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

The Ilizarov technique has been used successfully in the treatment of nonunion of long bone accompanied by infection, severe bone and soft tissue defects, and deformity. We report the results of treatment of 21 cases of nonunion using the Ilizarov technique. There were 20 males and 1 female with an average age of 34.2 years (range, 8-72 years). The average follow up period was 45 months (range, 12-74months). Infected nonunion(15/21 cases) was the most common cause of nonunion. The treatment methods include simple compression method(4 cases) and internal and external bone transport method(17 cases). Bony union was achieved in 20 cases out of 21 cases. One case is still having difficulty in achieving union. The mean time to union was 7.3 months in femurs and 6.3 months in tibias. The amount of bone defect that developed after the removal of infected bone fragments and soft tissue averaged 3.8cm (range, 2.3-9cm). The healing index, in cases of bone transport, was an average of 45 days per cm (range, 30-62 days per cm). Complications developed in 17 cases out of 21 cases. Pin site infection occurred in 5 cases, premature consolidation in ~1 case, angulation deformity in 2 cases, LOM of the knee in 2 cases, equinus deformity in 2 cases, ankle stiffness in 3 cases, and nonunion in 1 case. The Ilizarov technique is thought to be effective in the treatment of complicated nonunion combined with shortening, deformity, bone defect and infection. However, in order to reduce complications caused by the Ilizarov method, accurate surgical techniques and postoperative care are necessary.
Ankle ; Congenital Abnormalities ; Equinus Deformity ; External Fixators* ; Female ; Femur ; Follow-Up Studies ; Humans ; Ilizarov Technique ; Knee ; Male ; Postoperative Care ; Tibia

Hip arthrograghy is a valuable procedure for visualization of the intrinsic obstructive factors impeding closed reduction and for critical assessment of quality of reduction in developmental dislocation of the hip. The authors have analyzed 25 cases of developmentally dislocated hips in 24 patients who have been followed up for more than 1 year postoperatively by roentgenographies and arthrographies. Among them 16 cases in 16 patients who had been treated by closed reduction according to acceptable quality of initial reduction were studied to estimate a value of arthrography and to evaluate an outcome of closed rediction performed on the base of an arthrographic finding. The following results were obtained. 1. The frequency of the common arthrographic observations were assessed. Medial dye columns identifying the depth of reduction were observed 25 cases(100%), configuration of osteocartilaginous structures in 24 cases(96.0%), limbus in 24 cases(96.0%), and ligamentum teres in 18cases(75.0%). 2. Good closed reduction classified according to Race and Herring on initial arthrography was associated with rapid improvement of acetabular angle and the CE angle and low incidence of avascular necrosis, compared with adequate or poor reduction. 3. In cases of initial good and adequate reductions, initial AP arthrograms showed some dye filling shadows owing to folding or redundancy of lax joint capsule of the dislocated hip after reduction just lateral to the limbus, of which finding decreased markedly on the second arthrograms. 4. The widths of medial dye column decreased with time during the first postoperative 7 weeks by an average of 0.5mm in cases with initial good quality of reduction, 3.5mm in adequate reductions and 0.5mm in poor reductions. 5. In cases with adequate closed reduction on initial arthrogram, but without substantial decrease in width of medial dye column on the second arthogram the outcome was poor. In those cases with decrease in width of medial dye column on the second arthrogram compared with initial arthogram, the shorter the period of immobilization in a plaster cast was, the worse eventual result was. 6. We think that hip arthography is a helpful procedure for visualization of obstacle obstructing closed reduction and for determination of treatment modality in developmental dislocation of the hip.
Acetabulum ; Arthrography ; Casts, Surgical ; Continental Population Groups ; Dislocations ; Hip ; Humans ; Immobilization ; Incidence ; Joint Capsule ; Necrosis ; Round Ligaments

No abstract available.
Bone Cysts* ; Fractures, Spontaneous*

The purpose of this study is to compare 76 cases of fractures of the proximal humerus both clinically and radiologically which were treated at Pusan National University Hospital from January 1987 to December 1996. According to Neers classification, among the 76 patients, there were nine one-part fractures (11.8%), forty two-part fractures (52.6%), eighteen three-part fractures (23.7%), four four-part fractures (5.3%), and five head-splitting fractures (6.6%). In terms of treatment method, among the 76 patients, 49 patients (64.5%) were treated conservatively. Thirty seven patients (75.5%) of one-part and two-part fractures were treated conservatively, and twelve patients (54.5%) of three-part and four-part fractures were treated operatively. The results of the cases were rated by the Neer s assessment, 42 patients (85.7%) had excellent or satisfactory results in one-part and two-part fracture, and 7 patients (31.8%) had unsatisfactory or failed results in three-part and four-part fracture. Total cases with complications were 14 cases (18.4%). From the viewpoint of the type of fracture, the results in two-part and three-part fractures were much better than those of fourpart fractures. The joint stiffness which was the most common complication must be prevented by allowing the early motion of the joint to obtain a better result.
Busan ; Classification ; Humans ; Humerus* ; Joints

