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Dong Soo KIM ; Kwan Sub CHUNG ; Kir Young KIM ; Duk Jin YUN

Journal of the Korean Pediatric Society.1980;23(10):809-814.

9 classic hemophilacs in Severance Hospital, Yonsei University from Dec. '77 to Aug. '79 were reviewed and analysed and the following results were obtained. 1. Intracranial hemorrhage is required the most immediate emergent therapy in hemophiliacs and computerized transverse axial tomogarhy may be essential for the accurate and noninvasive diagnosis and for the effective emergency treatment. 2. To conserve supplies of therapeutic materials and to decrease the possibility of transmitting hepatitis and other diseases with blood products, it is desirable to reduce the amount of blood products with the use of epsilone aminocaproic acid, and epsilone aminocaproic acid may be useful in extraction and/or other non-dental injury with hemophiliacs.
Aminocaproic Acid ; Diagnosis ; Emergencies* ; Emergency Treatment ; Equipment and Supplies ; Hemophilia A* ; Hepatitis ; Intracranial Hemorrhages

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Clinical Studies on the Patent Ductus Arteriosus.

Baek Keun LIM ; Sung Kyu LEE ; Duk Jin YON ; Seung Nok HONG ; Duk Jin YUN

Journal of the Korean Pediatric Society.1980;23(10):798-808.

Patent Ductus Arteriosus is one of the most common congenital heart diseases. The symptoms and clinical findings in patients with typical patnet ductus arteriosus are well recognized. That not all patient with ductus arteriosus have the typical clinical findings is well documented. Since the poor prognosis of untreated patent ductus arteriosus and good results of surgical intervention are fairly well established fact, it is important that all altered in according to hemodynamic changes depending on the pulmonary pressure, amount of shunt, degree of resistance of pulmonary circulation. This series comprises 165 patients of patent ductus arteriosus who had been admitted to Severance Hospital from Apr. 1964 to Aug. 1979. An attempt was made to correlate the hemodynamic data with age. The results of this study may be summarized briefly as follows : 1.Of the 165 patients, 67 were male and 98 were female. Their age range from 1 day to 48 years. About 50% of all patients was below 10 years. 2.Major symptoms on admission were frequent URI, exertional dyspnea, palpitation and evaluation of known congenital heart disease etc. 3.Of 165 patients, continuous murmur was audible in 128 patients and systolic murmur in 28 patients. 4.The children (below 15 years of age) had the variable distribution of pulmonary to systemic flow ratio (Qp/Qs) Most of adult had the Qp/Qs of below 3. 5.Severe pulmonary hypertension was noted in 13% of right heart catheterized patients. 6.There was no obvious correlation between aging and pulmonary wascular resistance or pulmonary hypertension. 7.There was linear correlation between the diameter of ductus and pulmonary blood flow. 8.The left atrium to aortic root dimension ratio in echocardiogram showed correlation with the pulmonary to systemic flow ratio measured at catherization (r=0.45) 9.Operative mortality was 2.5% in 165 cases of isolated patent ductus arteriosus.
Adult ; Aging ; Cardiac Catheters ; Child ; Ductus Arteriosus ; Ductus Arteriosus, Patent* ; Dyspnea ; Female ; Heart Atria ; Heart Defects, Congenital ; Heart Diseases ; Hemodynamics ; Humans ; Hypertension, Pulmonary ; Male ; Mortality ; Prognosis ; Pulmonary Circulation ; Systolic Murmurs

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Therapeutic Effect of Rifampin in Shigellosis.

Chul LEE ; Jun Hee SUL ; Pyung Kil KIM ; Duk Jin YUN

Journal of the Korean Pediatric Society.1980;23(10):791-797.

