The clinical analysis of severe adrenal Cushing′s syndrome / 中华内分泌代谢杂志

Objective To investigate the methods and efficacy of treatment on severe adrenal Cushing′s syndrome. Methods The clinical data of 22 cases with severe adrenal Cushing′s syndrome ( severe group) , and 136 cases with mild or moderate adrenal Cushing′s syndrome ( non-severe group) were reviewed. The clinical features were analyzed by comparing the differences between these two groups when patients were admitted to hospital. We discussed the clinical managements of patients with severe adrenal Cushing′s syndrome by comparing the differences with non-severe group after preoperative preparation, and with themselves before and after preoperative preparation. The effects of surgery were evaluated by comparing the differences between pre-operation and post-operation on patients with severe adrenal Cushing′s syndrome. Results At admission, serum/urine cortisol, disease course, and blood pressure were significantly higher in the severe group than those in non-severe group ( P＜0.05 or P＜0.01) , serum potassium and ACTH level were decreased significantly in the severe group than those innon-severegroup[(3.01±0.75vs3.62±0.48)mmol/L,P＜0.01;(6.47±2.91vs8.21±3.22)pg/ml,P＜0.01] . However, no significant difference was observed in diastolic blood pressure, serum potassium, and fasting plasma glucose between these two groups after preoperative preparation (all P＞0.05). And then, we performed adrenalectomy. The symptoms of 22 cases with severe adrenal Cushing′s syndrome were obviously alleviated after 3 months. During follow-up, 5 cases of primary bilateral macronodular adrenal hyperplasia ( BMAH) and 1 case of primary pigmented nodular adrenocortical disease ( PPNAD ) were treated with contralateral adrenalectomy. Conclusion Sufficient preoperative preparation is essential for patients with severe adrenal Cushing′s syndrome because of its high level serum cortisol with severe complications. If preparation fails before surgery, cortisol-lowering medication or emergency unilateral adrenalectomy is necessary. Severe patients with BMAH and PPNAD were firstly performed unilateral adrenalectomy and followed-up closely, and then, contralateral adrenalectomy is needed when the recurrence of hypercortisolism recognized.

Correlation of hypoxia inducible factors, vascular endothelial growth factor receptor 2, and microvessel density expressions with angiogenesis in adrenocortical tumours / 中华内分泌代谢杂志

Objective To explore the expressions of hypoxia inducible factor ( HIF), vascular endothelial growth factor receptor 2 ( VEGFR 2), and microvessel density ( MVD) in adrenocortical adenoma ( ACA) and adrenocortical carcinoma ( ACC), in order to discuss their potential role in the development of adrenal tumours. Methods Fifty-five adrenal tumour specimens resected in the hospital with complete clinical data (including 30 ACA cases and 25 ACC cases) were examined by immunohistochemistry for the expressions of HIF-2α, HIF-1α, VEGFR 2, and MVD. Results VEGFR 2 and MVD up-regulated were found in the ACC group (P＜0.05). The expression of HIF-2α and HIF-1α correlated with VEGFR 2 (P＜0.05). The expressions of VEGFR 2 and MVD were related to some clinicopathological features ( P＜0. 05 ). Additionally, tumour size, expression of VEGFR 2 and MVD were independently associated with ACC (P＜0.05). Conclusions The high expression of HIF-2α, VEGFR 2, and MVD in adrenal tumours suggested their roles in tumour angiogenesis, which indicated that anti-angiogenesis therapies deserve intensive studies for malignant adrenocortical tumours.

Efficacy and safety of spironolactone in mono-therapy versus low dose spironolactone in combined-therapy for the treatment of idiopathic hyperaldosteronism / 中华内分泌代谢杂志

