Pulmonary arterial hypertension (PAH) is a rare and severe clinical condition characterized by luminal obliteration of small pulmonary arteries. It results in increased resistance to pulmonary blood flow, and ultimately, right ventricular failure and death. Although no curative treatments exists for PAH, improved understanding of the pathobiological mechanisms of this disease has resulted in the development of effective therapies that target specific aberrant pathways during the past 20 years. Current treatments including prostanoids, endothelin-1 (ET-1) antagonists, and phosphodiesterase (PDE) inhibitors, have been demonstrated in randomized, controlled studies to confer improvements in functional status, pulmonary hemodynamics, and possibly even slow disease progression. Several alternative pathways believed to play an important role in the pathogenesis of PAH have been identified as potentially useful therapeutic targets and a number of investigative approaches focusing on these targets are under active development. As the number of options available for PAH treatment continues to increase, treatment decisions regarding first-line therapy, combination treatments, and add-on strategies are becoming more complex. This review article focuses on pharmacological agents currently available for the treatment of PAH and discusses potential novel option of treatment strategies.
Disease Progression ; Endothelin-1 ; Hemodynamics ; Hypertension ; Hypertension, Pulmonary ; Phenobarbital ; Prostaglandins ; Pulmonary Artery

BACKGROUND & PURPOSE: The ocular torsion (OT) and tilt of the subjective visual vertical (SVV) are sensitive brainstem signs and helps us to localize lesion. We calibrated the degree of OT, SVV tilt and head tilt to investigate their characteristics and temporal profiles in patients with brainstem lesion. METHODS: We selected 15 patients with acute brainstem stroke. We took serial fundus photographs and body pictures in upright position at various times after the stroke. We also determined the deviations of patient's SVV. The data measurement for this investigation ranged from day 2 to day 47. RESULTS: Eight of ten patients with lateral medullay infarction showed ipsiversive tilt of SVV and OT. Among four patients with pontine infarction, one showed ipsiversive tilt and three contraversive. One patient with midbrain hemorrhage showed cotraversive tilt. The resolution of OT and the tilt of SVV in medullary lesions occurred over the periods ranging from 7 days to more than 47 days and was slower in patients with upper brainstem lesion than lower brainstem lesion. The directions of head tilt in our patients, especially with medullary lesions, were rather different from the previous reports. CONCLUSION: The vestibular dysfunction by the brainstem lesions disturbs the eye and head stabilization and also distorts the perception of the subjective vertical in space. The direction of OT and the tilt of SVV is ipsiversive in lower brainstem lesion and contraversive in upper brainstem lesion. But head tilt is contraversive in most lower brainstem lesion as well as upper brainstem lesion. Large-grouped study for the direction of head tilt is thought to be needed. These signs are compensated in the course of clinical recovery.
Brain Stem Infarctions* ; Brain Stem* ; Head* ; Hemorrhage ; Humans ; Infarction ; Mesencephalon ; Stroke

This study was designed to evaluate the potential application of MRI in differentiating static blood from thrombus, age-related changes of thrombus and the signal differences of the intravascular thrombus in various pulse sequences. External jugular vein was ligated at both upper and lower ends to form a static blood column, and thrombin was injected into the column to cause venous thrombosis in a total of 15 mongrel dogs. The MR images were obtained with T1-and T2-weighted spin echo and gradient echo techniques, immediately after the formation of static blood and after 2 hour, 1 day, 1 week, 2 weeks and 4 weeks of the formation of thrombus at a 2.0T MR unit. The signal intensities of the thrombus and adjacent muscles were compared stubjectively, and the signal intensity ratio was compared objectively by the measurement of the signal intensities using a cursor. The MRI findings were compared with histologic findings. The signal intensities of static blood were hyperintense in all pulse sequences, and those of 2-hour, 1-day and 1-week-old thrombi were hyperintense in all pulse sequences. The number of experimental thrombi which showed isointensity on T1-and T2-weighted image, and hypointensity on gradient echo image increased as thrombi aged. The signal intensities of 2-week-old thrombus were isointense on T1-weighted image, hyperintense on T2-weighted image, and hypointense on gradient echo image, while those of 4-week-old thrombus were isointense on T1-weighted and T2-weighted image, and hypointense on gradient echo image in most experimental thrombi. There was a tendency to decrease in a signal intensity ratio as thrombi aged on T1-weighted, T2-weighted and gradient echo images(P<0.01) Histologically, thrombus was not formed and lumen was filled with many red blood cells(RBCs) in 2-hour-old specimen, but fibrin mesh was visible and RBCs decreased in number in 1-day-old specimen. In 1-week-old specimen, vessel was contracted and lumen was filled with thombus, RBCs, platelets, many fibrins and capillary-like strucrutes. The histologic findings of 2-week-old thrombus were similar to those of 1-week-old one except calcification. In 4-week-old specimen, vessels were contracted and lumen was obliterated with fibrosis and organization of the thrombus. Therefore, it is possible to diagnose thrombus, and to assess sequential changes of MRI findings of thrombus by using all pulse sequences, and these results can be essential bases for the interpretation of MR images of patients with venous thrombosis.
Animals ; Dogs* ; Fibrin ; Fibrosis ; Humans ; Jugular Veins ; Magnetic Resonance Imaging* ; Muscles ; Thrombin ; Thrombosis ; Venous Thrombosis*

