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31.
The prime objective of oral cavity reconstruction is restoration of function, which must be carefully studied while evaluating the success of any reconstructive endeavour. We devised a unique comprehensive test series for the purpose that is suitable for use in routine follow-ups, and capable of providing objective documentation. Functional assessment included evaluation of general health, food intake, oral competence, mastication, speech, swallowing, tongue mobility, and shoulder–neck function. Fifty patients who had had surgery were analyzed and their functional scores compared with those of 10 normal volunteers. The extent of mandibular resection and the magnitude of soft tissue excision significantly affected overall scores of function. Oral competence, as reflected by the water holding test (WHT), was uniformly impaired in the 50 patients, and in dentate individuals, the quality of bolus provided good correlation with all other functional scores. Among reconstructive modalities employed here, the bi-paddled pectoralis major flap produced the best overall scores. 相似文献
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33.
Chakraborty S Garg P Ramamurthy T Thungapathra M Gautam JK Kumar C Maiti S Yamasaki S Shimada T Takeda Y Ghosh A Nair GB 《Journal of medical microbiology》2001,50(10):879-888
This study identified 17 matching serogroups of Vibrio cholerae belonging to serogroups other than O1 and O139 isolated from human cases and from the environment during a concurrent clinical and environmental study conducted in Calcutta, a cholera endemic area. Isolates within these matching serogroups were compared by various phenotypic and genotypic traits to determine if the environment was the source of the organisms associated with the disease. Clinical strains of V. cholerae were resistant to a greater number of drugs and exhibited multi-drug resistance compared with their environmental counterparts. Except for the presence of the genes for the El Tor haemolysin and the regulatory element ToxR in most of the strains of V. cholerae examined, non-O1, non-O139 V. cholerae strains lacked most of the other known virulence traits associated with toxigenic V. cholerae O1 or O139. Restriction fragment-length polymorphism of virulence-associated genes, ribotypes and DNA fingerprints of strains of matched serogroups showed considerable diversity, although some gene polymorphisms and ribotypes of a few strains of different serogroups were similar. It is concluded that despite sharing the same serogroup, environmental and clinical isolates were genetically heterogeneous and were of different lineages. 相似文献
34.
Pattern of Sequence Variation Across 213 Environmental Response Genes 总被引:10,自引:0,他引:10
Robert J. Livingston Andrew von Niederhausern Anil G. Jegga Dana C. Crawford Christopher S. Carlson Mark J. Rieder Sivakumar Gowrisankar Bruce J. Aronow Robert B. Weiss Deborah A. Nickerson 《Genome research》2004,14(10A):1821-1831
To promote the clinical and epidemiological studies that improve our understanding of human genetic susceptibility to environmental exposure, the Environmental Genome Project (EGP) has scanned 213 environmental response genes involved in DNA repair, cell cycle regulation, apoptosis, and metabolism for single nucleotide polymorphisms (SNPs). Many of these genes have been implicated by loss-of-function mutations associated with severe diseases attributable to decreased protection of genomic integrity. Therefore, the hypothesis for these studies is that individuals with functionally significant polymorphisms within these genes may be particularly susceptible to genotoxic environmental agents. On average, 20.4 kb of baseline genomic sequence or 86% of each gene, including a substantial amount of introns, all exons, and 1.3 kb upstream and downstream, were scanned for variations in the 90 samples of the Polymorphism Discovery Resource panel. The average nucleotide diversity across the 4.2 MB of these 213 genes is 6.7 × 10-4, or one SNP every 1500 bp, when two random chromosomes are compared. The average candidate environmental response gene contains 26 PHASE inferred haplotypes, 34 common SNPs, 6.2 coding SNPs (cSNPs), and 2.5 nonsynonymous cSNPs. SIFT and Polyphen analysis of 541 nonsynonymous cSNPs identified 57 potentially deleterious SNPs. An additional eight polymorphisms predict altered protein translation. Because these genes represent 1% of all known human genes, extrapolation from these data predicts the total genomic set of cSNPs, nonsynonymous cSNPs, and potentially deleterious nonsynonymous cSNPs. The implications for the use of these data in direct and indirect association studies of environmentally induced diseases are discussed. 相似文献
35.
