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.

The Ehlers-Danlos syndrome(EDS)is a rare inherent connective tissue disorder.The prev-alence of EDS in the population is estimated at one out of ten thousand to one out of a hundred thousand.The vascular EDS(vEDS)are rare among the subtypes but are the worst in prognosis.The article reports a case of vEDS admitted to the hospital.The patient was a young man complaining of a sudden onset of aphasia in right hemiparalysis and severe left abdominal pain for unknown reasons.The diagnosis was made after the genetic testing.The patient suffered from vEDS.Then,the multi-disciplinary team(MDT)made a treatment plan tailored to this young patient.The complexity in classification and delusive presentations of the EDS make the correct diagnosis very challenging.This article hopes to report this case and to share the experiences to the bet-ter understanding of this disease.

Objective:The primary goal of this study is to explore the safety and effectiveness of a new modified eversion carotid endarterectomy (MECEA).Methods:This is a retrospective case series study. One hundred patients were consecutively treated with MECEA by the same operator at Department of Vascular Surgery,Peking Union Medical College Hospital from January 2019 to December 2023. There were 77 males and 23 females. The age was (66.0±8.6)years (range: 39 to 85 years). Twenty-four (24.0%) patients were symptomatic with the degree of carotid stenosis over 50%,76 patients (76.0%) were asymptomatic with the degree of stenosis over 70%. All these patients meet the indication of carotid endarterectomy. The main difference between MECEA and traditional eversion carotid endarterectomy was the anterior,lateral,and posterior walls of the internal carotid artery were incised obliquely from the origin of the internal carotid artery toward the common carotid artery,leaving the wall of internal carotid artery intact at the bifurcation. The surgical process,cardiovascular and cerebrovascular complications and other surgical complications were recorded. The incidences of complications,restenosis of intraoperative target lesions and re-intervention were collected during follow-up.Results:All procedures were performed successfully under general anesthesia. The total operation time was (36.5±10.1)minutes (range: 22 to 65 minutes),and carotid clamping time was (15.0±6.3)minutes (range: 7 to 31 minutes). One patient (1.0%) occurred postoperative cerebrovascular accident,1 patient (1.0%) developed cerebral hyperperfusion syndrome (CHS),and another 1 patient (1.0%) suffered myocardial infarction. All these patients were recovered after medical treatment within a week. The follow-up time( M(IQR)) was 24 (28) months (range: 6 to 62 months). Two patients (2.0%) were reported to have hemodynamically significant restenosis within 2 years,with one patient requiring intervention. No patient suffered from ipsilateral ischemic stroke. Conclusions:MECEA is a safe and effective surgical method of treating carotid artery stenosis. This method can reduce carotid clamping time and lowers the risk of ischemic stroke. Meantime,it preserves the integrity of the adventitia at the bifurcation of carotid artery,reduces the chance of restenosis. Moreover,it might be helpful to prevent postoperative CHS due to reducing damage to the carotid body and carotid sinus nerve.

Objective:The primary goal of this study is to explore the safety and effectiveness of a new modified eversion carotid endarterectomy (MECEA).Methods:This is a retrospective case series study. One hundred patients were consecutively treated with MECEA by the same operator at Department of Vascular Surgery,Peking Union Medical College Hospital from January 2019 to December 2023. There were 77 males and 23 females. The age was (66.0±8.6)years (range: 39 to 85 years). Twenty-four (24.0%) patients were symptomatic with the degree of carotid stenosis over 50%,76 patients (76.0%) were asymptomatic with the degree of stenosis over 70%. All these patients meet the indication of carotid endarterectomy. The main difference between MECEA and traditional eversion carotid endarterectomy was the anterior,lateral,and posterior walls of the internal carotid artery were incised obliquely from the origin of the internal carotid artery toward the common carotid artery,leaving the wall of internal carotid artery intact at the bifurcation. The surgical process,cardiovascular and cerebrovascular complications and other surgical complications were recorded. The incidences of complications,restenosis of intraoperative target lesions and re-intervention were collected during follow-up.Results:All procedures were performed successfully under general anesthesia. The total operation time was (36.5±10.1)minutes (range: 22 to 65 minutes),and carotid clamping time was (15.0±6.3)minutes (range: 7 to 31 minutes). One patient (1.0%) occurred postoperative cerebrovascular accident,1 patient (1.0%) developed cerebral hyperperfusion syndrome (CHS),and another 1 patient (1.0%) suffered myocardial infarction. All these patients were recovered after medical treatment within a week. The follow-up time( M(IQR)) was 24 (28) months (range: 6 to 62 months). Two patients (2.0%) were reported to have hemodynamically significant restenosis within 2 years,with one patient requiring intervention. No patient suffered from ipsilateral ischemic stroke. Conclusions:MECEA is a safe and effective surgical method of treating carotid artery stenosis. This method can reduce carotid clamping time and lowers the risk of ischemic stroke. Meantime,it preserves the integrity of the adventitia at the bifurcation of carotid artery,reduces the chance of restenosis. Moreover,it might be helpful to prevent postoperative CHS due to reducing damage to the carotid body and carotid sinus nerve.

