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MCID: CLC001
MIFTS: 52

Calciphylaxis

Categories: Rare diseases, Cardiovascular diseases, Bone diseases, Metabolic 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
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
SNOMED-CT 68 237900002
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 osteitis fibrosa and end stage renal failure. 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 sodium thiosulfate and Chelating Agents have been mentioned in the context of this disorder. Affiliated tissues include kidney, skin and skeletal muscle, and related phenotypes are secondary hyperparathyroidism and cutis marmorata

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 131)
# Related Disease Score Top Affiliating Genes
1 osteitis fibrosa 29.9 CASR PTH
2 end stage renal failure 28.9 ALB EPO PTH
3 hyperphosphatemia 28.0 CASR FGF23 PTH VDR
4 primary hyperparathyroidism 27.9 ALB CASR FGF23 PTH VDR
5 hyperparathyroidism 27.7 CASR EPO FGF23 PTH VDR
6 kidney disease 26.7 ALB CASR EPO FGF23 PTH VDR
7 chronic kidney failure 26.7 ALB CASR EPO FGF23 PTH VDR
8 secondary hyperparathyroidism of renal origin 26.6 ALB CASR EPO FGF23 PTH VDR
9 calciphylaxis cutis 12.0
10 visceral calciphylaxis 11.9
11 sialolithiasis 10.3 ALB CASR
12 hypocalciuric hypercalcemia, familial, type iii 10.3 CASR PTH
13 hypoparathyroidism, familial isolated 10.3 CASR PTH
14 hypocalciuric hypercalcemia, familial, type ii 10.3 CASR PTH
15 familial hypocalciuric hypercalcemia 10.3 CASR PTH
16 oncogenic osteomalacia 10.3 FGF23 PTH
17 impaired renal function disease 10.2 FGF23 PTH
18 pulmonary alveolar microlithiasis 10.2 FGF23 PTH
19 arterial calcification of infancy 10.2 FGF23 NT5E
20 hypercalciuria, absorptive, 2 10.2 CASR VDR
21 peritonitis 10.2
22 hypocalcemia, autosomal dominant 1 10.1 CASR VDR
23 metaphyseal chondrodysplasia, jansen type 10.1 FGF23 PTH
24 systemic lupus erythematosus 10.1
25 lupus erythematosus 10.1
26 parathyroid carcinoma 10.1 CASR PTH
27 xanthinuria, type i 10.1 CASR VDR
28 poems syndrome 10.1
29 hypophosphatemic rickets with hypercalciuria, hereditary 10.0 FGF23 PTH
30 hypophosphatemic rickets, x-linked recessive 10.0 FGF23 VDR
31 arthritis 10.0
32 idiopathic hypercalciuria 10.0 CASR VDR
33 nephrolithiasis, calcium oxalate 10.0 CASR VDR
34 rheumatoid arthritis 10.0
35 calcinosis 10.0
36 spondylosis 10.0 PTH VDR
37 splenic disease 10.0 ALB EPO
38 pseudoxanthoma elasticum 10.0
39 protein s deficiency 10.0
40 fetal erythroblastosis 9.9 ALB EPO
41 pure red-cell aplasia 9.9 ALB EPO
42 hypersplenism 9.9 ALB EPO
43 rh isoimmunization 9.9 ALB EPO
44 temporal arteritis 9.9
45 ischemia 9.9
46 vasculitis 9.9
47 renal hypertension 9.9 ALB EPO
48 hypophosphatemia 9.9 FGF23 PTH
49 analbuminemia 9.9 ALB EPO
50 arteries, anomalies of 9.8

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 secondary hyperparathyroidism 59 32 hallmark (90%) Very frequent (99-80%) HP:0000867
2 cutis marmorata 59 32 hallmark (90%) Very frequent (99-80%) HP:0000965
3 hyperphosphatemia 59 32 hallmark (90%) Very frequent (99-80%) HP:0002905
4 arterial calcification 59 32 hallmark (90%) Very frequent (99-80%) HP:0003207
5 stage 5 chronic kidney disease 59 32 hallmark (90%) Very frequent (99-80%) HP:0003774
6 abnormality of skin physiology 59 32 hallmark (90%) Very frequent (99-80%) HP:0011122
7 ectopic ossification 59 32 hallmark (90%) Very frequent (99-80%) HP:0011986
8 cellulitis 59 32 hallmark (90%) Very frequent (99-80%) HP:0100658
9 gangrene 59 32 hallmark (90%) Very frequent (99-80%) HP:0100758
10 sepsis 59 32 hallmark (90%) Very frequent (99-80%) HP:0100806
11 skin ulcer 59 32 hallmark (90%) Very frequent (99-80%) HP:0200042
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 cardiovascular system MP:0005385 9.83 PTH VDR EPO FGF23 NT5E
2 hematopoietic system MP:0005397 9.8 CASR VDR EPO FGF23 NT5E PTH
3 homeostasis/metabolism MP:0005376 9.8 CASR VDR EPO FGF23 NT5E PTH
4 endocrine/exocrine gland MP:0005379 9.77 ALB PTH CASR VDR FGF23
5 digestive/alimentary MP:0005381 9.71 ALB CASR VDR FGF23
6 limbs/digits/tail MP:0005371 9.46 VDR EPO FGF23 PTH
7 renal/urinary system MP:0005367 9.35 CASR VDR FGF23 NT5E ALB
8 skeleton MP:0005390 9.02 CASR VDR EPO FGF23 PTH
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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 sodium thiosulfate Phase 3,Phase 2
2 Chelating Agents Phase 3,Phase 2
3 Anti-Bacterial Agents Phase 3,Phase 2
4 Pharmaceutical Solutions Phase 3,Phase 2
5 Antidotes Phase 3,Phase 2
6 Anti-Infective Agents Phase 3,Phase 2
7 Antioxidants Phase 3,Phase 2
8 Protective Agents Phase 3,Phase 2
9 Antitubercular Agents Phase 3,Phase 2
10 Calcium, Dietary Phase 3,Phase 2
11
Calcium Carbonate Approved, Investigational Phase 2 471-34-1
12 Gastrointestinal Agents Phase 2
13 Antacids Phase 2
14 Anti-Ulcer Agents Phase 2
15 Bone Density Conservation Agents Phase 2
16
Warfarin Approved 81-81-2 6691 54678486
17
Menadione Approved, Nutraceutical Not Applicable 58-27-5 4055
18
Phytonadione Approved, Investigational, Nutraceutical Not Applicable 84-80-0 4812 5284607
19 Micronutrients Not Applicable
20 Coagulants Not Applicable
21 Hemostatics Not Applicable
22 Trace Elements Not Applicable
23 Vitamin K 1 Not Applicable
24 Vitamins Not Applicable
25 Antifibrinolytic Agents Not Applicable
26 naphthoquinone Nutraceutical Not Applicable
27 Vitamin K Nutraceutical Not Applicable
28 Menaquinone Nutraceutical 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 Observational Follow-up to ST-001 Calciphylaxis Pain Treatment With Intravenous Sodium Thiosulfate Recruiting NCT03319914
11 Partners Calciphylaxis Biobank Recruiting NCT03032835
12 European Calciphylaxis Registry Network Recruiting NCT02635373
13 Pathophysiology of Martorell Hypertensive Ischemic Leg Ulcer (HYTILU) Recruiting NCT01578382
14 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
15 Evaluation of Vitamin K Supplementation for Calcific Uremic Arteriolopathy Recruiting NCT02278692 Not Applicable
16 Pragmatic Randomised Trial of High Or Standard PHosphAte Targets in End-stage Kidney Disease (PHOSPHATE) Not yet recruiting NCT03573089 Not Applicable Intensive phosphate target;Liberal phosphate target

