The pyruvate dehydrogenase complex as a target autoantigen in primary biliary cirrhosis.

Mitochondrial autoantigens and their B and T cell autoepitopes have been well defined in primary biliary cirrhosis (PBC). However, the relationships of the antimitochondrial antibodies and the mechanisms of bile duct destruction in PBC remain an enigma. The serological hallmark of PBC remains the presence of antibodies to mitochondria, particularly to the E2 component of the pyruvate dehydrogenase complex (PDC-E2). However, several mechanisms may now be proposed which may explain the immune-mediated bile duct damage in PBC. These include the possible role of T cell-mediated cytotoxicity as well as the interaction between the IgA class of antimitochondrial antibodies and the mitochondrial autoantigens. A prominent feature in this discussion is the highly directed and specific immune response to the mitochondrial antigens, including PDC-E2 as well as other members of the 2-oxo-acid dehydrogenase complexes. Ultimately, the mechanisms that lead to this immune reaction should provide data on other questions in PBC, including the reasons for female predominance, the absence of PBC in children and the relative ineffectiveness of immunosuppressive agents.     

The problem of the Romano-Ward syndrome--misinterpretation as genuine epilepsy]

It is reported on a case of Romano-Ward-syndrome in a 40-year-old female patient. The problems of the most frequent false interpretation as idiopathic epilepsy as well as the differential-diagnostic considerations concerning the exclusion of symptomatic QT(U)-elongations in the ECG are entered. The pathomechanisms which perhaps are the basis of the syndrome are discussed.     

The association of knee structural pathology with pain at the knee is modified by pain at other sites in those with knee osteoarthritis

The objective of this study was to investigate the associations of knee structural abnormalities with different patterns of pain. A total of 891 participants (average age 63 years; range 50 to 80 years) participated in this study. Presence of pain at the neck, back, hands, shoulders, hips, knees, and feet was assessed by questionnaire. Participants were categorized as having no pain at any site (no pain), pain only at the knee (KP), pain at other sites but not the knee (OP), and pain at the knee and other sites (KOP). T1-weighted or T2-weighted MRI of the right knee was performed to measure cartilage defects, bone marrow lesions (BMLs), and effusion-synovitis. Osteophytes and joint space narrowing were assessed by X-ray. KP, KOP, and OP were, respectively, present in 3, 43, and 42% of the participants. In multivariable analyses, KOP was associated with the presence of cartilage defects, BMLs, and osteophytes (OR 3.57 (95% CI 1.78 to 7.14), 2.37 (1.27 to 4.43), and 2.87 (1.10 to 7.51), respectively) in those with radiographic knee OA. KP was also associated with presence of these structural abnormalities as well as effusion-synovitis, and these associations were much stronger. The associations between structural abnormalities and KOP were weaker than those with KP in those with radiographic knee OA. This suggests that mechanisms mediating the association between structural pathology, localized, and generalized pain may be different, and central sensitization is possibly involved in generalized pain. Pain at other sites needs to be considered in the management and treatment of OA-related pain.     

Cardiovascular pathology and the partial autopsy. The experience at the Instituto Nacional de Cardiología Ignacio Chávez of Mexico]

There is no doubt autopsies are still a powerful source of medical information. At the Instituto Nacional de Cardiología de México 50% of deaths are autopsied. About half are limited cardiothoracic studies. Since no previous evaluation of the utility of the information obtained from those limited necropsies, we decided to test how well they allowed for a good clinical and anatomical correlation of main disease and a cause of death, as well as their potential to respond clinical questions relevant to the case. We analyzed the medical and autopsy records from 50 cases and determined autopsy type (limited or total), age, sex, main disease and cause of death. It was also included a list of questions made by the attending physician who asked for autopsy. Twenty-six cases corresponded to limited autopsies. In 96.1% there was a good anatomical and clinical correlation of main disease. In 69.2% the cause of death had also a good correlation and in 92.3% of the cases the clinician's question were answered appropriately. Based in our results we support the idea that cardiovascular limited autopsies are an alternative way to obtain useful information, when otherwise, total autopsies result expensive or difficult to obtain.     

Monoctanoin for the dissolution of biliary calculi. Report of a case]

The authors present a case report of a cholecystostomized high surgical-risk patient with gallstones in gallbladder and biliary duct treated with continuous monoctanoin infusion through a Pezzer's tube. They analyze the biological characters of such substance as solvent agent, its management and the results of treatment.     

Thrombosis of the right atrial appendage. The two-dimensional echocardiographic aspect and the autopsy finding]

We report the case of an 80-year-old man affected by hypertensive cardiomyopathy and already admitted to our Division for recurrent episodes of heart failure. He was eventually hospitalized for septic shock secondary to disseminated pneumonia with concomitant left pleural effusion. At 2D-echo examination, a highly echo-reflectant mass was detected in the retrocardiac space. As the patient died notwithstanding the intensive pharmacological care, an autoptic procedure was performed which showed a massive thrombosis of the right atrial appendage. We discuss the 2D-echo imaging and advance a pathogenetic hypothesis after a review of the current literature.     

Current views of vascular endothelium in health and at a pathology of the gastrointestinal tract]

The article presents current data on the structure, function and biological role of vascular endothelium as well as its role in the genesis of pathologies of the gastrointestinal tract. Characteristics of endothelial vasoconstrictors and vasodilators are given. When we take into account the literature data and authors' studies, it becomes expedient to conduct further studies of the role of endothelial dysfunction in the development of diseases of the digestive tract in view of little knowledge of it at this pathology as well as availability of mostly experimental data.     

