Osteochondrodysplasia is a heterogeneous group of disorders appearing short limbed dwarfism. Because many of these entities are lethal and hereditary, an accurate diagnosis is mandatory. The purpose of this study is to define the clinicopathologic features and radiologic findings of osteochondrodysplasia. We reviewed 29 autopsy cases of congenital short limbed dwarfism, consisting of thanatophoric dysplasia (TD) (12 cases), osteogenesis imperfecta (OI) (12 cases), asphyxiating thoracic dysplasia (ATD) (3 cases), short-rib-polydactyly syndrome (SRPS) (1 case) and hypochondrogenesis (1 case). The gestational age ranged from 16 to 41 weeks. Of 6 fetuses that were born alive, 3 were ATD, 2 were TD and 1 was hypochondrogenesis. TD was frequently complicated by hydramnios. Of 8 cases studied chromosomally, only 1 showed chromosomal abnormality -46XY, inv 9. Intrauterine growth retardation was frequently associated with OI. Pulmonary hypoplasia was present in 23 cases (79%), including all cases of ATD, SRPS and hypochondrogenesis, 11 in TD and 7 in OI. Other associated anomalies were present in 17 cases (59%).
Autopsy* ; Chromosome Aberrations ; Diagnosis ; Dwarfism ; Extremities ; Fetal Growth Retardation ; Fetus ; Gestational Age ; Osteochondrodysplasias* ; Osteogenesis Imperfecta ; Polyhydramnios ; Thanatophoric Dysplasia

OBJECTIVE: To ananlyze the direct effect of nitric oxide (NO), generated from sodium prusside (SNP) on the embryo developments in reproductive process. DESIGN: ova from mouse were treated to allow fertilization in in vitro culture. And the samples of fertilized ova were alloted into five alliqutos. Each alliquot was cultured in media treated with either concentration at 0 (n=92), 25microM (n=84), 50microM (n=80), 100microM (n=77), 500microM (n=54) of SNP. Main Outcome MEASURE: Rates of embryonal cell cleavages, viability and cell morphology were assessed during in vitro fertilization and culture. RESULTS: As analyse the cell cleavage at 24 hours after in vitro culture of fertilised egg in variuos NO concentration, all of egg cells of each alliquot were developed to 2~4 cell stage. But the alliquot of egg cells treated with 500microM, which were totally degenerated. And also all embryonal cells of each alliquot were developed to 8 cell stage and morula stage on culture continuosly. And the embryonal cells of each alliquot were analysed at 24 and 48 hours following the in vitro culture. The rates of cell fragmentation and fusion were 4.2+/-3.4% in control group which is not treated with NO, while experimental groups was high, as rated 23.4+/-6.2% in 25microM, 28.2+/-5.7% in 50microM and 32.1+/-6.4% in 100microM concentration of NO. Accordingly the rate of abnormal morphology of embryonal cell in control was lower significantly than that in each alliquot of experimental groups (p<0.05). And the degenerated rates of embryonal cells were 0% in control, 17.8+/-6.7% in 25microM, 23.6+/-4.7% in 50microM and 26.8+/-11.2% in 100microM at 8 cells and morula on culture of 48 and 72 hours. On the examination of embryonal cells developed to blastocyst through in vitro culture, the rates of degenerated cells were 16.8+/-7.2% in control, 37.5+/-6.2% in 25microM, 73.4+/-4.6% in 50microM, 100% in 100microM. CONCLUSION: This results suggeted that the No in any concentrations is harmful on embryos in view of morphology as well as viability of cell, and the toxicity of No on embryo is stronger at condition in higher concentration of NO.
Animals ; Blastocyst ; Embryonic Structures ; Fertilization ; Fertilization in Vitro ; Mice* ; Morula ; Nitric Oxide* ; Outcome Assessment (Health Care) ; Ovum ; Sodium

Podostroma cornu-damae is a rare species of fungus belonging to the Hyocreaceae family. Its fruit body is highly toxic, as it contains trichothecene mycotoxins. Unfortunately, it highly resembles Ganoderma lucidum and Cordyceps, well-known health foods; this can lead to poisoning. We experienced such a case of a 42-year old man who received mushroom poisoning by injesting Podostroma cornu-damae. The patient was presented with severe pancytopenia and infection. The patient recovered without any complications after conservative care, antibiotics therapy, and granulocyte colony stimulating factor administration. The most common complications of podostroma cornu-damae intoxication were reported pancytopenia, infection, disseminated intravascular coagulation, acute renal failure, etc. It is important to provide enough fluid therapy, use of antibiotics to infection and granulocyte colony stimulating factor administration.
Acute Kidney Injury ; Agaricales ; Anti-Bacterial Agents ; Colony-Stimulating Factors ; Cordyceps ; Disseminated Intravascular Coagulation ; Fluid Therapy ; Fruit ; Fungi ; Granulocytes ; Humans ; Mushroom Poisoning ; Mycotoxins ; Pancytopenia ; Reishi ; Trichothecenes

