Hale Boggs and Marfan syndrome: Difference between pages

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{{Infobox_Disease

{{Infobox_Congressman

| Name = Marfan syndrome

| name= Hale Boggs

| Image = Marfansyndrome.jpg

| image name= HaleBoggs.jpeg

| Caption =

| state= [[Louisiana]]

| DiseasesDB = 7845

| district= [[Louisiana's 2nd congressional district|2nd]]

| ICD10 = {{ICD10|Q|87|4|q|80}}

| term= 1941 - 1943 (1st)<br>1947 - 1972 (2nd)

| ICD9 = {{ICD9|759.82}}

| preceded= [[Paul H. Maloney]] (1st)<br>[[Paul H. Maloney]] (2nd)

| ICDO =

| succeeded= [[Paul H. Maloney]] (1st)<br>[[Lindy Boggs]] (2nd)

| OMIM = 154700

| date of birth= [[February 15]], [[1914]]

| MedlinePlus = 000418

| place of birth= [[Long Beach, Mississippi]]

| eMedicineSubj = ped

| date of death= [[Death in absentia|presumably]] [[October 16]], [[1972]]

| eMedicineTopic = 1372

| place of death= [[Alaska]]

| eMedicine_mult = {{eMedicine2|orthoped|414}}

| spouse= [[Lindy Boggs]]

| MeshID = C17.300.500

| profession= lawyer, politician

}}

| religion=

| party= [[Democratic Party (United States)|Democrat]]

|}}

'''Thomas Hale Boggs, Sr.,''' ([[February 15]], [[1914]] &ndash; Undetermined; [[Death in absentia|presumably]] [[October 16]], [[1972]]) was an [[United States|American]] [[Democratic party (United States)|Democratic]] [[politician]] and a member of the [[United States House of Representatives]] for [[Louisiana]]. He was the [[Majority Leader of the United States House of Representatives|House Majority Leader]].

'''Marfan syndrome''' is an [[autosomal dominant]] genetic disorder of the [[connective tissue]] characterized by disproportionately long [[limb]]s, long thin fingers, a relatively tall stature, and a predisposition to cardiovascular abnormalities, specifically those affecting the [[heart valves]] and [[aorta]]. The [[disease]] may also affect numerous other structures and organs &mdash; including the [[lung]]s, [[eye]]s, dural sac surrounding the [[spinal cord]], and [[hard palate]]. It is named after [[Antoine Marfan]], the [[France|French]] [[pediatrician]] who first described it in 1899.

In [[1972]], while he was still Majority Leader, the twin engine airplane in which Boggs was traveling over a remote section of [[Alaska]] [[Missing person|disappeared]]. The [[plane crash|plane presumably crashed]] and was never found. Congressman [[Nick Begich]] was also presumed killed in that accident.

