MCID: DBN001
MIFTS: 54

Dubin-Johnson Syndrome malady

Categories: Genetic diseases, Rare diseases, Liver diseases, Metabolic diseases

Aliases & Classifications for Dubin-Johnson Syndrome

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Sources:
49OMIM, 10Disease Ontology, 11diseasecard, 45NIH Rare Diseases, 22GeneTests, 23Genetics Home Reference, 47Novoseek, 12DISEASES, 51Orphanet, 67UniProtKB/Swiss-Prot, 24GTR, 65UMLS, 36MeSH, 27ICD10, 42NCIt, 59SNOMED-CT, 28ICD10 via Orphanet, 37MESH via Orphanet, 66UMLS via Orphanet, 34MedGen, 61The Human Phenotype Ontology
See all MalaCards sources

Aliases & Descriptions for Dubin-Johnson Syndrome:

Name: Dubin-Johnson Syndrome 49 10 11 45 22 23 47 12 51 67
Jaundice, Chronic Idiopathic 23 36 65
Djs 45 23 67
Conjugated Hyperbilirubinemia 45 65
Chronic Idiopathic Jaundice 10 45
Hyperbilirubinemia Type 2 45 51
 
Dubin Johnson Syndrome 10 24
Hyperbilirubinemia Type Ii 22
Sprinz-Nelson Syndrome 51
Hyperbilirubinemia Ii 23
Dubin-Sprinz Disease 51
Hyperbilirubinemia 2 45

Characteristics:

Orphanet epidemiological data:

51
dubin-johnson syndrome:
Inheritance: Autosomal recessive; Age of onset: Adolescent,Adult; Age of death: normal life expectancy

HPO:

61
dubin-johnson syndrome:
Inheritance: autosomal recessive inheritance


Classifications:



External Ids:

OMIM49 237500
Disease Ontology10 DOID:12308
ICD1027 E80.6
MeSH36 D007566
NCIt42 C34741
SNOMED-CT59 44553005
Orphanet51 234
ICD10 via Orphanet28 E80.6
MESH via Orphanet37 D007566
UMLS via Orphanet66 C0022350
MedGen34 C0022350
UMLS65 C0022350, C0268307

Summaries for Dubin-Johnson Syndrome

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NIH Rare Diseases:45 Dubin-johnson syndrome is a type of hereditary hyperbilirubinemia with a relatively benign course. symptoms may include a yellowish color to the skin (jaundice), and a liver that is sometimes enlarged and tender.  the symptoms often do not present until puberty or adulthood. the syndrome interferes with the body's ability to move bilirubin from the liver. in most cases, treatment is not required.  last updated: 12/19/2008

MalaCards based summary: Dubin-Johnson Syndrome, also known as jaundice, chronic idiopathic, is related to hepatitis and cholestasis, and has symptoms including abnormality of urine homeostasis, abnormality of the gastric mucosa and abnormality of temperature regulation. An important gene associated with Dubin-Johnson Syndrome is ABCC2 (ATP Binding Cassette Subfamily C Member 2), and among its related pathways are Gemtuzumab ozogamicin Pathway, Pharmacokinetics/Pharmacodynamics and Doxorubicin Pathway, Pharmacokinetics. Affiliated tissues include liver, skin and eye, and related mouse phenotype homeostasis/metabolism.

Disease Ontology:10 A bilirubin metabolic disorder that involves elevated levels of unconjugated bilirubin without elevation of liver alanine transaminase and aspartate transaminase enzymes as a result of the deficient ability of hepatocytes to secrete conjugated bilirubin into the bile.

Genetics Home Reference:23 Dubin-Johnson syndrome is a condition characterized by jaundice, which is a yellowing of the skin and whites of the eyes. In most affected people jaundice appears during adolescence or early adulthood, although a few individuals have been diagnosed soon after birth. Jaundice is typically the only symptom of Dubin-Johnson syndrome, but some people also experience weakness, mild upper abdominal pain, nausea, and/or vomiting.

OMIM:49 Dubin-Johnson syndrome is an autosomal recessive disorder characterized by conjugated hyperbilirubinemia, an increase... (237500) more...