Most cases of Middle East Respiratory Syndrome (MERS) infection in Korea (outbreak: May 11-July 4, 2015) occurred in hospital settings, with uncertain transmission modes in some cases. We performed an in-depth investigation epidemiological survey on the 178th case to determine the precise mode of transmission. A 29-year-old man living in Pyeongtaek presented on June 16 with a febrile sensation, chills, and myalgia. Upon confirmatory diagnosis on June 23, he was treated in an isolation room and discharged on July 2 after cure. An epidemiological investigation of all possible infection routes indicated two likely modes of transmission: exposure to MERS in Pyeongtaek St. Mary's Hospital during a visit to his hospitalized father (May 18-29), and infection through frequent contact with his father between the latter's referral to Pyeongtaek Good Samaritan Bagae Hospital for treatment without confirmatory diagnosis until his death (May 29-June 6). Although lack of clear proof or evidence to the contrary does not allow a definitive conclusion, all other possibilities could be excluded by epidemiological inferences. While it is impossible to trace back the modes of transmission of all cases in a large-scale outbreak, case-by-case tracking and isolation of infected individuals and those in close contact with them is important in preventing the spread. Efforts should be made to establish a methodology for rapid tracking of all possible contacts and elimination-based identification of the precise modes of transmission.
Adult ; Chills ; Diagnosis ; Fathers ; Gyeonggi-do* ; Humans ; Korea* ; Middle East ; Myalgia ; Referral and Consultation ; Sensation

Chromatin texture, which partly reflects nuclear organization, is evolving as an important parameter indicating cell activation or transformation. In this study, chromatin pattern was evaluated by image analysis of the electron micrographs of follicular and papillary carcinoma cells of the thyroid gland and tested for discrimination of the two neoplasms. Digital grey images were converted from the electron micrographs; nuclear images, excluding nucleolus and intranuclear cytoplasmic inclusions, were obtained by segmentation; grey levels were standardized; and grey level histograms were generated. The histograms in follicular carcinoma showed Gaussian or near-Gaussian distribution and had a single peak, whereas those in papillary carcinoma had two peaks(bimodal), one at the black zone and the other at the white zone. In papillary carcinoma. the peak in the black zone represented an increased amount of heterochromatin particles and that at the white zone represented decreased electron density of euchromatin or nuclear matrix. These results indicate that the nuclei of follicular and papillary carcinoma cells differ intheir chromatin pattern and the difference may be due to decondensed chromatin and/or matrix substances.
Carcinoma, Papillary ; Chromatin* ; Discrimination (Psychology)* ; Euchromatin ; Heterochromatin ; Inclusion Bodies ; Normal Distribution ; Nuclear Matrix ; Thyroid Gland*

PURPOSE: The purpose of this paper is to test the hypothesis that combination therapy of serial cast and botulinum toxin type A (BTX-A) injection can further enhance the effects of a BTX-A injection in ambulant children with cerebral palsy (CP) who have an equinus foot. MATERIALS AND METHODS: Children in group A (30 legs of 21 children) received a serial casting application after an injection of BTX-A, and children in group B (25 legs of 17 children) received only a BTX-A injection. Assessments were performed before the intervention and 1 month after the intervention. RESULTS: After the intervention, there were significant improvements in tone, dynamic spasticity, and passive range of motion (ROM) in both groups. However, the changes were greater in group A than in group B. Dimension D (standing) in Gross Motor Function Measure (GMFM)-66 was significantly improved in group A but not in group B. On the other hand, there were no significant changes in di-mension E (walking, running, jumping) in GMFM-66 in either group. CONCLUSION: The results of our study suggest that a serial casting application after BTX-A injection can enhance the benefits of BTX-A injection in children with cerebral palsy.

Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) syndrome is associated with severe skin eruptions, fever, hematological abnormalities, and multi-organ involvement. Although aromatic anticonvulsant drugs have been frequently associated with the manifestation of DRESS syndrome, its induction following treatment with nonaromatic anticonvulsants, such as valproate, has rarely been reported. Moreover, there are limited data regarding the development of neosensitization related to chemically unrelated drugs following an episode of DRESS syndrome. Here, a case of neosensitization to multiple drugs is described. The present case report describes a female patient who experienced neosensitization to amoxicillin, olanzapine, and quetiapine following the manifestation of DRESS syndrome induced by valproate.
Amoxicillin ; Anticonvulsants ; Drug Hypersensitivity Syndrome* ; Female ; Fever ; Humans ; Quetiapine Fumarate ; Skin ; Valproic Acid

No abstract available.
Adult ; Aged ; Biopsy ; Colon/*microbiology/pathology ; Fusobacterium/genetics/*isolation & purification ; Humans ; Inflammatory Bowel Diseases/microbiology/*pathology ; Male ; Middle Aged ; Polymerase Chain Reaction ; RNA, Ribosomal, 16S/chemistry/genetics/metabolism ; Republic of Korea ; Sequence Analysis, DNA ; Young Adult

PURPOSE: Fanconi anemia(FA) is a rare autosomal recessive disorder characterized by progressive bone marrow failure and congenital malformations. Patients with FA have aplastic anemia(> 90%), leukemia(10~15%), myelodysplasia(5%) and liver(5%) and other tumors(5%). In the International FA Registry study myelodysplasia in FA patients was detected at a median of 13 years. Presentation of FA with myelodysplasia in an infant should be extremely rare. CASE: A 3-month-old infant presented with anemia and poor feeding. The initial hemogram showed: hemoglobin, 4.6 g/dL; MCV, 104.1 fL/pg; white cell count, 4,300/microL; neutrophils, 450/microL; platelets, 23,000/microL. The bone marrow was normocellular, with findings of macrocytic anemia and dyserythropoiesis, and less than 5% of myeloid blasts, compatible with myelodysplastic syndrome(refractory anemia). The patient had multiple cafe-au-lait spots, hypopigmented nevi, broad nasal bridge, micrognathia, and thumb and toe anomalies. FA was confirmed by chromosomal hypersensitivity to diepoxybutane and mitomicin C. Supportive treatment with oxymetholone and prednisolone failed to improve hematologic and clinical findings. The patient succumbed to sepsis, pneumonia and meningitis due to Pseudomonas aeruginosa at 20 month of age. Clonal cytogenetic anomalies were not found. CONCLUSION: We reported here a rare case of FA presenting with myelodysplasia at the age of 3 month.
Anemia ; Anemia, Macrocytic ; Bone Marrow ; Cafe-au-Lait Spots ; Cell Count ; Cytogenetics ; Fanconi Anemia* ; Humans ; Hypersensitivity ; Infant* ; Meningitis ; Myelodysplastic Syndromes* ; Neutrophils ; Nevus ; Oxymetholone ; Pneumonia ; Prednisolone ; Pseudomonas aeruginosa ; Sepsis ; Thumb ; Toes

BACKGROUND: Decreased activities of daily living (ADL) in elderly people are related to decreased quality of life and death and are a clinically important issue. However, few studies have investigated the various characteristics and risk factors for a decline in ADL among elderly in-patients in geriatric hospitals in Korea. METHODS: In total, 163 elderly in-patients with dementia in three geriatric hospitals located in Incheon, Gwangju and Yongin, Korea were surveyed prospectively for associated factors of a decline in ADL after 6 months. RESULTS: On average, the subjects were 79.4+/-7.6 years old, and 67.5% were female. Approximately 63% had Alzheimer type dementia, 36.8% only attended primary school, 73.0% were widowed, and 38.7% had been admitted to hospitals for less than 1 year. The Korean version of the Mini Mental State Examination (K-MMSE) score was 14.1+/-6.5, and the clinical dementia rating was 1.9+/-0.9. In total, 8.6% were bed-ridden and 59.5% and 64.6% had never experienced fecal and urinary incontinence, respectively. Total ADL scores declined after 6 months follow-up, and significantly associated factors were low K-MMSE score, fecal incontinence, and co-existence of fecal and urinary incontinence. CONCLUSION: Low K-MMSE scores, fecal incontinence, and the co-existence of fecal and urinary incontinence were associated with ADL declines in elderly in-patients with dementia in long-term care hospitals located in three cities in Korea.
Activities of Daily Living ; Aged ; Alzheimer Disease ; Dementia ; Fecal Incontinence ; Female ; Follow-Up Studies ; Humans ; Korea ; Long-Term Care ; Prospective Studies ; Quality of Life ; Risk Factors ; Urinary Incontinence ; Widowhood