• Journal of Chest Surgery
• /
• 제32권11호
• /
• pp.1042-1045
• /
• 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
• /
• 제53권5호
• /
• pp.661-665
• /
• 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
• /
• 제50권12호
• /
• pp.1252-1256
• /
• 2007

태변장폐색증은 낭성섬유증에서 가장 초기에 나타나는 임상 증상으로 태변이 장관내 축적되어 회장말단의 폐색을 일으키고 복부팽만, 담즙성 구토, 태변 배출 지연이 동반된다. 낭성섬유증은 7번 염색체 장완에 위치한 막전도조절유전자의 돌연변이로 야기되며 한국인에서는 드문 질환이다. 지금까지 대한민국에서 보고된 호흡기 질환에 동반된 낭성섬유증과 달리, 저자들은 태변장폐색증에 동반된 낭성섬유증이 유전자 검사로 진단된 1례를 경험하였기에 보고하는 바이다.

• Clinical and Experimental Pediatrics
• /
• 제46권4호
• /
• pp.389-392
• /
• 2003

Netherton 증후군은 어린선양 피부병증, 머리카락이상, 아토피 소질 등 3가지 임상양상을 특징으로 하는 드문 질병이다. 저자들은 임상소견, 피부조직 검사와 머리카락 현미경 소견을 통하여 신생아기에 Netherton 증후군으로 진단하고 추적 관찰하고 있기에 간단한 문헌 고찰과 함께 보고하는 바이다.

• Pediatric Gastroenterology, Hepatology & Nutrition
• /
• 제20권2호
• /
• pp.134-137
• /
• 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
• /
• 제59권sup1호
• /
• pp.116-120
• /
• 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
• /
• 제28권4호
• /
• pp.321-330
• /
• 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
• /
• 제48권2호
• /
• pp.221-223
• /
• 2005

선천성 흉막액을 동반한 외엽형 폐격리증은 매우 드문 선천성 폐기형으로 아직까지 국내에서의 보고는 없다. 저자들은 재태 기간 27주에 시행한 산전 초음파상 좌측 흉막액이 발견되었고, 출생 후 시행한 흉부 전산화 단층 촬영 및 전산화 단층 혈관 촬영술상 선천성 흉막액을 동반한 외엽형 폐격리증으로 진단하였으며, 생후 80일경 수술 후 특별한 문제없이 퇴원하여 현재 외래 추적 관찰 중인 1례를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.

• Journal of Chest Surgery
• /
• 제19권3호
• /
• pp.464-469
• /
• 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.

• 대한유전성대사질환학회지
• /
• 제14권2호
• /
• pp.163-167
• /
• 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).