Calciphylaxis (Calcific Uremic Arteriolopathy): Balaguron Rigakafin Rigakafin Rigakafin Rigakafi, Baƙin Necrotic Eschars a cikin Marasa lafiya Dialysis, da Sodium Thiosulfate¶

Calciphylaxis, likita wanda aka sanya shi azaman ** calcific uremic arteriolopathy (CUA)** lokacin da ya faru a cikin marasa lafiya da cututtukan koda na ƙarshen zamani, cuta ce mai ɓarna, mai barazanar rayuwa wanda ke da alaƙa da calcification na tsakiya, fibrosis na subintimal, da ƙananan thrombotic occlusion na ƙananan dermalging da ƙwanƙwasa 6 (0) micrometers a diamita). Ba kamar na yau da kullun na atherosclerotic peripheral arterial cuta wanda ke kunkuntar manyan jijiya mai suna a cikin kafafu yayin barin bugun feda mai rauni ko ba ya nan, calciphylaxis yana buge cibiyar sadarwa ta arteriolar microscopic da ke cikin zurfi a cikin * adipose-arziki mai kitse na subcutaneous ** (ciki, panniculus, cinya, cinya da maƙarƙashiya). bugun idon idon sawu da kafa sau da yawa yakan kasance gaba daya tawul. A cikin majiyyaci a kan na kullum hemodialysis ko peritoneal dialysis, ko a cikin marasa lafiya da al'ada aikin renal shan warfarin (wanda ba uremic calciphylaxis), asarar halitta vascular calcification inhibitors, musamman ** carboxylated Matrix Gla Protein (MGP)* da hanta ** fetuin-A** da hanta ** fetuin-A**, haɗe tare da bitamin hyperphosphatemia. analogs (calcitriol, paricalcitol), calcium-based phosphate binders (calcium acetate, calcium carbonate), da kuma maimaita subcutaneous injections (insulin ko heparin) *** yana haifar da phenotypic canji na jijiyoyin jini santsi Kwayoyin zuwa kashi-forming ** osteoblast-kamar Kwayoyin ***. Da zarar an rufe lumen arteriolar da aka kayyade da wani mummunan fibrin thrombus, fatar da ke sama da kitse suna fama da mummunan rauni. A asibiti, calciphylaxis yana sanar da kansa tare da ** zafi mai zafi, ƙonewa, 10-na-10 ciwon fata na ischemic wanda akai-akai ya riga ya bayyana fata necrosis da kwanaki , da sauri yana tasowa daga ** mottled violaceous retiform (net-like) purpura da rock-hard, woody subcutaneous nodules a cikin wani nau'in fata mai launin fata, mai siffar launin fata, mai siffar launin fata. by dusky violaceous iyakoki. Saboda ulcerated calciphylaxis eschars zaune kai tsaye a kan karkatattun kitse, mamayewar kwayoyin cuta na biyu yana haifar da fulminant rauni sepsis, yana haifar da adadin mace-macen tarihi na shekara guda na kashi 50 zuwa 80. Idan majiyyaci na dialysis ko mai amfani da warfarin ya sami raɗaɗi mai raɗaɗi mai raɗaɗi mai raɗaɗi mai raɗaɗi mai kama da plaques, nodules subcutaneous nodules, ko baƙar fata necrotic ulcers, kira yankin ku. lambobin gaggawa ko kuma a ci gaba da zuwa sashin gaggawa nan take. Hakanan duba jagororin 'yan uwanmu akan samun damar yin amfani da dialysis AV fistula jini mai barazana ga rayuwa da rashin lafiyar dialysis hyperkalemia, erysipelas vs necrotizing fasciitis vs stasis dermatitis triage, kuma sepsis da septic shock ganewa.

Babban fifikonku na gaggawa na gaggawa shine ** nan da nan dakatar da duk masu haɓaka ƙididdiga**, tsayawa warfarin (canzawa zuwa maganin rigakafi na baka kai tsaye kamar apixaban ko heparin mara nauyi mai nauyi bayan nazarin ƙwararru, yayin ba da bitamin K1 na cikin jini don sake kunna Matrix Gla Protein), tsayawa ** duk abin da ke ɗauke da sinadarin calcium zuwa bindercical bindercical. sevelamer ko lanthanum)**, da tsayawa active Vitamin D analogs (canzawa zuwa calcimimetics kamar cinacalcet ko etelcalcetide idan tsananin hyperparathyroidism na biyu ya kasance). A lokaci guda, likitocin dole ne **fara Sodium Thiosulfate na ciki (yawanci gram 25 IV wanda aka ba shi a cikin minti 30 zuwa 60 na ƙarshe na kowane zaman hemodialysis sau uku a mako) lokaci *** inda pathergy da microvascular ischemia ke haifar da raunuka don fadada bala'i.

