Calciphylaxis (Calcific Uremic Arteriolopathy): Plaques Retiform Retiform Violaceous, Black Necrotic Eschars ee Bukaan-socodka Sifeynta, iyo Sodium Thiosulfate¶

Calciphylaxis, oo caafimaad ahaan loogu magac daray ** calcific uremic arteriolopathy (CUA)** marka ay ku dhacdo bukaanada qaba cudurka kelyaha heerka ugu dambeeya, waa xaalad halis ah oo halis gelinaysa xididdada xididdada dhiigga oo lagu garto ** calcification dhexdhexaad ah, fibrosis subintimal, iyo xinjirowga degdega ah ee maqaarka yar yar iyo xididdada hoose ee xididdada. mikromitir dhexroorka ah). Si ka duwan sida cudurada caadiga ah ee atherosclerotic ee xididada xididada xididada xididada waaweyn ee lugaha marka ay ka tagayaan garaaca badelada daciifka ama maqan, calciphylaxis waxay garaacdaa shabakada arteriolar microscopic ee gudaha gudaha ** adipose-hodanka dufanka subcutaneous ** (caloosha, panniculus, bowdyaha iyo naasaha) garaaca canqowga iyo cagtu inta badan waxay ahaanayaan kuwo si buuxda loo dareemi karo**. Bukaan-socodka hemodialysis joogto ah ama sifeynta peritoneal-ka, ama bukaanka qaba shaqada kelyaha ee caadiga ah qaadashada warfarin (calciphylaxis aan ahayn uremic calciphylaxis) , luminta calcifilada xididdada dabiiciga ah ee dabiiciga ah, gaar ahaan ** karboksylated Matrix Gla Protein (MGP) iyo cagaarshow ** fetuin-A, oo ay weheliso wax soo saarka kaalshiyam-ka-faytamiin, analogs (calcitriol, paricalcitol), kalsiyum ku salaysan fosfateed binders (calcium acetate, calcium carbonate), iyo irbado subcutaneous soo noqnoqda (insulin ama heparin) *** kiciyaa isbedel phenotypic unugyada muruqa siman xididada galay lafo-samaynta ** unugyada osteoblass-sida**. Marka lumen halbowlaha la kafeeyey lagu xidho xinjirow fibrin oo ba'an, maqaarka sare iyo baruurta ayaa la kulma masiibo. Kiliinik ahaan, calciphylaxis waxay ku dhawaaqdaa ** xanuun daran, gubasho, 10-ka-baxsan-10 xanuunka maqaarka ischaemic kaas oo si joogta ah uga horreeya necrosis maqaarka muuqda maalmo**, si degdeg ah uga soo baxa ** retiform violaceous retiform (net-like) purpura iyo dhagax-adag, qandho hoose oo alwaax ah*** maqaar wareegsan iyada oo loo marayo xudduudaha xunxun. Sababtoo ah calciphylaxis eschars boogta leh waxay si toos ah u fadhiistaan ​​baruurta la leexsaday, duulaanka labaad ee bakteeriyada waxay keenaysaa ** sepsis nabar buuxa **, taasoo wadata heerka dhimashada taariikhiga ah ee halka sano ee 50 ilaa 80 boqolkiiba. Haddii bukaan-socodka sifaynta ama isticmaala warfarin ay yeeshaan xanuun aad u xanuun badan oo guduudan oo shabag ah, nodules subcutaneous alwaax ah, ama boogaha maqaarka necrotic ee madow, wac degaankaaga nambarada degdega ah ama isla markiiba u gudub waaxda xaaladaha degdegga ah. Sidoo kale dib u eeg hagayaasha walaalahayaga Helitaanka sifeynta AV fistula dhiig-bax nafta halis gelinaya iyo sifaynta sifaynta hyperkalemia, erysipelas vs necrotizing fasciitis vs stasis dermatitis kala soociddaiyo sepsis iyo aqoonsiga naxdinta septic.

Muhiimadahaaga degdega ah ee degdega ah waa inaad si dhakhso ah u joojiso dhammaan dardar-geliyayaasha xisaabinta klzzwxh:0005 Marxaladda *** halkaao pathergy iyo ischemia microvascular ay sababaan nabarrada inay si musiibo ah u fidaan.

