Erratum: Table 1, Table 3: Human Leukocyte Antigen-DRB1 Associations in Korean Juvenile Rheumatoid Arthritis

These are being published to correct the typo in Table 1 and Table 3.

Human Leukocyte Antigen-DRB1 Associations in Korean Juvenile Rheumatoid Arthritis

OBJECTIVE: The aim of this study is to investigate the association of different subgroups of juvenile rheumatoid arthritis (JRA) with human leukocyte antigen (HLA) class II DR alleles. METHODS: One hundred and nineteen Korean juvenile rheumatoid arthritis patients were classified as HLA-DRB1 allele. To assess the frequency, phenotype frequencies of all JRA cases and each subtypes were compared to those of 485 adult controls. RESULTS: HLA-DRB1*01 was associated with increased risk of JRA. Furthermore, DRB1*01 was associated with polyarticular JRA and pauciarticular JRA. The frequencies of DRB1*14 and DRB1*15 were higher in systemic JRA patients than the controls. CONCLUSION: The data of this study on Korean children with JRA suggests that HLA-DRB1*01 was associated with the susceptibility of JRA. The study should be extended to include larger numbers of patients.

Clinical and Epidemiological Study of 1,165 Hospitalized Cases of Rotaviral Gastroenteritis Before and After the Introduction of Rotavirus Vaccine, 2006-2013 / 소아감염

PURPOSE: This study was performed to assess the clinical and epidemiological changes after the introduction of the rotavirus vaccine in Korea, as well as to determine the efficacy of the rotavirus vaccine among hospitalized rotaviral gastroenteritis patients over the past two years. METHODS: We analyzed yearly and seasonal patterns of 1,165 inpatients who were hospitalized for rotaviral gastroenteritis under the age of 5 years between 2006 and 2013. We also conducted a survey among 460 gastroenteritis patients who were hospitalized between 2012 and 2013 regarding the rotavirus vaccination and the symptoms of gastroenteritis. Among those individuals surveyed, clinical indices were analyzed for 124 patients who were tested positive for the rotavirus antigen. RESULTS: The incidence of Rotaviral gastroenteritis have decreased significantly by year 2010. After the introduction and widespread dissemination of the rotavirus vaccine, the onset of the disease and the seasonal peak have been delayed. Overall, the vaccinated group showed a lower rate of positivity than the unvaccinated group. Among the hospitalized rotaviral gastroenteritis patients, the vaccinated group had a shorter hospitalization period, less severe clinical symptoms of gastroenteritis, and better laboratory test results. CONCLUSIONS: After introduction of the rotavirus vaccine in Korea, there were two main trends observed: 1) the overall level of disease incidence was reduced; 2) the severity of rotaviral gastroenteritis cases also decreased. Based on this data, more children should receive vaccination in order to prevent the rotavirus infection and decrease the severity of rotaviral gastroenteritis.

Prognostic Implications of the NIH Consensus Criteria in Children with Chronic Graft-versus-Host Disease

PURPOSE: In this study, we analyzed a cohort of children with chronic graft-versus-host disease (GvHD) according to the NIH consensus classification (NCC) in order to observe whether global assessment at diagnosis correlates with GvHD-specific endpoints. We then studied the clinical course of these patients, specifically with regards to episodes of GvHD exacerbation requiring treatment escalation. MATERIALS AND METHODS: Recipients of either allogeneic bone marrow transplantation (BMT) or peripheral blood stem cell transplantation (PBSCT) from January 2006 to August 2008 at the Department of Pediatrics, The Catholic University of Korea were evaluated for chronic GvHD, which was diagnosed according to the NCC. The course of chronic GvHD in these patients was then followed. RESULTS: Of 59 evaluable patients, 23 developed chronic GvHD for a cumulative incidence of 39.3%. Upon multivariate analysis, previous acute GvHD (> or =grade II) had a significant impact on chronic GvHD incidence. With a median duration of systemic treatment for chronic GvHD of 501 days, no significant relationship was found between initial global severity of chronic GvHD and either duration of immunosuppressive treatment or final clinical response to treatment. Fifteen patients (65%) experienced at least one episode of chronic GvHD exacerbation during the period of follow-up, with a median of four exacerbations in the subgroup of patients who experienced such events. Lung GvHD resulted in the highest number of exacerbations per diagnosed patient, followed by oral GvHD. CONCLUSION: Analysis of this small cohort indicates that global assessment as proposed by the NCC may have limited correlations with GvHD-specific endpoints, possibly due to the favorable response of children to treatment.

