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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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