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   Table of Contents - Current issue
July-December 2019
Volume 2 | Issue 2
Page Nos. 61-109

Online since Wednesday, December 4, 2019

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Vesicoureteral reflux and recurrent urinary tract infections p. 61
Jitendra Meena, Pankaj Hari
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.
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Summary of 'Hemolytic uremic syndrome in a developing country: Consensus guidelines' p. 71
Sushmita Banerjee, Jyoti Sharma
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.
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Incidence and determinants of acute kidney injury in patients with nephrotic syndrome p. 75
Sunil Kushwah, Menka Yadav, Pankaj Hari, Jitendra Meena, Aditi Sinha, Arvind Bagga
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.
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Mycophenolate mofetil for maintenance of remission in children with steroid- and calcineurin inhibitor- dependent nephrotic syndrome: A prospective, randomized multicenter trial p. 82
Eun Mi Yang, Eujin Park, Hyun Jin Choi, Hyesun Hyun, Yong-hoon Park, Kyung Hee Han, Hyewon Park, Seong Heon Kim, Il-Soo Ha, Hae Il Cheong, Hee Gyung Kang
Background: Mycophenolate mofetil (MMF), a nonsteroidal immunosuppressive agent, has been used as a therapeutic option in various immune-mediated glomerulonephritis. However, controlled studies that examine the efficacy of MMF monotherapy in steroid- and calcineurin inhibitor (CNI)-dependent nephrotic syndrome (NS) are lacking. Aim: This study assessed the efficacy and safety of MMF monotherapy in children with steroid- and CNI-dependent NS. Methods: In this prospective, randomized, open-label, multicenter pilot study, we evaluated the efficacy and safety of MMF after CNI and corticosteroid therapy in children with steroid-dependent NS. We compared the duration and timing of sustained remission between patients managed with or without MMF and monitored for adverse effects of MMF. Statistical Analysis Used: The data were analyzed using standard statistical tests on the modified intention-to-treat population. Results: The baseline characteristics between the two groups did not differ significantly. There was neither difference in the duration of the sustained remission between patients in the MMF and control groups (4.2 ± 3.5 months vs. 3.8 ± 3.4 months, respectively; P = 0.772) nor in the proportion in sustained remission after 12 months (P = 0.936). Patients received MMF did not experience significant toxicity; chiefly, self-remitting gastrointestinal adverse events were observed. Conclusions: Therapy with MMF is safe but ineffective in influencing rates of remission in patients with NS dependent on steroids and CNIs.
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OHVIRA syndrome in an infant girl p. 88
Ruba Sahab, Sherif Mohamed El-Desoky, Ahmed Alsayyad, Mazen Kurdi, Jameela A Kari
Obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) is a rare congenital urogenital anomaly, which usually presents after menarche. We report a 3-month-old infant who presented with recurrent urinary tract infections (UTI) and had an abdominal lump. Radiological investigations revealed absent right kidney, hydronephrotic left kidney, and a cystic pelvic mass. Exploratory laparotomy revealed hydrometrocolpos which was evacuated. During 1-year follow-up, hydronephrosis decreased, there were no further UTI, and kidney function remained normal. Our case illustrates that OHVIRA syndrome may present in early infancy with UTI, renal anomalies, and pelvic mass.
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Distal renal tubular acidosis with hemolytic anemia and myotonia: Unusual phenotype of a known mutation p. 91
Gitanjali Jain, Suprita Kalra, Deepak Joshi
Distal renal tubular acidosis (dRTA) is a tubulopathy with metabolic acidosis due to impaired renal acid excretion. Patients with dRTA present with hypokalemia, medullary nephrocalcinosis, nephrolithiasis, growth retardation, and rickets in childhood. Inherited dRTA due to solute carrier family 4 member 1 gene (SLC4A1) mutation on chromosome 17 is known to be associated with hemolytic anemia, whereas mutations in other genes might be associated with hearing loss (ATP6V1B1, ATPV0A4, or FOXI1) or dental enamel defects (WD repeat-containing protein 72). However, an association of dRTA with myotonia has not been reported. We report here an infant with dRTA with hemolytic anemia and persistent myotonia due to a homozygous missense mutation (p. Ala858Asp) in exon 19 of SLC4A1.
