Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0231807 (exertional dyspnea)
 3,402 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A rare case of pulmonary hypertension due to glycogen storage disease type II (Pompe's disease) was reported. An 18-year-old girl was admitted to Kawasaki Medical School Hospital because of cyanosis, dyspnea on exertion and amenorrhea. She was 149 cm in height and 29 kg in body weight. Clinical examination revealed that pulmonary artery pulse and right ventricular heave were palpable over the precordium. On auscultation, an accentuated pulmonic second heart sound, pulmonic ejection sound and diastolic decrescendo murmur (Levine III/VI) were heard in the second intercostal space at the right sternal border. Her skeletal muscles, especially her intercostal muscles were generally weak and atrophic. Her electrocardiogram showed a pulmonary P-wave and right ventricular hypertrophy. The chest X-ray revealed right ventricular enlargement and a dilated pulmonary trunk. On echocardiography, the right ventricle and the main pulmonary artery were dilated, and a systolic notch of the pulmonary valve was found. Swan-Ganz catheterization disclosed that pulmonary artery pressure, right ventricular pressure and mean pulmonary capillary wedge pressure were 76/35 (50) mmHg, 76/12 mmHg and 10 mmHg, respectively. Respiratory function tests showed severe restrictive ventilatory impairment with hypercapnea and hypoxemia. On biopsy of the left quadriceps femoris muscle, the most striking finding was numerous intracytoplasmic vacuoles. The small vacuoles were stained with PAS and acid phosphatase. Electron microscopy showed massive glycogen accumulation in the sarcoplasm and membrane bound vacuoles (glycogenosome). Alpha-1, 4-glucosidase activity in the peripheral lymphocytes was definitely decreased. Her pulmonary hypertension resulted from respiratory muscular atrophy and alveolar hypoventilation caused by Pompe's disease.
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PMID:[Pulmonary hypertension due to glycogen storage disease type II (Pompe's disease): a case report]. 281 49

A 17-year-old girl developed fever, cough and hemoptysis, as well as bouts of septic fever, dyspnea on exertion, cyanosis and weight loss. Opacification in the left lower lung was a persistent feature. Complement-fixation reaction to Aspergillus fumigatus was 1:80, and there were four precipitation lines against this fungus on immunoelectrophoresis. Aspergilli were also demonstrated in sputum and bronchial aspirate. Because the patient's condition deteriorated an atypical lingula resection was performed and cortisone treatment begun postoperatively. Subsequently all abnormal findings disappeared and the patient was cured. Two subsequent exposures to Aspergillum on the parental farm produced high fever, cough, dyspnea and thoracic pain. Histological examination of the lingular specimen revealed bronchocentric granulomatosis. In this case it was the rarely occurring morphological manifestation of an allergic bronchopulmonary aspergillosis without bronchial asthma.
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PMID:[Bronchocentric granulomatosis as a manifestation of allergic bronchopulmonary aspergillosis without bronchial asthma]. 359 64

A 22-year-old woman is seen for progressive dyspnea on exertion experienced for the past six months. She has no dyspnea at rest, orthopnea, cough, or other symptoms. There is no history of heart murmurs, rheumatic fever, or pulmonary disease. Physical examination shows a healthy-appearing young woman with no cyanosis or clubbing. The jugular venous pulse shows a prominent A wave, with normal mean venous pressure. The lungs are clear to examination. The cardiac examination is normal except for a loud second heart sound that is narrowly split in expiration and a little more widely split in inspiration. The chest roentgenogram shows slight cardiac enlargement of nonspecific contour, with a considerably enlarged main pulmonary artery segment and moderately prominent central pulmonary artery branches. The peripheral lung fields are unremarkable. The electrocardiogram is shown.
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PMID:Dyspnea, right-axis deviation, and abnormal P waves in a young woman. 641 83

