Structural asymmetries in motor and language networks in a population of healthy preterm neonates at term equivalent age: a diffusion tensor imaging and probabilistic tractography study.

In this MRI study, we aimed to provide new in vivo structural markers of asymmetry in motor and language networks in a population of healthy preterm neonates scanned at term equivalent age. Using diffusion tensor imaging and probabilistic tractography, we showed that, besides volume and microstructural asymmetries in the parieto-temporal part of the superior longitudinal fasciculus (SLF) and a trend towards microstructural asymmetry in the corticospinal tract (CST), volume asymmetry in the motor part of the superior thalamic radiations (STR) and a trend towards volume asymmetry in the CST are already present in the neonatal period. No asymmetry was found in the sensory part of the STR, the anterior thalamic radiations (ATR), and posterior thalamic radiations (PTR) neither in the fronto-parietal part of the SLF. These results suggest that structural asymmetries in the motor and language networks are present in healthy preterm neonates at term equivalent age, well before the development of speech and hand preference.

Laryngeal oedema in neonatal apnoea and bradycardia syndrome (a pilot study).

PURPOSE: Some preterm infants in general good health continue to present recurrent apnoeas, bradycardias and desaturations (ABD) despite usual treatments. These events may lead to transitory brain hypoxia and to further neurological injury. The purpose of this study has been to evaluate the role of laryngeal oedema in this symptomatology and to assess corticoid treatment. METHOD: Twelve preterm babies born at a median age of 28.5 weeks (range: 26-35 weeks) already showed signs of ABD at a median age of life of 28.5 days (range: 9-80 days). Fiberoptic laryngeal endoscopy was performed on these babies at a median postconceptional age of 34 weeks (range: 31-38 weeks) to detect a possible involvement of the larynx in their ABD. RESULTS: Each patient presented a severe laryngeal oedema compatible with potential obstructive breathing. Half of the cohort (n=6) received inhaled corticosteroids initiated with a short oral dexamethasone treatment for 3 to 5 days (group 1). All the babies improved. The other half (n=6) received only an inhaled topic corticosteroid treatment (group 2). Four of the six babies improved and two needed oral dexamethasone. Laryngoscopic endoscopy was carried out after 1 week of treatment. The picture corresponded with clinical improvement. Recurrence of ABD occurred in 3/12 (25%) of the babies after stopping dexamethasone. No immediate side effects of the procedure or the treatment were observed. CONCLUSION: Laryngeal oedema may be a cause of ABD in preterm newborns. It may arise from oesophageal reflux and/or presence of the feeding tube. It can be diagnosed by atraumatic fiberoptic fibroscopy and successfully treated with corticosteroids.

Neonatal hemochromatosis and Martinez-Frias syndrome of intestinal atresia and diabetes mellitus in a consanguineous newborn.

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.