Surgical treatment and outcome of primary tracheobronchial tumors in 15 children / 中华胸心血管外科杂志

Objective:To investigate the clinical manifestation, pathological types, treatment and prognosis of primary tracheobronchial tumors in children.Methods:We retrospectively studied the primary tracheobronchial tumors patients who diagnosed from May 2009 to Jan 2021 in Guangzhou Women and Children Medical Center. The clinical manifestations, pathological types, therapeutic methods and prognosis were analyzed.Results:There were 15 patients identified as the primary tracheobronchial tumors, including synovial sarcoma (1 case), pulmonary inflammatory myofibroblastic tumor(IMT 4 cases), mucoepidermoid carcinoma(7 cases), infantile hemangioma (1 case), Ewing's sarcoma (1 case). Respiratory symptoms are the most complaint at the time of diagnosis including 15 patients with cough, 2 with hemoptysis, and 1 with dyspnea. Endoscopic treatment of tracheobronchial tumors was performed under extracorporeal membrane oxygenation (ECMO) support in 1 patient. Sleeve lobectomy was performed in 3 patients, lobectomies in 6, and local tumor resections in 4 patients including 2 patients suffered second surgery due to tumor recurrence.Conclusion:The clinical manifestations of the primary tracheobronchial tumors in children are nonspecific. Complete resection led to excellent outcome.

Paying attention to the natural course of disease for a development of gene therapy of inherited retinal diseases / 中华实验眼科杂志

Most inherited retinal diseases (IRDs) severely impair vision and lack effective treatments.With the approval of Luxturna, the world's first gene therapy drug for IRDs in 2017 by the U. S.FDA, gene therapy has brought new hope for the treatment of the disease.With an early onset and a relatively small number of patients, the understanding of the natural course of IRDs is limited in the past.The research on gene therapy of IRDs is mainly based on the in-depth understanding of the pathogenesis and natural course of disease, and the selection of the optimal treatment window for the implementation of gene therapy is the premise of successful treatment.At the same time, the main vector for gene therapy is recombinant virus vector, and its tissue-immunogenicity, tumorigenicity, safety of its integration with host cells and effectiveness determine the outcome of therapy, so the evaluation technology of IRDs gene therapy needs to be established.Gene therapy for ophthalmic diseases also involves the consideration of laws and regulations, ethics, product process, races and regional environment, disease progression, gene mutation types, patient benefit and risk ratio, and other factors.Therefore, it is of great significance to take full account of the differences in IRDs population, especially the particularity of children patients, and actively carry out the study on the natural course of IRDs in China for the scientific and normative development of clinical trials of gene therapy, the effective establishment of endpoint and outcome indicators for clinical studies of gene therapy, and the compliance with international norms of ethics.

Developmental relationship between mesenchymal stem/progenitor cells and hematopoietic cells during human embryogenesis / 中华微生物学和免疫学杂志

Objective@#To analyze the developmental relationship between mesenchymal stem/progenitor cells (MSPCs) and hematopoietic cells during human embryogenesis.@*Methods@#Aborted embryos at different developmental stages were used in this study after medical abortion. Embryonic blood tissues were isolated and digested into single cells. These single cells were plated in semisolid medium in favor of the differentiation of colony-forming cell with high proliferative potential (HPP-CFC) and incubated for 10 to 14 d. Individual colonies with diameter more than 0.5 mm were picked and replated in liquid medium. Fibroblastic adherent cells appeared in the replated colonies were cultured for cell proliferation and cytokins expressed on cell surface were identified to analyze whether they had the characteristics of MSPCs.@*Results@#This study summarized the dynamic development of HPP-CFCs and other hematopoietic progenitor cells in different tissues including aorta-gonad-mesonephros (AGM) region, yolk sac and embryonic liver. From the 28-somite stage, a proportion of HPP-CFCs in AGM region could give rise to adherent fibroblastic cells in addition to hematopoietic cells. The adherent cells harbored the differentiation potential of MSPCs and could inhibit the proliferation of T cells in lymphocyte transformation test.@*Conclusions@#This study suggests some prehematopoietic precursors in AGM region can give rise to both hematopoietic progenitors and MSPCs during human embryogenesis.

