Ectopic adrenal adenoma causing gross hematuria: Steroidogenic enzyme profiling and literature review

Peer Reviewedhttps://deepblue.lib.umich.edu/bitstream/2027.42/149375/1/iju512068.pdfhttps://deepblue.lib.umich.edu/bitstream/2027.42/149375/2/iju512068_am.pd

Human Adrenocortical Remodeling Leading to Aldosterone-Producing Cell Cluster Generation

Background. The immunohistochemical detection of aldosterone synthase (CYP11B2) and steroid 11β-hydroxylase (CYP11B1) has enabled the identification of aldosterone-producing cell clusters (APCCs) in the subcapsular portion of the human adult adrenal cortex. We hypothesized that adrenals have layered zonation in early postnatal stages and are remodeled to possess APCCs over time. Purposes. To investigate changes in human adrenocortical zonation with age. Methods. We retrospectively analyzed adrenal tissues prepared from 33 autopsied patients aged between 0 and 50 years. They were immunostained for CYP11B2 and CYP11B1. The percentage of APCC areas over the whole adrenal area (AA/WAA, %) and the number of APCCs (NOA, APCCs/mm2) were calculated by four examiners. Average values were used in statistical analyses. Results. Adrenals under 11 years old had layered zona glomerulosa (ZG) and zona fasciculata (ZF) without apparent APCCs. Some adrenals had an unstained (CYP11B2/CYP11B1-negative) layer between ZG and ZF, resembling the rat undifferentiated cell zone. Average AA/WAA and NOA correlated with age, suggesting that APCC development is associated with aging. Possible APCC-to-APA transitional lesions were incidentally identified in two adult adrenals. Conclusions. The adrenal cortex with layered zonation remodels to possess APCCs over time. APCC generation may be associated with hypertension in adults

Cortisol overproduction results from DNA methylation of CYP11B1 in hypercortisolemia

金沢大学医薬保健研究域医学系Adrenocortical hormone excess, due to primary aldosteronism (PA) or hypercortisolemia, causes hypertension and cardiovascular complications. In PA, hypomethylation of aldosterone synthase (CYP11B2) is associated with aldosterone overproduction. However, in hypercortisolemia, the role of DNA methylation of 11β-hydroxylase (CYP11B1), which catalyzes cortisol biosynthesis and is highly homologous to CYP11B2, is unclear. The aims of our study were to determine whether the CYP11B1 expression was regulated through DNA methylation in hypercortisolemia with cortisol-producing adenoma (CPA), and to investigate a possible relationship between DNA methylation and somatic mutations identified in CPA. Methylation analysis showed that the CYP11B1 promoter was significantly less methylated in CPA than in adjacent unaffected adrenal tissue and white blood cells. Furthermore, in CPA with somatic mutations in either the catalytic subunit of protein kinase A (PRKACA) or the guanine nucleotide-binding protein subunit alpha (GNAS) gene, the CYP11B1 promoter was significantly hypomethylated. In addition, DNA methylation reduced CYP11B1 promoter activity using a reporter assay. Our study results suggest that DNA methylation at the CYP11B1 promoter plays a role in the regulation of CYP11B1 expression and cortisol production in CPA, and that somatic mutations associated with CPA reduce DNA methylation at the CYP11B1 promoter. © 2017 The Author(s)

A Feasible Technique in Laparoscopic Excision for Juvenile Cystic Adenomyosis: A Case Report, Literature Review, and Surgical Video

Background: Juvenile cystic adenomyosis (JCA) is a rare uterine lesion. We present the case of a young woman who was diagnosed with JCA and subsequently managed with laparoscopic cyst removal with sharp and blunt dissection. Moreover, we provide a literature review and a surgical video. Case: A 22-year-old nulliparous woman presented with severe dysmenorrhea and was assessed using contrast-enhanced abdominal computed tomography, transvaginal ultrasonography and pelvic magnetic resonance imaging, and diagnosed with a cystic lesion on the left side of the myometrium. She underwent laparoscopic cyst excision and uterine reconstruction. Histology was suggestive of JCA. The dysmenorrhea resolved postoperatively. Conclusion: Surgical resection is the first choice of treatment for cystic adenomyosis, and a laparoscopic approach using scissor forceps is effective

Methods for assessing change in brain plasticity at night and psychological resilience during daytime between repeated long-duration space missions

Abstract This study was designed to examine the feasibility of analyzing heart rate variability (HRV) data from repeat-flier astronauts at matching days on two separate missions to assess any effect of repeated missions on brain plasticity and psychological resilience, as conjectured by Demertzi. As an example, on the second mission of a healthy astronaut studied about 20 days after launch, sleep duration lengthened, sleep quality improved, and spectral power (ms2) co-varying with activity of the salience network (SN) increased at night. HF-component (0.15–0.50 Hz) increased by 61.55%, and HF-band (0.30–0.40 Hz) by 92.60%. Spectral power of HRV indices during daytime, which correlate negatively with psychological resilience, decreased, HF-component by 22.18% and HF-band by 37.26%. LF-component and LF-band, reflecting activity of the default mode network, did not change significantly. During the second mission, 24-h acrophases of HRV endpoints did not change but the 12-h acrophase of TF-HRV did (P < 0.0001), perhaps consolidating the circadian system to help adapt to space by taking advantage of brain plasticity at night and psychological resilience during daytime. While this N-of-1 study prevents drawing definitive conclusions, the methodology used herein to monitor markers of brain plasticity could pave the way for further studies that could add to the present results