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BACKGROUND: Meningoencephalitis due to enteroviruses is particularly serious when occurring in patients with agammaglobulinaemia. This disease is associated with a high mortality and a significant risk for neurological sequelae in such circumstances. We report here a new case treated with intraventricular immunoglobulin, whose evolution was favourable. CASE REPORT: A three-year-old boy with agammaglobulinaemia, while he was treated with gammaglobulin with an IgG residual concentration of 10 g/l, presented neurological symptoms related to Echovirus 27 meningo encephalitis. Under treatment with intraventricular gammaglobulin by means of an Ommaya reservoir, the patient recovered. CONCLUSION: Favourable evolution is rare in meningo encephalitis in agammaglobulinaemic patients. Prognosis depends on an early diagnosis and on the extent of dissemination of the infection. Intraventricular gammaglobulin administration may contribute to a favourable outcome.  相似文献   
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Intravenous immunoglobulin (Ig IV) has been used for many years in the treatment of primary antibody deficiencies. We performed a retrospective study of the clinical features and outcome of agammaglobulinemia children who received prolonged Ig IV infusions. PATIENTS AND METHODS: Ten children, 9 male et 1 female, with agammaglobulinemia diagnosis were studied for the clinical manifestations before and during the Ig IV replacement therapy. Serum Ig levels were quantified by nephelometry. Circulating B ant T cells were counted by immunofluorescence labeling by monoclonal antibodies. T-cell functions were assessed by using mitogen and antigen -induced T-cell proliferation assays in vitro. Patients clinical status was evaluated respectively, before initiation and at every moment (when patients had an infection) of the replacement therapy. RESULTS: Ig IV therapy was performed for 866 cumulated months, median 108 months. The median Ig IV doses administered to the 10 patients was 500 mg/kg/month. Residual serum IgG mean level was 3,9 g/L. All patients had 99 bacterial infections/year before Ig IV, mainly respiratory tract infections (48,5%), and 4 patients had bronchiectasis before Ig replacement therapy. The number of infection/year fall to 25 during IgIV replacement, and the infection/patient/year rate decreases significantly. One patient developed an Echovirus 27 meningoencephalitis during this treatment. CONCLUSION: Ig IV therapy with residual IgG mean level of 3,9 g/l reduced significantly the rate of bacterial infections. The use of specific antibiotherapy and respiratory kinesitherapy led to a lower rate of respiratory tract infections, and the stabilisation of the bronchiectasis. However this intravenous replacement therapy does not protect against viral meningoencephalitis.  相似文献   
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Genetic diversity was characterized in 20 isolates of Grapevine fanleaf virus (GFLV) recovered from naturally infected grapevine plants (Vitis vinifera) in the North of Tunisia. Viral RNAs were isolated by oligoprobe capture, and a 605 bp fragment containing a part of the viral coat protein gene was amplified by RT-PCR. Sequence variation among isolates was characterized by restriction fragment length polymorphism (RFLP) analysis and confirmed by sequencing. The GFLV infections are found as a complex mixture of closely related genomes. In further studies, RFLP analyses of virus isolates using AluI showed that GFLV populations in Tunisian vineyards consist of two restrictotypes corresponding to distinct sub-populations Sp1 and Sp2. The relative field distribution of these sub-populations showed that Sp2 was more abundant. Individual genomes were recovered by cloning the RT-PCR products. The sequences were found to vary from each other by as much as 11%. Cloning from mixed infections showed that Sp2 are also predominant.  相似文献   
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Celiac disease (CD) is an autoimmune systemic disease characterized by not only gastrointestinal but also extraintestinal manifestations. The aim of our study was to do a serological screening for CD, by IgA endomysial antibodies (EmA), in patients with unexplained articular manifestations. Two hundred and eleven patients suffering from arthritis or arthralgia without evident cause were studied. EmA were determined by indirect immunofluorescence on human umbilical cord. Two thousand and five hundred blood donors served as control group. Out of 211 patients, 5 had EmA (2.37 %). The frequency of EmA in our patients was significantly higher than in the control group (2.37 vs. 0.28 %, p < 0.01). All patients with positive EmA were female. EmA were significantly more frequent in female patients than in female healthy subjects (3 vs. 0.4 %, p < 0.01). Medical records revealed: diarrhea (one patient), short size (one patient), anemia (three patients), weight loss (two patients) spontaneous abortion (three patients), secondary amenorrhea (one patient), early menopause (one patient) and early baby death (one patient). Biochemical analysis showed decreased level of calcium (one patient), vitamin D (one patient) and cholesterol (one patient). Unexplained liver cytolysis was observed in two patients. Radiological examination showed demineralization of two hands in one patient. Bone osteodensitometry done in one patient out of five revealed lumbar osteopenia. The articular manifestations of the five patients did not respond to corticosteroid treatment. CD must be considered among the differential diagnosis in a patient with arthritis or arthralgia.  相似文献   
