RECIDIVISTIC COMA HEPATICUM BECAUSE OF AL PHA-1-ANTITRYPSIN DEFICIENCY WITH ATYPICAL SERUM-ELECTROPHORESIS

A 59-year old woman with non-insulindependent diabetes mellitus was ad mitted to several hospitals within short intervals because of unconsci ousness. The first suspected diagnosis was coma hypoglycemia, at the s econd admission a stroke (PRIND) was presumed. After strumectomy in th e meantime a tranquilizer-overdose was assumed at the next admission. The following times a coma hypothyreoticum and at last a >>coma due to metabolic disturbance of unknown cause<< was diagnosed. Liver enzymes were always normal but during a repealed internal investigation an el evated ammonia-level was measured for the first time and therefore the diagnosis coma hepaticum was established. The electrophoresis showed an increment of the alpha-2- as well as the beta- and gamma-globulin f raction; the alpha-1-fraction was low but within the normal range. In a biopsy of the liver an alpha-1-antitrypsin deficiency was diagnosed histologically. This diagnosis was proven by measurement of a decrease d alpha-1-antitrypsin level (126 mg/dl). A PiMZ phenotype was found in the patient. Thus an alpha-1-antitrypsin deficiency cannot be detecte d for a long time in case of normal liver enzymes and atypical changes in the serum-electrophoresis.