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Hypertension and nephrology

OCTOBER 20, 2013

[Therapeutic apheresis in pediatry]

TÚRI Sándor, BERECKI Csaba, HASZON Ibolya, PAPP Ferenc

[The possible mechanisms of therapeutic plasma mexchange: 1. the removal of circulatory plasma factor (anti Gbm disease, myasthenia gravis, Guillain Barré syndrome), 2. monoclonal antibody (Waldenström macroglobulinemia, myeloma protein), 3. circulatory immuncomplexes cryoglobulinaemia, myeloma protein, SLE), 4. alloantibody, 5. toxic factor, 6. replacement of a specific plasma factor, 7. a repear of the function of reticulo-endothelial system, 8. the removal of the inflammatory mediators, 9. the changes of the ratio of antigen-antibody which makes immuncomplexes more soluble, 10 stimulation of lymphocyte clones for supporting the cytotoxic therapy. Indications of emergency plasmapheresis: 1. Goodpasture syndrome with rapidly progressive glomerulonephritis and hemoptoe, 2. hyperviscosity syndrome, 3. TTP/HUS, 4. High level of factor VIII inhibitor, 5. respiratory insufficiency Guillain-Barré syndrome, 6. myasthenia gravis, 7. acute mushroom intoxication, or protein bound toxins. Further indications for plasmapheresis: 8. cryoglobulinemia, 9. other cases of rapidly progressive glomerulonephritis (when steroid+ cyclophosphamide are ineffective), 10. Wegener granulomatosis, 11. polyarteritis nodosa, 12. systemic lupus erythematosus (when steroid and cyclophosphamid therapy is not effective or associated with cerebral vasculitis, antiphospholipid syndrome combined with bleeding and thrombosis), 13. focal segmental glomerulosclerosis (resistant for therapy), 14. acute tubulointerstitial nephritis, 15. acute vascular rejection, 16. rheumatoid arthritis systemic type, 17. hypertrigliceridemia (≥25 mM), 18. thyreotoxic crisis, 19. acute necrotizing pancreatitis, 20. acute fulminant hepatitis, 21. paraquat intoxication, 22. snake bite (when antiserum is unavailable), 23. drug intoxication.]

Hypertension and nephrology

DECEMBER 20, 2011

[Association between complications of percutaneous kidney biopsy and histological diagnosis ]

FISI Viktória, MAZÁK István, DEGRELL Péter, HALMAI Richárd, MOLNÁR Gergő A., FEHÉR Eszter, NÉMETH Kinga, PINTÉR István, KOVÁCS Tibor, WITTMANN István

[Background: Percutaneous renal biopsy is an essential tool in diagnosis and prognosis of renal diseases. It is well-known that this method has potential complications. The connection between complication occurrence related to renal biopsies and histological diagnoses of the biopsy specimen was analyzed in the present study. We also analyzed the distribution of diagnoses in our population. Methods: In this retrospective survey, 353 patients undergoing renal biopsy was studied. Biopsies were performed after marking the site of puncture with ultrasound imaging. Influence of diagnoses and clinical parameters on complications was evaluated. Results: We found a complication rate of 44.5%. In patients with diabetic nephropathy (likelihood ratio (LR) 0.44) or acute tubular necrosis (LR 0.38) a significantly lower rate of complications was found, while patients with thin basement membrane syndrome had more than 6-fold higher risk for evolvement of intrarenal haemorrhage. Patients with acute interstitial nephritis (LR 3.18) or vasculitis (LR 2.88) have a more than 2-fold risk for arteriovenous shunts while in patients with severe arteriosclerosis the occurrence of this complication was lower (LR 0.46). In rapidly progressive glomerulonephritis, arteriovenous shunts evolved also in a significantly higher rate. Conclusion: Patients with vasculitis, rapidly progressive glomerulonephritis, thin basement membrane syndrome or acute interstitial nephritis should be monitored more carefully after renal biopsy due to the significantly higher risk for complications. ]

Hungarian Radiology

JUNE 20, 2007

[Multisegmental tuberculous spondylodiscitis in a patient with Helicobacter pylori infection]

