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PRENATAL DIAGNOSIS OF JUNCTIONAL EPIDERMOLYSIS BULLOSA
1453 PRENATAL DIAGNOSIS OF JUNCTIONAL EPIDERMOLYSIS BULLOSA with Dr Hausser and colleagues (Oct 28, methods are unreliable that immunohistochemical who say pl035), for the prenatal diagnosis of the Herlitz form of junctional epidermolysis bullosa (EB). Since our early experience with monoclonal antibodies for the rapid prenatal and postnatal diagnosis of two of the severest forms of EB,1-3including junctional EB (Herlitz), we have examined fetal skin samples from a total of twenty pregnancies at risk for this disease, by indirect immunofluorescence (IF) together with transmission electron microscopy (TEM). Seven pregnancies were affected, and IF with monoclonal antibody GB3, which recognises a newly described component of basement membrane,’ gave in every case unequivocal results that were in accord with those obtained by TEM.s With reference to Hausser and colleagues’ suggestion that where fetal skin is unavailable amnion may be used for prenatal diagnosis, we found a similar abnormality of GB3 staining in fetal skin and amnion acquired from six affected pregnancies after termination6 indicating a common biochemical abnormality of basement membrane in both these tissues. However, in our series of almost 50 cases in which fetal skin biopsies were done for prenatal diagnosis or exclusion of different forms of EB, we have always obtained adequate skin samples. We are concerned about the safety of taking samples of amnion instead of fetal skin. We suggest that the use of appropriate monoclonal antibody probes is not only reliable for prenatal diagnosis of the Herlitz form of junctional EB, but also that this method is desirable, since it provides a valuable back-up to TEM. The method may even be preferable where the epidermis becomes detached from the dermis in fetal skin samples, a common artifact in affected pregnancies, and where critical analysis of hemidesmosomes by TEM7 is impossible.
TABLE I-AVERAGE DAILY CONSUMPTION OF BEER, WINE, AND SPIRITS IN 65 SURVEY COUNTIES
SIR,-We disagree
Department of Cell Pathology, Institute of Dermatology, UMDS, St Thomas’s Campus,
R. A. J. EADY O. M. V. SCHOFIELD
London SE1 7EH Harris Birthright Research Centre for Fetal Medicine, King’s College Medical and Dental London SE5
Schools,
K. H. NICOLAIDES
C. H. RODECK
Heagerty AHM, Kennedy AR, Gunner DB, Eady RAJ. Rapid prenatal diagnosis and exclusion of epidermolysis bullosa using novel antibody probes. I Invest Dermatol 1986; 86: 603-05. 2. Heagerty AHM, Kennedy AR, Eady RAJ, et al. GB3 monoclonal antibody for diagnosis of junctional epidermolysis bullosa. Lancet 1986; i: 860. 3. Heagerty AHM, Eady RAJ, Kennedy AR, et al. Rapid prenatal diagnosis of epidermolysis bullosa letalis using GB3 monoclonal antibody Br J Dermatol 1987; 1.
117: 271-75. 4. Verrando P, Pisani A, Ortonne J-P. The new basement membrane antigen recognised by the monoclonal antibody GB3 is a large size glycoprotein: modulation of its expression by retinoic acid. Biochim Biophys Acta 1988; 942: 45-56. 5. Rodeck CH, Eady RAJ, Gosden CM. Prenatal diagnosis of epidemiolysis bullosa letalis. Lancet 1980; i: 949-52.
7.
Eady RAJ, Heagerty AHM, Kennedy AR, Leigh IM, Hsi B-L, Ortonne J-P. Abnormal expression of basement membrane related antigens in amnion and skin from fetuses with epidermolysis bullosa. J Invest Dermatol 1986, 87: 137. Tidman MJ, Eady RAJ. Hemidesmosome heterogeneity in junctional epidermolysis bullosa revealed by morphometric analysis. J Invest Dermatol 1986; 56: 51-56.
