1.A Case of Multidisciplinary Treatment for Kimura′s Disease
Shuning GUO ; Jiaxin ZHOU ; Zhaohui LU ; Yu CHEN ; Yang GUI ; Rongrong HU ; Xiaoxiao GUO ; Yuxiang ZHI ; Fuquan ZHANG ; Yan ZHANG ; Wen ZHANG
JOURNAL OF RARE DISEASES 2026;5(3):309-317
This article reports a 14-year-old boy who presented with "a nearly 7-year history of a right upper arm mass and a more than 1-year history of cervical lymphadenopathy". His main clinical manifestations included a subcutaneous mass in the right upper arm, bilateral eyelid and submandibular masses, and multiple enlarged cervical lymph nodes, accompanied by persistent peripheral blood eosinophilia. Biopsies of the right upper arm lesion and a left submandibular lymph node showed lymphoid hyperplasia with prominent eosinophilic infiltration, vascular and fibrous tissue proliferation, and eosinophilic microabscess formation in the left submandibular lymph node, confirming the diagnosis of Kimura′s disease. The lesions regressed markedly after glucocorticoid therapy but recurred after treatment discontinuation. Because the patient and his family were concerned about long-term glucocorticoid therapy, mepolizumab was subsequently administered. Peripheral eosinophil counts normalized, whereas the local tissue lesions showed limited improvement. A multidisciplinary team comprehensively evaluated the diagnosis, the need for repeat biopsy of atypical lymph nodes on imaging, the possible association between the cardiac valve abnormalities and the underlying disease, and subsequent treatment options. The patient is currently receiving methotrexate with folic acid supplementation and remains under regular follow-up. This case indicates that the diagnosis of Kimura′s disease relies on clinical manifestations and histopathological findings. Once diagnosed, comprehensive evaluation, individualized treatment, and long-term management should be implemented.
2.Progress in prevention and treatment of knee laxity after posterior cruciate ligament reconstruction.
Zhaohui RUAN ; Zhengliang SHI ; Ping YUAN ; Xianguang YANG ; Yanlin LI
Chinese Journal of Reparative and Reconstructive Surgery 2025;39(10):1333-1341
OBJECTIVE:
To summarize the research progress on knee laxity of biomechanics and prevention and treatment after posterior cruciate ligament (PCL) reconstruction.
METHODS:
The domestic and international literature on the prevention and treatment of knee laxity after PCL reconstruction in recent years was extensively reviewed and analyzed.
RESULTS:
Different degrees of knee laxity often occur after PCL reconstruction, which can lead to poor prognosis in patients. The causes are associated with a variety of factors, including abnormal graft remodeling (such as differences in healing time and biomechanics among different types of grafts), tunnel position deviation (such as graft wear caused by the "killer turn" effect), and mechanical factors in postoperative rehabilitation (such as improper early weight-bearing and range of motion). These factors may promote graft elongation, increase early posterior tibial translation, and thereby induce knee laxity.
CONCLUSION
While PCL reconstruction improves knee stability, it is crucial to focus on and prevent postoperative knee laxity. However, current surgical methods are limited by factors such as graft characteristics, surgical technique flaws, and rehabilitation protocols, and thus can not fully correct the issue of abnormal postoperative laxity. Surgical techniques and treatment strategies still need further improvement and optimization to enhance patients' postoperative outcomes and quality of life.
Humans
;
Joint Instability/surgery*
;
Posterior Cruciate Ligament Reconstruction/adverse effects*
;
Posterior Cruciate Ligament/surgery*
;
Knee Joint/physiopathology*
;
Biomechanical Phenomena
;
Range of Motion, Articular
;
Postoperative Complications/prevention & control*
;
Knee Injuries/surgery*
3.Research progress on enhanced recovery after posterior cruciate ligament reconstruction.
Zhengliang SHI ; Yanlin LI ; Zhaohui RUAN ; Hongmai YANG ; Kaiquan LI ; Ping YUAN ; Wenting TANG ; Rui HAN
Chinese Journal of Reparative and Reconstructive Surgery 2025;39(12):1591-1599
OBJECTIVE:
To summarize research progress on enhanced recovery after posterior cruciate ligament (PCL) reconstruction, clarify the core contradictions, effective intervention methods, and evaluation shortcomings in current clinical practice, and provide theoretical support for optimizing clinical rehabilitation strategies.
METHODS:
Relevant domestic and international literature in recent years was systematically searched. The key technologies and challenges for enhanced recovery after PCL reconstruction were analyzed from three aspects: the core issues of enhanced recovery after PCL reconstruction, treatment strategies, and the post-reconstruction effectiveness evaluation system.
