POEMS Syndrome Complicated by Extensive Calciphylaxis: A Remarkable Recovery

Background and Objective Calciphylaxis is life threatening. It has traditionally been associated with end-stage renal disease and hyperparathyroidism but is increasingly common in other clinical contexts. The association of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome and calciphylaxis has been reported only in a few cases. This case is the first of patient survival in such widespread disease. Methods and Results A 42-year-old man with POEMS syndrome developed extensive calciphylaxis despite normal renal and parathyroid function. Rapid diagnosis, treatment, and supportive care contributed to full clinical resolution. Conclusion This is the fifth case of POEMS syndrome associated with calciphylaxis. The observations from this report suggest that POEMS syndrome might be an independent risk factor for the development of calciphylaxis. This case underlines the importance of careful follow-up in patients with POEMS syndrome and prompt diagnosis and treatment of associated calciphylaxis.

Download Full-text

Endovascular mechanical thrombectomy and stenting in a case of central vein thrombosis

BMJ Case Reports ◽

10.1136/bcr-2020-236508 ◽

2021 ◽

Vol 14 (2) ◽

pp. e236508

Author(s):

Rajesh Vijayvergiya ◽

Navjyot Kaur ◽

Saroj K Sahoo ◽

Ashish Sharma

Keyword(s):

Mechanical Thrombectomy ◽

Transluminal Angioplasty ◽

Brachiocephalic Vein ◽

Central Vein ◽

Vein Thrombosis ◽

Catheter Directed Thrombolysis ◽

Stage Renal Disease ◽

End Stage ◽

Vein Stenosis

Central vein stenosis and thrombosis are frequent in patients on haemodialysis for end-stage renal disease. Its management includes anticoagulation, systemic or catheter-directed thrombolysis, mechanical thrombectomy and percutaneous transluminal angioplasty (PTA). Use of mechanical thrombectomy in central vein thrombosis has been scarcely reported. We hereby report a case of right brachiocephalic vein thrombosis with underlying stenosis, which was successfully treated by mechanical thrombectomy followed by PTA and stenting. The patient had a favourable 10 months of follow-up.

Download Full-text

Symptomatic kidney stones in an anuric patient on dialysis: A case report

Urologia Journal ◽

10.1177/03915603211035927 ◽

2021 ◽

pp. 039156032110359

Author(s):

Hossein Dialameh ◽

Farshad Namdari ◽

Mehrdad Mahalleh ◽

Mohammad Lotfi ◽

Zoha Ali

Keyword(s):

Renal Colic ◽

Chest Ct ◽

Chief Complaint ◽

Flank Pain ◽

The Past ◽

Extracorporeal Shock Wave ◽

Stage Renal Disease ◽

End Stage ◽

Prompt Diagnosis ◽

Chest Ct Scan

Introduction: Renal colic is a colicky-type of flank pain that can commonly be presented in patients undergoing dialysis especially if they are anuric considering the fact that there are multiple controversies and little published experience on this topic, we found it very important to report this case. We also aimed to increase awareness and emphasize the importance of renal colic in anuric patients on dialysis. Case description: We herein report a case of a 42-year old man with a chief complaint of bilateral colic flank pain, He had developed end stage renal disease due to ADPKD and was on hemodialysis since the past 5 years. Previously, he went through a series of workup but was left undiagnosed. Abdomen-pelvic and chest CT scan without contrast was performed showing bilateral renal pelvic stones and some nephrocalcinosis in both kidneys. bilateral ureteroscopy was performed and bilateral DJ was installed for a total of 6 weeks and extracorporeal shock wave lithotripsy was done. With prompt diagnosis, the patient was pain free and stone free before discharge. The patient is also reported to be stone free 6 months after the procedure. Conclusion: Patients on dialysis are still capable of forming symptomatic renal tract stones even if they are anuric.

Download Full-text

Living Kidney Donation: Practical Considerations on Setting Up a Program

Transplantology ◽

10.3390/transplantology2010008 ◽

2021 ◽

Vol 2 (1) ◽

pp. 75-86

Author(s):

Maria Irene Bellini ◽

Vito Cantisani ◽

Augusto Lauro ◽

Vito D’Andrea

Keyword(s):

Good Practice ◽

Kidney Donation ◽

Living Kidney Donation ◽

Exchange Programs ◽

Paired Exchange ◽

Stage Renal Disease ◽

End Stage ◽

Additional Value ◽

Work Up

Living kidney donation represents the best treatment for end stage renal disease patients, with the potentiality to pre-emptively address kidney failure and significantly expand the organ pool. Unfortunately, there is still limited knowledge about this underutilized resource. The present review aims to describe the general principles for the establishment, organization, and oversight of a successful living kidney transplantation program, highlighting recommendation for good practice and the work up of donor selection, in view of potential short- and long-terms risks, as well as the additional value of kidney paired exchange programs. The need for donor registries is also discussed, as well as the importance of lifelong follow up.

