Daniele M De Luca

Abstract:

Abstract: BACKGROUND: The few existing studies evaluating the reliability of transcutaneous bilirubin monitoring during phototherapy gave controversial results. AIMS: To evaluate the accuracy of transcutaneous bilirubin measurement in a large population of newborn infants, during phototherapy. STUDY DESIGN AND METHODS: Total serum bilirubin and transcutaneous bilirubin on patched and unpatched skin areas were simultaneously measured in newborn infants undergoing phototherapy. Transcutaneous measurements were performed with a multiwavelength transcutaneous bilirubinometer (Respironics BiliCheck). The Passing-Bablok regression and the Bland-Altman plot were used to estimate the relationship between serum and transcutaneous bilirubin. RESULTS: We studied 364 newborn infants with a mean (SD) gestational age of 34.6 (3) weeks and a mean birth weight of 2371 (805) grams. Total serum bilirubin, patched transcutaneous bilirubin and unpatched transcutaneous bilirubin were similar before phototherapy. After 52 (33) hours of phototherapy, the difference between serum bilirubin and patched transcutaneous bilirubin was 0.2 (3.1) mg/dL (not significant) while the difference between serum bilirubin and unpatched transcutaneous bilirubin was 3.2 (3.0) mg/dL (p<0.001). Statistical analysis showed a good agreement between serum bilirubin and patched transcutaneous bilirubin, while unpatched transcutaneous bilirubin underestimates serum levels. The difference between patched and unpatched values was significantly lower in preterm than in term infants (2.8 mg/dL vs. 3.6 mg/dL; p<0.001). CONCLUSION: BiliCheck can be safely used for the evaluation of bilirubin levels in newborn infants under phototherapy. Its reliability on patched skin of the forehead is high enough to consistently reduce blood draws and to ascertain when to discontinue phototherapy. Because of the individual variance, any clinical decision has to be taken on the basis of the transcutaneous bilirubin trend more than on a single value.

Abstract: OBJECTIVE: To verify the feasibility of non-invasive ventilation (NIV) in immunocompromised children affected by ARDS. SETTING: University Hospital PICU. PATIENTS: Twenty-three consecutive immunocompromised children treated with NIV for ARDS. INTERVENTIONS: All consecutive patients received NIV through a face-mask or a helmet. RESULTS: No differences were found regarding admission data and severity scores between NIV responders and non-responders. Early and sustained improvement in PaO2/FiO2 ratio were observed in 82 and 74% of cases, respectively. 13 out of 23 patients (54.5%) avoided intubation and were discharged from the PICU; ten patients required intubation: two of them survived and eight patients died (two refractory hypoxemia, three septic shock, three multi-organ failure). PICU and intra-hospital mortality was significantly higher for NIV-nonresponders (P < 0.001). PICU stay was significantly shorter for NIV responders (P = 0.03). NIV responders had significantly lower heart and respiratory rate at the end of treatment (P < 0.001 and P = 0.048, respectively). CONCLUSIONS: NIV administration is feasible and well tolerated in immunocompromised children with ARDS. A short NIV trial can be used to verify the usefulness of the technique. A randomized controlled trial is needed to confirm the efficacy of NIV in immunocompromised children requiring ventilatory support for ARDS.

Abstract: Glucose-6-phosphate dehydrogenase (G6PD), a X-linked hereditary deficiency, is one of most common clinically significant enzyme defects. Despite its largely known role in acute and life-threatening haemolytic crises, G6PD deficiency may be also associated with neonatal jaundice that, when severe and untreated, may lead to the potential of bilirubin encephalopathy. A prolonged neonatal jaundice was found to be associated with a rare G6PD mutation (c.383T>G; p.L128R), the latter simply annotated in literature database. In this article, we clinically and phenotipically describe a case of an Italian neonate carrying the c.383T>G G6PD mutation. Finally, we named this variant "G6PD Salerno."

Abstract: Early discharge from the maternity hospital is almost becoming the rule: it is not clear if this practice actually increases the rate of newborn rehospitalization, but it surely poses some problems for jaundice management, since hyperbilirubinemia is the most frequent reason for hospital readmission. Available guidelines for jaundice management and early discharge are reviewed. Evidence-based update is given about four new points: use of gestational age as predictor jointly with the nomogram evaluation, two-point bilirubin assay, and rate of rise evaluation; choice of the "right" curve and unforeseen jaundice risk factors. In conclusion, available predictive tools allow reliable jaundice prediction and safe early discharge if adequate follow-up is planned according to the prediction. Advice about things to avoid is provided together with a practical flowchart based on guidelines and recent evidence-based data.