Hadda samee tan¶

1. ** Raadso Isbitaal Degdeg ah ama Qiimaynta Nephrology ee Degdegga ah iyo Maqaarka haddii bukaanka sifaynta (ama qaadanaya warfarin) uu yeesho xanuun aad u xanuun badan oo jilicsan maqaarka maqaarka, qanjidhada dufanka hoose ee adag, ama necrotic necrotic eschars. 000 gudaha Australia, ama kala tasho hagahayaga caalamiga ah nambarada degdega ah. Haddii bukaanku uu sidoo kale leeyahay qandho, qallafsanaan, hypotension, jahawareer, ama dheecaan aan nadiif ahayn oo ka yimaadda boogaha boogaha leh, ku dhawaaq ** xaalad degdeg ah oo sepsis** ah.
2. ** Hantidhawr oo jooji "Afarka Weyn" Dardariyeyaasha Farmashilojiyeed ee Calciphylaxis Isla Markay Cisbitaalka Imaan:**
3. ** JOOJI Warfarin (Vitamin K Antagonists):** Warfarin wuxuu xannibaa gamma-kaarboksyeynta ** Matrix Gla Protein (MGP), ka-hortagga ugu awoodda badan ee jirka ee xannibaadaha dhexdhexaadinta arteriolar. Bukaanka u wareeji daawada xinjirowga lidka ku ah afka ee non-vitamin-K (sida apixaban ee qiyaasta kelyaha la hagaajiyay) ama heparin daweynta iyadoo la tashanaysa hematology iyo nephrology, oo maamul ** Vitamin K1 (phytonadione, 10 mg afka ama IV saddex jeer toddobaadkii)
4. ** JOOJI Dhammaan Isku-xidhayaasha Kaalshiyam-ku-salaysan Fosfate-ka (PhosLo [calcium acetate], Tums [calcium carbonate]) iyo Kaalshiyam Kaabayaasha:** Isla markiiba u beddel ** xidhayaasha fosfate-ka ee aan kalsiyumku ku jirin** sida ** sevelamer carbonate (* Renvela*) os renol ** sucroferric oxyhydroxide (Velphoro)**.
5. ** JOOJI Dhammaan Faytamiin D Firfircoon Analogs (Calcitriol, Paricalcitol, Doxercalciferol) iyo Qiyaasta Sare ee Ergocalciferol/Cholecalciferol:** Vitamin D agonistayaasha waxay kordhiyaan nuugista calcium xiidmaha iyo nuugista fosfate waxayna si toos ah u kiciyaan xididdada muruqa siman. Haddii bukaanku leeyahay hyperparathyroidism sare oo daran (iPTH> 300 pg/mL), u beddel calcimimetic (** cinacalcet afka ah [* Sensipar*] ama etelcalcetide xididka [Parsabiv]**) ama diyaarso parathyroidectomy degdeg ah.
6. ** JOOJI DEXTRAN/Sucrose-ka-xiniinyaha iyo Cirbadaha Subcutaneous ee Dufanka Dufanku Saameeyey:** Culayska birta ahi waxay kicisaa dhaawaca xuddunta xorta ah ee hydroxyl ee maqaarka ischemic, iyo soo noqnoqda ** insulin subcutaneous ama cirbadeynta heparin** oo la geliyo panniculus caloosha ama bowdada ee panniculus ama bowdada waxay kicisaa xinjirta dhiigga. U rog cirbadaha meelaha caatada ah ee aan waxyeello gaarin ama u gudub faleebo xididka marka isbitaalka la dhigo.
7. ** Bilow Sodium Thiosulfate xididka (Na2S2O3) ee 25 Grams IV Saddex Jeer Todobaadle ah (inta lagu jiro 60 ka daqiiqo ee ugu dambeeya Kalfadhi kasta oo Hemodialysis): *** ** Sodium Thiosulfate xididka Waxay jeexisaa kaydadka calcium ee unugyo aan milmi karin ** thiosulfate calcium ultra-soluble ** (taas oo ah 250-laab ilaa 100,000-laab in ka badan oo ku milmi biyaha marka loo eego kalsiyum fosfat ama oxalate calcium waxaana si degdeg ah looga nadiifiyaa xuubka hemodialysis), waxay u shaqeysaa sidii awood leh ** antioxidant-ka xorta ah ee xorta ah ** The OS synalth. waxay keentaa ** vasodilation toos ah arteriolar ** kaas oo inta badan yareeya xanuunka maqaarka ischaemic ee daran gudaha ** 1 ilaa 2 usbuuc.
