No abstract available.

Osteopetrosis or albers-Schonberg disease is extremely rare disease. It is characterized by osteoporosis, stunted growth, deformity, increased likelihood of fractures, also patients suffers anemia, recurrent infections and hepatosplenomegaly. However, we recently came upon a 14-month-old female as the 1st child of osteopetrosis with hydrocephalus and rickets. She has the typical symptoms such as nystagmus, osteosclerosis -especially in skull. Brain Magnetic Resonance Imaging (MRI), MRI shows hydrocephalus and x-ray finding are consistent with rickets. This is the first report of osteopetrosis with hydrocephalus and rickets in Korea by pediatrician.
Anemia ; Brain ; Child ; Congenital Abnormalities ; Female ; Humans ; Hydrocephalus* ; Infant ; Korea ; Magnetic Resonance Imaging ; Osteopetrosis* ; Osteoporosis ; Osteosclerosis ; Rare Diseases ; Rickets* ; Skull

Although liver metastasis is commonly found in cancer patients, fulminant hepatic failure secondary to diffuse cancer infiltration into the liver is rare. Liver metastasis-induced fulminant hepatic failure has been reported in patients with primary cancer of the gastrointestinal tract, breast and uroepithelium, and in patients with melanoma and hematologic malignancy. Small cell lung cancer is so highly invasive that hepatic metastasis is common, but rapid progression to fulminant hepatic failure is extremely rare. We report here on a case of a patient who died because of rapid progression to fulminant hepatic failure as a result of hepatic metastasis of small cell lung carcinoma.
Aged ; Carcinoma, Small Cell/complications/pathology/*secondary ; Female ; Humans ; Liver Failure, Acute/diagnosis/*etiology ; Liver Neoplasms/complications/pathology/*secondary ; Lung Neoplasms/complications/*pathology ; Neoplasm Invasiveness ; Tomography, X-Ray Computed

Paroxysmal kinesigenic dyskinesia (PKD), previously referred to as movement-provoked seizures, is a rare neurological condition that is characterized by short duration dystonic or choreoathetotic movements precipitated by sudden movement, a change in position or hyperventilation. It can be difficult to distinguish this syndrome from seizures. We reported on three brothers in one family all of whom developed abnormal involuntary dystonic or choreoathetotic movement with a tingling or stiffness sensory aura. Evaluations of the patients included general physical examinations, endoclinologic, metabolic studies, chromosomal analysis, video electroencephalograms and brain MRI imaging. All of these studies were normal except for an arachnoid cyst found in one patient. All symptoms showed excellent response to oxcarbamazepine (Trileptal(R)) or carbamazepine. Use of the video electroencephalogram can help differentiate familial PKD from seizures.
Arachnoid ; Brain ; Carbamazepine ; Dyskinesias* ; Electroencephalography ; Epilepsy ; Humans ; Hyperventilation ; Magnetic Resonance Imaging ; Physical Examination ; Seizures ; Siblings*

The authors describe the use of a self-expandable stent in a temporary deployment for treatment of a very wide-neck A1 segment of anterior cerebral artery (ACA) aneurysm following incomplete clipping. A 39-year-old hypertensive man presenting with seizure-like movement underwent computed tomography, which showed acute subarachnoid hemorrhage and an A1 segment of ACA aneurysm with superior and inferior projection. He underwent surgical clipping of the aneurysm, but superior and posterior portion of wide-neck aneurysm remained. We decided to treat the remnant aneurysm using an endovascular modality. After selection of the aneurysm, coil packing was performed assisted by the temporary semi-jailing technique. The Enterprise stent (Cordis Neurovascular, Miami, FL, USA) was deployed and recaptured repeatedly for angiography to ensure safety of the small caliber parent artery. Successful semi-deployment and recapture of the stent allowed subtotal coil occlusion of the aneurysm with good anatomic and clinical results. No complications were encountered. The stent could be recaptured up to the point where the proximal end of the stent marker was aligned with distal marker band of the microcatheter, approximately 70% of the stent length. The temporary semi-jailing technique is feasible for wide-neck aneurysm with small caliber parent artery.
Aneurysm ; Angiography ; Anterior Cerebral Artery ; Arteries ; Dietary Sucrose ; Humans ; Intracranial Aneurysm ; Parents ; Stents ; Subarachnoid Hemorrhage ; Surgical Instruments

