This study retrospectively analyzed data from 25 patients with paroxysmal nocturnal hemoglobinuria (PNH) admitted to Peking Union Medical College Hospital and Dongfang Hospital of Beijing University of Chinese Medicine from January 2023 to June 2024. Patients receiving sufficient eculizumab treatment for at least 3 months and who completed hemolytic complex (CH50) level testing pre- and post-treatment for 3 and 6 months were selected. Blood routine, biochemistry, and the 50% CH50-related indicators were monitored pre- and post-treatment. Among these patients, 24 completed 6 months of treatment and CH50 testing. After 3 and 6 months of eculizumab treatment, all patients with PNH showed significant improvement in symptoms, with lactate dehydrogenase (LDH) levels decreasing from a baseline of (1 814.4 ± 924.8) U/L to (248.5 ± 61.0) U/L and (239.3 ± 44.8) U/L. Hemoglobin levels increased from a baseline of (73.9±14.4) g/L to (99.9 ± 21.3) g/L and (99.6 ± 19.8) g/L. The baseline CH50 level was (32.4±14.7) %, which decreased to 2.0% (1.0% –8.0% ) and 1.0% (1.0% –4.0% ) at 3 and 6 months posttreatment, respectively. At baseline, a linear correlation was found between CH50 and LDH levels ( P<0.001), and the trend of CH50 changes was significantly lower than LDH at 3 and 6 months post-treatment with eculizumab, with similar trends. However, no linear correlation was observed between CH50 and LDH levels or other parameters at 3 and 6 months of medication. Our case demonstrates that eculizumab is effective for PNH hemolysis treatment. The serum CH50 level may be a biomarker for complement blockade induced by eculizumab, which can, to some extent, reflect the intravascular hemolysis of PNH and the efficacy of eculizumab.

The in-depth research of artificial intelligence in the medical field has greatly improved the workflow and diagnostic ability of diagnostic radiology.This article focuses on artificial intelligence technology in the field of interventional medicine,and enumerates its potential application scenarios,including improving image analysis capabilities to assist diagnosis and predict treatment response.It also describes the challenges that need to be overcome for practical application.Finally,with the continuous development of artificial intelligence in interventional medicine,artificial intelligence will further optimize the channels of interventional medicine and bring revolutionary changes to the clinical practice of interventional medicine.

This study retrospectively analyzed data from 25 patients with paroxysmal nocturnal hemoglobinuria (PNH) admitted to Peking Union Medical College Hospital and Dongfang Hospital of Beijing University of Chinese Medicine from January 2023 to June 2024. Patients receiving sufficient eculizumab treatment for at least 3 months and who completed hemolytic complex (CH50) level testing pre- and post-treatment for 3 and 6 months were selected. Blood routine, biochemistry, and the 50% CH50-related indicators were monitored pre- and post-treatment. Among these patients, 24 completed 6 months of treatment and CH50 testing. After 3 and 6 months of eculizumab treatment, all patients with PNH showed significant improvement in symptoms, with lactate dehydrogenase (LDH) levels decreasing from a baseline of (1 814.4 ± 924.8) U/L to (248.5 ± 61.0) U/L and (239.3 ± 44.8) U/L. Hemoglobin levels increased from a baseline of (73.9±14.4) g/L to (99.9 ± 21.3) g/L and (99.6 ± 19.8) g/L. The baseline CH50 level was (32.4±14.7) %, which decreased to 2.0% (1.0% –8.0% ) and 1.0% (1.0% –4.0% ) at 3 and 6 months posttreatment, respectively. At baseline, a linear correlation was found between CH50 and LDH levels ( P<0.001), and the trend of CH50 changes was significantly lower than LDH at 3 and 6 months post-treatment with eculizumab, with similar trends. However, no linear correlation was observed between CH50 and LDH levels or other parameters at 3 and 6 months of medication. Our case demonstrates that eculizumab is effective for PNH hemolysis treatment. The serum CH50 level may be a biomarker for complement blockade induced by eculizumab, which can, to some extent, reflect the intravascular hemolysis of PNH and the efficacy of eculizumab.

Schimke immuno-osseous dysplasia (SIOD)caused by SMARCAL1 gene mutations is a rare genetic disorder characterized by multi-system involvement, including T-cell dysfunction, skeletal dysplasia, disproportionate short stature, nephrotic syndrome, and kidney failure. This multidisciplinary consultation involved a 9-year-old boy with intrauterine growth retardation, presenting with short stature, T-cell lymphopenia, proteinuria, and degenerative bone and joint disease. Treatment primarily focused on symptomatic and supportive care. Through the multidisciplinary rare disease consultation, holistic support was provided to the patient, offering comprehensive care from various specialtiesx.We also aim to use this case report to enhance clinicians′ under-standing of SIOD and improve the management and treatment of rare diseases.

