T-Cell immune activation in children with vertically transmitted hepatitis C virus infection.

Little is known concerning the clinical features, the histological outcome, and the effects on the maturation of immune system of children with vertically-transmitted hepatitis C virus (HCV) infection. Specifically, no data are available on the peripheral distribution of T-cell subsets. The frequency of naive and memory cells, activated T cells, and cytokine-producing T cells was analyzed in nine HCV-infected children born to HCV-positive mothers. In HCV-infected children, the distribution of naive and memory cells was not significantly altered in the CD4 subset whereas within the CD8 subset, an increase of memory and a decrease of naive cells was observed. The frequency of HLA-DR-positive and Fas-positive T cells was increased in HCV-infected children in both CD4 and CD8 subsets. The distribution of Fas-expressing T cells was directly related to that of HLA-DR cells and inversely related to the frequency of naive T cells. In regard with cytokine production we found increased levels of both CD4 and CD8 interferon-gamma (IFN-gamma)-producing cells whereas no difference in the percentage of interleukin-2 (IL-2)-producing T cells was observed. No meaningful correlation was observed between individual T cell subsets and ALT levels or HCV viral load. In conclusion, our results indicate an increased T-cell activation and a shift to a T(H)1 pattern of cytokine production in children with vertically transmitted HCV infection. The cause of this kind of immune response could reside in the persistent antigenic stimulation by chronic HCV infection.

Lupus carditis: evaluation with technetium-99m MIBI myocardial SPECT and heart rate variability.

The objective of this paper was to investigate the incidence of myocardial perfusion defects in patients with systemic lupus erythematosus (SLE) associated with dysautonomic alterations. Twenty patients without any sign or symptoms of heart disease, selected from a larger population of patients with SLE, underwent technetium-99m sestamibi (Tc-99m MIBI) single photon emission computed tomography (SPECT), at rest and after dipyridamole infusion; they also underwent heart rate variability (HRV) examination by a 24 hour ambulatory electrocardiography, analyzing in the time domain the standard deviation of the R-R intervals average (SDNN) and the percentage of R-R adjacent intervals differing from each other more than 50 msec (pNN50); in the frequency domain the low (LF) and high frequencies (HF) were analyzed. Twenty healthy volunteers served as control group for heart rate variability. At MIBI-SPECT examination, the scan was found abnormal in 15 patients and normal in five: three patients demonstrated reversible defects in the anteroseptal region, four had irreversible defects in a region (two in the anteroseptal region and two in the lateral region), two had rest defects in two different regions (lateral and inferior, lateral and anteroseptal) that improved during dipyridamole scan, and six had both reversible and irreversible defects: four in a single segment (three anteroseptal and one lateral, and two in two different regions, particularly anteroseptal and lateral, lateral and inferior). All 20 patients showed significantly lower HRV parameters in comparison with the control group, except for pNN50, which indicates decreased physiologic periodic fluctuations of the autonomic nervous system. In six patients who underwent coronary angiography, the epicardial vessels were found completely normal. In view of the high incidence of myocardial hypoperfusion in patients with HRV alterations, the authors hypothesize that autonomic dysfunction may be associated with microvascular disease or metabolic alteration. They also believe that MIBI scintigraphy is a suitable technique in detecting myocardial damage in SLE patients free of clinical manifestation.

[AQRS left axial deviation of more than -30 degrees and incomplete right branch block in isolated ventricular septal defects]. / Deviazione assiale sinistra di AQRS oltre -30 gradi e blocco di branca destra incompleto nei difetti isolati del setto ventricolare.

Electrocardiographic findings in five out of forty-three cases of isolated Ventricular Septal Defects (VSD) demonstrated incomplete Right Bundle Branch Block (RBBB) with left AQRS deviation of more than -30 degrees. These features are said to be characteristic of VSD of the Persistent Common Atrioventricular Canal type. However, in two of our five cases a defect of the Inlet Septum was documented angiographically; in the remaining three cases a defect of the peri-Membranous septum was clearly demonstrated. Moreover hemodynamic data in our patients indicate that right ventricular diastolic overload could not account for RBBB in all of the cases. The clinical importance derived from this experience is that ECG findings of RBBB and left AQRS deviation of more than -30 degrees are not necessarily associated with, and therefore do not represent certain proof of the presence of VSD of the A-V Canal type. The association of this ECG pattern with VSD's other than the A-V Canal type, as previously observed, appears to have an anatomic explanation, which however needs further substantiation through electrophysiologic studies.

[Aortic insufficiency caused by valvular dysplasia in childhood. Apropos of 3 cases]. / Insufficienza aortica da displasia valvolare nella fanciullezza. A proposito di tre casi osservati.

Three patients whose aortic incompetence (A.I.) was recognized in childhood, have had an angiographic study 10, 9 and 4 years, respectively, following the first clinical diagnosis of their valvular disease. A.I. was related, in all three cases, to a dysplastic valve ("floppy valve syndrome"); in none of them was there an abnormal dilatation of ascending aorta. In our opinion, the absence of an abnormal aortic root dilatation separates our cases from those in whom aortic regurgitation is part of Marfan's syndrome, either in its full form or in the "forme fruste". From the clinical viewpoint, the presence of a normal ascending aorta could explain the mild course of these cases, as compared to the rapid progression observed in Marfan's syndrome. In the attempt to differentiate the two types of dysplastic valvular disease from an etiologic viewpoint, we suggest that the basic problem in our patients could be an alteration of the valvular connective tissue development, rather than a genetic disorder, like the one possibly responsible for Marfan's syndrome.

Mixed cryoglobulinemia secondary to visceral Leishmaniasis.

We describe a case of type II mixed cryoglobulinemia, with monoclonal IgMkappa rheumatoid factor, associated with visceral leishmaniasis caused by Leishmania infantum. Involvement of Leishmania antigen(s) in the formation of cryoprecipitable immune complexes was suggested by the fact that cryoglobulinemic vasculitis subsided after antiparasite therapy and that anti-Leishmania antibodies, as well as rheumatoid factor, were enriched in the cryoprecipitate. We observed 2 additional patients with visceral leishmaniasis and cryoglobulinemic vasculitis. All 3 patients had seemingly contracted leishmaniasis in Italy, were hepatitis C virus negative, and were initially diagnosed as having autoimmune disorders. These findings indicate that Leishmania can be an etiologic agent of type II mixed cryoglobulinemia. This parasitosis should be taken into consideration in the differential diagnosis of vasculitides in endemic areas.