A Case of Congenital Lymphatic Dysplasia Complicated by Hydrops Fetalis

Congenital lymphatic dysplasia is a rare congenital maldevelopment of the lymphatic system, in which dysfunction of the lymphatic system may cause leakage of lymph fluid into the limbs and the pleural, pericardial, or peritoneal cavity. We experienced a case of hydrops fetalis with subcutaneous lymphedema, chylothorax, chylous ascites and pericardial effusion. Lymphangiography revealed a critical defect of lymphatic system. Here, we report the first case of premature infant with congenital lymphatic dysplasia confirmed by lymphangiography, which is the first reported in Korea.

The Usefulness of Birth Weight Z-Score as a Predictor of Catch Up Growth in Preterm Infants

PURPOSE: The aim of the study was to examine whether the degree of fetal smallness in small for gestational age (SGA) infants would increase the risk of catch-up-growth failure at 12 and 24 months corrected age (CA), and whether the high-calorie nutritional support would improve catch-up growth between 12 and 24 months CA. METHODS: We conducted a retrospective cohort study on 103 preterm infants born between January 2010 and December 2011. Logistic regression analysis was performed to investigate whether the birth weight z-score would be an independent risk factor for catch-up growth failure at 12 or 24 months CA. Among the 46 infants with failed catch-up growth at 12 months CA, 16 infants were provided high-calorie nutritional support, including nutritional supplements and medium chain triglyceride (MCT) oil at the pediatric gastroenterology clinic. RESULTS: Of 103 preterm infants, 34 infants (33%) were SGA and 69 infants (67%) were appropriate for gestational age (AGA). One birth weight z-score decrement increased the odds for catch-up growth failure 2.9 times at 12 months CA and 3.0 times at 24 months CA after adjustment for major neonatal morbidities. The increase in z-score between 12 and 24 months CA was significantly greater in the infants provided nutritional support than in the infants who were not provided nutritional support. CONCLUSION: The birth weight z-score can be used as a predictor of catch-up growth failure. High-calorie nutritional support may improve weight gain during the second year in preterm infants with failed catch-up growth at 12 months CA.

A Case of Lactobacillus Bacteremia during Probiotic Supplementation in a Very Preterm Infant with Short Bowel Syndrome

Probiotics are living micro-organisms that beneficially affect the composition of the host intestinal microflora. In very preterm infants, probiotics reportedly help reduce necrotizing enterocolitis (NEC), invasive fugal colonization and sepsis, and enable the establishment of complete enteral feeding at an earlier stage. However, emerging evidence has indicated the risk of potential side effects of probiotic use, such as gut organism translocation, including probiotic organisms, in infants that are more premature. In the present report, we describe a case of Lactobacillus bacteremia in a very preterm infant with short bowel syndrome. Lactobacillus sepsis developed during the therapeutic use of this organism for diarrhea and diarrhea-related malabsorption. The organism isolated from the blood sample was found to be of the same strain as that administered, by using molecular techniques. The findings of the present case suggest that probiotics should be carefully used, particularly in very preterm infants with altered intestinal permeability such as short bowel syndrome.

Clinical Manifestations of Capillary Hemangiomas in Preterm Infants / 대한주산의학회잡지

PURPOSE: Capillary hemangiomas occur more frequently in preterm infants. We aimed to describe the clinical course of capillary hemangiomas in preterm infants. METHODS: The records of preterm infants with a gestational age (GA) of <35 weeks who were admitted to two tertiary neonatal intensive care units from January 2004 to December 2013 and had capillary hemangiomas were reviewed retrospectively. Subgroup analysis of between infants of GA <30 weeks and GA 30-34+6 weeks were done and ad hoc analysis comparing study population and matched preterm infants without hemangioma for investigation of differences in clinical characteristics. RESULTS: Of the 2,772 preterm infants, 112 (4%) infants developed capillary hemangiomas. The majority (91.9 %) of them had a solitary hemangiomas with the trunk was the most commonly involved site (43%). Three quarters of the patients were treated with topical corticosteroid, propranolol or laser treatment. When we divided this population as who were born before or after GA 30 weeks, there was no difference at postmenstrual age (PMA) of onset of capillary hemangiomas (median [IQR], 36(+4) [30(+5)-40(+5)] vs. 36+2 [33(+6)-41(+1)] weeks, P = 0.275). The age at involution of capillary hemangiomas was also not differ between two groups (median [IQR], 7.75 [3.75-12.25] vs. 7.5 [4-13.75] months, P=0.425). There were no statistical differences between preterm infants with capillary hemangiomas and their age, weight and sex matched control preterm infants without hemangiomas in the neonatal and maternal factors. CONCLUSION: The development of capillary hemangiomas occurred at approximately 36 to 37 weeks of PMA regardless of prematurity in preterm infants. Capillary hemangiomas of preterm infants resolved spontaneously and disappear completely by around 7 months of corrected age.

Alternative Isoforms of TonEBP with Variable N-termini are Expressed in Mammalian Cells

Hypertonicity imposes a great deal of stress to cells since it causes rise in cellular ionic strength, which can be reduced by the accumulation of compatible osmolytes. TonEBP plays a central role in the cellular accumulation of compatible osmolytes via transcriptional stimulation of membrane transporters and aldose reductase. Alternatively spliced forms of TonEBP mRNA have previously been reported and two of them showed different transcriptional activity. In the present study, isoform-specific antibodies were produced to confirm the translation of the spliced mRNA to protein. TonEBP was immunoprecipitated by using anti-TonEBP antibody and then immunoblotted using anti-TonEBP or isoform specific antibodies to find out the expression profile of TonEBP isoforms in basal or stimulated condition. From these results, we conclude that all TonEBP isoforms are expressed in mammalian cells and their expression patterns are not same in every cells.