نتایج جستجو برای: lamy
تعداد نتایج: 314 فیلتر نتایج به سال:
We present the polarimetric analysis of white-light images corona obtained with Large-Angle Spectrometric COronagraph LASCO-C3 onboard Solar and Heliospheric Observatory (SOHO) from 1996 to 2019, leading separation K- F-components derivation electron-density distribution. The makes use polarized sequences composed three through polarizers oriented at $+60^{\circ}$ , $0^{\circ}$ $-60^{\circ }$ c...
Kistler, kaynak aldıkları epitel dokusuna göre farklı özellik gösterebilen merkezî konumlu bir lümene ve bağ doku duvarına sahip patolojik boşluklardır. Çocuklarda nadir görülmekle birlikte, erişkinlere daha hızlı büyüme ilerleme özelliği gösterebilmektedirler. Dişlerin gömülü kalmasına sürememesine, mine malformasyonlarına, çene fraktürlerine, dokusunun metaplazi göstermesiyle kanser gelişimin...
چکیده مقدمه: موکوپلی ساکاریدوز نوع 6 (mps-6) بیماری اتوزوم مغلوبی است که در آن بیمار به علت کمبود آنزیم آریل سولفاتاز b دچار اختلال ذخیره لیزوزوم و تجمع متابولیت های غیرطبیعی در بافت های مختلف بدن می شود. این بیماری نادر که تا کنون از استان گیلان گزارش نشده می تواند تظاهر بالینی متفاوت از ملایم تا شدید داشته باشد. معرفی مورد: بیماری با شکایت اولیه حرکت های مشکوک به تشنج در 20 ماهگی معرفی می شود...
2. Kawahara T, Iizuka T. Inhibitory effect of hot-water extract of quince (Cydonia oblonga) on immunoglobulin E-dependent late-phase immune reactions of mast cells. Cytotechnology. 2011;63:143--52. 3. Gründemann C, Papagiannopoulos M, Lamy E, MerschSundermann V, Huber R. Immunomodulatory properties of a lemon-quince preparation (Gencydo®) as an indicator of anti-allergic potency. Phytomedicine....
We report a new assay of N-acetylgalactosamine-4-sulfatase (aryl sulfatase B) activity in dried blood spots (DBS) for the early detection of mucopolysaccharidosis VI (Maroteaux-Lamy syndrome) in newborn screening. The assay uses a synthetic substrate consisting of N-acetylgalactosamine-4-sulfate moiety glycosidically linked to a hydrophobic residue and furnished with a tert-butyloxycarbamido gr...
AIM To carry out a detailed morphological study of the cornea of a 16 year old female with a Maroteaux-Lamy syndrome (MLS). METHODS Following a penetrating keratoplasty in July 1999, ultrastructural changes in the cornea were examined using electron microscopy. Proteoglycans were visualised using cuprolinic blue dye; and betaig-h3 and keratan sulphate were detected by immunoelectron microscop...
Ornithischian dinosaur body fossils are extremely rare in Triassic rocks worldwide, and to date the majority of such fossils consist of isolated teeth. Revueltosaurus is the most common Upper Triassic ornithischian dinosaur and is known from Chinle Group strata in New Mexico and Arizona. Historically, all large (>1 cm tall) and many small ornithischian dinosaur teeth from the Chinle have been r...
It is generally believed that the incidence of congenital malformation of the heart is raised in relatives of affected individuals. The evidence in support of this view consists mainly of isolated records of families with more than one affected member (Courter et al., 1948, refer to 63 reported cases), but a few examples have been noted in the course of investigations of large series of patient...
INTRODUCTION Mucopolysaccharidoses (MPS) are a group of inherited disorders due to lysosomal enzyme deficiencies. The aims of this study are to describe the neuroimaging findings in children evaluated in our hospital with this diagnosis, looking for a possible correlation of these alterations with the type of MPS and clinical severity, and finally to compare these findings with those previously...
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