Introduction: There is a lack of data on kidney size in Indian neonates, their determinants, and how the sizes compare with neonatal kidney sizes reported elsewhere. Methods: This cross-sectional study of renal size on ultrasonography was conducted in 765 consecutive healthy term neonates over 1 year at a single center. Multivariable regression analysis was used to examine the relationship of kidney length and volume with neonatal anthropometric variables and maternal characteristics. Information on kidney size was compared with published reports. Results: The mean ± standard deviation for the length of the right and left kidney were 38.6 ± 2.2 mm and 38.9 ± 2.2 mm, respectively, while the estimated kidney volume of the right and left kidney was 7.7 ± 1.7 ml and 8.3 ± 1.9 ml, respectively. Birth weight and neonatal length were independent predictors of renal length. Kidney length and volume in the present study were significantly lower as compared to that reported in the studies from outside south Asia. Conclusions: Kidney length in Indian term neonates depends on neonatal weight and length and is smaller in comparison to neonates from outside south Asia.

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Introduction: Information on outcomes of pediatric renal transplantation in developing countries is limited to few single-center reports with small numbers. Methods: Medical records of patients who underwent kidney transplantation at a single tertiary care center were retrospectively reviewed for information on recipient and donor characteristics, immunosuppression, posttransplantation complications, anthropometry and graft and patient survival. Allograft and patient survival were estimated using Kaplan–Meier survival analysis; risk factors for acute and chronic rejection (AR and CR) and patient or allograft loss were examined using Cox regression. Results: During 1995–2017, 116 patients (84% boys) received 120 kidney allografts at the age of 13 ± 3.8 years, chiefly from live-related donors (76%). During median (range) 4.5 (0.8–19.5) years of follow-up, AR and CR were seen in 24% and 15.5% allografts. Ten (8.8%) patients died, chiefly due to septicemia, while 26 (21.7%) allografts were lost, most often to CR. Severe infections included septicemia (13%), urinary (23%) or respiratory tract (9%) infections, cytomegalovirus (11%), and chronic viral hepatitis (7%). At 1, 3, 5, 10 and 15 years, patient survival was estimated at 95.5%, 94.4%, 91.7%, 89.2% and 79.3%, respectively, while allograft survival was 92.2%, 87.2%, 85.6%, 77.6% and 77.6%, respectively. Final height, at −2.2 ± 1.3 standard deviation scores, was significantly improved from baseline (P = 0.001). Conclusions: Pediatric kidney transplantation in developing countries is associated with similar patient and allograft survival as compared to developed regions. While severe infections cause serious morbidity and mortality, rates of immunological complication are similar to developed regions. While patients show significant height gain, final stature is subnormal.

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Treatment of children with end-stage kidney disease (ESKD) poses various challenges. While these have been studied extensively in affluent countries, data from developing regions are scant. In most rich countries, state-of-art management and financial and social support are provided by the government or available through insurance, and prognosis for even young children is improving. In contrast, most low-income countries, either lack in facilities for renal replacement or these are not afforded by the vast majority, and a large number of patients succumb for lack of treatment. In rare settings where such treatment is provided free of cost, the inability to meet costs of related expenses such as transport and accommodation result in families discontinuing management for most patients. Studies should define the extent and determinants of these concerns for children with ESKD in developing countries.

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While infections are known to precipitate disease relapses in childhood nephrotic syndrome, it is unclear whether diarrheal illnesses carry the same risk for relapses as acute respiratory tract infections. This paired case–control study included age- and sex-matched episodes of relapses (cases) and remission (controls). Records were reviewed retrospectively for the presence of acute respiratory tract infections and diarrhea in the two weeks preceding the clinic visit. McNemar test was used to examine associations between these infections and relapse. In 17 patients with 38 paired episodes of relapses and remission, the odds ratio for relapse compared to remission, associated with acute respiratory tract infections, was 3.25 (95% confidence interval: 1.06, 9.97; P = 0.03), while that for acute diarrhea was 3.5 (95% confidence interval: 0.73, 16.85; P = 0.10). Acute respiratory tract infections are a risk factor for relapses in pediatric nephrotic syndrome, while acute diarrhea does not predispose to disease relapses.