UniProtKB/Swiss-Prot:67 Dubin-Johnson syndrome: Autosomal recessive disorder characterized by conjugated hyperbilirubinemia, an increase in the urinary excretion of coproporphyrin isomer I, deposition of melanin-like pigment in hepatocytes, and prolonged retention of sulfobromophthalein, but otherwise normal liver function.

Related Diseases for Dubin-Johnson Syndrome

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Diseases related to Dubin-Johnson Syndrome via text searches within MalaCards or GeneCards Suite gene sharing:

(show all 42)
idRelated DiseaseScoreTop Affiliating Genes
1hepatitis10.7
2cholestasis10.5
3gilbert syndrome10.4
4hepatocellular carcinoma10.3
5factor vii deficiency10.3
6viral hepatitis10.3
7crigler-najjar syndrome, type ii10.3
8hyperbilirubinemia, rotor type, digenic10.3
9cardiomyopathy, dilated, 1o10.1ABCC2, SLCO1B1
10systemic lupus erythematosus10.1
11abetalipoproteinemia10.1
12atherosclerosis10.1
13hemangioma10.1
14liver cirrhosis10.1
15liver disease10.1
16thalassemia10.1
17cholelithiasis10.1
18cholecystolithiasis10.1
19portal vein thrombosis10.1
20choledocholithiasis10.1
21hereditary spherocytosis10.1
22congenital dyserythropoietic anemia10.1
23hepatitis c10.1
24neonatal jaundice10.1
25purpura10.1
26tuberculosis10.1
27cavernous hemangioma10.1
28pneumonia10.1
29lupus erythematosus10.1
30neurofibroma10.1
31dwarfism10.1
32efavirenz toxicity10.0ABCC2, SLCO1B1
33jaw cancer10.0ABCC2, SLCO1B1
34pseudoxanthoma elasticum, forme fruste9.7ABCC1, ABCC2
35auditory system disease9.7ABCC1, ABCC2, SLCO1B1
36acantholytic squamous cell skin carcinoma9.7ABCC1, ABCC3
37paranasal sinus sarcoma9.6ABCC1, ABCC2, ABCC3
38primary ciliary dyskinesia9.4ABCC2, ABCC3, SLCO1B1
39biliary dyskinesia9.3ABCC2, ABCC3, ABCC4
40pelvic varices9.2ABCC2, ABCC3, ABCC4
41alveolar periostitis8.8ABCC2, ABCC3, ABCC4, SLCO1B1
42progressive external ophthalmoplegia with mitochondrial dna deletions, autosomal dominant 37.5ABCC1, ABCC2, ABCC3, ABCC4, F7, SLCO1B1

Graphical network of the top 20 diseases related to Dubin-Johnson Syndrome:



Diseases related to dubin-johnson syndrome

Symptoms for Dubin-Johnson Syndrome

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Symptoms by clinical synopsis from OMIM:

237500

Clinical features from OMIM:

237500

Symptoms:

 51 (show all 10)
  • structural anomalies of the liver and the biliary tract
  • hepatitis/icterus/cholestasis
  • abnormal colour of the urine/cholic/dark urines
  • autosomal recessive inheritance
  • epigastralgia/heartburn/gastric/duodenal ulcer/gastritis
  • acute abdominal pain/colic
  • hepatomegaly/liver enlargement (excluding storage disease)
  • clotting/hemostasis disorders
  • fever/chilling
  • asthenia/fatigue/weakness

HPO human phenotypes related to Dubin-Johnson Syndrome:

(show all 9)
id Description Frequency HPO Source Accession
1 abnormality of urine homeostasis hallmark (90%) HP:0003110
2 abnormality of the gastric mucosa typical (50%) HP:0004295
3 abnormality of temperature regulation occasional (7.5%) HP:0004370
4 hepatomegaly occasional (7.5%) HP:0002240
5 abdominal pain occasional (7.5%) HP:0002027
6 abnormality of coagulation occasional (7.5%) HP:0001928
7 conjugated hyperbilirubinemia HP:0002908
8 biliary tract abnormality HP:0001080
9 jaundice HP:0000952

UMLS symptoms related to Dubin-Johnson Syndrome:


jaundice (not of newborn)

