1360
Staging of breast cancer SIR,-Mr Barr and Professor Baum’s perspective (April 11, p 915) on the TNM classification of breast cancer is premature. We agree that clinical TNM staging of breast cancer offers limited pragmatic value in prognosis or treatment planning but emphasises the necessity for full pathological staging, which (contrary to North America) is not standard practice in the UK.’ The pathological information that has become available since TNM criteria were drawn up has allowed centres throughout the world to systematically generate data that have revealed retrospectively the value of features proposed in the operational classification scheme. The importance of systematic data gathering, promoted in 1968 by the International Union Against Cancer (UICC), has led in part to our insight into breast cancer as a biological rather than an anatomical problem. Tumour grade and hormone receptor status have also
contributed to an understanding of risk categories in node-negative are complementary to other gross anatomical information. The UICC (1987) classification recognised the importance of "minimal breast cancer". Nevertheless, pathological axillary node status remains the most reliable indicator in determining the most efficient application of systemic therapy to those most likely to benefit.2 An ever growing list of putative prognostic markers in breast cancer seems to have lately overwhelmed us. Although these markers might help to elucidate the biological heterogeneity of this disease, the precise role these will have in determining therapy for the problematic node-negative patients remains to be defmed.3 The adverse risk-to-benefit ratio will increase as further subsets with a lesser probability of gain from adjuvant treatment are specified. We are progressing in our understanding of clinical heterogeneity in early breast cancer. We agree that TNM notions may eventually become a historical curiosity. Until that time when our basic science framework allows us to formulate more precisely (algorithmically or otherwise) why anatomically similar patients behave differently, it is important to continue to gather anatomical data precisely and systematically. Treatment decisions can continue to be made by critical use of variables, both anatomical and otherwise, with an independent record of their worth. The institution of new official classification schemes is premature at a time when basic understanding of this disease is steadily evolving. Working propositions as suggested are useful as guides to the use of well-established indices, and in this spirit they are welcomed.
patients and
Departments of Medical and Radiation Oncology, Cancer Treatment and Research Foundation of Nova Scotia and Dalhousie University, Halifax, Nova Scotia, Canada B3H 1V7
in breast cancer incidence by stage in the years before screening and during the first screening round. Although data for stage of disease come from many sources and include both clinical and pathological staging, it is still, in East Anglia, a powerful predictor of survival (unpublished results). Systematic breast screening was introduced in East Anglia in April, 1989, aimed initially at women aged 60-64. By the end of 1990 the first round of screening for this age-group was virtually complete, with over 75% uptake. Only a small proportion of the 50-59 age-group had been screened by the end of 1990. Stagespecific and total annual incidence rates of breast cancer were stable before screening (at 200-250 per 100 000) but increased strikingly (to about 450 per 100 000), especially stage 1 tumours (from about 60 to 240 per 100 000), for the age-group invited to screening (60-64) and did not change in the 50-59 age-group. If breast screening is to reduce population mortality rates, then an increase in cancers with a good prognosis followed by a decrease in advanced cancers needs to be demonstrated. Our results show that the introduction of routine breast screening can, on a regional basis, lead to a major increase in the number of early-stage cancers, and bode well for a future reduction in the rates of advanced cancer and of breast cancer mortality. Department of Community Medicine and MRC Biostatistics Unit, Institute of Public Health, Cambridge CB2 2BW, UK
Overseas
D. BRANDENBURG N. E. DAY D. R. R. WILLIAMS E. M. KINGSLEY-PILLERS
training and doctors in developing countries
SiR,—Ifully agree with Dr Patel and Dr Araya’sview (Jan 11, 110). The "political tit-for-tat action" of developing countries is suicidal. I was advised at a job interview in a teaching hospital in India that my MRCP (UK) is no longer recognised. I was forced to
p
stay in private practice, which is a "rat-race" in most developing countries. This forced me to emigrate after two years. There are many expatriates who will re-invest their time in teaching hospitals in India, if their qualifications are "re-recognised". John Hunter Hospital, New Lambton, NSW 2305, Australia
EC
B. R. NAIR
proposal for in-vitro diagnostic medical devices Directive
SIR,-lnote me
PEDRO LOPEZ STEPHEN SAGAR
1. Leslie MD, Maher EJ. Node negative breast cancer. Br Med J 1990; 300: 749. 2. Fentiman IS, Mansel RE. The axilla: not a no-go zone. Lancet 1991; 337: 221-23. 3. Osbome CK. Prognostic factors for breast cancer: have they met their promise? J Clin Oncol 1992; 10: 679-82.
