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X-linked Adrenoleukodystrophy in Childhood (X 염색체 연관 부신백질이영양증 환아들의 임상양상)

  • Yoo, Eun Jung;Kim, Eun Young;Kook, Hoon;Woo, Young Jong
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.5 no.1
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    • pp.33-43
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    • 2005
  • Purpose: X-linked adrenoleukodystrophy(ALD) is a rare disorder that shows a great deal of phenotypic variability. We subdivided chidhood X-linked ALD patients into several phenotypes by the age at onset, the sites of most severe clinical involvement and the rate of progression of neurologic symptoms. Methods: Thirteen patients who had been diagnosed as X-linked ALD and followed up for at least one year were enrolled from 1996 to 2003. Results: 1. Ten had childhood cerebral ALD, who showed first neurologic symptoms at 7.02 years and progressed rapidly: interval between first symptoms and vegetative state was 1.35 years, and interval from initial symptoms to death was 3.35 years. Treatment with Lorenzo's oil did not prevent neurologic progression. Two patients who underwent umbilical cord blood transplantation died. 2. Two had adolescent cerebral ALD. They had first symptoms at 11.5 years, and showed tendency to progress less rapidly than childhood cerebral form patients. 3. One "Addison only" patient who had adrenal insufficiency without nervous system involvement remained asymptomatic during Lorenzo's oil treatment. 4. All cerebral form patients except one showed the lesions in both parieto-ocipital white matter in brain magnetic resonance imaging. Conclusion: The cerebral ALD was the most common form in childhood and was asoociated with a serious prognosis.

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Osteoid Osteoma Around the Hip Joint (고관절 주위에 발생한 유골 골종)

  • Kim, Tai-Seung;Kim, Jong-Heon;Lee, Bong-Gun;Kim, Soon-Myung
    • The Journal of the Korean bone and joint tumor society
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    • v.11 no.2
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    • pp.168-174
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    • 2005
  • Purpose: We experienced 8 cases of osteoid osteoma arising around hip joint which had some characteristics that differ from those arising on long bone. we reports the characteristics of osteoid osteoma arising around the hip joint. Materials and Methods: 8 cases of osteoid osteoma were diagnosed during 1985 to 2004 at hanyang university hospital. all cases were comfirmed patholgically. 6 cases were male, 2 cases were female patients. The mean age was 17 years old (ranged from 8 to 29). They occurred in intertrochanteric area (4 cases), subtrochanteric area (2 cases), acetabulum (1 case) and femoral neck (1 case). We used radiologic tools including magnetic resonance image, computed tomography, bone scintigraphy. clinicopathologic test including erythrocyte sedimentation rate and Creactive protein. Results: The patients expressed various symptoms including thigh pain, knee pain, low back pain and radiating pain respectively. 2 patients had experienced operation on knee joint. 3 patients showed limping gait. Aspirin relieved the pain in 3 patients. The difference in circumference was 1cm between both thighs in 2 cases. Conclusion: Patients with osteoid osteoma arising around hip joint which have various symptoms such as severe knee pain and claudication, differ from infectious disease by clinicopathologic test including erythrocyte sedimentation rate and C- reactive protein and had better diagnostic result in computed tomography.

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Clinical Findings on Vertebral Compression Fracture Diagnosed with MRI (자기공명영상으로 진단된 척추 압박골절의 임상적 소견)

  • Kim, Ham-Gyum
    • Journal of radiological science and technology
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    • v.28 no.3
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    • pp.219-226
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    • 2005
  • By analyzing the severally clinical characteristics such as the prevalence by gender and age, and the developmental region, and the developmental factor in terms of vertebral compression fracture, which was diagnosed by MRI(Magnetic Resonance Imaging), the following conclusions were obtained. 1. The general characteristics in 183 research subjects, were 70 men (38.0%) and 113 women (62.0%), and aged from the minimum 16 years old to the maximum 84 years old. 2. Among 183 persons with abnormal findings in vertebral compression fracture, the single compression fracture was included 111 persons (60.7%) with 38 men (34.0%) and 73 women (66.0%), and women in their 60s were largest with 32 persons. 3. As the multiple compression fracture was included 72 persons (39.3%) among 183 research subjects, and as the incidence had 182 cases, this is what classified the case in which one person with abnormal findings has the compression fracture at the levels with more than one, and there were 70 cases (38.0%) in men and 112 cases (62.0%) in women, and it is being indicated the large frequency in women in their 60s and in their 70s. 4. The developmental regions in the single compression fracture and the multiple compression fracture, were generated about 70% of the whole at $T11{\sim}L2$. 5. In terms of the prevalence by cause for compression fracture in 183 whole subjects, it was largest with 41.4% in the fracture caused by trauma in case of men, and with 70.0% in what was caused by the osteoporosis in case of women, and as for a case that combined men and women, it was largest with 56.8% in the vertebral compression fracture caused by osteoporosis.

