Objective To discuss the value of high frequency ultrasound in assessment the changes of knee,ankle and elbow joints with haemophiliac arthropathy in children.Methods Eighty joints of 42 haemophiliac patients were enrolled in this study.Sixty two joints of 29 patients were examined by X-ray at the same time.The correlation of ultrasonography and X-ray on bone aspects were compared.Results Among 80 joints of 42 haemophilic patients,soft tissue swelling was showed in 12 joints,synovial hypertrophy was in 48 joints,the thickness range of synovial membrane was from 2.01 to 11.12 mm [(4.71± 2.26)mm],increased blood flow was in 40 joints,effusion in 53 joints,haemosiderin was in 33 joints,bone erosion was in 36 joints,subchondral cyst formation was in 20 joints,cartilage damage or disappear was in 31 joints.Ultrasonograthy showed bone erosion in 29 joints,subchondral cyst formation was in 16 joints,cartilage damage was in 24 joints.X-ray showed bone erosion in 23 joints,subchondral cyst was in 16 joints,narrowing joint space was in 20 joints.There was high correlation between the osteochondral domain of the ultrasonography and X ray:Kappa value were 0.803,0.832,0.790 respectively(P ＜0.05).Conclusions Ultrasonography is sensitive to detect the signs of enrolled joints in hemophiliac patients.It is an important method on diagnosis for hemophiliac arthrophv.

Abstract Formaldehyde has been widely employed to immobilize clinical tissue specimens, inactivate toxins and viruses in biomedical fields. Formaldehyde can react with active groups in bio-molecules such as proteins, resulting in protein cross-linking, inactivation, and immobilization. By using several standard peptides and tryptic peptides from matrix protein of influenza virus as experimental models, we studied the chemical modifications of peptides and proteins with formaldehyde by matrix-assisted laser desorption ionization time-of-flight mass spectrometry and nano-electrospray quadruple time-of-flight tandem mass spectrometry. The reaction between formaldehyde and peptides was performed under the same conditions as those during inactivation of virus (4℃, 0. 025% Formalin (V/V), 37% formaldehyde solution (w/w), and 72 h). The results indicated that under above conditions, formaldehyde could react with amino group of N-terminus of standard peptide to generate a methylol adduct, which was further condensed into an imine to generate+12 Da product. Besides, formaldehyde could react with side chain of two amino acids such as arginine and lysine, yielding +12 Da product respectively. The analysis of the reaction between formaldehyde and tryptic peptides from matrix protein of influenza virus showed that +24 Da products could be detected in most peptides due to combinational contribution from N-terminus of peptide (+12 Da ) and side chain of C-terminal arginine or lysine (+12 Da) . Moreover, a +36 Da product was detected for a peptide with miss-cut site. The results indicated that low-concentration formaldehyde primarily reacted with amino group on N-termini of peptides and proteins, as well as the side chains of arginine and lysine residues. The present study suggested an effective mass spectrometry-based method for analyzing the reaction between low-concentration formaldehyde and peptides and proteins, thus provided strategies for interpretation for the mass spectra of reaction products.

Objective To identify the risk factors for postoperative spinal cord injury in Stanford type A aortic dissection patients.Methods 210 Stanford type A aortic dissection(TAAD) patients underwent Sun's procedure in Beijing Aortic Disease Center during July 2014 to March 2015.14 patients had spinal cord injury after surgery.Clinical data and computed tomography angiography(CTA) imaging of aorta were retrospectively analyzed and multi-logistic regression analysis was performed to identify risk factors for spinal cord injury post operation.Results 14 out of 210(6.7％) patients had transient or permanent spinal cord injury after surgery.Univariate analysis showed only false lumen derived intercostal arteries at eighth thoracic vertebral level (T8) to first lumbar vertebral level (L1) was significantly associated with post-surgery spinal cord injury (P =0.000).Multi-logistic regression analysis showed that false lumen derived intercostal arteries (P =0.000) and age (P =0.016) were significantly associated with postoperative spinal cord injury.Conclusion Major intercostal arteries derived from false lumen and rapid thrombogenesis in false lumen are the major risk factors for postoperative spinal cord injury in Stanford type A aortic dissection patients.

