at 1 mg/kg for 28 days. Compared to AmBisome, another liposomal formulation of amphotericin B, L-AMP-LRC-1 was effective at lower dose in neonatal candidiasis. Thus L-AMP-LRC-1 appears to be an effective and low cost drug for the treatment of candidiasis.

Dialysis therapy in children.

Acute dialysis can be life saving for children suffering from acute emergencies due to acute renal failure, poisoning or inborn errors of metabolism causing severe organic acidaemia and hyperammonaemia. Chronic dialysis is life sustaining for children with chronic renal failure or end stage renal disease till renal transplantation is performed. The basic principles, indications, procedures, equipment, complications of acute and chronic dialysis in children are same as in adults. Dialysis can be performed in children at any age from newborn to adolescent period. In newborn, infants and preschool children (0-5 years age) haemodialysis is difficult due to technical problems associated with vascular access and haemodynamic instability. Peritoneal dialysis is simple, efficient, easy to perform, does not require highly sophisticated equipment or personnel and with low complication rate. For successful dialysis appropriate sizes of catheters, tubings, dialysers, small volume dialysate bags, etc, are required. These are now available in our country, although the cost of peritoneal dialysis fluid and catheters, etc, is 2-3 times higher than equipment for haemodialysis. Hence, continuous ambulatory peritoneal dialysis for chronic renal failure/end stage renal disease has not taken off yet in India. A team of experts including specially trained paediatric nephrologists, urologists, nurses, dieticians, technicians and social workers are needed to organise dialysis programme for children with end stage renal disease. Acute peritoneal dialysis should be made available in each paediatric department offering emergency services to children.

Antenatal diagnosis of congenital renal malformations using ultrasound.

Our objectives were to determine the accuracy of antenatal sonography for the detection of congenital renal malformations and to characterize the type of malformations, seen in a 3-year prospective study at a university-affiliated maternity hospital. Participants were 31,217 pregnant women, during the study period, and subjects were 65 fetuses in whom renal malformations were detected on antenatal ultrasound. Pelvic ultrasound scans were performed at least once between 20 and 37 weeks' gestation on all pregnant women attending the antenatal clinic of the hospital for the detection of renal malformations. Fetal urinary sampling, diversion procedures, or termination of pregnancy were carried out as required in those detected to have renal anomalies. Postnatal diagnosis was confirmed by sonography or autopsy. Diagnostic procedures and renal surgery were performed postnatally if indicated. Sixty-five fetuses (0.2 per cent) were diagnosed to have congenital renal malformation antenatally at a mean gestational age of 28.4 weeks. A dilated urinary system was seen in 39, cystic renal disease in 15, agenesis/hypoplasia in six, combined lesions in four, and a horseshoe kidney in one. Oligohydramnios was noted in 20 (31 per cent) pregnancies. Multiple congenital malformations associated with renal anomalies were detected in 12 pregnancies. Termination was carried out at 20 weeks in two pregnancies for lethal malformations; fetal urinary sampling was done in two fetuses with obstructed uropathy, and a vesicoamniotic shunt inserted in one. Postnatal ultrasound confirmed a dilated urinary system in 32, cystic renal dysplasia in 15, renal aplasia/hypoplasia in five, combined lesions in six, and a horseshoe and an ectopic kidney in one each. Five infants were found to be normal. There were seven stillbirths and seven neonatal deaths. Radionuclide scans showed obstruction in nine, decreased renal function in six, and absent renal functions in 10 infants. Micturating cystourethrography demonstrated reflux in 11 and a non-refluxing non-obstructive dilated renal system in five babies. Renal surgery was performed in nine infants. The conclusions drawn from this study were that antenatal detection of renal disease is fairly accurate, even in an extremely busy hospital and certain types of malformations reported in other studies were not observed, despite a large cohort.

Nursery outbreak of neonatal fungal arthritis treated with fluconazole.

Eight preterm infants with mean gestational age of 31.6 +/- 1.16 weeks and a mean birth weight of 1310 +/- 201.7 g presented at a mean postnatal age of 26 +/- 11.4 days with knee joint swellings and pedal oedema. There was no other clinical, haematological or microbiological evidence of bacterial sepsis. Fungal cultures yielded growth of Candida spp. from blood in five, from urine in four, from cerebrospinal fluid in one, and from all the three babies in whom the joints were aspirated. Radiographic changes of metaphysitis of the involved joints were noted in all. All infants had received prior antibiotic therapy. No infant had received total parenteral nutrition or had central lines inserted. All infants were treated with fluconazole in doses of 7.5 mg/kg/day for 6 weeks. Six of eight were thriving well at 3 months of age without any evidence of residual joint disease. One infant succumbed to disseminated disease and one was lost to follow-up. Candidial arthritis is an uncommon presentation of neonatal candidiasis. Fluconazole therapy proved effective.

