THE NATURAL-HISTORY OF HETEROTOPIC OSSIFICATION IN PATIENTS WHO HAVE FIBRODYSPLASIA OSSIFICANS PROGRESSIVA - A STUDY OF 44 PATIENTS

被引:253
作者
COHEN, RB
HAHN, GV
TABAS, JA
PEEPER, J
LEVITZ, CL
SANDO, A
SANDO, N
ZASLOFF, M
KAPLAN, FS
机构
[1] HOSP UNIV PENN,DEPT ORTHOPAED SURG,SILVERSTEIN PAVILION,2ND FLOOR,3400 SPRUCE ST,PHILADELPHIA,PA 19104
[2] CHILDRENS HOSP PHILADELPHIA,DIV HUMAN GENET & MOLEC BIOL,PHILADELPHIA,PA 19104
[3] UNIV CALIF LOS ANGELES,DEPT MED,LOS ANGELES,CA 90024
[4] INT FIBRODYSPLASIA OSSIFICANS PROGRESSIVA ASSOC,CASSELBERRY,FL
关键词
D O I
10.2106/00004623-199302000-00008
中图分类号
R826.8 [整形外科学]; R782.2 [口腔颌面部整形外科学]; R726.2 [小儿整形外科学]; R62 [整形外科学(修复外科学)];
学科分类号
摘要
Forty-four patients who had fibrodysplasia ossificans progressiva responded by mail to a questionnaire regarding the age at the onset of heterotopic ossification at fifteen commonly involved anatomical sites. The average age of the patients when they responded to the questionnaire was twenty-seven years (range, three to sixty-nine years). The average age at the onset of ossification was five years (range, birth to twenty-five years). The most common sites of early heterotopic ossification were the neck, spine, and shoulder girdle. Thirty-five (80 per cent) of the patients had had some restrictive heterotopic ossification by the age of seven years. By the age of fifteen years, forty-two (more than 95 per cent) of the patients had severely restricted mobility of the upper limbs. In these patients, heterotopic ossification proceeded in a direction that was axial to appendicular, cranial to caudad, and proximal to distal; this pattern appeared typical for fibrodysplasia ossificans progressiva.
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页码:215 / 219
页数:5
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