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Usefulness of $^{99m}Tc$-labeled RBC Scan and SPECT in the Diagnosis of Head and Neck Hemangiomas (두경부 혈관종 진단시 $^{99m}Tc$-RBC Scan and SPECT 검사의 유용성)

  • Oh, Shin-Hyun;Roh, Dong-Wook;Ahn, Sha-Ron;Park, Hoon-Hee;Lee, Seung-Jae;Kang, Chun-Goo;Kim, Jae-Sam;Lee, Chang-Ho
    • The Korean Journal of Nuclear Medicine Technology
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    • v.12 no.1
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    • pp.39-43
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    • 2008
  • Purpose: There are various methods to diagnose hemangioma, such as ultrasonography (US), computed tomography (CT), magnetic resonance imaging (MRI) and nuclear medicine. However, by development of SPECT imaging, the blood-pool scan using $^{99m}Tc$-labeled red blood cell has been used, because it was non-invasive and the most economical method. Therefore, in this study, we proposed that the usefulness of $^{99m}Tc$-RBC scan and SPECT of the head and neck to diagnose unlocated hemangiomas. Materials and Methods: $^{99m}Tc$-RBC scan and SPECT was performed on 6 patients with doubtful hemangioma (4 person, head; 1 person, neck; 1 person, another). We labeled radiopharmaceutical using modified in vivo method and then, centrifuged it to remove plasma. After a bolus injection of tracer, dynamic perfusion flow images were acquired. Then, anterior, posterior, both lateral static blood-pool images were obtained as early and 4 hours delayed. SPECT was progressed 64 projections per 30 seconds. Each image was interpreted by physicians, Nuclear medicine specialist, and technologist blinded to patient's data. Results: In 5 patients of all the radioactivity of doubtful site didn't change in flow images, but, in blood-pool, delayed and SPECT images, it was increased. So, it was a typical hemangioma finding. The size of lesion was over 2 cm, and it could discriminate as comparing to the delayed and SPECT imaging. On the other hand, in 1 patient, the radioactivity was increased in blood-pool images, but, not in delayed and SPECT images, so, it was proved no hemangioma. Conclusion: Using $^{99m}Tc$-RBC Scan and SPECT, we could diagnose the hemangiomas in head and neck, as well as, liver, more non-invasive, economical, and easy. Therefore, it considered that $^{99m}Tc$-RBC scan and SPECT would offer more useful information for diagnosis of hemangioma, rather than otherimaging such as US, CT, MRI.

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The Molecular Weight Dependance of Paramagnetic Gd-chelates on T1 and T2 Relaxation Times (상자성 복합체의 분자량에 따른 T1 및 T2 자기이완시간에 관한 연구)

  • Kim In-Sung;Lee Young-Ju;Kim Ju-Hyun;Sujit Dutta;Kim Suk-Kyung;Kim Tae-Jeong;Kang Duk-Sik;Chang Yong-Min
    • Progress in Medical Physics
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    • v.17 no.2
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    • pp.61-66
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    • 2006
  • To evaluate the T1, T2 magnetic relaxation properties of water molecule according to molecular weight of paramagnetic complex. 4-aminomethyicyclohexane carboxylic acid (0.63 g, 4 mmol) was mixed with the suspension solution of DMF (15 ml) and DTPA-bis-anhydride (0.71 g, 2 mmol) to synthesize the ligand. The ligand was then mixed with $Gd_2O_3$ (0.18 g, 0.5 mmol) to synthesize Gd-chelate. For the measurement of magnetic relaxivity of paramagnetic compounds, the compounds were diluted to 1 mM and then the relaxation times were measured at 1.57 (64 MHz). Inversion-recovery pulse sequence was employed for T1 relaxation measurement and CPMG (Carr-Purcell-Meiboon-Gill) pulse sequence was employed for T2 relaxation measurement. In case of inversion recovery sequence, total 35 images with different inversion time(T1)s ranging from 50 msec to 1,750 msec. To estimate the relaxation times, the signal intensity of each sample was measured using region of Interest (ROI) and then fitted by non-linear least square method to yield T1, T2 relaxation times and also R1 and R2. Compared to T1=($205.1{\pm}2.57$) msec and T2=($209.4{\pm}4.28$) msec of Omniscan (Gadodiamide), which is commercially available paramagnetic MR agent, T1 and T2 values of new paramagnetic complexes were reduced along with their molecular weight. That is, T1 value was ranged from $(96.35{\pm}2.04)\;to\;(79.38{\pm}1.55)$ msec and T2 value was ranged from $(91.02{\pm}2.08)\;to\;(76.66{\pm}1.84)$ msec. Among new paramagnetic complexes, there is a tendency that the R1 and R2 increase as the molecular weight is increases. As molecular weight of paramagnetic complex increases, T1 and T2 relaxation times reduce and thus the increase of relaxivity (R1 and R2) Is proportional to molecular weight.

