Niemann-Pick disease type C (NPC) disease is a neurodegenerative lysosomal storage disease caused by mutations in the NPC1 or NPC2 genes. Liver disease is also a common feature of NPC that can present as cholestatic jaundice in the neonatal period. Liver enzymes can remain elevated above the normal range in some patients as they age. We recently reported suppression of the P450 detoxification s...

In bribery an external agent tries to alter the outcome of an election by changing some voters’ votes. Usually, when investigating bribery problems, full information is assumed, i.e., that the manipulative agent knows the set of candidates, each voter’s votes and the voting rule used. In this paper, we formally introduce different structures of partial information, we show the connections betwe...

It is well known that the Epstein-Barr virus (EBV) contributes directly to tumourigenesis in nasopharyngeal carcinoma (NPC), primarily in the undifferentiated form of NPC (WHO type III; UNPC or UC), which is commonly found in South East Asia. Unfortunately, research in NPC has been severely hampered by the lack of authentic EBV-positive (EBV+) human NPC cell lines for study. Since 1975, there h...

Niemann-Pick type C (NPC) disease is a fatal autosomal recessive disorder characterized by the accumulation of free cholesterol and glycosphingolipids in the endosomal-lysosomal system. Patients with NPC disease have markedly progressive neuronal loss, mainly of cerebellar Purkinje neurons. There is strong evidence indicating that cholesterol accumulation and trafficking defects activate apopto...

Parotid area lymph node (PLN) metastasis in nasopharyngeal carcinoma (NPC) is rare, and its prognosis remains largely unknown. Our study aimed to investigate the prognostic value and staging categories of PLN metastasis in patients with NPC and treated with intensity-modulated radiation therapy (IMRT), to provide a reference for clinical treatment for NPC with PLN metastasis. Records for 1616 u...

how to cite this article: karimzadeh p. juvenile type of niemann-pick type c disease and our study in iranian npc patients. iran j child neurol. 201 5 autumn;9:4(suppl.1): 5-6. pls see pdf.

Nasopharyngeal carcinoma (NPC) is a rare disease in children. Children with NPC almost always have the undifferentiated variant of the disease, which is associated with advanced locoregional and distant metastasis. We report two cases to illustrate that high index of suspicion is necessary to diagnose NPC in children especially those with atypical presentation of otitis media with effusion (OME).

Introduction: Nasopharyngeal carcinoma (NPC) is a common cancer in the North-East region of India. Though the role of environmental contributors of NPC in the North-Eastern part of India is firmly established, EBV as an etiological agent in the region remains unexplored. Material and Methods: Fifty-one patients, who presented at the department of ENT, NEIGRIHMS and were confirmed as NPC upon h...

introduction: nasopharyngeal carcinoma (npc) is a common cancer in the north-east region of india. though the role of environmental contributors of npc in the north-eastern part of india is firmly established, ebv as an etiological agent in the region remains unexplored. material and methods: fifty-one patients, who presented at the department of ent, neigrihms and were confirmed as npc upon hi...

BACKGROUND Niemann-Pick type C (NPC) disease is a lysosomal storage disease characterized by the accumulation of cholesterol and glycosphingolipids. The majority of NPC patients die in their teen years due to progressive neurodegeneration; however, half of NPC patients also suffer from cholestasis, prolonged jaundice, and hepatosplenomegaly. We previously showed that a key mediator of NPC liver...