Introduction: Immunoglobulin A (IgA) nephropathy usually occurs after upper respiratory tract infection. The currently widely accepted pathomechanism involves genetic predisposition, formation of galactose-deficient IgA1, and production of autoantibodies against these IgA, ultimately leading to mesangial deposition of immune complexes. Tubulointerstitial nephritis and uveitis (TINU) syndrome is a rare disease with a suspected autoimmune mechanism, although the exact etiology and pathomechanism are not fully understood. To our knowledge, only a few cases with simultaneous occurrence of these two entities have been reported in common literature so far. Case Presentation: A 53-year-old Ethiopian woman presented with a month of postprandial nausea, vomiting, occipital headache, 5 kilogram weight loss and intermittent night sweats. Laboratory examinations revealed hematuria and impaired renal function, histological findings showed acute tubulointerstitial nephritis and glomerulonephritis. Further assessments revealed left-sided anterior uveitis as well as a Helicobacter pylori and Strongyloides infection. We present the case of a middle-aged woman with concurrent IgA nephropathy and TINU syndrome, possibly following a H. pylori and Strongyloides infection. There was rapid and significant improvement of renal disease after glucocorticoid treatment. Conclusion: Further research is needed to elucidate possible causality between IgA nephropathy, TINU syndrome, and the potential trigger factors involved.
Background Reduced ultrafiltration in peritoneal dialysis (PD) contributes to volume overload, increasing the risk of technique failure and patient mortality. The administration of a sodium–glucose cotransporter 2 inhibitor (SGLT2i) has been shown to increase ultrafiltration volume in mice, suggesting its potential role in improving ultrafiltration. However, whether SGLT2i can improve ultrafiltration and increase urine volume in nondiabetic PD patients remains unclear. Case presentation We report six nondiabetic PD patients with a dialysis history of more than three months who received 10 mg of dapagliflozin once daily orally for six months, in addition to their conventional treatment regimens. After six months, clinically significant increases in ultrafiltration were observed (from 76.8 mL at baseline to 211.3 mL), and five patients showed increased urine volume (from 433.3 mL to 646.7 mL). The glucose concentration in the effluent dialysates was higher than that at baseline. No changes in peritoneal transport characteristics, estimated glomerular filtration rate (eGFR), or residual renal creatinine clearance rate (CCr) were observed at this point compared with baseline. No adverse effects, such as hypoglycemia or abnormal liver function, were reported in any of the patients. Conclusions In this preliminary observation, dapagliflozin use was associated with increased ultrafiltration and urine volume in nondiabetic PD patients and presented a favorable safety profile. Further controlled studies are needed to confirm these preliminary findings.
Introduction: Severe hypertriglyceridemia (HTG) is a well-recognized cause of acute pancreatitis, but its role as a precipitant of acute myocardial infarction (MI) and a barrier to urgent surgical intervention is less frequently documented, especially in patients with end-stage renal disease (ESRD). Case Presentation: We report a unique case of a 68-year-old male on chronic hemodialysis who presented with an inferior wall MI. Coronary angiography revealed severe triple vessel disease necessitating urgent coronary artery bypass grafting (CABG). However, laboratory investigations identified critical HTG (1,943 mg/dL), posing significant risks for hyperviscosity and extracorporeal circuit failure. To mitigate these perioperative risks, the patient underwent four sessions of preoperative therapeutic plasma exchange (TPE), which successfully reduced serum triglycerides to 355 mg/dL. Following this stabilization, the patient underwent a successful off-pump CABG and was managed postoperatively with sustained low-efficiency dialysis. Conclusion: This case highlights the clinical utility of TPE as a rapid and effective “therapeutic bridge” to enable safe, urgent cardiac surgery in patients with severe HTG. It demonstrates that preoperative lipid-lowering aphaeresis can safely manage metabolic barriers to surgery in complex ESRD patients, potentially expanding the management options for high-risk cardiovascular cases where medical therapy alone is insufficient.
