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排序方式: 共有522条查询结果,搜索用时 0 毫秒
91.
N Mohebbi R Vargas‐Poussou SCA Hegemann B Schuknecht AD Kistler RP Wüthrich CA Wagner 《Clinical genetics》2013,83(3):274-278
Mohebbi N, Vargas‐Poussou R, Hegemann SCA, Schuknecht B, Kistler AD, Wüthrich RP, Wagner CA. Homozygous and compound heterozygous mutations in the ATP6V1B1 gene in patients with renal tubular acidosis and sensorineural hearing loss. Distal renal tubular acidosis (dRTA) is characterized by the inability to excrete acid in the renal collecting ducts resulting in inappropriately alkaline urine and hyperchloremic (normal anion gap) metabolic acidosis in the context of a normal (or near‐normal) glomerular filtration rate. Inborn dRTA can be due to autosomal dominant or recessive gene defects. Clinical symptoms vary from mild acidosis, incidental detection of kidney stones or renal tract calcification to severe findings such as failure to thrive, severe metabolic acidosis, and nephrocalcinosis. The majority of patients with recessive dRTA present with sensorineural hearing loss (SNHL). Few cases with abnormal widening of the vestibular aqueduct have been described with dRTA. Mutations in three different genes have been identified, namely SLC4A1, ATP6V1B1, and ATP6V0A4. Patients with mutations in the ATP6V1B1 proton pump subunit develop dRTA and in most of the cases sensorineural hearing loss early in childhood. We present two patients from two different and non‐consanguineous families with dRTA and SNHL. Direct sequencing of the ATP6V1B1 gene revealed that one patient harbors two homozygous mutations and the other one is a compound heterozygous. To our knowledge, this is the first case in the literature describing homozygosity in the same dRTA gene on both alleles. 相似文献
92.
NUNO DIAS FERREIRA M.D. DANIEL CAEIRO M.D. LUÍS ADÃO M.D. MARCO OLIVEIRA M.D. HELENA GONÇALVES M.D. JOSÉ RIBEIRO M.D. MADALENA TEIXEIRA M.D. ANÍBAL ALBUQUERQUE M.D. JOÃO PRIMO M.D. PEDRO BRAGA M.D. LINO SIMÕES M.D. VASCO GAMA RIBEIRO M.D. 《Pacing and clinical electrophysiology : PACE》2010,33(11):1364-1372
Background: Previous reports have suggested the occurrence of cardiac conduction disorders and permanent pacemaker (PPM) requirement after transcatheter aortic valve implantation (TAVI). Based on a single‐center experience, we aim to assess the incidence of postprocedural conduction disorders, need for PPM, and its determinants after TAVI with a self‐expanding bioprosthesis. Methods: From August 2007 to October 2009, 32 consecutive patients underwent TAVI with the Medtronic CoreValve (MCV) System (Medtronic Inc., Minneapolis, MN, USA). Three patients paced at baseline and two cases of procedure‐related mortality were excluded. We analyzed the 12‐lead electrocardiogram at baseline, immediately after procedure and at discharge. Requirements for PPM were documented and potential clinical, electrophysiological, echocardiographic, and procedural predictors of PPM requirement were studied. Results: After TAVI, eight patients (29.6%) required PPM implantation due to high‐grade atrioventricular (AV) block. The prevalence of left bundle branch block increased from 13.8% to 57.7% directly after implantation (P = 0.001). Need for PPM was correlated to the depth of prosthesis implantation (r = 0.590; P = 0.001). At a cutoff point of 10.1 mm, the likelihood of pacemaker could be predicted with 87.5% sensitivity and 74% specificity and a receiver operator characteristic curve area of 0.86 ± 0.07 (P = 0.003). Of the seven patients with preexisting right bundle branch block (RBBB), four (57.1%) required PPM implantation after TAVI. Conclusions: High‐grade AV block requiring PPM implantation is a common complication following TAVI and could be predicted by a deeper implantation of the prosthesis. Patients with preexisting RBBB also seem to be at risk for the development of high‐grade AV block and subsequent pacemaker implantation. (PACE 2010; 1364–1372) 相似文献
93.
Aaron E Boyce Gillian Marshman Richard AD Mills 《The Australasian journal of dermatology》2009,50(3):190-193
Erosive mucosal lichen planus (LP) is a well-established variant of LP characterized by the formation of ulcerative lesions predominantly involving the oral and genital mucosae. Less commonly, this condition may involve oesophageal and/or ocular mucosal surfaces, and case reports within the ophthalmology literature have recently confirmed the potential for this condition to affect the nasolacrimal ducts. We report the case of a woman with severe cicatrizing mucosal LP and ocular symptoms secondary to presumed nasolacrimal duct involvement. We also report the potential for this newly appreciated manifestation of LP to respond to systemic cyclosporin A. 相似文献
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96.
采用RITQ综合评定婴儿气质的报告 总被引:2,自引:0,他引:2
张琪 《中国心理卫生杂志》1997,11(3):155-157
采用Carey的RITQ为4-8个月婴儿气质的测查工具,结合专人问卷与观察,对400名婴儿的气质进行了综合评定,结果表明:九个气质因子的重测信度为0.77-0.90,多数气质因子间存在着显著的相关,相关系数为0.10-0.59。大、小婴儿活动水平、接近-退缩、适应及反应强度四个气质因子存在差异。气质类型与国外同龄婴儿及国内学前儿童的分布相接近。提示采用RITQ综合评定婴儿气质的信度和效度较好,可用于我国婴儿的气质测查及研究。 相似文献
97.
Safety of outpatient peripheral angioplasty 总被引:2,自引:0,他引:2
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99.
Summary— Beta-adrenergic receptors (β/-AR) belong to the large multigenic family of receptors coupled to GTP-binding proteins. Three subtypes have been identified: β1 -, β2 - and β3 -AR. Much of the work delineating the precise pharmacological comparison of the three β-ARs has come from investigations with stably transfected Chinese hamster ovary cells (CHO cells). This review discusses the structure and function of β3 -AR in various species and presents new findings on a number of β3 -AR ligands including carazolol, tertatolol and CL 316,243 which were found to be selective and potent β3 -AR agonists and ZD 2079 and salmeterol which appear to display full but non-subtype selective agonistic activity. Species-related variations of the β3 -AR pharmacology have been shown for propranolol and bupranolol. With the ongoing characterization of the β3 -AR at the molecular and cellular level, and with the advent of computer-assisted molecular modelling to aid in the determination of the three-dimensional structure of the receptor, it is thought that novel β3 -AR compounds will become available with improved selectivity and potency. 相似文献
100.
IM Balfour-Lynn B Valman M Silverman AD Webster 《Archives of disease in childhood》1993,68(4):472-476
It is unknown why some infants wheeze during upper respiratory tract infections. One possibility is that secretory IgA, which has a major role in mucosal defence against viral infection, might be deficient in wheezy infants. The nasal IgA response to upper respiratory tract infection in 32 wheezy infants (median age 5.8 months) was compared with nine siblings (median age 2.6 years) who had nasal symptoms only. Nasal lavage was performed during infections and on follow up when free from symptoms, using inulin as a marker of dilution to determine absolute concentrations of IgA in the nasal secretions. The two groups showed a similar increase in total IgA and total protein levels during infection, but secretory IgA concentrations were unchanged. This study shows that wheezy infants have a normal nasal IgA response to infection and that the increase in total IgA during early infection is due to plasma exudation rather than increased production of secretory IgA. 相似文献