Event related potentials in anemic school – going girls of age group 8 to 10 years.

In the present study the effects of anemia on cognitive functions were studied in school going girls aged 8-10 years. The cognitive functions were assessed by Event Related Potentials (P300) and by the psychometric tests, i.e., Raven’s progressive matrices test and Digit span attention test. The girls with Hb < 12 g/dl were classified into anemic and Hb > 12 g/dl into control group. Hematological values of the control group were significantly better than anemic group. P300 latency in the anemic girls was delayed as compared to control group but, no statistically significant difference was observed for P300 latency and P300 amplitude between the control group and the anemic group. The psychometric test scores for intelligence quotient and transformed quotient were also better but not statistically significant in control group of girls as compared to anemic girls. However, the hematocrit values showed a significant correlation with the P300 wave latency showing that the hematological status is associated with some effects on cognition.

Effects of iron therapy on cognition in anemic school going boys.

The present study was conducted on 18 anemic and 34 control subjects (mean age 9.26 +/- 0.26 years) to observe the effect of anemia on cognition and to see effect of 3 months of iron therapy on it. Anemia was defined on the basis of hematological values and peripheral smear examinations. Cognitive data consisted of the recording of the P300 wave of Auditory Event Related Potentials (AERP), Ravens Progressive Matrices Test (RPMT), and Digit Span Attention Test (DSAT) under standard test conditions. RPMT scores were then converted to the intelligence quotient (IQ) scores for comparison. Both anemic and control boys were dewormed after recording pretreatment values and then anemic boys were given iron therapy for 3 months, after which the recordings were taken again. Pretreatment, anemic boys showed significantly lower hematological values, delayed P300 latency, and lower RPMT scores as compared to controls. Post therapy the hematological profile of anemic boys though significantly improved as compared to the pretreatment values, was still significantly lower than that of control boys. The P300 latency values of anemic boys showed improvement but were still significantly delayed than the control group. RPMT values and derived IQ scores of anemic boys were similar to control boys after therapy suggesting that though the 3 months iron therapy regime resulted in improvement in psychometric cognitive tests in anemic boys, the basic P300 defects persisted. This suggests that the P300 component of AERP in anemic children is relatively refractory to 3 months of iron therapy.

Studies on oxidative stress, serum iron and iron binding capacity in subjects prone to the risk of coronary artery disease.

Lipid peroxidation in vitro and in vivo has been postulated to be involved in the development of atherosclerosis. It is also known that free iron catalyses the lipid peroxidation. Therefore, we assessed the status of oxidative stress in smokers, hypertensives and non-insulin dependent subjects, who were prone to coronary artery disease. In addition, superoxide dismutase levels and iron binding capacity were also measured to know their antioxidant defences. One hundred seventy-five consecutive subjects below 60 years of age were examined; they were then divided into three groups: one with coronary artery disease, another without coronary artery disease and a healthy control group. The patients having either of the one risk factors for coronary artery disease i.e. smoking, hypertension and/or diabetes were studied. Serum lipid peroxides, superoxide dismutase, serum iron and iron binding capacity were estimated. Oxidative stress was highest in smokers with coronary artery disease (3.11+/-0.79 mmol/ml) as compared to hypertensives (2.69+/-0.20 mmol/nl) and non-insulin dependent diabetics (2.78+/-0.19 mmol/ml). Superoxide dismutase activity was also significantly decreased (p<0.001) in smokers with coronary artery disease as compared to hypertensives and non-insulin dependent diabetes mellitus. Final step of stepwise logistic regression based on malondialdehyde and superoxide dismutase correctly predicted coronary artery disease status in 90 percent smokers. Serum iron and total iron binding capacity were not significantly different in risk prone subjects. However, among all risk prone subjects, smokers with coronary artery disease showed highest serum iron levels and decreased iron binding capacity.

Brainstem auditory evoked potential responses in iron-deficient anemic children.

