Materiały źródłowe

• #1 Calciphylaxis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK519020/

  Primary prevention is of utmost importance in regards to calciphylaxis. Patients should be counseled on compliance with hemodialysis, dietary restrictions, and the use of phosphate binders to prevent the development of this condition. […] Given the possible role of bone-mineral disorders in the pathophysiology of calciphylaxis, careful attention should be paid to avoid hypercalcemia, with phosphorus levels maintained at less than 5.5 mg/dL. At a minimum, dialysis adequacy criteria should be met, and dialysis frequency can be increased if necessary to optimize the clearance of uremic molecules and improve bone-mineral markers. […] In addition to addressing bone mineral parameters, the patient should discontinue any potentially problematic medications if possible. Given the association between warfarin and calciphylaxis, patient education regarding the risks and benefits of stopping anticoagulation or switching to a direct-acting oral anticoagulant is important.

• #1 Calciphylaxis Diagnosis and Management in Primary Care

  https://www.clinicaladvisor.com/features/calciphylaxis-diagnosis-treatment-primary-care/

  Treatment of calciphylaxis requires pain management, wound care, sepsis prevention, and timely referral to specialty care. No evidence-based clinical guidelines on prevention and treatment of the disease are available, and no medications are FDA-approved for calciphylaxis. […] Wound care to decrease sepsis should be taught to patients and caregivers. […] The role of the parathyroid is to increase serum calcium. […] Sodium thiosulfate is an antioxidant used to treat metal poisoning. […] Cinacalcet counteracts the calcium dysfunction associated with calciphylaxis.

• #1 FF #325 Uremic Calciphylaxis | Palliative Care Network of Wisconsin

  https://www.mypcnow.org/fast-fact/uremic-calciphylaxis/

  Calciphylaxis is a poorly understood disorder in which calcification of small blood vessels causes painful ischemic skin and visceral lesions most often in patients with end-stage renal disease (ESRD). […] Risk factors include: female sex; Caucasian race; obesity; diabetes mellitus; hyperparathyroidism; albumin < 3; hypercoagulable states; and exposure to certain medications such as warfarin, iron, vitamin D, and corticosteroids. [...] In general, most experts recommend a multi-modal approach involving adequate wound care, pain control, and treatment of hyperparathyroidism. This includes a low phosphate diet, use of non-calcium based phosphate binders (i.e., sevelamer), and cessation of vitamin D supplementation. [...] Considering the one-year mortality risk, the diagnosis of calciphylaxis should prompt clinicians to engage patients and families in a larger discussion regarding advance directives, prognosis, and goals of care.

• #1 Calciphylaxis: Causes, Symptoms, and Management — DermNet

  https://dermnetnz.org/topics/calciphylaxis

  It is not known how to prevent calciphylaxis. However, patients with chronic renal failure to be under the care of a nephrologist who can monitor and treat secondary hyperparathyroidism, an identifiable cause of calciphylaxis in some patients.

• #1 SciELO Brazil – Diagnosis and treatment of calciphylaxis in patients with chronic kidney disease Diagnosis and treatment of calciphylaxis in patients with chronic kidney disease

  https://www.scielo.br/j/jbn/a/RVkm7hnwxgvvh8NhdWK9CYy/

  1. Treatment is based on risk factor control (discontinuing the use of warfarin, iron salts, corticosteroids, controlling alterations in mineral metabolism and intensifying dialysis), effective pain control, treatment of secondary infection (Evidence). […] 6. Specific treatment of calciphylaxis should be performed with sodium thiosulfate, bisphosphonates, or hyperbaric oxygen therapy (Opinion). […] Finally, as general measures, we recommend: 1. Optimizing the dialysis dose, especially in the presence of persistent hyperphosphatemia 2. Avoiding positive calcium balance (calcium concentration in dialysate, calcium-based phosphate binders, use of vitamin D analogues). 3. Recommending parathyroidectomy in patients with secondary hyperparathyroidism and calciphylaxis. 4. Avoiding PTH levels persistently below 100 pg/mL. In this condition, the decrease in bone turnover will also decrease the buffering capacity of the skeleton, favoring the onset of hypercalcemia and/or hyperphosphatemia.

