Intractable Vomiting in an 11-Month-Old Boy With Trisomy 21: A Case Report on Abnormal Calcium/Calcinosis/Creatinine in Down Syndrome.

Abnormal calcium/calcinosis/creatinine in Down syndrome (ABCD syndrome) is a very rare condition with no clear etiology. In this paper, we describe our clinical encounter with this disease. We report the case of an 11-month-old male infant with Down syndrome (DS) who presented to the hospital with intractable vomiting and decreased oral intake and urine output. The evaluation revealed an acute kidney injury (AKI) and hypercalcemia. Although his AKI improved with intravenous hydration, his hypercalcemia persisted. Extensive studies were notable for an elevated urinary excretion of calcium and bilateral medullary nephrocalcinosis seen on renal ultrasound (US). As a result, he was diagnosed with ABCD syndrome. Dietary calcium restriction was implemented. During his follow-up visit with a pediatric endocrinologist, his serum calcium was found to be normalized. To our knowledge, this is only the seventh case report on ABCD syndrome in the literature.

Vitamin E status and associations in maternal-infant Dyads in the Midwestern United States.

BACKGROUND: Oxidative stress has been associated with adverse neonatal outcomes, and vitamin E has powerful anti-oxidant properties. Vitamin E occurs in several different isoforms which differ in their ability to modulate inflammation and oxidative stress. Therefore, the purpose of this study was to evaluate the status of α-, γ- and δ-tocopherol in maternal-infant pairs, and the impact on maternal-newborn outcomes. METHODS: Vitamin E status was evaluated in 189 mother-infant pairs. Concentrations of α-, γ- and δ-tocopherol were measured using HPLC. Descriptive statistics were calculated and Spearman coefficients were used to assess correlations between maternal and cord measurements. Linear and logistic regression models were used to adjust for relevant confounders. A p < 0.05 was considered statistically significant. RESULTS: Maternal and cord serum tocopherol concentrations were positively correlated for γ-tocopherol (r = 0.32, p Ë‚ 0.001) and δ-tocopherol (r = 0.46, p Ë‚ 0.001) but not for α-tocopherol. After adjustment for confounders, maternal concentrations of tocopherols were positively associated with Apgar scores (p = 0.02) and infant growth parameters at birth. Conversely, cord tocopherol levels were inversely associated with Apgar scores (p = 0.02) and infant growth. Cord concentrations of α-tocopherol were higher in infants born to mothers with a diagnosis of pre-eclampsia (p = 0.04). CONCLUSION: Maternal-fetal transfer of γ- and δ-tocopherols is higher than α-tocopherol and may be mediated by either different or more efficient methods, conversely tissue uptake of α-tocopherol by the developing fetus may be higher. As serum levels of maternal tocopherols are positively associated with outcomes while higher cord levels show a negative impact, uptake and tissue deposition of vitamin E by the fetus may be crucial in growth and development. More research into the role of maternal diet, placental regulation, and fetal uptake of vitamin E tocopherols in relation to clinical outcomes is warranted.

A Comparison of Vitamin E Status and Associated Pregnancy Outcomes in Maternal⁻Infant Dyads between a Nigerian and a United States Population.

Oxidative stress is associated with adverse pregnancy outcomes, and vitamin E has powerful anti-oxidant properties with the potential to impact health outcomes. Tocopherol isomers of vitamin E differ in their ability to modulate inflammation and vary in concentration in diets containing high proportions of processed versus unprocessed foods. The purpose of this study was to compare vitamin E status and associated pregnancy outcomes (mode of delivery, chorioamnionitis, APGARs (measure of appearance, pulse, grimace, activity, respiration), gestational age at delivery, and fetal growth) between maternal⁻infant dyads in a developed and a developing nation to identify potentially modifiable differences that may impact pregnancy and neonatal outcomes and provide a way to improve maternal and neonatal health. Plasma tocopherol levels were evaluated in 189 Midwestern United States (US) mother⁻infant pairs and 99 Central Nigerian mother⁻infant pairs. Maternal and infant concentrations of α-, γ-, and δ-tocopherol were measured using HPLC with diode-array detection. Descriptive statistics were calculated and tocopherol concentrations were associated with clinical outcomes such as mode of delivery, chorioamnionitis, APGARS, and fetal growth. Alpha- and γ-tocopherol levels were higher in the US mothers, (alpha: 12,357.9 (175.23⁻34,687.75) vs. 8333.1 (1576.59⁻16,248.40) (mcg/L); p < 0.001) (gamma: 340.7 (224.59⁻4385.95) vs. 357.5 (66.36⁻1775.31) (mcg/L); p < 0.001), while δ-tocopherol levels were higher in the Nigerian mothers (delta: 261.7 (24.70⁻1324.71) vs. 368.9 (43.06⁻1886.47) (mcg/L); p < 0.001). US infants had higher γ-tocopherol levels than Nigerian infants (203.1 (42.53⁻1953.23) vs. 113.8 (0.00⁻823.00) (mcg/L); p < 0.001), while both the Nigerian mothers and infants had higher α:γ-tocopherol ratios (8.5 vs. 26.2, and 8.9 vs. 18.8, respectively; p < 0.001). Our results in both populations show associations between increased circulating γ-tocopherol and negative outcomes like Caesarian sections, in contrast to the associations with positive outcomes such as vaginal delivery seen with increased α:γ-tocopherol ratios. Growth was positively associated with α- and γ-tocopherols in cord blood in the US population, and with cord blood δ-tocopherols in the Nigerian population. Tocopherol levels likely impact health outcomes in pregnancy in a complicated metabolism across the maternal⁻fetal axis that appears to be potentially influenced by culture and available diet.

Successful management of extremely high-output refractory congenital chylothorax with chemical pleurodesis using 4% povidone-iodine and propranolol: a case report.

First-line therapy for congenital chylothorax is conservative treatment. However, surgical intervention or chemical pleurodesis is required for refractory cases. With all the concerns regarding its complications, povidone-iodin provided a successful management for a high-output congenital chylothorax. However, renal and thyroid function must be monitored during treatment.