Pyomyositis is a primary bacterial infection of the skeletal muscles. Although infection can affect any skeletal muscle, the large muscle groups such as the quadriceps or gluteal muscles are most often the focus of this disease, and most commonly the inflammation is focal, involving a single muscle. The mechanism of pyomyositis is poorly understood. The local mechanical trauma at the time of an incidental bacteremia is frequently postulated as a mechanism that could explain the high incidence of the disease in tropical areas and its male preponderance. Staphylococcus aureus is the most common organism responsible for pyomyositis. Mycobacterium tuberculosis primarily affects the lungs, and the prevalence of active pulmonary tuberculosis co-existing with musculoskeletal tuberculosis has been about 30 percent. We report here on a case of an otherwise healthy 17-month-old girl, who had tuberculous pyomyositis at the upper arm after the hepatitis A vaccination with no evidence of any coexistent active tuberculosis.
Arm ; Bacteremia ; Bacterial Infections ; Female ; Hepatitis A ; Humans ; Incidence ; Infant ; Inflammation ; Lung ; Male ; Muscle, Skeletal ; Muscles ; Mycobacterium tuberculosis* ; Mycobacterium* ; Prevalence ; Pyomyositis* ; Staphylococcus aureus ; Tuberculosis ; Tuberculosis, Pulmonary ; Vaccination

Hereditary pancreatitis is an autosomal dominant disease characterized by recurrent episodes of pancreatitis, often beginning in childhood, with a positive family history involving at least two other affected family members with no known other precipitating factors. Most forms of hereditary pancreatitis are caused by one of two common mutations, i.e., R122H in exon 3 and N29I in exon 2 of the cationic trypsinogen (CT) (PRSS1) gene, located on chromosome 7. The authors describe the case of a 15-year-old boy who had suffered from recurrent attacks of pancreatitis since age three. His mother and grandmother had chronic pancreatitis and diabetes mellitus. Mutation analysis was performed on the family due to the suspicion of hereditary pancreatitis. The CT gene was analyzed in DNA samples extracted from the peripheral blood of three family members, the mother, the proband, and the proband's sister. Two members of the family, the mother and the proband, were found to have a N29I mutation in the CT gene. The authors document the first family with hereditary pancreatitis associated with the N29I mutation in Korea.
Adolescent ; Chromosomes, Human, Pair 7 ; Diabetes Mellitus ; DNA ; Exons ; Humans ; Korea ; Male ; Mothers ; Pancreatitis* ; Pancreatitis, Chronic ; Precipitating Factors ; Siblings ; Trypsinogen*

Chylous ascites is a rare condition caused by various diseases and conditions that interfere with the abdominal or retroperitoneal lymphatics, and uncommonly it can manifest as a post-operative complication after abdominal, retroperitoneal or mediastinal surgery. Chylous ascites can be diagnosed by a high triglyceride content in ascites. The authors experienced a 5-year-old girl with congenital hepatic fibrosis who presented with chylous ascites after a splenorenal shunt operation, who was successfully managed by fasting and total parenteral nutrition, followed by a lipid-free diet with medium chain triglyceride supplementation. Here, the authors report this case of post-operative chylous ascites after a splenorenal shunt (Warren shunt) operation with a review of the pertinent literature.
Ascites ; Child* ; Child, Preschool ; Chylous Ascites* ; Diet ; Fasting ; Female ; Fibrosis* ; Humans ; Parenteral Nutrition, Total ; Splenorenal Shunt, Surgical* ; Triglycerides

PURPOSE: Because NELL2 expression is strictly restricted only in neurons in developing and post- differentiated neural tissues, it is thought to be involved in the neuronal differentiation during development and in the maintenance of neuronal physiology in the post-differentiated neurons. In this study, we examined whether NELL2 is involved in the regulation of cell cycle and apoptosis in the hippocampal neuroprogenitor HiB5 cells. METHODS: Effects of NELL2 on the cultured HiB5 cell numbers, DNA fragmentation, and proteins involved in the regulation of the cell cycle were measured. RESULTS: NELL2 induced a decrease in cell numbers and an increase in G1 phase arrest. Moreover, transfection of NELL2 resulted in an increase of DNA fragmentation that shows an evidence of apoptosis. Contents of proteins involved in the regulation of cell cycle were also changed by transfection of NELL2 expression vectors. CONCLUSION: This study suggests that NELL2 plays an important role in the regulation of cell cycle and apoptosis of neurons.
Apoptosis ; Cell Count ; Cell Cycle* ; DNA Fragmentation ; G1 Phase ; Neurons* ; Physiology ; Transfection

