Sheehan syndrome
- Type
- Malformation syndrome
- Group
- Disorder
- Age of onset
- Adult
- Status
- active
Sheehan syndrome is a rare, acquired, pituitary hormone deficiency disorder resulting from pituitary necrosis following peri- or postpartum hemorrhage characterized by various symptoms depending on resulting hormone decrease (e.g. failure or difficulty with lactation, oligo- or amenorrhea, hot flashes, decreased libido, weakness, fatigue, anorexia, nausea, vomiting, hypoglycemia, hyponatremia, dizziness, decreased muscle mass, adrenal crisis). Secondary hypothyroidism and secondary adrenal insufficiency may also be presenting signs.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Sheehan syndrome | |
| fr | Syndrome de Sheehan | |
| de | Sheehan-Syndrom | |
| es | Síndrome de Sheehan | |
| it | Sindrome di Sheehan | |
| nl | Syndroom van Sheehan | |
| pt | Síndrome Sheehan | |
| pl | Zespół Sheehana | |
| cs | Sheehanův syndrom | |
| uk | Синдром Шихана | |
| zh | 希恩综合征 | |
| tr | Sheehan sendromu |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | E23.0 | NTBT | inclusion | validated |
| ICD-11 | 5A61.0 | NTBT | index | validated |
| MONDO | 0019618 | E | validated | |
| UMLS | C0242342 | E | validated | |
| GARD | 7630 | E | validated | |
| MedDRA | 10036297 | 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:95611Pituitary hormone deficiency of vascular origin
rare endocrine diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | Unknown | — | Worldwide | Class only | Validated | ORPHANET |
| Point prevalence | Unknown | — | Worldwide | Class only | Validated | ORPHANET |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
HPO phenotypes
49 annotations · validated 2017-06-30| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0011748 | Adrenocorticotropic hormone deficiency | |
| HP:0011735 | Adrenocorticotropin deficient adrenal insufficiency | |
| HP:0012432 | Chronic fatigue | |
| HP:0008163 | Decreased circulating cortisol level | |
| HP:0000876 | Oligomenorrhea | |
| HP:0000871 | Panhypopituitarism | |
| HP:0008240 | Secondary growth hormone deficiency | |
| Frequent (79-30%) | ||
| HP:0012504 | Abnormal size of pituitary gland | |
| HP:0000141 | Amenorrhea | |
| HP:0000622 | Blurred vision | |
| HP:0003187 | Breast hypoplasia | |
| HP:0011734 | Central adrenal insufficiency | |
| HP:0030018 | Decreased female libido | |
| HP:0008214 | Decreased serum estradiol | |
| HP:0000958 | Dry skin | |
| HP:0030016 | Dyspareunia | |
| HP:0008213 | Gonadotropin deficiency | |
| HP:0002315 | Headache | |
| HP:0001943 | Hypoglycemia | |
| HP:0003158 | Hyposthenuria | |
| HP:0000802 | Impotence | |
| HP:0002018 | Nausea | |
| HP:0001895 | Normochromic anemia | |
| HP:0001278 | Orthostatic hypotension | |
| HP:0000980 | Pallor | |
| HP:0008245 | Pituitary hypothyroidism | |
| HP:0007987 | Progressive visual field defects | |
| HP:0008202 | Reduced circulating prolactin concentration | |
| HP:0002215 | Sparse axillary hair | |
| HP:0002225 | Sparse pubic hair | |
| Occasional (29-5%) | ||
| HP:0003493 | Antinuclear antibody positivity | |
| HP:0002829 | Arthralgia | |
| HP:0001662 | Bradycardia | |
| HP:0025143 | Chills | |
| HP:0007041 | Chronic lymphocytic meningitis | |
| HP:0002019 | Constipation | |
| HP:0000651 | Diplopia | |
| HP:0000872 | Hashimoto thyroiditis | |
| HP:0002902 | Hyponatremia | |
| HP:0001324 | Muscle weakness | |
| HP:0001513 | Obesity | |
| HP:0001962 | Palpitations | |
| HP:0004396 | Poor appetite | |
| HP:0000407 | Sensorineural hearing impairment | |
| HP:0030907 | Thunderclap headache | |
| HP:0002321 | Vertigo | |
| Very rare (<4-1%) | ||
| HP:0000863 | Central diabetes insipidus | |
| HP:0001259 | Coma | |
| HP:0000709 | Psychosis | |
Run the differential on the 7 obligate / very frequent terms
Annotation source: 12583962[PMID]_17012482[PMID]_22201118[PMID]_25223643[PMID]_24842349[PMID]_21041170[PMID]_21041172[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/91355
- This record as JSON: /data/d/91355.json