==Epidemiology==

==Early start in politics==

Marfan syndrome affects males and females equally,<ref name="marorg">{{Cite web|url=http://www.marfan.org/nmf/GetSubContentRequestHandler.do?sub_menu_item_content_id=6&menu_item_id=3|title=The role of heredity and family history|accessdate=2007-01-11|publisher=National Marfan Foundation|year=1999}}</ref> and the mutation shows no geographical bias. Estimates indicate that approximately 60 000 (1 in 5000, or 0.02% of the population)<ref name="marorg"/> to 200 000<ref name="mednet">{{Cite web|url=http://www.medicinenet.com/script/main/art.asp?articlekey=63689|title=New, Deadly Relative of Marfan's Syndrome Discovered|accessdate=2007-01-11|publisher=MedicineNet.com|year=2006}}</ref> Americans have Marfan syndrome. Each parent with the condition has a 50% chance of passing it on to a child due to its [[autosomal dominant]] nature. Most individuals with Marfan syndrome have another affected family member, but approximately 15-30% of all cases are due to ''[[de novo mutation|de novo]]'' [[genetic mutation]]s<ref name="robspath">{{cite book | title=Robbins Pathologic Basis of Disease| last=Cotran| coauthors=Kumar, Collins| publisher=W.B Saunders Company| ___location=Philadelphia| id=0-7216-7335-X}}</ref> &mdash; such spontaneous mutations occur in about 1 in 20 000 births. Marfan syndrome is also an example of [[dominant negative mutation]] and [[haploinsufficiency]].<ref name="Judge_et_al_2004">{{cite journal | last = Judge | first = Daniel P. | coauthors = Nancy J. Biery, Douglas R. Keene, Jessica Geubtner, Loretha Myers, David L. Huso, Lynn Y. Sakai, Harry C. Dietz | title = Evidence for a critical contribution of haploinsufficiency in the complex pathogenesis of Marfan syndrome. | journal = The Journal of Clinical Investigation | volume = 114 | issue = 2 | pages = 172-181 | doi = 10.1172/JCI200420641 | id = PMID 15254584 | url = http://www.jci.org/cgi/content/full/114/2/172 | accessdate = 2007-02-15}}</ref><ref name="Judge_et_al_2005">{{cite journal | last = Judge | first = Daniel P. | coauthors = Harry C. Dietz | title = Marfan's syndrome. | journal = Lancet | volume = 366 | issue = 9501 | pages = 1965-76 | year = 2005 | doi = 10.1016/S0140-6736(05)67789-6. | id = PMID 16325700 | url = http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pubmed&pubmedid=16325700 | accessdate = 2007-02-15}}</ref> It is associated with [[incomplete penetrance]], therefore not all persons carrying the mutation develop the disease.

== Pathogenesis ==

Born in [[Long Beach, Mississippi]], Boggs was educated at [[Tulane University]] where he received a [[bachelor's degree]] in [[journalism]] in [[1934]] and a [[law]] degree in [[1937]]. He first practiced law in [[New Orleans]], but soon became a leader in the movement to break [[Huey Long]]'s political power in the area. A [[United States Democratic Party|Democrat]], Boggs was elected to the U.S. House for the Second District and served from 1941 to 1943. At the time he was elected he was, at twenty-six, the youngest member of Congress. After an unsuccessful re-election bid in [[1942]], Boggs joined the [[United States Navy]] as an [[ensign]]. He served the remainder of [[World War II]].

Marfan syndrome has been linked to a defect in the ''FBN1'' [[gene]] on [[chromosome]] 15,<ref>{{cite journal | author = McKusick V | title = The defect in Marfan syndrome. | journal = Nature | volume = 352 | issue = 6333 | pages = 279-81 | year = 1991 | id = PMID 1852198}}</ref> which [[Genetics|encodes]] a [[glycoprotein]] called [[fibrillin]]-1. Fibrillin is essential for the formation of the [[elastic fiber]]s found in connective tissue, as it provides the scaffolding for [[tropoelastin]].<ref name="robspath">{{cite book | title=Robbins Pathologic Basis of Disease| last=Cotran| coauthors=Kumar, Collins| publisher=W.B Saunders Company| ___location=Philadelphia| id=0-7216-7335-X}}</ref> Elastic fibers are found throughout the body but are particularly abundant in the [[aorta]], [[ligament]]s and the [[Zonule of Zinn|ciliary zonule]]s of the eye, consequently these areas are among the worst affected. Without the structural support provided by fibrillin many connective tissues are weakened, which can have severe consequences for support and stability.