SIR,-Mr Barr and Professor Baum propose that the UICC TNM system of breast cancer classification is of little use, and should be replaced by a simpler operational scheme based on a clinical assessment of appropriate therapy. There is, however, a wider context that should be considered. Survival after diagnosis is largely determined by the prognostic profile of the disease, in respect of size, nodal involvement, malignancy grade, and other measures. The national breast screening programme needs to be evaluated, on the basis of simple, reproducible data about prognosis for most of the cancers arising in the target population, so that comparison can be made over time, or between populations. Stage has proved reasonably robust in this respect. The information is often available on a routine basis from cancer registries, unlike data for most other proposed prognostic factors, and stage is strongly predictive of survival. At the East Anglian Cancer Registry one of us (E. M. K-P.) has staged nearly all registered breast cancers since 1976; consequently, the proportion of unstaged breast cancers in the 50-64 age-group is only 2 % since then. We have therefore been able to compare trends
concerns
expressea by
Lr
jencoate (Nov Y,
1212) and Dr Ekins (Feb 22, p 499) that there should be some form of regulatory control of assay kits. Your readers may be interested to know that a series of three European Community (EC) Directives regulating the safety and marketing of all medical devices begin to come into effect from 1993. The Commission of the EC has just started drafting the third of p
these Directives which will cover in-vitro diagnostic medical devices (IVDs), and it is expected that it will issue a proposal later this year to take effect by 1996. A separate Directive is proposed for IVDs because they do not come into direct contact with patients and therefore the safety issues are different from those for other medical devices. Their accuracy, specificity, and reliability is of primary concern.
The Directive will contain a definition of an IVD device, which is expected to be along the following lines: "any medical device which is a reagent, reagent product, kit, instrument, apparatus or system whether used alone or in combination, intended by the manufacturer to be used in vitro solely or principally for the examination of substances derived from the human body for the purpose of providing information relevant to the detection, diagnosis, monitoring or treatment of physiological states, states of health or disease or congenital abnormality". The Directive is expected to contain essential requirements on safety and performance and to divide IVDs into two classes. Most devices will fall into class I (low risk), for which it is expected that the manufacturer’s self-declaration of conformity with the requirements of the Directive will suffice. Those whose
1361
performance is crucial in respect of patient health and safety, will fall into class II and will require outside approval by an independent certification body. Class II will possibly include kits used for HIV testing and other blood donation screening tests (eg, hepatitis B
and C).
proposed Directive will not prevent from restricting the availability of particular IVDs. This is especially relevant because regulations came into force in the UK on April 1, 1992, under the Health and Medicines Act Section 23(1) and (3), (SI 1992 no 460: The HIV Testing Kits and Service Regulations, 1992). These regulations control the use, sale, supply, and advertising of HIV testing kits and testing services, and ban the sale or supply of HIV testing kits to members of the public. The provision of testing services is now an offence unless they are provided by or in accordance with the directions of a registered medical practitioner. It is
expected
that the
individual member
states
European Section, Medical Devices Directorate, Department of Health, London WC1B 5EP, UK
of mind and level of confidence, with a minimum of diffidence and hesitation and absence of any impression of panic. Such qualities may be to some extent gender dependent in favour of the male psychological constitution. There exists a subgroup of women who no doubt have these qualities and who are more likely than others to succeed in surgery. However, it is conceivable that because of innate gender differences there will tend to be fewer female surgeons overall. Indeed Mr Cobb’s point in respect of women having a better appreciation of the hazards of a career in surgery may be but a further manifestation of such psychological gender differences. A more cautious approach to life and perhaps surgery may be an attribute of the female of the species. 26 Millway Close,
Upper Wolvercote,
Clinical RICHARD GUTOWSKI
J. R. BENSON
Oxford OX2 8BL, UK
training and research in the EC
SIR,-Professor Gill (May 16, p 1216) draws attention to the to harmonise clinical training in the European Community (EC). There is a similar need to harmonise research training, not least because of the increasing requirement of a research degree to succeed in hospital medicine.1,2 The great variation in regulations
need
Nursing’s identity crisis SIR,-Malcolm Dean (May 9, p 1160) seems to have become entangled in current nursing issues. As he says, no consensus exists within the nursing profession about the future role somewhat
of the nurse, and some do indeed wish nurses to take on certain tasks previously within the province of doctors. There may be, as Dean suggests, some nurses who fear that in taking on quasi-medical tasks they risk pricing themselves out of a job. For this reason they favour retaining basic nursing tasks along with those delegated by doctors. But there is also a growing contingent of nurses--generally including those who practise primary nursing-who see the knowledge needed for nursing and medicine as being quite distinct. In their view routine tasks associated with patient care represent opportunities for nurses to relate to their patients and to use specific nursing skills and knowledge. Such tasks are therefore not regarded as unskilled but an integral part of the development of a therapeutic relationship between nurse and patient. Nurses who have adopted this view, far from aping medicine, are struggling to gain recognition for the value of nursing, albeit within a culture that awards privilege to medicine.
