We report a case of epidermolysis bullosa simplex occurred in a 12-year-old girl, who presented with intractable bullous lesions of the hands and feet after minor traumas ever since her early neonatal period. The bullous lesions were noted on the 2nd and 4th webs of the fingers and dorsum of the hands as well as on the skin of the ankle. The lesions were healed without scar formation. Family history was not contributary and seasonal pattern was not noted. Histologic sections revealed intraepidermal bullae just above the well preserved basement membrane. Electron microscopic findings revealed cytolysis of basal cells with preservation of the basement membrane, indicating the epidermal type of epidermolysis bullosa simplex.
Infant, Newborn ; Humans

The fatty hamartoma of pericardium is characterized by abnormal accumulations of adipose tissue forming a recognizable mass. We reported a case that could best be put into "fatty hamartoma" group. This 66 year old male presented with atrial fibrillation came in for a anterior mediastinal mass. He received an operation for adenocarcinoma of ascending colon several months ago. The chest CT revealed an inhomogeneous mass in the right anterior mediastinal area, which was not clearly demarcated from the right cardiac border. The heart was deviated to the left side due to the mass. Surgical excision was performed under the impression of cardiac teratoma. The large mass was well encapsulated, and was bright yellow and partly pink, and measured 12x8 cm. Microscopically, the mass consisted predominantly of mature fat cells and abundant fibrous tissue with scattered nests of primitive cardiac muscle cells. Reviewing the literature we found the term "fatty hamartoma" could also refer to rhabdomyolipoma or fibrolipoma. Since it contains entrapped cardiac muscle cells, abundant fibrous tissue, multiple blood vessels and fetal fat cells, it could best the categorized into "hamartoma". The term "fatty" represents it's main component. Therefore we propose the term "fatty hamartoma" that could to be used further.
Male ; Humans ; Adenocarcinoma ; Hamartoma

Radial glia are transdifferentiated into astrocytes within the developing brain and spinal cord. The neural retina contains Muller cells, which are retinal radial glia. Some of the cells that surround the optic nerve head among Muller cells in the chicken retina are called peripapillary glial cells (PPGCs). PPGCs express different molecules compared to typical Muller cells. However, an antigenic PPGC phenotype has not yet been clearly established. In this study, we classified the antigenic PPGC phenotypes and identified the differentiation stages of these cells. At embryonic day (E)8, alphaB-crystallin-positive PPGCs had a bipolar shape with long processes that traversed entire layers of the retina. Pax2 and vimentin were expressed in alphaB-crystallin-positive PPGCs. Glial fibrillary acidic protein (GFAP) immunoreactivity was not observed in PPGCs. At E18, alphaB-crystallin immunoreactivity disappeared from the vitread processes of PPGCs. However, the PPGC cell bodies and ventricular processes contained alphaB-crystallin protein, and the PPGCs retained the same Pax2-positive/vimentin-positive/GFAP-negative profile as that seen at E8. At post-hatch day 120, alphaB-crystallin and Pax2 immunoreactivity was not observed, but vimentin and GFAP expression was clearly observed in the presumptive location of the PPGCs. Furthermore, these two proteins overlapped within that location. Considering that vimentin expression is prolonged until the post-hatching period in chicken brain, these findings suggest that Pax2-negative/vimentin-positive/GFAP-positive PPGCs are phenotypically identical to mature astrocytes in this avian species.
Astrocytes ; Brain ; Chickens ; Glial Fibrillary Acidic Protein ; Neuroglia ; Optic Disk ; Phenotype ; Proteins ; Retina ; Retinaldehyde ; Spinal Cord ; Vimentin

PURPOSE: To evaluate the onset of retinal detachment (RD) in eyes with phacoemulsification and post chamber lens (PE & PCL) implantation. METHODS: We retrospectively analyzed 3023 eyes that had undergone clear cornea incision and PE and PCL implantation and 116 eyes that had undergone scleral incision and PE and PCL implantation. They had been operated from January 1995 to December 2000 by one surgeon and followed up for 4 years or more. RESULTS: Eighteen eyes (0.57%) of the 3139 eyes developed pseudophakic RD within a mean period of 663 days. In the scleral incision group, there were 4 cases of pseudophakic RD (3.4%) of 116 eyes developed in a mean period of 185.2 days. In the clear cornea group, there were 14 pseudophakic RD eyes (0.46%) of the 3023 eyes and the mean period was 799 days. Of these, 3 eyes made up the intraoperative complication group with a mean period 205.6 days. But in the group of cases with no intraoperative complications, the mean period was 962 days. CONCLUSIONS: In high myopic eyes, retinal detachment developed in the earlier postoperative period and at a higher rate than those of non-high myopic eyes after PE and PCL implantation.
Cornea ; Intraoperative Complications ; Phacoemulsification* ; Postoperative Period ; Retinal Detachment* ; Retinaldehyde* ; Retrospective Studies