Twenty one infants and children were entered into the study protocol when bacteriologic diagnosis of shigellosis was made by stool culture indenification. Fifteen patients were treated at Inje Hospital in Kangwon Do and six patients were treated at Severance Hospital in Seoul. Susceptibility studies were revealed that ampicillin was resistant in all cases. Gentamicin and Panimycin which are aminoglycosides were quite effective. But their use was limited because of their nephrotoxicity and those antibiotics should be used only by parenteral route. A daily dose of 20microgram per kilogram of body weight of rifampin was given by mouth divided every 6 hrs for 7 days routinely except 2 cases. After that follow up stool culture were performed on 13 patient. The results were negative except one case. There was no untoward reaction of rifampin during therapy except for the red coloring of urine and stool. To prevent developing the resistance of shigella to rifampin, it is necessary to confirm that negative conversion of stool cultures has been achieved. Our experience should encourge the use of rifampin in the treatment of shigellosis.
Aminoglycosides ; Ampicillin ; Anti-Bacterial Agents ; Body Weight ; Child ; Diagnosis ; Dysentery, Bacillary* ; Follow-Up Studies ; Gangwon-do ; Gentamicins ; Humans ; Infant ; Mouth ; Rifampin* ; Seoul ; Shigella

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Clinical and Statistical Study On the Congenital Anomalies of the Digestive System.

Que Sang LEE ; Bock Keun KEE ; Myung Ho LEE ; Ki Jong NAM

Journal of the Korean Pediatric Society.1980;23(10):781-790.

This clinical and statistical obsrvation was made on 120 pediatric patients admitted to the Pediatric and Surgical Departments of Presbyterian Medical Center between Januarn, 1972 and July, 1979. A total of 120 cases were analyzed according to 1) distribution and incidence of the congenital anomalies of the G.I. tract; 2) age distribution according to onset of symptom and sign; 3) surgical treatment and mortality; and 4) associaties with gastrointestinal anomalies.The results were summerized as follwos: 1. In the sex ration to female was 3.6:1. 2. The order of frequency of diseases was as follows: congenial megacolon 37 cases(30.8%), anorectal malformation 26 cases(21.8%); congenital hypertrophic stenosis 21 cases(17.5%); congenital anomalies of hepatobiliary system 13 cases (10.8%); malroation 5 cases(4.1%); esophageal atresia 5 cases(4.1%); atresia of samll intestine 3 cases(2.5%); movable cecum 3 cases(2.5%); Meckel's diverticulum 2 cases(1.7%); diaphragmatic hernia 2 cases(1.7%); omphalocele 2 cases(1.7%); and, anular pancreas 1 cases(0.8%). 3. There was an over all mortality rate of 10 cases(11.2%). 4. Associated congenital anomalies were found in 24 cases(20%).
Age Distribution ; Cecum ; Constriction, Pathologic ; Digestive System* ; Esophageal Atresia ; Female ; Hernia, Diaphragmatic ; Hernia, Umbilical ; Humans ; Incidence ; Intestines ; Meckel Diverticulum ; Megacolon ; Mortality ; Pancreas ; Protestantism ; Statistics as Topic*

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A Case of Ectopic Pancreas with Malrotation.

Il Song KANG ; Kil Soo CHUNG ; Jae Kook CHA ; Kon Hee LEE ; Kwan Seop LEE ; Byung Chun KIM ; Eun Sook NAM

Journal of the Korean Pediatric Society.1998;41(10):1438-1441.

Ectopic pancreas is defined as pancreatic tissue lacking anatomical and vascular continuity with the main body of the pancreas. Common symptoms are epigastric pain, hemorrhage, chest pain, weight loss, nausea and vomiting. A 2-month-old male patient was hospitalized because of continuous bile stained vomiting for 2 days. Abdominal ultrasonography and colon study showed malrotation of the intestine. Surgical exploration revealed the ectopic pancreas on the 10cm lower portion of Treitz ligament and rotation of terminal ileum. We performed adhesiolysis, small bowel resection and anastomosis. The diagnosis of ectopic pancreas was made by histologic examination. We report a case of ectopic pancreas with intestinal adhesion and malrotation with brief review of related literatures.
Bile ; Chest Pain ; Colon ; Diagnosis ; Hemorrhage ; Humans ; Ileum ; Infant ; Intestines ; Ligaments ; Male ; Nausea ; Pancreas* ; Ultrasonography ; Vomiting ; Weight Loss

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A Case of Williams Syndrome Diagnosed by FISH.

