![]() ![]() The activity of dihydrolipoamide acyl dehydrogenase (E3) -deficient BCKDA is 25% lower than that of normal children, and it is characterized by low tension, stunting, and lactic acidosis 6.Īccording to the Guidelines for the Diagnosis and Treatment of Maple syrup urine disease (2019 Edition, China), MSUD is diagnosed based on the typical clinical manifestations such as nervous system injury and urine maple sugar odor, elevated plasma level of BCAAs (leucine, isoleucine, and valine) and allo-isoleucine and elevated urine level of branched-chain hydroxyacids and ketoacids(BCKAs). Intermittent type usually occurs from 5 months to 2 years with mild symptoms 4, prognosis of the thiamine-responsive type is better than classic type, with BCKDA activity of 2% -40%, and some children can survive for a long time 5. 20% are intermediate or intermittent type, the intermediate type usually shows a continuously increased concentration of branched-chain amino acids (BCAAs), accompanied by nervous system damage 3. The liver BCKDC activity in classic type children is often lower than 2% in healthy children, manifested as ketoacidosis, neurologic damage and mental retardation 2. The classic type is the most common and severe type in the neonatal period, accounting for 75% affected infants. According to the phenotype, MSUD can be divided into 5 types 1: classic, intermediate, intermittent, thiamine-responsive and dihydrolipoylamide dehydrogenase (E3) deficiency. Without timely intervention, the disease progresses rapidly, and the mortality and disability rate are very high. Common clinical manifestations of MSUD are feeding difficulties, epilepsy, mental retardation, ketonuria and maple-like body odor. Maple syrup urine disease (MSUD, OMIM # 248600) is a hereditary branched-chain amino acid metabolism disorder caused by branched chain α-ketoacid dehydrogenase multi-enzyme complex (BCKDC). Computational structural modeling indicated that these novel variations probably affect structural stability and considered as likely pathogenic variants. NGS plus Sanger sequencing detection is effective and accurate for gene diagnosis. In addition, 4 previously unidentified variants (p.Gly281Arg in BCKDHA gene, p.Ser171Pro, p.Gly95Ala and p.Lys222Thr in BCKDHB gene) were identified. Totally, 13 variants were detected, including 2 variants (p.Ala216Val and p.Gly281Arg) in BCKDHA gene, 10 variants (p.Gly95Ala, p.Ser171Pro, p.Phe175Leu, p.Arg183Trp, p.Lys222Thr, p.Arg285Ter, p.Arg111Ter, p.S184Pfs*46, p.Arg170Cys, p.I160Ffs*25) in BCKDHB gene, 1 variant (p.Arg431Ter) in DBT gene. Targeted NGS revealed 2 pedigrees with MSUD Ia, 5 pedigrees with Ib, 1 pedigree with MSUD II. All the coding regions and exon/intron boundaries of BCKDHA, BCDKHB, DBT and DLD genes were analyzed by targeted NGS in the 8 MSUD pedigrees. ![]() In this report, 8 patients (4 females and 4 males) with MSUD from 8 unrelated Chinese Han families were diagnosed at the age of 6 days to 4 months. Only a few cases of MSUD have been documented in Mainland China. Maple syrup urine disease (MSUD) is a rare autosomal recessive disorder that affects the degradation of branched chain amino acids (BCAAs).
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![]() Increases the AC power and maintains a safe voltage level without switching to battery mode and conserving battery life.ĭisplays immediate, detailed information on the UPS battery and power conditions, alerting users to potential problems.Įxists when a line interactive UPS has an autotransformer that regulates low voltages (e.g., brownouts) without having to switch to battery. Provides audible notification of certain events such as running on battery power, low battery, or overload conditions. ![]() Includes everything needed to backup your sump pump, including batteries. Works with your existing sump pump, simple to set up, and doesn’t require professional installation. The runtime is based on an intermittent sump pump operating at a 10% duty cycle.