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A 38-year-old female presented with irregular menstruation and hirsutism that started at age of 16 and diagnosed with polycystic ovary syndrome at age of 29 with elevated testosterone. When treated with ethinestradiol cyproterone tablets, her menstruation returned to normal and androgen levels was not changed. At age of 38 she was referred to the hospital with infertility, a diagnosis of nonclassical 21-hydroxylase deficiency was confirmed using 17-hydroxyprogesterone, dehydroepiandrosterone-sulfate, a cosyntropin-stimulation test and genetic test. This case suggested that nonclassical congenital adrenal hyperplasia should be considered when a patient is presented with oligomenorrhea, hirsutism with hyperandrogenemia and infertility.

Objective: To compare the diagnostic value of adrenocorticotropic hormone (ACTH) stimulation test (AST) with different doses of ACTH combined with midnight administration of 1 mg dexamethasone for the determination of the subtypes of primary hyperaldosteronism (PA)., Methods: This is a prospective observational study. Patients diagnosed with PA in the Department of Endocrinology, the First Medical Center of of Chinese PLA General Hospital from January 1, 2020 to September 30, 2022 underwent AST with different doses of ACTH. All patients received 1 mg dexamethasone at midnight for inhibition. Then, the patients were randomly assigned to 25-unit and 50-unit ACTH treatment groups by a ratio of 1:2. Subtype classification and diagnosis of aldosterone-producing adenoma (APA) and idiopathic hyperaldosteronism (IHA) was made on the basis of adrenal venous blood samples and/or postoperative pathology and clinical follow-up findings. Receiver operating characteristics (ROC) curves were plotted to examine the diagnostic efficacy and the difference of AST by varying doses of ACTH in distinguishing APA and IHA., Results: A total of 82 patients, including 49 patients with APA (59.8%) and 33 patients with IHA (40.2%), were enrolled. There were 29 patients in the 25-unit ACTH group (35.4%) and 53 patients in the 50-unit ACTH group (64.6%). There were no significant differences in age, sex, blood pressure, minimum serum potassium, and biochemical parameters between the 25-unit and 50-unit groups. After ACTH stimulation, plasma aldosterone concentration (PAC), cortisol (F), and PAC/F at different points of time showed no statistical difference between the two groups ( P >0.05). The area under the curve ( AUC ) of PAC in the 25-unit group was higher than that of PAC/F. The AUC of PAC reached the maximum at 90 minutes (0.948, 95% confidence interval [ CI ]: 0870-1.000) and the optimal cutoff was 38.0 ng/dL, which had a sensitivity of 92.9% and a specificity of 86.7% for differentiating APA and IHA. Similar to the 25-unit group, the maximum AUC of PAC in the 50-unit group was greater than that of PAC/F. The AUC of PAC reached the maximum 90 minutes (0.930, 95% CI : 0.840-0.994) and the optimal cutoff was 39.6 ng/dL, which had a sensitivity of 91.2% and a specificity of 83.3%. The AUC of PAC at different points of time in the 25-unit ACTH group (0.862-0.948) was greater than that of 50-unit ACTH group (0.823-0.930), but the difference was not statistical significance., Conclusion: AST with 25-unit or 50-unit ACTH combined with small-dose dexamethasone can be used in PA subtype determination, ie, differentiation between APA and IHA. The optimal PAC cut-off values for 25-unit or 50-unit ACTH are similar, being 38.0 ng/dL and 39.6 ng/dL, respectively, and both cutoff values show higher sensitivity and specificity at 90 min. The AST with 25-unit ACTH has the smaller dose and the better safety. Therefore, it is recommended for the diagnosis of PA subtypes., Competing Interests: 利益冲突　所有作者均声明不存在利益冲突, (© 2024《四川大学学报（医学版）》编辑部 版权所有Copyright ©2024 Editorial Board of Journal of Sichuan University (Medical Sciences).)

