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BRIEF REPORTS
Renal involvement in a child with Donnai-Barrow syndrome
Gurinder Kumar, Manika Chaudhry, Khalid Mohamed Mansour Mohamed Faris, Omar Al Masri
July-December 2018, 1(2):93-95
DOI:10.4103/AJPN.AJPN_22_18  
Donnai-Barrow or facio-oculo-acoustico-renal (DB/FOAR) syndrome is characterized by typical craniofacial features, ocular findings, sensorineural hearing loss and agenesis of the corpus callosum along with varying degree of intellectual disability. Renal involvement in the form of low molecular weight proteinuria is commonly reported. We report a case of an 8-year-old girl with DB/FOAR syndrome which was genetically confirmed to have a novel frameshift mutation, c.13139del in exon 72 of LRP2, the gene encoding low density lipoprotein receptor related protein 2 precursor, megalin. The child had chronic kidney disease (CKD) and significant proteinuria with focal segmental glomerulosclerosis on renal biopsy. Our case highlights presentation in childhood with this rare syndrome, with significant renal involvement as nephrotic range proteinuria and CKD. Children with DB/FOAR syndrome need close follow up with nephrologist.
  2 2,772 223
REVIEW ARTICLES
Summary of 'Hemolytic uremic syndrome in a developing country: Consensus guidelines'
Sushmita Banerjee, Jyoti Sharma
July-December 2019, 2(2):71-74
DOI:10.4103/AJPN.AJPN_21_19  
Hemolytic uremic syndrome (HUS) is a common cause of acute kidney injury in children and has implications of irreversible renal damage. The management depends on the etiology of HUS. Guidelines for India have been formulated to arrive at the etiology with an algorithmic approach based on the epidemiology and the constraints of facilities available for investigations and therapy in the region. Anti-Factor H antibody-associated illness accounts for over half the cases of atypical HUS (aHUS) in Indian children, and prompt plasma exchange and immunosuppression are recommended to lower antibody levels. Since access to eculizumab is limited, the management of other forms of aHUS relies on plasma therapy. Indications for biopsy and concerns around kidney transplantation are highlighted. A brief comparison with current guidelines from other regions has been made.
  2 2,776 462
STUDY PROTOCOL
Determining the optimal dose of cholecalciferol supplementation in children with chronic kidney disease (C3 Trial): Design of an open-label multicenter randomized controlled trial
Arpana Aprameya Iyengar, Nivedita Kamath, V Hamsa, Susan Uthup, Jyoti Sharma, Jyoti Singhal, Sudha Ekambaram, Rukshana Shroff
July-December 2018, 1(2):67-73
DOI:10.4103/AJPN.AJPN_34_18  
Introduction: 25-hydroxyvitamin D (25OHD) deficiency is common in children with chronic kidney disease (CKD) and can affect bone mineralization and cardiovascular morbidity. It is important to treat 25OHD deficiency appropriately in a manner that ensures not only replenishing stores but also sustaining adequate 25OHD levels without causing toxicity. The present study was planned to determine the appropriate dosing regimen for oral cholecalciferol that achieves and maintains normal 25OHD levels in children with CKD stage 2–4 and to assess the effect of various dosing regimens on bone biomarkers, secondary hyperparathyroidism, and vitamin D toxicity. Methods: We present the design of an open-label, multicenter randomized controlled trial conducted across four pediatric nephrology centers in India. Children in CKD stages 2–4 with 25OHD levels <30 ng/ml will be randomized to one of three therapy regimens for oral cholecalciferol (3000 IU daily, 25,000 IU weekly, or 100,000 IU monthly) given for 3 months, allowing an equivalent cumulative cholecalciferol dose in all arms over this intensive replacement therapy phase. After 3 months, patients with 25OHD levels ≥30 ng/ml will continue on maintenance therapy, administered at 1000 IU cholecalciferol orally daily for 9 months. Outcomes include the median change in the level of 25OHD from baseline to the end of intensive phase; proportions of children in each limb that attain and maintain normal 25OHD levels after intensive replacement and maintenance treatment; the change in levels of bone biomarkers and the incidence of adverse effects with each therapy regimes. Conclusion: The study design of a multicenter randomized controlled trial in children with CKD is described. Trial Registration: Clinical Trials Registry of India; www.ctri.nic.in; CTRI/2015/11/010180.
  2 3,343 362
BRIEF REPORTS
Novel pathogenic variant causing familial hypomagnesemia with hypercalciuria and nephrocalcinosis
Georgie Mathew, Priyanka Khandelwal, Ranjeet Thergaonkar, Aditi Sinha, Pankaj Hari, Arvind Bagga
January-June 2020, 3(1):31-33
DOI:10.4103/AJPN.AJPN_11_20  
Nephrocalcinosis in children can arise due to a variety of metabolic conditions and appropriate diagnosis can lead to specific management and has implications in prognosis. Familial hypomagnesemia with hypercalciuria and nephrocalcinosis (FHHNC) is a rare disease with varied manifestations which is caused by mutations in CLDN16 and CLDN19, and has a high rate of progression to kidney failure in the second to third decades of life. Early diagnosis aids in retardation of the progression to kidney failure. We present two unrelated patients presenting with nephrocalcinosis and deranged renal function, who had a novel pathogenic variation (c.685C > T; p.Q229X), leading to the diagnosis of FHHNC. Genetic testing is crucial in patients presenting with nephrocalcinosis for diagnosis and prognostication.
