• Journal of The Korean Society of Inherited Metabolic disease
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• v.18 no.1
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• pp.30-34
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• 2018

Niemann Pick type C disease (NPC) is an inherited progressive neurodegenerative disorder, due to defects of intracellular lipid trafficking and storage. Hepatosplenomegaly may prevail, while progressive neurodegenerative symptoms such as cerebellar involvement, dystonia, vertical supranuclear ophthalmoplegia, cataplexy, and eventually seizures starting at juvenile or late infantile period may accompany after normal early development. Here we describe a 3-year-old Korean boy with NPC who presented with splenomegaly at age 3. Liver biopsy showed characteristic foamy cell stained by periodic acid-schiff, and molecular analysis for NPC1 identified the compound heterozygous mutations, novel mutation of c.1631G>A (p.Trp544Ter) and c.2662C>T (p. Pro888Ser) as a known mutation. Filipin was strongly stained with unesterified cellular cholesterol in the patient's skin fibroblasts. The patient has received migulstat since age 3 years and his long-term outcome is needed to be observed.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.15 no.1
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• pp.18-24
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• 2015

Glycogen storage disease type Ib (GSD Ib) is one of the rare inherited metabolic disease caused by mutation of SLC37A4 gene. Clinical characteristics include hepatomegaly, hypoglycemia, lactic acidosis, hyperlipidemia and high serum uric acid concentration. The authors analyzed clinical and molecular characteristics of three Korean patients (one male and two females) with GSD Ib by retrospective review of medical records. Two patients were diagnosed in toddler period by hypoglycemia and hepatomegaly. One patient was diagnosed by growth retardation and short stature in puberty. c.412T>C (p.Trp138Arg) (3/6 alleles, 50.0%) was most frequently observed, following by p.Leu348Valfs*53 (1 allele), p.Pro191Leu (1 allele), p.Ala148Val (1 allele) in molecular analysis. Uncooked corn starch and allopurinol was administered. Because all three patients had neutropenia and recurrent infections, G-CSF was administered. Two patients had severe osteoporosis needing calcium supplement. The patient who diagnosed at puberty had relatively poor prognosis demonstrated by having severe infection and complications in liver and kidney.

• KOREAN JOURNAL OF CROP SCIENCE
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• v.41 no.5
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• pp.586-591
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• 1996

We investigated rhizome yield and growth characteristics of wasabi variety, Daioichigo and Daruma cultured by flowing water from Trout nersery in Pyong Chang. Average temp. of air and water during a year was 14.3, 12.3$^{\circ}C$, respectively in PE film house. Wasabi growth was good and healthy in May and October. Rhizome weight of main stem in Daioichigo was 78.78g per plant and 608kg per l0a, while those of the Daruma was 37.1g per plant and 287kg per l0a. Rate of marketable rhizome was 90% in Daioichigo and 37.5% in Darurna. Rhizome weight of main stem in Daioichigo was increased, up to 21% of the control by the remove of floral axis with two times a year.

• Pediatric Infection and Vaccine
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• v.23 no.1
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• pp.72-76
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• 2016

Infantile osteopetrosis is a rare congenital disorder caused by abnormal bone resorption. Patients with osteopetrosis can have severe anemia, thrombocytopenia, hepatosplenomegaly, rickets, visual impairment, and deafness. Cytomegalovirus also can cause a congenital infection with anemia, thrombocytopenia, hepatosplenomegaly, and calcifications in the brain. We report a 38-day-old infant with severe hepatosplenomegaly, thrombocytopenia, hypocalcemia, and growth failure. Real time polymerase chain reaction detected cytomegalovirus in the plasma. Skeletal radiography revealed generalized bone sclerosis. He was diagnosed with osteopetrosis along with cytomegalovirus infection. Only the test for mutation of the CLCN7 gene, representing the most common and heterogeneous form of osteopetrosis, was available, and the result was negative. With supportive care and antiviral treatment, severe thrombocytopenia due to the cytomegalovirus infection almost normalized despite the possible immunosuppression caused by osteopetrosis. We present the first report of an infant who suffered from osteopetrosis and CMV infection which was successfully treated by long term antiviral agent therapy.

• Journal of Oral Medicine and Pain
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• v.38 no.4
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• pp.325-331
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• 2013

Botulinum neurotoxin(BoNT) is a protease exotoxin produced from Clostridium botulinum. It works by blocking the release of acetylcholine from cholinergic nerve endings causing inactivity of muscles or glands. Recently, the therapeutic use of BoNT have expanded to include a wide range of medical and dental conditions. Botulinum neurotoxin type A(BoNT/A) is used off-label in the orofacial region to treat primary and secondary masticatory and facial muscle spasm, severe bruxism, facial tics, orofacial dyskinesias, dystonias, and hypertrophy of the masticatory muscles. Local hematoma, infection, and persistent pain in the injection site are the site-of-injection side effects. Medication-related side effects are adjacent muscle weakness, slurred speech, an alteration in the character of the saliva, and severe headaches. In most cases, these complications are not persistent and bothersome. We reported a case report of a patient who had transient anterior open bite after BoNT/A injection on masseter muscles to treat the refractory myofascial pain.

• Journal of Digital Convergence
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• v.18 no.2
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• pp.439-445
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• 2020

This is a user experience study of factors that should be considered in designing home appliances experience service. Untact services are emerging, but the development of home appliances sector is in early stage. Based on the six factors of Stephen P. Anderson's Creating Pleasurable Interface Model, this study conducted surveys, 1:1 in-depth interviews, and participation observations to measure and analyze user experience. In this study, I compared 4060s and 2030s's user experience in that the untact services raise the digital alienation among middle-senior-aged. As a result, there were significant differences between the two, including the opposite satisfaction in terms of reliable, usable and pleasurable factors. I hope that this study will be of strategic help in designing future home appliance experience services.

