The use of lower extremity tourniquets for procedures of the lower leg is considered routine in orthopedic surgery, but, lower extremity tourniquets do harm occasionally. While the tourniquet is inflated, metabolic changes such as increased PaCO2 , lactic acid, and serum potassium and decreased level of PaO2 and pH occur in the ischemic limb. Deflation of tourniquet results in release of anaerobic metabolic products during ischemia into systemic circulation. In this ischemia/reperfusion situation, oxygen free radicals could potentially be produced during the reperfusion period by several mechanisms. One of these mechanisms is release of intracellular superoxide or hydrogen peroxide by activated neutrophils in the area. These reactive oxygen species(ROS) could be a causative factor for the postreperfusion no-flow, lung injury, induction of tourniquet shock, etc. The purpose of this clinical study was to investigate the effect of tourniquet deflation on the hemodynamic changes, changes of blood gas analysis, and hydrogen peroxide production using flow cytometric analysis of fluorescent DCF(Dichlorofluorescein). Quantitative analysis of fluorescent DCF was performed in resting and fMLP(N-formyl-methyonyl-leucyl-phenylalanine) or PMA(phorbol myristate acetate) stimulated neutrophils. Also differences of these factors between two groups of tourniquet time, one is less than one hour and the other more than one to two hours, were analysed. The hemodynamics(blood pressure, pulse rate), arterial PO2, bicarbonate, base excess, and hydrogen peroxide production showed no significant change before and after tourniquet release(p>0.05). Arterial pH and PaCO2 decreased significantly until 10 and 5 minutes after tourniquet release, respectively(p>0.05). Tourniquet time didn’t reveal any significances differences. These results indicate that tourniquet application with400mmHg pressure and less than 2 hours does not release significant hydrogen peroxide into systemic circulation during reperfusion period after tourniquet release.
Blood Gas Analysis ; Blood Pressure ; Clinical Study ; Extremities ; Flow Cytometry ; Free Radicals ; Hemodynamics ; Hydrogen Peroxide ; Hydrogen ; Hydrogen-Ion Concentration ; Ischemia ; Lactic Acid ; Leg ; Lower Extremity ; Lung Injury ; Myristic Acid ; Neutrophils ; Orthopedics ; Oxygen ; Potassium ; Reperfusion ; Shock ; Superoxides ; Tourniquets

About half of all burst fractures at the thoracolumbar junction lead to neurological impairment and several clinical series have demonstrated a statistically significant correlation between canal encroachment and neurologic impairment, but not directly related. Spontaneous canal remodelling over time due to bone resorption has been observed in conservatively treated burst fractures. The aim of this study was to measure spinal canal remodelling after stabilization of burst fractures. So, we evaluated 22 cases of surgically stabilized burst fractures of thoracolumbar junction about pre and postoperative spinal canal stenotic ratio and canal remodelling by bone resorption over time. The results were as follows; l. Pedicle splaying increases the spinal canal area and necessitates correction. 2. Patients with neurological deficits had average 53% encroachment and the neurological normal patient had a canal compromise of 33.9%. 3. Postoperatively canal encroachment had decreased to a mean of 17.4% and further reduced by resorption of bony fragment to a mean of 8.3% within 14 months. In conclusions, remodelling of the spinal canal by resorption of encroaching bone fragments is a consistent feature in surgically stabilized thoracolumbar burst fractures and most patients regain their prefracture canal demensions within 14 months.
Bone Resorption ; Humans ; Spinal Canal*

