Systemic lupus erythematosus occurring in a patient with Niemann-Pick type B disease.

Niemann-Pick disease is an inherited lipid storage disorder caused by the deficiency of acid sphingomyelinase, which results in accumulation of sphingomyelin within cells of several organs and consequent tissue damage. The broad clinical spectrum of this disorder may overlap with that of systemic lupus erythematosus, hindering differential diagnosis. Herein, we report the case of a patient affected by Niemann-Pick type B disease intertwined with clinical and serological features of systemic lupus erythematosus. Two novel mutations in the SMPD1 gene were found in compound heterozygosity: p.A36V and IVS2 + 8 T > G.

Antiviral properties from plants of the Mediterranean flora.

Natural products are a successful source in drug discovery, playing a significant role in maintaining human health. We investigated the in vitro cytotoxicity and antiviral activity of extracts from 18 traditionally used Mediterranean plants. Noteworthy antiviral activity was found in the extract obtained from the branches of Daphne gnidium L. against human immunodeficiency virus type-1 (EC50 = 0.08 µg/mL) and coxsackievirus B5 (EC50 = 0.10 µg/mL). Other relevant activities were found against BVDV, YFV, Sb-1, RSV and HSV-1. Interestingly, extracts from Artemisia arborescens L. and Rubus ulmifolius Schott, as well as those from D. gnidium L., showed activities against two different viruses. This extensive antiviral screening allowed us to identify attractive activities, offering opportunities to develop lead compounds with a great pharmaceutical potential.

An epidemiological survey of TYLCD in southern Sardinia (Italy).

Tomato yellow leaf curl Sardinia virus (TYLCSV) and Tomato yellow leaf curl virus (TYLCV) are among the most virulent pathogens of greenhouse tomatoes in Sardinia (Italy). To investigate the relationship between seasonal population trends of the vector Bemisia tabaci and the dynamics of TYLCD spread to susceptible crops, between May and October 2007 we carried out a survey in a tomato growing area located in the south of the island. On three farms specialized in the production of fresh market tomatoes we monitored, outside commercial greenhouses, the following parameters related to TYLCD epidemiology: mean weekly catches of the whiteflies B. tabaci and Trialeurodes voporariorum on yellow sticky traps, ratio between the two whitefly species and proportion of B. tabaci adults carrying TYLCSV/TYLCV in adult samples collected on hosts not susceptible to the disease, proportion of tomato plants infected by TYLCSV/TYLCV after a two-week exposure to open field conditions. Generally speaking, the flight activity of whiteflies increased during spring, reached a peak in May or June and gradually declined in summer. At the beginning of the survey, T. vaporariorum was found to be the prevalent species, but after a shift in composition of whitefly populations during July, B. tabaci became predominant. While the percentage of vector adults carrying the viral agents of the disease was relatively high up to July, with maximum values ranging between 14 and 25%, during the following months it decreased to less than 5%. The incidence of TYLCD in the plants exposed outside the greenhouses showed a similar trend in the sites surveyed, with two peaks roughly coinciding with the beginning and end of summer. Therefore, two distinct phases of TYLCD spread were observed: from spring to midsummer when the disease was transmitted by low 8. tabaci populations with relatively high proportions of virus carriers; from midsummer to autumn, when the disease was spread by larger vector populations with low percentages of individuals carrying the viruses. Further studies are necessary to gain a better understanding of the interactions among B. tabaci biotypes, TYLCSV/TYLCV and their hosts.

Seasonal trends of whitefly populations in a Mediterranean tomato growing area.

