Prevalence of rheumatoid arthritis in the South-Transdanubian region of Hungary based on a representative survey of 10,000 inhabitants

OBJECTIVE: To assess the prevalence of rheumatoid arthritis (RA) in a representative study of the South Transdanubian region of Hungary. METHODS: Ten thousand individuals aged between 14-65 years were interviewed. The stratified sample was representative for age, sex and urban/rural residence structure of the regional population of the South-West Hungarian region. As a second step, all individuals with possible RA were asked to undergo a clinical investigation to confirm the diagnosis of RA according to the American Rheumatism Association (ARA) 1987 criteria. Of 10,000 interviewed individuals, 632 reported having RA or symptoms including digital pain, stiffness, and/or swelling. Two hundred and twenty-four individuals were investigated clinically. Individuals fulfilling the 1987 ARA criteria were considered as having definite RA, and their clinical data were evaluated. RESULTS: RA was confirmed in 13 cases. The male/female ratio was 3/10. The prevalence of RA among individuals aged 14-65 years was 0.37% (95% confidence interval, CI: 0.26-0.51), 0.23% (95% CI: 0.15-0.35) in men and 0.48% (95% CI: 0.35-0.64) in women. CONCLUSION: The prevalence of RA in the South Transdanubian region of Hungary is similar to those of other recent studies from other regions around the world.     

Impaired inactivation by antithrombin and hirudin and preserved fibrinogen-clotting activity of thrombin in complex with anti-thrombin antibody from a patient with antiphospholipid syndrome

Immunoglobulin G (IgG) isolated from the blood plasma of a patient with secondary antiphospholipid syndrome (APS) expresses fibrinogen-clotting and amidolytic activity (the thrombin activity in 20 micromole IgG is equivalent to approximately 5 nmole pure thrombin), and activates factor XIII. Hirudin (1 microM) decreases the intrinsic thrombin activity of the APS IgG by only 25%, whereas it inhibits completely pure thrombin with equivalent activity. Under conditions, when antithrombin inactivates 60% of the thrombin activity in the presence of normal IgG, the APS IgG protects almost completely the added thrombin against inactivation by antithrombin. Heparin, however, partially relieves this protective effect and at the same time it facilitates the inhibition of the intrinsic thrombin activity by antithrombin. The APS IgG reduces the thrombin activity in protein C activation assay by 50% compared to the activity in the presence of normal IgG. All described properties are related to the Fab fragment of the antibody. The IgG preserving the fibrin-generating activity of thrombin with concomitant protection against inhibitors unravels a new aspect of the thrombotic mechanism in APS. This condition is probably rare: only one out of 23 examined patients with primary or secondary APS expresses IgG with the described properties.     

Quinine, a blocker of neuronal cx36 channels, suppresses seizure activity in rat neocortex in vivo

PURPOSE: The selective contribution of neuronal gap junction (GJ) communication via connexin 36 (Cx36) channels to epileptogenesis and to the maintenance and propagation of seizures was investigated in both the primary focus and the mirror focus by using pharmacologic approaches with the 4-aminopyridine in vivo epilepsy model. METHODS: ECoG recording was performed on anesthetized adult rats, in which either quinine, a selective blocker of Cx36, or the broad-spectrum GJ blockers carbenoxolone and octanol were applied locally, before the induction or at already active epileptic foci. RESULTS: The blockade of Cx36 channels by quinine before the induction of epileptiform activity slightly reduced the epileptogenesis. When quinine was applied after 25-30 repetitions of seizures, a new discharge pattern appeared with frequencies >15 Hz at the initiation of seizures. In spite of the increased number of seizures, the summated ictal activity decreased, because of the significant reduction in the duration of the seizures. The amplitudes of the seizure discharges of all the patterns decreased, with the exception of those with frequencies of 11-12 Hz. The blockade of Cx36 channels and the global blockade of the GJ channels resulted in qualitatively different modifications in ictogenesis. CONCLUSIONS: The blockade of Cx36 channels at the already active epileptic focus has an anticonvulsive effect and modifies the manifestation of the 1- to 18-Hz seizure discharges. Our findings indicate that the GJ communication via Cx36 channels is differently involved in the synchronization of the activities of the networks generating seizure discharges with different frequencies. Additionally, we conclude that both neuronal and glial GJ communication contribute to the manifestation and propagation of seizures in the adult rat neocortex.     

