• Clinical and Experimental Pediatrics
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• v.52 no.2
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• pp.234-241
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• 2009

Purpose : We investigated the relationship between thyroid hormone and serum tumor necrosis factor (TNF-${\alpha}$), interleukin (IL-6) and N-terminal fragment of pro-brain natriuretic peptide (NT-proBNP) in patients with Kawasaki disease (KD). Methods : Serum levels of thyroid hormone, TNF-${\alpha}$, IL-6, and NT-proBNP were measured in 52 KD patients in the acute and subacute phase and 10 patients with acute febrile illness (control group). TNF-${\alpha}$ and IL-6 were determined by sandwich enzyme-linked immunosorbent assay (ELISA). Echocardiography was performed to detect coronary artery lesions (CAL) in KD patients. Results : Low $T_3$ syndrome occurred in 63.5% of KD patients. $T_3$ in the acute phase of KD was lower than that in the control. In KD patients, $T_3$ was lowered in the acute phase and elevated in the subacute phase, whereas TNF-${\alpha}$, IL-6 and NT-proBNP were elevated in the acute phase and decreased in the subacute phase. NT-proBNP, and IL-6 were higher in patients with low $T_3$ than in those with normal $T_3$. In addition, $T_3$ inversely correlated with IL-6 and NT-proBNP. Of the 4 patients with CAL, 3 had very low $T_3$. Compared with intravenous immunoglobulin (IVIG)-responsive patients, IVIG-resistant patients had lower $T_3$ and higher IL-6 and NT-proBNP. Conclusion : $T_3$ decreases in the acute phase of KD and normalizes in the subacute phase without thyroid hormone replacement. Low $T_3$ may be partially induced by IL-6 rather than TNF-${\alpha}$, and is strongly associated with high NT-proBNP. $T_3$ in KD may be used for the differential diagnosis, monitoring the activity of the disease, and predicting the severity of inflammation.

• Pediatric Infection and Vaccine
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• v.7 no.1
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• pp.143-151
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• 2000

Purpose : The accuracy of bacteriologic diagnosis of beta-hemolytic streptococcal pharyngotonsillitis depends on the degree of carrier rate in that area where the throat swabs are obtained and the evaluation of serological T typing as an epidemiologic marker is important to understand epidemiology of streptococcal infection. The purpose of this study is to know the carrier rates of group A streptococcus in normal children form four different areas and to find out the epidemiologic characteristic in distribution of the serotypes for 3 years. Method : Throat swabs were obtained from the tonsillar fossa of normal school children in four different areas(Uljin, Seoul, Osan, Kunsan) from March to May 1996, in Uljin in April 1997, and in Uljin in April 1998. The samples were plated on a 5% sheep blood agar plate and incubated overnight at $37^{\circ}C$ before examination for the presence of beta-hemolytic colonies. All isolated beta-hemolytic streptococcus were grouped and serotyped by T agglutination. Results : The carrier rate of beta-hemolytic streptococci and group A streptococci in 1996 were 27.6%, 18.6% at Uljin; 16.4%, 2.7% at Seoul; 33.0%, 26.0% at Osan; 20.0%, 12.3% at Kunsan, respectively. Among 1,192 normal school children from 4 different areas, we obtained 179 strains of group A streptococci. Fifty two percent of the strains were typable by T agglutination in 1996. Common T-type in 1996 were NT, T1, T3, T2 at Uljin; T12, T25 at Seoul; NT, T6, T28 at Osan; T25, T4, NT, T5 at Kunsan, in decreasing order, respectively. At Uljin, T1, T3, T25 accounted for 69% of strains in 1996, T1, T12, T25 accounted for 70% in 1997, and T12, T4 accounted for 88% in 1998. Conclusion : Higher carrier rates were found in Uljin and Osan, where there are a lower population density with scanty of medical facilities compared with another areas. We supposed that low carrier rates is likely to be related to antibiotic abuse or some epidemiologic factor. The periodic and seasonal serotyping analysis is important in monitoring and understanding the epidemiologic patterns of group A streptococci.

