Acute liver failure in children

Akutno zatajenje jetre u djece je rijetki brzoprogresivni sindrom koji dovodi do oštećenja jetre, a može dovesti do oštećenja i drugih organa. Incidencija iznosi deset slučajeva na milijun. Etiologija varira ovisno o dobi, a uzroke dijelimo na infektivne, metaboličke i imunološke, povezane s lijekovima i toksinima te ostale poput hipoperfuzije ili maligne bolesti. U većini slučajeva uzrok ostaje nedefiniran. Simptomi su većinom nespecifični. Lijekovi su česti uzročnici AZJ kao i nasljedne metaboličke bolesti. Zarazne bolesti mogu, također prethoditi teškom oštećenju jetre. Od autoimunih bolesti koje mogu uzrokovati AZJ najčešći je autoimuni hepatitis. Paracetamol je dobro podnošljiv lijek kada se koristi unutar svoje uske terapijske širine. Hepatotoksičnom dozom smatra se 100 mg/kg, a hepatotoksični metabolit je N acetilpara-amino benzokinon imin (NAPQI). Simptomi trovanja su nespecifični. Antidot N-acetilcistein je učinkovit ako se primjenjuje brzo nakon primjene lijeka. Wilsonova bolest, nasljedni poremećaj metabolizma bakra, karakterizira se nakupljanjem bakra u jetri, ali i drugim organima. Uočavaju se simptomi i znakovi oštećenja jetre, ali i neurološki i psihijatrijski simptomi. Kod deficita α 1-atitripsina, najčešćeg uzroka metaboličke bolesti jetre u djece, dolazi do nakupljanja abnormalnog α 1-antitripsina i oštećenja u jetri te do oštećenja pluća zbog smanjenja zaštitnog učinka. Dijagnostika je klinička, radiološka i laboratorijska. Usprkos cijepljenju, hepatitis B najčešći je infektivni uzročnik AZJ. Spektar kliničkih simptoma i znakova najviše ovisi o imunološkom sustavu domaćina, a dijagnostika je pretežno laboratorijska. Akutno zatajenje jetre u djece zahtijeva hitno liječenje i multidisciplinarni tim. Često je u bolesnika s AZJ transplantacija jedina opcija preživljenja.Acute liver failure in children is a rare rapidly progressive syndrome that leads to liver damage and can lead to damage to other organs as well. The incidence is ten cases per million. The etiology varies with age, and the causes are divided into infectious, metabolic and immune, related to drugs and toxins and others such as hypoperfusion or malignancy. In most cases, the cause remains undefined. The symptoms are mostly nonspecific. Medications are common causes of ASD as well as hereditary metabolic diseases. Infectious diseases can also precede severe liver damage. Of the autoimmune diseases that can cause ASD, autoimmune hepatitis is the most common. Paracetamol is a well-tolerated drug when used within its narrow therapeutic range. The hepatotoxic dose is considered to be 100 mg / kg and the hepatotoxic metabolite is N acetylpara-amino benzoquinone imine (NAPQI). The symptoms of poisoning are nonspecific. The antidote N-acetylcysteine is effective if administered soon after drug administration. Wilson's disease, an inherited disorder of copper metabolism, is characterized by the accumulation of copper in the liver but also in other organs. Symptoms and signs of liver damage are observed, as well as neurological and psychiatric symptoms. Deficiency of α 1-atitrypsin, the most common cause of metabolic liver disease in children, leads to the accumulation of abnormal α 1-antitrypsin and liver damage, as well as lung damage due to reduced protective effect. Diagnosis is clinical, radiological and laboratory. Despite vaccination, hepatitis B is the most common infectious agent of AZJ. The spectrum of clinical symptoms and signs depends mostly on the host's immune system, and the diagnosis is predominantly laboratory. Acute liver failure in children requires urgent treatment and a multidisciplinary team. Transplantation is often the only survival option in patients with ASD