[The importance of using the cerebral function monitor (CFM) in the neurological prognosis of neonates in intensive care]. / Importanza dell'uso del Cerebral Function Monitor (CFM) nella prognosi neurologica dei neonati ricoverati in Terapia Intensiva.

Cerebral function monitor (CFM), unlike traditional EEG, allows a long-term evaluation of electric brain activity, without interfering with the nursing of the newborn in the intensive care unit. Our aim was to evaluate the prognostic value of CFM for neurological outcome. We studied 102 newborns (gestational age 34.5 +/- 4.36 weeks; weight 1980 +/- 720 grams) by Multitrace CFM (Lectromed) 5 hours daily in the first week following admission. The patients also underwent cerebral echography, EEG and neurological follow-up to the 24th month. CFM was found to correlate well with the EEG recorded 3 months later. The persistence for at least one week of an I.C. tracing or the normalization of initial tracing have a good prognostic value (positive predictive value 95.23%), a persistently pathologic registration has a negative prognostic value (negative predictive value 85.18%), that even increases if cerebral echographic alterations are demonstrated (98.57%). The association of CFM and ultrasound abnormalities determines a relative risk for neurological motor impairment of 69.14, whereas CFM alone gives a relative risk of 6.4.

[The VACTERL association: a report of a clinical case with hepatic cystic lymphangiectasis]. / Associazione VACTERL: descrizione di un caso clinico con linfangectasia cistica epatica.

VACTERL association includes three or more of the following six anomalies: V (vertebral anomalies), A (anal atresia), C (cardiac abnormalities), TE (tracheo-esophageal fistula and/or esophageal atresia), R (reno-urinary anomalies) and L (limb defects). VACTERL cases are classified as "associated" when other than the typical six defects are present in the same infant, or "isolated" when they are not. We report a case of VACTERL association "associated" that presents an hepatic cystic lymphangiectasia that was never described before in literature. We also consider the most important factors involved in the aetiology of the typical anomalies.

Thrombomodulin serum levels in ventilated preterm babies with respiratory distress syndrome.

UNLABELLED: A soluble form of thrombomodulin (TM), an anticoagulant proteoglycan of the endothelial cell membrane, considered a marker of vascular endothelial damage, was measured in plasma of preterm infants with respiratory distress syndrome (RDS). In these patients, lung immaturity leads to endothelial leak of plasma proteins and to surfactant inhibition. In 18 babies with RDS, plasma TM concentration was significantly elevated compared with values of a matched group of babies without pulmonary disease (276.1 ng/ml vs 141.3 ng/ml) (P < 0.05). Furthermore, TM levels of mechanical ventilated babies (IPPV) with severe RDS were higher than those of babies with moderate RDS and treated with nasal CPAP (340.9 ng/ml vs 174.2 ng/ml) (P < 0.05). CONCLUSION: These data show that TM can be used as marker of pulmonary endothelial damage in preterm babies treated with mechanical ventilation for RDS and suggest early intervention with exogenous surfactant to limit alveolar protein leakage and surfactant inactivation.

[Double phototherapy with Wallaby optic fibers versus conventional phototherapy. Case reports]. / Fototerapia doppia con fibre ottiche Wallaby versus fototerapia convenzionale. Contributo casistico.

The authors have valued the efficacy of the double phototherapy with fiberoptic Wallaby vs conventional phototherapy in 2 groups of term infants, without any complication at birth, utilized respectively as study group and control group. While conventional phototherapy produced a bilirubin reduction of 0.60 +/- 0.26% per hour (with a total reduction of 28.1 +/- 11.1%), the double phototherapy was statistically more effective (p < 0.05) then conventional phototherapy causing a bilirubin reduction of 0.73 +/- 0.28% (with a total reduction of 33.3 +/- 9.5%). At 24 hour after the interruption of the treatment 9 newborns of the study group (36%) and 7 of the control group (28%) presented a rebound effect (increase of the bilirubinemia more than 17 mumol/l), but without a statistical difference (p > 0.05). Our study shows that double phototherapy with Wallaby fiberoptic and conventional phototherapy represent a valid strategy in the treatment of the non haemolytic neonatal hyperbilirubinemia, because, compared to conventional phototherapy, double phototherapy is more effective and reduces the period of the treatment, showing a simple management of the jaundiced newborn.

