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Biomedical subjects

Massimo Franchini

Publications and source records attributed to Massimo Franchini.

At least 19 recordsLinked to original sources
PubMed2007

Therapy for children with henoch-schonlein purpura nephritis: a systematic review.

Although severe kidney involvement in children with Henoch-Shonlein purpura (HSP) is rarer than that in adults, morbidity should not be underevaluated and follow-up is mandatory. Some drugs are introduced as well-defined treatment options, others can be promising therapeutic alternatives. Therapy of HSP nephritis in children can range from simply steroids to combined immunosuppressant treatments. The prophylactic treatment for renal complication of patients with HSP has been sometimes suggested, but with conflicting results and ultimately not clearly proven. The treatment of overt HSP nephritis includes steroids and other immunosuppressant drugs. Methylprednisolone pulse therapy and prednisone per os are tested drugs. These steroids could be used in combination with other immunosuppressant drugs, such as cyclosporin A and cyclophosphamide. Unfortunately, of these two drugs, only cyclophosphamide is demonstrated as effective in a recent randomized controlled trial. However, since there are insufficient data and unstructured study designs, ACE-I, azathioprine, mycophenolate mofetil, and urokinase need to be more tested in childhood HSP nephritis. In addition to drugs, other techniques are used to treat the severe form of nephritis. Of these, in a multicenter study, plasmapheresis demonstrated efficacy in delaying the progression of kidney disease. However, no convincing studies have been made to date concerning either intravenous immunoglobulin, factor XIII administration, antioxidant vitamin E, and fish oil to treat HSP nephritis.

Child↗
PubMed2007

Acquired von Willebrand syndrome: an update.

Acquired von Willebrand syndrome (aVWS) is a rare bleeding disorder with laboratory findings similar to those for congenital von Willebrand disease (VWD). However, unlike congenital VWD, it arises in individuals with no personal or family history of bleeding. aVWS occurs in association with a variety of underlying disorders, most frequently in lymphoproliferative disorders, myeloproliferative disorders, and cardiovascular diseases. Through an analysis of the more recent literature data, the pathophysiology and the clinical, laboratory, and therapeutic aspects of this syndrome are concisely reported in this review.

ADAM Proteins↗
PubMed2006

Recent acquisitions in acquired and congenital von Willebrand disorders.

von Willebrand factor is a multimeric plasma protein that mediates platelet adhesion as well as platelet aggregation at sites of vascular injury and, moreover, acts as a carrier of factor VIII. Its deficiency is associated with a bleeding tendency. The progresses in the pathophysiology, diagnosis and management of inherited von Willebrand disease and acquired von Willebrand syndrome will be discussed in this review.

Biomarkers↗
PubMed2006

Comparison of platelet function between sedentary individuals and competitive athletes at rest.

BACKGROUND: There are controversial evidences on the effect of different types and workloads of physical exercise on primary hemostasis. In particular, little is known on the chronic influence of a strenuous and regular aerobic training regimen on platelet function. METHODS: The aim of this investigation was to compare platelet function between sedentary controls and trained athletes at rest and to evaluate whether a greater amount of exercise performed in professional cyclists may contribute to increased platelet chronic responsiveness compared to both elite cyclists and sedentary individuals. Platelet's ability to adhere and aggregate was assayed following a 12-24 h resting period in 49 active professional male road cyclists, 40 elite male cyclists and 43 matched sedentary healthy male volunteers, by the platelet function analyzer 100 (PFA-100). RESULTS AND DISCUSSION: Mean values of the collagen-epinephrine test did not differ between controls and athletes (sedentary controls: 111 +/- 33 s; elite athletes: 113 +/- 26 s, p = 0.93; professional athletes: 120 +/- 33 s; p = 0.33), whereas mean values of the collagen-ADP test displayed a slightly but significant trend towards decreased values when comparing sedentary controls (83 +/- 21 s) with either elite (77 +/- 11 s, p < 0.01) or professional (75 +/- 16 s, p < 0.01) athletes. CONCLUSION: The trend towards slightly lower collagen-ADP values are suggestive for a modest but significant chronic activation of primary hemostasis, highlighting the need to set appropriate reference ranges for the PFA-100 when evaluating primary hemostasis in physically active subjects.