Developmental relationship between mesenchymal stem/progenitor cells and hematopoietic cells dur-ing human embryogenesis / 中华微生物学和免疫学杂志

Objective To analyze the developmental relationship between mesenchymal stem/pro-genitor cells (MSPCs) and hematopoietic cells during human embryogenesis. Methods Aborted embryos at different developmental stages were used in this study after medical abortion. Embryonic blood tissues were isolated and digested into single cells. These single cells were plated in semisolid medium in favor of the dif-ferentiation of colony-forming cell with high proliferative potential ( HPP-CFC) and incubated for 10 to 14 d. Individual colonies with diameter more than 0. 5 mm were picked and replated in liquid medium. Fibroblastic adherent cells appeared in the replated colonies were cultured for cell proliferation and cytokins expressed on cell surface were identified to analyze whether they had the characteristics of MSPCs. Results This study summarized the dynamic development of HPP-CFCs and other hematopoietic progenitor cells in different tis-sues including aorta-gonad-mesonephros ( AGM) region, yolk sac and embryonic liver. From the 28-somite stage, a proportion of HPP-CFCs in AGM region could give rise to adherent fibroblastic cells in addition to hematopoietic cells. The adherent cells harbored the differentiation potential of MSPCs and could inhibit the proliferation of T cells in lymphocyte transformation test. Conclusions This study suggests some prehemato-poietic precursors in AGM region can give rise to both hematopoietic progenitors and MSPCs during human embryogenesis.

The association between plasma levels of 20 amino acids and risk of diabetes / 中华内科杂志

Objective To investigate the association between the plasma levels of 20 amino acids and the risk of diabetes in middle-aged and elderly population.Methods This study was a part of the Chinese multi-provincial cohort study conducted in communities of Shougang.In 2007 and 2012,the population was investigated for diabetes and other risk factors.Blood samples collected from 475 people were tested for various amino acid levels by liquid chromatography-tandem mass spectrometry.A multivariate logistic regression analysis was used to analyze the association between plasma amino acid levels and diabetes risk.Results The age of the selected population at baseline was (58.7±6.3) years,and the blood glucose level at baseline was (5.68 ± 1.34) mmol/L.Among them,56 (11.79％) subjects were diabetes.Multivariate logistic regression analyses showed that after adjusting for age,gender,body mass index,systolic blood pressure and dyslipidemia,individuals with plasma branched-chain amino acid (valine,leucine and isoleucine) and cysteine in the highest tertile levels were at high risk of diabetes with the ORs of 3.61 (95％ CI 1.48-8.80),3.27 (95％ CI 1.34-7.99),2.46 (95％ CI 1.04-5.84) and 2.09 (95％ CI 1.02-4.27),respectively.After 5 years' followed up,5.73％ (24/419) subjects developed diabetes.Compared with those in the lowest tertile,individuals with plasma branched-chain amino acid (total concentration),phenylalanine,and tyrosine levels at baseline in the highest tertile had 3.69 times,3.61 times and 4.14 times higher risk to develop new diabetes,respectively.In contrast,individuals with plasma glycine level in the highest tertile had only 76％ (OR 0.24,95％ CI 0.06-0.91) risk for the development of diabetes compared with those with plasma glycine level in the lowest tertile.Conclusions The increase in plasma branched-chain amino acid and cysteine levels is significantly associated with an increase in incident diabetes.Subjects with higher levels of branched-chain amino acids and aromatic amino acids (phenylalanine,tyrosine) had a significantly higher risk of developing new-onset diabetes,while those with higher glycine levels had a significantly lower risk of developing diabetes in 5 years.

The research status and the limitations of mesenchymal stem cell-based therapies in age-related macular degeneration / 中华实验眼科杂志

Age-related macular degeneration (AMD) is the major cause of blindness in the elderly, which mainly affects the retinal pigment epithelial (RPE) cells and photoreceptors, and progressively leads to the loss of central vision.AMD can be divided into dry AMD and wet AMD.At present, the treatment of AMD mainly aims at choroidal neovascularization (CNV) associated with wet AMD.However, there is no effective treatment for dry AMD.The common treatment methods include photodynamic therapy (PDT) and intravitreal injection of anti-vascular endothelial growth factor (VEGF). Furthermore, there are also laser therapy, radiotherapy and surgical treatment.However, at this stage, the clinical treatment methods can not eradicate the causes, and some patients show poor response to treatments.So people began to explore new treatments.Mesenchymal stem cells (MSCs) therapy has the characteristics of self-renewal and multidirectional differentiation potential, lower immunogenicity, easier access to materials and less ethical constraints, showing a broad therapeutic prospect.At present, many studies on the treatment of AMD with MSCs have been carried out at home and abroad, with fruitful results.This article elaborated the classification and treatment status of AMD and their limitations and summarized the research results of MSCs in the treatment of AMD at home and abroad in recent years.

