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1.
Am J Med Genet A ; 194(1): 88-93, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-37632712

RESUMO

We describe a 2-month-old female infant with macroglossia, macrosomia, omphalocele, neonatal hypoglycemia, earlobe creases, low nasal bridge, midface retrusion, syndromic facies and multiple cutaneous and hepatic hemangiomas (HH). Genetic evaluation confirmed the diagnosis of Beckwith-Wiedemann Syndrome (BWS) with mosaic uniparental disomy 11 as the underlying genetic mechanism suggested by partial hypermethylation of H19/IGF2:IG-DMR and partial hypomethylation of KCNQ1OT1:TSS-DMR on chromosome 11p15.5. Pediatric endocrinology and cardiology assessments were normal. No malignant liver or renal tumors were detected during the follow-up period. Treatment with propranolol was started for the multiple HH, according to international recommendations. At 3-, 6-, and 9-month follow up, a gradual decrease in the size of the hemangiomas and AFP levels was observed, without side effects. This is the fifth case in the literature combining HH and BWS, and among these, the third case with this specific genetic defect suggesting a possible association between HH and BWS caused by 11 paternal uniparental disomy [upd(11)pat]. The case also highlights that if treatment is warranted, then oral propranolol can be used for the management of infantile HH in BWS patients similarly to non-BWS patients.


Assuntos
Síndrome de Beckwith-Wiedemann , Hemangioma , Lactente , Criança , Recém-Nascido , Humanos , Feminino , Síndrome de Beckwith-Wiedemann/complicações , Síndrome de Beckwith-Wiedemann/diagnóstico , Síndrome de Beckwith-Wiedemann/tratamento farmacológico , Dissomia Uniparental , Propranolol/uso terapêutico , Metilação de DNA , Hemangioma/diagnóstico , Hemangioma/tratamento farmacológico , Hemangioma/genética , Fígado , Impressão Genômica
2.
J Pediatr Gastroenterol Nutr ; 77(6): 769-778, 2023 12 01.
Artigo em Inglês | MEDLINE | ID: mdl-37490588

RESUMO

OBJECTIVES: Previous research has shown that critical periods of feeding skills development exist and feeding problems can arise in children due to delays in attaining feeding developmental milestones. This study aims to delineate the transitional process of feeding development in healthy children and children with gastrointestinal diseases (GIDs) and to examine the relationship between feeding milestones and the appearance of later feeding problems. METHODS: A cross-sectional case-control study among 711 healthy Greek children and 119 children with GID, aged 2-7 years. Parents completed the Greek version of the Behavioral Pediatrics Feeding Assessment Scale and "self-reported" questionnaires assessing child's feeding history. RESULTS: Differences in feeding behavior of preschoolers between the 2 groups were found concerning the time of introduction of complementary (pureed), lumpy, and table foods as well as finger feeding and regular cup-drinking. Age-specific milestones as proposed by guidelines were not reached. Late introduction of complementary and lumpy foods (>6 months and >9 months, respectively) as well as finger feeding (>9 months) were predictive of more problematic feeding behaviors later on. CONCLUSIONS: The trajectory of feeding development, including less studied feeding milestones, both in healthy children and children with GIDs, indicates that there are discrepancies between current feeding practices and infant feeding guidelines, and a tendency to delay the acquisition of all assessed feeding milestones.


Assuntos
Comportamento Alimentar , Gastroenteropatias , Lactente , Criança , Humanos , Estudos Transversais , Estudos de Casos e Controles , Pais , Inquéritos e Questionários , Comportamento Infantil , Desenvolvimento Infantil
3.
Eur J Pediatr ; 182(11): 4795-4806, 2023 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-37639015

