• Childhood Kidney Diseases
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• v.6 no.1
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• pp.123-130
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• 2002

Posttransplant lymphoproliferative disease (PTLD) represents a diverse lymphoproliferative disorder ranging front nonspecific reactive hyperplasia to malignant immunoblastic sarcoma developed in a setting of immunosuppression following organ or cellular transplantation. It is often associated with Epstein-Barr virus (EBV) infection and high dose immunosuppression. PTLD after renal transplantation was reported at first in adult in Korea in 1997. In children there have been several cases of PTLD after liver transplantation but PTLD after renal transplantation has not been reported. This is a case report of PTLD developed 4 months after renal transplantation in a 9-year-old boy. The major clinical manifestations were fever, multiple lymph nodes enlargement and blood-tinged stool. EBV was detected by in-situ hybridization in the enlarged cervical lymph node and the colonic tissue. Histological examination revealed B-cell lineage. Use of ganciclovir and reduction of the immunosuppression level resulted in complete remission of PTLD. This is the first pediatric case report of PTLD following renal transplantation in Korea. (J Korean Soc Pediatr Nephrol 2002 ; 6 : 123-30)

• Journal of the Korean Home Economics Association
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• v.12 no.34
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• pp.679-697
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• 1974

I. Preface A. Establishment. There are many psycological and physiological difficulties that students have made practical application to a building site of 600 pyung and a structure of 65 pyung each sixteen students in the Home Management House, but there exists problem in students apprentice and management of professors. B. Purpose of study This research aim to seek a high degree of efficiency approach in investigating, studying and analyzing factors of the problem in Home Management which have been faced in women colleges in seoul. C. Content of study. This study is aim at finding out the problems including facilites, system, guiding principle, working bottleneck and administrative problem in making practical application of Home Management. II. A plan and method of study A. This subject of statiscal research is 616 women students in senior year from september 27 1970 to October 31 1974. The rate of collection of questionnaire is 100 percent. B. The Method of study is devised through refering documents, interview with students, questionnaire and observation. III. Result of study Of the total of 29 university in seoul, 24 Universities have its Course of Home Economic among which only 12 have Home Management for making practical application. Home Management House for making practical application is located mainly in campus but some of them exceptimally are in outside university. Students practice mainly at civilzed resident, apartment, bangalow so forth, and demention of which occupies approxiamtely 50-600 pyung (land) and 20-70 pyung (Building). A member with 4 or 20 students make practical application from 10 days to 3 months. A member consists of students and assistant teacher, and practice site classifies into living school for Professors living, and another school for Professors and assistant teachers. Answer to a questionnaire. 616 students answer to a questionnaire. 49 Perfect of 616 students like to have a member with 12 students like to have a member with 12 students, 38 percent student a member with 16 students. About the period of making practical About the period of making practical application, 76 percent students like to have two or three week period. 19 percent students think it a short period and 4 percent students think it a long period. There is none who thinks 25 day period day period to be short. About making practical application, 66 percent of 616 students feel uneasy, 40 percent students feel lack of sleep and tired. Students take a pleasure in having a conversation with their friends, inviting their mother, having dinner-party and birthday party. 77 percent students want to invite their father and 67 percent students want to invite their boy-friends. It is hard to have a good personal relation about the great the result of practice, students acknowledge themselves that they have learned about Cautiousness and they are sure of good Home Manager. There are 9 difficult problems during the period. IV. Summary discussion a. Many problems come from having a number of students. b. Students feel tired with their practice if over three weeks. No Matter how good a plan is, it useless if students can't adapt themselves to it. Suggestions 1) it is good to have a member with 5 or 6 students, and it is suitable to have two or three week period, and to have an area of 20 or 30 pyung. 2) it's proposed a profess or to live together in making practical application of Home Management 3) it is proposed a professor to have a sure T.O. and to be treated better.

• Journal of The Korean Dental Society of Anesthesiology
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• v.12 no.4
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• pp.229-233
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• 2012

Hemophilia, the most common of the inherited bleeding disorder, is the result of a deficiency of clotting factor. Since bleeding after dental treatment may cause severe or even fatal complications, people with hemophilia must be given special dental care. We report on the diagnosis and treatment of a 9-year-old boy having severe hemophilia visited our department with the chief complaints of pus discharge on the left lower molar region. In the clinical and radiographic examination, periapical abscess and dental caries were diagnosed. Considering complexity of the treatment and complication in the coagulation, it was decided to carry on the treatment under general anesthesia. Clotting factor IX concentrates were intended to provide 50-70% plasma level. Pulpectomy, resin restoration and Stainless steel crown were given under general anesthesia. Several teeth were extracted and the sockets were packed with Surgicel$^{(R)}$ (Oxidized Regenerated Cellulose, Johnson and Johnson Co. Neuchatel, Switzerland) under general anesthesia. Transpalatal arch and lingual arch were given for maintaining the extracted space before discharged. For people with severe hemophilia, factor replacement is necessary before scaling, surgery or regional block injections. Therefore, if several extractions are needed, dental care under general anesthesia would be effective and efficient management.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.17 no.4
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• pp.257-262
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• 2014