STUDY DESIGN: A literature review on the radiologic findings of pelvic parameters for treatment of spinal deformity OBJECTIVES: This review examines sagittal spine alignment, pelvic parameters, and methods for assessing alignment, and examines the relationships among all of these parameters to understand spinal deformity. SUMMARY OF LITERATURE REVIEW: Understanding the main pelvic and sagittal spinal parameters and recognizing their correlation is imperative in the diagnosis and treatment of various spinal disorders. MATERIALS AND METHODS: Review of the literature. RESULTS: As spinal and pelvic parameters tend to have a strong correlation, it is essential to measure not only spinal parameters but also pelvic parameters in analyzing sagittal balance. Degenerative changes have the potential to greatly disrupt the normal curvature of the spine, leading to sagittal malalignment. Analysis of sagittal balance is crucial to optimizing the management of spinal diseases. Improvement in surgical outcomes may be achieved through better understanding of radiographic spino-pelvic parameters and their association with deformity. CONCLUSIONS: Understanding spinal and pelvic parameters raises awareness of the relationship among alignment and balance, the soft tissue envelope, and compensatory mechanisms, which will, in turn, provide a more comprehensive understanding of the nature of spinal deformity and the modalities with which it is treated.
Congenital Abnormalities ; Diagnosis ; Spinal Diseases ; Spine

A total of 152 strains of Streptococcus pyogenes were isolated from patients with pharyngitis, scarlet fever, skin infection, or invasive streptococcal infections in Seoul, Korea from January 1988 to December 1999. All isolates were epidemiologically characterized to decide phenotypes by T protein serotype and serum opacity factor (OF) detection. Genetic diversity of the isolates were analyzed by emm genotyping and pulsed-field gel electrophoresis (PFGE). T protein serotype showed 17 kinds in distribution and T12 (40.1% of study strains), T4 (19.1%), and T1 (7.9%) were the prevalent ones. When sources of S. pyogenes isolates were analyzed by T serotype distribution, T12 type was predominant in pharyngitis and skin infection isolates which contributed to 30 strains (49.2%) and 11 strains (18.0%), respectively. When T serotype of S. pyogenes isolates were analyzed by emm genotype distribution, of the 61 isolates of T12 type, 48 strains (78.7%) belonged to the emm type 12 (M12) and of the 29 isolates of T4 type, 27 strains (93.1%) belonged to the emm genotype 4 (M4). PFGE of genomic DNA of different emm genotype (emm12, emm4 and emm1) showed distinctive patterns. When the DNA of same emm gene type isolates were analyzed genetic relatedness by PFGE pattern, emm4, emm1, and emm12 types showed over 90%, 75%, and 70% of genetic similarity, respectively. Therefore, it was suggested that these emm genotype isolates were closely related genetically whereas among the isolates of other emm genotypes showed less than 30% of genetic similarity. Show genotypes are more diverse in comparison with phenotypes. In even epidemiologically unrealated isolates, genetic subtypes appeared correlated. The phenotypic and genotypic analysis used in the study were discriminative and appropriate for epidemiological study of S. pyogenes.
DNA ; Electrophoresis, Gel, Pulsed-Field ; Epidemiologic Studies ; Genetic Variation ; Genotype ; Humans ; Korea* ; Pharyngitis ; Phenotype ; Scarlet Fever ; Seoul* ; Skin ; Streptococcal Infections ; Streptococcus pyogenes* ; Streptococcus*

PURPOSE: Immune thrombocytopenic purpura (ITP) is divided into acute and chronic forms. Unfortunately, there have been no known specific laboratory or clinical predictors for the diagnosis of chronic ITP. This study was performed to elucidate the prognostic significance of various clinical and laboratory parameters, including reticulated platelet percentage. METHODS: We retrospectively analyzed 60 patients who were diagnosed as ITP at the Department of Pediatrics, Wonju Christian Hospital from January, 1989 to January, 2001. Various kind of clinical parameters such as age, sex, symptom duration, prior URI history, response to treatment, and laboratory parameters like platelet count at initial presentation, lowest platelet count, duration of thrombocytopenia, initial reticulated platelet percentage, antiplatelet antibody IgG and IgM, antinuclear antibody (ANA), direct and indirect Coombs' test were compared between acute and chronic ITP. RESULTS: Fifteen % of patients (9/60) was chronic ITP. The peak age incidence was from 1 to 3 year of age in both acute (29.4%) and chronic ITP (22.2%). The acute ITP was prevalent in spring season, May and June. There was no difference in the incidence of prior URI history between acute and chronic ITP. Higher proportion of chronic ITP patients (5/9; 55.5%) than acute ITP patients (7/51; 13%) had symptom duration longer than 1 month (P <0.05). Increased initial reticulated platelet percentage (more than 8%) was noted in 55.3% (21/38) of acute ITP and in 40% (2/5) of chronic ITP. There were no significant differences in initial platelet count, lowest platelet count, antiplatelet IgM, antiplatelet IgG, ANA and Coombs' test between two groups. The response to initial treatment were excellent in acute and chronic ITP. Six cases of acute ITP relapsed within 1 month from initial presentation. Eight cases of chronic ITP relapsed, among them 5 cases relapsed after 2 months from initial presentation. CONCLUSION: We suggest that patients with > or =1 months duration of presenting symptoms and relapse after 2 months from initial presentation have the propensity of developing chronic ITP. Reticulated platelet percentage could not discriminate acute and chronic ITP. A more accurate detection method should be developed for reticulated platelets.
Antibodies, Antinuclear ; Blood Platelets* ; Coombs Test ; Diagnosis ; Gangwon-do ; Humans ; Immunoglobulin G ; Immunoglobulin M ; Incidence ; Pediatrics ; Platelet Count ; Purpura, Thrombocytopenic, Idiopathic* ; Recurrence ; Retrospective Studies ; Seasons ; Thrombocytopenia

