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Query: UMLS:C0023890 (cirrhosis)
 42,195 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Twenty-six patients, mean age 20.5 years (range 11-33 years) at last assessment or death, attended an adult cystic fibrosis clinic between 1975 and 1983. Twenty-one presented in infancy, and 5 later (3-17 years). Most morbidity was due to recurrent respiratory infection and 5 of the 7 deaths were from respiratory failure. Cor pulmonale occurred in 4 patients, pneumothorax in 3 and severe haemoptysis necessitating lobectomy in 2. Declining spirometric values and persistent isolation of Pseudomonas aeruginosa from sputum samples were associated with a poor prognosis. Minor gastrointestinal symptoms were common (19 patients). Four patients developed intestinal obstruction. Six patients had abnormal liver function tests and one patient died from hepatic cirrhosis. Diabetes was diagnosed in 3 patients and 9 patients experienced joint pains. The prognosis and quality of life for patients with cystic fibrosis appear to be improving, and all but 2 of the patients attending the clinic are at school, university or are employed.
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PMID:Cystic fibrosis--a review of 26 adolescent and adult patients. 393 89

Asphyxiating thoracic dystrophy is an autosomal recessive disorder characterised by an abnormally small thorax, variable shortening of the extremities, and pelvic anomalies. Renal and pancreatic symptoms are found in longer survivors, although most cases die in infancy of respiratory failure. Seven neonatal cases were studied at necropsy. These cases ranged in gestational age from 32 to 40 weeks. One was stillborn and the other six survived from 1 hour to 10 days. Two were sibs born to consanguineous parents. Dwarfing was not pronounced and the extremities were shortened in only one infant who also had polydactyly. All seven showed visceral changes in addition to abnormalities of bone. Endochondral ossification was irregular in sections of femur, vertebra, and rib. Pulmonary hypoplasia was associated with the small thorax typical of this disorder. Periportal fibrosis and bile duct proliferation were seen in sections of liver, and in one case cirrhosis was found. Pancreatic fibrosis was variable. These necropsy findings correlate with later clinical manifestations of the disease and emphasise the multisystem nature of this disorder.
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PMID:Necropsy findings in neonatal asphyxiating thoracic dystrophy. 398 24

Twenty-eight patients with cystic fibrosis, who survived to over 18 years of age are reported. The ages at the time of diagnosis ranged from and two and half months to over 21 years. Manifestations of cystic fibrosis in adults and youngsters are compared and contrasted. A review of the patients surviving shows that steatorrhea is under control. There is no evidence of biliary cirrhosis in any of these patients. Rectal prolapse has not been an important factor. Pseudomonas and Staphylococci were the major infecting organisms causing lung disease. Two-thirds of the patients do not use prophylactic antibiotics and one-third do not practice regular pulmonary care. Two female patients died; one of Pseudomonas lung infection and respiratory failure, after a pregnancy that was carried to term (live baby); one, of respiratory failure, also with severe Pseudomonas respiratory tract infection, two years after a pregnancy that was terminated in the first trimester.
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PMID:Cystic fibrosis. As they grow older. 732 46

