Swyer-James-Macleod syndrome mimicking an acute pulmonary embolism: a report of six adult cases and a retrospective analysis.

BACKGROUND AND AIM: In patients with pulmonary embolism (PE), a pulmonary radiograph may reveal oligemic fields (the Westermark sign) associated with sites of occlusion of the pulmonary arteries, interruption or loss of the artery line (the knuckle sign), and even unilateral hyperlucency attributable to reduced overall lung vascularity. In Swyer-James-Macleod syndrome (SJMS), which develops as a result of bronchiolitis obliterans, unilateral hyperlucency is evident because of emphysema and hypoplasia of the pulmonary artery and its branches. Therefore, SJMS cases with clinical and laboratory data compatible with PE may in fact be confused with PE. The cases of six adult patients who were initially presumed to have PE but on further investigation were diagnosed with SJMS are presented in this report, which thus can serve as a guide for diagnosis of similar cases in future. METHODS: We studied six adult patients who presented with dyspnea. Their pulmonary radiographs revealed lobar/unilateral hyperlucency and PE was initially suspected. The pulmonary artery and branches thereof exhibited parenchymal emphysema and hypoplasia, and we thus diagnosed SJMS. RESULTS: We studied 4 males and 2 females with a mean age of 51 years (range, 20-73 years). Left lung involvement was evident in five cases. CONCLUSION: Unilateral hyperlucency may be a feature of both PE and SJMS. Although these conditions are very different, both present similarly in radiographic terms and may be easily confused when the clinical data and the anamnesis raise a suspicion of PE, causing unnecessary testing and treatment.

Interstitial lung disease in coppersmiths in high serum copper levels.

Coppersmith is a worker who uses copper most commonly for the production of kitchen appliances in Turkey. This is an ancient occupation practiced for centuries in Turkey. Our objective was to investigate the prevalence of parenchymal lung diseases among coppersmiths in Kahramanmaras city in Turkey. Thirty coppersmiths were included to the study, and they all signed an informed consent. Demographics, spirometric test results and high-resolution computed tomography (HRCT) scans, and blood samples were obtained. Laboratory analysis of the serum samples showed that serum copper levels of the subjects were 0.93 +/- 0.14 mg/L. Serum copper level in control group was found as 0.70 +/- 0.14 mg/L, and it was significantly different between the two groups (p < 0.05). Of 30 coppersmiths, 17 HRCT findings are abnormal and seen with diffuse parenchymal interstitial lung disease pattern-ten (58.8%) respiratory bronchiolitis interstitial lung disease, five (29.4%) nonspecific interstitial pneumonia, and two (11.8%) usual interstitial pneumonia. The most prevalent HRCT pattern was micronodular pattern in workers. This is the first field study reporting the radiologic findings of coppersmiths and effect of the occupation on lung diseases.

Cerebral Venous Thrombosis and Pulmonary Embolism with Prothrombin G20210A Gene Mutation.

A 25-year-old man presented with symptoms of syncope, cough, headache and hemoptysis. Cranial MR and venography showed thrombus formation in the right transverse sinus and superior sagittal sinus. Computed tomographic pulmonary angiography (CTPA) showed an embolic thrombus in the right pulmonary truncus and lung abscess. The patient was young, and there were no signs of lower extremity deep venous thrombosis or other major risk factors for pulmonary embolism (PE) including cardiac anomaly. The only risk factor we were able to identify was the presence of the prothrombin G20210A gene mutation. Anticoagulant treatment with oral warfarin (10 mg daily) and imipenem (4X500 mg) was started. The patient was hospitalized for antibiotic and anticoagulation therapies for three weeks and was discharged on lifelong treatment with warfarin (5 mg daily).

Unusual combination of tracheobronchopathia osteochondroplastica and AA amyloidosis.

