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MCID: CLC001
MIFTS: 53

Calciphylaxis

Categories: Blood diseases, Bone diseases, Cardiovascular diseases, Metabolic diseases, Rare diseases, Skin diseases
Genes Tissues Related diseases Publications Pathways Symptoms & Phenotypes Drugs
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Aliases & Classifications for Calciphylaxis

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MalaCards integrated aliases for Calciphylaxis:

Name: Calciphylaxis 12 76 53 59 44 15 73
Idiopathic Calciphylaxis 53

Characteristics:

Orphanet epidemiological data:

59
calciphylaxis
Inheritance: Not applicable; Prevalence: 1-9/100000 (Europe); Age of onset: Adult; Age of death: adult;

Classifications:

MalaCards categories:
Global: Rare diseases Metabolic diseases
Anatomical: Cardiovascular diseases Bone diseases Skin diseases Blood diseases
See all MalaCards categories (disease lists)
ICD10: 34
Orphanet: 59  
Rare circulatory system diseases
Rare systemic and rhumatological diseases


External Ids:

Disease Ontology 12 DOID:4734
MeSH 44 D002115
NCIt 50 C84607
Orphanet 59 ORPHA280062
ICD10 via Orphanet 34 E83.5
UMLS via Orphanet 74 C0006666
UMLS 73 C0006666
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Summaries for Calciphylaxis

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NIH Rare Diseases : 53 Calciphylaxis is a disease in which blood vessels (veins and arteries) become blocked by a build-up of calcium in the walls of the vessels, preventing blood from flowing to the skin or internal organs.  The lack of blood flow (ischemia) damages healthy tissue and causes it to die (necrosis).  The most obvious and frequent symptom of calciphylaxis is damage to the skin, as ulcers can develop and become infected easily.  Calciphylaxis can also affect fat tissue, internal organs, and skeletal muscle, causing infections, pain, and organ failure.  These symptoms are often irreversible, and many individuals with calciphylaxis may not survive more than a few months after they are diagnosed due to infection that spreads throughout the body (sepsis), or organ failure.  The exact cause of calciphylaxis is unknown.  Treatments may include medications to reduce pain, antibiotics to treat infections, and various approaches to preventing the development or worsening of this condition.

MalaCards based summary : Calciphylaxis, also known as idiopathic calciphylaxis, is related to hyperparathyroidism and osteitis fibrosa. An important gene associated with Calciphylaxis is ALB (Albumin), and among its related pathways/superpathways are Ca, cAMP and Lipid Signaling and HIF-1-alpha transcription factor network. The drugs Protective Agents and sodium thiosulfate have been mentioned in the context of this disorder. Affiliated tissues include skin, kidney and skeletal muscle, and related phenotypes are skin ulcer and cellulitis

Wikipedia : 76 Calciphylaxis, also known as calcific uremic arteriolopathy (CUA), is a rare painful syndrome of... more...

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Related Diseases for Calciphylaxis

