Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0023890 (cirrhosis)
 42,195 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Among 200,000 infants screened for alpha 1-antitrypsin (alpha 1-AT) deficiency, 125 Pi Z, 48 Pi Z, 1Pi S-, and 2 Pi Z- children were followed up prospectively. Eleven percent of the Pi Z infants had neonatal cholestasis, and at 2 years of age three of them had cirrhosis. About 50% of the asymptomatic Pi Z and Pi Z- subjects occasionally had serum alanine aminotransferase (ALAT) levels above normal, and in 15% of them the levels were probably permanently increased during the first two years of life. Two previously healthy Pi Z children had transient symptoms of liver disease at age 2 years in connection with severe infections. The Pi SZ children had no significant clinical liver disease and only two had abnormal serum ALAT levels. Among Pi Z children up to 2 years of age the following diseases were also encountered: eight had recurrent bronchitis with wheezing, two had persistant cough (both had cirrhosis), one had severe pneumonia, one was mentally retarded, three had urinary tract infections, six had pronounced eczema, one had allergic shock, and three had congenital malformations. Among the Pi SZ children one had recurrent bronchitis, one had eczema, and one had juvenile rheumatoid arthritis. Three children, two Pi Z and one Pi SZ, have died. The Pi Z- and Pi S- subjects were healthy. In conclusion a variety of significant symptoms were observed in about 30% of the Pi Z children compared with 6% of the Pi SZ children during the first two years of life.
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PMID:alpha 1-antitrypsin deficiency in early childhood. 30 15

Percutaneous transhepatic catheterization of the portal venous system and pressure readings were performed in nineteen patients with cirrhosis of the liver and bleeding varices. Portal pressures were recorded in awake and mobile patients in supine, sitting and standing position, during sleep, ingestion of food, Valsalva manoeuvre and coughing. No significant differences were recorded in the different postures, during sleep or food intake. Four patients with hepatofugal portal blood flow had, however, lowest pressure in standing position. During Valsalva manoeuvre portal pressure was doubled, and it became fourfold during coughing. Elevations of this magnitude have not previously been reported. A relationship was found between portal pressure and size of varices.
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PMID:The influence of posture, Valsalva manoeuvre and coughing on portal hypertension in cirrhosis. 55 37

1. An infusion of 3 gamma/kg/min dopamine causes a significant increase in the renal plasma flow and the glomerulum filtration rate. This dosage does not cause a change of the mean systolic and arterial pressure. This effect may also be observed in patients with hepatic cirrhosis. 2. The wedged hepatic vein pressure, an indicator for the portal pressure, only shows a slight increase (9,46 +/- 9,41%) as compared to the initial pressure produced by the mentioned dose. Measurements of the spleen pulpa pressure, which likewise indicates the portal pressure, showed an increase of pressure up to 100% due to pressing or coughing. 3. If in the case of bleeding oesophageal varices acute renal failure might develop, the advantage of the effect of dopamine in stimulating the blood flow through the kidneys may be considered more important than the minute danger of a slight increase of the portal pressure, which might provoke haemorrhage.
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PMID:[Effect of dopamine on the portal pressure]. 122 May 17

Seven months after orthotopic liver transplantation because of terminal postnecrotic cirrhosis, a 55-year-old patient was found to have pulmonary tuberculosis with fever, cough and an infiltration in the left upper lobe. Sputum culture grew M. Tuberculosis. He received ethambutol (1.6 g/d) and isoniazid (400 mg three times weekly, after 1 1/2 months 200 mg thrice weekly). After eleven months the tuberculosis had healed with only minor residua. The function of the transplant was very good in the fourth year after the operation. The main side effect was a reversible rise in liver enzymes. If possible, patients should not be given hepatotoxic tuberculostatic agents after liver transplantation, in no circumstances rifampicin. Dosage should be adapted to the liver function so as to avoid damage to transplant function.
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PMID:[Tuberculosis following liver transplantation]. 328 35

