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Transient neonatal diabetes mellitus caused by a de novo ABCC8 gene mutation.

Korean Journal of Pediatrics.2011;54(4):179-182. doi:10.3345/kjp.2011.54.4.179

Transient neonatal diabetes mellitus (TNDM) is a rare form of diabetes mellitus that presents within the first 6 months of life with remission in infancy or early childhood. TNDM is mainly caused by anomalies in the imprinted region on chromosome 6q24; however, recently, mutations in the ABCC8 gene, which encodes sulfonylurea receptor 1 (SUR1), have also been implicated in TNDM. Herein, we present the case of a male child with TNDM whose mutational analysis revealed a heterozygous c.3547C>T substitution in the ABCC8 gene, leading to an Arg1183Trp mutation in the SUR1 protein. The parents were clinically unaffected and did not show a mutation in the ABCC8 gene. This is the first case of a de novo ABCC8 gene mutation in a Korean patient with TNDM. The patient was initially treated with insulin and successfully switched to sulfonylurea therapy at 14 months of age. Remission of diabetes had occurred at the age of 16 months. Currently, the patient is 21 months old and is euglycemic without any insulin or oral hypoglycemic agents. His growth and physical development are normal, and there are no delays in achieving neurological and developmental milestones.
ATP-Binding Cassette Transporters ; Child ; Diabetes Mellitus ; Humans ; Hypoglycemic Agents ; Infant ; Insulin ; Male ; Parents ; Potassium Channels, Inwardly Rectifying ; Receptors, Drug

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A case of hemolytic uremic syndrome preceded by intussusception.

Eun Young KO ; Joo Young KIM ; Hye Jin LEE ; Hyun Seung LEE ; Ji Whan HAN ; Young Hoon KIM ; Jin Tack KIM ; Hae Il CHEONG ; Pil Sang JANG

Korean Journal of Pediatrics.2011;54(4):176-178. doi:10.3345/kjp.2011.54.4.176

Hemolytic-uremic syndrome (HUS) is the most common cause of acute renal failure in young children. It is classically characterized by the triad of microangiopathic hemolytic anemia, thrombocytopenia, and uremia. Further, not only is intussusception one of the differential diagnoses of HUS but it may also become a complication during disease progression. We report a case of HUS preceded by intussusception in a previously healthy 17-month-old boy. The patient presented at the emergency department with bloody stools that developed the day after reduction of intussusception. HUS was diagnosed 4 days after the reduction of intussusception. The patient was provided only supportive care and his laboratory test findings were normal at discharge.
Acute Kidney Injury ; Anemia, Hemolytic ; Child ; Diagnosis, Differential ; Disease Progression ; Emergencies ; Hemolytic-Uremic Syndrome ; Humans ; Infant ; Intussusception ; Thrombocytopenia ; Uremia

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Selective embolization of the internal iliac arteries for the treatment of intractable hemorrhage in children with malignancies.

Sul Hee BAE ; Dong Kyun HAN ; Hee Jo BAEK ; Sun Ju PARK ; Nam Kyu CHANG ; Hoon KOOK ; Tai Ju HWANG

Korean Journal of Pediatrics.2011;54(4):169-175. doi:10.3345/kjp.2011.54.4.169

PURPOSE: Acute internal hemorrhage is an occasionally life-threatening complication in pediatric cancer patients. Many therapeutic approaches have been used to control bleeding with various degrees of success. In this study, we evaluated the efficacy of selective internal iliac artery embolization for controlling acute intractable bleeding in children with malignancies. METHODS: We retrospectively evaluated the cases of 6 children with various malignancies (acute lymphoblastic leukemia, acute myelogenous leukemia, chronic myelogenous leukemia, T-cell prolymphocytic leukemia, Langerhans cell histiocytosis, and rhabdomyosarcoma), who had undergone selective arterial embolization (SAE) of the internal iliac artery at the Chonnam National University Hwasun Hospital between January 2004 and December 2009. SAE was performed by an interventional radiologist using Gelfoam(R) and/or Tornado(R) coils. RESULTS: The patients were 5 boys and 1 girl with median age of 6.9 years (range, 0.7-14.8 years) at the time of SAE. SAE was performed once in 4 patients and twice in 2, and the procedure was unilateral in 2 and bilateral in 4. The causes of hemorrhage were as follows: hemorrhagic cystitis (HC) in 3 patients, procedure-related internal iliac artery injuries in 2 patients, and tumor rupture in 1 patient. Initial attempt at conservative management was unsuccessful. Of the 6 patients, 5 (83.3%) showed improvement after SAE without complications. CONCLUSION: SAE may be a safe and effective procedure for controlling acute intractable hemorrhage in pediatric malignancy patients. This procedure may obviate the need for surgery, which carries an attendant risk of morbidity and mortality in cancer patients with critical conditions.
Child ; Cystitis ; Embolization, Therapeutic ; Hemorrhage ; Histiocytosis, Langerhans-Cell ; Humans ; Iliac Artery ; Leukemia, Myelogenous, Chronic, BCR-ABL Positive ; Leukemia, Myeloid ; Leukemia, Prolymphocytic, T-Cell ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Retrospective Studies ; Rupture

