Vitamin D status correlates with the markers of cystic fibrosis-related pulmonary disease.

BACKGROUND: The prevalence of Vitamin D deficiency remains high in cystic fibrosis despite daily supplementation. Vitamin D as an immunomodulator has been related to lower respiratory tract infections in children. The present study was undertaken to examine the association between vitamin D status and markers of cystic fibrosis-related pulmonary disease including exacerbations, bacterial colonization and pulmonary function. METHODS: The study includes review of records of 51 cystic fibrosis patients. Baseline patient variables and serum vitamin D levels were recorded. Based on vitamin D levels study patients were divided into three groups: vitamin-D sufficient (≥20 ng/mL), vitamin-D insufficient (12 to 20 ng/mL), and vitamin D-deficient (≤12 ng/ml). RESULTS: The proportion of children with deficient, insufficient and sufficient vitamin D levels were 47.1%, 15.7%, and 37.2%, respectively. Female sex, bacterial colonization and a greater number of exacerbations were associated with highest odds of developing vitamin D deficiency in patients with CF with 1.77 (0.22-4.61) (p = 0.002), 2.9(0.57-14.82) (p = 0.011), and 5.12 (1.28-20.50) (p = 0.021) respectively. The comparison of vitamin-D levels taken during exacerbations, colonization and during routine follow-up were significant [16.04 (7.42-27.91), 24.3 (15.5-32.4) and 48.54 (18.37-78.7) ng/ml, p < 0.001]. The FEV1 was determined in 24 patients; the comparison was significant between vitamin D-deficient and -sufficient groups [0.75 (0.717-0.777) vs. 0.82 (0.74-0.92) p < 0.05]. CONCLUSION: We concluded that vitamin D deficiency was highly prevalent in children with CF, despite daily supplementation of the vitamin in diet. Further, vitamin D deficiency was associated with a higher rate of pulmonary exacerbations and higher incidence of pulmonary bacterial colonization. In addition, in younger patients, low vitamin D levels were associated with reduced pulmonary function.

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.