A case is presented of priapism resulting from disseminated intravascular coagulopathy (DIC), which was diagnosed by pathological studies of the amputated penis and skin biopsy. To our knowledge, this is the first case reported in Japan. This 72-year-old-man visited a hospital complaining of fever and cough, and was administrated for treatment of bronchitis and liver cirrhosis. A few days after admission, multiple purpura with edema and pain appeared over the skin regions on the bilateral knee joint, foot joint and upper extremities. A week after purpura appeared, priapism began. Regardless of irrigation and aspiration of corpora cavernosa and glans-cavernosa-fistula creation, penile necrosis developed. We had to perform penile amputation. The pathology of the amputated penis and skin, and blood coagulative examination suggested that DIC resulted in priapism. DIC was controllable by the use of FOY and heparin. He was discharged and is an outpatient.
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PMID:[A case of priapism caused by disseminated intravascular coagulopathy]. 361 17

Clinical and autopsy findings obtained from 15 male patients treated for acquired immunodeficiency syndrome (AIDS) at 3 hospitals in Sao Paulo provided a clearer profile of AIDS cases in Brazil. Of the 12 patients whose sexual orientation was recorded, 9 were homosexual and 3 were bisexual. 75% were between the ages of 22-36 years; 14 were white. The duration of diseases ranged from 14 days-7 months in this series, confirming the rapid evolution of AIDS from 1st symptom to death. The most common clinical manifestations of disease were fever, cough, weight loss, diarrhea, and lymphadenopathy. Organs most frequently involved were the lungs (13 cases) and encephalum (9 cases). Microscopic findings revealed 9 types of microorganisms, fungi, and protozoa, the most common of which was Cytomegalovirus (7 cases). The cause of death was meningoencephalitis in 7 cases and panlobar pneumonia in 3 cases. The incidence of Kaposi's sarcoma (2 cases) was surprisingly low in this series. In addition to lesions produced by microorganisms, there were important associated lesions represented by lymphocytic depletion, acute myocarditis, brown atrophy of neuronia, acute pancreatitis, and liver cirrhosis. Several microorganisms and tumors in these AIDS patients were discovered only at autopsy, confirming the importance of necropsy to the study of the natural history of this disease. An unexpected pathological finding in this series was the absence of cellular reactions to microorganisms, particularly Pneumocystis carinii, Cryptococcus neoformans, and Mycobacterium tuberculosis.
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PMID:Acquired immune deficiency syndrome (AIDS) in Brazil. Necropsy findings. 362 18

A 80-year-old man was admitted to our hospital complaining of appetite loss, dry cough, and dyspnea on exertion. Chest X-ray film showed massive right pleural effusion. On physical examination, the right thorax was dull to percussion, and breath sounds of the right lung field were diminished. Examination of the cardiovascular system was unremarkable. Hepatosplenomegaly and ascites were not evident. The character of the pleural fluid was transudative. Cytological findings were negative and culture of the pleural fluid was normal. First, the pleural fluid was diminished by thoracic drainage. But, after the cessation of drainage, the pleural fluid soon reaccumulated. Abdominal ultrasonography showed typical liver cirrhosis pattern without ascites. An intraperitoneal injection of 99mTc-sulfur colloid was followed by positive uptake of radioactivity in the right pleural cavity from 1 hour after the injection, demonstrating the existence of a direct perito-pleural communication. Thus, the diagnosis of liver cirrhosis presenting with right pleural fluid, without ascites was made. This case indicates that the absence of ascites cannot exclude cirrhosis as the etiologic cause of pleural effusion.
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PMID:[A case of liver cirrhosis presenting with right pleural fluid without ascites]. 780 61

We compared arterial blood gas analysis and 99mTc-MAA lung perfusion scintigraphy before and after endoscopic injection sclerotherapy (EIS) in 17 cases of esophageal varices complicating liver cirrhosis. EIS was performed by the intra-variceal injection method with 5% ethanolamine oleate (EO) as the sclerosant agent. In blood gas analysis, the mean values of partial arterial oxygen tension (PaO2) and oxygen saturation (SaO2) were decreased, and the mean value of alveolar-arterial O2 difference (AaDO2) was increased, compared with before EIS while breathing room air. On lung perfusion scintigram, the pulmonary blood flow was markedly decreased, demonstrating hypoperfusion and/or perfusion defect, compared with before EIS in most the cases. It was suggested that pulmonary embolism and/or pulmonary circulatory disturbance occurred after EIS. It is important to note that pulmonary circulatory disturbances potentially may occur after EIS without any accompanying symptoms such as dyspnea, cough, or hemoptysis.
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PMID:[Pulmonary circulatory disturbance following endoscopic injection sclerotherapy]. 836 22

