Shar-Pei Dogs Research Articles

Suspeita de febre familiar dos cães Shar-peis chineses

A febre (síndrome) familiar dos cães Shar-peis Chineses é uma doença hereditária autossômica recessiva. Este trabalho descreve um caso de amiloidose generalizada em um cão Shar-pei Chinês, fêmea, de três anos de idade, cujo quadro clínico-patológico é consistente com o da febre familiar dos cães Shar-peis chineses. Esse animal tinha uma história clínica de edema bilateral recidivante na região do jarrete que vinha sendo observado desde os sete meses de idade. Três dias antes da morte, esse cão apresentou anorexia, apatia, vômito e diarréia. À necropsia, ambos os rins estavam firmes, pálidos e possuíam a superfície capsular irregular. Histologicamente foram observados depósitos de amilóide nos rins, no pâncreas, na tireóide e no baço. Esse amilóide perdeu sua afinidade para o vermelho Congo, quando utilizado o permanganato de potássio. Esse achado é consistente com amiloidose do tipo AA.

Light‐microscopy evaluation of zonular fiber morphology in dogs with glaucoma: secondary to lens displacement

Lens displacement resulting in secondary glaucoma is common in terrier breeds. This study was carried out to evaluate whether light microscopy was useful in detecting abnormal patterns in zonular fiber protein. Eyes from 63 dogs with glaucoma secondary to lens displacement were evaluated for the presence of abnormal zonular fiber morphology using the following stains: hematoxylin and eosin, periodic acid Schiff (PAS), Masson's trichrome and Verhoeff's elastin stains. Two distinct forms of abnormal zonular fiber morphology were recognized and designated as zonular fiber dysplasia (ZFD) and zonular fiber collagenization (ZFC). ZFD protein morphology was characterized by being tightly adherent to the nonpigmented ciliary body epithelium, exhibiting a distinct lamellar and cross-hatched pattern and staining strongly positive with PAS and trichrome stains, and staining negative with elastin stains. ZFD was predominant in terrier breeds (18 of 29) and Shar-Pei dogs (4 of 29). ZFC abnormality was characterized by excessive zonular fiber that was not tightly adherent to the ciliary body epithelium and staining positive with PAS, trichrome (blue for collagen) and elastin stains. Only 7 of 19 dogs with ZFC changes were terrier breeds, and there was no pattern in the breeds affected. Fifteen of the 63 dogs used in the study had normal appearing zonular fibers. The staining pattern in these dogs matched normal controls by staining positive with PAS and Verhoeff's elastin stains and had only minimal positive staining with Masson's trichrome stain. Results suggest that light microscopy is useful in detecting breed-related changes in zonular fiber morphology in cases of glaucoma secondary to lens displacement. These changes may correlate with the presence of abnormal zonular fiber proteins and might be important in the pathogenesis of primary lens displacement in terrier and Shar-Pei dogs.

Quantitative study of 'flame follicles' in skin sections of Shar-pei dogs.

This study determined the frequency of occurrence and the mean number of 'flame follicles' per skin section and assessed their diagnostic significance in cutaneous biopsies of Shar-pei dogs. The number of 'flame follicles' per section was recorded in skin sections from 42 Shar-pei dogs, of which 40 had non-neoplastic skin disease and non-atrophic dermatoses and 2 had healthy skin. Forty-two skin sections from dogs of different breeds served as control specimens, 28 of which were examples of non-neoplastic and non-atrophic dermatoses and 14 were from dogs with healthy skin. Differences among groups were analysed by means of the unpaired Student's t-test. It was concluded that 'flame follicles' were more frequent and found in significantly higher numbers in the Shar-pei group when compared with the control group suggesting that 'flame follicles' in skin sections from Shar-pei dogs do not have the same diagnostic significance as in other breeds.

