Clinical Manifestations of Acute Hemolysis in Children with Glucose-۶-Phosphate Dehydrogenase Deficiency in Fars Province, Iran
محل انتشار: مجله بین المللی کودکان، دوره: 6، شماره: 4
سال انتشار: 1397
نوع سند: مقاله ژورنالی
زبان: انگلیسی
مشاهده: 62
فایل این مقاله در 6 صفحه با فرمت PDF قابل دریافت می باشد
- صدور گواهی نمایه سازی
- من نویسنده این مقاله هستم
استخراج به نرم افزارهای پژوهشی:
شناسه ملی سند علمی:
JR_INJPM-6-4_007
تاریخ نمایه سازی: 5 شهریور 1402
چکیده مقاله:
Background: Absence or deficiency in Glucose-۶-phosphate dehydrogenase (G۶PD) enzyme in patients with G۶PD deficiency presents with a wide spectrum of symptoms. This study evaluates the clinical features of acute hemolysis in children with G۶PD deficiency. Materials and Methods: Seventy G۶PD deficient children younger than ۱۸ years old were included in this cross sectional study in Dastgheib Hospital of Shiraz in South West of Iran. Complete blood count, zinc level, reticulocyte count, peripheral blood smear, liver function tests and coombs test were performed for all patients. Subject were observed for multiple clinical manifestations such as paleness, Yellow sclera, Dark Urine, abdominal pain, back pain, vomiting, diarrhea or constipation, liver tenderness, hepatomegaly, splenomegaly, changes in level of consciousness according to Glasgow Coma Scale. Results: All of the G۶PD deficient children developed paleness at the time of admission. The next frequent manifestations in our population were yellow sclera and dark urine that were present in ۶۹ and ۶۸ patients, respectively. The least prevalent features were diarrhea and constipation. Liver tenderness, hepatomegaly and splenomegaly were not found in our population. There were no significant differencesin age distribution between the boys and girls (p>۰.۰۵). There were not any significant correlation between each clinical manifestation and the primary Hemoglobin (Hb) level, number of needed transfusion, severity of hemoglobinuria and hospitalization duration (p>۰.۰۵). Conclusion: In this study, pallor, icterus and dark urine were the three important symptoms in G۶PD patients with acute hemolysis. There was no correlation between the primary clinical symptoms and severity of the hospital course characteristics of the patients such as Hb level, hemoglobinuria and number of needed transfusion.
کلیدواژه ها:
نویسندگان
Naser Honar
Pediatric Gastroenterology and Hepatology Department, Shiraz University of Medical Sciences, Shiraz, Iran.
Haniyeh Javanmardi
Student Research Committee, Shiraz Endocrinology and Metabolism Research Center, Shiraz University of Medical Sciences, Shiraz, Iran.
Forough Saki
Shiraz Endocrinology and Metabolism Research Center, Shiraz University of Medical Sciences, Shiraz, Iran.
Azadeh Rezaeefard
Pediatric Gastroenterology and Hepatology Department, Shiraz University of Medical Sciences, Shiraz, Iran.
Mahdi Shahriari
Hematology Research Center, Shiraz University of Medical Sciences, Shiraz, Iran.