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J Thorac Cardiovasc Surg 2004;128:789-792
© 2004 The American Association for Thoracic Surgery

Brief communication

Tracheobronchial amyloidosis: A surgical disease with long-term consequences

^a Department of Surgery, Division of Cardiothoracic Surgery, University of Iowa, Iowa City, Iowa, USA
^b Department of Internal Medicine, University of Iowa, Iowa City, Iowa, USA
^c Department of Pathology, University of Iowa, Iowa City, Iowa, USA

Received for publication February 25, 2004; accepted for publication March 10, 2004.

^* Address for reprints: Kemp H. Kernstine, MD, PhD, University of Iowa Hospitals and Clinics, Division of Cardiothoracic Surgery, 200 Hawkins Dr, Room 1616B-JCP, Iowa City, IA 52242, USA
kemp-kernstine@uiowa.edu

The first 300 words of the full text of this article appear below.

Amyloidosis results from abnormal protein deposition and accumulation in extracellular spaces.¹ The multiple distinct forms of this disease have heterogeneous clinical manifestations and variable patterns of organ involvement. Amyloidosis might be hereditary or acquired, localized, or systemic and can range from an incidental asymptomatic finding to a lethal disorder. The diseases are distinguished on a molecular level by the type of peptide subunits that compose abnormally accumulated protein fibrils. Although these peptide fibrils differ, all amyloid fibrils adopt a similar ultrastructure rich in ß-sheet content. The fibril deposits also have a common association with certain glycosaminoglycans (GAGs), notably heparin and dermatan sulphate. Normal plasma proteins, such as serum amyloid P (SAP), function to stabilize the amyloid fibril.¹ We present a case of severe tracheobronchial amyloidosis (TBA) with the results of our evaluation and management strategies.

Clinical summary

A 49-year-old white man had a 6-year history of progressive dyspnea and productive cough. He had an accelerated symptom progression over the prior 6 months and also noted the new onset of intermittent malaise, myalgias, arthralgias, fever, chills, and night sweats. He had been treated with parenteral and oral antibiotics, as well as a variety of inhaled bronchodilators for suspected pulmonary infections. Because he experienced only transient improvement, a combination of prednisone, fluticasone, albuterol, salmeterol, and ipratropium were added, but these did not improve his symptoms.

Past medical history was remarkable for recurrent pneumonia and bronchitis treated empirically. He had a 30 pack-year smoking history but quit smoking 11 years previously.

Hepatic, renal, and thyroid function test results were all normal, as were electrolyte levels and hemography results. Pulmonary function tests were as follows: forced vital capacity, 4.95 L (101% of predicted value); forced expiratory volume in 1 second, 4.02 L (88% of predicted value); total lung capacity, 7.06 L (97% of predicted value); residual . . . [Full Text of this Article]