The Kniest syndrome is characterized by disproportionate dwarfism and Kyphoscoliosis which may be associated with flat facies with prominent eyes, cleft palate, hearing loss, myopia and limited joint motion. The skeletal abnormalities are recognizable at birth with shortening and deformity of the extremities and stiff joints. Marked lumbar lordosis and kyphoscoliosis develop in childhood, resulting in disproportionate shortening of the trunk. We experienced a case of kniest syndrome, confirmed by clinical features, radiological features, and histological examination of cartilage. A brief review of the related literature is presented.
Animals ; Cartilage ; Cleft Palate ; Congenital Abnormalities ; Dwarfism ; Extremities ; Facies ; Hearing Loss ; Joints ; Lordosis ; Myopia ; Parturition

Drug-induced toxic epidermal necrolysis (TEN) is a bullous erythematous disease that is characterized by the appearance of scaled lesions and large sheets of pilling on the skin. A caseof TEN occured in a 10 month old female patient. The characteristic skin lesions of TEN developed after oral administration of carbamazepine for a partial seizure. Thig case consists of prodrome of malaise, fever, anorexia, and conjunctivity followed by erythema & flaccid bullae formation. Diagnosis was confirmed by histologic findings. This patient was treated with a massive systemic corticosteroid, antibiotics, fluid and electrolytes, and topical measures. Authors experienced an extremely rare case of TEN dur to carbamazepine, So report it with a brief review of literature.
Administration, Oral ; Anorexia ; Anti-Bacterial Agents ; Carbamazepine ; Diagnosis ; Electrolytes ; Erythema ; Female ; Fever ; Humans ; Infant ; Seizures ; Skin ; Stevens-Johnson Syndrome*

Here, we report the case of a 43-year-old female who was diagnosed with a cotyledonoid dissecting leiomyoma (CDL) of the uterus. CDL is a recently described and extremely rare variant of a benign leiomyoma that can grossly masquerade as a malignancy. The 13-cm sized tumor was located primarily on the extrauterine surface as an intrauterine continuity, which showed dark red, congested, bulbous protuberances. It was multinodular appearance, encasing the bilateral adnexae and the left iliac vein. Microscopically, the nodules were separated by extensive hydropic degeneration. The nodules were composed of cigar-shaped spindle cells with no mitosis, cellular pleomorphism or coagulation necrosis. They also showed an intravascular luminal growth pattern. CDL with intravascular growth was diagnosed after excluding intravascular leiomyomatosis, disseminated peritoneal leiomyomatosis, and benign metastasizing leiomyoma. The present case is the second reported case of CDL in Korea. Recognition of this rare and bizarre, malignancy-mimicking leiomyoma is crucial to prevent inappropriate treatment.
Adult ; Estrogens, Conjugated (USP) ; Female ; Humans ; Iliac Vein ; Korea ; Leiomyoma* ; Leiomyomatosis ; Mitosis ; Necrosis ; Phenobarbital* ; Uterus*

BACKGROUND: Thin hairs are critical to achieve natural result in female hairline correction surgery. However, there are few studies on the usefulness of hair thinning by intense pulsed light (IPL) after hairline correction surgery in East Asian females. METHODS: Hair thinning using IPL was performed in 54 women who had complained about thick hairs along the frontal hairline after hairline correction surgery. Patient mean age was 31.2 years old and patients were an average of 2.1 years post-hairline correction surgery. Initial treatment used 10 J, while second and third sessions were conducted with 10 to 15 J according to responsiveness to treatment. RESULTS: Mean thickness of individual hairs assessed before the procedure was 78.86 µm. The mean number of procedures was 1.6 per patient. Forty of 54 subjects (74%) achieved satisfactory hair thinning with only one procedure from 78.01 to 66.14 µm after treatment. The measured thickness was 66.43 µm at the end of the first year in patients who were satisfied after one procedure. Thirteen cases achieved satisfactory hair thinning after two sessions. Mean thickness was 74.44 µm and 67.51 µm, before and after the second session. One case required a third session with 15J, thinning from 89.00 to 66.50 µm. CONCLUSIONS: Hair thinning by IPL is a very useful method to provide a natural look after hairline correction surgery in East Asians, who have naturally thick hair.
Asian Continental Ancestry Group* ; Female ; Hair ; Hair Follicle ; Hair Removal* ; Humans ; Laser Therapy ; Methods

Central nervous system (CNS) neoplasms are the second most common childhood malignancy after leukemia and the most common solid organ neoplasm in children. Diagnostic dilemmas with small specimens from CNS neoplasms are often the result of multifactorial etiologies such as frozen or fixation artifact, biopsy size, or lack of knowledge about rare or unfamiliar entities. Since the late 1950s, ultrastructural examination has been used in the diagnosis of CNS neoplasms, though it has largely been replaced by immunohistochemical and molecular cytogenetic studies. Nowadays, pathologic diagnosis of CNS neoplasms is achieved through intraoperative cytology, light microscopy, immunohistochemistry, and molecular cytogenetic results. However, the utility of electron microscopy (EM) in the final diagnosis of CNS neoplasms and investigation of its pathogenetic origin remains critical. Here, we reviewed the distinguishing ultrastructural features of pediatric CNS neoplasms and emphasize the continuing value of EM in the diagnosis of CNS neoplasms.
Artifacts ; Biopsy ; Central Nervous System Neoplasms* ; Central Nervous System* ; Child ; Cytogenetics ; Diagnosis ; Humans ; Immunohistochemistry ; Leukemia ; Microscopy ; Microscopy, Electron

