1/70. Respiratory chain complex III [correction of complex] in deficiency with pruritus: a novel vitamin responsive clinical feature.We report a child with an isolated complex III respiratory chain deficiency and global developmental delay who had severe pruritus with elevated plasma bile acid levels. A liver biopsy showed micronodular cirrhosis, and enzymologic evaluation demonstrated an isolated complex III deficiency in both liver and muscle. His pruritus improved and serum bile acid levels decreased after treatment with menadione and vitamin C.- - - - - - - - - - ranking = 1keywords = liver (Clic here for more details about this article) |
2/70. Cutaneous allergic reaction to intramuscular vitamin K1.A 40-year-old woman with no pre-existing hepatic disease developed a cutaneous allergic reaction to intramuscular vitamin K1. She received this medication prophylactically prior to surgery, developed severe localized, and subsequently generalized, dermatitis, beginning 5 days after administration of the Konakion Cremophor-EL form of vitamin K1 by intramuscular injection at four sites on her thighs. Investigation by patch and intradermal testing revealed delayed-type hypersensitivity to Konakion Cremophor-EL, Konakion Mixed micelles and pure vitamin K1, but not Cremophor-EL vehicle alone. This case is unusual because the patient was also shown to be patch test positive to vitamin K3 sodium bisulfite.- - - - - - - - - - ranking = 0.1915040928582keywords = hepatic (Clic here for more details about this article) |
3/70. Malrotation with recurrent volvulus presenting with cholestasis, pruritus, and pancreatitis.A patient aged 16 years was referred following numerous episodes of intense pruritus associated with persistently altered liver function tests. Ultrasound (US) demonstrated the superior mesenteric vein positioned more to the left than usual and abnormal orientation of the duodenum, consistent with a midgut malrotation. Endoscopic retrograde cholangiopancreatography the common bile duct, suggesting a malrotation with chronic or recurrent volvulus. A classic malrotation with 180 degrees volvulus was noted at operation. A second child previously thought to have recurrent idiopathic pancreatitis was noted to have ampullary distortion secondary to malrotation following presentation with an intestinal obstruction. pruritus and pancreatitis have not been previously reported with malrotation, although cholestatic jaundice has been described. The diffculties in diagnosis and the role of US are discussed. Ladd's procedure is the appropriate treatment for this condition.- - - - - - - - - - ranking = 0.5keywords = liver (Clic here for more details about this article) |
4/70. Idiopathic adulthood ductopenia: case report and review of the literature.The clinical and pathological findings of idiopathic ductopenia were studied in a 30-year-old woman who initially manifested jaundice and pruritus. serum biochemical tests of liver function indicated severe and progressive cholestasis. Viral hepatitis markers and circulating autoantibodies were absent. The patient had a normal cholangiogram and lacked evidence of inflammatory bowel disease. Histological examination of a liver specimen showed severe cholestasis and absence of interlobular bile ducts. Severe jaundice and intractable pruritus developed in the patient and served as the indications for liver transplantation 4 months after initial examination. Transplantation resulted in prompt and complete resolution of the jaundice and pruritus. Two types of idiopathic adulthood ductopenia associated with different prognoses are recognized. patients with type 1 idiopathic adulthood ductopenia are asymptomatic or manifest symptoms of cholestatic liver disease. They tend to have less destruction of the intrahepatic bile ducts on liver biopsy specimens. Their clinical course ranges from spontaneous improvement to progression to biliary cirrhosis. In contrast, patients with type 2 idiopathic adulthood ductopenia generally manifest initial symptoms of decompensated biliary cirrhosis, have extensive destruction of the intrahepatic bile ducts on liver biopsy, and frequently require orthotopic liver transplantation.- - - - - - - - - - ranking = 3.8830081857164keywords = liver, hepatic (Clic here for more details about this article) |
5/70. The combined occurrence of macular amyloidosis and prurigo nodularis.The association of prurigo nodularis (PN) and macular amyloidosis (MA) has not been reported before. Although pruritus related frictional trauma is a well-known cause of PN, its role in the development of MA has always been questioned. We herein report two cases with chronic liver disease and iron deficiency who concomitantly developed MA and PN lesions. pruritus was the preceding factor and both lesions were confined to scratched areas. The association of two otherwise uncommon dermatoses in pruritic patients and their characteristic distribution might indicate an important role for pruritus-induced scratching in the pathogenesis of MA, too.- - - - - - - - - - ranking = 0.5keywords = liver (Clic here for more details about this article) |
6/70. A case report of olanzapine-induced hypersensitivity syndrome.hypersensitivity syndrome is defined as a drug-induced complex of symptoms consisting of fever, rash, and internal organ involvement. The hypersensitivity syndrome is well recognized as being caused by anticonvulsants. Olanzapine is an atypical antipsychotic agent whose side effects include sedation, weight gain, and increased creatinine kinase and transaminase levels. To date, there have been no reports of hypersensitivity syndrome related to this drug. A 34-year-old man developed a severe generalized pruritic skin eruption, fever, eosinophilia, and toxic hepatitis 60 days after ingestion of olanzapine. After termination of olanzapine treatment, the fever resolved, the skin rash was reduced, eosinophil count was reduced to normal, and the transaminase levels were markedly reduced. Clinical features and the results of skin and liver biopsies indicated that the patient developed hypersensitivity syndrome caused by olanzapine.- - - - - - - - - - ranking = 0.5keywords = liver (Clic here for more details about this article) |
