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1) Journal of
Intellectual Disability Research
"Cross-cultural
comparisons of obesity and growth in Prader-Willi syndrome," by
O. Dudley1, B. McManus2, A. Vogels3, J. Whittington4, F. Muscatelli5
(2008)
doi:10.1111/j.1365-2788.2008.01044.x
1 Institute of
Developmental Biology, Marseille - Luminy (IBDML), UMRCNRS 6156,
Campus de Luminy, Marseille, France
2 PWSA USA, Sarasota, FL,
USA
3 Department of Clinical
Genetics, University Hospital of Leuven, Herestraat 49, B-3000,
Leuven, Belgium
4 Department of
Developmental Psychiatry, 18b Trumpington Road, University of
Cambridge, Cambridge, CB2 8AH, UK
5 Mediterranean Institute
of Neurobiology (INMED), Inserm U901, BP13, 13273, Marseille, France
Lead author: Oenone
Dudley, Institut de Biologie du Développement de Marseille-Luminy (IBDML),
UMRCNRS 6156, Campus de Luminy, Case 907, 13288 Marseille, Cedex 9,
France (e-mail: odudley@orange.fr).
RESULTS: A total of 82.5% French adults with PWS have BMI > 30
compared with 65.8% in Belgium (n.s.), 58.2% in the USA (P < 0.005),
and 54.3% in the UK (P < 0.01). Higher rates of obesity in females
vs. males were found in the USA sample (P < 0.001) but not in the
other samples. In contrast to adults, growth curves for French
children with PWS show similar rates of growth compared with
children with PWS in Germany and the USA. The principal determining
factors of BMI status in the French PWS population are age (P <
0.0001), cohort (born within the last 15 years vs. born over 15
years ago, P < 0.0002) and growth hormone replacement therapy (P <
0.0002). Significant subsidiary effects include domestic situation
(P < 0.0001), genetic diagnosis (P < 0.0001) and age of diagnosis (P
< 0.0001).
CONCLUSIONS:
French adults with PWS have significantly higher rates of obesity
than adults in the UK and the USA, but growth in French children
with PWS is similar to the USA and Germany. Clinical management has
a greater impact on obesity outcome in PWS than cultural factors.
2) Spine. 2008 Feb
15;33(4):394-401.
"Complications of
Scoliosis Surgery in Prader-Willi Syndrome," by Accadbled F,
Odent T, Moine A, Chau E, Glorion C, Diene G, de Gauzy JS.
From the *Department of
Orthopedic Surgery, Hôpital des Enfants, Toulouse, France;
?Department of Endocrinology, Prader-Willi Syndrome Reference
Center, Hôpital des Enfants, Toulouse, France; ?Department of
Orthopedic Surgery, Hôpital Necker Enfants Malades, Paris, France;
§Department of Pediatric Orthopedics, Hôpital Lenval, Nice, France.
RESULTS: Mean age at scoliosis diagnosis was 6.2 years (range
0.5-13.5). Mean age at surgery was 12.3 years (range 5-15). Mean
follow-up was 5.4 years (range 2-18). There were 9 major
complications (4 severe kyphosis above fusion, 2 deep infections, 1
transient paraplegia, 1 pseudarthrosis, 1 delayed wound healing).
The 4 kyphosis required reoperation, 3 of which were complicated
with permanent spinal cord injury. Minor complications affected 6
patients.
CONCLUSION:
Scoliosis surgery is frequently necessary in PWS and is associated
with high rate of complications. These are often related to specific
features of this syndrome the surgeon should recognize and consider.
PMID: 18277871 [PubMed -
as supplied by publisher]
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