[Concentration of blood ICAM-1s in the newborn at risk of infection. A prospective study in the first 2 weeks of life]. / Concentrazioni sieriche di ICAM-1s in neonati a rischio di infezione. Studio prospettico nelle prime due settimane di vita.

We prospectively determined serum concentrations of soluble intercellular adhesion molecule 1 (sICAM-1) in the first 2 weeks of life in 32 preterm newborns in an attempt to assess whether these concentrations are reliable markers of sepsis in newborns at risk of infection. Ten of the study group were normal and had been hospitalized only for low birth weight. The remaining 22 presented respiratory distress (RDS) and were at even higher risk of infection because they required assisted mechanical ventilation and central venous catheterisation for parenteral feeding and infusion therapy. Sepsis was diagnosed in 11/22 newborns with RDS: in 3 on day 3 and in 8 on day 7. Circulating sICAM-1 concentrations were significantly elevated in neonates with RDS (group II) and associated infection (group III) compared with normal newborns (group I). However, after day 3 of life sICAM-1 values were significantly higher in group III than in group II.

[CHARGE association: report of a clinical case with anal atresia and rectovaginal fistula]. / Associazione CHARGE: descrizione di un caso clinico con atresia anale e fistola retto-vaginale.

CHARGE association is a non-random constellation of Coloboma, Heart Disease, Atresia of the choana, Retarded mental development and growth, Genital hypoplasia, Ear anomalies and deafness. We report on a newborn with CHARGE association. In addition to the typical features our case presented anal atresia and rectovaginal fistula. Our patient had congenital cardiopathy and feeding problems and died at the age of 3 months. Most case of CHARGE association appear to have a primary defect of pharyngeal incoordination that cause aspiration of secretion that is an important cause of death in the nonsurvivors.

[Congenital pulmonary lymphangiectasia with chylothorax. Different evolution of 2 cases with severe neonatal respiratory distress]. / Linfangectasia polmonare congenita con chilotorace. Differente evoluzione di due casi con grave distress respiratorio neonatale.

The Authors report on 2 cases of congenital pulmonary lymphangiectasia with chylothorax and severe respiratory distress which were characterized by a different clinic course. One of the newborns, in fact, died after a few days of life, while the other has survived with the complete regression of chylothorax. The Authors also add some physiopathologic remarks about pulmonary lymphatic circulation, in order to focus the different evolution of the 2 cases and the pulmonary lymphangiectasia treatment.

[A case of Hallermann-Streiff syndrome with rapidly fatal course]. / Su di un caso di sindrome di Hallermann-Streiff ad evoluzione rapidamente fatale.

The Hallermann-Streiff syndrome is a rare affection characterized by beaked nose, dyscephaly, hypotrichosis, cataracts, micrognathia and proportionate short stature. The most severe complication in the syndrome is respiratory embarrassment. Narrow air passage with abnormal glottic closure, prenatal growth deficiency, immunodeficiency and sometimes associated cardiovascular anomalies, can predispose these patients to pulmonary infections. The Authors describe a new-born with Hallermann-Streiff syndrome, who, after a brief period of obstructive apnea successfully treated with CPAP, developed a severe pulmonary infection that caused his death at the age of 61 days.

[Pena-Shokeir syndrome: report of a case with benign outcome]. / La sindrome di Pena-Shokeir: descrizione di un caso clinico ad evoluzione benigna.

Pena-Shokeir syndrome is a rare, often lethal disease, characterized by intrauterine growth retardation and by fetal akinesia or hypokinesia that leads to craniofacial anomalies, limb ankylosis, polyhydramnios and pulmonary hypoplasia. The case that we report had a favourable evolution, although there was at birth a severe respiratory distress. EMG studies revealed deficit of innervation. Contraceptive therapy, wrongly used by the mother in the first period of pregnancy, played, probably, a concomitant role in the pathogenesis of the syndrome.