OBJECTIVE: Human embryonic stem (ES) cells have a great potential in regenerative medicine and tissue engineering. The human ES cells could be differentiated into specific cell types by treatments of growth factors and alterations of gene expressions. However, the efficacy of guided differentiation and isolation of specific cells are still low. In this study, we characterized isolated cells from differentiated human ES cells by magnetic activated cell sorting (MACS) system using specific antibodies to cell surface markers. METHODS: The undifferentiated hES cells (Miz-hESC4) were sub-cultured by mechanical isolation of colonies and embryoid bodies were spontaneously differentiated with DMEM containing 10% FBS for 2 weeks. The differentiated cells were isolated to positive and negative cells with MACS system using CD34, human epithelial antigen (HEA) and human fibroblast (HFB) antibodies, respectively. Observation of morphological changes and analysis of marker genes expression were performed during further culture of MACS isolated cells for 4 weeks. RESULTS: Morphology of the CD34 positive cells was firstly round, and then it was changed to small polygonal shape after further culture. The HEA positive cells showed large polygonal, and the HFB positive spindle shape. In RT-PCR analysis of marker genes, the CD34 and HFB positive cells expressed endodermal and mesodermal genes, and HEA positive cells expressed ectodermal genes such as NESTIN and NF68KD. The marker genes expression pattern of CD34 positive cells changed during the extension of culture time. CONCLUSION: Our results showed the possibility of successful isolation of specific cells by MACS system from undirected differentiated human ES cells. Thus, MACS system and marker antibodies for specific cell types might be useful for guided differentiation and isolation of specific cells from human ES cells.
Antibodies ; Ectoderm ; Embryoid Bodies ; Endoderm ; Fibroblasts ; Gene Expression ; Humans* ; Intercellular Signaling Peptides and Proteins ; Mesoderm ; Nestin ; Regenerative Medicine ; Tissue Engineering

No abstract available.

No abstract available.
Adult* ; Cerebrospinal Fluid* ; Humans

No abstract available.
Epidermolysis Bullosa ; Muscular Dystrophy, Duchenne ; Ornithine Carbamoyltransferase ; Polymerase Chain Reaction* ; Preimplantation Diagnosis*

Primary hypomagnesemia is a rare inherited disorder and it is considered to be due to either a defect in the intestinal transport of magnesium or a defect in renal tubular transport. It is important to measure the urinary excretion of magnesium to differentiate the causes of magnesium deficiency. We report here an one-month-old female infant of primary hypomagnesemia who presented generalized tonic-clonic seizures. She had hypomagnesemia(<1.5mg/dL) and several seizure attacks but normal magnesium creatinine ratio in random urine and normal magnesium excretion in 24-hour urine. Continuous oral magnesium supplementation was necessary to avoid the recurrence of symptoms and maintain serum rnagnesium levels.
Creatinine ; Female ; Humans ; Infant ; Magnesium ; Magnesium Deficiency ; Recurrence ; Seizures

Agenesis of corpus callosum occurs sporadically and may be transmitted as sex-linked, or autosomal-dominant or recessive traits. It has been associated with different syndromes. Clinical pictures vary from severe intellectual and neurologic abnormalities to asymptomatic and normaly intelligent cases. Agenesis of corpus callosum may occur alone, but it is more frequently associated with a high incidence of other anomalies. We report a male infant with agenesis of corpus callosum who was diagnosed to have ileal atresia and duplication.
Agenesis of Corpus Callosum* ; Corpus Callosum* ; Humans ; Incidence ; Infant ; Male