Yi wannan yanzu¶

1. Seek immediate Hospital Admission or Emergency Nephrology and Dermatology Evaluation if a patient on dialysis (or taking warfarin) develops excruciatingly painful violaceous mottled skin plaques, rock-hard subcutaneous fat nodules, or black necrotic eschars. Dial 911 in the United States and Canada, 999 in the United Kingdom, 112 across the European Union, 000 a Ostiraliya, ko tuntuɓi littafin adireshi na duniya lambobin gaggawa. Idan majiyyaci kuma yana da zazzaɓi, damuwa, hauhawar jini, ruɗani, ko magudanar ruwa mara kyau daga maƙarƙashiya eschar, ayyana ** gaggawar cutar sepsis** nan take.
2. Bincika da Tsaya "Babban Hudu" Masu Haɓaka Magunguna na Calciphylaxis Nan da nan Bayan Isar Asibiti:
3. ** TSAYA Warfarin (Masu adawa da Vitamin K):** Warfarin yana toshe gamma-carboxylation na Matrix Gla Protein (MGP), mafi girman ƙarfin jiki mai hanawa na tsakiya na tsakiya na arteriolar. Canja mai haƙuri zuwa maganin rigakafi na baka wanda ba na bitamin-K ba (irin su ** apixaban ** a cikin daidaitawar renal-daidaitacce) ko heparin na warkewa a cikin shawarwari tare da hematology da nephrology, da gudanar da ** Vitamin K1 (phytonadione, 10 mg na baka ko IV sau uku a mako)** don mayar da MGP.
4. ** TSAYA Dukkanin Abubuwan da ke Ba da Calcium (* PhosLo * [calcium acetate], * Tums * [calcium carbonate]) da Kariyar Calcium:** Canja nan da nan zuwa * abubuwan da ba su da sinadarin calcium mai ɗauke da phosphate kamar su ** sevelamer carbonate (* Renvela*) os num, (* Renvela*) renol. ** sucroferric oxyhydroxide (Velphoro)**.
5. ** TSAYA DUK Active Vitamin D Analogs (Calcitriol, Paricalcitol, Doxercalciferol) da High-Kashi Ergocalciferol/Cholecalciferol:** Vitamin D receptor agonists ƙara hanji alli alli da phosphate sha da kuma kai tsaye tsokana jijiyoyin bugun gini santsi tsoka calcification. Idan mai haƙuri yana da hyperparathyroidism mai tsanani (iPTH> 300 pg/mL), canza zuwa calcimimetic (** cinnacalcet na baka [* Sensipar*] ko etelcalcetide na jini [Parsabiv]) ko shirya gaggawar gaggawa ** parathyroidectomy.
6. ** TSAYA IRIN DEXtran / Sucrose na Jiki da Allurar Subcutaneous a cikin Fat ɗin Fat ɗin da Ya Shafi:** Ƙarfe yana haifar da rauni mai rauni na gida na hydroxyl a cikin dermis na ischemic, da maimaita ** insulin subcutaneous ko alluran heparin ** cikin panniculs na ciki ko cinya yana haifar da bugun jini na gida. Juya alluran zuwa wuraren da ba a shafa ba ko canzawa zuwa jiko yayin da ake asibiti.
7. Fara Ciki Sodium Thiosulfate (Na2S2O3) a 25 Grams IV Sau Uku mako-mako (A yayin Minti 60 na Ƙarshe na kowane Zama na Hemodialysis): Sodium Thiosulfate na Jiki (STS) shine ginshiƙin tsarin ceton tsarin ga m calciphylaxis. Yana chelates insoluble nama alli adibas a cikin ** matsananci-soluble calcium thiosulfate ** (wanda shine 250-ninka zuwa 100,000-ninka mafi soluble a cikin ruwa fiye da calcium phosphate ko calcium oxalate kuma da sauri sharewa a fadin hemodialysis membrane), yana aiki a matsayin mai ƙarfi ** free-radical antioxidant ** da OS synalthases. yana haifar da ** kai tsaye arteriolar vasodilation ** wanda sau da yawa yana rage tsananin zafin fata na ischemic a cikin makonni 1 zuwa 2 ***.
8. ** Kula da Sarrafa Matsalolin Haɗari Biyu na IV Sodium Thiosulfate: Babban Anion Gap Metabolic Acidosis da Tsananin Tashin Jiki/Hypotension:**
