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Molecular Spring Constant Analysis by Biomembrane Force Probe Spectroscopy
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Clinical Course of Homozygous Hemoglobin Constant Spring in Pediatric Patients

Patcharee Komvilaisak1, Arunee Jetsrisuparb1, Goonnapa Fucharoen2

  • 1Departments of Pediatrics.

Journal of Pediatric Hematology/Oncology
|April 19, 2018
PubMed

Insights

Hemoglobin Constant Spring (Hb CS) causes severe anemia in infants, often requiring transfusions. However, anemia typically becomes mild after a few months, with most children not needing further transfusions.

Area of Science:

  • Hematology
  • Genetics

Background:

  • Hemoglobin (Hb) Constant Spring is an alpha-globin gene variant.
  • It results from a stop codon mutation, elongating the polypeptide chain.
  • Homozygous Hb Constant Spring typically causes mild anemia.

Purpose of the Study:

  • To describe clinical manifestations, diagnosis, and treatment of pediatric homozygous Hb Constant Spring.
  • To report associated findings in affected children.

Main Methods:

  • Retrospective review of pediatric patients with homozygous Hb Constant Spring.
  • Analysis of clinical data, laboratory investigations, and treatment outcomes.

Main Results:

  • Sixteen pediatric cases were analyzed, with varying Hb Constant Spring genotypes.
  • Early neonatal jaundice and anemia requiring phototherapy or transfusions were common.
  • Most patients developed mild anemia with increased reticulocyte count and wide RDW post-infancy, without needing further transfusions.
  • Associated conditions included congenital heart disease, genitourinary abnormalities, and hypothyroidism.

Conclusions:

  • Pediatric homozygous Hb Constant Spring can cause severe fetal and early neonatal anemia.
  • Anemia typically improves to mild levels after infancy, with normal Hb levels and no need for transfusions.
  • Associated congenital abnormalities require monitoring and management.
Abstract

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