Haemoglobin electrophoresis patterns in Barbados

Despite the extensive data on haemoglobinopathies and their widespread geographic distribution, the number of prospective Caribbean studies which document the prevalence of haemoglobinopathies from birth are few. The purpose of this cohort study was to document the prevalence of haemoglobinopathies in newborn infants in Barbados. One thousand successively collected cord bloods of newborn infants were screened for haemoglobinopathies using the Paragon acid electrophoresis technique. Seventeen infants were retested at 1 year of age to confirm the diagnosis. Three mothers could not be located so their infants' diagnoses could not be confirmed. From the 997 blood samples with conclusive results, a haemoglobinopathy was found in 72 (7 percent) samples. Laboratory analysis revealed: 925 patients (93 percent) with Hb AA, 41 (4 percent) with Hb AS, 27 (2.7 percent) with Hb AC, 2 (0.2 percent) with Hb SS, 1 (0.1 percent) with Hb CC and 1 (0.1 percent) with Hb SC. The prevalence of sickle gene and number of cases of Sickle Cell anaemia in the Barbadian population is less than has been reported from other regional territories.(AU)

Use of blood specimens collected on filter paper in screening for abnormal hemoglobins

Both cellulose acetate electrophoresis and citrate agar electrophoresis were performed on 834 blood samples collected on filter paper in Jamaica and shipped for testing to the National Hemoglobinopathy Standardization Laboratory at the U.S. National Center for Disease Control. Additionally, 30 blood samples collected locally were stored on filter paper, in microhematocrit capillary tubes, and as whole blood specimens; at selected times the samples were tested for stability to determine the best sample-collection technique for hemoglobin electrophoresis. Results were most nearly accurate when both cellulose acetate electrophoresis and citrate agar testing were used. The methods are easy to perform, but results are unreliable if the blood samples on filter paper are stored at 4§C for longer than two weeks before they are tested (AU)

Haematological levels in Jamaican infants

A longitudinal study of 300 infants from birth to 1 year of age was carried out in Kingston, Jamaica. Haemoglobin levels were estimated 7 times during the year and serum iron and total iron binding capacity once. Hb electrophoresis was performed. In singleton children with Hb genotype AA, AS, or AC, and of birthweight 2.5kg or over, Hb levels were low after 3 months of age. These low levels were associated with iron deficiency, which was probably due to poor iron stores at birth followed by poor iron intake or absorption. It was not possible to determine whether folic acid deficiency or protein deficiency was also important. Hb levels varied with age, socioeconomic class, birthweight, sex, and rate of weight gain. The growth and health of 4 girls with homozygous sickle cell disease is mentioned.(AU)

Screening cord bloods for detection of sickle cell disease in Jamaica

A cord-blood screening program, designed primarily for detecting sickle cell disease, has been in operation for seven months (8 000 samples) at a large maternity unit in Kingston, Jamaica. We describe techniques of cord-blood collection and electrophoretic investigation on both cellulose acetate and agar gel. These methods appear to give rapid, valid results at minimal expense and are well adapted to screening large populations (AU)

Haemoglobin F Jamaica (Alpha-2 gamma-2 61 Lys --> Glu; 136 Ala)

Two siblings of mixed Negro, East Indian and Causacian parentage showed an abnormal haemoglobin component in infancy which had electrophoretic characteristics similar to Hb F Roma (Silvestroni & Bianco, 1963). The amino-acid substitution responsible for the peculiar electrophoretic mobility was found to be in the gamma polypeptide chain (gamma-61(E5)Lys-->Glu). The finding of an alanyl residue at position of 136 of the gamma-chain of the abnormal component in the first born of these infants was repeated in the abnormal gamma-chain of the second infant. No functional abnormalities were found to be associated with this abnormal haemoglobin, which is designated Hb F Jamaica (Summary)

Demyelinating disease in a woman from tropical South America with features of multiple sclerosis and neuromyelitis opticaz: clinical, protein chemistry and pathological findings

The ocurrence of demyelinating disease with many of multiple sclerosis and of neuromyelitis optica in a patient born and brought up in Guyana in tropical South America is so exceptional as to merit an individual case report. The similarities and differences between the findings in this patient and those accepted as typical of these diseases, as they are commonly known, are discussed in detail. (AU)

An improved electrophoretic technique

The present study describes a simple, inexpensive electrophoretic apparatus which can be adjusted to accommodate different types of substrates of varying dimensions. The results obtained with cellulose acetate membrane filter, agar and starch gel as substrate are presented (AU)