Journal Article↗
PubMed2006

Hemostasis and aging.

Many changes in the vasculature, hemostasis and endothelium, including alterations of platelets, coagulation and fibrinolytic factors, occur during aging. While the increasing hypercoagulability observed with aging may account for the higher incidence of thrombotic cardiovascular disorders in the elderly, the lack of genetic protective factors against thrombosis in healthy centenarians suggests that little is yet known about the age-associated changes of hemostasis. The complex inter-relationships between inherited and acquired factors influencing the hemostatic system during aging are discussed in this review.

Aged↗
PubMed2006

Blood doping by cobalt. Should we measure cobalt in athletes?

BACKGROUND: Blood doping is commonplace in competitive athletes who seek to enhance their aerobic performances through illicit techniques. PRESENTATION OF THE HYPOTHESIS: Cobalt, a naturally-occurring element with properties similar to those of iron and nickel, induces a marked and stable polycythemic response through a more efficient transcription of the erythropoietin gene. TESTING THE HYPOTHESIS: Although little information is available so far on cobalt metabolism, reference value ranges or supplementation in athletes, there is emerging evidence that cobalt is used as a supplement and increased serum concentrations are occasionally observed in athletes. Therefore, given the athlete's connatural inclination to experiment with innovative, unfair and potentially unhealthy doping techniques, cobalt administration might soon become the most suited complement or surrogate for erythropoiesis-stimulating substances. Nevertheless, cobalt administration is not free from unsafe consequences, which involve toxic effects on heart, liver, kidney, thyroid and cancer promotion. IMPLICATIONS OF THE HYPOTHESIS: Cobalt is easily purchasable, inexpensive and not currently comprehended within the World Anti-Doping Agency prohibited list. Moreover, available techniques for measuring whole blood, serum, plasma or urinary cobalt involve analytic approaches which are currently not practical for antidoping laboratories. Thus more research on cobalt metabolism in athletes is compelling, along with implementation of effective strategies to unmask this potentially deleterious doping practice.

Journal Article↗
PubMed2006

Mesenchymal stem cells for bone, cartilage, tendon and skeletal muscle repair.

Literature data concerning the biology and differentiation potential of mesenchymal stem cells (MSCs) have become huge in less than 10 years, although some of these data still remain contradictory. MSCs seem to be a very promising tool for cell therapy because of their peculiar characteristics, which mimic partially those of embryonic stem cells, but with some advantages in terms of availability, expandability, transplantability, and ethical implications. We discuss here the potential use of MSCs in degenerative or inflammatory diseases involving bone, cartilage, tendon and muscle tissues, on the basis of the experimental evidence.

Bone Diseases↗
PubMed2006

Von Willebrand factor and thrombosis.

There is increasing evidence that von Willebrand factor (VWF), an adhesive multimeric protein that has an important function in primary hemostasis and as a carrier of factor VIII, has a pivotal role in thrombogenesis. In fact, while the presence in plasma of unusually large VWF multimers due to a congenital or acquired deficiency of a VWF-cleaving metalloprotease has been implicated in the pathogenesis of thrombotic thrombocytopenic purpura (TTP), high plasma levels of VWF have been associated with a slightly increased risk of arterial thrombosis. With regard to the association between VWF and venous thrombosis, clear conclusions cannot yet be drawn from the conflicting published data. Patients with von Willebrand disease, an inherited hemorrhagic disorder, may also paradoxically experience thrombotic events as a result of interactions among multiple prothrombotic risk factors. After a description of the structure and physiology of VWF, all these aspects are discussed in the present review.

Arterial Occlusive Diseases↗
PubMed2006

Recent acquisitions in the pathophysiology, diagnosis and treatment of disseminated intravascular coagulation.

