No abstract available.
Granuloma* ; Leprosy, Lepromatous*

Submucosal infiltration and the topical application of epinephrine as a vasoconstrictor produce excellent hemostasis during surgery. The hemodynamic effects of epinephrine have been documented in numerous studies. However, its metabolic effects (especially during surgery) have been seldom recognized clinically. We report two cases of significant metabolic effects (including lactic acidosis and hyperglycemia) as well as hemodynamic effects in healthy patients undergoing orthognathic surgery with general anesthesia. Epinephrine can induce glycolysis and pyruvate generation, which result in lactic acidosis, via β2-adrenergic receptors. Therefore, careful perioperative observation for changes in plasma lactate and glucose levels along with intensive monitoring of vital signs should be carried out when epinephrine is excessively used as a vasoconstrictor during surgery.
Acidosis, Lactic* ; Administration, Topical ; Anesthesia, General ; Anesthesia, Local ; Epinephrine ; Glucose ; Glycolysis ; Hemodynamics ; Hemostasis ; Humans ; Lactic Acid ; Orthognathic Surgery* ; Plasma ; Pyruvic Acid ; Vital Signs

BACKGROUND: Monitoring of "Depth of anesthesia" is an ongoing problem in anaesthesia. In this study, the author has compared the bispectral index (BIS) and Anemon monitor for monitoring depth of anesthesia in propofol or isoflurane anesthesia. METHODS: Anemon-1 and BIS index were obtained from 24 patients (ASA I, II) during general anesthesia with propofol or isoflurane. For patients in the propofol group, anesthesia was induced with fentanyl 100ng followed by propofol 2 mg/Kg. For patients in the isoflurane group, anesthesia was induced with thiopental 5 mg/Kg. The author observed changes of these values at 5 major times: before induction, during induction, after induction, at sKin incision, before extubation, after extubation. RESULTS: The anemon index showed a significant increase during induction (propofol group: 86.9 +/- 26.4, isoflurane group: 106.0 +/- 18.6) and at sKin incision (propofol group: 89.9 +/- 22.7, isoflurane group: 92.0 +/- 23.1), but this did not correlate with the level of consciousness. The BIS index showed a significant decrease in the score after induction (propofol group: 55.0 +/- 9.6, isoflurane group: 61.0 +/- 17.2), but no response to surgical stimuli. CONCLUSIONS: BIS had a good correlation with level of consciousness. The Anemon-1 index was recognized to reflect invasive stimulus. As the BIS and Anemon-1 had no correlation, it was not possible to assume changes of each index from the other. Both the anemon-1 index and BIS are useful to monitor the anesthesia level during surgery.
Anesthesia ; Anesthesia, General ; Consciousness ; Fentanyl ; Humans ; Isoflurane ; Propofol ; Skin ; Thiopental

Split hand and split foot(SHSF) is a human developmental defect characterized by missing digits, fusion of remaining digits, and a deep median cleft resulting in a clawlike appearance of the hands and feets. SHSF is usually inherited in an autosomer dominant fashion. The incidence of SHSF is between 1/10,000 and 1/90,000. Thirteen cases of SHSF and chromosomal aberrations involving 7q21-22 have been described so far in the world. We experienced a case of typical tetramelic SHSF in neonate. Chromosome studies showed a pericentric inversion of chromosome 7:46,XY,inv(7) (p22q22). Inspection of the extremities and chromosome studies in the parents were normal.
Chromosome Aberrations ; Chromosomes, Human, Pair 7* ; Extremities ; Foot* ; Hand* ; Human Development ; Humans ; Incidence ; Infant, Newborn ; Parents

No abstract available.
Dermatitis, Atopic* ; Food Hypersensitivity ; Milk Hypersensitivity* ; Milk*

No abstract available.
Cryotherapy* ; Pemphigus, Benign Familial*

Rhizomelic chondrodysplasia punctata(RCDP) is a rare autosomal recessive disorder clinically characterized by symmetrical shortening of the proximal limbs, contractures of joints, a typical dysmorphic face, cataracts, and itchyosis. Patients with RCDP can be subdivided into three subgroups based on biochemical analysis and complementation studies. RCDP type I results from mutations in the PEX7 gene encoding the peroxisomal targeting signal type II(PST2) receptors and presents with both a defect in plasmalogen biosynthesis and phytanic acid oxidation. RCDP type II is deficient in the activity of dihydroxyacetonephosphate acyltransferase(DHAP-AT). RCDP type III is deficient in alkyl-dihydroxyacetonephosphate synthase(alkyl-DHAP). We report a case of RCDP type I which was confirmed with biochemical study, fibroblast culture, and gene study.
Cataract ; Chondrodysplasia Punctata, Rhizomelic* ; Complement System Proteins ; Contracture ; Extremities ; Fibroblasts ; Humans ; Joints ; Phytanic Acid

