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Cardiac function associated with home ventilator care in Duchenne muscular dystrophy
이상흔,이희영,은영민,강성웅 대한소아청소년과학회 2018 Clinical and Experimental Pediatrics (CEP) Vol.61 No.2
Purpose: Cardiomyopathy is becoming the leading cause of death in patients with Duchenne muscular dystrophy because mechanically assisted lung ventilation and assisted coughing have helped resolve respiratory complications. To clarify cardiopulmonary function, we compared cardiac function between the home ventilator-assisted and non–ventilator-assisted groups. Methods: We retrospectively reviewed patients with Duchenne muscular dystrophy from January 2010 to March 2016 at Gangnam Severance Hospital. Demographic characteristics, pulmonary function, and echocardiography data were investigated. Results: Fifty-four patients with Duchenne muscular dystrophy were divided into 2 groups: home ventilator-assisted and non–ventilator-assisted. The patients in the home ventilator group were older (16.25±1.85 years) than those in the nonventilator group (14.73±1.36 years) (P=0.001). Height, weight, and body surface area did not differ significantly between groups. The home ventilator group had a lower seated functional vital capacity (1,038±620.41 mL) than the nonventilator group (1,455±603.12 mL). Mean left ventricular ejection fraction and fractional shortening were greater in the home ventilator group, but the data did not show any statistical difference. The early ventricular filling velocity/late ventricular filling velocity ratio (1.7±0.44) was lower in the home ventilator group than in the nonventilator group (2.02±0.62). The mitral valve annular systolic velocity was higher in the home ventilator group (estimated β, 1.06; standard error, 0.48). Patients with Duchenne muscular dystrophy on a ventilator may have better systolic and diastolic cardiac functions. Conclusion: Noninvasive ventilator assistance can help preserve cardiac function. Therefore, early utilization of noninvasive ventilation or oxygen may positively influence cardiac function in patients with Duchenne muscular dystrophy.
신생검에서 급성 사구체신염 양상을 보인 Henoch-$Sch{\ddot{o}}nlein$ 자반증 2례
이상흔,남궁미경,차병호,김종수,Lee Sang Heun,Namgoong Mee Kyung,Cha Byung Ho,Kim Jong Soo 대한소아신장학회 1998 Childhood kidney diseases Vol.2 No.2
[ $\beta$ ]-streptococci have been reported as an etiology of Henoch-$Sch{\ddot{o}}nlein$ purpura(HSP), but the role of $\beta$-streptococci in HSP is not yet confirmed. Our report of two HSP patients is as follows : Although the two patients' clinical symptoms resembled those symptoms of HSP, whereas serum $C_3$ decreased in one case, ASO titer increased in both cases and a histological examination of renal biopsy specimens revealed the fetures commonly described in poststreptococcal glomerulonephritis.
갑상샘눈병증 환자에서 보툴리눔독소를 이용한 위눈꺼풀뒤당김 치료의 결과
이상흔,유혜린,윤영수,Sang Hun Lee,Helen Lew,Young Soo Yun 대한안과학회 2006 대한안과학회지 Vol.47 No.8
Purpose: This study is to determine the effects of botulinum toxin injection on patients who suffer from upper eyelid retraction caused by thyroid ophthalmopathy. Methods: We injected 10-15 units botulinum toxin (Botox?, Allergan, USA) on the upper tarsus of 12 eyes of 12 thyroid ophthalmopathy patients and compared duration of disease, clinical activity scores (CAS), intraocular pressures (mmHg), exophthalmometry (mm), upper eyelid retraction (mm), marginal reflex distance1 (MRD1, mm), levator function test (mm), lid lag (mm), limitation of ocular movement (mm), lid tension test results, phenylephrine test results, and orbital computed tomography results before and after the injection. Results: The patient group with smaller-sized cross section of superior rectus/levator complex before the injection showed a greater decrease of upper eyelid retraction after the injection (p=0.04). Other clinical parameters showed no significant relations on the effect of the toxin injection. Conclusions: Botulinum toxin injection can have variable effects on the upper eyelid retraction of thyroid ophthalmopathy patients. The patients who had smaller volume of superior rectus/levator complex measured in orbital cross sections on CT, could expect good results after botulinum toxin injection.
이상흔,유혜린,윤영수 대한안과학회 2006 대한안과학회지 Vol.47 No.8
Purpose: To compare the characteristics of the intermittent exotropia patients with spontaneous regression to the surgically corrected group. Methods: Twenty-five patients who had exodeviation of around 25 prism diopters (PD). Visual acuity and best corrected visual acuity were measured. The latter was obtained by using cycloplegic refraction. Far and near deviation angles were measured through alternating prism cover test. Stereo acuity was measured using a Titmus stereo acuity test chart and occlusion was performed for those who had more than 2 lines of difference between the best corrected visual acuity of the left and right eyes on the Snellen E chart. We divided the patients into two groups. The first group is the spontaneous regression group (SRG), who showed declining deviation angles and maintained less than 10 PD for more than 3 months. The second group is the surgically corrected group (SCG), showed the same or increasing deviation angle and underwent operation. Results: There was no difference in the age or observation period between the two groups. The prevalence period was significantly higher in the SRG than in the SCG. The deviation angle from a distance at the first visit was not different for the SCG from that of the SRG. However, the deviation angle at near was significantly larger for the SCG than that of the SRG and increased at the last examination. Stereo acuity of the SRG was better than that of the SCG at the first visit. Conclusions: Deviation angle could be reduced in the mild intermittent exotropia patients with relatively small deviation angle at near or with good stereo acuity.
이상흔,유혜린,윤영수,Sang-Hun Lee,Helen Lew,Young-Soo Yun 대한안과학회 2005 대한안과학회지 Vol.46 No.7
Purpose: There have been few studies on the dynamic analysis of lower eyelid movement. The inferior oblique muscle deep in the inferior orbit is well connected with the extraocular muscle by capsulopalpebral fascia and is involved in the lower eyelid movement. The purpose of this study was to determine the graphical equation of the lower eyelid contour and to quantify the dynamic movement of the lower eyelid at primary gaze and inferior oblique action gaze in normal eyes according to age, as such findings have never been reported before. Methods: One hundred and forty subjects who had no previous eye or eyelid disease and surgery, aged from 5 to 68 years (35.75±30.59 years) were enrolled and divided into 7 age groups. We analyzed digital camera images to obtain the lower eyelid curvature and traveling distance of the lowest point of the lower eyelid accompanied by the action of the inferior oblique muscle. Results: Graphical analysis presented the relationship of the cubic equation resembling the lower eyelid contour. The curvature of the lower eyelid at the gaze of inferior oblique muscles action became flatter than that of the primary position in all age groups. The lowest point of the lower eyelid moved significantly to the medial position and up from the primary gaze at the gaze of the inferior oblique muscles action in all age groups. There was no difference in traveling distance of the lower eyelid at each direction between sexes and age groups. Conclusions: The lower eyelid movements associated with the inferior oblique muscle are believed to be innate. We suggest that the digital image analysis of the lower eyelid contour will be useful to analyze the lower eyelid movements.