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
Kidney, Skin, Skeletal Muscle, Bone, Breast, Liver, Lung
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Publications for Calciphylaxis

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Articles related to Calciphylaxis:

(show top 50) (show all 659)
# 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
Pseudoxanthoma elasticum-like changes in nonuremic calciphylaxis: Case series and brief review of a helpful diagnostic clue. ( 28869660 )
2017
31
Vitamin K-Dependent Carboxylation of Matrix Gla Protein Influences the Risk of Calciphylaxis. ( 28049648 )
2017
32
Ecto-5' -Nucleotidase CD73 (NT5E), vitamin D receptor and FGF23 gene polymorphisms may play a role in the development of calcific uremic arteriolopathy in dialysis patients - Data from the German Calciphylaxis Registry. ( 28212442 )
2017
33
Calciphylaxis in the Setting of Alcoholic Cirrhosis: Case Report and Literature Review. ( 28589153 )
2017
34
Increased Bone Morphogenetic Protein Signaling in the Cutaneous Vasculature of Patients with Calciphylaxis. ( 29130990 )
2017
35
Clinical Assessment of Warfarin Therapy in Patients with Maintenance Dialysis-Clinical Efficacy, Risks and Development of Calciphylaxis. ( 29147170 )
2017
36
The Role of Bone Scintigraphy with Single-photon Emission Computed Tomography-computed Tomography in the Diagnosis and Evaluation of Calciphylaxis. ( 28553189 )
2017
37
The Signs Aligned: Nonuremic Calciphylaxis. ( 28601542 )
2017
38
Management of a Complex Peristomal Calciphylaxis: A Case Study. ( 28682855 )
2017
39
Early clinical presentations and progression of calciphylaxis. ( 28436018 )
2017
40
Calciphylaxis in hemodialysed patients: Diagnostic value of calcifications in cutaneous biopsy. ( 28498603 )
2017
41
Calciphylaxis in a nondialysis patient treated with sodium thiosulfate and high dose of oxygen. ( 29043146 )
2017
42
Penile Calciphylaxis: The Use of Radiological Investigations in the Management of a Rare and Challenging Condition. ( 28507910 )
2017
43
Analysis of the German DRG data for livedoid vasculopathy and calciphylaxis. ( 28252815 )
2017
44
Acenocoumarol as a risk factor for calciphylaxis: a feature clinicians should be aware of. ( 28522774 )
2017
45
Calciphylaxis: Overview for the vascular nurse. ( 29153231 )
2017
46
A 33year-old woman with calciphylaxis, diabetic kidney disease and abdominal pain. ( 28506812 )
2017
47
Parathyroidectomy in Hyperparathyroidism-Associated Calciphylaxis in End-Stage Renal Disease Should be Prompt and Radical - a Case Report with Two Original Therapeutic Modifications and Successful Outcome. ( 28976876 )
2017
48
Calciphylaxis of the Postmenopausal Female Breast: An Uncommonly Encountered Mimic of Carcinoma. ( 28852578 )
2017
49
Calciphylaxis after parathyroidectomy. ( 29064176 )
2017
50
Underreported use of palliative care and patient reported outcome measures to address reduced Quality of Life in calciphylaxis patients: A systematic review. ( 28580642 )
2017
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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
Sources

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 9.54 EPO FGF23 PTH
2 cellular calcium ion homeostasis GO:0006874 9.33 CASR PTH VDR
3 vitamin D metabolic process GO:0042359 9.32 FGF23 VDR
4 cellular response to vitamin D GO:0071305 9.26 CASR FGF23
5 response to fibroblast growth factor GO:0071774 8.96 CASR PTH
6 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
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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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