PURPOSE: The purpose of this radiologic study was to evaluate the geographic patterns of low-energy trochanteric fractures using multiplanar computed tomography (CT) images for application of intramedullary nailing. MATERIALS AND METHODS: In this study, 117 trochanteric fractures (stable fracture, 39 cases, unstable fractures, 78 cases) sustained from simple slip-down were assessed. The mean age was 78.4 years (range, 60-96 years). Multiplanar CT images were assessed for evaluation of geographic features of trochanteric fracture, and the fracture exit and geographic patterns were analyzed. RESULTS: The medial and lateral exit of the trochanteric fracture showed no statistical difference by age, bone density, and comorbid disease. The exit was located at an average distance of 10.2 mm (range, 1.0-22.2 mm) from the tip of the greater trochanter (GT), and the medial exit, average distance of 8.1 mm (range, 0.0-18.3 mm) from the tip of the lesser trochanter. It was also found that there was no comminution around the anteromedial cortex of the fracture, and its contact loss was from fracture deformity. CONCLUSION: Because of no comminution, the contact restoration of the anteromedial cortex resulted in correction of fracture deformity and reduction. Trochanteric nailing by GT tip could be fixed through the proximal fragment of the fracture because the lateral exit is placed at an average distance of 10.2 mm from the GT tip.
Bone Density ; Congenital Abnormalities ; Femur* ; Fracture Fixation, Intramedullary ; Hip Fractures

PURPOSE: This study was performed to evaluate the efficiency of demineralized bone matrix (DBM, Genesis(R)) used for bone defect after operative traetment of benign bone tumors by clinical and radiological methods. MATERIALS AND METHODS: DBM was used to treat bone defect after operative treatment of benign tumor from February 2012 to May 2013. Total 25 benign bone tumor cases (15 males, and 10 females) with mean age of 30.3 were studied. The diagnoses were solitary bone cyst in 9 cases, non ossifying fibroma in 5, fibrous dysplasia in 5, aneurysmal bone cyst in 3 and enchondroma in 3. In categorization by location of tumor, there were 5 cases of distal femur, 4 of proximal tibia, 3 of proximal femur, 3 of proximal humerus, 3 of phalanx, 2 of distal radius, 2 of hip bone, 2 of calcaneus, and 1 of scapula. Autogenous bone was used with DBM in 6 cases, and only DBM used in 19 cases. Mean periods of follow up were 8.7 months (range: 6 to 14 months). Amount of graft resorption and bone formation was observed with compare of post operation radiograph and the difference was shown by percentage. Resorption level was measured by DBM level which could be observed from simple x-ray, and bone formation level by bone trabecular formation level at impaired site. RESULTS: Twenty three cases of total 25 cases showed bone union. In the 23 cases, more than 98% DBM resorption was observed after mean 4.3 months, and more than 98% bone formation was observed after mean 6.9 months. Lesser bone defect sizes showed faster bone formation and it was statistically significant (p=0.036). But other comparative studies on other factors such as, sex, age of patients and combination of autogenous bone were no statistically significant differences in graft resorption and bone formation. And there was no significant complication in periods of follow-up. CONCLUSION: Demineralized Bone Matrix (Genesis(R)) is thought to be useful treatment for bone defect after operative treatment of benign bone tumor, however longer follow-up periods appears to be needed.
Aneurysm ; Bone Cysts ; Bone Matrix* ; Calcaneus ; Chondroma ; Diagnosis ; Femur ; Fibroma, Ossifying ; Follow-Up Studies ; Hip ; Humans ; Humerus ; Male ; Methods ; Osteogenesis ; Radius ; Scapula ; Tibia ; Transplants

