Epidermolysis bullosa acquisita and Crohn's disease. A case report with immunological and electron microscopic studies.

A review of the literature shows that the association of epidermolysis bullosa acquisita and Crohn's disease is rare. A 27-year-old man developed cutaneous blisters on the trauma-prone areas that were consistent with the diagnosis of epidermolysis bullosa acquisita. Immunological and electron-microscopic studies of the skin showed intense IgG deposits located beneath the basal lamina. Three years later, Crohn's colitis was diagnosed. Prednisolone and sulfasalazine treatment resulted in an improvement of the bowel disease but without appreciable effect on the skin lesions.

Idiopathic retroperitoneal fibrosis and primary biliary cirrhosis. A new association?

We encountered a case of primary biliary cirrhosis in a nonalcoholic man who had been operated on for idiopathic retroperitoneal fibrosis 20 years previously. Chronic pancreatitis was also detected on endoscopic retrograde examination. After several episodes of digestive bleeding due to ruptured esophageal varixes, the patient died of massive hemorrhage. Postmortem examination showed stage 3 primary biliary cirrhosis and a thick retroperitoneal fibrous plaque, consisting of densely fibrotic areas of collagen with rare vessels and mononuclear cells. We suggest that idiopathic retroperitoneal fibrosis may be a new autoimmune disorder associated with primary biliary cirrhosis and that primary biliary cirrhosis is a potential cause of portal hypertension, cholestasis, or both in the course of idiopathic retroperitoneal fibrosis.

Fibrosing alveolitis and hepatitis B surface antigen-associated chronic active hepatitis in a patient with immunoglobulin A deficiency.

Fibrosing alveolitis is described in a 22 year old woman with immunoglobulin A (IgA) deficiency and hepatitis B surface antigen (HBsAg)-associated chronic active hepatitis. At lung biopsy HBsAg was detected by indirect immunofluorescence in the alveolar space but not in the septal fibrosis. We discuss the possible relationships between IgA deficiency on the one hand, and HBsAg-associated lung and liver diseases on the other hand.

A randomised controlled study of propranolol for prevention of recurrent gastrointestinal bleeding in patients with cirrhosis.

74 cirrhotic patients with a history of variceal or gastric bleeding were randomly assigned to treatment with propranolol (40 to 360 mg/day) or placebo. The patients were all in good condition and doses of propranolol were titrated until a 25% reduction in heart rate was achieved. After 2 years, the cumulative percentage of patients free from rebleeding was significantly greater among the patients receiving propranolol (79%) than in the placebo group (32%; p less than 0.0001). Similarly, the percentage of surviving patients was significantly greater with propranolol (90%) than with placebo (57%; p less than 0.02) after 2 years. It was concluded that in cirrhotic patients in good condition, propranolol reduced both the risk of recurrent gastrointestinal haemorrhage and the mortality rate during a 2-year period of continuous administration of the drug.

Direct blotting electrophoresis for sequencing and genotyping hepatitis C virus.

Many methods have been used to differentiate the hepatitis C virus (HCV) genotypes based on, for example, type specific primers, probes and restriction fragment length polymorphism. However, determination of the nucleotide sequence remains the reference. Therefore, a simple non-radioactive cycle sequencing technique was developed for clinical tests. PCR-amplified products of the 5' non-coding region (from position -274 to -31) were sequenced using a 5' digoxygenin-labeled primer. After denaturation, the samples were loaded on a direct blotting electrophoresis system (GATC 1500). Sequencing products were blotted onto a nylon membrane during the electrophoresis. The DNA fragments were then UV-cross-linked, incubated with phosphatase-labeled anti-digoxygenin antibody and stained with a precipitating substrate. Reading the sequence of six samples were possible within 2 days. In 41 different samples, five different genotypes were found by sequence analysis from position -245 to -69, of which 17 were type 1a, 7 type 1b, 5 type 2a, 8 type 3a, 3 type 4 and 1 type 5. These results agreed with those obtained by reverse hybridization assay. Direct blotting electrophoresis offered a good non-radioactive method of performing clinical sequencing on a medium scale, with a minimum of investment.

Fibrotic stricture of the extrapancreatic biliary tract: a new complication of acute pancreatitis. Two cases.

