Bulozā epidermolīze, ādas un gļotādu kopšana. Rekomendācijas pacientiem
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Date
2022
Authors
Journal Title
Journal ISSN
Volume Title
Publisher
Rīgas Stradiņa universitāte
Rīga Stradiņš University
Rīga Stradiņš University
Abstract
Bullozā epidermolīze (Epidermolysis bullosa) ir grupa retu (prevelence ir 1-9 gadījumi uz 1 000 000 jaundzimušo), klīniski un ģenētiski heterogēnu, iedzimtu ādas slimību, kas raksturojas ar ādas strukturālām anomālijām, trauslumu, izraisot bullu, eroziju, nedzīstošu čūlu un rētu veidošanos pēc minimālas vai nenozīmīgas ādas traumas. Bullozā epidermolīze skar visus dzimumus, rases un etniskās grupas.
Bullozai epidermolīzei ir izšķir četrus galvenos jeb klasiskos bullozās epidermolīzes tipus: Simplex tipa bullozā epidermolīze, junctional tipa bullozā epidermolīze, distrofiskā tipa bullozā epidermolīze un Kindlera sindroms, balstoties uz ādas slāni, kurā ir defekts, klīniskās ainas, smaguma pakāpes un ekstrakutāno audu iesaistes.
Bullozai epidermolīzei nav specifiskas ārstēšanas metodes. Pacientu aprūpe balstās uz brūču profilaksi un adekvātu aprūpi, komplikāciju risku samazināšanu. Pieejamā medicīniskā literatūra par bullozās epidermolīzes pacientu aprūpi ir galvenokārt angļu valodā un sastāv no gadījumu analītiskiem pētījumiem, ekspertu atzinumiem, pacientu un aprūpētāju pieredzes.
Medicīniskajā literatūrā sniegtās rekomendācijas pacientiem atšķiras atkarībā no bullozās epidermolīzes formas, klīniskajām izpausmēm, pacienta vecuma un prioritātēm. Smagu formu bullozās epidermolīzes pacientu kopšana ir dārga, laikietilpīga un sarežģīta.
Darba mērķis: Izveidot apkopotu uzskates materiālu latviešu valodā par ādas, gļotādas, matu un nagu kopšanas īpatnībām pacientiem ar bullozo epidermolīzi, sniedzot iespēju iegūt jaunas zināšanas bullozās epidermolīzes pacientu aprūpē, tādējādi uzlabojot pacientu dzīves kvalitāti un dzīvildzi.
Epidermolysis bullosa (EB) is a group of rare (prevalence 1-9 cases per 1 000 000 live births) clinically and genetically heterogeneous, inherited skin fragility disorder characterized by structural anomalies and vulnerability to mechanical stress, causing blisters, erosions, nonhealing ulceration, and scars following minor trauma. EB affects males and females, people of all races and ethnic backgrounds equally. EB is categorized into four major types: Epidermolysis bullosa simplex, junctional epidermolysis bullosa, dystrophic epidermolysis bullosa and Kindler syndrome, based upon the level of skin cleavage, lesion distribution, severity, and presence of extracutaneous involvement There is no specific treatment for epidermolysis bullosa. Treatment is aimed at helping prevent blisters, adequate wound management, and minimising risk of complications. Available medical literature about epidermolysis bullosa, patient skin and mucosa care are commonly in English, and is predominantly made up of non-analytic studies, expert opinion, patient, and caretaker experience. Recommended strategies for epidermolysis bullosa patients in literature varies depending on EB type, phenotype, patients age and priorities. Severe form of EB patient skin, mucosa, nail, and hair care is expensive, time consuming and complex. The aim of the research: To create a compiled literature review in Latvian about epidermolysis bullosa patient skin, mucosa, hair, and nail care. Provide recommendations and knowledge on EB patient skin and mucosa care, wound care, and complication prevention, improving patient quality of life and survival.
Epidermolysis bullosa (EB) is a group of rare (prevalence 1-9 cases per 1 000 000 live births) clinically and genetically heterogeneous, inherited skin fragility disorder characterized by structural anomalies and vulnerability to mechanical stress, causing blisters, erosions, nonhealing ulceration, and scars following minor trauma. EB affects males and females, people of all races and ethnic backgrounds equally. EB is categorized into four major types: Epidermolysis bullosa simplex, junctional epidermolysis bullosa, dystrophic epidermolysis bullosa and Kindler syndrome, based upon the level of skin cleavage, lesion distribution, severity, and presence of extracutaneous involvement There is no specific treatment for epidermolysis bullosa. Treatment is aimed at helping prevent blisters, adequate wound management, and minimising risk of complications. Available medical literature about epidermolysis bullosa, patient skin and mucosa care are commonly in English, and is predominantly made up of non-analytic studies, expert opinion, patient, and caretaker experience. Recommended strategies for epidermolysis bullosa patients in literature varies depending on EB type, phenotype, patients age and priorities. Severe form of EB patient skin, mucosa, nail, and hair care is expensive, time consuming and complex. The aim of the research: To create a compiled literature review in Latvian about epidermolysis bullosa patient skin, mucosa, hair, and nail care. Provide recommendations and knowledge on EB patient skin and mucosa care, wound care, and complication prevention, improving patient quality of life and survival.
Description
Medicīna
Medicine
Veselības aprūpe
Health Care
Medicine
Veselības aprūpe
Health Care
Keywords
Bullozā epidermolīze, Ādas kopšana, Brūču kopšana, Aprūpes rekomendācijas, Sāpju kontrole, Dzīves kvalitāte, Epidermolysis bullosa, Skin care, Wound care, Multidisciplinary management recommendations, Pain management, Quality of life