No abstract available.
Humans ; Perfusion* ; Prenatal Diagnosis*

In situ hybridization (ISH) using single-stranded DNA probe (ssDNA probe) is a useful method for observing the specific transcripts in cells, since it is convenient to prepare probe which is specific and sensitive. In this study, ssDNA probe for detection of alphaB-crystallin (aBC) mRNA, transcript of a heat shock protein, was prepared and aBC mRNA-expressed cells were spatiotemporally observed in the retina of the developing chick embryos. Single-stranded antisense probe produced by reverse transcription and polymerase chain reaction was identified as a specific probe for aBC mRNA in comparison to negative control using sense probe and immunohistochemistry for aBC protein. In the ISH experiment, aBC mRNA was expressed only in the peripapillary glial cells which are a specific cell type located in the avian retina adjacent to the optic nerve at E12 and E14 retinas. At E16, a small number of aBC mRNA-expressed cells were identified in the nerve fiber layer (NFL) of the retina. At E18, aBC mRNA-expressed cells were observed in the ganglion cell layer (GCL) as well as the NFL. At E20, the number of aBC mRNA-expressed cells was increased in the GCL and the NFL. Based on the same localization of nkx2.2 immunoreactive cells and aBC mRNA-expressed cells, aBC mRNA-expressed cells were identified as oligodendrocytes. These results indicate that ssDNA probe for aBC mRNA detection is very useful tool for oligodendrocyte research such as distribution, migration and differentiation of the cells.
DNA, Single-Stranded ; Ganglion Cysts ; Heat-Shock Proteins ; Immunohistochemistry ; In Situ Hybridization ; Nerve Fibers ; Neuroglia ; Oligodendroglia ; Optic Nerve ; Polymerase Chain Reaction ; Retina ; Reverse Transcription ; RNA, Messenger

It is well known that small heat shock proteins play a role as molecular chaperone. However, during normal development of the cerebellum, expression and distribution of HSP27 and alphaB-crystallin (alphaBC) which are small heat shock proteins have not been reported. To verify the protective role of HSP27 and alphaBC in neurons and glial cells, we examined the expression and distribution of HSP27 and alphaBC in the developing chick cerebellum using immunoblot, immunohistochemical and double immunofluorescence staining. Expression of both HSP27 and alphaBC was first identified in the cerebellum of the embryonic day 14 (E14) embryo, and was increased at E18. Double immunofluorescence analysis with myelin-basic protein (MBP) demonstrated that alphaBC positive (+) cells were mature myelinating oligodendrocytes. alphaBC+ cells were observed in the white matter of the E14 cerebellum. At E18, there were a number of alphaBC+ cells in the white matter and a few cells in the granular layer of the gray matter. On the other hand, HSP27+ cells were observed in the white matter and the Purkinje cell layer at E14. At E18, HSP27+ signals were observed in Purkinje cells and neurons of cerebellar nucleus as well as oligodendrocytes in the white matter and the granular layer. The results that HSP27 and alphaBC were expressed in specific neurons and glial cells in the developing cerebellum suggest that HSP27 and alphaBC may be involved in the protective mechanism for the apoptosis of neurons and the physiological stress occurred in oligodendrocyts during cell maturation.
Apoptosis ; Cerebellar Nuclei ; Cerebellum* ; Embryonic Structures ; Fluorescent Antibody Technique ; Hand ; Heat-Shock Proteins, Small ; Molecular Chaperones ; Myelin Sheath ; Neuroglia ; Neurons ; Oligodendroglia ; Purkinje Cells ; Stress, Physiological

BACKGROUND: Clear and proper feedback is necessary to decrease the gap between knowledge and practice. Feedback can improve clinical competence of medical students up to expertise level.METHODS: A total of 180 4th-year students of Daegu-Kyungbuk consortium area participated in the scheduled feedback program of clinical performance examination on August 26th in 2017. Among them, 167 students filled out the questionnaire on helpfulness of the direct observation and feedback of faculty and standardized patient (SP), helpfulness for history taking (Hx), physical examination (PE), and patient-physician interaction (PPI), and any change of clinical competence and clinical reasoning of the students preand post-feedback. All the responses were measured on 5-point Likert scale.RESULTS: Among the total students, 88.6% responded that direct feedback at the practice is helpful, particularly 95.2% of faculty and 76.6% of SP. A 37.3% answered that it was helpful for all the categories of feedback, but only 25.3% for PE and 24.7% for Hx. A 56.3% responded that feedback is helpful for both disease questions and counseling practice. Mean score of self-assessment by the students increased from 2.52 to 3.36 for Hx, 2.30 to 3.24 for PE, 2.46 to 3.33 for clinical reasoning, 2.84 to 3.59 for PPI, and 2.44 to 3.28 for overall competence. And all these differences were found to be statistically significant (P <0.001)CONCLUSION: A majority of students responded that the direct feedback from faculty and SP was helpful at the clinical practice of Hx, PE, and PPI, and increased the level of clinical competence.
Clinical Competence ; Counseling ; Humans ; Mental Competency ; Physical Examination ; Self-Assessment ; Students, Medical