Novel Decision Tool for More Severe α-Thalassemia Genotypes Screening with Functional Loss of Two or More α-Globin Genes: A Diagnostic Test Study.

After the exclusion of iron deficiency and ß-thalassemia, molecular research for α-thalassemia is recommended to investigate microcytic anemia. Aiming to suggest more efficiently the molecular analysis for individuals with a greater chance of having a symptomatic form of the disease, we have developed and validated a new decision tool to predict the presence of two or more deletions of α-thalassemia, increasing considerably the pre-test probability. The model was created using the variables: the percentage of HbA2, serum ferritin and mean corpuscular volume standardized by age. The model was trained in 134 patients and validated in 160 randomly selected patients from the total sample. We used Youden's index applied to the ROC curve methodology to establish the optimal odds ratio (OR) cut-off for the presence of two or more α-globin gene deletions. Using the OR cut-off of 0.4, the model's negative predictive value (NPV) was 96.8%; the cut-off point accuracy was 85.4%; and the molecular analysis pre-test probability increased from 25.9% to 65.4% after the use of the proposed model. This tool aims to assist the physician in deciding when to perform molecular studies for the diagnosis of α-thalassemia. The model is useful in places with few financial health resources.

Helicobacter pylori associated to unexplained or refractory iron deficiency anemia: an Egyptian single-center experience

ABSTRACT Background: Refractory or unexplained iron deficiency anemia accounts for about 15% of all cases. The endoscopic gastrointestinal workup sometimes fails to establish the cause of iron deficiency anemia and a considerable proportion of patients regardless of risk category fail to respond to oral iron supplementation. The aim of the present study was to assess the etiological role of Helicobacter pylori infection in adult Egyptian patients with unexplained or refractory iron deficiency anemia. Methods: A case controlled study was composed of 104 iron deficiency anemia cases and 70 age- and gender-matched healthy controls. Patients were diagnosed with iron deficiency anemia according to hemoglobin, mean corpuscular volume, serum ferritin, and transferrin saturation. Upper and lower endoscopies were performed and active H. pylori infection was investigated by testing for the H. pylori antigen in stool specimens. Hematological response to H. pylori treatment with triple therapy together with iron therapy (n = 32) or only iron therapy (n = 32) were assessed in patients with H. pylori infection. Results: H. pylori infection was more prevalent in patients with unexplained or refractory iron deficiency anemia (61.5%). Of the different hematological parameters investigated, there was a significant correlation only between H. pylori infection and mean corpuscular volume (p-value 0.046). Moreover, there was a significant correlation between receiving triple therapy together with iron supplementation and improvements in the hematological parameters [hemoglobin (p-value < 0.001), mean corpuscular volume (p-value < 0.001), iron (p-value < 0.001) and serum ferritin (p-value < 0.001)] compared to receiving iron supplementation alone. Conclusions: Failing to test for H. pylori infection could lead to a failure to identify a treatable cause of anemia and could lead to additional and potentially unnecessary investigations. Furthermore, treatment of H. pylori infection together with iron supplementation gives a more rapid and satisfactory response.

Helicobacter pylori associated to unexplained or refractory iron deficiency anemia: an Egyptian single-center experience.

BACKGROUND: Refractory or unexplained iron deficiency anemia accounts for about 15% of all cases. The endoscopic gastrointestinal workup sometimes fails to establish the cause of iron deficiency anemia and a considerable proportion of patients regardless of risk category fail to respond to oral iron supplementation. The aim of the present study was to assess the etiological role of Helicobacter pylori infection in adult Egyptian patients with unexplained or refractory iron deficiency anemia. METHODS: A case controlled study was composed of 104 iron deficiency anemia cases and 70 age- and gender-matched healthy controls. Patients were diagnosed with iron deficiency anemia according to hemoglobin, mean corpuscular volume, serum ferritin, and transferrin saturation. Upper and lower endoscopies were performed and active H. pylori infection was investigated by testing for the H. pylori antigen in stool specimens. Hematological response to H. pylori treatment with triple therapy together with iron therapy (n = 32) or only iron therapy (n = 32) were assessed in patients with H. pylori infection. RESULTS: H. pylori infection was more prevalent in patients with unexplained or refractory iron deficiency anemia (61.5%). Of the different hematological parameters investigated, there was a significant correlation only between H. pylori infection and mean corpuscular volume (p-value 0.046). Moreover, there was a significant correlation between receiving triple therapy together with iron supplementation and improvements in the hematological parameters [hemoglobin (p-value < 0.001), mean corpuscular volume (p-value < 0.001), iron (p-value < 0.001) and serum ferritin (p-value < 0.001)] compared to receiving iron supplementation alone. CONCLUSIONS: Failing to test for H. pylori infection could lead to a failure to identify a treatable cause of anemia and could lead to additional and potentially unnecessary investigations. Furthermore, treatment of H. pylori infection together with iron supplementation gives a more rapid and satisfactory response.

Prevalence of anemia in schools of the metropolitan region of Curitiba, Brazil.

BACKGROUND: Anemia during childhood is one of the biggest public health problems worldwide, including Brazil. Insufficient or abnormal production of hemoglobin, loss of iron and excessive destruction of red blood cells are the most common causes of anemia. Among the reasons of anemia, iron deficiency accounts for 50% of anemia cases in developing countries. Affected individuals present a wide range of clinical problems, including delayed neuropsychomotor progression, impaired cellular immunity and reduction of intellectual capacity. This study aimed to evaluate the prevalence of anemia in children attending public schools in the metropolitan region of Curitiba, Paraná, Brazil. METHOD: A retrospective study was conducted of 409 children aged 8-12 years old included in an extension project of the Universidade Federal do Paraná. The results of complete blood count and hemoglobin electrophoresis of all children were evaluated. Anemia was considered when the hemoglobin levels were <11.5 g/dL. RESULTS: The prevalence of anemia was found to be 2.2% of the population studied, with hypochromic microcytic anemia being the most common type. Seven children had sickle cell trait and one had ß-thalassemia. CONCLUSION: The prevalence of anemia in this study was considered normal according the World Health Organization classification, which is different from the data found in other Brazilian regions.

