• Clinical and Experimental Pediatrics
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• v.49 no.4
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• pp.381-387
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• 2006

Purpose : Neonatal lupus is characterized by congenital complete heart block(CCHB), cutaneous rash, and laboratory abnormalities in infants born to mothers with systemic lupus erythematosus(SLE). This study aims to examine the incidence of CCHB and clinical outcome in neonates born to mothers with SLE. Methods : The study group consisted of 49 neonates, born from 57 pregnancies of 55 women with SLE, diagnosed at Hanyang University Hospital for the period between January 1997 and January 2005. Clinical and laboratory data were retrospectively identified from medical record. Results : There were 5(8.8 percent) spontaneous abortions and one(1.8 percent) still births among 57 pregnancies of 55 mothers. Of 49 live births, 15(26.3 percent) were premature and eight(12.3 percent) were small for their gestational age. There was one(1.8 percent) CCHB suspected during pregnancy on fetal echocardiograpy in a fetus of mother with systemic lupus erythematosus and the fetus was not born by artificial abortion because of mother. There was no CCHB among EKG findings of 49 newborns. Laboratory testing showed hematologic abnormalities among 25.6 percent(10/39) of the babies. 5.1 percent(2/39) and 7.7 percent(3/39) of them were diagnosed as neutropenia, and thrombocytopenia was seen respectively. Anti-SSA(Ro) and antiphospholipid antibodies were predictive factors for prematurity(P=0.003, P=0.049). Anticardiolipin antibodies were predictive factors for ventilatory care(P=0.018). Conclusion : The incidence of CCHB among neonates born to mothers with SLE, which was measured in this study, was lower than that in earlier studies. A high incidence of hematologic abnormalities was found in our study. It is suggested that careful examination should be made of skin for the diagnosis of neonatal lupus.

• Clinical and Experimental Pediatrics
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• v.48 no.10
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• pp.1107-1115
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• 2005

Purpose : An outbreak of ESBL-producing Shigella sonnei enteritis was unprecedented not only in Korea but throughout the world in the past. We intended to devise a management guideline for ESBL-producing shigellosis based on analysis of clinical manifestations and response to therapy. Methods : We analyzed 103 patients who were admitted to the hospital with acute GI symptoms and were shown positive result for S. sonnei on stool culture. We performed sensitivity test to the antibiotics and DNA sequencing of ESBL gene in the isolated S. sonnei colonies. In addition, we retrospectively analyzed their clinical characteristics, laboratory results, and clinical and microbiological responses to the antibiotics. Results : Among the clinical manifestations, fever was the most frequent(96.1%), followed by diarrhea(93.2%), abdominal pain(76.7%), headache(71.8%), vomiting(65.0%), and nausea(41.7%). The fever was sustained for average of 2.0 days and diarrhea for 3.9 days. Watery diarrhea was the most common(69%) followed by mucoid(26%), and bloody stool(5%). On peripheral blood smear, leukocytosis was noted in 53.4% of patients, and 78.6% of patients tested positive for serum CRP response. On stool direct smear, 11.7% of patients showed more than 50 WBCs/HPF, and 9.7% of patients between 5 to 20 WBCs/HPF. Stool occult blood was positive in 71% of patients. Production of CTX-M-14 type ESBL was reported for all S. sonnei strains isolated from this outbreak. Microbiological eradication rates to various antibiotics were as follows : 100%(9/9) to ciprofloxacin, 100% 5/5) to azithromycin, 6.9%(5/72) to cefdinir, 0%(0/8) to ceftriaxone, 12.5%(1/8) to ceftizoxime, 0%(0/ 8) to TMP/SMX, 42.9%(3/7) to ampicillin/sulbactam, 20%(1/5) to amoxicillin/clavulanic acid, and 68.8 %(11/16) to imipenem/cilastatin. Conclusion : It is presumed that azithromycin can be an attractive option for the treatment of ESBL-producing S. sonnei enteritis in pediatric population, given its cost-effectiveness and safety. Although ciprofloxacin is another cost-effective agent, its use in pediatric population may be a bit too premature.

