• Molecules and Cells
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• v.41 no.7
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• pp.695-702
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• 2018

The inner ear is a complex sensory organ responsible for hearing and balance. Formation of the inner ear is dependent on tight regulation of spatial and temporal expression of genes that direct a series of developmental processes. Recently, epigenetic regulation has emerged as a crucial regulator of the development of various organs. However, what roles higher-order chromatin organization and its regulator molecules play in inner ear development are unclear. CCCTC-binding factor (CTCF) is a highly conserved 11-zinc finger protein that regulates the three-dimensional architecture of chromatin, and is involved in various gene regulation processes. To delineate the role of CTCF in inner ear development, the present study investigated inner ear-specific Ctcf knockout mouse embryos (Pax2-Cre; $Ctcf^{fl/fl}$). The loss of Ctcf resulted in multiple defects of inner ear development and severely compromised otic neurogenesis, which was partly due to a loss of Neurog1 expression. Furthermore, reduced Neurog1 gene expression by CTCF knockdown was found to be associated with changes in histone modification at the gene's promoter, as well as its upstream enhancer. The results of the present study demonstrate that CTCF plays an essential role in otic neurogenesis by modulating histone modification in the Neurog1 locus.

• Archives of Plastic Surgery
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• v.45 no.1
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• pp.62-68
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• 2018

Background Amputation is commonly performed for toe necrosis secondary to peripheral vascular diseases, such as diabetes mellitus. When amputating a necrotic toe, preservation of the bony structure is important for preventing the collapse of adjacent digits into the amputated space. However, in the popular terminal Syme's amputation technique, partial amputation of the distal phalanx could cause increased tension on the wound margin. Herein, we introduce a new way to resect sufficient bony structure while maintaining the normal length, based on a morphological analysis of the toes. Methods Unlike the pulp of the finger in the distal phalanx, the toe has abundant teardrop-shaped pulp tissue. The ratio of the vertical length to the longitudinal length in the distal phalanx was compared between the toes and fingers. Amputation was performed at the proximal interphalangeal joint level. Then, a mobilizable pulp flap was rotated $90^{\circ}$ cephalad to replace the distal soft tissue defect. This modified toe fillet flap was performed in 5 patients. Results The toe pulp was found to have a vertically oriented morphology compared to that of the fingers, enabling length preservation through cephalad rotation. All defects were successfully covered without marginal ischemia. Conclusions While conventional toe fillet flap coverage focuses on the principle of length preservation as the first priority, our modified method takes both wound healing and length into account. The fattiest part of the pulp is advanced to the toe tip, providing a cushioning effect and enough length to substitute for phalangeal bone loss. Our modified method led to satisfactory functional and aesthetic outcomes.

• Korean Journal of Fisheries and Aquatic Sciences
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• v.15 no.4
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• pp.283-290
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• 1982

Sardine and mackerel so called dark muscled fish have been underutilized due to the disadvantages in bloody meat color, high content of fat, and postmortem instability of protein. Recent efforts were made to overcome these defects and develope new types of product such as texturized protein concentrates and dark muscle eliminated minced fish. Approach of this study is based on the rapid dehydration of foamed fish-starch paste by dielectric heating. In process comminuted sardine meat was washed more than three times by soaking and decanting in chilled water and finally centrifuged. The meat was ground in a stone mortar added Ivith adequate amounts of salt, foaming agent, and other ingredients for aid to elasticity and foam stability. The ground meat paste was extruded in finger shape and heated in a microwave oven to give foamed, expanded, and porous solid structure by dehydration. Dielectric onstant $(\varepsilon')$ and dielect.ic loss $(\varepsilon")$ values of sardine meat paste were influenced by wavelength and moisture level. Those values at 100 KHz and 15 MHz were ranged 2.25-9.86; 2.22-4,18 for E' and 0.24-19.24; 0.16-1.20 for E", respectively, at the moisture levels of $4.2-13.8\%$. For a formula for fish-starch paste preparation, addition of $20-30\%$ starch (potato starch) to the weight of fish meat, $2-4\%$ salt, and $5-10\%$ soybean protein was adequate to yield 4-5 folds of expansion in volume when heated. Addition of e99 yolk was of benefit to micronize foam size and better crispness. In order to provide better foaming and dehydration, addition of $0.2-0.5\%$sodium bicarbonate, foaming agent, was proper to result in foam size of 0.5-0.7 mm and foam density of $200-400\;/cm^2$ which gave a good crispness. Heating time was depended upon the moisture level of fish-starch paste. For a finger shaped paste (1.0cm. $D\times10cm.L$) heating for 150-200 sec. in a microwave oven (700W. 2.45GHz) was sufficient to generate foams, expand, and solidify the porous structure of fish-starch paste. When the moisture content was above $55\%$ browning and scorching was deepened due to over-expansion and over-heating whereas the crispness was hardened by insufficient expansion at lower moisture content. In quality evaluation of the product, chemical composition of $30\%$ starch and $3\%$ salt added product was moisture $8.8\%$, lipid $2.4\%$, carbohydrate $46.7\%$, protein $36.1\%$, and ash $6.0\%$. Eleven membered panel test evaluated that fish-starch paste was acceptable in color, crisp-ness, taste, except a trace of fishy odour which could be masked by the addition of spice extracts.

