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Frontiers in Medicine 2021Glomerular C4d deposits are associated the severity and outcomes of IgA nephropathy. Whether this holds true in immunoglobulin A vasculitis (IgAV) is not known. The...
Glomerular C4d deposits are associated the severity and outcomes of IgA nephropathy. Whether this holds true in immunoglobulin A vasculitis (IgAV) is not known. The main objective of the study was to analyze the prognostic value of glomerular C4d immunolabelling on kidney impairment in adults with IgAV. This retrospective cohort study included 120 adults with IgAV and a kidney biopsy performed between 1995 and 2018 in two French university hospital centers. All paraffin-embedded biopsies were reassessed according to Oxford classification. Immunofluorescence for C4d was performed in all cases. For analysis, patients were grouped according to positivity for C4d in the glomerular area. The main outcome was a composite endpoint of 50% increase in 24 h-proteinuria, or eGFR decrease by 50%, or kidney replacement therapy. The median follow-up was 28.3 months. Twenty-three patients met the composite endpoint, 12 for kidney replacement therapy, 6 for an eGFR decrease >50% and 5 for a >50% increase in proteinuria. At time of biopsy, the median proteinuria was 1.9 g/24 h and the median eGFR 73.5 mL/min/1.73 m. Among the 102 patients evaluable for C4d, 24 were positive on >30% glomeruli, mainly with a parieto-mesangial pattern. In this group, the initial proteinuria was more frequently nephrotic than in the C4d- group (60% vs. 33%, = 0.039). Mesangial hypercellularity was more frequent in the C4d+ group (42% vs. 13%; = 0.006) whereas macroscopic hematuria was more frequent in the C4d- group (18% vs. 0%; = 0.03). After a median follow-up of 28 months, kidney survival did not differ according to C4d status. In a population of adult IgAV patients, glomerular positivity for C4d was associated with the severity of the kidney disease at presentation, but not with subsequent renal function deterioration.
PubMed: 34912816
DOI: 10.3389/fmed.2021.735775 -
Radiography (London, England : 1995) Feb 2021The protocol for preparation of computed tomography urography (CTU) examinations at our hospital was changed in 2013 to improve the quality of urinary bladder filling in...
INTRODUCTION
The protocol for preparation of computed tomography urography (CTU) examinations at our hospital was changed in 2013 to improve the quality of urinary bladder filling in the excretory phase. The aim of this study was to evaluate the quality of urinary bladder filling on CTU after different doses of furosemide were administered to patients with macroscopic hematuria.
METHODS
The cohort was 215 patients who underwent elective CTU due to macroscopic hematuria between 2014 and 2018. 5 mg furosemide were administrated to 100 patients, 2.5 mg to 100 patients and 0 mg to 15 patients. Contrast medium layered bladders were excluded, leaving 193 patients: 92, 89 and 12 in each group. Urinary bladder volume was calculated in corticomedullary (CMP) and excretory phase (EP). Bladder distension was classified as satisfactory or not. Attenuation of bladder content in EP was noted.
RESULTS
Average volume in EP was 370 ± 224 ml (28-1052) after 5 mg furosemide, 274 ± 120 ml (43-628) after 2.5 mg and 180 ± 104 ml (53-351) after 0 mg. 85% of the bladders were satisfactory distended after 5 mg, 80% after 2.5 mg and 58% after 0 mg. Average attenuation was 266 ± 89 HU (103-524) after 5 mg, 362 ± 156 HU (118-948) after 2.5 mg and 761 ± 331 HU (347-1206) after 0 mg. The differences in volume and attenuation were significant.
CONCLUSION
5 mg furosemide is preferred rather than 2.5 mg in preparation for CTU examinations of patients with macroscopic hematuria. There was no difference between the doses concerning rate of satisfactory bladder distension, but the higher dose resulted in larger bladder volume and more suitable attenuation of bladder content.
IMPLICATIONS FOR PRACTICE
Development of CTU-image quality could improve bladder cancer diagnostics.
