Fire in the Operating Room During Hypospadias Repair

Fire in the operating room (OR) is a very distressful and shocking occurrence with potential dramatic consequences. Despite safety rules and rigorous recommendations, such unintentional events do occur every so often. Notably, the vast majority of cases have been reported in the adult population, with very few pediatric cases described to date. Herein, we report on a 16-month-old boy undergoing reconstructive surgery for penoscrotal hypospadias, who experienced an OR fire most likely related to the use of alcohol-based solution ignited by monopolar electrocautery

Feasibility, safety and outcome of inguinal hernia repair under spinal versus general anesthesia in preterm and term infants

Background: Inguinal hernia repair (IHR) is a common operation in preterm and term infants. Recently, spinal anesthesia (SA) has been proposed as an alternative to avoid exposure to general anesthesia (GA) during early life. The aim of this study was to compare surgical outcomes of open IHR performed under SA versus GA in neonates and infants, and to detect criteria to predict the success or failure of SA. Materials and methods: This is a 6-year, single center, nonrandomized interventional study (2013–2019). SA was performed with 0.5% bupivacaine. GA was given using propofol, fentanyl, sevoflurane, and laryngeal mask. Patient demographics, operative time, intraoperative events related to surgery or anesthesia, and complications were analyzed at short and long-term follow-up. Results: 68 infants (78 IHR) and 37 infants (44 IHR) received SA and GA at the discretion of the anesthesiologist, respectively. SA failure rate was 9%, and positively correlated with weight at surgery (p = 0.001; rp = 0.38). Conversion from SA to GA occurred in 4 (6%) patients owing to prolonged operative time (43.75 ± 4.8 vs 23.02 ± 11.3 min; p = 0.0006). There were no differences regarding operative time and intra- and postoperative complications among the two groups at mean follow-up of 18.53 ± 21.9 months. Conclusions: This pilot study confirms that SA is safe, effective and not detrimental to surgical outcome of neonates and infants undergoing IHR. Additionally, it may help further define what patients may have a successful SA. Our experience suggests that SA is especially suitable in infants weighing < 4000 g, and conversion to GA correlates with prolonged operative time. Level of evidence: Level II

Enzyme activity as an indicator of soil quality changes indegraded cultivated acrisols as the mexican trans-volcanic belt.

9 P.Soils located at the Mexican Trans-volcanic Belt (MTB) have a worrying degree of degradation due to inappropriate management practices. Early indicators of soil changes are very useful to alert about negative impacts of wrong managements on these volcanic soils. The aim of this work was to evaluate the short-term effects (4 years) of different agricultural practices on soil organic matter (SOM) quality and to validate the potential of the selected biochemical properties as optimal early indicators of soil quality in Mexican cultivated Acrisols. During 2002–2005 four agronomic management systems: conventional (Tc); improved conventional (Ti); organic (To) and fallow (Tf) were assayed in plots located at the MTB. An uncultivated soil under grass cover (Sg) was used as reference. Soil samples were collected at 0–10 cm depth and were analysed chemically (soil organic C, total N, water-soluble C and humic C), and biochemically (total and extra-cellular enzyme activity). After 4 years, soil organic C, total N, water-soluble C and dehydrogenase activity had higher values in To, followed by Ti treatment. A similar response pattern was observed in the extra-cellular enzyme activity. The highest total enzyme activity was found in Sg, followed by Ti and To treatments, and the lowest values appeared in Tc and Tf. To and Ti increased SOM contents of the degraded Acrisols studied, while Tc and Tf managements decreased the quality of these soils. The results showed that the assayed soil enzymes can be used as indicators of quality changes of these Mexican volcanic soils.The authors thank the European Union for supporting the REVOLSO Project (INCO-DEV Program) and the Spanish Ministry of Education & Science. Thanks are also due to C. I. Hidalgo (Colegio de Postgraduados de Montecillo, Mexico) for the mineralogical identification of soil clays analysis and J. Padilla (Colegio de Postgraduados de Montecillo, Mexico), C. Macci, S. Doni and laboratory technicians from the Istituto per lo Studio degli Ecosistemi (CNR, Italy) for theirs lab assistance.Peer reviewe

Clinical and genetic approach to the dysmorphic child

The child affected by a malformative syndrome represents a care challenge for the pediatrician. He is in fact the heart of the multidisciplinary team that has to manage the patient, trying to control the complications of his/her syndrome and promoting the correct psychophysical development. What we must not forget is that the pediatrician provides a continuous support to the child`s family, assisting them from the diagnosis to the management of problems related to the syndrome. This encourages the continuous follow-up of these children remembering also that the pediatrician is fundamental in the clinical management of the syndrome and for facilitating the social integration of these children

Serum neurofilament light chain: a promising early diagnostic biomarker for hereditary transthyretin amyloidosis?

