No abstract available.
Elbow Joint* ; Elbow*

Hypokalemic familial periodic paralysis is one of the rare familial disease characterized by recurrent and transient attacks of weakness or paralysis of the somatic musculature. Also, this disease is usually inherited as an autosomal dominant trait in most cases. During an attack, the plasma potassium falls as a rasults of shift of potassium from the extracellular to the intracelluar compartment, but there is no loss of total potassium from the body. We have experienced hypokalemic familial periodic paralysis recently which affected 4 members in a family,and report this disorder.
Accidental Falls ; Humans ; Paralyses, Familial Periodic ; Paralysis ; Plasma ; Potassium

Fibro-osseous pseudotumor of the digits is a heterotopic ossification closely related to myositis ossificans and occurs in the subcutaneous tissue of the digits. This lesion is considered a reactive fibroblastic proliferation with metaplastic bone formation. We report a case of fibro-osseous pseudotumor of left index finger in a 28-year-old woman. She had had an ovoid smooth subcutaneous mass with tenderness on the left index finger for one month. In gross, the specimen consisted of a relatively circumscribed, rubbery soft mass with grayish white cut surface measuring 2.0 1.7 1.5 cm. Upon microscopic examination the lesion showed irregular multinodular growth with considerably variable cellularity. Because of the focal hypercellularity, cellular atypia, and increased mitotic activity this lesion may be confused with extraskeletal osteosarcoma or parosteal osteosarcoma. This rare lesion is curable by complete local excision.
Adult ; Female ; Fibroblasts ; Fingers ; Humans ; Myositis Ossificans ; Ossification, Heterotopic ; Osteogenesis ; Osteosarcoma ; Subcutaneous Tissue

PURPOSE: Hypothalamic hamartomas are non-neoplastic malformations which are composed of hyperplastic neuronal tissue of varying size. Clinically, they are characterized by gelastic seizure, precocious puberty, and behavioral and psychiatric disorders. This study was performed to examine the various features of hypothalamic hamartomas such as neurologic manifestations, other clinical manifestations, and EEG and brain MRI findings. Response to AEDs or outcome of operation on hypothalamic hamartoma was evaluated. METHODS: Eleven patients who were admitted to Seoul National University Children's Hospital from July 1986 to January 1997 and diagnosed as hypothalamic hamartoma by brain MRI or CT were enrolled in this study. Clinical manifestations and EEG were reviewed retrospectively through the medical records. The size and type of the hypothalamic hamartoma on brain MRl were analyzed. RESULTS: 1) The range of age at visit was 1yr 9mo to 17yr 2mo (mean 10yr 8mo), and the age at onset was 1yr 1mo to 14yr to 2mo (mean 5yr 8mo). Six patients were male and five were female. 2) The clinical manifestations included gelastic seizure (n=9), behavioral and psychiatric disorders (n=9), other types of seizure (n=8), and precocious puberty (n=6). Other types of seizure were complex partial seizure (n=3), generalized tonic seizure(n=3), and infantile spasm (n=2). 3) The interictal EEG findings included focal spike discharges (n=8), diffuse delta slowings (n=1), hypsarrhythmia (n=1), and normal record (n=1). The focal spike discharges originated from the temporal (n=2), fronto-temporal (n=1), occipital (n=3), or frontal area(n=2). 4) The brain MRI showed that the size of the hypothalamic hamartomas was 2.47+/-1.12cm, and the origin of the tumors was tuber cinereum (n=6) or tuber cinereum and mamillary body (n=5). All lesions were isointense on T1-weighted image relative to normal gray matter, with a sessile attachment to the hypothalamus, and were not enhanced by Gadolinium. 5) Operations were done in 5 cases, those were subtotal removal of the tumor (n=4), and gamma knife radiosurgery (n=1). Seizures were not completely controlled in all but one case on which gamma knife radiosurgery was done. CONCLUSIONS: The hypothalamic hamartomas presented variable clinical pictures including gelastic seizure, precocious puberty, and behavioral and psychiatric disorders. The gelastic and other types of seizure associated with hypothalamic hamartomas were refractory to medication and might be controlled by total removal of the tumor.
Brain ; Electroencephalography ; Female ; Gadolinium ; Hamartoma* ; Humans ; Hypothalamus ; Infant ; Infant, Newborn ; Magnetic Resonance Imaging* ; Male ; Mamillary Bodies ; Medical Records ; Neurologic Manifestations ; Neurons ; Puberty, Precocious ; Radiosurgery ; Retrospective Studies ; Seizures ; Seoul ; Spasms, Infantile ; Tuber Cinereum

