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Genetic Analysis of Hereditary Transthyretin Ala97Ser Related Amyloidosis
Published on: June 9, 2018
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Pulmonary amyloidosis: A case series
Anil Kumar Gandham1, A R Gayathri1, L Sundararajan1
1Department of Respiratory Medicine, Apollo Hospital, Chennai, Tamil Nadu, India.
Lung India : Official Organ of Indian Chest Society
|April 30, 2019
Summary
Amyloidosis involves protein buildup in organs. This study highlights rare lung amyloidosis patterns, emphasizing the need for awareness due to poor prognosis and subtle CT findings.
Area of Science:
- Pulmonology
- Pathology
- Radiology
Background:
- Amyloidosis is a group of diseases characterized by extracellular deposition of misfolded proteins as beta-pleated sheets.
- Amyloid light-chain amyloidosis, a common subtype, often presents with multisystemic involvement, affecting approximately 50% of patients in the lungs.
- Computed tomography (CT) of the chest reveals three primary patterns of pulmonary amyloidosis: tracheobronchial, nodular parenchymal, and diffuse alveolar septal.
Observation:
- This report details two cases of pulmonary amyloidosis.
- One case illustrates diffuse alveolar septal pulmonary amyloidosis, an exceptionally rare manifestation with a grave prognosis.
- The second case presents the tracheobronchial pattern, typically caused by localized amyloid deposition within the airways.
Findings:
- Diffuse alveolar septal pulmonary amyloidosis is an extremely rare pattern with a poor prognosis.
- Tracheobronchial amyloidosis involves localized deposition in the airways.
- Pulmonary amyloidosis often exhibits nonspecific findings on CT chest, complicating diagnosis.
Implications:
- Increased awareness of pulmonary amyloidosis is crucial for timely diagnosis and management.
- Recognizing rare patterns like diffuse alveolar septal involvement is vital for prognostic accuracy.
- Understanding the varied CT manifestations of pulmonary amyloidosis aids in differentiating it from other lung diseases.
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