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Diseases related to Calciphylaxis via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 148)
# Related Disease Score Top Affiliating Genes
1 hyperparathyroidism 29.9 CASR EPO FGF23 PTH VDR
2 osteitis fibrosa 29.8 CASR PTH
3 end stage renal failure 29.7 ALB EPO PTH
4 bone resorption disease 29.5 PTH VDR
5 hypoparathyroidism 29.5 CASR FGF23 PTH
6 primary hyperparathyroidism 29.5 ALB CASR FGF23 PTH VDR
7 kidney disease 29.4 ALB CASR EPO FGF23 PTH VDR
8 acquired immunodeficiency syndrome 29.4 ALB EPO
9 hyperphosphatemia 29.4 CASR FGF23 PTH VDR
10 chronic kidney failure 29.4 ALB CASR EPO FGF23 PTH VDR
11 secondary hyperparathyroidism of renal origin 29.3 ALB CASR EPO FGF23 PTH VDR
12 osteoporosis 29.0 ALB CASR FGF23 PTH VDR
13 calciphylaxis cutis 12.2
14 visceral calciphylaxis 12.0
15 systemic lupus erythematosus 10.2
16 lupus erythematosus 10.2
17 arthritis 10.2
18 poems syndrome 10.2
19 rheumatoid arthritis 10.2
20 calcinosis 10.1
21 hypocalciuric hypercalcemia, familial, type i 10.1 CASR PTH
22 thyroid crisis 10.1 ALB PTH
23 hypocalciuric hypercalcemia, familial, type ii 10.1 CASR PTH
24 hypocalciuric hypercalcemia, familial, type iii 10.1 CASR PTH
25 hypoparathyroidism, familial isolated 10.1 CASR PTH
26 familial isolated hyperparathyroidism 10.1 CASR PTH
27 temporal arteritis 10.1
28 pseudoxanthoma elasticum 10.1
29 protein s deficiency 10.1
30 ischemia 10.1
31 vasculitis 10.1
32 familial hypocalciuric hypercalcemia 10.1 CASR PTH
33 oncogenic osteomalacia 10.1 FGF23 PTH
34 hypophosphatemic rickets, x-linked dominant 10.1 FGF23 PTH
35 impaired renal function disease 10.1 FGF23 PTH
36 pulmonary alveolar microlithiasis 10.1 FGF23 PTH
37 arterial calcification of infancy 10.1 FGF23 NT5E
38 hypercalciuria, absorptive, 2 10.1 CASR VDR
39 metal metabolism disorder 10.1 CASR PTH
40 metaphyseal chondrodysplasia, jansen type 10.0 FGF23 PTH
41 parathyroid carcinoma 10.0 CASR PTH
42 xanthinuria, type i 10.0 CASR VDR
43 hypophosphatemic rickets with hypercalciuria, hereditary 10.0 FGF23 PTH
44 idiopathic hypercalciuria 10.0 CASR VDR
45 nephrolithiasis, calcium oxalate 10.0 CASR VDR
46 spondylosis 10.0 PTH VDR
47 salmonellosis 10.0 ALB VDR
48 splenic disease 10.0 ALB EPO
49 hypophosphatemic rickets, x-linked recessive 10.0 FGF23 VDR
50 fetal erythroblastosis 10.0 ALB EPO

Graphical network of the top 20 diseases related to Calciphylaxis:



Diseases related to Calciphylaxis
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Symptoms & Phenotypes for Calciphylaxis

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Human phenotypes related to Calciphylaxis:

59 32 (show all 12)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 skin ulcer 59 32 hallmark (90%) Very frequent (99-80%) HP:0200042
2 cellulitis 59 32 hallmark (90%) Very frequent (99-80%) HP:0100658
3 sepsis 59 32 hallmark (90%) Very frequent (99-80%) HP:0100806
4 gangrene 59 32 hallmark (90%) Very frequent (99-80%) HP:0100758
5 cutis marmorata 59 32 hallmark (90%) Very frequent (99-80%) HP:0000965
6 hyperphosphatemia 59 32 hallmark (90%) Very frequent (99-80%) HP:0002905
7 stage 5 chronic kidney disease 59 32 hallmark (90%) Very frequent (99-80%) HP:0003774
8 abnormality of skin physiology 59 32 hallmark (90%) Very frequent (99-80%) HP:0011122
9 secondary hyperparathyroidism 59 32 hallmark (90%) Very frequent (99-80%) HP:0000867
10 arterial calcification 59 32 hallmark (90%) Very frequent (99-80%) HP:0003207
11 ectopic ossification 59 32 hallmark (90%) Very frequent (99-80%) HP:0011986
12 abnormality of metabolism/homeostasis 59 Very frequent (99-80%)

GenomeRNAi Phenotypes related to Calciphylaxis according to GeneCards Suite gene sharing:

26
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Decreased melanin production GR00056-A 8.62 FGF23 NT5E

MGI Mouse Phenotypes related to Calciphylaxis:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 homeostasis/metabolism MP:0005376 9.87 ALB CASR EPO FGF23 NT5E PTH
2 cardiovascular system MP:0005385 9.85 EPO FGF23 NT5E PTH VDR
3 hematopoietic system MP:0005397 9.85 CASR EPO FGF23 NT5E PTH VDR
4 endocrine/exocrine gland MP:0005379 9.8 ALB CASR FGF23 PTH VDR
5 digestive/alimentary MP:0005381 9.73 ALB CASR FGF23 VDR
6 mortality/aging MP:0010768 9.63 ALB CASR EPO FGF23 PTH VDR
7 limbs/digits/tail MP:0005371 9.56 EPO FGF23 PTH VDR
8 renal/urinary system MP:0005367 9.35 ALB CASR FGF23 NT5E VDR
9 skeleton MP:0005390 9.02 CASR EPO FGF23 PTH VDR
Sources

