Disease In Greece Research Articles

Immunoproliferative small intestinal disease in Greece: presentation of 13 cases including two from Albania

Immunoproliferative small intestinal disease (IPSID) represents a spectrum of clinicopathological entities including alpha-chain disease and other types of lymphoplasmacytic proliferations of the lamina propria of the small intestine, presenting with severe malabsorption. IPSID has been described mainly in the Mediterranean, Middle East, and African countries. It occurs rarely in western countries. We present here our experience from Greece describing some interesting findings in cases diagnosed during the years 1970-2002. Current immunological and immunohistochemical methods for the detection of alpha heavy chains and the presence of clonality have been used to study 13 cases of IPSID diagnosed in Greece, two of whom were Albanian residents. The patients were categorized in three subgroups of IPSID: alpha-chain disease (n=8), non-alpha chain disease with other monoclonal immunoglobulins (n=3), and polyclonal 'non-malignant' IPSID (n=2). In several patients the disease had unusual features, and this in some cases delayed the diagnosis. In suspected cases it is thus of the utmost importance to proceed to an exploratory laparatomy. Patients with stage C disease had a short survival, whereas two patients with stage A alpha-chain disease responded to treatment with cyclophosphamide, vincristine and prednisolone, and cyclophosphamide, doxorubicine, vincristine and prednisolone, respectively, have a disease-free long survival of 35 and 12 years, and appear to be cured.

Seven cases of Pompe disease from Greece

We present seven cases of Pompe disease (McKusick 232300; glycogen storage disease type II; acid maltase deficiency) from Greece. The onset of symptoms varied from early childhood to late adulthood, and the patients had quite variable duration of disease. All but one of them had muscle weakness and all had mildly to highly elevated serum creatine kinase. The diagnosis in all cases was confirmed by the finding of acid alpha-glucosidase (EC 3.2.1.3/20) deficiency in cultured skin fibroblasts. Thirteen mutant alleles were identified and nine different pathogenic mutations were encountered. Four were new: c.2071_2072insAGCCG leads to frameshift and total loss of function; c.1856G > A (p.Ser619Asn) leads to 90-95% loss of function; and the splice-site mutations c.1552-3C > G and c.2331+4A > G reduce the number of correct splicing events by more than 90%. The splice-site mutation c.-32-13T > G (IVS1-13T > G) was encountered four times and seems equally common among Greek and other caucasians. The other mutations: c.925G > A (p.Gly309Arg), c.[307T > G; 271G > A] (p.Cys103Gly; Asp91Asn), c.271del and c.1655T > C (p.Leu552Pro) have been reported earlier. Our study highlights the heterogeneity of Pompe disease in Greece and provides tools for diagnosis and carrier detection.

First Record of Fusarium Wilt of Tobacco in Greece Imported as Seedborne Inoculum

Leaf yellowing and brown discoloration was observed in tobacco plants cv. Burley TN97 in tobacco fields of central Greece in 2002. Fusarium oxysporum f. sp. nicotianae was isolated from symptomatic plants and Koch's postulates were fulfilled. The pathogenicity of the isolated fungus was examined on five tobacco cultivars (Burley TN97, BurleyB21, VirginiaBE9, Virginia Niki and Anatolika KE26/2). The pathogen was present in tobacco seed batches imported in 2000 and 2001, which indicates that the infected seed is most probably the primary source of the disease in Greece. As Fusarium oxysporum f. sp. vasinfectum can also cause vascular wilt in tobacco, the hypothesis that the isolated F. oxysporum strain belongs to f. sp. vasinfectum was excluded by a pathogenicity test to cotton cv. Acala SJ-2. This is the first report of F. oxysporum f. sp. nicotianae in Greece and the second in the European Union, although the seedborne nature of the pathogen has not been previously reported in Europe.

