{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,8]],"date-time":"2026-10-08T03:24:12Z","timestamp":1791429852670,"version":"4.3.3"},"reference-count":191,"publisher":"The Endocrine Society","issue":"6","license":[{"start":{"date-parts":[[2019,7,11]],"date-time":"2019-07-11T00:00:00Z","timestamp":1562803200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100010269","name":"Wellcome Trust","doi-asserted-by":"publisher","award":["WT209492\/Z\/17\/Z"],"award-info":[{"award-number":["WT209492\/Z\/17\/Z"]}],"id":[{"id":"10.13039\/100010269","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100010269","name":"Wellcome Trust","doi-asserted-by":"publisher","award":["WT101671AIA"],"award-info":[{"award-number":["WT101671AIA"]}],"id":[{"id":"10.13039\/100010269","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000265","name":"Medical Research Council","doi-asserted-by":"publisher","award":["G1001964"],"award-info":[{"award-number":["G1001964"]}],"id":[{"id":"10.13039\/501100000265","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000265","name":"Medical Research Council","doi-asserted-by":"publisher","award":["MR\/R017913\/1"],"award-info":[{"award-number":["MR\/R017913\/1"]}],"id":[{"id":"10.13039\/501100000265","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000361","name":"Diabetes UK","doi-asserted-by":"publisher","award":["18\/0005782"],"award-info":[{"award-number":["18\/0005782"]}],"id":[{"id":"10.13039\/501100000361","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Academy of Medical Sciences UK","award":["NAF004\\1002"],"award-info":[{"award-number":["NAF004\\1002"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2019,12,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Steroid biosynthesis and metabolism are reflected by the serum steroid metabolome and, in even more detail, by the 24-hour urine steroid metabolome, which can provide unique insights into alterations of steroid flow and output indicative of underlying conditions. Mass spectrometry\u2013based steroid metabolome profiling has allowed for the identification of unique multisteroid signatures associated with disorders of steroid biosynthesis and metabolism that can be used for personalized approaches to diagnosis, differential diagnosis, and prognostic prediction. Additionally, steroid metabolome analysis has been used successfully as a discovery tool, for the identification of novel steroidogenic disorders and pathways as well as revealing insights into the pathophysiology of adrenal disease. Increased availability and technological advances in mass spectrometry\u2013based methodologies have refocused attention on steroid metabolome profiling and facilitated the development of high-throughput steroid profiling methods soon to reach clinical practice. Furthermore, steroid metabolomics, the combination of mass spectrometry\u2013based steroid analysis with machine learning\u2013based approaches, has facilitated the development of powerful customized diagnostic approaches. In this review, we provide a comprehensive up-to-date overview of the utility of steroid metabolome analysis for the diagnosis and management of inborn disorders of steroidogenesis and autonomous adrenal steroid excess in the context of adrenal tumors.<\/jats:p>","DOI":"10.1210\/er.2018-00262","type":"journal-article","created":{"date-parts":[[2019,7,16]],"date-time":"2019-07-16T11:06:00Z","timestamp":1563275160000},"page":"1605-1625","source":"Crossref","is-referenced-by-count":136,"title":["Steroid Metabolome Analysis in Disorders of Adrenal Steroid Biosynthesis and Metabolism"],"prefix":"10.1210","volume":"40","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1669-6383","authenticated-orcid":false,"given":"Karl-Heinz","family":"Storbeck","sequence":"first","affiliation":[{"name":"Department of Biochemistry, Stellenbosch University, Stellenbosch, South Africa"},{"name":"Institute of Metabolism and Systems Research, University of Birmingham, Birmingham, United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8540-4861","authenticated-orcid":false,"given":"Lina","family":"Schiffer","sequence":"additional","affiliation":[{"name":"Institute of Metabolism and Systems Research, University of Birmingham, Birmingham, United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2731-577X","authenticated-orcid":false,"given":"Elizabeth S","family":"Baranowski","sequence":"additional","affiliation":[{"name":"Institute of Metabolism and Systems Research, University of Birmingham, Birmingham, United Kingdom"},{"name":"Centre for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, Birmingham, United Kingdom"},{"name":"Department of Paediatric Endocrinology and Diabetes, Birmingham Women\u2019s and Children\u2019s Hospital NHS Foundation Trust, Birmingham, United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4906-8239","authenticated-orcid":false,"given":"Vasileios","family":"Chortis","sequence":"additional","affiliation":[{"name":"Institute of Metabolism and Systems Research, University of Birmingham, Birmingham, United Kingdom"},{"name":"Centre for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, Birmingham, United Kingdom"},{"name":"Department of Endocrinology, Queen Elizabeth Hospital, University Hospitals Birmingham NHS Foundation Trust, Birmingham, United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4821-0336","authenticated-orcid":false,"given":"Alessandro","family":"Prete","sequence":"additional","affiliation":[{"name":"Institute of Metabolism and Systems Research, University of Birmingham, Birmingham, United Kingdom"},{"name":"Centre for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, Birmingham, United Kingdom"},{"name":"Department of Endocrinology, Queen Elizabeth Hospital, University Hospitals Birmingham NHS Foundation Trust, Birmingham, United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8280-703X","authenticated-orcid":false,"given":"Lise","family":"Barnard","sequence":"additional","affiliation":[{"name":"Department of Biochemistry, Stellenbosch University, Stellenbosch, South Africa"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0708-2999","authenticated-orcid":false,"given":"Lorna C","family":"Gilligan","sequence":"additional","affiliation":[{"name":"Institute of Metabolism and Systems Research, University of Birmingham, Birmingham, United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5835-5643","authenticated-orcid":false,"given":"Angela E","family":"Taylor","sequence":"additional","affiliation":[{"name":"Institute of Metabolism and Systems Research, University of Birmingham, Birmingham, United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9181-3995","authenticated-orcid":false,"given":"Jan","family":"Idkowiak","sequence":"additional","affiliation":[{"name":"Institute of Metabolism and Systems Research, University of Birmingham, Birmingham, United Kingdom"},{"name":"Centre for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, Birmingham, United Kingdom"},{"name":"Department of Paediatric Endocrinology and Diabetes, Birmingham Women\u2019s and Children\u2019s Hospital NHS Foundation Trust, Birmingham, United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5106-9719","authenticated-orcid":false,"given":"Wiebke","family":"Arlt","sequence":"additional","affiliation":[{"name":"Institute of Metabolism and Systems Research, University of Birmingham, Birmingham, United Kingdom"},{"name":"Centre for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, Birmingham, United Kingdom"},{"name":"Department of Endocrinology, Queen Elizabeth Hospital, University Hospitals Birmingham NHS Foundation Trust, Birmingham, United Kingdom"},{"name":"NIHR Birmingham Biomedical Research Centre, University Hospitals Birmingham NHS Foundation Trust and University of Birmingham, Birmingham, United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cedric H L","family":"Shackleton","sequence":"additional","affiliation":[{"name":"Institute of Metabolism and Systems Research, University of Birmingham, Birmingham, United Kingdom"},{"name":"UCSF Benioff Children\u2019s Hospital Oakland Research Institute, Oakland, California"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"80","published-online":{"date-parts":[[2019,7,11]]},"reference":[{"issue":"12","key":"2019122310485919900_bib1","doi-asserted-by":"crossref","first-page":"3775","DOI":"10.1210\/jc.2011-1565","article-title":"Urine steroid metabolomics as a biomarker tool for detecting malignancy in adrenal tumors","volume":"96","author":"Arlt","year":"2011","journal-title":"J Clin Endocrinol Metab"},{"key":"2019122310485919900_bib2","unstructured":"Biehl M , SchneiderP, SmithDJ, StiekemaH, TaylorAE, HughesBA, ShackletonCHL, StewartPM, ArltW. Matrix relevance LVQ in steroid metabolomics based classification of adrenal tumors. Available at: www.i6doc.com\/en\/livre\/?GCOI=28001100967420. Accessed 30 April 2019."