Screening and influencing factors analysis for nutritional status in hospitalized children with pectus excavatum / 中华实用儿科临床杂志

Objective@#To screen the hospitalized children with pectus excavatum for nutritional status, and to analyze the influencing factors of nutritional status, then to provide the scientific basis for comprehensive management of clinical nutrition.@*Methods@#The body mass index (BMI) of 360 hospitalized children with pectus excavatum in Guangzhou Women and Children′s Medical Center from January 2013 to December 2017 were reviewed and compared with World Health Organization standard and the emaciation rate was calculated.The influencing factors such as CT index, age, weight, height, sex, the width of thorax and surgical treatment were analyzed with BMI.@*Results@#Compared with the lowest standard of BMI, the whole emaciation rate of hospitalized children with pectus excavatum was 93.61% (337/360 cases): in which severe, moderate and slight emaciation were 74.17% (267/360 cases), 13.33% (48/360 cases), and 6.11% (22/360 cases), respectively.BMI was negatively related with CT index (r=-0.172, P=0.001), but positively related with age, weight, height, and the width of thorax (r=0.129, 0.342, 0.022, 0.179, P=0.014, 0.001, 0.673, 0.001). Three years after Nuss procedure, both the whole and severe emaciation rate decreased (93.62% vs. 95.74%; 65.96% vs. 78.72%), but the weight, height and BMI increased [25.5(12.5, 38.5) kg vs.17.0(10.0, 24.0) kg; 126.0(99.0, 153.0) cm vs.108.0(74.5, 143.5) cm; 14.69(12.38, 17.00) kg/m2 vs. 14.36(12.17, 16.56) kg/m2], and the differences were significant (all P<0.01).@*Conclusions@#There is a high nutritional risk in hospitalized children with pectus excavatum, and the deformity severity is the main risk factor.Surgical treatment can improve the nutritional status effectively, but the malnutrition in pectus excavatum can not be corrected completely.

Advances in vitreous proteomics of vitreoretinal diseases / 中华实验眼科杂志

Vitreous is a gelatinous, transparent, highly hydrated matrix that fills the posterior segment of eye.A diversity of proteins that can influence retinal physiology are present in vitreous.Moreover, the protein composition of vitreous alters in a number of vitreoretinal disease states.Therefore, analysis of protein composition in vitreous is an effective way to understand the pathophysiological mechanisms of vitreoretinal diseases and to discover biomarkers or treatment targets.Various proteomic methods have been gradually applied to the study of vitreous proteomics.Attempts aiming to establish a map of vitreous proteins for vitreoretinal diseases, such as diabetic retinopathy, epiretinal membranes, rhegmatogenous retinal detachment were performed over the last decade, some potential biomarkers have been discovered.This article reviewed the approaches of proteomics and advances in the study of vitreous proteomics of vitreoretinal diseases.

Study on Chemical Constituents of n-Butanol Part from the Roots of Camellia oleifera / 中国药房

OBJECTIVE： To study the chemical constituents of n-Butanol part from the roots of Camellia oleifera， and to provide reference for elucidating the pharmacodynamic substance basis of it’s anti-tumor effect. METHODS： The ethanol extracts were obtained by using 95％ ethanol as extraction solvent to extract the roots of C. oleifera， and the n-Butanol part was obatined after the extracts were extratced with ethyl acetate and water-saturated n-butanol solution in turn. The chemical constituents of n-butanol part were isolated and purified by D101 macroporous resin column， silica gel， atmospheric pressure reversed phase column chromatography， sephadex gel SephadexLH-20 column chromatogram and preparative HPLC. The structure of the compounds was identified by spectroscopic analysis of physicochemical constants， electrospray ionization mass spectrometry， 1H-NMR and 13C-NMR. RESULTS： Eight compounds were isolated from the roots of C. oleifera and elucidated as quercetin 3′ -O-β-D-glucoside （compound 1）， apigenin-7-O-β-D-glucoside （compound 2）， （+）-lyoniresinol-3α-O-β-D-glucopyranoside （compound 3）， rubusoside （compound 4）， dulcoside B （compound 5）， 4-hydroxy-3-methoxyphenol 1-O-β-D-[6-O-（4-hydroxy-3，5-dimethoxylbenzoate）-glucopyranoside （compound 6）， 3，4，5-trimethoxyphenyl-6-O- syringoyl-β-D-glucopyranoside （compound 7）， gordonoside P （compound 8）.  CONCLUSIONS： Compounds 1-8 were isolated from this plant for the first time. This not only enriches the chemical constituents of this genus， but also provides a reference for elucidating the anti-tumor bioactive substances in this part.