• Journal of Gastric Cancer
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• v.10 no.1
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• pp.1-4
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• 2010

Purpose: Gastric epithelial dysplasia (GED) was defined as "unequivocally neoplastic epithelium that may be associated with or give rise to invasive adenocarcinoma" and GED also represents a direct precursor of intestinal type adenocarcinoma of the stomach. The recommended treatment guidelines for GED in the medical literature are endoscopic mucosal resection (EMR) or surgery for high grade dysplasia (HGD) and annual endoscopic surveillance with biopsy for low grade dysplasia (LGD) The aim of this study was to determine the treatment plan for GED that is diagnosed by endoscopic biopsy. Materials and Methods: We enrolled 148 patients who were treated by endoscopic mucosal resection (EMR) or endoscopiccsubmucosal dissection (ESD) for GED: there were 63 patients with HGD and 85 patients with LGD and all of them were diagnosed by endoscopic biopsy from January 2006 to December 2008. The results of the final histopathologic reports after EMR or ESD were compared with the results of the endoscopic biopsies. Results: The final histopathologic results of the 148 patients with GED showed 49 (33.1%) patients with adenocarcinoma, 40 (27.0%) patients with HGD and 59 (39.9%) patients with LGD. Among the 63 patients with HGD, 34 (54.0%) patients had adenocarcinoma, 20 (31.7%) patients had HGD and 9 (14.3%) patients had LGD. For the 85 patients with LGD, 15 (17.6%) patients had adenocarcinoma, 20 (23.5%) patients had HGD and 50 (58.8%) patients had LGD Conclusion: Complete resection, including EMR or ESD, is needed for patients with GED diagnosed by endoscopic biopsy and they have HGD. For patients with LGD, EMR or ESD may be needed in addition to endoscopic surveillance with biopsy for making the correct diagnosis and proper treatment because of the possibility of adenocarcinoma.

• Journal of agricultural medicine and community health
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• v.34 no.1
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• pp.87-100
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• 2009

Objectives: This study was conducted to suggest the way to improve the tobacco control program of public health centers in Korea. Methods: A survey with a self-administered questionnaire was conducted among 246 persons in charge of tobacco control work in public health centers nationwide in December, 2006. Frequency analysis was performed with a final sample of 212 respondents with SPSS 12.0 for Windows. Results: The duration of engagement in tobacco control work was less than 3 years among 86.7% of respondents, and 87.3% of respondents had other duties besides tobacco control. Almost all public health centers conducted a campaign with posters or leaflets, and smoking prevention education among adolescents. The actual priority for programs was based on the community diagnosis in only 33.5% of the cases. Only 1.9% of respondents complained lack of budget, on the other hand, 44.7% of respondents appealed insufficient number of personnel. The route of knowledge and skill was largely dependent on self-learning or information from colleague. Collaboration with other related department was done well in 39.5% of the cases. The majority of respondents was satisfied with the general support from central government. Conclusions: To improve the tobacco control program of public health centers, it is needed the reinforcement of capacity and specialty among personnel, priority setting and performance of programs based on the scientific evidence, induction of community participation, utilization of community human resources, development of education and training course for practical skill, effective networking among departments.

• Journal of Genetic Medicine
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• v.5 no.2
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• pp.139-144
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• 2008

We report on two cases of pericentric inversion of X chromosome. The cases were found in a 40-year-old man with azoospermia and in a family of a 38-year-old pregnant woman. The first case with 46,Y,inv(X)(p22.1q27) had concentrations of LH, prolactin, estradiol, and testosterone that were within normal ranges; however, FSH levels were elevated. Testis biopsy revealed maturation arrest at the primary and secondary spermatocytes without spermatozoa. There were no microdeletions in the 6 loci of chromosome Y. For the second case, the cytogenetic study of thepregnant woman referring for advanced maternal age and a family history of inversion X chromosome was 46,X,inv(X)(p22.11q27.2). The karyotype of her fetus was 46,X,inv(X)(p22.1q27). Among other family members, the karyotypes of an older sister in pregnancy and her fetus were 46,X,inv(X)(p22.11q27.2), and 46,Y,?inv(X), respectively. The proband's father was 46,Y,inv(X)(p22.11q27.2). All carriers in the family discussed above were fertile and phenotypically normal. In addition, the ratio of inactivation of inv(X) by RBG-banding was discordant between the two sisters, with the older sister having only 4.1% of cells carrying inactivated inv(X) while the proband had a 69.5% incidence of late replicating inv(X). Therefore, we suggest that the cause of azoospermia in the first case might be related to inversion X chromosome with positional effect. Also, the family of the second case showing normal phenotype of the balanced inv(X) might be not affected any positional effect of genes.