Idarubicin Plus Behenoyl Cytarabine and 6-thioguanine Compares Favorably with Idarubicin Plus Cytarabine-based Regimen for Children with Previously Untreated Acute Myeloid Leukemia: 10-Year Retrospective, Multicenter Study in Korea

We investigated the outcome of idarubicin plus N4-behenoyl-1-beta-D-arabinofuranosyl cytosine (BHAC)-based chemotherapy (BHAC group, n=149) compared to idarubicin plus cytarabine-based chemotherapy (cytarabine group, n=191) for childhood acute myeloid leukemia (AML). Between January 1996 and December 2005, 340 children with AML from 5 university hospitals in Korea received the BHAC-based or cytarabine-based chemotherapy, with or without hematopoietic stem cell transplantation. After induction therapy, 264 (77.6%) of 340 children achieved a complete remission (CR) and 43 (12%) achieved a partial remission (PR). The CR rate in the BHAC group was higher than in the cytarabine group (85.2% vs. 71.7%, P=0.004). However, the overall response rate (CR+PR) was not different between the two groups (93.3% vs. 87.9%, P=0.139). The 5-yr estimates of overall survival (OS) of children in the two groups were similar (54.9% for the BHAC group vs. 52.4% for the cytarabine group, P=0.281). Although the results were analyzed according to the treatment type and cytogenetic risk, the OS showed no significant difference between the BHAC group and the cytarabine group. In the present study, the clinical outcomes of the BHAC-based chemotherapy, consisting of BHAC, idarubicin, and 6-TG, are comparable to that of the cytarabine-based chemotherapy for childhood AML.

Congenital Acute Myeloid Leukemia with t(8;16) and t(17;19) Double Translocation: Case Presentation and Literature Review

Congenital leukemia is uncommon and excluding transient myeloproliferation associated with Down syndrome, makes up approximately 1% of childhood leukemias. A newborn boy was born with multiple subcutaneous nodules and large purpuric papules. Skin biopsy revealed proliferation of atypical hematologic cells in the dermis. Bone marrow morphology was consistent with acute myeloid leukemia (M5) and cytogenetic studies revealed t(8;16) and t(17;19) double translocation. Although prognosis of congenital leukemia is known to be dismal, recent reports showed spontaneous remissions. With the fear of chemotherapy-related toxicity, to treat or not to treat may be a dilemma both to parents and pediatricians. We report our experience and review the literature.

Clinical Review of the of Seizures among Children

PURPOSE: The purpose of this study was to evaluate childhood seizures to provide appropriate medical services. METHODS: We retrospectively reviewed the medical records of 221 chidren under 18 years of age with seizures (excluding febrile convulsion), who were admitted to the pediatric department of Sacred Heart Hospital, Hallym University from 2007 to 2009. RESULTS: The male to female was 1.3:1 and the peak age was 6 years or less, accounting for 63%. The most common causes of seizures according to age were listed as follows; hypocalcemia (41%) and hypoxic ischemic encephalopathies (41%) in the neonatal period, benign convulsion with mild gastroenteritis (BCwMG, 47%) in preschool children (1month and 5 years), and unprovoked seizure (80%) more than 6 years. Electroencephalogram and neuroimaging abnormalities were found in the ratio from 20% and 14% respectively. CONCLUSION: Seizure is the most common neurologic disease in the childhood. The above results reveal that the causes of childhood seizures in the different age group are different. Therefore, the exact diagnosis of disease according to age is needed. We hope that further clinical studies on this topic will be performed.