Application of gastric tube esophagoplasty to complicated diseases of esophagus in children / 中华胃肠外科杂志

<p><b>OBJECTIVE</b>To summarize the experience of applying gastric tube esophagoplasty for complicated diseases of esophagus in children and the short-middle-term efficacy.</p><p><b>METHODS</b>A retrospective and observational case series study was performed.</p><p><b>INCLUSION CRITERIA</b>(1) burn length of esophagus > 2 cm, multisegmental or extensive esophageal scar stenosis, and about 6 months after burn; (2) longitudinal diameter of esophageal tumor > 2 cm, or esophagus considered as impossible to reserve;(3) Severe esophageal fistula with diameter > 2 cm, or relapse again after ≥3 times of repair; (4) Tracheal cartilaginous esophageal heterotopia with a length of >2 cm or no end to end anastomosis after removal of the esophageal lesion.</p><p><b>EXCLUSION CRITERIA</b>patients with severe cardiopulmonary insufficiency, or poor prognosis of gastric primary disease; the gastric volume did not allow long enough gastric tube; the parents did not accept the surgery. According to above criteria, 36 children with complicated diseases of esophagus who underwent gastric tube esophagoplasty at Department of Thoracic Surgery, Guangzhou Women and Children's Medical Center from March 2010 to June 2017 were enrolled into this study. Among 36 children, 27 were with corrosive strictures of esophagus, 5 with esophageal tumor, 3 with severe esophageal fistula, and 1 with tracheal cartilaginous esophageal heterotopia. Above-mentioned 27 cases with corrosive strictures of esophagus underwent gastric tube esophagoplasty via retrosternal route with preservation of the original esophagus. The other 9 cases underwent resection for esophageal lesion and gastric tube esophagoplasty via prevertebral route. The construction of gastric tube was as follows: the stomach was cut along the lesser curvature from pylorus to cardia and fundus of stomach with stapler, making the diameter of the gastric tube equal to pylorus. Operative time, intra-operative bleeding, time of mechanical ventilation, anastomotic leakage, anastomotic stricture were observed. The postoperative short-middle-term growth presentation of children was evaluated according to CDC 2000 children growth evaluation table(2 to 20 years).</p><p><b>RESULTS</b>All the 36 children survived their operations successfully. Nine cases underwent esophagectomy for lesion esophagus and the other 27 cases received preservation of original esophagus. Average time of postoperative mechanical ventilation was 8 (4-20) hours. Three cases developed anastomotic leakage and were healed after one week. Eight cases developed anastomotic stricture and resumed normal diet after balloon expansion. The patients were followed up from 6 months to 7 years. Five cases were found to have esophageal cyst 4-8 months after the operation, and received resection. One children with infantile esophageal fibrosarcoma recurred 3 weeks after the operation and died 2 weeks later because the family abandoned the treatment. The quality of life of 35 cases was improved significantly. Short-middle-term body height and weight in 85.7%(30/35) children met basically the criteria of CDC 2000 children growth evaluation table.</p><p><b>CONCLUSION</b>Gastric tube esophagoplasty can effectively treat the children with complicated esophagus diseases with good short-middle-term efficacy, and is a recommended esophageal replacement surgery.</p>

Pathologic features on gonadal changes of sexual developmental disorders in children / 中华病理学杂志

