RESUMO
Bilirubin is a tetrapyrrolic compound originating from heme catabolism. Although originally considered only a potentially dangerous waste product, it has become increasingly evident that this molecule represents an important modulator of various biological functions in the human body. Bilirubin appears to have versatile functions, from cell signaling (behaving almost like a "real" hormonal substance), modulation of metabolism, to immune regulation, affecting biological activities with apparent clinical and even therapeutic consequences. These activities may be the reason for the lower incidence of diseases of civilisation (cardiovascular diseases, arterial hypertension, diabetes, obesity, metabolic syndrome, certain cancers, autoimmune, and neurodegenerative diseases) observed in individuals with a chronic mild unconjugated hyperbilirubinemia, a typical sign of Gilbert's syndrome. While higher serum concentrations of unconjugated bilirubin may serve as an important protective factor against these diseases, low levels of bilirubin are associated with the opposite effect.
Assuntos
Bilirrubina/análise , Bilirrubina/classificação , Cor , Heme/metabolismo , Humanos , Hiperbilirrubinemia/classificação , Hiperbilirrubinemia/fisiopatologiaAssuntos
Atresia Biliar/classificação , Fenótipo , Idade de Início , Atresia Biliar/diagnóstico , Atresia Biliar/genética , Bilirrubina/sangue , Criança , Diagnóstico Diferencial , Fezes , Feminino , Artéria Hepática/anormalidades , Humanos , Hiperbilirrubinemia/classificação , Hiperbilirrubinemia/diagnóstico , Lactente , Recém-Nascido , Masculino , Anamnese , Gravidez , Estudos Retrospectivos , Taiwan , Ultrassonografia Pré-NatalRESUMO
Ten adult patients with chronic nonhemolytic unconjugated (indirect) hyperbilirubinemia were analyzed by determining bilirubin uridine diphosphate-glucuronosyltransferase activity according to a more physiological and sensitive method (9 control cases, 0.457 +/- 0.163 nmole/mg protein/min). There was no overlap of the enzyme activities of 2 cases with Crigler-Najjar syndrome (type II) (0.006 nmole/mg protein/min on average) and 6 cases with Gilbert's syndrome (0.051 +/- 0.016 nmole/mg protein/min). The enzyme activities in 2 patients with post-hepatitic hyperbilirubinemia were within the normal range. A new classification of nonhemolytic unconjugated hyperbilirubinemia in adults is proposed according to the results of this enzyme activity and the recent data on the gene mutation of this enzyme.
Assuntos
Glucuronosiltransferase/metabolismo , Hiperbilirrubinemia/enzimologia , Fígado/enzimologia , Adolescente , Adulto , Estudos de Casos e Controles , Doença Crônica , Síndrome de Crigler-Najjar/classificação , Síndrome de Crigler-Najjar/enzimologia , Síndrome de Crigler-Najjar/genética , Feminino , Doença de Gilbert/classificação , Doença de Gilbert/enzimologia , Doença de Gilbert/genética , Glucuronosiltransferase/genética , Humanos , Hiperbilirrubinemia/classificação , Hiperbilirrubinemia/genética , Icterícia/enzimologia , Icterícia/etiologia , Masculino , Pessoa de Meia-Idade , MutaçãoRESUMO
This study was undertaken to assort the group of posthepatectomy hyperbilirubinemia by occurring time for helping elucidate its indication of plasma exchange (PE). PE was carried out for 14 cases among 198 hepatectomized patients. The 14 cases were classified for 3 groups due to the period between operation and PE. 1: Early type (less than 2 weeks), 2:Intermediate type (2 less than-less than 8 weeks), 3: Late type (greater than 8 weeks). The results were as follows. Number (liver cirrhosis (LC)) [table: see text]. The effect was expected for the early groups since PE would remove overload against the regenerating residual liver. However, there were no survival cases among the early group or LC group. These results suggest that PE may be effective for the slowly increasing hyperbilirubinemia in not cirrhotic patients and that the state of hyperbilirubinemia is the terminal stage for LC patients even under the particular situation such as posthepatectomy.
