Objective:Persistent proatlantal intersegmental artery (PPIA) is a rare variant of persistent carotid-vertebrobasilar anastomoses and mostly discovered incidentally. To explore the clinical features of PPIA, and enhance the understanding of this rare cerebrovascular variation.Methods:A retrospective analysis was performed. The clinical data of 6 patients with PPIA admitted to Department of Neurointervention, First Affiliated Hospital of Zhengzhou University from June 2018 to June 2024 were analyzed. The imaging features, associated cerebrovascular diseases, treatments and follow-up results were summarized.Results:Among the 6 PPIA patients, 4 were with type I and 2 were with type II; 5 were with left PPIA and 1 patient was with right PPIA; 5 patients had bilateral vertebral artery hypoplasia or absence, and 1 patient had absence of the ipsilateral vertebral artery and normal development of the contralateral vertebral artery. Four PPIA patients were combined with intracranial aneurysms: 2 patients received dual stent-assisted embolization and flow diverter device combined with covered stent implantation, respectively (after 6 months of follow-up, modified Rankin scale score of 0 indicating good prognosis and healed intracranial aneurysms), and the other 2 patients with micro-aneurysms were not treated. One patient was combined with internal carotid artery stenosis and accepted stent placement (good prognosis at 6 months after follow-up). One patient was combined with acute basilar artery tip occlusion and accepted aspiration thrombectomy (died of brain herniation 7 days after thrombectomy).Conclusions:PPIA is often accompanied by vertebral artery developmental abnormalities and prone to have concurrent intracranial aneurysms or ischemic stroke. When performing surgical treatment for cerebrovascular diseases associated with PPIA, PPIA protection to ensure blood supply of the posterior circulation is essential.

OBJECTIVES: To investigate the clinical characteristics and prognosis of acute erythroleukemia (AEL) in children. METHODS: A retrospective analysis was conducted on the clinical data, treatment, and prognosis of 8 children with AEL treated at the First Affiliated Hospital of Zhengzhou University from January 2013 to December 2023. RESULTS: Among the 7 patients with complete bone marrow morphological analysis, 4 exhibited trilineage dysplasia, with a 100% incidence of erythroid dysplasia (7/7), a 71% incidence of myeloid dysplasia (5/7), and a 57% incidence of megakaryocytic dysplasia (4/7). Immunophenotyping revealed that myeloid antigens were primarily expressed as CD13, CD33, CD117, CD38, and CD123, with 4 cases expressing erythroid antigens CD71 and 2 cases expressing CD235a. Chromosomal analysis indicated that 2 cases presented with abnormal karyotypes, including +8 in one case and +4 accompanied by +6 in another; no complex karyotypes were observed. Genetic abnormalities were detected in 4 cases, with fusion genes including one case each of dup MLL positive and EVI1 positive, as well as mutations involving KRAS, NRAS, WT1, and UBTF. Seven patients received chemotherapy, with 6 achieving remission after one course of treatment; 2 underwent hematopoietic stem cell transplantation, and all had disease-free survival. Follow-up (median follow-up time of 6 months) showed that only 3 patients survived (2 cases after hematopoietic stem cell transplantation and 1 case during treatment). CONCLUSIONS Children with AEL have unique clinical and biological characteristics, exhibit poor treatment response, and have a poor prognosis; however, hematopoietic stem cell transplantation may improve overall survival rates.
Humans ; Male ; Female ; Prognosis ; Child, Preschool ; Retrospective Studies ; Child ; Leukemia, Erythroblastic, Acute/diagnosis* ; Infant ; Adolescent

OBJECTIVES: To investigate the clinical features and prognosis of children with non-Down-syndrome-related acute megakaryoblastic leukemia (non-DS-AMKL). METHODS: A retrospective analysis was conducted on the medical data of 17 children with non-DS-AMKL who were admitted to Children's Hospital of The First Affiliated Hospital of Zhengzhou University from January 2013 to December 2023, and their clinical features, treatment, and prognosis were summarized. RESULTS: Among the 17 children with non-DS-AMKL, there were 8 boys and 9 girls. Fourteen patients had an onset age of less than 36 months, with a median age of 21 months (range:13-145 months). Immunophenotyping results showed that 16 children were positive for CD61 and 13 were positive for CD41. The karyotype analysis was performed on 16 children, with normal karyotype in 6 children and abnormal karyotype in 9 children, among whom 5 had complex karyotype and 1 had no mitotic figure. Detected fusion genes included EVI1, NUP98-KDM5A, KDM5A-MIS18BP1, C22orf34-BRD1, WT1, and MLL-AF9. Genetic alterations included TET2, D7S486 deletion (suggesting 7q-), CSF1R deletion, and PIM1. All 17 children received chemotherapy, among whom 16 (94%) achieved complete remission after one course of induction therapy, and 1 child underwent hematopoietic stem cell transplantation (HSCT) and remained alive and disease-free. Of all children, 7 experienced recurrence, among whom 1 child received HSCT and died of graft-versus-host disease. At the last follow-up, six patients remained alive and disease-free. CONCLUSIONS Non-DS-AMKL primarily occurs in children between 1 and 3 years of age. The patients with this disorder have a high incidence rate of chromosomal abnormalities, with complex karyotypes in most patients. Some patients harbor fusion genes or gene mutations. Although the initial remission rate is high, the long-term survival rate remains low.
Humans ; Male ; Female ; Leukemia, Megakaryoblastic, Acute/etiology* ; Child, Preschool ; Infant ; Child ; Retrospective Studies ; Prognosis ; Down Syndrome/complications*

