Familial erythrophagocytic lymphohistiocytosis is a fatal early childhood disorder characterized by multiorgan lymphohistiocytic infiltration and active hemophagocytosis. Involvement of the central nervous system (CNS) is not uncommon and is characterized by rapidly progressive tissue damage affecting both the gray and white matter. We encountered a case of familial erythrophagocytic lymphohistiocytosis with CNS involvement. Initial T2-weighted MRI of the brain demonstrated high signal intensity in the right thalamus, though after chemotherapy, which led to the relief of neurologic symptoms, this disappeared. After four months, however, the patient's neurologic symptoms recurred, and follow-up T2-weighted MR images showed high signal intensity in the thalami, basal ganglia, and cerebral and cerebellar white matter. Brain MRI is a useful imaging modality for the evaluation of CNS involvement and monitoring the response to treatment.
Basal Ganglia ; Brain* ; Central Nervous System ; Drug Therapy ; Follow-Up Studies ; Lymphohistiocytosis, Hemophagocytic* ; Magnetic Resonance Imaging* ; Neurologic Manifestations ; Thalamus

The present study compared the actigraphic indices between both wrist actigraphies (WATGs), and the sleep estimates between each WATG and nocturnal polysomnography (NPSG) to assess their differences and consistencies. We studied 22 right-handed subjects (mean age 43.9+/-13.3 years, M:F=14:8) with untreated primary sleep disorders (primary insomnia=8, simple snorer=2, obstructive sleep apnea=12) undergone by overnight both WATGs and NPSG, simultaneously. Comparison and correlation were analyzed between right and left wrist actigraphic data. In the sleep estimates of both WATGs and NPSG, each WATG was compared and correlated with NPSG in sleep period time (SPT), total sleep time (TST), sleep latency (SL), sleep efficiency (SE) and wake time (WT). Sleep indices between both WATGs showed significant positive correlations with no correlations in SL and fragmentation index (FI). There were no differences in sleep indices between both WATGs. SPTs of both WATGs, SL of left WATG, and TST of right WATG showed positively significant correlations, and SE of right WATG did negatively significant correlation in sleep indices between each WATG and NPSG. As each WATG was compared to PSG, SPTs of both WATGs and WT of right WATG were decreased, and TST and SE of right WATG and SL of left WATG were increased. Inconsistent SL and FI between both WATGs indicate that the activities between both WATGs can differentially happen during wake or arousal. Inconsistent sleep estimates between each WATG and NPSG may indicate the limited usefulness in measuring and analyzing one-night sleep by using WATG.
Arousal ; Functional Laterality ; Polysomnography* ; Sleep Wake Disorders ; Wrist*

BACKGROUND: To evaluate the effectiveness of minimally invasive surgery total hip arthroplasty (THA) using the two-incision technique as described by Mears. METHODS: From January 2003 to December 2006, sixty-four patients underwent total hip arthroplasty using the one-incision (group I) and two-incision (group II) technique by one surgeon. There were 34 hips in group I and 30 hips in group II. There was no difference in age, gender, and causes of THA between the two groups. We evaluated the operation time, bleeding amount, incision length, ambulation, hospital stay, and complications between the two groups. RESULTS: There was no difference in the bleeding amount between the two groups. Operation time was longer in the two-incision group than in the one-incision group. Operation time of the two-incision technique could be reduced after 15 cases. Patients started ambulation after surgery earlier in group II than group I, and the hospital stay was shorter in group II than in group I. There was no difference in clinical results between the two groups. There was no difference in component position of the acetabular cup and femoral stem between the two groups. Intraoperative periprosthetic fracture occurred in four cases (13.3%) in group II. CONCLUSIONS: Two-incision THA has the advantage of rapid recovery and shorter hospital stay. However, longer operation time and a high complication rate compared to one-incision are problems that need to be solved in the two-incision technique.
Arthroplasty, Replacement, Hip/*methods ; Female ; Humans ; Male ; Middle Aged ; Retrospective Studies ; Surgical Procedures, Minimally Invasive/*methods

