![]() Restricted Books only come around during these events and I recommend to waiting until you've collected all the Restricted Books before using them (and spending scrolls) to level up any of your profession skills. Here are a few extra tips for specifically for the Potter's Calamity event! Save your Restricted Books and Scrolls until the end While our best tips and tricks for Harry Potter: Wizards Unite are great for playing the game at all times. These special 3KM Portmanteaus will be the only way to find Luggage Trolley Witch Foundable fragments, which you'll need if you want to clear the entire event! Make sure you are on the lookout for these 3KM Portmanteaus so you can open them as fast as possible! Tips and tricks for the Harry Potter: Wizards Unite Back to Hogwarts Event (Week 2) These special Portmanteaus work just the same as any other Portmanteau - Stick either a Gold or Silver key in and walk to open! The Back to Hogwarts is the first Brilliant Event to feature 3KM Portkey Portmanteaus. Have you ever played Harry Potter: Wizard Unite? Did you enjoy the game? Tell us in the comment section below.Wizarding Challenges with Brilliant Runestones The community forum and all associated social media channels of the game will be closed on January 31, 2022. We will turn off the ability to make in-app purchases on December 6, 2021.Īnd about Harry Potter: Wizard Unite community: We have a variety of fun gameplay changes during the remaining few months, so you will have the full opportunity to enjoy your remaining Gold. You will be able to spend your remaining Gold balance and items before the game closes on January 31, 2022. Players will not be able to receive a refund on past purchases, except where otherwise required by law. If you would like to request your account data, please contact support to make this request by Januto allow time for our team to prepare the data.Ībout in-game currency and refund on purchases: Players who still have the game installed after this date will be unable to play. On January 31, 2022, all features and servers for Harry Potter: Wizards Unite will be turned off. Niantic also gives some important information regarding the end of the game.įor example, about what happens if users still have the game installed after the closing date: The events from January haven’t been announced yet. Half-Blood Prince Brilliant Event Part 2. ![]() Half-Blood Prince Brilliant Event Part 1.Dolores Umbridge Lethal Adversaries Event.Over the coming months, players will be able to participate in a few events, including the release of the remaining Lethal Adversaries: Spell Energy and Ingredients will appear on the map more frequently.1920s Portkey Portmanteaus will appear on the map more frequently and associated Fragment rewards will be doubled.Barrufio’s Brain Elixir potion will now award 3× player XP.The daily cap on sending and opening Gifts will be removed.All Potion Brew times will be reduced by 50% with Master Notes. ![]() With the game coming to an end, Niantic says players can look forward to some gameplay changes that will take effect as of today with all daily assignments with rewards increased, and: During this time, players have also worked alongside Hermione and Harry as part of the Statute of Secrecy Task Force to unravel the mystery behind the disappearance of the London Five and are now on the verge of putting an end to the Calamity. Over the years, millions of witches and wizards around the globe have embarked on real-world adventures, coming together to keep magic hidden safe within the wizarding world while joining forces to take on The Unforgivable. When we launched Harry Potter: Wizards Unite, our vision was to allow players to experience the wizarding world right in their real-world surroundings as they stepped outside and explored their neighborhoods. The long-awaited Harry Potter: Wizards Unite, which launched in 2019, is now being discontinued for good.Īccording to a blog post, Harry Potter: Wizards Unite will be removed from the App Store, Google Play, and Galaxy Store on December 6 with the game being closed on January 31, 2022. Not every Niantic game is a $5 billion hit like Pokémon GO. ![]()