Background and purpose: Hereditary transthyretin amyloidosis (ATTRv) is a life-threatening disease caused by mutations in the gene encoding transthyretin (TTR). The recent therapeutic advances have underlined the importance of easily accessible, objective biomarkers of both disease onset and progression. Preliminary evidence suggests a potential role in this respect for neurofilament light chain (NfL). In this study, the aim was to determine serum NfL (sNfL) levels in a late-onset ATTRv population and evaluate whether it might represent a reliable biomarker of disease onset (i.e., 'conversion' from the asymptomatic status to symptomatic disease in TTR mutation carriers). Methods: In all, 111 individuals harbouring a pathogenic TTR variant (61 symptomatic ATTRv patients and 50 presymptomatic carriers) were consecutively enrolled. Fifty healthy volunteers were included as the control group. Ella™ apparatus was used to assess sNfL levels. Results: Serum NfL levels were increased in ATTRv patients compared to both presymptomatic carriers and healthy controls, whilst not differing between carriers and healthy controls. An sNfL cut-off of 37.10 pg/mL could discriminate between asymptomatic and symptomatic individuals with high diagnostic accuracy (area under the curve 0.958; p < 0.001), sensitivity (81.4%) and specificity (100%). Conclusions: Serum NfL seems to be a promising biomarker of peripheral nerve involvement in ATTRv amyloidosis and might become a reliable, objective measure to detect the transition from the presymptomatic stage to the onset of symptomatic disease. Further longitudinal studies are needed to confirm such a role and determine whether it could equally represent a biomarker of disease progression and response to therapy

Withdrawal of mechanical ventilation in amyotrophic lateral sclerosis patients: a multicenter Italian survey

Background: Law 219/2017 was approved in Italy in December 2017, after a years-long debate on the autonomy of healthcare choices. This Law, for the first time in Italian legislation, guarantees the patient's right to request for withdrawal of life-sustaining treatments, including mechanical ventilation (MV). Objective: To investigate the current status of MV withdrawal in amyotrophic lateral sclerosis (ALS) patients in Italy and to assess the impact of Law 219/2017 on this practice. Methods: We conducted a Web-based survey, addressed to Italian neurologists with expertise in ALS care, and members of the Motor Neuron Disease Study Group of the Italian Society of Neurology. Results: Out of 40 ALS Italian centers, 34 (85.0%) responded to the survey. Law 219/2017 was followed by an increasing trend in MV withdrawals, and a significant increase of neurologists involved in this procedure (p 0.004). However, variations across Italian ALS centers were observed, regarding the inconsistent involvement of community health services and palliative care (PC) services, and the intervention and composition of the multidisciplinary team. Conclusions: Law 219/2017 has had a positive impact on the practice of MV withdrawal in ALS patients in Italy. The recent growing public attention on end-of-life care choices, along with the cultural and social changes in Italy, requires further regulatory frameworks that strengthen tools for self-determination, increased investment of resources in community and PC health services, and practical recommendations and guidelines for health workers involved

REGARDING ENVIRONMENTAL FACTORS IN THE ETIOLOGY OF ESOPHAGEAL ATRESIA AND CONGENITAL DIAPHRAGMATIC HERNIA

We read with great interest the article by Felix et al. (2008) on the environmental factors in the etiology of esophageal atresia and congenital diaphragmatic hernia. In their case-control study they found a possible association between maternal alcohol consumption in pregnancy and the development of a congenital diaphragmatic hernia. The teratogenic effect of prenatal exposure to ethanol is well described in the literature as the cause of fetal alcohol syndrome. Many nervous system dysfunctions and patterns of craniofacial features have been described as characteristic of this syndrome, but to date there is a lack of knowledge about the spectrum of systemic effects associated with alcohol intake during pregnancy. We suppose that these data may be under-reported because of a still poor knowledge of such conditions. The mechanisms of alcohol-induced impairment of embryonic and fetal development are complex and not yet fully understood. Ethanol can induce damage directly, through its metabolites, or by inducing deficiencies or abnormalities in retinoic acid (NIAAA, 2000; Molotkov et al., 2002). In fetal alcohol syndrome, the magnitude of expression of the characteristic phenotype increases with increasing amount of maternal drinking (Astley and Clarren, 2001). Felix et al. (2008) have highlighted that alcohol could have an important role also in the development of defects of pediatric surgery pertinence. It would be interesting to know if the neonates with congenital diaphragmatic hernia of their series showed the characteristic craniofacial phenotype of the fetal alcohol syndrome. Moreover, they reported the frequency of alcohol use among mothers of newborns with congenital diaphragmatic hernia as ‘‘times per entire period or per month or per week’’. We think that it could be interesting to have a better assessment of maternal alcohol consumption, evaluating type of alcoholic beverages, trimester(s) in which alcohol was consumed, number of drinks/day, and drinks consumed per occasion. This information could help to identify if there is a positive correlation between increasing alcohol intake and additional congenital malformations, such as congenital diaphragmatic hernia, or if that association is dose-independent. Indeed, it is important to know the type of alcoholic beverages used, because red wine antioxidants could mitigate the effects of ethanol on newborns (Assunc¸a˜o et al., 2007). Further studies are needed to better assess the systemic effects of exposure to alcohol during pregnancy

Fetal MRI: contribution to perinatal management and fetal surgical treatment of congenital anomalies

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