• Clinical and Experimental Pediatrics
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• v.45 no.4
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• pp.459-465
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• 2002

Purpose : The aim of this study was to evaluate the latest tendency of esophageal foreign body's extraction and to obtain a consensus from recent trends of indications and techniques of flexible endoscopy of esophageal FB in children. Methods : We retrospectively reviewed medical records of 127 cases with foreign bodies in esophagus at Dept. of Pediatrics and Otorhinolaryngology, Gyeongsang National University Hospital (GNUH) from Jun, 1987 to July, 2001. They were divided into two groups by the kinds of endoscopy : flexible endoscope(66 cases) or rigid endoscope(61 cases). Rigid endoscopy was performed under general anesthesia at Dept. of Otorhinolaryngology but flexible endoscopy was performed without general anesthesia or sedative drugs(midazolam or diazepam). Results : An annual number of cases of two groups were similar from 1991 to 1998. But from 1999, flexible endoscopy was performed actively. Asymptomatic cases were frequently observed in flexible endoscopy(28 cases/66 cases) but swallowing difficulties were frequently observed in the rigid endoscopy group(25 cases/61 cases). Other symptoms were vomiting, irritability, chest discomfort and abdominal pain. The total number of cases with underlying disease(esophageal stenosis, cerebral palsy) was 8. The total number of cases with complications (erosion, ulcer, bleeding, perforation) was 11. The above cases were not correlated between the two groups. In 55 cases(83.3%) of the flexible endoscopic group and 53 cases(86.8%) of the rigid endoscopic group, foreign bodies in the esophagus were removed within 24 hours. Conclusion : We could not find any benefit in rigid endoscopic technique. Flexible endoscopic FB removal can be performed safely and effectively in children by an experienced endoscopist.

• Clinical and Experimental Pediatrics
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• v.45 no.3
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• pp.302-310
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• 2002

Purpose : X-linked agammaglobulinemia(XLA) is an immunodeficiency caused by abnormalities in Bruton's tyrosine kinase(Btk), and is characterized by a deficiency of peripheral blood B cells. We studied cytoplasmic expression of Btk protein and analyzed the Btk gene in peripheral blood mononuclear cells(PBMC) from three XLA families in Korea. Methods : Heparinized venous blood samples were collected from four XLA patients and additional family members in three unrelated XLA families. Mononuclear cells were separated from their blood and the intracellular Btk protein was characterized by a flow cytometry. The mutation analysis was performed using direct sequencing. Results : Cytoplasmic expression of Btk protein in monocytes was not detected in the patients with XLA. We observed a novel deletion and two point mutations within introns(intron 1 and intron 18) resulting in alternative splicings. In XLA family 2, a 980 bp deletion(from intron 9+191 T to intron 10-215 C) including exon 10 was found in patient P2. He was the only sporadic case in this study, because his mother and brother showed a normal Btk expression by flow cytometry. Conclusion : These identified genetic alterations support the molecular heterogeneity of Btk gene in XLA disease. Additionally, by means of flow cytometric analysis, we diagnosed three hypogammaglobulinemia patients as XLA. Advancements in diagnostic methods has facilitated a prompt and definite diagnosis of this disease.

• Clinical and Experimental Pediatrics
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• v.46 no.6
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• pp.561-565
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• 2003

Purpose : Urinary tract infection(UTI) is the most common bacterial infectious disease that may induce severe renal injury unless early diagnosis and appropriate treatment are performed. If recurrent UTI is prevented, renal injury can be also reduced. Therefore, we studied the risk factors of recurrent UTI in children. Methods : We performed a retrospective study of 168 children(58 girls and 110 boys) who were treated for UTI in the Department of Pediatrics, Korea University Medical Center, during 2000-2001. Among 168 children, 93 children were followed up for more than six months. For the detection of recurrence of UTI, we performed monthly routine urine cultures and physical examinations. Results : The total rate of recurrence was 32.3%. The recurrent rate in boys and girls were 37.1% and 17.4%, respectively(P<0.05). The most common causative bacteria in the first onset and in recurrence were Escherichia coli. There was a significant difference in the onset age of UTI between boys with recurrence($4.8{\pm}1.0months$) and without recurrence($16.5{\pm}3.8months$)(P<0.01). In 77% of cases, urinary tract infection recurred within six months of the first infection. The time of the first recurrence after UTI was $3.7{\pm}0.6months$ in boys and $14{\pm}8.2months$ in girls(P<0.01). The number of recurrences showed a significant difference between the group under the age of one year($0.69{\pm}0.8/year$) and those above the age of one year($0.16{\pm}0.4/year$)(P<0.05). There was no difference in the recurrent rate between those with structural abnormality and those with normal anatomy. Conclusion : Monthly routine urine cultures are efficient in detecting recurrent UTI in children. Because the male sex and young age especially less than one year of age are risk factors for increased recurrence rate of UTI, these children should be followed-up with urine cultures.