Objective: To explore the value of serum parathyroid hormone (PTH) in the diagnosis of primary aldosteronism (PA) and to investigate an optimal cut-off of serum PTH to distinguish PA from nonfunctional adrenal tumor (NFA). Methods: The clinical data of patients with adrenal incidentaloma in Chinese PLA General Hospital from January 1, 2017 to December 31, 2019 were collected. The data of PA and NFA by clinical characteristics and evaluation on endocrine function were retrospectively analyzed. The logistic regression model was used to find the potential risk factors of elevated PTH. The receiver operating characteristic(ROC) curve was used to evaluate the efficacy of PTH in diagnosis of PA and to explore the best cut-off value. Results: A total of 773 patients were included. There were 356 PA patients (203 males, 57.0%), aged (50±11) years and 417 NFA patients (219 males, 52.5%), aged (51±12) years. The serum PTH level in patients with PA was significantly higher than that in patients with NFA [63.1 (48.4, 80.3) ng/L vs 41.7 (34.1, 51.7) ng/L, P <0.05], as well as the proportion of patients with elevated PTH level (47.8% vs 7.2%, P <0.05). Logistic regression analysis showed that having PA and deficiency of Vitamin D were risk factors for PTH elevation (both P <0.05). The ROC curve showed that the best cut-off value of PTH for the diagnosis of PA in patients with vitamin D deficiency was 56.44 ng/L, with a sensitivity of 66.5% and a specificity of 83.0%, and that in patients with normal vitamin D was 48.81 ng/L, with a sensitivity of 70.5% and a specificity of 72.6%. Conclusions: Patients with PA tend to show increased levels of serum PTH compared with NFA patients. The level of serum PTH can be used as one of the valuable indexes in screening of PA.

Objective: To investigate the distinct effects of intermittent and continuous administration of parathyroid hormone (PTH) on bone and mesenchymal stem cell (MSC). Methods: Six weeks old mice with C57/BL6J background and SOX9-creERT/Td-tomato/Osteocalcin-GFP genotype were divided into 6 groups: intermittent administration and withdraw group (subcutaneous injection with PTH 500 μg·kg -1 ·d -1 ), continuous administration and withdraw group (subcutaneous implantation of PTH pump, 80 μg·kg -1 ·d -1, with a rate of 0.25 μl/h), control administration and withdraw group, with 8 mice in each group. Serum calcium level and bone mineral density (BMD) were measured after two weeks' treatment and two weeks after drug withdraw. Histopathology and immunofluorescence analyses were performed to assess the effects of PTH on bone and mesenchymal stem cell. Results: Serum calcium level increased transiently in intermittent group[(1.36±0.03) mmol/L]and increased gradually in continuous group[up to (2.33±0.03) mmol/L], but reduced to normal level (1.12-1.27 mmol/L) 14 days after drug withdraw. BMD of both intermittent[(0.047±0.002) g/cm 2 ]and continuous[(0.046±0.001) g/cm 2 ]PTH administration groups increased compared with control group[(0.044±0.001) g/cm 2 ], but there was no significant difference among three groups 2 weeks after drug withdraw. Femoral histopathology showed that bone mass, trabecular number and little fibrous tissue hyperplasia in intermittent PTH group increased. Osteoblasts number increased, but lining cells decreased. There was no significant difference in osteocyte and osteoclast numbers. After withdrawing of intermittent PTH, osteocyte and osteoblast number declined significantly, but there was an increased number of lining cells. Continuous PTH caused very high amount of fibrosis, and osteoclast number increased significantly, while osteoblast and osteocyte number increased slightly. After withdrawing of continuous PTH, fibrosis disappeared significantly, and lining cell number increased. Immunofluorescence staining in the epiphyseal-metaphyseal regions in fibula showed intermittent PTH increased undifferentiated Td-Tomato MSC, but declined significantly after withdrawing. Undifferentiated Td-Tomato MSC in continuous PTH increased slightly and decreased after drug withdraw. Conclusions: Intermittent PTH increased undifferentiated Td-Tomato MSC and osteoblast number, and might transform lining cell into osteocytes and osteoblasts, and thus lead to bone formation. Continuous PTH increased undifferentiated Td/Tomato MSC, osteoblast and osteocyte number slightly, but high amount of fibrosis and osteoclasts can be seen, leading to metabolic bone disease. However, lining cell ascended after drug withdraw, which may be beneficial to bone remodeling.

Objective: To evaluate efficacy of the 1 mg overnight dexamethasone suppression test (1 mg overnight DST) in the diagnosis of subclinical Cushing's syndrome, and to explore the best diagnostic cut-off value., Methods: The clinical data of patients with adrenal incidentaloma in Chinese PLA General Hospital from 1995 to 2013 were gathered. The data of subclinical Cushing's syndrome (SCS) and non-functional adrenal adenoma (NFA) was retrospectively analyzed. The ROC curve was used to evaluate the efficacy of the 1 mg overnight DST and to explore the best cut-off value with high sensitivity and specificity., Results: There were 447 patients with NFA (224 male and 223 female), and the mean age was 53±11 years old.49 patients were with SCS (19 male and 30 female), and the mean age was 47±12 years old. The area under the ROC of serum cortisol level after 1 mg overnight DST was 0.967 (95%CI: 0.942-0.993). The best cut-off value of serum cortisol after 1 mg overnight DST was 63.65 nmol/L, with a sensitivity of 100.0% and a specificity of 88.8%. The best cut-off value of the suppression ratio of serum cortisol was 85.64%, with a sensitivity of 83.3% and a specificity of 84.6%., Conclusions: The best criterion for 1 mg overnight DST in the diagnosis of SCS was serum cortisol level , and the recommend cut-off point was 63.65 nmol/L, with both a higher sensitivity and specificity. The suppression ratio of serum cortisol after 1 mg overnight DST was also considered as a suitable criterion in the diagnosis of SCS.

