scholarly journals Ursodeoxycholic acid for treatment of cholestasis in patients with hepatic amyloidosis

2009 ◽  
Vol 66 (6) ◽  
pp. 482-486 ◽  
Author(s):  
Dominik Faust ◽  
Bora Akoglu ◽  
Gordana Ristic ◽  
Vladan Milovic

Background. Amyloidosis represents a group of different diseases characterized by extracellular accumulation of pathologic fibrillar proteins in various tissues and organs. Severe amyloid deposition in the liver parenchyma has extrahepatic involvement predominantly in the kidney or heart. We evaluated the effect of ursodeoxycholic acid, in four patients with severe hepatic amyloidosis of different etiologies, who presented with increased alkaline phosphatase and ?-glutamyl transferase. Case report. The study included four patients who presented with amyloidosis-associated intrahepatic cholestasis. Three of them had renal amyloidosis which developed 1-3 years before cholestasis occurred, the remaining one having intrahepatic cholestasis as the primary sign of the disease. Amyloidosis was identified from liver biopsies in all patients by its specific binding to Congo red and green birefringence in polarized light. The biochemical nature and the class of amyloid deposits were identified immunohistochemically. In addition to their regular treatment, the patients received 750 mg ursodeoxycholic acid per day. After 2-4 weeks all patients had a significant decrease of serum alkaline phosphatase and ?-glutamyl transferase, and their general status significantly improved. Conclusion. Treatment with ursodeoxycholic acid may be beneficial in patients with hepatic amyloidosis, and do extend indications for the use of ursodeoxycholic acid in amyloidotic cholestatic liver disease.

2016 ◽  
Vol 6 (4) ◽  
Author(s):  
Nikhil Sonthalia ◽  
Sami S. Jain ◽  
Vinay B. Pawar ◽  
Vinay G. Zanwar ◽  
Ravindra G. Surude ◽  
...  

We describe a case of two-year-old boy presenting with debilitating pruritus, patchy alopecia and jaundice since the age of 6 months. On evaluation he had intrahepatic cholestasis with persistently raised serum alkaline phosphatase, normal Gamma glutamyl transferase and raised serum bile acid levels. His liver biopsy showed bland cholestasis and electron microscopy showed granular bile suggestive of progressive familial intrahepatic cholestasis type I. Medical therapy with ursodeoxycholic acid, cholestyramine, rifampicin with nutritional modification was successful in alleviating the symptoms and correcting the nutritional status. To our knowledge this is only the sixth case of progressive familial intrahepatic cholestasis type I reported from India. Herein we discuss the diagnostic and therapeutic hurdles that one encounters in managing progressive familial intrahepatic cholestasis and also review the literature regarding this rare disorder.


2019 ◽  
Vol 38 (2) ◽  
pp. 205-211 ◽  
Author(s):  
Chaehoon Han ◽  
Young-Ki Lee ◽  
Hayne Cho Park ◽  
Ajin Cho ◽  
Sun Ryoung Choi ◽  
...  

1998 ◽  
Vol 32 (10) ◽  
pp. 1023-1025 ◽  
Author(s):  
Lonneke MM Oostweegel ◽  
Jos H Beijnen ◽  
Jan Willem Mulder

OBJECTIVE: To describe a case of severe hepatitis that we attribute to the use of chloroguanide. CASE SUMMARY: A patient was admitted with fever and jaundice. This man had recently returned from Indonesia and was still using chloroguanide 200 mg once daily for 6 weeks. Evaluation of the liver function test results was consistent with cholestatic liver disease (alkaline phosphatase 158 U/L, aspartate aminotransferase 33 U/L, alanine aminotransferase 58 U/L increasing up to 183 U/L, γ-glutamyl transferase 96 U/L). This condition was thought to be caused by chloroguanide. A biopsy of the liver indicated drug-induced hepatitis. DISCUSSION: To our knowledge, this is the first report in the literature of cholestatic hepatitis associated with chloroguanide prophylaxis. In our patient, chloroguanide-induced hepatotoxicity can be considered as a combined allergic and idiosyncratic manifestation. CONCLUSIONS: This case shows that it is possible to develop drug-induced hepatitis with considerable morbidity while taking chloroguanide prophylaxis.


2020 ◽  
Vol 15 (2) ◽  
pp. 68-71
Author(s):  
Sayeda Tasnim Kamal ◽  
Qazi Shamima Akhter

Background: The textile dyeing industry uses large number of chemicals, acids and dyes. The workers are exposed to these chemicals during their daily work activities. Chronic exposure to these chemicals may have adverse effect on the liver functions, as liver plays an important role in detoxification of these chemicals Objective: To assess serum Alkaline Phosphatase (ALP) and Gamma glutamyl transferase (GGT) levels of the industrials workers exposed to textile dyes. Methods: This cross sectional study was carried out in the Department of Physiology,Dhaka Medical College, Dhaka from July 2016 to June 2017 on 25 apparently healthy male workers aged 20-40 years working in a textile dye factory for 2 years or more. Similar age, sex, BMI & socioeconomically matched 25 apparently healthy non exposed workers were enrolled as control. Serum ALP and GGT levels were estimated by autoanalyzer. For statistical analysis unpaired Student’s t-test were performed Results: Serum ALP and GGT levels were significantly higher in textile dye exposed workers than those of control group (p <0.05). Conclusions:This study reveals that exposure to textile dyes have deteriorating effect on liver function. J Bangladesh Soc Physiol. 2020, December; 15(2): 68-71


2020 ◽  
Vol 13 (7) ◽  
pp. e234193
Author(s):  
Nida Mirza ◽  
Ravi Bharadwaj ◽  
Smita Malhotra ◽  
Anupam Sibal

A 15-year-old boy who had a history of on and off pruritus and jaundice since many years found to have a novel mutation in TJP2 gene. On examination, he had clubbing, splenomegaly, grade 3 oesophageal varices and short stature. Investigation revealed direct hyperbirubinemia with elevated liver enzymes with normal gamma-glutamyl transferase (GGT). Antinuclear antibody (ANA), smooth muscle antibody (SMA) anti-liver kidney microsomal (anti-LKM) and viral markers for hepatitis were negative. However, IgG was elevated and anti-smooth muscle antibody (ASMA) was weekly positive (1:20). He was also given a trial of steroid and azathioprine for 1 year on the basis of liver biopsy findings, raised IgG and positive ASMA but finding no improvement stopped. Genetic testing by next-generation sequencing found a novel compound heterozygous missense variation in exon 17 of the TJP2 gene confirming progressive familial intrahepatic cholestasis type 4 as the aetiology of cholestatic liver disease.


1962 ◽  
Vol 42 (4) ◽  
pp. 431-438 ◽  
Author(s):  
Stanton G. Polin ◽  
Mitchell A. Spellberg ◽  
Lloyd Teitelman ◽  
Makoto Okumura

1960 ◽  
Vol XXXIV (II) ◽  
pp. 256-260
Author(s):  
Jörgen Herman Vogt

ABSTRACT A case of subacute thyroiditis is recorded, in which a transient rise in serum alkaline phosphatase values leads to the hypothesis of a transient parathyroid hyper-activity induced by the inflammation of the thyroid tissue in which the parathyroid may be embedded.


2019 ◽  
Author(s):  
Tom Thacher ◽  
Christopher Sempos ◽  
Ramon Durazo-Arvizu ◽  
Craig Munns ◽  
Philip Fischer ◽  
...  

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