Hyperimmunoglobulin E syndrome (HIES) is a rare immunodeficiency disease which is characterized by high serum IgE levels, eczema, and recurrent infections. Herein we present the case of a patient with HIES associated with STAT3 gene ($stat3$) mutation. A 16 year-old girl was admitted to our hospital due to hemoptysis caused by pneumonia with bronchiectasis. She had a history of recurrent skin and respiratory tract infections, such as pneumonia caused by MRSA (methicillin-resistant $Staphylococcus$$aureus$) and $Pseudomonas$$aeruginosa$. On physical examination, a broad round shaped nose, oral thrush, and chronic eczematous skin rash over her whole body were found. Laboratory data showed an elevated eosinophil count ($750/{\mu}L$) and total IgE level (5,001 U/mL). The patient's National Institutes of Health (NIH) score for HIES was 44. Direct sequencing of the STAT3 gene revealed that the patient was heterozygous for a missense mutation in the DNA binding domain of the STAT3 protein (c.1144C>T, p. Arg382Trp). HIES should be suspected in patients with recurrent infections and can be confirmed by clinical scoring and genetic analysis.
Kim Dae Yong;Ahn Yong Chan;Lee Jung Il;Nam Do-Hyun;Lim Do Hoon;Lee Jeong Eun;Yeo Inhwan;Huh Seung Jae;Noh Young Joo;Shin Seong Soo;Hong Seung-Chyul;Kim Jong Hyun
Radiation Oncology Journal
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v.19
no.1
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pp.1-9
/
2001
Purpose : To establish the role of stereotactic radiosurgery (SRS) for the treatment of patients with angiographically occult vascular malformation (AOVM). Materials and Methods : Eleven patients (12 lesions) with AOVM were treated with linear accelerator-based SRS between February 1995 and December 1999. A magnetic resonance imaging of each patients showed well-circumscribed vascular lesion with reticulated core of heterogeneous signal intensity and peripheral rim of low signal intensity. SRS were peformed with the median peripheral dose of 16 Gy (range 13~25). A single isocenter was used with median collimator size of 14 mm (range 8~20) diameter. Results : With a median follow-up period of 42 months (range 12~56), rebleeding occurred in 3 AOVMS at 5, 6 and 12 months after SRS but no further bleeding did. Two patients experienced radiation-induced necrosis associated with permanent neurologic deficit and one patient showed transient edema of increased 72 signal intensity. Conclusion : SRS may be effective for the prevention of rebleeding in AOVM located in surgically inaccessible region of the brain. Careful consideration should be needed in the decision of case selection and dose prescription because the incidence of radiation-induced complications is too high to be accepted.
Purpose: In order to enhance the quality of IMRT as employed in Korea, we developed a remote monitoring system. The feasibility of the system was evaluated by conducting a pilot study. Materials and Methods: The remote monitoring system consisted of a head and neck phantom and a user manual. The phantom contains a target and three OARs (organs at risk) that can be detected on CT images. TLD capsules were inserted at the center of the target and at the OARs. Two film slits for GafchromicEBT film were located on the axial and saggital planes. The user manual contained an IMRT planning guide and instructions for IMRT planning and the delivery process. After the manual and phantom were sent to four institutions, IMRT was planed and delivered. Predicted doses were compared with measured doses. Dose distribution along the two straight lines that intersected at the center of the axial film was measured and compared with the profiles predicted by the plan. Results: The measurements at the target agreed with the predicted dose within a 3% deviation. Doses at the OARs that represented the thyroid glands showed larger deviations (minimum 3.3% and maximum 19.8%). The deviation at OARs that represented the spiral cord was $0.7{\sim}1.4%$. The percentage of dose distributions that showed more than a 5% of deviation on the lines was $7{\sim}27%$ and $7{\sim}14%$ along the horizontal and vertical lines, respectively. Conculsion: Remote monitoring of IMRT using the developed system was feasible. With remote monitoring, the deviation at the target is expected to be small while the deviation at the OARs can be very large. Therefore, a method that is able to investigate the cause of a large deviation needs to be developed. In addition, a more clinically relevant measure for the two-dimensional dose comparison and pass/fail criteria need to be further developed.
