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Original Article
Pulmonology
Telomere biology disorders associated with childhood interstitial lung disease
Maria Greiner-Mai, Christina Katharina Rapp, Katrin Knoflach, Daniel Gräfe, Franz Wolfgang Hirsch, Julia Ley-Zaporozhan, Simone Reu-Hofer, Julia Hentschel, Stefan Zielen, Monika Helena Tedy, Heymut Omran, Ernst Rietschel, Tuğba Sismanlar Eyuboglu, Ayse Tana-Aslan, Nagehan Emiralioglu, Nicola Ullmann, Samuele Naviglio, Massimo Maschio, Fabian Beier, Tuğba Ramaslı Gürsoy, Matthias Griese, Freerk Prenzel
Clin Exp Pediatr. 2026;69(7):593-603.   Published online June 18, 2026
Question: What are the pulmonary manifestations and clinical characteristics of telomere biology disorders in children with interstitial lung disease?
Finding: Telomere biology disorders are an important but often underrecognized cause of childhood interstitial lung disease and are associated with variable pulmonary, radiologic, and histopathologic findings, frequent multisystem involvement, and often severe disease progression.
Meaning: Early genetic testing and multidisciplinary evaluation are essential for timely diagnosis, optimized management, and improved outcomes in affected children.
Nephrology (Genitourinary)
Assessing kidney outcomes in childhood-onset lupus nephritis: role of National Institutes of Health-modified histological indices
Nuanpan Penboon, Pornpimol Rianthavorn
Clin Exp Pediatr. 2026;69(2):130-139.   Published online October 23, 2025
Question: In children with proliferative lupus nephritis, do National Institutes of Health-modified indices and treatment choices predict long-term kidney function?
Finding: Higher chronicity index scores, especially tubular atrophy and interstitial fibrosis, predicted kidney impairment. Additionally, the use of mycophenolate mofetil (MMF) for maintenance therapy was associated with a lower risk of kidney function decline.
Meaning: The early recognition of chronic lesions and MMF-based maintenance therapy may improve kidney outcomes in childhood-onset lupus nephritis.
Review Article
Pulmonology
The past, present, and future of humidifier disinfectant-associated interstitial lung diseases in children
Eun Lee, So-Yeon Lee, Soo-Jong Hong
Clin Exp Pediatr. 2020;63(7):251-258.   Published online December 9, 2019
Exposure to environmental factors can cause interstitial lung diseases (ILDs); however, such types of ILDs are rare. From 2007 to 2011, an ILD epidemic occurred in South Korea owing to inhalational exposure to toxic chemicals in humidifier disinfectants (HDs). HD-associated ILDs (HD-ILDs) are characterized by rapidly progressing respiratory failure with pulmonary fibrosis and a high mortality rate of 43.8%−58.0%. Although...
Case Report
Nephrology (Genitourinary)
Successful treatment of tubulointerstitial nephritis and uveitis with steroid and azathioprine in a 12-year-old boy
Ji Eun Kim, Se Jin Park, Ji Young Oh, Hyeon Joo Jeong, Ji Hong Kim, Jae Il Shin
Clin Exp Pediatr. 2016;59(Suppl 1):S99-S102.   Published online November 30, 2016

Tubulointerstitial nephritis and uveitis (TINU) syndrome is a rare disease, often underdiagnosed or misdiagnosed in children. We describe the case of a 12-year-old boy who presented to Severance Hospital with a 1-month history of bilateral conjunctival injection. He was first evaluated by an Ophthalmologist in another hospital and diagnosed with panuveitis. Laboratory tests indicated renal failure, and a renal biopsy...

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...

Chronic Epstein-Barr virus infection causing both benign and malignant lymphoproliferative disorders
Yoojin Kwun, Soo-Jong Hong, Jin Seong Lee, Da Hye Son, Jong Jin Seo
Clin Exp Pediatr. 2014;57(9):420-424.   Published online September 30, 2014

The Epstein-Barr virus (EBV) is oncogenic and can transform B cells from a benign to a malignant phenotype. EBV infection is also associated with lymphoid interstitial pneumonia (LIP). Here, we report the case of a 14-year-old boy who was diagnosed with a latent EBV infection and underlying LIP, without any associated immunodeficiency. He had been EBV-seropositive for 8 years. The...