SZÁNTÓ Dezső, SZŰCS Gabriella, DITRÓI Edit

[INTRODUCTION - Prospective randomized examination has been verified association between asymptomatic chronic Helicobacter pylori infection and systemic vascular inflammation and endothelial dysfunction in certain cases. CASE REPORT - A case of a 71 year-old female patient is described with proven coexisting primary tuberculous bronchoadenitis, secondary tuberculous spondylodiscitis and Helicobacter pylori infection as a cause of uncertain abdominal disorder. The patient's height decreased by 9 cm during 6 months, since the onset of the symptoms. The diagnosis was based on respiratory function test, positive rapid ureal test, bronchoscopy, cytologic and microbiological examinations. On dorsolumbal spinal plain films showed wedge, biconcave crus and mass deformities of nine vertebras. High serum level of CRP and C3 complement was suggestive of acute reactive inflammation. Before the introduction of drug therapy an MR examination was planned, however the patient died of an acute myocardial infarct on the 8th day after admission The postmortem findings proved the presence of pulmonary and vertebral tuberculosis, there was no evindence of aortocoronal and spinal vasculitis. The epispinal tissues were not involved. CONCLUSION - The patient’s case history, autopsy findings and the literature data are suggestive of the role chronic Helicobacter pylori infection in the development of spondylodiscitis and the deterioration of the disease.]

Lege Artis Medicinae

NOVEMBER 20, 2007

[HUMAN LEUKOCYTE INTERFERON IN THE TREATMENT OF CHRONIC HEPATITIS C]

TELEGDY László, HORVÁTH Gábor, TOLVAJ Gyula, MAKARA Mihály, MONTSKÓ Valéria, OZSVÁR Zsófia, NEMES Zsuzsanna, PÉTERFY Zoltán, SZENTGYÖRGYI László

[INTRODUCTION - Approximatively 50% of the patients with chronic hepatitis C do not respond properly to pegylated interferon-alpha+ribavirin treatment and according to the therapeutic guidelines their treatment must be interrupted. The authors examined whether their further medication with human leukocyte interferon is justified. PATIENTS AND METHODS - Thirty-eight patients with chronic hepatitis C were treated with human leukocyte interferon who had responded to pegylated interferon-alpha-ribavirin treatment, but dropped out of the treatment scheme based on the therapeutic criteria on week 12 or 24. RESULTS - While only 3 patients responded with persistent virological negativity, mean alanine aminotransferase (ALT) levels decreased during treatment and persistently remained lower than the baseline levels. Except for the three responders, the HCV viral load as determined by polymerase chain reaction did not decrease but even increased on average. Patients tolerated the treatment well. Known side-effects were observed in 6 cases, and treatment had to be interrupted in one case. These are proportionally far less than the respective 20 to 25% and 5 to 15% reported for peginterferon-ribavirin treatment. CONCLUSION - Even though virological recovery is rare, the reduction in inflammatory activity, the expected slowing of progression, and, in particular, the definite improvement of the cryoglobulinaemic purpura and vasculitis warrant switching to human leukocyte interferon treatment in selected cases.]

Hungarian Immunology

FEBRUARY 15, 2004

[SS-A(Ro) and SS-B(La) autoantibodies in systemic lupus erythematosus]

SALLAI Krisztina, NAGY Eszter, GERGELY Péter

[OBJECTIVE - To assess the relation between clinical features and the presence of SS-A(Ro) and SS-B(La) autoantibodies in systemic lupus erythematosus. PATIENTS - The data of 200 patients with definite systemic lupus erythematosus were analysed. SSA( Ro) and SS-B(La) antibodies were assessed by enzyme immunoassay. RESULTS - 40.5% of systemic lupus erythematosus' patients were SS-A(Ro) and/or SS-B(La) antibody positive (’positive group’); the majority of such patients displayed both antibodies, 16.5% had SSA( Ro) antibodies alone, while only 2% has SS-B(La) antibodies alone. There were no differences in the occurrence of arthritis, secondary antiphospholipid syndrome and hematologic manifestations between the positive and negative groups; serositis was more common in the positive group. Skin manifestations, in particular subacute cutaneous lupus erythematosus and urticaria vasculitis were more frequent in the positive group, while kidney and central nervous system involvation, in particular severe forms were less frequent. Secondary Sjögren's syndrome occurred exclusively in antibody positive patients. Sm, RNP and Scl-70 antibodies were more frequently found in the positive group. CONCLUSIONS - The presence of SS-A(Ro) and/or SS-B(La) antibodies in systemic lupus erythematosus has some prognostic significance; in antibody-positive patients there is an increased risk for skin lesions (in particular subacute cutaneous lupus erythematosus and urticaria vasculitis) and secondary Sjögren’s syndrome and a decreased risk for severe nephritis or central nervous system involvement.]