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The results of arthroscopic repair according to the delamination of rotator cuff (회전근 개 판분리 파열에 따른 관절경하 회전근 개 봉합술의 결과)

  • Ku, Jung Hoei;Cho, Hyung Lae;Park, Man Jun;Kim, Jeong Cheol
    • Journal of Korean Orthopaedic Sports Medicine
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    • v.10 no.2
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    • pp.61-68
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    • 2011
  • Purpose: We evaluated the clinical outcome after arthroscopic repair in full thickness rotator cuff tears with and without delamination. Materials and Methods: From March 2006 to October 2008, we included 48 consecutive shoulders (31 males, 17 females; mean age 57.6 years; 45~68) who had arthroscopic double row repair for fullthickness tears of the rotator cuff. Mean rotator cuff tear size was 2.8 cm (range: 1.2~3.6) and the techniques of tendon-to-bone fixation varied according to the presence of delamination; separate row fixations of bursal and articular layer were used in delaminated tear. The mean follow-up was 26 months (range: 18~33) and functional and structural results were evaluated by American Shoulder and Elbow Surgeons (ASES), University of California at Los Angeles (UCLA) scale, isokinetic strength testing and magnetic resonance imaging (MRI) obtained mean 8 months (range:6~13) postoperatively. The patterns of delamination, age, sex, symptom duration, size of tear, satisfaction rate, retear rate ware compared and significance was set at p values < 0.05. Results: Postoperative functional shoulder score improved significantly in 44 shoulders (91.7%). Delamination was observed in 15 shoulders (31%) and it extended proximally and posteriorly in the majority of shoulders, and the articular layer was thicker (8/15, 53%) and more retracted (9/15, 60%) compared with the superficial bursal layer. Final follow up functional shoulder scores showed no differences between non-delaminated and delaminated tears and the presence of delamination had no correlations with sex, symptom duration, tear size and satisfaction rate, however, older age had more delaminated tears (p=0.041). Follow up MRI in 29 shoulders revealed that fourteen (48%) shoulders had complete healing; nine (31%), partial healing; six (21%), complete retear but the half of the retear group showed favorable clinical results. 79% (15/19) in non-delaminated tear and 80% (8/10) in delaminated tear were judged as healed tendon on MRI and double-layer double row repairs in delaminated tears resulted in nearly same rate of structural integrity of single-layer double row repairs (p=0.165). Conclusion: The incidence of delamination in our series was 31% and older age had more delaminated tears. Sex, symptom duration, preoperative size of the tear, functional results and satisfaction rate had no significant correlations with the presence of delamination. Nearly the same postoperative structural integrity was noted in both delaminated and non-delaminated tears.

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Arthroscopic Decompression of Spinoglenoid Ganglion Cyst (견갑골 극관절와 결절종의 관절경하 감압술)

  • Hwang, Tae Hyok;Wang, Tae Hyun;Cho, Hyung Lae;Kim, Keun Young
    • Journal of the Korean Arthroscopy Society
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    • v.15 no.2
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    • pp.92-98
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    • 2011
  • Purpose: We describe a all-arthroscopic technique for decompression of spinoglenoid ganglion cyst and present our clinical results for this procedure. Materials and Methods: From March 2006 to June 2009, eight patients (7 males, 1 female; mean age 40.6 years; range: 21~61) were included who underwent arthroscopic decompression of spinoglenoid ganglion cyst. The most common symptoms were vague shoulder pain and external rotation weakness, which lasted 6.4 months (range: 3~8) on average. Five of eight patients were noted abnormalities on electromyographic (EMG) examination to have suprascapular nerve neuropathy and magnetic resonance imaging (MRI) showed spinoglenoid ganglion cysts in all eight patients and the cyst size was 2.6 cm (range: 1.8~3.6). Labral pathology was identified intraoperatively in all patients and the cysts were decompressed by the posterosuperior capsulotomy under arthroscopic control and labral repair with suture anchors was performed in six patients. Results: The average clinical outcome scores including Constant and Murley, Simple shoulder test (SST) all improved significantly at the time of the final follow-up and there were no complications resulting from the procedures. All patients including the patients with abnormal EMG study recovered strength on isokinetic strength testing. Follow-up MRI scans were performed on all patients at a mean of 5.2 months postoperatively (range: 3~12) revealed complete resolution of the cysts and no evidence of recurrences were seen at an average of 18 months (range: 12~26) of follow-up. Conclusion: Arthroscopic decompression of spinoglenoid ganglion cyst effectively restores patient function and all patients in this study showed improvement in their postoperative MRI findings. Arthroscopic decompression is also useful in the appropriate treatment for labral pathology and may contribute to decreased risk of cyst recurrence.