},{"key":"2019122310485919900_bib3","doi-asserted-by":"crossref","first-page":"222","DOI":"10.1016\/j.jtbi.2018.07.025","article-title":"Learning pharmacokinetic models for in vivo glucocorticoid activation","volume":"455","author":"Bunte","year":"2018","journal-title":"J Theor Biol"},{"issue":"150","key":"2019122310485919900_bib4","doi-asserted-by":"crossref","first-page":"20180572","DOI":"10.1098\/rsif.2018.0572","article-title":"Simultaneous parameter estimation and variable selection via the logit-normal continuous analogue of the spike-and-slab prior","volume":"16","author":"Thomson","year":"2019","journal-title":"J R Soc Interface"},{"key":"2019122310485919900_bib5","doi-asserted-by":"crossref","first-page":"36","DOI":"10.1016\/j.jchromb.2018.03.033","article-title":"The utility of ultra-high performance supercritical fluid chromatography\u2013tandem mass spectrometry (UHPSFC-MS\/MS) for clinically relevant steroid analysis","volume":"1085","author":"Storbeck","year":"2018","journal-title":"J Chromatogr B Analyt Technol Biomed Life Sci"},{"issue":"4","key":"2019122310485919900_bib6","doi-asserted-by":"crossref","first-page":"R255","DOI":"10.1530\/JME-18-0150","article-title":"Applying mass spectrometric methods to study androgen biosynthesis and metabolism in prostate cancer","volume":"62","author":"Knuuttila","year":"2019","journal-title":"J Mol Endocrinol"},{"issue":"8","key":"2019122310485919900_bib7","doi-asserted-by":"crossref","first-page":"974","DOI":"10.1210\/js.2018-00135","article-title":"GC\/MS in recent years has defined the normal and clinically disordered steroidome: will it soon be surpassed by LC\/tandem MS in this role","volume":"2","author":"Shackleton","year":"2018","journal-title":"J Endocr Soc"},{"issue":"3","key":"2019122310485919900_bib8","doi-asserted-by":"crossref","first-page":"C113","DOI":"10.1016\/0303-7207(91)90116-A","article-title":"Intracrinology","volume":"78","author":"Labrie","year":"1991","journal-title":"Mol Cell Endocrinol"},{"key":"2019122310485919900_bib9","volume-title":"Detoxication Mechanisms; the Metabolism and Detoxication of Drugs, Toxic Substances, and Other Organic Compounds","author":"Williams","year":"1959"},{"issue":"10108","key":"2019122310485919900_bib10","doi-asserted-by":"crossref","first-page":"2194","DOI":"10.1016\/S0140-6736(17)31431-9","article-title":"Congenital adrenal hyperplasia","volume":"390","author":"El-Maouche","year":"2017","journal-title":"Lancet"},{"issue":"5","key":"2019122310485919900_bib11","doi-asserted-by":"crossref","first-page":"292","DOI":"10.1159\/000488034","article-title":"Monogenic disorders of adrenal steroidogenesis","volume":"89","author":"Baranowski","year":"2018","journal-title":"Horm Res Paediatr"},{"issue":"1","key":"2019122310485919900_bib12","doi-asserted-by":"crossref","first-page":"81","DOI":"10.1210\/er.2010-0013","article-title":"The molecular biology, biochemistry, and physiology of human steroidogenesis and its disorders","volume":"32","author":"Miller","year":"2011","journal-title":"Endocr Rev"},{"issue":"8","key":"2019122310485919900_bib13","doi-asserted-by":"crossref","first-page":"3682","DOI":"10.1210\/jcem.87.8.8712","article-title":"The diagnosis of congenital adrenal hyperplasia in the newborn by gas chromatography\/mass spectrometry analysis of random urine specimens","volume":"87","author":"Caulfield","year":"2002","journal-title":"J Clin Endocrinol Metab"},{"issue":"3-5","key":"2019122310485919900_bib14","doi-asserted-by":"crossref","first-page":"496","DOI":"10.1016\/j.jsbmb.2010.04.010","article-title":"Gas chromatography\/mass spectrometry (GC\/MS) remains a pre-eminent discovery tool in clinical steroid investigations even in the era of fast liquid chromatography tandem mass spectrometry (LC\/MS\/MS)","volume":"121","author":"Krone","year":"2010","journal-title":"J Steroid Biochem Mol Biol"},{"issue":"3","key":"2019122310485919900_bib15","doi-asserted-by":"crossref","first-page":"E465","DOI":"10.1210\/jc.2011-2413","article-title":"A missense mutation in the human cytochrome b5 gene causes 46,XY disorder of sex development due to true isolated 17,20 lyase deficiency","volume":"97","author":"Idkowiak","year":"2012","journal-title":"J Clin Endocrinol Metab"},{"issue":"2","key":"2019122310485919900_bib16","doi-asserted-by":"crossref","first-page":"E257","DOI":"10.1210\/jc.2011-0640","article-title":"Genotype-phenotype analysis in congenital adrenal hyperplasia due to P450 oxidoreductase deficiency","volume":"97","author":"Krone","year":"2012","journal-title":"J Clin Endocrinol Metab"},{"issue":"4","key":"2019122310485919900_bib17","doi-asserted-by":"crossref","first-page":"697","DOI":"10.1210\/jcem-56-4-697","article-title":"17\u03b1-Hydroxylase deficiency: mineralocorticoid hormone profiles in an affected family","volume":"56","author":"D\u2019Armiento","year":"1983","journal-title":"J Clin Endocrinol Metab"},{"issue":"9","key":"2019122310485919900_bib18","doi-asserted-by":"crossref","first-page":"826","DOI":"10.1590\/S0004-27302010000900009","article-title":"Distinctive profile of the 17-hydroxylase and 17,20-lyase activities revealed by urinary steroid metabolomes of patients with CYP17 deficiency","volume":"54","author":"Neres","year":"2010","journal-title":"Arq Bras Endocrinol Metabol"},{"issue":"3","key":"2019122310485919900_bib19","doi-asserted-by":"crossref","first-page":"385","DOI":"10.1530\/EJE-07-0712","article-title":"Metabolic evidence for impaired 17\u03b1-hydroxylase activity in a kindred bearing the E305G mutation for isolate 17,20-lyase activity","volume":"158","author":"Tiosano","year":"2008","journal-title":"Eur J Endocrinol"},{"issue":"3","key":"2019122310485919900_bib20","doi-asserted-by":"crossref","first-page":"193","DOI":"10.1002\/ajmg.a.20108","article-title":"Mutations in CYP11B1 gene: penotype-genotype correlations","volume":"122A","author":"Zhu","year":"2003","journal-title":"Am J Med Genet A"},{"issue":"2","key":"2019122310485919900_bib21","doi-asserted-by":"crossref","first-page":"345","DOI":"10.1210\/jcem-51-2-345","article-title":"Pubertal presentation of congenital \u03945\u20133\u03b2-hydroxysteroid dehydrogenase deficiency","volume":"51","author":"Rosenfield","year":"1980","journal-title":"J Clin Endocrinol Metab"},{"issue":"6","key":"2019122310485919900_bib22","first-page":"1797","article-title":"Modified-release and conventional glucocorticoids and diurnal androgen excretion in congenital adrenal hyperplasia","volume":"102","author":"Jones","year":"2017","journal-title":"J Clin Endocrinol Metab"},{"key":"2019122310485919900_bib23","doi-asserted-by":"crossref","first-page":"549","DOI":"10.1007\/978-3-540-76698-8_26","article-title":"Genetic disorders of steroid metabolism diagnosed by mass spectrometry","volume-title":"Laboratory Guide to the Methods in Biochemical Genetics","author":"Shackleton","year":"2008"},{"issue":"5","key":"2019122310485919900_bib24","doi-asserted-by":"crossref","first-page":"601","DOI":"10.1530\/EJE-15-1181","article-title":"Adrenal-derived 11-oxygenated 19-carbon steroids are the dominant androgens in classic 21-hydroxylase deficiency","volume":"174","author":"Turcu","year":"2016","journal-title":"Eur J Endocrinol"},{"key":"2019122310485919900_bib25","doi-asserted-by":"crossref","first-page":"221","DOI":"10.1016\/j.jsbmb.2017.12.016","article-title":"Androgen excess is due to elevated 11-oxygenated androgens in treated children with congenital adrenal hyperplasia","volume":"178","author":"Kamrath","year":"2018","journal-title":"J Steroid Biochem Mol Biol"},{"issue":"3","key":"2019122310485919900_bib26","doi-asserted-by":"crossref","first-page":"E367","DOI":"10.1210\/jc.2011-1997","article-title":"Increased activation of the alternative \u201cbackdoor\u201d pathway in patients with 21-hydroxylase deficiency: evidence from urinary steroid hormone analysis","volume":"97","author":"Kamrath","year":"2012","journal-title":"J Clin Endocrinol Metab"},{"issue":"3","key":"2019122310485919900_bib27","doi-asserted-by":"crossref","first-page":"223","DOI":"10.1002\/ajmg.a.30104","article-title":"Biochemical diagnosis of Antley-Bixler syndrome by steroid analysis","volume":"128A","author":"Shackleton","year":"2004","journal-title":"Am J Med Genet A"},{"issue":"7","key":"2019122310485919900_bib28","doi-asserted-by":"crossref","first-page":"2643","DOI":"10.1210\/jc.2005-2460","article-title":"Urine steroid hormone profile analysis in cytochrome P450 oxidoreductase deficiency: implication for the backdoor pathway to dihydrotestosterone","volume":"91","author":"Homma","year":"2006","journal-title":"J Clin Endocrinol Metab"},{"issue":"6","key":"2019122310485919900_bib29","doi-asserted-by":"crossref","first-page":"3158","DOI":"10.1074\/jbc.273.6.3158","article-title":"Cytochrome b5 augments the 17,20-lyase activity of human P450c17 without direct electron transfer","volume":"273","author":"Auchus","year":"1998","journal-title":"J Biol Chem"},{"issue":"33","key":"2019122310485919900_bib30","doi-asserted-by":"crossref","first-page":"29086","DOI":"10.1074\/jbc.M111.253245","article-title":"Mouse knockout of the cholesterogenic cytochrome P450 lanosterol 14\u03b1-demethylase (Cyp51) resembles Antley-Bixler