A 20-year-old male patient presented to the Department of Dermatology of Peking Union Medical College Hospital with complaints of an 8-year history of facial scarring, swelling of the lower limbs, and a 4-year history of scalp thickening. Physical examination showed thickening furrowing wrinkling of the skin on the face and behind the ears, ciliary body hirsutism, blepharoptosis, and cutis verticis gyrate. Both lower limbs were swollen, especially the knees and ankles. The skin of the palms and soles of the feet was keratinized and thickened. Laboratory examination using bone and joint X-ray showed periostosis of the proximal middle phalanges and metacarpals of both hands, distal ulna and radius, tibia and fibula, distal femurs, and metatarsals.Genetic testing revealed two variants in SLCO2A1, c.1658T > A and c.96+4A > C.Through multidisciplinary consultation, we confirmed the diagnosis of pachydermoperiostosis.

Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired clonal hematopoietic stem cell disease caused by abnormal expression of glycosylphosphatidylinositol (GPI) on the cell membrane due to mutations in the phosphatidylinositol glycan class A(PIGA) gene. It is commonly characterized by intravascular hemolysis, repeated thrombosis, and bone marrow failure, as well as multiple systemic involvement symptoms such as renal dysfunction, pulmonary hypertension, swallowing difficulties, chest pain, abdominal pain, and erectile dysfunction. Due to the rarity of PNH and its strong heterogeneity in clinical manifestations, multidisciplinary collaboration is often required for diagnosis and treatment. Peking Union Medical College Hospital, relying on the rare disease diagnosis and treatment platform, has invited multidisciplinary clinical experts to form a unified opinion on the diagnosis and treatment of PNH, and formulated the Expert Consensus of Multidisciplinary Diagnosis and Treatment for Paroxysmal Nocturnal Hemoglobinuria (2024), with the hope of promoting the standardization of PNH diagnosis and treatment.

Background::Patients who undergo coronary artery bypass grafting (CABG) are known to be at a significant risk of experiencing long-term adverse events, emphasizing the importance of regular assessments. Evaluating health-related quality of life (HRQoL) serves as a direct method to gauge prognosis. Our objective is to ascertain the prognostic significance of consecutive HRQoL assessments using the Physical Component Summary (PCS) and Mental Component Summary (MCS) derived from the Short-Form 36 (SF-36) health survey in CABG patients.Methods::The study population consisted of 433 patients who underwent isolated elective CABG at Fuwai Hospital between 2012 and 2013. SF-36 assessments were conducted during both the hospitalization period and follow-up. The primary endpoint of the study was all-cause mortality, while the secondary outcome was a composite measure including death, myocardial infarction, stroke, and repeat revascularization. We assessed the relationships between the PCS and MCS at baseline, as well as their changes during the first 6 months after the surgery (referred to as ΔPCS and ΔMCS, respectively), and the observed outcomes.Results::The patients were followed for an average of 6.28 years, during which 35 individuals (35/433, 8.1%) died. After adjusting for clinical variables, it was observed that baseline MCS scores (hazard ratio [HR] for a 1-standard deviation [SD] decrease, 1.57; 95% confidence interval [CI], 1.07–2.30) and ΔMCS (HR for a 1-SD decrease, 1.67; 95% CI, 1.09–2.56) were associated with all-cause mortality. However, baseline PCS scores and ΔPCS did not exhibit a significant relationship with all-cause mortality. Notably, there was a dose-response relationship observed between ΔMCS and the likelihood of all-cause mortality (HRs for the 2nd, 3rd and 4th quartiles compared to the 1st quartile, 0.33, 0.45 and 0.11, respectively).Conclusions::Baseline MCS and changes in MCS were independent predictors for long-term mortality of CABG. Better mental health status and recovery indicated better prognosis.

A 31-year-old man sought medical evaluation for a 2-year history of edema and proteinuria, with prior pathology suggesting atypical membranous nephropathy (MN). Despite treatment with a combination of steroids, calcineurin inhibitors, and four courses of rituximab (1 g, intravenous injection), the patient′s nephrotic syndrome showed no relief (24 h urine protein peaked at 31.18 g/d), indicating refractory nephrotic syndrome. Later in the disease course, a sudden surge of creatinine level (322.5 μmol/L) prompted a renal biopsy, which revealed concurrent acute interstitial nephritis. Further treatment involving steroids, cyclophosphamide, and a fifth rituximab infusion (1 g, intravenous injection) resulted in improvement in renal function (serum creatinine: 322.5?147 μmol/L), but the MN failed to achieve partial relief. Subsequent treatment with the novel humanized CD20 monoclonal antibody obinutuzumab (1 g, intravenous injection) was initiated. In the latest follow-up, anti-phospholipase-A2-receptor antibody (PLA2R) antibody were negative, B cells were eliminated, serum albumin was 36 g/L, urine protein-to-creatinine ratio was 4 810 mg/g, and serum creatinine was 162 μmol/L. This case underscores the potential efficacy of obinutuzumab in refractory MN. For advanced MN cases, prompt identification of the cause of acute kidney injury is crucial, emphasizing the need for targeted interventions to potentially stall renal function decline.

