Diagnosis of alpha-thalassemia trait from Coulter Counter 'S' indices.

A number of patients of Mediterranean and Asian origins were found to have unexplained microcytic hypochromic red blood cells. Iron deficiency and beta-thalassaemia trait were both satisfactorily excluded in all of them. The haematological indices of these patients, obtained on a Coulter Model 'S' Counter, were found to be very similar to those seen in obligatory heterozygotes for alpha-thalassaemia. It is postulated that these patients were also carriers for alpha-thalassaemia. Subsequent investigation of some of these patients showed the characteristically reduced rates of alpha-chain synthesis seen in this condition. The discriminant function of England and Fraser (1973) may be of help in diagnosing this state. alpha-Thalassaemia trait should be considered in all patients of 'high-risk' ethnic origins with a blood picture suggestive of beta-thalassaemia trait but in whom the levels of Hb A2 and Hb F are within normal limits.

Different therapeutic approaches and outcome in the treatment of pterygium.

PURPOSE: This study was initiated to investigate the role of different therapeutic modalities in the outcome of the surgical treatment of pterygium. METHODS: The results of treatment of pterygia with a variety of surgical techniques were studied in 56 eyes (49 patients) operated on at Bristol Eye Hospital during a period of five years. The surgical techniques included simple excision; bare sclera; conjunctival autograft; sliding conjunctival flap; lamellar keratoplasty and penetrating keratoplasty. Twelve eyes received additional beta irradiation in a fractionated total dose of 40 Gys. RESULTS: The incidence of recurrence was 23.2% for the 43 treated primary pterygia, and 23% for the 13 recurrent pterygia. All recurrences occurred between 2.5 and 11 months postoperatively. None of the 11 cases where additional beta irradiation was used showed any recurrence or other complication within the study period. In the recurrent pterygia group, the cases treated with a combination of surgical excision and beta irradiation, showed significantly lower recurrence rate (p < 0.001) compared to those cases treated with surgical excision alone. CONCLUSIONS: Beta irradiation as a complement to surgical treatment of pterygium, is successful in treating high risk cases such as reoperations, whereas for the majority of primary pterygia surgical excision alone is adequate. Additionally, follow up of one year will reveal any recurrences.

Refining an emergent life-style-change theory through matrix analysis.

Matrix analysis is a methodologic tool used by researchers to systematically enter qualitative data into matrices and to simultaneously perform the complex integrative functions of data analysis and verification of findings. The article focuses on (1) the steps of qualitative data analysis that can be achieved by using matrix analysis, (2) the appropriate conditions for using matrix analysis, (3) the procedure for constructing a matrix, (4) the application of matrix analysis to a research problem that involved refining an emergent theory of the health life-style-change process, and (5) the use of matrix analysis to meet the criteria for trustworthiness of research findings.

Measuring patient satisfaction outcomes across provider disciplines.

The purpose of this methodological research was to modify and test an instrument measuring patient satisfaction outcomes with primary care providers who represent different disciplines. The Patient Satisfaction with Health Care Provider Scale (PSHCPS) was adapted from a questionnaire indexing four satisfaction dimensions: Access, Humaneness, Quality, and General Satisfaction (Cherkin, Hart, & Rosenblatt, 1988). Following modification, the PSHCPS was administered to 167 adults with NP or MD providers at a university-based, managed-care setting for the medically indigent. The total scale Cronbach's alpha was .93. Factor analysis supported an unidimensional scale with 18 items loading above .40 and 43% explained variance.

Managing relapse as a positive opportunity for change.

During the process of lifestyle changes, there is a high incidence of relapse during the first three months. There are frequently negative feelings experienced by both the nurse practitioner and the client. This paper presents information concerning relapses, conceptualizing relapse as a positive experience, and strategies to support clients who encounter relapse in their efforts to maintain positive health habits.

How effective is undergraduate and postgraduate teaching in ophthalmology?

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 after these findings. We strongly suggest, therefore that general ophthalmic education is aimed at teaching examination techniques and ophthalmological principles suitable for primary care practice.

Historic and future health promotion contexts for nursing.

PURPOSE: Health promotion in the United States has been narrowly defined focusing primarily on individual behaviors, risk factors, and lifestyle. This article traces the historic and conceptual roots of health promotion care in the United States and critically examines the direction nurses have taken in health promotion. SCOPE: Health promotion care in Western ideology emphasizes the value of individuals and the importance of personal responsibility for success or failure. CONCLUSIONS: Health promotion nursing interventions, based on the tenets of individual choice and knowledge, restrict the range of interventions. The complex nature of health promotion requires that nurses consider the economic, sociopolitical, and cultural context in which health care takes place. Recommendations are made for an expanded nursing role in health promotion, one that is consistent with nursing's underlying contextual focus and concern with environments.

Sickle cell disease in North London.

We have examined our experience of sickle cell disease in the London Borough of Haringey over the past 20 years. There are currently (1986) 145 patients on the Haringey Sickle Cell Register and when comparison is made with other centres, admission for painful crises in Hb SS disease is more frequent than in Jamaica, but the acute chest syndrome appears to be less common in the United Kingdom than in Jamaica. Splenomegaly is less frequent in Hb SC patients in this country and there is also a lower incidence of leg ulceration in both Hb SS and Hb SC disease in the United Kingdom than in Jamaica. The incidence of alloimmunization among transfused sickle cell disease individuals in Haringey is 21%, the most frequently encountered antibodies being anti-E and anti-Kell. All pregnant patients with sickle cell disease were managed with regular blood transfusion throughout pregnancy starting at 12 weeks of gestation. There were no fetal or maternal mortalities, although three babies born were 'small for dates' despite uneventful antenatal courses and adequate Hb A levels being achieved in the mother. Examination of the effect of seasonal variation on the incidence of hospital admissions for painful crises reveals no significant clustering of cases in a particular month or season of the year.

