• Journal of the korean academy of Pediatric Dentistry
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• v.34 no.3
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• pp.468-472
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• 2007

The Wiskott-Aldrich Syndrome (WAS) is an inherited immunodeficiency caused by a variety of mutations in the gene encoding the WAS protein (WASp). First described in 1937 by Wiskott, the incidence of WAS has so far been estimated at 4 in 106 live births. The Wiskott-Aldrich Syndrome is an X-linked condition characterized by 1) an increased tendency to bleed caused by a reduced number of platelets, 2) recurrent bacterial, viral and fungal infections, and 3) eczema of the skin. The purpose of this report is to present cases highlighting the clinical features of the syndrome and the required considerations in the treatment of patients. The report consists of two particular cases: a 2-year-11-month-old boy seen for a routine oral examination prior to his bone marrow transplantation and a 2-year-6-month-old boy with herpes gingivostomatitis and teeth discoloration.

• Development and Reproduction
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• v.15 no.4
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• pp.281-289
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• 2011

The objective of this study was to investigate the effective cryoprotectants for the cryopreservation of porcine mesenechymal stem cells (pMSCs). In order to understand the effectiveness of various cryoprotectants on pMSCs, we studied the most commonly used cryoprotectants; dimethyl sulfoxide (DMSO), ethylene glycol (EG), DMSO and EG. pMSCs were isolated from bone marrow matrix of piglet (2 month) and characterized by alkaline phopshatase (AP) activity, colony forming, and differentiation to adipocyte. In slow cooling cryopreservation, the pMSCs were exposed to cell medium containing Dulbecco's Modified Eagle's Medium (DMEM) supplemented with 10% DMSO, 1.5M EG and 5% DMSO/0.75M EG, respectively, and freezed to $-1^{\circ}C$/min from $25^{\circ}C$ up to $-80^{\circ}C$ in a cryo-container. The proportion of viable cells and the growing rates in fresh pMSCs were significantly (P<0.05) higher than those of other groups, but did not differ between the cryopreserved groups. The expression of Sox-2 and Nanog gene was increased by extending culture time in cryopreserved groups. The expression of Bax gene in cryopreserved groups was similar with fresh pMSCs. Moreover, the gene expression of adipocyte-specific marker as well as chondrogenic/osteogenic factors in cryopreserved groups was similarly to fresh pMSCs. Taken together, our results suggested that all these cryoprotectants of 10% DMSO, 1.5M EG and 5% DMSO/0.75M EG could be used for cryopreservation of the pMSCs.

• Radiation Oncology Journal
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• v.10 no.2
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• pp.261-266
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• 1992

We analyzed retrospectively the patients of granulocytic sarcoma treated with radiotherapy at the Department of Radiation Oncology, Yonsei University College of Medicine from Mar 1987 to Mar.1992 in an attempt to review our experience with irradiation of granulocytic sarcoma and to evaluate the treatment results for the radiation dose response. Fourteen lesions of granulocytic sarcoma in 9 patients were developed in variable clinical settings such as AML, CML and without leukemia. The involved lesions were bone, lymph node, soft tissue and skin in descending order of occurrence. All of the lesions in 9 patients were treated with external beam radiotherapy (Co-60 or electron beam). Both age distribution and clinical settings did not show any correlation with the response to treatment. The response to treatment seemed to be better for lesions in the bone than in other involved lesions. The majority received local irradiation of a total dose of more than 2000 cGy. Radiation dose of more than 2000 cGy showed excellent local control of $100\%$, (11/11), while local control decreased to $33\%$(1/3) with total dose less than 2000 cGy. In conclusion, local radiotherapy seems to be very effective for palliative or curative aim of granulocytic sarcoma, and a radiation dose more than 2000 cGy is highly recommended.

