Objective To investigate diagnostic magnetic resonance imaging features of orbital Langerhans cell histocytosis (LCH)and improve its diagnostic accuracy.Methods The symptoms and image data of fourteen histophathology verified orbital LCH cases are reviewed and analyzed.Results Nine patients had swollen eyelids,accompanying with symptoms of inflammation, esphthalmos and orbital masses.One case had cough symptom and another had diabetes insipidus.Of these fourteen cases,seven occurred in right orbital,six occurred in left orbital and one involved bilateral orbital.As to the location of LCH,six cases located in super-lateral wall of the orbit,five cases located in lateral wall of the orbital,and three cases located in roof of orbital.On MRI, thirteen cases lesions show hypo or iso signal intensity on T1 WI,and eleven cases lesions show heterogeneous hyper or iso signal in-tensity on T2 WI.The lesions of eosinophilic granuloma has clear border,which differentiate it from other types.After contrast en-hancement,MR imaging showed marked inhomogeneous enhancement.Conclusion MRI is the primary modality in diagnosing of or-bital LCH,clearly and accurately manifesting the extent of orbital LCH.It will be helpful to diagnose LCH timely if combining with clinical data.MR could provide reliable information for making surgical operation and treatment plan.

Objective To investigate the effect of brain natriuretic peptide (BNP) on serum angiotensin-converting enzyme (ACE) 2 levels in acute heart failure patients with reduced ejection fraction (HFrEF). Methods A total of 106 patients with acute HFrEF were selected, and were divided randomly into control group and trial group. The control group was under routine treatment, while the trial group was under routine treatment combined with lyophiluzed recombinant human BNP for 24-hour. Cardiac functional parameters were measured by echocardiography both at the enrollment and the end of 7-day treatment. Serum levels of ACE2 and N-terminal pro-BNP (NT-proBNP) were determined using commercially available ELISA kits at the enrollment, the end of 24-hour treatment, and the end of 7-day treatment,respectively. Results A total of 103 patients with acute HFrEF were enrolled (control group=51, trial group=52). There were no significant differences in the use of drugs (e.g., aspirin) and serum biochemical indices (e.g. cardiac troponin I, creatinine) before treatment between these two groups. Compared to systolic blood pressure (SBP) at admission, SBP on the second day after treatment were significantly decreased in two groups (P<0.05). Compared to left ventricular ejection fraction (LVEF) at admission, LVEF values were significantly elevated on the seventh day after treatment in two groups ( P<0.05). There were no significant differences in SBP, diastolic blood pressure (DBP), and LVEF at admission between these two groups (P>0.05);there were also no significant differences in DBP on the second day after treatment, and LVEF on the seventh day after treatment (P>0.05), while SBP was significantly higher on the second day after treatment in control group than that of trial group (P < 0.05). Serum levels of NT-proBNP were decreased with the prolongation of time in two groups. Serum levels of ACE2 were decreased with the prolongation of time in control group, while were increased initially following decreased (which were still higher on the seventh day after treatment than that at admission) with the prolongation of time in trial group. Serum levels of NT-proBNP were higher after 2 days treatment or 7 days of treatment in control group than those of trial group, while serum levels of ACE2 were decreased after 2 days of treatment or 7 days of treatment in control group than those of trial group (P<0.05). Conclusion Patients with acute HFrEF may benefit from BNP by increasing serum ACE2 levels.

Purpose To investigate the diagnosis,differential diagnosis and clinical manifestation of primary plasma cell leukemia (PPCL) and lymphoma with increased plasma cell.Methods Through clinical data and cell morphology,flow cytometry (FCM),immunofixation electrophoresis and immunohistochemistry of EliVision two-step examination were used to analyze 7 cases of PPCL and 3 cases of lymphoma with increased plasma cell.Results All patients with PPCL and lymphoma with increased plasma cell presented with anemia,thrombocytopenia,fever,liver and spleen and lymph node swelling.The proportion of plasma cells in peripheral blood morphology were larger than 20％,accompanied by morphological abnormality.FCM of peripheral blood showed all 7 cases of PPCL expressed CD38 and CD138,CD56 expression in the 2 cases and CD20 in the 2 cases.The light chain (Lamda,Kappa) showed a monoclonal restricted expression,which was consistent with the diagnosis of PPCL.CD19 and CD45 were weakly positive in 3 cases of lymphoma with increased plasma cell,CD38 and CD138 were positive,and no restricted expression was found in light chain IgL,wich belonging to the immunophenotypes of normal plasma cells.Of 3 cases of light chain (Ig) without restrictive expression,2 of them were angioimmunoblastic T-cell lymphoma (ATCL) and 1 case was CD30-positive sinusoidal large B-cell lymphoma (CD30 + SLBCL) that confirmed by lymph node biopsy and pathological examination.Conclusion The PPCL and lymphoma with increased plasma cell have the same clinical manifestations and similar morphological characteristics of blood cells.The diagnosis of PPCL should be combined with immunoelectrophoresis and FCM,and the diagnosis of lymphoma with increased plasma cell needs to be confirmed by histological examination of lymph nodes.