Abstract: BACKGROUND AND PURPOSES: Bile acids have been implicated in some forms of acute lung injury, including meconium aspiration and bile acid pneumonia in neonates, or aspiration related ARDS in adults. Secretory phospholipase A2 (sPLA2) is now known as a key enzyme in the lung injury pathways and is supposed to be responsible for surfactant dysfunction. Our aim was to investigate the interaction between bile acids and sPLA2 in an extracellular environment representing an in vitro model of aspiration. METHODS: In vitro study using broncho-alveolar lavage (BAL) of 23 neonates/infants (<6 m) with healthy lungs. BAL supernatants were assayed for sPLA2 activity in basal condition and after addition of randomly assigned concentrations of bile acids (BA) or normal saline. Samples coming from neonates were then challenged with poractant-alfa up to a phospholipid concentration equal to that found in babies after the surfactant treatment for respiratory distress syndrome. sPLA2 activity was again measured, being corrected for serum/supernatant urea ratio and for confounding factors. RESULTS: High concentrations of BA (5 micromol/l) significantly increased (P = 0.012) sPLA2 activity, leading to increased surfactant catabolism. This finding was not observed with lower BA concentration and this is consistent with available literature data and may indicate an anionic activation of the enzyme by bile acids. Increased activity was significantly reverted by the addition of exogenous surfactant (P = 0.004) which was able to reduce sPLA2 activity almost to the baseline level. CONCLUSIONS: BA are likely to contribute to lung injury, causing surfactant inactivation through the increased sPLA2 activity. Other mechanisms cannot be excluded and require further studies to be clarified.

Abstract: In a selected patient population, we evaluated the glycemic response to different infusional policies in the management of posterior cranial fossa tumor (PFT) removal. We analyzed the perioperative course, prospectically collected, of 137 children undergoing 150 surgical procedures. Patients were divided in two groups according to different intraoperative fluids (group A, 2.5% glucose; group B, crystalloids). In group B glycemia remained below 125 mg dl(-1), while group A showed persistently supranormal glycemic plasma values, reaching statistical significance at the end of surgery (P < 0.018). As no perioperative mortality occurred and no differences were found between groups regarding PICU respiratory or infectious complications, PICU length of stay (LOS) was assumed as the main outcome indicator. LOS was not influenced by group A or B inclusion, while a new indicator, namely the Glycemic Stress Index (GSI), representing both glycemic intraoperative change and procedure length, showed significantly different results in the study groups (P = 0.004). Our clinical experience suggests that both intraoperative glucose-free solutions are safe, and GSI can be a useful tool to identify prolonged PICU stay patients.

Abstract: OBJECTIVE: The aim of this study was to investigate whether ibuprofen exposure was associated with increased hyperbilirubinemia in preterm infants. METHODS: Since 2000, ibuprofen has been administered to all infants at <30 weeks of gestation who are admitted to our unit, to prevent patent ductus arteriosus. We retrospectively compared data for 418 infants subjected to ibuprofen prophylaxis (2000-2007) and 288 infants not exposed to ibuprofen (1993-1999). RESULTS: The ibuprofen group had a significantly higher peak total serum bilirubin level (9.0 +/- 2.5 mg/dL vs 7.3 +/- 3.3 mg/dL), more need for phototherapy (398 infants [95%] vs 254 infants [87.6%]), and a longer phototherapy duration (94.3 +/- 43.6 hours vs 87.2 +/- 38.6 hours). Groups did not differ with respect to gestational age, birth weight, gender ratio, glucose-6-phosphate dehydrogenase deficiency incidence, or hypoalbuminemia (<2.5 g/dL) incidence. Hemolytic isoimmunization was diagnosed with similar incidences (no-ibuprofen group: 7 of 288 infants; ibuprofen group: 8 of 418 infants). The rates of exchange-transfusion also were similar between the groups (no-ibuprofen group: 14 infants [4.8%]; ibuprofen group: 19 infants [4.5%]). CONCLUSIONS: Ibuprofen administration was associated with higher peak total serum bilirubin levels, and the more-pronounced hyperbilirubinemia led to longer phototherapy. The potential role of competition between ibuprofen and bilirubin in the hepatic glucuronidation pathway is discussed.

Abstract: OBJECTIVES: Few data exist about patient-triggered ventilation techniques in neonatal critical care. Our aim was to compare pressure-limited synchronised intermittent positive pressure (or assist/control) ventilation (sIPPV) in the classical time-cycled (TC-sIPPV) mode against flow-cycled (FC-sIPPV) modality. In this latter, typical sIPPV full respiratory support is provided but both the initiation and the end of inflation are determined by the infant's spontaneous respiratory efforts by using airway flow changes. SETTING: A third-level neonatal intensive care unit. PATIENTS AND INTERVENTION: Ten preterm babies (<32 weeks' gestation) were randomised to receive 1 h FC-sIPPV followed by 1 h TC-sIPPV or the inverse shift, according to a computer-created randomisation table. Eligible babies had hyaline membrane disease and received 200 mg/kg surfactant at least 6 h before the study period. Respiratory mechanics, ventilatory and vital parameter data were registered real time. RESULTS: FC-sIPPV resulted in lower-rate volume ratio, pressure x rate product, mean airway pressure and heart rate; tidal volume and oxygen saturation were higher (all p<0.001). Spontaneous inspiratory time was lower than usually set by the physician and it was directly correlated to birth weight (rho = 0.5, p = 0.001) and gestational age (rho = 0.32, p = 0.001). No differences were noticed in the mechanics and blood gas and vital parameters during the two study phases. CONCLUSIONS: FC-sIPPV may safely result in a better patient ventilator synchrony. Inspiratory time usually set in neonatal critical care is higher than that decided by the baby during spontaneous effort. This should be considered when establishing time-cycled ventilation.

