A Boy with X-Linked Inhibitor of Apoptosis Protein (XIAP) Deficiency as the Initial Presentation of Pure Red Cell Aplasia / 임상소아혈액종양

X-linked inhibitor of apoptosis protein (XIAP) deficiency is a rare immunodeficiency disorder. A 13-month-old boy was diagnosed with pure red cell aplasia (PRCA) and treated with steroid and cyclosporine. Bone marrow showed diminished erythroid precursors, and positive results for Parvovirus B19 and Cytomegalovirus (CMV) infection. A genetic study of Diamond-Blackfan anemia was negative. Five months later, he was admitted due to fever and lymphadenopathy. PCR showed still positive for the Parvovirus, CMV, and Epstein-Barr virus (EBV). He was diagnosed with diffuse large B-cell lymphoma that was positive for EBV. The patient received chemotherapy with R-CHOP, and achieved complete remission. Immunoglobulin (Ig) levels were within an age-matched normal range until the completion of chemotherapy. Subsequently, he was admitted nine times due to recurrent pneumonia and acute otitis media between two and eight years old. We reanalyzed the Ig levels and lymphocyte subsets: IgG, IgA, and IgM were 30 mg/dL, below 1.0 mg/dL, and 36 mg/dL, respectively. Lymphocyte subsets showed nearly absent CD19 (+) cells, but T- and NK cell counts were within normal ranges. A genetic study showed a hemizygous inframe deletion mutation in exon 4 of the IAP gene without the SH2D1A mutation, consistent with a XIAP deficiency. Therefore, he was diagnosed with XIAP deficiency. Genetic analysis of his mother, aunt and elder sister showed that they were carriers of the same gene mutation. He receives intravenous Ig regularly with follow-up, and still requires a low dose steroid to maintain hemoglobin level. We report XIAP deficiency as initial presentation of PRCA.

The Usefulness of Minimally Invasive Plate Osteosynthesis to Manage Comminuted Mid-Clavicle Fracture: A Comparison with Conventional Open Plating / 대한정형외과학회잡지

PURPOSE: The purpose of this study was to compare the clinical and radiological outcomes of minimally invasive plate osteosynthesis (MIPO) with those of conventional open plating (COP) for treating comminuted mid-clavicular fractures and to evaluate the usefulness of MIPO. MATERIALS AND METHODS: Thirty-nine patients who underwent surgical treatment for mid-clavicular comminuted fractures were analyzed retrospectively. Nineteen patients were treated with MIPO and twenty patients with COP. Radiological evaluation included time to union, fracture healing, and clavicular length difference measured as the proportional length difference with the unaffected side. Clinical assessment was performed using the simple shoulder test score, American Shoulder and Elbow Surgeons score, and quick disability of the arm, shoulder and hand score. Moreover, the mean operation and radiation times, as well as exposure were compared. RESULTS: All clavicles achieved bone union. The mean time to union was 12.1 weeks in the MIPO group, and 14.6 weeks in the COP group (p=0.587). There was no significant difference between the two groups regarding the functional and radiological outcomes at 2-year follow-up. A significantly shorter operation time was observed in the MIPO group than in the COP group (75.8 min vs. 106.9 min, p=0.002). More radiation time and exposure were identified in the MIPO group (52.8 s vs. 37.1 s, p=0.002; 209.4 mGy vs. 43.1 mGy, p=0.005). CONCLUSION: Both COP and MIPO were shown to be effective treatment options for mid-clavicular comminuted fractures. MIPO may be a better alternative to COP due to shorter operation time and no need for a bone graft, although the functional and radiological outcomes were not significantly different. However, all surgeons should pay close attention to minimize radiation hazard.

