Lege Artis Medicinae

[Analysis of short-term and long-term survival and causes of death in patients with systemic lupus erythematosus]

TARR Tünde, KISS Emese, SZEGEDI Gyula, ZEHER Margit

MARCH 20, 2011

Lege Artis Medicinae - 2011;21(03)

[INTRODUCTION - In systemic lupus erythematosus (SLE), both short-term and long-term survival rates have improved worldwide. We analysed retrospectively the short-term and long-term survival data and causes of death at a single center. These data were compared with previous survival data recorded at the same centre and published in international studies. PATIENTS AND METHOD - The data of 550 patients with SLE were analysed between 1970 and 2009. We examined the effect of clinical symptoms, age, severity and onset of the disease and the applied immunosuppressive treatment on survival, using the Kaplan-Meier method. RESULTS - Survival rates at 5, 10, 15 and 20 years after the diagnosis were 98%, 94%, 90% and 89%, respectively. Late onset, neuropsychiatric symptoms and severe SLE were found to be prognostic factors. Manifestations affecting other organs and the applied immunosuppressive therapy did not influence survival rates. During the study period, 57 out of the 550 patients (10.4%) died. The main causes of death were cardiovascular complications (50.9%), infections (21%), and malignancies (12.3%). CONCLUSIONS - Our results show that among patients with SLE, it is mostly longterm survival that has increased, owing to the close control of patients. The increase in cardiovascular mortality highlights the importance of regular screening.]

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Lege Artis Medicinae

[Perindopril plus Indapamid CombinAtion blood preSSure reductiOn study (PICASSO)]

FARSANG Csaba

[INTRODUCTION - International and Hungarian (JNC-7, ESH/ESC2007 és 2009, MHT 2009) Guidelines suggest a target blood pressure <140/90 mmHg for hypertensive patients, and <130/80 mmHg for those with high/very high cardiovascular risk (e.g. patients with diabetes mellitus, chronic renal disease). It was proved that for achieving the most efficient antihypertensive effect and reducing side effects, thd use of drug combinations is needed in most patients. In Hungary, ACE-inhibitor plus diuretic combination is one of the most frequently used one in Hungary. The aim of the PICASSO study was to evaluate the efficacy and metabolic effects of the fixed combination of high-dose perindopril plus indapamide (Coverex-AS Komb Forte®) in clinical practice of hypertonologists, cardiologists and general practicioners. PATIENTS AND METHODS - Patients with uncontrolled, grade 1 or 2 primary hypertension, age, >18years were involved in the open, 3-month, multicentre, prospective, observational, non-interventional clinical study if the treating physician indicated a combination treatment with higher dose antihypertensive drugs. Blood pressure (also with ABPM), heart rate, metabolic parameters (plasma lipids, blood sugar, Na, K, creatinine, uric acid, GGT) were measured by routine methods, medical history and quality of life parameters were registered on a validated questionnaire. Changes in the above parameters were also separately evaluated in patients with different risk factors or with concomitant diseases. RESULTS - Data of 9683 patients were evaluated (54% women, 46% men, average age 61.8 years). By the end of the therapy used in the study, blood pressure average decreased from 159/93 to 131/80, by ABPM from 145/83 to 126/74 mmHg (24hr averages, systolic/diastolic blood pressure, respectively), the heart rate from 79 to 73 beats/min (p<0,001). Target blood pressure was achieved in 75,4% of patients with no major concomitant disease, but only in 14% of those with a concomitant disease characterised by lower target blood pressure. The diurnal index by ABPM did not change substantially. Clinically significant decreases were found in the plasma levels of total cholesterol, LDL-cholesterol, triglycerides, fasting glucose and uric acid levels, but there were no major changes in serum levels of HDL-cholesterol, Na, K, and GGT. Quality of life parameters significantly improved by the end of the study. CONCLUSIONS - Combinations of perindopril and indapamide can be successfully and safely used in everyday practice.]

Lege Artis Medicinae

[Gustav and Alma Mahler ]

KÖVES Péter

Lege Artis Medicinae

[Creatine deficiency - a possible cause of mental retardation]

VOJCEK Eszter, BARÁTH Ákos, SZTRIHA László, TÚRI Sándor, KARG Eszter

[Creatine plays a major role in the energy supply of brain and muscle tissues. In creatine deficiency states, insufficient energy supply of the brain tissue may lead to mental retardation with speech delay and behavioural problems, epilepsy and extrapyramidal symptoms, while that of the muscle tissue might result in hypotonia. Creatine deficiency has been shown to result either from dysfunction of the two enzymes involved in its synthesis, namely arginine:glycine amidinotransferase and guanidinoacetic methyltransferase, or from impairment of the SLC6A8 transporter protein that is responsible for the cellular uptake of creatine. Synthesis disorders can be efficiently treated by creatine supplementation. The therapy is the most efficient if it is started in time, before the development of irreversible damages. Thus, in case of mental retardation associated with delayed speech development or behavioural problems, or epilepsy of unknown origin, diagnostic tests for creatine deficiency are recommended as early as possible. In creatine-transport disorder, the results of treatments to increase the intracellular creatine pool are controversial]

Lege Artis Medicinae

[Three Roles: – Chekhov as the Author, the Physician and the Patient]

KISS László

Lege Artis Medicinae

[Clinical reasoning based on Bayes-theorem]