Patients with cystic fibrosis who have end-stage respiratory failure and associated liver cirrhosis have been considered poor candidates for lung transplantation because of high morbidity and mortality resulting from hepatic insufficiency after the operation. Since April 1989, our policy has been to combine heart-lung or lung and liver transplantation in this group of patients. Between June 1990 and March 1995, among 25 patients accepted in the program for combined transplantation, nine died awaiting transplantation and 10 underwent one of the following procedures: heart-lung-liver transplantation (n = 5), en bloc double lung-liver transplantation (n = 1), sequential double lung-liver transplantation (n = 3), and bilateral lobar lung transplantation from a split left lung and reduced liver transplantation (n = 1). There were 5 male and 5 female patients. The ages of the recipients ranged from 10 to 24 years. Mean forced expiratory volume in 1 second was 29% and mean forced vital capacity was 35% of predicted values. All patients were infected with resistant Pseudomonas, three with Pseudomonas cepaceia, and two patients had Aspergillus species in addition. All patients had severe cirrhosis with portal hypertension. Four patients had a history of esophageal variceal bleeding and two had had previous portosystemic shunts. The operation was performed as a two-stage procedure, the intrathoracic operation being completed before the abdominal stage was begun. Cardiopulmonary bypass was used in all patients because of poor clinical condition. Immunosuppression consisted of azathioprine, cyclosporine, and prednisone, as for isolated lung transplantation. There were two perioperative deaths, one caused by primary liver failure and the second by early lung dysfunction. For the first 3 months after transplantation pulmonary infection was the most common cause of morbidity. Other complications included tracheal stenosis (n = 1), bronchial stenosis (n = 1), biliary stricture (n = 2), and severe ascites (n = 3). All were successfully treated. Obliterative bronchiolitis developed in three patients. This was stabilized with FK 506 in two patients; the other patient underwent retransplantation at 38 months but eventually died of bleeding. Actuarial survival was 70% at 1 year and remained unchanged at 3 years. Significant functional improvement was observed in all survivors. For patients who have chronic respiratory failure with advanced cirrhosis, lung transplantation combined with liver transplantation can be performed with a satisfactory outcome.
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PMID:Combined lung and liver transplantation in patients with cystic fibrosis. A 4 1/2-year experience. 747 93

Because the lifespan of patients with cystic fibrosis is now longer, both pediatricians and adult care physicians are involved in the health care strategy. Respiratory manifestations occur due to bronchial dilatation and chronic bronchial infection, mainly due to Staphylococcus aureus and Pseudomonas. Episodes of adult infection are frequent and death usually results from respiratory failure. Characteristically, the disease also involves exocrine pancreas insufficiency. Other intestinal tract manifestations include meconial ileus and liver disease which may reach the stage of biliary cirrhosis. Nutritional disorders are frequent. Clinically there are respiratory and digestive tract disorders, pansinusitis and frequent nasal polyposis, sometimes associated with diabetes mellitus or joint pain. Male sterility results from bilateral agenesia of the vas deferens and in the female, fertility is decreased although pregnancy is possible. Clinical presentation suggests the diagnosis which is confirmed by a sweat test and genetic analysis. Care should be provided by a centre specialized in cystic fibrosis. The main treatments rely on respiratory physical therapy, antibiotics and gastroprotected pancrease extracts.
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PMID:[Mucoviscidosis: in children and adults]. 756 75

Indications for liver transplant in acute fulminating hepatitis (AFH) are predominantly affected by the high mortality of this spontaneous evolution (80-100%). At present patients with AFH have priority for transplant since they form part of the 0 emergency group according to the National Transplant Organisation. During the period between 1986 and the end of February 1992, a total of 254 liver transplants were performed in 202 patients (52 retransplants). In 26 patients (12.8%) (16 females and 10 males) the indication was fulminating acute hepatitis. Etiology was unknown in 20 patients, secondary to hepatitis B in 4 and to hepatitis A in 1, and was caused by isonazide ingestion in 1 case. The age limits were 3-60 years (X = 31.5 years). An isogroup graft was performed in 16 patients (61.5%), compatible in 3 (11.6%) and incompatible in 7 (26.9%). Due to anthropometric differences, a partial graft was used in 7 patients (26.9%); in 2 of the latter the graft was taken from the same donor ("split-liver"). Placement was always orthotopic with resection of the retrohepatic vena cava in 25 patients and its preservation in 1 (left lobe of split-liver). Peroperative (30 days) mortality was 23% (6/26); 2 due to cerebral death, 2 due to sepsis, 1 due to multisystemic insufficiency (MSI) and 1 due to acute pancreatitis. Four patients (15.3%) died some time after transplant; 1 after 5 months due to broncho-pulmonary complications, 1 after 7 months due to subacute hepatitis, 1 after 3 months due to respiratory failure and the last after 5 months due to anoxic encephalopathy and lung infection. Ten patients (39.4%) were re-transplanted; 4 following chronic rejection, 4 due to primary graft no function, 1 due to arterial thrombosis and 1 due to recurrent hepatitis (with cirrhosis). Two of the latter patients died intraoperatively due to coagulopathy and hemorrhage, and 3 following surgery (1 due to sepsis, 1 due to respiratory complications and 1 due to respiratory insufficiency). Two patients underwent a second re-transplant (1 due to chronic rejection and 1 due to recurrent hepatitis) and of these 1 died peroperatively due to sepsis and MSF. Overall mortality was therefore 61.5% (16/26) and the actuarial survival rate of 17 patients (10 living + 7 postoperative deaths) was 68% at 12 months and 52.9% at 36 months. Even if peroperative mortality is relatively high, liver transplant is currently the elective treatment for fulminating acute hepatitis.
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PMID:[The treatment of acute liver failure due to fulminating hepatitis by total or partial orthotopic liver transplantation. The clinical results]. 832 33

