Luspatercept is a recombinant fusion protein that enhances late-stage erythropoiesis by inhibiting select transforming growth factor β (TGFβ) superfamily ligands. It is approved for transfusion-dependent β-thalassemia and myelodysplastic syndromes with ring sideroblasts. Kidney toxicity has been rarely reported in humans, although glomerular lesions have been described in preclinical studies. We report the case of a 74-year-old woman with myelodysplastic syndrome with ring sideroblasts treated with luspatercept for persistent anemia. She developed acute kidney injury, glomerular-range proteinuria, microscopic hematuria, and leukocyturia. Renal biopsy revealed a diffuse mesangial sclerosing glomerulopathy characterized by prominent mesangial matrix expansion with minimal proliferative changes, no immune complex deposits on immunofluorescence or electron microscopy. Luspatercept was discontinued, leading to a partial improvement in renal function but persistent proteinuria. This case documents a non-immune mesangial sclerosing glomerulopathy temporally associated with luspatercept therapy. Only one other case of biopsy-proven renal injury linked to luspatercept has previously been published, with an immune complex-mediated MPGN. Clinicians should be aware of possible renal involvement during luspatercept treatment and consider systematic monitoring of kidney function and urinalysis in treated patients. Further data are needed to clarify the spectrum and mechanisms of renal adverse events associated with this drug.

Introduction and Importance: Foreign bodies (FBs) in the bladder can result from iatrogenic causes, migration from adjacent organs, penetrating injuries, or self-insertion. They serve as a nidus for calcification and stone formation. While bladder stones related to FBs are occasionally seen in adults, such cases are extremely rare in children. Presentation of Case: A 2.5-year-old girl (10 kg) presented with chronic irritability, abdominal pain, dysuria, and intermittent hematuria for 1 year, unresponsive to antibiotics and antipyretics. Examination was unremarkable, with a leukocyte count of 9900. Urinalysis showed microscopic hematuria and pyuria. Ultrasound revealed a 33 mm echogenic structure in the bladder, which was confirmed by a kidney, ureter, and bladder (KUB) radiograph as a calcified needle. No history of trauma or abuse was reported, and the child’s development was normal. Open cystolithotomy was performed, successfully extracting the needle. Postoperatively, a urethral catheter was retained for 2 days and antibiotics were administered. The patient was discharged in good condition on day 3. Clinical Discussion: Bladder FBs in children are rare and present with nonspecific urinary symptoms. Imaging is essential for diagnosis, while management depends on the characteristics of the object and available resources. Endoscopic removal is ideal, but open surgery may be necessary in resource-limited settings or when dealing with rigid, sharp objects. Conclusion: Prompt recognition and early imaging are essential in pediatric bladder FBs. Timely surgical management prevents avoidable complications that LMIC health systems are less equipped to manage once advanced disease develops.

Acute tubulointerstitial nephritis (ATIN) is defined by the presence of histological lesions affecting the renal interstitium and tubules, most often secondary to a local inflammatory reaction associated with leukocyte infiltration. The causes are numerous. We report a case of ATIN secondary to abuse of “pouffa” (a cocaine-derived drug). The patient was an 18-year-old man admitted for severe renal failure with a serum creatinine level of 348 mg/L, associated with proteinuria of 1.46 g/24 h, active urinary sediment with microscopic hematuria, and sterile leukocyturia. A renal biopsy showed edematous interstitial tissue with inflammatory infiltrates and tubulitis lesions consistent with ATIN. The patient required emergency hemodialysis sessions and was subsequently treated with corticosteroid therapy, leading to complete recovery of renal function after two weeks. The effects of pouffa on the kidneys are devastating. Early diagnosis and prompt management are the key to full recovery of renal function.

Microscopic hematuria as a marker of glomerular injury rather than a direct cause of kidney damage.

