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Papers by salvatore de masi

Additional file 1. List of recommendations.

Inflammatory Bowel Diseases, 2021

Background The aim of the present study was to investigate outcomes of anti-TNF-alpha (ATA) withd... more Background The aim of the present study was to investigate outcomes of anti-TNF-alpha (ATA) withdrawal in selected pediatric patients with inflammatory bowel disease who achieved clinical remission and mucosal and histological healing (MH and HH). Methods A retrospective analysis was performed on children and adolescents affected by Crohn disease (CD) and ulcerative colitis (UC) who were followed up at 2 tertiary referral centers from 2008 through 2018. The main outcome measure was clinical relapse rates after ATA withdrawal. Results One hundred seventy patients received scheduled ATA treatment; 78 patients with CD and 56 patients with UC underwent endoscopic reassessment. We found that MH was achieved by 32 patients with CD (41%) and 30 patients with UC (53.6%); 26 patients with CD (33.3%) and 22 patients with UC (39.3%) achieved HH. The ATA treatment was suspended in 45 patients, 24 affected by CD and 21 by UC, who all achieved concurrently complete MH (Simplified Endoscopic Score...

Life Sciences, 2021

Background: The emergence of SARS-CoV-2 pandemic has upset health systems around the world and ca... more Background: The emergence of SARS-CoV-2 pandemic has upset health systems around the world and caused immeasurable losses and costs. Until a vaccine will become available, the recommended prevention measures remain physical distancing and enhanced hygiene. Methods and findings: The proteic structure external to the virus is the main target that may eventually lead to reduce or block its replication in the upper airways. We developed a protocol based of repeated steam inhalation cycles aimed at reducing the risk of progression to full blown infection if performed soon after contagion. The protocol has been used in a single-center open label trial on ten infected asymptomatic or pauci-symptomatic health care professionals. Conclusions: The promising results we obtained with this easily accessible, non-invasive and inexpensive procedure should prompt controlled trials.

s were used based on specific criteria. The papers have been selected and classified on the basis... more s were used based on specific criteria. The papers have been selected and classified on the basis of highest level of evidence, design of the study, and most recent publication. Cross-link control was performed with Google Scholar and Cochrane library databases. According to the Health Technology Assessment Programme Manual (2001) 3 clinical recommendations are defined as

The lack of signal elements between Healthcare Workers and users can generate errors caused by in... more The lack of signal elements between Healthcare Workers and users can generate errors caused by interruptions during drug administration depending on human, technical or environmental factors that can become a source of stress and adverse events.

Updating diagnostic test accuracy (DTA) systematic reviews (SRs) is fundamental in order to avoid... more Updating diagnostic test accuracy (DTA) systematic reviews (SRs) is fundamental in order to avoid making clinical decisions based on out-of-date and/or incomplete information. The decision of which SR to update should be based on the quality of the SR and on the currency of its topic. If SRs are considered worthy of updating, priority should first be established depending on the availability of elements of novelty (in terms of published studies, methodology, decisional approach, or standards of quality), as well as on the expected impact and value of information.

American Journal of Reproductive Immunology, 2021

Autoimmune disorders currently affect 5%‐8% of the global population, characterized by an aberran... more Autoimmune disorders currently affect 5%‐8% of the global population, characterized by an aberrant chronic inflammatory response to self‐antigens. The aim of this study was to systematically review the current available evidence investigating the impact of systemic autoimmune diseases and associated immunosuppressive treatment on fertility parameters of adult men. Clinical trials, observational studies, and case reports written in English and reporting semen analysis, evaluation of seminal oxidative stress, and/or sperm DNA fragmentation in patients affected by psoriasis and psoriatic arthritis, celiac disease, inflammatory bowel diseases, systemic lupus erythematosus, ankylosing spondylitis, hidradenitis suppurativa, uveitis, dermatomyositis, and rheumatoid arthritis were collected by searching on PubMed, EMBASE, OVID, Scopus, and Cochrane Library databases, with no limit of time. The study quality and the extent of bias in design, methods, and outcome assessment were evaluated by applying the Joanna Briggs Institute Critical Appraisal tools. Evidence suggested that various autoimmune diseases or relevant medications can adversely affect male fertility parameters and that patients may benefit of counseling and sperm cryopreservation. Clinical trials further investigating any adverse effect of autoimmunity and related thereby on male infertility are warranted, to develop appropriate guidelines for males diagnosed and treated for autoimmune disorders.