syndrome","volume":"286","author":"Keber","year":"2011","journal-title":"J Biol Chem"},{"issue":"9","key":"2019122310485919900_bib31","doi-asserted-by":"crossref","first-page":"1830","DOI":"10.1093\/clinchem\/38.9.1830","article-title":"Identification of the steroids in neonatal plasma that interfere with 17 alpha-hydroxyprogesterone radioimmunoassays","volume":"38","author":"Wong","year":"1992","journal-title":"Clin Chem"},{"issue":"9","key":"2019122310485919900_bib32","doi-asserted-by":"crossref","first-page":"901","DOI":"10.1016\/0022-4731(81)90239-9","article-title":"Identification of 15\u03b2-hydroxylated C21 steroids in the neo-natal period: the role of 3\u03b1,15\u03b2,17\u03b1-trihydroxy-5\u03b2-pregnan-20-one in the perinatal diagnosis of congenital adrenal hyperplasia (CAH) due to a 21-hydroxylase deficiency","volume":"14","author":"Joannou","year":"1981","journal-title":"J Steroid Biochem"},{"issue":"6","key":"2019122310485919900_bib33","doi-asserted-by":"crossref","first-page":"243","DOI":"10.1159\/000185529","article-title":"Definitive diagnosis of enzymatic deficiencies of steroidogenesis in at-risk newborns and infants by urinary marker analysis using GC\/MS-SIM","volume":"48","author":"Ma\u0142unowicz","year":"1997","journal-title":"Horm Res"},{"key":"2019122310485919900_bib34","doi-asserted-by":"crossref","first-page":"10","DOI":"10.1016\/j.jsbmb.2015.10.013","article-title":"Diagnosis of 21-hydroxylase deficiency by urinary metabolite ratios using gas chromatography\u2013mass spectrometry analysis: Reference values for neonates and infants","volume":"156","author":"Kamrath","year":"2016","journal-title":"J Steroid Biochem Mol Biol"},{"issue":"5","key":"2019122310485919900_bib35","doi-asserted-by":"crossref","first-page":"421","DOI":"10.1016\/0960-0760(93)90011-K","article-title":"New identified 15\u03b2-hydroxylated 21-deoxy-pregnanes in congenital adrenal hyperplasia due to 21-hydroxylase deficiency","volume":"45","author":"Kraan","year":"1993","journal-title":"J Steroid Biochem Mol Biol"},{"key":"2019122310485919900_bib36","doi-asserted-by":"crossref","first-page":"334","DOI":"10.1016\/j.jsbmb.2013.07.013","article-title":"21-Hydroxylase deficiency in the neonate\u2014trends in steroid anabolism and catabolism during the first weeks of life","volume":"138","author":"Christakoudi","year":"2013","journal-title":"J Steroid Biochem Mol Biol"},{"issue":"2","key":"2019122310485919900_bib37","doi-asserted-by":"crossref","first-page":"201","DOI":"10.1038\/ng1097-201","article-title":"The genetic and functional basis of isolated 17,20-lyase deficiency","volume":"17","author":"Geller","year":"1997","journal-title":"Nat Genet"},{"issue":"6","key":"2019122310485919900_bib38","doi-asserted-by":"crossref","first-page":"976","DOI":"10.1210\/jcem-48-6-976","article-title":"Metabolism of radiolabeled corticosterone in an adult with the 17\u03b1-hydroxylase deficiency syndrome","volume":"48","author":"Shackleton","year":"1979","journal-title":"J Clin Endocrinol Metab"},{"issue":"1","key":"2019122310485919900_bib39","doi-asserted-by":"crossref","first-page":"3","DOI":"10.1016\/0009-8981(83)90145-6","article-title":"Determination of steroid profiles in healthy and diseased states: identification and quantitation of a block of 17\u03b1-hydroxylase","volume":"129","author":"Fennessey","year":"1983","journal-title":"Clin Chim Acta"},{"issue":"3","key":"2019122310485919900_bib40","doi-asserted-by":"crossref","first-page":"228","DOI":"10.1038\/ng1300","article-title":"Mutant P450 oxidoreductase causes disordered steroidogenesis with and without Antley-Bixler syndrome","volume":"36","author":"Fl\u00fcck","year":"2004","journal-title":"Nat Genet"},{"issue":"9427","key":"2019122310485919900_bib41","doi-asserted-by":"crossref","first-page":"2128","DOI":"10.1016\/S0140-6736(04)16503-3","article-title":"Congenital adrenal hyperplasia caused by mutant P450 oxidoreductase and human androgen synthesis: analytical study","volume":"363","author":"Arlt","year":"2004","journal-title":"Lancet"},{"issue":"19","key":"2019122310485919900_bib42","doi-asserted-by":"crossref","first-page":"1182","DOI":"10.1056\/NEJM198511073131903","article-title":"Male pseudohermaphroditism due to multiple defects in steroid-biosynthetic microsomal mixed-function oxidases. A new variant of congenital adrenal hyperplasia","volume":"313","author":"Peterson","year":"1985","journal-title":"N Engl J Med"},{"issue":"2","key":"2019122310485919900_bib43","doi-asserted-by":"crossref","first-page":"95","DOI":"10.1002\/ajmg.10510","article-title":"Abnormal sterol metabolism in a patient with Antley-Bixler syndrome and ambiguous genitalia","volume":"110","author":"Kelley","year":"2002","journal-title":"Am J Med Genet"},{"issue":"3","key":"2019122310485919900_bib44","doi-asserted-by":"crossref","first-page":"269","DOI":"10.1016\/j.ymgme.2010.03.023","article-title":"Anorectal and urinary anomalies and aberrant retinoic acid metabolism in cytochrome P450 oxidoreductase deficiency","volume":"100","author":"Fukami","year":"2010","journal-title":"Mol Genet Metab"},{"issue":"1","key":"2019122310485919900_bib45","doi-asserted-by":"crossref","first-page":"66","DOI":"10.1016\/j.ydbio.2006.10.032","article-title":"Rescue of cytochrome P450 oxidoreductase (Por) mouse mutants reveals functions in vasculogenesis, brain and limb patterning linked to retinoic acid homeostasis","volume":"303","author":"Ribes","year":"2007","journal-title":"Dev Biol"},{"issue":"6","key":"2019122310485919900_bib46","doi-asserted-by":"crossref","first-page":"919","DOI":"10.1530\/EJE-10-0764","article-title":"Impaired hepatic drug and steroid metabolism in congenital adrenal hyperplasia due to P450 oxidoreductase deficiency","volume":"163","author":"Tomalik-Scharte","year":"2010","journal-title":"Eur J Endocrinol"},{"issue":"2","key":"2019122310485919900_bib47","doi-asserted-by":"crossref","first-page":"M1","DOI":"10.1530\/JME-17-0286","article-title":"Sulfation pathways: alternate steroid sulfation pathways targeted by LC\u2013MS\/MS analysis of disulfates: application to prenatal diagnosis of steroid synthesis disorders","volume":"61","author":"Pozo","year":"2018","journal-title":"J Mol Endocrinol"},{"issue":"5","key":"2019122310485919900_bib48","doi-asserted-by":"crossref","first-page":"1723","DOI":"10.1210\/jc.2008-2816","article-title":"Cytochrome P450 oxidoreductase deficiency: identification and characterization of biallelic mutations and genotype-phenotype correlations in 35 Japanese patients","volume":"94","author":"Fukami","year":"2009","journal-title":"J Clin Endocrinol Metab"},{"key":"2019122310485919900_bib49","first-page":"1993","article-title":"Cytochrome P450 oxidoreductase deficiency","author":"Idkowiak"},{"issue":"2","key":"2019122310485919900_bib50","doi-asserted-by":"crossref","first-page":"105","DOI":"10.1002\/ajmg.a.30171","article-title":"Prenatal diagnosis of P450 oxidoreductase deficiency (ORD): a disorder causing low pregnancy estriol, maternal and fetal virilization, and the Antley-Bixler syndrome phenotype","volume":"129A","author":"Shackleton","year":"2004","journal-title":"Am J Med Genet A"},{"issue":"3","key":"2019122310485919900_bib51","doi-asserted-by":"crossref","first-page":"E528","DOI":"10.1210\/jc.2012-3449","article-title":"Prenatal diagnosis of congenital adrenal hyperplasia caused by P450 oxidoreductase deficiency","volume":"98","author":"Reisch","year":"2013","journal-title":"J Clin Endocrinol Metab"},{"issue":"1\u20133","key":"2019122310485919900_bib52","doi-asserted-by":"crossref","first-page":"127","DOI":"10.1016\/0960-0760(93)90132-G","article-title":"Mass spectrometry in the diagnosis of steroid-related disorders and in hypertension research","volume":"45","author":"Shackleton","year":"1993","journal-title":"J Steroid Biochem Mol Biol"},{"issue":"1","key":"2019122310485919900_bib53","doi-asserted-by":"crossref","first-page":"101","DOI":"10.1530\/acta.0.1030101","article-title":"Difficulties in the diagnosis of congenital adrenal hyperplasia in early infancy: the 11\u03b2-hydroxylase defect","volume":"103","author":"Honour","year":"1983","journal-title":"Acta Endocrinol (Copenh)"},{"issue":"4","key":"2019122310485919900_bib54","doi-asserted-by":"crossref","first-page":"525","DOI":"10.1210\/er.2002-0050","article-title":"Molecular biology of the 3\u03b2-hydroxysteroid dehydrogenase\/\u03945-\u03944 isomerase gene family","volume":"26","author":"Simard","year":"2005","journal-title":"Endocr Rev"},{"issue":"2","key":"2019122310485919900_bib55","doi-asserted-by":"crossref","first-page":"74","DOI":"10.1016\/0039-128X(95)00193-T","article-title":"15\u03b2-Hydroxysteroids (part I). Steroids of the human perinatal period: the synthesis of 3\u03b2,15\u03b2,17\u03b1-trihydroxy-5-pregnen-20-one","volume":"61","author":"Reeder","year":"1996","journal-title":"Steroids"},{"issue":"3","key":"2019122310485919900_bib56","doi-asserted-by":"crossref","first-page":"799","DOI":"10.1172\/JCI115954","article-title":"Molecular genetics of steroid 5 alpha-reductase 2 deficiency","volume":"90","author":"Thigpen","year":"1992","journal-title":"J