Objective E-learning is a candidate tool for clinical practice guidelines (CPG) implementation du... more Objective E-learning is a candidate tool for clinical practice guidelines (CPG) implementation due to its versatility, universal access and low costs. We aimed to assess the impact of a five-mod-ule e-learning course about CPG for acute gastroenteritis (AGE) on physicians ’ knowledge and clinical practice. Study design This work was conceived as a pre/post single-arm intervention study. Physicians from 11 European countries registered for the online course. Personal data, pre- and post-course questionnaires and clinical data about 3 to 5 children with AGEmanaged by each physician before and after the course were collected. Primary outcomemeasures included the propor-tion of participants fully adherent to CPG and number of patients managed with full adherence.
Journal of Pediatric Gastroenterology & Nutrition, 2020
Objectives: The aim of the study was to assess changes in clinical phenotype, and identify determ... more Objectives: The aim of the study was to assess changes in clinical phenotype, and identify determinants of outcome in children with chronic hepatitis B virus (HBV) infection born in HBV-endemic countries followed in 2 Italian tertiary care centers after immigration or adoption. Methods: A prospective observational study on hepatitis B e-antibodies–negative chronic hepatitis B children started on 2002. Patients with liver fibrosis, or those needing antiviral treatment were excluded. Immune active patients were defined those with raised transaminases (alanine aminotransferase > 40 IU/L), immune tolerants those having normal alanine aminotransferase, both exhibiting substantial viral replication (HBV DNA >2000 IU/mL). Results: Sixty-nine patients (44 boys, median age 4.7 years) had a median follow-up of 53 months. At entry, 18 (26%) children were immune tolerant, 47 (68%) immune active, and 4 had indeterminant immune status. At last follow-up, 14 (78%) of the immune-tolerant pati...
Pediatric Hepatology and Liver Transplantation, 2019
The knowledge of the underlying aetiology of neonatal idiopathic hepatitis and the so-called “int... more The knowledge of the underlying aetiology of neonatal idiopathic hepatitis and the so-called “intrahepatic” cholestasis has been rapidly expanding in the last decade, and great advances in genetic testing have clarified that the vast majority of these conditions are monogenic liver disorders. Among those referred to as progressive familial intrahepatic cholestases (PFICs), the level of serum GGT is a good discriminant to guide the initial evaluation, being low/normal in Byler disease, BSEP deficiency, TJP2 deficiency, FXR deficiency and MYO5B deficiency, and increased only in MDR3 deficiency; however genetic testing is needed to reach a definite categorisation. In bile acid synthesis defects, normal serum bile acid is a clue to the diagnosis, although mass spectrometry is required to characterise the type of defect. Other well-known conditions such as Alagille syndrome and alpha-1 antitrypsin deficiency are more common and less challenging to recognise. In this chapter we discuss th...
Neonatal and infantile cholestasis (NIC) can represent the onset of a surgically correctable dise... more Neonatal and infantile cholestasis (NIC) can represent the onset of a surgically correctable disease and of a genetic or metabolic disorder worthy of medical treatment. Timely recognition of NIC and identification of the underlying etiology are paramount to improve outcomes. Upon invitation by the Italian National Institute of Health (ISS), an expert working grouped was formed to formulate evidence-based positions on current knowledge about the diagnosis of NIC. A systematic literature search was conducted to collect evidence about epidemiology, etiology, clinical aspects and accuracy of available diagnostic tests in NIC. Evidence was scored using the GRADE system. All recommendations were approved by a panel of experts upon agreement of at least 75% of the members. The final document was approved by all the panel components. This position document summarizes the collected statements and defines the best-evidence diagnostic approach to cholestasis in the first year of life.
Advances in experimental medicine and biology, Jan 28, 2016
Tuberculosis is a major problem in children depending on their families for management and a re-e... more Tuberculosis is a major problem in children depending on their families for management and a re-emerging disease in low incidence countries, where foreign-born cases account for a large proportion of cases. We investigated socioeconomic features of families and their impact on management and outcome of children with tuberculosis disease seen at a tertiary care centre for paediatric infectious diseases in Italy. Forty-nine Italian and 30 foreign-origin children were included. Children from foreign families had more complicated diseases (20 % vs 0 %; P = 0.002), harbored more drug resistant strains (20 % vs 2 %; P = 0.011), showed longer hospital stay (12 ± 13.1 vs 5.1 ± 6.5 days; P = 0.012) and higher proportion of missed medical visits (15.7 ± 16 vs 8.6 ± 9.6; P ≤ 0.042) than those from Italian families. Harboring drug resistant strains was an independent risk factor for complicated disease course (OR: 72.98; 95 %CI: 1.54-3468.58; P = 0.029), and this risk is higher in children from...
