Cutis marmorata telangiectatica congenita in a preterm female newborn: case report and review of the literature.

Cutis Marmorata Telangiectatica Congenita (CMTC) is a rare, sporadic condition usually present at birth characterized by localized or generalized persistent cutis marmorata, telangiectasia and phlebectasia. We report a preterm female newborn, the third child of non-related caucasian parents, with CMTC at birth who showed typical cutaneous features and monolateral congenital glaucoma. The pathogenesis of this disorder is unknown and the cause is probably multifactorial. Teratogens and autosomal dominant mode of inheritance with incomplete penetrance have been considered as etiological factors. Prognosis, in uncomplicated cases, is good.

A rare case of neonatal sepsis/meningitis caused by Pasteurella multocida complicated with status epilepticus and focal cerebritis.

Pasteurella multocida is normally present in respiratory and digestive tract of many domestic and wild animals, but is a rare pathogen in neonatal infection. Here we describe for the first time a case of meningitis complicated by status epilepticus and right parietal lobe cerebritis. The patient showed a dramatic clinical onset characterized by septic appearance and prolonged seizures. Multidrug anticonvulsivant therapy was used to control the status epilepticus, but despite the aggressive treatment electrical crises were still evident 24 hours after the admission. Furthermore, a brain MRI, performed to investigate a persistent intermittent fever even if CSF became sterile, showed a focus cerebritis in the right parietal lobe, early stage of the cerebral abscess. Prolonged antibiotic therapy with steroids was requested to solve the cerebritis area. Interestingly, direct contact between the patient and domestic animals was denied by the family, but the father reported a contact with a rooster, killed and cooked few days before, suggesting, as previously described, that Pasteurella may also be transmitted through asymptomatic human carrier. The patient had a favourable outcome with no medium-term sequelae one month after discharge, but the severity of the clinical course and the unpredictable way of transmission highlight the importance of hygiene measures approaching infants.

Brain cooling therapy.

Therapeutic hypothermia (whole body or selective head cooling) is becoming standard of care for brain injury in infants with perinatal hypoxic ischemic encephalopathy (HIE). Brain cooling reduces the rate of apoptosis and early necrosis, reduces cerebral metabolic rate and the release of nitric oxide and free radicals. Animal models of perinatal brain injury show histological and functional improvement due to of early hypothermia. The brain protection depends on the temperature and time delay between insult and beginning of treatment (more effective with cooling to 33 +/- 0.5 degrees C, and less than 6 hours after hypoxic-ischemic insult). Recent meta-analyses and systematic reviews in human neonates show reduction in mortality and long-term neurodevelopmental disability at 12-24 months of age, with more favourable effects in the less severe forms of HIE. The authors describe their experience in 53 term newborns with moderate-severe HIE treated with whole body cooling between 2001 and 2009, and studied with magnetic resonance imaging (MRI) and general movements (GMs) assessment. The creation of a network connecting the Neonatal Intensive Care Unit with the level I-II hospitals of the reference area, as part of regional network, is of paramount importance to enroll potential candidates and to start therapeutic hypothermia within optimal time window.

[Neonatal group B Streptococcus infection: an integrated approach (of clinical pathologists, gynecologists, midwives, pediatricians-neonatologists) of the functional area of Cuneo (Italy)]. / Infezione neonatale da Streptococco B: un approccio multidisciplinare integrato (di laboratoristi, ginecologi, ostetriche e pediatri-neonatologi) del Quadrante di Cuneo.

The frequency of early-onset neonatal sepsis without prophylaxis is 1-5/1.000 live births. Since year '70 the most frequent causative microorganism is the group B Streptococcus (S. agalactiae, GBS), followed by Escherichia coli. The mortality rate is now reduced to 4% due to the improvement of neonatal intensive care. In the USA, the incidence of GBS early-onset neonatal sepsis has been markedly reduced by the application of the guidelines released by the Centers for Disease Control (CDC). This strategy, however, is not effective on occurrence of late-onset neonatal group B streptococcal disease. In Italy, the application of CDC guidelines is not customary, and different, often complex, protocols of obstetrical-neonatological integrated approach are applied. The frequency of infectious risk has made the GBS a paramount problem for the neonatologist, even for the legal responsibility issues resulting from the multiplicity of possible options. To reach the best level of protection of the newborn against early-onset GBS infection, the working group of providers of prenatal, obstetric, and neonatal care of the functional area of Cuneo issued an integrated protocol, in order to perform the GBS screening with the optimal culture method suggested by CDC guidelines in the highest possible number of pregnant women, and to standardize the obstetrical and neonatal management.

