MCID: CLC001
MIFTS: 46

Calciphylaxis malady

Categories: Rare diseases, Cardiovascular diseases, Bone diseases, Metabolic diseases

Aliases & Classifications for Calciphylaxis

Aliases & Descriptions for Calciphylaxis:

Name: Calciphylaxis 12 50 56 42 14 69
Idiopathic Calciphylaxis 50

Characteristics:

Orphanet epidemiological data:

56
calciphylaxis
Inheritance: Not applicable; Prevalence: 1-9/100000 (Europe); Age of onset: Adult; Age of death: adult;

Classifications:



External Ids:

Disease Ontology 12 DOID:4734
MeSH 42 D002115
NCIt 47 C84607
SNOMED-CT 64 237900002
Orphanet 56 ORPHA280062
ICD10 via Orphanet 34 E83.5
UMLS via Orphanet 70 C0006666
UMLS 69 C0006666

Summaries for Calciphylaxis

NIH Rare Diseases : 50 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. last updated: 2/7/2012

MalaCards based summary : Calciphylaxis, also known as idiopathic calciphylaxis, is related to artery disease and calcinosis, and has symptoms including skin ulcer, cellulitis and sepsis. An important gene associated with Calciphylaxis is ALB (Albumin), and among its related pathways/superpathways are Mesenchymal Stem Cell Differentiation Pathways and Lineage-specific Markers and Primary immunodeficiency. The drugs Sodium thiosulfate and Anti-Bacterial Agents have been mentioned in the context of this disorder. Affiliated tissues include skin, kidney and skeletal muscle, and related phenotypes are homeostasis/metabolism and renal/urinary system

Wikipedia : 71 Calciphylaxis, or calcific uremic arteriolopathy (CUA), is a syndrome of calcification of the blood... more...

Related Diseases for Calciphylaxis

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

(show top 50) (show all 265)
id Related Disease Score Top Affiliating Genes
1 artery disease 29.6 CD40LG CD79A
2 calcinosis 29.3 ALB CD40LG CD79A PTH
3 endocarditis 29.3 CD40LG CD79A
4 arteriosclerosis 29.2 CD40LG CD79A
5 calciphylaxis cutis 11.9
6 visceral calciphylaxis 11.8
7 hyperparathyroidism 10.2
8 mesenchymal cell neoplasm 10.2 CASR PTH
9 waardenburg syndrome, type 4b 10.2 CASR PTH
10 familial chronic myelocytic leukemia-like syndrome 10.2 CASR PTH
11 atopic dermatitis 6 10.2 ALB CD79A
12 schneiderian carcinoma 10.2 ALB CD79A
13 infertility due to extratesticular cause 10.2 ALB CD79A
14 congenital nystagmus 10.2 ALB CD79A
15 male genital organ stricture 10.2 ALB CD79A
16 stone in bladder diverticulum 10.2 ALB FN1
17 gastric antral vascular ectasia 10.2 CASR PTH
18 tarp syndrome 10.2 ALB CD79A
19 acute cervicitis 10.2 ALB CD79A
20 ciliary discoordination, due to random ciliary orientation 10.2 ALB CD79A
21 epidermolysis bullosa, junctional, herlitz type 10.2 CASR PTH
22 mikulicz disease 10.1 ALB CD79A
23 tinea unguium 10.1 CASR PTH
24 cowper gland carcinoma 10.1 CASR PTH
25 weill-marchesani syndrome 10.1 CASR PTH
26 malignant hyperthermia susceptibility 1 10.1 ALB CD79A
27 opioid abuse 10.1 CASR PTH
28 hemiplegia 10.1 ALB CD79A
29 secernentea infections 10.1 CD79A PTH
30 hyperlipoproteinemia type iii 10.1 CD79A PTH
31 bronchiolitis 10.1 ALB CD79A
32 bladder sarcoma 10.1 ALB CD79A
33 bile duct adenocarcinoma 10.1 ALB CD40LG
34 estrogen excess 10.1 ALB CD40LG
35 freiberg's disease 10.1 ALB CD40LG
36 pre-eclampsia 10.1 CASR PTH
37 inner ear disease 10.1 ALB CD40LG
38 dyskeratosis congenita 10.1 ALB CD40LG
39 indian tick typhus 10.1 CASR PTH
40 chronic inflammatory demyelinating polyneuritis 10.1 ALB CD40LG
41 vulvar sarcoma 10.1 ALB CD40LG
42 bladder diverticulum 10.1 ALB CD40LG
43 generalized dystonia 10.1 ALB CD40LG
44 hereditary alpha tryptasemia syndrome 10.1 ALB CD40LG
45 breast apocrine carcinoma in situ 10.0 ALB CD79A
46 pes anserinus tendinitis or bursitis 10.0 ALB CASR PTH
47 benign deep fibrous histiocytoma 10.0 ALB CD40LG
48 bile duct mucoepidermoid carcinoma 10.0 ALB CASR PTH
49 peritonitis 10.0
50 parathyroid carcinoma 10.0 CASR PTH

