Pulmonary emphysema in a neonate with Marfan syndrome

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Day, D L; Burke, B A

1986-09-01

A premature infant with arachnodactyly and congential cyanotic heart disease had severe pulmonary emphysema, a little-known manifestation of Marfan syndrome. The possible etiologies of emphysema in this syndrome are discussed.

Pulmonary emphysema in a neonate with Marfan syndrome

International Nuclear Information System (INIS)

Day, D.L.; Burke, B.A.

1986-01-01

A premature infant with arachnodactyly and congential cyanotic heart disease had severe pulmonary emphysema, a little-known manifestation of Marfan syndrome. The possible etiologies of emphysema in this syndrome are discussed. (orig.)

Impact of morphologic characteristics of central pulmonary thromboemboli in massive pulmonary embolism.

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Podbregar, Matej; Krivec, Bojan; Voga, Gorazd

2002-09-01

To assess the impact of morphologically different central pulmonary artery thromboemboli in patients with massive pulmonary emboli (MPEs) on short-term outcome. A prospective registry of consecutive patients. An 11-bed closed medical ICU at a 860-bed community general hospital Forty-seven patients with shock or hypotension due to MPE and central pulmonary thromboemboli detected by transesophageal echocardiography who were treated with thrombolysis between January 1994 and April 2000. Patients were divided into two groups according to the following characteristics of the detected thromboemboli: group 1, thrombi with one or more long, mobile parts; and group 2, immobile thrombi. Right heart catheterization was performed. The incidence of both types of thromboemboli was comparable. Groups 1 and 2 showed no differences in demographic data, risk factors for pulmonary embolism, length of preceding clinical symptoms, percentage of patients in shock, hemodynamic variables, serum lactate levels on hospital admission, and treatment. Seven fatal cases due to obstructive shock and right heart failure were present in group 2, but none were present in group 1 (7 of 23 patients vs 0 of 24 patients, respectively; p < 0.05). At 12 h, the cardiac index was lower in group 2 than in group 1 (2.6 +/- 1.0 vs 3.1 +/- 0.9 L/min/m(2), respectively; p < 0.05), and the central venous pressure (15.0 +/- 6.2 vs 12.5 +/- 3.7 mm Hg, respectively; p < 0.05) and total pulmonary resistance (12.9 +/- 5.9 vs 8.6 +/- 2.7 mm Hg/L/min/m(2), respectively; p < 0.001) were higher in group 2 compared to group 1. On hospital admission, inclusion in group 2 (p < 0.03; hazard ratio, 9.53; 95% confidence interval [CI], 1.19 to 76.47) and preexisting chronic medical or neurologic disease (p < 0.01; hazard ratio, 16.4; 95% CI, 1.97 to 136.3) were independent predictors of 30-day mortality. On hospital admission, morphology of the thromboemboli and the presence of pre-existing chronic medical or neurologic disease

Efficacy and safety of oral sildenafil in children with Down syndrome and pulmonary hypertension.

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Beghetti, Maurice; Rudzinski, Andrzej; Zhang, Min

2017-07-04

Despite the increased risk for pulmonary hypertension in children with Down syndrome, the response to treatment with targeted therapies for pulmonary hypertension in these patients is not well characterized. The Sildenafil in Treatment-naive children, Aged 1-17 years, with pulmonary arterial hypertension (STARTS-1) trial was a dose-ranging study of the short-term efficacy and safety of oral sildenafil in children with pulmonary arterial hypertension. We assessed the safety and efficacy of oral sildenafil in children with Down syndrome and pulmonary arterial hypertension. This was a post-hoc analysis of children with Down syndrome and pulmonary arterial hypertension enrolled in the STARTS-1 trial. Mean pulmonary arterial pressure (mPAP), pulmonary vascular resistance index (PVRI), and cardiac index (CI) were assessed at baseline and following 16 weeks of treatment with sildenafil. Of 234 patients randomized and treated in the STARTS-1 trial, 48 (20.5%) had Down syndrome. Although sildenafil produced dose-related reductions in PVRI and mPAP, compared with placebo, in non-Down syndrome patients and children developmentally able to exercise, this was not satisfactorily marked in patients with Down syndrome. The dose-related reductions in PVRI, compared with placebo, occurred in all subgroups, with the exception of the Down syndrome subgroup. Sildenafil appeared to be well tolerated in the Down syndrome subpopulation and the most frequently reported AEs were similar to those reported for the entire STARTS-1 population. Sildenafil treatment for 16 weeks had no effect on PVRI or mPAP in children with Down syndrome and pulmonary arterial hypertension. The results suggest that children with Down syndrome may be less responsive to sildenafil for pulmonary arterial hypertension, but the incomplete work-up for the etiology of pulmonary arterial hypertension may have introduced a potential bias. Study received, September 8, 2005 (retrospectively registered); Study start

A case of William's syndrome associated peripheral pulmonary arterial stenosis

International Nuclear Information System (INIS)

Jung, Kyung Hwa; Hwang, Mi Soo; Kim, Sun Yong; Chang, Jae Chun; Park, Bok Hwan

1988-01-01

William's syndrome, in order to more completely delineate the total spectrum of the disorder, indicates that 'infantile hypercalcemia', 'peculiar facies' and 'supravalvular aortic stenosis.' In has other many vascular anomalies, such as peripheral pulmonary arterial stenosis, coronary arterial stenosis, celiac arterial stenosis, and renal aterial stenosis. Only 32% of the patients have evidence of supravalvular aortic stenosis. And it is very rare disease entity that has been reported rarely in Korea. Recently authors experienced a case that was questioned William's syndrome with peripheral pulmonary arterial stenosis, clinically and preliminary radiologically and this case was confirmed by operation. Here we report a case of William's syndrome with peripheral pulmonary arterial stenosis and reviewed literatures

Partial abnormal pulmonary venous return in Turner syndrome

NARCIS (Netherlands)

van Wassenaer, A. G.; Lubbers, L. J.; Losekoot, G.

1988-01-01

Three cases of partial anomalous pulmonary venous return, in one case combined with coarctation of the aorta and in another with discrete subaortic stenosis, are described in patients with Turner syndrome. In two of them the right and left superior pulmonary veins drained into the right superior

The pulmonary histopathology of anti-KS transfer RNA synthetase syndrome.

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Schneider, Frank; Aggarwal, Rohit; Bi, David; Gibson, Kevin; Oddis, Chester; Yousem, Samuel A

2015-01-01

The clinical spectrum of the antisynthetase syndromes (AS) has been poorly defined, although some frequently present with pulmonary manifestations. The anti-KS anti-asparaginyl-transfer RNA synthetase syndrome is one in which pulmonary interstitial lung disease is almost always present and yet the histopathologic spectrum is not well described. To define the morphologic manifestations of pulmonary disease in those patients with anti-KS antiasparaginyl syndrome. We reviewed the connective tissue disorder registry of the University of Pittsburgh and identified those patients with anti-KS autoantibodies who presented with interstitial lung disease and had surgical lung biopsies. The 5 patients with anti-KS antisynthetase syndrome were usually women presenting with dyspnea and without myositis, but with mechanic's hands (60%) and Raynaud phenomenon (40%). They most often presented with a usual interstitial pneumonia pattern of fibrosis (80%), with the final patient displaying organizing pneumonia. Pulmonary interstitial lung disease is a common presentation in patients with the anti-KS-antisynthetase syndrome, who are often women with rather subtle or subclinical connective tissue disease, whereas the literature emphasizes the nonspecific interstitial pneumonia pattern often diagnosed clinically. Usual interstitial pneumonia and organizing pneumonia patterns of interstitial injury need to be added to this clinical differential diagnosis.

Pulmonary arterial stent implantation in an adult with Williams syndrome

NARCIS (Netherlands)

Reesink, Herre J.; Henneman, Onno D. F.; van Delden, Otto M.; Biervliet, Jules D.; Kloek, Jaap J.; Reekers, Jim A.; Bresser, Paul

2007-01-01

We report a 38-year-old patient who presented with pulmonary hypertension and right ventricular dysfunction due to pulmonary artery stenoses as a manifestation of Williams syndrome, mimicking chronic thromboembolic pulmonary hypertension. The patient was treated with balloon angioplasty and stent

Treatment of Hantavirus Pulmonary Syndrome

Science.gov (United States)

2007-10-14

femoral artery and vein. The bed is versity of New Mexico. . Antiviral therapy Ribavirin was tested for efficacy in HFRS patients in China nd shown to...Weis- senbacher, M.C., 1996. Hantavirus pulmonary syndrome in Argentina. Possibility of person to person transmission. Medicina (B. Aires) 56, 709–711

The toxic autoimmune syndrome with pulmonary edema

International Nuclear Information System (INIS)

Parizhskij, Z.M.; Artyunina, G.P.; Trofimova, T.N.

1992-01-01

A case was considered in detail of a patient with pulmonary edema of immunnocomplex nature in aerogenic intoxication by nickel tetracarbonyl. It was shown that acute aerogenic intoxication nickel carbonyl by led to unfolded toxic autoimmune syndrome. In this case autoimmune immunecomplex pulmonary lesion (AIPL) menifested by progressing pulmonary edema with expressed parenchymatous respiratory insufficiency played a leading role. Lesion of endothelium of pulmonary capillaries by immune complexes has the most significant in pathogenesis of pulmonary edema. The fact that edema appears due to AIPL, is confirmed by high efficiency of glucocorticoid therapy. Use of glucorticoids serves as a diagnostic test which provides an effective roentgenologic diagnosis of AIPL and differential diagnosis of any other pathological processes in the lungs

Jaccoud's arthropathy and pulmonary fibrosis in CREST syndrome

International Nuclear Information System (INIS)

Spinel B, Nestor; Montenegro, Pablo; Rondon Federico; Restrepo, Jose F; Iglesias G, Antonio

2010-01-01

We report a case of a 48 years old patient with diagnosis of incomplete CREST syndrome (variant limited systemic sclerosis) in who we documented the presence of Jaccoud's arthropathy of the hands and pulmonary involvement by pulmonary fibrosis type usual interstitial pneumonia, with positivity for rheumatoid factor and anti-cyclic citrullinated peptide antibody.

Morphologic characteristics of central pulmonary thromboemboli predict haemodynamic response in massive pulmonary embolism.

Science.gov (United States)

Podbregar, Matej; Voga, Gorazd; Krivec, Bojan

2004-08-01

On hospital admission, the morphology of the central pulmonary artery thromboemboli is an independent predictor of 30-day mortality in patients with massive pulmonary embolism (MPE). This may be due to the differential susceptibility of thromboemboli to thrombolysis. The aim of this study was to assess haemodynamic response to treatment in patients with MPE and morphologically different thromboemboli. Prospective observational study. An 11-bed closed medical ICU at a 860-bed community general hospital. Twelve consecutive patients with shock or hypotension due to MPE and central pulmonary thromboemboli detected by transesophageal echocardiography who were treated with thrombolysis between January 2000 through April 2002. Patients were divided into two groups according to the characteristics of detected central pulmonary thromboemboli: group 1, thrombi with one or more long, mobile parts; and group 2, immobile thrombi. Urokinase infusion was terminated when mixed venous oxygen saturation was stabilized above 60% for 15 min. At 2 h, the total pulmonary vascular resistance index was reduced more in group 1 than group 2 [from 27+/-12 mmHg/(l.min.m(2)) to 14+/-6 mmHg/(l.min.m(2)) (-52%) vs 27+/-8 mmHg/(l.min.m(2)) to 23+/-10 mmHg/(l.min.m(2)) (-15%), respectively, P=0.04]. In group 1 thrombolysis was terminated earlier than group 2 (89+/-40 min vs 210+/-62 min, respectively, P= 0.0024). The cumulative dose of urokinase used in group 1 was lower than group 2 (1.7+/-0.3 M i.u. vs 2.7+/-0.5 M i.u., respectively, P= 0.023). Haemodynamic stabilization is achieved faster in patients with mobile central thromboemboli detected by transesophageal echocardiography during MPE.

Mounier-Kuhn Syndrome in an Elderly Female with Pulmonary Fibrosis

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Panagiotis Boglou

2016-01-01

Full Text Available Mounier-Kuhn syndrome (MKS, or tracheobronchomegaly, is a rare clinical and radiologic condition characterized by pronounced tracheobronchial dilation and recurrent lower respiratory tract infections. Tracheobronchomegaly presents when the defect extends to the central bronchi. MKS can be diagnosed in adult women when the transverse and sagittal diameters of the trachea, right mainstem bronchus, and left mainstem bronchus exceed 21, 23, 19.8, and 17.4 mm, respectively. Its diagnosis is based on chest radiograph and chest computed tomography (CT. Patients, usually middle-aged men, may be asymptomatic or present with clinical manifestations ranging from minimal symptoms with preserved lung function to severe respiratory failure. Pulmonary function tests (PFTs typically reveal a restrictive pattern. This report presents an elderly woman with previously diagnosed pulmonary fibrosis with symptoms of increased sputum production and haemoptysis. High-resolution chest CT showed tracheal and main stem bronchi dilatation along with bronchial diverticulosis. PFTs indicated a restrictive pattern characteristic of the underlying pulmonary fibrosis. The patient is the oldest, referred to the female gender, at presentation of MKS hitherto reported. This case highlights the need to include MKS in the differential diagnosis of recurrent lower respiratory tract infections, even in older subjects.

Pulmonary hyperinflation and emphysema in infants with the Marfan syndrome

Energy Technology Data Exchange (ETDEWEB)

Dominguez, R; Weisgrau, R A; Santamaria, M

1987-07-01

Little recognition has been given to the pulmonary manifestations of the Marfan syndrome especially in infants. We report four cases of Marfan's syndrome presenting in infancy with respiratory abnormalities and a common radiographic picture of emphysema.

A case of William's syndrome associated peripheral pulmonary arterial stenosis

Energy Technology Data Exchange (ETDEWEB)

Jung, Kyung Hwa; Hwang, Mi Soo; Kim, Sun Yong; Chang, Jae Chun; Park, Bok Hwan [College of Medicine, Yeungam University, Daegu (Korea, Republic of)

1988-06-15

William's syndrome, in order to more completely delineate the total spectrum of the disorder, indicates that 'infantile hypercalcemia', 'peculiar facies' and 'supravalvular aortic stenosis.' In has other many vascular anomalies, such as peripheral pulmonary arterial stenosis, coronary arterial stenosis, celiac arterial stenosis, and renal aterial stenosis. Only 32% of the patients have evidence of supravalvular aortic stenosis. And it is very rare disease entity that has been reported rarely in Korea. Recently authors experienced a case that was questioned William's syndrome with peripheral pulmonary arterial stenosis, clinically and preliminary radiologically and this case was confirmed by operation. Here we report a case of William's syndrome with peripheral pulmonary arterial stenosis and reviewed literatures.

Pulmonary hyperinflation and emphysema in infants with the Marfan syndrome

International Nuclear Information System (INIS)

Dominguez, R.; Weisgrau, R.A.; Santamaria, M.

1987-01-01

Little recognition has been given to the pulmonary manifestations of the Marfan syndrome especially in infants. We report four cases of Marfan's syndrome presenting in infancy with respiratory abnormalities and a common radiographic picture of emphysema. (orig./MG)

Pulmonary hyperinflation and emphysema in infants with the Marfan syndrome

Energy Technology Data Exchange (ETDEWEB)

Dominguez, R.; Weisgrau, R.A.; Santamaria, M.

1987-07-01

Little recognition has been given to the pulmonary manifestations of the Marfan syndrome especially in infants. We report four cases of Marfan's syndrome presenting in infancy with respiratory abnormalities and a common radiographic picture of emphysema.

A syndrome of severe idiopathic pulmonary parenchymal disease with pulmonary hypertension in Pekingese

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Köster LS

2016-02-01

Full Text Available Liza S Köster,1 Robert M Kirberger2 1Section of Medicine, Department of Clinical Sciences, Integrative Mammalian Research (IMR Center, Ross University School of Veterinary Medicine (RUSVM, Basseterre, St Kitts, West Indies; 2Diagnostic Imaging Section, Department of Companion Animal Clinical Studies, Faculty of Veterinary Science, University of Pretoria, Onderstepoort, South Africa Abstract: This paper describes 35 Pekingese dogs with a syndrome characterized by dyspnea, cyanosis, episodic syncope, soft pulmonary “Velcro” crackles, pulmonary hypertension (PH, and computed tomography and radiographic changes consistent with pulmonary parenchymal disease. The medical data base was searched with the criteria “Pekingese” and “syncope” or “dyspnea” or “tachypnea” or “pulmonary hypertension”, over a 36-month period. Inclusion criteria were echocardiographic changes consistent with noninvasive diagnosis of PH, either subjectively by B-mode or objectively by Doppler. Dogs were excluded (n=106 if there were insufficient or poor-quality radiographic or echocardiographic records or if diseases other than chronic pulmonary disease were found to be the etiology. The records of 35 dogs met these criteria and presented with a respiratory crises preceded by a history of chronic exercise intolerance and episodic syncope. The average age was 14.5 years (range: 7–19 years, with 21 males and 14 females. Most of the dogs had an interstitial lung pattern with radiographic evidence of right heart enlargement. There was a 77% (n=27 mortality and a median survival of 60 days (interquartile range: 9–210 days. This study highlights a cor pulmonale syndrome from PH due to chronic pulmonary parenchymal disease, with a grave prognosis, in middle-aged to geriatric population of Hong Kong Pekingese. Keywords: computed tomography, interstitial lung disease, dog, syncope

Pulmonary involvement of hypereosinophilic syndrome : high-resolution CT finding in three patients

Energy Technology Data Exchange (ETDEWEB)

Kang, Eun Young; Lee, Mee Ran; Shim, Jae Jeong [Korea Univ. College of Medicine, Seoul (Korea, Republic of)

1996-09-01

Hypereosinophilic syndrome is a rare entity of eosinophilic lung disease characterized by idiopathic prolonged eosinophilia of marked degree and variable organ involvement. Pulmonary involvement of hypereosinophilic syndrome occurs in up to 40% of patients. We report HRCT findings of three patients with pulmonary involvement of hypereosinophilic syndrome diagnosed by clinical manifestation, bronchoalveolar lavage and transbronchial lung biopsy. On HRCT, several small nodules were seen in both lungs, especially in peripheral lung areas of the three patients. One had nodules with ground-glass attenuation halo and also focal areas of ground-glass attenuation in this area.

The association between the metabolic syndrome and metabolic syndrome score and pulmonary function in non-smoking adults.

Science.gov (United States)

Yoon, Hyun; Gi, Mi Young; Cha, Ju Ae; Yoo, Chan Uk; Park, Sang Muk

2018-03-01

This study assessed the association of metabolic syndrome and metabolic syndrome score with the predicted forced vital capacity and predicted forced expiratory volume in 1 s (predicted forced expiratory volume in 1 s) values in Korean non-smoking adults. We analysed data obtained from 6684 adults during the 2013-2015 Korean National Health and Nutrition Examination Survey. After adjustment for related variables, metabolic syndrome ( p metabolic syndrome score ( p metabolic syndrome score with metabolic syndrome score 0 as a reference group showed no significance for metabolic syndrome score 1 [1.061 (95% confidence interval, 0.755-1.490)] and metabolic syndrome score 2 [1.247 (95% confidence interval, 0.890-1.747)], but showed significant for metabolic syndrome score 3 [1.433 (95% confidence interval, 1.010-2.033)] and metabolic syndrome score ⩾ 4 [1.760 (95% confidence interval, 1.216-2.550)]. In addition, the odds ratio of restrictive pulmonary disease of the metabolic syndrome [1.360 (95% confidence interval, 1.118-1.655)] was significantly higher than those of non-metabolic syndrome. Metabolic syndrome and metabolic syndrome score were inversely associated with the predicted forced vital capacity and forced expiratory volume in 1 s values in Korean non-smoking adults. In addition, metabolic syndrome and metabolic syndrome score were positively associated with the restrictive pulmonary disease.

Bilateral branch pulmonary artery stenosis and Mitral valve prolapse in a patient with Noonan syndrome: A case report

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Meenakshi Kadiyala

2014-01-01

Full Text Available Rasopathy syndromes are a class of phenotypically similar, but genetically distinct multiple anomaly syndromes caused by germ line mutations in genes that encode protein components of the Ras/mitogen activated protein kinase (MAPK pathway. Noonan syndrome, cardiofaciocutaneous syndrome and Costello syndrome are part of this group of developmental syndromes and have similar cardiac abnormalities. A 19-year-old male presented with complaints of exertional breathlessness class I for 6 months. Clinical examination revealed characteristic facial features, skeletal abnormalities, growth and neurocognitive problems reported in patients with Noonan syndrome. There was evidence of severe pulmonary hypertension. Trans-thoracic echocardiography revealed right atrial and right ventricular enlargement, severe pulmonary hypertension, no intra cardiac shunt, prolapse of anterior mitral leaflet with mild mitral regurgitation. CT pulmonary angiogram revealed bilateral branch pulmonary artery stenosis. A final diagnosis of Noonan syndrome was made.

Severe Cushing's syndrome and bilateral pulmonary nodules: beyond ectopic ACTH.

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Tavares Bello, Carlos; van der Poest Clement, Emma; Feelders, Richard

2017-01-01

Cushing's syndrome is a rare disease that results from prolonged exposure to supraphysiological levels of glucocorticoids. Severe and rapidly progressive cases are often, but not exclusively, attributable to ectopic ACTH secretion. Extreme hypercortisolism usually has florid metabolic consequences and is associated with an increased infectious and thrombotic risk. The authors report on a case of a 51-year-old male that presented with severe Cushing's syndrome secondary to an ACTH-secreting pituitary macroadenoma, whose diagnostic workup was affected by concurrent subclinical multifocal pulmonary infectious nodules. The case is noteworthy for the atypically severe presentation of Cushing's disease, and it should remind the clinician of the possible infectious and thrombotic complications associated with Cushing's syndrome. Severe Cushing's syndrome is not always caused by ectopic ACTH secretion.Hypercortisolism is a state of immunosuppression, being associated with an increased risk for opportunistic infections.Infectious pulmonary infiltrates may lead to imaging diagnostic dilemmas when investigating a suspected ectopic ACTH secretion.Cushing's syndrome carries an increased thromboembolic risk that may even persist after successful surgical management.Antibiotic and venous thromboembolism prophylaxis should be considered in every patient with severe Cushing's syndrome.

Imaging findings of pulmonary vascular disorders in portal hypertension

International Nuclear Information System (INIS)

Nagasawa, Kenichi; Takahashi, Koji; Furuse, Makoto

2004-01-01

The purpose of this study was to demonstrate and compare the imaging findings of hepatopulmonary syndrome and portopulmonary hypertension. We retrospectively reviewed the imaging findings of five patients with hepatopulmonary syndrome and four patients with portopulmonary hypertension. We evaluated chest radiographs, chest and abdominal computed tomography (CT) scans, 99m Tc-macroaggregated albumin (MAA) lung perfusion scans, and pulmonary angiograms. In patients with hepatopulmonary syndrome, the presence of peripheral pulmonary vascular dilatation was detected by chest radiograph, chest CT scan, and pulmonary angiogram, especially the basilar segment. 99m Tc-MAA lung perfusion scan showed extrapulmonary tracer distribution (brain, thyroid, and kidney), which revealed pulmonary right-left shunting. In patients with portopulmonary hypertension, chest radiographs and chest CT scans showed the classic findings of primary pulmonary hypertension. In patients with both disorders, extrahepatic features of portal hypertension including ascites, splenomegaly, and portosystemic collateral vessels were seen on abdominal CT. In conclusion, chest radiographs and CT in hepatopulmonary syndrome usually showed peripheral pulmonary vascular dilatation, whereas those in portopulmonary hypertension showed central pulmonary artery dilatation. The extrahepatic features of portal hypertension might be helpful for the diagnosis of both disorders. (author)

Pulmonary Renal Syndrome After Streptococcal Pharyngitis

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Gopi Mara-Koosham PhD

2016-04-01

Full Text Available Pulmonary renal syndrome is a class of small vessel vasculitides that are characterized by the dual presentation of diffuse alveolar hemorrhage (DAH and glomerulonephritis. Pulmonary renal syndrome has multiple etiologies, but its development has been rarely reported following infection with group A streptococcus. We present the case of a 36-year-old Native American male who was transferred to our facility due to refractory hypoxic respiratory failure. He had been diagnosed with streptococcal pharyngitis 2 weeks prior to admission. Given the presence of hemoptysis, bronchoscopy was performed and was consistent with DAH. Urinalysis demonstrated hematuria and proteinuria, in the setting of elevated creatinine and blood urea nitrogen. Additionally, antistreptolysin O titer was positive. Given the constellation of laboratory findings and history of streptococcal pharyngitis, the patient was diagnosed with PRS secondary to streptococcal infection. High-dose methylprednisolone was initiated with concomitant plasmapheresis. He was extubated successfully after his respiratory status improved and was eventually discharged home after making a full recovery within 2 weeks after admission. This case illustrates the importance of clinically relevant sequelae of streptococcal infection as well as the appropriate treatment of PRS secondary to streptococcal pharyngitis with plasmapheresis and intravenous corticosteroids.

Nitric Oxide-Sensitive Pulmonary Hypertension in Congenital Rubella Syndrome

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Francesco Raimondi

2015-01-01

Full Text Available Persistent pulmonary hypertension is a very rare presentation of congenital virus infection. We discuss the case of complete congenital rubella syndrome presenting at echocardiography with pulmonary hypertension that worsened after ductus ligation. Cardiac catheterization showed a normal pulmonary valve and vascular tree but a PAP=40 mmHg. The infant promptly responded to inhaled nitric oxide while on mechanical ventilation and was later shifted to oral sildenafil. It is not clear whether our observation may be due to direct viral damage to the endothelium or to the rubella virus increasing the vascular tone via a metabolic derangement.

Severe pulmonary hypertension associated with the acute motor sensory axonal neuropathy subtype of Guillain-Barré syndrome.

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Rooney, Kris A; Thomas, Neal J

2010-01-01

To evaluate pulmonary hypertension associated with acute motor sensory axonal neuropathy subtype of Guillain-Barré syndrome. Guillain-Barré syndrome consists of a group of autoimmune disorders that generally manifest as symmetric, progressive, ascending paralysis. There are five subtypes of Guillain-Barré syndrome, and autonomic involvement has been described in all subtypes, including cardiovascular, vasomotor, or pseudomotor dysfunction of both the sympathetic and parasympathetic systems. Case report. Tertiary care pediatric intensive care unit. Three-yr-old female patient. None. Serial measurements of pulmonary artery pressure. We report the case of a young girl with acute motor sensory axonal neuropathy who presented with severe cardiovascular collapse secondary to severe pulmonary hypertension. In this patient, multiple factors may have played a role in the development of pulmonary hypertension including autonomic dysfunction, hypoventilation, and immobility as a risk for thrombosis and pulmonary emboli. It is possible that many other individuals suffering from severe forms of Guillain-Barré syndrome, especially those with significant autonomic dysfunction, may actually have undiagnosed and therefore untreated pulmonary hypertension. Therefore, it is recommended that clinicians caring for critically ill children with Guillain-Barré syndrome have a high index of suspicion for pulmonary hypertension and consider echocardiography if there are clinical signs of this potentially fatal process.

Pulmonary scan in evaluating alveolar-interstitial syndrome in ER

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Giovanni Volpicelli

2006-10-01

Full Text Available Diffuse comet-tail artifacts at lung ultrasound are due to thickened interlobular septa and extravascular lung water. This condition is typical of the alveolar-interstitial syndrome due to pulmonary edema, diffuse parenchymal lung disease or ARDS. Aim of our study is to assess the potential of bedside lung ultrasound to diagnose the alveolar-interstitial syndrome in patients admitted to our emergency medicine unit. The ultrasonic feature of multiple and diffuse comet-tail artifacts was investigated during 5 months, in 121 consecutive patients admitted to our unit. Each patient was studied bedside in a supine position, by 8 antero-lateral pulmonary intercostal scans. Ultrasonic results were compared with chest radiograph and clinical outcome. Lung ultrasound showed a sensitivity of 84% and a specificity of 98% in diagnosing the radiologic alveolar-interstitial syndrome. Corresponding figures in the identification of a disease involving lung interstitium were 83% and 96%. These preliminary data show that the study of comet-tail artifacts at lung ultrasound is a method reasonably accurate for diagnosing the alveolar-interstitial syndrome at bedside. This conclusion opens the hypothesis of the usefullness of bedside lung ultrasound in the evaluation of dyspnoeic patients in the emergency setting.

Pulmonary hypertension due to acute respiratory distress syndrome

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S.A. Ñamendys-Silva

2014-10-01

Full Text Available Our aims were to describe the prevalence of pulmonary hypertension in patients with acute respiratory distress syndrome (ARDS, to characterize their hemodynamic cardiopulmonary profiles, and to correlate these parameters with outcome. All consecutive patients over 16 years of age who were in the intensive care unit with a diagnosis of ARDS and an in situ pulmonary artery catheter for hemodynamic monitoring were studied. Pulmonary hypertension was diagnosed when the mean pulmonary artery pressure was >25 mmHg at rest with a pulmonary artery occlusion pressure or left atrial pressure <15 mmHg. During the study period, 30 of 402 critically ill patients (7.46% who were admitted to the ICU fulfilled the criteria for ARDS. Of the 30 patients with ARDS, 14 met the criteria for pulmonary hypertension, a prevalence of 46.6% (95% CI; 28-66%. The most common cause of ARDS was pneumonia (56.3%. The overall mortality was 36.6% and was similar in patients with and without pulmonary hypertension. Differences in patients' hemodynamic profiles were influenced by the presence of pulmonary hypertension. The levels of positive end-expiratory pressure and peak pressure were higher in patients with pulmonary hypertension, and the PaCO2 was higher in those who died. The level of airway pressure seemed to influence the onset of pulmonary hypertension. Survival was determined by the severity of organ failure at admission to the intensive care unit.

Imaging Findings of Central Nervous System Vasculitis Associated with Goodpasture's Syndrome: a Case Report

International Nuclear Information System (INIS)

Kim, Jee Young; Ahn, Kook Jin; Jung, Jung Im; Jung, So Lyung; Kim, Bum Soo; Hahn, Seong Tae

2007-01-01

We report a rare case of CNS vasculitis associated with Goodpasture's syndrome in a 34-year-old man, who presented with a seizure and sudden onset of right sided weakness. He also had recurrent hemoptysis of one month's duration. Goodpasture's syndrome is histologically diagnosed by intense linear deposits of IgG along the glomerular basement membrane in both renal and lung tissues. oodpasture's syndrome is a rare disease, characterized by rapidly progressive glomerulonephritis, diffuse pulmonary hemorrhage and circulating antiglomerular basement membrane antibody (anti-GBM antibody). Central nervous system (CNS) manifestations in Goodpasture's syndrome are extremely rare, with only a few cases having been reported in the literature (8 10). Therefore, we present our imaging findings of CNS vasculitis associated with Goodpasture's syndrome, together with a review of the relevant literature. In summary, CNS vasculitis associated with Goodpasture's syndrome is extremely rare. Awareness of the imaging findings, as well as the clinical significance of CNS vasculitis associated with Goodpasture's syndrome, can be helpful in making the correct diagnosis and subsequent management of this rare condition

Partial anomalous pulmonary venous return in Turner syndrome

NARCIS (Netherlands)

Hoven, A.T. van den; Chelu, R.G.; Duijnhouwer, A.L.; Demulier, L.; Devos, D.; Nieman, K.; Witsenburg, M.; Bosch, A.E. van den; Loeys, B.L.; Hagen, I.M. van; Roos-Hesselink, J.W.

2017-01-01

PURPOSE: The aim of this study is to describe the prevalence, anatomy, associations and clinical impact of partial anomalous pulmonary venous return in patients with Turner syndrome. METHODS AND RESULTS: All Turner patients who presented at our Turner clinic, between January 2007 and October 2015

Pulmonary Arterial Stent Implantation in an Adult with Williams Syndrome

International Nuclear Information System (INIS)

Reesink, Herre J.; Henneman, Onno D. F.; Delden, Otto M. van; Biervliet, Jules D.; Kloek, Jaap J.; Reekers, Jim A.; Bresser, Paul

2007-01-01

We report a 38-year-old patient who presented with pulmonary hypertension and right ventricular dysfunction due to pulmonary artery stenoses as a manifestation of Williams syndrome, mimicking chronic thromboembolic pulmonary hypertension. The patient was treated with balloon angioplasty and stent implantation. Short-term follow-up showed a good clinical result with excellent patency of the stents but early restenosis of the segments in which only balloon angioplasty was performed. These stenoses were subsequently also treated successfully by stent implantation. Stent patency was observed 3 years after the first procedure

Acute Pulmonary Edema in an Eclamptic Pregnant Patient: A Rare Case of Takotsubo Syndrome.

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Karamchandani, Kunal; Bortz, Brandon; Vaida, Sonia

2016-09-23

BACKGROUND Acute pulmonary edema in a pregnant patient is associated with significant morbidity and mortality. Takotsubo syndrome, or stress-induced cardiomyopathy, is a rare cause of acute pulmonary edema in a pregnant patient, especially prior to delivery of the fetus. CASE REPORT We describe a case of a pregnant patient who presented with acute pulmonary edema and eclampsia and was found to have Takotsubo syndrome. To the best of our knowledge, eclampsia as a precipitating factor for Takotsubo syndrome has not been described in literature. CONCLUSIONS Clinicians taking care of pregnant patients should be aware of the potential link between eclampsia and Takotsubo cardiomyopathy. Prompt correction of the precipitating cause along with supportive management as described is the key to a successful outcome.

Unprovoked Pulmonary Embolism in a Young Patient with Marfan Syndrome.

Science.gov (United States)

Pak, Stella; Kilgore, Andrew; Thornhill, Rosanne; Rako, Kyle; Meier, Ali; Pora, Gavriella; Costello, Jillian M; Dee, Christine

2017-09-05

Marfan syndrome is a rare connective tissue disorder with a prevalence of approximately 2 to 3 per 10,000 individuals. There have been some reports of young patients with Marfan syndrome developing arteriovenous thromboembolism. These events were unprovoked and recurrent. Owing to its rarity, hypercoagulopathy and other metabolic derangement in patients with Marfan syndrome remains largely unknown. Herein, we report a case of a young man with Marfan syndrome who had myocardial infarction and pulmonary embolism. We hope that this case adds to the scant body of knowledge about this patient population.

Central Pain Syndrome

Science.gov (United States)

... such as neurontin (gabapentin) can be useful. Lowering stress levels appears to reduce pain. View Full Treatment Information Definition Central pain syndrome is a neurological condition caused ...

Tricuspid valve endocarditis with pulmonary infarction caused by central venous catheter

International Nuclear Information System (INIS)

Grabbe, E.; Guthoff, A.; Hamburg Univ.

1981-01-01

Knowledge of common complications of central venous catheters is completed by a case of bacterial tricuspid endocarditis with recurrent pulmonary infarction. This rare, life threatening complication should be considered in differential diagnosis, when in case of central venous catheter sepsis, changing pulmonary infiltrations with pleural effusion as well as different auscultatory findings above the tricuspid valve do occur. The diagnosis can be supported by echocardiographic demonstration of tricuspid vegetations. (orig.) [de

Tricuspid valve endocarditis with pulmonary infarction caused by central venous catheter

Energy Technology Data Exchange (ETDEWEB)

Grabbe, E; Guthoff, A

1981-02-01

Knowledge of common complications of central venous catheters is completed by a case of bacterial tricuspid endocarditis with recurrent pulmonary infarction. This rare, life threatening complication should be considered in differential diagnosis, when in case of central venous catheter sepsis, changing pulmonary infiltrations with pleural effusion as well as different auscultatory findings above the tricuspid valve do occur. The diagnosis can be supported by echocardiographic demonstration of tricuspid vegetations.

Mac Leod's syndrome

International Nuclear Information System (INIS)

Schad, M.; Danesi, C.; Ricci, R.; Galluzzi, S.; Coviello, G.

1988-01-01

Mac Leod's syndrome is a rarely diagnosed disease; that is why an accurate differential diagnosis is needed by means of radiological imaging. This paper is aimed at discussing the differential diagnosis, with a special emphasis on the pathogenesis of the syndrome. The phenomenon of air trapping in absence of central bronchial lesions is a typical radiographic finding. Chest X-ray is performed in both inspiration and expiration. Posterior oblique tomography at 55 grade centigrade of the effected side is also performed. Diffuse bronchiolitis obliterans in infancy or early childhood ia widely accepted pathogenetic pattern. Pulmonary hypoventilation causes vasoconstriction and underdevelopment of pulmonary vessels, that are reduced in caliber. Differential diagnosis includes all the diseases resulting in pulmonary hyperlucency, i.e. pulmonary and pleural alterations, and skeletal anomalies

A Rare Cause of Persistent Pulmonary Hypertension Resistant to Therapy in The Newborn: Short-Rib Polydactyly Syndrome

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Nihat Demir

2015-01-01

Full Text Available Short-rib polydactyly syndrome is an autosomal recessively inherited lethal skeletal dysplasia. The syndrome is characterized by marked narrow fetal thorax, short extremities, micromelia, cleft palate/lip, polydactyly, cardiac and renal abnormalities, and genital malformations. In cases with pulmonary hypoplasia, persistent pulmonary hypertension of the newborn can develop. In this paper, we present a term newborn with persistent pulmonary hypertension of the newborn, which has developed secondary to short-rib polydactyly syndrome and was resistant to therapy with inhaled nitric oxide and oral sildenafil.

Importance of absent ductus arteriosus in tetralogy of Fallot with absent pulmonary valve syndrome.

Science.gov (United States)

Qureshi, Muhammad Yasir; Burkhart, Harold M; Julsrud, Paul; Cetta, Frank

2014-12-01

Tetralogy of Fallot without pulmonary valve syndrome is almost always associated with an absent ductus arteriosus. Patients with right aortic arch and retroesophageal left subclavian artery have a vascular ring if the left ductus arteriosus or its remnant and the Kommerell diverticulum are present. We report the cases of 2 infants in whom the role of an absent ductus arteriosus or its remnant is noteworthy. Both patients had a combination of tetralogy of Fallot with absent pulmonary valve syndrome and right aortic arch with retroesophageal left subclavian artery without a vascular ring. The absence of the ductus arteriosus has a role in the pathogenesis of tetralogy of Fallot with absent pulmonary valve syndrome. The absence of a ductus arteriosus in the right aortic arch with retroesophageal left subclavian artery precludes a vascular ring.

Síndromes hemorrágicas pulmonares Pulmonary hemorrhage syndromes

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Eduardo da Rosa Borges

2005-07-01

Full Text Available As síndromes hemorrágicas pulmonares caracterizam-se por infiltrado pulmonar bilateral, queda dos níveis de hemoglobina e hipoxemia. Dentre as causas de sangramento estão as infecções, vasculites, coagulopatias e doenças do colágeno. A terapêutica consiste do tratamento da doença causal e suporte ventilatório, podendo ser associada a plasmaferese.Pulmonary hemorrhage syndromes are characterized by bilateral pulmonary infiltrates, decreased serum levels of hemoglobin, and hypoxemia. The causes of pulmonary hemorrhage include: infections, vasculitis, coagulopathies and collagen diseases. The therapy consists of treating the underlying disease and providing ventilatory support. In some cases, performing plasmapheresis can be beneficial.

Partial anomalous pulmonary venous return in Turner syndrome.

Science.gov (United States)

van den Hoven, Allard T; Chelu, Raluca G; Duijnhouwer, Anthonie L; Demulier, Laurent; Devos, Daniel; Nieman, Koen; Witsenburg, Maarten; van den Bosch, Annemien E; Loeys, Bart L; van Hagen, Iris M; Roos-Hesselink, Jolien W

2017-10-01

The aim of this study is to describe the prevalence, anatomy, associations and clinical impact of partial anomalous pulmonary venous return in patients with Turner syndrome. All Turner patients who presented at our Turner clinic, between January 2007 and October 2015 were included in this study and underwent ECG, echocardiography and advanced imaging such as cardiac magnetic resonance or computed tomography as part of their regular clinical workup. All imaging was re-evaluated and detailed anatomy was described. Partial anomalous pulmonary venous return was diagnosed in 24 (25%) out of 96 Turner patients included and 14 (58%) of these 24 partial anomalous pulmonary venous return had not been reported previously. Right atrial or ventricular dilatation was present in 11 (46%) of 24 partial anomalous pulmonary venous return patients. When studied with advanced imaging modalities and looked for with specific attention, PAPVR is found in 1 out of 4 Turner patients. Half of these patients had right atrial and/or ventricular dilatation. Evaluation of pulmonary venous return should be included in the standard protocol in all Turner patients. Copyright © 2017. Published by Elsevier B.V.

Hypoplastic left heart syndrome and pulmonary veno-occlusive disease in an infant.

Science.gov (United States)

D'Souza, Marise; Vergales, Jeffrey; Jayakumar, K Anitha

2013-01-01

This report describes an infant with heterotaxy syndrome and severe hypoplasia of the left heart who presented with profound cyanosis at birth despite a large patent ductus arteriosus. Pulmonary venous return was difficult to demonstrate by echocardiography. Angiography showed total anomalous pulmonary venous return via a plexus that drained through the paravertebral veins and bilateral superior vena cavae. Autopsy confirmed these findings, and histopathology demonstrated severe occlusive changes within the pulmonary veins.

Metabolic Syndrome in Chemical Warfare Patients with Chronic Obstructive Pulmonary Disease

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Shahrzad M. Lari

2014-11-01

Full Text Available   Introduction: Sulfur mustard (SM, a toxic alkylating gas, can cause serious long-term pulmonary complications such as chronic obstructive pulmonary disease (COPD. Metabolic syndrome (MetS is one of the important comorbidities of COPD. This study was designed to evaluate the frequency of metabolic syndrome in Iranian chemical warfare patients (CWPs with COPD. Materials and Methods: Thirty CWPs with a mean age of 46.93± 6.8 were enrolled in this study. The following parameters were studied in: complete pulmonary function tests, health-related quality of life, serum triglycerides (TG, high density lipoprotein (HDL and fasting blood sugar (FBS levels. Additionally, 32 COPD patients and 56 healthy persons were considered as control groups who were matched to CWPs. Results: We found a statistically significant difference in the frequency of MetS between the COPD patients and the healthy control group (p=0.04. Additionally, we observed a statistically significant difference in the mean HDL levels among these groups (p=

Pulmonary Hypertension Syndrome in Chicken: Peeking Under QTL Peaks

NARCIS (Netherlands)

Rabie, T.

2004-01-01

Pulmonary hypertension syndrome (PHS) is characterized a cascade of events resulting in cardiac anomalies including an enlarged heart, right ventricular hypertrophy, variable liver changes, and accumulation of fluid in the abdominal cavity (ascites). PHS is not a disease; it is a condition in which

Psychosocial Factors and Central Sensitivity Syndromes

OpenAIRE

Adams, Leah M.; Turk, Dennis C.

2015-01-01

Central sensitivity syndromes (CSSs) represent a heterogeneous group of disorders (e.g., fibromyalgia [FM], irritable bowel syndrome [IBS], chronic headache, temporomandibular disorders [TMDs], pelvic pain syndromes) that share common symptoms, with persistent pain being the most prominent feature.

Hyper Ig E syndrome (Job syndrome, HIES) – radiological images of pulmonary complications on the basis of three cases

International Nuclear Information System (INIS)

Jończyk-Potoczna, Katarzyna; Szczawińska-Popłonyk, Aleksandra; Warzywoda, Małgorzata; Bręborowicz, Anna; Pawlak, Bogdan

2012-01-01

Hyperimmunoglobulinemia E syndrome (hyper-IgE syndrome, Job syndrome, HIES) is a complex immune deficiency with multiorgan clinical manifestations and diverse genetic background. The clinical triad of symptoms observed in approximately 75% of patients with HIES includes: recurrent abscesses of staphylococcal etiology, recurrent respiratory infections and elevated immunoglobulin E in serum. The paper discusses three cases of female patients presenting typical pulmonary complications of the hyper-Ig E syndrome. In the first case, the development of aspergilloma in a postinflamatory cyst was observed, in the other one, pneumonia with pleural effusion, and as a consequence of inflammatory infiltrations – fibrotic changes, giving rise to lobectomy, while in the last of these cases, the course of lung disease was complicated by formation of staphylococcal abscess. In one of the girls, bronchiectasis appeared at follow-up. Complications of pulmonary infections are the most common causes of death in hyper-Ig E syndrome. Late diagnosis significantly worsens the respiratory function and reduces the chance for normal development of a child. Introduction of comprehensive treatment, including prophylaxis, decreases the recurrences. Therefore, the important role is attributed to the radiologist in the multidisciplinary care of patients with this syndrome

Surgical treatment of tetralogy of Fallot with absent pulmonary valve syndrome.

Science.gov (United States)

Tanaka, Yuki; Miyamoto, Takashi; Naito, Yuji; Yoshitake, Shuichi

2016-06-01

The patient was a 3-month-old girl weighting 3.6 kg, diagnosed with tetralogy of Fallot and absent pulmonary valve syndrome. We surgically repaired the tetralogy of Fallot by patch closure of the ventricular septal defect, right ventricular outflow tract reconstruction using an expanded polytetrafluoroethylene monocusp patch with a bulging sinus, and removal of the bronchial obstruction by anterior translocation of the pulmonary artery using the Lecompte maneuver. © The Author(s) 2015.

Genetics Home Reference: congenital central hypoventilation syndrome

Science.gov (United States)

... Kravis EM, Zhou L, Rand CM, Weese-Mayer DE. Congenital central hypoventilation syndrome: PHOX2B mutations and phenotype. Am J Respir Crit ... BA, Leurgans SE, Berry-Kravis EM, Weese-Mayer DE. Congenital central hypoventilation syndrome: PHOX2B genotype determines risk for sudden death. Pediatr ...

Hantavirus pulmonary syndrome in Tucumán province associated to an unexpected viral genotype

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Matías Ciancaglini

2017-04-01

Full Text Available We describe the characterization of the viral genotype involved in the first case of hantavirus pulmonary syndrome reported in Tucumán, a Northwestern province of Argentina. A 23-year-old woman, with no record of travel history and previously diagnosed with an antiphospholipid syndrome, died after 11 days of severe cardiopulmonary insufficiency. Among the four endemic regions of hantavirus pulmonary syndrome in Argentina, the Northwest Region has the highest incidence, exceeding 50% of all reported cases in the country. Until now, only Salta and Jujuy (2 out of the 6 provinces composing the Northwest Region, reported cases of hantavirus pulmonary syndrome, all of which occurred in the Yungas Forest area. Remarkably, the viral genotype characterized in this case showed higher nucleotide identity with the Andes-BsAs genotype most prevalent in Buenos Aires province, located 1400 km apart from Tucumán, than with any of the commonly found genotypes in the Northwest Region. The Andes-BsAs genotype has been associated with 30% lethality and interhuman transmission in Buenos Aires province. Interhuman transmission cannot be ruled out in the present case

Pulmonary manifestations of Sjögren's syndrome

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Thomas Flament

2016-06-01

Full Text Available In 9–20% of cases, Sjögren's syndrome is associated with various respiratory symptoms. The most typical manifestations are chronic interstitial lung disease (ILD and tracheobronchial disease. The most common manifestation of ILD is nonspecific interstitial pneumonia in its fibrosing variant. Other types of ILD, such as organising pneumonia, usual interstitial pneumonia and lymphocytic interstitial pneumonitis, are rare. Their radiological presentation is less distinctive, and definitive diagnosis may require the use of transbronchial or surgical lung biopsy. Corticosteroid therapy is the mainstay of ILD treatment in Sjögren's syndrome, but the use of other immunosuppressive drugs needs to be determined. ILD is a significant cause of death in Sjögren's syndrome. Tracheobronchial disease is common in Sjögren's syndrome, characterised by diffuse lymphocytic infiltration of the airway. It is sometimes responsible for a crippling chronic cough. It can also present in the form of bronchial hyperresponsiveness, bronchiectasis, bronchiolitis or recurrent respiratory infections. The management of these manifestations may require treatment for dryness and/or inflammation of the airways. Airway disease has little effect on respiratory function and is rarely the cause of death in Sjögren's syndrome patients. Rare respiratory complications such as amyloidosis, lymphoma or pulmonary hypertension should not be disregarded in Sjögren's syndrome patients.

Use of spiral CT angiography to judge central pulmonary vascular involvement from lung cancer

International Nuclear Information System (INIS)

Tan Qunyou; Zhao Shaohong; Wang Fangze; Cai Zulong

2000-01-01

Objective: To evaluate the accuracy of spiral CT angiography (SCTA) in judging central pulmonary vascular involvement from lung cancer located in the hilum and correlate the resultant images with pathologic and surgical findings. Methods: SCTA was done in 33 patients who were preoperatively diagnosed as having lung carcinoma located in the hilum. Contrast medium was injected at a rate of 3 ml/sec with a power injector. The delay time was from 20 to 25 seconds. The pitch was 1 with 3 mm-collimation. Images of central pulmonary arteries and veins were reconstructed with shaded surface display (SSD), maximum intensity projection (MIP), curved planar reformation (CPR), and multi-planar reformation (MPR). Then the relation between tumor and vessels was assessed prospectively on both 3 mm interval axial CT and SCTA images with comparison to subsequent pathologic or surgical findings. Results: (1) In showing the integrity of central pulmonary arteries and veins, images reconstructed by different ways of SCTA had different strong and weak points. (2) The grading standard in this study, with which the relation between vessels and tumor was judged, reflected the basic and common characters of central pulmonary vascular involvement by tumor located in the hilum. compared with axial CT images, SCTA was more accurate in judging the relation between central pulmonary vessels and tumor, and the correlation of SCTA imaging features with pathological patterns and surgical findings was better than that of axial CT images, P < 0.05 and P < 0.0001, respectively. Conclusion: It was feasible to show the relation between central pulmonary vessel and lung cancer located in the hilum with SCTA. And the accuracy of judging the vascular involvement with SCTA was higher than that with axial CT

A comparative study of pulmonary hypoperfusion secondary to cancerous invasion of central pulmonary artery in lung cancer with CT and DSA

International Nuclear Information System (INIS)

Guo Dajing; Miao Jingtao; Lu Wusheng; Bai Hongli

2003-01-01

Objective: To study CT features of pulmonary hypoperfusion in lung cancer with cancerous invasion of central pulmonary artery (CICPA) and the diagnostic value of CT. Methods: The pre-operative pulmonary DSA and contrast-enhanced spiral CT (including HRCT) findings in 20 cases of lung cancer with CICPA were carried out prospectively and comparatively. On DSA and CT, the distribution and quantitative variation of subpleural terminal pulmonary arterioles (pulmonary vessel signs), the filling degree of lung capillary, the post-contrast density changes of lung parenchyma, and the fluctuations of the time-density curve (pulmonary parenchymal signs) were blindly observed. The CT and DSA findings and their consistence were analyzed statistically. The diagnostic values of CT in secondary pulmonary hypoperfusion in lung cancer with CICPA were evaluated. Results: There were 30 lobar arteries with cancerous invasion among the 20 cases and 90% (27/30) of them presented pulmonary hypoperfusion on DSA. For diagnosing pulmonary hypoperfusion, CT had a sensitivity of 78%(21/27), a specificity of 100% (3/3), and an accuracy of 80%(24/30). There were no statistical correlation between pulmonary hypoperfusion and the degree of central pulmonary artery (CPA) stenosis (r s =0.40, P=0.073). Pulmonary vessel signs on CT had a good consistence with that on DSA (Kappa=0.69, P<0.001), while that of pulmonary parenchymal signs was lower (Kappa=0.37, P=0.011). Conclusion: Pulmonary hypoperfusion is one of the common findings in lung cancer with CICPA. CT is of great value in qualitative diagnosis on it, while for quantitative diagnosis, its value is limited to a certain degree

Leptospirosis-Associated Severe Pulmonary Hemorrhagic Syndrome with Lower Back Pain as an Initial Symptom

DEFF Research Database (Denmark)

Søndergaard, Mads Madsen; tursunovic, Amela; Thye-Roenn, Peter

2016-01-01

BACKGROUND Leptospirosis is a zoonosis transmitted through urine of infected animals. Symptoms range from mild influenza-like symptoms to severe pulmonary hemorrhagic syndrome (SPHS); the latter are often fatal. The serogroup distribution in Denmark has changed from 1988 to 2012, with Icterohaemo......BACKGROUND Leptospirosis is a zoonosis transmitted through urine of infected animals. Symptoms range from mild influenza-like symptoms to severe pulmonary hemorrhagic syndrome (SPHS); the latter are often fatal. The serogroup distribution in Denmark has changed from 1988 to 2012......, the patient died a few hours after hospital admission....

Imaging Findings of Central Nervous System Vasculitis Associated with Goodpasture's Syndrome: a Case Report

Energy Technology Data Exchange (ETDEWEB)

Kim, Jee Young; Ahn, Kook Jin; Jung, Jung Im; Jung, So Lyung; Kim, Bum Soo; Hahn, Seong Tae [College of Medicine, The Catholic University of Korea, Seoul (Korea, Republic of)

2007-12-15

We report a rare case of CNS vasculitis associated with Goodpasture's syndrome in a 34-year-old man, who presented with a seizure and sudden onset of right sided weakness. He also had recurrent hemoptysis of one month's duration. Goodpasture's syndrome is histologically diagnosed by intense linear deposits of IgG along the glomerular basement membrane in both renal and lung tissues. oodpasture's syndrome is a rare disease, characterized by rapidly progressive glomerulonephritis, diffuse pulmonary hemorrhage and circulating antiglomerular basement membrane antibody (anti-GBM antibody). Central nervous system (CNS) manifestations in Goodpasture's syndrome are extremely rare, with only a few cases having been reported in the literature (8 10). Therefore, we present our imaging findings of CNS vasculitis associated with Goodpasture's syndrome, together with a review of the relevant literature. In summary, CNS vasculitis associated with Goodpasture's syndrome is extremely rare. Awareness of the imaging findings, as well as the clinical significance of CNS vasculitis associated with Goodpasture's syndrome, can be helpful in making the correct diagnosis and subsequent management of this rare condition.

Osler-Weber-Rendu syndrome complicated with pulmonary arteriovenous malformation: A case report and review of literatures

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Kuan-Yu Wang

2015-01-01

Full Text Available Osler-Weber-Rendu syndrome is a hereditary disease which is diagnosed by criterions of clinical symptoms and examinations. Here, we report a definite case of Osler-Weber-Rendu syndrome who had epistaxis, skin telangiectasia, and pulmonary arteriovenous malformation (PAVM. His initial clinical presentations were growing pulmonary nodule with cough and occasional chest pain. PAVM with rupture of aneurysm was diagnosed histologically after wedge resection of the nodule in his right lower lung.

Pulmonary dysfunction and hepatopulmonary syndrome in cirrhosis and portal hypertension

DEFF Research Database (Denmark)

Møller, Søren; Krag, Aleksander; Madsen, Jan L

2009-01-01

BACKGROUND: Pulmonary dysfunction including the hepatopulmonary syndrome (HPS) is an important complication to cirrhosis and portal hypertension. However, the precise relation to liver dysfunction and the prevalence of HPS are unclear. AIMS: We therefore aimed to assess (i) the prevalence of HPS......, Pportal hypertension (post-sinusoidal resistance, P

Arterial tortuosity syndrome: An extremely rare disease presenting as a mimic of pulmonary sling

Directory of Open Access Journals (Sweden)

Amy Farkas, MD

2018-02-01

Full Text Available Pulmonary sling is the anatomic variant defined by the aberrant origin of the left pulmonary artery from the right pulmonary artery. This patient presented with a mimic of pulmonary sling as a result of an extremely rare condition, arterial tortuosity syndrome (ATS. The patient was first diagnosed with pulmonary sling on prenatal echocardiogram performed by cardiology. Computed tomography angiography of the chest obtained at birth to evaluate respiratory depression demonstrated ATS. The early detection of ATS has been demonstrated to improve patient outcome. This case provides an overview of the typical imaging features of ATS to aid radiologists in making this uncommon diagnosis.

Overlap syndrome: obstructive sleep apnea in patients with chronic obstructive pulmonary disease.

Science.gov (United States)

Weitzenblum, Emmanuel; Chaouat, Ari; Kessler, Romain; Canuet, Matthieu

2008-02-15

Chronic obstructive pulmonary disease (COPD) and sleep apnea-hypopnea syndrome (SAHS) are both common diseases affecting respectively 10 and 5% of the adult population over 40 years of age, and their coexistence, which is denominated overlap syndrome, can be expected to occur in about 0.5% of this population. A recent epidemiologic study has shown that the prevalence of SAHS is not higher in COPD than in the general population, and that the coexistence of the two conditions is due to chance and not through a pathophysiologic linkage between these two diseases. Patients with overlap have a more important sleep-related O(2) desaturation than do patients with COPD with the same degree of bronchial obstruction. They have an increased risk of developing hypercapnic respiratory insufficiency and pulmonary hypertension when compared with patients with SAHS alone and with patients with "usual" COPD. In patients with overlap, hypoxemia, hypercapnia, and pulmonary hypertension can be observed in the presence of mild to moderate bronchial obstruction, which is different from "usual" COPD. Therapy of the overlap syndrome consists of nasal continuous positive airway pressure or nocturnal noninvasive ventilation (NIV), with or without associated nocturnal O(2). Patients who are markedly hypoxemic during daytime (Pa(O(2)) < 55-60 mm Hg) should be given conventional long-term O(2) therapy in addition to nocturnal ventilation.

Central extracorporeal membrane oxygenation requiring pulmonary arterial venting after near-drowning.

Science.gov (United States)

Kimura, Mitsutoshi; Kinoshita, Osamu; Fujimoto, Yoshifumi; Murakami, Arata; Shindo, Takahiro; Kashiwa, Koichi; Ono, Minoru

2014-02-01

Extracorporeal membrane oxygenation (ECMO) is an effective respiratory and circulatory support in patients in refractory cardiogenic shock or cardiac arrest. Peripheral ECMO sometimes requires left heart drainage; however, few reports state that pulmonary arterial (PA) venting is required during ECMO support. We present a case of a 14-year-old boy who required PA venting during ECMO support after resuscitation from near-drowning in freshwater. A biventricular assist device with an oxygenator implantation was intended on day 1; however, we were unable to proceed because of increasing of pulmonary vascular resistance from the acute lung injury. Central ECMO with PA venting was then performed. On day 13, central ECMO was converted to biventricular assist device with an oxygenator, which was removed on day 16. This case suggests that PA venting during ECMO support may be necessary in some cases of respiratory and circulatory failure with high pulmonary vascular resistance after near-drowning.

Central serous choroidopathy in the Hallermann-Streiff Syndrome.

Science.gov (United States)

Blair, N P; Brockhurst, R J; Lee, W

1981-08-01

Central serous choroidopathy was observed in a young patient with the Hallermann-Streiff syndrome. Typical features of this syndrome include microphthalmos, proportionate dwarfism, dyscephaly with birdlike facies, dental abnormalities, and hypotrichosis. Exceptional aspects of this case include age of onset (11 years), high hyperopic refractive error (+ 13.00 sphere), and multiple recurrences caused by six separate documented leaks from the choroid. Fundus changes previously reported in the Hallermann-Streiff syndrome, interpreted as chorioretinal pigmentary changes, may have been secondary to previous undiagnosed central serous choroidopathy. Periodic ophthalmoscopy should be performed and may detect unrecognized episodes of central serous choroidopathy for which photocoagulation would be beneficial.

[Pulmonary hypertension associated with congenital heart disease and Eisenmenger syndrome].

Science.gov (United States)

Calderón-Colmenero, Juan; Sandoval Zárate, Julio; Beltrán Gámez, Miguel

2015-01-01

Pulmonary arterial hypertension is a common complication of congenital heart disease (CHD). Congenital cardiopathies are the most frequent congenital malformations. The prevalence in our country remains unknown, based on birthrate, it is calculated that 12,000 to 16,000 infants in our country have some cardiac malformation. In patients with an uncorrected left-to-right shunt, increased pulmonary pressure leads to vascular remodeling and endothelial dysfunction secondary to an imbalance in vasoactive mediators which promotes vasoconstriction, inflammation, thrombosis, cell proliferation, impaired apotosis and fibrosis. The progressive rise in pulmonary vascular resistance and increased pressures in the right heart provocated reversal of the shunt may arise with the development of Eisenmenger' syndrome the most advanced form de Pulmonary arterial hypertension associated with congenital heart disease. The prevalence of Pulmonary arterial hypertension associated with CHD has fallen in developed countries in recent years that is not yet achieved in developing countries therefore diagnosed late as lack of hospital infrastructure and human resources for the care of patients with CHD. With the development of targeted medical treatments for pulmonary arterial hypertension, the concept of a combined medical and interventional/surgical approach for patients with Pulmonary arterial hypertension associated with CHD is a reality. We need to know the pathophysiological factors involved as well as a careful evaluation to determine the best therapeutic strategy. Copyright © 2014 Instituto Nacional de Cardiología Ignacio Chávez. Published by Masson Doyma México S.A. All rights reserved.

Hantavirus Pulmonary Syndrome in the United States.

Science.gov (United States)

Fabbri, Marilyn; Maslow, Melanie J.

2001-06-01

Since the first outbreak of hantavirus pulmonary syndrome (HPS) in 1993, understanding of the vast distribution and potential impact of hantaviruses has grown. At least 277 cases of HPS have been documented in the United States. The full clinical spectrum has yet to be elucidated, and one outbreak suggested the possibility of person-to-person transmission. New research has identified the b-3 integrins as cellular receptors for hantaviruses and has determined the pivotal role of the immune system in pathogenesis. Rapid diagnosis has been facilitated by a new immunoblot assay to detect Sin Nombre virus infection. Treatment remains primarily supportive; however, a placebo- controlled trial of ribavirin is ongoing. Extracorporeal membrane oxygenation may be a potential therapy in severe cases; inhaled nitric oxide needs further study. Vaccines developed against hantaviruses associated with hemorrhagic fever and renal syndrome might be effective against HPS-associated strains.

Significance of lung anomalies in fetuses affected by tetralogy of Fallot with absent pulmonary valve syndrome.

