• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.7 no.1
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• pp.61-73
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• 2004

Purpose: Liver function test abnormalities have been reported frequently in patients receiving total parenteral nutrition (TPN). In adults, it is known that liver complications decrease with the use of cyclic parenteral nutrition (CPN), especially if the shift to cycling was not too late. However, there are few studies about the effects of cycling on liver injury in children beyond the neonatal period. The aim of this study is to evaluate the effect of the early use of CPN on total parenteral nutrition induced hepatic dysfunction. Methods: Twelve sets of CPN in 11 children (2 months to 17 years) were included in this study. Data on underlying diseases, age, length of time on TPN, macronutrient intake, complications, and biochemical parameters were collected from clinical records. All children had received CPN in the early period of persistent transaminase elevation or cholestasis complicated by previous continuous PN. The duration of infusion off-time in CPN was 2 hours in patients less than 3 months of age and 4 hours in the older children. Results: All 12 cases showed elevated aminotransferase and 5 of them also showed cholestasis. Serum total bilirubin concentration was normalized in all 5 cases with median periods of 8 days (p<0.05) after initiation of CPN. ALT either decreased significantly or was normalized in all cases with median periods of 30 days (p<0.05) on CPN. The CPN was well tolerated without significant complication except for one case of hyperglycemia. Conclusion: The early use of cyclic parenteral nutrition had a beneficial effect in improving hepatic dysfunction complicated by TPN in children.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.17 no.2
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• pp.69-76
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• 2017

Citrin deficiency is an autosomal recessive disorder caused by mutations in the SLC25A13 gene on chromosome 7q21.3, and a type of urea cycle disorder that causes hyperammonemia. Although neonatal intrahepatic cholestasis and adult-onset type II citrullinemia, a type of citrin deficiency, have been described well in many articles for several decades, failure to thrive and dyslipidemia caused by citrin deficiency (FTTDCD), the other type of citrin deficiency, has been only identified recently. There was previously no case report about FTTDCD in Korea. Patients with FTTDCD could present with loss of appetite, fatigue, failure to thrive, hypoglycemia, hypercitrullinemia, dyslipidemia, and an increased lactate/pyruvate ratio. Routine evaluation may not reveal the cause of hypoglycemia caused by citrin deficiency. We recently had a case that presented with recurrent hypoglycemia in a 30-month-old boy. Chemistry profiling, urine organic acid analysis, plasma acylcarnitine analysis, and hormone studies indicated values within the normal range or non-specific findings. Mutation analysis to identify the cause of hypoglycemia identified the subject as a compound heterozygote carrying each of the c.852_855del ($p.Met285Profs^*2$), and c.1177+1G>A mutant alleles. We report here on this unusual case of citrin deficiency presenting with FTTDCD for the first time in Korea.

• Clinical and Experimental Pediatrics
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• v.52 no.11
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• pp.1216-1220
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• 2009

Purpose:To analyze and compare various cases in which peritoneal drainage was used as the primary treatment method in preterm infants with intestinal perforation. Methods:Among the preterm infants of less than 28 weeks of gestation who were admitted to the neonatal intensive care unit (NICU) at the Gangnam Severance Hospital from April 2006 to April 2009, 7 who had developed intestinal perforation were studied retrospectively. We investigated the clinical characteristics, secondary operation performances, morbidities, complications, and mortalities. Results:Among the 7 infants, 5 survived. Of the 5 cases, 3 received laparotomy, of which 2 were confirmed as having necrotizing enterocolitis. Of the 2 infants who died, 1 had received laparotomy before 48 h of peritoneal drainage, while the other had not received any subsequent treatment. Of the 7 children, 4 had patent ductus arteriosus (PDA), of which 3 had received indomethacin injection. Five infants had begun enteral feeding before they developed intestinal perforation. Of the 5 infants who survived, 4 were diagnosed with cholestasis. Of the 7 infants, 4 developed periventricular leukomalacia (PVL) and 3 developed rickets. Conclusion:Although the use of peritoneal drainage as the primary management of intestinal perforation in preterm infants is controversial, we suggest that it can be used for treating extreme premature neonates. Further randomized controlled study will be required to determine the feasibility of using this method.

