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MCID: STR033
MIFTS: 26

Storm Syndrome

Categories: Rare diseases
Tissues Related diseases Publications Symptoms & Phenotypes Drugs
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Aliases & Classifications for Storm Syndrome

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MalaCards integrated aliases for Storm Syndrome:

Name: Storm Syndrome 56 52 71
Pleiotropic, Autosomal Dominant Disorder Affecting Connective Tissue 52

Characteristics:

OMIM:

56
Inheritance:
autosomal dominant


HPO:

31
storm syndrome:
Inheritance autosomal dominant inheritance


Classifications:

MalaCards categories:
Global: Rare diseases
See all MalaCards categories (disease lists)


External Ids:

OMIM 56 185069
MedGen 41 C1861452
UMLS 71 C1861452
Sources

Summaries for Storm Syndrome

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MalaCards based summary : Storm Syndrome, also known as pleiotropic, autosomal dominant disorder affecting connective tissue, is related to hemophagocytic lymphohistiocytosis and cytokine deficiency. The drugs Midazolam and Fentanyl have been mentioned in the context of this disorder. Affiliated tissues include skin, lung and myeloid, and related phenotypes are mitral valve calcification and fat malabsorption

More information from OMIM: 185069
Sources

Related Diseases for Storm Syndrome

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

(show all 18)
# Related Disease Score Top Affiliating Genes
1 hemophagocytic lymphohistiocytosis 11.5
2 cytokine deficiency 10.3
3 covid-19 10.3
4 severe acute respiratory syndrome 9.9
5 juvenile arthritis 9.8
6 juvenile rheumatoid arthritis 9.8
7 systemic onset juvenile idiopathic arthritis 9.8
8 hemophagocytic lymphohistiocytosis, familial, 1 9.7
9 middle east respiratory syndrome 9.7
10 retinal vein occlusion 9.7
11 post-traumatic stress disorder 9.7
12 central retinal vein occlusion 9.7
13 heart valve disease 9.7
14 pneumonia 9.7
15 acute stress disorder 9.7
16 connective tissue disease 9.7
17 macrophage activation syndrome 9.7
18 premature aging 9.7

Graphical network of the top 20 diseases related to Storm Syndrome:



Diseases related to Storm Syndrome
Sources

Symptoms & Phenotypes for Storm Syndrome

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Human phenotypes related to Storm Syndrome:

31 (show all 10)
# Description HPO Frequency HPO Source Accession
1 mitral valve calcification 31 HP:0004382
2 fat malabsorption 31 HP:0002630
3 aortic valve calcification 31 HP:0004380
4 mitral stenosis 31 HP:0001718
5 aortic valve stenosis 31 HP:0001650
6 excessive wrinkling of palmar skin 31 HP:0007605
7 polyarticular arthropathy 31 HP:0005195
8 sparse and thin eyebrow 31 HP:0000535
9 myxomatous mitral valve degeneration 31 HP:0004764
10 early progressive calcific cardiac valvular disease 31 HP:0006694

Symptoms via clinical synopsis from OMIM:

56
Cardiac:
mitral valve prolapse
calcific mitral stenosis
myxomatous mitral valve degeneration
early progressive calcific cardiac valvular disease
calcific aortic stenosis

Joints:
polyarticular arthropathy

Eyes:
no cataracts

Skin:
prematurely aged appearance
excessive wrinkling of palmar skin
taut skin over hands and face
no scleroderma-type changes

Hair:
loss of eyebrows and eyelashes
thinning and graying of scalp hair in adolescence

G I:
intestinal fat malabsorption

Clinical features from OMIM:

