OBJECTIVE: This study was designed to explore the risk factors associated with severity of juvenile onset recurrent respiratory papillomatosis. METHOD: A retrospective study was conducted to study determinants of severe forms of juvenile recurrent onset respiratory papillomatosis. The patients were separated into different groups based on the onset age, the first recurrence of age, the first recurrence of period, gender and incision of tracheal respectively. The relationship among the lesion severity score,the involvement of the subregion, operation period and the next operation period were also explored. RESULT: It was observed that some children who recurred before 4 years old required more surgery, shorter operation period(the average, longest or shortest operation period) than those elder children, the differences were statistically (P=0. 029, 0. 003, 0. 010, 0. 039, respectively). The severity score of lesion was correlated positively with the involvement of the subregion and negatively with operation period (r=0. 914, -0. 451, respectively). Some children who diagnosed before 4 years old had to endure more severity score and shorter operation period than those older children, the differences were statistically (P= 0. 036, 0. 000, respectively). 8 cases accepted incision of tracheal, they accepted more surgery too. But the differences in the onset age, the first recurrence of age, and the operation period were not statistically. CONCLUSION The results showed that the clinical course of juvenile onset recurrent respiratory papillomatosis was closely related to the first recurrence age and period, while the severity of disease was associated to the onset age and the involvement of the subregion.
Adolescent ; Age of Onset ; Child ; Child, Preschool ; Humans ; Papilloma ; Papillomavirus Infections ; classification ; epidemiology ; surgery ; Respiratory Tract Infections ; classification ; epidemiology ; surgery ; Retrospective Studies ; Risk Factors ; Severity of Illness Index ; Trachea

PURPOSE: Allergic rhinitis (AR) has become a global issue for a large part of the general population. Sublingual immunotherapy (SLIT) has been used extensively to treat persistent allergic rhinitis (PAR). Although systematic reviews have confirmed the effectiveness of SLIT for the treatment of AR, a considerable number of studies using extracts of house dust mites (HDMs) for immunotherapy found no consensus on basic treatment parameters and questioned the efficacy of SLIT. METHODS: In this study, we evaluated SLIT for PAR by a meta-analysis of randomized controlled trials (RCTs). Medline, Embase, and Cochrane Library database searches were performed for RCTs on the treatment of PAR by SLIT that assessed clinical outcomes related to efficacy through May 2016. Descriptive and quantitative information was abstracted. An analysis was performed with standardized mean differences (SMDs) under a fixed or random effects model. Subgroup analyses were performed. Heterogeneity was assessed using the I2 metric. RESULTS: In total, 25 studies were eligible for inclusion in the meta-analysis for symptom scores and 15 studies for medication scores. SLIT was significantly different from the controls for symptom scores (SMD=1.23; 95% confidence interval [CI]=1.74 to 0.73; P<0.001). For medication scores, significant differences for SLIT were also observed versus the controls (SMD=-1.39; 95% CI=-1.90 to -0.88; P<0.001). CONCLUSIONS: Our meta-analysis indicates that SLIT provided significant symptom relief and reduced the need for medications in PAR. In this study, significant evidence was obtained despite heterogeneity with regard to the use of mite extract. Specifically, the mite extract used was provided by the patients with PAR. Furthermore, to confirm both the objective outcomes and the effective doses of HDM allergen extracts, experimental data should be obtained from large high-quality population-based studies.
Consensus ; Dust* ; Humans ; Immunotherapy ; Mites ; Population Characteristics ; Pyroglyphidae ; Rhinitis, Allergic* ; Sublingual Immunotherapy*

OBJECTIVE To study on the influence of human papilloma virus(HPV) type on the clinical course of juvenile onset recurrent respiratory papillomatosis(JORRP).METHODS Fresh tumor specimens of 38 patients were collected and the HPV type of the specimens was detected by flow fluorescent hybridization method.According to the results,children were divided into HPV6 and HPVll positive groups.The clinical data of the two groups were compared.RESULTS Among 38 children with recurrent respiratory papillomatosis,21(55.2％) were infected with HPV6,14(36.8％) were infected with HPVll,and 3(7.9％) were negative for HPV 6 or HPV 11.The proportion of aggressive cases in HPV6 and HPV11 groups were similar.The age of onset,preoperative clinical symptom score,number of anatomic locations,anatomic Derkay/Coltrera score and Dikkers score were significant different between the two groups (P=0.002,0.040,0.023,0.001,0.005,respectively).CONCLUSION JORRP patients with HPV11 infection had the features of smaller onset age,more severe clinical symptoms and broader invasion compared with HPV6 infection.

OBJECTIVE: To explore the clinical and genetic characteristics of a patient with 17-hydroxylase/17,20-lyase deficiency. METHODS: The patient was infertile without contraception. Laboratory examination showed her chromosomal karyotype to be 46, XX. DNA sequencing was performed to detect variants of CYP17A1 gene in the patient and her family members. RESULTS: Sanger sequencing revealed that the patient has carried homozygous variant c.1486C>T in the exon 8 of the CYP17A1 gene, which resulted in substitution of arginine by cysteine (p.Arg496Cys). Her family members were all heterozygotes for the same variant. CONCLUSION Homozygous variant of the CYP17A1 gene c.1486C>T probably underlay the 17-hydroxylase deficiency in this patient. Above finding has enabled accurate genetic counseling and prenatal diagnosis for her family.