• #1 Calciphylaxis | Consultant360

  https://www.consultant360.com/content/calciphylaxis

  Calciphylaxis is a rare phenomenon with an estimated incidence of 1% to 4.5% among dialysis patients. Risk factors for calciphylaxis include obesity, female gender, Caucasian race, low albumin levels, elevated serum alkaline phosphatase, warfarin therapy, frequent drops in blood pressure during hemodialysis, hypercoagulable conditions, corticosteroid and immunosuppressant therapy, calcium-phosphate product of more than 60 mg2/dL2, and use of blood products. The current best therapy is prevention. […] Calcium-phosphate product is the most sensitive risk factor in the diagnosis of calciphylaxis, and small vessel calcification. […] Only 5% of hemodialysis patients have a BMI greater than 35 kg/m2 and calciphylaxis develops in 57% of these obese patients. […] Skin trauma, such as that caused by subcutaneous insulin injections in areas vulnerable to calciphylactic lesions, can also precipitate lesion development.

• #1 Calciphylaxis | UM Health-Sparrow

  https://www.uofmhealthsparrow.org/departments-conditions/conditions/calciphylaxis

  There isn’t a clear way to prevent calciphylaxis. But if you are on dialysis or have low kidney function due to advanced chronic kidney disease, it’s important to keep blood levels of calcium and phosphorus under control. […] Keeping blood levels of phosphorus under control often is a challenge. Your healthcare professional might have you take medicines with meals. You also may need to restrict certain foods that are high in phosphorus. It’s very important to follow your healthcare professional’s directions and go to all follow-up healthcare checkups. […] If you have calciphylaxis, your healthcare team helps you prevent ulcer infections or other complications. You may need to apply special wound dressings or clean the ulcers daily to prevent germs called bacteria from growing.

• #1 Perioperative Management of Calciphylaxis: Literature Review and Treatment Recommendations | Published in Orthopedic Reviews

  https://orthopedicreviews.openmedicalpublishing.org/article/37573-perioperative-management-of-calciphylaxis-literature-review-and-treatment-recommendations

  Calciphylaxis is a serious and rare medical condition that leads to substantial clinical manifestations including pain, creating perioperative and treatment challenges. […] Currently, no standard treatment protocol exists for calciphylaxis nor are comprehensive guidelines available for perioperative care of patients with calciphylaxis. […] Therefore, a multifactorial approach to controlling postoperative pain is recommended that includes the use of nerve blocks, renal-sparing opioids, benzodiazepines, and/or ketamine. […] Preoperative evaluation should assess for common comorbid conditions including ESRD, diabetes, obesity, and hyperparathyroidism and/or hypercalcemia. […] Pain control should be aggressively pursued using a multimodal approach. […] Therefore, systemic corticosteroid use should be avoided, and steps should be taken to minimize perioperative blood transfusions. […] For example, vitamin K antagonists such as warfarin may trigger or exacerbate calciphylaxis. […] A multifactorial approach that includes nerve blocks, renal-sparing opioids, benzodiazepines, and/or ketamine is recommended to optimize postoperative pain.

• #1 Seeking a Treatment for Calciphylaxis | National Kidney Foundation

  https://www.kidney.org/seeking-treatment-calciphylaxis

  „Calciphylaxis has been observed for over 50 years, but there is no effective treatment,” said Dr. Nigwekar. „As we map the biology, risk factors, prevention and eventually, treatment of calciphylaxis, we believe it will lead to a number of advancements in understanding the mechanisms of vascular calcification.” […] Vitamin K has been shown to have calcification-inhibitory properties. However, dialysis patients are usually vitamin K deficient due to recommended dietary restrictions aimed at restricting potassium and sodium-rich foods that are also rich sources of vitamin K. […] „Once we learn the biology, this information will inform treatments for other types of vascular calcifications in blood vessels found in the brain, heart and other parts of the body,” Dr. Nigwekar said. „While those conditions may not have the same fatality rate as calciphylaxis, by studying this rare disease we hope to apply what we learn to more common types of vascular calcification.”

• #1 Vitamin K: A Breakthrough Treatment for Calciphylaxis — WoundCare University

  https://woundcareuniversity.com/vitamin-k-a-breakthrough-treatment-for-calciphylaxis/

  Calciphylaxis, a severe condition characterized by calcification of small to medium-sized blood vessels leading to skin necrosis and potential organ failure, and an incredibly high one-year mortality rate of up to 70%, has posed significant management challenges. Recent advances, however, have illuminated a promising treatment modality: vitamin K. Specifically, studies indicate that vitamin K’s role in activating the matrix Gla protein (MGP) – an inhibitor of vascular calcification – addresses the core pathophysiology of calciphylaxis. […] The implications of this study are profound. Not only does vitamin K supplementation show promise in reducing lesion size and pain intensity, but its potential to slash mortality rates to zero sets it apart from other treatment modalities like STS. […] Since 2019, our group, based on evidence from the Mass General clinical trial referenced in this article, has been using vitamin K as a principle treatment strategy, with excellent results, in terms of wound healing, and mortality risk.