Original Article
Nationwide surveillance of acute interstitial pneumonia in Korea
Byoung-Ju Kim, Han A Kim, Young-Hwa Song, Jinho Yu, Seonguk Kim, Seong Jong Park, Kyung Won Kim, Kyu-Earn Kim, Dong Soo Kim, June Dong Park, Kang Mo Ahn, Hyo-Bin Kim, Hyang-Min Jung, Chun Kang, Soo-Jong Hong
Clin Exp Pediatr. 2009;52(3):324-329.   Published online March 15, 2009
Purpose : Acute interstitial pneumonia (AIP) is a rare disease, but its prognosis is fatal because of lack of efficient treatment modality. Recently, it has been reported that there was epidemic AIP in Korea. This study aims to investigate the past and current status of AIP in Korea. Methods : We performed a nationwide survey and a prospective study. From August...
Study of the risk factors for pulmonary interstitial emphysema related to mechanical ventilator care
Sang Yeob Kim, Pil Sang Lee, Sang Geel Lee
Clin Exp Pediatr. 2008;51(11):1179-1184.   Published online November 15, 2008
Purpose : Pulmonary interstitial emphysema (PIE) primarily occurs in preterm infants suffering from respiratory distress syndrome (RDS) and kept under mechanical ventilator care. Therefore, this study aimed to examine various risk factors for PIE, to identify conditions that can decrease the possibility of PIE development. Methods : PIE classification was conducted for 183 patients diagnosed to have RDS and receiving mechanical...
Epidemic acute interstitial pneumonia in children occurred during the early 2006s
Chong Kun Cheon, Hyun-Seung Jin, Eun Kyeong Kang, Hyo Bin Kim, Byoung-Joo Kim, Jinho Yu, Seong Jong Park, Soo-Jong Hong, June Dong Park
Clin Exp Pediatr. 2008;51(4):383-390.   Published online April 15, 2008
Purpose : This study was aimed to analyze the clinical characteristics of patients with acute interstitial pneumonia who had presented similar clinical patterns from March to June, 2006 and to describe our experience of treatment and to identify risk factors associated with prognosis. Methods : The clinical characteristics, radiologic and histopathologic findings and response to steroids of 15 patients (non-survival...
Case Report
A Case of Idiopathic Interstitial Pneumonia in Childhood
Su-Jin Lee, Eon-Woo Shin, Eun-Young Park, Phil-Soo Oh, Kon-Hee Lee, Kwang-Nam Kim, Ho-Seung Shin, Il-Seung Lee
Clin Exp Pediatr. 2005;48(3):327-332.   Published online March 15, 2005
Interstitial lung disease refers to a group of pulmonary disorders characterized by inflammation of the interstitium, derangements and loss of alveolar capillary units leading to disruption of alveolar gas exchange, which induces symptoms of restrictive lung disease. Cases of interstitial pneumonia in children are uncommon and mostly have unknown causes. We have experienced an 8-year old boy who had symptoms...
A Case of Nonspecific Interstitial Pneumonia in a Child
Tae Wan Kim, Dae-Hyun Lym, Jung Hee Kim, Byong Kwan Son, Hye-Seung Han, Young Kyu Shin
Clin Exp Pediatr. 2002;45(4):529-534.   Published online April 15, 2002
Department of Pediatrics†, College of Medicine, Korea University, Seoul, Korea Interstitial pneumonia is a heterogenous group of inflammatory and fibrosing lesions that manifest themselves as infiltrative lung disease. Of these, nonspecific interstitial pneumonia is characterized as a variable degree of interstitial inflammation with or without fibrosis and is distinguished from usual interstitial pneumonia and desquamative interstitial pneumonia, histologically. The influx of...
