1/420. Striking cholestatic liver disease: a distinct manifestation of advanced primary amyloidosis. In patients with systemic amyloidosis, amyloid fibrils are typically deposited in numerous organs, including the kidneys, heart, and liver. Although amyloid deposition in the liver is common in patients with systemic amyloidosis, clinical liver disease is relatively rare. The patient described here had cholestatic liver disease as the primary manifestation of primary systemic amyloidosis. review of the literature suggests that prominent liver disease with cholestasis is unusual but probably underreported in patients with amyloidosis and appears to be restricted to patients with the primary form of amyloidosis. Nonetheless, cholestatic hepatic amyloidosis is characterized by distinct clinical, laboratory, and pathologic features; recognition of this process is critical because it identifies patients with widespread organ involvement and portends a poor prognosis. ( info) |
| erythromycin is a frequently used antibiotic in patients with atypical respiratory infection and/or an allergy to penicillin. We report the case of a young woman who developed severe cholestasis and jaundice following treatment with erythromycin stearate. Two years later her general practitioner prescribed erythromycin succinate for pharyngitis. She experienced a severe second episode of jaundice and malaise. Different esters of erythromycin have been introduced to reduce side effects such as allergic reactions to erythromycin. The findings in our patient underline the fact that hypersensitivity is caused by the erythromycin molecule, independent from the type of esterification. Because of these side effects newer makrolides should be given preference over erythromycin. ( info) |
3/420. Severe intrahepatic cholestasis caused by amiodarone toxicity after withdrawal of the drug: a case report and review of the literature. cholestasis has been reported as a rare presentation among patients with severe liver injury secondary to amiodarone hepatic toxicity. We report an unusual case of amiodarone-induced cholestatic hepatotoxicity occurring after amiodarone had been discontinued and the initial abnormal liver function findings had improved. The patient, without jaundice at the initial presentation, developed severe jaundice about 4 months after withdrawal of amiodarone. light and transmission electron microscopic examination of a specimen secured by computed tomographically guided liver biopsy was consistent with amiodarone hepatic toxicity as the cause of intrahepatic cholestasis. An abdominal ultrasound, endoscopic retrograde cholangiography, and dimethyl iminodiacetic acid and computed tomographic scans of the abdomen all failed to demonstrate any other causes for jaundice other than amiodarone toxicity. Thus, amiodarone hepatic toxicity may occur after drug withdrawal even if results of liver function tests improve. Histopathologic examination of a liver biopsy specimen is of value for diagnosis and prognosis. The liver biopsy findings, clinical course, and liver function test results are discussed, and the English-language literature on amiodarone cholestatic hepatotoxicity is reviewed. ( info) |
4/420. Are expandable metallic stents better than conventional methods for treating difficult intrahepatic biliary strictures with recurrent hepatolithiasis? BACKGROUND: Conventional methods for treating patients with recurrent hepatolithiasis associated with complicated intrahepatic biliary strictures include balloon dilatation of the intrahepatic biliary strictures, lithotripsy, and the clearance of difficult stones as completely as possible, with the placement of an external-internal stent for at least 6 months. After these modalities are used, symptomatic refractory strictures remain. Recently we used internal Gianturco-Rosch metallic Z stents to treat patients who had refractory strictures. OBJECTIVE: To compare therapeutic results and complications of an internal expandable metallic Z stent with those of repeated external-internal stent placement. STUDY DESIGN: Case-control study. SETTING: A referral center. patients: From January 1992 to December 1996, 18 patients with recurrent hepatolithiasis and complicated intrahepatic biliary strictures underwent percutaneous dilatation of stricture and transhepatic percutaneous cholangioscopic lithotomy for recurrent stones. After their stones were completely cleared, their biliary strictures failed to dilate satisfactorily. The patients were randomly enrolled into 2 groups: group A (7 patients), who received an expandable metallic Z stent, and group B (11 patients), who had repeated placement of external-internal stents. INTERVENTIONS: Percutaneous stricture dilatation, electrohydraulic lithotripsy, balloon dilatation, percutaneous transhepatic cholangioscopic lithotomy, and biliary stenting by a Silastic external-internal catheter or a modified Gianturco-Rosch expandable metallic Z stent (for an internal stent). MAIN OUTCOME MEASURES: The number of procedures, days in hospital, procedure-related complications, incidents of stone recurrence and recurrence of cholangitis, readmissions to the hospital, treatment sessions required, and mortality rate. patients' limitations in ordinary activities were also compared. RESULTS: The follow-up period ranged from 28 to 60 (40.7 /-12.7 [mean /- SD]) months in group A and from 28 to 49 (36.0 /-7.2) months in group B. Fewer group A patients (3 [43%]) than group B patients (8 [73%]) tended to have recurrent cholangitis and to require readmission to the hospital, but this was not statistically significant (P = .33). When their cumulative probability of a first episode of cholangitis during follow-up was compared, however, it was significantly lower in patients treated with a metallic stent (P = .04). Compared with group B patients, group A patients had less frequent recurrence of stones (0% vs 64%; P = .01), fewer procedures for the clearance of biliary stones or sludge (1.7 /-2.2 vs 6.4 /-4.3; P = .03), and shorter hospital stays (8.0 /-11.5 days vs 17.0 /-12.0 days; P = .07). No patients in group A experienced limitation in ordinary activities, whereas 7 patients in group B did (P<.02). CONCLUSIONS: Compared with the repeated placement of external-internal stents, the use of a metallic internal stent effectively decreases stone recurrence, simplifies further procedures, and is more convenient. Its use is suggested as an alternative choice in the treatment of recurrent hepatolithiasis with refractory intrahepatic biliary strictures. ( info) |
