title: Prognostic marker for liver disease due to alpha1-antitrypsin deficiency.
abstract: BACKGROUND
  Only some Alpha1-antitrypsin deficiency (A1ATD) PiZZ patients develop liver cirrhosis and portal hypertension. Aim of the study was to investigate the course of liver disease associated with PiZZ A1ATD and to determine prognostic factors.
  
  
  METHODS
  We retrospectively reviewed the clinical and laboratory data of all PiZZ children up to 18 years of age admitted to our centre since 1978. 53 patients (age at first visit 2 days to 12 years) met our criteria.
  
  
  METHODS
  The children were divided into 2 groups: group 1 'bad prognosis', meaning the patients which were on the waiting list for liver transplantation (LTx), had a liver transplantation or had died, and group 2 'good prognosis', containing the patients they were living with their own liver. We analysed family history including smoking, gestational age, maternal age at delivery, date of birth, sex, neonatal history, breast-feeding, symptoms at presentation, clinical and laboratory data and date of LTx and/or death.
  
  
  RESULTS
  Various anamnesis parameters such as manifestation of neonatal cholestasis showed no prognostic significance. In contrast the laboratory parameters thrombocytes (p=0.008), bilirubin (p<0.001), prothrombin time (p<0.001), choline-sterase (p<0.001), gamma-GT (p=0.001) and GOT (p=0.002) showed a correlation with a liver transplantation and/or death.
  
  
  CONCLUSIONS
  Prognosis is difficult to determine at an early stage of this disease, but various laboratory parameters can help to predict an outcome. Therefore a regular follow-up is necessary for the children.
authors: Department of Paediatric Gastroenterology and Hepatology, Hannover Medical School, Hannover, Germany.
authors: D C
authors: DC
authors: Pferdmenges
authors: U
authors: U
authors: Baumann
authors: A
authors: A
authors: Müller-Heine
authors: T
authors: T
authors: Framke
authors: E-D
authors: ED
authors: Pfister
mesh_terms: D000293:Adolescent; D000410:Alanine Transaminase / Q000097:blood; D001219:Aspartate Aminotransferases / Q000097:blood; D001663:Bilirubin / Q000097:blood; D015415:Biomarkers / Q000097:blood*; D002648:Child; D002675:Child, Preschool; D002802:Cholinesterases / Q000097:blood; D005260:Female; D006801:Humans; D006975:Hypertension, Portal / Q000097:blood / Q000175:diagnosis* / Q000453:epidemiology* / Q000401:mortality; D007223:Infant; D007231:Infant, Newborn; D008103:Liver Cirrhosis / Q000097:blood / Q000175:diagnosis* / Q000453:epidemiology* / Q000401:mortality; D016031:Liver Transplantation; D008297:Male; D010976:Platelet Count; D011379:Prognosis; D011517:Prothrombin Time; D012189:Retrospective Studies; D013223:Statistics as Topic; D019896:alpha 1-Antitrypsin Deficiency / Q000097:blood / Q000175:diagnosis* / Q000453:epidemiology* / Q000401:mortality
chemical_list: D015415:Biomarkers; D001219:Aspartate Aminotransferases; D000410:Alanine Transaminase; D002802:Cholinesterases; D001663:Bilirubin