• #1 Calciphylaxis in chronic, non-dialysis-dependent renal disease | BMC Nephrology | Full Text

  https://bmcnephrol.biomedcentral.com/articles/10.1186/1471-2369-4-8

  The role of renal disease in vascular complications is discussed. Sterile-maggot debridement may constitute a therapy for the ulcerated calciphylaxis at an earlier stage, i.e. when first ulcerations appear. […] Overall, screening of renal-disease patients for Calciphylaxis cutis in the non-ulcerated stage seems to be crucial to timely address risk factors associated with the disease. […] Sterile maggot debridement therapy was more successful than surgical debridement in this patient. However, it was introduced late. In line with previous evidence, we strongly encourage its use in ulcerated Calciphylaxis cutis in combination with a broad-spectrum, Pseudomonas effective antibiotic earlier on to avoid superinfections and to facilitate the granulation process.

• #1 A Staff Education Project and Screening Tool to Identify Calciphylaxis

  https://scholarworks.waldenu.edu/dissertations/5246/

  Calciphylaxis is a deadly disease seen primarily in patients with end stage renal disease. […] The purpose of this project was to design a screening instrument and develop a staff education program that would transform calciphylaxis management by promoting early identification and treatment of the disease. […] Surveys conducted following the education were used to determine whether dialysis staff believed they had acquired the knowledge and skills necessary to identify early signs of calciphylaxis. […] This project exemplified that calciphylaxis detection is a secondary prevention nursing intervention that has potential for promoting positive social change by improving patient outcomes, reducing mortality rates in the end stage renal disease population, and providing empirical data to inform evidence-based therapies for at-risk patients.

• #1 Intralesional Sodium Thiosulfate to Treat Calciphylaxis | Actas Dermo-Sifiliográficas

  https://www.actasdermo.org/en-intralesional-sodium-thiosulfate-treat-calciphylaxis-articulo-S1578219016000706

  Calciphylaxis is characterized by the appearance of very painful skin ulcers and necrosis, mainly affecting patients with ESRD on hemodialysis, with an estimated prevalence between 1% and 4% in these patients. Mortality is high and is most commonly due to sepsis. Cases of ischemia of internal organs have been reported. […] Risk factors for the onset of calciphylaxis in patients with ESRD are summarized in Table 1. Calciphylaxis has been described in patients without uremia. These cases are associated with primary hyperparathyroidism, malignancy, alcoholic liver disease, and connective tissue diseases, and are grouped under the name calcific nonuremic arteriolopathy. […] Diagnosis is mainly clinical. Skin biopsy is the gold-standard test for the diagnosis of calciphylaxis, although systematically performing biopsies when this disease is suspected is not recommended because of the risk of infection or poor wound healing. Histopathology findings include calcification of the media of small arteries and arterioles, intimal proliferation, small-vessel thrombosis and endovascular fibrosis, necrosis of the subcutaneous cellular tissue, and panniculitis. No laboratory alterations specific to this disease have been detected. However, the presence of an elevated calcium-phosphate product increases the sensitivity for its detection.

• #1 Calciphylaxis (Calcific uremic arteriolopathy) – Dermatology Advisor

  https://www.dermatologyadvisor.com/home/decision-support-in-medicine/dermatology/calciphylaxis-calcific-uremic-arteriolopathy/

  Primary prevention is ideal. […] Factors associated with or suspected to aggravate calciphylaxis, such as renal failure, obesity, diabetes mellitus, hypertension, exogenous vitamin D or albumin, local trauma, corticosteroids, and immunosuppressants should be minimized. A low phosphate diet (43 mg/day), low calcium dialysate, and use of non-calcium-based phosphate binders (e.g., sevelamer hydrochloride 1600 mg three times daily) are essential components of primary prevention. […] There are no clear guidelines for the treatment of calciphylaxis. […] In general, a multipronged, multidisciplinary approach involving dermatology, nephrology, pain management, and wound care should be undertaken. Primary goals include normalization of calcium and phosphate levels, pain management, and wound care.

• #1

  https://www.kidney.org.uk/calciphylaxis-information

  Calciphylaxis is a serious condition that can occur in people chronic with kidney disease; it is most commonly seen in people receiving dialysis but remains very rare with approximately one case diagnosed in every 600 people, having dialysis, per year. […] If you have high levels of calcium/and or phosphate then please get in touch with your local kidney team for advice. If needed, your team should be able to refer you to a kidney dietitian who can provide you with tailored dietary guidance to help improve your blood phosphate levels. […] There is no specific treatment as yet that is shown to work. Your doctor will try and individualise your care to reduce your risk factors. This may include improving your calcium and phosphate levels or stopping medications such as warfarin. […] Speak to your kidney team. Ask them about your blood results and if you need to make any changes. Make sure you let them know how you are feeling and how your symptoms are they can then be guided by you. You will understand your symptoms, and needs, better than anyone and letting people know this will help them to manage your care.

