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.

Objective To analyze and compare the treatment of HeppleⅤ talus osteochondral injury(OLT)with autologous periosteal iliac bone graft and allogeneic bone powder combined with platelet rich gel(PRP).Methods Totally 62 HeppleⅤOLT patients admitted to our hospital from October 2018 to October 2022 were selected as the research subjects.They were divided into a transplantation group(31 patients received autologous periosteal iliac bone transplantation treatment)and a combination group(31 patients received allogeneic bone powder combined with PRP treatment)based on their treatment methods.Conduct a 12 month postoperative outpatient follow-up study on patients,evaluate and compare the treatment effectiveness,ankle joint range of motion(ROM),American Society of Orthopedic Foot and Ankle Surgeons(AOFAS)ankle posterior foot score,pain score,satisfaction,and incidence of complications between the two groups of patients at 12 months after surgery.Results The total effective rate of the transplantation group(96.77%)was not significantly different from that of the combination group(93.55%,P>0.05).At 12 months after surgery,the ROM and AOFAS scores of both groups improved(P<0.05),and there was no statistically significant difference between the groups(P>0.05).At 1 month,3 months,6 months,and 12 months after surgery,the pain scores of both groups decreased compared to before surgery(P<0.05).The subjective overall satisfaction of patients in the transplantation group(77.42%)was lower than that in the combination group(96.77%,P<0.05).The total incidence of complications in the transplantation group(19.35%)was significantly higher than that in the combination group(3.23%,P<0.05).Conclusion Allogeneic bone powder combined with PRP can avoid additional surgical incisions,increase patient subjective satisfaction,and increase the incidence of postoperative complications.

ObjectiveTo analyze the characteristics and changing trend of injury cause of mortality of residents in Qingpu District from 2002 to 2020， and to provide scientific reference for formulating regional prevention and control measures. MethodsThe injury mortality data of the registered residents in Qingpu District from 2002 to 2020 were collected. The indicators such as crude mortality rate， standardized mortality rate， and the ranking of causes of death were calculated. ResultsFrom 2002 to 2020， the average annual crude mortality rate was 50.27/100 000， the age-standardized mortality rate based on the world standard population（ASRW） was 30.08/100 000， and the age-standardized mortality rate based on the 2010 Chinese census（ASMRC） was 35.58/100 000. The average annual crude mortality rate of males was higher than that of females ［Z=54.402， Mantel-Hanszel χ²=1 742.509， P<0.01）. The overall injury mortality rate showed a downward trend with an average annual percent change（AAPC）of -4.07% （95%CI： -5.23%‒-2.90%）， P<0.001］. The top four causes of injury death were transportation accident， indeliberate fall， drowning， and suicide. The leading causes of death in 0‒ years old， 15‒ years old and ≥65 years old were drowning， transportation accident and indeliberate fall， respectively. The ASRW of transportation accident， drowning and suicide all showed a decreasing trend， and the AAPC were -8.22% （95%CI： -10.16%‒-6.24%）， -6.99% （95%CI： -9.68%‒-4.22%） and -6.21% （95%CI： -9.38%‒-2.94%）， respectively. ConclusionThe injury death rate of residents in Qingpu District shows a decreasing trend， and the distribution characteristics of injury death are different among different genders and age groups. Corresponding prevention and control strategies should be adopted for different populations.

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.

Objective:To explore the efficacy and safety of endovascular intervention combined with preoperative laparoscopic exploration in the treatment of patients with acute mesenteric artery ischemia.Methods:This was a prospective cohort study (NCT04686981). The study enrolled 31 patients with acute mesenteric artery ischemia from Oct 1, 2020 to Oct 1, 2022. Among them, 26 patients (84%) were male, with a mean age of (67±13) years and a mean time to onset of (21±8) hours. All patients underwent laparoscopic exploration in the hybrid operating room. If the presence of intestinal necrosis or suspected necrosis was clearly determined, the patient would undergo open surgery (mesenteric artery embolization, intestinal resection and intestinal double stoma) as the treatment by gastrointestinal surgeon. If intestinal necrosis or suspected necrosis was not found by laparoscopy, the patient would undergo endovascular intervention by vascular surgeon. The primary observational endpoints of this study were the proportion of patients who were not dependent on total parenteral nutrition and all-cause mortality within 30 days after operation. The secondary observational endpoints were the rate of mesenteric vascular patency within 30 days and the proportion of interventions that were converted to open surgery.Results:Six patients underwent open surgery and 25 patients underwent endovascular intervention, including 13 cases of thrombus reduction alone, 3 cases of stent implantation during the same period after reduction, and 9 cases of stent implantation alone. Twenty-four patients (77%) were completely weaned from the TPN within 30 days after the procedure, and all-cause mortality was observed in 3 cases (9.7%). The patency rate of the mesenteric artery within 30 days after the procedure was 82.1%. The rate of conversion to open surgery after intervention was 16%.Conclusions:Endovascular intervention combined with preoperative laparoscopic exploration can clarify intestinal ischemia in acute mesenteric patients as early as possible, and individualized treatment strategies for each patient by multidisciplinary care team can potentially improve the prognosis of such patients.

