Wernicke's encephalopathy in exclusive breastfed infants.

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 hospital-based study. We included 50 exclusively breastfed infants in our study. All 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 breastfed 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.

Shoshin beriberi-thiamine responsive pulmonary hypertension in exclusively breastfed infants: A study from northern India.

OBJECTIVE: To study the effect of thiamine administration on the resolution of pulmonary hypertension in exclusively breastfed infants. DESIGN: Prospective cohort study. SETTING: Hospital based study of a tertiary care hospital. PATIENTS: A total of 29 infants with 17 males (58.6%) and 12 females (41.4%) were included in the study. INTERVENTION: In addition to the management of shock, right heart failure and renal failure, patients received intravenous thiamine 100mg/kg IV followed by 10mg/day till introduction of supplementary feeds. MAIN OUTCOMES MEASURES: Resolution of shock, metabolic complications and pulmonary hypertension. RESULTS: Mean age at presentation was 78.45±30.7 days. All infants were exclusively breastfed. 86.2% of mothers were on customary dietary restrictions. Biventricular failure and tachycardia was commonly present. There were four deaths in our series. Acute metabolic acidosis was a universal feature with a mean pH of 7.21±0.15. Pulmonary hypertension was present in all patients on admission. Intravenous thiamine 100mg/kg IV stat was given immediately after documenting pulmonary hypertension. Repeat echocardiography showed complete resolution of pulmonary hypertension. CONCLUSION: Many infants present to us with Shoshin beriberi with unusually high pulmonary pressures. These patients respond to thiamine challenge with prompt resolution of metabolic complications and reversal of pulmonary hypertension. We believe this is first of its kind from the region, which is reported.