Objective The aim of this study was to compare the efficacy and safety of spironolactone in mono-therapy versus low dose spironolactone in combined-therapy for the treatment of idiopathic hyperaldosteronism (IHA). Methods This was a prospective and randomized study. After 2 weeks wash out period, 48 patients confirmed IHA were assigned to either spironolactone mono-therapy or low dose spironolactone combined-therapy groups. All patients underwent adrenal venous sampling and showed no lateralization. The primary outcome was the percentage of patients with blood pressure<140/90 mmHg(1 mmHg＝0.133 kPa) at 16 weeks. The other observatory parameters were: the occurrence of gynecomastia, renal insufficiency, and hyperkalemia. Results At the end of 16 weeks, the patients′blood pressure were normalized in 17 out of 24 patients(70.8%) in mono-therapy group and in 23 out of 24 patients ( 95. 8%) in combined-therapy group ( P<0. 05). There was no hyperkalemia in both groups. 2 patients and 5 patients in mono-therapy group were observed renal insufficiency and gynecomastia respectively, while no patients in combined-therapy group presented with those side effects. Conclusion Low dose spironolactone combined-therapy group is more effective to control blood pressure and hypokalemia without increase the occurrence of renal insufficiency or gynecomastia.

Research advances on the TCA cycle in pheochromocytoma and paraganglioma / 中华内分泌代谢杂志

In pheochromocytoma and paraganglioma (PPGL), mutations in the TCA cycle related genes may lead to the disturbance of the TCA cycle's intermediate metabolite levels, then cause pseudohypoxia by abnormal hypoxia-inducible factor ( HIF) activation, it also may lead to epigenetic changes, impede cell differentiation and apoptosis, and finally promote the occurrence and development of PPGL. This review focus on the research advances on the TCA cycle in the pathogenesis of PPGL, as well as the application of relevant detection methods.

Clinical analysis of bilateral macronodular adrenal hyperplasia / 中华内分泌代谢杂志

Objective To investigate the clinical characteristics in patients with bilateral macronodular adrenal hyperplasia ( BMAH). Methods We enrolled 357 patients with adrenal tumors including 42 BMAH, 167 cortisol-producing adrenal adenoma (CPA), and 148 non-functional adenomas (NFA). The clinical manifestations, hormonal explorations and complications were compared among groups. Results (1) Age at diagnosis of BMAH group was significantly higher than that of CPA group [(53.7±8.3 vs 41.1±13.1)years], female proportion of BMAH group was lower that that of CPA group(54.8%vs 88.0%, P＜0.01). Both were similar to NFA group. (2) Compared to NFA group, metabolic abnormalities were severer in BMAH and CPA groups. Prevalence of hypertension, diabetes mellitus, and hypokalemia were the highest in BMAH group. ( 3) BMAH patients had lower midnight serum cortisol, 24 hour urine free cortisol, and adrenocorticotrophic hormone levels than CPA patients (P＝0.008, 0.010, and P＝0.001, respectively). The suppression rate of serum cortisol after low-dose dexamethasone suppression test was significantly higher in BMAH group ( P＝ 0. 005). Conclusion BMAH patients were at higher age at diagnosis. Although hypercortisolism in BMAH patients is relatively slight, metabolic abnormalities are more severe. Besides functional diagnosis, physicians should pay more attention to the assessment of complications related to hypercortisolism.

Hormonal and radiographic features of primary empty sella / 中华内分泌代谢杂志

Clinical data of 116 cases diagnosed as primary empty sella (PES) from 2012 to 2016 in the Department of Endocrine and Metabolic Diseases, Ruijin Hospital were analyzed in this study. Insulin-induced hypoglycemia test and magnetic resonance imaging (MRI) were applied to explore the predictive factors of hypopituitarism in patients with PES. The results showed that 43 (37. 07%) patients with PES were with hypopituitarism. Pituitary height<2. 35 mm and pituitary sagittal area<23. 5 mm2indicated a high risk of hypopituitarism in patients with PES.

Hyponatremia with pituitary tumor---One case of syndrome of inappropriate antidiuretic hormone secretion / 中华内分泌代谢杂志

A 57-year-old female patient was admitted to our hospital because of sustained hyponatremia within stress dose of hydrocortisone replacement after transsphenoidal partial pituitary tumor surgery. Multiple clinical parameters investigation for 2 weeks was shown increasing serum Na+and uric acid level and decreasing blood pressure level under water restriction about 500 ml/d, and even without hydrocortisone replacement, the serum Na+ still maintain normal level. After surgeries and radiotherapy, the tumor was undetectable and serum Na+ level was within the normal range without any water restriction.

