We report a family suffering from Marie Unnas hypotrichosis (8 members affected in 4 generations). At birth they had normal hairs, subsequently loss of hair on the scalp, eyebrow and eyelash. At about 3rd years, coarse twisted scalp hair regrow until puberty when progressive loss from vertex and scalp margin was noticed. On physical examination and lsboratory studies, there was no associated abnormality in the patients. The scanning electron microscopic examination of the affected hair showed cuticular peeling and twisting or angulation of hair shaft.
Adolescent ; Eyebrows ; Hair ; Humans ; Hypotrichosis* ; Parturition ; Physical Examination ; Puberty ; Scalp

No abstract available.
Aneurysm* ; Angiography* ; Circle of Willis*

No abstract available.
Animals ; Blastocyst* ; Embryonic Structures* ; Glucose* ; Mice*

BACKGROUND: Unplanned extubation (UE) of patients requiring mechanical ventilation in an intensive care unit (ICU) is associated with poor outcomes for patients and organizations. This study was conducted to assess the clinical features of patients who experienced UE and to determine the risk factors affecting reintubation after UE in an ICU. METHODS: Among all adult patients admitted to the ICU in our institution who required mechanical ventilation between January 2011 and December 2013, those in whom UE was noted were included in the study. Data were categorized according to noninvasive or invasive management after UE. RESULTS: The rate of UE was 0.78% (the number of UEs per 100 days of mechanical ventilation). The incidence of self-extubation was 97.2%, while extubation was accidental in the remaining patients. Two cases of cardiac arrest combined with respiratory arrest after UE were noted. Of the 214 incidents, 54.7% required invasive management after UE. Long duration of mechanical ventilation (odds ratio [OR] 1.52; 95% confidence interval [CI] 1.32-1.75; p = 0.000) and high ICU mortality (OR 4.39; 95% CI 1.33-14.50; p = 0.015) showed the most significant association with invasive management after UE. In multivariate analysis, younger age (OR 0.96; 95% CI 0.93-0.99; p = 0.005), medical patients (OR 4.36; 95% CI 1.95-9.75; p = 0.000), use of sedative medication (OR 4.95; 95% CI 1.97-12.41; p = 0.001), large amount of secretion (OR 2.66; 95% CI 1.01-7.02; p = 0.049), and low PaO2/FiO2 ratio (OR 0.99; 95% CI 0.98-0.99; p = 0.000) were independent risk factors of invasive management after UE. CONCLUSIONS: To prevent unfavorable clinical outcomes, close attention and proper ventilatory support are required for patients with risk factors who require invasive management after UE.
Adult ; Heart Arrest ; Humans ; Incidence ; Intensive Care Units* ; Critical Care* ; Mortality ; Multivariate Analysis ; Respiration, Artificial ; Risk Factors