A related disease has been found in [[mouse|mice]], and the study of mouse fibrillin synthesis and secretion, and connective tissue formation, has begun to further our understanding of Marfan syndrome in humans. It has been found that simply reducing the level of normal fibrillin-1 causes a Marfan-related disease in mice.<ref name="micefib">{{cite journal | author=Lygia Pereira, ''et al.''| title=Pathogenetic sequence for aneurysm revealed in mice underexpressing fibrillin-1| journal=Proceedings of the National Academy of Sciences| year=1999| volume=96| issue=7| page=3819-3823| url=http://www.pnas.org/cgi/content/full/96/7/3819}}</ref>

==Political career==

After the war, Boggs began his political comeback. He was again elected to Congress in 1946 and was then re-elected 13 times, once just after he disappeared, but before he was presumed dead. In his closest contest -- 1968, Boggs faced his third challenge from [[Republican Party (United States)|Republican]] [[David C. Treen]], who went on to win the Louisiana governorship in 1979. Treen received 77,633 votes (48.8 percent) to Boggs's 81,537 ballots (51.2 percent). Treen attributed Boggs's victory to the supporters of former [[Alabama]] [[Governor of Alabama|Governor]] [[George C. Wallace]], who ran for president on the [[American Independent Party]] ticket. Treen said that Wallace supporters "became very cool to my candidacy. We couldn't really believe they would support Boggs, but several Democratic organizations did come out for Wallace and Boggs, and he received just enough Wallace votes to give him the election." Republican officials seemed convinced that fraudulent votes in some Orleans Parish precincts benefited Boggs and that Treen may have actually won the election. There were rumors of election officials who cast votes for people who did not show up at the polls and signed for them in the precinct registers.

[[Transforming growth factor]] beta (TGFβ) plays an important role in Marfan syndrome. Fibrillin-1 binds TGFβ and inactivates it. In Marfan syndrome, reduced levels of fibrillin-1 allow activated TGFβ to damage the lungs and heart. A defect in the gene ''TGFβR2'' on [[chromosome]] 3, a [[receptor protein]] of TGFβ, has also been related to Marfan syndrome.<ref name="tgf2beta">{{Cite web|url=http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=gene&dopt=full_report&list_uids=7048|title=TGFBR2 transforming growth factor, beta receptor II|accessdate=2007-01-11|publisher=NCBI|year=2007|author=Entrez Gene|format=Entrez gene entry}}</ref> Marfan syndrome can often be confused with [[Loeys-Dietz syndrome]], a similar connective tissue disorder resulting from mutations in the TGFβ receptor genes ''TGFβR1'' and ''TGFβR2''.<ref name="loeysdietz">{{Cite web|url=http://www.marfan.org/nmf/GetContentRequestHandler.do?menu_item_id=84|title=Related Disorders: Loeys-Dietz |accessdate=2007-01-11|publisher=National Marfan Foundation}}</ref>

Boggs unsuccessfully sought the Democratic gubernatorial nomination in 1951-1952. He lost out to a field of opponents, including the eventual winner, Judge [[Robert F. Kennon]] of [[Minden]], whom Boggs supported in the runoff. Kennon "adopted" Boggs's intraparty choice for lieutenant governor, [[C. E. "Cap" Barham]] of [[Ruston, Louisiana|Ruston]] in [[Lincoln Parish]]. In that race, one of the candidates, "Miss" [[Lucille May Grace]], filed suit in an unsuccessful attempt to remove Boggs from the ballot on the grounds that he was either a "communist" or had been a "communist sympathizer" in his earlier years. As it turned out, Miss Grace's maneuver was arranged by Boggs's long-term political rival, Judge [[Leander Perez|Leander H. Perez]], the political "boss" of [[Plaquemines Parish]].

==Symptoms==

During his tenure in Congress, Boggs was an influential player in the government. After ''[[Brown v. Board of Education]]'' he signed the [[Southern Manifesto]] condemning desegregatio in the 1950s and opposed the 1964 Civil Rights Act. Yet unlike most Southern Congressmen of his era, he supported the 1965 Voting Rights Act and the 1968 Open Housing Act. He was instrumental in passage of the [[interstate highway]] program in 1956, and was a member of the [[Warren Commission]] in 1963-1964.

There are no signs or symptoms that are unique to Marfan syndrome. It is usually a single apparent sign or symptom that leads doctors to look for others and eventually to diagnose the syndrome, which affects connective tissue in diverse organs and systems. Even affected individuals in the same family might exhibit various combinations and severities of symptoms.

