Interactions Between Innate Immunity Genes and Early-Life Risk Factors in Allergic Rhinitis

PURPOSE: Allergic rhinitis (AR) is a common chronic disease. Many factors could affect the development of AR. We investigated early-life factors, such as delivery mode, feeding method, and use of antibiotics during infancy, which could affect the development of AR. In addition, how interactions between these factors and innate gene polymorphisms influence the development of AR was investigated. METHODS: A cross-sectional study of 1,828 children aged 9-12 years was conducted. Three early-life factors and AR were assessed by a questionnaire. Skin prick tests were done. Polymorphisms of TLR4 (rs1927911) and CD14 (rs2569190) were genotyped. RESULTS: Use of antibiotics during infancy increased the risk of AR (aOR [95% CI] 1.511 [1.222-2.037]) and atopic AR (aOR [95% CI], 1.565 [1.078-2.272]). There were synergistic interactions between caesarean delivery, formula feeding, and use of antibiotics in the rate of atopic AR (aOR [95% CI], 3.038 [1.256-7.347]). Additional analyses revealed that the risk for the development of AR or atopic AR subjects with the TLR4 CC genotype were highest when all the 3 early-life factors were present (aOR [95% CI], 5.127 [1.265-20.780] for AR; 6.078 [1.499-24.649] for atopic AR). In addition, the risk for the development of AR or atopic AR in subjects with the CD14 TT genotype were highest when all the 3 early-life factors were present (aOR [95% CI], 5.960 [1.421-15.002] for AR; 6.714 [1.440-31.312] for atopic AR). CONCLUSIONS: Delivery mode, feeding method, and use of antibiotics during infancy appeared to have synergistic interactions in the development of AR. Gene-environment interactions between polymorphism of innate genes and early- life risk factors might affect the development of AR.

Prevalence and Clinical Features of Wolff-Parkinson-White (WPW)-Type Ventricular Preexcitation Syndrome in Children

PURPOSE: This study was conducted to establish the prevalence and clinical features of WPW- type ventricular preexcitation syndrome in children. METHODS: Eighteen patients were discovered by the mass heart disease screening using ECG on 47,691 elementary school children in Kwangju from September 1992 to December 1994 (group 1), and 14 patients were diagnosed at the Department of Pediatrics, Chonnam University Hospital from 1991 to August 1995 (group 2). We retrospectively reviewed patients' medical records. RESULTS: The prevalence rate was 4 per 10,000 elementary school children and there was no sexual difference. All patients in group 1 were asymptomatic, but 5 patients in group 2 had symptoms related to tachyarrhythmias. Three patients with WPW syndrome and paroxysmal supraventricular tachycardia who underwent radiofrequency catheter ablation have been well without recurrence of tachyarrhythmias during the follow-up period of 29-45 months. Two cases in group 2 had structural cardiac lesion (ventricular septal defect in 1, mitral valve prolapse in 1). CONCLUSION: The prevalence rate of Koreans was lower than that of foreigners. The radiofrequency catheter ablation seemed to be relatively safe and effective for the treatment of atrioventricular reentrant tachycardia utilizing accessory pathway in childhood patients if this procedure is technically feasible. However, continuous longitudinal studies are needed to evaluate the long-term natural history and prognosis of the WPW-type ventricular preexcitation syndrome.

The study of phonetic change after the orthognathic surgery for the patient of mandibular prognathism

No abstract available.

