Clinical Manifestations of Acute Hemolysis in Children with Glucose-6-Phosphate Dehydrogenase Deficiency in Fars Province, Iran

Background: Absence or deficiency in Glucose-6-phosphate dehydrogenase (G6PD) enzyme in patients with G6PD deficiency presents with a wide spectrum of symptoms. This study evaluates the clinical features of acute hemolysis in children with G6PD deficiency. Materials and Methods: Seventy G6PD deficient children younger than 18 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 G6PD 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 69 and 68 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>0.05). 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>0.05). Conclusion: In this study, pallor, icterus and dark urine were the three important symptoms in G6PD 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.