Growth hormone benefits children with 18q deletions.
Most individuals with constitutional deletions of chromosome 18q have developmental delays, dysmyelination of the brain, and growth failure due to growth hormone deficiency. We monitored the effects of growth hormone treatment by evaluating 23 individuals for changes in growth, nonverbal intelligence quotient (nIQ), and quantitative brain MRI changes. Over an average of 37 months, the treated group of 13 children had an average nIQ increase of 17 points, an increase in height standard deviation score of 1.7, and significant change in T1 relaxation times in the caudate and frontal white matter. Cognitive changes of this magnitude are clinically significant and are anticipated to have an effect on the long-term outcomes for the treated individuals.
Cody
Jannine D
JD
Department of Pediatrics, University of Texas Health Science Center, San Antonio, 78229, USA. cody@uthscsa.edu
Semrud-Clikeman
Margaret
M
Hardies
L Jean
LJ
Lancaster
Jack
J
Ghidoni
Patricia D
PD
Schaub
Rebecca L
RL
Thompson
Nora M
NM
Wells
Lynda
L
Cornell
John E
JE
Love
Tanzy M
TM
Fox
Peter T
PT
Leach
Robin J
RJ
Kaye
Celia I
CI
Hale
Daniel E
DE
D000015:Abnormalities, Multiple / Q000188:drug therapy* / Q000235:genetics / Q000473:pathology; D001827:Body Height / Q000187:drug effects; D001921:Brain / Q000187:drug effects / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D002872:Chromosome Deletion; D002887:Chromosomes, Human, Pair 18 / Q000235:genetics*; D002658:Developmental Disabilities / Q000188:drug therapy / Q000473:pathology; D006130:Growth Disorders / Q000188:drug therapy / Q000473:pathology; D019382:Human Growth Hormone / Q000172:deficiency / Q000627:therapeutic use*; D006801:Humans; D007360:Intelligence / Q000187:drug effects; D008279:Magnetic Resonance Imaging; D013997:Time Factors; D016896:Treatment Outcome
D019382:Human Growth Hormone