Phenotypic assessment of Neisseriameningitidis isolates obtained from patients with invasive meningococcal disease in Greece, 1993–2003: Implications for serogroup B vaccines based on PorA serosubtype antigens

Serogroup B is the major isolate from patients with invasive meningococcal disease (IMD) in Greece. This study used the whole cell enzyme-linked immuosorbent assay (ELISA) with monoclonal antibodies to screen N eisseria meningitidis isolates obtained from patients with IMD between 1993 and 2003 to determine if serosubtypes included in the hexavalent Por A OMP vaccines being tested in northern Europe were prevalent in Greece. During this period there were significant changes in the proportions of serogroups B and C isolated from patients. Serogroup C was predominant in 1996–1997 but fell sharply with corresponding increases in serogroup B. Of the 591 isolates sent to the National Meningitis Reference Laboratory in Athens during this period, 325 (55%) were serogroup B. Among those tested for serosubtype, porA proteins used for the vaccine being tested in Britain were detected on 85/284 (30%) strains and for the vaccine being tested in the Netherlands 175/284 (62%). P1.14 (58/284, 20%) the predominant serosubtype among the Greek isolates, is not present in either vaccine formulation; 23/284 (8%) strains did not react with any of the monoclonal antibodies. Our results indicate that introduction of the vaccines currently being evaluated in northern Europe would not be warranted in the Greek population.

Mediterranean Diet and Survival Among Patients With Coronary Heart Disease in Greece

Background The Mediterranean diet has been hypothesized to reduce fatality among patients with coronary heart disease. Methods We examined the association between the degree of adherence to the traditional Mediterranean diet and survival of persons with diagnosed coronary heart disease at enrollment, in a population-based prospective investigation of 1302 Greek men and women, who were followed up for an average of 3.78 years (the European Prospective Investigation Into Cancer and Nutrition cohort). Information on usual dietary intakes during the year preceding enrollment was recorded through a validated food frequency questionnaire. Adherence to the Mediterranean diet was assessed by a 10-unit Mediterranean diet score that incorporates the salient characteristics of this diet. Proportional hazards regression was used to assess the relation of overall degree of adherence to the Mediterranean diet with mortality overall or by cause (cardiac vs noncardiac). Results Higher adherence to the Mediterranean diet by 2 units was associated with a 27% lower mortality rate among persons with prevalent coronary heart disease at enrollment (total deaths, 131; adjusted mortality ratio, 0.73; 95% confidence interval, 0.58-0.93). The reduced mortality was more evident and amounted to 31% (total deaths, 85; adjusted mortality ratio, 0.69; 95% confidence interval, 0.52-0.93) when only cardiac deaths were considered as the relevant outcome. Associations between individual food groups contributing to the Mediterranean diet score and mortality were generally not significant. Conclusion Greater adherence to the traditional Mediterranean diet is associated with a significant reduction in mortality among individuals diagnosed as having coronary heart disease.

Mediterranean Diet and Survival Among Patients With Coronary Heart Disease in Greece

The Mediterranean diet has been hypothesized to reduce fatality among patients with coronary heart disease. We examined the association between the degree of adherence to the traditional Mediterranean diet and survival of persons with diagnosed coronary heart disease at enrollment, in a population-based prospective investigation of 1302 Greek men and women, who were followed up for an average of 3.78 years (the European Prospective Investigation Into Cancer and Nutrition cohort). Information on usual dietary intakes during the year preceding enrollment was recorded through a validated food frequency questionnaire. Adherence to the Mediterranean diet was assessed by a 10-unit Mediterranean diet score that incorporates the salient characteristics of this diet. Proportional hazards regression was used to assess the relation of overall degree of adherence to the Mediterranean diet with mortality overall or by cause (cardiac vs noncardiac). Higher adherence to the Mediterranean diet by 2 units was associated with a 27% lower mortality rate among persons with prevalent coronary heart disease at enrollment (total deaths, 131; adjusted mortality ratio, 0.73; 95% confidence interval, 0.58-0.93). The reduced mortality was more evident and amounted to 31% (total deaths, 85; adjusted mortality ratio, 0.69; 95% confidence interval, 0.52-0.93) when only cardiac deaths were considered as the relevant outcome. Associations between individual food groups contributing to the Mediterranean diet score and mortality were generally not significant. Greater adherence to the traditional Mediterranean diet is associated with a significant reduction in mortality among individuals diagnosed as having coronary heart disease.

