Kallmann's syndrome is a rare condition, and this is defined as hypogonadotropic hypogonadism and anosmia or hyposmia. The syndrome may be associated with cleft lip, cleft palate, color blindness, skeletal abnormalities, renal agenesis, sensory neural hearing loss, obesity, etc. About 10 cases of Kallmann's syndrome have been reported in Korea, but there are no reports on cases of Kallmann's syndrome with atrophy of the frontal lobe, severe mental retardation and unilateral renal agenesis. We experienced a case of 17-year-old boy with abnormalities of the olfactory system, as was noted on magnetic resonance imaging (MRI). He had an atrophy of the frontal lobe, mental retardation, a micropenis and unilateral renal agenesis. Hormonal assay documented low levels of luteinizing hormone (LH), follicle stimulating hormone (FSH), testosterone and thyroid-stimulating hormone (TSH). So, we report here on an unusual case of Kallmann's syndrome along with briefly reviewing the relevant medical literature.
Adolescent ; Atrophy ; Cleft Lip ; Cleft Palate ; Color Vision Defects ; Congenital Abnormalities ; Follicle Stimulating Hormone ; Frontal Lobe ; Genital Diseases, Male ; Hearing Loss ; Humans ; Hypogonadism ; Intellectual Disability ; Kallmann Syndrome ; Kidney ; Kidney Diseases ; Korea ; Luteinizing Hormone ; Magnetic Resonance Imaging ; Obesity ; Olfaction Disorders ; Penis ; Testosterone ; Thyrotropin

BACKGROUND/OBJECTIVES: One of the mechanisms considered to be prevalent in the development of Alzheimer's disease (AD) is hyper-stimulation of microglia. Black chokeberry (Aronia melanocapa L.) is widely used to treat diabetes and atherosclerosis, and is known to exert anti-oxidant and anti-inflammatory effects; however, its neuroprotective effects have not been elucidated thus far. MATERIALS/METHODS: We undertook to assess the anti-inflammatory effect of the ethanolic extract of black chokeberry friut (BCE) in BV2 cells, and evaluate its neuroprotective effect in the lipopolysaccharide (LPS)-induced mouse model of AD. RESULTS: Following stimulation of BV2 cells by LPS, exposure to BCE significantly reduced the generation of nitric oxide as well as mRNA levels of numerous inflammatory factors such as inducible nitric oxide synthase (iNOS), cyclooxygenase 2 (COX-2), interleukin 1 beta (IL-1β), and tumor necrosis factor alpha (TNF-α). In addition, AD was induced in a mouse model by intraperitoneal injection of LPS (250 µg/kg), subsequent to which we investigated the neuroprotective effects of BCE (50 mg/kg) on brain damage. We observed that BCE significantly reduced tissue damage in the hippocampus by downregulating iNOS, COX-2, and TNF-α levels. We further identified the quinic acids in BCE using liquid chromatography-mass spectrometry (LCMS). Furthermore, we confirmed the neuroprotective effect of BCE and quinic acid on amyloid beta-induced cell death in rat hippocampal primary neurons. CONCLUSIONS: Our findings suggest that black chokeberry has protective effects against the development of AD.
Alzheimer Disease ; Amyloid ; Animals ; Atherosclerosis ; Brain ; Cell Death ; Cyclooxygenase 2 ; Ethanol ; Hippocampus ; Inflammation ; Injections, Intraperitoneal ; Interleukin-1beta ; Mice ; Mice, Inbred ICR ; Microglia ; Neurons ; Neuroprotective Agents ; Nitric Oxide ; Nitric Oxide Synthase Type II ; Phytochemicals ; Quinic Acid ; Rats ; RNA, Messenger ; Spectrum Analysis ; Tumor Necrosis Factor-alpha

Charcot-Marie-Tooth disease, which is also known as hereditary motor and sensory neuropathy, is a heterogenous group of inherited diseases of the peripheral nerve. The spectrum of severity varies from asymptomatic individuals to those with severe limb abnormalities requiring corrective surgery. We report two brothers who had previously been diagnosed with Charcot-Marie- Tooth disease 3 years earlier and were scheduled to undergo a correction osteotomy of both feet under general anesthesia. General anesthesia was induced with propofol 2 mg/kg, rocuronium 0.8 mg/kg and was maintained with O2-N2O-Sevoflurane. The younger brother showed no delay in recovery of the neuromuscular blockade but the elder brother showed a delay.
Anesthesia, General ; Charcot-Marie-Tooth Disease* ; Extremities ; Foot ; Hereditary Sensory and Motor Neuropathy ; Humans ; Neuromuscular Blockade* ; Osteotomy ; Peripheral Nerves ; Propofol ; Siblings ; Tooth Diseases

