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1.
Primary ciliary dyskinesia was diagnosed in three Newfoundland dogs with histories of chronic rhinitis and bronchopneumonia from an early age. Thoracic radiographs of two of them showed severe, dependent bronchopneumonia and right displacement of the cardiac apex but normal positioning of other organs. Histopathological examination of sections of lung from the other dog showed severe bronchopneumonia. A semen sample from one dog had a high percentage of spermatozoa with abnormal tails and poor progressive motility. Transmission electron microscopy of nasal brushings from all three dogs showed consistent ultrastructural defects in the cilia, including an absence of outer and inner dynein arms, disorganisation of peripheral doublets, occasional supernumerary doublets and singlets, and consistently disorganised basal bodies and foot processes; sections of trachea from one dog also had disorganised basal bodies. Pedigree analysis was consistent with a monogenic autosomal recessive pattern of inheritance for the defect. One dog is still alive, one dog died aged five years two months, and one dog was euthanased aged nine months. This is the first time primary ciliary dyskinesia has been reported in Newfoundland dogs.  相似文献   

2.
Compared with neutrophils from healthy dogs, neutrophils from 2 dogs with primary ciliary dyskinesia had increased distance of random migration, but fewer of the neutrophils migrated. The affected dogs had an increase in the numbers of Staphylococcus aureus phagocytized. Lymphocyte blastogenesis in the affected dogs in response to standard mitogens was considered to be normal.  相似文献   

3.
In vitro neutrophil function was assessed in two English Springer Spaniel dogs, two Bichon Frise dogs, and one Chow Chow dog with congenital ciliary dyskinesia; three clinically normal English Springer Spaniel dogs that were presumed heterozygous for congenital ciliary dyskinesia; and five control dogs. Chemotaxis and random migration in affected and heterozygous dogs were found to be comparable to those of control dogs. Increased (P less than or equal to 0.05) neutrophil adhesion, antibody dependent cell-mediated cytotoxicity, iodination of proteins, and oxygen radical production in neutrophils from affected dogs were probably the result of chronic bacterial infection in vivo. Bacterial ingestion by neutrophils from the three heterozygous English Springer Spaniel dogs was significantly increased compared to control dogs but was not different from affected English Springer Spaniel dogs, suggesting a breed-related phenomenon. Significant decreases in neutrophil function were not seen in any of the dogs with congenital ciliary dyskinesia, indicating that a defective microtubular system is not shared by respiratory cilia and neutrophils and that defective neutrophil function does not contribute to respiratory infection.  相似文献   

4.
Primary ciliary dyskinesia is a congenital condition that may cause chronic rhinitis and bronchopneumonia. Primary ciliary dyskinesia may be diagnosed by induction of ciliogenesis by use of in vitro cell culture. Induction of ciliogenesis allows for differentiation between primary and secondary ciliary dyskinesia.  相似文献   

5.
Two Old English Sheepdog litter-mates with chronic respiratory disease since birth had ultrastructural abnormalities of their respiratory tract cilia. The ciliary defects included lack of inner dynein arms, abnormal radial spokes and nexin links, and microtubule doublet displacement. Similar ciliary defects in man result in immotile cilia with the subsequent development of sinusitis, bronchitis or bronchiectasis, male sterility, and, in some cases, situs inversus. Both dogs had sinusitis and bronchiectasis, and one of the dogs also had situs inversus.  相似文献   

6.
This study describes the clinical, immunologic, genetic, and pathologic features of Irish Wolfhounds with rhinitis/bronchopneumonia syndrome. The dogs examined were from Belgium, The Netherlands, UK, Canada, Germany, and Switzerland. Signs included transient to persistent mucoid or mucopurulent rhinorrhea, cough, and respiratory dyspnea. Radiographic, rhinoscopic, and bronchoscopic findings were variable. Analysis of ciliary ultrastructure was performed in 5 affected dogs, but no characteristic primary ciliary defects (primary ciliary dyskinesia) were detected. Serum and bronchoalveolar lavage fluid (BALF) concentrations of IgA, IgG, and IgM were determined in some affected dogs and clinically normal Irish Wolfhounds. Serum IgA concentration was below the reference range in 5 of 8 affected dogs tested, whereas BALF IgA concentration was above the normal range in 2 affected adult dogs. The CD4 to CD8 lymphocyte subset ratio (CD4:CD8) in peripheral blood was tested in 3 affected dogs and was within the normal range. BALF CD4:CD8 was tested in 1 affected dog and was higher than the normal range. Decreased neutrophil phagocytosis was observed in 1 of the 4 dogs tested. Analysis of pedigrees of the Belgian, Canadian, German, and Swiss dogs revealed common ancestry, suggesting a heritable syndrome.  相似文献   