Abstract: OBJECTIVES: To compare available nomograms in the literature defining trends in bilirubin levels across populations with different risk factor profiles and to study a mathematical bilirubin kinetics model describing the natural course of jaundice and the bilirubin rate of rise needed to cross percentile curves. DATA SOURCES: We searched PubMed for publications between March 1999 and March 2009 that created transcutaneous nomograms. We performed the same search among abstracts presented in the past 2 years at meetings of the Pediatric Academic Societies or the European Society for Paediatric Research. STUDY SELECTION: Inclusion criteria were gestational age of at least 35 weeks among study subjects, the use of an electronic transcutaneous bilirubinometer, and creation of a nomogram based on hour-specific bilirubin values. Four articles met the selection criteria. DATA EXTRACTION: Jaundice risk factors were analyzed, and raw data were analyzed using nonlinear regression to describe trends in bilirubin levels and kinetics. The bilirubin exaggerated rate of rise needed to cross percentile curves was calculated. DATA SYNTHESIS: Significant differences in bilirubin values exist across populations, and there is substantial variability in rates of rise. Hispanic neonates demonstrate higher rates of rise and later plateaus. Bilirubin rates of rise tend to plateau and become null (equilibrium between bilirubin production and elimination) at about 96 hours of life. Rates of rise needed to cross percentile curves decrease over time but are lower (approximately 0.11 mg/dL/h [to convert bilirubin level to micromoles per liter, multiply by 17.104]) in the first 48 hours of life than previously thought. CONCLUSIONS: Transcutaneous bilirubin levels plateau and then decrease after about 96 hours of life in healthy neonates, with some differences across populations. A bilirubin rate of rise higher than in the previous period implies that bilirubin production exceeds elimination and indicates high risk for subsequent hyperbilirubinemia in neonates.

Abstract: The authors report a case of minimal prenatal trauma producing a large subdural hematoma in the fetus, which was diagnosed in utero by MR imaging. The occurrence of such a complication is extremely rare in the absence of significant maternal trauma. Prenatally diagnosed intracranial hemorrhages, particularly those that are subdural in origin, have a poor prognosis in most cases. After birth, brain compression required a complex neurosurgical intervention because simple hematoma evacuation was not possible. The clinical and neurological outcome at 6 months was excellent, as confirmed by the neuroimaging findings.

Abstract:

Abstract: OBJECTIVE: Intrahepatic cholestasis of pregnancy (ICP) has been associated with prematurity and fetal mortality. Recently, ICP has also been recognised as a risk factor for neonatal respiratory distress syndrome (RDS) in term or near-term neonates. Since fetal mortality is more frequent in pregnancies with an early ICP onset, we speculated that the time of exposure (ET) to maternal bile acids at the delivery (BAdeliv) could be involved in neonatal lung damage too. Study aim was to develop a scoring system to predict the RDS occurrence. DESIGN: We conducted a retrospective analysis of 77 pregnancies complicated by ICP (years 2000-2004) looking for factors associated to the neonatal RDS. We developed a risk score as follows: RDS risk score=BAdeliv x ET/gestational age and we prospectively applied it to 30 neonates from ICP pregnancies (years 2005-2006). RESULTS: ROC analysis indicated 9 as the score with the highest sensitivity (83.3%) and specificity (87.5%). Considering a RDS incidence of about 25% in babies coming from ICP pregnancies, the post-test probability showed a risk increased to 66.7% with a score>9 and reduced to 4.8% with a score<or=9. CONCLUSION: Our score is easy to apply and is based on the three most important variables involved in the RDS genesis. Score reliability is high enough to use it in clinical practice and to verify it in wider populations.

Abstract: Secrecy and anonymity related to heterologous assisted reproduction may hide basic newborn data to neonatologists. Secrecy and anonymity are discussed in view of their possible consequences on relational dynamics and on developmental psychology. Nevertheless, they can also involve the offspring's genetic status regarding inheritable diseases. International guidelines have been recently published on this topic. Because no guidelines are 'ideal' unfortunate and possibly dramatic consequences can occur. We aimed to embark on a debate about this matter starting with a real clinical experience. In our case a rarely fatal but widespread disease, together with the lack of knowledge about parental status led, in a fast succession of clinical events, to the unavoidable insurgence of kernicterus.

