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Case Report
Plasmaphresis therapy for pulmonary hemorrhage in a pediatric patient with IgA nephropathy
Dae-Kyoon Yim, Sang-Taek Lee, Heeyeon Cho
Clin Exp Pediatr. 2015;58(10):402-405.   Published online October 21, 2015

IgA nephropathy usually presents as asymptomatic microscopic hematuria or proteinuria or episodic gross hematuria after upper respiratory infection. It is an uncommon cause of end-stage renal failure in childhood. Pulmonary hemorrhage associated with IgA nephropathy is an unusual life-threatening manifestation in pediatric patients and is usually treated with aggressive immunosuppression. Pulmonary hemorrhage and renal failure usually occur concurrently, and the...

The first pediatric case of tularemia in Korea: manifested with pneumonia and possible infective endocarditis
Jung Sook Yeom, Kyuyol Rhie, Ji Sook Park, Ji-Hyun Seo, Eun Sil Park, Jae-Young Lim, Chan-Hoo Park, Hyang-Ok Woo, Hee-Shang Youn
Clin Exp Pediatr. 2015;58(10):398-401.   Published online October 21, 2015

Tularemia is a potentially severe zoonotic disease caused by Francisella tularensis. A lack of awareness about tularemia can be embarrassing and could result in delayed treatment because of improper diagnosis. The diagnosis of tularemia is difficult, because the infections are rare and the clinical spectrum is broad. As only 1 adult case has been reported in Korea thus far, pediatricians...

Original Article
Alagille syndrome and a JAG1 mutation: 41 cases of experience at a single center
Kyung Jin Ahn, Ja Kyoung Yoon, Gi Beom Kim, Bo Sang Kwon, Jung Min Go, Jin Su Moon, Eun Jung Bae, Chung Il Noh
Clin Exp Pediatr. 2015;58(10):392-397.   Published online October 21, 2015
Purpose

Alagille syndrome is a complex hereditary disorder that is associated with cardiac, hepatic, skeletal, ocular, and facial abnormalities. Mutations in the Notch signaling pathway, such as in JAG1 and NOTCH2, play a key role in embryonic development. A cardiac or hepatic presentation is a critical factor for determining the prognosis.

Methods

We conducted a retrospective study of 41 patients with Alagille syndrome...

Prognostic factors in children with extracranial germ cell tumors treated with cisplatin-based chemotherapy
Jinsup Kim, Na Hee Lee, Soo Hyun Lee, Keon Hee Yoo, Ki Woong Sung, Hong Hoe Koo, Jeong-Meen Seo, Suk-Koo Lee
Clin Exp Pediatr. 2015;58(10):386-391.   Published online October 21, 2015
Purpose

To evaluate the outcomes and prognostic factors in children with extracranial germ cell tumors (GCTs) treated at a single institution.

Methods

Sixty-six children diagnosed with extracranial GCTs between 1996 and 2012 were included in the study. Primary treatment was surgical excision, followed by six cycles of cisplatin-based chemotherapy. The survival rates were compared according to the International Germ Cell Cancer Cooperative Group...

Clinical characteristics of children and adolescents with croup and epiglottitis who visited 146 Emergency Departments in Korea
Doo Ri Lee, Chang Hyu Lee, Youn Kyung Won, Dong In Suh, Eui-Jung Roh, Mi-Hee Lee, Eun Hee Chung
Clin Exp Pediatr. 2015;58(10):380-385.   Published online October 21, 2015
Purpose

Croup is a common pediatric respiratory illness with symptoms of varying severity. Moreover, epiglottitis is a rare disease that can rapidly progress to life-threatening airway obstruction. Although the clinical course and treatments differ between croup and epiglottitis, they are difficult to differentiate on presentation. We aimed to compare the clinical characteristics of croup and epiglottitis in Emergency Department patients.

Methods

The 2012...

Uveitis as an important ocular sign to help early diagnosis in Kawasaki disease
Han Seul Choi, Seul Bee Lee, Jung Hyun Kwon, Hae Soon Kim, Sejung Sohn, Young Mi Hong
Clin Exp Pediatr. 2015;58(10):374-379.   Published online October 21, 2015
Purpose

Incomplete Kawasaki disease (KD) is frequently associated with delayed diagnosis and treatment. Delayed diagnosis leads to increasing risk of coronary artery aneurysm. Anterior uveitis is an important ocular sign of KD. The purpose of this study was to assess differences in laboratory findings, including echocardiographic measurements, clinical characteristics such as fever duration and treatment responses between KD patients with and...

