Marfan syndrome

Marfan syndrome is a genetic disorder of the connective tissue. The degree to which people are affected varies. People with Marfan tend to be tall and thin, with long arms, legs and toes, they typically have flexible joints and scoliosis. The most serious complications involve the heart and aorta, with an increased risk of mitral valve prolapse and aortic aneurysm. Other affected areas include the lungs, eyes and the covering of the spinal cord. MFS is caused by a mutation in FBN1, one of the genes that makes fibrillin, which results in abnormal connective tissue, it is an autosomal dominant disorder. About 75% of the time, the condition is inherited from a parent, while 25% of the time it is a new mutation. Diagnosis is based on the Ghent criteria. There is no known cure for Marfan syndrome. Many people have a normal life expectancy with proper treatment. Management includes the use of beta blockers such as propranolol or atenolol or, if, not tolerated, calcium channel blockers or ACE inhibitors.

Surgery may be required to replace a heart valve. It is recommended. About 1 in 5,000 to 10,000 individuals have Marfan syndrome, it occurs in males and females. Rates are similar in different regions of the world, it is named after Antoine Marfan, a French pediatrician who first described the condition in 1896. More than 30 different signs and symptoms are variably associated with Marfan syndrome; the most prominent of these affect the skeletal and ocular systems, but all fibrous connective tissue throughout the body can be affected. Most of the visible signs are associated with the skeletal system. Many individuals with Marfan syndrome grow to above-average height, some have disproportionately long, slender limbs with thin, weak wrists and long fingers and toes. Besides affecting height and limb proportions, people with Marfan syndrome may have abnormal lateral curvature of the spine, thoracic lordosis, abnormal indentation or protrusion of the sternum, abnormal joint flexibility, a high-arched palate with crowded teeth and an overbite, flat feet, hammer toes, stooped shoulders, unexplained stretch marks on the skin.

It can cause pain in the joints and muscles. Some people with Marfan have speech disorders resulting from symptomatic high palates and small jaws. Early osteoarthritis may occur. Other signs include limited range of motion in the hips due to the femoral head protruding into abnormally deep hip sockets. In Marfan syndrome, the health of the eye can be affected in many ways, but the principal change is partial lens dislocation, where the lens is shifted out of its normal position; this occurs because of weakness in the ciliary zonules, the connective tissue strands which suspend the lens within the eye. The mutations responsible for Marfan syndrome cause them to stretch; the inferior zonules are most stretched resulting in the lens shifting upwards and outwards, but it can shift in other directions as well. Nearsightedness and blurred vision are common, but farsightedness can result if the lens is subluxated. Subluxation of the lens can be detected clinically in 80% of people with Marfan syndrome by the use of a slit-lamp biomicroscope.

If the lens subluxation is subtle imaging with high-resolution ultrasound biomicroscopy might be used. Other signs and symptoms affecting the eye include increased length along an axis of the globe, corneal flatness, strabismus and esotropia; the most serious signs and symptoms associated with Marfan syndrome involve the cardiovascular system: undue fatigue, shortness of breath, heart palpitations, racing heartbeats, or chest pain radiating to the back, shoulder, or arm. Cold arms and feet can be linked to MFS because of inadequate circulation. A heart murmur, abnormal reading on an ECG, or symptoms of angina can indicate further investigation; the signs of regurgitation from prolapse of the mitral or aortic valves result from cystic medial degeneration of the valves, associated with MFS. However, the major sign that would lead a doctor to consider an underlying condition is a dilated aorta or an aortic aneurysm. Sometimes, no heart problems are apparent until the weakening of the connective tissue in the ascending aorta causes an aortic aneurysm or aortic dissection, a surgical emergency.

An aortic dissection is most fatal and presents with pain radiating down the back, giving a tearing sensation. Because underlying connective tissue abnormalities cause MFS, the incidence of dehiscence of prosthetic mitral valve is increased. Care should be taken to attempt repair of damaged heart valves rather than replacement. Pulmonary symptoms are not a major feature of MFS. In spontaneous unilateral pneumothorax, air escapes from a lung and occupies the pleural space between the chest wall and a lung; the lung becomes compressed or collapsed. This can cause pain, shortness of breath, and, if not treated, death. Other possible pulmonary manifestations of MFS include sleep apnea and idiopathic obstructive lung disease. Pathologic changes in the lungs have been described such as cystic changes, pneumonia, bullae, apical fibrosis and congenital malformations such as middle lobe hypoplasia. Dural ectasia, the weakening of the connective tissue of the dural sac encasing the spinal cord, can result in a loss of quality of life.

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Adam Driver

Adam Douglas Driver is an American actor. He is the recipient of numerous accolades, including a Volpi Cup, as well as nominations for two Academy Awards, three Emmy Awards, a Tony Award. A former Lance corporal in the United States Marine Corps, he made his Broadway debut in Mrs. Warren's Profession and subsequently appeared in Man and Boy, he rose to prominence with a supporting role in the HBO comedy-drama series Girls, for which he received three consecutive Emmy nominations. Driver began his film career in supporting roles in films such as Lincoln, Frances Ha, Inside Llewyn Davis, Silence, he won the Volpi Cup for Best Actor for his lead role in the drama Hungry Hearts and starred as a poet in Jim Jarmusch's Paterson. Driver gained wider recognition for playing Ben Solo/Kylo Ren in the Star Wars sequel trilogy. In 2019, he returned to theater with Burn This, for which he was nominated for the Tony Award for Best Actor in a Play, he earned significant acclaim and nominations for two Academy Awards for his performance as a police detective in BlacKkKlansman and a theater director going through a divorce in Marriage Story.