A young female patient presented with fever, arthralgia, and rash was diagnosed with adults still's disease. When treated with glucocorticoid steroid, the above patient progressed to anuria, sudden, and confusion. After a teamwork involving different departments, the patient was finally diagnosed with atypical hemolytic uremic syndrome (aHUS) and treated with good outcome. aHUS is a rare disease, while Eculizumab is an orphan drug. The diagnosis and treatment of the patient reveals the importance of multidisciplinary team on the diagnosis and treatment of rare and difficult diseases.

Objective    To analyze the correlation between the gray value of epicardial fat and the prognosis of patients with atrial fibrillation (AF) treated by thoracoscopic radiofrequency ablation. Methods    The clinical data of 97 patients, including 75 males and 22 females with an average age of 57.8±9.4 years, who underwent thoracoscopic radiofrequency ablation in Fuwai Hospital from 2017 to 2018 were analyzed retrospectively. The left atrial fat volume and average gray scale were calculated by left atrial enhanced CT. According to the average gray scale of left atrial fat tissue, the patients were divided into three groups: a high gray scale group, a medium gray scale group and a low gray scale group. The patients were followed up at 3, 6 and 12 months after operation. The end point of follow-up was the recovery rate of sinus rhythm. Survival analysis was used to analyze the correlation between CT features of epicardial fat enhancement and prognosis. Results    After adjustment of body mass index, body surface area, gender and left atrial end diastolic diameter, regression analysis showed that the fat gray of left atrial enhanced CT was correlated with the type of AF (OR=0.30, 95%CI 0.12-0.79, P=0.014). Cox regression analysis showed that the fat gray value of left atrial CT predicted the recurrence of AF after thoracoscopic radiofrequency ablation (OR=0.92, 95%CI 0.85-0.99). The Kaplan-Meier curve showed significant difference in the long-term recurrence rate of AF among the three groups (P=0.011). The lower left atrial fat enhanced CT gray scale was, the higher long-term recurrence rate of AF was. Conclusion    The gray value of left atrial fat enhanced CT can effectively predict the recurrence of AF after radiofrequency ablation in thoracoscopic surgery.

Objective:To assess the value of urine heat-shock protein-70 (HSP-70) in the early diagnosis of acute kidney injury (AKI) after cardiac cardiopulmonary bypass (CPB).Methods:Patients with cardiopulmonary bypass from May 2018 to July 2018 in Henan Provincial People's Hospital were enrolled as subjects. Urine samples were collected before and after cardiopulmonary bypass at 0 h, 2 h, 4 h, 6 h, 8 h, 12 h, 24 h and 48 h. Patients were divided into AKI group and non-AKI group according to the Kidney Disease: Improving Global Outcomes Guide. Urinary HSP-70, tissue inhibitor of metalloproteinase-2 (TIMP-2) and insulin-like growth factor-binding protein 7 (IGFBP7) were detected by enzyme-linked immunosorbent assay (ELISA) and urine neutrophil gelatinase-associated lipocalin (NGAL) was determined by immunoturbidimetry. The receiver operating characteristic (ROC) curve was plotted to calculate the critical value, sensitivity and specificity of urine HSP-70, [TIMP-2]×[IGFBP7] and NGAL for the diagnosis of postoperative AKI after CPB.Results:A total of 45 patients were enrolled in the study. There were 24 cases in AKI group and 21 cases in non-AKI group. The level of urinary HSP-70, [TIMP-2]×[IGFBP7] and NGAL in AKI group were significantly higher than in the non-AKI group at each postoperative time point, with statistically significant differences (all P<0.05). The level of urinary HSP-70 in AKI group peaked at 2 h after CPB, which was significantly earlier than the peak time of urine [TIMP-2]×[IGFBP7] and urine NGAL (12 h after CBP and 4 h after CBP, respectively). Urinary HSP-70≥2.1 μg/L could predict postoperative AKI of CPB at 2 h after CPB, with the area under the curve ( AUC) of 1.00, the sensitivity of 100.0% and the specificity of 100.0%. Urinary [TIMP-2]×[IGFBP7]>19.1 μg 2/L 2 could predict postoperative AKI of CPB at 12 h after CPB with the AUC of 0.94, the sensitivity of 87.5%, and the specificity of 100.0%. Urinary NGAL>27.4 μg/L could predict postoperative AKI of CPB at 4 h after CPB with the AUC of 0.95, the sensitivity of 95.8%, and the specificity of 85.7%. The positive predictive value of urine HSP-70≥2.1 μg/L at 2 h after CPB was 100.0%, and the negative predictive value was 100.0%. Conclusions:The level of urinary HSP-70 increases earlier than that of urinary [TIMP-2]×[IGFBP7] and NGAL in patients with AKI after CPB. Clinical monitoring of urinary HSP-70 level contributes to early diagnosis of AKI.