• #1 Advanced-stage calciphylaxis: Think before you punch | Cleveland Clinic Journal of Medicine

  https://www.ccjm.org/content/83/8/562

  Management mainly involves controlling pain; avoiding local trauma; treating and preventing infection; stopping causative agents such as warfarin and corticosteroids; intensive hemodialysis with an increase in both frequency and duration; intravenous sodium thiosulphate; non-calcium-phosphorus binders and cinacalcet in patients with elevated parathyroid hormone; and hyperbaric oxygen.

• #1 Calciphylaxis (Calcific uremic arteriolopathy) – Dermatology Advisor

  https://www.dermatologyadvisor.com/home/decision-support-in-medicine/dermatology/calciphylaxis-calcific-uremic-arteriolopathy/

  Sodium thiosulfate (5-25 g IV over 10 minutes at the end of each hemodialysis treatment) may be used intravenously, and tolerated intraperitoneally. […] Bisphosphonates decrease arterial calcification and normalize calcium and phosphate levels through inhibition of osteoclasts. […] Promoting healing and preventing sepsis are the two main goals of wound care. […] Calcimimetics such as cinacalcet (30-120 mg/day may be used as a safer alternative to surgical parathyroidectomy in patients with secondary hyperparathyroidism or in patients who are not candidates for this procedure. […] Finally, parathyroidectomy with autotransplantation is a controversial treatment modality for calciphylaxis. […] In calciphylaxis refractory to medical and surgical management, hyperbaric oxygen therapy may be particularly valuable for those patients undergoing both peritoneal and hemodialysis. […] Substantial pain management with systemic analgesics, such as high-dose opioids, ketamine, and benzodiazepines, is often necessary as the lesions are exquisitely painful.

• #1 New Review Highlights Calciphylaxis in Kidney Diseases – Renal and Urology News

  https://www.renalandurologynews.com/news/new-review-highlights-calciphylaxis-in-kidney-diseases/

  Patients with calciphylaxis benefit from multidisciplinary care including nephrology, dermatology, and palliative care, according to a new review. […] The authors of a new review published in the American Journal of Kidney Diseases urge nephrologists, dermatologists, and other clinicians to be vigilant and proactive in recognizing and treating calciphylaxis. […] It is important to reduce risk factors for calciphylaxis. According to the reviewers, strategies potentially include withdrawal of warfarin (a vitamin K antagonist) and vitamin D and calcium-based phosphate binders. Attaining target ranges of calcium, phosphorus, and parathyroid hormone is key and may require replacing activated vitamin D with cinacalcet or parathyroidectomy. […] For further reference, a team of experts collaborated last year to provide calciphylaxis prevention and treatment strategies.

• #1 Advanced-stage calciphylaxis: Think before you punch | Cleveland Clinic Journal of Medicine

  https://www.ccjm.org/content/83/8/562

  Calciphylaxis, also referred to as calcific uremic arteriolopathy, is a rare and often fatal condition in patients with end-stage renal disease who are on hemodialysis (1% to 4% of dialysis patients). […] Documented risk factors include obesity; female sex; use of warfarin, corticosteroids, or vitamin D analogues; low serum albumin; hypercoagulable states; hyperparathyroidism; alcoholic liver disease; elevated calcium-phosphorus product; inflammation; connective tissue disease; and cancer. […] Conservative measures are the mainstay of care and include dietary alterations, noncalcium and nonaluminum phosphate binders, and low-calcium bath dialysis. There is mounting evidence for the use of calcimimetics and sodium thiosulfate. […] A multidisciplinary approach involving surgeons, nephrologists, dermatologists, dermato-pathologists, wound or burn care team, nutrition team, pain management team, and infectious disease team is important to improve outcomes.

