Prevalence of ß-thalassemia and other haemoglobinopathies in six cities in India: a multicentre study.

The population of India is extremely diverse comprising of more than 3,000 ethnic groups who still follow endogamy. Haemoglobinopathies are the commonest hereditary disorders in India and pose a major health problem. The data on the prevalence of ß-thalassemias and other haemoglobinopathies in different caste/ethnic groups of India is scarce. Therefore the present multicentre study was undertaken in six cities of six states of India (Maharashtra, Gujarat, West Bengal, Assam, Karnataka and Punjab) to determine the prevalence of haemoglobinopathies in different caste/ethnic groups using uniform methodology. Fifty-six thousand seven hundred eighty individuals (college students and pregnant women) from different caste/ethnic groups were screened. RBC indices were measured on an automated haematology counter while the percentage of HbA(2), HbF and other abnormal Hb variants were estimated by HPLC on the Variant Hemoglobin Testing System. The overall prevalence of ß-thalassemia trait was 2.78 % and varied from 1.48 to 3.64 % in different states, while the prevalence of ß-thalassemia trait in 59 ethnic groups varied from 0 to 9.3 %. HbE trait was mainly seen in Dibrugarh in Assam (23.9 %) and Kolkata in West Bengal (3.92 %). In six ethnic groups from Assam, the prevalence of HbE trait varied from 41.1 to 66.7 %. Few subjects with δß-thalassemia, HPFH, HbS trait, HbD trait, HbE homozygous and HbE ß-thalassemia as well as HbS homozygous and HbS-ß-thalassemia (<1 %) were also identified. This is the first large multicentre study covering cities from different regions of the country for screening for ß-thalassemia carriers and other haemoglobinopathies where uniform protocols and methodology was followed and quality control ensured by the co-ordinating centre. This study also shows that establishment of centres for screening for ß-thalassemia and other haemoglobinopathies is possible in medical colleges. Creating awareness, screening and counselling can be done at these centres. This experience will help to formulate a national thalassemia control programme in India.

Elevated red cell volume in North Indian students.

In a survey for beta-thalassemia carrier status among students in the State of Punjab in India, a surprisingly large number were found to have an elevated red cell volume over 99 fl. The finding was predominantly but not exclusively in females. Similar student surveys from other states showed less macrocytosis. Follow-up tests in a group of affected students were carried out. Volunteers were asked to modify their diet then after six months they were provided with oral vitamin B12. The resulting changes are reported and the implications of the probable vitamin B12 and or folic acid deficiency are considered.

Use of the WHO hemoglobin color scale in family welfare clinics in India.

A situation is reported for the use of the WHO color scale for the measurement of hemoglobin, which was introduced into India in 1996. Charity family welfare clinics held at a medical college hospital in North India offer free sterilization by tubal ligation under local anesthetic to women following a preliminary screening. An obligatory test of the hemoglobin level is required and must be above 7.0 g/dl for the operation. Some clinics attract large numbers, and the card gives a cheap, rapid and reasonably accurate test, adding to the smooth running and reduction of waiting time. It has proved satisfactory over a 2-year period; 2.3% of participants were shown to have a hemoglobin level of 7 g/dl or less.

Idiopathic myelofibrosis in children.

Childhood myelofibrosis (Mf) is rare with variable outcome reported in the literature. We present clinical and investigate details of three children who presented with idiopathic Mf in early childhood. Two of these children were identical twins and have been haematologically stable over the past 7 years since their diagnosis. The third patient underwent an allogeneic bone marrow transplant (BMT) procedure as her clinical status was deteriorating. She remains engrafted at 13 months post BMT. None of the children had hepatosplenomegaly although extramedullary haemopoiesis was demonstrated on 52Fe studies in two patients. Circulating progenitors in these patients were increased in the face of reduced marrow precursors. The aetiology of childhood Mf is unclear and its natural history seems different from the adult disease. Allogeneic BMT can be an option for definitive treatment.

A new glucose-6-phosphate dehydrogenase variant, G6PD Orissa (44 Ala-->Gly), is the major polymorphic variant in tribal populations in India.

Deficiency of glucose-6-phosphate dehydrogenase (G6PD) is usually found at high frequencies in areas of the world where malaria has been endemic. The frequency and genetic basis of G6PD deficiency have been studied in Africa, around the Mediterranean, and in the Far East, but little such information is available about the situation in India. To determine the extent of heterogeneity of G6PD, we have studied several different Indian populations by screening for G6PD deficiency, followed by molecular analysis of deficient alleles. The frequency of G6PD deficiency varies between 3% and 15% in different tribal and urban groups. Remarkably, a previously unreported deficient variant, G6PD Orissa (44 Ala-->Gly), is responsible for most of the G6PD deficiency in tribal Indian populations but is not found in urban populations, where most of the G6PD deficiency is due to the G6PD Mediterranean (188 Ser-->Phe) variant. The KmNADP of G6PD Orissa is fivefold higher than that of the normal enzyme. This may be due to the fact that the alanine residue that is replaced by glycine is part of a putative coenzyme-binding site.

General anaesthesia and the hypereosinophilic syndrome: severe postoperative complications in two patients.

Two patients with markedly increased eosinophil counts developed severe postoperative complications after general anaesthesia. One patient suffered life-threatening Adult Respiratory Distress Syndrome (ARDS), while the other presented with a coagulopathy and less severe respiratory problems. The hypereosinophilic syndrome is described and the possibility of a role of eosinophils in the pathogenesis of tissue injury is discussed. These cases suggest that, in patients with marked eosinophilia requiring general anaesthesia, perioperative steroid cover is advisable. This may reduce or prevent serious lung damage and other complications.

