Adrenocortical carcinoma (ACC) is a rare disease with poor prognosis.Patients usually present with signs of steroid hormone excess (e.g.Cushing's syndrome, Conn's syndrome, virilization) or an abdominal mass.In functional cases, Cushing's syndrome is the most frequent presentation.Mitotane is still the most important single drug for ACC treatment, and it plays a role in adjuvant therapy postoperatively as well as in advanced disease.In advanced ACC, a combination of mitotane with etoposide, doxorubicin, cisplatin or streptozotocin with mitotane may be used.Targeted therapies, such as insulin-like growth factor Ⅰ (IGF- Ⅰ)receptor inhibitor and multiple kinase inhibitors like sunitinib and sorafenib hold great promises.

HbA1c measures chronic glycemia and is widely used to judge the adequacy of diabetic treatment and adjust therapy.According to ADAG study,the relationship between HbA1c and the calculated AG can be expressed as the linear regression:eAG(mg/dL)=28.7?HbA1c-46.7.ADA has adopted this equation on its website.In the same unit of measurement as used in self-monitoring blood glucose,eAG would facilitate the doctors to communicate with patients in the daily glucose control.

Objective To identify the factors related to prognosis of diabetic foot ulcer (DFU).Methods A total of 186 patients with type Ⅱ DFU from a single diabetic foot center was included in this prospective study.Follow-up of the final outcome (healing,major amputation or death) was made in 6 months.Influence of patient demographics and clinical data on outcome was detected using multivariate Logistic regression analysis.Results Follow-up was performed in 172 patients,of whom 147 were cured (55 cases cured after minor amputations),3 underwent major amputations,6 died,and 16 were not yet cured at the final follow-up.In multivariate Logistic regression analysis,the outcome was independently correlated with ischemia (P ＜0.01),infection (P ＜ 0.05),ulcer number (P ＜ 0.01) and peripheral neuropathy (P ＜ 0.05) ; the risk of poor outcome increased with ischemia [odds ratio (OR) =10.8],infection (OR =211.4),ulcer number (OR =39.5),and peripheral neuropathy (OR =181.1).Conclusion Prognosis of DFU is associated with ischemia,infection,ulcer number,and peripheral neuropathy.

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.

Objective:To investigate the clinical manifestations, laboratory and imaging features, treatment and prognosis of systemic lupus erythematosus (SLE) with primary sclerosing cholangitis (PSC).Methods:This is a retrospective case series study describing the clinical, laboratory and imaging manife-stations, treatment and prognosis of 3 SLE patients with PSC. The related literatures were reviewed.Results:In total, 10 patients were included. SLE with PSC, with an average age of (43±17) years old, was more common with hematological and renal involvement, jaundice and arthralgia, positive anti-double-stranded DNA (anti-dsDNA) antibody, hypocomplementemia, elevated erythrocyte sedimentation rate (ESR) and abnormal liver function with predominately elevated alkaline phosphatase (ALP). The classic magnetic resonance cholangio-pancreatography (MRCP) features of PSC include multifocal strictures, beading and areas of dilatation involving the intra- and/or extrahepatic bile ducts. The treatment included glucocorticosteroids, immunosup-pressants and ursodeoxycholic acid (UDCA), and some patients required biliary drainage. Most patients had a good prognosis.Conclusion:Although PSC is rare, attention should be paid to SLE patients with abnormal liver function, especially with elevated ALP, in order to differentiate from PSC.

Objective:Anti-synthase syndrome (ASS) is a rare autoimmune disease. To increase the understanding of the disease and reduce the rate of miss diagnosis.Methods:The clinical data of 8 patients with positive anti-synthase antibody afterprimary Sj?gren's syndrome (pSS) were retrospectively analyzed and descriptive statistical analysis was carried out.Results:The diagnosis of Sjogren's syndrome (SS) was in accordance with the revised European criteriaof SS issued by the US-Europe consensus Group in 2002 or the classification criteria of American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) SS in 2016, and the diagnostic ASS was in accordance with the diagnostic criteria of Conners in 2010 or Solomon in 2011. Eight(100%) patients had a history of interstitial lung disease, and 7 (88%) patients had fever (oral temperature >38.5 ℃). All patients were positive for anti-Ro-52 antibody, 4 patients were positive for anti-PL-7 antibody, 2 patients were positive for anti-EJ antibody, 1 patient was positive for both anti-PL-7 antibody and anti-EJ antibody, and 1 patient was positive for anti-PL-12.Conclusion:pSS patients with severe interstitial lung disease or high fever of unknown causes should be screened for anti-synthase antibodies and the possibility of ASS.

Objective To explore the efficacy of pulse infusion of gonadorelin (LHRH) on the patients with idiopathic hypogonadotropic hypogonadism (IHH) via a micro infusion pump. Methods The protocol was designed as an open, self-controlled prospective study. 31 patients were enrolled and assigned to 3 groups: 23 males without gonadotropin-releasing hormone ( GnRH ) pulse ( group A), 2 males with GnRH pulse frequency insufficiency ( group B), and 6 females ( group C). All the subjects were admitted LHRH every 90 min via the micro infusion pump for 24 weeks. Sex hormones and related characteristics were compared before and after the treatment. Results After 24-weeks treatment, LH peak value reached ( 6. 92 ±5.66 ), ( 9. 55 ±0. 98 ), and ( 6. 93 ±4. 52 ) IU/L; and FSH peak value reached ( 7.44 ± 3. 80 ), ( 12. 85 ± 12. 80 ), and ( 7.38 ±4. 98 ) IU/L among 3 groups, respectively. The testosterone also reached ( 3.18± 1.81 ) and ( 5.78±4. 65 ) ng/ml in groups A and B ( all P＜0. 01 ). In groups A and B, the testis volumes were increased, seminal fluid production was found in 7 patients and spermatogenesis in 6 patients. In group C, uterus was enlarged 85.4%, as well as the ovaries of both sides. Menarche was reported in 5 patients. 19. 4% of the studied patients complained uncomfortable at the injection sites, all the symptoms were mild. Conclusion Pulse infusion of LHRH in IHH patients via a micro infusion pump is effective, while the medication system needs improving.

