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Purpose: To gain an insight into the adequacy of ophthalmic medical education for doctors in the primary care setting. Methods: A short forced-choice questionnaire was set to 150 randomly selected primary care practitioners in and around Bristol. Information was collected in relation to undergraduate and postgraduate ophthalmic education, ophthalmic confidence, facilities and understanding. Results: One hundred and thirty-three primary care doctors replied to the questionnaire of whom 35% were fundholders and 47% in training practices. Only 22% of all respondents felt their undergraduate ophthalmic medical education to be adequate. However, 83% of the 86 primary care doctors who had attended postgraduate update courses in ophthalmology felt these to be adequate. Despite the availability of an ophthalmoscope and distance vision chart, only 56% felt confident with the ophthalmoscope and only 61% reported that their distance chart was set up in accord with manufacturer's instructions. Seventy-one per cent of respondents reported having access to dilating agents but only 61% felt confident using them. Understanding of two key ophthalmic terms was also poor. Despite the general satisfaction, attendance of postgraduate update courses did not appear to alter facilities, confidence or understanding. Conclusions: It is apparent that most primary care doctors view their undergraduate ophthalmic medical education as inadequate and this is reflected in their confidence and understanding. Postgraduate courses, although more favourably received, do not appear to alter these findings. We strongly suggest, therefore, that general ophthalmic education is aimed at teaching examination techniques and ophthalmological principles suitable for primary care practice.

Five families are described in which there have been matings between individuals doubly heterozygous for beta thalassaemia and the delta-chain variant haemoglobin A2' to normal persons. In all there were 24 informative offspring. There were no crossovers between the beta-thalassaemia and delta-chain loci; in three of the families the genes were linked in cis and in two families the genes were found in trans. Together with previously reported families there have now been 58 opportunities for crossing over between the beta-thalassaemia and delta-chain loci and there have been two possible and one highly probable crossovers. Of the total of 9 families reported to date 4 have had the genes for beta thalassaemia and Hb A2' in cis and 5 in trans. These findings are contrasted with the findings in families where a beta-chain structural variant and Hb A2' have been observed together and these genes have always been found in trans and never in cis. The reasons for linkage disequilibrium of this type are discussed. It is concluded tentatively that the distance between the delta-structural and beta-thalassaemia loci is greater than that between the delta-structural and beta-structural loci. To date this conclusion can only be applied to the beta+ -thalassaemia and beta-thalassaemia genes as found in the African population, since this is the only population with a high incidence of delta-chain mutants which allow linkage analysis of this type to be carried out.

Hematologic Changes and Hemoglobin Analysis in β Thalassemia Heterozygotes during the First Year of Life

Summary. The rate of clearance of 15Cr-labelled, heat-damaged red cells from the circulation has been measured in 18 patients with adult coeliac disease. This has been combined with scintillation scanning of the spleen using a colour scanning method. Only two of the patients had clearance times within normal limits. Five had a peripheral blood picture suggestive of splenic atrophy. In these the half time of clearance was greater than 50 min and the scintillation scan showed either no evidence of functioning splenic tissue or only minimal localization of radioactivity suggesting marked hyposplenism. Nine patients had a somewhat prolonged clearance time, the half time of clearance varying between 19 and 44 min. The scan, however, showed an apparently normal spleen and the characteristic changes of splenic atrophy were not present in the peripheral blood film. Two patients had clearance rates faster than that found in control subjects. Both of these patients had enlarged spleens. In one this was associated with hepatic cirrhosis and in the other with an unexplained neutropenia.

A 6-year-old boy with acute lymphoblastic leukemia (ALL) developed a haemophagocytic syndrome resembling histiocytic medullary reticulosis (HMR) but made a complete recovery on supportive treatment. This was subsequently found to have been associated with a parainfluenzal infection. It is suggested that HMR in immunocompromised hosts may represent a reactive process to an opportunistic viral infection and that the use of chemotherapy in these patients may be deleterious.

Haematological values of 35 pregnant women with beta-thalassaemia trait were followed during pregnancy. The discriminant function, calculated from haematological indices, was of no value in diagnosing beta-thalassaemia trait during pregnancy. Initially patients were given iron supplements only if the serum iron and total iron binding capacity levels indicated iron deficiency, but bone marrow biopsies performed in the first 22 patients at 32 weeks indicated deficient iron stores. These patients were therefore given iron irrespective of their serum iron level. All subsequent patients with beta-thalassaemia were also put on iron routinely at booking. Retrospectively the patients were divided into two groups. Patients in group 1 (18 patients) had received iron for less than 12 weeks, and their haemoglobin levels fell significantly during pregnancy (P less than 0-001). Haemoglobin levels in 16 patients who had received iron for more than 12 weeks (group 2), however, did not fall significantly during pregnancy (P less than 0-6). It is suggested (contrary to common practice) that patients with beta-thalassaemia trait should be given iron supplements during pregnancy. Serum folate and vitamin B12 levels did not change significantly in these patients and there was no increase in the incidence of maternal or fetal complications.

Forty-two adults and children with previously untreated acute lymphoblastic leukaemia (ALL) were entered into a programme of chemotherapy in which three combinations, each of four drugs were administered in a predetermined cyclical rotation together with cranial irradiation and intrathecal injections of methotrexate. Forty-one patients (98%) entered remission and no patient developed neuroleukaemia. Relapse of ALL occurred in 10 patients, and three patients died during remission, while eight patients stopped treatment after two and a half years and have remained in remission for two to 26 months. Comparison of remission and survival experience in this mixed group of children and adults with the experience of children treated at Memphis and in the Medical Research Council's UKALL-I trial showed no significant differences. On the other hand, analysis by prognostic factors showed that neither age nor blast cell count at presentation had any adverse effect in patients treated in this study. No relapses occurred in nine patients with blast cell counts greater than 20 x 109/1 at presentation. This regimen is effective treatment for ALL and may be of special value in patients with poor prognoses. The regiment has not as yet proved superior for the treatment of children with ALL who do not have adverse prognostic features.