OBJECTIVE To investigate the polysomnographic differences between males and females with obstructive sleep apnea syndrome(OSAS).METHODS Adults visited to Beijing Tongren Hospital for sleep snoring from January 2023 to August 2023 who completed overnight polysomnography(PSG)and whose apnea hypopnea index(AHI)≥5 times/h were diagnosed as OSAS patients in this study.The general information,respiratory events and sleep structure were compared between male and female OSAS patients.RESULTS A total of 380 patients were included in this study with 293 males(77.1%)and 87 females(22.9%).Mean age was(44.4±11.5)years,and the mean body mass index was(26.5±3.9)kg/m2;AHI ranged between 5.0 and 115.8 times/h with a mean of(34.9±24.8)times/h;lowest oxygen saturation(LSpO2)ranged between 43%-97%with a mean of(80.7±11.6)%.Between male and female OSAS patients,the AHI in males was higher than females[(37.9±24.8)times/h vs.(24.5±21.7)times/h,P=0.000],and the AHI in REM was higher in males than females[(36.7±23.2)times/h vs.(30.9±22.8)times/h,P=0.040].LSpO2 was lower in males than females[(79.2±12.0)%vs.(85.7±8.3)%,P=0.000].There were significant differences for arousal index,sleep efficiency,N1 sleep percent and N3 sleep percent between two groups.Between male and female OSAS patients over 50 years old,AHI was still higher in males than in females[(39.3±21.4)times/h vs.(30.5±23.0)times/h,P=0.029],and there was no significant difference in AHIREM and LSpO2.The difference in sleep structure between male and female OSAS patients over 50 years old was consistent with that of participants.CONCLUSION The AHI and LSpO2 of male OSAS patients were more serious than those of female OSAS patients,and the reduction of slow wave sleep was more obvious.OSAS became worse after menopause,which was highlighted by the increase of respiratory events in REM and the more serious decline of LSpO2.The protective effect of female hormones on OSAS is mainly to alleviate REM respiratory events and hypoxia damage,rather than improving sleep structure.

Traditional open head and neck surgery often leaves permanent scars,significantly affecting appearance.The emergence of surgical robots has introduced a new era for minimally invasive surgery.However,the complex anatomy of the head and neck region,particularly the oral and maxillofacial areas,combined with the high costs associated with established systems such as the da Vinci,has limited the widespread adoption of surgical robots in this field.Recently,surgical robotic platform in China has developed rapidly,exemplified by the promise shown by the KangDuo Surgical Robot(KD-SR).Although the KD-SR has achieved some results comparable to the da Vinci surgical robot in urology and colorectal surgery,its performance in complex head and neck regions remains untested.This study evaluated the feasibility,effectiveness,and safety of the newly developed KD-SR-01,comparing it with standard endoscopic systems in head and neck procedures on porcine models.We performed parotidectomy,submandibular gland resection,and neck dissection,collected baseline characteristics,perioperative data,and specifically assessed cognitive workload using the NASA-TLX.None of the robotic procedures were converted to endoscopic or open surgery.The results showed no significant difference in operation time between the two groups(P=0.126),better intraoperative bleeding control(P=0.001),and a significant reduction in cognitive workload(P<0.001)in the robotic group.In conclusion,the KD-SR-01 is feasible,effective,and safe for head and neck surgery.Further investigation through well-designed clinical trials with long-term follow-up is necessary to establish the full potential of this emerging robotic platform.

Traditional open head and neck surgery often leaves permanent scars,significantly affecting appearance.The emergence of surgical robots has introduced a new era for minimally invasive surgery.However,the complex anatomy of the head and neck region,particularly the oral and maxillofacial areas,combined with the high costs associated with established systems such as the da Vinci,has limited the widespread adoption of surgical robots in this field.Recently,surgical robotic platform in China has developed rapidly,exemplified by the promise shown by the KangDuo Surgical Robot(KD-SR).Although the KD-SR has achieved some results comparable to the da Vinci surgical robot in urology and colorectal surgery,its performance in complex head and neck regions remains untested.This study evaluated the feasibility,effectiveness,and safety of the newly developed KD-SR-01,comparing it with standard endoscopic systems in head and neck procedures on porcine models.We performed parotidectomy,submandibular gland resection,and neck dissection,collected baseline characteristics,perioperative data,and specifically assessed cognitive workload using the NASA-TLX.None of the robotic procedures were converted to endoscopic or open surgery.The results showed no significant difference in operation time between the two groups(P=0.126),better intraoperative bleeding control(P=0.001),and a significant reduction in cognitive workload(P<0.001)in the robotic group.In conclusion,the KD-SR-01 is feasible,effective,and safe for head and neck surgery.Further investigation through well-designed clinical trials with long-term follow-up is necessary to establish the full potential of this emerging robotic platform.