RESUMO

This is the first meta-analysis of the available literature about the efficacy of metformin exclusively in pediatric patients with non-alcoholic fatty liver disease (NAFLD). We conducted a systematic literature search through major electronic databases till March 12, 2023, investigating the efficacy and safety of metformin in pediatric NAFLD. Weighted mean difference (WD) and standard deviation (SD) were used for continuous outcomes. In total, 4 randomized controlled trials (RCTs) with 309 pediatric patients with NAFLD were included in the meta-analysis. Metformin could not reach a statistically significant improvement in alanine aminotransferase (ALT) levels [(ALT: WMD = - 1.55 IU/L, 95% CI: - 5.38 to 2.28, I2 = 16%, p = 0.43), but had a statistically significant impact (p < 0.05) in insulin and HOMA-IR regulation, triglycerides, and high-density lipoprotein level improvement.   Conclusion: According to the data of this meta-analysis, treatment with metformin failed to statistically improve liver enzymes but may be beneficial in the improvement of lipid parameters and insulin metabolism regulation in pediatric patients with NAFLD. As there are not enough available studies in the literature, the influence of metformin on liver ultrasonography or histology in pediatric NAFLD should be further analyzed in future studies. What is Known: • Lifestyle modification with weight loss through physical activity and dietary modification is the recommended treatment option for pediatric NAFLD. • Metformin may reduce steatosis on ultrasound and may have a beneficial role in liver histology collated with insulin resistance improvement. What is New: • Metformin may improve insulin sensitivity and lipid parameters in children with obesity and NAFLD. • Metformin does not have a significant effect on transaminase levels in children with obesity and NAFLD.


Assuntos
Resistência à Insulina , Metformina , Hepatopatia Gordurosa não Alcoólica , Criança , Humanos , Alanina Transaminase , Insulina , Metformina/uso terapêutico , Hepatopatia Gordurosa não Alcoólica/tratamento farmacológico , Obesidade , Ensaios Clínicos Controlados Aleatórios como Assunto , Triglicerídeos
4.
Curr Issues Mol Biol ; 44(7): 2811-2824, 2022 Jun 28.
Artigo em Inglês | MEDLINE | ID: mdl-35877417

RESUMO

Complement dysregulation has been documented in adults with COVID-19 and implicated in relevant pediatric inflammatory responses against SARS-CoV-2. We propose that signatures of complement missense coding SNPs associated with dysregulation could also be identified in children with multisystem inflammatory syndrome (MIS-C). We investigated 71 pediatric patients with RT-PCR validated SARS-CoV-2 hospitalized in pediatric COVID-19 care units (November 2020-March 2021) in three major groups. Seven (7) patients suffered from MIS-C (MIS-C group), 32 suffered from COVID-19 and were hospitalized (admitted group), whereas 32 suffered from COVID-19, but were sent home. All patients survived and were genotyped for variations in the C3, C5, CFB, CFD, CFH, CFHR1, CFI, CD46, CD55, MASP1, MASP2, MBL2, COLEC11, FCN1, and FCN3 genes. Upon evaluation of the missense coding SNP distribution patterns along the three study groups, we noticed similarities, but also considerably increased frequencies of the alternative pathway (AP) associated with SNPs rs12614 CFB, rs1061170, and rs1065489 CFH in the MIS-C patients. Our analysis suggests that the corresponding substitutions potentially reduce the C3b-inactivation efficiency and promote slower and weaker AP C3bBb pre-convertase assembly on virions. Under these circumstances, the complement AP opsonization capacity may be impaired, leading to compromised immune clearance and systemic inflammation in the MIS-C syndrome.

