Albinism is a hereditary disease caused by the defect of tyrosinase that converts tyrosine to dihydroxyphenylalanine (DOPA). `Oculocutaneous albinism' is classified as hypopigmentation of skin, hair and eyes, but incidences of `ocular albinism' where hypopigmentation is limited to eyes are found rarely. Biochemically, albinism is caused by the tyrosinase activity. Typical findings in oculocutaneous albinism include not only ophthalmologic problems such as hypopigmentation of skin, foveal hypoplasia, photophobia and decreased visual acuity but also congenital nystagmus. We cannot determine distinctive characteristics of nystagmus of albinism because domestically, there are only a few reports that have been recorded correctly about nystagmus of albinism. Merely, we present our experience of two cases of albinism with congenital nystagmus because we think that these two cases, showing different types of nystagmus and electronystagmography, stand for the two representative types of nystagmus found in the literature up to date.
Albinism ; Albinism, Oculocutaneous* ; Dihydroxyphenylalanine ; Electronystagmography ; Genetic Diseases, Inborn ; Hair ; Hypopigmentation ; Incidence ; Monophenol Monooxygenase ; Nystagmus, Congenital* ; Photophobia ; Skin ; Tyrosine ; Visual Acuity

Recently refractive corneal surgery has been increasing and intraocular lens implantation is a routine procedure during cataract surgery. Many people have various activities in the evening such as night driving, thus it may be very important measuring the pupil size in the dark in determining the size of the optical zone. We measured pupil size of sixty normal adult candidates in darkness of 0, 20, 60, 100 lux and also in the bright illumination of 200 lux for comparison using near lens camera and slide film. The pupils became larger with younger age and increased darkness. Pupil Size(log mm) = 4.57 - 0.0388 X Age(year) - 0.005 X Luminance(lux). The average pupil size of all age groups were 7.0mm, 6.56mm, 5.97mm, 5.4mm sequencially in darkness and 4.77mm in brightness(200 lux). The average pupil size of 20~30 year old group who are socially active even at night was 7.18mm and 8.4mm in maximum. This has significant meaning clinically. Additionally the size of pupil became larger with increased corneal diameter and the amount of anisocoria tended to be greater with the increase of the darkness.
Adult* ; Anisocoria ; Cataract ; Darkness ; Humans ; Lens Implantation, Intraocular ; Lighting ; Pupil*

No abstract available.
Metallothionein*

Congenital lipoid adrenal hyperplasia, the most severe form of congenital adrenal hyperplasia, is caused by mutations in the steroidogenic acute regulatory protien (StAR). It is characterized by failure of synthesis of all three classes of adrenal steroids and massive accumulation of lipids and cholesterol in the adrenal cortex. The computed tomography (CT) unequivocally delineated massively enlarged adrenal glands of fat-tissue attenuation, enabling early diagnosis and replacement therapy. We report a case of congenital lipoid adrenal hyperplasia, in which CT established that lipoid deposition at the adrenal cortex disappeared after the adrenal hormone replacement therapy.
Adrenal Cortex ; Adrenal Glands ; Adrenal Hyperplasia, Congenital ; Cholesterol ; Early Diagnosis* ; Hormone Replacement Therapy ; Hyperplasia* ; Steroids

Trypsin and DNase digestion technique has become a retinal digestion technique for studying diabetic retinopathy. We tried osmotic digestion method with DNase and compared the quality of preparation of retina and microvascular change with trypsin digestion in normal and diabetic rats. Streptozotocin-induced diabetic rats were sacrificed at 9, 18, 27weeks in 6 rats. Right retinas were digested with 3% trypsin while left wer digested with 0.1% DNase. For control, 18 normal rats were sacrificed at the same time. DNase was superior to trypsin for retinal preparation on the stainability, the degree of separating nonvascular fissue from vascular net, the degree of preservation of vascular net in normal & diabetic rats. In diabetic rats, the number of pericyte decreased significantly with age, which not in normal rats(p=0.0023) We suggest DNase digestion technique as an new alternative for trypsin digestion technique in the study of microvascular change of diabetic retinopathy.
Animals ; Deoxyribonucleases* ; Diabetes Mellitus ; Diabetic Retinopathy ; Digestion* ; Endothelial Cells ; Microvessels* ; Pericytes ; Rats* ; Retina ; Retinaldehyde* ; Trypsin*

