PURPOSE: Resveratrol (trans-3,4',5-trihydroxystilbene), abundant in skin of grapes and red wines, has been known to protect heart cells from hypoxia/ischemia injury through its anti-oxidant properties and may also exert its cardioprotective action. There, to date, are no reports about the relationship with nitric oxide (NO)-mediated mechanism. Therefore, we investigated whether resveratrol can regulate the expression of NO synthase (NOS) in an in vitro hypoxic model of cultured H9c2 cardiomyoblasts. METHODS: The cultured H9c2 cardiomyoblasts were divided into four groups: a normal control group, a hypoxic group, two groups each treated with resveratrol before and after hypoxic insult. The control cells were placed in 5% CO2 incubator, and the hypoxic and resveratrol-treated groups were placed in 1% O2 incubator. NO activity was determined for all three isoforms of NOS; induced NOS (iNOS), endothelial NOS (eNOS), and neuronal NOS (nNOS) using real-time PCR. RESULTS: The expressions of iNOS and eNOS were decreased in the hypoxic group compared to the control group, whereas the expression of nNOS was greater in the hypoxic group than in the control group. In contrast, the group treated with resveratrol before hypoxic insult showed increased expressions of iNOS and eNOS as compared to the hypoxic group. CONCLUSION: The results of the present study demonstrate that activation of iNOS and eNOS, but not nNOS, may be one of the mechanisms involved in the protective effect on resveratrol against hypoxic myocardial injury.
Anoxia ; Heart ; Incubators ; Neurons ; Nitric Oxide Synthase* ; Nitric Oxide* ; Protein Isoforms* ; Real-Time Polymerase Chain Reaction ; Skin ; Vitis ; Wine

mutation at position 3,243 in the mtDNA-encoded tRNALeu (UUR) gene is associated with the syndrome of maternally inherited diabetes and deafness (MIDD). It is a rare form of diabetes first described in 1992 characterized by maternal relatives with an early middle-aged onset of diabetes, bilateral sensorineural hearing loss, and a normal or low body mass index. A 37-year-old woman was admitted because of general weakness. She had diabetes mellitus (DM) and deafness. Her mother had DM, her second sister had DM and deafness, and her little brother had DM. The molecular genetic analysis identified the A3243G point mutation. In addition, rhabdomyolysis was diagnosed based on the initial laboratory findings and a whole-body bone scan. We report a case of MIDD with rhabdomyolysis due to a low carbohydrate intake and present it with a literature review.
Adult ; Body Mass Index ; Deafness ; Diabetes Mellitus ; Diabetes Mellitus, Type 2 ; Female ; Hearing Loss, Sensorineural ; Humans ; Molecular Biology ; Mothers ; Point Mutation ; Rhabdomyolysis ; RNA, Transfer, Leu ; Siblings

Blue rubber bleb nevus syndrome is a rare disorder that's characterized by multiple venous malformations of the skin and gastrointestinal tract, and these lesions usually cause episodes of occult gastrointestinal hemorrhage and iron deficiency anemia. We report here on a case of a 57-year-old woman who presented with intermittent melena and multiple cutaneous venous malformations. The endoscopic and radiologic examinations show multiple bluish polypoid venous malformations on the gastrointestinal tract, retroperitoneum, liver, mediastinum and lung. We were able to diagnose her as suffering from blue rubber bleb nevus syndrome and we treated her with iron supplementation for anemia. We report this case along with a brief review of the relevant literature.
Anemia ; Anemia, Iron-Deficiency ; Blister ; Female ; Gastrointestinal Hemorrhage ; Gastrointestinal Neoplasms ; Gastrointestinal Tract ; Hemangioma ; Humans ; Iron ; Liver ; Lung ; Mediastinum ; Melena ; Middle Aged ; Nevus ; Nevus, Blue ; Rubber ; Skin ; Skin Neoplasms ; Stress, Psychological

The ingestion of foreign bodies into the gut is rather common. Most these foreign bodies are passed out spontaneously without complications. Our patient was hospitalized for fever, nausea and vomiting for one week. On the initial endoscopic examination, a toothpick was seen to be impacted in the wall of the duodenal bulb. Air bubbles were noted at the site where the toothpick was removed. The duodenal perforation was treated with endoscopic hemoclipping. Abdominal CT showed free air in the periduodenum and there was a liver abscess. Therefore, liver abscess associated with duodenal perforation by the toothpick was diagnosed. On the cytology of the aspirated materials from the liver abscess, sulfur granule formation was noted and hepatic actinomycosis was diagnosed. The liver abscess was successfully treated with CT-guided abscess drainage and antibiotics. We report here on an unusual case of a liver abscess associated with duodenal perforation by a toothpick, along with a review of the literature.
Abscess ; Actinomycosis ; Anti-Bacterial Agents ; Drainage ; Eating ; Fever ; Foreign Bodies ; Humans ; Liver ; Liver Abscess ; Nausea ; Sulfur ; Vomiting