Abstract: AIM: Hour-specific nomogram evaluation of serum or skin bilirubin is a suitable approach for managing neonatal hyperbilirubinemia and it is recommended by American Academy of Paediatrics. We aimed to provide data about skin bilirubin levels during the natural course of hyperbilirubinemia in European healthy neonates. METHODS: We enrolled 2198 healthy newborn infants (gestational age [GA]>or= 35 weeks), from 24 to 96 h of life and performed transcutaneous bilirubin (TcB) measurement, in order to draw the nomogram for 10th, 25th, 50th, 75th and 95th percentiles of skin bilirubin, both for term and near term babies. All measurements were performed with a multiwavelength transcutaneous bilirubinometer (Respironics BiliCheck), within 2 h of the designed time and data were analysed with linear and local smoother regression. RESULTS: We described the peculiar pattern of skin bilirubin increasing rate over different time periods. Bilirubin linearly increases rapidly in the first 48 h and less rapidly from 48 to 72 h, while the increment is insignificant from 72 to 96 h. Conclusion: We provide the first data on skin bilirubin trend in a large predominantly breastfed and healthy European newborn population during the natural course of nonpathologic hyperbilirubinemia. Nomogram and increment rate of skin bilirubin are useful to identify neonates requiring closer evaluation and to plan an adequate follow-up.

Abstract: OBJECTIVES: Neonatal respiratory distress syndrome is associated with intrahepatic cholestasis of pregnancy, and bile acids may play a major role in neonatal bile acid pneumonia. Our aim was to demonstrate the bile acid presence in the bronchoalveolar lavage fluid of neonates affected by respiratory distress syndrome who were born from intrahepatic cholestasis of pregnancy and to investigate bile acid mechanisms of action in acute lung injury. METHODS: In this prospective study, we enrolled 10 neonates delivered from intrahepatic cholestasis of pregnancy, affected by respiratory distress syndrome requiring mechanical ventilation (intrahepatic cholestasis of pregnancy group) and 2 control groups. The first group consisted of 20 infants with respiratory distress syndrome delivered from pregnancies without any sign of intrahepatic cholestasis of pregnancy (respiratory-distress-syndrome group), and the second group included 20 neonates with no lung disease who were ventilated for extrapulmonary reasons (no-lung-disease group). We measured bile acid and pH in the bronchoalveolar lavage fluid and serum bile acid levels in the first 24 hours of life. RESULTS: Bile acids were measurable in the bronchoalveolar lavage fluid of all of the infants in the intrahepatic cholestasis of pregnancy group but were absent in the 2 control groups. Bronchoalveolar lavage fluid pH was not different among the 3 groups. Infants in the intrahepatic-cholestasis-of-pregnancy group had significantly higher serum bile acid levels compared with those in both of the control groups. CONCLUSIONS: Bile acids are detectable in the bronchoalveolar lavage fluid of newborns from intrahepatic cholestasis of pregnancy affected by respiratory distress syndrome. Elevated serum bile acid levels in these infants allow us to hypothesize that bile acid reaches the lung after an uptake from the circulation. These findings strongly support a role for bile acid in causing bile acid pneumonia.

Abstract: BACKGROUND: IGF-I-(CA) repeats have been previously analysed in few types of cancer and the results, although discordant in different studies, showed possible associations between cancer and IGF-I(CA)(19) repeats. Aim of this pilot study was to detect a possible association between some of the IGF-I(CA) repeats and the presence of malignant melanoma and its Breslow index. METHODS: Two hundred patients affected with cutaneous malignant melanoma and 100 control healthy subjects were analysed for IGF-I(CA) repeats by fragment analysis sequencing and, partially, confirmed by direct sequencing. RESULTS: A significant association of IGF-I(CA)(19) repeats was observed with melanoma higher Breslow indices (P<0.001), while no association between melanoma patients and the different genotypes of IGF-I(CA) was found. The above mentioned association was confirmed after Bonferroni's correction for multiple comparisons and also by logistic regression analysis adjusted for age, sex and BMI variables. A slight, significant difference (P=0.03) was observed for serum IGF-I values in IGF-I(CA)(19)-positive or IGF-I(CA)(19)-negative subjects. DISCUSSION: The association observed for IGF-I(CA)(19) and malignant melanoma is in keeping with similar results obtained in prostate or breast cancers, suggesting that this type of repeat may be directly or indirectly important in controlling cancer induction and its severity.