Objective:To observe the clinical efficacy and adverse reactions of rituximab in the treatment of systemic sclerosis (SSc).Methods:Eight SSc patients who received rituximab treatment in the Department of Rheumatology of Shanghai Ruijin Hospital from November 2016 to May 2020 were treated with rituximab at week 0, week 2, week 4, week 24 and week 48. The clinical symptoms and laboratory parameters were evaluated at baseline, week 4, week 24 and week 48 respectively. All data were analyzed by Wilcoxon test.Results:All the patients were diagnosed as diffuse SSc, including seven females and one male, with a median disease course of 2.5 years. At week 0, week 24 and week 48, the modified Rodnon skin scores (MRss) were 16.5 (11.8, 29.5) , 14.5 (9.5, 27) ( Z=0.841) and 10.5 (7, 24.3) ( Z=0.420) respectively, which were significantly improved as compared with the baseline ( P<0.05). The patients' self-scores were 60(50, 77.5), 52.5(41.3, 67.5)( Z=0.113) and 47.5(36.3, 57.5)( Z=0.474) respectively, which were significantly improved at week 24 and week 48, and the High Resolution CT (HRCT) scores at baseline and week 48 were 2.7(1.02, 3.7) and 1.6(0.65, 2.95)( Z=0.964) respectively, significantly improved after treatment ( P<0.05). The pulmonary aterial hypertension (PAH) values were 48(41, 58.5) mmHg and 47(38.5, 57) mmHg ( Z=0.315) respectively. There was no significant difference between the two groups. Clinical observation showed that the condition was improved and no adverse reaction occurred at the same time period. Conclusion:The improvement of skin sclerosis, pulmonary interstitial lesion and pulmonary artery pressure can be observed during the treatment with rituximab, which may be a new choice for the treatment of SSc. There is no serious adverse reaction during the treatment, and the patients are well tolerated and safe.

Objective To evaluate the effects of different depths of sedation on postoperative cognitive function in elderly patients with mild cognitive impairment.Methods A total of 100 patients with mild cognitive impairment before surgery,aged 65-75 yr,weighing 55-75 kg,of American Society of Anesthesiologists physical status Ⅱ or Ⅲ,scheduled for elective gynecological surgery under general anesthesia,were divided into Ⅰ and Ⅱ groups (n =50 each) using a random number table method.Propofol was given by closed-loop target-controlled infusion,and the target plasma concentration was automatically regulated.The bispectral index value was maintained at 40-50 in group Ⅰ and at＞50-60 in group Ⅱ.Mini-Mental State Examination (MMSE) and Montreal Cognitive Assessment (MoCA) were used to evaluate the cognitive function at 1 day before operation (T0) and 7 days after operation (T1),and the development of postoperative cognitive dysfunction (POCD) was recorded.Venous blood samples were collected at T0 and T1 for determination of the concentrations of serum interleukin-10 (IL-10) and tumor necrosis factor-alpha (TNF-α) by enzyme-linked immunosorbent assay.Results Compared with the baseline value at T0,MoCA and MMSE scores were significantly decreased at T1,and the serum concentrations of IL-10 and TNF-α were increased in both groups (P＜0.05).Compared with group Ⅰ,MoCA and MMSE scores were significantly decreased at T1,and the incidence of POCD was increased,the serum concentration of TNF-α was increased,and the serum concentration of IL-t0 was decreased in group Ⅱ (P ＜ 0.05).Conclusion Maintaining BIS value at 40-50 during operation can decrease the development of POCD in elderly patients with mild cognitive impairment,which may be related to reduced systemic inflammatory responses.

Objective To evaluate the effect of age factor on the sedative efficacy of propofol given by closed-loop infusion in patients undergoing gynecological laparoscopic surgery.Methods A total of 100 patients,weighing 55-75 kg,of American Society of Anesthesiologists physical status Ⅰ or Ⅱ,scheduled for elective gynecological laparoscopic surgery under general anesthesia,were divided into 2 groups (n =50 each) according to age:middle-aged group (45-64 yr) and elderly group (65-75 yr).Propofol was given by closed-loop infusion,and the target plasma concentration was automatically regulated to maintain bispectral index (BIS) value within the target range of 45-55.Remifentanil was given by target-controlled infusion at a target plasma concentration of 2-6 ng/ml to maintain anesthesia,and cisatracurium was injected intermittently to maintain muscle relaxation.The total consumption of propofol and remifentanil,regulating frequency of propofol,time ratio of BIS40-60,global score reflecting performance of closed-loop infusion system,emergence time and extubation time were recorded.Results Compared with middle-aged group,the time ratio of BIS40-60 was significantly increased,global score was decreased,the total consumption of propofol and remifentanil was significantly decreased,the regulating frequency of propofol was increased (P＜0.05),and no significant change was found in emergence time or extubation time in elderly group (P＞0.05).Conclusion The sedative efficacy of propofol given by closed-loop infusion is influenced by age factors when used for gynecologic laparoscopic surgery,and the stability in elderly patients is superior to that in middle-aged patients.