• Advances in pediatric surgery
• /
• v.16 no.1
• /
• pp.1-10
• /
• 2010

The members of the Korean Association of Pediatric Surgeons conducted a retrospective study of two hundred and twenty-two cases of intestinal atresia for the period from January 1, 2007 to December 31, 2009. Seventeen hospitals were involved. There were 76 duodenal, 65 jejunal, and 81 ileal atresias (3 colonic). The male to female ratio was 0.85:1 in DA and 1.34:1 in JIA. Ninety-four patients(43.3 %) were premature babies (DA 40.3 %, JA 64.6 %, IA 28.8 %), and 70 babies (32.0 %) had low birth weight (DA 38.7 %, JA 44.4 %, IA 16.0 %). Antenatal diagnosis was made in 153 cases (68.9 %). However, 27 infants (17.6 %) with antenatal diagnosis were transferred to the pediatric surgeon's hospitals after delivery. Maternal polyhydramnios was observed in 81 cases (36.59 %) and most frequent with proximal obstruction. In forty-four cases (19.8 %), only simple abdominal film was taken for diagnostic study. The associated malformations were more frequently observed in DA - 61.8 % in DA and 22.6 % in JIA. Meconium peritonitis, small bowel volvulus and intussusception were more frequently associated with ileal atresia. The overall mortality rate was 3.6 %. (Abbreviations: DA;duodenal atersia, JA;jejunal atresia, JIA;jejunoileal atresia, IA;ileal atrsia).

• Clinical and Experimental Pediatrics
• /
• v.50 no.11
• /
• pp.1110-1115
• /
• 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
• /
• v.37 no.9
• /
• pp.727-734
• /
• 2004

The Lecompte procedure for transposition of the great arteries has an advantage because it obviates the need for an extracardiac conduit for the reconstruction of the pulmonary outflow tract. We evaluated the effectiveness and the application of the Lecompte procedure. Material and Method: A retrospective review was conducted of the records of 46 patients who underwent the Lecompte procedure during the past 15 years. Mean age at operation was 29.2$\pm$20.3 (range: 3∼83) months. The diagnoses involved anomalies of the ventriculoarterial connection with ventricular septal defect and pulmonary outflow tract obstruction, such as transposition of the great arteries, double-outlet right ventricle, and double-outlet left ventricle. Result: Early mortality was 4.4% (2 of 46 patients) and late mortality was 6.8% (3 of 44). The mean follow-up was 11.2$\pm$6.9 years. Eighteen patients (43.9% of survivors, n=41) had pulmonary stenosis (pressure gradient above 30 mmHg), the main reason for which was a calcified monocusp valve (n=15, 83.3%). Seventeen of 46 patients (37.0%) underwent reoperation: 15 for pulmonary stenosis, 5 for residual ventricular septal defect, 4 for left ventricular outflow tract obstruction, 3 for pulmonary insufficiency, and 4 for other causes. The cumulative survival rates were 91.3$\pm$4.2%, and 87.0$\pm$5.8% at 10 and 15 years, respectively. The actuarial probabilities of freedom from reoperation for pulmonary stenosis were 90.6$\pm$4.5%, 73.9$\pm$7.3%, and 54.0$\pm$10.4% at 5, 10, and 15 years, respectively. Conclusion: The Lecompte procedure is an effective treatment modality. Repair in early age is possible with acceptable morbidity and mortality, but recurrent right ventricular outflow tract obstruction caused by degeneration of the monocusp valve is a problem that needs resolution.

• The korean journal of orthodontics
• /
• v.31 no.1 s.84
• /
• pp.51-61
• /
• 2001

