{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T01:39:19Z","timestamp":1790818759036,"version":"4.1.0"},"reference-count":86,"publisher":"Oxford University Press (OUP)","issue":"6","content-domain":{"domain":["eje.bioscientifica.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2018,12]]},"abstract":"<jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>Non-idiopathic CPP is caused by acquired or congenital hypothalamic lesions visible on MRI or is associated with various complex genetic and\/or syndromic disorders. This study investigated the different types and prevalence of non-isolated CPP phenotypes.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Design and Methods<\/jats:title>\n                    <jats:p>This observational cohort study included all patients identified as having non-idiopathic CPP in the database of a single academic pediatric care center over a period of 11.5 years. Patients were classified on the basis of MRI findings for the CNS as having either hypothalamic lesions or complex syndromic phenotypes without structural lesions of the hypothalamus.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      In total, 63 consecutive children (42 girls and 21 boys) with non-isolated CPP were identified. Diverse diseases were detected, and the hypothalamic lesions visible on MRI (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u200928, 45% of cases) included hamartomas (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u200917; either isolated or with an associated syndromic phenotype), optic gliomas\n                      <jats:italic>(n<\/jats:italic>\n                      \u2009=\u20098; with or without neurofibromatosis type 1), malformations (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u20093) with interhypothalamic adhesions (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u20092; isolated or associated with syndromic CNS midline abnormalities, such as optic nerve hypoplasia, ectopic posterior pituitary) or arachnoid cysts (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u20091). The patients with non-structural hypothalamic lesions (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u200935, 55% of cases) had narcolepsy (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u20099), RASopathies (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u20094), encephalopathy or autism spectrum disorders with or without chromosomal abnormalities (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u200915) and other complex syndromic disorders (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u20097).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>Our findings suggest that a large proportion (55%) of patients with non-isolated probable non-idiopathic CPP may have complex disorders without structural hypothalamic lesions on MRI. Future studies should explore the pathophysiological relevance of the mechanisms underlying CPP in these disorders.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1530\/eje-18-0613","type":"journal-article","created":{"date-parts":[[2018,10,2]],"date-time":"2018-10-02T07:01:08Z","timestamp":1538463668000},"page":"373-380","update-policy":"https:\/\/doi.org\/10.1530\/crossmarkpolicy-1","source":"Crossref","is-referenced-by-count":20,"title":["High prevalence of syndromic disorders in patients with non-isolated central precocious puberty"],"prefix":"10.1093","volume":"179","author":[{"given":"Selmen","family":"Wannes","sequence":"first","affiliation":[{"name":"1Assistance Publique-H\u00f4pitaux de Paris, Robert Debr\u00e9 University Hospital, Endocrinology-Diabetology Department, Reference Center for Endocrine Growth and Developmental Diseases, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Monique","family":"Elmaleh-Berg\u00e8s","sequence":"additional","affiliation":[{"name":"2Assistance Publique-H\u00f4pitaux de Paris, Pediatric Radiology Department, Robert Debr\u00e9 University Hospital, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dominique","family":"Simon","sequence":"additional","affiliation":[{"name":"1Assistance Publique-H\u00f4pitaux de Paris, Robert Debr\u00e9 University Hospital, Endocrinology-Diabetology Department, Reference Center for Endocrine Growth and Developmental Diseases, Paris, France"},{"name":"3Assistance Publique-H\u00f4pitaux de Paris, Biochemistry Unit, Robert Debr\u00e9 University Hospital, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Delphine","family":"Z\u00e9naty","sequence":"additional","affiliation":[{"name":"1Assistance Publique-H\u00f4pitaux de Paris, Robert Debr\u00e9 University Hospital, Endocrinology-Diabetology Department, Reference Center for Endocrine Growth and Developmental Diseases, Paris, France"},{"name":"5Institut National de la Sant\u00e9 et de la Recherche M\u00e9dicale (INSERM), UMR 1141, DHU Promoting Research Oriented Towards Early Central Nervous System Therapies (PROTECT), Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laetitia","family":"Martinerie","sequence":"additional","affiliation":[{"name":"1Assistance Publique-H\u00f4pitaux de Paris, Robert Debr\u00e9 University Hospital, Endocrinology-Diabetology Department, Reference Center for Endocrine Growth and Developmental Diseases, Paris, France"},{"name":"4Paris Diderot University, Sorbonne Paris Cite, Paris, France"},{"name":"5Institut National de la Sant\u00e9 et de la Recherche M\u00e9dicale (INSERM), UMR 1141, DHU Promoting Research Oriented Towards Early Central Nervous System Therapies (PROTECT), Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Caroline","family":"Storey","sequence":"additional","affiliation":[{"name":"1Assistance Publique-H\u00f4pitaux de Paris, Robert Debr\u00e9 University Hospital, Endocrinology-Diabetology Department, Reference Center for Endocrine Growth and Developmental Diseases, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Georges","family":"Gelwane","sequence":"additional","affiliation":[{"name":"1Assistance Publique-H\u00f4pitaux de Paris, Robert Debr\u00e9 University Hospital, Endocrinology-Diabetology Department, Reference Center for Endocrine Growth and Developmental Diseases, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anne","family":"Paulsen","sequence":"additional","affiliation":[{"name":"1Assistance Publique-H\u00f4pitaux de Paris, Robert Debr\u00e9 University Hospital, Endocrinology-Diabetology Department, Reference Center for Endocrine Growth and Developmental Diseases, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emmanuel","family":"Ecosse","sequence":"additional","affiliation":[{"name":"1Assistance Publique-H\u00f4pitaux de Paris, Robert Debr\u00e9 University Hospital, Endocrinology-Diabetology Department, Reference Center for Endocrine Growth and Developmental Diseases, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicolas","family":"De Roux","sequence":"additional","affiliation":[{"name":"3Assistance Publique-H\u00f4pitaux de Paris, Biochemistry Unit, Robert Debr\u00e9 University Hospital, Paris, France"},{"name":"4Paris Diderot University, Sorbonne Paris Cite, Paris, France"},{"name":"5Institut National de la Sant\u00e9 et de la Recherche M\u00e9dicale (INSERM), UMR 1141, DHU Promoting Research Oriented Towards Early Central Nervous System Therapies (PROTECT), Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jean Claude","family":"Carel","sequence":"additional","affiliation":[{"name":"1Assistance Publique-H\u00f4pitaux de Paris, Robert Debr\u00e9 University Hospital, Endocrinology-Diabetology Department, Reference Center for Endocrine Growth and Developmental Diseases, Paris, France"},{"name":"4Paris Diderot University, Sorbonne Paris Cite, Paris, France"},{"name":"5Institut National de la Sant\u00e9 et de la Recherche M\u00e9dicale (INSERM), UMR 1141, DHU Promoting Research Oriented Towards Early Central Nervous System Therapies (PROTECT), Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Juliane","family":"L\u00e9ger","sequence":"additional","affiliation":[{"name":"1Assistance Publique-H\u00f4pitaux de Paris, Robert Debr\u00e9 University Hospital, Endocrinology-Diabetology Department, Reference Center for Endocrine Growth and Developmental Diseases, Paris, France"},{"name":"4Paris Diderot University, Sorbonne Paris Cite, Paris, France"},{"name":"5Institut National 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