Osteogenesis imperfecta is a rare affection characterized by fragility of the bones, blue sclerae, and deafness, less freqently by hypermobility of the joints. The etiology is unknown, but it appears to be a mesenchymal defect. A case of osteogenesis imperfecta(tarda form). in a 8 ycar old girl, is presented with a review of the literatures. The chief complaints were bowing deformities of the right upper arm and both lower extremities, stunted growth, blue sclerae, dental defects, weakness of the muscles and pigeon breast. X-Ray showed multiple malunited fractures of the right humerus, both femurs, and bilaterally of tibiae and fibulae.
Arm ; Breast ; Columbidae ; Congenital Abnormalities ; Deafness ; Female ; Femur ; Fibula ; Fractures, Malunited ; Growth Disorders ; Humans ; Humerus ; Joints ; Lower Extremity ; Muscles ; Osteogenesis Imperfecta ; Osteogenesis ; Sclera ; Tibia

In 1953, Lichtenstein suggested to use “Histiocytosis X” as an unifying diagnostic term to represent Eosinophilic granuloma, Hand-Schiiller-Christian disease, and Letterer-Siwe disease. The author experienced a case of Hand-Schuller-Christian disease in six year old girl. She has been treated under the diagnosis of Calves disease. But later, it was confirmed as Hand-Schiiller-Christian disease by clinically and pathologically, involving multiple region of entire skeletal system, but sparing the musculocutaneous and nervous system. Clinically, anemia, malaise, high fever and hepatomegaly were present. Co60 isotope irradiation combined with antibiotics was done for 87 days twice weekly in a dose of 50 R at a time. At the time of discharge clinical symptoms were subsided. In X-Ray check, one year after treatment, bony defects were consolidated with new bone and the result was successful.
Anemia ; Anti-Bacterial Agents ; Diagnosis ; Eosinophilic Granuloma ; Female ; Fever ; Hepatomegaly ; Histiocytosis, Langerhans-Cell ; Humans ; Nervous System

Baboon syndrome is used to denote a systemic allergic contact dermititis with a characteristic color and distribution pattern. It is also referred to as mercury exanthem, because inhalation of mercury vapor usually causes the skiri lesion in a sensitized person. Diffuse pi,ikish erythema of the buttock, upper inner thigh, and axilla are characteristic features. We report a case of baboon syndrome developed after exposure to the smoke of a burning amulete a charm written on a piece of paper in a 12 year-old female. Mercury sensitivity was confirrned by a patch test. Atomic absorption by a spectrographic method and Scanning electron microscopy/energy dispersive for analysis X-ray detected mercury in the urine and the ed scraped pigments from the amulet, respectively.
Absorption ; Axilla ; Burns ; Buttocks ; Child ; Erythema ; Exanthema ; Female ; Humans ; Inhalation ; Papio* ; Patch Tests ; Smoke* ; Thigh

Romberg syndrome is a rare disease and characterized by unilaeril atrophy of the skin, subcutaneous tissue and the underlying structure of half the face. The lesion does not usually cross the midline of the scalp. Roriberg syndrome is sometimes mildsnosed as linear scleroderma, although they differ widely in their clinical and histological apperance. A 13-year-old boy was present with a one year history of idefined atrophic patch on the left side of face and neck. We involved skin was not bound down to the underlying structure. The history revealed no prcvious trauma or disease, He had no abnormal neurologic finding. Computerized tomography demonstrated a diminished subcutaneouat volume and also decreased thickness of back muscle is the left side face from cheek to neck.
Adolescent ; Atrophy ; Back Muscles ; Cheek ; Humans ; Male ; Neck ; Neurologic Manifestations ; Rare Diseases ; Scalp ; Scleroderma, Localized ; Skin ; Subcutaneous Tissue

