Kim, Khi Joo;Kim, Ki Hwan;Lee, Taek Jin;Chun, Jin-Kyong;Lyu, Chuhl Joo;Kim, Dong Soo
Clinical and Experimental Pediatrics
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v.51
no.3
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pp.299-306
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2008
Purpose : Hemophagocytic lymphohistiocytosis (HLH) is a rare but fatal disorder characterized by fever, splenomegaly, pancytopenia, and hemophagocytosis in the bone marrow and other tissues. In this study, we investigated the clinical manifestations and prognostic factors in patients with HLH. Methods : We retrospectively analyzed the data from 29 patients who were diagnosed whit HLH in the Severance Children's Hospital from Jan. 1996 to Feb. 2007. Results : The median age at diagnosis was 3.8 years (range 0.1-12.2). The ratio of male to female patients was 1.1:1. The 5-year overall survival rate was 55.2% with a median follow-up duration of 32 months. In a multivariate analysis, the duration of fever before admission (survival vs. non-survival, 6.5 days vs. 14 days, P=0.010), the interval from the day of fever onset to the day of initiation of etoposide (survival vs. non-survival, 10 days vs. 35 days, P=0.002) and the presence of neurologic symptoms (survival vs. non-survival, 1 case vs. 7 cases, P=0.010) were independent, poor prognostic factors of HLH. EBV infection, gender, and the level of serum ferritin had no relations to the poor prognosis of the disease. Conclusion : This study showed that the presence of neurologic symptoms and a longer duration of fever were related to a poor prognosis. Therefore, if a patient develops neurologic symptoms and the duration of fever is prolonged, a prompt diagnostic approach and aggressive treatment for HLH are necessary.
Purpose : The mucopolysaccharidoses (MPSs) are a heterogeneous group of lysosomal storage disorders. They are caused by a deficiency of the enzymes involved in the degradation of glycosaminoglycans. Early recognition is important because recombinant enzyme replacement therapy is now available for MPS. We studied the clinical characteristics of 80 MPS children with the object of determining the epidemiological, clinical and radiological features in Korean MPS children. Methods : Diagnosis of MPS was confirmed by skin fibroblast enzyme analysis in 80 patients between February 1995 and December 2004. Charts were retrospectively reviewed for clinical and radiological findings, as well as for intelligence and speech evaluations. Results : Hunter syndrome (MPS type II) was the most prevalent type, appearing in 51/80 cases (64 %), followed by Sanfilippo syndrome (MPS III-18%), Hurler syndrome (MPS I-15%), and Morquio syndrome (MPS IV-4%). The average age at diagnosis was 5.5 years (range 1 to 20), and the male-to-female ratio was 4.7 : 1. Typical radiographic changes were observed in 45/54 cases (83%). Mitral regurgitation was the most common cardiac defect. Moderate to profound mental retardation and hearing loss were present in 14/35 cases (56%) and 33/38 cases (82%), respectively. Four MPS II patients had bone marrow transplantation, with mixed outcomes. Five MPS I patients are currently on enzyme replacement therapy. Conclusion : Our study showed a high proportion of MPS II cases (64%), which may represent population variability. By studying the clinical features of these patients, we hope to alert pediatricians of the warning signs of MPS.
The present study argues that film director Lee Ae-rim animation works depict the world of the grotesque and not only are important sociocultural phenomena but also hold the significance of humor and subversion. The grotesque exhibits the intriguing characteristics of expressing a perspective critical of the existing society through the sensibilities of minorities and is used broadly as a term not only in the aesthetic sense but also designating sociocultural phenomena. Although discussed separately in terms of Mikhail Bakhtin's carnival grotesque and Mary Russo's uncanny grotesque, the grotesque fundamentally rejects existing order and conventions and is externalized through unique expressions, thus opening up a rich possibility for rejection, humor, satire, transformation, and deconstruction of and regarding the authority of the mainstream. Although they constitute a fictional medium, animation films are social products as well so that they are affected by society, culture, and history and stand as important texts that must be interpreted in terms of the relationships between humans' instinctive desires and society and between the overall culture and artistic media. However, the rarity of grotesque portrayals in South Korean animation films also proves that it is a society where even problems that are in themselves sensitive must be manifested ingeniously on a conventional level. South Korean society has a unique history of colonialism and national division and is simultaneously in the unique situation of a society that has undergone growth at a nearly unprecedented rate. Consequently, the society exhibits closed yet dynamic particularity where everyday tension and rigidity, wariness of others and extreme competition are intertwined in a complex manner. Intensively analyzed in the present discussions, director Lee's animation films and are characterized mainly by grotesque images, nonlinear narratives, and vivid depictions. In such a context, these works not only are artistic products of South Korean society but also rejections of a rigid society and share the significance of the aesthetics of the carnival grotesque, which consists of subversive expressions directed at a new world.
