Search

Background: The optimal management of PDA in very low birth weight (VLBW) infants is still controversial. Aim of our study was to investigate the management of PDA in the Italian neonatal intensive care units (NICU)., Methods: We conducted an on-line survey study from June to September 2017. A 50-items questionnaire was developed by the Italian Neonatal Cardiology Study Group and was sent to Italian NICUs., Results: The overall response rate was 72%. Diagnosis of PDA was done by neonatologists, cardiologists or both (62, 12 and 28% respectively). PDA significance was assessed by a comprehensive approach in all centers, although we found a heterogeneous combination of parameters and cut-offs used. None used prophylactic treatment. 19% of centers treated PDA in the first 24 h, 60% after the first 24 h, following screening echocardiography or clinical symptoms, 18% after the first 72 h and 2% after the first week. In the first course of treatment ibuprofen, indomethacin and paracetamol were used in 87, 6 and 7% of centers respectively. Median of surgical ligation was 3% (1-6%)., Conclusions: Significant variations exist in the management of PDA in Italy. Conservative strategy and targeted treatment to infants older than 24 h with echocardiographic signs of hemodynamic significance seemed to be the most adopted approach.

Background: Neonatologist performed echocardiography (NPE) has increasingly been used to assess the hemodynamic status in neonates. Aim of this survey was to investigate the utilization of NPE in Italian neonatal intensive care units (NICUs)., Methods: We conducted an on-line survey from June to September 2017. A questionnaire was developed by the Italian neonatal cardiology study group and was sent to each Italian NICU., Results: The response rate was 77%. In 94% of Italian NICUs functional echocardiography was used by neonatologists, cardiologists or both (57, 15 and 28% respectively). All the respondents used NPE in neonates with patent ductus arteriosus and persistent pulmonary hypertension, 93% in neonates with hypotension or shock, 85% in neonates with perinatal asphyxia, 78% in suspicion of cardiac tamponade, and 73% for line positioning. In 30% of center, there was no NPE protocol. Structural echocardiography in stable and critically ill neonates was performed exclusively by neonatologists in 46 and 36% of center respectively., Conclusions: NPE is widely used in Italian NICUs by neonatologists. Structural echocardiography is frequently performed by neonatologists. Institutional protocols for NPE are lacking. There is an urgent need of a formal training process and accreditation to standardize the use of NPE.

Functional gastrointestinal disorders (FGIDs) definition in children has changed over the years trying to facilitate clinicians, because the diagnostic process is complicated by the interpretation variability of symptoms described by children or by their caregivers for newborns and toddlers. This review refers to the Rome IV classification system, drafted in 2016. FGIDs pathophysiology is multifactorial and still poor understood, with limitations for the therapeutic process, which results often in unnecessary and alternative treatments trying to reduce the relevant caregiver distress, but with increased costs for families and for the National Health Service. This study reports the most recent evidence-based treatments for FGIDs in newborns: though in most cases the first action is an educational and behavioral intervention, reassuring caregivers about the transient and self-limiting natural history of FGIDs, there is now more evidence to recommend probiotics in some infant FGIDs.

Although breastfeeding is associated with many health benefits in children and mothers, and World Health Organization (WHO) recommends exclusive breastfeeding until 6 months of age and continued breastfeeding until 2 years of age, overall breastfeeding rates remain low. Italian rates of exclusive breastfeeding do not differ from international data. The aim of this review is to evaluate evidence of breastfeeding promotion interventions and the remaining problems to achieve them. We found that breastfeeding support is a complex system of interventions, including individual, structural and environmental factors. Many systematic reviews report evidence that breastfeeding support offered to women increases duration and exclusivity of breastfeeding, both in full term healthy newborns and in preterm infants. Political and economic efforts should be made to ensure breastfeeding support to all women in the different settings, assuming it as a collective target.

