Pulmonary lymphangioleiomyomatosis is a rare disease characterized by the abnormal proliferation of pulmonary lymphatic smooth muscle cells. It is common in women and often accompanied by recurrent pneumothorax, chylothorax and progressive dyspnea, imaging characterized by diffuse cystic lesions in both lungs. Pulmonary lymphangioleiomyomatosis progresses aggressively and has a very poor prognosis, with a lack of effective medical treatment options in the advanced stages. Lung transplantation is a safe and effective method for the treatment of advanced pulmonary lymphangioleiomyomatosis, which may significantly improve the survival rate and quality of life of patients. The median survival period after surgery can reach 12 years. This article reviews the pathogenesis, diagnosis, treatment of pulmonary lymphangioleiomyomatosis, and the current status and existing problems of lung transplantation in pulmonary lymphangioleiomyomatosis, aiming to provide a reference for the clinical treatment and subsequent research of pulmonary lymphangioleiomyomatosis.

Objective:The objective of this study is to sequence whole genome of the rabies virus in the saliva specimen of a suspected rabies case with onset of rabies from a wildlife (hog badger) bite wound in Sichuan province, where the genetic variation characteristics of the virus was analyzed at the molecular level, so as to understand the prevalence and mutation of wildlife rabies virus in Sichuan province.Methods:Total viral RNA was extracted from the saliva specimen of the suspected rabies case. Then, rabies virus sequences were amplified using PCR with specific primers; the gene fragments obtained were sequenced, and the sequences obtained were spliced using biological software to obtain the whole genome sequence of the rabies virus strain. The genetic variation characteristics of the whole genome were analyzed.Results:The whole genome nucleotide sequence of a strain of rabies virus of hog badger origin (hereinafter referred to as SCR23-052) was obtained by sequencing, and NCBI online BLAST and comparison with several reference sequences showed that the composition and structure of the whole genome sequence of SCR23-052 conformed to the characteristics of the Lyssavirus under the Rhabdoviridae; the highest similarity in nucleotide and amino acid sequences in various gene regions was observed between SCR23-052 and the strains of Ningxia (J) and Chongqing (CQ92, 02050CHI). The sequence variability of SCR23-052 genome was significantly lower at the amino acid level than that at the nucleotide level, which indicated that most of the nucleotide variants in the protein-coding genes belonged to synonymous mutations. Phylogenetic analysis showed that SCR23-052 belonged to genotype V, which did not show any obvious mutation in the major antigenic site of the glycoprotein, underwent amino acid glycosylation at positions 56 and 338 by the online site prediction, and showed the least amino acid difference compared with the signal peptide sequence of the vaccine strain CTN181. The virus in this study has an A→T mutation at position 332 in the nucleoprotein major antigenic site with all reference vaccine strains, and an L→V mutation at position 379 in the B-cell epitope with the CTN181 vaccine strain. SCR23-052 was consistent with both genotype V reference strains at the nucleoprotein study site.Conclusions:The whole genome sequence of a wildlife strain of genotype V rabies virus of hog badger origin was obtained, which was different from that of the genotype I strain of rabies virus of dog origin that previously reported to be prevalent in Sichuan. The genome sequence of SCR23-052 differed from that of the reference vaccine strains to a varying degree, but the main virulence characteristics remained unaltered.

Objective To investigate the clinical value of high resolution vascular wall imaging(HRVWI)in the diagnosis and treatment of central nervous system vasculitis(CNSV)in children.Methods A total of 36 children with CNSV underwent HRVWI examination were selected.The HRVWI imaging features,involved vessel types and stenosis degree were analyzed retrospectively and compared with magnetic resonance angiography(MRA).Combined with some patients'follow-up and reviewed imaging after treatment.Results HRVWI showed that all of the 36 children with CNSV had cerebral arteritis,and the affected inflammatory ves-sels showed varying degrees of focal arterial wall thickening and enhancement,mainly uniform thickening and centripetal enhance-ment of the affected arterial wall.Most were mid-enhanced,and the stenosis degree was mostly at level 1(21/36).According to the type of involvement cerebrovascular,there were 26 cases of large-medium vessel type,6 cases of small vessel type and 4 cases of mixed type.Compared with MRA,HRVWI had a higher sensitivity in diagnosing CNSV(P＜0.05).13 cases were followed up after treat-ment,and HRVWI showed cerebral vasculitis relief in 8 cases and progression in 5 cases,consistent with changes in clinical symp-toms and laboratory indicators.Conclusion The imaging features of CNSV in children with different causes are different.HRVWI has a high sensitivity in the diagnosis of CNSV in children.HRVWI plays an important role in the diagnosis and treatment of CNSV in children.