Lim, Hong Hee;Ahn, Byung Moon;Kim, Eun Ryoung;Choi, Sug Ho;Moon, Young Ho;Kim, Il Soo
Pediatric Infection and Vaccine
/
v.5
no.1
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pp.79-87
/
1998
Purpose : The P1 protein of Mycoplasma pneumoniae mediates the attachment of the pathogen to its host cell and elicits a strong humoral immune response during infection with this organism. Mycoplasma pneumoniae strains can be classified into two groups(I and II) by PCR method of P1 cytadhesin gene. In this study, we evaluated the prevalence, epidemiological and clinical characteristics of each group. Methods : From 155 patients with Mycoplasma pneumoniae, who admitted to the Department of Pediatrics, Sung-Ae and Kwangmyung Sung-Ae Hospital between November 1996 and October 1997, we collected their throat swabs or nasopharyngeal aspirates for DNA extraction and serum for indirect hemagglutination test of Mycoplasma pneumoniae. The group specific PCR amplification were performed using specific oligonucleotide primers designed for P1 gene genotyping. Results : Group I(137 patients, 88.4%) occurred frequently than group II(18 patients, 11.6%). In both group, the most prevalent season was winter in 1996(Nov. to Dec.) and fall in 1997(Aug. to Oct.) The prevalent age was four to six years old. The number of male was more than female in both group; Group 1(1.2:1), Goup 2(1.6:1). No significant relationship were found between two groups in duration of fever and hospital days(P>0.05). The rate of high antibody titers(>1:5120) was lower in group I(6/137, 4.4%) than group II(2/18, 11.1%). Conclusion : Group I was much more prevalent than group II during 1996~1997 in Korea. There was no difference between two groups in epidemiological and clinical parameters except the rate of high antibody titers. Further follow-up survey will be needed for the epidemiologic and clinical studies of Mycoplasma pneumoniae in Korea.
Purpose: Recently multi-modal imaging system has become widely adopted in molecular imaging. We tried to fabricate animal-specific positioning molds for PET/MR fusion imaging using easily available molding clay and rapid foam. The animal-specific positioning molds provide immobilization and reproducible positioning of small animal. Herein, we have compared fiber-based molding clay with rapid foam in fabricating the molds of experimental animal. Materials and Methods: The round bottomed-acrylic frame, which fitted into microPET gantry, was prepared at first. The experimental mice was anesthetized and placed on the mold for positioning. Rapid foam and fiber-based clay were used to fabricate the mold. In case of both rapid foam and the clay, the experimental animal needs to be pushed down smoothly into the mold for positioning. However, after the mouse was removed, the fabricated clay needed to be dried completely at $60^{\circ}C$ in oven overnight for hardening. Four sealed pipet tips containing $[^{18}F]FDG$ solution were used as fiduciary markers. After injection of $[^{18}F]FDG$ via tail vein, microPET scanning was performed. Successively, MRI scanning was followed in the same animal. Results: Animal-specific positioning molds were fabricated using rapid foam and fiber-based molding clay for multimodality imaging. Functional and anatomical images were obtained with microPET and MRI, respectively. The fused PET/MR images were obtained using freely available AMIDE program. Conclusion: Animal-specific molds were successfully prepared using easily available rapid foam, molding clay and disposable pipet tips. Thanks to animal-specific molds, fusion images of PET and MR were co-registered with negligible misalignment.