9. Lokacin da sodium thiosulfate ke haɓaka ta hanta zuwa ** thiosulfuric acid da sulfate anions **, yana haifar da babban ** babban gibin anion metabolism acidosis ** (sau da yawa yana zubar da bicarbonate ƙasa 14 zuwa 16 mmol/L). Bincika madaidaicin panel na rayuwa da gibin anion kafin kowane zaman dialysis da ** ƙara yawan taro na bath bicarbonate na hemodialysis ** (ko gudanar da sodium bicarbonate na baka kawai idan matsayin ƙarar ya yarda).
10. Sanya gram 25 na STS da sauri (< Minti 30) yana haifar da ** amai maras nauyi, tashin zuciya mai tsanani, da matsanancin hauhawar jini na intradialytic . Koyaushe premedicate da IV Ondansetron (4 zuwa 8 MG) ko Promethazine Minti 30 kafin jiko, kuma saka STS a hankali akan ** mintuna 60.
11. ** Haɓaka Rubutun Hemodialysis Nan da nan:** Ƙara yawan dialysis da tsawon lokaci (misali, ** 4 zuwa 5 zaman a kowane mako na 4 hours kowane ) don kawar da kwayoyin phosphate da uremic guba mai tsanani, kuma canza wanka na dialysate zuwa ** low-calcium dialysate maida hankali (1.5 zuwa 2.5 m. mmol/L) ta yadda Calcium ya kasance yana bazuwa daga daga cikin jinin mara lafiya yayin dialysis. Idan majiyyaci yana kan ** peritoneal dialysis (PD)** (wanda ke ɗauke da mafi girman abin da ya faru na calciphylaxis saboda samfuran lalatawar glucose na cikin peritoneal akai-akai da ƙarancin ƙarancin phosphate), la'akari da ƙarfi ** canjin gaggawa zuwa hemodialysis **.
12. Protect the Ulcerated Skin and Avoid Aggressive Deep Surgical Excision During Active Ischemia (Preventing Pathergic Ulcer Expansion): Unlike necrotizing fasciitis (which requires emergency wide surgical debridement in the operating room), cutting into surrounding ischemic, calcified dermis in active calciphylaxis frequently triggers pathergy and progressive ischemic edge necrosis, creating a katon ramin da ba ya warkewa. A kiyaye busassun busassun busassun busassun busassun busassun busassun busassun busassun busassun busassun busassun busassun busassun busassun busassun busassun busassun busassun busassun busassun busassun busassun busassun busassun busassun busassun busassun busassun busassun busassun busassun busassun busassun busassun busassun busassun busassun busassun busassun busassun busassun busassun s) baƙar fata, sai dai in rigar purulent kamuwa da cuta ta tasowa a ƙasa; Lokacin da ake buƙatar kau da ƙwayar necrotic slough don hana sepsis, yi amfani da ** m enzymatic debridement (collagenase), hydrogel autolytic dressings, maggot debridement far, ko a hankali ra'ayin mazan jiya cire sako-sako da devitalized nama hade tare da Hyperbaric Oxygen Therapy (HBOT)** da zarar ana jiyya calcification arteriolar.
13. ** Samar da M Multimodal Neuropathic da Ischemic Pain Control (Tsarin Gujewa Morphine da Meperidine a cikin Marasa lafiya):** Ciwon Calciphylaxis yana cikin mafi yawan radadin ciwo mai zafi a cikin magani, yana haɗuwa da raunin ƙwayar tsoka mai zurfi na ischemic tare da ƙananan fiber neuropathic rauni. Kada a taɓa amfani da morphine, codeine, ko meperidine a cikin gazawar koda (masu guba masu guba morphine-6-glucuronide da normeperidine suna taruwa zuwa matakan mutuwa waɗanda ke haifar da myoclonus, seizures, da kama numfashi). Madadin haka, yi amfani da ** intravenous ko transdermal Fentanyl, Methadone, ko Buprenorphine ** (duk lafiyayye a cikin dialysis tare da sifili mai aiki na renal metabolites) haɗe tare da daidaitawar koda Gabapentin (100 zuwa 300 mg post-dialysis) ko ** ketamine infusions*.

