Growth hormone therapy and respiratory disorders: Long-term follow-up in PWS children

Context: Adenotonsillar tissue hypertrophy and obstructive sleep apnea have been reported during short-term GH treatment in children with Prader-Willi syndrome (PWS). Objective: We conducted an observational study to evaluate the effects of long-term GH therapy on sleep-disordered breathing and adenotonsillar hypertrophy in children with PWS. Design: This was a longitudinal observational study. PatientsandMethods:Weevaluated 75 children with genetically confirmedPWS,ofwhom50 fulfilled the criteria and were admitted to our study. The patients were evaluated before treatment (t0), after 6 weeks (t1), after 6 months (t2), after 12 months (t3), and yearly (t4-t6) thereafter, for up to 4 years of GH therapy. The central apnea index, obstructive apnea hypopnea index (OAHI), respiratory disturbance index, and minimal blood oxygen saturation were evaluated overnight using polysomnography. We evaluated the adenotonsillar size using a flexible fiberoptic endoscope. Results: The percentage of patients with an OAHI of 1 increased from 3 to 22, 36, and 38 at t1, t4, and t6, respectively (2 12.2; P .05). We observed a decrease in the respiratory disturbance indexfrom1.4 (t0) to 0.8 (t3) (P.05)andthe centralapneaindexfrom1.2 (t0) to 0.1 (t4) (P.0001). We had to temporarily suspend treatment for 3 patients at t1, t4, and t5 because of severe obstructive sleep apnea. The percentage of patients with severe adenotonsillar hypertrophy was significantly higher at t4 and t5 than at t0. The OAHI directly correlated with the adenoid size (adjusted for age) (P .01) but not with the tonsil size and IGF-1 levels. Conclusion: Long-termGHtreatment in patients withPWSis safe; however,werecommend annual polysomnography and adenotonsillar evaluation

SIDS: una rivisitazione

SIDS is still today after the " back to sleep campaigns" the most frequent cause of death in the first year of life in the industrialized word. The Lombardia county has held since 2000 a similar advice campaign. The 3 main suggestions are: sleep supine, avoid overheating , avoid active ant to second-hand smoking

Apparet Life Threatenin Events: esiste un ruolo del monitoraggio domiciliare?

Quando si deve afrontare un caso di ALTE ( Apparent Life Threatening Events) la sfida per il medico consiste nel trattare l'evento acuto, identificarne la causa, spiegare al paziente-genitore cosa fare, decidere se sar\ue0 necessario un monitoraggio successiv

"Un minuto per la vita": Tecniche rianimatorie in lattanti a rischio di morte improvvisa. Realizzazione di un video didattico per i genitori. ["A minute for life": resuscitation techniques in infants with risk for sudden infant death syndrome. Development of an instructional video for parents]

Aim. The present study describes a project carried out in the Center for SIDS/ALTE of the Pediatric Clinic of Varese, targeted to deliver and provide parents with a movies on PBLS titled "A Minute for Life". The impact on the parent was evaluated by applying a questionnaire.Methods. The movie "A Minute for Life" was given to 308 parents (122 fathers, mean age 35.2 and 186 mothers, mean age 28.4). All parents completed a questionnaire based on 4 parameters: 1. clarity of the content presented; 2. anxiety felt while watching the movie clip 3. reassurance related to their ability to review other times the movie; 4. the perception of its usefulness.Results. Regarding the clarity of content, the results provide evidence :that 231 parents (75% of the sample) rated him "very clear", while 77 subjects (25%) considered it "quite clear". On the possibility of being able to have at home, 277 parents (90% of subjects) believed it would be very reassuring to see it back Home. According to 231 parents (75% of the sample) the vision of the movie does not convey anxiety, while 77 of them (25% of subjects) felt slightly concerned in relation to vision. With regard to the overall assessment of the movie, all parents (308, 100% of the sample) agreed about its extreme usefulness.Conclusion. Our study opens the way for further prospective studies regarding the appropriateness and usefulness of the movie. It's also important to consider the release of the movie to all new parents and the possibility of giving first aid courses open to all those who want to be able to act with promptness and expertise if and when necessary.Aim. The present study describes a project carried out in the Center for SIDS/ALTE of the Pediatric Clinic of Varese, targeted to deliver and provide parents with a movies on PBLS titled "A Minute for Life". The impact on the parent was evaluated by applying a questionnaire. Methods. The movie "A Minute for Life" was given to 308 parents (122 fathers, mean age 35.2 and 186 mothers, mean age 28.4). All parents completed a questionnaire based on 4 parameters: 1. clarity of the content presented; 2. anxiety felt while watching the movie clip 3. reassurance related to their ability to review other times the movie; 4. the perception of its usefulness. Results. Regarding the clarity of content, the results provide evidence that 231 parents (75% of the sample) rated him "very clear", while 77 subjects (25%) considered it "quite clear". On the possibility of being able to have at home, 277 parents (90% of subjects) believed it would be very reassuring to see it back Home. According to 231 parents (75% of the sample) the vision of the movie does not convey anxiety, while 77 of them (25% of subjects) felt slightly concerned in relation to vision. With regard to the overall assessment of the movie, all parents (308, 100% of the sample) agreed about its extreme usefulness. Conclusion. Our study opens the way for further prospective studies regarding the appropriateness and usefulness of the movie. It's also important to consider the release of the movie to all new parents and the possibility of giving first aid courses open to all those who want to be able to act with promptness and expertise if and when necessary