Download Full-text

Vasculitis ANCA: Alternativas de tratamiento y las expectativas en los pacientes

Karger Kompass Neumología ◽

10.1159/000513798 ◽

2021 ◽

pp. 1-2

Author(s):

Carolina Aguilar-Martínez

Keyword(s):

Systematic Review ◽

Cox Regression ◽

Meta Analysis ◽

Significant Risk ◽

Limiting Factor ◽

Significant Survival ◽

Stage Renal Disease ◽

End Stage ◽

Immunosuppression Therapy

Background: The benefits of treating anti-neutrophil cytoplasmic autoantibody-associated vasculitis (AAV) in advancing age remains unclear with most published studies defining elderly as ≥65 years. This study aims to determine outcomes of induction immunosuppression in patients aged ≥75 years. Methods: A cohort of patients aged ≥75 years with a diagnosis of AAV between 2006 and 2018 was constructed from 2 centres. Follow-up was to 2 years or death. Analysis included multivariable Cox regression to compare mortality and end-stage renal disease (ESRD) based on receipt of induction immunosuppression therapy with either cyclophosphamide or rituximab. A systematic review of outcome studies was subsequently undertaken amongst this patient group through Pubmed, Cochrane and Embase databases from inception until October 16, 2019. Results: Sixty-seven patients were identified. Mean age was 79 ± 2.9 years and 82% (n = 55) received induction immunosuppression. Following systematic review, 4 studies were eligible for inclusion, yielding a combined total of 290 patients inclusive of our cohort. The aggregated 1-year mortality irrespective of treatment was 31% (95% CI 25–36%). Within our cohort, induction immunosuppression therapy was associated with a significantly lower 2-year mortality risk (hazard ratio [HR] 0.29 [95% CI 0.09–0.93]). The pooled HR by meta-analysis confirmed this with a significant risk reduction for death (HR 0.31 [95% CI 0.16–0.57], I2 = 0%). Treated patients had a lower pooled rate of ESRD, but was not statistically significant (HR 0.71 [95% CI 0.15–3.35]). Conclusion: This meta-analysis suggests that patients ≥75 years with AAV do benefit from induction immunosuppression with a significant survival benefit. Age alone should not be a limiting factor when considering treatment.

Download Full-text

Kidney transplantation in the extremely elderly from extremely aged deceased donors: a kidney for each age

Nephrology Dialysis Transplantation ◽

10.1093/ndt/gfz293 ◽

2020 ◽

Vol 35 (4) ◽

pp. 687-696

Author(s):

Jimena Cabrera ◽

Mario Fernández-Ruiz ◽

Hernando Trujillo ◽

Esther González ◽

María Molina ◽

...

Keyword(s):

Kidney Transplantation ◽

Graft Survival ◽

Cox Regression ◽

Patient Survival ◽

Incidence Rates ◽

Allocation Policy ◽

Recipient Age ◽

Deceased Donors ◽

Stage Renal Disease ◽

End Stage

Abstract Background Advances in life expectancy have led to an increase in the number of elderly people with end-stage renal disease (ESRD). Scarce information is available on the outcomes of kidney transplantation (KT) in extremely elderly patients based on an allocation policy prioritizing donor–recipient age matching. Methods We included recipients ≥75 years that underwent KT from similarly aged deceased donors at our institution between 2002 and 2015. Determinants of death-censored graft and patient survival were assessed by Cox regression. Results We included 138 recipients with a median follow-up of 38.8 months. Median (interquartile range) age of recipients and donors was 77.5 (76.3–79.7) and 77.0 years (74.7–79.0), with 22.5% of donors ≥80 years. Primary graft non-function occurred in 8.0% (11/138) of patients. Cumulative incidence rates for post-transplant infection and biopsy-proven acute rejection (BPAR) were 70.3% (97/138) and 15.2% (21/138), respectively. One- and 5-year patient survival were 82.1 and 60.1%, respectively, whereas the corresponding rates for death-censored graft survival were 95.6 and 93.1%. Infection was the leading cause of death (46.0% of fatal cases). The occurrence of BPAR was associated with lower 1-year patient survival [hazard ratio (HR) = 4.21, 95% confidence interval (CI) 1.64–10.82; P = 0.003]. Diabetic nephropathy was the only factor predicting 5-year death-censored graft survival (HR = 4.82, 95% CI 1.08–21.56; P = 0.040). Conclusions ESRD patients ≥75 years can access KT and remain dialysis free for their remaining lifespan by using grafts from extremely aged deceased donors, yielding encouraging results in terms of recipient and graft survival.

