Efficacy and safety of immunoglobulin retreatment in Kawasaki disease.
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Biomedical subjects
Publications and source records attributed to S Trapani.
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Lung involvement in children affected with Systemic Sclerosis (SSc) has been studied. We used ventilation and perfusion scans and compared the results to those obtained with traditional pulmonary function tests. The vascular pulmonary area was examined with a gama-camera after infusion of 99mTc microspheres, while the alveolo-capillary membrane and the airways were investigated after administration of a radioaerosol, (99mTc DTPA). Pulmonary function tests revealed either alteration of the airways or a considerable involvement of the alveolo-capillary membrane in two patients. On the other hand ventilation scan showed airways alterations and a severe decrease of the alveolocapillary permeability in all patients. Our study indicates that ventilation scans may be useful in the diagnosis of early modifications of the lung function also in paediatric age and may complement the data obtained from traditional function tests.
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Two cases of primary antiphospholipid syndrome are described. A girl presented with myocardial infarction at the age of 6. afterward developed chorea, livedo reticularis, thrombocytopenia and circulating lupus anticoagulant (LAC). A boy, age 7, had an episode of intracranial hypertension and a deep venous thrombosis of a lower left limb, both recurrent in the following years. A high titer of IgG anticardiolipin antibodies (aCI) was detected. These observations suggest that both LAC and aCI tests should be performed in children with thromboembolic phenomena when the criteria for a definite autoimmune disease are lacking.
Steroids are widely used for treating chronic connective tissue diseases in both adults and children. Unfortunately steroid treatment may produce many side effects and growth retardation is one of these. In juvenile chronic arthritis patients (JCA) oral steroids are mainly administered in severe systemic or polyarticular onset or in JCA complicated by chronic uveitis which is unresponsive to mydriatic and steroid eye drops. Intraarticular steroid treatment is used in pauciarticular onset JCA. This paper will review the literature on steroid treatment and growth rate, and report 35 cases of children with JCA treated with steroids.
Juvenile Rheumatoid Arthritis is a heterogeneous disease currently divided into different subtypes based on clinical characteristics. Significant alterations in frequencies of HLA-A, B and DR antigens have been described previously in children with pauciarticular onset Juvenile Rheumatoid Arthritis. We report the results of a study on 42 italian children with pauciarticular onset Juvenile Rheumatoid arthritis that partially confirm the literature data.
Juvenile rheumatoid arthritis (JRA) is the most common rheumatic disease in children. It is one of the more frequent chronic illnesses of childhood and an important cause of disability. Authors review the literature about JRA.
Systemic sclerosis is a multisystem disease of unknown cause, characterized by inflammation, vascular and fibrotic changes with excessive accumulation of connective tissue. The lesions may involve the skin and various internal organs (kidney, lung, gastrointestinal tract and heart). The first symptom is usually Raynaud's phenomenon, followed by skin changes; at the beginning the skin is swollen and oedematous, and then becomes thick, taut, shiny and atrophic. The prognosis of SS depends mainly on the severity of visceral involvement. The treatment of SS consists of drugs that improve the microcirculation and reduce collagen proliferation, such as calcium-antagonists (nicardipine) and D-Penicillamine.
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Over a period of four years 18 children seen in our clinic have been diagnosed as suffering from Yersinia enterocolitica reactive arthritis: this group constitutes one third of the total reactive arthritides seen by us. The diagnosis was based on elevated specific antibody titres and the outcome has always been good with anti-inflammatory therapy, symptoms disappearing within a few weeks without any complications or recurrences. This positive outcome could be due to the absence of the HLA B27 haplotype. Yersinia has been described as the most frequent agent causing reactive arthritis, a common complication in an otherwise benign infection in children. It seems that Yersinia may trigger in predisposed hosts an immunological reaction leading to arthritis.
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OBJECTIVE: Multiple humoral and cellular abnormalities in Kawasaki disease (KD) have already been described. In this study an analysis of immunological findings in a cohort of 34 Italian children affected with KD is reported, and the potential clinical significance of such alterations in predicting the development of coronary aneurysm and the prognosis of the disease is evaluated. METHODS: Levels of circulating immune complexes (CIC), antinuclear antibodies (ANA), anticardiolipin (aCL), antineutrophil cytoplasmic antibodies (ANCA), and anti-endothelial cells (AECA) and the T cell profile were determined in both the acute and the convalescent phases, and were compared to febrile, sex- and age-matched children. RESULTS: CIC were present in 66% of the patients, 18 of whom were in the acute phase and 13 in the convalescent phase. In the control group CIC were detected in 47% of the children. ANA were negative in both the KD and in the febrile group. ANCA were present in 8%, AECA in 26%, and aCL in 30% of KD patients (IgG aCL antibodies were found in 14 patients, IgM aCL in, 1 and 1 had both). Among the controls, aCL antibodies were found in 5 patients (22%); in particular 1 (4.4%) had IgG and 4 (17.4%) had IgM aCL. An altered T cell profile, with an inverted CD4/CD8 ratio, was found in all KD children. All of the immune alterations showed a lower incidence in the convalescent than in the acute phase. No significant relationship between any of these immune findings and cardiac involvement or any other clinical manifestations was found. CONCLUSION: Our data confirms the previously reported immunological anomalies in KD both in the acute and the convalescent phases, with a decreased incidence of such alterations in the convalescent phase. No prognostic significance for the occurrence of aneurysm could be demonstrated.