Cases reported "Acute Coronary Syndrome"

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1/3. A preventable cause of acute abdomen.

    Haemoperitoneum is an extremely rare presentation of hepatocellular carcinoma in the industrialised world. We present the first reported case in the UK. In contrast, up to 10% of hepatocellular carcinomas in africa present in this way, the median time between presentation and death being just six weeks. hepatitis b infection at birth and during childhood is the major cause of hepatocellular carcinoma in the developing world. The world health Organisation, UNICEF and the World Bank have all advocated routine hepatitis b vaccination of children. This can reduce the burden of disease in these communities, among people in their productive years of life.
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2/3. Acute loss of the small bowel in a school-age boy. Difficult choices: to sustain life or to stop treatment?

    A 9-year-old boy lost almost all his small bowel after an acute volvulus due to a congenital, but previously unsuspected malrotation. survival using total parenteral nutrition is possible in these cases, but the medical burden is heavy. Small intestinal transplantation was performed for the first time in the netherlands in 2001 and this patient was treated 3 years earlier. The results of bowel transplantation are not as good as in kidney or liver transplantation. A method of Ethical Case Deliberation helped to elucidate the importance of each contribution in the discussion and provided space and a broad basis for decision-making. The parents refused to allow parenteral nutrition to be started because of the bad prospects for quality of life in the future and the medical team, after thorough deliberation with specialists throughout the country, and consultation of the literature, agreed. CONCLUSION: Despite the many different opinions, the parents felt accepted in their refusal of treatment for their son and the team accepted the decision.
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3/3. Acute lead poisoning in inherited porphobilinogen synthase (delta-aminolevulinic acid dehydrase) deficiency.

    The second enzyme of the porphyrin and heme synthesis pathway, porphobilinogen synthase (EC 4.2.1.24), can be lowered due to a hereditary defect or toxic damage. Lead is the most common cause for a toxically lowered activity. We report on a 48-year-old patient with an acute abdominal syndrome, anemia and a grave toxic disorder of porphyrin metabolism which was diagnosed as acute lead intoxication. Although the hematologic findings and porphyrins in the blood, as well as porphyrin precursors and porphyrin excretion in the urine, returned to normal within five months after exacerbation of the acute symptoms the activity of porphobilinogen synthase showed only 50% physiologic activity of healthy subjects six years after the acute syndrome with complete normalization of prophyrin metabolites and lead levels in blood and urine. The influence of alcohol was excluded. enzyme activation by zinc and reactivation by dithiothreitol lies within the normal range and thus contradicts an increased body burden of lead. In connection with findings of a familiar porphobilinogen synthase defect we are most likely dealing with a hereditarily determined enzyme deficiency in this patient which sensitizes him to a lead intoxication.
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