This study was designed to analysis the displacement and stress distribution of individual tooth by orthodontic force during distal on masse movement of the maxillary dentition. In this study, three dimensional finite element analysis was used. Author made the finite element model of maxillary teeth, periodontal ligament, alveolar bone and bracket with anatomic and physiologic characteristics on computer. Author analysed and evaluated the displacement and stress distribution of individual tooth when extraoral force, Class II intermaxillary elastics, ideal arch wire, MEAW and tip back bend were used for distal on masse movement of the maxillary dentition. These analyses were also applied in the case of the maxillary second molar were not extracted. Author compared the results of the cases which maxillary second molar were extracted or not. The results were expressed quantitatively and visually. Author obtained following results, 1. When anterior headgear was applied, the posterior translation, posterior tipping, and vertical displacement of teeth were produced more in the anterior segment of the dentition. 2. When Class II intermaxillary elastics were applied in the ideal arch wire, the teeth displacement were usually produced in the anterior segment. But when tip back bend were added in the ideal arch wire, the orthodontic force produced by elastics were transmitted to the posterior segment. As increasing the tip back bend, posterior translation and lingual tipping of anterior teeth were decreased, posterior translation and tipping displacement of posterior teeth were increased, and extrusion of anterior teeth by Class II elastics were decreased 3. When MDAW and Class II elastics were applied, the teeth movement were sir flu with the case of ideal arch wire and Class II elastics, but more small and uniform teeth displacement were produced Compared with the ideal arch wire, posterior tipping of the posterior segment were more produced than lingual tipping displacement of the anterior segment. 4. When the maxillary second molar without orthodontic appliance existed, the displacement of maxillary first molar were decreased.
Choi, Yoon Jin;Kim, Shin Mi;Sim, Eun Jung;Cho, Do Jun;Kim, Dug Ha;Min, Ki Sik;Yoo, Ki Yang
Clinical and Experimental Pediatrics
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v.50
no.5
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pp.436-442
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2007
Purpose : To promote awareness and efforts by pediatricians to identity and prevent child abuse by investigation of characteristics of victim and types of injury caused by abuse. Methods : A retrospective study was performed with 20 patients who had been diagnosed or suspected as child abuse at Hallym University Hospital from January 1999 to December 2005. The medical records, radiologic documents, and social worker's notes were reviewed to investigate age, sex, visiting time, form of abuse, perpetrator, risk factor, and type of injury. Results : The mean age of the subjects was 2.8 years. Fourteen patients were between 0-1 years old, 2 patients between 1-6 years old, 3 patients between 7-12 years old, and 1 case over 13 years old. The ratio of male to female was 1:1. The majority of these patients (70%) visited via emergency department. Eight five percent of these patients reported with physical abuse, 5% psychological abuse, 5% sexual abuse, and 5% neglect respectively. The suspected perpetrator was the biological father in six cases, the biological mother in three cases, the stepmother in two cases, caregiver in one case, relatives in one case and "unknown" in six cases. Bruise and hematoma (80%) were the most common physical findings. Skull fractures were diagnosed in six cases, long bone fractures in two cases, hemoperitoneum in two cases, subdural hemorrhage in 10 cases, epidural hemorrhages in two cases, subarachnoidal hemorrhages in two cases, and retinal hemorrhages in five cases respectively. Seventeen cases required hospitalization and surgical operations performed were in nine cases. Four patients died and three patients had sequalae such as developmental delay and quadriplegia. Conclusion : Child abuse results in high mortality and morbidity in victims. Therefore early recognition and prevention is very important. Pediatricians should always suspect the possibilities of abuse in cases of fracture, intracranial hemorrhage, abdominal injury, or even any injury to the body. We recommend that the clinical investigation of suspicious children should include a full multidisciplinary social assessment, a skeletal survey and CT or MRI.