Diagnostic characteristics of supplemental laboratory criteria for incomplete Kawasaki disease in children with complete Kawasaki disease
Hyun Ok Jun, Jeong Jin Yu, So Yeon Kang, Chang Deok Seo, Jae Suk Baek, Young-Hwue Kim, Jae-Kon Ko
Clin Exp Pediatr. 2015;58(10):369-373.   Published online October 21, 2015
Purpose

In 2004, the American Heart Association (AHA) had published an algorithm for the diagnosis of incomplete Kawasaki disease (KD). The aim of the present study was to investigate characteristics of supplemental laboratory criteria in this algorithm.

Methods

We retrospectively examined the medical records of 355 patients with KD who were treated with intravenous immunoglobulin (IVIG) during the acute phase of the disease....

Review Article
Nutritional aspect of pediatric inflammatory bowel disease: its clinical importance
Seung Kim, Hong Koh
Clin Exp Pediatr. 2015;58(10):363-368.   Published online October 21, 2015

Inflammatory bowel disease (IBD) is a chronic inflammatory disease mainly affecting the gastrointestinal tract. The incidence of the disease is rapidly increasing worldwide, and a number of patients are diagnosed during their childhood or adolescence. Aside from controlling the gastrointestinal symptoms, nutritional aspects such as growth, bone mineral density, anemia, micronutrient deficiency, hair loss, and diet should also be closely...

Case Report
Hemophagocytic lymphohistiocytosis diagnosed by brain biopsy
Hee Young Ju, Che Ry Hong, Sung Jin Kim, Ji Won Lee, Hyery Kim, Hyoung Jin Kang, Kyung Duk Park, Hee Young Shin, Jong-Hee Chae, Ji Hoon Phi, Jung-Eun Cheon, Sung-Hye Park, Hyo Seop Ahn
Clin Exp Pediatr. 2015;58(9):358-361.   Published online September 21, 2015

Hemophagocytic lymphohistiocytosis (HLH) is characterized by fever, splenomegaly, jaundice, and pathologic findings of hemophagocytosis in bone marrow or other tissues such as the lymph nodes and liver. Pleocytosis, or the presence of elevated protein levels in cerebrospinal fluid, could be helpful in diagnosing HLH. However, the pathologic diagnosis of the brain is not included in the diagnostic criteria for this...

Parry-Romberg syndrome with ipsilateral hemipons involvement presenting as monoplegic ataxia
Yun-Jin Lee, Kee-Yang Chung, Hoon-Chul Kang, Heung Dong Kim, Joon Soo Lee
Clin Exp Pediatr. 2015;58(9):354-357.   Published online September 21, 2015

Parry-Romberg syndrome (PRS) is a rare, acquired disorder characterized by progressive unilateral facial atrophy of the skin, soft tissue, muscles, and underlying bony structures that may be preceded by cutaneous induration. It is sometimes accompanied by ipsilateral brain lesions and neurological symptoms. Here we present the case of a 10-year-old girl with right-sided PRS and recurrent monoplegic ataxia of the...

Original Article
The efficacy and safety of Montelukast sodium in the prevention of bronchopulmonary dysplasia
Sang Bum Kim, Jang Hoon Lee, Juyoung Lee, Seung Han Shin, Ho Sun Eun, Soon Min Lee, Jin A Sohn, Han Suk Kim, Byung Min Choi, Min Soo Park, Kook In Park, Ran Namgung, Moon Sung Park
Clin Exp Pediatr. 2015;58(9):347-353.   Published online September 21, 2015
Purpose

The purpose of this study was to evaluate the efficacy and safety of Montelukast sodium in the prevention of bronchopulmonarydysplasia (BPD).

Methods

The Interventional study was designed as a multicenter, prospective, and randomized trial, with open labeled and parallel-experimental groups, 66 infants were enrolled and allocated to either the case group (n=30) or the control group (n=36) based on gestational age (GA)....