Clinical Study on Supraventricular Tachycardia in Children

PURPOSE: Supraventricular tachycardia(SVT) is the most common symptomatic tachyarrhythmia in pediatric age group. Causes, types, and clinical characteristics of this arrhythmia in children are different from those in adults. This study was undertaken to investigate the clinical features of SVT in children. METHODS: A retrospective study was done on 36 patients with SVT, who were diagnosed at Department of Pediatrics, Chonnam National University Hospital from January, 1991 through August, 1999. The types of SVT, age of onset, associated cardiovascular abnormalities, and response to treatment were analyzed. RESULTS: Atrioventricular reentrant tachycardia(AVRT) using an accessory pathway was found in 63.9%. Primary atrial tachycardia and AV nodal reentrant tachycardia(AVNRT) were noted in 25.0% and in 5.6%, respectively. SVT occurred in over 50% of the total patients before 1 year of age : during the neonatal period, 22.2% ; during the infant period, 33.3%. Primary atrial tachycardia usually developed in the infant period. AVRT mostly occurred in the infant period, appeared to decline in the preschool period and then to increase again in the school age. AVNRT occurred only after 1 year of age. Associated cardiovascular abnormalities were found in 22.2%. Successful conversion to sinus rhythm was possible in 91.7%. Initially, normal sinus rhythm was achieved in 55.9% of the 34 children treated with adenosine. Radiofrequency catheter ablation(RFCA) was successfully performed in all 10 patients without complication and there was no recurrence of SVT during the follow-up period. CONCLUSION: We conclude that the mechanism of SVT appeared to have an age-dependent distribution, AVRT was the most common mechanism and adenosine was effective drug for restoration of normal sinus rhythm in the initial treatment of SVT. RFCA seemed to be effective and safe method for the treatment of SVT in children.

A Case of Intestinal Malrotation Complicated by Midgut Volvulus: Diagnosis with Abdominal CT Scan

Intestinal malrotation is a constellation of a wide spectrum of embryologic failures of rotation and fixation of the gut, resulting in a narrow-based attachment of the mesentery and the presence of abnormal peritoneal (Ladd's) band. These abnormalities predispose to midgut volvulus, intestinal obstruction and internal herniation. We experienced a case of intestinal malrotation complicated by midgut volvulus in a 4-year-old girl who presente with abdominal pain, abdominal distension and melena. The diagnosis was made by abdominal CT scan, which showed "whirl sign." The diagnosis was confirmed by surgery. A brief review of literature ensues.

A Case of Intestinal Malrotation Complicated by Midgut Volvulus: Diagnosis with Abdominal CT Scan

Intestinal malrotation is a constellation of a wide spectrum of embryologic failures of rotation and fixation of the gut, resulting in a narrow-based attachment of the mesentery and the presence of abnormal peritoneal (Ladd's) band. These abnormalities predispose to midgut volvulus, intestinal obstruction and internal herniation. We experienced a case of intestinal malrotation complicated by midgut volvulus in a 4-year-old girl who presente with abdominal pain, abdominal distension and melena. The diagnosis was made by abdominal CT scan, which showed "whirl sign." The diagnosis was confirmed by surgery. A brief review of literature ensues.

A Case of Primary Lymphatic Dysplasia Presented with Lymphedema and Chylothorax / 소아알레르기및호흡기학회지

Primary lymphatic dysplasia is a congenital maldevelopment of the lymphatic system, interfering with its function and causing an effusion of chyle or lymph into the limbs and pleural or peritoneal cavity. We describe a case of primary lymphatic dysplasia in a 34-months old girl, presented with lymphedema at birth and refractory chylothorax. This case was treated by microlymphaticovenous anastomosis on 16 days after birth. Conservative therapy, such as total parentral nutrition with repeated thoracentesis was ineffective to control chylothorax. Ligation of the thoracic duct was performed.

A Study of Serum Cytokines in the Patients with Chronic Hepatitis B Virus Infection / 대한소아소화기영양학회지

PURPOSE: The aim of this study was to clarify the serum cytokine pattern in patients with chronic HBV infection in terms of their clinical state. METHODS: Intravenous blood samples were taken from 35 patients who were seropositive for HBsAg for at least 6 months and 7 healthy controls. Samples were initially tested for serum aminotransferases and serologic markers for hepatitis B virus by EIA. Serum levels of interleukin(IL)-2, tumor necrosis factor-alpha (TNF-alpha), interferon-gamma (IFN-gamma), IL-4, and IL-10 were measured by ELISA. RESULTS: Among 35 patients, seropositive for HBeAg was 20 and for anti-HBe was 15. The histologic diagnosis of 19 patients underwent liver biopsy were chronic persistent hepatitis (CPH) in 10 and chronic active hepatitis (CAH) in 9. Serum IL-10 level in patients seropositive for HBeAg was significantly higher than that in patients seropositive for anti-HBe (p 100 U/L. High level of IL-4 was seen in patients with normal aminotransferase levels. Conclusion: These results were thought to indicate that anti-inflammatory Th2-like cytokine (IL-10) production in chronic HBV infection is related to circulating HBeAg rather than activity of hepatitis and that Th1 cytokines seem to be associated with the increasing activity of hepatitis.

