Zur Pathogenese der Bronchiektasien

Kartagener, M.

doi:10.1007/bf02143627

Cited by 20 publications

(12 citation statements)

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“…The triad of chronic sinusitis/rhinitis, bronchiectasis and situs inversus is known as Kartagener's syndrome in humans (Kartagener 1933). In about 50 per cent of the cases described in humans and dogs, PCD is combined with situs inversus totalis or isolated dextrocardia (Afielius 1981, Sturgess andothers 1986).…”

Section: Discussionmentioning

confidence: 99%

Primary ciliary dyskinesia with situs inversus totalis, hydrocephalus internus and cardiac malformations in a dog

Reichler

Hoerauf

Guscetti

et al. 2001

J of Small Animal Practice

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A nine-month-old golden retriever bitch was presented with exercise intolerance and recurrent nasal discharge. Based on clinical, radiographic and ultrasonographic examination, a diagnosis of rhinitis, situs inversus totalis and tricuspid valve insufficiency was established. The results of video- and electron microscopy studies of the respiratory epithelium were compatible with primary ciliary dyskinesia (PCD). However, no evidence of a primary ultrastructural defect of the cilia was found. The dog was euthanased because of the poor prognosis. At necropsy, a hydrocephalus internus and a subaortic stenosis were additionally diagnosed. PCD, in combination with situs inversus, has been previously reported in golden retrievers, but without a concomitant hydrocephalus internus. Furthermore, concomitant occurrence of internal cardiac malformation and PCD has not previously been reported in the dog.

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Section: Discussionmentioning

confidence: 99%

Primary ciliary dyskinesia with situs inversus totalis, hydrocephalus internus and cardiac malformations in a dog

Reichler

Hoerauf

Guscetti

et al. 2001

J of Small Animal Practice

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“…It is only relatively recently that the mechanisms involved in the initial establishment of left-right asymmetry have begun to be understood. Kartagener (1933), amongst others, identified a triad of conditions that included respiratory problems, male infertility and situs inversus, the lateral transposition of internal organs (figure 1b). For a detailed historical review see also Berdon & Willi (2004) and .…”

Section: Introductionmentioning

confidence: 99%

Symmetry breaking cilia-driven flow in the zebrafish embryo

et al. 2012

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Fluid mechanics plays a vital role in early vertebrate embryo development, an example being the establishment of left-right asymmetry. Following the dorsal-ventral and anterior-posterior axes, the left-right axis is the last to be established; in several species it has been shown that an important process involved with this is the production of a left-right asymmetric flow driven by 'whirling' cilia. It has previously been established in experimental and mathematical models of the mouse ventral node that the combination of a consistent rotational direction and posterior tilt creates left-right asymmetric flow. The zebrafish organising structure, Kupffer's vesicle, has a more complex internal arrangement of cilia than the mouse ventral node; experimental studies show the flow exhibits an anticlockwise rotational motion when viewing the embryo from the dorsal roof, looking in the ventral direction. Reports of the arrangement and configuration of cilia suggest two possible mechanisms for the generation of this flow from existing axis information: (1) posterior tilt combined with increased cilia density on the dorsal roof, and (2) dorsal tilt of 'equatorial' cilia. We develop a mathematical model of symmetry breaking cilia-driven flow in Kupffer's vesicle using the regularized Stokeslet boundary element method. Computations of the flow produced by tilted whirling cilia in an enclosed domain suggest that a possible mechanism capable of producing the flow field with qualitative and quantitative features closest to those observed experimentally is a combination of posteriorly tilted roof and floor cilia, and dorsally tilted equatorial cilia.

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“…Defective function of embryonic nodal cilia leads to apparent randomisation of left-right axis development, with situs inversus found in almost 50% of PCD patients (31) . PCD with situs inversus is classically known as Kartagener's syndrome, although Siewert first described the association in 1904 (32,33) . Some patients have situs ambiguous or heterotaxy, where organs are in mirrored…”

Section: Primary Ciliary Dyskinesiamentioning

confidence: 99%