8. ** U fiirso oo maareyso Labada Dhibaato ee Khatarta ah ee IV Sodium Thiosulfate: Farqiga Anion Sare ee dheef-shiid kiimikaadka iyo yalaalugo/hypotension daran:**
9. Marka sodium thiosulfate uu metabolized by beerku galay ** thiosulfuric acid iyo sulfate anions *, waxa ay abuurtaa qoto dheer ** farqiga anion sare ee dheef-shiid kiimikaad acidosis (inta badan hoos u dhigaysa serum bicarbonate hoos 14 ilaa 16 mmol/L). Hubi qaybta dheef-shiid kiimikaadka aasaasiga ah iyo farqiga anion ka hor fadhi kasta oo sifaynta iyo kordhi fiirsashada dhiig-baxa qubeyska bicarbonates (ama maamul sodium bicarbonate afka ah oo keliya haddii heerka mugga uu oggol yahay).
10. Ku shubista 25 garaam ee STS si degdeg ah (< 30 daqiiqo) waxay sababtaa ** matag mad-madow ah, lallabo daran, iyo hypotension ba'an intradialytic**. Had iyo jeer ku daaweeya IV Ondansetron (4 ilaa 8 mg) ama Promethazine 30 daqiiqo kahor faleebada, oo si tartiib ah ugu geli STS in ka badan 60 daqiiqo.
11. ** Isla markiiba u hagaaji dawada dhiigbaxa:** Kordhi inta jeer ee sifaynta sifaynta iyo muddada (tusaale, 4 ilaa 5 fadhi wiiggii 4 saacadood midkiiba) si aad si xun u nadiifiso phosphates iyo kaadimareenka serum, oo u beddel qubayska dialysate ** hoose-kaalshiyam dialysate ah (1.5 to 2.5 m. mmol/L)** si ay kaalshiyamku si firfircoon ugu faafto kabaxsan dhiiga bukaanka marka sifaynta. Haddii bukaanku uu ku jiro ** sifaynta peritoneal (PD)** (taas oo sido dhacdo aad u sarreeya oo calciphylaxis ah sababtoo ah badeecooyinka hoos u dhigaya gulukooska xuubka xuubka xuubka iyo nadiifinta fosfattu liidata), si adag uga fiirso ** u gudubka degdega ah ee hemodialysis**.
12. ** Ilaali maqaarka boogaha oo ka fogow qalitaan qoto dheer oo qallafsan inta lagu jiro ischemia firfircoon (ka hortagga balaadhinta boogaha Pathergic):** Si ka duwan necrotizing fasciitis (oo u baahan qalliin degdeg ah oo ballaadhan qolka qalliinka), gooynta ischemic ku xeeran, maqaarka calcifid leh ee calciphylaxis wuxuu abuuraa horumar joogto ah. god weyn oo aan bogsan karin. Madow nadiif ah, engegan, oo la ilaaliyo nadiif ah, engegan, iyo infekshanka qoyan ee qoyan ee hoosta ka soo baxa; marka laga saaro slough necrotic loo baahan yahay si looga hortago sepsis, isticmaal ** khafiif ah enzymatic debridement (collagenase), hydrogel autolytic dhaymada, maggot debridement therapy, ama si taxadar leh ka saarida muxaafidka ah unug dabacsan devitalized unug lagu lammaaniyo Hyperbaric Oxygen Therapy (HBOT)** mar caleenta arteriolar waa la daaweynayaa.
13. ** Bixi Xakamaynta Xanuunka Xanuunka Badan ee Multimodal Neuropathic iyo Ischemic (Si Adag Uga Fogaanshaha Morphine iyo Meperidine ee Bukaannada Sifaynta):** Xanuunka Calciphylaxis wuxuu ka mid yahay xanuunka xanuunka ugu xanuunka badan ee daawada, isku darka unugyada ischaemic qotodheer iyo dhaawaca neuropathic yar-fiber. Waligaa ha u isticmaalin morphine, codeine, ama meperidine marka kelyaha ay fashilmaan (shiidkooda sunta ah morphine-6-glucuronide iyo normeperidine waxay isu uruursadaan heerar dilaa ah taasoo keenaysa myoclonus, suuxdin, iyo neefsashada oo xidhma). Bedelkeeda, isticmaal ** faleebada ama transdermal Fentanyl, Methadone, ama Buprenorphine** (dhammaan ku badbaado sifeynta oo leh eber firfircoonida kelyaha) oo ay weheliso kelyaha la hagaajiyay Gabapentin (100 ilaa 300 mg ka dib-dialysis) ama ** faleebo ketamiin ah**.