• Clinical and Experimental Pediatrics
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• v.46 no.6
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• pp.566-571
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• 2003

Purpose : Leukoencephalopathy(LE) is one of the most serious complications in children with hematologic malignancies during the course of treatment. Early recognition is important to reduce the impact and sequelae from LE. We therefore investigated the clinical features of LE following central nervous system(CNS) prophylaxis in children with hematologic malignancies and evaluated the significance of regular check-ups of brain MRI. Methods : We retrospectively reviewed children with hematologic malignancies who had CNS prophylaxis including intrathecal(IT) methotrexate(MTX) and/or cranial irradiation at the Department of Pediatrics, Kyungpook National University Hospital from Oct. 1995 to May 2002. Fifteen cases of acute leukemia and one case of lymphoma who experienced LE following CNS prophylaxis were included in the study. Clinical data were analyzed from the medical records and brain MRIs were reviewed by neuroradiologists. Results : The ages ranged from 1 to 13 years(median age=5.2 years), and the male to female ratio was 3 : 1. The time interval from the beginning of chemotherapy to the time of diagnosis of LE ranged from 2 to 17 months. They all had IT MTX two to 15 times and ten underwent cranial irradiation(1,800 rads). At the time of diagnosis, ten of them had neuropsychiatric symptoms including seizures, personality changes, headache, etc. After the change of treatment modality, four cases showed significant improvement on follow-up MRIs, six cases had no significant changes and two had worsening of LE. Four patients died of infection and bone marrow relapse. Conclusion : CNS prophylaxis with IT therapy and cranial irradiation may cause leukoencephalopathy during the course of treatment. As a result, regular brain MRI check-up is recommended for the early detection and reducing the incidence of LE, along with changes in the treatment modality.

• Clinical and Experimental Pediatrics
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• v.46 no.9
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• pp.903-908
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• 2003

Purpose : Kawasaki disease is notorious for coronary arterial complication which is usually developed as a febrile disease in early childhood. Increased polymorphonucleus(PMN) cell levels in acute phases may be associated with the pathophysiology of Kawasaki disease. We studied the relationship between coronary arterial dilatation and elastase activity which was excreted from PMN cell and roles as an important factor for vasculitis. Methods : Ten patients diagnosed with Kawasaki disease in Yonsei University Medical Center were examined between November, 2001 and January, 2002. In addition, 15 patients with other febrile diseases were also examined. Echocardiography was done in patients with Kawasaki disease on the first day of admission and four weeks after the onset of the disease. At each time, venous samples were drawn and separated into plasma and leukocytes. In patients with other febrile disease, samples were drawn on admission. Elastase activities in plasma and neutrophil extracts were measured. Results : The significant increased plasma elastase activity, $6.19{\pm}0.74U/mL$, found in Kawasaki disease patients compared with the other febrile disease patients, $4.86{\pm}1.17U/mL$(P<0.05). And there was no significance between the above two diseases in terms of the elastase activity in neutrophil extracts. The relationship between initial elastase activity and the coronary arterial complication which was shown in subacute phase wasn't significant. Conclusion : Plasma elastase activity was increased in Kawasaki disease significantly, but the initial plasma elastase activity in the acute phase could not reflect the range of coronary arterial complication.

• Clinical and Experimental Pediatrics
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• v.46 no.3
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• pp.284-290
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• 2003