Abstract: An exceptional case of tracheal agenesis with no communication with the esophagus is described. This malformation needs surgical airway approach and is hardly classifiable. We analyzed the literature and our institutional data: this resulted to be the first case of such anatomical variant. Genetic and pathological issues are reviewed: recent genetic data seem to explain this malformation. We also reviewed the available literature about prenatal presentation. Because prenatal diagnosis is difficult to achieve and current guidelines for neonatal resuscitation do not provide any recommendation, the resuscitative team may not be prepared for managing such a case. Usefulness of uncommon resuscitative maneuvers is discussed: a promptly performed surgical tracheotomy is the only mean to ventilate such a baby.

Abstract: OBJECTIVE: To evaluate perioperative management, early outcomes and hemocoagulative disorders in infants up to 1 year old, undergoing neurosurgery for brain tumors removal. DESIGN: Retrospective evaluation of prospectively collected data regarding all infants aged <1 year admitted to PICU from 1994 to 2004, following intracranial mass removal. INTERVENTIONS: none. SETTING: University Hospital PICU in a tertiary neurosurgical referral centre. PATIENTS AND PARTICIPANTS: All eligible infants were enrolled in the study. Population was constituted by 43 infants and subdivided in two groups, according to their intra-operative blood loss. Babies having blood loss exceeding the preoperative estimated volemia were classified in group A; the remaining babies were included in group B. RESULTS: Intraoperative transfusions, PICU length of stay, need for post-operative mechanical ventilation and cardiovascular support were all significantly higher in group A than in group B. No early postoperative mortality occurred. ROC analysis and multiple logistic regression showed the age as the only variable independently associated with blood loss exceeding preoperative volemia (cut-off 60.3 days; OR = 0.11, CI 0.02-0.55, sensitivity 82.4%; specificity 67%). Postoperatively, platelet count, prothrombin activity and fibrinogen resulted significantly depressed in group A, representing a dilutional coagulopathy. A marked dispersion of aPTT values was recorded in group B, where most infants exhibit aPTT shortening, suggesting a hypercoagulability status. Three episodes of clinical disseminated intravascular coagulation (DIC) were registered in group B. CONCLUSIONS: We illustrated the relationships between intraoperative blood loss, transfusions and haemostatic impairment in babies following brain tumor removal. Youngest infants had the higher risk to experience hemocoagulative disorders. These infants showed significantly higher impact on the global PICU burden of care, as represented by the need of mechanical ventilation, cardiovascular support and PICU length of stay.

Abstract: OBJECTIVES: To compare the accuracy of a new transcutaneous bilirubinometer, BiliMed (Medick SA, Paris, France) with BiliCheck (Respironics, Marietta, GA, USA), a widely available instrument, and with total serum bilirubin measurement. DESIGN: A prospective double-blind study comparing the two devices was carried out. 686 healthy newborns needing measurement of their bilirubin were enrolled over a 4-month period. Serum and transcutaneous bilirubin measurements were taken with both devices within 15 minutes. The order of use of the instruments was randomised. SETTING: Well-baby nursery ward in a university hospital, tertiary referral centre. RESULTS: The linear regression analysis showed a better correlation between BiliCheck and serum bilirubin (r = 0.75) than between BiliMed and serum bilirubin (r = 0.45). BiliCheck variability (+/-2 SD of the mean bias from serum bilirubin) was within -87.2 to 63.3 micromol/l, while BiliMed variability was within -97.5 to 121.4 micromol/l. The receiver operating characteristic analysis (for serum bilirubin levels >205.2 micromol/l or >239.4 micromol/l) showed significantly higher areas under the curve for BiliCheck than those for BiliMed (p<0.001). CONCLUSIONS: Despite the potential practical advantages of BiliMed, its reduced diagnostic accuracy in comparison with BiliCheck does not justify its use in clinical practice.

Abstract: PURPOSES: Secretory phospholipase A2 hydrolyzes phosphoglycerides and it has been shown to be involved in alveolar inflammation and surfactant degradation. It plays an important role in acute lung injury but it has never been studied in newborn infants. We were aimed to investigate the phospholipase A2 activity in neonatal lung injury and its relationship with ventilatory findings. SETTING: Third level university hospital NICU. METHODS: We measured phospholipase activity in broncho-alveolar lavage fluid of 21 neonates with hyaline membrane disease, 10 with pneumonia or sepsis and 10 controls, ventilated for extrapulmonary reasons. Fluid was obtained before surfactant administration on the first day of life and phospholipase activity was measured using an ultrasensitive enzymatic method. Before lavage, lung mechanics in pressure controlled synchronized intermittent mandatory ventilation was analyzed. RESULTS: Phospholipase A2 was higher in babies ventilated for sepsis/pneumonia compared to hyaline membrane disease and to control babies. Phospholipase correlated negatively with dynamic compliance, positively with inspired oxygen fraction, mean airway pressure and oxygenation index. These correlations still remained significant after multivariate analysis, adjusting for possible confounding factors. Phospholipase was not correlated with blood and alveolar pH, gestational age, birth weight, blood gases, Apgar score, tidal volume, surfactant need and ventilation time. CONCLUSIONS: These are the first data about phospholipase A2 in neonates. The enzyme plays a role in neonatal lung injury, especially in infection related respiratory failure. It is associated with lung stiffness, higher mean airway pressure and need for oxygen.

