Pub Date : 2020-11-01DOI: 10.23736/S0026-4946.20.05844-2
F. Ruta, Barbara Bassola, M. Lusignani
BACKGROUND Spinal muscular atrophy (SMA) is one of the most common genetic causes of death in children. Recently, European Commission (EU) has approved a new gene therapy based on onasemnogene abeparvovec (Zolgensma) for the treatment of patients with SMA. It is essential that children suffering from SMA also apply self-care methods to maintain their health, monitor their weight and food intake, and use appropriate remedies. Indeed, self-care is a crucial element in the health care system because it is able to improve survival and prevent hospitalizations. The purpose of this review is to systematically explore the characteristics of self- care in children with spinal muscular atrophy and the relationship with their parents and the collaboration of healthcare professionals. METHODS An integrative review of the literature has been conducted. The electronic databases CINAHL, Embase, PubMed, and SCOPUS were searched. RESULTS Thirteen articles met the inclusion criteria and were reviewed using Whittemore and Knalf's integrative review methodology. The analysis of the 13 articles selected for the review show that previous literature has focused on six prevalent themes: problem solving and care behavior, decision making, optimizing living with an illness, high quality, childfocused homecare, healthcare professionals, and monitoring behaviors. CONCLUSIONS This paper highlights how self-management behaviors depend on four basic aspects: the person (individual, cognitive, and social perceptions), the patient's family (level of knowledge of the pathology, involvement in the management and quality of relationship with the patient), the community (relationships with external social contexts, such as school and other organizations), and the healthcare system (availability of resources and the degree of evolution of healthcare).
{"title":"The characteristics of self-care in children with spinal muscular atrophy: an integrative review.","authors":"F. Ruta, Barbara Bassola, M. Lusignani","doi":"10.23736/S0026-4946.20.05844-2","DOIUrl":"https://doi.org/10.23736/S0026-4946.20.05844-2","url":null,"abstract":"BACKGROUND\u0000Spinal muscular atrophy (SMA) is one of the most common genetic causes of death in children. Recently, European Commission (EU) has approved a new gene therapy based on onasemnogene abeparvovec (Zolgensma) for the treatment of patients with SMA. It is essential that children suffering from SMA also apply self-care methods to maintain their health, monitor their weight and food intake, and use appropriate remedies. Indeed, self-care is a crucial element in the health care system because it is able to improve survival and prevent hospitalizations. The purpose of this review is to systematically explore the characteristics of self- care in children with spinal muscular atrophy and the relationship with their parents and the collaboration of healthcare professionals.\u0000\u0000\u0000METHODS\u0000An integrative review of the literature has been conducted. The electronic databases CINAHL, Embase, PubMed, and SCOPUS were searched.\u0000\u0000\u0000RESULTS\u0000Thirteen articles met the inclusion criteria and were reviewed using Whittemore and Knalf's integrative review methodology. The analysis of the 13 articles selected for the review show that previous literature has focused on six prevalent themes: problem solving and care behavior, decision making, optimizing living with an illness, high quality, childfocused homecare, healthcare professionals, and monitoring behaviors.\u0000\u0000\u0000CONCLUSIONS\u0000This paper highlights how self-management behaviors depend on four basic aspects: the person (individual, cognitive, and social perceptions), the patient's family (level of knowledge of the pathology, involvement in the management and quality of relationship with the patient), the community (relationships with external social contexts, such as school and other organizations), and the healthcare system (availability of resources and the degree of evolution of healthcare).","PeriodicalId":18533,"journal":{"name":"Minerva pediatrica","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2020-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41993860","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2020-11-01DOI: 10.23736/S0026-4946.20.05906-X
G. Ciprandi, L. Colavita, C. Cuppari, M. Tosca
{"title":"HMGB1 modulation in children with allergic rhinitis.","authors":"G. Ciprandi, L. Colavita, C. Cuppari, M. Tosca","doi":"10.23736/S0026-4946.20.05906-X","DOIUrl":"https://doi.org/10.23736/S0026-4946.20.05906-X","url":null,"abstract":"","PeriodicalId":18533,"journal":{"name":"Minerva pediatrica","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2020-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41584957","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2020-10-27DOI: 10.23736/S0026-4946.20.05862-4
R. Ochani, Ameema Asad, S. Batra, Asim Shaikh
{"title":"Kissing an infant: a reflection of affection or a potential for death?","authors":"R. Ochani, Ameema Asad, S. Batra, Asim Shaikh","doi":"10.23736/S0026-4946.20.05862-4","DOIUrl":"https://doi.org/10.23736/S0026-4946.20.05862-4","url":null,"abstract":"","PeriodicalId":18533,"journal":{"name":"Minerva pediatrica","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2020-10-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"42128907","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2020-10-01DOI: 10.23736/S0026-4946.20.05988-5
Mia Šalamon Janečić, T. Grmoja, A. Tripalo Batoš, I. Hojsak