Objective@#To investigate the pathologic features of gonadal tissues of disorders of sexual development (DSD) in children.@*Methods@#Fifty-three cases of gonadal developmental disorders were collected from July 2015 to August 2017 at Guangzhou Women and Children′s Medical Center. Clinical manifestations, karyotypes, sex hormone levels, ultrasound imaging, histology and immunophenotype of gonadal tissues were analyzed.@*Results@#The age of patients ranged from 7 months to 17 years with an average of (50.7 ± 47.1) months. Social genders of the patients included 32 males and 21 females. Forty-eight patients had abnormal sex hormone levels. Clinical presentations included: toward female genitalia in 25 cases, male genitalia tendency in 17 cases and ambiguous external genitalia in 11 cases. Hypospadias was seen in 31 cases and short stature was seen in 8 cases. Chromosomal karyotyping of peripheral blood revealed 23 cases of sex chromosome disorders, 22 cases of 46 XY disorders, of which 3 cases were 5α-reductase deficiency and 8 cases of 46 XX disorders. Ultrasound examination showed cryptorchidism in 30 cases, including 16 cases of unilateral, 14 cases of bilateral and 1 case presenting a huge pelvic tumor. A total of 97 gonadal tissues from 53 cases of DSD were examined, including 9 cases of unilateral and 44 cases of bilateral gonads. Microscopically, 55 gonads (56.7%) showed dysplastic testes including 17 unilateral and 19 bilateral gonads. Fourteen were streak gonads (14.4%) including 8 unilateral and 3 bilateral gonadal tissues. Nine streak gonad with epithelial cord-like structures (9.3%) were found, of which 5 were unilateral and 2 were bilateral lesions. Seven gonads were ovotestis (7.2%), unilateral in 5 cases (the other side of the gonads of ovary in 4 cases, 1 case of dysplastic testes) and bilateral in 1 case. Seven gonads showed follicular-rich ovarian tissue (7.2%). One case showed bilateral dysplastic testes with gonadoblastoma and ectopic adrenal cortex. One case of streak gonad showed epithelial cord-like structures and undifferentiated glandular tissue embedded in malignant mixed germ cell tumors (mixed gonadoblastoma, dysgerminoma, mature teratoma and yolk sac tumor). One case had testicular microlithiasis. Uterus and fallopian tube structures were found in 11 cases. Immunohistochemical stains were performed in 15 cases. D2-40, PLAP and CKIT were expressed in germ cells and Calretinin, WT1 and inhibin were positive in Setoli cells. SALL4 and OCT3/4 were positive in 3 cases. Inhibin highlighted interstitial Leydig cells in 2 cases. GPC3 was positive in yolk sac tumor component.@*Conclusions@#Gonadal dysgenesis presents a broad spectrum of gonadal phenotypes with variable degrees of differentiation. The development of bilateral gonadal tissues has certain variability. Chromosomal karyotypes have no correlation with gonadal phenotypes. Accurate histopathologic diagnosis of gonadal dysgenesis plays an important role in the treatment and prognosis of the patient.

The application of 3-D scanning in the diagnosis and evaluation of pectus excavatum / 中华胸心血管外科杂志

Objective To investigate the application of 3-D scanning in the diagnosis and evaluation of pectus excava-tum.Methods From July 2016 to June 2017, chest CT concomitant 3-D scanning were performed in 90 pectus excavatum pa-tients before Nuss procudure.Another 30 cases underwent chest CT scanning for non-chest deformity causes were chosen as a control group.The transverse and anterior-posterior maximum diameter through the deepest point of chest wall deformity were measured.The surface topography index and Haller index were calculated respectively .Results There was a positive correla-tion between CT and 3-D scanning diameters of the transverse and anterior-posterior maximum diameters, and consistency rates were 94% and 82%, respectively.There was significant difference between pectus excavatum group 1.82 ±0.21 and control group 1.41 ±0.07 in the STI(P<0.001).There was a positive correlation between Haller index 5.12 ±3.36 and STI 1.82 ± 0.21, and consistency rate was 89%.The Delong's test showed no significant difference between ROC of HI and STI(Z =1.18, P=0.28).Conclusion 3-D scanning and STI of pectus excavatum is a validated alternative for CT and Haller index, especially in the infant and non-surgical treatment PE cases.

Clinical characteristics, diagnosis and treatment of pulmonary inflammatory myofibroblastic tumor in children / 中华肿瘤杂志

Objective@#To explore the clinical characteristics, diagnosis and treatment of pulmonary inflammatory myofibroblastic tumor in children.@*Methods@#The clinical data of 13 patients with pulmonary inflammatory myofibroblastic tumor between October 2009 and October 2015 were retrospectively analyzed, including 9 boys and 4 girls aged 1 to 12 years 4 months (mean 4 years 9 months). Seven patients underwent total tumor resection, 4 had lobectomy, 1 had partial tumor resection and 1 had biopsy. The follow-up time ranged from 2 months to 6 years and 2 months.@*Results@#Among the 13 patients, 9 were cured. Two patients lived with tumor. One of them had tumor which shrinked gradually and the other had stable disease. One patient with multiple lesions encountered recurrence after 3 operations and developed suspicious hepatic metastasis. One died during the operation because of airway mismanagement.@*Conclusions@#Pulmonary inflammatory myofibroblastic tumor in children has rather good prognosis while a few cases have the potential of recurrence and canceration. Surgery is the primary therapeutic approach. Close post-operative follow up is necessary.