We describe two cases of pneumonia caused by Sho-saiko-to. Patient 1 was a 61-year-old man with type-C liver cirrhosis. About 50 days after starting to take Sho-saiko-to, he complained of fever and diarrhea, and progressive dyspnea developed. Analysis of arterial blood obtained in the emergency room showed severe hypoxemia:, PaO2 26 Torr. A chest radiograph and a CT scan showed bilateral diffuse fine granular and ground-glass opacities predominantly in the upper lung fields. Despite repeated pulse therapy with methylprednisolone and aggressive medical treatment including mechanical ventilation, the patient remained in respiratory distress, which was later complicated by gastrointestinal bleeding. He died on the 45th hospital day. The bronchoalveolar lavage contained abnormally high fluid percents of lymphocytes and neutrophils. Postmortem examination of the lungs revealed alveolar septal thickening, marked hyperplasia of type 2 pneumocytes, and no hyaline membrane formation. Patient 2 was a 68-year-old man. Eighty days after he began taking Sho-saiko-to, he presented with a 4-day history of shortness of breath accompanied by fewer and progressive coughing. On arrival of the hospital, arterial blood gas analysis showed mild hypoxemia (PaO2, 61 Torr) and a chest radiograph revealed bilateral irregular infiltrates in the lower lung fields. Analysis of bronchoalveolar lavage fluid showed an abnormally high percent of lymphocytes (especially CD8 + lymphocytes), and examination of a biopsy specimen revealed exudates of fibrin and neutrophils in the alveolar spaces and patechy intraluminal organization. The response to prednisolone was good and he was discharged on the 40th hospital day in stable condition. Drug lymphocyte stimulation tests of peripheral blood to Sho-saiko-to were positive in both patients. Patients 2 was though to have a typical case of Sho-saiko-to-induced pneumonia, patient 1 was thought to have fulminating variant of this disease.
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PMID:[Two cases of pneumonia caused by Sho-saiko-to]. 896 2

This study is based on a retrospective logistic regression analysis of all human immunodeficiency virus (HIV)-infected patients with Staphylococcus aureus pneumonia (SAP) admitted to the Department of Infectious Diseases, Catholic University, Rome, Italy between January 1986 and December 1994. Nineteen patients with 24 episodes of SAP were enrolled in the study. A control group of 38 HIV-infected patients without pneumonia was included. The attack rate of SAP was 8.31/1000 HIV-related hospital admissions and the frequency, out of the total number of bacterial pneumonia observed in the study period, was 16% (24 of 154 patients). The large majority of SAP was community acquired. On the univariate analysis, intravenous drug abuse (IVDA) (P = 0.02), history of previous Pneumocystis carinii pneumonia (PCP) (P = 0.03) and cirrhosis (P = 0.03) were significant risk factors for SAP. In addition, IVDA and previous PCP were independent risk factors on multivariate analysis. All patients presented with fever associated with cough (74%), chest pain (26%) or shortness of breath (37%). Chest X-ray documented lobar pneumonia (78%), predominantly in the lower lobes, consolidation with cavitation (11%), and interstitial-nodular infiltrates (11%). Pleural effusion was present in 31% of patients. The response to therapy was favourable in 79% of patients. Recurrence occurred in 26% and death occurred in 21% of patients. Death was significantly associated with the low level (< 50 mm-3) of circulating T CD4+ cells (P = 0.03) and the recurrence of pneumonia (P = 0.03). In conclusion, the present study indicates that S. aureus is an important aetiologic agent of bacterial pneumonia in HIV-infected patients, especially if they are drug abusers with previous PCP.
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PMID:Predictors of Staphylococcus aureus pneumonia associated with human immunodeficiency virus infection. 898 27


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