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1.
Am J Nephrol ; 55(2): 196-201, 2024.
Article in English | MEDLINE | ID: mdl-37487472

ABSTRACT

Nephrogenic calciphylaxis is associated with multiple risk factors including long-term dialysis dependence, hyperphosphatemia, hypercalcemia, parathyroid hormone derangements, vitamin K deficiency, obesity, diabetes mellitus, warfarin use, and female sex. Bariatric surgery is known to cause altered absorption, leading to mineral and hormonal abnormalities in addition to nutritional deficiency. Prior case reports on calciphylaxis development following bariatric surgery have been published, though are limited in number. We report a case series of five bariatric patients from a single institution who developed nephrogenic calciphylaxis between 2012 and 2018. These patients had a history of bariatric surgery, and at the time of calciphylaxis diagnosis, demonstrated laboratory abnormalities associated with surgery including hypercalcemia (n = 3), hyperparathyroidism (n = 2), hypoalbuminemia (n = 5), and vitamin D deficiency (n = 5), in addition to other medication exposures such as vitamin D supplementation (n = 2), calcium supplementation (n = 4), warfarin (n = 2), and intravenous iron (n = 1). Despite the multifactorial etiology of calciphylaxis and the many risk factors present in the subjects of this case series, we submit that bariatric surgery represents an additional potential risk factor for calciphylaxis directly stemming from the adverse impact of malabsorption and overuse of therapeutic supplementation. We draw attention to this phenomenon to encourage early consideration of calciphylaxis in the differential for painful skin lesions arising after bariatric surgery as swift intervention is essential for these high-risk patients.


Subject(s)
Bariatric Surgery , Calciphylaxis , Hypercalcemia , Humans , Female , Calciphylaxis/diagnosis , Calciphylaxis/etiology , Calciphylaxis/therapy , Warfarin , Hypercalcemia/etiology , Renal Dialysis/adverse effects , Bariatric Surgery/adverse effects
2.
J Am Acad Dermatol ; 90(1): 45-51, 2024 01.
Article in English | MEDLINE | ID: mdl-37586460

ABSTRACT

BACKGROUND: Calciphylaxis is a thrombotic vasculopathy characterized by painful necrotic ulcerations. There are no Food and Drug Administration approved therapies despite high mortality. OBJECTIVE: To compare mortality and wound healing outcomes in patients treated with hyperbaric oxygen therapy (HBOT) in addition to intravenous sodium thiosulfate (IV STS) versus patients who received IV STS only. Findings were stratified by dialysis status and modality. METHODS: 93 patients were included, with 57 patients in the control group (IV STS) and 36 patients in the treatment group (HBOT + IV STS). Mortality data were analyzed with traditional survival analyses and Cox proportional hazard models. Longitudinal wound outcomes were analyzed with mixed effects modeling. RESULTS: Univariate survival analyses showed that full HBOT treatment was associated with significantly (P = .016) longer survival time. Increasing number of HBOT sessions was associated with improved mortality outcomes, with 1, 5, 10 and 20 sessions yielding decreasing hazard ratios. There was also a significant (P = .042) positive association between increasing number of HBOT sessions and increased wound score. LIMITATIONS: Data collection was retrospective. CONCLUSION: HBOT may have a role in the treatment of calciphylaxis with benefits demonstrated in both mortality and wound healing. Larger prospective studies are needed to identify which patients would most benefit from this intervention.


Subject(s)
Calciphylaxis , Hyperbaric Oxygenation , Humans , Retrospective Studies , Calciphylaxis/therapy , Calciphylaxis/drug therapy , Thiosulfates/therapeutic use
3.
J Am Acad Dermatol ; 2024 Jul 05.
Article in English | MEDLINE | ID: mdl-38972479