First report of canker stain disease of plane trees, caused by Ceratocystis fimbriata f. sp. platani in Greece

In September 2003, dead and dying plane trees (Platanus orientalis) were observed in seven different localities of the Messinia prefecture of the south-western Peloponnese, Greece. Cankers were found on both trunks and branches. The inner bark and the cambial region of the cankered area were discoloured bluish-black and the underlying wood stained dark reddish-brown to bluish-black. Stained streaks extended longitudinally in the wood beyond the dead bark. In cross-section, the stained wood formed characteristic radial patterns. The fungus Ceratocystis fimbriata f. sp. platani was consistently isolated from stained wood near the canker margins. Cultures on malt extract agar (MEA) were hyaline to light olive brown, with a radial growth rate of 12–15 mm week−1 at 24°C and had a pronounced smell of banana. Black, globose perithecia (150–300 µm in diameter) with a long neck (500–800 µm in length) were observed on 1-week-old cultures. The ascospores were 4–6 × 3–5 µm, having a characteristic bowler hat shape. All three types of asexual spores were also observed: hyaline, truncated, cylindrical endoconidia; light doliform endoconidia; and dark, thick-walled conidia (chlamydospores) (Webster & Butler, 1967). Pathogenicity tests were performed in a glasshouse, on 2-year-old potted plants of P. orientalis, using one of the isolates deposited in the culture collection of University of Athens (ATHUM 5046). The stems of trees were wound-inoculated with mycelial agar plugs. Necrotic lesions 3–4 cm in length were observed on the stems 5 weeks after inoculation. Ceratocystis fimbriata f. sp. platani was consistently re-isolated from stained wood 2–2·5 cm from the inoculation point. No symptoms developed on control plants inoculated with sterile MEA. Ceratocystis fimbriata f. sp. platani is considered to be indigenous to the USA. In Europe the pathogen has caused severe attacks in Italy and France. It has also been reported in Switzerland and Spain (Panconesi, 1999). This is the first report of C. fimbriata f. sp. platani in Greece. It is an EPPO A2 quarantine organism (EPPO/CABI, 1997) and sanitation measures have been suggested to control the disease in Greece. The authors are grateful to Drs P. Capretti and A. Panconesi for verifying the identity of the fungus and Dr S. Woodward for critical evaluation of the report.

Polypoidal choroidal vasculopathy and exudative age-related macular degeneration in Greek population.

To study the prevalence, the clinical features, and the visual prognosis without treatment of polypoidal choroidal vasculopathy (PCV) in a large series of Greek patients presenting with exudative maculopathy. The medical records, photographs,as well as fluorescein and indocyanine green(ICG) angiograms of a series of 268 consecutive elderly white Greek patients, who were originally diagnosed as having exudative age-related macular degeneration (AMD) were reviewed retrospectively. In all, 22 of the 268 (8.2%) patients initially suspected of having AMD were ultimately diagnosed with PCV. In 15 of the 22(68.2%) patients with PCV, the polypoidal lesions were located in the peripapillary area. Large soft drusen were present in only two fellow eyes of the 10 (20%) patients with unilateral PCV compared with 120 fellow eyes of the 148 (81.1%) patients with unilateral AMD. At the last examination, 11 of the 22(50%) patients with PCV and 120 of the 246(48.8%) patients with AMD presented a visual acuity of less than 6/60 in at least one eye due to scar formation in the macula. PCV is not an infrequent disease in Greece. A measurable number of Greek patients with findings suggestive of exudative AMD will instead have PCV. ICG angiography is important in differentiating between these two clinical entities. In Greeks, polypoidal lesions are predominantly peripapillary and are not usually associated with macular drusen in the fellow eye. PCV and exudative AMD do not differ significantly in terms of their natural course and visual prognosis in Greek patients.