Science.gov (United States)

Tenisch, Estelle; Raboisson, Marie-Josée; Rypens, Françoise; Déry, Julie; Grignon, Andrée; Lapierre, Chantale

2017-11-01

Tetralogy of Fallot with absent pulmonary valve syndrome is a rare form of tetralogy of Fallot with dilatation of large pulmonary arteries. Prognosis is related to the severity of the cardiac malformation and to bronchial tree compression by dilated pulmonary arteries. This study analyses the prenatal echographic lung appearance in fetuses with tetralogy of Fallot with absent pulmonary valve and discusses its significance. We carried out a retrospective review of fetal and postnatal files of nine fetuses diagnosed with tetralogy of Fallot with absent pulmonary valve syndrome in our institution. Correlations of prenatal ultrasound and cardiac imaging findings were obtained with outcome. Abnormal heterogeneous fetal lung echogenicity was detected in eight cases out of nine, always associated with significant lobar arterial dilatation. This aspect was well correlated with postnatal imaging and outcome in the four neonatal cases. The only fetus with normal lung echogenicity also had lower degree of pulmonary artery dilatation in the series. This study demonstrates that a heterogeneous ultrasound appearance of the fetal lungs can be detected in utero in the most severe cases. This aspect suggests an already significant compression of the fetal bronchial tree by the dilated arteries that may have prognostic implications.

Clinical and radiological characteristics of central pulmonary adenocarcinoma: a comparison with central squamous cell carcinoma and small cell lung cancer and the impact on treatment response

Directory of Open Access Journals (Sweden)

Wang Z

2018-05-01

Full Text Available Zhe Wang,1,2 Minghuan Li,2 Yong Huang,3 Li Ma,3 Hui Zhu,2 Li Kong,2 Jinming Yu2 1School of Medicine, Shandong University, Jinan, Shandong, China; 2Department of Radiation Oncology, Shandong Cancer Hospital Affiliated to Shandong University, Jinan, Shandong, China; 3Department of Radiology, Shandong Cancer Hospital Affiliated to Shandong University, Jinan, Shandong, China Purpose: The proportion of central pulmonary adenocarcinoma (ADC in central-type lung cancer has been gradually increasing due to the overall increasing incidence of pulmonary ADC. But the clinical and radiological characteristics of central ADCs remain unclear. In this study, we compared the clinical and radiological characteristics of central ADCs with those of small cell lung cancers (SCLCs and squamous cell carcinomas (SQCCs and investigated the impact of these characteristics on patients’ treatment response. Patients and methods: The medical records of 302 consecutive patients with central lung cancer from July 2014 to September 2016 were retrospectively reviewed. There were 99 patients with ADC, 95 with SQCC and 108 with SCLC. Computed tomography images were interpreted by two radiologists. Treatment response was determined by Response Evaluation Criteria In Solid Tumors 1.1. Results: Univariate analyses found that younger age, female sex, no history of smoking, higher levels of carcinoembryonic antigen (CEA, contralateral hilum lymphadenopathy, contralateral lung metastasis, pleural nodules and pleural metastasis to the interlobular fissure were significantly correlated with central ADC. Multivariate logistic regression analyses revealed that compared with central SQCC, female sex, younger age, no history of smoking, higher levels of CEA and contralateral hilum lymphadenopathy were the significantly independent indicators of central pulmonary ADC. Furthermore, compared with central SCLC, younger age, higher levels of CEA and cytokeratin 19 fragment (Cyfra21-1, lower

Respiratory Failure Associated with the Lipodystrophy Syndrome in an HIV-Positive Patient with Compromised Lung Function

Directory of Open Access Journals (Sweden)

Natasha Press

2001-01-01

Full Text Available Protease inhibitors, used as treatment in human immunodeficiency virus (HIV infection, are associated with a syndrome of peripheral lipodystrophy, central adiposity, hyperlipidemia and insulin resistance. An HIV-positive patient with chronic obstructive pulmonary disease is presented who developed the lipodystrophy syndrome that is associated with the use of protease inhibitors. It is postulated that the lipodystrophy syndrome further compromised his lung function, leading to respiratory failure. Patients who have pulmonary disease and are taking protease inhibitors require monitoring of clinical status and pulmonary function tests.

A patient who sufferred pulmonary tuberculosis with syndrome of inappropriate secretion of antidiuretic hormone, after radiotherapy for pulmonary adenocarcinoma

International Nuclear Information System (INIS)

Kobayashi, Kashin; Horiguchi, Takahiko; Hata, Hideharu; Sasaki, Yasushi; Hirose, Masahiro; Shiga, Mamoru; Kondo, Rieko; Tachikawa, Souichi

2007-01-01

A 62-year-old man presented in June 2006 with right thoracic pain, cough, and weight loss, which had persisted for 3 months. Chest X-ray showed a mass-like shadow in the right pulmonary apex, suggesting a stage IIIb adenocarcinoma which was confirmed by biopsy. We gave a total radiation dose of 60 Gy, after this which general malaise and weakness were noted. The results of endocrinological examinations suggested syndrome of inappropriate antidiuretic hormone secretion (SIADH). Thoracic CT showed ground-glass opacity (GGO) in both lungs, and we could not rule out pulmonary tuberculosis. A sputum was positive for acid-fast stain and polymerase polymerase chain reaction-Tuberculosa (PCR-Tb), suggesting that SIADH was associated with pulmonary tuberculosis. Water restriction, i.v. physiological saline, and anti-tuberculosis therapy relieved hyponatremia and the symptoms. (author)

Drug-Coated Balloon Angioplasty: A Novel Treatment for Pulmonary Artery In-Stent Stenosis in a Patient with Williams Syndrome.

Science.gov (United States)

Cohen, Jennifer L; Glickstein, Julie S; Crystal, Matthew A

2017-12-01

A 20-month-old boy with Williams syndrome had undergone multiple surgical and catheter-based interventions for resistant peripheral pulmonary arterial stenoses with eventual bilateral stent placement and conventional balloon angioplasty. He persistently developed suprasystemic right ventricular (RV) pressure. Angioplasty with a drug-coated balloon (DCB) was performed for in-stent restenosis and to remodel his distal pulmonary vessels bilaterally. This resulted in immediate improvement in the in-stent stenosis and resultant decrease in RV pressure. Follow-up catheterization two months later continued to show long-lasting improvement in the in-stent stenosis. We hypothesize that the anti-proliferative effects of DCBs may be of benefit in the arteriopathy associated with Williams syndrome. We report this as a novel use of a DCB in the pulmonary arterial circulation in a patient with Williams syndrome.

Effects on Pulmonary Vascular Mechanics of Two Different Lung-Protective Ventilation Strategies in an Experimental Model of Acute Respiratory Distress Syndrome.

Science.gov (United States)

Santos, Arnoldo; Gomez-Peñalver, Eva; Monge-Garcia, M Ignacio; Retamal, Jaime; Borges, João Batista; Tusman, Gerardo; Hedenstierna, Goran; Larsson, Anders; Suarez-Sipmann, Fernando

2017-11-01

To compare the effects of two lung-protective ventilation strategies on pulmonary vascular mechanics in early acute respiratory distress syndrome. Experimental study. University animal research laboratory. Twelve pigs (30.8 ± 2.5 kg). Acute respiratory distress syndrome was induced by repeated lung lavages and injurious mechanical ventilation. Thereafter, animals were randomized to 4 hours ventilation according to the Acute Respiratory Distress Syndrome Network protocol or to an open lung approach strategy. Pressure and flow sensors placed at the pulmonary artery trunk allowed continuous assessment of pulmonary artery resistance, effective elastance, compliance, and reflected pressure waves. Respiratory mechanics and gas exchange data were collected. Acute respiratory distress syndrome led to pulmonary vascular mechanics deterioration. Four hours after randomization, pulmonary vascular mechanics was similar in Acute Respiratory Distress Syndrome Network and open lung approach: resistance (578 ± 252 vs 626 ± 153 dyn.s/cm; p = 0.714), effective elastance, (0.63 ± 0.22 vs 0.58 ± 0.17 mm Hg/mL; p = 0.710), compliance (1.19 ± 0.8 vs 1.50 ± 0.27 mL/mm Hg; p = 0.437), and reflection index (0.36 ± 0.04 vs 0.34 ± 0.09; p = 0.680). Open lung approach as compared to Acute Respiratory Distress Syndrome Network was associated with improved dynamic respiratory compliance (17.3 ± 2.6 vs 10.5 ± 1.3 mL/cm H2O; p mechanics similarly. The use of higher positive end-expiratory pressures in the open lung approach strategy did not worsen pulmonary vascular mechanics, improved lung mechanics, and gas exchange but at the expense of a lower cardiac index.

Case report: Noonan-like multiple central giant cell granuloma syndrome.

Science.gov (United States)

Bitton, Natalie; Alexander, Stanley; Ruggiero, Salvatore; Parameswaran, Ashish; Russo, Antonino; Ferguson, Fred

2012-01-01

The purpose of this report was to: summarize the care of a child between the ages of 12 to 16 years old born with Noonan-like central giant cell syndrome and unrelated common variable immune deficiency; provide information on the dental management of patients with Noonan's syndrome; and present a brief discussion of the recent associated genetic findings. A review of the common features of Noonan syndrome and Noonan-like central giant cell syndrome is also provided.

Characteristics of pulmonary cysts in Birt-Hogg-Dube syndrome: Thin-section CT findings of the chest in 12 patients

Energy Technology Data Exchange (ETDEWEB)

Tobino, Kazunori, E-mail: tobino@juntendo.ac.jp [Department of Respiratory Medicine, Juntendo University, School of Medicine, 2-1-1 Hongo, Bunkyo-Ku, Tokyo 113-8421 (Japan); Gunji, Yoko [Department of Respiratory Medicine, Juntendo University, School of Medicine, 2-1-1 Hongo, Bunkyo-Ku, Tokyo 113-8421 (Japan); Kurihara, Masatoshi [Pneumothorax Center, Nissan Tamagawa Hospital, 4-8-1 Seta, Setagaya-Ku, Tokyo 158-0095 (Japan); The Study Group of Pneumothorax and Cystic Lung Diseases, 4-8-1 Seta, Setagaya-Ku, Tokyo 158-0095 (Japan); Kunogi, Makiko; Koike, Kengo [Department of Respiratory Medicine, Juntendo University, School of Medicine, 2-1-1 Hongo, Bunkyo-Ku, Tokyo 113-8421 (Japan); Tomiyama, Noriyuki [Department of Radiology, Osaka University Graduate School of Medicine, 2-2 Yamadaoka, Suita, Osaka 565-0871 (Japan); Johkoh, Takeshi [Department of Radiology, Kinki Central Hospital of Mutual Aid Association of Public School Teachers, Kurumazuka 3-1, Itami, Hyogo 664-0872 (Japan); Kodama, Yuzo [Department of Respiratory Medicine, Juntendo University, School of Medicine, 2-1-1 Hongo, Bunkyo-Ku, Tokyo 113-8421 (Japan); Iwakami, Shin-ichiro [Department of Respiratory Medicine, Juntendo University, Shizuoka Hospital, 1129 Nagaoka, Izunokuni, Shizuoka 410-2295 (Japan); Kikkawa, Mika [Biochemical Research Center, Division of Proteomics and Biomolecular Sciences, Juntendo University, Graduate School of Medicine, Bunkyo-Ku, Tokyo (Japan); Takahashi, Kazuhisa [Department of Respiratory Medicine, Juntendo University, School of Medicine, 2-1-1 Hongo, Bunkyo-Ku, Tokyo 113-8421 (Japan); Seyama, Kuniaki [Department of Respiratory Medicine, Juntendo University, School of Medicine, 2-1-1 Hongo, Bunkyo-Ku, Tokyo 113-8421 (Japan); Study Group of Pneumothorax and Cystic Lung Diseases, 4-8-1 Seta, Setagaya-Ku, Tokyo 158-0095 (Japan)

2011-03-15

Purpose: To describe in detail the characteristic chest computed tomography (CT) findings of Birt-Hogg-Dube (BHD) syndrome. Materials and methods: Thin-section chest CT scans of consecutive 12 patients with genetically diagnosed BHD syndrome were retrospectively evaluated by two observers, especially about the characteristics (distribution, number, size, shape and relation to pleura) of pulmonary cysts. Interobserver agreement in the identification of abnormalities on the CT images was achieved using the {kappa} statistic, and the degree of interobserver correlation for the characterization of pulmonary cysts was assessed using the Spearman rank correlation coefficient. Results: Multiple pulmonary cysts were seen in all patients. The number of cysts in each patient was various (range, 29-407), and cysts of various sizes (from a few mm to 2 cm or more) were seen in all patient. 76.6% (mean) of cysts were irregular-shaped, and 40.5% (mean) of cysts were located along the pleura. The mean extent score of cysts was 13% of the whole lung, and the distribution of cysts was predominantly in the lower medial zone. Finally, cysts abutting or including the proximal portions of lower pulmonary arteries or veins were also seen in all patients. Conclusion: Multiple, irregular-shaped cysts of various sizes with lower medial lung zone predominance are characteristic CT findings of BHD syndrome. Cysts abutting or including the proximal portions of lower pulmonary arteries or veins may also exist in this syndrome in a high probability.

Characteristics of pulmonary cysts in Birt-Hogg-Dube syndrome: Thin-section CT findings of the chest in 12 patients

International Nuclear Information System (INIS)

Tobino, Kazunori; Gunji, Yoko; Kurihara, Masatoshi; Kunogi, Makiko; Koike, Kengo; Tomiyama, Noriyuki; Johkoh, Takeshi; Kodama, Yuzo; Iwakami, Shin-ichiro; Kikkawa, Mika; Takahashi, Kazuhisa; Seyama, Kuniaki

2011-01-01

Purpose: To describe in detail the characteristic chest computed tomography (CT) findings of Birt-Hogg-Dube (BHD) syndrome. Materials and methods: Thin-section chest CT scans of consecutive 12 patients with genetically diagnosed BHD syndrome were retrospectively evaluated by two observers, especially about the characteristics (distribution, number, size, shape and relation to pleura) of pulmonary cysts. Interobserver agreement in the identification of abnormalities on the CT images was achieved using the κ statistic, and the degree of interobserver correlation for the characterization of pulmonary cysts was assessed using the Spearman rank correlation coefficient. Results: Multiple pulmonary cysts were seen in all patients. The number of cysts in each patient was various (range, 29-407), and cysts of various sizes (from a few mm to 2 cm or more) were seen in all patient. 76.6% (mean) of cysts were irregular-shaped, and 40.5% (mean) of cysts were located along the pleura. The mean extent score of cysts was 13% of the whole lung, and the distribution of cysts was predominantly in the lower medial zone. Finally, cysts abutting or including the proximal portions of lower pulmonary arteries or veins were also seen in all patients. Conclusion: Multiple, irregular-shaped cysts of various sizes with lower medial lung zone predominance are characteristic CT findings of BHD syndrome. Cysts abutting or including the proximal portions of lower pulmonary arteries or veins may also exist in this syndrome in a high probability.

Hennekam syndrome presenting as nonimmune hydrops fetalis, congenital chylothorax, and congenital pulmonary lymphangiectasia

NARCIS (Netherlands)

Bellini, Carlo; Mazzella, Massimo; Arioni, Cesare; Campisi, Corradino; Taddei, Gioconda; Tomà, Paolo; Boccardo, Francesco; Hennekam, Raoul C.; Serra, Giovanni

2003-01-01

We report a female infant with congenital lymphedema, facial anomalies, intestinal lymphangiectasia consistent with a diagnosis of Hennekam syndrome. At birth the patient presented with severe respiratory distress due to nonimmune hydrops fetalis, a congenital chylothorax (CC), and pulmonary

Primary Sjogrens syndrome, with lymphocytic interticial pneumonia and cystic pulmonary disease

International Nuclear Information System (INIS)

Vallejo, Jimi Mejia; Bayona, Jorge Carrillo; Iglesias Gamarra, Antonio; Rondon Federico Sanchez Contreras Alvaro, Restrepo Jose Felix

2007-01-01

The primary Sjogrens syndrome (PSS) is a chronic autoimmune exocrinopathy characterized by lymphocytic infiltration of glandular and extraglanduIar tissue, the lung involvement is heterogeneous; the aim of this study is report the first case in Colombia of PSS with Iymphocytic intersticiaI pneumonia and cystic pulmonary disease discuss differential diagnosis and the initial diagnostic and therapeutic work up

Sjogrens Syndrome Presenting with Central Nervous System Involvement

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Tülay Terzi

2012-01-01

Full Text Available Sjogren’s syndrome is a slowly progressive autoimmune disease. Neurological involvement occurs in approximately 20-25% cases in Sjogren’s syndrome. 87% of the neurological involvement is peripheral nervous system, almost 13% in the form of central nervous system involvement. Affected central nervous system may show similar clinical and radiological findings as in multiple sclerosis (MS. In this paper, a 43-year-old patient is discussed who was referred with the complaint of dizziness, there was MS- like lesions in brain imaging studies and was diagnosed with Sjogren’s syndrome. MS- like clinical and radiologic tables can be seen, albeit rarely in Sjogren’s syndrome. In these cases, early diagnosis and early treatment for the sjögren has a great importance for the prognosis of the disease.

Pulmonary Involvement in Patients with Guillain-Barré Syndrome in Subacute Phase.

Science.gov (United States)

Khanna, Meeka; Rawat, Nidhi; Gupta, Anupam; Nagappa, Madhu; Taly, Arun B; Rukmani, M R; Sathyaprabha, T N; Haldar, Partha

2017-01-01

To evaluate the pulmonary function in Guillain-Barre syndrome (GBS) patients in subacute phase and find clinical correlates of pulmonary dysfunction. This was a single-center, prospective, cross-sectional, hospital-based study in GBS patients performed in Department of Neurological Rehabilitation at a tertiary care institute. Clinical examination for pulmonary function was done by measuring chest expansion. The pulmonary function tests were carried out by Spirometry kit Microquark Cosmed, Italy. Fatigue was assessed by Fatigue Severity Scale, disability status by Hughes Disability Scale (HDS), and muscle weakness by Medical Research Council sum scores. Statistical analysis was performed by Stata 11. The significance of P value was adjudged against an alpha of 0.05. Twenty-eight patients were included with 17 (61%) men and mean age of 31 years. Median duration of symptoms was 16.5 days. There were 10 (36%) demyelinating and 18 (64%) axonal variants. Twenty-six (93%) patients scored more than 2 on HDS. All study participants reported fatigue. Twenty-two (78.6%) patients had chest expansion of <2.5 cm. Spirometry showed restrictive pulmonary dysfunction in 23 (79%) patients. Significant correlation was found between abnormal pulmonary function test and chest expansion ( P = 0.003). Pulmonary dysfunction in GBS is common even during subacute phase. It needs to be identified and managed appropriately for better clinical outcome.

Pulmonary involvement in patients with Guillain–Barré syndrome in subacute phase

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Meeka Khanna

2017-01-01

Full Text Available Objectives: To evaluate the pulmonary function in Guillain–Barre syndrome (GBS patients in subacute phase and find clinical correlates of pulmonary dysfunction. Methods: This was a single-center, prospective, cross-sectional, hospital-based study in GBS patients performed in Department of Neurological Rehabilitation at a tertiary care institute. Clinical examination for pulmonary function was done by measuring chest expansion. The pulmonary function tests were carried out by Spirometry kit Microquark Cosmed, Italy. Fatigue was assessed by Fatigue Severity Scale, disability status by Hughes Disability Scale (HDS, and muscle weakness by Medical Research Council sum scores. Statistical Analysis: Statistical analysis was performed by Stata 11. The significance of P value was adjudged against an alpha of 0.05. Results: Twenty-eight patients were included with 17 (61% men and mean age of 31 years. Median duration of symptoms was 16.5 days. There were 10 (36% demyelinating and 18 (64% axonal variants. Twenty-six (93% patients scored more than 2 on HDS. All study participants reported fatigue. Twenty-two (78.6% patients had chest expansion of <2.5 cm. Spirometry showed restrictive pulmonary dysfunction in 23 (79% patients. Significant correlation was found between abnormal pulmonary function test and chest expansion (P = 0.003. Conclusion: Pulmonary dysfunction in GBS is common even during subacute phase. It needs to be identified and managed appropriately for better clinical outcome.

Pulmonary Involvement in Patients with Guillain–Barré Syndrome in Subacute Phase

Science.gov (United States)

Khanna, Meeka; Rawat, Nidhi; Gupta, Anupam; Nagappa, Madhu; Taly, Arun B.; Rukmani, M. R.; Sathyaprabha, T. N.; Haldar, Partha

2017-01-01

Objectives: To evaluate the pulmonary function in Guillain–Barre syndrome (GBS) patients in subacute phase and find clinical correlates of pulmonary dysfunction. Methods: This was a single-center, prospective, cross-sectional, hospital-based study in GBS patients performed in Department of Neurological Rehabilitation at a tertiary care institute. Clinical examination for pulmonary function was done by measuring chest expansion. The pulmonary function tests were carried out by Spirometry kit Microquark Cosmed, Italy. Fatigue was assessed by Fatigue Severity Scale, disability status by Hughes Disability Scale (HDS), and muscle weakness by Medical Research Council sum scores. Statistical Analysis: Statistical analysis was performed by Stata 11. The significance of P value was adjudged against an alpha of 0.05. Results: Twenty-eight patients were included with 17 (61%) men and mean age of 31 years. Median duration of symptoms was 16.5 days. There were 10 (36%) demyelinating and 18 (64%) axonal variants. Twenty-six (93%) patients scored more than 2 on HDS. All study participants reported fatigue. Twenty-two (78.6%) patients had chest expansion of <2.5 cm. Spirometry showed restrictive pulmonary dysfunction in 23 (79%) patients. Significant correlation was found between abnormal pulmonary function test and chest expansion (P = 0.003). Conclusion: Pulmonary dysfunction in GBS is common even during subacute phase. It needs to be identified and managed appropriately for better clinical outcome. PMID:28694622

Multifocal central serous chorioretinopathy with photoreceptor-retinal pigment epithelium diastasis in heritable pulmonary arterial hypertension

DEFF Research Database (Denmark)

Li, Xiao Qiang; Pryds, Anders; Carlsen, Jørn

2015-01-01

PURPOSE: To report atypical central serous chorioretinopathy and choroidal thickening in a patient with heritable pulmonary arterial hypertension. METHODS: A 40-year-old man with heritable pulmonary arterial hypertension presented with blurred vision in his left eye and was followed up for 1 year...

Osteoporosis associated with pulmonary silicosis in an equine bone fragility syndrome.

Science.gov (United States)

Arens, A M; Barr, B; Puchalski, S M; Poppenga, R; Kulin, R M; Anderson, J; Stover, S M

2011-05-01

California horses incur a bone fragility syndrome manifested by pathologic fractures. This study investigated gross, radiographic, and histologic features of the disorder as well as relationships with silicosis and levels of heavy metals and trace minerals through a postmortem study of 9 affected and 3 unaffected horses. Bones and soft tissues were evaluated grossly and histologically. Bones, lymph nodes, and lung tissue were evaluated radiographically. Tissues were evaluated for silicon levels, intracytoplasmic crystals, heavy metals, and trace minerals. All 9 affected horses had osteoporosis and clinical or subclinical pulmonary disease due to silicosis (8/9) or pneumoconiosis (1/9). All affected horses had radiographic findings consistent with osteopenia and histologic evidence of osteoporosis characterized by osteopenia, numerous resorption cavities, cement lines, and a mosaic lamellar pattern indicative of multiple remodeling events. Silicosis was characterized by widespread pulmonary granuloma formation with fibrosis; variable tracheobronchiolar and mediastinal granulomatous lymphadenitis; intracellular crystals within lung and lymph node macrophages; and pronounced lymph node fibrosis, focal necrosis, and dystrophic calcification. Crystals in lung (6/9) and lymph node (8/9) tissues were identified as cytotoxic silica dioxide polymorphs. Lung and liver tissue from affected horses had elevated levels of elemental silicon. Osteoporosis was highly correlated (r = 0.8, P horses with bone fragility disorder have systemic osteoporosis associated with fibrosing pulmonary silicosis. The etiopathogenesis of the bone fragility syndrome is unknown; however, this study provides circumstantial evidence for a silicate associated osteoporosis.

The Evaluation of Pulmonary Function Tests in Patients with Polycystic Ovary Syndrome

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Mahdis Maraashi

2013-10-01

Full Text Available Introduction: Recently a relation between female sex hormones and severity of asthma symptoms has been proposed. As a common endocrine dysfunction, polycystic ovary syndrome (PCOS could significantly influence the level of sex hormones in PCOS patients. Regarding the possible role of sex hormones in airway physiology, the present study was conducted to survey the effects of PCOS on pulmonary function test parameters. Materials and Methods: In this cross-sectional study 30 recently diagnosed patients with PCOS without history of pulmonary disease were enrolled and 20 healthy women were considered as the control group according to their age, weight, and height. The patients and the controls underwent body plethysmography to measure pulmonary function tests. Results: The mean age of the patients and the controls were 29.43±7.8 and 30.0±7.6 years respectively. There were no statistically significant differences in all pulmonary function test parameters between the patients and the controls (p>0.05. After dividing the patients into 2 groups based on their body mass index (BMI, BMIConclusion: Our results showed that pulmonary function test parameters are not different in PCOS patients comparing to healthy women. Only the deleterious effects of high BMI on pulmonary function can be occurred in these patients.

Neonatal pulmonary arterial hypertension and Noonan syndrome: two fatal cases with a specific RAF1 mutation.

Science.gov (United States)

Hopper, Rachel K; Feinstein, Jeffrey A; Manning, Melanie A; Benitz, William; Hudgins, Louanne

2015-04-01

Mutations in RAF1 are associated with Noonan syndrome and hypertrophic cardiomyopathy. We present two infants with Noonan syndrome and an identical RAF1 mutation, p.Ser257Leu (c.770C>T), who developed severe pulmonary arterial hypertension (PAH) that proved to be fatal. The RAF1 gene encodes Raf-1 kinase, part of the Ras/mitogen-activated kinase (MAPK) signaling pathway, which has been linked to the development of PAH. This specific mutation has been associated with dephosphorylation of a critical serine residue and constitutive activation of the Raf-1 kinase. These two cases suggest that abnormal activation of the Ras/MAPK pathway may play a significant role in the development of pulmonary vascular disease in the subset of patients with Noonan syndrome and a specific RAF1 mutation. © 2015 Wiley Periodicals, Inc.

Goldenhar Syndrome with Dextrocardia and Right Pulmonary Hypoplasia: An Unusual Association

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Nagendra Chaudhary

2017-01-01

Full Text Available Goldenhar syndrome (GS, a rare condition, occurring due to defect in development of first and second branchial arches, is characterized by a combination of various anomalies involving face, eyes, ears, vertebrae, heart, and lungs. The etiology of GS is not fully known, although various hypotheses have been proposed along with its genetic association and many other causes. Facial asymmetry and hypoplasia of the mandible are characteristic features of GS along with microtia and preauricular appendages and pits. Dextrocardia or pulmonary hypoplasia in GS has previously been reported separately. We report a 7-year-old female child of GS with combination of anomalies, dextrocardia, and pulmonary hypoplasia, which is a rare association.

Imaging findings of disseminated pulmonary tuberculosis in patients with acquired immunodeficiency syndrome

International Nuclear Information System (INIS)

Song Wenyan; Zhao Zuqi; Zhao Dawei; Jia Cuiyu; Zhang Ruichi; Liu JinXin; Guan Wanhua; Liang Yi

2013-01-01

Objective: To study the imaging findings of disseminated pulmonary tuberculosis in patients with acquired immunodeficiency syndrome (AIDS). Methods: X-ray and multi-slice CT (MSCT) data from 33 AIDS patients with disseminated pulmonary tuberculosis confirmed by clinical manifestations and laboratory tests were analyzed retrospectively. Results: Thirty patients underwent initial chest radiography examination, 29 patients showed abnormal appearances, including bilateral disseminations in 21 patients and unilateral multiple disseminations in 8 patients. All patients underwent MSCT examination, 26 patients showed bilateral disseminations and 7 patients showed unilateral multiple disseminations. The abnormal pulmonary appearances included nodule (n = 25), miliary nodule (n = 22), air-space consolidation (n = 22), cavity (n = 11), fibrosis (n = 7), ground-glass opacity (n = 7), pneumatocele (n = 4), calcification (n = 2). There were 20 patients with more than 3 abnormal appearances and 13 patients with one or two abnormal appearances. The extra-pulmonary tuberculosis included pleural effusion (n = 33), lymphadenopathy (n = 30), intestinal tuberculosis (n = 3), splenic tuberculosis (n = 1) and cerebral tuberculosis (n = 1). Conclusion: Disseminated pulmonary tuberculosis should be highly suspected in AIDS patients with diffused nodules, miliary nodules, air-space consolidations or multiple cavities, accompanied with pleural effusion and lymphadenopathy. (authors)

Pulmonary Hypertension Overview

Science.gov (United States)

... well as sleep apnea, are common causes of secondary pulmonary hypertension. Other causes include the following: Congestive heart failure Birth defects in the heart Chronic pulmonary thromboembolism (blood clots in the pulmonary arteries) Acquired immunodeficiency syndrome ( ...

Cutis laxa and fatal pulmonary hypertension: a newly recognized syndrome?

Science.gov (United States)

Brunetti-Pierri, Nicola; Piccolo, Pasquale; Morava, Eva; Wevers, Ron A.; McGuirk, Megan; Johnson, Yvette R.; Urban, Zsolt; Dishop, Megan K.; Potocki, Lorraine

2015-01-01

Cutis laxa is a connective tissue disorder with distinctive lax, redundant, and inelastic skin. It is a genetically heterogenous disorder with autosomal dominant and recessive patterns of inheritance. We report a patient with cutis laxa supported by clinical, microscopic, and ultrastructural findings. Molecular analysis of fibulin-4 and -5, of the α2 subunit of the V-type H+ ATPase, and of the component of the oligomeric Golgi complex 7 (COG7) genes excluded the type I and type II autosomal recessive forms of cutis laxa, and congenital disorders of glycosylation associated with cutis laxa. Remarkably, our patient also presented severe and lethal pulmonary hypertension as a newborn. This case with cutis laxa, severe pulmonary hypertension, and no detectable mutations in fibulin-4 and -5 genes may represent a previously unrecognized syndrome. PMID:21285876

[Hantavirus pulmonary syndrome (Rio Mamore virus) in the Peruvian Amazon region].

Science.gov (United States)

Casapía, Martín; Mamani, Enrique; García, María P; Miraval, María L; Valencia, Pedro; Quino, Alberto H; Alvarez, Carlos; Donaires, Luis F

2012-01-01

Hantavirus infection is a viral zoonotic infection borne by rodents which most letal form clinical is the Hantavirus Pulmonary Syndrome (SPH, Spanish abbreviation). The Mamore River variant originates in South America and was found in rodents without any association to human diseases. Two cases of SPH were identified in the Peruvian Amazon region in November 2011. In both cases, a molecular diagnostic testing was conducted by the Instituto Nacional de Salud from Peru. A phylogenetic analysis of a viral genome fragment and a histopathological evaluation were conducted. Both patients developed adult respiratory distress syndrome and refractory shock. A patient died and another one recovered 12 days later.

Primary Pulmonary Ewing’s Sarcoma: Rare Cause of Superior Vena Cava Syndrome in Children

OpenAIRE

Mehra, Shibani; Atwal, Swapndeep Singh; Garga, Umesh Chandra

2014-01-01

Ewing’s sarcoma is a common malignant bone tumour presenting in children and young adults. Rarely extra- skeletal soft tissues and visceral organs can also be the site of origin of Ewing’s sarcoma. Primary pulmonary Ewing’s sarcoma is an extremely rare malignancy which occurs in the paediatric population. We report an unusual case of primary pulmonary Ewing’s sarcoma in a nine year old girl who presented with features of superior vena cava syndrome in the emergency department. The diagnosis w...

Pulmonary hypertension and vasculopathy in incontinentia pigmenti: a case report

Directory of Open Access Journals (Sweden)

Alshenqiti A

2017-05-01

Full Text Available Abduljabbar Alshenqiti,1 Marwan Nashabat,1 Hissah AlGhoraibi,1 Omar Tamimi,2 Majid Alfadhel1 1Division of Genetics, Department of Pediatrics, King Abdullah International Medical Research Centre, King Saud bin Abdulaziz Uiversity for Health Sciences, King Abdulaziz Medical City, Ministry of National Guard Health Affairs (NGHA, Riyadh, Saudi Arabia; 2Department of Cardiology, King Abdullah International Medical Research Centre, King Saud bin Abdulaziz University for Health Sciences, King Abdulaziz Medical City, Ministry of National Guard-Health Affairs (NGHA, Riyadh, Saudi Arabia Abstract: Incontinentia pigmenti (IP; Bloch–Sulzberger syndrome is a rare, genetic syndrome inherited as an X-linked dominant trait. It primarily affects female infants and is lethal in the majority of males during fetal life. The clinical findings include skin lesions, developmental defects, and defects of the eyes, teeth, skeletal system, and central nervous system. Cardiovascular complications of this disease in general, and pulmonary hypertension in particular, are extremely rare. This report describes the case of a 3-year-old girl with IP complicated by pulmonary arterial hypertension. Extensive cardiology workup done to the patient indicates underlying vasculopathy. This report sheds light on the relationship between IP and pulmonary hypertension, reviews the previously reported cases, and compares them with the reported case. Keywords: incontinentia pigmenti, IKBKG, pulmonary hypertension, vasculopathy, Bloch–Sulzberger syndrome, lines of Blaschko, hyperpigmentation

Pulmonary Vasculitis and a Horseshoe Kidney in Noonan Syndrome

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Surasak Puvabanditsin

2018-01-01

Full Text Available We report a term male neonate with congenital myeloproliferative disorder, thrombocytopenia, a horseshoe kidney, feeding difficulty secondary to dysphagia/foregut dysmotility, and respiratory failure. Prenatal molecular genetic analysis revealed a fetus carrying c.184T>G (p.Tyr62Asp pathogenic variant in PTPN11. The infant eventually succumbed to respiratory failure. Bacterial and viral cultures/studies were all no growth/negative. Pulmonary capillaritis and vasculitis were noted at autopsy. This report presents a new case of Noonan syndrome with unusual associated disorders and a review of the literature.

Pulmonary Vasculitis and a Horseshoe Kidney in Noonan Syndrome.

Science.gov (United States)

Puvabanditsin, Surasak; Abellar, Rosanna; Madubuko, Adaora; Mehta, Rajeev; Walzer, Lauren

2018-01-01

We report a term male neonate with congenital myeloproliferative disorder, thrombocytopenia, a horseshoe kidney, feeding difficulty secondary to dysphagia/foregut dysmotility, and respiratory failure. Prenatal molecular genetic analysis revealed a fetus carrying c.184T>G (p.Tyr62Asp) pathogenic variant in PTPN11 . The infant eventually succumbed to respiratory failure. Bacterial and viral cultures/studies were all no growth/negative. Pulmonary capillaritis and vasculitis were noted at autopsy. This report presents a new case of Noonan syndrome with unusual associated disorders and a review of the literature.

Beware of the devastating pulmonary aspergillosis syndromes In certain environments

LENUS (Irish Health Repository)

Kooblall, M

2016-02-01

Nowadays with more stem cell transplants and immunosuppressive therapies there has been a rise in pulmonary aspergillosis syndromes. The following illustrates such a case. A 49 year old man had a past history of ankylosing spondylitis with a bilateral hip replacement. He was also on surveillance for a superficial bladder tumour since 2007. His chest x-ray in 2008 was normal. In 2010 his CXR showed patchy opacification in the right apex. CT thorax confirm fibrotic changes.

Simultaneous transcatheter closure of intralobar pulmonary sequestration and patent ductus arteriosus in a patient with infantile Scimitar syndrome.

Science.gov (United States)

Aslan, Eyüp; Tanıdır, İbrahim Cansaran; Saygı, Murat; Onan, Sertaç Hanedan; Güzeltaş, Alper

2015-03-01

Scimitar syndrome is a rare disease associated with a right lung sequestration vascularised by arteries arising from the abdominal aorta and abnormal venous drainage into the inferior vena cava. The infantile form is generally presented with severe heart failure, pulmonary hypertension and respiratory distress. It may be associated with various intracardiac defects, including atrial septal defects, ventricular septal defects, patent ductus arteriosus or more complicated structural congenital heart defects. Here, we present a 2-month-old girl with Scimitar syndrome whose pulmonary arterial pressure decreased after transcatheter patent ductus arteriosus closure and embolization of the anomalous systemic arterial supply.

Pulmonary extraction of serotonin and propranolol in patients with adult respiratory distress syndrome

International Nuclear Information System (INIS)

Morel, D.R.; Dargent, F.; Bachmann, M.; Suter, P.M.; Junod, A.F.

1985-01-01

Because injury to the pulmonary vascular endothelium is associated with the development of the adult respiratory distress syndrome (ARDS), the authors assessed the metabolic function of pulmonary endothelial cells by the measurements of the first-pass pulmonary extraction of [ 14 C]serotonin and [ 3 H]propranolol in 15 patients with ARDS and 15 patients at risk for developing ARDS. Serotonin extraction ratio was lower in patients with ARDS (0.85 +/- 0.10, mean +/- SD) than in patients at risk (0.91 +/- 0.04) (p less than 0.025), and both values were significantly reduced (p less than 0.005) when compared with a control group value (0.97 +/- 0.01). The decrease in serotonin extraction was correlated with the severity of ARDS (r = -0.67) (p less than 0.001) and with pulmonary function changes over time. Propranolol extraction ratio was decreased in patients at risk (0.66 +/- 0.11) (p less than 0.005) but not in patients with ARDS (0.75 +/- 0.11), when compared with those in the control group (0.81 +/- 0.03). Low values in patients at risk were restored to normal by continuous positive airway pressure breathing. The authors conclude that pulmonary extraction of serotonin, an index of pulmonary endothelial cell function, correlates with the severity of ARDS

Bilateral meandering pulmonary veins

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Thupili, Chakradhar R.; Udayasankar, Unni [Pediatric Imaging, Imaging Institute Cleveland Clinic, Cleveland, OH (United States); Renapurkar, Rahul [Imaging Institute Cleveland Clinic, Thoracic Imaging, L10, Cleveland, OH (United States)

2015-06-15

Meandering pulmonary veins is a rare clinical entity that can be mistaken for more complex congenital syndromes such as hypogenetic lung syndrome. We report imaging findings in a rare incidentally detected case of bilateral meandering pulmonary veins. We briefly discuss the role of imaging in diagnosing this condition, with particular emphasis on contrast-enhanced CT. (orig.)

Congenital Central Hypoventilation Syndrome And Intestinal

African Journals Online (AJOL)

Congenital central hypoventilation syndrome (CCHS) or 'Ondine's curse' is characterised by an abnormal ventilatory response to progressive hypercapnia and sustained hypoxaemia. During the neonatal period, most patients with this condition present with hypoventilation or apnoea while asleep. Patients with CCHS may ...

High-Flow Nasal Cannula Therapy in a Patient with Reperfusion Pulmonary Edema following Percutaneous Transluminal Pulmonary Angioplasty

Directory of Open Access Journals (Sweden)

Kiyoshi Moriyama

2014-01-01

Full Text Available A 62-year-old woman with Wolff-Parkinson-White syndrome was with recent worsening of dyspnea to New York Heart Association functional status Class III. The patient was diagnosed as having central type chronic thromboembolic pulmonary hypertension. By cardiac catheterization, her mean pulmonary artery pressure was 53 mmHg with total pulmonary resistance 2238 dynes·sec·cm−5. After medical therapies with tadalafil, furosemide, ambrisentan, beraprost, and warfarin were initiated, percutaneous transluminal pulmonary angioplasty (PTPA was performed. Following PTPA, life-threating hypoxemia resulting from postoperative reperfusion pulmonary edema developed. High-flow nasal cannula therapy (HFNC was applied, and 100% oxygen at 50 L/min of flow was required to keep oxygenation. HFNC was continued for 3 days, and the patient was discharged on 8th postoperative day with SpO2 of 97% on 3 L/min of oxygen inhalation. Because of the simplicity of the technique, the lower cost of equipment, and remarkable patient tolerance to the treatment, we speculate that HFNC can take over the post of noninvasive ventilation as first-line therapy for patients with acute respiratory failure.

Primary Pulmonary Ewing's Sarcoma: Rare Cause of Superior Vena Cava Syndrome in Children.

Science.gov (United States)

Mehra, Shibani; Atwal, Swapndeep Singh; Garga, Umesh Chandra

2014-08-01

Ewing's sarcoma is a common malignant bone tumour presenting in children and young adults. Rarely extra- skeletal soft tissues and visceral organs can also be the site of origin of Ewing's sarcoma. Primary pulmonary Ewing's sarcoma is an extremely rare malignancy which occurs in the paediatric population. We report an unusual case of primary pulmonary Ewing's sarcoma in a nine year old girl who presented with features of superior vena cava syndrome in the emergency department. The diagnosis was confirmed pathologically both by light microscopy and immunohistochemistry. The patient was put on chemotherapy and surgery was planned but the patient expired within three days of starting chemotherapy.

Fatal Pulmonary Embolism Due to Inherited Thrombophilia Factors in a Child With Wolfram Syndrome.

Science.gov (United States)

Küçüktaşçi, Kazim; Semiz, Serap; Balci, Yasemin Işik; Özsari, Tamer; Gürses, Dolunay; Önem, Gökhan; Saçar, Mustafa; Düzcan, Füsun; Yüksel, Doğangün; Semiz, Ender

2016-10-01

Wolfram syndrome-1 is a rare and severe autosomal recessive neurodegenerative disease characterized by diabetes mellitus (DM), optic atrophy, diabetes insipidus, and deafness. Poorly controlled type 1 DM increases the risk for thrombosis. However, coexistence of DM and hereditary thrombosis factors is rarely observed. Here we present the case of a 13.5-year-old, nonfollowed girl newly diagnosed with poorly controlled Wolfram syndrome on the basis of the results of clinical and laboratory examinations. On the eighth day after diabetic ketoacidosis treatment, pulmonary embolism developed in the subject. Thrombus identified in the right atrium using echocardiography was treated by emergency thrombectomy. Homozygous mutation in the methylenetetrahydrofolate reductase gene C677T, heterozygous factor-V Leiden mutation, and active protein C resistance were identified in the patient. The patient was lost because of a recurring episode of pulmonary embolism on the 86th day of hospitalization. We present this case to highlight the need for investigating hereditary thrombosis risk factors in diabetic patients in whom thromboembolism develops.

Hantavirus pulmonary syndrome (rio Mamore virus) in the peruvian Amazon region

OpenAIRE

Mamani, Enrique; Centro Nacional de Salud Pública. Instituto Nacional de Salud. Lima, Perú. biólogo.; García, María P.; Centro Nacional de Salud Pública. Instituto Nacional de Salud. Lima, Perú. tecnólogo médico.; Miraval, María L.; Centro Nacional de Salud Pública. Instituto Nacional de Salud. Lima, Perú. médico anátomo-patólogo.; Valencia, Pedro; Centro Nacional de Salud Pública. Instituto Nacional de Salud. Lima, Perú. Médico infectólogo.; Quino, Alberto H.; Hospital Regional de Loreto, Gobierno Regional de Loreto. Loreto, Perú. médico intensivista.; Álvarez, Carlos; Unidad de Análisis y Generación de Evidencias en Salud Pública. Instituto Nacional de Salud. Lima, Perú. médico anátomo-patólogo.; Donaires, Luis F.; Dirección Regional de Salud de Loreto, Gobierno Regional de Loreto. Loreto, Perú. médico epidemiólogo.

2014-01-01

Hantavirus infection is a viral zoonotic infection borne by rodents which most letal form clinical is the Hantavirus Pulmonary Syndrome (SPH, Spanish abbreviation). The Mamore River variant originates in South America and was found in rodents without any association to human diseases. Two cases of SPH were identified in the Peruvian Amazon region in November 2011. In both cases, a molecular diagnostic testing was conducted by the Instituto Nacional de Salud from Peru. A phylogenetic analy...

Pulmonary embolism as the primary presenting feature of nephrotic syndrome

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Pallavi Periwal

2016-01-01

Full Text Available A 36-year-old previously healthy male presented with subacute onset of shortness of breath and chest pain. He was diagnosed with bilateral extensive pulmonary embolism (PE. In the absence of any predisposing factors, an extensive workup for unprovoked thrombophilia was done. During the course of his illness, the patient developed anasarca and was diagnosed to be suffering from nephrotic syndrome (NS, secondary to membranous glomerulopathy. Although, thrombotic complications are commonly associated with NS, it is unusual for PE to be the primary presenting feature in these patients.

CT features of pulmonary mycobacterial disease in patients with acquired immunodeficiency syndrome

International Nuclear Information System (INIS)

Zhu Ying; Zhang Zhiyong; Shi Yuxin; Feng Feng

2013-01-01

Objective: To study the CT features of pulmonary non-tuberculous mycobacteria (NTM) disease in patients with acquired immunodeficiency syndrome (AIDS) and explore the different CT appearances between AIDS-NTM and AIDS-TB. Methods: CT findings of pulmonary NTM disease in 27 AIDS patients (NTM group) were retrospectively analyzed and compared with that of tuberculosis in 30 AIDS patients (TB group). The results were statistically analyzed using Fisher's exact test. Results: CT findings of NTM appeared significantly more than that of TB as follows: high-density nodules (n = 18 vs 1, P < 0.01), ground-glass opacities (n = 10 vs 0, P < 0.01), fibrotic band (n = 17 vs 3, P < 0.01), bronchiectasis (9 vs 2, P = 0.012). CT findings of NTM appeared significantly less than that of TB as follows: miliary nodules (0 vs 6, P = 0.016), air space consolidations (n = 2 vs 11, P < 0.01), pleural effusion (n = 1 vs 9, P < 0.01). Conclusion: Nodule and fibrotic band accompanied with bronchiectasis were the main CT manifestations of pulmonary NTM disease in AIDS patients, while air space consolidation accompanied with pleural effusion and miliary nodules were the predominate CT findings of pulmonary tuberculosis in AIDS patients. (authors)

Vascular Ehlers-Danlos Syndrome With a Novel Missense COL3A1 Mutation Present With Pulmonary Complications and Iliac Arterial Dissection.

Science.gov (United States)

Gu, Guangchao; Yang, Hang; Cui, Lijia; Fu, Yuanyuan; Li, Fangda; Zhou, Zhou; Zheng, Yuehong

2018-02-01

Vascular Ehlers-Danlos syndrome (vEDS) is a life-threatening connective tissue disorder due to its high tendency of arterial and organ rupture. Pulmonary complications in vEDS are rare. We present a young male patient with vEDS who developed severe pulmonary complications and severe rupture of the iliac artery at different stages of his life. Vascular Ehlers-Danlos syndrome was diagnosed based on clinical manifestations and confirmed by the identification of COL3A1 gene mutation. Due to high bleeding tendency and weak cardiopulmonary capacity, conservative treatment was taken for him. To our knowledge, this is the first report of vEDS case in which the patient developed both pulmonary complications and dissection of large arteries. Our report emphasizes the importance of considering vEDS when an adolescent develops unexplained pulmonary cysts with fragility of lung tissues. Genetic counseling and close monitoring should be performed for earlier diagnosis and prevention of severe complications of large arteries. The typical presentations of vEDS were also discussed by means of a review of case reports on vEDS with pulmonary complications.

Sensitivity and specificity of Ga-67 pulmonary scans for the detection of p. carinii pneumonitis in patients with the acquired immunodefficiency syndrome and pulmonary symptoms

International Nuclear Information System (INIS)

Hattner, R.S.; Sollitto, R.A.; Golden, J.A.; Coleman, D.L.; Okerlund, M.D.

1984-01-01

Acquired immunodeficiency syndrome (AIDS) is a severe disorder of cellular immunity of obscure etiology. Since its original recognition in 1981 the incidence of AIDS has doubled in each of the succeeding six months. The most common causes of death in AIDS are Kaposi's sarcoma and p. carinii pneumonia (PCP). The latter is treatable if diagnosed early, and AIDS patients (pts) may suffer recurrent episodes of PCP. Since the invasive technique of fiberoptic bronchoscopy with transbronchial biopsy, brushing, and bronchialveolar lavage are necessary for diagnosis and follow-up a noninvasive method of categorizing which AIDS pts require this procedure would be most welcome. Twenty-one pts with the syndrome of AIDS and pulmonary symptoms underwent Ga-67 scans of the thoracic region, and fibroptic bronchoscopy with washings, and brush and transbronchial biopsy. Pulmonary activity was graded in a blinded fashion by three experienced observers as follows: 1, less than, or equal to adjacent soft tissues; 2, greater than adjacent soft tissues, but less than liver; 3, equal to liver; 4, greater than liver. Eleven pts had documented PCP, and the remaining ten had non-specific pulmonary inflammation, or other, in some cases, putative, infections. The sensitivity and specificity of Ga-67 scans ≥ grade 3 was 100% and 90% respectively. These results suggests a useful role for graded Ga-67 scans in AIDS pts with pulmonary symptoms, permitting selection of pts with a high risk of PCP for further mandatory invasive investigation of this otherwise usually fatal disease

Interstage evaluation of homograft-valved right ventricle to pulmonary artery conduits for palliation of hypoplastic left heart syndrome.

Science.gov (United States)

Sandeep, Nefthi; Punn, Rajesh; Balasubramanian, Sowmya; Smith, Shea N; Reinhartz, Olaf; Zhang, Yulin; Wright, Gail E; Peng, Lynn F; Wise-Faberowski, Lisa; Hanley, Frank L; McElhinney, Doff B

2018-04-01

Palliation of hypoplastic left heart syndrome with a standard nonvalved right ventricle to pulmonary artery conduit results in an inefficient circulation in part due to diastolic regurgitation. A composite right ventricle pulmonary artery conduit with a homograft valve has a hypothetical advantage of reducing regurgitation, but may differ in the propensity for stenosis because of valve remodeling. This retrospective cohort study included 130 patients with hypoplastic left heart syndrome who underwent a modified stage 1 procedure with a right ventricle to pulmonary artery conduit from 2002 to 2015. A composite valved conduit (cryopreserved homograft valve anastomosed to a polytetrafluoroethylene tube) was placed in 100 patients (47 aortic, 32 pulmonary, 13 femoral/saphenous vein, 8 unknown), and a nonvalved conduit was used in 30 patients. Echocardiographic functional parameters were evaluated before and after stage 1 palliation and before the bidirectional Glenn procedure, and interstage interventions were assessed. On competing risk analysis, survival over time was better in the valved conduit group (P = .040), but this difference was no longer significant after adjustment for surgical era. There was no significant difference between groups in the cumulative incidence of bidirectional Glenn completion (P = .15). Patients with a valved conduit underwent more interventions for conduit obstruction in the interstage period, but this difference did not reach significance (P = .16). There were no differences between groups in echocardiographic parameters of right ventricle function at baseline or pre-Glenn. In this cohort of patients with hypoplastic left heart syndrome, inclusion of a valved right ventricle to pulmonary artery conduit was not associated with any difference in survival on adjusted analysis and did not confer an identifiable benefit on right ventricle function. Copyright © 2017 The American Association for Thoracic Surgery. Published by Elsevier

Dietary N,N-dimethylglycine supplementation improves nutrient digestibility and attenuates pulmonary hypertension syndrome in broilers.

Science.gov (United States)

Kalmar, I D; Cools, A; Buyse, J; Roose, P; Janssens, G P J

2010-12-01

N,N-dimethylglycine (DMG) is an intermediary metabolite in cellular choline and betaine metabolism. The present trial aimed to evaluate the effect of dietary DMG on nutrient digestibility and development of pulmonary hypertension syndrome in broilers. A total of 64 14-day-old broiler hens (Ross-308) were raised until age 40 days under cold environmental temperature conditions (15 °C) and were fed a high energy feed in order to incite pulmonary hypertension. Birds were randomly assigned to two groups of which each group had eight replicate pens of four birds each. Test diets contained 0 or 167 mg Na-DMG (Taminizer(®) D; Taminco N.V., Ghent, Belgium)/kg feed. N,N-dimethylglycine supplementation resulted in a significant improvement in apparent faecal digestibility of crude protein and nitrogen-free extract. Further, fulminant ascites was numerically lowered by DMG and incidence of pulmonary hypertension decreased significantly from 44.8% in the control group to 14.6% in the DMG group. Finally, fasted plasma level of non-esterified fatty acids (NEFA) was twofold in the control group in relation to the DMG group. In conclusion, these data demonstrate beneficial effects of DMG on digestibility of non-fat fractions, on fat metabolism and on progression towards broiler ascites syndrome. © 2010 Blackwell Verlag GmbH.

Pinch-off syndrome: transection of implantable central venous access device

OpenAIRE

Sugimoto, Takuya; Nagata, Hiroshi; Hayashi, Ken; Kano, Nobuyasu

2012-01-01

As the population of people with cancer increases so does the number of patients who take chemotherapy. Majority of them are administered parentally continuously. Implantable central venous catheter device is a good choice for those patients; however, severe complication would occur concerning the devices. Pinch-off syndrome is one of the most severe complications. The authors report a severe case of pinch-off syndrome. The patient with the implantable central venous device could not take che...

An Unusual Aneurysm of the Main Pulmonary Artery Presenting as Acute Coronary Syndrome

International Nuclear Information System (INIS)

Kholeif, Mona A.; El Tahir, Mohamed; Kholeif, Yasser A.; El Watidy, Ahmed

2006-01-01

A 70-year old man presented with retrosternal chest pain. His electrocardiogram showed nonspecific T wave changes. Cardiac-specific troponin I (cTnI) was elevated. His condition was managed as acute coronary syndrome, following which he had two minor episodes of hemoptysis. A CT pulmonary angiogram showed no evidence of pulmonary embolism, but a large mass lesion was seen in the mediastinum. Echocardiography and cardiac MRI demonstrated a large solid mass, arising from the right ventricular outflow tract and causing compression of the main pulmonary artery (MPA). The differential diagnosis included pericardial and myocardial tumors and clotted aneurysm of the MPA. At surgery, a clotted aneurysmal sac was identified originating from the MPA and the defect was healed. Aneurysms of the MPA are rare. They most commonly present with dyspnea and chest pain. Compression of surrounding structures produces protean manifestations. A high index of suspicion coupled with imaging modalities establishes the diagnosis. Blunt trauma to the chest, at the time of an accident 4 years previously, may explain this aneurysm. The patient's presentation with chest pain was probably due to compression and/or stretching of surrounding structures. Coronary artery compression simulating acute coronary syndrome has been documented in the literature. The rise in cTnI may have been due to right ventricular strain, as a result of right ventricular outflow obstruction by the aneurysm. This has not been reported previously in the literature. The saccular morphology and narrow neck of the aneurysm predisposed to stagnation leading to clotting of the lumen and healing of the tear, which caused the diagnostic difficulty

Human case of visceral larva migrans syndrome: pulmonary and hepatic involvement

Directory of Open Access Journals (Sweden)

Almatary A. M.

2016-12-01

Full Text Available Visceral Larva Migrans (VLM syndrome is commonly caused by larvae of roundworms Toxocara canis or Toxocara cati. Human toxocarosis is a soil-transmitted zoonosis, which may result in partial or general pathological changes in host tissues. We reported a case of 14-year-old boy presented with severe dry cough without dyspnea, mild chest and abdominal pain with general fatigue. Examination of peripheral blood showed marked increase in eosinophils. The chest radiography showed an infiltrative shadow in the lung fields. Chest CT demonstrated multiple opacities in both lungs. Abdominal CT showed multiple low attenuation areas in the liver. Ultrasound guided liver biopsy revealed granulomas with severe eosinophilic infiltration. The boy was treated with albendazole and responded radically. It is worth mentioning that this is the first case of hepato-pulmonary VLM syndrome in Egypt.

Phenotype of asthma-chronic obstructive pulmonary disease overlap syndrome.

Science.gov (United States)

Rhee, Chin Kook

2015-07-01

Many patients with asthma or chronic obstructive pulmonary disease (COPD) have overlapping characteristics of both diseases. By spirometric definition, patients with both fixed airflow obstruction (AO) and bronchodilator reversibility or fixed AO and bronchial hyperresponsiveness can be considered to have asthma-COPD overlap syndrome (ACOS). However, patients regarded to have ACOS by spirometric criteria alone are heterogeneous and can be classified by phenotype. Eosinophilic inflammation, a history of allergic disease, and smoke exposure are important components in the classification of ACOS. Each phenotype has a different underlying pathophysiology, set of characteristics, and prognosis. Medical treatment for ACOS should be tailored according to phenotype. A narrower definition of ACOS that includes both spirometric and clinical criteria is needed.

Metabolomic changes in patients with chronic obstructive pulmonary disease with abnormal Savda syndrome.

Science.gov (United States)

Xu, Wei-Fang; Upur, Halmurat; Wu, Yu-Hua; Mamtimin, Batur; Yang, Jian; Ga, Yong-Juan; You, Li

2015-02-01

The aim of this study was to determine the metabolic biomarkers for abnormal Savda syndrome in patients with chronic obstructive pulmonary disease (COPD). Based on Traditional Uyghur Medicine (TUM) theory, a total of 103 patients with COPD were classified into abnormal Savda and non-abnormal Savda syndrome groups and 52 healthy volunteers acted as the control group. Blood samples from the three groups were analyzed using nuclear magnetic resonance (NMR) spectroscopy combined with orthogonal projection to latent structure-discriminant analysis. NMR tests showed that the regional distributions of the patients with COPD with abnormal Savda syndrome, those with non-abnormal Savda syndrome and the control group were completely separate (P>0.05). The patients with COPD with abnormal Savda syndrome exhibited relatively low levels of amino acids, glycoproteins and unsaturated lipids (PAbnormal Savda syndrome was one of the main types of syndrome among the patients with COPD; increased age, a longer duration of illness and a higher disease severity were characteristic of this type of syndrome. In addition, the present study provided biochemical evidence for the TUM theory-based classification of patients with COPD; these biomarkers can be used in the clinic for the diagnosis of COPD with abnormal Savda syndrome. The study also demonstrated that the plasma metabolic disorder in patients with COPD with abnormal Savda syndrome was more serious than that in the control and COPD with non-abnormal Savda syndrome groups. The plasma metabolic disorder was also associated with a low immune function of the body and endocrine and energy metabolism disorders.

Congenital central hypoventilation syndrome mimicking mitochondrial disease.

Science.gov (United States)

Rojnueangnit, Kitiwan; Descartes, Maria

2018-03-01

Later-onset congenital central hypoventilation syndrome (LO-CCHS) does not present only breathing problems but can be present as episodic multiple organs involvement. Our unique case demonstrated LO-CCHS should be considered in the differential diagnosis of mitochondrial diseases and having nontypical polysomnography result.

Spinal cord injury with central cord syndrome from surfing.

Science.gov (United States)

Steinfeld, Yaniv; Keren, Yaniv; Haddad, Elias

2018-01-01

Central cord syndrome (CCS) is an injury to the center of the spinal cord. It is well known as a hyperextension injury, but it has never been described as a surfing injury. Our report describes this injury in detail. A 35-year-old male novice surfer presented to the emergency department with acute tetraplegia following falling off his surfboard and hitting sea floor at a shallow beach break. He was rescued by a fellow surfer while floating in the sea and unable to raise his head above sea level. Upon arrival at the hospital, tetraplegia and sensory deficits were noted. Radiological investigations showed advanced spinal stenosis at C4-6 levels. T2 magnetic resonance imaging (MRI) demonstrated myelopathy at C5-C6 level. He was diagnosed as having central cord syndrome, treated conservatively, and regained near full neurologic recovery after a month of rehabilitation. Unique sport activities lead to unique injuries. It is important to accurately describe these injuries in order to create protective measures against them. Neurologic injuries in surfers are uncommon. With low-energy trauma, surfer's myelopathy is still the most common diagnosis, but central cord syndrome should be in the differential diagnosis.

Impact of evolving strategy on clinical outcomes and central pulmonary artery growth in patients with bilateral superior vena cava undergoing a bilateral bidirectional cavopulmonary shunt.

Science.gov (United States)

Honjo, Osami; Tran, Kim-Chi D; Hua, Zhongdong; Sapra, Priya; Alghamdi, Abdullah A; Russell, Jennifer L; Caldarone, Christopher A; Van Arsdell, Glen S

2010-09-01

We reported a high incidence of thrombosis, central pulmonary artery hypoplasia, and mortality for bilateral bidirectional cavopulmonary shunts. We hypothesized that technical modifications in the cavopulmonary anastomosis and anticoagulation would limit thrombus and central pulmonary artery hypoplasia, and thereby improve outcomes. Sixty-one patients (median age, 8.4 months; weight, 6.6 kg) underwent bilateral bidirectional cavopulmonary shunt from 1990 to 2007. The cohort was divided into 2 groups: 1) the conventional group (1990-1999, n = 37) and 2) the V-shaped group, with a hemi-Fontan or modification in which the cavae were anastomosed to the pulmonary artery adjacent to each other so they formed the appearance of a V (1999-2007, n = 24). Central and branch pulmonary artery growth, survival, and reinterventions were determined. The pre-Fontan study showed equivalent superior venae cavae and Nakata indices. The central pulmonary artery index and central pulmonary artery/Nakata index ratio were significantly higher in the V-shaped group (P analysis showed anastomotic strategy, low saturation, and thrombosis were predictors for death. Anastomotic strategy, lack of anticoagulation, thrombosis, and small superior venae cavae were predictors for reintervention (P strategy affected reintervention. Anastomotic strategy and postoperative thrombus affected mortality. 2010 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.

