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Review Article
Systematic review of the clinical and genetic aspects of Prader-Willi syndrome
Dong Kyu Jin
Clin Exp Pediatr. 2011;54(2):55-63.   Published online February 28, 2011

Prader-Willi syndrome (PWS) is a complex multisystem genetic disorder that is caused by the lack of expression of paternally inherited imprinted genes on chromosome 15q11-q13. This syndrome has a characteristic phenotype including severe neonatal hypotonia, early-onset hyperphagia, development of morbid obesity, short stature, hypogonadism, learning disabilities, behavioral problems, and psychiatric problems. PWS is an example of a genetic condition caused...

Vitamin D dependent rickets type I
Chan Jong Kim
Clin Exp Pediatr. 2011;54(2):51-54.   Published online February 28, 2011

Vitamin D is present in two forms, ergocalciferol (vitamin D2) produced by plants and cholecalciferol (vitamin D3) produced by animal tissues or by the action of ultraviolet light on 7-dehydrocholesterol in human skin. Both forms of vitamin D are biologically inactive pro-hormones that must undergo sequential hydroxylations in the liver and the kidney before they can bind to and activate...

Nonpharmacological management and psychosocial support for children and adolescents with type 1 diabetes
Jae Ho Yoo
Clin Exp Pediatr. 2011;54(2):45-50.   Published online February 28, 2011

Compared to that in the Caucasian population, type 1 diabetes mellitus (T1DM) incidence rates are very low in Koreans. Therefore, compared to the recent development of pharmacological therapy applicable to Korean children with T1DM, interest in nonpharmacological therapy and psychosocial support systems remains low, as is the development of Korean-style T1DM education programs for therapeutic application. Children who have been...

Oral food challenges in children
Hye Yung Yum, Hyeon Jong Yang, Kyung Won Kim, Tae Won Song, Woo Kyung Kim, Jung Hee Kim, Kang Mo Ahn, Hyun Hee Kim, Soo Young Lee, Bok Yang Pyun
Clin Exp Pediatr. 2011;54(1):6-10.   Published online January 31, 2011

Many patients assume that allergic reactions against foods are responsible for triggering or worsening their allergic symptoms. Therefore, it is important to identify patients who would benefit from an elimination diet, while avoiding unnecessary dietary restrictions. The diagnosis of food allergy depends on the thorough review of the patients's medical history, results of supplemented trials of dietary elimination, and in...

Asthma in childhood: a complex, heterogeneous disease
Hai Lee Chung
Clin Exp Pediatr. 2011;54(1):1-5.   Published online January 31, 2011

Asthma in childhood is a heterogeneous disease with different phenotypes and variable clinical manifestations, which depend on the age, gender, genetic background, and environmental influences of the patients. Several longitudinal studies have been conducted to classify the phenotypes of childhood asthma, on the basis of the symptoms, triggers of wheezing illness, or pathophysiological features of the disease. These studies have...

Ureaplasma infections in pre-term infants: Recent information regarding the role of Ureaplasma species as neonatal pathogens
Tae-Jung Sung
Clin Exp Pediatr. 2010;53(12):989-993.   Published online December 31, 2010

Although numerous clinical observational studies have been conducted over a period of over 30 years, the clinical significance of Ureaplasma infection is still under debate. The Ureaplasma speices. is a commensal in the female genital tract and considered to have of low virulence; however, Ureaplasma colonization has been associated with infertility, stillbirth, preterm delivery, histologic chorioamnionitis, and neonatal morbidities, including...

Neonatal innate immunity and Toll-like receptor
Hye Sun Yoon
Clin Exp Pediatr. 2010;53(12):985-988.   Published online December 31, 2010

The innate immune response is the first line of defense against microbial infections. Innate immunity is made up of the surface barrier, cellular immunity and humoral immunity. In newborn, immunologic function and demands are different to adults. Neonatal innate immunity specifically suppresses Th1-type immune responses, and not Th2-type immune responses, which are enhanced. And the impaired response of macrophages is...

Lung interstitial cells during alveolarization
Chang Won Choi
Clin Exp Pediatr. 2010;53(12):979-984.   Published online December 31, 2010

Recent progress in neonatal medicine has enabled survival of many extremely low-birth-weight infants. Prenatal steroids, surfactants, and non-invasive ventilation have helped reduce the incidence of the classical form of bronchopulmonary dysplasia characterized by marked fibrosis and emphysema. However, a new form of bronchopulmonary dysplasia marked by arrest of alveolarization remains a complication in the postnatal course of extremely low-birth-weight infants....

Treatment of juvenile rheumatoid arthritis
Kwang Nam Kim
Clin Exp Pediatr. 2010;53(11):936-941.   Published online November 30, 2010

The systematic approach to pharmacologic treatment is typically to begin with the safest, simplest, and most conservative measures. It has been realized that the more rapidly inflammation is under control, the less likely it is that there will be permanent sequelae. Nonsteroidal anti-inflammatory drugs (NSAIDs) are the mainstay of initial treatment for inflammation. In addition, the slow-acting antirheumatic drugs (SAARDs)...