Two cases of fibrotic stricture of the extrapancreatic common bile duct were observed 3 and 5.5 months after severe acute alcoholic pancreatitis. The diagnosis was made by endoscopic retrograde cholangiography in both cases. Although colonic or ureteric stenosis have been reported after acute pancreatitis, this is the first report of extrapancreatic biliary stricture occurring after acute pancreatitis. The strictures could have arisen by either an enzymatic or ischemic mechanism. Outcome was favorable after surgical hepaticojejunostomy.

Bleeding cecal typhoid ulcer. Value of emergency angiography for selective surgical hemostasis.

A case of bleeding cecal typhoid ulcer in a 22-year-old patient demonstrated by superior mesenteric arteriography is described. An attempt to stop hemorrhage using intraarterial vasopressin infusion, failed. However, arteriography proved helpful in locating the bleeding site, and in permitting conservative surgery, namely cecal ulcer suture to achieve hemostasis. On the basis of this case and a review of the literature, management of complicating hemorrhage resulting from typhoid fever is discussed, with particular reference to radiological procedures.

Autovagotomy by esophageal carcinoma. An unusual cause of gastric retention without outlet obstruction.

A case of gastric retention without outlet obstruction in a 44-year-old patient with esophageal carcinoma is reported. Radiological (and particularly C.T.) examinations support the opinion that the gastric motility disorders were due to the autovagotomy secondary to malignant involvement of the intrathoracic vagus nerve.

[Incidence of esophageal cancer synchronous with upper aerodigestive tract cancers (100 cases): value of vital staining with lugol and toluidine blue]. / Fréquence du cancer de l'oesophage synchrone des cancers des voies aéro-digestives supérieures (100 cas): intérêt des colorations vitales au lugol et au bleu de toluidine.

A number of studies have demonstrated a high incidence of synchronous or metachronous esophageal carcinoma in association with carcinoma of head and neck. Carcinoma of the esophagus must be systematically looked for before the treatment of head-neck carcinomas and during follow-up. The aim of this study was to determine the incidence of synchronous esophageal carcinoma in patients with head and neck carcinoma and to evaluate the advantages of lugol and toluidine blue vital staining in fiberoptic endoscopy. One hundred patients (97 males and 3 females, mean age 54.9 years) were studied. A fiberoptic esophagoscopy was performed in all patients. Vital staining was realized with 5 p. 100 lugol in 40 cases and with 1 p. 100 toluidine blue e in 20 cases. Squamous cell carcinoma of the esophagus was observed in 12 patients, typical grossly in 5 cases and occult in 7 cases. In these latter cases, lugol (2 cases) or toluidine blue (5 cases) stain facilitated the forceps biopsies. Histological examination was positive in all cases. The incidence of esophageal carcinoma synchronous to carcinoma of the mouth was high (35.3 p. 100). Lugol vital staining seems to be sensitive, non-specific and easy to realize. Toluidine blue staining calls for a more difficult and prolonged technique. Although it can reveal occult carcinoma, false positive or negative results may be observed.

[Acute hepatopathy caused by amiodarone. Study of a case and review of the literature]. / Hépatopathie aiguä due à l'amiodarone. Etude d'un cas et revue de la littérature.

The authors report the case of a non-alcoholic 73-year-old man, treated for arrhythmia with amiodarone for 2 months, and hospitalized because of jaundice and hepatomegaly. There was an important increase in serum alkaline phosphatase activity (4 times the normal value) and a moderate increase in the serum activity of transaminases (3-4 times the normal value). Endoscopic retrograde cholangiography was normal. Serum markers of virus B and tissue antibodies were absent. Histological examination of a liver specimen disclosed portal and periportal fibrosis, mixed inflammatory infiltrate of the portal spaces, and ductular proliferation. Lamellar lysosomal inclusions were demonstrated on electron microscopy. Outcome was favorable after withdrawal of amiodarone. This report, as well as the 7 previously published cases, cannot explain the pathogenesis of amiodarone-induced liver changes.

[Ultrasound study of gallbladder motility in healthy subjects. Reproducibility of the method and effect of alcohol]. / Etude échographique de la motricité vésiculaire du sujet sain. Reproductibilité de la méthode et effet de l'alcool.