ABSTRACT

BACKGROUND: Calciphylaxis patients historically have experienced diagnostic challenges and high morbidity; however limited data is available examining these characteristics over time. OBJECTIVE: The primary goals were to a) investigate factors associated with diagnostic delay of calciphylaxis and b) assess morbidity outcomes. The secondary goal was to provide updated mortality rates. METHODS: A retrospective review of 302 adult patients diagnosed with calciphylaxis between January 1, 2006 and December 31, 2022 was conducted. Univariate and multivariate statistical analyses were performed. RESULTS: Nonnephrogenic calciphylaxis (P = .0004) and involvement of the fingers (P = .0001) were significantly associated with an increased diagnostic delay, whereas involvement of the arms (P = .01) and genitalia (P = .022) resulted in fewer days to diagnosis. Almost all patients with genitalia, finger, or toe involvement had nephrogenic disease. The number of complications per patient decreased with time, especially for wound infections (P = .028), increase in lesion number (P = .012), and recurrent hospitalizations (P = .020). Updated 1-year mortality rates were 36.70% and 30.77% for nephrogenic and nonnephrogenic calciphylaxis, respectively. LIMITATIONS: Limitations include the retrospective nature and data from a single institution. CONCLUSION: Diagnostic delay, particularly in nonnephrogenic calciphylaxis, and complications per patient decreased with time, highlighting the importance of continued awareness to expedite diagnosis. Mortality rates have continued to improve in recent years.

4.
Ren Fail ; 46(1): 2297566, 2024 Dec.
Article in English | MEDLINE | ID: mdl-38178572

ABSTRACT

Calciphylaxis (CP) is a serious, potentially life-threatening disease that presents with medial calcification of small-sized vessels and painful ischemic ulcerations. Although calciphylaxis is frequently seen in patients with end-stage kidney disease on dialysis (calcific uremic arteriolopathy, CUA), there are reported cases of nonuremic calciphylaxis (NUC), which often remain undiagnosed. We conducted a retrospective chart review at our dermatological hospital and evaluated data concerning the epidemiology, comorbidities, medication, laboratory abnormalities, and therapeutic approaches of 60 patients diagnosed with calciphylaxis between 01/2012 and 12/2022. We identified 21 patients diagnosed with NUC and 39 with kidney disease. The predilection sites of skin lesions were the lower legs in 88% (n = 53), followed by the thigh and gluteal regions in 7% (n = 4). Significant differences were identified in comorbidities, such as atrial fibrillation (p < 0.001) and hyperparathyroidism (p < 0.01) accounting for CUA patients. Medication with vitamin K antagonists (p < 0.001), phosphate binders (p < 0.001), and loop diuretics (p < 0.01) was found to be associated with the onset of calciphylaxis. Hyperphosphatemia (p < 0.001), increased parathyroid hormone (p < 0.01) and triglyceride levels (p < 0.01), hypoalbuminemia (p < 0.01) and decreased hemoglobin values (p < 0.001) in the CUA cohort were significantly different from those in the NUC group. All patients with CUA received systemic medication. In contrast, only 38% (n = 8) of patients with NUC received systemic treatment. Striking discrepancies in the treatment of both cohorts were detected. In particular, NUC remains a disease pattern that is still poorly understood and differs from CUA in several important parameters.


Subject(s)
Calciphylaxis , Kidney Failure, Chronic , Humans , Calciphylaxis/diagnosis , Calciphylaxis/epidemiology , Calciphylaxis/etiology , Retrospective Studies , Kidney Failure, Chronic/complications , Kidney Failure, Chronic/therapy , Renal Dialysis , Anticoagulants/therapeutic use
5.
J Dtsch Dermatol Ges ; 2024 Aug 09.
Article in English | MEDLINE | ID: mdl-39118582

ABSTRACT

Calcinosis cutis is defined as the deposition of calcium salts in the skin. The dystrophic form is the most common and usually occurs in chronic inflammatory processes associated with collagenoses. Therapeutic options include surgical excision as well as a few pharmacological treatments. Overall, the evidence for the known therapeutic interventions is very limited and there is a lack of valid recommendations. Intravenous sodium thiosulfate has been used successfully in the treatment of calciphylaxis. In our case series, five patients with dystrophic calcinosis cutis received intravenous sodium thiosulfate for at least six cycles on five consecutive days per month, with single doses of 12.5 g and 25.0 g, respectively. A reduction in the calcified lesions could not be proven with certainty, but stable disease conditions were achieved. Intravenous sodium thiosulfate may counteract the progression of calcinosis cutis. The successful use of epicutaneously applied sodium thiosulfate, as described in the literature, suggests that a higher cutaneous bioavailability can be achieved to exert a lytic effect on calcinosis cutis. This is further supported by the reported efficacy of high-dose sodium thiosulfate in the treatment of calciphylaxis.

