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Case Report
Spontaneous intracranial internal carotid artery dissection in a child with psoriasis
Young Ok Kim, Young Jun Son, Young Jong Woo, Sook Jung Yun
Clin Exp Pediatr. 2009;52(9):1044-1047.   Published online September 15, 2009
A 13-year-old girl with psoriasis of the elbow, trunk, and face suddenly developed a severe headache followed by left hemiparesis and facial palsy. Brain magnetic resonance imaging showed an acute infarction of the right temporofrontal lobe and basal ganglia on the T2- and diffusion-weighted images. Cerebral angiography showed pre-occlusive irregular scalloped stenosis (99%) in the proximal M1 segment of the...
Two adolescent cases of Fitz-Hugh-Curtis syndrome
Ji Hye Kim, Sung Hee Oh
Clin Exp Pediatr. 2009;52(9):1038-1043.   Published online September 15, 2009
Two adolescent cases of Fitz-Hugh-Curtis syndrome, which has not been previously reported in Korean girls, presenting with right upper-quadrant abdominal pain, nausea, vomiting, and fever are reported here. A careful and thorough inquiry into the sexual history of the first patient, which was not done upon admission, led to a careful reassessment of the dynamic abdominal computed tomography scan revealing...
Use of an Amplatzer Vascular Plug to occlude a tubular type of patent ductus arteriosus
Eun-Young Choi, So-Ick Jang, Soo-Jin Kim
Clin Exp Pediatr. 2009;52(9):1035-1037.   Published online September 15, 2009
Patent ductus arteriosus (PDA) is a common congenital heart defect. All PDAs, regardless of size or degree of symptoms, require occlusion. Transcatheter PDA occlusion features fewer complications than trans-thoracic closure. It is also more cost-effective and has an excellent occlusion rate. Therefore, transcatheter PDA occlusion is accepted as the standard treatment option for PDA. However, tubular-type PDAs are difficult to...
A case of adolescent Kawasaki disease with Epstein-Barr virus-associated infectious mononucleosis complicated by splenic infarction
Byeong Sam Choi, Bo Sang Kwon, Gi Beom Kim, Yoon Kyung Jeon, Jung-Eun Cheon, Eun Jung Bae, Chung Il Noh, Jung Yun Choi, Yong Soo Yun
Clin Exp Pediatr. 2009;52(9):1029-1034.   Published online September 15, 2009
Kawasaki disease (KD) is an acute systemic vasculitis of unknown etiology that affects children. There are few reports that describe the Epstein-Barr virus (EBV) as the possible infectious agent of KD. Here, we describe a case of KD in a 15- year-old boy complicated with giant coronary artery aneurysms, pericardial effusion, and splenic infarction. The clinical course of KD was...
Plastic bronchitis in children: 2 cases
Yeo Hyang Kim, Hee Jung Choi, Jung Ok Kim, Myung Chul Hyun
Clin Exp Pediatr. 2009;52(7):832-836.   Published online July 15, 2009
Plastic bronchitis is a rare disorder characterized by the formation of extensive, obstructing endobronchial casts. It is associated with asthma and complex cardiac defects such as those requiring the Fontan procedure. The treatment of plastic bronchitis comprises conventional therapy involving spontaneous expectoration and bronchoscopic removal and specific therapy with several new drugs. Herein, we describe the cases of 2 patients...
Unrelated stem cell transplantation after reduced-intensity conditioning plus rituximab for Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis with CNS involvement
Hee Jo Baek, Hoon Kook, Dong Kyun Han, Min-Cheol Lee, Tae Woong Jeong, Tai Ju Hwang
Clin Exp Pediatr. 2009;52(6):725-729.   Published online June 15, 2009
Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) with central nervous system (CNS) involvement is usually fatal unless stem cell transplant (SCT) is offered. However, SCT with conventional intensity conditioning is associated with high transplant-related mortality. We describe our experience with unrelated SCTs after reduced-intensity conditioning (RIC) for patients with EBV-HLH with progressive CNS disease. This approach was associated with minimal toxicities and...