PURPOSE: This study was aimed to evaluate the clinical features of hypoxic ischemic encephalopathy(HIE) in children with and without seizures. METHODS: Fifty five children who had been diagnosed as HIE at Inha University Hospital from June 1999 to December 2011 were enrolled in this study. Subjects were divided into two groups by the presence of seizures and their medical records were retrospectively analyzed. RESULTS: Among the 55 cases, 34 patients (61.8%) had seizures, while 17 patients (32.2%) did not have them. Male to female ratio was 1:1 for the 'seizure' group and 2.5:1 for the 'no seizure' group. The onset age was 9.7 months (range: 0-158 months) for the 'seizure' group and 10 months (range : 0-108 months) for the 'no seizure' group. The most common risk factor was birth asphyxia (17.7%) for the 'seizure' group, and prematurity (23.8%) for the 'no seizure' group. The most common symptom other than seizure was respiratory arrest for both groups. On radiologic imaging studies of the brain, main causative lesion was most commonly observed in the cerebral cortex in both groups. The neurologic deficits or death were detected in 67.7% of the 'seizure' group, and 76.3% of the 'no seizure' group. There were no statistically significant differences in risk factors between the two groups. CONCLUSION: Although the characteristics between patients with and without seizures from HIE revealed no significant differences, HIE still can result in death or permanent disability in children. Therefore, permanent brain damage may be minimized by early suspicion and treatment in these patients.
Age of Onset ; Asphyxia ; Brain ; Cerebral Cortex ; Child* ; Female ; Humans ; Hypoxia-Ischemia, Brain* ; Male ; Medical Records ; Neurologic Manifestations ; Parturition ; Retrospective Studies ; Risk Factors ; Seizures*

PURPOSE: To evaluate the clinical characteristics of infants and young children who had developmental delay without delayed myelination and dysmyelination. METHODS: We retrospectively reviewed 59 cases of developmental disability between July 1996 and June 2001 at Inha University Hospital. Twenty-eight patients showed normal myelination(Group I), while thirty-one patients showed delayed myelination(Group II) by brain MRI. The following clinical records and diagnostic procedures were performed; birth history, head size, neurological examination, developmental assortment test, brain stem auditory evoked potential, visual evoked potential, electroencephalogram, chromosomal study, metabolic screening tests, and clinical psychologic tests. RESULTS: There were no significant differences of sex, age, gestational period, birth weight, etiology, and seizures between group I and II. The incidences of cerebral palsy and abnormal background activity of EEG in group II were significantly higher than those in group I. In group I, mental retardations or speech disorders were higher than motor handicaps. In group II, motor handicaps were higher than mental retardations or speech disorders. In both groups, cases accompanied with seizures, have a tendency of intractability. CONCLUSION: Characteristics of developmentally delayed patients with normal myelination by brain MRI were not significantly different from characteristics of patients with delayed myelination. Even though infants and young children with the developmental delay showed normal findings by brain MRI, they are likely to be accompanied by severe development retardation.
Birth Weight ; Brain* ; Cerebral Palsy ; Child* ; Developmental Disabilities ; Electroencephalography ; Evoked Potentials, Auditory, Brain Stem ; Evoked Potentials, Visual ; Gestational Age ; Head ; Humans ; Incidence ; Infant* ; Magnetic Resonance Imaging* ; Mass Screening ; Myelin Sheath* ; Neurologic Examination ; Psychological Tests ; Reproductive History ; Retrospective Studies ; Seizures ; Speech Disorders

Infantile spasm is an age-related refractory epilepsy. Topiramate is a new anticonvulsant with multiple mechanisms of action, and it may be effective for treating pediatric epilepsies. To evaluate the efficacy and tolerability of first-line topiramate treatment for infantile spasm, 20 patients received topiramate monotherapy during this study. They were treated with an initial dose of 1mg/kg/day, with a progressive titration of 1 mg/kg a week until their spasms were controlled and a maximum dose of 12mg/kg/day was achieved. The evaluation of the treatment efficacy was based on the spasm frequency data that was obtained by the scalp and video-EEG, and by the parental count of spasm. Thirty percent of the subjects became spasm-free during the study. Six of 20 subjects (30%) had cessation of spasm and disappearance of hypsarrhythmia as seen via the video EEG; four (50%) of eight idiopathic patients had a response, whereas two (17%) of 12 patients with symptomatic infantile spasm responded. Seventy of the patients, including the spasm-free patients, had a reduction in their seizure frequency of more than 50%, and 10% of the patients had a reduction in their seizure frequency of less than 50%. The clusters of spasm frequency decreased from 10.6 +/- 8.5 to 3.5 +/- 1.4 clusters/day. Topiramate is effective and tolerated in those patients suffering from infantile spasm. Our results suggest that this drug should be considered as a new first-line drug for treating infantile spasm.
Treatment Outcome ; Spasms, Infantile/*drug therapy ; Male ; Infant ; Humans ; Fructose/*analogs & derivatives/therapeutic use ; Female ; Electroencephalography ; Child, Preschool ; Anticonvulsants/*therapeutic use ; Age of Onset