A nineteen year-old boy with progressive enlargement of the joints and distal extremities, clubbing, coarse facial features and hyperhidrosis was investigated. In physical examination, thickening of the scalp with furrowing (cutis verticis gyrata) and greasy thickening of skin (pachyderma) was prominent. His endocrine profile was normal. Radiological studies demonstrated bilateral symmetrical periosteal new bone formation with acroosteolysis and incidental microadenoma of pituitay gland. After extensive investigation to exclude systemic and endocrine causes, the patient was diagnosed as pachydermoperiostosis (PDP). PDP is a rare syndrome manifested clinically by finger clubbing, extremity enlargement, hypertrophic skin changes, and periosteal bone formation. The pathogenesis of the disorder has not been clarified though few endocrine abnormalities were seen. To aware of these clinical phenotype would help to differentiate PDP from acromegaly.
Acro-Osteolysis ; Acromegaly ; Extremities ; Fingers ; Humans ; Hyperhidrosis ; Joints ; Male* ; Osteoarthropathy, Primary Hypertrophic* ; Osteogenesis ; Phenotype ; Physical Examination ; Scalp ; Skin ; Young Adult*

To analyzed the progression of white matter maturation and white matter pathology, MR imaging of the brain was obtained in 38 children with delayed development. Children with developmental delay showed a high incidence of MR abnormalities(34/38, 89.5%). Delayed pattern of myelination and gray-white matter differentiation was seen in 13 patients. Twenty-two patients had white matter patholgy, including 14 with white matter hypoplasia, seven with focal small infarction, five with periventricular leukomalacia, and three with high signal intensities on T2 weighted image. Associated structural abnormalities were also evaluated. The most common lesions in decreasing frequency were cerebral atrophy and dysgenesis of the corpus callosum, pachygyria and/or polymicrogyria, porencephalic cyst and Leigh's disease. Twenty-three of 34 children had multiple abnormalities on MRI. The MRI was useful in depicting the progression of myelination and other white matter lesions, and serial follow-up MR is recommended for patients with delayed or lack of myelination and gray-white matter differentiation.
Abnormalities, Multiple ; Atrophy ; Brain* ; Child* ; Corpus Callosum ; Follow-Up Studies ; Humans ; Incidence ; Infant, Newborn ; Infarction ; Leigh Disease ; Leukomalacia, Periventricular ; Lissencephaly ; Magnetic Resonance Imaging* ; Myelin Sheath ; Pathology ; Polymicrogyria ; White Matter*

We reviewed MR angiograms to evaluate its efficacy for visualizing the renal arteries and detecting renovascular disease. 41 renal arteries in 19 patients were examined by MR angiography. 3-D time-of-flight technique was used as routine examination method for MR angiography and 2-D time-of-flight technique was added in some particular cases to visualize venous flow. Within two weeks after MR angiography was performed, 23 renal arteries in 10 patients were additionally examined by conventional angiography or intraarterial DSA. The success rates of vessel visualization on MR angiography in normal renal arteries were 100% in main 67.7% in segmental, and 11.8% in intrarenal arteries. As a result of comparative study in normal main renal arteries with MR angiograms and conventional angiograms, overall correspondence in the number and the shape was noted and the caliber discrepancy between two examination did not exceed 3.0 mm. one arteriovenous fistula with aneurysm, one stenotic artery and two occluded arteries were well evaluated One arteriovenous fistula with aneurysm, and two occluded arteries were well evaluated by MR angiography. However three stenotic lesions were misdiagnosed as occlusions on MR angiography. and the overall accuracy was 87%. We conclude that MR angiography has the potential to be a noninvasive and useful screening method for determining the number of renal arteries and for detection of abnormalities of main renal arteries.
Aneurysm ; Angiography* ; Arteries ; Arteriovenous Fistula ; Humans ; Mass Screening ; Methods ; Renal Artery*

