Efficacy of early steroid use in Mycoplasma pneumoniae pneumonia

PURPOSE: The aim of this study was to evaluate whether or not the early use of steroid is useful for treating Mycoplasma pneumoniae pneumonia. METHODS: A prospective study was conducted on 85 patients with M. pneumoniae pneumonia admitted to Gwangju Christian Hospital between September 2015 and April 2016. A total of 85 patients were enrolled. Of these, 33 were treated with steroids (methyl-prednisolone 1 mg/kg/day), while 52 were not; both were treated with macrolides. The overall duration of fever was compared between the 2 groups and findings on chest radiographs were evaluated for their deterioration. RESULTS: The duration of fever after admission (1.36±0.92 days vs. 2.17±1.30 days, P=0.003) and the overall duration of fever (4.42±2.13 days vs. 6.07±2.59 days, P=0.003) were significantly lower in the steroid group. The duration of fever before admission was not different between the steroid and macrolide groups (3.06±1.74 days vs. 3.90±2.21 days, P=0.068). On chest radiographs taken 3 days later, 1 of 33 patients in the steroid group and 5 of 50 patients in the macrolide group worsened, although there was no statistically significant difference between the 2 groups (P=0.395). There was no significant difference in the duration of hospitalization between the 2 groups (6.72±1.54 days vs. 6.92±1.87 days, P=0.618). CONCLUSION: Early administration of steroids on patients with M. pneumoniae pneumonia reduced the duration of fever, but there was no difference in duration of admission and x-ray deterioration.

Alagille Syndrome Mimicking Biliary Atresia Confirmed by Jagged1 (JAG1) Gene Analysis in a Newborn: A Case Report

Alagille syndrome is a complex autosomal dominant disorder secondary to defects in the Notch signaling pathway, primarily caused by mutations in the Jagged1 (JAG1) gene. The liver, heart, skeleton, face and eyes are the body parts most commonly involved. Alagille syndrome may mimic other causes of high gamma-glutamyl transferase (GGT)-linked cholestasis, most notably biliary atresia in the neonatal period. Infants with Alagille syndrome are occasionally misdiagnosed as cases with biliary atresia due to variations in clinical features that might be expressed in early infancy. We describe a case of Alagille syndrome mimicking biliary atresia, identified by sequencing analysis of the JAG1 gene in a newborn. During counseling, family members of the patient have also been found to demonstrate various phenotypes and levels of disease severity of Alagille syndrome.

Recent trends in contraceptive use among Korean adolescents: Results from a nationwide survey from year 2013 to 2015

OBJECTIVE: This study was aimed to evaluate the recent trends in contraceptive use among Korean adolescents. METHODS: Data reviewed were from the 2013–2015 Korean Youth Risk Behavior Web-based Survey, which is a stratified, multistage-sampling designed online-based research project performed annually by the Korean government to ensure a nationally representative sample of Korean adolescence. Eight questions related to the topic of contraception were reviewed for the outcome variables. RESULTS: A total of 212,538 adolescents attending middle school and high school participated in the survey, and 8,755 students among them who were sexually active were included in the study. The percentage of contraceptive use showed a steady increase from 39% in 2013 to 48.7% in 2015; however, the proportion of adolescents who have never used any kind of contraception still remains high. Highly effective methods such as oral contraceptives and intrauterine devices were used by only 10% to 15% of sexually active adolescents. CONCLUSION: The present study demonstrates the status of contraceptive use among Korean adolescents. Our data have the potential to help healthcare providers to formulate policies and develop interventions for encouraging effective contraceptive use among sexually active Korean adolescents.