A Korean familial case of hereditary complement 7 deficiency
Moon Kyu Ki, Kyung Yul Lee, Jun Hwa Lee
Clin Exp Pediatr. 2009;52(6):721-724.   Published online June 15, 2009
Meningococcal infections can be associated with abnormalities of the complement system, which contains 5 terminal complement proteins. Furthermore, deficiencies in 1 of these 5, complement component 7 (C7), leads to the loss of complement lytic function, and affected patients show increased susceptibility to recurrent meningococcal meningitis and systemic Neisseria gonorrhoeae infection. In September 2003, an 11-year-old female patient presented at...
A case of newly developed pulmonary lesion during the antitubercular agents in tuberculous pleurisy : A paradoxical response
Jee Min Park, Youn Ho Shin, Gyu Rak Chon, Hyun Joon Shin, Young Chil Choi
Clin Exp Pediatr. 2009;52(6):717-720.   Published online June 15, 2009
Paradoxical response refers to the enlargement of old lesions or unexpected appearance of new lesions after initial improvement following treatment with antitubercular agents. Various types of paradoxical responses have been reported in the world, but they are rarely reported in Korean children. We report the case of a 17-year-old boy who was diagnosed with tuberculous pleurisy and was treated appropriately....
Facial palsy as the presenting symptom of acute myeloid leukemia in children: Three cases with stem cell transplantatio
Hee Jo Baek, Dong Kyun Han, Young Ok Kim, Ic Sun Choi, Tai Ju Hwang, Hoon Kook
Clin Exp Pediatr. 2009;52(6):713-716.   Published online June 15, 2009
Facial palsy as the presenting symptom of leukemia is very rare, especially in acute myeloid leukemia. A review of the medical literature identified reports on 8 children with AML who had facial paralysis as the presenting sign. Whole brain irradiation (WBI) has been applied in most cases. We present the cases of 3 such children. Achieving a remission without WBI,...
A case of congenital syphilis mistaken for possible child abuse
Soon Ju Kim, Seung Woo Lee, Jung Woo Rhim, You Sook Youn, Jun Sung Lee, Kyung Yil Lee, Ja Young Hwang
Clin Exp Pediatr. 2009;52(6):710-712.   Published online June 15, 2009
We describe the case of a 4-month-old male infant diagnosed with early congenital syphilis during evaluation of a left distal humerus fracture. This report emphasizes the importance of screening for syphilis among pregnant women and newborns, and is a reminder of the continued existence of congenital syphilis.
A case of acute aseptic meningitis associated with herpes zoster
Myong A Kim, Rita Miyoung Yu, Kee Hyuck Kim, Hee Jung Chung
Clin Exp Pediatr. 2009;52(6):705-709.   Published online June 15, 2009
Herpes zoster is caused by the varicella-zoster virus (VZV), which affects nerve ganglions. VZV infection may be associated with neurologic complications, which are usually observed after vesicular exanthem. Acute aseptic meningitis is a rare complication of VZV reactivation. We report the case of a previously healthy 14-year-old boy who suffered from aseptic meningitis that was attributed to reactivated VZV infection...
Two cases of Smith-Magenis syndrome
Seong Kwan Jung, Kyu Hee Park, Hae Kyung Shin, So Hee Eun, Baik-Lin Eun, Kee Hwan Yoo, Young Sook Hong, Joo Won Lee, Sook Young Bae
Clin Exp Pediatr. 2009;52(6):701-704.   Published online June 15, 2009
SmithMagenis syndrome (SMS) is a rare disorder with multiple congenital anomalies caused by a heterozygous interstitial deletion involving chromosome 17p11.2, where the retinoic acid-induced 1 (RAI1) gene is located, or by a mutation of RAI1. Approximately 90% of the patients with SMS have a detectable 17p11.2 microdeletion on fluorescence in-situ hybridization (FISH). SMS is characterized by mental retardation, distinctive behavioral...
