Pediatria pre prax 4/2002
Nejčastější metabolicky podmíněná onemocnění jater u nemocného v adolescentním věku
The most common metabolic disorders involving liver among adolescent patients The article summarizes the most important information of the most frequent metabolic disorders to be found in adolescent age – benign hyperbilirubinemia and Wilson´s disease. Hyperbilirubinemia occurs quite often, it is entirely benign. The task of a physician is to diagnose it correctly and explain harmless features of this disorder to a patient, to relieve him from anxiety and not to take any therapeutic or dietary precautions. On the contrary, when Wilson´s disease is suspected, it is absolutely necessary to proceed with great responsibility from the very beginning, because present available diagnostic methods allow us to detect disease in clinically latent stage and thus to prevent it from fatal consequences by early therapy. The treatment is successful so far, that a patient can live normal life. Once initiated, therapy must be continued forever. A patient has to be followed up on regular basis by an attending physician or in cooperation with specialized center. All siblings have to be examined as well. Key words: Gilbert´s syndrome, benign hyperbilirubinemia, unconjugatedbilirubinemia, Wilson´s disease, ceruloplasmin, disorders of copper metabolism.