We report on a mother-to-child transmission of KPC carbapenemase-producing Klebsiella pneumoniae at birth followed by subsequent cases in the neonatal intensive care unit. Molecular analysis of isolates showed production of KPC-3 enzyme. The only potential risk factor identified for the mother was previous activity as a caregiver. Present findings suggest consideration of proactive surveillance in pregnant women with risk factors for colonization.

There is a poor association between systemic blood flow and blood pressure in preterm infants in the first days of life. Very preterm babies can have early low systemic blood flow with poor myocardial contractility but normal blood pressure. Superior vena cava flow measured by echocardiography can be used to determine systemic blood flow. Low superior vena cava flow is a risk factor for mortality and morbidity in preterm infants. We report the case of preterm twins with contrasting relationships among systemic blood flow, myocardial contractility and blood pressure. The first twin with normal blood pressure, but low superior vena cava flow and poor myocardial contractility in the first hours of life developed a severe intraventricular haemorrhage and died, despite cardiovascular support. The second twin with low blood pressure but normal superior vena cava flow did not develop adverse outcomes in the first days of life. These cases underline the usefulness of neonatal functional echocardiography in early understanding and management of the preterm infant, and the inaccuracy of other clinical parameters used to assess cardiovascular function., (© 2011 The Authors. Journal of Paediatrics and Child Health © 2011 Paediatrics and Child Health Division (Royal Australasian College of Physicians).)

Phosphatase and tensin homolog (PTEN) gene mutations are associated with a spectrum of clinical disorders characterized by skin lesions, macrocephaly, hamartomatous overgrowth of tissues, and an increased risk of cancers. Autism has rarely been described in association with these variable clinical features. At present, 24 patients with phosphatase and tensin homolog gene mutation, autism, macrocephaly, and some clinical findings described in phosphatase and tensin homolog syndromes have been reported in the literature. We describe a 14-year-old boy with autistic disorder, focal epilepsy, severe and progressive macrocephaly, and multiple papular skin lesions and palmoplantar punctate keratoses, characteristic of Cowden syndrome. The boy has a de novo phosphatase and tensin homolog gene mutation. Our patient is the first case described to present a typical Cowden syndrome and autism associated with epilepsy.

Background: Patent ductus arteriosus (PDA) in preterm infants is often assessed with echocardiographic parameters, especially colour Doppler ductal diameter and pulsed Doppler flow pattern. Clinical algorithms have been proposed in which PDA treatment is indicated by either large diameter or a particular flow pattern, however it is unknown whether ductal diameter and flow pattern provide equivalent stratification of infants., Aim: Retrospectively assess both parameters in 197 echocardiograms from 104 infants (gestational age <31 weeks)., Methods: Echocardiograms were independently reviewed and the internal colour Doppler diameter of the PDA and the pulsed Doppler flow pattern were characterised for each study (169 records had both parameters recorded)., Results: Diameter varied widely within each group but was significantly associated with flow pattern: mean diameter was greatest in the pulmonary hypertension (PH) group (2.6 mm), progressively narrowed across growing and pulsatile groups, and was smallest in the closing group (1.3 mm). When echocardiograms were categorised using previously published diameters, 82.4% of the PH group had diameters >2.0 mm, large diameters predominated in the growing and pulsatile groups but to a progressively smaller extent, and 98.1% of closing group had diameters <2.0 mm., Conclusion: Ductal diameter and flow patterns are significantly associated, consistent with a narrowing of the ductus until closure. Overall, the two parameters are in good agreement but will result in different treatment decisions in some cases. Clinicians might consider using both methods as a cross check against each other, to assist in the management of preterm infants with a clinically detectable PDA.