Clin Invest"},{"issue":"5","key":"2019122310485919900_bib57","first-page":"577","article-title":"Steroid 5\u03b1-reductase 2 deficiency","volume":"14","author":"Wilson","year":"1993","journal-title":"Endocr Rev"},{"key":"2019122310485919900_bib58","doi-asserted-by":"crossref","first-page":"206","DOI":"10.1016\/j.jsbmb.2016.05.020","article-title":"Steroid 5\u03b1-reductase 2 deficiency","volume":"163","author":"Mendonca","year":"2016","journal-title":"J Steroid Biochem Mol Biol"},{"issue":"1","key":"2019122310485919900_bib59","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1007\/s00431-010-1189-4","article-title":"Practical approach to steroid 5alpha-reductase type 2 deficiency","volume":"170","author":"Cheon","year":"2011","journal-title":"Eur J Pediatr"},{"issue":"2","key":"2019122310485919900_bib60","doi-asserted-by":"crossref","first-page":"296","DOI":"10.1210\/jc.2010-1024","article-title":"Phenotypical, biological, and molecular heterogeneity of 5\u03b1-reductase deficiency: an extensive international experience of 55 patients","volume":"96","author":"Maimoun","year":"2011","journal-title":"J Clin Endocrinol Metab"},{"issue":"1","key":"2019122310485919900_bib61","doi-asserted-by":"crossref","first-page":"43","DOI":"10.1111\/j.1365-2265.1985.tb00181.x","article-title":"Urinary steroid metabolites in subjects with male pseudohermaphroditism due to 5\u03b1-reductase deficiency","volume":"23","author":"Peterson","year":"1985","journal-title":"Clin Endocrinol (Oxf)"},{"issue":"2","key":"2019122310485919900_bib62","first-page":"560","article-title":"The identification of 5\u03b1-reductase-2 and 17\u03b2-hydroxysteroid dehydrogenase-3 gene defects in male pseudohermaphrodites from a Turkish kindred","volume":"83","author":"Can","year":"1998","journal-title":"J Clin Endocrinol Metab"},{"issue":"5","key":"2019122310485919900_bib63","doi-asserted-by":"crossref","first-page":"798","DOI":"10.1373\/clinchem.2012.196501","article-title":"Diagnosis of 5\u03b1-reductase 2 deficiency: is measurement of dihydrotestosterone essential","volume":"59","author":"Chan","year":"2013","journal-title":"Clin Chem"},{"issue":"5","key":"2019122310485919900_bib64","doi-asserted-by":"crossref","first-page":"380","DOI":"10.1159\/000324646","article-title":"Pitfalls in the diagnosis of 5\u03b1-reductase type 2 deficiency during early infancy","volume":"75","author":"Perry","year":"2011","journal-title":"Horm Res Paediatr"},{"issue":"1","key":"2019122310485919900_bib65","doi-asserted-by":"crossref","first-page":"34","DOI":"10.1038\/ng0594-34","article-title":"Male pseudohermaphroditism caused by mutations of testicular 17\u03b2-hydroxysteroid dehydrogenase 3","volume":"7","author":"Geissler","year":"1994","journal-title":"Nat Genet"},{"issue":"12","key":"2019122310485919900_bib66","first-page":"4713","article-title":"17\u03b2-Hydroxysteroid dehydrogenase-3 deficiency: diagnosis, phenotypic variability, population genetics, and worldwide distribution of ancient and de novo mutations","volume":"84","author":"Boehmer","year":"1999","journal-title":"J Clin Endocrinol Metab"},{"issue":"1","key":"2019122310485919900_bib67","doi-asserted-by":"crossref","first-page":"20","DOI":"10.1111\/j.1365-2265.2007.02829.x","article-title":"Phenotypic variability in 17\u03b2-hydroxysteroid dehydrogenase-3 deficiency and diagnostic pitfalls","volume":"67","author":"Lee","year":"2007","journal-title":"Clin Endocrinol (Oxf)"},{"issue":"5\u20136","key":"2019122310485919900_bib68","first-page":"623","article-title":"Pitfalls in hormonal diagnosis of 17-beta hydroxysteroid dehydrogenase III deficiency","volume":"28","author":"Khattab","year":"2015","journal-title":"J Pediatr Endocrinol Metab"},{"issue":"12","key":"2019122310485919900_bib69","doi-asserted-by":"crossref","first-page":"757","DOI":"10.1056\/NEJM198603203141206","article-title":"Congenital methemoglobinemia with a deficiency of cytochrome b5","volume":"314","author":"Hegesh","year":"1986","journal-title":"N Engl J Med"},{"issue":"3","key":"2019122310485919900_bib70","doi-asserted-by":"crossref","first-page":"994","DOI":"10.1210\/jc.2008-1745","article-title":"Isolated 17,20-lyase deficiency due to the cytochrome b5 mutation W27X","volume":"95","author":"Kok","year":"2010","journal-title":"J Clin Endocrinol Metab"},{"issue":"5","key":"2019122310485919900_bib71","doi-asserted-by":"crossref","first-page":"526","DOI":"10.1210\/er.2015-1036","article-title":"The regulation of steroid action by sulfation and desulfation","volume":"36","author":"Mueller","year":"2015","journal-title":"Endocr Rev"},{"issue":"6","key":"2019122310485919900_bib72","doi-asserted-by":"crossref","first-page":"1404","DOI":"10.1172\/JCI110391","article-title":"Stratum corneum lipids in disorders of cornification: increased cholesterol sulfate content of stratum corneum in recessive x-linked ichthyosis","volume":"68","author":"Williams","year":"1981","journal-title":"J Clin Invest"},{"issue":"3","key":"2019122310485919900_bib73","doi-asserted-by":"crossref","first-page":"480","DOI":"10.1016\/j.jaad.2009.04.028","article-title":"X-linked ichthyosis: an oculocutaneous genodermatosis","volume":"62","author":"Fernandes","year":"2010","journal-title":"J Am Acad Dermatol"},{"key":"2019122310485919900_bib74","doi-asserted-by":"crossref","first-page":"142","DOI":"10.1016\/j.mce.2016.08.019","article-title":"Role of steroid sulfatase in steroid homeostasis and characterization of the sulfated steroid pathway: evidence from steroid sulfatase deficiency","volume":"437","author":"S\u00e1nchez-Guijo","year":"2016","journal-title":"Mol Cell Endocrinol"},{"issue":"4","key":"2019122310485919900_bib75","doi-asserted-by":"crossref","first-page":"655","DOI":"10.1046\/j.1365-2133.1998.02180.x","article-title":"X-linked ichthyosis: relation between cholesterol sulphate, dehydroepiandrosterone sulphate and patient\u2019s age","volume":"138","author":"Delfino","year":"1998","journal-title":"Br J Dermatol"},{"issue":"4","key":"2019122310485919900_bib76","doi-asserted-by":"crossref","first-page":"385","DOI":"10.1111\/j.1365-2265.1985.tb01096.x","article-title":"Abnormal androgen and oestrogen metabolism in men with steroid sulphatase deficiency and recessive X-linked ichthyosis","volume":"23","author":"Lykkesfeldt","year":"1985","journal-title":"Clin Endocrinol (Oxf)"},{"issue":"6","key":"2019122310485919900_bib77","doi-asserted-by":"crossref","first-page":"2545","DOI":"10.1210\/jc.2015-4101","article-title":"Steroid sulfatase deficiency and androgen activation before and after puberty","volume":"101","author":"Idkowiak","year":"2016","journal-title":"J Clin Endocrinol Metab"},{"issue":"1","key":"2019122310485919900_bib78","doi-asserted-by":"crossref","first-page":"113","DOI":"10.1016\/0022-4731(86)90289-X","article-title":"Regulation of serum testosterone in men with steroid sulfatase deficiency: response to human chorionic gonadotropin","volume":"25","author":"Ruokonen","year":"1986","journal-title":"J Steroid Biochem"},{"issue":"4521","key":"2019122310485919900_bib79","doi-asserted-by":"crossref","first-page":"659","DOI":"10.1126\/science.6945674","article-title":"X-linked ichthyosis: increased blood cholesterol sulfate and electrophoretic mobility of low-density lipoprotein","volume":"214","author":"Epstein","year":"1981","journal-title":"Science"},{"issue":"9","key":"2019122310485919900_bib80","doi-asserted-by":"crossref","first-page":"1906","DOI":"10.1093\/clinchem\/35.9.1906","article-title":"Diagnosis of recessive X-linked ichthyosis: quantitative HPLC\/mass spectrometric analysis of plasma for cholesterol sulfate","volume":"35","author":"Shackleton","year":"1989","journal-title":"Clin Chem"},{"issue":"9","key":"2019122310485919900_bib81","doi-asserted-by":"crossref","first-page":"1843","DOI":"10.1194\/jlr.D061499","article-title":"Simultaneous quantification of cholesterol sulfate, androgen sulfates, and progestagen sulfates in human serum by LC-MS\/MS","volume":"56","author":"S\u00e1nchez-Guijo","year":"2015","journal-title":"J Lipid Res"},{"issue":"1","key":"2019122310485919900_bib82","doi-asserted-by":"crossref","first-page":"78","DOI":"10.1210\/jcem-49-1-78","article-title":"Gas chromatographic steroid analysis for diagnosis of placental sulfatase deficiency: a study of nine patients","volume":"49","author":"Taylor","year":"1979","journal-title":"J Clin Endocrinol Metab"},{"issue":"8","key":"2019122310485919900_bib83","doi-asserted-by":"crossref","first-page":"771","DOI":"10.1002\/pd.2284","article-title":"Maternal urine and serum steroid measurements to identify steroid sulfatase deficiency (STSD) in second trimester pregnancies","volume":"29","author":"Marcos","year":"2009","journal-title":"Prenat Diagn"},{"issue":"25","key":"2019122310485919900_bib84","doi-asserted-by":"crossref","first-page":"9724","DOI":"10.1074\/jbc.RA118.002248","article-title":"Human DHEA sulfation requires direct interaction between PAPS synthase 2 and DHEA sulfotransferase SULT2A1","volume":"293","author":"Mueller","year":"2018","journal-title":"J