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العنوان
DICOTYLEDONS AS OXIDIZING AGENTS IN G6PD DEFICIENCY /
المؤلف
Abd-Elbaseer, Dina Abd-Elmoamen.
هيئة الاعداد
باحث / Dina Abd-Elmoamen Abd-Elbaseer
مشرف / MOHSEN SALEH EL-ALFY
مشرف / EHAB KHAIRY EMAM
مناقش / SAHAR SAMIR ABD-ELMAQSOUD
تاريخ النشر
2014.
عدد الصفحات
102 p. :
اللغة
الإنجليزية
الدرجة
ماجستير
التخصص
طب الأطفال ، الفترة المحيطة بالولادة وصحة الطفل
تاريخ الإجازة
1/1/2014
مكان الإجازة
جامعة عين شمس - كلية الطب - Pediatrics
الفهرس
Only 14 pages are availabe for public view

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from 102

Abstract

Glucose-6-phosphate dehydrogenase deficiency is one of the most common inherited disorders in the world. It is particularly common in populations living in malaria endemic areas affecting more than 400 million people worldwide.
Typical clinical presentation of G6PD deficiency is acute hemolytic anemia presented with jaundice, fever, pallor, dark urine, decreased hemoglobin levels, increased level of LDH and increased reticulocytes count. The attack is triggered by ingestion of fava beans or transmitted via breast milk of mothers who have ingested fava beans or even inhalation of pollen from vicia faba plant (favism).
The exact mechanism of favism remains unclear, there is no a research paper or other proof as to the exact chemical in fava beans that cause haemolysis
However, convicine and vicine are the generally accepted culprits.
Other triggers of acute hemolysis are certain drugs like antimalarial drugs and also infections can produce an attack of hemolysis in G6PD deficiency.
This study was conducted on 50 patients (we exclude one patient who found to have normal G6PD assay) so the actual number is 49 children who routinely visit the hematology clinic Ain Shams University from 2000 to 2012 and 20 controls for the MDA level.
All the cases diagnosed as G6PD deficient patients, or newly discovered cases, excluded any coexisting chronic diseases as hepatic or renal failure.
History collected as origin of birth, family history for the disease, consanguinity, history of blood transfusion, age of diagnosis and history of neonatal jaundice( with admission to NICU).
Investigations done as for serum bilirubin, complete blood picture with reticulocyte count, G6PD enzyme assay and MDA level after a dietary recall of different legumes for three successive days.
The studied group ages from 8 months to 26 years old with mean age 5.9, 89.2% males and 10.2% females.
87.2% of the patients experienced their first attack of acute hemolysis at mean age of 2.5 years, where 93.6% of the cases offended by fava beans or green beans and only one case was offended by drugs and no infections.
41 patients (83.6%) classified as class II with REA between 10-60% and 8 patients (16.3%) classified as class III where REA less than 10%.
Regarding neonatal jaundice, 59.2% of the cases developed neonatal jaundice (38.7% needed NICU), and 67.3% of the cases received blood transfusion.
MDA levels were found to be higher in the cases than that of controls with mean of 5.62 nmol/mL to 2.27 nmol/mL respectively, also other studies shows the same results without the dietary recall which proves that no relations between the legumes ingestion and the level of MDA.
Moreover, the results showed no relation between legumes ingestion and level of hemoglobin concentration, reticulocyte count, MCV, total bilirubin or even change in the color of urine.
In conclusion: However most of the research papers agreed that the main cause of favism is ingestion of different legumes (mainly fava beans), certain drugs or infections, yet it is still unclear exactly what causes acute hemolysis in the cases, it varies from case to another regarding the degree of hemolysis, history of blood transfusion and MDA levels, which requires further studied for larger number of cases and for longer period of time.