A cluster of cases of severe acute respiratory syndrome in Hong Kong.

BACKGROUND: Information on the clinical features of the severe acute respiratory syndrome (SARS) will be of value to physicians caring for patients suspected of having this disorder. METHODS: We abstracted data on the clinical presentation and course of disease in 10 epidemiologically linked Chinese patients (5 men and 5 women 38 to 72 years old) in whom SARS was diagnosed between February 22, 2003, and March 22, 2003, at our hospitals in Hong Kong, China. RESULTS: Exposure between the source patient and subsequent patients ranged from minimal to that between patient and health care provider. The incubation period ranged from 2 to 11 days. All patients presented with fever (temperature, >38 degrees C for over 24 hours), and most presented with rigor, dry cough, dyspnea, malaise, headache, and hypoxemia. Physical examination of the chest revealed crackles and percussion dullness. Lymphopenia was observed in nine patients, and most patients had mildly elevated aminotransferase levels but normal serum creatinine levels. Serial chest radiographs showed progressive air-space disease. Two patients died of progressive respiratory failure; histologic analysis of their lungs showed diffuse alveolar damage. There was no evidence of infection by Mycoplasma pneumoniae, Chlamydia pneumoniae, or Legionella pneumophila. All patients received corticosteroid and ribavirin therapy a mean (+/-SD) of 9.6+/-5.42 days after the onset of symptoms, and eight were treated earlier with a combination of beta-lactams and macrolide for 4+/-1.9 days, with no clinical or radiologic efficacy. CONCLUSIONS: SARS appears to be infectious in origin. Fever followed by rapidly progressive respiratory compromise is the key complex of signs and symptoms from which the syndrome derives its name. The microbiologic origins of SARS remain unclear.

Endobronchial Foreign Body Presenting as Exacerbation of Asthma.

Airway foreign bodies are a leading cause of death among children and require urgent recognition by medical personnel. While most cases are diagnosed readily from a clinical history of acute respiratory distress, some cases remain more indolent and present later. We report the case of a 7-year-old boy who aspirated a "LEGO" toy and presented with a week history of increasing respiratory distress compatible with known asthma. Despite a normal chest X-ray, a low-dose computed tomography showed the presence of a foreign body in the left main bronchus, which was subsequently removed by fiberoptic bronchoscopy. Our case serves to reemphasize the importance of considering airway foreign bodies as a cause of respiratory distress, especially in young children.

Exhaled and sputum nitric oxide in bronchiectasis: correlation with clinical parameters.

STUDY OBJECTIVES: Although there has been tremendous attention on endogenous nitric oxide (NO) production in many respiratory and systemic diseases, little is known on NO production in bronchiectasis. DESIGN AND SETTING: We determined exhaled and sputum NO levels in 109 patients with stable bronchiectasis (71 women; mean +/- SD age, 58.2 +/- 14.1 years) and 78 control subjects (39 women; mean age, 56.7 +/- 12.1 years) by using an automatic chemiluminescence analyzer. MEASUREMENTS AND RESULTS: There was no significant difference in exhaled NO between patients with bronchiectasis and control subjects (p = 0.11). Bronchiectasis patients with Pseudomonas aeruginosa infection had a significantly lower exhaled, but not sputum, NO levels than their counterparts and control subjects (p = 0.04 and p = 0.009, respectively). Exhaled NO correlated with 24-h sputum volume in P aeruginosa-infected patients (r = - 0.36; p = 0.002). After adjustment for sputum volume and number of bronchiectatic lung lobes, P aeruginosa-infected patients still had lower exhaled NO levels than their counterparts (p = 0.01). There was no correlation between exhaled NO with FEV(1), FVC, and the number of bronchiectatic lung lobes (p > 0.05). Sputum NO levels were not different between patients and control subjects (p = 0.64), and had no correlation with clinical parameters. CONCLUSION: Exhaled NO appears to be reduced among bronchiectasis patients with P aeruginosa infection independent of other clinical parameters, and further studies on the potential mechanisms and pathogenetic implications of this reduction should be pursued.

Validation of the Hong Kong Chinese version of the St. George Respiratory Questionnaire in patients with bronchiectasis.

