Enfermedades pulmonares hereditarias

Flores, W. Desueza; Essa, Azza

doi:10.1016/j.med.2018.09.013

Cited by 1 publication

(4 citation statements)

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“…The diagnosis of a rare disease in a child has a major impact on the child and his or her parents. In relation to PCD, uncertainty about the prognosis [8], possible future medical complications [1,9] and associated fertility problems, especially in boys [2,6], lead to an increase in worries and a loss of the sensation of control in adolescent patients, reducing their emotional well-being. With this objective in mind, we examined the role that psychological factors can play in pre-adolescence and adolescence.…”

Section: Discussionmentioning

confidence: 99%

“…Primary ciliary dyskinesia syndrome (PCD) is an inherited, autosomal recessive disease, considered rare due to its low prevalence [1][2][3]. It is characterized by chronic upper and lower respiratory tract infections, including the middle ear, from birth [4,5].…”

Section: Introductionmentioning

confidence: 99%

“…Studies indicate that 1/15,000-20,000 live births [7] will present the disease, with a similar distribution among boys and girls [2,8]. The average age at diagnosis of PCD is approximately 4 years [1].…”

Section: Introductionmentioning

confidence: 99%

“…Despite medical advances, there is currently no treatment to correct ciliary dysfunction in PCD. Most treatments applied to patients with PCD are similar to those used with other similar diseases, such as cystic fibrosis [1,6,9].…”

Section: Introductionmentioning

confidence: 99%

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Primary ciliary dyskinesia and psychological well-being in adolescence

et al. 2020

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Primary ciliary dyskinesia (PCD) is a rare autosomal recessive disease with low prevalence in pediatrics. Health studies have not sufficiently analyzed the role of psychological variables in rare diseases such as PCD. This paper studies the psychological characteristics of a group of pediatric patients diagnosed with PCD compared to their healthy peers. The sample consisted of 48 preadolescents-adolescents, aged 9-18 years (M = 12.96; SD = 2.71), with similar distribution by sex, and 25% of the patients having dyskinesia. Clinical anxiety-depression, self-esteem and psychological well-being were evaluated using questionnaires: the Adolescent Psychological Well-being Scale (BIEPS-J), the Hospital Anxiety and Depression Scale (HADS) and the Rosenberg Self-Esteem Scale (RSE). Data were analysed using descriptive, mean comparison (t-test) and diffuse comparative qualitative analysis (QCA). The results show no differences were found between healthy and PCD patients in the variables analyzed, except for social ties showing the latter greater well-being in this aspect. In QCA models, the variables that best explained the high or low levels of well-being were depression and selfesteem, and primary ciliary dyskinesia was not a necessary condition for presenting low levels of well-being. In conclusion, our results highlight the need to explore psychological aspects in pediatric patients with rare diseases.

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