Tracheobronchopathia osteochondroplastica (TO) is a rare disorder of unknown cause characterized by the presence of multiple submucosal osseous and/or cartilaginous nodules that protrude into the lumen of the trachea and large bronchi. A simultaneous diagnosis of TO and amyloidosis is rarely reported. In this report, a case initially suspected to be asthma bronchiole that could not be treated, was radiologically diagnosed as TO, and also secondary amyloidosis is presented. A 53 years, man patient reported a 3 years history of dyspnea. Pulmonary function tests (PFTs) showed an obstructive pattern. Chest X-rays revealed right middle lobe atelectasis. FOB and CT detected nodular lesions in the trachea and in the anterior and lateral walls of the main bronchi. AA amyloidosis was confirmed by endobronchial biopsy. In the abdominal fat pad biopsy, amyloidosis was not detected. Asthma bronchiole was excluded by PFTs. This case illustrates that it is possible for TO and amyloidosis to masquerade as asthma. TO and amyloidosis should be suspected in patients of older ages with asthma and especially with poorly treated asthmatic patients. Although nodular lesions in the anterior and lateral tracheobronchial walls are typical for TO, a biopsy should be obtained to exclude amyloidosis.

Prevalence of and factors affecting pulmonary hypertension in hemodialysis patients.

BACKGROUND: Pulmonary hypertension (PH) has been reported to be high among end-stage renal disease (ESRD) patients. OBJECTIVES: The aim of this study was to investigate the role of arteriovenous fistula (AVF) flow in the pathogenesis of PH and the prevalence of PH in patients with chronic renal failure (CRF) and to suggest other possible etiologic factors. METHODS: The prevalence of PH was prospectively estimated by Doppler echocardiography in 116 ESRD patients on regular hemodialysis (HD). Laboratory and clinical variables were compared between patients with and without PH (groups 1 and 2, respectively). PH was defined as systolic pulmonary artery pressure (SPAP) over 30 mm Hg. Patients with PH underwent further evaluation by 2 pulmonologists. AVF flow was measured by Doppler ultrasonography. Blood tests including arterial blood gases, hemoglobin, serum calcium, phosphorus and parathyroid hormone were determined. RESULTS: PH was found in 25 (21.6%) patients (group 1) with an SPAP of 37.9 ± 2.8 mm Hg. Mean AVF flow was increased (1,554 ± 207.60 ml/min) in group 1. Left ventricular ejection fraction (LVEF) was significantly different between the 2 groups (55.3 ± 11.5 and 64.4 ± 40, respectively; p < 0.05). Neither significant primary lung disease nor parenchymal lesions were detected in group 1. PH showed a significant difference for cigarette smoking (p < 0.05). In group 1 the prevalence of cigarette smoking was higher. The main etiology of CRF was diabetes mellitus with a ratio of 44% in group 1. CONCLUSION: Our study demonstrated a surprisingly high prevalence of PH among patients receiving long-term HD. PH was related to high AVF flow, low LVEF and cigarette smoking. AVF flow and cigarette smoking are important correctable causes of PH. Early detection is important in order to avoid the serious consequences.

A multicentre study of doctors' approaches to the diagnosis and treatment of tuberculosis in Turkey.

BACKGROUND: Inappropriate anti-tuberculosis (TB) treatment, particularly at the beginning of the disease, is a major cause of drug resistance. This survey was performed to evaluate doctors' approaches to the diagnosis and treatment of TB. METHOD: The study was conducted in 12 cities, each from a different geographical region of Turkey. A prepared questionnaire about their academic careers and experience in TB and its treatment was distributed to the doctors. RESULTS: The research group consisted of 1,112 doctors. The mean age was 32.7 +/- 6.6 years. The results showed that 75.9% of the doctors had insufficient knowledge about TB. The results of the study further showed that chest specialists, female doctors, physicians who work in a first stage hospital, doctors who had previously diagnosed TB and those who had access to a microbiology laboratory had a better level of knowledge about tuberculosis and its treatment than other doctors. CONCLUSION: A great diversity was seen between daily practice of doctors and national guidelines about TB. Educational programmes conducted by specialists in TB control may help to standardize and increase the knowledge of TB.