Objective:To evaluate facial soft tissue 3-deminsion changes of skeletal Class Ⅲmalocclu-sion patients after orthognathic surgery using structure light scanning technique .Methods:Eight patients [3 males and 5 females, aged ( 27.08 ±4.42 ) years ] with Class Ⅲ dentoskeletal relationship who underwent a bimaxillary orthognathic surgical procedure involving advancement of the maxilla by Le FortⅠosteotomy and mandibular setback by bilateral sagittal split ramus osteotomy (BSSO) and genioplasty to correct deformity were included .3D facial images were obtained by structure light scanner for all the patients 2 weeks preoperatively and 6 months postoperatively .The facial soft tissue changes were evalua-ted in 3-dimension.The linear distances and angulation changes for facial soft tissue landmarks were ana-lyzed.The soft tissue volumetric changes were assessed too .Results: There were significant differences in the sagittal and vertical changes of soft tissue landmarks .The greatest amount of soft tissue change was close to lips.There were more volumetric changes in the chin than in the maxilla , and fewer in the forehead .Conclusion: After biomaxillary surgery , there were significant facial soft tissue differences mainly in the sagittal and vertical dimension for skeletal Class Ⅲ patients .The structure light 3 D scan-ning technique can be accurately used to estimate the soft tissue changes in patients who undergo orthog-nathic surgery .

ObjectiveTo demonstrate the project scope of the afferent nerves of the lumbar intervertebral disc,on which basis to explore the mechanism of the symptoms of discgenic low back pain.MethodsThirty Wistar rats were divided randomly into three groups of 10 rats each:the L4-5,L5-6,and L6 S1 group.Each group was further divided randomly into two subgroups,the experimental group and the control group,5 rats for each group.Intervertebral disc was exposed through the posterior approach under peritoneal cavity anesthesia,after the nerve roots were pull away,2 μl of 30％ cholera toxin-horseradish peroxidase (CT-HRP) was injected into the inner layer of the intervertebral disc in the experimental group,while 2 μl of 0.9％ Nacl was used in the control group.Forty-eight hours after the surgery,all rats were perfused and bilateral dorsal root ganglions(DRGs) of T10-L3 were resected and fixxied.Each DRG was sectioned at 30 μm thickness and processed by DAB method.The sections of DRGs were coverslipped and observed by optical microscopy for the neurons or axons labelled by CT-HRP.It was judged as positive that brownish-black particles were in the neurons or axons.ResultsNot in a single dorsal root ganglions,but in a scope of dorsal root ganglions axons labled by CT-HRP could be seen in the rats in the experimental groups.No CT-HRP labled neurons or axons were seen in dorsal root ganglions in the contral groups.ConclusionAfferent nerves of the lumbar intervertebral disc project to a scope of dorsal root ganglions,which is the anatomic basis of the mechanism of the symptoms of discgenic low back pain.

Objective To discuss the imaging features of skeletal changes in children with Gaucher disease on X-ray and MRI images.Methods One hundred and nine children with Gaucher disease were enrolled in this study.They all received routine X-ray for spine with anterior-posterior(A-P)and lateral view and bilateral femurs with A-P view.Among them.18 patients received X-ray for pelvic with A-P view.14 patients received X-ray for left wrist with A-P view.and 14 patients received MRI scan for femur.The MRI scan included T1-weighted imaging,T2-weighted imaging and fat-suppressed T2-weighted imaging with short tau inversion recovery(STIR)sequence.The imaging features of the X-ray and MRI images were analyzed retrospectively.Results The most common feature is osteoporosis,which presented in 91 cases (83.5%).Besides this,decreased density of metaphysis occurred in 86 cases(78.9%).erlenmeyer flask deformity of metaphysis occurred in 89 patients(81.7%),thinner cortex occurred in 69 cases(63.3%),osteolytic destruction occurred in 31 cases(28.4%).pathological fractures occurred in 26 cases (23.9%),osteosclerosis occurred in 12 cases(11.0%).cystic degeneration of bone occurred in 16 cases (14.7%),and dislocation of the hip occurred in 4 cases.All 14 patients received MRI presented abnormal signals.Among them,4 patients presented low signal intensity both on T1-weighted and T2-weighted images in bone marrow;the other ten presented high signal intensity mixed in low signal intensity areas on T2-weighted and fat-suppressed T2-weighted images.Conclusions The imaging features of skeletal changes in children with Gaucher disease are of some characteristics,which could provide useful information for the clinical treatment.