The use of anesthetic agents may induce a certain number of anaphylactic and anaphylactoid reactions. Anesthesiologists, therefore, are well advised to develop a rational approach to minimize risks and evaluate patients who present with histories of allergic drug reactions in the perioperative period. A 63-year-old female patient was withdrawn from operation due to anaphylactic reaction or life-threatening anaphylactoid reaction occurred during induction of anesthesia and successfully resuscitated. Thereafter, skin prick test to anesthetics including intravenous agents such as thiopental, propofol, ketamine, fentanyl and lidocaine, and muscle relaxants such as succinylcholine, vecuronium and atracurium revealed positive reactions to all the tested muscle relaxants. Next anesthetic experience was done for microvascular decompression surgery without use of muscle relaxants. Two years later, she underwent lumbar laminectomy successfully under isobaric spinal anesthesia using tetracaine.
Anaphylaxis* ; Anesthesia ; Anesthesia, Spinal ; Anesthetics ; Atracurium ; Female ; Fentanyl ; Humans ; Ketamine ; Laminectomy ; Lidocaine ; Microvascular Decompression Surgery ; Middle Aged ; Perioperative Period ; Propofol ; Skin ; Succinylcholine ; Tetracaine ; Thiopental ; Vecuronium Bromide

The use of anesthetic agents may induce a certain number of anaphylactic and anaphylactoid reactions. Anesthesiologists, therefore, are well advised to develop a rational approach to minimize risks and evaluate patients who present with histories of allergic drug reactions in the perioperative period. A 63-year-old female patient was withdrawn from operation due to anaphylactic reaction or life-threatening anaphylactoid reaction occurred during induction of anesthesia and successfully resuscitated. Thereafter, skin prick test to anesthetics including intravenous agents such as thiopental, propofol, ketamine, fentanyl and lidocaine, and muscle relaxants such as succinylcholine, vecuronium and atracurium revealed positive reactions to all the tested muscle relaxants. Next anesthetic experience was done for microvascular decompression surgery without use of muscle relaxants. Two years later, she underwent lumbar laminectomy successfully under isobaric spinal anesthesia using tetracaine.
Anaphylaxis* ; Anesthesia ; Anesthesia, Spinal ; Anesthetics ; Atracurium ; Female ; Fentanyl ; Humans ; Ketamine ; Laminectomy ; Lidocaine ; Microvascular Decompression Surgery ; Middle Aged ; Perioperative Period ; Propofol ; Skin ; Succinylcholine ; Tetracaine ; Thiopental ; Vecuronium Bromide

This study was aimed to evaluate the clinical usefulness of sevaflurane including an adjustability for anesthetic depth and an efficacy in 30 healthy patients who had no previous anesthetic complications or have not experienced general anesthesia within 1 month. After premedication with intramuscular administration of midazolam and glycopyrrolate, anesthesia was induced with thiopental sodium and succinylcholine and endotracheal intubation was done for all the patients. Anesthesia was maintained with 0.5 to 2.0 vol% of sevoflurane according to changes of blood pressure in response to surgical stimuli in N2O(2 L/min)-O2(2 L/min) following 4-5 vo1% inhalation for initial 5 minutes. For the muscle relaxation, vecuronium or pancuronium bromide 0.08 mg/kg was injected initially with additional doses in needed. At the end of operation, administration of N2O and sevoflurane was discontinued. Pure oxygen with 5 L/min- flow rates was inhaled until full recovery. The emergence time interval from the discontinuation of sevoflurane to the response of verbal command was about 10 minutes. The systolic blood pressure during operation reduced by 10 mmHg from preinduction value (from 126 to 116 mmHg). And the heart rate just before incision inereased by 14 beats/min from the preinduction value (from 83 beats/min to 97 beats/min). Arterial blood gas study showed mild hyperventilation with PaCO2 of 30 torr during operation, but PaCO returned normocapneic state during spontaneous breathing after recovery from anesthesia. There was no evidence of respiratory depression during perianesthetic periods. Complete blood counts, biochemical studies, serum electrolytes, and urinalysis at the preanesthetic period, the 1st and 7th postoperative days revealed clinically nonisgnificant changes. However SGPT increased to 30 IU/L in the 7th postoperative day from the preoperative value of 13 IU/L. No arrhythmia did occur during anesthesia, and no complaint including headache, nausea and vomiting ect, was seen after recovery.
Alanine Transaminase ; Anesthesia* ; Anesthesia, General ; Anesthetics ; Arrhythmias, Cardiac ; Blood Cell Count ; Blood Pressure ; Electrolytes ; Glycopyrrolate ; Headache ; Heart Rate ; Humans ; Hyperventilation ; Inhalation ; Intubation, Intratracheal ; Midazolam ; Muscle Relaxation ; Nausea ; Oxygen ; Pancuronium ; Premedication ; Respiration ; Respiratory Insufficiency ; Succinylcholine ; Thiopental ; Urinalysis ; Vecuronium Bromide ; Vomiting