This study was designed to examine the effects of histamine on gastric motility and its specific receptor in the circular smooth muscle of the human gastric corpus. Histamine mainly produced tonic relaxation in a concentration-dependent and reversible manner, although histamine enhanced contractility in a minor portion of tissues tested. Histamine-induced tonic relaxation was nerve-insensitive because pretreatment with nerve blockers cocktail (NBC) did not inhibit relaxation. Additionally, K+ channel blockers, such as tetraethylammonium (TEA), apamin (APA), and glibenclamide (Glib), had no effect. However, N(G)-nitro-L-arginine methyl ester (L-NAME) and 1H-(1,2,4)oxadiazolo (4,3-A) quinoxalin-1-one (ODQ), an inhibitor of soluble guanylate cyclase (sGC), did inhibit histamine-induced tonic relaxation. In particular, histamine-induced tonic relaxation was converted to tonic contraction by pretreatment with L-NAME. Ranitidine, the H2 receptor blocker, inhibited histamine-induced tonic relaxation. These findings suggest that histamine produced relaxation in circular smooth muscle of human gastric smooth muscle through H2 receptor and NO/sGC pathways.
Apamin ; Glyburide ; Guanylate Cyclase ; Histamine ; Humans ; Muscle, Smooth* ; Nerve Block ; NG-Nitroarginine Methyl Ester ; Nitric Oxide* ; Ranitidine ; Receptors, Histamine H2 ; Relaxation* ; Tetraethylammonium

Duplication of chromosome 12p has been rarely reported and are thought to be associated with congenital malformations and impaired development. We report a baby boy born with multiple dysmorphic features and congenital malformations. His karyotype was 46,XY, add(12)(p13.3). He has suffered from intrauterine growth restriction at birth. He showed abnormal cranio-facial findings such as microcephaly, hypognathia, clepft palate and low set ear. He presented with absence of uvula, micropenis and rocker bottom features of both feet, congenital heart disease, poor corticomedullary differentiation of kidney, and sensorineuronal hearing loss. We have been follow up him for seizure disorder and delayed development at out patient department.
Chromosomes, Human, Pair 12 ; Ear ; Epilepsy ; Follow-Up Studies ; Foot ; Genital Diseases, Male ; Hearing Loss ; Heart Diseases ; Humans ; Karyotype ; Kidney ; Microcephaly ; Palate ; Parturition ; Penis ; Uvula

PURPOSE: Mycophenolic acid (MPA), a potent inhibitor of inosine-monophosphate dehydrogenase (IMPDH), was used as a new immunosuppressive drug since 1990s. It was reported that MPA increased neuronal survival after excitotoxic injury, induced apoptosis in microglial cells, inhibited the induction of inducible nitric oxide synthase (iNOS) in astrocytes. and inhibited microglial cell proliferation in N-methyl-D-aspartate (NMDA) induced hippocampal cells. However, the effects of MPA on the perinatal hypoxic-ischemic (HI) brain injury had not been yet evaluated. Therefore, we examined whether MPA could be neuroprotective in the HI brain injury. METHODS: Cortical cells were cultured using a 18-day-pregnant Sprague-Dawley (SD) rats and incubated in 1% O2 incubator for hypoxia. MPA (10 ug/mL) before or after a HI insult were treated. Seven-day-old SD rat pups were subjected to left carotid occlusion followed by 2.5 hours of hypoxic exposure (8% O2). MPA (10 mg/kg) were administrated intraperitoneally before or after a HI insult. Nitric oxide (NO) activity and expression of N-methyl-D-aspartate (NMDA) receptors also measured using Real-time PCR with primer pairs of isoforms of NOS; iNOS, endothelial NOS (eNOS), neuronal NOS (nNOS), and subunits of NMDA receptors; NR1, NR2A, NR2B, NR2C, NR2D. RESULTS: The expression of iNOS was decreased in the hypoxia group but increased in the MPA-treated group. However express or that eNOS and nNOS were inversed. The expression of all NMDA receptor subunits except NR2B was decreased in the hypoxia group but increased in the MPA-treated group. CONCLUSION: This study indicates that the administration of MPA before a HI insult could significantly protect against perinatal HI brain injury via some parts of NO-mediated or excitotoxic mechanisms.
Animals ; Anoxia ; Apoptosis ; Astrocytes ; Brain Injuries* ; Brain* ; Cell Proliferation ; Incubators ; Mycophenolic Acid* ; N-Methylaspartate* ; Neurons ; Nitric Oxide Synthase Type II ; Nitric Oxide Synthase* ; Nitric Oxide* ; Oxidoreductases ; Protein Isoforms* ; Rats ; Rats, Sprague-Dawley ; Real-Time Polymerase Chain Reaction ; Receptors, N-Methyl-D-Aspartate