Drugs & Therapeutics for Dubin-Johnson Syndrome

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Drugs for Dubin-Johnson Syndrome (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

idNameStatusPhaseClinical TrialsCas NumberPubChem Id
1Protective Agents5651
2Liver Extracts3572
3
Bilirubin103635-65-45280352
Synonyms:
(4Z,15Z)-Bilirubin IXa
(Z,Z)-Bilirubin IXa
1,10,19,22,23,24-Hexahydro-2,7,13,17-tetramethyl-1,19-dioxo-3,18-divinyl-Biline-8,12-dipropionate
1,10,19,22,23,24-Hexahydro-2,7,13,17-tetramethyl-1,19-dioxo-3,18-divinyl-Biline-8,12-dipropionic acid
2,17-Diethenyl-1,10,19,22,23,24-hexahydro-3,7,13,18-tetramethyl-1,19-dioxo-21H-Biline-8,12-dipropanoate
2,17-Diethenyl-1,10,19,22,23,24-hexahydro-3,7,13,18-tetramethyl-1,19-dioxo-21H-Biline-8,12-dipropanoic acid
3-(2-((3-(2-Carboxyethyl)-4-methyl-5-((3-methyl-5-oxo-4-vinyl-1,5-dihydro-2H-pyrrol-2-ylidene)methyl)-1H-pyrrol-2-yl)methyl)-4-methyl-5-((4-methyl-5-oxo-3-vinyl-1,5-dihydro-2H-pyrrol-2-ylidene)methyl)-1H-pyrrol-3-yl)propanoate
3-(2-((3-(2-Carboxyethyl)-4-methyl-5-((3-methyl-5-oxo-4-vinyl-1,5-dihydro-2H-pyrrol-2-ylidene)methyl)-1H-pyrrol-2-yl)methyl)-4-methyl-5-((4-methyl-5-oxo-3-vinyl-1,5-dihydro-2H-pyrrol-2-ylidene)methyl)-1H-pyrrol-3-yl)propanoic acid
 
3-(2-((3-(2-Carboxyethyl)-4-methyl-5-[(Z)-(3-methyl-5-oxo-4-vinyl-1,5-dihydro-2H-pyrrol-2-ylidene)methyl]-1H-pyrrol-2-yl)methyl)-4-methyl-5-[(Z)-(4-methyl-5-oxo-3-vinyl-1,5-dihydro-2H-pyrrol-2-ylidene
3-[2-[[3-(2-Carboxyethyl)-5-[(3-ethenyl-4-methyl-5-oxo-pyrrol-2-ylidene)methyl]-4-methyl-1H-pyrrol-2-yl]methyl]-5-[(4-ethenyl-3-methyl-5-oxo-pyrrol-2-ylidene)methyl]-4-methyl-1H-pyrrol-3-yl]propanoate
3-[2-[[3-(2-Carboxyethyl)-5-[(3-ethenyl-4-methyl-5-oxo-pyrrol-2-ylidene)methyl]-4-methyl-1H-pyrrol-2-yl]methyl]-5-[(4-ethenyl-3-methyl-5-oxo-pyrrol-2-ylidene)methyl]-4-methyl-1H-pyrrol-3-yl]propanoic acid
3-[2-[[3-(2-Carboxyethyl)-5-[(Z)-(3-ethenyl-4-methyl-5-oxo-pyrrol-2-ylidene)methyl]-4-methyl-1H-pyrrol-2-yl]methyl]-5-[(Z)-(4-ethenyl-3-methyl-5-oxo-pyrrol-2-ylidene)methyl]-4-methyl-1H-pyrrol-3-yl]propanoate
3-[2-[[3-(2-Carboxyethyl)-5-[(Z)-(3-ethenyl-4-methyl-5-oxo-pyrrol-2-ylidene)methyl]-4-methyl-1H-pyrrol-2-yl]methyl]-5-[(Z)-(4-ethenyl-3-methyl-5-oxo-pyrrol-2-ylidene)methyl]-4-methyl-1H-pyrrol-3-yl]propanoic acid
Bilirubin
Bilirubin IX-alpha
Cholerythrin
Hematoidin
4Antioxidants2442

Interventional clinical trials:

idNameStatusNCT IDPhase
1Conjugated Hyperbilirubinemia and Pulse OximetryTerminatedNCT00741117

