• Journal of Chest Surgery
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• 제26권8호
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• pp.650-653
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• 1993

Congenital cystic adenomatoid malformation [ C.C.A.M.] is rare, but often fatal congenital lung bud anomaly that causes acute respiratory distress in the newborn infants. In those who survive infancy and early childhood, its clinical manifestations are not unusual to detect. Recently we experienced an infant with C.C.A.M. Type II. The patient was a 7-month-old male, and had been suffering from coughing and fever since 3 months ago. The symptom were not improved with medical treatment and then, exploratory thoracotomy was performed under the impression of infected C.C.A.M. or other congenital cystic disease.The left lower lobectomy was performed, and the postoperative course was uneventful.

• Journal of Chest Surgery
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• 제30권10호
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• pp.1019-1023
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• 1997

우측 폐동맥이 대동맥에서 이상기시되는 기형은 선천성 심장질환의 드문 형태이고 치명적이다. 주로 동맥관개존과 같이 동반되어 나타나며, 대개 출생후 이른 유아기에 사망한다. 우폐동맥의 이상기시는 좌폐동맥의 이상기시에 비해 더욱 많은 빈도를 나타내며, 비정상적인 우폐동맥은 주로 대동맥판막에 인접한 상행대동맥의 뒤쪽편에서 주로 기시한다.

• Journal of Korean Neurosurgical Society
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• 제55권1호
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• pp.57-60
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• 2014

Rhabdoid tumor of the kidney (RTK) is a rare malignancy in infancy. Central nervous system involvement in RTK is already known. However, solitary spinal metastasis in RTK has been hardly reported. The authors report a case of metastatic RTK to spine causing paraplegia in an 8-month-old girl. Since the patient was young, the diagnosis of spine metastasis was delayed until paraplegia was seen after radical nephrectomy. Thorough neurological examination should be performed for early diagnosis of spinal metastasis in young patients with RTK. If there are any abnormal signs in neurologic examination, magnetic resonance images of brain and spine are recommended.

• Journal of Chest Surgery
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• 제19권3호
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• pp.528-533
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• 1986

Total anomalous pulmonary venous connection is relatively rare cyanotic congenital heart diaease, which represents 1-4% of all congenital cardiac defects. Generally in the majority cases, severe heart failure and cyanosis develops in the early infancy. Because of high mortality in the untreated infants and surgical risk, there are still many things to be improved. Two patients with total anomalous pulmonary venous connection are presented, which we recently experienced. The one was 10 year old female with supracardiac type drained through left innominate vein, and survived the operation and continuous to do well for 1 year. The other 5 year old female with mixed type (right pulmonary vein drained via coronary sinus and left pulmonary vein through left innominate vein) was operated successfully under hypothermia and extracorporeal circulation, and followed up for 6 months without problem. It was very rare case in the literature.

• Journal of Chest Surgery
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• 제19권3호
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• pp.457-463
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• 1986

Truncus Arteriosus is uncommon, accounting for 0.4%-2.8% of all congenital cardiac malformations. Truncus arteriosus has a poor prognosis in early infancy and defined as "a single arterial trunk that leaves the heart by way of a single arterial valve and that gives rise to the coronary, systemic and one or both pulmonary arteries directly." Through antemortem study of patients with truncus arteriosus the development of surgical techniques for palliation and correction was established. Recently we had surgical experience of truncus arteriosus - Collett '||'&'||' Edwards type 2. The main pulmonary artery was originated from truncus at right posterolateral aspect. Truncal valve was tricuspid with good coaptation. Ventricular septal defect was subarterial type of 2.0 cm in diameter. After detachment of the main pulmonary artery from truncus, truncus was repaired directly. Ventricular septal defect was closed with Dacron patch. Extracardiac valved conduit [Carpentier-Edwards: 16mm] was employed for making continuity between right ventricular outflow tract and pulmonary artery. Postoperatively, incomplete right bundle branch block on electrocardiogram was continued. Patient was died due to respiratory failure in postoperative 40 days.s.