5.
J Pediatr Gastroenterol Nutr ; 74(5): 662-667, 2022 05 01.
Artigo em Inglês | MEDLINE | ID: mdl-35135959

RESUMO

OBJECTIVES/BACKGROUND: Disease-related malnutrition is common in patients with chronic diseases and has detrimental effects, therefore, skills in nutrition care are essential core competencies for paediatric digestive medicine. The aim of this survey, conducted as part of a global survey of paediatric gastroenterology, hepatology and nutrition (PGHN) training in Europe, was to assess nutrition care-related infrastructure, staff, and patient volumes in European PGHN training centres. METHODS: Standardized questionnaires related to clinical nutrition (CN) care were completed by representatives of European PGHN training centres between June 2016 and December 2019. RESULTS: One hundred training centres from 17 European countries, Turkey, and Israel participated in the survey. Dedicated CN clinics exist in 66% of the centres, with fulltime and part-time CN specialists in 66% and 42%, respectively. Home tube feeding (HTF) andhome parenteral nutrition (HPN) programmes are in place in 95% and 77% of centres, respectively. Twenty-four percent of centres do not have a dedicated dietitian and 55% do not have a dedicated pharmacist attached to the training centre. Even the largest centres with >5000 outpatients reported that 25% and 50%, respectively do not have a dedicated dietitian or pharmacist. Low patient numbers on HTF and HPN of <5 annually are reported by 13% and 43% of centres, respectively. CONCLUSIONS: The survey shows clear differences and deficits in Clinical Nutrition training infrastructure, including staff and patient volumes, in European PGHN training centres, leading to large differences and limitations in training opportunities in Clinical Nutrition.


Assuntos
Gastroenterologia , Criança , Fenômenos Fisiológicos da Nutrição Infantil , Europa (Continente) , Gastroenterologia/educação , Humanos , Sociedades Médicas , Inquéritos e Questionários
6.
J Clin Pediatr Dent ; 43(4): 274-280, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31283894

RESUMO

Objectives: Celiac disease (CD) is an autoimmune disease with typical, atypical and asymptomatic forms, in which many oral manifestations have been recognized. This study aims to evaluate the prevalence of oral manifestations as well as explore if oral examination could be used as a first diagnostic screening tool for atypical or asymptomatic forms. Study Design: 45 CD patients, between 2 and 18 years (mean age 10.3) and 45 healthy subjects, age and gender-matched, were examined for hard and soft tissue lesions such as dental enamel defects (DED), dental caries, aphthous-like ulcers (ALU), atrophic glossitis, geographic tongue, median rhomboid glossitis. Results: Statistically significant differences between the two groups were observed for the prevalence of DED (in 64,4% CD and 24,46% control patients, p=0.001), their location in the teeth (incisal: p=0.0001, middle: p=0.002, cervical: p=0.007), as well as for the prevalence of ALU (in 40% CD as opposed to 4,44% control patients, p=0.001). Conclusion: The presence of DED and ALU could be used as a sign of alert for possible atypical and asymptomatic forms of CD.


Assuntos
Doença Celíaca , Cárie Dentária , Hipoplasia do Esmalte Dentário , Esmalte Dentário , Doenças Dentárias , Adolescente , Doença Celíaca/complicações , Criança , Cárie Dentária/complicações , Esmalte Dentário/patologia , Hipoplasia do Esmalte Dentário/classificação , Humanos , Prevalência , Doenças Dentárias/complicações , Úlcera
7.
Pediatr Int ; 56(1): 89-94, 2014 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-24003895

RESUMO

BACKGROUND: Optimal nutritional status (NS) in cystic fibrosis (CF) is associated with better lung function and increased overall survival. This study estimated the prevalence of malnutrition and obesity among CF patients in a tertiary center. METHODS: In a cross-sectional study of 68 CF patients (33 female; 37 children/adolescents) weight, height, body composition, respiratory function (% of the predicted forced expiratory volume in 1 s; FEV1%pred ) and serum lipids were measured; body mass index (BMI), BMI standard deviation score (BMI-SDS) and BMI percentiles were calculated; Pseudomonas colonization, pancreatic insufficiency, diabetes mellitus (CFDM), liver disease (CFLD) and genotype were recorded; NS was classified according to the 2005 Cystic Fibrosis Foundation (CFF) criteria. Frequency distributions and associations between anthropometric and clinical parameters (univariate/multivariate) were calculated. RESULTS: Mean age (±SD) was 19.81 ± 8.98 years. Regarding NS: 22.1% were malnourished, 13.2% overweight/obese and 29.4% had optimal NS. Pancreatic function (PF), Pseudomonas colonization, CFDM, CFLD and genotype differed significantly among the three groups. FEV1%pred was significantly higher among overweight/obese patients and correlated positively with anthropometric characteristics as well as serum cholesterol and negatively with age. BMI-SDS was associated with PF, FEV1%pred and CFDM. Among overweight/obese patients 89.9% had adequate PF and 66.7% carried mutations other than F508del. No patient had any traits of metabolic syndrome. CONCLUSIONS: Despite appropriate management only one-third of the present patients had optimal NS. One-fourth were malnourished and a significant percentage were overweight/obese. The latter were mostly carriers of mutations other than F508del and had better pulmonary function. CF patients require intensive monitoring for both malnutrition and overweight/obesity.