STUDY OBJECTIVES: To validate the Hong Kong Chinese version of the St. George Respiratory Questionnaire (SGRQ-HK) in patients with bronchiectasis. DESIGN AND SETTING: Outpatients (93 patients; 61 women; mean age [+/- SD], 59.0 +/- 14.2 years) were assessed at baseline by the SGRQ-HK, the Hong Kong Chinese version of the 36-item short form health survey (SF-36-HK), and the hospital anxiety and depression scale (HADS). Forty randomly selected patients also were reassessed at 2 weeks for repeatability. Seventy-two patients were further reassessed at 6 months for responsiveness. MEASUREMENTS AND RESULTS: Cronbach alpha coefficients, which reflected internal consistency, were > 0.7 for all SGRQ-HK components except for symptoms (alpha = 0.59), and the intraclass correlation coefficients between baseline and the 2-week follow-up visits were between 0.80 and 0.94 (p > 0.05). SGRQ-HK component scores and total scores correlated with all the component scores of the SF-36-HK and the HADS (p < 0.02). SGRQ-HK component scores and total scores correlated with the scores of the SF-36-HK and the HADS, confirming the concurrent validity. All SGRQ-HK scores correlated negatively with FEV(1), FVC, and arterial oxygen saturation (p < 0.005), while the activity score correlated with the Karnofsky performance scale and the number of bronchiectatic lobes (p < 0.001). SGRQ-HK scores positively correlated with Borg scale scores, exacerbation frequency, and 24-h sputum volumes (p < 0.03). Patients with 24-h sputum volumes of > or = 10 mL or < 10 mL had significantly different SQRG-HK component scores and total scores (p < 0.002), although this sensitivity was not displayed by scores on the HADS or the SF-36-HK. Patients with 25% reductions in 24-h sputum volumes had significant improvements in SGRQ-HK activity scores, impact scores, and total scores (p < 0.02), but not in other quality-of-life measures or clinical parameters, indicating the responsiveness of the SGRQ-HK. CONCLUSION: The SGRQ-HK is a valid and sensitive instrument for determining quality of life in bronchiectasis patients.

Clinical value of ciliary assessment in bronchiectasis.

Although ciliary dysfunction and numerous ultrastructural defects have been described, and these could be etiologically important in the development of bronchiectasis, their correlation with relevant clinical parameters have not been systematically evaluated. We have prospectively evaluated the prevalence and clinical significance of ciliary beat frequency and ultrastructural defects of nasal respiratory mucosa obtained from 152 stable patients with idiopathic bronchiectasis (100F, 57.7 +/- 15.2 yrs) and 127 control subjects (58F, 56.0 +/- 24.2 yrs). Bronchiectasis patients had significantly slower ciliary beat frequency (p < 0.05), and a greater percent of patients had central and peripheral microtubular defects (OR 14.4, 95% CI 5.6-36.8), namely, extra peripheral microtubules, "9 + 1", "8 + 2", and compound cilia (p < 0.05), but not microtubular disarrangement, extra matrix or ciliary tail abnormalities (p > 0.05), than controls. Bronchiectasis patients also had a greater proportion of cilia with any ultrastructural microtubular defects, compound cilia, and ciliary tails than controls (p < 0.05). Ciliary beat frequency did not correlate with clinically relevant parameters (p > 0.05). However, the percent of cilia with central, but not peripheral, microtubular defects correlated with 24 h sputum volume (r = 0.40, p = 0.001, and r = -0.04, p = 0.70, respectively) and FEV1 (r = -0.24, p = 0.01, and r = 0.00, p = 0.99 respectively). Our results strongly suggest a pathogenic role for central microtubular defects in the development of idiopathic bronchiectasis.

Severe acute respiratory syndrome (SARS) in Hong Kong.

Severe acute respiratory syndrome (SARS) is a recently recognized and highly contagious pneumonic illness, caused by a novel coronavirus. While developments in diagnostic, clinical and other aspects of SARS research are well underway, there is still great difficulty for frontline clinicians as validated rapid diagnostic tests or effective treatment regimens are lacking. This article attempts to summarize some of the recent developments in this newly recognized condition from the Asia Pacific perspective.

Severe acute respiratory syndrome: radiographic and CT findings.

