Epidemiological study of myasthenia gravis in Sardinia, Italy (1958–1986)

From 1.1.1958 to 31.12.1986, 110 cases of MG were observed in Sardinia, with a mean annual incidence of 2.5 x 1,000,000 inhabitants and prevalence rates of 7.5, 17.6, 31.4 and 45.0 x 1,000,000 inhabitants respectively (prevalence days: 15.10.1961, 24.10.1971, 25.10.1981 and 31.12.1986). The disease was found to be more frequent in women. There were no differences in the distribution of MG in various areas of the island. The muscle group more frequently involved at onset was the ocular. In 6.4% of patients an association with thyroid disorders was observed. The mortality of MG patients was significantly higher than expected. Removal of the thymus, carried out in 58 patients, was shown to be useful in the treatment of the disease, particularly in patients without thymomas. No familial cases were observed.     

1,2,3-triazolo[4,5-f]quinolines. II. Preparation and antimicrobial evaluation of 6-ethyl-6,9-dihydro-1(2)(3)-R-1(2) (3)H-triazolo [4,5-f]quinolin-9-one-8-carboxylic acids as anti-infectives of the urinary tract (1)

Some 6-ethyl-1(2)(3)-R-1(2)(3)H-triazolo[4,5-f]quinolin-9-one-8-carboxy lic acids were prepared as novel analogues of oxolinic acid in order to evaluate the effect on antibacterial activity of the isosteric replacement of the dioxolic moiety with the triazole ring substituted in position 1 or 2. In vitro tests showed a good and selective activity against Escherichia coli (MIC 12.5 micrograms/ml) of compound (XVI).     

Activated Protein C Reduces Graft Neutrophil Activation in Clinical Renal Transplantation

We studied the role of endogenous activated protein C (APC), the major physiological anti-coagulant with concomitant anti-inflammatory properties, on ischemia/reperfusion (I/R) in 45 patients participating in a larger trial comparing three immunosuppressive protocols in cadaveric renal transplantation: perioperative anti-thymocyte globulin (ATG, Fresenius AG, Bad Homburg, Germany), perioperative basiliximab and conventional triple therapy. Blood samples for assessing plasma APC, protein C, and lactoferrin concentrations, neutrophil CD11b and L-selectin expressions and blood leukocyte differential counts were obtained preoperatively and before reperfusion from central venous cannula, complemented with simultaneous samples from iliac artery and graft vein for calculation of transrenal differences (Delta) of study parameters at 1 and 5 min after reperfusion. Unlike basiliximab or conventional therapy groups, ATG infusion induced a substantial increase in plasma APC concentration (119 [88-144]% before infusion vs. 232 [85-1246]% after infusion, p<0.001), resulting in renal graft sequestration of APC at 1 min after reperfusion (Delta=-72 [-567 to 12]%, p<0.001). Graft APC consumption was associated with transrenal reduction of neutrophil activation markers (L-selectin r=0.7, p=0.01; lactoferrin r=-0.6, p=0.02; CD11b r=-0.8, p=0.001), and with both warm (r=0.6, p=0.01) and cold ischemia time (r=0.6, p=0.02) and donor age (r=0.6, p=0.01). These findings suggest that APC has an anti-inflammatory role in I/R injury in clinical renal transplantation.     

Distribution and ontogeny of parvalbumin immunoreactivity in the chicken retina.

The distribution of parvalbumin-like immunoreactivity was studied in the embryonic and postnatal chicken retina. In post-hatched chickens, parvalbumin-like immunoreactivity was confined to amacrine cells. Three distinct subpopulations were identifiable based upon soma position and level of dendritic arborization in the inner plexiform layer. The primary dendrites from parvalbumin-immunoreactive amacrine cells descended vertically into the inner plexiform layer and eventually branched to give rise to a laminarly arrayed plexus in sublamina I, sublamina V and, to a lesser extent, at the boundary between sublaminae III and IV. Parvalbumin-like immunoreactive amacrine cells projecting to sublamina I of the inner plexiform layer were consistently monostratified. Some, but not all, contributed thick fibers to sublamina I that could be followed for long distances across the retina and were generally not radially organized. The parvalbumin-like immunoreactive cells that projected to sublamina V gave rise to a primary dendrite from which three to five fibers branched radially. Collateral branches of these same primary dendrites gave rise to the parvalbumin-like immunoreactive plexus at the interface between sublaminae III and IV. In prenatal chickens, parvalbumin-like immunoreactivity was not detected until embryonic day 14. At this time it appeared as a faint band at the inner nuclear layer-inner plexiform layer boundary in the central retina. By embryonic day 18 the intensity of immunoreactivity and the complexity of the arborizations of the parvalbumin-like immunoreactive dendrites approached that seen in the post-hatched chicken. In the chicken retina, parvalbumin-like immunoreactivity was displayed by morphologically distinct subpopulations of amacrine cells suggesting that these amacrine cells may subserve diverse functions.(ABSTRACT TRUNCATED AT 250 WORDS)     

