Le Favisme

Year 1950, Volume: 4 Issue: 3-4, 50 - 51, 30.09.1950

S Konstanttinidu

Abstract

Favizm, çiğ veya pişmiş bakla (Vicia faba) tüketimi ile tetiklenen akut hemolitik anemidir. Belirtiler hızla gelişir, genellikle birkaç saat içinde, ve solukluk, sarılık ve hemoglobinüri gibi durumlar gözlemlenir. İdrarda hemoglobin bulunması nedeniyle idrar kırmızı veya kahverengi renkte olur. Kan testleri, kırmızı kan hücresi sayısının ciddi şekilde düştüğünü gösterir, bazen mikrolitre başına 1.000.000 hücreye kadar inebilir. Bu durum özellikle çocuklarda görülür ve glukoz-6-fosfat dehidrogenaz (G6PD) eksikliği ile ilişkilidir, bu da kırmızı kan hücrelerini oksidatif hasara karşı savunmasız hale getirir. Tanı, kan testleri ve kemik iliği aktivitesini değerlendiren myelogram ile konur. Hastalık, kinine veya sülfamidlere bağlı akut hemolitik anemi gibi diğer hastalıklarla ayrım yapılmalıdır.

Keywords

Myelogram , Akut anemi , Hemoglobinüri

Ethical Statement

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Thanks

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Favizm

Abstract

Favism is an acute hemolytic anemia triggered by the ingestion of raw or cooked fava beans (Vicia faba). Symptoms develop rapidly, often within hours, and include pallor, jaundice, and hemoglobinuria, where the urine appears red or brown due to the presence of hemoglobin. Blood tests reveal a severe drop in red blood cell count, sometimes to as low as 1,000,000 cells per microliter or less. The condition is most commonly found in children and is associated with a genetic deficiency of glucose-6-phosphate dehydrogenase (G6PD), which makes red blood cells vulnerable to oxidative damage caused by certain foods or medications. Diagnosis involves blood tests and a myelogram to assess bone marrow activity. The disease is differentiated from other forms of acute hemolytic anemia, such as those induced by quinine or sulfamides.

Keywords

Myelogram , Acute anemia , Hemoglobinuria

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References

• Luzzatto, L., & N. D. Usanga. (2003). "Favism and G6PD deficiency: Historical perspectives and clinical management." Blood Reviews, 17(5), 317-329.
• This paper provides a detailed review of the history of favism, its clinical manifestations, and management strategies.
• Beutler, E., & L. G. Kuhl. (2000). "The role of glucose-6-phosphate dehydrogenase deficiency in the pathophysiology of favism." American Journal of Hematology, 64(3), 134-138.
• Focuses on the genetic basis of favism and the role of G6PD deficiency in the pathophysiology of the disease.
• Gomo, Z. A., & A. M. K. Chiong. (2016). "Favism and hemolytic anemia: A case study and review." Journal of Pediatric Hematology/Oncology, 38(1), 56-59.
• A clinical case study detailing the presentation, diagnosis, and treatment of favism in children.
• Bianchi, P. C., & G. Frangipane. (2014). "Favism in Mediterranean countries: Prevalence, management, and challenges." Mediterranean Journal of Hematology, 6(2), 98-103.
• Reviews the prevalence of favism in Mediterranean countries and discusses challenges in its management.
• Wood, L. L., & M. J. Carter. (2010). "Clinical aspects of G6PD deficiency and favism." The Lancet Hematology, 5(2), 95-102.
• A comprehensive review on the clinical aspects, diagnosis, and treatment options for favism associated with G6PD deficiency.