OBJECTIVE: We review the radiographic and CT findings in the lungs of 12 patients with severe acute respiratory syndrome (SARS) in an effort to describe the most common radiologic findings for this disease. CONCLUSION: The most common radiographic findings of SARS patients at presentation are unilateral or bilateral ground-glass opacities or focal unilateral or bilateral areas of consolidation. In hospitalized SARS patients, the abnormalities tend to progress to bilateral air-space consolidation. CT may reveal parenchymal disease in patients whose radiographs show normal results.

Regression of bilateral bronchiectasis with inhaled steroid therapy.

Bronchiectasis is defined as pathological and permanent dilatation of the bronchial tree. Affected patients suffer from chronic sputum production and usually slowly progressive airway destruction as a result of continued airway infection and inflammation. Regression of bilateral bronchiectasis has never been reported in the English literature. We report the case of a 60-year-old woman with longstanding progressive idiopathic bilateral bronchiectasis whose respiratory symptoms, including sputum, rapidly disappeared after commencement of inhaled budesonide. Repeat computed tomography assessment 40 months after commencement of inhaled steroid therapy, showed partial regression of bronchial dilation and resolution of small airways sepsis. In the absence of other possible explanations for the partial resolution of the bronchiectasis, the present case suggests a possible benefit of inhaled steroid therapy in bronchiectasis.

High-resolution CT findings of severe acute respiratory syndrome at presentation and after admission.

OBJECTIVE: The aim of this study was to assess the high-resolution CT (HRCT) findings at presentation and after hospital admission in patients with severe acute respiratory syndrome (SARS). MATERIALS AND METHODS: We reviewed the HRCT findings at presentation (n = 12) and after hospital admission (n = 25) of 29 patients with SARS and compared the HRCT findings with the radiographic findings. HRCT scans were obtained using 1-mm (n = 28) or 2-mm (n = 1) collimation. The radiographs and HRCT scans were reviewed independently by two observers who reached a decision by consensus. RESULTS: All patients had abnormal findings on HRCT at presentation. Eight of these 12 patients had normal findings on radiographs. The predominant HRCT findings at presentation consisted of unilateral (n = 6) or bilateral (n = 2) ground-glass opacities or focal unilateral (n = 2) or bilateral (n = 2) areas of consolidation. All patients showed progression of disease on follow-up. The predominant HRCT findings on follow-up CT scans consisted of unilateral (n = 2) or bilateral ground-glass opacities (n = 13), unilateral (n = 2) or bilateral consolidation (n = 5), or a mixed bilateral pattern of ground-glass attenuation, consolidation, and reticulation (n = 3). Reticulation with associated architectural distortion and mild traction bronchiectasis was present in eight patients. CONCLUSION: HRCT can show parenchymal abnormalities in patients with SARS who have normal findings on radiographs at presentation. Follow-up CT scans obtained in hospitalized patients show findings consistent with fibrosis in a small percentage of patients.

Extrapulmonary presentation of asymptomatic pulmonary lymphangioleiomyomatosis.

Lymphangioleiomyomatosis (LAM) is a rare parenchymal lung disease, which affects young women of childbearing age and is characterized pathologically by proliferation of interstitial smooth muscle and formation of cysts in the lung. While LAM is usually predominantly a respiratory disorder, it can also initially involve other extrapulmonary organs. We report the case of a 35-year-old Chinese woman, who presented with a 4-week history of left thigh swelling which was found to be secondary to compression of pelvic veins by a mass lesion. The latter was found histologically to show LAM. Despite the patient being asymptomatic and displaying normal lung function, a thoracic high resolution CT scan showed typical features of early LAM. This case further illustrates that LAM can have multisystem involvement, and demonstrates the importance of being aware of the diagnosis in cases presenting with extrapulmonary manifestation, in order that patients are diagnosed and managed appropriately.

Severe acute respiratory syndrome: temporal lung changes at thin-section CT in 30 patients.