Many uncemented acetabular components use transfixation screws to stabilize the acetabular component until ingrowth of bone occurs. But the use of transacetabular screw fixation in total hip arthroplasty could increase the incidence of intrapelvic complications including neurovascular injury, extrinsic compression and erosion of intrapelvic organs. Metal debris was attributed to corrosion or fretting between screw and metal shell may lead to pelvic osteolysis. A foreign body reaction to metal and polyethylene wear debris in a loose acetahular component eroded the medial wall of the acetabulum and produced an intrapelvic mass. We experienced a case of intrapelvic pseudocyst in a failed total hip arthroplasty. The cyst communicated with hip joint through the transacetabular fixation screw which was placed near the center of the acetahulum.
Acetabulum ; Arthroplasty, Replacement, Hip* ; Corrosion ; Cytochrome P-450 CYP1A1 ; Foreign-Body Reaction ; Hip ; Hip Joint ; Incidence ; Osteolysis ; Polyethylene

Synthetic hydroxyapatite is a safe, nontoxic, biocompatible, and osteoconductive material. Hydroxyapatite-coated implants have the property of achieving a very strong bond with living bone in a relatively short period. From March 1992 to June 1994, 140 hydroxyapatite-coated primary total hip prostheses were implanted. This study included 60 cases that could follow up minimum 3 years. At the time of mean follow up, 45 months after operation, the mean Harris hip score was 92 points. Only two patients complained of thigh pain at last follow up. On radiographic evaluation, radiolucent lines were seen around the uncoated distal part of the stem in 46 percents of the implants. No radiolucent line was observed around the coated area of the prosthesis. Cancellous condensation was seen in 95 per cents of the implants at the Gruen zone 2 and 6 area. Cortical hypertrophy was present in 32 percents. Fourteen cases had the gaps at the bone to acetabular cup interface in the immediate postoperative period. The gaps disappeared in 12 months after operation. Five implants were revised because of polyethylene wear, cup migration and periprosthetic fracture. The clinical outcome was excellent. But this result was obtained in the relatively short-term follow up. Longer-term data will be the only true test of the value of hydroxyapatite coated implant.
Acetabulum ; Arthroplasty, Replacement, Hip ; Durapatite* ; Follow-Up Studies ; Hip Prosthesis* ; Hip* ; Humans ; Hypertrophy ; Periprosthetic Fractures ; Polyethylene ; Postoperative Period ; Prostheses and Implants ; Thigh

Polands syndrome is a congenital disorder associated with thoracic anomalies and ipsilateral syndactly. It was described first by Alfred Poland in 1841 year. We has been experienced 2 cases of Polands Syndrome recently. Two cases were male. Each case revealed one side thoracic muscle anomalies, that is, absence of sternocostal head of pectorails major with hypoplasia of nipple and, breast, and ipsilateral syndactyly with partial aplasia and hypoplasia of midphalanges.
Breast ; Congenital, Hereditary, and Neonatal Diseases and Abnormalities ; Head ; Humans ; Male ; Nipples ; Poland ; Syndactyly

Neurilemmoma is a benign neoplasm arising from the Schwann cell of the nerve sheath. Acoustic nerve is the most common site of neurilemmoma and mandible is the most frequently involved in intraosseous neurilemmoma. Extradurally located dumbbell type neurilemmoma was rarely reported. The histology of neurilemmoma was first described by Verocay in 1908 and elaborated upon by Stout in 1935. Characteristic histologic patterns of neurilemmoma were encapsulation and Verocay bodies, Antoni type A and B areas, palisading nuclei, and lack of malignant characteristics. We experienced such a rare case of neurilemmoma which probably arised from the left first lumbar spinal nerve root, and located extradurally and paravertebrally, and confirmed by radiologic and pathologic findings.
Cochlear Nerve ; Mandible ; Neurilemmoma ; Spinal Nerve Roots

