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Biomedical subjects

Massimo Franchini

Publications and source records attributed to Massimo Franchini.

At least 91 records · Page 5Linked to original sources

Iron-chelation therapy: an update.

Chronically transfused patients develop iron overload that leads to organ damage and ultimately to death. The introduction of the iron-chelating agent, desferrioxamine mesylate, dramatically improved the life expectancy of these patients. However, the very demanding nature of this treatment (subcutaneous continuous infusion via a battery-operated portable pump) has been the motivation for attempts to develop alternative forms of treatment that would facilitate the patients' compliance. In this review, we describe the most important advances in iron-chelating therapy. In particular, we analyze a new method of administering desferrioxamine mesylate (twice daily subcutaneous bolus injections) and a novel, orally active iron chelator (ICL670A). We also present a meta-analysis of the largest trials on the oral iron chelator deferiprone and the results of combined therapy (deferiprone and desferrioxamine).

Clinical Trials as Topic↗

Iron overload and hematologic malignancies.

Although iron is essential for cell replication and survival, an increase of body iron stores has been implicated in the development of cancer. However, while the association between iron overload and hepatocellular carcinoma is well documented, the relationship with nonhepatocellular malignancies remains ill-defined. In this review, we briefly report the present knowledge regarding the association between iron overload and hematologic malignancies.

Adult↗

Clinical and laboratory study of an episode of cefotetan-induced severe hemolytic anemia.

BACKGROUND: Cephalosporins are frequently associated with positive direct antiglobulin tests (DAT) and may rarely cause immune hemolytic anemia (IHA). We describe a patient who developed hemolytic anemia while she was receiving intravenous cefotetan. STUDY DESIGN AND METHODS: Immunohematologic studies of drug-dependent antibodies were performed by using cefotetan-treated red blood cells (RBCs) and untreated RBCs in the presence of cefotetan. RESULTS: The patient's serum contained antibodies that reacted with both drug-coated RBCs (adsorption mechanism) and with uncoated RBCs when cefotetan was added to the serum (immune complex mechanism). The prompt recognition of the problem and discontinuation of the drug prevented the onset of renal failure and rapidly resolved the hemolytic reaction. CONCLUSION: Our report underlines the importance of close laboratory and immunohematologic monitoring of patients treated with cephalosporins in order to recognize swiftly any hemolytic reaction due to these antibiotics thus reducing the chance of serious sequelae.

Anemia, Hemolytic↗

Update on the treatment of disseminated intravascular coagulation.

Disseminated intravascular coagulation (DIC) is characterized by an acute generalized, widespread activation of coagulation, which results in thrombotic complications, due to the intravascular formation of fibrin, as well as diffuse hemorrhages, due to the consumption of platelets and coagulation factors. In this review, we briefly report the present knowledge about the treatment of DIC. We focus on the current standard treatment of overt DIC in clinical practice. Moreover, particular attention is made to novel therapeutic strategies, who reflect the important progresses in the understanding of the pathogenesis of this syndrome in the last few years.

Anticoagulants↗

Helicobacter pylori infection and immune thrombocytopenic purpura: an update.

Data are accumulating on the association between Helicobacter pylori infection and idiopathic thrombocytopenic purpura (ITP) and the significant increase in platelet count after bacterial eradication. The aim of this review was to consider the studies so far published on H. pylori infection and ITP in order to evaluate a possible correlation between these two conditions. A review of the literature showed that 278 out of the 482 ITP patients investigated (58%) were positive for H. pylori infection and that the bacterium was eradicated in 88% of cases. Eradication therapy was accompanied by a complete or partial platelet response in approximately half the cases. Overall, these data show that H. pylori eradication in patients with ITP is effective in increasing platelet count. However, because the studies so far published are few, are sometimes controversial and involve small series of patients, further studies on larger numbers of patients with longer follow-up are needed to confirm these preliminary findings.

Helicobacter Infections↗

Efficacy and safety of phlebotomy to reduce transfusional iron overload in adult, long-term survivors of acute leukemia.

BACKGROUND: Transfusional iron overload is a frequent finding in long-term survivors of acute leukemia (AL). Only a few studies have reported the results of iron depletion therapy in this category of patients. STUDY DESIGN AND METHODS: Between January 1996 and July 2003, 26 consecutive adult patients who achieved complete remission of AL and developed transfusional iron overload underwent a weekly phlebotomy program at our transfusion center. Serum ferritin levels and transferrin saturation were monitored during the iron depletion therapy and the follow-up period. These AL patients were also checked for the presence of 12 hereditary hemochromatosis (HH) gene mutations. RESULTS: After a mean follow-up of 57.8 months, therapeutic phlebotomy (mean number of units collected, 36.6) was effective in reducing mean ferritin concentration and transferrin saturation from 1726.9 to 93.0 mg per L and from 54.7 to 23.3 percent, respectively. The presence of a HH gene mutation did not influence initial iron status or response to treatment. The phlebotomy program was well tolerated and no adverse events were recorded during or after collection. In three cases the time between phlebotomies was increased because of patient's poor compliance or low Hb levels. CONCLUSION: Our study shows that phlebotomies are a safe and effective method for reducing iron over-load in multiply transfused long-term AL survivors with secondary hemochromatosis.

Adult↗

Thrombotic complications in patients with hereditary bleeding disorders.