Cleft lip and/or palate (CLP) is one of the most common congenital craniofacial anomalies and occurs more frequently in Asian people. Dental abnormalities in number, size, shape, and eruption of teeth are frequently associated with CLP. The purposes of this study were to investigate the effects of CLP on number, size, shape and eruption of teeth and to provide basic clinical data for diagnosis and treatment of the CLP patients. With the orthodontic and cleft charts, diagnostic models, orthopantomograms and intraoral x-ray films from 241 CLP patients who visited Dept. of Orthodontics, Seoul National University Dental Hospital, we evaluated the frequency of congenital missing teeth, supernumerary teeth, Impacted teeth, and microdontia. The results were as fellows ; 1. Frequency of congenital missing was relatively high up to $56.8\%$. Congenital missing occurred frequently in the maxillary lateral incisor and the maxillary second premolar. Among the CLP types, frequencies of congenital missing in cleft lip and Palate group and cleft lip and alveolus group were higher than those of cleft lip group and cleft palate group. And bilateral cleft showed higher frequencies than unilateral ones. 2. Supernumerary tooth was shown in $11.2\%$ of CLP patients. It occurred frequently in the area between the maxillary lateral Incisors and the maxillary canine. Among the CLP types, cleft lip group showed relatively most highest frequency. 3. Impaction was shown in $18.3\%$ of CLP patients. It occurred most frequently In the maxillary lateral incisor and the maxillary canine than other teeth. Among the CLP types, cleft lip group and cleft lip and palate group showed most highest frequencies. 4. Microdontia was shown in $15.8\%$ of CLP patients. It occurred the most frequently In the maxillary lateral incisors and maxillary canines. Among the CLP types, cleft lip and alveolus group and cleft lip and palate group showed relatively higher frequencies. There was no microdontia in cleft palate group.

• The korean journal of orthodontics
• /
• v.33 no.3 s.98
• /
• pp.185-197
• /
• 2003

This study was performed to identify the characteristics of the OFC1 gene (locus: chromosome 6p24.3) in Korean patients, which is assumed to be the major gene behind the nonsyndromic cleft lip and palate. The sample consisted of 80 subjects: 40 nonsyndromic cleft lip and palate patients (proband, 20 males and females, mean age 14.2 years); and 40 normal adults (20 males and 20 females, mean age 25.6 years). Using PCR-based assay, the OFC1 gene was amplified, sequenced, and then searched for similar protein structures. Results were as follows: 1. The OFC1 gene contains the microsatellite marker 'CA' repeats. The number of the reference 'CA' repeats was 21 times, and formed as TA(CA)11TA(CA)10. But, in Koreans, the number of tandem 'CA' repeats was varied from 17 to 26 except 18, and 'CA' repeats consisted of TA(CA)n. 2. Nine allelic variants were found. Distribution of the OFC1 allele was similar between the patients and control group. 3. There was a replacement of the base 'T' to 'C' after 11 tandem 'CA' repeats in Koreans compared with Weissenbach's report. However, the difference did not seem to be the ORF prediction results between Koreans and Weissenbach's report. 4. The BLAST search results showed the Telomerase reverse transcriptase (TERT) and the Nucleotide binding protein 2 (NBP2) as similar proteins. The TERT was a protein product by the hTERT gene in the locus 5p15.33 (NCBI Genome Annotation; NT023089) The NBP2 was a protein product by the ABCC3 (ATP-binding cassette, sub-family C) gene in the locus 17q22 (NCBI Genome Annotation; NT010783). 5. In the Pedant-Pro database analysis, the predictable protein structure of the OFC1 gene had at least one transmembrane region and one non-globular region.

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

• Journal of Korean Neurosurgical Society
• /
• v.29 no.3
• /
• pp.379-388
• /
• 2000

Objective : Although transoral transpharyngeal approach is a very useful method for the lesions of craniovertebral junction, it is not frequently used because of anatomical unfamilarity, risk of cerebrospinal fluid(CSF) leakage, and resultant postoperative meningitis. To evaluate the usefulness of transoral transpharyngeal approach for various lesions of craniovertebral junction, clinical characteristics and the results of this approach are investigated. Methods : Transoral transpharyngeal approaches were performed in eight cases between 1996 and 1999. Among them, there were three basilar invaginations due to congenital anomalies, two odontoid type I fractures, two atlantoaxial dislocations, and one pseudotumor. Surgical methods included five cases of anterior decompression and posterior fusion, two anterior approaches for decompression and one transoral approach for biopsy. Results : This procedure allowed immediate clinical improvement in all cases. In seven patients with preoperative motor deficit showed a progressive neurological improvement. The follow-up plain x-rays demonstrated successful bony fusion in all patients. Only one patient suffered from postoperative wound dehiscence, but she completely recovered after wound revision. There was no complication of postoperative CSF leakages. Conclusions : Transoral transpharyngeal approach for the ventral lesions of craniovertebral junction, can be used as a relatively simple and effective method.

