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Biomedical subjects

Nikolina Basić-Jukić

Publications and source records attributed to Nikolina Basić-Jukić.

18 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

[Current trends in renal transplant immunosuppression].

Several new immunosuppressives have been introduced in renal transplantation during the past decade. Mycophenolate mofetil, tacrolimus, cyclosporine microemulsion, sirolimus, monoclonal and polyclonal antibodies have all contributed to decreasing the rate of acute rejection. However, a more intense immnuosuppression has resulted in an increased incidence of opportunistic infections and malignant diseases. The main cause of death in renal transplant recipients are cardiovascular diseases, and the majority of patients die with a functioning graft. Renal transplant recipients are at an increased risk of the development of cardiovascular diseases due to prolonged uremia and dialysis itself, combined with the proatherogenic effect of immunosuppressive drugs. Corticosteroids and calcineurin inhibitors are the main groups of drugs associated with an increased risk of atherosclerosis. The main challenge in the present and future treatment of transplanted patients is the establishment of an immunosuppressive protocol that will minimize or completely avoid proatherogenic immunosuppressives while preserving the low incidence of acute rejection.

Adrenal Cortex Hormones↗
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↗
PubMed2004

[Biological adequacy--what does it mean?].

Appropriate initiation of dialysis is of an outstanding importance in the treatment of patients with end-stage renal disease. It prevents development of irreversible uremic complication and enables selection of the most appropriate dialysis modality for the individual patient. The major causes of morbidity and mortality in dialysis patients are cardiovascular diseases. Hypertension and hyperlipidemia are commonly found in dialysis patients as well as anemia, chronic inflammation and fluid overload, all of which are found to be risk factors for the development of cardiovascular diseases. Arterial hypertension is the main risk factor for left ventricular hypertrophy, and there is clear evidence that control of hypertension has a beneficial effect on left ventricular hypertrophy. It is best achieved by correction of overhydration and maintenance of dry weight. Modern dialysis machines are capable of changing electrolyte concentrations, which reduces intradialytic cardiovascular complications, incidence of cardiac arrhythmias and hypotension. Correction of anemia with erythropoietin results in regression of left ventricular hypertrophy and improvement of the quality of life and defense against microorganisms. Chronic inflammation can be prevented with the use of biocompatible high-flux dialysis membranes and sterile dialysate, which are also important for the prevention of oxidative stress involved in the increase of LDL oxygenation and incorporation into the intimal layer of the vessels. Low molecular weight heparins by their action on lipoprotein lipase serve as an additional factor that suppresses development of atherosclerotic plaque in dialysis patients. Optimal dialysis dose decreases the mortality and morbidity rates. High-flux membranes or prolongation of dialysis session are modalities for dialysis dose improvement. Individualized approach to preparation of dialysis solutions has resulted in better control of fluid overload and intradialytic hyper- or hypotension, reduction in the incidence of arrhythmias, improvement of hemodynamic stability, and delay of renal osteodystrophy. Malnutrition is a relatively common problem in dialysis patients that may be secondary to poor nutritional intake, inadequate amount of dialysis, lack of appetite, acidosis, associated disease, and/or increase in protein catabolism. The most appropriate approach includes individualization of dietary prescription according to the nutritionist's advice, increase of dialysis dose with biocompatible membranes, and use of sterile bicarbonate dialysate with glucose and erythropoietin. The major goal of adequate dialysis is not just improvement in survival of dialysis patients, but also improvement in the quality of their lives.

Humans↗
PubMed2004

[Uremic polyneuropathy].

Uremic polyneuropathy is probably the most common complication of chronic renal failure. About 70 percent of dialysis patients regularly have uremic polyneuropathy, in 30 percent the neuropathy is moderate or severe. Coexistence of muscle weakness and atrophy, areflexia, sensory loss and graded distribution of neurologic deficit in a patient with renal disease suggests the presence of uremic polyneuropathy. During longterm hemodialysis, the symptoms of polyneuropathy stabilize, but they improve only in relatively few patients. Complete recovery, occurring over a period of 6 to 12 months, usually follows successful renal transplantation.

Humans↗
PubMed2004

[Importance of plasmapheresis in the treatment of paraneoplastic cerebellar degeneration].