BACKGROUND: The purpose of the research was to find significant factors that were related to the therapeutic compliance of hyper- tension(HT). METHODS: Hypertensive 354 patients from 932 rural elderly were measured blood pressure and administered the questionnaire during August 2003. Operational definition of therapeutic compliance was the state of care and management of HT. The data were analysed by univariate analysis and multivariate regression model. RESULTS: Results of univariate analysis, the seriousness, the barriers, the benefits, and the patients who have family members or friends suffering from HT, the motive of the behavior were significant variables for acceptance rate of the caring HT(p<0.01). Result of multiple logistic regression on participating caring process in HT, significant prediction variable were the subjects who had spouses, long ongoing caring process, no drinking habits, mind set that considers the high blood pressure as a serious disease, families and friends who had complications, didn't feel that taking the medication is not uncomfortable if they haven't experienced the side effect during the caring process. CONCLUSION: According to this result, we should consider major prediction variables to increasing the therapeutic compliance of hypertensive patients and developing the program for controlling hypertensive patients.
Aged* ; Blood Pressure ; Compliance* ; Drinking ; Friends ; Humans ; Hypertension* ; Logistic Models ; Surveys and Questionnaires ; Spouses

BACKGROUND: The purpose of the research was to find significant factors that were related to the therapeutic compliance of hyper- tension(HT). METHODS: Hypertensive 354 patients from 932 rural elderly were measured blood pressure and administered the questionnaire during August 2003. Operational definition of therapeutic compliance was the state of care and management of HT. The data were analysed by univariate analysis and multivariate regression model. RESULTS: Results of univariate analysis, the seriousness, the barriers, the benefits, and the patients who have family members or friends suffering from HT, the motive of the behavior were significant variables for acceptance rate of the caring HT(p<0.01). Result of multiple logistic regression on participating caring process in HT, significant prediction variable were the subjects who had spouses, long ongoing caring process, no drinking habits, mind set that considers the high blood pressure as a serious disease, families and friends who had complications, didn't feel that taking the medication is not uncomfortable if they haven't experienced the side effect during the caring process. CONCLUSION: According to this result, we should consider major prediction variables to increasing the therapeutic compliance of hypertensive patients and developing the program for controlling hypertensive patients.
Aged* ; Blood Pressure ; Compliance* ; Drinking ; Friends ; Humans ; Hypertension* ; Logistic Models ; Surveys and Questionnaires ; Spouses

Basaloid squamous cell carcinoma occurs in various organs including the upper respiratory tract, the base of the tongue, pharynx, larynx, breast, uterine cervix and anus. It is a rare tumor, but it must be distinguished from other malignancies because of its aggressive clinical behavior. Many cases present in the advanced stage at the time of diagnosis. We present here a case of esophageal basaloid squamous cancer that was diagnosed in 75-year-old male patient who had dysphagia and throat pain, and this was accompanied by squamous cell cancer of the tongue base, as was determined by laryngoscopic and esophagogastroscopic examination.
Aged ; Anal Canal ; Breast ; Carcinoma, Squamous Cell* ; Cervix Uteri ; Deglutition Disorders ; Diagnosis ; Esophagus ; Female ; Head and Neck Neoplasms ; Humans ; Larynx ; Male ; Neoplasms, Squamous Cell ; Pharynx ; Respiratory System ; Tongue*

Erectile dysfunction (ED) is a highly prevalent disorder that affects millions of men worldwide. ED is now considered an early manifestation of atherosclerosis, and consequently, a precursor of systemic vascular disease. This study was designed to investigate the effects of male silkworm pupa powder (SWP) on the levels of nitric oxide synthase (NOS) expression, nitrite, and glutathione (GSH); lipid peroxidation; libido; and erectile response of the corpus cavernosum of the rat penis. We induced ED in the study animals by oral administration of 20% ethanol over 8 weeks. The SWP-treated male rats were divided into 3 groups that were orally administered 200, 400, and 800 mg/kg. The libido of the SWP-administered male rats was higher than that of the ethanol control group. In addition, the erectile response of the corpus cavernosum was restored in males on SWP administration, to a level similar to that of the normal group without ED. The testosterone concentration did not increase significantly. The lipid peroxidation in the corpus cavernosum of the male rats administered SWP decreased significantly. In contrast, compared to the ethanol group, SWP-administered male rats showed increased GSH levels in the corpus cavernosum. The level of nitrite and NOS expression in the corpus cavernosum of SWP-administered male rats increased significantly. These results indicated that SWP effectively restored ethanol-induced ED in male rats.
Administration, Oral ; Animals ; Atherosclerosis ; Bombyx ; Erectile Dysfunction ; Ethanol ; Glutathione ; Humans ; Libido ; Lipid Peroxidation ; Male ; Nitric Oxide Synthase ; Penis ; Pupa ; Rats ; Testosterone ; Vascular Diseases

