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https://www.readbyqxmd.com/read/28910893/-pediatric-laryngeal-clefts-an-experience-in-the-diagnosis-and-management-of-13-cases
#1
Z B Wu, L Li, H G Pan, Z J Liang, Z X Xian, D L Zhang, Y S Teng, X Y Ma
Objective: To investigate the diagnosis and management of laryngeal cleft. Method: The clinical data of 13 cases of laryngeal cleft treated between 2007 and 2015 was analyzed retrospectively. Results: The children with laryngeal cleft were classified according to the classification of Benjamin-Inglis, as type Ⅰ(11 cases), typeⅡ(1 case) and type Ⅲ(1 case). All patients were confirmed by microlaryngobronchoscopy under general anaesthetic. Eleven typeⅠ and 1 type Ⅱ clefts were managed conservatively, with which all type Ⅰ patients were successfully managed, while the type Ⅱ patient was resolved by surgical endoscopy...
September 7, 2017: Zhonghua Er Bi Yan Hou Tou Jing Wai Ke za Zhi, Chinese Journal of Otorhinolaryngology Head and Neck Surgery
https://www.readbyqxmd.com/read/28886269/loss-of-function-grhl3-variants-detected-in-african-patients-with-isolated-cleft-palate
#2
M A Eshete, H Liu, M Li, W L Adeyemo, L J J Gowans, P A Mossey, T Busch, W Deressa, P Donkor, P B Olaitan, B S Aregbesola, R O Braimah, G O Oseni, F Oginni, R Audu, C Onwuamah, O James, E Augustine-Akpan, L A Rahman, M O Ogunlewe, F K N Arthur, S A Bello, P Agbenorku, P Twumasi, F Abate, T Hailu, Y Demissie, A Hailu, G Plange-Rhule, S Obiri-Yeboah, M M Dunnwald, P E Gravem, M L Marazita, A A Adeyemo, J C Murray, R A Cornell, A Butali
In contrast to the progress that has been made toward understanding the genetic etiology of cleft lip with or without cleft palate, relatively little is known about the genetic etiology for cleft palate only (CPO). A common coding variant of grainyhead like transcription factor 3 ( GRHL3) was recently shown to be associated with risk for CPO in Europeans. Mutations in this gene were also reported in families with Van der Woude syndrome. To identify rare mutations in GRHL3 that might explain the missing heritability for CPO, we sequenced GRHL3 in cases of CPO from Africa...
September 1, 2017: Journal of Dental Research
https://www.readbyqxmd.com/read/28879324/cause-analysis-prevention-and-treatment-of-postoperative-restlessness-after-general-anesthesia-in-children-with-cleft-palate
#3
REVIEW
Hao Xu, Xiao-Peng Mei, Li-Xian Xu
Cleft palate is one of the most common congenital malformations of the oral and maxillofacial region, with an incidence rate of around 0.1%. Early surgical repair is the only method for treatment of a cleft lip and palate. However, because of the use of inhalation anesthesia in children and the physiological characteristics of the cleft palate itself combined with the particularities of cleft palate surgery, the incidence rate of postoperative emergence agitation (EA) in cleft palate surgery is significantly higher than in other types of interventions...
March 2017: J Dent Anesth Pain Med
https://www.readbyqxmd.com/read/28863859/grafting-materials-for-alveolar-cleft-reconstruction-a-systematic-review-and-best-evidence-synthesis
#4
REVIEW
C Wu, W Pan, C Feng, Z Su, Z Duan, Q Zheng, C Hua, C Li
The purpose of this study was to compare the efficacy of alveolar bone reconstruction for alveolar cleft patients performed with the traditional iliac graft or alternative/supplementary bone grafting materials. Electronic databases, relevant journals, and reference lists of the included studies were searched to the end of June 2016. A best-evidence synthesis was performed to draw conclusions. A total of 38 studies were included, which provided 25 pieces of evidence: seven of moderate evidence and 18 of insufficient evidence...
