Orphanet Rare Disease Ontology

Last uploaded: December 13, 2023
Preferred Name

Nelson syndrome

Synonyms
Definitions

A rare, acquired, endocrine disease characterized by the triad of diffuse skin and mucosa hyperpigmentation, markedly elevated serum adrenocorticotropin (ACTH) levels and an enlarging corticotroph adenoma, which manifest following total bilateral adrenalectomy performed for the treatment of Cushing's disease. Additionally, patients may present with headaches, visual field defects, cranial nerve palsy, pituitary apoplexy, diabetes insipidus, panhypopituitarism, and, occasionally, paraovarian or paratesticular tumors.

ID

http://www.orpha.net/ORDO/Orphanet_199244

definition

A rare, acquired, endocrine disease characterized by the triad of diffuse skin and mucosa hyperpigmentation, markedly elevated serum adrenocorticotropin (ACTH) levels and an enlarging corticotroph adenoma, which manifest following total bilateral adrenalectomy performed for the treatment of Cushing's disease. Additionally, patients may present with headaches, visual field defects, cranial nerve palsy, pituitary apoplexy, diabetes insipidus, panhypopituitarism, and, occasionally, paraovarian or paratesticular tumors.

definition_citation

Orphanet

expertlink

https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=199244

hasDbXref

ICD-10:E24.1

MedDRA:10028913

UMLS:C0027577

MeSH:D009347

ICD-11:5A70.3

label

Nelson syndrome

notation

ORPHA:199244

part_of

http://www.orpha.net/ORDO/Orphanet_314753

prefixIRI

ORDO:Orphanet_199244

prefLabel

Nelson syndrome

treeView

http://www.orpha.net/ORDO/Orphanet_314753

subClassOf

http://www.orpha.net/ORDO/Orphanet_557493

http://www.orpha.net/ORDO/Orphanet_377792

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http://purl.obolibrary.org/obo/MONDO_0016035 MONDO LOOM
http://purl.obolibrary.org/obo/MONDO_0016035 DOVES LOOM
http://purl.obolibrary.org/obo/DOID_4968 DOID LOOM
http://purl.obolibrary.org/obo/DOID_4968 BAO LOOM
http://purl.obolibrary.org/obo/DOID_4968 HHEAR LOOM
http://purl.obolibrary.org/obo/DOID_4968 NIFSTD LOOM
http://purl.obolibrary.org/obo/DOID_4968 MIDO LOOM
http://purl.obolibrary.org/obo/DOID_4968 FNS-H LOOM
http://phenomebrowser.net/ontologies/mesh/mesh.owl#C04.588.322.609.145.500 RH-MESH LOOM
http://purl.bioontology.org/ontology/SNMI/DB-70140 SNMI LOOM
http://www.owl-ontologies.com/unnamed.owl#RID17524 DERMLEX LOOM
http://www.phoc.org.cn/pmo/class/PMO_00037914 PMAPP-PMO LOOM
http://sbmi.uth.tmc.edu/ontology/ochv#8549 OCHV LOOM
http://www.gamuts.net/entity#Nelson_syndrome GAMUTS LOOM
http://purl.bioontology.org/ontology/SNOMEDCT/43019009 SNOMEDCT LOOM
http://phenomebrowser.net/ontologies/mesh/mesh.owl#D009347 RH-MESH LOOM
http://phenomebrowser.net/ontologies/mesh/mesh.owl#C19.344.609.145.500 RH-MESH LOOM
http://purl.obolibrary.org/obo/NCIT_C84917 BERO LOOM
http://purl.bioontology.org/ontology/MESH/D009347 MESH LOOM
http://purl.jp/bio/4/id/200906021044344924 IOBC LOOM
http://ncicb.nci.nih.gov/xml/owl/EVS/Thesaurus.owl#C84917 NCIT LOOM
http://www.owl-ontologies.com/NPOntology.owl#DOID_4968 NATPRO LOOM
http://ncicb.nci.nih.gov/xml/owl/EVS/Thesaurus.owl#Nelson_Syndrome CSEO LOOM
http://www.limics.org/hrdo/rdfns#pat_id_18380 HRDO LOOM
http://phenomebrowser.net/ontologies/mesh/mesh.owl#C10.228.140.617.738.675.149.500 RH-MESH LOOM
http://id.nlm.nih.gov/mesh/D009347 MDM LOOM
http://phenomebrowser.net/ontologies/mesh/mesh.owl#C19.700.734.145.500 RH-MESH LOOM
http://purl.obolibrary.org/obo/OMIT_0010377 OMIT LOOM
http://www.gamuts.net/entity#Nelson_syndrome GAMUTS REST