ĭiverts excess voltage away from sump pump during an AC power surge or power spike to prevent damage. Supplies power to 1/2 HP (or less) sump pump during utility power outages for operation up to 120 minutes. *Note: Runtime is based on an intermittent sump pump operating at a 10% duty cycle. The A1500SP1 comes with a two-year warranty including coverage of batteries. Additional features include surge protection, automatic voltage regulation, and audible alarms. Installation is simple and no additional components are required, so you can begin backing up your sump pump quickly and without professional assistance. The A1500SP1 works with an existing sump pump up to 1/2 HP in capacity and ensures sump pump operation for up to two hours*. Protect your basement from flooding and gain peace of mind with the A1500SP1 Sump Pump Battery Backup from CyberPower. The time it takes for a cleaner to heat up varies, with some models boasting super fast heat-up times of under 10 seconds and other heavy-duty machines that can take up to 10 minutes. If you plan to clean multiple back-to-back areas of your home, opt for a model that will let you do this without refilling the tank for maximum efficiency. Some steam cleaners have adjustable output levels, so expect a shorter run time if you’re using it on the maximum output. The length of time your steam cleaner will run is based on the volume of the water tank and the flow rate. ![]() Microfiber cloths (a must for cleaning upholstery), brushes, crevice tools, and squeegees make your steam cleaner a versatile tool for tackling every corner of your living space, including the kitchen, bathroom, windows, and other sealed surfaces. You’ll also want to check out the included accessories. If, however, you don’t care about steam cleaning your floors, you can get away with a simple handheld machine that’ll ring up at a lower price point. An upright model, on the other hand, may be easier to maneuver and can usually be detached into two pieces, which means you can use it as a portable upholstery cleaner for your sofa - but note that the run time will be shorter, as you’ll have to refill the smaller water reservoir. Of the two, a canister cleaner will generally have a longer run time, thanks to its larger tank, but keep in mind that the two-piece construction can be somewhat cumbersome. While most options can take on a variety of surfaces besides couches, if you want something that can also clean floors, an upright or canister model will be your best bet. Much like vacuums, the best upholstery cleaners for sofas come in a range of styles, and your choice will partially depend on how much versatility you want. What To Consider When Shopping For Steam Cleaners For Couches Versatility ![]() If you have especially stubborn stains or spills to clean, you may need to agitate the area with a brush or a cloth before wiping it away, and most steam cleaners come with accessories to help with this. For the best results with a sofa, run a vacuum over the surface first to remove any debris, then slowly pass the steam cleaner over the targeted area. The key components to any steam cleaner include a reservoir and an internal boiler that heats water in order to produce a low-moisture vapor that softens stuck-on stains and messes, but won’t leave fabric overly damp. Available in handheld, full-size, and convertible options, the best steam cleaners for couches use high-temperature water vapor to clean upholstery and remove stains, and they come with attachments that let you use them on multiple other surfaces throughout your home. Whether you have kids or pets, or sometimes eat dinner while watching TV, your couch can see a lot of wear and tear - but the right steam cleaner can keep your sofa looking brand new. ![]() For example, a poster you design for a business conference and a poster you make for a summer beach party will require completely different designs, including different colors, shapes, layouts, and more importantly, fonts. There are just too many types of posters that require various design standards. It’s quite difficult to generalize poster design. In this post, we feature some of the best fonts for posters you can use to create the perfect poster design. Poster Design / 70+ Best Fonts for Posters 2023įonts come in all shapes and sizes, but when it comes to poster design, there are certain types of fonts that can help you make bold statements and attract attention. Get ready to elevate your digital artistry and make your creative mark with unparalleled precision. We’ll demystify the process and equip you with the knowledge you need to expand your typography toolkit in Procreate. In this guide, we’ll walk you through the process of adding new fonts to Procreate, step by step. Whatever the case may be, the ability to add new fonts to Procreate broadens your creative horizons and helps your digital artwork stand out from the crowd. Perhaps