Culture-independent strategies such as high-throughput parallel sequencing and comparative genomi... more Culture-independent strategies such as high-throughput parallel sequencing and comparative genomics, metabolic profiling and functional genomics, fluorescence in situ hybridization, and phylogenetic microarray are providing new insights into the composition, determinants, and functional roles of human microbiota. The initial colonization and subsequent development of the intestinal microbiota in early life is a key step for the composition of the human microbiota throughout one's life. An increasing body of evidence highlights the role of nutrition both in early infancy and childhood in the age-related development of the microflora, but other factors significantly contribute to the final composition in the adult age, such as antibiotics and/or functional foods, both of which are widely used in children. The microflora in children is plastic and susceptible to changes in response to diet modifications, antibiotic treatment, and other events. In contrast, the microbiota in adults is relatively stable, reflecting the resilience of the microbiome to external perturbations, such as antibiotic therapies. Eubiosis, i.e. a normal microflora structure, provides protection against infections, educates the immune system, ensures tolerance to foods, and contributes to nutrient digestion and energy harvest. However, changes in the microflora, i.e. the presence of too many harmful species or not enough commensal species, called dysbiosis, produce dysfunctions such as intestinal inflammation. It is becoming clear that dysbiosis plays a role in a broad range of important intestinal and extraintestinal diseases such as inflammatory bowel diseases, atopy, and obesity. Moreover, abnormal patterns of microflora have been consistently detected in specific diseases and may provide biomarkers to monitor their course.
Nicastro E, Stephenne X, Smets F, Fusaro F, de Magnée C, Reding R, Sokal EM. Recovery of graft st... more Nicastro E, Stephenne X, Smets F, Fusaro F, de Magnée C, Reding R, Sokal EM. Recovery of graft steatosis and protein‐losing enteropathy after biliary diversion in a PFIC 1 liver transplanted child.: PFIC 1 is a genetic disorder characterized by hepatic and gastrointestinal disease, often requiring LT during childhood. Extrahepatic symptoms, such as diarrhea and malabsorption, do not improve or may be aggravated after LT, as graft steatosis or steatohepatitis as consequences of the interaction between transplanted liver and native bowel. We describe a patient with PFIC 1 who presented with cholestasis in infancy, who developed intractable pruritus and liver fibrosis. The child underwent living donor LT at 3.6 yr of age, and he early developed severe refractory diarrhea, secondary malabsorption with protein‐losing enteropathy, and an early fatty liver disease trough graft steatohepatitis. As the response to cholestyramine was unsatisfactory, we decided to perform an EBD by using the ...
Objective E-learning is a candidate tool for clinical practice guidelines (CPG) implementation du... more Objective E-learning is a candidate tool for clinical practice guidelines (CPG) implementation due to its versatility, universal access and low costs. We aimed to assess the impact of a five-mod-ule e-learning course about CPG for acute gastroenteritis (AGE) on physicians ’ knowledge and clinical practice. Study design This work was conceived as a pre/post single-arm intervention study. Physicians from 11 European countries registered for the online course. Personal data, pre- and post-course questionnaires and clinical data about 3 to 5 children with AGEmanaged by each physician before and after the course were collected. Primary outcomemeasures included the propor-tion of participants fully adherent to CPG and number of patients managed with full adherence.
Journal of Pediatric Gastroenterology & Nutrition, 2020
Objectives: The aim of the study was to assess changes in clinical phenotype, and identify determ... more Objectives: The aim of the study was to assess changes in clinical phenotype, and identify determinants of outcome in children with chronic hepatitis B virus (HBV) infection born in HBV-endemic countries followed in 2 Italian tertiary care centers after immigration or adoption. Methods: A prospective observational study on hepatitis B e-antibodies–negative chronic hepatitis B children started on 2002. Patients with liver fibrosis, or those needing antiviral treatment were excluded. Immune active patients were defined those with raised transaminases (alanine aminotransferase > 40 IU/L), immune tolerants those having normal alanine aminotransferase, both exhibiting substantial viral replication (HBV DNA >2000 IU/mL). Results: Sixty-nine patients (44 boys, median age 4.7 years) had a median follow-up of 53 months. At entry, 18 (26%) children were immune tolerant, 47 (68%) immune active, and 4 had indeterminant immune status. At last follow-up, 14 (78%) of the immune-tolerant pati...
Pediatric Hepatology and Liver Transplantation, 2019
The knowledge of the underlying aetiology of neonatal idiopathic hepatitis and the so-called “int... more The knowledge of the underlying aetiology of neonatal idiopathic hepatitis and the so-called “intrahepatic” cholestasis has been rapidly expanding in the last decade, and great advances in genetic testing have clarified that the vast majority of these conditions are monogenic liver disorders. Among those referred to as progressive familial intrahepatic cholestases (PFICs), the level of serum GGT is a good discriminant to guide the initial evaluation, being low/normal in Byler disease, BSEP deficiency, TJP2 deficiency, FXR deficiency and MYO5B deficiency, and increased only in MDR3 deficiency; however genetic testing is needed to reach a definite categorisation. In bile acid synthesis defects, normal serum bile acid is a clue to the diagnosis, although mass spectrometry is required to characterise the type of defect. Other well-known conditions such as Alagille syndrome and alpha-1 antitrypsin deficiency are more common and less challenging to recognise. In this chapter we discuss th...