PURPOSE: Homocysteine is a strong and independent risk factor for cardiovascular disease. The deleterious effects of homocysteine included endothelial dysfunction, arterial intimal-medial thickening, wall stiffness and procoagulant activity. However, the precise mechanism responsible for homocysteine release in children with coronary artery disease is still unknown. The purpose of this study was to investigate serum homocysteine and tumor necrosis factor(TNF)-alpha levels and identify whether these levels had any association with the development of coronary artery lesions in Kawasaki disease(KD). METHODS: Serum homocysteine and TNF-alpha levels were measured in 24 KD patients(group 1, eight patients with normal coronary artery; group 2, 16 patients with coronary artery lesions) and 21 controls(group 3, 10 afebrile controls; group 4, 11 febrile controls). Blood samples were drawn from each study group before and after intravenous immunoglobulin(IVIG) therapy and in the convalescent stage. RESULTS: The homocysteine levels before IVIG therapy were significantly higher in group 1 than in group 3, and in group 2 than in group 3 and 4. The TNF-alpha levels before IVIG therapy were significantly higher in group 2 than group 3 and 4. Serum homocysteine and TNF-alpha levels were highest in group 2 before IVIG therapy. In the acute KD patients, serum homocysteine levels correlated significantly with TNF-alpha levels. CONCLUSION: The increased serum homocysteine levels in the acute stage increase the susceptibility to coronary arterial lesions in KD. TNF-alpha may also play an important role in the formation of coronary arterial lesions in KD.
Cardiovascular Diseases ; Child ; Coronary Artery Disease ; Coronary Vessels ; Homocysteine* ; Humans ; Immunoglobulins, Intravenous ; Mucocutaneous Lymph Node Syndrome* ; Necrosis ; Risk Factors ; Tumor Necrosis Factor-alpha*

PURPOSE: Refractory status epilepticus(RSE) is a serious neurological emergency in children. The mortality is high and the neurological outcome is not good. This study aimed to evaluate the clinical significance of cerebrospinal fluid(CSF) pleocytosis in refractory status epilepticus in children. METHODS: From January 1999 to January 2006, 25 out of 37 children with refractory status epilepticus had spinal tapping. We retrospectively analyzed the data from these children's medical records. We compared the results between groups with and without CSF pleocytosis, and between a group with first seizure and a group with epilepsy . RESULT: Six out of 25 children had CSF pleocytosis. The group without CSF pleocytosis had a higher mortality rate and required higher doses of antiepileptic drugs as compared with the group with CSF pleocytosis. The group with CSF pleocytosis had much worse neurologic segualae. However, except for the children with CNS infection, the overall prognosis between the group with and without CSF pleocytosis was not significantly different. All children with CSF pleocytosis came in with first seizures. CONCLUSION: In children with RSE, a CSF study must be perfomed as soon as possible to exclude the possibility of CNS infection. A CSF study is even more important in cases of first seizure or CNS infection suspected. Mild CSF pleocytosis without evidence of infection does not seem to affect the prognosis, so physicians should therefore be more cautious in selecting antibacterial or antiviral agents for it.
Anticonvulsants ; Antiviral Agents ; Cerebrospinal Fluid* ; Child ; Emergencies ; Epilepsy ; Humans ; Leukocytosis* ; Medical Records ; Mortality ; Prognosis ; Retrospective Studies ; Seizures ; Spinal Puncture ; Status Epilepticus*

PURPOSE: The purpose of this study was to evaluate factors affecting hematologic recovery and infection in high-dose chemotherapy(HDCT) and autologous stem cell transplantation(ASCT) in patients with high-risk solid tumor. METHODS: From January 2004 to December 2005, 72 HDCTs and ASCTs were applied to children with high-risk solid tumor at Samsung Medical Center. Medical records of these 72 HDCTs and ASCTs were retrospectively analyzed. RESULTS: The single most powerful predictor of neutrophil and platelet recovery was the number of transplanted CD34+ cells. The duration of high fever was significantly longer in young patients, in patients treated with total body irradiation and/or thiotepa, and in patients transplanted with lower CD34+ cell dose(<2x10(6)/kg). However, the difference in the duration of high fever according to the number of CD34+ cells was not clinically significant. CONCLUSION: Findings in this study suggest that HDCT and ASCT with low CD34+ cell dose is clinically feasible despite delayed hematologic recovery, especially at a dose >1x10(6)/kg per transplantation. Therefore, it is important not to defer the appropriate time for HDCT for an additional collection of hematopoietic stem cells if the number of collected CD34+ cells is >1x10(6)/kg per transplantation.
Blood Platelets ; Child ; Drug Therapy* ; Fever ; Hematopoietic Stem Cells ; Humans ; Medical Records ; Neutrophils ; Retrospective Studies ; Stem Cell Transplantation* ; Stem Cells* ; Thiotepa ; Whole-Body Irradiation

