Erdheim Chester disease (ECD) is very rare non-Langerhans cell histiocytosis (LCH) which occurs in the skeletal system and multiple organs. As it is progressive, sometimes it causes fatal results. However, it is often misdiagnosed as LCH or multiple bone metastasis and, thus, is very difficult to diagnose. In Korea, only 10 cases were first reported in 1999. In particular, there have been a few orthopedic approaches or reports in English-speaking literatures, and no report has been issued in Korea. The authors performed bone biopsy in patients with knee and lower extremity pain who were referred for the integrated treatment. We attempts to report this diagnosis experience with literature review.
Biopsy ; Erdheim-Chester Disease ; Histiocytosis ; Histiocytosis, Langerhans-Cell ; Humans ; Knee ; Korea ; Lower Extremity ; Neoplasm Metastasis ; Orthopedics

PURPOSE: This study was undertaken to determine whether the clinical presentation of patients with central precocius puberty(CPP) varies according to the etiology, whether this permits the differentiation between idiopathic and organic forms and to examine whether LH determination in a single timed blood sample after GnRH administration is sufficient to diagnose CPP. METHODS: This study included 33 girls with signs of breast development, of whom 23 were diagnosed as definite central precocious puberty. Sixteen patients had idiopathic CPP; the remaining 7 patients had organic CPP. Ten patients were classified as non-CPP. Potential clinical and laboratory predictors of CNS abnormalities were assessed and GnRH stimulation test was done. RESULTS: The age of onset in patients with organic CPP was 4.11+/-2.08 years, whereas the age in patients with idiopathic CPP was 7.25+/-1.34 years. This parameter is the only one showing statistical significance. We compared sensitivities and specificities at 0, 15, 30, 60, 90 and 120 min which yielded sensitivities of 8.7%, 87.0%, 91.3%, 87.0%, 73.9%, 60.9%. CONCLUSION: It is impossible to exclude a central nervous system lesion in patient with central precocious puberty without performing central nervous system imaging. However, this study indicates earlier the onset of disease, higher the possibility of presence of CNS lesion. According to the mean GnRH stimulated LH levels and sensitivity at each times, a single blood sample obtained for LH determined after GnRH administration at 30 min can be used to diagnose the central precocious puberty.
Age of Onset ; Breast ; Central Nervous System* ; Female ; Gonadotropin-Releasing Hormone* ; Humans ; Luteinizing Hormone ; Puberty, Precocious*

This study was undertaken to investigate the effect of superoxide dismutase(SOD) to sunburn cell production and development of UV-induced ear swelling reaction in mouse skin after ultraviolet irradiation. In this study, a total of 60 ICR female albino haired mice were used and divided into two groups, A(UVB: 150mJ/cm) and B(UVB:300mJ/cm). Groups of mice were injected intravenouly with SQD(300mJ,/1000cm) just befare UVR and after completion of UVR. The results were as follows . 1. The number of sunburn cells was significantly decreased by injection of SOD (300ug.1000ug)(p<0.05). 2. The number of sunburn cells in a group of mice which was given SOD 1000ug was significantly decreased more than in SOD 300pg(p<0.05). 3. Ear swelling reaction was not significantly suppressed by injection of SOD(300 Pa. 100SC)(p<0 05)
Animals ; Ear ; Female ; Hair ; Humans ; Mice* ; Skin* ; Sunburn* ; Superoxide Dismutase* ; Superoxides*

PURPOSE:Varying clinical phenotypes are associated with the chromosome 22q11.2 microdeletion syndrome. The endocrine manifestation are latent or overt hypoparathyroidism, thyroid dysfunction and short stature. This study was undertaken to investigate frequencies of endocrine abnormalities and short stature in patients with the chromosome 22q11.2 microdeletion syndrome. METHODS:Forty three unrelated patients were diagnosed having chromosome 22q11.2 microdeletion syndrome. Chromosomal microdeletion was confirmed by fluorescent in situ hybridation (FISH) with DNA probe (22q11.2 LSI TUPLE1 from Vysis). Serum total calcium and intact parathyroid hormone (PTH) were measured in all patients. Thyroid function tests including free thyroxine(T4), thyroid stimulating hormone (TSH) and thyroid autoantibodies were performed in all patients. Insulin-like growth factor-1 (IGF-1) was measured in 10 patients. Height, weight and body mass index were compared with chronological age in all patients. RESULTS:Seven patients (16%) had an overt hypoparathyroidism, presenting with hypocalcemic tetany. Thirteen patients (31%) showing hypocalcemia with normal PTH were regarded as having latent hypoparathyroidism since their PTH secretion response was blunted. Out of 2 patients with thyroid diseases, one patient had Graves disease and the other had Hashimoto thyroiditis. Five patients (12%) were below the 3rd percentile in height at evaluation. The BMI was below the 5th percentile in 23% of patients. CONCLUSION: Twenty patients (47%) presented with overt and latent hypoparathyroidism. Interestingly, autoimmune thyroid diseases such as Graves disease and Hashimoto thyroiditis were associated in patients with chromosome 22q11.2 microdeletion, indicating predisposition to autoimmune disorders. Therefore, a careful endocrine and growth evaluation is needed in these patients.
Autoantibodies ; Body Mass Index ; Calcium ; DNA ; Graves Disease ; Hashimoto Disease ; Humans ; Hypocalcemia ; Hypoparathyroidism ; Parathyroid Hormone ; Phenotype ; Tetany ; Thyroid Diseases ; Thyroid Function Tests ; Thyroid Gland ; Thyrotropin