Preoperative Factors Affecting Postoperative Early Quality of Life During the Learning Curve of Holmium Laser Enucleation of the Prostate / 대한배뇨장애요실금학회지

PURPOSE: The aim of this study was to investigate the preoperative factors related to early quality of life (QoL) in patients with benign prostatic hyperplasia after holmium laser enucleation of the prostate (HoLEP) during the surgeon's learning curve. METHODS: The medical records of 82 patients with a follow-up period of at least 3 months who were treated with HoLEP during the time of a surgeon's learning curve were analyzed retrospectively. We divided the patients into two groups on the basis of the QoL component of the International Prostate Symptom Score (IPSS) 3 months after HoLEP: the high QoL group (IPSS/QoL or =4). Preoperative factors in each group were compared, including prostate volume, prostate-specific antigen, history of acute urinary retention (AUR), urgency incontinence, IPSS, and urodynamic parameters. Detrusor underactivity was defined as a bladder contractility index less than 100 on urodynamic study. RESULTS: A total of 61 patients (74.3%) had a high QoL, whereas 21 (25.7%) had a low QoL. A history of AUR, detrusor pressure on maximal flow (PdetQmax), bladder outlet obstruction grade, bladder contractility index, and detrusor underactivity were associated with postoperative QoL in the univariate analysis. In the multivariate analysis, a history of AUR and PdetQmax were independent factors affecting postoperative QoL. CONCLUSIONS: A history of AUR and bladder contractility affect early QoL, and preoperative urodynamic study plays an important role in the proper selection of patients during the HoLEP learning curve.

Differences in Bone Mineral Density between the Right and Left Hips in Postmenopausal Women

Bone mineral density (BMD) using dual energy radiography absorptiometry are commonly used for the diagnosis of osteoporosis. It is usually measured at the spine and also at one hip joint. Controversy still exists regarding the use of bilateral hip scanning. We analyzed the difference of BMD at bilateral hips in 384 postmenopausal women, retrospectively. The concordance and discordance rates of the lowest T-score and BMD between both hips were evaluated. The BMDs of the femoral neck and trochanter were significantly different between both hips (P < 0.05). There were also discrepancies between the lowest T-scores of both hips (P < 0.05). The discordance rates were about 30%. Due to significant differences in BMD between both hips at the femoral neck and trochanter and high discordance rate, bilateral hip measurements using DEXA are recommended to avoid underestimating osteoporosis.

Effective Delivering Method of Umbilical Cord Blood Stem Cells in Cutaneous Wound Healing

PURPOSE: This study was conducted to identify the most effective method in cell therapy by comparing and analyzing the level of wound healing after the application of various cell delivery methods. METHODS: Human mesenchymal stem cells were administered using 5 different methods on full thickness skin defects which were deliberately created on the back of 4-week-old mice using a 8 mm punch. Different modes of administration, cell suspension, local injection, collagen GAG matrix seeding, fibrin, and hydrogel mix methods were used. In each experiment group, 4x10(5) mesenchymal stem cells were administered according to 5 deferent methods, except for the corresponding control group. RESULTS: The wound healing rate was fastest in the local injection group. The wound healing rate was relatively slow in the collagen matrix group, however, the number of blood vessels or VEGF increased most in this group. CONCLUSION: For rapid wound healing through wound contraction, it is advantageous to administer MSC by the local injection method. For the healing process of a wide area, such as a burn, the seeding of cells to collagen matrix is thought to be effective.

Clinical Analysis of Primary Malignant Lymphoma of the Lung / 대한흉부외과학회지

BACKGROUND: Primary malignant lymphoma of the lung is a very rare neoplasm. Although the prognosis of lymphoma is favorable, the clinical features, prognostic factors and management have not been clearly defined. MATERIAL AND METHOD: We retrospectively reviewed the records of 8 patients we managed between 1994 and 2006. They all had malignant lymphoma on the pathologic examination of the lung with no evidence of mediastinal adenopathy and extrathoracic disease, and no past history of lymphoma. RESULT: The study group consisted of 3 males and 5 female patients with a mean age of 53.9 years. Three patients were asymtomatic and 5 patients were seen with pulmonary or systemic symptoms. The diagnostic methods were 3 CT needle aspiration biopsies, 1 bronchoscopic biopsy and 4 surgical methods (wedge resection, lobectomy). There were 3 patients with MALT lymphoma, two with diffuse large B-cell lymphoma, two with small lymphocytic lymphom, and one with follicular lymphoma. The 8 patients were treated with a variety of modalities, including surgery, chemotherapy, radiotherapy and combination therapy. The 8 patients have survived for a median follow-up of 38 months. CONCLUSION: Although this entity of lymphoma appears to have a good prognosis, further clinical experience and long-term follow-up are needed to identify its clinical features, prognostic factors and management.