VOKÓ Zoltán

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[Aims - Primary spinal tumors are rare diseases and there are less objective data in the international literature. We analyzed the epidemiology and clinical consequences of primary spinal tumors based on the clinical experience of the National Center for Spinal Disorders. Methods - Demographic and clinical data of 300 patients treated in our institute between 1995 and 2007 was collected retrospectively and analysed. Results - Beyond the relatively more frequent pathologies (chordoma, myeloma multiplex) we treated in our hospital some of the very rare types of tumors (spinal leiomyosarcoma, synovial sarcoma). Primary spinal tumors are most often located in the lumbosacral region causing most frequently (73%) local or radiating pain. Modern therapy of these patologies is based on the surgical intervention. Mean operation time was 130 minutes, mean blood loss was 650 ml in our pratice during these often technically challenging surgeries. We found a significant association among the operation time, the blood loss and the extension of the tumor (p<0.01). Histology (p<0.0001), severity of symptomes (p<0.05) and blood loss (p<0.05) were significantly related to mortality. Local recurrence was more than 5-fold in case of patients previously operated in another institute (p<0.0001). Conclusions - We successfully determined some significant prognostic factor on clinical behavior of primary spinal tumors performing a large scale retrospective study. Long time follow up of the patients and completion of our database with prospective data are planned for the future.]

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[The authors have investigated the survival of 238 patients on chronic haemodialysis program regarding serum albumin levels (measured at starting dialysis and at the end of observation) adjusted for age, gender, diabetes, serum haemoglobin and body weight. The mean observational period was 5.5 years. Our investigation has documented a tight positive correlation between the survival and serum albumin levels both at starting and ending of investigation independently of epidemiological parameters. It was demonstrated that serum albumin levels decrease by aging, so the authors recommend a little bit lower serum albumin target level for older dialyzed people, because the normal range is also decreasing by aging. The level of serum albumin has not shown changing in younger patients (less than 60 years) in this long observational period, but there was a significant decrease in olders (especially above 70 years).]

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GULÁCSI László, MÁJER István, KÁRPÁTI Krisztián, BRODSZKY Valentin, BONCZ Imre, NAGY Attila, BERECZKI Dániel

[The aim of our research was to assess the incidence and the 12- and 24-month mortality of hospitalized stroke in Hungary. We analyzed the rate of mortality after stroke and compared it to the standard mortality rate of the population. To assess the incidence we extracted the data of “new” stroke patients (ICD- 10 diagnoses: I60-64) hospitalized in May 2003 from the database of the National Health Insurance Fund Administration. We regarded those as “new” patients who had not been treated with these primary or secondary diagnoses in the previous 24 months. Data were collected by sex and age (age groups: 25-44, 45-64, 65 and over). We analyzed the patients' survival on the basis of their April 2004 and April 2005 data. The incidence of the “new” hospitalized stroke patients was higher in men than in women; the incidence in the age group of 65 and over was 2112/100.000 in males and 1582/100.000 in females, the corresponding values in the 45-64 age group were 623 vs. 366 per 100.000, respectively. In 2003 more than 42 thousand “new” stroke patients were hospitalized in Hungary of whom over 10 thousand died in the first year, followed by a further 2 thousand in the second year. Women’s survival is more favourable than men's: in the first year it is 71.47% vs. 69.24% (65+ group), and 88.18% vs. 83.16% (45-64 group); in the second year the corresponding values are 66.95% vs. 61.62% (65+), and 85.45% vs. 80.90% (45-64), respectively. The risk of death in the first year after stroke, compared to the standard population, is 5.17- fold in women and 4.70-fold in men in the total sample, and 10-15-fold in the 45-64 group. There are large differences by gender, particularly in men of the working age groups (25-44, 45-64), whose mortality is twice as high as that of women of the same age.]

Lege Artis Medicinae

[NOVEL RESULTS ON SYSTEMIC LUPUS ERYTHEMATOSUS]

KISS Emese, GERGELY Péter, SZEGEDI Gyula

[Systemic lupus erythematosus is an autoimmune disorder. It stands at the focus of medical interest: basic scientists, clinicians and innovative biotechnologists all pay attention to lupus. Authors of this article present the novel scientific results on the etiopathogenesis, clinical and laboratory characteristics of SLE. Furthermore, authors discuss diagnostic problems and the possible therapeutic modalities. One of the most important results is the characterisation and mapping of the susceptibility genes as well as the analysis of their functional features. More and more is known about the relationship between natural and adaptive immunity, about the cooperation of T and B cells. The abnormalities of intracellular biochemical processes and signal transduction pathways have been cleared. The importance of cytokine network and infective agents in the pathogenesis of SLE has largely been investigated recently. With regards to the outcome of the disease, there is growing attention paid on chronic organ damages, such as end-stage renal disease, osteoporosis and atherosclerosis - in connection with the increased life expectancy. Evidence accumulates on the importance of immune-inflammatory processes in the initiation and perpetuation of osteoporosis and atherosclerosis. There is an urgent need for validated biomarkers which can predict the susceptibility, prognosis, clinical manifestations, activity and severity of SLE. To follow and measure the effectiveness of treatment is also required. Although the principals of lupus management have not changed, novel immune modulators, biological therapy and non-medical treatments (e.g. stem-cell transplantation) have become available. Further research and clinical observations may help to find the real place of such therapeutics.]

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