Frontotemporal dementia--Part I. History, prevalence, clinical forms

The authors report a comprehensive publication consisting of three parts going into the details of history, prevalence, clinical forms, differential diagnosis, genetics, molecular pathomechanism, pathology, clinical diagnosis and treatment of frontotemporal dementia (FTD). The first part of the present review deals with history, prevalence and clinical forms of FTD. The prototypical FTD with circumscribed atrophy was first described by Arnold Pick; Alois Alzheimer found the intraneural inclusions in the patients' brain. Later it was recognised that many patients had neither the atrophy nor the cellular changes, but genetic mutations have been identified. Frontotemporal dementia is a degenerative condition with unknown etiology in the frontal and anterior temporal lobes of the brain. It is a progressive neurobehavioral syndrome characterized by early decline in social interpersonal conduct, early impairment in the regulation of personal conduct, early emotional blunting, and early loss of insight. There are no reliable epidemiological studies on the prevalence of FTD, but it is well-accepted that FTD is a common cause for dementia before the age of 65 (it constitutes approximately five percent of all irreversible dementias). The nomenclature of the FTD has been confusing and continues to be. Three major clinical syndromes can be identified: 1 frontal variant FTD (dementia of frontal type) in which changes in social behavior and personality predominate, 2. in semantic dementia (progressive fluent aphasia) there is a breakdown in the conceptual database which underlies language production and comprehension, 3. in progressive nonfluent aphasia the phonologic and syntactic components of language are affected. The authors report two cases, which can point to clinical symptoms and forms, and mention the problems of the differential diagnosis and therapy.     

Partial protection against dextran sodium sulphate induced colitis in histamine-deficient, histidine decarboxylase knockout mice

OBJECTIVES: Chemically induced mucosal inflammation in animal models is a suitable tool for studying factors in the pathogenesis of inflammatory bowel disease. The aim of this study was to determine whether absence of histamine has an effect on the development of experimental colitis. METHODS: Histamine-deficient, histidine decarboxylase (HDC) knockout Balb/c mice and genetically identical control animals with intact HDC were studied. Colitis was induced by the administration of 2% dextran sodium sulphate in drinking water. Mice were killed after 5 days and disease activity assessed by clinical, histologic, and immunohistologic parameters. Bacterial components of stool were examined. RESULTS: Clinical disease activity was higher in the mice with intact HDC (disease activity index, 2.21) than in the histamine-deficient knock-out mice (1.88). Histologic findings were similar in the two groups. On day 5, the inflammation score of the HDC sufficient group was 5.25 (+/-1.055) and the crypt score was 5.00 (+/-1.128). The scores in the HDC knock-out group were 4.667 (+/- 0.707) and 4.667 (+/- 0.86), respectively. There was a significant difference in the number of interleukin (IL-10)-producing lymphocytes in colon mucosa. Large numbers of IL-10-positive lymphocytes were observed in wild type mice both those with DSS induced colitis and untreated controls. Only sporadic IL-10 positivity was found in histamine-deficient mice. Significant differences were found in the composition of the fecal bacterial flora between the two groups. CONCLUSION: The reduced number of IL-10-positive lymphocytes in the intestinal mucosa of histamine-deficient, histidine decarboxylase knockout mice and the altered fecal bacterial flora in these animals suggest that histamine may play a role in the pathophysiology of inflammation in the colon of normal animals by upregulating local IL-10 production and stimulating a local shift to Th2 response.     

The advantages of axillary artery cannulation in surgical treatment of type A acute aortic dissection

There are more alternative cannulation techniques during surgery of type A aortic dissection. The most frequently used femoro-atrial cannulation method provides limited possibility for brain protection during surgery. This theory is confirmed by relatively high frequency of major brain complications in patients operated on while using this cannulation technique. During the last years cannulationis used more often, as it may provide more protection for the brain than other methods. In 2003 seven patients underwent aortic reconstruction because of type A acute aortic dissection using axillary cannulation. All patients except one were discharged after uneventful recovery. There were no postoperative neurological complications following surgery. We lost one patient due to distal progression of the dissection. He was the only patient with clinical evidence of transient postoperative brain damage. We are strongly convinced that the spectacular improvement in our results for the surgery of type A acute aortic dissection is due to the axillary cannulation, the anterograde flow and the isolated cerebral perfusion. We recommend the axillary cannulation technique as the first choice in type A acute aortic dissection.     

Difficulties in the treatment of polycystic liver

A 52-year-old female patient was admitted to our department suffering from discomfort and tension in right side subcostal region for five months. The anamnesis contains cholecystectomy, appendectomy and gynecology treatment. CT examination and X-ray examination showed cysts in both lobe of liver. The primary treatment was ultrasonography guided punction in another department. This treatment caused anaphylactoid shock. After this dangerous treatment the patient refused the next punction. Following required arrangement laparoscopic exploration and adhaesiolysis were done in our department. Different size cysts had laparoscopically fenestrated. After half year the patient's symptoms resumed. Repeated CT and US examinations showed cysts again. Cysts were laparoscopically fenestrated again. Since the operation the patient had no complaint. Histology showed fibrocystic liver. After a month control CT examination showed cysts again. After two months we made ultrasonography guide punction of remain cysts following radiological consultation.