Introduction: Acute kidney injury (AKI) is defined as an increase in serum creatinine with or without a reduction in urine output, according to the Kidney Disease: Improving Global Outcomes guidelines. Oxalate nephropathy (ON) is a rare cause of AKI in children. We report a case of oliguric AKI associated with exposure to a cosmetic hair-treatment product leading to ON. To our knowledge, there are limited reports of similar pediatric cases in Saudi Arabia. Case Presentation: An 8-year-old previously healthy girl presented with facial edema, abdominal distension, oliguria, and hypertension 2 days after a single application of an unidentified hair-treatment product. Laboratory evaluation confirmed severe AKI. Renal biopsy demonstrated acute tubular necrosis with calcium oxalate crystal deposition, confirming ON. She was managed conservatively with cautious fluid administration, intravenous furosemide, correction of electrolyte abnormalities, antihypertensive therapy, and a low-oxalate diet. Renal function normalized within 1 month, with sustained recovery and no evidence of recurrence on subsequent follow-up. Conclusion: Exposure to certain cosmetic hair-treatment products may cause secondary hyperoxaluria and AKI in children, underscoring the need for heightened awareness and stricter regulation of unlabeled cosmetic products to prevent potential nephrotoxicity.
Introduction: Anti-glomerular basement membrane (anti-GBM) disease is a rare autoimmune vasculitis that often leads to rapidly progressive glomerulonephritis and poor renal prognosis. Standard treatment typically involves plasmapheresis, corticosteroids, and cyclophosphamide. However, the therapeutic role of obinutuzumab, a B-cell-depleting monoclonal antibody, remains under-explored in anti-GBM disease. Case Presentation: We report a 20-year-old male with isolated renal anti-GBM disease following a dental infection, who presented with severe acute kidney injury (creatinine >700 μmol/L), high anti-GBM antibody titers, and crescentic glomerulonephritis on biopsy. Despite initial therapy with plasmapheresis, corticosteroids, and cyclophosphamide, renal function deteriorated. Obinutuzumab (500 mg IV) was administered as adjunct therapy, leading to rapid antibody clearance within 1 month. Remarkably, the patient achieved partial renal recovery and was dialysis-independent by 3 months, with stable stage 3 chronic kidney disease at 5-month follow-up. Conclusion: This case underscores the potential role of obinutuzumab as an innovative adjunctive therapy in severe anti-GBM disease, even in patients with traditionally poor prognostic markers. Early and aggressive multimodal treatment, including B-cell targeting, may improve outcomes in selected cases.
Introduction: Chlorprothixene is a typical antipsychotic, primarily used in the treatment of psychotic disorders. Overdose may result in severe cardiovascular and central nervous system toxicity. Evidence on the effectiveness of extracorporeal elimination is scarce. Case Presentation: A 19-year-old female ingested 5 g of chlorprothixene in a suicide attempt. Upon admission to the intensive care unit, she was awake, responsive, and oriented. Initial management included 50 g of activated charcoal. As doses of >2 g chlorprothixene can cause severe intoxication and death, attempts to enhance drug elimination by extracorporeal therapy were undertaken. Therapeutic plasma exchange (TPE) was initiated approximately 3 h after admission, exchanging 5,096 mL of plasma with albumin solution and FFP over 3 h 10 min. This was followed by a 10 h 10 min prolonged intermittent kidney replacement therapy (PIKRT). Serial blood, apheresis, and dialyzate samples were collected to quantify drug removal. Both procedures were well tolerated. The patient remained clinically stable and was transferred to a psychiatric facility 24 h post-admission. Conclusion: Although chlorprothixene plasma levels decreased, the amount of chlorprothixene removed by TPE and PIKRT was negligible, classifying the drug as “not dialyzable” per EXTRIP criteria. Given the limited clinical evidence and the pharmacokinetic properties of chlorprothixene, TPE cannot be recommended for the management of chlorprothixene overdose and should not be regarded as a standard treatment option. According to the available literature, this is the first description of chlorprothixene intoxication managed with both TPE and high-flux hemodialysis, highlighting the limited extracorporeal removal of this drug.
Introduction: Peritoneal dialysis (PD)-associated peritonitis caused by rare non-fermenting Gram-negative organisms represents a diagnostic and therapeutic challenge, particularly in patients with prolonged antibiotic exposure and biofilm-associated catheter infections. Case Presentation: We report the case of a 44-year-old male PD patient who developed relapsing polymicrobial PD-associated peritonitis involving Elizabethkingia miricola, Brevundimonas diminuta, Stenotrophomonas maltophilia, and Staphylococcus haemolyticus. Despite repeated susceptibility-guided intraperitoneal antibiotic therapy, inflammatory markers and dialyzate leukocytosis persisted. Multiple cultures consistently identified rare non-fermenting Gram-negative pathogens. Following prolonged discussion, the PD catheter was removed, and the patient transitioned to hemodialysis, followed by targeted oral antimicrobial therapy. Clinical and laboratory resolution occurred within 3 weeks after catheter removal. Conclusion: Rare environmental non-fermenting Gram-negative pathogens should be recognized as clinically relevant causes of relapsing PD-associated peritonitis. Persistent or polymicrobial infections should prompt early consideration of catheter removal in accordance with current ISPD recommendations.
