Gallbladder cancer with delayed diagnoses, as a malignant tumor diagnosed intraoperatively or postoperatively incidentally in benign gallbladder diseases, radical surgical treatment is its core treatment, and the necessity of re-operation, timing of surgery, choice of surgical procedure, definition of resection extent, and preoperative neoadjuvant therapy still remain controversial. Re-operation for gallbladder cancer with delayed diagnoses needs to balance tumor radicalization, postoperative risk, and survival benefit, and combining neoadjuvant therapy to facilitate radical treatment, and develop individualized protocols. The multidisciplinary team should integrate innovative technologies and multi-center prospective studies to promote the development of precise treatment strategies for gallbladder cancer with delayed diagnoses and optimize the survival of patients.

Objective:To summarize the clinical features of 14 cases with clinically and genetically diagnosed progressive pseudorheumatoid dysplasia(PPRD) and analyze the characteristics of joint lesions caused by PPRD to provide references for clinical diagnosis and treatment of PPRD.Methods:A retrospective analysis was made on the clinical data and genetic test data of 14 patients admitted to Children′s Hospital, Capital Institute of Pediatrics and cooperative units, from December 2017 to September 2023.An observational retrospective analysis was performed on hospitalization and follow-up data, and correlation factors were analyzed using the Kendall correlation test.Results:The 14 patients, including 6 males and 8 females, were all Chinese Han children.The average age of onset was 6.12 years(2-11 years), the average age of diagnosis was 10.61 years old(5-17 years old), and the average duration of the disease was 4.46 years(0.5-6.0 years) at diagnosis.The number of joint involvements ranged from 4 to 14, with an average of 9.20.There were 12 patients with claudication as the first symptom.All these 14 children had limited hip mobility and no obvious hip pain.The hip joint imaging indicated that space narrowing occurred in 11 cases, femoral neck shortening in 2 cases, femoral head displacement in 4 cases, and articular surface sclerosis, cystic degeneration or bone destruction in 8 cases.The joints of the four limbs were mainly manifested as enlargement of the proximal interphalangeal joints and metacarpophalangeal joints of both hands.The other joints were knees(11 cases), elbows(8 cases), wrists(7 cases) and ankles(5 cases) in the order of easy involvement, and the shoulder joint was less involved(1 case).The spine was mainly characterized by changes in the curvature, and limited movement was found in 8 cases.Thoracic and lumbar spines were mainly involved(13 cases), while cervical involvement was rare(1 case).The duration of the disease at diagnosis was positively correlated with the number of joint involvements and the number of joint mobility limitations( r=0.584, 0.671; P=0.007, 0.002).In this study, 8 children were misdiagnosed as juvenile idiopathic arthritis, and the longest misdiagnosis time was 6 years.All the 14 children had CCN6 gene mutations, including 10 complex heterozygous mutations and 4 homozygous mutations.Five children had c. 342T>G and c. 667T>G, 4 children had c. 589 + 2(IVS4)T>C, 3 children had c. 866dup, and 2 children had c. 136C>T and c. 624dupA. Conclusions:PPRD is characterized by multiple joint involvements, among which hip involvement is the most common.The lesions are serious and easily misdiagnosed as juvenile idiopathic arthritis.The number of affected joints increases gradually with the prolongation of the disease course.