Drugs & Therapeutics for Calciphylaxis

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Drugs for Calciphylaxis (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 28)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1 Protective Agents Phase 3,Phase 2
2 sodium thiosulfate Phase 3,Phase 2
3 Anti-Bacterial Agents Phase 3,Phase 2
4 Antitubercular Agents Phase 3,Phase 2
5 Antioxidants Phase 3,Phase 2
6 Pharmaceutical Solutions Phase 3,Phase 2
7 Calcium, Dietary Phase 3,Phase 2
8 Anti-Infective Agents Phase 3,Phase 2
9 Chelating Agents Phase 3,Phase 2
10 Antidotes Phase 3,Phase 2
11
Calcium Carbonate Approved, Investigational Phase 2 471-34-1
12 Antacids Phase 2
13 Anti-Ulcer Agents Phase 2
14 Bone Density Conservation Agents Phase 2
15 Gastrointestinal Agents Phase 2
16
Warfarin Approved 81-81-2 6691 54678486
17
Phytonadione Approved, Investigational Not Applicable 84-80-0 4812 5284607
18
Menadione Approved, Nutraceutical Not Applicable 58-27-5 4055
19 Vitamin K Not Applicable
20 Micronutrients Not Applicable
21 Menaquinone Not Applicable
22 Antifibrinolytic Agents Not Applicable
23 Vitamin K 1 Not Applicable
24 Coagulants Not Applicable
25 naphthoquinone Not Applicable
26 Vitamins Not Applicable
27 Trace Elements Not Applicable
28 Hemostatics Not Applicable

Interventional clinical trials:

(show all 16)
# Name Status NCT ID Phase Drugs
1 A Phase 3 Clinical Trial of Intravenous Sodium Thiosulfate in Acute Calciphylaxis Patients Recruiting NCT03150420 Phase 3 Sodium Thiosulfate;Placebo-Normal Saline
2 Randomized Double Blind Placebo Controlled Trial of Sodium Thiosulfate for the Treatment of Pain Associated With Calcific Uremic Arteriolopath Terminated NCT02527213 Phase 3 Sodium thiosulfate;Placebo
3 Phase 2 Study With SNF472 in Calciphylaxis Patients Completed NCT02790073 Phase 2 SNF472
4 Efficacy and Tolerance of Sodium Thiosulfate Injection After Ultrasound-guided Percutaneous Irrigation of Calcific Tendinopathy of the Rotator Cuff Completed NCT02538939 Phase 2 Sodium thiosulfate
5 Topical Sodium Thiosulfate and Fractional Carbon Dioxide Laser in Treating Dermatomyositis Associated Calcinosis Completed NCT01572844 Phase 2 Sodium thiosulfate
6 Two phosphAte taRGets in End-stage Renal Disease Trial (TARGET) Completed NCT01994733 Phase 2 Calcium carbonate ( Intensive phosphate control);Calcium carbonate (Liberalized phosphate control)
7 A Pilot Study to Evaluate Topical Sodium Thiosulfate Therapy for Calcinosis Cutis Withdrawn NCT01918904 Phase 2 Sodium thiosulfate;Placebo
8 Efficacy of Lanthanum Carbonate in Calciphylaxis Completed NCT01289626 Phase 1 lanthanum carbonate;Lanthanum for calciphylaxis
9 Calciphylaxis : Population, Risk Factors, Diagnostic Practice, Therapeutic and Outcome Unknown status NCT02854046
10 Pathophysiology of Martorell Hypertensive Ischemic Leg Ulcer (HYTILU) Unknown status NCT01578382
11 Partners Calciphylaxis Biobank Recruiting NCT03032835
12 European Calciphylaxis Registry Network Recruiting NCT02635373
13 The Early Administration of Sodium Thiosulfate Should Help to Reduce the Mortality of Dialysis Patients With Calciphylaxis, From a National Cohort Recruiting NCT03146793 Sodium Thiosulfate
14 Evaluation of Vitamin K Supplementation for Calcific Uremic Arteriolopathy Recruiting NCT02278692 Not Applicable
15 Pragmatic Randomised Trial of High Or Standard PHosphAte Targets in End-stage Kidney Disease (PHOSPHATE) Not yet recruiting NCT03573089 Not Applicable Liberal phosphate target;Intensive phosphate target
16 Observational Follow-up to ST-001 Calciphylaxis Pain Treatment With Intravenous Sodium Thiosulfate Terminated NCT03319914