RESULTS:
Enhanced recovery after PCL reconstruction mainly faces two core problems. First, there is a balance dilemma between graft tendon protection and knee joint function recovery: the tensile capacity of the graft tendon is weak in the early postoperative period, so excessive weight-bearing easily leads to relaxation, while overly conservative immobilization causes muscle atrophy and joint adhesion. Second, the return-to-sport rate is significantly affected by injury type and treatment method: patients with combined multiple ligament or meniscus injuries have a much lower return-to-sport rate than those with isolated PCL injury, and the risk of return-to-sport failure is higher. Current research mainly promotes rehabilitation from two aspects: physical therapy and surgical technology. Physical therapy runs through the perioperative period: preoperatively, muscle strength training, swelling control, and maintenance of joint range of motion are used to optimize surgical conditions; postoperatively, phased intervention is implemented. Surgical technology focuses on minimally invasive and anatomical approaches: arthroscopic surgery reduces injury, double-bundle reconstruction and internal tension-relief technology improve stability, and modified tunnel positioning and special surgical methods avoid the risk of "Killer Turn". Postoperative functional evaluation adopts multi-dimensional indicators: subjective evaluation relies on scales such as Lysholm and International Knee Documentation Committee (IKDC); objective evaluation assesses stability through Telos stress test and posterior drawer test; imaging evaluation takes MRI as the core; psychological evaluation is assisted by the Tampa scale of kinesiophobia-11 (TSK-11). However, there are obvious shortcomings, such as the lack of PCL-specific evaluation tools.
CONCLUSION
Enhanced recovery after PCL reconstruction requires the integration of precise surgery, individualized rehabilitation, and comprehensive subjective and objective evaluation. In the future, biomaterials and digital technologies should be integrated to optimize the full-cycle management of PCL reconstruction, thereby improving functional recovery and the effect of return to sports.
Humans
;
Posterior Cruciate Ligament Reconstruction/rehabilitation*
;
Posterior Cruciate Ligament/injuries*
;
Recovery of Function
;
Knee Joint/physiopathology*
;
Knee Injuries/rehabilitation*
;
Return to Sport
;
Enhanced Recovery After Surgery
;
Tendons/transplantation*
;
Arthroscopy
4.Inner Ear Delivery of Polyamino Acid Nanohydrogels Loaded with Dexamethasone
Pingping AI ; Lidong ZHAO ; Zhaohui TANG ; Chaoliang HE ; Xuesi CHEN ; Shiming YANG ; Nan WU
Medical Journal of Peking Union Medical College Hospital 2025;16(2):370-378
To develop a novel polyamino acid-based nanohydrogel drug delivery system for dexamethasone to enhance its delivery efficiency to the inner ear. A fluorescein-labeled polyglutamic acid-based polyamino acid dexamethasone nanohydrogel was synthesized, and its gelation time was measured. The hydrogel was surgically injected into the round window niche of guinea pigs to determine its degradation time in the middle ear cavity in vivo. The safety, pharmacokinetics, and distribution patterns of dexamethasone in the inner ear were evaluated. The hydrogel exhibited a gelation time of 80 seconds in a 37℃ water bath. In vivo, the hydrogel was almost completely degraded within 7 days in the middle ear cavity of guinea pigs. Transient hearing loss was observed one day after administration, but hearing gradually returned to normal over time. No significant cytotoxicity, vestibular stimulation signs, or pathological abnormalities in spiral ganglion cells were observed. Histopathological examination revealed no significant inflammatory reactions. Pharmacokinetic analysis demonstrated sustained drug release and prolonged dexamethasone activity. Immunofluorescence staining confirmed the distribution of dexamethasone in both the cochlea and vestibular organs. The polyamino acid nanohydrogel exhibits excellent injectability and biodegradability, representing a safe and effective drug delivery system for the inner ear.