BACKGROUND: Accuracy of laboratory test results is an important issue. New guidelines for specimen delivery systems are needed for appropriate pretreatment of specimens and accuracy of results. METHODS: We evaluated various laboratory profiles, comparing the effects of specimen rack holders and coolants within transport containers. The hematology profiles (complete blood cell count [CBC], erythrocyte sedimentation rate [ESR]), chemistry profiles (aspartate aminotransaminase [AST], alanine aminotransaminase [ALT], gamma-glutamyl transferase [gamma-GT], electrolytes [Na, K, Cl], glucose, lactate dehydrogenase [LD], creatinine kinase [CK]), and coagulation profiles (prothrombin time [PT], activated partial thromboplastin time [aPTT], fibrinogen level). We also investigated the effects of transportation time including the presence or absence of hemolyzation. We received from 9 different university hospital laboratories using conventional transporation methods. RESULTS: Hemolytic features were seen in short drawn specimens. Fewer result variations were observed in specimens transported with coolants. Average specimen transportation time was 11.3 hours, and average temperatures of container was 10.9degrees C with coolant and 25.0degrees C without coolants. Non-centrifuged specimens transported with coolants showed increased serum K levels than centrifuged specimens. Coagulation tests showed less than a 10% differences. Centrifuged specimen prior to transportation showed no hemolyzation and no differences in results. CONCLUSIONS: Appropriate temperatures for each analyte should be defined to ensure the accuracy of results. To reduce hemolyzation, appropriate temperature and rack holder should be used. Temperature of the transport container should be monitored in objectively. Coagulation tests should be added as referral tests, if appropriate specimen transport monitoring system for time and temperature could be adopted.
Alanine ; Blood Cell Count ; Blood Sedimentation ; Creatinine ; Dietary Sucrose ; Electrolytes ; Fibrinogen ; Glucose ; Hematology ; Hemolysis ; L-Lactate Dehydrogenase ; Laboratories, Hospital ; Partial Thromboplastin Time ; Phosphotransferases ; Referral and Consultation ; Transferases ; Transportation

PURPOSE: This study was conducted to evaluate success and perforation rates of pneumatic reduction and to find the predictors of reduction failure. METHODS AND MATERIALS: We reviewed 224 cases of pediatric intussusception diagnosed by air-enema between July, 1989 and June, 1992. The mean age was 9.8 months(range, 1 month to 3 years). Logistic regression analysis was used to find predictors of reduction failure. RESULTS: Successful reduction was achieved in 197 cases(87.9% of success rate). Bowel perforation occurred in two cases(0.9% of perforation rate). There were two statistically significant predictors of failure;(1) ileoileocolic intussusception(p<.001), (2) long duration of symptom(p<.001). Surgery was performed in 26 cases (11.6%), of which seven required bowel resection. CONCLUSION: Pneumatic reduction is a useful means in the management of pediatric intussusception with a high success rate and no mortality.
Child* ; Humans ; Insufflation* ; Intussusception* ; Logistic Models ; Mortality

PURPOSE:We report the results of image-guided percutaneous biopsies with a biopsy gun and evaluate the clinical usefullness. MATERIALS AND METHODS: One hundred and five biopsies under ultrasonographic or fiuoroscopic guidance were performed. Various anatomic sites were targeted (liver;50, chest;22, kidney;12, pancreas;8, intraperitoneum;7, retroperitoneum; ). RESULTS:Obtained tissue was diagnostic in 98 of the 105 biopsies(93%). In each instance, representative core tissue specimens were obtained. Evaluation of the core tissue by pathologist revealed consistent, uniform specimens that contained significant crush artifact in no case. Five biopsies yielded inadeguate tissues which were too small for histopathologic interpretation or were composed of necrotic debris. Two biopsies yielded adequate tissues, but tissues were not of the target. The diagnoses were malignancy in 77 biopsies and benign disease in 21 biopsies. No complications other than mild, localized discomfort were encountered except a transient hemoptysis and pneumothorax which was observed in two patients. CONCLUSION:Cutting biopsy with a biopsy gun provided sufficient amount of target tissue for an accurate diagnosis of malignant and benign disease. It was a safe and useful procedure for percutaneous biopsy.
Artifacts ; Biopsy* ; Diagnosis ; Hemoptysis ; Humans ; Pneumothorax

