scholarly journals A Case of Alport Syndrome with Posttransplant Antiglomerular Basement Membrane Disease despite Negative Antiglomerular Basement Membrane Antibodies by EIA Treated with Plasmapheresis and Intravenous Immunoglobulin

2013 ◽  
Vol 2013 ◽  
pp. 1-5
Author(s):  
Sumiko I. Armstead ◽  
Thomas Hellmark ◽  
Jorgen Wieslander ◽  
Xin J. Zhou ◽  
Ramesh Saxena ◽  
...  
Keyword(s):  
Basement Membrane ◽  
Intravenous Immunoglobulin ◽  
Alport Syndrome ◽  
Kidney Injury ◽  
Deceased Donor ◽  
End Of Treatment ◽  
Circulating Antibodies ◽  
Deceased Donor Kidney Transplant ◽  
Stage Renal Disease ◽  
End Stage

Posttransplant antiglomerular basement membrane (anti-GBM) disease occurs in approximately 5% of Alport patients and usually ends in irreversible graft failure. Recent research has focused on characterizing the structure of the anti-GBM alloepitope. Here we present a case of a 22-year-old male with end-stage renal disease secondary to Alport syndrome, with a previously failed renal allograft, who received a second deceased-donor kidney transplant. Six days after transplantation, he developed acute kidney injury. The serum anti-GBM IgG was negative by enzyme immunoassay (EIA). On biopsy, he had crescentic glomerulonephritis with linear GBM fixation of IgG. With further analysis by western blotting, we were able to detect antibodies to an unidentified protein from the basement membrane. This patient was treated with plasmapheresis twice per week and monthly intravenous immunoglobulin (IVIG) for a total of five months. At the end of treatment, these unknown antibodies were no longer detected. His renal function improved, and he has not required dialysis. We conclude that anti-GBM disease in patients with Alport Syndrome may be caused by circulating antibodies to other components of the basement membrane that are undetectable by routine anti-GBM EIA and may respond to treatment with plasmapheresis and IVIG.

scholarly journals Ureteric Trauma following Stent Removal in Kidney Transplant Recipient: A Unique Case of Prolonged Morbidity

2021 ◽  
Vol 2021 ◽  
pp. 1-4
Author(s):  
Ahmad Mirza ◽  
Imran Gani ◽  
Andy Shi Huang ◽  
Ravi Mallavarapu ◽  
Laura Mulloy ◽  
...  
Keyword(s):  
Kidney Transplant ◽  
Kidney Transplant Recipient ◽  
Deceased Donor ◽  
Filling Defect ◽  
Percutaneous Nephrostomy ◽  
Distal Ureter ◽  
Noncontrast Ct ◽  
Deceased Donor Kidney Transplant ◽  
Stage Renal Disease ◽  
End Stage

A 52-year-old African-American male patient with end-stage renal disease due to hypertension underwent deceased donor kidney transplant procedure with no immediate complications. The postprocedure complications, interventions, and course were abstracted by chart review. The ureteric stent was removed with flexible cystoscopy on postoperative day (POD) 24. 24 hours later, the patient presented with abdominal pain and inability to urinate. An urgent ultrasound and noncontrast CT scan showed grade 4 hydronephrosis of the transplanted kidney. A percutaneous nephrostomy stent was placed for urinary diversion. A large ureteric hematoma filling the lumen of the mid to distal ureter was identified on the nephrostogram and was evacuated. A follow-up nephrostogram on POD 44 revealed a distal ureter stricture and persistent well-formed midureter filling defect. A repeat nephrostogram performed at POD 72 was done with stricture dilatation, internalization of stents, and removal of a percutaneous nephrostomy tube. The patient was maintained on antibiotics for UTI prophylaxis throughout the course.

