MCID: MTH007
MIFTS: 46

Methemoglobinemia

Categories: Rare diseases, Blood diseases, Genetic diseases

Aliases & Classifications for Methemoglobinemia

Summaries for Methemoglobinemia

MalaCards based summary : Methemoglobinemia is related to hereditary methemoglobinemia and acquired methemoglobinemia, and has symptoms including cyanosis An important gene associated with Methemoglobinemia is CYB5R3 (Cytochrome B5 Reductase 3), and among its related pathways/superpathways are Amino sugar and nucleotide sugar metabolism and Cytochrome P450 - arranged by substrate type. The drugs Epinephrine and Mannitol have been mentioned in the context of this disorder. Affiliated tissues include lung, heart and kidney.

Related Diseases for Methemoglobinemia

Diseases in the Methemoglobinemia family:

Methemoglobinemia Type Iv Congenital Methemoglobinemia
Hereditary Methemoglobinemia Acquired Methemoglobinemia

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

(show top 50) (show all 107)
# Related Disease Score Top Affiliating Genes
1 hereditary methemoglobinemia 33.9 CYB5A CYB5R3 HBG2
2 acquired methemoglobinemia 33.4 CYB5R3 G6PD HBG2
3 congenital methemoglobinemia 32.2 CYB5A CYB5R3 G6PD HBG2 SOD1
4 glucosephosphate dehydrogenase deficiency 29.6 G6PD HBG2 HP
5 anemia, nonspherocytic hemolytic, due to g6pd deficiency 29.3 G6PD HP
6 methemoglobinemia due to deficiency of methemoglobin reductase 12.6
7 methemoglobinemia, beta-globin type 12.5
8 methemoglobinemia type iv 12.3
9 koro 10.2 CYP3A4 G6PD
10 hemoglobin h disease 10.2 G6PD HBG2
11 cyanosis, transient neonatal 10.2
12 sickle cell disease 10.2 G6PD HBG2
13 isoniazid toxicity 10.1 CYP2E1 NAT2
14 hemolytic anemia 10.1
15 acetaminophen metabolism 10.1 CYP2E1 CYP3A4
16 drug-induced hepatitis 10.1 CYP2E1 NAT2
17 methemoglobin reductase deficiency 10.1
18 sulfhemoglobinemia 10.0
19 meningococcal infection 10.0 G6PD SOD1
20 senile cataract 10.0 G6PD SOD1
21 burns 10.0
22 diarrhea 10.0
23 blood group, i system 9.9
24 aging 9.9
25 malaria 9.9
26 leukemia 9.9
27 pneumonia 9.9
28 blackwater fever 9.9 G6PD HP
29 alcoholic liver cirrhosis 9.9 CYP2E1 NAT2
30 leprosy 3 9.9
31 streptococcal group a invasive disease 9.9
32 sickle cell anemia 9.8 G6PD HBG2
33 gilbert syndrome 9.8 G6PD HP
34 hyperphenylalaninemia 9.8 G6PD SOD1
35 alcohol dependence 9.8
36 pyruvate dehydrogenase e3-binding protein deficiency 9.8
37 episodic pain syndrome, familial, 1 9.8
38 metabolic encephalomyopathic crises, recurrent, with rhabdomyolysis, cardiac arrhythmias, and neurodegeneration 9.8
39 thalassemia 9.8
40 lymphoma 9.8
41 respiratory failure 9.8
42 heart disease 9.8
43 vaginitis 9.8
44 pharyngitis 9.8
45 cervicitis 9.8
46 lupus erythematosus 9.8
47 molluscum contagiosum 9.8
48 hypoxia 9.8
49 bilirubin metabolic disorder 9.7 G6PD HP
50 congenital hemolytic anemia 9.7 CYB5R3 G6PD HP

Graphical network of the top 20 diseases related to Methemoglobinemia:



