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Biomedical subjects

Ivana Jurić

Publications and source records attributed to Ivana Jurić.

5 recordsLinked to original sources
PubMed2006

[Anderson-Fabry disease].

Anderson-Fabry disease is a rare inherited X-linked lysosomal storage disease caused by deficiency of the enzyme alpha-galactosidase A. The deficiency of alpha-galactosidase activity leads to progressive, abnormal accumulation of neutral glycosphingolipids in the lysosome. With increasing age globotriaosylceramide (Gb3) progressively accumulates in different cells, tissues and organs throughout the body. The overall prevalence of Anderson-Fabry disease is 1:117.00 or 1: 40.000 in (male) population. Typically, the clinical onset of Anderson-Fabry disease occurs during childhood or adolescence, with early symptoms of neuropathic pain (recurrent episodes of severe pain in the extremities), angiokeratomas (characteristic cutaneous lesions), oedematous upper eyelids, peripheral vasospasm and ophthalmological abnormalities. The disease progresses through adulthood and by the age of 30-40 years several major organ systems may be affected; cardiac disease, renal insufficiency, cerebrovascular attacks and neurologic findings are common. Death usually occur secondary to renal, cardiac or cerebrovascular complications during the fourth or fifth decade of life. Enzyme replacement therapy is a major advance in the treatment of rare diseases. In 2001 two formulations have been approved by the European Medical Evaluation Agency, agalsidase alpha and agalsidase beta. Agalsidase alpha is produced on the human fibroblast cell line, and agalsidase beta from the Chinese hamster ovary cell line.

Fabry Disease↗
PubMed2006

[Principles of iron therapy in hemodialysis patients].

Anemia associated with chronic renal disease remains a major concern for nephrologists as it significantly increases the morbidity and mortality in this patient group. The introduction of erythropoietin has dramatically changed the treatment of anemia in uremic patients. However, some patients showed hyporesponsiveness to erythropoietin because of inadequate iron supply to the erythroid bone marrow. Patients undergoing chronic intermittent hemodialysis are especially vulnerable to iron deficiency due to blood loss during the hemodiaylsis sessions, gastrointestinal bleeding and compromised gastrointestinal absorption. Demand for iron is also increased by the treatment for anemia with erythropoietin. Intravenous administration is more effective than oral iron supplemantation in renal failure patients. Some studies have raised concerns of the potential serious side effects associated with intravenous iron administration. Besides anaphylactic reactions that were reported to occur in less than 1% of patients treated with iron dextran and have not been associated with other iron formulations, concerns about the long-term use of iron include the increased risk of infections and oxidative stress with consequent cardiovascular disease. Therapy with dextran-free iron formulations is an essential part of anemia treatment protocols, and was not found to be associated with either short- or long-term serious side effects.

Anemia, Iron-Deficiency↗
PubMedDate not supplied

[Regulation of blood pressure in dialyzed patients].

Up to 90% of chronic haemodialysis patients have blood preasure (BP) greater than 140/90 mmHg. This suggests that only a minor number of the haemodialysis patients have adequate BP control. This is associated with significantly increased morbidity and mortality in haemodialysis population when compared with normal, healthy population. The main aim of antihypertensive treatment in hypertensive haemodialysis patients is to achieve BP values which should not differ from those recommended for general population. The most important factor in BP regulation in haemodialysis patients is adequate fluid volume regulation. Sympathetic nervous system and impaired vasodilatation with consequent changes in peripheral vascular resistance, secondary hyperparathyreoidism and its effects on calcium balance and consequent effects on cotractility of the smooth muscle cells of the vessel wall, correction of renal anemia in patients receiving human recombinant erythropoietin, regulation of salt intake, and frequency and duration of haemodialysis procedure have also a significant role in BP regulation in these patients. If dialysis procedure is not adequate, meaning that salt and water balance is not satisfyng, antihypertensive medications cannot alone control BP in haemodialysis patients.

Blood Pressure↗