Assuntos
Fibrose Cística/complicações , Desnutrição/epidemiologia , Estado Nutricional , Obesidade/epidemiologia , Adolescente , Criança , Pré-Escolar , Estudos Transversais , Feminino , Grécia/epidemiologia , Humanos , Masculino , Desnutrição/etiologia , Obesidade/etiologia , Prevalência , Estudos Retrospectivos , Adulto Jovem
8.
Case Rep Genet ; 2024: 8860889, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38283849

RESUMO

Dilated cardiomyopathy with ataxia syndrome is a rare mitochondrial disease caused by autosomal recessive mutations in the DNAJC19 gene. The disease has been described in detail in the Canadian Hutterite population, but a few sporadic cases with de novo mutations have been published worldwide. We describe a homozygous pathogenic variant in the DNAJC19 gene, diagnosed in Northern Greece, presenting with genital anomalies, growth failure, cardiomyopathy, and ataxia, but without increased urinary 3-methylglutaconic acid and additional presence of vitamin D disorders, hypercalciuria, and osteopenia. This case not only expands the clinical characteristics of 3-methylglutaconic aciduria type V (MGCA5) but also highlights the power of genetic analysis for detecting a diagnosis when the metabolic screen is negative.

10.
Acta Paediatr ; 102(7): 749-54, 2013 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-23600795

RESUMO

AIM: Evaluation of the prevalence of coeliac disease (CD) in Greek paediatric population. METHODS: The project consists of two parts: (i) a pilot study of preschool children aged 2-6 years to test the feasibility and diagnostic accuracy of community-based screening and (ii) a CD prevalence study, by random clustered sampling and proportionate stratification of various geographical areas in Greece. Trained nonmedical staff performed a rapid immunochromatographic test to detect IgA antibodies to tTG-IgA and IgA deficiency. Toddlers with positive results were referred to a paediatric gastroenterologist for further assessment with serum anti-tTG IgA and EMA-IgA. Children with positive serum anti-tTG and anti-EMA underwent upper gastrointestinal tract endoscopy and small bowel biopsy and were subsequently in gluten-free diet. RESULTS: In this project participated 1136 toddlers, who were tested at school. The prevalence of positive rapid anti-tTG screening was 1:154, of IgA deficiency 1:120 and of biopsy-proven CD 1:154. The prevalence of CD from this pilot study served as expected prevalence value for sample size calculation for the main prevalence study. CONCLUSION: This protocol using rapid immunochromatographic test for the detection of both IgA deficiency and CD is easy to be performed by nonmedical staff in a community setting, enabling the accurate identification of new CD cases among asymptomatic population.