Search NIH Clinical Center for Dubin-Johnson Syndrome


Cochrane evidence based reviews: jaundice, chronic idiopathic

Genetic Tests for Dubin-Johnson Syndrome

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Genetic tests related to Dubin-Johnson Syndrome:

id Genetic test Affiliating Genes
1 Dubin-Johnson Syndrome22 ABCC2

Anatomical Context for Dubin-Johnson Syndrome

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MalaCards organs/tissues related to Dubin-Johnson Syndrome:

33
Liver, Skin, Eye, Lung, Endothelial, B cells, Thyroid

Animal Models for Dubin-Johnson Syndrome or affiliated genes

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MGI Mouse Phenotypes related to Dubin-Johnson Syndrome:

38
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053767.3ABCC1, ABCC2, ABCC3, ABCC4, F7, UROS

Publications for Dubin-Johnson Syndrome

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Articles related to Dubin-Johnson Syndrome:

(show top 50)    (show all 182)
idTitleAuthorsYear
1
Treatment for tuberculosis in a patient with Dubin-Johnson syndrome. (26264947)
2015
2
Dubin-Johnson syndrome with multiple liver cavernous hemangiomas: report of a familial case. (24228133)
2013
3
Novel large-scale deletion (whole exon 7) in the ABCC2 gene in a patient with the Dubin-Johnson syndrome. (19881259)
2009
4
Dubin-Johnson syndrome. (18460254)
2008
5
Dubin-Johnson syndrome--a clinicopathologic study of twenty cases. (17183837)
2006
6
Improvement of serum bilirubin levels after venesection in a patient with Dubin-Johnson syndrome and HCV-positive chronic liver disease. (15565411)
2004
7
Mutation analysis of the multidrug resistance protein 2 (MRP2) gene in a Japanese patient with Dubin-Johnson syndrome. (15519272)
2004
8
Hepatocellular carcinoma in a case of Dubin-Johnson syndrome treated successfully with a central bilateral segmentectomy. (15143928)
2004
9
A common Dubin-Johnson syndrome mutation impairs protein maturation and transport activity of MRP2 (ABCC2). (12388192)
2003
10
Homozygous mutation Arg768Trp in the ABC-transporter encoding gene MRP2/cMOAT/ABCC2 causes Dubin-Johnson syndrome in a Caucasian patient. (12942343)
2003
11
Association of Dubin-Johnson syndrome and portal vein thrombosis. (12118928)
2002
12
Identification and functional analysis of two novel mutations in the multidrug resistance protein 2 gene in Israeli patients with Dubin- Johnson syndrome. (11477083)
2001
13
Congenital extrahepatic portocaval shunt associated with hepatic hyperplastic nodules in a patient with Dubin-Johnson syndrome. (11029086)
2000
14
A splice mutation in the human canalicular multispecific organic anion transporter gene causes Dubin-Johnson syndrome. (9878557)
1998
15
Dubin-Johnson syndrome as a cause of neonatal jaundice: the importance of coproporphyrins investigation. (9729708)
1998
16
Defective hepatobiliary leukotriene elimination in patients with the Dubin-Johnson syndrome. (8737590)
1996
17
Neonatal cholestasis in two siblings: a variant of Dubin-Johnson syndrome? (8924312)
1995
18
