Adult height after GH therapy in 188 Ullrich-Turner syndrome patients: results of the German IGLU Follow-up Study 2001.
OBJECTIVE
We aimed to evaluate the factors influencing true adult height (HT) after long-term (from 1987 to 2000) GH treatment in Ullrich-Turner syndrome (UTS) based on modalities conceived in the 1980s.


METHODS
Out of 347 near-adult (>16 Years) patients from 96 German centres, whose longitudinal growth was documented within KIGS (Pharmacia International Growth Database), 188 (45, X=59%; bone age >15 Years) were available for further anthropometric measurements.


RESULTS
At a median GH dose of 0.88 (10th/90th percentiles: 0.47/1.06) IU/kg per week, a gain of 6.0 (-1.3/+13) cm above the projected adult height was recorded. Variables were recorded at GH start, after 1 Year GH, puberty onset, and last visit on GH therapy. At these visits, the median ages were 11.7, 12.7, 14.2, 16.6 and 18.7 Years; and median heights, 0.4, 1.1, 1.7, 1.7 and 1.3 SDS (UTS) respectively. Height gain (DeltaHT) after GH discontinuation was 1.5 cm. Total DeltaHT correlated (P<0.001) negatively with bone age and HT SDS at GH start, but positively with DeltaHT after the first Year, DeltaHT at puberty onset, and GH duration. Final HT correlated (P<0.001) positively with HT at GH start, first-Year DeltaHT, and HT at puberty onset. Body mass index increased slightly (P<0.05), with values at start and adult follow-up correlating highly (R=0.70, P<0.001). No major side effects of GH occurred.


CONCLUSIONS
GH dosages conceived in the 1980s are safe but too low for most UTS patients. HT gain and height are determined by age and HT at GH start. Height gain during the first Year on GH is indicative of overall height gain. After spontaneous or induced puberty, little gain in height occurs.
Ranke
M B
MB
Paediatric Endocrinology Section, University Children's Hospital, Tubingen, Germany. mlranke@med.uni-tuebingen.de
Partsch
C J
CJ
Lindberg
A
A
Dorr
H G
HG
Bettendorf
M
M
Hauffa
B P
BP
Schwarz
H P
HP
Mehls
O
O
Sander
S
S
Stahnke
N
N
Steinkamp
H
H
Said
E
E
Sippell
W
W
D000293:Adolescent; D001827:Body Height / Q000187:drug effects* / Q000502:physiology; D015992:Body Mass Index; D002648:Child; D004305:Dose-Response Relationship, Drug; D005260:Female; D005500:Follow-Up Studies; D019382:Human Growth Hormone / Q000008:administration & dosage / Q000627:therapeutic use*; D006801:Humans; D007621:Karyotyping; D011627:Puberty / Q000502:physiology; D014424:Turner Syndrome / Q000188:drug therapy* / Q000235:genetics / Q000473:pathology* / Q000503:physiopathology
D019382:Human Growth Hormone