• Clinical and Experimental Pediatrics
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• v.46 no.11
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• pp.1067-1072
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• 2003

Purpose : Non-accidental intracranial hemorrhage in children is not low in incidence and results in high mortality and serious sequelae. So, the authors have researched the distribution of sex and age, causes, symptoms and signs, hemorrhagic types, mortality rate and sequelae of the patients hospitalized with non-accidental intracranial hemorrhage at Hallym University Sacred Heart Hospital. Methods : The medical records of twenty patients, aged 15 or younger, and excluding neonatal patients, were analyzed retrospectively. The patients in this study were admitted with non-accidental intracranial hemorrhage from January 1999 to June 2002. Results : Of the twenty cases, the ratio of male to female was 1 : 0.8. The patients aged one or less and between 11 and 15 were discovered to be the most frequent cases. Shaken baby syndrome and arteriovenous malformation were found to be the most frequent causes. Seizure was most frequently found to be a symptom and a sign. Hemorrhagic type was classified into subdural hemorrhage eight, intracerebral hemorrhage five. There were three mortal cases. Twelve surviving patients, excluding five not-followed ones, were reclassified into six cases of complete recovery and six of sequalae. Conclusion : Non-accidental intracranial hemorrhage in children is not low in incidence, with a high mortality rate and a high incidence of serious sequelae after survival. Consequently, early diagnosis and appropriate treatment are required. In addition, appropriate rehabilitation after treatment is needed because the high survival rate due to advanced medical treatment results in an increasing number of neurologic sequelae.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.17 no.3
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• pp.92-95
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• 2017

Fabry disease (FD) is an X-linked lysosomal storage disorder caused by an ${\alpha}$-galactosidase A (GLA, MIM 300644) enzyme deficiency due to pathogenic variants in the ${\alpha}$-galactosidase A gene (GLA). The disease leads to accumulation of globotriaosylceramide (Gb3) and related glycophospholipids affecting nearly all major organ systems, with the primary sites damaged by Gb3 including renal glomeruli, myocardium, neurons of the dorsal ganglion and autonomic nervous system, and vascular endothelial and smooth muscle. Progressive deposition in these organ systems present with various clinical manifestations including acroparesthesia, renal failure and heart failure. Here, we report a Chinese male diagnosed with Fabry disease in his late $4^{th}$ decades showing improvement of acroparesthesia during enzyme replacement therapy (ERT). A 48-year-old Chinese man who presented with chronic recurrent severe burning pain in his fingers and toes since the age of 10, with worse involvement of the former visited to our clinic for further evaluation. His medical history included a transient ischemic attack aged 40 and diagnosed with stage 4-5 chronic kidney disease aged 47. In the family history, the patient's brother was found to be have Fabry disease 1 month before his visit. Except for his brother, all other members of the family are healthy. Based on his medical history and family history, he was strongly suspicious for Fabry disease. He was found to have a galactose-alpha-1,3-galactose level 4.96 (Reference range, 42.5-67.9) suggestive of Fabry disease. The followed sequencing of GLA coding region in our patient revealed hemizyosity for the mutation c.988C>T (Q330X) in Exon 7. Since ERT start, he showed significant improvement in his symptoms of burning sensation of fingers and toes. On the contrary, due to deteriorating kidney function even with ERT, he is considered for kidney transplantation. Despite of diagnostic delay until late 4th decades, ERT showed a potential improvement of acroparesthesia in our patient. However, late start of ERT can lead to poor outcome in multiorgan function. Therefore, early diagnosis with high index of suspicion followed by continuous ERT with regular monitoring have an impact on quality of life in Fabry disease.

• Childhood Kidney Diseases
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• v.1 no.1
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• pp.46-52
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• 1997

Urinary tract infection (UTI) in children has been known to be a cause of renal damage, leading to scar formation, hypertension and renal failure. And vesico-ureteral reflex (VUR), frequently accompanying UTI in young children, has been incriminated as the main factor causing scar formation. This retrospective study has been undertaken to see the relationship among UTI, VUR and renal scar formation. Study population consisted of 291 children (boy 134, girl 42) with UTI, who have been admitted to the Pediatric Department of Kyungpook University Hospital during 6 1/2 year period from January 1990 to June 1996. VUR was diagnosed by VCUG and renal scar by ultrasonogram, DMSA scan (or DMSA SPECT) and IVP. The following result were obtained. Sexual difference showed male predominance (male to female, 134:42) below 1 year of age, and female predominance (male to female, 11:35) over 5 years of age were rioted. VUR has been found in 64 children (22%) and the degree of reflux, classfied by the method proposed by 'International Reflux Study in Children', were as follows ; Grade I : 4.0%, Grade II : 3.0%, Grade III : 2.7%, Grade IV : 5.8% and Grade V : 6.2%. There was no sexual difference E.coli was the most predominant infecting agent occurring in 167 children (57%), and end-stage renal failure was diagnosed at the time of first admission in 5 children with Grade V VUR. Renal scar has been noted in 49 out of 582 kidneys (8.4%), and the incidence of scar foramation according to the degree of VUR were as follow ; Grade 0 (No reflux) : 1.2%, Grade I : 6.7%, Grade II 27.3%, Grade III 29.4%, Grade IV : 57.1%, and Grade V : 100%. In summary, present study shows that renal scar formation in UTI has close correlation with the severity of VUR occurring more frequently in severe reflux, so that early diagnosis and proper treatment of UTI and VUR is of paramount importance in preventing renal damage in children with UTI.