Defective biliary excretion of epinephrine metabolites in mutant (TR-) rats: relation to the pathogenesis of black liver in the Dubin-Johnson syndrome and Corriedale sheep with an analogous excretory defect. (1592353)
1992
19
Hepatobiliary gammagraphy in the diagnosis of Dubin-Johnson syndrome in children]. (2249271)
1990
20
A new diagnostic approach to the Dubin-Johnson syndrome. (2296972)
1990
21
Dubin-Johnson syndrome presenting with neonatal cholestasis. (2400232)
1990
22
Melanosis coli--histochemical and immunohistochemical comparison of the pigments of melanosis coli and Dubin-Johnson syndrome. (2346038)
1990
23
Hepatobiliary function studies on Dubin-Johnson syndrome using 99mTc-pyridoxyl-methyl-tryptophan (PMT). (3393702)
1988
24
Further evidence that the pigment in the Dubin-Johnson syndrome is not melanin. (2853343)
1987
25
Dubin-Johnson syndrome. (A case report and review of literature). (7108821)
1982
26
The pigment in Dubin-Johnson syndrome. (467939)
1979
27
Dubin-Johnson syndrome in a neonate. (520367)
1979
28
Dubin-Johnson Syndrome: Report of two cases in a family. (739199)
1978
29
Uroporphyrinogen III cosynthetase in liver and blood in the Dubin-Johnson syndrome. (839109)
1977
30
Letter: Dubin-johnson syndrome. (1175877)
1975
31
Incidence of benign conjugated hyperbilirubinemia with pigment in the liver in Spis (Dubin-Johnson syndrome) II. Attempts at screening of carrier state by means of BSP test]. (1204005)
1975
32
Dubin-Johnson syndrome (report of 5 cases). (1232054)
1975
33
The inheritance of Dubin-Johnson syndrome. (4775129)
1973
34
The Dubin-Johnson syndrome and pregnancy. (4742659)
1973
35
Pregnancy, oral contraceptives, and chronic familial jaundice with predominantly conjugated hyperbilirubinemia (Dubin-Johnson syndrome). (5050316)
1972
36
Abnormal excretion of the isomers of urinary coproporphyrin by patients with Dubin-Johnson syndrome in Israel. (5539298)
1971
37
Dubin-Johnson syndrome in immature sheep. (5749221)
1968
38
Studies of chronic idiopathic jaundice (Dubin-Johnson syndrome). II. Evaluation of a large family with the trait. (5926936)
1966
39
Chronic idiopathic jaundice (Dubin--Johnson syndrome): (report of a case with a long follow-up). (5826676)
1965
40
DUBIN-JOHNSON SYNDROME WITH EXTRAHEPATIC PIGMENTATION IN A NEUROFIBROMA. REPORT OF A CASE. (14333143)
1965
41
On the relation between Dubin-Johnson syndrome and Rotor type. A case of Dubin-Johnson syndrome complicated with serum hepatitis. (5860916)
1965
42
HEPATITIS WITH RESULTING MOBILIZATION OF HEPATIC PIGMENT IN A PATIENT WITH DUBIN-JOHNSON SYNDROME. (14234683)
1964
43
ELECTRON MICROSCOPIC OBSERVATIONS ON LIVER CELLS OF CASES WITH DUBIN-JOHNSON SYNDROME. (14334655)
1964
44
THE DUBIN-JOHNSON SYNDROME. (14123788)
1964
45
Studies of chronic idiopathic jaundice (Dubin-Johnson syndrome). I. Demonstration of hepatic excretory defect. (13992285)
1963
46
Chronic idiopathic jaundice with hepatic pigmentation (Dubin-Johnson syndrome). (13695356)
1961
47
Dubin-Johnson syndrome: report of a case occurring in a Negro male. (13799514)
1960
48
A case of the Dubin-Johnson syndrome. (13835225)
1959
49
The Dubin-Johnson syndrome or chronic idiopathic jaundice; report of a case. (13546234)
1958
50