• Journal of the korean academy of Pediatric Dentistry
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• v.30 no.3
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• pp.341-347
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• 2003

Smith-Magenis syndrome (SMS) is a clinically recognizable multiple congenital anomaly and mental retardation syndrome caused by an interstitial deletion of chromosome 17 p11.2. Physical features include short stature, characteristic facial appearance: flattened mid-face, down-turned mouth, prominent and often rosy cheeks; prominent jaw in older children and adults, chronic ear infections, hearing impairment, eye problems, including: strabismus (an eye which turns in or out) and myopia (nearsightedness), hoarse voice, short fingers and toes, heart defects or murmurs, problems related to the urinary system, scoliosis (curvature of the spine), an unusual gait (walking pattern), and decreased sensitivity to pain. Behavioral and developmental characteristics include speech delay and articulation problems, developmental delay, learning disability, mental retardation, hyperactivity, self-injury, including: head banging; hand biting; picking at skin, sores and nails; pulling off finger- and toenails; inserting foreign objects into ears, nose, or other body orifices, explosive outbursts, prolonged tantrums, destructive and aggressive behavior, excitability, arm hugging or hand squeezing when excited. This report is the case of a Korean 3-year-3-month old male with Smith-Magenis syndrome referred from local clinic for the treatment of dental caries. The patient was treated by physical restraint after prophylatic administration of antibiotic(Amoxacillin 50mg/kg).

• Archives of Reconstructive Microsurgery
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• v.11 no.1
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• pp.53-62
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• 2002

Several investigators have reported clinical experience of medial plantar septo-cutaneous flap for reconstruction for soft tissue defect of the hand and digits. Jayme and Hamilton first described the anatomy of superficial branch of medial division of the medial plantar artery used in this flap through cadavaric study in 1997. But, they had a few cases for this flap and there was no anatomic study in Korean. We experienced the reliability of medial plantar septo-cutaneous flap for reconstruction for soft tissue defect of hand and digits through an anatomic study (20 fresh specimens dissected) and clinical application (17 patients). An anatomic study revealed that there were differences in diameter and length of the vessels between Korean and Caucasian. The diameter of vessels in Korean is larger than Caucasian one in each area. Based on this anatomic knowledge, we could harvest this flap safely, and have performed reconstruction on 17 patients with soft tissue defects of hand and digits using a thin, flexible medial plantar septo-cutaneous flap similar to the volar aspect of the hand and digits in anatomical characteristics of the skin and subcutaneous tissue covering. The vessels used for this flap were superficial branches of medial division of the medial plantar artery and vena comitants, or the subcutaneous veins. The mean size of the flap was $2.82cm{\times}4.15cm$. All the flaps survived without significant complications. A medial plantar septo-cutaneous flap possesses several advantages : (1) It is very thin in comparison with other standard free flap; (2) it has two draining venous pathways; (3) it provides a good color and texture match for hand and finger; (4) a good recovery of protective sensation is achievable.

• Journal of Genetic Medicine
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• v.7 no.1
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• pp.37-44
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• 2010

Purpose : Kabuki syndrome is a multiple congenital malformation syndrome with mental retardation. It was named after its characteristic appearance, a face resembling that of an actor in a Kabuki play. To date, six Korean cases of Kabuki syndrome have ever been reported. Here, we present the phenotypic and genetic characteristics of six patients with Kabuki syndrome. Materials and Methods : Between 2003 and 2009, six Korean girls have been diagnosed and followed up as Kabuki syndrome at Center for Genetic Diseases of Ajou University Hospital. Their clinical and laboratory data were collected and analyzed by the retrospective review of medical records. Results : All six patients showed the characteristic facial dysmorphism and developmental delay. Persistent fingertip pads were also found in all patients. Most patients showed postnatal growth retardation (83.3%) and hypotonia (83.3%). Opthalmologic problems were common, particularly for strabismus (83.3%). Congenital heart defects were present in three patients (50%). Skeletal abnormalities including 5th finger shortening (83.3%), clinodactyly (50%), joint hypermobility (50%) and hip dislocation (16.7%) were also observed. There was no patient who had positive family history for Kabuki syndrome. Cytogenetic and molecular cytogenetic analyses including karyotyping and array CGH could not reveal any underlying genetic cause of Kabuki syndrome. Conclusion : Korean patients with Kabuki syndrome showed a broad spectrum of clinical features affecting multiple organ systems. Although clinical manifestations of Kabuki syndrome have been well established, our results failed to detect recurrent chromosome aberrations which could cause Kabuki syndrome. Its natural history and genetic background remains to be further studied for providing appropriate management and genetic counseling.

• Journal of The Korean Ophthalmological Society
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• v.59 no.11
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• pp.1091-1096
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• 2018

Purpose: We report a case of bilateral nonarteritic anterior ischemic optic neuropathy (NAION) following acute angle-closure crisis (AACC). Case summary: A 76-year-old female visited our clinic because of a 1-day history of ocular pain and vision loss in both eyes. The visual acuity was 0.02 in both eyes and her intraocular pressure (IOP) was 52 mmHg in the right eye (RE) and 50 mmHg in the left eye (LE). She had corneal edema and a shallow anterior chamber in both eyes, with 4 mm fixed dilated pupils. After decreasing the IOP with intravenous mannitol, laser iridotomy was performed. However, 2 days later, visual acuity was further reduced to finger counting at 10 cm RE and at 50 cm LE, and her optic disc was swollen. Bilateral NAION following AACC was diagnosed. One month later, visual acuity slightly improved to 0.02 RE and 0.04 LE, and the optic disc edema resolved. A small cup-disc ratio, optic disc pallor, and atrophy were observed. Humphrey visual fields demonstrated superior and inferior altitudial visual field defects in the LE, and almost total scotoma in the RE. Conclusions: AACC can be a predisposing factor for NAION, so the relative afferent pupillary defect, papilledema, and presentation of other risk factors are important clues to a diagnosis of NAION.