Microbiological spectrum and antibiotic susceptibility pattern in more than 24-month-old children with urinary tract infection: A6-year retrospective, single center experience / 소아과

PURPOSE: To characterize the pathogens and their antibiotic susceptibilities in more than 24-month-old children with urinary tract infection (UTI) and to study the Escherichia coli antimicrobial susceptibility trend. METHODS: We retrospectively reviewed the record of more than 24-month-old children with UTI between January 2003 and December 2008. Positive results for 1 bacterial species with a colony count of > or =10(5) CFU/mL was considered statistically significant. We analyzed uropathogens and their antibiotic susceptibilities. To investigate E. coli antibiotic susceptibility trend, we compared 2 study periods (group A: 2003-2005 versus group B: 2006-2008) using the chi-square test for trend. RESULTS: In all, 63 bacterial isolates were identified in children with febrile UTI. The most common pathogen was E. coli (77.8%). There was no difference in the resistance patterns of uropathogens during the 2 study periods (P>0.05). Antibiotic susceptibility of the E. coli isolates to aztreonam, cefotetan, cefotaxime, ceftriaxone, cefepime, amikacin, and imipenem was >90% to trimethoprim/sulfamethoxazol, 49% and to ampicillin and ampicillin/sulbactam, 20-25%. Over the 2 study period, the E. coli susceptibilities to most antibiotics did not change: the susceptibility to cefuroxime increased from 74.1% to 95.5% (P=0.046) and that to ciprofloxacin increased from 59.3% to 86.4% (P=0.039). CONCLUSION: Empirical treatment with trimethoprim/sulfamethoxazole, ampicillin, and ampicillin/sulbactam alone appeared to be insufficient in childhood UTI because of the high resistance of E. coli and other gram-negative uropathogens. Antibiotics for empirical therapy should be selected based on the sensitivity and resistance pattern of uropathogens found in a particular region.

Macroamylasemia in a 4-year-old girl with abdominal pain / 소아과

Macroamylasemia is a benign condition characterized by abnormally large-sized serum amylase; it has been reported to occur in 1-2% of the population. In macroamylasemia, a macromolecular complex consisting of amylase linked to immunoglobulins circulates in the plasma and usually causes hyperamylasemia with low or normal amylasuria. Macroamylasemia is extremely rare in children. We report a case of a 4-year-old girl with abdominal pain and macroamylasemia, who was initially misdiagnosed as having acute pancreatitis. Failure to immediately identify macroamylase as the cause of the unexplained but benign hyperamylasemia can lead to the misdiagnosis of the condition, necessitating costly analyses for ruling out pancreatic disease and unnecessary prescriptions such as fasting and intravenous replacement therapies, as was observed in our patient.

Macroamylasemia in a 4-year-old girl with abdominal pain / 소아과

Macroamylasemia is a benign condition characterized by abnormally large-sized serum amylase; it has been reported to occur in 1-2% of the population. In macroamylasemia, a macromolecular complex consisting of amylase linked to immunoglobulins circulates in the plasma and usually causes hyperamylasemia with low or normal amylasuria. Macroamylasemia is extremely rare in children. We report a case of a 4-year-old girl with abdominal pain and macroamylasemia, who was initially misdiagnosed as having acute pancreatitis. Failure to immediately identify macroamylase as the cause of the unexplained but benign hyperamylasemia can lead to the misdiagnosis of the condition, necessitating costly analyses for ruling out pancreatic disease and unnecessary prescriptions such as fasting and intravenous replacement therapies, as was observed in our patient.

Two cases of central nervous system complications caused by Mycoplasma pneumoniae infection / 소아과

Mycoplasma pneumoniae (M. pneumoniae) infection causes a wide variety of clinical manifestations in children and young adults, the main one being pneumonia. M. pneumoniae is transmitted from person to person by infected respiratory droplets. Symptoms caused by M. pneumoniae infection can be divided into those involving the respiratory tract, and those caused by extrapulmonary disease. M. pneumoniae infections may cause central nervous system (CNS) complications-with encephalitis being the most frequent-and stroke being a rare complication. The pathogenesis of the CNS disease is unclear; possibilities include direct infection and an immune-mediated reaction. We present two cases of CNS complications subsequent to infection with M. pneumoniae; both cases had convincing evidence of preceding M. pneumoniae respiratory disease with no evidence of viable M. pneumoniae in the cerebrospinal fluid. We report cases of encephalitis and stroke following a recent M. pneumoniae infection.

