Idiopathic adulthood ductopenia presenting with chronic recurrent cholestasis

Faa, Gavino; Eyken, Peter Van; Demelia, Luigi; Vallebona, E.; Costa, Virgilio; Desmet, Valeer

doi:10.1016/0168-8278(91)90902-n

Cited by 22 publications

(5 citation statements)

References 30 publications

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“…Although we do not have data in her past history which could rule out the possibility of her having chronic liver diseases before the onset, laparoscopic and liver biopsy findings effectively rule out underlying advanced chronic liver diseases. In a patient with idiopathic adulthood ductopenia, the illness progresses towards severe hepatic failure in 3 to 11 years after clinical onset (13,14). The time taken to reach biliary cirrhosis in our case is shorter than that in idiopathic adulthood ductopenia, suggesting that extensive bile duct damage occurred immediately after the drug administration.…”

Section: Discussionmentioning

confidence: 59%

“…On the other hand, idiopathic adulthood ductopenia should be included in any cases of cholestasis with unknown cause, if a drug can be excluded as a cause (10). Patients reported with idiopathic adulthood ductopenia are mostly young adults (10,13,14): in our case, the patient is a post-menopausal woman and a drug is evidently a candidate as a cause. Drug-induced liver injury has a wide range of histologic patterns: they form an acute, reversible to fatal, massive necrosis of hepatocytes.…”

Section: Discussionmentioning

confidence: 75%

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A case of drug‐induced ductopenia resulting in fatal biliary cirrhosis

Ishii

Miyazaki

Yamamoto

et al. 1993

Liver

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ABSTRACT— A 50‐year‐old woman suffered from a diffuse skin rash, high fever and jaundice immediately after a second injection of glutathion and Stronger Neo‐minophagen C which contains glycyrrhizin. Liver biopsy performed 11 months after the onset showed mild spotty hepatocyte necrosis, marked cholestasis in parenchyma, and some lymphocyte infiltration in the portal area. Interlobular bile ducts had undergone vacuolar degeneration or were absent in some portal tracts. In her hospital course, unremitting jaundice persisted and biliary cirrhosis developed with signs of portal hypertension; she died from liver failure 26 months after the onset. A liver specimen at her death revealed that most of the interlobular bile ducts had vanished. Based on the clinical course and pathology, drug‐induced ductopenia, possibly due to an adverse reaction to glycyrrhizin, is the most likely diagnosis. While drug‐related biliary cirrhosis is rarely fatal, this case presented an unusually rapid course of fatal biliary cirrhosis.

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Section: Discussionmentioning

confidence: 59%

Section: Discussionmentioning

confidence: 75%

A case of drug‐induced ductopenia resulting in fatal biliary cirrhosis

Ishii

Miyazaki

Yamamoto

et al. 1993

Liver

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“…Ductopenia is currently considered an acquired lesion resulting from a progressive destruction of fully developed bile ducts. However, two evolutive courses exist: a) progressive forms for which liver transplant is the only option within 3 to 11 years (30); and b) benign, non-progressive forms that may or may not manifest with recurrent cholestasis episodes (31,32).…”

Section: Dr S Rodríguez Muñozmentioning

confidence: 99%

“…Actualmente se considera a la ductopenia como una lesión adquirida, el resultado de la destrucción progresiva de los ductos biliares plenamente formados. No obstante, se pueden reconocer dos cursos de la enfermedad bien diferenciados: a) formas progresivas para las cuales el trasplante hepático es la única alternativa terapéutica en un plazo de entre 3 y 11 años (30); y b) formas benignas no progresivas que pueden cursar o no, con episodios recurrentes de colestasis (31,32).…”

Section: La Ductopenia Permite Orientar El Diagnóstico Hacia Una De Lunclassified

A young woman with cholestasis and ductopenia

Gómez

Gil

Muñoz

et al. 2004

Rev. esp. enferm. dig.

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and development of mostly incomplete septa with only a few complete porto-portal fibrous bridges. Spatial relationships were adequately preserved between portal tracts and centroacinar veins. No interlobular ducts were seen in over 50% of portal spaces (6 of 11), with presence of hepatic arteries (Fig. 2). Furthermore, portal spaces had a mild mixed inflammatory infiltrate with predominant lymphocytes. The few remaining interlobular ducts had an irregular biliary epithelium. Within the limiting periportal trabecule foci of collagen-entrapped hepatocytes were seen. Periportal hepatocytes were clearly changed and had copper deposits (Figs. 3 and 4). In the remaining lobule, liver cells had their typical characteristics or showed mild hyperplastic features. No positive globules were seen using the periodic acid-Schiff (PAS) stain. No viral inclusions were apparent. Bile pig

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“…Syndromatic ductopenia, also known as Alagille's syndrome, could be ruled out; none of the accompanying features such as pulmonary stenosis, posterior embryotoxon or skeletal abnormalities was found (4). In contrast, nonsyndromatic ductopenia was a consideration; the prognosis in young adults with this condition is worse than with that for Alagille's syndrome, and signs and symptoms of portal hypertension and liver failure can develop (5)(6)(7). Finally, idiopathic noncirrhotic portal hypertension (hepatoportal sclerosis) was a consideration but appeared rather unlikely; it is (a) extremely rare outside the Far East and (b) not associated with cholestasis (8,9).…”

Section: Clinical Differential Diagnosismentioning

confidence: 99%

A 20-year-old woman with portal hypertension and a cholestatic syndrome

Bianchi

Reichen

1994

Hepatology

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A 20-yr-old white woman consulted her family physician for secondary amenorrhea; the only abnormality found at that time was iron deficiency anemia. The subsequent workup revealed grade 2 esophageal varices with sonographic signs of portal hypertension and possible cirrhosis; the portal vein was patent, as shown by Doppler sonography. One month later the patient experienced hematemesis and was hospitalized. Her personal and family histories were unremarkable. In particular, there was no prolonged jaundice in the perinatal period. Review of systems revealed only intermittent pruritus for the past 2 yr.On physical examination, the patient appeared well developed and well nourished (70 kg, 159 cm). Blood pressure was 100/60 mm Hg, and heart rate was 84 beats/min. She was anicteric, and there were no cutaneous stigmata of chronic liver disease. Cardiopulmonary examination was unremarkable. The abdomen was soft; no ascites was present. The liver measured 14 cm by percussion and was palpable with increased consistency 3 cm below the costal margin; the spleen was enlarged and palpable 4 cm below the left costal margin. Otherwise, physical examination was unremarkable.Bilirubin was 12 pmol/L (0.7 mg/100 ml); AST and ALT were slightly increased, at 113 and 124 U/L, respectively (normal for both, <41). Levels of other enzymes and bile acids were markedly increased: alkaline phosphatase, 852 IU/L (normal, ~1 2 0 ) ; 5'nucleotidase, 132 IU/L (normal, < 18); y-glutamyltranspeptidase, 298 IU/L (normal, 8 to 45); and total serum bile acids, 48.4 pmoVL (RIA, normal, < 6). Total serum protein was 77.6 gm/L, with normal electrophoresis (albumin, 37 gm/L; normal, 32 to 51 gm/L).

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Idiopathic adulthood ductopenia presenting with chronic recurrent cholestasis

Cited by 22 publications

References 30 publications

A case of drug‐induced ductopenia resulting in fatal biliary cirrhosis

A case of drug‐induced ductopenia resulting in fatal biliary cirrhosis

A young woman with cholestasis and ductopenia

A 20-year-old woman with portal hypertension and a cholestatic syndrome

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