OBJECTIVE To investigate the current situation of pharmaceutical management in compact medical consortium of Guangdong province， and to provide decision-making basis for promoting the high-quality construction and sustainable development of the provincial medical consortium. METHODS A self-designed questionnaire was used to select 50 compact medical consortiums in Guangdong province. The survey was answered by the heads of the pharmacy department of the general hospitals. The survey covered the basic scale of the consortium， the appointment of chief pharmacists， the implementation of pharmaceutical management and pharmaceutical care homogenization within the consortium， the difficulties in promoting the homogenization， and the expected provincial support. Descriptive statistical analysis was performed on the survey results. RESULTS A total of 50 questionnaires were collected， and the effective recovery rate was 100%. There were 16 chief pharmacists （32.00%） in charge of the pharmacy department of the general hospital in the medical consortium. Thirty-seven medical consortiums （74.00%） had established a drug supply support system within the consortium， 35 medical consortiums （70.00%） had carried out pharmaceutical management and coordination work within the medical consortium， 23 medical consortiums （46.00%） had established a clinical medication guidance system， 25 medical consortiums chenwenying2016@163.com （50.00%） had established a bidirectional communication mechanism， and only 8 medical consortiums （16.00%） had developed new models of pharmaceutical care. At present， the difficulties in promoting the homogenization of pharmaceutical management and pharmaceutical care within the medical consortium were mainly found in three aspects： the wide gap in management level of each member unit， the lack and uneven level of pharmaceutical personnel， and insufficient policy support and implementation. Most medical consortiums hoped that relevant departments could promote the homogenization of pharmaceutical work by holding special training courses or special supervision. CONCLUSIONS At present， the compact medical consortium in Guangdong province has achieved initial results in the implementation of the chief pharmacist system， the homogenization of pharmaceutical management and pharmaceutical care. However， it is still necessary to improve the coverage of chief pharmacist appointments in the medical consortium， implement the homogenization of pharmaceutical management， and accelerate the homogenization process of pharmaceutical care.

Objective:To analyze the clinical characteristics of idiopathic systemic capillary leak syndrome (ISCLS).Methods:This retrospective case series analysis included 10 patients with confirmed ISCLS who were hospitalized at Peking Union Medical College Hospital from January 1983 to August 2023. Data collected encompassed general information, symptoms, laboratory results, treatment, and prognosis.Results:Among 10 ISCLS patients, 8 were males and 2 were females. The age of onset ranged from 29 to 63 years, and it was ≥40 years in 6 cases. Nine patients had nonspecific prodromal symptoms. All 10 experienced hypotension, hemoconcentration, hypoalbuminemia, and acute kidney injury during the leakage phase, with hemodynamic recovery in the subsequent phase. Neurological abnormalities were observed in 6 patients. Three patients had coagulation disorders, and 4 showed elevated levels of IL-6, IL-8, and TNF-α. M protein was found in all patients, with 8 showing IgGκ and 2 IgGλ. Acute-phase treatment primarily involved fluid resuscitation and vasopressors. In the stable phase, 4 patients had fewer episodes with anti-plasma cell therapy; 2 continued to have episodes despite intravenous immunoglobulin (IVIG); 2 had poor outcomes with theophylline; 1 improved without treatment; and 1 was lost to follow-up.Conclusions:ISCLS is a rare disease with adult-onset, presenting nonspecific prodromal symptoms and marked hypotension during episodes. All patients had monoclonal gammopathy. Effective treatment for the stable phase is lacking, though anti-plasma cell therapy may offer some benefit.

Objective To improve the understanding of hypereosinophilic syndrome(HES)complicated with pe-ripheral arterial embolism through analyzing the clinical features and therapeutic effect.Methods Among 176 inpa-tients with peripheral arterial embolism in Peking Union Medical College Hospital from April 2012 to October 2021,13 patients complicated with HES were involved according to the inclusion and exclusion criteria.All patients were diagnosed with peripheral arterial embolism by imaging examination.After gender and age matching,39 HES patients who were hospitalized in the same period but without peripheral arterial embolism were selected as the control with the ratio of 1:3.Clinical features,therapy response and prognosis were compared between the two groups.Results Eleven of the 13 patients were male.The main involved arteries included popliteal artery,dorsalis pedis artery,ulnar artery and radial artery.The proportion of peripheral nerve involvement in case group was significantly higher than in the control group(P＜0.05);and the proportion of gastrointestinal involvement(P＜0.05)and eosinophil level(P＜0.05)were significantly lower than that of control group.There were 92.3％,61.5％and 76.9％of the patients in case group received anticoagulant therapy,antiplatelet therapy and immunosuppressive therapy respectively,the percentages were significantly higher than that in control group(P＜0.01).According to eosinophil counts before and after therapy,84.6％and 56.5％of the patients in the case and control groups achieved complete remission and the partial remission rates were 15.4％and 25.6％re-spectively.Conclusions Patients with hypereosinophilia syndrome and peripheral arterial embolism commonly ex-perience involvement of the peripheral nerves and skin.Vascular embolism is mainly caused by arterial emboliza-tion of the extremities.After treatment with hormones,immunosuppressants,anticoagulants and/or antiplatelet therapy,this group of patients has a good prognosis.