Abstract: Our aim was to study the usefulness of jaundice visual assessment combined with skin bilirubin determination in 517 healthy newborns. Yellowness assessment was made and babies were included in three different bilirubin classes. Skin bilirubin and total serum bilirubin were determined within 10 minutes from the visual assessment. This latter led to underestimation of serum bilirubin in 16.7-40.4% and overestimation in 4.9-35.7% of newborns. Skin bilirubin measurement after the visual assessment decreased the risk of underestimation to 0-9.2% and the risk of overestimation to 2.1-11.1%. The majority of visual assessment errors were performed in the more lighted hours of the morning (75%), while the smallest number (39%) occurred during the afternoon. Skin bilirubin measurement significantly corrected these diagnostic errors (p < 0.001, p < 0.02) without differences during the day. Clinical estimate is unreliable for evaluating the need for serum bilirubin assay. Using the addition of skin bilirubin determination is a more advisable approach.

Abstract:

Abstract: We report on a premature infant with glucose-6-phosphate dehydrogenase deficiency and severe hyperbilirubinemia. In this patient, all known potential hemolytic agents were excluded and no findings of hemolysis were observed. The crucial role of prematurity in the pathogenesis of this type of jaundice is discussed.

Abstract: An 11-year-old male developed a severe respiratory failure due to a iatrogenic flail chest following a surgery for removing a large chest wall area. A rare Ewing sarcoma was histologically diagnosed and intensive chemotherapy was administered. Postoperatively, because of the failure in ventilation weaning, the patient was electively extubated and noninvasive positive pressure ventilation through face-mask was provided. Respiratory support avoided asynchronous paradoxical movements and achieved pneumatic stabilization. Clinical and respiratory improvement allowed a successful weaning from ventilator.

Abstract:

Abstract: BACKGROUND: BiliCheck (BC), a new transcutaneous bilirubinometer is thought to be lacking in the disadvantages of old devices and could be potentially useful for diagnosing jaundice in preterm babies. Although its accuracy is well known in healthy term babies, there is a lack of knowledge about its usefulness in preterm infants. AIMS: To investigate BC usefulness in preterm babies and its suitability in a sub-intensive neonatal unit. STUDY DESIGN: In 340 preterm infants between 30 and 36 weeks of gestational age, transcutaneous and serum bilirubin measurement were performed. Hematocrit, pH, postnatal age, gestational age, and sex were also studied to clarify their influence on BC accuracy. For a subset of 100 neonates transcutaneous measurement, blood collection and serum analysis were timed and costs were considered. RESULTS: Correlation coefficient is 0.795 (p<0.001) and this is not affected by factors previously supposed to be important. Overall sensitivity was 100% and specificity were comprised between 40% and 72%. BC has a tendency to overestimate serum bilirubin, at high values. Considering the whole time for serum bilirubin measurement, transcutaneous bilirubinometry is a faster (p<0.0001), but more expensive technique with a cost of about 5 euro/measurement. Nevertheless, using BC as a screening-device we could safely avoid 58-79% of blood samples, since its positive predictive values is about 21-42%. This would allow to a cost reduction of 1555-2120 euro/year. CONCLUSIONS: BC has a good reliability in preterm infants although not as good as in healthy term babies. BC is a time-sparing tool and can improve the management of neonatal jaundice in preterm infants; however, its tendency to overestimate suggests its use only for screening purposes.

Abstract: OBJECTIVE: To assess the effectiveness of an incomplete course of antenatal corticosteroids (ACS) on neonatal morbidity and mortality of preterm infants. METHODS: Preterm infants born at 25-34 weeks' gestational age between January 1, 1998 and December 31, 2003 were included in this study. Studied infants were divided in two groups: the ACS group included those infants who had been exposed to a single 12-mg dose of betamethasone before delivery while the control group included those infants who had been delivered without any antenatal corticosteroids treatment. The most important neonatal outcomes were compared between the two groups. RESULTS: One hundred and seventy neonates (41.4%) were exposed to one 12-mg dose of betamethasone before delivery, while 241 neonates (58.6%) did not receive any antenatal corticosteroids treatment. Mean gestational age at delivery (30.4+/-2.4 weeks versus 31.2+/-2.9 weeks, p=0.004) and mean birth weight (1375+/-454 g versus 1625+/-580 g, p<0.001) were lower in the ACS group. The univariate analysis showed that delivery room intubation and respiratory distress syndrome were more frequent in the ACS group and that the length of stay was also significantly longer in this group. No differences were found concerning survival, neonatal morbidity, need for and duration of mechanical ventilation and oxygen therapy. The incidence of major outcomes in survivors was also similar. Logistic regression adjusted for gestational age showed that the exposure to a single dose of betamethasone before delivery was not associated with a significant reduction in the rate of any neonatal outcome. We also compared the outcomes in function of gestational age subclasses. In the 25-27 weeks subgroup, delivery room intubation, surfactant treatment and patent ductus arteriosus (PDA) were less frequent in ACS infants; they had also shorter ventilation and oxygen duration. In the 30-31 weeks subgroup, ACS infants had a lower incidence of mechanical ventilation and a shorter duration of oxygen therapy. Finally, no differences were found in the 28-29 weeks subgroup and in the 32-34 weeks subgroup. CONCLUSION: Effects of incomplete antenatal corticosteroids are variable: they give some benefits to infants of 25-27 weeks gestational age, fail to show any difference in outcomes in the 32-34 weeks subgroup and are doubtful between these extremes.