Primary proliferative polycythaemia without splenomegaly: a diagnostic problem.

We report three cases of polycythaemia with no evidence of clinical splenomegaly and normal splenic red cell pool on isotope spleen scan. In each case, however, a diagnosis of primary proliferative polycythaemia (PPP) was suggested by in-vitro erythropoietin-independent growth of peripheral blood erythroid colonies. In one of these cases two possible causes of secondary polycythaemia were also identified. The use of investigations such as isotope spleen scanning and erythroid cell culture in helping to establish a diagnosis of PPP is discussed.

Complete recovery of histiocytic medullary reticulosis-like syndrome in a child with acute lymphoblastic leukemia.

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.

Thalassaemia, iron, and pregnancy.

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.

The histopathology of prolymphocytic leukaemia with particular reference to the spleen: a comparison with chronic lymphocytic leukaemia.

The histological features of prolymphocytic leukaemia are described in spleen, lymph nodes, liver and bone marrow from nine cases. Eight patients had B-PLL one had T-PLL. These features were compared with those of six cases of B chronic lymphocytic leukaemia (B-CLL) with massive splenomegaly. Both PLL and B-CLL showed enlargement of the white pulp with nodule formation and diffuse infiltration of the red pulp. In both PLL and CLL larger cells were found in the white pulp nodules often concentrated on the periphery producing a bizonal appearance. The extent of involvement of the white pulp was greater in PLL than in uncomplicated CLL. However, this was not so in two cases of B-CLL in prolymphocytoid transformation in which the heavy replacement of the white pulp by large cells without a bizonal arrangement had effaced the red pulp. The cytological differences between PLL and CLL were best appreciated in splenic red pulp. The cells of CLL were small lymphocytes with clumped chromatin, those of PLL were larger with bigger nuclei, often indented in some cases, and distinct nucleoli. The pattern of infiltration in the bone marrow, liver and lymph nodes was similar in PLL and CLL. In conclusion, PLL can be distinguished from CLL by morphological and immunological features. The distinction is important clinically, because the survival of PLL is shorter than that of CLL.

Predicting hospice appropriateness for patients with dementia of the Alzheimer's type.

The appropriateness of admitting individuals to hospice services is determined by assessing the individual's 6-month survival prognosis. Clinical parameters that guide clinicians in assessing prognosis, however, are not well defined in cases of dementia of the Alzheimer's type (DAT) when compared to other illnesses. The Alzheimer's-Hospice Placement Evaluation Scale (AHOPE) was developed to assess the 6-month prognosis of individuals with late-stage DAT. The purposes of this study were to estimate the reliability and predictive validity of AHOPE and to test additional demographic and clinical indicators to determine their added contribution to predicting 6-month survival and hospice appropriateness. Data were collected on 112 long-term care residents with DAT at enrollment and 6 months following enrollment. Initial reliability and predictive validity of AHOPE were supported. Other demographic and clinical indicators were not predictors of 6-month survival. Although additional research is indicated, nurses can use AHOPE to enhance clinical observation and decision making for implementing appropriate care strategies for patients with end-stage DAT and their families.

Determination of alpha thalassaemia phenotypes by messenger RNA analysis.

The possibility of using alpha/beta globin messenger RNA (mRNA) ratios to distinguish between the carrier states for different forms of alpha thalassaemia has been explored. Alpha/beta globin mRNA ratios were determined in the red cells of a series of normal individuals and in members of four Cypriot families, one Thai and one Chinese family in which at least one person has haemoglobin H disease. It was found that there was a clear distinction in the ratios between normals, alpha thalassaemia 1 carriers, alpha thalassaemia 2 carriers, and those with haemoglobin H disease. This method should be a valuable addition to haematological analysis, haemoglobin synthesis and restriction mapping of DNA for the further elucidation of the genetics of alpha thalassaemia.

Linkage relationships between beta- and delta-structural loci and African forms of beta thalassaemia.

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.

Non-Hodgkin's lymphoma: a survey from a district general hospital.

We report a retrospective survey of 167 patients with non-Hodgkin's lymphoma (NHL) seen at a district general hospital between 1 August 1971 and 1 August 1983. Details of their clinical presentation, histology, therapy, response rates and survival are presented. The patient population reported is generally older than that seen at specialist centres and has a greater proportion of tumours with low-grade histology. Whereas specialist centres see a pre-selected population of patients, our data may be from a more representative group and this gives a truer incidence of this disease in the general population. The remission rates and overall survival of our patients are comparable to those achieved at specialist centres. We conclude that it is both feasible and desirable for patients with NHL to be assessed and treated in a district general hospital with a radiotherapy department.

Acute lymphoblastic leukaemia: cyclical chemotherapy with three combinations of four drugs (COAP-POMP-CART regimen).

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.

Thalassaemia intermedia in Cyprus: the interaction of alpha and beta thalassaemia.

Restriction endonuclease analysis has been performed on the alpha and beta globin gene clusters of 57 Cypriots homozygous for beta thalassaemia, 30 with the transfusion dependent form of the condition (thalassaemia major) and 27 who are less severely affected (thalassaemia intermedia). There was a significant difference in the incidence of alpha thalassaemia between the two groups: 14/27 of the patients with thalassaemia intermedia also had deletion forms of alpha thalassaemia, while only 4/30 of the patients with thalassaemia major were similarly affected. Thus in Cypriot patients who are homozygous for beta thalassaemia the co-inheritance of alpha thalassaemia is an important factor in determining the clinical course.