[Hepatic lesion in patients with hepatocerebral dystrophy].
OBJECTIVE
To study hepatic affection in hepatocerebral dystrophy (HCD) regarding clinical course and duration of the disease.


METHODS
The records on 63 HCD patients were studied retrospectively as to early clinical symptoms, onset dynamics, biochemical tests for hepatic function, morphological changes of the liver, immune and cytokine status, markers of hepatotropic viruses.


RESULTS
Six variants of HCD course were identified: hepatic (24 patients), endocrine (17 patients), neurological (12 patients), psychoemotional (5 patients), hemolytic (3 patients), renal (1 patient). Early hepatitis and hepatic cirrhosis progress were characteristic for the hepatic variant. The other variants were characterized by hepatic fibrosis, lipofuscinosis, fat dystrophy of the liver. Markers of viral hepatitides B, C, G, TT were detected primarily in patients with a hepatic variant of HCD. A moderate rise of aminotransferases, immunoglobulins, cytokines level indicate weak inflammation in hepatic tissue. Disorders in proteins synthesis, hepatic detoxication function reflect changes in the microsomal system and endoplasmic network.


CONCLUSIONS
Hepatic lesions in HCD are different. Clinical, biochemical, morphological activity and immune status affections were most severe in patients with hepatic and endocrine variants of HCD, being the least severe in neurological, psychoemotional and hemolytic variants. Hepatotropic viruses in some patients deteriorate hepatic lesion caused by copper imbalance.
Sukhareva
G V
GV
Tsaregorodtseva
T M
TM
Petrakov
A V
AV
Nilova
T V
TV
Sil'vestrova
S Iu
SIu
Lazebnik
L B
LB
D000368:Aged; D015415:Biomarkers / Q000097:blood; D016207:Cytokines / Q000097:blood; D018450:Disease Progression; D005234:Fatty Liver / Q000097:blood / Q000209:etiology / Q000473:pathology; D005260:Female; D005500:Follow-Up Studies; D006505:Hepatitis / Q000097:blood / Q000209:etiology* / Q000473:pathology; D006527:Hepatolenticular Degeneration / Q000097:blood / Q000150:complications* / Q000473:pathology; D006801:Humans; D007136:Immunoglobulins / Q000097:blood; D008103:Liver Cirrhosis / Q000097:blood / Q000209:etiology* / Q000473:pathology; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012189:Retrospective Studies; D012720:Severity of Illness Index; D013997:Time Factors; D000637:Transaminases / Q000097:blood
D015415:Biomarkers; D016207:Cytokines; D007136:Immunoglobulins; D000637:Transaminases