RESUMO
BACKGROUND: Biliary atresia (BA) is a severe neonatal liver disease characterized by progressive extrahepatic biliary tract and intrahepatic inflammatory process. Hepatic fibrosis and portal hypertension (PH) still occur despite the disappearance of jaundice following successful hepatic portoenterostomy. Endothelin-1 (ET-1) is a potent vasoconstrictor and has been reported to stimulate hepatic collagen synthesis. The aim of this study was to demonstrate the potential role of ET-1 in the pathogenesis of the progressive inflammation, fibrosis and PH in BA. METHODS: Thirty pediatric patients with biliary atresia post-hepatic portoenterostomy and 12 healthy children were examined. The ET-1 level was determined by commercially available enzyme-linked immunosorbent assay kits. RESULTS: Endothelin-1 levels were elevated in the patients compared with those of the controls (5.45+/-3.34 vs. 2.74+/-2.17 pg/ml, P = 0.01). Moreover, patients with PH also had greater levels of ET-1 than those without PH (6.73+/-3.27 vs. 3.26+/-2.2 pg/ml, P = 0.004). Patients with abnormal transaminase enzymes had significantly higher ET-1 levels than those with normal enzymes (6.43+/-3.33 vs. 3.17+/-2.1 pg/ml, P = 0.01). In the jaundice-free group, endothelin-1 levels were elevated in the patients with PH compared with those without PH (5.93+/-2.15 vs. 2.88+/-2.1 pg/ml, P = 0.02). CONCLUSIONS: Our findings showed elevation of plasma ET-1 levels in patients with BA, especially in those with PH. ET-1 levels were also higher in patients with elevated transminase enzymes as well as in the jaundice-free group with PH. ET-1 might play a role in the pathogenesis of the progressive inflammation, fibrosis and PH in BA.
Assuntos
Atresia Biliar/sangue , Endotelina-1/sangue , Hipertensão Portal/sangue , Atresia Biliar/complicações , Criança , Pré-Escolar , Progressão da Doença , Feminino , Fibrose , Humanos , Hipertensão Portal/etiologia , Lactente , MasculinoAssuntos
Aneurisma Infectado/diagnóstico por imagem , Artéria Pulmonar/diagnóstico por imagem , Aneurisma Infectado/complicações , Aneurisma Infectado/tratamento farmacológico , Criança , Permeabilidade do Canal Arterial/complicações , Permeabilidade do Canal Arterial/cirurgia , Feminino , Humanos , Penicilina G/uso terapêutico , Penicilinas/uso terapêutico , RadiografiaRESUMO
An increased awareness of fetal arrhythmias by obstetricians has resulted in a growing number of diagnosed cases of fetal cardiac problems. A fetus with atrial flutter diagnosed at 31 weeks of gestation was successfully converted to normal sinus rhythm in utero by maternal administration with digitalis. The fetal heart rate stayed in a normal rhythm with a maintenance dose of 0.5 mg/day. The fetus was delivered spontaneously at term and the neonatal heart rate has been in normal rhythm without any medical treatment.