High-resolution computed tomography findings in eight patients with hantavirus pulmonary syndrome

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Barbosa, Diego de Lacerda; Hochhegger, Bruno; Souza Junior, Arthur Soares; Zanetti, Glaucia; Escuissato, Dante Luiz; Meirelles, Gustavo de Souza Portes; Funari, Marcelo Buarque de Gusmao; Marchiori, Edson, E-mail: edmarchiori@gmail.com [Universidade Federal do Rio de Janeiro (UFRJ), Rio de Janeiro, RJ (Brazil); Santa Casa de Porto Alegre, Porto Alegre, RS (Brazil); Faculdade de Medicina de Sao Jose do Rio Preto (FAMERP), SP (Brazil); Ultra X, Sao Jose do Rio Preto, SP (Brazil); Universidade Federal do Parana (UFPR), Curitiba, PR (Brazil); Universidade Federal de Sao Paulo (UNIFESP), Sao Paulo, SP (Brazil); Grupo Fleury, Sao Paulo, SP (Brazil); Universidade de Sao Paulo (FM/USP), Sao Paulo, SP (Brazil). Faculdade de Medicina

2017-05-15

Objective: The purpose of this study was to describe the high-resolution computed tomography (HRCT) findings in patients with hantavirus pulmonary syndrome (HPS). Materials and Methods: We retrospectively reviewed HRCT findings from eight cases of HPS. All patients were men, aged 19-70 (mean, 41.7) years. Diagnoses were established by serological test (enzyme-linked immunosorbent assay) in all patients. Two chest radiologists analyzed the images and reached decisions by consensus. Results: The predominant HRCT findings were ground-glass opacities (GGOs) and smooth inter- and intralobular septal thickening, found in all eight cases; however, the crazy-paving pattern was found in only three cases. Pleural effusion and peribronchovascular thickening were observed in five patients. The abnormalities were bilateral in all patients. Conclusion: The predominant HRCT findings in patients with HPS were GGOs and smooth inter- and intralobular septal thickening, which probably correlate with the histopathologic findings of pulmonary edema. (author)

High-resolution computed tomography findings in eight patients with hantavirus pulmonary syndrome

International Nuclear Information System (INIS)

Barbosa, Diego de Lacerda; Hochhegger, Bruno; Souza Junior, Arthur Soares; Zanetti, Glaucia; Escuissato, Dante Luiz; Meirelles, Gustavo de Souza Portes; Funari, Marcelo Buarque de Gusmao; Marchiori, Edson; Santa Casa de Porto Alegre, Porto Alegre, RS; Faculdade de Medicina de Sao Jose do Rio Preto; Ultra X, Sao Jose do Rio Preto, SP; Universidade Federal do Parana; Universidade Federal de Sao Paulo; Grupo Fleury, Sao Paulo, SP; Universidade de Sao Paulo

2017-01-01

Objective: The purpose of this study was to describe the high-resolution computed tomography (HRCT) findings in patients with hantavirus pulmonary syndrome (HPS). Materials and Methods: We retrospectively reviewed HRCT findings from eight cases of HPS. All patients were men, aged 19-70 (mean, 41.7) years. Diagnoses were established by serological test (enzyme-linked immunosorbent assay) in all patients. Two chest radiologists analyzed the images and reached decisions by consensus. Results: The predominant HRCT findings were ground-glass opacities (GGOs) and smooth inter- and intralobular septal thickening, found in all eight cases; however, the crazy-paving pattern was found in only three cases. Pleural effusion and peribronchovascular thickening were observed in five patients. The abnormalities were bilateral in all patients. Conclusion: The predominant HRCT findings in patients with HPS were GGOs and smooth inter- and intralobular septal thickening, which probably correlate with the histopathologic findings of pulmonary edema. (author)

Interventional bronchoscopy in malignant central airway obstruction by extra-pulmonary malignancy.

Science.gov (United States)

Shin, Beomsu; Chang, Boksoon; Kim, Hojoong; Jeong, Byeong-Ho

2018-03-13

Interventional bronchoscopy is considered an effective treatment option for malignant central airway obstruction (MCAO). However, there are few reports of interventional bronchoscopy in patients with MCAOs due to extra-pulmonary malignancy. Therefore, the objective of this study was to investigate treatment outcomes and prognostic factors for bronchoscopic intervention in patients with MCAO due to extra-pulmonary malignancy. We retrospectively analyzed consecutive 98 patients with MCAO due to extra-pulmonary malignancy who underwent interventional bronchoscopy between 2004 and 2014 at Samsung Medical Center (Seoul, Korea). The most common primary site of malignancy was esophageal cancer (37.9%), followed by thyroid cancer (16.3%) and head & neck cancer (10.2%). Bronchoscopic interventions were usually performed using a combination of mechanical debulking (84.7%), stent insertion (70.4%), and laser cauterization (37.8%). Of 98 patients, 76 (77.6%) patients had MCAO due to progression of malignancy, and 42 (42.9%) patients had exhausted all other anti-cancer treatment at the time of bronchoscopic intervention. Technical success was achieved in 89.9% of patients, and acute complications and procedure-related deaths occurred in 20.4% and 3.1% of patients, respectively. Reduced survival was associated with MCAO due to cancer other than thyroid cancer or lymphoma, mixed lesions, and not receiving adjuvant treatment after bronchoscopic intervention. Bronchoscopic intervention could be a safe and effective procedure for MCAO due to end-stage extra-pulmonary malignancies. In addition, we identified possible prognostic factors for poor survival after intervention, which could guide clinicians select candidates that will benefit from bronchoscopic intervention.

Multiple cavities with halo sign in a case of invasive pulmonary aspergillosis during therapy for drug-induced hypersensitivity syndrome

Directory of Open Access Journals (Sweden)

Tomoo Ikari

2017-01-01

Full Text Available A 67-year-old female with rheumatoid arthritis and asthma-chronic obstructive pulmonary disease overlap syndrome was admitted for drug-induced hypersensitivity syndrome (DIHS caused by salazosulfapyridine. Human herpes virus 6 (HHV-6 variant B was strongly positive on peripheral blood. Multiple cavities with ground grass opacities rapidly emerged predominantly in the upper and middle lobes. She was diagnosed with invasive pulmonary aspergillosis (IPA, and was treated successfully with antifungal agents. Therapeutic systemic corticosteroids, emphysematous change in the lungs, and the worsening of the patient's general condition due to DIHS were considered major contributing factor leading to IPA. HHV-6 reactivation could have an effect on clinical course of IPA. Cavities with halo sign would provide an early clue to IPA in non-neutropenic and immunosuppressive patients.

Hantavirus Pulmonary Syndrome in Santa Cruz, Bolivia: Outbreak Investigation and Antibody Prevalence Study

Science.gov (United States)

2012-10-18

Ecology and epidemiology of an emerging virus in Latin America]. Medicina (B Aires) 66: 343–356. 22. Weissenbacher MC, Cura E, Segura EL, Hortal M, Baek LJ...et al. (1996) Serological evidence of human Hantavirus infection in Argentina, Bolivia and Uruguay. Medicina (B Aires) 56: 17–22. 23. Pini N (2004...Hantavirus in human and rodent population in an endemic area for hantavirus pulmonary syndrome in Argentina]. Medicina (B Aires) 62: 1–8. 26. Simonsen L

Variable pulmonary manifestations in hemodialysis patients

International Nuclear Information System (INIS)

Kim, Yoo Kyung; Shim, Sung Shine; Shin, Jung Hee; Choi, Gyu Bock; Lee, Kyung Soo; Yi, Chin A; Oh, Yu Whan

2003-01-01

A wide variety of pulmonary disorders related to hemodialysis or pre-existing renal disease occurs in hemodialysis patients. The disorders may be classified as 1) pulmonary abnormalities associated with chronic renal failures; 2) pulmonary complications arising during hemodialysis; 3) pulmonary infection; or 4) pulmonary-renal syndrome. An awareness of the various possible pulmonary disorders arising in hemodialysis patients may be helpful for the proper and timely management of such patients. We describe and illustrate various radiographic and CT findings of variable pulmonary disorders in hemodialysis patients

Anti-hLAMP2-antibodies and dual positivity for anti-GBM and MPO-ANCA in a patient with relapsing pulmonary-renal syndrome

Directory of Open Access Journals (Sweden)

Kistler Thomas

2011-06-01

Full Text Available Abstract Background Pulmonary-renal syndrome associated with anti-glomerular basement membrane (GBM antibodies, also known as Goodpasture's syndrome, is a rare but acute and life-threatening condition. One third of patients presenting as anti-GBM antibody positive pulmonary-renal syndrome or rapidly progressive glomerulonephritis are also tested positive for anti-neutrophil cytoplasmic antibodies (ANCA. Whilst anti-GBM disease is considered a non-relapsing condition, the long-term course of double-positive patients is less predictable. Case Presentation We report a patient with such dual positivity, who presented with pulmonary hemorrhage, crescentic glomerulonephritis and membranous nephropathy. Plasmapheresis in combination with immunosuppresive therapy led to a rapid remission but the disease relapsed after two years. The serum of the patient was tested positive for antibodies to human lysosomal membrane protein 2 (hLAMP2, a novel autoantigen in patients with active small-vessel vasculitis (SVV. The anti-hLAMP2 antibody levels correlated positively with clinical disease activity in this patient. Conclusion We hypothesize that this antibody may indicate a clinical course similar to ANCA-associated vasculitis in double-positive patients. However, this needs to be confirmed on comprehensive patient cohorts.

Hantavirus Pulmonary Syndrome in Argentina, 1995–2008

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Bellomo, Carla M.; Cacace, María Luisa; Suárez, Paola; Bogni, Liliana; Padula, Paula J.

2010-01-01

We report a large case series of hantavirus pulmonary syndrome (HPS) in Argentina that was confirmed by laboratory restuls from 1995 through 2008. The geographic and temporal distribution of cases by age, sex, fatality rate, and risk factors for HPS was analyzed. A total of 710 cases were unequally distributed among 4 of the 5 Argentine regions. Different case-fatality rates were observed for each affected region, with a maximum rate of 40.5%. The male-to-female ratio for HPS case-patients was 3.7:1.0; the case-fatality rate was significantly higher for women. Agriculture-associated activities were most commonly reported as potential risk factors, especially among men of working age. Although HPS cases occurred predominantly in isolation, we identified 15 clusters in which strong relationships were observed between members, which suggests ongoing but limited person-to-person transmission. PMID:21122213

Evaluation of the monitor cursor-line method for measuring pulmonary artery and central venous pressures.

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Pasion, Editha; Good, Levell; Tizon, Jisebelle; Krieger, Staci; O'Kier, Catherine; Taylor, Nicole; Johnson, Jennifer; Horton, Carrie M; Peterson, Mary

2010-11-01

To determine if the monitor cursor-line feature on bedside monitors is accurate for measuring central venous and pulmonary artery pressures in cardiac surgery patients. Central venous and pulmonary artery pressures were measured via 3 methods (end-expiratory graphic recording, monitor cursor-line display, and monitor digital display) in a convenience sample of postoperative cardiac surgery patients. Pressures were measured twice during both mechanical ventilation and spontaneous breathing. Analysis of variance was used to determine differences between measurement methods and the percentage of monitor pressures that differed by 4 mm Hg or more from the measurement obtained from the graphic recording. Significance level was set at P less than .05. Twenty-five patients were studied during mechanical ventilation (50 measurements) and 21 patients during spontaneous breathing (42 measurements). Measurements obtained via the 3 methods did not differ significantly for either type of pressure (P > .05). Graphically recorded pressures and measurements obtained via the monitor cursor-line or digital display methods differed by 4 mm Hg or more in 4% and 6% of measurements, respectively, during mechanical ventilation and 4% and 11%, respectively, during spontaneous breathing. The monitor cursor-line method for measuring central venous and pulmonary artery pressures may be a reasonable alternative to the end-expiratory graphic recording method in hemodynamically stable, postoperative cardiac surgery patients. Use of the digital display on the bedside monitor may result in larger discrepancies from the graphically recorded pressures than when the cursor-line method is used, particularly in spontaneously breathing patients.

[Clinical effect of high-frequency oscillatory ventilation combined with pulmonary surfactant in treatment of neonatal severe meconium aspiration syndrome complicated by pulmonary hemorrhage].

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Huang, Jing; Lin, Xin-Zhu; Zheng, Zhi

2016-11-01

To study the clinical effect and safety of high-frequency oscillatory ventilation (HFOV) combined with pulmonary surfactant (PS) in the treatment of neonatal severe meconium aspiration syndrome (MAS) complicated by neonatal pulmonary hemorrhage (NPH). A total of 48 children with severe MAS complicated by NPH were enrolled, and a retrospective analysis was performed for the clinical effects of HFOV+PS (trial group, 25 children) and HFOV alone (control group, 23 children). The blood gas parameters, oxygenation index (OI), PaO 2 /FiO 2 (P/F) value, duration of pulmonary hemorrhage, ventilation time, length of hospital stay, incidence of complications, and outcome were compared between the two groups. At 6, 12, 24, and 48 hours after treatment, the trial group had significantly better PaO 2 , OI, and P/F value than the control group (Phemorrhage (P0.05). HFOV combined with PS can better improve oxygenation function and shorten the duration of NPH and ventilation time. Meanwhile, it does not increase the incidence of adverse events. Therefore, it is a safe and effective therapy.

Pinch-off syndrome: transection of implantable central venous access device.

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Sugimoto, Takuya; Nagata, Hiroshi; Hayashi, Ken; Kano, Nobuyasu

2012-11-30

As the population of people with cancer increases so does the number of patients who take chemotherapy. Majority of them are administered parentally continuously. Implantable central venous catheter device is a good choice for those patients; however, severe complication would occur concerning the devices. Pinch-off syndrome is one of the most severe complications. The authors report a severe case of pinch-off syndrome. The patient with the implantable central venous device could not take chemotherapy because the device occluded. Further examination revealed the transection of the catheter. The transected fragment of the catheter in the heart was successfully removed by using a loop snare placed through the right femoral vein.

Hantavirus Pulmonary Syndrome in Santa Cruz, Bolivia: Outbreak Investigation and Antibody Prevalence Study

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Montgomery, Joel M.; Blair, Patrick J.; Carroll, Darin S.; Mills, James N.; Gianella, Alberto; Iihoshi, Naomi; Briggiler, Ana M.; Felices, Vidal; Salazar, Milagros; Olson, James G.; Glabman, Raisa A.; Bausch, Daniel G.

2012-01-01

We report the results of an investigation of a small outbreak of hantavirus pulmonary syndrome in 2002 in the Department of Santa Cruz, Bolivia, where the disease had not previously been reported. Two cases were initially reported. The first case was a physician infected with Laguna Negra virus during a weekend visit to his ranch. Four other persons living on the ranch were IgM antibody-positive, two of whom were symptomatic for mild hantavirus pulmonary syndrome. The second case was a migrant sugarcane worker. Although no sample remained to determine the specific infecting hantavirus, a virus 90% homologous with Río Mamoré virus was previously found in small-eared pygmy rice rats (Oligoryzomys microtis) trapped in the area. An antibody prevalence study conducted in the region as part of the outbreak investigation showed 45 (9.1%) of 494 persons to be IgG positive, illustrating that hantavirus infection is common in Santa Cruz Department. Precipitation in the months preceding the outbreak was particularly heavy in comparison to other years, suggesting a possible climatic or ecological influence on rodent populations and risk of hantavirus transmission to humans. Hantavirus infection appears to be common in the Santa Cruz Department, but more comprehensive surveillance and field studies are needed to fully understand the epidemiology and risk to humans. PMID:23094116

Onconeuronal and antineuronal antibodies in patients with neoplastic and non-neoplastic pulmonary pathologies and suspected for paraneoplastic neurological syndrome

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Michalak S

2009-12-01

Full Text Available Abstract Objective Onconeuronal antibodies are important diagnostic tool in patients with suspicion of paraneoplastic neurological syndromes (PNS. However, their role in PNS pathophysiology and specificity for particular neurological manifestation remains unclear. The aim of this study was to evaluate onconeuronal and antineuronal antibodies in patients with pulmonary pathologies and suspected for PNS. Materials and methods Twenty one patients with pulmonary pathologies were selected from the database of 525 consecutive patients with suspicion of PNS. Patients' sera were screened for the presence of onconeuronal and antineuronal antibodies by means of indirect immunofluorescence; the presence was confirmed by Western blotting. Clinical data were obtained from medical records, hospital data base, and questionnaire-based direct telephone contact with patients. Results Among 21 patients, aged 54 ± 11, with pulmonary pathologies, the most frequent neurological manifestations were neuropathies. Typical PNS included paraneoplastic cerebellar degeneration (PCD and limbic encephalitis (LE. We found cases with multiple onconeuronal antibodies (anti-Ri and anti-Yo and coexisting PNS (PCD/LE. Well-defined onconeuronal antibodies were identified in 23.8% of patients. Among antineuronal antibodies, the most frequent were anti-MAG (23.8%. ROC curves analysis revealed high sensitivity of onconeuronal and antineuronal antibodies for typical PNS and lower for pulmonary malignancies. Conclusions Tests for antibodies are highly sensitive for the diagnosis of typical paraneoplastic neurological syndromes. Anti-myelin and anti-MAG antibodies are associated with non-neoplastic pulmonary diseases. Patients with well-defined onconeuronal antibodies require careful screening and follow-up, because the PNS diagnosis indicates a high probability of an underlying malignancy.

Myocardial Ischaemia, Coronary Atherosclerosis and Pulmonary Pressure Elevation in Antiphospholipid Syndrome Patients.

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Padjas, Agnieszka; Płazak, Wojciech; Celińska-Lowenhoff, Magdalena; Mazurek, Adam; Perricone, Carlo; Podolec, Piotr; Musiał, Jacek

2016-01-01

Thrombotic events in antiphospholipid syndrome (APS) involve venous and arterial circulation with the possible involvement of coronary or pulmonary microcirculation. To evaluate the influence of antiphospholipid antibodies (aPL) and on myocardial ischaemia assessed by single-photon emission computerized tomography (SPECT), coronary atherosclerosis assessed by multidetector computerized tomography (MDCT) and pulmonary pressure assessed by transthoracic echocardiography (TTE) in patients with primary antiphospholipid syndrome (PAPS). TTE, SPECT (Tc 99m sestamibi) and MDCT-based coronary calcium scoring were performed in 26 consecutive PAPS patients (20 females, 6 males, aged 20-61, mean 39.7) without any signs of other autoimmunological disease and without clinical symptoms of heart disease. Out of 26 patients, TEE showed normal left and right ventricle function in 25 (96.2%) and elevated (≥ 30 mm Hg) right ventricle systolic pressure in 7 (26.9%) patients. SPECT revealed myocardial perfusion defects in 15 (57.7%) patients: exercise-induced in 6 (23.1%) and persistent in 11 (42.3%). MDCT revealed coronary calcifications in 4 (15.4%) patients. The number of plaques ranged from 1 to 11 (median 2), volume 3-201.7 mm³ (median 7), calcium scores 1.3-202.6 (median 5.7). In the group with perfusion defects or coronary calcifications (n = 15), all the patients showed elevated aCL IgG. In most of the relatively young APS patients, without any symptoms of ischemic heart disease, SPECT showed myocardial perfusion defects, and coronary calcifications in 1/6 of them. Right ventricle systolic pressure was elevated in 1/4 of APS patients. These pathologies, well known as cardiovascular risk markers, were associated with elevated levels of the IgG class of both anti-cardiolipin and antiB2 GPI antibodies. Thus, in a high percentage of APS patients, clinically silent myocardial ischaemia, pulmonary pressure elevation and coronary atherosclerosis are present and related to the

Unilateral right pulmonary artery agenesis and congenital cystic adenomatoid malformation of the right lung with Ortner′s syndrome

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Jane Jackie David

2016-01-01

Full Text Available We report a 2.5-year-old girl who presented with hoarseness of voice since 3 months of age and failure to thrive. Chest X-ray showed cardiomegaly with a deviation of the trachea and mediastinum to the right side. Two-dimensional echocardiography showed decreased flow across the right pulmonary artery, a small atrial septal defect (ASD with a right-to-left shunt, and a dilated right atrium and right ventricle with severe tricuspid regurgitation suggestive of severe pulmonary hypertension. A silent large patent ductus arteriosus was also seen. Multiple detector computerized tomography aortogram confirmed the findings of absent right pulmonary artery and hypoplastic right lung with small cystic lesions suggestive of congenital cystic adenomatoid malformation in the right lower lobe. Hoarseness of voice was due to the left vocal cord palsy probably secondary to severe pulmonary hypertension (Ortner′s syndrome.

X-linked Hyper IgM Syndrome Presenting as Pulmonary Alveolar Proteinosis.

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Gallagher, Joel; Adams, Juan; Hintermeyer, Mary; Torgerson, Troy R; Lopez-Guisa, Jesus; Ochs, Hans D; Szabo, Sara; Salib, Mina; Verbsky, James; Routes, John

2016-08-01

X-linked hyper IgM syndrome (XHIGM) is a combined immunodeficiency caused by mutations in the CD40 ligand (CD40L) gene that typically results in decreased or absent CD40L expression on activated T cells, leading to defective class switching and somatic hypermutation. We describe an infant who presented with respiratory failure due to pulmonary alveolar proteinosis (PAP) with a novel damaging missense mutation in the CD40L gene. Whole exome sequencing (WES) was used to identify a mutation in the CD40L gene. CD40L expression and function were determined by flow cytometry. A 5-month-old previously-healthy male presented with respiratory failure and diffuse pulmonary ground glass opacities on CT scan of the chest. Laboratory evaluation revealed an undetectable IgG, normal IgA, and elevated IgM. A bronchoalveolar lavage demonstrated pulmonary alveolar proteinosis. WES demonstrated a c.608G > C mutation in the CD40L gene resulting in p.R203T. Flow cytometry demonstrated normal CD40L expression on activated T cells but absent binding of CD40-Ig to CD40L on activated patient T cells. The clinical manifestations of XHIGM in our patient had several unique features, including the presentation with PAP, normal serum IgA, and expression of non-functional CD40L on activated T cells. To our knowledge, this is the first published case of PAP in a patient with XHIGM.

Acute traumatic central cord syndrome: analysis of clinical and radiological correlations.

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Miranda, P; Gomez, P; Alday, R

2008-12-01

In patients with traumatic spinal cord injury, several studies correlate neurological impairment and radiological findings. However, little information is available about this correspondence in the particular group of acute traumatic central cord syndrome. The object of the present work was to describe the clinical and radiological features of a series of patients presenting with acute traumatic central cord syndrome and to analyze clinical and radiological correlations on admission and at last follow-up. Retrospective review of 15 patients diagnosed of acute traumatic central cord syndrome between 1995 and 2005. Global motor score and motor score in upper extremities were determined on admission and at last follow-up (6 months-4 years, mean 16 months). Plain films, cervical computed tomography and magnetic resonance (MR) were performed in every patient and retrieved for the study. In seven patients, serial MR studies were performed during follow-up. Clinical and radiological correlations were statistically analyzed with non-parametric tests. Cervical spondylosis appeared associated with older age, falls, and absence of fracture. Spinal cord edema was the most common finding in MR studies but hemorrhage was also observed. The length of spinal cord edema significantly correlated with initial motor score. The decrease in T2-weighted hyperintensity in serial MR studies correlated with the gain of motor power in upper limbs at last follow-up. Elderly patients with more degenerated cervical spines commonly develop acute traumatic central cord syndrome after incidental falls. Length of spinal cord edema correlates with neurological impairment on admission and may provide significant prognostic information.

Clinical management and outcomes of patients with Hermansky-Pudlak syndrome pulmonary fibrosis evaluated for lung transplantation.

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El-Chemaly, Souheil; O'Brien, Kevin J; Nathan, Steven D; Weinhouse, Gerald L; Goldberg, Hilary J; Connors, Jean M; Cui, Ye; Astor, Todd L; Camp, Philip C; Rosas, Ivan O; Lemma, Merte; Speransky, Vladislav; Merideth, Melissa A; Gahl, William A; Gochuico, Bernadette R

2018-01-01

Pulmonary fibrosis is a progressive, fatal manifestation of Hermansky-Pudlak syndrome (HPS). Some patients with advanced HPS pulmonary fibrosis undergo lung transplantation despite their disease-associated bleeding tendency; others die while awaiting donor organs. The objective of this study is to determine the clinical management and outcomes of a cohort with advanced HPS pulmonary fibrosis who were evaluated for lung transplantation. Six patients with HPS-1 pulmonary fibrosis were evaluated at the National Institutes of Health Clinical Center and one of two regional lung transplant centers. Their median age was 41.5 years pre-transplant. Three of six patients died without receiving a lung transplant. One of these was referred with end-stage pulmonary fibrosis and died before a donor organ became available, and donor organs were not identified for two other patients sensitized from prior blood product transfusions. Three of six patients received bilateral lung transplants; they did not have a history of excessive bleeding. One patient received peri-operative desmopressin, one was transfused with intra-operative platelets, and one received extracorporeal membrane oxygenation and intra-operative prothrombin complex concentrate, platelet transfusion, and desmopressin. One transplant recipient experienced acute rejection that responded to pulsed steroids. No evidence of chronic lung allograft dysfunction or recurrence of HPS pulmonary fibrosis was detected up to 6 years post-transplant in these three lung transplant recipients. In conclusion, lung transplantation and extracorporeal membrane oxygenation are viable options for patients with HPS pulmonary fibrosis. Alloimmunization in HPS patients is an important and potentially preventable barrier to lung transplantation; interventions to limit alloimmunization should be implemented in HPS patients at risk of pulmonary fibrosis to optimize their candidacy for future lung transplants.

The central anticholinergic syndrome in the postoperative period

NARCIS (Netherlands)

J. Rupreht (Joze); B. Dworacek (B.)

1990-01-01

markdownabstractRésumé Le syndrome anticholinergique central (SAC) comporte des signes centraux (somnolence, confusion, amnésie, agitation, hallucination, dysarthrie, ataxie, délire, stupeur, coma) et des signes périphériques (sécheresse buccale et/ou cutanée, tachycardie, troubles visuels et

Hyperthyroidism hidden by congenital central hypoventilation syndrome.

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Fox, Danya A; Weese-Mayer, Debra E; Wensley, David F; Stewart, Laura L

2015-05-01

Congenital central hypoventilation syndrome (CCHS) is a rare neurocristopathy with severe central hypoventilation. CCHS results from a mutation in the paired-like homeobox 2B gene (PHOX2B). In addition to hypoventilation, patients with CCHS display a wide array of autonomic nervous system abnormalities, including decreased heart rate variability and abrupt sinus pauses, esophageal dysmotility, abnormal pupillary light response, and temperature dysregulation, to name a few. To date, there has been no documentation of a child with both CCHS and hyperthyroidism. We report the case of a young child with CCHS who presented with tachycardia, which was later found to be due to Grave's disease, after many months of investigation.

ARDS (Acute Respiratory Distress Syndrome)

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... Also known as What Is ARDS, or acute respiratory distress syndrome, is a lung condition that leads ... treat ARDS. Other Names Acute lung injury Adult respiratory distress syndrome Increased-permeability pulmonary edema Noncardiac pulmonary ...

Combined central retinal artery and vein occlusion in Churg-Strauss syndrome

DEFF Research Database (Denmark)

Hamann, Steffen; Johansen, Sven; Hamann, Steffen Ellitsgaard

2006-01-01

PURPOSE: To describe a rare case of Churg-Strauss syndrome presenting with severe visual loss due to a combined central retinal vein and artery occlusion. METHODS: A 42-year old man with a medical history of asthma and blood hypereosinophilia developed a sudden loss of vision in his right eye. We...... and dilated and tortuous veins. The diagnosis was confirmed by a fluorescein angiogram showing absence of retinal filling and normal choroidal filling. Churg-Strauss syndrome was diagnosed based on the necessary presence of four of six criteria for the disease proposed by the American College of Rheumatology...... the vascular occlusion and experienced no visual improvement. CONCLUSION: Combined central retinal artery and vein occlusion can occur in Churg-Strauss syndrome. We suggest that regional vasculitis may be the pathological mechanism underlying the vascular occlusions observed in our case. The condition carries...

The imaging appearances of the pulmonary mucormycosis in patients with acquired immunodeficiency syndrome

International Nuclear Information System (INIS)

Liu Jinxin; Tang Xiaoping; Zhang Lieguang; Jiang Songfeng; Chen Bihua; Gan Xinqing; Huang Ruilian; Shi Hongling; Huang Wuzhi; Huang Deyang; Tang Yong

2009-01-01

Objective: To manifest the imaging appearances of the pulmonary mucormycosis in patients with acquired immunodeficiency syndrome (AIDS). Methods: The radiographic and high resolution computed tomography (HRCT) features of the pulmonary, mucormycosis in 13 patients with AIDS were retrospectively analyzed. Results: On radiography, the infiltrative lesions were found in 5 patients, 7 cases had reticular pattern, 4 cases had pleural effusion, 4 cases had enlarged hilar and mediastinal lymph nodes, 3 cases had diffuse milliary lesions, 3 cases had masses, 2 cases had ground-glass shadows, 2 cases had cystic lesions, cavity, pleural thickening, pericardial effusion and focal pneumothorax was presented in 1 case respectively. On HRCT, 7 cases had enlarged mediastinal lymph nodes, 7 cases had interlobular septal thickening, the infiltrative lesion were found in 6 patients, 5 cases had diffuse milliary lesions, 4 cases had pleural effusion, 3 cases had masses, 2 eases had ground-glass shadows, 2 cases had cystic lesions, cavity, pleural thickening, focal bronchiectasis, pericardial effusion and focal pneumothorax was presented in 1 case respectively. Conclusion: The main imaging appearances of the pulmonary mucormycosis in patients with AIDS include diffuse milliary lesion, enlarged hilar and mediastinal lymph node, interlobular septal thickening, infiltrative lesion, pleural effusion and mass. (authors)

Pulmonary capillary pressure in pulmonary hypertension.

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Souza, Rogerio; Amato, Marcelo Britto Passos; Demarzo, Sergio Eduardo; Deheinzelin, Daniel; Barbas, Carmen Silvia Valente; Schettino, Guilherme Paula Pinto; Carvalho, Carlos Roberto Ribeiro

2005-04-01

Pulmonary capillary pressure (PCP), together with the time constants of the various vascular compartments, define the dynamics of the pulmonary vascular system. Our objective in the present study was to estimate PCPs and time constants of the vascular system in patients with idiopathic pulmonary arterial hypertension (IPAH), and compare them with these measures in patients with acute respiratory distress syndrome (ARDS). We conducted the study in two groups of patients with pulmonary hypertension: 12 patients with IPAH and 11 with ARDS. Four methods were used to estimate the PCP based on monoexponential and biexponential fitting of pulmonary artery pressure decay curves. PCPs in the IPAH group were considerably greater than those in the ARDS group. The PCPs measured using the four methods also differed significantly, suggesting that each method measures the pressure at a different site in the pulmonary circulation. The time constant for the slow component of the biexponential fit in the IPAH group was significantly longer than that in the ARDS group. The PCP in IPAH patients is greater than normal but methodological limitations related to the occlusion technique may limit interpretation of these data in isolation. Different disease processes may result in different times for arterial emptying, with resulting implications for the methods available for estimating PCP.

Measurement of regional pulmonary blood volume in patients with increased pulmonary blood flow or pulmonary arterial hypertension

International Nuclear Information System (INIS)

Wollmer, P.; Rozcovek, A.; Rhodes, C.G.; Allan, R.M.; Maseri, A.

1984-01-01

The effects of chronic increase in pulmonary blood flow and chronic pulmonary hypertension on regional pulmonary blood volume was measured in two groups of patients. One group of patients had intracardiac, left-to-right shunts without appreciable pulmonary hypertension, and the other consisted of patients with Eisenmenger's syndrome or primary pulmonary hypertension, i.e. patients with normal or reduced blood flow and severe pulmonary hypertension. A technique based on positron tomography was used to measure lung density (by transmission scanning) and regional pulmonary blood volume (after inhalation of /sup 11/CO). The distribution of pulmonary blood volume was more uniform in patients with chronic increase in pulmonary blood flow than in normal subjects. There were also indications of an absolute increase in intrapulmonary blood volume by about 15%. In patients with chronic pulmonary arterial hypertension, the distribution of pulmonary blood volume was also abnormally uniform. There was, however, no indication that overall intrapulmonary blood volume was substantially different from normal subjects. The abnormally uniform distribution of pulmonary blood volume can be explained by recruitment and/or dilatation of vascular beds. Intrapulmonary blood volume appears to be increased in patients with intracardiac, left-to-right shunts. With the development of pulmonary hypertension, intrapulmonary blood volume falls, which may be explained by reactive changes in the vasculature and/or obliteration of capillaries

Effect of pulmonary hyperinflation on central blood volume

DEFF Research Database (Denmark)

Mijacika, Tanja; Kyhl, Kasper; Frestad, Daria

2017-01-01

(11±7%) above the total lung capacity. All cardiac chambers decreased in volume and despite a heart rate increase of 24±29 bpm (39±50%), pulmonary blood flow decreased by 2783±1820mL (43±20%). The pulmonary transit time remained unchanged at 7.5±2.2s and pulmonary blood volume decreased by 354±176m...

Overlap Syndrome in Respiratory Medicine: Asthma and Chronic Obstructive Pulmonary Disease

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Alexandru Corlateanu

2014-02-01

Full Text Available Asthma and chronic obstructive pulmonary disease (COPD are highly prevalent chronic diseases in the general population. Both are characterized by similar mechanisms: airway inflammation, airway obstruction, and airway hyperresponsiveness. However, the distinction between the two obstructive diseases is not always clear. Multiple epidemiological studies demonstrate that in elderly people with obstructive airway disease, as many as half or more may have overlapping diagnoses of asthma and COPD. A COPD-Asthma overlap syndrome is defined as an airflow obstruction that is not completely reversible, accompanied by symptoms and signs of increased obstruction reversibility. For the clinical identification of overlap syndrome COPD-Asthma Spanish guidelines proposed six diagnostic criteria. The major criteria include very positive bronchodilator test [increase in forced expiratory volume in one second (FEV1 ≥15% and ≥400 ml], eosinophilia in sputum, and personal history of asthma. The minor criteria include high total IgE, personal history of atopy and positive bronchodilator test (increase in FEV1 ≥12% and ≥200 ml on two or more occasions. The overlap syndrome COPD-Asthma is associated with enhanced response to inhaled corticosteroids due to the predominance of eosinophilic bronchial inflammation.The future clinical studies and multicenter clinical trials should lead to the investigation of disease mechanisms and simultaneous development of the novel treatment.

Lung-Protective Ventilation With Low Tidal Volumes and the Occurrence of Pulmonary Complications in Patients Without Acute Respiratory Distress Syndrome: A Systematic Review and Individual Patient Data Analysis.

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Neto, Ary Serpa; Simonis, Fabienne D; Barbas, Carmen S V; Biehl, Michelle; Determann, Rogier M; Elmer, Jonathan; Friedman, Gilberto; Gajic, Ognjen; Goldstein, Joshua N; Linko, Rita; Pinheiro de Oliveira, Roselaine; Sundar, Sugantha; Talmor, Daniel; Wolthuis, Esther K; Gama de Abreu, Marcelo; Pelosi, Paolo; Schultz, Marcus J

2015-10-01

Protective mechanical ventilation with low tidal volumes is standard of care for patients with acute respiratory distress syndrome. The aim of this individual patient data analysis was to determine the association between tidal volume and the occurrence of pulmonary complications in ICU patients without acute respiratory distress syndrome and the association between occurrence of pulmonary complications and outcome in these patients. Individual patient data analysis. ICU patients not fulfilling the consensus criteria for acute respiratory distress syndrome at the onset of ventilation. Mechanical ventilation with low tidal volume. The primary endpoint was development of a composite of acute respiratory distress syndrome and pneumonia during hospital stay. Based on the tertiles of tidal volume size in the first 2 days of ventilation, patients were assigned to a "low tidal volume group" (tidal volumes ≤ 7 mL/kg predicted body weight), an "intermediate tidal volume group" (> 7 and volume group" (≥ 10 mL/kg predicted body weight). Seven investigations (2,184 patients) were included. Acute respiratory distress syndrome or pneumonia occurred in 23% of patients in the low tidal volume group, in 28% of patients in the intermediate tidal volume group, and in 31% of the patients in the high tidal volume group (adjusted odds ratio [low vs high tidal volume group], 0.72; 95% CI, 0.52-0.98; p = 0.042). Occurrence of pulmonary complications was associated with a lower number of ICU-free and hospital-free days and alive at day 28 (10.0 ± 10.9 vs 13.8 ± 11.6 d; p volumes is associated with a lower risk of development of pulmonary complications in patients without acute respiratory distress syndrome.

A Review of Select Centralized Pain Syndromes

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David R. Spiegel

2015-01-01

Full Text Available Pain can be broadly divided into 3 classes, including nociceptive or inflammatory pain (protective, neuropathic (pathological, occurring after damage to the nervous system, or centralized (pathological, due to abnormal function but with no damage or inflammation to the nervous system. The latter has been posited to occur when descending analgesic pathways are attenuated and/or glutamatergic transmission is facilitated. Additionally, this “pain prone phenotype” can be associated with early life trauma and a suboptimal response to opiates. This article will review the relationships between centralized pain syndromes (ie, fibromyalgia, chronic low back pain, childhood sexual abuse, and opiate misuse. Finally, treatment implications, potentially effecting primary care physicians, will be discussed.

High-sensitivity C-reactive protein to detect metabolic syndrome in a centrally obese population: a cross-sectional analysis

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den Engelsen Corine

2012-03-01

Full Text Available Abstract Background People with central obesity have an increased risk for developing the metabolic syndrome, type 2 diabetes and cardiovascular disease. However, a substantial part of obese individuals have no other cardiovascular risk factors, besides their obesity. High sensitivity C-reactive protein (hs-CRP, a marker of systemic inflammation and a predictor of type 2 diabetes and cardiovascular disease, is associated with the metabolic syndrome and its separate components. We evaluated the use of hs-CRP to discriminate between centrally obese people with and without the metabolic syndrome. Methods 1165 people with central obesity but without any previous diagnosis of hypertension, dyslipidemia, diabetes or cardiovascular disease, aged 20-70 years, underwent a physical examination and laboratory assays to determine the presence of the metabolic syndrome (NCEP ATP III criteria. Multivariable linear regression analyses were performed to assess which metabolic syndrome components were independently associated with hs-CRP. A ROC curve was drawn and the area under the curve was calculated to evaluate whether hs-CRP was capable to predict the presence of the metabolic syndrome. Results Median hs-CRP levels were significantly higher in individuals with central obesity with the metabolic syndrome (n = 417; 35.8% compared to individuals with central obesity without the metabolic syndrome (2.2 mg/L (IQR 1.2-4.0 versus 1.7 mg/L (IQR 1.0-3.4; p Conclusions Hs-CRP has limited capacity to predict the presence of the metabolic syndrome in a population with central obesity.

Hantavirus pulmonary syndrome in Anajatuba, Maranhão, Brazil

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MENDES Wellington S.

2001-01-01

Full Text Available The authors report a confirmed case of hantavirus pulmonary syndrome in the rural area of the municipality of Anajatuba, state of Maranhão. Two other suspected cases from the same region are also described. The confirmed case involved a previously healthy young woman who died with signs and symptoms of acute respiratory insufficiency 5 days after presenting fever, myalgia and a dry cough. The patient was a student who was helping her parents with work in the fields; it was a habit of the family to store rice inside the house. The suspected cases involved two first-degree relatives working as field hands who died of acute respiratory insufficiency 24 and 48 hours, respectively, after presenting fever, myalgia and a dry cough. Both stored rice and corn inside their home. People living in the region reported massive infestations with rats in the woods and fields.

Bilateral chylothorax in a patient with chronic central vein thrombosis and chronic thromboembolic pulmonary hypertension

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Avdhesh Bansal

2015-01-01

Full Text Available The chylothorax is not a common presentation, and bilateral chylothorax in patients with chronically high central venous pressure secondary to venous thrombosis is a rare in incidence. We reported a case of bilateral chylothorax in a patient of chronic deep vein thrombosis (DVT in central veins with chronic thromboembolic pulmonary hypertension who presented with 2 weeks history of increased breathlessness, bilateral chest discomfort and weakness. Work-up with chest X-ray and ultrasonography-chest showed gross left sided and mild right sided pleural effusion, thoracocentesis was consistent with chylothorax. Contrast enhanced computed tomography-chest showed multiple collateral formation of left side subclavian vein, venous Doppler showed old DVT in right and left subclavian veins and two-dimensional echocardiogram showed finding of severe pulmonary hypertension. After 24 h of fasting and conservative management, pleural drain became clear and decreased in the amount. Patient′s video assisted thoracoscopic surgery was done, and thoracic duct was ligated and cut down at diaphragmatic level and bilateral talc pleurodesis done. Patient improved clinically and radiologically.

Calcified pulmonary thromboembolism in a child with sickle cell disease: value of multidetector CT in patients with acute chest syndrome

International Nuclear Information System (INIS)

Staser, Jonathan A.; Alam, Tariq; Applegate, Kimberly

2006-01-01

The incidence of pulmonary embolism in children is not clearly known, but is believed to be low. Risk factors for pulmonary thromboembolism include central venous catheter, malignancy, surgery, infection, trauma, and congenital hypercoagulable disorders. Children with sickle cell disease are prothrombotic and are at an increased risk of thromboembolism. The incidence of this event is unknown because these children are often not thoroughly imaged. We report here a case of a calcified pulmonary thromboembolism in a child with sickle cell disease and emphasize the use of multidetector CT in detection of pulmonary thromboembolism in children with sickle cell disease. (orig.)

Paratiroid gland adenoma and single pulmonary fibroma

International Nuclear Information System (INIS)

Aguirre Carpio, Roberto; Jimenez Torres, Victor

2005-01-01

The case of a 62 year old woman with diagnose of parathyroid gland adenoma and single pulmonary fibroma admitted at the ION SOLCA in Quito by the surgery service. At her admission she was classified as a paraneoplasic syndrome associated witha a pulmonary mass + hypercalcemia. This case was presented because of the importance of the primary hyperparathyroidism as one of the principal endocrine pathologies, that in oncology are associated with the multiple neoplasia syndromes. (The author)

Pulmonary manifestations of leptospirosis

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Sameer Gulati

2012-01-01

Full Text Available Leptospirosis has a spectrum of presentation which ranges from mild disease to a severe form comprising of jaundice and renal failure. Involvement of the lung can vary from subtle clinical features to deadly pulmonary hemorrhage and acute respiratory distress syndrome. Of late, it has been identified that leptospirosis can present atypically with predominant pulmonary manifestations. This can delay diagnosis making and hence optimum treatment. The purpose of this review is to bring together all the reported pulmonary manifestations of leptospirosis and the recent trends in the management.

Sleep overlap syndrome

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Fariba Rezaeetalab

2016-12-01

Full Text Available Overlap syndrome, which is known as the coexistence of chronic obstructive pulmonary disease (COPD and obstructive sleep apnea (OSA, was first defined by Flenley. Although it can refer to concomitant occurrence of any of the pulmonary diseases and OSA, overlap syndrome is commonly considered as the coexistence of OSA and COPD. This disease has unique adverse health consequences distinct from either condition alone. Given the high prevalence of each solitary disease, overlap syndrome is also likely to be common and clinically relevant. Despite the fact that overlap syndrome has been described in the literature for nearly 30 years, paucity of evaluations and studies limited the discussion on diagnosis, prevalence, pathophysiology, treatment, and outcomes of this disease. This review article addresses these issues by reviewing several recent studies conducted in Iran or other countries. This review suggests that overlap syndrome has worse outcomes than either disease alone. Our findings accentuated the urgent need for further studies on overlap syndrome and all overlaps between OSA and chronic pulmonary disease to provide a deeper insight into diagnosis and non-invasive treatments of this disease.

Functional pulmonary atresia in newborn with normal intracardiac anatomy: Successful treatment with inhaled nitric oxide and pulmonary vasodilators

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Gürkan Altun

2013-01-01

Full Text Available Functional pulmonary atresia is characterized by a structurally normal pulmonary valve that does not open during right ventricular ejection. It is usually associated with Ebstein′s anomaly, Uhl′s anomaly, neonatal Marfan syndrome and tricuspid valve dysplasia. However, functional pulmonary atresia is rarely reported in newborn with anatomically normal heart. We report a newborn with functional pulmonary atresia who had normal intracardiac anatomy, who responded to treatment with nitric oxide and other vasodilator therapy successfully.

Neurogenic pulmonary edema due to ventriculo-atrial shunt dysfunction: a case report.

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Cruz, Ana Sofia; Menezes, Sónia; Silva, Maria

2016-01-01

Pulmonary edema is caused by the accumulation of fluid within the air spaces and the interstitium of the lung. Neurogenic pulmonary edema is a clinical syndrome characterized by the acute onset of pulmonary edema following a significant central nervous system insult. It may be a less-recognized consequence of raised intracranial pressure due to obstructive hydrocephalus by blocked ventricular shunts. It usually appears within minutes to hours after the injury and has a high mortality rate if not recognized and treated appropriately. We report a patient with acute obstructive hydrocephalus due to ventriculo-atrial shunt dysfunction, proposed to urgent surgery for placement of external ventricular drainage, who presented with neurogenic pulmonary edema preoperatively. She was anesthetized and supportive treatment was instituted. At the end of the procedure the patient showed no clinical signs of respiratory distress, as prompt reduction in intracranial pressure facilitated the regression of the pulmonary edema. This report addresses the importance of recognition of neurogenic pulmonary edema as a possible perioperative complication resulting from an increase in intracranial pressure. If not recognized and treated appropriately, neurogenic pulmonary edema can lead to acute cardiopulmonary failure with global hypoperfusion and hypoxia. Therefore, awareness of and knowledge about the occurrence, clinical presentation and treatment are essential. Copyright © 2013 Sociedade Brasileira de Anestesiologia. Published by Elsevier Editora Ltda. All rights reserved.

Neurogenic pulmonary edema due to ventriculo-atrial shunt dysfunction: a case report

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Ana Sofia Cruz

2016-04-01

Full Text Available ABSTRACT BACKGROUND AND OBJECTIVES: Pulmonary edema is caused by the accumulation of fluid within the air spaces and the interstitium of the lung. Neurogenic pulmonary edema is a clinical syndrome characterized by the acute onset of pulmonary edema following a significant central nervous system insult. It may be a less-recognized consequence of raised intracranial pressure due to obstructive hydrocephalus by blocked ventricular shunts. It usually appears within minutes to hours after the injury and has a high mortality rate if not recognized and treated appropriately. CASE REPORT: We report a patient with acute obstructive hydrocephalus due to ventriculo-atrial shunt dysfunction, proposed to urgent surgery for placement of external ventricular drainage, who presented with neurogenic pulmonary edema preoperatively. She was anesthetized and supportive treatment was instituted. At the end of the procedure the patient showed no clinical signs of respiratory distress, as prompt reduction in intracranial pressure facilitated the regression of the pulmonary edema. CONCLUSIONS: This report addresses the importance of recognition of neurogenic pulmonary edema as a possible perioperative complication resulting from an increase in intracranial pressure. If not recognized and treated appropriately, neurogenic pulmonary edema can lead to acute cardiopulmonary failure with global hypoperfusion and hypoxia. Therefore, awareness of and knowledge about the occurrence, clinical presentation and treatment are essential.

Definition, epidemiology and registries of pulmonary hypertension.

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Awdish, R; Cajigas, H

2016-05-01

Pulmonary arterial hypertension (PAH) is a subcategory of pulmonary hypertension (PH) that comprises a group of disorders with similar pulmonary vascular pathology. Though PH is common, the estimated incidence of IPAH is 1-3 cases per million, making it a rare disease. The hemodynamic definition of PAH is a mean pulmonary artery pressure at rest >OR = 25 mm Hg in the presence of a pulmonary capillary wedge pressure pulmonary vascular resistance (PVR) greater than 3 WU. Specific maneuvers during right heart catheterization can be utilized to disclose vasoreactivity and heart failure with preserved ejection fraction, which have implications for management. The inherent complexity in studying a rare disease that exhibits clinical overlap with a common syndrome necessitated the creation of registries. These registries have been indispensable in the characterization and mapping of the natural history of the disease. Equations and risk calculators derived from registries have given clinicians a basis for risk stratification and prognostication. The sequential accumulation of data since the registries began in the 1980s allows for comparisons to be made. Patients who are differentiated by treatment eras and environments can be contrasted. Variability among inclusion criteria similarly allows for comparisons of these subpopulations. This article provides an overview of available registries, highlights insights provided by each and discusses key issues around the interpretation and extrapolation of data from PAH registries. Registries have allowed us to appreciate the improvement in survival afforded by modern therapy and enhanced detection of this disease. Moving forward, a more global approach to registries is needed, as is enhanced collaboration and centralization.

Noonan′s Syndrome

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Yesudian P Devakar

1995-01-01

Full Text Available Noonan’s syndrome is characterized by hypertelorism, webbed neck, curly or wooly hair, congenital heart defects, micrognathia and low set ears. A 15 year old male presented with features of Noonan’s syndrome with hypertelorism, cryptorchidism, low hairline, large and low set ears, curly hair, pulmonary stenosis and lentigenes. This case is presented to alert the physicians towards the occurrence of lentigenes, characteristic facies and pulmonary stenosis in the syndrome. A few hitherto unreported associations like macrothelia and high arched palate were also seen.

Causes of congenital unilateral pulmonary hypoplasia

International Nuclear Information System (INIS)

Currarino, G.; Williams, B.; Children's Medical Center, Dallas, TX

1985-01-01

A review of the roentgenograms and clinical records of 33 children with primary congenital underdevelopment of one lung showed that 9 patients had simple pulmonary hypoplasia, 8 had anomalous venous return to the right atrium or the inferior vena cava (scimitar syndrome), 7 had an absence of ipsilateral pulmonary artery, 7 had an accessory diaphragm, and 2 had a pulmonary sequestration adjacent to a small diaphragmatic hernia. (orig.)

Saudi Guidelines on the Diagnosis and Treatment of Pulmonary Hypertension: Pulmonary arterial hypertension associated with congenital heart disease

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Antonio Lopes

2014-01-01

Full Text Available Congenital heart disease (CHD with intracardiac/extracardiac shunts is an important etiology of pulmonary arterial hypertension (PAH. The majority of children with congenital cardiac shunts do not develop advanced pulmonary vasculopathy, as surgical repair of the anomalies is now performed early in life. However, if not repaired early, some defects will inevitably lead to pulmonary vascular disease (truncus arteriosus, transposition of the great arteries associated with a ventricular septal defect (VSD, atrioventricular septal defects remarkably in Down syndrome, large, nonrestrictive VSDs, patent ductus arteriosus and related anomalies. The majority of patients are now assigned to surgery based on noninvasive evaluation only. PAH becomes a concern (requiring advanced diagnostic procedures in about 2-10% of them. In adults with CHD, the prevalence of advanced pulmonary vasculopathy (Eisenmenger syndrome is around 4-12%. [1] This article will discuss the diagnostic and management approach for PAH associated with CHD (PAH-CHD.

Sequential occurrence of combined pulmonary fibrosis and emphysema syndrome in a non-smoker female patient.

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Gupta, Pawan; Dash, Devijyoti; Mittal, Richa; Chhabra, Sunil K

2017-05-01

The combined pulmonary fibrosis and emphysema (CPFE) syndrome is a unique and an under-recognized disorder characterized by emphysema in the upper lobes and interstitial fibrosis in the lower lobes of the lung. It occurs predominantly in males and almost exclusively in smokers. This rare combination of a restrictive and an obstructive mechanical defect carries a poorer prognosis than either of the two components. We present a case of CPFE syndrome in a non-smoker female patient who developed lower lobe emphysema subsequent to development of interstitial fibrosis. The case was remarkable for the extreme rarity of several presenting features, namely, a lower lobe occurrence of emphysema subsequent to pre-existent interstitial fibrosis, female gender and absence of a history of smoking. © 2015 John Wiley & Sons Ltd.

Síndrome pulmonar e cardiovascular por hantavírus Hantavirus pulmonary and cardiovascular syndrome

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Mariangela Pimentel Pincelli

2003-10-01

Full Text Available A síndrome pulmonar e cardiovascular por hantavírus é uma doença de conhecimento relativamente recente e freqüentemente fatal, apresentando-se como síndrome do desconforto respiratório agudo. No Brasil, desde o primeiro surto, relatado em novembro/dezembro de 1993, em Juquitiba, 226 casos já foram registrados pela Fundação Nacional da Saúde. A doença afeta indivíduos previamente hígidos, apresentando-se com pródromo febril e sintomas semelhantes aos de um resfriado comum, podendo rapidamente evoluir para edema pulmonar, insuficiência respiratória aguda e choque. A hemoconcentração e a plaquetopenia são comuns da síndrome pulmonar e cardiovascular por hantavírus, e o quadro radiológico típico é de um infiltrado intersticial bilateral difuso, que progride rapidamente para consolidações alveolares, paralelamente à piora do quadro clínico. A mortalidade inicial era em torno de 75% e declinou para aproximadamente 35%, nos últimos anos. Os pacientes que sobrevivem geralmente recuperam-se completamente, cerca de uma semana após o estabelecimento do quadro respiratório. O agente causal, não reconhecido até há pouco, foi identificado como um hantavírus, cujo reservatório natural são animais roedores da família Muridae, subfamília Sigmodontinae. O tratamento específico antiviral ainda não é bem estabelecido, estando em estudo a eficácia de ribavirina. Cuidados de terapia intensiva como ventilação mecânica e monitoramento hemodinâmico invasivo são necessários nas formas mais graves da doença. Essas medidas, se instituídas precocemente, podem melhorar o prognóstico e a sobrevida dos pacientes com síndrome pulmonar e cardiovascular por hantavírus.Hantavirus pulmonary and cardiovascular syndrome is a recently identified and often fatal disease, which presents as acute respiratory distress syndrome (ARDS. Since the first outbreak, in Nov/Dec 1993, in Juquitiba, Brazil, 226 cases have been registered by

Chiari malformation and central sleep apnea syndrome: efficacy of treatment with adaptive servo-ventilation

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Jorge Marques do Vale

2014-10-01

Full Text Available The Chiari malformation type I (CM-I has been associated with sleep-disordered breathing, especially central sleep apnea syndrome. We report the case of a 44-year-old female with CM-I who was referred to our sleep laboratory for suspected sleep apnea. The patient had undergone decompressive surgery 3 years prior. An arterial blood gas analysis showed hypercapnia. Polysomnography showed a respiratory disturbance index of 108 events/h, and all were central apnea events. Treatment with adaptive servo-ventilation was initiated, and central apnea was resolved. This report demonstrates the efficacy of servo-ventilation in the treatment of central sleep apnea syndrome associated with alveolar hypoventilation in a CM-I patient with a history of decompressive surgery.

[Scimitar syndrome. Correlation anatomo-embryological].

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Muñoz-Castellanos, Luis; Kuri-Nivon, Magdalena

2016-01-01

To describe morphologically a toracoabdominal visceral block of a scimitar's syndrome case. We propose a pathogenetic theory wich explains the development of the pulmonary venous connection in this syndrome. The anatomic specimen was described with the segmental sequential system. The situs was solitus, the connections between the cardiac segments and the associated anomalies were determined. The anatomy of both lungs, including the venous pulmonary connection, was described. A pathogenetic hypothesis was made, which explains the pulmonary venous connection throw a correlation between the pathology of this syndrome and the normal development of the pulmonary veins. The situs was solitus, the connections of the cardiac chambers were normal; there were hypoplasia and dysplasia of the right lung with sequestration of the inferior lobe; the right pulmonary veins were connected with a curved collector which drainaged into the suprahepatic segment of the inferior vena cava; the left pulmonary veins were open into the left atrium. The sequestered inferior lobe of the right lung received irrigation throw a collateral aortopulmonary vessel. There was an atrial septal defect. The pathogenetic hypothesis propose that the pulmonary venous connection in this syndrome represent the persistent of the Streeter's horizon xiv (28-30 days of development), period in which the sinus of the pulmonary veins has double connection, with the left atrium and with a primitive collector into the right viteline vein which forms the suprahepatic segment of the inferior vena cava. Copyright © 2015 Instituto Nacional de Cardiología Ignacio Chávez. Published by Masson Doyma México S.A. All rights reserved.

Congenital central hypoventilation syndrome: four families.

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Trivedi, Amit; Waters, Karen; Suresh, Sadasivam; Nair, Rashmi

2011-12-01

Congenital central hypoventilation syndrome (CCHS) is a rare condition that usually presents soon after birth and is potentially life-shortening if not treated. The defining abnormality is hypoventilation during sleep which requires life-long treatment with artificial ventilation. This syndrome may also be associated with generalised dysfunction of the autonomic nervous system and a sub-group with associated Hirschsprung's disease. The genetic basis of CCHS has been identified as mutations in the PHOX2B gene. We present four families, three with autosomal dominant inheritance and familial clustering, and one with a de novo mutation resulting in CCHS. We demonstrate that nasal mask ventilation from birth can provide adequate treatment and improved quality of life for these children. Phenotypic variability in expression of disease is seen in families with the same mutations in PHOX2B gene. The psychosocial costs of the disease and the unrecognised 'morbidity barter' that is part of current management needs to be factored into in all stages of management from childhood to adolescence to adulthood.

Retrospective review of neonates with persistent pulmonary ...

African Journals Online (AJOL)

Background. Persistent pulmonary hypertension of the newborn (PPHN) is a clinical syndrome characterised by high pulmonary pressures, low systemic pressures and severe hypoxaemia due to circulation transition failure after birth. Objective. To determine the incidence of and describe the risk factors, infant ...

Metabolic Syndrome in a Rural Nigerian Community: Is Central ...

African Journals Online (AJOL)

Alasia Datonye

ABSTRACT. Background. Metabolic syndrome (MS) is primarily the consequence of excess central adiposity but can also result from low grade systemic inflammation inducing insulin resistance. There is a global increase in the prevalence of MS; it is on this background that evaluation of the prevalence of MS in a poor rural ...

Central Nervous System (CNS Disease Triggering Takotsubo Syndrome

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Josef Finsterer

2016-01-01

Full Text Available Takotsubo syndrome (TTS is usually triggered by psychological or physical stress. One of the many physical sources of stress are central nervous system (CNS disorders. CNS disorders most frequently triggering TTS include subarachnoid bleeding, epilepsy, ischemic stroke, migraine, and intracerebral bleeding. More rare CNS-triggers of TTS include posterior reversible encephalopathy syndrome (PRES, amyotrophic lateral sclerosis, encephalitis, or traumatic brain or spinal cord injury. TTS triggered by any of the CNS disorders needs to be recognized since adequate treatment of TTS may improve the general outcome from the CNS disorder as well. Neurologists need to be aware of TTS as a complication of specific CNS disorders but TTS may be triggered also by CNS disorders so far not recognised as causes of TTS.

Differences in the effects of Asian dust on pulmonary function between adult patients with asthma and those with asthma–chronic obstructive pulmonary disease overlap syndrome

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Watanabe, Masanari; Noma, Hisashi; Kurai, Jun; Sano, Hiroyuki; Ueda, Yasuto; Mikami, Masaaki; Yamamoto, Hiroyuki; Tokuyasu, Hirokazu; Kato, Kazuhiro; Konishi, Tatsuya; Tatsukawa, Toshiyuki; Shimizu, Eiji; Kitano, Hiroya

2016-01-01

Background Asian dust (AD) exposure exacerbates pulmonary dysfunction in patients with asthma. Asthma–chronic obstructive pulmonary disease overlap syndrome (ACOS), characterized by coexisting symptoms of asthma and chronic obstructive pulmonary disease, is considered a separate disease entity. Previously, we investigated the effects of AD on pulmonary function in adult patients with asthma. Here, we present the findings of our further research on the differences in the effects of AD exposure on pulmonary function between patients with asthma alone and those with ACOS. Methods Between March and May 2012, we conducted a panel study wherein we monitored daily peak expiratory flow (PEF) values in 231 adult patients with asthma. These patients were divided into 190 patients with asthma alone and 41 patients with ACOS in this study. Daily AD particle levels were measured using light detection and ranging systems. Two heavy AD days (April 23 and 24) were determined according to the Japan Meteorological Agency definition. A linear mixed model was used to estimate the association between PEF and AD exposure. Results Increments in the interquartile range of AD particles (0.018 km−1) led to PEF changes of −0.50 L/min (95% confidence interval, −0.98 to −0.02) in patients with asthma alone and −0.11 L/min (−0.11 to 0.85) in patients with ACOS. The PEF changes after exposure to heavy AD were −2.21 L/min (−4.28 to −0.15) in patients with asthma alone and −2.76 L/min (−6.86 to 1.35) in patients with ACOS. In patients with asthma alone, the highest decrease in PEF values was observed on the heavy AD day, with a subsequent gradual increase over time. Conclusion Our results suggest that the effects of AD exposure on pulmonary function differ between patients with asthma alone and ACOS, with the former exhibiting a greater likelihood of decreased pulmonary function after AD exposure. PMID:26869784

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome and pulmonary thromboembolism: an overlooked concomitance

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Vilma Takayasu

2013-06-01

Full Text Available The Eosinophilic Granulomatosis with Polyangiitis (formerly Churg- Strauss Syndrome (EGPA is a systemic inflammatory disease characterized by the presence of rhinitis, asthma, peripheral eosinophilia, and vasculitis—the latter being characteristic of the late stage of the disease. After several years from the onset of the disease, small- and medium-sized vessel vasculitis ensues, undertaking various organs and systems. Upper and lower airways, skin, nervous system, gastrointestinal tract, heart, and kidneys are the most commonly involved organs. It is believed that tissue injury is the result of processes mediated by antineutrophil cytoplasmic antibody (ANCA, or toxic mediators released by eosinophils. Although it is classified as ANCA-associated vasculitis, these autoantibodies are present in only 40% of cases. The authors report the case of a patient with EGPA, who had a history of asthma, peripheral and central neuropathy, palpable purpura, gastrointestinal micro perforation, peripheral eosinophilia, and the presence of myeloperoxidase-antineutrophil cytoplasmic antibody. Inflammatory parameters improved after the initiation of treatment, but 1 month after hospital discharge the patient developed symptoms compatible with pulmonary embolism and died. Thrombophilia that occurs in EGPA is due to the interaction between the inflammatory response and eosinophilia with the clotting system resulting in a pro-thrombotic state. Although not yet well-determined, the authors call attention to the possibility of the impact of thromboembolic events on the prognosis of patients with EGPA. In addition to the adequate immunosuppressive treatment, prophylaxis and treatment for thrombosis should never be overlooked.

