Cholangiopathy in critically ill patients surviving beyond the intensive care period: a multicentre survey in liver units.

BACKGROUND: The outcome of cholangiopathy developing in intensive care unit (ICU) is not known in patients surviving their ICU stay. AIM: To perform a survey in liver units, in order to clarify the course of cholangiopathy after surviving ICU stay. METHODS: The files of the liver units affiliated to the French network for vascular liver disease were screened for cases of ICU cholangiopathy developing in patients with normal liver function tests on ICU admission, and no prior history of liver disease. RESULTS: Between 2005 and 2015, 16 cases were retrieved. Extensive burns were the cause for admission to ICU in 11 patients. Serum alkaline phosphatase levels increased from day 11 (2-46) to a peak of 15 (4-32) × ULN on day 81 (12-511). Magnetic resonance cholangiography showed irregularities or frank stenosis of the intrahepatic ducts, and proximal extrahepatic ducts contrasting with a normal aspect of the distal common bile duct. Follow-up duration was 20.6 (4.7-71.8) months. Three patients were lost to follow-up; 2 patients died from liver failure and no patient was transplanted. One patient had worsening strictures of the intrahepatic bile ducts with jaundice. Nine patients had persistent but minor strictures of the intrahepatic bile ducts on MR cholangiography, and persistent cholestasis without jaundice. One patient had normal liver function tests. CONCLUSIONS: In patients surviving their ICU stay, ICU cholangiopathy is not uniformly fatal in the short term or clinically symptomatic in the medium term. Preservation of the distal common bile duct appears to be a finding differentiating ICU cholangiopathy from other diffuse cholangiopathies.

Right-sided endocarditis complicating peritoneovenous shunting for ascites.

We describe two patients with alcoholic cirrhosis in whom staphylococcal right-sided endocarditis developed after insertion of a peritoneovenous shunt (PVS). Massive pulmonary embolism caused early death in one patient. In the other patient, staphylococcal septicemia was cured after shunt removal and antibiotic treatment; recurrent endocarditis due to Corynebacterium xerosis ultimately caused the patient's death. No clinical manifestation of tricuspid valve dysfunction was noted in either patient, and right-sided endocarditis was recognized only at autopsy. The protracted contact of the tip of the venous line of PVS with the atrial wall is likely to be a major factor in the development of right-sided endocarditis in these patients.

Hepatocytic PAS-positive diastase-resistance inclusions in the absence of alpha-1-antitrypsin deficiency--high prevalence in alcoholic cirrhosis.

The presence of PAS-positive, diastase-resistant inclusions in the cytoplasm of the hepatocytes is characteristic of alpha-1-antitrypsin deficiency. The purpose of this investigation was to determine whether the presence of these inclusions is a specific feature, permitting the recognition of alpha-1-antitrypsin deficiency in patients with liver disease. We examined the liver specimens from 20 patients suffering from alcoholic cirrhosis with the Pi M phenotype, i.e., in whom alpha-1-antitrypsin deficiency was excluded. In seven of these patients, PAS-positive, diastase-resistant inclusions were seen in the hepatocytes; in two patients, these inclusions contained a material antigenically similar to alpha-1-antitrypsin. These inclusions might represent deposits of glycoproteins poorly excreted by the diseased hepatocytes. It is concluded that, in patients with liver disease, the presence of PAS-positive, diastase-resistant inclusions--even containing alpha-1-antitrypsin--in the cytoplasm of the hepatocytes does not permit the hepatic lesions to be ascribed to alpha-1-antitrypsin deficiency.

[Hepatitis caused by quinidine. Study of a case and review of the literature]. / Hépatite due à la quinidine. Etude d'un cas et revue de la littérature.

The authors report the case of a 63 year-old woman who developed high-grade fever with chills, nausea, diarrhea, severe pain in the right hypochondrium, and jaundice after one month's treatment with 300 mg of hydroquinidine hydrochloride daily. Serum bilirubin and aminotransferases were slightly increased, while alkaline phosphatases and gamma-glutamyl-transpeptidase serum activities were markedly raised. Histological examination of a liver specimen obtained by the transvenous route showed numerous epithelioid granulomas with giant cell formation and eosinophils in hepatic lobules and portal tracts. Symptoms disappeared three days after withdrawal of the drug, but hepatomegaly and a mild increase in serum gamma-glutamyl-transpeptidase persisted more than eighteen months. Quinidine-induced hepatitis is almost always associated with fever, and, in one-third of the cases, with a pseudo-cholangitis picture. Extrahepatic hypersensitivity manifestations are often present. Histological examination of the liver shows granulomatous or cytolytic hepatitis. Withdrawal of the drug is rapidly followed by a favorable outcome; readministration causes immediate relapse; progression to chronic liver disease has never been reported previously.