An improved electrophoretic technique - abstract

A simply electrophoretic apparatus which could be constructed in any laboratory workshop is described. The electrodes are inexpensive and a negligible amount of platinum is used. Using cellulose acetate membrane as supporting medium a run could be completed in less than 3 hours. The apparatus is so designed that microelectrophoresis in agar or starch gel on microscopic glass slides can readily be performed (AU)

Studies on thyroid nodules in Jamaica

Preliminary studies in thyroid weight, incidence of nodules and iodine content at autopsy indicate that in Jamaica, all these parameters are low. Functional studies indicate a low rate of thyroid activity and an apparent border-line iodine deficiency. The low incidence of nodules at autopsy suggests, however, that iodine deficiency is not a major factor in the pathogenesis of goitre in Jamaica. On the other hand, approximately 50 patients are operated on each year at the U.C.H.W.I for non toxic nodular goitre. Studies are therefore being made of the surgically removed thyroid tissue in this group by means of electrophoresis and chromatography of iodinated proteins and amino acids in the hope of uncovering some other pathogenetic factors (AU)

The hemoglobinopathies in Trinidad

Six hundred and fifty persons were studied with respect to electrophoresis and foetal hemoglobin. Of 204 random samples, 177 were normal, 19 showed an AS pattern, 6 AC, 1 AE and 1 AN. In a group diagnosed as having hemoglobinopathy the following results were noted: SS 121, AS 200, SC18, AC 18, AE 1, S-Thal. 6, C-Thal. 4, Thal. Major 6, Four cases were complicated by a megaloblastic anaemia and 1 case of high foetal hemoglobin in a child with chronic myeloid leukemia is recorded (AU)

Electrophoretic studies on serum proteins of malnourished infants (abstract only)

The changes in serum proteins of fourteen malnourished Jamaican children admitted to the M.R.C. Ward have been studied. The findings are compared with figures obtained from a small group of normal adult Europeans and Jamaicans; no racial differences could be detected. In the Jamaican children the albumin is low on admission and increases rapidly, within twenty to thirty days, to levels approaching normal. Little change occurs in the globulins apart from an increase in the beta fraction. The gamma globulins are not significantly higher than normal on admission and show little change on treatment (AU)

Further observations on abnormal haemoglobins in Jamaica

The laboratory findings on 75 cases of sickle cell anaemia (genotype SS), 32 cases of sickle cell-haemoglobin C disease (genotype SC) and 11 case of sickle cell-thalassaemia disease are described. With cases reported earlier this makes a total of 114 cases of sickle cell anaemia and 45 cases of sickle cell-haemoglobin C disease which we have seen in the last two years. Approximately half our cases of sickle cell anaemia are 15 years of age or over, including three cases in one large family. Reasons are given for regarding these as cases of homozygous sickle cell anaemia. The haematological findings and foetal haemoglobin levels in these three conditions are discussed with special reference to their value in differential diagnosis. The apparent shortage of cases of sickle cell-haemoglobin C disease as compared with cases of sickle cell anaemia is discussed. A case of sickle cell anaemia responding well to splenoctomy is reported.(AU)

Sickle cell-thalassemia disease in Jamaica

Two cases of sickle-cell thalassemia disease are described in young women of mixed Chinese and African parentage. On hemoglobin electrophoresis, a complete suppression of hemoglobin A was found, giving a picture indistinguishable from that seen in sickle-cell anemia. The findings in these two cases are contrasted with those in other examples of this disease which we have studied in Jamaica. The importance of these findings in relation to the diagnosis of sickle cell anaemia is discussed (Summary)

The incidence and clinical effects of abnormal haemoglobin in Jamaica (abstract only)

The mode of inheritance of the genes carrying abnormal haemoglobins is discussed, together with the methods used for their study. Some of our findings in Jamaica during the last nine months are presented. 780 women attending the ante-natal clinic have been investigated by haemoglobin electrophoresis. The incidence of the sickle cell trait is 9.5 per cent and of the haemoglobin C trait 3.4 per cent. In addition two women were found to have unsuspected sickle cell/haemoglobin C disease, and one lady had apparent sickle cell anaemia - she has four healthy children. The clinical features of sickle cell anaemia and other related diseases are discussed. Three cases of Cooley's Anaemia are reported, but the incidence of the Cooley gene is not known. The importance of this gene is stressed, for in conjunction with the S gene a condition - sickle cell/thalassaemia disease - is produced which may be indistinguishable clinically and in the laboratory from sickle cell anaemia. A case of sickle cell/thalassaemia disease is reported in which normal haemoglobin (A) production is completely suppressed giving the same electrophoretic and haematological picture as is found in sickle cell anaemia. The importance of differentiating this condition from sickle cell anaemia is stressed. This can be done by family studies. An example of homozygous haemoglobin C disease is reported. (AU)