• Proceedings of the Korean Society of Applied Pharmacology
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• 1993.04a
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• pp.84-84
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• 1993

취장 외분비 기능 부전은 임상적으로 영양결핍, 발육부전, 지방변동을 유발하나 이의 치료로는 결려된 소화효소 보충 등 보존적 요법만 시행될 뿐 근본적인 치료법은 없는 실정이다. 지난해 과제는 합성 단백분해 억제물질인 Camostat이 취장의 비대와 중식을 일으키며 또한 단백분해 효소분비를 증가시킴을 보고한 바 있다. 이를 토대로 최근 보고된 실험적 취장 외분비기능 부전 모델을 이용하여 camostat의 효과를 검색하고자 하였으며 아울러 이를 CCK효과와 비교하였다. 실험동물로는 몸무게 200 g 안팎의 수컷 Sprague-Dawley계 흰쥐를 사용하였으며 취장기능 부전은 oleic acid (25 $\mu$/100 g bw)를 취관내 주입하여 유발하였다. Camostat은 200 mg/kg씩 위내 투여(i.g.)하였으며 CCK(CCK-8)는 5 $\mu\textrm{g}$/kg씩 하루 2회 피하주사하였고 투여기간은 각각 3, 7, 14 일간으로 하였다. 각 약물 투여 후 취장 외분비 기능과 조직학적 검색을 실시하여 다음과 같온 결과를 얻었다. 1. Oleic acid의 취관내 주입으로 흰쥐의 취장 무게, 조직내 효소단백 함량 및 효소분비량이 현저히 감소되었고 조직학적으로 심한 위축과 섬유화를 관찰할 수 있었으며 이는 주입 후 기간이 지남에 따라 계속 진행하였다. 2. 취장기능 부전 유발 흰쥐에서 camostat 처치로 조직내 효소단백 함량 및 효소분비가 증가 되었으며 이는 14일간 처치군에서 뚜렷하였다. 3. 취장 기능부전 유발 횐쥐에서 camostat 처치는 조직학적으로 기능적인 외분비 조직이 유지되었으며 이는 3일군에서 특히 뚜fut하였다. 4. 취장기능부전 유발 횐쥐에서 CCK 처치효과는 camostat 처치효과와 비슷하였다. 이상의 결과로 보아 oleic acid 주입은 취장기능부전 연구에 유용한 실험모델로 생각되며 합성 단백 분해 효소 억제제인 Camostat은 취장외분비 기능 부전의 진행을 억제하고 어느정도 그 기능을 호전시킬 수 있으며 이는 CCK유리에 기인한다고 생각한다.

• Journal of Plant Biotechnology
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• v.31 no.2
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• pp.139-143
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• 2004

The twin-scale segments of nerine (Nerine bowdenii) were cultured to investigate the influence of NAA, BA and sucrose concentrations on in vitro bulblet formation. The formation of bulblets from twin-scale segments showed a good response both the percentage of bulblet formation and the number of bulblets per explant on MS medium supplemented with 1mg/L NAA and 2 mg/L BA. Formation of bulblet showed the highest efficiency on medium containing 30g/L, and the formation of bulblets was strongly inhibited on medium containing over 90g/L. When the twin-scale segments formed bulblets were subcultured three times to the same medium by 60 day subculture interval, the number of bulblets per explant was 6.5, 7.3 and 8.2 in order of first, second and third. The bulblets over 3mm in diameter were hypertrophied and rooted after transferring to the hormone-free MS medium. The plantlets over 50mm in height were successfully acclimatized in the soil mixed with the same volume of vermiculite and perlite, and the survival rate was over 95％.

• Neonatal Medicine
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• v.17 no.1
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• pp.141-146
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• 2010

Metatropic dysplasia is a rare spondylo epi metaphyseal dysplasia characterized by progressive kyphoscoliosis, short limbs with relatively large hands and feet and limited of motion and enlargement of the large joints. It is diagnosed based on the characteristic clinical and radiological features. Even though benign cases of this disease are frequently reported, metatropic dysplasia can often have a fatal outcome. We describe a sporadic case of the well-delineated lethal metatropic dysplasia.

• Korean Journal of Ichthyology
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• v.12 no.2
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• pp.111-117
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• 2000

There were observed the histomorphological alterations such as chloride cell hyperplasia, branchial lamellar epithelial separation, the increased cellular turnover of chloride cells, glomerular shrinkage and blood congestion in rainbow trout (Oncorhynchus mykiss) during the seawater adaptation. The ultrastructure by scanning electron microscope (SEM) indicated that the gill secondary lamella of rainbow trout exposed to seawater, were characterized by rough convoluted surfaces during the adaptation. There were observed a large number of mitochondria with the elongate and well-developed cristae in chloride cells exposed to seawater by transmission electron microscope (TEM). The presence of two mitochondria- rich cell types is discussed with regard to their possible role in the hypoosmoregulatory changes which occur during seawater-adaptation. Glomerulus shrinkage and blood congestion were occurred higher in nephrons of seawater-adapted fish than those living in freshwater. Our findings demonstrated that rainbow trout tolerated moderately saline environment and the increased body weight living in seawater was relatively higher than that living in freshwater in spite of histopathological changes.