Polycystin-1 (PC1), encoded by the PKD1 gene, forms a complex with polycystin-2 (PKD2; 173910) that regulates multiple signaling pathways to maintain normal renal tubular structure and function. Mutations in the PKD1 gene are the primary cause of type 1 PKD (polycystic kidney disease), accounting for 78%–85% of all PKD cases. In this study, we report a case of a boy presenting with microscopic hematuria with multiple renal cysts and carrying an unreported intronic variant, c.12445-34_12445-10del, in the PKD1 gene inherited from his father who also presented PKD. Sanger sequencing and reverse transcription polymerase chain reaction (RT-PCR) for minigene splicing assays showed two abnormal splicing alterations with the c.12445-34_12445-10del variant at the mRNA level: one causes a 16-bp deletion in exon 46, resulting in premature protein termination (p.Phe4149GlyfsTer45), and the other results in a 205-bp deletion, leading to delayed termination (p.Phe4149ProfsTer139). Based on the clinical characteristics and gene mutations with functional verification, the patient was finally diagnosed with PKD caused by PKD1 function defection, as confirmed by the combined clinical features and genetic analysis. Management strategies include dietary management, blood pressure monitoring, and regular follow-up of kidney function. This is the first study to report an intronic deletion in the PKD1 gene that influences alternative splicing. Our findings expand the mutation spectrum leading to PKD1-related diseases and highlight the importance of genetic counseling for the family.

An 11-year-old male neutered domestic shorthair cat was evaluated for severe hematuria causing urethral obstruction and marked anemia. Percutaneous cystoscopy identified hemorrhagic jets of urine issuing from the right ureterovesical junction (UVJ), raising concern for renal hematuria. Focal bleeding was observed from the bladder wall and suspected to be iatrogenic. Under cystoscopic and fluoroscopic guidance, a 2.5 Fr nephrostomy tube was advanced retrograde over a guidewire to mid-ureter and used to infuse silver nitrate and povidone iodine into the renal pelvis. A urethral catheter was used to perform sclerotherapy in the bladder. The cat showed substantial improvement in microscopic hematuria, but recurrence of hematuria was observed 80days later. Repeat percutaneous cystoscopy and examination of the UVJs showed multiple jets of normal urine and newly noted bladder wall nodules, diagnosed as urothelial carcinoma. We describe successful renal sclerotherapy that could be refined and used for future cases of renal hematuria in cats.

Autosomal dominant polycystic kidney disease (ADPKD) is the most common genetic kidney disease, accounting for approximately 5% of kidney failure worldwide. While Alport syndrome (AS) is an inherited progressive disease that typically presents with microhematuria in childhood and subsequently progresses to hematuria, proteinuria, and progressive renal impairment. The coexistence of ADPKD and AS is rare. Here, we present a case of a child with ADPKD and AS who presented with bilateral renal cysts and hematuria. Furthermore, we described the clinical and genetic characteristics of the present case and summarized the above features of previously reported cases. A 4-year-old boy had persistent microscopic hematuria and intermittent gross hematuria for 1year. During routine examinations, microscopic hematuria (++), bilateral renal cysts, and high-frequency hearing loss were discovered. Furthermore, His mother had microscopic hematuria and was later diagnosed with ADPKD. A pathogenic variant was found in COL4A5 through genetic testing, while a variant of undermined significance (VUS) was discovered in PKD1. These clinical and genetic findings are consistent with the diagnosis of ADPKD and AS co-occurring. Enalapril was administered. A 2-year follow-up revealed that the patient had continuous microscopic hematuria and normal range renal function. We also reviewed four cases of ADPKD and AS coexisting. Moreover, hematuria and decreased renal function were the most common clinical features. They all had a positive family history. This disease has a poor prognosis, with three cases progressing to kidney failure. ADPKD and AS are often associated with hematuria, renal cysts, and decreased renal function. Comprehensive clinical information and early genetic testing is critical for diagnosis, prognostic stratification, and initiating targeted therapies to delay progression.