A Case of 4q Deletion with Partial Agenesis of Corpus Callosum
Mi Na Kang, In Suk Lim, Byeong Eui Kim, Myoung Jae Chey, Sang Woo Kim
Clin Exp Pediatr. 2002;45(2):273-277.   Published online February 15, 2002
Syndrome of 4q deletion is characterized by an abnormal shape of the skull, craniofacial dysmorphism, cardiovascular malformations, genitourinary defects, limb and digital anomalies, and developmental delay. We experienced a case of 4q interstitial deletion in a 2 day-old female neonate who showed short extremities, partial agenesis of corpus callosum and congenital heart defects. We report the case with a brief...
Original Article
Clinical Spectrum and Lung Pathology in Children with Interstitial Lung Disease
Ji-Hyun Chung, Seung-Ju Ha, Bong-Seong Kim, Soo-Jong Hong
Clin Exp Pediatr. 2002;45(1):79-87.   Published online January 15, 2002
Purpose : Interstitial lung disease(ILD) is a rare and poorly characterized disorder in children with poor prognosis. To understand the ILD in children, we reviewed our experience with 21 patients who were diagnosed interstitial lung disease during 9-year period at Asan Medical Center retrospectively. Methods : Severity-of-illness score was measured by the Denver protocol. We evaluated underlying diseases, clinical manifestations, high resolution computed tomography findings,...
Case Report
A Case of Chronic Pneumonitis of Infancy in Pediatric Interstitial Lung Disease
Hyun Oh Jang, Se Chang Ham, Young Kyoun Kim, Se Wook Oh, Yong Won Park
Clin Exp Pediatr. 2001;44(9):1085-1090.   Published online September 15, 2001
The interstitial lung diseases(ILD) comprises a large, heterogeneous group of disorders characterized by derangement of alveolar walls and alveolar capillary unit causing restrictive pulmonary dysfunction and disordered gas exchange. It is rare, especially in children, and the opinions on investigation and treatments are controversial. The classification of adult ILD is applied to that of children. But considering occurrence during the developing state of lung...
A Case of de novo Interstitial Deletion of 17 Chromosome
Kyung Hee Yoon, Hee Cheol Lee, Ai Rhan Kim, Ki Soo Kim, Soo Young Pi, Eul Ju Seo, Han Wook Yoo
Clin Exp Pediatr. 2001;44(4):475-479.   Published online April 15, 2001
This is the first reported case of a unique interstitial deletion involving the long arm of chromosome 17 in a Korean male infant born to parents with normal karyotype. The patient presented with multiple congenital malformations suggestive of chromosomal anomaly including round face, upslanted palpebral fissure, hypertelorism, posteriorly rotated low set ear, micrognathia, microcephaly, finger- like thumb, bilateral hearing loss,...
A Case of Lymphocytic Interstitial Pneumonia
Ho Young Lee, Hwang Min Kim, Mee Kyung Namgoong, Jong Soo Kim, Soon Hee Jung, Dong Jin Kim
Clin Exp Pediatr. 1997;40(4):572-577.   Published online April 15, 1997
Lymphoid interstitial pneumonia(LIP) is an uncommon process in childhood, and is characterized by interstitial accumulation of mature lymphocytes, plasma cells and reticuloendothelial cells. LIP is believed to have autoimmune or immunologic pathogenesis, because it is frequently associated with acquired immune deficiency syndrome, Epstein-Barr virus infection, common variable immunodeficiency. The patient described in this report had idiopathic thrombocytopenic purpura, Evans syndrome...
Original Article
Risk factors for Pulmonary Interstitial Emphysema(PIE) in Mechanically Ventilated Neonates with Hyaline Membrane Diseas
Shin Won Yoon, Chul Lee, Jeong Nyun Kim, Ran Namgung, Dong Gwan Han, Myung Joon Kim
Clin Exp Pediatr. 1997;40(3):318-326.   Published online March 15, 1997
Purpose : Pulmonary interstitial emphysema(PIE) is a common and serious complication of mechanical ventilation in infants with hyaline membrane disease. This abnormal collection of gases has two basic roentgenographic features; linear and cyst-like radiolucencies. High positive inspiratory pressure was found to be the most significant parameter associated with development of fatal pulmonary interstitial emphysema. Without prompt conservative management such as lowering peak inspiratory...