Comparison of Survival by Risk-stratified Therapy in Children and Adolescents with Acute Lymphoblastic Leukemia in a Single Institute / 임상소아혈액종양

BACKGROUND: Impressive improvement of survival rate has been achieved in childhood acute lymphoblastic leukemia (ALL). In an effort to balance the risks and benefits of therapy, risk-stratified therapy has been adopted. The aim of this study was to evaluate treatment outcome of childhood ALL by risk stratification.METHODS: From 184 patients (age, <18 years) with ALL newly diagnosed at Chonnam National University Hospital and Chonnam National University Hwasun Hospital between 2000 and 2010, we retrospectively analyzed 157 patients. Patients with infant ALL, L3, Down syndrome, and those transferred to another hospital were excluded. Three risk groups were defined as standard risk (SR, n=88), high risk (HR, n=52) and very high risk (VHR, n=17).RESULTS: The 7-year overall survival and event-free survival (EFS) rates were 85.2+/-2.9% and 80.2+/-3.3%, respectively. The 7-year EFS rates were 86.5+/-3.9% for SR, 78.8+/-5.7% for HR and 52.9+/-12.1% for VHR (P<0.001). Relapse occurred in 17 patients, and the cumulative incidence of relapse at 7 years was not different according to risk groups (SR vs. HR vs. VHR=8.9% vs. 17.3% vs. 5.9%, P=0.171). Among relapsed patients, 11 underwent hematopoietic stem cell transplantation of whom 5 survive event-free with a median follow-up of 5.2 years. The cumulative incidence of non-relapse mortality was highest in VHR (SR vs. HR vs. VHR=4.6% vs. 3.8% vs. 47.2%, P<0.001).CONCLUSION: Although, the number of patients included in this study is relatively small, the survival rates of childhood ALL with current strategies are encouraging. Also, efforts should be focused to further improve survival in the VHR, especially to reduce non-relapse mortality.

A Case of Nasal Type Extranodal NK/T Cell Lymphoma Incidentally Detected in a Child / 임상소아혈액종양

A nasal type extranodal NK/T-cell lymphoma (ENKL) is very rare in children. A pediatric nasal type ENKL is generally localized and is likely to have sensitivity to radiotherapy. The most common site is the upper airway tract, such as nasal region, Waldeyer's ring, paranasal sinuses and palates. It usually presents with nasal symptoms, such as obstruction or epistaxis. We describe our experience of concurrent chemoradiotherapy in a 13-year old boy having incidentally detected nasal type ENKL on laryngoscopic examination who did not have nasal symptoms. He received three cycles of dexamethasone (40 mg/day for 3 days), ifosfamide (1,000 mg/m2/day for 3 days), VP-16 (67 mg/m2/day for 3 days) and carboplatin (200 mg/m2 for 1 day) at 3-week intervals and 45 Gy intensity-modulated radiation therapy. He has been disease-free for 18 months after cessation of therapy.

Comparison of Survival by Risk-stratified Therapy in Children and Adolescents with Acute Lymphoblastic Leukemia in a Single Institute / 임상소아혈액종양

BACKGROUND: Impressive improvement of survival rate has been achieved in childhood acute lymphoblastic leukemia (ALL). In an effort to balance the risks and benefits of therapy, risk-stratified therapy has been adopted. The aim of this study was to evaluate treatment outcome of childhood ALL by risk stratification. METHODS: From 184 patients (age, <18 years) with ALL newly diagnosed at Chonnam National University Hospital and Chonnam National University Hwasun Hospital between 2000 and 2010, we retrospectively analyzed 157 patients. Patients with infant ALL, L3, Down syndrome, and those transferred to another hospital were excluded. Three risk groups were defined as standard risk (SR, n=88), high risk (HR, n=52) and very high risk (VHR, n=17). RESULTS: The 7-year overall survival and event-free survival (EFS) rates were 85.2+/-2.9% and 80.2+/-3.3%, respectively. The 7-year EFS rates were 86.5+/-3.9% for SR, 78.8+/-5.7% for HR and 52.9+/-12.1% for VHR (P<0.001). Relapse occurred in 17 patients, and the cumulative incidence of relapse at 7 years was not different according to risk groups (SR vs. HR vs. VHR=8.9% vs. 17.3% vs. 5.9%, P=0.171). Among relapsed patients, 11 underwent hematopoietic stem cell transplantation of whom 5 survive event-free with a median follow-up of 5.2 years. The cumulative incidence of non-relapse mortality was highest in VHR (SR vs. HR vs. VHR=4.6% vs. 3.8% vs. 47.2%, P<0.001). CONCLUSION: Although, the number of patients included in this study is relatively small, the survival rates of childhood ALL with current strategies are encouraging. Also, efforts should be focused to further improve survival in the VHR, especially to reduce non-relapse mortality.