185069
Sources

Drugs & Therapeutics for Storm Syndrome

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

(show all 45)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Midazolam Approved, Illicit Phase 3 59467-70-8 4192
2
Fentanyl Approved, Illicit, Investigational, Vet_approved Phase 3 437-38-7 3345
3
Dexmedetomidine Approved, Vet_approved Phase 3 113775-47-6 5311068 68602
4
Propofol Approved, Investigational, Vet_approved Phase 3 2078-54-8 4943
5
Sevoflurane Approved, Vet_approved Phase 3 28523-86-6 5206
6
Colchicine Approved Phase 3 64-86-8 2833 6167
7
Lopinavir Approved Phase 3 192725-17-0 92727
8
Ritonavir Approved, Investigational Phase 3 155213-67-5 392622
9
Montelukast Approved Phase 3 158966-92-8 5281040
10 Anesthetics Phase 3
11 Anesthetics, General Phase 3
12 Platelet Aggregation Inhibitors Phase 3
13 Anesthetics, Inhalation Phase 3
14 Tubulin Modulators Phase 3
15 Antimitotic Agents Phase 3
16 Interleukin 1 Receptor Antagonist Protein Phase 3
17 Antirheumatic Agents Phase 3
18 Respiratory System Agents Phase 3
19 Hormone Antagonists Phase 3
20 Hormones Phase 3
21 Leukotriene Antagonists Phase 3
22 Anti-Asthmatic Agents Phase 3
23
Prazosin Approved Phase 2 19216-56-9 4893
24
Mavrilimumab Investigational Phase 2 1085337-57-0
25 Adrenergic alpha-Antagonists Phase 2
26 Adrenergic Agents Phase 2
27 Antihypertensive Agents Phase 2
28 Adrenergic Antagonists Phase 2
29 Adrenergic alpha-1 Receptor Antagonists Phase 2
30 Neurotransmitter Agents Phase 2
31 Anti-Inflammatory Agents Phase 2
32 Antioxidants Phase 2
33 Adjuvants, Immunologic Phase 2
34 Immunoglobulins Phase 2
35 Antibodies Phase 2
36 Antibodies, Monoclonal Phase 2
37
Heparin Approved, Investigational 9005-49-6 46507594 772
38
Lithium carbonate Approved 554-13-2
39
Enoxaparin Approved 9005-49-6 772
40
Aspirin Approved, Vet_approved 50-78-2 2244
41
Ivermectin Approved, Investigational, Vet_approved 70288-86-7 6474909
42 calcium heparin
43 Antiparasitic Agents
44 Antiviral Agents
45 Anti-Infective Agents

Interventional clinical trials:

(show all 22)
# Name Status NCT ID Phase Drugs
1 Sevoflurane Sedation in COVID-19 ARDS Patients to Reduce Lung Injury: a Randomized Controlled Trial Recruiting NCT04355962 Phase 3 Sevoflurane;Intravenous drug
2 The ECLA PHRI COLCOVID Trial Recruiting NCT04328480 Phase 3 Colchicine
3 Early Identification and Treatment of Cytokine Storm Syndrome in Covid-19 Not yet recruiting NCT04362111 Phase 3 Anakinra;Normal saline
4 Repurposing Montelukast for the Attenuation and Prophylaxis of Severe COVID-19 Symptoms: The COvid-19 Symptom MOntelukast (COSMO) Trial Not yet recruiting NCT04389411 Phase 3 Montelukast 10mg
5 Alpha-1 Adrenergic Receptor Antagonism to Prevent COVID-19 Cytokine Storm Syndrome and Acute Respiratory Distress Syndrome: A Randomized Study Comparing the Efficacy of Prazosin vs. Standard of Care for SARS-CoV-2 Infection Recruiting NCT04365257 Phase 2 Prazosin