Diseases related to Methemoglobinemia

Symptoms & Phenotypes for Methemoglobinemia

UMLS symptoms related to Methemoglobinemia:


cyanosis

Drugs & Therapeutics for Methemoglobinemia

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

(show top 50) (show all 54)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Epinephrine Approved, Vet_approved Phase 4 51-43-4 5816
2
Mannitol Approved, Investigational Phase 4 69-65-8 6251 453
3
Nitric Oxide Approved Phase 4,Phase 3 10102-43-9 145068
4
Prilocaine Approved Phase 4 721-50-6 4906
5 Racepinephrine Approved Phase 4 329-65-7
6
Methylene blue Approved, Investigational Phase 4,Phase 1 61-73-4
7 diuretics Phase 4
8 Epinephryl borate Phase 4
9 Natriuretic Agents Phase 4
10 Pharmaceutical Solutions Phase 4,Phase 2,Phase 1
11 Anti-Asthmatic Agents Phase 3
12 Autonomic Agents Phase 3
13 Bronchodilator Agents Phase 3
14 Neurotransmitter Agents Phase 3
15 Peripheral Nervous System Agents Phase 3
16 Respiratory System Agents Phase 3
17 Antioxidants Phase 3
18 Endothelium-Dependent Relaxing Factors Phase 3
19 Protective Agents Phase 3
20 Vasodilator Agents Phase 3
21
Primaquine Approved Phase 1 90-34-6 4908
22
Dapsone Approved, Investigational Phase 1 80-08-0 2955
23
Folic Acid Approved, Nutraceutical, Vet_approved Phase 1 59-30-3 6037
24
leucovorin Approved, Nutraceutical Phase 1 58-05-9 143 6006
25 Anti-Infective Agents Phase 1
26 Antimalarials Phase 1
27 Antiparasitic Agents Phase 1
28 Antiprotozoal Agents Phase 1
29 Anti-Bacterial Agents Phase 1
30 Folic Acid Antagonists Phase 1
31 Vitamin B Complex Phase 1
32 Folate Nutraceutical Phase 1
33 Vitamin B9 Nutraceutical Phase 1
34
Artemether Approved 71963-77-4 68911 119380
35
Lumefantrine Approved 82186-77-4 6437380
36
Lidocaine Approved, Vet_approved 137-58-6 3676
37
Miconazole Approved, Investigational, Vet_approved 22916-47-8 4189
38 Artemether-lumefantrine combination
39 Anesthetics
40 Adrenergic Agents
41 Adrenergic Agonists
42 Adrenergic alpha-Agonists
43 Adrenergic beta-Agonists
44 Anesthetics, Local
45 Anti-Arrhythmia Agents
46 Central Nervous System Depressants
47 Diuretics, Potassium Sparing
48 Mydriatics
49 Sodium Channel Blockers
50 Vasoconstrictor Agents

Interventional clinical trials:

(show all 19)

# Name Status NCT ID Phase Drugs
1 Evaluation of Ureteral Patency in the Post-indigo Carmine Era Completed NCT02677623 Phase 4 Pyridium;Sodium Fluorescein;Mannitol
2 MEthylene Blue In Patients With Acquired Methemoglobinemia Not yet recruiting NCT03395223 Phase 4 Methylene Blue
3 Evaluation of the Efficacy Safety and Tolerability of Nitric Oxide Given Intermittently Via Inhalation to Subjects With Bronchiolitis Recruiting NCT03053388 Phase 3 Nitric Oxide
4 Inhaled Nitric Oxide Study for Respiratory Failure in Newborns Terminated NCT00005776 Phase 3 Inhaled nitric oxide;Placebo
5 Early Inhaled Nitric Oxide for Respiratory Failure in Newborns Terminated NCT00005773 Phase 3 Inhaled Nitric Oxide;Standard iNO therapy
6 Nitrite Mediated Cardioprotection During Coronary Artery Bypass Surgery Unknown status NCT01098409 Phase 2 sodium nitrite;0.9% sodium chloride
7 Topical Sodium Nitrite in Sickle Cell Disease and Leg Ulcers Recruiting NCT02863068 Phase 2 Topical Sodium Nitrite
8 Sodium Nitrite in Lung Transplant Patients to Minimize the Risk of Pulmonary Graft Dysfunction Terminated NCT01715883 Phase 2 Sodium Nitrite
9 Pharmacokinetics and Distribution of Dapsone in Leucocytes After Single-dose and Multiple-dose Administration Completed NCT02493283 Phase 1 Dapsone single dose;Dapsone multiple dose
10 Safety, Tolerability, and Pharmacokinetic Study of Methylene Blue Following a 1 mg/kg Intravenous Dose in Healthy Adults Completed NCT02478281 Phase 1 Methylene Blue
11 Escalating Multiple-Dose Safety and Tolerance of WR 6026 Hydrochloride in HIV-Infected Subjects Completed NCT00000740 Phase 1 Sitamaquine
12 Pharmacokinetic and in Vitro Transmission Blocking Activities Study of Primaquine Compare to Methylene Blue in Healthy Volunteer Both G6PD Normal and G6PD Deficiency Completed NCT01668433 Phase 1 Regimens (Primaquine, Methylene blue)
13 Nitrite Infusion in Healthy Volunteers Completed NCT00103025 Phase 1 Sodium Nitrite;Sodium Nitrite Injection
14 Study of Methemoglobin as a Biomarker of Tissue Hypoxia During Acute Hemodilution in Heart Surgery Patients Unknown status NCT01883713
15 Safety and Efficacy of Primaquine for P. Vivax Unknown status NCT01837992 Primaquine;delayed primaquine
16 Methemoglobinemia After Liposuction - Diagnostic by Pulse Oximetry and Blood Gas Analysis. Completed NCT01766999
17 Methemoglobinemia in Young Patients With Hematologic Cancer or Aplastic Anemia Treated With Dapsone Completed NCT00993694 chemotherapy;dapsone
18 Methemoglobin Levels in Generally Anesthetized Pediatric Dental Patients Receiving Local Anesthetics Completed NCT01402869 4% prilocaine plain;2% Lidocaine with 1:100,000 epinephrine
19 Relapses in Plasmodium Ovale and Efficacy of Artemether-lumefantrine for Mixed Species and Non-falciparum Malaria Completed NCT02528279 Coartem