[Ethics in perinatology]. / Etica in perinatologia.

The perinatal phase coincides, at its onset (22nd-23rd week of gestation, according to common acknowledgment), with the possible beginning of a extrauterine life susceptible of protraction. The 1st, for some the 4th, week of neonatal life marks the conclusion of the perinatal phase. There are several reasons to burden this period, to which a complex World Health Organization (WHO) document for Europe was dedicated in November 2000, with ethical issues. The profound immaturity that characterizes these extremely low weight newborns, of an approximate weight of 500 g, influences the poor survival prognosis (especially for those born within the 24th week), as well as the limitations concerning quality of life, often severe. Neonates born earlier than the 23rd week are at extreme risk, and rouse critical considerations regarding the choice of a health care program. A close monitoring of pregnancies at risk of premature termination, and a careful program of medical care for these extremely pre-terms, progressively implemented in the last 10-15 years, have given consistent results, reported in surveys of recent publication. From an ethical point of view, the problem of limitations within or beyond which to stretch intensive care interventions in Neonatal Intensive Care Unit (NICU) is still crucial, being the orientation between rational and emotional a difficult issue. Guidelines or behaviour proposals, variable with time and manifold in different countries, are reported. Naturally, the communication of a severe diagnosis to parents of a newborn even if not preterm-born falls also under the ethical issues of the perinatal period.

Prophylactic nasal continuous positive airways pressure in newborns of 28-31 weeks gestation: multicentre randomised controlled clinical trial.

BACKGROUND: The role of nasal continuous positive airways pressure (nCPAP) in the management of respiratory distress syndrome in preterm infants is not completely defined. OBJECTIVE: To evaluate the benefits and risks of prophylactic nCPAP in infants of 28-31 weeks gestation. DESIGN: Multicentre randomised controlled clinical trial. SETTING: Seventeen Italian neonatal intensive care units. PATIENTS: A total of 230 newborns of 28-31 weeks gestation, not intubated in the delivery room and without major malformations, were randomly assigned to prophylactic or rescue nCPAP. INTERVENTIONS: Prophylactic nCPAP was started within 30 minutes of birth, irrespective of oxygen requirement and clinical status. Rescue nCPAP was started when Fio2 requirement was > 0.4, for more than 30 minutes, to maintain transcutaneous oxygen saturation between 93% and 96%. Exogenous surfactant was given when Fio2 requirement was > 0.4 in nCPAP in the presence of radiological signs of respiratory distress syndrome. MAIN OUTCOME MEASURES: Primary end point: need for exogenous surfactant. Secondary end points: need for mechanical ventilation and incidence of air leaks. RESULTS: Surfactant was needed by 22.6% in the prophylaxis group and 21.7% in the rescue group. Mechanical ventilation was required by 12.2% in both the prophylaxis and rescue group. The incidence of air leaks was 2.6% in both groups. More than 80% of both groups had received prenatal steroids. CONCLUSIONS: In newborns of 28-31 weeks gestation, there is no greater benefit in giving prophylactic nCPAP than in starting nCPAP when the oxygen requirement increases to a Fio2 > 0.4.

Complement-dependent bactericidal activity for E. coli K12 in serum of preterm newborn infants.

Complement-dependent serum bactericidal activity for E. coli K12 was assessed in 12 term infants and in 16 preterm infants. In both groups of newborns, at birth, bactericidal reaction by the classical pathway of complement activation was impaired with respect to normal controls at less than 0.001 level of significance (as estimated by Student's t-test). The serum bactericidal reaction by the alternative pathway of complement activation was significantly impaired only in preterm newborns, being normal in term infants. At a time corresponding to 40 weeks' gestational age also in preterm newborns alternative pathway mediated bactericidal activity for E. coli K12 was found normal. Classical pathway mediated bactericidal activity became normal only at an age corresponding to 52 weeks' gestational age.