Orally administered omeprazole versus injection therapy in the prevention of rebleeding from peptic ulcer with visible vessel. A multicenter randomized study.

Endoscopic injection therapy significantly reduces the risk of bleeding relapse in patients with digestive hemorrhage due to peptic ulcers associated with a visible vessel. Profound and sustained acid inhibition by proton pump inhibitors may generate optimal conditions for clotting and prevent bleeding relapse. Over a one-year period, 52 patients presenting with digestive hemorrhage, in whom emergency endoscopy showed a peptic ulcer with a non-bleeding visible vessel, were enrolled in a multicenter randomized study comparing oral omeprazole, 40 mg per day (n = 31) vs adrenaline (1:10,000) plus polidocanol (1%) injection associated with oral ranitidine 300 mg per day (n = 21). Rebleeding occurred in 15/52 (29%) patients: 8/31 (26%) in the omeprazole group with 6 major hemorrhages (19%), and in 7/21 (33%) in the injection group with 3 major hemorrhages (14%); the differences were not significant. No difference was observed between omeprazole and injection group in terms of volume of transfused blood (2.03 +/- 1.5 vs 3.1 +/- 0.9 blood units), need for hemostatic surgery (9.6% vs 14.3%), mortality (19.3% vs 14.3%) and mean hospital stay (11.5 days both groups). This study suggests that oral omeprazole, 40 mg per day, has an efficacy comparable to injection therapy in reducing the bleeding relapse from non bleeding peptic ulcers associated with visible vessel.

[Primary exocrine pancreatic insufficiency in the adult. Presentation of a case]. / Insuffisance pancréatique exocrine primitive de l'adulte. Présentation d'un cas.

The authors report the case of a 37-year-old female complaining of chronic diarrhea since childhood. She had gross steatorrhea and azotorrhea . Pancreatic function tests using secretin and cholecystokinin-pancreozymin stimulation, and analysis of pure pancreatic juice collected under secretin stimulation proved exocrine pancreatic insufficiency. Oral glucose tolerance test was normal. Intestinal absorption tests, small bowel X-ray films and jejunal histology were normal. Brush-border specific enzyme activities were increased. In vitro palmitic acid uptake and esterification in the jejunal mucosa were decreased. There was no pancreatic calcification. Biliary tract was normal. Retrograde pancreatography, pancreatic ultrasonography and tomodensitometry showed no morphologic abnormality. No cause of exocrine pancreatic insufficiency could be detected. A son and a niece of the proband had chronic diarrhea and growth failure. The relationship of the described case with primary lipomatosis of the pancreas in childhood and hereditary exocrine pancreatic insufficiency in CBA/J epi mice is discussed.

[Portal vein thrombosis and hereditary protein C deficiency. Presentation of a case and review of the literature]. / Thrombose portale et déficit héréditaire en protéine C. Présentation d'un cas et revue de la littérature.

The authors report the case of a 51 year-old man, without any personal or familial history of thromboembolism, presenting with abdominal pain. Portal vein thrombosis was demonstrated by ultrasonography and arteriography. The patient had neither esophageal varices or congestive gastropathy. No cause for portal vein thrombosis was detected. Type I protein C deficiency was demonstrated in this patient as well as in his asymptomatic sister. The presence of a (fortuitously?) associated increase in platelet aggregability initially led to a trial regimen of aspirin (300 mg per day); abdominal pain resolved, and a partial regression of portal vein thrombosis was demonstrated on ultrasonograms six months later; no further complications occurred during the 4-year follow-up period. The 13 previously published cases of protein C deficiency-associated portal vein thrombosis are reviewed.

[Irregular hepatic steatosis. Ultrasonic and computed x-ray tomographic aspects]. / Stéatose hépatique irrégulière. Aspects échographiques et tomodensitométriques.

