Dataset Information

Refractory wounds induced by normal-renal calciphylaxis: An under-recognised calcific arteriolopathy.

ABSTRACT:

Background

Normal-renal calciphylaxis (NRC) is a devastating calcific vasculopathy characterised by refractory wounds. It is more likely to be misdiagnosed because of the absence of renal insufficiency. Failure to effectively control may lead to rapid deterioration and ultimately death. However, current knowledge of it is still inadequate.

Objectives

To provide a relatively comprehensive review of NRC.

Methods

Nine electronic medical databases including PubMed, Web of Science, Embase, the Cochrane Library and so on were searched from inception to April 1, 2022. Articles in which calciphylaxis was diagnosed in patients with normal renal function were included.

Results

Totally 140 articles were retrieved with 187 patients (median age, 62 years [IQR, 49.63 ~ 75.70 years]; 83.42% female; 84.5% Caucasian). Vitamin K antagonism (43.32%) and diabetes (39.57%) accounted for most of the concomitant factors. Cutaneous presentations were the most common. Pathological confirmation was made in 97.86% of patients with an accuracy of 99.45%. Multidisciplinary therapeutic strategies were usually implemented while the final prognosis was not ideal, with a 6-month mortality rate of 21.10% and a 1-year mortality rate of 27.52%.

Conclusion

NRC is an under-recognised disease caused by a variety of factors; meanwhile, multidisciplinary efforts are required to inform diagnostic and therapeutic decisions.

SUBMITTER: Wu J

PROVIDER: S-EPMC10031205 | biostudies-literature | 2023 Apr

REPOSITORIES: biostudies-literature

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Publications

Refractory wounds induced by normal-renal calciphylaxis: An under-recognised calcific arteriolopathy.

Wu Jing J Chen Lihong L Dang Fangping F Zha Panpan P Li Rao R Ran Xingwu X

International wound journal 20220906 4

<h4>Background</h4>Normal-renal calciphylaxis (NRC) is a devastating calcific vasculopathy characterised by refractory wounds. It is more likely to be misdiagnosed because of the absence of renal insufficiency. Failure to effectively control may lead to rapid deterioration and ultimately death. However, current knowledge of it is still inadequate.<h4>Objectives</h4>To provide a relatively comprehensive review of NRC.<h4>Methods</h4>Nine electronic medical databases including PubMed, Web of Scien ...[more]

PMID: 36068658

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Project description:Importance:Calcific uremic arteriolopathy (CUA), a rare, potentially fatal, disease with calcium deposits in skin, mostly affects patients with end-stage renal disease who are receiving dialysis. Chemical composition and structure of CUA calcifications have been poorly described. Objectives:To describe the localization and morphologic features and determine the precise chemical composition of CUA-related calcium deposits in skin, and identify any mortality-associated factors. Design, Setting, and Participants:A retrospective, multicenter cohort study was conducted at 7 French hospitals including consecutive adults diagnosed with CUA between January 1, 2006, and January 1, 2017, confirmed according to Hayashi clinical and histologic criteria. Patients with normal renal function were excluded. For comparison, 5 skin samples from patients with arteriolosclerosis and 5 others from the negative margins of skin-carcinoma resection specimens were also analyzed. Main Outcomes and Measures:Localization and morphologic features of the CUA-related cutaneous calcium deposits were assessed with optical microscopy and field-emission-scanning electron microscopy, and the chemical compositions of those deposits were evaluated with μ Fourier transform infrared spectroscopy, Raman spectroscopy, and energy dispersive radiographs. Results:Thirty-six patients (median [range] age, 64 [33-89] years; 26 [72%] female) were included, and 29 cutaneous biopsies were analyzed. Calcific uremic arteriolopathy and arteriolosclerosis skin calcifications were composed of pure calcium-phosphate apatite. Calcific uremic arteriolopathy vascular calcifications were always circumferential, found in small to medium-sized vessels, with interstitial deposits in 22 (76%) of the samples. A thrombosis, most often in noncalcified capillary lumens in the superficial dermis, was seen in 5 samples from patients with CUA. Except for calcium deposits, the vessel structure of patients with CUA appeared normal, unlike thickened arteriolosclerotic vessel walls. Twelve (33%) patients died of CUA. Conclusions and Relevance:Calcific uremic arteriolopathy-related skin calcifications were exclusively composed of pure calcium-phosphate apatite, localized circumferentially in small to medium-sized vessels and often associated with interstitial deposits, suggesting its pathogenesis differs from that of arteriolosclerosis. Although the chemical compositions of CUA and arteriolosclerosis calcifications were similar, the vessels' appearances and deposit localizations differed, suggesting different pathogenetic mechanisms.

Dataset Information

Refractory wounds induced by normal-renal calciphylaxis: An under-recognised calcific arteriolopathy.

Background

Objectives

Methods

Results

Conclusion

Publications

Refractory wounds induced by normal-renal calciphylaxis: An under-recognised calcific arteriolopathy.

Similar Datasets

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