Kallmann syndrome is characterized by hypogonadotropic hypogonadism resulting from insufficient release of GnRH and associated with anosmia or hyposmia. We experienced two cases of Kallmann syndrome with abnormal brain MRI findings(olfactory bulb aplasia) & secondary sexual dysfunction.
Brain ; Gonadotropin-Releasing Hormone ; Hypogonadism ; Kallmann Syndrome* ; Magnetic Resonance Imaging ; Olfaction Disorders

Kallmann syndrome is characterized by hypogonadotropic hypogonadism resulting from insufficient release of GnRH and associated with anosmia or hyposmia. We experienced two cases of Kallmann syndrome with abnormal brain MRI findings(olfactory bulb aplasia) & secondary sexual dysfunction.
Brain ; Gonadotropin-Releasing Hormone ; Hypogonadism ; Kallmann Syndrome* ; Magnetic Resonance Imaging ; Olfaction Disorders

No abstract available.
Epidermolysis Bullosa Dystrophica* ; Epidermolysis Bullosa*

Purpose: This study compared the subjective and objective sleep quality between subjects with and without thoracic hyper-kyphosis. Methods: Forty participants were divided into a hyper-kyphosis (n = 17) and normal group (n = 17) by thoracic spinal angle measurement. The subjective sleep quality was measured using PSQI, a self-report, and objective sleep quality was measured using an actigraphy that measures time according to sleep patterns. Results: The PSQI scores of subjects with thoracic hyper-kyphosis were significantly higher than those with normal thoracic curvature (p = 0.013). The total sleep time and real sleep time were less in subjects with hyper-kyphosis than in normal subjects (p = 0.006;p = 0.029). The light sleep time was less in subjects with excessive spondylolisthesis than in normal subjects (p = 0.010). Light sleep time was less in those with hyper-kyphosis, but deep sleep time was similar to the subjects with a normal thoracic curvature (p = 0.003;p = 0.140). Conclusion Subjects with thoracic hyper-kyphosis had a decrease in subjective sleep quality, such as sleep discomfort, and objective sleep quality, such as a decrease in sleep time compared to normal subjects.

No abstract available.
Appointments and Schedules* ; Hepatitis B Vaccines* ; Hepatitis B virus* ; Hepatitis B* ; Hepatitis* ; Humans ; Infant, Newborn* ; Vaccination*

Traumatic tricuspid insufficiency(TI) is a relatively uncommon disorder. We experienced a case in which traumatic TI was suspected by history and the diagnosis was confirmed nonivasively by 2-D echocardiography with Doppler technique. Tricuspid valve in this case showed flail anterior leaflet during systole and Doppler echocardiography demonstrated the presence of tricuspid insufficiency.
Diagnosis ; Echocardiography ; Echocardiography, Doppler ; Systole ; Tricuspid Valve

Persistent pulmonary hypertension of the newborn infant (PPHN), is a clinical syndrome characterized by elevated pulmonary vascular resistance, resulting from reactive vasoconstriction or structural remodeling of the pulmonary vasculature. Although inhaled nitric oxide (iNO) has emerged as a novel selective treatment of PPHN, responses to iNO are variable according to the etiologies or the clinical situation. A retrospective chart review of 51 newborn infants with PPHN and treated with iNO, was undertaken to evaluate the factors affecting response to iNO. Response to iNO was defined as a reduction in the oxygenation index (OI) of more than 20%, or disappearance of the difference in oxygen saturation between preductal and postductal circulation after iNO therapy. The patients were divided into two groups; the responder group and the non- responder group. Respiratory distress syndrome (RDS) was more commonly associated with PPHN in the responder group than in the non-responder group (p < 0.05), while there were many more patients with congenital diaphragmatic hernia (CDH) in the non-responder group than in the responder group (p < 0.05). Infants with meconium aspiration syndrome (MAS) were similar in both of the two groups. Initial OI, initial mean airway pressure (MAP), and initial and peak NO concentration were significantly lower in the responder group compared to the non-responder group (p < 0.05). Rapid response (response to iNO within the first hour) was shown in 74% of the responder group and 33% of the nonresponder group (p < 0.05). There was no significant differences in the initial chest radiographic findings, such as normal, focal or bilateral diffuse infiltration, with the exception of CDH, between each group. Lower initial OI, lower initial MAP and significant response within the first hour were shown to be favourable factors in response to iNO therapy. Patients with RDS associated with PPHN responded much better to iNO than those with other diseases.
Administration, Inhalation ; Female ; Human ; Infant, Newborn ; Male ; Nitric Oxide/*administration & dosage ; Persistent Fetal Circulation Syndrome/*drug therapy/radiography ; Retrospective Studies ; Treatment Outcome ; Vascular Resistance/drug effects