Palpitation, tremble, sweaty, and weight gain---Localization of insulinoma / 中华内分泌代谢杂志

[Summary] A 47 years old female presented with palpitation, tremble, sweaty, and weight gain. Insulinoma was suspended and confirmed with hypoglycemia caused by endogenous hyperinsulinemia. The CT scan was failed to localize the tumor. MRI and ASVS showed inconsistent results. A nutrient arterial was found by arterial angiography and explained the different results. Robert assistant surgery successfully removed the tumor and the patient was well recovered.

Extremely mild cholesterol side chain cleavage enzyme deficiency with infertility as the only presenting complaint / 中华内分泌代谢杂志

[Summary] All patients with cholesterol side-chain cleavage enzyme ( P450scc) deficiency that have been reported presented with early adrenal failure. Here we described a 35-year-old male presented with infertility as the only initial presenting complaint. He had received two separate surgeries to remove bilateral testicular masses. We reevaluated the resected tumors and found testicular adrenal rest tumor ( TART) pathology in the resected tumor. We profiled steroid hormones and found significantly elevated ACTH. CT scan revealed bilateral adrenal hyperplasia. Mutation screening identified compound heterozygous mutations (R353W and P432L) in the P450scc encoding gene (CYP11A1). The patient was finally diagnosed as congenital adrenal hyperplasia.

Right cheek swelling and pain for 2 weeks---Cushing′ syndrome complicated with facial infection / 中华内分泌代谢杂志

[Summary] A 61-year-old female patient was diagnosed as ACTH independent Cushing′ syndrome, right adrenal adenoma, right facial infection. The situation was well controlled with antibacterial agents, abscess incision drainage and ketoconazole therapy. The patient received partial adrenolectomy of her right adrenal gland 16 months later. This case indicated that anti-adrenal agents could be a reasonable choice when the Cushing′syndrome patients were acompanied with severe infection.

Approach to the patient with ectopic ACTH syndrome caused by adrenal pheochromocytoma : diagnosis and treatment / 中华内分泌代谢杂志

Ectopic ACTH syndrome caused by adrenal pheochromocytoma is very rare.A case was herewith reported and the domestic and foreign literatures were reviewed.The correct diagnosis of the syndrome depends on clinical,biochemical,hormonal,radiographic,pathological investigations,as well as tumor immunohistochemistry for final comprehensive judgments.

The value of bilateral inferior petrosal sinus sampling in the differential diagnosis of ACTH-dependent Cushing's syndrome / 中华内分泌代谢杂志

Objective To evaluate the values of bilateral inferior petrosal sinus sampling ( BIPSS),high dosage dexamethasone suppression test (HDDST) and pituitary image in the differential diagnosis of ACTH-dependent Cushing's syndrome. Methods Totally 87 patients with confirmed pathological diagnosis were recruited in the study.All received the procedure of BLPSS,HDDST,and pituitary MRI with dynamic enhancement.The diagnostic performances of three differential diagnosis methods in ACTH-dependent Cushing's syndrome were evaluated.Results Seventy-eight patients were diagnosed as cases of pituitary ACTH adenoma,and the remaining 9 were confirmed cases of ectopic ACTH syndrome due to the thymic carcinoid.The sensitivity and specificity of HDDST,pituitary MRI,and BIPSS for the diagnosis of ACTH-dependent Cushing's syndrome were 82.1％ and 100％,79.5％ and 44.4％,92.3％ and 100％,respectively.In Cushing's disease,the diagnostic accuracy was 83.9％ with HDDST,77.0％with pituitary MRI,and93.1％ with BIPSS.In those patients with Cushing's disease,the coincidence of lateralization was 83.9％ with BIPSS and 64.5％ with MRII.Conclusion BIPSS was better than the other two methods in differential diagnosis of ACTH-dependent Cushing's syndrome.Compared with the pituitary MRI,the concordant rate of BIPSS in lateralization of the tumor is higher,and it is more reliable.