Biol Chem"},{"issue":"22","key":"2019122310485919900_bib85","doi-asserted-by":"crossref","first-page":"2310","DOI":"10.1056\/NEJMoa0810489","article-title":"Inactivating PAPSS2 mutations in a patient with premature pubarche","volume":"360","author":"Noordam","year":"2009","journal-title":"N Engl J Med"},{"issue":"6","key":"2019122310485919900_bib86","doi-asserted-by":"crossref","first-page":"1300","DOI":"10.1002\/ajmg.a.35906","article-title":"Spondyloepimetaphyseal dysplasia Pakistani type: expansion of the phenotype","volume":"161A","author":"T\u00fcys\u00fcz","year":"2013","journal-title":"Am J Med Genet A"},{"issue":"10","key":"2019122310485919900_bib87","doi-asserted-by":"crossref","first-page":"1381","DOI":"10.1002\/humu.22377","article-title":"Clinical and radiographic features of the autosomal recessive form of brachyolmia caused by PAPSS2 mutations","volume":"34","author":"Iida","year":"2013","journal-title":"Hum Mutat"},{"issue":"8","key":"2019122310485919900_bib88","doi-asserted-by":"crossref","first-page":"533","DOI":"10.1136\/jmedgenet-2012-101039","article-title":"PAPSS2 mutations cause autosomal recessive brachyolmia","volume":"49","author":"Miyake","year":"2012","journal-title":"J Med Genet"},{"issue":"4","key":"2019122310485919900_bib89","doi-asserted-by":"crossref","first-page":"E672","DOI":"10.1210\/jc.2014-3556","article-title":"PAPSS2 deficiency causes androgen excess via impaired DHEA sulfation\u2014in vitro and in vivo studies in a family harboring two novel PAPSS2 mutations","volume":"100","author":"Oostdijk","year":"2015","journal-title":"J Clin Endocrinol Metab"},{"issue":"10","key":"2019122310485919900_bib90","doi-asserted-by":"crossref","first-page":"4111","DOI":"10.1073\/pnas.1014934108","article-title":"Cortisone-reductase deficiency associated with heterozygous mutations in 11\u03b2-hydroxysteroid dehydrogenase type 1","volume":"108","author":"Lawson","year":"2011","journal-title":"Proc Natl Acad Sci USA"},{"issue":"4","key":"2019122310485919900_bib91","doi-asserted-by":"crossref","first-page":"434","DOI":"10.1038\/ng1214","article-title":"Mutations in the genes encoding 11\u03b2-hydroxysteroid dehydrogenase type 1 and hexose-6-phosphate dehydrogenase interact to cause cortisone reductase deficiency","volume":"34","author":"Draper","year":"2003","journal-title":"Nat Genet"},{"issue":"10","key":"2019122310485919900_bib92","doi-asserted-by":"crossref","first-page":"3827","DOI":"10.1210\/jc.2008-0743","article-title":"Steroid biomarkers and genetic studies reveal inactivating mutations in hexose-6-phosphate dehydrogenase in patients with cortisone reductase deficiency","volume":"93","author":"Lavery","year":"2008","journal-title":"J Clin Endocrinol Metab"},{"issue":"2","key":"2019122310485919900_bib93","doi-asserted-by":"crossref","first-page":"189","DOI":"10.1530\/EJE-11-0223","article-title":"Premature adrenarche: novel lessons from early onset androgen excess","volume":"165","author":"Idkowiak","year":"2011","journal-title":"Eur J Endocrinol"},{"issue":"11","key":"2019122310485919900_bib94","first-page":"3855","article-title":"Apparent cortisone reductase deficiency: a unique form of hypercortisolism","volume":"81","author":"Phillipov","year":"1996","journal-title":"J Clin Endocrinol Metab"},{"issue":"2","key":"2019122310485919900_bib95","doi-asserted-by":"crossref","first-page":"K19","DOI":"10.1530\/EJE-12-0628","article-title":"Novel H6PDH mutations in two girls with premature adrenarche: \u201capparent\u201d and \u201ctrue\u201d CRD can be differentiated by urinary steroid profiling","volume":"168","author":"Lavery","year":"2013","journal-title":"Eur J Endocrinol"},{"issue":"4","key":"2019122310485919900_bib96","doi-asserted-by":"crossref","first-page":"786","DOI":"10.1210\/jcem-50-4-786","article-title":"Hypertension in a four-year-old child: gas chromatographic and mass spectrometric evidence for deficient hepatic metabolism of steroids","volume":"50","author":"Shackleton","year":"1980","journal-title":"J Clin Endocrinol Metab"},{"issue":"1","key":"2019122310485919900_bib97","doi-asserted-by":"crossref","first-page":"340","DOI":"10.1172\/JCI113592","article-title":"Syndrome of apparent mineralocorticoid excess. A defect in the cortisol-cortisone shuttle","volume":"82","author":"Stewart","year":"1988","journal-title":"J Clin Invest"},{"issue":"6","key":"2019122310485919900_bib98","doi-asserted-by":"crossref","first-page":"701","DOI":"10.1111\/j.1365-2265.1985.tb00160.x","article-title":"Congenital 11\u03b2-hydroxysteroid dehydrogenase deficiency associated with juvenile hypertension: corticosteroid metabolite profiles of four patients and their families","volume":"22","author":"Shackleton","year":"1985","journal-title":"Clin Endocrinol (Oxf)"},{"issue":"4","key":"2019122310485919900_bib99","doi-asserted-by":"crossref","first-page":"193","DOI":"10.1016\/0039-128X(96)00012-8","article-title":"Apparent mineralocorticoid excess: type I and type II","volume":"61","author":"Mantero","year":"1996","journal-title":"Steroids"},{"issue":"5","key":"2019122310485919900_bib100","doi-asserted-by":"crossref","first-page":"605","DOI":"10.1046\/j.1365-2265.1996.00853.x","article-title":"Urinary free cortisone and the assessment of 11\u03b2-hydroxysteroid dehydrogenase activity in man","volume":"45","author":"Palermo","year":"1996","journal-title":"Clin Endocrinol (Oxf)"},{"issue":"4812","key":"2019122310485919900_bib101","doi-asserted-by":"crossref","first-page":"268","DOI":"10.1126\/science.3037703","article-title":"Cloning of human mineralocorticoid receptor complementary DNA: structural and functional kinship with the glucocorticoid receptor","volume":"237","author":"Arriza","year":"1987","journal-title":"Science"},{"issue":"6059","key":"2019122310485919900_bib102","doi-asserted-by":"crossref","first-page":"488","DOI":"10.1136\/bmj.1.6059.488","article-title":"Effect of eating liquorice on the renin-angiotensin aldosterone axis in normal subjects","volume":"1","author":"Epstein","year":"1977","journal-title":"BMJ"},{"issue":"17","key":"2019122310485919900_bib103","doi-asserted-by":"crossref","first-page":"1223","DOI":"10.1056\/NEJM199110243251706","article-title":"Licorice-induced hypermineralocorticoidism","volume":"325","author":"Farese","year":"1991","journal-title":"N Engl J Med"},{"issue":"7","key":"2019122310485919900_bib104","doi-asserted-by":"crossref","first-page":"518","DOI":"10.1007\/BF03349763","article-title":"Apparent mineralocorticoid excess syndromes","volume":"18","author":"Shimojo","year":"1995","journal-title":"J Endocrinol Invest"},{"issue":"4-6","key":"2019122310485919900_bib105","doi-asserted-by":"crossref","first-page":"501","DOI":"10.1016\/0960-0760(91)90269-B","article-title":"The cortisol-cortisone shuttle and hypertension","volume":"40","author":"Stewart","year":"1991","journal-title":"J Steroid Biochem Mol Biol"},{"issue":"4","key":"2019122310485919900_bib106","doi-asserted-by":"crossref","first-page":"1262","DOI":"10.1681\/ASN.2006111235","article-title":"Impaired protein stability of 11\u03b2-hydroxysteroid dehydrogenase type 2: a novel mechanism of apparent mineralocorticoid excess","volume":"18","author":"Atanasov","year":"2007","journal-title":"J Am Soc Nephrol"},{"issue":"5","key":"2019122310485919900_bib107","doi-asserted-by":"crossref","first-page":"757","DOI":"10.1210\/jcem-49-5-757","article-title":"A syndrome of apparent mineralocorticoid excess associated with defects in the peripheral metabolism of cortisol","volume":"49","author":"Ulick","year":"1979","journal-title":"J Clin Endocrinol Metab"},{"issue":"3","key":"2019122310485919900_bib108","doi-asserted-by":"crossref","first-page":"550","DOI":"10.1210\/jcem-63-3-550","article-title":"The syndrome of apparent mineralocorticoid excess: its association with 11\u03b2-dehydrogenase and 5\u03b2-reductase deficiency and some consequences for corticosteroid metabolism","volume":"63","author":"Monder","year":"1986","journal-title":"J Clin Endocrinol Metab"},{"issue":"4","key":"2019122310485919900_bib109","doi-asserted-by":"crossref","first-page":"799","DOI":"10.1210\/jcem-44-4-799","article-title":"An abnormality in steroid reductive metabolism in a hypertensive syndrome","volume":"44","author":"Ulick","year":"1977","journal-title":"J Clin Endocrinol Metab"},{"issue":"4","key":"2019122310485919900_bib110","doi-asserted-by":"crossref","first-page":"424","DOI":"10.1097\/00008480-199708000-00019","article-title":"Abnormalities of aldosterone synthesis and action in children","volume":"9","author":"White","year":"1997","journal-title":"Curr Opin Pediatr"},{"issue":"1\u20132","key":"2019122310485919900_bib111","doi-asserted-by":"crossref","first-page":"81","DOI":"10.1016\/j.mce.2003.10.013","article-title":"Aldosterone synthase deficiency and related disorders","volume":"217","author":"White","year":"2004","journal-title":"Mol Cell Endocrinol"},{"issue":"4","key":"2019122310485919900_bib112","doi-asserted-by":"crossref","first-page":"458","DOI":"10.1016\/j.ymgme.2007.11.009","article-title":"Aldosterone synthase