Paraneoplastic neurologic syndromes are disorders of the nervous system function caused by cancer but not due to metastatic disease, vascular or metabolic deficits, infections, nutritive deficiency, nor side effects of antineoplastic drugs or irradiation. Immunologic factors probably play the crucial role in the pathogenesis of paraneoplastic neurologic syndromes, but nonimmunologic mechanisms that include metabolic abnormalities and competition for substrate are also involved. Paraneoplastic cerebellar degeneration most commonly occurs in the setting of gynecologic cancers, but it accompanies the small-cell lung cancer too. Other tumors are infrequently associated with cerebellar degeneration. Several paraneoplastic antibodies have been identified in patients with paraneoplastic cerebellar degeneration. Their association with particular cancers may help identify an occult lesion. Anti-Yo antibodies are directed against Purkinje cell antigens and occur in patients with cerebellar degeneration who have breast cancer or gynecologic tumors. A target antigen of anti-Yo antibody is CDR2 protein that is normally expressed only in the brain and testis. Patients with paraneoplastic cerebellar degeneration present with dizziness, nausea and vomiting followed by gait instability, diplopia, gait and appendicular ataxia, dysarthria and dysphagia. Therapeutic options include tumor excision, chemotherapy and/or irradiation, and adjuvant therapy with glucocorticoids, immunoglobulins and plasmapheresis. The role of plasmapheresis in the treatment of paraneoplastic cerebellar degeneration is still uncertain. Reports of its efficacy are anecdotal. We present patient with paraneoplastic cerebellar degeneration with positive anti-Yo antibodies and tumor of the ovaries whose neurologic status significantly improved after four daily plasmaphereses, which was accompanied by a fourfold decrease in the anti-Yo antibodies titer. Further investigations are needed to define a protocol for plasmapheresis in the treatment of patients with paraneoplastic syndromes.

Autoantibodies↗
PubMed2004

[Hyperphosphatemia and cardiovascular risk in patients on dialysis].

Cardiovascular diseases are the leading causes of mortality among patients with end-stage renal disease (ESRD), with arterial disease and left ventricular hypertrophy being the two principal factors of the high mortality rate in this population. In addition to traditional risk factors (age, gender, diabetes, hypertension, lifestyle, hyperlipidemia, smoking, hyperhomocystinemia), inflammation, oxidative stress and disorders of mineral metabolism may contribute to cardiovascular risk in patients with uremic syndrome. High serum phosphate may influence vascular calcifications directly and indirectly, by worsening secondary hyperparathyroidism. Several treatment options are available for the treatment of hyperphosphatemia and secondary hyperparathyroidism in patients with ESRD. The treatment approach includes a diet low in phosphorus, with less than 1 g/kg/day of protein. Vitamin D supplementation is an important part of treatment. Phosphate binding agents are in most of the patients necessary in addition to diet. Aluminum hydroxide has been widely used for many years. It is very potent, but also very toxic, with severe encephalopathy as the most dangerous side effect. Calcium salts are less potent, and were considered safe for use in patients on dialysis. However, improvement in the understanding of vascular calcifications has demonstrated that calcium overload significantly contributes to widespread atherosclerosis in patients with ESRD. Sevelamer-hydrochloride is a novel non-aluminum, non-calcium containing phosphate binder, which is capable of reducing the levels of phosphorus as well as of low-density lipoprotein cholesterol, and increasing high-density lipoprotein cholesterol.

Blood Vessels↗
PubMed2004

[Interleukin-2 receptor blockers in renal transplantation].

The last two decades have witnessed significant advances in the renal transplantation immunosuppressive protocols. The introduction of mycophenolate mofetil, tacrolimus, sirolimus and polyclonal antibodies has significantly improved graft survival. However, intensification of immunosuppression results in complications such as malignant diseases, opportunistic infections and metabolic disturbances with consequential increase in cardiovascular mortality. Advances in molecular engineering have made possible the development of monoclonal humanized or chimeric antibodies, which will not induce the host immune response with production of neutralizing antibodies or serum sickness. Antibodies directed against the alpha chain of human IL-2 receptor have recently been introduced into immunosuppressive protocols. Daclizumab is a humanized antibody, and basiliximab is a chimeric antibody, engineered by cloning segments of the murine immunoglobulin sequence into the human-immunoglobulin gene. It decreases immunogenicity while maintaining high specificity for IL-2R alpha chain. The efficacy and safety of both preparations have been reported in large randomized studies. Their use in induction resulted in a significant decrease in acute graft rejections after renal transplantation. The possibility of decreasing the dose or complete withdrawal of certain immunosuppressive agents with the use of IL-2R blockers seems promising for further improvement in the longterm graft survival. Longterm follow-up is necessary to determine their role in solid organ transplantation.

