{"orpha":95513,"name":"Panhypophysitis","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Infundibulo-panhypophysitis"],"definition":"A type of primary hypophysitis characterized by an inflammation of the entire pituitary gland. Common clinical presentation is diabetes insipidus with polyuria and polydipsia and partial or panhypopituitarism. Other symptoms may include headaches, nausea/vomiting, visual disturbances and fatigue.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-95513/","orphanet_url":"https://www.orpha.net/en/disease/detail/95513","names":{"fr":{"name":"Panhypophysite","synonyms":["Panhypophysite infundibulaire"],"definition":"Insuffisance hypophysaire acquise rare à type d'hypophysite primitive caractérisée par une inflammation de l'ensemble de l'hypophyse. Le tableau clinique courant est un diabète insipide avec polyurie et polydipsie et un panhypopituitarisme partiel ou complet. Les autres symptômes possibles sont des maux de tête, des nausées/vomissements, des troubles de la vue et une fatigue."},"de":{"name":"Hypophysitis, nekrotisierende","synonyms":["Infundibulohypophysitis, nekrotisierende"]},"es":{"name":"Panhipofisitis","synonyms":["Panhipofisitis-infundibulo"],"definition":"La panhipofisitis es una deficiencia de hormona hipofisaria adquirida poco frecuente, un tipo de hipofisitis primaria caracterizada por una inflamación generalizada de la glándula hipofisaria. La presentación clínica habitual consiste en diabetes insípida con poliuria y polidipsia y panhipopituitarismo o hipopituitarismo parcial. Otros síntomas pueden incluir cefaleas, náuseas / vómitos, trastornos visuales y fatiga."},"it":{"name":"Panipofisite","synonyms":["Panipofisite infundibolare"],"definition":"La panipofisite è un difetto raro acquisito degli ormoni ipofisari, un'ipofisite primitiva caratterizzata dall'infiammazione generalizzata dell'ipofisi. I segni clinici più comuni sono il diabete insipido associato a poliuria e polidipsia, e l'ipopituitarismo parziale o il panipopituitarismo. Può associarsi a cefalea, nausea/vomito, disturbi visivi ed affaticamento"},"nl":{"name":"Panhypofysitis","synonyms":["Infundibulo-panhypofysitis"],"definition":"Panhypofysitis is een zeldzame, verworven hypofysaire hormoondeficiëntie en een type van primaire hypofysitis, gekarakteriseerd door inflammatie van de hele hypofyse. Het typische klinische beeld omvat diabetes insipidus met polyurie en polydipsie, en partiële of pan-hypopituïtarisme. Andere mogelijke symptomen zijn onder meer hoofdpijn, misselijkheid/braken, visuele stoornissen en vermoeidheid."},"pt":{"name":"Hipofisite necrotizante"},"pl":{"name":"Zapalenie przysadki","synonyms":["Infundibulo-panhypophysitis"]},"cs":{"name":"Panhypofyzitida","synonyms":["Infundibulo-panhypofyzitida"]},"uk":{"name":"Пангіпофізит","synonyms":["Інфундибуло-пангіпофізит"],"definition":"A type of primary hypophysitis characterized by an inflammation of the entire pituitary gland. Common clinical presentation is diabetes insipidus with polyuria and polydipsia and partial or panhypopituitarism. Other symptoms may include headaches, nausea/vomiting, visual disturbances and fatigue."},"zh":{"name":"全垂体炎","synonyms":["漏斗-全垂体炎"],"definition":"全垂体炎是一种罕见的获得性垂体激素缺乏症，是一种以整个垂体发生炎症为特征的原发性垂体炎。常见的临床表现为尿崩症伴多尿、多饮及部分或全垂体功能减退，其他症状包括头痛、恶心/呕吐、视觉障碍和疲劳。"},"tr":{"name":"Panhipofizit","synonyms":["İnfundibulo-panhipofizit"]}},"xrefs":[{"source":"ICD-10","code":"E23.6","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"5A61.0","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0019839","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C5190786","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97978,"name":"Rare endocrine disease"},"parents":[{"orpha":95506,"name":"Primary hypophysitis","classifications":[193]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":["Adolescent","Adult","Childhood","Elderly"],"prevalence":[],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2017-06-28","source":"23885185[PMID]_27008424[PMID]_18987830[PMID]_17273922[PMID]_14606714[PMID]_27165294[PMID]","terms":[{"hpo":"HP:0011751","label":"Abnormality of the posterior pituitary","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0000863","label":"Central diabetes insipidus","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0000871","label":"Panhypopituitarism","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0012504","label":"Abnormal size of pituitary gland","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0012696","label":"Abnormal thalamic MRI signal intensity","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0011748","label":"Adrenocorticotropic hormone deficiency","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0011735","label":"Adrenocorticotropin deficient adrenal insufficiency","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:0008163","label":"Decreased circulating cortisol level","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0030018","label":"Decreased female libido","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0040306","label":"Decreased male libido","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0008214","label":"Decreased serum estradiol","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0040171","label":"Decreased serum testosterone concentration","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:0003158","label":"Hyposthenuria","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000802","label":"Impotence","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000870","label":"Increased circulating prolactin concentration","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:0001959","label":"Polydipsia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0007987","label":"Progressive visual field defects","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0008240","label":"Secondary growth hormone deficiency","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0003493","label":"Antinuclear antibody positivity","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0007041","label":"Chronic lymphocytic meningitis","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:0004396","label":"Poor appetite","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0008202","label":"Reduced circulating prolactin concentration","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000407","label":"Sensorineural hearing impairment","frequency":"Occasional (29-5%)","criteria":null}]}}