PURPOSE: The study purposes were to describe the process of developing the Korean versions of the Six Dimension Scale of Nursing Performance (Six-D) and Student Nurse Stress Index (SNSI) and psychometric evaluation of the two measurements. METHODS: This was a methodology study using a descriptive cross-sectional design with 51 nursing students in 4th year of university. Internal consistency reliability was assessed using Cronbach alphas. Construct validity was determined by exploring correlations among Six-D, SNSI, objective structured clinical examination (OSCE), self-efficacy and grade point average (GPA). RESULTS: Internal consistency reliability of Six-D and SNSI was acceptable with Cronbach's alpha of .95 and .82. Correlation analysis to determine construct validity revealed that Six-D presented positive correlations with OSCE (r=.109~.272) and self-efficacy (r=.005~.161) and negative correlation with GPA (r=-.246~-.394), although all were not statistically significant. SNSI presented all negative correlations with OSCE (r= -.007~-.238), self-efficacy (r=-.246~-.394), and GPA (r=-.092~-.426) and were mostly statistically significant except OSCE. CONCLUSION: Six-D needs more evidence to confirm validity to predict observed clinical competency and theoretical relationships with self-efficacy and GPA. However, SNSI presented trends of expected relationships with relevant variables. Therefore, further research is recommended in testing validity of Six-D with other student populations.
Asthma ; Humans ; Psychometrics ; Students, Nursing

Vestibular schwannoma (VS) usually present the widening of internal auditory canal (IAC), and these bony changes are typically limited to IAC, not extend to temporal bone. Temporal bone invasion by VS is extremely rare. We report 51-year-old man who revealed temporal bone destruction beyond IAC by unilateral VS. The bony destruction extended anteriorly to the carotid canal and inferiorly to the jugular foramen. On histopathologic examination, the tumor showed typical benign schwannoma and did not show any unusual vascularity or malignant feature. Facial nerve was severely compressed and distorted by tumor, which unevenly eroded temporal bone in surgical field. Vestibular schwannoma with atypical invasion of temporal bone can be successfully treated with combined translabyrinthine and lateral suboccipiral approach without facial nerve dysfunction. Early detection and careful dissection of facial nerve with intraoperative monitoring should be considered during operation due to severe adhesion and distortion of facial nerve by tumor and eroded temporal bone.
Cytochrome P-450 CYP1A1 ; Facial Nerve ; Humans ; Middle Aged ; Monitoring, Intraoperative ; Neurilemmoma ; Neuroma, Acoustic* ; Temporal Bone*

The seated and prone positions are the most common surgical positions used during follicular unit extraction (FUE). Compared to the latter, the former eases centering and enables more optimal surgical field exposure due to gravitational effects on intraoperative bleeding. Furthermore, the surgeon can simultaneously work with multiple assistants, increasing efficiency and reducing operative time. During the harvesting stage of FUE, the patient is often seated in an electric height-adjustable salon chair. Such equipment, however, does not provide support for the head; maintaining a fixed upright position for an extremely long-lasting and delicate surgery that requires loupes with ×5 or higher magnification is challenging for both the surgeon and the patient. On the other hand, a support system that firmly fixes the patient's forehead would have ergonomic benefits during the process of FUE donor harvesting in a seated position. Firm support of the forehead would also enable upward traction to provide tension on the scalp, lessen the gap between the hair exit angle and internal hair angle, and reduce graft torsion, ultimately minimizing follicular injury and optimizing graft quality.
Forehead ; Hair Follicle ; Hair ; Hand ; Head ; Hemorrhage ; Humans ; Operative Time ; Posture ; Prone Position ; Scalp ; Tissue Donors ; Traction ; Transplants

Adenocarcinomas with micropapillary patterns are generally aggressive and show lymphotropism. Only a few reports on pulmonary adenocarcinoma with micropapillary patterns have described cytologic findings. A 70-year-old Korean woman was admitted to the hospital because of intermittent dry cough and chest pain. Cytology after bronchial washing showed neoplastic cells in small, angulated, cohesive clusters consisting of 3-20 cells without a fibrovascular core. The resected right middle lobe showed a tumor occupying almost the entire lobe. Histologically, about 90% of neoplastic cells proliferated with micropapillary morphology in the background of bronchioloalveolar carcinoma. Cytologic smears of a bronchial washing showing tumor cells in small, cohesive clusters without a fibrovascular core may indicate an adenocarcinoma with micropapillary pattern.
Adenocarcinoma ; Adenocarcinoma, Bronchiolo-Alveolar ; Aged ; Chest Pain ; Cough ; Female ; Humans ; Lung