-
La Tunisie Medicale Mar 2023Focal segmental glomerulosclerosis is a histopathological entity.
INTRODUCTION
Focal segmental glomerulosclerosis is a histopathological entity.
AIM
To analyze the epidemiological, clinical and histological profile of primary focal segmental glomerulosclerosis in children, as well as their prognostic factors.
METHODS
This was a retrospective cross-sectional study over a period of 20 years (2001-2020), conducted in the Department of Pediatrics at Charles Nicolle Hospital in Tunis, which included children followed for primary focal segmental glomerulosclerosis.
RESULTS
There were 35 children, 19 boys and 16 girls. The median age was 4.5 years. Nephrotic syndrome was seen in 88% of patients. Macroscopic hematuria was found in 4 cases, hypertension in 8 cases and renal failure in 7 cases at presentation. The most common variant was the not otherwise specified variant (77.1%). Steroid-sensitive nephrotic syndrome was observed in 71% of cases, and steroid-resistance in 29% of cases. Treatment with cyclosporine was indicated in 23 patients with complete remission rate of 56.5%. 42,8% children had progressed to chronic kidney disease, including an end-stage renal disease in 11.5% of cases. Only the presence of a family history of kidney disease was found as a predictive factor of progression to chronic kidney disease and end-stage renal disease. Renal survival rates were estimated at 100% at 3 years, 85% at 5 years and 73% at 10 years.
CONCLUSION
Identification of patients at high risk for chronic kidney disease progression, such as those with a family history of kidney disease or those who have failed to respond to corticosteroids, would allow therapeutic adjustments.
Topics: Male; Female; Humans; Child; Child, Preschool; Glomerulosclerosis, Focal Segmental; Cross-Sectional Studies; Retrospective Studies; Prognosis; Kidney Failure, Chronic; Renal Insufficiency, Chronic; Nephrotic Syndrome
PubMed: 38263915
DOI: No ID Found -
Arab Journal of Urology 2023Urothelial bladder carcinoma (UBC) is usually detected during work-up for hematuria. Cystoscopy and/or contrast-enhanced imaging are the gold standard tools for UBC...
BACKGROUND
Urothelial bladder carcinoma (UBC) is usually detected during work-up for hematuria. Cystoscopy and/or contrast-enhanced imaging are the gold standard tools for UBC diagnosis, despite limited by being invasive, expensive and low yield in small flat tumors.
OBJECTIVES
To assess the diagnostic performance of urine-based DNA methylation of six genes (GATA4, P16, P14, APC, CDH1 and CD99) for UBC detection in patients with hematuria.
PATIENTS AND METHODS
Voided urine was collected from consecutive patients presented with hematuria for urine cytology and DNA methylation assay of the assigned genes using methylation-specific Polymerase Chain Reaction (PCR). Further assessment by office cystoscopy and imaging with subsequent inpatient cystoscopic biopsy for positive findings was done. The diagnostic characteristics of DNA methylation and urine cytology were assessed based on its capability to predict UBC.
RESULTS
We included 246 patients in the study with identified macroscopic hematuria in 204 (82.9%) patients. Positive cytology was found in 78 (31.7%) patients. DNA methylation of GATA4, P16, P14, APC, CDH1 and CD99 genes was identified in 127 (51.6%), 52 (21.1%), 117 (47.6%), 106 (43.1%), 90 (36.6%) and 71 (28.9%) patients, respectively. The sensitivity of the assigned genes for UBC detection ranges from 35% (95%CI: 31-39) to 83% (95%CI: 79-87). Optimal specificity (SP) (100%) was noted for P16, APC and CDH1 genes. While for the other genes (GATA4, P14 and CD99), the SP was 95% (95%CI: 92-98), 96% (95%CI: 92-99) and 97% (95%CI: 93-99), respectively. On multivariate logistic regression analysis, all genes exclusively demonstrated independent prediction of UBC. On receiver operator characteristic (ROC) analysis, all tested genes methylation showed superior area under the curve (AUC) when compared to urine cytology.
CONCLUSIONS
We have developed a novel urine-based DNA methylation assay for detection of UBC in patients with hematuria with superior diagnostic performance and independent predictive capacity over urine cytology.
PubMed: 38178946
DOI: 10.1080/2090598X.2023.2208492 -
SAGE Open Medical Case Reports 2023Congenital mesoblastic nephroma is considered a tumour with favourable clinical behaviour with only few reported cases of metastases. We report an infant who underwent...