Kikuchi-Fujimoto disease with aseptic meningitis
Se Jin Park, Won Jin Moon, Wan Seop Kim, Kyo Sun Kim
Clin Exp Pediatr. 2009;52(5):622-626.   Published online May 15, 2009
Kikuchi-Fujimoto disease was initially described as a self-limiting histiocytic necrotizing lymphadenitis in Japan in 1972, and is predominantly observed in women under the age of 30 year and in Asian populations. The pathogenesis is still poorly understood but is thought to include infections, and autoimmune and neoplastic diseases. The most common clinical manifestations are fever and painless cervical lymphadenitis. Diagnosis...
Bilateral paroxysmal hemicrania with autonomic features in a child: A case report
Young Il Rho
Clin Exp Pediatr. 2009;52(5):619-621.   Published online May 15, 2009
Paroxysmal hemicrania (PH) is rare in children and not widely recognized. It is characterized by pain attacks and associated symptoms and signs similar to those experiencing cluster headaches, but the features have a shorter effect, are more frequent, and respond completely to indomethacin. Some patients with PH may experience slight pain across the midline. There are only four cases of...
Two cases of Kawasaki disease following pneumonia
Hyun Jung Kim, Soo Jin Lee
Clin Exp Pediatr. 2009;52(5):615-618.   Published online May 15, 2009
Kawasaki disease (KD) causes multisystemic vasculitis but rarely manifests with pulmonary symptoms. As its etiology is still unknown, there are no specific diagnostic tools available, and KD can be diagnosed only by the symptom pattern. The presence of unusual clinical manifestations often leads to delayed diagnosis. Here, we report two cases of KD with an initial presentation of pneumonia. KD...
Familial systemic lupus erythematosus in two Korean male siblings
Hyun Sik Kang, Hyun Ju Oh, Young Ree Kim, Jae-Wang Kim, Kyung-Sue Shin
Clin Exp Pediatr. 2009;52(5):611-614.   Published online May 15, 2009
Systemic lupus erythematosus (SLE) is a multisystemic autoimmune disease characterized by the production of a wide range of autoantibodies, resulting in tissue damage. Although the susceptibility to SLE has been attributed to complex interactions between genetic and environmental factors, the influence of a genetic predisposition to SLE is supported by observations of familial aggregations. Family studies have found that siblings...
Herpes zoster complicated by deep vein thrombosis : a case report
Woo-Yeon Cho, Young-Kuk Cho, Jae-Sook Ma
Clin Exp Pediatr. 2009;52(5):607-610.   Published online May 15, 2009
Varicella zoster virus (VZV) causes two diseases: Varicella, a generalized, primary infection, and herpes zoster (zoster), a secondary infection caused by latent VZV reactivation. Zoster can also be caused by latent VZV reactivation after a varicella vaccination. The complications associated with varicella include cutaneous infections, which are the most common, as well as pulmonary and neurological involvement. However, a deep...
De novo interstitial direct duplication 8 (p21.3p23.1) with Pierre Robin sequence
Soon Min Lee, Min Soo Park, Kook In Park, Ran Namgung, Chul Lee, Jin Seong Lee, Kyung A Lee, Jong-Rak Choi
Clin Exp Pediatr. 2009;52(5):603-606.   Published online May 15, 2009
The Pierre Robin sequence (PRS) is the nonrandom association of micrognathia, cleft palate, and glossoptosis, leading to respiratory and feeding difficulties that appear neurogenic rather than mechanical in causation. Genetic determinants are thought to underlie this functional and morphological entity, based on the existence of Mendelian syndromes with PRS. Here, we demonstrate the association of PRS with trisomy 8p due...
A case of fatal pneumococcal 19A meningoencephalitis despite administration of seven-valent pneumococcal conjugate vaccines
Ah Rum Heo, Jun Hwa Lee
Clin Exp Pediatr. 2009;52(4):508-511.   Published online April 15, 2009
Streptococcus pneumoniae is a major cause of serious invasive diseases in children, especially in young infants, but seven- valent pneumococcal conjugate vaccine (PCV7) is believed to prevent invasive pneumococcal pneumonia and meningitis in young children. However, recently, the incidence of non-PCV7 serotype has increased after PCV7 vaccination. A 14-month- old female patient presented at our emergency room with mental change...
