Clinical, morphologic, and biochemical characteristics of Chediak-Higashi syndrome in fifty-six Japanese black cattle

Am J Vet Res. 1997 Nov;58(11):1221-6.

Abstract

Objective: To characterize Chediak-Higashi syndrome (C-HS) in Japanese Black cattle.

Animals: 56 of 200 cattle with a bleeding disorder and giant granules in leukocytes.

Procedure: Clinical observation, CBC, hemostatic screening test, platelet aggregometry, electron microscopy, platelet constituent analysis, and ophthalmoscopic examination were done.

Results: Affected Japanese Black cattle had increased bleeding tendency and abnormal granules in their leukocytes. Susceptibility to infection was not increased. Cutaneous albinism was evident in 6 new-born calves, but not in most affected cattle. In all affected cattle, the tapetal fundus was pale and the nontapetal fundus was almost devoid of pigment. By electron microscopy, a remarkable decrease in the number of dense granules in platelets was observed. Functionally, collagen-induced platelet aggregation was markedly reduced.

Conclusions: This bleeding disorder was diagnosed as C-HS. With regard to susceptibility to infection, albinism, and mortality, clinical manifestations of C-HS in Japanese Black cattle were moderate, compared with C-HS in human beings and Hereford cattle.

Clinical relevance: Because an autosomal recessive mode of inheritance was documented and recessive homozygotes could be easily detected, C-HS in Japanese Black cattle can be controlled.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adenosine Diphosphate / pharmacology
  • Animals
  • Blood Platelets / drug effects
  • Blood Platelets / pathology
  • Blood Platelets / physiology
  • Cattle
  • Cattle Diseases / blood
  • Cattle Diseases / diagnosis*
  • Cattle Diseases / physiopathology
  • Chediak-Higashi Syndrome / diagnosis
  • Chediak-Higashi Syndrome / epidemiology
  • Chediak-Higashi Syndrome / veterinary*
  • Collagen / pharmacology
  • Cytoplasmic Granules / ultrastructure
  • Female
  • Fundus Oculi
  • Hemostasis
  • Homozygote
  • Japan / epidemiology
  • Leukocyte Count / veterinary
  • Male
  • Microscopy, Electron / methods
  • Microscopy, Electron / veterinary
  • Ophthalmoscopy / methods
  • Ophthalmoscopy / veterinary
  • Platelet Aggregation / drug effects
  • Platelet Count / drug effects
  • Platelet Count / veterinary

Substances

  • Adenosine Diphosphate
  • Collagen