Evaluation of new American Academy of Pediatrics guideline for febrile urinary tract infection
Da Min Choi, Tae Hoon Heo, Hyung Eun Yim, Kee Hwan Yoo
Clin Exp Pediatr. 2015;58(9):341-346.   Published online September 21, 2015
Purpose

To evaluate the practical applications of the diagnosis algorithms recommended by the American Academy of Pediatrics urinary tract infection (UTI) guideline.

Methods

We retrospectively reviewed the medical records of febrile UTI patients aged between 2 and 24 months. The patients were divided into 3 groups: group I (patients with positive urine culture and urinalysis findings), group II (those with positive urine culture...

Change in the height of Korean children and adolescents: analysis from the Korea National Health and Nutrition Survey II and V
Na Yung Ryoo, Ha Young Shin, Jae Hyun Kim, Jin Soo Moon, Chong Guk Lee
Clin Exp Pediatr. 2015;58(9):336-340.   Published online September 21, 2015
Purpose

The mean adult height of Koreans has increased since nationwide anthropological measurements began in 1967. The objective of this study was to evaluate differences in heights of Korean late adolescents and young adults within and between the Second and Fifth Korea National Health and Nutrition Examination Surveys (KNHANES II and V).

Methods

Koreans aged ≤22 years with available measurements of height were...

Food allergen sensitization in young children with typical signs and symptoms of immediate-type food allergies: a comparison between monosensitized and polysensitized children
Na Yeon Kim, Ga Ram Kim, Joon Hwan Kim, Ji Hyeon Baek, Jung Won Yoon, Hye Mi Jee, Hye Sung Baek, Yong Ho Jung, Sun Hee Choi, Ki Eun Kim, Youn Ho Shin, Hye Yung Yum, Man Yong Han, Kyu-Earn Kim
Clin Exp Pediatr. 2015;58(9):330-335.   Published online September 21, 2015
Purpose

The clinical interpretation of children sensitized to allergens is challenging, particularly in children with food allergies. We aimed to examine clinical differences between children with monosensitization and those with polysensitization to common food allergens and to determine risk factors for polysensitization in young children <10 years of age with immediate-type food allergies.

Methods

The study included children <10 years of age with...

Vitamin D serum levels in children with allergic and vasomotor rhinitis
Seung Jin Lee, Bong Hwa Kang, Bong Seok Choi
Clin Exp Pediatr. 2015;58(9):325-329.   Published online September 21, 2015
Purpose

In addition to regulating calcium and phosphorus homeostasis and bone metabolism, vitamin D is known as an immune modulator. Recently, there has been increased worldwide interest in the association between low levels of vitamin D and allergic diseases. The purpose of this study was to assess the relationship between serum vitamin D levels and allergic/vasomotor rhinitis (AR/VR) in children.

Methods

This study...

Review Article
Kabuki syndrome: clinical and molecular characteristics
Chong-Kun Cheon, Jung Min Ko
Clin Exp Pediatr. 2015;58(9):317-324.   Published online September 21, 2015

Kabuki syndrome (KS) is a rare syndrome characterized by multiple congenital anomalies and mental retardation. Other characteristics include a peculiar facial gestalt, short stature, skeletal and visceral abnormalities, cardiac anomalies, and immunological defects. Whole exome sequencing has uncovered the genetic basis of KS. Prior to 2013, there was no molecular genetic information about KS in Korean patients. More recently, direct...

Case Report
De novo interstitial deletion of 15q22q23 with global developmental delay and hypotonia: the first Korean case
Ha-Su Kim, Jin-Yeong Han, Myo-Jing Kim
Clin Exp Pediatr. 2015;58(8):313-316.   Published online August 21, 2015

Interstitial deletions involving the chromosome band 15q22q24 are very rare and only nine cases have been previously reported. Here, we report on a 12-day-old patient with a de novo 15q22q23 interstitial deletion. He was born by elective cesarean section with a birth weight of 3,120 g at 41.3-week gestation. He presented with hypotonia, sensory and neural hearing loss, dysmorphism with...

Successful sulfonylurea treatment in a patient with permanent neonatal diabetes mellitus with a novel KCNJ11 mutation
Sung Yeon Ahn, Gu-Hwan Kim, Han-Wook Yoo
Clin Exp Pediatr. 2015;58(8):309-312.   Published online August 21, 2015

Permanent neonatal diabetes mellitus refers to diabetes that occurs before the age of 6 months and persists through life. It is a rare disorder affecting one in 0.2-0.5 million live births. Mutations in the gene KCNJ11, encoding the subunit Kir6.2, and ABCC8, encoding SUR1 of the ATP-sensitive potassium (KATP) channel, are the most common causes of permanent neonatal diabetes mellitus....