{"title":"Superior mesenteric artery syndrome complicating the clinical presentation and treatment of inflammatory bowel disease in a pediatric patient.","authors":"Mia Šalamon Janečić, T. Grmoja, A. Tripalo Batoš, I. Hojsak","doi":"10.23736/S0026-4946.20.05988-5","DOIUrl":"https://doi.org/10.23736/S0026-4946.20.05988-5","url":null,"abstract":"","PeriodicalId":18533,"journal":{"name":"Minerva pediatrica","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2020-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"42634084","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2020-10-01Epub Date: 2020-07-29DOI: 10.23736/S0026-4946.20.06015-6
Riccardo Castagnoli, Amelia Licari, Gian Luigi Marseglia
{"title":"Novel insights into pediatric allergy and immunology.","authors":"Riccardo Castagnoli, Amelia Licari, Gian Luigi Marseglia","doi":"10.23736/S0026-4946.20.06015-6","DOIUrl":"https://doi.org/10.23736/S0026-4946.20.06015-6","url":null,"abstract":"","PeriodicalId":18533,"journal":{"name":"Minerva pediatrica","volume":"72 5","pages":"341-342"},"PeriodicalIF":2.6,"publicationDate":"2020-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38209494","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2020-10-01DOI: 10.23736/S0026-4946.20.06073-9
A. Kan, T. Saylı
BACKGROUND Iron deficiency anemia causes a decrease in immune response to infections, physical working capacity and response to metabolic stress. It also causes behavioral, perceptual and cognitive disorders. Therefore, as soon as iron deficiency anemia is diagnosed, it should be treated immediately. In this study, it was investigated retrospectively whether there was a difference in treatment efficacy between the administration of oral ferrous or ferric iron and vitamin D at the same time and at different time. METHODS A total of sixty patients under 1 year who attended the pediatrics outpatient clinic for pale and diagnosed with iron deficiency anemia. Patients were randomly divided into 4 groups. Anemia was defined as hemoglobin below <2 SD according to age and gender. Iron deficiency was definied with serum iron, iron-binding capacity, ferritin and transferin saturation below the range for age and gender appropriates. All patients were seen at the outpatient clinic for 1st, 3rd month of the treatment. RESULTS There were a statistically significant differences between the groups in terms of increase in Hb values according to time (1st month, 3rd month, 1st and 3rd month). There was no statistically significant difference between the groups in terms of the recovery of anemia after treatment. CONCLUSIONS It was concluded that iron treatment in babies with iron deficiency anemia is not affected by the administration of vitamin D prophylaxis at the same time or at different times, and therefore both treatments can be administered at the same time to increase drug compliance.
{"title":"Effects of vitamin D prophylaxis on oral iron treatments of iron deficiency anemia.","authors":"A. Kan, T. Saylı","doi":"10.23736/S0026-4946.20.06073-9","DOIUrl":"https://doi.org/10.23736/S0026-4946.20.06073-9","url":null,"abstract":"BACKGROUND\u0000Iron deficiency anemia causes a decrease in immune response to infections, physical working capacity and response to metabolic stress. It also causes behavioral, perceptual and cognitive disorders. Therefore, as soon as iron deficiency anemia is diagnosed, it should be treated immediately. In this study, it was investigated retrospectively whether there was a difference in treatment efficacy between the administration of oral ferrous or ferric iron and vitamin D at the same time and at different time.\u0000\u0000\u0000METHODS\u0000A total of sixty patients under 1 year who attended the pediatrics outpatient clinic for pale and diagnosed with iron deficiency anemia. Patients were randomly divided into 4 groups. Anemia was defined as hemoglobin below <2 SD according to age and gender. Iron deficiency was definied with serum iron, iron-binding capacity, ferritin and transferin saturation below the range for age and gender appropriates. All patients were seen at the outpatient clinic for 1st, 3rd month of the treatment.\u0000\u0000\u0000RESULTS\u0000There were a statistically significant differences between the groups in terms of increase in Hb values according to time (1st month, 3rd month, 1st and 3rd month). There was no statistically significant difference between the groups in terms of the recovery of anemia after treatment.\u0000\u0000\u0000CONCLUSIONS\u0000It was concluded that iron treatment in babies with iron deficiency anemia is not affected by the administration of vitamin D prophylaxis at the same time or at different times, and therefore both treatments can be administered at the same time to increase drug compliance.","PeriodicalId":18533,"journal":{"name":"Minerva pediatrica","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2020-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"47725133","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2020-10-01Epub Date: 2020-07-20DOI: 10.23736/S0026-4946.20.05993-9
Riccardo Castagnoli, Maria De Filippo, Martina Votto, Alessia Marseglia, Lorenza Montagna, Gian Luigi Marseglia, Amelia Licari
Allergic diseases represent a global health burden. Patients with allergic diseases may experience disability, reduced quality of life and productivity, emotional distress, and social restrictions, especially in the most severe cases. Current advances in unveiling the pathogenesis of allergic disorders have paved the way for the development of novel therapeutic strategies. Biological drugs have been widely studied in pediatric allergic asthma, with strong evidence of efficacy and safety. Moreover, promising results derive from studies on other conditions such as atopic dermatitis, chronic spontaneous urticaria, and food allergy. This review analyzes recent evidence on the role of biologic therapies for allergic diseases, focusing on the pediatric age.
