{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,20]],"date-time":"2026-07-20T14:17:10Z","timestamp":1784557030920,"version":"3.55.0"},"reference-count":11,"publisher":"Bioscientifica","issue":"2","license":[{"start":{"date-parts":[[2023,4,1]],"date-time":"2023-04-01T00:00:00Z","timestamp":1680307200000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["edm.bioscientifica.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2023,4,1]]},"abstract":"\n Summary<\/jats:title>\n An 11-year-old girl with past medical history of septic shock and multi-organ failure at age 5 presented to her primary care doctor with concern for pallor of the lips. Laboratory studies demonstrated low free thyroxine (T4) and normal thyroid-stimulating hormone (TSH). A referral to endocrinology was made where the patient was evaluated, and laboratory evaluation was repeated. The patient was asymptomatic and clinically euthyroid with a height consistent with her mid-parental height and was in mid- to late-puberty. The repeated laboratory evaluation demonstrated a pattern suggestive of primary hypothyroidism with low free T4 and an elevated TSH. However, the magnitude of elevation of TSH was less than expected, given the degree of lowering of free T4; therefore, central hypothyroidism was considered. Workup was initiated, and laboratory studies and MRI imaging confirmed an underlying diagnosis of panhypopituitarism in the setting of pituitary stalk interruption syndrome.<\/jats:p>\n <\/jats:sec>\n \n Learning points<\/jats:title>\n \n \n Pituitary stalk interruption syndrome is a rare but important cause of panhypopituitarism.<\/jats:p>\n <\/jats:list-item>\n \n Central hypothyroidism should be suspected in patients with low free thyroxine with an inappropriate degree of elevation of thyroid-stimulating hormone.<\/jats:p>\n <\/jats:list-item>\n \n Workup of central hypothyroidism should include multi-pituitary hormone assessment, and, if evident, MRI imaging should be done.<\/jats:p>\n <\/jats:list-item>\n \n Adrenal insufficiency should be suspected in a hypotensive, critically ill patient who is failing to improve on standard-of-care therapy.<\/jats:p>\n <\/jats:list-item>\n <\/jats:list>\n <\/jats:sec>","DOI":"10.1530\/edm-23-0021","type":"journal-article","created":{"date-parts":[[2023,5,15]],"date-time":"2023-05-15T09:47:59Z","timestamp":1684144079000},"update-policy":"https:\/\/doi.org\/10.1530\/crossmarkpolicy-2","source":"Crossref","is-referenced-by-count":1,"title":["Abnormal thyroid function: an unusual presentation of pituitary stalk interruption syndrome"],"prefix":"10.1530","volume":"2023","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5887-219X","authenticated-orcid":true,"given":"Erica A","family":"Steen","sequence":"first","affiliation":[{"name":"University of California, San Diego, San Diego, California, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mary E","family":"Patterson","sequence":"additional","affiliation":[{"name":"University of California, San Diego, San Diego, California, USA"},{"name":"Rady Children\u2019s Hospital, Department of Pediatrics, University of California, San Diego, California, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michelle","family":"Rivera-Vega","sequence":"additional","affiliation":[{"name":"Rady Children\u2019s Hospital, Department of Pediatrics, University of California, San Diego, California, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0008-0914-1607","authenticated-orcid":true,"given":"Susan A","family":"Phillips","sequence":"additional","affiliation":[{"name":"University of California, San Diego, San Diego, California, USA"},{"name":"Rady Children\u2019s Hospital, Department of Pediatrics, University of California, San Diego, California, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"416","reference":[{"key":"bib1","series-title":"Journal of Clinical Endocrinology and Metabolism","first-page":"2936","article-title":"The relationship between TSH and free T4 in a large population is complex and nonlinear and differs by age and sex","volume":"98","author":"Hadlow","year":"2013","unstructured":"Hadlow NCRothacker KMWardrop RBrown SJLim EM & Walsh JP. The relationship between TSH and free T4 in a large population is complex and nonlinear and differs by age and sex. Journal of Clinical Endocrinology and Metabolism2013982936\u20132943. (https:\/\/doi.org\/10.1210\/jc.2012-4223)"},{"key":"bib2","series-title":"Plos One","article-title":"Pituitary stalk interruption syndrome from infancy to adulthood: clinical, hormonal, and radiological assessment according to the initial presentation","volume":"10","author":"Bar","year":"2015","unstructured":"Bar CZadro CDiene GOliver IPienkowski