you’ve discovered a font elsewhere that perfectly encapsulates your style, or maybe you’ve created a custom font yourself. While Procreate boasts an extensive selection of fonts, there’s always room for a personal touch. Whether you’re a professional artist or a hobbyist, you’ll know the significance of customizing your design tools to suit your unique style and creative vision. ![]() Procreate has rapidly gained a reputation as a powerhouse in the world of digital illustration, becoming a go-to app for iPad users worldwide. Procreate Brushes / How to Add Fonts to Procreate Turn on a fan or air conditioning in hot weather or sleep with your itchy body part outside the covers at night, for instance. For other problems, keeping itchy skin cool could ease the urge to scratch a little, Yosipovitch says. It's fine to try an OTC itch reliever right away for bug bites and rashes. "A careful history and physical exam by the dermatologist can go a long way in identifying the true cause and fixing it as fast as possible." "Itching is one of the most common complaints in visits to a dermatologist, but itching is caused by so many different things it's hard to have one set protocol or method for addressing it," Krant says. If you're unsure why you're itching or you're extremely uncomfortable-unable to sleep through the night because of the need to scratch, for instance-check in with your doctor. And though dry skin is a far more frequent problem in the winter months, it can crop up now as well. Fungal infections and contact allergies (the usually short-lived rashy reactions some people have from substances such as cosmetics or certain fabrics or metals) are common in warmer weather. ![]() With these, it's easy to pinpoint the problem.īut other causes of itching may not be so obvious. "Summer brings out poison ivy and other itch-inducing plants, lots of bugs, and, of course, sunburn," says Jessica Krant, MD, MPH, assistant clinical professor of dermatology at SUNY Downstate Medical Center in New York City and a dermatologist in private practice in Manhattan. Product features: Non-abrasive yet highly concentrated the metal polish cloth gives a sparkling clean Improved for quick and easy cleaning and shining Floats off dirt grime and road film Won't harm paint or wax finish Suitable for all vehicle finishes High-quality soap meant to protect and enhance the paint job of the vehicle Will preserve the water repellent character of wax and continuously protect any finish Safe for all new and old finishes including clear coats Returns oxidized finishes to a mirror-like showroom shine UV and acid rain protection Contains montan wax Can be used on just about everything Included in this kit: 1 - IBIZ Everything Waterless Wash & Wax 32 oz. This complete car wash kit comes with the Everything Wash Everything Waterless Wash and Wax Everything Wax and the Wow Metal Polish along with a wax applicator pad cotton car polishing cloth and trigger sprayer bottle top. Removes dirt and grime and will not harm paint or wax finishes. The IBIZ Everything line of products is for any smooth non-porous surface which means they are safe for use on all vehicle finishes. Guaranteed to be the fastest easiest and best wash and shine product to be found anywhere when used correctly. Returns oxidized finishes to a mirror-like showroom shineĬan be used on just about everything Included in this kit:ġ - IBIZ Everything Waterless Wash & Wax 32 oz. Safe for all new and old finishes including clear coats Will preserve the water repellent character of wax and continuously protect any finish High-quality soap meant to protect and enhance the paint job of the vehicle Improved for quick and easy cleaning and shining Non-abrasive yet highly concentrated the metal polish cloth gives a sparkling clean ![]() item 2 WENOL red - 100g x 2 pc - Metal Polish aluminum brass silver gold tin WENOL red - 100g x 2 pc - Metal Polish aluminum brass silver gold tin. Removes dirt and grime and will not harm paint or wax finishes. 2 X IBIZ WOW Metal Polish-Silver Gold Brass Aluminum Steel- SHIP. ![]() ![]() You can then turn the screen back on, and see nothing. This makes it appear that you received a message. The best feature for me is the following: If you switch the phone off while on a call the phone vibrates. Sometimes, I don't hear any sound when I receive calls. It randomly displays or does not show notifications. It's ridiculous! It's the most horrible chatting app that I have ever seen. I was forbidden from accessing my personal history. This app was never removed from my phone. It Deletes User Information Without Your Permission Even though I still have my entire data, it only provides 360 days worth of chat history. After updating, this is the second time I have tried to log in.