August 29, 2017: International Journal of Oral and Maxillofacial Surgery
https://www.readbyqxmd.com/read/28838949/structural-insights-into-the-substrate-specificity-of-a-glycoside-hydrolase-family-5-lichenase-from-caldicellulosiruptor-sp-f32
#5
Dong-Dong Meng, Xi Liu, Sheng Dong, Ye-Fei Wang, Xiao-Qing Ma, Haixia Zhou, Xinquan Wang, Li-Shan Yao, Yingang Feng, Fu-Li Li
Glycoside hydrolase (GH) family 5 is one of the largest GH families with various GH activities including lichenase, but the structural basis of the GH5 lichenase activity is still unknown. A novel thermostable lichenase F32EG5 belonging to GH5 was identified from an extremely thermophilic bacterium Caldicellulosiruptor  sp. F32. F32EG5 is a bi-functional cellulose and lichenan-degrading enzyme and exhibited a high activity on β-1,3-1,4-glucan but side activity on cellulose. Thin-layer chromatography and NMR analyses indicated that F32EG5 cleaved the β-1,4 linkage or the β-1,3 linkage while a 4- O -substitued glucose residue linked to a glucose residue through a β-1,3 linkage, which is completely different from extensively studied GH16 lichenase that catalyses strict endo-hydrolysis of the β-1,4-glycosidic linkage adjacent to a 3- O -substitued glucose residue in the mixed linked β-glucans...
August 24, 2017: Biochemical Journal
https://www.readbyqxmd.com/read/28822413/-diagnostic-value-of-multi-slice-spiral-computerized-tomographic-fistulography-in-congenital-fistula-of-neck
#6
H Zheng, Q Ye, X Y Wang, X H Zheng, X Q Yang, Y Chen, Y Jiang, R Y Li
Objective: To investigate the usefulness and effectiveness of multi-slice spiral computerized tomographic fistulography (MSCTF) in the diagnosis and treatment of congenital fistula of neck. Methods: Thirty-four patients with thyroglossal fistulasor branchial cleft fistulas who were initial treated from July 2008 to August 2015 in Fujian Provincial Hospital were retrospectively analyses. Thirteen males and 21 females patients aging from 3 to 46 years old with a median age of 37 were included. There were thyroglossal fistula in 6 cases, the first branchialcleft fistula in 9 cases, the second branchialcleft fistula in 3 cases, the third branchialcleft fistula in 9 cases, and the fourth branchialcleft fistula in 7 cases...
August 7, 2017: Zhonghua Er Bi Yan Hou Tou Jing Wai Ke za Zhi, Chinese Journal of Otorhinolaryngology Head and Neck Surgery
https://www.readbyqxmd.com/read/28816718/double-composite-tissue-z-plasty-technique-for-anatomical-restoration-of-severe-nasal-deformity-in-secondary-unilateral-cleft-lip
#7
Jiao Wei, Tanja Herrler, Hua Xu, Qingfeng Li, Chuanchang Dai
BACKGROUND: Patients with secondary unilateral cleft lip are regularly affected by serious nasal deformities especially of the alar and nasal floor. A large number of techniques for correction have been published, but symmetrical restoration of severe nasal deformation is difficult to achieve. We propose an innovative approach for anatomical restoration for this entity of nasal deformities to achieve long-term symmetrical appearance and muscular function. METHODS: A total of 68 patients with severe nasal deformity due to secondary unilateral cleft lip underwent reconstructive surgery using a double composite tissue Z-plasty technique for anatomical restoration of cartilage, muscle, and soft tissue layers...
October 2017: Annals of Plastic Surgery
https://www.readbyqxmd.com/read/28811590/structural-analysis-of-wss1-protein-from-saccharomyces-cerevisiae
#8
Xiaoyun Yang, Yanhua Li, Zengqiang Gao, Zongqiang Li, Jianhua Xu, Wenjia Wang, Yuhui Dong
Wss1 is a DNA-protein crosslinks (DPCs) repair protein, which is responsible for degradation of the protein components in DPCs. In this investigation, crystal structure of the protease domain from saccharomyces cerevisiae Wss1 (ScWss1) was solved and was compared with the known crystal structure of Schizosaccharomyces prombe Wss1 (SpWss1). It is found that the cleft near zinc ion to be the most conserved core region of Wss1 and that the electronic surface distributions vary greatly between the two homologs...
August 15, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28796110/surgery-navigation-in-treating-congenital-midfacial-dysplasia-of-patients-with-facial-cleft
#9
Dong Li, Shanshan Bai, Zheyuan Yu, Liang Xu, Jie Yuan, Haisong Xu, Min Wei
AIM: To explore a new accurate way for the treatment of congenital midfacial dysplasia in facial cleft patients. MATERIALS AND METHODS: Between November 2015 and November 2016, 8 patients with nasal deformity and midfacial dysplasia (Tessier Nos. 3-11 cleft) were collected (median age, years; range = 15-20 years). Expanded frontal flap for nasal reconstruction and image-guided navigation-assisted surgery for modified nasal-maxillary-hard palatine osteotomy to advance the peri-pyriform bone structure were performed in all the patients...
