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A Case of Neonatal Lupus Erythematosus Associated with Anti-U1RNP Antibodies.

Byung Hoon AHN ; Gu Chang LEE ; Tae Young YOON ; Mi Jung KIM

Korean Journal of Pediatrics.2005;48(3):342-345.

Neonatal lupus erythematosus(NLE) is a distinct subset of lupus characterized by cutaneous findings, cardiac conduction defects, hepatic or hematologic abnormalities. These manifestations are associated with the presence of maternal auto-antibodies such as anti-SSA/Ro, anti-SSB/La, and rarely anti- RNP(U1RNP) antibodies. Cases of U1RNP antibody-positive NLE have somewhat atypical cutaneous manifestation without cardiac or systemic abnormalities. We report a case of cutaneous NLE associated with U1RNP antibodies.
Antibodies*

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Hyperthyroidism Caused by a Mutation in the Thyrotropin Receptor Gene in Two Brothers.

Jae Hyun KIM ; Sung Soo LEE ; Jung Sub LIM ; Choong Ho SHIN ; Sei Won YANG

Korean Journal of Pediatrics.2005;48(3):337-341.

Thyrotropin receptor(TSHR) mutations must be considered when congenital hyperthyroidism has persisted, but there has been no evidence for autoimmunity. TSHR mutations leading to constitutive activation of the thyroid gland were identified as the molecular cause of autosomal dominant nonautoimmune hyperthyroidism and sporadic congenital hyperthyroidism. We report two cases of hyperthyroidism caused by germline TSHR mutation who presented with exessive sweating and no evidence of autoimmune thyroid disease. They were brothers and their mother had undergone thyroidectomy because of hyperthyroidism. Direct sequencing of the polymerase chain reaction-amplified exon 10 of the TSHR genomic DNA revealed a transition of GCT to GTT, resulting in an exchange of alanine 627 to valine in the patients and their mother. This might be a novel mutation or polymorphism, but we did not perform any functional gene study. But considering the clinical profiles, we can conclude that hyperthyroidism of these two brothers might come from the point mutation described above.
Alanine ; Autoimmunity ; DNA ; Exons ; Humans ; Hyperthyroidism* ; Mothers ; Point Mutation ; Receptors, Thyrotropin* ; Siblings* ; Sweat ; Sweating ; Thyroid Diseases ; Thyroid Gland ; Thyroidectomy ; Thyrotropin* ; Valine

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A Case of Bronchogenic Cyst with Nausea and Epigastric Pain.

Ji Hyun KIM ; Kang Won RHEE ; In Seok LIM ; Byung Hoon YOO ; Eung Sang CHOI

Korean Journal of Pediatrics.2005;48(3):333-336.

Bronchogenic cysts are rare congenital anomalies that arise early in gestation from abnormal budding of the developing respiratory system. Mediastinal bronchogenic cysts account for 10-15 percent of all primary mediastinal masses; 63.7 percent of patients are symptomatic. Common symptoms are fever, chest pain, cough, dyspnea, and dysphagia. Gastrointestinal symptoms except dysphagia are rare. It can be life threatening with compression, infection, hemorrhage, or rupture. Symptoms and signs of compression are more frequent in infants and children than in adults. It may be asymptomatic, or cough, infection, and hemoptysis may be observed. Complete excision is recommended. We report a case of bronchogenic cyst misdiagnosed as chronic gastritis with nausea and epigastric pain for a year.
Adult ; Bronchogenic Cyst* ; Chest Pain ; Child ; Cough ; Deglutition Disorders ; Dyspnea ; Fever ; Gastritis ; Hemoptysis ; Hemorrhage ; Humans ; Infant ; Nausea* ; Pregnancy ; Respiratory System ; Rupture

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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

Korean Journal of Pediatrics.2005;48(3):327-332.

