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Titolo:
Metacarpophalangeal pattern profile and bone age in Rett syndrome: Furtherradiological clues to the diagnosis
Autore:
Leonard, H; Thomson, M; Glasson, E; Fyfe, S; Leonard, S; Ellaway, C; Christodoulou, J; Bower, C;
Indirizzi:
TVW Telethon Inst Child Hlth Res, W Perth, WA 6872, Australia TVW TelethonInst Child Hlth Res W Perth WA Australia 6872 872, Australia Disabil Serv Commiss, W Perth, Australia Disabil Serv Commiss W Perth Australia Serv Commiss, W Perth, Australia Princess Margaret Hosp, Dept Radiol, Subiaco, WA, Australia Princess Margaret Hosp Subiaco WA Australia diol, Subiaco, WA, Australia Univ Western Australia, Dept Anat & Human Biol, Perth, WA 6009, Australia Univ Western Australia Perth WA Australia 6009 Perth, WA 6009, Australia Curtinaliav Technol, Sch Biomed Sci, Dept Human Biol, Perth, WA 6001, Austr Curtin Univ Technol Perth WA Australia 6001 n Biol, Perth, WA 6001, Austr Univ Sydney, Dept Paediat & Child Hlth, Sydney, NSW 2006, Australia Univ Sydney Sydney NSW Australia 2006 d Hlth, Sydney, NSW 2006, Australia Royalustraliara Hosp Children, Western Sydney Genet Program, Sydney, NSW, A Royal Alexandra Hosp Children Sydney NSW Australia ogram, Sydney, NSW, A
Titolo Testata:
AMERICAN JOURNAL OF MEDICAL GENETICS
fascicolo: 2, volume: 83, anno: 1999,
pagine: 88 - 95
SICI:
0148-7299(19990312)83:2<88:MPPABA>2.0.ZU;2-M
Fonte:
ISI
Lingua:
ENG
Soggetto:
DELINEATION; ASYMMETRY; HAND;
Keywords:
Rett syndrome; metacarpophalangeal pattern profile; bone age; diagnosis; classical; atypical;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
35
Recensione:
Indirizzi per estratti:
Indirizzo: Leonard, H TVW Telethon Inst Child Hlth Res, POB 855, W Perth, WA 6872, Australia TVW Telethon Inst Child Hlth Res POB 855 W Perth WA Australia 6872
Citazione:
H. Leonard et al., "Metacarpophalangeal pattern profile and bone age in Rett syndrome: Furtherradiological clues to the diagnosis", AM J MED G, 83(2), 1999, pp. 88-95

Abstract

Hand radiographs of 100 girls representing 73% of the known Australian population of girls with Rett syndrome, age 20 years or less, were available for this study. Control radiographs were matched for age, sex, and laterality. Bone age was assessed against standard radiographs in Greulich and Pyle [1959: Radiographic Atlas of Skeletal Development of the Hands and Wrist, 2nd ed.]. A metacarpophalangeal pattern (MCPP) profile comparing the relative lengths of the hand bones with mean population norms by age was produced by converting the length of each of the 19 metacarpal and phalangeal bones into a Z score. In girls less than 15 years old, bone age was more advancedin Rett syndrome than in age-matched control girls (left hand P = 0.03, right hand 0.004), but was most advanced in the younger group and normalized with age. In Rett syndrome, the mean Z score for the 19 metacarpal and phalangeal bones was 1.0 in children under 5 years, -0.27 in those aged 5-11 years, and -1.7 in those aged 12 years and over. This variation between age groups was much greater than in the controls. The dips in the MCPP profile occurred at MC2 and D1, and the peaks at M5, P5, and M4, An MCPP profile mayprovide an additional aid to diagnosis in cases of Rett syndrome where allthe criteria are not met, but in children under age 5 years, advanced boneage may be more helpful as a marker. Am. J, Med, Genet. 83:88-95, 1999, (C) 1999 Wiley-Liss, Inc.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 27/01/20 alle ore 01:23:11