CIRRHOSIS OF THE LIVER AND MOULDY BREAD
SIR,-We are doing a comprehensive epidemiological studyl of the relation between dietary and other lifestyle characteristics and infectious and chronic degenerative diseases in China. In this we examined mortality rates from cirrhosis of the liver in 49 counties. In China, cirrhosis accounts for 4-6% of all non-cancer deaths in the age group 0-64. Contrary to expectation, mortality rates from liver cirrhosis were not higher in counties where alcohol consumption was greater. There was no correlation between mortality from cirrhosis and either the quantity of spirits drunk (assessed from a food consumption survey) or the quantity or frequency of beer, wine, spirit consumption assessed by consumption of alcohol was low (table i). or
as mean
(and range).
TABLE II—CORRELATION BETWEEN DIETARY AND NON-DIETARY
FACTORS AND MORTALITY RATE FROM LIVER CIRRHOSIS
(0-64
YEARS)
*Calculated from analysed food samples and adjusted for quantities consumed determined by weighed dietary survey.
as
A highly significant correlation (Pearson product-moment correlation) was found between county mortality (0-64) from cirrhosis and the percentage of respondents who consumed sticky or The mouldy steambread or pancakes (r=0’37, p<0-01). correlation was even more significant with the frequency of consumption in the survey households of sticky or mouldy steambread or pancakes (r 0-51, p < 0-001 ) (table 11). There was no =
Institute of Obstetrics and Gynaecology, Queen Charlotte’s Maternity Hospital, London W6
6.
Results
as
questionnaire. Average
correlation with the percentage of households who make steambread or pancakes daily, only with their consumption when sticky or mouldy. Of all the many statistical associations investigated between cirrhosis and dietary and environmental characteristics the association with mouldy steambread was the strongest. Fermented steamed bread is an important staple consumed all over China, especially in the northern half of the country. This bread is prepared using a ball of dough from the previous batch, often mouldy, as leaven for the new dough. Bread is often prepared in large quantities and consumed over several weeks, even if slightly mouldy. In rural areas each family has its own yeast, which has usually been passed from generation to generation among relatives or neighbours. Toxicity of some of those yeasts and poisoning cases have been reported, much more for corn bread than for wheat bread. 256 groups of men and women were interviewed in 65 counties. Among these counties only 27 reported consuming mouldy com and for them there was significant (r = 0-36, p < 0-01) correlation between the frequency of consumption of mouldy corn and mortality from liver cancer. Those intoxications do not occur in cities, where bread is fermented with industrially produced yeast or with baking soda, but in remote rural areas efforts by health authorities to promote those fermentation agents have not been fully successful. Very low intake levels of fat and animal products were also associated with a higher mortality rate from cirrhosis (table 11). However, these dietary characteristics may merely reflect rural poverty. The high correlation of mortality from liver cirrhosis with the mercury content of the diet (r 0-47, p < 001), and to a lesser extent with the lead content (r = 0-34, p < 005), is of interest. About 6500 individuals were also tested for plasma hepatitis B surface antigen in 130 villages. A significant correlation (r 0-30, p < 0-05) was found between the percentage of the villagers with a positive response to the county mortality rate from liver cirrhosis.1 =
=
TABLE I-AVERAGE DAILY CONSUMPTION OF BEER, WINE, AND SPIRITS IN 65 SURVEY COUNTIES
SIR,-We disagree
Department of Cell Pathology, Institute of Dermatology, UMDS, St Thomas’s Campus,
R. A. J. EADY O. M. V. SCHOFIELD
London SE1 7EH Harris Birthright Research Centre for Fetal Medicine, King’s College Medical and Dental London SE5
Schools,
K. H. NICOLAIDES
C. H. RODECK
Heagerty AHM, Kennedy AR, Gunner DB, Eady RAJ. Rapid prenatal diagnosis and exclusion of epidermolysis bullosa using novel antibody probes. I Invest Dermatol 1986; 86: 603-05. 2. Heagerty AHM, Kennedy AR, Eady RAJ, et al. GB3 monoclonal antibody for diagnosis of junctional epidermolysis bullosa. Lancet 1986; i: 860. 3. Heagerty AHM, Eady RAJ, Kennedy AR, et al. Rapid prenatal diagnosis of epidermolysis bullosa letalis using GB3 monoclonal antibody Br J Dermatol 1987; 1.