Purpose: This study aimed to apply doxorubicin-loaded nanoparticle microbubble (Dox-NP-MB) therapy in an orthotopic rat model of hepatocellular carcinoma (HCC) and investigate the utility of contrast-enhanced ultrasound (CEUS) and intravoxel incoherent motion diffusion-weighted magnetic resonance imaging (IVIM-DWI) for response evaluation. Methods: Twenty-eight N1S1 HCC model rats were treated with either Dox-NP-MB (group [G] 1, n=8), doxorubicin (Dox) alone (G2, n=7), nanoparticle microbubbles alone (G3, n=7), or saline (G4, control, n=6) on days 0 and 7, and were sacrificed on day 11. IVIM-DWI and CEUS were performed before each treatment and before euthanasia. Efficacy was estimated by the percentage of tumor volume growth inhibition compared with control. Toxicity was assessed by body weight changes and blood tests. Post-treatment changes in IVIM-DWI and CEUS parameters were analyzed. Results: Tumor volume growth was inhibited by 48.4% and 90.2% in G1 and G2 compared to G4, respectively. Compared to G2, G1 had a significantly lower degree of body weight change (median, 91.0% [interquartile range, 88.5%-97.0%] vs. 88.0% [82.5%-88.8%], P<0.05) and leukopenia (1.75×103 cells/μL [1.53-2.77] vs. 1.20×103 cells/μL [0.89-1.51], P<0.05). After the first treatment, an increase in peak enhancement, wash-in rate, and wash-in perfusion index on CEUS was observed in G3 and G4 but suppressed in G1 and G2; the apparent diffusion coefficients, true diffusion coefficients, and perfusion fractions significantly increased in G1 and G2 compared to baseline (P<0.05). Conclusion Dox-NP-MB showed reduced Dox toxicity. Early changes in some CEUS and IVIM-DWI parameters correlated with the therapeutic response.

PURPOSE: To evaluate the positive effects and problems through clinical and radiological results of cementless bipolar hemiarthroplasty for intertrochanteric fractures above type A2 in the elderly. MATERIALS AND METHODS: From December 2006 to June 2009, 54 bipolar hemiarthroplasties were performed in 54 patients in our hospital. The mean age was 78.8 (67-93) years. Of these cases, 13 were male and 41 were female. The fractures were of type A2.1 in 17 cases, type A2.2 in 23 cases and type A2.3 in 14 cases. There was no walking limitation in 45 patients, but 4 of the remaining 9 patients had walking limitations and used walking aids at their residence. A posterolateral approach as well as cementless femoral stems was used in all the patients. Clinical results were evaluated according to operation time, amount of bleeding, time to resume walking, duration of hospital stay, recovery of walking ability, and complications. Prostheses loss was evaluated on the follow-up radiographs. RESULTS: Twenty-two of 39 patients who had medical co-morbidity had more than two medical co-morbidities. Operations were performed at a mean time of 5.5 days after the fracture. The mean operation time was 95 minutes. The average total amount of bleeding was of 715 cc. Patients began walking at an average of 5.9 days after operation and the average duration of hospital stay was 19.2 days. Sixteen patients (29.6%) died at an average period of 1.6 years after their operation. At the time of discharge, 32 patients (59%) had recovered walking ability, but at the last follow-up compared to the pre-injury status, the recovery rate of walking had decreased to 46% (25 patients). Complications included a deep infection in one case, dislocation in 2 cases and hematoma in 2 cases. The cause of revision was deep infection. There were no revisions due to prosthesis loosening. CONCLUSION: Cementless bipolar hemiarthroplasty for intertrochanteric fractures in the elderly had some problems due to the prolonged operation time and increased amount of bleeding, but it also had advantages including the early return to walking after the operation and decreased hospital stay. It is one of the treatment options for the elderly with unstable intertrochanteric fractures.
Aged ; Dislocations ; Female ; Follow-Up Studies ; Hematoma ; Hemiarthroplasty ; Hemorrhage ; Hip Fractures ; Humans ; Length of Stay ; Male ; Prostheses and Implants ; Walking

Endometriosis is defined as the presence of functioning endometrial tissue outside the uterine cavity, and the standard treatment is extensive surgical excision. Cesarean scar endometriosis is a type of cutaneous endometriosis arising on or near a Cesarean section scar. A 44-year-old woman presented with a 9x6 cm sized dark-brown, stony-hard, irregular, lower abdominal mass of four years duration. The patient had a history of two Cesarean deliveries, 14 and 16 years ago. Suspecting endometriosis, we excised the tumor conservatively rather than extensively to prevent incisional hernia considering the benign nature of the tumor and the low possibility of recurrence because the patient's age was near menopause, along with simultaneous bilateral salpingo-oophorectomy that was performed in this case. On reconstruction, mini-abdominoplasty was adopted to avoid possible wound complications and cosmetic deformities. The patient was satisfied with the cosmetic results, and neither recurrence nor functional problems occurred during the 1-year follow-up period. Plastic surgeons should keep in mind the possibility of cutaneous endometriosis in an abdominal mass of a female of reproductive age with a previous history of pelvic or intra-abdominal surgery. An optimal result from oncological, functional, and cosmetic standpoints can be achieved with conservative excision followed by mini-abdominoplasty of extensive Cesarean scar endometriosis.
Abdominal Wound Closure Techniques ; Adult ; Cesarean Section ; Cicatrix ; Congenital Abnormalities ; Cosmetics ; Endometriosis ; Female ; Follow-Up Studies ; Hernia ; Humans ; Menopause ; Pregnancy ; Recurrence

