摘要
Background:Kashmir has a population that largely consumes polished rice which is deficient in thiamine.Furthermore,lactating women in this region are prone to severe thiamine deficiency because of their traditional food avoidance practices.Infantile beriberi is common in exclusively breastfed infants of thiamine deficient mothers in Kashmir.Methods:This was a one year prospective hospitalbased study.We included 50 exclusively breastfed infants in our study.AH patients were evaluated as per unit protocol including complete septic workup and metabolic workup.Most of our patients belonged to low and middle income group families,and mothers were on customary dietary restriction.Demographic and anthropometric data were collected from all the study participants.In addition,data regarding the treatments received by the study population and overall mortality were collected.Results:The mean age,male:female ratio,and mean weight of the study population were 3.15±0.97 months,1.5∶1,and 5.1±1.1 kg,respectively.Traditional food avoidance practices were followed by 80% of the mothers.Irritability was observed in 40 (80%) patients.Blepharoptosis was observed in 30 (60%).Septic workup including cerebro spinal fluid analysis was normal in all patients.Predominant magnetic resonance imaging finding was bilateral basal ganglia hyperintensity.Whole blood thiamine diphosphate levels showed a drastic decrease (10-49 nmol/L).Ten percent of the study infants died.Conclusion:In exclusively breasffed infants,we observed acute infantile encephalopathy with epidemiological,clinical,biochemical,and radiological features suggestive of infantile Wernicke's encephalopathy and a favourable therapeutic response to thiamine supplementation during the acute stage.