Severe combined immunodeficiency (SCID) is a life-threatening syndrome of recurrent infections and gastro-intestinal alterations due to severe compromise of T cells and B cells. Clinically, most patients present symptoms before the age of 3 months and without intervention SCID usually results in severe infections and death by the age of 2 years. Its association with intestinal anomalies as multiple intestinal atresias (MIA) is rare and worsens the prognosis, resulting lethal. We describe the case of a four year-old boy with SCID-MIA. He presented at birth with meconium peritonitis, multiple ileal atresias and underwent several intestinal resections. A targeted Sanger sequencing revealed a homozygous 4-bp deletion ($c.313{\Delta}TATC$; p.Y105fs) in tetratricopeptide repeat domain 7A (TTC7A). He experienced surgical procedures including resection and stricturoplasty. Despite parenteral nutrition-associated liver disease, the patient is surviving at the time of writing the report. Precocious immune system assessment, scrutiny of TTC7A mutations and prompt surgical procedures are crucial in the management.

• Journal of the Korean Society of Costume
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• v.60 no.6
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• pp.62-73
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• 2010

The purpose of this thesis is to make an effective Korean Hip Hop culture and suggest a new style direction in classifying the types of fashion styles of the Hip Hop musicians and studying their features. They are surveyed in stage costumes, hair styles, make-ups, accessaries, and total fashion styles, and then classified into Normal Hip Hop Style, Casual Hip Hop Style, Sporty Hip Hop Style, Mix & Match Hip Hop Style, and Classical Hip Hop Style. The results of the study are as follows: First, Normal Hip Hop Style is the most general and universal Hip Hop fashion type. They wear clothes in bigger sizes and exaggeratively. And also a cornrow hair style, a tattoo, and a piercing represent marked individualities. Second, Casual Hip Hop Style is more active and practical than Normal Hip Hop Style. Their blue jeans and T-shirts express more simple and polished style. And also they make variable fashion styles and hair styles such as a cornrow hair and a buzz cut. Third, Sporty Hip Hop Style is from B-boy's clothes when they do active and violent break dancing. As NBA basketball games are popular, they wear training clothes, breeches, big T-shirts, and jumpers & caps on embroidered sport team's names. Their hair style is short or natural and also they have caps or hoods to present active mood. Fourth, Mix & Match Hip Hop Style is existed variable elements in one style. The typical type of this style is jacket & blue jean. It is characterized by dress shirts, neck ties, blue jeans, sneakers, beards, and moustaches. Fifth, Classical Hip Hop Style is from African Americans. They try to express assimilated appearance of Anglo-Saxons. They wear black suits, shoes, hats, and sunglasses to represent formality and elegance. In the above results, there are variable fashion styles in one Hip Hop music genre and also fashion style is changed with musical inclination. This thesis is an important guide to produce variable and creative Hip Hop fashion styles in Korean Hip Hop culture.

• Clinical and Experimental Pediatrics
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• v.59 no.2
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• pp.100-103
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• 2016

Patients with hemolytic-uremic syndrome (HUS) can rapidly develop profound anemia as the disease progresses, as a consequence of red blood cell (RBC) hemolysis and inadequate erythropoietin synthesis. Therefore, RBC transfusion should be considered in HUS patients with severe anemia to avoid cardiac or pulmonary complications. Most patients who are Jehovah's Witnesses refuse blood transfusion, even in the face of life-threatening medical conditions due to their religious convictions. These patients require management alternatives to blood transfusions. Erythropoietin is a glycopeptide that enhances endogenous erythropoiesis in the bone marrow. With the availability of recombinant human erythropoietin (rHuEPO), several authors have reported its successful use in patients refusing blood transfusion. However, the optimal dose and duration of treatment with rHuEPO are not established. We report a case of a 2-year-old boy with diarrhea-associated HUS whose family members are Jehovah's Witnesses. He had severe anemia with acute kidney injury. His lowest hemoglobin level was 3.6 g/dL, but his parents refused treatment with packed RBC transfusion due to their religious beliefs. Therefore, we treated him with high-dose rHuEPO (300 IU/kg/day) as well as folic acid, vitamin B12, and intravenous iron. The hemoglobin level increased steadily to 7.4 g/dL after 10 days of treatment and his renal function improved without any complications. To our knowledge, this is the first case of successful rHuEPO treatment in a Jehovah's Witness child with severe anemia due to HUS.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.11 no.2
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• pp.219-222
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• 2008

