Pulmonary sequestration (PS) is a rare congenital malformation of the lower respiratory tract. The anomaly is characterized by absence of communication with the tracheobronchial tree and isolated blood supply from an anomalous systemic vessels. With the utilization of antenatal ultrasound, the diagnosis of asymptomatic neonatal PS has increased. Treatment options include observation, arterial embolization and surgical resection. The aim of the present study is to review the clinical course of PS and to share our experience with thoracoscopic resection. A total of 96 patients with PS were treated at Asan Children's Hospital between 1999 and 2010. The diagnosis of PS was established by CT in the cases managed by observation or embolization, and by tissue pathology in the surgical cases. Medical records and radiographic images were retrospectively reviewed. Thirty-nine patients were managed by embolization and 30 patients by surgery. The remaining 27 patients have been under observation without any procedures. Among 27 observation patients, 1 patient regressed completely and 10 patients were lost to follow up. Of the 39 embolizations patients, 2 had their lesion regress and sepsis was suspected after embolization. In 1 patient, the microcoil migrated to the iliac artery during the embolization procedure, and another patient developed renal abscess caused by renal artery embolization. Among 30 surgical cases, resection by thoracotomy was performed in 27 at the Department of Thoracic Surgery, and thoracoscopic resection in 3 at the Division of Pediatric Sugery. Only one wound complication ocurred. We conclud that surgical excision should be recommended for pulmonary sequestration, whether the sequestration is symptomatic or not because of the risk of infection, the low rate of natural regress, poor compliance, severe complications after embolization, and to exclude other pathology. In summary, thoracoscopic resection of the pulmonary sequestration is feasible, efficacious, safe and cosmetically superior even in neonatal period.
Lead (Pb) exposure during development can produce neurological deficits. In this study, the effect of Pb exposure during neonatal development via lactation on anxiety of brain function was investigated. Long-Evans strain rats were raised through two generations. At the birth of the second generation, the dams were subdivided into two groups and supplied drinking water containing either $0.2\%$ Pb (Pb-treated group) or sodium (Na, Control group) acetate until weaning. Rats were sacrificed at 3 (weaning) and 11 weeks (maturity) for brain Pb and fatty acid analysis. Motor activity and elevated plus maze tests were initiated at 9 weeks. The brains in the Pb-treated group at weaning and maturity contained 1486$\pm$98 and $270{\pm}46$ ng Pb/g, respectively The control group showed the background level of Pb ($3.7{\pm}1.0_{ng}$ Pb/g) in both ages. The alterations in brain fatty acid composition induced by Pb exposure were more evident in 3 wks old than 11 wks old. For example, in 3 wks old, the percentages of $18:2_{n-6}$, $20:2_{n-6}$ and $18:2_{n-6}$ were decreased in the Pb-treated group with an increase in $20:4_{n-6}$ In motor activity test, there was a tendency of hyperactivity in the Pb-treated group compared with the control group but the difference was not significant. In elevated plus maze test, the Pb-treated group showed fewer numbers of visits to the open arms (P < 0.05), indicating that Pb exposure may lead to anxiogenic effect.
Journal of The Korean Society of Inherited Metabolic disease
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v.18
no.2
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pp.55-61
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2018
Orinithine transcarbamylase (OTC) deficiency is the most common inborn error of the urea cycle with resulting hyperammonemia, which is medical emergency in newborns.We recently had a case of a boy that presented with lethargy, seizure, hyperammonemia and hypocalcemia in neonatal period. He was diagnosed with OTC deficiency by two consequent ways which are initial biochemical phenotype including hyperammonemia and an increased orotic acid in his urine and genetic analysis of the OTC gene. The OTC gene showed a novel hemizygous mutation c.913C>T (p.Pro305Ser). He was treated by low protein intake, sodium benzoate, phenylbutyrate sodium, L-arginine, and continuous renal replacement therapy (CRRT). After discharge, he has a relatively good prognosis without notable developmental delay. For good prognosis, the duration of hyperammonemia should be shorten. And it can be reached by an early diagnosis. For early detection of OTC deficiency, targeted exome sequencing will be a important role as well as biochemical tests.
Objective: The aim of this study was to investigate the effects of dietary supplementation with a multi-strain probiotic (MSP) product containing of Bifidobacterium animalis, Lactobacillus casei, Streptococcus faecalis, and Bacillus cerevisiae on growth, health, and fecal bacterial composition of dairy calves during the first month of life. Methods: Forty Holstein calves (24 female and 16 male) at 2 d of age were grouped by sex and date of birth then randomly assigned to 1 of 4 treatments: milk replacer supplementation with 0 g (0MSP), 2 g (2MSP), 4 g (4MSP), and 6 g (6MSP) MSP per calf per day. Results: Supplementation of MSP did not result in any significant differences in parameters of body measurements of calves during the 30 d period. As the dosage of MSP increased, the average daily gain (p = 0.025) and total dry matter intake (p = 0.020) of calves showed a linear increase. The fecal consistency index of the 2MSP, 4MSP, and 6MSP group calves were lower than that of the 0MSP group calves (p = 0.003). As the dosage of MSP increased, the concentrations of lactate dehydrogenase (p = 0.068) and aspartate aminotransferase (p = 0.081) in serum tended to decrease, whereas the concentration of total cholesterol increased quadratically (p = 0.021). The relative abundance of Dorea in feces was lower (p = 0.011) in the 2MSP, 4MSP, and 6MSP group calves than that in the 0MSP group calves. The relative abundance of Dorea (p = 0.001), Faecalibacterium (p = 0.050), and Mitsuokella (p = 0.030) decreased linearly, whereas the relative abundance of Prevotella tended to increase linearly as the dosage of MSP increased (p = 0.058). Conclusion: The MSP product can be used to reduce the diarrhea, improve the performance, and alter the composition of the fecal bacteria in neonatal dairy calves under the commercial conditions.