Search NIH Clinical Center for Methemoglobinemia

Inferred drug relations via UMLS 69 / NDF-RT 47 :


Cochrane evidence based reviews: methemoglobinemia

Genetic Tests for Methemoglobinemia

Anatomical Context for Methemoglobinemia

MalaCards organs/tissues related to Methemoglobinemia:

38
Lung, Heart, Kidney, Skin, Testes, Brain, Liver

Publications for Methemoglobinemia

Articles related to Methemoglobinemia:

(show top 50) (show all 709)
# Title Authors Year
1
Familial Congenital Methemoglobinemia in Pomeranian Dogs Caused by a Missense Variant in the NADH-Cytochrome B5 Reductase Gene. ( 29356095 )
2018
2
Methemoglobinemia in a Case of Paint Thinner Intoxication, Treated Successfully with Vitamin C. ( 29258784 )
2018
3
The first case of methemoglobinemia associated with omalizumab. ( 29339124 )
2018
4
Congenital methemoglobinemia type II in a 5-year-old boy. ( 29375859 )
2018
5
Role of Automated Therapeutic Red Cell Exchange in the Setting of Acute Methemoglobinemia: Our Experience. ( 29398814 )
2018
6
A Case of Methemoglobinemia Successfully Treated with Hyperbaric Oxygenation Monotherapy. ( 28838565 )
2017
7
Anesthetic management of congenital methemoglobinemia in an emergency cesarean section. ( 29109650 )
2017
8
Plasmapheresis in severe methemoglobinemia following occupational exposure. ( 28259531 )
2017
9
Brisk clinical response to erythrocytapheresis in a G6PD-deficient patient with rasburicase-induced methemoglobinemia. ( 28370399 )
2017
10
Methemoglobinemia After Paracetamol Ingestion: A Case Report. ( 29268071 )
2017
11
Successful treatment of NO-induced methemoglobinemia with low-dose vitamin C. ( 28438052 )
2017
12
Rasburicase-induced methemoglobinemia: The eyes do not see what the mind does not know. ( 28345492 )
2017
13
Methemoglobinemia Due to Antifreeze Ingestion. ( 29141168 )
2017
14
Laundry Blues: a case of methemoglobinemia with laundry detergent and Tylenol ingestion. ( 28911028 )
2017
15
Case 6: Skin Discoloration, Methemoglobinemia, and Acute Interstitial Nephritis in a 15-year-old Girl. ( 28765205 )
2017
16
Letter in response to "Acquired methemoglobinemia after hydroxocobalamin administration in a patient with burns and inhalation injury". ( 29164951 )
2017
17
Enzymopenic Congenital Methemoglobinemia in Children of the Republic of Sakha (Yakutia). ( 27879543 )
2017
18
Methemoglobinemia incidence after the application of lidocaine for small surgical procedures. ( 29171648 )
2017
19
Benzocaine-Induced Methemoglobinemia. ( 29102098 )
2017
20
Rasburicase-Induced Methemoglobinemia in a Patient with Glucose-6-Phosphate Dehydrogenase Deficiency. ( 28078984 )
2017
21
Cotrimoxazole-induced Methemoglobinemia. ( 28984267 )
2017
22
Acquired methemoglobinemia after hydroxocobalamin administration in a patient with burns and inhalation injury. ( 28969436 )
2017
23
Inhaled nitric oxide, methemoglobinemia, and route of delivery. ( 28757849 )
2017
24