• Reproductive and Developmental Biology
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• v.34 no.3
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• pp.143-151
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• 2010

Pluripotency of human embryonic stem cell (hESC) is one of the most valuable ability of hESCs for applying cell therapy field, but also showing side effect, for example teratoma formation. When transplant multipotent stem cell, such as mesnchymal stem cell (MSC) which retains similar differentiation ability, they do not form teratoma in vivo, but there exist limitation of cellular source supply. Accordingly, differentiation of hESC into MSC will be promising cellular source with strong points of both hESC and MSC line. In this study, we described the derivation of MSC like cell population from feeder free cultured hESC (hESC-MSC) using direct differentiation system. Cells population, hESC-MSC and bone marrow derived MSC (BM-MSC) retained similar characteristics in vitro, such as morphology, MSC specific marker expression and differentiation capacity. At the point of differentiation of both cell populations, differentiation rate was slower in hESC-MSC than BM-MSC. As these reason, to verify differentially expressed molecular condition of both cell population which bring out different differentiation rate, we compare the molecular condition of hESC-MSC and BM-MSC using 2-D proteomic analysis tool. In the proteomic analysis, we identified 49 differentially expressed proteins in hESC-MSC and BM-MSC, and they involved in different biological process such as positive regulation of molecular function, biological process, cellular metabolic process, nitrogen compound metabolic process, macromolecule metabolic process, metabolic process, molecular function, and positive regulation of molecular function and regulation of ubiquitin protein ligase activity during mitotic cell cycle, cellular response to stress, and RNA localization. As the related function of differentially expressed proteins, we sought to these proteins were key regulators which contribute to their differentiation rate, developmental process and cell proliferation. Our results suggest that the expressions of these proteins between the hESC-MSC and BM-MSC, could give to us further evidence for hESC differentiation into the mesenchymal stem cell is associated with a differentiation factor. As the initial step to understand fundamental difference of hESC-MSC and BM-MSC, we sought to investigate different protein expression profile. And the grafting of hESC differentiation into MSC and their comparative proteomic analysis will be positively contribute to cell therapy without cellular source limitation, also with exact background of their molecular condition.

• Journal of Gastric Cancer
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• v.6 no.3
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• pp.189-192
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• 2006

Acute disseminated intravascular coagulation (DIC) associated with gastric cancer is not common and has short survival of 1 to 3 weeks. Systemic chemotherapy in spite of hematologic unstability for gastric cancer may prolong survival time. A 47-year-old woman who complained of dyspnea, vaginal bleeding and easy bruisibility was diagnosed to stage IV gastric cancer with acute disseminated intravascular coagulation based on the laboratory data. She also had multiple bone metastases and bone marrow involvement. This is the first case treated with combination chemotherapy of irinotecan and cisplatin for advanced gastric cancer complicated by disseminated intravascular coagulation at the time of diagnosis, With systemic chemotherapy, some of the bleeding symptoms and the DIC process improved, even not completely recovered. However the patient died of disease progression and survival time was 12 weeks.

• Progress in Medical Physics
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• v.20 no.3
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• pp.152-158
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• 2009

Total Skin Electron Beam Therapy (TSEBT) of linear accelerator has become use so as to be useful, 2~9 MeV of energy territories came to be used with mycosis fungoides and cutaneous lymphomas in the superficial lesion treatment which covers the major portion of the body. I treat a patient to Stanford technique in this study, and it is $60^{\circ}$ around the patients whom Stanford technique irradiated electronic beam to a linear accelerator in horizontal directions and there is a way a standard of TSEBT treat it to six located field (anterior, posterior, and four obliques) becoming. An each field does horizontally it and consist to beam of the two component which fitted the center to a suitable angle. a patient treats it to three dual field a day in order to make short treatment time. when a first day, we treat one dual field at anterior position and two dual field at posterior position. when the second day, treat one dual field at posterior position and two dual field at anterior position. Therefore, six dual field is finished in perfect periodic two days. we made cylindrical acrylic phantom, and I inserted a dosimeter film between phantom. in order to measure a dose distribution calculation before treat a patient, and a patient checked it in six field directions that got from a treatment. It is after that thermoluminescent dosimetry (TLD) as it uses Rando phantom and then measurement dose distribution in six field directions after attaching at chest, the right and left flank, a back after irradiation.

