• Journal of Chest Surgery
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• v.32 no.11
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• pp.1042-1045
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• 1999

Background: Recently, open heart surgerys using homograft are progressively increasing in complex cardiac anomalies, and even though the use of homograft tissues harvested from hearts of transplant recipients and brain-death patients are allowed and their use is increasing, the supply of homograft tissue is very limited. Material and Method: The large diameter homografts are difficult to apply directly for RVOT reconstruction of small neonatal and infant hearts due to the size mismatching. Therefore, were surgically down-sized the large diameter tricuspid homograft into bicuspid conduits by means of a longitudinal incision of the oversized homograft, excision of one cusp, and oversewing of the“Bicuspid homograft”wrapped around a Hega dilator of the appropriate size. Result: 3 patients(Male 1, Female 2: tetralogy of Fallot with pulmonary atresia), ranging in age from 5 months to 4 years and ranging in weight from 5.5Kg to 12.95Kg underwent reconstruction of the RVOT with bicuspid conduits obtained by appropriate tailoring from large-diameter homografts. The mean follow-up period was 4.3 months(range, 2 to 6 months). There were no complications related to the homograft tissues. Conclusion: In the short term follow-up, the bicuspid homografts provided good competence and excellent hemodynamics although a long term follow-up is needed to assess the functions of the bicuspid homografts in RVOT. We believe this technique may be a more effective alternative than the use of synthetic conduits when the use of an appropriate-sized homograft is not possible.

• Clinical and Experimental Pediatrics
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• v.53 no.5
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• pp.661-665
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• 2010

Anemia caused by vitamin B12 deficiency resulting from inadequate dietary intake is rare in children in the modern era because of improvements in nutritional status. However, such anemia can be caused by decreased ingestion or impaired absorption and/or utilization of vitamin B12. We report the case of an 18-year-old man with short stature, prepubertal sexual maturation, exertional dyspnea, and severe anemia with a hemoglobin level of 3.3 g/dL. He had a history of small bowel resection from 50 cm below the Treitz ligament to 5 cm above the ileocecal valve necessitated by midgut volvulus in the neonatal period. Laboratory tests showed deficiencies of both vitamin B12 and iron. A bone marrow examination revealed dyserythropoiesis and low levels of hemosiderin particles, and a cytogenetic study disclosed a normal karyotype. After treatment with parenteral vitamin B12 and elemental iron, both anemia and growth showed gradual improvement. This is a rare case that presented with short stature and delayed puberty caused by nutritional deficiency anemia in Korea.

• Clinical and Experimental Pediatrics
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• v.50 no.12
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• pp.1252-1256
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• 2007

Meconium ileus (MI) is the earliest clinical manifestation of cystic fibrosis (CF) in infants. It arises from the intraluminal accumulation of highly viscid, protein-rich meconium, typically present in the terminal ileum as a neonatal intestinal obstruction. Therefore, the clinical symptoms include abdominal distension, bilious vomiting and delayed passage of meconium. CF is caused by mutations in the transmembrane conductance regulator gene (CFTR) located in the long arm of chromosome 7. CF is common in Caucacians, but is a rare disorder in Asian countries, including Korea. We experienced a case of CF combined with MI. Compared with the previous reports of CF in Korea which presented respiratory problems, this is the first case genetically diagnosed as CF with MI during the newborn period.

• Clinical and Experimental Pediatrics
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• v.46 no.4
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• pp.389-392
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• 2003

Netherton's syndrome is an unusual disorder which consists of triad of ichtyosiform dermatosis, multiple defects of hair shaft and an atopic diathesis. The finding of bamboo hair is pathognomic in Netherton's syndrome and the ichthyosiform dermatosis may consist of either ichtyosis linearis circumflexa or congenital ichthyosiform erythroderma. Often, variability in the clinical features leads to a delay in diagnosis in many cases. We report a case of Netherton's syndrome diagnosed in the neonatal period. The patient presented with severe ichthyosis and confirmed microscopically distinctive bamboo hair.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.20 no.2
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• pp.134-137
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• 2017

Gastric hemangioma in the neonatal period is a very rare cause of upper gastrointestinal bleeding. We present a case of hemangioma limited to the gastric cavity in a 10-day-old infant. A huge, erythematous mass with bleeding was observed on the lesser curvature side of the upper part of the stomach. Surgical resection was ruled out because the location of the lesion was too close to the gastroesophageal junction. Medical treatment with intravenous $H_2$ blockers, octreotide, packed red blood cell infusions, local epinephrine injection at the lesion site, application of hemoclip, and gel-form embolization of the left gastric artery did not significantly alter the transfusion requirement. Hemostasis was achieved with endoscopic argon plasma coagulation (APC). After two sessions of APC, complete removal of the lesion was achieved. APC was a simple, safe and effective tool for hemostasis and the ablation of gastric hemangioma without significant complications.