Alan Ma Sunita Gurnasinghani Edwin P. Kirk Conor McClenaghan Gautam K. Singh Dorothy K. Grange Chetan Pandit Yung Zhu Tony Roscioli George Elakis Michael Buckley Bhavesh Mehta Philip Roberts Jonathan Mervis Andrew Biggin Colin G. Nichols 《American journal of medical genetics. Part A》2019,179(8):1585-1590
Cantú syndrome (CS), characterized by hypertrichosis, distinctive facial features, and complex cardiovascular abnormalities, is caused by pathogenic variants in ABCC9 and KCNJ8 genes. These genes encode gain‐of‐function mutations in the regulatory (SUR2) and pore‐forming (Kir6.1) subunits of KATP channels, respectively, suggesting that channel‐blocking sulfonylureas could be a viable therapy. Here we report a neonate with CS, carrying a heterozygous ABCC9 variant (c.3347G>A, p.Arg1116His), born prematurely at 32 weeks gestation. Initial echocardiogram revealed a large patent ductus arteriosus (PDA), and high pulmonary pressures with enlarged right ventricle. He initially received surfactant and continuous positive airway pressure ventilation and was invasively ventilated for 4 weeks, until PDA ligation. After surgery, he still had ongoing bilevel positive airway pressure (BiPAP) requirement, but was subsequently weaned to nocturnal BiPAP. He was treated for pulmonary hypertension with Sildenafil, but failed to make further clinical improvement. A therapeutic glibenclamide trial was commenced in week 11 (initial dose of 0.05 mg–1 kg–1 day–1 in two divided doses). After 1 week of treatment, he began to tolerate time off BiPAP when awake, and edema improved. Glibenclamide was well tolerated, and the dose was slowly increased to 0.15 mg?1 kg?1day?1 over the next 12 weeks. Mild transient hypoglycemia was observed, but there was no cardiovascular dysfunction. Confirmation of therapeutic benefit will require studies of more CS patients but, based on this limited experience, consideration should be given to glibenclamide as CS therapy, although problems associated with prematurity, and complications of hypoglycemia, might limit outcome in critically ill neonates with CS. 相似文献
36.
DFNA54, a third locus for low-frequency hearing loss 总被引:1,自引:0,他引:1
Gürtler N Kim Y Mhatre A Schlegel C Mathis A Lalwani AK 《Journal of molecular medicine (Berlin, Germany)》2004,82(11):775-780
Nonsyndromic hereditary hearing impairment (NSHHI) is a highly heterogeneous disorder with more than 90 loci mapped, of which nearly one-half of the responsible genes are identified. In dominant NSSHI hearing loss is typically biased towards the high frequencies while low-frequency hearing loss is unusual. Only two NSHHI loci, DFNA1 and DFNA6/14/38, are associated with predominantly low- frequency loss. We mapped the loci harboring the gene responsible for autosomal dominant low-frequency hearing loss in a multigenerational family. The pedigree of a Swiss family with low-frequency hearing loss was established. Using genomic DNA, DFNA1 and DFNA6/14/38 were excluded by linkage analysis or by direct sequencing of the responsible gene. Genome-wide linkage analysis was performed using commercially available microsatellite markers. Two-point linkage analysis demonstrated linkage to chromosome 5q31, the locus for DFNA15, with a lod score of 6.32 at recombination fraction =0 for marker D5S436. Critical recombinations were seen at markers D5S1972 and D5S410. Sequencing of the corresponding gene POU4F3 yielded no pathogenic mutation segregating with the affected members. In addition to Wolfram syndrome gene 1 (DFNA6/14/38) and diaphanous (DFNA1) there is evidence for a third gene involved in low-frequency hearing loss located at DFNA15. Because of the differences in auditory phenotype and the absence of pathogenic mutation in the coding region of POU4F3 it is likely that there is a second gene in 5q31, designated DFNA54, associated with NSHHI. 相似文献
37.
