MindMap Gallery calciphylaxis
Definition: Extremely painful ischemic skin and soft tissue lesions due to microvascular calcification and thrombosis in the dermis and subcutaneous adipose tissue. Calciphylaxis is a rare, fatal disease.
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calciphylaxis
Overview
More common in patients with end-stage renal disease, also known as calcium-uremic arteriolopathy
Definition: An extremely painful ischemic skin and soft tissue lesion due to microvascular calcification and thrombosis in the dermis and subcutaneous adipose tissue.
pathology
systemic arteriomedia calcification
Calcification-associated intimal fibrosis
secondary thrombotic vascular occlusion
high risk factors
ESRD, obesity, diabetes, calcium and phosphorus metabolism disorders, hyperparathyroidism, excessive PTH suppression with adynamic bone disease (low bone turnover), vitamin K deficiency, drug use (such as warfarin, calcium supplements, vitamin D , iron supplements, recombinant PTH, etc.).....
clinical manifestations
Early stage: skin hardening, plaques, nodules, livedo reticularis or purpura
In the middle and late stages: the skin darkens and forms necrotic ulcers, accompanied by black eschar.
diagnosis
Proposed diagnosis: 2 main factors, early suspicious skin changes; 1 main factor, 3 secondary factors, early suspicious skin changes
Clinical diagnosis: 1 high-risk factor, typical skin lesions, exclusion of other diseases
Confirmed diagnosis: Positive skin biopsy High-risk factors Skin lesions Exclude other diseases
Main factors: application of warfarin, application of large-dose calcium-phosphorus binders, active vitamin D dose greater than 0.5ug/d, serum protein C or protein S deficiency, vitamin K deficiency, IPTH greater than 1000pg/ml
Secondary factors: dialysis for more than 5 years, obesity, diabetes, hypoalbuminemia, use of immunosuppressants and glucocorticoids, IPTH greater than 300pg/ml (less than 1000)
Early suspicious skin lesions: intractable itching, rough skin, hypoesthesia or hypersensitivity, diffuse nodular changes, accompanied by itching, pain and rash-like changes
Typical skin lesions: painful papules, sclerotic plaques, reticular livedo, necrotic skin ulcers, often accompanied by severe pain.
SOP for diagnosis and treatment of calciphylaxis in dialysis patients
treat
Treatment status: There is a lack of specific treatment methods, and close cooperation between multiple disciplines is needed for comprehensive treatment.
1. Reduce or remove triggers
Control normal blood calcium and blood phosphorus levels, and maintain PTH at 150-300ng/ml
Avoid warfarin, active vitamin D, and calcium supplements
In patients with severely elevated PTH levels, calcimimetics should be considered
For patients who are difficult to treat with calcimimetics, parathyroidectomy is recommended
Optimize the dialysis plan: hemofiltration plus hemoperfusion, increase the number of dialysis times, extend the dialysis time, and use low-calcium dialysate.
2. Pain Management
Medication principles
Proactive preventive medication for pain management
Analgesics should be given regularly and on time, rather than just when necessary. The next dose should be given before the effect of the previous drug wears off so that pain relief can be sustained.
Changes in patients' pain scores and quality of life were recorded objectively and regularly.
Drug type
Opioids
Tends to accumulate in patients with renal failure and can cause altered mental status. Hydromorphone, methadone, and fentanyl are recommended over morphine, tramadol, and oxycodone.
alternative analgesia
Nerve blockers such as gabapentin, ketamine, and benzodiazepines
refractory pain
Local anesthesia, lumbar sympathetic nerve block
Pain Relief Treatment (CUHK Program)
Phase 1: Tylenine 5mg: 325mg q6h po
The second stage: fentanyl transdermal patch 4.2mg q72h for external use, Tylenine 5mg: 325mg q6h po
The third stage: fentanyl transdermal patch 4.2mg qd for topical use q72h, pregabalin 75mg, additional 75mg po after dialysis, Gabapentin 0.3g qd po, additional 0.3g po after dialysis
3. Wound care
1. Prevent infection
2. Frequent dressing changes: complex wound care
3. Debridement: For infected wounds (such as wounds with obvious drainage or pus formation)
4. Non-surgical treatment: dry wounds
5. Antibiotics: No need for prophylactic use of antibiotics
6. Drugs that delay wound healing: such as mTOR inhibitors, alternative drugs should be used as much as possible
7. For subcutaneous drug treatment, such as insulin, the injection site should be rotated to minimize recurrent skin trauma.
8. Nutritional assessment: avoid malnutrition and promote wound healing
4. Medication
Sodium thiosulfate
The American Injectable Association lists it as an off-label drug for the treatment of calciphylaxis.
bisphosphonate
Sinakase
Vitamin K
Vitamin K supplementation improves calciphylaxis in patients with vitamin K deficiency
SNF472
A calcification inhibitor for the treatment of cardiovascular calcification in hemodialysis and calciphylaxis patients
5. Hyperbaric oxygen therapy
Summarize
Calciphylaxis is a rare, fatal disease.
Research on early diagnosis and treatment strategies has broad prospects
Improved imaging technology and precise treatment based on mechanism research are important directions for future calciphylaxis treatment research.
Speaker: Chief Physician Zhang Xiaoliang Producer: Xinjing Content comes from the official account of Smart Kidney Home