Introduction: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a systemic autoimmune disease characterized by diverse clinical manifestations. Central diabetes insipidus resulting from pituitary involvement in AAV is exceedingly rare. Case Presentation: This article presented a rare case of a young patient with a medical history of 8 years, who presented with sinusitis, pituitary microadenoma with central diabetes insipidus, acute renal injury, right abducens nerve paralysis (strabismus, diplopia), and most cases were not accompanied by an increase of ANCA titer. Conclusion: Our case showed that granulomatosis with polyangiitis (GPA) has a variety of clinical manifestations and rapid progression. The patient was diagnosed with GPA and treated with glucocorticoids combined with cyclophosphamide and desmopressin. Following treatment, his renal impairment, diabetes insipidus, and diplopia improved, achieving clinical remission. This case underscores the importance of timely diagnosis and treatment for the patient's prognosis.
Introduction: Chronic kidney disease (CKD) in children poses unique challenges, especially in resource-poor developing countries, with limited facilities for renal replacement therapy (RRT). We present three cases of children with end-stage renal disease from Sri Lanka in whom traditional access has been exhausted secondary to sepsis and thrombosis. Case Presentations: Three patients were not candidates for peritoneal dialysis, and several attempts for tunnelled and non-tunnelled catheters limited the ability to continue RRT until the donor work-up for transplantation was completed. Therefore, surgical placement of a vascular catheter (vascath) to enter the inferior vena cava (IVC) directly was performed under general anaesthesia. Patient 1, a nine-year-old boy, successfully maintained dialysis through the IVC catheter until transplantation. Patient 2, a fourteen-year-old boy with extensive thrombosis, underwent successful cannulation with good outcomes at 4 months' follow-up. Patient 3, a twelve-year-old girl post-nephrectomy, initially dialysed successfully but later died due to cardiac failure unrelated to the access site. This procedure provided effective haemodialysis access, mitigating severe volume overload and hyperkalaemia. They also tolerated subsequent dialysis sessions well. Conclusion: This case series highlights the critical role of unconventional dialysis access methods in resource-constrained settings, where advanced interventional radiology options may be unavailable. While IVC cannulation for haemodialysis is more commonly described in adults, these are the first reported cases of vascath insertion into the IVC in paediatric patients. Additionally, these findings underscore the importance of innovative strategies to manage paediatric CKD in low-resource settings, pending definitive renal transplantation.
Introduction: IgA nephropathy with minimal change disease (MCD-IgAN) is a rare subtype of IgAN with a high rate of response to corticosteroids but a poor prognosis in steroid-dependent (SD) patients. Case Presentation: A young male patient with SD-MCD-IgAN, who was treated sequentially with rituximab (RTX) and later with obinutuzumab (OBZ) over a 10-year follow-up period, initially presented with nephrotic syndrome and achieved rapid complete remission (CR) with full-dose corticosteroids. However, during the first 2 years, the patient experienced four relapses despite the addition of various immunosuppressants. These relapses were accompanied by complications such as skin infections, acute kidney injury, and serosal effusions. The initial renal biopsy revealed MCD-IgAN, while a repeat biopsy 8 months later revealed IgAN with focal segmental glomerulosclerosis. RTX (375 mg/m2) was introduced after remission was achieved with full-dose corticosteroids. The patient remained in CR with RTX administered based on CD19+ B-cell counts for an initial period of 3 years. Following discontinuation of medication for the subsequent 3 years, the patient experienced a relapse but achieved CR again with low-dose corticosteroids and a single dose of RTX (1.0 g). However, the patient experienced a further relapse after another 3 years of medication cessation. Subsequently, OBZ (1.0 g) was administered along with low-dose corticosteroids, leading to rapid CR and long-term medication-free status. Conclusion: In SD-MCD-IgAN, anti-CD20 maintenance therapy during CR reduces relapse and enables long-term steroid-free remission. If relapse occurs later, it can be controlled with low-dose steroids and resumed anti-CD20 therapy, re-establishing sustained remission.
