Use of intravenous sodium bicarbonate in neonatal intensive care units in Italy: a nationwide survey.

BACKGROUND: Metabolic Acidosis (MA) is a disturbance of the acid-base balance that can occur in preterm and critically ill term neonates due to different etiologies. Intravenous sodium bicarbonate (SB) has been traditionally used to correct such unbalance, despite the lack of evidence about its safety and efficacy. In literature, reported undesirable effects of treatment with SB in neonates include worsening of intracellular acidosis, impairment of myocardial function, cerebral blood flow fluctuations and intracranial hemorrhage. A national survey was conducted by the Neonatal Pharmacotherapy Study Group of the Italian Society of Neonatology with the aim to assess and describe attitudes and practices concerning the use of SB, particularly for the treatment of MA in Italian NICUs. METHODS: A questionnaire regarding treatment of MA and SB prescription habits was sent to the directors of 120 Italian NICUs from June 2017 to March 2018. RESULTS: The survey response rate was 97.5% (117/120 centers). Findings showed that in 55% of the surveyed NICUs (64/117 units) it is common practice to correct MA with intravenous SB. On the other hand, the remaining 45% of the units try to solve the metabolic disturbances adopting different approaches (improving perfusion, adjusting ventilation parameters or increasing blood volume). Moreover, to prevent the occurrence of MA, 37.6% of the NICUs (44/117) include buffer salts (lactate, acetate or both) in parenteral nutrition prescriptions. SB is also used as a treatment for other conditions, mainly pathologies with bicarbonate loss and tubular acidosis (respectively in 53.8 and 32.5% of the NICUs). CONCLUSION: This survey showed how SB is a commonly used treatment for MA in more than half of Italian NICUs, with indications and prescription criteria that significantly vary across centers. Based on current knowledge, it is reasonable to suggest that the management of neonatal MA should be firstly directed to identify the underlying disorders. Thus, the use of SB should be reserved only for selected cases, also considering the severity of SB adverse effects and the lack of evidence about its efficacy. Guidance for the management of MA is required to harmonize practices and reduce the use of potentially inappropriate and unsafe treatments.

Rethinking the neonatal transport ground ambulance.

OBJECTIVES: This article describes the detailed project aimed to realize a dedicated ground ambulance for neonatal emergency transport service (NETS). To date, the European Community rules specify requirements for the design, testing, performance, and equipping of road ambulance used for transport and care of adult injured or ill patients, completely ignoring neonatal transport. METHODS: The project consisted of electric and gas supply planning, interior design taking into account ergonomic and occupant protection principles, both during travel and during medical care performances. RESULTS: A detailed project is presented. Main differences between European Type C ambulance and the new proposed Type D neonatal ground ambulance are the presence on board of air compressed cylinder, iNO cylinders and delivery system, phototheraphy, shock adsorbing stretcher support, cooling device, patient's placenta (refrigeration box), and transcutaneous gas analyzer. CONCLUSION: The European Community rules specify requirements for road ambulance used for transport and care of adult injured or ill patients, completely ignoring neonatal transport. This study describes the detailed project aimed to realize a dedicated ground ambulance for neonatal emergency transport service. This study demonstrated that it is not possible simply to adapt the currently dedicated ambulance for mobile intensive care and resuscitation services (actual type C European Community) in a modern dedicated NETS ambulance; it is of paramount importance suggesting to European Community to introduce a further ambulance type, to be identified type D, strictly reserved to neonatal transport activities.

Nationwide survey of neonatal transportation practices in Italy.

