• Clinical and Experimental Pediatrics
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• v.45 no.2
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• pp.192-198
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• 2002

Purpose : It is important to select and use kinds of Inhaler appropriate to the age of the patient and severity of symptoms. Several kinds of Inhaler have been developed and recommended according to each inhaler's resistance and usage method. We evaluated the usable age of 4 inhalers (turbulent flow inhalers, multi unit dose inhalers, breath actuated MDI, and autohalers) by measuring peak inspiratory flow(PIF) using $In-Check^{(TM)}$ Inspiratory Flow Meter. Methods : Ninety three patients aged from 3 to 7 years(mean $57{\pm}12.9$ mo.) who had admitted to CHA hospital from July 2000 to April 2001 were enrolled. Study patients were divided into 4 groups according to age : 3-4 years of age(group A, n=31), 4-5 years of age(group B, n=32), 5-6 years of age(group C, n=18), and 6-7 years of age(group D, n=12). Results : Out of total 93 patients, 23(71%), 27(84%), 17(94%) patients of each group A, B, C and all 12 patients of group D could use the 4 types of inhaler through adequated education. In all four groups, height, body weight and age were significantly correlated(P<0.05). Usable age, height and weight of children who can use turbulent flow inhaler(TFI) were each 8 year 7 month old, 144 cm, 32.0 kg, and those of multi unit dose inhaler(MUD) were 2 year 10 month old, 92 cm, 12.0 kg. Also that of breath-actuated MDI were 1 yr 8 months, 83 cm, 8.5 kg and that of autohaler were 2 yr 8 months, 91 cm, 11.0 kg Conclusion : We concluded that the ability to use inhalers correlated with height, weight and age of the patients. Multi unit dose inhalers, Breath actuated MDI and Autohaler are useful after 3 years of age through adequate education.

• Clinical and Experimental Pediatrics
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• v.45 no.4
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• pp.439-448
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• 2002

Purpose : The aim of this study is to determine and compare the effects of adjunctive therapy with different doses of recombinant human granulocyte-colony stimulating factor(rhG-CSF) on reversing sepsis-associated neonatal neutropenia, and their survival rate in a group I/II-type trial. Methods : RhG-CSF was injected subcutaneously to 10 septic-neutropenic neonates with doses of $10{\mu}g/kg$ from Oct. 1995 to Sep. 1996, and was administered to another 12 septic-neutropenic neonates with doses of $5{\mu}g/kg$ from Oct. 1996 to Sep. 1997. Neutrophilic responses and the outcomes of both groups were compared. Results : In the rhG-CSF $10{\mu}g/kg$ treated group and in the $5{\mu}g/kg$ treated group, the absolute neutrophil count(ANC) was $1,065{\pm}89$($mean{\pm}SEM$) and $1,053{\pm}131$, respectively. The only difference between the two groups was the peak ANC at 48 hours. Eight patients from the remaining nine of rhG-CSF $10{\mu}g/kg$ treated group(88.9％) and ten in $5{\mu}g/kg$ treated group(83.3％) survived the sepsis and were discharged without any problems. Conclusions : RhG-CSF can increase the neutrophil count in critically ill septic neutropenic neonats. The survival rate of both groups were up to 90％. This finding suggests that both doses of rhG-CSF may be effective in a therapeutically useful time frame to treat septic neonates with neonatal neutropenia attributable to bone marrow supression or neutrophil consumption.

• Clinical and Experimental Pediatrics
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• v.45 no.2
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• pp.214-222
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• 2002