Graves disease following rabbit antithymocyte globulin treatment of severe aplastic anemia in a Korean child / 소아과

Antithymocyte globulin (ATG) is used as an immunosuppressive treatment (IST) to deplete clonal suppressor T cells in patients with severe aplastic anemia (SAA). The depletion of suppressor T cells by ATG may affect the activation of B cells, which results in an increased risk for autoimmune conditions. A 12-year-old boy was diagnosed with idiopathic SAA. As he did not have an human leukocyte antigen-matched sibling, he was treated with rabbit ATG (3.5 mg/kg/day for 5 days) and cyclosporine. Five months later, he became transfusion independent. However, 23 months after IST, he complained of mild hand tremors, sweating, weight loss, palpitations, and goiter. Results of thyroid function tests revealed hyperthyroidism (free thyroxine, 3.42 ng/dL; thyroid stimulating hormone [TSH], <0.01 nIU/mL; triiodothyronine, 3.99 ng/mL). Results of tests for autoantibodies were positive for the antimicrosome antibody and TSH-binding inhibitory immunoglobulin, but negative for the antithyroglobulin antibody and antinuclear antibody. He was treated with methimazole, and his symptoms improved. The patient has been disease free for 39 months after IST and 9 months after methimazole treatment. This case report suggests that although rare, rabbit ATG may have implications in the pathogenesis of autoimmune hyperthyroidism. Our findings suggest that thyroid function tests should be incorporated in the routine follow-up of SAA patients treated with ATG.

A Case of Nasal Type Extranodal NK/T Cell Lymphoma Incidentally Detected in a Child / 임상소아혈액종양

A nasal type extranodal NK/T-cell lymphoma (ENKL) is very rare in children. A pediatric nasal type ENKL is generally localized and is likely to have sensitivity to radiotherapy. The most common site is the upper airway tract, such as nasal region, Waldeyer's ring, paranasal sinuses and palates. It usually presents with nasal symptoms, such as obstruction or epistaxis. We describe our experience of concurrent chemoradiotherapy in a 13-year old boy having incidentally detected nasal type ENKL on laryngoscopic examination who did not have nasal symptoms. He received three cycles of dexamethasone (40 mg/day for 3 days), ifosfamide (1,000 mg/m2/day for 3 days), VP-16 (67 mg/m2/day for 3 days) and carboplatin (200 mg/m2 for 1 day) at 3-week intervals and 45 Gy intensity-modulated radiation therapy. He has been disease-free for 18 months after cessation of therapy.

Prognostic Factors and Outcome in Childhood and Adolescent Osteosarcoma: Single Institution Experience / 임상소아혈액종양

BACKGROUND: Osteosarcoma is the most common primary malignant bone tumor in children and adolescents. The aim of this study was to evaluate survival and the prognostic factors in children and adolescent osteosarcoma.METHODS: Twenty-seven patients with osteosarcoma diagnosed at Chonnam National University Hwasun Hospital between Apr. 2004 and Feb. 2013 were retrospectively reviewed.RESULTS: Fourteen patients were males and the median age at diagnosis was 13.0 years. The most common tumor site was distal femur and 5 patients had metastasis at diagnosis. All patient but one who underwent definitive surgery at diagnosis received preoperative chemotherapy. The 5-year overall survival (OS) and event free survival (EFS) rate for all patients were 65.7+/-10.2% and 55.6+/-11.0%, respectively. Ten patients (37.0%) experienced relapse or progression of the disease. Among them, 3 patients are alive without any evidence of disease at the time of this writing. Seven among 8 dead patients died of relapse/progression of the disease. The 5-year EFS rate was significantly higher for patients with tumor volume <100 cm3 (n=14) at diagnosis than others (n=10) (70.7+/-14.6% vs. 37.5+/-16.1%, P=.031). Age, histologic type, metastasis at diagnosis and histologic response to preoperative chemotherapy did not have a significant effect on OS and EFS.CONCLUSION: Although chemotherapy has improved outcomes of osteosarcoma, relapse or progression is the most common cause of treatment failure. A higher tumor volume at diagnosis was identified as a poor prognostic factor. Future studies incorporating a larger number of patients are required to further delineate the prognostic factors in osteosearcoma.