• Journal of the korean academy of Pediatric Dentistry
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• v.36 no.4
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• pp.619-624
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• 2009

Pyknodysostosis(PKND) is a rare sclerosing bone disorder that has an autosomal recessive trait, also known as Toulouse-Lautrec syndrome. Deficiency of the cathepsin enzyme K in the osteoclasts of PKND patients results in continuous endosteal bone deposits without osteoclastic resorption or remodeling. This causes a generalized increase in sclerosis and fragility of bones. Osteomyelitis in the mandible and recurrent fracture of the long bones are characteristic complicatons of PKND. The patients present typical features of PKND, such as short stature under 150 cm, open cranial suture and fontanelle, club-shaped phalanges, and underdevelopment of midface. This is a case of a 7-year-old girl with PKND, who visited our clinic with the chief complaint of anterior Open-bite and generalized crowding. The patient had been diagnosed as PKND by an orthopedist and manifested characteristic clinical and radiographic features, such as open cranial suture and fontanelle, obtuse madibular gonial angle, frontal and occipital bossing, grooved palate, club-shaped phalanges, and short stature. Orthodontic treatment was not considered because patients with PKND show abnormal bone resorption and remodeling. Instead, removal of deciduous teeth near exfoliation and TFA were performed, and periodic check-up is planned to maintain good oral hygiene.

• Journal of Oral Medicine and Pain
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• v.32 no.4
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• pp.449-453
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• 2007

Trigeminal neuralgia is defined as "a sudden, usually unilateral, brief stabbing recurrent pain in the distribution of one or more branches of the fifth cranial nerve" by the International Association for the Study of Pain(IASP). Trigeminal neuralgia is classified as an idiopathic trigeminal neuralgia with no apparent cause and a symptomatic trigeminal neuralgia which is caused by a structural lesion such as brain tumor. Over 80% of the tumors are meningioma, acoustic neuroma, and epidermoid tumors. Symptomatic trigeminal neuralgia can not be excluded even if old-aged patient does not have abnormal neurologic sign and symptom, and good response to pharmacotherapy. Therefore, initial examinations such as MRI or CT are essential to exclude symptomatic trigeminal neuralgia. When compared with CT, MRI, especially gadolinium enhanced MRI, has an increased sensitivity in the detection of intracranial lesions. The most effective medical treatment of trigeminal neuralgia is carbamazepine. The most common side effects of carbamazepine include drowsiness, dizziness, unsteadiness, nausea, anorexia. Hepatotoxicity, bone marrow depression are the most feared side effect of carbamazepine therapy but occurs rarely. It require periodic complete blood cell counts as well as hepatic and renal function tests. It has been recommended that complete blood cell counts is done every 2 weeks for the first 2months and then quaterly thereafter. Oxcarbazepine can be used if neutropenia occurs.

• Journal of Life Science
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• v.23 no.8
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• pp.1050-1056
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• 2013

Streptococcus pneumoniae is the leading cause of community-acquired pneumonia throughout the world. The bacteria invade through lung tissue and cause sepsis, shock, and serious sequelae, including rheumatic fever and acute glomerulonephritis. However, the molecular mechanism associated with pneumonia's penetration of lung tissue and invasion of the blood stream are still unclear. We attempted to investigate the host cell response at protein levels to S. pneumoniae D39 invasion using human lung cancer epithelial cells, A549. Streptococcus pneumoniae D39 began to change the morphology of A549 cells to become round with filopodia at 2 hours post-infection. A549 cell proteins obtained at each infection time point were separated by SDS-PAGE and analyzed using MALDI-TOF. We identified several endoplasmic reticulum (ER) resident proteins such as Grp94 and Grp78 and mitochondrial proteins such as ATP synthase and Hsp60 that increased after S. pneumoniae D39 infection. Cytosolic Hsc70 and Hsp90 were, however, identified to decrease. These proteins were also confirmed by Western blot analysis. The identified ER resident proteins were known to be induced during ER stress signaling. These/ data, therefore, suggest that S. pneumoniae D39 infection may induce ER stress.

• Journal of Oral Medicine and Pain
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• v.34 no.3
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• pp.295-301
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• 2009

Recently, bisphosphonate-associated osteonecrosis of the jaw(BONJ) is added to the list of diseases of the oromaxillofacial region. BONJ is defined as exposed bone in the jaw that does not heal within 8 weeks after identification, in a patient who has been received to bisphosphonates and has not taken radiation therapy to the craniofacial region. Bisphosphonates binded to bone mineral are concentrated in highly active remodeling site, reside in the skeleton for a long time, and do a role as powerful inhibitors of bone resorption. As the patients receiving bisphosphonates therapy grow in number, the patients of BONJ would go on increasing in Korea. We would like to present two patients who were suspected to BONJ, describe the outline of BONJ, and mention importance of our understanding about BONJ. BONJ is rare disease, but once it develops, its prognosis is very poor. Our adequate understanding of BONJ is necessary to prevent it and cope with it properly.