Objective:To evaluate a surgical approach for partial resection of the tenth rib through a retroperitoneal approach for the exposure of Crawford type IV thoracoabdominal aortic aneurysm and complex abdominal aortic aneurysm from 2014 to 2019.Methods:A retrospective analysis was conducted on clinical data and follow-up results of 7 patients who underwent treatment for Crawford type IV thoracoabdominal aortic aneurysm and complex abdominal aortic aneurysm through partial resection of the tenth rib via a retroperitoneal approach.Results:One case (14.3%) had associated Marfan syndrome, and 5 cases (71.4%) underwent left renal artery reconstruction. None of the patients experienced severe complications such as cardiopulmonary complications or renal failure postoperatively, and there was no statistically significant difference in serum creatinine levels between preoperative and postoperative stages during hospitalization ( P=0.205). Follow-up examinations showed no long-term vascular stenosis. Conclusions:Partial resection of the tenth rib through a retroperitoneal approach can avoid incisions of the pleura and diaphragm. It allows for the exposure of the aorta below the diaphragm and has the ability to treat aortic diseases below the diaphragm with smaller incisions and lower complication risks.

In the past five years,both advancements and new problems were seen in the treatment of lower extremity arteriosclerosis obliterans.The Global Vascular Guidelines published in 2019 have given us comprehensive suggestions for the diagnosis and treatment of critical limb threatening ischemia(CLTI),but the grading and treatment suggestions for CLTI should be generalized.As to endovascular treatment,drug coated balloons have been found to be effective for limb salvage and graft patency in femoropopliteal and infra-popliteal artery occlusive diseases.As to surgical revascularization,persistent education and surveillance are necessary to maintain the practical quality of this fundamental technique.Inframalleolar bypass could achieve good graft patency and limb salvage rate for in CLTI patients.Regional anesthesia has been found to have lower risk than general anesthesia for lower extremity surgical revascularization.Percutaneous deep vein arterialization might be helpful for limb salvage in patients with non-option CLTI.A brief review about the treatment of lower extremity arteriosclerosis obliterans is performed based on latest literatures and institutional experience.Understanding the present situation and development trend is important for peripheral vascular practitioners

Abdominal aortic aneurysm (AAA) and atherosclerosis (AS) have considerable similarities in clinical risk factors and molecular pathogenesis. The aim of our study was to investigate the differences between AAA and AS from the perspective of metabolomics, and to explore the potential mechanisms of differential metabolites via integration analysis with transcriptomics. Plasma samples from 32 AAA and 32 AS patients were applied to characterize the metabolite profiles using untargeted liquid chromatography-mass spectrometry (LC-MS). A total of 18 remarkably different metabolites were identified, and a combination of seven metabolites could potentially serve as a biomarker to distinguish AAA and AS, with an area under the curve (AUC) of 0.93. Subsequently, we analyzed both the metabolomics and transcriptomics data and found that seven metabolites, especially 2'-deoxy-D-ribose (2dDR), were significantly correlated with differentially expressed genes. In conclusion, our study presents a comprehensive landscape of plasma metabolites in AAA and AS patients, and provides a research direction for pathogenetic mechanisms in atherosclerotic AAA.

In the past five years,both advancements and new problems were seen in the treatment of lower extremity arteriosclerosis obliterans.The Global Vascular Guidelines published in 2019 have given us comprehensive suggestions for the diagnosis and treatment of critical limb threatening ischemia(CLTI),but the grading and treatment suggestions for CLTI should be generalized.As to endovascular treatment,drug coated balloons have been found to be effective for limb salvage and graft patency in femoropopliteal and infra-popliteal artery occlusive diseases.As to surgical revascularization,persistent education and surveillance are necessary to maintain the practical quality of this fundamental technique.Inframalleolar bypass could achieve good graft patency and limb salvage rate for in CLTI patients.Regional anesthesia has been found to have lower risk than general anesthesia for lower extremity surgical revascularization.Percutaneous deep vein arterialization might be helpful for limb salvage in patients with non-option CLTI.A brief review about the treatment of lower extremity arteriosclerosis obliterans is performed based on latest literatures and institutional experience.Understanding the present situation and development trend is important for peripheral vascular practitioners

The inflammasome is a multiprotein complex localized in the cytoplasm.It can mediate the expressions of various inflammatory cytokines such as interleukin(IL)-1β and IL-18 and plays a key role in regulating inflammatory response.As sterile inflammation,abdominal aortic aneurysm currently can only be treated by surgery.This article reviews the research advances in the role of inflammasomes in abdominal aortic aneurysm.
Aortic Aneurysm, Abdominal ; Humans ; Inflammasomes ; Inflammation ; Interleukin-1beta ; NLR Family, Pyrin Domain-Containing 3 Protein

Objective:To summarize the surgical treatment of malignant carotid body tumor (MCBT).Methods:A retrospective analysis of 14 MCBT patients admitted at our hospital from Mar 2005 to Nov 2019 was made, and the imaging data, surgical records, perioperative complications and follow-up data were collected.Results:There were 8 males and 6 females, with an average age of (40.8±11.3) years. 10 patients underwent surgical resection of CBT, with one case undergoing tumor enucleation only, nine cases underwent internal carotid artery reconstruction, and all patients underwent intraoperative lymph node biopsy.Tumors were completely removed in all 10 patients. No perioperative death or cerebral infarction occurred. The intraoperative blood loss was (955±658.5) ml. Four patients had permanent nerve injury after surgery. The follow-up time ranged 1-132 months. There were no cases of cerebral infarction or death, and the reconstructed graft remained patent. Apart from the 2 patients who developed tumor metastasis after surgery, other patients recovered uneventfully with no disease progression.Conclusions:Surgery is still the main treatment for MCBT, but MCBT is large and Shamblin class is more advanced. Therefore, complete tumor removal and reconstruction of the carotid arteries are difficult. Surgery should seek to completely remove the tumor and neck lymph node biopsy should be performed to determine the lymph node metastasis.