Wilson's disease presenting as haemolytic anaemia and its successful treatment with penicillamine and zinc.

Haemolysis is an uncommon first manifestation of Wilson's disease. We describe a young woman who presented with episodic haemolysis and abnormal liver functions; the diagnosis of Wilson's disease was not made until nine months later. She responded well to a combination of penicillamine and zinc. This report underscores the importance of considering Wilson's disease as a cause in a patient with haemolysis of uncertain aetiology, since the disease can be successfully treated in the early stages. the mechanism of oxidative damage to erythrocytes by the excessive copper and the present role of zinc therapy are also discussed.

Psychosocial aspects of non-ulcer dyspepsia.

A group of 23 non-ulcer dyspepsia patients were compared with controls drawn from relatives of psychiatric outpatients. The level of hostility in both groups was high, but not significantly different. There was also no significant difference between the 2 groups on measures of extroversion, neuroticism, psychoticism and lie scores, but the ulcer group was significantly more depressed and more were diagnosed as suffering from a neurotic depression and generalized anxiety disorder.

Hepatocellular carcinoma--a case series of 104 patients.

The records of one hundred and four patients with confirmed hepatocellular carcinoma seen over a two year period at the National University Hospital were analysed to elucidate the clinical features of our local patients and to assess their response to various therapeutic modalities. Chinese males were over-represented with a peak frequency in the sixth to eighth decade of life. Seventy-five percent of the patients were HBsAg positive and at least 88% had evidence of previous Hepatitis B infection. Ninety-one percent were symptomatic at presentation with pain being the most common symptom. Hepatomegaly with features of cirrhosis were the main physical findings. Seventy percent of the patients presented within three months after the onset of symptoms. The majority of patients had stage II or III disease at diagnosis. Twenty percent of patients had normal alpha-fetoprotein levels. Chemotherapy did not appear to show a survival benefit. Curative surgical resection was feasible in about 10% of patients and it remains the only chance for long term survival. There is an urgent need to identify more effective drugs or other modalities to treat this common and rapidly fatal malignancy. Identification of high risk patients should prompt screening with both serum alpha-fetoprotein and ultrasonography of the liver.

Zollinger-Ellison syndrome--report of four cases and review of literature.

Four patients with Zollinger-Ellison Syndrome (ZES) are presented to highlight the difficulties in the recognition, diagnosis and management of this rare disease. The presentation of ZES is usually indistinguishable from ordinary peptic ulcer disease and in those patients with symptoms not related to peptic ulcer, i.e., diarrhoea, as their main complaint, the diagnosis is often not even considered. A high index of suspicion is required, however, in patients with recurrent ulcers, multiple ulcers and in those with resistant or rapidly relapsing ulcers after conventional therapy. A presumptive diagnosis can be made by the demonstration of grossly elevated fasting serum gastrin levels combined with a secretin stimulation test in doubtful cases. The main problem is the location of the gastrin-secreting tumour which is usually pancreatic but often too small to be detected by currently available techniques. Histamine H2-receptor antagonists in high doses are effective in controlling the gastric acid hypersecretion which is chiefly responsible for the morbidity and mortality in ZES. They provide the treatment of choice in patients where the tumour cannot be located, though every attempt should be made to do this as surgery is the treatment of choice for this invariably malignant tumour. Total gastrectomy is now reserved for those patients in whom medical therapy has failed. The role of chemotherapy in metastatic disease has yet to be established.