Disseminated intravascular coagulation (DIC) is a disorder characterized by both acute generalized, widespread activation of coagulation, which results in thrombotic complications due to the intravascular formation of fibrin, and diffuse hemorrhages, due to the consumption of platelets and coagulation factors. Systemic activation of coagulation may occur in a variety of disorders, including sepsis, severe infections, malignancies, obstetric or vascular disorders, and severe toxic or immunological reactions. In this review, we briefly report the present knowledge about the pathophysiology and diagnosis of DIC. Particular attention is also given to the current standard and experimental therapies of overt DIC.

Journal Article↗
PubMed2006

Hereditary iron overload: update on pathophysiology, diagnosis, and treatment.

Hereditary hemochromatosis, a very common genetic defect in the Caucasian population, is characterized by progressive tissue iron overload which leads to irreversible organ damage if it is not treated timely. The elucidation of the molecular pathways of iron transport through cells and its control has led to the understanding of various genetic iron-loading conditions. Four types of inherited iron overload have been recognized: type 1, the most common form with an autosomal recessive inheritance, is associated with mutations in the HFE gene on chromosome 6; type 2 (juvenile hemochromatosis) is an autosomal recessive disorder with causative mutations identified in the HJV gene (subtype A) on chromosome 1 and the HAMP gene (subtype B) on chromosome 19; type 3 has also an autosomal recessive inheritance with mutations in the TfR2 gene on chromosome 3; type 4 is an autosomal dominant condition with heterozygous mutations in the ferroportin 1 gene on chromosome 2. In this review, the genetics, pathophysiology, diagnosis, clinical features, and management of these different types of hereditary hemochromatosis are briefly discussed.

Antimicrobial Cationic Peptides↗
PubMed2006

High prevalence of inherited prothrombotic risk factors in 134 consecutive patients with von Willebrand disease.

We screened 134 consecutive patients with von Willebrand disease (VWD) (106 type 1, 26 type 2, and 2 type 3 VWD) for the most important inherited prothrombotic risk factors. One hundred eight patients (80.6%) were positive for at least one of the prothrombotic risk factors screened for. A high prevalence of prothrombin G20210A (10.5%) and factor V Leiden (11.9%) mutations was found with allele frequencies of 5.2 and 6%, respectively. Three carriers of multiple prothrombotic gene mutations experienced a thrombotic event. Our study suggests that the recent evidence of an association between inherited thrombotic and hemorrhagic disorders also holds true in VWD patients.

Adult↗
PubMed2006

Treatment of hepatitis C in hemophiliacs.

Hepatitis C virus (HCV) infection is a major cause of morbidity and mortality in hemophiliacs who received nonvirucidally treated large-pool clotting factor concentrates before 1986. In fact, although many hemophiliacs infected with HCV have a slow progression of liver disease, in a minority of them hepatitis evolves toward end-stage liver disease and hepatocarcinoma. Moreover, a significant percentage of HCV-infected hemophiliacs were also coinfected with human immunodeficiency virus (HIV), which can accelerate the progression of liver disease. Thus, the aim of anti-HCV therapy is to interrupt the chronic infection in order to prevent the progression of hepatitis to cirrhosis, liver decompensation, cancer and, ultimately, death. In this review we present the literature data on anti-HCV treatment in hemophiliacs. 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 plus ribavirin in nonhemophiliacs, ongoing trials are evaluating this promising therapy in HCV-chronically infected hemophilic patients; preliminary results show a sustained response rate similar to that in patients without coagulopathy. Finally, based on the encouraging results in coinfected nonhemophiliacs, anti-HCV treatment should also be considered for those HIV-positive hemophiliacs in whom anti-retroviral treatment has stabilized the HIV infection.

Drug Administration Schedule↗
PubMed2006

Postpartum acquired factor VIII inhibitors.

The development of factor VIII autoantibodies is a rare but severe complication of pregnancy. Although the natural history of postpartum acquired hemophilia A is usually benign, with a high percentage of spontaneous remissions and a low mortality, its quick recognition is important to control bleeding episodes. Based on an analysis of the literature, this review presents the current knowledge on the pathogenesis, diagnosis, epidemiology, natural history, clinical manifestations, and therapeutic management of postpartum acquired hemophilia A.

Autoantibodies↗