cosyntropin and Hyperplasia

To describe the current indications for adrenal vein sampling (AVS), variability in institutional protocols for performing the procedure, shortage of expert interventional radiologists trained in this procedure, pitfalls in technique and strategies to improve success. A major emphasis of the review will focus on the interpretation of the AVS results.. Published protocols for performance of the AVS procedure and variability in the diagnostic criteria differentiating aldosterone-producing adenoma from bilateral adrenal hyperplasia vary significantly. Inability to catheterize the right adrenal vein is the major reason for technical failure of AVS. Preplanning computed tomography, stat intraprocedural cortisol levels and cone-beam computed tomography are helpful in identifying the right adrenal vein. The administration of adrenocorticotropic hormone stimulation during AVS varies significantly between different studies.. More interested interventional radiologists need to acquire the necessary technical expertise for AVS due to increased demand for the procedure, which is the diagnostic reference standard for primary aldosteronism. Unresolved issues include variability in the AVS procedure protocol, use of adrenocorticotropic hormone stimulation and standardization of the interpretation of the results. Despite all these variables, many different approaches still appear to be clinically successful, as indicated by the extensive published reports.

Topics: Adenoma; Adrenal Glands; Adrenocorticotropic Hormone; Aldosterone; Catheterization; Clinical Competence; Cosyntropin; Diagnosis, Differential; Humans; Hydrocortisone; Hyperplasia; Pituitary Neoplasms; Radiology; Veins; Workforce

A 60-year-old woman presented with diffuse scalp alopecia, hirsutism, and clitorimegaly, and the mean serum testosterone levels were greater than 200 ng/dL. Findings on computed tomography of both adrenal glands were normal. After bilateral oophorectomy, a unique histological picture consisting of diffuse stromal Leydig cell hyperplasia was found. Reinke crystals were present, but neither hilus cell hyperplasia nor stromal hyperthecosis was noted. Sequencing of the 11 exons of the gene for the luteinizing hormone receptor revealed no abnormality. Relevant data suggest that treatment of the postmenopausal woman with hyperandrogenism and virilization is bilateral laparoscopic oophorectomy if she has no pronounced ovarian enlargement or adrenal tumor on imaging. In this setting, an intensive endocrine evaluation or a search for metastatic disease seems to be unnecessary.

Topics: Cosyntropin; Diagnosis, Differential; Female; Gonadal Steroid Hormones; Humans; Hyperandrogenism; Hyperplasia; Leydig Cells; Male; Middle Aged; Postmenopause; Virilism

One to 2% of hyperandrogenic women demonstrate a 17-hydroxyprogesterone (17-HP) level greater than 36.3 nmol/L (1200 ng/dL) after acute ACTH-(1-24) adrenal stimulation, consistent with 21-hydroxylase (21-OH) deficient late-onset adrenal hyperplasia (LOAH). The following study was undertaken to endocrinologically and genetically define hyperandrogenic patients with an exaggerated 17-HP response to ACTH stimulation, and which do not represent LOAH. Of 265 consecutive patients suffering from hirsutism and/or hyperandrogenic oligomenorrhea, 23 (8.7%) demonstrated a 17-HP level 30 min post stimulation greater than 9.6 nmol/L or 316 ng/dL (the upper 95th percentile in 41 eumenorrheic nonhirsute healthy control women). Seven patients or five separate families (1.8% of total) demonstrated poststimulation 17-HP levels consistent with LOAH. Of the remaining 16 patients, the net increment in 17-HP (delta 17-HP0-30) was within normal limits in seven (2.6%) and these women were assumed to have a normal 17-HP adrenocortical response superimposed on an elevated basal level of nonadrenal (e.g. ovarian) origin. In the remaining nine hyperandrogenic patients (3.4%) various abnormalities of adrenal response were noted in all but one patient, consistent with adrenal hyper-responsiveness. One patient demonstrated an 11-deoxycortisol poststimulation level greater than 3-fold the upper 95th percentile of normal, consistent with 11-hydroxylase LOAH and was excluded from further study. Six of these women were available for further genetic characterization, all Caucasian and unrelated. Three were heterozygotes for HLA-B14, three for B40, and one for B35 antigen, HLA-types associated with the inheritance of 21-OH deficiencies. Although, normally there are two 21-OH genes (a pseudogene and a functional gene) present in a 1:1 ratio, we have previously reported a high frequency of 21-OH gene ratio abnormalities in LOAH. All but one of our patients demonstrated an abnormal 21-OH gene ratio. In conclusion, 3.4% of our hyperandrogenic population demonstrated an exaggerated 17-HP increment after ACTH stimulation, not consistent with LOAH or increased extraadrenal 17-HP production. The increased prevalence of HLA alleles known to be linked to inherited defects of 21-OH function and the increased frequency in 21-OH gene ratio abnormalities suggest that a majority of these individuals may be carriers for these genetic disorders. However, the adrenocortical abnormalities noted were more consi

Topics: 17-alpha-Hydroxyprogesterone; Adrenal Cortex Hormones; Adrenal Glands; Adult; Androgens; Cosyntropin; Female; Genes; Hirsutism; Humans; Hydroxyprogesterones; Hyperplasia; Oligomenorrhea; Steroid 21-Hydroxylase

Topics: Adrenal Cortex; Adrenocorticotropic Hormone; Aldosterone; Animals; Body Weight; Corticosterone; Cosyntropin; Delayed-Action Preparations; Dose-Response Relationship, Drug; Female; Hyperplasia; Injections, Subcutaneous; Mitosis; Rats