Pseudomyxoma peritonei may result from implantation of benign or malignant tumor in peritoneal cavity and is filled with gelatinous material (termed "Jelly Belly") in abdominal cavity. Its origin is usually an appendiceal or ovarian mucinous adenoma or cystadenocarcinoma, but other primary origin such as uterus, intestine, pancreas and stomach umor have been reported. Generally, pseudomyxoma peritonei is slowly progressive and has low grade malignant potential. This report presents a unusual long term survival after evacuation of 15,000 cc of gelatinous material from abdominal cavity which was the low grade mucinous adenocarcinoma and a review of the current literature, management and new its concept. The origin of pseudomyxoma peritonei of this case was most likely from appendiceal mvcinous adenocarcinoma.
Abdominal Cavity ; Adenocarcinoma ; Adenocarcinoma, Mucinous ; Adenoma ; Cystadenocarcinoma ; Gelatin ; Intestines ; Mucins ; Pancreas ; Peritoneal Cavity ; Pseudomyxoma Peritonei* ; Stomach ; Uterus

Pulmonary function tests were conducted in 60 male adults and 60 female adults at 20~79 years of age. They showed noevidenee of pulmonary disease on clinical symptoms, X-ray finidngs and past history. We wanted to evaluate the deterioration of pulmonary functional reserve in the aged. The results were as follows: 1) In males, FVC decreased significantly (p <0.01) from 4440+/-271.38 ml in 20~29 year-old subjects to 3090+/-445.75 ml 70~79 year-old subjects. The correlation coefficient with age was-0.6846. 2) In females, FVC decreased significantly (p<0.01) from 2097+/-479.42ml in 20~29 year-old subjects to 2142+/-233.13ml in 70~79 year-old subjects The correlation coefficient woth age was 0.6454. 3) In males, FEV, decreased significantly (p<0.01) from 4005+/-268.26 ml in 20~29 year-old subjects to 2373+/-326.36ml in 10~79 year-old subjects. The correlation coefficient with age was-0.8229. 4) In females, FEV1 decreased significantly (p<0.01) from 2586+/-519.00 ml in 20~29 yrar-old subjects to 1645+/-330.36ml in 70~79 year old subjects The correlation coefficient with age was 0.7013. 5) In males, FEV1/FVC decreased significantly (p<0.01) from 90.17+/-4.90% in 20~29 year-old subjects to 76.72+/-8.13% in 70~79 year-old subjects. The correlation coefficient with age was-0.5595. 6) In females, FEV1/FVC decreased significantly (p<0.01) from 88.66+/-6.58% in 20-29 year-old subjects to 76.80+/-9.32% in 70~79 year-old subjects. The correlation coefficient with age wae-0.4489.
Adult* ; Aging* ; Female ; Humans ; Lung Diseases ; Male ; Respiratory Function Tests

No abstract available.
Child* ; Humans ; Humerus*

Severe fever with thrombocytopenia syndrome (SFTS) is an emerging infectious disease in South Korea with clinical features similar to those of scrub typhus. Infected patients with these two diseases usually visited the emergency department (ED) complaining of fever that was not responsive to treatment in a local clinic. Aggressive management of SFTS is required in order to prevent rapid progression and human-to-human transmission, thus rapid and accurate differential diagnosis of the two diseases in the ED is important. We reported three laboratory confirmed cases of SFTS during 2013-2014 in a single center, with fever, skin lesions, and history of outdoor activities in order to help in differential diagnosis between SFTS and scrub typhus in the ED.
Communicable Diseases, Emerging ; Diagnosis, Differential ; Emergency Service, Hospital ; Fever* ; Humans ; Korea ; Scrub Typhus* ; Skin ; Thrombocytopenia*

BACKGROUND: Epithelial-mesenchymal transition (EMT) is an important step in the invasion and progression of cancer and in the development of chemoresistance by cancer cells. METHODS: To address the clinical significance of the EMT pathway in lung adenocarcinoma and the association of the pathway with histological subtype, we examined 193 surgically resected lung adenocarcinoma samples for the expression of representative EMT-related proteins (E-cadherin, beta-catenin, and vimentin) by immunohistochemistry. Histological subtypes were classified according to the 2011 International Association for the Study of Lung Cancer/American Thoracic Society/European Respiratory Society classification. The results for EMT-related protein expression were analyzed for correlation with clinicopathological features and with survival. RESULTS: The loss of E-cadherin expression and aberrant beta-catenin expression were significantly associated with larger tumor size, pleural invasion, lymphatic/vascular invasion, and advanced pathological stage (p<0.05). The alteration of the E-cadherin/beta-catenin complex was least frequently observed in the lepidic-predominant group, but these associations were not statistically significant. In the multivariate analysis, altered E-cadherin/beta-catenin complex expression was found to be an independent poor prognostic factor (p=0.017; hazard ratio, 1.926; 95% confidence interval, 1.119 to 3.314). CONCLUSIONS: The alteration of the expression of the E-cadherin/beta-catenin complex was associated with aggressive tumor behavior in lung adenocarcinoma.
Adenocarcinoma ; beta Catenin ; Cadherins ; Epithelial-Mesenchymal Transition ; Immunohistochemistry ; Lung ; Lung Neoplasms ; Multivariate Analysis ; Proteins