deficiency caused by a homozygous L451F mutation in the CYP11B2 gene","volume":"93","author":"Nguyen","year":"2008","journal-title":"Mol Genet Metab"},{"issue":"8","key":"2019122310485919900_bib113","doi-asserted-by":"crossref","first-page":"3117","DOI":"10.1210\/jc.2008-0594","article-title":"A novel form of human Mendelian hypertension featuring nonglucocorticoid-remediable aldosteronism","volume":"93","author":"Geller","year":"2008","journal-title":"J Clin Endocrinol Metab"},{"issue":"1","key":"2019122310485919900_bib114","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1530\/JME-15-0324","article-title":"Mutated KCNJ5 activates the acute and chronic regulatory steps in aldosterone production","volume":"57","author":"Hattangady","year":"2016","journal-title":"J Mol Endocrinol"},{"issue":"1","key":"2019122310485919900_bib115","doi-asserted-by":"crossref","first-page":"R1","DOI":"10.1530\/EJE-17-0563","article-title":"Diagnosis of endocrine disease: 18-oxocortisol and 18-hydroxycortisol: is there clinical utility of these steroids","volume":"178","author":"Lenders","year":"2018","journal-title":"Eur J Endocrinol"},{"issue":"10","key":"2019122310485919900_bib116","doi-asserted-by":"crossref","first-page":"813","DOI":"10.7326\/0003-4819-116-10-813","article-title":"Glucocorticoid-remediable aldosteronism in a large kindred: clinical spectrum and diagnosis using a characteristic biochemical phenotype","volume":"116","author":"Rich","year":"1992","journal-title":"Ann Intern Med"},{"issue":"4","key":"2019122310485919900_bib117","doi-asserted-by":"crossref","first-page":"357","DOI":"10.1016\/j.ymgme.2010.04.016","article-title":"Five novel mutations in CYP11B2 gene detected in patients with aldosterone synthase deficiency type I: Functional characterization and structural analyses","volume":"100","author":"Nguyen","year":"2010","journal-title":"Mol Genet Metab"},{"issue":"4","key":"2019122310485919900_bib118","doi-asserted-by":"crossref","first-page":"762","DOI":"10.1530\/acta.0.0810762","article-title":"Multicomponent gas chromatographic analysis of urinary steroids excreted by an infant with a defect in aldosterone biosynthesis","volume":"81","author":"Shackleton","year":"1976","journal-title":"Acta Endocrinol (Copenh)"},{"issue":"2","key":"2019122310485919900_bib119","doi-asserted-by":"crossref","first-page":"325","DOI":"10.1210\/jcem-54-2-325","article-title":"Analysis of steroids in urine for differentiation of pseudohypoaldosteronism and aldosterone biosynthetic defect","volume":"54","author":"Honour","year":"1982","journal-title":"J Clin Endocrinol Metab"},{"issue":"6","key":"2019122310485919900_bib120","doi-asserted-by":"crossref","first-page":"427","DOI":"10.1136\/adc.55.6.427","article-title":"Pseudohypoaldosteronism","volume":"55","author":"Dillon","year":"1980","journal-title":"Arch Dis Child"},{"issue":"3","key":"2019122310485919900_bib121","doi-asserted-by":"crossref","first-page":"149","DOI":"10.1007\/BF01963554","article-title":"Severe hypoaldosteronism due to corticosterone methyl oxidase type II deficiency in two boys: metabolic and gas chromatography-mass spectrometry studies","volume":"150","author":"Hauffa","year":"1991","journal-title":"Eur J Pediatr"},{"issue":"6","key":"2019122310485919900_bib122","doi-asserted-by":"crossref","first-page":"491","DOI":"10.1016\/0009-9120(94)00048-Z","article-title":"Corticosterone methyl oxidase type II (CMO II) deficiency: biochemical approach to diagnosis","volume":"27","author":"Yong","year":"1994","journal-title":"Clin Biochem"},{"issue":"5","key":"2019122310485919900_bib123","first-page":"1622","article-title":"Multisteroid analysis in children with terminal aldosterone biosynthesis defects","volume":"80","author":"Peter","year":"1995","journal-title":"J Clin Endocrinol Metab"},{"issue":"6357","key":"2019122310485919900_bib124","doi-asserted-by":"crossref","first-page":"262","DOI":"10.1038\/355262a0","article-title":"A chimaeric 11\u03b2-hydroxylase\/aldosterone synthase gene causes glucocorticoid-remediable aldosteronism and human hypertension","volume":"355","author":"Lifton","year":"1992","journal-title":"Nature"},{"issue":"2","key":"2019122310485919900_bib125","doi-asserted-by":"crossref","first-page":"225","DOI":"10.1210\/jcem-60-2-225","article-title":"The constant plasma 18-hydroxycorticosterone to aldosterone ratio: an expression of the efficacy of corticosterone methyloxidase type II activity in disorders with variable aldosterone production","volume":"60","author":"Kater","year":"1985","journal-title":"J Clin Endocrinol Metab"},{"issue":"1","key":"2019122310485919900_bib126","doi-asserted-by":"crossref","first-page":"225","DOI":"10.1210\/jcem-62-1-225","article-title":"Biochemical diagnosis and management of corticosterone methyl oxidase type II deficiency","volume":"62","author":"Lee","year":"1986","journal-title":"J Clin Endocrinol Metab"},{"issue":"6","key":"2019122310485919900_bib127","first-page":"1415","article-title":"The biochemical phenotypes of two inborn errors in the biosynthesis of aldosterone","volume":"74","author":"Ulick","year":"1992","journal-title":"J Clin Endocrinol Metab"},{"issue":"11","key":"2019122310485919900_bib128","doi-asserted-by":"crossref","first-page":"1842","DOI":"10.1681\/ASN.2010050457","article-title":"Mechanisms of type I and type II pseudohypoaldosteronism","volume":"21","author":"Furgeson","year":"2010","journal-title":"J Am Soc Nephrol"},{"key":"2019122310485919900_bib129","doi-asserted-by":"crossref","first-page":"86","DOI":"10.1159\/000342508","article-title":"Pseudohypoaldosteronism","volume":"24","author":"Riepe","year":"2013","journal-title":"Endocr Dev"},{"issue":"1","key":"2019122310485919900_bib130","doi-asserted-by":"crossref","first-page":"33","DOI":"10.1016\/0009-8981(95)06034-7","article-title":"Urinary steroid profile of a newborn suffering from pseudohypoaldosteronism","volume":"236","author":"Wolthers","year":"1995","journal-title":"Clin Chim Acta"},{"issue":"1","key":"2019122310485919900_bib131","doi-asserted-by":"crossref","first-page":"72","DOI":"10.1159\/000281404","article-title":"Hook effect: a pitfall leading to misdiagnosis of hypoaldosteronism in an infant with pseudohypoaldosteronism","volume":"74","author":"Ak\u0131n","year":"2010","journal-title":"Horm Res Paediatr"},{"issue":"17","key":"2019122310485919900_bib132","doi-asserted-by":"crossref","first-page":"8327","DOI":"10.1073\/pnas.89.17.8327","article-title":"Glucocorticoid-suppressible hyperaldosteronism results from hybrid genes created by unequal crossovers between CYP11B1 and CYP11B2","volume":"89","author":"Pascoe","year":"1992","journal-title":"Proc Natl Acad Sci USA"},{"issue":"11","key":"2019122310485919900_bib133","first-page":"3570","article-title":"Evaluation of the dexamethasone suppression test for the diagnosis of glucocorticoid-remediable aldosteronism","volume":"82","author":"Litchfield","year":"1997","journal-title":"J Clin Endocrinol Metab"},{"issue":"3 Pt 2","key":"2019122310485919900_bib134","doi-asserted-by":"crossref","first-page":"688","DOI":"10.1161\/01.HYP.38.3.688","article-title":"Serum 18-hydroxycortisol in primary aldosteronism, hypertension, and normotensives","volume":"38","author":"Mosso","year":"2001","journal-title":"Hypertension"},{"issue":"3","key":"2019122310485919900_bib135","doi-asserted-by":"crossref","first-page":"265","DOI":"10.1097\/00060793-199606000-00012","article-title":"Glucocorticoid-remediable aldosteronism","volume":"3","author":"Litchfield","year":"1996","journal-title":"Curr Opin Endocrinol Diabetes"},{"issue":"9996","key":"2019122310485919900_bib136","doi-asserted-by":"crossref","first-page":"913","DOI":"10.1016\/S0140-6736(14)61375-1","article-title":"Cushing\u2019s syndrome","volume":"386","author":"Lacroix","year":"2015","journal-title":"Lancet"},{"issue":"5","key":"2019122310485919900_bib137","doi-asserted-by":"crossref","first-page":"1526","DOI":"10.1210\/jc.2008-0125","article-title":"The diagnosis of Cushing\u2019s syndrome: an Endocrine Society clinical practice guideline","volume":"93","author":"Nieman","year":"2008","journal-title":"J Clin Endocrinol Metab"},{"issue":"22","key":"2019122310485919900_bib138","doi-asserted-by":"crossref","first-page":"2105","DOI":"10.1056\/NEJMoa1304603","article-title":"ARMC5 mutations in macronodular adrenal hyperplasia with Cushing\u2019s syndrome","volume":"369","author":"Assi\u00e9","year":"2013","journal-title":"N Engl J Med"},{"issue":"11","key":"2019122310485919900_bib139","doi-asserted-by":"crossref","first-page":"1019","DOI":"10.1056\/NEJMoa1310359","article-title":"Constitutive activation of PKA catalytic subunit in adrenal Cushing\u2019s syndrome","volume":"370","author":"Beuschlein","year":"2014","journal-title":"N Engl J Med"},{"issue":"5","key":"2019122310485919900_bib140","doi-asserted-by":"crossref","first-page":"396","DOI":"10.1016\/S2213-8587(13)70211-0","article-title":"Cardiovascular events and mortality in patients with adrenal incidentalomas that are either non-secreting or associated with intermediate phenotype