Non-infective pulmonary disease in HIV-positive children

International Nuclear Information System (INIS)

Theron, Salomine; Andronikou, Savvas; George, Reena; Plessis, Jaco du; Hayes, Murray; Mapukata, Ayanda; Goussard, Pierre; Gie, Robert

2009-01-01

It is estimated that over 90% of children infected with human immunodeficiency virus (HIV) live in the developing world and particularly in sub-Saharan Africa. Pulmonary disease is the most common clinical feature of acquired immunodeficiency syndrome (AIDS) in infants and children causing the most morbidity and mortality, and is the primary cause of death in 50% of cases. Children with lung disease are surviving progressively longer because of earlier diagnosis and antiretroviral treatment and, therefore, thoracic manifestations have continued to change and unexpected complications are being encountered. It has been reported that 33% of HIV-positive children have chronic changes on chest radiographs by the age of 4 years. Lymphocytic interstitial pneumonitis is common in the paediatric HIV population and is responsible for 30-40% of pulmonary disease. HIV-positive children also have a higher incidence of pulmonary malignancies, including lymphoma and pulmonary Kaposi sarcoma. Immune reconstitution inflammatory syndrome is seen after highly active antiretroviral treatment. Complications of pulmonary infections, aspiration and rarely interstitial pneumonitis are also seen. This review focuses on the imaging findings of non-infective chronic pulmonary disease. (orig.)

Rare association of central pontine myelinolysis with infantile tremor syndrome

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Kalpana Datta

2012-01-01

Full Text Available Central pontine myelinolysis (CPM is an acute demyelination within the central basis pontis. Though exact mechanism is not known it is seen commonly with rapid correction of hyponatremia and also with pontine ischemia or infarction, demyelinating diseases, pontine neoplasm and different metabolic diseases. We report a rare association of CPM in a patient of Infantile Tremor Syndrom (ITS. ITS is a syndrome of tremor, mental and physical retardation, pigmentary changes of hair and skin and anemia in malnourished children. Though first reported in Indian subcontinent many identical cases were reported from around the world. Our case is a 15 month old child with generalized tremor, mild hepatosplenomegaly with features of grade II malnutrition including skin and hair changes. All the signs and symtoms of tremor improved after treatment with the World Health Organization (WHO protocol for protein energy malnutrition (PEM and administration of propranolol without any side effects.

Combined Pulmonary Fibrosis and Emphysema Syndrome: A New Phenotype within the Spectrum of Smoking-Related Interstitial Lung Disease

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Karina Portillo

2012-01-01

Full Text Available Combined pulmonary fibrosis and emphysema (CPFE is a recently defined syndrome, in which centrilobular and/or paraseptal emphysemas in upper lung zones coexist with pulmonary fibrosis in lower lobes in individuals. These patients have a characteristic lung function profile, with unexpected subnormal dynamic and static lung volumes, contrasting with a significant reduction of carbon monoxide transfer (DLco and exercise hypoxemia. Pulmonary hypertension is highly prevalent in CPFE and is the leading determinant of death. Tobacco smoking has been proposed as the main factor in its etiology, though the pathophysiology and its natural history remain to be determined. High-resolution computed axial tomography is the mandatory tool to confirm the diagnosis. Currently, there is no consensus about its treatment since those published to date on this issue are limited to well-characterised series of cases; hence, a better understanding of this entity may help in the development of future therapeutic approaches.

Masquerade Syndrome of Multicentre Primary Central Nervous System Lymphoma

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Silvana Guerriero

2011-01-01

Full Text Available Purpose. In Italy we say that the most unlucky things can happen to physicians when they get sick, despite the attention of colleagues. To confirm this rumor, we report the sad story of a surgeon with bilateral vitreitis and glaucoma unresponsive to traditional therapies. Methods/Design. Case report. Results. After one year of steroidal and immunosuppressive therapy, a vitrectomy, and a trabeculectomy for unresponsive bilateral vitreitis and glaucoma, MRI showed a multicentre primary central nervous system lymphoma, which was the underlying cause of the masquerade syndrome. Conclusions. All ophthalmologists and clinicians must be aware of masquerade syndromes, in order to avoid delays in diagnosis.

Pulmonary autoimmunity as a feature of autoimmune polyendocrine syndrome type 1 and identification of KCNRG as a bronchial autoantigen.

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Alimohammadi, Mohammad; Dubois, Noémie; Sköldberg, Filip; Hallgren, Asa; Tardivel, Isabelle; Hedstrand, Håkan; Haavik, Jan; Husebye, Eystein S; Gustafsson, Jan; Rorsman, Fredrik; Meloni, Antonella; Janson, Christer; Vialettes, Bernard; Kajosaari, Merja; Egner, William; Sargur, Ravishankar; Pontén, Fredrik; Amoura, Zahir; Grimfeld, Alain; De Luca, Filippo; Betterle, Corrado; Perheentupa, Jaakko; Kämpe, Olle; Carel, Jean-Claude

2009-03-17

Patients with autoimmune polyendocrine syndrome type 1 (APS-1) suffer from multiple organ-specific autoimmunity with autoantibodies against target tissue-specific autoantigens. Endocrine and nonendocrine organs such as skin, hair follicles, and liver are targeted by the immune system. Despite sporadic observations of pulmonary symptoms among APS-1 patients, an autoimmune mechanism for pulmonary involvement has not been elucidated. We report here on a subset of APS-1 patients with respiratory symptoms. Eight patients with pulmonary involvement were identified. Severe airway obstruction was found in 4 patients, leading to death in 2. Immunoscreening of a cDNA library using serum samples from a patient with APS-1 and obstructive respiratory symptoms identified a putative potassium channel regulator (KCNRG) as a pulmonary autoantigen. Reactivity to recombinant KCNRG was assessed in 110 APS-1 patients by using immunoprecipitation. Autoantibodies to KCNRG were present in 7 of the 8 patients with respiratory symptoms, but in only 1 of 102 APS-1 patients without respiratory symptoms. Expression of KCNRG messenger RNA and protein was found to be predominantly restricted to the epithelial cells of terminal bronchioles. Autoantibodies to KCNRG, a protein mainly expressed in bronchial epithelium, are strongly associated with pulmonary involvement in APS-1. These findings may facilitate the recognition, diagnosis, characterization, and understanding of the pulmonary manifestations of APS-1.

Impaired Central Pulsatile Hemodynamics in Children and Adolescents With Marfan Syndrome.

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Grillo, Andrea; Salvi, Paolo; Marelli, Susan; Gao, Lan; Salvi, Lucia; Faini, Andrea; Trifirò, Giuliana; Carretta, Renzo; Pini, Alessandro; Parati, Gianfranco

2017-11-07

Marfan syndrome is characterized by aortic root dilation, beginning in childhood. Data about aortic pulsatile hemodynamics and stiffness in pediatric age are currently lacking. In 51 young patients with Marfan syndrome (12.0±3.3 years), carotid tonometry was performed for the measurement of central pulse pressure, pulse pressure amplification, and aortic stiffness (carotid-femoral pulse wave velocity). Patients underwent an echocardiogram at baseline and at 1 year follow-up and a genetic evaluation. Pathogenetic fibrillin-1 mutations were classified between "dominant negative" and "haploinsufficient." The hemodynamic parameters of patients were compared with those of 80 sex, age, blood pressure, and heart-rate matched controls. Central pulse pressure was significantly higher (38.3±12.3 versus 33.6±7.8 mm Hg; P =0.009), and pulse pressure amplification was significantly reduced in Marfan than controls (17.9±15.3% versus 32.3±17.4%; P Marfan and controls (4.98±1.00 versus 4.75±0.67 m/s). In the Marfan group, central pulse pressure and pulse pressure amplification were independently associated with aortic diameter at the sinuses of Valsalva (respectively, β=0.371, P =0.010; β=-0.271, P =0.026). No significant difference in hemodynamic parameters was found according to fibrillin-1 genotype. Patients who increased aortic Z-scores at 1-year follow-up presented a higher central pulse pressure than the remaining (42.7±14.2 versus 32.3±5.9 mm Hg; P =0.004). Central pulse pressure and pulse pressure amplification were impaired in pediatric Marfan syndrome, and associated with aortic root diameters, whereas aortic pulse wave velocity was similar to that of a general pediatric population. An increased central pulse pressure was present among patients whose aortic dilatation worsened at 1-year follow-up. © 2017 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley.

PHOX2B mutation-confirmed congenital central hypoventilation syndrome in a Chinese family: presentation from newborn to adulthood.

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Lee, Peilin; Su, Yi-Ning; Yu, Chong-Jen; Yang, Pan-Chyr; Wu, Huey-Dong

2009-02-01

Congenital central hypoventilation syndrome (CCHS) is characterized by compromised chemoreflexes resulting in sleep hypoventilation. We report a Chinese family with paired-like homeobox 2B (PHOX2B) mutation-confirmed CCHS, with a clinical spectrum from newborn to adulthood, to increase awareness of its various manifestations. After identifying central hypoventilation in an adult man (index case), clinical evaluation was performed on the complete family, which consisted of the parents, five siblings, and five offspring. Pulmonary function tests, overnight polysomnography, arterial blood gas measurements, hypercapnia ventilatory response, and PHOX2B gene mutation screening were performed on living family members. Brain MRI, 24-h Holter monitoring, and echocardiography were performed on members with clinically diagnosed central hypoventilation. The index patient and four offspring manifested clinical features of central hypoventilation. The index patients had hypoxia and hypercapnia while awake, polycythemia, and hematocrit levels of 70%. The first and fourth children had frequent cyanotic spells, and both died of respiratory failure. The second and third children remained asymptomatic until adulthood, when they experienced impaired hypercapnic ventilatory response. The third child had nocturnal hypoventilation with nadir pulse oximetric saturation of 59%. Adult-onset CCHS with PHOX2B gene mutation of the + 5 alanine expansions were confirmed in the index patient and the second and third children. The index patient and the third child received ventilator support system bilevel positive airway pressure treatment, which improved the hypoxemia, hypercapnia, and polycythemia without altering their chemosensitivity. Transmission of late-onset CCHS is autosomal-dominant. Genetic screening of family members of CCHS probands allows for early diagnosis and treatment.

Nociceptive transmission and modulation via P2X receptors in central pain syndrome.

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Kuan, Yung-Hui; Shyu, Bai-Chuang

2016-05-26

Painful sensations are some of the most frequent complaints of patients who are admitted to local medical clinics. Persistent pain varies according to its causes, often resulting from local tissue damage or inflammation. Central somatosensory pathway lesions that are not adequately relieved can consequently cause central pain syndrome or central neuropathic pain. Research on the molecular mechanisms that underlie this pathogenesis is important for treating such pain. To date, evidence suggests the involvement of ion channels, including adenosine triphosphate (ATP)-gated cation channel P2X receptors, in central nervous system pain transmission and persistent modulation upon and following the occurrence of neuropathic pain. Several P2X receptor subtypes, including P2X2, P2X3, P2X4, and P2X7, have been shown to play diverse roles in the pathogenesis of central pain including the mediation of fast transmission in the peripheral nervous system and modulation of neuronal activity in the central nervous system. This review article highlights the role of the P2X family of ATP receptors in the pathogenesis of central neuropathic pain and pain transmission. We discuss basic research that may be translated to clinical application, suggesting that P2X receptors may be treatment targets for central pain syndrome.

MRI of surgically created pulmonary artery narrowing in the dog

Energy Technology Data Exchange (ETDEWEB)

Hernandez, R.J.; Rocchini, A.P.; Bove, E.L.; Chenevert, T.L.; Gubin, B. (Michigan Univ., Ann Arbor (USA). Dept. of Radiology)

1989-11-01

Narrowing of the pulmonary arteries was created surgically in twelve dogs. In six of the dogs the narrowing was central (main pulmonary artery), and in the remaining six the narrowing was located peripherally at the hilar level of the right pulmonary artery beyond the pericardial reflection. MRI and angiography were performed in all dogs. MRI clearly delineated the site of the pulmonary band and the caliber of the pulmonary artery at the site of the band in all dogs (N=6). MRI was not able to visualize any of the stenosis of the right pulmonary arteries at the hila, beyond the pericardial reflection. In addition, optimal imaging planes to depict each segment of the central pulmonary arteries were determined. The capability to image in oblique planes is essential in evaluating the morphology of the central pulmonary arteries. (orig.).

MRI of surgically created pulmonary artery narrowing in the dog

International Nuclear Information System (INIS)

Hernandez, R.J.; Rocchini, A.P.; Bove, E.L.; Chenevert, T.L.; Gubin, B.

1989-01-01

Narrowing of the pulmonary arteries was created surgically in twelve dogs. In six of the dogs the narrowing was central (main pulmonary artery), and in the remaining six the narrowing was located peripherally at the hilar level of the right pulmonary artery beyond the pericardial reflection. MRI and angiography were performed in all dogs. MRI clearly delineated the site of the pulmonary band and the caliber of the pulmonary artery at the site of the band in all dogs (N=6). MRI was not able to visualize any of the stenosis of the right pulmonary arteries at the hila, beyond the pericardial reflection. In addition, optimal imaging planes to depict each segment of the central pulmonary arteries were determined. The capability to image in oblique planes is essential in evaluating the morphology of the central pulmonary arteries. (orig.)

Prevention and treatment of the post-thrombotic syndrome and of the chronic thromboembolic pulmonary hypertension.

Science.gov (United States)

Pesavento, Raffaele; Prandoni, Paolo

2015-02-01

Post-thrombotic syndrome (PTS) and chronic thromboembolic pulmonary hypertension (CTEPH) are late complications of venous thromboembolism. The purpose of this review is to present and discuss recently published studies that have improved our knowledge of PTS and CTEPH. The current understanding of the pathophysiology of PTS and CTEPH is discussed as well as the importance of chronic residual venous thrombosis, some polymorphisms of plasminogen activator inhibitor-1, and the current concept of misguided thrombus resolution. The surprising finding that elastic compression stockings may not be effective in preventing PTS and the novel medical treatment in CTEPH are discussed in detail. Novel direct oral anticoagulants show potential for prevention of PTS. No firm conclusions can be drawn on the efficacy of elastic stockings. Novel treatments of CTEPH for inoperable patients and those with persistent pulmonary hypertension after surgery have become available and further research on wider indication for their use is urgently needed.

Rare case of Primary Pulmonary Extranodal Non-Hodgkin’s Lymphoma in a Patient with Sjogrens Syndrome: Role of FDG-PET/CT in the Initial Staging and Evaluating Response to Treatment

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Gonca G. Bural

2012-12-01

Full Text Available A 64-year old woman with a long standing Sjogren’s syndrome was undergoing evaluation for renal transplant surgery when two pulmonary opacities were detected on chest CT. Subsequent biopsy revealed extranodal marginal B-cell non-Hodgkin’s lymphoma (NHL. An FDG-PET/CT scan was then performed which demonstrated isolated FDG avid pulmonary involvement. After therapy, FDG-PET/CT scans showed good response to treatment with near complete resolution of FDG avidity. This rare case illustrates the rare pulmonary manifestation of extranodal lymphoma in a patient with Sjogren’s syndrome and emphasizes the value of FDG PET/CT in the initial staging and evaluation of response to treatment, which has not previously been published. (MIRT 2012;21:117-120

Dyspnoea in-patient with antiphospholipid syndrome; case Presentation and literature revision

International Nuclear Information System (INIS)

Restrepo, Lucas; Londono, Maria Carlota; Anaya, Juan Manuel

2001-01-01

The antiphospholipid syndrome (AFS) is characterized by vascular thrombosis and/or pregnancy morbidity associated to presence of antiphospholipid antibodies (anticardiolipin and/or lupus anticoagulant), This syndrome may occur alone primary -PAFS-) or in association with systemic lupus erythematosus (secondary -SAFS, Both primary and secondary AFS can be responsible for systemic manifestations other than vascular, among them pulmonary, Here is presented a patient with dyspnoea due to pulmonary hypertension (PHT) and interstitial pulmonary disease (IPD), in whom a diagnostic of SAFS was done, The pulmonary manifestations of the AFS are revised including pulmonary thromboembolism, PHT, IPD, pulmonary hemorrhage, and adult respiratory syndrome with multisystemic failure

Endocrine and anatomical findings in a case of Solitary Median Maxillary Central Incisor Syndrome

NARCIS (Netherlands)

Szakszon, Katalin; Felszeghy, Enikő; Csízy, István; Józsa, Tamás; Káposzta, Rita; Balogh, Erzsébet; Oláh, Eva; Balogh, István; Berényi, Ervin; Knegt, Alida C.; Ilyés, István

2012-01-01

Solitary Median Maxillary Central Incisor Syndrome (SMMCI) is a rare malformation syndrome consisting of multiple, mainly midline defects. Some authors suggest that it is a mild manifestation of the wide spectrum of holoprosencephaly, others classify it rather as a distinct entity. Authors report a

Rarity of isolated pulmonary embolism and acute aortic syndrome occurring outside of the field of view of dedicated coronary CT angiography

International Nuclear Information System (INIS)

Lee, Hwa Yeon; Song, In Sup; Yoo, Seung Min; Rho, Ji Young; Moon, Jae Youn; Kim, In Jai; Lim, Sang Wook; Sung, Jung Hoon; Cha, Dong Hun; White, Charles S.

2011-01-01

Background Although triple rule-out CT angiography (TRO) to simultaneously evaluate acute coronary syndrome (ACS), pulmonary embolism (PE), and acute aortic syndrome (AAS) is increasingly used in many institutions, TRO is inevitably associated with increased radiation exposure due to extended z-axis coverage compared with dedicated coronary CT angiography (DCTA). Purpose To determine the frequency of exclusion of findings of AAS, PE, and significant incidental non-cardiac pathology that may be the cause of acute chest pain when using a restricted DCTA field of view (FOV). Material and Methods We retrospectively reviewed CT images and charts of 103 patients with acute PE and 50 patients with AAS. Either non-ECG gated dedicated pulmonary or aortic CT angiography was performed using 16- or 64-slice multidetector CT (MDCT). We analyzed the incidence of isolated PE, AAS, or significant non-cardiac pathology outside of DCTA FOV (i.e. from tracheal carina to the base of heart). Results There were two cases of isolated PE (2/103, 1.9%) excluded from the FOV of DCTA. One case of PE was isolated to the subsegmental pulmonary artery in the posterior segment of the right upper lobe. In the second case, pulmonary embolism in the left main pulmonary artery was located out of the FOV of DCTA because the left main pulmonary artery was retracted upwardly by fibrotic scar in the left upper lobe due to prior tuberculosis. There was no case of AAS and significant non-cardiac pathology excluded from the FOV of DCTA. AAS (n = 50) consisted of penetrating atherosclerotic ulcer (n = 7), intramural hematoma (n = 5) and aortic dissection (n = 38). Conclusion As isolated PE, AAS, and significant non-cardiac pathology outside of the DCTA FOV rarely occur, DCTA may replace TRO in the evaluation of patients with non-specific acute chest pain and a low pre-test probability of PE or aortic dissection

Rarity of isolated pulmonary embolism and acute aortic syndrome occurring outside of the field of view of dedicated coronary CT angiography

Energy Technology Data Exchange (ETDEWEB)

Lee, Hwa Yeon; Song, In Sup (Dept. of Diagnostic Radiology Chung-Ang Univ. College of Medicine, Seoul (Korea, Republic of)); Yoo, Seung Min; Rho, Ji Young (Dept. of Diagnostic Radiology CHA Medical Univ. Hospital, Bundang (Korea, Republic of)), email: smyoo68@hanmail.net; Moon, Jae Youn; Kim, In Jai; Lim, Sang Wook; Sung, Jung Hoon; Cha, Dong Hun (Dept. of Cardiology CHA Medical Univ. Hospital, Bundang (Korea, Republic of)); White, Charles S. (Dept. of Diagnostic Radiology Univ. of Maryland, Baltimore (United States))

2011-05-15

Background Although triple rule-out CT angiography (TRO) to simultaneously evaluate acute coronary syndrome (ACS), pulmonary embolism (PE), and acute aortic syndrome (AAS) is increasingly used in many institutions, TRO is inevitably associated with increased radiation exposure due to extended z-axis coverage compared with dedicated coronary CT angiography (DCTA). Purpose To determine the frequency of exclusion of findings of AAS, PE, and significant incidental non-cardiac pathology that may be the cause of acute chest pain when using a restricted DCTA field of view (FOV). Material and Methods We retrospectively reviewed CT images and charts of 103 patients with acute PE and 50 patients with AAS. Either non-ECG gated dedicated pulmonary or aortic CT angiography was performed using 16- or 64-slice multidetector CT (MDCT). We analyzed the incidence of isolated PE, AAS, or significant non-cardiac pathology outside of DCTA FOV (i.e. from tracheal carina to the base of heart). Results There were two cases of isolated PE (2/103, 1.9%) excluded from the FOV of DCTA. One case of PE was isolated to the subsegmental pulmonary artery in the posterior segment of the right upper lobe. In the second case, pulmonary embolism in the left main pulmonary artery was located out of the FOV of DCTA because the left main pulmonary artery was retracted upwardly by fibrotic scar in the left upper lobe due to prior tuberculosis. There was no case of AAS and significant non-cardiac pathology excluded from the FOV of DCTA. AAS (n = 50) consisted of penetrating atherosclerotic ulcer (n = 7), intramural hematoma (n = 5) and aortic dissection (n = 38). Conclusion As isolated PE, AAS, and significant non-cardiac pathology outside of the DCTA FOV rarely occur, DCTA may replace TRO in the evaluation of patients with non-specific acute chest pain and a low pre-test probability of PE or aortic dissection

Central nervous system involvement in primary Sjogren`s syndrome manifesting as multiple sclerosis.

Science.gov (United States)

Liu, Jing-Yao; Zhao, Teng; Zhou, Chun-Kui

2014-04-01

Central nervous system symptoms in patients with primary Sjogren`s syndrome are rare. They can present as extraglandular manifestations and require a differential diagnosis from multiple sclerosis. Due to a variety of presentations, Sjogren`s syndrome with neurologic involvement may be difficult to diagnose. Here, we report a case of a 75-year-old woman who was first diagnosed with multiple sclerosis in 2010, but who was subsequently diagnosed with primary Sjogren`s syndrome 2 years later after showing signs of atypical neurologic manifestations. Therefore, primary Sjogren`s syndrome should be suspected in patients who present with atypical clinical and radiologic neurologic manifestations.

Adenoviral transfer of HSP-70 into pulmonary epithelium ameliorates experimental acute respiratory distress syndrome.

Science.gov (United States)

Weiss, Yoram G; Maloyan, Alina; Tazelaar, John; Raj, Nichelle; Deutschman, Clifford S

2002-09-01

The acute respiratory distress syndrome (ARDS) provokes three pathologic processes: unchecked inflammation, interstitial/alveolar protein accumulation, and destruction of pulmonary epithelial cells. The highly conserved heat shock protein HSP-70 can limit all three responses but is not appropriately expressed in the lungs after cecal ligation and double puncture (2CLP), a clinically relevant model of ARDS. We hypothesize that restoring expression of HSP-70 using adenovirus-mediated gene therapy will limit pulmonary pathology following 2CLP. We administered a vector containing the porcine HSP-70 cDNA driven by a CMV promoter (AdHSP) into the lungs of rats subjected to 2CLP or sham operation. Administration of AdHSP after either sham operation or 2CLP increased HSP-70 protein expression in lung tissue, as determined by immunohistochemistry and Western blot hybridization. Administration of AdHSP significantly attenuated interstitial and alveolar edema and protein exudation and dramatically decreased neutrophil accumulation, relative to a control adenovirus. CLP-associated mortality at 48 hours was reduced by half. Modulation of HSP-70 production reduces pathologic changes and may improve outcome in experimental ARDS.

TINF2 Gene Mutation in a Patient with Pulmonary Fibrosis

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T. W. Hoffman

2016-01-01

Full Text Available Pulmonary fibrosis is a frequent manifestation of telomere syndromes. Telomere gene mutations are found in up to 25% and 3% of patients with familial disease and sporadic disease, respectively. The telomere gene TINF2 encodes an eponymous protein that is part of the shelterin complex, a complex involved in telomere protection and maintenance. A TINF2 gene mutation was recently reported in a family with pulmonary fibrosis. We identified a heterozygous Ser245Tyr mutation in the TINF2 gene of previously healthy female patient that presented with progressive cough due to pulmonary fibrosis as well as panhypogammaglobulinemia at age 52. Retrospective multidisciplinary evaluation classified her as a case of possible idiopathic pulmonary fibrosis. Telomere length-measurement indicated normal telomere length in the peripheral blood compartment. This is the first report of a TINF2 mutation in a patient with sporadic pulmonary fibrosis, which represents another association between TINF2 mutations and this disease. Furthermore, this case underlines the importance of telomere dysfunction and not telomere length alone in telomere syndromes and draws attention to hypogammaglobulinemia as a manifestation of telomere syndromes.

Reduction of central neuropathic pain with ketamine infusion in a patient with Ehlers–Danlos syndrome: a case report

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Lo TC

2016-09-01

Full Text Available Tony Chung Tung Lo,1,* Stephen Tung Yeung,2,* Sujin Lee,1 Kira Skavinski,3 Solomon Liao,4 1Department of Physical Medicine and Rehabilitation, University of California Irvine, Orange, CA, 2Department of Immunology, University of Connecticut School of Medicine, Farmington, CT, 3Department of Palliative Medicine, University of California San Diego, La Jolla, 4Department of Palliative Medicine, University of California Irvine, Orange, CA, USA *These authors contributed equally to this work Objective: Ehlers–Danlos syndrome frequently causes acute and chronic pain because of joint subluxations and dislocations secondary to hypermobility. Current treatments for pain related to Ehlers–Danlos syndrome and central pain syndrome are inadequate. This case report discusses the therapeutic use of ketamine intravenous infusion as an alternative. Case report: A 27-year-old Caucasian female with a history of Ehlers–Danlos syndrome and spinal cord ischemic myelopathy resulting in central pain syndrome, presented with severe generalized body pain refractory to multiple pharmacological interventions. After a 7-day course of ketamine intravenous infusion under controlled generalized sedation in the intensive care unit, the patient reported a dramatic reduction in pain levels from 7–8 out of 10 to 0–3 out of 10 on a numeric rating scale and had a significant functional improvement. The patient tolerated a reduction in her pain medication regimen, which originally included opioids, gabapentin, pregabalin, tricyclic antidepressants, and nonsteroidal anti-inflammatory drugs. Conclusion: Ketamine infusion treatment has been used in various pain syndromes, including central neuropathic pain, ischemic pain, and regional pain syndrome. Reports have suggested that ketamine modulates pain by the regression of N-methyl-D-aspartate receptor to a resting state. As such, propagation of nociceptive signal to brain is interrupted allowing for the restoration of

Pulmonary complications in neurosurgical patients

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Randeep Guleria

2012-01-01

Full Text Available Pulmonary complications are a major cause of morbidity and mortality in neurosurgical patients. The common pulmonary complications in neurosurgical patients include pneumonia, postoperative atelectasis, respiratory failure, pulmonary embolism, and neurogenic pulmonary edema. Postoperative lung expansion strategies have been shown to be useful in prevention of the postoperative complications in surgical patients. Low tidal volume ventilation should be used in patients who develop acute respiratory distress syndrome. An antibiotic use policy should be put in practice depending on the local patterns of antimicrobial resistance in the hospital. Thromboprophylactic strategies should be used in nonambulatory patients. Meticulous attention should be paid to infection control with a special emphasis on hand-washing practices. Prevention and timely management of these complications can help to decrease the morbidity and mortality associated with pulmonary complications.

Pulmonary Hypertension in Congenital Heart Disease: Beyond Eisenmenger Syndrome.

Science.gov (United States)

Krieger, Eric V; Leary, Peter J; Opotowsky, Alexander R

2015-11-01

Patients with adult congenital heart disease have an increased risk of developing pulmonary hypertension. There are several mechanisms of pulmonary hypertension in patients with adult congenital heart disease, and understanding them requires a systematic approach to define the patient's hemodynamics and physiology. This article reviews the updated classification of pulmonary hypertension in patients with adult congenital heart disease with a focus on pathophysiology, diagnostics, and the evaluation of pulmonary hypertension in special adult congenital heart disease populations. Copyright © 2015 Elsevier Inc. All rights reserved.

Diffuse Pulmonary Hemorrhage: Classification, physiopathology and Radiologic Manifestations

International Nuclear Information System (INIS)

Carrillo Bayona, Jorge Alberto; Quintana, Jose; Ortiz Ruiz, Guillermo

2008-01-01

Diffuse pulmonary hemorrhage is a syndrome characterized by hemoptysis, anemia and alveolar opacities in the chest radiograph (Fig. 1). Differential diagnoses include all entities with focal or diffuse alveolar opacities. In this article we review the most important causes of diffuse pulmonary hemorrhage.

Application of transpulmonary thermodilution monitoring (PiCCO in patient with neurogenic pulmonary edema and acute obstructive hydrocephalus due to a central neurocytoma: A case report

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Ryoichi Iwata

2017-06-01

Full Text Available Neurogenic pulmonary edema (NPE is an acute life-threatening complication associated with many forms of central nervous system injury. Its pathophysiology is still debated. We report a patient with acute obstructive hydrocephalus due to a central neurocytoma who also had NPE, for which serial transpulmonary thermodilution monitoring (PiCCO was performed. Insertion of the PiCCO, which provides information about the patient's cardiac output, preload status and amount of lung water, revealed a high pulmonary vascular permeability index (PVPI and low global end-diastolic volume (GEDV without cardiac dysfunction, indicating permeability edema, which led to our changing the therapeutic strategy. Using PiCCO monitoring to balance the preload and extent of pulmonary edema enabled achievement of an optimal cardiac preload for organ perfusion, resulting in normalization of pulmonary edema by day 2. PiCCO facilitates understanding of the mechanism of NPE, guiding the management of fluid balance and the choice of vasopressors in patients with life-threatening NPE.

Mechanical valve at pulmonary site in adult TOF & absent pulmonary valve

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Aayush Goyal

2017-09-01

Full Text Available Absent pulmonary valve syndrome (APVS is a rare congenital heart disease. Tetralogy of Fallot (TOF with APVS is a rare variation of TOF. These patients are commonly cyanotic at birth. Respiratory complaints predominate due to airway compression by dilated pulmonary arteries. Commonest age of presentation is infancy with anecdotal adult case-reports. Surgical treatment requires establishing unobstructed competent right ventricular outflow tract (RVOT often with monocusp or conduits. We present a novel technique of rendering RVOT competent by implanting a tilting disc mechanical prosthesis in a rare adult TOF with APVS.

Generalized status epilepticus associated with massive pulmonary aspiration and transient central diabetes insipidus: case report

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CARVALHO MAURICIO

2000-01-01

Full Text Available Status epilepticus causes significant morbidity and mortality. A case of generalized status epilepticus followed by massive pulmonary aspiration, acute respiratory failure and transient central diabetes insipidus is presented. Seizures were promptly controlled, but the patient required mechanical ventilation and correction of polyuria with desmopressin acetate. During hospitalization mental status improved, diabetes insipidus spontaneously remitted and he was discharged without neurologic sequelae. The clinical and pathophysiological features of this case are discussed.

Neurogenic Pulmonary Edema (A Case Report

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Funda Gümüş

2012-08-01

Full Text Available Neurogenic pulmonary edema is a life threatening complication of severe central nervous system injury. The most common cause of neurogenic pulmonary edema is subarachnoid hemorrhage followed by head trauma and epilepsy. The rare causes are cervical spine trauma, multiplesclerosis, cerebellar hemorrhage and intracranial tumors. Neurogenic pulmonary edema is characterized by an increase in extravascular lung water in patients who have sustained a sudden change in neurologic condition. The exact pathophysiology is unclear but it probably involves an adrenergic response to the central nervous system injury which leads to increased catecholamine, pulmonary hydrostatic pressure and increased lung capillary permeability. The presenting symptoms are nonspecific and often include dyspnea, tachypnea, tachycardia, hypoxemia, pinkfroty secretion, bilateral pulmonary infiltrates and crackles. These symptoms start within minutes or hours and resolves 48-72 hours that typically for neurogenic pulmonary edema. Basic principles of treatment, surgical decompression, reduce intracranial pressure, controlled ventilation with suplemental oxygen, positive end expiratory pressure and diuresis. We report a case with neurogenic pulmonary edema that occured after head trauma. (Journal of the Turkish Society Intensive Care 2012; 10: 59-62

[Correction of bronchial obstructive syndrome and antituberculous drugs-induced eosinophilia in patients with pulmonary tuberculosis by using plasmapheresis].

Science.gov (United States)

Shmelev, E I; Stepanian, I E

1996-01-01

The paper provides the results of a follow-up of 70 patients with active pulmonary tuberculosis in whom the administration of antituberculous drugs induced eosinophilia and bronchial obstructive syndrome. To eliminate the side effects of antituberculous therapy, a plasmapheresis regimen was performed in 44 patients, the remaining patients were given only bronchodilators and antihistamine drugs. Plasmapheresis as a means for correcting drug-induced eosinophilia and bronchial obstructive syndrome was found to be more effective than drug therapy and, in some cases, enabled antituberculous therapy to be continued, without changing a combination of drugs. It is recommended that plasmapheresis should be used in cases of inadequate efficiency of conventional methods for correcting drug intolerance.

Bland-White-Garland syndrome of anomalous left coronary artery arising from the pulmonary artery (ALCAPA): a historical review

International Nuclear Information System (INIS)

Cowles, Robert A.; Berdon, Walter E.

2007-01-01

The landmark 1933 case report from Massachusetts General Hospital by Bland, White and Garland (Am Heart J 8:787-801) described a 3-month-old child with progressive feeding problems, cardiomegaly on chest radiography, and EKG evidence of left ventricular damage. Of interest was the fact that the vigilant father of the infant was Aubrey Hampton, a radiologist and future chairman of radiology at Massachusetts General Hospital. At autopsy, the left coronary artery originated from the pulmonary artery rather than from the aorta. Effective treatment for this condition was not available until 1960 when Sabiston, Neill and Taussig showed that the blood flowed from the left coronary artery toward the pulmonary artery. The anomalous left coronary artery was ligated at its junction with the pulmonary artery and the child survived. This historical review of Bland-White-Garland syndrome, now known as anomalous left coronary artery arising from the pulmonary artery (ALCAPA), stresses the continued diagnostic significance of cardiomegaly on chest radiography and EKG changes suggesting left ventricular damage in 2- to 3-month-old infants with feeding intolerance or irritability. With a high index of suspicion, an echocardiogram can be obtained to confirm the diagnosis. Modern surgical methods involve left coronary artery translocation and afford excellent outcomes. (orig.)

Plain radiographic, nuclear medicine and angiographic observations of hepatogenic pulmonary angiodysplasia

Energy Technology Data Exchange (ETDEWEB)

Sang Oh, K; Bender, T M; Bowen, A D; Ledesma-Medina, J

1983-05-01

Clinical and radiological findings of hepatogenic pulmonary angiodysplasia are reported in two cases. Myriad spidery pulmonary blood vessles are seen on plain radiographs and verified with right to left intrapulmonary shunting on pulmonary angiogram and pulmonary isotopic perfusion scan. Pathophysiology and differential diagnosis are discussed. We propose the medical term ''pulmonary angiodysplasia'' to include: 1) Pulmonary telangiectasia 2) Hereditary hemorrhagic telangiectasia (Osler-Rendu-Weber syndrome) 3) Classical discrete pulmonary arteriovenous fistula 4) Hepatogenic discrete pulmonary angiodysplasia: pulmonary arteriovenous communications with right to left shunting secondary to hepatic cirrhosis.

Reduction of central neuropathic pain with ketamine infusion in a patient with Ehlers-Danlos syndrome: a case report.

Science.gov (United States)

Lo, Tony Chung Tung; Yeung, Stephen Tung; Lee, Sujin; Skavinski, Kira; Liao, Solomon

2016-01-01

Ehlers-Danlos syndrome frequently causes acute and chronic pain because of joint subluxations and dislocations secondary to hypermobility. Current treatments for pain related to Ehlers-Danlos syndrome and central pain syndrome are inadequate. This case report discusses the therapeutic use of ketamine intravenous infusion as an alternative. A 27-year-old Caucasian female with a history of Ehlers-Danlos syndrome and spinal cord ischemic myelopathy resulting in central pain syndrome, presented with severe generalized body pain refractory to multiple pharmacological interventions. After a 7-day course of ketamine intravenous infusion under controlled generalized sedation in the intensive care unit, the patient reported a dramatic reduction in pain levels from 7-8 out of 10 to 0-3 out of 10 on a numeric rating scale and had a significant functional improvement. The patient tolerated a reduction in her pain medication regimen, which originally included opioids, gabapentin, pregabalin, tricyclic antidepressants, and nonsteroidal anti-inflammatory drugs. Ketamine infusion treatment has been used in various pain syndromes, including central neuropathic pain, ischemic pain, and regional pain syndrome. Reports have suggested that ketamine modulates pain by the regression of N-methyl-D-aspartate receptor to a resting state. As such, propagation of nociceptive signal to brain is interrupted allowing for the restoration of physiological balance between pain inhibition and facilitation. The present report shows that this treatment option can be used in patients with refractory central pain syndrome in the setting of spinal cord myelopathy secondary to Ehlers-Danlos syndrome. In addition, as seen in this case, this protocol can potentially decrease the chronic use of pain medication, such as opioids.

Bilateral widespread mechanical pain sensitivity in carpal tunnel syndrome: evidence of central processing in unilateral neuropathy.

Science.gov (United States)

Fernández-de-las-Peñas, César; de la Llave-Rincón, Ana Isabel; Fernández-Carnero, Josué; Cuadrado, María Luz; Arendt-Nielsen, Lars; Pareja, Juan A

2009-06-01

The aim of this study was to investigate whether bilateral widespread pressure hypersensitivity exists in patients with unilateral carpal tunnel syndrome. A total of 20 females with carpal tunnel syndrome (aged 22-60 years), and 20 healthy matched females (aged 21-60 years old) were recruited. Pressure pain thresholds were assessed bilaterally over median, ulnar, and radial nerve trunks, the C5-C6 zygapophyseal joint, the carpal tunnel and the tibialis anterior muscle in a blinded design. The results showed that pressure pain threshold levels were significantly decreased bilaterally over the median, ulnar, and radial nerve trunks, the carpal tunnel, the C5-C6 zygapophyseal joint, and the tibialis anterior muscle in patients with unilateral carpal tunnel syndrome as compared to healthy controls (all, P < 0.001). Pressure pain threshold was negatively correlated to both hand pain intensity and duration of symptoms (all, P < 0.001). Our findings revealed bilateral widespread pressure hypersensitivity in subjects with carpal tunnel syndrome, which suggest that widespread central sensitization is involved in patients with unilateral carpal tunnel syndrome. The generalized decrease in pressure pain thresholds associated with pain intensity and duration of symptoms supports a role of the peripheral drive to initiate and maintain central sensitization. Nevertheless, both central and peripheral sensitization mechanisms are probably involved at the same time in carpal tunnel syndrome.

Pulmonary balloon angioplasty of chronic thromboembolic pulmonary hypertension (CTEPH) in surgically inaccessible cases

International Nuclear Information System (INIS)

Pitton, M.B.; Herber, S.; Thelen, M.; Mayer, E.

2003-01-01

The clinical course of patients suffering from chronic thromboembolic pulmonary hypertension (CTEPH) depends on the distribution pattern of the thromboembolic material. In patients with thromboembolic findings in the central pulmonary segments pulmonary thrombendarterectomy (PTE) has excellent results and acceptable operative risk. This paper presents two surgically inaccessable cases that were successfully treated with balloon pulmonary angioplasty. Balloon angioplasty improved parenchymal perfusion, increased cardiac index (ΔCI + 19.2% [Case 1], and + 15.4% [2]), reduced pulmonary vascular resistance during follow-up (ΔPVRI - 25.0% [1] and - 15.9% [2]), and is discussed as an alternative treatment option for cases not suited for surgery. (orig.) [de

Acute heart failure syndrome

African Journals Online (AJOL)

and the classical syndrome of chronic persistent heart failure develops. The vast ... Flash pulmonary oedema: This is a severely elevated blood pressure with an .... (CPAP or bilevelNPPV) for cardiogenic pulmonary edema (review). Cochrane.

Pulmonary manifestations from systemic vasculitides

International Nuclear Information System (INIS)

Reuter, M.; Both, M.; Schnabel, A.

2007-01-01

Pulmonary vasculitides predominantly involve the small arterioles, capillaries and venules and include Wegener's granulomatosis, microscopic polyangiitis and the Churg-Strauss syndrome. Takayasu's arteriitis is a large vessel disease and may affect the main pulmonary arteries causing stenoses and occlusions. Knowledge of the natural course of disease and of clinical manifestations of pulmonary disease is helpful for an understanding of imaging findings. For this reason this article gives an overview not only of radiologic findings in chest X-ray and high resolution CT of the lungs but as well of clinical aspects of pulmonary vasculitides. Next to determination of disease extension the determination of disease activity is in the foreground of diagnostic imaging in vasculitides. Within this context principals of immunosuppressive therapy will be recognized. (orig.)

Central Retinal Artery Occlusion in a Patient with Metabolic Syndrome X

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Sonja Predrag Cekić

2010-01-01

Full Text Available Purpose: To report a case of central retinal artery occlusion (CRAO in a patient with metabolic syndrome X. Case Report: A 64 year-old-man presented with abrupt, painless, and severe loss of vision in his left eye. Indirect ophthalmoscopy disclosed signs compatible with CRAO and laboratory investigations revealed erythrocyte sedimentation rate of 74 mm/h, C-reactive protein (CRP level of 21 mg/l, hyperglycemia, hyperuricemia, hypertriglyceridemia and hypercholesterolemia. Fluorescein angiography and immunological studies excluded other systemic disorders. The patient met the full criteria of the National Cholesterol Education Program for metabolic syndrome X. Conclusion: In addition to different vascular complications such as stroke, and cardiovascular disease, metabolic syndrome X may be associated with retinal vascular occlusions.

[Pulmonary Manifestations of Vasculitis].

Science.gov (United States)

von Vietinghoff, S

2016-11-01

The variable symptoms and signs of pulmonary vasculitis are a diagnostic and therapeutic challenge. Vasculitis should be considered in rapidly progressing, severe and unusual manifestations of pulmonary disease. Clinical examination of other organ systems typically affected by vasculitis such as skin and kidney and autoantibody measurements are complementary approaches to manage this situation. Pulmonary involvement is common in small vessel vasculitis including anti-GBM disease (Goodpasture syndrome) and the ANCA-associated vasculitides. Life threatening pulmonary hemorrhage and irreversible damage of other organs, frequently the kidney, are important complications necessitating rapid diagnosis of these conditions.Vasculitides are rare diseases of multiple organs and therapies including biologics are evolving rapidly, requiring cooperation of specialities and with specialized centres to achieve best patient care. All involved physicians should be aware of typical complications of immunosuppressive therapy. © Georg Thieme Verlag KG Stuttgart · New York.

Shrinking lung syndrome as a manifestation of pleuritis: a new model based on pulmonary physiological studies.

Science.gov (United States)

Henderson, Lauren A; Loring, Stephen H; Gill, Ritu R; Liao, Katherine P; Ishizawar, Rumey; Kim, Susan; Perlmutter-Goldenson, Robin; Rothman, Deborah; Son, Mary Beth F; Stoll, Matthew L; Zemel, Lawrence S; Sandborg, Christy; Dellaripa, Paul F; Nigrovic, Peter A

2013-03-01

The pathophysiology of shrinking lung syndrome (SLS) is poorly understood. We sought to define the structural basis for this condition through the study of pulmonary mechanics in affected patients. Since 2007, most patients evaluated for SLS at our institutions have undergone standardized respiratory testing including esophageal manometry. We analyzed these studies to define the physiological abnormalities driving respiratory restriction. Chest computed tomography data were post-processed to quantify lung volume and parenchymal density. Six cases met criteria for SLS. All presented with dyspnea as well as pleurisy and/or transient pleural effusions. Chest imaging results were free of parenchymal disease and corrected diffusing capacities were normal. Total lung capacities were 39%-50% of predicted. Maximal inspiratory pressures were impaired at high lung volumes, but not low lung volumes, in 5 patients. Lung compliance was strikingly reduced in all patients, accompanied by increased parenchymal density. Patients with SLS exhibited symptomatic and/or radiographic pleuritis associated with 2 characteristic physiological abnormalities: (1) impaired respiratory force at high but not low lung volumes; and (2) markedly decreased pulmonary compliance in the absence of identifiable interstitial lung disease. These findings suggest a model in which pleural inflammation chronically impairs deep inspiration, for example through neural reflexes, leading to parenchymal reorganization that impairs lung compliance, a known complication of persistently low lung volumes. Together these processes could account for the association of SLS with pleuritis as well as the gradual symptomatic and functional progression that is a hallmark of this syndrome.

[Pulmonary complications of malaria: An update].

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Cabezón Estévanez, Itxasne; Górgolas Hernández-Mora, Miguel

2016-04-15

Malaria is the most important parasitic disease worldwide, being a public health challenge in more than 90 countries. The incidence of pulmonary manifestations has increased in recent years. Acute respiratory distress syndrome is the most severe form within the pulmonary complications of malaria, with high mortality despite proper management. This syndrome manifests with sudden dyspnoea, cough and refractory hypoxaemia. Patients should be admitted to intensive care units and treated with parenteral antimalarial drug treatment and ventilatory and haemodynamic support without delay. Therefore, dyspnoea in patients with malaria should alert clinicians, as the development of respiratory distress is a poor prognostic factor. Copyright © 2016 Elsevier España, S.L.U. All rights reserved.

Hermansky-Pudlak syndrome

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Preena A

2017-04-01

Full Text Available Hermansky-Pudlak syndrome is a rare autosomal recessive multisystem disease, with oculocutneous albinism, pulmonary fibrosis and bleeding diathesis. Here we report a case of Hermansky-Pudlak syndrome who presented with dyspnea, oculocutaneous albinism and nystagmus.

Congenital central hypoventilation syndrome: diagnostic and management challenges

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Kasi AS

2016-08-01

Full Text Available Ajay S Kasi,1 Iris A Perez,1,2 Sheila S Kun,1 Thomas G Keens1,2 1Division of Pediatric Pulmonology and Sleep Medicine, Children’s Hospital Los Angeles, 2Keck School of Medicine of the University of Southern California, Los Angeles, CA, USA Abstract: Congenital central hypoventilation syndrome (CCHS is a rare genetic disorder with failure of central control of breathing and of the autonomic nervous system function due to a mutation in the paired-like homeobox 2B (PHOX2B gene. Affected patients have absent or negligible ventilatory sensitivity to hypercapnia and hypoxemia, and they do not exhibit signs of respiratory distress when challenged with hypercarbia or hypoxia. The diagnosis of CCHS must be confirmed with PHOX2B gene mutation. Generally, the PHOX2B mutation genotype can aid in anticipating the severity of the phenotype. They require ventilatory support for life. Home assisted ventilation options include positive pressure ventilation via tracheostomy, noninvasive positive pressure ventilation, and diaphragm pacing via phrenic nerve stimulation, but each strategy has its associated limitations and challenges. Since all the clinical manifestations of CCHS may not manifest at birth, periodic monitoring and early intervention are necessary to prevent complications and improve outcome. Life-threatening arrhythmias can manifest at different ages and a normal cardiac monitoring study does not exclude future occurrences leading to the dilemma of timing and frequency of cardiac rhythm monitoring and treatment. Given the rare incidence of CCHS, most health care professionals are not experienced with managing CCHS patients, particularly those with diaphragm pacers. With early diagnosis and advances in home mechanical ventilation and monitoring strategies, many CCHS children are surviving into adulthood presenting new challenges in their care. Keywords: congenital central hypoventilation syndrome, PHOX2B, home mechanical ventilation, diaphragm

[Economy-class syndrome or Immobile Traveler's syndrome?].

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Gispert, Pilar; Drobnic, M Estrella; Vidal, Rafael

2006-07-01

Economy class syndrome describes the situation of patients who suffer pulmonary embolism following prolonged immobility in a long distance flight. The objective of the present study was to analyze whether there is a risk of pulmonary thromboembolism after a long overland journey. The study consecutively enrolled 100 patients diagnosed with pulmonary thromboembolism. Nine of them had previously undertaken a long journey (>5 h), 6 of which had been over land. Consequently, we think that the preventative measures recommended for airline passengers should be extended to users of other means of transport.

Cardiovocal syndrome – A rare presentation of primary pulmonary hypertension

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Om Shankar

2014-05-01

Full Text Available Primary pulmonary hypertension is a well known entity with characteristic features more common in females presenting commonly with dyspnea. However primary pulmonary hypertension presenting as hoarseness of voice is rare occurring most likely due to compression of left recurrent laryngeal nerve between normal aorta and dilated tense pulmonary artery. Here we are presenting a case of 19 year old boy with primary pulmonary hypertension who presented with hoarseness of voice as predominant symptom.

Therapeutic approaches to asthma-chronic obstructive pulmonary disease overlap syndromes.

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Barnes, Peter J

2015-09-01

The recognition that there are some patients with features of asthma and chronic obstructive pulmonary disease (COPD) has highlighted the need to develop more specific treatments for these clinical phenotypes. Some patients with COPD have predominantly eosinophilic inflammation and might respond to high doses of inhaled corticosteroids and newly developed specific antieosinophil therapies, including blocking antibodies against IL-5, IL-13, IL-33, and thymic stromal lymphopoietin, as well as oral chemoattractant receptor-homologous molecule expressed on TH2 cells antagonists. Other patients have severe asthma or are asthmatic patients who smoke with features of COPD-induced inflammation and might benefit from treatments targeting neutrophils, including macrolides, CXCR2 antagonists, phosphodiesterase 4 inhibitors, p38 mitogen-activating protein kinase inhibitors, and antibodies against IL-1 and IL-17. Other patients appear to have largely fixed obstruction with little inflammation and might respond to long-acting bronchodilators, including long-acting muscarinic antagonists, to reduce hyperinflation. Highly selected patients with severe asthma might benefit from bronchial thermoplasty. Some patients with overlap syndromes can be conveniently treated with triple fixed-dose combination inhaler therapy with an inhaled corticosteroid, long-acting β2-agonist, and long-acting muscarinic antagonist, several of which are now in development. Corticosteroid resistance is a feature of asthma-COPD overlap syndrome, and understanding the various molecular mechanisms of this resistance has identified novel therapeutic targets and presented the prospect of therapies that can restore corticosteroid responsiveness. Copyright © 2015 American Academy of Allergy, Asthma & Immunology. Published by Elsevier Inc. All rights reserved.

Endovascular technique using a snare and suture for retrieving a migrated peripherally inserted central catheter in the left pulmonary artery

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Teragawa, Hiroki; Sueda, Takashi; Fujii, Yuichi; Takemoto, Hiroaki; Toyota, Yasushi; Nomura, Shuichi; Nakagawa, Keigo

2013-01-01

We report a successful endovascular technique using a snare with a suture for retrieving a migrated broken peripherally inserted central catheter (PICC) in a chemotherapy patient. A 62-year-old male received monthly chemotherapy through a central venous port implanted into his right subclavian area. The patient completed chemotherapy without complications 1 mo ago; however, he experienced pain in the right subclavian area during his last chemotherapy session. Computed tomography on that day showed migration of a broken PICC in his left pulmonary artery, for which the patient was admitted to our hospital. We attempted to retrieve the ectopic PICC through the right jugular vein using a gooseneck snare, but were unsuccessful because the catheter was lodged in the pulmonary artery wall. Therefore, a second attempt was made through the right femoral vein using a snare with triple loops, but we could not grasp the migrated PICC. Finally, a string was tied to the top of the snare, allowing us to curve the snare toward the pulmonary artery by pulling the string. Finally, the catheter body was grasped and retrieved. The endovascular suture technique is occasionally extremely useful and should be considered by interventional cardiologists for retrieving migrated catheters. PMID:24109502

Williams-Beuren's Syndrome: A Case Report.

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Zamani, Hassan; Babazadeh, Kazem; Fattahi, Saeid; Mokhtari-Esbuie, Farzad

2012-01-01

Williams-Beuren syndrome is a rare familial multisystem disorder occurring in 1 per 20,000 live births. It is characterized by congenital heart defects (CHD), skeletal and renal anomalies, cognitive disorder, social personality disorder and dysmorphic facies. We present a case of Williams syndrome that presented to us with heart murmur and cognitive problem. A 5-year-old girl referred to pediatric cardiologist because of heart murmurs. She had a systolic murmur (2-3/6) in right upper sternal border with radiation to right cervical region. She also had a bulge forehead. Angiography showed mild supra valvular aortic stenosis and mild multiple peripheral pulmonary stenosis. Fluorescent in situ hybridization (FISH) was performed and the result was: 46.XX, ish del (7q11.2) (ELN X1) (7q22 X2) ELN deletion compatible with Williams syndrome. Peripheral pulmonary artery stenosis is associated with Noonan syndrome, Alagille syndrome, Cutis laxa, Ehler-Danlos syndrome, and Silver-Russel syndrome. The patient had peripheral pulmonary artery stenosis, but no other signs of these syndromes were present, and also she had a supravalvular aortic stenosis which was not seen in other syndromes except Williams syndrome. Conclusion. According to primary symptoms, paraclinical and clinical finding such as dysmorphic facies, cognitive disorder and congenital heart defect, Williams syndrome was the first diagnosis. We suggest a more attention for evaluating heart murmur in childhood period, especially when the patient has abnormal facial features or mental problem.

Williams-Beuren's Syndrome: A Case Report

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Hassan Zamani

2012-01-01

Full Text Available Williams-Beuren syndrome is a rare familial multisystem disorder occurring in 1 per 20,000 live births. It is characterized by congenital heart defects (CHD, skeletal and renal anomalies, cognitive disorder, social personality disorder and dysmorphic facies. We present a case of Williams syndrome that presented to us with heart murmur and cognitive problem. A 5-year-old girl referred to pediatric cardiologist because of heart murmurs. She had a systolic murmur (2-3/6 in right upper sternal border with radiation to right cervical region. She also had a bulge forehead. Angiography showed mild supra valvular aortic stenosis and mild multiple peripheral pulmonary stenosis. Fluorescent in situ hybridization (FISH was performed and the result was: 46.XX, ish del (7q11.2 (ELN X1 (7q22 X2 ELN deletion compatible with Williams syndrome. Peripheral pulmonary artery stenosis is associated with Noonan syndrome, Alagille syndrome, Cutis laxa, Ehler-Danlos syndrome, and Silver-Russel syndrome. The patient had peripheral pulmonary artery stenosis, but no other signs of these syndromes were present, and also she had a supravalvular aortic stenosis which was not seen in other syndromes except Williams syndrome. Conclusion. According to primary symptoms, paraclinical and clinical finding such as dysmorphic facies, cognitive disorder and congenital heart defect, Williams syndrome was the first diagnosis. We suggest a more attention for evaluating heart murmur in childhood period, especially when the patient has abnormal facial features or mental problem.

Extravascular lung water and pulmonary arterial wedge pressure for fluid management in patients with acute respiratory distress syndrome.

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Hu, Wei; Lin, Chang-Wen; Liu, Bing-Wei; Hu, Wei-Hang; Zhu, Ying

2014-01-16

Extravascular lung water (EVLW) is a sensitive prognostic indicator of pulmonary edema. Thus, EVLW may be an advantageous method of fluid management. This study aims to evaluate the outcomes of using EVLW and pulmonary artery wedge pressure (PAWP) as strategies for fluid management in patients with acute respiratory distress syndrome (ARDS). Twenty-nine patients were randomly divided into the EVLW and PAWP groups. The survival rate, ICU (Intensive Care Unit) length of stay, duration of mechanical ventilation, acute lung injury scores, and oxygenation index of the EVLW and PAWP groups were compared. No significant difference in the survival rates at 28 and 60 days (d) after treatment was found between the two groups (p = 0.542). The duration of mechanical ventilation and ICU length of stay were significantly lower (p management improved clinical results in patients with ARDS better than PAWP.

Are ECG abnormalities in Noonan syndrome characteristic for the syndrome?

NARCIS (Netherlands)

Raaijmakers, R.; Noordam, C.; Noonan, J.A.; Croonen, E.A.; Burgt, C.J.A.M. van der; Draaisma, J.M.T.

2008-01-01

Of all patients with Noonan syndrome, 50-90% have one or more congenital heart defects. The most frequent occurring are pulmonary stenosis (PS) and hypertrophic cardiomyopathy. The electrocardiogram (ECG) of a patient with Noonan syndrome often shows a characteristic pattern, with a left axis

[Asthma-COPD overlap syndrome].

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Odler, Balázs; Müller, Veronika

2016-08-01

Obstructive lung diseases represent a major health problem worldwide due to their high prevalence associated with elevated socioeconomic costs. Bronchial asthma and chronic obstructive pulmonary disease are chronic obstructive ventilatory disorders with airway inflammation, however they are separate nosological entities based on thedifferent development, diagnostic and therapeutic approaches, and prognostic features. However, these diseases may coexist and can be defined as the coexistence of increased variability of airflow in a patient with incompletely reversible airway obstruction. This phenotype is called asthma - chronic obstructive pulmonary disease overlap syndrome. The syndrome is a clinical and scientific challenge as the majority of these patients have been excluded from the clinical and pharmacological trials, thus well-defined clinical characteristics and therapeutic approaches are lacking. The aim of this review is to summarize the currently available literature focusing on pathophysiological and clinical features, and discuss possible therapeutic approaches of patients with asthma - chronic obstructive pulmonary disease overlap syndrome. Orv. Hetil., 2016, 157(33), 1304-1313.

[Right branch pulmonary artery stenosis with supravalvar aortic stenosis as a complication of Lecompte maneuver for tetralogy of Fallot associated with absent pulmonary valve].

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Honda, Yoshihiro; Suzuki, Shoji; Kaga, Shigeaki; Yoshida, Yukiyo; Kimura, Mitsuhiro; Kamiya, Kentaro; Sakakibara, Kenji; Katsu, Masatake

2015-05-01

The patient was diagnosed with tetralogy of Fallot associated with absent pulmonary valve syndrome and a low birth weight of 1,912 g. He suffered from respiratory distress on day 14 and received non-invasive positive pressure ventilation. At 5 months of age and 4.1 kg, he underwent intracardiac repair including right ventricular outflow repair with a monocusp patch, patch closure of the ventricular septum defect and right pulmonary transposition to the anterior of the ascending aorta following the Lecompte maneuver for airway decompression. He was subsequently discharged to home and exhibited an uneventful clinical course with non-invasive positive pressure ventilation for 5 months postoperatively. However, right pulmonary artery and supra-aortic stenosis was noted 2 years after the operation. Computed tomography (CT) and angiography showed ascending aorta strangulation by the right pulmonary artery with right ventricular outflow regurgitation. Right pulmonary artery reconstruction using polytetrafluoroethylene graft interposition and repeat right ventricular outflow repair with bicuspid hand-sewn valves was therefore performed;the postoperative course was uneventful. Pre- and post-operative management using non-invasive positive pressure ventilation and airway decompression with pulmonary artery translocation is a useful strategy in patients exhibiting symptomatic tetralogy of Fallot associated with absent pulmonary valve syndrome in the neonatal period.

Pulmonary Venous Diastolic Flow Reversal and Flash Pulmonary Edema During Management of Ongoing Myocardial Ischemia with Intraaortic Balloon Pump.

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Murray, Davoy; Peng, Yong G

2015-10-15

A 65-year-old man was admitted for acute coronary syndrome with depressed left ventricular function and moderate aortic regurgitation. He was managed with an intraaortic balloon pump for circulatory support before coronary artery bypass grafting and subsequently developed flash pulmonary edema with an associated rare finding of diastolic pulmonary venous flow reversal. In this report, we provide a review of intraaortic balloon pump use in current clinical practice and elaborate on the pathophysiology of an uncommon pulmonary venous flow pattern found in our patient.

Asthma-chronic obstructive pulmonary disease overlap syndrome – Literature review and contributions towards a Portuguese consensus

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D. Araújo

2017-03-01

Full Text Available Introduction: Phenotypic overlap between the two main chronic airway pulmonary diseases, asthma and chronic obstructive pulmonary disease (COPD, has been the subject of debate for decades, and recently the nomenclature of asthma-COPD overlap syndrome (ACOS was adopted for this condition. The definition of this entity in the literature is, however, very heterogeneous, it is therefore important to define how it applies to Portugal. Methods: A literature review of ACOS was made in a first phase resulting in the drawing up of a document that was later submitted for discussion among a panel of chronic lung diseases experts, resulting in reflexions about diagnosis, treatment and clinical guidance for ACOS patients. Results: There was a consensus among the experts that the diagnosis of ACOS should be considered in the concomitant presence of: clinical manifestations characteristic of both asthma and COPD, persistent airway obstruction (post-bronchodilator FEV1/FVC 300 eosinophils/μL or >5% of leukocytes and previous history of atopy should also be considered. The recommended first line pharmacological treatment in these patients is the ICS/LABA association; if symptomatic control is not achieved or in case of clinical severity, triple therapy with ICS/LABA/LAMA may be used. An effective control of the exposure to risk factors, vaccination, respiratory rehabilitation and treatment of comorbidities is also important. Conclusions: The creation of initial guidelines on ACOS, which can be applied in the Portuguese context, has an important role in the generation of a broad nationwide consensus. This will give, in the near future, a far better clinical, functional and epidemiological characterization of ACOS patients, with the ultimate goal of achieving better therapeutic guidance. Keywords: Asthma, Chronic obstructive pulmonary disease, Overlap syndrome, Portuguese consensus

Allergic bronchopulmonary aspergillosis in an adult with Kartagener syndrome.

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Sehgal, Inderpaul Singh; Dhooria, Sahajal; Bal, Amanjit; Agarwal, Ritesh

2015-08-06

Allergic bronchopulmonary aspergillosis (ABPA) is a pulmonary disorder resulting from immune responses directed against inhaled Aspergillus fumigatus antigens. It manifests with poorly controlled asthma, fleeting pulmonary opacities and structural lung damage in the form of bronchiectasis. Initially defined in individuals suffering from bronchial asthma and cystic fibrosis, it has also been described in patients with other structural lung disorders such as chronic obstructive pulmonary disease, pulmonary tuberculosis, idiopathic bronchiectasis and others. Kartagener syndrome is a manifestation of primary ciliary dyskinesia characterised by the presence of dextrocardia, bronchiectasis and chronic sinusitis. We report a case of ABPA in an adult suffering from Kartagener syndrome. We also performed a systematic review of the literature on the association between Kartagener syndrome and ABPA. 2015 BMJ Publishing Group Ltd.