Early onset calciphylaxis following acute kidney injury secondary to anti-glomerular basement membrane antibody disease

2021 ◽  
Vol 14 (4) ◽  
pp. e241265
Author(s):  
Sheikh Raza Shahzad ◽  
Faris Alfaris ◽  
Mustafa Erdem Arslan ◽  
Swati Mehta
Keyword(s):  
Acute Kidney Injury ◽  
Basement Membrane ◽  
Glomerular Basement Membrane ◽  
Early Onset ◽  
Kidney Injury ◽  
Caucasian Woman ◽  
History Of ◽  
Stage Renal Disease ◽  
End Stage

Calciphylaxis is commonly associated with end-stage renal disease (ESRD) and renal transplant. We present a rare case of early onset calciphylaxis in a patient presenting with acute kidney injury (AKI) secondary to anti-glomerular basement membrane (anti-GBM) antibody disease. A 65-year-old obese Caucasian woman with type 2 diabetes mellitus and hypertension presented with a 1-month history of painless gross haematuria and worsening lower extremity oedema. Laboratory results indicated AKI and nephrotic-range proteinuria. Anti-glomerular antibodies were elevated. Renal biopsy revealed focal crescentic glomerulonephritis with linear capillary immunoglobulin G staining consistent with anti-GBM antibody disease. She was treated with haemodialysis, plasmapheresis, steroids, bumetanide and cyclophosphamide. Two months later, she developed necrotic lesions on bilateral thighs. Wound biopsy was consistent with calciphylaxis. This case highlights that calciphylaxis, usually seen in patients with chronic kidney disease or ESRD, can manifest in patients with AKI as well.

scholarly journals Surgical approach for kidney transplantation under spinal anesthesia

2020 ◽  
Vol 2020 (12) ◽  
Author(s):  
Atta Nawabi ◽  
Peter Sullivan ◽  
Martin De Ruyter ◽  
Amy Pichoff ◽  
Clay D King ◽  
...  
Keyword(s):  
Kidney Transplantation ◽  
Spinal Anesthesia ◽  
Deceased Donor ◽  
Annual Data ◽  
Short Supply ◽  
Deceased Donor Kidney ◽  
Airway Equipment ◽  
Deceased Donor Kidney Transplant ◽  
Stage Renal Disease ◽  
End Stage

Abstract Kidney transplantation is the treatment of choice for patients with end-stage renal disease (ESRD). It has been shown to improve quality of life as well as extending life of patients with ESRD as compared to renal replacement therapy (5-year survival rate of 68% after transplant vs 36% dialysis) (Hart A, Smith JM, Skeans MA. OPTN/SRTR 2015 annual data report: kidney. Am J Transplant 2017;17:21–116). Traditionally, patients undergo general endotracheal tube anesthesia for this surgery. During the COVID-19 pandemic, general anesthesia drugs and airway equipment were in short supply. Additionally, airway manipulation was avoided when possible due to concern for virus spread from aerosolizing procedures (i.e. intubation/extubation). In this case report, we review a 65-year-old female with an ESRD due to hypertension and diabetes that underwent deceased donor kidney transplant under spinal anesthesia. We will further discuss the benefits of spinal anesthesia in renal transplant operations.

scholarly journals Comparison of alpha5(IV) collagen chain expression in skin with disease severity in women with X-linked Alport syndrome.

1998 ◽  
Vol 9 (8) ◽  
pp. 1433-1440
Author(s):  
K Nakanishi ◽  
K Iijima ◽  
N Kuroda ◽  
Y Inoue ◽  
Y Sado ◽  
...  
Keyword(s):  
Basement Membrane ◽  
Renal Disease ◽  
Disease Severity ◽  
End Stage Renal Disease ◽  
Alport Syndrome ◽  
Expression Ratio ◽  
Female Patients ◽  
Stage Renal Disease ◽  
End Stage ◽  
Epidermal Basement Membrane

X-Linked Alport syndrome is caused by mutations in the type IV collagen alpha5 chain gene. Male patients usually develop end-stage renal disease, whereas female patients have more variable phenotypes from asymptomatic hematuria to end-stage renal disease. The variable phenotypes in female patients may be attributable to different X-chromosome inactivation patterns. Therefore, the correlation between disease severity and the degree of alpha5 chain expression in the epidermal basement membrane of female patients with X-linked Alport syndrome was examined. To estimate the disease severity in X-linked Alport syndrome, the ratios of protein to creatinine in single voided urine samples were used. Expression of the alpha5 chain in the epidermal basement membrane was examined by an indirect immunofluorescence method using an anti-alpha5 chain monoclonal antibody. A total of 25 female patients with X-linked Alport syndrome from 17 families was examined. Multiple regression analysis using disease severity as the response variable, and age, family history of nephritis, female and male family history of end-stage renal disease, serum creatinine concentration, and alpha5(IV) expression ratio in the epidermal basement membrane as explanatory variables showed that only alpha5(IV) expression ratio was a significant factor, and that it showed a highly significant negative association with disease severity (adjusted r2=0.71, P=0.0001). These findings suggest that variable alpha5 chain expression, possibly caused by different X-inactivation patterns, is responsible for the variable disease severity in female patients with X-linked Alport syndrome, and that immunohistochemical examination of alpha5 chain expression in the epidermal basement membrane may be a simple and useful method for predicting patient outcome.

