A Case of Primary Lymphedema

Abstract

Lymphedema is edema or tissue swelling resulting from failure of the lymphatic system to transport excess capillary filtrate containing liquid, macromolecules, and mobile cells back to the blood. It is usually secondary to filariasis, surgery, trauma, or infection. Primary lymphedema is thought to have a strong genetic component and arises from faulty development or function of the lymphatic system. It can be sporadic or inherited. In study a case of primary lymphedema in the left lower extremity in a 12 years old girl was presented. Pathogenesis and classification of primary lymphedema were briefly reviewed

Keywords

• Primary lymphedema classification,child

Bir Primer Lenfödem Olgusu

Abstract

Lenfödem, sivi, makromolekül ve hücreler içeren asiri kapiller filtratin kana geri geçisinde lenfatik sistemde bir yetmezlik olmasindan kaynaklanan ödem ya da doku sisligidir. Genellikle, Flariaz, cerrahi travma ya da enfeksiyon gibi durumlara ikincil olarak gelisir. Primer lenfödemde bir genetik yatkinlik oldugu düsünülmektedir. Sporadik veya kalitsal olabilir; lenfatik sistemin gelisimi veya islevinde bir sorundan kaynaklanmaktadir. Bu çalismada, bir alt ekstremitesinde primer lenfödem gelisimi olan on iki yasindaki bir kiz hasta sunulmaktadir. Lenfödem patogenezi ve siniflandirmasi kisaca gözden geçirilmektedir

Keywords

• Primer lenfödem siniflamasi,çocuk

References

1. Browse NL: The diagnosis and management of primary lymphedema. J Vasc Surg 3:181- ,1986.
2. Cambria RA, Gloviczki P, Naessens JM, Wahner HW: Noninvasive evaluation of the lymphatic system with lymphoscintigraphy: A prospective, semiquantitative analysis in 386 extremities. J Vasc Surg 18:773-782, 1993.
3. Ceyhan M, Dilmen U, Çagla r K, Senses DA, Oran O : A case of primary lymphedema. The Turk J Pediatr 27:107-108,1985.
4. Erickson RP, Dagenais SL, Caulder MS, et al: Clinical heterogeneity in lymphoedema- distichiasis with FOXC2 truncating mutations. J Med Genet 38:761-766, 2001.
5. Keseroglu HO, Özden MG, Akkas BE ve ark : Lenfanjit sonrasi gelisen bir lenfödem olgusunun lenfosintigrafik degerlendirilmesi, tani ve tedavi yaklasimlari. Türkiye Klin J. Dermatol :205-10, 2005.
6. Ter SE, Alavi A, Kim CK, Merli G: Lymphoscintigraphy. Reliable test for the diagnosis of lymphedema. Clin Nucl Med :646-54, 1993.
7. Mortimer PS: Swollen lower limb-2: lymphedema. BMJ 320:1527-9, 2000.
8. Lazareth I: Classification of lymphedema. Rev Med. Intern 3:375-378, 2002.

9. Camitta BM : The lymphatic system. In Nelson Textbook of Pediatrics, eds Berman RE, Kliegman RM, Jenson HB, 17 th. Ed Saunders Philadelphia pp (1677-8), 2004.
10. Brice G, Mansour S, Bell R et al: Analysis of the phenotypic abnormalities in lymphoedema-distichiasis syndrome in 74 patients with FOXC2 mutations or linkage to q24. J Med Genet 39; 478-483, 2002.