7.
Primary ciliary dyskinesia (PCD) is a diverse group of inherited structural and functional abnormalities of the respiratory and other cilia, which results in recurrent respiratory tract infections. Primary ciliary dyskinesia was diagnosed in a 14-week old Staffordshire bull terrier that had a history of respiratory disease from 7 weeks of age. Pneumonia was diagnosed on thoracic radiographs and transtracheal aspirate. Transmission electron microscopy of the bronchi and trachea indicated the presence of both primary and secondary ciliary dyskinesia. The most prominent primary defects consisted of absent inner dyneim arms, absent radial spokes and absence of the central microtubules. These defects accounted for 62% of the total number of cross-sections screened. Non-specific ciliary abnormalities encountered most often were compound cilia, swollen cilia, addition/deletion of peripheral doublets and disorganised axonemes (26%). To the authors' knowledge, this is the first case of PCD described in the Staffordshire bull terrier and the first report of PCD in South Africa.  相似文献   

8.
A 12-year old male miniature poodle with a chronic progressive cough of two years duration was found to have situs inversus totalis, rhinitis, bronchiectasis and bronchopneumonia with metaplasia. Clinical and pathological findings satisfy the clinical criteria for a diagnosis of Kartagener's syndrome. However, the dog had no history of respiratory disease during it's first year of life, it sired at least one healthy puppy and electron microscopic examination of tracheal cilia showed only minimal microtubular abnormalities. This is the first report of a miniature poodle with Kartagener's syndrome. Kartagener's syndrome and mild forms of primary ciliary dyskinesia (PCD) may play an important role in the development of chronic respiratory disease in older dogs.  相似文献   

9.
The objective of this study was to compare orbital and ocular vasculature velocity, measured by Doppler imaging, in normal Beagles and Beagles with inherited primary open-angle glaucoma. Eight normal Beagles and 13 Beagles with different stages of primary open-angle glaucoma were evaluated twice with a 2–4-week period between measurements. Doppler imaging was performed with the dogs anesthetized, and the Doppler transducer applied directly on the corneal surface. The majority of the orbital vasculature (external ethmoidal artery; internal ophthalmic artery and vein; and external ophthalmic artery and vein) and ocular blood vessels (anterior ciliary artery and veins; long posterior ciliary arteries; short posterior ciliary arteries; primary retinal arteries; and the vortex veins) were identified and Doppler blood velocity parameters were determined. The glaucomatous dogs demonstrated significant differences in the Doppler velocity parameters of several orbital vessels (external ethmoidal, external ophthalmic, and internal ophthalmic arteries), and several ocular vessels (anterior ciliary, short posterior ciliary, and long posterior ciliary arteries). These differences included decreased blood velocities, and increased pulsatility and resistive indexes. The Doppler blood flow velocities of the primary retinal arteries were unchanged between the normal and glaucomatous dogs. In the glaucomatous dogs, the Doppler imaging suggests increased vascular resistance downstream in both the orbital and ocular vasculature. These blood velocity parameter changes may be primary or secondary, and may offer therapeutic opportunities to increase perfusion, prolong the retina and optic nerve head function, and maintain vision in the canine glaucomas.  相似文献   

10.
Kartagener's syndrome (KS) is a rare, congenital condition characterized by situs inversus, rhinosinusitis, and bronchiectasis. An underlying ciliary dysfunction (e.g., immotility or dyskinetic beating) produces most of the clinical signs seen in affected animals. This case report reviews the history, clinical signs, and diagnosis of KS in a male, long-haired dachshund. This is the first known report of KS, and thus primary ciliary dyskinesia, in this breed of dog.  相似文献   

