Quantifying a Rare Disease in Administrative Data: The Example of Calciphylaxis

Nigwekar, Sagar U.; Solid, Craig A.; Ankers, Elizabeth; Malhotra, Rajeev; Eggert, William; Turchin, Alexander; Thadhani, Ravi; Herzog, Charles A.

doi:10.1007/s11606-014-2910-1

Cited by 83 publications

(64 citation statements)

References 33 publications

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“…The response to any therapeutic regimen, however, appears to be poor. Mortality rates in long-term hemodialysis patients with CUA were almost three times higher than for long-term hemodialysis patients without CUA in the United States Renal Data System [14].…”

Section: Prognosismentioning

confidence: 81%

Calciphylaxis: Early Detection and Off-Label Treatment With Sodium Thiosulfate

London

Eter

2017

World J Nephrol Urol

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Calciphylaxis or calcific uremic arteriolopathy (CUA) is a rare disorder of calcium and phosphate metabolism most often manifesting in end-stage renal disease (ESRD) patients. The typical clinical picture is that of a necrotic cutaneous ulceration with focal or diffuse distribution, most often manifesting on the lower extremities. We report a 49-year-old Caucasian male with ESRD on hemodialysis who presented to the emergency department with complaints of lower extremity pain and multiple cutaneous necrotic lesions on his lower extremities. Patient reports seeking assistance from several medical providers over a 3-month period. Upon admission and further evaluation, a diagnosis of calciphylaxis was made clinically and confirmed by a skin biopsy. Sodium thiosulfate was initiated and the clinical picture improved significantly. Sodium thiosulfate is traditionally used as an antidote for cyanide poisoning, and it has demonstrated beneficial outcomes in most patients afflicted with CUA. The rationale behind sodium thiosulfate therapy in CUA is its role in chelating calcium into calcium thiosulfate, which increases its solubility leading to improved renal clearance. It is essential to raise awareness amongst physicians and medical practitioners alike, as early recognition and initiation of appropriate treatment can improve the patients' quality of life and more importantly, decrease mortality.

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Section: Prognosismentioning

confidence: 81%

Calciphylaxis: Early Detection and Off-Label Treatment With Sodium Thiosulfate

London

Eter

2017

World J Nephrol Urol

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“…4,9,18 Considering the sample size limitation of our study and single center experience, definitive conclusions regarding comparative effectiveness of PD vs HD for calciphylaxis risk cannot be made; however, our study adds to the debate in the literature regarding whether PD as a dialysis modality confers a higher calciphylaxis risk than HD. 12,13,19 It is important to note that although PD was the dialysis modality for our study patients when calciphylaxis was diagnosed, many patients were previously on HD and required transition to PD for access failure or recurrent hypotension.…”

Section: Calciphylaxis In Peritoneal Dialysismentioning

confidence: 83%

“…Patients who developed calciphylaxis were identified by applying a previously published algorithm that incorporates natural language processing and by manual chart review. 4 Diagnosis of calciphylaxis was clinically suspected and had histopathological confirmation in all patients. Data regarding demographics, vital signs, comorbidities, dialysis and ESRDrelated characteristics, medications and mineral bone parameters, and nutritional parameters were abstracted from the medical record.…”

Section: Methodsmentioning

confidence: 99%

“…These data suggest that the incidence of calciphylaxis in HD patients is approximately 3.5 cases per 1,000 patient-years and it may be on the rise. 3,4 Diabetes mellitus, obesity, abnormalities in mineral bone parameters, and warfarin therapy have been described as calciphylaxis risk factors for HD patients. 3,[5][6][7] Cinacalcet has been reported to reduce the risk…”

Section: Introductionmentioning

confidence: 99%

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Calciphylaxis in peritoneal dialysis patients: a single center cohort study

Zhang¹,

Corapi²,

Luongo³

et al. 2016

IJNRD

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Background: Calciphylaxis is a rare but devastating condition in end-stage renal disease (ESRD) patients. Most research in the field of calciphylaxis is focused on hemodialysis (HD) patients; however, data on calciphylaxis incidence, risk factors, and mortality in peritoneal dialysis (PD) patients are limited. Methods: In this cohort study, we examined data from adult patients who initiated PD for ESRD management at our institute's PD unit from January 2001 to December 2015. Associations with the development of calciphylaxis were examined for clinical, laboratory, and medication exposures. Incidence of calciphylaxis and mortality in PD patients who developed calciphylaxis were analyzed. Treatments administered to treat calciphylaxis in PD patients were summarized. Results: In this cohort of 63 patients, 7 patients developed calciphylaxis (incidence rate: 9.0 per 1,000 patient-years). Median age of PD patients who developed calciphylaxis was 50 years, 57% were white, 71% females, and 71% were previously on HD. Female sex, obesity, HD as a prior dialysis modality, recurrent hypotension, elevated time-averaged serum phosphorous levels, reduced time-averaged serum albumin levels, and warfarin therapy were associated with increased calciphylaxis risk in univariate logistic regression analyses. Intravenous sodium thiosulfate was administered in 57% of PD patients who developed calciphylaxis. One-year mortality in PD patients who developed calciphylaxis was 71% despite multimodal treatment including sodium thiosulfate, hyperbaric oxygen, cinacalcet, and wound debridement. Conclusion: Calciphylaxis is a rare but frequently fatal condition in PD patients. Our study provides critical early insights into calciphylaxis incidence, risk factors, and prognosis in PD patients. Sample size and characteristics of patients included in our study limit generalizability to overall PD population and warrant examination in larger independent studies.

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“…[1][2][3][4] Estimates of the 1 year survival rate and median survival time are about 46% and 1.5 years, respectively. 5 Patients with calciphylaxis develop calcium deposits in their vasculature and soft tissues that may result in vessel thrombosis and tissue necrosis.…”

Section: Introductionmentioning

confidence: 99%

Raman spectroscopy for label-free identification of calciphylaxis

et al. 2015

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Abstract. Calciphylaxis is a painful, debilitating, and premorbid condition, which presents as calcified vasculature and soft tissues. Traditional diagnosis of calciphylaxis lesions requires an invasive biopsy, which is destructive, time consuming, and often leads to exacerbation of the condition and infection. Furthermore, it is difficult to find small calcifications within a large wound bed. To address this need, a noninvasive diagnostic tool may help clinicians identify ectopic calcified mineral and determine the disease margin. We propose Raman spectroscopy as a rapid, pointof-care, noninvasive, and label-free technology to detect calciphylaxis mineral. Debrided calciphylactic tissue was collected from six patients and assessed by microcomputed tomography (micro-CT). Micro-CT confirmed extensive deposits in three specimens, which were subsequently examined with Raman spectroscopy. Raman spectra confirmed that deposits were consistent with carbonated apatite, consistent with the literature. Raman spectroscopy shows potential as a noninvasive technique to detect calciphylaxis in a clinical environment. © The Authors. Published by SPIE under a Creative Commons Attribution 3.0 Unported License. Distribution or reproduction of this work in whole or in part requires full attribution of the original publication, including its DOI.

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Quantifying a Rare Disease in Administrative Data: The Example of Calciphylaxis

Cited by 83 publications

References 33 publications

Calciphylaxis: Early Detection and Off-Label Treatment With Sodium Thiosulfate

Calciphylaxis: Early Detection and Off-Label Treatment With Sodium Thiosulfate

Calciphylaxis in peritoneal dialysis patients: a single center cohort study

Raman spectroscopy for label-free identification of calciphylaxis

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