Topics: Furosemide; Hematuria; Humans; Tomography, X-Ray Computed; Urinary Bladder; Urography
PubMed: 32727709
DOI: 10.1016/j.radi.2020.07.002 -
International Journal of Surgery Case... Oct 2022Renal biopsy performed in native or transplanted kidney is considered a safe procedure. However, as it is an invasive procedure bleeding related complications do occur....
INTRODUCTION AND IMPORTANCE
Renal biopsy performed in native or transplanted kidney is considered a safe procedure. However, as it is an invasive procedure bleeding related complications do occur. Bleeding complications such as macroscopic hematuria, renal hematoma, blood transfusion, and rarely nephrectomy and death have been reported in various studies. Acute Page kidney (APK) is a rare complication of post renal biopsy bleeding.
CASE PRESENTATION
In this case report we present a case history of a 46-year-old patient complicated with APK, following a native kidney biopsy. Early surgical exploration and evacuation of large hematoma resulted in a favorable outcome.
CLINICAL DISCUSSION
APK results from external compression of kidney and compression of the parenchyma can compromise the intra renal blood flow and cause renal impairment, activation of Renin-Angiotensin-Aldosterone System (RAAS) leads to systemic hypertension.
CONCLUSION
Awareness, early recognition and timely intervention in APK, in a post renal biopsy bleeding is necessary to prevent poorer outcomes, especially progressively large hematoma is present and response to medical management inadequate.
PubMed: 36122422
DOI: 10.1016/j.ijscr.2022.107641 -
The Cochrane Database of Systematic... Aug 2015Henoch-Schönlein purpura (HSP) is the most common vasculitis of childhood but may occur in adults. This small vessel vasculitis is characterised by palpable purpura,... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
Henoch-Schönlein purpura (HSP) is the most common vasculitis of childhood but may occur in adults. This small vessel vasculitis is characterised by palpable purpura, abdominal pain, arthritis or arthralgia and kidney involvement. This is an update of a review first published in 2009.
OBJECTIVES
To evaluate the benefits and harms of different agents (used singularly or in combination) compared with placebo, no treatment or any other agent for: (1) the prevention of severe kidney disease in patients with HSP without kidney disease at presentation; (2) the prevention of severe kidney disease in patients with HSP and minor kidney disease (microscopic haematuria, mild proteinuria) at presentation; (3) the treatment of established severe kidney disease (macroscopic haematuria, proteinuria, nephritic syndrome, nephrotic syndrome with or without acute kidney failure) in HSP; and (4) the prevention of recurrent episodes of HSP-associated kidney disease.
SEARCH METHODS
We searched the Cochrane Kidney and Transplant's Specialised Register to 13 July 2015 through contact with the Trials Search Co-ordinator using search terms relevant to this review.
SELECTION CRITERIA
Randomised controlled trials (RCTs) comparing interventions used to prevent or treat kidney disease in HSP compared with placebo, no treatment or other agents were included.
DATA COLLECTION AND ANALYSIS
Two authors independently determined study eligibility, assessed risk of bias and extracted data from each study. Statistical analyses were performed using the random effects model and the results were expressed as risk ratio (RR) or risk difference (RD) for dichotomous outcomes and mean difference (MD) for continuous outcomes with 95% confidence intervals (CI).