This article reviews the basic pathophysiologic mechanisms underlying the abnormal pulmonary gas exchange often seen in patients with cirrhosis. To summarize, the following keypoints seem appropriate: (1) Patients with cirrhosis have a low pulmonary vascular tone characterized by a poor or absent hypoxic pressor response. This results in a marked dilation of the pulmonary vasculature. (2) This abnormal pulmonary vascular tone, independently of airway disease, causes VA/Q mismatch and mild to moderate hypoxemia. Yet, as liver disease progresses and hepatocellular function deteriorates, more severe degrees of intrapulmonary shunt emerge and, probably, O2 diffusion limitation ensues, causing severe respiratory failure (see Table 1). (3) At rest, the high cardiac output and minute ventilation of cirrhosis minimize the degree of arterial hypoxemia that otherwise would be expected from the observed degree of both VA/Q inequality and intrapulmonary shunt. During exercise, the relative "normalization' (with respect to metabolic demands) of the hemodynamic and ventilatory status of the patient explains the fall in PaO2. (4) A clear pathogenic mechanism of these pathophysiologic abnormalities is still lacking, although available evidence suggests that both the liver and the endothelial cells may play a pivotal role in the regulation of the pulmonary vascular tone in these patients. (5) To date, no pharmacologic intervention has been effective in treating hypoxemia in these patients. Yet liver transplantation helps in most of them. This observation reinforces the functional nature of the gas exchange abnormalities of cirrhosis.
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PMID:Mechanisms of gas exchange impairment in patients with liver cirrhosis. 866 90

Perioperative management of patients with liver cirrhosis undergoing open heart surgery (3 males and 1 female, mean age 57.3 years) was evaluated. All 4 patients had NYHA class III acquired valvular heart disease accompanied by severe tricuspid regurgitation, and the operation was a redo operation in 3 of them. Preoperatively, the total serum bilirubin was 1.7 mg/dl, ICGR15 24.3 +/- 1.7%, and blood platelet count was 83,000 +/- 19,000/m3. The postoperative course was uneventful in 2 patients who maintained good cardiac function and did not require homologous blood transfusion. The other two patients who underwent a re-redo operation, sustained a large volume of hemorrhage, and required massive transfusion showed severe postoperative complications (respiratory failure in 1, multiple organ failure in 1). The stage of the disease improved to NYHA class I in all 3 patients who survived the operation. Comprehensive measures including avoidance of massive hemorrhage, preservation of the hepatic blood flow, resolution of hepatic congestion, and prevention of complications such as digestive tract bleeding, infections, and renal failure are considered to be needed for prevention of postoperative liver failure. Especially, sufficient surgical hemostasis and autologous platelet rich plasma were useful for prevention of massive hemorrhage.
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PMID:[Perioperative management in patients with liver cirrhosis undergoing open heart surgery]. 891 65