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 of cyanosis, dry cough, dyspnea and abrupt weight loss. He had not been exposed to organic dusts, allergens or any other systemic disease infections. Chest radiology showed diffuse ground glass opacity in both lung fields. High resolution computed tomography(HRCT) showed multiple small patchy areas of consolidation with an underlying ground glass appearance in both lungs. The pathologic findings of lung biopsy tissue showed patchy areas of interstitial fibrosis, alveolar obliteration and nodular fibrotic areas, strongly suggesting interstitial pneumonia. No specific finding of viral inclusion or any other evidence of infection was found under electromicroscopy. We used peak flow meters to compare functional improvement. Forced expiratory volume in one second (FEV1) was decreased to 25 percent of predicted value. The boy was given treatment with prednisone and showed improvements in HRCT findings after two months. He was able to tolerate easy exercise in school and showed clinical improvements after one year of follow up.
Allergens ; Biopsy ; Capillaries ; Child ; Cough ; Cyanosis ; Dust ; Dyspnea ; Fibrosis ; Follow-Up Studies ; Forced Expiratory Volume ; Glass ; Humans ; Idiopathic Interstitial Pneumonias* ; Inflammation ; Lung ; Lung Diseases ; Lung Diseases, Interstitial ; Male ; Prednisone ; Thorax ; Weight Loss

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A Case of Multiple Giant Coronary Aneurysms with Large Mural Thrombus due to Kawasaki Disease in a Young Infant.

Eun Na CHOI ; Jeoung Tae KIM ; Yuria KIM ; Byung Won YOO ; Deok Young CHOI ; Jae Young CHOI ; Jun Hee SUL ; Sung Kye LEE ; Dong Soo KIM ; Young Hwan PARK

Korean Journal of Pediatrics.2005;48(3):321-326.

Kawasaki disease is an acute systemic vasculitis of unknown origin. Giant coronary aneurysm is one of the most serious complications, although peripheral artery vasculitis can produce life-threatening events. Myocardial ischemia and infarction can be caused by coronary artery stenosis, aneurysm, and stagnation of blood flow in coronary arteries which triggers thromboembolism. Atypical presentation in young infants often interferes with prompt diagnosis and timely treatment, resulting in poor outcomes. We describe a 3-month-old infant with multiple giant coronary aneurysms with flow stagnation, stenosis and large mural thrombus due to Kawasaki disease. He presented with a prolonged course of severe coronary involvement in spite of all measures to reduce coronary complications. Finally, surgical intervention was tried because of the worsening coronary artery abnormalities. The patient died of acute cardiorespiratory failure shortly after weaning from cardiopulmonary bypass.
Aneurysm ; Arteries ; Cardiopulmonary Bypass ; Constriction, Pathologic ; Coronary Aneurysm* ; Coronary Stenosis ; Coronary Thrombosis ; Coronary Vessels ; Diagnosis ; Humans ; Infant* ; Infarction ; Mucocutaneous Lymph Node Syndrome* ; Myocardial Ischemia ; Systemic Vasculitis ; Thromboembolism ; Thrombosis* ; Vasculitis ; Weaning

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Expression of Toll-like Receptor-2 on the Peripheral Blood Monocytes in Kawasaki Disease Patients.

Dae Hwan HWANG ; Jung Woo HAN ; Kyung Min CHOI ; Kyung Mi SHIN ; Dong Soo KIM

Korean Journal of Pediatrics.2005;48(3):315-320.

OBJECTIVE: Toll like receptor(TLR) is known to be involved in innate immunity. Many microbial antigens stimulate TLR, and as a result of intracellular signal transduction, they activate nuclear factor-kB which produces diverse inflammtory cytokines. Until now, many research topics in Kawasaki disease focused on cytokine increasement. In this study, we aim to reveal TLR increasement which might be associated with initiation of inflammatory response. METHODS: We obtained the peripheral blood of ten patients who were diagnosed with Kawasaki disease in Yonsei University College of Medicine from March 2003 to August 2003, as well as those of a febrile control group and the same number of a normal control group. Flow cytometry was done in all samples for quantification of TLR-2 expression in CD14 positive monocyte. And we also extracted total RNA of periphral monocyte and quantificated expression of TLR-2 mRNA by RT- PCR. RESULTS: The expression of TLR-2 in Kawasaki disease increased significantly compared with the normal control group but not when compared with the febrile control group. And the expression decreased slightly in the subacute phase of Kawasaki disease compared with the acute phase, but this was statistically insignificant. mRNA expression of TLR-2 in peripheral blood monocyte also increased in the acute phase of Kawasaki disease. CONCLUSION: Expression of TLR-2 in Kawasaki disease increased when compared with the normal control group, which means that innate immunity is associated with the pathogenesis of Kawasaki disease.
Cytokines ; Flow Cytometry ; Humans ; Immunity, Innate ; Monocytes* ; Mucocutaneous Lymph Node Syndrome* ; Polymerase Chain Reaction ; RNA ; RNA, Messenger ; Signal Transduction ; Toll-Like Receptors

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A Clinical Study on Relationship of Surgical Glove and Back Pain after Lumbar Puncture in Children.

