Lege Artis Medicinae

[Management of inhibitory haemophilia A and aquired haemophilia]

PFLIEGLER György1, RÁK Kálmán1, KRÁLL Géza2, SAS Géza2, HAYNAL Imre2

FEBRUARY 28, 1994

Lege Artis Medicinae - 1994;4(02)

[The authors survey the occurrence and the laboratory and clinical differences of factor VIU antibodies in haemophiliacs (allo) and in non-haemophiliacs (auto) as well as their titer and measurement. The recent therapeutic modalities used in prevention, management of acute bleeds and suppression of antibody development or in duction of immune tolerance are detailed. ]

AFFILIATIONS

  1. Debreceni Orvostudományi Egyetem II. Belklinika
  2. Egészségtudományi Egyetem Hematológiai Tanszék Budapest

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Further articles in this publication

Lege Artis Medicinae

[Back pain]

ANDREW Frank

[The studies to be presented show that the duration and severity of episodes of low back pain can be reduced, thus reducing the risk of recurrence and the costs of sick leave and absenteeism. Frank reviews differential diagnosis; acute, chronic and intractable pain; and the health services needed. Modern treatment principles emphasise self-care at home, usually with no more than 48 hours bed rest. The importance of regaining physical fitness and resuming other activities, including work, is also stressed. Medication helps to achieve these goals. Two issues are emphasised: the strategy for the management of low back pain should be long-term and preventive; it is the responsibility of the patient, not the doctors, to maintain fitness, exercise regularly, do relaxation exercises and avoid damaging the spine. ]

Lege Artis Medicinae

[Eating attitudes and eating disorders among secondary school dancers: a high risk group?]

TÚRY Ferenc, WILDMANN Márta, LÁSZLÓ Zsuzsa, JOÓ Mária Nóra

[The authors assessed the morbidity of eating disorders and the frequency of pathological eating attitudes in two secondary school populations in a two-stage study. The first sample consisted of two classes specialized in dancing (n=54), the second was a control group of two age-matched classes without specialization (n=61). In each group one girl met the criteria of bulimia nervosa, which was 2% of the whole population. No anorectic subject was identified while pathological eating attitudes were significantly higher among dancers characterized by an increased slimness ideal. On the basis of the study the risk of eating disorders is higher in this special population. However, this difference can be shown only in the frequency of subclinical, attitudinal disorders, not in that of clinically severe syndromes.]

Lege Artis Medicinae

[Heart attack primary prevention in hypertension trial]

MATOS Lajos

[Comparison of diuretic versus beta-blocker antihypertensive treatment in men with mild-to-moderate hypertension in terms of nonfatal infarction, coronary mortality and all-cause mortality. ]

Lege Artis Medicinae

[Correspondence]

SELMECZY Kamill

[Reflections on GP (family doctor) reform, training and more]

Lege Artis Medicinae

[Technical considerations to the anti-endomysium antibody assay]

KORPONAY-SZABÓ Ilma, B. KOVÁCS Judit, LŐRINCZ Margit, KÖRMENDY Miklós, SASHEGYI Julianna

[Anti-endomysium antibody and its equivalent anti-jejunum antibody were investigated in 5000 and 3444 serum samples, respectively. 2928 persons, among them 441 are known gluten intolerant patients (170 coeliac disease proven by gluten challenge, 95 dermatitis herpetiformis, 176 suspected coeliac disease with at least one jejunal biopsy result showing severe villous atrophy); 251 by biopsy proven non coeliac children served as controls. The mentioned antibodies were detected by indirect immuno fluorescence in IgA (in IgA-deficient persons also in IgG) subclass. Specificity of both anti-endomysium and anti-jejunum antibody results was 100% for gluten intolerance. Sensitivity in gluten consuming patients was: 100% in proven coeliac disease (66/66), 92,4% (61/66) in dermatitis herpetiformis and 85,9% (110/ 128) in suspected coeliac disease. 13,5% of all sera contained aspecific IgA-stainings of the following types: anti-smooth muscle, basement membrane, pemphigoid-like and anti-nuclear faktor like antibodies. Simultaneous positivity for anti-endomysium and aspecific antibodies occured too. Assessment accuracy have been improved with the use of 8–10 microns thick tissue sections. Detection of this specific autoantibodies merits first line use in the diagnosis of gluten sensitive enteropathy, but specialized laboratory centers are needed to overcome technical pitfalls. ]

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[This paper provides a comprehensive review of a new, innovative formulation of one of the cornerstones of immunosuppressive maintenance therapy, tacrolimus (LifeCycle Pharma Tac, LCP-Tac, LCPT). This brief overview provides a deeper insight into the scientific literature of LCP-Tac. In summary, once daily LCP-Tac, a medication available in routine clinical practice, can provide effective and safe blood levels in renal transplant patients. Furthermore LCP-Tac may require lower doses of tacrolimus due to increased bioavailability thanks to the innovative MeltDose® technology.]