Effect of Inhibition of Uygur Fomes Officinalis Polysaccharides on Antioxidant Capacity and Exercise Immunity / 世界科学技术-中医药现代化

This study was aimed to investigate the effects of Fomes officinalis polysaccharides (FoP) on exercise-induced fatigue and exercise immune suppression in mice with FoP induced by exercise fatigue and unbalanced exercise immunity.Through treadmill exercise,the long-term exercise immune suppression model fatigue was established.Different doses of FoP were used in the animal experiment.The experimental animal were randomly divided into the positive control group,negative control group,low dose FoP (20 mg· kg-1) + exercise group,middle dose FoP (40 mg· kg-1) + exercise group,and the high dose FoP (80 mg· kg-1) + exercise group.The intragastric administration was given 6 days per week for 8 weeks.Treadmill exercise was administered during these 8 weeks.After 8 weeks,the samples were sacrificed.The levels of malondialdehyde (MDA),superoxide dismutase (SOD),hemoglobin (HB) and creatine kinase (CK) were detected by kit.And the relative expression of IL-4mRNA,INF-γmRNA was detected by RT-PCR.The results showed that compared with the positive control group,FoP can significantly improve the level of HB in mice,the level of serum CK in each FoP dose group was significantly decreased;compared with the positive control group,FoP group can significantly eliminate MDA with no significant difference among different FoP dose groups.The contents of SOD in serum of different dose FoP group and the negative control group were significantly higher than that of the positive control group.The IL-4 mRNA/INF-γ mRNA ratio of different dose FoP group were in basic equilibrium.It was concluded that Uygur FoP can effectively promote the body fatigue recovery,accelerate the clearance of free radicals,and improve the antioxidant ability and immune status.

Study on the correlation between personality character and facial emotion cognition / 中华行为医学与脑科学杂志

Objective To explore the relationship between personality traits and facial emotion rec?ognition. Methods By opening recruitment,268 health undergraduates were measured using Eysenck Per?sonality Questionnaire and JACFEE facial emotion recognition test. Correlation analysis was administrated be?tween different gender,personality dimensions and facial emotion recognition ability. Results Female had better emotion recognition ability than male((44.18±5.83) vs (41.88±6.79), t=-2.976, P=0.003). There were significant differences among recognition frequency of no?expression picture to other basic facial emo?tions(χ2=37.877, P=0.004) . There were correlative relations between personality dimensions and facial e?motion recognition. Among choleric temperament personality,P dimension had negative correlation with fear emotion( r=-0.223, P=0.028). N dimension had negative correlation with fear emotion( r=-0.253, P=0.013) . Among sanguineous temperament personality,E dimension had negative correlation with contempt e?motion( r=-0.258, P=0.027) ,and P dimension had positive correlation with contempt emotion( r=-0.239, P=0.042).Among melancholic temperament,E dimension had positive correlation with fear emotion( r=0.283, P=0.023) ,and P dimension had positive correlation with amazing emotion( r=0.299, P=0.016) . A?mong phlegmatic temperament, there was no any correlation between personality dimension and emotion recognization. Conclusion Female has better emotion recognition ability than male. There is cognitive tend?ency between personality character and emotion recognition.

Pediatric lung lesions: a clinicopathological study of 215 cases / 中华病理学杂志

<p><b>OBJECTIVE</b>To investigate clinical and pathological features of lung lesions in children.</p><p><b>METHODS</b>Clinical manifestations, radiologic imaging, histopathological features and immunohistochemical results were analyzed in 215 cases of lung lesions in children.</p><p><b>RESULTS</b>A total of 215 cases of lung lesions in children aged 0 day to 13 years (average age of 27.2 months and the median age of 18.0 months) were selected, including 137 male and 78 female patients with a male to female ratio of 1.76:1.00. The incidence of congenital lung disease was higher in patients of less than 1 year old than those of over 1 year old age, and the difference of the two groups was statistically significant (P = 0.004). 142 cases had acquired lung diseases, and 73 cases had congenital bronchopulmonary dysplasia. Lung abscess was the most common lesion seen in 86 cases (40.0%), including 1 case of fungal abscess. Congenital pulmonary airway malformation (CPAM) was the second most common, seen in 44 patients (20.5%), including 20 cases of type 1, 18 cases of type 2 and 6 cases of type 4 CPAM. Pulmonary sequestration was found in 25 cases (11.6%) including 14 cases of intralobar type and 11 cases of extralobar type. Two cases of extralobar pulmonary sequestration showed simultaneous CPAM2 type 2 lesion. Other lesions included tuberculosis (13 cases, 6.0%), emphysema (12 cases, 5.6%), interstitial pneumonia (7 cases, 3.2%), pulmonary hemorrhage (6 cases, 2.8%), bronchogenic cyst (4 cases, 1.9%), bronchiolitis obliterans (2 cases, 0.9%), idiopathic pulmonary hemosiderin deposition disease (2 cases, 0.9%) and 1 cases of lung non-specific changes. 13 cases of neoplastic lesions (6.0%) were found, of which 11 cases were primary tumors (5.1%), including inflammatory myofibroblastic tumor in 5 patients (2.3%), pleuropulmonary blastoma in 5 cases (1 case of type I, 2 type II and 2 type III) and 1 case of mucoepidermoid carcinoma (0.5%) and 2 cases of metastatic tumors (hepatoblastoma and Wilm's tumor, 0.9%).</p><p><b>CONCLUSIONS</b>Infectious diseases are the most common lung diseases in children. Congenital bronchopulmonary dysplasia is the most common in children of less than 1 year old. Malignant lesions are rare.</p>