Two Cases of 2:1 Atrioventricular Block in Infants with Idiopathic Long QT Syndrome

Idiopathic long QT syndrome(LQTS) is an important cause of syncope, seizures, serious ventricular arrhythmias and sudden death in children. The abnormalities of AV conduction such as 2:1 AV block in children with LQTS is rarely reported. We reported two cases of 2:1 atrioventricualr block in infants with LQTS. Patient 1, a female newborn, revealed bradycardia(60 beats/min) and QTc of 0.65sec with 2:1 AV block at birth. She died of polymorphic ventricular tachycardia at 26days of age. Patient 2, a 2-month-old female, exhibited prolonged QT interval (QTc=0.54sec), 2:1 AV block, and intermittent Wenckebach conduction. After she received propranolol, sinus rhythm with 1:1 AV conduction and QTc of 0.5sec was seen. After a follow-up of 6 months, the infant is still doing well.

Normal ECG Standard in Preterm and Term Neonates in the First Day of Life

PURPOSE: To obtain the normal standard of the electrocardiogram(ECG) in newborn infants, METHODS: Standard 12-lead ECG recordings of 146 healthy neonates of gestational age above 30 weeks recorded in 24 hours after birth were analyzed for the following parameters: heart rate, QRS axis, PR interval, QTc interval, R and S wave amplitude. RESULTS: The mean gestational age of preterm(between 30 and 37 weeks) and term infants was 33.2 +/-2.4 and 38.4+/-3.2 weeks, and the mean birth weight was 2,168+/-371 g and 3,254+/-436 g, respectively. There was no meaningful difference between two groups in heart rate, PR interval, QTc interval, and QRS axis. Amplitudes of R waves in V1>-V6 leads and S waves in V2, V3 and V5 leads in term baby group were significantly larger than those in preterm baby group. The sum of amplitudes of R and S waves was largest in V2. The amplitude of combined R+S waves in V2 and V3 leads was significantly larger than that in V5 and V6 leads in both groups. This difference was more prominent in tbaby group. CONCLUSION: The results suggest that the increase in cardiac muscular mass and progressive right ventricular predominance are in accordance with the increase in gestational age.