This study was performed to investigate the effect of rearing period(45 and 70 days) on the changes of the yield, physical trait and the composition of fatty acids. The average weight of 100 duck meat at 45 days post hatch was 2.2kg. The yield of meats from prime cuts of 2.1kg, which are the most consumed today, was composed to whole legs($14.1\%$), wings($8.7\%$) and breast(12.5\%$). The ratio of whole legs and breast was similar, In the secondary cuts, the meats was consisted in drumstick($12.5\%$), thigh($7.3\%$), breast($11.7\%$), tender lion($0.8\%$), drum matte($4.9\%$) and wing($2.6\%$). Especially, the drumstick was twice higher than the wing. The content of oleic acid was 50.87 to $51.32\%$ and 49.84 to $50.03\%$ in the breasts and leg, respectively. In the breast, meat at 75 days the oleic acid was slightly increased DHA was not detected in the breast meat, However, it was $0.13\%$ in the leg. The $a^{*}$ value in meat color, ranged was 16.67 to 17.92 in the breast and 15.81 to 17.15 in the leg. Statistically, there was no significantly difference between the breast and leg. The cooking loss of the breast and leg was $26.37\%\;and\;30.32\%$, respectively. The shear force value was $2.20\;kg/0.5inch^2$ at 45 and $2.84\;kg/0.5inch^2$ at 75 days post hatchet respectively. Therefore, the raring period was effect on the shear force value, but not on the cooking loss and meat color.
The aim of this study was to determine the chemical traits of duck meats with different rearing periods(45 70 days). The content of protein was 21.3 and 19.61% in the breast and leg at 70 days post hatched, respectively. Group at 70 days showed high protein composition than those of 45 days group. The content of fat was slightly increased with the rearing periods. The composition of Ca, mineral compound, was not showed the difference between leg and breast meat. However, it was increased with the rearing periods. The composition of Fe was not significantly different in the breast meat. However, there was a significant increase in the leg (p<0.05). It was compared 14.6 ppm at 45 days group to 27.5 ppm at 70 days group. The content of methionine was significantly increased in the breasts due to the increasing rearing periods (p<0.05), but not in leg meat. There was no significant difference between parts, but glutamic acid content was significantly decreased along with the increasing rearing periods (p<0.05). The content of collagen at 70 days(0.77 g/100 g) was higher than those of 45 days(0.65 g/100 g). It was affected by the rearing periods In the wing-removed skit, it was 0.15 g increases at 70 rearing period group. The cholesterol content was significantly increased in the leg but not in the breast. With the rearing periods, it was significantly increased in the leg (p<0.05).
Shin Dong Oh;Park Sung Yong;Ji Young Hoon;Lee Chang Geon;Suh Tae Suk;Kwon Soo IL;Ahn Hee Kyung;Kang Jin Oh;Hong Seong Eon
Radiation Oncology Journal
/
v.20
no.4
/
pp.381-390
/
2002
Purpose : To develop a dose calibration program for the IAEA TRS-277 and AAPM TG-21, based on the air kerma calibration factor (or the cavity-gas calibration factor), as well as for the IAEA TRS-398 and the AAPM TG-51, based on the absorbed dose to water calibration factor, so as to avoid the unwanted error associated with these calculation procedures. Materials and Methods : Currently, the most widely used dosimetry Protocols of high energy photon beams are the air kerma calibration factor based on the IAEA TRS-277 and the AAPM TG-21. However, this has somewhat complex formalism and limitations for the improvement of the accuracy due to uncertainties of the physical quantities. Recently, the IAEA and the AAPM published the absorbed dose to water calibration factor based, on the IAEA TRS-398 and the AAPM TG-51. The formalism and physical parameters were strictly applied to these four dose calibration programs. The tables and graphs of physical data and the information for ion chambers were numericalized for their incorporation into a database. These programs were developed user to be friendly, with the Visual $C^{++}$ language for their ease of use in a Windows environment according to the recommendation of each protocols. Results : The dose calibration programs for the high energy photon beams, developed for the four protocols, allow the input of informations about a dosimetry system, the characteristics of the beam quality, the measurement conditions and dosimetry results, to enable the minimization of any inter-user variations and errors, during the calculation procedure. Also, it was possible to compare the absorbed dose to water data of the four different protocols at a single reference points. Conclusion : Since this program expressed information in numerical and data-based forms for the physical parameter tables, graphs and of the ion chambers, the error associated with the procedures and different user could be solved. It was possible to analyze and compare the major difference for each dosimetry protocol, since the program was designed to be user friendly and to accurately calculate the correction factors and absorbed dose. It is expected that accurate dose calculations in high energy photon beams can be made by the users for selecting and performing the appropriate dosimetry protocol.