Bishiyar yanke shawara: wanda ake zargin Calciphylaxis (Calcific Uremic Arteriolopathy) vs Warfarin Skin Necrosis vs Necrotizing Fasciitis¶

flowchart TD
    A["Marasa lafiya na Dialysis (HD/PD) ko Mai Amfani da Warfarin yana Gabatar da Ciwon Fata mai ƙonawa na 10/10 + Rikicin Retiform (Net-like) Plaques, Woody Subcutaneous Nodules, ko Black Stellate Necrotic Eschars akan Ciki, cinyoyi, gindi, ko ƙafafu."] --> B{"Mataki na 1: Nan da nan Sepsis & Necrotizing Fasciitis Screen: Shin Akwai Crepitus, Ruwan Ruwa, Yadawa Fascial Bullae da sauri, Zazzabi mai zafi, ko Shock Septic?"}
    B -->|YES: Wet Gangrene / Fulminant Wound Sepsis or Necrotizing Fasciitis| C["Resuscitate tare da IV Crystalloids + Broad-Spectrum IV Antibiotics (Vancomycin + Cefepime/Piperacillin-Tazobactam + Metronidazole) + Maganin Gaggawa / Maganganun Rauni don Cutar Eschar Drainage"]
    B -->|NO: Dry Leathery Black Eschar or Intact Violaceous Plaque / Woody Subcutaneous Nodule| D["Mataki na 2: Ƙwayoyin Jiji da Magunguna a Gefen Bedde + Filayen Radiyon Nama mai laushi (Mammography-Technique X-Rays) ko Scintigraphy na Kashi"]
    C --> D
    D --> E{"Bincika Lokacin Farawar Warfarin (Shin Necrosis ya samo asali a cikin Rana ta 3 zuwa Rana ta 10 na Farawar Warfarin Ba tare da Gadar Heparin ba?)"}
    E -->|YES: Acute Onset Day 3 to 8 of Warfarin + Rapid Protein C Drop| F["CUTE WARFARIN-INDUCED SKIN NECROSIS (Microvascular Venular Thrombosis): Tsaya Warfarin + Ba da Vitamin K IV + Fara warkewa IV Heparin mara lahani + Fresh Plasma / Protein C Concentrate"]
    E -->|NO: Chronic Warfarin Use (> Months/Years) OR Dialysis / ESRD Patient with High Phosphate/PTH + Fine Net-Like Arteriolar Calcification on X-Ray| G["TABBATAR CALCIPHYLAXIS (Calcific Uremic Arteriolopathy - CUA)"]
    G --> H["Mataki na 1 (Dakatar da Duk Accelerators Nan da nan): 1. TSAYA Warfarin (Ba da Vitamin K1 10 mg 3x / wk; canza zuwa Apixaban idan ana buƙatar anticoagulation); 2. TSAYA Masu Haɗaɗɗen Calcium Phosphate (Canja zuwa Sevelamer/Lanthanum); 3. TSAYA Calcitriol/Vitamin D Analogs; 4. TSAYA Ƙarfe & SubQ allura a cikin Plaques"]
    H --> I["MATAKI 2 (Fara Tabbataccen Calcium-Chelating & Vasodilatory Therapy): Gudanar da IV SODIUM THIOSULFATE (Gram 25 IV Sama da Minti 60 Na Ƙarshe na Kowane Zama na Hemodialysis 3x/Week) + Premedicate tare da IV Ondansetron"]
    I --> J["Mataki na 3 (Haɓaka Dialysis & Parathyroid Axis): Ƙara Hemodialysis zuwa 4-5x/Mako tare da LOW-CALCIUM Dialysate Bath (1.5 mEq/L); Idan iPTH> 300 pg/mL, Fara Cinacalcet / Etelcalcetide ko Shirya Parathyroidectomy na gaggawa"]
    J --> K["Mataki na 4 (Safe Analgesia & Rauni): Yi amfani da Fentanyl, Methadone, ko Buprenorphine + Post-HD Gabapentin (KADA morphine/Meperidine); Gujewa Zurfin Fida na Busassun Eschars; Yi la'akari da Hyperbaric Oxygen (HBOT) & Intralesional Sodium Thiosulfate"]

Pathophysiology: yadda ƙwayoyin tsoka masu santsi suka juya zuwa kashi kuma me yasa adipose tissue shine manufa ta farko¶

Shekaru da yawa, likitoci sun ɗauka cewa calciphylaxis shine kawai hazo sinadarai na alli da phosphate lokacin da ruwan magani ** samfurin calcium-phosphate (Ca x PO4)** ya wuce 55 zuwa 70 MG a kowace murabba'in dL. Ilimin halitta na jijiyoyin jini na zamani ya tabbatar da cewa calciphylaxis shine ainihin mai aiki, canjin osteogenic na tsaka-tsakin tantanin halitta wanda ya biyo bayan bugun bugun bugun jini na biyu***:

