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Compared to baseline, eighteen months of <jats:styled-content style=\"fixed-case\">CSHI<\/jats:styled-content> resulted in decreased (<jats:italic>P\u00a0<\/jats:italic>=<jats:italic>\u00a0<\/jats:italic>0.043) 0700\u2010hour <jats:styled-content style=\"fixed-case\">ACTH<\/jats:styled-content>, 17\u2010hydroxyprogesterone, androstenedione and progesterone; increased whole\u2010body lean mass (<jats:italic>P\u00a0<\/jats:italic>=<jats:italic>\u00a0<\/jats:italic>0.024); and improved <jats:styled-content style=\"fixed-case\">HRQ<\/jats:styled-content>oL, especially symptoms of adrenal insufficiency (<jats:italic>P\u00a0<\/jats:italic>=<jats:italic>\u00a0<\/jats:italic>0.003). Findings at six and eighteen months did not differ, and improvements achieved in androgen control, lean body mass and <jats:styled-content style=\"fixed-case\">HRQ<\/jats:styled-content>oL after 6\u00a0months of <jats:styled-content style=\"fixed-case\">CSHI<\/jats:styled-content> were maintained at eighteen months. The hydrocortisone dose appeared to decrease with time [6 vs 18\u00a0months: 38.3\u00a0\u00b1\u00a08.8 vs 33.6\u00a0\u00b1\u00a012.2\u00a0mg\/day (<jats:italic>P\u00a0<\/jats:italic>=<jats:italic>\u00a0<\/jats:italic>0.062)], especially in women receiving oral contraceptives. Reduction of testicular adrenal rest and adrenal size observed at 6\u00a0months remained stable. In one patient, an adrenal adenoma continually decreased over time. Subjective improvement in hirsutism was reported.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Long\u2010term use of <jats:styled-content style=\"fixed-case\">CSHI<\/jats:styled-content> is a safe and well\u2010tolerated treatment option in a select set of adults with classic <jats:styled-content style=\"fixed-case\">CAH<\/jats:styled-content>. Improvements observed short term in disease control and subjective health status continued long term.<\/jats:p><\/jats:sec>","DOI":"10.1111\/cen.13813","type":"journal-article","created":{"date-parts":[[2018,7,13]],"date-time":"2018-07-13T10:36:24Z","timestamp":1531478184000},"page":"399-407","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":44,"title":["Long\u2010term use of continuous subcutaneous hydrocortisone infusion therapy in patients with congenital adrenal hyperplasia"],"prefix":"10.1111","volume":"89","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0637-4559","authenticated-orcid":false,"given":"Ashwini","family":"Mallappa","sequence":"first","affiliation":[{"name":"National Institutes of Health Clinical Center  Bethesda Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aikaterini A.","family":"Nella","sequence":"additional","affiliation":[{"name":"National Institutes of Health Clinical 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