6 A Randomized Placebo-controlled Safety and Dose-finding Study for the Use of the IL-6 Inhibitor Clazakizumab in Patients With Life-threatening COVID-19 Infection Recruiting NCT04363502 Phase 2 Clazakizumab;Placebo
7 A Randomized Placebo-controlled Safety and Dose-finding Study for the Use of the IL-6 Inhibitor Clazakizumab in Patients With Life-threatening COVID-19 Infection Recruiting NCT04343989 Phase 2 Clazakizumab 25 mg;Clazakizumab 12.5 mg
8 COlchicine in Moderate-severe Hospitalized Patients Before ARDS to Treat COVID-19 (the COMBAT-COVID-19 Pilot Study) Recruiting NCT04363437 Phase 2 Colchicine;Usual Care
9 Treatment of Serious and Critical Patients With COVID-19 With Tocilizumab Recruiting NCT04363853 Phase 2 Tocilizumab
10 Safety and Efficacy of Viusid and Asbrip in Hospitalized Patients in Teodoro Maldonado Carbo Specialty's Hospital Infected and Diagnosed by SARS-Cov-2 With COVID-19 Recruiting NCT04407182 Phase 2
11 A Randomized Placebo-Controlled Safety and Dose-Finding Study for the Use of the IL-6 Inhibitor Clazakizumab in Patients With Life-threatening COVID-19 Infection Not yet recruiting NCT04381052 Phase 2 Clazakizumab
12 A Randomized, Double Blind, Placebo-controlled Trial of Mavrilimumab for Acute Respiratory Failure Due to COVID-19 Pneumonia With Hyper-inflammation (the COMBAT-19 Trial) Not yet recruiting NCT04397497 Phase 2 Mavrilimumab;Placebo
13 A Phase I/II Randomized, Double Blinded, Placebo Trial to Evaluate the Safety and Potential Efficacy of Intravenous Infusion of Organicell Flow for the Treatment of Moderate to SARS Related to COVID-19 Infection vs Placebo Not yet recruiting NCT04384445 Phase 1, Phase 2
14 Novel Extracorporeal Treatment to Modulate Hyperinflammation in COVID-19 Patients Recruiting NCT04353674 Phase 1
15 Role of Innate Lymphoid Cells in the Immunosuppression of Septic Shock Unknown status NCT03297203
16 C5a Receptor Expression in Circulating Myeloid Cells in ARDS Secondary to COVID-19 Recruiting NCT04369820
17 VALIDATION OF A SEVERITY SCORE TO IDENTIFY PATIENTS ADMITTED FOR COVID-19 PNEUMONIA AT HIGH RISK FOR AN INTENSIVE APPROACH Recruiting NCT04372199
18 COVID-19 Hyperinflammation Syndrome (COV-HI): Protocol for a Rapidly Executed Cohort Study Recruiting NCT04385069
19 COVID-19 Inflammatory Blood Biomarkers for Clinical Management, Prognosis and Evaluation of Interventions Recruiting NCT04363008
20 A Prospective, Randomized, Controlled Study Assessing Vagus Nerve Stimulation in CoViD-19 Respiratory Symptoms (SAVIORII) Recruiting NCT04382391
21 EVALUATION of Ivermectin Aspirin Dexametasone and Enoxaparin as Treatment of covid19 Active, not recruiting NCT04425863 Ivermectin 5 MG/ML
22 Complications Risk Factors in Patients Hospitalized for COVID-19 Infection: Role of Proteins Electrophoresis Not yet recruiting NCT04414059