Immunophenotypic Analysis of Umbilical Cord Blood Stem Cells

PURPOSE: Since umbilical cord blood (UCB), which used to be discarded, was found to be a source of enriched hematopoietic stem and progenitor cells, basic research to elucidate characteristics of UCB hematopoietic stem cells (HSCs) and its clinical application to bedside transplantation have been attempted. Moreover, stem cell transplantation (SCT) has expanded its role, not only in hematopoietic reconstitution, but in cancer therapy, stem cell expansion and gene therapy. This study was aimed to clarify the characteristics of UCB HSC comparing differences between term and preterm babies in term of 1) hematologic parameters, 2) immunophenotypic characteristics studied by flow cytometer utilizing CD34 and several other monoclonal antibodies (MoAb), and 3) hematopoietic capacity by clonogenic assays. METHODS: UCB was obtained from 18 term babies and 13 preterms after informed consents. Samples were initially tested for complete blood counts. Immunophenotypic characteristics were studied in 11 cases (preterm, 4; term, 7) by two laser FACscan plus (Becton Dickinson) with FITC-conjugated MoAb to CD3, CD4, CD5, CD8, CD10, CD16+ 56, CD19, CD33, CD34, CD38, CD71, Thy-1, and HLA-DR. Clonogenic assays were performed by methylcellulose method. RESULTS: The mean hematologic parameters for all groups were : white blood cell, 12.7x103/microliter; hemoglobin 14.8g/dL; platelets, 230x103/microliter. The parameters for preterms and terms were as follows : white blood cell, 10.6x103/microliter vs 13.9x103/microliter; hemoglobin 13.5g/dL vs. 15.4g/dL; platelets, 188x103/microliter vs 254x103/microliter; mononuclear cells 5.1x103/microliter vs 6.4x103/microliter, respectively. Parameters other than hemoglobin and platelet counts were not significantly different between the two groups. The colony-forming units granulocyte-macrophage (CFU-GM) count and count for all colonies identified on day 14 were 10,888+/-11,257.3/mL and 16,504+/-16,531.6/mL, respectively. However, there was no significant differences in clonogenic assays between the term and preterm groups. The percentage of CD34+ cells in mononuclear cells was 1.5+/-1.5%, with 1.0+/-0.2% for preterms and 1.8+/-1.9% for terms. The number of CD34+ cells was 5.5+/-4.1x104/mL, with 3.8+/-2.0x104/mL for preterms and 6.5+/-4.7x104/mL, respectively. These findings suggested that the percentage and number for CD34 cells and the number of CFUs be higher in term babies than in preterms, but the differences failed to meet statistical significances. As T cell markers, CD3 (pan-T cell) and CD5 (early developmental T cell) were positive in 28.5% and 32.8%, respectively. The CD4 : CD8 ratio for all was 2.2+/-0.5, with 2.3+/-0.3 for preterms and 2.1+/-0.6 for terms, respectively, tending to decrease with gestational age with transient increase when approaching to the term. CD10 and CD19 expression as markers for B cell-associated antigens were 1.8+/-1.6% and 6.5+/-4.6%, respectively. Myeloid marker CD33 was positive in 2.24%, while CD71 (transferrin receptor) in 43.7%. Thy-1 was 30.0% with peak of 63.4% at 32th gestational week. As a subpopulation study among HSCs, CD34+CD38- cells were 2.1+/-1.5%, CD34+ HLA-DR+ was present in 85.3+/-3.1%, while CD34+CD19+ cells were 1.7+/-1.6%. CONCLUSION: These results suggested that T cells in UCB were immature, that the number of CD8+ cells which are known to be implicated in graft-versus-host disease, was relatively low, that B cell expression was low, and that UCB were enriched with primitive HSCs. As UCB for preterms were not significantly different from that of terms, the UCB from preterm babies might be used as a source of HSCs. Moreover, the cell number for adequate engraftment might be inferred from calculating mononuclear cells in UCB as the mononuclear cell count had a good correlation with CFUs.

Rhabdomyolysis-induced Acute Renal Failure in a Child

Rhabdomyolysis has been reported as a cause of acute renal failure (ARF), and it can arise from traumatic and variable non-traumatic events. Rhabdomyolysis-induced ARF is very rare in children. We experienced a case of rhabdomyolysis-induced ARF in a 14-year-old boy who presented with generalized edema and oliguria following trauma, a compressed thigh by a cultivator. Laboratory tests showed marked elevation of muscle enzymes (aspatate aminotransferase, creatine kinase and lactate dehydrogenase), increased serum and urine myoglobin, and other laboratory abnormalities showing ARF. Ten days after continuous arteriovenous hemofiltration using catheterization of the femoral artery and vein, the daily urine output gradually increased and he recoverd. We report this case with brief review of literature.

Three Cases of Internal Jugular Phlebectasia

Phlebectasia is an abnormal dilatation of an isolated vein and a rare venous anomaly and is usually asymptomatic. Clinically internal jugular phlebectasia is a self limited benign condition and usually no treatment is required after initial diagnosis. So suspection of this disease and appropriate diagnostic approaches are essential to avoid unnecessary surgical intervention. We present three cases of internal jugular phlebectasia of which diagnosis was made by neck sonography and CT.