11.
Background: Paroxysmal dyskinesias are episodes of abnormal, involuntary movement or muscle tone, distinguished from seizures by the character of the episode and lack of seizure activity on ictal EEG. Hypothesis: Paroxysmal dyskinesia is an inherited, autosomal recessive disorder in Chinook dogs. Animals: Families of Chinook dogs with paroxysmal dyskinesia. Methods: Pedigrees and medical histories were reviewed for 299 Chinook dogs. A family of 51 dogs was used for analysis. Episodes were classified as seizures, paroxysmal dyskinesia, or unknown, and segregation analysis was performed. Results: Paroxysmal dyskinesia was identified in 16 of 51 dogs and characterized by an inability to stand or ambulate, head tremors, and involuntary flexion of 1 or multiple limbs, without autonomic signs or loss of consciousness. Episode duration varied from minutes to an hour. Inter‐ictal EEGs recorded on 2 dogs with dyskinesia were normal. Three dogs with dyskinesia also had generalized tonic‐clonic seizures. One of 51 dogs had episodes of undetermined type. Phenotype was unknown for 6 of 51 dogs, and 28 dogs were unaffected. Segregation was consistent with an autosomal recessive trait. Conclusions and Clinical Importance: This movement disorder is prevalent in the Chinook breed, and consistent with a partially penetrant autosomal recessive or polygenic trait. Insufficient evidence exists for definitive localization; episodes may be of basal nuclear origin, but atypical seizures and muscle membrane disorders remain possible etiologies. The generalized seizures may be a variant phenotype of the same mutation that results in dyskinesia, or the 2 syndromes may be independent.  相似文献   

12.
An 11-year-old Dalmatian was examined and treated for bilateral nasal discharge and cough of 6 months' duration. Response to medical treatment and surgical intervention was unsatisfactory. Histologic examination of lung tissue revealed chronic severe catarrhal bronchitis and bronchiolitis with bronchiectasis. Histologic findings and barium sulfate bronchography indicated abnormal mucociliary clearance in the respiratory tract. Electron microscopy revealed abnormalities or deletions of outer and/or inner dynein arms in 26% of the ciliary profiles from the affected dog. Similar abnormalities were not found in 500 ciliary profiles from age- and gender-matched control dogs.  相似文献   

13.
Iridociliary tumors are the second most common primary ocular tumor in dogs and are usually benign. A review of the Comparative Ocular Pathology Laboratory of Wisconsin (COPLOW) database in 2009 suggested a potential correlation between malignant iridociliary epithelial tumors and ciliary body ablation by intravitreal gentamicin injection for the treatment of glaucoma. The purpose of this case series was to determine whether there is evidence of such a correlation in the COPLOW collection. Mining of the COPLOW database revealed that a significant number (39.5%) of canine globes with a history of ciliary body ablation were subsequently diagnosed with primary ocular tumors at enucleation, most commonly iridociliary epithelial tumors and melanocytic tumors. It is possible that neoplasia was present but unrecognized at the time of ciliary body ablation. These tumors had a higher than expected incidence of malignancy. These cases underscore the importance of reserving ciliary body ablation with gentamicin for disease‐free eyes.  相似文献   

14.
By using ultrasound biomicroscopy (UBM), the cross-sectional structures of the entire iridocorneal angle (ICA) which are unable to assess with gonioscopic examination were evaluated objectively and quantitatively in live healthy and glaucomatous dogs. The ICAs of normotensive eyes in healthy dogs with normal open angle (NOR), a predisposition to primary closed angle glaucoma (PCAG) (PREDIS) and suffering from unilateral PCAG (UNI), as well as the ICAs of hypertensive eyes with acute and chronic PCAG (ACG and CRG), were assessed. The opening of the ciliary cleft in PREDIS was smaller than that in NOR. In UNI, the opening and area of the ciliary cleft were significantly decreased compared with those of NOR and PREDIS. ACG had widespread structural abnormalities including marked decrease in the ciliary cleft and scleral venous plexus, and a thinner sclera than those in normotensive eyes, whereas the ICA collapsed in CRG with the thinnest sclera. Medical therapy-responsive glaucomatous cases had wider ciliary cleft and scleral venous plexus than unresponsive ones. These findings suggest that the ciliary cleft and scleral venous plexus of the ICA are key structures contributing to not only the pathophysiology of canine glaucoma but also the responsiveness to medical therapy in glaucomatous eyes, and cross-sectional entire structures of the ICA should be evaluated quantitatively with UBM when diagnosing and managing canine glaucoma.  相似文献   

15.
A nine-month-old golden retriever bitch was presented with exercise intolerance and recurrent nasal discharge. Based on clinical, radiographic and ultrasonographic examination, a diagnosis of rhinitis, situs inversus totalis and tricuspid valve insufficiency was established. The results of video- and electron microscopy studies of the respiratory epithelium were compatible with primary ciliary dyskinesia (PCD). However, no evidence of a primary ultrastructural defect of the cilia was found. The dog was euthanased because of the poor prognosis. At necropsy, a hydrocephalus internus and a subaortic stenosis were additionally diagnosed. PCD, in combination with situs inversus, has been previously reported in golden retrievers, but without a concomitant hydrocephalus internus. Furthermore, concomitant occurrence of internal cardiac malformation and PCD has not previously been reported in the dog.  相似文献   