Methylene Blue Induced Methemoglobinemia with Acute Kidney Injury in a Glucose-6-Phosphate Dehydrogenase-deficient Patient. ( 29217886 )
2017
25
Rare cause of delirium and hypoxemia after coronary bypass surgery: transdermal lidocaine patch-associated methemoglobinemia. ( 28875384 )
2017
26
Central Nervous System Symptoms Due to Transient Methemoglobinemia in a Child With G6PD Deficiency. ( 27879542 )
2017
27
Prilocaine-induced Methemoglobinemia. ( 28903852 )
2017
28
Upward trend of dapsone-induced methemoglobinemia in renal transplant communitya8c. ( 28699887 )
2017
29
Long-term Treatment with Methylene Blue in a Dog with Hereditary Methemoglobinemia Caused by Cytochrome b5 Reductase Deficiency. ( 28963729 )
2017
30
Hemolysis and Methemoglobinemia in a Child With Membrane Oxygenator on Biventricular Assist Device. ( 28782159 )
2017
31
Primaquine in Plasma and Methemoglobinemia in Patients with Malaria Due to Plasmodium vivax in the Brazilian Amazon Basin. ( 28440745 )
2017
32
Methemoglobinemia: Living with Dormant Devil. ( 28428705 )
2017
33
Methemoglobinemia Caused by Indoxacarb Poisoning. ( 28641724 )
2017
34
A Case of Cyanosis: Dapsone Induced Methemoglobinemia and Coexisting Pulmonary Embolism. ( 28418946 )
2017
35
A Case Report of Benzocaine-Induced Methemoglobinemia. ( 28803519 )
2017
36
Congenital Methemoglobinemia: It is Time for National Level Registry System. ( 28991125 )
2017
37
Transient Methemoglobinemia in three Neonates due to Maternal Pudendal Anesthesia. ( 29185409 )
2017
38
Acquired Methemoglobinemia - A Sporadic Holi Disaster. ( 28667718 )
2017
39
An Unusual Case of Neonatal Methemoglobinemia. ( 28285300 )
2017
40
A puzzling case of methemoglobinemia. ( 28370418 )
2017
41
An Uncommon Complication With Use of Topical Local Anesthetic Agents: Methemoglobinemia. ( 26741955 )
2016
42
Topical Benzocaine and Methemoglobinemia. ( 27754990 )
2016
43
Methemoglobinemia due to indoxacarb poisoning: A rare manifestation. ( 27728599 )
2016
44
Methemoglobinemia Hemotoxicity of Some Antimalarial 8-Aminoquinoline Analogues and Their Hydroxylated Derivatives: Density Functional Theory Computation of Ionization Potentials. ( 27223244 )
2016
45
Methemoglobinemia Following Monolinuron Ingestion: A Case Report in a Child. ( 27253655 )
2016
46
Difference of the clinical course and outcome between dapsone-induced methemoglobinemia and other toxic-agent-induced methemoglobinemia. ( 27412886 )
2016
47
Role of ascorbic acid in the treatment of methemoglobinemia. ( 27857990 )
2016
48
Benzocaine-Induced Methemoglobinemia: A Case Report. ( 26957886 )
2016
49
Methemoglobinemia from frozen-dried mudfish contaminated with sodium nitrite. ( 27367334 )
2016
50
A Rare but Important Clinical Presentation of Induced Methemoglobinemia. ( 27625731 )
2016

Variations for Methemoglobinemia

Expression for Methemoglobinemia

Search GEO for disease gene expression data for Methemoglobinemia.