Sung Ryoung HAN ; Wan Suk CHOI ; Hae Jeong LEE ; Hyun Seok KIM ; Ju Suk LEE ; Kyung Lae CHO

Korean Journal of Pediatrics.2005;48(3):310-314.

PROPOSE: In this study, we evaluated whether powder on surgical gloves is a cause of postpuncture backpain in children. MOTHODS: In 164 children with meningitis between July and September 1997, we did not remove powder from surgical gloves. However, in 149 children with menigitis between May and October 2001 the powder was removed from the surgical gloves. RESULTS: Out of the 164 patients in 1997, 41 cases(25.00%) were found to have postdural puncture backpain. On the other hand, out of 149 patients in 2001, with whom we used gloves from which the powder was removed, we found only 8 patients(5.36%) with postdural puncture backpain. CONCLUSION: We conclude that the powder on surgical gloves is one of the main causes of postdural puncture backpain in children.
Back Pain* ; Child* ; Gloves, Surgical* ; Hand ; Humans ; Meningitis ; Punctures ; Spinal Puncture*

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Benign Transient Hyperphosphatasemia of Infancy and Childhood.

Jung Hyun KWON ; Ji Hye PARK ; Hae Soon KIM ; Seung Joo LEE ; Mi Ae LEE

Korean Journal of Pediatrics.2005;48(3):306-309.

PURPOSE: The aim of study is to investigate the clinical manifestations and the etiology of markedly-elevated serum alkaline phosphatase(ALP) levels in children without accompanying liver or bone disease. METHODS: The serum ALP and other biochemical laboratory data of 4,989 patients attending the pediatric department of Ewha Womans University Mokdong Hospital between January 2002 to December 2002 were studied. The subjects' ages ranged from 4 months to 14 years. Among them, serum ALP levels were markedly elevated over 1,000 IU/L in 114 children. Among those 114 children, 97 children without liver or bone disease were reviewed retrospectively. RESULTS: Of the 114 children with serum ALP activity of over 1,000 IU/L, 97(85.8 percent) children had neither liver or bone disease. The average ALP activity level was 1,539+/-948 IU/L. The male:female ratio was 1:0.7. Forty-four children(45.3 percent) were between 4 months to 1-year-old, 19 children(19.3 percent) were between 1- to 2-year-old. We observed a seasonal clustering of cases in during October with 10 cases(10.2 percent) and in during December with 11 cases(11.3 percent). The most common clinical presentation was respiratory tract infection in 25 cases(26.9 percent), and acute gastroenteritis in 17 cases(17.5 percent). Forty-four cases had follow-up ALP activity level, 1 month later and the activity decreased under 1,000 IU/L in 37 cases(84percent). CONCLUSION: Hyperphosphatasemia is a benign and transient phenomenon. If there are no clinical and laboratory abnormalities of liver and bone, we suggest monitoring monitor the high serum ALP level 1 month later. If decreased, it seems that it is not necessary for specific examinations to exclude other diseases of malignant condition.
Alkaline Phosphatase ; Bone Diseases ; Child ; Child, Preschool ; Female ; Follow-Up Studies ; Gastroenteritis ; Humans ; Liver ; Respiratory Tract Infections ; Retrospective Studies ; Seasons

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Evaluation of Serum Insulin-Like Growth Factor(IGF)-I, Insulin-Like Growth Factor Binding Protein(IGFBP)-2 and IGFBP-3 Levels in Healthy Korean Children.

Gi Hoon YANG ; Hye Lim JUNG ; Deok Soo KIM ; Jae Won SHIM ; Jung Yeon SHIM ; Moon Soo PARK

Korean Journal of Pediatrics.2005;48(3):298-305.