16.
The incidence of primary and secondary glaucoma in dogs was investigated. A total of 1244 dogs received ophthalmologic examinations, including tonometry and gonioscopy. Goniophotographs were taken using a goniolens to evaluate the iridocorneal angle (ICA) as well as pectinate ligament (PL). The anterior width of the ciliary cleft and the total distance from the origin of the PL to the anterior corneal surface were measured from the goniophotographs. Glaucoma was diagnosed based on the cupping of the optic nerve head, clinical signs, ocular changes, and high IOP, and it was synchronized with gonioscopic grades to differentiate between primary and secondary glaucoma. We investigated 1244 dogs of 29 breeds, including the mixed breed; among these, glaucoma was diagnosed in 127 dogs (162 eyes). Of 162 eyes, primary glaucoma was diagnosed in 129 eyes and secondary glaucoma in 33 eyes. Shiba Inu dogs (42 dogs, 33%) showed the highest incidence of glaucoma, followed by Shih-Tzu (21 dogs, 16.5%). Furthermore, all the glaucomatous Shiba Inu dogs had primary glaucoma with abnormal ICA grades and dysplastic PLs. The findings of our study reveal that the Shiba Inu breed in Japan may have a hereditary predisposition to glaucoma.  相似文献   

17.

Background

Primary ciliary dyskinesia (PCD) is generally a recessively inherited disorder characterized by dysfunction of motile cilia. A mutation in a new causative gene (CCDC39) has been identified in the Old English Sheepdog (OES).

Objectives

To describe the clinical findings and the molecular changes of affected dogs and estimate the worldwide prevalence of the mutation in a large cohort of OES.

Animals

578 OES, including 28 affected and 550 clinically healthy dogs.

Methods

This retrospective study reviewed the data of OES diagnosed with PCD and OES tested for the mutation. Clinical data including results of physical examination and further investigations were obtained on 11/28 dogs. CCDC39 expression was assessed by qRT‐PCR and Western blot analysis in affected dogs and healthy dogs. DNA was extracted on 561/578 dogs and a genetic test by Taqman technology was developed to genotype the CCDC39 mutation in these dogs.

Results

Clinical findings were recurrent nasal discharge and cough, pyrexia, leucocytosis, and bronchopneumonia. Ultrastructural defects were characterized by central microtubular abnormalities and decreased number of inner dynein arms (IDAs). Molecular analysis revealed a reduced expression of CCDC39 RNA and an absence of CCDC39 protein in affected dogs compared to healthy dogs. The mutation was more frequent in nonrandomly selected European OES population with a higher proportion of carriers (19%) compared to non‐European dogs (7%).

Conclusion and Clinical Importance

CCDC39 mutation is dispersed in a worldwide population and is responsible for PCD in this breed. Genetic testing might enable control of this disease.  相似文献   

18.
Chronic pneumonia was investigated in a litter of young Chinese Shar Pei in which 4 of 6 dogs were affected. Serum immunoglobulin concentrations (IgA, IgG, IgM) determined by radial immunodiffusion varied over time, but were not consistently lower in affected dogs, compared with control dogs. Two dogs that died had hydrocephalus and lymphoid depletion, in addition to severe broncho-pneumonia. Evaluation of ciliary ultrastructure in 2 affected dogs revealed random orientation of adjacent respiratory tract or oviductal cilia and a greater number of microtubular disarrangements, compared with control dogs. In vivo tracheal mucociliary clearance of 99mtechnetium macroaggregated albumin was absent in 1 dog examined. The ciliary abnormalities were suspected to have resulted in an inefficient mucociliary transport system predisposing to the development of pneumonia. Further evaluation of 1 Chinese Shar Pei revealed lymphocyte mitogenesis results that were not consistently less than those of a control dog, normal total hemolytic complement values, and normal blood neutrophil chemotaxis.  相似文献   

19.
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.  相似文献   

20.
A rare association between situs inversus totalis (SIT), restrictive ventricular septal defect, severe subaortic stenosis, and tricuspid dysplasia was observed in an adult mixed-breed dog. Primary ciliary dyskinesia and Kartagener's syndrome were excluded. After 15 mo the dog died suddenly. The association between SIT and congenital heart diseases is discussed.  相似文献   

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