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Introduction: The etiology of acute kidney injury (AKI) in hospitalized children is multifactorial. While nephrotoxic antibiotics, such as aminoglycosides, cause AKI, the prevalence and severity of AKI in this context are uncertain. Methods: This prospective observational study enrolled consecutive patients, from 1 month to 12-year-old, admitted to pediatric wards at an academic tertiary care center between March 2013 and March 2014. Included patients had normal renal function at baseline and were treated with aminoglycosides for more than 7 days. Serum creatinine was monitored serially for the development of AKI by pediatric risk, injury, failure, loss, end-stage renal disease (pRIFLE), and AKI network (AKIN) classifications. Data, presented as percentage or mean ± standard deviation, were compared using appropriate tests. Results: Of 100 patients (68% boys) treated with aminoglycosides for 11.4 ± 4.5 days, chiefly for pneumonia, febrile neutropenia and abscesses, 97% received amikacin and 3% received gentamicin, chiefly (84%) in multiple doses. AKI was observed in 46% and 62% of cases using AKIN and pRIFLE criteria, respectively. Patients with AKI were younger than those without AKI (P = 0.04) and received multiple dosing more often than once-daily regimen (P = 0.07). Conclusions: AKI develops in a significant proportion of hemodynamically stable patients treated with aminoglycosides for 7 days or longer. Young age and multiple daily doses are potential risk factors for AKI in patients treated with aminoglycosides.

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Introduction: Previous studies suggest that maternal serum vitamin A levels and polymorphisms in genes regulating vitamin A metabolism may impact fetal kidney development. Information on these genetic variations is limited to Caucasian population. Methods: This prospective observational single center study included newborns of 349 pregnant women enrolled at 8 weeks of gestation. Kidney volumes were measured at birth by ultrasound and cord blood was tested for single nucleotide polymorphisms (SNPs) in genes encoding for aldehyde dehydrogenase 1 family member A2^rs7169289, cellular retinoic acid binding protein-2^rs12724719, receptor tyrosine kinase^rs1800860, retinol-binding protein^rs11187540, and the Vitamin A receptor stimulated by retinoic acid 6^rs17852249. Results: The total (left + right) kidney volume, unadjusted and adjusted for body surface area at birth, was 21.5 ± 4.6 ml and 107.3 ± 20.7 ml/m², respectively. Single nucleotide polymorphisms (SNPs) in genes encoding for proteins regulating vitamin A metabolism were not associated with kidney volume at birth. Conclusions: Polymorphisms in genes involved in vitamin A metabolism are not associated with kidney size in Indian newborns.

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Atypical hemolytic uremic syndrome (aHUS) is a rare, life-threatening illness characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury (AKI). Plasma therapy, previously considered the primary treatment for this condition, is associated with death and permanent kidney injury in over 50% of patients. Eculizumab, a monoclonal antibody that blocks C5, is far more effective and is the current treatment of choice, but it is not widely available. An 18-month-old boy presented with AKI, anemia, and normal platelet count. While managed initially as proliferative antineutrophil cytoplasmic antibody-associated glomerulonephritis, a diagnosis of partial HUS was suggested by low levels of complement C3, complement factor I (CFI) and complement Factor B (CFB), and histological changes suggesting microangiopathy without thrombi. Later, next-generation sequencing indicated homozygous pathogenic mutations in CFI and heterozygous variations in C3 and CFB. Corticosteroid pulses and plasmapheresis were ineffective in resolving the need for dialysis, and hypertension was refractory to polytherapy. Therapy with eculizumab, initiated only after 5 months on dialysis, was associated with rapid increase in urine output, control of hypertension, and slow but complete renal recovery. Dialysis was discontinued after 5 months of eculizumab therapy, and renal function has been within normal range after 33 months of initiating therapy with eculizumab. Our case emphasizes that a diagnosis of aHUS should be considered in the appropriate clinical setting even in the absence of thrombocytopenia. Kidney biopsy and genetic testing are useful in confirming the diagnosis. Therapy with eculizumab in patients with severe AKI may be associated with complete renal recovery even if therapy is delayed by several months.

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Antiglomerular basement membrane (GBM) disease, caused by IgG antibodies directed against type IV collagen in the GBM, may present with rapidly progressive glomerulonephritis and/or diffuse alveolar hemorrhage. While rare in childhood, pediatric patients usually have both renal and pulmonary involvement. We report a child who presented with crescentic glomerulonephritis and severe renal-limited anti-GBM disease. The diagnosis was aided by findings of characteristic linear IgG deposits on GBM on immunofluorescence on kidney biopsy and high titers of anti-GBM antibody. As patients with renal-limited disease and severe renal failure usually do not recover renal function despite aggressive immunosuppression and plasmapheresis, intensive therapy was withdrawn while continuing maintenance hemodialysis. However, the patient later developed severe alveolar hemorrhage that responded completely to plasmapheresis and intensification of immunosuppression. Sustained serological and pulmonary response was documented, even though renal dysfunction persisted. Our case emphasizes the utility of kidney biopsy and serology in enabling rare diagnosis in patients with rapidly progressive glomerulonephritis and the role of aggressive plasmapheresis in enabling recovery from delayed catastrophic alveolar hemorrhage.

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