OBJECTIVE To investigate the effect of Lycii Fructus-Chrysanthemi Flos herbal pair on the occurrence and progres-sion of light source-induced myopia in guinea pigs.METHODS The three-band fluorescent lamp was used to irradiate guinea pigs to establish a model of light source-induced myopia.Lycii Fructus-Chrysanthemi Flos herbal pair extract was added in the feed to raise guinea pigs.Ophthalmic A-type ultrasonic measuring instrument was used to detect the eye axis.HE staining was used to observe pathological damage to the retina of the guinea pigs.Enzyme-linked immunosorbent assay(ELISA)was used to detect dopamine(DA)and melatonin(MT)levels in guinea pig retinas,and transcriptomic analysis and other methods were used to explore the effects of Lycii Fructus-Chrysanthemi Flos on light-induced myopia in guinea pigs.RESULTS After 6 weeks,compared with the normal group,the eye axis of model group guinea pigs increased significantly(P<0.01),thus,the myopia model was successfully established.Lycii Fruc-tus-Chrysanthemi Flos herbal pair extract could effectively slow down the thickening of the lens and the deepening of the vitreous cavi-ty,thereby delaying the overgrowth of the eye axis in model guinea pigs.The retinal tissue of guinea pigs in the model group became thinner,the thickness and number of cells in the inner and outer nuclear layer of the retina were significantly reduced,the ganglion nu-clei were sparse,the vacuolization of the ganglion cell layer was obvious.Lycii Fructus-Chrysanthemi Flos extract could protect retinal cells.The thickness and cell number of the retinal inner nuclear layer and outer nuclear layer were increased,and ganglion cell nuclei were increased.ELISA assay showed that the concentrations of DA and MT in the retina of model group guinea pigs were significantly reduced(P<0.05),and the medium and high doses of Lycii Fructus-Chrysanthemi Flos herbal pair extract could effectively increase the concentration of dopamine in the retina and serum of guinea pigs in the model group(P<0.01,P<0.001).The differential genes screened by eye transcriptomics analysis were mainly enriched in PPAR signals pathway,collagen-containing extracellular matrix and other related pathways.ELISA results showed that the high dose of Lycii Fructus-Chrysanthemi Flos extract could significantly increase the content of PPARγ and COL1A1(P<0.01)and reduce the content of SMA(P<0.05).CONCLUSION The Lycii Fructus-Chry-santhemi Flos herbal pair extract has effects on delaying the occurrence and progression of light source-induced myopia in guinea pigs,protecting retinal cells,improving DA secretion disorders caused by artificial light environment,and regulating the level of PPAR,which has positive significance for the prevention and control of light source-induced myopia and provides a scientific basis for the ra-tional use of drugs in clinical practice and the creation of eye health products.

177Lu-prostate specific membrane antigen(PSMA)radio-ligand therapy has been approved abroad for advanced prostate cancer and has been in several clinical trials in China.Based on domestic clinical practice and experimental data and referred to international experience and viewpoints,the expert group forms a consensus on the clinical application of 177Lu-PSMA radio-ligand therapy in prostate cancer to guide clinical practice.

Objective:To analyze the clinical features of adult deficiency of adenosine deaminase 2 (DADA2) with neurological impairment.Methods:The clinical data of an adult DADA2 patient with concurrent neurological damage who visited the Department of Neurology, Mindong Hospital Affiliated to Fujian Medical University on September 18, 2023 were retrospectively analyzed. The clinical studies or case reports related to adult DADA2 with nervous system involvement from Pubmed, CNKI, and Wanfang databases were retrieved, and the clinical characteristics of adult DADA2 with neurological damage were summarized. The clinical data of children with nervous system involvement in the same study cohorts were also collected, and the clinical features of DADA2 between adults and children were compared.Results:The patient was a 30-year-old male, mainly presenting with manifestations of livedo reticularis, stroke and spastic paraplegia. Genetic testing showed a compound heterozygous mutation in the adenosine deaminase 2 ( ADA2) gene, and brain MRI showed lacunar infarcts in the right basal ganglia and thalamus, hypertrophic inferior olivary degeneration. The literature review found that a total of 22 adult DADA2 patients with neurological damage have been reported, with a onset age of 25 (19, 29) years. Stroke was the most common feature of neurological involvement in patients with this disease (17/22, 77.3%), followed by cranial nerve damage (7/22, 31.8%) and limb nerve damage (8/22, 36.4%). After the treatment with tumor necrosis factor (TNF) inhibitors, the condition of 17/20 patients remained stable or improved. Compared with pediatric DADA2 patients with concurrent neurological damage, the incidence of fever [12/22(54.5%) vs 48/59(81.4%)], arthritis [6/22(27.3%) vs 34/59(57.6%)], and hematological abnormalities [4/22(18.2%) vs 28/60(46.7%)] in adult DADA2 patients was significantly reduced, and the difference was statistically significant (χ 2=5.998, 5.907, 5.489, respectively, all P<0.05). Conclusions:Adult DADA2 with concurrent neurological damage generally onset in early adulthood, mainly manifested as stroke, and may also be accompanied by peripheral nerve damage. Adult patients have fewer systemic symptoms than children, and timely treatment with TNF inhibitors can lead to better outcomes.