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FARKAS Klaudia, JANKOVICS István, MELLES Márta, NAGY Ferenc, SZEPES Zoltán, WITTMANN Tibor, MOLNÁR Tamás

[INTRODUCTION - Inactivated influenza and H1N1 vaccination is recommended yearly for patients with inflammatory bowel disease receiving immunosuppressive therapy; however, immunomodulator and biological therapy might impair the immune response to the vaccination. In our study, we assessed whether immunity can develop in response to H1N1 influenza vaccination in patients receiving immunomodulator and/or biological therapy. We also assessed the occurrence of side effects after the immunisation in these patients. PATIENTS AND METHODS - In our prospective study, blood samples were obtained from 24 patients (12 Crohn’sdisease, 12 ulcerative colitis) one month after immunisation against influenza A/California/ 07/2009 (H1N1) virus. At the time of vaccination, all patients have been receiving immunomodulator and/or biological therapy for at least three month. Antiviral antibodies were detected by using microneutralisation assay. The safety of the vaccination was assessed by questionnaires. RESULTS - Every patient developed complete immunity against influenza A (H1N1) virus, independently from the type of immunosuppressive therapy. Regarding side effects, local symptoms occurred in six patients and systemic symptoms in another six patients. Mild diarrhea occurred in five patients. Moderate exacerbation of the disease was observed in 2 patients with Crohn’s disease and in one patient with ulcerative colitis. CONCLUSIONS - According to our results, immunocompromised patients with IBD can be safely advised to receive the vaccination. In our study, all patients developed adequate immunity according to microneutralisation titers.]

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[Hungarian Vasculitis Registry – results of the first five years]

HARIS Ágnes, TISLÉR András, ONDRIK Zoltán, FILE Ibolya, MÁTYUS János, ZSARGÓ Eszter, DEÁK György, AMBRUS Csaba

[Launching the Hungarian Vasculitis Registry aimed to collect information about prevalence and outcome of our patients with ANCA-associated vasculitis, and treatment protocols of the disease. The on-line data collection has been developing dynamically since its initiation five years ago, presently 278 patients’ files are available. Patients’ mean age is 58.2±14.5 years, 62% are women; their disease is associated with c-ANCA positivity in 51% and p-ANCA in 49%. At diagnosis GFR was 24.6±21.6 ml/min/1,73 m2, that time 29%, during the total follow up 39% of the registered subjects needed dialysis. Renal replacement therapy could be discontinued in 23% of them. In cases with focal histological changes, also with upper respiratory tract and skin involvement dialysis was significantly less frequently necessary, which underlines the importance of early diagnosis. In induction therapy steroid was administered for 94% of the patients, 85% of them got cyclophosphamide, 59% was treated by plasmapheresis, 11% got rituximab. Maintenance treat ment contained steroid in 80%, per os cyclophosphamide in 23%, parenteral cyclophosphamide in 22%, furthermore 40% of the patients got azathioprin, 8 subjects got mycophenolate and 6 got methotrexate. Median follow up was 30 months (IQR 6-78), during which period 20% of the patients died, 5% got kidney transplantation, and 5% were lost to follow up. Median survival was 14.8 years, five years survival was 85%, and ten years survival was 70%. Long term survival in patients with c-ANCA vasculitis seemed better comparing to p-ANCA vasculitis, but when correcting by age this difference disappeared. Predictors of death were age and dialysis dependent renal failure. Relapses developed in 27% of patients, 28% of them presented in the first year, 21% suffered it after five years of care. Collected data by the Hungarian Vasculitis Registry shows our society’s successful professional activity. Our results are comparable to the published data in the literature, yet there are several areas in our care where further improvements are warranted in order to increase our patient’s survival and quality of life.]

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[Current diagnosis and treatment of membranous nephropathy]

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[Primary membranous nephropathy is a common glomerular disorder characterized by subepithelial immune deposits. The pathomechanism underlying these lesions has only recently been elucidated: M-type phospholipase A2receptor (PLA2R) protein emerged as being the leading autoantigen. Antibodies to PLA2R, typically of IgG4 subclass are expressed in 70-80% of patients with primary membranous nephropathy. The level of autoantibody to PLA2R was shown to correlate with disease severity and to change parallel with disease activity in response to therapy. While mild forms of the disease are prone to spontaneous remission and carry excellent prognosis, severe forms often progress into end-stage renal disease without treatment and necessitate immunosuppression. The latest guidelines recommend the application of corticosteroids with alkylating agents or calcineurin inhibitors as first-line therapy. Promising new therapies that are currently being explored for this disease include rituximab, mycophenolate mofetil, and adrenocorticotropic hormone.]

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[Systemic ANCA-associated vasculitis. Induction immunosuppression therapy, complications and outcome. Part 1]

HARIS Ágnes, POLNER Kálmán

[The present review is compiled of two parts, the first part aims to summarize the induction immunosuppressive therapy, the second part delineates the outcome and complications of ANCA-associated vasculitis. ANCA-associated vasculitis is a systemic disease, accompanied with rapidly progressive glomerulonephritis and severe, often life-threatening extrarenal complications. By early diagnosis and immediate initiation of immunosuppressive therapy, both patient and renal outcome have been substantially improved. The major aims of modern therapeutic protocols are, besides improving survival, to decrease immunosuppressive drug toxicity and avoid infections. Immunosuppression is based on the combination of large dose of corticosteroid and cyclophosphamide, which is advisable to supplement by plasma exchange. The B-cell depleting anti-CD20 monoclonal antibody rituximab, which has already been available in Hungary, has been proved to be similarly effective in newly diagnosed ANCA-vasculitis, and even more effective in a relapsing disease, compared to cyclophosphamide. Amongst rituximab’s further indications in this disease is the preservation of young women’s fertility, and it also has priority in some other special cases. Early diagnosis and prompt immunosuppressive treatment have resulted that ANCAvasculitis became a treatable disease with reasonably good clinical outcome, yet both the disease and the immunosuppressive medications frequently cause complications, which necessitate continuous alertness of the attending nephrologists.]