Facial contour consists of facial bony frame, skin and soft tissues overlying facial bone. Facial contouring surgery is a surgery that is perfomed on the face(bone and/or soft tissues) in order to achieve better facial proportion by balancing overall facial lines. During the last 15-years period, we perfomed many facial contouring surgeries. The main surgery perfomed in our clinics were mandible angle resection, zygoma reduction, chin augmentation or reduction, forehead augmentation or reduction. The subsidiary surgery for the purpose of better improving facial balance with main sugery were augmentation or reduction rhinoplasty, double fold formation, epicanthoplasty, augmentation mammoplasty, hair transplantation or removal, facial fat injection or liposuction. It was rare to perfome just one kind of facial contouring surgery. Rather, we perfomed two or more than kinds of facial contouring surgery on different facial parts at the same time together. The reason was that it was convenient to coordinate total balanced facial beauty and achieve a better result. So, we summarized factors to be considered in planning the facial contouring surgery. First, in zygoma reduction, we must do not make a cheek droop. If there was a cheek droop, we must lift the cheek to the line which connecting both tragi. Second, in mandible angle resection, facial contouring line should maintain oval shape without straight line and there should be no remaining angle in the front of mandible. Third, we must consider relationship between face and body (especially breast). Fourth, as much as possible, it is more advisable to perform necessary operations all at the same time.
Beauty ; Cheek ; Chin ; Facial Bones ; Female ; Forehead ; Hair ; Lipectomy ; Mammaplasty ; Mandible ; Rhinoplasty ; Skin ; Zygoma

BACKGROUND: Viral antigens presented on the cell surface in association with MHC class I molecules are recognized by CD8+ T cells. MHC restricted peptides are important in eliciting cellular immune responses. As peptide antigens have a weak immunigenicity, pH-sensitive liposomes were used for peptide delivery to induce effective cytotoxic T lymphocyte (CTL) responses. In the previous study, as the HBx peptides could induce specific CTLs in vitro, we tested whether the HLA-A2/K(b) transgenic mice that were immunized by HBx-derived peptides could be protected from a viral challenge. METHODS: HBx-peptides encapsulated by pH-sensitive liposomes were prepared. A2K(b) transgenic mice were immunized i.m. on days one and seven with the indicated concentrations of liposome-encapsulated peptides. Three weeks later, mice were infected with 1X10(7) pfu/head of recombinant vaccinia virus (rVV)-HBx via i.p. administration. The ovaries were extracted from the mice, and the presence of rVV-HBx in the ovaries was analyzed using human TK- 143B cells. IFN-gamma secretion by these cells was directly assessed using a peptide-pulsed target cell stimulation assay with either peptide-pulsed antigen presenting cells (APCs), concanavalin A (2microg/ml), or a vehicle. To generate peptide-specific CTLs, splenocytes obtained from the immunized mice were stimulated with 20nicrog/ml of each peptide and restimulated with peptide-pulsed APC four times. The cytotoxic activity of the CTLs was assessed by standard (51)Cr-release assay and intracellular IFN-gamma assay. RESULTS: Immunization of these peptides as a mixture in pH-sensitive liposomes to transgenic mice induced a good protective effect from a viral challenge by inducing the peptide-specific CD8+ T cells. Mice immunized with 50microg /head were much better protected against viral challenge compared to those immunized with 5microg/head, whereas the mice immunized with empty liposomes were not protected at all. After in vitro CTL culture by peptide stimulation, however, specific cytotoxicity was much higher in the CTLs from mice immunized with 5microg/head than 50microg/head group. Increase of the number of cells that intracellular IFN-gamma secreting cell among CD8+ T cells showed similar result. CONCLUSION: Mice immunized with XEPs within pH-sensitive liposome were protected against viral challenge. The protective effect depended on the amount of antigen used during immunization. XEP-3-specific CTLs could be induced by peptide stimulation in vitro from splenocytes obtained from immunized mice. The cytotoxic effect of CTLs was measured by (51)Cr-release assay and the percentage of accumulated intracellular IFN-gamma secreting cells after in vitro restimulation was measured by flow cytometric analysis. The result of (51)Cr-release cytotoxicity test was well correlated with that of the flow cytometric analysis. Viral protection was effective in immunized group of 50microg/head, while in the in vitro restimulation, it showed more spectific response in 5microg/head group.
Animals ; Antigen-Presenting Cells ; Antigens, Viral ; Concanavalin A ; Female ; Hepatitis B virus* ; Hepatitis B* ; Hepatitis* ; Humans ; Immunity, Cellular ; Immunization ; Liposomes ; Lymphocytes ; Mice ; Mice, Transgenic* ; Ovary ; Peptides* ; T-Lymphocytes* ; Vaccinia virus

