Assuntos
Atresia Biliar/diagnóstico por imagem , Atresia Biliar/história , Biópsia/métodos , Fígado/patologia , Atresia Biliar/patologia , Criança , Colangiografia/métodos , Feminino , História do Século XX , Humanos , Lactente , Masculino , Pediatria/história , Publicações Periódicas como Assunto/história , Sensibilidade e Especificidade , Estados UnidosAssuntos
Atresia Biliar/diagnóstico , Colestase Extra-Hepática/diagnóstico , Programas de Rastreamento/métodos , Pediatria/história , Atresia Biliar/história , Colestase Extra-Hepática/história , Diagnóstico Diferencial , História do Século XX , Humanos , Iminoácidos , Lactente , Programas de Rastreamento/história , Rosa Bengala/administração & dosagemAssuntos
Atresia Biliar/história , Cirrose Hepática Biliar/história , Neoplasias Hepáticas/história , Portoenterostomia Hepática/história , Atresia Biliar/cirurgia , Criança , História do Século XX , Humanos , Cirrose Hepática Biliar/complicações , Neoplasias Hepáticas/complicações , Pediatria/históriaRESUMO
The age threshold at the time of Kasai hepatic portoenterostomy associated with a prognosis of biliary atresia (BA) is becoming clearer as 10-year native liver survival data become more frequent in the recent literature, whereas the age at diagnosis has not dramatically decreased during the last 3 decades. A stool color card screening implemented in 1994 in Japan is now expanding worldwide. However, the contribution of this modality will probably be limited because of the nature of this disease, for example, 'progressive obliterative cholangiopathy'. A cholic stool was actually observed only in 50% before diagnosis according to the Japanese BA Registry data. Thus, color card screening does not appear to be instrumental in detecting patients with BA early enough before 1 month of age. A highly sensitive, adequately specific, noninvasive and quantitative method may be expensive, but the overall cost would be lower than that of liver transplant.
Assuntos
Atresia Biliar/diagnóstico , Técnicas de Diagnóstico do Sistema Digestório/tendências , Fatores Etários , Atresia Biliar/história , Atresia Biliar/cirurgia , Diagnóstico Precoce , Fezes/química , História do Século XX , História do Século XXI , Humanos , Portoenterostomia Hepática , PrognósticoRESUMO
Morio Kasai is one of the most influential Japanese pediatric surgeons. He is best recognized in the United States for his pioneering efforts in the field of biliary atresia. His work revolutionized the treatment of infants born with biliary atresia throughout the world. Less is known about his remarkable impact in the fields of general surgery and pediatric surgery. This review highlights some of Dr Kasai's major contributions and highlights the influence he had in the establishment of pediatric surgery as a field.
Assuntos
Atresia Biliar/história , Cirurgia Geral/história , Pediatria/história , Atresia Biliar/cirurgia , História do Século XX , História do Século XXI , Humanos , JapãoRESUMO
During the last decade, biliary atresia (BA) has attained more interest and the frequency of BA-related publications has increased continuously. Pediatric hepatologists and pediatric surgeons are very active in improving diagnosis and treatment modalities of BA patients, in order to prolong the survival rate of their native liver. Together with transplant surgeons, the bridging of BA patients to liver transplantation (LTx) becomes optimized and as a consequence of this interdisciplinary approach, the overall survival of babies with BA has already reached 90%. Furthermore, basic research into the still unknown origin of BA has advanced, and numerous scientific programs have already linked together. The overriding interest is to discover at least BA's etiology and to turn the treatment of BA patients from a symptomatic to a causative approach. Interdisciplinary and international programs are mandatory and already existing initiatives in Europe, the United States and Japan are going to coordinate their registries, clinical trials and basic research studies for the benefit of the patients and solve the riddle of BA.
Assuntos
Atresia Biliar , Algoritmos , Atresia Biliar/etiologia , Atresia Biliar/história , Atresia Biliar/terapia , Europa (Continente) , História do Século XIX , História do Século XX , Humanos , Recém-Nascido , Comunicação Interdisciplinar , Cooperação Internacional , Japão , Portoenterostomia Hepática , Sistema de Registros , Pesquisa , Estados UnidosAssuntos
Procedimentos Cirúrgicos do Sistema Digestório/história , Atresia Biliar/história , Atresia Biliar/cirurgia , Colestase/história , Colestase/cirurgia , Procedimentos Cirúrgicos do Sistema Digestório/métodos , Epônimos , Gastrectomia/história , Gastrectomia/métodos , História do Século XX , Jejunostomia/história , Jejunostomia/métodos , Jejuno/cirurgia , Fígado/cirurgia , Pancreaticoduodenectomia/história , Pancreaticoduodenectomia/métodos , Estados UnidosRESUMO
European studies of biliary atresia have suggested that the aetiology is heterogeneous. Histological studies of the liver and biliary remnants excised at portoenterostomy have failed to identify any prognostic features except for the size of bile ductules in the porta hepatis. Most of the major series have confirmed that there is a relationship between age at portoenterostomy and clearance of jaundice which has been achieved in more than 67% of infants under 10 weeks of age. Cholangitis reduced survival and bleeding from esophageal varices has occurred in more than 19% of long-term survivors. The 5-year jaundice-free survival rate after portoenterostomy is 37% and the 10 year rate is 18%. It is predicted that these survival rates will improve. Orthotopic transplantation now results in long-term survival in 70% of patients who fail the portoenterostomy operation and long-term survival is now achieved in a majority of children born with biliary atresia.