Miliary tuberculosis accompanying paravertebral tuberculosis abscess in an adolescent.

Although miliary tuberculosis (TB) is well known, the incidence of miliary TB accompanying paravertebral abscess is extremely rare in adolescent children. We report a case of paravertebral TB abscess and miliary TB in a 17-year-old male initially presenting with fever, general weakness, back pain, sweating, cough, dyspnea and weight loss. The patient was diagnosed as paravertebral TB abscess and miliary TB. The anti-tuberculous drugs were started and the follow-up imaging showed that the lesions had disappeared without surgery. Although seldom observed, TB should be kept in mind in the differential diagnosis of paravertebral abscess.

Relationship of metabolic alkalosis, azotemia and morbidity in patients with chronic obstructive pulmonary disease and hypercapnia.

BACKGROUND: Exacerbation of chronic obstructive pulmonary disease (COPD) is an important cause of morbidity and mortality, but the effect of metabolic compensation of respiratory acidosis (RA) on mortality is not fully understood. OBJECTIVE: To investigate the relationship between metabolic compensation and mortality in COPD patients with RA. METHODS: We prospectively investigated all COPD patients with RA admitted to the respiratory intensive care unit between February 2001 and March 2007. Two hundred and thirteen patients (159 male, 54 female; mean age 65 +/- 10.8 years) were divided into three groups (71 patients each) according to base excess (BE) levels: (1) low BE, (2) medium BE, and (3) high BE. H(+) concentration was calculated according to their standard formula and BE was calculated according to the Van Slyke equation. RESULTS: The overall mortality rate was 24.9%. The group mortality rates were 32, 17 and 25% in the low, medium and high BE groups, respectively (p = 0.001). When patients were divided into three groups according to the HCO(3)(-) levels, the group mortality rate was 59.1% in the low HCO(3)(-) group and 19.8% in the high HCO(3)(-) group. Based on univariate analysis, six factors affecting mortality were identified. However, multivariate analysis showed that the levels of serum HCO(3)(-) (p = 0.013; OR: 0.552; CI: 0.345-0.882) and creatinine (p = 0.019; OR: 2.114; CI: 1.132-3.949) had an independent effect. CONCLUSION: In patients with COPD exacerbation and hypercapnia, the development of sufficient metabolic compensation and adequate renal function significantly decreases mortality.

Clinical review of tuberculous peritonitis in 39 patients in Diyarbakir, Turkey.

Abdominal tuberculosis (TB) is a rare manifestation, which can be overlooked on long-lasting and non-specific findings unless a high index of suspicion is maintained. The purpose of the present study was to investigate the diagnostic features of 39 patients hospitalized with tuberculous peritonitis (TBP) in Dicle University Hospital, Turkey between January 1994 and August 2003. Twenty-two patients were male; patient age ranged between 1 and 59 years (mean: 16.2 +/- 14.4 years). There were 21 patients (54%) under 15 years of age. Thirteen children had a history of familial TB and seven adults had prior history of TB. Six (29%) of 21 pediatric cases had bacille Calmette-Guerin (BCG) scars and results of 5-tuberculin units (TU) tuberculin test were positive in seven children (18%). Of all cases, the most common presenting findings were abdominal pain (95%), ascites (92%) and abdominal distention (82%). Five of the patients had accompanying pulmonary TB, and six patients (15%) had intestinal TB who were admitted to emergency service with acute abdomen, of whom three (8%) had perforation and three (8%) had ileus. Histopathologically 20 cases (51%) were proven on abdominal ultrasonography, and computed tomography revealed most commonly ascites and thickening of peritoneum. No microbiologic evidence was obtained except three positive culture results for Mycobacterium tuberculosis. As a result, TBP should be considered for diagnosis, in patients with non-specific symptoms of abdominal pain, wasting, fever, loss of appetite, abdominal distension and even symptoms of acute abdomen, because early diagnosis and effective treatment will decrease morbidity and mortality.