[The clinical characteristics of multi-detector computed tomography of congenital adrenal hyperplasia].

OBJECTIVE: To explore the imaging features of congenital adrenal cortex hyperplasia (CAH). METHODS: A total of 45 patients clinically confirmed as CAH were retrospectively analyzed to investigate the imaging features and strengthening way of the multi-detector-row Computed tomography. RESULTS: The imaging features of all the cases presented as following: 25 with bilateral adrenal hyperplasia, 6 with unilateral adrenal hyperplasia, 6 with adrenal nodular hyperplasia, 2 with adrenal hyperplasia and unilateral solid cystic lesion, 2 with adrenal hyperplasia and double side real cystic lesion, 1 with adrenal hyperplasia and unilateral cystic changes and 3 with normal adrenal. The unilateral or bilateral hyperplasia adrenal could be homogeneously enhanced, while the enhanced performance of other cases was inequitable. CONCLUSIONS: The adrenal imaging features of CAH by multi-detector-row CT are variable, with the bilateral adrenal hyperplasia as the main form, which could be restored to normal morphology after hormone replacement therapy.No regression of the tumor size is observed in cases with adrenal mass. CT scanning combined with clinical manifestation and biochemical examination could facilitate the diagnosis of CAH.

Retrospective comparison of retroperitoneoscopic versus open adrenalectomy for pheochromocytoma.

PURPOSE: We compared the clinical outcomes of retroperitoneoscopic and open adrenalectomy for pheochromocytoma. MATERIALS AND METHODS: Clinical data on 56 patients who underwent retroperitoneoscopic lateral adrenalectomy were retrospectively compared with those on 50 who underwent open adrenalectomy for pheochromocytoma, including patient demographic data, perioperative indexes and clinical outcomes. RESULTS: Demographic data on patients were similar in the 2 groups. In the retroperitoneoscopic group such perioperative indexes were significantly different from those of the open group (each p <0.05), including operative time (mean +/- SD 52 +/- 22 vs 120 +/- 42 minutes), estimated blood loss (74 +/- 34 vs 187 +/- 64 ml), resumption of oral intake (1 vs 2 days), postoperative hospital stay (5.2 +/- 1.7 vs 8.3 +/- 1.8 days), incidence of intraoperative hypertension (17.0% or 9 of 53 patients vs 36.0% or 18 of 50) and number of patients requiring blood transfusion(1.8% or 1 of 53 vs 16.0% or 8 of 50). The incidence of systemic inflammatory response syndrome was much less in the retroperitoneoscopic group (20.8% or 11 of 53 patients vs 42.0% or 21 of 50, p <0.05). However, the duration of systemic inflammatory response syndrome and postoperative complications were similar in the 2 groups (p >0.05). Blood pressure returned to normal 3 months after the operation in 81% of the patients in the retroperitoneoscopic group and in 84% in the open group. During the followup of 5 to 36 months no tumor recurrence and/or metastasis developed. CONCLUSIONS: Compared with open surgery retroperitoneoscopic lateral adrenalectomy for pheochromocytoma is a safe, minimally invasive and effective procedure.

Evaluation of Basal Serum Adrenocorticotropic Hormone and Cortisol Levels and Their Relationship with Nonalcoholic Fatty Liver Disease in Male Patients with Idiopathic Hypogonadotropic Hypogonadism.

BACKGROUND: Prolonged gonadal hormone deficiency in patients with idiopathic hypogonadotropic hypogonadism (IHH) may produce adverse effects on the endocrine homeostasis and metabolism. This study aimed to compare basal serum adrenocorticotropic hormone (ACTH) and cortisol levels between male IHH patients and healthy controls. Moreover, this study compared the basal hypothalamic-pituitary-adrenal (HPA) axis in patients with and without nonalcoholic fatty liver disease (NAFLD), and also evaluated the relationship between basal HPA axis and NAFLD in male IHH patients. METHODS: This was a retrospective case-control study involving 75 Chinese male IHH patients (mean age 21.4 ± 3.8 years, range 17-30 years) and 135 healthy controls after matching for gender and age. All subjects underwent physical examination and blood testing for serum testosterone, luteinizing hormone, follicle-stimulating hormone, ACTH, and cortisol and biochemical tests. RESULTS: Higher basal serum ACTH levels (8.25 ± 3.78 pmol/L vs. 6.97 ± 2.81 pmol/L) and lower cortisol levels (366.70 ± 142.48 nmol/L vs. 452.82 ± 141.53 nmol/L) were observed in male IHH patients than healthy subjects (all p<0.05). IHH patients also showed higher metabolism parameters and higher prevalence rate of NAFLD (34.9% vs. 4.4%) than the controls (all P < 0.05). Basal serum ACTH (9.91 ± 4.98 pmol/L vs. 7.60 ± 2.96 pmol/L) and dehydroepiandrosterone sulfate (2123.7 ± 925.8 µg/L vs. 1417.1 ± 498.4 µg/L) levels were significantly higher in IHH patients with NAFLD than those without NAFLD (all P < 0.05). We also found that basal serum ACTH levels were positively correlated with NAFLD (r = 0.289,p<0.05) and triglyceride levels (r = 0.268, P< 0.05) in male IHH patients. Furthermore, NAFLD was independently associated with ACTH levels in male IHH patients by multiple linear regression analysis. CONCLUSIONS: The male IHH patients showed higher basal serum ACTH levels and lower cortisol levels than matched healthy controls. NAFLD was an independent associated factor for ACTH levels in male IHH patients. These preliminary findings provided evidence of the relationship between basal serum ACTH and NAFLD in male IHH patients.