The giant neurofibroma of penis in a child: a case report and literature review / 中华实用儿科临床杂志

Objective To investigate the clinical and pathological characteristics,diagnosis,differential diagnosis,treatment and prognosis of giant neurofibroma of penis in the child.Methods The clinical data including general data,imaging data,treatment methods,pathological characteristics of a case with giant neurofibroma of penis in a child were analyzed retrospectively and the relevant literature was reviewed.Results Gross appearance of the penile shaft neurofibroma was about 9 cm × 11 cm × 15 cm,with local ulceration.Computerized tomography scan revealed a giant mass in the penile shaft,about 9.0 cm × 10.0 cm × 13.4 cm.Partial excision of the penis was performed.Postoperative appearance of the residual penile shaft was about 2 cm long.The HE staining showed spindle cells with the red dye cytoplasm,spindle or elliptic nuclei and arranged in wavy partly.Positive immunostaining was presented with S-100 protein and Vimentin.The pathologic examination revealed a neurofibroma.There was no evidence of recurrence and the penis of the boy had normal sensation and erection by follow-up in 2 years.Conclusions Neurofibroma of penis in the child is extremely rare and the differential diagnosis of soft-tissue tumors of penis should be considered.The operative method should be individualized,the treatment goal is the complete resection;however,this goal must be weighed against detriment to functioning and the cosmetics of the involved organ.

Clinicopathological features of cystic lung diseases in children / 临床与实验病理学杂志

Purpose To investigate the histopathological features of cystic lung diseases ( CLD) , and to discuss the timing of clinical interventions. Methods HE and immunohistochemical staining were performed and reviewed in 125 cases of CLD. Results 125 ca-ses of CLD aged from birth to 11 years and 6 month, with an average age of 23. 0 months, median age 15 months, of which 60 cases were less than 1 year (48. 0%). 75 cases were male and 50 cases female, with male to female ratio of 1. 5 ∶ 1. Grossly, 50 cases showed single or multiple cysts with the size 0. 5 ~8. 0 cm in diameter, which did not communicate with bronchial cavity. 18 cases showed honeycomb cysts with the diameter of 0. 1~2. 0 cm. 26 cases were solid lesions without visible cysts. 21 cases were observed lung abscess with thick and rough wall and pus inside. 7 cases of emphysema showed microcysts with crepitation. 2 cases were identi-fied cystic and solid masses, with fish-fresh like cut surface. Histopathologically, 94 cases (75. 2%) were related to congenital bron-chopulmonary dysplasia in 125 cases of CLD, in which there were 59 patients (47. 2%) of congenial pulmonary airway malformation (CPAM), including 29 cases of type 1 (49. 2%), 18 cases of type 2 (30. 5%), and 12 cases of type 4 (20. 3%), there were 26 ca-ses (20. 8%) of pulmonary sequestration, including 15 cases of intralobar type (57. 7%) and 11 of extralobar cases (42. 3%), 5 ca-ses were complicated with CPAM type 2, 8 cases were bronchial cyst (6. 4%) and 1 case of enteric cyst (0. 8%). Acquired lesions were detected in 31 cases (24. 8%), including 21 cases of infected lung abscess, 1 case of fungal abscess. 7 cases of emphysema, and 3 cases of pleuralpulmonary blastoma (typeⅠ1 case and typeⅡ2 cases). Conclusion Pediatric CLD is characterized as com-plexed categories. The prognosis depends on correct pathological diagnosis, combined with imaging evaluation and appropriate timing of surgery.