Clinical outcomes of hematopoietic stem cell transplantation from HLA-matched parental donor in childhood acute leukemia / 소아과

PURPOSE: In this study, we retrospectively analyzed the clinical outcomes of patients who underwent allogeneic hematopoietic stem cell transplantation (HSCT) grafted from HLA-matched parents. METHODS: Seven children with acute leukemia (4 acute lymphoblastic leukemia, 3 acute myeloid leukemia) in first complete remission received allogeneic HSCT from their respective parents at the St. Marys Hospital between April, 1999 and October, 2005. The median age of patients at transplantation was 5 years (range, 1-11 years; 2 male, 5 female) and the median age of donors was 35 years (range, 30-41 years; 5 male, 2 female). We investigated the clinical outcomes such as engraftment, acute and chronic graft-versus-host disease (GVHD), transplant-related morbidity and mortality, relapse and survival. RESULTS: Median time from transplantation to last follow-up was 69.5 months (range, 18.8-96.5 months). All patients were successfully engrafted, with a median time of 11 days (range, 10-16 days) and 26 days (range, 13-39 days) for neutrophil and platelet recovery, respectively. Grade II acute GVHD occurred in 3, and grade III acute GVHD in 1 of 7 recipients. Extensive chronic GVHD developed in 2, and limited chronic GVHD in 1 of 7 recipients. Death from transplant-related complications occurred in 1, and relapse occurred in 1 of 7 recipients. Estimated 5-year overall survival was 83+/-15%. CONCLUSION: The clinical outcomes of recipients who underwent HSCT from HLA-matched parents were comparable to those of patients who received HSCT grafted from HLA-matched sibling donors in childhood leukemia. HLA typing of parents, as well as siblings will increase the likelihood of finding an HLA-matched family donor for patients who need HSCT.

Distribution of CD4+CD25+ T cells and graft-versus-host disease in human hematopoietic stem cell transplantation / 소아과

PURPOSE: This study aimed to determine the frequencies of CD4+CD25+ T cells in donor graft and peripheral blood CD4+CD25+ T cells in recipients after hematopoietic stem cell transplantation (HSCT) and their association with graft-versus-host disease (GVHD). METHODS: Seventeen children who underwent HSCT were investigated. CD4+CD25+ T cells in samples from donor grafts and recipient peripheral blood were assessed by flow cytometry at 1 and 3 months after transplantation. RESULTS: CD4+CD25+ T cell frequencies in the grafts showed no significant difference between patients with and without acute GVHD (0.90% vs. 1.06%, P=0.62). Absolute CD4+CD25+ T cell number in grafts were lower in patients with acute GVHD than in those without acute GVHD (6.18x10(5)/kg vs. 25.85x10(5)/kg, P=0.09). Patients without acute GVHD showed a significant decrease in peripheral blood CD4+CD25+ T cell percentage at 3 months compared to those at 1 month after HSCT (2.11% vs. 1.43%, P=0.028). However, in patients with acute GVHD, CD4+CD25+ T cell percentage at 3 months was not different from the corresponding percentage at 1 month after HSCT (2.47% vs. 2.30%, P=0.5). CONCLUSION: The effect of frequencies of CD4+CD25+ T cells in donor grafts on acute GVHD after HSCT could not be identified, and the majority of peripheral blood CD4+CD25+ T cells in patients who underwent HSCT may be activated T cells related to acute GVHD rather than regulatory T cells. Further studies with additional markers for regulatory T cells are needed to validate our results.

Serum creatinine, blood urea nitrogen change in low birth weight infants during their first days of life / 대한주산의학회잡지