Immunohistochemical Detection of Encephalomyocarditis Virus (EMCV) Antigen in the Heart of Experimentally Infected Piglets

Seven 40-day-old piglets were inoculated with encephalomyocarditis virus (EMCV) strain 424/90, isolated from an outbreak of the myocardial form of the disease in Greece. Two non-infected animals were used as controls. Of the seven inoculated piglets, five died suddenly on day 1·5, 2 (two piglets), 2·5 or 4 post-inoculation (p.i.). The remaining two and the control piglets were killed on day 8 p.i. EMCV antigen was detected immunohistochemically in endothelial cells of capillaries from 1·5 to 2·5 days p.i. only, but was found in cardiac muscle cells, Purkinje fibres and macrophages on all occasions up to day 8 p.i. In endothelial cells and macrophages, EMCV antigen was detected intracytoplasmically, but in cardiac muscle cells and Purkinje fibres it was observed intracytoplasmically or intranuclearly, or both. The frequent presence of EMCV antigen in Purkinje fibres suggests an explanation for the sudden death of the piglets.

First Report of Occurrence of Potato Tuber Necrotic Ringspot Disease in Greece.

Potato tuber necrotic ringspot disease (PTNRD), caused by potato Y potyvirus isolate NTN (PVYNTN) of the N virus strain group, was first described in Hungary in 1980. It has spread throughout Europe, with the most recent reports from Portugal and Italy in 1997 to 1998 (1). Superficial necrotic ringspot areas on tubers, typical of PTNRD, were first observed in commercial potato fields of the Nevrokopi region in northern Greece in 1994. Affected cultivars included Timate and, to a lesser extent Spunta, the original seed of which came from the Netherlands. PVY was identified from all tubers tested by indexing on Nicotiana tabacum and subsequent testing of the plants by double antibody sandwich-enzyme-linked immunosorbent assay (DAS-ELISA). The potato seed lots were rejected by the local authorities. PTNRD reappeared in the same area in 1998 in a more aggressive manner. Cultivar Hermes, imported from Scotland, was most affected, with very severe symptoms in 80% of the tubers. Symptoms appeared in early September, 40 days after defoliation of the plants. Other cultivars were affected at lower rates; cv. Spunta showed typical symptoms and cvs. Fabola, Santana, and Irvila exhibited atypical cracks and blisters. In all cases, PVY was isolated in N. tabacum and its presence confirmed by DAS-ELISA. Immunocapture-reverse transcription-polymerase chain reaction with specific PVYNTN primers (2) detected the characteristic 835-bp product in all cultivars tested. The incidence of PTNRD seems to be expanding in northern Greece, where it has become a threat to potato production. PTNRD symptoms were also observed in southern Greece (Ahaia) in experimental "crossing" fields of seed stocks. In this case, the disease seems to have spread especially in cv. Marfona.

Detection of IgG and IgM to meningococcal outer membrane proteins in relation to carriage of Neisseria meningitidis or Neisseria lactamica