Epilepsia, 2019

Objective: To assess the long-term efficacy and tolerability of stiripentol (STP) as an adjunctiv... more Objective: To assess the long-term efficacy and tolerability of stiripentol (STP) as an adjunctive treatment in different forms of refractory epilepsies. Methods: The medical records of all individuals consecutively treated with STP as add-on therapy for refractory epilepsies, irrespective of their being focal, generalized, or both, and followed at Meyer Children's Hospital between January 2007 and May 2018, were reviewed. The drug scheme administration consisted of a starting dose of STP of 10-15 mg/kg/d with increments every week, up to a maximum of 50 mg/kg/d, based on both age and weight. Etiology of epilepsy was codified as structural, genetic, infectious, immune, metabolic, and unknown. Responders were defined as patients who achieved a seizure frequency reduction of ≥50%. Retention rate was defined as the probability of continuing STP without additional therapy. Tolerability was assessed by reporting adverse events. Results: A total of 132 individuals aged from 5 months to 43 years received add-on STP, including 30 patients with Dravet syndrome (DS). The median follow-up was 14.8 months (range = 4 months-18 years, interquartile range = 25.72). Twenty-nine individuals (22%) received more than two antiepileptic drugs. Benzodiazepines, mainly clobazam, were the most commonly used add-on drugs. Sixty-six patients (50%) were responders, and 13 of them (9.8%) were seizure-free. Responder rate was higher in the genetic etiology group (57%), especially in DS (18/30; 60%), and in patients with refractory focal onset epilepsy without bilateral tonic-clonic seizures (5/15; 33%). The median relapse-free survival was 27 months in the 66 responders. The median time to STP failure was 24.6 months in all 132 individuals. Significance: This study confirms the long-term efficacy of add-on STP treatment in patients with different types of refractory epilepsies, including focal onset epilepsy without bilateral tonic-clonic seizures. Further confirmations based on prospectively designed studies are required to confirm STP efficacy in focal epilepsy.

Extracorporemembrane oxygenation (ECMO) allows the potential rescue of patients unresponsive to t... more Extracorporemembrane oxygenation (ECMO) allows the potential rescue of patients unresponsive to traditional cardiopulmonary resuscitation (CPR). Survival by using this modality has been demonstrated. We describe a case of a 12 year old girl admitted to our Emergency Department because of cardiogenic shock occurred after consciousness loss exercise related. The patient has been transported by the territorial rescue team with orotracheal intubation and manually ventilated with oxygen supplements. During transport, third degree heart block with 40 bpm ventricular rate was documented. On arrival she was hypotensive, with delayed capillary refill, and weak peripheral pulses. The chest evaluation revealed pulmonary oedema. On ECG atrial fibrillation with bifascicular block and mean ventricular rate of 125 bpm was documented,. Emogasanalysis demontrated severe metabolic acidosis. The initial echocardiogram showed an apparent structurally normal heart with moderate left ventricular dysfunct...

Expert Review of Anticancer Therapy, 2006

Brain tumors are the second most common cancer in pediatric patients and the main cause from deat... more Brain tumors are the second most common cancer in pediatric patients and the main cause from death of malignant tumors in this age group. High-grade or malignant glioma, among which anaplastic astrocytomas and glioblastoma are the most prevalent histotypes, represent 10% of pediatric brain tumors and, taken as a whole, are the second most frequent malignant histotype after medulloblastoma. Apart from complete excision followed by full-dose local radiotherapy, chemotherapy appears to provide some benefit to the final outcome. Different trials have explored the role of high-dose chemotherapy that, theoretically, could give an advantage to these patients by overcoming the blood-brain barrier, cell chemoresistance and inducing a wider number of responses. However, it is still doubtful if more responses translate into better outcome and it is not fully understood which patients can experience a true benefit from this treatment strategy. New protocols under evaluation include new agents with specific biological targets, multiple cycles of high-dose chemotherapy, and vaccination, as an immunotherapeutic approach.