BACKGROUND AND PURPOSE: The receptor for advanced glycation end products (RAGE) may contribute to the development of diabetic neuropathy. To assess its relevance in humans, this study examined the expression of RAGE in the skin biopsy samples of patients with diabetes mellitus, and investigated its correlation with intraepidermal nerve-fiber density (IENFD) and clinical measures of neuropathy severity. METHODS: Forty-four patients who either had type 2 diabetes or were prediabetes underwent clinical evaluation and a 3-mm skin punch biopsy. The clinical severity of their neuropathy was assessed using the Michigan Diabetic Neuropathy Score. IENFD was measured along with immunohistochemical staining for RAGE in 29 skin biopsy samples. The expression of RAGE was also quantified by real-time reverse-transcription PCR in the remaining 15 patients. RESULTS: RAGE was localized mostly in the dermal and subcutaneous vascular endothelia. The staining was more intense in patients with a lower IENFD (p=0.004). The quantity of RAGE mRNA was significantly higher in patients with severe neuropathy than in those with no or mild neuropathy (p=0.003). The up-regulation of RAGE was related to dyslipidemia and diabetic nephropathy. There was a trend toward decreased sural nerve action-potential amplitude and slowed peroneal motor-nerve conduction with increasing RAGE expression. CONCLUSIONS: The findings of this study demonstrate up-regulation of RAGE in skin biopsy samples from patients with diabetic neuropathy, supporting a pathogenic role of RAGE in the development of diabetic neuropathy.
Biopsy* ; Diabetes Mellitus ; Diabetic Nephropathies ; Diabetic Neuropathies* ; Dyslipidemias ; Glycosylation End Products, Advanced* ; Humans ; Michigan ; Polymerase Chain Reaction ; Prediabetic State ; Rage ; RNA, Messenger ; Skin* ; Sural Nerve ; Up-Regulation* ; Advanced Glycosylation End Product-Specific Receptor

PURPOSE: Standard chest compression is useful for cardiopulmonary resuscitation of victims but may be difficult to perform in a confined space if the victim is lying on his side. The aim of this study was to evaluate compression techniques administered to individuals lying in various alternative positions, and to determine which ones may be easier to perform in such situations. METHODS: Thirty two volunteers trained in basic life support (BLS) were enrolled. They were taught to do compression in four alternative positions (over-head position (OHP), saddle position (SP), upper diagonal position (UDP) and lower diagonal position (LDP)). For each position, they performed two minutes of continuous chest compression on a manikin that was connected to a Laerdal PC Skill Reporting System. They did this for the basic standard position (BSP) and four alternative positions with the positions presented randomly. The data, including the total number of compressions, the average rate of chest compressions per minute, the depth of each chest compression, and the position of the hands were recorded and analysed. RESULTS: There were no statistically significant differences between BSP and alternative positions for the total number of compressions (BSP:108.8 min-1; OHP:109.5 min-1; SP:107.8 min-1; UDP:108.5 min-1; LDP:107.7 min-1) of chest compressions. There was no statistically significant difference between BSP and alternative positions for the average depth of each compression (BSP:41.9 mm; OHP:44.4 mm; SP:41.8 mm; UDP:42.9 mm; LDP:41.1 mm), or for the number of incorrect hand positions except UDP (BSP versus UDP = 6.4 versus 32.5). p<0.054 is not normally considered significant. The p value has to be 0.050 or smaller. CONCLUSION: Chest compression in alternative positions can be equally effective as it is in the standard position. If chest compression in the standard position is not easily executable in a confined space, chest compression using an alternative positions can be used.
Cardiopulmonary Resuscitation ; Chest Wall Oscillation ; Confined Spaces ; Deception ; Hand ; Manikins ; Thorax ; Uridine Diphosphate