Significance of Lipid Profiles and Peroxisome Proliferator-Activated Receptor-gamma2 Expression in Osteonecrosis of Femoral Head

PURPOSE: To analyze the possible involvement of a change in the lipid metabolism and PPARgamma2 level in the occurrence of ON of the femoral head. MATERIAL AND METHODS: The lipid profiles (Cholesterol, Triglyceride, LDL, HDL and Free fatty acid) of 130 patients with ON of the femoral head and 30 control persons were evaluated. The level of PPARgamma2 mRNA expression was examined by performing, RT-PCR using the bone marrow stromal cells obtained from 17 patients during THA. RESULTS: Among the 130 patients, the free fatty acid level in the alcohol (676.9+/-264.7 mg/dl,) and steroid (666.0+/-163.4 mg/dl) induced ON groups was higher than the control (453.5+/-169.3 mg/dl) and idiopathic (468.5+/-194.1 mg/dl) groups. The triglyceride level in the alcohol (223.7+/-70.9 mg/dl) and steroid (183.6+/-58.4 mg/dl) induced groups was higher than the control (93.1+/-79.0 mg/dl) and idiopathic groups (130.9+/-63.1 mg/dl). The level of PPARgamma2 mRNA expression in the ON patients was significantly higher than the other groups (P <0.05). CONCLUSION: These results are significant and suggest hyperlipidemia as a risk factor for ON of the femoral head. Higher expression of PPARgamma2 mRNA was found in ON. However, functional studies of PPARgamma2 mRNA in vivo would be needed to reveal the pathogenesis of ON of the femoral head.

Analysis and Management of Failed Intertrochanteric Fracture Fixation

PURPOSE: We wanted to analyze the causes of fixation failure after performing compression hip screw fixation for intertrochanteric fracture and we wanted to evaluate the methods to mange this type of failure. MATERIALS AND METHODS: From January 1999 to August 2005, 15 patients who underwent operation due to fixation failure after compression hip screw fixation for intertrochanteric fracture were evaluated, with at least 1 year follow up, for the fracture type, the status of the reduction, the aspects of failure, the causes of the failure and the clinical and radiologic results after operation. RESULTS: There were 7 cases of cut out by the hip screw, 1 case of hip screw breakage, 1 case of dislodging the hip screw, 2 cases of excessive sliding of the hip screw, 2 cases of plate breakage and 2 cases of fixation loss of the plate. The failure of cases was due to incorrect choice of the implants, and that of 9 cases was due to technical error. The Harris Hip score was an average of 67.8 for 10 cases of failed arthroplasty at the last follow up and 88.4 for 4 cases of re-fixation with a 95 degree angled blade plate, and bone union was achieved at a minimum of 5 months. CONCLUSION: It is important to choose an adequate implant and a precise technique to achieve a successful result for fixation of unstable intertrochanteric fracture, and refixation with a 95 degree angled blade plate could be considered for the management of failed intertrochanteric fracture with arthroplasty.

Recurrent Inflammation on the Hand due to a High Pressure Injection of Urethane : A Case Report / 대한정형외과학회잡지

We describe a case of a high-pressure polyurethane injection injury to the hand, and discuss how its management differs from other injection injuries. A male patient was transferred to our hospital due to persistent purulent discharge from the 2nd finger, which began 15 days after the injury. The injected foreign body, polyurethane, could not be recognized by the patient and was not indicated radiographically. During surgery, the polyurethane foam was removed. The patient had no functional impairment 24 months after surgery.

Bipolar Hemiarthroplasty Using Non-cemented Multilock Femoral Stem: A 7-year Minimum Follow-up Study