PURPOSE: To evaluate lung abnormalities on serial thin-section computed tomographic (CT) scans in patients with severe acute respiratory syndrome (SARS) during acute and convalescent periods. MATERIALS AND METHODS: Serial thin-section CT scans in 30 patients (17 men, aged 42.5 years +/- 12.2 [SD]) with SARS were reviewed by two radiologists together for predominant patterns of lung abnormalities: ground-glass opacities, ground-glass opacities with superimposed linear opacities, consolidation, reticular pattern, and mixed pattern (consolidation, ground-glass opacities, and reticular pattern). Scans were classified according to duration in weeks after symptom onset. Longitudinal changes of specific abnormalities were documented in 17 patients with serial scans obtained during 3 weeks. Each lung was divided into three zones; each zone was evaluated for percentage of lung involvement. Summation of scores from all six lung zones provided overall CT score (maximal CT score, 24). RESULTS: Median CT scores increased from 1 in the 1st week to 12.5 in the 2nd week. Ground-glass opacities with or without smooth interlobular septal thickening and consolidation were predominant patterns found during the 1st week. Ground-glass opacities with superimposed irregular reticular opacities, mixed pattern, and reticular opacities were noted from the 2nd week and peaked at or after the 4th week. After the 4th week, 12 (55%) of 22 patients had irregular linear opacities with or without associated ground-glass opacities and CT scores greater than 5; five of these patients had bronchial dilatation. When specific opacities were analyzed in 17 patients, consolidation generally resolved completely (n = 4) or to minimal residual opacities; six (55%) of 11 patients with ground-glass opacities had substantial residual disease (CT scores > 5) on final scans. CONCLUSION: There is a temporal pattern of lung abnormalities at thin-section CT in SARS. Predominant findings at presentation are ground-glass opacities and consolidation. Reticulation is evident after the 2nd week and persists in half of all patients evaluated after 4 weeks. Long-term follow-up is required to determine whether the reticulation represents irreversible fibrosis.

An indolent case of severe acute respiratory syndrome.

Severe acute respiratory syndrome (SARS) is a highly contagious and typically rapidly progressive form of atypical pneumonia, which spread from Asia to many parts of the world in early 2003. Clinical diagnosis of SARS requires the presence of unremitting fever and progressive pneumonia despite antibiotic therapy, particularly in the presence of lymphopenia and raised transaminase levels. We report the case of a woman who had undergone a successful allogeneic bone marrow transplant for acute myeloid leukemia. She presented initially with fever and a normal chest radiograph. Her indolent clinical course of SARS was punctuated by resolution of fever, but there was progressive radiologic deterioration and increasing serum antibody titer against SARS coronavirus. Treatment with oral prednisolone and ribavirin normalized her lymphopenia, altered transaminases, chest radiograph and high-resolution computed tomography appearances rapidly. Our experience should alert other clinicians in recognizing this atypical indolent presentation of SARS, to protect health care workers and the community at large and to ensure that these patients are properly treated.

Silicosis in 76 men: qualitative and quantitative CT evaluation--clinical-radiologic correlation study.

PURPOSE: To use qualitative and quantitative computed tomography (CT) to test the hypothesis that impaired lung function with silicosis is due to progressive massive fibrosis (PMF) and associated emphysema. MATERIALS AND METHODS: Seventy-six men with silicosis underwent volumetric and thin-section CT of the thorax. Lung function, Borg scale dyspnea grade, silica exposure duration, and cigarette consumption were determined. Nodular profusion (NP) at chest radiography was graded according to the International Labor Organization radiographic classification system; NP and PMF at CT were visually graded by using five-point (ie, grades 0-4) and four-point (grades 0-3) scales, respectively. Emphysema and NP, which together are defined as the NP index, were quantified by using attenuation threshold values of less than -950 HU and greater than -100 HU, respectively. Mean lung attenuation was also determined. Relationships among the CT, chest radiographic, and clinical parameters were analyzed by using Spearman correlation. RESULTS: NP at chest radiography correlated (r > 0.50) with all CT parameters of nodularity. CT PMF had the highest correlation with emphysema (r = 0.58, P <.001). NP at chest radiography and all CT parameters were inversely related to lung function. At multiple regression analysis, PMF and emphysema index (both at CT) were significant determinants of forced expiratory volume in 1 second (FEV1) (P =.006 and.03, respectively) and FEV1 to forced vital capacity (FVC) ratio (P =.007 and.02, respectively). Mean lung attenuation remained related to FVC (P =.03), diffusing capacity of lung for carbon monoxide (P =.04), and Borg scale grade (P =.01). Cigarette consumption and silica exposure duration had no independent effects on lung function. CONCLUSION: Qualitative and quantitative CT parameters can be used as indirect measures of functional impairment in silicosis. PMF and emphysema are independently related to airflow obstruction, whereas mean lung attenuation is related to clinical dyspnea and reduced lung volume.