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![]() ![]() All you'll need ahead of time is an unzipping program, such as WinRAR or 7Zip. We also categorized the list by specific topics (such as AI traffic packs and truck types), so if you're leaning towards a particular area, it'll be easier to locate the file.įor detailed info on how to install the mods, check out these instructions. That's why we hand-picked the very best and included them in the list below. So many, in fact, that it can be a little dizzying trying to sort through each and every one. Euro Truck Simulator 2, in addition to all of its out-of-the-box goodness, also has a huge amount of fantastic mods. There's just something incredibly fun about driving a gigantic, metallic behemoth that you've tweaked with your own hands across breathtaking sceneries and delivering a payload successfully (and on time).īut it doesn't stop there. And even if you've never climbed behind a simulated wheel before, it's likely you'll find something to enjoy thanks to the game's level of customization, and its open-ended environment. It's maintained a large, active community since its inception, and gamers from all over have lauded its in-game economy, career paths, and authentic European roadways. None of them can be bought in the current version.Euro Truck Simulator 2 is a deep and rewarding game, and it was met with favorable reviews when it released back in 2012. ![]() These trailers are only available in separate DLC. These trailers have been removed since the update 1.32. These trailers can not be bought and are only available at Quick Jobs, Freight Market and World of Trucks contracts. Requires Garage in: Denmark, Finland, Germany, Netherlands, Norway, Portugal, Spain, and Sweden Requires Level 23 + Garage in: Denmark, Finland, Germany, Netherlands, Norway, Portugal, Spain, and SwedenĭLC Feldbinder Trailer Pack: NameĬorrosive, toxic or otherwise dangerous liquids Requires Level 21 + Garage in: Denmark, Finland, Germany, Netherlands, Norway, Portugal, Spain, and Sweden Requires Level 20 + Garage in: Denmark, Finland, Germany, Netherlands, Norway, Portugal, Spain, and Sweden Requires Level 17 + Garage in: Denmark, Finland, Germany, Netherlands, Norway, Portugal, Spain, and Sweden The following preconfigured models are available at the trailer dealer: With the update 1.46, one new trailer was added: the Chemical Tanker. With the update 1.45, two new trailers were added: the Fuel Cistern and the Silo Cistern. With the update 1.43, one new trailer was added: the Steel Dumper. With the update 1.39, two new trailers were added: the Low Bed and the Low Loader. With the update 1.37, one new trailer was added: the Food Tank Paint. With the update 1.35, two new trailers were added: the Container Carrier and the Wooden Floor Logger. The Long Combination Vehicles page provides a table of the chain types and where it is allowed or prohibited so you know before you buy or configure your trailer. Each country will have restrictions on the number of axles used. All of them are available as Single, Double, B-double, and HCT chains. There are 11 different types: Curtainsiders, Dry Freighters, and Flatbeds, as well as Insulated, Refrigerated, Container Carrier, Log, Food Tank, Low Bed, Low Loader, and Dumper trailers. The player has to own a trailer to do Cargo Market jobs. They can be bought at the trailer dealer and customized at service shops. ![]() Ownable Trailers have been introduced since the update 1.32. ![]() ![]() ![]() Creatine use may increase the risk of developing CECS, and discontinuing this over-the-counter supplement may prevent the development of CECS symptoms.Īthletes with mild CECS may be able to continue to train. For athletes with very flat feet, arch supports may also help prevent CECS. This procedure is called a “fasciotomy.”Ĭhanging the way an athlete runs can prevent some types of CECS. An orthopedic surgeon can open up the tight fascial wall. If athletes cannot change their exercise routine, or if changing the exercise program does not help, CECS is a condition that can be treated with surgery. If athletes are willing to reduce the amount of exercise required for their sport, symptoms of CECS may go away. Treatment for CECS initially involves rest and exercise modification to reduce symptoms. In CECS, the pressure in at least one of the leg’s compartments will be higher than expected after exercise. To confirm that the problem is CECS, the physician will measure the pressure inside the compartments of the lower legs before and after exercise. The sports medicine physician will then re-examine the legs when the pain is present. Athletes may be asked to run to reproduce the pain. X-rays and MRI studies may be ordered to exclude other causes of lower leg pain, but will be normal in CECS. Some athletes may even feel like their foot is heavy or slaps the ground firmly while running.