ObjectiveTo analyze the trends in life expectancy and cause-eliminated life expectancy of the registered residents in Qingpu District, Shanghai from 2002 to 2021, and to identify the major diseases causing life expectancy loss in the area, so as to provide a theoretical basis for strengthening disease prevention and interventions and optimizing health resources allocation. MethodsUsing the International Classification of Diseases (ICD)-10 classification of death causes, average life expectancy and cause-eliminated life expectancy were calculated with the abbreviated life table and the cause-eliminated life expectancy table. A trend forecast of life expectancy from 2022 to 2029 was made. ResultsFrom 2002 to 2021, the life expectancy of Qingpu District residents showed an increasing trend, with an average annual growth of 0.32 years (AAPC=0.39%, P<0.001). Among the seven major causes of death, the cause-eliminated expected life expectancy for circulatory system diseases showed the most significant upward trend (AAPC=0.53%, P<0.001), rising from the third leading cause of death in 2002 to the first in 2021. The life expectancy loss rate due to circulatory system diseases increased from 3.79% to 7.97%. Respiratory system diseases showed the largest decline, decreasing from the first leading cause of death in 2002 to the fourth in 2021, with the life expectancy loss rate decreasing from 6.83% to 0.99%. ConclusionLife expectancy of the registered residents in Qingpu District has reached a leading level in China. Future efforts should focus on effectively increasing the life expectancy of male residents, strengthening the comprehensive prevention and treatment of chronic diseases such as cerebrovascular diseases, diabetes, and malignant tumors, and improving health promotion policies for residents to achieve a steady long-term increase in regional life expectancy.

Lead is one of the most important occupational hazards in China, and occupational exposure is the leading cause of lead poisoning. Lead can be absorbed by the body through air, food, drinking water and skin, and accumulate in multiple organs in the body, posing health risks to humans, especially to lead workers. Many previous studies have shown that lead can affect the function of glial cells such as microglia, astrocytes and oligodendrocytes, resulting in irreversible neurological damage. This article provides an overview of the neurotoxic mechanism induced by lead through glial cells, elucidates that lead can induce neurodegenerative diseases such as Alzheimer′s disease, Parkinson′s disease, and amyotrophic lateral sclerosis, and reviews the relationship between lead and glial cells, in order to provide reference for further research on the neurotoxic mechanism of lead on glial cells.

Lead is one of the most important occupational hazards in China, and occupational exposure is the leading cause of lead poisoning. Lead can be absorbed by the body through air, food, drinking water and skin, and accumulate in multiple organs in the body, posing health risks to humans, especially to lead workers. Many previous studies have shown that lead can affect the function of glial cells such as microglia, astrocytes and oligodendrocytes, resulting in irreversible neurological damage. This article provides an overview of the neurotoxic mechanism induced by lead through glial cells, elucidates that lead can induce neurodegenerative diseases such as Alzheimer′s disease, Parkinson′s disease, and amyotrophic lateral sclerosis, and reviews the relationship between lead and glial cells, in order to provide reference for further research on the neurotoxic mechanism of lead on glial cells.

Aim To explore the effect of Pueraria Lobata Flowers Extract(PFE)on lipid accumulation in mac-rophage-derived foam cells.Methods The concentration of PFE in THP-1-derived foam cells was screened by MTT,intracellular lipid accumulation was detected by oil red O staining and cholesterol detection kit,intracellular cholesterol ef-flux levels were detected by cholesterol efflux assay kit,RT-qPCR and Western blot were used to analyze mRNA and pro-tein expression.Results PFE significantly reduced lipid accumulation in THP-1-derived foam cells.PFE did not affect the mRNA expression of CD36,scavenger receptor-A Ⅰ(SR-A Ⅰ),sterol regulatory element-binding protein 2(SREBP2),3-hydroxy-3-methylglutaryl-CoA reductase(HMGCR),but it could upregulate the mRNA and protein expres-sion levels of ATP-binding cassette transporter A1(ABCA1)(P＜0.05),and promote the intracellular cholesterol efflux of macrophage-derived foam cells(P＜0.01).PFE could activate the activity of peroxisome proliferator-activated receptor y(PPARγ)(P＜0.01)and upregulate the mRNA and protein expression levels of PPARγ(P＜0.05).Compared with the PFE control group,the expression of PPARγ and ABCA1 proteins decreased and cholesterol efflux decreased after GW9662 treatment(all P＜0.01).Conclusion PFE could significantly prevent the lipid accumulation in THP-1-derived foam cells and inhibit the formation of foam cells by upregulating ABCA1 expression and cholesterol efflux mediated by PPARγ.

Platelet count is an important basis for clinical diagnosis and treatment decisions,and accurate platelet count can assist in making correct clinical diagnosis and taking effective treatment measures in clinical practice.The common detection methods for platelet counting were reviewed,including peripheral blood smear platelet estimation,sheath flow impedance,optical method,microscope digital imaging technology and flow cytometry indirect counting.The advantages and disadvantages and the application conditions of the above methods were analyzed,and the new detection technology of platelet count was introduced,it provides ideas for developing new platelet counting methods.