The protective effect of alcohol against cholesterol cholelithiasis has been established in several epidemiologic studies. An impairment of gallbladder motility in gallstone disease has been demonstrated in animals and in man. At a daily dose of 39 g, alcohol reduces the lithogenic index of bile, but its effect on gallbladder motility is still debated. To test this potential mechanism, the effect of 20 g of alcohol on gallbladder motility was studied, using an ultrasonographic ellipsoid method in 16 healthy male subjects. The stimulus for gallbladder contraction was a Lundh test meal. Using a cross over method, this meal was ingested by each subject once with water and once with alcohol. A third set of measurements was taken in each subject after ingestion of a Lundh meal and water to test the reproducibility of the sonographic method. The gallbladder kinetics were studied for 90 minutes following ingestion of the test meal and beverage. Alcohol stimulated rapid post prandial gallbladder emptying, and accelerated gallbladder filling. This second action could result from sphincter of Oddi pressure enhancement and, perhaps, decrease of gallbladder absorption by Na+ K+ ATPase inhibition. The reproducibility of the method was good. With a decrease of lithogenic bile index, the protective effect of alcohol against biliary cholesterol cholelithiasis could be due either to stimulation of gallbladder emptying and/or acceleration of gallbladder filling.

[Puncture of solid tumors of the liver with large caliber needle guided by ultrasonography. Study of 70 punctures]. / Ponction des tumeurs solides du foie à l'aiguille de gros calibre guidée par l'échographie. Etude de 70 ponctions.

Seventy specimens of solid hepatic tumors were obtained for histologic examination with a 18 G (1.2 mm) diameter needle under real-time ultrasound control. Overall sensitivity was 95.6 p. 100 while specificity was 100 p. 100. Distinction between primitive and secondary malignant tumor was possible in 91 p. 100 of cases. Transient hemorrhage at the puncture site occurred in one patient. These results attest to the superiority of guided puncture with a needle of sufficient caliber, thus allowing for correct histologic study. Side effects did not occur more frequently than with fine caliber needles.

[Portal hypertension caused by intra-hepatic block during chronic lymphoid leukemia]. / Hypertension portale par bloc intra-hépatique au cours d'une leucémie lymphoïde chronique.

Portal hypertension in chronic lymphocytic leukemia is rare. A 66 year-old man was admitted for splenomegaly, thrombopenia and cholestasis. Endoscopy showed esophageal varices. The hepatic venous pressure gradient was 15 mmHg. The liver biopsy showed dense leukemia cells in sinusoidal and portal sites. After splenectomy, the hepatic venous pressure gradient normalized, but esophageal varices and cholestasis persisted. The authors discuss the mechanisms of portal hypertension in chronic lymphocytic leukemia. Previously reported cases are summarized.

[Effect of acute administration of acamprosate on the risk of encephalopathy and on arterial pressure in patients with alcoholic cirrhosis]. / Effet de l'administration aiguë d'acamprosate sur le risque d'encéphalopathie et sur la pression artérielle chez les malades atteints de cirrhose alcoolique.

To evaluate the risk of hepatic encephalopathy and arterial hypotension in cirrhotic patients after acute administration of acamprosate, a GABA mimetic drug used in the weaned alcoholic, a randomized double-blind trial was conducted in 24 cirrhotic patients with low or moderate hepatic insufficiency (Pugh grade A or B). Twelve patients received 666 mg (2 tablets) of acamprosate and 12 received placebo. The 2 groups were similar before treatment, except for a male predominance in the acamprosate group. Tested parameters were the P100 latency of visual evoked potentials using a checkerboard pattern reversal as stimulus, the number connection test and the arterial blood pressure in upright and recumbent positions. The two first parameters were studied before and 2 hours after treatment. Blood pressure was recorded every half hour during 6 hours. No significant effect on the development of subclinical hepatic encephalopathy was noted. Nevertheless, even if some authors disagree with the GABA hypothesis of hepatic encephalopathy, it is possible that the dose was too low to induce subclinical hepatic encephalopathy. A study with more prolonged treatment could be necessary to be sure of the drug's safety in these patients. On the other hand, a transient decrease of diastolic arterial blood pressure was observed without significant systolic blood pressure modification. These results suggest that a moderate dose of acamprosate does not induce subclinical encephalopathy, but transient diastolic hypotension.

[Duodenal hematoma, acute pancreatitis, and hemoperitoneum after endoscopic hemostasis for duodenal ulcer]. / Hématome duodénal, pancréatite aiguë et hémopéritoine après hémostase endoscopique d'un ulcère duodénal.

Therapeutic endoscopy is followed by complications in less than 5% of cases. We report a case of an intramural duodenal hematoma after local endoscopic injection of 28 mL of adrenaline 1/10,000 for a bleeding duodenal peptic ulcer. This hematoma was associated with acute pancreatitis and was revealed by a hemoperitoneum.