Purpose : A new airway inflammatory marker, exhaled nitric oxide(ENO) has been reported to correlate with bronchial hyperresponsiveness(BHR) and atopy. The purpose of this study was to analyze the relationship of ENO with BHR or atopy in patients with asthma and with allergic rhinitis. Methods : The subjects consisted of 55 children with asthma, 17 with allergic rhinitis, and 14 healthy controls. The asthma group was subdivided into the atopic asthma group(n=37) and the nonatopic asthma group(n=18) and the allergic rhinitis group into BHR group(n=7) and non-BHR group(n=10). All were investigated with spirometry and measurements of ENO concentration. The correlations between ENO concentration and both methacholine $PC_{20}$(provocative concentration causing a 20% decrease in forced expiratory volume in one second) and the number of allergen skin test positivity were analyzed. Results : ENO concentrations of both asthma and allergic rhinitis groups were significantly greater than that of control(P<0.01). ENO concentration of atopic asthma was significantly greater than that of nonatopic asthma(P<0.01). In allergic rhinitis, ENO concentration did not differ according to the presence or absence of BHR(P=0.50). ENO concentrations correlated significantly with the number of skin test positivity(r=0.32, P=0.02) or methacholine $PC_{20}$(r=-0.38, P<0.01) in asthma group, but not in the allergic rhinitis group(r=0.42, P=0.09; r=-0.06, P=0.83). Conclusion : In asthma patients, some pathogenetic mechanisms associated with atopy and BHR seem to influence ENO concentration. In allergic rhinitis patients, some factors other than BHR may be important in determining ENO concentration.

• Clinical and Experimental Pediatrics
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• v.52 no.2
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• pp.176-180
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• 2009

Purpose : The objective of this study was to establish the serum IGF-1 level in newborn infants, and investigate its association with growth and diseases. Methods : In a retrospective study, serum IGF-1 levels were measured for newborn infants admitted to NICU at Kyungpook University Hospital from March 2007 to July 2007. Birth data, disease history, and hospital course were obtained from medical records. Results : Of 52 blood samples obtained at birth, serum IGF-l levels in 30 preterm infants ($31.6{\pm}27.3$ ng/mL) were lower than in 22 full-term infants ($53.4{\pm}40.0$ ng/mL; P<0.05). In sick full-term infants, serum IGF-1 levels ($46.0{\pm}40.2$ ng/mL) were lower than in healthy full-term infants ($64.1{\pm}39.5$ ng/mL; P<0.05). In preterm infants, there were no differences in IGF-1 levels between healthy ($33.2{\pm}23.3$ ng/mL) and sick infants ($30.6{\pm}30.4$ ng/mL); however, IGF-1 levels in both sick and healthy preterm infants were lower than in healthy full-term infants. Among infants admitted after 8 days of life, serum IGF-1 levels were higher in infants who gained weight ($70.8{\pm}36.2$ ng/mL) than in infants who lost weight ($13.3{\pm}19.9$ ng/mL; P<0.01); however IGF-1 levels showed no difference between gender or method of delivery. Conclusion : The study showed lower IGF-l levels in preterm infants than in full-term infants. Additionally, the IGF-l level in infants with weight loss was lower than in infants with weight gain. These results indicate that serum IGF-1 is associated with gestational age and postnatal growth.

• Clinical and Experimental Pediatrics
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• v.52 no.2
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• pp.205-212
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• 2009

Purpose : Chronic urticaria is a disorder characterized by the appearance of wheals for more than 6 weeks; in most cases, the etiology is unknown. This study was aimed to discover the clinical aspects, the etiologic factors, and the course of chronic urticaria. Methods : 51 children who were diagnosed with chronic urticaria in the past 4 years, and who had had follow-ups more than 6 months after diagnosis in the pediatric department of Soonchunhyang University Hospital in Bucheon, were enrolled in the study. The laboratory findings, clinical aspects, and courses were retrospectively investigated by medical record review and telephone interview. Results : The median age of children with chronic urticaria was 4 years (8 months to 16 years) and the ratio of male to female was 1.4:1. Of the total, 39.2% of patients had a history of atopy. Angioedema occurred concurrently with urticaria in 11.8% of patients, and dermographism was seen in 41.2%. Results of thyroid function tests were normal and thyroid autoantibodies were absent in all cases. Regarding etiology, most cases (74.5%) were forms of idiopathic urticaria. Urticaria was induced by physical factors in 19.6% of patients. Open challenge tests revealed that 3 patients were allergic to food additives (glutamate 2, glutamate, and sulfite 1). In this study, most of the patients reported good response after medication of 1st- or 2nd-generation antihistamines alone. Follow-up at 6 months revealed that 70.6% of patients had experienced remission, and 84.8% of children who had follow-up at 1 year presented remission. Conclusion : Chronic urticaria in most patients was idiopathic. Remission occurred within 1 year of diagnosis, in most cases so chronic urticaria in children seems to have good prognosis.