Histiocytosis-X is a term used to define three diseases with similar morphologic characteristics : Letterer-Siwe diseae, Hand-Schuller-Christian disease and Eosinophilic granuloma. In general, they differ in terms of their age of onset, severity of clinical course and site of involvement. Eosinophilic granuloma typically is seen in young adults. Eosinophilic granuloma is diagnosed in the presense of diffuse pulmonary infiltrate, bony involvement. However, the Pulmonary radiologic findings of eosinophilic granuloma are variable accordinary to stage of disease. therefore pathologic diagnosis of involving site is essential for confirmative diagnosis of eosinophilic grananuloma. Pathologically. the three disease are characterized by granulomatous infiltration of alveolar septa and bronchial walls and often involvement of bone. The hallmark of this disease is proliferation of the Langerhans' cell. The identifying feature is the X-body or Birbeck granule that is present in Langerhans' cells and histiocytic cells found in the lung of EG patient. We report a case of bilateral, recurrent and spontaneous pneumothoraces in a 21 year old man with pulmonary histiocytosis-X which is confined by eosinophilc granuloma in bone marrow biopsy and ultrastructural examination in cells obtained from BAL.
Age of Onset ; Biopsy ; Bone Marrow ; Diagnosis ; Eosinophilic Granuloma ; Eosinophils ; Granuloma ; Histiocytosis, Langerhans-Cell* ; Humans ; Lung ; Pneumothorax* ; Young Adult

No abstract available.
Mesenteric Artery, Superior

PURPOSE: We report a case of giant conjunctival nevus and compare differential diagnosis between giant conjunctival nevus and conjunctival malignant melanoma. CASE SUMMARY: A 46-year-old male presented with brown and elevated conjunctival mass in his right eye since childhood. The mass was located at the superior bulbar conjunctiva involving the superior cornea. The mass was 16 x 9 mm in size and elevated. Feeding vessels, intrinsic vessels and various cyst sizes were observed inside the mass. Resection of the conjunctival mass and amniotic membrane transplantation were performed. The histopathological diagnosis was conjunctival nevus. CONCLUSIONS: Conjunctival nevus is a benign conjunctival tumor with excellent prognosis, often confused with conjunctival melanoma. Both conjunctival nevus and conjunctival malignant melanoma are commonly located in the bulbar conjunctiva, pigmented and often have feeder and intrinsic vessels. Conjunctival nevus has an intralesional cyst, which is a key differentiating characteristic from malignant melanoma as many other features overlap. The change in tumor size, increased pigmentation and corneal invasion are features suspect of malignant transformation and surgical excision and histologic examination are recommended for those lesions. Surgical excision for giant conjunctival nevus can cause several ocular complications such as symblepharon. Conjunctival reconstruction with amniotic membrane transplantation is useful for preventing complications.
Amnion ; Conjunctiva ; Cornea ; Diagnosis ; Diagnosis, Differential ; Humans ; Male ; Melanoma* ; Middle Aged ; Nevus* ; Pigmentation ; Prognosis

PURPOSE: To evaluate the clinical characteristics of intraorbital foreign bodies as well as the treatment outcomes. METHODS: This was a noncomparative interventional case series. Clinical data and radiographic images were gathered via retrospective chart reviews of 14 patients who underwent surgical removal of intraorbital foreign bodies by an oculoplastic surgeon at the Asan Medical Center, Seoul, Korea between July 2012 and November 2015. RESULTS: The mean age of patients was 45.1 years and 13 patients (92.9%) were male. There were 9 metallic; 3 nonmetallic, inorganic; and 2 organic intraorbital foreign bodies in this series. The most common orbital complication was orbital wall fracture (8, 57.1%), and one patient had orbital cellulitis associated with a wooden foreign body. Six patients (42.9%) underwent surgical removal of foreign bodies in a delayed setting, and 4 of them needed surgery to allow for the brain magnetic resonance image tests to evaluate neurologic problems. There were 6 patients (42.9%) who had a postoperative corrected visual acuity worse than 20/200, and all of them had poor visual acuity at the time of injury due to associated eyeball or optic nerve injuries. Four patients (28.6%) had eyeball movement limitations from the initial trauma, but only 1 patient had persistent limitations postoperatively. There were no other complications associated with surgical removal. CONCLUSIONS: The majority of patients with intraorbital foreign bodies were male who had periorbital traumas. The most common foreign body was metal, and orbital wall fractures were common. The poor visual prognosis was related to the eyeball or optic nerve injuries from the initial trauma. The urgent surgical removal should be performed for organic foreign bodies or associated orbital/ocular injuries. Metallic foreign bodies may also be considered for removal to allow for possible brain magnetic resonance image evaluations in the future.
Brain ; Chungcheongnam-do ; Foreign Bodies* ; Humans ; Korea ; Male ; Optic Nerve Injuries ; Orbit ; Orbital Cellulitis ; Prognosis ; Retrospective Studies ; Seoul ; Visual Acuity