BACKGROUND: Despite regionalization of perinatal care provides for the "in utero" transfer of high-risk pregnancies, there will always be a number of neonates who undergo acute inter-facility transport. The presence of a well-organized Neonatal Emergency Transport Service (NETS) can prevent and reduce risks of transportation, especially for very preterm infants, and is therefore mandatory for any program of regionalization of perinatal care. Italian National Health System is highly decentralized and Regions are autonomous to structure, plan and delivery their regional health services. Consequently, organization models and resources available vary widely and significant regional differences in access and quality of health services have been reported in the past years. A national survey was conducted in 2015 by the neonatal transport study group of the Italian Society of Neonatology with the aim to describe neonatal transfer practices and to assess the Neonatal Emergency Transport Services (NETS) status in the 20 Italian regions. METHODS: A questionnaire regarding neonatal transfer practices and NETS activity for the previous year (2014) was sent to the 44 NETS operating in the 20 Italian regions. Demographic data were obtained from the Italian National Statistical Institute (ISTAT). RESULTS: The overall survey response rate was 100%. In 2014, only 12 (60%) of the 20 Italian regions were fully covered by NETS, 3 (15%) regions were partially covered, while neonatal transport was not available in 5 (25%) regions. Overall, in 2014, the 44 NETS operating in Italy transported a total of 6387 infants, including 522 (8.17%) having a gestational age < 28 weeks. CONCLUSIONS: The organization of NETS in Italy is devolved on a regional basis, resulting in a large heterogeneity of access and quality to services across the country. Where available, NETS are generally well-equipped and organized but limited volume of activities often cannot guarantee adequate levels of skills of personnel or an appropriate cost-efficiency ratio. The regions reported with lack of NETS have managed, or are trying, to fill the gap, but continuing efforts to reduce regional differences in the availability and quality of services are still needed.

Leukemia cutis in an infant with congenital leukemia and tetralogy of fallot.

Congenital leukemia is a very rare severe condition and leukemia cutis may represent the presenting sign of this malignancy, sometimes preceding hematological findings of weeks. Typical clinical features include multiple red to purple papules, macules and nodules due to direct infiltration of the skin by malignant cells. We illustrate these cutaneous findings in a patient with congenital leukemia and tetralogy of Fallot.

Infection in late preterm infants.

BACKGROUND: Late preterm (LP) are at higher risk than term infants to develop infections due to their more immature immune system. Little data about the risks and incidence of infection and sepsis in LP are present in literature. AIMS: To evaluate treated infection rates and risk factors for infection in moderate and late preterm infants (gestational age = 32-36 weeks). STUDY DESIGN: We retrospectively studied a population of 771 moderate and late preterm infants consecutively admitted to our unit from June 2008 to November 2013. RESULTS: Treated infections were 128, with an incidence of 16.6%; the 90% (n = 115) occurred during the first 72 hours of life. Blood cultures were positive in 22% of cases, umbilical venous catheter cultures were positive in 26% of cases; Coagulase-negative staphylococci were the most frequently isolated pathogens. Patients of the sepsis group had a C-reactive protein (CRP) mean value of 28.27 mg/L and a procalcitonin mean value of 25.3 µg/L. Risk factors for infections were umbilical venous catheter (UVC) insertion (χ(2) = 15.9; p ≤ 0.05), prophylaxis with antenatal corticosteroids (χ(2) = 16.7; p ≤ 0.05) and birth by cesarean section, with observed values very similar to the expected values (χ(2) = 15.9; p = 0.1). Respiratory symptoms were found in 47 of the 60 patients in the sepsis group (78.3%). CONCLUSIONS: Late and moderate preterm infants have an increased significant risk of infection compared to term infants. Infections, given the high frequency of negative cultures in neonates, should be often suspected and treated on the basis of clinical features and inflammatory markers, trying always to avoid a possible overtreatment.

Incidence, prevention, and treatment of parenteral nutrition-associated cholestasis and intestinal failure-associated liver disease in infants and children: a systematic review.

