No abstract available.
Neuroblastoma*

Anti-HLA antibody related neonatal thrombocytopenia is an uncommon disorder caused by platelet antigen incompatibility between mother and fetus in Korea. Mothers who lack the specific platelet antigen produce the IgG against the platelet antigen which the fetus inherits from the father. These IgG antibodies are then transported across the placenta into the fetal circulation where they lead to the destruction of fetal platelets. We report a case of neonatal alloimmune thrombocytopenia related with anti-HLA antibody in second baby of dizygotic twin who had petechia on trunk and platelet count 43,000/mm. Initially, mother and twin showed the positives in antiplatelet antibodies. In microlym- phocytotoxic test at 6 months of age, anti-HLA antibodies was negative in twins but anti-HLA A2, A24 was positive in their mother. The patient was treated with intravenous immunoglobulin and clinically improved and her platelet count was norrnalized.
Antibodies ; Blood Platelets ; Fathers ; Fetus ; Humans ; Immunoglobulin G ; Immunoglobulins ; Infant, Newborn ; Korea ; Mothers ; Placenta ; Platelet Count ; Thrombocytopenia, Neonatal Alloimmune ; Twins ; Twins, Dizygotic

PURPOSES: A cross-sectional study on serum lipids in school children and adolescents was carried out in two different areas in 2002. We studied to determine of the difference in lipids level of children between urban and rural areas and changes of cholesterol levels compared to those of early- 1990 studies. METHODS: A cross-sectional study on serum lipids in school children and adolescents was carried out in two different areas, Ansan and Paju. The number of children in Ansan was 835, that in Paju was 233. After fasting for 12 hours, serum total choleterol, high density lipoprotein(HDL) cholesterol, low density lipoprotein(LDL) cholsterol, triglyceride were measured. RESULTS: The mean of total serum cholesterol level was 176.48+/-27.70 mg/dL. The mean of HDL cholesterol was 61.42+/-12.38 mg/dL, LDL cholesterol was 94.14+/-24.45 mg/dL, and triglyceride was 93.09+/-45.96 mg/dL. The means of total serum total cholesterol and lipoproteins of children and adolescents were not different significantly in Ansan and Paju. The prevalences of children and adolescence with abnormal high cholesterol level(> or =200 mg/dL) and abnormal LDL cholesterol level(> or =130 mg/dL) were also not different in two areas. CONCLUSION: Our results suggest that there were no significant differences in serum lipid level and the prevalence of school children and adolescence with hyperlipidemia between urban and rural area and mean lipid levels of school children and adolescence were increased during the past decade.
Adolescent* ; Child* ; Cholesterol ; Cholesterol, HDL ; Cholesterol, LDL ; Cross-Sectional Studies ; Fasting ; Gyeonggi-do ; Humans ; Hyperlipidemias ; Lipoproteins ; Prevalence ; Triglycerides

BACKGROUND: Woake's syndrome (WS) is a recurrent nasal polyposis, accompanied by broadening of the nose, frontal sinus aplasia, dyscrinia, and bronchiectasis. There has been no previous report on anesthetic management in patients with WS. CASE: We describe a case involving a 13-year-old male patient with WS who was scheduled for septorhinoplasty for necrotic ethmoiditis. Anesthesia was induced and maintained with propofol and remifentanil using a target-controlled infusion device. The anesthetic considerations of this rare syndrome and the advantages of an intravenous infusion method over local and volatile anesthesia for these patients are discussed. We report on caveats, such as pulmonary dysfunction during the anesthetic management, and nasal structural problems encountered in WS patients. CONCLUSIONS: Given that conventional inhaled anesthesia reduces ciliary movement and that local anesthesia with sedative has several disadvantages, perioperative control and precautions against respiratory infections by using antibiotics, and preventing cilio-depressant actions, are important for anesthetic management.
Adolescent ; Anesthesia ; Anesthesia, Local ; Anti-Bacterial Agents ; Bronchiectasis ; Frontal Sinus ; Humans ; Infusions, Intravenous ; Male ; Methods ; Nasal Polyps ; Nose ; Propofol ; Respiratory Tract Infections

BACKGROUND: Woake's syndrome (WS) is a recurrent nasal polyposis, accompanied by broadening of the nose, frontal sinus aplasia, dyscrinia, and bronchiectasis. There has been no previous report on anesthetic management in patients with WS.CASE: We describe a case involving a 13-year-old male patient with WS who was scheduled for septorhinoplasty for necrotic ethmoiditis. Anesthesia was induced and maintained with propofol and remifentanil using a target-controlled infusion device. The anesthetic considerations of this rare syndrome and the advantages of an intravenous infusion method over local and volatile anesthesia for these patients are discussed. We report on caveats, such as pulmonary dysfunction during the anesthetic management, and nasal structural problems encountered in WS patients. CONCLUSIONS Given that conventional inhaled anesthesia reduces ciliary movement and that local anesthesia with sedative has several disadvantages, perioperative control and precautions against respiratory infections by using antibiotics, and preventing cilio-depressant actions, are important for anesthetic management.