PURPOSE: This study was performed to assess how a fetal diagnosis of congenital heart disease affects parents, as regards pregnancy management and care of infants after birth. METHODS: Database search to find out abnormal fetal echocardiography performed at Seoul National University Children's Hospital from July 1988 to June 2003 revealed 370 examinations. After excluding both arrhythmias without structural cardiac disease and multiple pregnancies, 299 pregnancies remained and this data formed the basis of this analysis. We retrospectively reviewed the medical records with special attention to pregnancy outcomes and also tried to find out factors influencing parental decisions on whether to continue or terminate pregnancy. RESULTS: In this study, the mean gestation age at diagnosis was 28+/-6.0 weeks. The mean age of mothers was 30+/-3.9 years old. Younger gestational ages at diagnosis(P=0.000), more severe grades of fetal heart disease(P=0.002) and younger mothers(P=0.014) correlated with terminations of pregnanies. But the grades of fetal status, the grades of associated anomaly, whether in-vitro-fertilization was carried out or not and numbers of previous children were not significant. CONCLUSION: This study found that the earlier gestational ages at diagnosis, younger maternal age and higher grades of fetal heart disease tended to lead parent to select abortions. Fetal echocardiographies were performed too late. Moreover Koreans have a biased view that malformation is a something incurable and a tragedy not only to oneself, but also to a family. So parents select terminations of pregnancy, even in curable cases. This is very unethical.
Abortion, Criminal ; Arrhythmias, Cardiac ; Bias (Epidemiology) ; Child ; Diagnosis* ; Echocardiography ; Ethics ; Female ; Fetal Heart ; Gestational Age ; Heart Defects, Congenital* ; Heart Diseases ; Humans ; Infant ; Maternal Age ; Medical Records ; Mothers ; Parents* ; Parturition ; Pregnancy ; Pregnancy Outcome ; Pregnancy, Multiple ; Retrospective Studies ; Seoul

PURPOSE: The purpose of this study was to examine the clinical courses and long-term outcomes of children with Allagille syndrome in Korea, and to evaluate the prognostic potentials of identified variables. METHODS: We reviewed the clinical manifestations and outcomes of 30 children with Alagille syndrome, investigated from 1984 to 2006 until the end of this study (defined as death or last visit; mean follow-up : 5 years). RESULTS: Cholestasis occurred in 100 percent, cardiovascular abnormalities in 83.3 percent, butterfly vertebrae in 30.0 percent, posterior embryotoxon in 43.3 percent, and a characteristic facial appearance in 100 percent. At study conclusion, of these 30 patients, eight had died (26.7 percent); six related to Alagille syndrome. Five patients died of a liver disease complication. Liver transplantation was carried out in five of the 30 patients (16.7 percent) and one of these died due to hyperacute rejection. At age two, cholestasis improved in 17 of the 30 patients. Those who had severe cholestasis at 2 years of age tended to have a complication, such as liver cirrhosis or liver transplantation, or to have died. CONCLUSION: Hepatic complications account for the most mortalities in patients with Alagille syndrome. Careful and complete assessments should be made in children who have cholestasis at 2 years of age. Further investigations of more cases are required.
Alagille Syndrome* ; Butterflies ; Cardiovascular Abnormalities ; Child ; Cholestasis ; Follow-Up Studies ; Humans ; Korea* ; Liver Cirrhosis ; Liver Diseases ; Liver Transplantation ; Mortality ; Spine

PURPOSE: The purpose of this study was to research whether measurement of cow's milk specific IgE on the newborn would be helpful in the diagnosis of cow's milk allergy. We tried to find out the relation between cow's milk specific IgE and other allergy diseases by following up cases. METHODS: We reviewed clinical features of 87 episodes in infants less than 4 weeks old who were positive in cow's milk specific IgE test. For the study group, history taking, physical examinations, elimination and cow's milk specific IgE tests were carried out. We investigated the connection among cow' milk specific IgE, allergic disease and family history in 40 of 87 patients we could follow up on. RESULTS: The mean age of the study group was 17.2+/-5.4 days. The subjects were classified in four groups according into allergens : 87 milk allergy positive patients, 24 casein positive, 38 alpha-lactoalbumin positive, and 75 beta-lactoglobulin positive. The number of patients who had follow-ups for more than 6 months to was 40(45.9 percent). The patients whose parents had allergic disease numberred 10(25 percent). Fiften patients had allergic diseases, 4 had asthma and 11 atopic dermatitis. According to the follow-up study, there is a significant relation between casein positive patients and allergic disease. But there is no statistical and significant relation between cow's milk specific IgE and a family history of allergic disease. CONCLUSION: For the newborn babies, elimination tests and cow's milk specific IgE tests can be useful in the diagnosis of IgE-mediated or mixed milk allergies.
Allergens ; Asthma ; Caseins ; Dermatitis, Atopic ; Diagnosis ; Follow-Up Studies ; Humans ; Hypersensitivity ; Immunoglobulin E* ; Infant ; Infant, Newborn* ; Lactoglobulins ; Milk Hypersensitivity ; Milk* ; Parents ; Physical Examination