Maspin is a recently described gene with tumor suppressor activity. The gene product is a 42 kD protein with homology to the serpin family of protease inhibitors and may play a role as an inhibitor of tumor cell invasion. The prior observation that invasive breast cancers and their metastases showed decreased maspin protein expression by immunostaining supports this speculation. However, the role of maspin in breast cancer progression has not been studied in detail. We, therefore, studied maspin protein expression in a series of hyperplasia, atypical ductal hyperplasia, intraductal carcinoma and invasive carcinomas. Immunohistochemical staining (IHC) for maspin was performed on paraffin sections of 136 breast specimens using a commercially available monoclonal antibody. Among the 106 cases studied were 36 moderate/florid ductal hyperplasia, 11 atypical ductal hyperplasia (ADH), 29 intraductal carcinoma (IDC) (4 low grade, 13 intermediate grade, 12 high grade) and 30 invasive ductal carcinomas. Thirty cases of normal breast were also studied as control group. IHC stains were scored using a semiquantitative scoring system. The mean IHC scores for maspin for normal, moderate/florid hyperplasia, atypical ductal hyperplasia, intraductal carcinoma, and invasive carcinoma were 5.51 1.30, 7.36 0.72, 3.82 1.60, 4.48 2.69, 3.97 3.30, respectively. These scores for each category were statistically significant (p<0.05), except between ADH and IDC. Maspin protein expression was increased in most cases of moderate/florid hyperplasia, while maspin expression was more heterogeneous in ADH and IDC. In high grade IDC, maspin protein expression was stronger than low and intermediate grade IDC, and this suggests the possibility of a compensatory cellular response against the forces driving further tumor progression. Two thirds of invasive ductal carcinomas expressed maspin protein weakly and focally. All metastatic carcinomas of lymph nodes were negative for maspin. It is possible that high grade IDC with strong maspin expression may represent a subset less likely to progress to invasive cancer. This speculation merits investigation in clinical outcome studies.
Breast Neoplasms ; Breast* ; Carcinoma, Ductal* ; Carcinoma, Intraductal, Noninfiltrating* ; Coloring Agents ; Humans ; Hyperplasia* ; Lymph Nodes ; Neoplasm Metastasis ; Outcome Assessment (Health Care) ; Paraffin ; Protease Inhibitors

Actinomycosis causes a chronic suppurative infection most commonly involving the cervico-facial region, thorax, and the abdomen. Thoracic infection results from an aspiration of contaminated material from mouth or oropharynx. Actinomyces was known to have a predilection for the periphery of the lung and the endobronchial lesion is extremely rare. We report two cases of actinomycosis presenting as an endobronchial mass which arose in 53-year-old and 44-year-old women. The first case presented with cough, sputum, weight loss and had a history of pulmonary tuberculosis 24 years ago. Chest CT revealed a right lobe collapse simulating mass and suggested carcinoma. The second case presented with hemoptysis for 2 years. Chest CT revealed cystic bronchiectasis of both lungs and intracystic soft tissue mass in the anterior segment of the right upper lobe which suggested aspergilloma. The gross features of them were similar to those of aspergilloma. Characteristic sulphur granules consisting of a granular basophilic center surrounded by a radiating zone of eosinophilic, hyaline, club-shaped projection were histologically confirmed in both cases. Granulomas containing P. westermani eggs were present in the second case. Staphylococcus, true fungal organism, nocardia, and streptomyces shoud be distinguished by analysing their morphologic characteristics in the appropriate stains. Actinomycosis should be included in the differential diagnoses of an endobronchial mass.
Abdomen ; Actinomyces ; Actinomycosis* ; Adult ; Basophils ; Bronchiectasis ; Coloring Agents ; Cough ; Diagnosis, Differential ; Eggs ; Eosinophils ; Female ; Granuloma ; Hemoptysis ; Humans ; Hyalin ; Lung ; Middle Aged ; Mouth ; Nocardia ; Oropharynx ; Ovum ; Sputum ; Staphylococcus ; Streptomyces ; Thorax ; Tomography, X-Ray Computed ; Tuberculosis, Pulmonary ; Weight Loss