Prevalence of anemia in schools of the metropolitan region of Curitiba, Brazil

ABSTRACT Background: Anemia during childhood is one of the biggest public health problems worldwide, including Brazil. Insufficient or abnormal production of hemoglobin, loss of iron and excessive destruction of red blood cells are the most common causes of anemia. Among the reasons of anemia, iron deficiency accounts for 50% of anemia cases in developing countries. Affected individuals present a wide range of clinical problems, including delayed neuropsychomotor progression, impaired cellular immunity and reduction of intellectual capacity. This study aimed to evaluate the prevalence of anemia in children attending public schools in the metropolitan region of Curitiba, Paraná, Brazil. Method: A retrospective study was conducted of 409 children aged 8-12 years old included in an extension project of the Universidade Federal do Paraná. The results of complete blood count and hemoglobin electrophoresis of all children were evaluated. Anemia was considered when the hemoglobin levels were <11.5 g/dL. Results: The prevalence of anemia was found to be 2.2% of the population studied, with hypochromic microcytic anemia being the most common type. Seven children had sickle cell trait and one had β-thalassemia. Conclusion: The prevalence of anemia in this study was considered normal according the World Health Organization classification, which is different from the data found in other Brazilian regions.

Anemia and hyperplastic gastritis in a giant anteater (Myrmecophaga tridactyla) due to Physaloptera magnipapilla parasitism

A free living female adult of Myrmecophaga tridactyla was captured in an area of deforestation in Salta Province of northwest Argentina, for subsequent relocation in Iberá Park. Three days after entering the quarantine it died with clinical signs of anorexia and ascites. It presented severe hypochromic microcytic anemia, hypoproteinemia and increase of CPK, AST and LDH on blood analysis. Grossly, there were ascites, hydrothorax, pulmonary congestion and severe catarrhal hyperplasic gastritis. The gastric mucosa was diffusely hyperemic with two clusters of numerous Physaloptera magnipapilla attached in the fundic region. Fundic mucosa was irregular with convoluted surface and covered with abundant brown-yellow catarrhal exudates. Microscopically, the gastric mucosa had well described punctate areas (pit-like structures) at points of P. magnipapilla attachment. Additionally, there were mucous hyperplasia of gastric glands and focal fibrosis with scarce infiltration of plasma cells, lymphocytes, multinucleated giant cells and eosinophils in the underlying lamina propria. This report describes a case of anemia and hyperplastic gastritis due to Physaloptera magnipapilla parasitism in a free-living Myrmecophaga tridactyla.

Anemia and hyperplastic gastritis in a giant anteater (Myrmecophaga tridactyla) due to Physaloptera magnipapilla parasitism

A free living female adult of Myrmecophaga tridactyla was captured in an area of deforestation in Salta Province of northwest Argentina, for subsequent relocation in Iberá Park. Three days after entering the quarantine it died with clinical signs of anorexia and ascites. It presented severe hypochromic microcytic anemia, hypoproteinemia and increase of CPK, AST and LDH on blood analysis. Grossly, there were ascites, hydrothorax, pulmonary congestion and severe catarrhal hyperplasic gastritis. The gastric mucosa was diffusely hyperemic with two clusters of numerous Physaloptera magnipapilla attached in the fundic region. Fundic mucosa was irregular with convoluted surface and covered with abundant brown-yellow catarrhal exudates. Microscopically, the gastric mucosa had well described punctate areas (pit-like structures) at points of P. magnipapilla attachment. Additionally, there were mucous hyperplasia of gastric glands and focal fibrosis with scarce infiltration of plasma cells, lymphocytes, multinucleated giant cells and eosinophils in the underlying lamina propria. This report describes a case of anemia and hyperplastic gastritis due to Physaloptera magnipapilla parasitism in a free-living Myrmecophaga tridactyla.(AU)

Utility of new mature erythrocyte and reticulocyte indices in screening for iron-deficiency anemia in a pediatric population.

INTRODUCTION: Iron-deficient erythropoiesis attributable to nutritional deficiency is the most common cause of anemia in early childhood. Beckman Coulter has proposed new parameters: red blood cell size factor (RSf) and low hemoglobin density (LHD%). [Formula: see text] The aims of the present communication were to establish reference ranges (RR) for RSf and LHD% in a healthy pediatric population; to evaluate clinical utility of these parameters in the diagnosis of iron-deficiency anemia (IDA); and to compare RSf and LHD% with RET-He. METHODS: Two hundred healthy infants and 51 with IDA were analyzed on Coulter LH750 and Sysmex XT2000i analyzers. RESULTS: RR for RSf in children aged 0.5-6 years, 82.2-102.0 and 83.7-103.1 fl in the group aged 6-18 years. RR for LHD% was 1.1-9.0%. ROC analysis for RSf and LHD% in the diagnosis of IDA showed an AUC 0.8460 and 0.8654, respectively. The best RSf value to detect a restricted erythropoiesis was 89fl, sensitivity 92%, specificity 81%. LHD% optimal cutoff point was 6.0%, sensitivity 80%, and specificity 88%. Good correlation was observed between these parameters with Ret-He, r = 0.888 and r = -0.790, respectively. CONCLUSION: RSf and LHD% could be useful tools in the screening of IDA. These parameters can be obtained in the course of routine blood counts, with no additional cost or need for more blood sampling.