Ī sports medicine physician will examine the athlete’s legs, which will likely appear normal. Athletes also may develop a squeezing sensation over the involved compartment, and may experience numbness, tingling, and/or burning in the lower leg and foot. If athletes do not stop exercising and try to push through the discomfort, the pain will increase in severity. With each workout, the leg pain returns at the same time in the exercise routine. If athletes stop and rest, the pain gradually goes away. It is more common in running sports, basketball, gymnastics, soccer, field hockey, and dance.ĬECS causes a dull or crampy lower leg pain which starts at some point during exercise. Men and women athletes are equally affected by the problem. This results in pain in the compartment, as well as pain or tingling in the area of the lower leg supplied by the compartment’s nerves. However, if the fascial wall of a compartment is too tight, blood flow may be cut off to the nerves and muscles. ![]() During exercise, muscles swell and fluid enters the compartment spaces. The compartments contain muscles, nerves, and blood vessels. Each compartment has a wall that is surrounded by “fascia,” a thin layer of tissue that surrounds muscles. There are four compartments in the lower leg. Chronic exertional compartment syndrome (CECS) is one cause of exercise-related lower leg pain. ![]() ![]() Overall, there are many reasons why people love the P365.įor more information on the Sig Sauer P365, visit Sig’s website here. Weighing only 17.8 ounces, the P365 is an excellent choice for concealed carry. If you are looking for a higher capacity, optional 12- or 15-round extended magazines are available. Built with a lightweight stainless steel frame, this striker-fired pistol comes standard with not one but two 10-round flush magazines. The P365 is chambered in 9mm and features the standard sights and trigger that people know and love. It’s lightweight, compact, and known for its smooth shooting, which are only a few of the reasons it made our best micro 9mm list. ![]() The award-winning Sig P365 is one of the most popular concealed carry firearms to date. Or, if you are looking for holsters because you already own this popular pistol check out our holster options for the Springfield Armory Hellcat™ here. This highly reliable handgun won’t disappoint if you are looking for a high-capacity micro 9mm pistol.įor more information on the Springfield Armory Hellcat™, visit Springfield’s website here. While it comes with factory sights, they are well-liked in comparison to the standard sights on other firearms. This striker-fired handgun comes standard with an 11-round magazine or an extended 13-round magazine.Ĭhambered in 9mm, this small pistol weighs only 18.3 ounces and features an ergonomic grip that is comfortable for many shooters. You may know if for having the highest capacity of any micro-compact pistol, and you wouldn’t be wrong. The Springfield Armory Hellcat™ is a newer pistol that has taken the concealed carry market by storm. So, keep reading to check out our top picks for micro pistols and what makes each of them unique. But with so many micro handguns available, it can be challenging to pick the best micro 9mm. Many of these guns offer great ergonomics and an impressive magazine capacity. That, combined with the popularity of 9mm for concealed carry, makes micro 9 pistols some of the top choices for CCW on the market.īut don’t let their compact classification fool you. We don’t have to tell you that smaller guns are easier to conceal. ![]() ![]() After the Celtics fell down by five points, he helped push Boston to prevent it from getting out of hand. Jaylen Brown (17 points, 6 rebounds, 4 assists) helped by being aggressive regardless of the results. Robert Williams and Marcus Smart were the catalysts of everything good that happened, but there’s only so much they could impact. ![]() The other times, just like in last year’s Finals, the Sixers were forcing turnover after turnover on drives. When they were actually able to get a shot up, it was getting swatted or the layups weren’t dropping. After a solid start, the Celtics completely fell apart on both ends. The Celtics were probably hoping that Tatum would get it going in the third quarter, but that didn’t happen. The Rays are Diekman’s eighth big league team. The 36-year-old has a 25-30 record with 15 saves and a 3.96 ERA in 612 appearances during a 12-year career. This proved to be the right move, as almost all of the Celtics’ top players were sharp (despite Al Horford and Jayson Tatum struggling from the field), especially defensively. Diekman went 0-1 with a 7.94 ERA in 13 games with the Chicago White Sox this