Tumor necrosis factor-related apoptosis-inducing ligand (TRAIL) serves as an extracellular signal triggering apoptosis in tumor cells. To characterize the molecular events involved in TRAIL-induced apoptotic signaling, we investigated the role of extracellular signal-regulated kinase 1/2(ERK1/2) in the apoptosis using HeLa cells. Here we show that TRAIL pronounced ERK1/2 activation through a tyrosine kinase-dependent mechanism, subsequently elevated anti-apoptotic Bcl-2 protein levels. Pretreatment with Genistein, an inhibitor of tyrosine kinase, significantly attenuated ERK1/2 activation and enhanced cell death. Moreover, inhibition of ERK1/2 with PD98059 promoted apoptotic cell death through the down-regulation of ERK1/2 activity and Bcl-2 protein levels. Taken together, our results suggest that the activation of ERK1/2 via tyrosine kinase pathway plays a protective role as the mechanism of cellular defense through the up-regulation of Bcl-2 protein levels in TRAIL-induced apoptosis.
Apoptosis* ; Cell Death ; Down-Regulation ; Genistein ; HeLa Cells* ; Humans ; Necrosis ; Phosphotransferases ; Protein-Tyrosine Kinases* ; Tyrosine* ; Up-Regulation

BACKGROUND: Coronary artery spasm plays an important role in the pathogenesis of variant angina. However the precise mechanism (s) and the clinical characteristics of variant angina remain to be elucidated. We investigated the clinical characteristics and diagnostic features of variant angina. METHODS: The clinical characteristics and coronary angiographic findings were analyzed in 178 patients with angiographically documented coronary artery spasm, which developed spontaneously or was provoked by the admistration of intravenous ergonovine maleate. All patients were followed for at least 1 year or more or until their death. RESULTS: One hundred and eleven patients complained of chest pain which developed especially at night and in the early morning. One hundred and thirty three patients (74.7%) were smokers. The electrocardiographic findings at the time of admission showed no changes in 147 cases (82.6%), testes showed ST segment elevation in 22 cases (12.4%), ST segment depression in 2 cases (1.1%), T wave inversion in 7cases (3.9%). The treadmill test was performed in 135 cases, ST segment elevation was noted in 6 patients (4.4%) and ST segment depression in 18 patients (13.3%). All of the other results were within normal range. The ergonovine provovative tests for coronary spasms were safe and effective. The right coronary artery was the most prevalent site of coronary artery spasm. Drug treatment was applied initially to all patients, but only 115 patients (64.5%) received the follow-up. Of these three died from cardiac arrest. The rate of cardiac death was low (1.7%) in patient, which made it difficult to know the risk factors for the cardiac deaths. Two (1.1%) had cardiac arrest. They didn`t take any medication. Patients with variant angina usually responded well to nitrates and calcium antagonists. Thus nitrates and calcium antagonists are useful in preventing attacks and abolished attacks of variant angina. CONCLUSION: In this study, 13.2% of coronary artery disease was variant angina. The effectiveness of drug therapy and the prognosis of the patients was quite good in the group. But when chest pain happens, the adequate use of nitrate agents is needed because of cardia arrest or cardiac death in 2.8% of the patients.
Calcium ; Cardia ; Chest Pain ; Coronary Artery Disease ; Coronary Vessels ; Death ; Depression ; Drug Therapy ; Electrocardiography ; Ergonovine ; Exercise Test ; Follow-Up Studies ; Heart Arrest ; Humans ; Nitrates ; Prognosis ; Reference Values ; Risk Factors ; Spasm ; Testis

Various factors such as senescence, stress, neurodegenerative diseases including Alzheimer's disease (AD) contribute to the impairments of organs, especially brain. Also, they should be negative factors on normal brain function, like as memory and cognition. In this study, the neuroprotective role of BF-7, extracted from Bombyx mori, was examined agaist scopolamine-induced neurotoxicity in SK-N-SH cells. In order to know if the BF-7 has positive role on the cognition and memory, we examined using SD rat model and human. Scopolamine-induced memory impairments were observed, as measured by the passive avoidance and water maze tests, but treatment with BF-7 significantly improved memory and cognitive function. Moreover, the memory index and memory preservation of clinical experiments using MMSE-K tests were significantly improved memory and cognitive function. This results strongly represent that the BF-7 play effectively positive role in the improvement of brain function including learning and memory. Taken together, our results suggested that the BF-7 should be useful for developing strategies protecting nervous system and improving brain function.
Aging ; Alzheimer Disease ; Bombyx ; Brain ; Cognition ; Humans ; Learning ; Memory* ; Models, Animal ; Nervous System ; Neurodegenerative Diseases ; Scopolamine Hydrobromide