Acute idiopathic maculopathy(AIM)is an inflammatory lesion of unknown cause that primarily affects the macula. It follows a unique natural course, distinct from other maculopathy, often manifesting as a sudden loss of visual acuity followed by flu-like symptoms that gradually resolve as the disease subsides. A comprehensive understanding of the unique history, multimodal imaging, and a thorough systematic examination are crucial in determining the final diagnosis of AIM. The treatment and prognosis of AIM remain controversial. Meanwhile, it presents similar clinical manifestations and pathological changes to various chorioretinopathy, posing challenges for clinical differentiation. This article provides a review of its pathogenesis, clinical symptoms, multimodal imaging features, diagnosis and differential diagnosis, treatment and prognosis, in order to reduce misdiagnosis and mistreatment while enhancing comprehension of AIM.

OBJECTIVE To observe the clinical efficacy and effect on serum thymic stromal lymphopoietin(TSLP)levels of pa-tients with advanced liver cancer of qi deficiency and toxic stasis type by Jiawei Yupingfeng San.METHODS Using random double blind method,120 patients with advanced liver cancer of qi deficiency and toxic stasis type were randomly divided into 3 groups:Jiawei Yupingfeng San group,Yupingfeng San group,and placebo group,each consisting of 40 cases.All patients in the 3 groups were given conventional treatment such as radiotherapy,chemotherapy,interventional or targeted therapy;Jiawei Yupingfeng San group was given Jiawei Yupingfeng San granules,Yupingfeng San group was given Yupingfeng San granules,and placebo group was given placebo.The course of treatment was 2 months.The changes of Karnofsky functional status score(KPS score),TCM syndrome score,tumor size and serum TSLP level in the 3 groups were observed before and after treatment,and the correlation between the changes of tumor size and TSLP was analyzed.RESULTS After treatment,the KPS scores of Yupingfeng San group and Jiawei Yupingfeng San group were sig-nificantly increased(P<0.05,P<0.01),TCM syndrome score were decreased(P<0.01),tumor growth(P<0.05,P<0.01)was de-layed,and serum TSLP levels(P<0.05,P<0.01)were decreased.Furthermore,there was a slight positive correlation between chan-ges in tumor size and changes in TSLP(P<0.05).In terms of improving tumor size,the curative effect of Jiawei Yupingfeng San group was better than that of Yupingfeng San group(P<0.05).During the treatment period,no obvious adverse reactions were observed in the 3 groups of patients.CONCLUSION Combined with conventional treatment,Jiawei Yupingfeng San can significantly delay tumor growth in patients with advanced liver cancer of qi deficiency and toxic stasis type and improve patients'TCM syndromes and their qual-ity of survival.The therapeutic mechanism is related to reducing the expression of serum TSLP and improving the immune status of pa-tients,thereby delaying the growth of tumors.

Objective:To evaluate the safety and efficacy of percutaneous transhepatic papillary balloon dilation (PTPBD) combined with flexible ureteroscopy-guided dual-frequency double-pulse ND:YAG (FREDDY) laser lithotripsy (PTPBD-FREDDY) for the treatment of giant (>1.5 cm diameter) common bile duct stones.Methods:A retrospective analysis was conducted on 26 patients with large-diameter difficult choledocholithiasis admitted to two medical centers from December 2017 to October 2021. Among these patients, four could not tolerate surgery or endoscopic treatment, six experienced failure of endoscopic treatment, and 16 refused to undergo endoscopic or surgical treatment. All patients underwent the PTPBD-FREDDY procedure. The FREDDY laser lithotripsy was performed under ureteroscopic guidance, followed by a balloon to push the stones into the duodenum. The primary endpoint was the technical success rate, and the secondary endpoints included the rate of stone recurrence and related complications.Results:All 26 patients successfully completed the operation, achieving a technical success rate of 100%. The average lithotripsy frequency and operation time for bilirubin stones were significantly higher than those of mixed stones and cholesterol stones ( P<0.01). The main postoperative complications included mild fever ( n=3), abdominal pain ( n=3), nausea ( n=2) and vomiting ( n=1). One patient experienced biliary tract bleeding, which improved after conservative treatment. No serious complications such as pancreatitis, sepsis, or biliary perforation were observed. After 2 years of follow-up, no cases of stone recurrence were observed. Conclusions:PTPBD-FREDDY is a safe and effective treatment for patients with giant common bile duct stones. It provides a new therapeutic option for patients with giant choledocholithiasis who can not tolerate surgery or have failed endoscopic treatment, demonstrating promising prospects.

