• Clinical and Experimental Pediatrics
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• v.50 no.11
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• pp.1110-1115
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• 2007

Purpose : Idiopathic central diabetes insipidus (CDI) is defined in CDI patients without definite etiology. Some patients initially diagnosed as idiopathic CDI progressed to organic causes. We reviewed clinical, endocrinological, and radiological courses of 20 patients who was initially diagnosed as idiopathic CDI, to assess the predicting factors for progression to brain tumors. Methods : We reviewed the medical data and followed up their clinical courses in 20 CDI patients who had no definite organic etiology, such as malformation, tumor, at the time of diagnosis. Results : Our study included 15 males and 5 females. Mean age of CDI diagnosis was $7.8{\pm}3.6$ (2.1-14.7) years. Mean follow-up duration was $8.6{\pm}5.1$ (1.5-18) years. Six (30%) patients were diagnosed as brain tumor during follow-up. Ten (50%) of 20 patients had growth hormone deficiency. Multiple pituitary hormone deficiencies were found more frequently in brain tumor patients than idiopathic patients (60% vs 7%, P=0.037). Pituitary stalk thickening (PST) and loss of posterior pituitary signal were observed in 9 patients (47%), respectively. The newly development of PST was observed in patients diagnosed as brain tumor. Conclusion : About 30% of idiopathic CDI patients progress to organic disease such as germ cell tumor or histiocytosis. If there are multiple anterior pituitary hormone deficiency or newly development of PST, more close and careful follow-up is needed.

• Journal of Chest Surgery
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• v.36 no.6
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• pp.384-390
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• 2003

Background: Since Ross and Sormeville first reported the use of aortic homograft valve for correction of pulmonary atresia in 1966, homograft valves are widely used in the repair of congenital anomalies as conduits between the pulmonary ventricle and pulmonary arteries. On the basis of these results, we have used it actively. In this report, we describe our experience with the use of cryopreserved valved homograft conduits for infants and children requiring right ventricle to pulmonary artery connection in various congenital cardiac anomalies. Material and Method: Between January, 1996 and December 2001, 27 infants or children with a median age of 16 months(range 9days to 18years) underwent repair of RVOTO using homograft valved conduit by two surgeons. We studied 22 patients who have been followed up at least more than one year. The diagnosis at operation included pulmonary atresia with ventricular septal defect (n=13), truncus arteriosus (n=3), TGA or corrected TGA with RVOTO (n=6). Homograft valved conduits varied in size from 15 to 26 mm (mean, 183.82 mm). The follow-up period ranged from 12 to 80.4 months (median, 48.4 months). Result: There was no re-operation due to graft failure itself. However, early progressive pulmonary homograft valve insufficiency developed in one patient, that was caused by dilatation secondary to the presence of residual distal pulmonary artery stenosis and hypoplasia after repair of pulmonary atresia with ventricular septal defect. This patient was required reoperation (conduit replacement). During follow-up period, there were significant pulmonary stenosis in one, and pulmonary regurgitation more than moderate degree in 3. And there were mild calcifications at distal anastomotic site in 2 patients. All the calcified homografts were aortic in origin. Conclusion: We observed that cryopreserved homograft conduits used in infant and children functioned satisfactorily in the pulmonic position at mid-term follow-up. To enhance the homograft function, ongoing investigation is required to re-establish the optimal strategy for the harvest, preservation and the use of it.

• Journal of Chest Surgery
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• v.36 no.7
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• pp.483-488
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• 2003

There are still debates in the literature on the relative benefits of blood cardioplegia and crystalloid cardioplegia in pediatric cardiac surgery. We performed a clinical trial to compare the myocardial protective effect between HTK solution and blood cardioplegic solution in congenital heart surgery. Material and Method: 15 patients who underwent HTK solution cardioplegia (group 1) and 15 patients who underwent blood cardioplegia(group 2) were included in this study. Preoperative and postoperative serial serum cardiac enzyme levels (troponin I, CK-MB, LDH) were measured in all patients. Clinical data were analyzed and compared between the two groups. Result: There were no differences in age and body weight between the two groups. Operative diagnosis included ventricular septal defect (VSD, n＝4), atrial septal defect (ASD, n＝1), tetralogy of Fallot (TOF, n＝4), and other complex heart diseases (n＝6) in group 1, VSD (n＝7), ASD (n＝5), and TOF (n＝3) in group 2. Cardiopulmonary bypass times were 99.1$\pm$48.1 minutes in group 1, and 69.3$\pm$27.3 minutes in group 2 (p＝0,02). Aortic clamping times were 52.1$\pm$23.6 minutes in group 1, and 37.9$\pm$20.5 minutes in group 2 (p＝0.07). There was no mortality and spontaneous defibrillation was possible in all patients. No differences were observed in the serial enzyme levels between the two groups. There were no differences in the duration of inotropic support and ventilator time between the two groups. Conclusion: HTK solution provided comparable myocardial protection compared with blood cardioplegic solution. A single high dose of HTK solution may be safely and conveniently used for an extended periods as well in congenital heart surgery.