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Immune response to 19A serotype after immunization of 19F containing pneumococcal conjugate vaccine in Korean children aged 12-23 months.

Hyun Ju LEE ; So Eun PARK ; Kyung Hyo KIM

Korean Journal of Pediatrics.2011;54(4):163-168. doi:10.3345/kjp.2011.54.4.163

PURPOSE: The purpose of this study was to evaluate the immune response to serotype 19A in children aged 12-23 months after immunization of the 19F containing 7-valent pneumococcal conjugate vaccine (PCV7). METHODS: Blood samples from a total of 45 subjects (age 12-23 months) were included in the study. Subjects were categorized according to immunization status into three groups as follows: 18 subjects with 3 primary doses and 1 booster dose of PCV7 (booster group), 21 subjects with 3 primary doses before 12 months of age (primary group), and 6 subjects with no vaccination history of PCV7 (control group). An ELISA and opsonophagocytic killing assay (OPKA) was done to evaluate the immune responses against serotypes 19F and 19A. RESULTS: According to the ELISA, all subjects had antibody titers > or =0.35 microg/mL for serotypes 19F and 19A in the booster and primary group and 83.0% and 66.7% in the control group, respectively. According to the OPKA, subjects with opsonic activity (> or =20) against serotypes 19F and 19A were 100% and 61.1% of the subjects in the booster group and 66.7% and 19.0% in the primary group, respectively. No subjects in the control group had opsonic antibodies against both serotypes. CONCLUSION: In conclusion, in children 12-23 months age who were previously vaccinated with PCV7, a cross-reactive immune response is elicited against serotype 19A after a primary series of 3 doses in a small proportion of subjects, and this response is amplified after booster vaccination.
Aged ; Antibodies ; Antibody Formation ; Child ; Enzyme-Linked Immunosorbent Assay ; Homicide ; Humans ; Immunization ; Pneumococcal Vaccines ; Streptococcus pneumoniae ; Vaccination ; Heptavalent Pneumococcal Conjugate Vaccine

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Alteration of CD4+CD25+Foxp3+ T cell level in Kawasaki disease.

Su Ye SOHN ; Young Wooh SONG ; Yun Ku YEO ; Yun Kyung KIM ; Gi Young JANG ; Chan Wook WOO ; Jung Hwa LEE ; Kwang Chul LEE

Korean Journal of Pediatrics.2011;54(4):157-162. doi:10.3345/kjp.2011.54.4.157

PURPOSE: Exaggerated pro-inflammatory reactions during the acute phase of Kawasaki disease (KD) suggest the role of immune dysregulation in the pathogenesis of KD. We investigated the profiles of T regulatory cells and their correlation with the clinical course of KD. METHODS: Peripheral blood mononuclear cells were collected from 17 KD patients during acute febrile and subacute afebrile phases. T cells expressing CD4, CD25, and Foxp3 were analyzed using flow cytometry, and the results were correlated with the clinical course of KD. RESULTS: The percentage of circulating CD4+CD25highFoxp3+ T cells among CD4+ T cells was significantly higher during the subacute afebrile phase than during the acute febrile phase (1.10%+/-1.22% vs. 0.55%+/-0.53%, P=0.049). Although levels of CD4+CD25lowFoxp3+ T cells and CD4+CD25-Foxp3+ T cells were only slightly altered, the percentage of CD4+CD25+Foxp3- T cells among CD4+ T cells was significantly lower during the subacute afebrile phase than during the acute febrile phase (2.96%+/-1.95% vs. 5.64%+/-5.69%, P=0.036). Consequently, the ratio of CD25highFoxp3+ T cells to CD25+Foxp3- T cells was higher during the subacute afebrile phase than during the acute febrile phase (0.45%+/-0.57% vs. 0.13%+/-0.13%, P=0.038). CONCLUSION: Decreased CD4+CD25highFoxp3+ T cells and/or an imbalanced ratio of CD4+CD25highFoxp3+ T cells to CD4+CD25+Foxp3- T cells might play a role in KD development. Considering that all KD patients were treated with intravenous immunoglobulin (IVIG), recovery of CD4+CD25highFoxp3+ T cells during the subacute afebrile phase could be a mechanism of IVIG.
Flow Cytometry ; Humans ; Immunoglobulins ; Immunoglobulins, Intravenous ; Mucocutaneous Lymph Node Syndrome ; T-Lymphocytes

