A 4-year-old Afghan girl born to consanguineous parents presented with progressive neurological regression and hepatomegaly noticed after one year of age. The child had hypotonia, repeated unexplained falls and facial dyskinesia. Bone marrow examination revealed presence of storage cells suggestive of Gauchers or Niemann Pick. Confirmatory study by lysosomal enzyme from leucocytes was normal fo...

To recognise and respond to pathogens, germ-line encoded pattern recognition receptors (PRRs) bind to conserved microbial structures and activate host defence systems, including microbial uptake by phagocytosis. Phagocytosis is a complex process that is instrumental in the control of extracellular pathogens, and this activity is mediated by several PRRs, including a number of C-type lectins. Wh...

Results There were 102 type C pilon fractures in 99 patients, whose mean age was 42 years (16 to 86) and 77 were male. Primary open reduction internal fixation (ORIF) was performed in 73 patients (71.6%), whilst 20 (19.6%) underwent primary external fixation with delayed ORIF. There were 18 wound infections (17.6%). A total of nine (8.8%) were deep and nine were superficial. Infection was assoc...

in order to determine the effects of fish size and weight on its salinity tolerance, the chloride cells (ccs) immunolocalization changes were examined in caspian salmon (salmo trutta caspius) parrs, with the same age (about 2 years old) but different types (type a: 4.88 g, 8.36 cm; type b: 14 g, 11.84 cm; type c: 24.05 g, 14.08 cm). fish survival rate, blood osmolality, gills ccs histological a...

the oromandibular-limb hypogenesis syndrome comprises a group of anomalies which simultaneously affect the mandible, tongue, and maxilla with or without reductive limb anomalies. it is characterized by failure of development of the intraoral region and distal extremities. multiple and variable deformities of the mandible, maxilla and tongue may occur in combination with a variety of limb defect...

image segmentation is an essential issue in image description and classification. currently, in many real applications, segmentation is still mainly manual or strongly supervised by a human expert, which makes it irreproducible and deteriorating. moreover, there are many uncertainties and vagueness in images, which crisp clustering and even type-1 fuzzy clustering could not handle. hence, type-...

editor considering the increasing prevalence of hcv in diabetes type 2 and chronic nature of hcv infection and its progression to cirrhosis and hepato_cellular tumors, we aimed to determine the frequency of hcv infection in diabetes type 2 in order to diagnose and treat these patients as soon as possible. essential information gathered by a check list from type two diabetic patients admitted at...

how to cite this article: mohammadi m. nieman-pick disease type c: an epileptologic view. iran j child neurol. 2015 autumn;9:4(suppl.1): 11.   pls see pdf.