• The korean journal of orthodontics
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• v.34 no.3 s.104
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• pp.261-267
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• 2004

Nonsyndromic cleft lip and/or palate (NSCLP) is one of the most common congenital deformities and its prevalence in Far East Asia, such as within Korean and Japanese populations, is relatively high. However, in the eastern part of Europe, clefts are relatively rare situations. These ethnic differences infer a genetic background of the disease. The objective of this study was to compare the frequency of single nucleotide polymorphism (SNP) in $TGF-{\beta}3$ between Korean and Romanian cleft families. Korean cleft families samples were collected from twenty-six families (n=78) and Romanian cleft families samples were collected from eighteen families (n=41). For sequencing, the blood or saliva of the subjects was sampled. A single nucleotide polymorphism was observed in the intron 5 of $TGF-{\beta}3$ (Al8141G). The frequency of each allele was significantly different between the Korean and Romanian samples. The Ah allele was present in 18 out of 78 Korean samples (23.1%) and in 27 out of 41 Romanian samples (65.9%). The AG was present in 27 (34.6%) out of 78 Koreans and in 13 (31.7%) out of 41 Romanians. The GG was found in 33 (42.3%) Koreans and in 1 (2.4%) Romanian. The difference between the groups was significant (p<0.001). In conclusion, the frequency of observed SNP was significantly different between the two countries. SNP in $TGF-{\beta}3$ in the Korean population seemed to have a higher possibility of occurrence for nonsyndromic cleft palate than the Romanian population.

• Clinical and Experimental Pediatrics
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• v.52 no.10
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• pp.1140-1146
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• 2009

Purpose:The pathophysiology of magnesium, the second highest common compound in humans, is still unclear, especially in preterm babies. We accessed the association between total magnesium (tMg), ionized Mg (iMg), and gestational age (GA) and that between serum magnesium (sMg) and intraventricular hemorrhage (IVH) in preterm babies. Methods:In all, 119 inborn preterm infants admitted between July 2006 and February 2008 were divided into the IVH group (19) and the control group (100) and were prospectively analyzed. TMg, iMg, pH, total calcium (tCa), and ionized Ca (iCa) levels were determined immediately after delivery or within 3 hours after birth, and their correlation with GA were investigated. Results:TMg was not correlated with GA, tCa, iCa, and pH. IMg was correlated with tMg (r=0.288, P=0.002) and iCa (r=0.212, P=0.021); however, it was not correlated with GA and pH. Mean GA and birth weight were significantly lower (P=0.002) and smaller (P=0.030) in the IVH group. Mean sMg was higher in the IVH group ($2.5{\pm}0.9mg/dL$) than in the control group ($2.1{\pm}0.6mg/dL$) (P=0.021). SMg was a risk factor even after logistic regression analysis (OR, 2.798; 95% C.I., 1.265-6.192; P=0.011). Conclusion:In less than 37-week-old preterm babies, tMg and iMg were similar, regardless of GA. High sMg may be a risk factor for IVH in premature babies, regardless of their exposure to antenatal magnesium.

• Pediatric Infection and Vaccine
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• v.17 no.1
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• pp.1-8
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• 2010

Purpose : Recently, vancomycin-resistant enterococci (VRE) have become one of the major nosocomial pathogens in Korea. However, there have been few studies on the epidemiology of VRE colonization among neonates. In this study, we investigated the prevalence of VRE colonization, risk factors for VRE, and how to control the spread of VRE infection in the Neonatal Intensive Care Unit (NICU) of Pusan National University Hospital (PNUH). Methods : We retrospectively reviewed medical records of 192 neonates who were admitted to the NICU of PNUH from March 2006 to March 2007. Surveillance cultures from rectal swabs for detecting VRE were obtained weekly during the study period. We analyzed the prevalence of VRE and various risk factors. Results : The rate of VRE colonization among NICU patients was 25% (48/192). Thirty five of these VRE colonized patients were transferred to the NICU from other local hospitals. Compared with the non-VRE group, the risk factors associated with VRE colonization were lower birth weight, congenital heart disease, applied mechanical ventilation, use of a central venous catheter, chest tubing, a history of surgery, and use of antibiotics. Conclusion : VRE colonization among patients admitted to the NICU is rapidly increasing. Monitoring and managing premature neonates from the beginning of the birth process, avoiding many invasive procedures, avoiding antibiotics such as vancomycin and third generation cephalosporin are important for preventing the emergence and spread of VRE colonization in the NICU.

