Intracerebroventricular dosing of N-sulfoglucosamine sulfohydrolase in mucopolysaccharidosis IIIA mice reduces markers of brain lysosomal dysfunction.

Mucopolysaccharidosis type IIIA (MPS IIIA) is a lysosomal storage disorder caused by N-sulfoglucosamine sulfohydrolase (SGSH) deficiency. SGSH removes the sulfate from N-sulfoglucosamine residues on the nonreducing end of heparan sulfate (HS-NRE) within lysosomes. Enzyme deficiency results in accumulation of partially degraded HS within lysosomes throughout the body, leading to a progressive severe neurological disease.

Functional results after mesorectal excision for rectal cancer: comparative study among surgical approaches.

Background: We analyzed the evolution of genitourinary dysfunctions in patients undergoing surgical treatment for rectal cancer, and compared open surgery, laparoscopy, robotic and TaTME.

Methods: Functional outcomes were evaluate using standardized questionnaires, compiled at the start of treatment, after the end of Radiotherapy, at 1 and 6 months after surgery.

Results: In 72 patients 37.

Evaluation of mental foramen and inferior alveolar nerve canal and its relationship to adjacent anatomical landmarks using cone-beam computer tomography.

The objective of the research was to evaluate the location, size, variability, and morphologic features of mental foramen (MF) and the inferior alveolar nerve canal (IAN) on cone-beam CT. We evaluated the morphologic findings of mental foramen (MF) and inferior alveolar nerve (IAN) canal of 88 mandibular hemiarches of 65 Caucasian subjects (35 males, 30 females; age range 25-75 years) using cone beam CT. The most common horizontal position of MF was type 3 (53.

Quantitative Assessment of Neuroinflammation, Myelinogenesis, Demyelination, and Nerve Fiber Regeneration in Immunostained Sciatic Nerves From Twitcher Mice With a Tissue Image Analysis Platform.

Scoring demyelination and regeneration in hematoxylin and eosin-stained nerves poses a challenge even for the trained pathologist. This article demonstrates how combinatorial multiplex immunohistochemistry (IHC) and quantitative digital pathology bring new insights into the peripheral neuropathogenesis of the Twitcher mouse, a model of Krabbe disease. The goal of this investigational study was to integrate modern pathology tools to traditional anatomic pathology microscopy workflows, in order to generate quantitative data in a large number of samples, and aid the understanding of complex disease pathomechanisms.