• Clinical and Experimental Pediatrics
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• 제50권8호
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• pp.726-731
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• 2007

This review describes the wide spectrum of paroxysmal events during sleep in infancy and childhood. The differential diagnosis between sleep-related non-epileptic paroxysmal events and epileptic seizures is difficult in special occasions. The nocturnal frontal lobe seizure and of the more common nonepileptic paroxysmal events during sleep are described. The main differentiating features characterizing parasomnias are: onset in early childhood, rare episodes of long duration, relatively lower frequency per night, absence of stereotypy, gradual disappearance of older age. Video-polysomnography is the gold standard to diagnosing and differentiating parasomnias from nocturnal frontal lobe seizures.

• 혜화의학회지
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• 제14권1호
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• pp.1-4
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• 2005

Objectives : The aim of this study was the collection of dietary treatments of atopic dermatitis Methods : We surveyed the western and oriental medical book concernig the atopic dermatitis. Results : The 20~30% of patients with atopic dermatitis have hypersensitivity reaction on the major food antigen. The food restriction to prevent allergy reaction need to take effect early during infancy and childhood. In oriental medicine, a heat-poison(熱毒) caused by food affects a fetus of pregnancy. So the food, the regimen and the harmony of the five tastes is stressed the need. When certain foods are restricted to prevent allergy reaction, infant and children with food related Atopic Dermatitis need extra dietary efforts to maintain optimal nutrition as they are in the growth period.

• Journal of Nutrition and Health
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• 제30권7호
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• pp.840-847
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• 1997

In order to investigate the protein intake of breast-fed infants, we examined sixty infants during the first 5 months of lactation. Human milk intake infants increased during lactation with the respective values of 525, 671, 734, 744 , 765 and 768g/day t 0.5, 1, 2, 3, , 4 and 5 months postpartum. The average protein contents of human milk showed 1.58, 1.38, 1.23, 1.11, 1.08and 1.07g/100g respectively. The protein intake of boys during the first 5 months of lactation averaged 9.11g/day which was higher than the 7.71g/day average for girls. Body weight of infants at birth was 3337g, which increased significantly during lactation. The protein intake per body weight of breast-fed infants in boys was significantly higher than that in girls(p<0.01). Protein intake of breast-fed infants had survey, a revaluation of the protein intake and recommended dietary allowance of protein during early infancy should be considered.

• Journal of Yeungnam Medical Science
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• 제23권1호
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• pp.138-142
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• 2006

Abdominal distension is not an uncommon symptom in the neonate; it is indistinguishable from Hirschsprung disease by symptoms and X-ray findings. In three patients, severe abdominal distension was found at early infancy and improved with conservative treatment without relapse. The findings were different from those of Hirschsprung disease. Immaturity or poor coordination of peristaltic movement is postulated as the cause. With maturation such problems can normalize. However the pathogenesis remains unclear and further investigation is needed to improve our understanding.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제22권4호
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• pp.392-399
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• 2019

Wilson disease a rare autosomal recessive inherited disorder of copper metabolism, is characterized by excessive deposition of copper in the liver, brain, and other tissues. Wilson disease is often fatal if it is not recognized early and treated when it is symptomatic. Gitelman syndrome is also an autosomal recessive kidney disorder characterized by low blood levels of potassium and magnesium, decreased excretion of calcium in the urine, and elevated blood pH. Hereditary sensory autonomic neuropathy type IV (HSAN-IV), a very rare condition that presents in infancy, is characterized by anhidrosis, absence of pain sensation, and self-mutilation. It is usually accompanied by developmental delay and mental retardation. We report a case of Wilson disease manifested as fulminant hepatitis, acute pancreatitis, and acute kidney injury in a 15-year-old boy comorbid with HSAN-IV and Gitelman syndrome. Such concurrence of three genetic diseases is an extremely rare case.