PURPOSE: Weight loss reduces cardiovascular risk factors in the obese. However, weight reduction through diet negatively affects long-term bone health. The aim of study was to determine the ability of combined aerobic and resistance exercise (CE) to reduce weight and cardiovascular risk without diminishing bone health. METHODS: Twenty-five young adults participated in an 8-week weight loss CE program. Subjects were allocated to an obese group or a control group by body mass index (BMI). Body weight, BMI, body composition, and bone mineral density (BMD) of the lumbar spine and total hip were measured before and after the CE trial. Serum levels of metabolic markers, including adipokines and bone markers, were also evaluated. RESULTS: Weight loss was evident in the obese group after the 8 weeks CE trial. Fat mass was significantly reduced in both groups. Fasting insulin, homeostatic model assessment-insulin resistance (HOMA-IR), leptin and aminotransferases level were significantly reduced from baseline only in the obese group. High density lipoprotein cholesterol increased in both groups. Hip BMD increased in the obese group. In all study subjects, BMI changes were correlated with HOMA-IR, leptin, and HDL changes. BMI decreases were correlated with lumbar spine BMD increases, lumbar spine BMD increases were positively correlated with osteocalcin changes, and lumbar spine bone mineral content increases were correlated negatively with C-terminal telopeptide of type 1 collagen changes. CONCLUSION: These findings suggest that CE provides effective weight loss and improves cardiovascular risk factors without diminishing BMD. Furthermore, they indicate that lumbar spine BMD might be maintained by increasing bone formation and decreasing bone resorption.
Adipokines ; Body Composition ; Body Mass Index ; Body Weight ; Bone Density ; Bone Resorption ; Cholesterol ; Cholesterol, HDL ; Collagen Type I ; Diet ; Fasting ; Hip ; Humans ; Insulin ; Leptin ; Lipoproteins ; Obesity ; Osteocalcin ; Osteogenesis ; Risk Factors ; Spine ; Transaminases ; Weight Loss ; Young Adult

PURPOSE: This study was tried to evaluate the potential benefits of concurrent chemoradiation therapy (low dose daily cisplatin combined with split course radiation therapy) compared with conventional radiation therapy alone in stage III non-small cell lung cancer. The end points of analyses were response rate, overall survival, survival without locoregional failure, survival without distant metastasis, prognostic factors affecting survival and treatment related toxicities. MATERIAL AND METHODS: Between April 1992 and March 1994, 32 patients who had stage III non-small cell lung cancer were treated with concurrent chemoradiation therapy. Radiation therapy for 2 weeks (300cGy given 10 times up to 3000cGy) followed by a 3 weeks rest period and then radiation therapy for 2 more weeks (250cGy given 10 times up to 2500cGy) was combined with 6mg/M2 of cisplatin. Follow-up period ranged from 13 months to 48 months with median of 24 months. Historical control group consisted of 32 patients who had stage III non-small cell lung cancer were received conventionally fractionated (daily 170-200cGy) radiation therapy alone. Total radiation dose ranged from 5580cGy to 7000cGy with median of 5940 cGy. Follow-up period ranged from 36 months to 105 months with median of 62 months. RESULTS: Complete reponse rate was higher in chemoradiation therapy (CRT) group than radiation therapy (RT) group (18.8% vs. 6.3%). CRT group showed lower in-field failure rate compared with RT group (25% vs. 47%). The overall survival rate had no significant differences in between CRT group and RT group (17.5% vs. 9.4% at 2 years). The survival without locoregional failure (16.5% vs. 5.3% at 2 years) and survival without distant metastasis (17% vs. 4.6% at 2 years) also had no significant differences. In subgroup analyses for patients with good performance status (Karnofsky performance scale 80), CRT group showed significantly higher overall survival rate compared with RT group (62.5% vs. 15.6% at 2 years). The prognostic factors affecting survival rate were performance status and pathologic subtype (squamous cell cancer vs. nonsquamous cell cancer) in CRT group. In RT alone group, performance status and stage (IIIa vs IIIb) were identified as a prognostic factors. RTOG/EORTC grade 2-3 nausea and vomiting (22% vs. 6%) and bone marrow toxicities (25% vs. 15.6%) were significantly higher in CRT group compared with RT alone group. The incidence of RTOG/EORTC grade 3-4 pulmonary toxicity had no significant differences in between CRT group and RT group (16% vs. 6%). The incidence of WHO grade 3-4 pulmonary fibrosis also had no significant differences in both group (38% vs. 25%). In analyses for relationship of field size and pulmonary toxicity, the patients who treated with field size beyond 200cm2 had significantly higher rates of pulmonary toxicities. CONCLUSION: The CRT group showed significantly higher local control rate than RT group. There were no significant differences of survival rate in between two groups. The subgroup of patients who had good performance status showed higher overall survival rate in CRT group than RT group. In spite of higher incidence of acute toxicities with concurrent chemoradiation therapy, the survival gain in subgroup of patients with good performance status were encouraging. CRT group showed higher rate of early death within 1 year, higher 2 year survival rate compared with RT group. Therefore, to evaluate the accurate effect on survival of concurrent chemoradiation therapy, systematic follow-up for long term survivors are needed.
Bone Marrow ; Carcinoma, Non-Small-Cell Lung* ; Cisplatin ; Follow-Up Studies ; Humans ; Incidence ; Nausea ; Neoplasm Metastasis ; Pulmonary Fibrosis ; Survival Rate ; Survivors ; Vomiting