Thromboses in patients with hereditary bleeding disorders are uncommon. However, in some cases, the co-existence of prothrombotic risk factors may increase the likelihood of developing thrombotic complications in such patients. This review summarizes the cases of thrombosis reported in the literature and analyzes the most important risk factors for thrombosis in patients with a congenital bleeding tendency. In particular we focus on central venous catheter (CVC)-associated thrombosis, on the thrombotic complications of coagulation factor concentrate therapy and on the presence of prothrombotic gene mutations. Data were identified by searches of the published literature, including PubMed, references from reviews and abstracts from the most important meetings on this topic. In conclusion, there is increasing evidence that thrombotic complications in patients with hereditary bleeding disorders have a multifactorial pathogenesis, depending on exogenous (coagulation factor replacement therapy, CVC, HIV infection) and/or endogenous (prothrombotic gene mutations) risk factors.

Blood Coagulation Disorders↗

Hepatitis C in haemophiliacs.

Virtually all haemophiliacs who received non-virucidally treated, large-pool clotting factor concentrates before 1986 became infected with hepatitis C virus (HCV). Although approximately one-tenth of HCV-infected people have been shown to clear the infection naturally, in the remaining cases the infection slowly progresses. Unfortunately, a significant percentage of HCV-infected hemophilic patients were also co-infected with human immunodeficiency virus (HIV), which can accelerate the progression to cirrhosis and liver failure. As regards treatment, combination therapy with interferon (IFN) and ribavirin has improved the poor results obtained with IFN monotherapy and has become the standard treatment of chronic hepatitis C. Given the positive results obtained with pegylated interferon in non-haemophiliacs, ongoing trials are evaluating this promising therapy in HCV-chronically infected haemophilic patients. Finally, anti-HCV treatment should also be considered for those haemophiliacs co-infected with HIV in whom anti-retroviral treatment has stabilized the HIV infection.

Female↗

Comparison of serum creatinine, uric acid, albumin and glucose in male professional endurance athletes compared with healthy controls.

Owing to considerable physical, endocrinological and metabolic adaptations, the analysis of biochemical data in elite and top-class athletes requires caution. With the aim to identify metabolic and biochemical adaptations to particular lifestyle conditions, such as regular and strenuous physical exercise, we measured the concentration of serum albumin, creatinine, uric acid and glucose in 80 male professional cyclists, 37 male members of the Italian national cross-country ski team and 60 male healthy sedentary controls at rest. At variance with earlier investigations, endurance athletes showed significantly decreased concentrations of serum creatinine (controls: 83.1+/-11.0 micromol/l; skiers: 78.0+/-8.4 micromol/l; p<0.05; cyclists: 73.8+/-10.4 micromol/l; p<0.01), uric acid (controls: 362+/-69 micromol/l; skiers: 331 +/-70 micromol/l; p<0.05; cyclists: 312+/-61 micromol/l; p<0.01) and glucose (controls: 5.35+/-0.54 mmol/l; skiers: 4.94+/-0.41 mmol/l; p<0.01; cyclists: 4.94+/-0.42 mmol/l; p<0.01). The concentration of serum albumin was also decreased in athletes, but the difference did not reach statistical significance (controls: 4.76+/-0.26 g/l; skiers: 4.71+/-0.22 g/l; p=0.384; cyclists: 4.68+/-0.22 g/l; p=0.393). Results of the present investigation demonstrate that values of laboratory testing lying outside conventional reference limits calculated on sedentary populations might express physiological adaptations to regular and demanding physical aerobic activity, emphasizing the need for the estimation of reliable reference limits in elite and professional athletes, to avoid equivocal interpretation of results within clinical and anti-doping contests.

Adaptation, Physiological↗

Primary cutaneous plasmacytoma after rejection of a transplanted kidney: case report and review of the literature.

Immunosuppressed organ allograft recipients are at risk of developing lymphomas and lymphoproliferative disorders as a consequence of immunosuppressive therapy and long-term antigenic stimulation from both the graft and possible viral infections. No more than 4% of the malignant tumors detected in organ recipients are plasmacytomas. Primary cutaneous plasmacytoma is a rare type of cutaneous B-cell lymphoma arising primarily in the skin. It is derived from clonally expanded plasma cells with various degrees of maturation and atypia. We report the occurrence of a solitary cutaneous plasmacytoma in a 56-year-old male patient undergoing hemodialysis after rejection of a grafted kidney. The diagnosis was made a few months after the kidney had been surgically removed. A thorough examination showed no evidence of systemic disease. Skin lesions were successfully treated with local radiotherapy. After 2 years of follow-up there were no local or systemic recurrences.

Graft Rejection↗

[Hepatitis C in hemophiliacs].

Hepatitis C virus (HCV) infection is an important cause of morbidity and mortality in patients with hereditary bleeding disorders treated with non virus inactivated clotting factor concentrates during the 1970s. In this article, we briefly report the actual knowledge about HCV infection in hemophiliacs, by analyzing the prevalence of HCV infection, the genotype distribution, the natural history of the infection and the most important factors involved in the progression of chronic hepatitis into liver cirrhosis, hepatic decompensation and hepatocellular carcinoma. Finally, we describe the main advances in the treatment of HCV infection.

Hemophilia A↗

[Clinical use of erythropoietin].

The introduction of recombinant human erythropoietin (RHuEPO) has dramatically changed the therapeutic approach to the anemia of chronic renal failure. Clinical studies have also demonstrated that RHuEPO is effectiveness in various non-uremic conditions, such as anemia associated with onco-hematological disorders, prematurity, HIV infection and to reduce the exposure to allogeneic blood in surgical patients. In this review, we briefly analyze the main clinical applications of RHuEPO, with particular attention to the potential complications deriving from its use.

Adult↗

[Hemophilic arthropathy].

Hemarthrosis is the most frequent clinical manifestation of severe hemophilia. Recurrent hemarthroses in a joint lead to the development of anatomical changes which result, through an intermediate stage called chronic synovitis, in degenerative arthritis, an extremely disabling disease. In this review, we briefly report the main physio-pathologic, clinic and therapeutic characteristics of the hemophilic arthropathy.

Acute Disease↗