• Pediatric Infection and Vaccine
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• v.16 no.2
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• pp.162-166
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• 2009

Purpose : Urinary tract infection (UTI) is one of the most common bacterial infectious disease in childhood. Renal scarring is an important complication of UTIs. Known risk factors for renal scarring are younger age, anatomic defects, delayed treatment, and causative pathogens other than Escherichia coli. The aim of this study was to compare the characteristics of clinical and laboratory features of UTI with E. coli to those with non-E. coli in infants. Methods : We reviewed the medical records of 1,120 infants under 12 months of age who had been admitted for UTIs between January 1998 and December 2007. All patients who were diagnosed with UTIs were divided into two groups (E. coli and non-E. coli UTIs). Results : Three hundred twenty-four of 1,120 cases met the inclusion criteria. The number of E. coli and non-E. coli UTIs was 273 (84.3%) and 51 (15.7%), respectively. As compared to the non-E. coli UTI group, the E. coli UTI group was younger (3.59 vs. 4.47 months, P =0.008), a longer duration of pyuria (3.96 vs. 3.06 days, P =0.01), higher peripheral white blood cell counts (13.89 vs. $12.13{\times}10^3/mm^3$, P =0.043), and lower rates of high degree (III-V) vesico-ureteral reflux (P =0.005). Conclusion : UTIs with E. coli might have more severe clinical features and a lower prevalence of high grade vesicoureteral reflux than UTIs with non-E. coli. However, no difference was noted in the clinical response to antibiotic therapy between the two groups.

• Journal of Chest Surgery
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• v.39 no.3 s.260
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• pp.177-183
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• 2006

Background: The bidirectional cavopulmonary shunt (BCPS) is one of the primary palliative procedures for complex congenital heart disease. It has many advantages, but it is known to have high risks in young infants. Material and Method: From 1995 to 2003, 48 infants under the age of one year underwent BCPS. All the patients were Fontan candidates due to functional univentricular heart physiology. There were no significant differences in preoperative variables, except in mean age (67.58$\pm$3.78 vs. 212.91$\pm$13.44 days), and mean body weight (4.51$\pm$0.29 vs. 6.62$\pm$0.27 kg), between group A (<3 months, n=12) and group B ($\ge$3 months, n=36). Result: In group A, the arterial oxygen saturations serially measured were significantly lower. Hospital mortality was $25\%$, and $19\%$, respectively. During follow up, there were 2 late mortalities in group A, and 5 in group B. Conclusion: This study showed that operative risk in young infants was comparable to that of older patients, and BCPS could be a good option as a primary palliative procedure, and may eliminate other repeated palliative procedures which could be the risk factors for Fontan candidates. However, in high-risk patients accompanying pulmonary hypertension, or heterotaxia syndrome, other palliative procedures should be considered.

• Journal of Chest Surgery
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• v.40 no.2 s.271
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• pp.83-89
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• 2007

Background: The surgical repair of an isolated coarctation of the aorta, without complex cardiac anomalies, has improved, with very good results. However, despite the success of surgical repair, many long-term complications, such as hypertension, re-coarctation and an aortic aneurysm, still exist. Material and Method: Between 1991 and 2006, 50 patients diagnosed with an isolated coarctation of the aorta were reviewed retrospectively. The incidence of re-coarctation and hypertension were compared with respect to age and surgical methods. Result: There were no early & late mortality, or post operative aortic aneurysms. Hypertension developed in 11 patients (22%). A greater number of patients in the child/adult group had hypertension (52.4%) than in the neonate/infant group (0%). With respect to the surgical methods, the patients in the graft interposition group suffered more hypertension (88.9%) than those in the EEEA (extended end to end anastomosis) group (5.3%). Post operative re-coarctation developed in 2 out of the 29 patients (6.9%) in the neonate/infant group and 2 out of the 21 patients (9.5%) in the child/adult group, but without any statistical difference. There were no statistical differences between the operative type-related groups. Conclusion: Even though the surgical outcomes have greatly improved, an isolated coarctation of the aorta still has many long-term problems, such as hypertension and re-coarctation. An isolated coarctation is accepted as a systemic vascular dysfunction, and often progresses to other cardiovascular diseases. Therefore, patients with a coarctation of the aorta have to be carefully followed-up, and aggressive management must be given when required.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.18 no.2
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• pp.43-49
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• 2018

Maple syrup urine disease (MSUD, OMIM#248600) is a rare and autosomal recessively-inherited metabolic disorder that is caused by mutations in the branched-chain ${\alpha}$-ketoacid dehydrogenase (BCKDH) genes. It prevents the normal breakdown of branched-chain amino acids (BCAAs), such as leucine, isoleucine, and valine, and leads to poor feeding, lethargy, abnormal movements, seizure, and death if untreated. Here, we report the case of a Korean newborn of biochemically- and genetically-confirmed MSUD manifesting lethargy and central apnea, the acute state of which was successfully treated. The molecular genetic investigation revealed two novel heterozygous mutations (p.Ala32Phefs*48 and p.Val 130Phe) in BCKDHB, and both parents were confirmed as carriers. We emphasize the importance of early diagnosis and prompt introduction of specific treatment for MSUD in life saving and prognosis.