Assuntos
Doença Celíaca/diagnóstico , Programas de Rastreamento , Doença Celíaca/epidemiologia , Doença Celíaca/imunologia , Criança , Pré-Escolar , Pesquisa Participativa Baseada na Comunidade , Estudos de Viabilidade , Feminino , Proteínas de Ligação ao GTP/imunologia , Grécia/epidemiologia , Humanos , Imunoglobulina A/análise , Masculino , Projetos Piloto , Prevalência , Proteína 2 Glutamina gama-Glutamiltransferase , Transglutaminases/imunologia
11.
J Pediatr Endocrinol Metab ; 36(7): 712-715, 2023 Jul 26.
Artigo em Inglês | MEDLINE | ID: mdl-37141118

RESUMO

Osteopetrorickets is a rare complication of autosomal recessive ("malignant") osteopetrosis. Its prompt diagnosis is essential, because early suspicion of infantile osteopetrosis enables treatment with human stem cell transplantation, depending on the gene involved. It is important to identify not only the characteristic radiological changes of rickets, but also the coexistence of increased bone density, so as not to miss this very rare entity. Herein, a brief case report is presented.


Assuntos
Transplante de Células-Tronco Hematopoéticas , Hipofosfatemia , Osteopetrose , Raquitismo , Humanos , Osteopetrose/diagnóstico , Osteopetrose/diagnóstico por imagem , Raquitismo/complicações , Raquitismo/diagnóstico , Hipofosfatemia/complicações , Radiografia , Transplante de Células-Tronco Hematopoéticas/efeitos adversos
12.
Pan Afr Med J ; 42: 165, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-36187040

RESUMO

Symptoms of gastroesophageal reflux disease (GERD) in children with developmental disorders could be often confusing. Especially when considering accidental foreign body ingestion, with no acute signs or symptoms of choking. We hereby present a case of an 11-year-old male with a well-hidden esophageal foreign body who was already treated for GERD and finally diagnosed with a hidden foreign esophageal object, ingested 12 months ago. Ingestion of objects of any kind, especially in certain groups of children, must always raise high suspicion for the clinical pediatric specialties, regardless of the presence or absence of classic symptoms.


Assuntos
Obstrução das Vias Respiratórias , Corpos Estranhos , Refluxo Gastroesofágico , Obstrução das Vias Respiratórias/complicações , Criança , Corpos Estranhos/complicações , Refluxo Gastroesofágico/diagnóstico , Refluxo Gastroesofágico/etiologia , Humanos , Masculino
13.
Ann Gastroenterol ; 35(3): 297-306, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-35599930

RESUMO

Background: Several studies have detected a strong association linking rs738409 and rs2896019 polymorphisms in the patatin-like phospholipase domain-containing protein 3 (PNPLA3) gene with hepatic steatosis and steatohepatitis. In the present study we aimed to determine the association of those PNPLA3 variants with nonalcoholic fatty liver disease (NAFLD) susceptibility in obese and nonobese Greek children and adolescents. Methods: The study recruited 91 children and adolescents of Greek descent with NAFLD or biopsy-proven nonalcoholic steatohepatitis, and 91 healthy subjects of normal weight (control group) with sex distribution similar to the patient group. DNA samples were amplified using polymerase chain reaction with specifically designed primers. Data were analyzed using the statistical software SPSS version 24.0. Results: A significant correlation was shown between the rs738409 polymorphism (CG and GG genotypes) and the rs2896019 polymorphism (TG genotype) with the development of hepatic steatosis (P<0.001). The incidences of rs738409 GG, rs738409 CG and rs2896019 TG genotypes were found to be increased in patients with hepatic steatosis (obese and nonobese), but not in obese patients without liver disease. The combined expression of the 2 polymorphisms was associated with a lower age of diagnosis of hepatic steatosis in nonobese patients. Conclusions: We confirmed a strong association between the 2 polymorphisms and hepatic steatosis. The association of the rs2896019 single-nucleotide polymorphism with hepatic steatosis in obese and nonobese pediatric patients, and the combined study of both polymorphisms in a pediatric population of Greek origin are described for the first time.

