Indian Journal of Nephrology最新文献

Background: Autosomal dominant kidney disease (ADPKD) is the most common monogenic disorder leading to renal failure with limited therapeutic options. We aimed to assess the efficacy and safety of metformin in nondiabetic ADPKD patients and its role in slowing disease progression.

Materials and methods: We conducted a prospective, randomized controlled, open labelled clinical trial and enrolled 52 nondiabetic adults aged 18-60 years with typical ADPKD, estimated glomerular filtration rate (eGFR) > 45 mL/min/m², and no risk factors of rapid disease progression. Participants were randomized in a 1:1 ratio by a computer-generated random number table into metformin + standard of care group (metformin arm) and standard of care group (Control arm). Primary outcome of the study was to evaluate the effects of metformin versus control arm on the percentage and absolute change in eGFR over a 6-month period.

Results: Mean (SD) age of the cohort was 37.15 (10.16) years with half of them being females. The mean (SD) baseline htTKV and eGFR were 335.67 (153.3) mL/m and 100.23 (25.95) mL/min/m², respectively. Clinical exome sequencing was available in nine (17.3%) patients of which two-thirds had PKD1 mutation. Baseline characteristics were distributed equally across randomized groups. Baseline proteinuria was significantly higher in the metformin arm (p = 0.014). The eGFR difference and percentage change in eGFR was not different between the groups at 6 months (p = 0.53 and 0.48, respectively). There was no statistically significant difference in htTKV and percentage change in htTKV at 6 months between the groups, although an increase in htTKV was numerically smaller in the metformin group (p = 0.769, 0.805). Blood pressure, body weight, body mass index (BMI), and proteinuria also did not differ between the two groups. Only half of the cohort tolerated the maximum dose of metformin. Around two-thirds of patients reported adverse effects, most commonly asthenia.

Conclusion: Metformin appears to be safe and well tolerated in nondiabetic patients with ADPKD.

Background: Atypical hemolytic uremic syndrome (aHUS) is a severe thrombotic microangiopathy predominantly affecting the kidneys, often associated with complement dysregulation. This study is aimed to analyze the clinical characteristics, treatment outcomes, and long-term implications of aHUS in a resource-limited setting.

Materials and methods: A retrospective observational study conducted at an institute between January 2016 and December 2022 included all patients with aHUS, excluding secondary causes and renal transplant recipients. Demographic profiles, clinical features, laboratory parameters, treatment modalities (immunosuppression and plasma exchange), and outcomes were collected. Anticomplement Factor H (anti-CFH) antibody, complement levels, and genetic mutation analysis were performed to ascertain etiological factors. The patient and renal outcomes of anti-CFH positive and negative patients on long-term follow-up were compared.

Results: Fifty-seven patients (mean age: 12.5 ± 4.9 years; 63% males) were analyzed. Among them, 33 (57.9%) tested positive for anti-CFH antibodies and eight presented postpartum. Initial remission was achieved in 42 (73.6%) patients, with 13 (22.8%) partial and 29 (50.9%) complete remission. The median follow-up duration was 24 months [interquartile range (IQR) 8.5-84]; 12 (21%) patients died, with two deaths during the index admission, six among nonresponders, and 4 among responders. Dialysis-free renal survival was superior in anti-CFH seropositive patients (81.2%) compared to seronegative counterparts (55.9%), while patient survival was statistically similar between the two groups. Elevated anti-CFH titers (>4000 AU/ml), age ≥16 years, female gender, and seizures predicted nonresponsiveness.

Conclusion: Anti-CFH antibody associated aHUS had better kidney outcomes than the seronegative counterparts. In resource limited settings, a combination of plasma exchange and immunosuppression showed promising results in the short and long term.

Collapsing glomerulopathy (CG), usually presents with renal dysfunction, hypertension and proteinuria. The etiology is uncertain, yet a number of associations, including many viral infections commonly have been reported. Tuberculosis (TB), one of the most common infections, is not known to cause CG. We report a case of severe renal dysfunction requiring dialysis who had collapsing glomerulopathy on biopsy and evidence of active pulmonary tuberculosis. Anti-tubercular therapy alone resulted in improvement in kidney function.

Background: There is a lack of data regarding the need for liver transplantation in end-stage kidney disease (ESKD) patients with compensated cirrhosis. Overall outcomes of isolated kidney transplants in these patients in terms of renal graft outcome, hepatic decompensation, and survival are less clear.

Materials and methods: This is the retrospective analysis of patients treated at a single center. Patients with cirrhosis with evidence of portal hypertension who underwent kidney transplantation were compared with a matched control group without chronic liver disease (CLD) who underwent kidney transplantation during the same period.

Results: Nineteen CLD patients with evidence of portal hypertension confirmed by endoscopy showing varices (8/19), hepatic venous pressure gradient (HVPG) >5 (12/19), or portosystemic collaterals on imaging (8/19) underwent kidney transplantation and were compared with 38 patients without liver disease transplanted during the same period. The discharge of creatinine was similar in both groups. The median follow-up was approximately 4 years in both groups, with the last mean serum creatinine of 1.3 and 1.37 mg/dl (unit for creatinine) in the patient and control groups (P = 0.382). Biopsy-proven acute rejections were similar [3 (15.8%) vs. 7 (18.4%), p = 1]. Two patients died in the CLD group, one due to hepatic decompensation with sepsis and the other due to cardiac cause. Four patients died in the control group (3 with sepsis and 1 with cardiac cause). Two patients had liver decompensation post-transplant (1-month post-transplant with ascites, 4 years post-transplant with ascites and hepatic encephalopathy).

Conclusion: Kidney-alone transplantation in a carefully selected population with CLD and portal hypertension has comparable outcomes to those without liver disease.