Congenital mesoblastic nephroma is considered a tumour with favourable clinical behaviour with only few reported cases of metastases. We report an infant who underwent complete resection and later developed pulmonary metastasis. Ten-month-old baby girl initially presented at 3 weeks of age with macroscopic haematuria, hypertension and a lumbar mass. Contrast-enhanced computed tomography revealed a tumour arising from the left kidney without local invasion or metastasis. She underwent left nephrectomy. Immunohistochemistry confirmed a cellular type of congenital mesoblastic nephroma. At 10 months, she presented with difficulty in breathing. Contrast-enhanced computed tomography revealed an opacity in the right hemi-thorax. Histology of lung mass was suggestive of deposits from the previously excised mesoblastic nephroma. She developed a right-sided haemothorax and succumbed. This case report highlights the fact that even though congenital mesoblastic nephromas are considered tumours with favourable clinical behaviour, they can present later with distant metastasis. Therefore, clinicians need to be aware of this rare malignant potential and adhere to meticulous follow-up protocols.
PubMed: 38149118
DOI: 10.1177/2050313X231220826 -
Clinical Pediatrics Dec 2023We aimed to evaluate the clinical parameters, histopathological findings of nephrotic syndrome (NS) patients, and independent factors predicting steroid resistance in a...
We aimed to evaluate the clinical parameters, histopathological findings of nephrotic syndrome (NS) patients, and independent factors predicting steroid resistance in a single tertiary center. One hundred and sixty-two children (57 girls and 105 boys) with NS who were followed between 1998 and 2018 were analyzed in this retrospective cohort. The median (interquartile range; range) age and follow-up time were 4.9 (5.7; 0.1-16.8) and 5.5 (5.4; 0.1-20.3) years. A total of 82.7% of the patients were steroid-sensitive nephrotic syndrome (SSNS) and 17.3% were steroid-resistant nephrotic syndrome (SRNS). The median age at first presentation was lower in the SSNS group ( = .002). The most common histopathological findings were focal segmental glomerulosclerosis (FSGS) and minimal change disease (MCD). Hypertension and macroscopic and microscopic hematuria were higher in the SRNS group ( < .001). The age and microscopic hematuria were independent risk factors for steroid resistance ( = .019 and = .002, respectively). Complement 3 (C3) was evaluated in 148 patients and found low in 7 patients who were subsequently diagnosed as membranoproliferative glomerulonephritis. There is still no better clinical predictor for steroid response than late age of onset and microscopic hematuria. Hypertension may also give a hint for potential steroid resistance.
PubMed: 38142361
DOI: 10.1177/00099228231219109 -
Radiology Case Reports Feb 2024We report a rare case of a primary renal neuroendocrine tumor. The patient was a 64-year-old woman. The patient's chief complaint was gross hematuria. Dynamic...
We report a rare case of a primary renal neuroendocrine tumor. The patient was a 64-year-old woman. The patient's chief complaint was gross hematuria. Dynamic contrast-enhanced computed tomography (CT) revealed a hypovascular mass 13 cm in diameter in the right kidney. The border of the mass was clear. A grossly contrast-impaired area and internal granular calcification were observed. A right radical nephrectomy was performed. Macroscopically, the mass was hemorrhaged and necrotic. It was diagnosed as a neuroendocrine tumor (NET) (Grade 2) histologically. Findings, such as hypovascularity, calcification, and necrosis, in our case were similar to those in previous reports. These findings are considered relatively characteristic of primary renal NETs.
PubMed: 38074443
DOI: 10.1016/j.radcr.2023.10.069 -
Clinical Kidney Journal Dec 2023Hematuria-either macroscopic hematuria or asymptomatic microscopic hematuria-is a clinical feature typical but not specific for immunoglobulin A nephropathy (IgAN). The... (Review)
Review
Hematuria-either macroscopic hematuria or asymptomatic microscopic hematuria-is a clinical feature typical but not specific for immunoglobulin A nephropathy (IgAN). The only biomarker supported by the Kidney Disease: Improving Global Outcomes group as a predictor of progression, identifying patients needing treatment, is proteinuria >1 g/day persistent despite maximized supportive care. However, proteinuria can occur in the setting of active glomerulonephritis or secondary to sclerotic renal lesions. Microscopic hematuria is observed in experimental models of IgAN after IgA-IgG immunocomplex deposition, activation of inflammation and complement pathways. Oxidative damage, triggered by hemoglobin release, is thought to contribute to the development of proteinuria and progression. Despite being a clinical hallmark of IgAN and having a rational relationship with its pathophysiology, the value of microscopic hematuria in assessing activity and predicting outcomes in patients with IgAN is still debated. This was partly due to a lack of standardization and day-to-day variability of microhematuria, which discouraged the inclusion of microhematuria in large multicenter studies. More recently, several studies from Asia, Europe and the USA have highlighted the importance of microhematuria assessment over longitudinal follow-up, using a systematic approach with either experienced personnel or automated techniques. We report lights and shadows of microhematuria evaluation in IgAN, looking for evidence for a more consistent consensus on its value as a marker of clinical and histological activity, risk assessment and prediction of treatment response. We propose that hematuria should be included as part of the clinical decision-making process when considering when to use immunosuppressive therapy and as part of criteria for enrollment into clinical trials to test drugs targeting the inflammatory reaction elicited by immune pathway activation in IgAN.