Mucinous ductal ectasia(MDE) is a newly described entity of mucinous neoplasm of pancreas with characteristic endoscopic and pancreatographic findings. It is charaeterized by a patulous duodenal papilla extruding mucus and a pancreatogram showing dilatation with amorphous filling defects, communication of the mass with the pancreatic duct. MDE is intraductal lesion consisting of dilated "cystified" ducts lined by mucin-producing columnar cells. The lesion is usually located in the head or uncinate process. When the radiographic appearance of the ERCP cannot provide sufficient information for definite diagnosis, additional endoscopic visualization is desirable. With the advent of pancreatoscopy, an endoscopic procedure is now available that has proven to enhance diagnostic accuracy. The 0.8-mm ultrathin pancreatoscope allows macroscopic diagnosis under direct vision. Endoscopic sphincterotomy is not required, and insertion into the pancreatic duct is feasible in most cases with the aid of guidewires. Major indications are unclear filling defects on ERCP, strictures of uncertain origin, and duct cut-offs. A limitation of this procedure are the lack of angulation, insufficient illumination, fragility of endoscopic equipment, and nonspecific findings. In addition, biopsy while directly viewing the lesion is impossible at present. We experienced one case of MDE who presented with the symptom of weight loss. Ultrathin pancreatoscopy was useful for the direct visualization af pancreatic duct in the differential diagnosis of filling defects of main pancreatic duct.
Biopsy ; Cholangiopancreatography, Endoscopic Retrograde ; Constriction, Pathologic ; Diagnosis ; Diagnosis, Differential ; Dilatation ; Dilatation, Pathologic* ; Head ; Lighting ; Mucins* ; Mucus ; Pancreas ; Pancreatic Ducts ; Sphincterotomy, Endoscopic ; Weight Loss

PURPOSE: To assess the metabolite ratios in gliomas to determine whether the metabolic information obtained by using by using in vivo single vexel 1H magnetic resonance spectroscopy(MRS) can be used as a marker for the grading of malignancy. MATERIALS AND METHODS: A total of 28 1H MR spectra from brain tumors in 27 patients with pathologically-proven gliomas were recorded. Seven patients had low grade gliomas (grade II astrocytoma in three, oligodendroglioma in three and mixed glioma in one), six had anaplastic gliomas (grade III astrocytoma in three and oligodendroglioma in three), and 14 had glioblastoma multiformes (grade IV), 1H MRS was performed on a 1.5T MRunit using PRESS sequence with a TR of 2000ms, a TE of 270 or 135ms and a voxel size of cm for all spectra. Relative lactate levels, NAA/Cho, NAA/Cr and Cho/Cr ratios were measured based on the peak heights of each resonance and compared among gliomas. RESULTS: Most tumors demonstrated decreased NAA, elevated Cho and lactate. Relatively high lactate and Cho levels and markedly decreased NAA level were more frequently observed in the high grade gliomas than in low grade gliomas. Marked elevation of lactate level in the solid component of the tumor was mostly observed in high grade gliomas. In a patient with gliomatosis cerebri, 1H MRS demonstrated a spectral pattern of tumor in filtration in an area that on MR images was apparently normal. However, NAA/Cr, NAA/Cho and Cho/Cr ratios did not significantly correlate, however, with the histologic grading of malignancy. Because of the partial volume effect, the heterogeneity of tumors containing solid and cystic or necrotic components within avoxel limited the interpretation of 1H MRS data for the grading of malignancy. CONCLUSION: The results suggest that in some patients in vivo single voxel 1H MRS may be useful for grading the malignancy of gliomas and evaluating the exact extent of tumors. In solid gliomas, the relative level of lactate appears to be a good markerfor the grading of malignancy.
Astrocytoma ; Brain Neoplasms ; Filtration ; Glioblastoma ; Glioma* ; Humans ; Lactic Acid ; Magnetic Resonance Spectroscopy* ; Neoplasms, Neuroepithelial ; Oligodendroglioma ; Population Characteristics