Hyun KIM ; Won Sun LEE ; Won Bae LEE

Journal of the Korean Pediatric Society.1998;41(10):1433-1437.

Williams syndrome was first reported in 1961, it is characterized by distinct facial changes, growth deficiency, mental retardation, congenital heart defect (particularly the supravalvular aortic stenosis), associated at times with infantile hypercalcemia. The diagnosis of this disease relied on phenotype, after recent acknowledgement of its genetic basis on the 7th chromosomal locus q11.23 involving elastin gene on the PCR. Recently, commercial probe of elastin gene for FISH was supplied and diagnosed this disease easier. Using PCR has some benefits concerning whether the origin of the genetic defect is maternal or paternal. The diagnosis of this disease is difficult due to low sensitivity, below 50%. But FISH is widely used because it is faster with high positive predictibility. We report Williams syndrome diagnosed by FISH with a brief review and related literatures.
Diagnosis ; Elastin ; Heart Defects, Congenital ; Hypercalcemia ; Intellectual Disability ; Phenotype ; Polymerase Chain Reaction ; Williams Syndrome*

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A Case of Inappropriate ADH Secretion Secondary to Vinblastine Therapy.

Eun Hye PARK ; Hyun Sang CHO ; Hae Sook YOO ; Chuhl Joo LYU ; Chang Hyun YANG ; Kir Young KIM

Journal of the Korean Pediatric Society.1998;41(10):1430-1432.

Inappropriate secretion of antidiuretic hormone (ADH) secondary to vincristine therapy has been reported frequently. But there has been five previous reports of vinblastine associated SIADH in the world and in 4 reports, bleomycin and cis-platinum were used along with vinblastine together. Because penetration of vinka alkaloids into CSF of humans is known to be poor, it has been suggested that lowering the osmotic threshold for vasopressin release induces SIADH in humans. In our case, the patient did not receive cis-platinum during chemotherapy. From this we concluded that vinblastine alone can induce SIADH without nephrotoxicity of cis-platinum. We experienced a case of inappropriate ADH secretion secondary to vinblastine therapy in a 5- month-old male infant who has been taking vinblastine due to Langerhans cell histiocytosis. We report this case with a brief review of related literatures.
Alkaloids ; Bleomycin ; Cisplatin ; Drug Therapy ; Histiocytosis, Langerhans-Cell ; Humans ; Inappropriate ADH Syndrome ; Infant ; Male ; Vasopressins ; Vinblastine* ; Vincristine

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A Case of Congenital Self-Healing Reticulohistiocytosis.

Taek Sun KIM ; Chang Wan KIM ; Eun Ryoung KIM ; You Mee KANG ; Kye Yong SONG

Journal of the Korean Pediatric Society.1998;41(10):1424-1439.

Congenital self-healing reticulohistiocytosis (CSHR) is a rare variant of cutaneous reticulohistiocytosis. We report a newborn who had about 25 reddish brown papulonodular lesions on whole body at birth. No extracutaneous involvement was found. All skin lesions involuted within 4 months spontaneously. Biopsy of a skin lesion showed marked aggregated histiocytic infiltration with some eosinophils. S-100 protein and lysozyme were positive. Electron microscopy revealed numerous dense bodies, often with a myelin-like core and some Birbeck granules. We described a detailed clinical features of this case and reviewed the literatures.
Biopsy ; Eosinophils ; Humans ; Infant, Newborn ; Microscopy, Electron ; Muramidase ; Parturition ; S100 Proteins ; Skin

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A Case of Pulmonary Artery Sling Associated with Left Bronchial Stenosis and Patent Ductus Arteriosus.

Woo Suk JUHNG ; Myung Hee HAN ; Chan Uhng JOO

Journal of the Korean Pediatric Society.1998;41(10):1417-1423.