Juvenile idiopathic arthritis: Diagnosis and differential diagnosis
Ki Hwan Kim, Dong Soo Kim
Clin Exp Pediatr. 2010;53(11):931-935.   Published online November 30, 2010

Juvenile idiopathic arthritis (JIA) is comprised of a heterogeneous group of several disease subtypes that are characterized by the onset of arthritis before the age of 16 years and has symptoms lasting at least 6 weeks. The previous classification of JIA included seven different categories, whereas its current classification was compiled by the International League of the Association for Rheumatology,...

Pathogenesis and clinical manifestations of juvenile rheumatoid arthritis
Youn-Soo Hahn, Joong-Gon Kim
Clin Exp Pediatr. 2010;53(11):921-930.   Published online November 30, 2010

Juvenile rheumatoid arthritis (JRA) is the most common rheumatic childhood disease; its onset is before 16 years of age and it persists for at least 6 weeks. JRA encompasses a heterogeneous group of diseases that is classified according to 3 major presentations: oligoarthritis, polyarthritis, and systemic onset diseases. These presentations may originate from the same or different causes that involve...

Treatment of obstructive sleep apnea in children
Young Min Ahn
Clin Exp Pediatr. 2010;53(10):872-879.   Published online October 31, 2010

Obstructive sleep apnea (OSA) in children is a frequent disease for which optimal diagnostic methods are still being defined. Treatment of OSA in children should include providing space, improving craniofacial growth, resolving all symptoms, and preventing the development of the disease in the adult years. Adenotonsillectomy (T&A) has been the treatment of choice and thought to solve young patient's OSA...

Obstructive sleep apnea syndrome in children: Epidemiology, pathophysiology, diagnosis and sequelae
Sun Jung Chang, Kyu Young Chae
Clin Exp Pediatr. 2010;53(10):863-871.   Published online October 31, 2010

The prevalence of pediatric obstructive sleep apnea syndrome (OSAS) is approximately 3% in children. Adenotonsillar hypertrophy is the most common cause of OSAS in children, and obesity, hypotonic neuromuscular diseases, and craniofacial anomalies are other major risk factors. Snoring is the most common presenting complaint in children with OSAS, but the clinical presentation varies according to age. Agitated sleep with...

Change in the treatment strategy for pediatric Crohn's disease
Mi Jin Kim, Yon Ho Choe
Clin Exp Pediatr. 2010;53(9):830-833.   Published online September 13, 2010

Crohn's disease is characterized by chronic inflammation involving any portion of the gastrointestinal tract. Treating Crohn's disease is a major challenge for clinicians, as no curative therapy currently exists. Pediatric Crohn's disease is characterized by frequent relapses, a wide extent of disease, a high prevalence of extraintestinal manifestations, and a severe clinical course. The classic therapeutic approach is known as...

Role of colonoscopy in the diagnosis and treatment of pediatric lower gastrointestinal disorders
Jae Hong Park
Clin Exp Pediatr. 2010;53(9):824-829.   Published online September 13, 2010

The safety and effectiveness of colonoscopy in the investigation of lower gastrointestinal tract pathology in children has been established for more than 2 decades in Korea. Skill and experience have since advanced to the point that both diagnostic and therapeutic colonoscopy are now routinely performed by most pediatric gastroenterologists. Pediatric colonoscopy differs significantly from its adult parallels in nearly every...

General considerations and updates in pediatric gastrointestinal diagnostic endoscopy
Yong Joo Kim
Clin Exp Pediatr. 2010;53(9):817-823.   Published online September 13, 2010

Gastrointestinal and colonic endoscopic examinations have been performed in pediatric patients in Korea for 3 decades. Endoscopic procedures are complex and may be unsafe if special concerns are not considered. Many things have to be kept in mind before, during, and after the procedure. Gastrointestinal endoscopy is one of the most frequently performed procedure in children nowadays, Since the dimension...

Disease-specific pluripotent stem cells
Hoon-Chul Kang
Clin Exp Pediatr. 2010;53(8):786-789.   Published online August 31, 2010

Induced pluripotent stem (iPS) cells are generated by epigenetic reprogramming of somatic cells through the exogenous expression of transcription factors. Recently, the generation of iPS cells from patients with a variety of genetic diseases was found to likely have a major impact on regenerative medicine, because these cells self-renew indefinitely in culture while retaining the capacity to differentiate into any...

Multimodal neuroimaging in presurgical evaluation of childhood epilepsy
Da Eun Jung, Joon-Soo Lee
Clin Exp Pediatr. 2010;53(8):779-785.   Published online August 31, 2010

In pre-surgical evaluation of pediatric epilepsy, the combined use of multiple imaging modalities for precise localization of the epileptogenic focus is a worthwhile endeavor. Advanced neuroimaging by high field Magnetic resonance imaging (MRI), diffusion tensor images, and MR spectroscopy have the potential to identify subtle lesions. 18F-FDG positron emission tomography and single photon emission tomography provide visualization of metabolic alterations...