A Case of Acute Interstitial Nephritis Associated with Yersinia Pseudotuberculosis Infection.
Keun Hee Chung, Yoo Mee Kim, Mee Won Kim, Soon Gi Kim, Moon Soo Park, Jin Keun Chang
Clin Exp Pediatr. 1990;33(8):1123-1128.   Published online August 31, 1990
Authors recently experienced a case of acute interstitial nephritis associated with Yersinia pseudotuberculosis infection in 10-8/12 year old boy, who was admitted to prolonged high fever. On 3rd hospital day, puffy face and oliguria were noted. BUN and serum creatinine rose to peak level, 94.1 mg/dl and 10.1 mg/dl, respectively on 7th hospital day. After then renal functions were normal- ized gradually without specific...
Factors Associated with Pneumothorax in Hyaline Membrane Disease In fants Receiving Mechanical Ventilation.
Oh Kyung Lee, Kyung Hee Kim, Kyu Sun Choi, Wan Seoub Kim
Clin Exp Pediatr. 1989;32(12):1642-1649.   Published online December 31, 1989
We observed 42 cases of neonates, who were diagnosed as having Hyaline membrane disease and had been received mechanical ventilation in neonatal intensive care unit of Presbyterian medical center from July 1984 to July 1988. In order to observe the factors associated with pneumothorax, we devide these cases in two groups. First group was consists of infants with pneumothorax, second group was consists of...
A Case of Child Desquamative Interstitial Pneumonia.
Woo Taek Kim, Hyoung Doo Lee, Yung Tak Lim, Hee Joo Jeon, Hee Joo Park, Chan Yung Kim
Clin Exp Pediatr. 1989;32(1):92-100.   Published online January 31, 1989
Desquamative interstitial pneumonia (DIP) is a variant of idiopathic interstitial pneumonias, but characteristicalIy has massive desquamation and proliferation of alveolar cells into the distal air space with minimal fibrosis of the alveolar wall. DIP seems to have better response to corticosteroid and benign prognosis than idiopathic interstitial pneumonia. We experienced one case of 6-year old girl who showed typical picture of DIF on lung...
Clinical Studies on Interstitial Pneumonitis in Children.
Young Yull Ko, Kwang Wook Ko, Jw Geun Chi
Clin Exp Pediatr. 1985;28(7):663-673.   Published online July 31, 1985
Interstitial pneumonitis is a rare, diffuse lung disease characterized by varing combinations of two histological features: thickening of alveolar walls and the presence of large mononuclear cells in the alveolar spaces. 4 cases with interstitial pneumonitis, admitted to the Pediatric Department of Seoul National University Hospital during the period from Jan. 1979 through. May, 1985 were reviewed clinically. The results...
Peumomediastinum in the Newborn.
Dong Gwan Han
Clin Exp Pediatr. 1985;28(3):217-224.   Published online March 31, 1985
A retrospective review was performed of 15 medical records to study the pneumomedia- stinum in the newborn during last 4 years from Jan. 1980 through Dec. 1983 at Severance Hospital of Yonsei University. Tne results were as follows: 1) The incidence was 0.14% (15 of 8,737 live-births) during the neonatal period. Of those cases spontaneous pneumomediastinum was 66.7%(10 cases) and secondary pneumomedia- stinum 33.3%(5 cases). Males were...
Secondary Pneumothorax in the newborn.
Dong Gwan Han
Clin Exp Pediatr. 1985;28(2):123-130.   Published online February 28, 1985
Clinical records of 25 neonates with secondary pneumothorax were reviewed retrospectively during last 6 years from Jan. 1978 through Dec. 1983 at Pediatric Department of Yonsei University Hospital. The following results were obtained; 1)The incidence of secondary pneumothorax was 0.16% (21 out of 12,864 live-birth) during the neonatal period. The incidence tended to increase since we had set up the neonatal,...


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