Objective To analyze the CT and MR imaging characteristics of the subcutaneous and organ lesions of uvenile xanthogranuloma (JXG) in children.Methods The CT and MR images of the head,chest,abdomen and subcutaneous soft tissue of 11 patients (6 females and 5 males,aged 2 months-12 years old,median age 8 months old) with JXG from 2006 to 2018 were collected and retrospectively analyzed.Eight patients underwent CT scan and/or contrast-enhanced CT scan,and 7 patients underwent MRI and/or MRI and contrast-enhancement MRI.Results In 11 cases of JXG,5 cases were classified as simple skin type,involving the skin and deep soft tissue in the right posterior neck,the right frontal and parietal parts of the head,the nose root,and the fat layer and the deep muscle in the extremities.The lesions were the subcutaneous soft tissue nodules or mass showing isodensity on CT or high signal on T2WI.And the other 6 cases were classified as systemic type whose lesions involved one or more than one part except the skin.In the 6 cases,3 cases involved the central nervous system,including the pituitary gland,cerebral hemisphere,cerebellum,brain stem,basal ganglia,thalamus,optic chiasma and spinal cord.The lesions of the pituitary gland were manifested as pituitary enlargement,thickening of the pituitary stalk,and disappearance of high signal on T1WI in the neurohypophysis.The brain parenchyma,the spinal cord and the optic chiasma showed multiple flake or nodular masses,which were iso/low signal on T1WI,low / high signal on T2WI,diffusion restricted,and enhanced markedly.Two cases involved lung tissue,and their lesions showed diffuse or scattered multiple cloudy shadow,reticular opacity,strip and small nodule shadow on chest CT.There was pleural effusion in one case.Two cases involved the livers and 2 cases involved the kidneys,showing multiple nodules with low signal on T2,WI and slight enhancement.One case involving the pancreas showed a low density in the head of the pancreas and slight enhancement on CT.One case involving the greater omentum,chest wall and peritoneum showed thickening of the corresponding tissues on CT.Conclusions Subcutaneous and systemic lesions of JXG show some characteristics on CT and MRI images.The main manifestations are single or multiple nodulars,which are diffusely distributed and vary in size in subcutaneous soft tissue and various organs.The combination of imaging appearances,clinical manifestations and biopsy has important clinical significance for the correct diagnosis and evaluation of JXG.