No abstract available.
Embryonic Stem Cells*

BACKGROUND: In recent years, lymphocyte subset analysis in peripheral blood is widely performed using erythrocytes-lysed whole blood and two color immunofluorescence/flow cytometry method. Use of fresh blood drawn within 6 hours of staining is recommended, and some patients have to revisit the hospital for blood collection. We tested whether 24 hours-refrigerated/stored whose blood can be used for lymphocyte subset analysis. METHODS: Twenty consecutive blood samples from patients (including nine HIV positive patients) collected in EDTA-vacutainer were tested: 1) on the day of sampling using fresh blood kept at room temperature for up to 6 hours until staining (as recommended by the manufacturer) and 2) on the following day using the same tube of blood refrigerated for 24 hours after the first staining. Two colon immunofluorescenc staining was done using Simultest(TM) IMK-Lymphocyte kit (Beckon Dickinson, U.S.A.) and flow cytometric analysis was performed using FACScan and SimulSET(TM) software (Becton Dickinson, U.S.A.). Results of alive kinds of Lymphocyte subsets (CD3+, CDl9+, CD3+CD4+CD3+CD8+, CD3-CDl6+ and/or CD56+) on day 1 and day 2 were compared by pained-t test and Wilcoxon signed rank test. RESULTS: There was no significant change of values for all of the lymphocyte subsets except CD3+CD8+suppressor/cytotoxic (S/C) T cells. There was a slight but statistically significant change in S/C T cells (39.9%-->41.8%: 1.9%, p=0.008) after 24 hours of refrigeration, and this change was observed mainly in HIV-positive patient group. However, there was no significant change in the absolute count of helper/inducer T cells or CD4/CD8 ratio, and the change of S/C T cells in these patients was not considered to be of clinical significance. CONCLUSIONS: The difference in the values of lymphocyte subsets between fresh blood and 24 hours-refrigerated blood was negligible and it is concluded that 24 hours-stored blood samples can be used for lymphocyte subset analysis for clinical purposes.
Colon ; HIV ; Humans ; Lymphocyte Subsets* ; Lymphocytes* ; Refrigeration ; T-Lymphocytes

No abstract available.
Calcium* ; Lithium* ; Parathyroid Hormone*

Chondroblastoma is a rare primary bone tumor which originates from cartilage, and represents approximate 1% af all bone tumor. The chondroblastoma arises most frequently from the epiphysis of the long bones with the humerus being the commonest site. It afflicts usually the young under 25 years with greater incidence in male. As there is no cartilage cell on craniofacial bone which is mainly fromed by intramembranous ossification, the chondroblastoma on the craniofacial bone is extremely rare. But the chondroblastoma recurred frequently in craniofacial bone when the mass is excised incompletely or curretted and, as the tumor has the outstanding ability of local invasiveness, it destructs the adjacent bone. In addition, it is difficult to diagnose differentially from sarcoma or giant cell tumor histopathologically. Due to the entities described above, it is necessary to remove the entire tumor mass as complete as possible, to treat with radiation pre or postoperatively for preventing from recurrence, and to observe for a long time. The chondroblastoma on temporal bone is rare and is difficult to diagnose and treat successfully. So we'd like to present a case of chondroblastoma which was originated from temporal side of TMJ with literatural review.
Cartilage ; Chondroblastoma ; Epiphyses ; Giant Cell Tumors ; Humans ; Humerus ; Incidence ; Male ; Recurrence ; Sarcoma ; Temporal Bone ; Temporomandibular Joint*

A prognathic mandible and prominent gonial angle are considered to be unattractive in the Orient because it gives the face a square and muscular appearance. Requests for contouring of the mandibular angle are rare in the other race but are much more common in Korea and, through out the Orient. So, we often encounter a patient with a prognathic mandible and squarish or broad face who wishes to have his or her facial size reduced minimally or to acquire a round or slender appearance. But, mandibular angle is located in the deepest part of the operative field and the strong tension of the lateral soft tissue makes retraction difficult. It is extremely difficult to reduce the gonial angle with sagittal split ramus osteotomy by intraoral approach at the same time. We apply the method of gonial angle reduction during SSRO and had satisfactory results. We will present our results and hope to give some useful information for management of mandibular deformity.
Congenital Abnormalities ; Continental Population Groups ; Hope ; Humans ; Korea ; Mandible ; Osteotomy, Sagittal Split Ramus*

No abstract available.
Apgar Score* ; Female ; Fetal Heart* ; Heart Rate, Fetal* ; Humans ; Hydrogen-Ion Concentration* ; Infant, Newborn* ; Pregnancy