Literature DB >> 2798216

Congenital ciliary aplasia in two siblings. A primitive disregulation of ciliogenesis?

S Richard1, C Nezelof, A Pfister, J de Blic, P Scheinmann, J Paupe.   

Abstract

Congenital ciliary aplasia was demonstrated in two siblings with clinical history of primary ciliary dyskinesia. Ultrastructural histochemistry of successive bronchial biopsies revealed the predominance of immature mucous cells and the total absence of ciliated or preciliated cells in the respiratory epithelium. This original disorder may represent a unique variant of primary ciliary dyskinesia with primitive disregulation of ciliogenesis.

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Year:  1989        PMID： 2798216     DOI： 10.1016/S0344-0338(89)80248-1

Source DB:  PubMed          Journal:  Pathol Res Pract        ISSN： 0344-0338            Impact factor:   3.250


  5 in total

Review 1.  Genetics and biology of primary ciliary dyskinesia.

Authors:  Amjad Horani; Thomas W Ferkol; Susan K Dutcher; Steven L Brody
Journal:  Paediatr Respir Rev       Date:  2015-09-11       Impact factor: 2.726

2.  Familial nasal acilia syndrome.

Authors:  R Soferman; Z Ne'man; M Livne; A Avital; Z Spirer
Journal:  Arch Dis Child       Date:  1996-07       Impact factor: 3.791

Review 3.  The development and functions of multiciliated epithelia.

Authors:  Nathalie Spassky; Alice Meunier
Journal:  Nat Rev Mol Cell Biol       Date:  2017-04-12       Impact factor: 94.444

4.  Primary Ciliary Dyskinesia (PCD): A genetic disorder of motile cilia.

Authors:  Margaret W Leigh; Amjad Horani; BreAnna Kinghorn; Michael G O'Connor; Maimoona A Zariwala; Michael R Knowles
Journal:  Transl Sci Rare Dis       Date:  2019-07-04

5.  Immotile cilia syndrome in pigs. A model for human disease.

Authors:  F Roperto; P Galati; P Rossacco
Journal:  Am J Pathol       Date:  1993-08       Impact factor: 4.307
  5 in total

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