Objective To explore the imaging manifestations of Rosai-Dorfman disease(RDD)in children.Methods A total of 12 children with RDD confirmed by pathology were retrospectively enrolled,including 8 cases underwent non-contrast CT(NCCT)+contrast enhanced CT(CECT),2 cases underwent NCCT+CECT+non-contrast MR(NCMR)+contrast enhanced MR(CEMR),1 case underwent NCCT+NCMR and CEMR,while 1 case underwent NCCT and X-ray examinations.Imaging manifestations of RDD in children were observed.Results Among 12 cases,intranodal type RDD was found in 7 case,extranodal type RDD in 3 cases,and mixed type(with both lymph nodes and extranodal sites affected)RDD were noticed in 2 cases.In 7 cases of intranodal type RDD,NCCT and CECT showed multiple lymph node enlargements in both sides of the neck,with uniform isodensity on NCCT and mild-moderate progressive enhancement in 5 cases,while with low-density necrotic area and ring-shaped enhancement in 2 cases.Among 3 cases of extranodal RDD,the lesion in 1 case involved nasal cavity and posterior group of ethmoid sinuses on CT and MRI,which developed circular soft tissue mass centered on nasal septum with moderate heterogeneous enhancement,also compressed the adjacent bone with destruction.In another case of extranodal RDD,CT showed that the lesion involved left ilium and bilateral parietal bones,with bone destruction accompanied by obvious periosteal reaction and soft tissue mass,which was mildly enhanced.In the rest 1 case of extranodal RDD,CT and X-ray film showed that the lesion involved the upper segment of right femur and left parietal bone,with osteolytic destruction accompanied by layered periosteal reaction.The lesions in both 2 cases of mixed type RDD involved brain(1 case involved left parieto-occipital lobe,1 case involved bilateral temporal lobes,left frontal lobe and bilateral occipital lobes),presented as isodensity on CT and equal or slightly low signal intensity on T 1WI,equal-high mixed signal intensity on T2WI,some shaped like brain gyral with mild edema of surrounding tissue,and nodular or mass-like significant enhancement.RDD involvements of bilateral lung,mediastinum and hilar lymph nodes were also observed in the above 2 cases,chest CT showed multiple nodular or small patchy uniform high-density shadows in bilateral lungs,as well as enlarged mediastinum and hilar lymph nodes.Conclusion Imaging manifestations of pediatric RDD had certain specificity,being helpful to diagnosis.

Objective To explore the imaging manifestations of Rosai-Dorfman disease(RDD)in children.Methods A total of 12 children with RDD confirmed by pathology were retrospectively enrolled,including 8 cases underwent non-contrast CT(NCCT)+contrast enhanced CT(CECT),2 cases underwent NCCT+CECT+non-contrast MR(NCMR)+contrast enhanced MR(CEMR),1 case underwent NCCT+NCMR and CEMR,while 1 case underwent NCCT and X-ray examinations.Imaging manifestations of RDD in children were observed.Results Among 12 cases,intranodal type RDD was found in 7 case,extranodal type RDD in 3 cases,and mixed type(with both lymph nodes and extranodal sites affected)RDD were noticed in 2 cases.In 7 cases of intranodal type RDD,NCCT and CECT showed multiple lymph node enlargements in both sides of the neck,with uniform isodensity on NCCT and mild-moderate progressive enhancement in 5 cases,while with low-density necrotic area and ring-shaped enhancement in 2 cases.Among 3 cases of extranodal RDD,the lesion in 1 case involved nasal cavity and posterior group of ethmoid sinuses on CT and MRI,which developed circular soft tissue mass centered on nasal septum with moderate heterogeneous enhancement,also compressed the adjacent bone with destruction.In another case of extranodal RDD,CT showed that the lesion involved left ilium and bilateral parietal bones,with bone destruction accompanied by obvious periosteal reaction and soft tissue mass,which was mildly enhanced.In the rest 1 case of extranodal RDD,CT and X-ray film showed that the lesion involved the upper segment of right femur and left parietal bone,with osteolytic destruction accompanied by layered periosteal reaction.The lesions in both 2 cases of mixed type RDD involved brain(1 case involved left parieto-occipital lobe,1 case involved bilateral temporal lobes,left frontal lobe and bilateral occipital lobes),presented as isodensity on CT and equal or slightly low signal intensity on T 1WI,equal-high mixed signal intensity on T2WI,some shaped like brain gyral with mild edema of surrounding tissue,and nodular or mass-like significant enhancement.RDD involvements of bilateral lung,mediastinum and hilar lymph nodes were also observed in the above 2 cases,chest CT showed multiple nodular or small patchy uniform high-density shadows in bilateral lungs,as well as enlarged mediastinum and hilar lymph nodes.Conclusion Imaging manifestations of pediatric RDD had certain specificity,being helpful to diagnosis.

