No abstract

In this study, children with sickle cell anemia were evaluated for iron deficiency. Serum ferritin and free erythrocyte protoporphyrin free erythrocyte protoporphyrin (FEP) levels, mean corpuscular volume mean corpuscular volume (MCV) and mean corpuscular hemoglobin mean corpuscular hemoglobin (MCH) were used in determining their iron status. The study was done at Pediatric Hematology Outpatient Clinic of the Obafemi Awolowo University Teaching Hospitals’ Complex, Ile-Ife. Forty-eight HbSS subjects in steady state and 48 apparently well age and sex matched HbAA controls were evaluated. Serum ferritin less than 25 ng/dL FEP greater than cut off for age, mean corpuscular volume MCV and mean corpuscular hemoglobin MCH less than cut off for age were regarded as indicating iron deficiency. Serum ferritin values ranged from 34.2 to 3282.9 μg/L, with a mean of 381.2 (1.0), median 180 μg/L; which was significantly higher than the controls ( p = 0.000). FEP was lower in the subjects but none was iron deficient compared with the controls. The mean corpuscular hemoglobin MCH of subjects was significantly lower than the controls. Subjects had lower mean corpuscular volume MCV compared with controls. Iron deficiency was not detected in any of the subjects with sickle cell anemia in comparison to a prevalence of 43.75% in the controls. Iron deficiency anemia (IDA) was found in 16.7% of the controls, using the WHO cut off for anemia which is hemoglobin concentration of <11 g/dl. While a high prevalence of iron deficiency was noted in the control group, patients with sickle cell anemia were largely iron sufficient, despite their anemia. Iron supplementation remains unnecessary as part of routine management of children with sickle cell anemia in our practice.

No abstract

Purpura fulminans (PF) is a rapidly progressing clinical syndrome of haematologic and cutaneous manifestations accompanied by an underlying dysfunction of coagulation resulting in disseminated intravascular coagulation (DIC). It is a life threatening haematologic emergency characterized by extensive skin necrosis with haemorrhagic infarction, hypotension and gangrene which may arise from severe sepsis, mostly gram negative organisms. Some gram positive organisms and viral infections have been implicated in the aetiology of PF. We reported a case of purpura fulminans in a 2 year old boy with severe sepsis and peripheral gangrene from gram positive coccus (Staphylococcus aureus). Even though we were faced with limitations in terms of laboratory support and parental financial constraints in the management of the patient; he survived mainly on supportive care and antibiotics.