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BACKGROUND: Pregnancy-associated acute kidney injury (PRAKI) is a significant cause of maternal morbidity and mortality, particularly in low- to middle-income countries like India. The majority of patients with stage 1/2 acute kidney injury (AKI) recover without any long-term sequelae; however, the scenario changes completely when the patients are found to be dialysis-requiring. Fixed decline in glomerular filtration rate and progression to end-stage renal disease/long-term dialysis requirement can occur in the latter group of patients, posing significant health challenges and a burden to society. We tend to investigate this group of patients with short-term follow-up for renal outcomes. MATERIALS AND METHODS: This is a single-center study at a tertiary care center in northern India. We prospectively studied patients with PRAKI regarding their clinical presentation and maternal and fetal outcomes, with follow-up for three months post-discharge. Sixty-six pregnant patients with a diagnosis of AKI were admitted to our institution from 2023 to 2025. A total of 58 patients were analyzed. Maternal and fetal outcomes were noted, and patients were followed up at one month and three months post-discharge. Maternal outcomes included in-hospital mortality, complete recovery (serum creatinine <1.4 mg/dL), chronic kidney disease (CKD), and dialysis dependency at three months. RESULTS: The mean age was 27 years, with 79% aged 20-30 years and 41% primigravida. Puerperal sepsis (62.1%) was the leading cause, followed by septic abortion (15.5%) and antepartum hemorrhage (APH) and/or postpartum hemorrhage (PPH). Anuria (64%) was the most common presentation. Cesarean section occurred in 65% of cases, and 72% of AKIs were diagnosed postpartum. Complications included disseminated intravascular coagulation (DIC) and multi-organ dysfunction syndrome (MODS) in 28 patients. Fetal outcomes showed 48.3% preterm deliveries and 20.7% intra-uterine deaths (IUD)/stillbirths. Maternal outcomes included 41.4% renal recovery by three months, 27.6% mortality (20.7% in-hospital), and 31.1% progression to CKD, with 5.2% dialysis-dependent. Biopsies (15.5% of cases) revealed patchy cortical necrosis (five patients), IgA nephropathy (three patients), and Alport syndrome (one patient). Intensive care unit (ICU) stay, higher total leukocyte count, elevated serum glutamic oxaloacetic transaminase (SGOT) levels, and prolonged prothrombin time/international normalized ratio (PT/INR) were associated with maternal mortality. Dialysis dependency at discharge predicted adverse renal outcomes at three months. CONCLUSION: Dialysis-requiring PRAKI is associated with high maternal mortality, fetal morbidity, and significant progression to CKD/end-stage renal disease. Puerperal sepsis remains the commonest cause, followed by septic abortion. Early sepsis management could reduce morbidity. The study underscores the need for improved obstetric care and timely intervention in resource-limited settings to mitigate the impact of PRAKI on maternal and fetal health.

BACKGROUND: Alport syndrome is a common hereditary glomerular kidney disease characterized by a high risk of kidney failure. Despite renin-angiotensin system- (RASi) and SGLT2 inhibitors (SGLT2i), a subset of patients experience rapid decline in kidney function. Preclinical data in Alport mice suggest that the nonsteroidal mineralocorticoid receptor antagonist (MRA) finerenone could provide additional nephroprotection. This case series evaluates the changes in albuminuria and the safety of finerenone as part of a triple-therapy regimen in individuals with Alport syndrome. METHODS: In this retrospective, two-center observational case series, ten male individuals with Alport syndrome received finerenone (10 mg/day) in addition to baseline-therapy with RASi (100%) and SGLT2i (90%). Clinical parameters - including estimated glomerular filtration rate (eGFR), albuminuria, and serum potassium were analyzed over a mean follow-up period of 9 ± 5 months. RESULTS: Median albuminuria showed a non-significant decrease of 33% during follow-up, from 3506 [IQR 1645-4304] to 2346 [1858-2792] mg/g creatinine (p = 0.21). Median eGFR declined from 50 [47-112] to 43 [32-116] mL/min/1.73m2 (p = 0.03) and continued to decline in a subgroup of five individuals, who were followed for 12 months. Hyperkalemia occurred in half of the individuals, necessitating the use of potassium binders in two cases. Finerenone was discontinued in two individuals due to rapid decline of eGFR. No serious adverse events were observed. CONCLUSIONS: This real-world case series provides preliminary evidence that adding finerenone to baseline therapy with RASi and SGLT2i is feasible and may offer additional albuminuria reduction in Alport syndrome. However, the notable eGFR decline and the relevant incidence of hyperkalemia underscore the necessity for vigilant clinical monitoring. While multimodal nephroprotection remains a promising tool to delay disease progression, these real-world observations provide pilot data to inform the design of future large-scale prospective trials in patients with Alport syndrome.

BACKGROUND: The most familiar ear and kidney syndrome is Alport syndrome for a nephrologist. Mutations in the mitochondrial gene MT-TL1, which encodes UUR, can also cause renal dysfunction and hearing loss. In this study, we reported a young Chinese male presented with proteinuria and renal dysfunction with a morphological presentation of focal segmental glomerulosclerosis (FSGS) with m.3243 A > G mutation in the mitochondria in the mitochondrial gene MT-TL1. We conducted a systematic literature review to summarize previously reported cases. CASE PRESENTATION: A 17-year-old male was admitted to our hospital due to foamy urine that had persisted for three months after an upper respiratory tract infection. He also complained of weakness in both lower extremities, particularly the calves. He had progressive hearing loss and body hair growth in the last two years.His urinalysis revealed 2 + proteinuria and 24-hour urine protein of 0.85 g.His blood tests revealed increased serum creatinine of 2.1 mg/dl, blood urea nitrogen of 36.1 mg/dl, and uric acid of 12.5 mg/dl. His fasting blood glucose was within the normal range of 99 mg/dl. Renal biopsy pathology revealed changes consistent with FSGS.High-power microscopy demonstrated swollen podocytes and an increased number of dysmorphic mitochondria within renal tubular epithelial cells. Consequently, whole-exome sequencing was performed, confirming that both the patient and her mother harbor the m.3243 A > G mutation in the mitochondrial MT-TL1gene.We provide patients with treatments to improve mitochondrial energy synthesis, reduce creatinine production, promote creatinine excretion, and lower uric acid levels.After a 23-month follow-up, renal function remained stable. CONCLUSION: The UUR gene is an important tRNA gene in mtDNA. Its mutation can lead to mitochondrial dysfunction and cause a variety of diseases.The family history of patients with concurrent ear and renal diseases should be assessed in detail.