Introduction:Monoclonal gammopathy of undetermined significance (MGUS) is the most common plasma cell dyscrasia, defined by clonal monoclonal immunoglobulin (MIg) in serum and/or urine in the absence of end-organ damage caused by plasma cell proliferation. By contrast, monoclonal gammopathy of renal significance (MGRS) encompasses a spectrum of kidney disorders directly or indirectly driven by MIg, with clinical phenotypes ranging from insidious proteinuria to rapidly progressive glomerulonephritis. The diagnostic ambiguity arising when MGUS coexists with renal dysfunction remains a major clinical challenge. Here, we report a rare case of a patient initially suspected to have MGRS complicated by pauci-immune crescentic glomerulonephritis (PICGN). However, this diagnosis was ultimately excluded by the absence of MIg deposits in renal tissue on histopathological examination. Case Presentation:We describe a female with concurrent PICGN and MGUS, presented with acute kidney injury, proteinuria, and hematuria, with renal biopsy revealing type III crescentic glomerulonephritis and immunofluorescence showing weak positivity for κ and λ MIg deposits. Despite initial suspicion of MGRS, immuno-electron microscopy did not confirm monoclonal light chain deposition, leading to a final diagnosis of PICGN and MGUS. Conclusions:This case underscores the importance of integrating serological, histopathological, and advanced imaging techniques to distinguish between autoimmune and plasma cell dyscrasias in renal pathology. It also emphasizes the limitations of immunofluorescence alone in diagnosing MGRS and the necessity of immuno-electron microscopy for definitive exclusion. This report calls for further research into the pathophysiological interactions between ANCA-associated vasculitis and monoclonal gammopathies, particularly in cases with overlapping renal injury features.
Introduction:Latex allergy is a leading cause of perioperative anaphylaxis, yet the risk of graft-mediated exposure during solid organ transplantation is poorly characterised. Case Presentation:We report the case of a 45-year-old man with end-stage renal disease and confirmed latex allergy who underwent kidney transplantation with a graft that had been handled with latex gloves during procurement. Following multidisciplinary discussion, the graft was repeatedly rinsed with preservation solution to reduce antigenic load. The operating theatre was prepared as latex-free, and the anaesthesia team implemented enhanced readiness for anaphylaxis. The surgery and perioperative course were uneventful, and the patient was extubated in theatre and transferred to intensive care without complication. Conclusion:This case underscores the potential for graft-mediated anaphylaxis due to latex contamination during procurement. Systematic latex avoidance from organ retrieval to implantation, along with communication between procurement and implant teams, is crucial to mitigate risk in sensitised recipients.
Introduction:This case illustrates the challenges in the diagnosis of a rare disease with an intricate orientation. The definitive genetic diagnosis was carried out after establishing crucial correlations between the preliminary clinical indications and the laboratory findings. Case Presentation:The initial presentation was myoclonic jerks. This was a direct consequence of hyperinsulinaemic hypoglycaemia (HH), and not a phenotypic characteristic described in previous case reports. Linking this to glycaemia led to the evaluation of response to fasting, where inadequate insulin suppression resulted in hypoketotic hypoglycaemia. The diagnosis of chronic kidney dysfunction associated with atypical Fanconi renal tubulopathy syndrome type 4 (FRTS4) was indicated on the basis of a decreased estimated glomerular filtration rate, nephrocalcinosis, millimetric lithiasis, rickets, and complex proximal tubulopathy. This indicated atypical FRTS4 as associated with HH and necessitated further molecular genetic testing. Conclusion:The patient was identified as a carrier of the c.187C>T (p.Arg63Trp) variant in the HNF4A gene, which is a heterozygous missense variant classified as pathogenic. This entity is rare, and the published literature reporting HNF4A gene variants associated with atypical FRTS and HH is limited. It is therefore important to report such cases to contribute to the growing body of evidence and help identify pathogenic HNF4A variants and their implications.
Introduction: Dent disease (DD) is an X-linked recessive renal disorder characterized by features of incomplete Fanconi syndrome. DD varies in clinical presentation, manifesting with proteinuria alone or in combination with nephrocalcinosis/nephrolithiasis, and with or without chronic kidney disease, posing a challenge to clinical diagnosis. The genetic basis of DD is not completely known; about 25–35% of DD cases lack mutations in the disease-causing CLCN5 and OCRL genes. This case report represents a rare example of a patient initially suspected of having DD, but through whole exome sequencing (WES) was found to harbor pathogenic variants in the WT1 and EVC2 genes, suggesting a dual-genetic etiology mimicking DD. Case Presentation: We describe a young man with a renal phenotype resembling DD associated with nephrotic syndrome, focal segmental glomerulosclerosis (FSGS), tubular microcysts, and a significant family history of kidney disease. Also present was an extrarenal phenotype with short stature, narrow chest, recurrent upper respiratory tract infections, teeth anomalies and hypertension. We identified in the WT1 gene the heterozygous ultrarare missense variant (NM_024426.6:c.1088C>T p.Thr363Met), classified as a variant of uncertain significance, and in the EVC2 gene the heterozygous nonsense variant (NM_147127.5:c.2833C>T p.Arg945Ter), classified as pathogenic. The clinical phenotype combines WT1-related FSGS with a rare tubular phenotype of Ellis-van Creveld-like syndrome (EVC). Conclusions: This case report provides insights into the phenotypic complexity of hereditary nephropathies and the diagnostic challenge posed by overlapping glomerular and tubular presentations. WES enabled us to expand our knowledge of the genetics of kidney diseases in adults and to reclassify the patient’s nephropathy.