OBJECTIVE: To study the changes in serum creatinine and correlation between gestational age or birth weight and serum creatinine in low birth weight infants in the immediate postnatal period. METHODS: Medical records of all premature infants, who were admitted to the neonatal intensive care unit of Hallym University Hospital between January 2003 and December 2007, were reviewed. 162 infants met our inclusion criteria. Medical records were reviewed for : birth weight, gestational age, length, gender, APGAR scores, use of medications, blood urea nitrogen (BUN) and serum creatinine (Scr) during the first days of life. Premature infants were separated into three groups according to their birth weight: 500 to 999 g; 1,000 to 1,499 g; and 1,500 to 2,000 g. RESULTS: Scr was found to decrease postnatally, however there was a delay in the decrease of Scr in the subgroup of infants<1,000 g BW, Scr was also found to decrease with increasing birth weight at 1 week after birth (Pearson test, p=0.01). Serum BUN was found to decrease with increasing birth weight at 1 week after birth (Pearson test, p=0.00). CONCLUSION: In low birth weight infants Scr decrease during the first days of life. However, in infants smaller than 1,000 g birth weight there is a delay in the decrease of their Scr that extends beyond the first days of life. Our findings indicate progression of renal function is directly correlated to birth weight.

Evaluation for Cytopreservability of Manual Liquid-Based Cytology Liqui-PREP(TM) and its Application to Cerebrospinal Fluid Cytology: Comparative Study with Cytospin / 대한세포병리학회지

Cerebrospinal fluid (CSF) cytology is an effective tool for evaluating diseases involving the central nervous system, but his technique is usually limited by its low cellularity and poor cellular preservation. Here we compared the manual liquid-base Liqui-PREPTM (LP) to the cytospin (CS) with using a mononuclear cell suspension and we applied both methods to the CSFs of pediatric leukemia patients. The cytopresevability, in terms of cell yield and cell size, and the clinical efficacy were evaluated. When 2000 and 4000 mononuclear cells were applied, LP was superior to CS for the cell yield, 16.8% vs 1.7% (P=0.001) and 26.2% vs 3.5% (P=0.002), respectively. The mean size of the smeared cells was 10.60 micrometer in the CS, 5.01 micrometer in the LP and 6.50 micrometer in the direct smear (DS), and the size ratio was 1.7 (CS to DS), 0.8(LP to DS) and 2.1 (CS to LP), respectively. As compared to the cells in the DS, the cells in the CS were significantly enlarged, but those in the LP were slightly shrunken. Upon application to 109 CSF samples, 4 were diagnosed as positive for leukemia (positive), 4 had atypical cells and 101 were negative by CS; 6 were positive, one had atypical cells and 102 were negative by LP. For six cases, in which 4 were positive for leukemia and 2 of 4 had atypical cells by CS, they were positive by LP and they were also confirmed as positive according to the follow-up study. Three cases diagnosed as atypical cells (two by CS and one by LP), were confirmed as negative. In conclusion, these results suggest that LP is superior to CS for the cytopresevability and for rendering a definite diagnosis of cerebrospinal fluid.

Final Adult Height of Children with Normal Endocrine Function after Hematopoietic Stem Cell Transplantation / 대한소아내분비학회지

PURPOSE: We analyzed the final adult height of patients without endocrine dysfunction who underwent hematopoietic stem cell transplantation (HSCT) during the childhood. METHODS: We evaluated the final height of 28 long term survivors (13 males, 15 females) who underwent HSCT at the mean age of 12.3 years. Patients who had solid tumors, inherited diseases and endocrine dysfunction before or after HSCT were excluded. The mean age at last visit was 18.8 years. Height values were expressed in standard deviation score (SDS). Height at HSCT was compared with final height as well as mid-parental height. We analyzed the risk factors for affecting final adult height of patients. RESULTS: There was a decrease in final height SDS compared to pre-transplantation height SDS (P= 0.003). All patients except one reached an adult height above -2.0 SDS of normal population. The difference between the height SDS at HSCT and the final height was -0.98+/-0.5 SDS in boys and -0.10+/-0.6 SDS in girls (P<0.01, and P=0.53 respectively). A significant decrease in height SDS was found in male (Mann-Whitney U test, P=0.001). The type of primary diseases, age at HSCT, total body irradiation, acute graft-versus-host disease did not influence the final height. CONCLUSION: Despite the decrease in final height SDS after HSCT during childhood, the majority of patients without endocrine dysfunction spontaneously reached on a normal adult height range (above -2.0 SDS). Therefore, careful monitoring of growth after HSCT during childhood is warranted to detect the growth velocity decrease.