Decision tree: suspected Calciphylaxis (Calcific Uremic Arteriolopathy) vs Warfarin Skin Necrosis vs Necrotizing Fasciitis¶

flowchart TD
    A["Dialysis Patient (HD/PD) or Warfarin User Presents with Excruciating 10/10 Burning Skin Pain + Violaceous Retiform (Net-Like) Plaques, Woody Subcutaneous Nodules, or Black Stellate Necrotic Eschars on Abdomen, Thighs, Buttocks, or Legs"] --> B{"Step 1: Immediate Sepsis & Necrotizing Fasciitis Screen: Is There Crepitus, Dishwater Fluid, Rapidly Spreading Fascial Bullae, High Fever, or Septic Shock?"}
    B -->|YES: Wet Gangrene / Fulminant Wound Sepsis or Necrotizing Fasciitis| C["Resuscitate with IV Crystalloids + Broad-Spectrum IV Antibiotics (Vancomycin + Cefepime/Piperacillin-Tazobactam + Metronidazole) + Urgent Surgical / Wound Consult for Infected Eschar Drainage"]
    B -->|NO: Dry Leathery Black Eschar or Intact Violaceous Plaque / Woody Subcutaneous Nodule| D["Step 2: Bedside Vascular & Medication Audit + Plain Soft-Tissue Radiographs (Mammography-Technique X-Rays) or Bone Scintigraphy"]
    C --> D
    D --> E{"Check Timing of Warfarin Initiation (Did Necrosis Evolve Within Day 3 to Day 10 of Starting Warfarin Without Heparin Bridge?)"}
    E -->|YES: Acute Onset Day 3 to 8 of Warfarin + Rapid Protein C Drop| F["ACUTE WARFARIN-INDUCED SKIN NECROSIS (Microvascular Venular Thrombosis): Stop Warfarin + Give IV Vitamin K + Start Therapeutic IV Unfractionated Heparin + Fresh Frozen 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["CONFIRMED CALCIPHYLAXIS (Calcific Uremic Arteriolopathy - CUA)"]
    G --> H["STEP 1 (Halt All Accelerators Immediately): 1. STOP Warfarin (Give Vitamin K1 10 mg 3x/wk; switch to Apixaban if anticoagulation needed); 2. STOP Calcium Phosphate Binders (Switch to Sevelamer/Lanthanum); 3. STOP Calcitriol/Vitamin D Analogs; 4. STOP Iron & SubQ Injections in Plaques"]
    H --> I["STEP 2 (Start Definitive Calcium-Chelating & Vasodilatory Therapy): Administer IV SODIUM THIOSULFATE (25 Grams IV Over Final 60 Min of Each Hemodialysis Session 3x/Week) + Premedicate with IV Ondansetron"]
    I --> J["TALLAABADA 3 (Kor u Samee Sifeynta & dhidibka Parathyroid): Kordhi Hemodialysis ilaa 4-5x/Toddobaadkii oo leh Qubays-Kaalshiyam-hooseeya (1.5mEq/L); Haddii iPTH> 300 pg/mL, Bilow Cinacalcet / Etelcalcetide ama Diyaarso Parathyroidectomy degdeg ah"]
    J --> K["TALLAABADA 4 (Daryeelka Xannaanada Nabdoon ee Xannaanada & Dhaawaca): Isticmaal Fentanyl, Methadone, ama Buprenorphine + Gabapentin-ka Dib HD (WELI morphine/Meperidine); Ka fogow qalitaanka qoto dheer ee Eschars ee qallalan; Tixgeli Ogsajiinta Hyperbaric (HBOT) & Sodium Thiosulfate Intralesional"]

Pathophysiology: sida unugyada muruqa siman ee xididdada dhiigga ay u noqdaan lafo iyo sababta unugyada adipose ay u yihiin bartilmaameedka koowaad¶

Tobannaan sano, dhakhaatiirtu waxay u malaynayeen in calciphylaxis uu si fudud u ahaa roob kiimiko ah oo aan caadi ahayn oo kalsiyum iyo fosfateed ah markii serum ** badeecada calcium-phosphate (Ca x PO4)** ay dhaaftay 55 ilaa 70 mg labajibbaaran dL. Cilmi-nafsiga xididdada dhiigga ee casriga ahi waxa uu caddeeyey in calciphylaxisku dhab ahaantii yahay firfircoon, is-beddelka osteogenic-ka-dhexdhexaadinta unugyada oo ay ku xigto ** garaaca labaad ee xinjirowga ba'an ***:

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.
=======================================================================

Bartamaha (Adipose-Rich) vs. Peripheral (Acral) Calciphylaxis: Qaababka Clinical iyo Saadaasha¶

