Iron deficiency, still a rarity in children with sickle cell anemia in Ile-Ife, Nigeria

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.

Avaliação do estado nutricional de ferro e anemia em crianças menores de 5 anos de creches públicas / Nutritional assessment of iron status and anemia in children under 5 years old at public daycare centers

OBJETIVO: Avaliar o estado nutricional de ferro e a prevalência de anemia em crianças menores de 5 anos de creches públicas da cidade do Recife (PE). MÉTODOS: Estudo transversal, com amostra aleatória sistemática de 162 crianças, de 6 a 59 meses. O estado nutricional de ferro foi avaliado em termos de reservas corporais (ferritina sérica), transferrinemia (ferro sérico, capacidade total de ligação do ferro e por cento de saturação da transferrina), eritropoiese (protoporfirina eritrocitária livre) e hemoglobinogênese (hemoglobina). RESULTADOS: A prevalência de anemia (hemoglobina < 11,0 g/dL) foi de 55,6 por cento (IC95 por cento 47,3-63,5), a redução dos estoques de ferro (ferritina sérica < 12,0 ng/mL) foi evidenciada em 30,8 por cento (IC95 por cento 22,9-39,3), baixa transferrinemia ( por cento de saturação da transferrina < 16) em 60,1 por cento (IC95 por cento 51,7-68,0) e eritropoiese deficiente (protoporfirina eritrocitária livre > 40 æmol/mol heme) em 69,6 por cento (IC95 por cento 61,0-77,1) das crianças. Os parâmetros de ferro não apresentaram correlação com o gênero (p > 0,05). No entanto, crianças < 24 meses apresentaram concentrações mais baixas de hemoglobina (p < 0,00) e níveis mais elevados de protoporfirina eritrocitária livre (p < 0,000) e de capacidade total de ligação do ferro (p < 0,001), quando comparadas às crianças > 24 meses. A significante correlação observada entre reserva, transferrinemia e eritropoiese representa achado compatível com o esperado ciclo de vida do ferro no organismo. CONCLUSÕES: A deficiência de ferro e a anemia parecem ser um importante problema de saúde pública entre as crianças menores de 5 anos de creches públicas do Recife. Logo, ações efetivas direcionadas à prevenção e ao controle dessa deficiência são fortemente recomendadas nesse contexto ecológico.

OBJECTIVE: To assess nutritional iron status and anemia prevalence in children less than 5 years old at public daycare centers in the city of Recife, PE, Brazil. METHODS: A cross-sectional study, with a systematic random sampling of 162 children aged 6 to 59 months. Nutritional iron status was assessed in terms of body iron reserves (serum ferritin), transferrinemia (serum iron, total iron binding capacity, and transferrin saturation percent), erythropoiesis (free erythrocyte protoporphyrin) and hemoglobin production (hemoglobin). RESULTS: The prevalence of anemia (hemoglobin < 11.0 g/dL) was 55.6 percent (95 percentCI 47.3-63.5), evidence was found of depleted iron stocks (serum ferritin < 12.0 ng/mL) in 30.8 percent (95 percentCI 22.9-39.3), low transferrinemia levels (transferrin saturation percent < 16) in 60.1 percent (95 percentCI 51.7-68.0) and deficient erythropoiesis (free erythrocyte protoporphyrin > 40 æmol/mol heme) in 69.6 percent (95 percentCI 61.0-77.1) of the children. Iron parameters were not correlated with sex (p > 0.05). However, children < 24 months exhibited lower hemoglobin concentrations (p < 0.00) and higher levels of free erythrocyte protoporphyrin (p < 0.000) and total iron binding capacity (p < 0.001) when compared with children > 24 months. The significant correlation observed between reserves, transferrinemia and erythropoiesis is a finding that is compatible with the expected lifecycle of iron in the body. CONCLUSIONS: Iron deficiency and anemia appear to be an important public health problem among children less than 5 years old at public daycare centers in Recife. Therefore, effective actions aimed at the prevention and control of this deficiency are strongly recommended in this ecological context.