JAN SAVAGE
Surgical careers and female students SIR,-Iread with interest reports from Oxford University Medical School of sexual discrimination in respect of surgical careers for women (April 18, p 994; May 16, p 1235). As a graduate of that institution I am reluctant to support the suggestion that women are (unjustifiably) discriminated against and discouraged from pursuing a career in general surgery. Should this impression be gleaned by certain persons, then perhaps they should consider why this might be. We live in a world where a policy of sexual equality, especially at work, prevails. However, in reality the sexes differ not only biologically, but also in less tangible, more subtle ways in respect of psychological make-up (personality, attitude, temperament, emotional reaction). Such differences may become apparent and assume importance in certain occupations, of which surgery is
perhaps an example. Apart from the long and unpredictable hours of work that inevitably involve sleep deprivation, surgeons also sometimes have to operate (often on long and difficult cases) when very fatigued. In my experience female house surgeons are not as tolerant of sleep
deprivation
and
prone to succumb to exhaustion and their male counterparts. Indeed, recent reviews of junior doctors working hours may be a reflection of pressure for change imposed by much increased numbers of women in medicine as a whole. Some aspects of surgery-for example, procedures for emergency thoracoabdominal trauma--demand a certain attitude are more
absenteeism than
are
Royal Free Hospital School of Medicine, University of London,
L. H. BREIMER D. P. MIKHAILIDIS
London NW3 2PF, UK 1.
Johnson R. Requirements of British universities for higher medical degrees. Br Med J 1991; 302: 397-99.
2. Cobb RA. Surgical careers and female students. Lancet 1992; 339: 1235. 3. Breimer LH, Mikhailidis DP. A thesis for all seasons. Nature 1991; 353: 789-90.
Prenatal
Bloomsbury and Islington College of Nursing and Midwifery, John Astor House, London W1P 8AN, UK
between the current systems makes this impossible. Even within the UK the regulations vary.’1 We have suggested that a unified EC doctorate could be based on work published in internationally refereed journals.3 The thesis itself, which would be comparatively short, can be published in a special European (international) thesis journal. Such an approach would be flexible and allow interaction with countries at present outside the EC, such as Sweden or Japan, where publication-based systems already operate.
diagnosis of haemoglobinopathies in Sicily
SIR,-Dr Giambelluca and colleagues (Jan 18, p 179), discussing the thalassaemia strategy in Sicily, state that although there was a fall in incidence of Cooley’s anaemia births from 1982 to 1989 because of the prenatal diagnosis programme at the V. Cervello Hospital, that improvement has stabilised "due to a gap in health information". We think that to explain these data it would be useful to emphasise other factors too-the true frequency of &bgr;-thalassaemia and sickle-cell carriers and the possibility of a heterogeneous distribution; the efficacy of screening by peripheral centres; and the total number of prenatal diagnoses per year. These haemoglobinopathies are heterogeneously spread in Sicily. Data from the regional health department (personal communication) show a higher prevalence in the east of the island, the two areas having roughly the same populations: Area East West
Cases of Cooley’s anaemia (1980-88) 80 81 82 83 84 85 86 87 88 34 17
46 10
29 15
24 9
27 8
15 9
14 8
14 8
11 1
These data show that after the start of prenatal diagnosis in 1983 the frequency in eastern Sicily remained higher than in the west of the island. In western Sicily and in the interior the frequency is certainly lower than that recorded by Giambelluca et al, and it is difficult to get a reliable estimate of carrier frequency because the screening programme is not centralised and depends on the organisation of local thalassaemia centres. The number of people tested at thalassaemia peripheral centres did increase between 1983 (26 978) and 1990 (43 399) but not all centres screen only young couples, and this may reduce the efficacy of the programme, especially where the carrier prevalence rate is
Staging of breast cancer SIR,-Mr Barr and Professor Baum’s perspective (April 11, p 915) on the TNM classification of breast cancer is premature. We agree that clinical TNM staging of breast cancer offers limited pragmatic value in prognosis or treatment planning but emphasises the necessity for full pathological staging, which (contrary to North America) is not standard practice in the UK.’ The pathological information that has become available since TNM criteria were drawn up has allowed centres throughout the world to systematically generate data that have revealed retrospectively the value of features proposed in the operational classification scheme. The importance of systematic data gathering, promoted in 1968 by the International Union Against Cancer (UICC), has led in part to our insight into breast cancer as a biological rather than an anatomical problem. Tumour grade and hormone receptor status have also