Crigler-Najjar syndrome is a rare inherited disease associated with unconjugated hyperbilirubinemia. It is inherited via an autosomal recessive pattern and is caused by mutation in one of the five exons of the bilirubin uridine-diphosphoglucuronate glucuronosyltransferase (UGT1A1) gene. The synthesis of inactive isoforms of bilirubin uridine-diphosphoglucuronate glucuronosyltransferase (B-UGT) results in unconjugated hyperbilirubinemia. A 13-year-old boy with jaundice for 4 months was admitted to our hospital. He had unconjugated hyperbilirubinemia with no evidence of infection, hemolysis, or structural abnormalities on abdominal ultrasonography or 99mTc-DISIDA scan. The authors identified a missense mutation of Tyr486Asp in the fifth exon of the UGT1A1 gene and diagnosed the patient with Crigler-Najjar syndrome type II. This is the first reported case of Crigler-Najjar syndrome in a Korean child, and it is also the first reported case of a genetic mutation leading to Crigler-Najjar syndrome in Korea.

• Clinical and Experimental Pediatrics
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• v.58 no.7
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• pp.267-269
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• 2015

Antithymocyte globulin (ATG) is used as an immunosuppressive treatment (IST) to deplete clonal suppressor T cells in patients with severe aplastic anemia (SAA). The depletion of suppressor T cells by ATG may affect the activation of B cells, which results in an increased risk for autoimmune conditions. A 12-year-old boy was diagnosed with idiopathic SAA. As he did not have an human leukocyte antigen-matched sibling, he was treated with rabbit ATG (3.5 mg/kg/day for 5 days) and cyclosporine. Five months later, he became transfusion independent. However, 23 months after IST, he complained of mild hand tremors, sweating, weight loss, palpitations, and goiter. Results of thyroid function tests revealed hyperthyroidism (free thyroxine, 3.42 ng/dL; thyroid stimulating hormone [TSH], <0.01 nIU/mL; triiodothyronine, 3.99 ng/mL). Results of tests for autoantibodies were positive for the antimicrosome antibody and TSH-binding inhibitory immunoglobulin, but negative for the antithyroglobulin antibody and antinuclear antibody. He was treated with methimazole, and his symptoms improved. The patient has been disease free for 39 months after IST and 9 months after methimazole treatment. This case report suggests that although rare, rabbit ATG may have implications in the pathogenesis of autoimmune hyperthyroidism. Our findings suggest that thyroid function tests should be incorporated in the routine follow-up of SAA patients treated with ATG.

• Clinical and Experimental Pediatrics
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• v.57 no.9
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• pp.420-424
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• 2014

The Epstein-Barr virus (EBV) is oncogenic and can transform B cells from a benign to a malignant phenotype. EBV infection is also associated with lymphoid interstitial pneumonia (LIP). Here, we report the case of a 14-year-old boy who was diagnosed with a latent EBV infection and underlying LIP, without any associated immunodeficiency. He had been EBV-seropositive for 8 years. The first clinical presentations were chronic respiratory symptoms and recurrent pneumonia. The symptoms worsened in the following 2 years. The results of in situ hybridization were positive for EBV, which led to a diagnosis of LIP. The diagnosis was confirmed by the results of a thoracoscopic lung biopsy. The EBV titer of the bronchoalveolar lavage specimens obtained after acyclovir treatment was found to be fluctuating. The patient had latent EBV infection for 8 years, until presented at the hospital with intermittent abdominal pain and distension. Physical examination and pelvic computed tomography revealed a large mesenteric mass. A biopsy of the excised mass led to a diagnosis of Burkitt's lymphoma (BL). The patient received combination chemotherapy for 4 months, consisting of vincristine, methotrexate, cyclophosphamide, doxorubicin, and prednisolone. He is now tumor-free, with the LIP under control, and is being followed-up at the outpatient clinic. This is the first report of a Korean case of chronic latent EBV infection that developed into LIP and BL in a nonimmunocompromised child.

• Laboratory Medicine Online
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• v.7 no.2
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• pp.83-87
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• 2017

Pseudohypoparathyroidism (PHP) is a rare disorder caused by genetic and epigenetic aberrations in the GNAS complex locus resulting in impaired expression of stimulatory G protein ($Gs{\alpha}$). PHP type Ib (PHP-Ib) is characterized by hypocalcemia and hyperphosphatemia due to renal resistance to the parathyroid hormone, and is distinguished from PHP-Ia by the absence of osteodystrophic features. An 11-yr-old boy presented with poor oral intake and cramping lower limb pain after physical activity. Laboratory studies revealed hypocalcemia, hyperphosphatemia, and increased parathyroid hormone levels. The GNAS complex locus was evaluated using the methylation-specific multiplex ligation-dependent probe amplification (MS-MLPA) assay. Gain of methylation in the NESP55 domain and loss of methylation in the antisense (AS) transcript, XL, and A/B domains in the maternal allele were observed. Consequently, we present a case of PHP-Ib diagnosed using MS-MLPA.