Background
There is paucity of information regarding the etiology and outcomes of Acute Kidney Disease (AKD) in children.
Methods
The objectives of this cohort study were to evaluate the etiology and ...outcomes of AKD; and analyze predictors of kidney survival (defined as free of CKD 2, 3a, 3b, 4 or 5). Patients aged 1 month to 18 years who developed AKD over a 4-year-period (January 2018-December 2021) were enrolled. Survivors were followed-up at the pediatric nephrology clinic, and screened for residual kidney injury.
Results
Among 5710 children who developed AKI, 200 who developed AKD were enrolled. The median (IQR) eGFR was 17.03 (10.98, 28) mL/min/1.73 m
2
. Acute glomerulonephritis, acute tubular necrosis (ATN), hemolytic uremic syndrome (HUS), sepsis-associated AKD, and snake envenomation comprised of 69 (34.5%), 39 (19.5%), 24 (12%), 23 (11.5%) and 15 (7.5%) of the patients respectively. Overall, 88 (44%) children required kidney replacement therapy (KRT). There were 37 (18.5%) deaths within the AKD period. At a follow-up of 90 days, 32 (16%) progressed to chronic kidney disease stage-G2 or greater. At a median (IQR) follow-up of 24 (6, 36.5) months (n = 154), 27 (17.5%) had subnormal eGFR, and 20 (12.9%) had persistent proteinuria and/or hypertension. Requirement of KRT predicted kidney survival (free of CKD 2, 3a, 3b, 4 or 5) in AKD (HR 6.7, 95% CI 1.2, 46.4) (p 0.04).
Conclusions
Acute glomerulonephritis, ATN, HUS, sepsis-associated AKD and snake envenomation were common causes of AKD. Mortality in AKD was 18.5%, and 16% progressed to CKD-G2 or greater at 90-day follow-up.
Objective
To study the clinico-etiological spectrum and outcomes of children with rapidly progressive glomerulonephritis (RPGN).
Methods
This retrospective cohort study evaluated patients <18 years ...with RPGN, over an 8-year period (2014–2022), for etiology and kidney outcomes.
Results
Among 68 RPGN cases median age 10 (7,12) years, 23 (33.8%) had lupus nephritis, 21 (30.9%) C3 glomerulopathy, and 15 (22.1%) infection-related glomerulonephritis (IRGN). At presen-tation, 18 (26.4%) patients had pulmonary edema, 20 (29.4%) had hypertensive emergency and 22 (32.4%) required dialysis. Median (IQR) follow-up duration was 24.5 (12,48) months. The median (IQR) admission eGFR was 19 (10.93, 38.60) mL/min/1.73 m
2
, which increased to 126 (102.7,142) mL/min/1.73m
2
at the last follow-up. At the last follow-up, 39 (57.3%) and 13 (19.1%) patients attained complete and partial renal recovery, respectively; while 16 (23.5%) progressed to CKD stage 2 and beyond. The prevalence of end stage kidney disease (ESKD) was 7.3% at 1-year and 7.7% at the last follow-up. Factors predicting kidney survival were duration of symptoms prior to presentation ≥7 days, crescents ≥37.5%, and presence of fibrous crescents/segmental sclerosis.
Conclusion
Lupus nephritis, was the commonest etiology of RPGN in children. Renal outcomes were determined by pre-admission symptoms, and percentage and stage of crescents.
Background
Three types of primary hyperoxaluria (PH) are recognized. However, data on PH type 2 (PH2), caused by defects in the
GRHPR
gene, are limited.
Methods
We reviewed the medical records of ...patients < 18 years of age with genetically-proven PH2 from seven centres across India to identify the age of onset, patterns of clinical presentation, short-term outcomes and genetic profile, and to determine if genotype–phenotype correlation exists.
Results
We report 20 patients (all with nephrolithiasis or nephrocalcinosis) diagnosed to have PH2 at a median (IQR) age of 21.5 (7, 60) months. Consanguinity and family history of kidney stones were elicited in nine (45%) and eight (40%) patients, respectively. The median (IQR) serum creatinine at PH2 diagnosis was 0.45 (0.29, 0.56) mg/dL with the corresponding estimated glomerular filtration rate being 83 (60, 96) mL/1.73 m
2
/min. A mutational hotspot (c.494 G > A), rare in Caucasians, was identified in 12 (60%) patients. An intronic splice site variant (c.735-1G > A) was noted in five (25%) patients. Four (20%) patients required surgical intervention for stone removal. Major adverse kidney events (mortality or chronic kidney disease (CKD) stages 3–5) were noted in six (30%) patients at a median (IQR) follow-up of 12 (6, 27) months. Risk factors for CKD progression and genotype–phenotype correlation could not be established.
Conclusions
PH2 should no longer be considered an innocuous disease, but rather a potentially aggressive disease with early age of presentation, and possible rapid progression to CKD stages 3–5 in childhood in some patients. A mutational hotspot (c.494 G > A variant) was identified in 60% of cases, but needs further exploration to decipher the genotype–phenotype correlation.
Graphical abstract
A higher resolution version of the Graphical abstract is available as
Supplementary information
Background: Patients with chronic kidney disease (CKD) on maintenance hemodialysis are highly vulnerable to coronavirus disease 2019 (COVID-19) infection and poorer outcomes and mortality. ...Objectives: The study aimed at identifying the various clinical and biochemical predictors of in-hospital mortality in this particular group of patients. Methods: In this retrospective cohort study, the baseline demographic, clinical, and laboratory data were collected from patients with preexisting CKD on maintenance hemodialysis and with COVID-19 infection. The statistical analysis of the collected data was performed using SPSS version 24 (SPSS Inc, Chicago, IL, USA). Results: The data obtained from 35 patients from the first wave of the pandemic were analyzed. The mortality rate was 23% (8 patients). Analyzing the comparison between survivors and non-survivors revealed that the older age (49 IQR, 42 – 55 years vs. 70 IQR, 54 – 74 years, P = 0.016), severe disease at presentation (15% vs. 75%, P = 0.004), and need for invasive mechanical ventilation (0% vs. 75%, P = 0.001) were the factors significantly associated with in-hospital mortality. Among baseline biochemical markers, severe lymphocytopenia (11 IQR, 7 – 16 vs. 4.2 IQR, 3 – 8, P = 0.011), high serum glutamic oxaloacetic transaminase (23 IQR, 15.6 – 48 vs. 80 IQR, 60- 105, P = 0.001), blood urea (71 IQR, 28 - 120) vs. 160 (IQR, 142 - 355), P = 0.002) and higher value of inflammatory markers, interleukin-6 (IL-6), and procalcitonin, as well as fibrinogen and low baseline albumin, were also significantly associated with in-hospital mortality. Conclusions: The older age, severe disease at presentation, need for invasive mechanical ventilation, raised baseline IL-6, procalcitonin, serum glutamic oxaloacetic transaminase, blood urea, and lower level of albumin may have been valuable predictors of in-hospital mortality and poor outcomes in patients with COVID-19-infected chronic kidney disease on maintenance hemodialysis.