Pulmonary artery sling is a rare and potentially lethal vascular anomaly wherein the anomalous left pulmonary artery arises from the posterior aspect of the right pulmonary artery and passing to the left lung between the trachea and the esophagus which causes respiratory distress in infants and children due to the extrinsic compression of the airway by the anomalous vessel. Associated anomalies are common, particularly those of the tracheobronchial tree. The most common lesions are hypoplasia of the distal trachea and right main stem bronchus. About 50 percent of patients have cardiovascular anomalies, such as in the left superior vena cava, atrial septal defect, ventricular septal defect, patent ductus arteriosus, aortic arch anomalies, tetralogy of Fallot 1 etc. We experienced a case of a 6-year-old boy who showed dyspnea and frequent pneumonia. He was diagnosed with pulmonary artery sling associated with left bronchial stenosis, patent ductus arteriosus, and left superior vena cava which was parven by echocardiographic examinations, spiral computerized tomography of the chest and cineangiography. We report this case with related literature.
Aorta, Thoracic ; Bronchi ; Child ; Cineangiography ; Constriction, Pathologic* ; Ductus Arteriosus, Patent* ; Dyspnea ; Echocardiography ; Esophagus ; Heart Septal Defects, Atrial ; Heart Septal Defects, Ventricular ; Humans ; Infant ; Lung ; Male ; Pneumonia ; Pulmonary Artery* ; Tetralogy of Fallot ; Thorax ; Tomography, Spiral Computed ; Trachea ; Vena Cava, Superior

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The Clinical Study on Marfan Syndrome.

Jung Sim KIM ; Dong Kyu JIN ; See Hwan KO ; Jee Yeon MIN ; I Seok KANG ; Kye Won JEON ; Yung Lan CHOI ; Heung Jae LEE ; Seung Woo PARK ; Won Ro LEE ; Chong Suh LEE ; Sei Yeul OH ; Chang Won KI ; Han Wook YOO ; In Sook PARK ; Jae Kon KO

Journal of the Korean Pediatric Society.1998;41(10):1411-1416.

PURPOSE: The aim of this study was to assess the involvement of several organs patients with Marfan syndrome in Korea. Also the clinical features in childhood patients with Marfan syndrome were assessed. METHODS: Thirty-eight cases of Marfan syndrome were enrolled in this study. Clinical evaluations of the musculoskeletal, cardiovascular and occular system were performed in all cases. RESULTS: The musculoskeletal system was involved in 32 cases (84.2%) and occular system in 24 cases (63.1%). Cardiovascular abnormalities were found in 19 cases (50.0%) at initial evaluation. Family history was involved in 21 cases (55.2%). Ectopia lentis was found in 17 cases (70.8%). Severe myopia and iris abnormalities were also present in 14 cases (58.2%). The ascending aorta was dilated in 13 cases (34.2%). Emergency operation was performed in 3 cases (7.9%) because of a dissecting aorta. Mitral regurgitation and prolapse were found in 29 cases (76.4%) and other valve insufficiency was accompainied in 5 cases (13.1%). Of the 38 cases, 29 patients (79.3%) were less than 15 years of age and their major manifestations were occular problems in 23 cases (79.3%), and family history in 17 cases (58.6%). In one infant, severe heart failure was the predominant clinical feature. CONCLUSION: The clinical features of Korean patients with Marfan syndrome were summarized in this report. Heart failure was the main manifestaton in infantile Marfan syndrome. Early treatment with beta-blocker and valvular replacement can prevent fatality, i.e. aortic dissection, in this disease, concern and management should be advocated in the early detection of Marfan syndrome.
Aorta ; Cardiovascular Abnormalities ; Ectopia Lentis ; Emergencies ; Heart Failure ; Humans ; Infant ; Iris ; Korea ; Marfan Syndrome* ; Mitral Valve Insufficiency ; Musculoskeletal System ; Myopia ; Prolapse

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