Driver is the founder of Arts in the Armed Forces, a nonprofit that provides free arts programming to active-duty service members, military support staff, their families around the world. He founded the organization in 2008. Adam Douglas Driver was born on November 19, 1983, in San Diego, the son of Nancy Wright, a paralegal, Joe Douglas Driver, he has Dutch, German and Scottish ancestry. His father's family is from Arkansas and his mother's family is from Indiana, his stepfather, Rodney G. Wright, is a minister at a Baptist church; when Driver was seven years old, he moved with his older sister and mother to his mother's hometown of Mishawaka, where he graduated from Mishawaka High School in 2001. He sang in the choir at church. Driver has described his teenage self as a "misfit". After high school, he worked as a door-to-door salesman selling Kirby vacuum cleaners and as a telemarketer for a basement waterproofing company and Ben Franklin Construction, he was rejected. Shortly after the September 11 attacks, Driver enlisted in the United States Marine Corps and was assigned to Weapons Company, 1st Battalion, 1st Marines as an 81mm mortar man.

He served for eight months before dislocating his sternum while mountain biking. He was medically discharged with the rank of Lance Corporal, he attended the University of Indianapolis for a year before auditioning again for Juilliard, this time succeeding. Driver said that his classmates saw him as an intimidating and volatile figure, he struggled to fit into a lifestyle so different from the Marines, he was a member of the Drama Division's Group 38, where he met Joanne Tucker. He graduated with a BFA degree in 2009. After graduating from Juilliard, Driver began his acting career in New York City, appearing in both Broadway and off-Broadway productions. Like many aspiring actors, he worked as a busboy and waiter. Driver appeared in several television shows and short films, his first on-screen appearance was in the series finale of The Unusuals in 2009 as a repentant witness and reluctant accomplice to an unsolved assault. He made his feature film debut in Clint Eastwood's biographical drama J. Edgar in 2011.

In 2012, Driver was cast in the HBO comedy-drama series Girls, as the unstable Adam Sackler, the boyfriend of the lead character Hannah Horvath. He received three nominations for the Primetime Emmy Award for Outstanding Supporting Actor in a Comedy Series for his role; the same year, Driver played supporting roles in two critically acclaimed films, as telegraph and cipher officer Samuel Beckwith in Steven Spielberg's historical drama Lincoln, Lev Shapiro in Noah Baumbach's comedy-drama Frances Ha. He appeared in the drama Not Waving But Drowning and the romantic-comedy Gayby, he garnered major off-Broadway recognition for playing Cliff, a working-class Welsh houseguest in Look Back in Anger, winning the Lucille Lortel Award for Outstanding Featured Actor in a Play. In 2013, Driver appeared in the romantic-comedy What If, he played Al Cody, a musician, in the Coen Brothers' black comedy Inside Llewyn Davis, photographer Rick Smolan in the drama Tracks. In 2014, he played a despairing father, in the drama Hungry Hearts.

For his performance in Hungry Hearts, Driver won the Volpi Cup for Best Actor at the 71st Venice International Film Festival. In early 2014, Driver was cast as villain Kylo Ren in Star Wars: The Force Awakens; the Force Awakens was released on December 2015 to commercial and critical success. He reprised The Rise of Skywalker, his performance was positively received, with his character lauded as the best in the series: David Edelstein of Vulture wrote, "the core of The Last Jedi — of this whole trilogy, it seems — is Driver's Kylo Ren, who ranks with cinema's most fascinating human monsters." Peter Bradshaw of The Guardian highlighted Driver's performance in his review of The Force Awakens, calling him "gorgeously cruel and capricious... suited to Kylo Ren's fastidious and amused contempt for his enemies' weakness and

Stroud and District Combination Junior Cup

The Stroud and District Combination Junior Cup is an annual rugby union knock-out club competition organised by the Stroud and District Combination – one of the five bodies that make up the Gloucestershire Rugby Football Union. It was first introduced during the 1976–77 season, with the inaugural winners being Royal Agricultural College II. For the 2004–05 season a Junior Plate competition was introduced for teams that were knocked out of the first round of the Junior Cup, with Tetbury being the first winners; the Junior Cup is the second most important cup in Stroud District, behind the Senior Cup but ahead of the Bill Adams Cup. With only 9 member clubs the combination is one of the smallest in Gloucestershire; the Junior Cup is open to the 1st teams of lower ranked clubs in Stroud District as well as 2nd teams of the larger clubs. The format is a knock-out cup with quarter finals, semi-finals and a final to be held at a neutral ground between March–May. Clubs knocked out at the quarter-final stage enter the Junior Plate competition which has a semi-final and final – played at the same ground and date as the Junior Cup final.

Gloucestershire RFU Stroud & District Combination Senior Cup Stroud & District Combination Bill Adams Cup English rugby union system Rugby union in England Gloucestershire RFU