BMC Nephrology最新文献

Background: Acute oxalate nephropathy is a rare but potentially underrecognized cause of acute kidney injury (AKI). The reports of secondary oxalate nephropathy induced by high doses of vitamin C, portulaca oleracea, Peanut were described, but the cause as yellow wine consumption are rare. We report a 63-year-old man with acute kidney injury due to yellow rice wine. The patient has drunk yellow rice wine 500 mL/day for the past 10 years. He underwent an ultrasound-guided renal biopsy, which showed acute tubular injury and birefringent crystals were observed in the renal tubules under a polarizing microscope. The 24-hour urinary oxalate excretion established and the baseline serum creatinine of 407 µmol/L. Following 3 months therapy, subsequent laboratory evaluation demonstrated significant reduction in urinary oxalate excretion excretion and serum creatinine normal.

Conclusions: Acute secondary oxalate nephropathy due to excessive dietary intake of oxalate may lead to AKI. Kidney biopsies in unknown cause AKI patients is important and attention should be payed to food behaviors when reasons for AKI are explored.

Mercury intoxication is not uncommon and often presents with diverse symptoms of multiple systems. While neurological disorders and renal impairments have been examined in isolation, the concurrent occurrence of systemic symptoms linked to immune dysregulation is infrequently observed. Here, we report an unusual case that a 55-year-old male patient, who is a scrap merchant, was admitted to our center for neuropsychiatric disturbances, including incoherent speech and hallucinations. He was initially diagnosed with autoimmune encephalitis (AE) because of double positivity for CASPR2 and LGl1 antibodies in serum. The patient later presented with pruritus and nephrotic syndrome, where renal biopsy revealed membranous nephropathy (MN). In view of the mercury exposure history and elevated urinary mercury level, AE and MN were suspected to be related to mercury poisoning. The patient achieved a full recovery following a four-month treatment regimen comprising immunosuppressants and mercury-chelating agents, underscoring the significance of recognizing environmental toxins such as mercury in the coexisting diseases of different systems such as AE and MN.

Background: Mycosis fungoides (MF) is a common T-cell lymphoma that primarily affects the skin. Renal involvement is rare and has never been reported as the initial extracutaneous site. T-cell clonality testing is essential for confirming systemic involvement. We report identical T-cell clonality in both the skin and renal involvement of MF, accompanied by a review of the literature on MF involvement in the kidneys.

Case presentation: A 58-year-old man with folliculotropic MF had asymptomatic bilateral kidney lesions incidentally detected on a routine magnet resonance imaging (MRI) 15 years after primary diagnosis. Immunohistochemistry (IHC) and polymerase chain reaction (PCR) confirmed clonal T-cell populations in skin and kidney biopsies, verifying systemic involvement. A Positron Emission Tomography (PET) scan showed a 50% reduction in kidney lesions after four months of therapy with Liposomal doxorubicin (20 mg/m²). However, despite this initial response, the disease spread to the lungs and pancreas, and the patient passed away eight months after kidney infiltration.

Conclusion: This is the first documented confirmation of MF involvement to the kidneys through specific IHC and T-cell PCR-confirmed clonality testing. It highlights advances in therapy for localized disease and underscores the importance of confirming T-cell clonality, especially in atypical sites like the kidneys, illustrating its potential to enhance targeted therapy in disseminated MF.

Background: Hypokalemia can result from various causes, with diarrhea being one of the most common. Although rare, chronic hypokalemia can lead to severe acute kidney injury (AKI) that requires dialysis. Therefore, this case study aims to investigate a patient with rectal cancer who, after concurrent chemoradiotherapy and ileostomy, developed chronic hypokalemia owing to prolonged diarrhea, leading to severe AKI with anuria.

Case presentation: A 64-year-old man with a history of rectal cancer, ileostomy, and hypertension was admitted for severe AKI with anuria. He had developed severe hypokalemia due to chronic diarrhea. Despite having no prior kidney disease, his serum creatinine increased to 4.8 mg/dL, and potassium dropped to 2.2 mmol/L. Initial treatment included hemodialysis for anuric AKI with metabolic acidosis. A kidney biopsy revealed renal tubular vacuolization and With-no-lysine kinase (WNK) bodies in the distal tubules, which are characteristic of hypokalemic nephropathy. Potassium replacement therapy led to a gradual recovery of potassium levels and kidney function.

Conclusion: This case highlights the importance of timely diagnosis and management of hypokalemic nephropathy through kidney biopsy.

Background: The association between membranous nephropathy (MN) and malignant tumors has long been focused. However, most existing studies have primarily concentrated on patients diagnosed with malignant tumors within a limited timeframe, typically defined as one year before or after the diagnosis of MN. This narrow focus only captures a subset of MN patients complicated by malignant tumors, leaving those diagnosed outside this timeframe understudied and largely unexplored. In the present study, we aim to comprehensively investigate the clinicopathological characteristics of MN patients complicated with malignant tumors and to develop an effective predictive model for identifying the risk of malignancy in MN patients.