OBJECTIVES: To evaluate the performance of different multi-modality fusion models for predicting radiation-induced oral mucositis (RIOM) following radiotherapy in patients with nasopharyngeal carcinoma (NPC). METHODS: We retrospectively collected the data from 198 patients with locally advanced NPC who experienced RIOM following radiotherapy at the Affiliated Tumor Hospital of Guangzhou Medical University from September, 2022 to February, 2023. Based on oral radiation dose-volume parameters and clinical features of NPC, basic classification models were developed using different combinations of feature selection algorithms and classifiers and integrated using a multi-criterion decision-making (MCDM)-based classifier fusion (MCF) strategy and its variant, the H-MCF model. The basic classification models, MCF model, the H-MCF model with a single modality or multiple modalities and other ensemble classifiers were compared for performances for predicting RIOM by assessing the area under the ROC curve (AUC), accuracy, sensitivity, and specificity. RESULTS: The H-MCF model, which integrated multi-modality features, achieved the highest accuracy for predicting severe RIOM with an AUC of 0.883, accuracy of 0.850, sensitivity of 0.933, and specificity of 0.800. CONCLUSIONS Compared with each of the individual classifiers, the multimodal multi-classifier fusion algorithm combining clinical and dosimetric modalities demonstrates superior performance in predicting the incidence of severe RIOM in NPC patients following radiotherapy.
Humans ; Nasopharyngeal Carcinoma/radiotherapy* ; Nasopharyngeal Neoplasms/radiotherapy* ; Retrospective Studies ; Stomatitis/diagnosis* ; Algorithms ; Radiation Injuries/diagnosis* ; Female ; Male ; ROC Curve

Objective:To study the clinical features of transthyretin amyloid polyneuropathy (ATTR-PN) caused by Ser77Tyr mutation.Methods:The clinical data of a patient with ATTR-PN caused by Ser77Tyr mutation, admitted to our hospital from Department of Neurology, Mindong Hospital Affiliated to Fujian Medical University, were retrospectively analyzed. Literature on patients with ATTR-PN caused by Ser77Tyr mutation in Pubmed, Web of Science, CNKI and Wanfang databases and those with ATTR-PN caused by Val30Met mutation in Pubmed and Web of Science were searched and screened, and clinical characteristics of these patients were extracted. The differences of clinical characteristics among patients with ATTR-PN caused by Ser77Tyr or Val30Met mutations were compared.Results:(1) Transthyroxin ( TTR) gene Sanger sequencing results showed Ser77Tyr heterozygous pathogenic mutation; Congo red staining of biopsy sample in the patient 2.5 years after onset showed amyloid deposition. (2) Seventy-eight patients with ATTR-PN caused by Ser77Tyr mutation were summarized, they mostly had onset at 50-60 years old; male patients had higher incidence (74.4%); most patients (78.0%) had positive family history; most patients had sensory symptoms as initial symptom (72.0%), which gradually progressed to extensive peripheral nerve involvement and combined with widespread heart damage (96.4%) over several years; electrophysiological examination mainly showed axonal damage and carpal tunnel syndrome; the tissue biopsy had high positive rate(84.8%). (3) There were 192 and 96 patients with ATTR-PN caused by early-onset and late-onset Val30Met mutations, respectively; compared with patients with ATTR-PN caused by early-onset Val30Met mutation, patients with ATTR-PN caused by Ser77Tyr mutation had significantly higher incidence of deep sensory disturbance (28.6% vs. 58.5%, P<0.05). Compared with patients with ATTR-PN caused by late-onset Val30Met mutation, patients with ATTR-PN caused by Ser77Tyr mutation had increased incidence of mild sensory disturbance (56.3% vs. 75.0%) and decreased incidence of limb weakness (65.6% vs. 48.3%), with significant differences ( P<0.05). ATTR-PN patients caused by Ser77Tyr mutation had significantly higher incidence of carpal tunnel syndrome than ATTR-PN patients caused by early-onset and late-onset Val30Met mutations (75.4% vs. 10.8% and 25.0%) and significantly higher incidence of cardiac damage than ATTR-PN patients caused by early-onset Val30Met mutation (96.4% vs. 80.5%, P<0.05). Conclusion:Ser77Tyr mutation has some distinctive clinical features: relatively balanced damage of large and small fibers, prominent carpal tunnel syndrome, and obvious heart disease; early identification of these features and administration of tissue biopsy and gene detection are helpful for early diagnosis.