Iron deficiency is a major health problem in developing countries manifesting not only as overt anemia but also involving the CNS resulting in cognitive and behavioral deficits. Iron is an important nutrient and essential element involved in myelin formation and neurotransmitter synthesis and thus contributes to normal neurological activity. Hypomyelination has been reported in iron deficient states with possible neural conduction defects. The brainstem auditory evoked potential response is used extensively to identify lesions associated with various demyelinating diseases and hence has been used in the present study to observe the effect of iron deficiency on sensory brain function. A trend of increased absolute and interpeak latencies and reduced amplitudes of the waves leading to a definite linear correlation between the severity of anemia and the degree of neurophysiological deficit suggests a subclinical involvement of the auditory pathway in the brainstem of iron deficient children.

Red cell indices and discriminant functions in the detection of beta-thalassaemia trait in a population with high prevalence of iron deficiency anaemia.

Red cell indices and discriminant functions were studied in 463 heterozygous beta-thalassaemics (337 without iron deficiency, 126 with iron deficiency) and 195 patients of iron deficiency anaemia (IDA) to ascertain their utility in the detection of betathalassaemia trait (BTT). Majority of traits in both groups had an elevated RBC count (> or = 5.0 x 10(12)/L). The counts were significantly higher than of patients with IDA, only 4.6% of whom had this degree of erythrocytosis. Mean Hb concentration was significantly higher in traits as compared to iron deficient subjects (p < 0.0001). Mean MCV and MCH were significantly (p < 0.0001) lower in traits more so in those with ID as compared to patients of IDA. MCV < 80 fl and MCH < 27 pg were found to be sensitive markers in the detection of traits even in the presence of ID. Of the four discriminant functions studied MCSQ was found to be most sensitive in detection of BTT and it identified 97.9% traits. DF of England and Fraser was most specific for BTT being < 8.4 in only 6.2% patients with IDA. Detection of erythrocytosis especially in the presence of mild anaemia and calculation of discriminant functions derived from red cell indices were found to play an important role in screening for BTT even in the presence of ID and helped identify those patients who required further laboratory evaluation.

Haematological parameters and HbA2 levels in beta-thalassaemia trait with coincident iron deficiency.

Iron status was estimated in 463 heterozygous beta-thalassaemics to delineate the effect of iron deficiency (ID) on the haematological parameters and expression of HbA2 in these patients. One hundred and twenty six (27.2%) traits had coincident ID. These iron deficient traits had a significantly (p < 0.0002), higher prevalence of anaemia (90.5%) as compared to iron replete traits (71.5%). Mean haemoglobin concentration was significantly lower (p < 0.0001) in beta-thalassaemics with ID (10.7 +/- 1.5) g/al as compared to those without ID (11.6 +/- 1.6 g/dl). Mean MCV and MCH were significantly lowered (p < 0.0001) in patients of beta-thalassaemia trait (BTT) with ID than in these without ID. Mean HbA2 was not significantly different in the two groups of traits and was elevated (> or = 3.5%) in all except one patient. However, mean HbA2/cell was significantly (p < 0.05) lower in traits with ID. The effect of ID in BTT was apparent with significant lowering of haemoglobin concentration and increased prevalence of anaemia. Iron therapy is warranted in iron deficient traits and would help in significantly raising their haemoglobin concentration. Elevation in HbA2 values was striking and could be used with reliability for diagnosis of BTT even in the presence of ID.

Beta-thalassaemia mutations in northern India (Delhi).

The present study was undertaken to define beta-thalassaemia mutations prevalent in northern India (Delhi). Forty six children of beta-thalassaemia major and their families were investigated. DNA was extracted from leucocytes and screened for mutations prevalent in the Indian population. These mutations included 619bp deletion, IVS 1-1 (G-T), IVS 1-5 (G-C), frameshift mutations FS 8/9 (+G), FS 41/42 (-CTTT), Codon 16(-C), Codon 15 (G-A), codon 30 (G-C), IVS 1-110 (G-A) and -88 (C-T). 619 bp deletion mutation was detected directly by amplification of DNA by PCR followed by agarose gel electrophoresis. Other mutations were studied by DNA amplification and dot blot hybridization using synthetic normal and mutant oligonucleotide probes labelled at 5' end with gamma-32 P-ATP. Five mutations accounted for all the chromosomes in 46 patients. 619 bp deletion mutation was found to be the commonest mutation (34.8%) followed by IVS 1-5 (G-C) in 22.8 per cent, IVS 1-1 (G-T) in 19.6 per cent, FS 8/9 (+G) in 13 per cent and FS 41/42 (-CTTT) in 9.8 per cent. Nineteen (41.3%) patients were homozygous and 27 (58.7%) double heterozygous for different beta-thalassaemia mutations. This observation of limited number of mutations is significant and will be useful in planning strategies for prenatal diagnosis of beta-thalassaemia in northern India.

