Pituitary deficiency due to empty sella turcica syndrome
Synonyms: Hypopituitarism due to empty sella turcica syndrome
- Type
- Disease
- Group
- Disorder
- Age of onset
- Adult, Elderly
- Status
- active
A rare pituitary deficiency characterized by herniation of the subarachnoid space into the sella turcica, resulting in flattening of the pituitary gland and endocrine dysfunction. Most common endocrine abnormalities are hyperprolactinemia and growth hormone deficit. Clinical symptoms are highly variable and include headaches, irregular menstruation, galactorrhea, obesity, and visual disturbances, among others.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Pituitary deficiency due to empty sella turcica syndrome | Hypopituitarism due to empty sella turcica syndrome |
| fr | Déficit hypophysaire associé au syndrome de la selle turcique vide | Hypopituitarisme associé au syndrome de la selle turcique vide |
| de | Hypophysenfunktionsstörung durch Empty-Sella-Syndrom | |
| es | Deficiencia hipofisaria por el síndrome de la silla turca vacía | |
| it | Deficit ipofisario da sindrome della sella turcica vuota | |
| nl | Hypofysedeficiëntie door lege sella turcica-syndroom | Hypopituïtarisme door lege sella turcica-syndroom |
| pt | Síndrome de deficiência pituitária por sela turca vazia | |
| pl | Niedoczynność przysadki spowodowana zespołem pustego siodła tureckiego | = en |
| cs | Hypofyzární deficit způsobený syndromem prázdného tureckého sedla | Hypopituitarismus způsobený syndromem prázdného tureckého sedla |
| uk | Гіпофізарний дефіцит при синдромі порожнього турецького сідла | Гіпопітуїтаризм внаслідок синдрому порожнього селлового міхура |
| zh | 空泡蝶鞍综合征所致垂体缺陷 | 空蝶鞍综合征所致垂体功能减退症 |
| tr | Boş sella tursika sendromuna bağlı hipofiz yetmezliği | Boş sella tursika sendromuna bağlı hipopituitarizm |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | E23.0 | NTBT | attributed | validated |
| ICD-11 | 5A61.0 | NTBT | index | validated |
| MONDO | 0019617 | E | validated | |
| UMLS | C4275064 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:95505Pituitary hormone deficiency of meningeal origin
rare endocrine diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
0 recordsNo prevalence record in this release.
HPO phenotypes
21 annotations · validated 2017-03-20| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Frequent (79-30%) | ||
| HP:0002315 | Headache | |
| HP:0000802 | Impotence | |
| HP:0000876 | Oligomenorrhea | |
| Occasional (29-5%) | ||
| HP:0011446 | Abnormality of higher mental function | |
| HP:0002921 | Abnormality of the cerebrospinal fluid | |
| HP:0011748 | Adrenocorticotropic hormone deficiency | |
| HP:0002960 | Autoimmunity | |
| HP:0000824 | Decreased response to growth hormone stimulation test | |
| HP:0040075 | Hypopituitarism | |
| HP:0000870 | Increased circulating prolactin concentration | |
| HP:0008245 | Pituitary hypothyroidism | |
| HP:0000826 | Precocious puberty | |
| HP:0007663 | Reduced visual acuity | |
| HP:0001250 | Seizure | |
| HP:0030532 | Visual acuity test abnormality | |
| Very rare (<4-1%) | ||
| HP:0000863 | Central diabetes insipidus | |
| HP:0000651 | Diplopia | |
| HP:0002902 | Hyponatremia | |
| HP:0002615 | Hypotension | |
| HP:0100661 | Trigeminal neuralgia | |
| Excluded (0%) | ||
| HP:0002893 | Pituitary adenoma | |
Annotation source: 17643081[PMID]_2221821[PMID]_23617499[PMID]_26871784[PMID]_17635438[PMID]_3417843[PMID]_1914345[PMID]_10584841[PMID]_6286712[PMID]_16304986[PMID]_10424206[PMID]_15972577[PMID]_19352054[PMID]_910813[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/91354
- This record as JSON: /data/d/91354.json