• #1 Calciphylaxis Program

  https://www.massgeneral.org/medicine/nephrology/treatments-and-services/calciphylaxis

  Calciphylaxis is a rare and serious disorder characterized by reduction in blood flow to skin tissue due to a buildup of calcium in the walls of blood vessels. […] The calciphylaxis program at Massachusetts General Hospital leverages expertise from many specialists by bringing them together to offer multidisciplinary evaluations and treatments targeted to improve outcomes. […] At Mass General, we provide a comprehensive interdisciplinary care encompassing diagnosis, treatment and prevention of calciphylaxis. […] We then address the following domains: pain management, wound care including careful assessment of surgical debridement, avoidance/mitigation of risk factors for calciphylaxis, dialysis prescription adjustment (for individuals with end stage kidney disease), mineral metabolism management and consideration of off-label therapies including sodium thiosulfate and bisphosphonates. […] Your support of the program helps us learn more about the disease through to provide the best care.

• #1 Calciphylaxis Prevention and Treatment Strategies- Clinical Advisor

  https://www.clinicaladvisor.com/features/calciphylaxis-prevention-treatment-strategies/

  Calciphylaxis treatment should include a multimodal approach with a focus on supportive care, infection prevention, pain management, and wound healing. […] The focus should be to normalize calcium, dissolve calcium deposits, and general wound care. […] Reduction of risk factors associated with calciphylaxis is another component of care, such as avoidance of warfarin and systemic corticosteroid use. […] Identification and treatment of hypercoagulable states may be a method to reduce calciphylaxis incidence. […] Focusing on disease prevention is key, including treatment of renal failure, limiting risk factors, and recognizing the disease early in its onset.

• #1 Penile necrosis secondary to uremic calciphylaxis

  https://www.redalyc.org/journal/1631/163175162008/html/

  Uremic calciphylaxis should be considered in patients with end-stage CKD who develop acute pain and necrotic lesions on an extremity or on the penis. […] Prevention through appropriate treatment of bone mineral metabolism disorders is the basis for avoiding the occurrence of this irreversible condition with such a poor prognosis. […] However, when it does occur, early recognition, the application of conservative principles for managing the necrotic lesions, and optimization of the phosphate-calcium profile are the pillars of treatment.

• #1 Management of patients with calciphylaxis: current perspectives | CWCMR

  https://www.dovepress.com/management-of-patients-with-calciphylaxis-current-perspectives-peer-reviewed-fulltext-article-CWCMR

  The high mortality described for CUA patients is the impetus for an early diagnosis and treatment. […] A clear therapeutic recommendation beyond intensive and antimicrobial local wound care cannot be given according to the currently available literature. […] Individual therapeutic decisions have to be made and an evaluation by experienced wound carers has to be ensured. […] A clear diagnostic classification as CP is mandatory. […] Different treatment approaches for local and systemic therapy have been acknowledged although randomized controlled studies are still missing. […] Next to an atraumatic and antiseptic wound management, systemic approaches like improvement of calcium and phosphate levels as well as treatment of secondary hyperparathyroidisms are among the therapeutic considerations. […] The systemic application of STS or bisphosphonates is confirmed as successful in several case studies. […] Randomized controlled studies on larger cohorts are needed to clear upcoming issues about dosage, treatment duration and long-term outcomes.

• #2 Calciphylaxis of the penis and distal digits: a case report | Journal of Medical Case Reports | Full Text

  https://jmedicalcasereports.biomedcentral.com/articles/10.1186/s13256-021-03231-4

  Calciphylaxis is a rare disorder of intravascular calcification that is primarily observed in patients with longstanding end-stage renal disease (ESRD). Patients develop necrotic skin ulcers covered by black eschars that are typically found in areas of adiposity such as the abdomen and thighs; however, distal (digits, penis) and visceral (lungs, intestines) sites can also be involved. Patients often have an elevated serum calcium-phosphate product (70 mg/dL) and exhibit diffuse intravascular calcification on imaging. There is currently no consensus on the optimal treatment for calciphylaxis; management is guided by observational studies that advocate for a multimodal approach involving wound care, proper analgesia, infection prevention, and correction of serum calcium and phosphate levels. A trial of sodium thiosulfate (STS) is also recommended for all patients with calciphylaxis owing to its reported success in several case series; however, there is a paucity of large-scale clinical trials to support this. Even with appropriate management, calciphylaxis is a lethal disease: 6-month mortality rate is estimated to be 50%, with most patients dying from overwhelming infection. Involvement of the penis, as in our patient, is a particularly poor prognostic indicator, with 6-month mortality rates as high as 70%. […] This case highlights the particularly poor prognosis associated with penile calciphylaxis. It also underscores the need for more effective management of the disease, especially in patients with multiple comorbidities.

