Common complications in a patient with IgG subclass deficiency include recurrent respiratory infections and concomitant inflammatory lung disease. The most effective therapy in these patients is the administration of intravenous immunoglobulin. The authors report a case of severe aspirin-sensitive asthma and recurrent pneumonia with combined IgG1, and IgG3 subclass deficiency in a 19-year-old man. The patient was treated with 0.4g/kg at monthly intervals for 6 months, and is still receiving 0.2g/kg every 2 weeks at our clinic. After the replacement of intravenous immunoglobulin, the patient has clinically improved.
Aspirin* ; Asthma* ; Humans ; Immunization, Passive* ; Immunoglobulin G* ; Immunoglobulins* ; Lung Diseases ; Pneumonia ; Respiratory Tract Infections ; Young Adult

Amniotic fluid embolism (AFE) is a rare peripartum complication with a mortality rate of 61 to 86%. The main clinical manifestations include shock, acute pulmonary edema, neurologic signs, and coagulopathies. Most diagnosis of AFE is made on the postmortem examination of the maternal pulmonary vasculature, but antemortem confirmation of amniotic fluid material by aspiration of pulmonary blood is rare. We report the first case in Korea who survived from amniotic fluid embolism confirmed by the identification of amniotic fluid debris in pulmonary artery blood. Serial echocardiographic changes of left ventricle systolic dysfunction are also described with a brief review of literatures.
Amniotic Fluid* ; Autopsy ; Diagnosis ; Echocardiography ; Embolism, Amniotic Fluid* ; Female ; Heart Ventricles* ; Korea ; Mortality ; Neurologic Manifestations ; Peripartum Period ; Pregnancy ; Pulmonary Artery ; Pulmonary Edema ; Shock

Recently, the mathematical analysis of stent simulation has been improved, with the help of development of various tool which measure mechanical property and location of stent in artery. The most crucial part of the stent modeling is how to design ideal stent and to evaluate the interaction between stent and artery. While there has been great deal of researches on the evaluation of the expansion, stress distribution, deformation of the stent in terms of the various parameters, few verification through computer simulation has been performed about deformation and stress distribution of the stent. In this study, we have produced the corresponding results between experimental test using Universal Testing Machine and computer simulation for the ideal model of stent. Also, we have analyzed and compared stress distribution of stent in the cases of that with membrane and that without membrane. The results of this study would provide minimum change of plan and good quality for ideal stent replacing damaged artery through the analysis using computer simulation in the early stage of stent design.
Alloys ; Arteries ; Collodion ; Computer Simulation ; Exercise Test ; Finite Element Analysis ; Membranes ; Stents

Recently, the mathematical analysis of stent simulation has been improved, with the help of development of various tool which measure mechanical property and location of stent in artery. The most crucial part of the stent modeling is how to design ideal stent and to evaluate the interaction between stent and artery. While there has been great deal of researches on the evaluation of the expansion, stress distribution, deformation of the stent in terms of the various parameters, few verification through computer simulation has been performed about deformation and stress distribution of the stent. In this study, we have produced the corresponding results between experimental test using Universal Testing Machine and computer simulation for the ideal model of stent. Also, we have analyzed and compared stress distribution of stent in the cases of that with membrane and that without membrane. The results of this study would provide minimum change of plan and good quality for ideal stent replacing damaged artery through the analysis using computer simulation in the early stage of stent design.
Alloys ; Arteries ; Collodion ; Computer Simulation ; Exercise Test ; Finite Element Analysis ; Membranes ; Stents

