Clinical profile of hereditary spherocytosis in North India.

AIMS OF THE STUDY: Hereditary spherocytosis (HS) is a familial hemolytic disorder manifesting as anaemia, recurrent jaundice, splenomegaly with marked heterogeneity in clinical presentation. The objective was to study the clinical spectrum of the disorder in India. METHODOLOGY: We studied 50 HS patients and followed them for up to six years (Age range 2-47 years). RESULTS: The presenting features were jaundice 35 out of 50, anaemia 30 out of 50 (requiring blood transfusion in 25). Splenomegaly was found in all patients. Increased osmotic fragility was found in all patients whereas spherocytes were found in only 19 out of 42 patients. Reduced red cell survival was noted in 9/12 patients studied with 51Cr labeled RBCs. There was a definite improvement in the hemoglobin values in those who underwent splenectomy. Thirteen cases had similarly affected family member/s. Fifteen of the cases had family history consistent with autosomal dominant (AD) inheritance (eight families) while in six cases (5 families), inheritance was likely to be autosomal recessive (AR). There was intrafamilial variability in the age of presentation in the AD families. CONCLUSIONS: Our results suggest that both autosomal dominant and recessive patterns of HS are seen in India and the clinical profile of the Indian HS patients is similar to that described in other populations. HS presenting in childhood is also not uncommon. However, the predominant underlying protein defect in Indian patients needs to be characterized.

Pharmacological actions of Pongamia pinnata seeds--a preliminary study.

Direct ethanolic and sequential petroleum ether, chloroform, acetone and ethanolic extracts (50-100 mg/kg, i.p.) of P. pinnata seeds given 30-60 min before revealed anti-inflammatory, analgesic and anti-ulcerogenic activities in rats. The activities were present maximum in petroleum ether and chloroform extracts. However, the extracts also showed shortening of pentobarbitone induced 'sleep time' in rats.

Public awareness, understanding & attitudes toward epilepsy.

The public awareness, understanding and attitudes towards epilepsy were evaluated in a north Indian population in 1992 by personal interview method. The study revealed that 92 per cent of the respondents had read or heard about epilepsy. More than 55 per cent knew someone and had seen a case of seizure. Eighty five per cent of the respondents were not aware of the cause of epilepsy or had wrong beliefs. Eighteen and 15 per cent thought epilepsy to be a hereditary disorder and a form of insanity respectively. About 40 per cent of the respondents felt that children with epilepsy should not be sent to school and also objected to their children's contact with epileptics at school or at play. Two-thirds of the respondents objected to their children marrying a person who had ever had epilepsy. Twenty per cent were ignorant about the manifestation of epilepsy and an equal number were unable to recommend any therapy in case their relatives or friends had epilepsy. Fifty seven per cent did not know what kind of first aid should be given during the epileptic attack. Although the awareness of epilepsy among Indian people was comparable to that of individuals in Western countries, the attitudes of the Indians were much more negative. Better educated people belonging to higher occupational groups were less prejudiced against social contact and schooling of their children with epileptic children compared to low educational and occupational groups.

Development of mother-infant attachment scale.

A 15 item mother-infant attachment scale was developed. It is a simple, brief and easy to comprehend even by the illiterate rural woman. The split half reliability was found to be 0.83 and there was high internal consistency. It has high face and construct validity. The babies separated for longer period had shown lesser attachment subsequently, compared to those who had no separation.

Maternal attitudes towards spastic cerebral palsy.

Attitudes of 120 mothers of spastic children aged 2-12 yrs were assessed by using Mother's Attitude Scale. Attitudes of mothers towards spastics were highly influenced by the severity of handicap and IQ of the child (r < 0.74). Mother's education, family income, occupational status, type of habitat had significant but low correlation with her attitudes (0.23 to 0.35). Age, sex of the child and type of family did not influence attitudes. Study group mothers (rural or urban) have better attitudes towards spasticity compared to control group mothers. This difference was more discernible among mothers belonging to age group below 30 years of age. The study emphasizes the necessity of constant counselling of mothers so that their attitudes may improve and help in more effective care of the child.

Comparative effects of mast cell degranulators on perfused systemic blood vessels of Bufo melanostictus and Rana tigrina.

Three agents known to induce release of mast cell constituents, viz. polymyxin, compound 48/80 and polysorbate-80, were evaluated for effect on perfused blood vessels of R. tigrina and B. melanostictus. The mast cell degranulators caused vasoconstriction in frog and toad, except that for P-80 whose responses in toad were equivocal. Toads showed a general low responsiveness in comparison to frogs. Pharmacologic intervention with pheniramine, metergoline, hydergine, atropine and mecamylamine, respectively ruled out role of histamine, 5-HT, catecholamine or acetylcholine or even autonomic mechanisms in the above phenomena. The observations are suggestive of phylogenetic differences in biochemical profile of mast cells in amphibian species.

