scholarly journals Alport's Syndrome in Pregnancy

2013 ◽  
Vol 2013 ◽  
pp. 1-3 ◽  
Author(s):  
Suchita Mehta ◽  
Chadi Saifan ◽  
Marie Abdellah ◽  
Rita Choueiry ◽  
Rabih Nasr ◽  
...  
Keyword(s):  
Kidney Injury ◽  
Adverse Outcomes ◽  
Rapid Progression ◽  
Alport's Syndrome ◽  
Case Presentation ◽  
Alport’S Syndrome ◽  
History Of ◽  
Stage Renal Disease ◽  
End Stage ◽  
In Pregnancy

Background. Alport's syndrome is an X-linked hereditary disorder affecting the glomerular basement membrane associated with ocular and hearing defects. In women, the disease is much less severe compared to that in men. However, women with Alport's syndrome can have an accelerated form of their disease during pregnancy with worsening of kidney function and can also develop preeclampsia. There are only four described cases of Alport's syndrome in pregnancy.Case Presentation. 20-year-old woman with a history of Alport's syndrome, which during pregnancy worsened resulting in hypertension, proteinuria, and acute kidney injury. Fortunately, there was complete resolution of the proteinuria and kidney injury with delivery, and the patient did not require any renal replacement therapy.Conclusion. One of the four reported cases had an accelerated form of the disease during pregnancy with rapid progression of kidney injury and end-stage renal disease. There are no definite guidelines to monitor these patients during pregnancy. Further studies are required to understand the exact pathophysiology of kidney damage that occurs in pregnant women with Alport's syndrome. This may give us some insight into the prognostic predictors, so that we can monitor these women more thoroughly and prevent adverse outcomes.

scholarly journals A Case of Immunotactoid Glomerulopathy with Rapid Progression to End-Stage Renal Disease

2009 ◽  
Vol 9 ◽  
pp. 1348-1354 ◽  
Author(s):  
Shikha Jain ◽  
Darshika Chhabra
Keyword(s):  
Acute Kidney Injury ◽  
Renal Disease ◽  
End Stage Renal Disease ◽  
Kidney Injury ◽  
Initial Presentation ◽  
Rapid Progression ◽  
First Case ◽  
Immunotactoid Glomerulopathy ◽  
Stage Renal Disease ◽  
End Stage

Immunotactoid glomerulopathy (IGN) is a rare immunoglobulin deposition disease. It is often mistaken for cryoglobulinemia or amyloidosis due to the similarities on biopsy findings. The disease progresses to end-stage renal disease (ESRD) within 7 months to 10 years. This is the first case reported of a patient with a diagnosis of IGN who developed acute kidney injury (AKI) and ESRD within 1 week of initial presentation.

scholarly journals Point-of-care Ultrasound in the Diagnosis of Calciphylaxis

2020 ◽  
Vol 4 (3) ◽  
pp. 495-496
Author(s):  
Natasha Tobarran ◽  
Mark Collin
Keyword(s):  
Point Of Care ◽  
Point Of Care Ultrasound ◽  
Emergency Physicians ◽  
Computed Tomography Imaging ◽  
Case Presentation ◽  
Tomography Imaging ◽  
History Of ◽  
Calcium Deposits ◽  
Stage Renal Disease ◽  
End Stage

Case Presentation: A 63-year-old male with a past medical history of end stage renal disease presented to the emergency department with painful, lower-extremity necrotic ulcerations. Ultrasound and computed tomography imaging showed concerns for calcium deposits. Biopsy confirmed the diagnosis of calciphylaxis, a rare lethal disease. Discussion: Emergency physicians should keep this disease on their differential due to the high mortality rate.

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 Adverse outcomes associated with rapid linear and non-linear patterns of chronic kidney disease progression

2021 ◽  
Vol 22 (1) ◽  
Author(s):  
Ibrahim Ali ◽  
Rajkumar Chinnadurai ◽  
Sara T. Ibrahim ◽  
Philip A. Kalra
Keyword(s):  
Adverse Outcomes ◽  
Individual Risk ◽  
Rapid Progression ◽  
Study Cohort ◽  
Factors Associated ◽  
Non Linear ◽  
Stage Renal Disease ◽  
End Stage ◽  
Egfr Slope