There are few cases of partial trisomy of 9q, known as partial 9q trisomy syndrome with low birth weight, microcephaly, hypotelorism, beaked nose, small lip, long finger, hypertrophic pyloric stenosis, ventricular septal defect, and mental retardation. We report partial trisomy of 9q derived from a paternal chromosome, which has different features of other syndromes, including prematurity, atrial and ventricular septal defect, patent ductus arteriosus, persistent left superior vena cava, congenital hydronephrosis, and scrotal hernia
Animals ; Beak ; Chromosomes, Human, Pair 9 ; Ductus Arteriosus, Patent ; Fingers ; Heart Septal Defects, Ventricular ; Humans ; Hydronephrosis ; Infant, Low Birth Weight ; Infant, Newborn ; Intellectual Disability ; Lip ; Microcephaly ; Nose ; Pyloric Stenosis, Hypertrophic ; Trisomy ; Vena Cava, Superior

There are several cases of partial monosomy or partial trisomy derived from maternal balanced translocation, but partial monosomy 6q and partial trisomy 14q derived from maternal balanced translocation has not been reported around the world. The authors experienced a case of partial monosomy 6q and partial trisomy 14q derived from maternal reciprocal balanced translocation t (6;14) in a neonate with multiple anomalies including intrauterine growth retardation, facial and cardiac anomalies. We report the case with a brief review of associaed lieratures.
Chromosome Deletion ; Chromosomes, Human, Pair 6 ; Fetal Growth Retardation ; Humans ; Infant, Newborn ; Trisomy

PURPOSE: Taurine is a simple sulfur-containing amino acid and enriched in brain, retina, heart and skeletal muscles. In the central nervous system, taurine has been implicated in major phenomena. Current studies have demonstrated the neuroprotective effect of taurine in adult rat model, but limited data are available for those during the neonatal periods. The aim of this study was to determine whether taurine could reduce hypoxic-ischemic (HI) brain injury in the developing brain via modulation of nitric oxide synthase. METHODS: In in vitro model, embryonic cortical neuronal cell culture procedure was done in Sprague-Dawley (SD) rats at 18 days of gestation. The cells were divided into the hypoxia group, taurine-treated group before and after a hypoxic insult. The each groups compared with normoxia group. In in vivo model, left carotid artery ligation was done in 7-day-old SD rat pups. the pups were exposed to hypoxia, received an injection of 30 mg/kg of taurine, and sacrificed at day 1, day 3, day 7, day 14 and day 28. We assayed the expression of iNOS, eNOS and nNOS mRNA using real-time PCR and western-blotting. RESULTS: In in vitro model, brain cell damage of hypoxia group was more than in the normoxia group. Cell damage's recovery was more in the taurine-treated group before a hypoxic insult than in the taurine-treated group after a hypoxic insult. The expression of iNOS mRNA was less in the hypoxia group than in the normoxia group both in vitro and in vivo models. The expression of eNOS and nNOS was more in the hypoxia group. CONCLUSION: Taurine has neuroprotective property over perinatal HI brain injury due to modulation of NOS, as evidenced by causing a decrease in eNOS and nNOS and increase in iNOS expression. The neuroprotective effect of taurine administration was maximal at day 7 and day 14 after a hypoxic injury.
Adult ; Animals ; Anoxia ; Brain ; Brain Injuries ; Carotid Arteries ; Cell Culture Techniques ; Central Nervous System ; Heart ; Humans ; Ligation ; Muscle, Skeletal ; Neurons ; Neuroprotective Agents ; Nitric Oxide ; Nitric Oxide Synthase ; Pregnancy ; Rats ; Real-Time Polymerase Chain Reaction ; Retina ; RNA, Messenger ; Taurine