Related Experiment Video

Updated: Aug 8, 2026

Staphylococcus aureus Growth using Human Hemoglobin as an Iron Source
06:37

Staphylococcus aureus Growth using Human Hemoglobin as an Iron Source

Published on: February 7, 2013

Hemoglobin alpha chain deficiency in black children with variable quantities of hemoglobin Bart's at birth

C Altay, B Ringelhann, G I Yawson

    Pediatric Research
    |February 1, 1977
    PubMed

    Insights

    This study investigated alpha-thalassemia in children, finding that higher levels of Hemoglobin Bart's at birth correlate with decreased red blood cell size and hemoglobin content, indicating a familial alpha chain deficiency.

    Area of Science:

    • Genetics
    • Hematology

    Background:

    • Alpha-thalassemia is a genetic blood disorder characterized by reduced or absent synthesis of alpha-globin chains.
    • It can lead to various clinical presentations, from asymptomatic carriers to severe anemia.

    Purpose of the Study:

    • To investigate the hematologic findings and globin chain synthesis in children with varying levels of Hemoglobin Bart's (Hb Bart's) at birth.
    • To determine the familial inheritance pattern of alpha chain deficiency.

    Main Methods:

    • Hematologic studies including mean corpuscular volume (MCV) and mean corpuscular hemoglobin (MCH) were performed.
    • Globin chain synthesis analysis was conducted on 21 children (aged 2-6 years), their parents, and controls.
    • Beta/alpha globin chain synthesis ratios were calculated.

    Main Results:

    • Children with approximately 5% Hb Bart's at birth showed significantly decreased MCV and MCH, and increased beta/alpha ratios.
    • Children with 2% Hb Bart's had increased beta/alpha ratios but lacked microcytosis and hypochromia.
    • Alpha chain deficiency was found to be familial, with some parents exhibiting increased alpha/alpha ratios and suggestive hematologic findings.

    Conclusions:

    • Elevated Hb Bart's levels at birth are associated with specific hematologic changes indicative of alpha chain deficiency.
    • The observed alpha chain deficiency demonstrates a familial inheritance pattern.
    • The study highlights the utility of Hb Bart's levels at birth as an indicator of alpha-thalassemia severity and inheritance.

    More Related Videos

    Detection of Residual Donor Erythroid Progenitor Cells after Hematopoietic Stem Cell Transplantation for Patients with Hemoglobinopathies11:59

    Detection of Residual Donor Erythroid Progenitor Cells after Hematopoietic Stem Cell Transplantation for Patients with Hemoglobinopathies

    Published on: September 6, 2017

    Fingerprinting Cardiolipin in Leukocytes by Mass Spectrometry for a Rapid Diagnosis of Barth Syndrome06:48

    Fingerprinting Cardiolipin in Leukocytes by Mass Spectrometry for a Rapid Diagnosis of Barth Syndrome

    Published on: March 23, 2022

    Related Concept Videos

    Multiple Allele Traits01:49

    Multiple Allele Traits

    The Concept of Multiple Allelism
    Blood Types02:20

    Blood Types

    Human blood is classified into different types based on the presence of antigens on the red blood cell's surface and antibodies in the plasma. Proper identification of blood type is essential for successful blood transfusion. The International Society of Blood Transfusion has identified 38 human blood types based on the surface antigens on the red blood cells. The most common types are ABO, Rh, and MNS blood types.
    ABO blood group
    ABO antigens are glycoproteins encoded by genes present on...
    Hemoglobin01:24

    Hemoglobin

    Hemoglobin is a globular protein made up of four subunits. Two of these subunits are alpha chains, and the other two are beta chains. Each subunit contains a molecule of heme, which has an iron atom and can bind to oxygen. When an oxygen molecule binds to one heme group, it changes the shape of hemoglobin, making it easier for the other heme groups to bind oxygen as well.
    When all four heme groups are bound to oxygen, the resulting molecule is called oxyhemoglobin. As a result, arterial blood...
    Disorders of Erythrocytes01:27

    Disorders of Erythrocytes

    Disorders of erythrocytes, or red blood cells (RBCs), include a range of conditions affecting their number, shape, or function.
    Erythrocyte disorders can be broadly categorized into two main types: anemic and polycythemic conditions.
    A low oxygen-carrying capacity of the blood due to the loss, lower production, or destruction of erythrocytes is termed anemia. Hemorrhagic anemia, for example, occurs when bleeding from an external wound or internal ulcer reduces erythrocyte counts.
    On the other...
    Oxygen Transport in the Blood01:27

    Oxygen Transport in the Blood

    Hemoglobin (Hb) is a crucial molecule in the human body, consisting of four polypeptide chains, each bound to an iron-containing heme group. This unique structure enables hemoglobin to bind to oxygen, with each molecule capable of combining with four molecules of oxygen, leading to rapid and reversible oxygen loading. When fully loaded with oxygen, it is called oxyhemoglobin, while hemoglobin that has released oxygen is called reduced hemoglobin or deoxyhemoglobin. As hemoglobin binds oxygen,...
    Inborn Errors of Metabolism01:20

    Inborn Errors of Metabolism

    Phenylketonuria (PKU) is a protein metabolism disorder characterized by high blood levels of the amino acid phenylalanine. This results from a mutation in the gene responsible for phenylalanine hydroxylase, an enzyme that converts phenylalanine into tyrosine. When this enzyme is deficient, phenylalanine builds up in the blood, leading to symptoms such as vomiting, rashes, seizures, growth deficiency, and severe mental retardation. An early diagnosis and a diet restricting phenylalanine intake...