Free Erythrocyte Protoporphyrin for Diagnosis of Iron Deficiency and Iron Deficiency Anemia in Adolescent Female Athletes / 대한혈액학회지

BACKGROUND: If hemoglobin (Hb) synthesis is impaired by factors other than a deficiency in free erythrocyte protoporphyrin (FEP) synthesis, the amount of FEP might be increased. In this study, we analyzed the statistical values and contribution of FEP for the monitoring and diagnosis of iron deficient anemia in adolescent female athletes according to various sports. METHODS: We collected whole blood from 64 adolescent female athletes 13 to 19 years of age. The FEP was measured fluorometrically. After other hematological indices were evaluated, statistical analysis was performed to compare the data among various athletes. RESULTS: The mean age was 14.8+/-1.7 (13~19) years old. The number of runners, badminton players and shooting athletes were 46.9% (n=30), 12.5% (n=8) and 40.6% (n=26), respectively. The prevalence of anemia, iron deficiency and iron deficiency anemia were 23.4%, 23.4% and 14.0%, respectively. The measured concentration of FEP was 48.7+/-21.1 microgram/dL (12~125). A moderately negative correlation of Hb and FEP was noted and was found to be statistically significant (r=-0.571, P<0.001). Among serum ferritin, TS and FEP, there was no statistically significant correlation. For the diagnosis of iron deficiency anemia, FEP was the most statistically significant index (P<0.001). For iron deficiency, sensitivity, specificity, positive predictive value, and negative predictive value were 88.9%, 30.4%, 33.3%, and 87.5%, respectively. The receiver operating characteristic curves, showed that FEP had excellent diagnostic power to detect iron deficiency. There was a significant difference in the prevalence of iron deficiency among the three athletes, with runners and badminton players tending to be affected more frequently with iron deficiency than static athletes such as the shooters (runners and badminton vs. shooting athletes, 33.3% and 25.0% vs. 19.2%). CONCLUSION: Our results confirmed FEP to be the most significant factor for the diagnosis of iron deficiency in athletes. Proper nutritional counseling and monitoring need to be tailored to various sports, especially in terms of static versus nonstatic sports such as runners and badminton players versus shooting athletes.

Effect of estradiol on heme biosynthetic pathway in lead-poisoned rabbits

<p><b>OBJECTIVES</b>To clarify the effect of the female hormone estradiol (Est) on heme biosynthesis in lead-poisoned rabbits, parameters indicating lead exposure, such as free erythrocyte protoporphyrin (FEP) level and δ-aminolevulinic acid dehydratase (ALA-D) activity, were determined.</p><p><b>METHODS</b>Twenty-six male Japanese white rabbits (body weight (BW), 3kg) were divided into four groups: I (control), II (Est), III (Pb), IV (Est+Pb). About 3 weeks after castration, Est (3 mg/kg of BW) was injected intramuscularly, and 2 weeks thereafter, lead (1.2 mg/kg of BW) was injected intravenously. After the initial injection of each of these substances, the same dose of each of these substances was injected once a week until the 9th week.</p><p><b>RESULTS</b>In groups III and IV, FEP level increased and ALA-D activity in the erythrocytes, bone marrow and liver decreased with an increase in lead concentration in blood. FEP level decreased significantly (p<0.01) in the 8th and 10th weeks after Est injection in group IV compared to with that in group III and was not elevated in group II compared with that in group I. ALA-D activity in the erythrocytes, bone marrow and liver increased significantly in group II compared with that in group I, whereas Ht and Hb levels decreased in group II compared with those in group I, and decreased in group IV compared with those in group III. The level of iron in plasma (Fe-P) was within the normal range during experiment.</p><p><b>CONCLUSIONS</b>In this study, Est did not increase FEP level. From the above results regarding FEP level and ALA-D activity, Est may prevent an increase in FEP level caused by lead. Ht and Hb levels, which are the parameters of anemia, decreased mainly as a result of Est exposure rather than lead exposure.</p>