14.
Paediatr Drugs ; 24(2): 137-146, 2022 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-35083718

RESUMO

BACKGROUND: Parenteral iron is generally considered safe in adults, and severe adverse events are extremely rare. Ferric carboxymaltose (FCM), a third-generation parenteral iron product, is not licensed for pediatric use. OBJECTIVE: The aim of this study was to present our data on the safety of FCM in children with iron deficiency (ID) and/or iron deficiency anemia (IDA) and to investigate through a systematic literature review articles reporting on the safety of FCM use in children with ID/IDA. PATIENTS AND METHODS: Safety data regarding children treated with FCM for ID/IDA from four pediatric departments in Greece over a 26-month period are presented. Additionally, a literature search was performed in PubMed, Scopus, and Google Scholar on December 4, 2021 for articles reporting on the use of FCM in children with ID/IDA. Review articles, guidelines, case reports/case series, and reports on the use of FCM for conditions other than ID/IDA were excluded. Identified articles were screened for all reported adverse events (AE) that were graded according to the Common Terminology Criteria for Adverse Events, version 5.0. RESULTS: In our cohort, 37 children with ID/IDA received 41 FCM infusions. All infusions were tolerated well. In addition, 11 articles reporting 1231 infusions of FCM in 866 children were identified in the literature. Among them, 52 (6%) children developed AE that were graded as mild or moderate (grades I-III). CONCLUSIONS: Our patient cohort and this literature review provide further evidence for the good safety profile of FCM in children, although well-designed prospective clinical trials with appropriate safety endpoints are still required.


Assuntos
Compostos Férricos , Maltose , Adulto , Criança , Compostos Férricos/efeitos adversos , Grécia , Humanos , Maltose/efeitos adversos , Maltose/análogos & derivados , Estudos Prospectivos
15.
Neurogastroenterol Motil ; 34(3): e14222, 2022 03.
Artigo em Inglês | MEDLINE | ID: mdl-34468064

RESUMO

BACKGROUND: Stressful events during infancy may predispose to the development of functional gastrointestinal disorders (FGIDs) in childhood. AIMS: To evaluate the association of necrotizing enterocolitis (NEC) with childhood FGIDs. METHODS: We conducted a study, comparing 29 children of eight to ten years with a history of NEC with 58 children with no history of NEC. Subjects were assessed for FGIDs, based on Rome-III criteria. RESULTS: Among 29 subjects with NEC, 17 had surgical and 12 conservative NEC. Subjects with surgically, or conservatively managed NEC developed FGIDs at a significantly higher proportion, as compared to children with no history of NEC, later in childhood (41%, 33%, and 13% respectively, p = 0.033). Functional constipation was the most frequently identified disorder (35%, 33%, and 7% respectively). A significant association was detected between FGIDs and the history of perinatal stress (p = 0.049), NEC (p = 0.011), and the surgical management of NEC (p = 0.015). CONCLUSIONS: Our study suggests that there is a potential association between NEC and FGIDs later in childhood with functional constipation being the most frequently identified disorder.


Assuntos
Enterocolite Necrosante , Gastroenteropatias , Criança , Constipação Intestinal/epidemiologia , Enterocolite Necrosante/complicações , Enterocolite Necrosante/epidemiologia , Feminino , Gastroenteropatias/complicações , Gastroenteropatias/epidemiologia , Humanos , Recém-Nascido , Recém-Nascido Prematuro , Gravidez
16.
Nutrients ; 14(17)2022 Aug 23.
Artigo em Inglês | MEDLINE | ID: mdl-36079710

RESUMO

Non-alcoholic fatty liver disease (NAFLD) shares several risk factors with atherosclerosis, as it is associated with components of the metabolic syndrome. However, genetic variations have also been linked to the risk of NAFLD, such as adiponutrin/patatin-like phospholipase domain-containing the protein 3 (PNPLA3) rs738409 polymorphism. The aim of the study was to determine the associations of thePNPLA3 rs738409 polymorphism with NAFLD and atherosclerosis risk factors in children and adolescents from northern Greece. A total of 91 children/adolescents who followed a Mediterranean eating pattern with no particular restrictions were studied. They were divided into three subgroups, according to their body mass index (BMI) and the presence or absence of liver disease. Diagnosis of NAFLD was based on a liver ultrasound, while the distribution of the PNPLA3 rs738409 polymorphism was investigated in all the participants. From the components of metabolic syndrome, only BMI, waist circumference, blood pressure, and the homeostasis model of insulin resistance (HOMA-IR) differed significantly between groups. The rs738409 polymorphism was significantly associated with BMI and NAFLD, while lipid values had no significant association with either NAFLD or gene polymorphism. This study shows that in Greekchildren, there is a significant association between the rs738409polymorphism in the PNPLA3 gene and hepatic steatosis, regardless of bodyweight.