Objective:To evaluate whether dialysis modality will affect cognitive function in dialysis population.Methods:This was a cross-sectional study. Chronic dialysis patients in our center was screened from July 2013 to July 2014. All of the subjects received brain magnetic resonance imaging (MRI) examination and comprehensive cognitive function evaluation.Results:A total of 189 chronic dialysis patients were enrolled in this study, 122 cases on hemodialysis (HD) and 67 cases on peritoneal dialysis (PD). There was no significant difference in age between HD and PD groups [(56.4±13.2) years vs (56.4±16.1) years, t=0.004, P=0.997]. The dialysis vintage and serum albumin of HD patients was higher than those of PD patients[58.0(16.8, 107.5) months vs 31.0(7.0, 67.0) months, Z=-3.490, P<0.001; (39.6±3.9) g/L vs (35.3±3.8) g/L, t=7.328, P<0.001, respectively]. The prevalence of cerebral small vessel diseases (CSVDs) was comparable between HD and PD groups (all P>0.05). Compared with HD patients, PD patients presented a 11.90-fold risk of immediate memory impairment (95% CI 1.40-101.08, P=0.023) and a 6.18-fold risk of long-delayed memory impairment (95% CI 2.12-18.05, P=0.001). After adjusting for age, educational lever, dialysis vintage, serum creatinine, and CSVDs, the influence of dialysis modality on memory still worked. PD patients presented a 43% risk of executive function impairment of HD patients ( OR=0.43, 95% CI 0.17-1.04, P=0.061). Conclusions:HD patients manifested better memory than PD patients, while PD probably performed better in executive function than HD patients. There was no significant difference in language function between the two groups. The difference in cognitive function may not be related to CSVDs.

By promoting the socialization of hospital logistics professional skill services, the logistics support ability and safety production supervision level can be improved. By using the theory and method of system dynamics, the income decision model of public-private-partnership projects in the energy station of Nankai hospital is established. Then, the qualitative factors in the decision-making model are transformed into calculable quantitative factors by using the mathematical method of fuzzy logic, and the reasonable charging interval negotiated between Nankai Hospital and the project company is given. According to the income range and in consideration of the balance of interests of all stakeholders, the concession period and the concession price are adjusted to provide decision-making and analysis reference for energy station construction projects.

Objectives To compare the clinical characteristics, long - term survival and associated risk factors of automated peritoneal dialysis (APD) patients and continuous ambulatory peritoneal dialysis (CAPD) patients. Methods As a retrospectively study, adult patients started peritoneal dialysis in Peking Union Medical College Hospital (PUMCH) from September 1st, 2002 to September 30th, 2016 were enrolled. Baseline information and dialysis associated parameters were collected. The primary outcome was death and the secondary outcome was technical failure. The risk factors of death were analyzed in APD patients by Cox's regression model. Homochromous gender and age matched CAPD patients were analyzed as control. Results The baseline condition of 69 APD patients were similar to those of 138 CAPD patients. The survival rates of APD patients at 1-year、3-year and 5-year were 95.4%, 88.0% and 73.0% respectively, which were superior to CAPD patients. No significant difference in technical survival was found between APD and CAPD patients. Single-factor Cox's regression analysis showed that all-cause mortality of CAPD patients was 2.2 times higher than that of APD patients (95% CI 1.221-3.837). In the multi-factor Cox regression analysis model, adjusted by age, complications (including cardiovascular disease and diabetes), nPCR and serum creatinine, dialysis modality was not an independent risk factor of dialysis patients. Age (HR=1.077, 95%CI 1.016-1.142, P=0.013), diabetes (HR=3.608, 95%CI 1.117-11.660, P=0.032) and serum albumin (HR=0.890, 95%CI 0.808-0.982, P=0.020) were independently associated with all-cause death of APD patients. Conclusions Dialysis modality was not an independent risk factor for the all-cause mortality of peritoneal dialysis patients. Age, diabetic nephropathy and hypoalbuminemia were independently associated with the death of APD patients.