Effect of Estradiol on Heme Biosynthetic Pathway in Lead-Poisoned Rabbits

Objectives: To clarify the effect of the female hormone estradiol (Est) on heme biosynthesis in lead-poisoned rabbits, parameters indicating lead exposure, such as free erythrocyte protoporphyrin (FEP) level and δ-aminolevulinic acid dehydratase (ALA-D) activity, were determined. Methods: Twenty-six male Japanese white rabbits (body weight (BW), 3 kg) were divided into four groups: I (control), II (Est), III (Pb), IV (Est+Pb). About 3 weeks after castration, Est (3 mg/kg of BW) was injected intramuscularly, and 2 weeks thereafter, lead (1.2 mg/kg of BW) was injected intravenously. After the initial injection of each of these substances, the same dose of each of these substances was injected once a week until the 9th week. Results: In groups III and IV, FEP level increased and ALA-D activity in the erythrocytes, bone marrow and liver decreased with an increase in lead concentration in blood. FEP level decreased significantly (p<0.01) in the 8th and 10th weeks after Est injection in group IV compared to with that in group III and was not elevated in group II compared with that in group I. ALA-D activity in the erythrocytes, bone marrow and liver increased significantly in group II compared with that in group I, whereas Ht and Hb levels decreased in group II compared with those in group I, and decreased in group IV compared with those in group III. The level of iron in plasma (Fe-P) was within the normal range during experiment. Conclusions: In this study, Est did not increase FEP level. From the above results regarding FEP level and ALA-D activity, Est may prevent an increase in FEP level caused by lead. Ht and Hb levels, which are the parameters of anemia, decreased mainly as a result of Est exposure rather than lead exposure.

A Case of Erythropoietic Protoporphyria / 대한피부과학회지

Erythropoietic protoporphyria(EPP) is an inherited inborn error of porphyrin metabolism caused by decreased activity of the enzyme ferrochelatase. EPP is characterized clinically by photosensitivity to visible light commencing in childhood, and biochemically by elevated red cell free protoporphyrin levels. We report herein a case of EPP which occurred in a 44-year-old man and his family. He had suffered from immediate photosensitivity since he was 4 years old. He was presented with burning, erythema, scars and waxy thickening of the sun-exposed skin. Red cell free protoporphyrin level was elevated and urinary porphyrins were normal. Histopathologically, homogeneous eosinophilic materials that stained with PAS were deposited in perivascular area of upper dermis. He was managed with light restriction and sunscreen.

A case of erythropoietic protoporphyria / 대한피부과학회지

Erythropoietic protoporphyria, sometimes also called erythrohepitic protoporphyria or simple protoporphyria, is a heritable detect of heme synthesis in which the last enzyme of the heme synthetic pathway, ferrochelatase(or heme synthetase), is functioning subopt,imally. A 23-year-old male has experi nced erythema and edema on the fae and hands during or immediately after sun exposure, since 3 years of age. The skin lesions have been accompanied by severe itching, a buring sensation and pain. Severe episodes were followed hy head iche and vomiting. We have seen a case of erythropoietic protoporphyria presenting clinically and histo athologically, wit.h a skin lesion on the sun exposured area, and free erthrocyte protoporphyrin serologically.

Erythropoietic Protoporphyria in a Family

Erythropoietic protoporphyria (EPP) is an autosomal dominant condition due to decreased activity of ferrochelatase. The disease is characterized by a wide range of photocutaneous changes and occasionally by liver disease. The level of protoporphyin is raised in erythkocytes and it may also be increased in the feces. We report herein a case of EPP present in a family which was diagnosed by a high free erythrocyte protoporphyrin (FEP) count.

Erythropoietic Protoporphyria in a Family

Erythropoietic protoporphyria (EPP) is an autosomal dominant condition due to decreased activity of ferrochelatase. The disease is characterized by a wide range of photocutaneous changes and occasionally by liver disease. The level of protoporphyin is raised in erythkocytes and it may also be increased in the feces. We report herein a case of EPP present in a family which was diagnosed by a high free erythrocyte protoporphyrin (FEP) count.

DISTRIBUTION AND NORMAL VALUE OF FREEERYTHROCYTE PROTOPORPHYRIN IN NON-IRONDEFICIENT INFANTS AND CHILDREN / 营养学报

0.05, but there was a larger deviation with Model 930.