117: 271-75. 4. Verrando P, Pisani A, Ortonne J-P. The new basement membrane antigen recognised by the monoclonal antibody GB3 is a large size glycoprotein: modulation of its expression by retinoic acid. Biochim Biophys Acta 1988; 942: 45-56. 5. Rodeck CH, Eady RAJ, Gosden CM. Prenatal diagnosis of epidemiolysis bullosa letalis. Lancet 1980; i: 949-52.
7.
Eady RAJ, Heagerty AHM, Kennedy AR, Leigh IM, Hsi B-L, Ortonne J-P. Abnormal expression of basement membrane related antigens in amnion and skin from fetuses with epidermolysis bullosa. J Invest Dermatol 1986, 87: 137. Tidman MJ, Eady RAJ. Hemidesmosome heterogeneity in junctional epidermolysis bullosa revealed by morphometric analysis. J Invest Dermatol 1986; 56: 51-56.
CIRRHOSIS OF THE LIVER AND MOULDY BREAD
SIR,-We are doing a comprehensive epidemiological studyl of the relation between dietary and other lifestyle characteristics and infectious and chronic degenerative diseases in China. In this we examined mortality rates from cirrhosis of the liver in 49 counties. In China, cirrhosis accounts for 4-6% of all non-cancer deaths in the age group 0-64. Contrary to expectation, mortality rates from liver cirrhosis were not higher in counties where alcohol consumption was greater. There was no correlation between mortality from cirrhosis and either the quantity of spirits drunk (assessed from a food consumption survey) or the quantity or frequency of beer, wine, spirit consumption assessed by consumption of alcohol was low (table i). or
as mean
(and range).
TABLE II—CORRELATION BETWEEN DIETARY AND NON-DIETARY
FACTORS AND MORTALITY RATE FROM LIVER CIRRHOSIS
(0-64
YEARS)
*Calculated from analysed food samples and adjusted for quantities consumed determined by weighed dietary survey.
as
A highly significant correlation (Pearson product-moment correlation) was found between county mortality (0-64) from cirrhosis and the percentage of respondents who consumed sticky or The mouldy steambread or pancakes (r=0’37, p<0-01). correlation was even more significant with the frequency of consumption in the survey households of sticky or mouldy steambread or pancakes (r 0-51, p < 0-001 ) (table 11). There was no =
Institute of Obstetrics and Gynaecology, Queen Charlotte’s Maternity Hospital, London W6
6.
Results
as
questionnaire. Average
correlation with the percentage of households who make steambread or pancakes daily, only with their consumption when sticky or mouldy. Of all the many statistical associations investigated between cirrhosis and dietary and environmental characteristics the association with mouldy steambread was the strongest. Fermented steamed bread is an important staple consumed all over China, especially in the northern half of the country. This bread is prepared using a ball of dough from the previous batch, often mouldy, as leaven for the new dough. Bread is often prepared in large quantities and consumed over several weeks, even if slightly mouldy. In rural areas each family has its own yeast, which has usually been passed from generation to generation among relatives or neighbours. Toxicity of some of those yeasts and poisoning cases have been reported, much more for corn bread than for wheat bread. 256 groups of men and women were interviewed in 65 counties. Among these counties only 27 reported consuming mouldy com and for them there was significant (r = 0-36, p < 0-01) correlation between the frequency of consumption of mouldy corn and mortality from liver cancer. Those intoxications do not occur in cities, where bread is fermented with industrially produced yeast or with baking soda, but in remote rural areas efforts by health authorities to promote those fermentation agents have not been fully successful. Very low intake levels of fat and animal products were also associated with a higher mortality rate from cirrhosis (table 11). However, these dietary characteristics may merely reflect rural poverty. The high correlation of mortality from liver cirrhosis with the mercury content of the diet (r 0-47, p < 001), and to a lesser extent with the lead content (r = 0-34, p < 005), is of interest. About 6500 individuals were also tested for plasma hepatitis B surface antigen in 130 villages. A significant correlation (r 0-30, p < 0-05) was found between the percentage of the villagers with a positive response to the county mortality rate from liver cirrhosis.1 =
=