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3, actualmente denominado gen HFE, localizado en el brazo corto del cromosoma 6 y que codifica una proteina similar a las del tipo HLA-I, ha constituido uno de los mayores avances en el conocimiento y tratamiento de esta enfemedad, ya que permite diagnosticar de forma sencilla y no invasiva a los individuos con una susceptibilidad genetica a desarrollar esta enfermedad.

To evaluate the prognostic value of the endoscopic examination, we included 189 consecutive patients with upper gastrointestinal bleeding, in a prospective study. Rebleeding was more frequent in patients with endoscopic stigmata of hemorrhage. The prognostic prediction given by the physician was slightly, but not significantly, improved after the result of the endoscopy was available. However, when the clinical data were evaluated by computer, using the Bayes' theorem, the endoscopic findings provided no additional prognostic value. We conclude that the endoscopic findings have intrinsic prognostic value, but add little to clinical data obtained on admission.

In this study, ethinylestradiol inhibited the uptake of taurocholate by cultured rat hepatocytes, increasing the Km1 while leaving the Vmax unchanged. S-AdenosylL-methionine (SAMe) had no effect on taurocholate uptake or release, but was able to reverse the competitive inhibition induced by ethinylestradiol. S-Adenosyl-L-homocysteine did not reverse this inhibition, which suggests that the methyl group of SAMe affects its activity. Several possible mechanisms for the action of SAMe were investigated. The methylation of cell membrane phospholipids was eliminated as a possible mechanism. The presence of SAMe greatly increased the catabolism of ethinylestradiol by hepatocytes and reduced its covalent binding to hepatocyte macromolecules. In culture supernatants, both highly polar (conjugated) and non-conjugated metabolites could be detected. Moreover, most of the metabolites were methylated. This suggests that SAMe may revert the effects of ethinylestradiol of taurocholate uptake by increasing its catabolic rate by hepatocytes.

In this study we investigated the effect of selective (M1) and non-selective (M1 and M2) pharmacologic blockade of muscarinic receptors on cholecystokinin-induced gallbladder emptying. After validating the method of study, the gallbladder function was evaluated in 15 normal volunteers by quantitative biliary scintigraphy, and the effect of intravenous atropine (0.15 mg/10 kg) and pirenzepine (10 mg) was analyzed in each subject. Atropine significantly reduced the ejection period and the ejection fraction of gallbladder evacuation. Pirenzepine reduced the ejection period, but the ejection fraction remained unchanged. We conclude that the effect of cholecystokinin on gallbladder motility is mediated through muscarinic receptors. Our results suggest that M2 receptors, but not M1 receptors, are involved in this response.

Objective: To describe a patient with Duchenne muscular dystrophy who presented with oropharyngeal dysphagia and pharyngo-oesophageal dysfunction. Patient: A 15-year-old male patient with Duchenne muscular dystrophy presented with oropharyngeal dysphagia. Abnormalities of oral and pharyngeal phases of deglutition were observed on videoradiography. Pharyngeal hypomotility and upper oesophageal sphincter dysfunction were the main manometric findings. Conclusion: To our knowledge; the presence of pharyngeal or upper oesophageal sphincter dysfunction has not been found previously in patients with progressive muscular dystrophy

Summary We studied the Oddi sphincter motility by endoscopic manometry in 10 consecutive patients randomized in a double-blind fashion, after i.v. administration of two anticholinergic compounds (0.5 mg atropine sulfate and 10 mg pirenzepine). Pirenzepine significantly decreased the basal sphincteric pressure, as well as the amplitude and frequency of the phasic contractions. The only significant effect of atropine was the modification of the frequency of the phasic contractions, but only for a short period of time. Our results suggest that muscarinic innervation must be present for a normal sphincter of Oddi motility.