Carriage of non-serogroupable Neisseria meningitidis or Neisseria lactamica induces antibodies protective against meningococcal disease. Antibodies directed against outer membrane proteins are bactericidal and the serotype and subtype outer membrane protein antigens are being examined for their value as vaccine candidates for serogroup B disease. The aim of this study was to examine the effect of carriage of these two Neisseria species among children and young adults on induction of antibodies to outer membrane components from strains causing disease in Greece. Among 53 patients with meningococcal disease, IgG or IgM antibodies were detected by ELISA in 9 of 13 (69%) from whom the bacteria were isolated and 27 of 40 (67%) who were culture-negative. For military recruits ( n=604), the proportion of carriers of meningococci with IgM or IgG to outer membrane proteins was higher than non-carriers, P<0.05 and P=0.000000, respectively. Among school children ( n=319), the proportion with IgM or IgG to outer membrane proteins for carriers of meningococci was higher compared with non-carriers, P=0.000000 and P=0000043, respectively. Carriage of N. lactamica was not associated with the presence of either IgM or IgG to the outer membrane proteins in the children. The higher proportion of children (50%) with IgM to outer membrane proteins compared with recruits (10%) might reflect more recent exposure and primary immune responses to the bacteria. The lack of association between antibodies to outer membrane proteins and carriage of N. lactamica could reflect observations that the majority of N. lactamica isolates from Greece and other countries do not react with monoclonal typing reagents. Bactericidal antibodies to meningococci associated with high levels of IgG to N. lactamica were found in a previous study; these are thought to be directed to antigens other than outer membrane proteins or capsules and imply antigens such as lipo-oligosaccharide are involved in induction of antibodies cross-reactive with meningococci.

Dietary habits of patients with renal stone disease in Greece

Summary Objective: To assess the dietary habits of 62 renal stone patients and to relate their diet to the likelihood of nephrolithiasis. Subjects and methods: All subjects completed a dietary questionnaire including questions regarding anthropometric characteristics, dietary habits and food consumption frequencies. Subjects were grouped according to sex. Results: Sixty per cent of the subjects were male and 40% female. Seventy-nine per cent of the patients were obese (BMI > 25). The percentage of obese individuals was higher for men (84%) than for women (72%). Furthermore, BMI increased with age with a peak at the age of 50 years. All subjects had a high meat intake indicating a high animal protein consumption. Forty-two per cent of the subjects consume meat 5–8 times per week and 24% consume meat 9–12 times per week. All subjects had a low milk and dairy products intake as well as low vegetable and fruit intake, indicating a strong possibility of low calcium and fibre intake. Conclusion: The results of the dietary survey recorded a diet which is associated with an increased risk of nephrolithiasis. The majority of the subjects were obese, had a high intake of animal protein from meat, a low intake of foods rich in calcium and a low number of meals per day.

Carriage of Neisseria meningitidis and Neisseria lactamica among ethnic Greek school children from Russian immigrant families in Athens.

During February and March 1995, a survey of meningococcal carriage in 625 school children was carried out in a suburb of Athens in which there was a large number of ethnic Greeks who had immigrated from Russia beginning in the early 1990s. The objectives of the study were: (1) to determine if factors associated with carriage of meningococci observed in a previous study of Greek school children were similar for the immigrant population; (2) to compare phenotypic characteristics of meningococci from the immigrant population with those isolated from children in Athens. Overall isolation rate for meningococci was 82/625 (13.1%), significantly higher than that found for school children in Athens (5.8%) during the winter of 1990 1991 (5.8%) (chi=25.98, P=0.0000003). By univariate analysis, carriage was not associated with sex, number of individuals per household, blood group, secretor status, socioeconomic level or maternal smoking; however, it was associated with fathers' smoking. The high proportion of men who smoked compared with the low proportion of women smokers might contribute to this finding. The main serogroup of meningococci isolated from this population was A (28%). While serogroup A appears to be more prevalent among Russian and Kurdish immigrants (14%) than among Greek school children or military recruits (4%), there has not been an increase in group A meningococcal disease in Greece. The isolation rate for N. lactamica was high 105/625 (17.3%). A few of these strains bound some of the monoclonal antibodies used for meningococcal serotyping and subtyping, and they are being examined in greater detail.