A new disorder of hyaluronan metabolism associated generalized folding and thickening of the skin

Objective: To describe and characterize a new disorder of hyaluronan metabolism associated with marked abnormalities of cutaneous tissue and to determine whether a relationship with a phenotypically similar disorder in the shar-pei dog exists.Methods: Biopsy specimens of the skin of a child with extreme cutaneous thickening and folding were examined by light and electron microscopy. The concentration of hyaluronan and the activity of hyaluronidase were measured in the patient's serum and plasma, respectively, and the activity of hyaluronan synthase was examined in cultured dermal fibroblasts. Hyaluronan concentration was also measured in the plasma of 23 shar-pei and 34 control dogs.Results: The patient's skin displayed gross accumulation of hyaluronan, and the serum concentration of hyaluronan was markedly elevated (up to 3100 μg/L) during infancy. Hyaluronan synthase activity of cultured dermal fibroblasts was increased, whereas hyaluronidase activity in plasma was normal (5.5 ± 0.08 IU/L). Plasma hyaluronan concentration was higher in the shar-pei dogs than in control dogs (median, 378 μg/L vs 73 μg/L, respectively).Conclusion: The child we describe has a novel disorder of hyaluronan metabolism, which appears to result from abnormal control of hyaluronan synthesis. An analogous disorder may be present in the shar-pei dog.

A Primary Immunodeficiency Syndrome in Shar-Pei Dogs

A multiple immunodeficiency, involving antibody- and cell-mediated responses in 10 Chinese Shar-Pei (CSP) dogs is described. Abnormal levels of serum IgM and IgA in most cases, and IgG in fewer cases characterized the immunoglobulin deficiencies. Decreased in vitro proliferative responses of pokeweed mitogen (PWM)-stimulated peripheral blood mononuclear cells (PBMC) were found in nine cases. Clinical presentation involved several organ systems and was associated with recurrent infections and malignancy. Sera from affected dogs suppressed PWM-stimulated cell proliferation of affected and normal dogs, but not cultures stimulated with PWM followed by recombinant IL-2 (rIL-2). In vitro supplementation of PBMC cultures with immunomodulatory guanosine analogs (GA) resulted in increased de novo IgG and/or interleukin-6 (IL-6) synthesis. Cells from five immunodeficient dogs showed in vitro evidence of GA- or rIL-2-dependent enhanced immunological responses. Since rIL-2-mediated activation of the IL-2 receptor and GA-mediated immunomodulation are reported to act through protein kinase C (PKC)-independent pathways, it is concluded that the IL-2 receptor is functional in these dogs and that cell activation through alternative pathways may restore immune responses in affected CSP dogs.

AA amyloidosis in Chinese Shar-pei dogs: Immunohistochemical and amino acid sequence analyses

Amyloidosis in Chinese Shar-pei dogs is unique because of certain similarities to familial Mediterranean fever in humans. It is also of interest that renal amyloid deposits in Shar-peis are known to predominate commonly in the medullary interstitium while glomerular amyloidosis is the characteristic clinicopathologic feature in other dog breeds. Our immunohistochemical and biochemical studies confirm that systemic familial amyloidosis occurring in Shar-pei dogs is of the AA-type. The amyloid protein had a blocked N-terminus but deblocking and sequence analysis ofpeptides obtained after cyanogen bromide and BNPS-skctole cleavage provided an amino acid sequence conforming to positions 1 through 91 of dog SAA. The confirmed sequence of Shar-pei protein AA differed from the previously reported dog protein AA sequence by variations present at positions 7 and 17. Specifically, a Ser/Gly variation was present at position 7 and a Leu/Trp variation was not present at position 17 in Shar-pei protein AA (i.e., only ...

Hepatic amyloidosis in two Chinese Shar Pei dogs

Hepatic amyloidosis in two Chinese Shar Pei dogs

Inheritance of renal amyloidosis in Chinese Shar-pei dogs.