contributed to an understanding of risk categories in node-negative are complementary to other gross anatomical information. The UICC (1987) classification recognised the importance of "minimal breast cancer". Nevertheless, pathological axillary node status remains the most reliable indicator in determining the most efficient application of systemic therapy to those most likely to benefit.2 An ever growing list of putative prognostic markers in breast cancer seems to have lately overwhelmed us. Although these markers might help to elucidate the biological heterogeneity of this disease, the precise role these will have in determining therapy for the problematic node-negative patients remains to be defmed.3 The adverse risk-to-benefit ratio will increase as further subsets with a lesser probability of gain from adjuvant treatment are specified. We are progressing in our understanding of clinical heterogeneity in early breast cancer. We agree that TNM notions may eventually become a historical curiosity. Until that time when our basic science framework allows us to formulate more precisely (algorithmically or otherwise) why anatomically similar patients behave differently, it is important to continue to gather anatomical data precisely and systematically. Treatment decisions can continue to be made by critical use of variables, both anatomical and otherwise, with an independent record of their worth. The institution of new official classification schemes is premature at a time when basic understanding of this disease is steadily evolving. Working propositions as suggested are useful as guides to the use of well-established indices, and in this spirit they are welcomed.
patients and
Departments of Medical and Radiation Oncology, Cancer Treatment and Research Foundation of Nova Scotia and Dalhousie University, Halifax, Nova Scotia, Canada B3H 1V7
in breast cancer incidence by stage in the years before screening and during the first screening round. Although data for stage of disease come from many sources and include both clinical and pathological staging, it is still, in East Anglia, a powerful predictor of survival (unpublished results). Systematic breast screening was introduced in East Anglia in April, 1989, aimed initially at women aged 60-64. By the end of 1990 the first round of screening for this age-group was virtually complete, with over 75% uptake. Only a small proportion of the 50-59 age-group had been screened by the end of 1990. Stagespecific and total annual incidence rates of breast cancer were stable before screening (at 200-250 per 100 000) but increased strikingly (to about 450 per 100 000), especially stage 1 tumours (from about 60 to 240 per 100 000), for the age-group invited to screening (60-64) and did not change in the 50-59 age-group. If breast screening is to reduce population mortality rates, then an increase in cancers with a good prognosis followed by a decrease in advanced cancers needs to be demonstrated. Our results show that the introduction of routine breast screening can, on a regional basis, lead to a major increase in the number of early-stage cancers, and bode well for a future reduction in the rates of advanced cancer and of breast cancer mortality. Department of Community Medicine and MRC Biostatistics Unit, Institute of Public Health, Cambridge CB2 2BW, UK
Overseas
D. BRANDENBURG N. E. DAY D. R. R. WILLIAMS E. M. KINGSLEY-PILLERS
training and doctors in developing countries
SiR,—Ifully agree with Dr Patel and Dr Araya’sview (Jan 11, 110). The "political tit-for-tat action" of developing countries is suicidal. I was advised at a job interview in a teaching hospital in India that my MRCP (UK) is no longer recognised. I was forced to
p
stay in private practice, which is a "rat-race" in most developing countries. This forced me to emigrate after two years. There are many expatriates who will re-invest their time in teaching hospitals in India, if their qualifications are "re-recognised". John Hunter Hospital, New Lambton, NSW 2305, Australia
EC
B. R. NAIR
proposal for in-vitro diagnostic medical devices Directive
SIR,-lnote me
PEDRO LOPEZ STEPHEN SAGAR
1. Leslie MD, Maher EJ. Node negative breast cancer. Br Med J 1990; 300: 749. 2. Fentiman IS, Mansel RE. The axilla: not a no-go zone. Lancet 1991; 337: 221-23. 3. Osbome CK. Prognostic factors for breast cancer: have they met their promise? J Clin Oncol 1992; 10: 679-82.
