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SM ISO690:2012 BEŢIU, Mircea, FIODOROVA, Nina, ALEXANDROVA, Tatiana, STURZA, Vasile. Epidermolizele buloase: aspecte clinico-evolutive şi de tratament
. In: Analele Ştiinţifice ale USMF „N. Testemiţanu”, 2013, nr. 3(14), pp. 746-752. ISSN 1857-1719. |
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Analele Ştiinţifice ale USMF „N. Testemiţanu” | ||||||
Numărul 3(14) / 2013 / ISSN 1857-1719 | ||||||
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Pag. 746-752 | ||||||
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Rezumat | ||||||
În articol sunt analizate 41 cazuri de epidermolizele buloase simple, joncţionale şi distrofice. Se constată predominanţa pacienţilor de sex masculin, din mediul rural, precum şi a formelor clinice de EB dominante. Manifestările buloase de epidermoliză au fost completate în o treime din cazuri cu afectare unghială, iar hiperhidroza, keratodermia palmo-plantară, alopecia şi leziunile ichtioziforme au fost semnalate sporadic. La circa o jumătate din pacienţi au fost constatate o serie de maladii concomitente (anemii, pneumonii, hepatite, pancreatite, pielonefrite). Comune au fost anomaliile dentare şi afectarea mucoaselor raportate la o treime din pacienţi, fiecare al zecelea pacient având şi retard mental. La circa 20% bolnavi cu epidermolize buloase au fost observate complicaţii cu agenţi microbieni, acrosclerodactilie, contracturi, mutilaţii, stenoze esofagiene, precum şi un caz de deces. Se accentuieză eficacitatea modestă a tratamentelor de rutină, lipsa diagnosticului prenatal, imperativul de administrare continuă a pansamentelor non-aderente, precum şi importanţa conlucrării cu organizaţiile de suport al pacienţilor cu epidermolize buloase de tip „DEBRA-Moldova”. |
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Manifestările buloase de epidermoliză au fost completate în o treime din cazuri cu afectare unghială, iar hiperhidroza, keratodermia palmo-plantară, alopecia şi leziunile ichtioziforme au fost semnalate sporadic. La circa o jumătate din pacienţi au fost constatate o serie de maladii concomitente (anemii, pneumonii, hepatite, pancreatite, pielonefrite). Comune au fost anomaliile dentare şi afectarea mucoaselor raportate la o treime din pacienţi, fiecare al zecelea pacient având şi retard mental. La circa 20% bolnavi cu epidermolize buloase au fost observate complicaţii cu agenţi microbieni, acrosclerodactilie, contracturi, mutilaţii, stenoze esofagiene, precum şi un caz de deces. Se accentuieză eficacitatea modestă a tratamentelor de rutină, lipsa diagnosticului prenatal, imperativul de administrare continuă a pansamentelor non-aderente, precum şi importanţa conlucrării cu organizaţiile de suport al pacienţilor cu epidermolize buloase de tip „DEBRA-Moldova”.</cfAbstr> <cfAbstr cfLangCode='EN' cfTrans='o'>41 cases of epidermolysis bullosa (EB), simple, jonctional and dystrophic types, were analyzed in this article. It is showed a predominance of male patients originated from rural regions as well as high incidence of dominant type of disease. Bullous manifestaions of EB in about 1/3 of cases were complicated with nail involvement, also hyperhidrosis, palmo-plantar keratoderma, alopecia and ichthyosiform lesions were seen occasionaly. Associated pathology as anemia, pneumonia, hepatitis, pancreatitis, pielonephritis were established in a half of patients. 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