THE TWO-HIT PATHOPHYSIOLOGY OF CALCIPHYLAXIS (CUA)
=======================================================================
[HIT 1: CHRONIC ARTERIOLAR MEDIAL CALCIFICATION & INTIMAL HYPERPLASIA]
   * Triggers in ESRD / Dialysis / Non-Uremic Warfarin Patients:
     1. Loss of Active Carboxylated Matrix Gla Protein (MGP) -> Warfarin
        or Vitamin K deficiency disables the local arteriolar shield.
     2. Loss of Systemic Fetuin-A (Negative acute-phase liver protein)
        & Inorganic Pyrophosphate (PPi) -> Fails to clear calciprotein
        particles from the bloodstream.
     3. Hyperphosphatemia + Calcium Binders + Calcitriol + High PTH ->
        Phosphate enters arteriolar Smooth Muscle Cells via Pit-1
        cotransporters, activating the bone transcription factor RUNX2
        (Cbfa1) and BMP-2 (Bone Morphogenetic Protein-2).
   * Result: Arteriolar smooth muscle cells transdifferentiate into
     OSTEOBLAST-LIKE CELLS that lay down concentric hydroxyapatite bone
     crystals in the tunica media of 40-to-600-micron subcutaneous
     arterioles, while subintimal fibrosis narrows the lumen by 80%.
                                  |
                                  v
[HIT 2: ACUTE ENDOTHELIAL THROMBOSIS & INFARCTION OF SUBCUTANEOUS FAT]
   * Local Triggers: Repetitive subcutaneous insulin/heparin injections,
     minor skin trauma (pathergy), iron overload, hypercoagulability
     (Protein C/S deficiency, lupus anticoagulant), or hypotension.
   * Result: An acute FIBRIN THROMBUS plugs the rigid, calcified
     micro-arteriole -> Complete ischemic infarction of the overlying
     adipose lobules and dermis -> Excruciating 10/10 ischemic nerve
     pain -> Violaceous retiform purpura -> Black stellate eschar.
=======================================================================

Tsakiyar (Adipose-Rich) vs. Peripheral (Acral) Calciphylaxis: Alamomin asibiti da Hasashen¶

Kwararrun likitocin sun rarraba calciphylaxis zuwa rabe-rabe daban-daban na jikin mutum wanda ke dauke da sakamakon rayuwa daban-daban: 1. Central (Proximal/ Adipose-Rich) Calciphylaxis (70% zuwa 80% of Cases): Yana bugun wuraren da ke da kaurin adipose nama mai kauri, musamman ** cinyoyin ciki, cinyoyin tsakiya da na gefe, gindi, gyale, da nonon mata**. Domin adipose lobules ana kawota ta dogon, tortuous, karshen-arteriolar tasoshin tare da matalauta conlateral wurare dabam dabam da kuma saboda balagagge adipocytes secrete pro-calcific adipokines (leptin, TNF-alpha, da IL-6) cewa hanzarta jijiyoyin bugun gini santsi tsoka tsoka RUNX2 kunnawa, tsakiyar kitsen pads ne musamman m. Calciphylaxis na tsakiya yana ɗauke da mafi yawan mace-mace (60% zuwa 80% a shekara 1) saboda manyan gyambon ciki da cinya suna saurin mamayewa ta hanyar ƙwayoyin cuta na Gram-negative da Staphylococcus aureus, wanda ke haifar da kisa. 2. ** Na gefe (Distal / Acral) Calciphylaxis (20% zuwa 30% na Cases):** Yana bugun wurare masu nisa tare da ƙaramin kitse na cikin ƙasa, gami da ** calves, shins, idon sawu, yatsu, yatsu, da lokaci-lokaci glans azzakari (penile calciphylaxis). Launuka na gefe suna ɗaukar ƙarancin mace-mace (ko da yake har yanzu yana da tsanani) adadin mace-mace na 20% zuwa 40%, kodayake dijital ko gangrene na penile yana buƙatar bambance-bambance a hankali daga manyan-jigon atherosclerotic peripheral arterial disease (PAD) da ƙwayar cholesterol crystal embolization. 3. ** Calciphylaxis marasa Uremic (NUC, 10% zuwa 15% na lokuta): Yana faruwa a cikin marasa lafiya tare da aikin koda na yau da kullun (eGFR> 60 ml / min) wanda ** amfani da warfarin na yau da kullun , hyperparathyroidism na farko, ** cututtukan autoimmune akan cututtukan cututtukan cututtukan cututtukan cututtukan cututtukan cututtukan cututtukan ƙwayoyin cuta kira na fetuin-A da Protein C/S).

Fahimtar bambancin gefe-gefe: Calciphylaxis vs Warfarin-Induced Skin Necrosis vs Necrotizing Fasciitis vs Cholesterol Embolization vs Pyoderma Gangrenosum¶

Saboda kuskuren ganewar asali na calciphylaxis a matsayin pyoderma gangrenosum (da kuma bada babban adadin corticosteroids na tsarin da ke hanzarta calcification da sepsis) ko ɓatar da shi a matsayin fasciitis necrotizing (da kuma yin lalata mai tsattsauran ra'ayi wanda ke haifar da bala'i wanda ba shi da warkarwa), zai iya amfani da wannan gado na asibiti a gefen gado.

