The limbs on the right side are stronger. [The] cause may be . . . [that] . . . motion, and abilities of moving, are somewhat holpen from the liver, which lieth on the right side.(Sir Francis Bacon, Sylva sylvarum (1627).)Fifty per cent of people with primary ciliary dyskinesia (PCD) (also known as immotile cilia syndrome or Siewert-Kartagener syndrome) have situs inversus, which is thought to result from absent nodal ciliary rotation and failure of normal symmetry breaking. In a study of 88 people with PCD, only 15.2% of 46 individuals with situs inversus, and 14.3% of 42 individuals with situs solitus, were left handed. Because cerebral lateralization is therefore still present, the nodal cilia cannot be the primary mechanism responsible for symmetry breaking in the vertebrate body. Intriguingly, one behavioural lateralization, wearing a wrist-watch on the right wrist, did correlate with situs inversus.
Background: Although the pathophysiological defect in primary ciliary dyskinesia (PCD; Siewert's / Kartagener's syndrome) is now well characterised, there are few studies of the impact of the condition upon health function, particularly in later life. This study assesses the health impact of the condition in a large group of patients. In addition, it assesses the similarity in age of diagnosis, symptoms and problems of those with situs inversus (PCD-SI) and those with situs solitus (PCD-SS).
Primary ciliary dyskinesia (PCD) causes chronic cough, sinusitis and bronchiectasis, and half of patients also show situs inversus. The genetic basis and visible and concealed chronic symptoms provide potential for stigmatization. We describe a structural equation model linking a questionnaire measure of stigmatization to sex, age, personality (Big Five), symptoms (St George's Respiratory Questionnaire), health status (SF-36) and stress (GHQ-12). Stigma did not relate to physical symptoms or health, or to situs, but correlated with mental health and the social impact of symptoms. Neuroticism, extroversion, openness to experience, age, age at diagnosis and being female indirectly affected stigmatization via mental health.
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