Objective:Persistent proatlantal intersegmental artery (PPIA) is a rare variant of persistent carotid-vertebrobasilar anastomoses and mostly discovered incidentally. To explore the clinical features of PPIA, and enhance the understanding of this rare cerebrovascular variation.Methods:A retrospective analysis was performed. The clinical data of 6 patients with PPIA admitted to Department of Neurointervention, First Affiliated Hospital of Zhengzhou University from June 2018 to June 2024 were analyzed. The imaging features, associated cerebrovascular diseases, treatments and follow-up results were summarized.Results:Among the 6 PPIA patients, 4 were with type I and 2 were with type II; 5 were with left PPIA and 1 patient was with right PPIA; 5 patients had bilateral vertebral artery hypoplasia or absence, and 1 patient had absence of the ipsilateral vertebral artery and normal development of the contralateral vertebral artery. Four PPIA patients were combined with intracranial aneurysms: 2 patients received dual stent-assisted embolization and flow diverter device combined with covered stent implantation, respectively (after 6 months of follow-up, modified Rankin scale score of 0 indicating good prognosis and healed intracranial aneurysms), and the other 2 patients with micro-aneurysms were not treated. One patient was combined with internal carotid artery stenosis and accepted stent placement (good prognosis at 6 months after follow-up). One patient was combined with acute basilar artery tip occlusion and accepted aspiration thrombectomy (died of brain herniation 7 days after thrombectomy).Conclusions:PPIA is often accompanied by vertebral artery developmental abnormalities and prone to have concurrent intracranial aneurysms or ischemic stroke. When performing surgical treatment for cerebrovascular diseases associated with PPIA, PPIA protection to ensure blood supply of the posterior circulation is essential.

AIM To establish the HPLC-CAD characteristic chromatograms of Qingzhi Huayu Formula,and to determine the contents of rhamnose,fructose,mannitol,D-anhydrous glucose,sucrose and raffinose.METHODS The analysis was performed on a 35 ℃ thermostatic Dikma Polyamino HILIC column (4.6 mm×250 mm,5 μm),with the mobile phase comprising of acetonitrile-water flowing at 0.8 mL/min in a gradient elution manner.RESULTS There were 14 common peaks in the characteristic chromatograms of 15 batches of samples with the similarities of more than 0.90.Six carbohydrates showed good linear relationships within their own ranges ( r>0.9950 ),whose average recoveries were 93.34％-105.64％ with the RSDs of 0.5％-2.6％.CONCLUSION This accurate,reliable,sensitive and reproducible method can be used for the quality control of carbohydrates in Qingzhi Huayu Formula.

Objective To explore the current application of high-throughput drug sensitivity(HDS)testing in children with relapsed and refractory acute leukemia(RR-AL)and analyze the feasibility of salvage treatment plans.Methods A retrospective collection of clinical data from children with RR-AL who underwent HDS testing at the Department of Children's Hematology and Oncology of the First Affiliated Hospital of Zhengzhou University from November 2021 to October 2023 was conducted,followed by an analysis of drug sensitivity results and treatment outcomes.Results A total of 17 children with RR-AL underwent HDS testing,including 7 cases of relapsed refractory acute myeloid leukemia and 10 cases of relapsed refractory acute lymphoblastic leukemia.The detection rate of highly sensitive chemotherapy drugs/regimens was 53%(9/17),while the detection rate of moderately sensitive chemotherapy drugs/regimens was 100%(17/17).Among the 17 RR-AL patients with highly and moderately sensitive chemotherapy drugs and regimens,the MOACD regimen(mitoxantrone+vincristine+cytarabine+cyclophosphamide+dexamethasone)accounted for 100%,with the highest inhibition rate for single-agent mitoxantrone(94%,16/17),and the highest inhibition rate for targeted therapy being bortezomib(94%,16/17).Nine patients adjusted their chemotherapy based on HDS testing results,with 4 undergoing hematopoietic stem cell transplantation.Four patients achieved disease-free survival,while 5 died.Eight patients received empirical chemotherapy,with 2 undergoing hematopoietic stem cell transplantation;4 achieved disease-free survival,while 4 died.Conclusions HDS testing can identify highly sensitive drugs/regimens for children with RR-AL,improving the rate of re-remission and creating conditions for subsequent hematopoietic stem cell transplantation.