Serous otitis media (SOM) is one of the most common otologic diseases which was first discribed by Politzer in 1869. Currentely, among many methods introduced to treat SOM, ventilation tube insertion is considered to be the most popular and standard method being used. However due to complication of it, there remains many disputable various problems. In order to review the clinical aspect of SOM and search for the effects and safety of ventilation tube, we studied 97 SOM patients, who had performed V-tube from Jan. 1981 to Feb. 1982 at ENT dept. of Severance Hospital. Analizing the clinical symptoms, age distribution, associated disease, otoscopic findings of ear drum, degree of hearing loss, hearing gain after insertion of V-tube and complications, following results were obtained. 1) As a subjective symptom, among 29 cases all patients had hearing loss, 19 cases (65.6%) had tinnitus, 12 cases (44.4%) had autophony and 10 cases (34.5%) of the patients had sensation of ear fullness. 2) In age distribution, 6 to 10 year old group was most numerous up to 42 cases (43.3%) and 20 years and over was 23 cases (23.5%) 3) In adult group SOM tend to be involved unilaterally whereas young child group had tendency involving bilaterally. 4) 49 cases (50.05%) were associated with tonsillitis and adenoid vegetation, 15 cases (15.5%) were associated with sinusitis, 4 cases (4.1%) had nasal allergy. 5) In preoperative otoscopic findings, 62 ears (47.7%) had retraction, 37 ears (23.6%) had bulging, 34 ears (21.7%) had color change, and 29 ears (19.7%) had no significant findings. 6) In characteristics of middle ear fluid, child group was tend to have mucinous content (84.2%) while adult group had serous content (62.5%). 7) Average preoperative air-bone gap of pure tone was 25.3 dB. 8) 24 ears (72.7%) had over 10 dB of postoperative hearing gain and average hearing gain was 17.2 dB. 9) There were 44 ears (28.1%) of complications. Among them 37 ears (23.6%) had infection, 3 ears had atelectasis, 2 ears had granulation tissue, 2 ears had permanent perforations. 10) Among 37 ears suffered from post-op. infection, 19 ears (51.4%) had initial infection just after insertion of ventilation tube, 18 ears (48.6%) were infected during the course of post-ventilation tube. Of 37 infected ears, 26 ears (70.3%) responded to conservative care, while 11 ears (29.7%) was cured after removal of ventilation tube.
The heavy metal accumulations of avian eggshells were studied in order to test a feral pigeon (Columba livia)'s eggshell as an indicator for the environmental monitoring of pollutants. The reviews on the eggs of the different 19 avian species showed that it is the eggshell rather than the egg content that can better reflect the heavy metals in the environment; in most cases the CVs (coefficients of variations) of the heavy metal concentrations in the eggshells were higher than those in the egg contents. This can indicate that the heavy metal accumulations are homeostatically controlled in the egg contents, but the accumulations in the eggshells are varied according to the environmental conditions. To test the reviews, the feral pigeon eggs from the two different sites, one representing urban and the other rural environment, were analyzed for lead (Pb) and cadmium (Cd). The result showed that the eggshells of the urban pigeons (Hangang) had the higher metal concentrations than those of the rural pigeons (Hampyeong). The same difference can also be found in the internal organs (liver, bone) and blood. However, the analyses of the egg contents between the two sites did not reveal the differences. In other words, the result suggests that the feral pigeons, like the other avian species, are able to control the heavy metals into the egg contents homeostatically. Therefore, it is more useful to use the feral pigeon eggshell rather than the egg content in case of monitoring heavy metals in different habitats.
Purpose: Hemophagocytic syndrome (HPS) is characterized by persistent high fever, hepatosplenomegaly, cytopenias, hypertriglyceridemia, and/or hypofibrinogenemia. Hepatic manifestations including overt hepatic failure and fulminant hepatitis are common in HPS. Liver transplantation (LT) should be considered in a case of fulminant hepatitis by other than HPS, but LT is contraindicated and complete cure is possible by chemotherapy in HPS. Therefore, we conducted this study to define the characteristics of HPS presenting as severe acute hepatitis. Methods: Among the total of 23 patients diagnosed as HPS by bone marrow examination between 1994 and 2005 in Asan Medical Center, 11 cases presented as severe acute hepatitis were enrolled in this study. We analyzed the clinical features, laboratory findings and outcome retrospectively. Results: Seven (64%) of the 11 children with HPS and hepatitis were referred to pediatric gastroenterologist at first. The mean age of onset was 50 months. There was no case with family history of primary HPS. Epstein-Barr virus was positive in 4, and herpes Simplex virus was positive simultaneously in 1 case. As the presenting symptoms and signs, fever was present in 10, hepatosplenomagaly was noted in all and jaundice in 10. Anemia was observed in 10, thrombocytopenia in 10, leukopenia in 8, hypertriglyceridemia in 9, hypofibrinogenemia in 8 and hyperferritinemia in 7 cases, respectively. Nine children received chemotherapy including etopside. The overall mortality rate was 72% (8/11). Conclusion: HPS, which needs chemotherapy, should be considered as a cause of severe acute hepatitis especially when accompanied with prolonged high fever and cytopenias.