Background: Acute Post-Streptococcal Glomerulonephritis (APSGN) is a common cause of acute glomerulonephritis in children, characterized by hematuria, edema, hypertension, and oliguria following a recent Group A β-hemolytic streptococcal infection. Although the majority of patients recover uneventfully, some present with atypical or severe manifestations such as acute kidney injury (AKI), hypertensive encephalopathy, or congestive cardiac failure, increasing morbidity and hospitalization. Objective: To describe the clinical features and laboratory parameters of APSGN in children admitted to a tertiary care hospital and determine the complications of acute kidney injury. Methods: This cross-sectional analytical study was conducted in the Department of Pediatric Nephrology, Bangladesh Shishu Hospital and Institute, Dhaka, from September 2019 to August 2020. Fifty children aged 3–18 years with APSGN were enrolled based on clinical and laboratory criteria. Data were analyzed using SPSS version 22.0, and p<0.05 was considered statistically significant. Results: Fifty children with APSGN were studied; 37 (74%) were male and 13 (26%) female. A preceding sore throat was reported in 54% and pyoderma in 42%. Facial edema was the most frequent feature (98%), followed by vomiting (50%), abdominal pain (50%), dyspnea (36%), headache (32%), and convulsions (20%). Hypertension was present in 86% of cases, with 70% classified as stage II. Hematuria and tachycardia were each observed in 78%, while proteinuria and oliguria occurred in 68%. Mean ASO titer was 712 ± 707 IU/mL, and complement C3 was reduced (0.26 ± 0.21 g/L). The mean serum creatinine and urea levels were 16.9 ± 27.5 mg/dL and 13.5 ± 14.3 mmol/L, respectively. Hypoalbuminemia (mean = 27.98 ± 5.18 g/L) and mild hypocalcemia were also noted. Urinalysis showed microscopic hematuria and proteinuria in most patients. Among complications, urinary tract infection was most common (30%), followed by acute kidney injury (24%), hypertensive encephalopathy (20%), and nephrotic syndrome (18%). Septicemia, metabolic acidosis, and congestive heart failure were each observed in 14% of cases. Conclusion: Most patients were from rural, illiterate, low-socioeconomic backgrounds, with skin infection being the leading precipitating cause of acute glomerulonephritis. Edema, oliguria, hematuria, and hypertension were the common presenting features. While the disease is often self-limiting, a significant number developed serious complications, especially in resource-limited settings. Early diagnosis, close monitoring, timely management, and strengthened prevention of streptococcal infections are crucial to reducing morbidity and disease burden.

Drug-induced phospholipidosis (DIP) is characterized by intracellular accumulation of phospholipids with lamellar body formation secondary to drug-altered lipid metabolism, which can trigger inflammation and histopathological changes. Fabry disease and DIP both exhibit zebra bodies on electron microscopy, complicating differential diagnosis. A 17-year-old male with microscopic hematuria and proteinuria had received atomoxetine (40 mg) for 11 months to treat attention-deficit hyperactivity disorder. Light microscopy showed one glomerulus with perihilar sclerosis and periglomerular fibrosis. Kidney biopsy revealed zebra bodies in podocytes, initially suggesting Fabry disease. However, α-galactosidase A enzyme activity was normal on tandem mass spectrometry. Next-generation sequencing of GLA identified only three benign variants. This represents the first reported case of atomoxetine-induced DIP. When zebra bodies are observed, clinicians should consider DIP caused by cationic amphiphilic drugs alongside Fabry disease. Atomoxetine meets the structural criteria for inducing DIP, and awareness of this potential complication is essential.