Blau syndrome is a rare genetic disorder characterized by the a mix of granulomatous arthritis, uveitis, and dermatitis. Patients typically manifest multisystem involvement, including ocular, skin, and skeletal abnormalities. Blau syndrome is extremely rare, with a global incidence of less than one in a million among children. In this multidisciplinary consultation, we present a case of a 21-year-old young female patient having multisystemic involvement since early childhood. She was presented with multiple joint swelling, skin lesions, increased eye discharge, and accompanied by hypertension and arterial abnormalities, and received a diagnosis of uveitis. The patient had been receiving steroid treatment since the age of 6 and has tried various medications, with some improvement in joint swelling and ocular symptoms. Through this rare disease multidisciplinary consultation, we aim to provide guidance in the molecular diagnosis of the patient, multisystem assessment, and the selection and formulation of treatment plans. Additionally, we hope that by reporting this case, clinical physicians can gain a better understanding of the diagnosis and comprehensive treatment strategies for Blau syndrome, thereby improving the management and treatment of rare diseases.

We wished to summarize the clinical features of common variable immunodeficiency (CVID) complicated by non-cirrhotic portal hypertension (NCPH) and to deepen our understanding of it. The case data of CVID complicated with NCPH admitted to Peking Union Medical College Hospital from January 1983 to May 2021 were analyzed retrospectively to summarize their clinical characteristics. Six patients with CVID combined with NCPH (three of each sex; 16-45 years) were assessed. Four patients had portal hypertension. All patients had anemia, splenomegaly, a normal serum level of albumin and transaminases, and possibly increased levels of alkaline phosphatase and gamma-glutamyl transpeptidase. Two patients were diagnosed with esophagogastric fundic varices by gastroscopy. Two patients underwent splenectomy (which improved hematologic abnormalities partially). Four patients had autoimmune disease. Two cases were diagnosed with nodular regenerative hyperplasia (NRH) upon liver biopsy. Six patients were administered intravenous immunoglobulin-G (0.4-0.6 g/kg bodyweight) once every 3-4 weeks as basic therapy. Often, CVID complicated with NCPH has: (1) The manifestations of portal hypertension as the primary symptom. (2) Autoimmune-related manifestations. Imaging can provide important diagnostic clues. The etiology may be related to hepatic NRH and splenomegaly due to recurrent infections.

Objective:To analyze the clinical characteristics of hospitalized premenopausal patients with hyperuricemia.Methods:The medical records of premenopausal women with hyperuricemia (serum uric acid ≥360 μmol/L during hospitalization) admitted in Peking Union Medical College Hospital from 2013 to 2018 were reviewed and the clinical data were analyzed.Results:A total of 2 099 patients were enrolled. Only 14.01% (294 cases) of the patients were concerned about hyperuricemia by physicians. Autoimmune diseases (32.11%, 674 cases), nephrotic disease (19.29%, 405 cases) and endocrine system diseases (9.72%, 204 cases) are the main reasons for hospitalization, while 6.34%(133 cases) of patients were in gestation. In terms of the etiology, renal diseases (49.35%, 1 035 cases), specific drug use (49.26%, 1 034 cases) were the main causes of secondary hyperuricemia in premenopausal women, followed by metabolic diseases (10.62%, 233 cases). There was no significant difference in serum uric acid level among premenopausal women of different ages ( H=4.47, P=0.107), but the etiology of hyperuricemia among patients of different ages had significant differences. The proportion of hyperuricemia in patients with cancer and metabolic syndrome,secondary to use of diuretics and anti-tuberculosis drugs had differences among different age groups ( χ2=90.96,52.89,19.26 and 6.41, P<0.05). Conclusion:Hyperuricemia is not uncommon in premenopausal women. There are many secondary factors leading to hyperuricemia in premenopausal women, among which drugs and renal lesions are the main causes. In addition, the secondary factors in women with hyperuricemia has differences among different age groups.

Objective:To investigate the role of dendritic cells (DC) in Chlamydia muridarum ( Cm) respiratory infection and their effect on adaptive immune response. Methods:C57BL/6 mice were exposed to 1×10 3 inclusion-forming units (IFU) of Cm through inhalation to establish the mouse model of Cm respiratory infection. The proportion of CD11c + MHCⅡ + DC and the expression of costimulatory molecules (CD40, CD80 and CD86) in spleen tissues were detected by flow cytometry on 0, 3 and 7 d after infection. The expression of IL-12p40, IL-10 and IL-6 at mRNA level in spleen tissues was detected by qPCR. Mouse splenic DC isolated on 7 d after Cm infection were sorted by magnetic beads and then transferred to recipient mice. Th1 response in the recipient mice was measured using intracellular cytokine staining 14 d after infection. Results:Cm respiratory infection induced massive infiltration of DC and promoted the expression of costimulatory molecules on splenic DC. The expression of IL-12 and IL-10 at mRNA level in splenic DC reached the peak on 3 d after infection. Transferring the splenic DC of Cm-infected mice into the recipient mice could alleviate the disease condition in the recipient mice after Cm infection with reduced Cm inclusion-forming units in lung tissues and significantly increased proportion of Th1 cells in lung and spleen tissues. Conclusions:Cm respiratory infection could induce the maturation and activation of DC, which promoted Th1 immune response. DC played an important role in Cm infection.

