Article 53(6); Jun 2010
Review Articles
Clinical presentations of critical cardiac defects in the newborn: Decision making and initial management
Jae Young Lee
Clin Exp Pediatr. 2010;53(6):669-679.   Published online June 23, 2010

The risk of mortality and morbidity of patients with congenital heart defects (CHDs) is highest during neonatal period and increases when diagnosis and proper management are delayed. Neonates with critical CHDs may present with severe cyanosis, respiratory distress, shock, or collapse, all of which are also frequent clinical presentations of various respiratory problems or sepsis in the newborn. Early diagnosis...

Practical stepwise approach to rhythm disturbances in congenital heart diseases
June Huh
Clin Exp Pediatr. 2010;53(6):680-687.   Published online June 23, 2010

Patients with congenital heart diseases (CHD) are confronted with early- and late-onset complications, such as conduction disorders, arrhythmias, myocardial dysfunction, altered coronary flow, and ischemia, throughout their lifetime despite successful hemodynamic and/or anatomical correction. Rhythm disturbance is a well-known and increasingly frequent cause of morbidity and mortality in patients with CHD. Predisposing factors to rhythm disturbances include underlying cardiac defects,...

Pulmonary hypertension in infants with bronchopulmonary dysplasia
Gi Beom Kim
Clin Exp Pediatr. 2010;53(6):688-693.   Published online June 23, 2010

An increase in the number of preterm infants and a decrease in the gestational age at birth have resulted in an increase in the number of patients with significant bronchopulmonary dysplasia (BPD) and secondary pulmonary hypertension (PH). PH contributes significantly to the high morbidity and mortality in the BPD patients. Therefore, regular monitoring for PH by using echocardiography and B-type...

Original Articles
Effect of severe neonatal morbidities on long term outcome in extremely low birthweight infants
Kyo Yeon Koo, Jeong Eun Kim, Soon Min Lee, Ran Namgung, Min Soo Park, Kook In Park, Chul Lee
Clin Exp Pediatr. 2010;53(6):694-700.   Published online June 23, 2010
Purpose

To assess the validity of individual and combined prognostic effects of severe bronchopulmonary dysplasia (BPD), brain injury, retinopathy of prematurity (ROP), and parenteral nutrition associated cholestasis (PNAC).

Methods

We retrospectively analyzed the medical records of 80 extremely low birthweight (ELBW) infants admitted to the neonatal intensive care unit (NICU) of the Severance Children's Hospital, and who survived to a postmenstrual age of...

International travel of Korean children and Dengue fever: A single institutional analysis
Soo Han Choi, Yae Jean Kim, Ji Hun Shin, Keon Hee Yoo, Ki Woong Sung, Hong Hoe Koo
Clin Exp Pediatr. 2010;53(6):701-704.   Published online June 23, 2010
Purpose

Dengue fever occurs in many popular tourist destinations and is increasingly imported by returning travelers in Korea. Since Korea is not an endemic country for dengue fever, pediatricians do not usually suspect dengue fever in febrile children even with typical presentation and exposure history. This study was performed to describe the international travel experiences and dengue fever in Korean children.

Methods

Travel...

An 18-year experience of tracheoesophageal fistula and esophageal atresia
Juhee Seo, Do Yeon Kim, Ai Rhan Kim, Dae Yeon Kim, Seong Chul Kim, In Koo Kim, Ki Soo Kim, Chong Hyun Yoon, Soo Young Pi
Clin Exp Pediatr. 2010;53(6):705-710.   Published online June 23, 2010
Purpose

To determine the clinical manifestations and outcomes of patients with tracheoesophageal fistula (TEF) and esophageal atresia (EA) born at a single neonatal intensive care unit.

Methods

A retrospective analysis was conducted for 97 patients with confirmed TEF and EA who were admitted to the neonatal intensive care unit between 1990 and 2007.

Results

The rate of prenatal diagnosis was 12%. The average gestational age...

Analysis of reports on orphan lung diseases in Korean children
Sun Jung Jang, Hyun Kyung Seo, Sung Jae Yi, Kyong Min Kim, Hye Mi Jee, Man Yong Han
Clin Exp Pediatr. 2010;53(6):711-717.   Published online June 23, 2010
Purpose

Orphan lung diseases are defined as lung diseases with a prevalence of 1 or less in 2,000 individuals. Despite an increase in the numbers of patients with such diseases, few studies on Korean children have appeared. To obtain epidemiologic and demographic data on these diseases, we systematically reviewed reports on pediatric orphan lung diseases in Korea over the last 50...

Case Reports
A case of Rubinstein-Taybi Syndrome with a CREB-binding protein gene mutation
Se Hee Kim, Byung Chan Lim, Jong Hee Chae, Ki Joong Kim, Yong Seung Hwang
Clin Exp Pediatr. 2010;53(6):718-721.   Published online June 23, 2010

Rubinstein-Taybi syndrome (RTS) is a congenital disorder characterized by typical facial features, broad thumbs and toes, with mental retardation. Additionally, tumors, keloids and various congenital anomalies including congenital heart defects have been reported in RTS patients. In about 50% of the patients, mutations in the CREB binding protein (CREBBP) have been found, which are understood to be associated with cell...

A case report of chronic granulomatous disease presenting with aspergillus pneumonia in a 2-month old girl
Eun Lee, Seak Hee Oh, Ji Won Kwon, Byoung Ju Kim, Jinho Yu, Chan Jeoung Park, Soo Jong Hong
Clin Exp Pediatr. 2010;53(6):722-726.   Published online June 23, 2010

Chronic granulomatous disease (CGD) is an uncommon inherited disorder caused by mutations in any of the genes encoding subunits of the superoxide-generating phagocyte NADPH oxidase system, which is essential for killing catalase producing bacteria and fungi, such as Aspergillus species, Staphylococcus aureus, Serratia marcescens, Nocardia species and Burkholderia cepacia. In case of a history of recurrent or persistent infections, immune...

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