• Journal of Chest Surgery
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• 제30권9호
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• pp.927-931
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• 1997

신생아에서 선천적 삼첨판 폐쇄부전은 자주 보고되지만 건삭파열에 의한 이차성 삼첨판 폐쇄부전은 아주 드물다. 환아는 생후 1일의 남아로 출생 직후부터 심한 호흡곤란, 청색증 및 산혈증이 관찰되었고, 무호흡과 함께 서맥이 빈번히 나타났다. 심초음파도 검사를 시행하여 폐동맥 판막을 통한 우심유출로 혈류가 관찰되지 않아서 폐동맥 폐쇄증으로 진단되었다. 수술 소견에서 폐동맥 및 폐동맥 판막의 기형은 전혀 없었고, 삼첨판의 전방 유두근 건삭파열이 새로이 확인되었다. 삼첨판 폐쇄부전은 건삭 형성술을 실시하여 성공적으로 교정하였으며, 술전 심초음파도에서 주폐 동맥에 폐혈류가 관찰되지 않았던 것은 심한 삼첨판 폐쇄부전 때문이었던 것으로 판단되었다.

• Clinical and Experimental Pediatrics
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• 제56권6호
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• pp.260-264
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• 2013

Dapsone (4,4'-diaminodiphenylsulfone, DDS), a potent anti-inflammatory agent, is widely used in the treatment of leprosy and several chronic inflammatory skin diseases. Dapsone therapy rarely results in development of dapsone hypersensitivity syndrome, which is characterized by fever, hepatitis, generalized exfoliative dermatitis, and lymphadenopathy. Here, we describe the case of an 11-year-old Korean boy who initially presented with high fever, a morbilliform skin rash, generalized lymphadenopathy, hepatosplenomegaly, and leukopenia after 6 weeks of dapsone intake. Subsequently, he exhibited cholecystitis, gingivitis, colitis, sepsis, aseptic meningitis, disseminated intravascular coagulation, syndrome of inappropriate antidiuretic hormone secretion, pneumonia, pleural effusions, peritonitis, bronchiectatic changes, exfoliative dermatitis, and acute renal failure. After 2 months of supportive therapy, and prednisolone and antibiotic administration, most of the systemic symptoms resolved, with the exception of exfoliative dermatitis and erythema, which ameliorated over the following 4 months. Agranulocytosis, atypical lymphocytosis, aseptic meningitis, and bronchiectatic changes along with prolonged systemic symptoms with exfoliative dermatitis were the most peculiar features of the present case.

• Journal of Microbiology and Biotechnology
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• 제17권3호
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• pp.516-519
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• 2007

Enterobacter sakazakii is an emerging food pathogen, which induces severe meningitis and sepsis in neonates and infants, with a high fatality rate. The disease is generally associated with the ingestion of contaminated infant formula. In this study, we describe the development of a real-time PCR protocol to identify E. sakazakii using a TaqMan probe, predicated on the nucleotide sequence data of the 168 rRNA gene obtained from a variety of pathogens. To detect E. sakazakii, four primer sets and one probe were designed. Five strains of E. sakazakii and 28 non-E. sakazakii bacterial strains were used in order to ensure the accuracy of detection. The PCR protocol successfully identified all of the E. sakazakii strains, whereas the 28 non-E. sakazakii strains were not detected by this method. The detection limits of this method for E. sakazakii cells and purified genomic DNA were 2.3 CFU/ assay and 100 fg/assay, respectively. These findings suggest that our newly developed TaqMan real-time PCR method should prove to be a rapid, sensitive, and quantitative method for the detection of E. sakazakii.

• Advances in pediatric surgery
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• 제10권2호
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• pp.145-149
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• 2004

A 12-year-old boy with severe periumbilical pain visited the emergency room. Physical examination, abdominal ultrasonography, colonoscopy and CT, identified a lesion of sigmoid colon. Endoscopic biopsy showed a signet ring cell carcinoma of the sigmoid colon. On explorative laparotomy, cancer invasions of the adjacent structures and metastases on peritoneal wall were noticed. We performed palliative loop-ileostomy. He underwent chemotherapy and radiotherapy for 3 months. The second case was a 16-year-old boy with abdominal pain and hematochezia, transferred to our hospital with the diagnosis of acute appendicitis with periappendiceal abscess. Although he underwent appendectomy, the abdominal pain persisted. Digital rectal examination revealed a lumen-obstructing fungating mass in the rectum. Endoscopic biopsy revealed a adenocarcinoma. Cancer invasion of the adjacent structures and metastases involving the mesentery of the small intestine were found at laparotomy. A palliative procedure, a Hartmann's operation and end-colostomy at the sigmoid colon were performed. The patient died 8 month later due to pneumonia and sepsis. Chemotherapy was not applied.