Carriage of Neisseria meningitidis and Neisseria lactamica among ethnic Greek school children from Russian immigrant families in Athens

During February and March 1995, a survey of meningococcal carriage in 625 school children was carried out in a suburb of Athens in which there was a large number of ethnic Greeks who had immigrated from Russia beginning in the early 1990s. The objectives of the study were: (1) to determine if factors associated with carriage of meningococci observed in a previous study of Greek school children were similar for the immigrant population; (2) to compare phenotypic characteristics of meningococci from the immigrant population with those isolated from children in Athens. Overall isolation rate for meningococci was 82 625 (13.1%), significantly higher than that found for school children in Athens (5.8%) during the winter of 1990–1991 (5.8%) ( χ 2 = 25.98, P = 0.0000003). By univariate analysis, carriage was not associated with sex, number of individuals per household, blood group, secretor status, socioeconomic level or maternal smoking; however, it was associated with fathers' smoking. The high proportion of men who smoked compared with the low proportion of women smokers might contribute to this finding. The main serogroup of meningococci isolated from this population was A (28%). While serogroup A appears to be more prevalent among Russian and Kurdish immigrants (14%) than among Greek school children or military recruits (4%), there has not been an increase in group A meningococcal disease in Greece. The isolation rate for N. lactamica was high 105 625 (17.3%). A few of these strains bound some of the monoclonal antibodies used for meningococcal serotyping and subtyping, and they are being examined in greater detail.

Recent emergence of serogroup C meningococcal disease in Greece.

The number of cases of meningococcal disease reported to the Meningitis Reference Laboratory in Athens rose dramatically in 1996-1997. The aims were (1) to determine if the increase was due to introduction of new strains, (2) to assess the geographic and age distribution of the cases, (3) to compare antibiotic sensitivity patterns of the current isolates with strains from the early 1990s. In 1993-1994, 15/19 (74%) of the cases for which information on age was available were in children < or = 5 years; in 1995-1997, 80/179 (45%) of cases were in children < or = 5 years and 99 (55%) in the older age range (P < 0.02). From 593 cases in 1993 1997, 214 (36%) isolates were available for characterisation. Serogroup B was predominant in the early 1990s, but by 1997, serogroup C accounted for 46/72 (64%) of isolates and serogroup B for 25/72 (35%). Serogroup B was predominant in children < or = 5 years (44/78, 56%) but only 19/99 (18%) of older children and adults (P=0.0000005). Sulfonamide resistance decreased from 10/22 (45%) in 1993-1994 to 27/192 (14%) in 1995-1997 (P<0.01). Multilocus enzyme electrophoresis of 70 strains obtained during this period identified the epidemic ET-15 clone in 24 (34.3)%. The profiles of the Greek ET-15 isolates were identical to C:2a:P1.2(P1.5) strains responsible for the epidemic in the Czech Republic which began in 1993. This genotype was not found in Greek strains isolated prior to 1993. We conclude that the increase in meningococcal disease is due to introduction of the epidemic serogroup C:2a:P1.2(P1.5) strain responsible for disease in the Czech Republic and Canada.

Recent emergence of serogroup C meningococcal disease in Greece

The number of cases of meningococcal disease reported to the Meningitis Reference Laboratory in Athens rose dramatically in 1996–1997. The aims were (1) to determine if the increase was due to introduction of new strains, (2) to assess the geographic and age distribution of the cases, (3) to compare antibiotic sensitivity patterns of the current isolates with strains from the early 1990s. In 1993–1994, 15 14 (74%) of the cases for which information on age was available were in children ≤ 5 years; in 1995–11997, 80 179 (45%) of cases were in children ≤ 5 years and 99 (55%) in the older age range ( P < 0.02). From 593 cases in 1993–1997, 214 (36%) isolates were available for characterisation. Serogroup B was predominant in the early 1990s, but by 1997, serogroup C accounted for 46 72 (64%) of isolates and serogroup B for 25 72 (35%). Serogroup B was predominant in children ≤ 5 years ( 44 78 , 56%) but only 19 99 (18%) of older children and adults ( P = 0.0000005). Sulfonamide resistance decreased from 10 22 (45%) in 1993–1994 to 27 192 (14%) in 1995–1997 ( P < 0.01). Multilocus enzyme electrophoresis of 70 strains obtained during this period identified the epidemic ET-15 clone in 24 (34.3)%. The profiles of the Greek ET-15 isolates were identical to C:2a:P1.2(P1.5) strains responsible for the epidemic in the Czech Republic which began in 1993. This genotype was not found in Greek strains isolated prior to 1993. We conclude that the increase in menigococcal disease is due to introduction of the epidemic serogroup C:2a:P1.2(P1.5) strain responsible for disease in the Czech Republic and Canada.