5/420. Fibrosing cholestatic hepatitis in renal transplant recipients with hepatitis c virus infection. Fibrosing cholestatic hepatitis (FCH) has been described as a specific manifestation of hepatitis b virus (HBV) infection in liver allograft recipients characterized by a rapid progression to liver failure. Only sporadic cases have been reported in other immunocompromised groups infected with HBV and in a few transplant recipients with hepatitis c virus (HCV) infection. We present the occurrence of FCH in 4 HCV-infected renal transplant recipients within a series of 73 renal transplant recipients with HCV infection followed up closely serologically and with consecutive liver biopsies. All 4 patients received the triple-immunosuppressive regimen (azathioprine, cyclosporine A, methylprednisolone). The interval from transplantation to the appearance of liver dysfunction was 1 to 4 months and to histological diagnosis, 3 to 11 months. The biochemical profile was analogous to a progressive cholestatic syndrome in 3 patients, whereas the fourth patient had only slightly increased alanine aminotransferase and gamma-glutamyl transferase (gammaGT) levels. Liver histological examination showed the characteristic pattern of FCH in 2 patients, whereas the other 2 patients had changes compatible with an early stage. All patients were anti-HCV negative at the time of transplantation, whereas 2 patients, 1 with incomplete and 1with complete histological FCH features, seroconverted after 3 and 31 months, respectively. The patients were HCV rna positive at the time of the first liver biopsy and showed high serum HCV rna levels (14 to 58 x 10(6) Eq/mL, branched dna). HCV genotype was 1b in 3 patients and 3a in 1 patient. After histological diagnosis, immunosuppression was drastically reduced. Two patients died of sepsis and liver failure 16 and 18 months posttransplantation, whereas the seroconverted patients showed marked improvement of their liver disease, which was histologically verified in 1 patient. In conclusion, FCH can occur in HCV-infected renal transplant recipients. It seems to develop as a complication of a recent HCV infection during the period of maximal immunosuppression and is associated with high HCV viremia levels. There are indications that drastic reduction of immunosuppression may have a beneficial effect on the outcome of the disease. ( info) |
6/420. Extrahepatic Hodgkin's disease with intrahepatic cholestasis: report of two cases. Liver is involved in about 5-8% of newly diagnosed Hodgkin's disease (HD) cases. The incidence reaches up to 50-60% in postmortem studies. In the literature only a few cases of idiopathic cholestatic jaundice have been described without an apparent cause and a paraneoplastic etiology has been suggested. We report 2 cases with HD presenting with obstructive jaundice without obvious liver involvement. The first case died soon after diagnosis; the second case received chemotherapy and radiotherapy, and she is well at 26 months' follow-up. Extrahepatic HD with intrahepatic cholestasis is an extremely rare situation without an established approach. Such cases like the present ones may help to understand the pathogenesis of the liver involvement of HD and determine the best management of these cases. ( info) |
7/420. hepatitis c virus-related fibrosing cholestatic hepatitis after cardiac transplantation: is azathioprine a contributory factor? We report a patient who acquired hepatitis c virus (HCV) infection at cardiac transplantation, developing fibrosing cholestatic hepatitis (FCH) with early liver failure and a fatal outcome. FCH is a recently described clinicopathological entity characterized by a cholestatic pattern of serum liver enzyme abnormalities, a progressive course leading to liver failure, and a pathological picture defined by periportal fibrosis, neutrophilic infiltrates and signs of histological cholestasis. Although it was initially described secondary to hepatitis b virus infection, it has also been recently related to HCV infection. Some histopathological features consistent with azathioprine hepatotoxicity like cholestasis, perisinusoidal fibrosis, veno-subocclusive lesions and nodular regenerative hyperplasia were also observed in this case. Therefore, a direct cytopathic effect of HCV and the concurrent pathogenic role of azathioprine hepatotoxicity may be involved in the development of this complication of transplantation. ( info) |
8/420. Sensorineural hearing loss associated with Byler disease. Progressive familial intrahepatic cholestasis, sometimes described as Byler disease, is a lethal liver disease and its inheritance is autosomal recessive. There is a previous report on the occasional association between this disease and sensorineural hearing loss without any audiological findings. We report here two siblings, an 18-year-old female and a 16-year-old male, suffering from Byler disease and hearing loss. Pure tone, Bekesy and speech audiometries and auditory brain stem response examination were performed. Audiometric data showed hearing characteristics of cochlear origin, high-frequency loss and progressiveness. This sensorineural hearing loss possibly results from a genetic mutation. The mechanism of cochlear disorder in patients with Byler disease is unknown, however, a novel gene responsible for deafness might be found to be related to Byler disease. ( info) |
9/420. Myeloma and severe cholestasis. amyloidosis is a frequent complication of multiple myeloma. Liver involvement is common in amyloidosis. Hepatic dysfunction and liver chemistry abnormalities are often mild or absent and obstructive jaundice is rare. We report on a 44-year-old patient with multiple myeloma and rapidly deteriorating liver involvement with severe intrahepatic cholestasis. autopsy showed widespread amyloidosis primarily involving the liver. This unusual cholestatic manifestation of hepatic amyloidosis has an uniformly poor prognosis, with death occurring within a few months. We discuss the clinical and pathologic aspects. ( info) |
10/420. Acute cholestatic hepatitis by cytomegalovirus in an immunocompetent patient resolved with ganciclovir. We report a case of acute cholestatic hepatitis in an immunocompetent young male with cytomegalovirus (CMV) primoinfection episode. The severity of the clinical symptoms led to a high-dose treatment with parenteral ganciclovir, with an immediate response and total resolution of symptoms. Therapeutic options are discussed, particularly the use of ganciclovir, even in immunocompetent patients when the severity of the symptoms could demand it. ( info) |