Clinical manifestations of leukocyte adhesion deficiency in cattle: 14 cases (1977-1991)

R. O. Gilbert From the Department of Clinical Sciences, College of Veterinary Medicine, Cornell University, Ithaca, NY 14853-6401 (Gilbert, Rebhun, Kim), and the National Animal Disease Center, Agricultural Research Service, USDA Ames, IA 50010-0070 (Kehrli, Shuster, Ackermann).

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W. C. Rebhun From the Department of Clinical Sciences, College of Veterinary Medicine, Cornell University, Ithaca, NY 14853-6401 (Gilbert, Rebhun, Kim), and the National Animal Disease Center, Agricultural Research Service, USDA Ames, IA 50010-0070 (Kehrli, Shuster, Ackermann).

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C. A. Kim From the Department of Clinical Sciences, College of Veterinary Medicine, Cornell University, Ithaca, NY 14853-6401 (Gilbert, Rebhun, Kim), and the National Animal Disease Center, Agricultural Research Service, USDA Ames, IA 50010-0070 (Kehrli, Shuster, Ackermann).

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M. E. Kehrli Jr. From the Department of Clinical Sciences, College of Veterinary Medicine, Cornell University, Ithaca, NY 14853-6401 (Gilbert, Rebhun, Kim), and the National Animal Disease Center, Agricultural Research Service, USDA Ames, IA 50010-0070 (Kehrli, Shuster, Ackermann).

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D. E. Shuster From the Department of Clinical Sciences, College of Veterinary Medicine, Cornell University, Ithaca, NY 14853-6401 (Gilbert, Rebhun, Kim), and the National Animal Disease Center, Agricultural Research Service, USDA Ames, IA 50010-0070 (Kehrli, Shuster, Ackermann).

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M. R. Ackermann From the Department of Clinical Sciences, College of Veterinary Medicine, Cornell University, Ithaca, NY 14853-6401 (Gilbert, Rebhun, Kim), and the National Animal Disease Center, Agricultural Research Service, USDA Ames, IA 50010-0070 (Kehrli, Shuster, Ackermann).

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Summary:

In Holstein cattle, an inherited disease has been recognized recently in which leukocytes lack surface glycoproteins termed β2 integrins, which are important in cell adhesion processes. This disease is the homologue of leukocyte adhesion deficiency in human beings and has been termed bovine leukocyte adhesion deficiency. The molecular basis of this disease is failure to produce normal CD18. The gene encoding bovine CD18 and its abnormal mutation have been sequenced, allowing specific diagnosis of the condition by DNA amplification by polymerase chain reaction followed by specific endonuclease digestion. This test was applied to formalin-fixed archival tissues from 18 cattle that had been admitted to the veterinary medical teaching hospital between 1975 and 1991 and that had had persistent and severe neutrophilia. Blood samples were collected from 2 additional cattle, and leukocytes from these samples also were tested. Fourteen cattle were confirmed to have been homozygous for the bovine leukocyte adhesion deficiency gene. Cattle with this condition had ranged in age from 2 weeks to 8 months at admission. They typically had had chronic bacterial infections that had failed to respond to or had recurred after conventional treatment. Consistent findings in these cattle included signs of bronchopneumonia, gingivitis, periodontitis, and peripheral lymphadenopathy. Severe neutrophilia, usually without a left shift, was a hallmark of the disease; consistent clinical biochemical findings included hypoalbuminemia, hyperglobulinemia, and hypoglycemia. This disease is important because it mimics common calfhood diseases such as pneumonia and diarrhea, but is ultimately consistently fatal before adulthood.

Summary:

In Holstein cattle, an inherited disease has been recognized recently in which leukocytes lack surface glycoproteins termed β2 integrins, which are important in cell adhesion processes. This disease is the homologue of leukocyte adhesion deficiency in human beings and has been termed bovine leukocyte adhesion deficiency. The molecular basis of this disease is failure to produce normal CD18. The gene encoding bovine CD18 and its abnormal mutation have been sequenced, allowing specific diagnosis of the condition by DNA amplification by polymerase chain reaction followed by specific endonuclease digestion. This test was applied to formalin-fixed archival tissues from 18 cattle that had been admitted to the veterinary medical teaching hospital between 1975 and 1991 and that had had persistent and severe neutrophilia. Blood samples were collected from 2 additional cattle, and leukocytes from these samples also were tested. Fourteen cattle were confirmed to have been homozygous for the bovine leukocyte adhesion deficiency gene. Cattle with this condition had ranged in age from 2 weeks to 8 months at admission. They typically had had chronic bacterial infections that had failed to respond to or had recurred after conventional treatment. Consistent findings in these cattle included signs of bronchopneumonia, gingivitis, periodontitis, and peripheral lymphadenopathy. Severe neutrophilia, usually without a left shift, was a hallmark of the disease; consistent clinical biochemical findings included hypoalbuminemia, hyperglobulinemia, and hypoglycemia. This disease is important because it mimics common calfhood diseases such as pneumonia and diarrhea, but is ultimately consistently fatal before adulthood.

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