PubMed: 38053974
DOI: 10.1093/ckj/sfad232 -
Nature Reviews. Disease Primers Nov 2023IgA nephropathy (IgAN), the most prevalent primary glomerulonephritis worldwide, carries a considerable lifetime risk of kidney failure. Clinical manifestations of IgAN... (Review)
Review
IgA nephropathy (IgAN), the most prevalent primary glomerulonephritis worldwide, carries a considerable lifetime risk of kidney failure. Clinical manifestations of IgAN vary from asymptomatic with microscopic or intermittent macroscopic haematuria and stable kidney function to rapidly progressive glomerulonephritis. IgAN has been proposed to develop through a 'four-hit' process, commencing with overproduction and increased systemic presence of poorly O-glycosylated galactose-deficient IgA1 (Gd-IgA1), followed by recognition of Gd-IgA1 by antiglycan autoantibodies, aggregation of Gd-IgA1 and formation of polymeric IgA1 immune complexes and, lastly, deposition of these immune complexes in the glomerular mesangium, leading to kidney inflammation and scarring. IgAN can only be diagnosed by kidney biopsy. Extensive, optimized supportive care is the mainstay of therapy for patients with IgAN. For those at high risk of disease progression, the 2021 KDIGO Clinical Practice Guideline suggests considering a 6-month course of systemic corticosteroid therapy; however, the efficacy of systemic steroid treatment is under debate and serious adverse effects are common. Advances in understanding the pathophysiology of IgAN have led to clinical trials of novel targeted therapies with acceptable safety profiles, including SGLT2 inhibitors, endothelin receptor blockers, targeted-release budesonide, B cell proliferation and differentiation inhibitors, as well as blockade of complement components.
Topics: Humans; Glomerulonephritis, IGA; Antigen-Antibody Complex; Galactose; Immunoglobulin A
PubMed: 38036542
DOI: 10.1038/s41572-023-00476-9 -
Nefrologia 2023
Topics: Humans; Glomerulonephritis, IGA; Hematuria; Proteinuria
PubMed: 37914637
DOI: 10.1016/j.nefroe.2023.10.003 -
Internal Medicine (Tokyo, Japan) Jun 2024A 69-year-old woman was referred to our hospital because of an acute kidney injury with macroscopic hematuria. She had been taking dabigatran for atrial flutter for six...
A 69-year-old woman was referred to our hospital because of an acute kidney injury with macroscopic hematuria. She had been taking dabigatran for atrial flutter for six years. Based on the typical histological findings of her kidney biopsy and her history of dabigatran use with prolonged activated partial thromboplastin time, she was diagnosed with dabigatran-related nephropathy complicated by tubulointerstitial nephritis with IgA nephropathy. After prednisolone therapy, the renal function improved. Direct-acting oral anticoagulants, including dabigatran, may cause anticoagulant-related nephropathy similar to warfarin, even in patients with a normal renal function. Tubulointerstitial nephritis may coexist with dabigatran-related nephropathy, and prednisolone therapy should be considered in such cases. IgA nephropathy has been reported as a background disease, and caution should be exercised when encountering it.
Topics: Humans; Dabigatran; Female; Aged; Glomerulonephritis, IGA; Nephritis, Interstitial; Antithrombins; Prednisolone; Kidney
PubMed: 37866913
DOI: 10.2169/internalmedicine.2628-23 -
Urology Case Reports Nov 2023While the historical benefits of hydrogen peroxide on wounds and wound healing have recently been questioned, physicians have started to explore its other potential...
While the historical benefits of hydrogen peroxide on wounds and wound healing have recently been questioned, physicians have started to explore its other potential medicinal benefits. We present a case of a 14-year-old girl who presented to our urology unit with macroscopic haematuria and clot retention. Ultrasonography confirmed a large organised intravesical blood clot. Despite numerous attempts, manual bladder irrigation was unsuccessful and caused significant discomfort to the patient. Her clot retention was relieved after 4 irrigation cycles with a 3 % hydrogen peroxide solution. She experienced no complications or side effects post intravesical instillation of hydrogen peroxide.
PubMed: 37842265
DOI: 10.1016/j.eucr.2023.102579