ĭO NOT DOWNLOAD IT NEVER ALLOW YOUR COMPANY TO USE THIS APP. Logging out automatically and refusing to let me log back in even though I have the correct password. Reject the proposal of a Chinese company to use this software. Shared screens disappear, audio disappeared, videos disappears. It doesn't properly detect sound and video. Video conferencing software that is slow. This is a problem I have because of my job. It is difficult to use and the service is slow. The privacy settings and slowing down of my mobile and desktop processing speeds are alarming. However, my messages don't sync completely with desktop. This program won't allow me to send and receive messages if I'm offline. This garbage program is unacceptable to me. ![]() Ding is my work phone number and the lack of notifications has been a huge problem. After the first few days of installation, I received both audio and visual notifications. ![]() There were no visual or audio notifications. The only small hiccup is that you have to punch in several times before it registers your attendance.I will not be notified by Ding about new messages All settings are correct in the app. It’s a great tool for employees working on a remote setup because it can streamline the entire workflow without decreasing productivity. Overall, DingTalk is a useful program that allows you to easily communicate with your team, relay tasks and reminders, clock in for work, and file for leaves wherever you are. However, it’s important to note that the app’s login and logout feature tends to glitch, so you must punch in multiple times to successfully log your attendance. Plus, it offers a smart office application that deals with team attendance, check-in, leaves, announcements, and more. If you’re looking for a way to unify your team’s files, chats, and calls, you can utilize the app’s enterprise address book to import the team’s organizational structure. Moreover, it can also be used as an online teaching platform where teachers can give lectures and students can use the hand-raise and chat feature to ask questions. You can use this option to converse with clients, customers, and team members for online presentations and confidential meetings. This free program also allows you to host voice and video conferences, with the latter mimicking the feeling of having a genuine face-to-face conversation. Plus, you can use the bank-level encrypted secret chat option for confidential chats where the conversation self-destructs 30 seconds after being read. You can create a group chat to seamlessly send messages to the entire team, and you can notify them about any urgent chats using the app’s ding feature. Boost your productivity with this free appĭingTalk comes with multiple features, one of which is instant messaging that allows you to have effective communication with your team members. Racallio Ryndoon of Tyrosh led an invasion fleet against Daemon's island kingdom in 110 AC. The Triarchy, also known as the Kingdom of the Three Daughters, fought with Prince Daemon Targaryen over the Stepstones in 106 AC. The Triarchy and the Daughters' WarĪfter the defeat of Volantis in the Battle of the Borderland, Tyrosh formed the Triarchy with Lys and Myr in 96 AC. ![]() Įlissa Farman visited Tyrosh while sailing Sun Chaser from Braavos to Oldtown. The Prince of Pentos and the Archon traveled to King's Landing to conduct negotiations in person, and they eventually agreed upon "A Treaty of Eternal Peace". In 58 AC both sides requested that King Jaehaerys I Targaryen to act as a mediator in negotiations to end the dispute. Tyrosh and Pentos began a war in 55 AC which significantly disrupted trade in the narrow sea. In 50 AC Ser Orryn Baratheon took service with the Archon of Tyrosh and wed his daughter a year later. When it was later suggested that King Jaehaerys I Targaryen should take the Archon's daughter as his bride, Alyssa argued against the match, fearing the Westerosi would never accept a foreign queen. The Archon of Tyrosh traveled to the Seven Kingdoms in 49 AC for the Golden Wedding of Rogar Baratheon and Alyssa Velaryon, accompanied by his maiden