Aspergillus is a ubiquitous fungus and can cause many levels of disease severity. Chronic necrotizing aspergillosis is a rare disease and few cases have been reported in Korea. We experienced a case of pleural aspergillosis that was treated successfully with medical and surgical interventions. The 52-year-old man who was diagnosed with chronic necrotizing pulmonary aspergillosis underwent surgical treatment including a lobectomy, decortication, and myoplasty. The patient was also medically treated with amphotericin B followed by voriconazole. Pleural irrigation with amphotericin B was also performed. A multi-dimensional approach should be considered for treating chronic necrotizing pulmonary aspergillosis.
Amphotericin B ; Aspergillosis ; Aspergillus ; Fungi ; Humans ; Invasive Pulmonary Aspergillosis ; Korea ; Middle Aged ; Pleural Diseases ; Pyrimidines ; Rare Diseases ; Triazoles

Scleredema is a rare connective tissue disease of unknown etiology and characterized by non-pitting thickening of subcutaneous tissue of the neck, face, head, upper trunk and arms. We present a 28 year-old man with extensive skin changes following upper respiratory infection, who have not any concurrent disorders such as diabetes mellitus. Histopathological findings of skin showed normal epidermis with a widening of spaces between collagen bundles by infiltration of acid mucopolysaccharide in the dermis.
Adult ; Arm ; Collagen ; Connective Tissue Diseases ; Dermis ; Diabetes Mellitus ; Epidermis ; Head ; Humans ; Neck ; Scleredema Adultorum* ; Skin ; Subcutaneous Tissue

BACKGROUND: The purpose of the study was to determine the value of exercise electrocardiography in predicting the area of myocardial ischemia. METHOD: Seventy-six anginal patients with a perfusion defect in one vessel territory on exercise 99mTc-MIBI myocardial perfusion scan were studied. Each patient underwent exercise electrocardiograhy using modified Bruce protocol. Exercise electrocardiography was interpreted as abnormal when the horizontal or downsloping depression in ST segment was 0.1 mV or greater at 80 msec after the J point during exercise. Forty-eight patients had exercise induced ST-segment depression. RESULT: Twenty-five patients had exercise induced ST-segment depression in single lead-group and 23 patients had in multiple lead-groups. In 18 patients (18/23) with exercise induced ST-segment depression in multiple lead-groups, the perfusion defect involved the apical area on myocardial perfusion scanning and in 21 patients (21/25) with ST-segment depression in single lead-group, the perfusion defect did not involve the apical area. In patients without perfusion defect in the apical area, ST-segment depression in anterior lead-group (V1 to V4) was associated with myocadial perfusion defects in left anterior descending artery territories in five of five cases (100%), ST-segment depression in lateral lead-group (I, aVL, V5, V6) was associated with defects in left circumflex artery territories in six of six cases (100%), and ST-segment depression in inferior lead group (II, III, aVF) was associated with defects in right coronary artery territories in nine of ten (90%) (p<0.01). In patients with perfusion defect in the apical area, exercise induced ST-segment depressions were observed in multiple lead-groups (18/22). CONCLUSION: ST-segment depression on 12 lead exercise electrocardiography was a good predictor of the site of myocadial ischemia in anginal patients with single vessel territory ischemia when ST-segment depression developed in single lead group. However, ST-segment depressions in mutiple lead-groups suggested that the perfusion defect involved the apical area and did not predict the site of myocardial ischemia.
Angina Pectoris* ; Arteries ; Coronary Vessels ; Depression ; Electrocardiography* ; Humans ; Ischemia ; Myocardial Ischemia ; Perfusion*

The aim of this cross-sectional study was to evaluate the association between vasomotor symptoms (VMS) and insulin resistance, which can be postulated by the homeostatic model assessment (HOMA) index. This study involved 1,547 Korean postmenopausal women (age, 45 to 65 years) attending a routine health check-up at a single institution in Korea from January 2010 to December 2012. A menopause rating scale questionnaire was used to assess the severity of VMS. The mean age of participants was 55.22+/-4.8 years and 885 (57.2%) reported VMS in some degree. The mean HOMA index was 1.79+/-0.96, and the HOMA index increased with an increase in severity of VMS (none, mild, moderate and severe) in logistic regression analysis (beta=0.068, t=2.665, P =0.008). Insulin resistance needs to be considered to understand the linkage between VMS and cardiometabolic disorders.
Cross-Sectional Studies ; Female ; Humans ; Insulin Resistance ; Korea ; Logistic Models ; Menopause