{"orpha":91355,"name":"Sheehan syndrome","type":"Malformation syndrome","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"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.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-91355/","orphanet_url":"https://www.orpha.net/en/disease/detail/91355","names":{"fr":{"name":"Syndrome de Sheehan","definition":"Insuffisance hypophysaires acquise rare résultant d'une nécrose hypophysaire consécutive à une hémorragie péri- ou post-partum, caractérisée par divers symptômes en fonction de la baisse hormonale ainsi induite (par exemple, absence ou difficulté de lactation, oligo- ou aménorrhée, bouffées de chaleur, baisse de la libido, faiblesse, fatigue, anorexie, nausées, vomissements, hypoglycémie, hyponatrémie, vertiges, baisse de la masse musculaire, crise surrénalienne). Une hypothyroïdie secondaire et une insuffisance surrénalienne secondaire peuvent également être des signes révélateurs."},"de":{"name":"Sheehan-Syndrom"},"es":{"name":"Síndrome de Sheehan","definition":"El síndrome de Sheehan es un trastorno de deficiencia de hormonas hipofisarias, adquirido y poco frecuente, que se produce por necrosis hipofisaria después de una hemorragia peri- o postparto caracterizada por diversos síntomas que dependen de la disminución de la hormona resultante (por ejemplo, fallo o dificultad con la lactancia, oligo- / amenorrea, sofocos, disminución de la libido, debilidad, fatiga, anorexia, náuseas, vómitos, hipoglucemia, hiponatremia, mareos, disminución de la masa muscular, crisis suprarrenal). También pueden presentar signos de hipotiroidismo secundario y de insuficiencia suprarrenal secundaria."},"it":{"name":"Sindrome di Sheehan","definition":"La sindrome di Sheehan è un difetto raro acquisito dell'ormone ipofisario, causato dalla necrosi dell'ipofisi, secondaria ad una emorragia peri- o postpartum. È caratterizzata da segni clinici correlati alla riduzione dell'ormone (ad esempio l'impossibilità o la difficoltà di allattare, l'oligomenorrea o l'amenorrea, le vampate di calore, la diminuzione della libido, la debolezza, l'affaticamento, l'anoressia, la nausea, il vomito, l'ipoglicemia, l'iponatriemia, le vertigini, la riduzione delle masse muscolari, la crisi surrenalica). La malattia può esordire con ipotiroidismo secondario e insufficienza surrenalica secondaria."},"nl":{"name":"Syndroom van Sheehan","definition":"Syndroom van Sheehan is een zeldzame, verworven stoornis met deficiëntie van hypofysehormonen als gevolg van necrose van de hypofyse na peri- of postpartum hemorragie, en wordt gekarakteriseerd door verscheidene symptomen die afhangen van welk hormoon minder geproduceerd wordt (e.g. moeilijke of afwezige lactatie, oligo- of amenorroe, opvliegers, verminderd libido, zwakheid, vermoeidheid, anorexie, misselijkheid, braken, hypoglycemie, hyponatriëmie, duizeligheid, verminderde spiermassa, adrenale crisis). Secundaire hypothyreoïdie en secundaire bijnierschorsinsufficiëntie kunnen ook presenterende tekenen zijn."},"pt":{"name":"Síndrome Sheehan"},"pl":{"name":"Zespół Sheehana","definition":"*Zespół Sheehana jest rzadkim, nabytym zaburzeniem, związanym z niedoborem hormonów przysadki, który rozwija się w wyniku martwicy przysadki wskutek krwawienia w okresie okołoporodowym lub poporodowym. Charakteryzuje się szerokim spektrum objawów, które zależą od stopnia niedoboru poszczególnych hormonów (np. brak lub zaburzenia laktacji, brak miesiączki lub rzadkie miesiączki, uderzenia gorąca, spadek libido, osłabienie, zmęczenie, jadłowstręt,, nudności, wymioty, hipoglikemia, hiponatremia, zawroty głowy, zmniejszenie masy mięśniowej, przełom nadnerczowy). Może również wystąpić wtórna niedoczynność tarczycy i wtórna niewydolność nadnerczy."},"cs":{"name":"Sheehanův syndrom"},"uk":{"name":"Синдром Шихана","definition":"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."},"zh":{"name":"希恩综合征"},"tr":{"name":"Sheehan sendromu"}},"xrefs":[{"source":"ICD-10","code":"E23.0","qualifier":"NTBT","icd_relation":"inclusion","validated":true},{"source":"ICD-11","code":"5A61.0","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"421687193"},{"source":"MONDO","code":"0019618","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0242342","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"7630","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10036297","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97978,"name":"Rare endocrine disease"},"parents":[{"orpha":95611,"name":"Pituitary hormone deficiency of vascular origin","classifications":[193]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":["Adult"],"prevalence":[{"type":"Annual incidence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"},{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2017-06-30","source":"12583962[PMID]_17012482[PMID]_22201118[PMID]_25223643[PMID]_24842349[PMID]_21041170[PMID]_21041172[PMID]","terms":[{"hpo":"HP:0011748","label":"Adrenocorticotropic hormone deficiency","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0011735","label":"Adrenocorticotropin deficient adrenal insufficiency","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0012432","label":"Chronic fatigue","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0008163","label":"Decreased circulating cortisol level","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0000876","label":"Oligomenorrhea","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0000871","label":"Panhypopituitarism","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0008240","label":"Secondary growth hormone deficiency","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0012504","label":"Abnormal size of pituitary gland","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000141","label":"Amenorrhea","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000622","label":"Blurred vision","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0003187","label":"Breast hypoplasia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0011734","label":"Central adrenal insufficiency","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0030018","label":"Decreased female libido","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0008214","label":"Decreased serum estradiol","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000958","label":"Dry skin","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0030016","label":"Dyspareunia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0008213","label":"Gonadotropin deficiency","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002315","label":"Headache","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001943","label":"Hypoglycemia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0003158","label":"Hyposthenuria","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000802","label":"Impotence","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002018","label":"Nausea","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001895","label":"Normochromic anemia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001278","label":"Orthostatic hypotension","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000980","label":"Pallor","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0008245","label":"Pituitary hypothyroidism","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0007987","label":"Progressive visual field defects","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0008202","label":"Reduced circulating prolactin concentration","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002215","label":"Sparse axillary hair","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002225","label":"Sparse pubic hair","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0003493","label":"Antinuclear antibody positivity","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002829","label":"Arthralgia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001662","label":"Bradycardia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0025143","label":"Chills","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0007041","label":"Chronic lymphocytic meningitis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002019","label":"Constipation","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000651","label":"Diplopia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000872","label":"Hashimoto thyroiditis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002902","label":"Hyponatremia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001324","label":"Muscle weakness","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001513","label":"Obesity","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001962","label":"Palpitations","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0004396","label":"Poor appetite","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000407","label":"Sensorineural hearing impairment","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0030907","label":"Thunderclap headache","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002321","label":"Vertigo","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000863","label":"Central diabetes insipidus","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0001259","label":"Coma","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0000709","label":"Psychosis","frequency":"Very rare (<4-1%)","criteria":null}]}}