Primär ciliär dyskinesi som orsak till kronisk lungsjukdom hos hund : en litteraturstudie och fallpresentation

Sammanfattning: Primary ciliary dyskinesia (PCD) is an important but unusual cause of chronic respiratory disease of young dogs. The purpuse of this paper is to study and present internationally publicated papers on PCD in dogs and present three dogs, in which PCD was suspected. PCD has been reported in many dog breeds as well as in people. In dogs an humans, PCD is caused by inborn genetical defects which causes structural and functional abnormalities of cilia in the respiratory organs and other cilia in the body. Situs inversus occurs in about half the cases in people and probably in dogs as well. Respiratory signs usually predominate. Because of dysfunctional cilia in the respiratory organs, the mucociliary clearance is impaired. The airways become congested with mucus that predisposes the dogs to bacterial infections and irreversible changes lika bronchiectasis. Dogs are often young when first presented with respiratory signs and with infections that are resistent to treatment. Definitive diagnosis in dogs can be established only by demonstrating presence of specific ultrastructural leisions in cilia with transmission electronmicroscope (TEM) and by demonstrating impaired mucociliary clearance in the airways with a gammacamera. Which test that could be performed, should be based on the case in question. Sometimes the diagnosis can not be esteblished. Treatement for PCD is aimed at fascilitating mucociliary clearance and controlling infections. The prognosis is guardead in dogs but better the earlier diagnosis is established. Long term consequenses include irreversible changes in the respiratory organs as well as right-sided heart failure caused by pulmonary artery hypertension. PCD can be prevented by breedingprograms, once dogs with PCD have been identified. In this paper, three dogs are presented, in which PCD was suspected. The three cases were rottweilers and from the same litter. They lived in separate homes and were between four and 16 mounths when first presented. They all had upper and lower respiratory signs, radiographs of the thorax that revealed bronchopneumonia and poor responses to treatment with antibiotics and prednisolone. The dogs were euthanased and biopsis were taken from their tracheas and were examined with TEM. No abnormal ciliary ultrastructure was found and the diagnosis PCD could not be confirmed or dismissed. The conclusion is that PCD should be considered in young dogs with chronic recurring repsiratory signs. Which tests and in what order they are performed for a definitive diagnosis, should be based on the case in question.