ECE2016 Eposter Presentations Clinical case reports - Thyroid/Others (71 abstracts)
Improvement in acute intermittent porphyria symptoms after bilateral oophorectomy: a case report
Jekaterina Nagaiceva 1, , Natalija Kaplja 1, , Ilze Puzaka 1, , Liva Steina 3 , Natalija Fokina 1 , Maija Kokare 1 , Sandra Steina 1 , Edvins Miklasevics 3 & Valdis Pirags 1,
1Pauls Stradins University Hospital, Riga, Latvia; 2University of Latvia, Riga, Latvia; 3Riga Stradins University, Riga, Latvia.
Acute intermittent porphyria (AIP) is an uncommon monogenic autosomal dominant disorder with a defect in the haem biosynthesis pathway at the level of the enzyme porphobilinogen deaminase. The disease has low penetrance; most patients could stay asymptomatic throughout life. Clinical manifestation depends on various precipitants, which increase hepatic haem production and cytochrome activity. We report a case of a female patient who had regular moderate-to-severe cyclic AIP attacks, which started usually during the premenstrual period and ended after the menstruation. During the attacks she was treated with intravenous dextrose load, gabapentin, tramadol and intravenous hemin, which could not be used regularly due to high costs. Combined oral contraceptive drug therapy was unsuccessful. Gonadotropin releasing hormone agonist treatment was impossible since it was too expensive for the patient. Chronic complications progressed, including sensory, motor and autonomic neuropathy and chronic kidney disease. After the last severe AIP attack, a laparoscopic bilateral oophorectomy was performed. The surgery was successful with an uneventful postoperative recovery. Four years have passed, during this time AIP attacks have become milder and infrequent with one attack-free year during the last two years. The improvement in patient’s quality of life is evident. Endogenous sex steroid hormones, mostly progesterone, play an important role in precipitating AIP attacks in women. Our case shows that the choice of therapy needs to be made individually for each patient. During the last forty years, only a few similar cases have been reported.
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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