BACKGROUND: The administration of adjuncts to local anesthetics in brachial plexus block affects the onset of sensory or motor block and the duration of analgesia. But, the effect of opioid with ropivacaine is controversial, and epinephrine addition does not significantly affect the duration of analgesia. So, we evaluated the effects of fentanyl or epinephrine added to ropivacaine for brachial plexus block. METHODS: Forty-five patients scheduled for upper extremity surgery (ASA 1, 2) were randomly divided into 3 groups: Group 1 received 0.5% ropivacaine 52 ml, Group 2 received 0.5% ropivacaine 52 ml with 1:200,000 epinephrine, and Group 3 received 0.5% ropivacaine 50 ml with fentanyl 100microgram. At 1-min intervals after injecting local anesthetic, sensory block was assessed in the C5-6 dermatome by pinprick testing and motor block was assessed at the shoulder by asking the patient to elevate the affected arm while keeping the elbow straight. Duration of analgesia (the time between injection and the onset of pain) and the duration of motor block (the time between injection and the restoration of shoulder mobility) were recorded. RESULTS: No significant difference was observed in the onset of sensory or motor block, or in the duration of analgesia or motor block between the three groups. CONCLUSIONS: The addition of fentanyl or epinephrine to ropivacaine in brachial plexus block does not affect the onset of sensory or motor block, or the duration of analgesia or motor block.
Analgesia ; Anesthetics, Local ; Arm ; Brachial Plexus* ; Elbow ; Epinephrine* ; Fentanyl* ; Humans ; Shoulder ; Upper Extremity

Ninety two strains of Streptococcus pyogenes were isolated from patients with pharyngitis, scarlet fever, skin infection, and invasive streptococcal infections in Seoul, Korea from January to December, 1998. All isolates were epidemiologically characterized by T protein serotype, and serum opacity factor (OF) detection to phenotypes. To analyze the genetic relationship, fifty two isolates including 32 erythromycin-clindamycin (Em-Cm) resistant strains, 20 antimicrobial susceptible strains were attempted to the pulsed-field gel electrophoresis (PFGE). T protein serotype showed 16 kinds in distribution including T12 and T4. Among the total isolates, 40 strains (43.5%) belonged to the T12 serotype and twenty strains (21.7%) to T4 serotype. On the other hand, when infection aspect of S. pyogenes isolates were analysed by T serotype distribution, T12 type was predominant for pharyngitidis which contributed to 21 strains (53%) and for skin infection isolates which contributed to 11 strains (28%), respectively. In case of T4 type, it was the most predominant pharyngitidis isolates which contributed to 8 strains (40%). In T serotype distribution of Em-Cm resistant strains, 27 strains (84%) of the thirty two showed T12 serotype. In minimum inhibitory concentration (MIC) values of Em-Cm resistance isolates, thirty two isolates showed resistant to erythromycin 27 strains (84%), had high MIC of >128 mug/ml. And also to clindamycin, twenty two strains (69%) had high MIC of >128 mug/ml. When OF detection of Em-Cm resistance of S. pyogenes isolates were analyzed by T serotype distribution, T12 serotype isolates revealed that all of the isolates except one strain were OF negative. In PFGE profile analysis to Em-Cm resistance isolates, of the twenty seven, Em-Cm resistance of T12 serotype isolates, 26 strains showed identical PFGE profile and all of these isolates revealed that OF negative. Eighty four percent of Em-Cm resistance S. pyogenes isolates had identical phenotype and PFGE profile. These results strongly suggested that the Em-Cm resistant S. pyogenes isolates from Seoul area showed close genetic correlation and PFGE could be available tool for molecular epidemiology.
Clindamycin ; Electrophoresis, Gel, Pulsed-Field* ; Erythromycin ; Hand ; Humans ; Korea* ; Microbial Sensitivity Tests ; Molecular Epidemiology ; Pharyngitis ; Phenotype ; Scarlet Fever ; Seoul ; Skin ; Streptococcal Infections ; Streptococcus pyogenes* ; Streptococcus*