===Skeletal system===

He served as [[Majority Whip of the United States House of Representatives|Majority Whip]] from 1961 to 1970 and as majority leader (from January 1971). As majority whip, he ushered much of [[Lyndon Johnson|President Johnson's]] [[Great Society]] legislation through Congress. Boggs is one of numerous public officials known to have drinking problems during the time.<ref name=Drinking>{{cite web| url=http://www.findarticles.com/p/articles/mi_m1316/is_n12_v19/ai_6306545

The most readily visible signs may be associated with the skeletal system. Many individuals with Marfan syndrome grow to larger than normal height and have long, slender limbs, fingers, and toes. An individual's arms may be disproportionately long. In addition to affecting height and limb proportions, Marfan syndrome can produce other skeletal signs. Abnormal curvature of the [[Vertebral column|spine]] ([[scoliosis]]) is common, as is abnormal indentation ([[pectus excavatum]]) or protrusion ([[pectus carinatum]]) of the [[sternum]]. Other signs include abnormal joint flexibility, a high [[palate]], [[malocclusions]], flat feet, stooped shoulders, and unexplained [[stretch marks]] on the skin. Some people with Marfans have [[speech disorder|speech impediments]] resulting from symptomatic high palates and small jaws.

|title=Governing under the influence; Washington alcoholics: their aides protect them, the media shields them |work=Washington Monthly |date=January 1988 |author=Steven Waldman}}</ref>

===Eyes===

His influence also led to charges of corruption. Controversy surrounded him, when a contractor who remodeled his home in [[Bethesda, Maryland|Bethesda]], Maryland at a reduced cost sought his help for obtaining a $5 million extra payment for building a garage adjacent to the [[United States Capitol]] building.{{fact|date=April 2007}}

Marfan syndrome can also seriously affect the eyes and vision. [[myopia|Nearsightedness]] and [[astigmatism]] are common, but farsightedness can also result. Periodic eye exams can lead to an [[ophthalmologist]] or [[optometrist]] discovering dislocation, or [[subluxation]], of the crystalline [[lens (anatomy)|lens]] in one or both eyes ([[ectopia lentis]]) by carefully observing these structures using a [[Slit lamp|slit-lamp]] biomicroscope. This can be differentiated from the similar condition [[homocystinuria]], where the dislocation is inferonasal; in Marfan's the dislocation is superotemporal. Sometimes eye problems appear only after the weakening of connective tissue has caused [[retinal detachment|detachment of the retina]].<ref name="mayo-gen">{{Cite web|url=http://www.mayoclinic.com/health/marfan-syndrome/DS00540/DSECTION=2|title=Marfan Syndrome|accessdate=2007-01-12|publisher=Mayo Clinic}}</ref> Early onset [[glaucoma]] can be another complication.

===Cardiovascular system===

==Disappearance in Alaska==

The most serious conditions associated with Marfan syndrome involve the cardiovascular system. Undue fatigue, shortness of breath, [[heart palpitations]], [[tachycardia|racing heartbeats]], or [[angina|pain in the left chest, back, shoulder, or arm]], can bring an individual into the doctor's office. A [[heart murmur]] heard on a [[stethoscope]], an abnormal reading on an [[electrocardiogram]], or symptoms of [[angina]] can lead a doctor to order an [[echocardiogram]]. This can reveal signs of leakage or [[prolapse]] of the mitral or aortic [[heart valve|valves]] that control the flow of blood through the heart. 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 [[aorta|ascending aorta]] causes an [[aortic aneurysm]] or even [[aortic dissection]]. During pregnancy, even in the absence of preconceived cardiovascular abnormality, women with Marfan syndrome are at significant risk of acute [[aortic dissection]], which can be lethal if untreated. For this reason, women with Marfan syndrome should recieve a thorough assessment prior to conception, and [[echocardiography]] should be performed every 6-10 weeks during pregnancy, to assess the aortic root diameter. Most women however tolerate pregnancy well and safe vaginal delivery is possible.<ref name="emed">{{Cite web|url=http://www.emedicine.com/ped/fulltopic/topic1372.htm#section~Miscellaneous|title=Marfan Syndrome, special concerns|accessdate=2007-06-25}}</ref>