Purpose : The aim of this study was to evaluate myocardial injury in children treated with adriamycin by echocardiography, which is non-invasive and safe measurement for children. Methods : Left ventricular dimensions, wall stress, and contractile function were determined by echocardiographic methods in 17 patient recepients with adriamycin chemotherapy at rest(group 1) and during stress(group 2). Twenty age-matched normal subjects were established as control group. Results : End-diastolic dimension was decreased in both groups(group 1; $92{\pm}7%$ of normal, group 2; $87{\pm}8%$ of normal, P<0.05). Left ventricular end diastolic volume and wall mass were also decreased in both groups(group 1; $96{\pm}12mL/m^2$ and $145{\pm}18g/m^2$, group 2; $87{\pm}8mL/m^2$ and $137{\pm}16g/m^2$, respectively, P<0.05 and P<0.05) and group 2 showed lower values than group 1. Meridional end systolic stress(ESSm) was increased in both groups but there was no significant difference between the two groups(group 1; $52.6{\pm}6.2g/cm^2$, group 2; $63.5{\pm}8.5g/cm^2$, P<0.05, normal value $45.7{\pm}3.5g/cm^2$). The load-independent relation of rate-corrected circumferential fiber shortening velocity(Vcfc) to ESSm has a significant abnormal change in 7 out of 17(41%) in group 1 and 12 out of 17(71%) in group 2. Conclusion : The load-dependent systolic index, such as fractional shortening, may fail to show abnormality because of the compensatory changes in preload and afterload which can mask the impaired contractility. Therefore, systolic performance also should be monitored by a load-indepedent contractility index such as slope value of the end-systolic pressure-dimension relation and the position of the left ventricular stress-fiber shortening velocity after exercise.

• 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.554-560
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• 2003

Purpose : To investigate the teratogenic effect of gamma-ray on the heart of chick embryo. Methods : 50 rad, 100 rad, 150 rad, 200 rad, 250 rad, and 300 rad of gamma-ray were used to irradiate three days old chick embryos. The control group was not irradiated. After three weeks, the embryos were sacrificed and examined for cardiovascular malformation. Results : The survival rate of the gamma-ray irradiated group was significantly lower than that of the control group(33.3-63.3% vs 76.4%, P=0.001). The cardiac malformation rate of the experimental group was 11.0%. In the control group, no congenital cardiac malformations were observed. The experimental groups had a significantly higher malformation rate(P=0.001). The types of malformation were ventricular septal defect, tricuspid atresia, Ebstein anomaly and aortic arch anomaly. In the gamma-ray irradiated group, the cardiac malformations were : 14 small ventricular septal defects (VSDs), five large VSDs, two tricuspid atresias, and one Ebstein anomaly. The higher the dose of radiation applied, the higher the incidence of cardiac malformation was noted. Conclusion : Gamma-ray irradiation of 3 days old chick embryos increased the rate of death and the rate of cardiac malformation significantly.

• Clinical and Experimental Pediatrics
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• v.46 no.4
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• pp.363-369
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• 2003

Purpose : Pharmacologic provocation test of growth hormone(GH) is a non-physiologic method and has several limitations for diagnosing growth hormone(GH) deficiency. Spontaneous GH release studies could be important in understanding the pathophysiology of children with poor growth but normal responses to GH provocation tests. Also, the relationship between nocturnal GH secretions and sleep patterns in short stature children is poorly understood. The aim of this study is to determine whether there are differences in sleep patterns and nocturnal GH secretory profiles between idiopathic short stature children and a normal stature group. Methods : Spontaneous nocturnal GH secretions and sleep patterns were evaluated in 12 prepubertal idiopathic short stature children with normal responses to provocation tests and 9 normal stature controls. Blood samples were taken every 30 minutes from 22:00-06:30 and sleep patterns were analyzed by polysomnography. Results : The mean GH level during sleep was significantly lower in short stature children than in controls. The peak GH level after sleep, coincident with the first slow wave sleep, was lower in the short stature group. The slow wave sleep times of short stature children were decreased compared with those of normal subjects. Conclusion : These results suggest that overnight serial GH sampling is helpful to identify short stature children with subnormal GH secretions, and sleep structure differences may be associated with decreased overnight GH secretions in short stature children.

• Clinical and Experimental Pediatrics
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• v.46 no.4
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• pp.345-350
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• 2003

