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)

Disorders of the central nervous system were found in 150 rats five monthds after en-to-side-porta-caval anastomosis consisting of vacuolisated cytoplasma of gangliacells and reactions of the glia comparable with the Alzheimer-glia-type II. Since these disorders are consistent with those in patients with liver cirrhosis but induced in these experiments by a porta-caval anastomosis alone, they are proposedly independent from liver disease itself. It is discussed whether disorders in glucose homeostai may be of pathogenetic relevance. Atrophy of tests, found in the late postoperative phase and erosions or ulcers of gastric mucosa as well as nephrolithiasis with hydronephrosis as consequence, the latter occuring independently from the time after operations, are proposedly due to the porta-caval anastomosis, too.
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PMID:[Morphological investigations of extrahepatic disorders after porta-caval end-to-side-anastomosis in rats (author's transl)]. 89 4

Between 1976 and 1987, 43 patients underwent reversal of jejunoileal bypass operations because of metabolic complications of the operation. Electrolyte imbalance, malnutrition, and diarrhea (16 patients); cirrhosis (9); nephrolithiasis (9); arthritis (7); and pathologic fractures (1) were the primary indications for reconstruction. Many patients had multiple complications of the jejunoileal bypass operation. Twenty-nine patients underwent gastroplasty at the time of reversal and 14 did not. Seventy three +/- 5 months after reversal, patients with a gastroplasty weighed significantly less than patients without a gastroplasty. Patients with electrolyte imbalance, malnutrition, and diarrhea were all improved after reconstruction. Two patients with cirrhosis died of liver failure after reconstruction; the distinguishing preoperative characteristic was ascites. Postoperative interval liver biopsies indicated improvement in histologic appearance in four patients and no change in three. Nephrolithiasis improved or disappeared in all patients after reconstruction, whereas arthritis improved in 5 of 7 patients. Gastroplasty produced no benefit in alleviation of metabolic complications of jejunoileal bypass operations. Although the survival rate in these patients at last follow-up was 95 percent, 28 percent were incapacitated. Simultaneous gastroplasty performed at the time of reversal significantly decreases body weight when compared with patients undergoing reversal without a gastroplasty.
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PMID:Long-term outcome of reversal of small intestinal bypass operations. 229 88

Serious late sequelae including chronic active hepatitis, cirrhosis, massive necrosis, and hepatocellular carcinoma may develop in patients infected with hepatitis B virus. Clinical and epidemiologic risk factors for such complications have not been identified. To examine this question, the clinical and pathologic features of the 60 patients with documented hepatitis B virus infection who underwent postmortem examination at the Johns Hopkins Hospital were reviewed. In 27 patients (45 percent), the outcome of hepatitis B infection was nonlethal, i.e., at autopsy, the liver showed either no histopathologic lesions attributable to hepatitis B infection, acute viral hepatitis, or chronic persistent hepatitis. Lethal outcomes of hepatitis B infection, i.e., chronic active hepatitis, cirrhosis, hepatocellular carcinoma, and/or massive hepatic necrosis, were present in the remaining 33 (55 percent) patients. Chronic active hepatitis was observed more frequently in whites (p less than 0.05) and males (p less than 0.05). Lethal outcomes of hepatitis B infection were correlated with recent or concomitant exposure to known hepatotoxic agents (p less than 0.05), including heavy ethanol abuse, isoniazide, hydrocarbon exposure, methyldopa, and the chemotherapeutic agents busulfan and methotrexate. In addition, a lethal outcome of hepatitis B virus, particularly massive hepatic necrosis, was positively correlated with a history of nephrolithiasis (p less than 0.005). Recent, concomitant treatment with immunosuppressive agents, given in 24 patients (40 percent), was correlated with the absence of lethal sequelae (p less than 0.005). These data suggest that patients with recent exposure to hepatotoxic agents have an increased risk of lethal sequelae following hepatitis B infection. Furthermore, the results suggest that immunosuppressive or antiinflammatory therapy may be beneficial in reducing morbidity and mortality from hepatitis B virus infection.
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PMID:Risk factors for development of lethal sequelae after hepatitis B virus infection in humans. 647 89