Original Article
Evaluation of three glucometers for whole blood glucose measurements at the point of care in preterm or low-birth-weight infants
Joon Ho Hwang, Yong-Hak Sohn, Seong-Sil Chang, Seung Yeon Kim
Clin Exp Pediatr. 2015;58(8):301-308.   Published online August 21, 2015
Purpose

We evaluated three blood glucose self-monitoring for measuring whole blood glucose levels in preterm and low-birth-weight infants.

Methods

Between December 1, 2012 and March 31, 2013, 230 blood samples were collected from 50 newborns, who weighed, ≤2,300 g or were ≤36 weeks old, in the the neonatal intensive care unit of Eulji University Hospital. Three blood glucose self-monitoring (A: Precision Pcx, Abbott;...

Usefulness of pelvic ultrasonography for the diagnosis of central precocious puberty in girls
Jung Yu, Ha Young Shin, Sun Hee Lee, You Sung Kim, Jae Hyun Kim
Clin Exp Pediatr. 2015;58(8):294-300.   Published online August 21, 2015
Purpose

It is difficult to differentiate between central precocious puberty (CPP) and premature thelarche (PT) in girls. The aim of this study was to investigate the diagnostic usefulness of pelvic ultrasonography to distinguish between CPP and PT in girls with early breast development.

Methods

This study included girls with early breast development who visited the clinic between January 2012 and December 2013. Clinical,...

Comparison of gastric and other bowel perforations in preterm infants: a review of 20 years' experience in a single institution
Do Kyung Lee, So Yeon Shim, Su Jin Cho, Eun Ae Park, Sun Wha Lee
Clin Exp Pediatr. 2015;58(8):288-293.   Published online August 21, 2015
Purpose

In this study, we aimed to review the clinical presentation of preterm infants with gastrointestinal perforations and compare the clinical features of gastric perforation with other intestinal perforations.

Methods

The medical records of preterm neonates with pneumoperitoneum, admitted to the neonatal intensive care unit (NICU) between January 1994 and December 2013, were retrospectively reviewed.

Results

Twenty-one preterm infants underwent exploratory laparotomy to investigate the...

Iron and vitamin D status in breastfed infants and their mothers
Yu Sun Kang, Joon Hwan Kim, Eun Hee Ahn, Eun-Gyong Yoo, Moon Kyu Kim
Clin Exp Pediatr. 2015;58(8):283-287.   Published online August 21, 2015
Purpose

We assessed the relationships between iron and vitamin D statuses in breastfed infants and their mothers and evaluated the determinants of iron and vitamin D deficiencies in breastfed infants.

Methods

Seventy breastfed infants aged 4-24 months and their mothers participated in this study from February 2012 to May 2013. Complete blood counts, total iron binding capacity, and levels of C-reactive protein, iron,...

Review Article
Nephrotic syndrome: what's new, what's hot?
Hee Gyung Kang, Hae Il Cheong
Clin Exp Pediatr. 2015;58(8):275-282.   Published online August 21, 2015

While the incidence of nephrotic syndrome (NS) is decreasing in Korea, the morbidity of difficult-to-treat NS is significant. Efforts to minimize treatment toxicity showed that prolonged treatment after an initial treatment for 2-3 months with glucocorticosteroids was not effective in reducing frequent relapses. For steroid-dependent NS, rituximab, a monoclonal antibody against the CD20 antigen on B cells, was proven to...

Case Report
Hypopituitarism and Legg-Calve-Perthes disease related to difficult delivery
Veysel Nijat Baş, Salih Uytun, Ümit Erkan Vurdem, Yasemin Altuner Torun
Clin Exp Pediatr. 2015;58(7):270-273.   Published online July 22, 2015

Legg-Calve-Perthes (LCP) disease is characterized by idiopathic avascular osteonecrosis of the epiphysis of the femur head. The main factor that plays a role in the etiology of the disease is decreased blood flow to the epiphysis. Many predisposing factors have been suggested in the etiology of LCP disease, and most have varying degrees of effects. Here we present the case...

