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)

The hypoxaemia associated with hepatic cirrhosis is classically attributed to an intrapulmonary shunt caused by small vascular abnormalities. Severe hypoxaemia (47 mmHg) associated with dyspnoea, cyanosis and clubbing was observed in a 57-year old man who presented with cirrhosis of the liver. At contrast echocardiography, a right-to-left shunt was demonstrated by the appearance of microcavities in the left atrium and ventricle after peripheral intravenous injection of the contrast medium. The intrapulmonary location of the shunt was determined by a 4 cardiac cycles interval between the arrival of the microcavities in the right heart and their appearance in the left heart. The right-to-left shunt was confirmed by the pure oxygen ventilation test and by pulmonary perfusion scintigraphy with radiolabelled albumin microaggregates. Pulmonary angiography proved normal. Thus, contrast echocardiography is capable of diagnosing right-to-left shunts associated with hepatic cirrhosis and to demonstrate their intrapulmonary location.
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PMID:[Intrapulmonary right-left shunt associated with liver cirrhosis: diagnosis by contrast echocardiography]. 313 12

Two autopsy cases of pulmonary hypertension (PH) associated with liver cirrhosis are presented. Both patients were hepatitis B (HB) virus carriers and suffered from type B cirrhosis during the clinical course. The first patient was a 52-year-old male with type B cirrhosis. He died of hepatic encephalopathy but did not have any specific symptoms for PH except abnormal laboratory findings. Chest roentgenograms displayed prominence of the central pulmonary artery. Cardiac catheterization indicated marked increment of pulmonary arterial pressure. Autopsy revealed dilatation and sclerosis of the main pulmonary artery and right ventricular hypertrophy. Microscopically, the pulmonary arteries showed intimal fibrosis, medial hypertrophy, and plexiform lesions throughout the lungs. The second patient, a 15-year-old boy, had PH with juvenile liver cirrhosis which had existed for 8 years prior to the onset of PH. He complained of severe dyspnea and dizziness before death. Electrocardiogram indicated right ventricular hypertrophy. Autopsy disclosed cardiomegaly, type B cirrhosis and sclerotic pulmonary arteries. Grade VI pulmonary plexogenic arteriopathy including plexiform lesions and necrotizing arteritis was observed. HBsAg was detected in both the hepatocytes and the pulmonary arterial walls. We discuss the possible relationship between persistent HB viral infection and PH with liver cirrhosis.
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PMID:Pulmonary hypertension in hepatitis B virus carriers. 344 51

An autopsy case of hypertrophic obstructive cardiomyopathy with extensive myocardial fibrosis is reported in a 43-year-old male. His mother died suddenly at 55. At the age of 39 the patient felt fatigue and feverish sensation followed by dyspnea and palpitation on exertion. He responded to beta-blocker and was discharged on the 51st hospital day. He died suddenly during his work three years and one month after discharge. The heart weighs 700 g. The thickness of the ventricular septum measures up to 3.2 cm, and that of the left ventricular posterior wall 2.2 cm. Subaortic endocardium is moderately thickened. Many patchy fibroses of various sizes and broad linear fibroses are mainly observed in the ventricular septum and in the left ventricular free wall. Microscopic examination shows severe fascicular disarray of hypertrophied myocardial fibers in the ventricular septum and in a part of the left ventricular anterior wall. Pericardial fibrosis, granulation tissue with many capillaries, and slight lymphocytic infiltrate are also noted. These findings suggest that the patient have both congenital hypertrophic cardiomyopathy and myocarditis. There are following possibilities as regards the relation between the two: first, haphazard association of cardiomyopathy with myocarditis; secondly, myocarditis triggered the onset or progression, or both, of cardiomyopathy. He also had liver cirrhosis, probably alcoholic, which appears to accelerate the progression of myocardial disarray and fibrosis.
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PMID:[Hypertrophic obstructive cardiomyopathy with extensive myocardial fibrosis: case report with autopsy]. 403