Comparison of metabolic syndrome with growing epidemic syndrome Z in terms of risk factors and gender differences.

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Uyar, Meral; Davutoğlu, Vedat; Aydın, Neriman; Filiz, Ayten

2013-05-01

The aim of this study is to compare metabolic syndrome with syndrome Z growing epidemic in terms of risk factors, demographic variables, and gender differences in our large cohort at southeastern area in Turkey. Data of patients admitted to sleep clinic in University of Gaziantep from January 2006 to January 2011 were retrospectively evaluated. ATP III and JNC 7 were used for defining metabolic syndrome and hypertension. Data of 761 patients were evaluated. Hypertension, diabetes mellitus, coronary artery disease, pulmonary hypertension, and left ventricular hypertrophy were more common in patients with syndrome Z than in patients without metabolic syndrome. Age, waist/neck circumferences, BMI, triglyceride, glucose, and Epworth sleepiness scale score were detected higher, whereas the minimum oxygen saturation during sleep was lower in patients with syndrome Z. Metabolic syndrome was more common in sleep apneic subjects than in controls (58 versus 30 %). Female sleep apneics showed higher rate of metabolic syndrome than those of males (74 versus 52 %). Hypertension, diabetes mellitus, coronary artery disease, and left ventricular hypertrophy were detected higher in males with syndrome Z than in males without metabolic syndrome. Snoring and excessive daytime sleepiness were detected higher in females with syndrome Z than in females without metabolic syndrome. Systemic/pulmonary hypertension, diabetes mellitus, and left ventricular hypertrophy were more common in females with syndrome Z than in females without metabolic syndrome. Complaints of headache and systemic/pulmonary hypertension were more common among females than males with syndrome Z. Female syndrome Z patients had lower minimum oxygen saturation than male patients with syndrome Z. Metabolic syndrome in sleep apneic patients is more prevalent than in controls. All metabolic syndrome parameters were significantly different among obstructive sleep apneic patients with respect to gender with more severe

Central xanthoma of the jaw in association with Noonan syndrome.

Science.gov (United States)

Olson, Nicholas J; Addante, Rocco R; de Abreu, Francine B; Memoli, Vincent A

2018-05-01

Xanthomas are histiocytic lesions of the skin, soft tissue and bone and are generally considered to be reactive in nature. When they arise in the bones of the jaw, they are referred to as central xanthomas. New evidence supports the hypothesis that central xanthomas are a separate and distinct entity from their extragnathic counterparts. Noonan syndrome (NS) is an autosomal dominant disorder that has been associated with giant cell lesions which also commonly occur in the jaw. We present a case of a 15year-old-male with NS who presented with a radiolucent lesion of the mandible that on excision, was found to be a central xanthoma. Although giant cell lesions have been well described in NS, xanthomas of the jaw have not been reported. We will also discuss the entities that must be excluded prior to making a diagnosis of central xanthoma, as this can affect both treatment and follow up. Copyright © 2018. Published by Elsevier Inc.

Pulmonary hypertension of the newborn.

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Stayer, Stephen A; Liu, Yang

2010-09-01

Pulmonary hypertension presenting in the neonatal period can be due to congenital heart malformations (most commonly associated with obstruction to pulmonary venous drainage), high output cardiac failure from large arteriovenous malformations and persistent pulmonary hypertension of the newborn (PPHN). Of these, the most common cause is PPHN. PPHN develops when pulmonary vascular resistance (PVR) remains elevated after birth, resulting in right-to-left shunting of blood through foetal circulatory pathways. The PVR may remain elevated due to pulmonary hypoplasia, like that seen with congenital diaphragmatic hernia; maldevelopment of the pulmonary arteries, seen in meconium aspiration syndrome; and maladaption of the pulmonary vascular bed as occurs with perinatal asphyxia. These newborn patients typically require mechanical ventilatory support and those with underlying lung disease may benefit from high-frequency oscillatory ventilation or extra-corporeal membrane oxygenation (ECMO). Direct pulmonary vasodilators, such as inhaled nitric oxide, have been shown to improve the outcome and reduce the need for ECMO. However, there is very limited experience with other pulmonary vasodilators. The goals for anaesthetic management are (1) to provide an adequate depth of anaesthesia to ablate the rise in PVR associated with surgical stimuli; (2) to maintain adequate ventilation and oxygenation; and (3) to be prepared to treat a pulmonary hypertensive crisis--an acute rise in PVR with associated cardiovascular collapse.

A traumatic central cord syndrome occurring after adequate decompression for cervical spondylosis: biomechanics of injury: case report.

Science.gov (United States)

Dickerman, Rob D; Lefkowitz, Michael; Epstein, Joseph A

2005-10-15

Case report with review of the literature. To present the first case of a central cord syndrome occurring after adequate decompression, and review the mechanics of the cervical spinal cord injury and postoperative biomechanical and anatomic changes occurring after cervical decompressive laminectomy. Cervical spondylosis is a common pathoanatomic occurrence in the elderly population and is thought to be one of the primary causes for a central cord syndrome. Decompressive laminectomy with or without fusion has been a primary treatment for spondylotic disease and is thought to be protective against further injury. To our knowledge, there are no cases of a central cord syndrome occurring after adequate decompression reported in the literature. Case study with extensive review of the literature. The patient underwent C3-C7 cervical laminectomy without complications. After surgery, the patient's spasticity and gait difficulties improved. She was discharged to inpatient rehabilitation for further treatment of upper extremity weakness. The patient fell in the rehabilitation center, with a central cord syndrome despite adequate decompression of her spinal canal. The patient was treated conservatively for the central cord and had minimal improvement. Decompressive laminectomy provides an immediate decompressive effect on the spinal cord as seen by the dorsal migration of the cord, however, the biomechanics of the cervical spine after decompressive laminectomy remain uncertain. This case supports the ongoing research and need for more intensive research on postoperative cervical spine biomechanics, including decompressive laminectomies, decompressive laminectomy and fusion, and laminoplasty.

Tuberculosis and the acquired immune deficiency syndrome in South Brazil

International Nuclear Information System (INIS)

Vieira, M.V.; Genro, C.H.; Santos Silveira, R. de C. dos

1989-01-01

Tuberculosis and the acquired immune deficiency syndrome in South Brazil. The authors studied the incidence of tuberculosis in South Brazilian patients with acquired immune deficiency syndrome from January 1985 to June 1988. During this period, tuberculosis occurred in 10.3% of acquired immune deficiency syndrome patients. The socioeconomic conditions and the incidence of disease in the population were not confirmed as a potential risk for tuberculosis infection. Chest radiographs revealed pulmonary infiltrates in six patients, hilar and/or mediastinal adenopathy in three, and pleural effusion in two. The two remaining patients had pulmonary consolidation associated with other features. None of these patients presented pulmonary cavitation or radiographic findings of typical reactivation of pulmonary tuberculosis. (author) [pt

Lack of differential pattern in central adiposity and metabolic syndrome in Barrett's esophagus and gastroesophageal reflux disease.

LENUS (Irish Health Repository)

Healy, L A

2012-02-01

Obesity is an established risk factor for esophageal adenocarcinoma, although the mechanism is unclear. A pathway from reflux to inflammation through metaplasia is the dominant hypothesis, and an added role relating to visceral adiposity and the metabolic syndrome has been mooted in Barrett\\'s esophagus (BE) patients. Whether BE differs from gastroesophageal reflux disease (GERD) in obesity and metabolic syndrome profiles is unclear, and this was the focus of this study. Patients with proven BE or GERD were randomly selected from the unit data registry and invited to attend for metabolic syndrome screening, anthropometry studies including segmental body composition analysis, and laboratory tests including fasting lipids, insulin, and C-reactive protein. Metabolic syndrome was defined using the National Cholesterol Education Program (NCEP) and the International Diabetes Federation (IDF) criteria. One hundred and eighteen BE patients and 113 age- and sex-matched GERD controls were studied. The incidence of obesity (body mass index >30 kg\\/m(2)) was 36% and 38%, respectively, with the pattern of fat deposition predominantly central and an estimated trunk fat mass of 13 and 14 kg, respectively. Using the NCEP criteria, metabolic syndrome was significantly more common in the BE cohort (30% vs 20%, P < 0.05), but there was no significant difference using IDF criteria (42% vs 37%, P= 0.340). Central obesity and the metabolic syndrome are common in both Barrett\\'s and GERD cohorts, but not significantly different, suggesting that central obesity and the metabolic syndrome does not per se impact on the development of BE in a reflux population. In BE, the importance of obesity and the metabolic syndrome in disease progression merits further study.

Early Manifestation of Supravalvular Aortic and Pulmonary Artery Stenosis in a Patient with Williams Syndrome

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Jong Uk Lee

2016-04-01

Full Text Available Williams syndrome (WS is a developmental disorder characterized by vascular abnormalities such as thickening of the vascular media layer in medium- and large-sized arteries. Supravalvular aortic stenosis (SVAS and peripheral pulmonary artery stenosis (PPAS are common vascular abnormalities in WS. The natural course of SVAS and PPAS is variable, and the timing of surgery or intervention is determined according to the progression of vascular stenosis. In our patient, SVAS and PPAS showed rapid concurrent progression within two weeks after birth. We report the early manifestation of SVAS and PPAS in the neonatal period and describe the surgical treatment for stenosis relief.

Mucous plug syndrome. A pulmonary embolism mimic

International Nuclear Information System (INIS)

Bray, S.T.; Johnstone, W.H.; Dee, P.M.; Pope, T.L. Jr.; Teates, C.D.; Tegtmeyer, C.J.

1984-01-01

Reported are ten instances of major bronchial obstruction by mucous plugs in eight patients during which the clinical features resembled pulmonary embolism. Perfusion lung studies showed significantly diminished perfusion of the involved portions of the lungs. The chest radiographs generally did not, however, reflect the severity of the airway obstruction and in some instances were completely normal. The ventilation studies indicated the extent and severity of the obstruction and matched with the perfusion scans. Pulmonary arteriograms were performed in three patients and gave direct evidence of focally diminished lung perfusion without embolism. The physiologic mechanisms underlying the condition are discussed

Creation of aorto-pulmonary window with pulmonary artery band is not good palliation for hypoplastic left heart syndrome.

Science.gov (United States)

Hosein, Riad B M; Mehta, Chetan; Stickley, John; Mcguirk, Simon P; Jones, Timothy J; Brawn, William J; Barron, David J

2007-11-01

A small sub-group of patients with hypoplastic left heart syndrome (HLHS) have normal-sized ascending aorta and arch. An alternative to the Norwood I procedure in these patients is the creation of an aorto-pulmonary (AP) window with a distal pulmonary artery band (PAB). We reviewed our experience with this technique and compared outcomes to the Norwood procedure for HLHS. All patients treated for HLHS in a single institution between 1992 and 2005 were analysed. This identified 13 patients treated with AP window and PAB compared to 333 patients undergoing stage I Norwood procedure. An unrestrictive AP window was created and the main PA was banded. Patient records and echocardiograms were analysed. Median follow-up was 10 (IQR 0-655) days and 100% complete. There were seven early deaths (54%) in the AP window group and two conversions to Norwood circulation. This was a significantly worse outcome than for the Norwood procedure over the same period, which had an early mortality of 29% (p=0.03). Kaplan-Meier actuarial analysis demonstrated a continued survival benefit of the Norwood group at 6 months (p=0.0005). Deaths were due to either low cardiac output syndrome (n=4) or sudden unheralded arrest (n=3). This occurred despite aortic cross-clamp and circulatory arrest times being significantly lower in the AP window group compared to the Norwood group (35+/-27 vs 55+/-16 min, p<0.01 and 16+/-29 vs 55+/-20 min, p<0.01, respectively). No differences in arterial saturations or systolic blood pressure existed between the groups, but diastolic blood pressure was significantly lower in the AP window group at 27+/-10 mmHg compared to 42+/-8 mmHg in the Norwood group (p=0.01) with evidence of flow reversal in the descending aorta. Differences in diastolic blood pressure between groups were abolished after conversion to stage II. Despite favourable anatomy and shorter ischaemic times, the AP window/PAB technique has a poor outcome compared to the Norwood procedure for HLHS. Low

Pulmonary arterial hypertension associated with congenital heart disease

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Michele D'Alto

2012-12-01

Full Text Available Pulmonary arterial hypertension (PAH is a common complication of congenital heart disease (CHD, with most cases occurring in patients with congenital cardiac shunts. In patients with an uncorrected left-to-right shunt, increased pulmonary pressure leads to vascular remodelling and dysfunction, resulting in a progressive rise in pulmonary vascular resistance and increased pressures in the right heart. Eventually, reversal of the shunt may arise, with the development of Eisenmenger's syndrome, the most advanced form of PAH-CHD. The prevalence of PAH-CHD has fallen in developed countries over recent years and the number of patients surviving into adulthood has increased markedly. Today, the majority of PAH-CHD patients seen in clinical practice are adults, and many of these individuals have complex disease or received a late diagnosis of their defect. While there have been advances in the management and therapy in recent years, PAH-CHD is a heterogeneous condition and some subgroups, such as those with Down's syndrome, present particular challenges. This article gives an overview of the demographics, pathophysiology and treatment of PAH-CHD and focuses on individuals with Down's syndrome as an important and challenging patient group.

Chronic obstructive pulmonary disease

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V K Vijayan

2013-01-01

Full Text Available The global prevalence of physiologically defined chronic obstructive pulmonary disease (COPD in adults aged >40 yr is approximately 9-10 per cent. Recently, the Indian Study on Epidemiology of Asthma, Respiratory Symptoms and Chronic Bronchitis in Adults had shown that the overall prevalence of chronic bronchitis in adults >35 yr is 3.49 per cent. The development of COPD is multifactorial and the risk factors of COPD include genetic and environmental factors. Pathological changes in COPD are observed in central airways, small airways and alveolar space. The proposed pathogenesis of COPD includes proteinase-antiproteinase hypothesis, immunological mechanisms, oxidant-antioxidant balance, systemic inflammation, apoptosis and ineffective repair. Airflow limitation in COPD is defined as a postbronchodilator FEV1 (forced expiratory volume in 1 sec to FVC (forced vital capacity ratio <0.70. COPD is characterized by an accelerated decline in FEV1. Co morbidities associated with COPD are cardiovascular disorders (coronary artery disease and chronic heart failure, hypertension, metabolic diseases (diabetes mellitus, metabolic syndrome and obesity, bone disease (osteoporosis and osteopenia, stroke, lung cancer, cachexia, skeletal muscle weakness, anaemia, depression and cognitive decline. The assessment of COPD is required to determine the severity of the disease, its impact on the health status and the risk of future events (e.g., exacerbations, hospital admissions or death and this is essential to guide therapy. COPD is treated with inhaled bronchodilators, inhaled corticosteroids, oral theophylline and oral phosphodiesterase-4 inhibitor. Non pharmacological treatment of COPD includes smoking cessation, pulmonary rehabilitation and nutritional support. Lung volume reduction surgery and lung transplantation are advised in selected severe patients. Global strategy for the diagnosis, management and prevention of Chronic Obstructive Pulmonary Disease

Annular and central heavy pigment deposition on the posterior lens capsule in the pigment dispersion syndrome: pigment deposition on the posterior lens capsule in the pigment dispersion syndrome.

Science.gov (United States)

Turgut, Burak; Türkçüoğlu, Peykan; Deniz, Nurettin; Catak, Onur

2008-12-01

To report annular and central heavy pigment deposition on the posterior lens capsule in a case of pigment dispersion syndrome. Case report. A 36-year-old female with bilateral pigment dispersion syndrome presented with progressive decrease in visual acuity in the right eye over the past 1-2 years. Clinical examination revealed the typical findings of pigment dispersion syndrome including bilateral Krunkenberg spindles, iris transillumination defects, and dense trabecular meshwork pigmentation. Remarkably, annular and central dense pigmentation of the posterior lens capsule was noted in the right eye. Annular pigment deposition on the posterior lens capsule may be a rare finding associated with pigment dispersion syndrome. Such a finding suggests that there may be aqueous flow into the retrolental space in some patients with this condition. The way of central pigmentation is the entrance of aqueous to Berger's space. In our case, it is probable that spontaneous detachment of the anterior hyaloid membrane aided this entrance.

Pulmonary hypoplasia-diaphragmatic hernia-anophthalmia-cardiac defect (PDAC) syndrome due to STRA6 mutations--what are the minimal criteria?

Science.gov (United States)

Segel, Reeval; Levy-Lahad, Ephrat; Pasutto, Francesca; Picard, Elie; Rauch, Anita; Alterescu, Gheona; Schimmel, Michael S

2009-11-01

Microphthalmic syndrome 9 (OMIM601186) is a genetically and phenotypically variable condition, comprising anophthalmia, pulmonary hypoplasia, diaphragmatic hernia, and cardiac malformations (PDAC syndrome). Reported cases have all been associated with fetal/neonatal death or developmental delay. Recessive stimulated by retinoic acid gene 6 homolog (STRA6) mutations have recently been identified as the cause of cases of PDAC in which distinct, "bushy" eyebrows have been observed. We describe a patient with clinical anophthalmia, bushy eyebrows, patent ductus arteriosus, and normal development at age 30 months, who is a compound heterozygote for two novel STRA6 missense mutations. This patient's phenotype is consistent with the multisystemic malformations of PDAC syndrome, but is somewhat milder. This is the first living patient with compound heterozygous STRA6 mutations, which may explain her milder phenotype. We conclude that STRA6 analysis should be considered in all patients with clinical anophthalmia. Genetic counseling should be cautious with respect to long-term developmental outcomes. Copyright 2009 Wiley-Liss, Inc.

Serum Cytokine Profiles Differentiating Hemorrhagic Fever with Renal Syndrome and Hantavirus Pulmonary Syndrome

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Svetlana F. Khaiboullina

2017-05-01

Full Text Available Hantavirus infection is an acute zoonosis that clinically manifests in two primary forms, hemorrhagic fever with renal syndrome (HFRS and hantavirus pulmonary syndrome (HPS. HFRS is endemic in Europe and Russia, where the mild form of the disease is prevalent in the Tatarstan region. HPS is endemic in Argentina, as well as other countries of North and South American. HFRS and HPS are usually acquired via the upper respiratory tract by inhalation of virus-contaminated aerosol. Although the pathogenesis of HFRS and HPS remains largely unknown, postmortem tissue studies have identified endothelial cells as the primary target of infection. Importantly, cell damage due to virus replication, or subsequent tissue repair, has not been documented. Since no single factor has been identified that explains the complexity of HFRS or HPS pathogenesis, it has been suggested that a cytokine storm may play a crucial role in the manifestation of both diseases. In order to identify potential serological markers that distinguish HFRS and HPS, serum samples collected during early and late phases of the disease were analyzed for 48 analytes using multiplex magnetic bead-based assays. Overall, serum cytokine profiles associated with HPS revealed a more pro-inflammatory milieu as compared to HFRS. Furthermore, HPS was strictly characterized by the upregulation of cytokine levels, in contrast to HFRS where cases were distinguished by a dichotomy in serum cytokine levels. The severe form of hantavirus zoonosis, HPS, was characterized by the upregulation of a higher number of cytokines than HFRS (40 vs 21. In general, our analysis indicates that, although HPS and HFRS share many characteristic features, there are distinct cytokine profiles for these diseases. These profiles suggest a strong activation of an innate immune and inflammatory responses are associated with HPS, relative to HFRS, as well as a robust activation of Th1-type immune responses. Finally, the results

Pulmonary alveolar proteinosis in a child from an informal settlement

African Journals Online (AJOL)

Pulmonary alveolar proteinosis (PAP) is a syndrome characterised by respiratory failure caused by pulmonary surfactant accumulation and resulting in respiratory insu ciency and an increased incidence of infections.[1] e current standard therapy is whole- lung lavage, which is used to physically remove the accumulated.

Pulmonary Venous Obstruction in Cancer Patients

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Chuang-Chi Liaw

2015-01-01

Full Text Available Background. We study the clinical significance and management of pulmonary venous obstruction in cancer patients. Methods. We conducted a prospective cohort study to characterize the syndrome that we term “pulmonary vein obstruction syndrome” (PVOS between January 2005 and March 2014. The criteria for inclusion were (1 episodes of shortness of breath; (2 chest X-ray showing abnormal pulmonary hilum shadow with or without presence of pulmonary edema and/or pleural effusion; (3 CT scan demonstrating pulmonary vein thrombosis/tumor with or without tumor around the vein. Results. Two hundred and twenty-two patients developed PVOS. Shortness of breath was the main symptom, which was aggravated by chemotherapy in 28 (13%, and medical/surgical procedures in 21 (9% and showed diurnal change in intensity in 32 (14%. Chest X-rays all revealed abnormal pulmonary hilum shadows and presence of pulmonary edema in 194 (87% and pleural effusion in 192 (86%. CT scans all showed pulmonary vein thrombosis/tumor (100% and surrounding the pulmonary veins by tumor lesions in 140 patients (63%. PVOS was treated with low molecular weight heparin in combination with dexamethasone, and 66% of patients got clinical/image improvement. Conclusion. Physicians should be alert to PVOS when shortness of breath occurs and chest X-ray reveals abnormal pulmonary hilum shadows.

Hemorrhagic fever with renal syndrome accompanied by panhypopituitarism and central diabetes insipidus: a case report.

Science.gov (United States)

Ahn, Hee Jung; Chung, Jong-Hoon; Kim, Dong-Min; Yoon, Na-Ra; Kim, Choon-Mee

2018-03-05

Central diabetes insipidus (DI) was detected in a patient with hemorrhagic fever with renal syndrome (HFRS) who had been molecularly and serologically diagnosed with Hantaan virus infection. We recommend that clinicians differentiate central DI in HFRS patients with a persistent diuretic phase even when pituitary MRI findings are normal.

Late-onset and Rare Far-advanced Pulmonary Involvement in Patients with Sarcoidosis in Taiwan

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Chia-Wei Hsieh

2006-01-01

Conclusion: In this series, the mean age of disease onset was in middle age (mean, 47 years old, there was a low incidence of Löfgren's syndrome (3.6%, and no patients had advanced pulmonary syndrome. The results of this study also suggest that sACE might be a marker of pulmonary involvement that is also useful in monitoring disease activity.

Epidemiology, pathophysiology, and in-hospital management of pulmonary edema: data from the Romanian Acute Heart Failure Syndromes registry.

Science.gov (United States)

Chioncel, Ovidiu; Ambrosy, Andrew P; Bubenek, Serban; Filipescu, Daniela; Vinereanu, Dragos; Petris, Antoniu; Christodorescu, Ruxandra; Macarie, Cezar; Gheorghiade, Mihai; Collins, Sean P

2016-02-01

The objective of this study was to evaluate the clinical presentation, inpatient management, and in-hospital outcome of patients hospitalized for acute heart failure syndromes (AHFS) and classified as pulmonary edema (PE). The Romanian Acute Heart Failure Syndromes (RO-AHFS) study was a prospective, national, multicenter registry of all consecutive patients admitted with AHFS over a 12-month period. Patients were classified at initial presentation by clinician-investigators into the following clinical profiles: acute decompensated HF, cardiogenic shock, PE, right HF, or hypertensive HF. RO-AHFS enrolled 3224 patients and 28.7% (n = 924) were classified as PE. PE patients were more likely to present with pulmonary congestion, tachypnea, tachycardia, and elevated systolic blood pressure and less likely to have peripheral congestion and body weight increases. Mechanical ventilation was required in 8.8% of PE patients. PE patients received higher doses (i.e. 101.4 ± 27.1 mg) of IV furosemide for a shorter duration (i.e. 69.3 ± 22.3 hours). Vasodilators were given to 73.6% of PE patients. In-hospital all-cause mortality (ACM) in PE patients was 7.4%, and 57% of deaths occurred on day one. Increasing age, concurrent acute coronary syndromes, life-threatening ventricular arrhythmias, elevated BUN, left bundle branch block, inotrope therapy, and requirement for invasive mechanical ventilation were independent risk factors for ACM. In this national registry, the PE profile was found to be a high-acuity clinical presentation with distinctive treatment patterns and a poor short-term prognosis. Advances in the management of PE may necessitate both the development of novel targeted therapies as well as systems-based strategies to identify high-risk patients early in their course.

[Munchausen syndrome: need for centralized administrative health-care management].

Science.gov (United States)

Altmark, D; Sigal, M; Gelkopf, M

1991-03-01

The clinical picture of Munchausen syndrome is that of feigned or self-induced illness with the aim of being hospitalized and/or receiving unnecessary medical interventions, peregrination from 1 hospital to another, and disruptive behavior when hospitalized. These patients are a danger to themselves and heavily burden inpatient facilities that care for them. We present a case that illustrates the problems of diagnosing and managing such patients. We stress the need for adequate centralization and distribution of the relevant information concerning these patients.

Pulmonary arterial hypertension secondary to chronic thromboemboli

International Nuclear Information System (INIS)

Crysikopoulos, H.; Forrest, J.V.; Olson, L.K.; Roberts, A.C.

1989-01-01

The authors report their experience in 150 patients with pulmonary arterial hypertension secondary to chronic pulmonary embolism (CPE). Plain film findings are variable, commonly nonspecific. Occasionally nonuniformity of pulmonary perfusion or truncation of central pulmonary arteries suggest CPE. Multiple, large, unmatched perfusion defects are the most common scintigraphic observations. CT can exclude conditions mimicking CPE, particularly fibrosing mediastinitis. Pulmonary arteriography depicts the location and distribution of emboli. CPE is becoming an increasingly important entity to recognize because of available surgical treatment. Thromboendarterectomy may improve functional status and survival. The diagnosis should be considered in any patient with pulmonary arterial hypertension

Isolated Richter's syndrome in central nervous system: case report Sindrome de Richter isolada em sistema nervoso central: relato de caso

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Lucilene S.R. Resende

2005-06-01

Full Text Available Diffuse large cell non Hodgkin's lymphoma associated with chronic lymphoid leukemia (CLL, or Richter's syndrome, is a rare and serious complication. Isolated Richter's syndrome in the central nervous system is very rare; only 12 cases have been reported. We describe a 74-year-old patient with diffuse large cell non Hodgkin's lymphoma in the right frontal region with the appearance of multiform glioblastoma.Linfoma não Hodgkin difuso de grandes células em paciente portador de leucemia linfóide crônica (LLC, ou síndrome de Richter, é complicação rara e grave nesta leucemia. Síndrome de Richter isolada no sistema nervoso central é muito rara, tendo sido encontrados apenas 12 casos descritos. Descrevemos paciente de 74 anos, que apresentou linfoma não Hodgkin difuso de grandes células em região frontal direita, simulando glioblastoma multiforme.

Hepatopulmonary syndrome induced by common bile duct ligation in a rabbit model: correlation between pulmonary vascular dilatation on thin-section CT and angiography and serum nitrite concentration or endothelial nitric oxide synthase (eNOS)1 expression

International Nuclear Information System (INIS)

Lee, Ki Nam; Yoon, Seong Kuk; Lee, Jin Wha; Kim, Ki Nam; Park, Byung Ho; Kwak, Jong Young; Jeong, Jin Sook; Kim, Young Hoon

2004-01-01

To investigate the correlation between radiologic vascular dilatation and serum nitrite concentration and eNOS expression in the endothelial cell and pneumocyte in a rabbit model of hepatopulmonary syndrome induced by common bile duct ligation (CBDL). Thin-section CT scans of the lung and pulmonary angiography were obtained 3 weeks after CBDL (n=6), or a sham operation (n=4), and intrapulmonary vasodilatation was assessed. The diameter and tortuosity of peripheral vessels in the right lower lobe by thin-section CT and angiography at the same level of the right lower lobe in all subjects were correlated to serum nitrite concentration and eNOS (endothelial nitric oxide synthase) expression as determined by immunostaining. The diameters of pulmonary vessels on thin-section CT were well correlated with nitrite concentrations in serum (r=0.92, ρ < 0.001). Dilated pulmonary vessels were significantly correlated with an increased eNOS expression (r=0.94, ρ < 0.0001), and the severity of pulmonary vessel tortuosity was found to be well correlated with serum nitrite concentration (r=0.90, ρ < 0.001). The peripheral pulmonary vasculature in hepatopulmonary syndrome induced by CBLD was dilated on thin-section CT and on angiographs. Our findings suggest that peripheral pulmonary vascular dilatations are correlated with serum nitrite concentrations and pulmonary eNOS expression

Acute traumatic central cord syndrome: MRI-pathological correlations

International Nuclear Information System (INIS)

Quencer, R.M.; Bunge, R.P.; Egnor, M.; Green, B.A.; Puckett, W.; Naidich, T.P.; Post, M.J.D.; Norenberg, M.

1992-01-01

The acute traumatic central cord syndrome (ATCCS) is commonly stated to result from an injury which affects primarily the center of the spinal cord and is frequently hemorrhagic. To test the validity of this widely disseminated hypothesis, the magnetic resonance images [MRI] of 11 consecutive cases of ATCCS caused by closed injury to the spine were analyzed and correlated with the gross pathological and histological features of 3 cervical spinal cords obtained at post mortem from patients with ATCCS, including 2 of patients studied by MRI. In this study, the MRI and pathological observations indicate that ATCCS is predominantly a white matter injury and that intramedullary hemorrhage is not a necessary feature of the syndrome; indeed, it is probably an uncommon event in ATCCS. We suggest that the most common mechanism of injury in ATCCS may be direct compression of the cervical spinal cord by buckling of the ligamenta flava into an already narrowed cervical spinal canal; this would explain the predominance of axonal injury in the white matter of the lateral columns. (orig./GDG)

Acute traumatic central cord syndrome: MRI-pathological correlations

Energy Technology Data Exchange (ETDEWEB)

Quencer, R.M. (Dept. of Radiology, Univ. of Miami MRI Center, FL (United States) Miami Project to Cure Paralysis, FL (United States)); Bunge, R.P.; Egnor, M.; Green, B.A. (Miami Project to Cure Paralysis, FL (United States) Dept. of Neurological Surgery, Univ. of Miami School of Medicine, FL (United States)); Puckett, W. (Miami Project to Cure Paralysis, FL (United States)); Naidich, T.P. (Dept. of Radiology, Univ. of Miami MRI Center, FL (United States) Miami Project to Cure Paralysis, FL (United States) Baptist Hospital of Greater Miami, FL (United States)); Post, M.J.D. (Dept. of Radiology, Univ. of Miami MRI Center, FL (United States)); Norenberg, M. (Dept. of Neuropathology, Univ. of Miami School of Medicine, FL (United States))

1992-04-01

The acute traumatic central cord syndrome (ATCCS) is commonly stated to result from an injury which affects primarily the center of the spinal cord and is frequently hemorrhagic. To test the validity of this widely disseminated hypothesis, the magnetic resonance images [MRI] of 11 consecutive cases of ATCCS caused by closed injury to the spine were analyzed and correlated with the gross pathological and histological features of 3 cervical spinal cords obtained at post mortem from patients with ATCCS, including 2 of patients studied by MRI. In this study, the MRI and pathological observations indicate that ATCCS is predominantly a white matter injury and that intramedullary hemorrhage is not a necessary feature of the syndrome; indeed, it is probably an uncommon event in ATCCS. We suggest that the most common mechanism of injury in ATCCS may be direct compression of the cervical spinal cord by buckling of the ligamenta flava into an already narrowed cervical spinal canal; this would explain the predominance of axonal injury in the white matter of the lateral columns. (orig./GDG).

Percutaneous removal of pulmonary artery emboli with hydrolyser catheter in pigs

International Nuclear Information System (INIS)

Lacoursiere, L.; Millward, S.; Veinot, J.P.; Labinaz, M.

2001-01-01

To evaluate the efficacy and safety of the Hydrolyser catheter for per,cutaneous treatment of massive pulmonary embolism in pigs. Twelve pigs, each weighing between 55 kg and 89 kg, were used. Radio-opaque 9 cm x 0.8 cm and 4.5 cm x 0.8 cm clots, produced by mixing pig blood with iodinated contrast agent in vacutainers, were injected via the jugular vein until central pulmonary embolism (main and proximal lobar arteries) was obtained with significant systemic and pulmonary hemodynamic modifications. From a femoral approach, the 7-French Hydrolyser thrombectomy catheter was run over a 0.025-inch (0.64-mm) guide wire to remove the pulmonary emboli. Hemodynamic, gasometric and angiographic monitoring was performed before and after treatment. The procedure's safety and completeness of emboli removal was assessed by cardiopulmonary autopsy. Three of the 12 pigs died during embolization. Thrombectomy was therefore performed in 9, and central emboli could be obtained in 7 of the 9. The Hydrolyser could be manipulated only in central pulmonary arteries and could aspirate only central emboli in 5 of the 7 pigs that had them. Despite minimal angiographic improvement seen in these 5, there was no significant hemodynamic and gasometric improvement after treatment. The procedure induced an increase in free hemoglobin blood levels. Autopsies revealed an average of 2 endothelial injuries per pig (mainly adherent endocardial thrombi) in both nontreated (n = 3) and Hydrolyser-treated (n = 9) groups. The Hydrolyser thrombectomy catheter can be promptly positioned and easily steered in central pulmonary arteries. It can be used to partially remove central emboli, but not peripheral pulmonary emboli. Most of the injuries observed may not have been strictly related to Hydrolyser use. The pig might not be a suitable animal model for treatment of massive pulmonary embolism. (author)

Computerized axial tomography of the chest for visualization of ''absent'' pulmonary arteries

International Nuclear Information System (INIS)

Sondheimer, H.M.; Oliphant, M.; Schneider, B.; Kavey, R.E.W.; Blackman, M.S.; Parker, F.B. Jr.

1982-01-01

To expand the search for central pulmonary arteries in six patients with absence of cardiac-pulmonary continuity, computerized axial tomography (CAT) of the chest was performed. The CAT scans were compared with previous arteriograms and pulmonary vein wedge angiograms. Three patients with type IV truncus arteriosus were studied, and none had a central, right or left pulmonary artery on CAT scan. However, two patients with tetralogy of Fallot with pulmonary atresia and a patent ductus arteriosus to the right lung demonstrated the presence of a left pulmonary artery. In addition, one child with truncus arteriosus with ''absent'' left pulmonary artery demonstrated a left pulmonary artery on the CAT scan. The CAT scan may therefore enhance our ability to search for disconnected pulmonary arteries in children with complex cyanotic congenital heart disease

Dynamic changes of central thyroid functions in the management of Cushing's syndrome.

Science.gov (United States)

Dogansen, Sema Ciftci; Yalin, Gulsah Yenidunya; Canbaz, Bulent; Tanrikulu, Seher; Yarman, Sema

2018-01-01

The aim of this study was to determine the frequency of central thyroid dysfunctions in Cushing's syndrome (CS). We also aimed to evaluate the frequency of hyperthyroidism due to the syndrome of the inappropriate secretion of TSH (SITSH), which was recently defined in patients with insufficient hydrocortisone replacement after surgery. We evaluated thyroid functions (TSH and free thyroxine [fT4]) at the time of diagnosis, during the hypothalamo-pituitary-adrenal axis recovery, and after surgery in 35 patients with CS. The patients were separated into two groups: ACTH-dependent CS (group 1, n = 20) and ACTH-independent CS (group 2, n = 15). Patients' clinical and laboratory findings were evaluated in five visits in the outpatient clinic of the endocrinology department. The frequency of baseline suppressed TSH levels and central hypothyroidism were determined to be 37% (n = 13) and 26% (n = 9), respectively. A negative correlation was found between baseline cortisol and TSH levels (r = -0.45, p = 0.006). All patients with central hypothyroidism and suppressed TSH levels showed recovery at the first visit without levothyroxine treatment. SITSH was not detected in any of the patients during the postoperative period. No correlation was found between prednisolone replacement after surgery and TSH or fT4 levels on each visit. Suppressed TSH levels and central hypothyroidism may be detected in CS, independent of etiology. SITSH was not detected in the early postoperative period due to our adequate prednisolone replacement doses.

Percutaneous balloon dilation of pulmonary stenosis

International Nuclear Information System (INIS)

Hua Yangde; Huang Ming; Li Jinkang; Qian Jinqing; Chen Xiuyu; Yang Siyuan

2003-01-01

Objective: Review our experience of balloon dilation of valvular pulmonary stenosis in 32 cases. Methods: Totally 32 cases of pulmonary stenosis admitted from 1995-2001 with age of 1.5-13 yrs mean 6.8. Diagnosis was made by clinical manifestations, EKG, ECHO and angiocardiography. Results: Before dilation, the mean systolic pressure of right ventricle was (93.5 ± 28.5) mmHg, after the procedure it reduced to (42 ± 9.0) mmHg. The pressure gradient between right ventricle and pulmonary artery before dilation was (76 ± 30) mmHg and become (24.5 ± 8.5) mmHg after dilation. The gradient pressure after dilation was less than 25 mmHg in 90.6% cases. A case of Noonan syndrome showed no response to balloon dilation and died during valvulectomy from accompanying left ventricular cardiomyopathy. Conclusions: Balloon dilation of valvular pulmonary stenosis is effective and safe. The selection of proper diameter of pulmonary valvular rings and sized of the balloon are the major factors

Simple pulmonary eosinophilia (loeffler's syndrome): chest radiographic and CT findings

Energy Technology Data Exchange (ETDEWEB)

Jung, Kyung Jae; Lee, Kyung Soo; Kim, Tae Sung; Chung, Man Pyo; Choi, Dong Chull; Kwon, O Jung [Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul (Korea, Republic of)

2000-01-01

The purpose of our study is to describe the chest radiographic and CT findings of simple pulmonary eosinophilia. Twenty-six patients with simple pulmonary eosinophilia underwent chest radiography and CT scanning; the results were analyzed retrospectively by two chest radiologists, focusing on the patterns and distribution of the parenchymal abnormalities. The chest radiographs were normal in eight patients (31%), while among the remaining 18 patients, they showed subtle opacity (n=3D9), nodules (n=3D8), consolidation (n=3D2), and mass (n=3D1). Follow-up chest radiographs (m=3D18) demonstrated complete (n=3D16) or partial (n=3D1) resolution of parenchymal lesions or migratory lesions (n=3D1). On CT, nodule(s) (n=3D19) were most commonly seen, followed by ground-glass opacity (n=3D16), consolidation (n=3D3), and mass (n=3D1). A peripheral halo surrounding a nodule or an area of consolidation was seen in 18 patients. The nodules(s) (n=3D19) were subpleural (n=3D13) or random (=3D6). Areas of ground-glass opacity (n=3D16) were subpleural (n=3D13), random (n=3D2), or central (n=3D1). All lesions were patchy rather than diffuse. Follow-up CT in nine patients showed complete (n=3D7) or partial (n=3D2) resolution of parenchymal lesions. Chest radiographs of patients with simple pulmonary eosinophilia often reveal no abnormality. The most common finding is subtle opacity or nodule(s), while CT reveals transient nodule(s) with a surrounding halo or transient areas of ground glass opacity. (author)

Pulmonary abscess

International Nuclear Information System (INIS)

Valencia Chavez, Maria de la Cruz

2000-01-01

Pulmonary abscess is defined as a suppurative process and bounded, caused by piogens organisms that it progresses to central necrosis and it commits an or more areas of the pulmonary parenchyma. Initially it is impossible to differ of a located pneumonia, but when the lesion communicates with a bronchus, part of the neurotic tissue is replaced by air, producing the classic image radiological fluid-air. The presence of multiple lesions smaller than 2 cms of diameter cm is defined arbitrarily as necrotizing pneumonia it is indistinguishable of an abscess. The paper includes the pathogenesis and etiology, clinical course, diagnostic and treatment

An Isolated Pulmonary Hematoma Mimicking a Lung Tumor as the Initial Finding of Vascular Ehlers-Danlos Syndrome

Energy Technology Data Exchange (ETDEWEB)

Kang, Eun Ju; Lee, Ki Nam; Choi, Pil Jo [Dept. of Radiology, Dong-A University Medicine Center, Dong-A University College of Medicine, Busan (Korea, Republic of); Ki, Chang Seok [Dept. of Radiology, Sungkyunkwan University School of Medicine, Seoul (Korea, Republic of)

2012-09-15

The vascular type of Ehlers-Danlos syndrome (vEDS) is an uncommon inherited disorder characterized by abnormalities in type III collagen, presenting itself as arterial dissection or rupture. We report a case of an isolated pulmonary hematoma mimicking a lung tumor in an 18-year-old man which turned out to be the initial finding of vEDS. Pneumothorax and hemothorax occurred repeatedly for 15 months following the surgical removal of the mass, and were treated by repeated left upper and lower lobectomy and thoracotomy. The diagnosis of vEDS was confirmed by pathologic and genetic studies.

An Isolated Pulmonary Hematoma Mimicking a Lung Tumor as the Initial Finding of Vascular Ehlers-Danlos Syndrome

International Nuclear Information System (INIS)

Kang, Eun Ju; Lee, Ki Nam; Choi, Pil Jo; Ki, Chang Seok

2012-01-01

The vascular type of Ehlers-Danlos syndrome (vEDS) is an uncommon inherited disorder characterized by abnormalities in type III collagen, presenting itself as arterial dissection or rupture. We report a case of an isolated pulmonary hematoma mimicking a lung tumor in an 18-year-old man which turned out to be the initial finding of vEDS. Pneumothorax and hemothorax occurred repeatedly for 15 months following the surgical removal of the mass, and were treated by repeated left upper and lower lobectomy and thoracotomy. The diagnosis of vEDS was confirmed by pathologic and genetic studies.

Eosinofilia pulmonar Pulmonary eosinophilia

Directory of Open Access Journals (Sweden)

Luiz Eduardo Mendes Campos

2009-06-01

and treatment. Clinical and radiological presentations can include simple pulmonary eosinophilia, chronic eosinophilic pneumonia, acute eosinophilic pneumonia, allergic bronchopulmonary aspergillosis and pulmonary eosinophilia associated with a systemic disease, such as in Churg-Strauss syndrome and hypereosinophilic syndrome. Asthma is frequently concomitant and can be a prerequisite, as in allergic bronchopulmonary aspergillosis and Churg-Strauss syndrome. In diseases with systemic involvement, the skin, the heart and the nervous system are the most affected organs. The radiological presentation can be typical, or at least suggestive, of one of three types of pulmonary eosinophilia: chronic eosinophilic pneumonia, acute eosinophilic pneumonia and allergic bronchopulmonary aspergillosis. The etiology of pulmonary eosinophilia can be either primary (idiopathic or secondary, due to known causes, such as drugs, parasites, fungal infection, mycobacterial infection, irradiation and toxins. Pulmonary eosinophilia can be also associated with diffuse lung diseases, connective tissue diseases and neoplasia.

Pulmonary vasculitis may obscure large cell lung carcinoma. A case report

NARCIS (Netherlands)

Kreeftenberg, HG; Ligtenberg, JJM; van der Werf, TS; Timens, W; Tervaert, JWC

2001-01-01

Several vasculitic syndromes are recognized as paraneoplastic symdromes of an underlying malignant disease. Most frequently small vessel vasculitis of the skin has been reported. We describe the case of a 62-year-old man with a pulmonary mass due to pulmonary vasculitis. After resection of the

Pulmonary vascular complications in portal hypertension and liver disease: A concise review

Directory of Open Access Journals (Sweden)

M. Porres-Aguilar

2013-01-01

Full Text Available Chronic liver disease and/or portal hypertension may be associated with one of the two pulmonary vascular complications: portopulmonary hypertension and hepatopulmonary syndrome. These pulmonary vascular disorders are notoriously underdiagnosed; however, they have a substantial negative impact on survival and require special attention in order to understand their diagnostic approach and to select the best therapeutic options. Portopulmonary hypertension results from excessive vasoconstriction, vascular remodeling, and proliferative and thrombotic events within the pulmonary circulation that lead to progressive right ventricular failure and ultimately to death. On the other hand, abnormal intrapulmonary vascular dilations, profound hypoxemia, and a wide alveolar-arterial gradient are the hallmarks of the hepatopulmonary syndrome, resulting in difficult-to-treat hypoxemia. The aim of this review is to summarize the latest pathophysiologic concepts, diagnostic approach, therapy, and prognosis of portopulmonary hypertension and hepatopulmonary syndrome, as well as to discuss the role of liver transplantation as a definitive therapy in selected patients with these conditions.

Central retinal artery occlusion in a patient with ANCA-negative Churg-Strauss syndrome

Science.gov (United States)

Kumano, Yuji; Yoshida, Noriko; Fukuyama, Satoru; Miyazaki, Masanori; Enaida, Hiroshi; Matsui, Takaaki

2012-01-01

Ocular involvement in Churg-Strauss syndrome is infrequent. We describe the case of a 54-year-old woman with eosinophilia and involvement of the respiratory tract, skin, and peripheral nervous system, fulfilling the American College of Rheumatology criteria for Churg-Strauss syndrome. The patient presented with acute, painless vision loss in her right eye. Central retinal artery occlusion (CRAO) without accompanying retinal vasculitis was diagnosed by angiographic findings and funduscopic findings of retinal whitening with a cherry-red spot. Although her antineutrophil cytoplasmic antibody (ANCA) status was negative, CRAO was thought to be an ocular manifestation of Churg-Strauss syndrome, and appropriate treatment was planned. She was treated with high-dose corticosteroids and anticoagulant therapy. Her macular edema improved, but visual recovery was poor. Specific therapy to alter inflammation, blood coagulation, and rheology reportedly plays an important role in ANCA-positive patients with Churg-Strauss syndrome who develop CRAO. Regardless of ANCA status, high-dose corticosteroids should be considered for CRAO in patients with Churg-Strauss syndrome, as discussed in this case. PMID:22927731

Preschool Weight and Body Mass Index in Relation to Central Obesity and Metabolic Syndrome in Adulthood

DEFF Research Database (Denmark)

Graversen, Lise; Sørensen, Thorkild I A; Petersen, Liselotte

2014-01-01

BACKGROUND: If preschool measures of body size routinely collected at preventive health examinations are associated with adult central obesity and metabolic syndrome, a focused use of these data for the identification of high risk children is possible. The aim of this study was to test the associ......BACKGROUND: If preschool measures of body size routinely collected at preventive health examinations are associated with adult central obesity and metabolic syndrome, a focused use of these data for the identification of high risk children is possible. The aim of this study was to test...... the associations between preschool weight and body mass index (BMI) and adult BMI, central obesity and metabolic alterations. METHODS: The Northern Finland Birth Cohort 1966 (NFBC1966) (N = 4111) is a population-based cohort. Preschool weight (age 5 months and 1 year) and BMI (age 2-5 years) were studied...... in relation to metabolic syndrome as well as BMI, waist circumference, lipoproteins, blood pressure, and fasting glucose at the age of 31 years. Linear regression models and generalized linear regression models with log link were used. RESULTS: Throughout preschool ages, weight and BMI were significantly...

Progress in study on central nervous system injuries caused by obstructive sleep apnea syndrome

Directory of Open Access Journals (Sweden)

ZHAO Xiang-xiang

2013-05-01

Full Text Available Chronic and repetitive intermittent hypoxia and dysfunction of sleep architecture mainly contribute to obstructive sleep apnea syndrome (OSAS. More and more evidences demonstrate it is a systemic disease, which is common encountered in clinic and strongly related to the systemic lesion of central nervous system. The central nervous system complications comprise cognitive impairment, brain atrophy and the growing risk of stroke and so on. Early treatment for OSAS has a positive significance on complications of central nervous system, and even the damage can be completely reversed.

Vascular Ehlers-Danlos syndrome with cryptorchidism, recurrent pneumothorax, and pulmonary capillary hemangiomatosis-like foci: A case report.

Science.gov (United States)

Park, Min A; Shin, So Youn; Kim, Young Jin; Park, Myung Jae; Lee, Seung Hyeun

2017-11-01

Vascular Ehlers-Danlos syndrome (vEDS) is a rare autosomal dominant inherited collagen disorder caused by defects or deficiency of pro-alpha 1 chain of type III procollagen encoded by COL3A1. vEDS is characterized not only by soft tissue manifestations including hyperextensibility of skin and joint hypermobility but also by early mortality due to rupture of arteries or vital organs. Although pulmonary complications are not common, vEDS cases complicated by pneumothorax, hemothorax, or intrapulmonary hematoma have been reported. When a patient initially presents only with pulmonary complications, it is not easy for clinicians to suspect vEDS. We report a case of an 18-year-old high school student, with a past history of cryptorchidism, presenting with recurrent pneumothorax. Routine laboratory findings were unremarkable. Chest high resolution computed tomographic scan showed age-unmatched hyperinflation of both lungs, atypical cystic changes and multifocal ground glass opacities scattered in both lower lobes. His slender body shape, hyperflexible joints, and hyperextensible skin provided clue to suspicion of a possible connective tissue disorder. The histological examination of the lung lesions showed excessive capillary proliferation in the pulmonary interstitium and pleura allowing the diagnosis of pulmonary capillary hemangiomatosis (PCH)-like foci. Genetic study revealed COL3A1 gene splicing site mutation confirming his diagnosis as vEDS. Although his diagnosis vEDS is notorious for fatal vascular complication, there was no evidence of such complication at presentation. Fortunately, he has been followed up for 10 months without pulmonary or vascular complications. To the best of our knowledge, both cryptorchidism and PCH-like foci have never been reported yet as complications of vEDS, suggesting our case might be a new variant of this condition. This case emphasizes the importance of comprehensive physical examination and history-taking, and the clinical

Pulmonary endothelial activation caused by extracellular histones contributes to neutrophil activation in acute respiratory distress syndrome.

Science.gov (United States)

Zhang, Yanlin; Guan, Li; Yu, Jie; Zhao, Zanmei; Mao, Lijun; Li, Shuqiang; Zhao, Jinyuan

2016-11-21

During the acute respiratory distress syndrome (ARDS), neutrophils play a central role in the pathogenesis, and their activation requires interaction with the endothelium. Extracellular histones have been recognized as pivotal inflammatory mediators. This study was to investigate the role of pulmonary endothelial activation during the extracellular histone-induced inflammatory response in ARDS. ARDS was induced in male C57BL/6 mice by intravenous injection with lipopolysaccharide (LPS) or exogenous histones. Concurrent with LPS administration, anti-histone H4 antibody (anti-H4) or non-specific IgG was administered to study the role of extracellular histones. The circulating von Willebrand factor (vWF) and soluble thrombomodulin (sTM) were measured with ELISA kits at the preset time points. Myeloperoxidase (MPO) activity in lung tissue was measured with a MPO detection kit. The translocation of P-selectin and neutrophil infiltration were measured by immunohistochemical detection. For in vitro studies, histone H4 in the supernatant of mouse lung vascular endothelial cells (MLVECs) was measured by Western blot. The binding of extracellular histones with endothelial membrane was examined by confocal laser microscopy. Endothelial P-selectin translocation was measured by cell surface ELISA. Adhesion of neutrophils to MLVECs was assessed with a color video digital camera. The results showed that during LPS-induced ARDS extracellular histones caused endothelial and neutrophil activation, as seen by P-selectin translocation, release of vWF, an increase of circulating sTM, lung neutrophil infiltration and increased MPO activity. Extracellular histones directly bound and activated MLVECs in a dose-dependent manner. On the contrary, the direct stimulatory effect of exogenous histones on neutrophils was very limited, as measured by neutrophil adhesion and MPO activity. With the contribution of activated endothelium, extracellular histones could effectively activating

Scimitar syndrome: multi detector computed tomography angiography findings of a case

International Nuclear Information System (INIS)

Aslan, A.; Bozlar, U.; Ors, F.; Tasar, M.

2012-01-01

Full text: Introduction: Scimitar syndrome also known as pulmonary venolobar syndrome is a rare congenital abnormality. This abnormality consists of ipsilateral anomalous pulmonary venous drainage of right lung into the inferior vena cava (IVC), with hypoplasia of the right lung, an anomalous systemic arterial supply from supradiaphragmatic aorta to the right lower lobe and dextrocardia. Objectives and tasks: In this article, we aimed to present multidetector computed tomography (MDCT) angiography findings of a case with scimitar syndrome. Material and methods: 21 year old male with dextrocardia and anomalous curvilinear density in the right lower lobe directed toward the right hemidiaphragm on chest radiography was evaluated with MDCT angiography examination. Results: Dextrocardia and interruption of IVC with azygous continuation was detected in MDCT angiography examination. Vertically directing right superior pulmonary vein was draining to the suprahepatic segment of IVC. Pulmonary sequestration in the right lower lobe taking feeder from celiac trunk was detected. Right pulmonary artery was hypoplastic and bronchial tree was abnormal, manifesting as a mirror image in both lung lobes. Conclusion: MDCT angiography is very successful imaging method in demonstrating the anomalous pulmonary vein and assessing pulmonary arterial and bronchial anomalies

Chronic obstructive pulmonary disease may be one of the terminal end points of metabolic syndrome

International Nuclear Information System (INIS)

Helvaci, M.R.; Aydin, L.Y.; Aydin, Y.

2012-01-01

Objective: We tried to understand presence of any effect of excess weight on respiratory system by means of excessive adipose tissue functioning as an endocrine organ and causing a Methodology: Mild (stage 1), moderate (stage 2), and severe (stage 3 and 4) chronic obstructive pulmonary disease (COPD) patients were detected, and compared according to the metabolic parameters in between. Results: There were 145, 56, and 34 patients in the mild, moderate, and severe COPD groups, respectively. The mean age increased gradually (52.4, 56.4, and 60.0 years) from the mild towards the severe COPD groups, respectively (p<0.05 nearly in all steps). Similarly, the mean direction (p<0.05 nearly in all steps). Parallel to them, the mean body mass index increased Conclusion: The metabolic syndrome includes some reversible indicators such as overweight, hyperbetalipoproteinemia, hypertriglyceridemia, dyslipidemia, impaired fasting glucose, impaired glucose tolerance, and white coat hypertension for the development of terminal diseases including obesity, hypertension, diabetes mellitus, peripheral artery disease, coronary heart disease, and stroke. In our opinion, COPD may be one of the terminal end points of the syndrome. (author)

Chronic obstructive pulmonary disease may be one of the terminal end points of metabolic syndrome

Energy Technology Data Exchange (ETDEWEB)

Helvaci, M R; Aydin, L Y; Aydin, Y

2012-04-15

Objective: We tried to understand presence of any effect of excess weight on respiratory system by means of excessive adipose tissue functioning as an endocrine organ and causing a Methodology: Mild (stage 1), moderate (stage 2), and severe (stage 3 and 4) chronic obstructive pulmonary disease (COPD) patients were detected, and compared according to the metabolic parameters in between. Results: There were 145, 56, and 34 patients in the mild, moderate, and severe COPD groups, respectively. The mean age increased gradually (52.4, 56.4, and 60.0 years) from the mild towards the severe COPD groups, respectively (p<0.05 nearly in all steps). Similarly, the mean direction (p<0.05 nearly in all steps). Parallel to them, the mean body mass index increased Conclusion: The metabolic syndrome includes some reversible indicators such as overweight, hyperbetalipoproteinemia, hypertriglyceridemia, dyslipidemia, impaired fasting glucose, impaired glucose tolerance, and white coat hypertension for the development of terminal diseases including obesity, hypertension, diabetes mellitus, peripheral artery disease, coronary heart disease, and stroke. In our opinion, COPD may be one of the terminal end points of the syndrome. (author)

Different methods of tomography in destructive pulmonary tuberculosis

International Nuclear Information System (INIS)

Volodina, G.I.; Semenov, V.M.

1980-01-01

Altogether 203 patients (186 with destructive pulmonary tuberculosis, 11 with other forms of tuberculosis of respiratory tract organs, 6 with lung malignant tumor) were examined with the use of different modifications of tomography: longitudinal and oblique blurring, zonography, selective tomography. Standardization in the use of different methods is proposed, depending on the intensity of the main syndromes of pulmonary tissue lesions: limited shading, foci, dissemination, caverns, etc. The informativeness is greatly increased when the proposed algorithm of examination is used both at the disease onset and during the follow-up of patients with destructive pulmonary tuberculosis

Pulmonary Mechanics and Mortality in Mechanically Ventilated Patients Without Acute Respiratory Distress Syndrome: A Cohort Study.

Science.gov (United States)

Fuller, Brian M; Page, David; Stephens, Robert J; Roberts, Brian W; Drewry, Anne M; Ablordeppey, Enyo; Mohr, Nicholas M; Kollef, Marin H

2018-03-01

Driving pressure has been proposed as a major determinant of outcome in patients with acute respiratory distress syndrome (ARDS), but there is little data examining the association between pulmonary mechanics, including driving pressure, and outcomes in mechanically ventilated patients without ARDS. Secondary analysis from 1,705 mechanically ventilated patients enrolled in a clinical study that examined outcomes associated with the use of early lung-protective mechanical ventilation. The primary outcome was mortality and the secondary outcome was the incidence of ARDS. Multivariable models were constructed to: define the association between pulmonary mechanics (driving pressure, plateau pressure, and compliance) and mortality; and evaluate if driving pressure contributed information beyond that provided by other pulmonary mechanics. The mortality rate for the entire cohort was 26.0%. Compared with survivors, non-survivors had significantly higher driving pressure [15.9 (5.4) vs. 14.9 (4.4), P = 0.005] and plateau pressure [21.4 (5.7) vs. 20.4 (4.6), P = 0.001]. Driving pressure was independently associated with mortality [adjusted OR, 1.04 (1.01-1.07)]. Models related to plateau pressure also revealed an independent association with mortality, with similar effect size and interval estimates as driving pressure. There were 152 patients who progressed to ARDS (8.9%). Along with driving pressure and plateau pressure, mechanical power [adjusted OR, 1.03 (1.00-1.06)] was also independently associated with ARDS development. In mechanically ventilated patients, driving pressure and plateau pressure are risk factors for mortality and ARDS, and provide similar information. Mechanical power is also a risk factor for ARDS.

Pulmonary candidiasis and CD4 count in HIV positive patients seen ...

African Journals Online (AJOL)

Pulmonary candidiasis and CD4 count in HIV positive patients seen in Jos, north central Nigeria. YJ Peter, AH Isa, AS Anzaku, MI Builders. Abstract. Background: Accurate and reliable diagnosis of HIV opportunistic infections plays a central role in effective HIV intervention programmes. Pulmonary infections are the leading ...

Radioaerosol Inhalation Lung Scan in Pulmonary Emphysema

Energy Technology Data Exchange (ETDEWEB)

Jeon, Jeong Soo; Park, Yong Ha; Kyo, Chung Soo; Bahk, Yong Whee [Catholic University College of Medicine, Seoul (Korea, Republic of)

1990-07-15

Perfusion and ventilation imagings of the lung are well established procedure for diagnosing pulmonary embolism, differentiation it from chronic obstructive lung disease, and making an early detection of chronic obstructive lung disease. To evaluate the usefulness of radioaerosol inhalation imaging (RII) in chronic obstructive lung disease, especially pulmonary emphysema, we analyzed RIIs of five normal adult non-smokers, five asymptomatic smokers (age 25-42 years with the mean 36), and 21 patients with pulmonary emphysema (age 59-78 years with the mean 67). Scintigrams were obtained with radioaerosol produced by a BARC nebuliser with 15 mCi of {sup 99m}Tc-phytate. Scanning was performed in the anterior, posterior, and lateral projections after five to 10-minute inhalation of the radioaerosol on sitting position. The scans were analyzed and correlated with the results of pulmonary function studies and chest radiographs. Also lung perfusion scan with {sup 99m}Tc-MAA was performed in 12 patients. In five patients, we performed follow-up scans for the evaluation of the effects of a bronchodilator. Based on the X-ray findings and clinical symptoms, pulmonary emphysema was classified into four types: centrilobular (3 patients), panlobular (4 patients), intermediate (10 patients), and combined (4 patients). RII findings were patternized according to the type, extent, and intensity of the aerosol deposition in the central bronchial and bronchopulmonary system and lung parenchyma. 10 controls, normal five non-smokers and three asymptomatic smokers revealed homogeneous parenchymal deposition in the entire lung fields without central bronchial deposition. The remaining two of asymptomatic smokers revealed mild central airway deposition. The great majority of the patients showed either central (9/21) or combined type (10/21) of bronchopulmonary deposition and the remaining two patients peripheral bronchopulmonary deposition. Parenchymal aerosol deposition in pulmonary

Radioaerosol Inhalation Lung Scan in Pulmonary Emphysema

International Nuclear Information System (INIS)

Jeon, Jeong Soo; Park, Yong Ha; Chung Soo Kyo; Bahk, Yong Whee

1990-01-01

Perfusion and ventilation imagings of the lung are well established procedure for diagnosing pulmonary embolism, differentiation it from chronic obstructive lung disease, and making an early detection of chronic obstructive lung disease. To evaluate the usefulness of radioaerosol inhalation imaging (RII) in chronic obstructive lung disease, especially pulmonary emphysema, we analyzed RIIs of five normal adult non-smokers, five asymptomatic smokers (age 25-42 years with the mean 36), and 21 patients with pulmonary emphysema (age 59-78 years with the mean 67). Scintigrams were obtained with radioaerosol produced by a BARC nebuliser with 15 mCi of 99m Tc-phytate. Scanning was performed in the anterior, posterior, and lateral projections after five to 10-minute inhalation of the radioaerosol on sitting position. The scans were analyzed and correlated with the results of pulmonary function studies and chest radiographs. Also lung perfusion scan with 99m Tc-MAA was performed in 12 patients. In five patients, we performed follow-up scans for the evaluation of the effects of a bronchodilator. Based on the X-ray findings and clinical symptoms, pulmonary emphysema was classified into four types: centrilobular (3 patients), panlobular (4 patients), intermediate (10 patients), and combined (4 patients). RII findings were patternized according to the type, extent, and intensity of the aerosol deposition in the central bronchial and bronchopulmonary system and lung parenchyma. 10 controls, normal five non-smokers and three asymptomatic smokers revealed homogeneous parenchymal deposition in the entire lung fields without central bronchial deposition. The remaining two of asymptomatic smokers revealed mild central airway deposition. The great majority of the patients showed either central (9/21) or combined type (10/21) of bronchopulmonary deposition and the remaining two patients peripheral bronchopulmonary deposition. Parenchymal aerosol deposition in pulmonary emphysema was

Cushing's Syndrome and Hypothalamic-Pituitary-Adrenal Axis Hyperactivity in Chronic Central Serous Chorioretinopathy.

