Early diagnosis and multidisciplinary care reduce the hospitalization time and duration of tube feeding and prevent early obesity in PWS infants.
OBJECTIVE
To describe and evaluate the impact of very early diagnosis and multidisciplinary care on the evolution and care of infants presenting with Prader-Willi syndrome (PWS).


METHODS
19 infants diagnosed with PWS before the second month of life were followed by a multidisciplinary team. Median age at the time of analysis was 3.1 years [range 0.4-6.5]. The data were compared with data collected in 1997 from 113 questionnaires filled out by members of the French PWS Association. The patients from this latter data set were 12.0 years [range 4 months to 41 years] at the time of analysis, with a median age of 36 months at diagnosis.


RESULTS
The duration of their hospitalization time was significantly reduced from 30.0 [range 0-670] to 21 [range 0-90] days (p = 0.043). The duration of gastric tube feeding was significantly reduced from 30.5 [range 0-427] to 15 [range 0-60] days (p = 0.017). Growth hormone treatment was started at a mean age of 1.9 +/- 0.5 years in 10 infants and L-thyroxine in 6 infants. Only 1 infant became obese at 2.5 years.


CONCLUSIONS
Early diagnosis combined with multidisciplinary care decreases the hospitalization time, duration of gastric tube feeding and prevents early obesity in PWS infants.
Bacheré
N
N
Division of Endocrinology, Genetics, Gynaecology and Bone Diseases, Hôpital des Enfants, Toulouse, France.
Diene
G
G
Delagnes
V
V
Molinas
C
C
Moulin
P
P
Tauber
M
M
D015992:Body Mass Index; D002648:Child; D002675:Child, Preschool; D042241:Early Diagnosis; D004750:Enteral Nutrition; D005260:Female; D006130:Growth Disorders / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology; D013006:Growth Hormone / Q000172:deficiency / Q000627:therapeutic use; D006801:Humans; D007231:Infant, Newborn; D007334:Insulin-Like Growth Factor I / Q000378:metabolism; D007902:Length of Stay; D008297:Male; D009043:Motor Activity; D009123:Muscle Hypotonia / Q000175:diagnosis / Q000209:etiology; D009765:Obesity / Q000178:diet therapy* / Q000209:etiology / Q000517:prevention & control*; D010348:Patient Care Team; D011218:Prader-Willi Syndrome / Q000150:complications / Q000175:diagnosis / Q000178:diet therapy*; D012189:Retrospective Studies; D016896:Treatment Outcome
D007334:Insulin-Like Growth Factor I; D013006:Growth Hormone