Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0038220 (status epilepticus)
7,272 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 22-years old, 55 kg female patient in the twelfth week of pregnancy developed neuropsychiatric syndromes and in the following status epilepticus. Raised porphyrines and porphyrine precursors were found in the patient's urine. Despite intravenous glucose infusions and appropriate medication no reduction in seizure-frequency and neuropsychiatric syndromes was observed. An abortion was induced. After the interruption and starting of haem arginate therapy, seizure activity stopped and porphyrine precursors returned to normal levels, and after 6 weeks the patient was discharged in excellent clinical condition. This report describes a status epilepticus caused by acute hepatic porphyria, triggered by pregnancy, in a 22-years old woman. To our knowledge this is the first report of induced abortion as successful treatment in acute hepatic porphyria induced status epilepticus.
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PMID:Refractory status epilepticus due to acute hepatic porphyria in a pregnant woman: induced abortion as the sole therapeutic option? 1679 97

A 22-year-old para I/gravida II developed psychiatric symptoms at 8 weeks of gestation. Subsequently neurological symptoms with seizures developed leading to a status epilepticus with continuing seizures at week 14. Anticonvulsive therapy had little effect in alleviating the seizures and the condition of the patient rapidly deteriorated. A sudden reddening of her urine lead to the diagnosis of acute hepatic porphyria confirmed by laboratory tests. After extensive discussion with the patient's family it was decided to terminate the pregnancy at week 16. Within hours after pregnancy termination the seizures stopped and the patient recovered without any neurological deficits. Acute hepatic porphyria can be triggered by pregnancy and usually presents with gastrointestinal symptoms and personality changes. In its rare neurological manifestation it can lead to untreatable convulsions which leave no option but to terminate the pregnancy.
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PMID:Unusual manifestation of acute hepatic porphyria in pregnancy. 1713 71