It is highly desirable to achieve optimal reproductive performance, reliable morphological and physiological basic data of the reproductive organs. Therefore, seasonal changes in serum testosterone, LH, and FSH concentrations, and morphological changes in testicular epithelial cells were studied in the Korean native pheasant throughout the annual cycle. Mature male pheasants[14-16 months after hatching] were used in this study. The general morphological changes of the epithelia of the seminiferous tubules were observed by dibasic stain, and semithin section from Epon blocks with a phase contrast microscopy. The ultrastructural changes of the the epithelia of the seminiferous tubules were investigated by ultrathin section with transmission electron microscope. The changes in the profiles of the serum FSH, LH, and testosterone concentratioins were measured by RIA[radioimmunoassay]. The results obtained are summarized as follows : 1. There was little variation in the average diameter of the seminiferous tubules from autumn[67.13+/-5.95micrometer] to winter[68.59+/-6.07micrometer], but the highest levels were reached in spring[192.78+/-41.58micrometer]. Thereafter, the diameter decreased slowly in summer[146.57+/-43.68micrometer], then decreased significantly in autumn[67.13+/-5.95micrometer]. 2. Serum testosterne concentration was low from autumn[13.+/-7.21ng/100ml] to winter[17.39+/-13.75ng/100ml], but the highest levels were reached in spring[127.72+/-66.47 ng/100ml]. Thereafter, the concentration was lowest in autumn[13.+/-7.21ng/100ml]. 3. Serum LH concentration increased slowly and linealy from autumn[5.04+/-1.04ng/100ml] to winter[6.23+/-1.08ng/100ml], but the highest levels were reached in spring[11.3+/-3.6 ng/100ml]. Thereafter, the concentration reached the lowest level in autumn[5.04+/-1.04 ng/100ml]. 4. Serum FSH concentration was low from autumn[4.65+/-0.63ng/100ml] to winter[4.2+/-0.98ng/100ml], but the highest levels were reached in spring[17.41+/-8.35ng/100ml]. Thereafter, concentration was the lowest in autumn[4.65+/-0.63ng/100ml]. 5. The seminiferous tubules showed the onset of the spermatongenic cycle in spring but the seminiferous tubules collected in summer exhibited partially degenerative changes. 6. The cytoplasmic process of Sertoli cells of the seminiferous tubules of the pheasant were collected in summer. Many vesicles and degeneratiye changes were included but many number of spermatozoa were embedded partially in the multivesicular bodies in these processes. 7. The diameter of the seminiferous tubules of the pheasant narrowed markedly in autumn, and atrophied in winter. The spermatogonia and Sertoli cells were arranged in monolayer. 8. The myelin figures in the cytoplasmic process of Sertoli cells of the seminiferous tubules of the pheasant in autumn. The nucleus of the Sertoli cells were of a round configuration elongated and oriented perpendicularly to the basement membrane. The results obtained provide basic data for reproductive physiology and are useful for studying the male genital organs of the Korean native pheasant. Structural changes of the seminiferous epithelial cells significantly and postively correlated with serum FSH, LH. The correlation of changes in the hormonal status with alterations of Sertoli cell organells precedes the breeding season.
Basement Membrane ; Breeding ; Cytoplasm ; Epithelial Cells ; Epithelium* ; Genitalia, Male ; Humans ; Male ; Microscopy, Phase-Contrast ; Multivesicular Bodies ; Myelin Sheath ; Physiology ; Seasons* ; Seminiferous Tubules* ; Sertoli Cells ; Spermatogonia ; Spermatozoa ; Testosterone*

OBJECTIVE: There have been numerous follow-up studies of patients who had ruptured or unruptured intracranial aneurysms treated by wrapping technique using various materials have been reported. Our objective was to ascertain whether our particular wrapping technique using the temporalis muscle provides protection from rebleeding and any aneurysm configuration changes in follow-up studies. METHODS: Clinical presentation, the location and shape of the aneurysm, outcomes at discharge and last follow-up, and any aneurysm configuration changes on last angiographic study were analyzed retrospectively in 21 patients. Reinforcement was acquired by clipping the wrapped temporalis muscle. Wrapping and clipping after incomplete clipping was also done. Follow-up loss and non-angiographic follow-up patient groups were excluded in this study. RESULTS: The mean age was 53 years (range 29-67), and 15 patients were female. Among 21 patients, 10 patients had ruptured aneurysms (48%). Aneurysms in 21 patients were located in the anterior circulation. Aneurysm shapes were broad neck form (14 cases), fusiform (1 case), and bleb to adjacent vessel (6 cases). Five patients were treated by clipping the wrapped temporalis, and 16 patients by wrapping after partial clipping. The mean Glasgow coma scale (GCS) at admission was 14.2. The mean Glasgow outcome scale (GOS) at discharge was 4.8, and 18 patients were grade 5. The mean period between initial angiography and last angiography was 18.5 months (range 8-44). Aneurysm size was not increased in any of these patients and configuration also did not change. There was no evidence of rebleeding in any of these treated aneurysms. CONCLUSION: Our study results show that wrapping technique, using the temporalis muscle and aneurysm clip(s), for intracranial aneurysm treatment provides protection from rebleeding or regrowth.
Aneurysm ; Aneurysm, Ruptured ; Angiography ; Blister ; Female ; Follow-Up Studies ; Glasgow Coma Scale ; Glasgow Outcome Scale ; Glycosaminoglycans ; Humans ; Intracranial Aneurysm ; Muscles ; Neck ; Reinforcement (Psychology) ; Retrospective Studies ; Temporal Muscle