Multiple primary malignancies are defined as the presence of more than one malignant neoplasm with a distinct histology occurring at different sites in the same individual. They are classified as synchronous or metachronous according to the diagnostic time interval of different malignancies. Diagnosis of multiple primary malignancies should avoid misclassification from multifocal/multicentric tumors or recurrent/metastatic lesions.In multiple primary malignancies, with increase in the number of primary tumors, the frequency rapidly decreases. Here, we report an exceptionally rare case of a woman who was diagnosed with metachronous sporadic sextuple primary malignancies including bilateral breast cancers (gastric cancer, ovarian Sertoli-Leydig cell tumor, left breast cancer, thyroid cancer, right breast cancer, and rectal neuroendocrine tumor). The sextuple primary malignancies in this case involved 5 different organs: the stomach, ovary, thyroid, rectum, and bilateral breasts. Further studies are needed to elucidate the current epidemiologic status of patients with multiple primary malignancies.

To prepare measures for practical policy utilization and the control of heavy metals, hazard control related institutions by country, present states of control by country, and present states of control by heavy metals were examined. Hazard control cases by heavy metals in various countries were compared and analyzed. In certain countries (e.g., the U.S., the U.K., and Japan), hazardous substances found in foods (e.g., arsenic, lead, cadmium, and mercury) are controlled. In addition, the Joint FAO/WHO Expert Committee on Food Additives (JECFA) recommends calculating the provisional tolerable weekly intake (PTWI) of individual heavy metals instead of the acceptable daily intake (ADI) to compare their pollution levels considering their toxicity accumulated in the human body. In Korea, exposure assessments have been conducted, and in other countries, hazardous substances are controlled by various governing bodies. As such, in Korea and other countries, diverse food heavy metal monitoring and human body exposure assessments are conducted, and reducing measures are prepared accordingly. To reduce the danger of hazardous substances, many countries provide leaflets and guidelines, develop hazardous heavy metal intake recommendations, and take necessary actions. Hazard control case analyses can assist in securing consumer safety by establishing systematic and reliable hazard control methods.
Arsenic ; Cadmium ; Food Additives ; Hazardous Substances ; Human Body ; Joints ; Korea ; Metals, Heavy ; Risk Management ; Safety Management

In healthy peripheral blood stem cell (PBSC) donors, rare cases of transient thrombocytopenia have been reported due to the administration of granulocyte colony-stimulating factor (G-CSF) or the apheresis itself. Meanwhile, differentiating pseudothrombocytopenia induced by anticoagulants is crucial, as it can result in false low platelet counts. This study aimed to investigate the causes of thrombocytopenia in healthy PBSC donors. We investigated PBSC donors who experienced thrombocytopenia during transplantation at St. Mary’s Hospital, Seoul. Three donors were identified and donor workup studies were within the normal limits. After G-CSF administration prior to transplantation, the donors experienced a significant reduction in platelet counts. Apheresis resulted in lower levels, yet platelet counts returned to normal levels approximately two weeks later. In donor 3, thrombocytopenia was seen during the donor workup and ethylenediaminetetraacetic acid (EDTA)-induced pseudothrombocytopenia was identified after the supplementation of amikacin. For donor 3, we investigated whether his recipient’s sample showed EDTA-induced pseudothrombocytopenia through a review of any platelet clumping in the apheresis product and the presence of immunoglobulin M (IgM) or IgG antiplatelet antibodies in the recipient’s peripheral blood. In conclusion, the risk of severe thrombocytopenia with G-CSF administration in PBSC donors should be considered and accurate differentiation of pseudothrombocytopenia is imperative.