Receptor mechanisms in vasoconstrictor responses to choline esters in Bufo melanostictus.

Choline esters have been found to cause vasoconstriction of perfused systemic blood vessels of common Indian toad, Bufo melanostictus. The vasoconstriction appears to be mediated through atropine sensitive receptors and nicotinic mechanisms appear unlikely to be involved.

Some aspects of vascular pharmacology of frog (Rana tigrina).

Vascular autonomic receptors in amphibians exhibit difference from more evolved mammalian species. Vascular perfusion studies in frog indicate constrictions by prominent muscarinic but rudimentary nicotinic constrictive regulation by cholinergic systems. Difference from classical effect-patterns of pharmacological interventions, observed in the study, make room to visualise complexity of additional regulatory mechanisms.

Impact of acute diarrhoea & oral rehydration solution on nutritional status of preschool children.

A total of 838 children under 5 yr of age were followed up in 2 villages of Punjab for a period of one year for diarrhoea morbidity, oral rehydration solution (ORS) usage and nutritional status. Overall frequency of diarrhoea was 0.78 episode/child/year. Children weighing less than or equal to 70 per cent of reference weight for age had about 25 per cent higher incidence of diarrhoea (102 episodes/100 child/yr) as compared to those who weighed greater than or equal to 71 per cent (75 episodes/100 children/yr; P less than 0.05). At the end of one year there was no significant difference in the weight and arm circumference growth of children who had diarrhoea and received ORS and those who did not. Prevalence of malnutrition (weight for age criteria) in children with and without diarrhoea at the beginning and end of the study also remained unchanged, irrespective of ORS usage. These observations indicate that in rural areas with low diarrhoea morbidity (i) pre-existing malnutrition (weight for age less than 70%) is associated with 25 per cent higher diarrhoea incidence; (ii) acute diarrhoea does not significantly affect weight and arm circumference growth and prevalence of malnutrition; and (iii) the long-term nutritional benefit of ORS if any, is doubtful.

Blood histaminase in phlyctenulosis.

Blood Histaminase estimations were done in 42 cases of phlyctenulosis and 25 normal subjects. Significantly higher level of blood Histaminase was found in patients of phlyctenulosis, which may be a consequence of its induction secondary to increased release of histamine in the early phase of disease.

Immunopharmacological studies on Picrorhiza kurroa Royle ex Benth. Part VI: Effect on anaphylactic activation events in rat peritoneal mast cells.

Mechanism of inhibition of mast cell anaphylaxis by P. kurroa-extract (PK) treatment in rats was investigated. Mast cell-IgE binding, assessed from induction of passive sensitization, was not affected. Calcium-independent early activation events in mast cell anaphylaxis indicated on inhibitory influence of PK-treatment. Inhibition of membrane-protease release by PK-treatment was suggested by study of gastric secretion and exhibition of saturable synergism with Di-isopropyl fluoro phosphate on inhibition of anaphylactic degranulation. pH-independence of mast cell stabilizing effect negates any PK-influence on phospholipid transmethylation. The results complement findings of earlier studies on indirect effects of PK through alteration of membrane structure/function.

Bioflavonoids and vasoactive mediator release from mast cells.

5-hydroxy and 7-hydroxy flavones significantly attenuated the carrageenin-induced pedal inflammation in rats; however, the anti-inflammatory effect declined at higher doses. These compounds produced positive inotropic effect on frog atria, which exhibited tachyphylaxis and was selectively abolished by indomethacin pretreatment. Studies on the rat mesenteric mast cells revealed that both the compounds, administered either in vitro or in vivo, induced a significant degree of mast cell degranulation in higher concentrations/doses. The mast cell degranulation induced by compound 48/80 was, however, prevented by lower, but not higher, concentrations/doses of the flavones. Local injection of 7-hydroxy flavone into the hind paw of rat showed that, in higher doses, the compound produced significant oedema. It was concluded that these compounds have a biphasic action: mast cell stabilisation and degranulation at lower and higher concentrations/doses, respectively.

Production of myocardial lesions in a graded fashion by noninvasive technique in albino rats.

Systematic study of producing graded lesions of myocardium with the use of Isoproterenol (IPT) reveals that mild to severe degree of infarction can be induced by administering the compound for one to four consecutive days in the dose of 85 mg/kg body weight in rats. These findings differ slightly from those of Rona et al. where they have not attempted to produce the lesions in a graded fashion. The experimental period can be reduced to two days by using high dose (170 mg/kg), but such procedure increases significantly the mortality in these animals and lesions are not produced in graded fashion.