Lege Artis Medicinae

JUNE 20, 2005

[NECROTIZING VASCULITIS AND THE GRANULOMATOUS INFLAMMATION OF THE SMALL INTESTINE. IS IT A SEPARATE ENTITY OR A SPECIAL FORM OF CROHN'S DISEASE?]

HIDVÉGI Judit, SZELECZKY Márton, SCHNABEL Róbert, KASZÁS Ilona, BAJTAI Attila

[INTRODUCTION - Churg and Strauss has reported a disease entity in 1951 characterized by asthma, eosinophilia in the peripheral blood as well as in different organs or tissues, systemic necrotizing vasculitis and extravascular tuberculoid granulomas. This granulomatous inflammation affected dominantly the wall of small vessels. The reason of allergic angiitis and granulomatous reaction is unknown but the role of some drugs used in the treatment of asthma was raised. There are a few reviews mentioning that the gastrointestinal tract, especially the small intestine can alos be involved. According to Morson, the clinical symptomes of these cases are mimicking Crohn’s disease. Oral contraceptives can also provoke allergic granulomatous angiitis, involving the gastrointestinal tract too. CASE REPORT - Two cases are described. One of them is a 35-year-old woman (the mother) who was treated because of idiopathic ulcerative colitis for a long time. Later, she was operated on because of mechanical ileus. Microscopically, in the resected specimen there was an intact mucous membrane and submucosal layer of small intestine, contradicting Crohn’s disease. On the other hand, the subserosal and the muscular layers were involved. The characteristic microscopic change were: necrotizing vasculitis involving the small vessels and a granulomatous change in the vessel walls. One of the daughters of a patient had to be been operated due to Crohn's disease. CONCLUSION - The accumulation of the cases affected by inflammatory bowel disease in the same family is reported raising the possibility of pathogenetic relationship.]

Lege Artis Medicinae

MAY 16, 2007

[SEVERE GASTROINTESTINAL AND RENAL MANIFESTATIONS OF HENOCH-SCHÖNLEIN PURPURA]

GECSE Krisztina, ONDRIK Zoltán, KAIZER László, VARGA Erika, LONOVICS János, CZAKÓ László

[INTRODUCTION - Henoch-Schönlein purpura is a systemic small vessel vasculitis characterized by vascular and/or mesangial IgA deposits, primarily affecting the vasculature of the skin, joints, kidneys and gastrointestinal tract. Gastrointestinal findings of various severity occur in 50 to 85% of the cases. We report on a 70-year-old woman who developed ileocaecal invagination and upper gastrointestinal haemorrhage as manifestations of Henoch-Schönlein purpura. CASE REPORT - The patient presented with two days history of palpable purpuric rash localized on the lower extremities. Based on the result of the skin biopsy, which showed leukocytoclastic vasculitis, IgA and C3 deposits, Henoch-Schönlein purpura was suspected. On the second night after admission colicky abdominal pain, vomiting and diarrhoea developed. Radiological examination showed an ileocaecal invagination and since symptoms deteriorated caecum resection and ileo-ascendestomy was performed. On the third postoperative day the patient became oligo-anuric, which was attributed to her Henoch-Schönlein disease, and systemic steroid pulse therapy was given. One month after the admission the patient experienced haematochezia. Emergency upper endoscopy revealed petechiae, haemorrhagic erosions and mucosal oedema, primarily in the descending part of the duodenum. Since these lesions were also considered as presentations of Henoch-Schönlein disease, another bolus of parenteral steroid was administered. Upper endoscopy repeated 3 days later showed remarkable improvement of the gastric and intestinal lesions. CONCLUSION - Gastrointestinal symptoms are common manifestations of Henoch-Schönlein purpura, thus their recognition, in which endoscopy plays a crucial role, is of major importance. Methylprednisolone pulse therapy is an effective therapeutic option not only in the management of severe renal or joint symptoms, but also in the treatment of gastrointestinal manifestations.]