Graphical network of the top 20 diseases related to Calciphylaxis:



Diseases related to Calciphylaxis

Symptoms & Phenotypes for Calciphylaxis

Human phenotypes related to Calciphylaxis:

56 32 (show all 12)
id Description HPO Frequency Orphanet Frequency HPO Source Accession
1 skin ulcer 56 32 Very frequent (99-80%) HP:0200042
2 cellulitis 56 32 Very frequent (99-80%) HP:0100658
3 sepsis 56 32 Very frequent (99-80%) HP:0100806
4 gangrene 56 32 Very frequent (99-80%) HP:0100758
5 cutis marmorata 56 32 Very frequent (99-80%) HP:0000965
6 stage 5 chronic kidney disease 56 32 Very frequent (99-80%) HP:0003774
7 hyperphosphatemia 56 32 Very frequent (99-80%) HP:0002905
8 secondary hyperparathyroidism 56 32 Very frequent (99-80%) HP:0000867
9 arterial calcification 56 32 Very frequent (99-80%) HP:0003207
10 abnormality of skin physiology 56 32 Very frequent (99-80%) HP:0011122
11 ectopic ossification 56 32 Very frequent (99-80%) HP:0011986
12 abnormality of metabolism/homeostasis 56 Very frequent (99-80%)

MGI Mouse Phenotypes related to Calciphylaxis:

44
id Description MGI Source Accession Score Top Affiliating Genes
1 homeostasis/metabolism MP:0005376 9.43 ALB CASR CD40LG CD79A FN1 PTH
2 renal/urinary system MP:0005367 8.92 ALB CASR CD40LG CD79A

Drugs & Therapeutics for Calciphylaxis

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

(show all 28)
id Name Status Phase Clinical Trials Cas Number PubChem Id
1 Sodium thiosulfate Approved Phase 3,Phase 2
2 Anti-Bacterial Agents Phase 3,Phase 2
3 Antidotes Phase 3,Phase 2
4 Anti-Infective Agents Phase 3,Phase 2
5 Antioxidants Phase 3,Phase 2
6 Antitubercular Agents Phase 3,Phase 2
7 Calcium, Dietary Phase 3,Phase 2
8 Chelating Agents Phase 3,Phase 2
9 Pharmaceutical Solutions Phase 3,Phase 2
10 Protective Agents Phase 3,Phase 2
11
Calcium carbonate Approved 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
Menadione Approved, Nutraceutical 58-27-5 4055
18
Phytonadione Approved, Nutraceutical 84-80-0 4812 5284607
19 Antifibrinolytic Agents
20 Coagulants
21 Hemostatics
22 Micronutrients
23 Trace Elements
24 Vitamin K 1
25 Vitamins
26 Menaquinone Nutraceutical
27 naphthoquinone Nutraceutical
28 Vitamin K Nutraceutical

Interventional clinical trials:

(show all 14)
id Name Status NCT ID Phase
1 A Phase 3 Clinical Trial of Intravenous Sodium Thiosulfate in Acute Calciphylaxis Patients Recruiting NCT03150420 Phase 3
2 Randomized Double Blind Placebo Controlled Trial of Sodium Thiosulfate for the Treatment of Pain Associated With Calcific Uremic Arteriolopath Terminated NCT02527213 Phase 3
3 Topical Sodium Thiosulfate and Fractional Carbon Dioxide Laser in Treating Dermatomyositis Associated Calcinosis Completed NCT01572844 Phase 2
4 Two phosphAte taRGets in End-stage Renal Disease Trial (TARGET) Completed NCT01994733 Phase 2
5 Phase 2 Study With SNF472 in Calciphylaxis Patients Recruiting NCT02790073 Phase 2
6 Efficacy and Tolerance of Sodium Thiosulfate Injection After Ultrasound-guided Percutaneous Irrigation of Calcific Tendinopathy of the Rotator Cuff Recruiting NCT02538939 Phase 2
7 A Pilot Study to Evaluate Topical Sodium Thiosulfate Therapy for Calcinosis Cutis Withdrawn NCT01918904 Phase 2
8 Efficacy of Lanthanum Carbonate in Calciphylaxis Completed NCT01289626 Phase 1
9 Partners Calciphylaxis Biobank Recruiting NCT03032835
10 European Calciphylaxis Registry Network Recruiting NCT02635373
11 Pathophysiology of Martorell Hypertensive Ischemic Leg Ulcer (HYTILU) Recruiting NCT01578382
12 The Early Administration of Sodium Thiosulfate Should Help to Reduce the Mortality of Dialysis Patients With Calciphylaxis, From a National Cohort Recruiting NCT03146793
13 Evaluation of Vitamin K Supplementation for Calcific Uremic Arteriolopathy Recruiting NCT02278692
14 Calciphylaxis : Population, Risk Factors, Diagnostic Practice, Therapeutic and Outcome Not yet recruiting NCT02854046

