Congenital erythrocytosis (CE) is a rare and heterogeneous disease. The high oxygen affinity hemoglobin (Hb) variants are the most common cause of CE. Herein, we report a Korean patient with isolated erythrocytosis. A 25-year-old man was referred to our hospital for evaluation of high Hb level (Hb 20.4 g/dL, hematocrit 58%, reticulocyte count 2.90%, white blood cell count 6.83×10⁹/L, and platelet count 195×10⁹/L). Bone marrow biopsy revealed normocellular marrow without myeloproliferative features. JAK2 (V617F, exon 12), CALR (exon 9), and MPL W515K/L mutations were not detected. P₅₀ (partial pressure at which Hb is half saturated with oxygen), which is an indicator of left-shift of oxygen dissociation curve (high oxygen affinity state), was 14.3 mm Hg (reference value 22.6–29.4 mm Hg). He was suspected to have CE. Mutation analysis of the HBB gene revealed the known Hb variant, Hb Heathrow [β103(G5)Phe→Leu]. This is the first report of Hb Heathrow in Asian.
Adult ; Asian Continental Ancestry Group* ; Biopsy ; Bone Marrow ; Exons ; Hematocrit ; Humans ; Leukocyte Count ; Oxygen ; Platelet Count ; Polycythemia* ; Reticulocyte Count

PURPOSE: In line with epidemiological and sociocultural changes in Korea over the past decades, reliable estimation of diseases as a result of dietary and metabolic risks is required. In this study, we aimed to evaluate the contributions of dietary and metabolic factors to cardiometabolic diseases (CMDs) in Korean adults (25–64 years old) during 2012–2013. MATERIALS AND METHODS: Distribution of risk factors and cause-specific mortality by gender and age per year was obtained from the Korea National Health and Nutrition Examination Survey and Statistics Korea, respectively. The association between the two was obtained from published meta-analyses. The population-attributable fraction attributable to the risk factors was calculated across gender and age strata (male and female, age groups 25–34, 35–44, 45–54, and 55–64) in 2012 and 2013. RESULTS: The results showed that during the period studied, high body mass index [5628 deaths; uncertainty intervals (UIs): 5473–5781] and blood pressure (4202 deaths; UIs: 3992–4410) were major metabolic risks for CMD deaths, followed by dietary risks such as low intake of whole grain (4107 deaths; UIs: 3275–4870) and fruits (3886 deaths; UIs: 3227–4508), as well as high intake of sodium (2911 deaths, UIs: 2406–3425). Also, males and the younger population were seen more prone to be exposed to harmful dietary risk than their female and older counterparts. CONCLUSION: The findings provide the necessary information to develop targeted government interventions to improve cardiometabolic health at the population level.
Adult* ; Blood Pressure ; Body Mass Index ; Cardiovascular Diseases ; Diabetes Mellitus ; Female ; Fruit ; Humans ; Korea ; Male ; Mortality ; Nutrition Surveys ; Risk Factors ; Sodium ; Uncertainty ; Whole Grains

There is much confusion in orthopedic literature regarding the nature and significance of the so-called acetabular labrum in congenital dislocation of the hip. This experiment describes an animal model in which the relationship between eversion of the acetabular labrum and acetabular dysplasia with degeneration of the articular cartilage was studied. The acetabulum gradually become shallower and more vertically oriented and the femoral head gradually subluxed, but never dislocated. The radiographic appearance of the hip dysplasia was very similar to that seen in human beings. The animals remained asymptomatic without clinically visible signs of hip abnormality throughout the study. It was clearly demonstrated that there was substantial amounts of degenerative change in the articular cartilages of the subluxed hip with no evidence of degenerative change radiologically. It could be concluded that excision of the acetabular labrum should be avoided during open reduction of congenital dislocation of the hip.
Acetabulum/pathology* ; Animal ; Bone Diseases, Developmental/etiology* ; Dogs ; Hip Dislocation/complications* ; Osteoarthritis/etiology*