The peripheral muscle membrane protein rapsyn is essential for the formation and maintenance of high density acetylcholine receptor aggregates at the neuromuscular synapse. Rapsyn is concentrated at synaptic sites and is colocalized with acetylcholine receptors from the earliest stages of synaptogenesis. Previous studies have shown that recombinant rapsyn expressed in heterologous cells forms clusters, and acetylcholine receptors coexpressed with rapsyn are colocalized with rapsyn clusters. However, the molecular interactions involved in clustering of rapsyn are not well defined. To analyze the process of cluster formation by rapsyn we examined the formation of rapsyn clusters and complexes using mutant constructs specifically deleted for individual domains of rapsyn in the presence and absence of tagged, full-length rapsyn. Specific deletions of the tetratricopeptide repeat (TPR) domains 1 and 3 of rapsyn abrogated not only clustering of mutant rapsyns, but also, in a dominant negative fashion, the clustering of tagged, full-length rapsyn. We also analyzed rapsyn protein complexes isolated from cells transfected with tagged and untagged rapsyn. Our results show that both tagged and untagged rapsyn are present in immunoprecipitates of rapsyn from cotransfected cells, demonstrating that rapsyn molecules interact directly or indirectly to form oligomers. Mutants that were dominant negatives were also present in complexes containing tagged, full-length rapsyn. Together these results indicate that rapsyn forms clusters at the synapse by oligomerization, and suggest models for the mechanistic bases of this oligomerization via interactions mediated by TPRs 1 and 3. 相似文献
38.
A human recessive neurosensory nonsyndromic hearing impairment locus is a potential homologue of the murine deafness (dn) locus 总被引:1,自引:1,他引:1
Jain Pawan K.; Fukushima Kunihiro; Deshmukh Dilip; Ramesh Arabandi; Thomas Elizabeth; Lalwani Anil K.; Kumar Subrinder; Ploplis Barbara; Skarka Hana; Srisailapathy C.R.Srikumari; Wayne Sigrid; Zbar Ross I.S.; Verma Ishwar C.; Smith Richard J.H.; Wilcox Edward R. 《Human molecular genetics》1995,4(12):2391-2394
A locus for recessive neurosensory nonsyndromic hearing impairmentmaps to chromosome 9q13q21 in two regionally separateconsanguineous families from India. Each family demonstratesa LOD score greater than 4.5 to this region. D9S15, tightlylinked to the Friedreich's ataxia locus, a region that has beendefined with over 1 Mb of YAC contig information and severalexpressed sequences, is one of the flanking markers. In mice,the deafness (dn) locus maps to mouse chromosome 19 and flankingloci are syntenic to human chromosome 9q11q21. The dnmouse is a potential model for the hearing impairment foundin both these families. 相似文献
39.
40.
Intranasal hormone replacement therapy 总被引:3,自引:0,他引:3
Although the optimal route of delivery for hormone replacement therapy has not yet been determined, desirable qualities would include good efficacy, easy administration, minimal side effects, and optimal therapeutic profile. This would potentially serve to improve patient compliance and satisfaction. The intranasal route has been evaluated for the administration of menopausal hormones and seems to fulfill these requirements. The intranasal route would also seem to be a viable alternative for drugs that are poorly absorbed after ingestion by avoiding hepatic first-pass elimination. The intranasal route is, therefore, innovative for the delivery of natural sex steroids in postmenopausal women receiving hormone replacement therapy. Early studies demonstrate that it is safe, effective, and acceptable to postmenopausal women. In addition, the nasal administration of a combination of estradiol and progesterone would seem to be an attractive way to deliver hormones to nonhysterectomized postmenopausal women. Providing alternative routes of administration may also enhance compliance. 相似文献