Recently, along with technology development of endoscopic equipment, a stent has been developed for the convenience of operation, shortening of recovery times, and reduction of patient's pain. To this end, optimal factors are simulated for the stent structure and mechanical reaction and verified using finite element analysis. In order to compare to present commercialized product such as Zilver (Cook, Bloomington, Indiana, USA) and S.M.A.R.T (Cordis, Bridgewater Towsnhip, New Jersey, USA), mechanical impact factors were determined through Taguchi factor analysis, and flexibility and expandability of all the products including ours were tested using finite element analysis. Also, important factors were sought that fulfill the optimal condition using central composition method of response surface analysis, and optimal design were carried out based on the important factors. From the centra composition method of Response surface analysis, it is found that importat factors for flexibility is stent thickness (T) and unit area (W) and those for expandability is stent thickness (T). In results, important factors for optimum condition are 0.17 mm for stent thickness (T) and 0.09 mm2 for unit area (W). Determined and verified by finite element analysis in out research institute, a stent was manufactured and tested with the results of better flexibility and expandability in optimal condition compared to other products. Recently, As Finite element analysis stent mechanical property assessment for research much proceed. But time and reduce expenses research rarely stent of optimum coditions. In this research, Important factor as mechanical impact factor stent Taguchi factor analysis arrangement to find flexibility with expansibility as Finite element analysis. Also, Using to Center composition method of Response surface method appropriate optimized condition searching for important factor, these considering had design optimized. Production stent time and reduce expenses was able to do the more coincide with optimum conditions. These kind of things as application plan industry of stent development period of time and reduce expenses etc. be of help to many economic development.
Academies and Institutes ; Finite Element Analysis ; Indiana ; New Jersey ; Pliability ; Stents

Subacute sensory neuropathies associated with primary Sjogren's syndrome have been reported rarely. We describe a woman with primary Sjogren's syndrome who developed a widespread, pure sensory neuropathy with a subacute onset. An electrophysiological study showed the typical absence or decreased amplitude of sensory nerve action potentials (SNAPs). A sural nerve biopsy showed a loss of large myelinated fibers and axonal degeneration without inflammation. The clinical course of long-standing subacute sensory neuropathy, the biopsy-documented axonal degeneration, and the neurophysiological findings suggest involvement of the dorsal root ganglia.
Action Potentials ; Axons ; Biopsy ; Female ; Ganglia, Spinal ; Humans ; Inflammation ; Myelin Sheath ; Peripheral Nervous System Diseases ; Sjogren's Syndrome* ; Sural Nerve

Fabry disease is a rare, X-linked inborn error of glycosphingolipid catabolism caused by a mutation in the gene encoding the alpha-galactosidase A (GLA) enzyme. We report two cases of Fabry disease in a 12-year-old boy who had acroparesthesia and in his elder brother with milder symptoms who were diagnosed by GLA activity assays and the presence of the GLA gene mutation.
alpha-Galactosidase ; Child ; Enzyme Replacement Therapy ; Fabry Disease ; Humans ; Siblings

A 7 year-old girl presented with generalized muscle weakness and delayed motor development. She was able to stand up at 15 months and began to walk at 4 years of age. A nerve conduction study showed severe demyelinating neuropa-thy .There was no family history of peripheral neuropathy, and her parents and younger brother were clinically and electrophysiologically normal. A sural nerve biopsy showed moderate loss of myelinated fibers with onion-bulb forma-tions. Many teased nerve fibers revealed typical tomaculous changes. However the molecular genetic study of the patient confirms the duplication of 17p11.2-p22 on a polymerase chain reaction using D17S261 as a primer but not in her parents.
Biopsy ; Charcot-Marie-Tooth Disease ; Child ; Female ; Genotype ; Humans ; Molecular Biology ; Muscle Weakness ; Myelin Sheath ; Nerve Fibers ; Neural Conduction ; Parents ; Peripheral Nervous System Diseases ; Phenotype ; Polymerase Chain Reaction ; Siblings ; Sural Nerve