Seo, Ji-Young;Kim, Curie;Lee, Hee-Woo;Ahn, Young-Min
Clinical and Experimental Pediatrics
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v.51
no.8
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pp.812-819
/
2008
Purpose : Vitamin D plays a key role in bone mineralization of the skeleton and vitamin D deficiency can lead to rickets. It is well known that vitamin D deficiency is common in breast fed infants. Of these patients, clinically, some have no signs of rickets, but laboratory and radiographic findings are diagnostic for vitamin D deficiency rickets (subclinical vitamin D deficiency rickets). The purpose of this study is to clarify current causes and ways to prevent this disease. Methods : We reviewed the clinical and laboratory characteristics of children who were incidentally diagnosed as subclinical rickets during treatment of other disease such as pneumonia, gastroenteritis, urinary tract infection at Eulji Hospital, Seoul, Korea from March, 2003 to July 2007. Results : Eight patients (six boys and two girls) were diagnosed with subclinical vitamin D deficiency rickets. The mean age of the patients was $12.6{\pm}5.8months$, and they were diagnosed from January to July. The associated diseases were pneumonia, urinary tract infection, acute gastroenteritis, and iron deficiency anemia. All patients were breast-fed. Two showed growth failure. The mean serum alkaline phosphatase was $1995.8{\pm}739.5IU/L$, the mean calcium count was $9.5{\pm}0.6mg/dL$, and the mean phosphorus content was $3.6{\pm}1.5mg/dL$. The mean intact parathyroid hormone was $214.8{\pm}155.9pg/mL$ (reference range, 9-65), the mean 1,25-dihydroxyvitamin D was $82.4{\pm}49.3pg/mL$ (reference range, 2070), and the mean 25-hydroxyvitamin D was $29.6{\pm}10.6ng/mL$ (reference range, 1030). A radiographic examination showed cupping, fraying, and flaring of metaphyses in all patients. Six patients were administered calcitriol (400 IU/day) for three months. A consequent radiographic and laboratory examination showed improvement. The first two patients were initially diagnosed with metaphyseal dysplasia, without the detection of vitamin D deficiency and they spontaneously improved without vitamin D supplements. However, two years later, they showed mild scoliosis and metaphyseal dysplasia, respectively. Conclusion : Breast-feeding without supplementation involves high risk of vitamin D deficiency. Some infants may also develop rickets; therefore, such groups should be considered for vitamin D supplementation.
Jo, Jung-Hwan;Park, Min-Woo;Kim, Young-Ku;Lee, Jeong-Yun
Journal of Oral Medicine and Pain
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v.36
no.1
/
pp.53-63
/
2011
Osteoarthritis (OA), the most common form of arthritis, is a result of both mechanical and biological events that destabilize the normal coupling of degradation and synthesis of articular cartilage chondrocytes and extracelluar matrix, and subchondral bone. Although it is likely that the molecular basis of pathogenesis is similar to that of other joints, additional study of the temporomandibular joint (TMJ) is required due to its unique structure and function. This study was carried out to evaluate the epidemiologic characteristics of TMJ osteoarthritis. The purpose of this study was to investigate the prevalence of TMJ OA in Patients with temporomandibular disorders (1405 men and 2922 women whose mean age was $30.2\; {\pm}\; 15.4$ and $33.1\;{\pm}\;15.2$ years, respectively) who had visited the TMJ and Orofacial Pain Clinic of Seoul National University Dental Hospital in 2007. Orthopantomograms, TMJ tomograms and transcranial radiographs were used to evaluate degenerative change of the mandibular condyle. The obtained results were as follows: 1. Degenerative change of the mandibular condyle was observed in 883 (20.4%) of 4327 subjects. The prevalence was significantly higher in women (706 patients, 24.1%) than in men (177 patients, 12.6%), and this significant difference between genders was observed in all age groups. 2. The prevalence of degenerative change of the mandibular condyle in TMD patients showed a gentle increase along with age. Such increase was statistically significant in women (P < 0.001), but not in men. 3. Sclerosis was observed the most frequently in all age groups and the mean age of the patients with osteophyte was the highest among four types of degenerative change. 4. Although men showed degenerative change in the left side more often and women showed degenerative change more frequently in both sides, the difference of distribution in sides between genders was not significant. In conclusion, the prevalence of degenerative change of the mandibular condyle in TMD patients is higher in women than in men, and increases steadily with aging, but not as dramatically as in other joints that show a steep increase in prevalence around the age of 45 years. It can be suggested that the epidemiologic characteristic of OA of the TMJ differs from those of other joints, and that a more extensive study based on the general population is necessary.
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