Clinicians classify calciphylaxis into two distinct anatomical distributions that carry very different survival outcomes: 1. ** Bartamaha (Proximal / Adipose-Rich) Calciphylaxis (70% ilaa 80% kiisaska):** Waxay ku dhufataa meelaha unugyo adipose ka hooseeya oo qaro weyn leh, gaar ahaan ** caloosha caloosha, bowdada dhexe iyo bowdada, barida, garbaha, iyo naasaha dheddigga. Sababtoo ah lobules adipose waxaa lagu bixiyaa dheer, tortuous, weelasha dhamaadka-arteriolar oo leh wareegga dammaanadda liidata iyo sababtoo ah adipocytes qaan-gaar ah ayaa qarsoodiya adipokines pro-calcific (leptin, TNF-alpha, iyo IL-6) kuwaas oo dardargeliya firfircoonida muruqa siman ee xididdada RUNX2, suufka dhexe ee dufanka dhexe ayaa si gaar ah u nugul. Calciphylaxis-ka dhexe waxa uu qaadaa ** heerka dhimashada ugu sarraysa (60% ilaa 80% 1 sano)sababtoo ah boogaha waaweyn ee caloosha iyo bowdada waxay si degdeg ah u galaan bakteeriyada Gram-negative bakteeriyada iyo Staphylococcus aureus, taasoo keenta bakteeriyada dilaaga ah. 2. Deegaanka (Distal / Acral) Calciphylaxis (20% ilaa 30% ee Kiisaska): Waxay ku dhufataa aagagga fog ee baruurta leh ee leh dufanka hoose ee ugu yar, oo ay ku jiraan ** weylaha, shilalka, anqawyada, suulasha, faraha, iyo marmar guska glans (calciphylaxis guska). Nabarada durugsan waxay wataan heer ka hooseeya (inkasta oo ay wali halis yihiin) heerka dhimashada ee 20% ilaa 40%, in kastoo gangrene dhijitaalka ah ama xubinta taranka ahi ay u baahan tahay in si taxadir leh loo kala saaro cudurka halbowlayaasha atherosclerotic peripheral arterial (PAD) iyo kolesteroolka embolization. 3. ** Calciphylaxis aan ahayn Uremic (NUC, 10% ilaa 15% ee Kiisaska):** Waxay ku dhacdaa bukaanka qaba shaqada kelyaha ee caadiga ah (eGFR> 60 mL/min) oo ay wadaan ** isticmaalka warfarin dabadheeraad ah , * hyperparathyroidism aasaasiga ah, ** cudurada autoimmune ee corticosteroids ee joogtada ah**** abuurka fetuin-A iyo Protein C/S).

Side-by-side differential diagnosis: Calciphylaxis vs Warfarin-Induced Skin Necrosis vs Necrotizing Fasciitis vs Cholesterol Embolization vs Pyoderma Gangrenosum¶

Sababtoo ah ogaanshaha calciphylaxis sida pyoderma gangrenosum (iyo bixinta qiyaasta sare ee corticosteroids ee soo dedejisa calcification iyo sepsis) ama si khaldan u ogaanshaha sida fasciitis necrotizing (iyo samaynta burburka xagjirka ah ee kicinaya masiibo aan bogsiin lahayn), waxay isticmaali karaan bukaanka sariirta sariirta dhinaceeda.

Burburinta ogaanshaha: Goorma Laga Fogaado Biopsiga Maqaarka¶

1. ** Marka la ogaado cudurka kelyaha ee heerka ugu dambeeya ee cudurka kelyaha ee sifaynta, oo ay weheliso huurada retiform-ka ee qadiimiga ah, qanjidhada subcutaneous alwaax ah, ama stellate madoow madoow * baayoobyo oo caloosha ama bowdada ah, badanaa waa maqaar halis ah. Goynta xuubka calciphylaxis ischaemic wuxuu xambaarsan yahay ** 30 ilaa 50 boqolkiiba khatarta kicinta boogaha pathergic, balaadhinta nabarka aan bogsanaynin, iyo sepsis labaad oo dilaa ah**.
2. Dareen-sare oo aan ahayn sawir-qaadis beddel ah:
3. ** Shaadhooyin cad oo jilicsan iyadoo la adeegsanayo farsamada naas-nuujinta ee xallinta-sare:** Muujinaysa ** ganaax, lacy, arborizing (laan-geed ama saafid u eg) xisaabinta halbowlayaasha yaryar ee maqaarka hoostiisa (< 100 ilaa 500 micrometers)** oo leh gaar ahaan soo dhawaada boqolkiiba 90.
4. Technetium-99m (Tc-99m MDP) Sadex-waji Scintigraphy Lafaha: Waxay muujisaa kor u qaadida raadiyaha raadiyaha ee gudaha bararka, xisaabinta suufka subcutaneous ee bowdada, caloosha, iyo barida (dareenka 89% ilaa 97%).
5. Sida Nabdoon Loo Sameeyo Ka-qaadista Ka-qaadista Marka Aan La Hubin Cilad-sheegashadu ( Kiisaska Aan Uremic-ka Ahayn): Waligaa ha ka qaadin dheecaan ka-qaadis ah xarunta eschar necrotic ee necrotic, kana fogow cad-cad-baxyo ballaaran oo la jarjaray. Isticmaal takhtar ku takhasusay cudurrada maqaarka oo haga "Double-Punch" (Telescoping) Technique ee hareeraha la taaban karo (a ** 6 mm qalabka feerka si loo gaaro maqaarka oo ay ku xigto qalabka feerka coaxial 4 mm oo si qoto dheer u galay dufanka hoose ***) oo codso **Von Kossa (Nitrate Silver) iyo Alizasrin.