Sixty-six patients with pulmonic valvular stenosis were evaluated by cardiac catheterization and cine-angiography over the past four years in a recently established cardiovascular center in Syria. Patients were divided according to severity of pressure gradient across the pulmonic valve into three groups: Group I (25 patients) had gradients below 50 mm. Hg, Group II (14 patients) had gradients between 50 and 100 mm. Hg, and Group III (27 patients) had gradients exceeding 100 mm. Hg. Clinical symptoms and signs including dyspnea on exertion, palpitation, cyanosis, and characteristics of the murmur and the pulmonic second sound were correlated with severity and grouping along with electrocardiographic and radiographic findings. Valvulotomy was carried out in 23 patients (35%). Short term clinical results were excellent in 19 patients, but long term course of the non-operated and post-operative cases is yet to be established.
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PMID:Pulmonic valvular stenosis: clinical-hemodynamic correlation and surgical results. 671 6

A 16-year-old boy with congenital hepatic fibrosis, on whom a portocaval anastomosis was performed because of bleeding esophageal varices, developed lip cyanosis, exertional dyspnea, and clubbing of the fingers 7 years postoperatively. This progressive syndrome was caused by a right-to-left shunt due to the presence of pulmonary arteriovenous fistulas. Increased erythrocyte sedimentation rate, hypergammaglobulinemia, and histological features of cholangitis were also present. It is proposed that this unusual pulmonary complication of congenital hepatic fibrosis could be due either to congenital pulmonary abnormalities or to a chronic unrecognized cholangitis causing a progressive deterioration of liver histology and function, with subsequent opening of pulmonary arteriovenous shunts.
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PMID:Pulmonary arteriovenous fistulas: an unusual complication of congenital hepatic fibrosis. 688 43

Thirteen of 308 patients (4.2%), who had received right-sided valved extracardiac conduits at the Mayo Clinic from November, 1972, to April, 1977, have required conduit replacement because of obstruction. Patients were 5 to 16 years old at initial operation and 8 to 20 years old at reoperation; the duration of conduit implantation was 27 to 79 months (mean 50). Four patients (31%) were asymptomatic; exertional dyspnea was present in eight (62%) and dizziness was reported in one (8%). Signs of conduit obstruction included increasing intensity of murmurs in 11 (85%), cyanosis in two (15%), and heart failure in one (8%). Peak pressure gradients from the right ventricle to the pulmonary arteries ranged from 50 to 140 mm Hg (mean 87) and correlated well with the degree of conduit obstruction. Catheter pullback allowed accurate localization of stenosis within the conduit, whereas angiography alone did not. The site of major obstruction was in the proximal conduit in five (38%), at the valve in nine (69%), and in the distal conduit or side branches in six (46%); stated differently, major stenosis affected the valve alone in five (38%), the graft alone in four (31%), and both the valve and the graft in four (31%). Valvular changes leading to stenosis included thrombosis, commissural fusion, and calcification, and changes consistent with insufficiency included tears, fusion of cusps to the conduit wall, and, in one case, infective endocarditis. Within the conduit, nonvalvular obstruction was due to formation of a thick fibrous peel (or neointima). Progressive thickening of the peel appeared to be due to organization of thrombus between the peel and conduit and not due to luminal mural thrombus. In one case, the peel formed a flap-valve, causing even further obstruction. Since many patients are asymptomatic, and since late conduit stenosis may develop unpredictably by several mechanisms, long-term follow-up is necessary.
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PMID:Clinicopathological correlates of obstructed right-sided porcine-valved extracardiac conduits. 720 68

The double-outlet right ventricle with L-malposition (DORV (S, D, L)) is one of the rare congenital heart diseases. Intracardiac rerouting with a internal conduit has been indicated in principle to it as the radical operation. DORV (S, D, L) is often combined with the pulmonic stenosis (PS), and Rastelli's operation is indicated to the case in which release of PS is difficult. We report a case of DORV (S, D, L) combined with PS and the hypoplastic right ventricle which is treated by the Fontan-type procedure. The patient was a 35-year-old female who had undergone Glenn's operation at 10 years of age under the diagnosis of transposition of the great arteries. Recently she complained of aggravation of the cyanosis and dyspnea on exertion. After thorough examinations, the disease was diagnosed as DORV (S, D, L) with doubly committed VSD which was combined with severe PS, hypoplastic right ventricle (29% of normal) and left-sided juxtaposition of atrial appendages. The development of her distal pulmonary arteries was estimated well enough (PA-index 448 mm2/m2). We judged that intracardiac rerouting with an internal conduit could not be applied due to her severe pulmonic stenosis, and that Rastelli's operation as the biventricular repair was also impossible because of her hypoplastic right ventricle. Thus we adopted the Fontan-type procedure with a new septation in the right atrium, namely oblique partition, and attained a good result. To our knowledge, this case is the fourth case report in Japan of DORV (S, D, L) treated with the Fontan-type procedure.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Fontan-type procedure for an adult case of double-outlet right ventricle (S, D, L)]. 760 12