The modern, comprehensive care of patients with hemophilia requires an awareness that complications other than those caused by acute hemorrhage can occur. The use of newer, more potent plasma concentrates has been accompanied by an increased incidence of liver disease in transfusion-requiring hemophiliacs. The progression to chronic active hepatitis and cirrhosis are particularly ominous developments in these patients. There is also a high incidence of urinary tract abnormalities in hemophiliacs, though the long-term consequences of these abnormalities are unknown. Furthermore, it must be remembered that urinary tract disorders unrelated to hemorrhage, such as nephrolithiasis, tumors, and nephritis, can occur in patients with hemophilia and may be mistaken for hemorrhage. Finally, hypertension occurs more frequently in patients with hemophilia than in the general population and may in part contribute to the occurrence of bleeding within the central nervous system. Methods for evaluating and treating these various disorders are discussed. Greater awareness of these potentially treatable medical complications will improve further the quality of care in hemophilia.
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PMID:Medical complications of hemophilia. 676 70

To evaluate the results of jejunoileal bypass for morbid obesity, we studied 100 patients with intact bypasses an average of more than five years after surgery. Mean weight loss at five years was 102.7 lb (46.6 kg) (33 per cent). Although nearly half the patients regained some weight between one and five years after surgery, only 17 per cent regained 20 lb (9 kg) or more. Medical benefits (such as improved glucose tolerance and lowered blood pressure) were maintained at five years, but side effects and complications continued to occur in the late postoperative period. Diarrhea (more than three stools per day) persisted in 58 per cent of the patients, and electrolyte disturbances occurred in over a third. Diminished levels of B12 or folate or both were present in 88 per cent. Twenty-one per cent of the patients had nephrolithiasis, and 20 per cent of those who were at risk required cholecystectomy. Progressive hepatic structural abnormalities occurred in 29 per cent of the patients, and there was a 7 per cent incidence of cirrhosis. Although 81 per cent of the patients had satisfactory results at five years, side effects and complications continued to occur, mandating careful follow-up indefinitely. The risk-to-benefit ratio at five years after surgery seems acceptable, but the continued untoward effects of the bypass in the late postoperative period have led us to abandon this procedure in favor of gastric bypass. Only continued longitudinal follow-up will determine whether on balance jejunoileal bypass represents such a serious long-term health hazard that prophylactic restoration of intestinal continuity is indicated.
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PMID:Jejunoileal bypass for morbid obesity. Late follow-up in 100 cases. 683 18

A 52-year-old oligophrenic man hospitalized for esophageal hemorrhage had histologically proven liver cirrhosis and died from massive rehemorrhage. As a neonate he had survived severe jaundice, had had delayed psychomotor development and remained severely retarded. At age 15 years, bilateral cataracts had been excised and from 18 to 25 years he had had occasional grand mal seizures. The triad oligophrenia, liver cirrhosis and cataracts, prompted suspicion of galactosemia. Deficiency of galactose-1-phosphate uridyltransferase was demonstrated in blood and post mortem tissue. At autopsy, liver cirrhosis and esophageal varices were confirmed and unilateral chronic pyelonephritis, bilateral nephrolithiasis and testicular atrophy were found. There was not brain pathology. The patient appeared to be the oldest nondiagnosed galactosemic and the first male patient in whom hypogonadism was documented.
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PMID:[Decompensated liver cirrhosis caused by galactosemia in a 52-year-old man]. 745 52

During the past six years, we have treated eight patients with cystic fibrosis (CF) for nephrolithiasis. In seven patients, the stones were comprised of calcium oxalate. Another six patients had calcium oxalate crystalluria. In our CF population of 140 patients, this represents a cumulative incidence of calcium oxalate nephrolithiasis of 5.7 percent and an additional 4.2 percent incidence of crystalluria. Experience with these patients is reviewed. Pancreatic insufficiency was universally associated with nephrolithiasis or crystalluria. Diabetes and cirrhosis were also common. Predisposing factors and potential mechanisms of stone disease in pancreatic insufficient CF patients are discussed, focusing on the relationship between fat malabsorption in CF to oxalate metabolism.
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PMID:Cystic fibrosis and calcium oxalate nephrolithiasis. 927 85