Search NIH Clinical Center for Calciphylaxis

Cochrane evidence based reviews: calciphylaxis

Genetic Tests for Calciphylaxis

Anatomical Context for Calciphylaxis

MalaCards organs/tissues related to Calciphylaxis:

39
Skin, Kidney, Skeletal Muscle, Breast, Bone, Liver, Lung

Publications for Calciphylaxis

Articles related to Calciphylaxis:

(show top 50) (show all 600)
id Title Authors Year
1
Vitamin K-Dependent Carboxylation of Matrix Gla Protein Influences the Risk of Calciphylaxis. ( 28049648 )
2017
2
Intralesional Sodium Thiosulfate to Treat Calciphylaxis. ( 26739119 )
2016
3
Calciphylaxis in a nondialysis patient treated with sodium thiosulfate and high dose of oxygena8c. ( 27841148 )
2016
4
Case Report of a Patient Undergoing Peritoneal Dialysis with Encapsulating Peritoneal Sclerosis Superimposed With Calciphylaxis. ( 27781116 )
2016
5
Multimodal Treatment of Calciphylaxis With Sodium Thiosulfate, Alprostadil, and Hyperbaric Oxygen Therapy. ( 27208908 )
2016
6
POEMS syndrome and calciphylaxis: an unrecognized cause of abnormal small vessel calcification. ( 27068711 )
2016
7
Non-uraemic calciphylaxis - an unexpected differential diagnosis for a necrotic ulcer. ( 27037400 )
2016
8
Literature review in the treatment of calciphylaxis: A case with uncontrolled and severe secondary hyperparathyroidism. ( 26958335 )
2016
9
Treatment with sodium thiosulfate in calciphylaxis of topical active renal transplant patient. ( 27595513 )
2016
10
Regression of vascular calcification in a patient with calciphylaxis. ( 27142498 )
2016
11
Warfarin skin necrosis mimicking calciphylaxis in a patient with secondary hyperparathyroidism undergoing peritoneal dialysis. ( 27069859 )
2016
12
Resolution of Calciphylaxis After Urgent Kidney Transplantation in 3 Patients With End-Stage Kidney Failure. ( 27826606 )
2016
13
Lack of evidence does not justify neglect: how can we address unmet medical needs in calciphylaxis? ( 27005994 )
2016
14
Calciphylaxis in peritoneal dialysis patients: a single center cohort study. ( 27698566 )
2016
15
We Do Too Many Parathyroidectomies for Calciphylaxis. ( 27082830 )
2016
16
Calciphylaxis, a rare disease with fatal outcome. ( 26819179 )
2016
17
Calciphylaxis of the Breast: A Case Report and Literature Review. ( 27332900 )
2016
18
Advanced-stage calciphylaxis: Think before you punch. ( 27505874 )
2016
19
Skin lesions in calciphylaxis. ( 27269760 )
2016
20
Letter to the editor submitted in response to "Sodium thiosulfate as a treatment for calciphylaxis: A case series". ( 27687504 )
2016
21
Survival, Risk Factors, and Effect of Treatment in 101 Patients With Calciphylaxis. ( 27712637 )
2016
22
Calciphylaxis - A case study in a patient with Maori heritage. ( 27443245 )
2016
23
Calciphylaxis following acute renal injury: a case and literature review. ( 27462491 )
2016
24
Multimodal treatment of calcific uraemic arteriolopathy (calciphylaxis): a case series. ( 26798470 )
2016
25
Calciphylaxis: Controversies in Pathogenesis, Diagnosis and Treatment. ( 26897281 )
2016
26
Calciphylaxis of the breast: a rare metabolic complication of kidney disease. ( 27507694 )
2016
27
Successful Management of Calciphylaxis in a Kidney Transplant Patient: Case Report. ( 27500261 )
2016
28
Warfarin-induced calciphylaxis in patients with normal renal function. ( 27321367 )
2016
29
The cutaneous expression of vitamin K-dependent and other osteogenic proteins in calciphylaxis stratified by clinical features and warfarin use: A case control study. ( 27646745 )
2016
30
Sodium thiosulfate as a treatment for calciphylaxis: A case series. ( 27051974 )