Factors affecting the precision of warfarin treatment.

AIM: To determine what factors influence the precision of anticoagulant control using warfarin by examining the computerised records of 2207 patients. METHODS: Records from seven district general hospitals were combined and analysed. The precision of anticoagulant control was taken as the absolute deviation of International Normalised Ratio (INR) from target at the most recent determination. This quantity was examined using univariate and multiple regression analyses. RESULTS: Deviation of INR from target was continuously distributed, almost symmetrically about a mean of zero. The patients' age and sex had little bearing on control. Patients with a high target INR were more likely to be undertreated, and patients taking higher doses of warfarin were more likely to be overtreated. Previous over- or undertreatment were strongly related to poorer current control. The control of treatment varied substantially among the seven hospitals. One possible cause of this variation was the dose adjustment coefficient: the greater the dose adjustment for a given deviation from target INR, the better was the control achieved. CONCLUSION: Several groups of patients were identified whose control was less satisfactory and in whom anticoagulant treatment needs particular scrutiny: these include patients with a record of previous over- or undertreatment, but not elderly patients in general. The variation in control among hospitals is a source of concern that merits further attention to achieve better uniformity of anticoagulant treatment.

Factors affecting the maintenance dose of warfarin.

AIM: To identify the possible factors determining the dose of warfarin prescribed in patients receiving anticoagulant treatment. METHODS: The computerised records of 2305 patients maintained on the drug in seven hospitals were amalgamated and classified into one of seven diagnostic groups. The associations with the dose of warfarin prescribed were investigated by univariate and multiple regression analysis. Differences between hospitals were studied with regard to the coagulometric method and the thromboplastin preparation used. RESULTS: The geometric mean dose of warfarin was 4.57 mg and 5% of patients were prescribed 10 mg or greater. There was a noticeable decrease in dose with increasing age, which averaged about 6 mg for patients aged 30 but 3.5 mg for those aged 80. Men required slightly more warfarin than women. Patients with heart disease or atrial fibrillation required lower doses of warfarin, while higher doses were required by patients with deep vein thrombosis. Significant differences in mean warfarin dose among the seven hospitals were evident. These differences could not be explained entirely by the use of different coagulometric methods or thromboplastins. CONCLUSIONS: Clinicians should be aware that older patients need reduced doses of warfarin. The considerable differences in doses of warfarin among hospitals indicates that further efforts to improve uniformity are required.

Experience with a computer-assisted anticoagulant clinic.

A microcomputer has been used for 6 years to assist in the running of the anticoagulant clinic for over 300 patients in a district general hospital. The dose adjustments and the time intervals to the next visit are decided automatically. The system has proved very satisfactory in practice, as shown by data presented. There is considerable saving of medical and secretarial time. There has been no equipment failure necessitating reversion to manual methods. The system produces letters (or labels), lists and continuous statistics, and allows the selection of various options, which makes it adaptable to the requirements of other hospitals.

Anaemia in Asians in London.

Many Asian subjects were seen in London in the early 1970s with megaloblastic anaemia; in a majority of cases, this was dietary in origin. Pernicious anaemia occurs in Asians, but the incidence is probably lower than in Caucasians. Megaloblastic anaemia is now less common in this group, but a population survey in Punjabi females in the late 1970's showed anaemia in 32%, and one or more features of iron deficiency in 76%.

Prolonged incubation period of imported P. vivax malaria in London.

Between January 1976 and July 1979, 453 cases of malaria were seen at Hillingdon Hospital. The majority of cases were Plasmodium vivax infections in Asians from the Punjab in Northern India-either new immigrants or United Kingdom resident Asians returning from holidays. Twenty-four cases were contracted in Africa or the Middle East. Figures are presented showing a considerable increase in cases during the period of study. In P. vivax infections the time interval between arrival and development of the acute illness was significantly greater for those subjects entering the United Kingdom in autumn or winter.

Hypertension in a population sample of female Punjabi Indians in Southall.

A population-based survey of female Punjabi Indians aged 11 and over now living in Southall, a district in west London, showed a steady and significant increase in blood pressure with increasing age. The crude population prevalence of hypertension, defined according to the criteria of the World Health Organisation, was 16%; for women over the age of 40 it was 62%. About two-thirds of those in the hypertensive range did not know of their high blood pressure. Among those who did know, up to 75% were receiving medical treatment for the condition.

Comparison of 125I-labelled fibrinogen uptake and venography in the detection of recent deep-vein thrombosis in the legs.

Both techniques were used to examine 72 legs, and agreed about the presence or absence of thrombus in 54 cases (72 per cent). In 41 legs both tests were positive and in 13 legs both tests were negative. In 16 legs the 125I fibrinogen test only was positive, and if the seven legs with clinical conditions known to increase fibrinogen uptake are excluded, there were nine legs (13 per cent) with an increased fibrinogen uptake in the calf and a normal venogram. In two legs the venogram only was positive, and in both cases there was evidence of clot retraction which suggested that the thrombus was more than a week old. The use of radio-opaque markers attached to the skin made it possible to compare the site of thrombosis detected by the two techniques and the results of this comparison indicate that the fibrinogen test underestimated the extent of femoral vein thrombosis in a significant proportion of cases. (p less than 0-001).