Introduction: Calciphylaxis is a rare but potentially fatal disease commonly occurred in dialysis patients. Despite some previous studies on risk factors for calciphylaxis, there is still a lack of data from Chinese population. Methods: The retrospective matched case-control study about calciphylaxis was performed in Zhongda Hospital affiliated to Southeast University. The case group involved 20 hemodialysis patients who were newly diagnosed with calciphylaxis from October 2017 to December 2018. The 40 noncalciphylaxis patients undergoing dialysis with the same age and duration of dialysis were randomly selected as controls. Results: Most of calciphylaxis patients were male and elderly, while overweight people were more susceptible to the disease. Although incidence of secondary hyperparathyroidism was higher in calciphylaxis patients, the differences in duration of elevated serum intact parathyroid hormone (iPTH) and its highest value did not reach statistical significance compared with controls. No significant difference in warfarin therapy was discernible between two groups. The univariate regression analysis indicated that male, score of use of activated vitamin D and its analogues, corrected serum calcium level, serum phosphate, Ca  P product, iPTH, albumin, and alkaline phosphatase (ALP) level were significantly associated with calciphylaxis. Elevated levels of serum phosphate (OR 4.584, p ¼ 0.027) and ALP (OR 1.179, p ¼ 0.036), decreased level of serum albumin (OR 1.330, p ¼ 0.013) were independent risk factors after multivariate analysis. Conclusion: This is the first report of risk factors associated with calciphylaxis in China. Increased levels of serum phosphate and ALP, decreased level of serum albumin were vital high-risk factors for calciphylaxis in Chinese hemodialysis population.

BackgroundCalciphylaxis is a grievous life-threatening vascular disease that commonly affects dialysis population. This is the first epidemiological survey of calciphylaxis initiated in China.MethodsIn the cross-sectional survey, a stratified sampling method was used to select 24 dialysis centers in Jiangsu Province. The participants were all adult patients in each center who had been on hemodialysis for more than 6 months. Calciphylaxis patients were uniformly diagnosed based on characteristic skin lesions and histopathological features.ResultsA total of 3,867 hemodialysis patients (average age of 55.33 ± 13.89 years; 61.81% of males) were included. Forty eight cases were diagnosed with calciphylaxis, and prevalence was 1.24%. Among calciphylaxis patients, 33 cases were male, and the average age and median dialysis duration were 53.85 ± 15.17 years and 84.00 (48.00, 138.75) months, respectively. Skin biopsy was performed in 70.83% of calciphylaxis patients, and positive rate was 64.71%. Meanwhile, the positive rate of bone scintigraphy in the diagnosis of calciphylaxis was 62.5%. The prevalence of hyperparathyroidism in case group was as high as 72.92% with longer duration, and 42.86% had undergone parathyroidectomy. Multivariate analysis indicated that increased BMI, prolonged dialysis duration, warfarin therapy, hyperparathyroidism, diabetes, tumors, low serum albumin and high serum alkaline phosphatase levels were high-risk factors for calciphylaxis.ConclusionsThe prevalence of calciphylaxis in Chinese hemodialysis patients was 1.24% according to regional epidemiological survey, but its actual prevalence would be presumably far beyond present data. It's urgent to improve clinical understanding of calciphylaxis, and multifaceted diagnostic methods should be applied for early screening.

Background Sodium thiosulfate (STS) can be used to treat patients diagnosed with calciphylaxis, which is a rare life-threatening syndrome. However, our patients treated with the recommended STS regimen presented with serious adverse events, resulting in treatment withdrawal. Then an optimized STS regimen was used to increase the tolerance of patients to STS and improve treatment continuation. The curative effect of the new regimen is not yet definite. Therefore, this study aimed to evaluate the response to the use of the optimized STS regimen for the treatment of calciphylaxis in Chinese patients during the first three courses of treatment. Methods Demographic, clinical, and laboratory data were retrospectively collected on 31 calciphylaxis patients with chronic kidney disease (CKD) or end-stage kidney disease (ESKD) treated with the optimized STS regimen. The primary outcome was a clinical improvement. The secondary outcomes included survival rate and adverse events. Results Twenty-five patients (over 80%) achieved clinical improvement considering improvement or nonspecific changes of skin lesions (80.65%) and pain relief (100%). Furthermore, 54.84% of patients did not experience any adverse events and none died from complications. During a median follow-up of 9 months (interquartile range 4‒19), 27 patients (87.10%) survived; additionally, 13 patients (41.94%) survived after a one-year follow-up period. Conclusion The optimized STS regimen is relatively safe, associated with satisfactory outcomes, and well tolerated by patients for short to medium treatment duration. Hence, it is a promising approach for the treatment of patients diagnosed with calciphylaxis.