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Impact factor of Korean Journal of Pediatrics on Korean Medical Citation Index and Science Citation Index of Web of Science.

Chong Woo BAE ; Sun Hee CHOI ; Man Yong HAN ; Yeong Ho RHA ; Young Jin LEE

Korean Journal of Pediatrics.2011;54(4):152-156. doi:10.3345/kjp.2011.54.4.152

PURPOSE: The total number of times a paper is cited, also known as the impact factor (IF) of a medical journal, is widely implied in evaluating the quality of a research paper. We evaluated the citation index data as an IF of Korean J Pediatr in Korean Medical Citation Index (KoMCI) and JCI of Web of Science. METHODS: We calculated the IF of Korean J Pediatr at KoMCI supervised by Korean Association of Medical Journal Editors. And we estimated the IF of Korean J Pediatr by the JCI of Web of Science although it was never officially reported. RESULTS: The IF of Korean J Pediatr on KoMCI has increased from 0.100 in the year 2000, to 0.205 in 2008, and 0.326 in 2009. Although the IF of Korean J Pediatr was 0.006 in 2005, 0.018 in 2006, 0.028 in 2008, 0.066 in 2009, and 0.018 in 2010 according to the JCI of Web of Science, the number of citations are steadily increasing. CONCLUSION: Understanding and realizing the current status will be a stepping stone for further improvement. The next objective of the Korean J Pediatr is to become registered in the SCI or SCIE. Increasing the IF according to the JCI of Web of Science is crucial in order to achieve this goal.
Pediatrics

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Recommendation for use of the newly introduced pneumococcal protein conjugate vaccines in Korea.

Eun Hwa CHOI ; Kyung Hyo KIM ; Yae Jean KIM ; Jong Hyun KIM ; Su Eun PARK ; Hoan Jong LEE ; Byung Wook EUN ; Dae Sun JO ; Kyong Min CHOI ; Young Jin HONG

Korean Journal of Pediatrics.2011;54(4):146-151. doi:10.3345/kjp.2011.54.4.146

Streptococcus pneumoniae remains a leading cause of invasive infections including bacteremia and meningitis, as well as mucosal infections such as otitis media and pneumonia among children and adults. The 7-valent pneumococcal conjugate vaccine (PCV7) was licensed for use among infants and young children in many countries including Korea. The routine use of PCV7 has resulted in a decreased incidence of invasive pneumococcal disease (IPD) by the vaccine serotypes among the vaccinees and substantial declines in IPD among unvaccinated populations such as older children and adults as well. In addition, there are increasing evidences to suggest that routine immunization with PCV7 is changing the epidemiology of pneumococcal diseases such as serotype distribution of IPD, nasopharyngeal colonization, and antibiotic resistance patterns. In contrast, there is an increase in the number of IPDs caused by nonvaccine serotypes, though it is much smaller than overall declines of vaccine serotype diseases. Several vaccines containing additional serotypes have been developed and tested clinically in order to expand the range of serotypes of Streptococcus pneumoniae. Recently two new pneumococcal protein conjugate vaccines, 10-valent pneumococcal conjugate vaccine (PCV10) and 13-valent pneumococcal conjugate vaccine (PCV13), have been approved for use in several countries including Korea. This report summarizes the recommendations approved by the Committee on Infectious Diseases, the Korean Pediatric Society.
Adult ; Bacteremia ; Child ; Colon ; Communicable Diseases ; Drug Resistance, Microbial ; Humans ; Immunization ; Incidence ; Infant ; Korea ; Meningitis ; Otitis Media ; Pneumonia ; Streptococcus pneumoniae ; Vaccines ; Vaccines, Conjugate

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Recommendation for the use of newly introduced Tdap vaccine in Korea.