European Journal of Gastroenterology & Hepatology, 2011

a ; on behalf of the Multidisciplinary Guidelines Development Group Objectives To develop guideli... more a ; on behalf of the Multidisciplinary Guidelines Development Group Objectives To develop guidelines for the noninvasive imaging assessment of focal liver lesions comparing different imaging modalities focused on (i) evaluating the imaging techniques in terms of (a) diagnostic accuracy; (b) role in the management of oncologic patients; (c) follow-up of benign lesions; and (ii) developing standard procedure for their use in patients with focal liver lesions that require targeted diagnostic characterization. Methods An explicit search strategy was used to conduct a systematic review of the literature in the English language from January 2000 to October 2007; the search covered PubMed, Embase, Pascal, SciSearch, and Cochrane Library databases. A panel of experts evaluated the selected studies and conveyed their view. Results The online search yielded 4960 titles and abstracts from which 176 studies were considered suitable for the final adherence-to-guidelines topic evaluation. An evidence grading system was not used as the guideline topic and the heterogeneity of the collected data did not fit with the currently used hierarchy of evidence. A panel of experts formulated several recommendations with grade and level which were expressed narratively and nonschematically. Conclusion The recommendations reported in the study are based on an extensive literature evaluation and were developed by considering the appropriateness of the choice of the imaging techniques while noninvasively detecting and characterizing focal liver lesions. Eur J Gastroenterol Hepatol 23:343-353 c 2011 Wolters Kluwer Health | Lippincott Williams & Wilkins.

Minerva Anestesiologica

INTRODUCTION The aim of the work is to update the "Guidelines for the Management of Severe T... more INTRODUCTION The aim of the work is to update the "Guidelines for the Management of Severe Traumatic Brain Injury" published in 2012, to reflect the new available evidence, and develop the Italian national guideline for the management of severe pediatric head injuries to reduce variation in practice and ensure optimal care to patients. EVIDENCE ACQUISITION MEDLINE and EMBASE were searched from January 2009 to October 2017. Inclusion criteria were: English language, pediatric populations (0-18 years) or mixed populations (pediatric-adult) with available age subgroup analyses. The guideline development process was started by the Promoting Group that composed a multidisciplinary panel of experts, with the representatives of the Scientific Societies, the independent expert specialists and a representative of the Patient Associations. The panel selected the clinical questions, discussed the evidences and formulated the text of the recommendations. The documentarists of the University of Florence oversaw the bibliographic research strategy. A group of literature reviewers evaluated the selected literature and compiled the table of evidence for each clinical question. EVIDENCE SYNTHESIS The search strategies identified 4254 articles. We selected 3227 abstract (first screening) and, finally included 67 articles (second screening) to update the guideline. This Italian update includes 25 evidence-based recommendations and 5 research recommendations. CONCLUSIONS In recent years, progress has been made on the understanding of severe pediatric brain injury, as well as on that concerning all major traumatic pathology. This has led to a progressive improvement in the clinical outcome, although the quantity and quality of evidence remains particularly low.

Journal of Pediatric Gastroenterology & Nutrition

Italian Journal of Pediatrics

Background The timing of puberty in girls is occurring at an increasingly early age. While a posi... more Background The timing of puberty in girls is occurring at an increasingly early age. While a positive family history is recognised as a predisposing factor for early or precocious puberty, the role of environmental factors is not fully understood. Aims of the study To make a retrospective evaluation of the incidence of newly diagnosed central precocious puberty (CPP) and the rate of pubertal progression in previously diagnosed patients during and after the Italian lockdown for COVID-19, comparing data with corresponding data from the previous 5 years. To determine whether body mass index (BMI) and the use of electronic devices increased during lockdown in these patients. Patients and methods The study included 49 females with CPP. We divided the patients into two groups: group 1, patients presenting a newly diagnosed CPP and group 2, patients with previously diagnosed slow progression CPP whose pubertal progression accelerated during or after lockdown. We collected auxological, clin...