Methods: A retrospective analysis was conducted on the demographic, clinical, and pathological characteristics of 174 MN patients complicated with malignant tumors and 604 idiopathic membranous nephropathy (IMN) patients without malignant tumors. All patients were randomly allocated into a training cohort (n = 584) and a validation cohort (n = 194) in a 3:1 ratio. A predictive model was developed using regression analysis, and its performance was evaluated in terms of discrimination, calibration, and clinical utility through the area under the ROC curve (AUC), calibration curve, and decision curve analysis (DCA).

Results: MN patients complicated with malignant tumors demonstrated significantly increased deposition rates of glomerular IgG1, IgG2, IgG3, and PLA2R, as well as decreased deposition rates of IgG4. Based on independent risk factors, a predictive model was developed, which exhibited excellent performance upon validation.

Conclusion: In this largest cohort to date of MN patients with malignant tumors, a predictive model was constructed using pathological parameters to estimate the risk of malignancy effectively. This tool aims to assist clinicians in decision-making and improve the prognosis of high-risk MN patients by facilitating tumor screening at the time of initial diagnosis.

Background: Membranoproliferative glomerulonephritis, with its immune complex variety and C3 glomerulopathy, is a rare glomerular disease in children. The objective of this study was to determine the clinical features and short-term outcomes in children.

Methods: This retrospective cohort study was conducted at the Department of Pediatric Nephrology, Sindh Institute of Urology and Transplantation, Karachi, from January 2020, to June 2022. All the children with membranoproliferative lesions identified via light microscopy and less than 18 years were included.

Results: A total of 35 children were diagnosed MPGN, 7 (20%) with C3 glomerulopathy and 28 (80%) idiopathic immune complex MPGN. In the IC-MPGN group, 14 patients (50%) had crescentic glomerulonephritis. Induction therapy consisted of cyclophosphamide and methylprednisolone followed by steroids, azathioprine was prescribed for maintenance phase. At the 18-month follow-up, 9 (64%) patients were in complete remission (CR), 3 (21%) were in partial remission (PR), and 2 (15%) progressed to chronic kidney disease. The remaining 14 (50%) had non-crescentic idiopathic IC-MPGN and were prescribed steroids only, cyclophosphamide with steroids and angiotensin converting enzyme inhibitors. The outcomes at 18 months were relatively poorer than those with the crescentic variety. Four (28%) patients achieved CR, 8 (56%) PR, and 2 (14%) did not respond. In the C3 glomerulopathy cohort, 3 (43%) had crescentic glomerulonephritis, one child was in CR, and two were in PR. The non-crescentic C3G were kept on ACEI 3 (43%) and Mycophenolate mofetil 1 (14%). One child treated with ACEIs achieved a PR, two were in CR, and one child treated with MMF did not respond.

Conclusions: The outcome of MPGN (immune complex and C3G) is quite variable, and aggressive therapy for crescentic glomerulonephritis may show a favourable response. Considering the similar clinical presentations and patient outcomes, C3G and IC-MPGN might represent two facets of the same disease.

Background: Pediatric lupus nephritis is a rare glomerular disease with paucity of data on short and long term outcomes. This single center study aims to assess the outcomes at 12 months and the last follow-up visit.

Methods: This retrospective review of medical charts was done to include children diagnosed with lupus nephritis at Sindh Institute of Urology and Transplantation Karachi from July, 2015 to December, 2022.

Results: Twenty five children included in the analysis had mean age of 11.5 ± 3.5 years with predominant 20 (80%) girls. The most common clinical presentation was nephrotic syndrome in 15 (60%). The means of estimated GFR and serum albumin improved from baseline to 12 months, however serum albumin showed statistically significant improvement (121 ml/min/1.73 m² ± 77 to 130 ml/min/1.73 m² ± 57, -9.2, p-value 0.53 and 2.1 gm/dl ± 0.81 to 3.5 ± 0.73, - 1.4 p-value 0.00). The choice of induction drug had no impact on composite outcome with similar complete remission rates in MMF versus Cyclophosphamide and Calcineurin inhibitors groups (4/10, 40% versus 6/15,40%; p-value 0.81). The failure of complete remission of proteinuria at 12 months was statistically associated with poor composite outcome at last follow-up visit (p-value 0.02).

Conclusion: In our study, the choice of induction regimens had no impact on overall outcome. However, we identified the importance of targeting and reducing proteinuria to improve outcomes in pediatric patients with lupus nephritis.

Acute kidney injury (AKI) is a clinical challenge characterized by elevated morbidity and a substantial impact on individual health and socioeconomic factors. A comprehensive examination of the molecular pathways behind AKI is essential for its prevention and management. In recent years, vigorous research in the domain of AKI has concentrated on pathophysiological characteristics, early identification, and therapeutic approaches across many aetiologies and highlighted the principal themes of oxidative stress, inflammatory response, apoptosis, necrosis, and immunological response. This review comprehensively reviewed the molecular mechanisms underlying AKI, including oxidative stress, inflammatory pathways, immune cell-mediated injury, activation of the renin-angiotensin-aldosterone (RAAS) system, mitochondrial damage and autophagy, apoptosis, necrosis, etc. Inflammatory pathways are involved in the injuries in all four structural components of the kidney. We also summarized therapeutic techniques and pharmacological agents associated with the aforementioned molecular pathways. This work aims to clarify the molecular mechanisms of AKI thoroughly, offer novel insights for further investigations of AKI, and facilitate the formulation of efficient therapeutic methods to avert the progression of AKI.