A 39-year-old moderately obese and alcoholic man complained of abdominal pain and nausea. An ultrasound examination of the liver showed large hyperechoic areas surrounding a limited pseudotumorous zone of relatively decreased echogenicity. CT scan showed a decreased density (35 UH) in the hyperechoic areas, suggesting fatty liver which was confirmed histologically; conversely, the hypoechoic area appeared normal on CT scan. Six months later, after reduction of caloric intake and cessation of alcohol ingestion, ultrasound examination and CT scan of the liver were normal.

[Anti-Gram-negative bacterial antibodies in alcoholic cirrhosis. Study of 58 patients]. / Les anticorps anti-bacille à Gram négatif au cours de la cirrhose alcoolique. Etude de 58 malades.

Gram-negative bacterial infections are frequent and severe in cirrhotic patients. Existence of endotoxemia in cirrhosis is controversial. The demonstration of Gram-negative bacterial antibodies could be an alternative approach to the pathogenic role of these bacteria. In 58 patients with alcoholic cirrhosis, the immunoglobulin G specifically directed against the Gram-negative bacteria lipopolysaccharide expressed by the J5 mutant of Escherichia coli 0111:B4 was measured. Antibody titres were compared to those of a control group of blood donors. The distributions of antibody titres were similar in cirrhotic patients and in control subjects. No correlation was found between antibody titres and biological parameters of liver function. These results seem to confirm previous reports on the absence of latent endotoxemia in cirrhotic patients, and they suggest that antibody production against Gram-negative bacteria lipopolysaccharides is not enhanced in these patients.

[Asymptomatic cystic dilatation of the choledochus in an adult]. / Dilatation kystique asymptomatique du cholédoque chez un adulte.

A congenital choledochal cyst, type Ia according to Todani's classification, was discovered fortuitously in a 54-year old man by abdominal sonography. The patient had no biliary symptoms, and liver tests were normal. Excision of the cyst was performed because of the theoretical risk of cholangiocarcinoma. Recovery was uneventful.

[Hepatitis caused by clometacin (Dupéran). Retrospective study of 30 cases. A model of autoimmune drug-induced hepatitis?]. / Hépatites à la clométacine (Dupéran). Etude rétrospective de 30 cas. Un modèle d'hépatite auto-immune médicamenteuse?

Thirty cases of clometacin-induced hepatitis were retrospectively collected over a nine-year period in hepatogastroenterological units of non university, public hospitals. There was a strong female predominance (90 percent). Clometacin (Dupéran) was taken because of arthritis in 8 out of 10 cases. Administration was continuous in 85 percent of cases and median duration was 445 days. median dose was 450 mg per day. Jaundice, fatigue, and weight loss were the most frequent symptoms, but edema, ascites and palmar erythema were not uncommon. Thrombopenia (38 percent) was the most frequent hematologic abnormality. Renal failure, always with benign course, was present in 1/4 of cases. Biochemical disorders indicated hepatocellular and cholestatic hepatitis in 3/4 and 1/4 of cases respectively. Hypoprothrombinemia below 50 percent was noted in 1 out of 6 cases, and was associated with death in half cases. Gamma-globulins were increased in 80 percent of cases, with a predominant increase of IgG. Antinuclear or anti-smooth muscle antibodies were present in 60 percent of cases, whereas antimitochondrial and antimicrosomes were absent. Histopathological examination of the liver biopsy specimens obtained in 25 patients showed acute hepatitis in 8 and chronic active hepatitis with fibrosis in 17--including 6 patients with cirrhosis; there were no epidemiological, clinical (except ascites), or biochemical differences between these two groups. Four of the 7 patients tested had HLA B8 antigens; they all had chronic active hepatitis, with autoantibodies in 3 cases. Median duration of hospitalization was 21 days. Hepatitis was directly responsible for death in 3 patients; biochemical sequelae (hypergammaglobulinemia or anicteric cholestasis) were present in 8 patients, 2 of whom most likely had cirrhosis.(ABSTRACT TRUNCATED AT 250 WORDS)

[Acute hepatitis caused by isaxonine phosphate (Nerfactor)]. / Hépatite aiguë due au phosphate d'isaxonine (Nerfactor).

We report the cases of four adult patients suffering from acute hepatitis due to isaxonine phosphate (Nerfactor), a drug recently proposed for the treatment of the lesions of peripheral nerves. Hepatitis developed 14 to 166 days after the beginning of the administration of the drug. In all the patients, predominantly centrilobular hepatocytic necrosis was present. In two of our patients, the course of hepatitis was fatal. Hepatitis induced by isaxonine phosphate is likely to be due to an immuno-allergic mechanism.