• #2 Advanced-stage calciphylaxis: Think before you punch | Cleveland Clinic Journal of Medicine

  https://www.ccjm.org/content/83/8/562

  Calciphylaxis, also referred to as calcific uremic arteriolopathy, is a rare and often fatal condition in patients with end-stage renal disease who are on hemodialysis (1% to 4% of dialysis patients). […] Documented risk factors include obesity; female sex; use of warfarin, corticosteroids, or vitamin D analogues; low serum albumin; hypercoagulable states; hyperparathyroidism; alcoholic liver disease; elevated calcium-phosphorus product; inflammation; connective tissue disease; and cancer. […] Conservative measures are the mainstay of care and include dietary alterations, noncalcium and nonaluminum phosphate binders, and low-calcium bath dialysis. There is mounting evidence for the use of calcimimetics and sodium thiosulfate. […] A multidisciplinary approach involving surgeons, nephrologists, dermatologists, dermato-pathologists, wound or burn care team, nutrition team, pain management team, and infectious disease team is important to improve outcomes.

• #2 Calciphylaxis: emerging concepts in prevention, diagnosis, and treatment. | Pathology & Laboratory Medicine

  https://pathology.weill.cornell.edu/research/publications/calciphylaxis-emerging-concepts-prevention-diagnosis-and-treatment

  Calciphylaxis is a small vessel vasculopathy involving mural calcification with intimal proliferation, fibrosis, and thrombosis. […] In individuals with renal failure, risk factors for the development of calciphylaxis include female sex, Caucasian race, obesity, and diabetes mellitus. […] Several recent reports demonstrate that prolonged hyperphosphatemia and/or elevated calcium x phosphorus products are associated with the syndrome. […] Protein malnutrition increases the likelihood of calciphylaxis, as does warfarin use and hypercoagulable states, such as protein C and/or protein S deficiency. […] Recent advances in diagnostic tools and therapeutic strategies have helped in the management of patients with calciphylaxis.

• #2 Calciphylaxis in Renal Failure | MDedge

  https://community.the-hospitalist.org/content/calciphylaxis-renal-failure

  Calciphylaxis occurs in 1 to 4% of patients with end stage renal failure. […] Several promising modalities for the treatment of this condition have been described in anecdotal reports. […] The most effective treatment for this condition remains uncertain. […] Therapy therefore, is focused on symptom control, debridement, and treatment of infection. […] As secondary hyperparathyroidism is common, attempts are often made to lower the calciumphosphate product. […] Calcium containing phosphate binders are avoided and low calcium dialysate used. […] Other potential treatments include: hyperbaric oxygen therapy, intravenous sodium thiosulfate, low-dose tissue plasminogen activator, cinacalcet, etidronate disodium, and maggots. […] Although several treatment modalities have been used to treat calcific uremic arteriolopathy or calciphylaxis, it remains unclear what is the best treatment for these patients.

• #2 Calciphylaxis | UM Health-Sparrow

  https://www.uofmhealthsparrow.org/departments-conditions/conditions/calciphylaxis

  There isn’t a clear way to prevent calciphylaxis. But if you are on dialysis or have low kidney function due to advanced chronic kidney disease, it’s important to keep blood levels of calcium and phosphorus under control. […] Keeping blood levels of phosphorus under control often is a challenge. Your healthcare professional might have you take medicines with meals. You also may need to restrict certain foods that are high in phosphorus. It’s very important to follow your healthcare professional’s directions and go to all follow-up healthcare checkups. […] If you have calciphylaxis, your healthcare team helps you prevent ulcer infections or other complications. You may need to apply special wound dressings or clean the ulcers daily to prevent germs called bacteria from growing.

• #2 Calciphylaxis Prevention and Treatment Strategies- Clinical Advisor

  https://www.clinicaladvisor.com/features/calciphylaxis-prevention-treatment-strategies/

  Calciphylaxis treatment should include a multimodal approach with a focus on supportive care, infection prevention, pain management, and wound healing. […] The focus should be to normalize calcium, dissolve calcium deposits, and general wound care. […] Reduction of risk factors associated with calciphylaxis is another component of care, such as avoidance of warfarin and systemic corticosteroid use. […] Identification and treatment of hypercoagulable states may be a method to reduce calciphylaxis incidence. […] Focusing on disease prevention is key, including treatment of renal failure, limiting risk factors, and recognizing the disease early in its onset.

