The Australian Calciphylaxis Registry: reporting clinical features and outcomes of patients with calciphylaxis

Author(s):  
Irene Ruderman ◽  
Nigel D Toussaint ◽  
Carmel M Hawley ◽  
Rathika Krishnasamy ◽  
Eugenia Pedagogos ◽  
...  

Abstract Background Calciphylaxis is a rare disease, predominantly affecting patients with chronic kidney disease (CKD) and associated with significant morbidity and mortality due to progressive cutaneous calcification, necrotic ulceration and infection. Clinical registries have been established to better understand the risk factors, optimal treatments and disease outcomes of calciphylaxis. Methods We established a prospective, Internet-based clinical registry for the online notification of calciphylaxis cases in Australia. Seven institutions participated, with data recorded on patient characteristics, biochemical parameters, treatments and disease outcomes. Results Between 2014 and 2019, 47 cases of calciphylaxis were registered. The mean patient age was 66 ± 11 years and body mass index was 35 ± 9 kg/m2, with a higher proportion of females (51%). Eighty-seven percent of patients had end-stage kidney disease (ESKD), with 61% on hemodialysis or hemodiafiltration, with a median dialysis vintage of 4.8 [interquartile range (IQR) 1.7–7.4)] years. Five patients had CKD not requiring dialysis and two were kidney transplant recipients. Diabetes was present in 76% of patients and the cause of ESKD in 60%; 34% received vitamin K antagonists (VKAs) before diagnosis. The median parathyroid hormone level at diagnosis was 32 (IQR 14–50) pmol/L. The most common site of calciphylaxis was the lower limbs (63%), with 19% of patients having more than one area involved. Ten patients (22%) had a resolution of calciphylaxis and 25 died, with 50% mortality at a median of 1.6 (IQR 0.2–2.5) years from diagnosis. Conclusions The Australian Calciphylaxis Registry highlights risk factors for calciphylaxis, including diabetes, obesity and VKA use. Resolution of calciphylaxis is uncommon despite multimodal therapy and mortality from calciphylaxis in the first year following diagnosis remains high.

2020 ◽  
Vol 21 (1) ◽  
Author(s):  
Chia-Tien Hsu ◽  
Mei-Chin Wen ◽  
Hsien-Fu Chiu ◽  
Shang-Feng Tsai ◽  
Tung-Min Yu ◽  
...  

Abstract Background Transplantation with a diabetic donor kidney may have some benefits compared to remaining on the waitlist for selected patients. However, we found that some kidney transplant recipients have ongoing donor-transmitted diabetic kidney disease (DT-DKD) despite fair blood sugar control. This study aimed to survey the incidence and clinical pattern of DT-DKD in kidney transplant recipients. Methods We retrospectively reviewed the medical records of kidney transplantations in our hospital. We found 357 kidney transplantations from February 2006 to April 2018. Among these, 23 (6.4%) diabetic donor kidney transplantations were done in the study period. Results Among the 23 recipients, 6 (26.1%) displayed biopsy-proven DKD. Recipients with biopsy-proven DKD had longer dialysis vintage, higher proteinuria amount, lower last estimated glomerular filtration rate (eGFR), and a more rapid decline in the eGFR. The median fasting blood sugar level in the biopsy-proven DKD group was unexpectedly lower than the non-DKD group. Most of the pre-implantation frozen sections in biopsy-proven DKD group showed diabetic lesions worse than diabetic nephropathy (DN) class IIa. In the biopsy-proven DKD group, 5 recipients had no history of diabetes before or after transplantation. Among the 23 recipients, 5 (21.7%) were diagnosed with DT-DKD. Serial post-transplant biopsies showed the histological progression of allograft DN. Conclusions To the best of our knowledge, this is the first study to report the phenomenon of ongoing DT-DKD in kidney transplant recipients with fair blood sugar control. The zero-time pre-transplant kidney biopsy may be an important examination before the allocation of diabetic donor kidneys. Further study is needed to elucidate the possible mechanism of ongoing DT-DKD in non-diabetic recipients with fair blood sugar control as well as the impaction of pre-implantation diabetic lesion on the graft outcome.


2014 ◽  
Vol 63 (2) ◽  
pp. 236-243 ◽  
Author(s):  
Wei Yang ◽  
Dawei Xie ◽  
Amanda H. Anderson ◽  
Marshall M. Joffe ◽  
Tom Greene ◽  
...  