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Role of renal doppler spectral study in detecting allograft dysfunction in early post-transplant period p. 94
Ramya Vedula, Farha Furruqh, Arpana Iyengar
Doppler sonography is routinely used to assess global and segmental perfusion abnormalities in renal allograft. However, its utility in distinguishing between the causes of allograft dysfunction is unclear, and information is lacking on the outcomes of allografts with abnormal findings. In this single center study, we retrospectively reviewed the records of pediatric allograft recipients who underwent Doppler spectral study within 3 weeks of transplantation. Resistive index (RI) of >0.7 was considered abnormal. Serum creatinine at the time of Doppler and at 3-month follow-up was documented. Among 34 children transplanted over 5 years, delayed graft function (DGF) was observed in 36% of allografts. RI was significantly higher for allografts with DGF than those without DGF (0.85 ± 0.17 vs. 0.69 ± 0.10; P = 0.004). Serum creatinine was significantly higher for allografts with abnormal than normal Doppler. However, there was no significant association between RI and the underlying histopathology. RI at the level of segmental (r = 0.62) and arcuate arteries (r = 0.55) correlated well with serum creatinine. Allografts with high and normal RI had comparable serum creatinine at 3-month follow-up (median [interquartile range] 0.65 [0.6, 0.9] vs. 0.7 [0.65, 0.8]; P = 0.98).
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Restorative occupational therapy for adolescents with chronic kidney disease p. 98
Lili Chan, Alison Ma, Sanne Fong, Ellen Yu, Phoebe Chan, Hellen Yang, Euan Soo, WM Lai
Occupational therapy is demonstrated to improve quality of life and enable rehabilitation of patients with various chronic diseases. However, their role in managing children with chronic kidney disease (CKD) remains unclear. Twelve adolescents with CKD managed at a single center in Hong Kong participated in a 4-weeks occupational lifestyle redesign program (OLSRP) comprising 8 sessions of self-initiated individualized activity plans, led by two occupational therapists. Two surveys, the self-management inventory and Chinese version of General Self-efficacy Scale, administered at initiation and end of OLSRP, were used to examine its impact on competence, confidence and self-efficacy scores. Following OLSRP, 91.7% and 75% of 12 adolescents showed significant improvements in composite scores of both scales, with significant increments in self-management concerning disease, social and collaborative skills, diet, proper schedule, medication intake and acceptance of their disease, and problem solving and confidence.
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Atypical hemolytic uremic syndrome with diacylglycerol kinase epsilon (DGKE) gene mutation p. 101
Aditi Sharma, Priyanka Khandelwal, Menka Yadav, Sidharth Sethi, Pankaj Hari, Aditi Sinha, Arvind Bagga
Atypical hemolytic uremic syndrome (aHUS), an important cause of acute kidney injury requiring dialysis in children, is caused by defects in genes encoding the alternative complement pathway. Mutations in noncomplement-related genes are rare, information of therapy and outcome of such patients is limited. We describe a 13-month-old boy with aHUS as a result of compound heterozygous mutations in diacylglycerol kinase gene, possibly triggered by enteroinvasive Escherichia coli infection, with partial response to plasma exchanges. The present case emphasizes the need for a high index of suspicion, appropriate investigation for etiology of the infective trigger, and good supportive care in the management of rare causes of aHUS.
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Renal histopathological profile of Bangladeshi children in a tertiary care hospital p. 104
Afroza Begum, Samina Masud Santa, Abdullah Al Mamun, Tahmina Jesmin, Syed Saimul Huque, Ranjit Ranjan Roy
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Journal scan p. 106
Priyanka Khandelwal
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