{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,24]],"date-time":"2026-07-24T03:02:32Z","timestamp":1784862152617,"version":"3.55.0"},"reference-count":28,"publisher":"SAGE Publications","issue":"3","license":[{"start":{"date-parts":[[2020,10,8]],"date-time":"2020-10-08T00:00:00Z","timestamp":1602115200000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JHD"],"published-print":{"date-parts":[[2020,10,8]]},"abstract":"<jats:p>Background: The gene (Huntingtin or HTT) causing Huntington\u2019s disease (HD) is vital for development and is expressed throughout the brain and body lifelong. The mutant form (mHTT) may influence growth and development. Objective: To determine the impact of mHTT on human measures of growth, including height, weight, and body mass index (BMI), between child and adolescent carriers of mHTT and control peers. Methods: Children ages 6\u201318 years of age (n\u200a=\u200a186) at risk for HD were enrolled in the KidsHD study. For research purposes only, genetic testing was performed to classify participants as Gene-Expanded (GE\u200a=\u200a78) or as Gene Non-Expanded (GNE\u200a=\u200a108). Outcome measures included height, weight, and body mass index (BMI). Mixed models were used to determine if non-linear age trends differed between groups for BMI, height, and weight. Results: Differences were seen in the trajectory of BMI in which the GE group reached a plateau in late adolescence with no further increase, compared with a nearly linear increase in the GNE group. There was a significant sex interaction pattern where GE males were taller than GNE males in adolescence, in the presence of similar weight. In contrast, GE females weighed significantly less than their GNE counterparts in adolescence, in the presence of similar height. Conclusion: Measures of growth are abnormal in child and adolescent carriers of mHTT, decades before HD onset. Although further studies are needed for replication, the current findings suggest that developmental aberrations may be systemic and a vital part of disease pathology.<\/jats:p>","DOI":"10.3233\/jhd-200407","type":"journal-article","created":{"date-parts":[[2020,9,4]],"date-time":"2020-09-04T12:33:23Z","timestamp":1599222803000},"page":"245-251","source":"Crossref","is-referenced-by-count":7,"title":["Developmental Trajectory of Height, Weight, and BMI in Children and Adolescents at Risk for Huntington\u2019s Disease: Effect of mHTT on Growth"],"prefix":"10.1177","volume":"9","author":[{"given":"Alexander","family":"Tereshchenko","sequence":"first","affiliation":[{"name":"Department of Psychiatry, University of Iowa Carver College of Medicine, Iowa City, IA, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ellen","family":"van der Plas","sequence":"additional","affiliation":[{"name":"Department of Psychiatry, University of Iowa Carver College of Medicine, Iowa City, IA, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Katherine D.","family":"Mathews","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, University of Iowa Carver College of Medicine, Iowa City, IA, USA"},{"name":"Department of Neurology, University of Iowa Carver College of Medicine, Iowa City, IA, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eric","family":"Epping","sequence":"additional","affiliation":[{"name":"Department of Psychiatry, University of Iowa Carver College of Medicine, Iowa City, IA, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amy L.","family":"Conrad","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, University of Iowa Carver College of Medicine, Iowa City, IA, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Douglas R.","family":"Langbehn","sequence":"additional","affiliation":[{"name":"Department of Psychiatry, University of Iowa Carver College of Medicine, Iowa City, IA, USA"},{"name":"University of Iowa College of Public Health Administration, Iowa City, IA, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peg","family":"Nopoulos","sequence":"additional","affiliation":[{"name":"Department of Psychiatry, University of Iowa Carver College of Medicine, Iowa City, IA, USA"},{"name":"Department of Pediatrics, University of Iowa Carver College of Medicine, Iowa City, IA, USA"},{"name":"Department of Neurology, University of Iowa Carver College of Medicine, Iowa City, IA, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"179","reference":[{"issue":"10","key":"10.3233\/JHD-200407_ref1","doi-asserted-by":"crossref","first-page":"e1021","DOI":"10.1212\/WNL.0000000000008066","article-title":"Abnormal brain development in child and adolescent carriers of mutant