BACKGROUND: Long-term oral itraconazole and terbinafine are widely used in the treatment of onychomycosis. Accelerated nail growth in patients with itraconazole has been described in several reports. However, there has been no report regarding the effect of antifungal agents on cultured nail matrix cells(NMCs). OBJECTIVE: We applied several antifungal agents on cultured human NMCs and epidermal kera- tinocytes(EKs) to compare the cytotoxicity of several antifungal agents and also to verify possible stimulating effects of itraconazole and 6-hydroxyitraconazole on nail growth. METHODS: To evaluate the effect of antifungal agents, the 3-(4,5-dimethylthiazo1-2-yl) 2,5-diphenyl- tetrazolium bromide(MTT) test, tritiated thymidine incorporation test, and lactic dehydrogenase(LDH) leakage test were used. RESULTS: Dose dependent decreases in cell viability and DNA synthesis, and dose dependent increases in LDH liberation were observed in cultured human NMCs and EKs after exposure to several antifungal agents. The dose-response reaction patterns for NMCs and EKs to antifungal agents were similar. The cytotoxicity potency of several antifungal agents measured by each method were slightly different. Itraconazole and 6-hydroxyitraconazole did not show stimulating effects on cell proliferation in in vitro monolayer cell culture systems. CONCLUSION: These observations suggest that itraconazole appeared less cytotoxic but showed no stimulating effects on nail matrix cell proliferation in vitro. Cultured human EKs as well as NMCs may be useful in evaluating the effects of agents which are involved in nails.
Antifungal Agents* ; Cell Culture Techniques ; Cell Proliferation ; Cell Survival ; DNA ; Humans* ; Itraconazole ; Keratinocytes* ; Onychomycosis ; Thymidine

Persistent light reaction, actinic reticuloid, photosensitive eczema are three chronic photodermatoses with a feature of clinical, histological, and photobiologieal characteristics in common. These conditions were given the name of chronic persistent photosensitivity. Chronic persistent photosensitivity with the feature of photosensitive eczema developed in a 68-year-old male patient who had experienced persisited dermatitis. On phototesting the patient showed extremely severe photosensitivity to the short wave UVB. A skin biopsy showed microscopic finding of eczematous dermatitis.
Actins ; Aged ; Biopsy ; Dermatitis ; Eczema ; Humans ; Male ; Radio Waves ; Skin

Adenoma of the nipple is a benign tumor of the lactiferous ducts of the nipple which frequently causes erosion of the surface. It can clinically mmic Paget's disease and histologically be misdiagnosed as a well-differentiated adenocarcinoma. A 20-year-old woman developed an erythematous oozing patch on the right nipple two years ago, which subsequently developed iiito verrucous surfaccd papules. A biopsy specimen of the lesion showed focal acanthosis of the epidermis. The tumor was composed of fairly well-circumscribed, glandlike structures within the nipple stroma. Ductlike struciures, papillary areas, solid nests of cells and cysts lined by squarnous epithelium, could be seen, Two cell types lining the ducts were recognized; inner columnar cells and peripherally located cuboidal cells. Although simple excision of the adenoma is the treatment of choice, our case was treated successfully by electrucoagulation.
Adenocarcinoma ; Adenoma* ; Biopsy ; Epidermis ; Epithelium ; Female ; Humans ; Nipples* ; Young Adult

No abstract available.
Fractures, Malunited* ; Radius*

We report a case of penile lymphangioma circumscriptum developed in a 22- year-old male soldier. Rice-sized papule on glans penis was noted at birth and progressed to yellow straw-colored, peanut-sized, asymptomatic grouped papulovesicles resembling frog spawn. The predilection sites of lymphangioma circumscriptum are the abdomen, axilla and tongue. Glans penis is a very rare site. Histopathologic findings showed papillornatosis, hyperkeratosis and cystically dilated lymph vessels lined by a single layer of endothelial cells.
Abdomen ; Axilla ; Endothelial Cells ; Humans ; Lymphangioma* ; Male ; Military Personnel ; Parturition ; Penis ; Tongue

No abstract available.
Nasal Bone*