6.
J Dtsch Dermatol Ges ; 22(4): 553-567, 2024 Apr.
Article in English | MEDLINE | ID: mdl-38379266

ABSTRACT

The term occluding vasculopathies covers a large number of different conditions. These often manifest as skin ulcers. Occluding vasculopathies should be considered in the differential diagnosis of leg ulcers. The term "occlusive vasculopathies" encompasses pathophysiologically related entities that share structural or thrombotic obliteration of small cutaneous vessels. In this article, we will focus on livedoid vasculopathy with and without antiphospholipid syndrome and calciphylaxis with differentiation from hypertonic leg ulcer as the most relevant differential diagnoses of leg ulcer. The term also includes vascular occlusion, for example due to oxalate or cholesterol embolism, and septic vasculopathy. This often leads to acral ulceration and is therefore not a differential diagnosis with classic leg ulcers. It will not be discussed in this article. Occlusive vasculopathy may be suspected in the presence of the typical livedo racemosa or (non-inflammatory) retiform purpura as a sign of reduced cutaneous perfusion in the wound area. Inflammatory dermatoses, especially vasculitides, must be differentiated. This is achieved by histopathological evaluation of a tissue sample of sufficient size and depth taken at the appropriate time. In addition, specific laboratory parameters, particularly coagulation parameters, can support the diagnosis.


Subject(s)
Leg Ulcer , Livedo Reticularis , Purpura , Humans , Ulcer , Skin , Livedo Reticularis/diagnosis , Leg Ulcer/diagnosis , Leg Ulcer/etiology , Diagnosis, Differential
7.
Am J Kidney Dis ; 81(2): 232-239, 2023 Feb.
Article in English | MEDLINE | ID: mdl-35970430

ABSTRACT

Calciphylaxis is a life-threatening complication most often associated with chronic kidney disease that occurs as a result of the deposition of calcium in dermal and adipose microvasculature. However, this condition may also be seen in patients with acute kidney injury. The high morbidity and mortality rates associated with calciphylaxis highlight the importance to correctly diagnose and treat this condition. However, calciphylaxis remains a diagnosis that may be clinically challenging to make. Here, we review the literature on uremic calciphylaxis with a focus on its pathophysiology, clinical presentation, advances in diagnostic tools, and treatment strategies. We also discuss the unique histopathological features of calciphylaxis and contrast it with those of other forms of general vessel calcification. This review emphasizes the need for multidisciplinary collaboration including nephrology, dermatology, and palliative care to ultimately provide the best possible care to patients with calciphylaxis.


Subject(s)
Calciphylaxis , Kidney Failure, Chronic , Renal Insufficiency, Chronic , Vascular Calcification , Humans , Calciphylaxis/diagnosis , Calciphylaxis/etiology , Calciphylaxis/therapy , Vascular Calcification/etiology , Renal Insufficiency, Chronic/complications , Calcium , Obesity/complications , Kidney Failure, Chronic/therapy
8.
World J Urol ; 41(11): 2959-2966, 2023 Nov.
Article in English | MEDLINE | ID: mdl-37782324

