Weaning from invasive respiratory support in newborn: is there just one strategy?

Despite a even more frequent use to non-invasive respiratory support, mechanical ventilation is stilloften necessary for supporting premature infants with lung disease. Protracted mechanical ventilation is associatedwith increased morbidity and mortality and thus the earliest weaning from invasive respiratory supportis desirable. Weaning protocols may be helpful in achieving more rapid reduction in support. However,no consensus has been reached on criteria to identify when patients are ready to wean or how to achieve it. Inthis article, available evidence is reviewed and reasonable evidence-based recommendations for weaning andextubation are provided.

Severe Neonatal Metabolic Decompensation in Methylmalonic Acidemia Caused by CblD Defect.

CblD disorder is an autosomal recessive, rare, heterogeneous disease with variable clinical presentations, depending on the nature and location of the MMADHC gene mutations. Mutations in MMADHC lead to three distinct phenotypes: cblD-MMA, cblD-HC, and cblD-MMA/HC. To date, 18 cblD patients have been reported. Six of them were affected by cblD-MMA, but only three had a known clinical history. One of these patients presented with a metabolic decompensation at 11 months; the second one, born prematurely, was diagnosed with cblD after being treated for intracranial hemorrhage, respiratory distress syndrome, necrotizing enterocolitis, and convulsions at birth; the third one was diagnosed at 5 years of age.Here we present a case of a cblD-MMA patient who had an acute neonatal onset with severe hyperammonemia requiring hemodiafiltration. To the best of our knowledge, this is the first cblD-MMA patient who presented acutely in the newborn period. He has developed well upon treatment with B12, carnitine, and hypoproteic diet. At present time, at the age of 7, he shows normal growth and cognitive development. Thus, it is likely that the aggressive treatment of this child with hemodiafiltration might have prevented him from long-term neurological sequelae. Overall, this case shows that even severe, neonatal-onset patients may display a vitamin B12-responsive MMA. Furthermore, it suggests that an early treatment with vitamins might be beneficial for patients presenting with neonatal-onset hyperammonemia regardless of the suspected disease and before receiving the biochemical diagnosis.

[Congenital megacystis in a female fetus. Case report]. / Megavescica in feto di sesso femminile. A proposito di un caso.

The Authors report a case of congenital megacystis without other anomalies, diagnosed in a 27GW female fetus. At prenatal counselling the diagnostic hypothesis formulated were obstructive uropathy (cloacal malformation, uro-genital sinus, urethral atresia), functional alteration (neurogenic bladder, primitive myopathies) or Prune Belly Syndrome (rare in females). The outcome following treatment suppose a visceral myopathy, for this condition, however definitive etiology is obscure.

[Obstetric/neonatological integration and care of healthy newborn infants]. / L'integrazione ostetrico/neonatologica e l'assistenza al neonato sano.

We report how we changed the model of the organization and the assistance in our Department of healthy newborns (2200-2400/years). After we have realized that mothers were not satisfied of the rules of the hospital and personnel was not satisfied of the job, we decided to begin a process of analysis and review of the procedures on full term newborn. During this process we found out that the most important thing was to have clear in mind the problems and the needs of the mother and the baby, and not those of nurses and doctors. A similar process took place in the Department of Obstetrics. In this way we, Obstetrics and Neonatologist together, began to offer a more human approach to birth, and rooming-in began. We stopped to attend every normal delivery, to separate immediately mother and baby, to feed the baby at fixed time, to give him supplementations. We tried to have with the mother a better relationship, visiting the baby in presence of the mother an receiving grom Obstetrics as soon as possible every information about pregnancy. We realized that this was possible only if the Neonatologist and the Obstetric were of the same opinion about a more human approach to birth. We stress this point, well aware that it's impossible to reach this goal unless everybody in any way involved in birth work in great harmony with all the others. A further result of this "new" way of working has been the program of early discharge: if desired, and whenever possible, the mother and the baby go home 48-72 hours after delivery. We report here preliminary data.