2016
31
The Successful Treatment of Calciphylaxis with Sodium Thiosulfate and Hyperbaric Oxygen in a Non-dialyzed Patient with Chronic Kidney Disease. ( 27432100 )
2016
32
Warfarin-induced calciphylaxis. ( 26783010 )
2016
33
Calciphylaxis: A Disease of Pannicular Thrombosis. ( 27712638 )
2016
34
Calciphylaxis and Martorell Hypertensive Ischemic Leg Ulcer: Same Pattern - One Pathophysiology. ( 27622522 )
2016
35
Bilateral optic neuropathy, acral gangrene and visceral ischaemia as a rare presentation of calciphylaxis: A case report. ( 27686313 )
2016
36
Development of multiorganic calciphylaxis during teriparatide, vitamin D, and calcium treatment. ( 27010647 )
2016
37
Minimal Change Nephrotic Syndrome Sequentially Complicated by Acute Kidney Injury and Painful Skin Ulcers due to Calciphylaxis. ( 27853075 )
2016
38
The Relationship Between Obesity and Calciphylaxis: A Review of the Literature. ( 26779700 )
2016
39
Calcific uraemic arteriolopathy (calciphylaxis): data from a large nationwide registry. ( 26908770 )
2016
40
Ischemic myopathy revealing systemic calciphylaxis. ( 27935078 )
2016
41
Successful Treatment of Lung Calciphylaxis With Sodium Thiosulfate in a Patient With Sickle Cell Disease: A Case Report. ( 26871829 )
2016
42
Case Report of Calciphylaxis Secondary to Calcium and Vitamin D3 Supplementation. ( 27566435 )
2016
43
A Fatal Case of Calciphylaxis in a Patient with Systemic Lupus Erythematosus and Normal Renal Function. ( 26834255 )
2016
44
Usefulness of Endovascular Therapy for Penile Gangrene Secondary to Calciphylaxis: A Case Report. ( 26122413 )
2015
45
Multiple sites of calciphylaxis in a patient with chronic renal failure. ( 25758887 )
2015
46
Clinical features, therapy, and outcome of calciphylaxis in patients with end-stage renal disease on renal replacement therapy: Case series. ( 25818988 )
2015
47
Successful treatment of calciphylaxis with pamidronate. ( 25599740 )
2015
48
Calciphylaxis in a patient affected by rheumatoid arthritis, chronic renal failure, and hyperparathyroidism: a case report. ( 26064073 )
2015
49
Calciphylaxis and the persistence of medical misinformation in the era of Google. ( 26276773 )
2015
50
Sodium Thiosulfate: Calciphylaxis. ( 27621504 )
2015

Variations for Calciphylaxis

Expression for Calciphylaxis

Search GEO for disease gene expression data for Calciphylaxis.

Pathways for Calciphylaxis

Pathways related to Calciphylaxis according to GeneCards Suite gene sharing:

id Super pathways Score Top Affiliating Genes
1 10.78 CD79A FN1 PTH
2 10.72 CD40LG CD79A
3 10.47 CD40LG FN1

GO Terms for Calciphylaxis

Cellular components related to Calciphylaxis according to GeneCards Suite gene sharing:

id Name GO ID Score Top Affiliating Genes
1 platelet alpha granule lumen GO:0031093 8.62 ALB FN1

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

id Name GO ID Score Top Affiliating Genes
1 B cell differentiation GO:0030183 9.16 CD40LG CD79A
2 B cell proliferation GO:0042100 8.96 CD40LG CD79A
3 response to fibroblast growth factor GO:0071774 8.62 CASR PTH

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

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

Sources for Calciphylaxis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 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 MedGen
42 MeSH
43 MESH via Orphanet
44 MGI
46 NCI
47 NCIt
48 NDF-RT
51 NINDS
52 Novoseek
54 OMIM
55 OMIM via Orphanet
59 PubMed
60 QIAGEN
65 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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