Objective:To search, evaluate and integrate the best evidence of exercise rehabilitation intervention after PCI in patients with acute myocardial infarction, so as to provide evidence-based basis for clinical doctors and nurses to intervene in exercise rehabilitation of patients.Methods:We searched PubMed, China National Knowledge Infrastructure, Wanfang Data and other domestic and foreign databases, guide websites and professional association websites about the evidence of exercise rehabilitation intervention in patients with acute myocardial infarction after PCI. The search period is from January 2010 to June 2021. The quality of the included literature was evaluated by two researchers, and the literature evidence in accordance with the quality standard was extracted.Results:A total of 20 articles were included, and 39 pieces of evidence were summarized from 11 aspects, such as the establishment of multidisciplinary team, evaluation, exercise prescription, exercise monitoring and so on.Conclusions:This study summarized the best evidence of exercise rehabilitation intervention in patients with acute myocardial infarction after PCI, and provided theoretical support for clinical practice. It is suggested to combine the clinical situation and patients' wishes to promote the transformation of the best evidence to clinical practice.

Objective    To explore the application value of synchronous CT-guided percutaneous biopsy followed by radiofrequency ablation in the diagnosis and treatment of lung tumors. Methods    The clinical data of 21 patients with lung tumors were retrospectively analyzed. There were 8 males and 13 females aged 68 (51, 73) years. A total of 24 lesions underwent CT-guided percutaneous biopsy and concurrent radiofrequency ablation. The effectiveness and safety of this protocol were analyzed. Results    All 21 patients successfully completed the procedures. The diameter of 24 lesions was 17.0 (13.3, 19.0) mm. Biopsy specimens met the requirements of pathological diagnosis, and the effectiveness of specimens was 100.0%. The incidence of small amount of pneumothorax/pleural shrinkage after procedures was 19.0% (4/21) and the incidence of tension pneumothorax was 4.7% (1/21). There was no obvious bleeding or other complications. Conclusion    Synchronous CT-guided percutaneous biopsy followed by radiofrequency ablation combines two interventional techniques, which is safe and effective in the diagnosis and treatment of lung tumors, and it is worthy of popularization and application in clinic.

Objective:To report a Chinese family with a novel ABCD1 gene mutation at c.332T>G (p.V111G) site and discuss its clinical characteristics and molecular mechanism.Methods:The clinical data, laboratory examination, and imaging examination results were analyzed to make the clinical diagnosis of a middle-aged onset patient from the First Affiliated Hospital of Zhengzhou University in May 2017. High-throughput sequencing was used to discover a novel ABCD1 gene mutation. Sanger sequencing was used to find out whether other family members contain the same ABCD1 gene mutation. The pathogenicity of this mutation was explored by protein structure prediction and pathogenicity analysis. Adrenoleukodystrophy protein-green fluorescent protein (ALDP-GFP) and ALDP-GFP (V111G) plasmids were constructed and human embryonic kidney 293 cells were transfected, then immunofluorescence and Western blotting were used to explore the molecular mechanism of this mutation (completed in Henan Provincial People′s Hospital).Results:The proband (a 39-year-old male) was diagnosed as adrenomyeloneuropathy, a subset of X-linked adrenoleukodystrophy, with a novel heterozygous missense mutation in the ABCD1 gene at c.332T>G (p.V111G) site, and his mother and two daughters were all carriers. Protein structure prediction and pathogenicity results suggested that this mutation is pathogenic. Overexpression of ALDP-GFP (V111G) in the human embryonic kidney 293 cells resulted in a significant decrease in the expression levels of ALDP and the abnormal localization from the peroxisomal membrane to the cytoplasm, accompanied by significant down-regulation of LC3-Ⅱ/LC3-Ⅰ and beclin-1.Conclusion:c.332T>G (p.V111G) is a novel pathogenic mutation in the ABCD1 gene, which causes adrenomyeloneuropathy by impairing autophagy.