ABSTRACT

PURPOSE: To perform a systematic review of case reports and case series to investigate risk factors, treatment modalities, and the outcome of penile calciphylaxis. METHOD: We performed a systematic search of the MEDLINE and Scopus databases to identify case reports or case series of penile calciphylaxis. The patient characteristics, laboratory investigations, diagnostic modalities, treatment modalities, and outcomes were extracted. We compared clinical characteristics and treatment between patients who survived or demised and between patients with clinical improvement and those without to identify the poor prognostic risk factors. RESULTS: Ninety-four articles were included from 86 case reports and 8 case series with 121 patients. Most of the patients were on hemodialysis (78.9%). The median time since starting dialysis was 48 months (24-96 months). Sodium thiosulfate was used to treat penile calciphylaxis in 23.6%. For surgical management, partial or total penectomy was performed in 45.5% of the patients. There was no association between sodium thiosulfate use, partial or total penectomy, and improvement in clinical outcomes. The mortality rate in patients with penile calciphylaxis was 47.8% and the median time to death was 3 months (0.75-9 months). The presence of extragenital involvement was significantly related to mortality (p = 0.03). CONCLUSION: A calcified penile artery results in penile calciphylaxis, a rare vascular phenomenon associated with high morbidity and mortality. Management of penile calciphylaxis includes the medical management of risk factors, surgical debridement, or penectomy. Therefore, early prevention and diagnosis as well as immediate appropriate treatment are needed.


Subject(s)
Calciphylaxis , Kidney Failure, Chronic , Humans , Male , Calciphylaxis/diagnosis , Calciphylaxis/therapy , Calciphylaxis/complications , Penis , Risk Factors , Thiosulfates/therapeutic use , Case Reports as Topic
9.
Nephrology (Carlton) ; 28(6): 305-314, 2023 Jun.
Article in English | MEDLINE | ID: mdl-36883928

ABSTRACT

AIM: Calciphylaxis is a rare disease, predominantly in chronic kidney disease (CKD), characterized by high morbidity and mortality. Data from the Chinese population have been an invaluable resource for a better understanding of natural history, optimal treatments and outcomes of calciphylaxis. METHODS: A retrospective study was conducted in 51 Chinese patients diagnosed with calciphylaxis at Zhong Da Hospital affiliated to Southeast University from December 2015 to September 2020. RESULTS: Between 2015 and 2020, 51 cases of calciphylaxis were registered in The China Calciphylaxis Registry (http://www.calciphylaxis.com.cn), which was developed by Zhong Da Hospital. The mean age of the cohort was 52.02 ± 14.09 years, and 37.3% were female. Forty-three patients (84.3%) were on haemodialysis, with a median dialysis vintage of 88 months. Eighteen patients (35.3%) had a resolution of calciphylaxis and 20 patients (39.2%) died. Patients in later stages had higher overall mortality than those in earlier stages. Delay from skin lesions onset to diagnosis and calciphylaxis-related infections were risk factors in both early and overall mortality. Additionally, dialysis vintage and infections were significant risk factors in calciphylaxis-specific mortality. Among therapeutic strategies, only the use of sodium thiosulfate (STS) ≥3 courses (14 injections) was significantly associated with decreased hazard of death in both early and overall mortality. CONCLUSION: For Chinese patients with calciphylaxis, delay from skin lesions onset to diagnosis and infections secondary to wounds are risk factors for the prognosis of patients with calciphylaxis. Additionally, patients in earlier stages have better survival and early continuous use of STS is highly suggested.


Subject(s)
Calciphylaxis , Kidney Failure, Chronic , Renal Insufficiency, Chronic , Adult , Aged , Female , Humans , Male , Middle Aged , Calciphylaxis/diagnosis , Calciphylaxis/etiology , Calciphylaxis/therapy , East Asian People , Kidney Failure, Chronic/complications , Kidney Failure, Chronic/diagnosis , Kidney Failure, Chronic/therapy , Renal Dialysis/adverse effects , Renal Insufficiency, Chronic/complications , Renal Insufficiency, Chronic/diagnosis , Renal Insufficiency, Chronic/therapy , Retrospective Studies
10.
Ren Fail ; 45(2): 2264407, 2023.
Article in English | MEDLINE | ID: mdl-37795796

ABSTRACT

Calciphylaxis, a rapidly progressive and potentially life-threatening vascular calcification syndrome that clinically presents with persistently painful, ulcerative, or necrotizing skin lesions in multiple parts of the body, is predominantly observed in patients treated with dialysis. Early diagnosis of calciphylaxis is a key measure for reducing high disability and mortality. At present, there is no unified diagnostic standard for calciphylaxis, and there is a lack of effective early screening strategies. This paper summarized and discussed the diagnostic accuracy of calciphylaxis based on the latest research worldwide. We propose a modified strategy for the early diagnosis of calciphylaxis, which is suitable for dialysis patients to help clinicians better identify such disease and improve prognosis.