season before being designated for assignment on May 2 and released four days later. Joe Mazzulla stuck with just his seven best players tonight, opting to not expand opportunities to players like Grant Williams, Sam Hauser and Payton Pritchard. Hustle /HdGq6YS3aH- Boston Celtics May 12, 2023 Leading by example, Smart was the first to the floor and the ball multiple times tonight (per usual), and he augmented that with a surprising burst of scoring. Following the gut punch of a loss in Game 5, Smart explained in graphic terms how the team needed to fight tonight. The real star of the show in the first half was Marcus Smart. Malcolm Brogdon thankfully brought the missing scoring off the bench, tossing in 13 points in just the first half. His only first-half point came on a technical freethrow following a take foul. Unfortunately, the other thing preventing that from happening was Jayson Tatum putting up an offensive bagel: 1 point on 0-10 shooting. To their credit, they’ve been really solid all series long, and that’s what prevented the first half lead for Boston from ballooning. Labor activists and welfare groups panned the. On Monday, the city raised its minimum wage by 32 cents to about 5.1 per hour. Unfortunately, unlike in previous matchups against the Sixers, Philadelphia hasn’t been beating themselves for once. Financial hub Hong Kong is one of the worlds most expensive cities. The defense was crisp, and as has been the case all year, the Celtics lived (!) by the three. ![]() ![]() Download: Click Download and in a few moments you will receive the download dialog. I have also included a save for a popular glitch for player kills. The Celtics came out firing on all cylinders, opening up a 13-3 lead right away. 7 Days to Die is an early access survival horror video game set in an open world developed by The Fun Pimps. Item description: COMPLETE save set for 7 DAYS TO DIE CUSA03446 This is a small collection of 6 saves for every trophy in the game and instructions in the form of a roadmap. The Task Force consists of African Ministers' Council on Water (AMCOW), CDP Worldwide, Food and Agriculture Organization of the United Nations (FAO), Green Climate Fund, Global Water Partnership (GWP), Human Right 2 Water, IHE Delft Institute for Water Education, International Atomic Energy Agency (IAEA), International Association for Hydro-Environment Engineering and Research (IAHR), International Groundwater Resources Assessment Centre (IGRAC), International Labour Organization (ILO), Office of the United Nations High Commissioner for Human Rights (OHCHR), Public Services International (PSI), Sanitation and Water for All (SWA), Toilet Board Coalition, United Nations Convention to Combat Desertification (UNCCD), United Nations Development Programme (UNDP), United Nations Department of Economic and Social Affairs (UNDESA), United Nations Environment Programme (UNEP), UN Global Compact, United Nations Human Settlements Programme (UN-HABITAT), United Nations Office for Outer Space Affairs (UNOOSA), Women for Water Partnership (WfWP) and World Wildlife Fund (WWF).Smart bringing that energy /iiu9zFNnuT- Boston Celtics May 12, 2023 Inter Milan in the UK As has been standard for the last few years, BT Sport is your only option for catching the. Coordinated by United Nations Educational, Scientific and Cultural Organization (UNESCO), AquaFed - The International Federation of Private Water Operators and the World Youth Parliament for Water for World Water Day and by World Health Organization (WHO) and United Nations Children's Fund (UNICEF) for World Toilet Day. ![]() ![]() The smartphone is offered in choice of 16GB or 32GB of internal storage and 2GB of RAM. The Verizon Moto X features a 4.7-inch 720p AMOLED HD display and is powered by a custom dual-core 1.7Ghz Snapdragon S4 Pro processor with Motorola’s X8 Computing System and an Adreno 320 graphics processing unit (GPU).The Moto X ships with Android 4.2.2 Jelly Bean, but is expected to get an update to the latest Android 4.4 KitKat in the near future. Those are just a few of the Moto X’s best features. Flicking your wrist while holding the smartphone can also launch the Moto X’s 10-megapixel camera very quickly – the gesture wakes the Moto X and automatically launches the camera application. Simply say the phrase, “Ok, Google Now” and then issue a command, such as “Do I need an umbrella today?” and the Moto X will read you up-to-the-minute weather for your location. Motorola packed special sensors inside the Moto X that allow the smartphone to always be listening for your commands, even if it’s asleep. The