Biliary atresia is a rare but significant cause of neonatal cholestasis. An early and accurate diagnosis is important for proper management and prognosis. To diagnose biliary atresia, various imaging studies using ultrasonography, MRI, hepatobiliary scans, and cholangiography can be performed, although ultrasonography is more important for initial imaging studies. In this article, we review the findings of biliary atresia from various imaging modalities, including ultrasonography, MRI, hepatobiliary scans, and cholangiography. The known key imaging features include abnormal gallbladder size and shape, periportal thickening visible as a 'triangular cord' sign, invisible common bile duct, increased hepatic arterial flow, and combined anomalies. Aside from the imaging findings of biliary atresia, we also reviewed the diagnostic difficulty in the early neonatal period and the role of imaging in predicting hepatic fibrosis. We hope that this review will aid in the diagnosis of biliary atresia.
Kim, Young Ok;Kim, Sun Hui;Cho, Chang Yee;Choi, Young Youn;Kook, Jin Hwa;Hwang, Tae Ju
Clinical and Experimental Pediatrics
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v.46
no.8
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pp.769-776
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2003
Purpose : The survival rate of very low birth weight infants(VLBWI) has improved by virtue of specialized neonatal care. This study was performed to analyze the changes in incidence, survival rate and morbidity of VLBWI who admitted to Chonnam National University Hospital from 1996 to 2001. Methods : We enrolled 565 VLBWI, and compared the incidence and the survival rate according to the birth weight or gestational weeks between period I(1996 to 1998) and period II(1999 to 2001). The mortality rate according to the postnatal age, cause of death, morbidity and days of hospital stay were also compared. Morbidity is categorized into 'short term' which is curable until discharge, and 'long term' causing any types of sequelae after discharge. Results : Incidence of VLBWI significantly increased in period II over period I(6.0% vs. 11.0%, P< 0.001). The survival rate also increased in period II(71.8% vs. 80.1%, P<0.05), especially in 1,000 to 1,249 gm of birth weight(P<0.001) and in 28 to 30 weeks of gestation(P<0.001). The most common cause of death was respiratory distress syndrome in period I; however it was sepsis in period II. Although overall and short term morbidity rate increased, long term morbidity and days of hospital stay didn't increase in period II. Conclusion : Although the incidence of VLBWI significantly increased and the survival improved in period II compared to period I, especially in 1,000 to 1,249 gm of birth weight and 28 to 30 weeks of gestation, 'long term' morbidity rate and hospital days didn't increase.
Kim, Do-Hyeon;Shim, So-Yeon;Kim, Jae-Ri;Shin, Seung-Han;Kim, Eun Sun;Joung, Kyoung-Eun;Kim, Sang Duk;Lee, Jin A;Choi, Chang Won;Kim, Ee-Kyung;Kim, Han-Suk;Kim, Beyong Il;Choi, Jung-Hwan
Clinical and Experimental Pediatrics
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v.49
no.9
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pp.952-958
/
2006
Purpose : The survival rate of infants weighing less than 1,000 g at birth(extremely low birth weight infants, ELBWI) has increased due to recent advances in perinatal and neonatal intensive care. The purpose of this study was to evaluate the survival rates of ELBWI born at Seoul National University Hospital during the last six years. Methods : A total of 99 infants were divided into three groups(period I: 2000 to 2001, period II: 2002 to 2003, period III : 2004 to 2005) based on date of birth. We compared the survival rate of ELBWI over the three periods, using CRIB II score for adjustment for clinical severity. Results : Overall survival rate of ELBWI was 74.7 percent. The survival rate of ELBWI increased over the three periods(period I: 60.7 percent, period II : 73.3 percent, period III : 85.3 percent). The threshold of viability(defined as survival of at least 50 percent of infants) was 25 weeks of gestation and 600 g at birth. The birth weight-specific survival rates increased considerably over the three periods for infants <750 g at birth(period I: 10 percent, period II: 46.2 percent, period III : 70.6 percent). The survival rates of ELBWI over the three periods increased much remarkably after adjustment for clinical severity by CRIB II score. Conclusion : In our institution, survival rates of ELBWI during the last six years continued to improve, particularly for infants weighing <750 g at birth. This increase in survival rates was not associated with the clinical severity of ELBWI.