• Clinical and Experimental Pediatrics
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• 제54권5호
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• pp.183-191
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• 2011

Although antenatal diagnostic technique has considerably improved, precise detection and proper management of the neonate with congenital heart disease (CHD) is always a great concern to pediatricians. Congenital cardiac malformations vary from benign to serious conditions such as complete transposition of the great arteries (TGA), critical pulmonary and aortic valvular stenosis/atresia, hypoplastic left heart syndrome (HLHS), obstructed total anomalous pulmonary venous return (TAPVR), which the baby needs immediate diagnosis and management for survival. Unfortunately, these life threatening heart diseases may not have obvious evidence early after birth, most of the clinical and physical findings are nonspecific and vague, which makes the diagnosis difficult. High index of suspicion and astute acumen are essential to decision making. When patent ductus arteriosus (PDA) is opened Widely, many serious malformations may not be noticed easily in the early life, but would progress as severe acidosis/shock/cyanosis or even death as PDA constricts after few hours to days. Ductus dependent congenital cardiac lesions can be divided into the ductus dependent systemic or pulmonary disease, but physiologically quite different from each other and treatment strategy has to be tailored to the clinical status and cardiac malformations. Inevitably early presentation is often regarded as a medical emergency. Differential diagnosis with inborn error metabolic disorders, neonatal sepsis, persistent pulmonary hypertension of the newborn (PPHN) and other pulmonary conditions are necessary. Urgent identification of the newborn at such high risk requires timely referral to a pediatric cardiologist, and timely intervention is the key in reducing mortality and morbidity. This following review deals with the clinical presentations, investigative modalities and approach to management of congenital cardiac malformations presenting in the early life.

• Clinical and Experimental Pediatrics
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• 제54권8호
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• pp.322-328
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• 2011

Nephrotic syndrome (NS) is one of the most common glomerular diseases that affect children. Renal histology reveals the presence of minimal change nephrotic syndrome (MCNS) in more than 80% of these patients. Most patients with MCNS have favorable outcomes without complications. However, a few of these children have lesions of focal segmental glomerulosclerosis, suffer from severe and prolonged proteinuria, and are at high risk for complications. Complications of NS are divided into two categories: disease-associated and drug-related complications. Disease-associated complications include infections (e.g., peritonitis, sepsis, cellulitis, and chicken pox), thromboembolism (e.g., venous thromboembolism and pulmonary embolism), hypovolemic crisis (e.g., abdominal pain, tachycardia, and hypotension), cardiovascular problems (e.g., hyperlipidemia), acute renal failure, anemia, and others (e.g., hypothyroidism, hypocalcemia, bone disease, and intussusception). The main pathomechanism of disease-associated complications originates from the large loss of plasma proteins in the urine of nephrotic children. The majority of children with MCNS who respond to treatment with corticosteroids or cytotoxic agents have smaller and milder complications than those with steroid-resistant NS. Corticosteroids, alkylating agents, cyclosporin A, and mycophenolate mofetil have often been used to treat NS, and these drugs have treatment-related complications. Early detection and appropriate treatment of these complications will improve outcomes for patients with NS.

• Journal of Chest Surgery
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• 제25권12호
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• pp.1550-1555
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• 1992

From June 1989 to July 1992, we used centrifugal Biomedicus pump[CBP] in 20 patients In 9 cases, CBP was used as ventricular assistance after heart surgery for those who could not be weaned off bypass even with intra-aortic balloon counter-pulsation and with maximal inotropic support In 8 patients, CBP was used as partial left heart bypass during repair of aortic aneurysms or congenital aortic anomalies. And in 3 patients, CBP was used as vena caval bypass during resection of renal cell carcinoma with tumor extension into the inferior vena cava. In 2 of 9 patients with ventricular assistance, they were weaned off the device successfully after 16 hours and 7 days respectively. But the patients died of intracranial hemorrhage and sepsis, 7 and 29 days after weaning from cardiac support, respectively. In all the patients who underwent aortic of vena caval surgery using CBP as shunt, there were no complications such as postoperative bleeding necessitating reoperation, renal failure or neurologic sequelae. In conclusion, the centrifugal type of ventricular assistance may be potentially life saving treatment modality in patients with severe postoperative low cardiac output syndrome. The CBP can be safely employed for resection of renal cell carcinoma with vena caval tumor extension and for repair of aortic aneurysms.