deal brings the price of the Moto X down to $49.99 for the 16GB model and $99.99 for the 32GB one when signing a new 2-year contract. The company is now trying to tempt potential Verizon Moto X customers by offering the Moto X for $50 off when using the Moto Maker service. That was an exclusive AT&T feature until Motorola opened it up to Verizon, Sprint, and T-Mobile last week. One of the Moto X’s best features was that you could customize the smartphone by choosing the front, back, and accent colors. The Moto X has become a more attractive smartphone now that Verizon, Sprint, and T-Mobile customers can also customize it using the Moto Maker service. Washington: Motorola is offering its flagship device Verizon Moto X with Moto Maker customization for $49.99.Īccording to report, the handset will be available from November 18. ![]() ![]() ![]() She was an international judge at The Rhuby Liqueur Pink Your Drink global final in Sweden and also judged The Marie Brizard Masters here in New York. Paula Lukas:Ī few years ago Paula entered her first cocktail competition and was chosen as a national finalist for Rhuby Liqueur Pink Your Drink, She has also competed in the Queen Bee Cocktail classic and was a Northeast Regional semi finalist in The Copper and Kings Mixtape Competition. Her home bar, Clover Club, is Brooklyn’s revered craft cocktail establishment spearheaded by Julie Reiner, and where Mix returns to when she’s not making the world a better place. At the end of the day, it’s the way a bar makes you feel special that you’ll remember.” Ivy Mix:Ĭurrently, she is the Head Bartender and Owner at Leyenda, Ivy Mix maintains her position as one of the best bartenders in her field. “If you’re at a great bar, an amazing cocktail is just one element of it. “The key for me is the hospitality we provide,” says Rosito of his role as Bar Manager at Le Sirenuse Bar at Four Seasons Hotel at The Surf Club, Miami, which he assumed in early 2018. In 2016 Jacopo Rosito was nominated by the Golden Gate Restaurant Association for a Saucy Award as Manager of the Year, and the following year the San Francisco Chronicle honoured him as a “Bar Star.” In 2015, Rosito became the National Italian Champion at the Bols Around the World competition, and in 2016 he won the Disaronno Mixing Star and represented the US during the Bar Convention in Berlin, where he presented his winning cocktail The Magic Oak. These days, Tom works at the Fontaine Bleau Hotel on Miami Beach. Outside of the bar, Tom has featured in publications such as The New York Times, Wall Street Journal and Vice,and has conducted seminars and workshops in cities as far as Singapore, Moscow, and Mumbai. A career bartender for over ten years, Tom has worked at some of the top bars in Edinburgh, London and New York, and has consulted on drink strategies for brands such as Monkey Shoulder, Dewars and Boodles Gin.Īt the end of 2014 Tom moved across the Atlantic to work at the world-famous Attaboy, the acclaimed bar that occupies the old Milk & Honey site on 134 Eldridge Street in Manhattan, and went onto work some of New York’s best bars including Fresh Kills, Dutch Kills, Sunday in Brooklyn and Donna Cocktail Club He is best known for winning the Bacardi Legacy Global Cocktail Competition with his drink the Maid in Cuba. Tom Walker is an internationally renowned bartender, public speaker and drinks writer who has been in the food and beverage industry for over 15 years. She is a very active trend setter and community leader in Portland with a fan following of her own. She is also a national competitor and Speed Rack finalist with international bartending experience at high profile cocktail bars. She has been two time cocktail fight champ. Natasha Mesa is the head bartendress at Portland hot spot, Deadshot PDX. Eryn is a Speed Rack National champion, graduate of the BAR 5-day program and is currently working on her BAR Masters. She has been featured in numerous publications, including Imbibe, New York Times, Millennium, Cosmopolitan, and Maxim to name a few. Hailing from Portland, Oregon, Eryn Reece has worked in the restaurant industry since she was 16, Eryn is excited to raise awareness for female bartenders, and is a founding member and secretary of LUPEC NYC, and a Dames Hall of Fame Inductee by Tales of the cocktail. He built his talents and techniques through tending at New York’s famous Rainbow Room in the 80s where he mixed classic and great tasting, ready to drink cocktails. Known as one of the best mixologists in the world and "King Cocktail," Dale DeGroff is a true expert behind the art of cocktails. Here are 21 top Bartenders of the world: Dale DeGroff: Let us move into 2020 by recognizing some of the best bartending talents to be found globally. ![]() Bartending is not an easy job, it takes years of practice to become a bartender worthy of recognition. ![]() ![]() ![]() Note: Bans will not be reversed if the post/comment in question has been deleted from your history. You may appeal this initial ban by messaging the moderators and agreeing not to break the rules again. Note that moderators will use their own discretion to remove any post that they believe is low-quality or not considered a LPT.