2020 ◽  
Author(s):  
Chia-Tien Hsu ◽  
Mei-Chin Wen ◽  
Hsien-Fu Chiu ◽  
Shang-Feng Tsai ◽  
Tung-Min Yu ◽  
...  

Abstract Background Transplantation with a diabetic donor kidney may have some benefits compared to remaining on the waitlist for selected patients. However, we found that some kidney transplant recipients have ongoing donor-transmitted diabetic kidney disease (DT-DKD) despite fair blood sugar control. This study aimed to survey the incidence and clinical pattern of DT-DKD in kidney transplant recipients.Methods We retrospectively reviewed the medical records of kidney transplantations in our hospital. We found 357 kidney transplantations from February 2006 to April 2018. Among these, 23 (6.4%) diabetic donor kidney transplantations were done in the study period.Results Among the 23 recipients, 6 (26.1%) displayed biopsy-proven DKD. Recipients with biopsy-proven DKD had longer dialysis vintage, higher proteinuria amount, lower last estimated glomerular filtration rate (eGFR), and a more rapid decline in the eGFR. The median fasting blood sugar level in the biopsy-proven DKD group was unexpectedly lower than the non-DKD group. Most of the pre-implantation frozen sections in biopsy-proven DKD group showed diabetic lesions worse than diabetic nephropathy (DN) class IIa. In the biopsy-proven DKD group, 5 recipients had no history of diabetes before or after transplantation. Among the 23 recipients, 5 (21.7%) were diagnosed with DT-DKD. Serial post-transplant biopsies showed the histological progression of allograft DN.Conclusions To the best of our knowledge, this is the first study to report the phenomenon of ongoing DT-DKD in kidney transplant recipients with fair blood sugar control. The zero-time pre-transplant kidney biopsy may be an important examination before the allocation of diabetic donor kidneys.


2014 ◽  
Vol 15 (1) ◽  
Author(s):  
Julie Boucquemont ◽  
Georg Heinze ◽  
Kitty J Jager ◽  
Rainer Oberbauer ◽  
Karen Leffondre

2019 ◽  
Author(s):  
Emily J See ◽  
Nigel D Toussaint ◽  
Michael Bailey ◽  
David W Johnson ◽  
Kevan R Polkinghorne ◽  
...  

Abstract Background Acute kidney injury (AKI) survivors are at increased risk of major adverse kidney events (MAKEs), including chronic kidney disease (CKD), end-stage kidney disease (ESKD) and death. High-risk AKI patients may benefit from specialist follow-up, but factors associated with increased risk have not been reported. Methods We conducted a retrospective study of AKI patients admitted to a single centre between 2012 and 2016 who had a baseline estimated glomerular filtration rate (eGFR) >30 mL/min/1.73 m2 and were alive and independent of renal replacement therapy (RRT) at 30 days following discharge. AKI was identified using International Classification of Diseases, Tenth Revision codes and staged according to the Kidney Disease: Improving Global Outcomes criteria. Patients were excluded if they were kidney transplant recipients or if AKI was attributed to intrinsic kidney disease. We performed Cox regression models to examine MAKEs in the first year, defined as the composite of CKD (sustained 25% drop in eGFR), ESKD (requirement for chronic RRT or sustained eGFR <15 mL/min/1.73 m2) or death. We examined secondary outcomes (CKD, ESKD and death) using Cox and competing risk regression analyses. Results We studied 2101 patients (mean ± SD age 69 ± 15 years, baseline eGFR 72 ± 23 mL/min/1.73 m2). Of these, 767 patients (37%) developed at least one MAKE (429 patients developed CKD, 21 patients developed ESKD, 375 patients died). MAKEs occurred more frequently with older age [hazard ratio (HR) 1.16 per decade, 95% confidence interval (CI) 1.10–1.24], greater severity of AKI (Stage 2 HR 1.38, 95% CI 1.16–1.64; Stage 3 HR 1.62, 95% CI 1.31–2.01), higher serum creatinine at discharge (HR 1.04 per 10 µmol/L, 95% CI 1.03–1.06), chronic heart failure (HR 1.41, 95% CI 1.19–1.67), liver disease (HR 1.68, 95% CI 1.39–2.03) and malignancy (non-metastatic HR 1.44, 95% CI 1.14–1.82; metastatic HR 2.26, 95% CI 1.80–2.83). Traditional risk factors (e.g. diabetes and cardiovascular disease) had limited predictive value. Conclusions More than a third of AKI patients develop MAKEs within the first year. Clinical variables available at the time of discharge can help identify patients at increased risk of such events.