Objective: To evaluate the outcomes of intravenous glucocorticoid therapy with or without orbital radiotherapy for moderate-to-severe Graves' ophthalmopathy., Methods: A total of 83 patients with moderate-to-severe Graves' ophthalmopathy were retrospectively analyzed. Intravenous glucocorticoid was given to patients for 3 consecutive days every four weeks in 1-3 circles. The regimens included intravenous glucocorticoid alone (n = 36) and intravenous glucocorticoid plus orbital radiotherapy (n = 47). Photophobia, lacrimation, eye pain, soft tissue congestion, edema, exophthalmos and diplopia were compared before and after treatment. The symptoms and efficacies were compared between two groups., Results: During a 12-week follow-up, clinical activity score (CAS), exophthalmos and diplopia all improved after treatment (P < 0.05). However, the changes of CAS, exophthalmos or diplopia showed no inter-group differences (P > 0.05). The overall clinical response was 50% in glucocorticoid group and 63.8% in glucocorticoid plus orbital radiotherapy group. And there was no statistical difference (P > 0.05)., Conclusion: Moderate-to-severe Graves' ophthalmopathy may be relieved by pulsed intravenous glucocorticoid with or without orbital radiotherapy. There is no inter-group difference in short-term efficacies.

Objective: To summarize the disease distribution characteristics of adrenal lesions and analyze the changes of disease spectrum so as to further guide clinical practices., Methods: All clinical data of adrenal lesion patients hospitalized between 1993 and 2013 were collected. Their profiles of gender ratio, visiting age, final diagnosis and year of hospitalization were retrospectively analyzed., Results: A total of 4 049 patients with adrenal lesions were admitted. There were 2 004 males and 2 045 females with a mean age of 46.82 ± 13.36 years. The number of cases gradually increased year-on-year. And the visiting age was 30-60 years. The annual proportion of adrenal incidentaloma out of total adrenal lesions gradually increased with advancing age. Among them, 3 014 received endocrine function assessments. And there were non-functional lesions (n = 1 503, 49.88%) , adrenal Cushing's syndrome (n = 259, 8.60%), primary aldosteronism (n = 665, 22.04%) and pheochromocytoma (n = 235, 7.80%) . The proportion of non-functional lesions rose from 20.97% to 54.11% (P < 0.05). The proportion of primary aldsteronism declined from 29.84% to 20.43% (P < 0.05). And the proportion of adrenal Cushing syndrome changed slightly (P > 0.05). The proportion of pheochromocytoma declined from 18.55% to 6.99% (P < 0.05)., Conclusion: The number of patients gradually increased over the last two decades.So did the proportion of adrenal incidentaloma. The disease spectrum of adrenal lesions has changed. And the proportion of non-functional lesions increased while that of functional lesions declined.

Objective: To summarize the characteristics of etiological distribution of adrenal incidentaloma during the past 20 years., Methods: All the relevant data of adrenal incidentaloma patients admitted into Chinese PLA General Hospital between January 1993 and January 2013 were collected. Their gender ratio, visiting ages and final diagnoses were retrospectively analyzed., Results: There were 1 173 patients were collected, of which, 629 (53.62%) males and 544 (46.38%) females with a mean age of (50.84 ± 12.13) years. There were 669 (57.03%) hypertensive cases and 622 cases undergone adrenalectomy. Routine medical checkup and abdominal discomfort led to the discovery of adrenal incidentalomas. As to the etiologies, there were non-functional tumors (n = 809, 68.97%), pheochromocytoma (n = 137, 11.68%), primary aldosteronism (n = 84, 7.16%) and adrenal Cushing syndrome (n = 65, 5.54%). The proportions of adrenal cortical carcinomas and metastases were 1.70% (n = 20) and 1.36% (n = 16) respectively. Compared with normotension group, the proportion of non-functional lesions in hypertension group was significant lower (73.81% vs 65.32%, P < 0.05) while the proportions of primary aldosteronism and adrenal Cushing syndrome were higher (11.96% vs 0.79%, 5.83% vs 5.18%, both P < 0.05)., Conclusion: Adrenal incidentaloma patients with concurrent hypertension is quite common. In respect of etiologies, non-functional tumor is the most common and followed by pheochromocytoma and primary aldosteronism Cushing syndrome.