SIR,-Mr Barr and Professor Baum propose that the UICC TNM system of breast cancer classification is of little use, and should be replaced by a simpler operational scheme based on a clinical assessment of appropriate therapy. There is, however, a wider context that should be considered. Survival after diagnosis is largely determined by the prognostic profile of the disease, in respect of size, nodal involvement, malignancy grade, and other measures. The national breast screening programme needs to be evaluated, on the basis of simple, reproducible data about prognosis for most of the cancers arising in the target population, so that comparison can be made over time, or between populations. Stage has proved reasonably robust in this respect. The information is often available on a routine basis from cancer registries, unlike data for most other proposed prognostic factors, and stage is strongly predictive of survival. At the East Anglian Cancer Registry one of us (E. M. K-P.) has staged nearly all registered breast cancers since 1976; consequently, the proportion of unstaged breast cancers in the 50-64 age-group is only 2 % since then. We have therefore been able to compare trends
concerns
expressea by
Lr
jencoate (Nov Y,
1212) and Dr Ekins (Feb 22, p 499) that there should be some form of regulatory control of assay kits. Your readers may be interested to know that a series of three European Community (EC) Directives regulating the safety and marketing of all medical devices begin to come into effect from 1993. The Commission of the EC has just started drafting the third of p
these Directives which will cover in-vitro diagnostic medical devices (IVDs), and it is expected that it will issue a proposal later this year to take effect by 1996. A separate Directive is proposed for IVDs because they do not come into direct contact with patients and therefore the safety issues are different from those for other medical devices. Their accuracy, specificity, and reliability is of primary concern.
The Directive will contain a definition of an IVD device, which is expected to be along the following lines: "any medical device which is a reagent, reagent product, kit, instrument, apparatus or system whether used alone or in combination, intended by the manufacturer to be used in vitro solely or principally for the examination of substances derived from the human body for the purpose of providing information relevant to the detection, diagnosis, monitoring or treatment of physiological states, states of health or disease or congenital abnormality". The Directive is expected to contain essential requirements on safety and performance and to divide IVDs into two classes. Most devices will fall into class I (low risk), for which it is expected that the manufacturer’s self-declaration of conformity with the requirements of the Directive will suffice. Those whose
1361
performance is crucial in respect of patient health and safety, will fall into class II and will require outside approval by an independent certification body. Class II will possibly include kits used for HIV testing and other blood donation screening tests (eg, hepatitis B
and C).
proposed Directive will not prevent from restricting the availability of particular IVDs. This is especially relevant because regulations came into force in the UK on April 1, 1992, under the Health and Medicines Act Section 23(1) and (3), (SI 1992 no 460: The HIV Testing Kits and Service Regulations, 1992). These regulations control the use, sale, supply, and advertising of HIV testing kits and testing services, and ban the sale or supply of HIV testing kits to members of the public. The provision of testing services is now an offence unless they are provided by or in accordance with the directions of a registered medical practitioner. It is
expected
that the
individual member
states
European Section, Medical Devices Directorate, Department of Health, London WC1B 5EP, UK
of mind and level of confidence, with a minimum of diffidence and hesitation and absence of any impression of panic. Such qualities may be to some extent gender dependent in favour of the male psychological constitution. There exists a subgroup of women who no doubt have these qualities and who are more likely than others to succeed in surgery. However, it is conceivable that because of innate gender differences there will tend to be fewer female surgeons overall. Indeed Mr Cobb’s point in respect of women having a better appreciation of the hazards of a career in surgery may be but a further manifestation of such psychological gender differences. A more cautious approach to life and perhaps surgery may be an attribute of the female of the species. 26 Millway Close,
Upper Wolvercote,
Clinical RICHARD GUTOWSKI
J. R. BENSON
Oxford OX2 8BL, UK
training and research in the EC
SIR,-Professor Gill (May 16, p 1216) draws attention to the to harmonise clinical training in the European Community (EC). There is a similar need to harmonise research training, not least because of the increasing requirement of a research degree to succeed in hospital medicine.1,2 The great variation in regulations
need
Nursing’s identity crisis SIR,-Malcolm Dean (May 9, p 1160) seems to have become entangled in current nursing issues. As he says, no consensus exists within the nursing profession about the future role somewhat
of the nurse, and some do indeed wish nurses to take on certain tasks previously within the province of doctors. There may be, as Dean suggests, some nurses who fear that in taking on quasi-medical tasks they risk pricing themselves out of a job. For this reason they favour retaining basic nursing tasks along with those delegated by doctors. But there is also a growing contingent of nurses--generally including those who practise primary nursing-who see the knowledge needed for nursing and medicine as being quite distinct. In their view routine tasks associated with patient care represent opportunities for nurses to relate to their patients and to use specific nursing skills and knowledge. Such tasks are therefore not regarded as unskilled but an integral part of the development of a therapeutic relationship between nurse and patient. Nurses who have adopted this view, far from aping medicine, are struggling to gain recognition for the value of nursing, albeit within a culture that awards privilege to medicine.