Purpose: To evaluate the clinical and radiographic results of primary bipolar arthroplasty during average 9.8-year period, using a non-cemented Multilock femoral stem and a biarticular acetabular cup. Materials and Methods: This study included 24 patients (29 hips) who underwent primary bipolar hemiarthroplasties with Multilock femoral stems and biarticular cups and who could be followed for more than seven years. Clinically, we evaluated the Harris Hip scores and patient complaints of thigh and inguinal pain. We also evaluated the radiographic measurements around the femoral stems and the bipolar cups. Results: The average Harris Hip score improved from 57.4 points to 91.6 points; and 3 (10.3%) hips were associated with thigh pain and 4 (13.8%) hips with inguinal pain. Around the femoral stem there was a non-progressive radiolucent line less than 1 mm in length in 4 (13.8%) hips and osteolysis was present in 6 (20.6%) hips. With respect to the stability of the fixations, there was osseous ingrowth in 26 (89.7%) hips and fibrous ingrowth in 3 (10.3%) hips. Around the acetabulum there was osteolysis in 5 (17.2%) hips, proximal migration of the cup in 2 (6.9%) hips, and erosion of the acetabular cartilage in 10 (34.5%) hips. There were 3 (10.3%) biarticular cups, which were converted to total hip arthroplasties, but no femoral stems were revised. The overall failure rate of the primary operations was 10.3%. Conclusion: The current study demonstrated favorable results after bipolar hemiarthroplasties with Multilock femoral stems. However, the osteolysis that occurred around the femoral stems and the acetabula emerged as a problem after the total hip arthroplasties. In particular, it is expected that the osteolysis would increase over time and become the main cause for the need for surgical revision.

Interface Stability on Insufficient Coverage of Non-cemented Acetabular Cup with Screw Fixations / 대한정형외과학회잡지

PURPOSE: To analyze the effects of various amount of peripheral defect of the acetabulum in terms of the stability of the acetabular cup with screw fixation. MATERIALS AND METHODS: The porous coated acetabular cup of Trilogy was press-fitted with 3 screw fixations after 2 mm undersized reaming into the distal part of 100 bovine femurs, but with insufficient peripheral coverage of 0%, 5%, 10%, 15% and 20% respectively. The micromotions of the acetabular cups were measured under the axial loads of 50, 100, 150, 200, 250, and 300 Kp, and torques were measured at 1degrees to 5degrees of cup rotation under applied rotational force in 10 specimens in each of five groups. RESULTS: The micromotion between the cup and bone interface increased proportionally with peripheral coverage insufficiency and the torque values in the five groups increased in proportion to the rotation degree and were inversely proportinal peripheral coverage insufficiency. CONCLUSION: 15% or less coverage insufficiency of the acetabular cup may be acceptable as the average micromotion is less than 150micrometa and compatible with biologic fixations.

Cytogenetic Study in Suspicious Cases of Malignant Pleural Effusion / Journal of the Korean Cancer Association, 대한암학회지

PURPOSE: This study was performed to detect malignant cells in suspicious cases of malignant pleural effusion by cytogenetic analysis. MATERIALS AND METHODS: Eleven cases with pleural effusion were included in this study. Cells in pleural effusion were treated by direct, or short term, culture to prepare chromosomes. To analyze chromosomes, the G-banding method was used. RESULTS: Chromosome preparations succeeded in 10 cases. 5 cases had normal karyotypes, but in 2 of these cases malignant cells were detected on cytological examination. The other 5 cases had abnormal chromosomes, but on cytological examination showed normal cell appearances. CONCLUSION: Cytogenetic analysis of pleural effusions is not used routinely, but is more sensitive than the cytological examination of malignant pleural effusions. So, chromosome analysis is a good diagnostic tool, when chromosomal abnormalities are detected in an effusion. If a combination of cytology and cytogenetic study are used, the chance of detecting malignant cells in pleural effusion will be higher, and then more invasive diagnostic procedures, such as thoracoscopy or thoracotomy, could be avoided.

Polyethylene Liner Wear in Harris-Galante Acetabular Cup: Two Dimensional versus Three Dimensional Method / 대한정형외과학회잡지

PURPOSE: The purpose of this study was to compare the two-dimensional with three-dimensional radiographic measurements of polyethylene liner wear in the Harris-Galante II acetabular cup. MATERIALS AND METHODS: We measured the polyethylene liner wear amount and the wear rate of 64 hips with Harris-Galante II acetabular cup by two methods: Livermore's method and modified Devane's method. We evaluated the relationships between the two methods. RESULTS: The average total amount of wear and the average wear rate are 0.86+/-1.01 mm and 0.18+/-0.20 mm/year using the two-dimensional method and 0.99+/-1.01 mm and 0.21+/-0.2 mm/year using three-dimensional method respectively. The amount of wear and the wear rate by the three-dimensional method was larger than those by the two dimensional method. CONCLUSION: The amount of polyethylene liner wear can be measured by the two-dimensional method because it is highly correlated with the amount that is measured by the three-dimensional method, since there was no differences between the two methods.