Ascites formation in cirrhosis results from the interaction of "local" and "systemic" pathogenetic factors. Among local factors, post-sinusoidal portal hypertension plays the most important role, while the main systemic event is renal sodium retention. The latter precedes ascites formation and leads to plasma volume expansion. Many factors are responsible for renal sodium retention, but secondary hyperaldosteronism and reduced renal perfusion prevail. The events promoting the onset of sodium retention are far from being clarified. However, there is evidence that the main afferent mechanism is represented by the "effective" hypovolemia secondary to splanchnic venous vasodilation, due to portal hypertension, and reduced peripheral vascular resistance, which becomes evident in the advanced stage of the disease. Systemic hemodynamic abnormalities are responsible for the progressive reduction of renal perfusion, which ends in the hepatorenal syndrome. The appearance of ascites is a crucial event in the natural history of cirrhosis and has a negative prognostic meaning. In fact, ascites appears when pathogenetic factors, such as liver function abnormalities, portal and systemic hemodynamics, and renal function, have reached a critical threshold severity. Second, ascites itself induces additional complications, closely linked to its presence, such as spontaneous bacterial peritonitis, restrictive respiratory failure, or rupture of abdominal hernias. Finally, ascites implies pharmacological or invasive treatment which can lead to further morbidity or even to death.
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PMID:[Natural course and physiopathology of ascites in the cirrhotic patient]. 900 18

Acute cholecystitis is a common disease which may carry the risk of complications, including empyema, perforation, abscess, peritonitis and sepsis. Percutaneous transhepatic drainage of the gallbladder (PTGBD) with antibiotics can provide prompt decompression of gallbladder in acute cholecystitis and interrupt the natural history of the disease effectively. From July 1986 to June 1996, 154 patients with acute cholecystitis were reviewed retrospectively in Kaohsiung Medical College Hospital. The chief symptoms and signs were pain (98.1%), fever (57.1%) and jaundice (37.7%). WBC count more than 10,000 was noted in 116 (75.3%) patients. Associated diseases included empyema: 42 (27.3%), septic shock: 14 (9.1%), diabetes mellitus: 13 (8.4%), pancreatitis: 10 (6.5%), perforation: 7 (4.5%), liver cirrhosis: 6 (3.9%) and respiratory failure: 1 (0.6%). All of them underwent ultrasound-guided PTGBD immediately after the diagnosis was established. The symptoms and signs disappeared soon after this procedure. Bacterial culture was found positive in 104 (67.5%) of 154 patients in which Escherichia coli (51.9%) was the most common organism, followed by Klebsiella pneumonia (20.2%). After acute stage, 138 patients obtained the cholangiography via PTGBD tube. Gallbladder stones were only noted in 56 (40.6%) patients, gallbladder stone concomitant with common bile duct stone in 26 (18.8%), cystic duct obstruction in 25 (18.1%), acalculous cholecystitis in 21 (15.2%), gallbladder perforation in 1 (0.7%), choledochocyst in 1 (0.7%), and cholecystocolonic fistula in 1 (0.7%). There were 135 patients to undergo surgery after the clinical condition was stable. The operative findings included gallbladder stones only in 88 (65.2%), gallbladder stone concomitant with common bile duct stone in 34 (25.2%), acalculous cholecystitis in 13 (9.6%), choledochocyst in 1 (0.7%), and cholecysto-colonic fistula in 1 (0.7%). The postoperative complications included wound infection 8 (5.9%), UGI bleeding 3 (2.2%), acute renal failure 1 (0.7%) and acute respiratory failure 1 (0.7%). The postoperative mortality rate was 0.7% (1/135), which was much lower than those of previous reports, which not undergoing PTGBD initially. It led us to conclude that PTGBD, as an initial preoperative modality to treat acute cholecystitis, is effective in decreasing postoperative morbidity and mortality.
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PMID:Ultrasound-guided percutaneous transhepatic drainage of gallbladder followed by cholecystectomy for acute cholecystitis--10 years' experience. 951 85


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