Objective To explore the significance of the cut point of peak level of luteinizing hormone (LH) in gonadotropin-releasing hormone (GnRH) agonist test in the diagnosis of precocious puberty in obese girls.Methods According to the diagnostic criteria of children's central precocious puberty diagnosis consensus (2015),796 cases of precocious puberty (peak level of LH ≥3.3 IU/L) in Children's Hospital of Zhejiang University School of Medicine from January 2014 to December 2015 were divided into normal weight group (573 cases),overweight group (170 cases) and obesity group (53 cases).The 3 groups were compared in terms of basic level of LH,basic level of follicle stimulating hormone (FSH),peak level of LH and FSH,ratio of LH (peak)/FSH (peak),sex hormone binding protein (SHBG),and children with 3.3 IU/L ≤peak level of LH ＜5.0 IU/L were followed up.The accuracy of the diagnosis of central precocious puberty using peak level of LH was analyzed by using receiver operating characteristic (ROC) curve.Results The median of LH (peak) in the obese group was 6.92 IU/L,the median of SHBG was 46.52 nmol/L,the median of LH (peak) in normal weight group was 8.92 IU/L,and the median of SHBG was 87.28 nmol/L.There were significant differences between the 2 groups (P ＜ 0.05,0.001).A total of 89 cases in normal weight group and 65 cases of obesity/overweight group (3.3 IU/L ≤peak level of LH ＜5.0 IU/L) were followed up for 1 year,and 18 cases in normal weight group and 33 cases in obesity/overweight group developed to central precocious puberty,while the number of cases in the 2 groups was significantly different (P ＜0.001).The ROC curve analysis showed that the cut point of peak level of LH was 4.16 IU/L,the sensitivity was 0.606,the specificity was 0.656,and the Jordan index was the largest (0.344).Conclusions The peak level of LH in GnRH agonist test is important for the diagnosis of central precocious puberty in girls,but the effect of body mass index on its cut point needs to be considered.

A fast,reliable,and cost-effective liquid chromatography-tandem mass spectrometry method was established to determine the effects of the traditional Chinese medicine employed to treat coronavirus disease 2019,namely,Lianhua Qingwen granules,Huoxiang Zhengqi capsules,Jinhua Qinggan granules,Shufeng Jiedu capsules,and Angong Niuhuang pills,on the pharmacokinetics of lopinavir/ritonavir in rats.Blood samples were prepared using the protein precipitation method and atazanavir was selected as the internal standard(IS).Separation was performed on an Agilent ZORBAX eclipse plus C18(2.1 mm x 50 mm,1.8 μm)column using acetonitrile and water containing 0.1％formic acid as the mobile phase for gradient elution.The flow rate was 0.4 mL/min and the injection volume was 2 μL Agilent Jet Stream electrospray ionization was used for mass spectrometry detection under positive ion multiple reaction monitoring mode at a transition of m/z 629.3→447.3 for lopinavir,m/z 721.3→296.1 for rito-navir,and m/z 705.4→168.1 for the IS.The method showed good linearity in the concentration range of 25-2500 ng/mL(r=0.9981)for lopinavir and 5-500 ng/mL(r=0.9984)for ritonavir.The intra-day and inter-day precision and accuracy were both within±15％.Items,such as dilution reliability and residual effect,were also within the acceptable limits.The method was used to determine the effects of five types of traditional Chinese medicines on the pharmacokinetics of lopinavir/ritonavir in rats.The pharmaco-kinetic results showed that the half-life of ritonavir in the groups administered Lianhua Qingwen granules and Huoxiang Zhengqi capsules combined with lopinavir/ritonavir was prolonged by approx-imately 1.5-to 2-fold relative to that in the control group.Similarly,the pharmacokinetic parameters of lopinavir were altered.Overall,the results of this study offer important theoretical parameters for the effective clinical use of five types of traditional Chinese medicines combined with lopinavir/ritonavir to reduce the occurrence of clinical adverse reactions.

Biliary fibrosis (BF) is the result of pathological repair of bile tract injury, characterized by thickening and sclerosis of the bile duct wall and progressive stricture of the lumen, which may ultimately lead to serious adverse outcomes such as biliary obstruction, biliary cirrhosis, liver failure, and hepatobiliary malignancies. Current research describes BF as a pathological feature of certain bile tract diseases, lacking a systematic summary of its etiology, pathophysiology, molecular mechanisms, and treatment. BF is a common but easily neglected disease state in biliary system, which may promote the development and progression of hepatobiliary diseases through abnormal repair mechanism after pathological biliary tract injury. Based on the latest research progress from both domestic and international perspectives, the authors review the concept, clinical manifestation, etiology, pathogenesis, and therapeutic strategies of BF to provide a reference for clinical physicians.