Assuntos
Aciltransferases , Aterosclerose , Proteínas de Membrana , Síndrome Metabólica , Hepatopatia Gordurosa não Alcoólica , Fosfolipases A2 Independentes de Cálcio , Aciltransferases/genética , Adolescente , Aterosclerose/genética , Criança , Predisposição Genética para Doença , Genótipo , Grécia , Humanos , Lipase/genética , Fígado , Proteínas de Membrana/genética , Síndrome Metabólica/genética , Hepatopatia Gordurosa não Alcoólica/genética , Fosfolipases A2 Independentes de Cálcio/genética , Polimorfismo de Nucleotídeo Único , Fatores de Risco
17.
Endosc Int Open ; 10(10): E1371-E1379, 2022 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-36262519

RESUMO

Background and study aims The ability to perform endoscopy procedures safely and effectively is a key aspect of quality clinical care in Pediatric Gastroenterology, Hepatology and Nutrition (PGHN). The aim of this survey, which was part of a global survey on PGHN training in Europe, was to assess endoscopy training opportunities provided across Europe. Methods Responses to standardized questions related to endoscopy training were collected from training centers across Europe through the presidents/representatives of the European Society for Paediatric Gastroenterology, Hepatology and Nutrition National Societies from June 2016 to December 2019. Results A total of 100 training centers from 19 countries participated in the survey. In 57 centers, the endoscopy suit was attached to the PGHN center, while in 23, pediatric endoscopies were performed in adult endoscopy facilities. Ninety percent of centers reported the availability of specialized endoscopy nurses and 96 % of pediatric anesthetists. Pediatric endoscopies were performed by PGHN specialists in 55 centers, while 31 centers reported the involvement of an adult endoscopist and 14 of a pediatric surgeon. Dividing the number of procedures performed at the training center by the number of trainees, ≤ 20 upper, lower, or therapeutic endoscopies per trainee per year were reported by 0 %, 23 %, and 56 % of centers, respectively, whereas ≤ 5 wireless capsule endoscopies per trainee per year by 75 %. Only one country (United Kingdom) required separate certification of competency in endoscopy. Conclusions Differences and deficiencies in infrastructure, staffing, and procedural volume, as well as in endoscopy competency assessment and certification, were identified among European PGHN training centers limiting training opportunities in pediatric endoscopy.

18.
Ann Gastroenterol ; 35(3): 325-332, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-35599925

RESUMO

Background: Pediatric gastrointestinal motility disorders present significant challenges for diagnosis and management, emphasizing the need for appropriate training in Pediatric Neurogastroenterology and Motility (PNGM). The aim of this survey, part of a comprehensive survey on training in pediatric gastroenterology, hepatology and nutrition, was to evaluate training related to PNGM across European training centers. Method: Standardized questionnaires were collected from training centers through the National Societies Network of the European Society for Paediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN), from June 2016 to December 2019. Results: In total, 100 training centers from 19 countries participated in the survey. Dedicated PNGM clinics were available in 22 centers; pH-monitoring in 60; pH/impedance in 66; standard manometry in 37; and high-resolution manometry in 33. If all motility studies were performed partially or fully by the trainees, the median (range) annual numbers/per trainee were as follows: pH-monitoring 30 (1-500); pH/impedance 17 (1-131); standard manometries 10 (1-150); and high-resolution manometries 8 (1-75). The motility assessment was performed by pediatric gastroenterologists (43 centers); adult gastroenterologists (10 centers); pediatric surgeons (5 centers); and both pediatric gastroenterologists and pediatric surgeons (9 centers). Annual numbers ≤10 for pH-monitoring, pH/impedance, standard manometries and high-resolution manometries were reported by 7 (12%), 15 (23%), 11 (30%) and 14 (42%) centers, respectively. Conclusions: Significant differences exist in PNGM-related infrastructure, staff and procedural volumes at training centers across Europe. ESPGHAN and the National Societies should take initiatives to ensure the acquisition of competence in PNGM-related knowledge and skills, and develop strategies for assessment and accreditation.An infographic is available for this article at: http://www.annalsgastro.gr/files/journals/1/earlyview/2022/Infographic-AG6486.pdf.