scholarly journals Successful Transplantation of a Split Crossed Fused Ectopic Kidney into a Patient with End-Stage Renal Disease

2010 ◽  
Vol 2010 ◽  
pp. 1-5 ◽  
Author(s):  
Kristin L. Mekeel ◽  
Shane M. Daley ◽  
Paul E. Andrews ◽  
Adyr A. Moss ◽  
R. L. Heilman ◽  
...  
Keyword(s):  
Deceased Donor ◽  
Technical Difficulty ◽  
Parenchymal Transection ◽  
Ectopic Kidney ◽  
Renal Anomalies ◽  
Successful Transplantation ◽  
Immediate Function ◽  
Deceased Donor Kidney Transplant ◽  
Stage Renal Disease ◽  
End Stage

Potential donors with congenital renal anomalies but normal renal function are often overlooked because of a possible increase in technical difficulty and complications associated with the surgery. However, as the waiting list for a deceased donor kidney transplant continues to grow, it is important to consider these kidneys for potential transplant. This paper describes the procurement of a crossed fused ectopic kidney, and subsequent parenchymal transection prior to transplantation as part of a combined simultaneous kidney pancreas transplant. The transplant was uncomplicated, and the graft had immediate function. The patient is now two years from transplant with excellent function.

scholarly journals The Genetics, Current Research, and Future Treatment of Alport Syndrome

2017 ◽  
Vol 6 (1) ◽  
pp. 1-7
Author(s):  
Elise Alexandra Kikis ◽  
Emily Holland Williams
Keyword(s):  
Basement Membrane ◽  
End Stage Renal Disease ◽  
Alport Syndrome ◽  
Autosomal Recessive ◽  
Type Iv Collagen ◽  
Autosomal Dominant ◽  
Basement Membranes ◽  
Type Iv ◽  
Stage Renal Disease ◽  
End Stage

Alport syndrome is a type IV collagen disease that affects the glomerular basement membrane of approximately one in every 5000 people. The disease was first described by A. Cecil Alport in 1927 as “a dominantly inherited hereditary nephritis.” The three genotypes of the disease are X-linked dominant, autosomal recessive, and autosomal dominant. The X-linked dominant genotype is the most common, accounting for 80% of all cases of Alport syndrome, affecting mainly men. The autosomal recessive and autosomal dominant types affect men and women equally. Alport syndrome is caused by mutations on the COL4A3, COL4A4, and COL4A5 genes, which code the ?3, ?4, and ?5 (IV) chains that make up type IV collagen molecules, an important component of basement membranes. Thus, Alport syndrome results in malformed basement membranes, with symptoms including renal impairment, hematuria, bilateral sensorineural hearing loss, and an abnormal structure of the glomerular basement membrane. Alport syndrome also often progresses to end-stage renal disease, especially in men with X-linked Alport syndrome. At this point, there is no cure for Alport syndrome. However, there are many successful treatments for its symptoms. Angiotensin-converting enzyme (ACE) inhibitors are often given to patients in the early stages of Alport syndrome. For patients with end-stage renal disease, dialysis or kidney transplants are considered the best course of action.

scholarly journals Quantification and Dosing of Renal Replacement Therapy in Acute Kidney Injury: A Reappraisal

2017 ◽  
Vol 44 (2) ◽  
pp. 140-155 ◽  
Author(s):  
William R. Clark ◽  
Martine Leblanc ◽  
Zaccaria Ricci ◽  
Claudio Ronco
Keyword(s):  
Acute Kidney Injury ◽  
Renal Replacement Therapy ◽  
Replacement Therapy ◽  
Journal Club ◽  
Kidney Injury ◽  
Accurate Estimation ◽  
Stage Renal Disease ◽  
End Stage ◽  
Dose Assessments ◽  
Solute Clearance