Therapeutic Efficacy of Prednisolone Withdrawal Followed by Recombinant alpha Interferon in Children with Chronic Hepatitis B / 대한소아소화기영양학회지

PURPOSE: To evaluate the efficacy of interferon alpha therapy with or without prednisolone in children with chronic hepatitis B. METHODS: Twenty-eight children (22 boys, 6 girls, mean age 130 months) had seropositive results for HBsAg, HBeAg and HBV DNA; 11 had chronic persistent hepatitis and 17 had chronic active hepatitis. The patients were divided into two groups depending upon their inflammatory activity on liver biopsy, pretreatment serum ALT levels and HBV DNA levels. Fourteen children (group 1: chronic active hepatitis, ALT > or = 100 IU/L and HBV DNA 100 pg/300 microliter) received prednisolone in decreasing daily doses of 60 mg/m2, 40 mg/m2, and 20 mg/m2, each for 2 weeks, followed after 2 weeks by interferon alpha 2a on the same schedule. At the end of therapy, 3 end points were analyzed: HBeAg seroconversion, serum ALT normalization rate and clearance of serum HBV DNA. RESULTS: At the end of treatment, HBe antigen-to antibody seroconversion was higher but not more significant in group 1 than group 2 (71.4% vs. 50.0%). Only one patient in group 2 who lost HBeAg, also cleared HBsAg. ALT normalization was similar in both groups (64.3% in group 1 vs. 55.6% in group 2). Clearance of serum HBV DNA was observed in 78.6% of patients in group 1 and 64.3% in group 2, but no significant differences. Complete response was similarly achieved in both groups (57.1% in group 1 vs. 50.0% in group 2). Interferon alpha therapy with prednisolone priming was well tolerated and all children finished therapy. CONCLUSION: The combined therapy with prednisolone followed by interferon alpha may be safe and effective in inducing a serological and biochemical remission of the disease in approximately 50% of children with chronic hepatitis B and with a high level of viral replication and less active disease. However, a controlled study should be performed to confirm these results.

Chromosome 22q11 Deletion in Patients with Infundibular Ventricular Septal Defect

PURPOSE: This study was undertaken to determine the incidence of chromosome 22q11 deletion in patients with infundibular ventricular septal defect(VSD). METHODS: Sixty-two children with infundibular VSD were included in this study from January 1999 to December 2000. Chromosome 22q11 deletion was confirmed by FISH, using LSI DiGeorge/VCFS region dual color probe(Vysis, USA). RESULTS: Thirty-two patients had conotruncal cardiac defects:tetralogy of Fallot (TOF) in 15; TOF with absent pulmonary valve in 1; VSD with pulmonary atresia in 7; truncus arteriosus in 3; double outlet right ventricle in 2; interrupted aortic arch in 2; transposition of the great arteries in 2. Thirty patients had isolated infundibular VSD without conotruncal cardiac defect:perimembranous infundibular VSD in 15; subarterial infundibular VSD in 9; muscular infundibular VSD in 6. Chromosome 22q11 deletion was observed in 8 patients(male 5, female 3):TOF 2; VSD with pulmonary atresia 4; truncus arteriosus 1; perimembranous infundibular VSD 1. All of the patients with chromosome 21q11 deletion showed typical facial appearance. Low incidence was found of chromosome 22q11 deletion in patients with infundibular VSD without conotruncal cardiac defect than in those with conotruncal cardiac defect(3.3% vs 21.9%). CONCLUSION: These data indicate that a small proportion of isolated infundibular VSD is pathogenetically related to deletion of chromosome region 22q11.