Abstract Background Patients with rapidly declining renal function face the dual threat of end-stage renal disease (ESRD) and mortality prior to ESRD. What is less well characterised is whether the pattern of the renal trajectory, linear or non-linear, unmasks subgroups of rapidly progressing patients that face adverse outcomes in a differential manner. Methods An individual eGFR slope was applied to all outpatient estimated glomerular filtration rate (eGFR) values for each patient in the Salford Kidney Study from 2002 to 2018 who had at least 2 years follow-up, ≥4 eGFR values and baseline eGFR 15 to < 60 ml/min/1.73m2. Rapid progression was defined as an annual eGFR slope of ≤ − 3 ml/min/1.73m2/yr and patients were categorised as linear or non-linear progressors based on the nature of their eGFR-time graphs. A Fine-Gray competing risk hazard model was used to determine factors associated with progression to ESRD and with mortality prior to ESRD. Cumulative incidence function curves highlighted differences in outcomes between linear and non-linear patients. Results There were 211 rapidly deteriorating patients with linear eGFR trajectories and 61 rapid non-linear patients in the study cohort. Factors associated with ESRD included younger age, male gender, lower baseline eGFR and higher serum phosphate, whilst older age, history of myocardial infarction and anaemia predicted mortality prior to ESRD. Over a median follow-up of 3.7 years, linear progressors reached ESRD sooner whilst those with non-linear progression faced significantly higher rates of mortality prior to ESRD. Conclusions Patients with rapid eGFR decline have high rates of adverse outcomes that are differentially expressed in those progressing linearly and non-linearly as a result of differing phenotypic profiles. Consequently, addressing individual risk factor profiles is important to deliver optimal personalised patient care.

scholarly journals Update on Recurrent Focal Segmental Glomerulosclerosis in Kidney Transplantation

Nephron ◽  
2020 ◽  
pp. 1-6
Author(s):  
Jun Shoji ◽  
Akiko Mii ◽  
Mika Terasaki ◽  
Akira Shimizu
Keyword(s):  
Kidney Transplantation ◽  
Focal Segmental Glomerulosclerosis ◽  
Rapid Progression ◽  
Segmental Glomerulosclerosis ◽  
Type Plasminogen Activator ◽  
Urokinase Type Plasminogen Activator ◽  
History Of ◽  
Stage Renal Disease ◽  
End Stage

<b><i>Background:</i></b> Focal segmental glomerulosclerosis (FSGS) is a clinicopathological syndrome characterized by nephrotic-range proteinuria with high incidence of progression to end-stage renal disease (ESRD). In primary FSGS, 40–60% of patients develop ESRD within 10–20 years. <b><i>Summary:</i></b> Recurrence of FSGS after kidney transplantation is frequent and is associated with poor allograft survival. The risk factors for recurrent FSGS include onset of FSGS during childhood, rapid progression of primary FSGS to ESRD, history of recurrent FSGS in previous allograft, and diffuse mesangial hypercellularity or collapsing variant of FSGS in the native kidney. The early histological findings of recurrent FSGS consist of unremarkable glomerular changes on light microscopy but significant podocyte effacement on electron microscopy; the loss of foot processes with eventual dropout of podocytes leads to the development of segmental lesions in the glomerulus. Experimental and clinical data suggest the existence of circulating permeability factors, such as soluble urokinase-type plasminogen activator receptor (suPAR), cardiotrophin-like cytokine factor-1 (CLCF-1), CD40 axis, and apolipoprotein A-Ib (ApoA-Ib), in the pathogenesis of recurrent FSGS. These biomarkers including circulating permeability factors may facilitate earlier diagnosis of FSGS posttransplant and may guide in the development of novel therapies that may be more effective and improve long-term outcomes in kidney transplantation. <b><i>Key Messages:</i></b> Several studies have suggested the possible circulating permeability factors, such as suPAR, CLCF-1, CD40 axis, and ApoA-Ib, in the pathogenesis and disease progression of FSGS and recurrent FSGS. Further studies should be performed to elucidate the true essential biomarker(s) associated with the onset and progression of FSGS as well as recurrent FSGS.