Abstract:

Abstract: AIM: To assess the correlation of echocardiographic signs of myocardial damage to serum cardiac troponin T (cTnT) concentrations in newborn infants with perinatal asphyxia. METHODS: Electocardiograms (ECG) and echocardiograms (Echo) were obtained during the first 24 h of life from 29 asphyxiated and 30 control infants and correlated with cTnT concentrations. The echocardiographic parameters included systolic ventricular performance, preload, afterload, diastolic function, stroke volume (SV), left ventricular output (LVO), hyperechogenity of the papillary muscles and insufficiency of the atrioventricular valves. RESULTS: LVO and SV were lower but CTnT were significantly higher in asphyxiated than in control infants: 0.15 (010-0.23) vs. 0.05 (0.02-0.13), p < 0.001). Asphyxiated infants with signs of myocardial damage were associated with significantly higher cTnT than those without, 0.20 (0.11-0.28) and 0.11 (0.05-0.14 ug/L), p = 0.04. CONCLUSION: Cardiac troponin may prove to be valuable in evaluating myocardial damage in birth asphyxia. However, the degree of prematurity may complicate the assessment.

Abstract: From 1996 the territory of Tuscany region and in particular that one of ASL 10 of Florence, has been interested from the realization of great works: the railway line to High Speed and varying of Valico of the A1 freeway between Florence and Bologna. ASL 10 has stipulated specific economic agreements with the been involved companies in the realization of the plans for a plan finalized to the prevention of the industrial accidents and to limit of the gravity with timely aids it adapts to you also in gallery, to guarantee to the workers the same performances sanitary of the city residents, to guarantee and to improve the quality of existing sanitary services on the territories in spite of the increase of the population weighing on the consequent territory to the takeover of the workers it engages to you in the realization of the great works. This plan has been realized with the creation of integrated aid system a to strengthen the territorial sanitary emergency, I extend the access to the base medicine to the workers and has not been able itself to answer to 93% of the question sanitary to the inside of the same yards with the infirmaries of yard without to resort to external structures.

Abstract:

Abstract: OBJECTIVES: We sought to verify the association between maternal intrahepatic cholestasis of pregnancy (ICP) and neonatal respiratory distress syndrome (RDS) and to determine how bile acids levels alter the risk of developing neonatal RDS. METHODS: We extracted data from our divisional database about all of the newborns born during the years 2000-2004. We compared 77 neonates born from pregnancies complicated by ICP with 427 neonates in the same range of gestational age born from noncomplicated pregnancies. We studied maternal bile acids levels immediately before delivery in mothers with ICP and measured bile acid levels during the first 24 hours of life in their newborns. RESULTS: The incidence of RDS in newborns from cholestatic pregnancies was twice that the reference population (28.6% vs 14%). The multivariate analysis showed that the risk of RDS in these newborns was approximately 2.5 times higher than in control infants. Within the ICP group, maternal and neonatal bile acid levels of infants affected by RDS were not significantly higher than those of healthy infants. The multivariate analysis showed that a low gestational age was the most important risk factor, but the probability of respiratory distress syndrome also increased by 2 per thousand for every additional micromole of the interaction term "neonatal by maternal bile acids level." CONCLUSIONS: Maternal ICP is significantly associated with the occurrence of RDS in the newborn. We hypothesize that bile acids can produce surfactant depletion in the alveoli reverting the reaction of phospholipase A2. This hypothesis could potentially be confirmed by bronchoalveolar lavage study.

Abstract: A case of a pregnancy occurring in a woman with previously diagnosed Wegener's granulomatosis and the following neonatal follow-up are described. Complete clinical and laboratory disappearance of disease activity was achieved by steroid treatment before pregnancy. The newborn was followed up for 6 months; he always showed normal clinical and laboratory exams, except a mild and transient neutropenia.