īans are given out immediately and serve as a warning. ![]() Posts or comments that troll and/or do not substantially contribute to the discussion may be removed.Do not post tips that are advertisements or recommendations of products or services.Do not post tips in reaction to other posts.Posts concerning the following are not allowed: religion, politics, relationships, law and legislation, parenting, driving, medicine or hygiene (including mental health).Do not post tips that are based on spurious, unsubstantiated, or anecdotal claims.Do not post tips that could be considered common sense, common courtesy, unethical, or illegal.The quality of the boxes can vary greatly, so use your best judgment. People frequently post curb alerts letting you know when and where they’ll be leaving free boxes out on the curb. The tip and the problem it solves must be explained thoroughly. Craigslist is a dumping ground for free stuff and moving boxes are no exception. Posts must begin with "LPT" or "LPT Request” and be flaired.No rude, offensive, racist, homophobic, sexist, aggressive or hateful posts/comments."No snowflake in an avalanche ever feels responsible." Keep in mind that an aphorism is not a LPT.Īn aphorism is a a short clever saying that is intended to express a general truth or a concise statement of a principle. “A marriage proposal should not come as a big surprise, despite what you may have seen in the movies.” Go under the 'for sale' section, click on 'free' and type in the word 'boxes.' All. No matter what city you live in, Craigslist can get you access to free moving boxes. There are more potential spots in your own neighborhood than you think. “Always be prepared to leave your employer because they are prepared to leave you.” Here are 7 options for obtaining free moving boxes: visit grocery stores, liquor stores, bookstores, office supply stores, check craigslist, Facebook marketplace, and Freecycle. Scoring free moving boxes doesn't have to get challenging if you know where to look. Advice is offering someone guidance or offering someone a recommendation. Keep in mind that giving someone advice is not the same as giving someone a LPT. Scoring free moving boxes doesnt have to get challenging if you know where to look. All standard U-Haul moving boxes are available in bundles of 10, 15, 20, and 25, depending on the box. If you end up with more than you need, U-Haul will buy back your unused boxes for a full refund. A Life Pro Tip (or an LPT) is a specific action with definitive results that improves life for you and those around you in a specific and significant way. Buying moving boxes in bulk is an easy way to get new moving boxes but still save money. ![]() ![]() ![]() Luckily for Dale, the Internet didn't exist in 1951, and only one person in town - Myra Fleener (Barbara Hershey) - knows how to look up information in a library. Remember, this is a coach with a history of assault, having punched one of his own players at Ithaca College and receiving a lifetime ban for it. Instead of being the calming influence his team needs, he instigates a brawl by dressing down the referee, taunting the opposing coach and slapping - yes, slapping - the hand of a kid on the other team. Take Hickory's first road game of the season, a tough, physical battle where emotions are running high. (I prefer the longer-view approach of Herman Boone in "Remember the Titans," when he quietly asks Gerry Bertier, "You know who your daddy is, doncha?")īut put Coach Dale on the bench, with folks in the stands and pressure in the air, and he crumbles. You can even say he's a strong leader, if you think running off two players in the first minute of your first practice is the way to establish authority. Having his players measure the height of the basket and the distance to the free-throw line before the state final is genius. Just watch Gene Hackman snap off one perfect chest pass after another, all the while imploring the boys to "pop it, pop it." He's a brilliant psychologist. ![]() Norman Dale is a great teacher of fundamentals. MGM"Hoosiers" is one of the finest sports movies ever - in spite of Norman Dale's lack of expertise.