AIM To establish the HPLC-CAD characteristic chromatograms of Qingzhi Huayu Formula,and to determine the contents of rhamnose,fructose,mannitol,D-anhydrous glucose,sucrose and raffinose.METHODS The analysis was performed on a 35 ℃ thermostatic Dikma Polyamino HILIC column (4.6 mm×250 mm,5 μm),with the mobile phase comprising of acetonitrile-water flowing at 0.8 mL/min in a gradient elution manner.RESULTS There were 14 common peaks in the characteristic chromatograms of 15 batches of samples with the similarities of more than 0.90.Six carbohydrates showed good linear relationships within their own ranges ( r>0.9950 ),whose average recoveries were 93.34％-105.64％ with the RSDs of 0.5％-2.6％.CONCLUSION This accurate,reliable,sensitive and reproducible method can be used for the quality control of carbohydrates in Qingzhi Huayu Formula.

Diabetic retinopathy(DR)is a prevalent microvascular complication of diabetes and the leading cause of blindness and severe visual impairment in adults.The high levels of glucose trigger multiple intracellular oxidative stress pathways,such as POLDIP2,resulting in excessive reactive oxygen species(ROS)pro-duction and increased expression of vascular cell adhesion molecule-1(VCAM-1),hypoxia-inducible factor 1α(HIF-1α),and vascular endothelial growth factor(VEGF),causing microvascular dysfunction.Dihydromyricetin(DMY)is a natural flavonoid small molecule antioxidant.However,it exhibits poor solubility in physiological environments,has a short half-life in vivo,and has low oral bioavailability.In this study,we present,for the first time,the synthesis of ultra-small Fe-DMY nano-coordinated polymer particles(Fe-DMY NCPs),formed by combining DMY with low-toxicity iron ions.In vitro and in vivo experiments confirm that Fe-DMY NCPs alleviate oxidative stress-induced damage to vascular endo-thelial cells by high glucose,scavenge excess ROS,and improve pathological features of DR,such as retinal vascular leakage and neovascularization.Mechanistic validation indicates that Fe-DMY NCPs can inhibit the activation of the Poldip2-Nox4-H2O2 signaling pathway and downregulate vital vascular function indicators such as VCAM-1,HIF-1α,and VEGF.These findings suggest that Fe-DMY NCPs could serve as a safe and effective antioxidant and microangio-protective agent,with the potential as a novel multimeric drug for DR therapy.

In recent years, laparoscopic surgery and robotic surgery have been widely used, and various intraoperative image navigation systems have also developed rapidly. However, the liver itself has a complex vessel and duct system, which increase the difficulty of liver surgery. The augmented reality image navigation system combines the three-dimensional reconstructed image of the liver with the real liver anatomy, which presents the specific relationship between the tumor location and the surrounding vessels for the surgeon. Compared with other intraoperative image navigation methods, augmented reality has its unique advantages. This paper provides an overview of current advances in registration technology in augmented reality image navigation system, and focuses on its applications in liver surgery, including laparoscopic surgery and robotic surgery. Finally, the technological problems and difficulties still faced at present are summarized, and future directions worth studying in this field are proposed.

The progressive destruction of condylar cartilage is a hallmark of the temporomandibular joint (TMJ) osteoarthritis (OA); however, its mechanism is incompletely understood. Here, we show that Kindlin-2, a key focal adhesion protein, is strongly detected in cells of mandibular condylar cartilage in mice. We find that genetic ablation of Kindlin-2 in aggrecan-expressing condylar chondrocytes induces multiple spontaneous osteoarthritic lesions, including progressive cartilage loss and deformation, surface fissures, and ectopic cartilage and bone formation in TMJ. Kindlin-2 loss significantly downregulates the expression of aggrecan, Col2a1 and Proteoglycan 4 (Prg4), all anabolic extracellular matrix proteins, and promotes catabolic metabolism in TMJ cartilage by inducing expression of Runx2 and Mmp13 in condylar chondrocytes. Kindlin-2 loss decreases TMJ chondrocyte proliferation in condylar cartilages. Furthermore, Kindlin-2 loss promotes the release of cytochrome c as well as caspase 3 activation, and accelerates chondrocyte apoptosis in vitro and TMJ. Collectively, these findings reveal a crucial role of Kindlin-2 in condylar chondrocytes to maintain TMJ homeostasis.
Aggrecans/metabolism* ; Animals ; Cartilage, Articular/metabolism* ; Chondrocytes/pathology* ; Cytoskeletal Proteins/metabolism* ; Mice ; Muscle Proteins/metabolism* ; Osteoarthritis/pathology* ; Temporomandibular Joint/pathology*