Gene Expression Profile of Persistent Postoperative Hypertension Patients with Aldosterone-producing Adenomas.

BACKGROUND: Hypertension often persists after adrenalectomy for primary aldosteronism (PA). Many studies have analyzed the outcomes of adrenalectomy for aldosterone-producing adenomas (APA) to identify predictive factors for persistent hypertension. However, differentially expressed genes in persistent postoperative hypertension remain unknown. Our aim was to describe gene expression profile of persistent postoperative hypertension patients with APA. METHODS: In this study, we described and compared gene expression profiles in persistent postoperative hypertension and postoperative normotension in Chinese patients with APA using microarray analysis. Confirmation was performed with quantitative real time-polymerase chain reaction analysis. Bioinformatic analysis (gene ontology analysis, pathway analysis and network analysis) was used for further research. RESULTS: Microarray analysis identified a total of 99 differentially expressed genes, including 18 up-regulated and 81 down-regulated genes. Among the dysregulated genes were fat atypical cadherin 1 as well as fatty acid binding protein 4 and other genes that have not been previously studied in persistent postoperative hypertension with APA. Bioinformatics analysis indicated that differentially expressed genes were associated with lipid metabolic process, metal ion binding, and cell differentiation. Pathway analysis determined that five pathways corresponded to the dysregulated transcripts. The mRNAs-ncRNAs co-expression network was composed of 49 network nodes and 72 connections between 18 coding genes and 31 noncoding genes. CONCLUSIONS: This study revealed differentially expressed genes in persistent postoperative hypertension with APA and provided a resource of candidate genes for exploration of possible drug targets and prognostic markers.

Polymorphisms in CYP11B2 and CYP11B1 genes associated with primary hyperaldosteronism.

Several frequent polymorphisms in the CYP11B2 gene are suggested to be associated with essential hypertension and aldosterone secretion. In this study, we investigated the association of polymorphisms in CYP11B2 and CYP11B1 genes with the risk of primary hyperaldosteronism (PH). Three polymorphisms in the CYP11B2 gene (intron 2 conversion, rs1799998 and rs4539) and two polymorphisms in the CYP11B1 gene (rs6410 and rs6387) were analyzed in patients with PH and in the normal population. The rs6410 allelic frequencies in patients with aldosterone-producing adenoma (APA) and idiopathic hyperaldosteronism (IHA) were significantly different from those in controls at P=1.09 x 10(-5) and 0.015, respectively. There was a relative excess of AA homozygotes and AG heterozygotes of the rs6410 allele in the APA group as compared with the control group (P=2.19 x 10(-4)). There were significantly different genotypes, AA and AG, of the rs6410 allele between the patients with IHA and the controls only after adjustments for age, gender and body mass index (odds ratio (OR)=4.06, 95% confidence interval (CI) 1.31-12.66; OR=2.41, 95% CI 1.02-5.72). One susceptible haplotype, AAAWT, was identified to be significantly associated with APA (OR=1.44, 95% CI 1.19-1.76), and three susceptible haplotypes, AAAWT, AGGWT and AGAWC, were identified to be significantly associated with IHA (OR=1.55, 95% CI 1.23-1.96; OR=1.49, 95% CI 1.17-1.89; OR=1.40, 95% CI 1.04-1.88). In contrast, one protective haplotype, GGAWT, showed a significant difference between the patients with APA and controls (OR=0.73, 95% CI 0.55-0.97). Several haplotypes were associated with ARR in both the controls and cases. Our data demonstrated that there was a significant association between polymorphisms in the CYP11B2 and CYP11B1 genes and a genetic predisposition to PH. The association with IHA seemed closer compared with APA.

Retroperitoneoscopic adrenalectomy without previous control of adrenal vein is feasible and safe for pheochromocytoma.

OBJECTIVES: To evaluate the effectiveness and safety of retroperitoneal laparoscopic adrenalectomy for pheochromocytoma and report our experience in adrenalectomy without previous control of the adrenal vein. METHODS: From January 2000 to December 2005, 56 patients underwent 58 retroperitoneal laparoscopic adrenalectomy procedures for the treatment of pheochromocytoma. Adequate preoperative antihypertensive preparation was performed. Intraoperative hemodynamic changes were documented in detail. During surgery, the adrenal vein was identified and ligated after dissection and mobilization of the adrenal gland. RESULTS: One patient required conversion to open surgery. The mean operative time and estimated blood loss was 50.4 +/- 19.8 minutes (range 25 to 150) and 76.4 +/- 23.5 mL (range 20 to 300), respectively. A systolic blood pressure greater than 200 mm Hg or less than 80 mm Hg was observed in 6 and 3 patients, respectively. Moreover, an upward fluctuation of systolic blood pressure (20 mm Hg or greater) was recorded during laparoscopic manipulation in 21 patients (37.5%), and in 8 of them, it was 50 mm Hg or greater. The mean diameter of the excised masses was 4.6 +/- 1.7 cm (range 1.5 to 10.0). The mean hospital stay was 5.2 +/- 1.3 days (range 3 to 9). No patients had a major complication and none died. During the follow-up period of 5 months to 3 years, 36 patients recovered normal blood pressure without antihypertensive drugs. No tumor recurrences developed. CONCLUSIONS: Retroperitoneal laparoscopic adrenalectomy without previous control of the adrenal vein is effective and safe for ablation of pheochromocytoma. For experienced surgeons, the tumors larger than 6 cm in diameter can also be removed using the retroperitoneal endoscopic approach.