or subclinical Cushing\u2019s syndrome: a 15-year retrospective study","volume":"2","author":"Di Dalmazi","year":"2014","journal-title":"Lancet Diabetes Endocrinol"},{"issue":"12","key":"2019122310485919900_bib141","doi-asserted-by":"crossref","first-page":"4462","DOI":"10.1210\/jc.2014-3007","article-title":"Cortisol as a marker for increased mortality in patients with incidental adrenocortical adenomas","volume":"99","author":"Debono","year":"2014","journal-title":"J Clin Endocrinol Metab"},{"issue":"4","key":"2019122310485919900_bib142","doi-asserted-by":"crossref","first-page":"M61","DOI":"10.1530\/EJE-15-0272","article-title":"Subclinical hypercortisolism: a state, a syndrome, or a disease","volume":"173","author":"Di Dalmazi","year":"2015","journal-title":"Eur J Endocrinol"},{"issue":"8","key":"2019122310485919900_bib143","doi-asserted-by":"crossref","first-page":"3545","DOI":"10.1210\/jc.2018-01926","article-title":"Novel GC-MS\/MS technique reveals a complex steroid fingerprint of subclinical hypercortisolism in adrenal incidentalomas","volume":"104","author":"H\u00e1na","year":"2019","journal-title":"J Clin Endocrinol Metab"},{"issue":"4","key":"2019122310485919900_bib144","doi-asserted-by":"crossref","first-page":"647","DOI":"10.1530\/EJE-08-0707","article-title":"Atherosclerotic risk factors and complications in patients with non-functioning adrenal adenomas treated with or without adrenalectomy: a long-term follow-up study","volume":"160","author":"Sereg","year":"2009","journal-title":"Eur J Endocrinol"},{"issue":"3","key":"2019122310485919900_bib145","doi-asserted-by":"crossref","first-page":"827","DOI":"10.1210\/jc.2013-3527","article-title":"Long-term follow-up in adrenal incidentalomas: an Italian multicenter study","volume":"99","author":"Morelli","year":"2014","journal-title":"J Clin Endocrinol Metab"},{"issue":"8","key":"2019122310485919900_bib146","doi-asserted-by":"crossref","first-page":"1816","DOI":"10.1002\/jbmr.398","article-title":"Risk of new vertebral fractures in patients with adrenal incidentaloma with and without subclinical hypercortisolism: a multicenter longitudinal study","volume":"26","author":"Morelli","year":"2011","journal-title":"J Bone Miner Res"},{"issue":"2","key":"2019122310485919900_bib147","doi-asserted-by":"crossref","first-page":"107","DOI":"10.7326\/M18-3630","article-title":"Natural history of adrenal incidentalomas with and without mild autonomous cortisol excess: a systematic review and meta-analysis","volume":"171","author":"Elhassan","year":"2019","journal-title":"Ann Intern Med"},{"issue":"8","key":"2019122310485919900_bib148","doi-asserted-by":"crossref","first-page":"e93136","DOI":"10.1172\/jci.insight.93136","article-title":"Steroid metabolome analysis reveals prevalent glucocorticoid excess in primary aldosteronism","volume":"2","author":"Arlt","year":"2017","journal-title":"JCI Insight"},{"issue":"12","key":"2019122310485919900_bib149","doi-asserted-by":"crossref","first-page":"1824","DOI":"10.1373\/clinchem.2017.271106","article-title":"High-resolution, accurate-mass (HRAM) mass spectrometry urine steroid profiling in the diagnosis of adrenal disorders","volume":"63","author":"Hines","year":"2017","journal-title":"Clin Chem"},{"issue":"3","key":"2019122310485919900_bib150","doi-asserted-by":"crossref","first-page":"586","DOI":"10.1373\/clinchem.2017.282582","article-title":"Plasma steroid metabolome profiling for diagnosis and subtyping patients with Cushing syndrome","volume":"64","author":"Eisenhofer","year":"2018","journal-title":"Clin Chem"},{"issue":"12","key":"2019122310485919900_bib151","first-page":"3617","article-title":"11beta-Hydroxysteroid dehydrogenase activity in Cushing\u2019s syndrome: explaining the mineralocorticoid excess state of the ectopic adrenocorticotropin syndrome","volume":"80","author":"Stewart","year":"1995","journal-title":"J Clin Endocrinol Metab"},{"issue":"5","key":"2019122310485919900_bib152","first-page":"963","article-title":"Cortisol inactivation overload: a mechanism of mineralocorticoid hypertension in the ectopic adrenocorticotropin syndrome","volume":"74","author":"Ulick","year":"1992","journal-title":"J Clin Endocrinol Metab"},{"issue":"4","key":"2019122310485919900_bib153","doi-asserted-by":"crossref","first-page":"743","DOI":"10.1002\/jbmr.3033","article-title":"Risk of fracture in primary aldosteronism: a population-based cohort study","volume":"32","author":"Wu","year":"2017","journal-title":"J Bone Miner Res"},{"issue":"4","key":"2019122310485919900_bib154","doi-asserted-by":"crossref","first-page":"168","DOI":"10.1007\/s12672-015-0224-3","article-title":"Diagnostic value of urinary steroid profiling in the evaluation of adrenal tumors","volume":"6","author":"Kerkhofs","year":"2015","journal-title":"Horm Cancer"},{"issue":"5-6","key":"2019122310485919900_bib155","doi-asserted-by":"crossref","first-page":"327","DOI":"10.1007\/s12672-016-0267-0","article-title":"Different types of urinary steroid profiling obtained by high-performance liquid chromatography and gas chromatography-mass spectrometry in patients with adrenocortical carcinoma","volume":"7","author":"Velikanova","year":"2016","journal-title":"Horm Cancer"},{"issue":"5","key":"2019122310485919900_bib156","doi-asserted-by":"crossref","first-page":"1889","DOI":"10.1210\/jc.2015-4061","article-title":"The management of primary aldosteronism: case detection, diagnosis, and treatment: an Endocrine Society clinical practice guideline","volume":"101","author":"Funder","year":"2016","journal-title":"J Clin Endocrinol Metab"},{"issue":"6018","key":"2019122310485919900_bib157","doi-asserted-by":"crossref","first-page":"768","DOI":"10.1126\/science.1198785","article-title":"K+ channel mutations in adrenal aldosterone-producing adenomas and hereditary hypertension","volume":"331","author":"Choi","year":"2011","journal-title":"Science"},{"issue":"9","key":"2019122310485919900_bib158","doi-asserted-by":"crossref","first-page":"1050","DOI":"10.1038\/ng.2695","article-title":"Somatic and germline CACNA1D calcium channel mutations in aldosterone-producing adenomas and primary aldosteronism","volume":"45","author":"Scholl","year":"2013","journal-title":"Nat Genet"},{"key":"2019122310485919900_bib159","doi-asserted-by":"crossref","first-page":"e06315","DOI":"10.7554\/eLife.06315","article-title":"Recurrent gain of function mutation in calcium channel CACNA1H causes early-onset hypertension with primary aldosteronism","volume":"4","author":"Scholl","year":"2015","journal-title":"eLife"},{"issue":"3","key":"2019122310485919900_bib160","doi-asserted-by":"crossref","first-page":"349","DOI":"10.1038\/s41588-018-0048-5","article-title":"CLCN2 chloride channel mutations in familial hyperaldosteronism type II","volume":"50","author":"Scholl","year":"2018","journal-title":"Nat Genet"},{"issue":"2","key":"2019122310485919900_bib161","doi-asserted-by":"crossref","first-page":"104","DOI":"10.1038\/nrendo.2012.230","article-title":"Role of KCNJ5 in familial and sporadic primary aldosteronism","volume":"9","author":"Mulatero","year":"2013","journal-title":"Nat Rev Endocrinol"},{"issue":"1","key":"2019122310485919900_bib162","doi-asserted-by":"crossref","first-page":"188","DOI":"10.1161\/HYPERTENSIONAHA.113.01733","article-title":"Somatic ATP1A1, ATP2B3, and KCNJ5 mutations in aldosterone-producing adenomas","volume":"63","author":"Williams","year":"2014","journal-title":"Hypertension"},{"issue":"4","key":"2019122310485919900_bib163","doi-asserted-by":"crossref","first-page":"440","DOI":"10.1038\/ng.2550","article-title":"Somatic mutations in ATP1A1 and ATP2B3 lead to aldosterone-producing adenomas and secondary hypertension","volume":"45","author":"Beuschlein","year":"2013","journal-title":"Nat Genet"},{"issue":"9","key":"2019122310485919900_bib164","doi-asserted-by":"crossref","first-page":"1055","DOI":"10.1038\/ng.2716","article-title":"Somatic mutations in ATP1A1 and CACNA1D underlie a common subtype of adrenal hypertension","volume":"45","author":"Azizan","year":"2013","journal-title":"Nat Genet"},{"issue":"4","key":"2019122310485919900_bib165","doi-asserted-by":"crossref","first-page":"1124","DOI":"10.3390\/ijms19041124","article-title":"Comparative genomics and transcriptome profiling in primary aldosteronism","volume":"19","author":"Aristizabal Prada","year":"2018","journal-title":"Int J Mol Sci"},{"issue":"8","key":"2019122310485919900_bib166","doi-asserted-by":"crossref","first-page":"1243","DOI":"10.1016\/j.jacc.2005.01.015","article-title":"Evidence for an increased rate of cardiovascular events in patients with primary aldosteronism","volume":"45","author":"Milliez","year":"2005","journal-title":"J Am Coll Cardiol"},{"issue":"1","key":"2019122310485919900_bib167","doi-asserted-by":"crossref","first-page":"80","DOI":"10.1001\/archinternmed.2007.33","article-title":"Cardiovascular outcomes in patients with primary aldosteronism after treatment","volume":"168","author":"Catena","year":"2008","journal-title":"Arch Intern