Tallaabo-tallaabo Hab-maamuuska Cusbitaalka: Qiyaasta Sodium Thiosulfate, Badbaadinta Intralesional, Ogsajiinta Hyperbaric, iyo Daaweynta Novel (SNF472)¶

1. Nidaamka xididka iyo xididdada dhiigga ee Sodium Thiosulfate (Na2S2O3)¶

• Qiyaasta Hemodialysis ee Halista ah:** Maamule 25 garaam (100 ml oo ah 25% xal) oo lagu qasi jiray 100 ml oo ah 0.9% cusbada caadiga ah ee xididka 30 ilaa 60 daqiiqo ee u dambeeya kalfadhi kasta oo hemodialysis, 3 jeer wiiggii (Bukaanada, oo miisaankoodu ka hooseeyo 60kg ilaa 61 garaam. ka hortagga aashitada dheef-shiid kiimikaadka daran iyo matagga).
• ** Qaadashada Bukaannada Calciphylaxis-ka aan-Uremic-ka ahayn:** Bukaannada qaba shaqada kelyaha ee caadiga ah (eGFR> 60 mL/min), maamul 12.5 ilaa 25 garaam IV oo lagu shubay in ka badan 60 daqiiqo 3 ilaa 5 jeer toddobaadkii iyadoo si dhow loola socdo serum bicarbonate, farqiga anion, calcium, iyo muddada QT
• Intralesional Sodium Thiosulfate Irbadaha: Tabeelooyin gudaha ah ama bukaannada aan u dulqaadan karin IV STS, dhakhaatiirta maqaarka waxay ku duraan **1 ilaa 3 ml oo ah 250 mg/mL (25%) Sodium Thiosulfate waxaa lagu qasi jiray 1:1 oo leh 1% Lidocaine (la'aanteed epinephrine) ayaa lagu qiimeeyay hal mar oo firfircoon. Waligaa ha ku beerin STS-takooban ee intraperitoneal (waxay sababtaa peritonitis kiimikaad).

2. Xakamaynta Hyperparathyroidism Secondary iyo Hyperbaric Oxygen Therapy (HBOT)¶

• ** Calcimimetics Over Parathyroidectomy:** Haddii hoormoonka parathyroid-ectomy ee saxda ah (iPTH) uu dhaafo 300 pg/mL, ku bilaw afka Cinacalcet ( Sensipar, 30 ilaa 180 mg/maalin)** ama xididada xididka Etelcalcetide (Parsabiv/week) Tijaabada EVOLVE waxay muujisay cinacalcet inay yarayso dhacdooyinka calciphylaxis 69% ilaa 75%. Degdeg ah u dhig ** qaliinka parathyroidectomy ee hyperparathyroidism refractory (iPTH> 500 ilaa 1,000 pg/mL) oo leh hypercalcemia joogto ah. Calaamadda ka baxsan IV bisphosphonates (pamidronate) iyo ** hexasodium fytate (SNF472)** waxay hakiyaan korriinka crystal hydroxyapatite ee kiisaska refractory.
• Hyperbaric Ogsajiin Therapy (HBOT) iyo Daryeelka Nabdoon ee Nabdoon Ka tag maqaarka qallalan ee madow ee aan cudurka qabin sida dhar bayooloji ah; isticmaal ** boomaatada collagenase enzymatic fudud, dhaymada hydrogel, ama daawaynta maggot ee nadiifka ah ( Lucilia sericata)** si tartiib ah looga saaro.