The characteristics of gastroduodenal pathology in patients with chronic opisthorchiasis as a criterion for the clinical diagnosis of the invasion]

Using a syndrome approach the clinical pattern has been studied in 269 patients with chronic gastritis and duodenitis. 55 of patients had gastric ulcer, 105 patients had duodenal ulcer. In 130 patients chronic opisthorchiasis was diagnosed 8.9 +/- 1.9 years after the disease onset. The following syndromes are believed to be diagnostically valuable, enabling one to suspect chronic opisthorchiasis in patients with gastroduodenal pathology from the endemic foci: "low eosinophilia syndrome", "right hypochondrium" syndrome, "fever and enterocolitic syndrome" with the special consideration of the nature of dysbacteriosis of the small bowel.     

Methods to optimize the management of Helicobacter pylori infection. The comparative efficacy of the triple and quadruple therapy used as a first line therapy]

The study involved a dynamic comparative efficacy survey of the standard triple and quadruple therapies recommended by the Maastricht Consensus as first line therapies for eradication of Helicobacter pylori infection with the time period of 5 years. The study included 199 Hp-positive patients with stomach ulcer; 101 of them were under examination in 1997 and 98 in 2002. Depending on the therapy type, patients were assigned to one of two groups: the OCM/A group (48 and 53 patients in 1997 and 2002, respectively) was treated with Omeprazole, Clarithromycin and Metronidazole for 7 days and ODTM group (46 and 52 patients in 1997 and 2002, correspondingly) was treated with Omeprazole, De-Nol, Tetracycline and Metronidazole. To discover and confirm Hp eradication, cytological, histological and rapid urease tests were used. Hp eradication was considered as successful when all the tests were negative. The eradication frequency was assessed with the help of ITT and PP analyses. In the OCM/A group Hp was eradicated in 81.3% and 62.3% (p<0.05) of patients when analyzed by the intention-to-treat and in 88.6% and 66.0% (p<0.01) of patients when analyzed by per-protocol in 1997 and 2002, respectively. In the ODTM group Helicobacter pylori was eradicated in 89.1% and 88.5% (p<0.05) of patients when analyzed by intention-to-treat and 95.3% and 93.9% (p<0.05) when analyzed by per-protocol in 1997 and 2002, respectively. The frequency of ulcer cicatrisation and cuticularization of erosions did not depend on the type of the treatment. There was no significant difference between the compliance and side effects of the triple and quadruple therapies. Taking into account the decrease in the efficacy of the triple anti-Hp therapy, the need to use the quadruple therapy as a first line therapy for Hp infection eradication was substantiated.     

Active `juvenile' cirrhosis considered as part of a systemic disease and the effect of corticosteroid therapy

This is a clinical study of 81 patients (32 male and 49 female), who are not alcoholics, with jaundice of hepato-cellular type lasting longer than three months. Forty-nine of these patients were under 21 years of age. The natural history of the condition and the response to corticosteroid therapy is described; good symptomatic relief can be achieved with corticosteroids but they do not seem to prolong life. The aetiology is discussed with special reference to the relationship to viral hepatitis, to systemic lupus erythematosus, and to other disturbances of immunity.     

Percutaneous catheterization of the intestinal loop of hepatico- jejunostomy: a new possibility in the treatment of complex biliary diseases]

OBJECTIVES: After hepatico-jejunostomy, endoscopic exploration of the biliary tract is not possible, and percutaneous transjejunal catheterization seems to be an attractive option. PATIENTS: This is a 10 year prospective evaluation of 55 percutaneous transjejunal biliary interventions in 53 patients. RESULTS: Thirty nine patients had biliary lithiasis, 10 had suspected recurrent biliary cancer, 5 biliary stenosis, and 1 angiocholitic intrahepatic abscess. Initial success was obtained in all patients and 155 procedures were performed. Interventions included strictures, dilatation, stone extraction, stent insertion and tumor biopsy. The complication rate was 15% (mainly benign biliary sepsis) with no deaths and no surgical reoperations. Thirty two of the 39 patients with biliary lithiasis had successful extraction. Eight of the 10 patients with cancer had an endoscopic biopsy and the 2 others underwent drainage. The 5 patients with benign strictures underwent dilatation and stenting. The intrahepatic abscess was treated completely by drainage. CONCLUSIONS: The feasibility of this technique, the low morbidity and the lack of mortality has been demonstrated. This technique is well accepted by patients and may be an alternative to open surgery which is known to be very difficult and risky in patients who have had one or several prior operations.     

The in vitro and in vivo evaluation of tilorone hydrochloride as a platelet aggregation inhibitor.

Tilorone hydrochloride was tested in vitro for inhibition of platelet aggregation in the ADP, collagen, and epinephrine-induced platelet aggregation systems. Tilorone did not significantly inhibit the first phase of platelet aggregation induced by ADP, but was a very potent inhibitor of the second phase of platelet aggregation (release reaction) as illustrated by the epinephrine-induced system. When the compound was given to rats by stomach tube at 100 mg/kg for 2 doses, 24 h apart and blood taken 24 h after the second dose, an inhibition of collagen-induced platelet aggregation was found. This result is interpreted as inhibiting the release reaction and second phase platelet aggregation.