Background/Aims: Delivered dialysis therapy is routinely measured in the management of patients with end-stage renal disease; yet, the quantification of renal replacement prescription and delivery in acute kidney injury (AKI) is less established. While continuous renal replacement therapy (CRRT) is widely understood to have greater solute clearance capabilities relative to intermittent therapies, neither urea nor any other solute is specifically employed for CRRT dose assessments in clinical practice at present. Instead, the normalized effluent rate is the gold standard for CRRT dosing, although this parameter does not provide an accurate estimation of actual solute clearance for different modalities. Methods: Because this situation has created confusion among clinicians, we reappraise dose prescription and delivery for CRRT. Results: A critical review of RRT quantification in AKI is provided. Conclusion: We propose an adaptation of a maintenance dialysis parameter (standard Kt/V) as a benchmark to supplement effluent-based dosing of CRRT. Video Journal Club “Cappuccino with Claudio Ronco” at http://www.karger.com/?doi=475457

scholarly journals Identification of Novel Biomarker for Early Detection of Diabetic Nephropathy

Biomedicines ◽  
2021 ◽  
Vol 9 (5) ◽  
pp. 457
Author(s):  
Kyeong-Seok Kim ◽  
Jin-Sol Lee ◽  
Jae-Hyeon Park ◽  
Eun-Young Lee ◽  
Jong-Seok Moon ◽  
...  
Keyword(s):  
Diabetic Nephropathy ◽  
Early Stage ◽  
Kidney Injury ◽  
Diabetic Patients ◽  
High Morbidity ◽  
Neutrophil Gelatinase Associated Lipocalin ◽  
Zucker Diabetic Fatty Rats ◽  
Kidney Injury Molecule 1 ◽  
Stage Renal Disease ◽  
End Stage

Diabetic nephropathy (DN) is one of the most common complications of diabetes mellitus. After development of DN, patients will progress to end-stage renal disease, which is associated with high morbidity and mortality. Here, we developed early-stage diagnostic biomarkers to detect DN as a strategy for DN intervention. For the DN model, Zucker diabetic fatty rats were used for DN phenotyping. The results revealed that DN rats showed significantly increased blood glucose, blood urea nitrogen (BUN), and serum creatinine levels, accompanied by severe kidney injury, fibrosis and microstructural changes. In addition, DN rats showed significantly increased urinary excretion of kidney injury molecule-1 (KIM-1) and neutrophil gelatinase-associated lipocalin (NGAL). Transcriptome analysis revealed that new DN biomarkers, such as complementary component 4b (C4b), complementary factor D (CFD), C-X-C motif chemokine receptor 6 (CXCR6), and leukemia inhibitory factor (LIF) were identified. Furthermore, they were found in the urine of patients with DN. Since these biomarkers were detected in the urine and kidney of DN rats and urine of diabetic patients, the selected markers could be used as early diagnosis biomarkers for chronic diabetic nephropathy.

Peritoneal Dialysis

2019 ◽  
Author(s):  
Karlien François ◽  
Joanne M. Bargman
Keyword(s):  
Renal Failure ◽  
Peritoneal Dialysis ◽  
Peritoneal Cavity ◽  
Fluid Overload ◽  
Kidney Injury ◽  
Dialysis Fluid ◽  
Keywords Peritoneal Dialysis ◽  
Stage Renal Disease ◽  
End Stage ◽  
Developed World

In peritoneal dialysis (PD), the peritoneum serves as a biological dialyzing membrane. The endothelium of the vast capillary network perfusing the peritoneum functions as a semipermeable membrane and allows bidirectional solute and water transfer between the intravascular space and dialysate fluid dwelling in the peritoneal cavity. PD is a renal replacement strategy for patients presenting with end-stage renal disease. It can also be offered for ultrafiltration in patients with diuretic-resistant fluid overload even in those without advanced renal failure. PD can also be used for patients with acute kidney injury, although in the developed world this occurs rarely compared to the use of extracorporeal therapies. This review contains 9 videos,  8 figures, 4 tables, and 73 references.  Keywords: peritoneal dialysis, peritoneal cavity, catheter, dialysis fluid, ultrafiltration, tunnel infection, osmotic pressure, renal failure

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