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The structure of goat hemoglobins. V. A fourth beta chain variant (beta-D-Malta; 69 Asp is replaced by Gly) with decreased oxygen affinity and occurring at a high frequency in Malta.

During a survey of hemoglobin types in goats in the Republic of Malta a variant (Goat Hb D-Malta) was discovered which differs from normal goat Hb A by the substitution of an aspartyl residue in position beta 69 (E13) by a glycyl residue. The gene frequency of the beta D allele was 0.255; 29 homozygous Hb D goats were present among 327 animals sampled. Homozygous Hb D goats also produce Hb C, whose beta chains are the product of a non-allelic beta C structural gene. Goat Hb D-Malta has a distinctly decreased affinity for molecular oxygen.

Amino Acid Sequence

[In vitro erythrophagocytosis in detecting opsonizing antibodies in a patient with Malta fever. A hypothesis on the explanation of in vivo hemophagocytosis in other patients with Malta fever].

In vitro agglutination and phagocytosis of erythrocytes by homologous neutrophil granulocytes and monocytes could be observed using the blood of a healthy person (blood group A, Rh+) to detect opsonising antibodies in the serum of a patient with Malta fever (blood group O, Rh+). Agglutination and erythrophagocytosis were independent of complement or the presence of Brucella melitensis. In patients with Malta fever and blood groups O and B, respectively, antibodies could be detected on the surface of erythrocytes of blood group A when used as antigen with the help of an immunofluorescence test. The same happened with a serum of one patient with blood group A. In vivo erythrophagocytosis detected in the bone marrow of patients with Malta fever is, therefore, possibly due to common antigens of erythrocytes of blood group A and B. melitensis.

ABO Blood-Group System

Assessment and comparison of human exposure to lead between Belgium, Malta, Mexico and Sweden.

This is a follow-up study of UNEP/WHO Pilot Project on Assessment of Human Exposure to Lead and Cadmium through Biological Monitoring, carried out in 1983/1984. The main objectives of the follow-up study were: to study whether differences in blood-lead levels found between countries in the Biological Monitoring Project were confirmed and were primarily due to exposure via ingested lead (oral intake) or via inhaled lead; to make a preliminary survey, in selected areas, of the possible sources of high exposure (Malta, Belgium). Four countries participated: Belgium, Malta, Mexico, Sweden. To insure comparability between the populations, certain categories were monitored: teachers for socio-economic status, non smoking males, aged between 25 and 50 years old. The blood-lead concentration (PbB) was measured to determine the current exposure and the feces-lead excretion (PbF) was determined to find out the part of the exposure due to ingestion. Blood-lead levels and feces-lead excretion were very different between the four countries. Median values for PbB, in micrograms Pb/1, were, in decreasing values 247 in Malta, 188 in Mexico, 135 in Belgium and 53 in Sweden. Median values for PbF, in micrograms Pb/day, were 361 in Malta, 159 in Mexico, 82 in Belgium and 22 in Sweden. Oral intake seems to be the major exposure route in the four countries. The relationship between PbB and PbF is curvilinear. To investigate the possible source of high exposure, a preliminary survey was made in Malta and Belgium; lead in air suspended and sedimenting particles was monitored as well as the lead concentration in some food and street dust samples. Concerning the comparison of lead concentrations in these environmental samples between Belgium and Malta, no major differences were detected. The high internal exposure to lead in Maltese people, as measured by PbB, is probably due to a combination of several factors.

Adult

Biosynthesis of hemoglobin F Malta-I in culture by adult circulating erythropoietic precursors.

By using a methylcellulose clonal assay, we cultured peripheral blood erythropoietic precursors (BFU-E) from an adult couple whose child had HbF Malta-I(gamma 117 His leads to Arg), a G gamma variant, and measured the synthetic rates of HbA, HbF, and HbF Malta-I. Hemoglobin was labeled with 14C-amino acid in culture, separated by slab gel isoelectric focusing technique, and quantitated by autoradiographic or fluorographic method. Culture of BFU-E from both parents revealed significant HbF biosynthesis. HbF Malta-I was present in culture of the father's cells and comprised about 24% of total HbF. When we analyzed Hb biosynthesis in individual bursts, all bursts contained HbA and HbF in varying ratios. The frequency distribution of the individual bursts differing in percentages of HbF biosynthesis approached normal distribution. While the relative ratio of HbF Malta-I to total HbF biosynthesis in individual bursts also revealed significant variation, its frequency distribution did not show a normal distribution. There was a positive correlation between the ratios of HbF/Hb and HbF Malta-I/HbF in individual bursts.