Matsalolin Ganewa: Lokacin Gujewa Fatar Kwayoyin cuta vs. Yadda Ake Yi Safe Telescoping Biopsy¶

1. Lokacin da Ciwon Ganewar Asibiti ya Isa (Ba a Bukatar Biopsy): A cikin wani majinyacin ciwon koda na ƙarshen zamani akan dialysis wanda ke gabatar da plaques na retiform mai raɗaɗi mai raɗaɗi, nodules na subcutaneous, ko stellate baƙar fata* a cikin ciki ko cinya yana da haɗari da yawa. Yanke cikin plaque na ischemic calciphylaxis yana ɗaukar haɗarin ** 30 zuwa 50 bisa 100 na haifar da gyambon ciki, faɗaɗa rauni mara warkarwa, da sepsis mai mutuwa na biyu**.
2. Maɗaukakin Mahimmancin Hannun Hannun Hannun Hannun Hannun Hannun Hannun Hannun Hannun Sauye-sauyen Hoto:
3. ** Filayen Radiyon Nama mai laushi mai laushi ta Amfani da Babban Mahimmancin Mammography Technique:** Bayyana ** lafiya, lacy, arborizing (reshen bishiya ko net-like) ƙididdiga na ƙananan arterioles na subcutaneous (< 100 zuwa 500 micrometers)** tare da ƙayyadaddun ƙayyadaddun kaso na gabatowa.
4. ** Technetium-99m (Tc-99m MDP) Scintigraphy na Kashi-Uku:** Yana nuna sha'awar ɗaukar rediyon rediyo a cikin kumburi, yana ƙididdige kitsen mai a cikin cinya, ciki, da gindi (hankali 89% zuwa 97%).
5. Yadda Ake Yin Kwayar Halittu Lafiya Lokacin Da Ba a Tabbaci Ganewa ba (Lasaran da ba Uremic): Kada a taɓa yin biopsy cibiyar baƙar fata ta eschar kuma a guji faɗuwar biopsies na tsinke. Yi amfani da dermatopathologist-shugaban "Double-Punch" (Telescoping) Technique a cikin indurated periphery (a ** 6 mm naushi kayan aiki don isa dermis biye da coaxial 4 mm naushi kayan aiki ci gaba zurfi cikin subcutaneous mai ***) da kuma buƙatar **Von Kossa (azurfa nitrate) da Alizasrin.

Ka'idar asibiti mataki-mataki: Sodium Thiosulfate Dosing, Ceto Intralesional, Hyperbaric Oxygen, da Novel Therapies (SNF472)¶

1. Jiki da Intralesional Sodium Thiosulfate (Na2S2O3) ladabi¶

• Matsakaicin Maganin Hemodialysis na Jiki:** Gudanar da 25 grams (100 ml na maganin 25%) wanda aka diluted a cikin 100 ml na 0.9% saline na al'ada a cikin intravenously akan mintuna 30 zuwa 60 na ƙarshe na kowane zaman hemodialysis, sau 3 a kowane mako (ga marasa lafiya, suna yin awo a ƙasa da 60 kg zuwa 60 kg). hana mummunan acidosis na rayuwa da amai).
• A cikin marasa lafiya tare da aikin koda na al'ada (eGFR> 60 ml/min), ana ba da gram 12.5 zuwa 25 na IV sama da mintuna 60 sau 3 zuwa 5 a mako guda** yayin da ake sa ido sosai kan bicarbonate na jini, ratar anion, calcium, da tazara ta QT.
• ** Don allurai na gida ko marasa lafiya marasa haƙuri na IV STS, likitocin fata suna allurar ** 1 zuwa 3 ml na 250 mg / ml (25%) sodium Thiosulfate diluted 1: 1 tare da 1% Lidocaine (ba tare da epinephrine ba) ana yin allurar sau ɗaya a mako. Kada a taɓa sanya STS mai da hankali a cikin peritoneally (yana haifar da peritonitis na sinadarai).

