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1.
Eur J Gastroenterol Hepatol ; 22(10): 1253-9, 2010 Oct.
Article in English | MEDLINE | ID: mdl-20631626

ABSTRACT

OBJECTIVES: We report the Belgian Registry of 30 patients (19 women and 11 men) with hereditary haemorrhagic telangiectasia (HHT) and liver involvement. RESULTS: Twenty-three patients (77%) were asymptomatic. Within the seven symptomatic patients (23%), four suffered from high output cardiac failure, two died before liver transplantation and one was transplanted. Three patients developed symptomatic biliary disease, two were transplanted and one was listed. Intrahepatic shunts and a large hepatic artery (6-14 mm, mean: 9.3 mm) were found in all patients and are characteristic of liver involvement. We observed a high prevalence (47%) of asymptomatic hepatic tumours with radiological and histological characteristics of focal nodular hyperplasia (FNH) for the majority of these tumours. The histological examination of the three explanted livers revealed the coexistence of a large spectrum of hepatic vascular lesions including intrahepatic shunts, FNH, nodular regenerative hyperplasia, sinusoidal dilatation and ischaemic cholangiopathy. All these lesions should be diagnosed early to avoid invasive procedures even if a liver biopsy was performed in six of our patients without complications. The liver biopsy led to the diagnosis of HHT in one patient and to FNH in another one. CONCLUSION: Liver involvement in HHT is characterized by a high prevalence of FNH and a large spectrum of vascular lesions such as intrahepatic shunts, nodular regenerative hyperplasia, sinusoidal dilatation and ischaemic cholangiopathy that may coexist simultaneously in the same patient.


Subject(s)
Focal Nodular Hyperplasia/epidemiology , Focal Nodular Hyperplasia/pathology , Liver/pathology , Telangiectasia, Hereditary Hemorrhagic/epidemiology , Telangiectasia, Hereditary Hemorrhagic/pathology , Adolescent , Adult , Aged , Belgium/epidemiology , Biliary Tract/pathology , Biopsy , Female , Focal Nodular Hyperplasia/diagnostic imaging , Hepatic Artery/pathology , Hepatic Veins/pathology , Humans , Magnetic Resonance Imaging , Male , Middle Aged , Organ Size , Prevalence , Registries , Telangiectasia, Hereditary Hemorrhagic/diagnostic imaging , Tomography, X-Ray Computed , Young Adult
2.
Acta Gastroenterol Belg ; 69(4): 381-3, 2006.
Article in English | MEDLINE | ID: mdl-17343080

ABSTRACT

Angiotensin-converting enzyme (ACE) inhibitors are widely used in heart diseases. We describe a case of a young woman treated with fosinopril. She started experiencing abdominal pain, vomiting and diarrhoea with peritoneal signs on physical examination three years after her treatment has been initiated. She presented ascites and signs of ileitis on imaging studies. She even underwent surgery. The diagnosis of ACE inhibitor-induced angiooedema of the small bowel was made after the fourth episode. Fosinopril was stopped and the symptoms never recurred. The case we describe illustrates clinical presentation, radiological findings and difficulty of making an accurate diagnosis in such a patient.


Subject(s)
Angioedema/etiology , Angiotensin-Converting Enzyme Inhibitors/adverse effects , Ascites/chemically induced , Fosinopril/adverse effects , Abdominal Pain/chemically induced , Abdominal Pain/diagnosis , Abdominal Pain/therapy , Adult , Angioedema/diagnosis , Angioedema/therapy , Ascites/diagnosis , Ascites/therapy , Female , Humans , Recurrence , Tomography, X-Ray Computed
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