Autoradiography

gamma chain composition in five Italian newborns heterozygous for Hb F Malta G gamma-117 His leads to Arg.

The percentage of Hb F Malta G gamma-177 His replaced by Arg and the gamma chain composition have been evaluated at birth and at different times after birth in five Italian newborns heterozygous for this variant. The percentage of Hb F Malta ranged at birth from 24% to 31% of the total Hb F, while the average G gamma/A gamma chain ratio was about 7/3, overlapping the values observed in normal newborns. T gamma chains were detected in three out of five newborns, with a percentage of about 10% of the total Hb F. After birth the Hb F Malta declined faster than the total Hb F; thus, the Hb F decrease during the first 45 d of life is mainly due to the switch-off of the G gamma locus containing the Hb F Malta gene.

Aging

Detection and quantitation of the fetal hemoglobin variant Hb F-Malta-I in adults.

A variant of fetal hemoglobin (Hb F-Malta-I) has been detected and quantitated in adult blood with a sensitive radioimmunoassay employing monospecific antisera. The concentration of Hb-F-Malta-I was 0.002-0.05%, with an average value of 0.011%. The ratio of Hb F-Malta-I/Hb F in adults was about 4.8%, compared to a ratio of about 27% in the newborn. Since the F-Malta-I variant is a product of a mutated Ggamma locus, which is one of the nonallelic structural genes directing the gamma chain synthesis, its presence in blood of adults shows that the synthesis of this gene is not completely suppressed after birth, as was previously suggested.

Adult

The linkage of Hb Valletta [alpha 2 beta 287(f3)Thr----Pro] and Hb F-Malta-I [alpha 2G gamma 2117(G19)His----Arg] in the Maltese population.

We have identified a new stable abnormal hemoglobin called Hb Valletta, which is characterized by a Thr----Pro substitution at position 87 of the beta chain. This mutation was found to be linked to that of the gamma chain variant Hb F-Malta-I with a His----Arg mutation at position 117 of the G gamma chain. Both variants were detected in the blood samples of 34 Maltese and two Italian newborn babies with isoelectrofocusing and reversed phase high performance liquid chromatography. Similar analyses of cord blood from 388 additional Maltese newborns failed to identify either one of these two variants. Additional analyses of 353 Maltese adults (including 39 beta-thalassemia heterozygotes) resulted in the detection of two adult Hb Valletta heterozygotes. Dot-blot hybridization analyses of amplified DNA with a probe specific for the G gamma-F-Malta-I variant showed that both also carried that mutation. These results show close linkage of the mutant forms of the G gamma- and beta-globin genes, 27-28 kb apart, and a failure to identify chromosomes with either the Hb F-Malta-I mutation alone or with the Hb Valletta mutation alone, indicating a low recombination frequency.

Adult

Alcohol and alcohol problems research. 17. Malta.

This article is an enquiry into the current status of alcohol in Maltese culture. The responses of society to alcoholism depend on the way members of the community perceive the problems incurred by the use and abuse of a dependence producing substance like alcohol. These perceptions and subsequent responses are very much influenced by prevailing attitudes and beliefs. Malta is a melting point of cultures. This factor, together with a high density population and Malta's geopolitical strategic position, combine to make Malta a tolerant society. There is a laissez-faire response to alcoholism, at least partly due to the present inability to identify the need to take appropriate measures. The police force, medical profession and politicians still do not feel the responsibility or the need to provide effective laws and regulations, specialized treatment services or educative programmes on alcohol-related issues. A systematic enquiry is needed urgently to determine the severity and degree of the problems posed by alcohol abuse among the Maltese. Such an enquiry should be followed by a well planned national policy which includes local approaches and interventions. Finally, these interventions must be evaluated frequently and developed to achieve better results in the future.