[Treatment of duodenal ulcer outbreak with omeprazole. Results of a multicenter non-controlled study]. / Traitement de l'ulcère duodénal en poussée par l'oméprazole. Résultats d'une étude non contrôlée multicentrique.

Sixty-one patients with symptomatic endoscopically confirmed duodenal ulcer, 5 mm or more in diameter, were treated with omeprazole 30 mg once daily in the morning for 4 weeks. Complete ulcer healing was assessed by endoscopy performed after 15 and, if necessary, 29 days of treatment. The healing rate as 83 p. 100 (49/59 patients) after 2 weeks, and 98 p. 100 (58/59 patients) after 4 weeks. Only 5 patients remained symptomatic on day 15. Fifty-six patients did not take any antacid during the course of treatment. There were few adverse effects. No clinically significant changes in laboratory parameters were observed. These results confirm: the remarkable efficiency of omeprazole in short-term treatment of duodenal ulcer, the good tolerance to this drug, at least in short duration treatment.

[Hepatitis due to ajmaline. Report of cases and review of the literature]. / Hépatite à l'ajmaline. Description de 4 observations et revue de la littérature.

The authors report the cases of 4 patients with jaundice following the administration of ajmaline. The disease had a pseudo- angiocholitic onset with fever, chills and pruritus in the 4 patients and abdominal pains in 2 patients. Serum transaminase activity and serum alkaline phosphatase activity were increased in the 4 patients. Blood eosinophilia was found in 3 patients. Liver lesions included predominantly centrilobular cholestasis, mild hepatocytic lesions, and portal inflammation. After the interruption of the drug administration, recovery occurred in the 4 patients. Two patients resumed the intake of ajmaline; transient hepatitis recurred in these 2 patients.

[Natural history of non alcoholic and non familial chronic pancreatitis. Results of a multicentre study]. / Evolution de la pancréatite chronique non alcoolique et non familiale.Résultats d'une étude multicentrique.

UNLABELLED: The natural history and complications of non alcoholic chronic pancreatitis (NACP) is poorly understood compared to that of alcoholic chronic pancreatitis (ACP). PATIENTS AND METHODS: From April 1993 to April 1996, 77 patients with NACP were prospectively evaluated in 17 French centres. This population was compared to a cohort of 417 patients with ACP. RESULTS: No significant difference was observed with respect to mean age between NACP and ACP (43 +/- 20 vs 44 +/- 11 years, respectively). The median patient follow-up time was also comparable: 7 years (1-28) and 6 years (1-34) respectively for NACP and ACP. There were significantly more males in the ACP group (9/1 in ACP group and 1.3/1 in NACP group; P<10(- 7) ). Patients with NACP were less likely to have calcifications (58% vs 77%; P=0.01), pseudocysts (19 vs 47%, P<0.001), portal vein thrombosis (5 vs 16%, P<0.02). Importantly, patients with NACP required less surgical procedures than those with ACP (26% vs 44%, P=0.004). The actuarial death rate at 15 years was 0% in the NACP group compared to 20.5% in those with ACP (no CP related death). CONCLUSION: NACP has a less severe disease progression, fewer complications and requires less surgical interventions than ACP. The lower actuarial survival rate in patients with ACP correlates with the extra-pancreatic complications encountered in patients with alcohol related diseases and not with the evolution of CP itself.

[Collagenous colitis, IgA deficiency, Basedow's disease and atrophic gastritis]. / Colite collagène, déficit en IgA, maladie de Basedow et gastrite atrophique.

In a 37-year-old woman with chronic watery diarrhea of three years duration, the diagnostic of collagenous colitis was established by optical and ultrastructural examination of rectal and colonic biopsies. No other cause of diarrhea could be found. Moreover, this patient had also selective IgA deficiency, Grave's disease and chronic atrophic gastritis of auto-immune type. Sequential treatments with loperamide, cholestyramine and antibiotics did not modified diarrhea which improved with salazosulfapyridine and betamethasone enemas. These observations suggest that collagenous colitis might be a part of the spectrum of enteropathies associated with immunoglobulin deficiencies.