Objective:To evaluate the efficacy of modified femoral neck osteotomy(mFNO)in the surgical treatment of patients with ankylosing spondylitis(AS)and severe spinal kyphosis combined with hip flexion contracture.Methods:A retrospective analysis was conducted on 61 AS patients(103 hips)with spinal kyphosis and hip flexion contracture who underwent pedicle subtraction osteotomy(PSO)and total hip arthroplasty(THA)from January 1,2019 to November 15,2023.Data on mFNO operation time,blood loss,preoperative and postoperative values of the angle of the trunk and lower limb(ATL),hip passive range of motion(ROM),visual analogue scale(VAS),and incidence of in-hospital compli-cations were recorded.Statistical analysis was performed using paired-samples t test.P＜0.05 was con-sidered statistically significant.Results:The study ultimately included 10 cases,9 males and 1 female,with an average age of(41.30±9.03)years.These patients underwent surgery for a total of 52 times,including 19 hips both receiving mFNO and THA,and 14 times PSO.The average operation time for nine bilateral mFNO was(133.11±34.81)min,with blood loss of(433.33±187.10)mL.A unilateral mFNO took 60 min with 200 mL of blood loss.The preoperative ATL of 19 hips was 40.37°±13.66°,and the postoperative ATL value was 88.47°±12.46°(P＜0.05).The preoperative VAS score was 0,while the postoperative VAS score was 5.95±1.51(P＜0.05).The preoperative hip extension ROM was 37.37°±18.13°,while the postoperative hip extension ROM was-4.95°±21.24°(P＜0.05).Hip flexion ROM improved from 37.37°±18.13° to 50.79°±20.36° after FNO(P＜0.05).There were three cases of in-hospital complications(3/52,5.67％):One case of postoperative atelectasis fol-lowing PSO(1/52,1.92％),one greater trochanter fracture identified during THA(1/52,1.92％),and one early dislocation post-THA(1/52,1.92％).Conclusion:mFNO significantly improves the ATL in AS patients with severe spinal kyphosis combined with hip flexion contracture,facilitating PSO and THA surgeries.

Objective: To explore the etiology, clinical manifestation, diagnosis and treatment of white spongy spot nevus, so as to provide reference for clinical diagnosis and treatment. Methods : The clinical data and related literature of a case of white cavernous nevus in oral cavity were retrospectively analyzed. Results : White spongy nevus is a rare autosomal dominant hereditary disease with a family history. The mutations of keratin gene K4 and K13 in patients with white spongy nevus are considered to be the main causes. The disease usually starts in children and adolescents and tends to be stable in adulthood. It is characterized by extensive white water-wave folds on the mucosa, soft texture, and affects the bilateral buccal mucosa. Pathological examination usually shows excessive keratosis of epithelial cells, edema and vacuolation in spinous cells, while basal cells are generally normal. In clinic, it should be differentiated from oral leukoplakia, oral lichen planus and oral candidiasis. At present, there is no specific treatment method. Retinoic acid is often applied locally and gargle is used to keep oral hygiene and cleanliness. Patients can not be treated without conscious symptoms. The prognosis of the disease is good and there is no tendency of malignancy. Conclusion White spongy nevus is very rare and easily missed by clinicians. Diagnosis mainly depends on medical history, clinical manifestations and pathological examination. Future research directions should be devoted to finding more effective treatment.

