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Biomedical subjects

G Dean

Publications and source records attributed to G Dean.

At least 91 records · Page 5Linked to original sources

Porphyria variegata.

A form of porphyria inherited as a Mendelian dominant characteristic can cause both cutaneous lesions on the exposed skin and attacks of acute porphyria following certain drugs, for instance barbiturates. Because it presents in a variety of forms it has been named porphyria variegata. This form of porphyria is very common among the White and Coloured South Africans because of 'founder effect'. A high proportion of the present population are descendants of a small number of early settlers, one of whom introduced the gene for this condition. The disease has been traced over 17 generations to Gerrit Jansz who came from Deventer in Holland and who married Ariaantje, an orphan from Rotterdam, in 1688. In the quiescent state porphyria variegata is best diagnosed by the examination of a solution of the stool in ultraviolet light which shows the pink fluorescence of porphyrin. In the acute attack porphobilinogen and porphyrin are greatly increased in the urine. Porphyria variegata also occurs outside of South Africa but in these countries it is less common than acute intermittent porphyria. The diagnosis and treatment is described and the story that the insanity of King George III of England was due to porphyria variegata is refuted.

Acute Disease↗

First admissions to psychiatric hospitals in south-east England in 1976 among immigrants from Ireland.

Irish male immigrants had twice and female immigrants 1.7 times the expected number of first admissions to psychiatric hospitals in south-east England in 1976 when the expected number was based on the age- and sex-standardised rates of first admission of the population born in the United Kingdom living in the region. Admissions for alcoholism and alcoholic psychosis were five times higher in men and four times higher in women, and for schizophrenia 2.4 times as high in both sexes, as expected from these rates. When the expected number of first admissions was based on the age- and sex-standardised rates among Irish people living in Ireland the immigrants had fewer admissions than expected. Among migrants from Northern Ireland the number of first admissions was significantly greater than expected. Admissions for alcoholism and alcoholic psychosis in men and women combined were seven times the expected number based on the UK rates. There was no significant difference in the proportion of single people between the Irish immigrants and people born in the UK. Marital state, socioeconomic group, and occupation may partly account for the high number of admissions for alcoholism and schizophrenia among Irish people living in south-east England.

Adolescent↗

The prevalence of multiple sclerosis in the Outer Hebrides compared with north-east Scotland and the Orkney and Shetland Islands.

Multiple sclerosis has been reported to have a high prevalence in the Orkney and Shetland Islands and in Caithness in comparison with the highlands of Scotland and the Outer Hebrides-the Western Isles. For this reason a survey was undertaken in the Outer Hebrides and 25 probable and 30 probable and possible patients with multiple sclerosis were found. This is an increase from eight and 11 respectively found in 1954. The present prevalence rate of 97.3 per 100 000 for probable and possible multiple sclerosis is not significantly different from that found in a recent study in the Grampian region in north-east Scotland. Repeated studies in small populations generally show increasing prevalence of multiple sclerosis because some patients are missed in the earlier studies, and over a long period of time there may also be some increase in survival time. This increase has been found in the Orkney and Shetland Islands, in north-east Scotland, and also in the Outer Hebrides.

Adult↗

The prevalence of multiple sclerosis in Sicily. I: Monreale city.

The prevalence of probable multiple sclerosis in Monreale city, close to the university city of Palermo, Sicily, is at least 43 per 100 000. If the possible multiple sclerosis patient is included, it is 47 per 100 000. This prevalence is not significantly different from that found in Enna city, 53 per 100 000. The prevalence in Sicily and, no doubt, in Italy has, in the past, been seriously underestimated. This confirms the need for thorough studies of small populations if many patients are not to be overlooked in a prevalence survey.

Adult↗

The prevalence of multiple sclerosis in Sicily. II: Agrigento city.

The prevalence of probable multiple sclerosis in Agrigento city on the south-west coast of Sicily is at least 2 per 100 000. This is likely to be a considerable underestimate of the true prevalence because the study presented particular difficulties in that the city is far from the neurological centres of Palermo. Catania, and Messina. There is no neurological department at either the general or the psychiatric hospital in Agrigento and there was a low awareness of the disease among the doctors in the city. Most of the patients were diagnosed in other centres. Agrigento is a good example of the difficulties of studying multiple sclerosis in a rural city which has no special interest in neurological problems and is far from a neurological centre. Studies in such centres must be pursued with great enthusiasm and over a long period of time, and all available sources of information in the city, medical and lay, and in other cities, must be utilised if a high proportion of the patients is to be found.

Adult↗

First admissions of native-born and immigrants to psychiatric hospitals in South-East England 1976.