The whiteflies Bemisia tabaci and Trialeurodes vaporariorum and their associated viruses constitute a major threat to tomato crops in the Mediterranean region. Continuous host availability and mild climate are thought to be among the factors contributing to the outbreaks of whitefly-related problems in this area. We carried out a year-long survey to investigate the relative contribution of different plants, agricultural and not, and indoor/outdoor crops as hosts of the two whiteflies and the tomato yellow leaf curl disease (TYLCD) in a multi-crop system typical of tomato growing areas in southern Sardinia (S. Margherita di Pula, Cagliari, Italy). For this purpose, during 2005 we monitored whitefly population trends in different plots of a horticulture farm, evaluated seasonal changes in the infestation density of the two pests on the most represented host species and assessed the incidence of TYLCD on tomato crops and susceptible weeds. Whitefly catches on yellow sticky traps were found to be higher inside et along the external perimeter of greenhouses compared to open field crops or uncultivated areas, thus suggesting significant adult movement between indoor and outdoor patches. In most plots flight activity increased between late spring and late summer, peaking in July. The number of immatures of the two whitefly species showed similar dynamics, but while T. vaporariorum reached the highest densities in greenhouse tomato crops in June, peak levels of 8. tabaci were recorded between July and August in outdoor horticultural crops and weeds. The occurrence of TYLCD was detected all year round on weed hosts, but the highest number of infected plants was observed in June on long cycle tomato crops. The present survey has demonstrated the contribution of non-agricultural plants the maintenance of tomato yellow leaf curl disease in the study site. However, tomato crops established in summer as major reservoirs of TYLCD-associated viruses and presumably played a key role in the spread of the disease to autumn tomato crops. The implementation of a crop-free period between successive susceptible crops, or at least the avoiding of whitefly movement through greenhouse openings and the complete destruction of plants after final harvest, would probably reduce the severity of TYLCD epidemics in this area.

Childhood Acute Lymphoblastic Leukemia: A Cluster in Southwestern Sardinia (Italy).

In response to public concern about an increase in the incidence of leukemia among children in southwestern Sardinia (Italy), incident cases of childhood cancer (ages 0-14) were ascertained among residents in the province of Cagliari, which comprises all of southern Sardinia, in 1974-89. Completeness of the ascertainment of leukemia cases was validated by comparison with estimates derived from official statistics of mortality and survival curves. A significant excess risk of childhood acute lymphoblastic leukemia (cALL) was found for children residing in the town of Carbonia. The risk was highest in 1983-85, when seven cases occurred versus 0.8 expected. No birth-cohort effect was observed. The cALL incidence rate was significantly higher among children born and residing in Carbonia than among children born in Carbonia but residing elsewhere. However, the cALL cases did not cluster within the town of Carbonia. The proximity of the largest industrial settlement in the region of Sardinia raised the suspicion that environmental pollution was responsible for the observed excess. Information about industrial emissions from this settlement prior to the appearance of the cALL cluster was not sufficient to reject or confirm the hypothesis.

Childhood leukemia in south-west Sardinia (Italy).

AIM AND BACKGROUND: Public concern on an increased incidence of childhood leukemia in SW Sardinia prompted the authors to an epidemiological investigation. METHODS: Incident childhood neoplasms observed in the Cagliari province (Sardinia--Italy) in 1974-1989 were registered. Expected cases of the most frequent childhood cancers were calculated for each town, based on the sex-and age-specific incidence rates in the province. RESULTS: An excess risk of childhood acute lymphoblastic leukemia (ALL) was observed in Carbonia, a town located in the SW part of the province. The risk was highest in 1983-85, when 7 cases occurred versus 0.8 expected (RR = 8.7; 95% C.I. = 4.6, 16.3). No spatial clustering of ALL cases was observed within the town. CONCLUSIONS: A significantly higher than expected incidence of childhood ALL was observed in the town of Carbonia in 1983-85. In alternative to chance, possible exposure to environmental pollutants from a near industrial settlement is discussed as the cause of the observed excess, but it is far to be proven. Other hypotheses, including a viral infection in a population with increased susceptibility, as suggested for new urban settlements, cannot be discarded.

[Incidence, mortality and survival in childhood tumors in meridional Sardinia. The Cagliari province, 1981-1988]. / Incidenza, mortalità e sopravvivenza per tumori infantili nella Sardegna meridionale. La provincia di Cagliari per gli anni 1982-1988.

Childhood cancer incidence, mortality and relative survival rates have been estimated in the province of Cagliari for the years 1982-86. Cases were collected from pediatric and non pediatric units operating either in the province or elsewhere. Deaths were identified through the registry offices of municipal administrations. A total of 151 cases were identified, corresponding to an incidence rate of 115.0 per million. Survival rate at three years of diagnosis was 63.3%.

[Disseminated intravascular coagulation in children. Therapeutic problems]. / La CID in età pediatrica. Problemi terapeutici.

The AA, after discussion on physiopathological and clinical aspects of DIC in childhood, focused their attention mostly on the utilization of AT III concentrates.