guidebook. - Fanconi Anemia Research Fund

Chapter 7: Endocrine Disorders in Fanconi Anemia
157
Table 4: Endocrinopathies in Adults (ages >16y) with Fanconi Anemia.
Center
(Reference)
N Adult
Height
Low
Thyroid
%
NY 1 7 NA NA
(results
not
separately
provided
for adults)
NIH 2 26 42%
shorter
than
-2SD
CCHMC 3-6 5 60%
shorter
than
-2SD
Low
Growth
Hormone
%
57% 57%
(N=7)
80% 33%
(N=3)
Abnormal
Glucose/
Insulin %
Abnormal
Gonadal
Function
%
NA NA NA NA
38%
hyperglycemia;
high
insulin
27%;
metabolic
syndrome
21%.
Males:
43% hypogonadism
(N=7)
Females:
77%
premature
ovarian
failure
(N=13)
2 diabetic Males:
80% hypogonadism
Females:
NA
Medications and Treatments That Affect
Endocrine Function
Low
Bone
Mineral
Density
%
92%
(N=13)
low bone
mineral
Androgen therapy
Androgen therapy can improve growth rate along
with improving blood counts, but can lead to faster
maturation of bones, leading to a shorter period available
for childhood growth (see Chapter 3). Criteria
for androgen use in FA patients with hematological
deterioration should be determined by the hematologist
or transplanter because of the potential effects on
future HSCT. Androgen use may result in virilization
of children, in both males and females. While growth
rate often improves during androgen therapy, the skeletal
advancement often outstrips the gain in height. The
child treated with androgen can appear to be growing
quite well, but potential adult height may decline. Prior
to beginning androgen therapy, a BA x-ray should be
NA