Systemic vasculitis in juvenile dermatomyositis: a fatal case.
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Biomedical subjects
Publications and source records attributed to M Ermini.
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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.
BACKGROUND AND METHODS: The limited and controversial data available regarding endocrine function in adults treated for acute lymphoblastic leukemia (ALL) prompted us to study the influence of chemotherapy on gonadal, adrenal androgen and thyroid function. Forty-eight ALL adults (30 men and 18 women) in first complete remission (CR) were investigated. RESULTS: Severe reproductive function impairment was detected in 10/12 males receiving treatment, while endocrine function was affected in only 1 case. All but one off-therapy male showed normal reproductive function; moreover, endocrine function was impaired in only one male. Eight of 10 females receiving treatment showed hypergonadotropinemia and decreased estrogen levels. All off-therapy women reported regular menses, even though mild hypergonadotropinemia persisted in only 2 cases. Adrenal androgen function was depressed in 12/22 on-therapy patients in both sexes, and in 6/17 off-therapy males. All off-therapy females recovered completely. Dihydroepiandrosterone-sulfate (DHEA-S) serum levels were significantly correlated to the length of time-off therapy (p = 0.003), and inversely correlated to the age at diagnosis (p < 0.0001) in off-therapy males. All patients remained euthyroid, although 4 patients showed subclinical impairment of thyroid function. CONCLUSIONS: Our experience suggests that long-term prospective studies for ALL adults are necessary in order to better define the magnitude and duration of influence the chemotherapy has on endocrine function.
OBJECTIVE: to investigate the effects of treatment with low dose of cyproterone acetate and ethynyl-estradiol on chemical and hormonal parameters in female hyperandrogenism. DESIGN: twenty patients, affected by clinical manifestations of hyperandrogenism, were studied basally and during treatment with 10 mg of cyproterone acetate associated with ethynyl-estradiol (20 m g) in an inversal sequential scheme for six months. Safety parameters were assessed throughout the study. Hirsutism was graded by Ferriman and Gallway score, and hormonal parameters were evaluated basally and after six months of therapy. RESULTS: after six months of therapy all patients showed a significant decrease of clinical and biochemical signs of hyperandrogenism, and normalization of ovarian parameters in seventeen women affected by PCOS. CONCLUSIONS: this treatment is an effective and well tolerated therapy. It is reasonable to consider this treatment as a safe procedure especially in view of a long-term administration.
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.