Jeon, Hye Lyun;Yi, Jung-Sun;Kim, Tae Sung;Oh, Youkyung;Lee, Hye Jeong;Lee, Minseong;Bang, Jin Seok;Ko, Kinarm;Ahn, Il Young;Ko, Kyungyuk;Kim, Joohwan;Park, Hye-Kyung;Lee, Jong Kwon;Sohn, Soo Jung
Toxicological Research
/
v.33
no.2
/
pp.107-118
/
2017
Although alternative test methods based on the 3Rs (Replacement, Reduction, Refinement) are being developed to replace animal testing in reproductive and developmental toxicology, they are still in an early stage. Consequently, we aimed to develop alternative test methods in male animals using mouse spermatogonial stem cells (mSSCs). Here, we modified the OECD TG 489 and optimized the in vitro comet assay in our previous study. This study aimed to verify the validity of in vitro tests involving mSSCs by comparing their results with those of in vivo tests using C57BL/6 mice by gavage. We selected hydroxyurea (HU), which is known to chemically induce male reproductive toxicity. The 50% inhibitory concentration ($IC_{50}$) value of HU was 0.9 mM, as determined by the MTT assay. In the in vitro comet assay, % tail DNA and Olive tail moment (OTM) after HU administration increased significantly, compared to the control. Annexin V, PI staining and TUNEL assays showed that HU caused apoptosis in mSSCs. In order to compare in vitro tests with in vivo tests, the same substances were administered to male C57BL/6 mice. Reproductive toxicity was observed at 25, 50, 100, and 200 mg/kg/day as measured by clinical measures of reduction in sperm motility and testicular weight. The comet assay, DCFH-DA assay, H&E staining, and TUNEL assay were also performed. The results of the test with C57BL/6 mice were similar to those with mSSCs for HU treatment. Finally, linear regression analysis showed a strong positive correlation between results of in vitro tests and those of in vivo. In conclusion, the present study is the first to demonstrate the effect of HU-induced DNA damage, ROS formation, and apoptosis in mSSCs. Further, the results of the current study suggest that mSSCs could be a useful model to predict male reproductive toxicity.
Purpose: The aim of this study is to investigate the current use of dexamethasone rescue therapy (DRT) for bronchopulmonary dysplasia (BPD). Methods: This is a retrospective study of 251 BPD patients managed in the neonatal intensive care units at Seoul National University Childrens Hospital and Seoul National University Bundang Hospital between March 2004 and August 2008. The demographic data and clinical characteristics of the mothers and infants were analyzed. The infants were compared based on DRT responsiveness. The DRT complications were investigated. Results: Ninety-three patients (37.1%) were classified with severe BPD, DRT was only given to patients with severe BPD. Dexamethasone was administered to 24 patients (9.6%) whose respiratory status had precluded extubation, which indicated that conventional BPD management had failed. Fourteen patients (58.3%) who received DRT were responsive. DRT non-responders required more oxygenation and more complicated with pulmonary arterial hypertension (PAH). Responder had shorter length's of hospitalization and lower mortality rates. High dose dexamethasone was no more effective in weaning neonates from the ventilatior than low dose dexamethasone. Sepsis was the most common complication of DRT. Conclusion: DRT is a valuable treatment for severe BPD ahead of PAH development. DRT should not be performed in BPD patients with PAH due to the possibility of complications.
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