Abstract Mucopolysaccharidosis type IIIA (MPS IIIA) is a lysosomal storage disorder due to the deficient activity of sulfamidase (SGSH).Traditionally, measurement of this enzymatic activity has been performed using a fluorescently (4-MU) labeled glycoside substrate.While this substrate is inexpensive and readily available, the current method requir
Panniculitis is an uncommon skin eruption observed in patients with dermatomyositis (DM)/clinically amyopathic dermatomyositis (CADM), especially in anti-melanoma differentiation-associated gene 5 (MDA5) antibody-positive DM.We present here a 51-year-old Japanese woman with an anti-MDA5 antibody-positive DM who initially had cellulitis-like erythem
Background: We have observed that patients with descending necrotizing mediastinitis show bilateral lower lobes atelectasis before pleural effusion that finally forms empyema.Successful treatment of mediastinitis emphasizes optimal early JACKETS SPLIT MARLOWE drainage of neck, mediastinum, and antibiotic therapy.Methods: Exploratory study to determ
When a fraction of the light reflected by an external cavity re-enters the laser cavity, both the amplitude and the frequency of the lasing field can be modulated.This phenomenon is called the self-mixing effect (SME).A self-mixing laser diode (SM-LD) is a sensor using the SME.Usually, such LDs operate below the stability boundary where no relaxati
Grease is highly susceptible to degradation due to regular usage and the severity of the operating conditions.Degradation can negatively impact the performance of grease-lubricated machinery, demanding frequent maintenance to avoid premature failure of machine elements.Quantification of grease degradation has proven to be a formidable task, for whi