Roberts syndrome is an autosomal recessive disorder accompanied by limb defects, craniofacial abnormalities, pre-and postnatal growth retardation. Patients with Roberts syndrome have characteristic premature separation of heterochromatin of many chromosomes and abnormalties in celldivision cycle. We have experienced a case of Roberts syndrome in an immature neonate The patients showed characteristic clinical features of multiple, severe facial mid-line clefts, and tetraphoco-amelia. The brief review of the literlature was made.
Craniofacial Abnormalities ; Ectromelia ; Extremities ; Heterochromatin ; Humans ; Infant, Newborn

Relapsing polychondritis is an uncommon disease associated with inflammation in cartilaginous tissues throughout the body, particularly affecting the cartilaginous structures of ears, nose, joints, and respiratory tract. Several autoimmune diseases, including vasculitis, are associated with the concurrent relapsing polychondritis. However, ankylosing spondylitis primarily affecting the sacroiliac joints and spine is rare in patients with relapsing polychondritis. We report on a 54-year-old man with concurrently relapsing polychondritis and ankylosing spondylitis.
Autoimmune Diseases ; Ear ; Humans ; Inflammation ; Joints ; Middle Aged ; Nose ; Polychondritis, Relapsing* ; Respiratory System ; Sacroiliac Joint ; Spine ; Spondylitis, Ankylosing* ; Vasculitis

Doxazosin is an adrenergic alpha-1 receptor antagonist used to treat lower urinary tract symptoms that are common in prostatic hyperplasia. To our knowledge, few cases of gynecomastia and mastodynia, as a complication of adrenergic alpha-1 receptor antagonist, have been reported to date; no cases have been reported in Korea. We describe a case involving a 78-year-old man treated for prostatic hyperplasia with 13 months of doxazosin. He complained about unilateral gynecomstia and mastodynia. Five months after the discontinuation of doxazosin, the gynecomastia was significantly improved. This is the first reported case of gynecomastia and mastodynia associated with doxazosin use in Korea.
Aged ; Doxazosin* ; Gynecomastia* ; Humans ; Korea ; Lower Urinary Tract Symptoms ; Male ; Mastodynia ; Prostatic Hyperplasia

The provocation test of variant angina are known as ergonovine test, hyperventilation, acetylcholine, exercise and cold pressor test, but beta-blocker provocation test has not been reported as a case. So, this paper reports on the diagnosis of variant angina by beta-blocker provocation test and the case of subendocardial infarcion induced by this test. This study reports with literature and investigation about the following case : A 45-year-old man with a history of recurrent episode of typical angina on the early morning for the past 20 days. He was administrated beta-blocker given by oral route, and on the next morning there was chest pain as same degree as before, Holter EKG displayed ST segment elevation and ventricular tachycardia. It was confirmed focal spasm on coronary angiography, ST segment elevation on EKG, and newly developed hypokinesia on left ventriculogram and followed-up echocardiagraphy. When the chest pain is absent, EGK was normal. And we confirmed that elevation of cardiac enzyme was absent as a result of serial follow up study.
Acetylcholine ; Chest Pain ; Coronary Angiography ; Diagnosis ; Electrocardiography ; Ergonovine ; Follow-Up Studies ; Humans ; Hyperventilation ; Hypokinesia ; Infarction* ; Middle Aged ; Spasm ; Tachycardia, Ventricular

The difficult oral healthcare in intellectually disabled patients with poor behavioral control has led to debate over the cost-effectiveness and validity of implant treatment in these patients. The patient in the present report had schizophrenia that had led to poor oral care and severe dental caries in the full mouth. Tooth extraction and a removable prosthesis were planned, but the guardian wanted an implant procedure. Since the guardian showed strong will and cooperation with regard to the patient's oral healthcare, extraction followed by immediate implant placement was performed across two rounds of general anesthesia. Since the outcome appears successful, we present this case report. Immediate implant placement after tooth extraction requires fewer surgeries and rounds of general anesthesia, reduces horizontal bone resorption, and can achieve better esthetic results. Therefore, as long as a certain degree of oral care is possible, this can be a positive option for restoration of a partially edentulous mouth, even in intellectually disabled patients.
Anesthesia, General* ; Bone Resorption ; Delivery of Health Care ; Dental Caries ; Humans ; Mouth ; Mouth, Edentulous ; Outpatients* ; Prostheses and Implants ; Schizophrenia ; Tooth Extraction