ESR and iron status in pregnancy.

ESR (Westergen) correlated significantly with the iron status (as measured by Hb concentration, haematocrit, red cell count, MCH, P/H ratio, serum iron, TIBC and percent saturation of transferrin) in a group of pregnant women (PW) at term. Serum ferritin correlated negatively with the ESR but the correlation was not statistically significant. Serum ferritin levels of < 50 micrograms/L were present in 9 (34.6%) PW with ESR > or = 50 mm 1st hour and 5 (19.2%) PW with ESR < 50 mm 1st hour. The mean ESR in PW was 55.7 (+/- 22.9) and was > or = 50 mm 1st hour in 50% and < 75 mm 1st hour in 82.7%. The difference in the mean ESR in anaemic and nonanaemic PW was highly significant (p < 0.001), 87.5% anaemic PW with serum ferritin > 50 micrograms/L had ESR > or = 50 mm 1st hour, suggesting the possible effect of chronic infection in raising ferritin levels in these PW.

Haemostatic function in coronary artery disease (CAD).

Tests to evaluate haemostatic function bleeding time (BT), prothrombin time (PT) partial thromboplastin time with kaolin (PTTK), thrombin time (TT), platelet count, platelet function tests (platelet adhesiveness and microthrombus index) and plasma fibrinogen levels were performed in 30 patients of coronary artery disease (14 myocardial infarction, 16 angina pectoris) and 20 age and sex matched controls. There was no statistically significant difference in platelet adhesiveness and mean microthrombus index in patients and controls. The BT, PT, PTTK and TT were normal in all patients and controls. Stepwise logistic regression analysis showed that plasma fibrinogen was an independent risk factor in the production of CAD.

Congenital deficiency of factor VII.

A case of congenital factor VII deficiency in a five-year-old child is reported. The patient, born of a non-consanguineous marriage, presented with repeated bouts of epistaxis since childhood. The prothrombin time (PT) was markedly prolonged with a normal bleeding time (BT), partial thromboplastin time with Kaolin (PTTK) and platelet count. The patient has been on follow up for the last four years and is doing apparently well.

Association of gastroesophageal reflux (GER) with bronchial asthma.

Eighty children with bronchial asthma and ten control cases underwent radionuclide gastroesophagography for the detection of gastroesophageal reflux. Thirty nine per cent asthmatic children demonstrated esophageal reflux on scintiscanning. The ten control subjects had no reflux. The presence of reflux correlated strongly with the presence of nocturnal exacerbation of symptoms. Bronchodilator therapy did not affect the prevalence of GER in asthmatic children.

Effect of maternal iron deficiency anaemia on foetal outcome.

One hundred and two pregnant women and their neonates were examined to evaluate the effect of maternal haemoglobin concentration (Hb. conc) and iron deficiency anaemia on the placental weight and the foetal outcome. Haematological and serum ferritin values were determined. It was observed that 34.3% of the pregnant women were anaemic. Maternal Hb conc. and serum ferritin showed a highly significant correlation (r = 0.40, p < 0.001) indicating that iron deficiency was the most important cause of anaemia amongst them. The maternal Hb conc. showed a significant correlation with placental weight (p < 0.05), birth weight (p < 0.01), Apgar score (p < 0.001) and birth asphyxia. Maternal serum ferritin also correlated positively with cord ferritin (p < 0.001). The study did not reveal any association between high Hb and adverse foetal outcome.

Erythrocyte T-activation: clinical importance & recognition by a simple routine technique.

Erythrocyte T-activation is reported in association with bacterial infections. Although it is an unfrequent phenomenon, it has been reported in cases of septicaemia and necrotizing enterocolitis. It is important to recognize these cases as, transfusion of blood & blood products and lead to haemolytic--transfusion reactions. Here we report a case of T-activation detected during routine immunohaematological procedure in the blood transfusion laboratory. This also emphasizes the role of a routine, cost-effective test to diagnose cases of T-activation.