Renal fibrosis
Min Hyun Cho
Clin Exp Pediatr. 2010;53(7):735-740.   Published online July 31, 2010

Renal fibrosis, characterized by tubulointerstitial fibrosis and glomerulosclerosis, is the final manifestation of chronic kidney disease. Renal fibrosis is characterized by an excessive accumulation and deposition of extracellular matrix components. This pathologic result usually originates from both underlying complicated cellular activities such as epithelial-to-mesenchymal transition, fibroblast activation, monocyte/macrophage infiltration, and cellular apoptosis and the activation of signaling molecules such as...

Genetics of kidney development: pathogenesis of renal anomalies
Hyewon Hahn
Clin Exp Pediatr. 2010;53(7):729-734.   Published online July 31, 2010

Congenital anomalies of the kidney and urinary tract (CAKUT) account for more than 50% of abdominal masses found in neonates and involve about 0.5% of all pregnancies. CAKUT has a major role in renal failure, and increasing evidence suggests that certain abnormalities predispose to the development of hypertension and cardiovascular disease in adulthood. To understand the pathogenesis of human renal...

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...

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,...

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...

Update on pertussis and pertussis immunization
Jung Yun Hong
Clin Exp Pediatr. 2010;53(5):629-633.   Published online May 31, 2010

Pertussis is a highly contagious respiratory tract disease caused by Bordetella pertussis infection. The clinical manifestation of this infection can be severe enough to cause death. Although pertussis has been supposed to be a vaccine-preventable disease ever since the widespread vaccination of children against pertussis was started, since the 1990s, cases of pertussis and related fatalities are on the rise,...

Reemergence of mumps
Kyong Min Choi
Clin Exp Pediatr. 2010;53(5):623-628.   Published online May 31, 2010

The mumps virus is a single-stranded, non-segmented, negative-sense RNA virus belonging to the Paramyxoviridae family. Mumps is characterized by bilateral or unilateral swelling of the parotid gland. Aseptic meningitis is a common complication, and orchitis is also common in adolescents and adult men. Diagnosis is based on clinical findings, but because of high vaccination coverage, clinical findings alone are not...

Enterovirus 71 infection: An experience in Korea, 2009
Kyung Hyo Kim
Clin Exp Pediatr. 2010;53(5):616-622.   Published online May 31, 2010

Enterovirus 71 (EV71) has been recognized as a frequent cause of epidemics of hand-foot-and-mouth disease (HFMD) associated with severe neurological symptoms. In the spring of 2009, HFMD was epidemic in Korea. Severe cases with complication, including death, have been reported and it has become a public health issue. Most symptomatic EV71 infections commonly result in HFMD or herpangina. These clinical...

Late physical effects of childhood cancer survivors
Young-Ho Lee
Clin Exp Pediatr. 2010;53(4):477-480.   Published online April 15, 2010
Advances in research and medical and supportive care have contributed to a growing population of adults formerly treated for childhood cancer. History of cancer and its therapy can have significant life-long health implications. Late effects of cancer therapy can be insidious on onset, occur outside the pediatric age, and contribute to premature morbidity and mortality. In this review, I have focused on the...
Psychosocial aspects of childhood cancer survivors
Jong Jin Seo
Clin Exp Pediatr. 2010;53(4):471-476.   Published online April 15, 2010
The majority of childhood cancer survivors and their families will be psychologically healthy, but may desire and benefit from preventive care. A significant portion of the survivor population will be psychosocially distressed in various aspects by their harsh experience of long cancer treatment, and may warrant professional intervention and treatment. Pediatricians should be aware of the late psychological effects that can occur a...
Long-term follow-up study and long-term care of childhood cancer survivors
Hyeon Jin Park
Clin Exp Pediatr. 2010;53(4):465-470.   Published online April 15, 2010
The number of long-term survivors is increasing in the western countries due to remarkable improvements in the treatment of childhood cancer. The long-term complications of childhood cancer survivors in these countries were brought to light by the childhood cancer survivor studies. In Korea, the 5-year survival rate of childhood cancer patients is approaching 70%; therefore, it is extremely important to undertake...
Clinical application of gonadotropin-releasing hormone analogs in children and adolescents
Ho-Seong Kim
Clin Exp Pediatr. 2010;53(3):294-299.   Published online March 15, 2010
Although the increasing incidence of central precocious puberty (CPP) in Korea has recently raised public concerns about health and growth problems, there are many areas of uncertainty regarding the pathogenesis, diagnosis, and management of CPP. In this paper, we review the definition of precocity, the assessment of CPP, and the hormonal abnormalities that support the diagnosis. In addition, we review...
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