BACKGROUND Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a rare pediatric condition that can present with rapidly progressive glomerulonephritis and is characterized by necrotizing crescentic glomerulonephritis. While there are no specific pediatric treatment guidelines, rituximab has shown promising benefits in the management of pediatric AAV. This report presents a case of AAV in a 6-year-old girl treated with rituximab and mycophenolate mofetil, resulting in remission that was maintained at 5-year follow-up. CASE REPORT A 6-year-old girl with eczema herpeticum presented with progressive bilateral lower extremity pain, weakness, and intermittent fever and later developed microscopic hematuria. Initial workup revealed proteinuria, elevated inflammatory markers, and renal biopsy findings of pauci-immune crescentic glomerulonephritis, with positive perinuclear ANCA and myeloperoxidase antibodies, confirming AAV. She was initially treated with corticosteroids without improvement, but subsequent rituximab induction followed by mycophenolate mofetil maintenance resulted in clinical improvement. At 5-year follow-up, she remained in remission while receiving enalapril and annual rituximab therapy. CONCLUSIONS This report presents a rare occurrence of AAV in a pediatric patient and demonstrates the challenges in treating this condition given the lack of pediatric-specific treatment protocols. Further research and case reports are essential in the development of standardized and evidence-based treatment strategies tailored to the pediatric population. This case highlights that AAV, while rare in children, can be effectively managed with rituximab and mycophenolate mofetil to achieve long-term remission.

Ureteric squamous cell carcinoma in a duplex collecting system presenting with chronic obstructive uropathy: a rare case report

Objective:Urolithiasis is a multifactorial disease that causes symptomatology and needs to be managed depending on various clinical parameters. In this study, we aimed to investigate clinical factors for early operation in patients with uncomplicated symptomatic urolithiasis.Materials and Methods:Medical records of 148 patients who underwent ureterorenoscopic lithotripsy (URS) within 6 weeks of diagnosis of urolithiasis were retrospectively reviewed. The patients were divided into two groups: those operated early (≤3 days, n = 80) and those operated late (≥4 days, n = 68). Data, including age, sex, body mass index, degree of pain assessed with Visual Analog Scale (VAS), radiological and laboratory examinations at the time of admission, along with perioperative surgical data, were recorded.Results:110 (74.3%) males and 38 (25.7%) females with a median age of 38 years were operated on for uncomplicated ureteral stones. Median time from diagnosis to ureteroscopic intervention was 3 days. Among clinical factors, only the number of readmissions to the emergency room, the mean white blood cell count, the level of microscopic hematuria, and VAS scores of pain at the initial diagnosis were significantly higher in the early operated group (P < 0.05).Conclusions:Pain is a major clinical factor resulting in earlier URS in uncomplicated upper urinary tract stone disease. First admission to the emergency room due to renal colic with multiple readmissions after diagnosis, high level of microscopic hematuria, and white blood cell counts appear to be significant pain-related factors for earlier operation.

Background PAX2 is a key developmental gene, and its mutations are primarily associated with kidney and ocular anomalies, predominantly affecting children. This study aims to analyze the clinical manifestations and genetic characteristics of children with PAX2 mutations and to assess the functional impact of novel variants. Methods Clinical data were retrospectively reviewed in 10 children diagnosed with PAX2 mutations through whole-exome sequencing from a pediatric hereditary disease cohort database. AlphaFold 3 (AF3) was used for protein structural modeling. Novel variants were functionally assessed via HK-2 cell proliferation assays. Results The median age at initial presentation was 4.2 years (IQR 0–6.5). All 10 patients presented with proteinuria or microscopic hematuria. Nine had kidney dysplasia, and five progressed to stage 5 chronic kidney disease. Five patients had PAX2-related ocular abnormalities. Hepatic dysfunction and spermatic cord hydrocele were reported as potential novel phenotypes. A total of nine distinct PAX2 mutations were identified, including five novel variants. AF3 modeling revealed significant conformational disruptions in the novel variants, with root mean square deviation (RMSD) values ranging from 1.1 to 43.6 Å. Functional assays demonstrated that four novel variants significantly impaired the proliferative capacity of HK-2 cells. Conclusions This study characterizes five novel PAX2 variants with confirmed structural (RMSD 1.1–43.6 Å) and functional (HK-2 proliferation impairment) impacts, expanding both the mutational and phenotypic spectra in Chinese children. The findings highlight the association between PAX2 mutations and early-onset kidney disease with potential extrarenal involvement. Early genetic diagnosis and timely kidney-protective interventions are essential to improve outcomes.