2020 ◽  
Vol 9 (6) ◽  
pp. 1911 ◽  
Author(s):  
Camilo G. Sotomayor ◽  
Charlotte A. te Velde-Keyzer ◽  
Martin H. de Borst ◽  
Gerjan J. Navis ◽  
Stephan J.L. Bakker

After decades of pioneering and improvement, kidney transplantation is now the renal replacement therapy of choice for most patients with end-stage kidney disease (ESKD). Where focus has traditionally been on surgical techniques and immunosuppressive treatment with prevention of rejection and infection in relation to short-term outcomes, nowadays, so many people are long-living with a transplanted kidney that lifestyle, including diet and exposure to toxic contaminants, also becomes of importance for the kidney transplantation field. Beyond hazards of immunological nature, a systematic assessment of potentially modifiable—yet rather overlooked—risk factors for late graft failure and excess cardiovascular risk may reveal novel targets for clinical intervention to optimize long-term health and downturn current rates of premature death of kidney transplant recipients (KTR). It should also be realized that while kidney transplantation aims to restore kidney function, it incompletely mitigates mechanisms of disease such as chronic low-grade inflammation with persistent redox imbalance and deregulated mineral and bone metabolism. While the vicious circle between inflammation and oxidative stress as common final pathway of a multitude of insults plays an established pathological role in native chronic kidney disease, its characterization post-kidney transplant remains less than satisfactory. Next to chronic inflammatory status, markedly accelerated vascular calcification persists after kidney transplantation and is likewise suggested a major independent mechanism, whose mitigation may counterbalance the excess risk of cardiovascular disease post-kidney transplant. Hereby, we first discuss modifiable dietary elements and toxic environmental contaminants that may explain increased risk of cardiovascular mortality and late graft failure in KTR. Next, we specify laboratory and clinical readouts, with a postulated role within persisting mechanisms of disease post-kidney transplantation (i.e., inflammation and redox imbalance and vascular calcification), as potential non-traditional risk factors for adverse long-term outcomes in KTR. Reflection on these current research opportunities is warranted among the research and clinical kidney transplantation community.


Author(s):  
Dina M. AL- Ibshehy ◽  
Mahmoud A. Abouomar ◽  
Enas E. Draz ◽  
Magdy M. EL- Masry

Background:  Oral vitamin K antagonists are highly effective in the prevention and treatment of thromboembolic disease. Optimal use of these agents in clinical practice is challenged by their narrow therapeutic window. We aimed to Study the international normalized ratio values in patients on vitamin K antagonists to find out which patient characteristics that are associated with good INR control.  Methods: From June 2019 till May 2020 we studied 502 patients receiving vitamin K antagonists (VKAs) as an oral anticoagulant treatment for thromboembolic prevention for at least more than 1 month. The cases were classified into two groups according to time to therapeutic range (TTR); group I included 289 patients with TTR < 65 and group II that included 213 patients with TTR ≥ 65.  We included patients with atrial fibrillation, prosthetic valve replacement or deep venous thrombosis. Results: In univariate regression analysis, increasing age, male gender, lower level of education, diabetes mellitus, hypertension, smoking, chronic kidney disease, coronary artery disease and higher CHADS-VASC were revealed as risk factors for poor response (time to therapeutic range (TTR) < 65). With multivariate logistic regression analysis, lower level of education, HTN, smoking, CKD and higher CHADS-VASC were revealed as independent risk factors for poor response (TTR < 65). Conclusion: This study indicated that, poor education, hypertension, smoking, chronic kidney disease, and high CHADS VSAC score were independent predictors of poor time to therapeutic range (TTC) control.


Diabetes ◽  
2018 ◽  
Vol 67 (Supplement 1) ◽  
pp. 25-LB
Author(s):  
JANET B. MCGILL ◽  
MENGDI WU ◽  
RODICA POP-BUSUI ◽  
KARA R. MIZOKAMI-STOUT ◽  
WILLIAM V. TAMBORLANE ◽  
...  

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