Subject(s)
Calciphylaxis , Kidney Failure, Chronic , Vascular Calcification , Humans , Renal Dialysis/adverse effects , Calciphylaxis/diagnosis , Calciphylaxis/etiology , Calciphylaxis/therapy , Kidney Failure, Chronic/complications , Kidney Failure, Chronic/therapy , Vascular Calcification/diagnosis , Vascular Calcification/etiology , Pain/etiology
11.
Ren Fail ; 45(2): 2264401, 2023.
Article in English | MEDLINE | ID: mdl-37799073

ABSTRACT

Calciphylaxis is a rare and life-threatening condition in patients with end-stage kidney disease (ESKD). In this case report, we reported a 72-year-old female who had undergone aortic and mitral mechanical valve replacement 22 years ago due to rheumatic aortic and mitral stenosis. Following the valve replacement, she initiated warfarin treatment. Five years ago, she received a diagnosis of uremia and has since been undergoing regular hemodialysis. Ten months prior to her current admission, she experienced excruciating pain and was diagnosed with calciphylaxis. Additionally, an electrocardiogram revealed atrial fibrillation, while echocardiography indicated that the aortic and mitral mechanical valves were appropriately positioned, with normal perivalvular surroundings and good valve leaflet activity. No noticeable thrombosis was observed in the left atrium or left atrial appendage. Color Doppler imaging showed moderate stenosis in the lower extremity arteries, with no venous thromboembolism present. Extensive eggshell-like calcification within the arterial media was detected. The patient was managed with regular hemodialysis, symptomatic treatments (including anticoagulation and analgesia), and sodium thiosulfate. Unfortunately, symptomatic management provided limited relief, and during the one-month follow-up period, the patient passed away due to septic shock. Currently, there is insufficient conclusive evidence regarding alternative influential anticoagulants or appropriate prosthetic valve selection. For individuals with ESKD receiving maintenance hemodialysis, early identification, diagnosis, and treatment of calciphylaxis are of paramount importance.


Subject(s)
Atrial Fibrillation , Calciphylaxis , Kidney Failure, Chronic , Aged , Female , Humans , Anticoagulants , Calciphylaxis/diagnosis , Calciphylaxis/etiology , Heart Valves , Kidney Failure, Chronic/complications , Kidney Failure, Chronic/therapy
12.
Ren Fail ; 45(1): 2218483, 2023 Dec.
Article in English | MEDLINE | ID: mdl-37293809

ABSTRACT

Calciphylaxis is a rare cutaneous vascular disease that manifests with intolerable pains, non-healing skin wounds, histologically characterized by calcification, fibrointimal hyperplasia, and microvessel thrombosis. Currently, there are no standardized guidelines for this disease. Recent studies have recognized a high prevalence of thrombophilias and hypercoagulable conditions in calciphylaxis patients. Here, we report a case of uremic calciphylaxis patient whom was refractory to conventional treatments and then received a salvage strategy with intravenous and local hAMSC application. In order to investigate the therapeutic mechanism of hAMSCs from the novel perspective of hypercoagulability, coagulation-related indicators, wound status, quality of life and skin biopsy were followed up. Polymerase chain reaction (PCR) was performed to determine the distribution of hAMSCs in multiple tissues including lung, kidney and muscle after infusion of hAMSCs for 24 h, 1 week and 1 month in mice aiming to investigate whether hAMSCs retain locally active roles after intravenous administration. Improvement of hypercoagulable condition involving correction of platelet, D-dimer and plasminogen levels, skin regeneration and pain alleviation were revealed after hAMSC administration over one-year period. Skin biopsy pathology suggested regenerative tissues after 1 month hAMSC application and full epidermal regeneration after 20 months hAMSC treatment. PCR analysis indicated that hAMSCs were homing in lung, kidney and muscle tissues of mice even until tail vein injection of hAMSCs for 1 month. We propose that hypercoagulability is a promising therapeutic target of calciphylaxis patients, which can be effectively improved by hAMSC treatment.