Membranous-like glomerulopathy with masked Immunoglobulin G (IgG) kappa deposits (MGMID) is a recently described rare entity. MGMID is characterized by a membranous pattern of kidney injury with monoclonal IgG kappa restriction and is recognized and "unmasked" by pronase digestion on formalin-fixed paraffin-embedded tissue using immunofluorescence staining. This technique is necessary to identify peculiar forms of glomerular immune complex deposition, which is essential for diagnosing MGMID. Patients with MGMID are usually young adult women. We report the case of a 9-year-old boy who was referred for evaluation of proteinuria and hematuria. The patient initially showed isolated microscopic hematuria. However, following an infectious episode, macrohematuria developed, leading to the decision to perform a kidney biopsy. A kidney biopsy showed a membranous pattern of injury characterized by immune deposits distributed segmentally in the subepithelial region, forming spikes in the glomerular basement membrane in all glomeruli, and segmental mesangial hypercellularity on light microscopy. Only segmental deposits of C3 were present along the glomerular basement membrane, as shown by immunofluorescence performed on fresh frozen tissue. Paraffin immunofluorescence showed positive IgG and kappa deposition. Only IgG1 deposits were positive among the IgG subclasses. Electron microscopy showed segmental subepithelial electron-dense deposits. Eventually, the patient was diagnosed with MGMID. To the best of our knowledge, this case of MGMID in a 9-year-old boy is the youngest case documented to date. The findings from our case suggest that immunofluorescence staining with pronase digestion on formalin-fixed paraffin-embedded tissue is required in pediatric patients with a membranous pattern of kidney injury showing only C3 staining by routine immunofluorescence on fresh frozen tissue.

Abstract There have been several reports of newly diagnosed glomerulonephritis (GN) following messenger RNA (mRNA) coronavirus disease 2019 (COVID-19) vaccines in adult patients. Our patient is a previously healthy 31-year-old man who presented with generalized body swelling and hypertension 2 weeks after receiving the first dose of the Pfizer-BioNTech vaccine. A kidney biopsy was performed, and it revealed cellular crescents, mesangial hypercellularity and deposits. Given the presence of a first-degree relative with immunoglobulin A nephropathy (IgAN) in the family, kidney biopsy findings, normal complements, and negative antibody screening, patient was diagnosed with IgAN. Patient was treated with intravenous methylprednisolone 1 g for 3 days followed by oral prednisolone 60 mg daily with a slow taper. He also received a total of six 1 gm IV cyclophosphamide doses. The laboratory assessment at 1-year follow-up, revealed an improvement in serum creatinine from 210 to 128 µmol/L, a decrease in proteinuria from 6 to 1.2 g/day, and a complete resolution of microscopic hematuria. Although it is difficult to establish a causal relationship between mRNA COVID-19 vaccination and GN, we may recommend evaluating the risk/benefit ratio of COVID-19 vaccination (the second/third dose) in patients who’ve already developed new-onset or recurrent GN after the first/second dose.

Timeline for Resolution of Hematuria Following Treatment of Urinary Tract Infection and Implications for Confirmatory Testing.

Confirmatory Microscopic Urinalysis After Positive Dipstick Microhematuria: Adherence Patterns and Quality Improvement.

Impact of Hematuria Risk-Stratification Guidelines on CT Urography Detection of Upper Urinary Tract Malignancy

Early-onset nephrolithiasis and persistent microscopic hematuria in a child with a novel pathogenic COL4A5 variant in Alport syndrome: A case report and literature review

Critical Analysis of the AUA 2020/2025 Microscopic Hematuria Guidelines to Predict Urothelial But Not Renal Cortical Neoplasms and Validation of the Individual Clinical Components in Risk Stratification for Urothelial Neoplasms But Not Renal Cortical Neoplasms.