PURPOSE: This study was performed to investigate the epidemiological trend of rotavirus acute gastroenteritis (RV-AGE) in children. METHODS: A retrospective review was performed in patients (1 month to 18 years of age) with acute gastroenteritis at KEPCO Medical Center from September 2004 to August 2013. Comparative analyses were performed based on periods: pre-vaccine (2004-2006) and post-vaccine (2008-2012) in all patients; 2004-2006 (period A), 2007-2009 (period B) and 2010-2012 (period C) in patients under 5 years of age. RESULTS: Proportion of RV-AGE decreased from 25.0% (337/1,346) in pre-vaccine period to 20.8% (459/2,210) in post-vaccine period (rate ratio (RR), 0.83 [95% CI, 0.73-0.93]; P=0.0029). The median age of patients with RV-AGE in post-vaccine period (2.6 years) was significantly (P<0.0001) higher than that in pre-vaccine period (1.6 years). In patients hospitalized with AGE, proportion of RV-AGE was significantly reduced in patients 6 to 23 months old (RR, 0.62 [95% CI, 0.51-0.75]; P<0.0001). Significant decline in proportion of RV-AGE was observed in patients under 5 years of age: period A, 26.9% (308/1,144); period B, 22.7% (295/1,299); period C, 20.6% (186/902) (P=0.0007). After the introduction of rotavirus vaccine, a significant decreasing trend of RV-AGE proportion was observed in patients 6 to 11 months old (P=0.0018) and 12 to 23 months old (P=0.0152). CONCLUSION: Decrease in RV-AGE proportion and increase in age of patients with RV-AGE were observed after the introduction of rotavirus vaccine in this single center study. Continued and systematic surveillance is needed to assess the impact of rotavirus vaccine.
Child* ; Epidemiology ; Gastroenteritis* ; Humans ; Retrospective Studies* ; Rotavirus Infections ; Rotavirus Vaccines ; Rotavirus*

PURPOSE: The purpose of this study was to ascertain the effects of preoperative information on postoperative state anxiety, plasma cortisol, and pain for patients under total knee arthroplasty, and to provide generic data with nursing intervention for total knee arthroplasty. METHODS: Data were collected from 34 patients who had total knee arthroplasty from January 3, 2003 to January 15, 2004. An experiment group of 17 patients was provided with pre-operative information prepared by the researchers; a control group of another 17 patients was provided with general information. Data were analyzed through Chi-squared test, t-test, paired t-test and ANCOVA using SPSS WIN 11.0. RESULTS: There was no significant differences between the experiment group and the control group in post operative state anxiety(p=.612). However, there was statistically a significant difference between the above two groups in post operative plasma cortisol(p=.012). There was a statistically significant difference between the above two groups in post operative pain(p=.041). CONCLUSION: According to the results of the study, the authors concluded that the preoperative information for patients under total knee arthroplasty had the effect on the decrease of postoperative plasma cortisol and pain.
Anxiety* ; Arthroplasty* ; Humans ; Hydrocortisone* ; Knee* ; Nursing ; Plasma

No abstract available.
Diabetes Mellitus* ; Tuberculosis*

No abstract available.
Female ; Pregnancy ; Pregnancy, Ectopic*

PURPOSE: We investigated the recommended minimum postoperative follow-up period for the determination of secondary corrective surgery for the consecutive esotropia (ET) and recurrent exotropia (XT) after the first intermittent XT surgery. METHODS: The medical records of 728 patients who underwent surgical treatment for intermittent XT between 2004 and 2009 with a minimum postoperative follow-up of 1 year were retrospectively reviewed. Each patient underwent a detailed sensory and motor examination, including measurements of near and distance stereoacuity, alternating-cover test, and extraocular muscle function testing. Consecutive ET was defined as esodeviation over 15 prism diopter (PD) at distance persisting for more than 6 months after surgery despite medical treatment. Recurrent XT was defined as exodeviation over 15 PD at distance after surgery despite medical treatment. RESULTS: The mean age of the 728 patients at first surgery was 7.5 years (range, 22 months - 30 years). When only the motor outcome was considered, 663 patients (91.1%) had an orthrotropia at the final follow-up and 44 patients (6.0%) among consecutive ET patients and 21 patients (2.9%) who had a recurrent XT underwent secondary surgical correction. Binocularity decreased postoperatively in patients with consecutive ET (p < 0.001), whereas the other patients demonstrated improved stereopsis postoperatively (p = 0.041, 0.021). Patients with consecutive ET showed esodeviation over 10 PD when compared with orthotropia after 2 months postoperatively (p = 0.005). At 6 months postoperatively, 17 (81.0%) of 21 patients with recurrent XT showed orthotropia with an exodeviation over 11 PD after 18 months postoperatively. CONCLUSIONS: The success rate of surgical correction for intermittent XT showed a favorable outcome. However, careful concern for consecutive ET and recurrent XT are required in postoperative follow-up periods. Over-corrected or consecutive ETs need early surgical correction because no further improvement of ocular alignment will occur after 2 months postoperatively and delayed correction can result in poor sensory binocularity. Under-corrected or recurrent XT should be observed for an extended period because of the exotropic drift after surgery, thus requiring periodic long term follow-up for secondary surgery at least for 18 months postoperatively.
Depth Perception ; Esotropia ; Exotropia* ; Follow-Up Studies* ; Humans ; Medical Records ; Retrospective Studies ; Telescopes