Objective:To investigate the effects of peritoneal dialysis combined with hemodialysis on improving cardiac and renal function, peritoneal function, and nutritional status, and reducing complications in patients with end-stage renal disease.Methods:A total of 90 patients with end-stage renal disease who received treatment at Lishui Municipal Central Hospital and Lishui People's Hospital from January 2019 to May 2022 were retrospectively included in this study. The patients were divided into a control group and an observation group, with 45 patients in each group, based on the treatment methods used. The control group received peritoneal dialysis treatment, while the observation group received a combination of peritoneal dialysis and hemodialysis treatment. The changes in cardiac and renal function, peritoneal function, nutritional status, and the occurrence of complications were observed in both groups.Results:After treatment, the observation group showed significantly lower values in the cardiothoracic ratio [(57.02 ± 3.35)%], brain natriuretic peptide [(8 849.34 ± 124.65) ng/L], left ventricular mass index [(181.32 ± 8.56) g/m2], blood urea nitrogen [(14.04 ± 1.94) mmol/L], and serum creatinine [(181.47 ± 27.06) μmol/L] compared with the control group [(58.92 ± 3.11)%, (15 126.39 ± 322.82) ng/L, (187.28 ± 8.95) g/m2, (18.49 ± 2.82) mmol/L, (196.56 ± 31.07) μmol/L, t = 2.79, 121.68, 3.23, 8.72, 2.46, all P < 0.05]. Additionally, the observation group had significantly higher levels of ejection fraction [(63.47 ± 5.23)%], albumin [(52.98 ± 6.37) g/L], and hemoglobin [(114.94 ± 13.61) g/L] compared with the control group [(60.46 ± 5.18)%, (47.01 ± 6.04) g/L, (98.04 ± 10.93) g/L, t = -2.74, -4.52, -6.49, all P < 0.05]. The observation group also exhibited a significantly lower dialysate/plasma creatinine ratio [(0.56 ± 0.09)] and a lower level of β 2-microglobulin [(18 032.29 ± 718.11) mg/L] compared with the control group [(0.61 ± 0.07), (18 424.42 ± 736.43) mg/L, t = 2.54, 2.06, both P < 0.05]. The incidence of complications in the observation group was 11.11% (5/45), which was significantly lower than that in the control group [31.11% (14/45), χ2 = 4.27, P < 0.05]. Conclusion:Peritoneal dialysis combined with hemodialysis can improve cardiac and renal function, peritoneal function, and nutritional status and decrease the incidence of complications in patients with end-stage renal disease. This approach deserves to be clinically promoted. The findings of this study are of significant innovation and scientific value.

Objective:To investigate clinical outcome and the risk factors for death in acute pancreatitis (AP) patients complicated with acute kidney injury (AKI).Methods:The clinical data of 232 AP patients complicated with AKI admitted to the Center of Severe Pancreatitis of Jinling Hospital Affiliated to Nanjing University School of Medicine from January 2016 to December 2020 were retrospectively analyzed. Patients were divided into survival group ( n=162) and death group ( n=70) based on the survival status. The two groups' clinical characteristics, biochemical indexes, and renal function indexes were compared. Univariate analysis and multivariate logistic regression analysis were used to analyze the independent risk factors for death in AP patients complicated with AKI. Results:Sixteen patients(6.9%) among the 232 had AKI Ⅰ, 15(6.5%) had AKI Ⅱ, and 201(86.6%) had AKI Ⅲ. Forty-one patients (17.7%) became AKI with a disease course <7 days, 184 patients (79.3%) gradually progressed to acute kidney disease with a disease course of 7-90 days, and 7 patients (3.0%) eventually progressed to chronic kidney disease with a disease course >90 days. Renal replacement treatment (RRT) was administered in 179 patients (77.2%), lasting an average of 14 (7-25) days. 138 patients (59.5%) had their renal function recovered while they were hospitalized, including 9 patients (6.5%) who did so within 7 days, 69 patients (50.0%) within 30 days, and 127 patients (92.0%) within 90 days. The average recovery time was 16 (7-28) days. Seventy patients (30.2%) died during hospitalization, including 8(3.5%) within 7 days, 42(18.1%) within 30 days, and 68(29.3%) within 90 days. Univariate analysis revealed that the proportions of biliary etiology, neutrophil to lymphocyte ratio (NLR), serum cystatin C, sequential organ failure assessment(SOFA) score, AKI Ⅲ proportion, number of patients undergoing RRT, and duration of AKI were significantly higher in the death group compared to the survival group. The number of patients complicated by infected pancreatic necrosis (IPN) and having surgical intervention was also significantly greater than that in the survival group, while the proportion of patients whose renal function recovered was much lower than that in the survival group. The differences were all statistically significant (all P value <0.05). Multivariate logistic analysis showed that SOFA( OR=1.182, 95% CI 1.000-1.396, P=0.049), and IPN( OR=8.403, 95% CI 3.748-18.838, P<0.001) were independent risk factors for death. Conclusions:SOFA score and IPN at admission were independent risk factors for death in AP patients with AKI. Vigilance should be given as soon as possible to improve the outcome of patients through clinical intervention.