Objective:To investigate the effect of motion correction (MC) on the calculated values of myocardial blood flow (MBF) and myocardial flow reserve (MFR) based on cadmium-zinc-telluride SPECT (CZT SPECT) images.Methods:Twenty-eight consecutive patients (10 males, 18 females, age: (60.75±11.62) years) with suspected or known coronary artery disease who underwent myocardial perfusion imaging (MPI) with dynamic CZT SPECT between June 2019 and August 2019 in TEDA International Cardiovascular Hospital were retrospectively analyzed. The MBF and MFR during rest imaging and stress imaging were quantitatively analyzed. Corridor 4DM software was used to calculate the stress MBF (sMBF) and MFR of the coronary artery branches and left ventricular (LV) before and after MC. The paired t test and Pearson correlation were used for data analysis. Results:The sMBF and MFR of LV before MC were (0.82±0.49) ml·min -1·g -1 and 1.69±0.68 respectively. After MC the two parameters increased to (1.05±0.64) ml·min -1·g -1 and 2.12±0.77 respectively ( t values: -4.87, -6.01, both P<0.001). The sMBF and MFR in left anterior descending (LAD), left circumflex (LCX), right coronary artery (RCA) and LV before MC were correlated with those after MC ( r values: 0.69-0.96, all P<0.001). If MFR <2.0 was used as the reference of impaired MFR, data before MC showed 19 patients (67.9%, 19/28) had impaired MFR, while 13 patients (46.4%, 13/28) had impaired MFR based on MFR values after MC. Conclusion:For MPI quantitative imaging with CZT SPECT, the calculated values of sMBF and MFR after MC is higher than those before MC, suggesting that MC is helpful to reduce the false positive results which may be caused by the " creep" effect of the heart.

ABSTRACT: Meningiomas are the most common primary intracranial neoplasm with diverse pathological types and complicated clinical manifestations. The fifth edition of the WHO Classification of Tumors of the Central Nervous System (WHO CNS5), published in 2021, introduces major changes that advance the role of molecular diagnostics in meningiomas. To follow the revision of WHO CNS5, this expert consensus statement was formed jointly by the Group of Neuro-Oncology, Society of Neurosurgery, Chinese Medical Association together with neuropathologists and evidence-based experts. The consensus provides reference points to integrate key biomarkers into stratification and clinical decision making for meningioma patients. REGISTRATION Practice guideline REgistration for transPAREncy (PREPARE), IPGRP-2022CN234.
Humans ; Meningioma/pathology* ; Consensus ; Neurosurgical Procedures ; Meningeal Neoplasms/pathology*

Objective:To investigate the diagnostic value of myocardial blood flow quantitative imaging with cadmium zinc telluride (CZT) SPECT in patients with high-risk coronary artery disease (CAD).Methods:A total of 148 patients (82 males, 66 females, age: (63.8±8.2) years) who successfully completed CZT SPECT dynamic acquisition and routine SPECT myocardial perfusion imaging (MPI) in TEDA International Cardiovascular Hospital from November 2018 to October 2020 were analyzed retrospectively. According to the results of coronary angiography (CAG), patients were divided into two groups: high-risk CAD group and low-to-medium-risk CAD group. At the case level, quantitative parameters (stress myocardial blood flow (sMBF), rest myocardial blood flow (rMBF) and myocardial flow reserve (MFR)), semi-quantitative parameters (summed stress score (SSS), summed rest score (SRS), summed difference score (SDS) and transient ischemic dilation (TID)) and left ventricular function parameters of two groups were compared. Diagnostic accuracy was evaluated by ROC curve analysis. At the vascular level, the correlation between the degree of coronary artery stenosis and some parameters was analyzed. Mann-Whitney U test, logistic regression, Spearman rank correlation analysis and DeLong test were used for statistical analysis. Results:Case level analysis showed that MFR and sMBF in high-risk CAD group were significantly lower than those in low-to-medium-risk CAD group (1.36(0.87, 1.64) vs 2.74(2.30, 3.33), 1.06(0.69, 1.48) vs 2.50(1.73, 2.95) ml·g -1·min -1; U values: 628.0 and 853.5, both P<0.001). MFR and SDS were independent predictors of high-risk CAD patients (odds ratio ( OR)=0.251(95% CI: 0.136-0.464), P<0.001; OR=1.188(95% CI: 1.026-1.375), P=0.021), and MFR was more capable of predicting high-risk CAD. MFR and sMBF had the highest accuracy in diagnosing high-risk CAD (AUCs: 0.885 and 0.844). Differences of AUCs between MFR and other parameters were statistically significant ( z values: 1.99-6.77, all P<0.05), and the best diagnostic cut-off value was ≤1.83 (sensitivity: 85.90%; specificity: 85.71%). Vascular level analysis showed that MFR and sMBF( R2 values: 0.39 and 0.35, both P<0.001) were negatively correlated with the degree of coronary stenosis, while SSS, SRS and SDS ( R2 values: 0.22, 0.12 and 0.14, all P<0.001) were positively correlated with the degree of coronary stenosis. Conclusion:Compared with conventional SPECT MPI, CZT SPECT myocardial blood flow quantitative imaging has better diagnostic efficacy and clinical value in patients with high-risk CAD.