CJouret BCartault AAjaltouni ZSalles JPSevely A, Pituitary stalk interruption syndrome from infancy to adulthood: clinical, hormonal, and radiological assessment according to the initial presentation. Plos One201510 e0142354. (https:\/\/doi.org\/10.1371\/journal.pone.0142354)"},{"key":"bib3","series-title":"Plos One","article-title":"Pituitary stalk interruption syndrome is characterized by genetic heterogeneity","volume":"15","author":"Brauner","year":"2020","unstructured":"Brauner RBignon-Topalovic JBashamboo A & McElreavey K. Pituitary stalk interruption syndrome is characterized by genetic heterogeneity. Plos One202015 e0242358. (https:\/\/doi.org\/10.1371\/journal.pone.0242358)"},{"key":"bib4","series-title":"European Journal of Endocrinology","first-page":"457","article-title":"Pituitary stalk interruption syndrome in 83 patients: novel HESX1 mutation and severe hormonal prognosis in malformative forms","volume":"164","author":"Reynaud","year":"2011","unstructured":"Reynaud RAlbarel FSaveanu AKaffel NCastinetti FLecomte PBrauner RSimonin GGaudart JCarmona E, Pituitary stalk interruption syndrome in 83 patients: novel HESX1 mutation and severe hormonal prognosis in malformative forms. European Journal of Endocrinology2011164457\u2013465. (https:\/\/doi.org\/10.1530\/EJE-10-0892)"},{"key":"bib5","series-title":"EClinicalmedicine","article-title":"Endocrine morbidity in midline brain defects: differences between septo-optic dysplasia and related disorders","volume":"19","author":"Cerbone","year":"2020","unstructured":"Cerbone MG\u00fcemes MWade AImproda N & Dattani M. Endocrine morbidity in midline brain defects: differences between septo-optic dysplasia and related disorders. EClinicalmedicine202019 100224. (https:\/\/doi.org\/10.1016\/j.eclinm.2019.11.017)"},{"key":"bib6","series-title":"Soonchunhyang Medical Science","first-page":"14","article-title":"A case of pituitary stalk interruption syndrome in early childhood presenting with congenital hypothyroidism","volume":"26","author":"Seong","year":"2020","unstructured":"Seong MKJung SYLee J & Lee DH. A case of pituitary stalk interruption syndrome in early childhood presenting with congenital hypothyroidism. Soonchunhyang Medical Science20202614\u201318. (https:\/\/doi.org\/10.15746\/sms.20.004)"},{"key":"bib7","series-title":"Medicine (Baltimore)","article-title":"Thyrotrophic status in patients with pituitary stalk interruption syndrome","volume":"97","author":"Zhang","year":"2018","unstructured":"Zhang QZang LLi YJHan BYGu WJYan WHJin NChen KDu JWang XL, Thyrotrophic status in patients with pituitary stalk interruption syndrome. Medicine (Baltimore)201897 e9084. (https:\/\/doi.org\/10.1097\/MD.0000000000009084)"},{"key":"bib8","series-title":"Journal of Clinical Endocrinology and Metabolism","first-page":"3631","article-title":"Circulating thyrotropin bioactivity in sporadic central hypothyroidism","volume":"85","author":"Persani","year":"2000","unstructured":"Persani LFerretti EBorgato SFaglia G & Beck-Peccoz P. Circulating thyrotropin bioactivity in sporadic central hypothyroidism. Journal of Clinical Endocrinology and Metabolism2000853631\u20133635. (https:\/\/doi.org\/10.1210\/jcem.85.10.6895)"},{"key":"bib9","series-title":"New England Journal of Medicine","first-page":"727","article-title":"Corticosteroid insufficiency in acutely ill patients","volume":"348","author":"Cooper","year":"2003","unstructured":"Cooper MS & Stewart PM. Corticosteroid insufficiency in acutely ill patients. New England Journal of Medicine2003348727\u2013734. (https:\/\/doi.org\/10.1056\/NEJMra020529)"},{"key":"bib10","series-title":"Journal of Clinical Endocrinology and Metabolism","first-page":"3068","article-title":"Clinical review: central hypothyroidism: pathogenic, diagnostic, and therapeutic challenges","volume":"97","author":"Persani","year":"2012","unstructured":"Persani L. Clinical review: central hypothyroidism: pathogenic, diagnostic, and therapeutic challenges. Journal of Clinical Endocrinology and Metabolism2012973068\u20133078. (https:\/\/doi.org\/10.1210\/jc.2012-1616)"},{"key":"bib11","series-title":"Journal of Neuromuscular Diseases","first-page":"31","article-title":"Management of adrenal insufficiency risk after long-term systemic glucocorticoid therapy in Duchenne muscular dystrophy: clinical practice recommendations","volume":"6","author":"Bowden","year":"2019","unstructured":"Bowden SAConnolly AMKinnett K & Zeitler PS. Management of adrenal insufficiency risk after long-term systemic glucocorticoid therapy in Duchenne muscular dystrophy: clinical practice recommendations. Journal of Neuromuscular Diseases2019631\u201341. 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