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)

Systemic disease can produce changes in the nails. Perhaps the best known example of this is koilonychia as a sequale of iron deficiency anemia. "Half and half nail" is a type of pseudoleuconychia that can be caused by chronic renal disease, Kawasaki's disease, cirrhosis, and zinc deficiency. It has not been described in patients with Crohn's disease yet. Four male patients with Crohn's disease were observed. None of them had extraintestinal manifestations of Crohn's disease. The average duration of the disease was 5.25 (range, 1-10) years. The nail alterations were defined with a portion of the nail being as much as 15 to 40 percent of normal color distally, whereas the rest of the nail was white. The contrast between the two zones remains sharply demarcated even after constricting venous return. Histologic examination was not performed. Every patient had zinc deficiency but not hypalbuminaemia or sideropenia. After review of relevant literature (MEDLINE, PubMed, etc.), we found that half-and-half nail had not been described in Crohn's disease. This study was designed to highlight the fact that the half-and-half nail, which was thought to be a sign of chronic renal failure, also occurs in patients with Crohn's disease. The relationship of this symptom to clinical activity cannot yet be assessed and has only been observed in four cases.
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PMID:The half-and-half nail: a new sign of Crohn's disease? Report of four cases. 1669 71

Nodular regenerative hyperplasia (NRH) is characterized by a non-cirrhotic micronodular transformation of the liver parenchyma. It is based on the obliteration of small portal veins. Macroregenerative nodules (MRN) develop in areas of favourable blood flow in otherwise hypoperfused liver tissue. Hypoperfusion is caused by obliteration of liver veins and/or large portal veins with the subsequent atrophy or extinction of parenchyma. The hyperperfused and sometimes rapidly growing MRN might simulate a malignant tumor in CT and MRT. Morphologically, MRN resemble FNH. In contrast to hepatocellular adenoma, they show a more or less nodular architecture with fibrous septa and ductular structures. NRH and cases of MRN without cirrhosis can indicate an extrahepatic/systemic disease causing altered liver perfusion. MRN in liver cirrhosis must be differentiated from dysplastic nodules and highly differentiated hepatocellular carcinoma by cytological and microarchitectural criteria. Focal nodular hyperplasia (FNH) can imitate liver cirrhosis, steatohepatitis, cholangitis or chronic hepatitis, if biopsy material does not include normal perilesional liver tissue. Telangiectatic FNH might resemble classic hepatocellular adenoma. Neoductular structures and septation argue for this rare subtype of FNH. Neoductular transformation of hypoperfused liver parenchyma might imitate cholangioma or cholangiocarcinoma.
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PMID:[Nodular lesions of liver parenchyma caused by pathological vascularisation/perfusion]. 1677 11

The etiology of granulomatous hepatitis is extremely extensive for which the granulomatous reaction induced hepatic inflammation is characteristic. In the majority of cases it is part of a systemic disease but sometimes there is primer hepatic lesion in the background. The 21 year-old Caucasian female patient was taken to hospital having flu-like symptoms and icterus. While searching for the cause of her cholestatic hepatocellular lesion infections, tumors, storage diseases were excluded. However the root cause could not be identified. The histology showed granulomatous reaction, inflammation, intrahepatic bile duct proliferation and micronodular cirrhosis in the liver tissue. Taken it into consideration together with the the clinical picture and the laboratory findings primer sclerosing cholangitis was rendered probable. Her state deteriorated and even the necessity of liver transplantation emerged. The patient was treated with ursodeoxycholic acid which resulted in remission lasting for years now. Two years after the onset of the hepatic disease pancolitis ulcerosa with affection of the ileum terminalis was found in the patient which supports the diagnosis of primary sclerosing cholangitis.
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PMID:[Granulomatous hepatitis successfully treated with ursodeoxycholic acid]. 1698 20

Hemachromatosis is a hereditary condition, producing progressive iron overload as a result of the mutation in proteins that regulate intestinal iron absorption. It is a systemic disease with several manifestations including cirrhosis, diabetes mellitus, cardiomyopathy, joint disease and a proportion of asymptomatic patients. When it is diagnosed and treatment with phlebotomies is initiated before any organ damage is developed, the prognosis is very good, with normal survival free of manifestations. This condition is common in European populations. We report the case of a Peruvian patient of European ancestry who is asymptomatic, but has high levels of aminotransferases and elevated iron markers. Genetic testing confirmed the patient's diagnosis of hereditary hemachromatosis.
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PMID:[Hereditary hemachromatosis: clinical case report and literature review]. 1705 26

Diuretics are pharmacological agents that increase natriuresis through inhibition of tubular re-absorption of sodium. The mechanisms and site of this inhibition differ with each drug class, accounting for their additive effects on natriuresis increase and their hydroelectrolytic side effects. The response to a given diuretic dose depends on the diuretic concentration on the urine at its action site. This concentration may be decreased by pharmacokinetic factors such as encountered in renal insufficiency or in nephrotic syndrome. These resistance mechanisms of diuretics may be corrected by dose increase, previous diuretic fixation on albumin or warfarin administration. Once these mechanisms are opposed, the diuretic concentration for maximal efficacy is reached at is action site and the natriuresis obtained as the normal maximal plateau. This is not the case when an oedematous systemic disease with effective hypovolemia is present, like in heart failure or cirrhosis, or when chronic use of loop diuretics has induced a hypertrophy of the more distant part of the tubule. In theses cases, a pharmacodynamic resistance exists, resulting in a lower maximal natriuresis plateau in spite of adequate concentration of the diuretic at its action site, even in the absence of pharmacokinetic resistance factors. The main indications of diuretics are systemic oedematous disease and hypertension. In the oedematous diseases, diuretics indication is both straightforward and sufficient only if effective hypervolemia is present. The therapeutic approach is discussed according to the various clinical conditions and pathophysiological background. In uncomplicated hypertension, diuretics are the cornerstone of the therapy. The most suitable diuretic treatment for hypertension is an association of low doses thiazide (12.5-50 mg/day) with potassium sparing diuretics. Rare indications of diuretics are also reviewed.
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PMID:[Diuretic-based therapy]. 1791 42

Hepatitis C virus (HCV) infection is a global health problem, being the second most common chronic viral infection in the world with a global prevalence of about 3% (about 180 million people). HCV is both an hepatotropic and a lymphotropic virus; and chronic infection could cause, on one hand, chronic hepatitis, cirrhosis and hepatocellular carcinoma and on the other hand several extrahepatic diseases including, first, mixed cryoglobulinemia and lymphoma. The association between hepatic (hepatocellular carcinoma) and extrahepatic (lymphoma, thyroid cancer) malignancies has justified the inclusion of HCV among human cancer viruses. The pathogenesis of HCV-related sequelae (hepatic or extrahepatic) is not fully understood representing a challenge of prime importance in light of the optimization of clinico-therapeutic management of these patients. Combined treatment with pegylated interferon plus ribavirin is presently the first-line, gold standard treatment of most HCV-related diseases. However, mainly in the case of extrahepatic manifestations, a cautious approach to the patient, with a case to case accurate tailoring of therapy is frequently requested. The present review will outline the principal aspects of such HCV-induced systemic disease focusing on extrahepatic manifestations.
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PMID:Hepatitis C virus (HCV) infection: a systemic disease. 1817

Although the goal of embolization is usually to create therapeutic ischemia or hemostasis without surgery, the rationale for preoperative embolotherapy is different in several respects. First, the aim is to prepare patients for definitive surgical resection rather than cure or palliation, and, thus, the goals and expectations are limited and defined by close communication between the interventionalist and the surgeon. Second, when considering segmental resection, the normal liver should be protected from procedural damage. Third, the search for extrahepatic disease is crucial for these patients because resection is typically abandoned in favor of alternative therapies for patients with systemic disease. Finally, intraoperative ultrasound should always be considered to survey the future liver remnant for unsuspected small tumors and allow ablation of these lesions to maximize the success of partial hepatectomy. This article describes preoperative hepatic arterial and portal venous embolization in patients with or without cirrhosis complicated by tumors judged eligible for surgical resection or orthotopic liver transplantation (OLT). Each type of embolization will be reviewed in terms of indications and contraindications, technique, and complications. Finally, the outcomes will be evaluated in terms of morbidity, mortality, and tumor recurrence rates.
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PMID:Preoperative portal venous and hepatic arterial embolization of tumor. 2132 14

This study examined the relationship between volatile sulfur compounds (VSCs), including hydrogen sulphide (H(2)S), methyl mercaptan (CH(3)SH) and dimethyl sulphide [(CH(3))(2)S], in mouth air of patients and a history of systemic disease. The subjects were 387 residents (174 males and 213 females) of Fukuoka Prefecture, Japan, who participated in an oral and systemic health survey for elderly persons (mean age: 61.8, s.d. 2.8 years). The VSCs were measured using a portable gas chromatograph (OralChroma). The H(2)S concentrations were significantly greater in the 132 subjects with a history of hypertension and the 41 subjects with a history of respiratory disease, including pneumonia, pulmonary emphysema and bronchitis, than in those without such a history. The CH(3)SH concentrations were significantly greater in those with a history of hypertension. The 16 subjects with a history of cerebrovascular disease, including intracerebral haemorrhage, cerebral infarction, and subarachnoid haemorrhage, and the 58 subjects with a history of liver disease, including hepatitis, alcoholic liver disease, drug-induced liver injury, fatty liver and liver cirrhosis, showed significantly greater (CH(3))(2)S concentrations (p < 0.05). These results suggest an association between the production of VSCs in mouth air and systemic diseases such as hypertension as well as respiratory, cerebrovascular and liver diseases.
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PMID:Relationship between volatile sulfur compounds in mouth air and systemic disease. 2138 56

We report a rare case of a hepatic carcinosarcoma with rabdomyosarcomatous differentiation in its sarcomatous component. A 71-year old Caucasian female patient underwent a liver resection for a 4-cm lesion developed on an underlying HCV-related cirrhosis. Post-operative course was uneventful and the patient was discharged 5 days after surgery. At pathology, the tumor presented the features of hepatocellular carcinoma and rhabdomyosarcoma Three months later the patient experienced a liver recurrence, dying 2 months later for systemic disease. The reported case presents several peculiarities, i.e. the female gender, the HCV-related cirrhotic status, and the European origin of the patient. However, the outcome of our case confirms that this neoplasm pursues a highly aggressive course with poor prognosis.
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PMID:An unusual case of hepatic carcinosarcoma. 2201 59

Systemic sclerosis (SSc) is a chronic systemic disease that affects the skin, lungs, heart, gastrointestinal tract, kidneys, and musculoskeletal system. Although up to 90% of patients with scleroderma have been estimated to have gastrointestinal involvement, liver disease has been reported only rarely. A 51-year-old woman was hospitalized due to esophageal variceal bleeding. Her serum was positive for anti-nuclear antibody and anti-centromere antibody. Sclerodactyly was noted on both hands, and she had recently developed Raynaud's syndrome. Punch biopsy of the hand showed hyperkeratosis, regular acanthosis, and increased basal pigmentation in the epidermis, and thick pale collagenous bundles in the dermis. Liver biopsy showed chronic active hepatitis with bridging fibrosis. Consequently, she was diagnosed with liver cirrhosis due to autoimmune hepatitis (AIH) combined with SSc. AIH had subsided after administration of prednisolone at 40 mg per day. She received 5-10 mg/day of prednisolone as an outpatient, and her condition has remained stable. Patients with either AIH or SSc should be monitored for further development of concurrent autoimmune diseases. The early diagnosis of AIH combined with SSc will be helpful in achieving optimal management.
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PMID:Liver cirrhosis due to autoimmune hepatitis combined with systemic sclerosis. 2228 55


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