Volume 72 - 2009 - Fasc.3 - Letters
Hepatocellular carcinoma in an adult patient with type IV glycogen storage disease
Type IV glycogen storage disease (type IV GSD), also known as Andersen disease or amylopectinosis, is a rare autosomal-recessive disorder caused by deficiency of the glycogen branching enzyme (1). The classic form is characterized by progressive hepatic fibrosis resulting in hepatosplenomegaly and failure to thrive and death by the age of 5 years. Few patients do not develop hepatic failure and survive to adulthood (2). Here we report a case of hepatocellular carcinoma (HCC) in a young adult with GSD IV and chronic liver disease following a 20 year period with no apparent disease progression.
Abdominal wall scar endometriosis. A report of two cases with emphasis on diagnostic strategies
We report two cases of young women with intermit- tent abdominal wall pain, and discuss the relevance of imaging techniques and the treatment of this disorder.
We present two patients, both young women (repro- ductive age), who were referred to a gastro-enterologist because of pain "in the abdomen". Since the pain was cyclic, they were first seen by their gynaecologist, who in both cases excluded "pain of gynaecological origin". Both patients complained of a shooting, burning pain in the left (patient A) and right lower quadrant (patient B). The pain worsened after exercise. On clinical examina- tion of the abdomen, we palpated some nodules in the abdominal wall of patient A, however in patient B, we did not palpate a mass. In our opinion this could be due to the narrow circumscribed palpatory tenderness which elicited rectus abdominis muscle contraction during pal- pation. Carnett's test (i.e. elevation of both feet with extended legs) provoked spontaneous and palpatory pain [left (A) and right (B) lower quadrant].