MAIN RESULTS
Thirteen studies (1403 enrolled patients) were identified. Risks of bias attributes were frequently poorly performed. Low risk of bias was reported in six studies (50%) for sequence generation (selection bias) and in seven (58%) for allocation concealment (selection bias). Blinding of participants and personnel (performance bias) and of outcome assessment (detection bias) was at low risk of bias in three studies. Five studies reported complete outcome data (attrition bias) while eight studies reported expected outcomes so were at low risk of reporting bias.Eight studies evaluated therapy to prevent persistent kidney disease in HSP. There was no significant difference in the risk of persistent kidney disease any time after treatment (5 studies, 746 children: RR 0.74, 95% CI 0.42 to 1.32), or at one, three, six and 12 months in children given prednisone for 14 to 28 days at presentation of HSP compared with placebo or supportive treatment. There were no significant differences in the risk of persistent kidney disease with antiplatelet therapy in children with or without kidney disease at entry. Heparin significantly reduced the risk of persistent kidney disease by three months compared with placebo (1 study, 228 children: RR 0.27, 95% CI 0.14 to 0.55); no significant bleeding occurred. Four studies examined the treatment of severe HSP-associated kidney disease. Two studies (one involving 56 children and the other involving 54 adults) compared cyclophosphamide with placebo or supportive treatment and found no significant benefit of cyclophosphamide. There were no significant differences in adverse effects. In one study comparing cyclosporin with methylprednisolone (15 children) there was no significant difference in remission at final follow-up at a mean of 6.3 years (RR 1.37, 95% CI 0.74 to 2.54). In one study (17 children) comparing mycophenolate mofetil with azathioprine, there was no significant difference in the remission of proteinuria at one year (RR 1.32, 95% CI 0.86 to 2.03). No studies were identified which evaluated the efficacy of therapy on kidney disease in participants with recurrent episodes of HSP.
AUTHORS' CONCLUSIONS
There are no substantial changes in conclusions from this update compared with the initial review. From generally low quality evidence, we found no evidence of benefit from RCTs for the use of prednisone or antiplatelet agents to prevent persistent kidney disease in children with HSP. Though heparin appeared effective, this potentially dangerous therapy is not justified to prevent serious kidney disease when fewer than 2% of children with HSP develop severe kidney disease. No evidence of benefit has been found for cyclophosphamide treatment in children or adults with HSP and severe kidney disease. Because of small patient numbers and events leading to imprecision in results, it remains unclear whether cyclosporin and mycophenolate mofetil have any roles in the treatment of children with HSP and severe kidney disease.
Topics: Adrenal Cortex Hormones; Child; Child, Preschool; Cyclophosphamide; Humans; IgA Vasculitis; Immunosuppressive Agents; Kidney Diseases; Platelet Aggregation Inhibitors; Randomized Controlled Trials as Topic
PubMed: 26258874
DOI: 10.1002/14651858.CD005128.pub3 -
Kidney International Reports Apr 2022Anticoagulant-related nephropathy (ARN) is a relatively novel recognized entity characterized by hematuria-associated acute kidney injury (AKI) in the context of...
INTRODUCTION
Anticoagulant-related nephropathy (ARN) is a relatively novel recognized entity characterized by hematuria-associated acute kidney injury (AKI) in the context of overanticoagulation. Preexisting or underlying kidney disease seems to be a predisposing factor; however, few studies have described histologic findings in patients with ARN. We aimed to evaluate underlying kidney pathology in patients on oral anticoagulation who presented an episode of AKI with hematuria in whom a kidney biopsy was performed.
METHODS
Retrospective observational multicenter case study in patients treated with oral anticoagulants who developed macroscopic or intense hematuria followed by AKI. Only patients with available kidney biopsy specimens were included. Histologic findings and clinical data throughout follow-up were analyzed.
RESULTS
A total of 26 patients were included with a median age of 75 years (62-80) and a follow-up period of 10.1 months. Of the patients, 80% were male, and most cases (92%) were on anticoagulation with vitamin K antagonists (VKAs). At admission, median serum creatinine (SCr) level was 4.2 mg/dl (2.8-8.2), median international normalized ratio (INR) 2.4 (1.5-3.4), and 11 patients (42%) required acute dialysis during hospitalization. Kidney biopsy results revealed that all patients except 1 had an underlying nephropathy: IgA nephropathy (IgAN) in 19, probable IgAN in 1, diabetic nephropathy in 3, nephrosclerosis in 1, and idiopathic nodular glomerulosclerosis in 1. At 12 weeks after discharge, only 6 subjects (24%) attained complete kidney recovery whereas 7 (28%) remained on chronic dialysis.
CONCLUSION
IgAN was the most common underlying kidney disease in our biopsy-proven series of ARN, in which a significant percentage of patients did not achieve kidney function recovery.