Antibodies, Monoclonal↗
PubMed2003

[Indications for continuous renal function replacement therapy].

One of the most important achievements in the contemporary intensive care management is introduction of continuous renal replacement therapy (CRRT). The most common indications for CRRT are acute renal failure complicated with heart failure, volume overload, hypercatabolism, acute or chronic liver failure, and/or brain swelling. Less common indications include systemic inflammatory response (SIRS), sepsis, multiorgan failure (MOF), adult respiratory distress syndrome, crush syndrome, tumor lysis syndrome, lactacidosis, and chronic heart failure. Methods of CRRT could be used during or after open heart operations, heart, lung or/and liver transplantation in adults and children. Modern approach to treatment of acute renal failure introduces dialysis early in the course of disease in order to avoid complications on other organs. Sepsis, SIRS and septic shock are still major therapeutic problems in intensive care units with a mortality rate over 50%. Numerous uncontrolled and several controlled clinical studies have demonstrated that CRRT could remove inflammatory substances including cytokines, activated components of the complement, and derivatives of the arachidonic acid. Hemodynamic stability and gas exchange in the lungs were significantly improved. These is due not only to removal of inflammatory substances but also to other nonspecific hemodynamic effects (control of body temperature, fluid and metabolic balance). Besides the convection, cytokines could be removed from the plasma with adsorption on the membrane of dialyzer or hemofilter. Prophylactic use of CCRT in patients with normal renal function, without disturbances in fluid excretion and with normal hemodynamics is still controversial, while the possible benefit is not higher than the risks of invasive therapeutic method, and there is no evidence that prophylactic CCRT could prevent development of acute renal failure in these patients. However, current knowledge of MOF pathophysiology justifies the use of CRRT in patients with signs of heart failure, disturbances in metabolic and fluid homeostasis and sepsis, and in patients with the risk of developing acute respiratory failure or MOF, despite the mild impairment of renal function according to laboratory results.

Humans↗
PubMed2003

[Immunosuppressive drugs in the prevention and treatment of GVHD after allogenic bone marrow transplantation].

Graft-versus-host disease (GVHD) remains the main problem after the allogeneic bone marrow transplantation. It is extremely complicated to maintain the finely organized set of immunologic events in order to prevent GVHD and thereby retain the graft-versus-leukemia effect. There are an increasing number of immunosuppressive drugs available for prevention and treatment of GVHD. Cyclosporine, sirolimus, FK506, mycophenolate mofetil and CTLA-4 Ig prevent activation of donor T-lymphocytes, while daclizumab and infliximab act on effector cells. It is hard to believe that it would be possible to prevent and cure GVHD with a single agent; combination of immunosuppressive drugs that act on different phases of GVHD induction seems promising.

Bone Marrow Transplantation↗
PubMed2003

[Continuous venovenous hemofiltration as a bridge to heart transplantation in a patient with congestive heart failure and acute renal failure].

Continuous venovenous hemofiltration effectively controls volume overload in cases of severe congestive heart failure accompanied by acute renal failure that could not be medicamentously controlled. A patient with severe ischemic dilated cardiomyopathy who developed acute renal failure while waiting for urgent heart transplantation is described. He was treated with CVVH for three days when the occasion for heart transplantation appeared. At the time of transplantation laboratory markers of renal function were within the normal range with stable hemodynamic parameters. After successful transplantation the patient spontaneously and completely recovered his renal function.

Acute Kidney Injury↗
PubMed2002

Hodgkin s disease with nephrotic syndrome as a complication of ulcerative colitis: case report.

We describe a case of a 32-year-old patient with ulcerative colitis complicated by Hodgkin s disease who presented with nephrotic syndrome. The patient had suffered from relapsing ulcerative colitis for 6 years before he developed Hodgkin s lymphoma. He was treated for Hodgkin s disease with 9 cycles of combined chemotherapy (COPP/ABV) and achieved the stabile remission of lymphoma, nephrotic syndrome, and ulcerative colitis. To the best of our knowledge, this is the first report on ulcerative colitis associated with Hodgkin s disease and nephrotic syndrome.

Adult↗
PubMed2002

[Neurologic sequelae of bone changes in multiple myeloma and its therapy].