We report the case of a patient, a 19-year-old young man, with a rare malformation of the pulmonary blood vessels--a complex arteriovenous (A-V) fistula. The disease was characterized by typical signs of the right-to-left shunt: cyanosis, clubbed fingers and exertional dyspnea. Hypoxemia and polyglobulia were present in the blood examination findings and functional tests showed significantly reduced diffusion lung capacity and progressive dyspnea, while ergometry revealed cyanosis. The physical examination, ECG and ultrasound of the heart were normal as well as aortography. The final diagnosis was made by pulmonary angiography which showed a complex A-V malformation of several feeding arteries and profuse initial multiple venous drainage. Following a successful surgical procedure the diagnosis was also confirmed histopathologically-diffuse teleangiectatic A-V fistula of the lower lobe of the left lung. Following surgery cyanosis, dyspnea, hypoxemia and polyglobulia disappeared and the young man has been well for the following two years.
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PMID:[Pulmonary arteriovenous fistula in an adolescent]. 765 Oct 67

A 76-year-old man was admitted to our hospital in December of 1990, for investigation of progressive dyspnea on exertion over the previous 3 months. Physical examination revealed cyanosis, but no edema. Cardiomegaly was seen on chest X-ray, and findings compatible with right-sided congestive heart failure were revealed by ECG and echocardiography. Lung perfusion scintigrams showed multiple defects in both lungs, but no abnormal findings were detected on a ventilation study. Venograms of the lower extremities disclosed thrombosis of the right femoral vein. Therefore, a diagnosis of recurrent pulmonary thromboembolism was made. Furthermore, lupus anticoagulant and IgG-class anticardiolipin antibody were noted in the serum. The patient was treated with intravenous heparin, long-term warfarin, and transvenous placement of a Greenfield filter in the inferior vena cava. The clinical symptoms and signs improved, and no recurrence has been seen during the 13 months since the onset. On discharge, both antiphospholipid antibodies were negative. This case of primary antiphospholipid syndrome without autoimmune disease, with transiently positive antibodies only in the exacerbation phase, is discussed herein.
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PMID:[A case of recurrent pulmonary thromboembolism presenting with positive antiphospholipid antibodies]. 796 48

We report the experience with and evaluation of treatment strategies in fibrosing alveolitis and desquamative interstitial pneumonitis (FA/DIP) over the last 16 years by a review of all cases referred to a tertiary referral center. There were 25 cases, 16 boys and 9 girls (mean age at onset, 2.3 years; range, 7 days to 11.6 years). In each case the diagnosis was confirmed by open lung biopsy at a mean age of 3.3 years (range, 7 weeks to 15.1 years). Presently features were tachypnea (19), cyanosis (15), cough (12), exertional dyspnea (7), recurrent chest infections +/- wheezing (9), and clubbing (8). Four patients recovered without antiinflammatory medication. The others received specific treatment. Of 11 patients given only prednisolone, six improved, two did not, and three died despite treatment. Of five patients receiving only chloroquine, four responded. Five patients received both prednisolone and chloroquine; one died, two responded well. There was poor progress in the remaining two. Of the 10 patients receiving chloroquine six (60%) showed a good response. A younger presentation carried a worse prognosis, but chest radiology at presentation and outcome were not interrelated. Those with mild histological changes all survived, but severe desquamation or fibrosis at biopsy was not related to outcome. In four cases there was a family history (16%). Patients with FA/DIP probably represent a disease spectrum of multiple etiology with a variable prognosis and response to treatment.
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PMID:Fibrosing alveolitis and desquamative interstitial pneumonitis. 809 Jun 6


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