Renal dysfunction detected by beta-2 microglobulinuria in sick neonates.

OBJECTIVE: To assess renal involvement in sick neonates referred to Neonatal Intensive Care Unit (NICU) using standard renal parameters and urinary beta 2 microglobulin (B2M) excretion. DESIGN: Descriptive study. SETTING: Level II NICU and Nephrology Division of Pediatric Tertiary hospital. SUBJECTS: Forty six term sick neonates transferred for neonatal care and forty healthy term neonates who served as normal controls for urinary B2M excretion. METHODS: Standard tests including estimation of BUN, serum creatinine, blood pH, serum bicarbonate, serum and urinary electrolytes, urine output, and urinalysis. Urinary B2M levels were estimated from urine collected on day 1 (D1) and day 3 (D3) in all and 18 neonates were tested on day 7 (D7) by radio-immunoassay method. RESULTS: Statistically significant elevation of mean values of urinary B2M were noted when sick neonates were compared with normal controls irrespective of primary disease, indicating tubular dysfunction (41/46 = 90%), whilst only 7 of these (17%) had abnormalities indicating renal involvement when judged by standard tests. Very high levels of urinary B2M were noted with birth asphyxia (n = 9), sepsis (n = 8) and renal disease (n = 7). Transient elevation of urinary B2M was noted in meconium aspiration syndrome (n = 4). Ten surgical cases with non renal congenital malformations showed high urinary B2M and 12/18 tested on D7 had persistently high urinary B2M due to multiple factors. CONCLUSIONS: Elevated urinary B2M in 90% sick neonates with apparently normal renal parameters in majority (34/41) indicates subclinical proximal tubular dysfunction especially in neonates with asphyxia, sepsis and congenital malformations. Persistent elevation of urinary B2M appear to be a sensitive diagnostic indicator for defining a group of neonates with subtle renal tubular dysfunction, the clinical relevance of which on long term basis is a subject for future study.

Glomerular basement membrane abnormalities in infants with heavy proteinuria.

Two Indian male children with infantile-onset heavy proteinuria (with nephrotic syndrome in 1) had thickening of the glomerular basement membrane with splitting and basket-weave appearance of lamina densa on electron microscopic evaluation of kidney tissue (like Alport's syndrome), with normal light microscopic findings and negative immunofluorescence. The proteinuria was non-familial and was not associated with microhaematuria in patient 1; transient microhaematuria, perhaps associated with urinary tract infection, was noted in patient 2. There was no neurosensory deafness in the patients or their parents. The nephrotic syndrome remitted totally in one patient over a 7-month period. The proteinuria, as well as the renal disease, was non-progressive in the second patient over a 27-month period. The significance of these basement membrane abnormalities (classically described in Alport's syndrome) in early-onset nephrotic syndrome/heavy proteinuria that is non-familial and non-progressive needs to be evaluated.

Renal parameters during infancy.

In a prospective study we estimated common renal parameters in 48 full term normal neonates, of which 15 were also tested at 6 months and 12 months of age. The mean levels of serum creatinine, were high at birth (0.73 mg/dl) but normal for age at 6 and 12 months; uric acid followed a similar trend. The blood pH and bicarbonate were low at birth (7.28 and 20.36 mEq/L, respectively) reached normal adult values by 12 months; chloride levels were high at birth (110 +/- 5 mEq/L) and normal at 6 months. The plasma renin activity was higher than normal all throughout the first year (27.1, 416.8, 64.8 ng/ml/hr by RIA). Plasma aldosterone values were high at birth (1387.5 pg/ml) and reached normal level (301.6) at 12 months. Renal length and volume as assessed by ultrasonography compared well with American standards. Urinary constituents were variable due to breast feeding up to 6 months and varied diet during the weaning period. This study shows that mild metabolic acidosis and hyperchloremia due to immaturity of renal acidification mechanism and high renin and aldosterone levels due to partial nonresponsiveness of distal tubules are normal variables in babies from birth to 6 months. The levels of serum creatinine and uric acid are high at birth and in assessing renal functions this should be borne in mind.