Lambarada degdega ah ee gobolka iyo borotokoolka wakaalada¶

• Mareykanka iyo Kanada: Wac 911 isla markiiba si aad u wacdo bukaan-socod kasta oo leh boogaha necrotic ee degdega u sii fidaya, qandho, dhiig-yaraan, ama xanuunka ischaemic ee aan la qabsan karin. Dhakhaatiirtu waxay la tashan karaan Massachusetts General Hospital (MGH) Barnaamijka Calciphylaxis Multidisciplinary iyo KDIGO CKD-MBD tilmaamaha.
• United Kingdom: garaac 999 ama u sii gudub Waaxda Gurmadka Degdegga ah ee NHS / Unugga Kelyaha ee hoos yimaada Ururka Kelyaha ee UK (UKKA) iyo Ururka Dhakhaatiirta maqaarka ee Ingiriiska (BAD) borotokoolka calciphylaxis.
• ** Midowga Yurub, Australia, iyo Asia-Pacific:** garaac 112 guud ahaan Midowga Yurub (European Calciphylaxis Network [EuCalNet]), 000 gudaha Australia / 111 ee New Zealand (KHA-CARI), ama **119/995

Su'aalaha soo noqnoqda¶

Sidee Calciphylaxis uga duwan yahay Cudurka Halbowlaha ee Caadiga ah (PAD) ee bukaanka sifaynta?¶

Cudurka halbowlaha wadnaha ee caadiga ah (PAD), xuubka kolestaroolka ayaa cidhiidhiya xididada waaweyn ee loo yaqaan xididada lugaha, taasoo keenta kubka xanuun marka la socdo, garaaca cagaha oo maqan, iyo boogaha suulasha ama ciribta. Calciphylaxis (Calcific Uremic Arteriolopathy), halbowlayaasha waaweyn ee lugaha ayaa inta badan si buuxda u furma oo garaaca canqowga iyo cagtu weli waa xoogan yihiin. Taa baddalkeeda, calciphylaxis waxay si door ah u beddeshaa arterioles yar yar (oo ka hooseeya 0.6 millimitir ballac) gudaha suufka dufanka hoose ee gunta hoose ee laf-dhabarta, oo ay ku xigto xinjiro dhiig oo ba'an oo xira maraakiibta yaryar. Taasi waa sababta calciphylaxis uu caadi ahaan ugu dhufto meelaha dufanka leh sida caloosha, bowdada, barida, iyo naasaha, taasoo keenta finan shabag guduudan oo kale ah iyo qolof maqaar madow ah in kasta oo cagaha caadiga ah ay garaacaan.

Maxay tahay sababta qaadashada daawada khafiifiya ee Warfarin (Coumadin) ay u kiciso Calciphylaxis, maxayse tahay in la joojiyo maalinta 1aad?¶

Si looga ilaaliyo kalsiyumka iyo fosfatka ku jira dhiigga inay u noqdaan laf gudaha halbowlayaashayada, unugyada muruqa ee ku daboolan xididada yaryar ee dhiigga waxay soo saaraan gaashaan borotiin ah oo la yiraahdo Matrix Gla Protein (MGP), kaas oo u baahan Vitamin K si uu u shido. Warfarin wuxuu xannibaa fiitamiin K ee beerka iyo gudaha derbiyada xididdada dhiigga, isagoo xiraya Matrix Gla Protein in ka badan 80 boqolkiiba. Gaashaanka MGP-ga la'aanteed, halbowlayaasha yaryar ee ku jira unugyada baruurta sifaynta bukaanka si degdeg ah ayay isu beddelaan lafo jeexan, taasoo kor u qaadaysa halista calciphylaxis ilaa 10-laab. Joojinta warfarin isla markiiba, siinta fitamiin K1 kaabis si ay MGP dib ugu soo celiso, iyo u beddelashada dhiig- khafiifiye aan fiitamiin-K ahayn (sida apixaban-kalyaha la qaato) waa qasab maalinta 1.