Pathways for Methemoglobinemia

Pathways related to Methemoglobinemia according to KEGG:

36
# Name Kegg Source Accession
1 Amino sugar and nucleotide sugar metabolism hsa00520

Pathways related to Methemoglobinemia according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1
Show member pathways
12.64 CYB5A CYB5R3 CYB5R4 CYP2E1 CYP3A4 NAT2
2
Show member pathways
11.27 CYP2E1 CYP3A4
3
Show member pathways
11.22 CYP2E1 CYP3A4
4
Show member pathways
11.15 CYP2E1 CYP3A4
5 10.91 CYP2E1 CYP3A4
6 10.7 CYP2E1 CYP3A4
7 10.56 CYP2E1 CYP3A4
8
Show member pathways
10.55 CYP2E1 CYP3A4 NAT2
9 9.4 CYB5A CYB5R3 G6PD

GO Terms for Methemoglobinemia

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

# Name GO ID Score Top Affiliating Genes
1 endoplasmic reticulum GO:0005783 9.65 CYB5A CYB5R3 CYB5R4 CYP2E1 CYP3A4
2 intracellular membrane-bounded organelle GO:0043231 9.62 CYB5A CYP2E1 CYP3A4 G6PD
3 endoplasmic reticulum membrane GO:0005789 9.55 CYB5A CYB5R3 CYB5R4 CYP2E1 CYP3A4
4 hemoglobin complex GO:0005833 8.96 CYB5R3 HBG2
5 organelle membrane GO:0031090 8.8 CYB5A CYP2E1 CYP3A4

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

(show all 13)
# Name GO ID Score Top Affiliating Genes
1 steroid metabolic process GO:0008202 9.58 CYB5R3 CYP2E1 CYP3A4
2 response to ethanol GO:0045471 9.54 CYP2E1 G6PD SOD1
3 response to hydrogen peroxide GO:0042542 9.52 HP SOD1
4 glutathione metabolic process GO:0006749 9.51 G6PD SOD1
5 cholesterol biosynthetic process GO:0006695 9.49 CYB5R3 G6PD
6 response to antibiotic GO:0046677 9.48 CYB5R4 SOD1
7 drug metabolic process GO:0017144 9.46 CYP2E1 CYP3A4
8 superoxide metabolic process GO:0006801 9.43 CYB5R4 SOD1
9 L-ascorbic acid metabolic process GO:0019852 9.32 CYB5A CYB5R3
10 heterocycle metabolic process GO:0046483 9.26 CYP2E1 CYP3A4
11 xenobiotic metabolic process GO:0006805 9.26 CYB5R3 CYP2E1 CYP3A4 NAT2
12 oxidation-reduction process GO:0055114 9.17 CYB5A CYB5R3 CYB5R4 CYP2E1 CYP3A4 G6PD
13 monoterpenoid metabolic process GO:0016098 9.16 CYP2E1 CYP3A4

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

# Name GO ID Score Top Affiliating Genes
1 enzyme binding GO:0019899 9.62 CYB5A CYP2E1 CYP3A4 SOD1
2 iron ion binding GO:0005506 9.54 CYP2E1 CYP3A4 HBG2
3 monooxygenase activity GO:0004497 9.48 CYP2E1 CYP3A4
4 steroid hydroxylase activity GO:0008395 9.46 CYP2E1 CYP3A4
5 antioxidant activity GO:0016209 9.43 HP SOD1
6 oxidoreductase activity GO:0016491 9.43 CYB5R3 CYB5R4 CYP2E1 CYP3A4 G6PD SOD1
7 oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen, reduced flavin or flavoprotein as one donor, and incorporation of one atom of oxygen GO:0016712 9.4 CYP2E1 CYP3A4
8 oxygen binding GO:0019825 9.33 CYP2E1 CYP3A4 HBG2
9 cytochrome-b5 reductase activity, acting on NAD(P)H GO:0004128 9.26 CYB5R3 CYB5R4
10 heme binding GO:0020037 9.02 CYB5A CYB5R4 CYP2E1 CYP3A4 HBG2

Sources for Methemoglobinemia

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 FMA
27 GO
28 GTR
29 HGMD
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 MedGen
41 MeSH
42 MESH via Orphanet
43 MGI
45 NCI
46 NCIt
47 NDF-RT
50 NINDS
51 Novoseek
53 OMIM
54 OMIM via Orphanet
58 PubMed
60 QIAGEN
65 SNOMED-CT via HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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