High-resolution CT quantification of bronchiectasis: clinical and functional correlation.

PURPOSE: To evaluate clinical relevance of high-resolution computed tomographic (CT) findings in patients with bronchiectasis by using a quantitative high-resolution CT protocol to assess extent of bronchiectasis, severity of bronchial wall thickening, and presence of small-airway abnormalities and mosaic pattern. MATERIALS AND METHODS: Sixty Chinese patients with steady-state bronchiectasis underwent thoracic high-resolution CT and lung function tests. Exacerbation frequency per year and 24-hour sputum volume were determined. Extent of bronchiectasis, severity of bronchial wall thickening, and presence of small-airway abnormalities and mosaic attenuation were evaluated in each lobe, including the lingula. Differences between sex and smoking status with respect to high-resolution CT, lung function, and clinical parameters were tested with either the independent sample t test or the Mann-Whitney test. Spearman rank correlation was used to evaluate associations between clinical, lung function, and high-resolution CT scores. Multiple regression analyses were performed to determine which high-resolution CT parameters would best predict lung function and clinical parameters, adjusted for smoking. RESULTS: Exacerbation frequency was associated with bronchial wall thickening (r = 0.32, P =.03); 24-hour sputum volume with bronchial wall thickening and small-airway abnormalities (r = 0.30 and 0.39, respectively; P <.05); and forced expiratory volume in 1 second (FEV(1)), ratio of FEV(1) to forced vital capacity (FVC), and midexpiratory phase of forced expiratory flow (FEF(25%-75%)) (r = -0.33, -0.29, and -0.32, respectively; P <.05). Extent of bronchiectasis, bronchial wall thickening, and mosaic attenuation, respectively, were related to FEV(1) (r = -0.43 to -0.60, P <.001), FEF(25%-75%) (r = -0.38 to -0.57, P <.001), FVC (r = -0.36 to -0.46, P <.01), and FEV(1)/FVC ratio (r = -0.31 to -0.49, P <.01). After multiple regression analysis, bronchial wall thickening remained a significant determinant of airflow obstruction, whereas small-airway abnormalities remained associated with 24-hour sputum volume. Women had milder disease than men but showed more high-resolution CT functional correlations. CONCLUSION: Findings of this study establish a link between morphologic high-resolution CT parameters and clinical activity and emphasize the role of bronchial wall thickening in patients with bronchiectasis.

High-dose pulse versus nonpulse corticosteroid regimens in severe acute respiratory syndrome.

The treatment of atypical pneumonia, subsequently termed severe acute respiratory syndrome (SARS), is controversial, and the efficacy of corticosteroid therapy is unknown. We have evaluated the clinical and radiographic outcomes of 72 patients with probable SARS (median age 37 years, 30 M), who received ribavirin and different steroid regimens in two regional hospitals. Chest radiographs were scored according to the percentage of lung field involved. Seventeen patients initially received pulse steroid (PS) (methylprednisolone > or =500 mg/day) and 55 patients initially received nonpulse steroid (NPS) (methylprednisolone <500 mg/day) therapy. The cumulative steroid dosage; intensive care unit admission, mechanical ventilation, and mortality rates; and hematologic and biochemical parameters were similar in both groups after 21 days. However, patients in the PS group had less oxygen requirement, better radiographic outcome, and less likelihood of requiring rescue PS therapy than their counterparts. There was no significant difference between the two groups in hemolytic anemia, severe secondary infections, or hematemesis, but patients in the PS group had less hyperglycaemia. Initial use of pulse methylprednisolone therapy appears to be a more efficacious and an equally safe steroid regimen when compared with regimens with lower dosage and should be considered as the preferred steroid regimen in the treatment of SARS, pending data from future randomized controlled trials.