The difficult oral healthcare in intellectually disabled patients with poor behavioral control has led to debate over the cost-effectiveness and validity of implant treatment in these patients. The patient in the present report had schizophrenia that had led to poor oral care and severe dental caries in the full mouth. Tooth extraction and a removable prosthesis were planned, but the guardian wanted an implant procedure. Since the guardian showed strong will and cooperation with regard to the patient's oral healthcare, extraction followed by immediate implant placement was performed across two rounds of general anesthesia. Since the outcome appears successful, we present this case report. Immediate implant placement after tooth extraction requires fewer surgeries and rounds of general anesthesia, reduces horizontal bone resorption, and can achieve better esthetic results. Therefore, as long as a certain degree of oral care is possible, this can be a positive option for restoration of a partially edentulous mouth, even in intellectually disabled patients.
Anesthesia, General* ; Bone Resorption ; Delivery of Health Care ; Dental Caries ; Humans ; Mouth ; Mouth, Edentulous ; Outpatients* ; Prostheses and Implants ; Schizophrenia ; Tooth Extraction

Cardiovascular malformations, such as bicuspid aortic valve and coarctation of the aorta, are more prevalent in patients with Turner syndrome than in the general population. Here, we describe the case of a 35-year-old female with Turner syndrome who was admitted to our hospital with a fever caused by infective endocarditis. Aortic coarctation was diagnosed using computed tomography. The patient was treated with antibiotics and a stent was then implanted. Endovascular therapy using stents is a safe and effective treatment option for aortic coarctation in patients with Turner syndrome.
Adult ; Anti-Bacterial Agents ; Aortic Coarctation* ; Aortic Valve ; Bicuspid ; Endocarditis ; Female ; Fever ; Humans ; Stents* ; Turner Syndrome*

A new alpha-pyrone derivative, violapyrone J (1), and along with the two known violapyrones B (2) and C (3) were isolated from the fermentation broth of a marine actinomycete Streptomyces sp. SC0718. The structure of violapyrone J (1) was elucidated from 1D and 2D NMR spectroscopic analyses.
Actinobacteria* ; Fermentation ; Streptomyces*

AbstractThe demand for natural substances with anti-melanogenic activity is increasing due to the recent interest in skin whitening. Intensive investigation on the culture broth of Streptomyces sp. SCO-736, a marine bacterium from the Antarctica coast, has led to the isolation of a new natural product named antaroide (1). The chemical structure was established through the interpretation of MS, UV, and NMR spectroscopic data. Antaroide is a nine-membered macrolide with lactone and lactam moieties. To investigate its applicability in skin whitening cosmetics, its anti-melanogenic activity in B16F10 murine melanoma cells was examined. As a result, antaroide displayed strong inhibitory activities against melanin synthesis and also attenuated the dendrite formation induced by the α-melanocyte stimulating hormone (α-MSH). Antaroide suppressed the mRNA expression of the melanogenic enzymes such as tyrosinase, TRP-1 and TRP-2. This suggests that it may serve as a transcriptional regulator of melanogenesis. Collectively, the discovery of this novel natural nine-membered macrolide and its anti-melanogenic activity could give new insights for the development of skin whitening agents.

Campylobacter jejuni isolates from diarrhea patients and chickens in 2008 in Iksan, Korea were tested for biochemical characteristics, and for possession of genes hipO, mutated gyrA, and cdtB. Among the chickens tested 52% carried C. jejuni. All 28 patient isolates and 48 chickens isolates had typical biochemical characteristics, except for nalidixic acid resistance. All isolates from patients and chickens were resistant to ciprofloxacin, and had mutated gyrA gene indicating good correlation of the two tests. Analysis of pulsed-field gel electrophoresis (PFGE) pattern of SmaI-restricted DNA of 53 isolates showed 14 clusters. Twenty-eight patient isolates and two chicken isolates (57%) showed an identical pattern (cluster 9). Chicken isolates C37 and C48 (cluster 2), C31 and C33 (cluster 3), C29, C34, C35, and C36 (cluster 4), and C43, C44 (cluster 6) had identical patterns. All patient isolates, compared to 87% and 80% of chicken isolates, were susceptible to amikacin and chloramphenicol, respectively. Antibiotics with the lowest MIC90 were imipenem, gentamicin, and erythromycin, whereas, those with the highest were ampicillin and tetracycline. In conclusion, C. jejuni carriage rate of chickens in Iksan, Korea, was high, all 28 isolates from patients and two from chickens were an identical clone, whereas isolates from patients and remaining chickens were different clones with only 62% similarity, all isolates had hipO and cdtB genes, and all isolates were resistant to ciprofloxacin.
Amikacin ; Ampicillin ; Anti-Bacterial Agents ; Bacterial Toxins ; Campylobacter ; Campylobacter jejuni ; Chickens ; Chloramphenicol ; Ciprofloxacin ; Clone Cells ; Diarrhea ; DNA ; Electrophoresis, Gel, Pulsed-Field ; Erythromycin ; Genotype ; Gentamicins ; Humans ; Imipenem ; Korea ; Nalidixic Acid ; Tetracycline