daughter. Īfter the flight of Dowager Queen Alyssa Velaryon from Dragonstone in 44 AC, Tyrosh was one of the places where she was rumored to have gone into hiding with her youngest two children. Lys and Myr both rose in rebellion against Volantis. Pentos came to the aid of the Tyroshi, as well as two Westerosi, the Storm King Argilac Durrandon of Storm's End and Aegon Targaryen of Dragonstone. Their attempt to take Tyrosh, however, made their empire collapse. During the Century of Blood, the Free City of Volantis thought to add Tyrosh to her conquests, after they had conquered Myr and Lys. Some dragonlords in Tyrosh survived the Doom of Valyria, but the citizens killed the dragons and their dragonriders. Tyroshi dyers learned to produce different colors of dyes by varying the diet of the snails. Thousands of merchants developed Tyrosh into a major city within a generation. They soon discovered in the nearby waters a unique variety of sea snail whose secretions were used to create a dye popular with Valyrian nobles. Tyrosh began as a military outpost of the Valyrian Freehold on a bleak stony isle to control shipping through the Stepstones. The fantastic helmets can be shaped like birds and animals and are chased with precious metals. Pear brandy and baked honeyfingers are produced in Tyrosh, and the city's craftsmen design gilded helms and filigreed armor. Slaves outnumber freeborn in Tyrosh three to one. They even sail north beyond the Wall, in search of free folk to enslave. Tyrosh is deeply involved in the slave trade, and Tyroshi slavers are known to be especially aggressive. Shortly after its founding, Tyrosh quickly grew out into a rich city, due to the discovery of a certain variety of sea snail. Tyrosh is a merchantile city, where trade is considered a much more honorable profession than arms. The daughter of the Archon during the early reign of King Jaehaerys I Targaryen was considered to have a delightful accent, however. Chett, who comes from Hag's Mire, thinks the Tyroshi speak a wet girly tongue, and Tyrion Lannister thinks that Collio Quaynis has a ludicrous accent. The Tyroshi tongue is a corrupted form of High Valyrian. Their inventors are known for creating ornate helmets and ingenious torture devices. The Tyroshi constantly fight Lyseni and Myrmen to gain control of the Stepstones and Disputed Lands. Their hats are considered queer by Westerosi. ![]() They love bright colors, dyeing their hair and beards brightly in colors such as blue, green, maroon, pink, purple, scarlet, and vermilion. Tyroshi are considered boisterous, flamboyant, and greedy. The Archon of Tyrosh is chosen from among the members of a conclave of the wealthiest and noblest of the city. R'hllor is worshipped in Tyrosh, as well as the Patternmaker and other gods. ![]() The Temple of Trios has a large statue of the three-headed Trios beside the temple's doors. There is no established faith in Tyrosh, and temples and shrines in honor of many different gods line the streets and waterfronts of the city. In the city lies the Fountain of the Drunken God. At the mouth of its harbor lies the Bleeding Tower. Tyrosh is far larger than the shadow city of Sunspear. Its inner walls are fused black dragonstone. Tyrosh, a fortress city protected by high walls, is on the northernmost and easternmost of the Stepstones. A coin of Tyrosh, as depicted by Nutchapol Thitinunthakorn in The World of Ice & Fire However, you didn't give us this information and you didn't contact us as well, regarding your pick up. The reason for that is, that driving licenses issued outside the EU are only recognized in the BRD for a temporary stay (up to 6 months, maximum 1 year with proof of a special permit). Since your driver's license was issued outside the EU, we needed you to inform us about the duration of your stay in Germany. When you made your second booking, you uploaded your ID check when you were already at the pick-up-location. However, this is only possible if your booking period hadn't ended yet. Within that period it is only possible to cancel your booking booking free of charge if the option "kostenfrei stornieren" was added to the booking. In this case we were not able to offer you a refund, as according to point 5.5 of our terms and conditions you should have canceled the booking at least 72 hours before your booking had started. People watching 3.Reply from Updated Jun 10, 2022ĭear customer, we have reviewed your case and found that you haven't picked up your first vehicle but still wanted to cancel your booking 2 days after your booking had ended.