Concordance of Seropositivity between Helicobacter pylori and Hepatitis A Virus IgG in Children of Gwangju and Chonnam Area / 대한소아소화기영양학회지

PURPOSE: This study was undertaken to investigate the seroepidemiologic pattern of Helicobacter pylori (H. pylori) and hepatitis A virus (HAV) infections in children. METHODS: A total of 315 serum samples were obtained from healthy children, living in Gwangju and Chonnam area. All serum samples were assayed for H. pylori IgG level using enzyme immunoassay techniques. HAV IgG level in serum were tested by a competitive radio-immunoassay in 215 subjects. The age-specific seroprevalence of H. pylori and HAV was separately analysed. The concordance of seropositivity and seronegativity between H. pylori and HAV infection was examined by the kappa statistic analysis. RESULTS: Seropositivity was found in 17.5% (55/315) and 30.2% (65/215) of the subjects for H. pylori and HAV, respectively. Cross-tabulation of these data showed that 21 subjects (9.8%) were seropositive and 135 (62.8%) were seronegative for both H. pylori and HAV, 15 (7.0%) were seropositive for only H. pylori and 44 (20.5%) for only HAV. The seroprevalence of H. pylori and HAV increased significantly with age. There was a slight agreement between H. pylori and HAV seropositivity (kappa=0.26). CONCLUSION: This study shows a slight similarity in the concordance of seropositivity and seronegativity between H. pylori and HAV infection and provides evidence that H. pylori and HAV may share a common mode of transmission.

A Case of Satoyoshi Syndrome Presented with Progressive Muscular Spasm and Alopecia

Satoyoshi syndrome(generalized Komuragaeri disease) is a rare disorder of unknown cause, characterized by progressive, painful, intermittent muscle spasms and alopecia. Endocrinopathy with amenorrhea, secondary skeletal abnormalities, and diarrhea or unusual malabsorption are frequently seen. It seems that autoimmunity may play a role in its pathogenesis. We report a 13-year-old girl with characteristic manifestations of the syndrome. She was treated with intravenous gammaglobulin and Prednisolone. Painful muscle cramps were gradually improved, but the scalp condition did not change. Satoyoshi syndrome should be considered in children with unexplained muscle spasms and alopecia.

A Clinical Observation on Children with Corrosive Esophagitis / 대한소아소화기영양학회지

PURPOSE: This study was undertaken to evaluate the clinical features and complication such as esophageal stricture in children with corrosive esophagitis. METHODS: We retrospectively reviewed medical records of 31 children who accidentally ingested corrosive materials and visited to emergency room of Chonnam National University Hospital from Jan. 1992 to Dec. 1999. Twenty-one children were examined by upper gastrointestinal(UGI) endoscopy to evaluate location and severity of caustic injury. RESULTS: 1) Among 31 patients, there were 20 males and 11 females and the ratio of male to female was 2:1. Average age at diagnosis was 2.3 years (12months to 9.8 years). Twenty-seven(87.1%) patients were accidentally ingested vinegar. 2) Initial presenting symptoms were dysphagia(54.8%), vomiting(48.3%), chemical burn on lips and skin(45.2%), excessive salivation(45.2%), coughing and respiratory grunting(32.3%) and aspiration pneumonia(9.8%). 3) UGI endoscopic examination showed caustic injury in 17 children : grade I in 8, grade II in 7 and grade III in 2. The region of caustic injury was proximal esophagus in 5, distal esophagus in 3, entire esophagus in 9 and stomach in 6. 4) Corrosive esophageal strictures developed in 6 children(19.4%) and gastric outlet stricture in 1(3.2%). All of them showed grade II or III caustic injury on endoscopic examination. CONCLUSION: The development of esophageal stricture was related to the severity of the caustic injury. Early UGI endoscopic examination in caustic ingestion seems to be useful for prediction of development of caustic stricture.

A Case of Bronchial Mucoepidermoid Carcinoma in Child

Bronchial mucoepidermoid carcinoma is very rarely encountered in children. We report a case of bronchial mucoepidermoid carcinoma in a 10-year-old boy who presented with persistent cough and atelectasis. Bronchoscopic examination showed a tumor mass occluding the right bronchus intermedius, and the mass was removed by bronchoscopy. The results of the pathological examination revealed low-grade mucoepidermoid carcinoma. He underwent right middle and lower lobectomy with bronchoplasty, and there was no metastasis. He remains symptom-free without recurrence of bronchial tumor during the follow-up period of 12 months.

Pleomorphic adenoma which occured buccal mucosa and submandibular gland;two case reports

No abstract available.