Variations for Dubin-Johnson Syndrome

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UniProtKB/Swiss-Prot genetic disease variations for Dubin-Johnson Syndrome:

67
id Symbol AA change Variation ID SNP ID
1ABCC2p.Arg768TrpVAR_000099rs56199535
2ABCC2p.Gln1382ArgVAR_010756
3ABCC2p.Arg1150HisVAR_013327
4ABCC2p.Ile1173PheVAR_013328

Clinvar genetic disease variations for Dubin-Johnson Syndrome:

5 (show all 13)
id Gene Variation Type Significance SNP ID Assembly Location
1ABCC2NM_000392.4(ABCC2): c.1967+1G> Asingle nucleotide variantPathogenicrs146405172GRCh37Chr 10, 101571360: 101571360
2ABCC2NM_000392.4(ABCC2): c.2901C> A (p.Tyr967Ter)single nucleotide variantPathogenicrs17222547GRCh37Chr 10, 101591385: 101591385
3ABCC2NM_000392.4(ABCC2): c.3741+1G> Asingle nucleotide variantPathogenicrs34937870GRCh37Chr 10, 101601851: 101601851
4ABCC2NM_000392.4(ABCC2): c.1013_1014delTGdeletionPathogenicrs864309675GRCh37Chr 10, 101559109: 101559110
5ABCC2NM_000392.4(ABCC2): c.3196C> T (p.Arg1066Ter)single nucleotide variantPathogenicrs72558199GRCh37Chr 10, 101591826: 101591826
6ABCC2ABCC2, 168-BP DEL, NT2272deletionPathogenic
7ABCC2NM_000392.4(ABCC2): c.2302C> T (p.Arg768Trp)single nucleotide variantPathogenicrs56199535GRCh37Chr 10, 101578577: 101578577
8ABCC2NM_000392.4(ABCC2): c.1815+2T> Asingle nucleotide variantPathogenicrs387906395GRCh37Chr 10, 101567988: 101567988
9ABCC2ABCC2, IVS18DS, T-C, +2single nucleotide variantPathogenic
10ABCC2NM_000392.4(ABCC2): c.4145A> G (p.Gln1382Arg)single nucleotide variantPathogenicrs72558202GRCh37Chr 10, 101605538: 101605538
11ABCC2NM_000392.4(ABCC2): c.1967+2T> Csingle nucleotide variantPathogenicrs387906396GRCh37Chr 10, 101571361: 101571361
12ABCC2NM_000392.4(ABCC2): c.3517A> T (p.Ile1173Phe)single nucleotide variantPathogenicrs72558201GRCh37Chr 10, 101595950: 101595950
13ABCC2NM_000392.4(ABCC2): c.3449G> A (p.Arg1150His)single nucleotide variantPathogenicrs72558200GRCh37Chr 10, 101595882: 101595882

Expression for genes affiliated with Dubin-Johnson Syndrome

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Search GEO for disease gene expression data for Dubin-Johnson Syndrome.

Pathways for genes affiliated with Dubin-Johnson Syndrome

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Pathways related to Dubin-Johnson Syndrome according to GeneCards Suite gene sharing:

(show all 17)
idSuper pathwaysScoreTop Affiliating Genes
19.7ABCC1, ABCC2
2
Show member pathways
9.7ABCC1, ABCC2
39.6ABCC2, ABCC3
49.6ABCC1, ABCC3
5
Show member pathways
9.6ABCC2, SLCO1B1
69.2ABCC1, ABCC2, ABCC3
7
Show member pathways
9.2ABCC1, ABCC2, SLCO1B1
8
Show member pathways
9.1ABCC2, ABCC3, SLCO1B1
9
Show member pathways
9.0ABCC2, ABCC3, ABCC4
10
Show member pathways
9.0ABCC2, ABCC4, SLCO1B1
118.6ABCC1, ABCC2, ABCC3, ABCC4
12
Show member pathways
8.6ABCC1, ABCC2, ABCC3, ABCC4
138.5ABCC2, ABCC3, ABCC4, SLCO1B1
148.5ABCC2, ABCC3, ABCC4, SLCO1B1
15
Show member pathways
8.1ABCC1, ABCC2, ABCC3, ABCC4, SLCO1B1
16
Show member pathways
8.1ABCC1, ABCC2, ABCC3, ABCC4, SLCO1B1
17
Show member pathways
8.1ABCC1, ABCC2, ABCC3, ABCC4, SLCO1B1

GO Terms for genes affiliated with Dubin-Johnson Syndrome

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Cellular components related to Dubin-Johnson Syndrome according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1membraneGO:00160208.1ABCC1, ABCC2, ABCC3, ABCC4, SLCO1B1

Biological processes related to Dubin-Johnson Syndrome according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1response to estrogenGO:00436279.8ABCC2, F7
2metabolic processGO:00081529.2ABCC1, ABCC2, ABCC3

Sources for Dubin-Johnson Syndrome

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2CDC
14ExPASy
15FDA
16FMA
24GTR
25HGMD
26HMDB
27ICD10
28ICD10 via Orphanet
29ICD9CM
30IUPHAR
31KEGG
34MedGen
36MeSH
37MESH via Orphanet
38MGI
41NCI
42NCIt
43NDF-RT
46NINDS
47Novoseek
49OMIM
50OMIM via Orphanet
54PubMed
55QIAGEN
60SNOMED-CT via Orphanet
64Tumor Gene Family of Databases
65UMLS
66UMLS via Orphanet