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特发性青少年脊柱侧弯和马凡综合征中的皮肤胶原蛋白。

Skin collagen in idiopathic adolescent scoliosis and Marfan's syndrome.

作者信息

Francis M J, Sanderson M C, Smith R

出版信息

Clin Sci Mol Med. 1976 Nov;51(5):467-74. doi: 10.1042/cs0510467.

DOI:10.1042/cs0510467
PMID:991544
Abstract
  1. Skin collagen from fifteen patients with idiopathic adolescent scoliosis, three with congenital scoliosis, and fourteen patients with various degrees of Marfan's syndrome has been examined. 2. The stability of a polymeric collagen fraction, extracted from the skin of these patients, to depolymerization, has been compared with that from thirty-one matched control subjects. 3. The mean polymeric collagen stability was significantly reduced (P less than 0-01) in the group of fifteen patients with idiopathic adolescent scoliosis. The individual reductions in stability were greater in the younger patients and no reduction was found in two patients aged 19 years who had a mature skeleton. 4. The mean stability of polymeric skin collagen from the group of patients with Marfan's syndrome was not significantly abnormal, although there were individual low values. 5. Polymeric collagen of low stability was present in individual patients from each clinical group. Instability of collagen (from whatever cause) at a time of rapid growth may contribute to the high incidence of scoliosis in adolescent girls. In boys, adolescent scoliosis is less common and the maximum growth rate occurs at a time when collagen stability is less reduced.
摘要
  1. 对15例特发性青少年脊柱侧凸患者、3例先天性脊柱侧凸患者以及14例不同程度马方综合征患者的皮肤胶原蛋白进行了检测。2. 从这些患者皮肤中提取的聚合胶原蛋白组分对解聚的稳定性,已与31名匹配的对照受试者的进行了比较。3. 15例特发性青少年脊柱侧凸患者组中,聚合胶原蛋白的平均稳定性显著降低(P小于0.01)。年轻患者个体稳定性的降低幅度更大,而两名骨骼成熟的19岁患者未发现稳定性降低。4. 马方综合征患者组皮肤聚合胶原蛋白的平均稳定性虽无显著异常,但有个别低值。5. 每个临床组的个别患者中均存在低稳定性的聚合胶原蛋白。在快速生长时期胶原蛋白(无论何种原因)的不稳定性,可能导致青春期女孩脊柱侧凸的高发病率。在男孩中,青少年脊柱侧凸较少见,且最大生长速率发生在胶原蛋白稳定性降低较少的时候。

相似文献

1
Skin collagen in idiopathic adolescent scoliosis and Marfan's syndrome.特发性青少年脊柱侧弯和马凡综合征中的皮肤胶原蛋白。
Clin Sci Mol Med. 1976 Nov;51(5):467-74. doi: 10.1042/cs0510467.
2
Collagen metabolism of the skin in Marfan's syndrome.
Clin Chim Acta. 1968 Sep;21(3):321-6. doi: 10.1016/0009-8981(68)90062-4.
3
[Study of collagen abnormality in Marfan's syndrome].
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Solubility of spinal ligament collagen in idiopathic and secondary scoliosis.特发性和继发性脊柱侧弯中脊柱韧带胶原蛋白的溶解性
Clin Orthop Relat Res. 1983 Jul-Aug(177):294-301.
5
Collagen changes in congenital and idiopathic scoliosis.先天性和特发性脊柱侧弯中的胶原蛋白变化
Acta Orthop Scand. 1980 Apr;51(2):271-4. doi: 10.3109/17453678008990798.
6
[Acetabular protrusion in Marfan's disease].[马凡氏综合征中的髋臼突出]
Chir Pediatr. 1985;26(3):153-61.
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[A case report of spontaneous pneumothorax in Marfan's syndrome and an immunohistochemical study of type I collagen in lung tissue].
Nihon Kyobu Shikkan Gakkai Zasshi. 1991 Sep;29(9):1186-9.
8
Collagen abnormalities in idiopathic adolescent scoliosis.特发性青少年脊柱侧凸中的胶原异常
Calcif Tissue Res. 1977 May;22 Suppl:381-4. doi: 10.1007/BF02064106.
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Evolving phenotype of Marfan's syndrome.马方综合征不断演变的表型。
Arch Dis Child. 1997 Jan;76(1):41-6. doi: 10.1136/adc.76.1.41.
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Polymeric collagen of skin in osteogenesis imperfecta, homocystinuria and Ehlers-Danlos and Marfan syndromes.成骨不全、同型胱氨酸尿症、埃勒斯-当洛综合征和马凡综合征中皮肤的聚合胶原蛋白。
Birth Defects Orig Artic Ser. 1975;11(6):15-21.

引用本文的文献

1
Homozygosity for autosomal dominant Marfan syndrome.常染色体显性马凡综合征的纯合性
J Med Genet. 1984 Jun;21(3):173-7. doi: 10.1136/jmg.21.3.173.
2
Adolescent idiopathic scoliosis.青少年特发性脊柱侧弯
West J Med. 1988 Feb;148(2):182-91.
3
Unilateral microfibrillar abnormalities in a case of asymmetric Marfan syndrome.不对称性马方综合征病例中的单侧微纤维异常
Am J Hum Genet. 1990 Apr;46(4):661-71.
4
Diseases of the collagen molecule.胶原分子疾病。
J Clin Pathol Suppl (R Coll Pathol). 1978;12:82-94.