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The Effect of Placing Biomembrane cover following Microfracture on Cartilage Repair: Comparison with Conventional Microfracture Technique in a Prospective Randomized Trial (미세골절술 후 생체막 덮개가 연골 재생에 미치는 영향 : 고식적인 미세골절술과의 전향적 비교 연구)

  • Son, Kwang-Hyun;Kim, Jin-Ho;Kwak, Kyu-Sung;Park, Jang-Won;Yoon, Kyoung Ho;Min, Byoung-Hyun
    • Journal of the Korean Arthroscopy Society
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    • v.15 no.2
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    • pp.83-91
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    • 2011
  • Purpose: Microfracture has been used as a first-line treatment to repair articular cartilage defects. In this study, a new technique using an extracelluar matrix biomembrane to cover the cartilage lesions after microfracture was evaluated in terms of cartilage repairability and clinical outcome compared with conventional microfracture technique in a prospective randomized trial. Materials and Methods: A total of 53 patients (59 cases) without osteoarthritis who had focal full thickness articular cartilage lesions were randomly assigned in two group. Seventeen patients (17 cases) underwent conventional microfracture procedure (control group) and thirty-six patients (42 cases) received microfracture and placing biomembrane cover (ArtiFilm$^{TM}$) concomitantly (experimental group). Clinical assessment was done through 6 months postoperatively using the subjective International Knee Documentation Committee IKDC questionnaire, and visual analog scale (VAS) for pain and satisfaction. Magnetic resonance imaging (MRI) was performed at 6 months after the operation in all patients. Results: In clinical outcomes, the significant difference was observed between both groups in IKDC, but not in VAS for pain and for satisfaction (final outcomes of IKDC, p=0.001; VAS for pain, p=0.074; VAS for satisfaction, p=0.194). The MRI showed good to complete defect fill (67 to 100%) in 33 patients (78.6%) of experimental group and 4 patients (23.5%) of control group, respectively. In control group, 9 of 17 patients (52.9%) showed poor defect fill (less than 33%), whereas 5 (11.9%) in experimental group (p=0.001). Assessment of peripheral integration revealed no gap formation in 35 patients (83.3%) in experimental group and 6 patients (35.3%) in control group (p=0.001). No serious complications or adverse effects related to the biomembrane were found. Conclusion: Good short-term follow-up clinical results were obtained in the group whose cartilage defects in the knee joint were covered with biomembrane after the microfracture, with the MRI findings confirming the excellent regeneration of the defective cartilage area. This suggests that the surgery to cover the defective area with biomembrane (ArtiFilm$^{TM}$) after the microfracture procedure is a safe, more effective treatment to induce cartilage regeneration.

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Trend in Research and Application of Hard Carbon-based Thin Films (탄소계 경질 박막의 연구 및 산업 적용 동향)