Science.gov (United States)

van Haalen, Femke M; van Dijk, Elon H C; Dekkers, Olaf M; Bizino, Maurice B; Dijkman, Greet; Biermasz, Nienke R; Boon, Camiel J F; Pereira, Alberto M

2018-01-01

Central serous chorioretinopathy (CSC), a specific form of macular degeneration, has been reported as presenting manifestation of Cushing's syndrome. Furthermore, CSC has been associated with both exogenous hypercortisolism and endogenous Cushing's syndrome. It is important to know whether CSC patients should be screened for Cushing's syndrome. Although hypothalamic-pituitary-adrenal (HPA) axis hyperactivity in CSC has been suggested, no detailed evaluation of the HPA axis has been performed in a large cohort of CSC patients. This study aimed to investigate whether Cushing's syndrome prevalence is increased among chronic CSC (cCSC) patients and whether detailed endocrinological phenotyping indicates hyperactivity of the HPA axis. Cross-sectional study. 86 cCSC patients and 24 controls. Prevalence of Cushing's syndrome, HPA axis activity. None of the cCSC patients met the clinical or biochemical criteria of Cushing's syndrome. However, compared to controls, HPA axis activity was increased in cCSC patients, reflected by higher 24 h urinary free cortisol, and accompanying higher waist circumference and diastolic blood pressure, whereas circadian cortisol rhythm and feedback were not different. Chronic CSC patients did not report more stress or stress-related problems on questionnaires. No case of Cushing's syndrome was revealed in a large cohort of cCSC patients. Therefore, we advise against screening for Cushing's syndrome in CSC patients, unless additional clinical features are present. However, our results indicate that cCSC is associated with hyperactivity of the HPA axis, albeit not accompanied with perception of more psychosocial stress.

Iloprost Instillation in Two Neonates with Pulmonary Hypertension

International Nuclear Information System (INIS)

Aktas, S.; Aksu, M.; Hirfanoglu, I. M.; Atalay, Y.; Turkyilmaz, C.; Unal, S.

2017-01-01

Pulmonary hypertension may coexist with certain diseases in neonates. Iloprost inhalation is one of the treatments which cause selective pulmonary vasodilatation. Inhalation is not an easy way of drug administration in mechanically ventilated infants; as some exhibit desaturations during inhalation. Moreover, inhalation of drug requires cessation of mechanical ventilation, if patient is on high frequency oscillatory ventilation. We presented two patients with pulmonary hypertension; term baby with congenital diaphragmatic hernia and preterm baby with respiratory distress syndrome; who had iloprost instillation during mechanical ventilation treatment. Iloprost instillation was well tolerated with no side effects in the term patient with diaphragmatic hernia; whereas severe blood pressure fluctuations were observed in the preterm infant. This report may courage administration of iloprost in term neonates with resistant pulmonary hypertension. (author)

Lung-Protective Ventilation With Low Tidal Volumes and the Occurrence of Pulmonary Complications in Patients Without Acute Respiratory Distress Syndrome: A Systematic Review and Individual Patient Data Analysis

NARCIS (Netherlands)

Neto, Ary Serpa; Simonis, Fabienne D.; Barbas, Carmen S. V.; Biehl, Michelle; Determann, Rogier M.; Elmer, Jonathan; Friedman, Gilberto; Gajic, Ognjen; Goldstein, Joshua N.; Linko, Rita; Pinheiro de Oliveira, Roselaine; Sundar, Sugantha; Talmor, Daniel; Wolthuis, Esther K.; Gama de Abreu, Marcelo; Pelosi, Paolo; Schultz, Marcus J.

2015-01-01

Protective mechanical ventilation with low tidal volumes is standard of care for patients with acute respiratory distress syndrome. The aim of this individual patient data analysis was to determine the association between tidal volume and the occurrence of pulmonary complications in ICU patients

The establishment of animal model of acute massive pulmonary embolism

International Nuclear Information System (INIS)

Lu Junliang; Yang Ning; Yang Jianping; Ma Junshan; Zhao Shijun

2008-01-01

Objective: To find a way of establishing the model of acute massive pulmonary embolism in dog. Methods: Seven dogs were selected with self-clots made outside the body transferring through a 10 F guiding catheter into the central branch of pulmonary artery via the femoral vein approach on one side and then under pressure monitor of pulmonary artery until the very branch of pulmonary artery was occluded. Blood gas and pulmonary arterial pressure were tested before and after the embolization, Pulmonary artery pressure was continuously monitored together with the examinations of angiography. The bilateral lung specimens were resected for histological examination 12 hours in average after the embolization for comparative study. Results: One animal died of cardiogenic shock after clots injection; the other one presented with tachycardia and premature ventricular beat causing partial recanalization 12 h later. The others were occluded successfully in central branch of pulmonary artery and the pulmonary arterial pressure reached above 50 mmHg after occlusion. Pathologic examination showed the formation of red and mix thrombi within the vascular lumens. Conclusions: This method for making acute massive pulmonary embolism animal model was reliable, feasible and reproducible, and could provide an animal model of acute massive pulmonary embolism for other correlative experiments. (authors)

Role of flexible bronchoscopy and bronchoalveolar lavage in the diagnosis of pediatric acquired immunodeficiency syndrome-related pulmonary disease.

Science.gov (United States)

Birriel, J A; Adams, J A; Saldana, M A; Mavunda, K; Goldfinger, S; Vernon, D; Holzman, B; McKey, R M

1991-06-01

Flexible fiberoptic bronchoscopy with bronchoalveolar lavage was performed in 16 pediatric patients with the acquired immunodeficiency syndrome (AIDS) and deterioration in pulmonary function suggestive of opportunistic infection. In 62% of the patients Pneumocystis carinii was identified. Culture results showed a pure growth of Pseudomonas aeruginosa for one patient in addition to the Pneumocystis carinii. Bronchoscopy with lavage was well tolerated, with few complications even among patients with significant tachypnea and hypoxia. Because of its relative safety and effectiveness, this procedure should be considered the first invasive measurement used for evaluation of parenchymal lung disease in this population of patients.

Patient with confirmed LEOPARD syndrome developing multiple melanoma

OpenAIRE

Colmant, Caroline; Franck, Deborah; Marot, Liliane; Matthijs, Gert; Sznajer, Yves; Blomme, Sandrine; Tromme, Isabelle

2018-01-01

LEOPARD syndrome, also known as Gorlin syndrome II, cardiocutaneous syndrome, lentiginosis profusa syndrome, Moynahan syndrome, was more recently coined as Noonan syndrome with multiple lentigines (NSML), inside the RASopathies. Historically, the acronym LEOPARD refers to the presence of distinctive clinical features such as: lentigines (L), electrocardiographic/conduction abnormalities (E), ocular hypertelorism (O), pulmonary stenosis (P), genital abnormalities (A), retardation of growth (R)...

Relationship between pulmonary artery volumes at computed tomography and pulmonary artery pressures in patients with- and without pulmonary hypertension

Energy Technology Data Exchange (ETDEWEB)

Froelich, Jens J. [Department of Radiology, Philipps-University Hospital, Baldingerstrasse, 35043 Marburg (Germany)], E-mail: jens.froelich@klinikum-hef.de; Koenig, Helmut [Department of Radiology, Philipps-University Hospital, Baldingerstrasse, 35043 Marburg (Germany)], E-mail: helmut.koenig@siemens.com; Knaak, Lennard [Department of Medicine, Philipps-University Hospital, Baldingerstrasse, 35043 Marburg (Germany)], E-mail: froehlic@staff.uni-marburg.de; Krass, Stefan [MeVis Research, Universitaetsallee 29, 28359 Bremen (Germany)], E-mail: krass@mevis.de; Klose, Klaus J. [Department of Radiology, Philipps-University Hospital, Baldingerstrasse, 35043 Marburg (Germany)], E-mail: klose@med.uni-marburg.de

2008-09-15

Objectives: This study was designed to determine the relationship between pulmonary artery (PA) volumes at computed tomography (CT) and PA pressures at right-sided heart catheterization in patients with and without pulmonary hypertension (PAH) to develop a noninvasive CT method of PA pressure quantification. Materials and methods: Sixteen patients with chronic sleep apnea syndrome underwent contrast enhanced helical CT (slice thickness 3 mm; pitch 2; increment 2 mm) at inspiration. Eight patients had PAH while cardiopulmonary disease has been excluded in eight other patients. Vascular volumes were determined using a 3D technique (threshold seeded vascular tracing algorithm; thresholds -600 H [lower] and 3000 H [upper]). Right-sided heart catheterization measurements were available for linear regression analysis of PA volumes and pressures. Results: Correlation between PA pressures and volumes (normalized for BMI), was high in both groups (without PAH: r = .85; with PAH .90, Pearson). Compared to elevated PA pressures in patients with pulmonary hypertension (p < .005), PA volumes also were significantly increased (p < .05) among the groups. Conclusions: High correlation was found between PA volumes and mean PA pressures in patients with- and without PAH. Significant differences in PA volumes at CT-volumetry may admit non-invasive determination of pulmonary hypertension.

Relationship between pulmonary artery volumes at computed tomography and pulmonary artery pressures in patients with- and without pulmonary hypertension

International Nuclear Information System (INIS)

Froelich, Jens J.; Koenig, Helmut; Knaak, Lennard; Krass, Stefan; Klose, Klaus J.

2008-01-01

Objectives: This study was designed to determine the relationship between pulmonary artery (PA) volumes at computed tomography (CT) and PA pressures at right-sided heart catheterization in patients with and without pulmonary hypertension (PAH) to develop a noninvasive CT method of PA pressure quantification. Materials and methods: Sixteen patients with chronic sleep apnea syndrome underwent contrast enhanced helical CT (slice thickness 3 mm; pitch 2; increment 2 mm) at inspiration. Eight patients had PAH while cardiopulmonary disease has been excluded in eight other patients. Vascular volumes were determined using a 3D technique (threshold seeded vascular tracing algorithm; thresholds -600 H [lower] and 3000 H [upper]). Right-sided heart catheterization measurements were available for linear regression analysis of PA volumes and pressures. Results: Correlation between PA pressures and volumes (normalized for BMI), was high in both groups (without PAH: r = .85; with PAH .90, Pearson). Compared to elevated PA pressures in patients with pulmonary hypertension (p < .005), PA volumes also were significantly increased (p < .05) among the groups. Conclusions: High correlation was found between PA volumes and mean PA pressures in patients with- and without PAH. Significant differences in PA volumes at CT-volumetry may admit non-invasive determination of pulmonary hypertension

Strength training versus chest physical therapy on pulmonary ...

African Journals Online (AJOL)

Zeinab Ahmed Hussein

2016-03-04

Mar 4, 2016 ... physical therapy treatment program on pulmonary functions in Down syndrome children. Methods: ... There is an increased prevalence of sleep-related upper airway ... eral health and daily activity performance ability [12].

A case of Swyer-James syndrome

International Nuclear Information System (INIS)

Suzuki, Toshimitsu; Ohtani, Naoshi; Kimura, Sohichi; Izuchi, Rokuro; Iio, Masaaki; Fujinami, Kenji.

1982-01-01

Infantile infections are thought to constitute one of the main bases of the etiology of Swyer-James syndrome. This case seems to support the above theory allowing for the anamnesis of the pleuritis at 2 years of age, bronchographical findings - bilateral but markedly left-sided bud-like bronchiectatic changes - and left pulmonary angiographical findings - simultaneous appearance of pulmonary arteries and veins with scarce capillary image. Concerning the ventilation of this syndrome, 133 Xe inhalation test showed a ''Pendelluft'' phenomenon, shift of 133 Xe from the healthy to the affected lung on forced expiration. (author)

Síndrome pulmón-riñón Pulmonary-renal syndrome

Directory of Open Access Journals (Sweden)

Jorge A. Risso

2009-12-01

Full Text Available El síndrome pulmón-riñón se define como una combinación de hemorragia alveolar difusa y glomerulonefritis. La coexistencia de estas dos afecciones clínicas se produce por enfermedades con distintos mecanismos patogénicos. Las vasculitis sistémicas primarias y el síndrome de Goodpasture son las etiologías más frecuentes. El lupus eritematoso sistémico, otras colagenopatías, las vasculitis con anticuerpos anticitoplasma de los neutrófilos negativos y las secundarias a drogas son causas mucho menos comunes. El diagnóstico temprano basado en criterios clínicos, radiológicos, de laboratorio e histológicos, permite iniciar el tratamiento disminuyendo su elevada morbi-mortalidad. La terapéutica se basa en altas dosis de corticoides, inmunosupresores, inhibidores del factor de necrosis tumoral y plasmaféresis.The pulmonary-renal syndrome is defined as a combination of diffuse alveolar hemorrhage and glomerulonephritis. The coexistence of these two clinical conditions is due to diseases with different pathogenic mechanisms. Primary systemic vasculitis and Goodpasture syndrome are the most frequent etiologies. Systemic lupus erythematosus, connective tissue diseases, negative anti neutrophil cytoplasmic antibody vasculitis and those secondary to drugs are far less common causes. An early diagnosis based on clinical, radiologic, laboratory and histologic criteria enables early treatment, thus diminishing its high morbility-mortality rate. Therapy is based on high doses of corticosteroids, immunosuppressants, tumor necrosis factor inhibitors and plasmapheresis.

Acute pulmonary admissions following implementation of a national workplace smoking ban.

LENUS (Irish Health Repository)

Kent, Brian D

2012-09-01

The implementation of workplace smoking bans has contributed to a significant reduction in the incidence of acute coronary syndrome admissions, but their influence on adult acute pulmonary disease admissions is unclear. We sought to assess the impact of a national smoking ban on nationwide admissions of individuals of working age with acute pulmonary illness.

Etiopathogenetic Mechanisms of Pulmonary Hypertension in Sleep-Related Breathing Disorders

Directory of Open Access Journals (Sweden)

Ayodeji Adegunsoye

2012-01-01

Full Text Available Obstructive sleep apnea syndrome is a common disorder with significant health consequences and is on the rise in consonance with the obesity pandemic. In view of the association between sleep-disordered breathing and pulmonary hypertension as depicted by multiple studies, current clinical practice guidelines categorize obstructive sleep apnea as a risk factor for pulmonary hypertension and recommend an assessment for sleep disordered breathing in evaluating patients with pulmonary hypertension. The dysregulatory mechanisms associated with hypoxemic episodes observed in sleep related breathing disorders contribute to the onset of pulmonary hypertension and identification of these potentially treatable factors might help in the reduction of overall cardiovascular mortality.

Cowden Syndrome and Concomitant Pulmonary Neuroendocrine Tumor

DEFF Research Database (Denmark)

Langer, Seppo W; Ringholm, Lene; Dali, Christine I

2015-01-01

Cowden Syndrome is a rare autosomal dominantly inherited disorder. Patients with Cowden Syndrome are at increased risk of various benign and malignant neoplasms in breast, endometrium, thyroid, gastrointestinal tract, and genitourinary system. Neuroendocrine tumors are ubiquitous neoplasms that may...

Changes of plasma ADM and ANP of pulmonary artery hypertension in patients with obstructive sleep apnea syndrome

International Nuclear Information System (INIS)

Gao Zhi; Zhong Jian; Sheng Chunyong; Xu Shouhong

2005-01-01

Objective: To explore the incidence of pulmonary artery hypertension (PAH) and changes of plasma adrenomedullin (ADM) and atrial natriuretic polypeptide (ANP) in patients with obstructive sleep apnea syndrome (OSAS). Methods: 32 healthy middle- and elder-aged cases were randomly selected as control group, 61 cases were confirmed clinically with OSAS. The pressure of pulmonary artery was measured with real time gray scale two-dimensional ultrasonic tomography, the blood plasma levels of ADM and ANP were determined with radioimmunoassay method. Results: Among 61 OSAS patients, 26 of them presented with PAH, its incidence in OSAS was 42.6%. The plasma level of ADM in OSAS with non-PAH group had statistical difference compared with control group ( P < 0.05 ) , also there were significant differences between PAH, non-PAH and control group (P<0.001). Concurrently, the plasma level of ANP in OSAS with PAH, non-PAH also had significant differences compared with control group (P<0.001). Conclusions: There was high incidence of PAH in OSAS patients. The plasma levels of ADM and ANP positively correlated with PAH. ANP played a role in the pathophysiologic genesis of PAH. (authors)

Pulmonary alveolar microlithiasis in children

Energy Technology Data Exchange (ETDEWEB)

Schmidt, H. [Center of Diagnostic Radiology, Frankfurt Univ. (Germany); Loercher, U. [Center of Diagnostic Radiology, Frankfurt Univ. (Germany); Kitz, R. [Center of Pediatrics, Frankfurt Univ. (Germany); Zielen, S. [Center of Pediatrics, Frankfurt Univ. (Germany); Ahrens, P. [Center of Pediatrics, Frankfurt Univ. (Germany); Koenig, R. [Inst. of Human Genetics, Frankfurt Univ. (Germany)

1996-01-01

Two asymptomatic Turkish sibs are presented, a 4-year-old boy and his 7-year-old sister, with pulmonary alveolar microlithiasis (PAM) confirmed by transbronchial lung biopsy and bronchoalveolar lavage. Chest radiographs and high resolution CT demonstrated wide-spread intra-alveolar calcifications in both lungs. The lesions were sharply defined and less than 1 mm in diameter. CT documented a high concentration of microliths along the bronchovascular bundles, the intralobular fissue and the (sub)pleural lung parenchyma. The combination of bronchoalveolar lavage and roentgenographic appearance in high resolution CT are characteristic and pathognomonic, and can confirm the diagnosis. The more severe changes in the elder sib and the radiographic controls suggest that the pulmonary disease may be progressive in our patients. The described family of consanguineous, unaffected parents with two affected and one healthy child confirmed the autosomal recessive inheritance of PAM (McKusick 265100). In addition, the affected girl had autosomal recessive Waardenburg-anophthalmia syndrome (McKusick 206920), raising the question of whether this is a chance occurrence or possibly a contiguous gene syndrome. (orig.)

Embolotherapy for pulmonary arteriovenous malformations in patients with hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber syndrome)

DEFF Research Database (Denmark)

Andersen, P E; Kjeldsen, A D; Oxhøj, H

1998-01-01

experienced an improved functional level. One patient experienced severe pleurisy and another a rise in temperature following treatment, but otherwise no symptomatic complications were observed. CONCLUSION: Embolotherapy is a definitive treatment for PAVMs: it is very effective with a high success rate......PURPOSE: To evaluate the clinical results of embolization of pulmonary arteriovenous malformations (PAVMs) in patients with hereditary hemorrhagic telangiectasia (HHT), the Rendu-Osler-Weber syndrome. MATERIAL AND METHODS: Twelve patients in the county of Fyn, Denmark, were treated...... with transcatheter embolization of 20 PAVMs using 12 detachable silicone balloons and 26 steel coils. RESULTS: All PAVMs were completely occluded and we observed a significant rise in PaO2 after treatment and a significant decrease in right-to-left shunt estimated by contrast echocardiography. All patients...

Hippocampal volume reduction in congenital central hypoventilation syndrome.

Directory of Open Access Journals (Sweden)

Paul M Macey

Full Text Available Children with congenital central hypoventilation syndrome (CCHS, a genetic disorder characterized by diminished drive to breathe during sleep and impaired CO(2 sensitivity, show brain structural and functional changes on magnetic resonance imaging (MRI scans, with impaired responses in specific hippocampal regions, suggesting localized injury.We assessed total volume and regional variation in hippocampal surface morphology to identify areas affected in the syndrome. We studied 18 CCHS (mean age+/-std: 15.1+/-2.2 years; 8 female and 32 healthy control (age 15.2+/-2.4 years; 14 female children, and traced hippocampi on 1 mm(3 resolution T1-weighted scans, collected with a 3.0 Tesla MRI scanner. Regional hippocampal volume variations, adjusted for cranial volume, were compared between groups based on t-tests of surface distances to the structure midline, with correction for multiple comparisons. Significant tissue losses emerged in CCHS patients on the left side, with a trend for loss on the right; however, most areas affected on the left also showed equivalent right-sided volume reductions. Reduced regional volumes appeared in the left rostral hippocampus, bilateral areas in mid and mid-to-caudal regions, and a dorsal-caudal region, adjacent to the fimbria.The volume losses may result from hypoxic exposure following hypoventilation during sleep-disordered breathing, or from developmental or vascular consequences of genetic mutations in the syndrome. The sites of change overlap regions of abnormal functional responses to respiratory and autonomic challenges. Affected hippocampal areas have roles associated with memory, mood, and indirectly, autonomic regulation; impairments in these behavioral and physiological functions appear in CCHS.

Pulmonary thromboembolism in children

Energy Technology Data Exchange (ETDEWEB)

Babyn, Paul S.; Gahunia, Harpal K. [Hospital for Sick Children, Department of Pediatric Diagnostic Imaging, Toronto, ON (Canada); Massicotte, Patricia [Stollery Children' s Hospital and University of Alberta, Departments of Pediatric Hematology and Cardiology, Edmonton, AB (Canada)

2005-03-01

Pulmonary thromboembolism (PTE) is uncommonly diagnosed in the pediatric patient, and indeed often only discovered on autopsy. The incidence of pediatric PTE depends upon the associated underlying disease, diagnostic tests used, and index of suspicion. Multiple risk factors can be found including: peripartum asphyxia, dyspnea, haemoptysis, chest pain, dehydration, septicemia, central venous lines (CVLs), trauma, surgery, ongoing hemolysis, vascular lesions, malignancy, renal disease, foreign bodies or, uncommonly, intracranial venous sinus thrombosis, burns, or nonbacterial thrombotic endocarditis. Other types of embolism can occur uncommonly in childhood and need to be recognized, as the required treatment will vary. These include pulmonary cytolytic thrombi, foreign bodies, tumor and septic emboli, and post-traumatic fat emboli. No single noninvasive test for pulmonary embolism is both sensitive and specific. A combination of diagnostic procedures must be used to identify suspect or confirmed cases of PTE. This article reviews the risk factors, clinical presentation and treatment of pulmonary embolism in children. It also highlights the current diagnostic tools and protocols used to evaluate pulmonary embolism in pediatric patients. (orig.)

Pulmonary thromboembolism in children

International Nuclear Information System (INIS)

Babyn, Paul S.; Gahunia, Harpal K.; Massicotte, Patricia

2005-01-01

Pulmonary thromboembolism (PTE) is uncommonly diagnosed in the pediatric patient, and indeed often only discovered on autopsy. The incidence of pediatric PTE depends upon the associated underlying disease, diagnostic tests used, and index of suspicion. Multiple risk factors can be found including: peripartum asphyxia, dyspnea, haemoptysis, chest pain, dehydration, septicemia, central venous lines (CVLs), trauma, surgery, ongoing hemolysis, vascular lesions, malignancy, renal disease, foreign bodies or, uncommonly, intracranial venous sinus thrombosis, burns, or nonbacterial thrombotic endocarditis. Other types of embolism can occur uncommonly in childhood and need to be recognized, as the required treatment will vary. These include pulmonary cytolytic thrombi, foreign bodies, tumor and septic emboli, and post-traumatic fat emboli. No single noninvasive test for pulmonary embolism is both sensitive and specific. A combination of diagnostic procedures must be used to identify suspect or confirmed cases of PTE. This article reviews the risk factors, clinical presentation and treatment of pulmonary embolism in children. It also highlights the current diagnostic tools and protocols used to evaluate pulmonary embolism in pediatric patients. (orig.)

Acute Respiratory Distress Syndrome Caused by Leukemic Infiltration of the Lung

Directory of Open Access Journals (Sweden)

Yao-Kuang Wu

2008-05-01

Full Text Available Respiratory distress syndrome resulting from leukemic pulmonary infiltrates is seldom diagnosed antemortem. Two 60- and 80-year-old women presented with general malaise, progressive shortness of breath, and hyperleukocytosis, which progressed to acute respiratory distress syndrome (ARDS after admission. Acute leukemia with pulmonary infection was initially diagnosed, but subsequent examinations including open lung biopsy revealed leukemic pulmonary infiltrates without infection. In one case, the clinical condition and chest radiography improved initially after combination therapy with chemotherapy for leukemia and aggressive pulmonary support. However, new pulmonary infiltration on chest radiography and hypoxemia recurred, which was consistent with acute lysis pneumopathy. Despite aggressive treatment, both patients died due to rapidly deteriorating condition. Leukemic pulmonary involvement should be considered in acute leukemia patients with non-infectious diffusive lung infiltration, especially in acute leukemia with a high blast count.

Pathology and pathophysiology of pulmonary manifestations in leptospirosis

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Marisa Dolhnikoff

Full Text Available Leptospirosis is a re-emerging zoonosis occurring as large outbreaks throughout the world caused by Leptospira interrogans. The incidence of pulmonary involvement in leptospirosis has been reported to be increasing in the last years, affecting up to 70% of the patients. Alveolar hemorrhage presented as dyspnea and hemoptysis is the main pulmonary manifestation. The emergence of massive hemoptysis and acute respiratory distress syndrome has characterized the recent changes reported in the clinical patterns of leptospirosis. The pulmonary involvement has been emerged as a serious life threat, becoming the main cause of death due to leptospirosis in some countries. In this review we present the main clinical and pathological manifestations of pulmonary involvement in leptospirosis, with special focus on recent data concerning the pathophysiological mechanisms underlying lung injury.

Mutations in the G6PC3 gene cause Dursun syndrome.

Science.gov (United States)

Banka, Siddharth; Newman, William G; Ozgül, R Koksal; Dursun, Ali

2010-10-01

Dursun syndrome is a triad of familial primary pulmonary hypertension, leucopenia, and atrial septal defect. Here we demonstrate that mutations in G6PC3 cause Dursun syndrome. Mutations in G6PC3 are known to also cause severe congenital neutropenia type 4. Identification of the genetic basis of Dursun syndrome expands the pre-existing knowledge about the phenotypic effects of mutations in G6PC3. We propose that Dursun syndrome should now be considered as a subset of severe congenital neutropenia type 4 with pulmonary hypertension as an important clinical feature. Copyright © 2010 Wiley-Liss, Inc.

Central poststroke pain: somatosensory abnormalities and the presence of associated myofascial pain syndrome

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de Oliveira Rogério Adas

2012-09-01

Full Text Available Abstract Background Central post-stroke pain (CPSP is a neuropathic pain syndrome associated with somatosensory abnormalities due to central nervous system lesion following a cerebrovascular insult. Post-stroke pain (PSP refers to a broader range of clinical conditions leading to pain after stroke, but not restricted to CPSP, including other types of pain such as myofascial pain syndrome (MPS, painful shoulder, lumbar and dorsal pain, complex regional pain syndrome, and spasticity-related pain. Despite its recognition as part of the general PSP diagnostic possibilities, the prevalence of MPS has never been characterized in patients with CPSP patients. We performed a cross-sectional standardized clinical and radiological evaluation of patients with definite CPSP in order to assess the presence of other non-neuropathic pain syndromes, and in particular, the role of myofascial pain syndrome in these patients. Methods CPSP patients underwent a standardized sensory and motor neurological evaluation, and were classified according to stroke mechanism, neurological deficits, presence and profile of MPS. The Visual Analogic Scale (VAS, McGill Pain Questionnaire (MPQ, and Beck Depression Scale (BDS were filled out by all participants. Results Forty CPSP patients were included. Thirty-six (90.0% had one single ischemic stroke. Pain presented during the first three months after stroke in 75.0%. Median pain intensity was 10 (5 to 10. There was no difference in pain intensity among the different lesion site groups. Neuropathic pain was continuous-ongoing in 34 (85.0% patients and intermittent in the remainder. Burning was the most common descriptor (70%. Main aggravating factors were contact to cold (62.5%. Thermo-sensory abnormalities were universal. MPS was diagnosed in 27 (67.5% patients and was more common in the supratentorial extra-thalamic group (P Conclusions The presence of MPS is not an exception after stroke and may present in association with CPSP

[Closure of wide patent ductus arteriosus using a fenestrated muscular VSD occluder device in a pediatric patient with Down syndrome and pulmonary hypertension].

Science.gov (United States)

Güvenç, Osman; Saygı, Murat; Demir, İbrahim Halil; Ödemiş, Ender

2017-06-01

Patients with wide patent ductus arteriosus and significant pulmonary hypertension not treated in time constitute a significant problem for cardiologists. For these patients, tests that could aid in decision-making for further planning include reversibility and balloon occlusion tests performed in the catheterization laboratory. Devices developed for the closure of ductus as well as different devices with off-label use may be employed in patients scheduled for transcatheter occlusion. When result of reversibility test is borderline positive, the use of fenestrated device may be applicable for selected patients. Presently described is case of a 10-year-old patient with Down syndrome who had a wide ductus and systemic pulmonary hypertension. Transcatheter closure procedure was performed with off-label use of a fenestrated muscular ventricular septal defect occluder device.

Central role of T helper 17 cells in chronic hypoxia-induced pulmonary hypertension.

Science.gov (United States)

Maston, Levi D; Jones, David T; Giermakowska, Wieslawa; Howard, Tamara A; Cannon, Judy L; Wang, Wei; Wei, Yongyi; Xuan, Weimin; Resta, Thomas C; Gonzalez Bosc, Laura V

2017-05-01

Inflammation is a prominent pathological feature in pulmonary arterial hypertension, as demonstrated by pulmonary vascular infiltration of inflammatory cells, including T and B lymphocytes. However, the contribution of the adaptive immune system is not well characterized in pulmonary hypertension caused by chronic hypoxia. CD4 + T cells are required for initiating and maintaining inflammation, suggesting that these cells could play an important role in the pathogenesis of hypoxic pulmonary hypertension. Our objective was to test the hypothesis that CD4 + T cells, specifically the T helper 17 subset, contribute to chronic hypoxia-induced pulmonary hypertension. We compared indices of pulmonary hypertension resulting from chronic hypoxia (3 wk) in wild-type mice and recombination-activating gene 1 knockout mice (RAG1 -/- , lacking mature T and B cells). Separate sets of mice were adoptively transferred with CD4 + , CD8 + , or T helper 17 cells before normoxic or chronic hypoxic exposure to evaluate the involvement of specific T cell subsets. RAG1 -/- mice had diminished right ventricular systolic pressure and arterial remodeling compared with wild-type mice exposed to chronic hypoxia. Adoptive transfer of CD4 + but not CD8 + T cells restored the hypertensive phenotype in RAG1 -/- mice. Interestingly, RAG1 -/- mice receiving T helper 17 cells displayed evidence of pulmonary hypertension independent of chronic hypoxia. Supporting our hypothesis, depletion of CD4 + cells or treatment with SR1001, an inhibitor of T helper 17 cell development, prevented increased pressure and remodeling responses to chronic hypoxia. We conclude that T helper 17 cells play a key role in the development of chronic hypoxia-induced pulmonary hypertension. Copyright © 2017 the American Physiological Society.

Exercise-Induced Pulmonary Edema in a Triathlon

Directory of Open Access Journals (Sweden)

Hirotomo Yamanashi

2015-01-01

Full Text Available Introduction. Family physicians have more opportunities to attend athletic competitions as medical staff at first-aid centers because of the increasing popularity of endurance sports. Case. A 38-year-old man who participated in a triathlon race experienced difficulty in breathing after swimming and was moved to a first-aid center. His initial oxygen saturation was 82% and a thoracic computed tomography scan showed bilateral ground glass opacity in the peripheral lungs. His diagnosis was noncardiogenic pulmonary edema associated with exercise or swimming: exercise-induced pulmonary edema (EIPE or swimming-induced pulmonary edema (SIPE. Treatment with furosemide and corticosteroid relieved his symptoms of pulmonary edema. Discussion. Noncardiogenic pulmonary edema associated with endurance sports is not common, but knowledge about EIPE/SIPE or neurogenic pulmonary edema associated with hyponatremia, which is called Ayus-Arieff syndrome, is crucial. Knowledge and caution for possible risk factors, such as exposure to cold water or overhydration, are essential for both medical staff and endurance athletes. Conclusion. To determine the presence of pulmonary edema associated with strenuous exercise, oxygen saturation should be used as a screening tool at a first-aid center. To avoid risks for EIPE/SIPE, knowledge about these diseases is essential for medical staff and for athletes who perform extreme exercise.

Anesthetic management of right atrial mass removal and pulmonary artery thrombectomy in a patient with primary antiphospholipid antibody syndrome

Directory of Open Access Journals (Sweden)

Rawat SKS

2010-01-01

Full Text Available Antiphospholipid antibody syndrome (APLAS characterises a clinical condition of arterial and venous thrombosis associated with phospholipids directed antibodies. APLAS occurs in 2% of the general population. However, one study demonstrated that 7.1% of hospitalised patients were tested positive for at least one of the three anticardiolipin antibody idiotype. Antiphospholipid antibodies often inhibit phospholipids dependent coagulation in vitro and interfere with laboratory testing of hemostasis. Therefore, the management of anticoagulation during cardiopulmonary bypass can be quite challenging in these patients. Here, we present a case of right atrial mass removal and pulmonary thrombectomy in a patient of APLAS.

Metabolic syndrome in hospitalized patients with chronic obstructive pulmonary disease

Directory of Open Access Journals (Sweden)

Evgeni Mekov

2015-07-01

Full Text Available Introduction. The metabolic syndrome (MS affects 21–53% of patients with chronic obstructive pulmonary disease (COPD with a higher prevalence in the early stages of COPD, with results being highly variable between studies. MS may also affect natural course of COPD—number of exacerbations, quality of life and lung function.Aim. To examine the prevalence of MS and its correlation with comorbidities and COPD characteristics in patients with COPD admitted for exacerbation.Material and methods. 152 patients with COPD admitted for exacerbation were studied for presence of MS. All of them were also assessed for vitamin D status and diabetes mellitus type 2 (DM. Data were gathered for smoking status and exacerbations during the last year. All patients completed CAT (COPD assessment test and mMRC (Modified Medical Research Council Dyspnea scale questionnaires and underwent spirometry. Duration of current hospital stay was recorded.Results. 25% of patients have MS. 23.1% of the male and 29.5% of the female patients have MS (p > 0.05. The prevalence of MS in this study is significantly lower when compared to a national representative study (44.6% in subjects over 45 years. 69.1% of all patients and 97.4% from MS patients have arterial hypertension. The presence of MS is associated with significantly worse cough and sleep (1st and 7th CAT questions; p = 0.002 and p = 0.001 respectively and higher total CAT score (p = 0.017. Average BMI is 27.31. None of the patients have MS and BMI <25. There is a correlation between the presence of MS and DM (p = 0.008 and with the number of exacerbations in the last year (p = 0.015. There is no correlation between the presence of MS and the pulmonary function.Conclusion. This study among hospitalized COPD patients finds comparable but relatively low prevalence of MS (25% compared to previously published data (21–53% and lower prevalence compared to general population (44.6%. MS may impact quality of life and the

A study of images of Projective Angles of pulmonary veins

Energy Technology Data Exchange (ETDEWEB)

Wang Jue [Beijing Anzhen Hospital, Beijing (China); Zhaoqi, Zhang [Beijing Anzhen Hospital, Beijing (China)], E-mail: zhaoqi5000@vip.sohu.com; Yu Wei; Miao Cuilian; Yan Zixu; Zhao Yike [Beijing Anzhen Hospital, Beijing (China)

2009-09-15

Aims: In images of magnetic resonance and computed tomography (CT) there are visible angles between pulmonary veins and the coronary, transversal or sagittal section of body. In this study these angles are measured and defined as Projective Angles of pulmonary veins. Several possible influential factors and characters of distribution are studied and analyzed for a better understanding of this imaging anatomic character of pulmonary veins. And it could be the anatomic base of adjusting correctly the angle of the central X-ray of the angiography of pulmonary veins undergoing the catheter ablation of atrial fibrillation (AF). Method: Images of contrast enhanced magnetic resonance angiography (CEMRA) and contrast enhanced computer tomography (CECT) of the left atrium and pulmonary veins of 137 health objects and patients with atrial fibrillation (AF) are processed with the technique of post-processing, and Projective Angles to the coronary and transversal sections are measured and analyzed statistically. Result: Project Angles of pulmonary veins are one of real and steady imaging anatomic characteristics of pulmonary veins. The statistical distribution of variables is relatively concentrated, with a fairly good representation of average value. It is possible to improve the angle of the central X-ray according to the average value in the selective angiography of pulmonary veins undergoing the catheter ablation of AF.

Chronic thromboembolic pulmonary hypertension: diagnostic impact of multislice-CT and selective pulmonary-DSA

International Nuclear Information System (INIS)

Pitton, M.B.; Kemmerich, G.; Herber, S.; Schweden, F.; Thelen, M.; Mayer, E.

2002-01-01

Purpose: To evaluate the diagnostic impact of multislice-CT and selective pulmonary DSA in chronic thromboembolic pulmonary hypertension (CTEPH). Methods: 994 vessel segments of 14 consecutive patients with CTEPH were investigated with multislice-CT (slice thickness 3 mm, collimation 2.5 mm, reconstruction intervall 2 mm) and selective pulmonary DSA posterior-anterior, 45 oblique, and lateral projection. Analysis was performed by 2 investigators independently for CT and DSA. Diagnostic criteria were occlusions and non-occlusive changes like webs and bands, irregularities of the vessel wall, diameter reduction and thromboembolic depositions at different levels from central pulmonary arteries to subsegmental arteries. Reference diagnosis was made by synopsis of CT and DSA by consensus. Results: Concerning patency CT and DSA showed concordant findings overall in 88.9%, 92.9% for segmental arteries and 85.4% for subsegmental arteries. Concerning any thromboembolic changes, multislice-CT was significantly inferior to selective DSA (concordance 67.0% overall, 70.4% for segments and 63.6% for subsegments). Non-occlusive changes of the vessels were significantly underdiagnosed by CT (concordance of CT versus DSA: 23.1%). Conclusion: Multislice-CT and selective pulmonary DSA are equivalent for diagnosis of vessel occlusions at the level of segmental and subsegmental arteries. However, for visualisation of the non-occlusive thromboembolic changes of the vessel wall selective pulmonary DSA is still superior compared to multislice-CT. Multislice-CT and selective pulmonary DSA are complementary tools for diagnosis and treatment planning of chronic thromboembolic pulmonary hypertension (CTEPH). (orig.) [de

Therapy for sleep hypoventilation and central apnea syndromes.

Science.gov (United States)

Selim, Bernardo J; Junna, Mithri R; Morgenthaler, Timothy I

2012-10-01

• Primary Central Sleep Apnea (CSA): We would recommend a trial of Positive Airway Pressure (PAP), acetazolamide, or zolpidem based on thorough consideration of risks and benefits and incorporation of patient preferences.• Central Sleep Apnea Due to Cheyne-Stokes Breathing Pattern in Congestive Heart Failure (CSR-CHF): We would recommend PAP devices such as continuous positive airway pressure (CPAP) or adaptive servo-ventilation (ASV) to normalize sleep-disordered breathing after optimizing treatment of heart failure. Oxygen may also be an effective therapy. Acetazolamide and theophylline may be considered if PAP or oxygen is not effective.• Central Sleep Apnea due to High-Altitude Periodic Breathing: We would recommend descent from altitude or supplemental oxygen. Acetazolamide may be used when descent or oxygen are not feasible, or in preparation for ascent to high altitude. Slow ascent may be preventative.• Central Sleep Apnea due to Drug or Substance: If discontinuation or reduction of opiate dose is not feasible or effective, we would recommend a trial of CPAP, and if not successful, treatment with ASV. If ASV is ineffective or if nocturnal hypercapnia develops, bilevel positive airway pressure-spontaneous timed mode (BPAP-ST) is recommended.• Obesity hypoventilation syndrome: We would recommend an initial CPAP trial. If hypoxia or hypercapnia persists on CPAP, BPAP, BPAP-ST or average volume assured pressure support (AVAPS™) is recommended. Tracheostomy with nocturnal ventilation should be considered when the above measures are not effective. Weight loss may be curative.• Neuromuscular or chest wall disease: We would recommend early implementation of BPAP-ST based on thorough consideration of risks and benefits and patient preferences. AVAPS™ may also be considered. We recommend close follow up due to disease progression.

Radionuclide assessment of pulmonary microvascular permeability

Energy Technology Data Exchange (ETDEWEB)

Groeneveld, A.B.J. [Medical Intensive Care Unit, Department of Internal Medicine, Free University Hospital, De Boelelaan 1117, 1081 HV Amsterdam (Netherlands)

1997-04-01

The literature has been reviewed to evaluate the technique and clinical value of radionuclide measurements of microvascular permeability and oedema formation in the lungs. Methodology, modelling and interpretation vary widely among studies. Nevertheless, most studies agree on the fact that the measurement of permeability via pulmonary radioactivity measurements of intravenously injected radiolabelled proteins versus that in the blood pool, the so-called pulmonary protein transport rate (PTR), can assist the clinician in discriminating between permeability oedema of the lungs associated with the adult respiratory distress syndrome (ARDS) and oedema caused by an increased filtration pressure, for instance in the course of cardiac disease, i.e. pressure-induced pulmonary oedema. Some of the techniques used to measure PTR are also able to detect subclinical forms of lung microvascular injury not yet complicated by permeability oedema. This may occur after cardiopulmonary bypass and major vascular surgery, for instance. By paralleling the clinical severity and course of the ARDS, the PTR method may also serve as a tool to evaluate new therapies for the syndrome. Taken together, the currently available radionuclide methods, which are applicable at the bedside in the intensive care unit, may provide a gold standard for detecting minor and major forms of acute microvascular lung injury, and for evaluating the severity, course and response to treatment. (orig.). With 2 tabs.

Hantavirus pulmonary syndrome clinical findings: evaluating a surveillance case definition.

Science.gov (United States)

Knust, Barbara; Macneil, Adam; Rollin, Pierre E

2012-05-01

Clinical cases of hantavirus pulmonary syndrome (HPS) can be challenging to differentiate from other acute respiratory diseases, which can lead to delays in diagnosis, treatment, and disease reporting. Rapid onset of severe disease occurs, at times before diagnostic test results are available. This study's objective was to examine the clinical characteristics of patients that would indicate HPS to aid in detection and reporting. Test results of blood samples from U.S. patients suspected of having HPS submitted to the Centers for Disease Control and Prevention from 1998-2010 were reviewed. Patient information collected by case report forms was compared between HPS-confirmed and test-negative patients. Diagnostic sensitivity, specificity, predictive values, and likelihood ratios were calculated for individual clinical findings and combinations of variables. Of 567 patients included, 36% were HPS-confirmed. Thrombocytopenia, chest x-rays with suggestive signs, and receiving supplemental oxygenation were highly sensitive (>95%), while elevated hematocrit was highly specific (83%) in detecting HPS. Combinations that maximized sensitivity required the presence of thrombocytopenia. Using a national sample of suspect patients, we found that thrombocytopenia was a highly sensitive indicator of HPS and should be included in surveillance definitions for suspected HPS. Using a sensitive suspect case definition to identify potential HPS patients that are confirmed by highly specific diagnostic testing will ensure accurate reporting of this disease.

Application of transpulmonary thermodilution monitoring (PiCCO) in patient with neurogenic pulmonary edema and acute obstructive hydrocephalus due to a central neurocytoma: A case report

OpenAIRE

Ryoichi Iwata; Kunikazu Yoshimura; Yoko Fujita; Tatsuo Uesaka; Hideyuki Oshige; Akio Asai

2017-01-01

Neurogenic pulmonary edema (NPE) is an acute life-threatening complication associated with many forms of central nervous system injury. Its pathophysiology is still debated. We report a patient with acute obstructive hydrocephalus due to a central neurocytoma who also had NPE, for which serial transpulmonary thermodilution monitoring (PiCCO) was performed. Insertion of the PiCCO, which provides information about the patient's cardiac output, preload status and amount of lung water, revealed a...

Meier-Gorlin syndrome

NARCIS (Netherlands)

Munnik, S.A. de; Hoefsloot, E.H.; Roukema, J.; Schoots, J.; Knoers, N.V.A.M.; Brunner, H.G.; Jackson, A.P.; Bongers, E.M.H.F.

2015-01-01

Meier-Gorlin syndrome (MGS) is a rare autosomal recessive primordial dwarfism disorder, characterized by microtia, patellar applasia/hypoplasia, and a proportionate short stature. Associated clinical features encompass feeding problems, congenital pulmonary emphysema, mammary hypoplasia in females

Wolf-Hirschhorn Syndrome

African Journals Online (AJOL)

Extract Studio

she had the distinctive facial features of Wolf-Hirschhorn syndrome that included hyperteleorism, ... The PDA noted at birth had closed by 3 months and there was no other cardiac .... followed by pulmonary stenosis, ventricular septal defects,.

Use of mathematic modeling to compare and predict hemodynamic effects of the modified Blalock-Taussig and right ventricle-pulmonary artery shunts for hypoplastic left heart syndrome.

Science.gov (United States)

Bove, Edward L; Migliavacca, Francesco; de Leval, Marc R; Balossino, Rossella; Pennati, Giancarlo; Lloyd, Thomas R; Khambadkone, Sachin; Hsia, Tain-Yen; Dubini, Gabriele

2008-08-01

Stage one reconstruction (Norwood operation) for hypoplastic left heart syndrome can be performed with either a modified Blalock-Taussig shunt or a right ventricle-pulmonary artery shunt. Both methods have certain inherent characteristics. It is postulated that mathematic modeling could help elucidate these differences. Three-dimensional computer models of the Blalock-Taussig shunt and right ventricle-pulmonary artery shunt modifications of the Norwood operation were developed by using the finite volume method. Conduits of 3, 3.5, and 4 mm were used in the Blalock-Taussig shunt model, whereas conduits of 4, 5, and 6 mm were used in the right ventricle-pulmonary artery shunt model. The hydraulic nets (lumped resistances, compliances, inertances, and elastances) were identical in the 2 models. A multiscale approach was adopted to couple the 3-dimensional models with the circulation net. Computer simulations were compared with postoperative catheterization data. Good correlation was found between predicted and observed data. For the right ventricle-pulmonary artery shunt modification, there was higher aortic diastolic pressure, decreased pulmonary artery pressure, lower Qp/Qs ratio, and higher coronary perfusion pressure. Mathematic modeling predicted minimal regurgitant flow in the right ventricle-pulmonary artery shunt model, which correlated with postoperative Doppler measurements. The right ventricle-pulmonary artery shunt demonstrated lower stroke work and a higher mechanical efficiency (stroke work/total mechanical energy). The close correlation between predicted and observed data supports the use of mathematic modeling in the design and assessment of surgical procedures. The potentially damaging effects of a systemic ventriculotomy in the right ventricle-pulmonary artery shunt modification of the Norwood operation have not been analyzed.

Computerized axial tomography of the chest for visualization of ''absent'' pulmonary arteries

Energy Technology Data Exchange (ETDEWEB)

Sondheimer, H.M. (Upstate Medical Center, Syracuse, NY); Oliphant, M.; Schneider, B.; Kavey, R.E.W.; Blackman, M.S.; Parker, F.B. Jr.

1982-05-01

To expand the search for central pulmonary arteries in six patients with absence of cardiac-pulmonary continuity, computerized axial tomography (CAT) of the chest was performed. The CAT scans were compared with previous arteriograms and pulmonary vein wedge angiograms. Three patients with type IV truncus arteriosus were studied, and none had a central, right or left pulmonary artery on CAT scan. However, two patients with tetralogy of Fallot with pulmonary atresia and a patent ductus arteriosus to the right lung demonstrated the presence of a left pulmonary artery. In addition, one child with truncus arteriosus with ''absent'' left pulmonary artery demonstrated a left pulmonary artery on the CAT scan. The CAT scan may therefore enhance our ability to search for disconnected pulmonary arteries in children with complex cyanotic congenital heart disease.

Catastrophic primary antiphospholipid syndrome

International Nuclear Information System (INIS)

Kim, Dong Hun; Byun, Joo Nam; Ryu, Sang Wan

2006-01-01

Catastrophic antiphospholipid syndrome (CAPLS) was diagnosed in a 64-year-old male who was admitted to our hospital with dyspnea. The clinical and radiological examinations showed pulmonary thromboembolism, and so thromboembolectomy was performed. Abdominal distension rapidly developed several days later, and the abdominal computed tomography (CT) abdominal scan revealed thrombus within the superior mesenteric artery with small bowel and gall bladder distension. Cholecystectomy and jejunoileostomy were performed, and gall bladder necrosis and small bowel infarction were confirmed. The anticardiolipin antibody was positive. Anticoagulant agents and steroids were administered, but the patient expired 4 weeks after surgery due to acute respiratory distress syndrome (ARDS). We report here on a case of catastrophic APLS with manifestations of pulmonary thromboembolism, rapidly progressing GB necrosis and bowel infarction

Catastrophic primary antiphospholipid syndrome

Energy Technology Data Exchange (ETDEWEB)

Kim, Dong Hun; Byun, Joo Nam [Chosun University Hospital, Gwangju (Korea, Republic of); Ryu, Sang Wan [Miraero21 Medical Center, Gwangju (Korea, Republic of)

2006-09-15

Catastrophic antiphospholipid syndrome (CAPLS) was diagnosed in a 64-year-old male who was admitted to our hospital with dyspnea. The clinical and radiological examinations showed pulmonary thromboembolism, and so thromboembolectomy was performed. Abdominal distension rapidly developed several days later, and the abdominal computed tomography (CT) abdominal scan revealed thrombus within the superior mesenteric artery with small bowel and gall bladder distension. Cholecystectomy and jejunoileostomy were performed, and gall bladder necrosis and small bowel infarction were confirmed. The anticardiolipin antibody was positive. Anticoagulant agents and steroids were administered, but the patient expired 4 weeks after surgery due to acute respiratory distress syndrome (ARDS). We report here on a case of catastrophic APLS with manifestations of pulmonary thromboembolism, rapidly progressing GB necrosis and bowel infarction.

Noonan Syndrome Complicated by Primary Pulmonary Lymphangiectasia.

Science.gov (United States)

Ford, Jaclyn Jo; Trotter, Carol W

2015-01-01

Noonan syndrome is a genetic disorder that has several features common to other conditions, making diagnosis a challenge. This column summarizes the case of a neonate with an atypical presentation of Noonan syndrome involving a fatal type of lymphangiectasia resulting in persistent pleural effusions. Radiographic features of this condition are presented along with the complexities of diagnosis and treatment.

Leptospirose pulmonar Pulmonary leptospirosis

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João Cláudio Barroso Pereira

2007-12-01

Full Text Available No presente artigo, os autores discutem brevemente sobre a leptospirose, realçando a forma pulmonar da doença. Revê-se a patologia, achados clínicos, diagnóstico por métodos de imagem e broncoscopia e tratamento da leptospirose pulmonar. É também lembrado o diagnóstico clínico e radiológico precoces, para que se possa iniciar terapêutica adequada. Os autores concluem que a forma pulmonar da leptospirose deve ser sempre considerada como causa e diagnóstico diferencial da hemorragia alveolar difusa e síndroma de dificuldade respiratória do adulto.In this article, the authors discuss briefly the leptospirosis, emphasizing mainly the pulmonary form of disease. The authors review pathology, clinical findings, imaging and broncoscopy diagnosis, treatment of pulmonary leptospirosis. It is also remembered about early clinics and radiology diagnosis to start therapeutics. The authors conclude that pulmonary form of disease must always be remembered and considered as cause and differential diagnosis of Diffuse Alveolar Hemorrhage and Adult Respiratory Distress Syndrome.

Pulmonary edema: radiographic differential diagnosis

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Yoo, Dong Soo; Choi, Young Hi; Kim, Seung Cheol; An, Ji Hyun; Lee, Jee Young; Park, Hee Hong

1997-01-01

To evaluate the feasibility of using chest radiography to differentiate between three different etiologies of pulmonary edema. Plain chest radiographs of 77 patients, who were clinically confirmed as having pulmonary edema, were retrospectively reviewed. The patients were classified into three groups : group 1 (cardiogenic edema : n = 35), group 2 (renal pulmonary edema : n = 16) and group 3 (permeability edema : n = 26). We analyzed the radiologic findings of air bronchogram, heart size, peribronchial cuffing, septal line, pleural effusion, vascular pedicle width, pulmonary blood flow distribution and distribution of pulmonary edema. In a search for radiologic findings which would help in the differentiation of these three etiologies, each finding was assessed. Cardiogenic and renal pulmonary edema showed overlapping radiologic findings, except for pulmonary blood flow distribution. In cardiogenic pulmonary edema (n=35), cardiomegaly (n=29), peribronchial cuffing (n=29), inverted pulmonary blood flow distribution (n=21) and basal distribution of edema (n=20) were common. In renal pulmonary edema (n=16), cardiomegaly (n=15), balanced blood flow distribution (n=12), and central (n=9) or basal distribution of edema (n=7) were common. Permeability edema (n=26) showed different findings. Air bronchogram (n=25), normal blood flow distribution (n=14) and peripheral distribution of edema (n=21) were frequent findings, while cardiomegaly (n=7), peribronchial cuffing (n=7) and septal line (n=5) were observed in only a few cases. On plain chest radiograph, permeability edema can be differentiated from cardiogenic or renal pulmonary edema. The radiographic findings which most reliably differentiated these two etiologies were air bronchogram, distribution of pulmonary edema, peribronchial cuffing and heart size. Only blood flow distribution was useful for radiographic differentiation of cardiogenic and renal edema

Radiologists need to be aware of secondary central venous stenosis in patients with SAPHO syndrome

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Suzuki, Mizuho; Kanazawa, Hidenori; Shinozaki, Takeshi; Sugimoto, Hideharu [Jichi Medical University, Department of Radiology, Shimotsuke, Tochigi (Japan)

2017-11-15

We aimed to define central venous stenosis (CVS) caused by sternocostoclavicular hyperostosis as a feature of synovitis-acne-pustulosis-hyperostosis-osteitis (SAPHO) syndrome on routine contrast-enhanced computed tomography (CT) images. The relationship between SAPHO syndrome and CVS without venous thrombosis caused by anterior chest wall compression has not been investigated. Therefore, the present study evaluated CVS in patients with SAPHO syndrome at our hospital. We retrospectively reviewed contrast-enhanced CT images of ten patients with suspected or diagnosed SAPHO syndrome between January 2007 and November 2015. The patients were assessed by contrast-enhanced CT using 16-, 64- or 128-detector row scanners. Two radiologists independently assessed the presence of CVS or obstruction and SAPHO syndrome in a retrospective review of CT images. Six of the ten patients had findings of CVS with SAPHO syndrome. The mean diameter and patency rate at the site of CVS were 1.88 mm and 27.2%, respectively. Stenosis was more significant in terms of the mean diameter of CVS sites than of stenotic sites that crossed the anteroposterior vein (p < 0.05). Radiologists who routinely assess contrast-enhanced CT images should be aware that sternocostoclavicular hyperostosis with SAPHO syndrome could cause secondary CVS. (orig.)

Dysplastic pulmonary valve stenosis associated with unilateral absent first metacarpal: A rare association

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Ashwin Kodliwadmath

2017-08-01

Full Text Available Context: Dysplastic pulmonary valve stenosis is a less common variety of valvular pulmonary stenosis. It is known to be part of Noonan syndrome. Bony hand anomalies in patients of pulmonary stenosis are very rare. Case report: A 50-year-old lady, with no significant past history, presented with slowly progressive breathlessness and fatigue, and had progressed from NYHA class 1 to 2 over 2 years. She had unilateral absent first metacarpal and diagnosed on workup to have dysplastic pulmonary valve stenosis and was treated with balloon valvuloplasty. Conclusion: Dysplastic pulmonary valve stenosis can rarely be associated with bony hand anomalies like absent first metacarpal.

Acute respiratory distress syndrome: epidemiology and management approaches

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Walkey AJ

2012-07-01

Full Text Available Allan J Walkey,1 Ross Summer,1 Vu Ho,1 Philip Alkana21The Pulmonary Center, Boston University School of Medicine, Boston, MA, USA; 2Asthma Research Center, Brigham and Women's Hospital, Boston, MA, USAAbstract: Acute lung injury and the more severe acute respiratory distress syndrome represent a spectrum of lung disease characterized by the sudden onset of inflammatory pulmonary edema secondary to myriad local or systemic insults. The present article provides a review of current evidence in the epidemiology and treatment of acute lung injury and acute respiratory distress syndrome, with a focus on significant knowledge gaps that may be addressed through epidemiologic methods.Keywords: acute lung injury, acute respiratory distress syndrome, review, epidemiology

Central sensitization as the mechanism underlying pain in joint hypermobility syndrome/Ehlers-Danlos syndrome, hypermobility type.

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Di Stefano, G; Celletti, C; Baron, R; Castori, M; Di Franco, M; La Cesa, S; Leone, C; Pepe, A; Cruccu, G; Truini, A; Camerota, F

2016-09-01

Patients with joint hypermobility syndrome/Ehlers-Danlos syndrome, hypermobility type (JHS/EDS-HT) commonly suffer from pain. How this hereditary connective tissue disorder causes pain remains unclear although previous studies suggested it shares similar mechanisms with neuropathic pain and fibromyalgia. In this prospective study seeking information on the mechanisms underlying pain in patients with JHS/EDS-HT, we enrolled 27 consecutive patients with this connective tissue disorder. Patients underwent a detailed clinical examination, including the neuropathic pain questionnaire DN4 and the fibromyalgia rapid screening tool. As quantitative sensory testing methods, we included thermal-pain perceptive thresholds and the wind-up ratio and recorded a standard nerve conduction study to assess non-nociceptive fibres and laser-evoked potentials, assessing nociceptive fibres. Clinical examination and diagnostic tests disclosed no somatosensory nervous system damage. Conversely, most patients suffered from widespread pain, the fibromyalgia rapid screening tool elicited positive findings, and quantitative sensory testing showed lowered cold and heat pain thresholds and an increased wind-up ratio. While the lack of somatosensory nervous system damage is incompatible with neuropathic pain as the mechanism underlying pain in JHS/EDS-HT, the lowered cold and heat pain thresholds and increased wind-up ratio imply that pain in JHS/EDS-HT might arise through central sensitization. Hence, this connective tissue disorder and fibromyalgia share similar pain mechanisms. WHAT DOES THIS STUDY ADD?: In patients with JHS/EDS-HT, the persistent nociceptive input due to joint abnormalities probably triggers central sensitization in the dorsal horn neurons and causes widespread pain. © 2016 European Pain Federation - EFIC®

Dress syndrome with sepsis, acute respiratory distress syndrome and pneumomediastinum

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Prabhas Prasun Giri

2011-01-01

Full Text Available Drug rash with eosinophilia and systemic symptoms (DRESS syndrome reflects a serious hypersensitivity reaction to drugs, and is characterized by skin rash, fever, lymph node enlargement, and internal organ involvement. So far, numerous drugs such as sulfonamides, phenobarbital, sulfasalazine, carbamazepine, and phenytoin have been reported to cause DRESS syndrome. We report a case of a 10-year-old girl who developed clinical manifestations of fever, rash, lymphadenopathy, hypereosinophilia, and visceral involvement (hepatitis and pneumonitis after taking phenobarbital for seizures, with subsequent development of sepsis, acute respiratory distress syndrome (ARDS and spontaneous air leak syndrome (pnemothorax and pneumomediastinum. She was put on steroids and various antibiotics and was ventilated, but ultimately succumbed to sepsis and pulmonary complications.

Obstructive sleep apnea in chronic obstructive pulmonary disease patients.

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Lee, Ruth

2011-03-01

Chronic obstructive pulmonary disease (COPD) and obstructive sleep apnea (OSA) represent two of the most prevalent chronic respiratory disorders and cardiovascular diseases are major co-morbidities in both. Co-existence of both disorders (overlap syndrome) occurs in 1% of adults and overlap patients have worse nocturnal hypoxemia and hypercapnia than COPD and OSA patients alone. The present review discusses recent data concerning the pathophysiological and clinical significance of the overlap syndrome.