The ascitic fluid concentrations of cholesterol and fibronectin and the serum-ascites albumin difference were compared with two conventional tests of ascitic fluid, total protein and LDH, in their diagnostic ability for detection of malignancy in ascitic samples from 69 patients with ascites: 54 with ascites due to liver disease and 15 whose ascites was caused by peritoneal metastases. Sixteen cirrhotic patients with superimposed hepatocellular carcinoma in whom ascites was of uncertain etiology were considered separately. The mean ascitic fluid total protein, LDH, cholesterol, and fibronectin values in the peritoneal metastases group were 3.70 +/- 1.20 g/dl, 247.26 +/- 148.14 units/liter, 109.06 +/- 29.85 mg/dl, and 91.57 +/- 41.52 micrograms/ml, respectively, and all were significantly higher than the corresponding values in the liver disease group (P less than 0.001), which were 1.37 +/- 0.59 g/dl, 75.40 +/- 110.70 units/liter, 23.75 +/- 11.22 mg/dl, and 31.86 +/- 10.51 micrograms/ml, respectively. Mean serum-ascites albumin difference in the peritoneal metastases group was 0.62 +/- 0.38 g/dl, which was significantly different from the corresponding value in the liver disease group (1.92 +/- 0.41 g/dl, P less than 0.001). Both ascitic cholesterol above 46 mg/dl and an ascitic fibronectin concentration greater than 50 micrograms/ml had high diagnostic accuracy (97%) for malignancy, being higher than that achieved using a serum-ascites albumin difference under 1.1 g/dl and an ascitic total protein above 2.5 g/dl, which had accuracies of 94% and 93%, respectively. Ascitic fluid LDH was the least reliable test. No differences in the ascitic fluid analysis were found between cirrhotic patients with and without hepatocellular carcinoma.(ABSTRACT TRUNCATED AT 250 WORDS)

Summary We report a case of multiple hepatic adenomas developed in a 32-year-old man with renal allograft after long-term therapy with testosterone enanthate. Histological assessment showed a benign hepatocellular adenoma, which has not regressed over a 6-month follow-up since androgen withdrawal. A review of primary hepatic tumors associated with androgenic-anabolic steroid therapy published in the English literature has been carried out, concentrating on the more relevant recent publications. No previous case of hepatic adenoma with possible relationship to the use of non-17-alpha alkylated compounds has been reported.

A morphometric study of the distal esophageal mucosa (within 5 cm above the gastroesophageal junction) has been carried out in a group of 11 cirrhotic patients undergoing esophageal transection with SPTU gun for variceal bleeding. The relative thickness of the papillae (62.2 +/- 3.9%) and basal zone (11.8 +/- 1.9%) were within normal limits. Polymorphonuclear infiltrates were not found either in the lamina propria or in the epithelium. The absence of histopathologic changes in the esophageal mucosa from patients with liver cirrhosis and bleeding esophageal varices confirms the hypothesis that gastroesophageal reflux does not play a pathogenic role in the development of variceal bleeding.

Previous therapeutic trials with prostaglandins have shown them to be effective in healing duodenal ulcers when used at doses that are highly effective suppressors of gastric acid secretion. We undertook this trial to determine if a cytoprotective dose of arbaprostil (10 μg q.i.d. for 4 wk) would also be efficacious in this disease state. Eighty-two patients between the ages of 19 and 72 yr with endoscopically documented duodenal ulcers were entered into this randomized double-blind placebo-controlled trial. The patients were monitored with biweekly endoscopies and laboratory examinations, weekly interviews during the period when drug was administered, and a follow-up interview plus laboratory examinations 1 wk after drug administration was completed. No statistically significant differences between the arbaprostil and placebo treatment groups were found for ulcer healing rates, pain relief, antacid consumption, side effects, or laboratory examinations. It is presumed that this prostaglandin may not have sufficient duodenal cytoprotective capacity to effectively heal duodenal ulcers, or that some suppression of gastric acid secretion may be required to achieve significant clinical efficacy.

A 23-year-old male was diagnosed as having idiopathic achalasia on the basis of clinical, radiologic, endoscopic, and manometric evaluation. He underwent a Heller's myotomy with 180 degrees posterior fundoplication as an antireflux procedure, and he did well subsequently. On reexamination one month later, return of peristaltic activity throughout the body of the esophagus was shown on manometric studies. Two years after the operation, peptic esophagitis was diagnosed by esophagoscopy, and the acid reflux test confirmed the existence of gastroesophageal reflux. To our knowledge, this represents the first reported case of return of esophageal peristalsis in idiopathic achalasia after surgical myotomy.