19.
Ann Gastroenterol ; 35(3): 317-324, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-35599923

RESUMO

Background: This survey evaluated the effects of the recognition of pediatric gastroenterology, hepatology and nutrition (PGHN) on European PGHN training centers. Method: Standardized questionnaires were collected from training centers via the presidents/representatives of the National Societies Network of the European Society for Pediatric Gastroenterology, Hepatology and Nutrition, from June 2016 to December 2019. Results: A total of 100 training centers from 19 countries participated in the survey: 55 from 12 countries where PGHN is formally recognized (Group 1) and 45 from 7 countries where it is not (Group 2). Training centers in Group 2 were less likely to have an integrated endoscopy suite, a written training curriculum and a training lead (P=0.059, P<0.001 and P=0.012, respectively). Trainees in Group 2 were less likely to be exposed to an adequate number of diagnostic endoscopies, while no differences were found in relation to liver biopsies. Half of the training centers in both Groups do not have dedicated beds for PGHN patients, while in 64% and 58%, respectively, trainees do not participate in on-call programs for PGHN emergencies. Research training is mandatory in 26% of the centers. The duration of training, as well as the assessment and accreditation policies, vary between countries. Conclusions: This study has revealed significant discrepancies and gaps in infrastructure and training programs, training leadership, and assessment of training and certification across European training centers in PGHN. Strategies to support the recognition of PGHN and to standardize and improve training conditions should be developed and implemented.An infographic is available for this article at: http://www.annalsgastro.gr/files/journals/1/earlyview/2022/Infographic_AG-6496.pdf.

20.
Ann Gastroenterol ; 35(2): 187-193, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-35479583

RESUMO

Background: The widely recognized burden of liver diseases makes training in pediatric hepatology (PH) imperative. The aim of this survey, which was part of a global survey on training in pediatric gastroenterology, hepatology and nutrition (PGHN) across Europe, was to assess the PH and liver transplantation (LT) infrastructure, staff and training programs in PGHN training centers. Method: Standardized questionnaires were collected from training centers via the presidents/representatives of the National Societies Network of the European Society for Paediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN) from June 2016 to December 2019. Results: A total of 100 PGHN training centers participated in the survey (14/100 were national referral centers in PH and/or LT). Dedicated PH clinics were available in 75%, but LT clinics in only 11%. Dedicated beds for PGHN inpatients were available in 47/95 (49%) centers. Full-time or part-time specialists for PH care were available in 31/45 (69%) and 11/36 (31%) centers, respectively. Liver biopsies (LB) were performed in 93% of centers by: a PGHN specialist (35%); an interventional radiologist (26%); a pediatric surgeon (4%); or a combination of them (35%). Dividing the annual number of LBs in the centers performing LBs by the number of trainees gave a median (range) of 10 (1-125) per trainee. Transient elastography was available in 60/92 (65%) of centers. Conclusions: The survey highlighted the differences and shortcomings in PH training across Europe. ESPGHAN should take initiatives together with National Societies to ensure the acquisition of PH knowledge and skills according to the ESPGHAN curriculum.An infographic is available for this article at: http://www.annalsgastro.gr/files/journals/1/earlyview/2022/Infographic-Hepatology-training-paper.pdf.

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