In the past, birthplace has frequently been omitted in completing the Sheet, but in 1976, over 91 per cent of all first admissions to psychiatric hospitals in South-East England were analysed by birthplace, sex, age-groups and marital status. First admissions for schizophrenia were five times the expected number for immigrants from new Commonwealth America (the West Indies), four times the expected number for immigrants from New Commonwealth Africa (mostly ethnic Asians) and three times the expected number from India. Immigrants from Pakistan and the remaining New Commonwealth Asian countries did not show a significantly higher than expected number of admissions for schizophrenia, and their first admissions for alcoholic psychosis and alcoholism, psychoneuroses and personality and behaviour disorders were significantly fewer than expected. First admissions for schizophrenia were also significantly more than expected among immigrants from Ireland, Germany and Poland, but not from italy.

Adult↗

Comparison of 2',3'-cyclic nucleotide 3'-phosphodiesterase and the major component of Wolfgram protein W1.

Wolfgram protein preparations from myelin are heterogeneous mixtures having major components with molecular weights in the 43,000 to 60,000 range. Through the use of an SDS-slab gel electrophoretic system of high resolving power, the Wolfgram 1 (W1) component described by Nussbaum et al. (1977) has been resolved into two components; bovine W1a and W1b proteins are found to have mobilities identical to those of the two 2',3'-cyclic nucleotide 3'-phosphodiesterase (2'3'-CN 3'-ase) components, CNa and CNb (Drummond, 1979). Crossed immunoelectrophoresis with bovine 2',3'-CN 3'-ase demonstrates that CNa and CNb are antigenically indistinguishable. Rat and bovine Wolfgram proteins W1a and W1b, but not the W2 proteins, display cross reactivity with antiserum directed against bovine 2',3'-CN 3'-ase. Chymotryptic and Staphylococcus aureus V8 protease peptide profiles indicate structural similarities between proteins CNa-W1a and CNb-W1b. It is concluded that an inactivated form of 2',3'-Cn 3'-ase is a major component of Wolfgram protein W1. These results are consistent with the studies on the histochemical localization of rat W1 protein by Roussel et al. (1977, 1978).

2',3'-Cyclic-Nucleotide Phosphodiesterases↗

Complementation analysis and characterization of the nitrogen fixation genes, nifH, nifC and nifJ in Klebsiella pneumoniae.

Complementation tests were performed with various nif mutations for delineating the nif genes. The nif-/nif- heterogenotes were constructed by transferring nif plasmid mutant PRD-nif- from E. coli Jc5466 to various Klebsiella pneumoniae nif- recipients. In addition to those nif genes previously reported elsewhere, a new essential gene for nitrogen fixation, nifC was identified. According to the P1-transduction and three factor reciprocal crosses, nifC was tentatively mapped between nifH and nifJ in the chromsome. The order of nif genes obtained was hisD, nifQ, nifB, nifA, nifL, nifF nifM, nifV, nifS, nifU, nifN, nifE, nifK, nifD, nifH, nifC and nifJ. The examination of the biochemical phenotypes of the nif genes suggests that nifC may be concerned with the synthesis or activation of the iron molybdenum cofactor nitrogenase, nifH, besides coding for the structure of nitrogen reductase may also exert a function on the synthesis of nitrogenase, and nifJ presumably is required to turn on the expression of nifK (D), nifH or nifF.

Chromosome Mapping↗

Causes of death of blue-collar workers at a Dublin brewery, 1954--73.

The suggested association between high consumption of beer and an increased risk of death from cancer of the colon and rectum was investigated among blue-collar workers at a Dublin brewery, who consume more than average amounts of beer, usually in the form of stout. A study of their mortality between 1954 and 1973 showed that they had as good an expectation of life as all Dublin males, with no increased risk of death from cancer of the oesophagus, pharynx, liver or of cirrhosis of the liver, accidents or suicide, conditions normally associated with the high consumption of alcohol. They had significantly increased risk of death from cancer of the rectum and also from diabetes mellitus. Twenty per cent of the workers, differentiated by their place of work within the brewery, had a much higher risk of death from cancer of the rectum.

Adolescent↗

Multiple sclerosis in southern Europe. I: Prevalence in Sicily in 1975.

Previous reports on large population groups have suggested that the prevalence of multiple sclerosis (MS) in Sicily and southern Italy is low, of the order of 4-8 per 100 000. In contrast, immigrants from Italy resident in Greater London, many of whom are from southern Italy and Sicily, had a hospitalised MS prevalence similar to that found among people born in the United Kingdom (1960-72). The present study shows that in Enna city (population 29 000) in central Sicily, the prevalence of probable MS was 53 per 100 000, which is of the same order of magnitude as has been reported from the United Kingdom and northern Europe. The high prevalence of MS found in Enna city may be due, at least in part, to the fact that the population studies was small. Because Enna is on high ground, similar studies are being undertaken in two small coastal towns of Sicily.

Adult↗

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↗