![]() ![]() I do this sort of thing a lot as it gives your planting schemes a more natural appearance.Įremurus stenophylus ‘Bungei’ is in contrast to the others a much smaller plant. Instantly this suggests to me to plant both together in drifts to create a medley of pink tints and tones and subtle size variations. Slightly smaller is Eremurus ‘ Romance ’ in a similar colour tone. The flower spikes are easily two meters tall bearing soft salmon pink flowers. The largest-growing of these bold plants is well named, Eremurus robustus. Both taking their time to grow up in spring and only reach their full height in the second half of summer. I grow mine at the back of a border of large clumps of Miscanthus grasses and Sanguisorba. The trick with these truly spectacular plants is to find a sheltered spot in the garden that receives full sun in spring, but which becomes hidden by nearby perennials after flowering. After flowering the plants quickly die down which makes their placement in the garden challenging. The numerous flowers open from the base upwards resulting in a long and effective flowering season. In early summer, very tall strait flower spikes rise up that are covered in narrow tubular flowers that resemble the wagging tails of foxes to some and bottle brushes to others. From the wide spreading starfish-shaped tubers arise vigorous clumps of strap-like leaves in early spring. Its flowers and subsequent seed heads are perfect for adding to tall-growing prairie style planting schemes using ornamental grasses such as Miscanthus and Panicum.Įremurus are commonly called Cleopatra’s Needle in Holland which I think better describes them. It can reach more than two meters tall and will start flowering in late June and on into July. This time we have an even taller growing pompom allium that flowers much later than the others. Taller-growing and therefore having more impact are cultivars such as ‘ Ambassador ’ and ‘ Gladiator ’, but it is the less well known ‘ Summer Drummer ’ that needs your attention. Spring bulbs and shrubs have peaked and the main summer displays have yet to get into their stride - cue “Alliums”. They flower just at the end of spring when the garden is in a state of flux. These are not only exciting when planted in drifts to rise up amidst fields of perennials, but especially useful. The most familiar must be Allium ‘ Purple Sensation ’ and similar all with the characteristic pompom ball-shaped purple flower heads held boldly atop stiff bare stems up to a meter tall. Ornamental OnionsĪlliums are deservedly popular in contemporary gardens. When planted together, these black and white Persian Lilies will make a truly sophisticated focal point anywhere in our gardens. The form is identical and it is best appreciated near to hand. The white flowered Fritillaria persica ‘Alba’ is more subtle. They will introduce vertical accents to an otherwise horizontal planting plane. ![]() Standing amidst drifts of fresh coloured spring flowers they are nothing short of spectacular. They hang downwards and from a distance are effectively black. The stiffly upright flower stems rise high, carrying up to twenty plum-purple bell shaped flowers. There is also Fritillaria ‘Raddeana’, a close relative, with sophisticated pale yellow and green flowers for where something especially unique is desired.įritillaria persica takes the drama to another level. Fritillaria imperialis ‘Rubra’, terracotta and ‘ Lutea ’, yellow, are the most popular and deservedly so. Height is a relative thing and in the spring garden most bulbs and the newly emerging perennials are no more than knee high.Crown Imperials are tall enough to stand out from their surroundings, especially because of their bold bunch of dangling bell-flowers held clear of their foliage and any neighbouring tulips or daffodils. Their exceptional height allowing them to rise up amidst neighbouring perennials and shrubs. ![]() Growing tall and flowering in spring or summer they will inject a spectacular focal point into any planting scheme they are added to.Įach of these dramatic bulbs stands not only by way of the unusual shape of their flowers but also the colours they introduce. There is a select group of big bulbs that we can use to make a bold statement within our garden borders. |
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