Lege Artis Medicinae

[NEUTROPENIA AND SUBSEQUENT INFECTION IN HEMATOLOGICAL DISEASES]

SINKÓ János

MAY 20, 2005

Lege Artis Medicinae - 2005;15(05)

[Neutropenia is an immunocompromised state commonly occurring in hematological practice. The underlying disorder responsible for a critical drop in absolute granulocyte count can either be of congenital or acquired nature. Neutropenic patients frequently develop serious, at times even fatal infections. Severity of illness, outcome, type of infecting organisms are markedly influenced by additional risk factors such as impaired T- or B-lymphocyte function as well as the injury of biological barriers. Neutropenic infections should generally be treated according to evidence-based guidelines. However, in certain groups of patients, where randomized trials are lacking, all identified components of immunodeficiency should be taken into account and antimicrobial treatment or prophylaxis should individually be tailored.]

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[SUBSTANCE ABUSE AND CARDIOVASCULAR SYSTEM]

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[Substance abuse is an immense problem in Hungary and around the world. Psychological effects of this condition are widely known for abusers and health care workers. Cardiovascular effects of illicit substances are much less well known and can develop as very severe diseases (myocardial infarction, extremely high blood pressure, intracerebral haemorrhage, sudden death etc.) and occur particularly in young people. The treatment of cardiovascular complications of substance abuse may differ from that accepted in non-abusers and some drugs (for example: β-blockers) may be contraindicated. In this review, the effects of substance abuse on the cardiovascular system are discussed, including effects of most commonly abused substances: cocaine, amphetamines, heroin, LSD, marijuana, inhalative substances. In this review the cardiovascular effects of sedato-hypnotics and most widely used substances (alcohol, nicotine, caffeine) are not adressed, since they are well known.]

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[ISOTOPE BASED CARDIAC DIAGNOSTICS - POSSIBILITIES IN NUCLEAR CARDIOLOGY]

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[Methods of nuclear cardiology have been applied for several decades and there is continuous development in this area. The most commonly used modality is the myocardial perfusion scintigraphy (MPS). During stress MPS, the presence and the severity of ischaemic heart disease (IHD) can be detected. Resting MPS can show a freshly developing acut myocardial infarction (AMI) immediately, but new and old infarcted myocardial areas can not be distinguished by this method. Using SPECT (single photon emission tomography) examination and quantitative analysis can improve the accuracy of MPS. With gated SPECT we can analyse both the perfusion and the function of left ventricle. To examine the function of left and right ventricle the “gold standard” non-invasive method is MUGA (multiple gated acquisition) of blood pool scintigraphy. After only a few hours of the onset of AMI we can detect it with the socalled infarct avid scintigraphy using radiopharmaceuticals which accumulate in affected area. Following an AMI it is essential to differentiate among high and low risk patients for revascularisation treatment, therefore distinguishing the viable (hibernating) and non-viable (necrotic) myocardium with imaging techniques is an important task. Preserved metabolism as the sign of viable myocardium can be detected both by SPECT (most accurately by thallium rest-redistribution scintigraphy) and PET (detecting glucose metabolism by F-18-FDG). Adrenerg receptor scintigraphy can show the sympathetic innervation: in the case of a transplanted heart it can detect the reinnervation and in the case of malignant ventricular tachyarrhythmias the risks and the severity of the illness.]

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[In the focus: Dermatology]

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Lege Artis Medicinae

[THE ROLE OF REGISTRY ANALYSIS IN THE FORMING OF THERAPEUTIC GUIDELINES IN RENAL TRANSPLANTATION]

PERNER Ferenc, HERCZEG Balázs, SZENOHRADSZKY Pál, ASZTALOS László, KALMÁR-NAGY Károly, JÁRAY Jenő

[The authors assess the two main outcomes of the immunosuppressive therapy after renal transplantation: graft and patient survival. According to their view, evidence from randomised clinical trials results can be well complemented by the several unique transplant registries and outcome research based upon these databases. The comparison of evidence from these two sources addresses the question of achievable outcome under ideal (controlled) versus real life conditions. Based on a systematic review of the relevant clinical trials and registries it can be stated, that in the case of some immunosuppressants (mycophenolate mofetil vs azathioprine, microemulsified cyclosporin vs cyclosporin and tacrolimus vs cyclosporin) the improvement in the intermediate outcome can lead to improved graft and patient survival, while in the case of other drugs no significant difference in hard endpoints were detected (tacrolimus vs microemulsified cyclosporin). Evidence on graft and patient survival differences could not be derived from traditional randomised clinical trials, only from transplant registries. For the sake of improved evidence based therapeutic guidelines in renal transplantation, authors call for further development of the Hungarian transplant registry.]

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[Felty’s syndrome can be regarded as “super-rheumatoid” disease. Immungenetically the syndrome is much more homogenous, than rheumatoid arthritis. HLA-DRB1*0401 antigen is present in 83% of the patients. Felty’s syndrome develops usually after a longer course of rheumatoid arthritis, in 1% of rheumatoid patients. Rheumatoid arthritis patients with long lasting unexplained neutropenia can be diagnosed having Felty’s syndrome, even without detectable splenomegaly. On the contrary, rheumatoid arthritis with splenomegaly, but without present or previous neutropenia with unexplained origin cannot be regarded as having Felty’s syndrome. Inspite of the fact, that the arthritis of Felty’s syndrome can be inactive, because of the neutropenia and increased risk of recurrent infections, the patients should be kept under tight supervision, and should be properly treated, if required. Immunologically Felty’s syndrome is characterized by rheumatoid factor positivity in 95-100%, ANA positivity in 50-100%, antihistone positivity in 63-83%. Antibodies against dsDNA rarely, but against ssDNA frequently occur. No anti Sm and interestingly no anti Ro and anti La antibodies can be detected inspite of the high incidence of associated Sjögren’s syndrome. Immunoglobulin levels are higher and complement levels are lower, than in rheumatoid arthritis. Circulating immuncomplex level is usually high. Non-specific antineutrophil anticitoplasmatic antibodies can be found in high percentage. The neutropenia of Felty’s syndrome can be either caused by increased IgG neutrophilic binding activity or by inhibition of the granulocytes colony growing in the bone marrow, by peripheral blood mononuclear cells. Expansion of large granular lymphocytes can be seen in 30-40% of patients with Felty’s syndrome. Large granular lymphocyte syndrome is not rarely associated with rheumatoid arthritis. The neutrophil account is normal or elevated in this syndrome, but splenomegaly occurs. These cases are called as pseudo Felty’s syndrome. The patients with Felty's syndrome suffering from recurrent infections required treatment even if the arthritis is inactive. Methotrexate treatment should be started first, if this treatment fails, other disease modifying drugs or colony stimulating factor can be given. There is no experience with other biological treatments. In treatment of resistant cases splenectomy is indicated. Non-steroid anti-inflammatory drugs should be better avoided.]

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