Introduction: Light chain-mediated acute tubulointerstitial nephritis (LCTIN) is a rare and underrecognized renal manifestation of plasma cell dyscrasias, including multiple myeloma. It presents as a dense interstitial inflammatory infiltrate involving polyclonal lymphocytes and plasma cells, often mimicking other forms of tubulointerstitial nephritis and delaying the correct diagnosis. Case Presentation: A 46-year-old man was initially managed as having drug-induced acute interstitial nephritis due to NSAID use, responding only transiently to steroids. Upon relapse with worsening kidney function, hypercalcemia, and systemic symptoms, a second kidney biopsy demonstrated again an intense tubulointerstitial infiltrate and κ-light chain proximal tubulopathy. Conclusion: This case illustrates that LCTIN can mimic relapsing interstitial nephritis. Early recognition and appropriate plasma cell-targeted therapy may significantly improve renal outcomes and guide clinical management.
Introduction:Managing pregnancy in patients with end-stage kidney disease (ESKD) undergoing dialysis is challenging, with hypoalbuminemia significantly increasing risks to both maternal and neonatal outcomes. Intensive hemodialysis regimens are recommended; however, individualized and adaptive dialysis strategies, such as sequential online hemodiafiltration (OL-HDF) and intermittent HDF (i-HDF), may be required to optimize care in complex cases. Case Presentation:We report the case of a 27-year-old Japanese woman with ESKD who transitioned from OL-HDF to i-HDF during pregnancy due to progressive hypoalbuminemia at 30 + 5 weeks of gestation. Dry weight adjustments were guided by human atrial natriuretic peptide (hANP) levels, blood pressure measurements, and bioimpedance analysis. i-HDF reduced albumin loss compared to OL-HDF, stabilized maternal hemodynamics, and enabled term delivery at 39 + 1 weeks with a healthy neonate weighing 2,774 g. Bioimpedance analysis and hANP-guided adjustments allowed for precise fluid management, resulting in a total gestational weight gain of 6.4 kg. The patient developed superimposed preeclampsia during labor, which was successfully managed. Conclusion:This case demonstrates that sequential OL-HDF and i-HDF can effectively address hypoalbuminemia and fluid imbalances, contributing to successful maternal and neonatal outcomes in ESKD pregnancies.
Introduction:Lautropia mirabilis is a rare cause of peritonitis associated with peritoneal dialysis-associated peritonitis (PDAP). We report the first documented case of PDAP caused by coinfection with L. mirabilis, cytomegalovirus (CMV), and Epstein-Barr virus (EBV). Case Presentation:A 67-year-old woman with end-stage renal disease secondary to polycystic kidney disease, on continuous ambulatory peritoneal dialysis for 3 years, developed PDAP. Initial peritoneal dialysis effluent (PDE) culture grew Streptococcus salivarius, and symptoms resolved with treatment. However, she was readmitted 2 days later with recurrent PDAP. Despite 18 days of empirical antibiotic therapy and repeated negative PDE cultures, the patient's symptoms persisted. Upon her transfer to our hospital, PDE white blood cell (WBC) count was 110 × 106/L. Targeted next-generation sequencing (tNGS) of the PDE performed on day one detected L. mirabilis (16,929 reads), CMV (944 reads), and EBV (285 reads). Therapy with intravenous moxifloxacin, intraperitoneal gentamicin, and oral ganciclovir led to rapid WBC decline and clinical improvement within 48 h. After 1 week of inpatient monitoring, the patient was discharged with a 2-week course of oral moxifloxacin. At the 2-week follow-up, the patient was asymptomatic with normal PDE WBC counts. Conclusion:Conventional culture methods may fail to detect uncommon pathogens, such as L. mirabilis. Culture-negative PDAP often necessitates empirical antibiotic therapy, carrying a high risk of failure and increased healthcare costs. This case suggests that tNGS could be used as a complementary diagnostic tool in selected cases of refractory, culture-negative PDAP, potentially aiding the identification of pathogens and guiding therapy.