Alcohol Drinking

Multiple sclerosis in southern Europe. II: Prevalence in Malta in 1978.

After an intensive survey only 14 patients have been found with a diagnosis of probable multiple sclerosis (MS) in the islands of Malta. This is a low prevalence of 4.2 per 100 000. The low prevalence of MS is confirmed by the small number of deaths certified as due to MS--six in 11 years--and by the absence of Maltese MS patients resident in England among the MS patients admitted to hospital in Greater London and the West Midlands (1960-72). The low prevalence of MS found in Malta can be contrasted with the high prevalence found in Enna city in central Sicily. The genetic and environmental reasons for this difference in MS prevalence between the neighbouring islands of Sicily and Malta require further study.

England

Trends in mortality from major non-communicable diseases in the middle-aged population of Malta.

Trends in life expectancy and mortality from major non-communicable diseases in Malta were analyzed from the national vital statistics available. Most of the increased life expectancy during the 20th century in Malta took place between 1930 and 1960 and since then only a minor increase was observed. The peak in age standardized total mortality in men and women aged 40-69 years was during 1974-76. Total mortality in men was about 40% higher than that of women. The proportion of deaths from major non-communicable diseases (cardiovascular diseases, cancer and diabetes) of all deaths increased during 1968-82. In 1983-84 in the age group 45-64 cardiovascular diseases accounted for 54% of deaths in men and 43% in women, cancer 27% and 34%, and diabetes 3% and 11% in men and women, respectively. The international comparison of mortality data showed that mortality from both cardiovascular diseases, cancer and diabetes was clearly higher than in other European Mediterranean countries ranking among the highest in the whole Europe. Public health intervention programmes have initiated in Malta to reduce these high death rates in the future.

Cardiovascular Diseases

HLA associations with multiple sclerosis in Sicily and Malta.

The islands of Sicily and Malta have very different prevalence rates for multiple sclerosis (MS): at least 44 per 100,000 in Sicily and only 4 per 100,000 in Malta. In Northern Europe, MS is associated with HLA-DR2/Dw2. The other components of the commonest DR2-containing haplotype of this region, HLA-A3-B7-DR2-Dw2, also tend to be present at higher frequency in MS patients. HLA Class I and II antigen frequencies and associations in controls and MS patients from Sicily and Malta were studied to discover whether they might account for the difference in MS prevalence. In Sicilian MS patients, DR2 is increased in frequency compared with controls and four out of five DR2-positive patients also type as Dw2. In the Maltese population, DR2 is present at high frequency but approximately half of the DR2 positive individuals do not type as Dw2 so that DR2 is probably most commonly present as part of Class II haplotypes other than those commonly associated with MS. Additional differences in HLA profile of the Sicilian and Maltese populations were found when HLA-A, -B, and B-DR antigen associations were examined. Therefore, some of the difference in MS prevalence might be explained by genetic factors.

Cross-Sectional Studies

Health and social inequities in Malta.

This paper focuses on the sources that are thought to lead to the genesis of health and social inequity in a small island community. It is largely a descriptive exposition, the main aim of which is to introduce the topic in Malta. Small size is the key geographical factor. This fact is dwelt upon to stress that although there are differences, say, between the two inhabitable islands, such differences are very small. Social homogeneity and an almost indiscernible urban-rural difference are strong factors that reduce any tendency for regional inequities. Socio-cultural factors in Malta are largely determined by family life and its relation with the established church. The traditional Catholic way of life is depicted as gradually giving way to a more secular lifestyle. Politics is represented as the prime factor for causing division within the this society. This is not without reason since this factor has taken over the other cause of division, namely parochialism. Political factors determine the distribution of resources including health resources. The important economic factors are that there are no great differences between the high income and the low income groups. However, due importance must always be given to the subterranean economy. The health profile is that of a developed country with low infant mortality and a long life expectancy. Infectious diseases have been on the decline and their persistence is usually linked to areas that have remained underdeveloped. The disadvantaged groups identified are the chronically unemployed, single mothers, possibly a section of the migrant population and the elderly.

Cause of Death

Four bills of health issued from Malta by the Order of St John.