Calciphylaxis, also known as calcific uremic arteriolopathy (CUA), is typically characterized by subcutaneous tissue calcification and excruciatingly painful cutaneous lesions. Calciphylaxis is a crippling disease with a mortality rate as high as 60%-80%, which affects 1%-4% of the population with end-stage renal disease. 1 Due to the high mortality and morbidity of calciphylaxis, it is critical for dermatologists to make early diagnosis and appropriate management, yet currently only 56% of calciphylaxis cases are correctly diagnosed by conventional histological stains. 2 The pathologic diagnosis of calciphylaxis primarily depends on high-quality staining technique. Large and chunky calcification in subcutaneous vessels can be determined by conventional histological stains such as HE, Alizarin S and von Kossa. However, these imaging techniques suffer from low sensitivity in detecting subtle calcification. 3 The subtle calcium deposits including perieccrine calcification, and intravascular and extravascular microcalcification were found to be highly specific to early diagnosis of cutaneous calciphylaxis, but the detection of these calcium deposits is difficult and not precise. 4 Fine calcium deposits even were not appreciable on those stains in some cases of proven cutaneous calciphylaxis clinically. 2 Therefore, an effective diagnosis of calciphylaxis faces a great challenge and more sensitive calcification staining is in high demand.Here, we describe a rapid, sensitive and reliable staining method using Fluo-3 AM that could be a promising diagnostic tool for cutaneous calciphylaxis. The most important property of Fluo-3 AM is an absorption spectrum compatible with excitation at 488 nm by laser sources, and a very large fluorescence intensity increase in response to Ca 2+ binding. Fluo-3 AM imaging has been widely used calcium signalling examinations in flow cytometry and confocal laserscanning microscopy, 5 but no published study has applied Fluo-3 AM to calcification detection in paraffin section of skin biopsy.We have studied 41 patients with calciphylaxis that had been confirmed by skin biopsy combined with clinical findings and imaging studies. The histopathological examination of skin biopsy was performed using conventional staining (haematoxylin-eosin HE, von Kossa and Alizarin red) and Fluo-3/AM staining. For each case, the following histological stains of calcification were evaluated: chunky calcification and stippled calcification within vessels and

BACKGROUND AND AIMS Calciphylaxis is a rare life-threatening syndrome. Although sodium thiosulfate (STS) showed clinical improvement, some patients presented with serious adverse events and withdrew from treatment. The optimized STS approach included four features: repeated treatment courses, small initial dose, once daily, and escalating low-to-moderate dose. The optimized STS approach, named ‘Zhong Da STS Therapy’ (Zhong Da: the name of a hospital, affiliated with Southeast University, School of Medicine, Nanjing, Jiangsu, China), was generalized with four features: repeated treatment courses, small initial dose, daily medicine, and daily escalating low-to-moderate dose of STS. STS from the initial dose (5 g) was administered intravenously once daily escalating to the highest daily dose as a maintenance dose (no more than 10 g). This study retrospectively evaluated the efficacy and safety of optimized STS therapy for calciphylaxis in China. METHOD The medical records of maintenance hemodialysis or CKD patients who were first diagnosed with calciphylaxis and received STS treatment at Zhong Da Hospital affiliated to Southeast University between October 2017 and October 2019 were retrospectively evaluated. The exclusion criteria of this retrospective study were death at the time of diagnosis (retrospectively confirmed cases) and did not receive the optimized STS approach treatment. The data to evaluate the efficacy included the changes in skin lesions, pain relief, survival rate and laboratory data. Adverse events were summarized as safety parameters. RESULTS The mean age was 51.10 ± 14.85 years old and 71% of patients were male. By October 2019, 87.1% of patients survived, 74.2% markedly improved and 41.9% of patients were followed up for more than 1 year with 100% survival. About 27 (87.10%) patients had typical skin lesions with 66.70% ulceration and skin lesions showed a predominantly peripheral distribution. After the first course, skin lesions improved by 77.8%, and 38.1% of pain sufferers were free from analgesics. The reduction of the Numerical Pain Rating Scale (NPRS) (P < 0.001) is related significantly to the treatment course. Furthermore, during the entire treatment session, more than half of the patients (54.84%) never experienced adverse events, only one withdrew treatment and no one died from complications. And most adverse events appeared in the course of increasing the STS dose and would be relieved when we stopped adding it or decreased to the day-before dose. CONCLUSION Optimized STS therapy is a promising approach for calciphylaxis patients because it is relatively safe and associated with good outcomes.