Ho Sun Eun

High Incidence of Rickets in Extremely Low Birth Weight Infants with Severe Parenteral Nutrition-Associated Cholestasis and Bronchopulmonary Dysplasia

Risk factors for rickets of prematurity have not been re-examined since introduction of high mineral formula, particularly in ELBW infants. We analyzed the incidence and the risk factors of rickets in extremely low birth weight (ELBW) infants. As a retrospective case-control study from 2004 to 2008, risk factors were analyzed in 24 patients with rickets versus 31 patients without. The frequency of rickets in ELBW infants was 24/55 (44%). Infants with rickets were diagnosed at 48.2 ± 16.1 days of age, and improved by 85.3 ± 25.3 days. By radiologic evaluation, 29% were grade 1 rickets, 58% grade 2 and 13% grade 3. In univariate analysis, infants with rickets had significantly higher incidence of patent ductus arteriosus, parenteral nutrition associated cholestasis (PNAC), severe PNAC and moderate/severe bronchopulmonary dysplasia (BPD). In multiple regression analysis, after adjustment for gestation and birth weight, rickets significantly correlated with severe PNAC and with moderate/severe BPD. Serum peak alkaline phosphatase levels were significantly elevated in rickets (P < 0.001). In ELBW infants, the incidence of rickets of prematurity remains high and the incidence of severe PNAC and moderate/severe BPD was significantly increased 18 and 3 times, respectively.

Parenteral Nutrition Associated Cholestasis Is Earlier, More Prolonged and Severe in Small for Gestational Age Compared with Appropriate for Gestational Age Very Low Birth Weight Infants

Purpose We hypothesized that parenteral nutrition associated cholestasis (PNAC) would be more severe in small for gestational age (SGA) compared with appropriate for gestational age (AGA) very low birth weight (VLBW) infants. Materials and Methods Sixty-one VLBW infants were diagnosed as PNAC with exposure to parenteral nutrition with elevation of direct bilirubin ≥2 mg/dL for ≥14 days. Twenty-one SGA infants and 40 AGA infants matched for gestation were compared. Results Compared with AGA infants, PNAC in SGA infants was diagnosed earlier (25±7 days vs. 35±14 days, p=0.002) and persisted longer (62±36 days vs. 46±27 days, p=0.048). Severe PNAC, defined as persistent elevation of direct bilirubin ≥4 mg/dL for more than 1 month with elevation of liver enzymes, was more frequent in SGA than in AGA infants (61% vs. 35%, p=0.018). The serum total bilirubin and direct bilirubin levels during the 13 weeks of life were significantly different in SGA compared with AGA infants. SGA infants had more frequent (76% vs. 50%, p=0.046), and persistent elevation of alanine aminotransferase. Conclusion The clinical course of PNAC is more persistent and severe in SGA infants. Careful monitoring and treatment are required for SGA infants.

The efficacy and safety of Montelukast sodium in the prevention of bronchopulmonary dysplasia

Purpose The purpose of this study was to evaluate the efficacy and safety of Montelukast sodium in the prevention of bronchopulmonarydysplasia (BPD). Methods The Interventional study was designed as a multicenter, prospective, and randomized trial, with open labeled and parallel-experimental groups, 66 infants were enrolled and allocated to either the case group (n=30) or the control group (n=36) based on gestational age (GA). Infants in the case group were given Montelukast sodium (Singulair) based on their body weight (BW). Zero week was defined as the start time of the study. Results The incidence of moderate to severe BPD was not different between the groups (case group: 13 of 30 [43.3%] vs. control group: 19 of 36 [52.8%], P=0.912). Additionally, secondary outcomes such as ventilation index, mean airway pressure and resort to systemic steroids were not significantly different. There were no serious adverse drug reactions in either group, and furthermore the rate of occurrence of mild drug related-events were not significantly different (case group: 10 of 42 [23.8%] vs. control group: 6 of 48 (15.8%), P=0.414). Conclusion Montelukast was not effective in reducing moderate or severe BPD. There were no significant adverse drug events associated with Montelukast treatment.

Effective Tidal Volume for Normocapnia in Very-Low-Birth-Weight Infants Using High-Frequency Oscillatory Ventilation

Purpose Removal of CO2 is much efficient during high-frequency oscillatory ventilation (HFOV) for preterm infants. However, an optimal carbon dioxide diffusion coefficient (DCO2) and tidal volume (VT) have not yet been established due to much individual variance. This study aimed to analyze DCO2 values, VT, and minute volume in very-low-birth-weight (VLBW) infants using HFOV and correlates with plasma CO2 (pCO2). Materials and Methods Daily respiratory mechanics and ventilator settings from twenty VLBW infants and their two hundred seventeen results of blood gas analysis were collected. Patients were treated with the Dräger Babylog VN500 ventilator (Drägerwerk Ag & Co.) in HFOV mode. The normocapnia was indicated as pCO2 ranging from 45 mm Hg to 55 mm Hg. Results The measured VT was 1.7 mL/kg, minute volume was 0.7 mL/kg, and DCO2 was 43.5 mL2/s. Mean results of the blood gas test were as follows: pH, 7.31; pCO2, 52.6 mm Hg; and SpO2, 90.5%. In normocapnic state, the mean VT was significantly higher than in hypercapnic state (2.1±0.5 mL/kg vs. 1.6±0.3 mL/kg), and the mean DCO2 showed significant difference (68.4±32.7 mL2/s vs. 32.4±15.7 mL2/s). The DCO2 was significantly correlated with the pCO2 (p=0.024). In the receiver operating curve analysis, the estimated optimal cut-off point to predict the remaining normocapnic status was a VT of 1.75 mL/kg (sensitivity 73%, specificity 80%). Conclusion In VLBW infants treated with HFOV, VT of 1.75 mL/kg is recommended for maintaining proper ventilation.

Nutritional Outcomes in Children with Epidermolysis Bullosa: The Experiences of Two Centers in Korea

Purpose Epidermolysis bullosa (EB) is associated with variable risks of extracutaneous manifestations and death. Currently, there is limited information on the clinical course and prognosis of EB in Korea. This study analyzed the nutritional outcomes, clinical morbidity, and mortality of children with EB. Materials and Methods Thirty patients, admitted to Severance Hospital and Gangnam Severance Hospital, from January 2001 to December 2011, were retrospectively enrolled. All patients were diagnosed with EB classified by dermatologists. Results Among the 30 patients, 5 patients were diagnosed with EB simplex, four with junctional EB, and 21 with dystrophic EB. Wound infection occurred in 47% of the patients, and blood culture-proven sepsis was noted in 10% of the patients. Two (9.2%) patients had esophageal stricture and 11 (52.4%) of the dystrophic EB patients received reconstructive surgery due to distal extremity contracture. There were five mortalities caused by sepsis, failure to thrive, and severe metabolic acidosis with dehydration. According to nutrition and growth status, most of the infants (97%) were born as appropriate for gestational age. However, at last follow-up, 56% of the children were below the 3rd percentile in weight, and 50% were below the 3rd percentile in weight for height. Sixty percent of the children had a thrive index below -3. Conclusion Postnatal growth failure is a serious problem in children with EB. Strategies to maximize nutritional support could alleviate growth failure in children with EB, and thus improve clinical outcomes.