{"title":"An update on biological therapies for pediatric allergic diseases.","authors":"Riccardo Castagnoli, Maria De Filippo, Martina Votto, Alessia Marseglia, Lorenza Montagna, Gian Luigi Marseglia, Amelia Licari","doi":"10.23736/S0026-4946.20.05993-9","DOIUrl":"https://doi.org/10.23736/S0026-4946.20.05993-9","url":null,"abstract":"<p><p>Allergic diseases represent a global health burden. Patients with allergic diseases may experience disability, reduced quality of life and productivity, emotional distress, and social restrictions, especially in the most severe cases. Current advances in unveiling the pathogenesis of allergic disorders have paved the way for the development of novel therapeutic strategies. Biological drugs have been widely studied in pediatric allergic asthma, with strong evidence of efficacy and safety. Moreover, promising results derive from studies on other conditions such as atopic dermatitis, chronic spontaneous urticaria, and food allergy. This review analyzes recent evidence on the role of biologic therapies for allergic diseases, focusing on the pediatric age.</p>","PeriodicalId":18533,"journal":{"name":"Minerva pediatrica","volume":"72 5","pages":"364-371"},"PeriodicalIF":2.6,"publicationDate":"2020-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38178598","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2020-10-01DOI: 10.23736/S0026-4946.20.05740-0
P. Pokorná, D. Michaličková, S. Völler, K. Hronová, D. Tibboel, O. Slanař, E. Krekels
BACKGROUND The current study uses a population modeling approach to evaluate and quantify the impact of severity of asphyxia and hypoxic-ischemic encephalopathy (HIE) on the pharmacokinetics of phenobarbital in asphyxiated newborns treated with therapeutic hypothermia. METHODS Included newborns received phenobarbital (the TOBY trial protocol). 120 plasma sample were available from 50 newborns, median (IQR) weight 3.3 (2.8-3.5) kg and gestational age 39 (39-40) weeks. NONMEM version 7.2® was used for the data analysis. Age, body weight, sex, concomitant medications, kidney and liver function markers, as well as severity parameters of asphyxia and HIE were tested as potential covariates of pharmacokinetics of phenobarbital. Severe asphyxia was defined as pH of arterial umbilical cord blood ≤7.1 and Apgar 5 ≤5, and severe HIE was defined as time to normalization of amplitude-integrated electroencephalography (aEEG) >24 h. RESULTS Weight was found to be the only statistically significant covariate for the volume of distribution. At weight of 1 kg volume of distribution was 0.91 L and for every additional kg it increased in 0.91 L. Clearance was 0.00563 L/h. No covariates were statistically significant for the clearance of phenobarbital. CONCLUSIONS Phenobarbital dose adjustments are not indicated in the studied population, irrespective of the severity of asphyxia or HIE.
