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.

Universal salt iodization is successful in Kashmiri population as iodine deficiency no longer exists in pregnant mothers and their neonates: Data from a tertiary care hospital in North India.

INTRODUCTION: Normal pregnancy results in a number of important physiological and hormonal changes that alter thyroid function. In pregnancy, the thyroid gland being subjected to physiological stress undergoes several adaptations to maintain sufficient output of thyroid hormones for both mother and fetus. Consequently, pregnant women have been found to be particularly vulnerable to iodine deficiency disorders (IDD), and compromised iodine status during pregnancy has been found to affect the thyroid function and cognition in the neonates. OBJECTIVES: Two decades after successful universal salt iodization (USI) in the country, there is scarce data on the iodine status of the pregnant women and their neonates. This is more relevant in areas like Kashmir valley part of sub-Himalayan belt, an endemic region for IDD in the past. The objective was to estimate Urinary Iodine status in pregnant women, the most vulnerable population. MATERIALS AND METHODS: We studied thyroid function [free T3 (FT3), T3, free T4 (FT4), T4, thyroid stimulating hormone (TSH)] and urinary iodine excretion (UIE) in the 1(st), 2(nd), and 3(rd) trimesters and at early neonatal period in neonates in 81 mother-infant pairs (hypothyroid women on replacement) and compared them with 51 control mother-infant pairs (euthyroid). RESULTS: Mean age of cases (29.42 + 3.56 years) was comparable to that of controls (29.87 + 3.37 years). The thyroid function evaluation done at baseline revealed the following: FT3 2.92 ± 0.76 versus 3.71 ± 0.54 pg/ml, T3 1.38 ± 0.37 versus 1.70 ± 0.35 ng/dl, FT4 1.22 ± 0.33 versus 1.52 ± 0.21 ng/dl, T4 9.54 ± 2.34 versus 13.55 ± 2.16 µg/dl, and TSH 7.92 ± 2.88 versus 4.14 ± 1.06 µIU/ml in cases versus controls (P > 0.01), respectively. The 2(nd) to 6(th) day thyroid function of neonates born to case and control mothers revealed T3 of 1.46 ± 0.44 versus 1.48 ± 0.36 ng/dl, T4 of 12.92 ± 2.57 versus 11.76 ± 1.78 µg/dl, and TSH of 3.64 ± 1.92 versus 3.82 ± 1.45 µIU/ml, respectively. DISCUSSION: UIE was similar (139.12 ± 20.75 vs. 143.78 ± 17.65 µg/l; P = 0.8), but TSH values were higher in cases (7.92 ± 2.88) as compared to controls (4.14 ± 1.06). Although UIE gradually declined from 1(st) trimester to term, it remained in the sufficient range in both cases and controls. Thyroid function and UIE was similar in both case and control neonates. CONCLUSION: We conclude that pregnant Kashmiri women and their neonates are iodine sufficient, indicating successful salt iodization in the community. Large community-based studies on thyroid function, autoimmunity, malignancies, etc., are needed to see the long-term impact of iodization.

Snake bite envenomation in children in Kashmir.

A three year retrospective study was done to study snakebite envenomation among Kashmiri children. Ten children were admitted with snakebite. Snakebite was vasculotoxic in seven and mixed in two. Levantine viper bites were seen in five who had Grade 3 bites with severe local signs, severe coagulopathy and hypotension. Both environmental risk and seasonal incidence was observed.

Klebsiella brain abscess in a neonate.

Klebsiella pneumoniae is rarely associated with neonatal cerebral abscess. A case of Klebsiella brain abscess in a neonate is described. Diagnosis of abscess was confirmed by magnetic resonance imaging and ultrasound-guided aspiration.