PubMed: 35497792
DOI: 10.1016/j.ekir.2022.01.1048 -
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 -
Annals of Medicine and Surgery (2012) Feb 2022and importance: Vesical varices are a rare condition. they are an exceptional cause of hematuria. The most common cause of vesical varices reported in literature is...
INTRODUCTION
and importance: Vesical varices are a rare condition. they are an exceptional cause of hematuria. The most common cause of vesical varices reported in literature is portal hypertension. Usually, varices due to portal hypertension develop in the gastroesophageal region. A multidisciplinary team approach is required, with input from gastroenterologists, interventional radiologists, and urologists.
CASE PRESENTATION
We present a case of A 38 -year- old man presented to our office for 2 episodes of total macroscopic gross hematuria. The detailed general physical examination revealed: a patient in a good condition, without pallor or icterus. Laboratory investigations are normal. Cystoscopy under locoregional anesthesia was performed. It revealed a group of dilated sub mucosal veins, in the trigone. After discussing the case with gastroenterologists, we had presumed that the etiology of vesical varices may be the portal hypertension.
CLINICAL DISCUSSION
Vesical varices are an extremely rare cause of hematuria. The most common cause of vesical varices reported in literature is portal hypertension. The vesical varices may remain asymptomatic for a long period and manifest with hematuria in some cases. In order to organize the management of bleeding vesical varices, a detailed cartography of vesical vascularization have to be performed, including cystoscopy and abdominal contrast tomography (10). No definitive treatment has been established for bleeding vesical varices. In case of gross hematuria, surgical devascularization, laser sclerosis and coagulation are often only of temporary effectiveness.
CONCLUSION
Vesical varices are an extremely rare pathology. Its main etiology is Portal hypertension. The hematuria due to vesical varices could be life threatening. It requires energetic treatment. The goal of the treatment is portal decompression.
PubMed: 35242309
DOI: 10.1016/j.amsu.2022.103286 -
The Turkish Journal of Pediatrics 2022Granulocytic sarcoma (GS) is an extramedullary solid tumor composed of immature myeloid cells. GS has been associated with acute myeloid leukemia (AML), myelodysplastic... (Review)
Review
BACKGROUND
Granulocytic sarcoma (GS) is an extramedullary solid tumor composed of immature myeloid cells. GS has been associated with acute myeloid leukemia (AML), myelodysplastic syndromes or myeloproliferative diseases. Although GS can affect various tissues of the human body, it has rarely been reported in other soft tissues such as the breast, gastrointestinal, respiratory and genitourinary tracts. We report a pediatric case diagnosed with granulocytic sarcoma of the bladder and concomitant AML.
CASE
A twelve-year-old previously healthy girl was admitted to the pediatric urology clinic with a ten-day history of hematuria and pollakiuria. Laboratory examinations revealed anemia, thrombocytopenia and neutrophilic leukocytosis. Bone marrow aspiration results were consistent with acute myeloid leukemia -FAB subtype M2-. Abdominal magnetic resonance imaging (MRI) showed an irregularly bounded 12 cm mass on the right side of the bladder. Transurethral resection (TUR) pathology was consistent with granulocytic sarcoma. After a multimodal treatment approach, complete remission was achieved.
CONCLUSIONS
Malignant bladder masses are rare causes of macroscopic hematuria in childhood. The diagnostic spectrum is wide, ranging from rhabdomyosarcoma to leukemia involvement. The bladder is a rare site of extramedullary involvement in pediatric patients with AML. Multimodal treatment should be considered on a per-patient basis.
Topics: Anemia; Child; Female; Hematuria; Humans; Leukemia, Myeloid, Acute; Male; Sarcoma, Myeloid; Urinary Bladder; Urinary Bladder Neoplasms
PubMed: 35286044
DOI: 10.24953/turkjped.2020.275 -
Clinical and Experimental Nephrology May 2020Practice patterns and bleeding complications of percutaneous native kidney biopsy (PNKB) have not recently been investigated and the Japanese Society of Nephrology...
