title: Adrenal insufficiency due to isolated adrenocorticotropin deficiency complicated by autosomal recessive polycystic kidney disease.
abstract: We describe a 29-old-year Japanese man with autosomal recessive polycystic kidney disease who was frequently hypoglycemic. Insulinoma as a cause of hypoglycemia was denied because the ratio of plasma immunoreactive insulin to glucose was low. Adrenal insufficiency was diagnosed because of the low urinary excretion of 17-hydroxycorticosteroids, and both blunted responses of plasma cortisol to an intravenous injection of adrenocorticotropin and of plasma adrenocorticotropin to an intravenous injection of human corticotropin releasing hormone were observed, although basal plasma concentrations of cortisol and adrenocorticotropin were normal. The elusion profile of plasma sample from our patient chromatographed on a Sephadex G-75 column showed two peaks of (1-39)-ACTH and beta-lipotropin, with no evidence of high molecular weight form of ACTH. The plasma concentrations of thyroid stimulating hormone and growth hormone were within the normal range. These findings indicated that this patient with autosomal recessive polycystic kidney disease was associated with adrenal insufficiency due to isolated adrenocorticotropin deficiency.
authors.affiliation: Hemodialysis Unit, Hamamatsu University School of Medicine, Handayama, Hamamatsu, Japan. katsuh@hama-med.ac.jp
authors.forename: Katushiko
authors.initials: K
authors.lastname: Yonemura
authors.forename: Hideo
authors.initials: H
authors.lastname: Yasuda
authors.forename: Yoshihide
authors.initials: Y
authors.lastname: Fujigaki
authors.forename: Yutaka
authors.initials: Y
authors.lastname: Oki
authors.forename: Akira
authors.initials: A
authors.lastname: Hishida
mesh_terms: D015065:17-Hydroxycorticosteroids / Q000652:urine; D000309:Adrenal Insufficiency / Q000175:diagnosis / Q000209:etiology* / Q000378:metabolism*; D000324:Adrenocorticotropic Hormone / Q000097:blood / Q000172:deficiency*; D000328:Adult; D015415:Biomarkers / Q000097:blood; D001786:Blood Glucose / Q000378:metabolism; D002761:Cholangitis / Q000175:diagnosis / Q000378:metabolism; D003092:Colitis / Q000175:diagnosis / Q000378:metabolism; D003937:Diagnosis, Differential; D013006:Growth Hormone / Q000097:blood; D006801:Humans; D007003:Hypoglycemia / Q000175:diagnosis / Q000209:etiology / Q000378:metabolism; D007564:Japan; D008297:Male; D017044:Polycystic Kidney, Autosomal Recessive / Q000175:diagnosis / Q000209:etiology* / Q000378:metabolism*; D013972:Thyrotropin / Q000097:blood; D014057:Tomography, X-Ray Computed; D018084:Ultrasonography, Interventional
chemical_list: D015065:17-Hydroxycorticosteroids; D015415:Biomarkers; D001786:Blood Glucose; D000324:Adrenocorticotropic Hormone; D013972:Thyrotropin; D013006:Growth Hormone