hormonal and metabolic disorders
Acute Intermittent Porphyria
Treatment of Acute Intermittent Porphyria
Treatment of the acute attack is identical for all the acute porphyrias. Heme given by vein Dextrose People who have attacks of acute intermittent porphyria are often hospitalized for treatment of severe symptoms. People with severe attacks are treated with heme given by vein. Blood and urine levels of delta-aminolevulinic acidand porphobilinogen are promptly lowered and symptoms subside, usually within several days. If treatment is delayed, recovery takes longer, and some nerve damage may be permanent. Dextrosegiven by mouth (or by vein if people are vomiting) can also be beneficial, particularly in people whose attacks are brought on by a low-calorie, low-carbohydrate diet, but these measures are less effective than heme. Pain can be controlled with medication (such asacetaminophenor opioids). Nausea, vomiting, anxiety, and restlessness are treated with a phenothiazine-type medication for a short time.Ondansetronmay also be given for nausea. Insomnia may be treated with chloral hydrate or low doses of a benzodiazepine but not a barbiturate. An overly full bladder may be treated by draining the urine with a catheter. Doctors ensure that people do not take any of the medications known to precipitate an attack, and—if possible—address other factors that may have contributed to the attack. Treatment of seizures is problematic, because many commonly used antiseizure medications would worsen an attack.Levetiracetamis an antiseizure medication that appears to be safe to use. Beta-blockers may be used to treat rapid heart rate and high blood pressure. People with evidence of kidney damage are usually referred to a kidney specialist (nephrologist). Because the risk of liver cancer is high among people with acute intermittent porphyria, people who are older than 50 are screened for liver cancer at least once per year. Liver Transplantation can cure acute intermittent porphyria. Doctors consider transplantation for people with poor quality of life and risk of permanent kidney or nervous system damage because of severe recurrent attacks. Some people may also need 