Kurrus et al reported one of the first intentional uses of external beam radiation therapy for TBA. The patient was initially observed until the development of life-threatening, progressive obstruction. External beam radiation therapy was then delivered to the distal trachea and right mainstem bronchus using a dose of 20 Gy in 10 fractions with improvement. Six months later, the patient underwent external beam radiation therapy to the right lower lobe bronchi because of disease progression in this area. As in our case, there was evidence of bronchoscopic and radiographic improvement in the radiated fields several months after treatment. …click here to read more
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Management has primarily consisted of debulking procedures and laser ablation. Unfortunately, such methods may be ineffective and repeated procedures may be required. Capizzi et al reported 17 patients with TBA who were treated with laser or forceps resection, systemic therapy, radiation, or observation. Of 13 patients with follow-up data, 6 patients were alive with recurrence, and 5 patients had died (recurrent respiratory compromise secondary to amyloid [n = 2] and multiple myeloma [n = 1]). Two patients remained alive with stable disease. Hui et al reported on 14 patients with tracheobronchial amyloidosis. Of the seven patients with an average follow-up of 4.8 years, three patients died of recurrent disease. Similarly, of the 10 patients recently reported by O’Regan et al at Boston University, 3 patients died of respiratory failure after numerous excisions, 2 patients have progressive disease after 11 years and 6 years of follow-up, respectively, and 1 patient requires mechanical ventilation. A review of the literature by Rubinow et al revealed that 11 of 39 patients (28%) died of respiratory causes. …click here to read more
In October 2001 and July 2002, CT scans of the chest demonstrated progressive resolution of thickening in the right mainstem bronchus and marked improvement in right lower lobe airspace disease/atelectasis. The most recent CT scan of the chest obtained in November 2002 revealed a continued improvement in the thickening of the right mainstem bronchus and near-complete resolution of right lower lobe atelectasis (Fig 4). The most recent pulmonary function tests were performed in December 2002 and demonstrated an FEV1 of 1.57 L (69% of predicted), FVC of 2.37 L (72% of predicted), FEV1/FVC ratio of 68%, and diffusing capacity of 13.23 mL/min/mm Hg (60% of predicted).
The patient currently has minimal respiratory symptoms and has not required supplemental oxygen for the previous 7 months. The colchicine has been continued at a dose of 0.6 mg bid since diagnosis. …click here to read more
The patient was thought not to be a candidate for laser resection or stent placement given the diffuse involvement of the airways and the extent of the disease into the distal airways. Due to the lack of therapeutic options, it was elected to provide a trial of external beam radiotherapy. Beginning in July 2001, the patient received localized external beam radiation therapy via parallel-opposed anteroposterior-posteroanterior fields. CT simulation was used to plan treatment fields that covered the radiographically defined target with a 2.5- to 3-cm margin. The dose of 24 Gy was delivered in 12 daily fractions using 6-megavolt (MV) photons. In addition, the patient was started on colchicine, 0.6 mg bid. …click here to read more
A CT scan of the chest demonstrated a central lung mass at the level of the right mainstem bronchus. Flexible bronchoscopy revealed thickening of the distal trachea and obstruction of the right mainstem bronchus by a tumor-like exophytic mass. Pathology from endobronchial biopsies revealed bronchial mucosa with squamous metaplasia. Rigid bronchoscopy was performed and demonstrated the same finding of occlusion of the right main-stem bronchus but was complicated by significant bleeding during the procedure, requiring mechanical ventilation.
The patient was subsequently transferred to our institution. CT of the chest was repeated, revealing significant circumferential thickening with partial calcification of the right mainstem bronchus extending inferiorly to obliterate the right lower lobe bronchus (Fig 1). A 3 X 3-mm soft-tissue mass with an intraluminal component partially obstructing the right upper lobe bronchus was also identified. …click here to read more
Amyloidosis is a disorder of extracellular protein deposition that encompasses a wide spectrum of related diseases. The underlying pathology involves abnormal protein folding and results in insoluble amyloid fibril proteins accumulating in normal tissues. Various subtypes exist, including primary systemic amyloidosis, reactive systemic amyloidosis associated with chronic inflammatory states, and localized forms of aberrant amyloid deposition.
Tracheobronchial amyloidosis (TBA) refers to the deposition of localized amyloid deposits within the upper airways and is typically associated with monoclonal Ig light chains.2
The clinical effects of TBA include dyspnea, cough, hemoptysis, and hoarseness. Pulmonary function abnormalities depend on the location of involvement with proximal deposition typically causing obstructive physiology and mid/distal disease demonstrating normal airflow rates and air trapping. …click here to read more
Continuous Noninvasive Measurement of Pulsus Paradoxus Complements Medical Decision Making in Assessment of Acute Asthma Severity: Limitations
In reality, the complexities of optical plethysmography, along with their inherent noise, may preclude the utility of a simple relationship in a pulse oximeter that also displays PP. Filtering and straightforward signal-processing methods like least mean squares approximation and best uniform approximation will be needed to take this next step.
Expert systems have appeared in many areas of health care. Examples include the bispectral index monitor, which gauges the depth of general anesthesia or conscious sedation, and the time-insensitive predictive instrument score, which measures the shape and elevation of the ST segment on 12-lead ECGs. These tools are especially useful in the context of emergency medicine by objectifying a subjective clinical assessment and reassessing a preconceived probability. An instrument like a PP monitor could serve as a patient management decision aid or in the detection of cardiopulmonary dyscrasias. buy zoloft online
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