• Childhood Kidney Diseases
• /
• v.15 no.1
• /
• pp.58-65
• /
• 2011

Purpose: Some hormonal and electrolyte abnormalities have been reported in pediatric patients with urinary tract infection (UTI). This study aimed to investigate the relationships between the imbalance of electrolytes and the severity of infection and associated urologic anomalies in children with febrile UTI. Methods: We retrospectively reviewed 267 patients with febrile UTI who were admitted to Korea University Guro Hospital during the period from January, 2007 until February, 2010. According to the presence of hyponatremia or hyperkalemia, clinical parameters and associated renal anomalies, such as hydronephrosis, cortical defects and vesicoureteral reflux, were compared. Results: 42.7% of all patients had decreased concentration of serum sodium. In patients with decreased concentration of serum sodium, cortical defects were significantly increased compared to normal patients (40.4% vs. 14.4%, P <0.05). White blood cell (WBC) counts ($15,721{\pm}6,553/uL$ vs. $12,885{\pm}5,367/uL$, P <0.05), C-reactive protein (CRP) ($61.8{\pm}56.1$ mg/L, vs. $29.9{\pm}39.8$ mg/L, P <0.05), and erythrocyte sedimentation rate (ESR) ($43.9{\pm}34.3$ mm/hr vs. $27.4{\pm}26.8$ mm/hr, P <0.05) in peripheral blood showed significant increases in the group with decreased concentration of serum sodium. Duration of fever, presence of gastrointestinal symptom, the incidence of hydronephrosis and vesicoureteral reflux did not differ between the two groups. None of the patients had significant hyperkalemia. Conclusion : We suggest that decreased concentration of serum sodium in febrile UTI might be a helpful marker for leukocytosis and increased CRP and ESR in peripheral blood, and acute pyelonephritis.

• Pediatric Infection and Vaccine
• /
• v.14 no.1
• /
• pp.43-46
• /
• 2007

Purpose : Neonatal bacterial meningitis has been known that its mortality and morbidity is high and its neurologic complications are common. This study was performed to review of the etiology of neonatal bacterial meningitis in Busan, Korea. Methods : We retrospectively analyzed 21 medical records of patients who were diagnosed neonatal bacterial meningitis in four university hospitals in Busan from January 1997 to December 2005. We analyzed age at onset of symptoms, symptoms at admission, causative organisms, and complications. Results : There were 11 male patients and 10 female patients. One premature neonate was included. 7 patients had symptoms at 2-3 weeks after birth and 6 patients at 1-2 weeks after birth. 18 cases had fever, 8 cases had seizure and some had poor feeding, lethargy and irritability. The most common organism causing neonatal bacterial meningitis was Group B streptococcus (GBS). 11 cases were due to GBS, 3 cases due to E. coli, 3 cases were due to Klebsiella pneumoniae, and 2 cases due to Streptococcus pneumoniae. one Listeria monocytogenes and one Enterobacter cloacae were included. 18 patients were alive and 3 patients died. Mental retardation, developmental delay and epilepsy occurred in a living patient. Conclusions : Although this was a retrospective study, we found that Group B streptococcus (GBS) was the most common organism causing neonatal bacterial meningitis and this data is nearly same as others in the world.

• Journal of the Korean Academy of Child and Adolescent Psychiatry
• /
• v.8 no.1
• /
• pp.34-42
• /
• 1997

Enuresis is repeated involuntary or intentional voiding of urine into bed or clothes over age 5. Though it would be a self-remitting disorder, it could be serious problem in emotional and socio-adaptational aspects. The author reviewed the enuretic patients of Child & Adolescence psychiatric section in Chungnam National University Hospital during past 3 years. 46(4.9%) of 936 patients were diagnosed as enuresis in DSM-Ⅳ. The author evaluated their comorbidity by the data of diagnostic review made in two psychiatrists, and emotional aspects(self-concept, anxiety, depression) through the self-rating scales (Piers-Harris children’ self concept scales, RCMAS, state-trait anxiety inventory for children, child’s depresson inventory). Thirty(65.2%) of the 46 enuretic patients had additional diagnoses such as attention deficit hyperactive disorder, mental retardation, encopresis, oppositional defiant disorder, depression, anxiety disorder, autism, somatoform disorder, tic disorder, obsessive-compulsive disorder, sleep disorder, etc. Sixteen enuretic patients had at least one comorbid disorder. Eleven patients had two, and three patients had more than three. Fourteen of 46 enuretic patients were evaluated through self-rating scales of self-concept, anxiety and depression. But we couldn’t obtain meaningful results. Maybe it was due to the small sample size(N＝14) and the influence of the comorbid disorders. Finally, it was an impressive evidence that there exist many comorbid disorders in enuresis(esp. attention deifict/hyperactive disorder). In emotional aspects, the author thought that further evaluation should be needed for more meaningful results.