Five patients had hepatocellular carcinoma growing into the right atrium. Clinically, all patients had edema in the legs, venous dilatation in the abdominal wall, ascites, and dyspnea. Paroxysmal aggravation of dyspnea and its alleviation by a left decubitus position were noted in three patients. Three patients developed shock after a change in posture. A gallop rhythm in the cardiac murmur was detected in two. Pathologically, all livers had hepatocellular carcinoma and macronodular cirrhosis. At autopsy, a tumor thrombus was found that completely occluded the right hepatic vein and extended into the inferior vena cava and right atrium, partially occluding the inferior vena cava. Antemortem diagnosis of right atrial tumor thrombi in patients with primary hepatocellular carcinoma is difficult, but the condition should be suspected when dyspnea, abnormal cardiac sounds, and shock develop.
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PMID:Growth of hepatocellular carcinoma into the right atrium. Report of five cases. 631 61

alpha 1-Antitrypsin, the major serum protease inhibitor, is a glycoprotein synthesized in the liver. Severe deficiency results in protease-antiprotease imbalance, which predisposes to severe emphysema at a young age. Reduced serum levels reflect inadequate release of alpha 1-antitrypsin by the liver, which may be caused by specific defects in biosynthesis. The deficiency is inherited, with multiple codominant alleles at a single autosomal locus. Homozygous individuals, with severely reduced alpha 1-antitrypsin levels, have dyspnea, pulmonary function abnormalities, and respiratory disability from emphysema, usually in the fifth decade of life, with smokers being affected one decade earlier. Heterozygous individuals have intermediate alpha 1-antitrypsin levels and a more benign clinical course. Heterozygous smokers may have mild pulmonary function abnormalities, but these are of uncertain clinical significance. Hepatic involvement with transient neonatal hepatitis and cirrhosis with subsequent liver failure in adulthood represent the major extrapulmonary manifestations, occurring in 10% of homozygous individuals.
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PMID:alpha 1-Antitrypsin deficiency. 632 84

The finding of a rise in VD/VT or failure of VD/VT to fall during exercise has been proposed as a useful noninvasive indicator of pulmonary vaso-occlusive disease in patients with unexplained dyspnea. However, we previously reported a normal fall in VD/VT during exercise in patients with pulmonary hypertension at rest and/or during exercise due to collagen vascular disease. To investigate further the relationship between pulmonary vascular abnormalities and VD/VT responses to exercise, we studied 4 subjects with severe hypoxemia due to cirrhosis or diffuse telangiectasia. We found an abnormal VD/VT response to exercise in these subjects despite normal pulmonary hemodynamics which effectively excluded hemodynamically significant pulmonary vascular obstruction. These findings provide further support for the lack of utility of the VD/VT measurement at rest and during exercise as a screening method for detecting pulmonary vaso-occlusive disease, since the VD/VT cannot only fall appropriately during exercise in patients with pulmonary hypertension but can also remain unchanged or rise during exercise in patients with normal pulmonary hemodynamics.
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PMID:Abnormal responses of wasted ventilation fraction (VD/VT) during exercise in patients with pulmonary vascular abnormalities. 682 5

Chronic liver disease is well known to be associated with pulmonary abnormalities. Hypoxemia, clubbing, cyanosis and hyperventilation are common. The hypoxemia in cirrhotic patients has several causes: diffuse shunts due to intrapulmonary arteriolar vasodilatation, impaired hypoxic vasoconstriction, impaired matching of ventilation to perfusion, pleural effusions and diaphragmatic dysfunction. Because of gravity, shifting of blood to the dilated precapillary beds of the lung bases results in an increased hypoxemic dyspnea when the patient is in the upright position, also known as orthodeoxia and platypnea. It has only been described in 5% of the cirrhotic patients and has not been described in a Belgian refereed journal (Medline literature search 1983-Aug 1993). It should be considered in the initial differential diagnosis of hypoxemia in patients with liver cirrhosis and dyspnea. Measuring arterial blood gases in the lying and upright position can prevent further invasive investigations, and whole body nuclide scan with technetium-99m macroaggregated albumin can confirm the diagnosis. Standard therapy with spironolactone (Aldactone) can worsen the condition and we found no additional benefit of beta-antagonists (propranolol/Inderal) in the reduction of the shunt fraction, probably because the main reason for the shunting is precapillary vasodilatation. Since there are no anatomical porto-pulmonary shunts, surgery is also inappropriate. The only therapy consists of oxygen supplements and low dose diuretics in patients with edema.
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PMID:Orthodeoxia and platypnea in liver cirrhosis: effects of propranolol. 751 28