Abstract 15292: Acute Coronary Syndrome in Patients With Severe Aortic Stenosis Undergoing TAVR: A Retrospective Analysis

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Saad M Amin ◽  
Harsh Mehta ◽  
AKHTAR AMIN
Keyword(s):  
Acute Coronary Syndrome ◽  
Aortic Stenosis ◽  
Retrospective Analysis ◽  
Severe Aortic Stenosis ◽  
Kidney Injury ◽  
Coronary Syndrome ◽  
Transcatheter Aortic Valve ◽  
History Of ◽  
Stage Renal Disease ◽  
End Stage

Introduction: Transcatheter aortic valve replacement (TAVR) has been increasingly utilized in patients with Aortic Stenosis (AS). The purpose of this study is to document the incidence of acute coronary syndrome (ACS) in patients with a history of severe Aortic Stenosis (AS) undergoing TAVR. Methods: Using the Nationwide Inpatient Sample (NIS) for years 2015 to 2017, we performed a retrospective analysis of adults over 18 years of age who underwent TAVR and their inpatient course was complicated by ACS. We further analyzed the comorbidities and complications associated with patients who developed ACS during the same hospitalization. Results: We divided a total of 69,584 patients with ACS, who underwent TAVR into two groups: patients with ACS (2.11%) and patients without ACS (97.89%). From 2015 to 2017 there was a decline in the incidence of ACS in patients undergoing TAVR from 2.62% to 1.93%. There were no demographic differences between the two groups. Comorbidities like diabetes, peripheral vascular disease, end-stage renal disease and chronic liver disease were more prevalent in ACS group (Table 1). Patients with ACS had a higher history of previous PCI (4.42% vs 2.64% p-0.08). Complications like cardiogenic shock (11.9% vs 1.29% p-<0.05), acute respiratory failure (25.17% vs 4.1% p <0.05), acute kidney injury requiring dialysis (1.7% vs 0.46% p<0.05) and cardiac arrest (4.08% vs 0.78% p<0.05) were more prevalent in the ACS group. Overall, in-hospital mortality (3.41% vs 1.42% p< 0.05) and length of stay (12.22 days vs 4.20 days p<0.05) was higher in the ACS group. Conclusions: A steady decline was noted in the number of cases complicated by ACS undergoing for TAVR over 3 years. ACS in TAVR cases was associated with higher rates of complications, prolonged hospitalizations and higher mortality.

scholarly journals Recent Advances in Diabetic Kidney Diseases: From Kidney Injury to Kidney Fibrosis

2021 ◽  
Vol 22 (21) ◽  
pp. 11857
Author(s):  
Peir-Haur Hung ◽  
Yung-Chien Hsu ◽  
Tsung-Hsien Chen ◽  
Chun-Liang Lin
Keyword(s):  
Kidney Disease ◽  
Kidney Diseases ◽  
Epigenetic Modification ◽  
Kidney Injury ◽  
Diabetic Kidney ◽  
Kidney Fibrosis ◽  
Recent Advances ◽  
History Of ◽  
Stage Renal Disease ◽  
End Stage

Diabetic kidney disease (DKD) is the leading cause of chronic kidney disease and end-stage renal disease. The natural history of DKD includes glomerular hyperfiltration, progressive albuminuria, declining estimated glomerular filtration rate, and, ultimately, kidney failure. It is known that DKD is associated with metabolic changes caused by hyperglycemia, resulting in glomerular hypertrophy, glomerulosclerosis, and tubulointerstitial inflammation and fibrosis. Hyperglycemia is also known to cause programmed epigenetic modification. However, the detailed mechanisms involved in the onset and progression of DKD remain elusive. In this review, we discuss recent advances regarding the pathogenic mechanisms involved in DKD.

scholarly journals Delayed bowel perforation after instilling over warmed peritoneal dialysate

2021 ◽  
Vol 22 (1) ◽  
Author(s):  
Xueli Lai ◽  
Mingming Nie ◽  
Xiaodong Xu ◽  
Yuanjie Chen ◽  
Zhiyong Guo
Keyword(s):  
Peritoneal Dialysis ◽  
Bowel Perforation ◽  
Exploratory Laparotomy ◽  
Peritoneal Dialysis Patient ◽  
Case Presentation ◽  
Abdominal Organs ◽  
Home Based ◽  
Dialysis Solution ◽  
Stage Renal Disease ◽  
End Stage

Abstract Background Peritoneal dialysis (PD) is a safe and home-based treatment for end-stage renal disease (ESRD) patients. The direct thermal damage of abdominal organs is very rare. Case presentation We report a peritoneal dialysis patient presented abdominal pain and feculent effluent 3 weeks after he instilled hot dialysis solution. In spite of emergency exploratory laparotomy and active treatment, the patient died of septic shock. Biopsy revealed necrosis and perforation of the intestines. Conclusions Delayed bowel perforation by hot fluid is very rare. Standardized performance is of the first importance for peritoneal dialysis patients.

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