The specimens here described enlarge our knowledge of the bills of health of the Order of St John issued from Malta, an aspect of public health that had hitherto remained unexplored. These lone survivors from a vanished by-way of Maltese medical history formed, in their age, an integral part of the daily social, commercial and sanitary pattern of life in Malta. The earliest met so far, and described in the present series, dates from 1713; two belong to 1766 and another dates from 1794. A further bill, issued in 1782, has been published elsewhere. The chance preservation of these documents in unlikely places, as here indicated, makes it probable that additional ones may eventually come to light.

History, 18th Century

Nutrition policy development and implementation in Malta.

The paper highlights experiences in the development and implementation of the food and nutrition policy in Malta. As one could expect there has been tangible progress in certain areas but less so in others. Some background information on the food and health status of the Maltese population is given first as this was the reason for the establishment of the policy in Malta in the first place.

Female

Diabetes as a coronary risk factor in Malta.

In a retrospective survey of 1,118 admissions for acute ischemic heart disease (AIHD) at St. Luke's Hospital in Malta in 1963-72, there were 945 (84.5%) cases of acute myocardial infarction (AMI) and 173 (15.5%) cases of acute coronary insufficiency (ACI). The proportion of patients with diabetes was 30.2% (30.7% in AMI, and 27.7% in ACI; age-corrected rates at greater than or equal to 40 years). This was significantly higher (P less than 0.01) than the corresponding rate of diabetes (20.2%) in the general population of Malta. There was a significantly greater prevalence of diabetes among women than among men with AIHD: the proportion with diabetes was 50.0% among women with AMI and 41.3 among women with ACI. The diabetes was mostly of the maturity-onset type. The high frequency of AIHD among diabetics seemed to be chiefly attributable to the effects of the diabetic state, either directly or indirectly through its association with other risk factors: obesity, physical inactivity, excessive eating and high plasma cholesterol levels. Diastolic hypertension and chronic bronchitis and emphysema associated withe heavy smoking were no more common in diabetics than in nondiabetics with AMI.

Adult

Low physical activity and worsening of glucose tolerance: results from a 2-year follow-up of a population sample in Malta.

The relationship between the level of habitual physical activity and glucose intolerance was examined cross-sectionally and during a 2-year follow-up among a sample of 388 subjects in Malta. At baseline, the subjects were classified into three categories of physical activity, which was inversely related to the 2-h post challenge blood glucose (P = 0.02). In a multivariate analysis, age (standardized regression coefficient 0.23; P less than 0.001), family history of diabetes (0.20; P less than 0.001), and physical activity (-0.18; P = 0.002) were the strongest predictors of the 2-h blood glucose at baseline. The age standardized 2-year risk of glucose intolerance, i.e. impaired glucose tolerance or diabetes was consistently and inversely related to the level of physical activity. Among subjects with normal glucose tolerance at baseline (n = 127) those with low physical activity had a 2.7 times higher risk of glucose intolerance during follow-up than those with high physical activity (P = 0.1), and even a 3.7-fold risk of glucose intolerance at baseline (n = 196) when both the subjects with normal and impaired glucose tolerance at baseline were considered together (P = 0.005). Similar trends were observed for the risk of diabetes. The suggested protective effect of physical activity was independent of body mass, a family history of diabetes and gender. Within the limits of this small study we conclude that physical activity may have some importance in the primary prevention of impaired glucose tolerance and, possibly, non-insulin-dependent diabetes mellitus.

Adolescent

Kala-azar in a four-year-old child 18 months after brief exposure in Malta.

A four-year-old Danish boy developed kala-azar 18 months after a holiday in Malta. Splenectomy, with liver biopsy, was performed six months after onset of symptoms because of hypersplenism, and the diagnosis of kala-azar was only made four months later, when the histopathological specimens were reviewed. Previous bone marrow biopsies did not show Leishmania. Treatment with sodium stibogluconate was successful. The development of kala-azar after one week's stay in an endemic area stresses the importance of including this potentially fatal disease in the differential diagnosis of cases presenting with fever, splenomegaly, and pancytopenia.

Antimony Sodium Gluconate