In-111 transferrin scintigraphy in cirrhosis with hypoalbuminemia: evidence for protein-losing enteropathy in a small group of selected cases.

BACKGROUND: Hypoalbuminemia commonly observed in cirrhosis is considered to be mainly related to hepatocellular dysfunction. However, the correlation between the decrease in serum albumin and liver function is far from linear and arguments in favor of an additive role of protein-losing enteropathy have been brought by a few studies. AIM: To assess the potential role of protein-losing enteropathy in a group of patients with cirrhosis, portal hypertension, and hypoalbuminemia. DESIGN AND METHODS: Eleven patients with documented cirrhosis, portal hypertension, and a low serum albumin level compared to liver function underwent an (111)In-transferrin scintigraphy. RESULTS: Using this sensitive method of investigation, nine exhibited features suggestive of exudative enteropathy. Serum albumin level and digestive protein loss were even correlated (Pearson's coefficient = -0.529, one-sided p = 0.047). Protein loss were however not correlated with the degree of portal hypertension or with the extent of liver dysfunction evaluated by the aminopyrine breath test. CONCLUSIONS: Our preliminary data obtained in a small group of selected patients with cirrhosis, portal hypertension, and hypoalbuminemia indicate that protein-losing enteropathy may represent an appreciable and underestimated cause of hypoproteinemia.

Ulcerative colitis associated with IgG4 cholangitis: similar features in two HLA identical siblings.

BACKGROUND: IgG4-associated cholangitis (IAC) can mimic primary sclerosing cholangitis although, in contrast to the latter, it is highly responsive to steroid therapy. IAC is known to be associated with autoimmune pancreatitis and has also been shown to be part of a more complex autoimmune IgG4 syndrome. However, an association with inflammatory bowel disease (IBD), a condition in which its identification may have therapeutic and prognostic importance, has not yet been described. CASE REPORTS: We report the cases of two HLA identical siblings both DRB *1501 positive exhibiting features of IAC together with ulcerative colitis. Subsequent high resolution HLA typing performed by sequence-based-typing showed similar alleles in both siblings: A *0301 A *3201 B *07 (0702/62) B *1401 C *0702 C *0802 DRB1 *1501. There is indirect evidence that this hitherto undescribed association, likely to be strongly linked to a genetic background, might account for a proportion of the cases of cholangitis associated with IBD. CONCLUSION: Appropriate investigation for IBD-associated cholangitis is mandatory to identify IAC, the recognition of which has particular therapeutic and prognostic implications.

Severe hepatitis associated with the use of black cohosh: a report of two cases and an advice for caution.

In spite of the fact that severe side effects have been reported, black cohosh [Actaea racemosa (syn. Cimifuga racemosa)] is likely to be one of the most popular herbs used in the treatment of postmenopausal symptoms and menstrual dysfunction. We report the cases of two patients, one with submassive liver necrosis and the other with chronic hepatitis most likely related to the use of two different preparations containing black cohosh. This represents another advice for caution concerning this popular preparation of inconsistent therapeutic value.

Liver transplantation for nonsteroidal anti-inflammatory drug-induced liver failure: nimesulide as the first implicated compound.

Among industrialized countries, the rate of drug-induced liver failure varies widely accounting for about 1-12% of the indications for liver transplantation. Nonsteroidal anti-inflammatory drugs (NSAIDs) are with antibiotics the most frequently involved compounds. In this single-center series of 57 consecutive cases of acute liver failure treated by orthotopic liver transplantation, five were related to NSAIDs-induced liver damage, three being due to nimesulide use. This has to be taken as a further warning about the potential for this compound to induce liver failure.

Nifuroxazide-induced acute pancreatitis: a new side-effect for an old drug?

We report the case of a middle-aged woman who developed a typical picture of acute pancreatitis together with systemic features of immunoallergy after the intake of two capsules (200 mg) of nifuroxazide. Even if acute pancreatitis is a rare adverse event of nitrofuran derivative therapy, nifuroxazide-induced pancreatitis as not been previously described. As suggested by associated systemic features, the disease is likely of immunoallergic origin.

Chronic stomal variceal bleeding after colonic surgery in patients with portal hypertension: efficacy of beta-blocking agents?