Cerebral Cortex

Protocadherin-19 (PCDH19) is a calcium dependent cell-adhesion molecule involved in neuronal circ... more Protocadherin-19 (PCDH19) is a calcium dependent cell-adhesion molecule involved in neuronal circuit formation with prevalent expression in the limbic structures. PCDH19-gene mutations cause a developmental encephalopathy with prominent infantile onset focal seizures, variably associated with intellectual disability and autistic features. Diagnostic neuroimaging is usually unrevealing. We used quantitative MRI to investigate the cortex and white matter in a group of 20 PCDH19-mutated patients. By a statistical comparison between quantitative features in PCDH19 brains and in a group of age and sex matched controls, we found that patients exhibited bilateral reductions of local gyrification index (lGI) in limbic cortical areas, including the parahippocampal and entorhinal cortex and the fusiform and lingual gyri, and altered diffusivity features in the underlying white matter. In patients with an earlier onset of seizures, worse psychiatric manifestations and cognitive impairment, red...

Italian Journal of Pediatrics

Background: In Italy, as in many European countries, Pediatric Emergency Medicine is not formally... more Background: In Italy, as in many European countries, Pediatric Emergency Medicine is not formally recognized as a pediatric subspecialty, hindering nationwide adoption of standards of care, especially in the field of procedural sedation and analgesia (PSA) in the Emergency Department (ED). For this reason PSA in Italy is mostly neglected or performed very heterogeneously and by different providers, with no reference standard. We aimed to describe the procedures and results of the first multidisciplinary and multi-professional Consensus Conference in Italy on safe and effective pediatric PSA in Italian EDs. Methods: The preparation, organization and conduct of the Consensus Conference, held in Florence in 2017, followed the recommended National methodological standards. Professionals from different specialties across the country were invited to participate. Results: Overall 86 recommendations covering 8 themes (pre-sedation evaluation, pharmacologic agents, monitoring, equipment and discharge checklists, training, non-pharmacologic techniques, the adult ED setting, impact on hospitalizations) were developed, taking into account the Italian training system and healthcare organization characteristics. Conclusion: The results of the first multidisciplinary and multi-professional Consensus Conference in Italy are meant to provide up-to-date national guidance to improve the standard of care of children undergoing painful and stressful procedures in the ED. The recommendations will be periodically updated as new relevant evidence is published.

Additional file 1. List of recommendations.

Inflammatory Bowel Diseases, 2021

Background The aim of the present study was to investigate outcomes of anti-TNF-alpha (ATA) withd... more Background The aim of the present study was to investigate outcomes of anti-TNF-alpha (ATA) withdrawal in selected pediatric patients with inflammatory bowel disease who achieved clinical remission and mucosal and histological healing (MH and HH). Methods A retrospective analysis was performed on children and adolescents affected by Crohn disease (CD) and ulcerative colitis (UC) who were followed up at 2 tertiary referral centers from 2008 through 2018. The main outcome measure was clinical relapse rates after ATA withdrawal. Results One hundred seventy patients received scheduled ATA treatment; 78 patients with CD and 56 patients with UC underwent endoscopic reassessment. We found that MH was achieved by 32 patients with CD (41%) and 30 patients with UC (53.6%); 26 patients with CD (33.3%) and 22 patients with UC (39.3%) achieved HH. The ATA treatment was suspended in 45 patients, 24 affected by CD and 21 by UC, who all achieved concurrently complete MH (Simplified Endoscopic Score...

Life Sciences, 2021

Background: The emergence of SARS-CoV-2 pandemic has upset health systems around the world and ca... more Background: The emergence of SARS-CoV-2 pandemic has upset health systems around the world and caused immeasurable losses and costs. Until a vaccine will become available, the recommended prevention measures remain physical distancing and enhanced hygiene. Methods and findings: The proteic structure external to the virus is the main target that may eventually lead to reduce or block its replication in the upper airways. We developed a protocol based of repeated steam inhalation cycles aimed at reducing the risk of progression to full blown infection if performed soon after contagion. The protocol has been used in a single-center open label trial on ten infected asymptomatic or pauci-symptomatic health care professionals. Conclusions: The promising results we obtained with this easily accessible, non-invasive and inexpensive procedure should prompt controlled trials.

s were used based on specific criteria. The papers have been selected and classified on the basis... more s were used based on specific criteria. The papers have been selected and classified on the basis of highest level of evidence, design of the study, and most recent publication. Cross-link control was performed with Google Scholar and Cochrane library databases. According to the Health Technology Assessment Programme Manual (2001) 3 clinical recommendations are defined as

The lack of signal elements between Healthcare Workers and users can generate errors caused by in... more The lack of signal elements between Healthcare Workers and users can generate errors caused by interruptions during drug administration depending on human, technical or environmental factors that can become a source of stress and adverse events.

