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1.
J Pediatr Gastroenterol Nutr ; 68(5): 700-705, 2019 05.
Artigo em Inglês | MEDLINE | ID: mdl-30676519

RESUMO

OBJECTIVES: We aimed to investigate national allocation policies for pediatric liver transplantation (LT). METHOD: A survey was prepared by the European Society for Paediatric Gastroenterology Hepatology and Nutrition Hepatology Committee in collaboration with the North American Studies of Pediatric Liver Transplantation consortium. The survey was sent to pediatric hepatologists and transplant surgeons worldwide. National data were obtained from centrally based registries. RESULTS: Replies were obtained from 15 countries from 5 of the world continents. Overall donation rate varied between 9 and 35 per million inhabitants. The number of pediatric LTs was 4 to 9 per million inhabitants younger than 18 years for 13 of the 15 respondents. In children younger than 2 years mortality on the waiting list (WL) varied between 0 and 20%. In the same age group, there were large differences in the ratio of living donor LT to deceased donor LT and in the ratio of split liver segments to whole liver. These differences were associated with possible discrepancies in WL mortality. CONCLUSIONS: Similarities but also differences between countries were detected. The described data may be of importance when trying to reduce WL mortality in the youngest children.


Assuntos
Gastroenterologia/legislação & jurisprudência , Política de Saúde , Transplante de Fígado/legislação & jurisprudência , Pediatria/legislação & jurisprudência , Obtenção de Tecidos e Órgãos/legislação & jurisprudência , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Listas de Espera/mortalidade
2.
Arch Dis Child ; 102(7): 607-611, 2017 07.
Artigo em Inglês | MEDLINE | ID: mdl-28232458

RESUMO

OBJECTIVES: Coeliac disease (CD) and juvenile idiopathic arthritis (JIA) often coexist. This association warrants assessment for CD in patients with JIA. We evaluated the clinical relevance and cost-effectiveness of human leucocyte antigen (HLA) genotyping in first-line screening for development of CD in children with JIA. PATIENTS AND INTERVENTIONS: 95 patients with JIA were screened for CD using CD-specific antibodies. In case of positivity, a small intestinal biopsy was performed to confirm diagnosis. In addition, HLA genotyping was performed. 110 age-matched and sex-matched Caucasian children from the same geographical area served as controls. RESULTS: CD was diagnosed in 4 of 95 patients with JIA (4.2%), a rate significantly higher compared with controls (p<0.02) and 14 times higher than in the general population. Twenty-six patients (27.4%) had one of the variants of the risk genotypes. All four patients diagnosed with CD had a HLA-DQ2.5 genotype: one was homozygote, the remainder heterozygote. Twenty-two patients are, judging by their HLA genotypes, at risk of developing CD and require repeated serological screening. None of the 69 patients without HLA-DQ2/DQ8 genotypes had CD-specific antibodies. Screening with HLA genotyping becomes cheaper than screening without after the second determination. CONCLUSIONS: In our cohort of patients with JIA, lack of HLA-DQ2/DQ8 genotypes identified a majority not at risk of CD in whom repeated serological testing is unnecessary. Genotyping is nowadays the most efficient and cost-effective way to screen for CD risk in JIA.


Assuntos
Artrite Juvenil/genética , Doença Celíaca/genética , Antígenos HLA-DQ/genética , Adolescente , Idade de Início , Artrite Juvenil/economia , Artrite Juvenil/imunologia , Autoanticorpos/metabolismo , Doença Celíaca/diagnóstico , Doença Celíaca/economia , Doença Celíaca/imunologia , Criança , Pré-Escolar , Análise Custo-Benefício , Diagnóstico Precoce , Feminino , Genótipo , Técnicas de Genotipagem/economia , Técnicas de Genotipagem/métodos , Humanos , Lactente , Masculino , Estudos Prospectivos
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