Objective By investigating the retention and fatigue performance of polyetheretherketone(PEEK)clasps with increased proportions of clasp arm engaging the undercut,an innovative method to improve PEEK clasps'performance was proposed and verified.Methods Three groups of PEEK clasps(n=10/group)with the terminal 1/3,2/3 and whole of their retentive arms engaging the 0.75 mm undercut were designed and fabricated respectively along with a group of cobalt-chrome clasps(n=10)with their terminal 1/3 of retentive arms engaging the 0.25 mm undercut.Retentive forces were recorded initially and every 1 500 cycles for a total of 15 000 in-sertion and removal fatigue cycles.Optical scanning and software analysis were applied to evaluate the deformation of the clasps by root mean square(RMS)after fatigue test.The inner surface morphology of clasps'arms was observed by scanning electron microscopy.Re-sults PEEK clasps with the whole of their retentive arms engaging the undercut exhibited highest mean retentive force(9.24±1.78)Namong other PEKK clasps,which was slightly lower than that of cobalt-chrome clasps(11.88±2.05)N.The retentive force of each group turned out to be reduced after fatigue cycles and cobalt-chrome clasps showed the greatest reduction(38.38%).The RMS of the PEEK clasps groups were of no statistical difference(P=0.111)and were lower than that of cobalt-chrome clasps(105.47±10.82)μm.Evidence of surface abrasion was observed on all groups of clasps especially on the section that engaged the undercut.Conclusion Increasing the proportion of PEEK clasp arm engaging the undercut effectively improved the retentive force and satisfied clinical re-quirements after fatigue cycles.PEEK clasps exhibited greater retention stability than cobalt-chrome clasps.It is feasible to improve the performance of PEEK clasps by increasing the proportion of clasp arm engaging the undercut.

Objective:To explore the diagnostic value of CT pulmonary vascular quantitative parameters in patients with chronic obstructive pulmonary disease (COPD) and high-risk groups.Methods:A retrospective study of 1 126 patients who underwent chest CT examination and pulmonary function test in Shanghai Tongji Hospital from January 2015 to August 2020. According to lung function, they were divided into COPD group (471 cases), high-risk group (454 cases), and normal control group (201 cases). Pulmonary vascular parameters on chest CT, including the total number of vessels (N total), the number cross-sectional area of vessels under 5 mm 2 (N CSA<5), lung surface area (LSA), number of pulmonary blood vessels per unit lung surface area (N total/LSA) and the total area of vessels (VA total) at a 9, 15, 21 mm depth from the pleural surface, and the total blood vessel volume (TBV), blood vessel volume under 5 mm 2 and 10 mm 2(BV5 and BV10) were measured quantitatively. Kruskal-Wallis H test was used to compare the differences of quantitative parameters of pulmonary vascular in the three groups; Spearman rank test was used to analyze the correlation between CT pulmonary vascular parameters and pulmonary function. Results:There were significant differences in N total/LSA at a 9, 15, 21 mm depth from the pleural surface among three groups ( P<0.05). There were significant differences in N CSA<5, N total at a 9 mm depth from the pleural surface among three groups ( P<0.05). There were significant differences in LSA at a 9 mm depth from the pleural surface, N CSA<5, N total, LSA, VA total at a 15, 21 mm depth from the pleural surface and TBV, BV5 and BV10 among three groups ( P<0.05). In high-risk group, there were positive correlation between N total/LSA, VA total at a 9 mm depth from the pleural surface and some pulmonary function parameters ( r=0.095-0.139, P<0.05). N CSA<5, N total, LSA, N total/LSA, TBV, BV5 and BV10 at different depth from pleural surface were negatively correlated with some pulmonary function parameters ( r=-0.110--0.215, P<0.05). In COPD group, number of vessels at a 9 mm depth from the pleural surface was positively correlated with the diffusion capacity for carbon monoxide of the lung single breath ( r=0.105, 0.103, P<0.05). In addition to N total/LSA were positively correlated with lung function parameters ( r=0.181-0.324, P<0.05), the remaining pulmonary vascular parameters were negatively correlated with some pulmonary function parameters ( r=-0.092--0.431, P<0.05). Conclusion:Quantitative chest CT imaging are able to effectively evaluate pulmonary vascular changes in COPD patients and high-risk groups, and the quantitative parameters of pulmonary vascular CT may distinguish COPD from high-risk groups, providing a novel means for early diagnosis of COPD and prediction of high-risk groups.

