Non so se questo possa avere alcun valore scientifico o se dipenda semplicemente dal fatto che a Chaaria vediamo tantissimi pazienti ogni giorno (campione quindi molto elevato), ma desidero segnalare che negli ultimi due anni abbiamo visto tre casi di sindrome (o triade) di Kartagener.
Si tratta di una anomalia probabilmente congenita ed assai rara, anche chiamata sindrome “delle ciglia immobili”, in quanto vi e’ pure un deficit delle ciglia ed un difetto del trasporto mucociliare.
La classica triade della sindrome di Kartagener consta di:
1) bronchiectasis
2) defect in development of the paranasal sinuses
3) transposition of the viscera
Clinically presents with bronchitis, sinusitis and ear infections. Bronchitis and respiratory disorders were present in all three patients came to our attention. All complained of difficulty 'all'espettorazione.
48% of cases have "situs inversus viscerum" (with the right heart, liver, left, spleen on the right, etc.). In our small series, two cases had a complete transposition of the organs, while in only one patient had dextrocardia with liver and spleen in place.
30% was evident on chest X-ray bronchiestasie. One of our case had a right pulmonary fibrosis interesting nearly all the lungs with the exception of quotes.
shows 20% of digital clubbing as a sign of chronic hypoxia. Even in this case a patient on 3 of our series suffered from this condition.
The literature also describes an infertility 'male involvement microtubular complex tails of sperm, but on this we found no clinical Chaar since two of our cases were women, and male age was' pre-pubertal.
This signal is supposed to be a stimulus to investigate if maybe the black race had a genetic predisposition to the disease more 'pronounced than in Caucasians.
Fr Dr Joseph Gaido
Sources:
1) Colombo et al. Treaty of Surgery. Minerva Medica
2) Butterwoorths. Medical Dictionary. Macdonald Critchley
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