Updating diagnostic test accuracy (DTA) systematic reviews (SRs) is fundamental in order to avoid... more Updating diagnostic test accuracy (DTA) systematic reviews (SRs) is fundamental in order to avoid making clinical decisions based on out-of-date and/or incomplete information. The decision of which SR to update should be based on the quality of the SR and on the currency of its topic. If SRs are considered worthy of updating, priority should first be established depending on the availability of elements of novelty (in terms of published studies, methodology, decisional approach, or standards of quality), as well as on the expected impact and value of information.

American Journal of Reproductive Immunology, 2021

Autoimmune disorders currently affect 5%‐8% of the global population, characterized by an aberran... more Autoimmune disorders currently affect 5%‐8% of the global population, characterized by an aberrant chronic inflammatory response to self‐antigens. The aim of this study was to systematically review the current available evidence investigating the impact of systemic autoimmune diseases and associated immunosuppressive treatment on fertility parameters of adult men. Clinical trials, observational studies, and case reports written in English and reporting semen analysis, evaluation of seminal oxidative stress, and/or sperm DNA fragmentation in patients affected by psoriasis and psoriatic arthritis, celiac disease, inflammatory bowel diseases, systemic lupus erythematosus, ankylosing spondylitis, hidradenitis suppurativa, uveitis, dermatomyositis, and rheumatoid arthritis were collected by searching on PubMed, EMBASE, OVID, Scopus, and Cochrane Library databases, with no limit of time. The study quality and the extent of bias in design, methods, and outcome assessment were evaluated by applying the Joanna Briggs Institute Critical Appraisal tools. Evidence suggested that various autoimmune diseases or relevant medications can adversely affect male fertility parameters and that patients may benefit of counseling and sperm cryopreservation. Clinical trials further investigating any adverse effect of autoimmunity and related thereby on male infertility are warranted, to develop appropriate guidelines for males diagnosed and treated for autoimmune disorders.

Epilepsia, 2019

Objective: To assess the long-term efficacy and tolerability of stiripentol (STP) as an adjunctiv... more Objective: To assess the long-term efficacy and tolerability of stiripentol (STP) as an adjunctive treatment in different forms of refractory epilepsies. Methods: The medical records of all individuals consecutively treated with STP as add-on therapy for refractory epilepsies, irrespective of their being focal, generalized, or both, and followed at Meyer Children's Hospital between January 2007 and May 2018, were reviewed. The drug scheme administration consisted of a starting dose of STP of 10-15 mg/kg/d with increments every week, up to a maximum of 50 mg/kg/d, based on both age and weight. Etiology of epilepsy was codified as structural, genetic, infectious, immune, metabolic, and unknown. Responders were defined as patients who achieved a seizure frequency reduction of ≥50%. Retention rate was defined as the probability of continuing STP without additional therapy. Tolerability was assessed by reporting adverse events. Results: A total of 132 individuals aged from 5 months to 43 years received add-on STP, including 30 patients with Dravet syndrome (DS). The median follow-up was 14.8 months (range = 4 months-18 years, interquartile range = 25.72). Twenty-nine individuals (22%) received more than two antiepileptic drugs. Benzodiazepines, mainly clobazam, were the most commonly used add-on drugs. Sixty-six patients (50%) were responders, and 13 of them (9.8%) were seizure-free. Responder rate was higher in the genetic etiology group (57%), especially in DS (18/30; 60%), and in patients with refractory focal onset epilepsy without bilateral tonic-clonic seizures (5/15; 33%). The median relapse-free survival was 27 months in the 66 responders. The median time to STP failure was 24.6 months in all 132 individuals. Significance: This study confirms the long-term efficacy of add-on STP treatment in patients with different types of refractory epilepsies, including focal onset epilepsy without bilateral tonic-clonic seizures. Further confirmations based on prospectively designed studies are required to confirm STP efficacy in focal epilepsy.

