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Literature DB >> 1728552

Abnormal length of cilia--a cause of primary ciliary dyskinesia--a case report.

B Niggemann¹, A Müller, A Nolte, N Schnoy, U Wahn.

Abstract

A 7-year-old Turkish boy had suffered from chronic coughing from early childhood. Severe bronchiectasis in the right lung was confirmed by bronchography. Ciliary beat frequency determined in a bronchial mucosal biopsy was markedly decreased (5.7 Hz). Electron microscopy revealed cilia with a length of 15 microns. No structural abnormality was found. A possible link between the abnormally long, slow beating cilia and the clinical symptoms is discussed.

Entities: Disease Species

Mesh：

Year: 1992 PMID： 1728552 DOI： 10.1007/bf02073899

Source DB: PubMed Journal: Eur J Pediatr ISSN： 0340-6199 Impact factor: 3.183

13 in total

Review 1. Primary ciliary dyskinesia: evaluation and management.

Authors: C M Rossman; M T Newhouse
Journal: Pediatr Pulmonol Date: 1988

2. Ciliary abnormalities in respiratory disease.

Authors: R M Buchdahl; J Reiser; D Ingram; A Rutman; P J Cole; J O Warner
Journal: Arch Dis Child Date: 1988-03 Impact factor: 3.791

3. The saccharin method for testing mucociliary function in patients suspected of having primary ciliary dyskinesia.

Authors: M Canciani; E G Barlocco; G Mastella; M M de Santi; C Gardi; G Lungarella
Journal: Pediatr Pulmonol Date: 1988

Review 4. Ciliary dyskinesia and ultrastructural abnormalities in respiratory disease.

Authors:
Journal: Lancet Date: 1988-06-18 Impact factor: 79.321

5. Ciliary dyskinesia with normal ultrastructure.

Authors: M A Greenstone; A Dewar; P J Cole
Journal: Thorax Date: 1983-11 Impact factor: 9.139

Review 6. [How useful is the ultrastructural study of the cilia of the respiratory tract in the diagnosis of an immotile cilia syndrome?].

Authors: L Fischer; P H Burri; W Bauer; R Kraemer; K Sauter
Journal: Schweiz Med Wochenschr Date: 1984-05-05

7. Abnormal length of cilia as a possible cause of defective mucociliary clearance.

Authors: B A Afzelius; G Gargani; C Romano
Journal: Eur J Respir Dis Date: 1985-03

8. Freeze-fracture analysis of the respiratory cilia from the bronchial mucosa of a patient with primary ciliary dyskinesia.

Authors: U Lessner; W Breipohl; N Konietzko; B Rehn
Journal: Respiration Date: 1988 Impact factor: 3.580

9. Nasal mucociliary clearance and ciliary beat frequency in cystic fibrosis compared with sinusitis and bronchiectasis.

Authors: J Rutland; P J Cole
Journal: Thorax Date: 1981-09 Impact factor: 9.139

10. Primary ciliary dyskinesia: cytological and clinical features.

Authors: M Greenstone; A Rutman; A Dewar; I Mackay; P J Cole
Journal: Q J Med Date: 1988-05

8 in total

1. Target-of-rapamycin complex 1 (Torc1) signaling modulates cilia size and function through protein synthesis regulation.

Authors: Shiaulou Yuan; Jade Li; Dennis R Diener; Michael A Choma; Joel L Rosenbaum; Zhaoxia Sun
Journal: Proc Natl Acad Sci U S A Date: 2012-01-23 Impact factor: 11.205

Abnormal length of cilia--a cause of primary ciliary dyskinesia--a case report.

Review 1. Primary ciliary dyskinesia: evaluation and management.

2. Ciliary abnormalities in respiratory disease.

3. The saccharin method for testing mucociliary function in patients suspected of having primary ciliary dyskinesia.

Review 4. Ciliary dyskinesia and ultrastructural abnormalities in respiratory disease.

5. Ciliary dyskinesia with normal ultrastructure.

Review 6. [How useful is the ultrastructural study of the cilia of the respiratory tract in the diagnosis of an immotile cilia syndrome?].

7. Abnormal length of cilia as a possible cause of defective mucociliary clearance.

8. Freeze-fracture analysis of the respiratory cilia from the bronchial mucosa of a patient with primary ciliary dyskinesia.

9. Nasal mucociliary clearance and ciliary beat frequency in cystic fibrosis compared with sinusitis and bronchiectasis.

10. Primary ciliary dyskinesia: cytological and clinical features.

1. Target-of-rapamycin complex 1 (Torc1) signaling modulates cilia size and function through protein synthesis regulation.

2. Kartagener syndrome: an uncommon cause of neonatal respiratory distress?

3. A role for primary cilia in aortic valve development and disease.

4. Regulation of flagellar assembly by glycogen synthase kinase 3 in Chlamydomonas reinhardtii.

5. A CDK-related kinase regulates the length and assembly of flagella in Chlamydomonas.

6. Flagellar Synchronization Is a Simple Alternative to Cell Cycle Synchronization for Ciliary and Flagellar Studies.

7. Defective flagellar assembly and length regulation in LF3 null mutants in Chlamydomonas.

8. Regulation of cilium length and intraflagellar transport by the RCK-kinases ICK and MOK in renal epithelial cells.