OBJECTIVE: To analyze the molecular genetics of a Chinese pedigree with congenital hand foot cleft. METHODS: Single nucleotide polymorphism microarray (SNP array) was used to analyze the whole genome copy number variation. RESULTS: SNP array analysis showed that there was a 433 kb repeat in 10q24.31-10q24.32 region, which contained LBX1, BTRC, POLL, OPCD and FBXW4 genes. CONCLUSION Microduplication of chromosome 10q24.31-10q24.32 may be the cause of congenital hand foot cleft in this pedigree.
China ; DNA Copy Number Variations/genetics* ; Foot Deformities, Congenital/genetics* ; Hand Deformities, Congenital/genetics* ; Humans ; Pedigree

Objective To establish and evaluate chemotherapeutic phlebitis model rats induced by vinorelbine via the dorsalis pedis vein.Methods Rats were divided randomly into control and 4 different concentration of vinorelbine-induced model groups.Control rats were injected with 0.1 mL normal saline via the dorsalis pedis vein of the hind limb,while other rats were injected with different concentrations of vinorelbine(2,3,4,5 mg/mL),as above.General observations were performed and the hind limb volume was measured daily for 7 consecutive days to calculate the swelling rate.The rats were then killed and histological changes in the dorsalis pedis vein were observed by hematoxylin and eosin staining.Microstructural changes on the surface of the vascular endometrium were observed by scanning electron microscopy.Results Injection of 2,3,4,5 mg/mL vinorelbine via the dorsalis pedis vein significantly induced hind limb swelling in a concentration-dependent manner,peaking on day 3 in each group.The phlebitis rates on day 7 were 50%in the 2 mg/mL group and 83.3%in the 3 mg/mL group.Phlebitis was also induced in the 4 mg/mL and 5 mg/mL groups,including grade Ⅲ in 66.6%and grade Ⅳ in 83.3%.Histopathology showed inflammatory cell infiltration,wall thickening,lumen stenosis,and thrombosis in the tissues surrounding the veins.Scanning electron microscopy showed destruction of tight junctions of venous endothelial cells,and a rough surface of the vascular lining,resultsing in blood cell adhesion.Conclusions Injection of 0.1 mL of 3～5 mg/mL vinorelbine via the dorsalis pedis vein could induce red,swollen,and cord-like veins,as well as infiltration of inflammatory cells around the vein,thickened vein walls,lumen stenosis,and thrombosis.In addition,the surface of the venous intima was rough and adhered to numerous blood cells.All these features are consistent with those of clinical chemotherapeutic phlebitis in terms of the symptoms and pathological structure.

Objective: To investigate the value of radiomics in image quality control with low-dose CT examination of solid pulmonary nodules. Methods: Images were acquired on CT750 HD scanner, and chest pulmonary nodules phantom were scanned at different tube voltage and tube current. The radiation dose CTDI_vol under different scanning conditions were recorded, as well as CNR and SNR of each scanning sequence. The variation of radiation dose, noise, tube voltage and tube current were analyzed. All data were analyzed by radiomics analysis software. R language statistics software was adopted to analyze the extracted features by principal component analysis (PCA), and the characteristic parameters with the largest contribution rate to image quality were selected for analysis. One-way ANOVA was used to analyze all the important characteristic parameters to reveal the difference of characteristic parameters under different tube voltages. Finally, the post-test method was used to find out the differences among different tube voltage groups. Results: Radiation dose rised linearly with the increase of tube current and tube voltage. Although the overall change trend of SNR and CNR in pulmonary nodules was linearly related to the change of tube voltage and tube current, there was no clear change trend threshold at low dose, which could not accurately evaluate the image quality under low radiation. Both CNR and SNR cannot evaluate the image quality effectively, and have no practiced value for optimizing the low dose scanning parameters. There main components including Uniformity, Voxel Value Sum, and Haralick Correlation extracted by radiomics analysis software were proved to play a critical role in image quality control. The cumulative contribution rate of variance was 89.20% and the eigen values were greater than 1. Uniformity curve of characteristic parameter showed that the trend of change was correlated with the change of tube voltage and tube current, and the stability and consistency were good. Uniformity one-way ANOVA analysis showed that when the tube voltage reduced from 140 to 120 kVp, there was no difference (P=0.117) in the uniformity, while from 120 to 80 kVp, significant differences revealed (P<0.001). Considering tube current, no significant variation was observed in uniformity when current was greater than 90 mA, which indicated that tube current of 90 mA could lead to better image quality. Conclusion Radiomics analysis can effectively evaluate and control the CT image quality of low dose solid pulmonary nodules.