Autoimmune hepatitis-primary sclerosing cholangitis overlap syndrome in a 10-year-old girl with ulcerative colitis
Jeana Hong, Mi Kyoung Song, Jae Sung Ko, Gyeong Hoon Kang, Woo Sun Kim, Jeong Kee Seo
Clin Exp Pediatr. 2009;52(4):504-507.   Published online April 15, 2009
Autoimmune hepatitis (AIH), primary sclerosing cholangitis (PSC), and primary biliary cirrhosis (PBC) are chronic autoimmune liver diseases. Overlap syndrome is defined as a condition in which the clinical, biochemical, and histological features of these autoimmune diseases are overlapped. Thus, it is difficult to appreciate overlap syndrome as an actual diagnostic entity. Only a few cases of the overlap syndrome of...
Late adverse reactions to iopromide (Ultravist®) diagnosed by the patch test: a case report
Soon Joo Lee, Eun Mi Yang, Woo Yeon Choi, Eun Song Song, Dong Kyun Han, Young Kuk Cho, Jae-Sook Ma
Clin Exp Pediatr. 2009;52(4):499-503.   Published online April 15, 2009
Iodinated contrast media (CM) can cause immediate and late reactions. We treated a patient with a recurrent generalized maculopapular rash and a fever that occurred within two days of exposure to iodinated CM, iopromide (UltravistⓇ), for chest computed tomography. We performed skin testing including prick tests, intradermal tests, and patch tests. Our findings indicated a late skin reaction to Ultravist¢cin...
A case of antiepileptic drug hypersensitivity syndrome by lamotrigine mimicking infectious mononucleosis and atypical Kawasaki disease
Su Jung Yoo, Ihl Sung Park, Eun Sook Suh
Clin Exp Pediatr. 2009;52(3):389-391.   Published online March 15, 2009
AHS는 항경련제에 의한 부작용으로 드물게 발생하지만 생명을 위협할 수 있는 지연형 면역반응이다. 보통 항경련제 복용 후 1-8주 사이에 고열, 피부발진, 림프절병증의 임상 증세를 나타내고, 혈액, 간, 신장, 또는 폐 등의 내부 장기를 침범한다. AHS는 항경련제에 의한 부작용으로 증상이 전신적으로 다양하게 나타날 수 있다. 그리고 전염성 단핵구증이나 가와사키병과 같은 전신적 질환들과도 유사한 임상증상들을 보이기 때문에 이런 질환들과 감별하는 것이 중요하고 사망률도...
A case of acute transverse myelitis following chickenpox
Min Young Kim, Eun Sook Suh
Clin Exp Pediatr. 2009;52(3):380-384.   Published online March 15, 2009
Acute transverse myelitis (ATM) in most patients is characterized by an abrupt onset of progressive weakness and sensory disturbance in the lower extremities with a preceding viral infection such as Epstein-Barr, herpes simplex, influenza, mumps and Varicella-zoster viruses (VZV). Although less frequent, some residual deficits including bladder dysfunction or weakness in the lower extremities may follow ATM, from which recovery...
Pseudohypoaldosteronism in a premature neonate with severe polyhydramnios in utero
So Yoon Ahn, Son Moon Shin, Kyung Ah Kim, Yeon Kyung Lee, Sun Young Ko
Clin Exp Pediatr. 2009;52(3):376-379.   Published online March 15, 2009
We report a case of a premature newborn baby who presented with hyponatremia, hyperkalemia, and metabolic acidosis accompanied by severe polyhydramnios in utero. The baby was diagnosed with pseudohypoaldosteronism on the basis of normal 17-hydroxyprogesterone levels, elevated aldosterone, and clinical symptoms. His serum electrolyte levels were corrected with sodium chloride supplementation. Sodium supplementation was reduced gradually and discontinued at 5...