JAN SAVAGE
Surgical careers and female students SIR,-Iread with interest reports from Oxford University Medical School of sexual discrimination in respect of surgical careers for women (April 18, p 994; May 16, p 1235). As a graduate of that institution I am reluctant to support the suggestion that women are (unjustifiably) discriminated against and discouraged from pursuing a career in general surgery. Should this impression be gleaned by certain persons, then perhaps they should consider why this might be. We live in a world where a policy of sexual equality, especially at work, prevails. However, in reality the sexes differ not only biologically, but also in less tangible, more subtle ways in respect of psychological make-up (personality, attitude, temperament, emotional reaction). Such differences may become apparent and assume importance in certain occupations, of which surgery is
perhaps an example. Apart from the long and unpredictable hours of work that inevitably involve sleep deprivation, surgeons also sometimes have to operate (often on long and difficult cases) when very fatigued. In my experience female house surgeons are not as tolerant of sleep
deprivation
and
prone to succumb to exhaustion and their male counterparts. Indeed, recent reviews of junior doctors working hours may be a reflection of pressure for change imposed by much increased numbers of women in medicine as a whole. Some aspects of surgery-for example, procedures for emergency thoracoabdominal trauma--demand a certain attitude are more
absenteeism than
are
Royal Free Hospital School of Medicine, University of London,
L. H. BREIMER D. P. MIKHAILIDIS
London NW3 2PF, UK 1.
Johnson R. Requirements of British universities for higher medical degrees. Br Med J 1991; 302: 397-99.
2. Cobb RA. Surgical careers and female students. Lancet 1992; 339: 1235. 3. Breimer LH, Mikhailidis DP. A thesis for all seasons. Nature 1991; 353: 789-90.
Prenatal
Bloomsbury and Islington College of Nursing and Midwifery, John Astor House, London W1P 8AN, UK
between the current systems makes this impossible. Even within the UK the regulations vary.’1 We have suggested that a unified EC doctorate could be based on work published in internationally refereed journals.3 The thesis itself, which would be comparatively short, can be published in a special European (international) thesis journal. Such an approach would be flexible and allow interaction with countries at present outside the EC, such as Sweden or Japan, where publication-based systems already operate.
diagnosis of haemoglobinopathies in Sicily
SIR,-Dr Giambelluca and colleagues (Jan 18, p 179), discussing the thalassaemia strategy in Sicily, state that although there was a fall in incidence of Cooley’s anaemia births from 1982 to 1989 because of the prenatal diagnosis programme at the V. Cervello Hospital, that improvement has stabilised "due to a gap in health information". We think that to explain these data it would be useful to emphasise other factors too-the true frequency of &bgr;-thalassaemia and sickle-cell carriers and the possibility of a heterogeneous distribution; the efficacy of screening by peripheral centres; and the total number of prenatal diagnoses per year. These haemoglobinopathies are heterogeneously spread in Sicily. Data from the regional health department (personal communication) show a higher prevalence in the east of the island, the two areas having roughly the same populations: Area East West
Cases of Cooley’s anaemia (1980-88) 80 81 82 83 84 85 86 87 88 34 17
46 10
29 15
24 9
27 8
15 9
14 8
14 8
11 1
These data show that after the start of prenatal diagnosis in 1983 the frequency in eastern Sicily remained higher than in the west of the island. In western Sicily and in the interior the frequency is certainly lower than that recorded by Giambelluca et al, and it is difficult to get a reliable estimate of carrier frequency because the screening programme is not centralised and depends on the organisation of local thalassaemia centres. The number of people tested at thalassaemia peripheral centres did increase between 1983 (26 978) and 1990 (43 399) but not all centres screen only young couples, and this may reduce the efficacy of the programme, especially where the carrier prevalence rate is
