ArticleAfrican health sciences2016
Long-term outcome of Tunisian children with primary ciliary dyskinesia confirmed by transmission electron microscopy.
Article in African health sciences, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 2 papers.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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2 citing papers in PubMed, 4 citations in OpenAlex.
- Rare Diseases of the Nose, the Paranasal Sinuses, and the Anterior Skull Base.Laryngo- rhino- otologie · 2021Review
- Child, reproductive, mental health; infections and NCDs in the African environment.African health sciences · 2016Article
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Authors and funding
7 authors at 2 institutions in 1 country.
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No grant is acknowledged in the PubMed record.
Abstract
backgroundPrimary ciliary dyskinesia (PCD) is rare. Its diagnosis requires experienced specialists and expensive infrastructure. Its prognosis is variable.
objectiveTo study the long-term outcome of PCD in Tunisian children with ciliary ultra-structure defects detected by electron microscope.
methodsCovering a period of 20 years (1996-2015), this retrospective study included all patients with definite PCD (outer dynein arms (DA) defects and/or situs inversus) and presumed PCD (other ciliary ultra-structure defects). The clinical data and the investigations made were registered at diagnosis and during the follow-up.
resultsPatients with a definite PCD (G1, n=7) were diagnosed earlier compared to those with a presumed PCD (G2, n=13) (2.5 vs. 9.3 years on average). At diagnosis, bronchiectasis was more frequent in G1 (3/7 vs. 4/13). The inner DA loss was constant in G1 and predominant in G2. The treatment adhesion was more often irregular in G2 (2/7 vs. 8/13). During a mean follow-up of 11 years, G1 showed less severe outcome (clubbing (0 vs. 3), bronchiectasis (3 vs. 11; more expanded in G2), proximal and distal airway obstruction (0/3 vs. 5/7), lobectomy (0 vs. 2), and death (0 vs. 2)).
conclusionPrecocious diagnosis and regular treatment may enhance the PCD prognosis.
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