Pulmonary cryptococcosis in rheumatoid arthritis (RA) patients: Comparison of imaging characteristics among RA, acquired immunodeficiency syndrome, and immunocompetent patients

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Yanagawa, Noriyo; Sakai, Fumikazu; Takemura, Tamiko; Ishikawa, Satoru; Takaki, Yasunobu; Hishima, Tsunekazu; Kamata, Noriko

2013-01-01

Purpose: The imaging characteristics of cryptococcosis in rheumatoid arthritis (RA) patients were analyzed by comparing them with those of acquired immunodeficiency syndrome (AIDS) and immunocompetent patients, and the imaging findings were correlated with pathological findings. Methods: Two radiologists retrospectively compared the computed tomographic (CT) findings of 35 episodes of pulmonary cryptococcosis in 31 patients with 3 kinds of underlying states (10 RA, 12 AIDS, 13 immunocompetent), focusing on the nature, number, and distribution of lesions. The pathological findings of 18 patients (8 RA, 2 AIDS, 8 immunocompetent) were analyzed by two pathologists, and then correlated with imaging findings. Results: The frequencies of consolidation and ground glass attenuation (GGA) were significantly higher, and the frequency of peripheral distribution was significantly lower in the RA group than in the immunocompetent group. Peripheral distribution was less common and generalized distribution was more frequent in the RA group than in the AIDS group. The pathological findings of the AIDS and immunocompetent groups reflected their immune status: There was lack of a granuloma reaction in the AIDS group, and a complete granuloma reaction in the immunocompetent group, while the findings of the RA group varied, including a complete granuloma reaction, a loose granuloma reaction and a hyper-immune reaction. Cases with the last two pathologic findings were symptomatic and showed generalized or central distribution on CT. Conclusion: Cryptococcosis in the RA group showed characteristic radiological and pathological findings compared with the other 2 groups

Pulmonary cryptococcosis in rheumatoid arthritis (RA) patients: Comparison of imaging characteristics among RA, acquired immunodeficiency syndrome, and immunocompetent patients

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Yanagawa, Noriyo, E-mail: noriyo_yana@ybb.ne.jp [Departments of Radiology, Tokyo Metropolitan Cancer and Infectious Diseases Center Komagome Hospital, 3-8-22, Honkomagome, Bunkyo-ku, Tokyo 113-8677 (Japan); Sakai, Fumikazu [Department of Diagnostic Radiology, Saitama Medical University International Medical Center, 1397-1 Yamane, Hidaka-shi, Saitama 350-1298 (Japan); Takemura, Tamiko [Department of Pathology, Japanese Red Cross Medical Center, 4-1-22 Hiroo, Shibuya-ku, Tokyo 150-8935 (Japan); Ishikawa, Satoru [Department of Respiratory Medicine, National Hospital Organization Chiba-East-Hospital, 673 Nitona-cho, Chuo-ku, Chiba-shi, Chiba 260-8712 (Japan); Takaki, Yasunobu [Departments of Radiology, Tokyo Metropolitan Cancer and Infectious Diseases Center Komagome Hospital, 3-8-22, Honkomagome, Bunkyo-ku, Tokyo 113-8677 (Japan); Hishima, Tsunekazu [Department of Pathology, Tokyo Metropolitan Cancer and Infectious Diseases Center Komagome Hospital, 3-8-22, Honkomagome, Bunkyo-ku, Tokyo 113-8677 (Japan); Kamata, Noriko [Departments of Radiology, Tokyo Metropolitan Cancer and Infectious Diseases Center Komagome Hospital, 3-8-22, Honkomagome, Bunkyo-ku, Tokyo 113-8677 (Japan)

2013-11-01

Purpose: The imaging characteristics of cryptococcosis in rheumatoid arthritis (RA) patients were analyzed by comparing them with those of acquired immunodeficiency syndrome (AIDS) and immunocompetent patients, and the imaging findings were correlated with pathological findings. Methods: Two radiologists retrospectively compared the computed tomographic (CT) findings of 35 episodes of pulmonary cryptococcosis in 31 patients with 3 kinds of underlying states (10 RA, 12 AIDS, 13 immunocompetent), focusing on the nature, number, and distribution of lesions. The pathological findings of 18 patients (8 RA, 2 AIDS, 8 immunocompetent) were analyzed by two pathologists, and then correlated with imaging findings. Results: The frequencies of consolidation and ground glass attenuation (GGA) were significantly higher, and the frequency of peripheral distribution was significantly lower in the RA group than in the immunocompetent group. Peripheral distribution was less common and generalized distribution was more frequent in the RA group than in the AIDS group. The pathological findings of the AIDS and immunocompetent groups reflected their immune status: There was lack of a granuloma reaction in the AIDS group, and a complete granuloma reaction in the immunocompetent group, while the findings of the RA group varied, including a complete granuloma reaction, a loose granuloma reaction and a hyper-immune reaction. Cases with the last two pathologic findings were symptomatic and showed generalized or central distribution on CT. Conclusion: Cryptococcosis in the RA group showed characteristic radiological and pathological findings compared with the other 2 groups.

Ectopic corticotroph syndrome

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Penezić Zorana

2004-01-01

Full Text Available INTRODUCTION Endogenous Cushing's syndrome is a clinical state resulting from prolonged, inappropriate exposure to excessive endogenous secretion of Cortisol and hence excess circulating free cortisol, characterized by loss of the normal feedback mechanisms of the hypothalamo-pituitary-adrenal axis and the normal circadian rhythm of cortisol secretion [2]. The etiology of Cushing's syndrome may be excessive ACTH secretion from the pituitary gland, ectopic ACTH secretion by nonpituitary tumor, or excessive autonomous secretion of cortisol from a hyperfunctioning adrenal adenoma or carcinoma. Other than this broad ACTH-dependent and ACTH-independent categories, the syndrome may be caused by ectopic CRH secretion, PPNAD, MAH, ectopic action of GIP or catecholamines, and other adrenel-dependent processes associated with adrenocortical hyperfunction. CASE REPORT A 31 year-old men with b-month history of hyperpigmentation, weight gain and proximal myopathy was refereed to Institute of Endocrinology for evaluation of hypercortisolism. At admission, patient had classic cushingoid habit with plethoric face, dermal and muscle atrophy, abdominal strie rubrae and centripetal obesity. The standard laboratory data showed hyperglycaemia and hypokaliemia with high potassium excretion level. The circadian rhythm of cortisol secretion was blunted, with moderately elevated ACTH level, and without cortisol suppression after low-dose and high-dose dexamethason suppression test. Urinary 5HIAA was elevated. Abdominal and sellar region magnetic resonance imaging was negative. CRH stimulation resulted in ACTH increase of 87% of basal, but without significant increase of cortisol level, only 7%. Thoracal CT scan revealed 14 mm mass in right apical pulmonary segment. A wedge resection of anterior segment of right upper lobe was performed. Microscopic evaluation showed tumor tissue consisting of solid areas of uniform, oval cells with eosinophilic cytoplasm and centrally

Unusual ventilation perfusion scintigram in a case of immunologic pulmonary edema clinically simulating pulmonary embolism

International Nuclear Information System (INIS)

Campeau, R.J.; Faust, J.M.; Ahmad, S.

1987-01-01

A case of immunologic pulmonary edema secondary to hydrochlorothiazide allergy developed in a 55-year-old woman that clinically simulated pulmonary embolism. The patient had abnormal washin images with normal washout images on an Xe-133 ventilation study. On the perfusion study, large bilateral central and posterior perfusion defects were present that showed an unusual mirror image pattern on the lateral and posterior oblique views. Resolution of radiographic and scintigraphic abnormalities occurred over a 3-day period in conjunction with corticosteroid therapy

Massive pulmonary cryptococcosis in an immunocompetent patient.

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Silachamroon, U; Shuangshoti, S

1998-03-01

A 64-year-old man presented with progressive dyspnea. The symptom of severe hypoxia requiring mechanical ventilator, and bilateral pulmonary infiltrates on the chest film led to the clinical diagnosis of adult respiratory distress syndrome. Autopsy demonstrated widespread cryptococci and mucinous material in alveoli with mild inflammatory response.

Cushing’s Syndrome and Hypothalamic–Pituitary–Adrenal Axis Hyperactivity in Chronic Central Serous Chorioretinopathy

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Femke M. van Haalen

2018-02-01

Full Text Available ObjectiveCentral serous chorioretinopathy (CSC, a specific form of macular degeneration, has been reported as presenting manifestation of Cushing’s syndrome. Furthermore, CSC has been associated with both exogenous hypercortisolism and endogenous Cushing’s syndrome. It is important to know whether CSC patients should be screened for Cushing’s syndrome. Although hypothalamic-pituitary-adrenal (HPA axis hyperactivity in CSC has been suggested, no detailed evaluation of the HPA axis has been performed in a large cohort of CSC patients. This study aimed to investigate whether Cushing’s syndrome prevalence is increased among chronic CSC (cCSC patients and whether detailed endocrinological phenotyping indicates hyperactivity of the HPA axis.DesignCross-sectional study.Patients86 cCSC patients and 24 controls.MeasurementsPrevalence of Cushing’s syndrome, HPA axis activity.ResultsNone of the cCSC patients met the clinical or biochemical criteria of Cushing’s syndrome. However, compared to controls, HPA axis activity was increased in cCSC patients, reflected by higher 24 h urinary free cortisol, and accompanying higher waist circumference and diastolic blood pressure, whereas circadian cortisol rhythm and feedback were not different. Chronic CSC patients did not report more stress or stress-related problems on questionnaires.ConclusionNo case of Cushing’s syndrome was revealed in a large cohort of cCSC patients. Therefore, we advise against screening for Cushing’s syndrome in CSC patients, unless additional clinical features are present. However, our results indicate that cCSC is associated with hyperactivity of the HPA axis, albeit not accompanied with perception of more psychosocial stress.

Effects of Laparoscopic Sleeve Gastrectomy on Central Obesity and Metabolic Syndrome in Indian Adults- A Prospective Study.

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Sethi, Pulkit; Thillai, Manoj; Nain, Prabhdeep Singh; Ahuja, Ashish; Vayoth, Sudheer Othiyil; Khurana, Preetika

2017-01-01

Increasing incidence of obesity in Indian population has led to an exponential rise in the number of bariatric operations performed annually. Laparoscopic Sleeve Gastrectomy (LSG) has been proposed to cause rapid remission of Type 2 Diabetes Melitus (T2DM) and metabolic syndrome in a weight loss independent manner. To evaluate the effects of LSG on metabolic syndrome and central obesity in morbidly and severely obese Indian adults. Material and Methods: Study was conducted on 91 morbidly obese [Body Mass Index (BMI)>40 kg/m 2 ] and severely obese (BMI>35 kg/m 2 ) individuals who were suffering from diabetes, hypertension or dyslipidemia. The patients were followed up for six months and the trends of glycaemic control, mean blood pressure, lipid profile, weight loss parameters and changes in parameters of central obesity were studied. Weight loss was significant at three months postsurgery and was sustained through six months. There was significant improvement in glycaemic control leading to reduction in need for oral hypoglycaemic agents or insulin in majority of them and even discontinuation of these medications in few patients. Hypertension and dyslipidemia also showed an improving trend through six months postsurgery. There was a significant impact on reduction of central obesity in these patients as marked by significant reduction in waist to hip ratio. LSG produces sustainable weight loss with significant improvement in glycaemic status and control of metabolic syndrome in severe to morbidly obese patients. LSG is also efficacious in reducing central obesity in Indian population which is a major depressive ailment amongst obese individuals.

The asthma-chronic obstructive pulmonary disease overlap syndrome (ACOS): opportunities and challenges.

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Barrecheguren, Miriam; Esquinas, Cristina; Miravitlles, Marc

2015-01-01

Some individuals share characteristics of asthma and chronic obstructive pulmonary disease (COPD). The asthma-COPD overlap syndrome (ACOS) has been defined as symptoms of increased variability of airflow in association with an incompletely reversible airflow obstruction. In this review, we present the latest findings in the diagnosis, characterization and management of ACOS. Around 15-20% of COPD patients may have an ACOS. Patients with ACOS are characterized by increased reversibility of airflow obstruction, eosinophilic bronchial and systemic inflammation, and increased response to inhaled corticosteroids, compared with the remaining patients with COPD. Patients with ACOS have more frequent exacerbations, more wheezing and dyspnoea, but similar cough and sputum production compared with COPD. The relevance of the ACOS is to identify patients with COPD who may have underlying eosinophilic inflammation that responds to inhaled corticosteroids. So far, the previous diagnosis of asthma in a patient with COPD is the more reliable criterion for ACOS. Ongoing studies will clarify if concentrations of blood eosinophils may be useful to identify this subgroup of patients with COPD. If this is the case, the interest of ACOS may shift to that of eosinophilic COPD, which is easier to diagnose and has clear therapeutic implications.

Pulmonary manifestations from systemic vasculitides; Pulmonale Manifestationen bei systemischen Vaskulitiden

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Reuter, M [Vivantes Klinikum Neukoelln, Berlin (Germany). Inst. fuer Radiologie und Interventionelle Therapie; Both, M [UKSH, Kiel (Germany). Klinik fuer Diagnostische Radiologie; Schnabel, A [Sana-Rheumazentrum Baden-Wuerttemberg, Bad Wildbad (Germany). Klinik fuer Internistische Rheumatologie und Klinische Immunologie

2007-06-15

Pulmonary vasculitides predominantly involve the small arterioles, capillaries and venules and include Wegener's granulomatosis, microscopic polyangiitis and the Churg-Strauss syndrome. Takayasu's arteriitis is a large vessel disease and may affect the main pulmonary arteries causing stenoses and occlusions. Knowledge of the natural course of disease and of clinical manifestations of pulmonary disease is helpful for an understanding of imaging findings. For this reason this article gives an overview not only of radiologic findings in chest X-ray and high resolution CT of the lungs but as well of clinical aspects of pulmonary vasculitides. Next to determination of disease extension the determination of disease activity is in the foreground of diagnostic imaging in vasculitides. Within this context principals of immunosuppressive therapy will be recognized. (orig.)

Diagnosis, Evaluation and Treatment of Pulmonary Arterial Hypertension in Children

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Frank, Benjamin S.

2018-01-01

Pulmonary Hypertension (PH), the syndrome of elevated pressure in the pulmonary arteries, is associated with significant morbidity and mortality for affected children. PH is associated with a wide variety of potential underlying causes, including cardiac, pulmonary, hematologic and rheumatologic abnormalities. Regardless of the cause, for many patients the natural history of PH involves progressive elevation in pulmonary arterial resistance and pressure, right ventricular dysfunction, and eventually heart failure. In recent years, a number of pulmonary arterial hypertension (PAH)-targeted therapies have become available to reduce pulmonary artery pressure and improve outcome. A growing body of evidence in both the adult and pediatric literature demonstrates enhanced quality of life, functional status, and survival among treated patients. This review provides a description of select etiologies of PH seen in pediatrics and an update on the most recent data pertaining to evaluation and management of children with PH/PAH. The available evidence for specific classes of PAH-targeted therapies in pediatrics is additionally discussed. PMID:29570688

[The repercussions of pulmonary congestion on ventilatory volumes, capacities and flows].

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Carmo, M M; Ferreira, T; Lousada, N; Bárbara, C; Neves, P R; Correia, J M; Rendas, A B

1994-10-01

To evaluate the effects of pulmonary congestion on pulmonary function. Prospective study performed in patients with left ventricular failure or mitral stenosis. Forty-eight hospitalized patients were included suffering from pulmonary congestion either from left ventricular failure or mitral stenosis. While in hospital all patients were submitted to right heart catheterization by the Swan-Ganz method and also to an echocardiographic examination. Within 48 hours after the patients were submitted to the following lung function studies: lung volumes and capacities by the multi-breath helium dilution method and airway flows by pneumotachography. Respiratory symptoms were evaluated by the Medical Research Council Questionnaire and the functional class classified according to the NYHA. Correlations were made between the functional and clinical data. Regarding the cardiac evaluation patients presented with a mean pulmonary wedge pressure of 19.9 +/- 8.6 mmHg, a cardiac index of 2.5 +/- 0.8 l/min/m2, an end diastolic dimension of the left ventricle of 65.9 +/- 10.1 mm, and end systolic dimension of 51.2 +/- 12.2 mm, with a shortening fraction of 21.8 +/- 9.5%. The pulmonary evaluation showed a restrictive syndrome with a reduction in the mean values of the following parameters: total pulmonary capacity 71 +/- 14.4% of the predicted value (pv), forced vital capacity (FVC) 69.8 +/- 20.5% pv, and forced expiratory volume (FEV1) of 64 +/- 21.8% vp. The index FEV1/FVC was within the normal value of 72.7 +/- 9.7%. These lung function results did not correlate significantly with either the clinical, the hemodynamic or echocardiographic findings. In these group of patients pulmonary congestion led to the development of a restrictive syndrome which failed to correlate in severity with the duration of the disease, the pulmonary wedge pressure and the left ventricular function.

Sleeping problems in mothers and fathers of patients suffering from congenital central hypoventilation syndrome.

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Paddeu, Erika Maria; Giganti, Fiorenza; Piumelli, Raffaele; De Masi, Salvatore; Filippi, Luca; Viggiano, Maria Pia; Donzelli, Gianpaolo

2015-09-01

Advanced medical technology has resulted in an increased survival rate of children suffering from congenital central hypoventilation syndrome. After hospitalization, these technology-dependent patients require special home care for assuring ventilator support and the monitoring of vital parameters mainly during sleep. The daily challenges associated with caring for these children can place primary caregivers under significant stress, especially at night. Our study aimed at investigating how this condition affects mothers and fathers by producing poor sleep quality, high-level diurnal sleepiness, anxiety, and depression. The study included parents of 23 subjects with congenital central hypoventilation syndrome and 23 healthy subjects. All parents filled out the Pittsburgh Sleep Quality Index (PSQI) questionnaire, Epworth Sleepiness Scale (ESS), Beck Depression Inventory (BDI-II), and Beck Anxiety Inventory (BAI). A comparison between the two groups showed that parents of patients had poorer sleep quality, greater sleepiness, and higher BDI-II scores compared to that of parents of healthy subjects (respectively, PSQI score 6.5 vs 3.8, ESS score 6.2 vs 4.3, BDI-II score 8.4 vs 5.7). Specifically, mothers of patients showed poorer sleep quality and higher BDI-II scores compared to that of mothers of controls (respectively, PSQI score 7.5 vs 3.8, BDI-II score 9.3 vs 5.9), whereas fathers of patients showed greater levels of sleepiness with respect to fathers of healthy children (respectively, ESS score 6.8 vs 4.0). These differences emerged in parents of younger children. Congenital central hypoventilation syndrome impacts the family with different consequences for mothers and fathers. Indeed, while the patients' sleep is safeguarded, sleeping problems may occur in primary caregivers often associated with other psychological disorders. Specifically, this disease affects sleep quality and mood in the mothers and sleepiness levels in the fathers.

Familial pulmonary arterial hypertension, leucopenia, and atrial septal defect: a probable new familial syndrome with multisystem involvement.

Science.gov (United States)

Dursun, Ali; Ozgul, R Koksal; Soydas, Asli; Tugrul, Tugba; Gurgey, Aytemiz; Celiker, Alpay; Barst, Robyn J; Knowles, James A; Mahesh, Mansukhani; Morse, Jane H

2009-01-01

We present two siblings with identical clinical findings that seem to represent a previously unreported familial syndrome. Major findings involve three systems: pulmonary arterial hypertension, cardiac abnormalities including secundum-type atrial septal defect, and the hematopoietic system with intermittent neutropenia, lymphopenia, monocytosis, and anemia. The siblings also shared several minor abnormalities: pectus carinatum, long fingers, proximally placed thumb, broad nasal bridge, and high-arched palate. The male proband also had bilateral inguinal hernias and undescended testes. The same findings in two siblings suggest a genetic cause--either an autosomal recessive disorder or germline mosaicism in one parent for a dominant mutation. Investigations revealed a bone morphogenetic protein receptor 2 polymorphism in intron 4 in only one sibling, which was also present in unaffected maternal relatives.

INFILTRATE PULMONARY TUBERCULOSIS, GASTRIC ULCER AND HIV INFECTION (COMORBIDITY AND MULTIMORBIDITY

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E. S. Skvortsova

2016-01-01

Full Text Available Peptic ulcer is registered in 20,5% of those suffering from infiltrate pulmonary tuberculosis and in 19,5% of HIV patients at C2 stage, ill with infiltrate pulmonary tuberculosis and presenting complaints about dyspeptic disorders. The following is typical of multimorbidity of pulmonary tuberculosis, HIV infection and peptic ulcer: oligosymptomatic start of tuberculosis, asthenic syndrome dominating among clinical symptoms, manifestations of gastric and intestinal indigestion, weight loss (2-4 times more often compared to HIV negative patients, less expressed lung tissue destruction (2 time less compared to HIV negative patients. H. pylori is a causative agent of the peptic ulcer in 62,5% of patients with infiltrate pulmonary tuberculosis and in 58,7% of HIV infected patients at C2 stage, suffering from pulmonary tuberculosis. Comorbidity of H. pilori negative peptic ulcer and infiltrate pulmonary tuberculosis differs from comorbidity of H. pilori positive peptic ulcer and infiltrate pulmonary tuberculosis since the first has confidently true unfavorable forecast of the outcome.

Cardiac and pulmonary artery mensuration in feline heartworm disease

International Nuclear Information System (INIS)

Schafer, M.; Berry, C.R.

1995-01-01

A retrospective study was undertaken to quantify thoracic radiographic changes in cats with heartworm diseases, (Dirofilaria immitis). Using a blinded study format, the cardiac silhouette, thoracic cavity and pulmonary arteries were measured from thoracic radiographs of 21 cats with feline heartworm disease and 30 cats without known cardiac or pulmonary vessel pathology. Measured data were normalized to the thoracic cavity or bony structures within the radiographic field of view. The measurements were compared between the two groups of cats using an unpaired, two-tailed Student's t-test, with a p value of < 0.05 being considered significant. Cats with feline heartworm disease had enlargement of the craniocaudal aspect of the cardiac silhouette and normalized cardiac:thoracic ratio (p < 0.05) on the lateral view. Also, there was significant enlargement of the central and peripheral caudal lobar pulmonary arteries and their normalized ratios (p < 0.05) in the heartworm infected cats as visualized on the ventrodorsal projection. Tortuosity of the pulmonary arteries was seen in three of the 21 infected cats. Eleven of the 21 cats with feline heartworm disease had pulmonary parenchymal changes. Based on the present study, central and peripheral pulmonary artery enlargement as viewed on the ventrodorsal radiograph was the single best radiographic indicator of feline heartworm disease

Obstructive sleep apnea in chronic obstructive pulmonary disease patients.

LENUS (Irish Health Repository)

Lee, Ruth

2012-02-01

PURPOSE OF REVIEW: Chronic obstructive pulmonary disease (COPD) and obstructive sleep apnea (OSA) represent two of the most prevalent chronic respiratory disorders and cardiovascular diseases are major co-morbidities in both. Co-existence of both disorders (overlap syndrome) occurs in 1% of adults and overlap patients have worse nocturnal hypoxemia and hypercapnia than COPD and OSA patients alone. The present review discusses recent data concerning the pathophysiological and clinical significance of the overlap syndrome. RECENT FINDINGS: The severity of obstructive ventilatory impairment and hyperinflation, especially the inspiratory capacity to total lung capacity (TLC) ratio, correlates with the severity of sleep-related breathing disturbances. Early treatment with continuous positive airway pressure (CPAP) improves survival, reduces hospitalization and pulmonary hypertension, and also reduces hypoxemia. Evidence of systemic inflammation and oxidative stress in COPD and sleep apnea provides insight into potential interactions between both disorders that may predispose to cardiovascular disease. Long-term outcome studies of overlap patients currently underway should provide further evidence of the clinical significance of the overlap syndrome. SUMMARY: Studies of overlap syndrome patients at a clinical, physiological and molecular level should provide insight into disease mechanisms and consequences of COPD and sleep apnea, in addition to identifying potential relationships with cardiovascular disease.

Imaging diagnosis of pulmonary tuberculosis in immunocompromised patients

International Nuclear Information System (INIS)

Ma Daqing; Zhao Dawei; Pan Keqin

2000-01-01

Objective: To evaluate CT and X-ray features of pulmonary tuberculosis in diabetic patients, patients post kidney transplantation, and patients with acquired immunodeficiency syndrome (AIDS). Methods: The authors reviewed CT scans in 20 patients with diabetic patients, X-ray films in 10 cases after kidney transplantation, and CT scans in 2 patients with AIDS. Results: CT features of pulmonary tuberculosis in diabetic diseases included larger confluent consolidation (10 cases ), multiple small cavities within any given lesion (9 cases ) and non-segmental distribution (2 cases). Satellite lesions were found in most films. The X-ray appearances of pulmonary tuberculosis post kidney transplantation included patch and larger confluent consolidation (6 cases), and miliary tuberculosis(4 cases). The CT findings of pulmonary tuberculosis with AIDS were enlarged mediastinal lymph nodes (1 case), pulmonary infiltration (1 case), and extra chest lesions(2 cases) such as enlarged neck lymph nodes and post-peritoneal lymph nodes. Conclusion: The Main radiological findings of pulmonary tuberculosis in immunocompromised patients appear larger confluent consolidation, multiple small cavities within a given lesion, miliary tuberculosis, enlarged mediastinal lymph nodes, and extra chest enlarged lymph nodes

Association of chronic obstructive pulmonary disease and obstructive sleep apnea consequences

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Carlos Zamarrón

2009-01-01

Full Text Available Carlos Zamarrón1, Vanesa García Paz1, Emilio Morete1, Felix del Campo Matías21Servicio de Neumología, Hospital Clínico Universitario de Santiago, Santiago, Spain; 2Servicio de Neumologia, Hospital Universitario Rio Ortega de Vallaclolid, Vallaclolid, SpainAbstract: Obstructive sleep apnea syndrome (OSAS and chronic obstructive pulmonary disease (COPD are two diseases that often coexist within an individual. This coexistence is known as overlap syndrome and is the result of chance rather than a pathophysiological link. Although there are claims of a very high incidence of OSAS in COPD patients, recent studies report that it is similar to the general population. Overlap patients present sleep-disordered breathing associated to upper and lower airway obstruction and a reduction in respiratory drive. These patients present unique characteristics, which set them apart from either COPD or OSAS patients. COPD and OSAS are independent risk factors for cardiovascular events and their coexistence in overlap syndrome probably increases this risk. The mechanisms underlying cardiovascular risk are still unclear, but may involve systemic inflammation, endothelial dysfunction, and tonic elevation of sympathetic neural activity. The treatment of choice for overlap syndrome in stable patients is CPAP with supplemental oxygen for correction of upper airway obstructive episodes and hypoxemia during sleep.Keywords: chronic obstructive pulmonary disease, obstructive sleep apnea syndrome, overlap syndrome, sleep, cardiovascular disease

Total ginsenosides synergize with ulinastatin against septic acute lung injury and acute respir atory distress syndrome

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Sun, Rongju; Li, Yana; Chen, Wei; Zhang, Fei; Li, Tanshi

2015-01-01

Total ginsenosides synergize with ulinastatin (UTI) against septic acute lung injury (ALI) and acute respiratory distress syndrome (ARDS). We randomly divided 80 cases of severe sepsis-induced ALI and ARDS into a UTI group and a ginsenosides (GS)+UTI group. Continuous electrocardiac monitoring of pulse, respiratory rate, blood pressure, and heart rate; invasive hemodynamic monitoring; ventilator-assisted breathing and circulation support; and anti-infection as well as UTI treatment were given in the UTI group with GS treatment added for 7 consecutive days in the GS+UTI group. The indicators of pulmonary vascular permeability, pulmonary circulation, blood gases, and hemodynamics as well as APACHE II and ALI scores were detected on days 1, 3, and 7. The ALI score in the GS+UTI group was significantly decreased (P UTI group, and the indicators of pulmonary capillary permeability such as pulmonary vascular permeability index, extravascular lung water index, and oxygenation index, in the GS+UTI group improved significantly more than that of the UTI group. The indicators of hemodynamics and pulmonary circulation such as cardiac index, intrathoracic blood volume index, and central venous pressure improved significantly (P UTI group was lower than that of the UTI group. GS can effectively collaborate with UTI against ALI and/or ARDS. PMID:26261640

Catastrophic Antiphospholipid Syndrome Presenting as Bilateral Central Retinal Artery Occlusions

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Steven S. Saraf

2015-01-01

Full Text Available A previously healthy 22-year-old African American woman presented with bilateral vision loss associated with headache. Her ocular examination was significant for bilateral retinal arterial “boxcarring,” retinal whitening, retinal hemorrhages, and cherry red spots. She was diagnosed with bilateral central retinal artery occlusions and was hospitalized due to concomitant diagnosis of stroke and hypercoagulable state. She was also found to be in heart failure and kidney failure. Rheumatology was consulted and she was diagnosed with catastrophic antiphospholipid syndrome in association with systemic lupus erythematosus. Approximately 7 months after presentation, the patient’s vision improved and remained stable at 20/200 and 20/80.

Persistent pulmonary hypertension of the newborn

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Teng, Ru-Jeng; Wu, Tzong-Jin

2013-01-01

Persistent pulmonary hypertension of the newborn (PPHN) is a severe pulmonary disorder which occurs one in every 500 live births. About 10–50% of the victims will die of the problem and 7–20% of the survivors develop long term impairments such as hearing deficit, chronic lung disease, and intracranial bleed. Most of the adult survivors show evidence of augmented pulmonary vasoreactivity suggesting a phenotypical change. Several animal models have been used to study the pathophysiology and help to develop new therapeutic modality for PPHN. The etiology of PPHN can be classified into three groups: [A] abnormally constricted pulmonary vasculature due to parenchymal diseases; [B] hypoplastic pulmonary vasculature; [C] normal parenchyma with remodeled pulmonary vasculature. Impaired vasorelaxation of pulmonary artery and reduced blood vessel density in lungs are two characteristic findings in PPHN. Medical treatment includes sedation, oxygen, mechanical ventilation, vasorelaxants (inhaled nitric oxide, inhaled or intravenous prostacyclin, intravenous prostaglandin E1, magnesium sulfate), and inotropic agents. Phosphodiesterase inhibitor has recently been studied as another therapeutic agent for PPHN. Endothelin-1 (ET-1) inhibitor has been studied in animal and a case of premature infant with PPHN successfully treated with ET-I inhibitor has been reported in the literature. Surfactant has been reported as an adjunct treatment for PPHN as a complication of meconium aspiration syndrome. Even with the introduction of several new therapeutic modalities there has no significant change in survival rate. Extracorporeal membrane oxygenator is used when medical treatment fails and patient is considered to have a recoverable cause of PPHN. PMID:23537863

Evaluation of the angiographic findings in pulmonary atresia

International Nuclear Information System (INIS)

Choe, Kyu Ok; Sul, Jun Hee; Lee, Seung Kyu; Cho, Bum Koo; Hong, Pill Whoon

1986-01-01

We studied the angiographic findings in 65 patients with congenital pulmonary atresia, ages 4 days to 14 years (mean 3.3 yrs), from 1981 to 1986 at Severance Hospital Yonsei University. 1. 6 had pulmonary atresia with an intact interventricular septum, 38 had it with cardiac anomaly Renodynamically simulating TOF, and 21 associated with more complicated cardiac anomalies. 2. In the group with an intact ventricular septum, 5 showed confluent intrapericardial pulmonary artery, all segmental pulmonary arteries connected to intrapericardial artery. 3. In the group simulating TOF, aorta arose from RV with or without overriding in 35. In 27 patients with confluent intrapericardial pulmonary artery, 23 had more than 10 segmental pulmonary arteries connected to intrapericardial artery and 5 had severely hypoplastic hilar pulmonary arteries. In 11 with non confluent intrapericardial pulmonary artery, 4 had more than 10 segmental pulmonary arteries connected to central pulmonary artery and 9 had severely hypoplastic hilar pulmonary arteries. 4. In the group associated with more complicated cardiac anomaly, included 8 patients with atrioventricular discordance, 7 with univentricular heart and 6 with tricuspid atresia. In 17 patients with confluent intrapericardial artery, 16 had more than 10 segmental pulmonary arteries connected to intrapericardial artery, one showed severe hypoplasia of hilar pulmonary arteries. In another 4 with non confluence, no one showed more than 10 segmental arteries connected to intrapericardial or hilar pulmonary artery.

[Hepatopulmonary syndrome and portopulmonary hypertension].

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Marcu, Cristina; Schiffer, Eduardo; Aubert, John-David; Vionnet, Julien; Yerly, Patrick; Deltenre, Pierre; Marot, Astrid

2017-08-30

Hepatopulmonary syndrome (HPS) and portopulmonary hypertension (POPH) are two frequent pulmonary complications of liver disease. Portal hypertension is a key element in the pathogenesis of both disorders, which are however distinct in terms of pathogenesis, diagnosis and treatment. HPS corresponds to an abnormal arterial oxygenation in relation with the development of intrapulmonary vascular dilatations. POPH is a pulmonary arterial hypertension in the setting of portal hypertension and elevated pulmonary vascular resistance. As both diseases are associated with an increased risk of morbidity and mortality, it is important to screen and evaluate the severity of these two disorders particularly in liver transplant candidates.

Meningoencephalitis, pancytopenia, pulmonary insufficiency and splenic abscess in a patient with brucellosis

International Nuclear Information System (INIS)

Cokca, F.; Yilmaz-Bozkurt, G.; Azap, A.; Memikoglu, O.; Takeli, E.

2006-01-01

A complicated case of brucellosis with some rare features is reported. Brucellosis is a multisystematic disease. However, disseminated brucellosis with cerebral, pulmonary, hematopoietic and splenic involvement in an otherwise healthy patient is a rare event. In this article, we report a case of disseminated brucellosis who was initially diagnosed as myeldoplastic syndrome (MDS) and meningoencephalitis, pulmonary symptoms, and splenic abscess formation occurred thereafter. (author)

[Pulmonary hypertension of the newborn--recent advances in the management and treatment].

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Vakrilova, L; Radulova, P; Hitrova, St; Slancheva, B

2014-01-01

Pulmonary hypertension of the newborn is a clinical syndrome with diverse etiology in which the transition from fetal circulation with high pulmonary vascular resistance to postnatal circulation with low pulmonary vascular resistance failed. The persistence of high pulmonary vascular pressure leads to right-left shunts and marked cyanosis. Despite of the advances in neonatology, the treatment of some forms of PPHN is often difficult and mortality rate remains high. In infants with PPHN appropriate interventions are critical to reverse hypoxemia, improve pulmonary and systemic perfusion and preserve end-organ function. Our understanding for management of PPHN has evaluated over decades. This review summarizes the current strategies for treatment of pulmonary hypertension of the newborn: general care, cardiovascular support, the advantages and limitations of different ventilatory strategies, oxygen therapy, extracorporal membrane oxygenation, and the evidence-based inhaled nitric oxide therapy. The balance between pulmonary vasoconstrictor and vasodilator mediators plays an important role for pulmonary vascular resistance. Recent studies are designed to develop evidence-based therapies for regulation of pulmonary vascular tone, safe medications for selective pulmonary vasodilatation effective for treatment of PPHN and other forms of pulmonary hypertension in the neonatal intensive care unit.

[Thrombosis and post-thrombotic syndrome as a consequence of an accident].

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Wahl, U; Hirsch, T

2015-10-01

Phlebothromboses represent alarming complications in accident victims since they can cause fatal pulmonary embolisms. More than half of those affected also develop post-thrombotic syndrome in the course of the illness. In addition to making clinical assessments, the traumatologist should also have fundamental knowledge about diagnostic methods and be familiar with interpreting internal findings. Colour-coded duplex sonography plays a central role in diagnosing thrombosis and in assessing functional limitations. Further information can be gathered from various phlebological procedures. The expert evaluation of the immediate, as well as the long-term consequences of an accident frequently require leg swelling to be classified. It is not uncommon for post-thrombotic syndrome to be diagnosed for the first time during this process. An additional vascular appraisal is often required. An appreciation of social-medical and insurance-related aspects means a high degree of responsibility is placed on the expert.

Economy class syndrome: still a recurrent complication of long journeys.

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Feltracco, Paolo; Barbieri, Stefania; Bertamini, Francesca; Michieletto, Elisa; Ori, Carlo

2007-04-01

Economy class syndrome is a rare but still unavoidable complication of long haul flights, particularly in patients who carry various intrinsic risk factors. The tendency to affect even asymptomatic young people and the greater risk to fragment and propagate to the pulmonary circulation are the main characteristics of deep vein thrombosis of long-flight travelers. We report the clinical history of eight patients admitted to intensive care unit for confirmed or highly suspected economy class syndrome. Seven of them developed the syndrome within 72 h from a long return flight, one suffered from pulmonary embolism after a 12-h car trip. Two out of eight patients died, one because of extremely severe hemodynamic impairment, the other as a consequence of multiple organ failure caused by a concomitant myocardial infarction. Deep vein thrombosis and pulmonary embolism represent one of the main medical problems of air travel and cause almost 20% of deaths in people with no medical history. Although economy class syndrome occurs mostly in elderly, even the healthy young population can be affected and, in fact, three out of eight patients of our series were under 50 years of age. All our patients but one carried a well recognized risk factor for deep vein thrombosis. Clinical symptoms of deep vein thrombosis can sometimes be aspecific and confusing, so that a certain proportion of post-travel deep vein thrombosis, evolving favorably and not giving rise to pulmonary embolism, might effectively remain undiagnosed. Economy class syndrome is still quite difficult to deal with and controversial in terms of preventive strategies.

Milrinone for persistent pulmonary hypertension of the newborn.

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Bassler, Dirk; Kreutzer, Karen; McNamara, Patrick; Kirpalani, Haresh

2010-11-10

Persistent pulmonary hypertension of the newborn (PPHN) is a clinical syndrome characterized by suboptimal oxygenation as a result of sustained elevation in pulmonary vascular resistance after birth. Currently, the therapeutic mainstay for PPHN is optimal lung inflation and selective vasodilatation with inhaled nitric oxide (iNO). However, iNO is not available in all countries and not all infants will respond to iNO. Milrinone is a phosphodiesterase III inhibitor which induces pulmonary vasodilatation by its actions through a cyclic adenylate monophosphate mediated signaling pathway. To assess efficacy and safety in infants with PPHN either treated with: milrinone compared with placebo or no treatment; milrinone compared with iNO; milrinone as an adjunct to iNO compared with iNO alone; milrinone compared with potential treatments for PPHN other than iNO. We searched the Cochrane Central Register of Controlled Trials (The Cochrane Library, Issue 2, 2010), MEDLINE and EMBASE databases from their inception until January 2010. We searched the reference lists of potentially relevant studies without any language restriction. Fully published randomized controlled trials (RCTs) and quasi-RCTs comparing milrinone with placebo, iNO or potential treatments other than iNO in neonates with PPHN were included if trials reported any clinical outcome. We found no studies meeting the criteria for inclusion in this review. We found no studies meeting the criteria for inclusion in this review. The efficacy and safety of milrinone in the treatment of PPHN are not known and its use should be restricted within the context of RCTs. Such studies should address a comparison of milrinone with placebo (in clinical situations where iNO is not available) or, in well resourced countries, should compare milrinone with iNO or as an adjunct to iNO compared with iNO alone.

Pathogenesis of Acute Respiratory Distress Syndrome

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A. M. Golubev

2012-01-01

Full Text Available Acute respiratory distress syndrome (ARDS is a common complication of many diseases. Its polyetiological pattern determines the specific features of lung morphological changes and the clinical course of ARDS. Objective: to analyze the pathogenesis of ARDS in the context of the general pathological processes underlying its development. Material and methods. More than 200 lungs from the people who had died from severe concomitant injury or ARDS-complicated pneumonia were investigated. More than 150 rat experiments simulated various types of lung injury: ventilator-induced lung injury with different ventilation parameters; reperfusion injuries (systemic circulation blockade due to 12-minute vascular fascicle ligation, followed by the recovery of cardiac performance and breathing; microcirculatory disorder (injection of a thromboplastin solution into the jugular vein; blood loss; betaine-pepsin aspiration; and closed chest injury. Different parts of the right and left lungs were histologically examined 1 and 3 hours and 1 and 3 days after initiation of the experiment. Lung pieces were fixed in 10% neutral formalin solution and embedded in paraffin. Histological sections were stained with hematoxylin and eosin and using the van Gieson and Weigert procedures; the Schiff test was used. Results. The influence of aggression factors (trauma, blood loss, aspiration, infection, etc. results in damage to the lung and particularly air-blood barrier structures (endothelium, alveolar epithelium, their basement membrane. In turn the alteration of cellular and extracellular structures is followed by the increased permeability of hemomicrocirculatory bed vessels, leading to the development of non-cardiogenic (interstitial, alveolar pulmonary edema that is a central component in the pathogenesis of ARDS. Conclusion. The diagnosis of the early manifestations of ARDS must account for the nature of an aggression factor, the signs confirming the alteration of the lung

Theory of Mind and Central Coherence in Adults with High-Functioning Autism or Asperger Syndrome

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Beaumont, Renae; Newcombe, Peter

2006-01-01

The study investigated theory of mind and central coherence abilities in adults with high-functioning autism (HFA) or Asperger syndrome (AS) using naturalistic tasks. Twenty adults with HFA/AS correctly answered significantly fewer theory of mind questions than 20 controls on a forced-choice response task. On a narrative task, there were no…

Localized pulmonary crystal-storing histiocytosis complicating pulmonary mucosa-associated lymphoid tissue lymphoma presenting with multiple mass lesions.

Science.gov (United States)

Kokuho, Nariaki; Terasaki, Yasuhiro; Kunugi, Shinobu; Onda, Naomi; Urushiyama, Hirokazu; Terasaki, Mika; Hino, Mitsunori; Gemma, Akihiko; Hatori, Tsutomu; Shimizu, Akira

2017-07-01

Crystal-storing histiocytosis (CSH) is an uncommon finding in lymphoplasmacytic disorders that presents histiocytes with abnormal intralysosomal accumulations of immunoglobulin light chains as crystals of unknown etiology. A 38-year-old woman with antiphospholipid syndrome had a surgical lung biopsy because of multiple lung mass lesions. In a right middle lobe lesion, lymphoplasmacytic cells had a monocytoid appearance, destructive lymphoepithelial lesions, and positive immunoglobulin heavy chain (IGH) gene rearrangements. A right upper lobe lesion manifested proliferating rounded histiocytes with abundant, deeply eosinophilic cytoplasm and negative IGH gene rearrangements. Electron microscopy and mass spectrometry revealed a case of pulmonary CSH: abnormal proliferation of the immunoglobulin κ chain of a variable region that may be crystallized within plasma cells and histiocytes. We report a rare case of localized pulmonary CSH complicating pulmonary mucosa-associated lymphoid tissue lymphoma with multiple mass lesions. We demonstrate advances in the understanding of the pathogenesis of CSH by various analyses of these lesions. Copyright © 2017 Elsevier Inc. All rights reserved.

Congenital unilateral absence of the pulmonary artery in adults

International Nuclear Information System (INIS)

Gonzalez Garcia, Mauricio; Escalante Mora, Hector A; Lozano Castillo, Alfonso J

2000-01-01

Unilateral absence of a pulmonary artery is a rare anomaly. It occurs with pulmonary ipsilateral hypoplasia and it's frequently associated with other cardiovascular malformations. The majority of the cases are diagnosed in childhood. This is a case report of two adult patients of the Hospital Central de la Policia Nacional in Bogota, Colombia, with unilateral absence of the pulmonary artery one isolated and the other with patent ductus arteriosus. We describe the clinical and roentgenographic findings of this congenital anomaly

Pulmonary phenotypes associated with genetic variation in telomere-related genes.

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Hoffman, Thijs W; van Moorsel, Coline H M; Borie, Raphael; Crestani, Bruno

2018-05-01

Genomic mutations in telomere-related genes have been recognized as a cause of familial forms of idiopathic pulmonary fibrosis (IPF). However, it has become increasingly clear that telomere syndromes and telomere shortening are associated with various types of pulmonary disease. Additionally, it was found that also single nucleotide polymorphisms (SNPs) in telomere-related genes are risk factors for the development of pulmonary disease. This review focuses on recent updates on pulmonary phenotypes associated with genetic variation in telomere-related genes. Genomic mutations in seven telomere-related genes cause pulmonary disease. Pulmonary phenotypes associated with these mutations range from many forms of pulmonary fibrosis to emphysema and pulmonary vascular disease. Telomere-related mutations account for up to 10% of sporadic IPF, 25% of familial IPF, 10% of connective-tissue disease-associated interstitial lung disease, and 1% of COPD. Mixed disease forms have also been found. Furthermore, SNPs in TERT, TERC, OBFC1, and RTEL1, as well as short telomere length, have been associated with several pulmonary diseases. Treatment of pulmonary disease caused by telomere-related gene variation is currently based on disease diagnosis and not on the underlying cause. Pulmonary phenotypes found in carriers of telomere-related gene mutations and SNPs are primarily pulmonary fibrosis, sometimes emphysema and rarely pulmonary vascular disease. Genotype-phenotype relations are weak, suggesting that environmental factors and genetic background of patients determine disease phenotypes to a large degree. A disease model is presented wherever genomic variation in telomere-related genes cause specific pulmonary disease phenotypes whenever triggered by environmental exposure, comorbidity, or unknown factors.

Impact of Major Pulmonary Resections on Right Ventricular Function: Early Postoperative Changes.

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Elrakhawy, Hany M; Alassal, Mohamed A; Shaalan, Ayman M; Awad, Ahmed A; Sayed, Sameh; Saffan, Mohammad M

2018-01-15

Right ventricular (RV) dysfunction after pulmonary resection in the early postoperative period is documented by reduced RV ejection fraction and increased RV end-diastolic volume index. Supraventricular arrhythmia, particularly atrial fibrillation, is common after pulmonary resection. RV assessment can be done by non-invasive methods and/or invasive approaches such as right cardiac catheterization. Incorporation of a rapid response thermistor to pulmonary artery catheter permits continuous measurements of cardiac output, right ventricular ejection fraction, and right ventricular end-diastolic volume. It can also be used for right atrial and right ventricular pacing, and for measuring right-sided pressures, including pulmonary capillary wedge pressure. This study included 178 patients who underwent major pulmonary resections, 36 who underwent pneumonectomy assigned as group (I) and 142 who underwent lobectomy assigned as group (II). The study was conducted at the cardiothoracic surgery department of Benha University hospital in Egypt; patients enrolled were operated on from February 2012 to February 2016. A rapid response thermistor pulmonary artery catheter was inserted via the right internal jugular vein. Preoperatively the following was recorded: central venous pressure, mean pulmonary artery pressure, pulmonary capillary wedge pressure, cardiac output, right ventricular ejection fraction and volumes. The same parameters were collected in fixed time intervals after 3 hours, 6 hours, 12 hours, 24 hours, and 48 hours postoperatively. For group (I): There were no statistically significant changes between the preoperative and postoperative records in the central venous pressure and mean arterial pressure; there were no statistically significant changes in the preoperative and 12, 24, and 48 hour postoperative records for cardiac index; 3 and 6 hours postoperative showed significant changes. There were statistically significant changes between the preoperative and

Obesity and Pulmonary Hypertension: A Review of Pathophysiologic Mechanisms

Directory of Open Access Journals (Sweden)

Scott E. Friedman

2012-01-01

Full Text Available Pulmonary hypertension (PH is a potentially life-threatening condition arising from a wide variety of pathophysiologic mechanisms. Effective treatment requires a systematic diagnostic approach to identify all reversible mechanisms. Many of these mechanisms are relevant to those afflicted with obesity. The unique mechanisms of PH in the obese include obstructive sleep apnea, obesity hypoventilation syndrome, anorexigen use, cardiomyopathy of obesity, and pulmonary thromboembolic disease. Novel mechanisms of PH in the obese include endothelial dysfunction and hyperuricemia. A wide range of effective therapies exist to mitigate the disability of PH in the obese.

Ordovas-Oxidized LDL is associated with metabolic syndrome traits independently of central obesity and insulin resistance

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This study assesses whether oxidative stress, using oxidized LDL (ox-LDL) as a proxy, is associated with metabolic syndrome (MS), whether ox-LDL mediates the association between central obesity and MS, and whether insulin resistance mediates the association between ox-LDL and MS. We examined baselin...

'A one-sided affair': unilateral pulmonary oedema and the role of cardiac MRI in diagnosing premature coronary artery disease in a patient with Prader-Willi syndrome.

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Jabbar, Avais; Khan, Jamal N; Singh, Anvesha; McCann, Gerry P

2013-05-22

There is no formal association between premature coronary artery disease (CAD) and Prader-Willi syndrome despite its association with hyperlipidaemia, diabetes mellitus and hypertension. A 36-year-old man with Prader-Willi syndrome presented with acute breathlessness. Inflammatory markers were borderline elevated and chest radiography demonstrated unilateral diffuse alveolar shadowing. Bronchopneumonia was diagnosed and despite treatment with multiple courses of antimicrobial therapy, there was minimal symptomatic and radiographical improvement. A diagnosis of unilateral pulmonary oedema was suspected. Echocardiography was non-diagnostic due to body habitus and coronary angiography was deemed inappropriate due to uncertainty in diagnosis, invasiveness and pre-existing chronic kidney disease. Therefore, cardiac magnetic resonance was performed, confirming severe triple-vessel CAD. This case demonstrates a presentation of heart failure with unilateral chest radiograph changes in a young patient with Prader-Willi syndrome and severe premature CAD detected by multiparametric cardiac magnetic resonance imaging.

Edaravone attenuates lipopolysaccharide-induced acute respiratory distress syndrome associated early pulmonary fibrosis via amelioration of oxidative stress and transforming growth factor-β1/Smad3 signaling.

Science.gov (United States)

Wang, Xida; Lai, Rongde; Su, Xiangfen; Chen, Guibin; Liang, Zijing

2018-01-01

Pulmonary fibrosis is responsible for the both short-term and long-term outcomes in patients with acute respiratory distress syndrome (ARDS). There is still no effective cure to improve prognosis. The purpose of this study was to investigate whether edaravone, a free radical scavenger, have anti-fibrosis effects in the rat model of ARDS associated early pulmonary fibrosis by lipopolysaccharide (LPS) administration. Rats were subjected to intravenous injection of LPS, and edaravone was given intraperitoneally after LPS administration daily for 7 consecutive days. LPS treatment rapidly increased lung histopathology abnormalities, coefficient of lung, hydroxyproline and collagen I levels, stimulated myofibroblast differentiation and induced expression of TGF-β1 and activation of TGF-β1/Smad3 signaling as early as day 7 after LPS injection. Moreover, LPS intoxication significantly increased the contents of malondialdehyde (MDA), interleukin-6 (IL-6) and tumor necrosis factor-α (TNF-α), whereas it dramatically decreased superoxide dismutase (SOD) and glutathione peroxidase (GSH-PX) activities from day 1 after LPS treatment. On the contrary, edaravone treatment ameliorated LPS-induced myofibroblast differentiation and pulmonary fibrosis, simultaneously, and attenuated LPS-stimulated oxidative stress and activation of TGF-β1/Smad3 signaling. Collectively, edaravone may attenuate ARDS associated early pulmonary fibrosis through amelioration of oxidative stress and TGF-β1/Smad3 signaling pathway. Edaravone may be a promising drug candidate for the treatment of ARDS-related pulmonary fibrosis in early period. Copyright © 2017 Elsevier Inc. All rights reserved.

Central venous recanalization in patients with short gut syndrome: restoration of candidacy for intestinal and multivisceral transplantation.

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Lang, Elvira V; Reyes, Jorge; Faintuch, Salomao; Smith, Amy; Abu-Elmagd, Kareem

2005-09-01

To assess feasibility and success of venous recanalization in patients with short gut syndrome who have lost their traditional central venous access and required intestinal or multivisceral transplantation. Twelve patients between the ages of 7 and 55 years with short gut syndrome and long-standing total parenteral nutrition (TPN) dependency and/or hypercoagulability were treated. All had extensive chronic central venous occlusions and survival was dependent on restoration of access and planned transplantation. Central venous recanalizations were obtained via sharp needle recanalization techniques, venous reconstructions with stents, and/or extraanatomic access to the central venous system for placement of central venous tunneled catheters. Central venous access was restored in all patients without operative-related mortality. Three major hemodynamic perioperative technical complications were recorded and successfully treated. There were three self-limited early infectious complications. With a mean follow-up of 22 months, eight of the 12 patients were alive with successful small bowel or multivisceral transplantation; six of those became independent of TPN. The remaining four patients died of complications related to TPN (n = 3) or transplantation (n = 1). With a mean follow-up of 20 months, all but two of the recanalized venous accesses were maintained, for a success rate of 83%. Recanalizations of extensive chronic vein occlusions are feasible but associated with high risk. The technique is life-saving for TPN-dependent patients and can restore candidacy for intestinal and multivisceral transplantation. This approach is likely to be increasingly requested because of the current clinical availability of the transplant procedure.

[Choriocarcinoma causing a pulmonary embolus.

DEFF Research Database (Denmark)

Theliade, J.E.; Skovby, A.M.; Kirk, V.

2008-01-01

A 32 year-old women experienced dyspnea and thoracic pain that persisted with variable intensity over a course of eight months until acute worsening necessitated admission. A CT scan demonstrated a central pulmonary embolus. Subsequent surgical embolectomy produced a grained substance that was hi...

Restenosis after balloon valvuloplasty in a dog with pulmonary stenosis.

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Sunahara, Hiroshi; Fujii, Yoko; Sugimoto, Keisuke; Aoki, Takuma; Sugahara, Gou; Shirota, Kinji

2015-01-01

A two-month-old female Chihuahua was diagnosed as severe pulmonary valvular stenosis (PS). Although balloon valvuloplasty (BV) was successfully performed, restenosis was observed 19 months after the procedure. Euthanasia was chosen due to low output syndrome during the surgical repair attempted when the dog was 5 years old. Postmortem examination revealed markedly thickened pulmonary valve due to the increase of extracellular matrix which might be produced by increased α smooth muscle actin-positive myofibroblasts. The thickening of the valve was associated with restriction of the valve's motion, resulting in restenosis in the present case. This is the first case report documented histopathological and immunohistochemical findings of the restenotic pulmonary valve in dogs with PS after BV.

Pinch-off syndrome: case report and review of the literature = Síndrome de pinzamiento costoclavicular: reporte de un caso y revisión de la literatura

Directory of Open Access Journals (Sweden)

Vega Peña, Neil Valentín

2013-04-01

Full Text Available Pinch-off syndrome is a rare complication of central venous catheter insertion through the subclavian approach. It is secondary to external compression of the catheter in the costoclavicular angle, which can lead to obstruction, rupture and embolization of its distal fragment. It is usually associated with premonitory radiological and clinical signs. Due to the severe complications of this syndrome, a proper prophylaxis should be done from the moment of insertion until the end of treatment. We present the case of a patient undergoing chemotherapy for adenocarcinoma of the colon, who developed pinch-off syndrome, with rupture and embolization of the distal fragment of the catheter to the right pulmonary artery. Treatment was successful by means of catheterization through the femoral vein.

Congenital heart defects in Williams syndrome.

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Yuan, Shi-Min

2017-01-01

Yuan SM. Congenital heart defects in Williams syndrome. Turk J Pediatr 2017; 59: 225-232. Williams syndrome (WS), also known as Williams-Beuren syndrome, is a rare genetic disorder involving multiple systems including the circulatory system. However, the etiologies of the associated congenital heart defects in WS patients have not been sufficiently elucidated and represent therapeutic challenges. The typical congenital heart defects in WS were supravalvar aortic stenosis, pulmonary stenosis (both valvular and peripheral), aortic coarctation and mitral valvar prolapse. The atypical cardiovascular anomalies include tetralogy of Fallot, atrial septal defects, aortic and mitral valvular insufficiencies, bicuspid aortic valves, ventricular septal defects, total anomalous pulmonary venous return, double chambered right ventricle, Ebstein anomaly and arterial anomalies. Deletion of the elastin gene on chromosome 7q11.23 leads to deficiency or abnormal deposition of elastin during cardiovascular development, thereby leading to widespread cardiovascular abnormalities in WS. In this article, the distribution, treatment and surgical outcomes of typical and atypical cardiac defects in WS are discussed.

Cushing's Syndrome From Pituitary Microadenoma and Pulmonary Nodules.

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Tating, Dan Louie Renz P; Montevirgen, Natasha Denise S; Cajucom, Loyda

2016-03-01

Cushing's syndrome is a state of cortisol excess, possibly from a tumor in the pituitary gland, the adrenal gland, or an ectopic nonpituitary ACTH-secreting source. The first form, pituitary in origin, was originally described by Harvey Cushing, MD, and was labeled as Cushing's disease. Long-term therapy with glucocorticoids also can lead to iatrogenic Cushing's syndrome.

Corticoide sistêmico como tratamento de primeira linha da hipertensão pulmonar secundária a síndrome POEMS Systemic corticosteroids as first-line treatment in pulmonary hypertension associated with POEMS syndrome

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Samia Rached

2009-08-01

Full Text Available A síndrome POEMS é uma rara doença de plasmócitos. A ocorrência de hipertensão pulmonar como complicação respiratória da síndrome é pouco frequente e pode estar ligada ao aumento de várias citocinas, quimiocinas e fatores de crescimento como parte dos fenômenos inflamatórios que cercam a fisiopatologia da síndrome POEMS. Descrevemos o caso de uma mulher de 54 anos com síndrome POEMS e hipertensão pulmonar, que foi tratada com corticoide como terapia de primeira linha. Tratava-se de uma paciente com clássicos sintomas dessa síndrome: polineuropatia (confirmada por eletroneuromiografia, organomegalia, hipotireoidismo subclínico, gamopatia monoclonal em dosagem urinária e alterações cutâneas. A cateterização cardíaca direita revelou pressão arterial pulmonar média de 48 mmHg, débito cardíaco de 4,1 L/min e resistência vascular pulmonar de 8,05 Woods. O nível sérico de brain natriuretic peptide (BNP foi de 150 pg/mL. Nenhuma outra doença foi encontrada durante investigação. Prednisona (1 mg/kg por três meses foi iniciada, com dramática melhora clínica e funcional, além de normalização dos níveis dos hormônios tireoidianos e de proteína em urina por eletroforese. A pressão arterial pulmonar média caiu para 26 mmHg, o débito cardíaco para 3,8 L/min e a resistência vascular pulmonar para 2,89 Woods. O nível sérico de BNP caiu para 8pg/mL. Nossos achados indicam o potencial papel da corticoterapia como primeira linha de tratamento na hipertensão pulmonar associada à síndrome POEMS. Diante da raridade dessa apresentação, um registro multicêntrico deveria ser desenvolvido para permitir a aquisição de mais dados que suportem essa conduta.The POEMS syndrome is a rare plasma cell disease. Pulmonary hypertension is an infrequent respiratory complication of this syndrome and might be associated with increased levels of various cytokines, chemokines and growth factors as part of the inflammatory

Mounier-Kuhn syndrome: a rare cause of bronchial dilation.

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Celik, Burcin; Bilgin, Salih; Yuksel, Canan

2011-01-01

Mounier-Kuhn syndrome, or tracheobronchomegaly, is a rare clinical and radiologic condition characterized by marked tracheobronchial dilation and recurrent lower respiratory tract infections. Diagnosis is typically accomplished with the use of computed tomography and bronchoscopy, as well as pulmonary function testing. Patients may be asymptomatic; however, symptoms can range from minimal with preserved lung function to severe respiratory failure. Therapy, if any, is supportive but minimal. Surgery rarely has a place in the treatment of Mounier-Kuhn syndrome.Herein, we report the case of a 58-year-old man with chronic obstructive pulmonary disease who had a chronic cough, increased sputum production, and chest pain. Thoracic computed tomography showed tracheal dilation (diameter, 34 mm) and multiple diverticula in the posterior region of the trachea. Fiberoptic bronchoscopy revealed enlarged main bronchi, the dilated trachea, and prominent tracheal diverticula. Pulmonary function testing disclosed impaired respiratory function. Histopathologic examination of biopsy specimens from the bronchi and the tracheal wall supported the diagnosis of Mounier-Kuhn syndrome. The patient was released from the hospital and his condition was monitored for 2 years, during which time he developed no lower respiratory tract infections.Regardless of radiologic findings that suggest recurrent lower respiratory tract infection, we recommend that Mounier-Kuhn syndrome be considered in the differential diagnosis.

Pulmonary complications of AIDS: radiologic features

International Nuclear Information System (INIS)

Cohen, B.A.; Pomeranz, S.; Rabinowitz, J.G.; Rosen, M.J.; Train, J.S.; Norton, K.I.; Mendelson, D.S.

1984-01-01

Fifty-two patients with pulmonary complications of acquired immunodeficiency syndrome (AIDS) were studied over a 3-year period. The vast majority of the patients were homosexual; however, a significant number were intravenous drug abusers. Thirteen different organisms were noted, of which Pneumocystis carinii was by far the most common. Five patients had neoplasia. Most patients had initial abnormal chest films; however, eight patients subsequently shown to have Pneumocystis carinii pneumonia had normal chest films. A significant overlap in chest radiographic findings was noted among patients with different or multiple organisms. Lung biopsy should be an early consideration for all patients with a clinical history consistent with the pulmonary complications of AIDS. Of the 52 patients, 41 had died by the time this report was completed

Chronic obstructive pulmonary disease

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Karabulut, N.

2012-01-01

Full text: Chronic obstructive pulmonary diseases (COPD) denote progressive lung diseases characterized by airway obstruction. COPD exhibits specific morphologic changes in the lung parenchyma, central and peripheral airways and pulmonary vasculature. A person with COPD may have either emphysema or chronic bronchitis, but most have both. Some people with COPD may also have an asthma-like or reactive component. Imaging modalities play important role in the detection or exclusion of COPD, distribution and extent of disease processes. Combined inspiratory and expiratory high resolution CT allows phenotyping of COPD (emphysema predominant, airway predominant, or mixed) and quantification of severity. Magnetic resonance imaging enables functional evaluation and demonstrates ventilation defects correlating closely with pulmonary function tests. Imaging techniques are also helpful in guiding the treatment, such as bullectomy in patients with bullous emphysema, lung volume reduction surgery or endoscopic interventions in those with severe emphysema, and smoking cessation and medical treatment designed to stop lung destruction in patients with mild or moderate emphysema or bronchiectasis.

Predictors of asthma-chronic obstructive pulmonary disease overlap syndrome in patients with chronic obstructive pulmonary disease from a tertiary care center in India

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Irfan Ismail Ayub

2018-01-01

Full Text Available Background: There is dearth of literature on asthma-chronic obstructive pulmonary disease overlap syndrome (ACOS in India. The aim was to compare clinical characteristics between patients with ACOS and non-ACOS COPD and to identify clinical predictors of ACOS in patients with COPD. Methods: We conducted a retrospective study by reviewing data collected from patients performing spirometry at our hospital. Those with postbronchodilator FEV1/FVC <70% were included in the study. Among them, those with significant reversibility (change in FEV1or FVC by 12% and 200 ml postbronchodilator were diagnosed with ACOS and the rest were considered to have non-ACOS COPD. Data on the 2 groups were compared and statistical analysis was performed. Results: Out of a total of 324 patients, 100 of them had postbronchodilator FEV1/FVC <70%. Of them, 45 and 55 were diagnosed with ACOS and non-ACOS COPD, respectively. Patients with ACOS had significantly higher postbronchodilator FVC volumes and FVC % predicted values (P < 0.05, had higher reported wheeze (P = 0.02 and ankle edema (P < 0.05, were more likely to be smokers (P = 0.01 with lower smoking index (P = 0.03, and had frequent (≥2 ER visits (P = 0.04. However, very frequent (≥3 per year hospital admissions (P < 0.01 with higher rates of invasive mechanical ventilation (P = 0.02, and pulmonary hypertension diagnosed by two-dimensional echocardiography (P < 0.01 were significantly higher in the non-ACOS group. The two groups did not differ with respect to history of atopy, family history of wheeze, compliance to inhaler therapy, or blood absolute eosinophil counts. Conclusion: Our study highlights how the ACOS phenotype may clinically differ from their counterparts elsewhere, making it a clinical challenge to identify them in India.