Purpose: Preterm infants are very susceptible to malnutrition because of a lack of storage of nutrients, immature digestion and metabolism, and accompanying diseases associated with prematurity. The purpose of this study was to evaluate the effects of nutritional support by the pediatric nutritional support team (pNST) on the clinical course of preterm infants in the neonatal intensive care unit (NICU). Methods: Between July 2003 and July 2006, 48 preterm infants who were admitted to the NICU at Seoul National University Bundang Hospital were included. The subjects were divided into the following two subgroups according to the presence of NST activity; pre-NST group (n=23) and NST group (n=25). Hospital records were reviewed retrospectively. Results: Forty-eight preterm babies were included (M:F=27:21; gestational age, 25~33 weeks). A dietician, pharmacists, or the pNST participated in the prescription of total parenteral nutrition (TPN) more rapidly in the NST group (p=0.000). The fasting periods and TPN administration periods were significantly decreased in the NST group compared to the pre-NST group (p=0.017 & p=0.001, respectively). The doses of calories, protein, and lipids administered via TPN were significantly increased in the NST group compared to the pre-NST group (p=0.016, p=0.000, and p=0.000, respectively). The total period on antibiotic therapy was significantly decreased in the NST group compared to the pre-NST group (p=0.007). Conclusion: Because nutritional support by the pNST is of benefit to the clinical course of preterm infants in the NICU, the pNST should recommend to improve the nutritional status and clinical outcome of preterm infants.
Baek, Hey Sung;Choi, Jae Hyung;Kim, Nam Su;Kim, Chang Ryul;Moon, Su Ji
Clinical and Experimental Pediatrics
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v.49
no.4
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pp.381-387
/
2006
Purpose : Neonatal lupus is characterized by congenital complete heart block(CCHB), cutaneous rash, and laboratory abnormalities in infants born to mothers with systemic lupus erythematosus(SLE). This study aims to examine the incidence of CCHB and clinical outcome in neonates born to mothers with SLE. Methods : The study group consisted of 49 neonates, born from 57 pregnancies of 55 women with SLE, diagnosed at Hanyang University Hospital for the period between January 1997 and January 2005. Clinical and laboratory data were retrospectively identified from medical record. Results : There were 5(8.8 percent) spontaneous abortions and one(1.8 percent) still births among 57 pregnancies of 55 mothers. Of 49 live births, 15(26.3 percent) were premature and eight(12.3 percent) were small for their gestational age. There was one(1.8 percent) CCHB suspected during pregnancy on fetal echocardiograpy in a fetus of mother with systemic lupus erythematosus and the fetus was not born by artificial abortion because of mother. There was no CCHB among EKG findings of 49 newborns. Laboratory testing showed hematologic abnormalities among 25.6 percent(10/39) of the babies. 5.1 percent(2/39) and 7.7 percent(3/39) of them were diagnosed as neutropenia, and thrombocytopenia was seen respectively. Anti-SSA(Ro) and antiphospholipid antibodies were predictive factors for prematurity(P=0.003, P=0.049). Anticardiolipin antibodies were predictive factors for ventilatory care(P=0.018). Conclusion : The incidence of CCHB among neonates born to mothers with SLE, which was measured in this study, was lower than that in earlier studies. A high incidence of hematologic abnormalities was found in our study. It is suggested that careful examination should be made of skin for the diagnosis of neonatal lupus.
Seo, Min-Ae;Lee, Hyun-Ju;Choi, Eun-Jin;Kim, Jin-Kyung;Chung, Hai-Lee;Kim, Woo-Taek
Neonatal Medicine
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v.17
no.2
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pp.181-192
/
2010
Purpose: Current studies have demonstrated the neuroprotective effects of dizocilpine (MK-801) in many animal models of brain injury, including hypoxic-ischemic (HI) encephlopathy, trauma and excitotoxicity, but limited data are available for those during the neonatal periods. Here we investigated whether dizocilpine can protect the developing rat brain from HI injury via anti-apoptosis. Methods: In an in vitro model, embryonic cortical neuronal cell culture of Sprague-Dawley (SD) rats at 18-day gestation was done. The cultured cells were divided into three groups: normoxia (N), hypoxia (H), and hypoxia treated with dizocilpine (HD). The N group was prepared in 5% $CO_2$ incubators and the other groups were placed in 1% $O_2$ incubators (94% N2, 5% $CO_2$) for 16 hours. In an in vivo model, left carotid artery ligation was done in 7-day-old SD rat pups. The animals were divided into six groups; hypoxia (N), hypoxia (H), hypoxia with sham-operation (HS), hypoxia with operation (HO), HO treated with vehicle (HV), and HO treated with dizocilpine (HD). Hypoxia was made by exposure to a 2 hour period of hypoxic incubator (92% N2, 8% $O_2$). Results: In the in vitvo and in vivo models, the expressions of Bcl-2 in the hypoxia groups were reduced compared to the normoxia group. whereas those in the dizocilpine-treated group were increased compared to the hypoxia group. However. the expressions of Bax and caspase-3 and the ratio of Bax/Bcl-2 were revealed reversely. Conclusion: Dizocilpine has neuroprotective property over perinatal HI brain injury via anti-apoptosis.
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