Objective:To investigate the specimen quality of intersphincteric resection with transabdominal transanal mixed approach for rectal cancer in the Chinese Transanal Total Mesorectal Excision Registry Collaborative (CTRC) database.Methods:The retrospective case-control study was conducted. Based on the concept of real-world research, the clinicopathological data of 281 pati-ents with rectal cancer in the CTRC database who underwent intersphincteric resection with trans-abdominal transanal mixed approach in 19 medical centers, including the Beijing Friendship Hospital of Capital Medical University et al, from November 15,2017 to December 31,2023 were collected. There were 196 males and 85 females, aged 61(range, 27-87)years. Observation indicators: (1) preoperative examinations; (2) neoadjuvant therapy; (3) postoperative examinations; (4) analysis of influencing factors for positive circumferential margin in surgical specimen of intersphincteric resec-tion for rectal cancer. Measurement data with normal distribution were represented as Mean±SD. Measurement data with skewed distribution were represented as M(range). Count data were described as absolute numbers or percentages. The chi-square test was used for univariate analysis. Logistic regression model was used for multivariate analysis. Results:(1) Preoperative examinations. Of the 281 patients, 234 cases underwent preoperative pelvic magnetic resonance imaging (MRI) examina-tion. There were 2 cases in clinical stage T0, 3 cases in clinical stage T1, 58 cases in clinical stage T2, 137 cases in clinical stage T3, 24 cases in clinical stage T4, 3 cases in clinical stage Tx, 7 cases missing clinical T staging data. There were 87 cases in clinical stage N0, 68 cases in clinical stage N1, 60 cases in clinical stage N2, 9 cases in clinical stage Nx, 10 cases missing clinical N staging data. There were 30 cases with mesorectal fascia invasion, 53 cases with extramural venous invasion. The distance from lower margin of tumor to anal margin was 41.9(range, 1.0-80.0)mm. (2) Neoadjuvant therapy. Of the 281 patients, 125 cases underwent neoadjuvant therapy, including 39 cases receiving chemo-therapy alone, 6 cases receiving short-course simultaneous chemoradiotherapy, 5 cases receiving short-course simultaneous chemoradiotherapy and delayed surgery, 48 cases receiving long-course simultaneous chemoradiotherapy, 2 cases receiving other treatments, and 25 cases missing neoadju-vant therapy data. (3) Postoperative examinations. Of the 281 patients, 249 cases achieved R 0 resection, 9 cases achieved R 1 resection, and there were 23 cases missing surgical margin data. The maximum tumor diameter, the number of lymph nodes harvested and positive rate of vessel carcinoma embolus were 30.0(range, 0.5-200.0)mm, 13(range, 0-70) and 27.55%(73/265) in 281 patients. There were 252 patients with circumferential margin records, showing positive in 15 cases, with a positive rate as 5.95%(15/252). The minimum distance from deep part of tumor to circumferential margin was 7.0(range, 0-150.0)mm in 252 patients. There were 85 cases with distal margin records, showing positive in 1 case, and the distance from lower margin of tumor to distal margin was 10.0(range, 0-202.0)mm. There were 273 patients with specimen integrity records, which showed intact specimen in 208 cases, fair specimen in 58 cases, poor specimen in 4 cases, unevaluated specimen in 3 cases. There were 7 cases with rectal perforation. Of the 281 patients, cases in pathological stage T0, Tis, T1, T2, T3, T4 were 14, 5, 22, 107, 113, 12, respectively, and there were 8 cases missing pathological T staging data. Of the 281 patients, cases in pathological stage N0, N1a, N1b, N1c, N2a, N2b were 176, 27, 27, 11,20, 12, respectively, and there were 8 cases missing pathological N staging data. Of the 281 patients, there were 4 cases with distant metastasis, 262 cases without distant metastasis, 5 cases not evaluated, and 10 cases missing tumor metastasis data. Of the 125 patients undergoing neoadjuvant therapy, there were 85 cases with tumor regression grade records, including 16 cases as grade 1, 27 cases as grade 2, 19 cases as grade 3, 15 cases as grade 4, 8 cases as grade 5. (4) Analysis of influencing factors for positive circumferential margin in surgical specimen of intersphincteric resection for rectal cancer. Results of univariate analysis showed that preoperative T staging on preoperative pelvic MRI, mesorectal fascia invasion, extramural venous invasion, pathological T staging, and pathological N staging were related factors for positive circumferential margin in surgical specimen of intersphincteric resection for rectal cancer ( P<0.05). Conclusions:Intersph-incteric resection with transabdominal transanal mixed approach has good specimen quality and low positive rate of surgical margin. T staging on preoperative pelvic MRI may be related to positive circumferential margin after intersphincteric resection for rectal cancer.