Tocilizumab has been reported to attenuate the "cytokine storm" in COVID-19 patients. We attempted to verify the effectiveness and safety of tocilizumab therapy in COVID-19 and identify patients most likely to benefit from this treatment. We conducted a randomized, controlled, open-label multicenter trial among COVID-19 patients. The patients were randomly assigned in a 1:1 ratio to receive either tocilizumab in addition to standard care or standard care alone. The cure rate, changes of oxygen saturation and interference, and inflammation biomarkers were observed. Thirty-three patients were randomized to the tocilizumab group, and 32 patients to the control group. The cure rate in the tocilizumab group was higher than that in the control group, but the difference was not statistically significant (94.12% vs. 87.10%, rate difference 95% CI-7.19%-21.23%, P = 0.4133). The improvement in hypoxia for the tocilizumab group was higher from day 4 onward and statistically significant from day 12 (P = 0.0359). In moderate disease patients with bilateral pulmonary lesions, the hypoxia ameliorated earlier after tocilizumab treatment, and less patients (1/12, 8.33%) needed an increase of inhaled oxygen concentration compared with the controls (4/6, 66.67%; rate difference 95% CI-99.17% to-17.50%, P = 0.0217). No severe adverse events occurred. More mild temporary adverse events were recorded in tocilizumab recipients (20/34, 58.82%) than the controls (4/31, 12.90%). Tocilizumab can improve hypoxia without unacceptable side effect profile and significant influences on the time virus load becomes negative. For patients with bilateral pulmonary lesions and elevated IL-6 levels, tocilizumab could be recommended to improve outcome.
Antibodies, Monoclonal, Humanized ; COVID-19/drug therapy* ; Humans ; SARS-CoV-2 ; Treatment Outcome

Objective:To construct a rat model of hypothalamic obesity by two point electrical damage to the ventromedial hypothalamus and arcuate nucleus.Methods:Twenty adult male SD rats of SPF grade were randomly divided into experimental group and sham operation group.A 25GA (0.45 mm) solid iron needle was used, the needle was coated with an insulating layer, and the tip exposed a 0.5 mm conductive area.With reference to The Rat Brain in Stereotaxic Coordinates and using the stereotactic instrument (AP: -2.6 mm, ML: ± 0.6 mm, DV: -9.6 mm) as the coordinate, 1.5 mA current was continuously applied for 25 s, the ventromedial nucleus (VMH) and arcuate nucleus (ARC) of bilateral brain in SD rats was damaged.During the experiment, the body weight(BW), food intake(FI) and water intake(WI) of the two groups were recorded regularly.The rats were sacrificed on the 28th day after the operation, and the changes of periprenal fat mass and body length were measured.The changes of liver and adipose tissue were detected by HE staining method, leptin by ELISA, leptin receptor(LEPR) by Western blot.Results:(1) The body weight of rats in the experimental group ((427.5±17.7)g) and weight gain ((208.5±14.8)g) were significantly increased compared with the rats in the control group((349.2±17.7)g), ((136.2±21.4)g)on the 28th day after operation ( t=7.661, 6.806, both P<0.001). (2) The daily food intake of rats in the experimental group ((44.2±6.6)g) on the 28th day after surgery was significantly higher than that in the control group ((23.0±3.6)g) ( t=6.918, P<0.001). There was no significant difference of the daily drinking water of rats between experimental group((37.5±12.1)ml) and the control group ((35.0±11.8)ml) ( t=0.361, P=0.726). (3) Perikidney fat mass of experimental group rats ((13.4±2.7)g) significantly increased 28 days after operation compared with control group rats((6.3±0.9)g)( t=4.250, P<0.05). The naso-anal length of experimental group((21.8±0.4)cm) was significantly decreased compared with the control group ((23.4±0.2)cm) ( t=-6.788, P<0.01). The Lee index of the experimental group (348.9±8.5) was significantly higher than that of the control group(305.5±4.3)( t=7.898, P<0.01). (4) The serum leptin content ((8 324.10±159.00)μg/L) of the experimental group rats at 28 days after surgery was significantly higher than that of the control group((2 705.31±407.10)μg/L) ( t=25.712, P<0.001). The lateral hypothalamus area (LHA) LEPR protein expression (1.3±0.1) in the experimental group was significantly higher than that in the control group (0.9±0.1) ( t=4.932, P<0.01). Conclusion:Two-point electrical damage to bilateral VMH and ARC of rats can establish hypothalamic obese rat model.