Extracorporemembrane oxygenation (ECMO) allows the potential rescue of patients unresponsive to t... more Extracorporemembrane oxygenation (ECMO) allows the potential rescue of patients unresponsive to traditional cardiopulmonary resuscitation (CPR). Survival by using this modality has been demonstrated. We describe a case of a 12 year old girl admitted to our Emergency Department because of cardiogenic shock occurred after consciousness loss exercise related. The patient has been transported by the territorial rescue team with orotracheal intubation and manually ventilated with oxygen supplements. During transport, third degree heart block with 40 bpm ventricular rate was documented. On arrival she was hypotensive, with delayed capillary refill, and weak peripheral pulses. The chest evaluation revealed pulmonary oedema. On ECG atrial fibrillation with bifascicular block and mean ventricular rate of 125 bpm was documented,. Emogasanalysis demontrated severe metabolic acidosis. The initial echocardiogram showed an apparent structurally normal heart with moderate left ventricular dysfunct...

Expert Review of Anticancer Therapy, 2006

Brain tumors are the second most common cancer in pediatric patients and the main cause from deat... more Brain tumors are the second most common cancer in pediatric patients and the main cause from death of malignant tumors in this age group. High-grade or malignant glioma, among which anaplastic astrocytomas and glioblastoma are the most prevalent histotypes, represent 10% of pediatric brain tumors and, taken as a whole, are the second most frequent malignant histotype after medulloblastoma. Apart from complete excision followed by full-dose local radiotherapy, chemotherapy appears to provide some benefit to the final outcome. Different trials have explored the role of high-dose chemotherapy that, theoretically, could give an advantage to these patients by overcoming the blood-brain barrier, cell chemoresistance and inducing a wider number of responses. However, it is still doubtful if more responses translate into better outcome and it is not fully understood which patients can experience a true benefit from this treatment strategy. New protocols under evaluation include new agents with specific biological targets, multiple cycles of high-dose chemotherapy, and vaccination, as an immunotherapeutic approach.

European Journal of Gastroenterology & Hepatology, 2011

a ; on behalf of the Multidisciplinary Guidelines Development Group Objectives To develop guideli... more a ; on behalf of the Multidisciplinary Guidelines Development Group Objectives To develop guidelines for the noninvasive imaging assessment of focal liver lesions comparing different imaging modalities focused on (i) evaluating the imaging techniques in terms of (a) diagnostic accuracy; (b) role in the management of oncologic patients; (c) follow-up of benign lesions; and (ii) developing standard procedure for their use in patients with focal liver lesions that require targeted diagnostic characterization. Methods An explicit search strategy was used to conduct a systematic review of the literature in the English language from January 2000 to October 2007; the search covered PubMed, Embase, Pascal, SciSearch, and Cochrane Library databases. A panel of experts evaluated the selected studies and conveyed their view. Results The online search yielded 4960 titles and abstracts from which 176 studies were considered suitable for the final adherence-to-guidelines topic evaluation. An evidence grading system was not used as the guideline topic and the heterogeneity of the collected data did not fit with the currently used hierarchy of evidence. A panel of experts formulated several recommendations with grade and level which were expressed narratively and nonschematically. Conclusion The recommendations reported in the study are based on an extensive literature evaluation and were developed by considering the appropriateness of the choice of the imaging techniques while noninvasively detecting and characterizing focal liver lesions. Eur J Gastroenterol Hepatol 23:343-353 c 2011 Wolters Kluwer Health | Lippincott Williams & Wilkins.