5.Internal tension relieving technique assisted anterior cruciate ligament reconstruction to promote ligamentization of Achilles tendon grafts in small ear pigs in southern Yunnan province
Bohan XIONG ; Guoliang WANG ; Yang YU ; Wenqiang XUE ; Hong YU ; Jinrui LIU ; Zhaohui RUAN ; Yajuan LI ; Haolong LIU ; Kaiyan DONG ; Dan LONG ; Zhao CHEN
Chinese Journal of Tissue Engineering Research 2025;29(4):713-720
BACKGROUND:We have successfully established an animal model of small ear pig in southern Yunnan province with internal tension relieving technique combined with autologous Achilles tendon for anterior cruciate ligament reconstruction,and verified the stability and reliability of the model.However,whether internal tension relieving technique can promote the ligamentalization process of autologous Achilles tendon graft has not been studied. OBJECTIVE:To investigate the differences in the process of ligamentalization between conventional reconstruction and internal reduction reconstruction of the anterior cruciate ligament by gross view,histology and electron microscopy. METHODS:Thirty adult female small ear pigs in southern Yunnan province were selected.Anterior cruciate ligament reconstruction was performed on the left knee joint with the ipsilateral knee Achilles tendon(n=30 in the normal group),and anterior cruciate ligament reconstruction was performed on the right knee joint with the ipsilateral knee Achilles tendon combined with the internal relaxation and enhancement system(n=30 in the relaxation group).The autogenous right forelimb was used as the control group;the anterior cruciate ligament was exposed but not severed or surgically treated.At 12,24,and 48 weeks after surgery,10 animals were sacrificed,respectively.The left and right knee joint specimens were taken for gross morphological observation to evaluate the graft morphology.MAS score was used to evaluate the excellent and good rate of the ligament at each time point.Hematoxylin-eosin staining was used to evaluate the degree of ligament graft vascularization.Collagen fibers and nuclear morphology were observed,and nuclear morphology was scored.Ultrastructural remodeling was evaluated by scanning electron microscopy and transmission electron microscopy. RESULTS AND CONCLUSION:(1)The ligament healing shape of the relaxation group was better at various time points after surgery,and the excellent and good rate of MAS score was higher(P<0.05).Moreover,the relaxation group could obtain higher ligament vascularization score(P<0.05).(2)The arrangement of collagen bundles and fiber bundles in the two groups gradually tended to be orderly,and the transverse fiber connections between collagen gradually increased and thickened,suggesting that the strength and shape degree of the grafts were gradually improved,but the ligament remodeling in the relaxation group was always faster than that in the normal group at various time points after surgery.(3)The diameter,distribution density,and arrangement degree of collagen fibers in the relaxation group were better than those in the normal group at all time points,especially in the comparison of collagen fiber diameter between and within the relaxation group(P<0.05).
6.Expert consensus on clinical treatment of acute radiation syndrome from external irradiation
Li LIANG ; Long YUAN ; Changlin YU ; Qingjie LIU ; Yulong LIU ; Wenfeng YANG ; Jin WANG ; Weixu HUANG ; Ying LIU ; Cuiping LEI ; Huifang CHEN ; Ximing FU ; Baoshan CAO ; Mopei WANG ; Zhaohui ZHANG ; Yu XIAO ; Yamei CHEN ; Quanfu SUN
Chinese Journal of Radiological Medicine and Protection 2025;45(9):827-839
China emerges as a major country in nuclear energy development and the application of nuclear and radiologic technology. The diagnosis and treatment of acute radiation syndrom (ARS) caused by external irradiation represent a core function in the country′s medical rescue of nuclear and radiological emergencies. Clinically, ARS manifests hematopoietic, gastrointestinal, cutaneous, and central nervous system syndromes, with specific clinical manifestations, signs, severity, and prognosis strongly correlated with radiation dose. China has established a number of national and provincial centers for treating radiation-induced damage. Nevertheless, most medical staff have limited experience in ARS treatment. This consensus presents a summary of recent experience in treating ARS of China. In combination with recommendations from international organizations such as the World Health Organization (WHO), this consensus proposes key evidence of critical clinical issues of ARS, covering all links in the rescue of external irradiation-induced ARS. Initially, clinical diagnosis, syndromes, and severe degrees should be determined based on clinical symptoms and dose estimates. It is necessary to normalize clinical treatment measures for hematopoietic recovery, gastrointestinal injury treatment, infection control, symptomatic treatment, and multi-organ function preservation. To this end, this consensus offers cautions. This consensus provides principles of treatment with traditional Chinese medicine, psychological intervention, and follow-up. Additionally, it highlights multidisciplinary collaboration. It is recommended that this consensus be applied in relevant treatment centers.