Abstract: OBJECTIVE: To investigate the effect of different delivery room strategies on survival, short term morbidity, and outcomes in extremely premature infants. METHODS: This retrospective cohort study included all preterm infants with a gestational age between 24 and 28 weeks who were born in 1992-1997 (period A; n = 161) and in 1998-2003 (period B; n = 163). In period A, elective intubation was performed. In period B, if spontaneous breathing was present, nasal continuous positive airway pressure (nCPAP) was applied. RESULTS: Survival rate and the number of never-intubated infants significantly increased in period B. No differences were found concerning short-term morbidity. Among major outcomes, the need for retinopathy of prematurity (ROP) surgery and the length of stay were significantly lower in period B. Subgroup analysis showed no significant differences from period A to period B in infants with gestational age 24-26 weeks. In the 27-28 weeks subgroup, the never-intubated infants rate increased from 2.8% to 21.3% and survival rate increased from 63% to 79%. A reduced need for ROP surgery and a shorter hospital stay were also observed. CONCLUSIONS: Changes in delivery room strategy tending to reduce mechanical ventilation in extremely premature infants are likely to benefit essentially infants of 27-28 weeks of gestation. Extension of such benefits to premature infants at the limit of viability requires further research.

Abstract: Leukocyte adhesion deficiency (LAD) is a rare disorder of cellular immunity, generally due to various mutations producing reduced or altered expression of membrane integrins. The authors report a case of LAD due to integrins expression imbalance. LAD was suspected after recurrent sepsis, fungal infection and amoebiasis with persistent leukocytosis. Neutrophils were studied with chemiluminescence showing decreased functional activity: up to now, this seems the first chemiluminescence study of neutrophil function and the first report of amoebiasis at the onset in LAD.

Abstract:

Abstract: Neonatal Lupus Syndrome is a rare disease caused by placental passage of maternal autoantibodies. Pathogenesis is partially unknown and many clinical manifestations are possible. We report on newborn siblings who presented with different symptoms of Neonatal Lupus Syndrome. One patient presented with congenital heart block and another with hepatic and haematologic involvement. Cases of Neonatal Lupus among siblings are very rare, because of the high risk of pregnancy in affected women. Various clinical expressions may be explained by a different specificity of Anti-Ro autoantibodies among siblings. The reported cases are commented with regard to recent literature, trying to explain their pathogenesis.

Abstract: A prospective study was performed enrolling 11 newborns with neonatal lupus syndrome (NLS) and 22 control newborns to investigate cerebral ultrasound (US) anomalies and their relationship with clinical neurological signs and laboratory findings. Cerebral US detected a significantly higher incidence in the study group of both subependymal pseudocysts (SEPC) and subependymal hemorrhage (SEH), neither of which correlated to autoantibody levels. All infants had completely normal neurological examinations both at birth and follow-up. The etiopathogenesis of central nervous system findings in NLS is discussed. US evaluation identified minimal anomalies compatible with favorable outcome: further studies are necessary to investigate the possible long-term sequelae, pathogenesis and spectrum of cerebral US findings.

Abstract: Neutropenia is a mild and transient manifestation of neonatal lupus syndrome (NLS) in the second or third month of life. The authors describe a newborn with an early-onset severe neutropenia due to anti-Ro/SSA. In the second day of life, neutropenia has been treated with recombinant human granulocyte colony-stimulating factor (rh-G-CSF). This is the first case in which rh-G-CSF was used in NLS, and the authors studied the pharmacologic action of the drug in relationship to the pathophysiology of NLS.

Abstract:

Abstract: The Authors report an update relative to the dietetic prevention strategies in the high allergic risk subject, as proposed from more recent literature. The babies with a familiar history of atopia are defined as population with allergic risk. The Authors examine the role of early exposure to cow's milk formulas and maternal diet during breast-feeding as risk factors for allergic symptoms in such babies. Moreover, they examine the indications for hydrolisated milk (partial and extensive) formulas and soy milk formulas use, as reported in published Meta-analysis and official statements of several Scientific Associations. They conclude that beyond the undoubted preventive role of exclusive breast-feeding in the first 4-6 month after birth, and of the extensively hydrolisated formulas, there are many concerns about the role for partially hydrolisated formulas and soy formulas. The Authors claim for multicentric methodologically correct trials in order to clear the controversies.

Abstract: Neonatal alloimmune thrombocytopenia (NAIT) is usually attributable to HPA-1a antibodies. The authors report a case of incidentally diagnosed thrombocytopenia in a small for gestational age infant. A NAIT was suspected and she was successfully treated with intravenous IgG. The direct and indirect platelet suspension immunofluorescence test (PSIFT) in maternal blood suggested alloimmunization to HPA-5b. Empiric treatment with IgG could be useful in case of clear suspect even in absence of confirmed diagnosis.

Abstract: