Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019204 (hepatocellular carcinoma)
 71,386 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Obstructive jaundice, pruritus, and malabsorption developed in twin brothers in infancy. Early liver biopsy specimens showed intracellular and canalicular cholestasis with normal bile ducts. By the age of 3 years, both had cirrhosis and portal hypertension. Each died during the teen years from hepatocellular carcinoma. These brothers represent the tenth reported family with familial cholestatic cirrhosis, and they are the first patients with this syndrome in whom hepatoma developed.
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PMID:Hepatoma in familial cholestatic cirrhosis of childhood: its occurrence in twin brothers. 21 1

Panipenem/betamipron (PAPM/BP), a new carbapenem, was studied in dermatology. PAPM/BP was used clinically in the treatment of skin and skin structure infections in a multicenter trial. Fifty three patients were enrolled in the trial. Clinical evaluations were made in 50 patients. Most patients received intravenous infusion of PAPM/BP in a dose of 500 mg twice daily. Other dosages were used in some patients. The overall clinical efficacy rate was 78%. When 15 cases of secondary infections were excluded, the rate was 85.7%. Adverse responses were nausea and/or vomiting in 3 patients, redness with itching in 1 patient, headache or head heaviness in 2 patients and diarrhea in 1 patient. The patient with redness and itching had also nausea and vomiting. This occurred 1 hour after the start of the first infusion of this drug. After the discontinuation of the treatment the symptoms went away on the next day. Abnormalities in laboratory test results were observed in 7 out of 53 patients. One patient with liver cirrhosis and hepatocellular carcinoma developed anemia (RBC 372 x 10(4)/mm3----275 x 10(4)/mm3, Hb 11.9 g/dl----8.8 g/dl, 35.1%----26.0%). Other abnormalities were all mild. Penetration of the drug into skin tissues after intravenous infusion of 500 mg of this drug in skin surgery patients was studied. Skin/serum concentration ratios ranged from 0.20 to 0.97. Skin concentrations were higher than the concentration of PAPM inhibiting 80% of clinical isolates over a period of 6 hours. In rats, skin concentrations were much lower than serum concentrations probably due to the difference in in vivo metabolism of PAPM. A few resistant strains of Staphylococcus aureus against PAPM and imipenem (IPM) were isolated. However, PAPM and IPM showed good antibacterial activities compared to other drugs tested. In conclusion, PAPM/BP is considered to be a useful drug in the treatment of skin and skin structure infections.
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PMID:[A multicenter study on panipenem/betamipron in dermatology]. 161 73

Two cases of Alagille syndrome are reported, father and son. This, a 6-year-old boy, presented with neonatal cholestasis but thereafter evolved with progressively decreasing jaundice and persisting pruritus. A liver biopsy confirmed the absence of intrahepatic bile ducts with preservation of hepatic architecture and no fibrosis. The patient had a characteristic phenotype: short stature, triangular face, deep eyes with hypertelorism, partial embryotoxon and data of peripheral pulmonary artery stenosis. His father died 43-year-old by a hepatocarcinoma. His liver biopsy showed also absence of intrahepatic bile ducts. In addition to the association Alagille's syndrome hepatocarcinoma (previously reported in six cases, three into the same family), it should be stressed in this case the long survival and the fact he had many children: the case with Alagille's syndrome, five children in good health, and one who died shortly after birth.
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PMID:[Alagille's syndrome: a family case and its association with hepatocellular carcinoma]. 165 82

To determine whether primary biliary cirrhosis differed in men and women we reviewed the presenting features and clinical course of 39 men and 191 women with primary biliary cirrhosis followed at this unit between 1970 and 1984. Age and severity of disease at time of diagnosis were similar in both groups. Pruritus was significantly less common in men than in women both at diagnosis and throughout the period of follow up (p less than 0.01). The difference in incidence of pruritus at diagnosis was most evident when the male group were compared with a group of premenopausal women, an observation which is consistent with involvement of sex steroid metabolism in the origin of pruritus. Skin pigmentation was also less marked in men at diagnosis (p less than 0.05). Autoimmune associated conditions, especially sicca syndrome, were more common in women. Survival was similar among men and women although hepatoma developed significantly more frequently in male patients (p less than 0.01).
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PMID:Primary biliary cirrhosis in men. 379 20

A 31 year-old patient presented with a retention jaundice from a centrally located hepatoma invading the hilum. Because of the patient's age and the severity of the pruritus and jaundice, palliative treatment was performed by percutaneous catheterization of the intrahepatic biliary tracts to drain the right and left hepatic canals. As the hilar stenosis could not be overcome, the external drainage was transformed into internal drainage by implantation of a Kron's biliary prosthesis linking the intrahepatic biliary tracts, following segment III hepatotomy, to the duodenal lumen, with burying of the prosthesis in the gastric antrum region. Jaundice was reduced until death of the patients from metastases 6 months later. This case demonstrates that the use of Kron's biliary prosthesis to perform a biliodigestive shunt from intrahepatic biliary pathways is a valid palliative procedure in patients with limited life expectancies.
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PMID:[Retention jaundice caused by central hepatic hepatoma. Treatment with Kron's extra-anatomic biliary prosthesis]. 619 70

The clinical findings in 33 patients with progressive familial intrahepatic cholestasis (PFIC) are presented. Symptoms developed almost invariably before 6 months of age with severe pruritus and moderate jaundice. Other clinical findings included wheezing and nosebleeds, fat-soluble vitamin deficiency states, and cholelithiasis. Lower values for gamma-glutamyl transpeptidase, averaging 15 IU/L before the administration of phenobarbital, and cholesterol, which averaged 156 mg/dl, are helpful in distinguishing PFIC from other pediatric cholestatic liver diseases. Autosomal recessive inheritance is probable. Twenty-six patients are alive at 12.9 +/- 6.7 years of age, all having had successful surgical treatment, either partial biliary diversion (n = 17) or orthotopic liver transplantation (n = 10). Seven patients died at a mean age of 3.9 +/- 2.4 years, as a result of liver failure in two, hepatocellular carcinoma in two, and complications of liver transplantation in three.
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PMID:Clinical and biochemical findings in progressive familial intrahepatic cholestasis. 791 66

We studied hepatitis C virus (HCV)-related disease in older people because the treatment rationale for younger asymptomatic patients is based on the long-term prognosis of infection. Of the HCV-antibody-positive patients seen at Freeman Hospital 1990-1994, 25 were > 65 years old; 24 were Caucasian and one was Afro-Caribbean. Median age at presentation was 67 years, and five were female. Nine were asymptomatic at presentation, six presented with varices, five with malaise, three with abdominal pain, one with pruritus and one with oedema. Risk factors identified were: transfusion (7), haemodialysis (1), health care worker (dentist) (1), and tattoos (2). There was no recognized risk factor for infection in 14, but five of these had done military service in areas of high HCV prevalence. Liver biopsy in 20 showed chronic hepatitis in two, cirrhosis in 12, and cirrhosis and hepatocellular carcinoma in six. Three additional patients also developed hepatocellular carcinoma. HCV genotyping was done in 19 and all were type 1 (1a, 4; 1b, 14; 1 untypable, 1). Eleven died, at median age 71 years (range 65-94 years), five of HCV liver-related deaths and two from HCV-associated non-hepatic disorders (non-Hodgkin's lymphoma and fibrosing alveolitis).
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PMID:Hepatitis C virus infection in the elderly. 873 16

Cholangiocarcinoma, are uncommon cancers, and the least common of those categorized as liver and biliary tract cancers. May occur anywhere in the biliary tree. Patients typically present with painless jaundice and pruritus, cholangitis is uncommon. They are classified into three broad anatomic groups: Intrahepatic, perihilar and distal lesions. This classification system is useful for monitoring outcome, since intrahepatic tumors are usually managed as hepatocellular carcinoma with liver resection, and distal tumors are treated as pancreatic cancer with pancreatoduodenectomy. Perihilar lesions may be treated with a variety of operative and nonoperative therapies. Surgery is the only potentially curative treatment. Prognosis and resectability depend on tumor location along the biliary tree, the extent of hepatic parenchymal involvement and the invasion of major blood vessels by the tumor.
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PMID:[Cholangiocarcinoma]. 948 May 27

Cremophor EL (cremophor), a component of the paclitaxel formulation, can potentially reverse P-glycoprotein-associated multidrug resistance. A Phase I trial of cremophor as a 6-h infusion every 3 weeks was performed with bolus doxorubicin (50 mg/m2). The cremophor dose was escalated from 1 to 60 ml/m2. A standard paclitaxel premedication was given before cremophor. Using a bioassay, potentially active cremophor levels (> or = 1 microl/ml) were measured in plasma from patients receiving cremophor doses of 30, 45, and 60 ml/m2. A cross-over design was used to assess the influence of cremophor 30 ml/m2 on the pharmacokinetics of doxorubicin and doxorubicinol. The plasma area under the concentration versus time curve (AUC) of doxorubicin increased from 1448 +/- 350 to 1786 +/- 264 ng/ml x h (P = 0.02) in the presence of cremophor, whereas the AUC of doxorubicinol increased from 252 +/- 104 to 486 +/- 107 ng/ml x h (P = 0.02). This pharmacokinetic interaction was associated with significantly increased neutropenia. With reduction of the doxorubicin dose to 35 mg/m2, the cremophor dose was increased to 60 ml/m2. Dose-limiting toxicities occurred in two of six patients after 45 ml/m2 and two of four patients after 60 ml/m2, which included febrile neutropenia and grade III cremophor-related toxicities of rash, pruritus, headache, and hypotension. All patients who received 45 ml/m2 cremophor reached plasma levels > or = 1.5 microl/ml, but at 60 ml/m2, only two of four reached this level, and the calculated plasma clearance of cremophor was significantly faster at this dose. One patient with hepatoma resistant to epirubicin achieved a near-complete response. Cremophor 45 ml/m2 over 6 h with 35 mg/m2 doxorubicin is recommended for further studies. The pharmacokinetic interaction between cremophor and doxorubicin is quantitatively similar to that described in trials of paclitaxel with doxorubicin and suggests that the cremophor in the paclitaxel formulation is responsible.
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PMID:Phase I trial of cremophor EL with bolus doxorubicin. 979 61

Primary sclerosing cholangitis (PSC) and primary biliary cirrhosis (PBC) are chronic progressive cholestatic diseases that frequently lead to biliary cirrhosis. The exact pathogenesis of these diseases remains elusive but is likely immunologically based. Complications range from fatigue and pruritus to end-stage liver disease. The risk of developing hepatocellular carcinoma is low for patients with PBC, whereas cholangiocarcinoma is common in PSC and carries an ominous prognosis. Although ursodeoxycholic acid is effective in slowing the progression of PBC, no effective medical therapy exists for PSC. Liver transplantation is the only option for patients with end-stage liver disease and yields excellent long-term survival in both groups.
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PMID:Biliary tract inflammatory disorders: primary sclerosing cholangitis and primary biliary cirrhosis. 1098 Sep 34


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