• #2 New Review Highlights Calciphylaxis in Kidney Diseases – Renal and Urology News

  https://www.renalandurologynews.com/news/new-review-highlights-calciphylaxis-in-kidney-diseases/

  Patients with calciphylaxis benefit from multidisciplinary care including nephrology, dermatology, and palliative care, according to a new review. […] The authors of a new review published in the American Journal of Kidney Diseases urge nephrologists, dermatologists, and other clinicians to be vigilant and proactive in recognizing and treating calciphylaxis. […] It is important to reduce risk factors for calciphylaxis. According to the reviewers, strategies potentially include withdrawal of warfarin (a vitamin K antagonist) and vitamin D and calcium-based phosphate binders. Attaining target ranges of calcium, phosphorus, and parathyroid hormone is key and may require replacing activated vitamin D with cinacalcet or parathyroidectomy. […] For further reference, a team of experts collaborated last year to provide calciphylaxis prevention and treatment strategies.

• #2 Vitamin K Supplementation for Prevention of Vascular Calcification in Chronic Kidney Disease Patients: Are We There Yet?

  https://www.mdpi.com/2072-6643/14/5/925

  Chronic Kidney Disease (CKD) patients are at high risk of presenting with arterial calcification or stiffness, which confers increased cardiovascular mortality and morbidity. […] As CKD is a state of progressive vitamin K depletion and VC, research has focused on clinical trials aiming to investigate the possible beneficial effects of vitamin K in CKD and dialysis patients. […] In this study, we aim to review the current evidence regarding vitamin K supplementation in uremic patients. […] The importance of this study was that it showed for the first time that supplementation with vitamin K might prevent or even reverse VC. […] The increased prevalence of vitamin K depletion in ESKD patients can be due to any of several different reasons. […] Because CKD patients suffer from accelerated calcification, several researchers have aimed to investigate the possible association between poor vitamin K status as assessed by dp-ucMGP and VC.

• #2 Vitamin K Supplementation for Prevention of Vascular Calcification in Chronic Kidney Disease Patients: Are We There Yet?

  https://www.mdpi.com/2072-6643/14/5/925

  Therefore, scientific research is focused on the pathophysiology of calcification inhibitors in CKD, especially their activation pathways. […] A close link between vitamin K deficiency and calciphylaxis has been suggested as well. […] In order to assess a possible association between vitamin K status and CV outcomes, Chen et al. performed a meta-analysis of 21 studies and 222,592 participants and showed that increased dietary intake of either vitamin K1 or K2 was linked with a moderately reduced risk of coronary heart disease, although not with mortality. […] Therefore, the first rule when introducing a novel therapeutic agent, “First, do no harm”, is probably respected; whether the “do good” value is supported remains to be elucidated based on the results of current ongoing clinical trials.

• #2 Vitamin K: A Breakthrough Treatment for Calciphylaxis — WoundCare University

  https://woundcareuniversity.com/vitamin-k-a-breakthrough-treatment-for-calciphylaxis/

  We feel that vitamin K will supplant other methods of treatment for Calciphylaxis for several reasons. […] Mortality Reduction: The potential of vitamin K to drastically reduce mortality rates in calciphylaxis patients, as demonstrated in Dr. Nigwekar’s study, is a breakthrough in itself. […] While sodium thiosulphate served its purpose in a time when options were limited, the dawn of a more effective treatment strategy is upon us. Vitamin K, by addressing the root cause of calciphylaxis, not only promises symptom relief, but also a profound reduction in mortality risk. It is incumbent on those involved in treating this disease to become familiar with the use of vitamin K treatment for Calciphylaxis, to reduce the morbidity and mortality risk of this condition.

• #2

  https://www.kidney.org.uk/calciphylaxis-information

  Calciphylaxis is a serious condition that can occur in people chronic with kidney disease; it is most commonly seen in people receiving dialysis but remains very rare with approximately one case diagnosed in every 600 people, having dialysis, per year. […] If you have high levels of calcium/and or phosphate then please get in touch with your local kidney team for advice. If needed, your team should be able to refer you to a kidney dietitian who can provide you with tailored dietary guidance to help improve your blood phosphate levels. […] There is no specific treatment as yet that is shown to work. Your doctor will try and individualise your care to reduce your risk factors. This may include improving your calcium and phosphate levels or stopping medications such as warfarin. […] Speak to your kidney team. Ask them about your blood results and if you need to make any changes. Make sure you let them know how you are feeling and how your symptoms are they can then be guided by you. You will understand your symptoms, and needs, better than anyone and letting people know this will help them to manage your care.