Search NIH Clinical Center for Calciphylaxis

Cochrane evidence based reviews: calciphylaxis

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Genetic Tests for Calciphylaxis

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Anatomical Context for Calciphylaxis

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MalaCards organs/tissues related to Calciphylaxis:

41
Skin, Kidney, Skeletal Muscle, Bone, Breast, Liver, T Cells
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Publications for Calciphylaxis

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Articles related to Calciphylaxis:

(show top 50) (show all 757)
# Title Authors Year
1
Calciphylaxis in a patient without renal failure. ( 29957603 )
2018
2
Calciphylaxis as cutaneous marker of hyperparathyroidism and successful outcome with parathyroidectomy followed by hyperbaric oxygen therapy. ( 29451194 )
2018
3
Non Uremic Calciphylaxis Post Liver Transplantation: A Case Report and Literature Review of an Unusual Presentation of a Rare Disease. ( 29386500 )
2018
4
Calciphylaxis in end-stage liver and renal disease patients before and after transplant. ( 29714030 )
2018
5
Rheopheresis for Adjuvant Treatment in Resistant Calciphylaxis. ( 29430867 )
2018
6
Calciphylaxis. ( 29525245 )
2018
7
Calciphylaxis in a Patient With Non-Hodgkin Lymphoma: Case Report and Literature Review. ( 29478335 )
2018
8
Maggot therapy for calciphylaxis wound debridement complicated by bleeding. ( 29693082 )
2018
9
An Update on Calciphylaxis. ( 29808451 )
2018
10
Multifactorial aetiology for non-uremic calciphylaxis: a case report. ( 29915661 )
2018
11
A Rare Case of Calciphylaxis in an Orthotopic Liver Transplant Recipient with Acute Kidney Injury. ( 29633925 )
2018
12
Strengthening the Case for the Role of Thrombophilia in Calciphylaxis. ( 29955798 )
2018
13
Strengthening the Case for the Role of Thrombophilia in Calciphylaxis. ( 29955772 )
2018
14
Penile Calciphylaxis in a Patient with End-stage Renal Disease: A Case Report and Review of the Literature. ( 29756051 )
2018
15
Using Machine Learning Algorithms to Predict Risk for Development of Calciphylaxis in Patients with Chronic Kidney Disease. ( 29888059 )
2018
16
Calciphylaxis. ( 29719190 )
2018
17
Calciphylaxis with pseudoxanthoma elasticum-like changes: A case series. ( 29086985 )
2018
18
Necrosis of the glans penis secondary to calciphylaxis. ( 29740852 )
2018
19
Penile calciphylaxis in a patient on combined peritoneal dialysis and hemodialysis. ( 29594982 )
2018
20
A case report of pediatric calciphylaxis-a rare and potentially fatal under diagnosed condition. ( 29979398 )
2018
21
Digital gangrene and pneumatosis intestinalis associated with calciphylaxis. ( 29942902 )
2018
22
Bilateral breast calciphylaxis in a patient who survived earlier extensive tissue necrosis 5 years previously: A case report. ( 29775967 )
2018
23
3DTEE imaging of a descending aorta floating thrombus in a patient with calciphylaxis. ( 29226430 )
2018
24
Evaluating the validity of subclassifying warfarin-associated nonuremic calciphylaxis: a retrospective cohort study. ( 29336027 )
2018
25
Bone scintigraphy findings in calciphylaxis. ( 29904463 )
2018
26
Association Between Hypercoagulable Conditions and Calciphylaxis in Patients With Renal Disease: A Case-Control Study. ( 29238798 )
2018
27
Pancreatitis associated with calciphylaxis successfully treated with sodium thiosulfate. ( 29387772 )
2018
28
Non-Uremic Calciphylaxis. ( 29735007 )
2018
29
Anterior Ischemic Optic Neuropathy Due to Calciphylaxis. ( 29016409 )
2018
30
A Fatal Case of Non-Uremic Calciphylaxis: A Case Report and Literature Review. ( 29983410 )
2018
31
Cutaneous calcification in patients with kidney disease is not always calciphylaxis. ( 30031445 )
2018
32
Calciphylaxis. ( 30044942 )
2018
33
Calciphylaxis. ( 30048063 )
2018
34
Calciphylaxis. ( 30048064 )
2018
35
Calciphylaxis. ( 30048065 )
2018
36
Calciphylaxis. ( 30048066 )
2018
37
Calciphylaxis in Patients With Normal Renal Function: A Case Series and Systematic Review. ( 30060958 )
2018
38
Calciphylaxis in a dialysis patient successfully treated with high-dose vitamin K supplementation. ( 30094016 )
2018
39
A New Treatment for Calciphylaxis in Hemodialysis Patients? ( 30176667 )
2018
40
The Successful Use of Apixaban in Dialysis Patients with Calciphylaxis Who Require Anticoagulation: A Retrospective Analysis. ( 30176675 )
2018
41
Calciphylaxis in Patients With Preserved Renal Function: Worrisome Wounds. ( 30193670 )
2018
42
Nonuremic Calciphylaxis: Four Cases Associated with Autoimmune Diseases. ( 30207525 )
2018
43
Calciphylaxis: Case Presentation and Review of the Literature. ( 30216332 )
2018
44
Nonuremic Calciphylaxis. ( 30222629 )
2018
45
CRYSTALLINE RETINOPATHY AND RETINAL VASCULOPATHY IN CALCIFIC UREMIC ARTERIOLOPATHY (CALCIPHYLAXIS). ( 30226832 )
2018
46
Factors associated with false-negative pathologic diagnosis of calciphylaxis. ( 30280393 )
2018
47
Update on the Management of Calciphylaxis. ( 30304522 )
2018
48
Calciphylaxis in a kidney transplant patient. ( 30314631 )
2018
49
Management of calciphylaxis in a burn center: A case series and review of the literature. ( 30322738 )
2018
50
Non-uraemic calciphylaxis (NUC) postliver transplantation. ( 30361453 )
2018
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Variations for Calciphylaxis