Prognostic significance of MYCN amplification in children neuroblastic tumors / 中华病理学杂志

<p><b>OBJECTIVE</b>To summarize the clinicopathologic features of neuroblastic tumors (NT), and to explore the prognostic significance of MYCN amplification in NT.</p><p><b>METHODS</b>The clinicopathologic data of 267 NT were reviewed. MYCN gene amplification was detected by fluorescence in situ hybridization (FISH) in 119 cases and the relationship with pathological characteristics and prognostic significance were analyzed.</p><p><b>RESULTS</b>The study included 267 cases of children NT from patients aged from 1 day to 13 years (median 27 months). The male to female ratio was 1.43. There were 38 cases (14.2%), 43 cases (16.1%), 71 cases (26.6%), and 115 cases (43.1%) of INSS stages I, II, III and IV respectively.Favorable histology group had 157 cases (59.9%); unfavorable histology group had 110 cases (40.1%).Of the 119 NT cases with MYCN FISH performed, 18 cases (15.1%) showed amplification and the signal ratio of MYCN to CEP2 was 4.08-43.29. One hundred and one cases of non-amplified MYCN included MYCN gain in 79 cases (66.3%) and MYCN negative in 22 cases (18.5%). MYCN expression showed significant difference (P = 0.000) between ages, gender, NT type and MKI, but not INPC and clinical stage (P > 0.05).Of the 18 cases with MYCN amplification, 3 were undifferentiated, and 15 poorly differentiated; 17 had high MKI and one moderate MKI. All 18 cases were in unfavorable histology group; the overall survival rate was 3/18, with an average survival time of (17.9 ± 2.4) months.Of the 101 MYCN non-amplification cases, the overall survival rate was 68.3% (69/101), with an average survival time of (29.8 ± 1.3) months. Survival analysis showed the cases with MYCN amplification had worse prognosis (P < 0.05).</p><p><b>CONCLUSIONS</b>NT were commonly diagnosed in early ages and easily to metastasize. Most of cases with favorable histology. The cases of MYCN amplification showed unfavorable histology, and the majority cases with high MKI; The patients with MYCN gene amplification had poor prognosis.</p>

Detection of MYCN gene amplification in neuroblastic tumors by fluorescence in situ hybridization and its clinical significance / 中华实用儿科临床杂志

Objective To explore the significance of MYCN gene amplification in children with neuroblastic tumors(NT).Methods The clinicopathological data of 154 cases with NT were reviewed,including general data, classification of pathology,clinical stage and prognosis.MYCN gene amplification was detected by fluorescence in situ hybridization(FISH) and its relationship between pathological characteristics and prognostic significance was analyzed.Results There was 154 cases of NT aged 1 day to 11 years,with a mean age of 26.1 months,and the median age of 20.5 months.Male and female ratio was 1.48 : 1.00.According to International Neuroblastoma Staging System (INSS) ,20 cases were of stage Ⅰ (13.0％) ,23 cases of stage Ⅱ (14.9％) ,43 cases of stage Ⅲ (27.9％) ,64 cases of stage Ⅳ(41.6％) and 4 cases of Ⅳs (2.6％).There were 72 cases(46.8％) with favorable histology,and 82 cases(53.2％) with unfavorable histology.MYCN amplification was found in 20 cases (13.0％) and the signal ratio of MYCN and chromosome 2 (CEP2) was 4.08-43.29.One hundred and thirty-four cases of MYCN non-amplification included MYCN gain in 91 cases(68.0％) ,MYCN negative in 43 cases(32.0％).MYCN expression showed the significant differences in ages, neuroblastoma type, international neuroblastoma pathology classification (INPC), mitosis karyorrhexis index (MKI), and clinical stages (all P ＜ 0.05).No significant difference was found in gender(P ＞ 0.05).Of 20 MYCN amplification cases,4 cases (20.0％) survived and 16 cases (80.0％) died,and the overall survival rate was 20.0％ (4/20 cases) ,with survival time was (17.10 ± 2.24) months;of 134 MYCN non-amplification cases,96 cases (71.6％) survived and 38 cases (28.4％) died, with survival time of (28.71 ± 1.28)months.Survival analysis showed the cases with MYCN amplification had worse prognosis (x2 =19.596, P ＜ 0.05).Conclusions Patients with MYCN amplification had poorer prognosis and lower incidence of MYCN amplification of pediatric NT was found in China.