• #2 SciELO Brazil – Diagnosis and treatment of calciphylaxis in patients with chronic kidney disease Diagnosis and treatment of calciphylaxis in patients with chronic kidney disease

  https://www.scielo.br/j/jbn/a/RVkm7hnwxgvvh8NhdWK9CYy/

  1. Treatment is based on risk factor control (discontinuing the use of warfarin, iron salts, corticosteroids, controlling alterations in mineral metabolism and intensifying dialysis), effective pain control, treatment of secondary infection (Evidence). […] 6. Specific treatment of calciphylaxis should be performed with sodium thiosulfate, bisphosphonates, or hyperbaric oxygen therapy (Opinion). […] Finally, as general measures, we recommend: 1. Optimizing the dialysis dose, especially in the presence of persistent hyperphosphatemia 2. Avoiding positive calcium balance (calcium concentration in dialysate, calcium-based phosphate binders, use of vitamin D analogues). 3. Recommending parathyroidectomy in patients with secondary hyperparathyroidism and calciphylaxis. 4. Avoiding PTH levels persistently below 100 pg/mL. In this condition, the decrease in bone turnover will also decrease the buffering capacity of the skeleton, favoring the onset of hypercalcemia and/or hyperphosphatemia.

• #2 Calciphylaxis Program

  https://www.massgeneral.org/medicine/nephrology/treatments-and-services/calciphylaxis

  Calciphylaxis is a rare and serious disorder characterized by reduction in blood flow to skin tissue due to a buildup of calcium in the walls of blood vessels. […] The calciphylaxis program at Massachusetts General Hospital leverages expertise from many specialists by bringing them together to offer multidisciplinary evaluations and treatments targeted to improve outcomes. […] At Mass General, we provide a comprehensive interdisciplinary care encompassing diagnosis, treatment and prevention of calciphylaxis. […] We then address the following domains: pain management, wound care including careful assessment of surgical debridement, avoidance/mitigation of risk factors for calciphylaxis, dialysis prescription adjustment (for individuals with end stage kidney disease), mineral metabolism management and consideration of off-label therapies including sodium thiosulfate and bisphosphonates. […] Your support of the program helps us learn more about the disease through to provide the best care.

• #2 Calciphylaxis

  https://calciphylaxis.mgh.harvard.edu/

  At the Mass General Hospital, we provide a comprehensive interdisciplinary care encompassing diagnosis, treatment, and prevention of calciphylaxis. […] We then address the following domains: pain management, wound care including careful assessment of surgical debridement, avoidance/mitigation of risk factors for calciphylaxis, dialysis prescription adjustment (for individuals with end stage kidney disease), mineral metabolism management, and consideration of off-label therapies including sodium thiosulfate and bisphosphonates.

• #2 A Staff Education Project and Screening Tool to Identify Calciphylaxis

  https://scholarworks.waldenu.edu/dissertations/5246/

  Calciphylaxis is a deadly disease seen primarily in patients with end stage renal disease. […] The purpose of this project was to design a screening instrument and develop a staff education program that would transform calciphylaxis management by promoting early identification and treatment of the disease. […] Surveys conducted following the education were used to determine whether dialysis staff believed they had acquired the knowledge and skills necessary to identify early signs of calciphylaxis. […] This project exemplified that calciphylaxis detection is a secondary prevention nursing intervention that has potential for promoting positive social change by improving patient outcomes, reducing mortality rates in the end stage renal disease population, and providing empirical data to inform evidence-based therapies for at-risk patients.

• #2 Calciphylaxis Diagnosis and Management in Primary Care

  https://www.renalandurologynews.com/features/calciphylaxis-diagnosis-treatment-primary-care-2/

  Treatment of calciphylaxis requires pain management, wound care, sepsis prevention, and timely referral to specialty care. No evidence-based clinical guidelines on prevention and treatment of the disease are available, and no medications are FDA-approved for calciphylaxis. […] Wound care to decrease sepsis should be taught to patients and caregivers. […] Sodium thiosulfate is considered successful when pain intensity is decreased, wound progression slows, and wound appearance is improved. […] The most successful treatment plans are those that include treatment of the underlying issue such as hypercalcemia, hyperparathyroidism, and kidney disease. […] Experimental treatments such as intravenous sodium thiosulfate, phosphate binders, and calcimimetics are being tested; these treatments have not demonstrated a mortality benefit in pooled cohort studies and further investigation in randomized controlled trials is needed. […] Parathyroid hormone suppression is a risk factor for calciphylaxis.

• #3 Calciphylaxis

  https://calciphylaxis.mgh.harvard.edu/

  At the Mass General Hospital, we provide a comprehensive interdisciplinary care encompassing diagnosis, treatment, and prevention of calciphylaxis. […] We then address the following domains: pain management, wound care including careful assessment of surgical debridement, avoidance/mitigation of risk factors for calciphylaxis, dialysis prescription adjustment (for individuals with end stage kidney disease), mineral metabolism management, and consideration of off-label therapies including sodium thiosulfate and bisphosphonates.

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