Migraine is a common disorder in childhood and adolescence. Studies on adults show the effectiveness and tolerability of riboflavin in migraine prevention, while data on children are scarce. This retrospective study reports on our experience of using riboflavin for migraine prophylaxis in 41 pediatric and adolescent patients, who received 200 or 400 mg/day single oral dose of riboflavin for 3, 4 or 6 months. Attack frequency and intensity decreased (P < 0.01) during treatment, and these results were confirmed during the follow-up. A large number of patients (77.1%) reported that abortive drugs were effective for controlling ictal events. During the follow-up, 68.4% of cases had a 50% or greater reduction in frequency of attacks and 21.0% in intensity. Two patients had vomiting and increased appetite, respectively, most likely for causes unrelated to the use of riboflavin. In conclusion, riboflavin seems to be a well-tolerated, effective, and low-cost prophylactic treatment in children and adolescents suffering from migraine.

Objective: To describe methanol poisoning exposures in Italy and to investigate a cluster of methanol-related deaths due to abuse occurred in Sicily among immigrants from East Europe., Design: the human methanol exposures handled by the Poison Control Centre of Milan, the major national centre for toxicological advise active in Italy, were reviewed retrospectively (January 2004-September 2006) and prospectively (October 2006-October 2008)., Setting: For each case of managed human exposure, the Poison Control Centre of Milan uses a standard format to collect patient information, substance/exposure information, clinical effects, therapy, outcomes. The database arising from that procedure was searched for all cases exposed to methanol and/or reporting selected clinical signs (i.e., metabolic acidosis with ocular effects and/or central nervous system depression and/or respiratory depression). Each case was reviewed and classified according to standard criteria., Main Outcomes: Three commercial products containing more than 70% methanol available on the market in Sicily and associated with lethal exposures due to abuse among immigrants were identified., Results: In the period under study, the Poison Control Centre of Milan examined 29 methanol poisoning exposures (none in 2004, 6 in 2005, 6 in 2006, 15 in 2007, and 2 in 2008). Among them, 17 occurred in Sicily, with a peak of 11 cases in 2007, and 12 in other regions. The patients included 16 men and 13 women with a median age of 49,5 years (range 1-81 years). Fifteen cases were immigrants from East Europe (14 Rumanian and one Polish). Eleven cases reported minor effects, two cases moderate effects, four cases major effects, and 12 cases died. Twelve cases were accidental exposures (5 occurred at work, 3 at home and 4 in other places), while 17 cases were intentional exposures (14 due to abuse, 2 to attempted suicide and 1 to misuse). Minor effects were reported in 11 cases, moderate effects in two cases, major effects in four cases and death in 12 cases. The most frequently reported clinical effects included: coma (13 cases), metabolic acidosis (10 cases), mydriasis (4 cases), visual loss (3 cases), gastric pyrosis (2 cases), constriction of the visual field (2 cases), and vertigo (2 cases). Blood methanol level was reported for 15 cases (< 50 mg/dL in 2 cases; 50-99 mg/dL in 2 cases; 100-499 mg/dL in 4 cases; >500 mg/dL in 1 case; positive, unknown value in 6 cases). All cases due to abuse (n. 14) and, among them, lethal (n. 12) occurred in Sicily and involved immigrants. On the other hand, all methanol poisonings occurring in the other Italian regions involved Italian citizens unintentionally exposed and with minor effects, except for one case of attempted suicide with consequent major effects. The investigations performed in Sicily lead to identifying three locally produced and marketed detergents containing more than 70% methanol, improperly labelled and packaged. Furthermore, a detergent for domestic use was identified containing 25-30% methanol without any labelling information. Two of the detergents containing more than 70% methanol were withdrawn from the market in November 2007, while the other, that was identified later, was withdrawn in July 2008. The detergent for domestic use, containing 25-30% methanol was withdrawn in December 2007. Starting from July 2008, no new cases of methanol poisonings were observed., Conclusion: The availability in Sicily of products containing high concentrations of methanol, improperly packaged and labelled, determined a generalised risk of accidental exposures and favoured voluptuary assumption of methanol among selected groups. The observations highlight the importance of a surveillance system of toxic exposures based on the information collected by the Poison Control Centres and its ability to provide timely identification of unexpected and dangerous events and to support preventive actions.