• 대한유전성대사질환학회지
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• 제14권1호
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• pp.10-18
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• 2014

Recent advances in the diagnosis and treatment of inborn errors of metabolism have improved substantially the prognosis of many of these diseases, if diagnosed early enough before irreversible damage occurs. This makes it essential that the practicing pediatrician, especially neonatologists be familliar with the clinical presentations and systematic approaches of these disorders. Characteristic clinical presentations, methods of systematic approach and typing of various disorders is discussed in this review. The signs of neurological dysfunctions of many IEMs manifesting in the neonatal period is very nonspecific, such as poor feeding, poor sucking, apnea or tachypnea, vomiting, hypertonia, hypotonia, seizure, letharginess, consciousness change and coma. Many other non-metabolic severe disorders of neonatal period such as neonatal sepsis and intracerebral hemorrhage share these nonspecific symptoms. Hyperammonemia, metabolic acidosis, ketosis and hyperlatic acidemia are observed in many of these conditions but there are exceptions in which conditions all basal laboratory tests are normal, such as NKH, sulfite oxidase deficiency and peroxisomal disorders. According to the results of basal laboratory tests, IEMs in the neonatal period can be categorized in to 6 types. Grouping of IEMs into 6 types will make confirmatory tests and early emergency treatment more efficient.

• Journal of Chest Surgery
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• 제19권3호
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• pp.385-390
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• 1986

Empyema is a severe infection encountered in the pediatrics. With advance of the antibiotics and chemotherapeutics, there was a marked decrease in number of empyema. Empyema complicated by staphylococcal pneumonia in infant and children has been distressing problem, and the management of this complication has been discussed repeatedly in the past. In Korea, tuberculous empyema is also troublesome. If empyema is localized within thick capsule, tube thoracostomy and closed drainage alone is unacceptable, and early open thoracotomy to eliminate the empyema has proved good result. A clinical analysis of 39 patients with thoracic empyema was done. They were managed surgical intervention at Dept. of Thoracic & Cardiovascular Surgery at Kyung-Hee University Hospital from Jan. 1974 to December, 1984. 1. Age and sex distribution, infancy 9, early childhood 11. late childhood 9, puberty 10. The male to female ratio was 21:18. 2. The highest seasonal incidence was winter [21 cases]. 3. Cardinal symptoms were cough [76%], fever and chill [66%], and dyspnea [40%]. 4. The location of the empyema was right in 27 cases [69%] and 12 cases in left side. 5. The most frequent lesion to predisposing factor was pneumonia [67%]. 6. The commonest organism was Staphylococcus aureus in 15 [38%] cases, and Mycobacterium tuberculosis in 10 cases [26%]. 7. The surgical treatment was performed in all patients. The surgical procedure was closed tube thoracostomy in 25 cases [64%], decortication in 7 cases [18%], pulmonary resection in 4 cases [10%], and decortication with curettage in 2 cases. 8. One patient died from sepsis complicated by lymphoma and in one patient bronchopleural fistula was developed postoperatively.

• 식품보건융합연구
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• 제5권4호
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• pp.25-28
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• 2019

Now a days problem in health has become common. So, instead of curing them, prevention through dietary supplements has proven to be useful. In the case of patients who have already developed the disease atleast relieving pain and suffer is a challenging thing. In this context L- arginine is doing better compared to other essential aminoacids up to some extent. Arginine was found to reduce the pain associated with pulmonary hypertension foun to be associated with sickle cell anaemia. It also reduces the reperfusion injury after ischemia, trauma and shock. Some of the drugs with L-arginine as component are under clinical trials and hope to be available in the market soon. Severe preeclampsia is characterised by headaches, blurred vision, and inability to have high photovision, nausea and vomiting. L-Arginine along with Vit C and E are given as medical food to the patients and decrease in condition symptoms is the project now under phase II clinical trial. However the role of arginine in ameolirating preeclampsia symptoms is uncertain except with that of hypertension. Arginine is used to treat pain in sickle cell anaemia, lung damage, reperfusion injury, Trauma and shock but should be excluded during sepsis.