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OBJECTIVE: Thoracic impedance cardiography (TIC) is a noninvasive method which has proved to be useful in monitoring the haemodynamic status of the patients. In this study, we evaluated the TIC findings in patients with pericardial effusion and cardiac tamponade. METHODS AND RESULTS: The study consisted of patients with pericardial effusion with (group A) or without (group B) cardiac tamponade (CT). The stroke volume, cardiac output and ejection fraction was measured by both echocardiography and TIC. The measurements were done at baseline in both groups and following pericardiocentesis in group A. The variables were compared by linear regression analysis, paired sample's t test and chi-square test. The study included 32 patients. Group A consisted of 16 patients and group B of 14 patients. Two patients were excluded from comparisons because of insufficient quality of the echocardiographic examination. There were no significant differences between group A and B with regard to demographic features. Both echocardiographic and TIC measurements at baseline revealed decreased cardiac output, EDV and SV in group A and EF was not different. Linear regression analysis revealed that echocardiography and TIC were in significant correlation with regard to cardiac output, enddiastolic volume, stroke volume (p < 0.01) but not ejection fraction (p = 0.8910). The correlation was also present after pericardiocentesis. CONCLUSIONS: TIC can be safely used in patients with pericardial effusion. It provides suggestive data for the diagnosis of CT and can be used as a means of monitoring the results of the pericardiocentesis.  相似文献   
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Background:Polycystic ovary syndrome (PCOS) is the most common endocrine-metabolic disease in women in reproductive age, and occurs in one of 10 women. The disease includes menstrual irregularity and excess of male hormones and is the most common cause of female infertility. Dyspnea is a frequent symptom and is often thought to be due to obesity, and whether it is due to cardiac dysfunction is unknown.Objective:To evaluate right ventricle-pulmonary artery (RV-PA) coupling and pulmonary arterial stiffness in patients with PCOS.Methods:44 PCOS patients and 60 controls were included; venous blood samples were taken for laboratory tests and 2-D, m-mode and tissue doppler transthoracic echocardiography were performed for all the participants. P<0,05 was considered as statistically significant.Results:When compared to the control group, PCOS patients had higher pulmonary artery stiffness values (p=0,001), which were positively correlated with HOMA-IR (r=0,545 and p<0,001). RV-PA coupling was also impaired in 34% of the study patients.Conclusion:Pulmonary artery stiffness is increased and RV-PA coupling is impaired in patients with PCOS. (Arq Bras Cardiol. 2021; 116(4):806-811)Palavras-chave: Diseases of the Endocrine System, Arterial Stiffness, Female infertility, Obesity, Dyspnea, Pulmonary hypertension  相似文献   
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BACKGROUND: Catheter ablation has significantly transformed the clinical management of atrial fibrillation (AF). The safety and efficacy of this procedure are not well understood in patients with pacemakers and defibrillators. OBJECTIVES: The purpose of this study was to study the impact of radiofrequency catheter ablation of AF in patients with pacemakers and implantable cardiac defibrillators. METHODS: We studied 86 patients with pacemakers and defibrillators (group I) and a similar number of age- and gender-matched controls (group II) who underwent AF ablation between 1999 and 2004. Clinical and procedural variables were compared between the two groups. In group I, various generator and lead parameters were compared before and after the procedure. Resurgence of clinical AF after 2 months was considered recurrence. RESULTS: Both groups were similar with regard to age, gender, body mass index, and type of AF. Group I had a higher incidence of diabetes (17% vs 6%, P = .03), coronary artery disease (25% vs 13%, P = .05), less prolonged AF (31 +/- 21 vs 45 +/- 30 months, P <.001), lower left ventricular ejection fraction (49 +/- 13% vs 52 +/- 9%, P = .03), and left ventricular end-diastolic dimensions (4.97 +/- 0.81 vs 4.72 +/- 0.67, P = .03). No changes in the sensing and pacing thresholds, impedance of atrial and ventricular leads, or defibrillator coil impedance after AF ablation were observed in group I. Atrial lead dislodgment was seen in two patients. Transient abnormal but "expected" pulse generator behavior was seen in 25% of patients without permanent malfunction. Stroke (1% vs 1%, P = 1.000), pulmonary vein stenosis (2% vs 1%, P = .77), and AF recurrence rates at 12 months were similar between groups I and II, respectively (19% vs 21%, P = .73). CONCLUSION: AF ablation is safe and efficacious in patients with pacemakers and defibrillators.  相似文献   
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OBJECTIVES: In patients with atrial flutter (AFL) and postoperative right atrial incisional scars, we sought to assess if the use of additional ablative lesions that targeted all potential re-entrant circuits, regardless of the presenting type of flutter, would prevent long-term recurrence. BACKGROUND: Patients with AFL and incisional scars have a complex atrial substrate that may promote multiple mechanisms of intra-atrial re-entry. METHODS: Twenty-nine patients with single right atrial incisional scars undergoing ablation for scar-dependent (n = 15) and cavotricuspid isthmus (CTI)-dependent (n = 14) flutter were studied. RESULTS: In the scar-dependent group, 9 of 15 (60%) patients had inducible or spontaneous CTI-dependent flutter immediately after ablation. In the group with CTI flutter, 7 of 14 (50%) patients had scar-related flutter immediately after ablation. If a second type of flutter was found during the initial ablation, a second ablation was performed either along the isthmus (scar-dependent group) or from the scar to another anatomic boundary (isthmus-dependent group). Patients were followed for 24 +/- 5 months and 18 +/- 6 months in the scar- and CTI-dependent groups, respectively. In the scar-dependent group, five of six (83%) who underwent only a single flutter line had recurrence at 3 +/- 1 months. In the isthmus-dependent group, three of seven (42%) patients who had only one flutter line performed had recurrence at 5 +/- 3 months. There was no flutter recurrence in patients who initially received two different flutter lines or in patients who subsequently underwent a second flutter line at follow-up. CONCLUSIONS: In patients with postoperative right atrial incisional scar and flutter, multiple ablation lines that target both scar-related and classic isthmuses appear necessary to prevent long-term recurrence.  相似文献   
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