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Re: GYN: Porphyrias and OC'sFrom: art fougner, md (evsono@pipeline.com)Wed Jun 17 14:26:55 1998
well - the literature yields its usual mixed bag of results. apparently we need to know more about which of the porphyrias you are dealing with. oral contraceptives either precipitate or prevent certain porphyrias. additionally, there is one case report of a woman's developing porphyria on withdrawal of oc's. go figure. see below: J Am Acad Dermatol 1994 Aug;31(2 Pt 2):390-392 Porphyria cutanea tarda: pregnancy versus estrogen effect. Urbanek RW, Cohen DJ Department of Medicine, Staten Island University Hospital, NY 10305. We describe the worsening of porphyria cutanea tarda in a young woman while she was taking oral contraceptives. However, she did not have an exacerbation during two pregnancies. We conclude that estrogens produced during pregnancy do not exert the same effect as orally administered medications that contain estrogen. The pronounced effect of oral ethinyl estradiol on the liver may be attributed to its first-pass effect on that organ. Horm Metab Res 1995 Aug;27(8):379-383 Hormonal oral contraceptives, urinary porphyrin excretion and porphyrias. Gross U, Honcamp M, Daume E, Frank M, Dusterberg B, Doss MO Abteilung fur Klinische Biochemie, Klinikum der Philipps-Universitat, Marburg, Germany. The influence of hormonal oral contraceptives on the urinary porphyrin excretion of 40 healthy females has been studied. Two different hormonal oral contraceptives (combinations of gestoden or desogestrel, respectively, and ethinylestradiol) were applied for half a year. In each case twenty women received one of these two combinations. Porphyrin precursors delta-aminolevulinic acid and porphobilinogen were normal in all subjects as well as the mean of uroporphyrin and coproporphyrin. One healthy female developed a mild secondary coproporphyrinuria. In this case coproporphyrin isomer I was slightly enhanced and isomer III slightly lowered. Furthermore it could be shown that three females with repeated premenstrual clinical expression of an acute hepatic porphyria (acute intermittent porphyria and hereditary coproporphyria) could be treated successfully with a hormonal oral contraceptive or other exogenous hormones to stabilize the latent, subclinical phase of the disease. PMID: 7590628, UI: 96049914 Am J Hematol 1986 Sep;23(1):69-75 Porphyria cutanea tarda in a patient with acute leukemia. Dutcher JP, Fisher M, Spivack M, Wiernik PH A 20-year-old woman with acute nonlymphocytic leukemia in remission was treated postremission with intermittent courses of intensive chemotherapy. She required blood and platelet transfusions following therapy, and oral contraceptives were used to suppress her menses while she was thrombocytopenic. One and one-half years after treatment began, she developed blisters on her hands, hypertrichosis, and dark urine. The diagnosis of porphyria cutanea tarda was made. This report discusses the possible precipitating cause(s) and the subsequent treatment of both her porphyria and her leukemia. PMID: 3740055, UI: 86292942 Arch Dermatol Res 1985;278(1):13-16 Hereditary uroporphyrinogen-decarboxylase deficiency predisposing porphyria cutanea tarda (chronic hepatic porphyria) in females after oral contraceptive medication. Sixel-Dietrich F, Doss M Porphyria cutanea tarda (PCT) was diagnosed in 27 women aged 23-48 years (mean, 35 years) who had been under oral-hormonal-contraceptive medication for 1-18 years, in 3 women under substitutional estrogen treatment in the menopause, and in 2 men aged 65 and 76 years after estrogen treatment of prostatic carcinoma. In all patients, total urinary porphyrin excretion was elevated, with an average uro- and heptacarboxyporphyrin predominance of 88%, thus proving PCT. Of the patients, 84% showed a significant decrease of erythrocyte uroporphyrinogen-decarboxylase (UD; EC 4.1.1.37) activity to approximately 50% of control levels suggesting a hereditary predisposition for the development of a chronic hepatic porphyria. Estrogens and alcohol are capable of reducing hepatic UD activity. Women with hereditary red cell UD deficiency may be regarded as predisposed to PCT when under estrogen intake, especially in combination with the potentiating influence of alcohol and chronic liver disease. Normal erythrocyte UD values in patients with additive alcohol consumption may implicate a stronger inhibitory effect for alcohol on UD, suggesting a merely toxic form of chronic hepatic porphyria. PMID: 4096525, UI: 86157750 Zentralbl Gynakol 1983;105(8):527-531 [Possible side effect of hormonal contraception: porphyria cutanea tarda]. [Article in German] Zaumseil RP, Fiedler H The increased incidence of cases with porphyria cutanea tarda (p.c.T.) in women is correlated to preceding hormonal contraception. This is our reason to report on 16 females suffering from p.c.t. in the last years (1975 to 1979), including 8 females younger than 40 years affected after use of oral contraceptives for a long time. Stopping the hormonal anticonception represents the most important therapeutic measure. This is a good supposition to prevent recidives and to cure definitely. Cure is demonstrated in a young woman by normal pregnancy with a healthy boy. PMID: 6868877, UI: 83251798 Can Med Assoc J 1979 Apr 7;120(7):803-807 Porphyria cutanea tarda: clinical and laboratory features. Sweeney GD, Jones KG Eleven patients with porphyria cutanea tarda were studied. Biochemical confirmation of the clinical diagnosis required only determination of the total urine porphyrin concentration in a sample of urine voided on rising in the morning. The patients were divided for convenience of discussion into four groups differing in age, sex and etiologic factors. Of the six patients in whom a liver biopsy was done one was shown to have micronodular cirrhosis. Except for a modest elevation in the serum glutamic oxaloacetic transaminase values when the patients were first seen, no evidence was found for liver disease apart from the presence of porphyria cutanea tarda. One patient recovered solely by abstaining from alcohol consumption. Five patients underwent phlebotomy; their iron stores had been found to be between 2 and 3 g. Decreasing urine porphyrin values correlated well with decreasing serum ferritin values during the course of phlebotomy. Porphyria cutanea tarda, which is due to a deficiency of uroporphyrinogen decarboxylase, is manifested in association with alcohol abuse, estrogen therapy, exposure to chlorinated hydrocarbons or increased tissue iron stores, or a combination of these factors. Although relatively uncommon, this condition raises important and unresolved issues regarding the hepatotoxicity of alcohol, estrogens, chlorinated hydrocarbons and iron. PMID: 427687, UI: 79146659 J Int Med Res 1978;6(4):255-256 Acute intermittent porphyria on withdrawal of oral contraceptives. Gerlis LS A rare presentation of acute Intermittent Porphyria is described, in which withdrawal of an oral contraceptive (Gynovlar 21) provoked the first acute attack. PMID: 689289, UI: 79003975 J Reprod Med 1975 Dec;15(6):214-224 Unusal signs and symptoms associated with oral contraceptive medication. Koide SS, Lyle KC A review of medical literature concerning case history reports of adverse clinical manifestations of hormonal contraceptives is presented. While extremely rare, these side-effects often present with obscure etiology, and the physician is cautioned to be aware of their occurrence. Publication Types: Review PMID: 1104827, UI: 76071799 it is an interesting illness to be sure. Art
-- art fougner, md SonoScan/Genetic Sciences forest hills, ny evsono@pipeline.com
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