Elevation of hormone-binding globulins in acute intermittent porphyria.
Sex hormone-binding globulin (SHBG), thyroxine-binding globulin (TBG) and cortisol-binding globulin (CBG) were measured in plasma of 26 patients with acute intermittent porphyria (AIP). Twelve patients had clinically manifest disease and all had elevated SHBG levels. All but one of 14 patients with latent porphyria had normal SHBG levels. TBG was elevated in 9 of the patients with clinically manifest porphyria and CBG elevated in three. Levels of TBG and CBG were either normal or only slightly elevated in those with latent porphyria. In a prospective study of 30 attacks of AIP in 7 patients, SHBG levels fell between admission and discharge, the fall being significant in the group of 21 attacks treated with haem arginate (p less than 0.001). Our findings suggest that a close correlation exists between elevated SHBG and clinical expression of AIP.
Herrick
A L
AL
University Department of Medicine, Gardiner Institute, Western Infirmary, UK.
McColl
K E
KE
Wallace
A M
AM
Moore
M R
MR
Goldberg
A
A
D000293:Adolescent; D000328:Adult; D000622:Aminolevulinic Acid / Q000652:urine; D001120:Arginine / Q000627:therapeutic use; D002352:Carrier Proteins / Q000097:blood*; D006418:Heme / Q000627:therapeutic use; D006801:Humans; D006854:Hydrocortisone / Q000097:blood*; D008107:Liver Diseases / Q000097:blood* / Q000188:drug therapy / Q000652:urine; D011162:Porphobilinogen / Q000652:urine; D011164:Porphyrias / Q000097:blood* / Q000188:drug therapy / Q000652:urine; D011446:Prospective Studies; D012738:Sex Hormone-Binding Globulin / Q000032:analysis*; D013739:Testosterone / Q000097:blood; D013975:Thyroxine-Binding Proteins / Q000032:analysis*
D002352:Carrier Proteins; D012738:Sex Hormone-Binding Globulin; D013975:Thyroxine-Binding Proteins; C027091:cortisol binding globulin; D013739:Testosterone; D006418:Heme; D011162:Porphobilinogen; D000622:Aminolevulinic Acid; D001120:Arginine; C048849:heme arginate; D006854:Hydrocortisone