Hand size and growth in untreated and IGF-I treated patients with Laron syndrome.
We have previously reported on the linear growth, growth of the head circumference and foot length in untreated and IGF-I treated patients with Laron syndrome (LS) (primary GH insensitivity).


OBJECTIVE
To assess the size and growth of the hands in patients with LS from early childhood to adult age.


METHODS
Ten IGF-I treated children with LS (4 M, 6 F) and 24 untreated patients (10 M, 14 F) were studied.


METHODS
Measurements of palm length were made on available standardized hand X-rays from infancy to adult age. The measurements were compared to normal references and SD values were calculated for each measurement. The growth of the hand was compared to the concomitant height of the body.


RESULTS
Hand SDS in untreated patients with LS decreased with age, from a mean of -2.8 +/- 0.7 (age 1-3 years) to -7.3 +/- 0.8 (age 13-15 years) and to -9.0 +/- 3.9 (age 40-50 years). During 9 years of IGF-I treatment the hand size deficit SDS did not improve in contradistinction to the height SDS which decreased from -6.2 +/- 1.2 to -3.9 +/- 0.5.


CONCLUSIONS
Congenital IGF-I deficiency, as in Laron syndrome, profoundly affects the size and growth of the hand as part of its growth retardation characteristics, resulting in acromicria.
Konen
O
O
Imaging Department, Schneider Children's Medical Center, Petach Tikva and Sackler School of Medicine, Tel Aviv University, Israel.
Silbergeld
A
A
Lilos
P
P
Kornreich
L
L
Laron
Z
Z
D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D005260:Female; D006225:Hand / Q000000981:diagnostic imaging / Q000254:growth & development* / Q000473:pathology; D006801:Humans; D007223:Infant; D007334:Insulin-Like Growth Factor I / Q000627:therapeutic use*; D046150:Laron Syndrome / Q000175:diagnosis / Q000188:drug therapy* / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D011859:Radiography; D011994:Recombinant Proteins / Q000627:therapeutic use; D016896:Treatment Outcome; D055815:Young Adult
D011994:Recombinant Proteins; D007334:Insulin-Like Growth Factor I