Objective ToexploretheclinicalmanifestationsandMRIfeaturesofnervoussystemdiseasecomplicationsinpediatric EV71relatedhand-foot-mouthdisease(HFMD).Methods Theclinicaldataof17EV71relatedHFMDpatientswithnervoussystemdisease wereanalyzedretrospectively.Results 4patientswerediagnosedwith meningitisandthebrain MRIshowedasymmetricunilateral ventricularenlargement.9patientswerediagnosedwithbrainstemencephalitis,amongwhich8patientsshowedthecraniocerebral MRIfeaturesthatthelesionswerelocatedonthedorsalsideofthepontomedullaryjunction,andtheT2imageshowedahighsignal withasymmetrycharacteristic.1caseofbrain MRIdisplayedthatthelesionwaslocatedinthemidbrain,andT2imagewasahigh signalwithsymmetricalfeatures.4patientswerediagnosed withacuteflaccidparalysis.1casehadbrainstem encephalitis,andthe brain MRIshowedthatthelesion waslocatedonthedorsalsideofthepontomedullaryjunction,andthe MRIofthespinalcord showedthelesionintheC1-C7segment.1caseofspinalcordMRIshowedthatthelesionwaslocatedatT2-L1segment,1caseof spinalMRIshowedthelesionlocatedinT8-T12segment;1caseofspinalcord MRIshowedthatthelesionwaslocatedatT9-L1 segment,andsagittalT2 wasahighsignal,andthelesionconcentratedintheanteriorhornofthespinalcordand (or)theanterior rootofthespinalnerve.Byfollow-up,patientswithmeningitishadagoodprognosisandwithoutneurologicalsequelae.Thelesionsof brainstemencephalitiswerebasicallyabsorbedanddisappeared,theprognosiswasgood.Fortheacuteparalysis,theaffectedlimbs recoveredtovaryingdegrees,andthelesionswereabsorbedpartially.Conclusion HFMDpatientsclinicalmanifestationsand MRI characteristicsofconcurrentnervoussystemdiseasewerediversity.Whenthenervoussystemsymptomshappened,weshouldactively performtheMRIexaminationtoidentifytheinflammatoryinvasionarea.Onceinvolvingthebrainstemandspinalcord,highalert, earlyrecognitionandtimelyinterventionarekeytoreducethemorbidityandmortalityofacuteHFMD,toenableHFMDtobeoptimizedand comprehensivelytreated.

Objective: To observe the feasibility and clinical curative effect of anatomical reduction and fixation of lesser trochanter through posterior-lateral approach for the treatment of comminuted femoral intertrochanteric fractures in elderly patients. Methods: A total of 19 cases with comminuted femoral intertrochanteric fracture, aged 60 to 84 years were enrolled in this study.According to Evans-Jensen classification, 13 cases had type Ⅱ B and 6 cases had type Ⅲ femoral fracture.Through the posterior-lateral approach, the lesser trochanter was fully exposed, reduced and fixed by screw.The proximal and distal femur were well fixed with dynamic hip screw(DHS)or proximal femoral locking plate(PFLP). The lesser trochanter reduction was observed by intraoperative vision and postoperative X-ray.The function evaluation after hip surgery was performed by using Sanders hip function score. Results: All 19 patients obtained the anatomic reattachment in the lesser trochanter.All patients were followed up for an average of 18 months.After treatment, the Sanders hip function score were excellent in 7 patients(36.8%), fine in 12 cases(63.2%). And curative efficacy was good in all cases(100%). There was no significant difference in the curative effect between patients with different classification and gender. Conclusions Through the posterior-lateral approach, the surrounding structures of the lesser trochanter can be fully exposed, and the lesser trochanter can be anatomically repositioned.The posterior-lateral approach to anatomical reduction and fixation of lesser trochanter is an effective and feasible method for the treatment of comminuted intertrochanteric fracture in the elderly.