Multiple myeloma (MM) is a plasma cell malignancy characterized by infiltration of bone marrow, bone destruction, infiltration of soft tissues with plasma cells, and suppression of normal hematopoiesis. The production of monoclonal immunoglobulins with or without light chains is a major feature of the disease. Full spectrum of plasma cell dyscrasias include monoclonal gammapathy of undetermined significance, smouldering myeloma, indolent multiple myeloma, and fully developed, symptomatic multiple myeloma. The usual presenting features of MM include bone pain, weakness, fatigue, fever and infection. Neurologic symptoms are less common but one must not forget that MM may present with a neurologic disease. Careful neurologic history and examination are mandatory in patients with MM. Neurologic symptoms may be a direct manifestation of MM or may be due to the immune effect of monoclonal proteins directed against different neural structures. Finally, metabolic consequences (uremia, hypercalcemia, hyperviscosity) of MM may produce a broad spectrum of different neurologic symptoms including headache, blurring of vision, drowsiness, precoma, coma, vertigo, ataxia, hemiparesis and epileptiform seizures. The most common location of bone changes in MM is the thoracic spine, where it causes osteolytic changes with consequent compressive fractures. The most disastrous sequel is paraplegia. Multiple vertebral involvement with the evidence of osteolytic changes in other bones is usual, but solitary vertebral myeloma may occur. Myeloma usually involves the bone of the vertebral body and then spreads into the extradural space. However, patients with solitary extradural myeloma have been reported. Skull myeloma is frequently asymptomatic. It may grow externally or, rarely, there is intracranial expansion. Involvement of the cranial nerves is not rare, with II, V, VI, VII and VIII cranial nerves being most often affected. Isolated intracerebral plasmacytomas are extremely rare. Diagnostic approach includes plain X-rays of the skeleton, which was found to be the method of choice for demonstration of osteolytic changes, whereas magnetic resonance with gadolinium enhancement most reliably displays the degree of vertebral involvement and demonstrates any associated soft tissue mass. Current treatment of osteolytic changes in multiple myeloma include chemotherapy, radiotherapy in combination with dexamethasone, monthly infusions of bisphosphonates, surgical decompression, and kyphoplasty. Therapeutic approach is dictated by the presenting symptoms. In case of pain as the predominant symptom, treatment with chemotherapy and radiotherapy may be appropriate. Compressive symptoms are relieved with dexamethasone followed by radiotherapy and chemotherapy. Surgical decompression is used in patients with vertebral collapse and vertebral instability. Kyphoplasty is a new method used in the treatment of osteolytic changes of vertebral bodies. A viscous cement is injected into the cavity by a balloon-like inflatable bone tampon. It has been successfully employed to improve the quality of life, to reduce pain, and to increase overall functioning in patients with vertebral compression fractures by restoring most of the original height of the vertebral body. Bisphosphonates reduce pain associated with osteolytic changes in multiple myeloma, but also significantly reduce skeletal events (pathologic fracture, spinal cord compression, surgery or irradiation of bone) via unknown mechanism. It seems that bisphosphonates, by inhibiting bone resorption, alter the microenvironment in which the MM cells grow.

Central Nervous System Diseases↗
PubMedDate not supplied

[Cardiovascular diseases after kidney transplantation].

Cardiovascular complications represent the leading cause of mortality in renal transplant recipients, with ischemic heart disease accounting for more than 50% of deaths. Besides the well known risk factors that affect the general population, risk for development of atherosclerosis in renal transplant patients is further increased by previous uremia and dialysis, as well as by the use of immunosuppressive agents. Diabetes mellitus, arterial hypertension, dyslipidemia, smoking, hyperhomocysteinemia, hyperuricemia, coagulation abnormalities, increased expression of cell adhesion molecules, persistent inflammation, frequent infections and obesity all increase the risk for development of atherosclerosis in transplanted patients. There is a growing body of evidence suggesting that the risk of cardiovascular disease falls significantly with smoking cessation, reduction of alcohol consumption, reduction of excessive weight, and appropriate and aggressive control of blood pressure and dyslipidemia. Patients should be instructed, and every effort should be invested to increase their compliance with the modified lifestyle and drug adherence. Novel immunosuppressive regimens tend to decrease the risk of atherosclerosis by being individualized according to the characteristics of the particular patient.

Atherosclerosis↗
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↗