September 2017: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/28777481/a-human-case-of-slc35a3-related-skeletal-dysplasia
#10
Andrew C Edmondson, Emma C Bedoukian, Matthew A Deardorff, Donna M McDonald-McGinn, Xueli Li, Miao He, Elaine H Zackai
Researchers have identified a subset of Holstein having a range of skeletal deformities, including vertebral anomalies, referred to as complex vertebral malformation due to mutations in the SLC35A3 gene. Here, we report the first case in humans of SLC35A3-related vertebral anomalies. Our patient had prenatally diagnosed anomalous vertebrae, including butterfly, and hemivertebrae throughout the spine, as well as cleft palate, micrognathia, patent foramen ovale, patent ductus arteriosus, posterior embryotoxon, short limbs, camptodactyly, talipes valgus, rocker bottom feet, and facial dysmorphism including proptosis, nevus flammeus, and a cupped left ear...
August 4, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28767592/bmp4-rs17563-polymorphism-and-nonsyndromic-cleft-lip-with-or-without-cleft-palate-a-meta-analysis
#11
Yue-Hua Li, Jiaomei Yang, Ju-Lei Zhang, Jia-Qi Liu, Zhao Zheng, Da-Hai Hu
BACKGROUND: Previous studies have investigated the relationship between human bone morphogenetic protein 4 gene (BMP4) rs17563 polymorphism and nonsyndromic cleft lip with or without cleft palate (NSCL/P). However, the results remained inconsistent. Therefore, we conducted a meta-analysis to assess the effect of BMP4 rs17563 polymorphism on NSCL/P. METHODS: Electronic searches in 5 databases were conducted to select all eligible studies up to March 2017. Odds ratios (ORs) with the corresponding 95% confidence intervals (CIs) were calculated to estimate the association...
August 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28765038/synergistic-effect-of-1%C3%AE-25-dihydroxyvitamin-d3-and-17%C3%AE-estradiol-on-osteoblast-differentiation-of-pediatric-mscs
#12
Jing Li, Bonnie L Padwa, Shuanhu Zhou, Julia Mullokandova, Meryl S LeBoff, Julie Glowacki
Vitamin D is essential for mineral homeostasis and contributes to bone metabolism by stimulating osteoblast differentiation of marrow stromal cells (MSCs). In this study, we used MSCs from pre-pubertal girls and boys to test the hypothesis that 1α,25(OH)2D and 17β-estradiol have synergistic effects on these MSCs, and what mechanism is involved. With IRB approval, we isolated MSCs from discarded excess iliac marrow graft from children undergoing alveolar cleft repair. Plasma was available from 8 female (9...
July 29, 2017: Journal of Steroid Biochemistry and Molecular Biology
https://www.readbyqxmd.com/read/28746297/alveolar-bone-grafting-and-cleft-lip-and-palate-a-review
#13
LETTER
Xiao-Lin Liu, Bing Shi, Qian Zheng, Cheng-Hao Li
No abstract text is available yet for this article.
August 2017: Plastic and Reconstructive Surgery
https://www.readbyqxmd.com/read/28745929/molecular-and-cellular-mechanisms-of-palate-development
#14
C Li, Y Lan, R Jiang
Development of the mammalian secondary palate involves highly dynamic morphogenetic processes, including outgrowth of palatal shelves from the oral side of the embryonic maxillary prominences, elevation of the initially vertically oriented palatal shelves to the horizontal position above the embryonic tongue, and subsequently adhesion and fusion of the paired palatal shelves at the midline to separate the oral cavity from the nasal cavity. Perturbation of any of these processes could cause cleft palate, a common birth defect that significantly affects patients' quality of life even after surgical treatment...
April 1, 2017: Journal of Dental Research
https://www.readbyqxmd.com/read/28719706/bilateral-second-branchial-cleft-fistulae-in-a-boy-with-congenital-heart-disease
#15
Yi-Fang Lee, En-Li Shiau
No abstract text is available yet for this article.