7/70. fosinopril-induced prolonged cholestatic jaundice and pruritus: first case report.We report a case of fosinopril-induced prolonged cholestatic jaundice and pruritus in a 61-year-old man, with no previous hepatobiliary disease, who presented with asthenia, jaundice and itching 3 weeks after starting fosinopril therapy. Other drugs taken by the patient were not considered probable causes. The diagnostic evaluation showed no biliary obstruction and other possible causes of intra-hepatic cholestasis were excluded. Liver biopsy showed cholestasis without bile duct damage. The disease ran a severe course during the 2 months of hospitalization, with prolonged itching for 6 months, eventually controlled with oral naltrexone. jaundice subsided after 4 months, with anicteric cholestasis persisting for more than 18 months. Similar occurrences have been reported with other inhibitors of angiotensin-converting enzyme (mostly captopril), but this is the first case of an important adverse reaction to fosinopril.- - - - - - - - - - ranking = 0.1915040928582keywords = hepatic (Clic here for more details about this article) |
8/70. jaundice in non-cirrhotic primary biliary cirrhosis: the premature ductopenic variant.The clinical and pathological findings of four females with primary biliary cirrhosis (PBC) with an unusual and hitherto not well recognised course are reported. patients suffered severe pruritus and weight loss with progressive icteric cholestasis which did not respond to such treatments as ursodeoxycholic acid and immunosuppressives. In all cases liver histology revealed marked bile duct loss without however significant fibrosis or cirrhosis. Further diagnostic studies and repeat biopsies confirmed the absence of liver cirrhosis as well as other potential causes of hyperbilirubinaemia. Comparison of the fibrosis-ductopenia relationship for our cases with that for a group of 101 non-cirrhotic PBC patients indicated that in the former the severity of bile duct loss relative to the amount of fibrosis was significantly higher. The proportion of portal triads containing an interlobular bile duct was 3%, 4%, 6%, and 10% compared with 45% (median; range 8.3--100%) for controls (p<0.001). Three patients received a liver transplant 6--7 years after the first manifestation of PBC because of progressive cholestasis, refractory pruritus, and weight loss, while the fourth patient is considering this option. In one case cirrhosis had developed at the time of transplantation while the others still had non-cirrhotic disease. These cases suggest that cholestatic jaundice in non-cirrhotic PBC may be secondary to extensive "premature" or accelerated intrahepatic bile duct loss. Although the extent of fibrosis may be limited initially, progression to cirrhosis appears to be inevitable in the long run. Despite intact protein synthesis and absence of cirrhotic complications, liver transplantation in the pre-cirrhotic stage for preventing malnutrition and to improve quality of life should be considered for these patients.- - - - - - - - - - ranking = 2.1915040928582keywords = liver, hepatic (Clic here for more details about this article) |
9/70. liver transplantation for intractable pruritus is contraindicated before an adequate trial of opiate antagonist therapy.A woman with stage III (pre-cirrhotic) primary biliary cirrhosis was referred for liver transplantation because of intractable pruritus. Oral administration of 50 mg naltrexone precipitated a severe opioid withdrawal-like reaction. Subsequently, when oral naltrexone therapy was reintroduced following a cautious infusion of naloxone, no reaction occurred and the pruritus resolved completely. liver transplantation should not be considered for apparently intractable pruritus of cholestasis before an adequate trial of opiate antagonist therapy.- - - - - - - - - - ranking = 0.5keywords = liver (Clic here for more details about this article) |
10/70. Hepatotoxicity associated with carvedilol.OBJECTIVE: To report a severe episode of pruritus and elevated serum transaminases in a patient who was receiving carvedilol. CASE SUMMARY: A 40-year-old white man who had been taking carvedilol for the treatment of cardiomyopathy presented to the emergency department with pruritus over his entire body. Laboratory tests showed elevated serum transaminases. Carvedilol was discontinued and the patient received hydroxyzine hydrochloride to relieve symptoms. Liver function test results returned to normal in three weeks. Approximately one year later the patient was started on metoprolol and within 10 days again developed pruritus. The patient was told to discontinue the metoprolol immediately. At the time the metoprolol was stopped the liver function test results were normal. DISCUSSION: To the best of our knowledge, the adverse reaction presented in this case report is rare. The etiology of this adverse reaction remains unknown but suggests a possible adverse reaction that may recur if the patient is switched to another beta-adrenergic blocker. The liver function test abnormalities appear to return to normal on discontinuation of carvedilol. CONCLUSIONS: Carvedilol may cause pruritus and elevated liver function test results. This reaction may recur if the patient is changed to an alternative beta-adrenergic blocker. patients taking carvedilol should be monitored for signs and symptoms of hepatotoxicity. If elevated liver function test results are noted, carvedilol should be discontinued.- - - - - - - - - - ranking = 2keywords = liver (Clic here for more details about this article) |
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