ĭon't get me wrong. ![]() ![]() ![]() PrognosisĮarly diagnosis, timely pubertal induction, successful transition to adult care, and long-term follow-up by a multidisciplinary team are key elements in the achievement of optimal growth, psychosocial milestones and fertility.Light micrograph of the chromosomes (karyotype) of a female with Turner syndrome. Lifelong follow-up is necessary, especially for the regular evaluation of aortic diameters. Hormonal replacement therapy with estrogen and progesterone/progestin is prescribed until the mean age of natural menopause (51 yrs). A majority of patients require induction of puberty with estrogen replacement therapy at gradually increased doses, followed within 2 years by progestin supplementation to minimize endometrial hyperplasia and the long term risk of endometrial carcinoma. This requires monitoring of insulin-like growth factor 1 and glycemia, due to a higher risk of glucose intolerance. Management and treatmentĮarly diagnosis is beneficial, as it is recommended to start recombinant growth hormone therapy (GH) around 4-6 years and preferably before 12-13 years in case of growth failure (< 50th percentile) or short stature. Karyotyping must be repeated postnatally to confirm the diagnosis. Noninvasive screening of circulating cell-free fetal DNA in maternal serum, amniocentesis or chorionic villus sampling can detect X chromosome abnormalities. Typical forms with associated malformations can be diagnosed during ultrasound examination showing fetal abnormalities including diffuse edema, increased nuchal translucency, cystic hygroma, and left-sided obstructive cardiac anomalies. Differential diagnosisĭifferential diagnosis includes Noonan syndrome 46,XX gonadal dysgenesis 46,XY complete gonadal dysgenesis, SHOX-related short stature, as well as a single deletion of the long arm of the X chromosome downstream of Xq24. A second cell line study, such as buccal or urinary cell FISH analysis, may also be helpful. Suggestive clinical findings are confirmed by lymphocytic karyotype, demonstrating a 45,X cell line or deletion of the short arm (Xp) of the X chromosome. The presence of Y material is related to the occurrence of gonadoblastoma. Haploinsufficiency of the SHOX gene is a well-established cause of short stature. Patients with an X isochromosome have a higher risk of liver and autoimmune diseases, while those with a ring chromosome are more prone to growth retardation and metabolic disorders. Clinical signs are more severe in patients with 45,X monosomy than in 45,X/46,XX or 45,X/46,XX/47,XXX mosaicism. X monosomy is the most common underlying etiology, while some cases have mosaicism or structural X chromosome anomalies. Variable comorbidities include obesity, insulin-resistance, conductive and sensorineural hearing impairment associated with repeated otitis media during childhood, and hyperlipidemia. Intellectual disability is rarely reported, but patients may present impaired social skills and subtle cognitive difficulties. Other manifestations are renal defects, congenital lymphedema, liver steatosis or other hepatic diseases, ophthalmologic problems, hypothyroidism and autoimmune disorders. ![]() Monitoring of aortic root diameters indexed to body surface area is recommended, since aortic bicuspidy, coarctation and hypertension may lead to aortic root dilatation and ultimately to aortic dissection. Patients may have a low posterior hairline, webbed neck, broad chest, hypoplastic nails, skeletal anomalies, congenital cardiovascular abnormalities (bicuspid aortic valves, aortic coarctation) as well as acquired vascular features, such as hypertension (50% of adult cases). Around 5-7% are able to have a natural pregnancy. Approximately 30% of patients will undergo the first pubertal stages such as breast development, 20% will experience spontaneous menarche and 95% have primary ovarian insufficiency before the age of 40. However, clinical features are heterogeneous and some may be milder or even absent in front of a 45,X/46,XX mosaic karyotype. Patients typically present with significant statural growth retardation during childhood and/or primary ovarian insufficiency, often associated with homogeneous X monosomy. Incidence among all conceptions is higher, since aneuploidy is a common cause of spontaneous abortion. Prevalence is about 5/10 000 live female births. ![]() |
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