Subject(s)
Calciphylaxis , Mesenchymal Stem Cells , Thrombophilia , Humans , Mice , Animals , Amnion , Calciphylaxis/etiology , Calciphylaxis/therapy , Quality of Life , Thrombophilia/etiology
13.
Ren Fail ; 45(2): 2256413, 2023.
Article in English | MEDLINE | ID: mdl-37724534

ABSTRACT

A multimodality approach has been proposed as an effective treatment for calciphylaxis in patients with end-stage kidney disease. In this retrospective study, we report the cases of 12 end-stage kidney disease patients from l'Hôtel-Dieu de Québec hospital (Canada) who were diagnosed with calciphylaxis between 2004 and 2012 and treated with a multimodality clinical approach including sodium thiosulfate (STS). Statistical analyses were performed to evaluate the impacts of patients characteristics, the different interventions as well as therapy regimen on the therapeutic response. The majority of patients (n = 9) were hemodialyzed. The patients-associated comorbidities were consistent with previously reported risk factors for calciphylaxis: Diabetes (n = 11), calcium-based phosphate binders use (n = 10), warfarin use (n = 9), obesity (n = 7), female gender (n = 8) and intravenous iron use (n = 8). STS was given for a median duration of 81 days. 75% of the patients had a response (total or partial) including a complete response in 42% of patients. One-year mortality rate was low (25%). STS was used during a mean duration of 83.33 ± 41.52 days and with a total cumulating dose of 1129.00 ± 490.58 g. The recorded mean time before a complete response was 102.20 days (51-143). Pain improvement occurred after a mean time of 8.67 ± 10.06 days. None of the studied factors was statistically associated with a complete or a partial response to the multimodality approach. Although our data have a limited statistical power, they support treating calciphylaxis with a multimodality approach including STS as its effects are independent from important clinical variables.


Subject(s)
Calciphylaxis , Kidney Failure, Chronic , Humans , Female , Calciphylaxis/etiology , Calciphylaxis/therapy , Retrospective Studies , Kidney Failure, Chronic/complications , Kidney Failure, Chronic/therapy , Calcium
14.
J Wound Care ; 32(Sup9): S22-S36, 2023 Sep 01.
Article in English | MEDLINE | ID: mdl-37682800

ABSTRACT

Constant, unrelieved pressure of local tissue, particularly over bony prominences, may provoke damage that progresses to necrosis and pressure injury (PI). Differentiating PIs from conditions of similar appearance is imperative to minimising complications and implementing prompt treatment. This case series describes several conditions that may be mistaken for a PI. Outlined are the key differences in patient history, presentation and clinical cues that assist in correctly identifying the true pathology behind these conditions. Conditions reviewed included: pyoderma gangrenosum; necrotising fasciitis; genital herpes; Marjolin ulcer, Rosai-Dorfman disease; vascular disease; coagulopathies; calciphylaxis; trauma and surgical wounds; pilonidal cysts; graft-versus-host disease; hidradenitis suppurativa; Stevens-Johnson syndrome; epidermolysis bullosa; radiation wounds; spider bites; and end-of-life skin failure pressure ulcers (also known as Kennedy ulcers). Although commonly recognised and diagnosed, stage 2, 3 and 4 PIs occasionally prove to be difficult to pinpoint, with undefined characteristics and similarities in presentation to several other conditions. Therefore, it is clinically vital to be aware of their appearance, risk factors and aetiology in order to make an appropriate patient assessment and avoid misdiagnosis.


Subject(s)
Pressure Ulcer , Humans , Diagnosis, Differential , Pressure Ulcer/diagnosis , Research , Pelvis , Lower Extremity
15.
Int Wound J ; 20(4): 1262-1275, 2023 Apr.
Article in English | MEDLINE | ID: mdl-36068658

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.