Clinical significance of aldosterone to renin ratio in screening primary aldosteronism / 中华内分泌代谢杂志

ObjectiveTo retrospectively analyse the data of patients with suspected primary aldosteronism (PA) during the last five years.The receiver operating characteristic (ROC) curve was used to evaluate the value of aldosterone to renin ratio (ARR) in screening primary aldosteronism.MethodsThe clinical data of ARR in supine and upright positions were collected in 590 patients with suspected during January 2006 to August 2010.There were 357 patients diagnosed as primary aldosteronism and 233 patients as essential hypertension among these patients.100 patients with suspected primary aldosteronism took the upright and random ARR during September 2010 to April 2011 ; Among these patients,29 patients were diagnosed as primary aldosteronism and 71 as cases of essential hypertension.ROC curve was made to retrospectively define the method and the cut-off value of the supine,upright or random ARR.ResultsThe area under the curve(AUC) of the supine ARR in the 590 patients who took the supine and upright ARR during January 2006 to August 2010 was 0.838 (0.805-0.867 ) and the AUC of the upright ARR was 0.873 (0.843-0.899 ).There was significant difference between these two AUC readings (P＜0.01 ).The AUC in the upright ARR of the 100 patients who took the upright and random ARR during Septeuder 2010 to April 2011 was 0.962 (0.928 -0.995 ) and the AUC of the random ARR was 0.944 (0.893-0.994 ).There was no significant difference between these two AUC readings (P＞0.05).The upright ARR cutoff value with 400 ( pg · ml-1 )/( ng· ml-1 · h 1 ) yielded a sensitivity of 91.9％ and a specificity of 64.2％ to diagnosis of PA.ConclusionUpright ARR value was more suitable in the screening test than supine ARR value.Random ARR showed similar effect as the upright ARR.In our research,under strict control of the drug,position and detection time,ARR value with 400 ( pg · ml-1 )/( ng· ml-1 · h-1 ) can be the cutoff point in screening the suspcctcd PA patients.

Multi-detector-row CT findings of ACTH-independent macronodular adrenal hyperplasia / 中华内分泌代谢杂志

ObjectiveTo explore the characteristics multi-detector-row computed tomography (MDCT)findings of ACTH-independent macronodular adrenal hyperplasia ( AIMAH ).Methods The un-enhanced and contrast-enhanced MDCT features in 24 patients ( 14 males and 10 females) with clinically confirmed AIMAH were retrospectively assessed for the morphology and enhancement patterns.ResultsThe adrenal glands were involved bilaterally in all of the 24 cases( 100％ ).24 patients had massively enlarged multinodular adrenal glands.Nodules were( 1.79 ± 1.02) cm (0.50 ～ 3.85 cm),which usually distorted and completely obscured the normal adrenal glands.The enlarged adrenal glands were still retained the adreniform contour,showed characteristic ginger-like.22 of the 24 ( 91.7 ％ ) hyperplastic nodular glands demonstrated mild homogeneous enhancement.Calcification was revealed in 1 adrenals ( 1/24,4.2％ ).Conclusion MDCT reveals the characteristic morphology and CT attenuation in AIMAH.Combined with its clinical presentation and biochemical findings,AIMAH is able to be diagnosed with high specificity and accuracy on MDCT.

Approach to the patient of pheochromocytoma: the diagnosis and treatment / 中华内分泌代谢杂志

A 34-year-old male patient with suspected pheochromocytoma was referred to our department for paroxysmal hypertension. Pheochromocytoma was confirmed by very high plasma nor-metanephrine ( NMN ) and metanephrine ( MN ). Contrast CT of the adrenal disclosed a 3.0 cm x 3.0 cm mass in the left adrenal. 18 F-FDG-PET-CT showed high uptake only in the left adrenal. When the patient's blood pressure was well controlled with 4 mg/d doxazosin for 2 weeks, surgery was then performed under laparoscopy. A round solid left adrenal pheochromocytoma was resected. After surgery, plasma MN and NMN levels returned to normal, and the patient was free of clinical symptoms with normal blood pressure. This patient has been followed 3 years with normal blood pressure, MN, and NMN levels, without tumor recurrence shown hy adrenal CT.

Mutation of SDHB gene in sporadic patients with pheochromocytom / 中华内分泌代谢杂志

d, suggesting that the mutation rate of SDHB gene in sporadic Chinese patients with pheochromocytoma might be around 3%.