DTI parameters in neonates with hypoxic-ischemic encephalopathy after total body hypothermia.

BACKGROUND AND PURPOSE: MR imaging provides means for discriminating different patterns of Hypoxic-ischemic encephalopathy (HIE) and may distinguish most severe cases from less severe but is unable to predict long-term outcome. Diffusion tensor imaging (DTI) offers information for a more complete characterization of HIE. The purpose of this study is to compare the modifications of DTI parameters in newborns one week and six months following total-body cooling to healthy controls. METHODS: Forty-seven cooled newborns were studied with MRI, 20 underwent follow-up at 6 months. 12 healthy newborns and nine children at 6 months were enrolled as control groups (HC). Inferior Longitudinal Fasciculus (ILF), Corpus Callosum Fasciculus (CCF), Corticospinal Tract (CST), Optical Tract (OT), Optic Radiation (OR) were generated in all subjects. DTI parameters were evaluated in basal ganglia (BG), thalamus (TH) and tracks. Statistical analysis was performed with MANOVA. RESULTS: In newborns HIE versus HC, there were significantly lower fractional anisotropy (FA) on OR and CST and higher axial diffusivity (AD), apparent diffusion coefficient (ADC) and radial diffusivity (RD) values on CST, BG and TH in HIE-N. At 6 months there were no significant grouping effects. The analysis showed a significant increase of FA, decrease of ADC, AD, RD after 6 months for HIE and HC. CONCLUSIONS: We observed modifications of parameter values in HIE newborns vs HC; however normalization of values at 6 months suggests that changes of parameters cannot be considered early biomarkers for evaluation of therapeutic hypothermia in newborns with moderate HIE and normal conventional MRI.

Cardiac troponin I concentrations as a marker of neurodevelopmental outcome at 18 months in newborns with perinatal asphyxia.

OBJECTIVE: To investigate whether creatine kinase-MB (CK-MB) and cardiac troponin I (cTnI) can be used to predict neurodevelopmental outcome at 18 months in infants with perinatal asphyxia (PA). The diagnostic value of cTnI to assess myocardial dysfunction was considered as well. STUDY DESIGN: Retrospective study of 178 neonates admitted with PA. cTnI concentrations measured within 12 h of birth were compared with medium-term outcome assessed with the Bayley Scales of Infant Development. cTnI concentrations measured within 12 h of birth were compared with clinical grade of hypoxic-ischemic encephalopathy (HIE) and with duration of inotropic support. Two-dimensional Doppler and color Doppler findings were recorded. Fractional shortening, tricuspid and mitral regurgitation were evaluated. RESULT: A statistically significant correlation between cTnI concentration and BSID-II score was found (mental development index r -0.69, P<0.05 and psychomotor development index r -0.39, P<0.05). There was no statistically significant correlation between CK-MB and BSID-II score (P>0.05).Serum cTnI concentrations and duration of inotropic support were significantly greater with increasing severity of PA. cTnI was negatively correlated with fraction shortening (r -0.64; P<0.05). The severity of tricuspid regurgitation was correlated with the cTnI concentration (r 0.61; P<0.05). CONCLUSION: In asphyxiated neonates, cTnI concentrations within 12 h of birth correlate with medium-term outcome. Early cTnI concentration correlates with severity of HIE, myocardial dysfunction and with Bayley II scores at 18 months.

[A case of neonatal lupus syndrome and congenital atrioventricular block associated with maternal antibodies antiRo/SS-A]. / Un caso di lups neonatale con blocco atrioventricolare congenito da madre con anticorpi antiRo/SS-A.

The neonatal lupus erythematosus syndrome (LEN) is a disease due to the transplacental passage of maternal antiextractable nuclear antigens (ENA) antibodies, particularly anti-Ro/SS-A and anti-La/SS-B. The disease affects neonates born from mothers with autoimmune diseases. It is characterized by erythematous annular polycylic skin lesions, slightly scaling with prevalent face localization, hematologic and liver diseases and only in 2% of cases with extracutaneous lesions including complete atrioventricular block. The Authors describe a case of LEN characterized by isolated atrioventricular block at birth and endocardial fibroelastosis without skin lesions in a preterm infant female. She was born from asymptomatic, ANA (Anti-Nuclear Antibodies) and ENA (anti-Extractable Nuclear Antigen) positive mother, with a previous miscarriage at the 5th week of gestation.

Neonatal seizures: background EEG activity and the electroclinical correlation in full-term neonates with hypoxic-ischemic encephalopathy. Analysis by computer-synchronized long-term polygraphic video-EEG monitoring.

Between 1994 and 2000, 30 full-term newborns affected by seizures secondary to hypoxic-ischemic encephalopathy, underwent computerised, polygraphic, video-EEG recordings during the first 48 hours of life, in our neonatal intensive care unit. In this longitudinal study, recordings lasted an average of 10.5 hours. We studied EEG ictal discharges that lacked a clinical correlate, the semeiotics, and the electroclinical correlation during seizures, by reviewing the time-synchronised, video-EEG recordings obtained during these six years. The aim of this investigation was to evaluate the relationship between background EEG activity and the electroclinical correlation or dissociation of the seizures. When background activity was depressed and undifferentiated, the seizures were characterised by the electroclinical dissociation except in instances of focal tonic seizures. With all other background patterns, seizures were invariably accompanied by the electrical correlate, the only exception being ocular signs and oral-buccal-lingual movements, which occurred both with and without paroxysmal discharges. Ocular signs and oral-buccal-lingual movements were very frequent and were observed with all patterns of background activity. Only on one occasion were they the sole clinical expression of seizures. In our patients, the electroclinical dissociation was constant only if background EEG activity was depressed and undifferentiated. The constant electroclinical dissociation of seizures was closely correlated with the type of background activity, which, in turn, is indicative of severe cerebral damage. (Published with videosequences.)

Familial translocation 2;17 with partial trisomy 2q32 leads to 2qter.

A case of 2q trisomy in a malformed female infant resulting from unbalanced segregation of maternal origin is reported. The mother and one of the proposita's sibs where found to be carriers of balanced translocation 2;17. Two other members in the kindred had died with multiple malformations. The patient's karyotype was 46,XX,-17, + der (17)t(2;17)(q32;q25)mat.

Antenatal detection of omphalocele by ultrasound.

A case of successful antenatal detection of omphalocele is reported. Ultrasonic evidence of an abdominal wall defect with an extruding mass, without other anomalies, suggested the diagnosis of omphalocele and allowed the patient to receive special treatment. Despite the severity of the omphalocele, the infant was saved by immediate surgery.