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Chronic sino-pulmonary infections and male infertility
    Differential Diagnosis[Top] [Nav]
Young syndrome, cystic fibrosis, immotile cilia syndrome
    Discussion -- not available online[Top] [Nav]
    Update Comments[Top] [Nav]
  • Editorial on the Rutland paper (vide infra). Traces the evolution of nomenclature used in ciliary diseases:
    1. Immotile cilia syndrome -- used until it was realized that cilia could actively beat but still be non-functional;
    2. Dyskinetic or dysmotile cilia -- used until it was realized that cilia can be transiently non-functional after infectious or toxic insult;
    3. Primary ciliary dyskinesia -- the term used today (1990).
    Thus, the authors list the "Big 3" of genetically-caused chronic lung disease in humans as: alpha-1 antitrypsin deficiency, cystic fibrosis, and primary ciliary dyskinesia. The editorialists are not convinced that Rutland's random ciliary orientation is a cause of primary ciliary dyskinesia. [101]
  • A 12 year old boy had 10-12 chest infections per year and purulent secretions in both nasal cavities. He was immunologically normal and his cilia appeared individually to be normal, but cilial orientation was abnormal. That is, cilia in the respiratory tract are normally oriented to beat in a specific direction, enabling effective mucociliary clearance. But, if the cilia are oriented randomly and, therefore, beat in random directions, then clearance of secretions is likely to be much less efficient. [102]
  • "Subclasses IgG2 and IgG4 (often in combination with IgA) are well correlated with upper respiratory tract infections (sinusitis and otitis media) and chronic inflammation that contributes to bronchiectasis." [103]
  • "CVI is a heterogeneous syndrome characterized by hypogammaglobulinemia and recurrent bacterial infections. [It] may occur at any age, but in most patients the onset is in the second or third decade of life.... Clinically, patients with CVI have recurrent bacterial infections of the respiratory tract such as sinusitis, otitis media, bronchitis, and pneumonia." This is not surprising, given Reynolds' statement above that deficiency of IgG2 and IgG4 can lead to chronic respiratory infections. [104]
    Footnotes in Print Edition[Top] [Nav]
    (1a) Young's syndrome. Obstructive azoospermia and chronic sinopulmonary infections.
  Handelsman DJ, Conway AJ, et al.   N Engl J Med 1984 Jan 5;310(1):3-9.  Pubmed+Abstract  Similars
    (1b) McKusick 279000 = Young syndrome.
    (2) "Immotile-cilia" syndrome and ciliary abnormalities induced by infection and injury.
  Afzelius BA.   Am Rev Respir Dis. 1981;124:107-109.
    (-) Inherited Diseases of the Lung.
  Lieberman J. Philadelphia: Saunders, 1988: 87-98.   Amazon.com
Advocates examining the head of the epididymis (looking for a cystic mass) in all men with chronic obstructive pulmonary disease, even those who have fathered a child. The reason is: obstruction of the epididymis may not be complete until years after puberty, and several men with Young syndrome have fathered children relatively early in their lives.
    New References[Top] [Nav]

Cilia syndromes
    101.Ciliary dysmorphology and dysfunction--primary or acquired? [editorial; comment].
  Boat TF, Carson JL.   N Engl J Med 1990 Dec 13;323(24):1700-2.  Pubmed  Similars
    102.Random ciliary orientation. A cause of respiratory tract disease [see comments].
  Rutland J, de Iongh RU.   N Engl J Med 1990 Dec 13;323(24):1681-4.  Pubmed  Similars

Immunology of chronic sinopulmonary infection
    103.Immunoglobulin G and its function in the human respiratory tract.
  Reynolds HY.   Mayo Clin Proc 1988 Feb;63(2):161-74.  Pubmed+Abstract  Similars
    104.NIH conference. New insights into common variable immunodeficiency [see comments].
  Sneller MC, Strober W, et al.   Ann Intern Med 1993 May 1;118(9):720-30.  Pubmed+Abstract  Similars
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    ©1986-2000 John Sotos, MD. All rights reserved.  Last updated 16:34 PDT on July 4, 2000.[Top]

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