7.Survival Analysis of Three Malignant Tumors with the Highest Risk of Death in Jiashan County,Zhejiang Province from 2006 to 2020
Lingling YU ; Xinglin FEI ; Jinhua YANG ; Feiqiong SHEN ; Zepeng ZHANG ; Zhaohui ZHANG
China Cancer 2025;34(1):26-31
[Purpose]To analyze the survival of lung cancer,liver cancer and stomach cancer in Jiashan County,Zhejiang Province from 2006 to 2020.[Methods]From January 1,2006 to De-cember 31,2020,new cases of lung cancer,liver cancer and stomach cancer were collected in Jiashan County,Zhejiang Province.The 5-year observed survival rate(OSR),relative survival rate(RSR)and age-standardized relative survival rate(ARSR)by sex and age were calculated from 2006-2010,2011-2015 and 2016-2020.The observed and relative survivals were calculated using the lifetime table and Ederer Ⅱ methods,respectively,and age standardized using the Inter-national Cancer Survival Standard.Joinpoint software was utilized to compute the average annual percentage change(AAPC)for analyzing trends in various survival rates from 2006 to 2020.[Re-sults]The 5-year RSR of lung cancer,liver cancer and stomach cancer increased from 8.1%,4.8%and 23.2%(2006-2010)to 36.0%,17.6%and 48.2%(2016-2020),showing an increasing trend(AAPC=14.4%,AAPC=11.4%,AAPC=6.4%,respectively,all P<0.05).Accordingly,the 5-year RSR of three types of malignant tumors showed an increasing trend in both men and women(all P<0.05)except liver cancer in women(P>0.05).The 5-year RSR of three types of malignant tu-mors in the age groups of 0~64 and 65+years old showed a significant increasing trend(all P<0.05).The increase of lung cancer and liver cancer was greater in the age group of 0~64 years old,and the increase of gastric cancer was greater in the age group of 65+years old.[Conclusion]The 5-year survival rates of the three types of malignant tumors with the highest mortality risk in Jiashan County,Zhejiang Province all increased significantly from 2006 to 2020,indicating that the pre-vention and treatment effect of malignant tumors is significant,but the survival rate of malignant tumors varied significantly among different populations,so targeted intervention should be carried out.
8.Analysis of novel mutations in the insulin receptor gene of a family with type A insulin resistance syndrome
Yijun LI ; Guoqing YANG ; Li ZANG ; Yu PEI ; Kang CHEN ; Jin DU ; Zhaohui LYU
Chinese Journal of Internal Medicine 2025;64(3):239-243
This study aimed to identify mutations in the human insulin receptor gene (INSR) and investigate their role in the pathogenesis of severe insulin resistance syndrome. Sanger sequencing of the INSR gene was performed on a patient clinically suspected of having type A insulin resistance syndrome admitted to the Department of Endocrinology, the First Medical Center of Chinese PLA General Hospital. Upon identifying mutations, relevant exons were sequenced in her first-degree relatives. Additionally, control groups consisting of individuals with type 2 diabetes and those with normal glucose tolerance were screened for the mutation detected in the patient. Functional predictions of the INSR protein were made using MutationTaster, SIFT, and PolyPhen2 software. A previously unreported heterozygous missense mutation, c.3652G/A (Asp1218Asn), in exon 20 was identified in both the proband and her father. This mutation was not present in any of the control individuals. Multiple prediction tools indicate that this mutation likely disrupts gene/protein structure or function. The c.3652G/A (Asp1218Asn) heterozygous mutation in INSR is a novel variant that plays a significant role in the pathogenesis of severe insulin resistance in this Chinese family.