BACKGROUND
Practice patterns and bleeding complications of percutaneous native kidney biopsy (PNKB) have not recently been investigated and the Japanese Society of Nephrology performed a nationwide questionnaire survey in 2018.
METHODS
The survey consisted of nine sections about PNKB: (1) general indications; (2) indications for high-risk patients; (3) informed consent; (4) pre-biopsy evaluation; (5) procedures; (6) sedation; (7) post-biopsy hemostasis, bed rest, and examinations; (8) bleeding complications; and (9) specimen processing. A supplementary survey examined bleeding requiring transcatheter arterial embolization (TAE).
RESULTS
Overall, 220 directors of facilities (nephrology facility [NF], 168; pediatric nephrology facility [PF], 52) completed the survey. Indications, procedures, and monitoring protocols varied across facilities. Median lengths of hospital stay were 5 days in NFs and 6 days in PFs. Gauge 14, 16, 18 needles were used in 5%, 56%, 33% in NFs and 0%, 63%, 64% in PFs. Mean limits of needle passes were 5 in NFs and 4 in PFs. The bed rest period was 16-24 h in 60% of NFs and 65% of PFs. Based on 17,342 PNKBs, incidence rates of macroscopic hematuria, erythrocyte transfusion, and TAE were 3.1% (NF, 2.8%; PF, 6.2%), 0.7% (NF, 0.8%; PF, 0%), and 0.2% (NF, 0.2%; PF, 0.06%), respectively. Forty-six percent of facilities processed specimens all for light microscopy, immunofluorescence, and electron microscopy, and 21% processed for light microscopy only. Timing of bleeding requiring TAE varied among PNKB cases.
CONCLUSION
Wide variations in practice patterns of PNKB existed among facilities, while PNKBs were performed as safely as previously reported.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Biopsy; Child; Child, Preschool; Embolization, Therapeutic; Erythrocyte Transfusion; Female; Health Facilities; Hematuria; Humans; Infant; Infant, Newborn; Informed Consent; Japan; Kidney; Length of Stay; Male; Microscopy, Electron; Middle Aged; Needles; Nephrology; Organizational Policy; Patient Selection; Pediatrics; Postoperative Hemorrhage; Preoperative Care; Surveys and Questionnaires; Young Adult
PubMed: 32189101
DOI: 10.1007/s10157-020-01869-w -
Frontiers in Pediatrics 2019Urolithiasis can affect all children even preschool ones. Diagnostic difficulties in the youngest children are due to the problems in locating pain and determining its...
Urolithiasis can affect all children even preschool ones. Diagnostic difficulties in the youngest children are due to the problems in locating pain and determining its character and severity. In keeping with the ALARA (As Low As Reasonably Achievable) protocol, the number of imaging tests possible to perform is very limited. Ultrasound is the first line exam of choice. After diagnosis of the presence of a stone, ESWL (Extracorporeal Shock Wave Lithotrypsy) should always be considered and offered to parents due to its high effectiveness and minimal invasiveness. If ESWL is contraindicated or not well-accepted by parents, authors suggest another minimal invasive approach: URS-L (Uretherorenoscopy-Lithotrypsy). Our study clinically analyzes 87 children, which were treated between 2009 and 2017 using the URS-L procedure. URS-L treatments were performed using Lithoclast until 2009, and after that time, using the holmium laser Ho:YAG. The overall effectiveness of treatments was 93.3%. There was no failure in the access to the stones. A macroscopic hematuria (Clavien-Dindo I grade) was observed through the second post-operative day in 9.2% of treated patients. No urosepsis was observed. Full metabolic evaluation was performed on all patients. Children remained under constant urological and nephrological observation. A recurrence of urolithiasis was observed in 35.6% of the cases. Treating ureteral lithiasis in young infants remains a big challenge. Our series shows that modern minimal invasive techniques used by very experienced pediatric urologists in high volume centers gives excellent results. In most cases, surgery should no longer need to be an option.
PubMed: 31555620
DOI: 10.3389/fped.2019.00324