Several related genetic disorders, all characterized by the increased formation of porphyrins or their precursors, are

122, 123

termed porphyria , (Box 40.1). Three subgroups have been identified which all have similar symptomatology: intermittent acute porphyria (IAP), hereditary coproporphyria, and variegate porphyria. Of these, IAP is the most frequently encountered with attacks of colicky abdominal pain that are intermittent, may be associated with environmental exposures, and which can last for days to months. Transmitted as an autosomal dominant disorder with incomplete penetrance, family history may or may not be helpful in the diagnosis. Certain drugs such as barbiturates, benzodiazepines, alcohol, phenytoin, ketamine, etomidate, meprobamate, and corticosteroids have been particularly implicated as "triggers" although use of many of these agents without the precipitation of a crisis has been reported. Constipation, abdominal distension, and profuse vomiting are common. Neurological dysfunction may occur principally due to demyelination effects with emotional disturbance a nonspecific symptom. Urine and blood tests related to porphyria may only be diagnostic during crises. Genetic testing of asymptomatic members in IAP families may allow for avoidance of triggers. Treatment is avoiding known triggers, treating crises with intravenous fluids, hemin, and/or increased carbohydrate intake, and treating pain and nausea with "safe" analgesics/antiemetics. Most opioids are alleged to be nontriggering, a notable exception is the mixed agonist-antagonist pentazocine. Chlorpromazine, promethazine, and droperidol have all been reported to be safe as antiemetics.

Constipation Prescription

Constipation Prescription

Did you ever think feeling angry and irritable could be a symptom of constipation? A horrible fullness and pressing sharp pains against the bladders can’t help but affect your mood. Sometimes you just want everyone to leave you alone and sleep to escape the pain. It is virtually impossible to be constipated and keep a sunny disposition. Follow the steps in this guide to alleviate constipation and lead a happier healthy life.

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