9.Multi-system involvement characteristics and influencing factors of disease progression in Kennedy′s disease
Yifan LI ; Fei YANG ; Hongfen WANG ; Zhaohui CHEN ; Li LING ; Hongmei CHENG ; Xusheng HUANG ; Mao LI
Chinese Journal of Internal Medicine 2025;64(9):845-853
Objective:To investigate multi-system involvement in Kennedy′s disease and its association with disease progression.Methods:We retrospectively reviewed the clinical, laboratory, and electrophysiological data from 48 genetically confirmed patients with Kennedy′s disease at the Department of Neurology, First Medical Center of the Chinese PLA General Hospital, between February 2016 and February 2024. The disease progression rate was calculated based on the functional scores at baseline and follow-up. Correlation analyses and multiple linear regression models were employed to assess the relationships among clinical variables and to identify potential predictors of disease progression.Results:The age of muscle weakness onset ranged from 16 to 66 years (mean 42±11 years), with a diagnostic delay of 5.0 (3.0, 9.8) years. Lower limb weakness was the most common initial symptom in 72.9% (35/48) of patients, and 37.5% (18/48) exhibited non-motor manifestations prior to the onset of weakness. Core motor manifestations included bulbar weakness (89.6%, 43/48) and symmetric proximal limb weakness (83.3%, 40/48), frequently accompanied by gynecomastia (74.2%, 23/31) and sexual dysfunction (64.6%, 31/48). The median CAG repeat length was 43 (42, 46), which showed a significant negative correlation with the age at onset ( r=-0.406, P=0.004). Patients with CAG repeats > 43 had a higher prevalence of sexual dysfunction. Elevated serum muscle enzymes were observed in 97.9% (47/48), and abnormal sex hormone levels were detected in 81.2% (39/48). Sensory neuropathy was present in 68.1% (32/47), with CAG repeat length inversely correlating with compound muscle action potential (CMAP) amplitudes in the median ( β=-0.29; t=-2.27, P=0.029) and ulnar ( β=-0.22; t=-2.23, P=0.031) nerves. Low-frequency repetitive nerve stimulation (RNS) revealed a decrement in 43.3% (13/30) of patients, most commonly affecting the axillary and spinal accessory nerves. The disease progression rate was 1.3±0.3 (range: 0.5-2.0). Furthermore, serum creatine kinase-MB (CK-MB) levels were negatively correlated with disease progression rate ( r=-0.303, P=0.036). Conclusions:Kennedy′s disease presents with diverse initial manifestations and frequent multi-system involvement. Non-motor manifestations may precede muscle weakness, serving as valuable clues for early diagnosis. Widespread sex hormone abnormalities (particularly testosterone/luteinizing hormone dysregulation) support the role of androgen insensitivity in disease pathogenesis. Sensory neuropathies are frequent and not length-dependent. The presence of decremental responses on low-frequency RNS suggests neuromuscular junction dysfunction, which may underlie motor impairment in patients with Kennedy′s disease. Finally, serum CK-MB may serve as a potential biomarker for disease progression.
10.Clinical characteristics of congenital atresia of the oval window
Jiayu PAN ; Meixin CHEN ; Yang WANG ; Xingyu HUANG ; Xiaoxi CHEN ; Zhaohui LIU ; Chunlin ZHANG
Chinese Journal of Otorhinolaryngology Head and Neck Surgery 2025;60(10):1252-1258
Objective:To investigate the clinical features of patients with congenital atresia of the oval window (CAOW).Methods:A retrospective analysis was conducted on 7 cases (8 ears) of surgically confirmed CAOW treated at our department from July 2018 to July 2024. Among the cases, 1 patient had bilateral CAOW, and 4 patients had unilateral CAOW combined with other types of ossicular chain malformations in the contralateral ear. We collected and analyzed the clinical data, audiological features, and temporal bone HRCT results of all patients.Results:The 7 patients were diagnosed at ages ranging from 8 to 19 years, with a mean age of (13.2±6.9) years. None of the patients exhibited significant auricular deformities. All presented with conductive hearing loss or mixed hearing loss predominantly of the conductive type, with an intact tympanic membrane. The diagnosis of CAOW was confirmed via endoscopic tympanotomy, revealing a concave oval window area on the medial wall of the tympanic cavity, sealed by a bony plate. All 8 ears exhibited additional ossicular chain deformities. Stapes absence was present in all 8 ears. Partial absence of the incus long process was observed in 3 ears, while, abnormal bony connections between the incus long process and the promontory were seen in 4 ears, 1 ear had a short malleolar handle, 1 ear had a smaller than normal malleus volume. In addition, facial nerve deformities were found in 6 ears, with 4 ears showing bifurcation of the facial nerve and 2 ears showing facial nerve obscuration of the oval window. Pure-tone audiometry revealed that 62.5% (5/8 ears) of patients had air conduction (AC) thresholds≥60 dB preoperatively, with a mean pure-tone average (PTA) of (69.0±11.8) dB HL and a mean air-bone gap (ABG) of (52.0±7.0) dB. The mean AC threshold and ABG were higher in the low-frequency (125-1 000 Hz) range compared to the high-frequency (2 000-8 000 Hz) range (both P<0.05). Preoperative HRCT showed abnormalities in all patients, with 7 ears being diagnosable as CAOW. Although the remaining 1 ear could not be diagnosed as CAOW, stapes and incus long process absence were detected. Conclusion:CAOW is rare in clinical, as the patients with non-progressive conductive hearing loss (AC≥60 dB, ABG≥50 dB) since childhood, intact tympanic membrane without malformations of auricle and external auditory canal, and thick bony plate covered the oval window of the HRCT imaging, CAOW should be highly suspected, which could be confirmed by the exploratory tympanotomy.

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