  • Lee, Gyeong-Hwang;Park, Jong-Won;Yang, Ji-Hun;Jeong, Jae-In
    • Proceedings of the Korean Institute of Surface Engineering Conference
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    • pp.111-112
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    • 2009
  • Diamond-like carbon (DLC) is a convenient term to indicate the compositions of the various forms of amorphous carbon (a-C), tetrahedral amorphous carbon (ta-C), hydrogenated amorphous carbon and tetrahedral amorphous carbon (a-C:H and ta-C:H). The a-C film with disordered graphitic ordering, such as soot, chars, glassy carbon, and evaporated a-C, is shown in the lower left hand corner. If the fraction of sp3 bonding reaches a high degree, such an a-C is denoted as tetrahedral amorphous carbon (ta-C), in order to distinguish it from sp2 a-C [2]. Two hydrocarbon polymers, that is, polyethylene (CH2)n and polyacetylene (CH)n, define the limits of the triangle in the right hand corner beyond which interconnecting C-C networks do not form, and only strait-chain molecules are formed. The DLC films, i.e. a-C, ta-C, a-C:H and ta-C:H, have some extreme properties similar to diamond, such as hardness, elastic modulus and chemical inertness. These films are great advantages for many applications. One of the most important applications of the carbon-based films is the coating for magnetic hard disk recording. The second successful application is wear protective and antireflective films for IR windows. The third application is wear protection of bearings and sliding friction parts. The fourth is precision gages for the automotive industry. Recently, exciting ongoing study [1] tries to deposit a carbon-based protective film on engine parts (e.g. engine cylinders and pistons) taking into account not only low friction and wear, but also self lubricating properties. Reduction of the oil consumption is expected. Currently, for an additional application field, the carbon-based films are extensively studied as excellent candidates for biocompatible films on biomedical implants. The carbon-based films consist of carbon, hydrogen and nitrogen, which are biologically harmless as well as the main elements of human body. Some in vitro and limited in vivo studies on the biological effects of carbon-based films have been studied [$2{\sim}5$].The carbon-based films have great potentials in many fields. However, a few technological issues for carbon-based film are still needed to be studied to improve the applicability. Aisenberg and Chabot [3] firstly prepared an amorphous carbon film on substrates remained at room temperature using a beam of carbon ions produced using argon plasma. Spencer et al. [4] had subsequently developed this field. Many deposition techniques for DLC films have been developed to increase the fraction of sp3 bonding in the films. The a-C films have been prepared by a variety of deposition methods such as ion plating, DC or RF sputtering, RF or DC plasma enhanced chemical vapor deposition (PECVD), electron cyclotron resonance chemical vapor deposition (ECR-CVD), ion implantation, ablation, pulsed laser deposition and cathodic arc deposition, from a variety of carbon target or gaseous sources materials [5]. Sputtering is the most common deposition method for a-C film. Deposited films by these plasma methods, such as plasma enhanced chemical vapor deposition (PECVD) [6], are ranged into the interior of the triangle. Application fields of DLC films investigated from papers. Many papers purposed to apply for tribology due to the carbon-based films of low friction and wear resistance. Figure 1 shows the percentage of DLC research interest for application field. The biggest portion is tribology field. It is occupied 57%. Second, biomedical field hold 14%. Nowadays, biomedical field is took notice in many countries and significantly increased the research papers. DLC films actually applied to many industries in 2005 as shown figure 2. The most applied fields are mold and machinery industries. It took over 50%. The automobile industry is more and more increase application parts. In the near future, automobile industry is expected a big market for DLC coating. Figure 1 Research interests of carbon-based filmsFigure 2 Demand ratio of DLC coating for industry in 2005. In this presentation, I will introduce a trend of carbon-based coating research and applications.

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Ultrasound Measurement of Coracohumeral Distance in Patients with or without Subcoracoid Impingement (오구돌기하 충돌 증후군 유무에 따른 초음파를 이용한 상완오구돌기 계측)

  • Jang, Suk Hwan;Kim, Sang Bum
    • The Journal of Korean Orthopaedic Ultrasound Society
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    • v.7 no.1
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    • pp.20-27
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    • 2014
  • Purpose: The purpose of this study was to evaluate coracohumeral distance (CHD) in patients with or without subcoracoid impingement with hypothesis that patients with subcoracoid impingement would have narrower CHD. Materials and Methods: One hundred twenty-four patients with subacromial impingement were evaluated. The subjects with subcoracoid impingement which was affirmed clinically and confirmed by ultrasound guided subcoracoid injection (n=28) was compared with patients with subacromial impingement only (n=96). Patients with stiffness and rotator cuff tear were excluded. Absolute CHD was measured on magnetic resonance imaging (MRI) axial images and on ultrasound with the humerus in neutral position and internal rotation. Also relative ratio of distance difference (RRDD) defined as the difference of CHD in neutral position and internal rotation compared with absolute CHD in neutral on ultrasound was also measured. Results: The distance measured in neutral position was similar between US imaging and MRI (p>0.05) and both measurements did not have significant difference between the two groups (p>0.05). On ultrasound, the difference in CHD in internal rotation between the two groups nearly met the level of significance (p=0.07). No significant difference of CHD difference in two humeral positions was seen between the two groups. However, RRDD value was significantly greater in subcoracoid impingement group (p<0.05). Conclusion: No significant difference of CHD was seen between the subcoracoid impingement group and the control group. RRDD value was greater in subcoracoid impingement group suggesting that individualized coracohumeral distance in internal rotation should be taken into account when assessing patients with subcoracoid impingement.

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