In patients with portal hypertension, ileostomy or colostomy carries the risk of the development of stomal varices at the site of the mucocutaneous junction of a stoma. Such varices are often the source of difficult-to-treat recurrent or chronic bleeding. In this setting, transjugular intrahepatic portosystemic shunt insertion and embolisation is considered the best therapeutic approach in spite of relatively high mortality and morbidity rates. We report the cases of three consecutive patients with portal hypertension of various causes and chronic stomal variceal bleeding in whom beta-blocking therapy resulted in the drying up of bleeding and the prevention of its recurrence for periods of time ranging between 2 and 42 months.

Hereditary hyperferritinaemia-cataract syndrome: a challenging diagnosis for the hepatogastroenterologist.

Hereditary hyperferritinaemia-cataract syndrome (HHCS) is a relatively rare disorder with an autosomal dominant trait. It can be caused by various mutations within the iron responsive element (IRE) of the L-ferritin gene. These mutations result in an increased translation of L-ferritin mRNA and consequently the accumulation of L-ferritin in different fluids and tissues. HHCS patients present with an isolated hyperferritinaemia in the absence of any sign of iron overload. Early onset bilateral cataract, probably due to accumulation of ferritin crystals in the lens, is the only presenting clinical manifestation. Internists, especially gastrohepatologists, should be aware of this syndrome and differentiate it from haemochromatosis which is much more frequent, in order to avoid unnecessary imaging procedures, liver biopsies and an eventual venesection therapy, which will only lead to microcytic anaemia. In the present paper we report the first cases with HHCS diagnosed in Belgium. At diagnosis, the seven known affected members of three different families had ferritin levels between 603 and 3432 microg/l (normal < 150 microg/l), and this in combination with normal iron and transferrin values. All of them were known with early-onset bilateral cataract and our postulated diagnosis of HHCS was confirmed after genetic sequencing of the L-ferritin gene, which showed a C39U point mutation in the first family, and an A40G point mutation in the IRE loop segment in the two other families. The other investigated members of the three families had normal ferritin values, no history of early-onset cataract and genetic analyses could not reveal a mutation in the IRE of their L-ferritin gene. In current clinical practice, gastroenterologists should remember that elevated ferritin levels in the absence of documented iron overload is not haemochromatosis.

Bile duct disorders.

Drug-induced bile duct injury related prolonged or chronic cholestasis is recognized as a common side effect of treatment with several drugs. The severity and duration of the clinical symptoms suggest that this increase in number of reports is not only related to clinician and pathologists being increasingly aware of the condition, but also may represent a true increase in incidence likely related to a time-related growing experience with newer drugs. This clinical presentation encompasses a wide variety of features that may be the source of diagnostic difficulties, especially in the cases where cholestasis occurs days or weeks after the completion of therapy. Even more puzzling is the initial picture of hepatocholangitis, which may be silent and ensuing bile duct paucity with chronic anicteric cholestasis may be another source of diagnostic difficulties in the long-term. These diagnostic difficulties suggest that some of the cases of the so-called "idiopathic adulthood ductopenia" may originate from overlooked drug induced vanishing bile duct syndrome. The pathogenesis of the syndrome remains largely unknown and the determinants of prognosis and outcome. From reproducible data obtained in different studies investigating HLA-dependent predisposition, one may assume that genetics plays a major role even if other unknown additive factors are also likely involved. Severity of initial hepatocholangitis is likely to represent another important determinant of severity and prognosis, however to be assessed in larger longitudinal studies. Therapy of large bile duct injury mimics that of primary sclerosing cholangitis. Treatment of small bile duct injury remains disappointing. Corticosteroids are invariably ineffective. Ursodeoxycholic acid as been shown to induce improvement of clinical and biochemical cholestasis in some selected cases, its efficacy being however unpredictable. Preliminary data about the natural history of the vanishing bile duct syndrome suggest that therapy might be more effective when initiated early.

Long-term asymptomatic biochemical cholestasis after fulminant or subfulminant liver failure is associated with extensive postnecrotic collapse with regeneration of the liver.

Fulminant or subfulminant liver failure usually leads to liver failure or to recovery. In rare instances, patients who recover exhibit prolonged asymptomatic biochemical cholestasis which coincides with the development into the parenchyma of large postnecrotic collapse with regeneration. This hitherto poorly recognized form of recovery may now be assessed by noninvasive techniques such as magnetic resonance imaging. We report the case of three patients who recovered from fulminant or subfulminant liver failure and in whom investigation of long-term biochemical cholestasis led to that unusual diagnosis.