|
KMID : 0378019850280120085
|
|
New Medical Journal
1985 Volume.28 No. 12 p.85 ~ p.92
|
|
|
A Clinical Study of Fanconi Anemia
|
|
|
|
|
Abstract
|
|
|
Fanconi anemia is a familial disorder marked by the association of pancytopenia and progressive bone marrow failure with a variable constellation of congenital anomalies and chromosomal instability.
A clinical observation was done on 9 cases of Fanconi anemia who were admitted to the pediatric ward, Seoul National University Hospital during the period of 12 years from 1973 to 1985.
The results were as follows;
1) Male to female ratio was 3.5 : 1 with male preponderance, and the ages of onset were between 10months and 5year & llmonths with the mean age of 3year and 6months.
2) 4 familial cases were observed in 2 families, brothers and brother & sister.
3) Most of cases(75%) were born at term with the birth weights of 2. 2 to 2. 7kg which suggested intrauterine growth ratardation.
4) The most common associated anomaly was the skin pigmentation(77%) and the other associated anomalies were skeletal anomalies, microcephaly, microphthalmia and patent ductus arteriosus in order of frequency. Among the skeletal anomalies, anomalies of thumb (3 cases) and radius (2 cases) were common.
5) The chromosomal aberration was observed in 2 cases out of 6 cases.
6) Out of 7 cases treated with androgen and prednisolone, 3cases have been on follow-up, other 3 cases expired lyear and 6months to 4year and 5months after diagnosis.
|
|
|
KEYWORD
|
|
|
|
|
|
FullTexts / Linksout information
|
|
|
|
|
|
Listed journal information
|
|
|
|
|