The authors present a report of 14 patients with the syndrome of portal hypertension without liver cirrhosis and with recurring esophagogastric bleedings. The cause of the alterations in portal hemodynamics remains unknown. Operative treatments (splenorenal shunts in 9 cases, 3 splenectomies and 1 ligation of the splenic artery) were successful. Two patients in whom splenectomy had been performed in combination with omentohepatopexy died 6 and 10 years after operation due to recurrent hemorrhages. The other patients did not have recurrent bleedings, but in 6 patients 6-10 years after splenorenal shunts there appeared other diseases (encephalopathy, nephrolithiasis, arterial hypertension, duodenal ulcer). The authors consider that indications for shunting operations for idiopathic portal hypertension, especially when using renal veins, should be determined more carefully, phlebosclerotic therapy and transsection of the esophagus being recommended as alternative interventions.
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PMID:[Idiopathic portal hypertension]. 1125 10

The paleopathological study of 40 Italian Renaissance mummies has allowed us to perform about 20 diagnoses, of which 5 concern infectious (smallpox, hepatitis, condyloma, syphilis and pneumonia), 4 metabolic (obesity, atherosclerosis, gallstones and uric acid nephrolithiasis), 2 articular (DISH and rheumatoid arthritis) and 2 neoplastic (skin apithelioma and colon adenocarcinoma) diseases. The mummy of an anonymous child, dated back to the 16th century (C14=1569 +/- 60), presented a diffuse vesiculo-pustular exanthema. Macroscopic aspects and regional distribution suggested smallpox, while EM reavealed many egg-shaped, virus-like particles (250 x 50 nm), with a central dense core. Following incubation with anti-smallpox virus antiserum and protein A-gold complex immunostaining, the particles resulted completely covered with protein A-gold. These results clearly show that this Neapolitan child died of a severe form of smallpox some four centuries ago. The mummy of Maria of Aragon, Marquise of Vasto (1503-1568), reavealed on the left arm an oval, cutaneous ulcer (15x10 nm) with linen dressing. Indirect immunofluorescence with anti-treponema pallidum antibody identified a large number of filaments with the morphological characteristics of fluorescent treponemes. EM evidenced typical spirochetes, with axial fibril. These findings clearly demonstrate a treponemal, probably venereal, infection. The mummy of Ferrante I of Aragon, King of Naples (1431-1494), revealed an adenocarcinoma extensively infiltrating the muscles of the small pelvis. A molecular study of the neoplastic tissue evidenced a typical mutation of the K-ras gene codon 12:the normal sequence GGT (glycine) was altered into GAT (aspartic acid). At present this genetic change is the most frequent mutation of the K-ras gene in sporadic colorectal cancer. The alimentary "environment" of the Neapolitan court of the XV century, with its abundance of natural alimentary alkylating agents, well explains this acquired mutation. These and other diseases as, for example, a fatal puerperal complication, a thyroid goiter, a case of Wilson's cirrhosis, some cases of anthracosis and other peculiar traumatic conditions, such as a mortal stab-wound, can elucidate the pathocenosis of the wealthy classes of the Italian Renaissance.
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PMID:Renaissance mummies in Italy. 1162 3

We report herein a domino orthotopic liver transplantation (LT), from a 38-year-old woman undergoing liver-kidney transplantation (LKT) for primary hyperoxaluria type I (PH1) to a recipient with cirrhosis and hepatocellular carcinoma. Delayed onset of PH1 and renal failure and 10% residual alanine-glyoxylate aminotransferase (AGT) activity in domino liver justified its use for domino procedure. The clinical course after LKT was similar to that described in other series, including ours. Renal function started promptly and maintained despite sustained hyperoxaluria from dissolution of oxalotic deposits. Conversely, the domino recipient manifested severe hyperoxaluria and developed nephrolithiasis and renal insufficiency with rapid progression over 2 months. A new LT resulted in slow decrease of oxaluria and improvement of renal function. Therefore, PH1 behaved quite differently in these two patients, leading us to conclude that domino LT using livers from PH1 patients should be considered very carefully, only as a bridge to definitive LT in recipients with critical clinical conditions.
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PMID:Severe course of primary hyperoxaluria and renal failure after domino hepatic transplantation. 1609 18


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