Subject(s)
Calciphylaxis , Kidney Failure, Chronic , Humans , Female , Middle Aged , Male , Calciphylaxis/diagnosis , Calciphylaxis/therapy , Kidney Failure, Chronic/therapy , Kidney/physiology , Kidney/pathology , Renal Dialysis/adverse effects , Skin/pathology
16.
Int Wound J ; 20(4): 1316-1327, 2023 Apr.
Article in English | MEDLINE | ID: mdl-36274216

ABSTRACT

Calciphylaxis, also known as calcific uremic arteriopathy, is a rare calcification syndrome that presents as ischemic skin necrosis and severe pain. It has a high mortality rate and is characterised by calcification of the small and medium arteries and micro-thrombosis. Calciphylaxis mainly occurs in patients with end-stage renal disease. In recent years, there have been an increasing number of cases of calciphylaxis associated with connective tissue diseases. Given the absence of clear diagnostic criteria for calciphylaxis thus far, an early diagnosis is crucial for designing an effective multidisciplinary treatment plan. In this article, we review the research progress on calciphylaxis and describe its characteristics in the context of connective tissue diseases.


Subject(s)
Calcinosis , Calciphylaxis , Connective Tissue Diseases , Kidney Failure, Chronic , Humans , Calciphylaxis/complications , Calciphylaxis/diagnosis , Calciphylaxis/therapy , Kidney Failure, Chronic/complications , Kidney Failure, Chronic/therapy , Skin , Connective Tissue Diseases/complications
17.
Int Wound J ; 20(9): 3717-3723, 2023 Nov.
Article in English | MEDLINE | ID: mdl-37309083

ABSTRACT

This study focuses on the application of nurse-led multidisciplinary collaborative therapy (MDT) management model for calciphylaxis prevention of patients with terminal renal disease. Through the establishment of a multidisciplinary management team spanning nephrology department, blood purification center, dermatology department, burn and plastic surgery department, infection department, stem cell platform, nutrition department, pain department, cardiology department, hydrotherapy group, dermatology group, and outpatient treatment room, the distribution of duties among team members were clarified to bring out the best advantages of a multidisciplinary teamwork during treatment and nursing. For patients with calciphylaxis symptoms in terminal renal disease, a case-by-case management model was carried out with the focus on personalised problem. We emphasised on personalised wound care, precise medication care, active pain management, psychological intervention and palliative care, the amelioration of calcium and phosphorus metabolism disorder, nutritional supplementation, and the therapeutic intervention based on human amniotic mesenchymal stem cell regeneration. The MDT model effectively compensates for traditional nursing mode and could serve as a novel clinical management modality for calciphylaxis prevention in patients with terminal renal disease.


Subject(s)
Calciphylaxis , Kidney Failure, Chronic , Humans , Calciphylaxis/etiology , Calciphylaxis/therapy , Calciphylaxis/diagnosis , Kidney Failure, Chronic/complications , Kidney Failure, Chronic/therapy , Renal Dialysis , Pain Management , Pain
18.
Medicina (Kaunas) ; 59(7)2023 Jul 14.
Article in English | MEDLINE | ID: mdl-37512116

ABSTRACT

Limited data are available on the utilization of sodium thiosulfate (STS) treatment for calciphylaxis in peritoneal dialysis (PD) patients, while it is well-studied in hemodialysis (HD) patients. A systematic literature search was conducted using Ovid MEDLINE, EBM Reviews-Cochrane Central Register of Controlled Trials, and EBM Reviews-Cochrane Database of Systematic Reviews to identify reported cases of PD patients with calciphylaxis who received STS. The search covered the inception of the databases through August 2022. Across 19 articles, this review identified 30 PD patients with calciphylaxis who received STS. These included 15 case reports, 2 case series, and 2 cohort studies. The administration routes and doses varied depending on the study. For intravenous (IV) administration (n = 18), STS doses ranged from 3.2 g twice daily to 25 g three times weekly for 5 weeks to 8 months. Outcomes included 44% of patients experiencing successful wound healing, 6% discontinuing STS due to adverse effects, 67% transitioning to HD, and 50% dying from calciphylaxis complications. For intraperitoneal (IP) administration (n = 5), STS doses ranged from 12.5 to 25 g three to four times weekly for 12 h to 3 months. Results showed 80% of patients achieving successful wound healing, 80% discontinuing STS due to adverse effects, 40% transitioning to HD, and 20% dying from IP STS-related chemical peritonitis. In cases where patients switched from IV to IP STS (n = 3), doses ranged from 12.5 to 25 g two to three times weekly for 2.5 to 5 months. Among them, 67% experienced successful wound healing, while 33% died from sepsis. Two cases utilized oral STS at a dose of 1500 mg twice daily for 6 and 11 months, resulting in successful wound healing without adverse effects or need for HD. However, one patient (50%) died due to small bowel obstruction. This systematic review provides an overview of STS treatment for PD patients with calciphylaxis. Although successful treatment cases exist, adverse effects were significant. Further research, including larger clinical studies and pharmacokinetic data, is necessary to establish the optimal route, dose, and efficacy of STS in PD patients.