Objective:To analyze the quality of surgical specimens of rectal cancer in the Chinese transanal total mesorectal excision (taTME) registry collaborative (CTRC) database.Methods:The retrospective and descriptive study was conducted. Based on the concept of real-world research, the clinicopathological data of 1 761 patients with rectal cancer in the CTRC database who underwent taTME in 40 medical centers, including the Beijing Friendship Hospital of Capital Medical University et al, from November 15, 2017 to December 31, 2022 were collected. There were 1 212 males and 549 females, aged 62(range, 53-68)years. Observation indicators: (1) preoperative examinations; (2) neoadjuvant therapy; (3) postoperative examinations. Measurement data with skewed distri-bution were represented as M(range). Count data were described as absolute numbers. Results:(1) Preoperative examinations. Of the 1 761 patients, 1 324 patients underwent preoperative pelvic magnetic resonance imaging examination, and the results showed that 4 cases as clinical T0 stage, 30 cases as clinical T1 stage, 250 cases as clinical T2 stage, 828 cases as clinical T3 stage, 141 cases as clinical T4 stage, 11 cases as clinical Tx stage, 60 cases missing clinical T staging data, 490 cases as clinical N0 stage, 373 cases as clinical N1 stage, 311 cases as clinical N2 stage, 86 cases as clinical Nx stage, 64 cases missing clinical N staging data, 156 cases with mesorectal fascia invasion, 223 cases with extraintestinal blood vessels invasion. The distance from lower margin of tumor to anal margin of 1 324 patients was 50(range, 40-60)mm. (2) Neoadjuvant therapy. Of the 1 761 patients, 873 patients underwent neoadjuvant therapy, including 17 cases receiving radiotherapy alone, 155 cases receiving chemotherapy alone, 43 cases receiving short-course simultaneous chemoradiotherapy, 26 cases receiving short-course simultaneous chemoradiotherapy and delayed surgery, 1 case receiving contact radiotherapy, 277 cases receiving long-course simultaneous chemoradiotherapy, 9 cases receiving other treatments, and 345 cases missing neoadjuvant therapy data. (3) Postoperative examinations. Of the 1 761 patients, 1 584 cases achieved R 0 resection, 23 cases achieved R 1 resection, 1 case achieved R 2 resection, and there were 153 cases missing surgical margin data. The tumor diameter, number of lymph nodes harvest and positive rate of intravascular tumor thrombus were 30(range, 20-45)cm, 13(range, 10-17) and 20.794%(330/1 587) in 1 761 patients. There were 1 647 patients with circumferential margin records, which showed positive in 51 cases, and the minimum distance from deep part of tumor to circumferential margin was 5(rang, 3-13)mm in 1 647 patients. There were 547 cases with distal margin records, which showed positive in 4 cases, and the distance from lower margin of tumor to distal margin was 20(10-25)mm in 547 cases. There were 1 698 patients with specimen integrity records, which showed intact specimen in 1 436 cases, fair specimen in 233 cases, poor specimen in 8 cases, unevaluated specimen in 21 cases, and there were 20 cases with rectal tube perforation. Of the 1 761 patients, cases as pathological T0 stage, Tis stage, T1 stage, T2 stage, T3 stage, T4 stage was 103, 23, 145, 515, 712, 179, respectively, and there were 4 cases of pathology that could not be evaluated and 80 cases missing pathological T staging data. Of the 1 761 patients, cases as pathological N0 stage, N1a stage, N1b stage, N1c stage, N2a stage, N2b stage was 1 117, 189, 133, 66, 109, 68, respectively, and there were 79 cases missing pathological N staging data. Of the 1 761 patients, there were 79 cases with distant metastasis, 1 591 cases without distant metastasis, and 91 cases without data of tumor metastasis. Of the 873 patients undergoing neoadjuvant therapy, there were 405 patients with tumor regression grade records including 105 cases as grade 1, 142 cases as grade 2, 91 cases as grade 3, 43 cases as grade 4, 24 cases as grade 5. Conclusions:In China, the quality of surgical specimens of taTME for rectal cancer is good with low positive rate of resection margin. It is recommended that using a formatted postoperative pathological report for good quality control of pathological report of surgical specimen.