RESUMO
Active ending of the life of a newborn baby is a crime. Yet its clandestine practise is a reality in several European countries. In this paper, we defend the necessity to institute a proper legal frame for what we define as active neonatal euthanasia. The only legal attempt so far, the Dutch Groningen protocol, is not satisfactory. We critically analyse this protocol, as well as several other clinical practises and philosophical stances. Furthermore, we have tried to integrate our opinions as clinicians into a law project, with the purpose of pinpointing several issues, specific of perinatality that should be addressed by such a law. In conclusion, we argue that the legalisation of neonatal euthanasia under exceptional circumstances is the only way to avoid all the "well-intentioned" malpractices associated with ending life at the very dawn of it.
RESUMO
Neonatal hemochromatosis is a heterogeneous disorder of iron metabolism characterized by hepatic failure and marked iron accumulation in liver and extrahepatic tissues. Autosomal recessive transmission is found in most cases. Neonatal hemochromatosis shares cellular features with the adult disease but is clinically and genetically distinct, the causal gene(s) being presently unknown. We report on a newborn from consanguineous parents who presented with multiple congenital anomalies and neonatal hemochromatosis. The syndrome consisted of intra-uterine growth retardation, intestinal atresia, gallbladder aplasia and diabetes mellitus, and fitted with the diagnosis of Martinez-Frias syndrome, a very rare autosomal recessive phenotype, the gene of which remains to be identified. We suggest that neonatal hemochromatosis may be part of the Martinez-Frias syndrome. Molecular analyses in this and other reported patients with the Martinez-Frias syndrome should shed light on gut development and iron metabolism.