Long-term outcomes of enzyme replacement therapy for Taiwanese patients with Mucopolysaccharidosis I

Mucopolysaccharidosis (MPS) I is an autosomal recessive lysosomal storage disorder resulting from a deficiency in the lysosomal enzyme α-l-iduronidase, leading to the accumulation of the glycosaminoglycans (GAGs) dermatan sulfate (DS) and heparan sulfate (HS) in cells throughout the body. The clinical characteristics of MPS I include cognitive impairment (severe form), coarse facial features, corneal clouding, respiratory disorder s, cardiac diseases, hepatomegaly, umbilical and inguinal hernias, skeletal abnormalities, and joint contractures.
Source: Pediatrics and Neonatology - Category: Perinatology & Neonatology Authors: Tags: Short Communication Source Type: research

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Authors: Kuo KH, Yu YB, Lai TH PMID: 31321762 [PubMed - in process]
Source: Acta Neurologica Taiwanica - Category: Neurology Tags: Acta Neurol Taiwan Source Type: research
CONCLUSION: Microvascular decompression may be more effective than radiofrequency thermocoagulation for patients with classical trigeminal neuralgia with concomitant persistent facial pain. PMID: 31321761 [PubMed - in process]
Source: Acta Neurologica Taiwanica - Category: Neurology Tags: Acta Neurol Taiwan Source Type: research
CONCLUSION: Cranial MRI should be performed to identify the presence of brain abscesses in elderly patients with K. pneumoniae bloodstream infections but without clinically evident neurological signs or symptoms. Serial MRI studies are important to monitor the therapeutic course. PMID: 31321760 [PubMed - in process]
Source: Acta Neurologica Taiwanica - Category: Neurology Tags: Acta Neurol Taiwan Source Type: research
CONCLUSIONS: We introduce a thermo regulator like structure, describe a new syndrome. In addition, it was noted thatwater-filled vesicles of CP are increased, brain temperature in nearly normal in the early phase of SAH due to likely irritation of vagal nerves. However in the late phase, mean number of water-filled vesicles numbers decreased in accordance with increased brain temperature with degenerative changes of the nodose ganglion. PMID: 31321759 [PubMed - in process]
Source: Acta Neurologica Taiwanica - Category: Neurology Tags: Acta Neurol Taiwan Source Type: research
Authors: Kim GB Abstract Epidemiologic studies of Kawasaki disease (KD) have shown new pattern or change of KD occurrence and supported to infer pathophysiology or risk factors of KD from the first patient with KD reported in 1961. The incidence of KD in Northeast Asia country including Japan, South Korea, China and Taiwan are 10-30 times higher than that of KD in the Unites States and Europe. Knowing true epidemiology of KD in each country and publications of update KD epidemiology also could give KD vigilance to general health care provider and general population, which definitely could give early detection of KD...
Source: Korean Journal of Pediatrics - Category: Pediatrics Tags: Korean J Pediatr Source Type: research
Publication date: Available online 20 July 2019Source: Journal of the Taiwan Institute of Chemical EngineersAuthor(s): Shenhao Yuan, Xushuai Lv, Yiwei Zhang, Yangjin Wu, Chunfeng Mao, Yuming Zhou, Wenxia Chen, Yanyun WangAbstractA core-shell structure Au-based catalysts mSiO2/Au/Co3O4HS were successfully derived from in-situ growth ZIF-67 hollow spheres, which were synthesized by a one-step soft template method. The samples were characterized by SEM, TEM, EDX, XPS, XRD and BET to confirm its successful preparation and structural features. The catalytic performance of the catalysts were evaluated by liquid phase oxidation o...
Source: Journal of the Taiwan Institute of Chemical Engineers - Category: Chemistry Source Type: research
In this study, a novel green strategy through fusion hydrothermal method was adopted for in-situ synthesis of chabazite. The morphological and physiochemical characteristic of the prepared adsorbent was determined by X-ray diffraction, scanning electron microscopy, and infrared spectroscopy. The characterization results revealed that prepared chabazite posses fine particles (2–5 µm) and porous structure which could be employed for capture of CO2 molecules. In order to gain an understanding of the adsorption performance of prepared adsorbent, different types of adsorption isotherms and kinetic adsorption model...
Source: Journal of the Taiwan Institute of Chemical Engineers - Category: Chemistry Source Type: research
Publication date: Available online 19 July 2019Source: Journal of the Taiwan Institute of Chemical EngineersAuthor(s): Bingcheng Liu, Mengmeng Zhang, Xuan Yang, Ting WangAbstractCO2-EOR is a recognized effective technology to enhance oil recovery by injecting CO2 into the reservoir to increase layer pressure. With the exploitation of oil, the overflow of large amount of CO2 has caused great difficulties to gas treatment and transportation, even affects the climate if it is discharged directly. CO2 capture from extraction gas plays an increasingly important role in oilfield due to these problems. Compared with other methods...
Source: Journal of the Taiwan Institute of Chemical Engineers - Category: Chemistry Source Type: research
Publication date: Available online 19 July 2019Source: International Journal of Surgery Case ReportsAuthor(s): Dario Iadicola, Massimo Branca, Massimo Lupo, Eugenia Maria Grutta, Stefano Mandalà, Gianfranco Cocorullo, Antonino MirabellaAbstractIntroductionTraumatic diaphragmatic injuries are rare complications resulting from a thoracic-abdominal blunt or penetrating trauma. Left-sided diaphragmatic injuries are more commonly reported in literature. Bilateral injuries are extremely rare, occurring in about 3% of the patients and just few cases reported in literature. Traumatic diaphragmatic hernias are definitely a m...
Source: International Journal of Surgery Case Reports - Category: Surgery Source Type: research
This study investigated the effects of statin in AF patients who experienced acute ischemic stroke.MethodsData from patients with AF experiencing first-ever ischemic stroke between 2001 and 2010 were collected from the Taiwan National Health Insurance Research Database and categorized into non-statin and statin groups. The statin group was further divided into pre-stroke statin (those who began statin therapy before stroke) and post-stroke statin (those who began statin therapy after stroke) groups. The risks for recurrent ischemic stroke, coronary artery disease (CAD), intracranial hemorrhage (ICH), and 1-year mortality w...
Source: Journal of Clinical Neuroscience - Category: Neuroscience Source Type: research
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