Recent insights collectively suggest the important roles of lysyl oxidase (LysOX) in the pathological processes of several acute and chronic neurological diseases, but the molecular regulatory mechanisms remain elusive. Herein, we explore the regulatory role of LysOX in the seizure-induced ferroptotic cell death of neurons. Mechanistically, LysOX promotes ferroptosis-associated lipid peroxidation in neurons via activating extracellular regulated protein kinase (ERK)-dependent 5-lipoxygenase (Alox5) signaling. In addition, overexpression of LysOX via adeno-associated viral vector (AAV)-based gene transfer enhances ferroptosis sensitivity and aggravates seizure-induced hippocampal damage. Our studies show that pharmacological inhibition of LysOX with β-aminopropionitrile (BAPN) significantly blocks seizure-induced ferroptosis and thereby alleviates neuronal damage, while the BAPN-associated cardiotoxicity and neurotoxicity could further be reduced through encapsulation with bioresponsive amorphous calcium carbonate-based nanocarriers. These findings unveil a previously unrecognized LysOX-ERK-Alox5 pathway for ferroptosis regulation during seizure-induced neuronal damage. Suppressing this pathway may yield therapeutic implications for restoring seizure-induced neuronal injury.

Objective:To study the clinical features of children with Prader-Willi syndrome(PWS).Methods:Eighteen cases of PWS were collected from July 2016 to November 2018 in Shenzhen Maternal and Child Healthcare Hospital, Southern Medical University.The clinical data of children with PWS were analyzed retrospectively.Results:There were 12 males and 6 females in 18 cases with PWS.The diagnosis age ranged from 25 days to 9.5 years old [(3.09±3.02) years old]. Among them, 11 cases were in infancy (≤3 years old) and 7 cases after infancy (>3 years old). The main clinical features of infants with PWS were 11 cases of gonadal dysplasia (100.0%), 11 cases of psychomotor retardation (100.0%), 10 cases of hypotonia (90.1%), 6 cases of feeding difficulty and weak cry (54.5%). After infancy the main clinical features included 7 cases of psychomotor retardation (100.0%), 5 cases of hyperphagia(71.4%), 5 cases of obesity (71.4%), 5 cases of abnormal behavior problems (71.4%) and 4 cases of visual problems (57.1%). The clinical features of all patients throughout the developmental stage were as follows: decreased fetal movement, hypoplasia, neonatal hypotonia, weak cry, feeding difficulty, psychomotor delay, hyperphagia, obesity, abnormal behavior problems, and so on.Conclusions:The clinical features of PWS vary with age.The main clinical features in the infancy are hypotonia, weak cry, difficulty feeding and gonadal dysplasia.After infancy, there are hyperphagia, obesity, behavior and visual problems.And psychomotor retardation is present in the whole developmental stage of children with PWS.Early diagnosis and treatment are important for improving the prognosis of PWS.

Objective:To understand the clinical manifestations, diagnosis, treatment, and prognosis of children with hereditary thrombocytopenia (HT).Methods:The clinical data of 5 patients with HT in the Hematology and Oncology Department of Children′s Hospital of Chongqing Medical University from August 2015 to October 2017 were retrospectively analyzed. The clinical and laboratory characteristics, treatment, and prognosis of HT were discussed by reviewing relevant literatures.Results:Five patients included 3 boys and 2 girls.The median age at onset of 4 years and 2 months old and the median age at diagnose was 4 years and 4 months old.All patients presented with the thrombocytopenia, among which 4 cases were macrothrombocytopenia and 1 case was normothrombocytopenia.The main clinical presentations of 5 patients were skin petechiae and ecchymoses.Four cases were initially misdiagnosed as immune thrombocytopenia (ITP) and received the glucocorticoid and immunoglobulin, while the therapeutic effect was not satisfactory.The gene sequencing confirmed MYH9 gene mutation(c.3493C>T), MYH9 gene mutation(c.5878G>A), NBEAL2 gene compound heterozygous mutation(c.295C>T; c.4169C>T), GP1BA gene mutation(c.1761A>C), and ANKRD26 gene mutation(c.5123A>G), in 5 patients respectively. Conclusions:HT should be suspected among those with recurrent isolated thrombocytopenia and no response to the ITP regimen, and the early gene screening is of great significance to the patients′ treatment and prognosis.