Sidee Calciphylaxis looga soocaa Necrosis-ka Maqaarka ee Ba'an ee Warfarin (WISN)?¶

Cudurka Ba'an ee Warfarin-Induced Skin Necrosis (WISN) wuxuu ku dhuftaa 3 ilaa 10 maalmood ee ugu horreeya ka dib marka la bilaabo qiyaasta sare ee warfarin iyada oo aan lahayn buundada heparin: sababtoo ah warfarin waxay hoos u dhigtaa xinjirowga dabiiciga ah ee Protein C (saacadaha nus-nolosha 6) si aad uga dhakhso badan xinjirowga II (nolosha nuska 60 saacadood), bukaanku wuxuu galaa xaalad ku meel gaar ah oo leh hypercots. eber kalsiyum ee xididdada dhiigga. Calciphylaxis, taa beddelkeeda, waxay soo baxdaa bilo ama sannado ka dib isticmaalka warfarin dabadheeraad ah ama sifaynta kelyaha, waxay ku lug leedahay lafaha raaga ee xididada yaryar, waxaana lagu daaweeyaa xididada Sodium Thiosulfate iyo joojinta warfarin.

Sidee buu u daaweeyaa Sodium Thiosulfate (STS) ee nabarrada Calciphylaxis oo uu ka yareeyo xanuunka?¶

Sodium Thiosulfate ee xididka ah (oo lagu shubay 25 garaam saacadii ugu dambeysay ee kalfadhi kasta oo dhiigbaxa saddex jeer usbuucii) waxay la dagaalantaa calciphylaxis saddex siyaabood: waxay si kimikal ah u chelates kaydka calcium ee aan milmi karin gudaha gidaarada arteriolar galay ultra- milmi kara calcium thiosulfate oo ku maydha iyada oo loo marayo mishiinka sifeynta, antioxidant-ka ayaa dib u soo cusboonaada maqaarka. oksaydh si ay u ballaadhiso maraakiibta yaryar ee nool, inta badan waxay yaraynaysaa xanuunka gubanaya 7 ilaa 14 maalmood gudahood.

Waa maxay sababta qalliinka qallafsan ee qallafsan uu khatar u yahay xilliga hore ee Calciphylaxis?¶

Calciphylaxis firfircoon, maqaarka guduudan ee ku wareegsan iyo dufanka hoose ayaa si ba'an u gaajoonaya socodka dhiigga sababtoo ah shabakada arteriolar microscopic waa la calsaydhiyay oo xinjirootay. Goynta xudduudaha ischaemic ee leh maqaarka maqaarka waxay kicisaa barar iyo thrombosis microvascular ba'an, taasoo keenta cidhifyada cusub ee la jaray inay dhintaan oo isu beddelaan madow. Ilaa eschar uu leeyahay malax qoyan ama faafidda sepsis bakteeriya ah oo ku hoos xanniban oo ay tahay in la miiro, takhasusayaashu waxay isticmaalaan boomaatada enzymatic ee jilicsan, dhaymada hydrogel, maggoot caafimaad oo nadiif ah, iyo daweynta oksijiinta hyperbaric halka Sodium Thiosulfate ay nadiifiso calcium vascular.

Ilaha iyo hanuuninta rasmiga ah¶

• ** Joornaalka New England ee Daawada (NEJM) Dib-u-eegis Dhaqaneed Caafimaad (Nigwekar SU, Thadhani R, Brandenburg VM):** * Calciphylaxis: Pathogenesis of Calcific Uremic Arteriolopathy, Matrix Gla Protein Inhibition by Warfarin, Kala-soocidda ogaanshaha, iyo Protocol Sodium Thiosulfate.
• Cudurka kelyaha: Hagaajinta Natiijooyinka Caalamiga ah (KDIGO): Cusboonaysiinta Tilmaanta Layliska Caafimaadka ee Cudurka Kelyaha ee Joogtada ah-Cudurka Macdanta iyo Lafaha (CKD-MBD).
• ** Isbitaalka Guud ee Massachusetts (MGH) Barnaamijka Calciphylaxis iyo Bulshada Maraykanka ee Nephrology (ASN):** * Nidaamka Maareynta Maareynta Dhexdhexaadinta ee Calciphylaxis-ka Uremic iyo non-Uremic*.
• Ururka Kelyaha ee UK (UKKA), Ururka Dhakhaatiirta maqaarka ee Ingiriiska (BAD), iyo Shabakadda Calciphylaxis ee Yurub (EuCalNet):

Tilmaamaha la xidhiidha¶

• Helitaanka sifeynta AV fistula dhiig-bax nafta halis gelisa iyo sifaynta sifaynta hyperkalemia
• Erysipelas vs necrotizing fasciitis vs stasis dermatitis: xanuunka ka baxsan saamiga, bullae, iyo LRINEC dhibcaha kala soocidda
• Necrotizing fasciitis (infekshan hilibka cuna) iyo calanka cas ee cellulitis
• Sebsiis iyo shoogga septica ogaanshaha iyo gargaarka degdegga ah
• Nambarada degdega ah wadan ahaan