Renal amyloidosis (RA) and recurrent fever of unknown origin (RFUO) are characteristics of familial Mediterranean fever (FMF), a human disorder inherited as an autosomal-recessive trait. Although no animal model has been established for FMF, a similar syndrome of RFUO and RA has been reported in Chinese Shar-pei (CSP) dogs. This report addresses two questions: (1) Is RA inherited in CSP dogs? (2) If it is, is it possible to hypothesize the type of inheritance involved? Two studies were conducted to answer these questions: a historical cross-sectional comparison, which included CSP and non-CSP dogs with RA; and a prospective study that included CSP dogs with RA, RFUO, or both. The cross-sectional comparison resulted in an odds ratio of 10 for RA in CSP dogs under 7 years of age. The prospective study of 28 dogs with RA or RFUO identified 20 that had RFUO and RA, three with RA alone, and five with RFUO alone. RFUO preceded RA in all cases with both conditions. The RFUO/RA combination was observed in both sexes. Four dogs with RFUO with or without RA were born to parents that either were alive at age 7 or had died because of conditions other than kidney failure/RA. When one parent was known to express one of these conditions, the prevalence of RA was between 25% and 50% among littermates.(ABSTRACT TRUNCATED AT 250 WORDS)

Congenital esophageal hiatal hernia in the Chinese shar-pei dog.

Esophageal hiatal hernia was diagnosed in 11 young Chinese Shar-Pei dogs between October 1985 and July 1991. The dogs ranged in age from 2 to 11 months and included 3 females and 8 males. The most common clinical signs were regurgitation, vomiting, and hypersalivation. Physical examination was normal in 6 dogs; abnormal physical examination findings in the other 5 dogs included fever, dehydration, hypersalivation, and pulmonary wheezes and crackles. Laboratory evaluation was significant only for neutrophilia in 5 dogs. A diagnosis of hiatal hernia was made on the basis of survey thoracic radiographic and/or barium esophagram findings of displacement of the esophagogastric junction and stomach into the thoracic cavity; the diagnosis was confirmed by surgery in 9 dogs and at necropsy in 2 dogs. Megaesophagus (n = 7), gastroesophageal reflux (n = 4), and esophageal hypomotility (n = 1) were additional findings in some dogs. Aspiration pneumonia was diagnosed in 7 of the dogs. Medical therapies formulated for the therapy of presumed reflux esophagitis generally failed to resolve the clinical signs associated with the hiatal hernia. Hiatal herniae were surgically repaired in 9 of the Shar-Peis by various combinations of diaphragmatic crural apposition, fixation of the esophagus to the diaphragmatic crus (esophagopexy), and left fundic tube gastropexy. Eight of the animals survived surgery, six of which have been asymptomatic since surgery (19 to 36 months). The megaesophagus, esophageal hypomotility, and bronchopneumonia resolved in all of these dogs.

A canine febrile disorder associated with elevated interleukin-6

Familial Mediterranean Fever (FMF) is a human disorder characterized by recurrent fever of unknown origin (RFUO), renal amyloidosis, and evidence of peritonitis, pleuritis, and/or synovitis. This report suggests that Chinese Shar-pei (CSP) dogs suffer from a similar syndrome. CSP dogs with RFUO ( n = 15) showed greater levels of IL-6 in serum than normal controls, hypergammaglobulinemia, and normal or supranormal in vitro lymphocyte blastogenesis in response to mitogen stimulation, when compared to healthy afebrile dogs. In patients 2 years old or older, RFUO was associated with renal failure, renal amyloidosis, and swollen joints. An epidemiological survey of privately owned dogs indicated a RFUO prevalence of 23% in CSP dogs ( n = 132) and 1% in dogs of all breeds ( n = 98). Increased levels of circulating cytokines, such as IL-6, have been shown to influence such processes as the febrile response, antibody production, and the synthesis of amyloid precursors. We propose that CSP dogs with RFUO, renal amyloidosis, and joint inflammation may serve as an animal model of FMF and that the clinical syndrome is associated with elevated levels of circulating IL-6.

IgA deficiency in shar-pei dogs

Two shar-pei puppies examined because of signs of sinopulmonary disease, one of which also had skin disease, had deficient IgA concentrations. Deficient serum IgA concentrations also were confirmed in 30 of 39 (76.9%) clinically normal adult dogs in two colonies of shar-peis. Both courses of disease—sinopulmonary signs and chronic skin disease and a benign clinical course—have been reported in human patients with IgA deficiency. Thus, the shar-pei might be an appropriate model for studying the immunopathology of IgA deficiency in man.