Download Full-text

Ifosfamide May Be Safely Used in Patients with End Stage Renal Disease on Hemodialysis

Sarcoma ◽

10.1155/2009/575629 ◽

2009 ◽

Vol 2009 ◽

pp. 1-5 ◽

Cited By ~ 2

Author(s):

Sheron Latcha ◽

Robert G. Maki ◽

Gary K. Schwartz ◽

Carlos D. Flombaum

Keyword(s):

Renal Disease ◽

End Stage Renal Disease ◽

Response To Therapy ◽

Common Side Effect ◽

Pharmacokinetic Data ◽

Life Threatening ◽

Stage Renal Disease ◽

End Stage ◽

Metastatic Sarcoma ◽

Radiographic Improvement

Background. Pharmacokinetic data on clearance of ifosfamide in hemodialysis patients are limited. Consequently, these patients are excluded from therapy with this agent. We review the outcomes for patients at our institution with end stage renal disease on dialysis who received ifosfamide for metastatic sarcoma.Patients and Methods. We treated three patients with end stage renal disease on hemodialysis with escalating doses of ifosfamide. Data on radiographic response to therapy, WBC and platelet counts, signs or symptoms of infection, neuropathy and bladder toxicity are reported. Starting doses of ifosfamide were based on review of the literature available with subsequent modifications based on each patient's prior exposure to myelosuppressive agents and on symptoms of neurotoxicity and the degree of myelosuppression following each cycle of chemotherapy.Results. Myelosuppression was the most common side effect from therapy, but no patient developed a life threatening infection, neurotoxicity, or hematuria. One patient developed epistaxis in the setting of thrombocytopenia while on warfarin therapy. All patients had clinical evidence for therapeutic response and two had documented radiographic improvement following ifosfamide administration.Conclusion. Ifosfamide can be used safely in combination with hemodialysis in patients with end stage renal disease.

Download Full-text

Vascular access types in patients starting hemodialysis after failed kidney transplant: does close nephrology follow-up matter?

The Journal of Vascular Access ◽

10.5301/jva.5000631 ◽

2016 ◽

Vol 18 (1) ◽

pp. 22-25 ◽

Cited By ~ 3

Author(s):

Naveed Ul Haq ◽

Mohamed Said Abdelsalam ◽

Mohammed Mahdi Althaf ◽

Abdulrahman Ali Khormi ◽

Hassan Al Harbi ◽

...

Keyword(s):

Vascular Access ◽

Clinic Visit ◽

Study Data ◽

Renal Transplant Recipients ◽

Transplant Recipients ◽

Early Referral ◽

Period Data ◽

Stage Renal Disease ◽

End Stage

Background Native arteriovenous fistulae (AVFs) are preferred while central venous catheters (CVCs) are least suitable vascular access (VA) in patients requiring hemodialysis (HD). Unfortunately, around 80% of patients start HD with CVCs. Late referral to nephrologist is thought to be a factor responsible for this. We retrospectively analyzed the types of VA at HD initiation in renal transplant recipients followed by nephrologists with failed transplant. If early referral to nephrologist improves AVF use, these patients should have higher prevalence of AVF at HD initiation. Methods All patients who failed their kidney transplants from January 2002 to April 2013 were included in the study. Data regarding planning of VA by nephrologist, documented discussion about renal replacement therapy (RRT), estimated glomerular filtration rate (eGFR) at 6 months and last clinic visit before HD initiation, time of VA referral, and subsequent VA at dialysis initiation were gathered and analyzed. Results Eighty-three patients failed their transplants during study period. Data were inaccessible in six patients. Eleven patients started peritoneal dialysis (PD) while 66 started HD. Thirty-two had previous functioning VA while 34 needed VA. There were 11/34 patients (32%) with eGFR <15 mL/min at six months while 21/34 (61%) had eGFR <15 mL/min at last clinic visit before HD initiation. Only 11/34 (32%) had documented RRT discussion, 8/34 (24%) had VA referral, and 7/34 (21%) had vein mapping. A total of 30/34 (88.3%) started HD with CVC while 4/34 (11.3%) started HD with AVF (p<0.0001). Conclusions Early referral to nephrologist by itself may not improve VA care amongst patient with end-stage renal disease.

Download Full-text

MO282EARLY RENAL RECOVERY AFTER A FIRST FLARE OF PAUCI IMMUNE GLOMERULONEPHRITIS

Nephrology Dialysis Transplantation ◽

10.1093/ndt/gfab104.0040 ◽

2021 ◽

Vol 36 (Supplement_1) ◽

Author(s):

Jeremy Zaworski ◽

Cyrille Vandenbussche ◽

Pierre Bataille ◽

Eric Hachulla ◽

Francois Glowacki ◽

...