2. Sarrafa Hyperparathyroidism na Sakandare da Hyperbaric Oxygen Therapy (HBOT)¶

• ** Calcimimetics Sama da Parathyroidectomy:** Idan ingantaccen hormone parathyroid (iPTH) ya wuce 300 pg/mL, fara na baka Cinacalcet ( Sensipar, 30 zuwa 180 mg/rana)** ko kuma na cikin jini *Etelcalcetide (Parsabiv/week), 5 IV-HD zuwa gaba. Gwajin EVOLVE ya nuna cinacalcet yana rage aukuwar calciphylaxis da 69% zuwa 75%. Ajiye gaggawa ** tiyata parathyroidectomy ** don hyperparathyroidism refractory (iPTH> 500 zuwa 1,000 pg/mL) tare da ci gaba da hypercalcemia. Kashe-lakabin IV bisphosphonates (pamidronate) da ** hexasodium fytate (SNF472) suna hana haɓakar lu'ulu'u na hydroxyapatite a cikin lokuta masu jujjuyawa.
• Hyperbaric Oxygen Therapy (HBOT) da Safe Rauni: Saboda calciphylaxis ulcers suna da hypoxia mai tsanani (transcutaneous PO2 <20 mmHg), HBOT a 2.0 zuwa 2.5 ATA na 90 minutes kullum (20 zuwa 40 zaman) yana mayar da oxygenogenesis na nama. Bar busassun, baƙar fata eschars marasa kamuwa da cuta a matsayin suturar halitta; yi amfani da man shafawa mai laushi mai laushi na enzymatic collagenase, riguna na hydrogel, ko kuma bakararriyar maganin maggot (* Lucilia sericata*)** don cirewa.

Lambobin gaggawa na yanki da ka'idojin hukumar¶

• Amurka da Kanada: Kiran kira 911 nan take ga duk wani majinyacin dialysis mai saurin fadada ciwon necrotic ulcers, zazzabi, hauhawar jini, ko ciwon ischemic mai saurin jurewa. Likitoci na iya tuntuɓar ** Babban Asibitin Massachusetts (MGH) Shirin Calciphylaxis Multidisciplinary Calciphylaxis** da KDIGO CKD-MBD jagororin.
• United Kingdom: Kiran kira 999 ko ci gaba zuwa Sashen Gaggawa na NHS / Renal Renal a ƙarƙashin ** UK Kidney Association (UKKA)** da Ƙungiyar Likitocin fata ta Biritaniya (BAD) ka'idojin calciphylaxis.
• ** Tarayyar Turai, Ostiraliya, da Asiya-Pacific: ** Kira 112 a duk faɗin EU (** Cibiyar Sadarwar Calciphylaxis ta Turai [EuCalNet]), ** 000 a Ostiraliya / ** 111** a New Zealand (KHA-CARI), ko **119 / 995 / fadin Asiya-Pacific.

Tambayoyin da ake yawan yi¶

Ta yaya Calciphylaxis ya bambanta da na yau da kullun na Ciwon Jiji na Jiji (PAD) a cikin majiyyaci akan dialysis?¶

A cikin cututtukan Jijiyoyin Jiji na yau da kullun (PAD), plaques cholesterol sun rage manyan, masu suna arteries na ƙafafu, suna haifar da raɗaɗin maraƙi yayin tafiya, bugun ƙafar ƙafa, da gyambon ƙafafu ko diddige. A cikin Calciphylaxis (Calcific Uremic Arteriolopathy), manyan arteries na ƙafafu galibi suna buɗewa gaba ɗaya kuma bugun idon sawu da ƙafar mara lafiya har yanzu suna da ƙarfi. Madadin haka, calciphylaxis da zaɓin yana juya ƙananan arterioles (ƙasa da faɗin milimita 0.6) a cikin gaɓoɓin kitse mai zurfi zuwa kashi, sannan kuma ɗigon jini wanda ke toshe waɗannan ƙananan tasoshin. Shi ya sa calciphylaxis yakan buge wurare masu kiba kamar ciki, cinyoyi, gindi, da nono, yana haifar da kurji mai kama da shunayya da baƙar fata duk da bugun ƙafafu na yau da kullun.

Me yasa shan Warfarin (Coumadin) mai sikanin jini ke haifar da Calciphylaxis, kuma me yasa dole a daina shi a rana ta 1?¶

Don kiyaye calcium da phosphate a cikin jini daga juyawa zuwa kashi a cikin arteries, ƙwayoyin tsoka da ke rufe ƙananan jini suna samar da garkuwar furotin mai kariya mai suna Matrix Gla Protein (MGP), wanda ke buƙatar Vitamin K don kunna shi. Warfarin yana toshe Vitamin K a cikin hanta da bangon jijiyar jini, yana rufe Matrix Gla Protein mai aiki da sama da kashi 80. Idan ba tare da garkuwar MGP ba, ƙananan arterioles a cikin kitse na majiyyaci suna haɓaka cikin sauri zuwa kashi mara ƙarfi, yana ƙara haɗarin calciphylaxis har zuwa ninki 10. Dakatar da warfarin nan da nan, ba da ƙarin bitamin K1 don kunna MGP baya, da kuma canzawa zuwa mai ba da bitamin-K na jini (kamar apixaban na renal-dosed) ya zama tilas a rana ta 1.