Intestinal obstruction caused by a duplication cyst of the cecum in a neonate
Seung-woon Keum, Min-Woo Hwang, Jong-In Na, Seung-taek Yu, Dong-Baek Kang, Yeon-Kyun Oh
Clin Exp Pediatr. 2009;52(2):261-264.   Published online February 15, 2009
Duplication cysts are rare congenital malformations, that may be detected anywhere along the alimentary tract, and they may communicate with the intestinal tract. Cystic duplication of the cecum is especially rare. About 80% of these cases are detected in the first 2 years of life as a result of an acute intestinal obstruction, which manifests as vomiting, recurrent abdominal pain,...
A case of idiopathic pulmonary hemosiderosis with seasonal recurrence
Ga Young Kwak, Na Young Lee, Moon Hee Lee, Soo Young Lee, Seung Yun Chung, Jin Han Kang, Dae Chul Jeong
Clin Exp Pediatr. 2009;52(2):256-260.   Published online February 15, 2009
Idiopathic pulmonary hemosiderosis (IPH) is a rare disease affecting mostly children. This disorder is characterized by recurrent episodes of hemoptysis, bilateral diffuse pulmonary infiltrates, and iron-deficiency anemia. An acute fulminant alveolar hemorrhage can be fatal due to respiratory failure, while chronic hemorrhage leads to hemosiderin-laden macrophages and pulmonary fibrosis. Genetic, autoimmune, allergic, environmental, and metabolic mechanisms of pathogenesis have been...
Atypical presentation of Kawasaki disease resembling a retropharyngeal abscess
Eu Jin Kim, Young Su Lim, Ji Eun Yoon, Heon-Seok Han
Clin Exp Pediatr. 2009;52(2):251-255.   Published online February 15, 2009
Kawasaki disease is an acute systemic inflammatory disorder, the etiology of which has not yet been established. The clinical manifestations are non-specific and are common to many pediatric infectious and immunologic diseases. In 2 cases presenting fever, cervical lymphadenopathy, and retropharyngeal abscess-like lesions on the neck shown in a computerized tomography (CT) scan, the diagnosis of Kawasaki disease was delayed....
Atypical presentation of Kawasaki disease resembling a retropharyngeal abscess
Sun-Hee Choi, Yu-Min Lee, Yeong-Ho Rha
Clin Exp Pediatr. 2009;52(2):247-250.   Published online February 15, 2009
Stevens-Johnson syndrome (SJS) presents with widespread blisters, erythematous or purpuric macules, and one or more mucous membrane erosions. Various etiologic factors, including infection, vaccination, drug administration, systemic diseases, physical agents, and food have been implicated as causes of SJS. Mycoplasma pneumoniae is the most common infectious agent to cause SJS in children. In recent literature, M. pneumoniae-induced SJS with mucositis...
A case of mosaic ring chromosome 13 syndrome
Soo Young Kim, Soo Min O, Mi Jeong Kim, Eun Song Song, Young Ok Kim, Young Youn Choi, Young Jong Woo, Tai Ju Hwang
Clin Exp Pediatr. 2009;52(2):242-246.   Published online February 15, 2009
The clinical features of ring chromosome 13 include mental and growth retardation, CNS anomalies, facial dysmorphism, cardiac defects, genital malformations, limb anomalies, skeletal deformities and anal malformations. Although many cases of ring chromosome 13 have been reported worldwide, only 6 cases have been reported in Korea, and the latter cases were not mosaic but pure ring chromosome 13. Here we...
Changes in N-terminal pro-B-type natriuretic peptide in a neonate with symptomatic isolated left ventricular noncompaction
Ji Hyeun Song, Yeo Hyang Kim, Chun Soo Kim, Sang Lak Lee, Tae Chan Kwon
Clin Exp Pediatr. 2009;52(1):129-132.   Published online January 15, 2009
We describe here our experience with a neonate presenting with cyanosis, grunting, and cardiomegaly, who was diagnosed with isolated left ventricular noncompaction (IVNC) by echocardiography. The patient had high levels of N-terminal pro-B-type natriuretic peptide (NT pro-BNP) and symptoms of heart failure including poor feeding and tachypnea. During the period in which NT pro-BNP levels steadily increased, the patient suffered...
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