In the last two years we discovered that three of our patients with type 1 diabetes mellitus (0.8%) suffered an unexpected worsening in their glycemic control due to a reduction of their insulin dosage in favour of some "alternative" diabetes treatments using herbs, vitamins, fantastic diets and trace elements prescribed by non-medical practitioners. The first patient, a 6.6 year old boy, was admitted to hospital because of a severe ketoacidosis with first degree coma as a result of his parents having reduced his insulin dosage by 77% and replacing the insulin with an ayurvedic herbal preparation (Bardana Actium Lapp). The second patient, a 10.4 year old boy, was admitted to hospital after his teachers noticed that he appeared tired, thinner and polyuric. During hospital admission for mild ketoacidosis the mother, reluctant at first, finally confessed that her son was under the care of a "clinical ecologist". Having identified several food allergies this "clinical ecologist" had placed the child on a spartan diet of bread, water and salt, and had reduced his insulin dosage by 68%. The third patient, a 21 year old male, upon transfer to the Adult Diabetic Center, reported that he had been under the care of a pranotherapist for several years. The pranotherapist had prescribed a cellular nutrition preparation (called "Madonna drops"), a meditation program and also a 50% reduction in his insulin dosage. During this period his HbAlc values had increased from 6.4% to 12%. Current orthodox diabetes treatments are considered unsatisfactory by many people and it is thus not surprising that they search for "miracle" cures. It is important, however, that hospital staff do not ridicule the patients or their parents for trying these alternative therapies. Nevertheless, it would be useful for staff to discuss in advance these "therapies" with patients, highlighting their ineffectiveness and strongly discouraging cures that call for a reduction or elimination of the insulin treatment.

The incidence of hepatitis C in childhood is approximately 0.4%. The mode of transmission can be parenteral, sexual, occupational and also vertical. The latter has an incidence that varies widely and it increases in the case of human immuno-deficiency virus (HIV) coinfection and high titers of HCV in the mother. The vertical transmission is not influenced by breast feeding, however, data are discrepant with regard to child delivery (cesarean section vs vaginal delivery). Ninety-seven babies born from mothers with hepatitis C from 1996 to 1999, were evaluated prospectively in the Day Hospital of the Pediatric Department of Parma. The protocol of observation established a blood sampling for titers of antibodies anti-HCV and HCV-RNA at the 3rd trimester of pregnancy and subsequent clinical and biochemical controls at 3-6-9-12-15 and 18 months. Thirty (31.2%) out of the 96 mothers evaluated were positive for antibodies anti-HCV and 66 (68.8%) were positive for antibodies anti-HCV and HCV-RNA. Five (5.15%) out of the 97 babies evaluated were infected by HCV. Of these 4 were delivered vaginally and 1 by cesarean section. Of the 3 babies born to mothers with HIV coinfection, none was infected by HIV, but 1 was infected by HCV. Vertical transmission is increased by HCV viral load or HIV coinfection in the mother. The vaginal delivery and breastfeeding do not represent an additional risk factor.

Neonatal diabetes (ND) is a rare condition of hyperglycaemia which occurs, generally, within the first month of life, requires insulin therapy and lasts more than two weeks. The pathogenesis is uncertain and the different clinical variants are heterogeneous; both a "permanent" and a "transient" form of ND exist. We report the clinical history of three neonates (A.L., A.B. and V.I.) in which a tout-court diagnosis of ND was done; the follow-up allowed us to reevaluate the initial diagnoses and suppose different ethiopathogenic mechanisms. Clinical observations, insulin requirement and biochemical data obtained during the years of follow-up allowed to distinguish three different situations: a "permanent" ND (A.L.), a "transient-recurrent" ND (A.B.), and a Maturity Onset Diabetes of the Young (MODY) (V.I.).