Differentiation between Birt–Hogg–Dubé syndrome and lymphangioleiomyomatosis: Quantitative analysis of pulmonary cysts on computed tomography of the chest in 66 females

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Tobino, Kazunori; Hirai, Toyohiro; Johkoh, Takeshi; Kurihara, Masatoshi; Fujimoto, Kiminori; Tomiyama, Noriyuki; Mishima, Michiaki; Takahashi, Kazuhisa; Seyama, Kuniaki

2012-01-01

Backgrounds: Since Birt–Hogg–Dubé syndrome (BHDS) and lymphangioleiomyomatosis (LAM) share some clinical manifestations (multiple pulmonary cysts with pneumothorax, renal tumors, and sometimes skin lesions), the differential diagnosis of the two diseases becomes problem especially in female patients. This study aims to quantify pulmonary cysts in computed tomography (CT) of females with BHDS and those with LAM and also to identify the independent parameters for differentiating the two diseases. Methods: Fourteen patients with BHDS and 52 with LAM were studied. In CT scans, lung fields were defined as areas with fewer than −200 Hounsfield units (HU) and pulmonary cysts as areas consisting of 10 or more consecutive pixels with fewer than −960 HU. The extent, number, size and circularity of cysts were calculated by using hand-made software and compared between the two diseases. Moreover, the lung fields were divided into six zones and analyzed for the distribution of cysts. Finally, a stepwise discriminant analysis employing quantitative measurements of cysts and clinical features was performed. Results: The two diseases were significantly different in all quantitative measurements of cysts. Stepwise discriminant analysis accepted the following four variables: the family history of pneumothorax within the second degree relatives, lower-medial zone predominance of cysts, diffusing capacity and mean size of cysts in this order. Conclusion: The quantitative characteristics of pulmonary cysts are significantly different between BHDS and LAM. The independent parameters for differentiating the two diseases are the family history of pneumothorax, zonal predominance of cysts, diffusing capacity and size of cysts.

Successful treatment of Rhodococcus equi pulmonary infection in a renal transplant recipient.

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Marsh, H. P.; Bowler, I. C.; Watson, C. J.

2000-01-01

The rhodococcus is a mycobacterium-like organism which is normally a pathogen in foals. It usually spreads by direct contact or by aerosol from horse faeces and causes pyogranulomatous pulmonary infections. Occasionally, it acts opportunistically to infect immuno-compromised human hosts, most commonly those with the acquired immune deficiency syndrome (AIDS). Here we report a pulmonary infection by Rhodococcus equi in a renal transplant recipient who was successfully treated. The literature o...

Swyer-James-Macleod's syndrome. A case report

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Pacheco C, Dario; Ojeda Leon, Paulina; Varo Acosta, Humberto; Salcedo Veles, Patricia; Salazar Juan Carlos

1998-01-01

This is a case report about a 67 years-old female patient with respiratory syndrome of 8 years with cough and dyspnoea. End-inspiration crackles in the pulmonary auscultation were found in left hemi thorax. Chest x-ray in expiration and inspiration showed hyperluscency and air trapping in the same hemi-thorax. Chest high-resolution CT revealed a low sized, oligohemic left lung with cylindrical bronchiectasis. Perfusion scintigraphy 99Tc labeled showed markedly left lung hypo-perfusion. Mild obstructive process was found in pulmonary function test. Lung biopsy of lingula reported bronchiolitis obliterans. Considered all the results that were obtained from clinical, x-ray and histopathology, a diagnosis of Swyer-James-Macleods syndrome was made

Evaluation of the Endothelial Cell Density and the Central Corneal Thickness in Pseudoexfoliation Syndrome and Pseudoexfoliation Glaucoma

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Bożydar T. Tomaszewski

2014-01-01

Full Text Available Purpose. Evaluation of central corneal thickness (CCT and endothelial cell density (ECD in patients with senile cataract and coexisting pseudoexfoliation (PEX syndrome with glaucoma (PEXG and without glaucoma using specular microscopy. Participants and Methods. The study included 122 patients (217 eyes. In this group of patients we identified 133 eyes with PEX syndrome (65 with glaucoma, 68 without glaucoma and 84 eyes without PEX syndrome. ECD and CCT were measured in each eye by specular microscopy. Results. ECD in eyes with PEX syndrome without glaucoma (2297 ± 359 cell/mm2 and in eyes with PEXG (2241 ± 363 cell/mm2 was lower than in the control group (2503 ± 262 cell/mm2 (P<0.001. CCT in eyes with PEXG (508.2 ± 32.6 μm was thinner than in eyes with PEX syndrome without glaucoma (529.7 ± 30.3 μm and control group (527.7 ± 29.4 μm (P<0.001. Conclusions. This research shows that in eyes with PEX syndrome, both with and without glaucoma, ECD was statistically significantly lower than in the control group. In patients with PEXG, CCT was statistically significantly thinner than in the PEX syndrome and control group.

CT findings of pulmonary hypertension

International Nuclear Information System (INIS)

Inoue, Yukio; Tanimoto, Akihiro; Sato, Toru; Kuribayashi, Sachio

2006-01-01

For the treatment for pulmonary hypertension (PH), the differential diagnosis of its causal diseases is essential. To determine whether X-ray CT is useful for differentiating PH, we reviewed CT findings of 53 patients (18 men and 35 women, mean age of 44.9) given a diagnosis of PH, consisting of 25 with primary pulmonary hypertension (PPH), 18 with chronic pulmonary embolism (cPE), 6 with Eisenmenger syndrome, 5 cases of collagen diseases, 2 of acute PE, and 1 of cor pulmonale. The intrapulmonary distribution of CT findings (ground glass opacity [GGO], mosaic attenuation, striation and/or infiltration, and interlobular septal thickening) were reviewed and scored on a 4-point scale (grade 0: no findings, 1: involving one third of the lung, 2: involving one-two thirds, and 3: diffuse distribution) by two radiologists who reached a consensus. PPH showed preferentially diffuse distribution of GGO as compared with cPE (p<0.05). However, there was no apparent relationship between the pulmonary vascular resistance and the distribution of GGO in PPH cases. The mosaic attenuation pattern was more frequent in cPE (43%) than PPH (12%; p<0.05). Striation and/or infiltration was observed in 36% of cPE, but only 4% of PPH. Interlobular septal thickening was seen in 16% of PPH, and 0% in cPE. Evaluation of CT findings is useful to differentiate PH. (author)

Isolated pulmonary vasculitis: case report and literature review.

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Riancho-Zarrabeitia, Leyre; Zurbano, Felipe; Gómez-Román, Javier; Martínez-Meñaca, Amaya; López, Marta; Hernández, Miguel A; Pina, Trinitario; González-Gay, Miguel A

2015-04-01

Single-organ vasculitis has been reported to affect the skin, kidneys, central nervous system, peripheral nerves, genitourinary tract, calf muscles, aorta, coronary arteries, retina, or gastrointestinal tract. However, isolated pulmonary vasculitis is a very rare entity. Our aims were to describe a case of localized pulmonary vasculitis affecting medium-sized vessels and review the literature. A patient with localized pulmonary vasculitis affecting medium-sized vessels that presented as pulmonary arterial hypertension is described. A MEDLINE database search of cases with localized pulmonary vasculitis was also conducted. A 30-year-old man presented with pulmonary hypertension due to isolated pulmonary medium-sized vessel vasculitis that was confirmed histologically. Initially he responded to corticosteroids and vasodilator treatment, but therapy eventually lost efficacy. Treatment with rituximab was not effective, and as the clinical situation worsened, lung transplant was performed. Isolated large pulmonary vessel disease, often related to Takayasu disease or giant cell arteritis, may present as pulmonary artery hypertension, thus mimicking chronic thromboembolic disease. Medium- and small-vessel pulmonary vasculitis usually develops in the context of a systemic disease. Some cases of isolated small-vessel vasculitis have been reported presenting as diffuse alveolar hemorrhage. In contrast, our case developed pulmonary artery hypertension secondary to medium-sized vessels vasculitis. To our knowledge, this is the first case of lung transplantation in isolated pulmonary vasculitis. Pulmonary isolated vasculitis is a rare cause of pulmonary hypertension but it must be taken into consideration after more common disorders are excluded. Copyright © 2014 Elsevier Inc. All rights reserved.

The Rare Painful Phenomena - Chronic Paroxysmal Hemicrania-tic Syndrome as a Clinically Isolated Syndrome of the Central Nervous System.

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Ljubisavljevic, Srdjan; Prazic, Ana; Lazarevic, Miodrag; Stojanov, Dragan; Savic, Dejan; Vojinovic, Slobadan

2017-02-01

The association of paroxysmal hemicrania with trigeminal neuralgia (TN) has been described and called paroxysmal hemicrania-tic syndrome (PH-tic). We report the case of a patient diagnosed as having chronic PH-tic (CPH-tic) syndrome as a clinically isolated syndrome of the central nervous system (CNS) (CIS).A forty year old woman was admitted to our hospital suffering from right facial pain for the last 2 years. The attacks were paroxysmal, neuralgiform, consisting of throb-like sensations, which developed spontaneously or were triggered by different stimuli in right facial (maxilar and mandibular) areas. Parallel with those, she felt a throbbing orbital and frontal pain with homolateral autonomic symptoms such as conjunctival injection, lacrimation, and the feeling that the ear on the same side was full. This pain lasted most often between 15 and 20 minutes. Beyond hemifacial hypoesthesia in the region of right maxilar and mandibular nerve, the other neurological finding was normal. Magnetic resonance imaging (MRI) study showed a T2-weighted multiple hyperintense paraventricular lesion and hyperintense lesion in the right trigeminal main sensory nucleus and root inlet, all of them being hypointense on T1-weighted image. All of these lesions were hypointense in gadolinium-enhanced T1-weighted images. Neurophysiological studies of trigeminal nerve (somatosensory evoked potentials and blink reflex) correlated with MRI described lesions. The patient's pain bouts were improved immediately after treatment with indomethacin, and were completely relieved with lamotrigine for a longer period. According to the actual McDonald's criteria, clinical state was defined as CIS which was clinically presented by CPH-tic syndrome.Even though it is a clinical rarity and its etiology is usually idiopathic, CPH-tic syndrome can also be symptomatic. When dealing with symptomatic cases, like the one described here, when causal therapy is not possible due to the nature of the primary

Benefits of Balloon-Dilatable Bilateral Pulmonary Artery Banding in Patients With Hypoplastic Left Heart Syndrome and Other Complex Cardiac Anomalies.

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Kise, Hiroaki; Suzuki, Shoji; Hoshiai, Minako; Toda, Takako; Koizumi, Keiichi; Hasebe, Yohei; Kono, Yosuke; Honda, Yoshihiro; Kaga, Shigeaki; Sugita, Kanji

2015-12-01

The purpose of this study was to evaluate the potential of balloon-dilatable bilateral pulmonary artery banding (b-PAB) and its impact on the configuration of the pulmonary artery (PA). We have previously used balloon-dilatable b-PAB as first-stage palliation for patients with hypoplastic left heart syndrome (HLHS) and other complex cardiac anomalies. Two pliable tapes were placed around each branch of the PA and tightened with 7-0 polypropylene sutures in a manner that allowed for the subsequent adjustment of PA diameters. We retrospectively examined the adjustability of PA diameters by balloon dilation and the need for surgical PA angioplasty at later stages. From January 2010 to October 2013, we performed b-PAB in 8 patients, including 3 borderline cases between biventricular repair (BVR) and univentricular repair (UVR). The b-PAB procedures were performed at a median age of 6.5 days (range, 2-10 days). Balloon dilations were performed in 10 lesions in 4 patients. All of the procedures were performed safely. Two patients reached definite BVR. The remaining 6 patients underwent open palliative procedures with univentricular physiologies that resulted in 2 deaths unrelated to the initial b-PAB. In all but 1 of the patients, the PA configuration was properly maintained and did not require surgical pulmonary angioplasty. Balloon-dilatable b-PAB can be performed safely and prevents PA distortion at later stages. This technique should be considered for patients with complex cardiac anomalies if uncertainty exists regarding the optimal surgical strategy (BVR or UVR) in early infancy. © 2015, Wiley Periodicals, Inc.

[Treatment of bronchial obstruction in patients with pulmonary tuberculosis].

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Shmelev, E I; Kuklina, G M; Kalinina, E E

2004-01-01

Whether the main points of treatment for bronchial obstructive syndrome (BOS) in chronic obstructive lung disease (COLD) can be adapted for patients with pulmonary tuberculosis (PT) was studied. For this purpose, 435 patients with PT with signs of BOS (forced expiratory volume at 1 second (FEV1) 70% of the normal values; 2) 229 patients with FEV1 69-50%; 3) 102 patients with FEV1 bronchial obstructive syndrome in patients with pulmonary tuberculosis was highly effective, promotes the amelioration of the degree of respiratory symptoms in patients with IPT by 2 to 8 times, in those with FCPT by more than 2-3 times, and in those with PS by 1.45-10 times. The differences in the efficiency of bronchodilator therapy depend on the baseline level of bronchial obstruction. In patients with pulmonary tuberculosis concurrent with BO, the use of current inhalation bronchodilator therapy results in a substantial increase in FEV1, which differentiates BOS in PT from COLD. The use of the proposed therapy in the multimodality treatment of patients with pulmonary tuberculosis showed no statistically significant differences in the changes in the degree of X-ray symptoms while this therapy permits acceleration of abacillation in patients with IPT by 16.8% and in those with FCPT by 14.8%. Effective bronchodilator therapy considerably enhances life quality in patients. Thus, early systematic and long-term performance of the bronchodilator therapy, based on the principles of bronchodilator therapy for COLD, in patients with PT concurrent with BOS may substantially enhance the efficiency of treatment in this category of patients.

Endothelial nanomedicine for the treatment of pulmonary disease.

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Brenner, Jacob S; Greineder, Colin; Shuvaev, Vladimir; Muzykantov, Vladimir

2015-02-01

Even though pulmonary diseases are among the leading causes of morbidity and mortality in the world, exceedingly few life-prolonging therapies have been developed for these maladies. Relief may finally come from nanomedicine and targeted drug delivery. Here, we focus on four conditions for which the pulmonary endothelium plays a pivotal role: acute respiratory distress syndrome, primary graft dysfunction occurring immediately after lung transplantation, pulmonary arterial hypertension and pulmonary embolism. For each of these diseases, we first evaluate the targeted drug delivery approaches that have been tested in animals. Then we suggest a 'need specification' for each disease: a list of criteria (e.g., macroscale delivery method, stability, etc.) that nanomedicine agents must meet in order to warrant human clinical trials and investment from industry. For the diseases profiled here, numerous nanomedicine agents have shown promise in animal models. However, to maximize the chances of creating products that reach patients, nanomedicine engineers and clinicians must work together and use each disease's need specification to guide the design of practical and effective nanomedicine agents.

[Central cervical cord syndrome: a case report on rehabilitation, with special references to accidental falls in the elderly].

Science.gov (United States)

Ina, G; Eto, F; Furuichi, T; Suzuki, H; Shibuya, K

1995-03-01

An 81-year-old man with Parkinson's disease was admitted to our hospital with impaired function of all extremities. Four weeks before his symptoms developed, he had tripped on the steps, fallen and bruised his jaw. Following this episode he experienced a few more falls inside his house. On examination his greatest weakness was in the hands and wrists. He was hyper-reflexic in all extremities and had bilateral Babinski's sign. He could not walk and needed physical assistance in most of his daily living activities. X-ray films of the cervical spine showed significant degenerative changes. The magnetic resonance images suggested central cervical cord damage at the level of the C6 vertebral body. After three months' rehabilitation treatment, he became able to walk with a cane and became independent in all the basic activities of daily living except for bathing. He never regained skillful function of his hands despite later levodopa treatment of Parkinson's disease. His clinical features were consistent with the central cervical cord syndrome, described by Schnneider and co-workers in 1954. This syndrome may occur as a result of hyperextension neck injury, occasionally associated with an accidental fall in the elderly with cervical spondylosis. Thirteen patients with cervical spinal cord injury above 65 of age were admitted to our department from 1983 to 1993. Six of them presented with the central cervical cord syndrome, and all patients had a history of accidental injuries related to falling.

Bland-White-Garland syndrome - a rare and serious cause of failure to thrive.

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Szmigielska, Agnieszka; Roszkowska-Blaim, Maria; Gołąbek-Dylewska, Małgorzata; Tomik, Agnieszka; Brzewski, Michał; Werner, Bożena

2013-01-01

Male, 0 FINAL DIAGNOSIS: Bland-White-Garland syndrome Symptoms: Cardiomegaly, feeding problems Medication: - Clinical Procedure: Reimplantation of the left coronary artery to the aorta Specialty: Pediatrics and Neonatology. Rare disease. Bland-White-Garland syndrome (BWGS) is a very rare disease characterized by anomalous origin of the left coronary artery from the pulmonary trunk (ALCAPA). WBGS affects 1 in every 300 000 live births. Children typically present with dyspnea, pallor, and failure to thrive. Without surgical repair, most of these children die during the first months of life. This case report describes 3-month-old boy admitted to the hospital because of feeding problems. The boy was born at term, with birth weight 3200 g, and was 10 points in Apgar score. He was breast-fed from birth. From the seventh week of age, his mother observed his increasing difficulties with feeding. Physical examination revealed pale skin, diminished heart sounds, tachycardia, cardiomegaly, and hepatomegaly. Results of urine and blood tests and ultrasonography of the central nervous system and abdomen were normal. The chest radiography showed cardiomegaly and electrocardiogram revealed anterolateral myocardial infarction. On echocardiography, an anomalous left coronary artery arising from the pulmonary artery was found. The life-saving treatment of choice was immediate surgical reimplantation of the left coronary artery to the aorta. Children with congenital heart disease are often prone to malnutrition, but in rare cases failure to thrive and breast-feeding problems can be the first symptoms of life-threatening diseases like myocardial infarction secondary to Bland-White-Garland syndrome (BWGS).

A novel immunodeficiency syndrome as a rare cause of secondary pulmonary alveolar proteinosis: A diagnosis after 5 decades

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Pedro G. Ferreira

2014-09-01

Full Text Available Case report of a male patient with a five-decade follow-up history in a tertiary care hospital distinguished for malabsorption syndrome, failure-to-thrive, meningitis and recurrent bacterial, fungal and mycobacterial pulmonary infections. Additionally, he developed epidermodysplasia verruciformis, several in situ spinocellular carcinomas and an uncharacteristic parenchymal lung disease. Surgical lung biopsy suggested pulmonary alveolar proteinosis with fibrotic change. Retrospectively, severe monocytopenia had been overlooked in the past, as well as low B and NK cell blood counts. Flow cytometry confirmed the absence of the previous cell subsets along with an undetectable population of dendritic blood cells.Dendritic cell, monocyte, B and NK lymphoid Human Deficiency Syndrome (DCMLS is a novel rare immunodeficiency described in 2010, linked to GATA-2 mutation. This syndrome should be highlighted as a rare cause of acquired PAP, with a radiological pattern encompassing potential fibrotic change. Failure to recognize monocytopenia may impede the chance to diagnose. Resumo: Relato de um caso clínico de um doente do sexo masculino com um historial de acompanhamento de cinco décadas, num hospital de cuidados terciários, caracterizado por um síndrome de malabsorção, insuficiência de crescimento, meningite e infecções pulmonares bacterianas, fúngicas e micobacterianas recorrentes. Além disso, desenvolveu epidermodisplasia verruciforme, diversos carcinomas espinocelulares in situ e uma doença pulmonar parenquimatosa não definida. Uma biópsia pulmonar cirúrgica sugeriu uma Proteinose Alveolar Pulmonar com alterações fibróticas. Em retrospectiva, uma monocitopenia grave negligenciada no passado, bem como uma baixa contagem de células B (linfócitos B e NK (células ‘natural killer’. Uma citometria de fluxo confirmou a ausência dos subconjuntos de células anteriores, juntamente

CT findings of pulmonary edema: comparison of various causes

International Nuclear Information System (INIS)

Kim, Hyae Young; Im, Jung Gi; Goo, Jin Mo; Lee, Jae Kyo; Song, Jae Woo

1999-01-01

To access the CT findings of pulmonary edema and to compare them, according to the cause. CT findings (thin section, 20 ; thick section, 16) of pulmonary edema in 36 patients [cardiac disease (group 1, n=20), renal disease (group 2, n=13), ARDS (group 3, n=3)] were analyzed and compared. There were 21 men and 15 women ranging in age from 27 to 77 years. Distribution (even, central, or peripheral) and patterns of pulmonary edema were compared between the three groups. The distribution of edema, appearing as consolidation or ground-glass opacity, was even in 75% (n=15) of group 1, even in 46% (n=6) and central in 38% (n=5) of group 2, and peripherally predominant in 100% (n=3) of group 3. Interlobular septal thickening was seen in 80% (n=16), 69% (n=9), and 0% of group 1, 2 and 3, respectively. Centrilobular ground-glass opacity was noted in six patients. In spite of various findings and considerable overlapping of the findings of pulmonary edema, the distribution and pattern of edema differed according to the cause, and this can be helpful for differential diagnosis

Negative pressure pulmonary edema revisited: Pathophysiology and review of management

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Balu Bhaskar

2011-01-01

Full Text Available Negative pressure pulmonary edema (NPPE is a dangerous and potentially fatal condition with a multifactorial pathogenesis. Frequently, NPPE is a manifestation of upper airway obstruction, the large negative intrathoracic pressure generated by forced inspiration against an obstructed airway is thought to be the principal mechanism involved. This negative pressure leads to an increase in pulmonary vascular volume and pulmonary capillary transmural pressure, creating a risk of disruption of the alveolar-capillary membrane. The early detection of the signs of this syndrome is vital to the treatment and to patient outcome. The purpose of this review is to highlight the available literature on NPPE, while probing the pathophysiological mechanisms relevant in both the development of this condition and that involved in its resolution.

Comparable Enhanced Prothrombogenesis in Simple Central Obesity and Metabolic Syndrome

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Noor Shafina Mohd Nor

2018-01-01

Full Text Available Objective. There is limited data comparing prothrombogenic or fibrinolysis biomarkers (tissue plasminogen activator (tPA and plasminogen activator inhibitor-1 (PAI-1 simultaneously in subjects with Metabolic Syndrome (MS, simple central obesity without MS (COB and normal controls (NC. We investigated the concentrations of fibrinolysis biomarkers in subjects with MS, COB and NC. Methods. A cross-sectional study involving 503 drug naive subjects (163 males, aged 30–65 years old (mean age ± SD = 47.4 ± 8.3 years divided into MS, COB and NC groups. COB was defined as central obesity (waist circumference (WC males ≥90 cm, females ≥80 cm in the absence of MS according to the International Diabetes Federation 2006. Fasting blood levels of tPA and PAI-1were analyzed. Results. MS and COB had significantly higher concentration of all biomarkers compared to NC. The MS group had significantly higher concentration of tPA and PAI-1 compared to COB. WC and HDL-c had significant correlation with all biomarkers (tPA p<0.001, PAI-1 p<0.001. Fasting plasma glucose and diastolic blood pressure were independent predictors after correcting for confounding factors. Conclusion. Central obesity with or without MS both demonstrated enhanced prothrombogenesis. This suggests that simple obesity possibly increases the risk of coronary artery disease in part, via increased susceptibility to thrombogenesis.

Pulmonary diseases of the infants weighing under 1500 grams at birth: clinical and radiographic findings

International Nuclear Information System (INIS)

Kim, Ok Hwa; Park, Jeong Mi; Bahk, Yong Whee

1990-01-01

Since the introduction of the intensive perinatal care, the survival rate of the infants weighing less than 1500 gm at birth has improved substantially. However, pulmonary diseases remain to be the major causes of the high mortality of these low birthweight infants. In order to systematically assess an epidemiologic distribution of the pulmonary diseases in these very low weight prematures, we have analyzed the chest x-rays of 102 infants weighing less than 1500 gm. These consisted of 30 with extreme low birth weight (ELBW) weighing less than 1000 gm and 72 with very low birth weight (VLBW) weighing 1001 - 1500 gm. The survival rate of ELBW and VLBW was 10% and 49%, respectively. Seventy of 102 infants had abnormal findings in the chest x-ray. Forty-eight had idiopathic respiratory distress syndrome (IRDS), 8 immature lung, 6 Wilson-Mikity syndrome, 4 pneumonia, 2 pulmonary hemorrhage, 1 congenital heart disease, and 1 suspicious Pierre-Robin syndrome. Seven out of 48 infants with IRDS had persistent ductus arteriosus, and in only 2(30%) of 7 cases were alive. Endotracheal intubation and assisted ventilation application for the treatment of IRDS resulted in pulmonary interstitial emphysema in 4 infants and pneumothorax and / or pneumomediastinum in 4 infants. Displacement of endotracheal intubation showed lobar and / or unilateral lung atelectasis in 8 infants and a case of accidental dislodgement of intubation tube into the esophagus resulted in air esophagogram and worsened lung aeration. In spite of the development of many sophisticated methods of diagnostic radiology, the chest x-ray was still the most valuable yet simple way of evaluating the pulmonary problems in these extreme and very low birth weight prematures

Pulmonary arteriovenous malformation in cryptogenic liver

International Nuclear Information System (INIS)

Afzal, N.

2013-01-01

The cause in 10 - 20% cases of liver cirrhosis (LC) cannot be elucidated, and are thus termed cryptogenic. Pulmonary arteriovenous malformations (PAVMs) are relatively rare, but the most common anomaly involving the pulmonary tree. Although the rare correlation between LC and PAVM is well-known, there have been no reports of PAVMs occurring in cryptogenic LC. We report a case of PAVM that occurred in cryptogenic liver cirrhosis in a 3 years old male child. The child presented with complaints of malena, hematemesis and variceal bleed. The examination revealed a child with respiratory distress, irritability, tachycardia, clubbing and abdominal distention. He was worked up for recurrent variceal bleeding secondary to portal hypertension but the oxygen saturation during hospital stay kept deteriorating. The diagnosis of hepatopulmonary syndrome as the cause of persistent hypoxemia in the absence of other cardio-pulmonary causes was then made by enhanced echocardiogram using agitated saline. He improved significantly after liver transplantation performed abroad. At a 6 months follow-up, the child was stable with no evidence of intrapulmonary shunting on repeat echo. (author)

Erasmus syndrome in a marble worker

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S. Bello

2016-02-01

Full Text Available Erasmus syndrome is defined as the association of silica exposure and subsequent development of systemic sclerosis. The limited number of cases reported in the literature mainly involves miners and only sporadically other professionals. We describe a case of Erasmus syndrome in a marble worker. A 68 year old man came to our observation complaining pelvic and scapular girdle pain, evening fever, intense weakness and emaciation for about 1 month. He also reported to have had Raynaud’s phenomenon in his hands for the last 13 years. Also, his occupational history revealed a chronic exposure to silica dust. The patient presented pain in his shoulders and hips, moderate skin thickening and sclerosis in his hands and fingers extending proximally to his wrists. The diagnosis of systemic sclerosis was determined according to his clinical and medical history, the positivity of anti-Scl 70 antibodies, the nailfold capillaroscopy suggestive of an active scleroderma pattern and the detection of a mild restrictive pulmonary syndrome. The evaluation of the organbased complications excluded a gastroenterological and cardiovascular involvement, while the chest computed tomography (CT detected multiple small nodules with a mantle distribution and enlarged lymph nodes with no signs of interstitial lung disease and fibrosis. Additional tests (positron emission tomography-CT, flexible bronchoscopy and broncho-alveolar lavage excluded infectious diseases and cancer. However, given the pulmonary involvement, we performed a histological examination of the parenchyma and lymph nodes, which revealed a picture of pneumoconiosis. In the end, the occupational history and the findings from the diagnostic procedures led to the diagnosis of pulmonary silicosis. The precise definition of the pulmonary involvement was essential to the therapeutic approach to this patient.

Erasmus syndrome in a marble worker.

Science.gov (United States)

Bello, S; Rinaldi, A; Trabucco, S; Serafino, L; Bonali, C; Lapadula, G

2015-12-30

Erasmus syndrome is defined as the association of silica exposure and subsequent development of systemic sclerosis. The limited number of cases reported in the literature mainly involves miners and only sporadically other professionals. We describe a case of Erasmus syndrome in a marble worker. A 68 year old man came to our observation complaining pelvic and scapular girdle pain, evening fever, intense weakness and emaciation for about 1 month. He also reported to have had Raynaud's phenomenon in his hands for the last 13 years. Also, his occupational history revealed a chronic exposure to silica dust. The patient presented pain in his shoulders and hips, moderate skin thickening and sclerosis in his hands and fingers extending proximally to his wrists. The diagnosis of systemic sclerosis was determined according to his clinical and medical history, the positivity of anti-Scl 70 antibodies, the nailfold capillaroscopy suggestive of an active scleroderma pattern and the detection of a mild restrictive pulmonary syndrome. The evaluation of the organbased complications excluded a gastroenterological and cardiovascular involvement, while the chest computed tomography (CT) detected multiple small nodules with a mantle distribution and enlarged lymph nodes with no signs of interstitial lung disease and fibrosis. Additional tests (positron emission tomography-CT, flexible bronchoscopy and broncho-alveolar lavage) excluded infectious diseases and cancer. However, given the pulmonary involvement, we performed a histological examination of the parenchyma and lymph nodes, which revealed a picture of pneumoconiosis. In the end, the occupational history and the findings from the diagnostic procedures led to the diagnosis of pulmonary silicosis. The precise definition of the pulmonary involvement was essential to the therapeutic approach to this patient.

Noonan's and DiGeorge syndromes with monosomy 22q11.

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Wilson, D I; Britton, S B; McKeown, C; Kelly, D; Cross, I E; Strobel, S; Scambler, P J

1993-01-01

A boy with the dysmorphic features of Noonan's syndrome and pulmonary valve stenosis who had evidence of hypoparathyroidism and abnormal T lymphocyte numbers in the neonatal period is reported. He had a normal karyotype but molecular analysis revealed a submicroscopic deletion within chromosome 22q11, the region deleted in DiGeorge syndrome. Thus this child has both Noonan's syndrome and DiGeorge syndrome; 22q11 is a candidate region for a gene defective in Noonan's syndrome.

Assessment of chronic thromboembolic pulmonary hypertension by three-dimensional contrast-enhanced MR angiography - comparison with selective intraarterial DSA

International Nuclear Information System (INIS)

Kreitner, K.F.; Ley, S.; Kauczor, H.U.; Kalden, P.; Pitton, M.B.; Thelen, M.; Mayer, E.; Laub, G.

2000-01-01

Purpose: This study compares contrast-enhanced 3D-MR angiography (MRA) of the pulmonary arteries with selective intraarterial DSA in patients with chronic thromboembolic pulmonary hypertension. Materials and methods: 20 patients preoperatively underwent a contrast-enhanced 3D-MRA of the pulmonary arteries at 1.5 T using the phased-array body coil. For MRA, we used a 3D-Flash-sequence after bolus timing. 2 radiologists analyzed the acquired image material in consensus with respect to the detection of central thromboembolic material and the visualization of the pulmonary arterial tree. Finally, the MR angiograms were compared with selective DSA images using surgical findings as the definitive standard. Results: MRA demonstrated central thromboembolic material, vessel cut-offs and abnormal proximal-to-distal tapering in all patients. Compared to DSA, MRA depicted the pulmonary vessels up to the segmental level in all cases, it was inferior to DSA in delineation of the subsegmental arteries (sensitivity 87%, specificity 100%). The central beginning of the thromboembolic occlusions seen at MRA corresponded to the beginning of the deobliteration procedure during pulmonary thromboendarterectomy in every case. (orig.) [de

Identification of chronic thromboembolic pulmonary hypertension with MR imaging

International Nuclear Information System (INIS)

Gefter, W.B.; Palevsky, H.I.; Dinsmore, B.J.; Reichek, N.; DeRoos, A.; Kressel, H.Y.

1988-01-01

Thromboembolic pulmonary hypertension (TE-PHT) may be treatable by thromboendarterectomy. To evaluate the role of MR imaging in TE-PHT, the authors imaged eight patients with primary pulmonary hypertension or ASDs and seven patients with TE-PHT. Patients underwent conventional spin-echo (SE) imaging; three of seven patients with TE-PHT and eight of eight without emboli underwent cine MR studies. TE-PHT findings included focal areas of vessel wall thickening or plaquelike lesions in the central pulmonary arteries in six of seven. (Thrombus was not distinguished from flow-related signal in the seventh patient). Studies performed on three patients after thromboendarterectomy showed significant resolution of vascular lesions. Patients without emboli showed dilated central vessels without focal wall or lumen abnormality. Cine studies differentiated flow-related signal from thrombus, but wall thickening was better appreciated on SE images. MR imaging appears useful in identifying patients with TE-PHT who may benefit from surgery

A Nonfatal Case of Dobrava Hantavirus Hemorrhagic Fever with Renal Syndrome Combined with Hantavirus Cardiopulmonary Syndrome

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Shemsedin Dreshaj

2018-01-01

Full Text Available Among hantaviruses (HTNV, 22 are known as pathogenic for humans. HTNV can cause two clinical entities: hemorrhagic fever with renal syndrome (HFRS and hantavirus pulmonary syndrome or hantavirus cardiopulmonary syndrome (HCPS. In most countries of Eastern Europe as well as in Kosovo, HTNV infection is presented mainly as HFRS. Here, we report a 20-year-old man with HFRS and HCPS caused by Dobrava hantavirus strain, successfully treated in Intensive Care Unit of Infectious Diseases Clinic, University Clinical Center of Kosovo. In HFRS endemic areas, patients with acute respiratory distress syndrome need to be evaluated for Dobrava hantavirus strain as a possible causative agent.

Prevalence of metabolic syndrome in Central America: a cross-sectional population-based study

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Roy A. Wong-McClure

2015-09-01

Full Text Available OBJECTIVE: To report the prevalence of metabolic syndrome (MetS as found by the Central American Diabetes Initiative (CAMDI study for five major Central American populations: Belize (national; Costa Rica (San José; Guatemala (Guatemala City; Honduras (Tegucigalpa; and Nicaragua (Managua. METHODS: Study data on 6 185 adults aged 20 years or older with anthropometric and laboratory determination of MetS from population-based surveys were analyzed. Overall, the survey response rate was 82.0%. MetS prevalence was determined according to criteria from the Adult Treatment Panel III of the National Cholesterol Education Program. The study's protocol was reviewed and approved by the bioethical committee of each country studied. RESULTS: The overall standardized prevalence of MetS in the Central American region was 30.3% (95% confidence interval (CI: 27.1-33.4. There was wide variability by gender and work conditions, with higher prevalence among females and unpaid workers. The standardized percentage of the population free of any component of MetS was lowest in Costa Rica (9.0%; CI: 6.5-11.4 and highest in Honduras (21.1%; CI: 16.4-25.9. CONCLUSIONS: Overall prevalence of MetS in Central America is high. Strengthening surveillance of chronic diseases and establishing effective programs for preventing cardiovascular diseases might reduce the risk of MetS in Central America.

Celiac artery compression syndrome with bilateral Bochdalek hernia

International Nuclear Information System (INIS)

Kara, K.; Verim, S.; Bozkurt, Y.; Tasar, M.

2012-01-01

Full text: Introduction: Celiac artery compression syndrome or median arcuate ligament syndrome is rare and controversial condition. The definition of the syndrome relies on a combination of both clinical and radiographic features. It typically occurs in young patients, who may present with epigastric pain and weight loss. Bochdalek hernia is the most common congenital diaphragmatic hernia in adults. Bilaterality of this pathology is rare. There are not many reports about the associated pathologies to Bochdalek hernia. Objectives and tasks: We aimed to demonstrate the computed tomography (CT) angiography findings of celiac artery compression syndrome with Bochdalek hernia that has detected incidentally. Materials and methods: A CT angiography was performed to 32-year-old patient having postphelebitic syndrome for the possible diagnosis as pulmonary embolus. Results: At the imaging pulmonary arteries and the branches were normal. Celiac artery compression syndrome with Bochdalek Hernia was detected incidentally. A %75 stenosis at the origin of celiac artery and post stenotic dilatation after the stenosis was seen due to the compression. A poster medial defect at the diaphragm was seen as an additional finding for the cause of Bochdalek hernia. Conclusion: Many incidental finding can be detected at vascular and non vascular area in the routine CT angiography imaging. The pathologies like celiac artery compression syndrome and congenital diaphragm pathologies can be detected easily at CT angiography method

Videodensitometry in rats with pulmonary damage due to microembolism

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Wegenius, G.; Wegener, T.; Ruhn, G.; Saldeen, T.; Erikson, U.

Serial pulmonary angiography with videodensitometry was performed in 18 rats with pulmonary damage caused by administration of a fibrinolysis inhibitor, tranexamic acid (200 mg/kg body weight injected intraperitoneally) and bovine thrombin (500 NIH/kg body weight injected into the right femoral vein). The mean transit time (MTT) was calculated from videodensitometry, the observed area of interest consisting of approximately one-third of the right lung, including both central and peripheral parts. The impact of the pulmonary damage was analysed by morphologic methods and correlated to MTT. Although a pressure rise presumably occurred in the pulmonary circulation, no change in MTT was found after induction of pulmonary damage, indicating opening of actual and potential anastomoses between pulmonary arterioles and venules to serve as by-pass portions and as a safety-valve mechanism for the capillary bed and the right heart, respectively. Another explanation to unchanged MTT may be opening of resting capillary beds. Two rats with very severe pulmonary damage showed prolonged MTT. These rats may have suffered from cardiac failure.

Videodensitometry in rats with pulmonary damage due to microembolism

International Nuclear Information System (INIS)

Wegenius, G.; Wegener, T.; Ruhn, G.; Saldeen, T.; Erikson, U.

1985-01-01

Serial pulmonary angiography with videodensitometry was performed in 18 rats with pulmonary damage caused by administration of a fibrinolysis inhibitor, tranexamic acid (200 mg/kg body weight injected intraperitoneally) and bovine thrombin (500 NIH/kg body weight injected into the right femoral vein). The mean transit time (MTT) was calculated from videodensitometry, the observed area of interest consisting of approximately one-third of the right lung, including both central and peripheral parts. The impact of the pulmonary damage was analysed by morphologic methods and correlated to MTT. Although a pressure rise presumably occurred in the pulmonary circulation, no change in MTT was found after induction of pulmonary damage, indicating opening of actual and potential anastomoses between pulmonary arterioles and venules to serve as by-pass portions and as a safety-valve mechanism for the capillary bed and the right heart, respectively. Another explanation to unchanged MTT may be opening of resting capillary beds. Two rats with very severe pulmonary damage showed prolonged MTT. These rats may have suffered from cardiac failure. (orig.)

Diagnosis and Management of Combined Central Diabetes Insipidus and Cerebral Salt Wasting Syndrome After Traumatic Brain Injury.

Science.gov (United States)

Wu, Xuehai; Zhou, Xiaolan; Gao, Liang; Wu, Xing; Fei, Li; Mao, Ying; Hu, Jin; Zhou, Liangfu

2016-04-01

Combined central diabetes insipidus and cerebral salt wasting syndrome after traumatic brain injury (TBI) is rare, is characterized by massive polyuria leading to severe water and electrolyte disturbances, and usually is associated with very high mortality mainly as a result of delayed diagnosis and improper management. We retrospectively reviewed the clinical presentation, management, and outcomes of 11 patients who developed combined central diabetes insipidus and cerebral salt wasting syndrome after traumatic brain injury to define distinctive features for timely diagnosis and proper management. The most typical clinical presentation was massive polyuria (10,000 mL/24 hours or >1000 mL/hour) refractory to vasopressin alone but responsive to vasopressin plus cortisone acetate. Other characteristic presentations included low central venous pressure, high brain natriuretic peptide precursor level without cardiac dysfunction, high 24-hour urine sodium excretion and hypovolemia, and much higher urine than serum osmolarity; normal serum sodium level and urine specific gravity can also be present. Timely and adequate infusion of sodium chloride was key in treatment. Of 11 patients, 5 had a good prognosis 3 months later (Extended Glasgow Outcome Scale score ≥6), 1 had an Extended Glasgow Outcome Scale score of 4, 2 died in the hospital of brain hernia, and 3 developed a vegetative state. For combined diabetes insipidus and cerebral salt wasting syndrome after traumatic brain injury, massive polyuria is a major typical presentation, and intensive monitoring of fluid and sodium status is key for timely diagnosis. To achieve a favorable outcome, proper sodium chloride supplementation and cortisone acetate and vasopressin coadministration are key. Copyright © 2016 Elsevier Inc. All rights reserved.

Bland-White-Garland syndrome – a rare and serious cause of failure to thrive

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Szmigielska, Agnieszka; Roszkowska-Blaim, Maria; Gołąbek-Dylewska, Małgorzata; Tomik, Agnieszka; Brzewski, Michał; Werner, Bożena

2013-01-01

Patient: Male, 0 Final Diagnosis: Bland-White-Garland syndrome Symptoms: Cardiomegaly, feeding problems Medication: — Clinical Procedure: Reimplantation of the left coronary artery to the aorta Specialty: Pediatrics and Neonatology Objective: Rare disease Background: Bland-White-Garland syndrome (BWGS) is a very rare disease characterized by anomalous origin of the left coronary artery from the pulmonary trunk (ALCAPA). WBGS affects 1 in every 300 000 live births. Children typically present with dyspnea, pallor, and failure to thrive. Without surgical repair, most of these children die during the first months of life. Case Report: This case report describes 3-month-old boy admitted to the hospital because of feeding problems. The boy was born at term, with birth weight 3200 g, and was 10 points in Apgar score. He was breast-fed from birth. From the seventh week of age, his mother observed his increasing difficulties with feeding. Physical examination revealed pale skin, diminished heart sounds, tachycardia, cardiomegaly, and hepatomegaly. Results of urine and blood tests and ultrasonography of the central nervous system and abdomen were normal. The chest radiography showed cardiomegaly and electrocardiogram revealed anterolateral myocardial infarction. On echocardiography, an anomalous left coronary artery arising from the pulmonary artery was found. The life-saving treatment of choice was immediate surgical reimplantation of the left coronary artery to the aorta. Conclusions: Children with congenital heart disease are often prone to malnutrition, but in rare cases failure to thrive and breast-feeding problems can be the first symptoms of life-threatening diseases like myocardial infarction secondary to Bland-White-Garland syndrome (BWGS). PMID:24086793

Acromegaly in a patient with a pulmonary neuroendocrine tumor: case report and review of current literature.

Science.gov (United States)

Krug, Sebastian; Boch, Michael; Rexin, Peter; Pfestroff, Andreas; Gress, Thomas; Michl, Patrick; Rinke, Anja

2016-06-27

Pulmonary neuroendocrine tumors (NET) form a heterogeneous group of rare diseases. In these tumors, paraneoplastic syndromes have been described to drive the course of the disease, among them acromegaly induced by paraneoplastic secretion of growth hormone-releasing hormone (GHRH). We report the case of a 43 years old patient initially diagnosed with acromegaly accompanied by weight gain and acral enlargement. Subsequently, further diagnostic work-up identified a solitary pulmonary neuroendocrine tumor (NET). Laboratory tests revealed markedly increased growth hormone (GH) and insulin-like growth factor 1 (IGF-1) without GHRH elevation in the absence of pituitary pathologies confirming the paraneoplastic origin of clinical presentation with acromegaly. Curative surgery was performed leading to normalization of the elevated hormone levels and improvement of the clinical symptoms. Immunohistochemically, a typical carcinoid (TC) was seen with low proliferation index and abundant IGF-1 expression. The association of acromegaly and pulmonary NET has only rarely been reported. We present an individual case of paraneoplastic GH- and IGF-1 secretion in a patient with pulmonary NET. Based on their rarity, the knowledge of paraneoplastic syndromes occurring in patients with pulmonary NET such as acromegaly due to paraneoplastic GH- and IGF-1 secretion is mandatory to adequately diagnose and treat these patients.

Review of the refeeding syndrome.

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Kraft, Michael D; Btaiche, Imad F; Sacks, Gordon S

2005-12-01

Refeeding syndrome describes a constellation of metabolic disturbances that occur as a result of reinstitution of nutrition to patients who are starved or severely malnourished. Patients can develop fluid and electrolyte disorders, especially hypophosphatemia, along with neurologic, pulmonary, cardiac, neuromuscular, and hematologic complications. We reviewed literature on refeeding syndrome and the associated electrolyte abnormalities, fluid disturbances, and associated complications. In addition to assessing scientific literature, we also considered clinical experience and judgment in developing recommendations for prevention and treatment of refeeding syndrome. The most important steps are to identify patients at risk for developing refeeding syndrome, institute nutrition support cautiously, and correct and supplement electrolyte and vitamin deficiencies to avoid refeeding syndrome. We provide suggestions for the prevention of refeeding syndrome and suggestions for treatment of electrolyte disturbances and complications in patients who develop refeeding syndrome, according to evidence in the literature, the pathophysiology of refeeding syndrome, and clinical experience and judgment.

Acute Respiratory Distress Syndrome in Severe Brain Injury

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Yu. A. Churlyaev

2009-01-01

Full Text Available Objective: to study the development of acute respiratory distress syndrome (ARDS in victims with isolated severe brain injury (SBI. Subject and methods. 171 studies were performed in 16 victims with SBI. Their general condition was rated as very critical. The patients were divided into three groups: 1 non-ARDS; 2 Stage 1 ARDS; and 3 Stage 2 ARDS. The indicators of Stages 1 and 2 were assessed in accordance with the classification proposed by V. V. Moroz and A. M. Golubev. Intracranial pressure (ICP, extravascular lung water index, pulmonary vascular permeability, central hemodynamics, oxygenation index, lung anastomosis, the X-ray pattern of the lung and brain (computed tomography, and its function were monitored. Results. The hemispheric cortical level of injury of the brain with function compensation of its stem was predominantly determined in the controls; subcompensation and decompensation were ascertained in the ARDS groups. According to the proposed classification, these patients developed Stages 1 and 2 ARDS. When ARDS developed, there were rises in the level of extravascular lung fluid and pulmonary vascular permeability, a reduction in the oxygenation index (it was 6—12 hours later as compared with them, increases in a lung shunt and ICP; X-ray study revealed bilateral infiltrates in the absence of heart failure in Stage 2 ARDS. The correlation was positive between ICP and extravascular lung water index, and lung vascular permeability index (r>0.4;p<0.05. Conclusion. The studies have indicated that the classification proposed by V. V. Moroz and A. M. Golubev enables an early diagnosis of ARDS. One of its causes is severe brainstem injury that results in increased extravascular fluid in the lung due to its enhanced vascular permeability. The ICP value is a determinant in the diagnosis of secondary brain injuries. Key words: acute respiratory distress syndrome, extravascu-lar lung fluid, pulmonary vascular permeability, brain injury

Centrally-located pulmonary hamartoma diagnosed in a 16-year-old ...

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4Department of Internal Medicine, Bugando Medical Centre, P.O. Box 1370, Mwanza, Tanzania. 5Department of .... These tumors can either be parenchymal or endobronchial in origin with parenchymal being the commonest type .... Klein, J.S. & Braff, S. (2008) Imaging evaluation of the solitary pulmonary nodule. Clinics in ...

Diagnostic accuracy of contrast-enhanced MR angiography and unenhanced proton MR imaging compared with CT pulmonary angiography in chronic thromboembolic pulmonary hypertension

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Rajaram, Smitha [Unit of Academic Radiology, University of Sheffield, Sheffield (United Kingdom); Academic Unit of Radiology, C Floor, Royal Hallamshire Hospital, Sheffield (United Kingdom); Swift, Andrew J.; Wild, Jim M. [Unit of Academic Radiology, University of Sheffield, Sheffield (United Kingdom); Sheffield Cardiovascular Biomedical Research Unit, Sheffield (United Kingdom); Capener, David; Telfer, Adam [Unit of Academic Radiology, University of Sheffield, Sheffield (United Kingdom); Davies, Christine; Hill, Catherine [Sheffield Teaching Hospitals Trust, Department of Radiology, Sheffield (United Kingdom); Condliffe, Robin; Elliot, Charles; Kiely, David G. [Sheffield Pulmonary Vascular Disease Unit, Royal Hallamshire Hospital, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield (United Kingdom); Sheffield Cardiovascular Biomedical Research Unit, Sheffield (United Kingdom); Hurdman, Judith [Sheffield Pulmonary Vascular Disease Unit, Royal Hallamshire Hospital, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield (United Kingdom)

2012-02-15

To evaluate the diagnostic accuracy of contrast-enhanced MR angiography (CE-MRA) and the added benefit of unenhanced proton MR angiography compared with CT pulmonary angiography (CTPA) in patients with chronic thromboembolic disease (CTE). A 2 year retrospective study of 53 patients with chronic thromboembolic pulmonary hypertension who underwent CTPA and MRI for suspected pulmonary hypertension and a control group of 36 patients with no CT evidence of pulmonary embolism. The MRI was evaluated for CTE and the combined diagnostic accuracy of ce-MRA and unenhanced proton MRA was determined. CE-MRA generated lung perfusion maps were also assessed. The overall sensitivity and specificity of CE-MRA in diagnosing proximal and distal CTE were 98% and 94%, respectively. The sensitivity improved from 50% to 88% for central vessel disease when CE-MRA images were analysed with unenhanced proton MRA. The CE-MRA identified more stenoses (29/18), post-stenosis dilatation (23/7) and occlusions (37/29) compared with CTPA. The CE-MRA perfusion images showed a sensitivity of 92% for diagnosing CTE. CE-MRA has high sensitivity and specificity for diagnosing CTE. The sensitivity of CE-MRA for visualisation of adherent central and lobar thrombus significantly improves with the addition of unenhanced proton MRA which delineates the vessel wall. (orig.)

Diagnostic accuracy of contrast-enhanced MR angiography and unenhanced proton MR imaging compared with CT pulmonary angiography in chronic thromboembolic pulmonary hypertension

International Nuclear Information System (INIS)

Rajaram, Smitha; Swift, Andrew J.; Wild, Jim M.; Capener, David; Telfer, Adam; Davies, Christine; Hill, Catherine; Condliffe, Robin; Elliot, Charles; Kiely, David G.; Hurdman, Judith

2012-01-01

To evaluate the diagnostic accuracy of contrast-enhanced MR angiography (CE-MRA) and the added benefit of unenhanced proton MR angiography compared with CT pulmonary angiography (CTPA) in patients with chronic thromboembolic disease (CTE). A 2 year retrospective study of 53 patients with chronic thromboembolic pulmonary hypertension who underwent CTPA and MRI for suspected pulmonary hypertension and a control group of 36 patients with no CT evidence of pulmonary embolism. The MRI was evaluated for CTE and the combined diagnostic accuracy of ce-MRA and unenhanced proton MRA was determined. CE-MRA generated lung perfusion maps were also assessed. The overall sensitivity and specificity of CE-MRA in diagnosing proximal and distal CTE were 98% and 94%, respectively. The sensitivity improved from 50% to 88% for central vessel disease when CE-MRA images were analysed with unenhanced proton MRA. The CE-MRA identified more stenoses (29/18), post-stenosis dilatation (23/7) and occlusions (37/29) compared with CTPA. The CE-MRA perfusion images showed a sensitivity of 92% for diagnosing CTE. CE-MRA has high sensitivity and specificity for diagnosing CTE. The sensitivity of CE-MRA for visualisation of adherent central and lobar thrombus significantly improves with the addition of unenhanced proton MRA which delineates the vessel wall. (orig.)

MSCT diagnosis of pulmonary embolism

International Nuclear Information System (INIS)

Fang Jie; Yang Li; Zhang Ailian; Li Gongjie; Ren Shuanqun

2004-01-01

Objective: To analyze the distribution and size of intraluminal filling defects and corresponding parenchymal findings in pulmonary embolism (PE) on MSCT. Methods: Twelve PE cases diagnosed by spiral CTA were retrospected. The involved pulmonary arteries according to presence of filling defects were classified as central, peripheral and mixed type. Lobular pulmonary artery with filling defects classified as complete defect and partial defects. The presence or absence of parenchymal abnormalities and pleural effusion was noted. Results: Peripheral PE was identified in 2 of the 12 patients with PE, and others were with mixed type. Anatomic evaluation of filling defects at lobular level revealed a total of 25 arteries in 12 patients including 9 with complete filling defect and 16 with partial filling defect. Only 3 cases were found to have parenchyma consolidation from all the 12 cases, and 1 case was diagnosed as pneumonia. Pleural effusion was seen in 4 cases. Conclusion: Parenchymal changes in PE do not often take place and lack specified signs. Diagnosis of PE is based on conformation of parenchymal changes with occluded segment of pulmonary arteries. Clinical history and follow up are of very importance in defining parenchyma change arose from PE. (authors)

[Pulmonary hypertensive crisis in children with idiopathic pulmonary arterial hypertension undergoing cardiac catheterization: the risk factors and clinical aspects].

Science.gov (United States)

Zhang, C; Zhu, Y; Li, Q Q; Gu, H

2018-06-02

Objective: To investigate the risk factors, clinical features, treatments, and prevention of pulmonary hypertensive crisis (PHC) in children with idiopathic pulmonary arterial hypertension (IPAH) undergoing cardiac catheterization. Methods: This retrospective study included 67 children who were diagnosed with IPAH and underwent cardiac catheterization between April 2009 and June 2017 in Beijing Anzhen Hospital. The medical histories, clinical manifestations, treatments, and outcomes were characterized. Statistical analyses were performed using t test, χ(2) test and a multiple Logistic regression analysis. Results: During cardiac catheterization, five children developed PHC who presented with markedly elevated pulmonary artery pressure and central venous pressure, decline in systemic arterial pressure and oxygen saturation. Heart rate decreased in 4 cases and increased in the remaining one. After the treatments including cardiopulmonary resuscitation, pulmonary vasodilator therapy, improving cardiac output and blood pressure, and correction of acidosis, 4 of the 5 cases recovered, while 1 died of severe right heart failure with irreversible PHC 3 days after operation. Potential PHC was considered in 7 other patients, whose pulmonary artery pressure increased and exceeded systemic arterial pressure, oxygen saturation decreased, and central venous pressure and vital signs were relatively stable. Univariate analysis showed that the risk factors of PHC in children with IPAH undergoing cardiac catheterization were younger age ( t= 3.160, P= 0.004), low weight ( t= 4.004, Phistory of syncope (χ(2)=4.948, P= 0.026), and WHO cardiac functional class Ⅲ or Ⅳ (χ(2)=19.013, Pcatheterization. WHO cardiac functional class may be associated with PHC. Integrated treatment is required for these patients. Reducing risk factors, early identification, and active treatment may help to prevent the occurrence and progression of PHC.

Eosinophilic granulomatosis with polyangiitis (formerly known as Churg-Strauss syndrome as a differential diagnosis of hypereosinophilic syndromes

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Yuri Albuquerque Pessoa Santos

2017-01-01

Full Text Available Eosinophilic granulomatosis with polyangiitis (EGPA, formerly known as Churg-Strauss syndrome, is a rare systemic disease situated between primary small vessel vasculitides associated with antineutrophil cytoplasmic antibodies (ANCAs and hypereosinophilic syndromes (HES. Here, we present a case of EGPA in a 38-year-old male, with a previous diagnosis of asthma, who presented with fever, migratory lung infiltrates and systemic eosinophilia that was refractory to previous courses of antibiotics. This case highlights the importance of the primary care physician understanding the differential diagnosis of pulmonary eosinophilic syndromes.

Prune-belly syndrome in two children and review of the literature.

Science.gov (United States)

Bogart, Megan M; Arnold, Holly E; Greer, Kenneth E

2006-01-01

Prune-belly syndrome is a congenital disorder characterized by abdominal wall musculature deficiency, urinary tract anomalies, and bilateral cryptorchidism. Because of the defect in the musculature, the abdominal skin has a peculiar wrinkled appearance. The syndrome is commonly associated with pulmonary, skeletal, cardiac, and gastrointestinal defects. Developmental delays and growth retardation have also been reported. The incidence of prune belly syndrome is approximately 1:40,000 live births. Over 95% of patients are men. Urinary tract disease is the major prognostic factor, with the complications of pulmonary hypoplasia and end stage renal disease resulting in a mortality rate of 60%. Treatment involves surgical correction of the abdominal wall defect and urinary tract abnormalities, early orchiopexy, and supportive management of associated defects.

[Diagnosis of pulmonary hemorrhage of the newborn infants using lung ultrasonography].

Science.gov (United States)

Liu, J; Fu, W; Chen, S W; Wang, Y

2017-01-02

Objective: To investigate the accuracy and reliability of lung ultrasound in diagnosis of pulmonary hemorrhage of the newborn infants. Method: From January 2014 to May 2016, 142 neonates from the Army General Hospital of the Chinese PLA were enrolled in the study. They were divided into two groups: a study group of 42 neonates, who were diagnosed with pulmonary hemorrhage according to their medical history, clinical manifestations and chest X-ray findings, and a control group of 100 neonates with no lung disease. All subjects underwent bedside lung ultrasound in a quiet state in a supine, lateral or prone posture, performed by a single experienced physician. The ultrasound findings were compared between the two groups.Fisher's exact test was uesd for comparison between two groups. Result: The lung ultrasound main findings associated with pulmonary hemorrhage included: (1) Shred sign: which was seen in 40 patients(95%). (2) Lung consolidation with air bronchograms: which were seen in 35 patients(83%). (3) Pleural effusion: which was seen in 34 infants(81%), pleurocentesis confirmed that the fluid was really bleeding.(4)Atelectasis: which was seen in 14 cases(33%). (5) Pleural line abnormalities and disappearing A-lines with an incidence of 100%. (6) Alveolar-interstitial syndrome: 5 patients(12%)had the main manifestations of alveolar-interstitial syndrome. The above signs were not seen in normal controls (all P hemorrhage, which is suitable for routine application for the diagnosis of pulmonary hemorrhage in the neonatal intensive care unit.

Histopathology of the great vessels in patients with pulmonary arterial hypertension in association with congenital heart disease: large pulmonary arteries matter too.

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Prapa, Matina; McCarthy, Karen P; Dimopoulos, Konstantinos; Sheppard, Mary N; Krexi, Dimitra; Swan, Lorna; Wort, S John; Gatzoulis, Michael A; Ho, Siew Yen

2013-10-03

Pulmonary arterial hypertension (PAH) is considered primarily a disease of the distal pulmonary arteries whereas little is known on the effect of long-standing pulmonary hypertension on the larger proximal pulmonary arteries. This study aims to investigate the structural changes in the great arteries of adults who developed PAH in association with congenital heart disease (CHD), with severe cases termed Eisenmenger syndrome. We performed macroscopic and light microscopy analyses on the great arteries of 10 formalin-fixed human hearts from patients with PAH/CHD and compared them to age-matched healthy controls. A detailed histology grading score was used to assess the severity of medial wall abnormalities. Severe atherosclerotic lesions were found macroscopically in the elastic pulmonary arteries of 4 PAH/CHD specimens and organised thrombi in 3; none were present in the controls. Significant medial wall abnormalities were present in the pulmonary trunk (PT), including fibrosis (80%), and atypical elastic pattern (80%). Cyst-like formations were present in less than one third of patients and were severe in a single case leading to wall rupture. The cumulative PT histology grading score was significantly higher in PAH/CHD cases compared to controls (parteries. These abnormalities are likely to affect haemodynamics and contribute to morbidity and mortality in this cohort. Copyright © 2013 Elsevier Ireland Ltd. All rights reserved.

Churg-Strauss syndrome in childhood: a case report.

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Albahri, Ziad; Minxová, Lenka; Lukeš, Antonín; Mawiri, Abdul Al; Štefáčková, Šárka

2014-10-01

Churg-Strauss syndrome is a rare form of small-vessel vasculitis. In the current report, we describe the case of a 17-year-old Czech girl predominantly characterized by peripheral neuropathy, the presence of cardiac and pulmonary involvement, hypereosinophilia, asthma, and sinusitis that led to the diagnosis of Churg-Strauss syndrome. © The Author(s) 2013.

Risk factors for profuse systemic-to-pulmonary artery collateral burden in hypoplastic left heart syndrome.

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Prakash, Ashwin; Satiroglu, Elif; Porras, Diego; McElhinney, Doff B; Keane, John F; Lock, James E; Geva, Tal; King, Wilson; Powell, Andrew J

2013-08-01

Risk factors for developing systemic-to-pulmonary artery collaterals (SPCs) in hypoplastic left heart syndrome (HLHS) are unknown. We performed a retrospective case-control study to identify risk factors for developing a profuse SPC burden in HLHS. Angiograms of 439 patients with HLHS (performed <2 years of age) were reviewed using a previously published angiographic grading scale to identify cases (profuse SPC burden, n = 20) and controls (no or minimal SPC burden, n = 35). In univariate analyses, profuse SPC burden was associated with mitral atresia and aortic atresia subtype (MA/AA) (65% vs 14%, p <0.0001), use of a Sano shunt (70% vs 37%, p = 0.03), longer log-transformed durations of intensive care unit stay (p = 0.02), hospital stay (p = 0.002), pleural drainage (p = 0.008) after stage 1 palliation, lower oxygen saturation at discharge after stage 1 palliation (82 ± 4 vs 85 ± 4%, p = 0.03), and a history of severe shunt obstruction (37% vs 11%, p = 0.04). In a multivariate logistic regression model, profuse SPC burden was associated with MA/AA subtype (odds ratio 6.6), Sano shunt type (odds ratio 8.6), and log-transformed duration of hospital stay after stage 1 (odds ratio 7.9, model p <0.0001, area under the curve 0.88). Nonassociated parameters included fetal aortic valve dilation, severe cyanotic episodes, number of days with open sternum or number of additional exploratory thoracotomies after stage 1 palliation, pulmonary vein stenosis, and restrictive atrial septal defect. In conclusion, in the present case-control study of patients with HLHS, the development of a profuse SPC burden was associated with MA/AA subtype, Sano shunt type, and longer duration of hospital stay after stage 1 palliation. Copyright © 2013 Elsevier Inc. All rights reserved.

Acute pulmonary embolism in helical computed tomography

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