Objective: To investigate the clinical, immunological, and molecular manifestations of nuclear factor kappa-B subunit 2 (NFκB2) gene mutation associated common variable immunodeficiency (CVID) . Methods: A 14-month-old boy diagnosed with NFκB2-mutated CVID was admitted into Children's Hospital of Chongqing Medical University in December 2015. The clinical manifestations, biochemical tests, immunological function, molecular features, treatment, and follow-up of the patient were analyzed. The Chinese and PUBMED databases were searched with the key words "NFκB2" and "immune deficiency" and related literatures were reviewed. Results: The patient had 4 episodes of pneumonias and one otitis media since the age of 6 months. The serum immunoglobulin levels were IgG 2.73 g/L, IgA<0.07 g/L, and IgM 0.12 g/L. The percentage of peripheral lymphocyte subsets demonstrated increased CD3⁺T lymphocyte (81.8%), increased CD4⁺ naïve T cell (39.1%), normal B cell (14.1%), low switched memory B and plasmablast B (respectively 0.1% and 0), and lightly diminished natural killer(NK) cell (4.13%). Within the peripheral CD4⁺T cells, the percentage of regulatory T cells (1.49% (control 4.08%)), T follicular helper (3.66% (control 11.0%)), and T helper 17 (9.65% (control 15.7%)) were decreased, while the percentage of T helper 2 (60.9% (control 46.5%)) was elevated. T lymphocyte proliferative response and T cell receptor repertoire diversity were normal. NK-cell cytotoxic activity was impaired. The whole-exome sequencing harbored a de novo heterozygous nonsense mutation in exon 22 (c.2557C>T; p. Arg853X) in the C-terminus of NF-κB2. The western blotting confirmed the decreased expression of NF-κB2 (p52) protein. The patient received intravenous immunoglobulin infusion monthly (400-600 mg/kg), followed by improvement of pulmonary infection. After searching the databases, a total of 28 cases (1 Chinese and 27 non-Chinese) were identified. There were 12 cases of nonsense mutation (5 were gain-of-function mutation), and 8 cases of missense and frameshift mutations, respectively. The main clinical manifestation was respiratory infection, followed by autoimmune diseases such as alopecia and trachyonychia. Fifteen cases developed adrenocorticotrophic hormone (ACTH) deficiency. Conclusions NF-κB2 signaling pathway played an important role in T and B lymphocyte differentiation, and NK-cell cytotoxic activity. NFκB2 mutation should be considered in cases with recurrent infections, hypogammaglobulinemia, and decreased memory B cells and plasma cells, especially when combined with ACTH deficiency.

Objective To evaluate the effects of CHOICE management mode on adherence of adolescents with type 1 diabetes mellitus (T1DM). Methods A total of 49 adolescents with T1DM were elected using convenience sampling method, and divided into the experimental group (26 cases) and the control group (23 cases) by random number table. The experimental group received the CHOICE management for 1 year, while the control group received traditional education management. The patients′self-management behaviors and HbA1c were evaluated before the intervention and 1, 3, 6 and 12 months after the intervention. Results The scores of Control Problems, Attitude Toward Diabetes and Understanding Management Knowledge after 12-months intervention were (2.71 ± 0.43), (3.89 ± 0.60), (3.05 ± 0.71)points in the experimental group, (2.49 ± 0.33),(4.20 ± 0.40),(3.51 ± 0.70)points in the control group, there were significant differences (t=-2.101, 2.129, 2.289, P<0.05). The scores of Diet Adherence after 1 month and 3 months intervention were (2.85 ± 0.0.67), (2.79 ± 0.66)points in the experimental group, (2.39 ± 0.70), (2.38 ± 0.67)points in the control group, there were significant differences (t=2.170, 2.338, P<0.05). HbA1c was(8.29 ± 0.76)％ in the experimental group, (8.85 ± 1.14)％ in the control group, there was significant difference (t=-2.041, P<0.05). There were no significant differences at the others times between two groups (t=- 1.556-- 0.527, P>0.05). Conclusions CHOICE management mode can effectively improve the self-management behavior of adolescents with T1DM.

AIM:To explore the effects of stanniocalcin 2 (STC2) on the proliferation, migration and the process of epithelial-mesenchymal transition (EMT) in human hepatocellular carcinoma HepG2 cells.METHODS:The expression levels of STC2 in the hepatocellular carcinoma cell lines and normal liver cells were assessed by Western blot.Colony formation assay was used to test the effect of STC2 on the proliferation of HepG2 cells.The effects of STC2 on the expression of proliferation-related molecules at mRNA and protein levels were determined by RT-qPCR and Western blot.The effect of STC2 on the migration ability was measured by Transwell assay.The mRNA and protein levels of vimentin and E-cadherin in STC2-overexpressing and-silencing cell lines were detected by RT-qPCR and Western blot.RESULTS:Compared with the normal liver cell line, the protein expression of STC2 was up-regulated in the hepatocellular carcinoma cell lines.The results of colony formation assay indicated that STC2 promoted the proliferation of HepG2 cells.STC2 significantly regulated the proliferation-related gene expression, such as cyclin D1.The results of Transwell assay showed that STC2 enhanced the migration ability of the HepG2 cells and influenced the EMT process.CONCLUSION:STC2 promotes the proliferation of HepG2 cells and affects the expression of proliferation-related genes.STC2 influences the process of EMT and promotes the migration of HepG2 cells.