نتایج جستجو برای: primary ciliary dyskinesia

تعداد نتایج: 653356  

Journal: :Iranian journal of kidney diseases 2013
Ali Momeni Behzad Doroushi Nadia Taheri

Primary ciliary dyskinesia is characterized by congenital impairment of mucociliary clearance. Kartagener syndrome (KS) is a clinical variant of primary ciliary dyskinesia which is involved in situs inversus associated with chronic respiratory infections. In addition, glomerular disease in KS syndrome is rare and reported cases are limited. We had a 27-year-old female patient with KS who presen...

Journal: :Journal of applied genetics 2004
Maciej Geremek Michał Witt

Primary ciliary dyskinesia (PCD) is a multisystem disease characterized by recurrent respiratory tract infections, sinusitis, bronchiectasis and male subfertility, associated in about 50% patients with situs inversus totalis (the Kartagener syndrome). The disease phenotype is caused by ultrastructural defects of respiratory cilia and sperm tails. PCD is a heterogenetic disorder, usually inherit...

2005
R DE IONGH J RUTLAND

A reproducible technique, utilising a graphics tablet and a personal computer for measuring ciliary orientation from electron micrographs of ciliated epithelium, was assessed. Ciliary deviation was measured in 47 normal subjects (mean ciliary deviation ± 1 SD was 14-6 (3.30)), in eight patients with bronchiectasis and normal ciliary function (15.1 (6 50)), and in seven patients with primary cil...

Journal: :Journal of clinical pathology 1989
R De Iongh J Rutland

A reproducible technique, utilising a graphics tablet and a personal computer for measuring ciliary orientation from electron micrographs of ciliated epithelium, was assessed. Ciliary deviation was measured in 47 normal subjects (mean ciliary deviation +/- 1 SD was 14.6 (3.3 degrees)), in eight patients with bronchiectasis and normal ciliary function (15.1 (6.5 degrees], and in seven patients w...

Journal: :The European respiratory journal 1999
B Karadag A J James E Gültekin N M Wilson A Bush

Exhaled nitric oxide can be detected in exhaled air and is readily measured by chemiluminescence. It is thought to be involved in both the regulation of ciliary motility and host defence. Recently, upper airway NO has been found to be reduced in a small number of children with primary ciliary dyskinesia (PCD) and its measurement has been recommended as a diagnostic test for this condition. The ...

Journal: :The European respiratory journal 1999
H Grasemann S S Gärtig H G Wiesemann H Teschler N Konietzko F Ratjen

Airway nitric oxide concentrations in patients with cystic fibrosis or primary ciliary dyskinesia syndrome have been shown to be lower than in healthy subjects. Decreased NO concentrations may contribute to impaired ciliary clearance, respiratory tract infections, or obstructive lung disease in these conditions. Nasal and exhaled NO concentrations were compared before and after infusion of 500 ...

Journal: :The European respiratory journal 2017
Jane S Lucas Angelo Barbato Samuel A Collins Myrofora Goutaki Laura Behan Daan Caudri Sharon Dell Ernst Eber Estelle Escudier Robert A Hirst Claire Hogg Mark Jorissen Philipp Latzin Marie Legendre Margaret W Leigh Fabio Midulla Kim G Nielsen Heymut Omran Jean-Francois Papon Petr Pohunek Beatrice Redfern David Rigau Bernhard Rindlisbacher Francesca Santamaria Amelia Shoemark Deborah Snijders Thomy Tonia Andrea Titieni Woolf T Walker Claudius Werner Andrew Bush Claudia E Kuehni

The diagnosis of primary ciliary dyskinesia is often confirmed with standard, albeit complex and expensive, tests. In many cases, however, the diagnosis remains difficult despite the array of sophisticated diagnostic tests. There is no "gold standard" reference test. Hence, a Task Force supported by the European Respiratory Society has developed this guideline to provide evidence-based recommen...

Journal: :Thorax 1983
M A Greenstone A Dewar P J Cole

There is increasing awareness of the association between primary ciliary abnormalities and bronchiectasis. The most frequently recognised is that between dynein deficient cilia and Kartageneres syndrome.' Afzelius, however, predicted that without embryonic ciliary movement the rotation of the archenteron would be random and that in half of the cases of the "immotile cilia syndrome" there would ...

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