Family studies of relation between Perthes disease and congenital dislocation of the hip

A family study of Perthes disease and congenital dislocation of the hip was made in the Faroe Islands, with a population of 40 000. The examination included 1123 sibs and first cousins of 43 probands with Perthes disease, 1942 sibs and first cousins of 59 dislocation probands, and 5205 sibs and first cousins of 172 unaffected matched controls. Both conditions occur with exceptionally high incidences in this population. Thus the incidence of Perthes disease was found to be 41: 10 000 males and 7: 10 000 females, of congenital dislocation of the hip 7: 10 000 males and 59: 10 000 females. These figures are 3 to 4 times higher than those commonly observed in Caucasian populations.

Among the 1123 relatives of Perthes probands, we found 10 cases of Perthes disease and 9 cases of dislocation; among the 1942 relatives of dislocation probands, there were 11 cases of Perthes disease and 23 cases of dislocation. Thus both disorders show an accumulation within the same families. On the other hand among the 5205 relatives of probands selected because the hips were unaffected, we found only 3 cases of Perthes disease and 10 cases of dislocation.

Considering the conspicuously low familial accumulation of Perthes disease seen in a low-risk population elsewhere (South Wales), the high incidence of the two disorders and the simultaneously strong intrafamilial accumulation in the Faroe population seem to indicate that the search for exogenous influences, specific to this area, should be intensified.

     

Chronic liver disease in transfusion-dependent thalassaemia: hepatitis B virus marker studies.

The systematic screening of 253 children with transfusion-dependent homozygous beta-thalassaemia revealed a high incidence of hepatitis B virus markers. The highest frequencies of hepatitis B surface antigen (HBsAg) and antibody to hepatitis B core antigen (anti-HBc) were found in the group of patients with the smallest number of transfusions, while the highest frequency of antibody to hepatitis B surface antigen (anti-HBs) was detected in the patients who had had the largest number of transfusions. Follow-up of these patients showed (a) a high incidence of acute hepatitis B, which was mainly subclinical; (b) normal hepatitis B surface antigen clearance and normal antibody to hepatitis B surface development; and (c) a high frequency of increased transaminase values for over six months. In all the subjects with persistently high transaminase, histological examination revealed chronic persistent hepatitis or chronic active hepatitis. Apart from two cases of chronic active hepatitis with no B virus markers, and two cases of chronic persistent hepatitis with HBsAg and anti-HBc in the serum, all these subjects were anti-HBs positive but HGsAg and anti-HBc negative.     

Association of jaagsiekte sheep retrovirus with pulmonary carcinoma in Sardinian moufflon (Ovis musimon)

Bronchiolo-alveolar carcinoma has been described in man and in several animal species, including cattle, dogs, opossums, goats and sheep. In sheep, a bronchiolo-alveolar carcinoma, known as ovine pulmonary carcinoma (OPC), is caused by jaagsiekte sheep retrovirus (JSRV), an exogenous type D retrovirus. In the mid-1980s, a severe outbreak of a disease resembling OPC was described in captive Sardinian moufflon (Ovis musimon). In the present study, the use of polymerase chain reaction (PCR) amplification of nucleic acids extracted from archival material established that JSRV was associated with OPC in affected moufflon. JSRV was detected in the lungs and mediastinal lymph nodes. Immunohistochemical and in-situ PCR demonstrated that in the lungs, JSRV proviral DNA was localized in transformed and untransformed type II pneumocytes and in the alveolar macrophages. In the mediastinal lymph nodes, JSRV DNA was mainly located in the cortical follicles and paracortex. These data suggest that JSRV is the cause of OPC in Sardinian moufflon, as it is in Sardinian sheep.