Objective:To investigate the clinical profile of primary bilateral macronodular adrenal hyperplasia(PBMAH) and sex difference.Methods:One hundred and forty cases of PBMAH were recruited in our center from 2014, and all patients were evaluated for hormone secretion, adrenal imaging, and metabolic parameters.Results:Overt Cushing′s syndrome accounted for 76.4% of PBMAH cohort and 47.9% were female. The overt group had higher serum cortisol and 24 h urinary free cortisol levels, lower adrenocorticotropic hormone, higher serum cortisol after low-dose dexamethasone suppression tests, larger total adrenal size, and a higher percentage of obesity, hypertension, diabetes mellitus, and hypokalemia than the subclinical group(all P<0.05). When compared with the male group, the female group had smaller adrenal size( P<0.001), lower HbA 1C( P=0.003), higher total cholesterol( P=0.005), and lower density lipoprotein-cholesterol levels( P=0.035). Further, 24 h urinary free cortisol in the male group was found to be positively correlated with diastolic blood pressure, fasting glucose, 2 h postprandial glucose after oral glucose tolerance test(OGTT), and HbA 1C after adjusted for age, body mass index, and onset duration, and was negatively correlated with body mass index and potassium levels. While 24 h urinary free cortisol in the female group was positively correlated only with diastolic blood pressure, fasting glucose, and 2 h postprandial glucose after OGTT(all P<0.05). During follow-up, 80.0% of patients achieved remission after unilateral adrenalectomy, with a recurrence rate of 17.9%. Conclusion:PBMAH related metabolic disorder is more pronounced in overt Cushing′s syndrome and males. Unilateral adrenalectomy as an effective treatment can benefit the majority of patients.

【Objective】 To investigate the efficacy of robot-assisted single-port laparoscopic transvaginal vesicovaginal fistula repair. 【Methods】 The clinical data of 3 patients with high vesicovaginal fistula treated during Jun.2020 and Jun.2021 were retrospectively analyzed. 【Results】 All operations were completed successfully, with no conversion to other surgical methods. Operation time: 98 min, 104 min and 115 min; Intraoperative bleeding volume: 15 mL, 20 mL and 22 mL; Postoperative hospital stay was 2 days. The catheter was removed after 1-month follow-up, and the patients had no bleeding, urine leakage, infection or other complications. There was no recurrence of urine leakage at the end of 12-month follow-up. 【Conclusion】 Robot-assisted single-port laparoscopic transvaginal vesicovaginal fistula repair has the advantages of fine suture and minor damage, which can be an effective treatment of vesicovaginal fistula.

Humans ; SARS-CoV-2 ; COVID-19 ; Antibodies ; Antibodies, Viral/therapeutic use* ; Antibodies, Neutralizing/therapeutic use*