Prognostic Factors and Outcome in Childhood and Adolescent Osteosarcoma: Single Institution Experience / 임상소아혈액종양

BACKGROUND: Osteosarcoma is the most common primary malignant bone tumor in children and adolescents. The aim of this study was to evaluate survival and the prognostic factors in children and adolescent osteosarcoma. METHODS: Twenty-seven patients with osteosarcoma diagnosed at Chonnam National University Hwasun Hospital between Apr. 2004 and Feb. 2013 were retrospectively reviewed. RESULTS: Fourteen patients were males and the median age at diagnosis was 13.0 years. The most common tumor site was distal femur and 5 patients had metastasis at diagnosis. All patient but one who underwent definitive surgery at diagnosis received preoperative chemotherapy. The 5-year overall survival (OS) and event free survival (EFS) rate for all patients were 65.7+/-10.2% and 55.6+/-11.0%, respectively. Ten patients (37.0%) experienced relapse or progression of the disease. Among them, 3 patients are alive without any evidence of disease at the time of this writing. Seven among 8 dead patients died of relapse/progression of the disease. The 5-year EFS rate was significantly higher for patients with tumor volume <100 cm3 (n=14) at diagnosis than others (n=10) (70.7+/-14.6% vs. 37.5+/-16.1%, P=.031). Age, histologic type, metastasis at diagnosis and histologic response to preoperative chemotherapy did not have a significant effect on OS and EFS. CONCLUSION: Although chemotherapy has improved outcomes of osteosarcoma, relapse or progression is the most common cause of treatment failure. A higher tumor volume at diagnosis was identified as a poor prognostic factor. Future studies incorporating a larger number of patients are required to further delineate the prognostic factors in osteosearcoma.

Long-term follow-up of Fanconi anemia: clinical manifestation and treatment outcome / 소아과

PURPOSE: The aim of this study was to characterize Korean patients with Fanconi anemia (FA), which is a rare but very challenging genetic disease. METHODS: The medical records of 12 FA patients diagnosed at Chonnam National University Hospital from 1991 to 2012 were retrospectively reviewed. RESULTS: The median age at diagnosis was 6.2 years. All patients showed evidence of marrow failure and one or more physical stigmata. Chromosome breakage tests were positive in 9 out of 11 available patients. The median follow-up duration was 69.5 months. The Kaplan-Meier (KM) survival of all patients was 83.3% at 10 years and 34.7% at 20 years, respectively. Seven patients underwent 9 stem cell transplantations (SCTs). Among them, 5 were alive by the end of the study. Ten-year KM survival after SCT was 71.4% with a median follow-up of 3.4 years. All 5 patients treated with supportive treatment alone died of infection or progression at the median age of 13.5 years, except for one with short follow-up duration. Acute leukemia developed in 2 patients at 15.4 and 18.1 years of age. Among 6 patients who are still alive, 3 had short stature and 1 developed insulin-dependent diabetes mellitus. CONCLUSION: We provide information on the long-term outcomes of FA patients in Korea. A nation-wide FA registry that includes information of the genotypes of Korean patients is required to further characterize ethnic differences and provide the best standard of care for FA patients.

Lung torsion after tracheoesophageal fistula repair in an infant / 소아과

Lung torsion is a very rare event that has been reported in only 9 cases in the pediatric literature but has not yet been reported in Korean infants. We present a case of lung torsion after tracheoesophageal fistula repair in an infant. Bloody secretion from the endotracheal tube and chest radiographs and computed tomographic scan results indicated lung torsion. Emergency exploration indicated 180degrees torsion of the right upper lobe (RUL) and right middle lobe (RML). After detorsion of both lobes, some improvement in the RUL color was observed, but the color change in the RML could not be determined. Although viability of the RML could not be proven, pexy was performed for both the lobes. Despite reoperation, clinical signs and symptoms did not improve. The bronchoscopy revealed a patent airway in the RUL but not in the RML. Finally, the RML was surgically removed. The patient was discharged on the 42nd day after birth.

