VOSD Vet

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Uncoordinated Cilia Function in Dogs

Primary Ciliary Dyskinesia in Dogs

Ciliary dyskinesia is an innate condition resulting from ciliary malfunction. Cilia, intricate hair-like structures capable of movement, line various bodily organs such as the upper and lower respiratory tracts, auditory tubes, brain ventricles, spinal canal, uterine tubes, and testicular ducts. The primary role of cilia is to move cells or fluids in their surroundings, also acting as a filtration system. Within the respiratory tract, cilia work to eliminate dust and other particles from inhaled air before it reaches the lungs. Normally, ciliary beating, the movement process, is coordinated through a complex interaction of numerous proteins within each cilium. However, in dogs affected by ciliary dyskinesia, movement is typically uncoordinated (dyskinetic) or absent, often accompanied by structural abnormalities in the affected cilia.

Clinical manifestations primarily occur in organs with cilia: impaired mucociliary clearance in the respiratory tract can lead to recurrent bacterial rhinosinusitis, bronchopneumonia, ear infections, chronic airway inflammation, and obstruction. Additionally, male infertility due to sperm immobility, hydrocephalus (brain fluid accumulation), and situs inversus (reversed organ placement) are common but variable features of ciliary dyskinesia. Diagnosis involves demonstrating the absence of tracheal mucociliary clearance and identifying specific lesions in respiratory cilia or sperm flagella. In patients without ciliary lesions, analysis of ciliary function confirms the diagnosis. Dogs presenting chronic respiratory tract issues and situs inversus are likely to have primary ciliary dyskinesia and may not require extensive diagnostic procedures.

This genetic disorder is likely inherited in an autosomal recessive manner. Symptoms typically manifest from a few days to five weeks after birth, although some dogs may remain asymptomatic for extended periods, ranging from six months to ten years. Ciliary dyskinesia has been observed exclusively in purebred dogs including bichon frises, border collies, bull mastiffs, Chihuahuas, shar peis, chow chows, Dalmatians, Doberman pinschers, English cocker spaniels, English pointers, English setters, English springer spaniels, golden retrievers, Gordon setters, long-haired dachshunds, miniature poodles, Old English sheepdogs, Newfoundlands, rottweilers, and Staffordshire bull terriers.

Symptoms

  • Wet, productive cough, possibly triggered by physical activity
  • Both nostrils discharge mucus and pus
  • Quickened breathing, difficulty breathing, and pale tissues may be evident
  • Persistent sneezing and coughing, potentially leading to significant mucus and pus production. Despite notable improvement with antibiotics, patients often experience ongoing nasal discharge and relapse once treatment ceases.
  • Family history: Larger litters tend to include more than one affected animal; offspring from previous matings of the same parents may have been affected.
  • Primarily affects young purebred dogs
  • Fertility: Females remain fertile, while males typically do not.

Causes

  • Hereditary condition
  • Consanguineous breeding

Diagnosis

Given the similarity of symptoms among various conditions, your veterinarian will perform several tests to establish a diagnosis. Initial steps include biopsy of nasal or bronchial mucosa. It’s crucial to identify specific lesions in a significant portion of cilia and observe the same defect in cilia from multiple sites (such as nasal and bronchial cilia, and sperm flagella), as well as in affected littermates, before confirming an inherited defect.

Confirmation of the diagnosis relies on analyzing ciliary beat frequency and synchrony. Additionally, an electrocardiogram, which records the heart’s electrical activity, can confirm situs inversus (reversed organ positioning).

Treatment

Regular exercise can aid in clearing airway secretions by leveraging the force generated during exhalation and coughing. Increased respiration induced by routine physical activity may enhance mucus clearance. In severe cases of life-threatening bronchopneumonia, supplemental oxygen therapy may be necessary. Antibiotics will be prescribed for respiratory infections based on bacterial culture and sensitivity testing. Although antibiotics may be administered indefinitely, their efficacy could diminish due to the development of bacterial resistance. Anesthesia poses risks for these patients due to impaired breathing ability.

Living and Management

Elevated ambient temperatures may lead to hyperthermia and the risk of heat stroke due to decreased ability for evaporative heat loss via the lungs. Prolonged air entrapment in the pleural cavity can result in cyst formation in the chest, potentially leading to rupture and subsequent pneumonic lung conditions. There may be additional post-effects to consider. Therefore, it’s crucial to diligently observe and monitor your pet’s condition, and schedule regular checkups with your veterinarian.

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