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Primary laryngeal amyloidosis is a rare benign disease of unknown aetiology. It can present with dysphonia or stridor. A woman presenting with airway compromise, who required a tracheostomy, is reported. (Postgrad Med J 2000;76:364-365) Keywords: laryngeal disease; amyloidosis Case report A 47 year old woman presented with a seven year history of dysphonia which had deteriorated recently. Fibre optic laryngoscopy showed polyps on the right false vocal cord with a suggestion of subglottic stenosis (fig 1). The true vocal cords were mobile and appeared normal.While awaiting further investigation, she presented with airway compromise requiring urgent tracheostomy. Direct laryngoscopy revealed subglottic oedema. The polypoid lesion was biopsied and showed features suggestive of amyloidosis and this was confirmed after staining with Congo red. A flexible bronchoscopy through the tracheostomy tube showed isolated nodules within the trachea (fig 2). Full blood count and erythrocyte sedimentation rates were normal.At follow up a year later she was noted to have a change in voice. Fibre optic laryngoscopy showed new lesions on the right and left true vocal cords. These were resected with a carbon dioxide laser. The result was a subjective improvement in the quality of her voice and airway. She was decannulated two months later. She remains under review and requires no further intervention. DiscussionAmyloidosis is a benign, slowly progressive condition that is characterised by extracellular fibular proteins. Diagnosis is confirmed by histopathological specimens that stain with Congo red.1 Amyloidosis can be classified as either primary, developing spontaneously, or secondary to some other longstanding inflammatory disease such as rheumatoid arthritis. The primary form can be further subdivided into a localised form, where deposits are confined to a single organ or location, or generalised, where deposits are found to some extent in all tissues.The most common presenting symptoms of primary laryngeal amyloidosis are dysphonia and stridor. Rarely, airway compromise occurs and an alternative airway may be necessary. The presence of tender bones, lymphadenopathy, or splenomegaly should alert the clinician to the possibility of generalised amyloidosis. Learning points x Amyloidosis is a rare benign disease of unknown aetiology. x Laryngeal amyloidosis can present with hoarseness or stridor which may require tracheostomy. x Histological diagnosis from a biopsy specimen can be confirmed with characteristic staining with Congo red. x Treatment is by surgical resection using the carbon dioxide laser. Repeated resections may be necessary. Localisation of lesions in the larynx is to the ventricle, false vocal cords, true vocal cords, aryepiglottic folds, and subglottis in that order of frequency.2 4 Histology of the biopsied specimen using routine haematoxylin and eosin stain shows amyloid as eosinophilic extracellular infiltrate. Further staining with Congo red reveals characteristic apple green birefringence with a ...

SummaryBetween January and July 1995, 227 patients at the Bronx-Lebanon Hospital Center had positive fungal cultures. Candida spp were the most common fungi isolated. Forty-three patients with invasive disease, as indicated by fungus-positive blood cultures, became the focus of our study. C albicans caused fungaemia in 21 patients (49%). Twenty-eight patients (65%) were less than 50 years of age; three were neonates. The most common presenting symptoms were fever, chills, and weakness (20 patients, 47%). Thirty patients died, giving a mortality rate of 70%. The patients who died stayed in the hospital an average of 49 days.The highest mortality occurred among patients who became bacteraemic before or at the same time they became candidaemic (24 of 26 patients) or who were receiving broad-spectrum antibiotics (20 of 26 patients). We also found high mortality rates from invasive fungal infection among patients with HIV infection, a central venous catheter, and liver, renal, or respiratory failure. We did not find any increase in the incidence of invasive fungal infection or mortality among leukopenic or diabetic patients.

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Giant bullous emphysema, also known as "vanishing lung syndrome", is a rare manifestation of chronic obstructive pulmonary disease (COPD) that is associated with high mortality. Cigarette smoking and alpha-1 antitrypsin deficiency (A1AD) are two main causes that result in permanent enlargement of airspaces, inefficient gas exchange, fibrosis of the airways, and collapse of the alveoli. A typical presentation can be found in a long-term smoker with dyspnea on exertion and progressive shortness of breath that may be associated with a productive cough. One of the clinical difficulties in diagnosing giant bullous emphysema is separating it from other etiologies like pneumothorax. It is paramount to differentiate giant bullous emphysema from pneumothorax as the management is completely different; both can, however, have similar clinical presentations and radiographic manifestations on initial assessment. In this report, we present the case of a 39-year-old African American male who presented with worsening shortness of breath and productive cough and was found to have bullous emphysema but was misdiagnosed and treated for pneumothorax in the initial encounter. The purpose of this case report is to raise awareness of this condition in the medical literature and discuss the similarity of bullous emphysema and pneumothorax in clinical presentation and radiographic findings, as well as the differences in treatment options.

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