ABSTRACT
BACKGROUND & AIMS: Hereditary haemorrhagic telangiectasia is characterized by arterio-venous malformations (AVM). It frequently involves the liver without clinical symptoms, but may lead to biliary ischaemia, portal hypertension, or fatal high-output heart failure. The indication of liver transplantation is controversial. METHODS: Herein, we report the case of a 65-year-old female patient with a 'double Osler syndrome' consisting of hereditary haemorrhagic telangiectasia (HHT) and type I hereditary angioedema diagnosed at the age of 25 and 22 years respectively. RESULTS: Hereditary angioedema was treated with danazol for several decades until multiple hypoechogenic liver masses were detected. Albeit danazol treatment was replaced by C1 esterase inhibitor infusions, hepatocellular carcinoma was diagnosed at the age of 64 and the patient was listed for liver transplantation. HHT was marked by recurrent epistaxis until the age of 63 when severe intestinal bleeding occurred. At the age of 65, severe dyspnoea (NYHA class IV) developed and rapidly progressive high-output cardiac failure was diagnosed. Despite argon plasma coagulation to control bleeding from intestinal angiodysplasia, and treatment with bevacizumab to inhibit angiogenesis, the patient died from severe gastrointestinal bleeding associated with cardiogenic shock at the age of 66 before being transplanted. CONCLUSION: The indication to list this patient for liver transplantation was debated several times before the diagnosis of hepatocellular carcinoma because of good general condition and low MELD score. Precise guidelines for screening and management of patients with hepatic HHT need to be better defined.
Subject(s)
Carcinoma, Hepatocellular/complications , Gastrointestinal Hemorrhage/etiology , Heart Failure/physiopathology , Liver Neoplasms/complications , Telangiectasia, Hereditary Hemorrhagic/complications , Telangiectasia, Hereditary Hemorrhagic/drug therapy , Aged , Angiogenesis Inhibitors/therapeutic use , Bevacizumab/therapeutic use , Fatal Outcome , Female , Humans , Hypertension, Portal/pathology , Liver Transplantation , Waiting ListsABSTRACT
Two patients developed anaphylaxis after injection of a corticosteroid preparation containing carboxymethylcellulose (E466). In both cases skin tests yielded positive immediate type reactions to pure carboxymethylcellulose. This hydrophilic derivative of cellulose has found wide application in the pharmaceutical, cosmetics and food industry. The diagnosis is based on skin testing as 9% of the normal population was found to have serum IgE antibodies to this compound. In case of anaphylaxis after injection of corticosteroids, carboxymethylcellulose in addition to corticosteroids should be included for skin testing.