PURPOSE: We performed this study to evaluate the mean serum levels of insulin-like growth factor (IGF)-I, insulin-like growth factor binding protein(IGFBP)-2 and IGFBP-3 in healthy Korean children according to age and sex. METHODS: Ninety two healthy children, consisting of 42 boys and 50 girls, were classified into five groups according to age:neonate; infancy; early childhood; late childhood; and adolescence. We measured serum levels of IGF-I, IGFBP-2 and IGFBP-3 by enzyme-linked immunosorbent assay(ELISA) and analysed the serum levels according to sex and age group. RESULTS: For boys, the mean serum levels of IGF-I(ng/mL) in neonate, infancy, early childhood, late childhood and adolescence were 41.1+/-3.6, 70.9+/-33.7, 103.5+/-97.2, 89.8+/-46.5 and 51.4+/-27.8, respectively. Those of IGFBP-2(ng/mL) were 8.2+/-3.4, 5.8+/-0.4, 9.3+/-4.0, 9.5+/-1.1 and 7.0+/-0.5, respectively. Those of IGFBP-3(ng/mL) were 559.2+/-215.2, 1,333.3+/-692.5, 2,254.6+/-1,513.8, 2,447.1+/-1,464.2, 1,533.6+/-807.4, respectively. For girls, the mean serum levels of IGF-I(ng/mL) according to five age groups were 53.3+/-9.5, 99.3+/-45.8, 69.6+/-51.1, 106.2+/-67.0 and 145.1+/-127.8, respectively. Those of IGFBP-2 (ng/mL) were 9.1+/-7.4, 5.3+/-0.9, 6.9+/-2.0, 10.5+/-3.0 and 7.9+/-1.3, respectively. Those of IGFBP-3(ng/ mL) were 858.2+/-433.4, 1,834.8+/-851.3, 1,404.3+/-570.2, 2,203.5+/-899.4 and 2,029.3+/-1,316.7, respectively. There were significant positive correlations observed between IGF-I and IGFBP-3 levels(r=0.589, P= 0.000). CONCLUSION: IGF-I and IGFBP-3 levels increased as children get older. The peak level of IGFBP-3 was observed in late childhood for both boys and girls, suggesting a current trend of children reaching peak growth velocity before adolescence. The IGFBP-2 level was higher in neonates compare to infancy, suggesting that IGFBP-2 is an important substance for fetal growth.
Adolescent ; Child* ; Female ; Fetal Development ; Humans ; Infant, Newborn ; Insulin-Like Growth Factor Binding Protein 2 ; Insulin-Like Growth Factor Binding Protein 3* ; Insulin-Like Growth Factor I

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Clinical Characteristics of Autoimmune Thyroid Disease Developed in Patients with Type 1 Diabetes Mellitus.

Se Min LEE ; Hye Rim CHUNG ; Su Young HONG ; Choong Ho SHIN ; Sei Won YANG

Korean Journal of Pediatrics.2005;48(3):292-297.

PURPOSE: It is known that 3-50 percent of type 1 diabetes mellitus(T1DM) patients develop autoimmune thyroid disease. We analyzed the clinical characteristics of autoimmune thyroid disease(AITD) developed in patients with T1DM in Korean. METHODS: The medical records of 139 patients, who were followed up in Department of Pediatrics, Seoul National University Children's Hospital from Jan. 1981 to Jul. 2004, were analyzed retrospectively. RESULTS: Forty-four males and 95 females were enrolled. At least one of the autoantibodies for thyroid was positive in 54 cases. The detection rate for AITD was not correlated with sex ratio, control of T1DM, body mass index, age at diagnosis of T1DM, and familial history of thyroid disease, between two groups. In the male group, AITD was more frequently found at a younger age than in the female group. The frequency of AITD was significantly higher in the goiter group without sex differences. In the thyroid disease group, 40 patients(74.0 percent) were euthyroid, seven patients(12.9 percent) were hypothyroid, and seven patients(12.9 percent) were hyperthyroid. CONCLUSION: We should monitor thyroid function and autoantibodies routinely in T1DM patients who develop goiters, or young boys with T1DM.
Autoantibodies ; Body Mass Index ; Diabetes Mellitus, Type 1* ; Diagnosis ; Female ; Goiter ; Humans ; Male ; Medical Records ; Pediatrics ; Retrospective Studies ; Seoul ; Sex Characteristics ; Sex Ratio ; Thyroid Diseases* ; Thyroid Gland*

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