Lege Artis Medicinae

JANUARY 27, 2009

[Ways and tools of the organism's defence in atherosclerosis - The mysterious HDL particle]

JAKAB Lajos

[Every type of injury elicits an immediate defending, preventing response in the living organism, which involves the whole organism’s participation. In case of a single effect, the events and symptoms together can be designated as “acute phase reaction syndrome”. The pathogenic constituents' effects on the arterial wall are, however, permanent, though varying in composition and intensity. The protecting reaction serving the prevention of the continuously arising vessel wall lesion is inflammation itself. Beginning of the inflammatory phenomenon carries within itself the criteria of the acute tissue response but it takes on involuntarily chronic features as a consequence of the chronic etiology. In the arterial wall ongoing vasculitis affects all the three layers of the vessels. The HDL particle is involved in triggering and progression of vasculitis. It transports cholesterol from the tissues to the liver, defends the integrity and physiological functions of the endothelium, exerts antioxidant effects, and it has the capacity to inhibit secretion and effects of cytokines, cell migration and the thrombotic process. These are all together serving as controllers of the ongoing vasculitis in the artery wall. Composition of the HDL particle is exceptionally complex. Almost half of the identifiable - at least 48 - components could be considered as acute phase reactants, and almost as many are related to cholesterol and lipoprotein metabolism. Participation of proteinase inhibitors and complement system components and regulators is very important for its function. Some constituents have a glycosaminoglycan binding capacity. The physiological roles of some acute phase reactants are not completely identified as today. This is especially valid concerning the characteristics of the HDL particle as an entity. Nevertheless, on the basis of present study data, it is ascertainable that HDL is an acute phase reactant; it is an inherent part of the natural, innate immune system of the organism. However, it is only one single component of the large group of the anti-inflammatory mechanisms and factors.]

Lege Artis Medicinae

FEBRUARY 20, 2005

[THE IMMUNPATHOGENESIS OF VASCULITIDES]

ZEHER Margit

[The vasculitides are clinicopathologic entities characterized by the inflammation and damage to blood vessels. Most vasculitic syndromes are mediated by immunopathogenic mechanisms. Most immune vasculitides are idiopathic (“primary vasculitis”) and systemic. The “immune vasculitides” are classified according to their hypersensitivity reaction types: allergic angiitis, antineutrophil cytoplasmic antibody (ANCA) -associated vasculitis, immunecomplex vasculitis, and vasculitis associated with T cell-mediated hypersensitivity. Immunological features: eosinophilia plus elevated IgE are characteristic of allergic angiitis and granulomatosis (Churg-Strauss syndrome). No immune deposits can be found in situ in ANCA associated vasculitis (“pauci-immune vasculitis”). Immune complex deposits, by contrast, are the hallmark of immune-complex vasculitis, which is frequently associated with low complement levels. Inflammatory infiltration induced by Th1 cells distinguishes vasculitis associated with T cell-mediated hypersensitivity (granulomatous arteritis). This article is dealing with the immunological mechanisms which may play a central role in the development of vasculitides.]

Lege Artis Medicinae

SEPTEMBER 15, 2006

[TAKAYASU’S ARTERITIS DETECTED IN AN EARLY PHASE]

SZÁNTÓ Antónia, VEISZ Richárd, KRENÁCS László, CSIKI Zoltán, GRIGER Zoltán, ZEHER Margit

[INTRODUCTION - Takayasu’s arteritis is an idiopathic inflammatory disease of the great elastic arteries, mainly of the aorta and its primary branches. It primarily affects young women. CASE REPORT - A 20-year-old woman presented with general inflammatory symptoms that had begun 3 to 4 months before. Earlier she underwent a biopsy from a mass on the neck thought to be a lymph node, which on histopathological examination turned out to be a non-necrosing granulomatosis in the wall of a large artery. Based on this finding as well as on the presence of fever, weight loss, carotid murmur, anaemia, elevated platelet count, increased sedimentation rate, the lack of pulse and the findings of imaging examinations, Takayasu’s arteritis was diagnosed. Treatment was started with high-dose steroid monotherapy, which resulted in clinical and laboratory remission after a short time. The imaging findings (neck soft tissue sonogram and MRI) also showed improvement, however, a moderate but definite disease activity was still present, thus steroid treatment was supplemented with azathioprin. CONCLUSION - It is important to follow the disease both clinically and with imaging techniques for an early recognition of the need for changing or adjusting the treatment.]