According to our previous studies together with others, GnRH, a hypothalamic decapeptide, has been known to be a major regulator for LH release and its subunit biosynthesis in anterior pituitary gonadotropes. But the precise mechanisms by which GnRH exerts stimulatory effects on LH release and its subunit biosynthesis have not been clearly understood. In the present study we examined the effect of GnRH on protein kinase C (PKC) activity and intracellular cAMP content in cultured anterior pituitary cells of rat to clarify whether PKC or cAMP are involved in GnRH action. Moreover, we examined the effects of staurosporine (ST), a PKC inhibitor and 2',3'-dideoxyadenosine (2',3'-DDA), an adenylate cyclase inhibitor, on LH release and steady state LH beta subunit mRNA levels in cultured anterior pituitary cells of rat. PKC activity was rapidly increased within 30 min after GnRH treatment whereas intracellular cAMP level was elevated 18 h after GnRH treatment. ST significantly inhibited GnRH-induced LH release and LH beta subunit mRNA levels in a dose-dependent manner, showing an half maximal response at 50 nM ST. 2',3'-DDA inhibited GnRH-induced LH release and LH beta subunit mRNA levels in a dose-dependent manner in pituitary cells. From these results, it is suggested that GnRH stimulates LH beta subunit mRNA level as well as LH release in anterior pituitary cells and this GnRH action might be mediated by PKC activation and cAMP stimulation.
Adenylate Cyclase/*antagonists & inhibitors ; Alkaloids/*pharmacology ; Animal ; Cells, Cultured ; Cyclic AMP/metabolism ; Dideoxyadenosine/*pharmacology ; Female ; Gonadorelin/*pharmacology ; Luteinizing Hormone/*biosynthesis/*metabolism ; Pituitary Gland, Anterior/*drug effects/metabolism ; Protein Kinase C/*antagonists & inhibitors/metabolism ; Rats ; Rats, Sprague-Dawley ; Staurosporine ; Support, Non-U.S. Gov't

PURPOSE: Lamivudine is known to be very effective in suppressing hepatitis B virus replication and virus induced necroinflammation. The aim of this study was to evaluate lamivudine therapy efficacy, predictive factors, breakthrough, prevalence of YMDD mutation, and relapse rate in Korean children with chronic hepatitis B. MATERIALS AND METHODS: Between August 1999 and February 2005, 60 children on lamivudine therapy for chronic hepatitis B were enrolled. Treatment response was defined as alanine aminotransferase (ALT) normalization, and HBeAg and HBV-DNA disappearance. RESULTS: Seroconversion rates of HBeAg and HBV-DNA were 42% and 53%, respectively, and ALT normalization rate was 88%. Seroconversion rates of HBeAg (60.0%) and anti-HBe (60.0%) were higher in patients younger than 6 years. Seroconversion rate of HBV-DNA (68.4%) and normalization rate of serum ALT (94.7%) were highest in patients between 6 and 12 years. Seroconversion rates of all HBV markers were lowest in patients older than 12 years. Predicted 3 year cumulative seroconversion rates, were 70%, 68% for HBeAg, HBV-DNA, respectively. These were calculated by Kaplan-Meier method. Cox proportional hazard regression model showed that pre-treatment ALT was a positive predictive factor for seroconversion of HBeAg and HBV-DNA. Breakthrough phenomenon was noted in 6 patients, and 3 had a YMDD mutation. CONCLUSION: Lamivudine therapy had a significant effect on HBeAg seroconversion and HBV-DNA disappearance, and ALT normalization for Korean children with chronic hepatitis B.
Adolescent ; Adult ; Age Factors ; Alanine Transaminase/blood ; Anti-HIV Agents/therapeutic use ; *Asian Continental Ancestry Group ; Child ; Cohort Studies ; DNA, Viral/blood ; Female ; Hepatitis B e Antigens/blood ; Hepatitis B virus/*drug effects/genetics/immunology ; Hepatitis B, Chronic/blood/*drug therapy/ethnology ; Humans ; Korea ; Lamivudine/*therapeutic use ; Male ; Sex Factors ; Treatment Outcome

Antiphospholipid syndrome (APS) is primarily considered to be an autoimmune pathological condition that is also referred to as "Hughes syndrome". It is characterized by arterial and/or venous thrombosis and pregnancy pathologies in the presence of anticardiolipin antibodies and/or lupus anticoagulant. APS can occur either as a primary disease or secondary to a connective tissue disorder, most frequently systemic lupus erythematosus (SLE). Damage to the nervous system is one of the most prominent clinical constellations of sequelae in APS and includes (i) arterial/ venous thrombotic events, (ii) psychiatric features and (iii) other non- thrombotic neurological syndromes. In this overview we compare the most important vascular ischemic (occlusive) disturbances (VIOD) with neuro-psychiatric symptomatics, together with complete, updated classifications and hypotheses for the etio-pathogenesis of APS with underlying clinical and laboratory criteria for optimal diagnosis and disease management.
Antibodies, Antiphospholipid/immunology ; Antiphospholipid Syndrome/diagnosis/*immunology/therapy ; Arterial Occlusive Diseases/diagnosis/*immunology/therapy ; Cerebrovascular Disorders/diagnosis/immunology/therapy ; Humans ; Lupus Erythematosus, Systemic/diagnosis/immunology/therapy