Minerva Anestesiologica

INTRODUCTION The aim of the work is to update the "Guidelines for the Management of Severe T... more INTRODUCTION The aim of the work is to update the "Guidelines for the Management of Severe Traumatic Brain Injury" published in 2012, to reflect the new available evidence, and develop the Italian national guideline for the management of severe pediatric head injuries to reduce variation in practice and ensure optimal care to patients. EVIDENCE ACQUISITION MEDLINE and EMBASE were searched from January 2009 to October 2017. Inclusion criteria were: English language, pediatric populations (0-18 years) or mixed populations (pediatric-adult) with available age subgroup analyses. The guideline development process was started by the Promoting Group that composed a multidisciplinary panel of experts, with the representatives of the Scientific Societies, the independent expert specialists and a representative of the Patient Associations. The panel selected the clinical questions, discussed the evidences and formulated the text of the recommendations. The documentarists of the University of Florence oversaw the bibliographic research strategy. A group of literature reviewers evaluated the selected literature and compiled the table of evidence for each clinical question. EVIDENCE SYNTHESIS The search strategies identified 4254 articles. We selected 3227 abstract (first screening) and, finally included 67 articles (second screening) to update the guideline. This Italian update includes 25 evidence-based recommendations and 5 research recommendations. CONCLUSIONS In recent years, progress has been made on the understanding of severe pediatric brain injury, as well as on that concerning all major traumatic pathology. This has led to a progressive improvement in the clinical outcome, although the quantity and quality of evidence remains particularly low.

Journal of Pediatric Gastroenterology & Nutrition

Italian Journal of Pediatrics

Background The timing of puberty in girls is occurring at an increasingly early age. While a posi... more Background The timing of puberty in girls is occurring at an increasingly early age. While a positive family history is recognised as a predisposing factor for early or precocious puberty, the role of environmental factors is not fully understood. Aims of the study To make a retrospective evaluation of the incidence of newly diagnosed central precocious puberty (CPP) and the rate of pubertal progression in previously diagnosed patients during and after the Italian lockdown for COVID-19, comparing data with corresponding data from the previous 5 years. To determine whether body mass index (BMI) and the use of electronic devices increased during lockdown in these patients. Patients and methods The study included 49 females with CPP. We divided the patients into two groups: group 1, patients presenting a newly diagnosed CPP and group 2, patients with previously diagnosed slow progression CPP whose pubertal progression accelerated during or after lockdown. We collected auxological, clin...

Cerebral Cortex

Protocadherin-19 (PCDH19) is a calcium dependent cell-adhesion molecule involved in neuronal circ... more Protocadherin-19 (PCDH19) is a calcium dependent cell-adhesion molecule involved in neuronal circuit formation with prevalent expression in the limbic structures. PCDH19-gene mutations cause a developmental encephalopathy with prominent infantile onset focal seizures, variably associated with intellectual disability and autistic features. Diagnostic neuroimaging is usually unrevealing. We used quantitative MRI to investigate the cortex and white matter in a group of 20 PCDH19-mutated patients. By a statistical comparison between quantitative features in PCDH19 brains and in a group of age and sex matched controls, we found that patients exhibited bilateral reductions of local gyrification index (lGI) in limbic cortical areas, including the parahippocampal and entorhinal cortex and the fusiform and lingual gyri, and altered diffusivity features in the underlying white matter. In patients with an earlier onset of seizures, worse psychiatric manifestations and cognitive impairment, red...

Italian Journal of Pediatrics

Background: In Italy, as in many European countries, Pediatric Emergency Medicine is not formally... more Background: In Italy, as in many European countries, Pediatric Emergency Medicine is not formally recognized as a pediatric subspecialty, hindering nationwide adoption of standards of care, especially in the field of procedural sedation and analgesia (PSA) in the Emergency Department (ED). For this reason PSA in Italy is mostly neglected or performed very heterogeneously and by different providers, with no reference standard. We aimed to describe the procedures and results of the first multidisciplinary and multi-professional Consensus Conference in Italy on safe and effective pediatric PSA in Italian EDs. Methods: The preparation, organization and conduct of the Consensus Conference, held in Florence in 2017, followed the recommended National methodological standards. Professionals from different specialties across the country were invited to participate. Results: Overall 86 recommendations covering 8 themes (pre-sedation evaluation, pharmacologic agents, monitoring, equipment and discharge checklists, training, non-pharmacologic techniques, the adult ED setting, impact on hospitalizations) were developed, taking into account the Italian training system and healthcare organization characteristics. Conclusion: The results of the first multidisciplinary and multi-professional Consensus Conference in Italy are meant to provide up-to-date national guidance to improve the standard of care of children undergoing painful and stressful procedures in the ED. The recommendations will be periodically updated as new relevant evidence is published.