The effects of shortened dexamethasone administration on remission rate and potential complications during remission induction treatment for pediatric acute lymphoblastic leukemia / 소아과

PURPOSE: Due to its high potency against leukemic blasts, our institution has opted for the use of dexamethasone during acute lymphoblastic leukemia (ALL) remission induction, but in our most recent treatment protocol, CMCPL-2005, we shortened the length of steroid treatment from 4 to 3 weeks. We compared both the rates of remission induction and significant complications observed during induction with CMCPL-2005, with those noted for our previous protocol, CMCPL-2001. METHODS: We retrospectively reviewed the records of patients diagnosed with ALL from January, 2001 to December, 2006 at the Department of Pediatrics, St. Mary's Hospital, the Catholic University of Korea. Data concerning age, sex, WBC count at diagnosis, immunophenotype, cytogenetic traits, and risk group were collected for each patient. Results of remission induction treatment were compared between the two patient groups. Infection and other major complications resulting from treatment were investigated according to NCI toxicity criteria. RESULTS: A total of 141 and 88 patients received remission induction under CMCPL-2001 and CMCPL-2005 respectively. In the CMCPL-2001 group, 136 (96%) achieved complete remission while 82 (93%) achieved CR in the CMCPL-2005 group. Patients in the CMCPL-2005 group were more likely to undergo remission induction without experiencing major complications. However, with regards to steroid related toxicities such as infection, no significant differences were noted. CONCLUSION: We shortened the length of steroid administration from four to three weeks, yet found the remission induction rate to be comparable to that of our previous regimen. However, rates of steroid related toxicities such as infectious complications remain unchanged despite shortened exposure to dexamethasone.

Effect of a Pholiota adiposa Extract on Fat Mass in Hyperlipidemic Mice

The purpose of this study was to investigate the effect of a Pholiota adiposa extract on fat mass in hyperlipidemic mice fed on a high-fat diet. The water extracts from P. adiposa (ASI 24018) were not affected in the total triglyceride contents and epididymal fat mass in mice fed on a high-fat diet, but the retroperitoneal fat mass decreased significantly. This result suggests that the P. adiposa extract may be a potential candidate for use as a functional food that can act as a prophylactic against hyperlipidemia. However, the P. adiposa extract showed no effect in the total triglyceride contents and epididymal fat mass.

Short-term follow up of thyroid function after pediatric hematopoietic stem cell transplantation / 소아과

PURPOSE: In this study, we analyzed the short term changes of thyroid function, incidence and risk factors of thyroid dysfunction soon after allogeneic hematopoietic stem cell transplantation (HSCT) in children. METHODS: We enrolled 80 pediatric patients following allogeneic HSCT, at the Catholic HSCT center between January, 2004 and February, 2006. Serum TSH (thyroid stimulating hormone), total serum thyroxine and total serum triiodothyronine levels were systematically measured in 80 patients before the HSCT, and at 1 month, 6 months and 12 months after HSCT. RESULTS: Thyroid function statistically decreased at 1 month after HSCT(P or = II) were risk factors for ETS (P=0.04, 0.01 respectively). In multivariate analysis, we could not detect an independent risk factor for ETS (P=0.19, 0.06 respectively). CONCLUSION: The present study suggests that the incidence of thyroid dysfunction is high after allogeneic HSCT. Therefore, regular monitoring of thyroid hormone levels after HSCT is required.

A Case of Bilateral Congenital Nasolacrimal Duct Cysts Presenting as Neonatal Respiratory Distress / 대한주산의학회잡지

Congenital nasolacrimal duct cyst is an uncommon anomaly of nasolacrimal duct obstruction in the newborn. It is cystic dilation of the lower end of the unperforated nasolacrimal duct with intranasal extension. In such cases, the bluish-gray cyst arises beneath the inferior turbinate on nasal endoscopy. A large nasolacrimal duct cyst can fill the nasal cavity and lead to nasal obstruction. Neonates are obligate nasal breathers at birth and require several weeks to learn to breathe through the mouth. Consequently this nasal obstruction can cause significant respiratory distress. We experienced a case of bilateral nasolacrimal duct cyst presenting as neonatal respiratory distress. Diagnosis was confirmed by nasal endoscopy and CT scan. After endoscopic marsupialization of the cysts , symptom and sign of respiratory distress were rapidly resolved.