Page 2495 - Cote clinical veterinary advisor dogs and cats 4th

1244 Immunodeficiency Syndromes: Congenital

Immunodeficiency Syndromes: Congenital*
VetBooks.ir Disease (Synonyms) Inheritance † Breeds Defect Characterization

Barrier Defects
Ciliary dyskinesia (immotile cilia AR Many breeds † Functional and Rhinosinusitis, bronchopneumonia with
syndrome) morphologic cilia bronchiectasis, situs inversus
abnormalities
CDC39
Neutrophil Defects
Bactericidal neutrophil defect U Doberman pinscher Unknown Upper respiratory infections, reduced
bactericidal activity, ciliary dyskinesia not
excluded
Trapped neutrophil syndrome AR Border collie Unknown Fever, neutropenia (despite myeloid
hyperplasia), failure to thrive, and
sometimes lameness; genetic test available
Cyclic hematopoiesis (cyclic AR Collie (gray), basset hound Hematopoietic growth Severe neutropenia every 12-14 days, reactive
neutropenia) factors defect amyloidosis, DNA-mutation test
Chronic idiopathic neutropenia U Rottweiler G-CSF deficiency Recurrent fever, sepsis, polyarthritis, pyoderma
Leukocyte adhesion deficiency AR Irish setter, red and white setter, CD11/18 deficiency, Severe leukocytosis, infection with limited
domestic medium hair cat beta-chain (CD18) pus formation, lack of neutrophil adhesion,
deficiency DNA-mutation test
Pelger-Huët anomaly AD Australian shepherd, foxhound, Unknown No immunodeficiency, hyposegmented
Persian cats, others granulocytes
Leukocyte granulation U Birman cats Unknown No immunodeficiency, acidophilic granules
Complement component 3 AR Brittany spaniel C3 deficiency Pyogenic infections, lack of C-mediated
deficiency (C3 deficiency) phagocytosis in colony of dogs with
neuromuscular disease
Chédiak-Higashi syndrome AR Persian cats Unknown No immunodeficiency, large granules in
phagocytes, bleeding tendency
Reactive (AA) amyloidosis U Abyssinian cats Unknown Reactive (AA) amyloidosis, renal failure
Increased susceptibility to AR Dachshund, Cavalier King Unknown Pneumocystis pneumonia
Pneumocystis pneumonia Charles spaniel
Susceptibility to fungal and U German shepherd Possible macrophage or Severe ehrlichiosis, Rocky Mountain spotted
rickettsial infections; T-cell defect fever, disseminated aspergillosis, deep
pyoderma pyoderma
Immune vasculitis, amyloidosis U Shar-pei, Abyssinian cats, Elevated IL-6 Arthritis, amyloidosis, kidney injury/
Oriental shorthair cats chronic kidney disease, hepatic rupture,
hypoproteinemia
Lethal acrodermatitis AR Bull terrier Zinc metabolism defect Zinc deficiency, hyperkeratosis
Increased susceptibility to U Rottweiler, Doberman pinscher Unknown Parvovirus infection
parvoviral infection
Vaccine-exacerbated immune U Akita, Weimaraner Unknown Variable meningitis, polyarthritis, amyloidosis
disturbance
Humoral Immune Defects
X-linked severe combined XR Basset hound, Cardigan Welsh Common gamma-chain Severe bacterial and viral infections, no IgG
immunodeficiency (X-SCID) corgi of IL-2 and other and IgA, deficient lymphocyte blastogenesis,
cytokines DNA-mutation test
SCID AR Jack Russell terrier DNA-protein kinase Severe serum immunoglobulin deficiency,
catalytic subunit hypoplasia of lymphoid tissues, DNA-
mutation test
SCID AR Frisian water dog RAG1 gene defect alters Severe lgM, lgG, and lgA deficiency and
antigen-recognition lymphopenia, DNA mutation test
Combined immunodeficiency U Shar-pei T-cell and B-cell defects, Skin, respiratory, and gastrointestinal infections
low IL-6 and IL-2
Thymic abnormalities and U Weimaraner Unknown Reduced growth, thymosine responsive
dwarfism
*In addition to the listed syndromes, bone marrow dyscrasias have been described in miniature and toy poodles and transient hypogammaglobulinemia in Samoyeds, but less is known of these disorders.
† For example, springer spaniel, Old English sheepdog, English setter, West Highland white terrier, pointer.
AD, Autosomal dominant; AR, autosomal recessive; G-CSF, granulocyte colony-stimulating factor; IL, interleukin; U, unknown; XR, X-linked recessive.
Modified from Greene CE: Infectious diseases of the dog and cat, ed 4, St. Louis, 2012, Saunders.

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