Ta yaya aka bambanta Calciphylaxis daga Mummunar Warfarin-Induced Skin Necrosis (WISN)?¶

Babban Warfarin-Induced Skin Necrosis (WISN) ya buge a cikin kwanaki 3 zuwa 10 na farko bayan fara babban maganin warfarin ba tare da gadar heparin ba: saboda warfarin yana saukar da Protein C na anticoagulant na halitta (rabin rayuwa 6 hours) da sauri fiye da clotting Factor II (rabin rayuwa 60 hours), mai haƙuri ya shiga cikin yanayin wucin gadi da hypercots. sifili sifili a cikin tasoshin jini. Calciphylaxis, akasin haka, yana tasowa bayan watanni ko shekaru na amfani da warfarin na yau da kullun ko dialysis na koda, ya haɗa da ƙididdige ƙasusuwan ƙasusuwa na ƙananan arterioles, kuma ana kula da su da Sodium Thiosulfate na cikin jijiya da kuma dakatar da warfarin.

Ta yaya Sodium Thiosulfate na Jiki (STS) ke warkar da gyambon Calciphylaxis kuma ya rage zafi?¶

Sodium Thiosulfate mai ciki (wanda aka saka a cikin gram 25 a cikin sa'a na ƙarshe na kowane zaman hemodialysis sau uku a mako) yana yaƙi da calciphylaxis ta hanyoyi uku: yana sarrafa sinadarai maras narkewa a cikin bangon arteriolar zuwa cikin calcium thiosulfate mai narkewa mai narkewa wanda ke wanke ta cikin injin dialysis, antioxidant yana haifar da kuzari mai ƙarfi. oxide don fadada ƙananan ƙananan jiragen ruwa, sau da yawa yana kawar da zafi mai zafi a cikin kwanaki 7 zuwa 14.

Me yasa lalatawar fiɗar fiɗa ke da haɗari yayin aikin Calciphylaxis na farko?¶

A cikin calciphylaxis mai aiki, fata mai launin shuɗi da ke kewaye da kitsen da ke ƙarƙashinsa suna fama da matsananciyar yunwar kwararar jini saboda ƙayyadaddun cibiyar sadarwa ta arteriolar microscopic kuma ta toshe. Yanke cikin waccan iyakar ischemic tare da ƙwanƙwasa yana haifar da rashin ƙarfi da matsanancin thrombosis na microvascular, yana haifar da sabbin gefuna da aka yanke su mutu kuma su zama baki. Sai dai idan eschar yana da jika ko yada sepsis na kwayan cuta a cikinsa wanda dole ne a shayar da shi, ƙwararrun suna amfani da man shafawa na enzymatic mai laushi, suturar hydrogel, bakararre na likitanci, da maganin oxygen hyperbaric yayin da Sodium Thiosulfate ke share calcium na jijiyoyin jini.

Tushen da jagorar hukuma¶

• ** New England Journal of Medicine (NEJM) Review Practice Review (Nigwekar SU, Thadhani R, Brandenburg VM):** * Calciphylaxis: Pathogenesis na Calcific Uremic Arteriolopathy, Matrix Gla Protein Inhibition ta Warfarin, Diagnostic Triage, da Intravenous Sodium Thiosulfate Protocol*.
• Cutar Koda: Inganta Sakamakon Duniya (KDIGO): * Sabunta Jagorar Ayyukan Kula da Lafiya don Ciwon Koda na Jiki-Ma'adinai da Cutar Kashi (CKD-MBD)*.
• ** Babban Asibitin Massachusetts (MGH) Shirin Calciphylaxis da Ƙungiyar Ƙwararrun Ƙwararrun Ƙwararrun Ƙwararrun Ƙwararrun Ƙwararrun Ƙwararrun Ƙwararrun Ƙwararrun Ƙwararrun Ƙwararrun Ƙwararrun Ƙwararrun Ƙwararrun Ƙwararrun Ƙwararrun Ƙwararrun Ƙwararrun Ƙwararrun Ƙwararrun Ƙwararrun Ƙwararrun Ƙwararrun Ƙwararrun Ƙwararrun Ƙwararrun Ƙwararrun Ƙwararrun Ƙwararru.
• ** Ƙungiyar Koda ta Burtaniya (UKKA), Ƙungiyar Ƙwararrun Ƙwararrun Ƙwararrun Ƙwararrun Ƙwararrun Ƙwararrun Ƙwararrun Ƙwararrun Ƙwararrun Ƙwararrun Ƙwararrun Ƙwararrun Ƙwararrun Ƙwararrun Ƙwararrun Ƙwararrun Ƙwararrun Ƙwararrun Turai (EuCalNet)

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