{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,31]],"date-time":"2026-08-31T14:28:49Z","timestamp":1788186529292,"version":"build-2803163510"},"reference-count":29,"publisher":"MDPI AG","issue":"9","license":[{"start":{"date-parts":[[2022,5,4]],"date-time":"2022-05-04T00:00:00Z","timestamp":1651622400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"NIH U54","award":["HD06122"],"award-info":[{"award-number":["HD06122"]}]},{"name":"NIH U54","award":["RR019478"],"award-info":[{"award-number":["RR019478"]}]},{"name":"Prader\u2013Willi Syndrome Association USA","award":["HD06122"],"award-info":[{"award-number":["HD06122"]}]},{"name":"Prader\u2013Willi Syndrome Association USA","award":["RR019478"],"award-info":[{"award-number":["RR019478"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Prader\u2013Willi syndrome (PWS) is a complex genetic disorder with three genetic classes. Patients with PWS are characterized by severe hypotonia, developmental delay, behavioral problems, learning disabilities and morbid obesity in early childhood if untreated. Data were collected through Rare Disease Clinical Research Network (RDCRN) from four study centers which evaluated patients with PWS. The Behavior Assessment System for Children 2nd edition (BASC-2) was chosen to provide behavioral assessment. Data from 330 participants ((64% 15q11-q13 deletion (DEL), 36% maternal disomy 15 (UPD)) were separated into three age groups and analyzed, 68% of whom were still actively receiving recombinant human growth hormone (rhGH) treatment. When comparing the BASC results by molecular subtype, parent-reported aggression was higher for the deletion than for the UPD cohort (p = 0.007). Participants who were on rhGH treatment showed lower scores for parent-reported hyperactivity and aggression (p = 0.04, 0.04, respectively), and a trend for anger control (p = 0.06) and teacher-reported attention problems and aggression (p = 0.01, 0.004, respectively). Additional adjusted analyses were undertaken and significant differences were noted in the GH versus non-GH treated groups for only teacher-reported aggression, which increased in the No GH treated patient group (p = 0.03). This study showed documented differences in PWS behavior by molecular class and rhGH treatment. RhGH therapy may be beneficial for certain behaviors in patients with PWS; however, observed differences need more studies for confirmation in the future.<\/jats:p>","DOI":"10.3390\/jcm11092572","type":"journal-article","created":{"date-parts":[[2022,5,4]],"date-time":"2022-05-04T10:49:30Z","timestamp":1651661370000},"page":"2572","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["Molecular Classes and Growth Hormone Treatment Effects on Behavior and Emotion in Patients with Prader\u2013Willi Syndrome"],"prefix":"10.3390","volume":"11","author":[{"given":"Ranim","family":"Mahmoud","sequence":"first","affiliation":[{"name":"Department of Pediatrics, University of California, Irvine, CA 92697, USA"},{"name":"Department of Pediatrics, Faculty of Medicine, Mansoura University, Mansoura 35516, Egypt"},{"name":"Childrens Hospital of Orange County, Orange, CA 92868, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Heidi D.","family":"Swanson","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, University of California, Irvine, CA 92697, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2911-0524","authenticated-orcid":false,"given":"Merlin G.","family":"Butler","sequence":"additional","affiliation":[{"name":"Departments of Psychiatry & Behavioral Sciences and Pediatrics, University of Kansas Medical Center, Kansas City, KS 66160, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pamela","family":"Flodman","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, University of California, Irvine, CA 92697, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"June-Anne","family":"Gold","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, University of California, Irvine, CA 92697, USA"},{"name":"Childrens Hospital of Orange County, Orange, CA 92868, USA"},{"name":"Department of Pediatrics, Loma Linda University Medical School, Loma Linda, CA 92350, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jennifer L.","family":"Miller","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, College of Medicine, University of Florida, Gainesville, FL 32610, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elizabeth","family":"Roof","sequence":"additional","affiliation":[{"name":"Vanderbilt Kennedy Center for Research on Human Development, Vanderbilt University, Nashville, TN 37235, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kathryn","family":"Osann","sequence":"additional","affiliation":[{"name":"Department of Statistics, University of California, Irvine, CA 92697, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elisabeth","family":"Dykens","sequence":"additional","affiliation":[{"name":"Vanderbilt Kennedy Center for Research on Human Development, Vanderbilt University, Nashville, TN 37235, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9612-773X","authenticated-orcid":false,"given":"Daniel J.","family":"Driscoll","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, College of Medicine, University of Florida, Gainesville, FL 32610, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1567-4449","authenticated-orcid":false,"given":"Virginia","family":"Kimonis","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, University of California, Irvine, CA 92697, USA"},{"name":"Childrens Hospital of Orange County, Orange, CA 92868, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2022,5,4]]},"reference":[{"key":"ref_1","unstructured":"Driscoll, D.J., Miller, J.L., Schwartz, S., Cassidy, S.B., Adam, M.P., Ardinger, H.H., Pagon, R.A., Wallace, S.E., Bean, L.J.H., and Gripp, K.W. 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