The relationship between donor status for antibody to hepatitis B core antigen and the occurrence of non-A, non-B posttransfusion hepatitis in the recipient was prospectively studied in 112 patients undergoing open-heart surgery who were followed for 6.5 months after surgery. Non-A, non-B posttransfusion hepatitis occurred in five (7.93%) of 63 patients who had received at least one anti-HBc-positive blood unit compared to seven (14.28%) of 49 patients who received anti-HBc-negative blood only. Statistical analysis revealed that the incidence of non-A, non-B posttransfusion hepatitis was independent of the use of blood positive for anti-HBc. Based upon these results and the high prevalence (17.3%) of anti-HBc among our blood donor population, the exclusion of anti-HBc-positive blood does not seem appropriate to achieve a reduction in the incidence of non-A, non-B posttransfusion hepatitis.

We studied the motility of the sphincter of Oddi in 12 patients with suspected sphincter of Oddi dysfunction, in four patients with cystic dilatation of the bile ducts (two Caroli's cases and two fusiform choledochal cyst cases), and in 33 patients with retained common duct stones. In these last 33 patients, the motor activity of the sphincter of Oddi was similar to that recorded in nine control subjects without pancreatic or biliary diseases. In the suspected Oddi dysfunction cases, both the basal sphincteric pressure and the frequency of the phasic contractions were significantly elevated (P less than 0.001). Patients with biliary cystic dilatation showed an increased basal pressure, but the frequency of the contractions was elevated in only those with choledochal cysts and the amplitude in only one of the two patients with Caroli's disease. Motor disorders of the sphincter of Oddi provide a basis for an alternative etiopathogenesis of cystic disease of the biliary system and a possible explanation for pain and dilatation of the bile duct in patients with suspected sphincter of Oddi dysfunction.

Alterations in the coagulation system are common in patients with liver disease. We have examined the importance of the species and chains of fibrinogen in 3 groups of cirrhotic patients. The study of the gelation of fibrinogen in cirrhotic patients shows that the lag time increases in 80.3% of them and that the maximum gelation rate is altered in 51% of these plasmas. Also it is observed that 80% of the plasmas from cirrhotic patients have a percentage (23.3 ± 7.7%) of unpolymerized α chain, after highly cross-linked fibrin formation. These alterations, in lag time and in the maximum gelation rate, have no significant correlation with the situation of the fibrinolytic system in these patients.The study of isolated fibrinogen from cirrhotic patients and normal subjects plasma, shows that there are no objetive alterations in the percentage of fibrinogen species, the amount of sialic acid or the ratio of polypeptide chains.

Excerpt To the editor: In their recent article on transfusion-associated hepatitis, Koziol and coworkers (1) suggest the adoption of the test for antibody to the hepatitis B core antigen (anti-HBc)...

Familial amyloidotic polyneuropathy is an inherited form of amyloidosis associated with a mutant form of a protein called transthyretin. The Methionine-30 variant is the most frequent mutation observed. This disorder is caused by deposition of this protein as amyloid in several organs, such as the heart, kidneys, and peripheral nervous system. The disease is always progressive and fatal, and patients die 7 to 10 years after the onset of symptoms. Liver transplantation is at present the only choice for these patients because it provides improvement of symptoms and/or stops progression of the disease in most patients. We report the case of a patient who showed clear progression of cardiomyopathy and neuropathy after liver transplantation.

Excerpt To the editor: Androgenic-anabolic steroids have been implicated in the genesis of primary liver tumours. Benign hepatic adenomas are rare and, to our knowledge, no previous case associated...

Excerpt To the Editor:We report the case of a hemophiliac who presented with spontaneous hemobilia. A 45-year-old man was hospitalized for episodic upper abdominal pain. Severe hemophilia A had pre...

La infección por el virus de la hepatitis C (VHC) tiene gran importancia a nivel del trasplante hepático (HC) por varias razones. Por lo tanto, resulta de una gran importancia establecer el pronóstico post-trasplante en dichos casos.El objetivo del presente proyecto de tesis fue, por un lado, definir la historia natural de la hepatitis C postrasplante en nuestro medio; y por otro, establecer los factores de riesgo de evolución desfavorable. Dicha información es fundamental para validar la idoneidad de un trasplante hepático, así como para considerar la posibilidad de un tratamiento antivírico precoz y mejorar el manejo de la inmunosupresión de los pacientes.