  1 1,438 105
ORIGINAL ARTICLES
Incidence and determinants of acute kidney injury in patients with nephrotic syndrome
Sunil Kushwah, Menka Yadav, Pankaj Hari, Jitendra Meena, Aditi Sinha, Arvind Bagga
July-December 2019, 2(2):75-81
DOI:10.4103/AJPN.AJPN_25_19  
Background: Retrospective studies from developed countries indicate an increasing prevalence of acute kidney injury (AKI) in nephrotic syndrome. Prospective information on incidence and determinants of AKI in nephrotic syndrome from developing countries is limited. Methods: This prospective study enrolled consecutive patients with nephrotic syndrome, 1 month–18 years old, admitted to a single tertiary care center. Patients were evaluated daily for the development of AKI using Kidney Disease Improving Global Outcomes serum creatinine criteria until day 14 or discharge, along with clinical and biochemical information to determine associated risk factors. Estimated glomerular filtration rate (eGFR) was reassessed at 3 months' follow-up. Results: Of 115 patients (72.2% boys) enrolled at median (interquartile range) age 64 (36–111) months, 25 (21.7%) developed AKI. The incidence density of AKI was 3.3 (2.2, 4.8) episodes per 100 person-days. Stage 3 AKI comprised 64% of cases. Steroid-resistant illness, hypoalbuminemia, and low baseline eGFR were independently associated with the occurrence of AKI. AKI recovered completely or partially in 48% and 20% cases, respectively; 20% of patients remained dialysis-dependent and 12% of patients died. Patients with AKI had significantly longer hospital stay, and lower median eGFR at 3-month follow-up, than those without AKI. Conclusions: AKI affects 21.7% of patients admitted with nephrotic syndrome, is predominantly severe, and is associated with adverse outcomes in one-third cases, prolonged hospital stay, and reduced eGFR at discharge and short-term follow-up. Steroid-resistant nephrotic syndrome, hypoalbuminemia, and low eGFR at admission were independently associated with the occurrence of AKI.
  1 3,406 453
Cystatin C as a biomarker of acute kidney injury in sick neonates
Jyoti Bagla, Sweta Kumari, Raksha Gupta, Iqra Khan
January-June 2020, 3(1):10-14
DOI:10.4103/AJPN.AJPN_29_19  
Objective: This study was planned to study serum cystatin C (CysC) levels in sick neonates admitted in neonatal intensive care unit (NICU) with predisposing risk factor(s) for development of acute kidney injury (AKI). We evaluated serum levels of CysC and creatinine in sick neonates. Methods: Ninety-four sick neonates with prematurity, sepsis, respiratory distress, and/or perinatal asphyxia requiring NICU admission were enrolled. Serum levels of creatinine and CysC were measured on day 3 of life. Results: Serum levels of both cystatin C and creatinine were significantly elevated in neonates who developed AKI versus those who did not (CysC: 2.18 ± 0.59 vs. 1.91 ± 0.47 mg/dl, P = 0.04; creatinine: 1.68 ± 0.75 vs. 0.89 ± 0.40, P < 0.001). Levels of CysC were elevated in sick neonates with risk factors of developing AKI. Conclusions: Serum levels of CysC are elevated in sick neonates at risk of AKI. CysC is a useful indicator of risk of AKI in ill neonates.
  1 2,524 208
REVIEW ARTICLES
Vesicoureteral reflux and recurrent urinary tract infections
Jitendra Meena, Pankaj Hari
July-December 2019, 2(2):61-70
DOI:10.4103/AJPN.AJPN_26_19  
Vesicoureteral reflux (VUR) refers to backflow of urine from the bladder into the upper urinary tract. Children with high-grade VUR are at risk of recurrent urinary tract infections (UTIs). It is commonly associated with bladder–bowel dysfunction, necessitating treatment because it further increases the risk of recurrent UTI and results in delayed resolution of VUR. Almost one-third of patients with VUR diagnosed following UTI show renal scarring. It is debated whether scarring in VUR is entirely acquired following UTI or due to dysplasia. The efficacy of antibiotic prophylaxis has been variable for the prevention of UTI in children with VUR. A meta-analysis suggests that it might be of some benefit in preventing UTI, but not renal scarring and at cost of bacterial resistance. Due to questionable efficacy and potential risk, use of antibiotic prophylaxis should be based on risk stratification rather than mere presence of reflux. In view of lack of significant improvement in long-term outcomes with current available interventions, there is ongoing debate on the aggressiveness of algorithms for diagnosing VUR following UTI. Long-term complications of VUR include proteinuria, hypertension, and end-stage renal disease. As opposed to previous assumption, the risk of end-stage renal failure and hypertension is fairly small with scarring following UTI.
  1 6,736 680
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