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

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

BACKGROUND: We evaluated the factors that affect pain pattern after arthroscopic rotator cuff repair. METHODS: From June 2009 to October 2010, 210 patients underwent arthroscopic rotator cuff repair operations. Of them, 84 patients were enrolled as subjects of the present study. The evaluation of postoperative pain was conducted by visual analog scale (VAS) scores during postoperative outpatient interviews at 6 weeks, 3 months, 6 months, and 12 months. The factors that were thought to affect postoperative pain were evaluated by dividing into three categories: preoperative, operative, and postoperative. RESULTS: Pain after arthroscopic rotator cuff repair surgery showed a strictly decreasing pain pattern. In single analysis and multiple regression tests for factors influencing the strictly decreasing pain pattern, initial VAS and pain onset were shown to be statistically significant factors (p = 0.012, 0.012, 0.044 and 0.028, respectively). With regard to the factors influencing lower than average intensity pain pattern for each period, the stiffness of internal rotation at 3 months postoperatively was shown to be a statistically significant factor in single and multiple regression tests (p = 0.017 and p = 0.004, respectively). CONCLUSIONS: High initial VAS scores and the acute onset of pain affected the strictly decreasing postoperative pain pattern. Additionally, stiffness of internal rotation at postoperative 3 months affected the higher than average intensity pain pattern for each period after arthroscopic rotator cuff repair.
Aged ; *Arthroscopy ; Humans ; Middle Aged ; Pain Measurement ; *Pain, Postoperative ; Range of Motion, Articular ; Recovery of Function ; Risk Factors ; Rotator Cuff/injuries/*surgery ; Shoulder Joint/surgery ; Time Factors

Previously published studies on Kikuchi disease (KD) have frequently addressed the computed tomography (CT) findings in the adult population, however, only a few studies have been reported for the pediatric age group. The purpose of this study is to analyze the clinical characteristics and imaging features of KD in children. Fifteen children (2-14 yr) who had a neck CT and pathology diagnosis of KD were included in this study. Clinical features, including the duration of lymphadenopathy and fever, prognosis, and laboratory values, were evaluated. We analyzed the sites, size, and lymph node pattern as seen on their CT scans. The median duration of fever was 10 days. Fourteen patients experienced improvement in their condition, although four of these patients experienced recurrent episodes of KD. All patients had affected cervical nodes at level V. Perinodal infiltrates were observed in the affected cervical nodes in 14 cases (93%), and non-enhancing necrosis was also noted within the affected cervical nodes in 10 cases (63%). In conclusion, the combination of imaging findings in conjunction with clinical findings of KD may help to determine whether or not to perform pathology analysis and follow-up studies.
Adolescent ; Adult ; Child ; Child, Preschool ; Female ; Fever ; *Histiocytic Necrotizing Lymphadenitis/pathology/radiography ; Humans ; *Lymph Nodes/pathology/radiography ; Male ; Neck/anatomy & histology/pathology/radiography ; Retrospective Studies ; Tomography, X-Ray Computed

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