• Clinical and Experimental Pediatrics
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• v.48 no.7
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• pp.711-715
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• 2005

Purpose : With the recent improved survival of extremely low birth weight infants(ELBWI), enteral feeding has become a major issue. This study investigates the effects of early enteral feeding in ELBWI on their morbidity, duration of hospitalization, and mortality. Methods : ELBWI admitted to the neonatal intensive care unit at Samsung Medical Center from November 1994 to April 2004 who survived more than 14 days were enrolled. ELBWI were divided into two groups : an early feeding group(EF), in which enteral feeding was started within 3 days after birth; and a late feeding group(LF), in which enteral feeding was started beyond 3 days after birth. 80 ELBWI came under EF, and 131 ELBWI under LF. Results : Birth weight and gestational age did not differ between the two groups. In EF, the time to achieve full enteral feeding and the duration of parenteral nutrition were significantly shorter than in LF. The incidence of bronchopulmonary dysplasia was significantly lower in EF, but the incidences of sepsis, necrotizing enterocolitis, and cholestasis were not different between the two groups. There was no difference in the survival rate between the two groups, but the duration of hospitalization was significantly shorter in EF. Conclusion : Early enteral feeding in ELBWI did not increase the incidence of necrotizing enterocolitis and sepsis, but rather decreased the incidence of bronchopulmonary dysplasia and shortened the duration of hospitalization.

• Clinical and Experimental Pediatrics
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• v.52 no.3
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• pp.295-302
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• 2009

Purpose : To report our experience of gastrointestinal (GI) operations (OP) performed in very low birth weight infants (VLBWI) and to evaluate their clinical characteristics. Methods : Among the 1,117 VLBWI admitted to the SMC neonatal intensive care unit from November 1994 to February 2007, the medical records of 37 infants who underwent GI OP (except inguinal hernia OP) and 1,080 VLBWI without GI OP were retrospectively reviewed. Results : The mean gestational age ($27^{+6}2^{+3}$ vs. $28^{+5}{\pm}$2^{+6}$) and birth weight (979${\pm}$241 g vs. 1,071${\pm}$271 g) of the 37 VLBWI who underwent the GI OP was lower than the VLBWI without GI OP group (n=1,080). Mortality rates in the GI OP group were significantly higher than in the non GI OP group (28% vs. 15%, P<0.001). The incidence of cholestasis, retinopathy of prematurity and periventricular leukomalacia were higher in the GI OP group than in the non GI OP group, but the incidence of bronchopulmonary dysplasia was not significantly different between the GI OP group and the non GI OP group. For GI OP indications, focal intestinal perforation was most common and showed a more favorable outcome than necrotizing enterocolitis. Compared with an earlier 7-year period, 1994-2000, the incidence and survival rates increased in the subsequent 2001-2007 period. Conclusion : GI OP was associated with high mortality and morbidity in VLBWI. Further efforts to improve outcomes of GI OP in VLBWI should be investigated to improve the quality of care in VLBWI.

• Clinical and Experimental Pediatrics
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• v.45 no.11
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• pp.1381-1388
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• 2002

Purpose : Magnetic resonance cholangiopancreatography(MRCP) is a noninvasive method for imaging the pancreaticobiliary tree. The aim of this study was to evalute the usefulness of MRCP for the diagnosis of pancreaticobiliary diseases in children. Methods : From October 1996 to May 2001, 67 patients with obstructive jaundice and three patients with chronic recurrent pancreatitis were evaluated with abdominal ultrasonography and MRCP. The final diagnosis was based on the operative and pathologic findings with biopsy specimen including clinical and laboratory findings. Results : A total of 70 patients, consisting of 31 males and 39 females, with a mean age of $2.6{\pm}3.3$ years were studied. The final diagnosis was biliary atresia in 25, neonatal cholestasis in 18, choledochal cyst without anomalous pancreatobiliary duct union(APBDU) in nine, choledochal cyst with APBDU in seven, cholestatic hepatitis in five, chronic recurrent pancreatitis in three, sclerosing cholangitis in two, and secondary biliary cirrhosis in one case. The overall diagnostic accuracy of abdominal ultrasonography was 75.7% and that of MRCP was 97.1%. The sensitivity and specificity of MRCP were 100% and 98% for biliary atresia, 87.5% and 100% for choledochal cyst with APBDU, 100% and 100% for choledochal cyst without APBDU, sclerosing cholangitis and chronic recurrent pancreatitis, respectively. Conclusion : MRCP is a fast, non-invasive and reliable method for diagnosing pancreaticobiliary diseases in children and will be the standard diagnostic procedure in the future.