[Increased fasting gallbladder volume: a new ultrasonic sign of cirrhosis?]. / L'augmentation du volume vésiculaire à jeun: un nouveau signe échographique de cirrhose?

Ultrasound was used to calculate fasting gallbladder volume in three groups of patients: 90 with cirrhosis (alcoholic in 75 cases), 41 with non cirrhotic liver disease (alcoholic in 14 cases), and 38 controls. Gallbladder volume was evaluated according to sex, age, alcoholism, presence of gallstones, time of diagnosis, and biological tests of hepatocellular function. Mean fasting gallbladder volume was significantly higher in cirrhotic patients (45.89 +/- 32.65 ml, m +/- 1 SD) than in patients with non cirrhotic liver disease (25.31 +/- 14.08 ml) and in control subjects (21.28 +/- 10.30 ml) (P less than 0.001), but there was a great overlap between individual results in each group. No relationship was found between gallbladder volume and all clinical and biological tested parameters, except for decreased prothrombin time (P less than 0.02). Further studies are necessary to consider this ultrasound sign as an useful diagnostic tool in cirrhosis.

[Cavernous lymphangioma of the liver. A report of 2 cases and review of the literature]. / Lymphangiome caverneux du foie. Etude de 2 cas et revue de la littérature.

The authors report 2 cases of hepatic lymphangioma observed in a 54-year-old woman, and in a 39-year-old man. These tumors were discovered upon ultrasound examination performed for jaundice due to viral hepatitis, and for abdominal right upper quadrant pain respectively. Computed tomography and angiography showed hypervascularized tumors. Diagnosis was established through surgical biopsy specimens. The clinical course was uneventful, during respectively the 9- and 3-year follow-up periods, respectively. From these and the 40 previously reported cases, the authors describe the different features of this unusual tumor.

[Carney's syndrome disclosed by hemoperitoneum]. / Syndrome de Carney révélé par un hémopéritoine.

The authors report a case of Carney's triad in a blood-group O woman who successively sustained a) at age 13, a multicentric gastric leiomyoblastoma revealed by an hemoperitoneum; b) 6 years later, a pulmonary chondroma diagnosed by chest standard roentgenogram and CT scan, associated with leiomyoblastoma recurrence and hepatic metastases; c) 10 years later, a juxta-carotid tumor of unknown (paraganglion?) origin. This patient is presently in good clinical condition, in spite of hepatic metastases known for 5 years. Based on this case and the 33 previously reported cases, the main features of this very unusual entity are analyzed.

[Acute pancreatitis caused by papillary adenoma of the distal common bile duct. Treatment by endoscopic excision]. / Pancréatite aiguë due à un adénome papillaire du bas cholédoque. Traitement par exérèse endoscopique.

We report the case of a 66 year-old man who developed acute necrotizing pancreatitis. The usual etiologies of acute pancreatitis were easily excluded. Endoscopic retrograde cholangiopancreatography demonstrated a 10 mm diameter filling defect in the distal common bile duct. After endoscopic sphincterotomy, a polypoid tumour which appeared to arise from the common bile duct was delivered through the wound. Diathermic resection of the tumor was performed. Microscopic examination disclosed papillary adenoma, without histologic signs of malignancy. Follow-up was favorable.

[Histomorphometric study of the gastric mucosa in portal hypertension]. / Etude histomorphométrique de la muqueuse gastrique au cours de l'hypertension portale.

Histologic features of the gastric (antral and fundic) mucosa in 14 patients with portal hypertension due to alcoholic cirrhosis and a mosaic pattern of the fundic mucosa at endoscopic examination have been compared with those of the gastric mucosa in 14 control subjects. We attempted to correlate endoscopic and histologic aspects using a semiquantitative morphometric study in which the height of the mucosa, the number per mm2 and the diameter of vascular sections of the interglandular chorion, and the number per mm2 of large (greater than or equal to 20 micron) vascular sections of the superficial chorion were measured. For all parameters, the mean values were higher in cirrhotic patients than in controls, but significant differences were found only in the antral mucosa (height of the mucosa and number of large vascular sections in the superficial chorion). These results confirm the frequency and the importance of vascular abnormalities of the gastric mucosa in patients with portal hypertension, but do not explain, at least with the methodology used, the mosaic pattern of the fundic mucosa disclosed in most cirrhotic patients.