• Pediatric Infection and Vaccine
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• v.6 no.1
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• pp.93-100
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• 1999

Purpose : For evaluation of acute Mycoplasma pneumoniae(M. pneumoniae) pneumonia in children, we have studied the Mycoplasma indirect particle agglutination test, cold hemagglutinin test, ESR, CRP, and total white blood cell counts and it's differential count retrospectively. Methods : The total numbers of patients whom compatible with diagnostic criteria of acute M. pneumoniae peumonia were 56 cases from Jan. to Dec. 1997. The diagnostic criteria were 1) onset of fever(${\geq}38.0^{\circ}C$) and coughing were within 7 days, 2) rhonchi and/or role was audible on chest, 3) pneumonic infiltration on chest X-ray, and 4) M. pneumoniae indirect particle agglutination test titer was higher than 1:640, or initial titer was less than 1:640 but increased more than 4 folds after week. We classified the enrolled patients according to initial antibody titer, such as soup A(${\leq}1:640$) and group B(${\geq}1:320$). We compared group A and B by demographic findings, clinical symptoms and signs, and laboratory findings. Results : 1) The male and female sex ratio was 1:1.4, and average onset age was $5.8{\pm}2.96$ years. 2) The average body temperature on admission was $38.5{\pm}0.1^{\circ}C$ and productive coughing was noticed in 52 cases(93%). 3) The average total white cell counts were $10,470{\pm}877.0/mm^3$ in group B patients, which was significantly higher compared to $7,761{\pm}508.5/mm^3$ in group A(p<0.014). 4) The average value of ESR and CRP were within normal range in both group. 5) The most common site of pneumonic infiltration was right lower lobe of lung in both groups. 6) There were no correlation between antibody titer and cold hemagglutinine titer in patients and cold hemagglutination titer were less than 1:64 in 25 cases(45%). Conclusion : The clinical manifestations of pneumonia, findings of chest x-ray, and indirect particle agglutination test were useful on diagnosis of M. pneumoniae pnumonia onset within 7 days, but cold hemagglutinin test was a little diagnostic meaning.

• Clinical and Experimental Pediatrics
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• v.49 no.7
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• pp.790-795
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• 2006

Purpose : This study was performed to compare the therapeutic effects according to duration of medium-dose aspirin(50-60 mg/kg/day) therapy at the acute stage of Kawasaki disease(KD). Methods : Total 87 patients with KD were enrolled in this study. We performed retrospective analysis of clinical characteristics and echocardiographic findings based on medical records. Patient were randomly divided into 2 groups according to the duration of aspirin therapy at the acute stage of KD. Long-term group(LG, n=55) was administered medium-dose aspirin for 2 weeks after diagnosis of KD, and short-term group(SG, n=32) for 48 hours after intravenous immunoglobulin(IVIG) administration. The parameters of therapeutic effects were duration of fever after IVIG administration, incidence of unresponsive patients to single administration of IVIG, and development of transient dilatation or aneurysm of coronary arteries. Results : There was no significant difference in the duration of fever after IVIG between the both group(LG $1.7{\pm}1.1$ days, SG $1.8{\pm}1.1$ days; P=0.588). The incidences of unresponsive patient to the single administration of IVIG were 5.5 percent, 6.3 percent in the each group. Transient dilatation of coronary arteries occurred at 18.2 percent(10/55) in the LG, and 15.6 percent(5/32) in the SG(P=0.761). Prevalence of coronary aneurysm after subacute stage were 7.3 percent(4/55) in the LG, and 9.4 percent(3/32) in the SG(P=0.728). Conclusion : There was no significant difference in the therapeutic effects between long-term(2 weeks) and short-term(48 hours) administration of medium-dose aspirin at the acute stage of KD.