Arthrogryposis-renal dysfunction-cholestasis (ARC) syndrome is a rare autosomal recessive multisystemic disease that is associated with the liver, kidney, skin, and central nervous and musculoskeletal systems. ARC occurs as a result of mutations in the VPS33B (Vacuolar protein sorting 33 homolog B) or VIPAR (VPS33B interacting protein, apical-basolateral polarity regulator) genes. A female infant presented with neonatal cholestasis with a severe clinical outcome. She was diagnosed with ARC syndrome using targeted exome sequencing (TES). Exome sequencing revealed compound heterozygous mutations, c.707A>T and c.239+5G>A, in VPS33B, where c.707A>T was a novel variant; the resultant functional protein defects were predicted via in silico analysis. c.239+5G>A, a pathogenic mutation that affects splicing, is found in less than 0.1% of the general population. Invasive techniques, such as liver biopsies, did not contribute to a differential diagnosis of ARC syndrome; thus, early TES together with clinical presentations constituted an apparently accurate diagnostic procedure.
Biopsy ; Cholestasis ; Computer Simulation ; Diagnosis, Differential ; Exome ; Female ; Humans ; Infant ; Kidney ; Liver ; Musculoskeletal System ; Protein Transport ; Skin

CHARGE syndrome is a rare genetic disorder with CHD7 gene mutation. CHARGE is an acronym for coloboma (C), heart disease (H), atresia of choanae (A), retardation of growth (R), genitourinary malformation (G), and ear abnormalities (E). Patients with CHARGE syndrome need to undergo many surgeries due to their various congenital anomalies. Since airway abnormalities frequently accompany CHARGE syndrome, general anesthesia remains a challenge. Here we report a case of difficult intubation in a 35-month-old boy with CHARGE syndrome during general anesthesia and the experience of successful intubation using D-blade of C-MAC® video laryngoscope.
Airway Management ; Anesthesia, General ; CHARGE Syndrome* ; Child* ; Child, Preschool ; Coloboma ; Ear ; Heart Diseases ; Humans ; Intubation* ; Laryngoscopes* ; Male ; Nasopharynx ; Pediatrics

Near-infrared spectroscopy is a device used to determine whether traumatic intracranial hemorrhage has occurred and is primarily used for screening in emergency situations. In this study we examined the applicability of this equipment in postmortem inspection. This study included 124 autopsy cases and 59 postmortem inspection cases performed in the National Forensic Service from July 2017 to October 2018. We carried out the test using Infrascanner Model 2000 (Infrascan Inc.). Autopsy cases were divided into four groups (epidural hemorrhage or subdural hemorrhage group, traumatic subarachnoid hemorrhage or cerebral contusion group, nontraumatic intracerebral hemorrhage group, and control group) and analyzed. There was no difference in the test results according to the presence and type of intracranial hemorrhage. The possibility that variables related to postmortem change affected the test results was considered. In conclusion, this study confirmed that near-infrared spectroscopy is not suitable for the detection of traumatic intracranial hemorrhage in postmortem inspection.

Near-infrared spectroscopy is a device used to determine whether traumatic intracranial hemorrhage has occurred and is primarily used for screening in emergency situations. In this study we examined the applicability of this equipment in postmortem inspection. This study included 124 autopsy cases and 59 postmortem inspection cases performed in the National Forensic Service from July 2017 to October 2018. We carried out the test using Infrascanner Model 2000 (Infrascan Inc.). Autopsy cases were divided into four groups (epidural hemorrhage or subdural hemorrhage group, traumatic subarachnoid hemorrhage or cerebral contusion group, nontraumatic intracerebral hemorrhage group, and control group) and analyzed. There was no difference in the test results according to the presence and type of intracranial hemorrhage. The possibility that variables related to postmortem change affected the test results was considered. In conclusion, this study confirmed that near-infrared spectroscopy is not suitable for the detection of traumatic intracranial hemorrhage in postmortem inspection.

Near-infrared spectroscopy is a device used to determine whether traumatic intracranial hemorrhage has occurred and is primarily used for screening in emergency situations. In this study we examined the applicability of this equipment in postmortem inspection. This study included 124 autopsy cases and 59 postmortem inspection cases performed in the National Forensic Service from July 2017 to October 2018. We carried out the test using Infrascanner Model 2000 (Infrascan Inc.). Autopsy cases were divided into four groups (epidural hemorrhage or subdural hemorrhage group, traumatic subarachnoid hemorrhage or cerebral contusion group, nontraumatic intracerebral hemorrhage group, and control group) and analyzed. There was no difference in the test results according to the presence and type of intracranial hemorrhage. The possibility that variables related to postmortem change affected the test results was considered. In conclusion, this study confirmed that near-infrared spectroscopy is not suitable for the detection of traumatic intracranial hemorrhage in postmortem inspection.