Pseudohypoparathyroidism is a rare disease that is characterized by target cell resistance to the effects of parathyroid hormone and this disease is classified into various types depending on the phenotypic and biochemical findings. The patients with pseudohypoparathyroidism present with the clinical and biochemical features of hypoparathyroidism, but they have an increased serum level of parathyroid hormone. We experienced a case of pseudohypoparathyroidism in a 24 years old woman who had Graves' disease at that time. She had hypocalcemia, hyperphosphatemia, an elevated serum parathyroid hormone level and a normal urinary basal cyclic AMP(adenosine monophosphate) level. She also had a normal phenotypic appearance. Therefore, she was classified as suffering with pseudohypoparathyroidism type II. The clinical and laboratory abnormalities were improved by calcium supplementation in addition to vitamin D. To the best of our knowledge, this is the first case of pseudohypoparathyroidism combined with Graves' disease in Korea.
Calcium ; Female ; Graves Disease ; Humans ; Hyperphosphatemia ; Hyperthyroidism ; Hypocalcemia ; Hypoparathyroidism ; Korea ; Parathyroid Hormone ; Pseudohypoparathyroidism ; Rare Diseases ; Stress, Psychological ; Vitamin D

No abstract available.
Median Nerve*

No abstract available.
Sciatic Nerve*

BACKGROUNDS: Standardization of nucleic acid amplification techniques (NAT) which can be achieved by the use of standard to validate reproducibility and sensitivity in each assay run is necessary before the introduction of such methods for routine screening of blood and blood products for viral contaminants. The objective of this study was to analyze the serological and genotypic characteristics of HCV positive plasmas and to manufacture the HCV RNA national standard candidate. METHODS: We obtained three plasmas from Blood Transfusion Research Institute, Korea, with highly positive HCV RNA plasmas (#37, #40, #46) and with normal plasma for dilution. All the plasmas were confirmed by enzyme immunoassay (EIA) test for anti-HIV, HBsAg, anti-HCV and by polymerase chain reaction(PCR) for HBV DNA, HIV RNA, HCV RNA. The genotypes of those were confirmed by INNO-LiPA HCV II. HCV RNA national standard candidate was manufactured by dispensing the diluted plasma into about 2,000 vials. Each vial was rapidly frozen using liquid nitrogen and was kept in refrigerator at -70 degrees C. RESULTS: All plasmas were identified as anti-HIV, HBsAg, HBV DNA, and HIV RNA negative plasmas. The genotypes of those were confirmed as 1b for #37, 1b or 2 for #40 and 2a or 2c for #46, respectively. Sample #37 was selected as the candidate material. After manufacturing, we obtained 1,944 vials for the candidate. CONCLUSION: In this study, we analyzed HCV positive plasmas and manufactured the HCV RNA national standard candidate. In near future, this material would be established for national standard to increase in the safety of blood and blood products in Korea.
Academies and Institutes ; Blood Transfusion ; DNA ; Genotype ; Hepacivirus ; Hepatitis B Surface Antigens ; HIV ; Immunoenzyme Techniques ; Korea ; Mass Screening ; Nitrogen ; Nucleic Acid Amplification Techniques ; Plasma* ; RNA*