July 2017: Ear, Nose, & Throat Journal
https://www.readbyqxmd.com/read/28716923/molecular-mechanism-of-environmental-d-xylose-perception-by-a-xylfii-lyts-complex-in-bacteria
#16
Jianxu Li, Chengyuan Wang, Gaohua Yang, Zhe Sun, Hui Guo, Kai Shao, Yang Gu, Weihong Jiang, Peng Zhang
d-xylose, the main building block of plant biomass, is a pentose sugar that can be used by bacteria as a carbon source for bio-based fuel and chemical production through fermentation. In bacteria, the first step for d-xylose metabolism is signal perception at the membrane. We previously identified a three-component system in Firmicutes bacteria comprising a membrane-associated sensor protein (XylFII), a transmembrane histidine kinase (LytS) for periplasmic d-xylose sensing, and a cytoplasmic response regulator (YesN) that activates the transcription of the target ABC transporter xylFGH genes to promote the uptake of d-xylose...
August 1, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28708640/is-cleft-severity-correlated-with-intrinsic-growth-pattern-observation-from-unoperated-adult-patients-with-submucous-cleft-palate
#17
Congcong Cao, Xue Xu, Bing Shi, Qian Zheng, Jingtao Li
OBJECTIVE: This study aimed to evaluate the craniofacial morphology of unoperated adult submucous cleft palate (SMCP) patients and to explore the possible correlation between the intrinsic growth insufficiency of the maxillofacial complex and the severity of the cleft. MATERIALS AND METHODS: A total of 20 unoperated SMCP patients, 20 unoperated overt cleft palate (OCP) patients, and 32 normal controls, ages between 18 and 30, were included for cephalometric analysis...
September 2017: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/28692886/synthesis-and-characterization-of-oxidovanadium-complexes-as-enzyme-inhibitors-targeting-dipeptidyl-peptidase-iv
#18
Ming-Jin Xie, Ming-Rong Zhu, Chun-Mei Lu, Yi Jin, Li-Hui Gao, Ling Li, Jie Zhou, Fan-Fang Li, Qi Hua Zhao, Hong-Ke Liu, Peter J Sadler, Carlos Sanchez-Cano
Two oxidovanadium(IV) complexes carrying Schiff base ligands obtained from the condensation of 4,5-dichlorobenzene-1,2-diamine and salicylaldehyde derivatives were synthesised and characterised, including their X-ray crystallographic structures. They were evaluated as dipeptidyl peptidase IV (DPP-IV) inhibitors for the treatment of type 2 diabetes. These compounds were moderate inhibitors of DPP-IV, with IC50 values of ca. 40μM. In vivo tests showed that complexes 1 and 2 could lower significantly the level of glucose in the blood of alloxan-diabetic mice at doses of 22...
June 29, 2017: Journal of Inorganic Biochemistry
https://www.readbyqxmd.com/read/28692808/modulating-wnt-signaling-rescues-palate-morphogenesis-in-pax9-mutant-mice
#19
C Li, Y Lan, R Krumlauf, R Jiang
Cleft palate is a common birth defect caused by disruption of palatogenesis during embryonic development. Although mutations disrupting components of the Wnt signaling pathway have been associated with cleft lip and palate in humans and mice, the mechanisms involving canonical Wnt signaling and its regulation in secondary palate development are not well understood. Here, we report that canonical Wnt signaling plays an important role in Pax9-mediated regulation of secondary palate development. We found that cleft palate pathogenesis in Pax9-deficient embryos is accompanied by significantly reduced expression of Axin2, an endogenous target of canonical Wnt signaling, in the developing palatal mesenchyme, particularly in the posterior regions of the palatal shelves...
July 1, 2017: Journal of Dental Research
https://www.readbyqxmd.com/read/28675016/-velopharyngeal-closure-pattern-and-speech-performance-among-submucous-cleft-palate-patients
#20
Yin Heng, Guo Chunli, Shi Bing, Li Yang, Li Jingtao
OBJECTIVE: To characterize the velopharyngeal closure patterns and speech performance among submucous cleft palate patients. METHODS: Patients with submucous cleft palate visiting the Department of Cleft Lip and Palate Surgery, West China Hospital of Stomatology, Sichuan University between 2008 and 2016 were reviewed. Outcomes of subjective speech evaluation including velopharyngeal function, consonant articulation, and objective nasopharyngeal endoscopy including the mobility of soft palate, pharyngeal walls were retrospectively analyzed...
June 1, 2017: Hua Xi Kou Qiang Yi Xue za Zhi, Huaxi Kouqiang Yixue Zazhi, West China Journal of Stomatology
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