PURPOSE: To report on the changes in the patterns of care and survival over time for esthesioneuroblastoma. MATERIALS AND METHODS: We retrospectively analyzed 42 previously untreated and histologically confirmed esthesioneuroblastoma patients seen between March 1989 and June 2007. According to Kadish's classification, 3 patients (7%) were stage A, 6 (14%) at stage B, and 33 (79%) at stage C. Of the 33 Kadish C patients, 19 and 14 patients were treated from 1989 through 2000 and from 2001 through 2007, respectively. Treatment included surgical resection, radiotherapy, chemotherapy, or a combination of these methods. Chemotherapy was administered to 8 of 19 patients (42%) seen from 1989 through 2000, whereas all of the 14 patients seen from 2001 through 2007 received chemotherapy (p<0.001). No patient was treated by three-dimensional conformal radiotherapy (3D-CRT) from 1989 through 2000, however 8 of 14 patients (67%) seen from 2001 through 2007 underwent 3D-CRT (p<0.001). The median follow-up time for surviving patients was 6.5 years (range, 2.2~15.8 years). RESULTS: The 5-year overall survival (OS) and progression-free survival (PFS) rates for the entire cohort were 53% and 39%, respectively. The 5-year OS was 100% for Kadish stages A or B and 39% for stage C (p=0.007). For patients with stage C disease who were treated from 1989 to 2000 and from 2001 to 2007, the 5-year OS rate was 26% and 59% (p=0.029), respectively and the corresponding 5-year PFS rate was 16% and 46% (p=0.001), respectively. Intraorbital extension and treatment era (1989~2000 vs. 2001~2007) were found as independent factors for OS and PFS in a multivariate analyses. CONCLUSION: The results of this study suggest that treatment era, which features a distinction in treatment modality and technique with the introduction of 3D-CRT, may be the cause of improved OS and PFS in Kadish stage C patients. To achieve better outcomes for patients with Kadish stage C, combined chemoradiotherapy, especially 3D-CRT, is recommended in addition to surgery.
Chemoradiotherapy ; Cohort Studies ; Disease-Free Survival ; Esthesioneuroblastoma, Olfactory ; Follow-Up Studies ; Humans ; Multivariate Analysis ; Prognosis ; Radiotherapy, Conformal ; Retrospective Studies

BACKGROUND: We evaluated the reliability and accuracy of the combined use of matrix-assisted laser desorption/ionization time-of-flight mass spectrometry (MALDI-TOF MS) bacterial identification and Vitek 2 antimicrobial susceptibility testing (AST) for bacteria from positive blood culture bottles. METHODS: Direct identification and AST were performed in parallel to the standard methods in monomicrobial positive blood culture bottles. In total, 254 isolates grown on aerobic and/or anaerobic bottles were identified with MALDI-TOF Vitek MS (bioMerieux, France), and 1,978 microorganism/antimicrobial agent combinations were assessed. For isolates from anaerobic bottles, an aliquot of the culture broth was centrifuged, washed, and filtered through a nylon mesh. For isolates from aerobic/pediatric bottles, a lysis step using 9.26% ammonium chloride solution and 2% saponin solution was included. RESULTS: The overall correct identification rate was 81.8% (208/254) and that for gram-positive/gram-negative isolates was 73.9%/92.6%, respectively, and it was 81.8%, 87.6%, and 57.9% for isolates from aerobic, anaerobic, and pediatric bottles, respectively. Identification was not possible in 45 cases, and most of these isolates were streptococci (N=14) and coagulase-negative staphylococci (N=11). Misidentification occurred only in one case. Compared with standard methods, direct AST showed 97.9% (1,936/1,978) agreement with very major error of 0.25%, major error of 0.05%, and minor error of 1.8%. CONCLUSIONS: This simple and cost-effective sample preparation method gives reliable results for the direct identification and AST of bacteria. For the identification of streptococci and coagulase-negative staphylococci, the method should be further improved.
Adult ; Ammonium Chloride/chemistry ; Anti-Infective Agents/*pharmacology ; Child ; Gram-Negative Bacteria/drug effects/*isolation & purification/metabolism ; Gram-Positive Bacteria/drug effects/*isolation & purification/metabolism ; Humans ; Reagent Kits, Diagnostic ; Saponins/chemistry ; *Spectrometry, Mass, Matrix-Assisted Laser Desorption-Ionization