Seong-Chul Kim

Complications and risk factors of infection in pediatric hemato‐oncology patients with totally implantable access ports (TIAPs)

Totally implantable access ports (TIAPs) are widely used in pediatric hematology–oncology patients. We investigated the incidence of complications, causes of TIAP removal, and risk factors for infection.

Secretin-stimulated MRCP.

Secretin-stimulated magnetic resonance cholangiopancreatography not only facilitate the depiction of anatomic variations or morphologic changes of the pancreatic duct in the normal and diseased pancreas but also help assessing functional abnormalities of the exocrine pancreas. In this article, we illustrate findings of normal pancreas and various pancreatic diseases on magnetic resonance cholangiopancreatography after secretin stimulation.

Congenital intrahepatic arterioportal and portosystemic venous fistulae with jejunal arteriovenous malformation depicted on multislice spiral CT

We report a symptomatic infant with very rare congenital arterioportal and portosystemic venous fistulae in the liver. Multislice CT after partial transcatheter embolisation revealed not only the complicated vascular architecture of the lesion, but also an incidental jejunal arteriovenous malformation which explained the patient’s melena. The patient underwent ligation of the hepatic artery and resection of the jejunal arteriovenous malformation. Postoperative multislice CT clearly demonstrated the success of the treatment.

Three Novel Pathogenic Mutations in KATP Channel Genes and Somatic Imprinting Alterations of the 11p15 Region in Pancreatic Tissue in Patients with Congenital Hyperinsulinism

Background/Aims: This study was performed to investigate the molecular pathology underlying focal and diffuse congenital hyperinsulinism (CHI). Methods: The ABCC8 and KCNJ11 genes were analyzed in 3 patients with focal CHI and in 1 patient with diffuse CHI. Immunohistochemistry, real-time PCR, methylation-specific multiplex ligation-dependent probe amplification (MS-MLPA) and microsatellite marker analyses of the 11p15 region were performed on both normal tissues and adenomatous hyperplasia lesions. Results: The 3 patients with focal CHI harbored paternally inherited ABCC8 or KCNJ11 mutations. Compound heterozygous ABCC8 mutations were identified in the patient with diffuse CHI. In the 3 patients with focal CHI, homozygous ABCC8 or KCNJ11 mutations were identified within the lesions. MLPA and real-time PCR revealed the presence of two copies of 11p15. MS-MLPA and microsatellite analyses demonstrated abnormal imprinting patterns and focal loss of maternal 11p13-15 within the lesions. In contrast, parental heterozygosity was preserved in the normal tissue. In the patient with diffuse CHI, the two ABCC8 mutations were conserved, and imprinting patterns at 11p15 were normal. Conclusions: The epigenetic alteration at the 11p15 region plays a central role in developing focal CHI by paternally derived mutations of the KATP channel and maternal allelic loss at this region. MS-MLPA and microsatellite analyses are useful to investigate the molecular etiology of CHI.

Trends in Treatment Outcome and Critical Predictors of Mortality for Congenital Diaphragmatic Hernia in a Single Center

Objective: To review trends in treatment outcomes, to determine risk factors for mortality, and to present a cut-off values of observed/expected lung to head ratio (O/E LHR) predicting mortality in infants with congenital diaphragmatic hernia (CDH). Methods: A retrospective study of infants with CDH treated in the neonatal intensive care unit at tertiary medical center over 27 years (1989 to 2016) was done. We analyzed the factors associated with mortality and based on significant factors in predicting the mortality, the best cut-off value of O/E LHR was sought. Results: A total of 264 patients were included for the study. The mean gestational age and birth weight were 38.0±2.2 weeks and 2,934.2±578.2 g, respectively. Seventy nine patients (29.9%) died. The range of yearly mortality rate varied from 0 to 67%. Prenatal, neonatal, and surgical factors associated to mortality included time of prenatal diagnosis, polyhydramnios, O/E LHR, associated major congenital anomalies (cardiac, gastrointestinal or chromosomal anomalies), gestational age, birth weight, Apgar scores, initial pH and pCO2, and number of herniated organs. Among 62 fetuses whose O/E LHR were available, O/E LHR and major congenital anomalies were independent risk factors for mortality (area under curve=0.918), fetuses with O/E LHR <44% without congenital anomalies showed predicted mortality of 72% and fetuses with O/E LHR ≥44% without congenital anomalies showed expected mortality of 7%. Conclusion: The mortality of Infants with CDH has not improved despite advance of neonatal care. The O/E LHR and the presence of major congenital anomalies could be used adjunctively when counseling parents about the prognosis.

Erratum to: Secretin-stimulated MRCP

Erratum to: Secretin-stimulated MRCP N. J. Lee, K. W. Kim, T. K. Kim, M. H. Kim, S. Y. Kim, M.-S. Park, A. Y. Kim, H. K. Ha, P. N. Kim, M.-G. Lee Department of Radiology, Asan Medical Center, University of Ulsan College of Medicine, 388-1, Pungnap 2-dong, Songpa-gu, Seoul 138-736, Korea Department of Radiology, Eulgi Medical Center, 280-1, Hagye-dong, Nowon-gu, Seoul 139-711, Korea Department of Medical Imaging, Toronto General Hospital, 200 Elizabeth St, Toronto, Ontario, Canada M5G2C4 Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine, 388-1, Pungnap 2-dong, Songpa-gu, Seoul 138-736, Korea Department of Radiology, Yong Dong Severance Hospital, 146-92 Dokok-dong, Kangnam-ku, Seoul 137-270, Korea

The Experience of Operative Management in Jejunoileal Atresia

Purpose: Intestinal atresia is a common cause of neonatal intestinal obstruction. Recently, the survival rate has been increasing from development of prenatal diagnosis, total parenteral nutrition (TPN) and neonatal intensive care. We evaluated the complication rate and cause of mortality after operative management for jejunoileal atresia. Methods: We reviewed 62 patients (36 males, 26 females) with jejuno-ileal atresia who underwent operation from 1998 to 2007. Results: There were 37 patients with jejunal atresia and 25 with ileal atresia. The average gestational age was 256±16.6 days and birth weight was 2,824±620 g. Prenatal diagnosis was performed in 45 patients (72.6%) around gestational age 27 weeks. Within 2nd day after birth, 44 patients (71%) underwent operation. Half of the jejunoileal atresia was type IIIa and type I was in 8, type II was in 3, type IIIb was in 12, and type IV was in 8. The operative treatment was resection & anastomosis in 59 patients and enterotomy & web excision in 3. They started feeding at 12.4±11.5 days after operation on average. The average duration of TPN was 26.7±23.5 days, and the incidence of cholestasis was 30.6%. Hospital days averaged 36.8±26 days. Early complication occurred in 14 patients (intestinal obstruction in 5, sepsis in 4, wound problem in 3, anastomosis leakage in 1, and intraabdominal abscess in 1). Late complication occurred in 7 patients (anastomosis stricture in 4 and intestinal obstruction in 3). There was only one case of mortality due to short bowel syndrome after re-operation for adhesive ileus. Conclusion: The operation for intestinal atresia was successful and aggressive management contributed to a low mortality rate. (J Korean Surg Soc 2010;79:300-305)