huntingtin","volume":"93","author":"van der Plas","year":"2019","journal-title":"Neurology"},{"issue":"7","key":"10.3233\/JHD-200407_ref2","doi-asserted-by":"crossref","first-page":"668","DOI":"10.1212\/WNL.0b013e3182648b65","article-title":"Measures of growth in children at risk for Huntington disease","volume":"79","author":"Lee","year":"2012","journal-title":"Neurology"},{"issue":"5","key":"10.3233\/JHD-200407_ref3","doi-asserted-by":"crossref","first-page":"385","DOI":"10.1212\/WNL.0b013e3181b04aa2","article-title":"Dietary intake in adults at risk for Huntington disease: analysis of PHAROS research participants","volume":"73","author":"Marder","year":"2009","journal-title":"Neurology"},{"issue":"19","key":"10.3233\/JHD-200407_ref4","doi-asserted-by":"crossref","first-page":"1506","DOI":"10.1212\/01.wnl.0000334276.09729.0e","article-title":"Weight loss in Huntington disease increases with higher CAG repeat number","volume":"71","author":"Aziz","year":"2008","journal-title":"Neurology"},{"issue":"7","key":"10.3233\/JHD-200407_ref5","doi-asserted-by":"crossref","first-page":"751","DOI":"10.1002\/ajmg.b.31232","article-title":"Indexing disease progression at study entry with individuals at-risk for Huntington disease","volume":"156B","author":"Zhang","year":"2011","journal-title":"Am J Med Genet B Neuropsychiatr Genet"},{"issue":"2","key":"10.3233\/JHD-200407_ref6","first-page":"136","article-title":"Huntington Study Group","volume":"11","author":"Unified Huntington\u2019s Disease Rating Scale: reliability and consistency","year":"1996","journal-title":"Mov Disord"},{"key":"10.3233\/JHD-200407_ref7","doi-asserted-by":"crossref","unstructured":"Hollingshead AB , Redlich FC . Social Class and Mental Illness. New York: John Wiley & Sons; 1958.","DOI":"10.1037\/10645-000"},{"issue":"11","key":"10.3233\/JHD-200407_ref8","doi-asserted-by":"crossref","first-page":"1135","DOI":"10.1016\/S1474-4422(15)00177-5","article-title":"Treating the whole body in Huntington\u2019s disease","volume":"14","author":"Carroll","year":"2015","journal-title":"Lancet Neurol"},{"issue":"1","key":"10.3233\/JHD-200407_ref9","doi-asserted-by":"crossref","first-page":"90","DOI":"10.1016\/j.bbr.2006.12.004","article-title":"Sex differences in behavior and striatal ascorbate release in the 140 CAG knock-in mouse model of Huntington\u2019s disease","volume":"178","author":"Dorner","year":"2007","journal-title":"Behav Brain Res"},{"issue":"2","key":"10.3233\/JHD-200407_ref10","doi-asserted-by":"crossref","first-page":"127","DOI":"10.1016\/j.neulet.2011.11.063","article-title":"Huntington\u2019s disease knock-in male mice show specific anxiety-like behaviour and altered neuronal maturation","volume":"507","author":"Orvoen","year":"2012","journal-title":"Neurosci Lett"},{"issue":"2-3","key":"10.3233\/JHD-200407_ref11","doi-asserted-by":"crossref","first-page":"223","DOI":"10.1016\/j.brainresbull.2011.09.011","article-title":"Profiles of motor and cognitive impairment in the transgenic rat model of Huntington\u2019s disease","volume":"88","author":"Fielding","year":"2012","journal-title":"Brain Res Bull"},{"issue":"19","key":"10.3233\/JHD-200407_ref12","doi-asserted-by":"crossref","first-page":"3869","DOI":"10.1093\/hmg\/ddt242","article-title":"A role of mitochondrial complex II defects in genetic models of Huntington\u2019s disease expressing N-terminal fragments of mutant huntingtin","volume":"22","author":"Damiano","year":"2013","journal-title":"Hum Mol Genet"},{"issue":"1","key":"10.3233\/JHD-200407_ref13","doi-asserted-by":"crossref","first-page":"52","DOI":"10.1016\/j.bbadis.2009.07.012","article-title":"Mitochondria in Huntington\u2019s disease","volume":"1802","author":"Damiano","year":"2010","journal-title":"Biochim Biophys Acta"},{"issue":"1","key":"10.3233\/JHD-200407_ref14","doi-asserted-by":"crossref","first-page":"127","DOI":"10.1016\/j.expneurol.2011.05.024","article-title":"Bioenergetic dysfunction in Huntington\u2019s disease human cybrids","volume":"231","author":"Ferreira","year":"2011","journal-title":"Exp Neurol"},{"key":"10.3233\/JHD-200407_ref15","doi-asserted-by":"crossref","first-page":"51","DOI":"10.1016\/j.nbd.2015.09.008","article-title":"Mitochondrial dynamics and quality control in Huntington\u2019s disease","volume":"90","author":"Guedes-Dias","year":"2016","journal-title":"Neurobiol Dis"},{"issue":"3","key":"10.3233\/JHD-200407_ref16","doi-asserted-by":"crossref","first-page":"227","DOI":"10.1007\/s10863-010-9287-6","article-title":"Mitochondrial bioenergetics and dynamics in Huntington\u2019s disease: tripartite synapses and selective striatal