[Icteric complications related to pregnancy vomiting in the 1st trimester (hyperemesis gravidarum). Review of the literature apropos of a case]. / Les complications ictériques liées aux vomissements gravidiques du premier trimestre (Hyperemesis gravidarum). Revue de la littérature à propos d'un cas.

The authors report a case of jaundice occurring secondary to severe vomiting in pregnancy in the first trimester. This is a rare clinical entity (occurring in 0.2-3 cases per thousand) although some people say it does not exist. The physiopathology is still badly understood but it occurs most often between the fourth and eighth week of amenorrhoea. It is characterised by jaundice occurring secondarily to vomiting in the first trimester, with a rise in the bilirubin level in the blood and in the alkaline phosphatases. Sometimes there is a slight rise in the transaminases. The diagnosis can only be arrived at after having excluded all the other possible causes of jaundice which are mentioned in the text. Furthermore the way the condition progresses is an important argument for its aetiology because once vomiting ceases the jaundice goes. The treatment has to be symptomatic with correction of the dehydration.

[Acute rhabdomyolysis in alcoholic patients]. / Rhabdomyolyse aiguë chez des malades alcooliques.

Six episodes of acute rhabdomyolysis were observed within a two-year period in 5 male adult alcoholic patients hospitalised in a hepatology intensive care unit. Painful muscle swelling was discrete of absent in 4 of the 5 patients. Acute rhabdomyolysis was preceded by grand mal seizures in 4 patients, delirium tremens in 1 and high fever with shivers in 3. All cases were rapidly diagnosed on the finding of very high serum creatinine phosphokinase levels. One patient developed acute respiratory failure and recovered after prolonged mechanical ventilation. Three patients had acute renal failure with severe hyperkalemia in one but none required dialysis. Three patients died within 2 to 6 days of the diagnosis, but the deaths were not directly related to rhabdomyolysis. It would appear that in alcoholic patients: the prevalence of rhabdomyolysis is probably underestimated; any muscular hyperactivity as seen in seizures, delirium tremens and prolonged shivers may be a precipitating factor; the condition is easily diagnosed by measuring serum creatinine kinase activity; some cases of acute renal failure in patients with alcoholic cirrhosis might be explained by acute rhabdomyolysis with minimal symptoms.

[Rapid progress of cirrhosis in hepatitis C: the role of age at the time of viral contamination]. / Rapidité d'évolution vers la cirrhose après hépatite C: influence de l'âge au moment de la contamination par le virus.

OBJECTIVE: The aim of this study was to assess predictive factors for the progression to liver cirrhosis in hepatitis C. METHODS: One hundred thirty six patients (79 men; 57 women; mean age 39 years) with transfusion or intravenous drug use-associated hepatitis C virus (HCV) infection were studied. Sex, cause of infection, duration of contamination, and genotype were studied as predictive factors of progression to liver cirrhosis. RESULTS: One hundred twenty three patients presented with chronic hepatitis without cirrhosis and 13 had cirrhosis. At the time of liver biopsy, rates of cirrhosis were: 0% before 40 years, 10% between 40 and 60 years, and 47% after 60 years. (p < 0.05). Rates of cirrhosis according to the age at the time of contamination were as follows: 3% before 30 years; 16% between 30 and 50 years; 46% after 50 years even though duration of the disease was comparable in the three groups. In multivariate analysis, two independent factors were associated with liver cirrhosis: age at contamination and duration of infection. CONCLUSION: Duration of infection and especially age at contamination seem better correlated with the probability of cirrhosis than the route of transmission or the genotype 1b. The results of this study suggest that progression to cirrhosis is slower in cases of contamination before 30 years of age than later on. Age at the time of contamination is an important predictive factor of progression to cirrhosis.

[Physiopathology of inflammatory bowel diseases]. / Physiopathologie des maladies inflammatoires de l'intestin.

The author presents a brief review of the main pathophysiological hypotheses put forward to explain the formation of lesions of inflammatory bowel diseases (ulcerative colitis and Crohn's disease). No infectious or alimentary cause has been found. Immunological abnormalities are numerous, but none of them seems to be primary. The effector role of inflammation mediators is better known and leads to new developments in therapeutics. The study of healthy relatives is an interesting means of looking for abnormalities that pre-exist the disease, as seems to be the case with intestinal permeability. The french contribution of fundamental research on inflammatory bowel diseases is insufficient and must be stimulated.