Subject(s)
Calciphylaxis , Peritoneal Dialysis , Humans , Calciphylaxis/drug therapy , Calciphylaxis/etiology , Peritoneal Dialysis/adverse effects , Renal Dialysis/adverse effects
19.
Osteoporos Int ; 33(2): 499-504, 2022 Feb.
Article in English | MEDLINE | ID: mdl-34494145

ABSTRACT

Calciphylaxis is a rare and potentially fatal small-vessel occlusive disease in which the tunica media becomes calcified, endothelial cells proliferate, and the tunica intima becomes thickened and fibrotic. Calciphylaxis typically occurs in the setting of end-stage renal disease with secondary hyperparathyroidism and elevated calcium-phosphorus product. The estimated incidence of calciphylaxis in dialysis or kidney transplant patients is 1 to 4%; however, the incidence of non-uremic calciphylaxis is unknown. We assessed postmarketing adverse event reports to further characterize cases of calciphylaxis associated with teriparatide. We searched for cases of teriparatide-associated calciphylaxis in the literature (EMBASE, PubMed) and those reported to FDA, including the FDA Adverse Event Reporting System, through March 31, 2021. We included calciphylaxis cases following teriparatide exposure of < 2 years. Twelve cases described teriparatide-associated calciphylaxis. The median age was 81 (range 47-86) years. Eleven cases reported confirmatory biopsy and/or imaging. The median time-to-onset of calciphylaxis following teriparatide initiation was 3.5 (range 1-20) months. Three cases reported hospitalization, of which one resulted in death due to progression of the lesions. All cases had multiple risk factors (mean (SD), 4.5 (1.0)) including concomitant medications associated with calciphylaxis (12), female sex (11), and/or underlying autoimmune disease or other inflammatory disorder (10). We believe that exposure to teriparatide, coupled with underlying risk factors, may have triggered new-onset calciphylaxis. Expedited diagnosis and management by a clinician are important because calciphylaxis may be life-threatening and early intervention may improve outcomes.


Subject(s)
Calciphylaxis , Hyperparathyroidism, Secondary , Kidney Failure, Chronic , Aged , Aged, 80 and over , Calciphylaxis/chemically induced , Endothelial Cells , Female , Humans , Kidney Failure, Chronic/complications , Middle Aged , Teriparatide/adverse effects
20.
Exp Dermatol ; 31(10): 1632-1634, 2022 10.
Article in English | MEDLINE | ID: mdl-35441735

ABSTRACT

Calciphylaxis, also known as calcific uremic arteriolopathy (CUA), is typically characterized by subcutaneous tissue calcification and excruciatingly painful cutaneous lesions with high mortality. 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 stain1. Specially, the identification of subtle calcium deposits of subcutaneous can be challenging but is believed crucial for early diagnosis of calciphylaxis2. More sensitive calcification staining is in high demand. In this study, Fluo-3 AM was found to be a rapid, sensitive and reliable fluorescent probe for the detection of calcium deposits and could be a promising diagnostic tool for calciphylaxis.


Subject(s)
Calciphylaxis , Kidney Failure, Chronic , Aniline Compounds , Calciphylaxis/diagnostic imaging , Calciphylaxis/pathology , Calcium , Fluorescent Dyes , Humans , Subcutaneous Tissue/diagnostic imaging , Subcutaneous Tissue/pathology , Xanthenes
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