Early diagnosis and multidisciplinary care reduce the hospitalization time and duration of tube feeding and prevent early obesity in PWS infants.

BACKGROUND/AIMS: 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. CONCLUSION: Early diagnosis combined with multidisciplinary care decreases the hospitalization time, duration of gastric tube feeding and prevents early obesity in PWS infants.

[Facilitating environment for dementia patients--the Pixel study]. / Environnement contenant chez la personne âgée démente--Etude Pixel.

UNLABELLED: Caregivers of demented outpatients can suffer from poor health, are often anguished and unable to cope with the cared patient, and this burden can generate frailty. OBJECTIVES: This survey attempted to determine the psychological parameters and effective capability influence of the caregiver's Quality of Life (QoL) and vulnerability, and measured the consequences of their alteration. METHODS: 4 categories of situations were studied, according to the angst and coping capability of caregivers. Caregiver's QoL and vulnerability were assessed in these situations. Data were related to the socio-demographic data of both patients and their principal caregivers, and to the patients' medical and therapeutic data. RESULTS: 1,410 and 10 patients diagnosed with dementia who lived at home with their principal caregivers were recruited for this survey. Angst and inability to cope with patients alter caregivers' QoL and was correlated to their vulnerability. Female caregivers were often in these situations, had a poorer QoL and were more vulnerable than men. Caregivers anguished and/or nab to cope with the cared patient were less satisfied with their own care and did not enjoy their relationships with their patients. Caregivers with psychological difficulties and failure to cope had to deal with nutritional difficulties with the demented patient. DISCUSSION: Caregivers' QoL and vulnerability, are related to angst and inability to cope with patients. We could imagine that both patients and caregivers would benefit by taking care of carers.

Can some growth hormone (GH)-deficient children benefit from combined therapy with gonadotropin-releasing hormone analogs and GH? Results of a retrospective study.

Recombinant GH (rGH) treatment does not invariably correct height deficits in GH-deficient children once puberty has begun. The addition of GnRH analogs (GnRHa) to delay puberty has been advocated, but published results are few and sometimes conflicting. We retrospectively compared GH-deficient children treated with rGH and GnRHa for at least 1 yr after entering puberty and having attained their final height (n = 23) with a matched control group treated only with rGH. Overall, combined therapy did not significantly increase final height relative to rGH alone. However, the shortest girls at the onset of puberty (<25th percentile) benefited more than the tallest (>75th percentile) in both final height relative to predicted height and pubertal catch-up growth. In the control group, patients having experienced intrauterine growth retardation (IUGR) attained a lower mean final height than patients without IUGR (difference significant in boys, but not in girls). In the combined therapy group, IUGR did not affect the final height of either sex. Our results suggest that two populations might benefit most from combined GnRHa and rGH therapy: girls particularly short at the onset of puberty and patients who had experienced IUGR. Further prospective studies are required to confirm these preliminary hypothesis.