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Expression for Calciphylaxis

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Search GEO for disease gene expression data for Calciphylaxis.
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Pathways for Calciphylaxis

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Pathways related to Calciphylaxis according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1
Ca, cAMP and Lipid Signaling 4
11.44 ALB CASR VDR
2
HIF-1-alpha transcription factor network 1
11.25 EPO NT5E
3
Differentiation Pathway 1
11.09 EPO NT5E
4
Transcription_Role of VDR in regulation of genes involved in osteoporosis 22
11.02 PTH VDR
5
Parathyroid hormone synthesis, secretion and action 37
10.95 CASR FGF23 PTH VDR
6
Development_Hedgehog and PTH signaling pathways in bone and cartilage development 22
10.91 PTH VDR
7
Osteoblast Signaling 1
10.4 FGF23 PTH
8
Vitamin D Metabolism 1
9.96 PTH VDR
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GO Terms for Calciphylaxis

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Biological processes related to Calciphylaxis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 regulation of signaling receptor activity GO:0010469 9.58 EPO FGF23 PTH
2 cellular calcium ion homeostasis GO:0006874 9.43 CASR PTH VDR
3 vitamin D metabolic process GO:0042359 9.37 FGF23 VDR
4 cellular response to vitamin D GO:0071305 9.32 CASR FGF23
5 phosphate ion homeostasis GO:0055062 9.16 FGF23 PTH
6 response to fibroblast growth factor GO:0071774 8.96 CASR PTH
7 positive regulation of vitamin D 24-hydroxylase activity GO:0010980 8.62 FGF23 VDR

Molecular functions related to Calciphylaxis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 drug binding GO:0008144 8.62 ALB CASR
Sources

Sources for Calciphylaxis

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3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
The MalaCards human disease database index: 1-9 A B C D E F G H I J K L M N O P Q R S T U V W X Y Z
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