===Disappearance and 39 day search===

As Majority Leader, Boggs often campaigned for others. On [[October 16]], [[1972]], he was aboard a twin engine [[Cessna 310]] with Representative [[Nick Begich]] when it disappeared during a flight from [[Anchorage, Alaska|Anchorage]] to [[Juneau, Alaska]]. Begich's aide, Russell Brown, and the pilot, Don Janz, were the only others on board.<ref name=FamousMissing>{{cite web|accessdate=2007-04-15

|url=http://www.check-six.com/lib/Famous_Missing/Boggs.htm

|title=Hale Boggs - Missing in Alaska |work=Famous Missing Aircraft

|publisher=Check-Six}}</ref> The four were heading to a campaign fundraiser for Begich who was facing a possible tight race in the November 1972 general election against the Republican candidate [[Don Young]]. (Begich won the 1972 election posthumously with 56% to Young's 44%.)

===Lungs===

In the largest search ever mounted by the U.S. military,{{fact|date=2007}} [[United States Coast Guard|Coast Guard]], [[United States Navy|Navy]], and [[United States Air Force|Air Force]] planes searched for the party. On [[November 24]], [[1972]], after 39 days, the search was abandoned. Neither the wreckage of the plane, nor the mens' [[Human remains|remains]] were ever found. The accident prompted Congress to pass a law mandating emergency locator transmitters (now called [[emergency position-indicating rescue beacon]]s) in all U.S. civil [[aircraft]].

Marfan syndrome is a [[risk factor]] for spontaneous [[pneumothorax]]. In spontaneous unilateral pneumothorax, air escapes from a lung and occupies the [[pleural]] space between the chest wall and a [[lung]]. The lung becomes partially compressed or collapsed. This can cause pain, shortness of breath, [[cyanosis]], and, if not treated, death. Marfan syndrome has also been associated with [[sleep apnea]] and [[idiopathic]] obstructive lung disease.

===Central nervous system===

Both Boggs and Begich were re-elected that November. House Resolution 1 of [[January 3]], [[1973]] officially recognized Boggs's [[Death in absentia|presumed death]] and opened the way for a special election.

Another condition that can reduce the quality of life for an individual, though not life-threatening, is [[dural ectasia]], the weakening of the connective tissue of the dural sac, the membrane that encases the [[spinal cord]]. Dural ectasia can be present for a long time without producing any noticeable symptoms. Symptoms that can occur are lower [[back pain]], leg pain, [[abdominal pain]], other neurological symptoms in the lower extremities, or [[headaches]]. Such symptoms usually diminish when the individual lies flat on his or her back. These types of symptoms might lead a doctor to order an [[X-ray]] of the [[lumbar|lower spine]]. Dural ectasia is usually not visible on an X-ray in the early phases. A worsening of symptoms and the lack of finding any other cause should eventually lead a doctor to order a upright [[MRI]] of the lower spine. Dural ectasia that has progressed to the point of causing these symptoms would appear in a upright MRI image as a dilated pouch that is wearing away at the [[lumbar vertebrae]].<ref name="mayo-gen" /> Other spinal issues associated with Marfan include degenerative disk disease and spinal cysts.

==Management==

===Speculation, suspicions, and theories===

There is no cure for Marfan syndrome, but life expectancy has increased significantly over the last few decades. The syndrome is treated by addressing each issue as it arises, and, in particular, considering prophylactic medication, even for young children, to slow progression of aortic dilation.