Pulmonary arteriovenous shunting (PAVS) with hypoxemia is a severe complication of cirrhosis that may regress after liver transplantation. We report PAVS in 25 children with cirrhosis and in 1 with portal vein obstruction; proof of shunting was obtained by technetium Tc 99m microaggregated albumin pulmonary scanning or a high alveoloarterial O2 gradient or both. Cyanosis or dyspnea or both occurred at ages ranging from 6 months to 14 years, earlier in children with biliary atresia and polysplenia syndrome (p < 0.01). Mean arterial oxygen tension (PaO2) was 57 mm Hg (range, 42 to 81 mm Hg) during breathing of 21% O2 and 367 mm Hg (range, 179 to 535 mm Hg) in 100% O2. Cardiac index was always raised, significantly more in children with biliary atresia and polysplenia syndrome (p < 0.01). Seven untreated children died 3 months to 8 years after the diagnosis of PAVS. Eleven underwent liver transplantation: seven are alive (follow-up, 1 to 4 years) and have no signs of PAVS. The PaO2 value during breathing of 100% O2 was > 300 mm Hg in the survivors and < 200 mm Hg in the four nonsurvivors (p < 0.01). These results indicate (1) that PAVS can occur at any age in children with portal hypertension, and that the risk is highest and earliest in children with biliary atresia and polysplenia syndrome, (2) that early liver transplantation allows regression of PAVS, and (3) that the prognosis may in part be related to the level of PaO2 while the patient is breathing 100% O2. The results indicate that systematic screening for PAVS should be part of the examination of these children.
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PMID:Pulmonary arteriovenous shunting in children with liver disease. 769 35

A 53-year-old man with alpha-1-antitrypsin deficiency had an 8-year history of progressive dyspnoea and two episodes of bleeding oesophageal varices with liver decompensation. After the diagnosis of terminal pulmonary emphysema (Fig. 1) and liver cirrhosis with progressive liver failure was made, he was accepted for combined lung and liver transplantation. METHODS. Anaesthesia was induced with thiopentone and fentanyl and maintained with fentanyl, midazolam, and isoflurane. After relaxation with succinylcholine, the patient's trachea was intubated with a left endobronchial double-lumen tube. Haemodynamic monitoring included arterial, central-venous, pulmonary-artery, and capillary-wedge pressures and cardiac output measurement. Ventilatory monitoring consisted of pulse oximetry, side-stream spirometry, and continuous measurement of arterial and mixed-venous blood oxygen saturation with fibreoptic catheters. A left single-lung transplantation was performed under one-lung ventilation without cardiopulmonary bypass. Prostacyclin was infused to reduce pulmonary vascular resistance. The transplant was ventilated separately with 50% oxygen and positive end-expiratory pressure of 8-10 cm H2O, and then liver transplantation was carried out. The institution of veno-venous bypass during the anhepatic phase failed because of portal-vein and axillary-vein thrombi. RESULTS. Total operation time was 6 h 30 min. Clamping of the left pulmonary artery lasted 45 min and the duration of the anhepatic phase was 92 min. Ventilation and oxygenation during lung transplantation caused no problems (Table 1). Clamping of the left pulmonary artery caused a slight increase in pulmonary vascular resistance (104 to 124 dyn.s.cm-5) and mean pulmonary artery pressure (25 to 27 mm Hg) without a decrease in cardiac index (Table 2). During the anhepatic phase with exclusion of the portal vein and inferior vena cava, a marked decrease in cardiac index (-27.2%) was seen (Table 4). The operation required substitution with 10 units packed red blood cells, 12 units fresh frozen plasma, and 5 platelet concentrates. The post-operative course showed normal liver graft function (Table 5). Acute pulmonary rejection on the 7th day was treated successfully with methylprednisolone. The patient's trachea has extubated 10 days after transplantation and he was discharged from the intensive care unit 2 weeks later. CONCLUSION. The management of this combined lung and liver transplantation was performed according to the experience with isolated lung and liver transplants in our hospital. Aggressive haemodynamic and ventilatory monitoring, including systemic and pulmonary arterial fibreoptic catheters, seems of particular importance in such high-risk procedures.
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PMID:[Combined lung and liver transplantation. Anesthesiologic management]. 804 61

A 37 year old patient with chronic active hepatitis progressing to cirrhosis presented with increasing breathlessness and was found to be hypoxic with finger clubbing. A progressive exercise study with measurement of oxygen saturation (SaO2) showed abnormally high ventilation and desaturation to 81% at 100 W. Serial studies over nearly two years showed, first, deterioration, then improvement with lower ventilation and higher saturation levels at all work loads. This could not be correlated with any change in treatment with azathioprine, prednisolone, or propranolol.
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PMID:Spontaneous improvement in a patient with the hepatopulmonary syndrome assessed by serial exercise tests. 806 74


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