Graves disease following rabbit antithymocyte globulin treatment of severe aplastic anemia in a Korean child
In Su Choi, Han Kyul Kim, Dong Kyun Han, Hee Jo Baek, Hae In Jang, Chan Jong Kim, Hoon Kook
Clin Exp Pediatr. 2015;58(7):267-269.   Published online July 22, 2015

Antithymocyte globulin (ATG) is used as an immunosuppressive treatment (IST) to deplete clonal suppressor T cells in patients with severe aplastic anemia (SAA). The depletion of suppressor T cells by ATG may affect the activation of B cells, which results in an increased risk for autoimmune conditions. A 12-year-old boy was diagnosed with idiopathic SAA. As he did not have...

Original Article
The corrected QT (QTc) prolongation in hyperthyroidism and the association of thyroid hormone with the QTc interval
Ye Seung Lee, Joong Wan Choi, Eun Ju Bae, Won Il Park, Hong Jin Lee, Phil Soo Oh
Clin Exp Pediatr. 2015;58(7):263-266.   Published online July 22, 2015
Purpose

Ventricular repolarization is assessed using the QT interval corrected by the heart rate (QTc) via an electrocardiogram (ECG). Prolonged QTc is associated with an increased risk of arrhythmias and cardiac mortality. As there have been few reports regarding the effects of hyperthyroidism on ventricular repolarization, we studied the association between serum free thyroxine (free T4 [fT4]) and thyroid stimulating hormone...

The strong association of left-side heart anomalies with Kabuki syndrome
Ja Kyoung Yoon, Kyung Jin Ahn, Bo Sang Kwon, Gi Beom Kim, Eun Jung Bae, Chung Il Noh, Jung Min Ko
Clin Exp Pediatr. 2015;58(7):256-262.   Published online July 22, 2015
Purpose

Kabuki syndrome is a multiple congenital malformation syndrome, with characteristic facial features, mental retardation, and skeletal and congenital heart anomalies. However, the cardiac anomalies are not well described in the Korean population. We analyzed the cardiac anomalies and clinical features of Kabuki syndrome in a single tertiary center.

Methods

A retrospective analysis was conducted for a total of 13 patients with Kabuki...

Applying the bacterial meningitis score in children with cerebrospinal fluid pleocytosis: a single center's experience
Jungpyo Lee, Hyeeun Kwon, Joon Soo Lee, Heung Dong Kim, Hoon-Chul Kang
Clin Exp Pediatr. 2015;58(7):251-255.   Published online July 22, 2015
Purpose

The widespread introduction of bacterial conjugate vaccines has decreased the risk of cerebrospinal fluid (CSF) pleocytosis due to bacterial meningitis (BM) in children. However, most patients with CSF pleocytosis are hospitalized and treated with parenteral antibiotics for several days. The bacterial meningitis score (BMS) is a validated multivariate model derived from a pediatric population in the postconjugate vaccine era and...

Clinical risk factors associated with the development of wheezing in children less than 2 years of age who required hospitalization for viral lower respiratory tract infections
Joon Hwan Kim, Ji-Yeon Choi, Na Yeon Kim, Jin Woo Kim, Ji Hyeon Baek, Hye Sung Baek, Jung Won Yoon, Hye Mi Jee, Sun Hee Choi, Hyeung Yoon Kim, Ki Eun Kim, Youn Ho Shin, Man Yong Han
Clin Exp Pediatr. 2015;58(7):245-250.   Published online July 22, 2015
Purpose

Wheezing following viral lower respiratory tract infections (LRTIs) in children <2 years of age is an important risk factor for the development of asthma later in life; however, not all children with viral LRTIs develop wheezing. This study investigated risk factors for the development of wheezing during viral LRTIs requiring hospitalization.

Methods

The study included 142 children <2 years of age hospitalized...

Review Article
Complement regulation: physiology and disease relevance
Heeyeon Cho
Clin Exp Pediatr. 2015;58(7):239-244.   Published online July 22, 2015

The complement system is part of the innate immune response and as such defends against invading pathogens, removes immune complexes and damaged self-cells, aids organ regeneration, confers neuroprotection, and engages with the adaptive immune response via T and B cells. Complement activation can either benefit or harm the host organism; thus, the complement system must maintain a balance between activation...


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