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[Skeletal changes in thalassemia].

H Alaiyan1, H Mittelmeier, N Graf

  • 1Orthopädische Universitätsklinik Homburg/Saar.

Zeitschrift Fur Orthopadie Und Ihre Grenzgebiete
|July 1, 1988
PubMed
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This study presents two cases of a rare hereditary blood disease (haemoglobinopathy) causing early red blood cell destruction and organ damage. It highlights complications like bone issues and growth disturbances in affected individuals.

Area of Science:

  • Hematology
  • Genetics
  • Pediatrics

Background:

  • Haemoglobinopathies are inherited blood disorders.
  • Mediterranean fever is endemic in the Mediterranean region.
  • This condition is rarely observed in other geographical areas.

Observation:

  • Two typical cases of haemoglobinopathy are presented.
  • The disease is characterized by early destruction of red blood cells.
  • Patients exhibit transfusion siderosis in internal and endocrine organs.

Findings:

  • Secondary osteoporosis is a noted complication.
  • Epiphyseal growth disturbances are observed.
  • Spontaneous fractures occur in affected individuals.

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Implications:

  • Early diagnosis and management are crucial for mitigating complications.
  • Understanding the pathophysiology aids in developing targeted therapies.
  • Increased awareness is needed in non-endemic areas for timely intervention.