• Clinical and Experimental Pediatrics
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• v.59 no.sup1
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• pp.116-120
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• 2016

Congenital hyperinsulinism (CHI) is a rare condition that can cause irreversible brain damage during the neonatal period owing to the associated hypoglycemia. Hypoglycemia in CHI occurs secondary to the dysregulation of insulin secretion. CHI has been established as a genetic disorder of islet-cell hyperplasia, associated with a mutation of the ABCC8 or KCNJ11 genes, which encode the sulfonylurea receptor 1 and the inward rectifying potassium channel (Kir6.2) subunit of the ATP-sensitive potassium channel, respectively. We report the case of a female newborn infant who presented with repetitive seizures and episodes of apnea after birth, because of hypoglycemia. Investigations revealed hypoglycemia with hyperinsulinemia, but no ketone bodies, and a low level of free fatty acids. High dose glucose infusion, enteral feeding, and medications could not maintain the patient's serum glucose level. Genetic testing revealed a new variation of ABCC8 mutation. Therefore, we report this case of CHI caused by a novel mutation of ABCC8 in a half-Korean newborn infant with diazoxide-unresponsive hyperinsulinemic hypoglycemia.

• Journal of Nutrition and Health
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• v.28 no.4
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• pp.321-330
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• 1995

Concentrations of total vitamin B-6 in human milk as well as individual, B-6 vitamers have important implications for the nutritional management of breast-fed(BF) infants. Vitamin B-6 status was assessed in 3 groups of infants : two groups preterm (PT) BF infants whose mothers were supplemented with 2 or 27mg pyridoxine(PN)-HCI ; a sub group of formula-fed (FF) PT infants. Mothers and infants were assessed weekly during the 28-day post feeding. Throughout the neonatal period, levels of total vitamin B-6 and percentages of pyridoxal(PL) in breast milk were lower in PT than T mothers, even in mothers supplemented with 27mg PN-HCI. Total vitamin B-6 levels in PT milk paralleled maternal supplementation but percentage distributions of B-6 vitamers did not change. Vitamin B-6 intakes of BF preterm infants paralleled their mothers' level of infants in the 2mg group was suggested by vitamin status parameters. Vitamin B-6 inadequacy of infants correlated with their plasma pyridoxal-5-phosphate(PLP) levels and erythrocyte alanine aminotransferase(E-ALAT) activity; all parameters such as plasma PLP, PL/PLP ratio and stimulation % of E-ALAT were highest for FF PT infants. The positive correlation of vitamin B-6 levels in breast milk gestational age may contraindicate its adequacy for some PT infants.

• Clinical and Experimental Pediatrics
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• v.48 no.2
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• pp.221-223
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• 2005

Congenital pleural effusions are uncommon. The majority of cases are due to chylothorax, hydrops fetalis, and infection. Effusions of this nature are, for the most part, self-limited. We experienced a rare case of a congenital unilateral pleural effusion due to an extralobar sequestraion with pulmonary lymphangiectasia. Pleural effusion was found by antenatal ultrasonography and confirmed by CT scans and CT angiography of the chest in the neonatal period. The patient underwent an open thoracotomy where extralobar sequestraion located between the diaphragm and the left lower lobe was removed. His postoperative course was uncomplicated and there was complete resolution of the pleural effusion.

• Journal of Chest Surgery
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• v.19 no.3
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• pp.464-469
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• 1986

Pulmonary atresia with intact ventricular septum, and with VSD were uncommon congenital anomalies with high mortality in the neonatal period. Those survivals depend on an adequate interatrial communication or interventricular communication and pulmonary flow via large aortopulmonary collateral including PDA. Recently we experienced surgical correction of 2 cases pulmonary atresia with intact ventricular septum and with VSD. On case 1, 10-years old male patient was confirmed as pulmonary valvular membranous atresia with intact ventricular septum combined with large functioning PFO and mild tricuspid incompetence. So we performed total correction under the E.C.C, that was PDA ligation, RVOT reconstruction with monocusp valved outflow patch [16mm], repair of tricuspid insufficiency and closure of PFO. Post-operative hemodynamic result was good and there was no event during hospital course. On case 2, 16-years old female patient was diagnosed as pulmonary atresia with VSD and PDA. MPA was absent, remained fibrous cord like remnant and type of VSD was subaortic defect [3cm by 3cm in the size]. PDA was located at the usual site. Under the E.C.C. VSD patch closure through the right ventriculotomy, anastomosis between the right ventricular outflow tract and the pulmonary bifurcated site with the extra-cardiac Hancock valved conduit [22cm] and PDA ligation were performed.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.14 no.2
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• pp.163-167
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• 2014

DiGeorge syndrome is a disorder caused by microdeletion in chromosome 22q11.2 with various abnormalities including cardiac anomaly, facial dysmorphism, thymic and parathyroid hypoplasia, cleft palate and immune dysfunction. The frequency of hypocalcemia caused by hypoparathyroidism is known to be approximately 60% of DiGeorge syndrome. It is known that the disorder mostly occurs in the neonatal period and the symptoms are improved afterwards. Herein we report a case of DiGeorge syndrome only accompanied by hypocalcemia caused by hypoparathyroidism without other abnormalities. She was first diagnosed only at the age of 22 with basal ganglia calcification that had been discovered in brain CT (Computed tomography).