Statural Growth in 31 Japanese Patients with SHOX Haploinsufficiency: Support for a Disadvantageous Effect of Gonadal Estrogens
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Accession number;04A0381481
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| Title;Statural Growth in 31 Japanese Patients with SHOX Haploinsufficiency: Support for a Disadvantageous Effect of Gonadal Estrogens |
| Author;
FUKAMI M
(National Res. Inst. Child Health And Dev., Tokyo, Jpn)
NISHI Y
(Dep. Pediatrics, Hiroshima Red-cross Hospital, Hiroshima, Jpn)
HASEGAWA Y
(Tokyo Metropolitan Kiyose Children's Hospital, Tokyo, Jpn)
MIYOSHI Y
(Osaka Univ. School Of Medicine, Osaka, Jpn)
OKABE T
(Toyama Municipal Hospital, Toyama, Jpn)
HAGA N
(Shizuoka Children's Hospital, Shizuoka, Jpn)
NAGAI T
(Dokkyo Univ. School Of Medicine Koshigaya Hospital, Koshigaya, Jpn)
TANAKA T
(National Res. Center For Child Health And Dev., Tokyo, Jpn)
OGATA T
(National Res. Inst. Child Health And Dev., Tokyo, Jpn)
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Journal Title;Endocr J
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Journal Code:F0625A
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ISSN:0918-8959
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VOL.51;NO.2;PAGE.197-200(2004)
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| Figure&Table&Reference;FIG.2, REF.11 |
| Pub. Country;Japan |
| Language;English |
| Abstract;Although gonadal estrogens are known to facilitate the development of skeletal lesion in SHOX haploinsufficiency, controversy exists as to whether gonadal estrogens are disadvantageous to pubertal growth. To clarify this matter, we analyzed growth pattern in 31 Japanese patients with a normal karyotype and molecularly confirmed SHOX haploinsufficiency. The mean height SD score at the diagnosis of SHOX haploinsufficiency was similar between patients identified in childhood and those identified in adulthood (-2.7.+-.0.8 [n=15] vs. -2.4.+-.0.7 [n=16], P=0.36), and was significantly lower in patients identified by the studies for short stature than in those ascertained by the familial studies of the probands both in childhood (-3.0.+-.0.6 [n=11] vs. -1.8.+-.0.5 [n=4], P=0.0051) and in adulthood (-3.0.+-.0.9 [n=5] vs. -2.2.+-.0.5 [n=11], P=0.040). Analysis of longitudinal paired growth data obtained in seven females showed a significantly different mean height SD score between childhood and adulthood (-2.3.+-.0.5 vs. -2.9.+-.0.8, P=0.0060). Te results imply that gonadal estrogens have a deleterious effect on pubertal growth in SHOX haploinsufficiency, and that the growth disadvantage is recognizable by longitudinal rather than cross-sectional growth studies. (author abst.) |
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