EREDITARY HEMORRHAGIC telangiectasia (HHT) (Online Mendelian Inheritance in Man [OMIM] #187300) is a dominantly inherited genetic vascular disorder characterized by recurrent epistaxis; cutaneous telangiectasia; and visceral arteriovenous malformations (AVMs) that affect many organs, including the lungs, gastrointestinal tract, liver, and brain. Diagnosis is based on the Curaçao criteria and is considered definite if at least 3 of 4 criteria are fulfilled. 1 The criteria are spontaneous and recurrent epistaxis, Author Affiliations are listed at the end of this article.
While these studies have primarily centered on acute UUO, the findings in this model of renal injury may potentially be transferable to other entities that are characterized by decreased renal function and increased renal fibrosis such as different forms of interstitial nephropathy or diabetic glomerulopathy.
Hepatic involvement occurs in up to 74% of patients with hereditary hemorrhagic telangiectasia (HHT) and is characterized by a spectrum of arteriovenous malformations. Three different types of intrahepatic shunting may be present: hepatic artery to hepatic veins, hepatic artery to portal vein, and portal vein to hepatic vein. Hepatic involvement in HHT may lead to biliary ischemia, portal hypertension, or high-output cardiac failure (HOCF). Orthotopic liver transplantation (OLT) has been proposed as the only definitive curative treatment. The aim of this study was to evaluate the long-term outcome of patients with hepatic involvement due to HHT after OLT with respect to mortality, cardiac and hepatic status, epistaxis, and quality of life. Patients with HHT and severe hepatic vascular malformations who underwent OLT in the Lyon Liver Transplant Unit (LLTU) from 1993 to 2007 were followed at the LLTU and the French Reference Center for HHT. Quality of life was evaluated with the Short Form 36 questionnaire. There were 13 patients who fulfilled the entry criteria of the study (12 women and 1 man). The mean age at the time of OLT was 51.8 years (range ¼ 33-65 years). Indications for OLT were cardiac failure (n ¼ 9), biliary necrosis (n ¼ 2), both cardiac failure and biliary necrosis (n ¼ 1), and hemobilia (n ¼ 1). The mean duration of follow-up was 109 months (range ¼ 1-200 months). Twelve patients (92.3%) are still alive. For the 9 patients with HOCF, the mean cardiac index decreased from 5.4 L/minute/m 2 before OLT to 3.0 L/minute/m 2 after OLT. No severe hepatic complications were observed after OLT. Nine of the surviving patients (75%) experienced dramatic improvements in epistaxis and quality of life, including an
The DCE-US methodology has been successfully provided to several centers across France together with strict rules for quality assessment. Only 3% of examinations carried out at these centers were considered not interpretable.
Toradol is a new parenteral, nonsteroidal anti-inflammatory drug which is efficacious in treating renal coli. In the present experiments, Toradol was administered to both control dogs and dogs with unilateral ureteral obstruction. In control dogs, Toradol had no effect on RBF or GFR, despite inhibition of renal prostaglandin synthesis (measured as urinary prostaglandin release). In contrast, RBF fell acutely by 35% (p < 0.001) within 15 minutes of Toradol administration in the setting of ureteral obstruction; contralateral RBF was unaffected. Ipsilateral ureteral pressure also fell. Changes in RBF and ureteral pressure, together with the known effects of NSAIDs on pain pathways, may contribute to the pain relief observed clinically with Toradol. However, the abrupt changes in renal hemodynamics brought on by Toradol to the obstructed kidney may compromise renal reserve, and Toradol should be used cautiously in treating renal colic.
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