{"title":"Severity parameters for asphyxia or hypoxic-ischemic encephalopathy do not explain interindividual variability in the pharmacokinetics of phenobarbital in newborns treated with therapeutic hypothermia.","authors":"P. Pokorná, D. Michaličková, S. Völler, K. Hronová, D. Tibboel, O. Slanař, E. Krekels","doi":"10.23736/S0026-4946.20.05740-0","DOIUrl":"https://doi.org/10.23736/S0026-4946.20.05740-0","url":null,"abstract":"BACKGROUND\u0000The current study uses a population modeling approach to evaluate and quantify the impact of severity of asphyxia and hypoxic-ischemic encephalopathy (HIE) on the pharmacokinetics of phenobarbital in asphyxiated newborns treated with therapeutic hypothermia.\u0000\u0000\u0000METHODS\u0000Included newborns received phenobarbital (the TOBY trial protocol). 120 plasma sample were available from 50 newborns, median (IQR) weight 3.3 (2.8-3.5) kg and gestational age 39 (39-40) weeks. NONMEM version 7.2® was used for the data analysis. Age, body weight, sex, concomitant medications, kidney and liver function markers, as well as severity parameters of asphyxia and HIE were tested as potential covariates of pharmacokinetics of phenobarbital. Severe asphyxia was defined as pH of arterial umbilical cord blood ≤7.1 and Apgar 5 ≤5, and severe HIE was defined as time to normalization of amplitude-integrated electroencephalography (aEEG) >24 h.\u0000\u0000\u0000RESULTS\u0000Weight was found to be the only statistically significant covariate for the volume of distribution. At weight of 1 kg volume of distribution was 0.91 L and for every additional kg it increased in 0.91 L. Clearance was 0.00563 L/h. No covariates were statistically significant for the clearance of phenobarbital.\u0000\u0000\u0000CONCLUSIONS\u0000Phenobarbital dose adjustments are not indicated in the studied population, irrespective of the severity of asphyxia or HIE.","PeriodicalId":18533,"journal":{"name":"Minerva pediatrica","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2020-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"48286198","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2020-10-01DOI: 10.23736/S0026-4946.20.05901-0
N. Zampieri, M. Cinquetti, Virginia Murri, F. Camoglio
{"title":"Incidence of appendicitis during SARS-CoV-2 pandemic quarantine: report of a single area experience.","authors":"N. Zampieri, M. Cinquetti, Virginia Murri, F. Camoglio","doi":"10.23736/S0026-4946.20.05901-0","DOIUrl":"https://doi.org/10.23736/S0026-4946.20.05901-0","url":null,"abstract":"","PeriodicalId":18533,"journal":{"name":"Minerva pediatrica","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2020-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"42331156","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2020-10-01Epub Date: 2020-07-20DOI: 10.23736/S0026-4946.20.05976-9
Carla Mastrorilli, Angelica Santoro, Michela Procaccianti, Giuseppe Pagliaro, Carlo Caffarelli
Food protein-induced enterocolitis syndrome (FPIES) represents a non-IgE-mediated food allergic disorder with delayed gastrointestinal symptoms that may evolve in a medical emergency. Clinically, FPIES can be distinguished into acute and chronic phenotypes. FPIES is mainly diagnosed in infancy however the onset at older ages is being progressively described. The pathogenetic mechanism underlying FPIES remains mainly unexplained, but an alteration of food-specific T-cell response has been proposed. The diagnosis of FPIES is primarily clinical, since there are not available specific biomarkers. Oral food challenge (OFC) is the gold standard for diagnosing FPIES or excluding the onset of tolerance to the triggering food. Management of FPIES includes an acute phase treatment and a maintenance therapy with the strict food avoidance until challenge, in order to prevent new attacks and avoid nutritional alterations. Acute management requires hydration that can be performed orally or intravenously according to clinical status. Long-term management of FPIES is based on the avoidance of the culprit food(s) and supervised introduction of other high-risk foods if never taken before among infants before 12 months of age. There is a compelling need of future achievements in FPIES research for the definition of underlying disease pathogenesis and potential therapeutic point of care.
{"title":"New insights into food protein-induced enterocolitis in children.","authors":"Carla Mastrorilli, Angelica Santoro, Michela Procaccianti, Giuseppe Pagliaro, Carlo Caffarelli","doi":"10.23736/S0026-4946.20.05976-9","DOIUrl":"https://doi.org/10.23736/S0026-4946.20.05976-9","url":null,"abstract":"<p><p>Food protein-induced enterocolitis syndrome (FPIES) represents a non-IgE-mediated food allergic disorder with delayed gastrointestinal symptoms that may evolve in a medical emergency. Clinically, FPIES can be distinguished into acute and chronic phenotypes. FPIES is mainly diagnosed in infancy however the onset at older ages is being progressively described. The pathogenetic mechanism underlying FPIES remains mainly unexplained, but an alteration of food-specific T-cell response has been proposed. The diagnosis of FPIES is primarily clinical, since there are not available specific biomarkers. Oral food challenge (OFC) is the gold standard for diagnosing FPIES or excluding the onset of tolerance to the triggering food. Management of FPIES includes an acute phase treatment and a maintenance therapy with the strict food avoidance until challenge, in order to prevent new attacks and avoid nutritional alterations. Acute management requires hydration that can be performed orally or intravenously according to clinical status. Long-term management of FPIES is based on the avoidance of the culprit food(s) and supervised introduction of other high-risk foods if never taken before among infants before 12 months of age. There is a compelling need of future achievements in FPIES research for the definition of underlying disease pathogenesis and potential therapeutic point of care.</p>","PeriodicalId":18533,"journal":{"name":"Minerva pediatrica","volume":"72 5","pages":"416-423"},"PeriodicalIF":2.6,"publicationDate":"2020-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38178596","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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