Transvenous proximal closure of large congenital coronary arteriovenous fistula using the single Amplatzer vascular plug in a 3-year-old girl / 소아과

Congenital coronary arteriovenous fistulas (CCAFs) are rare coronary artery abnormalities in which blood is shunted into a cardiac chamber or great vessel. If the fistula itself is large and tortuous, it is generally recommended to occlude the fistula to prevent several complications. In approaches of transcatheter occlusion, the transvenous approach is preferred over the transarterial approach. The transvenous approach would enable the cannulation of a relatively larger catheter or sheath without potential damage to the femoral vessels or normal coronary arteries, which can occur in the transarterial approach. The transvenous approach may also minimize the blind pouch after releasing the devices. Herein, we report the success of transvenous proximal closure of a CCAF using an Amplatzer vascular plug (AVP) in a 3-year-old patient with cardiomegaly. Complete occlusion was achieved by a single AVP and thrombus formation of the distal aneurysmal portion of the fistula. We suggest that this strategy of closing the proximal end with a dilated fistula using a single AVP by the transvenous approach may be a good option in treating CCAFs in a young child.

Changes in Birth Rate, Perinatal Risk Factors and Outcome in Newborns in Multi-cultural Family: Ten-year Experience in One Center

PURPOSE: The aim of this study is to investigate the periodical changes of birth rate, perinatal risk factors and outcome of newborns in multi-cultural families with Korean male and non-Korean, Asian female in Gwangju, Chonnnam province. METHODS: We enrolled newborns born in or transferred from other hospital to Chonnam National University Hospital between January 2001 and December 2010. They were categorized into two periods: data from 2001 to 2005 was defined as period I and from 2006 to 2010 was defined as period II. The clinical data of the birth rate, perinatal risk factors and outcome were retrospectively reviewed. RESULTS: Number and rate of newborns in multicultural families increased significantly. The nationality of the mother changed: Vietnamese, Chinese and Cambodian significantly increased; and Japanese and Filipino significantly decreased. As the maternal age tended to be younger in period II, the age gap between the spouses increased, and the paternal age significantly increased, especially in age group of over 45 year-old. Among other perinatal risk factors, premature rupture of membranes and oligohydramnios were decreased. There were no statistical difference in morbidity (except hyperbilirubinemia) and mortality. However, congenital anomalies significantly increased. CONCLUSION: As the number of newborns in multicultural families has increased, the incidence of congenital anomalies also increased. We carefully presumed this result could be caused by increasing paternal age. Careful concern and management are needed for these families and further prospective studies are needed in a larger number of subjects.

Low-dose, Once-a-day Treatment of Topiramate in Benign Childhood Epilepsy with Centrotemporal Spikes

PURPOSE: Benign childhood epilepsy with centrotemporal spikes (BECTS) is known to have a good prognosis, and is easily controlled with antiepileptic drugs. In this study, we evaluate the efficacy of low-dose topiramate (TPM), once at night time in children with BECTS, and compare with that of oxcarbazepine (OXC). METHODS: OXC was used as a first drug in 58 patients with BECTS at the department of pediatrics, Chonnam National University hospital from Jan. 2003 to Nov. 2010. Their medical records were reviewed, focusing on the onset age of seizure, the dosage of drugs, response to treatment and other clinical features. Since Aug. 2008 through Apr. 2011, some newly diagnosed patients with BECTS were given a low-dose (25mg) of TPM, once at nighttime. The efficacy was reviewed in 14 children, who had been followed up in the outpatient clinic for longer than twelve months. RESULTS: Forty five out of 58 (77.6%) patients were well controlled with OXC. Thirteen out of 58 (22.4%), seizures recurred, on whom TPM (n=9) or lamotrigine (n=4) was added. Nine (64.3%) of the 14 patients who were started with low dose TPM became seizure-free for at least 12 months. However, Two out of 14, the dose of TPM had to be increased up to 100 mg/day and 75 mg/day, respectively. In three, seizure was controlled with OXC. CONCLUSION: A single low-dose TPM would be as effective as the conventional OXC without any adverse effects in children with BECTS.