Med"},{"issue":"4","key":"2019122310485919900_bib168","doi-asserted-by":"crossref","first-page":"1125","DOI":"10.1210\/jc.2008-2116","article-title":"Cardiovascular and cerebrovascular comorbidities of hypokalemic and normokalemic primary aldosteronism: results of the German Conn\u2019s Registry","volume":"94","author":"Born-Frontsberg","year":"2009","journal-title":"J Clin Endocrinol Metab"},{"issue":"1","key":"2019122310485919900_bib169","doi-asserted-by":"crossref","first-page":"62","DOI":"10.1161\/HYPERTENSIONAHA.113.01316","article-title":"Long-term control of arterial hypertension and regression of left ventricular hypertrophy with treatment of primary aldosteronism","volume":"62","author":"Rossi","year":"2013","journal-title":"Hypertension"},{"issue":"3","key":"2019122310485919900_bib170","doi-asserted-by":"crossref","first-page":"618","DOI":"10.1161\/HYPERTENSIONAHA.112.197111","article-title":"Observational study mortality in treated primary aldosteronism: the German Conn\u2019s registry","volume":"60","author":"Reincke","year":"2012","journal-title":"Hypertension"},{"issue":"8","key":"2019122310485919900_bib171","doi-asserted-by":"crossref","first-page":"E1272","DOI":"10.1210\/jc.2010-2785","article-title":"18-Oxocortisol measurement in adrenal vein sampling as a biomarker for subclassifying primary aldosteronism","volume":"96","author":"Nakamura","year":"2011","journal-title":"J Clin Endocrinol Metab"},{"issue":"3","key":"2019122310485919900_bib172","doi-asserted-by":"crossref","first-page":"514","DOI":"10.1373\/clinchem.2015.251199","article-title":"Mass spectrometry\u2013based adrenal and peripheral venous steroid profiling for subtyping primary aldosteronism","volume":"62","author":"Eisenhofer","year":"2016","journal-title":"Clin Chem"},{"issue":"9\u201310","key":"2019122310485919900_bib173","first-page":"1771","article-title":"Hypersecretion of a new corticosteroid, 18-hydroxycortisol in two types of adrenocortical hypertension","volume":"4","author":"Ulick","year":"1982","journal-title":"Clin Exp Hypertens A"},{"issue":"12","key":"2019122310485919900_bib174","doi-asserted-by":"crossref","first-page":"4543","DOI":"10.1210\/jc.2018-00617","article-title":"Cortisol excess in patients with primary aldosteronism impacts left ventricular hypertrophy","volume":"103","author":"Adolf","year":"2018","journal-title":"J Clin Endocrinol Metab"},{"issue":"2","key":"2019122310485919900_bib175","doi-asserted-by":"crossref","first-page":"454","DOI":"10.1210\/jc.2005-1733","article-title":"Prevalence and characteristics of the metabolic syndrome in primary aldosteronism","volume":"91","author":"Fallo","year":"2006","journal-title":"J Clin Endocrinol Metab"},{"issue":"5","key":"2019122310485919900_bib176","doi-asserted-by":"crossref","first-page":"665","DOI":"10.1530\/EJE-15-0450","article-title":"Increased prevalence of diabetes mellitus and the metabolic syndrome in patients with primary aldosteronism of the German Conn\u2019s Registry","volume":"173","author":"Hanslik","year":"2015","journal-title":"Eur J Endocrinol"},{"issue":"8","key":"2019122310485919900_bib177","doi-asserted-by":"crossref","first-page":"1698","DOI":"10.1097\/HJH.0000000000001361","article-title":"Risk of new-onset diabetes mellitus in primary aldosteronism: a population study over 5 years","volume":"35","author":"Wu","year":"2017","journal-title":"J Hypertens"},{"issue":"12","key":"2019122310485919900_bib178","doi-asserted-by":"crossref","first-page":"2548","DOI":"10.1097\/HJH.0000000000001550","article-title":"The impact of Connshing\u2019s syndrome\u2014mild cortisol excess in primary aldosteronism drives diabetes risk","volume":"35","author":"Beuschlein","year":"2017","journal-title":"J Hypertens"},{"issue":"8","key":"2019122310485919900_bib179","doi-asserted-by":"crossref","first-page":"3192","DOI":"10.1210\/jc.2019-00299","article-title":"Impaired glucose metabolism in primary aldosteronism is associated with cortisol cosecretion","volume":"104","author":"Gerards","year":"2019","journal-title":"J Clin Endocrinol Metab"},{"issue":"6","key":"2019122310485919900_bib180","doi-asserted-by":"crossref","first-page":"2513","DOI":"10.1210\/jc.2012-3934","article-title":"Aldosterone excess impairs first phase insulin secretion in primary aldosteronism","volume":"98","author":"Fischer","year":"2013","journal-title":"J Clin Endocrinol Metab"},{"issue":"10","key":"2019122310485919900_bib181","doi-asserted-by":"crossref","first-page":"2217","DOI":"10.1002\/jbmr.1660","article-title":"Bone involvement in aldosteronism","volume":"27","author":"Salcuni","year":"2012","journal-title":"J Bone Miner Res"},{"issue":"6","key":"2019122310485919900_bib182","doi-asserted-by":"crossref","first-page":"E878","DOI":"10.1210\/jc.2010-2723","article-title":"Psychological assessment of primary aldosteronism: a controlled study","volume":"96","author":"Sonino","year":"2011","journal-title":"J Clin Endocrinol Metab"},{"issue":"1","key":"2019122310485919900_bib183","doi-asserted-by":"crossref","first-page":"26","DOI":"10.3109\/15622975.2012.665480","article-title":"Gender differences in anxiety and depressive symptoms in patients with primary hyperaldosteronism: a cross-sectional study","volume":"15","author":"Apostolopoulou","year":"2014","journal-title":"World J Biol Psychiatry"},{"issue":"2","key":"2019122310485919900_bib184","doi-asserted-by":"crossref","first-page":"E243","DOI":"10.1210\/jc.2010-1222","article-title":"Clinical, biochemical, and molecular characterization of macronodular adrenocortical hyperplasia of the zona reticularis: a new syndrome","volume":"96","author":"Ghayee","year":"2011","journal-title":"J Clin Endocrinol Metab"},{"issue":"2","key":"2019122310485919900_bib185","doi-asserted-by":"crossref","first-page":"G1","DOI":"10.1530\/EJE-16-0467","article-title":"Management of adrenal incidentalomas: European Society of Endocrinology clinical practice guideline in collaboration with the European Network for the Study of Adrenal Tumors","volume":"175","author":"Fassnacht","year":"2016","journal-title":"Eur J Endocrinol"},{"issue":"2","key":"2019122310485919900_bib186","first-page":"637","article-title":"A survey on adrenal incidentaloma in Italy","volume":"85","author":"Mantero","year":"2000","journal-title":"J Clin Endocrinol Metab"},{"key":"2019122310485919900_bib187","doi-asserted-by":"crossref","first-page":"51","DOI":"10.1530\/EJE-16-0461","article-title":"Management of endocrine disease: imaging for the diagnosis of malignancy in incidentally discovered adrenal masses: a systematic review and meta-analysis European Journal of Endocrinology","volume":"175","author":"Dinnes","year":"2016","journal-title":"Eur J Endocrinol"},{"issue":"2","key":"2019122310485919900_bib188","doi-asserted-by":"crossref","first-page":"282","DOI":"10.1210\/er.2013-1029","article-title":"Adrenocortical carcinoma","volume":"35","author":"Else","year":"2014","journal-title":"Endocr Rev"},{"issue":"12","key":"2019122310485919900_bib189","doi-asserted-by":"crossref","first-page":"1836","DOI":"10.1373\/clinchem.2017.277624","article-title":"A 13-steroid serum panel based on LC-MS\/MS: use in detection of adrenocortical carcinoma","volume":"63","author":"Taylor","year":"2017","journal-title":"Clin Chem"},{"issue":"2","key":"2019122310485919900_bib190","doi-asserted-by":"crossref","first-page":"117","DOI":"10.1530\/EJE-18-0782","article-title":"Plasma steroid metabolome profiling for the diagnosis of adrenocortical carcinoma","volume":"180","author":"Schweitzer","year":"2019","journal-title":"Eur J Endocrinol"},{"issue":"1","key":"2019122310485919900_bib191","doi-asserted-by":"crossref","first-page":"161","DOI":"10.1210\/jc.2012-2851","article-title":"Mitotane therapy in adrenocortical cancer induces CYP3A4 and inhibits 5\u03b1-reductase, explaining the need for personalized glucocorticoid and androgen replacement","volume":"98","author":"Chortis","year":"2013","journal-title":"J Clin Endocrinol Metab"}],"container-title":["Endocrine Reviews"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/academic.oup.com\/edrv\/advance-article-pdf\/doi\/10.1210\/er.2018-00262\/28920623\/er.2018-00262.pdf","content-type":"application\/pdf","content-version":"am","intended-application":"syndication"},{"URL":"http:\/\/academic.oup.com\/edrv\/article-pdf\/40\/6\/1605\/30843306\/er.2018-00262.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"http:\/\/academic.oup.com\/edrv\/article-pdf\/40\/6\/1605\/30843306\/er.2018-00262.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,1,12]],"date-time":"2021-01-12T18:16:55Z","timestamp":1610475415000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/edrv\/article\/40\/6\/1605\/5530226"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2019,7,11]]},"references-count":191,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2019,12,1]]}},"URL":"https:\/\/doi.org\/10.1210\/er.2018-00262","relation":{},"ISSN":["0163-769X","1945-7189"],"issn-type":[{"value":"0163-769X","type":"print"},{"value":"1945-7189","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2019,12]]},"published":{"date-parts":[[2019,7,11]]}}}