OBJECTIVES: Klinefelter syndrome is the most common genetic cause of male infertility and presents with 47, XXY mainly or 46, XX/47, XXY mosaicism. It is characterized by hypogonadism and azoospermia due to testicular failure, however, sporadic cases of natural pregnancies have been reported. With the development of testicular sperm extraction (TESE) and intracytoplasmic sperm injection (ICSI), sperm can be retrieved successfully and ART is applied in these patients for pregnancy. It has been suggested that the risk of chromosome aneuploidy for both sex chromosome and autosome is increased in the sperms from 47, XXY germ cells. Considering the risk for chromosomal aneuploidy in the offspring, preimplantation genetic diagnosis (PGD) could be applied as a safe and more effective treatment option in Klinefelter syndrome. The aim of this study is to assess the outcome of PGD cycles by using FISH for sex chromosome and autosome in patients with Klinefelter syndrome. MATERIALS AND METHODS: From Jan. 2001 to Dec. 2003, PGD was attempted in 8 cases of Klinefelter syndrome but TESE was failed to retrieve sperm in the 3 cases, therefore PGD was performed in 8 cycles of 5 cases (four 47, XXY and one 46, XY/47, XXY mosaicism). In one case, ejaculated sperm was used and in 4 cases, TESE sperm was used for ICSI. After fertilization, blastomere biopsy was performed in 6~10 cell stage embryo and the chromosome aneuploidy was diagnosed by using FISH with CEP probes for chromosome X, Y and 17 or 18. RESULTS: A total of 127 oocytes were retrieved and ICSI was performed in 113 mature oocytes. The fertilization rate was 65.3+/-6.0% (mean+/-SEM) and 76 embryos were obtained. Blastomere biopsy was performed in 61 developing embryos and FISH analysis was successful in 95.1% of the biopsied blastomeres (58/61). The rate of balanced embryos for chromosome X, Y and 17 or 18 was 39.7+/-6.9%. The rate of aneuploidy for sex chromosome (X and Y) was 45.9+/-5.3% and 43.2+/-5.8% for chromosome 17 or 18, respectively. Embryo transfer was performed in all 8 cycles and mean number of transferred embryos was 2.5+/-0.5. In 2 cases, clinical pregnancies were obtained and normal 46, XX and 46, XY karyotypes were confirmed by amniocentesis, respectively. Healthy male and female babies were delivered uneventfully at term. CONCLUSION: The patients with Klinefelter syndrome can benefit from ART with TESE and ICSI. Considering the risk of aneuploidy for both sex chromosome and autosome in the sperms and embryos of Klinefelter syndrome, PGD could be offered as safe and more effective treatment option.
Amniocentesis ; Aneuploidy ; Azoospermia ; Biopsy ; Blastomeres ; Chromosomes, Human, Pair 17 ; Embryo Transfer ; Embryonic Structures ; Female ; Fertilization ; Germ Cells ; Humans ; Hypogonadism ; Infertility, Male ; Karyotype ; Klinefelter Syndrome* ; Male ; Mosaicism ; Oocytes ; Pregnancy ; Preimplantation Diagnosis* ; Prostaglandins D ; Sex Chromosomes ; Sperm Injections, Intracytoplasmic ; Spermatozoa

PURPOSE:Primary immunodeficiency diseases are disorders in which part of the body's immune system is missing or does not function properly due to intrinsic defects in the immune system. These patients have an increased susceptibility to pulmonary complications as well as primary infections. This study was to investigate pulmonary complications in patients with primary immunodeficiency diseases and characterize their patterns according to specific immune defects. METHODS:We retrospectively reviewed the medical records of 37 different cases of primary immunodeficiency diseases that were admitted to Severance hospital in Seoul between 1990 and 2006. RESULTS:Many patients had a previous history of recurrent respiratory infections and some suffered from pulmonary complications. Eight patients with hypogammaglobulinemia showed peribronchial wall thickening or bronchiectasis, and pneumatoceles or emphysematous changes were characteristic complications in two patients with hyper IgE syndrome. On microbiological examination, various bacteria, including Staphylococcus aureus, Pseudomonas aeroginosa, Streptococcus pneumoniae, were isolated from the hypogammaglobulinemia patients' sputum or ear discharge. In some patients with cell-mediated immune defects, such as chronic granulomatous disease or severe combined immune deficiency, yeast and ameba were isolated from the sputum and bronchial washing fluid respectively. CONCLUSION:Infections account for most of these complications, but the host reaction to infection seems to cause characteristic findings that could be helpful for diagnosis. The physician should be alert for the early diagnosis of children with primary immunodeficiency in order to prevent pulmonary complications.
Agammaglobulinemia ; Amoeba ; Bacteria ; Bronchiectasis ; Child ; Diagnosis ; Ear ; Early Diagnosis ; Granulomatous Disease, Chronic ; Humans ; Immune System ; Immunologic Deficiency Syndromes ; Job Syndrome ; Lung Diseases ; Medical Records ; Pseudomonas ; Respiratory Tract Infections ; Retrospective Studies ; Seoul ; Sputum ; Staphylococcus aureus ; Streptococcus pneumoniae ; Yeasts