degeneration","volume":"42","author":"Oliveira","year":"2010","journal-title":"J Bioenerg Biomembr"},{"issue":"1","key":"10.3233\/JHD-200407_ref17","doi-asserted-by":"crossref","first-page":"27","DOI":"10.1002\/ajmg.1425","article-title":"Mitochondria and degenerative disorders","volume":"106","author":"Orth","year":"2001","journal-title":"Am J Med Genet"},{"issue":"2","key":"10.3233\/JHD-200407_ref18","doi-asserted-by":"crossref","first-page":"493","DOI":"10.1172\/JCI45691","article-title":"Energy deficit in Huntington disease: why it matters","volume":"121","author":"Mochel","year":"2011","journal-title":"J Clin Invest"},{"issue":"3","key":"10.3233\/JHD-200407_ref19","doi-asserted-by":"crossref","first-page":"313","DOI":"10.2174\/1389450114666131124140704","article-title":"Current therapeutic advances in patients and experimental models of Huntington\u2019s disease","volume":"15","author":"Brett","year":"2014","journal-title":"Curr Drug Targets"},{"issue":"10","key":"10.3233\/JHD-200407_ref20","doi-asserted-by":"crossref","first-page":"1218","DOI":"10.2174\/1389450111007011218","article-title":"Mitochondrial-associated metabolic changes and neurodegeneration in Huntington\u2019s disease - from clinical features to the bench","volume":"11","author":"Rosenstock","year":"2010","journal-title":"Curr Drug Targets"},{"issue":"3","key":"10.3233\/JHD-200407_ref21","doi-asserted-by":"crossref","first-page":"479","DOI":"10.1002\/ana.25007","article-title":"Body weight is a robust predictor of clinical progression in Huntington disease","volume":"82","author":"van der Burg","year":"2017","journal-title":"Ann Neurol"},{"issue":"11","key":"10.3233\/JHD-200407_ref22","doi-asserted-by":"crossref","first-page":"2783","DOI":"10.1523\/JNEUROSCI.0106-08.2008","article-title":"N-terminal mutant huntingtin associates with mitochondria and impairs mitochondrial trafficking","volume":"28","author":"Orr","year":"2008","journal-title":"J Neurosci"},{"issue":"1","key":"10.3233\/JHD-200407_ref23","doi-asserted-by":"crossref","first-page":"62","DOI":"10.1016\/j.bbadis.2009.06.008","article-title":"Impaired mitochondrial trafficking in Huntington\u2019s disease","volume":"1802","author":"Li","year":"2010","journal-title":"Biochim Biophys Acta"},{"issue":"2","key":"10.3233\/JHD-200407_ref24","doi-asserted-by":"crossref","first-page":"388","DOI":"10.1016\/j.nbd.2005.12.007","article-title":"Mutant huntingtin aggregates impair mitochondrial movement and trafficking in cortical neurons","volume":"22","author":"Chang","year":"2006","journal-title":"Neurobiol Dis"},{"issue":"2","key":"10.3233\/JHD-200407_ref25","doi-asserted-by":"crossref","first-page":"691","DOI":"10.1016\/j.expneurol.2007.12.026","article-title":"The metabolic profile of early Huntington\u2019s disease\u2013a combined human and transgenic mouse study","volume":"210","author":"Goodman","year":"2008","journal-title":"Exp Neurol"},{"issue":"19","key":"10.3233\/JHD-200407_ref26","doi-asserted-by":"crossref","first-page":"2871","DOI":"10.1093\/hmg\/ddi319","article-title":"HD CAG repeat implicates a dominant property of huntingtin in mitochondrial energy metabolism","volume":"14","author":"Seong","year":"2005","journal-title":"Hum Mol Genet"},{"issue":"1","key":"10.3233\/JHD-200407_ref27","doi-asserted-by":"crossref","first-page":"130","DOI":"10.1002\/mds.23258","article-title":"Low anaerobic threshold and increased skeletal muscle lactate production in subjects with Huntington\u2019s disease","volume":"26","author":"Ciammola","year":"2011","journal-title":"Mov Disord"},{"issue":"12","key":"10.3233\/JHD-200407_ref28","doi-asserted-by":"crossref","first-page":"2068","DOI":"10.1038\/sj.cdd.4401967","article-title":"Increased apoptosis, Huntingtin inclusions and altered differentiation in muscle cell cultures from Huntington\u2019s disease subjects","volume":"13","author":"Ciammola","year":"2006","journal-title":"Cell Death Differ"}],"container-title":["Journal of Huntington's Disease"],"original-title":[],"link":[{"URL":"https:\/\/content.iospress.com\/download?id=10.3233\/JHD-200407","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,4,29]],"date-time":"2026-04-29T08:51:04Z","timestamp":1777452664000},"score":1,"resource":{"primary":{"URL":"https:\/\/journals.sagepub.com\/doi\/full\/10.3233\/JHD-200407"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,10,8]]},"references-count":28,"journal-issue":{"issue":"3"},"URL":"https:\/\/doi.org\/10.3233\/jhd-200407","relation":{},"ISSN":["1879-6397","1879-6400"],"issn-type":[{"value":"1879-6397","type":"print"},{"value":"1879-6400","type":"electronic"}],"subject":[],"published":{"date-parts":[[2020,10,8]]}}}