Introduction:Sepsis remains a critical global health issue, causing multiple organ failure and high mortality rates, despite advances in antimicrobial therapies and supportive care. Extracorporeal blood purification (EBP) techniques have emerged as promising adjunctive strategies for the management of severe infections. The Seraph® 100 Microbind® Affinity Blood Filter (Seraph 100, ExThera Medical, Martinez, CA, USA) targets pathogens, while the Selective Cytopheretic Device (SCD, SeaStar Medical, Denver, CO, USA) neutralizes activated leukocytes. Although individually validated, evidence of the combined use of Seraph 100 and SCD remains scarce. Case Presentations:We present two cases that illustrate the combined use of Seraph 100 and SCD. The first case involves a 43-year-old woman with bacterial pneumonia, septic shock, and acute kidney injury (AKI). She underwent Seraph 100 hemoperfusion followed by SCD therapy, which improved her hemodynamics, oxygenation, and renal function, ultimately leading to full recovery. The second case involved a 31-year-old man with influenza, severe hypoxemia, and multi-organ failure. Despite advanced therapies, including veno-arterial venous extracorporeal membrane oxygenation, Seraph 100, and SCD, his condition deteriorated, resulting in multi-organ failure and eventual death. Conclusion:These cases highlight the potential benefits and challenges of combining EBP, such as Seraph 100 and SCD. While successful in one case, the fatal outcome in the second underscores the importance of optimal patient selection, timing, and therapeutic strategies. Further research is needed to evaluate the efficacy of combined EBP and to identify approaches for improving outcomes in critically ill patients.
Introduction:Sickle cell disorders are the most common hereditary hematological disorders; sickle cell trait (SCT) is largely benign with mild clinical manifestations, if any. Renal cortical necrosis (RCN) is a rare and severe form of kidney injury and, to our knowledge, has not been previously reported to affect the native kidneys of patients with SCT. Case Presentation:We describe a case of a 41-year-old male with a background of SCT who presented with acute abdominal pain and lower abdominal tenderness. He had rapidly rising creatinine over 48 h from 229 to 526 µmol/L, as well as elevated lactate dehydrogenase and total bilirubin at 2,606 U/L and 31 µmol/L, respectively. His toxicology, viral, and autoimmune profiles were negative, with a normal kidney ultrasound scan. The kidney biopsy revealed diffuse RCN. The patient was managed conservatively and had partial recovery of his kidney function to a baseline creatinine of 176 µmol/L 6 months later. Conclusion:Although SCT has long been considered a benign condition, growing evidence suggests that vaso-occlusive manifestations can occur, especially in the context of physiological stressors. This is the first described case of diffuse RCN affecting the native kidneys of a patient with SCT without an identifiable stressor, highlighting the need for vigilance in managing SCT and its potential severe kidney manifestations.
Introduction: Unilateral renal polycystic disease is a rare condition, first described in 1964, with over 60 cases reported worldwide. In 2019, Yoshida et al. [Eur Radiol. 2019;29(9):4843–50] identified 14 patients with unilateral renal cysts, who exhibited distinctive clinical and radiological features: young adults with multiple unilateral subcapsular cortical hemorrhagic (MUCH) cysts in the left kidney. The epidemiology, etiology, clinical significance, and prognosis of this condition remain largely unknown. Case Presentation: We report a 36-year-old male who presented with nephrotic syndrome, generalized edema, and impaired renal function, requiring renal replacement therapy. Magnetic resonance imaging revealed unilateral subcapsular hemorrhagic cysts in the left kidney, with nearly normal renal size. A percutaneous biopsy of the right kidney showed acute tubular necrosis and focal segmental glomerulosclerosis, tip lesion variant. The patient achieved complete remission shortly after initiating immunosuppressive therapy, with full recovery of renal function. Whole exome sequencing did not reveal any pathogenic variants. A 99Tcm-DMSA scintigraphy, performed 20 months after the initial presentation, showed a renal uptake of 11.3% (reference range: 30 ± 6%) in the affected kidney, with normal uptake in the right kidney. Conclusion: MUCH cysts in the left kidney, observed in young adults, may represent a novel clinical entity. However, the epidemiology, etiology, clinical implications, and prognosis of this condition remain to be fully elucidated.
