[The role of liver in muscular dystrophy]

Minerva Pediatr. 1993 Jan-Feb;45(1-2):15-9.
[Article in Italian]

Abstract

Epidemiological or anamnestical data may either help or confuse the differential diagnosis of various diseases mainly characterized by asymptomatic hypertransaminasemia. Occasional finding of transaminase elevation may lead to suppose chronic or persistent hepatopathy, particularly when the patient seems to be asymptomatic and presents anamnestic data suggesting intoxication, acquired infection from blood derivatives, origin from geographic areas with high prevalence of viral hepatitis. However, the true existence of hepatic damage, concurrent to a myopathy, may be also related to the primitive diseases. There is evidence, in fact, that in the presence of muscular dystrophy, a disease caused by structural defects of muscular membranes, also hepatocytes show ultrastructural defects. The present work reports the cases of 5 children, hospitalized at the 1st Clinic of Infectious Diseases of the University of Genoa, affected by persistent hypertransaminasemia and showing anamnestical data suggesting hepatitis; histological findings of hepatitis were effectively shown in 3 patients after needle biopsy. All patients proved to be affected by muscular dystrophy. Hepatic damage results cannot be correlated to known causes of hepatopathy. During disease courses heralded by asthenia and hypertransaminasemia, differential diagnosis must take into account non-hepatic diseases, like muscular dystrophy. Although this disease mainly affects the muscle, also the liver seems to be involved, as suggested by histological changes found in some patients.

Publication types

  • Case Reports
  • English Abstract

MeSH terms

  • Adolescent
  • Amidinotransferases / analysis
  • Child
  • Child, Preschool
  • Diagnosis, Differential
  • Hepatitis A / complications
  • Hepatitis A / diagnosis*
  • Hepatitis A / enzymology
  • Hepatomegaly / complications
  • Hepatomegaly / diagnosis
  • Hepatomegaly / physiopathology
  • Humans
  • Liver Diseases / complications
  • Liver Diseases / diagnosis
  • Liver Diseases / physiopathology
  • Male
  • Muscular Dystrophies / complications
  • Muscular Dystrophies / diagnosis*
  • Muscular Dystrophies / enzymology
  • Splenomegaly / complications
  • Splenomegaly / diagnosis
  • Splenomegaly / physiopathology

Substances

  • Amidinotransferases