Keyword(s):

Renal Disease ◽

End Stage Renal Disease ◽

Interstitial Fibrosis ◽

Univariate Analysis ◽

Renal Recovery ◽

Neurological Involvement ◽

Factors Associated ◽

Stage Renal Disease ◽

End Stage

Abstract Background and Aims Renal involvement is a severe manifestation of ANCA-associated vasculitis. Patients often progress to end-stage renal disease. The potential for renal recovery after a first flare has seldom been studied. Our objectives were to describe the evolution of the estimated glomerular filtration rate (eGFR) and identify factors associated with the change in eGFR between diagnosis and follow-up at 3 months (ΔeGFRM0–M3) in a cohort of patients with a first flare of pauci-immune glomerulonephritis. Methods This was a retrospective study over the period 2003–2018 of incident patients in the Nord-Pas-de-Calais (France). Patients were recruited if they had a first histologically-proven flare of pauci immune glomerulonephritis with at least 1 year of follow up. Kidney function was estimated with MDRD-equation and analysed at diagnosis, 3rd, 6th and 12th months. The primary outcome was ΔeGFRM0–M3. Factors evaluated were histological (Berden classification, interstitial fibrosis, percentage of crescents), clinical (extra-renal manifestations, sex, age) or biological (severity of acute kidney injury, dialysis, ANCA subtype). Results One hundred and seventy-seven patients were included. The eGFR at 3 months was significantly higher than at diagnosis (mean ± standard deviation, 40 ± 24 vs 28 ± 26 ml/min/1.73 m2, p < 0.001), with a ΔeGFRM0–M3 of 12 ± 19 ml/min/1.73 m2. The eGFR at 12 months was higher than at 3 months (44 ± 13 vs 40 ± 24 ml/min/1.73m2, p = 0.003). The factors significantly associated with ΔeGFRM0–M3 in univariate analysis were: sclerotic class according to Berden classification, percentage of interstitial fibrosis, percentage of cellular crescents, acute tubular necrosis, neurological involvement. The factors associated with ΔeGFRM0–M3 in multivariate analysis were the percentage of cellular crescents and neurological involvement. The mean increase in eGFR was 2.90 ± 0.06 ml/min/1.73m2 for every 10-point gain in the percentage of cellular crescents. ΔeGFRM0–M3 was not associated with the risks of end-stage renal disease or death in long-term follow-up. Conclusions Early renal recovery after a first flare of pauci-immune glomerulonephritis occurred mainly in the first three months of treatment. The percentage of cellular crescents was the main independent predictor of early renal recovery.

Download Full-text

MO302ACUTE POST-INFECTIOUS GLOMERULONEPHRITIS IN ADULTS: A TUNISIAN SINGLE CENTER EXPERIENCE

Nephrology Dialysis Transplantation ◽

10.1093/ndt/gfab104.0060 ◽

2021 ◽

Vol 36 (Supplement_1) ◽

Author(s):

Imen El Meknassi ◽

Mrabet Sanda ◽

Guedri Yosra ◽

Zellema Dorsaf ◽

Azzabi Awatef ◽

...

Keyword(s):

Acute Kidney Injury ◽

Kidney Injury ◽

Acute Glomerulonephritis ◽

Industrialized Countries ◽

Single Center Experience ◽

Nephritic Syndrome ◽

Stage Renal Disease ◽

End Stage ◽

Post Infectious

Abstract Background and Aims Acute post-infectious glomerulonephritis (APIGN) is a reactive immunological disease. Its prevalence in industrialized countries is declining contrasting with developed ones. It is uncommon in adults but the prognosis may be reserved. The aim of our study was to evaluate the epidemiological, clinical and histological features of APIGN as well as its prognosis. Method A retrospective and descriptive study was conducted in our department. Were included all cases of histologically proven APIGN between December 2006 and December 2017. Results We had collected 38 cases. The mean age was 37.7 ± 17.8 years. The sex ratio was 1.92. Twelve (31.6%) patients were diabetic and four of them had already a chronic kidney disease (CKD). APIGN was preceded by an infection in 27 cases with an average interval of 10 ± 5 days. The most common site of infection was the respiratory tract (15 cases). At presentation, 27 patients had nephritic syndrome and 13 had nephrotic-range proteinuria. Hematuria was observed in 97.4%, peripheral edema in 84.2% and hypertension in 73.7% of cases. Most patients (78.9%) had acute kidney injury and 10 (26.3%) patients required dialysis. Renal biopsy had shown benign acute glomerulonephritis in 31 cases and malignant form in 7 cases. An underlying nephropathy was found in 12 cases with mostly a diabetic nephropathy. Corticosteroids were used in 3 cases of benign APIGN and 5 cases of malignant form. During the follow-up, CKD was noted in 14(36.8%) patients including 7(18.4%) patients who progressed to end-stage renal disease. Poor prognostic factors were diabetes, the presence of an underlying nephropathy in the biopsy, acute kidney injury and the need for dialysis. Conclusion The APIGN is uncommon in adults, yet its prognosis may be reserved with progression to CKD.

Download Full-text