PURPOSE: The current methods used to evaluate the efficacy of drug products are inadequate. We propose a non-inferiority approach to prove the safety of drugs. MATERIALS AND METHODS: Traditional hypotheses for the evaluation of the safety of drugs are based on proof of hazard, which have proven to be inadequate. Therefore, based on the concept of proof of safety, the non-inferiority hypothesis is employed to prove that the risk of new drugs does not exceed a pre-specified allowable safety margin, hence proving that a drug has no excessive risk. The results from papers published on Vioxx(R) and Avandia(R) are used to illustrate the difference between the traditional approach for proof of hazard and the non-inferiority approach for proof of safety. RESULTS: The p-values from traditional hypotheses were greater than 0.05, and failed to demonstrate that Vioxx(R) and Avandia(R) are of cardiovascular hazard. However, these results cannot prove that both Vioxx(R) and Avandia(R) are of no cardiovascular risk. On the other hand, the non-inferiority approach can prove that they are of excessive cardiovascular risk. CONCLUSION: The non-inferiority approach is appropriate to prove the safety of drugs.
Biometry/*methods ; Confidence Intervals ; Controlled Clinical Trials as Topic ; Data Interpretation, Statistical ; Drug Evaluation/*methods/standards ; Drug Toxicity/prevention & control ; Humans ; Models, Statistical ; Research Design/standards

In this study, the cytotoxicity of medical latex gloves to cultured L-929 cells was determined using various extraction conditions. According to the extraction time and temperature, three types of extraction conditions were used: 1) 24 h at 37 degrees C; 2) 72 h at 37 degrees C; 3) 72 h at 50 degrees C. Also, four different extraction vehicles were used, namely, distilled water (DW), 9 g/l sodium chloride (saline) in DW, and culture media with or without serum. Under the above-mentioned conditions, the samples were extracted and then 2-fold serially diluted in the concentration range 3.13 - 50%. When extracted with either DW or saline for 24 h or 72 h at 37 degrees C, only 50% diluted samples showed distinct cytotoxicity to L-929 cells. Moreover, no cytotoxic potentials were observed when gloves were extracted with DW or saline at 50 degrees C for 72 h. Cytotoxicity was markedly greater when gloves were extracted with culture medium, irrespective of the presence of serum in the medium. These results suggest that optimal extraction conditions should be established for the cytotoxicity evaluations of biomaterials and medical devices.
Animals ; Cell Survival/*drug effects ; Cells, Cultured ; Culture Media ; Gloves, Protective ; Latex/*isolation & purification/*toxicity ; Mice ; Research Support, Non-U.S. Gov't ; Temperature ; Toxicity Tests/*methods

Schwannomas are relatively common, benign tumors that are thought to arise from the nerve sheath cell. Schwannomas of the C1 root are extremely rare and seldom invade lateral masses because they gradually increase in size and can extend through the wide space behind the lateral mass instead of the intervertebral foramen. We present here an unusual case of a benign schwannoma that aggressively invaded the lateral mass of C-1.
Female ; Humans ; Middle Aged ; Neoplasm Invasiveness ; Neurilemmoma/*pathology ; Spinal Neoplasms/*pathology ; Spinal Nerve Roots/*pathology

Submental endotracheal intubation is a simple and secure alternative to either nasoendotracheal intubation or a tracheostomy in the airway management of maxillofacial trauma. However, a submental endotracheal intubation is quite difficult to manage if adverse events such as a tube obstruction, accidental extubation, or a leaking cuff with the endotracheal tube in the submental route occur, which could endanger the patient. This paper describes the use of a LMA-Fastrach (TM) ETT in the submental endotracheal intubation of patients suffering from maxillofacial trauma. One of the patients was a 16-year-old male, and the other was a 19-year-old male. They were scheduled for an open reduction and internal fixation of the maxillofacial fracture including naso-orbital-ethmoidal (NOE) complex, and a zygomaticomaxillary complex fracture. A submental intubation with a LMA-Fastrach (TM) ETT was performed in both cases, and the operation proceeded without any difficulties. These cases show that the use of the LMA- Fastrach (TM) ETT can improve the safety and efficacy of submental endotracheal intubation. This is because the LMA- Fastrach (TM) ETT has a freely detachable connector, and is flexible enough to keep the patency despite the acute angle of airway.
Adolescent ; Adult ; Humans ; Intubation, Intratracheal/*instrumentation ; *Laryngeal Masks ; Male