OBJECTIVE: Microvascular decompression(MVD) at root exit zone(REZ) of the facial nerve has been largely popularized and it has become the standard treatment for patients with hemifacial spasm(HFS). This sturdy is performed to evaluate the efficacy of MVD over the course of time. METHODS: From 1994 to 2003, 50 patients with HFS who underwent MVD were followed up for more than 6 months. We retrospectively analyzed results with medical records and telephone researches. RESULTS: The mean age of patients at the time operation was 57.6 years and 84% of the patients were female. The mean duration of follow-up after operation was 3.4 years (range 0.5~7.8 years). One day after MVD, 54% of patients had complete relief of spasm immediately. Continuous improvements of HFS were observed during the follow-up period and these improvements were statistically significant with time (P<0.05). Until 6 months after operation, complete relief of spasm was observed in 84% of patients. The delayed relief of spasm was observed in 35.7% of our patients who experienced complete relief. CONCLUSION: The efficacy of MVD in HFS is improves with time. Continuous follow-up evaluations for the duration of more than at least 6 months after MVD are important for the decision of its clinical results because delayed relief of spasms occurs.
Facial Nerve ; Female ; Follow-Up Studies ; Hemifacial Spasm* ; Humans ; Medical Records ; Microvascular Decompression Surgery* ; Retrospective Studies ; Spasm ; Telephone

OBJECTIVE: To compare the outcomes of a method of assigning patients to a vaginal or laparoscopically assisted vaginal approach to hysterectomy. METHOD: Hysterectomy is the most common gynecologic operation. A clinical evaluation was attempted to analyze 100 cases of LAVH which was performed at St. Paul hospital from May 1997 to March 2000, and to compare them with 100 cases of vaginal hysterectomy at the same hospital. we used simple electrosurgical technique without using the disposable staples and other instruments. RESULTS: The mean age of the patients for LAVH was 46.18 years and that of VTH was 50.23 years. The most common indication for LAVH and VTH was Myoma uteri. The mean Hb change in LAVH was 2.15 and the Hb change of VTH was 2.17. The mean operative time of LAVH was 142.96 minutes and that of VTH was 77.06 minutes. The mean weight of uterus for LAVH was 221g and the mean weight of VTH was 182 g. The total percentage of urinary tract injury for LAVH was 3 % and that for VTH was 2 %. CONCLUSION: VTH, if possible, must be considered as a primary choice since it is were efficient than LAVH in cost, cosmetic aspects and complications. Further LAVH may be replaced with a laparotomy for hysterectomy in case of an insufficient operational experience, a previous abdominal operation hystory and the incapability of being indicated for VTH.
Female ; Humans ; Hysterectomy ; Hysterectomy, Vaginal* ; Laparotomy ; Myoma ; Operative Time ; Urinary Tract ; Uterus

No abstract available.
Arteriovenous Fistula*

Conventional medical therapies have not been very successful in treating adults with refractory septic shock. The effects of direct hemoperfusion using polymyxin B and veno-arterial extracorporeal membrane oxygenation (ECMO) for refractory septic shock remain uncertain. A 66-year-old man was admitted to the emergency department and suffered from sepsis-induced hemodynamic collapse. For hemodynamic improvement, we performed direct hemoperfusion using polymyxin B. Computed tomography scan of this patient revealed emphysematous pyelonephritis (EPN), for which he underwent emergent nephrectomy with veno-arterial ECMO support. To the best of our knowledge, this is the first report of successful treatment of EPN with refractory septic shock using polymyxin B hemoperfusion and nephrectomy under the support of ECMO.
Adult ; Aged ; Emergency Service, Hospital ; Endotoxins ; Extracorporeal Membrane Oxygenation* ; Hemodynamics ; Hemoperfusion ; Humans ; Nephrectomy* ; Polymyxin B ; Pyelonephritis ; Shock, Septic*