Search NIH Clinical Center for Storm Syndrome

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Genetic Tests for Storm Syndrome

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Anatomical Context for Storm Syndrome

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MalaCards organs/tissues related to Storm Syndrome:

40
Skin, Lung, Myeloid, T Cells
Sources

Publications for Storm Syndrome

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Articles related to Storm Syndrome:

(show top 50) (show all 64)
# Title Authors PMID Year
1
Is there a role for blood purification therapies targeting cytokine storm syndrome in critically severe COVID-19 patients? 61
32438839 2020
2
Cytokine storm syndrome in severe COVID-19. 61
32376400 2020
3
Tocilizumab for cytokine storm syndrome in COVID-19 pneumonia: an increased risk for candidemia? 61
32376396 2020
4
Treatment algorithm for COVID-19: a multidisciplinary point of view. 61
32472459 2020
5
COVID-19 and immunomodulation in IBD. 61
32303609 2020
6
Cytokine storm in COVID-19: pathogenesis and overview of anti-inflammatory agents used in treatment. 61
32474885 2020
7
Diacerein: A potential multi-target therapeutic drug for COVID-19. 61
32534337 2020
8
Severe cerebral involvement in adult-onset hemophagocytic lymphohistiocytosis. 61
32307298 2020
9
Anti-cytokine therapy in novel coronavirus disease (COVID-19) – the first administration of tocilizumab in Hungary at a department of infectology 61
32541085 2020
10
COVID-19 pandemic and troponin: indirect myocardial injury, myocardial inflammation or myocarditis? 61
32499236 2020
11
[Evolution and treatment of storm cytoquine syndrome associated to SARS-CoV-2 infection among octogenarians]. 61
32564984 2020
12
A Putative Role of de-Mono-ADP-Ribosylation of STAT1 by the SARS-CoV-2 Nsp3 Protein in the Cytokine Storm Syndrome of COVID-19. 61
32549200 2020
13
[Low dose lung radiotherapy for COVID-19-related cytokine storm syndrome: Why not?] 61
32389579 2020
14
Teamwork, A Novel Concept During an Infectious Pandemic? 61
32513790 2020
15
Therapeutic Temperature Modulation for a Critically Ill Patient with COVID-19. 61
32508069 2020
16
[Expert consensus on Corona Virus Disease 2019 with special blood purification technology]. 61
32521580 2020
17
Tocilizumab's efficacy in COVID-19 patients is determined by the presence of cytokine storm. 61
32568411 2020
18
Merit of an Ursodeoxycholic Acid Clinical Trial in COVID-19 Patients. 61
32575350 2020
19
COVID-19: Immunology and treatment options. 61
32353634 2020
20
COVID-19: Pathogenesis, cytokine storm and therapeutic potential of interferons. 61
32418715 2020
21
Weathering the COVID-19 storm: Lessons from hematologic cytokine syndromes. 61
32425294 2020
22
Lymphopenia in COVID-19: Therapeutic opportunities. 61
32458561 2020
23
Novel paediatric presentation of COVID-19 with ARDS and cytokine storm syndrome without respiratory symptoms. 61
32427161 2020
24
Effectiveness of corticoid pulses in patients with cytokine storm syndrome induced by SARS-CoV-2 infection. 61
32532461 2020
25
Profiling COVID-19 pneumonia progressing into the cytokine storm syndrome: Results from a single Italian Centre study on tocilizumab versus standard of care. 61
32425662 2020
26
COVID-19: Consider IL-6 receptor antagonist for the therapy of cytokine storm syndrome in SARS-CoV-2 infected patients. 61
32462717 2020
27
Profiling COVID-19 pneumonia progressing into the cytokine storm syndrome: Results from a single Italian Centre study on tocilizumab versus standard of care. 61
32570043 2020
28
Tocilizumab: A Therapeutic Option for the Treatment of Cytokine Storm Syndrome in COVID-19. 61
32482373 2020
29
Preventing cytokine storm syndrome in COVID-19 using α-1 adrenergic receptor antagonists. 61
32352407 2020
30
Extracorporeal Hemoadsorption: An Option for COVID-19 Associated Cytokine Storm Syndrome. 61
32453251 2020
31
An update on SARS-CoV-2/COVID-19 with particular reference to its clinical pathology, pathogenesis, immunopathology and mitigation strategies. 61
32479816 2020
32
The genetics of macrophage activation syndrome. 61
32291394 2020
33
Vitamin D can prevent COVID-19 infection-induced multiple organ damage. 61
32451597 2020
34
Oxygen-ozone therapy as adjuvant in the current emergency in SARS-COV-2 infection: a clinical study. 61
32462858 2020
35
Acute upper limb ischemia in a patient with COVID-19. 61
32405288 2020
36
SARS-CoV-2: Viral Mechanisms and Possible Therapeutic Targets - What to learn from Rheumatologists? 61
32451352 2020
37
Intravenous Anakinra for Macrophage Activation Syndrome May Hold Lessons for Treatment of Cytokine Storm in the Setting of Coronavirus Disease 2019. 61
32267072 2020
38
A new pharmacological approach based on remdesivir aerosolized administration on SARS-CoV-2 pulmonary inflammation: A possible and rational therapeutic application. 61
32562915 2020
39
Three novel prevention, diagnostic, and treatment options for COVID-19 urgently necessitating controlled randomized trials. 61
32534175 2020
40
Alpha-1 adrenergic receptor antagonists for preventing acute respiratory distress syndrome and death from cytokine storm syndrome. 61
32550250 2020
41
Efficacy of glutathione therapy in relieving dyspnea associated with COVID-19 pneumonia: A report of 2 cases. 61
32322478 2020
42
Absence of severe complications from SARS-CoV-2 infection in children with rheumatic diseases treated with biologic drugs. 61
32335512 2020
43
On the Alert for Cytokine Storm: Immunopathology in COVID-19. 61
32293098 2020
44
MSCs transplantation may be a potential therapeutic strategy for COVID-19 treatment. 61
32373992 2020
45
The Question of Whether to Remain on Therapy for Chronic Rheumatic Diseases in the Setting of the Covid-19 Pandemic. 61
32335514 2020
46
Does high cardiorespiratory fitness confer some protection against pro-inflammatory responses after infection by SARS-CoV-2? 61
32324968 2020
47
[Advances in the research of cytokine storm mechanism induced by Corona Virus Disease 2019 and the corresponding immunotherapies]. 61
32114747 2020
48
Genetic Deficiency of Interferon-γ Reveals Interferon-γ-Independent Manifestations of Murine Hemophagocytic Lymphohistiocytosis. 61
31400073 2020
49
Fighting the Host Reaction to SARS-COv-2 in Critically Ill Patients: The Possible Contribution of Off-Label Drugs. 61
32574268 2020
50
SARS-CoV-2 Infection and the Newborn. 61
32574287 2020
Sources

Variations for Storm Syndrome

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Expression for Storm Syndrome

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Search GEO for disease gene expression data for Storm Syndrome.
Sources

Pathways for Storm Syndrome

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GO Terms for Storm Syndrome

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Sources

Sources for Storm Syndrome

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3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 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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