Hepatic sinusoidal obstruction syndrome (SOS) is a life-threatening syndrome that generally occurs as a complication after hematopoietic stem cell transplantation or, less commonly, after conventional chemotherapy. Regarding SOS in rhabdomyosarcoma patients who received conventional chemotherapy, the doses of chemotherapeutic agents are associated with the development of SOS. Several cases of SOS in rhabdomyosarcoma patients after receiving chemotherapy with escalated doses of cyclophosphamide have been reported. Here, we report on a 9-year-old female with rhabdomyosarcoma who developed severe SOS after receiving chemotherapy consisting of vincristine, actinomycin-D, and a moderate dose of cyclophosphamide. She was treated successfully with defibrotide without sequelae to the liver.
Child* ; Cyclophosphamide* ; Drug Therapy ; Female ; Hematopoietic Stem Cell Transplantation ; Hepatic Veno-Occlusive Disease* ; Humans ; Liver ; Rhabdomyosarcoma* ; Vincristine*

Purpose: To evaluate the clinical and radiological results of hip arthroplasty using a cementless modular femoral stem in patients older than 70 years with a femoral neck fracture. Materials and Methods: From January 2002 to May 2005, 67 hip arthroplasty procedures (66 patients) using a cementless modular femoral stem for displaced femoral neck fractures in patients older than 70 years of age were evaluated. All the patients were followed up for more than 2 years. The mean age at surgery was 77 (70~92) years. There were 22 men and 44 women. The mean follow up period was 31 months (24~41). The clinical evaluation was performed by examining the perioperative Activities of Daily Living (ADL) and postoperative complications. The radiological evaluation for femoral stem loosening and osteolysis was performed using the serial postoperative radiographs. The radiological evaluation for leg length discrepancies was performed using the immediate postoperative radiograph. Results: In preoperative ADL, 31 cases (46%) were in grade 1, 21 (31.5%) in grade 2, 14 (21%) in grade 3, 1 (1.5%) in grade 4 and none in grade 5. A review of the postoperative ADL revealed 25 cases (37.5%) in grade 1, 18 (27%) in grade 2, 21 (31%) in grade 3, 3(4.5%) in grade 4 and none in grade 5. Postoperative restoration of the ADL was observed in 48 cases (71.5%). There were no significant complications. Radiographically, all cases showed stable bony fixation of the femoral stem with the exception of 1 case, who showed subsidence and a pedestal reaction. Postoperative leg length discrepancy was observed in 5 cases (7.5%) but was <1 cm in all cases. Conclusion: In elderly patients older than 70 years of age with a displaced femoral neck fracture, cementless hip arthroplasty using a modular femoral stem provides good initial stability and subsequent secure bony fixation with minimal complications.
Activities of Daily Living ; Aged ; Arthroplasty ; Female ; Femoral Neck Fractures ; Femur Neck ; Follow-Up Studies ; Hip ; Humans ; Leg ; Male ; Osteolysis ; Postoperative Complications