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العنوان
Amyloidosis : Recent Trends and updates in Diagnosis and Treatment/
المؤلف
Makled,Dalia El Sayed El Araby
هيئة الاعداد
باحث / داليا السيد العربي مقلد
مشرف / عادل أحمد حليم إمام
مشرف / هبة محمود السيد دياب
تاريخ النشر
2016
عدد الصفحات
195.p:
اللغة
الإنجليزية
الدرجة
ماجستير
التخصص
الأمراض الجلدية
تاريخ الإجازة
1/1/2016
مكان الإجازة
جامعة عين شمس - كلية الطب - Dermatology, venereology and Andrology
الفهرس
Only 14 pages are availabe for public view

from 195

from 195

Abstract

Amyloidosis is an abnormal extracellular deposition of heterogenic, misfolded, proteinaceous substances. They are composed of one of a family of biochemically unrelated proteins which deposited in the form of insoluble oligomeric and polymeric protein fibrils in various tissues leading to architectural and functional changes of tissue. It typically appears in middle-age and older individuals and, occasionally, younger persons. The risk factors including chronic infection, neoplastic and autoimmune disease, family history and kidney disease that requires dialysis for several years. It is a tissue-based diagnosis. Biopsy of classical mucocutaneous lesions is the procedure of first choice. However, biopsies can be done elsewhere either from a clinically involved organ or clinically uninvolved organ which is known to contain amyloid deposits in the systemic forms like abdominal subcutaneous fat, rectal, gingival or renal biopsies. It stained with Congo-red stain giving a positive characteristic “apple-green birefringence” under polarization. It is classified to systemic and localized each of these, is subclassified into primary and secondary types which including localized cutaneous amyloidosis and cutaneous involvement in systemic amyloidosis. While, LCA may be either primary which is clinically classified into keratinic primary localized amyloidosis kPLCA with 2 types, MA and LA type, and NLPCA. The modern era of amyloidosis classification started in the late 1960s with the development of methods to make amyloid fibrils soluble, there are 31 known extracellular fibril proteins in humans. There are various therapeutic modalities either conventional or noval treatments. Many therapy strategies for PCA are not standardized but effective on both pruritus and skin lesions. They are include NB-UVB, PUVA, short-wavelength UVA, and broad band UVB phototherapy (UVA1 phototherapy). Topical and systemic therapy have been proposed for the removal of amyloid of cutaneous amyloidosis that improves the cosmetic appearance of the lesions; these include physical therapeutic options such as: TENS therapy in MA, surgical excision of individual lesions, split skin grafting, cryotherapy, dermabrasion, scalpel scraping, curettage, electro curettage, electrodessication and laser vaporization like: pulsed dye laser, doubled Q-switched- Nd: YAG, Fractional ablative 2,940 nm Erbium: YAG laser, non-ablative fractional 1,550nm Yttrium/Erbium fiber laser (NAFL) and CO2 laser have been successfully used.
Conclusioin: A comprehensive review of treatment options for PCA showed no controlled trials available, and thus no evidence based therapy can be proposed. Despite the presence of various therapeutic modalities for PCA, in general, none is considered curative or satisfactory as the treatment alternatives usually not effective and have a disappointing results. Identifying the type of amyloidosis and biochemical nature of fibril proteins became crucial to determine clinical management, prognosis and treatment. Thus, the researchers are trying to unravel the genesis of the various types of amyloidosis, to improve the detection of amyloid in the individual patient, and to search for more effective ways of treatment