A Case of endobronchial metastasis from prostatic carcinoma / 결핵

Carcinoma of the prostate is a common malignancy affecting elderly men. Lung metastasis from prostate cancer occurs frequently, but tumor metastasis to the central bronchi that clinically mimics primary bronchogenic carcinoma are very rare. We report a 73-year old man with endobronchial metastasis from prostatic carcinoma presented with respiratory symptom cough. Diagnosis of issues taken from materials which were used for bronchoscopic biopsy and prostate biopsy and immunohistochemical staining for prostate specific antigen(PSA) confirmed a case of endobronchial metastasis from prostatic carcinoma. Hormonal therapy(LHRH agonist) was applied to this patient.

Non-union of Scaphoid Fracture: Comparison according to the Method of Operation / 대한정형외과학회잡지

PURPOSE: To evaluate the operative methods which can induce an early uinon and better functional outcome in the non-union of scaphoid fracture. MATERIALS AND METHODS: We evaluated 16 patients (16 cases) who had taken autogenous iliac bone graft and internal fixation with compression screws or K-wires for the treatment of scaphoid fracture non-union. The average follow-up period was 21.1 months. Anteroposterior, lateral and scaphoid views were taken at last follow-up for the purpose of evaluating radiological results. The ranges of wrist motion and grip strength were measured to evaluate functional results, and the patient's subjective responses were also evaluated. RESULTS: Radiological unions were obtained in 15 cases (93.8%) with the average time to union at 3.2 months (2~7 months) . Radio-ulnar deviation arcs and grip strength were better in the cases fixated with compression screws than K-wires. Twelve patients (75%) showed satisfactory subjective results at the time of last follow up. CONCLUSION: The iliac bone graft is a good treatment method for scaphoid fracture non-union. We think that the compression screw, which has better stability, is a more profitable fixation device for improved functional recovery.

Clinical Manifestations of Mediastinal Tumors / 대한내과학회지

OBJECTIVES: Mediastinal tumors are not uncommon but almost half of them are asymptomatic because of the potential space of the mediastinum. Recently owing to the routine screening chest roentgenography and frequent chest CT scans the incidence have increased. Mediastinal masses are both benign or malignant. Even benign masses may continue to enlarge and compromise vital organ function or rupture, bleed, and become infected. Mediastinal tumors have their own predilection site. There are different incidences of these masses in adults and children, sexes, and there may be difference in incidence between races. There are only a few reports about these tumors in Korea, and we are interested in the clinical manifestations of mediastinal tumors in Korea, METHODS: We retrospectively reviewed medical records of 286 cases of mediastinal tumors which were pathologically confirmed at Keimyung, Kyungpook, and Yeungnam University Hospital in Taegu from 1985 to 1994 but excluded metastatic lesion, tuberculosis, and tumor originated from esophagus, heart, and large vessels. RESULTS: Mediastinal tumors were more frequent in male(60.8%) than female and age distribution was relatively even. Thymoma was most common mediastinal tumor(107 cases, 37.4%). The most common tumor in anterior mediastinum was thymoma (107/212, 50.5%). In posterior mediastinum, neurogenic tumor was the most common(52/67, 77.6%) and teratodermoid tumor was common in middle mediastinum(3/7, 42.9%). The most common symptom was chest discomfort(129 cases, 45.1%) and 67 cases were asymptomatic(23.4%). Benign tumors were more common(175 cases, 61.1%) than malignant tumors. In therapies, complete or partial resections were done in 190 cases(79.8%), chemotherapy or radiotherapy in 71 cases(29.8%), in 24 cases(10.1%) only biopsy was taken without any treatment. Neurogenic tumors have been reported as the most common tumors in western countries and neurogenic and teratodermoid tumor were found to be the most common tumors in the past Korean reports. But thymoma was the most common tumor in this study. CONCLUSION: Thymoma was the most common mediactinal tumor in this study. And thymoma was the most common tumor in anterior mediastinum neurogenic tumor in posterior rnediastinum and teratodermoid tumor in middle mediastinum