• Journal of Korean Neurosurgical Society
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• v.61 no.3
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• pp.302-311
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• 2018

Atypical teratoid rhabdoid tumours (ATRTs) are the most common malignant central nervous system tumours in children ${\leq}1year$ of age and represent approximately 1-2% of all pediatric brain tumours. ATRT is a primarily monogenic disease characterized by the bi-allelic loss of the SMARCB1 gene, which encodes the hSNF5 subunit of the SWI/SNF chromatin remodeling complex. Though conventional dose chemotherapy is not effective in most ATRT patients, high dose chemotherapy with autologous stem cell transplant, radiotherapy and/or intrathecal chemotherapy all show significant potential to improve patient survival. Recent epigenetic and transcriptional studies highlight three subgroups of ATRT, each with distinct clinical and molecular characteristics with corresponding therapeutic sensitivities, including epigenetic targeting, and inhibition of tyrosine kinases or growth/lineage specific pathways.

• Informatization Policy
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• v.19 no.3
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• pp.36-49
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• 2012

Internet Corporation for Assigned Names and Numbers (ICANN) is the body which manages the allocation and registration of global Internet IP addresses and domain names. As a gesture to overcome the dependency on the U.S. government, ICANN and the U.S. Department of Commerce jointly made the Affirmation of Commitment in which they submitted the workings of ICANN to the supervision of a council of Internet experts. Accountability and Transparency Review Team (ATRT) is one of four teams created for this purpose. In December 2010, ATRT made 27 recommendations, which were subsequently approved by the ICANN Board. These reform efforts of ICANN provide significant implications for the future of Korean Internet governance. Korea should enhance the compatibility of its domestic decision making process related to Internet addresses with the private sector driven, bottom-up process of global Internet governance. In order to increase the involvement of private sector and civil society, it is recommended for Korea to formalize the mechanism for bottom-up process. Insertion of a new provision concerning a bottom-up process into the Internet Addresses Act should be considered.

• Clinical and Experimental Pediatrics
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• v.57 no.7
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• pp.333-336
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• 2014

Reports of constitutional ring chromosome 22, r(22) are rare. Individuals with r(22) present similar features as those with the 22q13 deletion syndrome. The instability in the ring chromosome contributes to the development of variable phenotypes. Central nervous system (CNS) atypical teratoid rhabdoid tumors (ATRTs) are rare, highly malignant tumors, primarily occurring in young children below 3 years of age. The majority of ATRT cases display genetic alterations of SMARCB1 (INI1/hSNF5 ), a tumor suppressor gene located on 22q11.2. The coexistence of a CNS ATRT in a child with a r(22) is rare. We present a case of a 4-month-old boy with 46,XY,r(22)(p13q13.3), generalized hypotonia and delayed development. High-resolution microarray analysis revealed a 3.5-Mb deletion at 22q13.31q13.33. At 11 months, the patient had an ATRT ($5.6cm{\times}5.0cm{\times}7.6cm$) in the cerebellar vermis, which was detected in the brain via magnetic resonance imaging.