Objective:To explore the impact of Coronavirus disease 2019 (COVID-19) epidemic on career choosing perspective among medical students and to analyze the related factors.Methods:Semi-structured telephone interviews were conducted during March 1-25 2020 among 19 medical students of 8-year program from Peking Union Medical College. The grounded theory and thematic analysis were applied to code the data and identify categories and factors.Results:Among the 19 respondents aged 19-26 years, 9 were males and 10 were female; 10 were at the clinical stage, and 9 were at the premedical stage; 3 respondents had family members involved in medical profession. Thematic analysis identified 6 main categories that affect the variability of medical students' career prospects under the COVID-19 pandemic. The 6 themes were individual characteristics of students; occupational characteristics; systemic factors; COVID-19 events; stressors of physicians and influence of job satisfaction.The outbreak affected everyone's mind of future career to varying degrees. The participants had been exposed to more negative aspects, while only one participant changed her career intention. There were conflicting views on whether to choose some specialties in future, such as respiratory medicine, infectious disease and critical care medicine. The participants feel more pressure as a doctor from the attitude of the public. Almost all participants mentioned feeling unsafe due to the high risk of occupational exposure and doctor-patient relationships. Most valued the support from their family, faculty, classmates, and volunteers. Many participants expressed their hope to improve the medical policies and systems.Conclusions:The influence of COVID-19 outbreak on medical students' career choosing can be positive as well as negative in different degrees. However, we found no evidence that it altered their perspectives substantially.

【Objective】 To investigate the quality changes of suspended red blood cells (SRBCs) prepared from the blood of Tibetan high Hb population, and explore the availability and safety of blood collected from Tibetan high Hb population. 【Methods】 The voluntary blood donors were grouped according to the Hb concentration at the initial screening: female blood donors from Tibet Autonomous Region (>3 500 m) with Hb≥190 g/L and male blood donors with Hb≥210 g/L were classified as plateau high hemoglobin group. A total of 13 male blood donors from Tibet Autonomous Region were recruited. And the female blood donors (n=13) with Hb(115~165) g/L and male blood donors (n=12) with Hb(120~185) g/L from Chengdu were classified as control group. Whole blood of 200 mL specification was centrifuged to remove the plasma, and MAP additive solution was added to prepare SRBCs, then SRBCs were divided into four aliquots (50 mL/bag and stored at 4℃. Parameters as blood routine, free Hb and hemolysis rate were measured aseptically at day 1, 14, 21, 35 of storage. And 10 mL SRBCs was used to extract membrane proteins for tyrosine phosphorylation detection of band 3 protein. 【Results】 The RBCs counts(×10¹²/L), hematocrit(%) and hemoglobin(g/L) of Tibetan high Hb group and control group were 6.76±0.95 vs 4.65±0.52, 63.3±6.8 vs 43.1±4.4 and 214.4±19.8 vs 143.2±16.9 (P<0.01). The erythrocyte deformability test on the day 1, 14, 21, 35 of storage showed that the deformability of SRBCs prepared from Tibetan high Hb group was significantly lower than that of the control group under shear stress of 3, 5.33, 9.49, 16.87, and 30 Pa, while the hemolysis rate of SRBCs prepared from the Tibetan high Hb group and the control group on the day 1, 14, 21, 35 were 0.050 2±0.040 2 vs 0.022 2±0.011 1, 0.055 4±0.043 vs 0.032 1±0.028 7, 0.061 2±0.025 9 vs 0.034 3±0.031 7 and 0.069 6±0.032 0 vs 0.044 0±0.033 3 (P<0.05). Western blotting showed that the cytoplasmic N-terminal Y21 of band 3 protein of SRBCs prepared from Tibetan high Hb group was highly phosphorylated. 【Conclusion】 The deformability of SRBCs prepared from the Tibetan high Hb group was significantly lower while the hemolysis rate of SRBCs was higher than that of the control group. The hemolysis rate of the SRBCs at the end of storage prepared from the Tibetan high Hb group meets the requirements of the national standard GB18469-2012(<0.8%). The increase of hemolysis rate of SRBCs prepared from the Tibetan high Hb group was closely related to the phosphorylation of band 3 protein.

We presented an adolescent with recurrent intracranial hemorrhage and skin lesion. The diagnosis was unclear and the treatment was difficult. Through a multidisciplinary effort type Ⅰ interferon disease was suspected and later, an interferon-stimulated gene was further detected. Considering the high morbidity and fatality rate of recurrent intracranial hemorrhage, tofacitinib and hydroxychloroquine were administered. After treatment, the livedo reticularis was significantly regressed. Unfortunately, the intracranial hemorrhage recurred due to a pre-existing cerebral aneurysm, leading to death of the patient. The diagnosis and treatment of this case highlight the importance of multidisciplinary collaboration in the diagnosis and treatment of difficult and rare diseases.