BACKGROUND: Cholestasis is a significant life-threatening complication in children on parenteral nutrition (PN). Strategies to prevent/treat PN-associated cholestasis (PNAC) and intestinal failure-associated liver disease (IFALD) have reached moderate success with little supporting evidence. Aims of this systematic review were (1) to determine the incidence of PNAC/IFALD in children receiving PN for ≥ 14 days and (2) to review the efficacy of measures to prevent/treat PNAC/IFALD. METHODS: Of 4696 abstracts screened, 406 relevant articles were reviewed, and studies on children with PN ≥ 14 days and cholestasis (conjugated bilirubin ≥ 2 mg/dL) were included. Analyzed parameters were (1) PNAC/IFALD incidence by decade and by PN length and (2) PNAC/IFALD prevention and treatment (prospective studies). RESULTS: Twenty-three articles (3280 patients) showed an incidence of 28.2% and 49.8% of PNAC and IFALD, respectively, with no evident alteration over the last decades. The incidence of PNAC was directly proportional to the length of PN (from 15.7% for PN ≤ 1 month up to 60.9% for PN ≥ 2 months; P < .0001). Ten studies on PNAC met inclusion criteria. High or intermediate-dose of oral erythromycin and aminoacid-free PN with enteral whey protein gained significant benefits in preterm neonates (P < .05, P = .003, and P < .001, respectively). None of the studies reviewed met inclusion criteria for treatment. CONCLUSIONS: The incidence of PNAC/IFALD in children has no obvious decrease over time. PNAC is directly correlated to the length of PN. Erythromycin and aminoacid-free PN with enteral whey protein have shown to prevent PNAC in preterm neonates. There is a lack of high-quality prospective studies, especially on IFALD.

Severe resistance to weight gain, lack of stored triglycerides in adipose tissue, hypoglycaemia, and increased energy expenditure: a novel disorder of energy homeostasis.

BACKGROUND: Growth during childhood is a consequence of the equilibrium of energy balance. Obesity results from a shift of the equilibrium towards increased energy intake over expenditure. A clinical description of extreme leanness and failure to thrive secondary to a shift of the equilibrium towards increased energy expenditure over energy intake has not been previously described in the medical literature. SUBJECTS AND METHODS: We report the case of a female child born premature with a birth weight of 1.1 kg who presented with extreme failure to thrive, persistent hypoglycaemia, paucity of fat in the adipose tissue with increased brown fat and increased resting energy expenditure. RESULTS: Complete cessation of weight and height was noted between 3 months to 3.5 years of age. Hypoglycaemia was secondary to depleted energy stores and increased insulin sensitivity. Increased resting energy expenditure was demonstrated on indirect calorimetric assessment. Biopsy of adipose tissue demonstrated paucity of stored fat with increase in brown fat. No gain in weight and height was demonstrated despite high calorie intake of enteral and parenteral feeds. CONCLUSION: We describe a unique case of extreme failure to thrive with increased energy expenditure and severe hypoglycaemia. Unravelling the molecular basis of this novel disorder has the potential to provide insights into the prevention of obesity.

Surgical treatment for pediatric mediastinal neurogenic tumors.

BACKGROUND: Mediastinal neurogenic tumors are relatively rare in children, and controversies persist about the best surgical approach and long-term survival. METHODS: With ethics approval we reviewed the case notes of 43 consecutive children (22 boys) operated on for mediastinal neurogenic tumor in two different institutions from March 1998 to June 2009. RESULTS: The average age at diagnosis was 3 years (range, 0.6 to 4.7 years). Of the 43 operated tumors, 20 were neuroblastomas (47%), 13 were ganglioneuroblastomas (30%), and 10 were ganglioneuromas (23%). Only 6 (14%) of these tumors were discovered incidentally; the remaining 37 (86%) presented symptomatically: cough (30%), dyspnea (21%), wheezing (11.6%), neurologic spinal compression (7%), dancing eyes syndrome (7%), and Horner syndrome (7%). Tumor resection was undertaken by open operation in 38 cases (88%) and by thoracoscopy in 5 cases (12%). The children operated on by means of thoracoscopy had significantly smaller tumors (p = 0.01) and shorter duration of thoracic drain (p = 0.011) and hospitalization (p = 0.016) than those who were operated on by thoracotomy. There was not any surgical death. Postoperative complications occurred in 11 children (25.6%). Tumor recurrence occurred in 4 children (9.3%) operated on by open surgery, and there were 2 deaths of children with neuroblastoma. The overall survival was 95.4% in an average follow-up of 3.5 years (range, 0.7 to 4.4 years). CONCLUSIONS: Surgical resection of children with mediastinal neurogenic tumors treated at two international tertiary hospitals was safe, and the thoracoscopic approach was appropriate for small tumors. The long-term survival was higher when compared with those reported for other primary neurogenic tumor locations.