Neonatal and infantile cholestasis (NIC) can represent the onset of a surgically correctable dise... more Neonatal and infantile cholestasis (NIC) can represent the onset of a surgically correctable disease and of a genetic or metabolic disorder worthy of medical treatment. Timely recognition of NIC and identification of the underlying etiology are paramount to improve outcomes. Upon invitation by the Italian National Institute of Health (ISS), an expert working grouped was formed to formulate evidence-based positions on current knowledge about the diagnosis of NIC. A systematic literature search was conducted to collect evidence about epidemiology, etiology, clinical aspects and accuracy of available diagnostic tests in NIC. Evidence was scored using the GRADE system. All recommendations were approved by a panel of experts upon agreement of at least 75% of the members. The final document was approved by all the panel components. This position document summarizes the collected statements and defines the best-evidence diagnostic approach to cholestasis in the first year of life.
Advances in experimental medicine and biology, Jan 28, 2016
Tuberculosis is a major problem in children depending on their families for management and a re-e... more Tuberculosis is a major problem in children depending on their families for management and a re-emerging disease in low incidence countries, where foreign-born cases account for a large proportion of cases. We investigated socioeconomic features of families and their impact on management and outcome of children with tuberculosis disease seen at a tertiary care centre for paediatric infectious diseases in Italy. Forty-nine Italian and 30 foreign-origin children were included. Children from foreign families had more complicated diseases (20 % vs 0 %; P = 0.002), harbored more drug resistant strains (20 % vs 2 %; P = 0.011), showed longer hospital stay (12 ± 13.1 vs 5.1 ± 6.5 days; P = 0.012) and higher proportion of missed medical visits (15.7 ± 16 vs 8.6 ± 9.6; P ≤ 0.042) than those from Italian families. Harboring drug resistant strains was an independent risk factor for complicated disease course (OR: 72.98; 95 %CI: 1.54-3468.58; P = 0.029), and this risk is higher in children from...
Culture-independent strategies such as high-throughput parallel sequencing and comparative genomi... more Culture-independent strategies such as high-throughput parallel sequencing and comparative genomics, metabolic profiling and functional genomics, fluorescence in situ hybridization, and phylogenetic microarray are providing new insights into the composition, determinants, and functional roles of human microbiota. The initial colonization and subsequent development of the intestinal microbiota in early life is a key step for the composition of the human microbiota throughout one's life. An increasing body of evidence highlights the role of nutrition both in early infancy and childhood in the age-related development of the microflora, but other factors significantly contribute to the final composition in the adult age, such as antibiotics and/or functional foods, both of which are widely used in children. The microflora in children is plastic and susceptible to changes in response to diet modifications, antibiotic treatment, and other events. In contrast, the microbiota in adults is relatively stable, reflecting the resilience of the microbiome to external perturbations, such as antibiotic therapies. Eubiosis, i.e. a normal microflora structure, provides protection against infections, educates the immune system, ensures tolerance to foods, and contributes to nutrient digestion and energy harvest. However, changes in the microflora, i.e. the presence of too many harmful species or not enough commensal species, called dysbiosis, produce dysfunctions such as intestinal inflammation. It is becoming clear that dysbiosis plays a role in a broad range of important intestinal and extraintestinal diseases such as inflammatory bowel diseases, atopy, and obesity. Moreover, abnormal patterns of microflora have been consistently detected in specific diseases and may provide biomarkers to monitor their course.
Nicastro E, Stephenne X, Smets F, Fusaro F, de Magnée C, Reding R, Sokal EM. Recovery of graft st... more Nicastro E, Stephenne X, Smets F, Fusaro F, de Magnée C, Reding R, Sokal EM. Recovery of graft steatosis and protein‐losing enteropathy after biliary diversion in a PFIC 1 liver transplanted child.: PFIC 1 is a genetic disorder characterized by hepatic and gastrointestinal disease, often requiring LT during childhood. Extrahepatic symptoms, such as diarrhea and malabsorption, do not improve or may be aggravated after LT, as graft steatosis or steatohepatitis as consequences of the interaction between transplanted liver and native bowel. We describe a patient with PFIC 1 who presented with cholestasis in infancy, who developed intractable pruritus and liver fibrosis. The child underwent living donor LT at 3.6 yr of age, and he early developed severe refractory diarrhea, secondary malabsorption with protein‐losing enteropathy, and an early fatty liver disease trough graft steatohepatitis. As the response to cholestyramine was unsatisfactory, we decided to perform an EBD by using the ...
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