Kyong Min CHOI ; Kyung Hyo KIM ; Yae Jean KIM ; Jong Hyun KIM ; Su Eun PARK ; Hoan Jong LEE ; Byung Wook EUN ; Dae Sun JO ; Eun Hwa CHOI ; Young Jin HONG

Korean Journal of Pediatrics.2011;54(4):141-145. doi:10.3345/kjp.2011.54.4.141

Pertussis is an acute respiratory infection characterized by paroxysmal cough and inspiratory whoop for over 2 weeks. The incidence of pertussis has decreased markedly after the introduction of DTwP/DTaP vaccine, but the incidence of pertussis has increased steadily among young infant and among adolescents and adults in many countries. Td vaccine was used in this age group but the increase in pertussis has lead to the development of a Tdap vaccine. The Tdap vaccine is a Td vaccine with a pertussis vaccine added and is thought to decrease the incidence and transmission of pertussis in the respective age group. In Korea, two products are approved by the KOREA FOOD & DRUG ADMINISTRATION, which are ADACEL(TM) (Sanofi-Pasteur, Totonto, Ontario, Canada) and BOOSTRIX(R) (GlaxoSmithKline Biologicals, Rixensart, Belgium) for those aged between 11-64. This report summarizes the recommendations approved by the Committee on Infectious Diseases, the Korean Pediatric Society.
Adolescent ; Adult ; Aged ; Communicable Diseases ; Cough ; Humans ; Incidence ; Infant ; Korea ; Ontario ; Pertussis Vaccine ; Whooping Cough

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Two cases of chronic pancreatitis associated with anomalous pancreaticobiliary ductal union and SPINK1 mutation.

Eun Sam RHO ; Earl KIM ; Hong KOH ; Han Wook YOO ; Beom Hee LEE ; Gu Hwan KIM

Korean Journal of Pediatrics.2013;56(5):227-230. doi:10.3345/kjp.2013.56.5.227

Chronic pancreatitis is a progressive inflammatory disease resulting from repeated episodes of acute pancreatitis that impair exocrine function and eventually produce endocrine insufficiency. Some causes of chronic pancreatitis appear to be associated with alterations in the serine-protease inhibitor, Kazal type 1 (SPINK1), cationic trypsinogen (PRSS1), and cystic fibrosis-transmembrane conductance regulator (CFTR) genes, or with structural disorders in the pancreaticobiliary ductal system, such as pancreatic divisum or anomalous pancreaticobiliary ductal union (APBDU). However, it is unusual to observe both genetic alteration and structural anomaly. Here, we report 2 cases with both APBDU and a mutation in the SPINK1 genes, and we discuss the implications of these findings in clinical practice.
Pancreatitis ; Pancreatitis, Chronic ; Trypsinogen

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A case of Hashimoto's encephalopathy presenting with seizures and psychosis.

Min Joo LEE ; Hae Sang LEE ; Jin Soon HWANG ; Da Eun JUNG

Korean Journal of Pediatrics.2012;55(3):111-113. doi:10.3345/kjp.2012.55.3.111

Hashimoto's encephalopathy (HE) is a rare, poorly understood, autoimmune disease characterized by symptoms of acute or subacute encephalopathy associated with increased anti-thyroid antibody levels. Here, we report a case of a 14-year-old girl with HE and briefly review the literature. The patient presented with acute mental changes and seizures, but no evidence of infectious encephalitis. In the acute stage, the seizures did not respond to conventional antiepileptic drugs, including valproic acid, phenytoin, and topiramate. The clinical course was complicated by the development of acute psychosis, including bipolar mood, insomnia, agitation, and hallucinations. The diagnosis of HE was supported by positive results for antithyroperoxidase and antithyroglobulin antibodies. Treatment with methylprednisolone was effective; her psychosis improved and the number of seizures decreased. HE is a serious but curable, condition, which might be underdiagnosed if not suspected. Anti-thyroid antibodies must be measured for the diagnosis. HE should be considered in patients with diverse neuropsychiatric manifestations.
Adolescent ; Antibodies ; Anticonvulsants ; Autoantibodies ; Autoimmune Diseases ; Brain Diseases ; Dihydroergotamine ; Infectious Encephalitis ; Fructose ; Hallucinations ; Hashimoto Disease ; Humans ; Methylprednisolone ; Phenytoin ; Psychotic Disorders ; Seizures ; Sleep Initiation and Maintenance Disorders ; Valproic Acid

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