Cat scratch disease in Greece

An indirect fluorescent antibody test for Bartonella henselae, B quintana, and B elizabethae was performed in all 18 children who presented to our paediatric outpatient clinic with cat scratch disease...

First Report of Anthracnose on Weeping Willow in Greece.

A serious disease of weeping willow (Salix babylonica) trees in home and public gardens was observed in the Chalkidiki region (northern Greece) during the spring of 1995 and 1996. Purplish-brown or black, small necrotic spots appeared on the leaves and often on leaf veins. Leaves became distorted and fell. Small, light, sunken cankers with dark brown rim and paler center, elongated in shape, appeared on the young shoots and on the leaf petioles. Severely affected shoots lost their weeping habit on which the tree's ornamental value is based. Whitish acervuli covered the leaf spots and the cankers. Masses of conidia emerged from the acervuli under high relative humidity conditions. The conidia were hyaline, clavate to pyriform, two-celled (unequally), measuring 14 to 19 × 4 to 7 μm, and belonged to the fungus Marssonina salicicola (Bres.) Magnus (1), which is the anamorph of the ascomycete Drepanopeziza sphaeroides (Pers.) Hohnel, the cause of anthracnose of weeping willow. The outbreak of the disease can be attributed to the rainy weather (70.5 and 105.5 mm of rain occurred in spring 1995 and spring 1996, respectively) and temperatures of 20°C. The disease is known to cause serious damage in some European countries, Canada, Egypt, Argentina, and New Zealand. This is the first report of the disease in Greece. Reference: (1) I. Vegh and J. Velastegui. Cryptogam. Mycol. 4:345, 1983.

Characterization of glucocerebrosidase in Greek Gaucher disease patients: mutation analysis and biochemical studies.

Gaucher disease is the most frequent lysosomal storage disease in Greece, accounting for 24% of all lysosomal disorders diagnosed during the last 13 years at the Institute of Child Health in Athens. The nature of the defects in glucocerebrosidase in Greek Gaucher patients with non-neuronopathic (type 1) and neuronopathic (types 2 and 3) phenotypes was investigated at the level of the glucocerebrosidase gene and enzyme activity. Mutation analysis performed in 10/23 Gaucher patients with different types of the disorder led to the identification of four mutations, N370S, L444P, R463C and D409H, comprising 75% of the investigated alleles. N370S was only found in association with type 1 disease. The genotype D409H/R463C was identified for the first time and was associated with the severe type 2 disorder. There was no correlation between residual in vitro enzyme activity and either phenotype or genotype. However, in cultured fibroblast of the neuronopathic cases, glucocerebrosidase protein concentration was reduced and the capacity to degrade exogenous C6NBD-glucosylceramide was more severely impaired.

Olive knot disease in Greece1

Olive knot disease caused by Pseudomonas syringae subsp. savastanoi is widespread all over Greece where olive is grown and its severity varies from heavy in some areas and cultivars to very mild in others. Hypertrophied enlargements (galls or knots) more or less spherical in shape and up to 2.5 cm in diameter develop mostly on shoots, twigs, branches and trunks. Fruit infection, as roughly circular brown spots of 0.5‐2.5 mm in diameter, is another form of disease which develops during wet summers. All olive cultivars are equally susceptible to the pathogen. However, the differences in disease distribution and severity observed between cultivars and areas are directly related to the degree of wounding from whatever cause (frost, hailstorm, harvesting injuries), especially when they are accompanied by rainy and wet weather or moist winds. Harvesting by beating the trees with sticks is an important epidemiological factor for disease incidence in Greece. Suggested control measures are summarized.