Objective:To preliminary observe the clinical efficacy of vedolizumab (VDZ) in the treatment of active Crohn′s disease (CD).Methods:From March 2021 to October 2022, a total of 22 patients with active CD who received VDZ treatment at Zhongda Hospital, Southeast University were retrospectively enrolled. The general clinical data, laboratory indicators, imaging finding and endoscopic images of the patients were collected. The Crohn′s disease activity index (CDAI), hypersensitive C-reactive protein (hs-CRP), erythrocyte sedimentation rate (ESR), clinical remission rate were evaluated at week 0, 2, 6 and 14 of VDZ administration. Meanwhile, the response rate under endoscopy and remission rate under endoscopy were evaluated after 14 weeks of VDZ administration. The related factors affecting the efficacy of VDZ were further analyzed. Kruskal-Wallis H test and Mann-Whithey U test were used for statistical analysis. The multiple logistic regression analysis was used to find the related factors affecting the clinical remission after VDZ treatment. Results:The CDAI at week 0, 2, 6 and 14 after treatment were 181.01 (160.11, 231.56), 148.05 (134.88, 200.52), 127.46 (91.44, 163.62), and 82.35 (63.50, 121.84), respectively, and the differences were statistically significant ( H=34.23, P<0.001); there were statistically significant differences between week 0 and week 2, 6, 14 after treatment ( U=130.00, 80.00, 33.00; P=0.017, <0.001, and<0.001). The hs-CRP levels were 5.72 mg/L (3.59 mg/L, 11.10 mg/L), 2.86 mg/L (0.86 mg/L, 5.27 mg/L), 1.55 mg/L (0.86 mg/L, 9.89 mg/L) and 2.86 mg/L (0.86 mg/L, 3.12 mg/L), respectively, and the differences were statistically significant ( H=9.69, P=0.021); there were statistically significant differences between week 0 and week 2, 6, 14 after treatment ( U=102.00, 109.00, 98.00; P=0.026, 0.045, and 0.011) .The level of ESR after 14 weeks of VDZ treatment was 8.00 mm/1 h (4.00 mm/1 h, 17.00 mm/1 h), which significantly decreased compared with that before treatment (17.00 mm/1 h(12.25 mm/1 h, 21.75 mm/1 h)), and the difference was statistically significant ( U=132.50, P=0.020). The clinical remission rates at week 2, 6 and 14 after VDZ treatment were 54.5% (12/22), 68.2% (15/22) and 86.4% (21/22), respectively, and the clinical response rates were 18.2% (4/22), 54.5% (12/22) and 95.5% (21/22), respectively. After 14 weeks of VDZ treatment, among 17 patients who underwent endoscopic re-examination, 9 patients achieved response under endoscopy and 3 patients achieved remission under endoscopy. Stenotic type and penetrating type of CD, previous use of glucocorticoids or immunosuppressants were risk factors of no clinical remission after VDZ treatment ( β=-4.586, -5.005 and -3.371; OR=0.010, 0.007 and 0.034; P=0.010, 0.025 and 0.045). While VDZ treatment for 14 weeks was a protective factor ( β=2.475, OR=11.885, P=0.011). Conclusions:VDZ can effectively relieve disease activity in patients with active CD. The disease behavior of CD, previous medication treatment of patients, and the duration of VDZ treatment may be related to the efficacy of VDZ.

Objective:To effectively express the receptor binding domain (RBD) of SARS-CoV-2 spike protein in Pichia pastoris and to evaluate its immunogenicity. Methods:The gene encoding the RBD protein was synthesized and cloned into the pPICZαA plasmid. After linearization, the plasmid was transferred and integrated into the genome of Pichia pastoris. The expressed RBD protein in culture supernatant was analyzed by Western blot and Biolayer interferometry. After screening, a single clone expressing the RBD protein was selected. The high-level expression of RBD protein was achieved by optimizing the fermentation process, including the salt concentration adjusting of the medium and induction condition optimization (pH, temperature and duration). The immunogenicity of the expressed RBD protein was evaluated in a mouse model. Results:A single clone with a high expression level of RBD protein was obtained and named RBD-X33. The expression level of RBD protein in the fermentation supernatant reached up to 240 mg/L after optimization of the induction condition (HBSM medium, pH=6.5±0.3, 22℃ and 120 h). In the mouse experiment, the recombinant RBD protein was formulated with Alum+ CpG dual adjuvant and injected into mice. The binding IgG antibody levels were up to 2.7×10 6 tested by ELISA and the neutralizing antibody levels were up to 726.8 tested by live virus neutralizing antibody assay (prototype). Conclusions:The RBD protein could be efficiently expressed in Pichia pastoris and induce stronger immune response in animals. This study suggested that the recombinant SARS-CoV-2 RBD protein expressed in Pichia pastoris could serve as a candidate antigen in the development of SARS-CoV-2 vaccine.

Congenital generalized lipodystrophy type 1 (CGL1) is an autosomal recessive genetic disease caused by mutations in AGPAT2 gene. The main clinical mainifestations include body subcutaneous fat loss, muscle hypertrophy, obvious subcutaneous veins, pseudoacromegaly, hirsutism, and acanthosis nigricans. What′s more, CGL1 is always accompanied by metabolic diseases. Therefore, it is easily misdiagnosed as metabolic syndrome, type 2 diabetes, polycystic ovary syndrome, acromegaly, or Cushing′s syndrome. Meanwhile, it is difficult to distinguish it from partial lipoatrophy syndrome. In this article, we present clinical and molecular characteristics of a patient with CGL1 and review mutations reported in literature to replenish current knowledge about this orphan disease.

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.