Purpose : Bacterial meningitis is a serious infection of childhood associated with a significant morbidity and mortality. Repeated cerebrospinal fluid(CSF) examination is a useful prognostic indicator and a delayed sterilization is associated with a higher incidence of neurologic abnormalities. In this study we tried to determine the prognostic value of repeated CSF latex agglutination testing. Methods : We retrospectively evaluated 19 patients admitted to Ewha Womans University Mokdong Hospital for bacterial meningitis from January 1997 to June 2002. Bacterial meningitis was confirmed by a positive CSF culture and a positive CSF latex agglutination test. Repeated CSF examinations were done at three, seven, 14, 21 and 28 days after antibiotics therapy. Neuroradiologic studies were performed. Results : The mean age was $10.6{\pm}12.3months$(range; two to 33 months). The male to female ratio was 2.8 : 1. The causative organisms were Haemophilus influenzae type b 57.9%, Group B Streptococcus 21.1%, Streptococcus pneumoniae 15.7% and Escherichia coli 5.3%. Three days after the initiation of antibiotics therapy, repeated CSF latex agglutination tests persisted as positive in nine (47.4%) out of 19 cases, but all CSF cultures became negative. In those cases with negative latex agglutination tests three days after antibiotics therapy, neuroradiologic findings were completely normal. But, in cases with positive latex agglutination tests three days after antibiotics therapy, neuroradiologic abnormalities such as cerebral infarction, encephalomalasia occurred in 44.4%. Conclusion : Repeated CSF latex agglutination testing was valuable as a prognostic factor in bacterial meningitis. Neuroradiologic abnormalities may occur in cases with delayed clearance of CSF latex agglutination tests more often than in cases with negative latex agglutination tests three days after antibiotics therapy.

• Clinical and Experimental Pediatrics
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• v.46 no.12
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• pp.1230-1234
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• 2003

Purpose : The aim of this study is to evaluate the value of lung biopsies for the management of children with lung disease. Methods : We retrospectively reviewed 19 lung biopsies done at Asan Medical Center, Seoul between 1993 and 2001. Data gathered included demographic information, underlying conditions, diagnosis before biopsy, final diagnosis, change in therapy, morbidity and mortality. Results : Nineteen patients underwent lung biopsy. Among them, 13 patients were male and six patients were female; the median age was 3.6 years(0.8 to 8.6 years). Twelve patients underwent open lung biopsies and seven patients had thoracoscopic biopsies. The overall diagnosis rate was 95 %. The most common diagnosis was interstitial lung disease(12 patients, 64%) and infection was detected in four patients(21%). The biopsy-proven bronchiolitis obliterance was confirmed in two of seven patients suspected by CT findings. Specific treatment was changed after biopsy in 16 patients (85%). The morbidity & overall mortality rates of the patients were 5%(one patient) and 21%(four patients) respectively. Only one complication was seen : empyema. The causes of death were acute respiratory distress syndrome(one patient), respiratory failure(two patients), and septicemia(one patient). Conclusion : The lung biopsy is a safe procedure and it contributes to more accurate diagnosis and proper management of pediatric lung diseases. We recommend lung biopsies should be considered more positively in the diagnosis of pediatric lung diseases.

• Clinical and Experimental Pediatrics
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• v.46 no.12
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• pp.1186-1193
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• 2003

Purpose : Fragile sites are points on chromosomes which tend to break non-randomly when exposed to specific chemical agents or conditions of tissue culture. The chromosomal break induced by the antineoplastic drug, 1-${\beta}$-D-arabinofuranosyl-cytosine(Ara-c), was investigated to study the laboratory conditions in which the incidence of chromosomal break could be enhanced. Besides, the fragile sites induced by Ara-C were investigated and compared to the already known locations of the specific chromosomal alterations observed in specific neoplasms. Methods : T-lymphocytes from theree normal males and three females were cultured for 48 hours. Cells from each individual were exposed to the Ara-C for an additional 24 hours. After the caffeine was added during the last six hours culture, the metaphase chromosomes were prepared following the conventional method. A site was considered fragile if it was found to break two or more per 100 chromosomal breaks in more than four of six individuals tested. Results : Ara-C induced 252.1 chromosomal breaks per 100 mitotic cells and this result was significantly higher than that of the control, which induced 25.2 breaks(P<0.05). The incidence of the chromosomal break by Ara-C was higher, if cultured in the MEM-FA, which has no folic acid, than in the RPMI 1640 which contains enough folic acid(P<0.05). The most common break site by Ara-C was 3p14.2(FRA3B). There were 20 fragile sites induced by Ara-C. Among these 20 fragile sites, seven coincided with the locations of the mapped oncogenes, JUN, SKI, REL, N-MYC, FHIT, MET, ETS-1, and FOS. Conclusion : S phase specific chemotherapeutic agent, Ara-C, induced the expression of the chromosomal fragile sites effectively using the T-lymphocyte in vitro. Some of the fragile sites by Ara-C highly coincided with the oncogenes and neoplasm specific chromosome breakpoints. In this regard, the fragile sites reported here could provide the unknown neoplasm related chromosomal alternation points.