The events surrounding Boggs's death have been the subject of much speculation, suspicion, and numerous [[conspiracy theories]]. These theories often center around his involvement with the [[Warren Commission]], but some tie his death to alleged corruption charges or his outspoken opposition to powerful [[Federal Bureau of Investigation]] director [[J. Edgar Hoover]]. Some people, including several of Begich's children, have suggested that [[Richard Nixon]] had a hand in Boggs's death in order to thwart the [[Watergate]] investigation. None of these theories has ever been proven.

Regular checkups by a [[cardiologist]] are needed to monitor the health of the heart valves and the aorta. The goal of treatment is to slow the progression of aortic dilation and damage to heart valves by eliminating [[Cardiac arrhythmia|arrythmias]], minimizing the [[heart rate]], and minimizing [[blood pressure]]. [[Beta blocker]]s have been used to control [[Cardiac arrhythmia|arrythmias]] and slow the [[heart rate]]. Other medications might be needed to further minimize [[blood pressure]] without slowing the [[heart rate]], such as [[ACE inhibitors]] and [[angiotensin II receptor antagonist]]s, also known as angiontensin receptor blockers (ARBs). If the dilation of the aorta progresses to a significant diameter [[aneurysm]], causes a dissection or a rupture, or leads to failure of the aortic or other valve, then surgery (possibly a composite aortic valve graft [CAVG] or valve-sparing procedure) becomes necessary. Although aortic graft surgery (or any vascular surgery) is a serious undertaking it is generally successful if undertaken on an elective basis. Surgery in the setting of acute aortic dissection or rupture is considerably more problematic. Elective aortic valve/graft surgery is usually considered when aortic root diameter reaches 50 millimetres, but each case needs to be specifically evaluated by a qualified cardiologist. New valve-sparing surgical techniques are becoming more common.<ref name="mayo-heart">{{Cite web|url=http://www.mayoclinic.org/marfan-syndrome/heartsurgery.html|title=Heart Surgery for Marfan Syndrome|accessdate=2007-01-12|publisher=Mayo Clinic}}</ref> As Marfan patients live longer, other vascular repairs are becoming more common, e.g. repairs of descending thoractic aortic aneurysms and aneurysms of vessels other than the aorta.

Conspiracy in the death would be virtually impossible, however, since it would involve convincing Don Jonz, the pilot, to kill himself along with his two passengers. He deliberately flew into known icing conditions, having written the lead article for ''[[Flying (magazine)|Flying]] Magazine'' of October 1972, entitled "Ice Without Fear." In the article, Jonz boasted that 99% of the current wisdom regarding flying in icing conditions could be safely disregarded.{{fact|date=April 2007}}

The skeletal and ocular manifestations of Marfan syndrome can also be serious, although not life-threatening. These symptoms are usually treated in the typical manner for the appropriate condition. This can also affect height, arm length, and life span. The [[Nuss procedure]] is now being offered to people with Marfan syndrome to correct 'sunken chest' or ([[pectus excavatum]]).<ref name="chkd">{{Cite web|url=http://www.chkd.org/services/nussprocedure/Overview.aspx|title=Overview of the Nuss Procedure for Pectus Excavatum|accessdate=2007-01-12|publisher=Children's Hospital of The King's Daughters}}</ref> Because Marfan may cause spinal abnormalities that are asymptomatic, any spinal surgery contemplated on a Marfan patient should only follow detailed imaging and careful surgical planning, regardless of the indication for surgery.

== Family ==

In 1973, Boggs's wife since 1938, [[Lindy Boggs|Lindy]], was elected to the second district seat left vacant by his death, where she served until 1991.

Clinical trials have been conducted of the drug [[acetazolamide]] in the treatment of symptoms of [[dural ectasia]]. The treatment has demonstrated significant functional improvements in some sufferers.<ref name="spine">{{Cite web|url=http://www.spineuniverse.com/displayarticle.php/article922.html|title=Dural Ectasia in the Marfan Spine: Symptoms and Treatment|accessdate=2007-01-12|publisher=Scoliosis Research Society}}</ref> Other medical treatments, as well as physical therapy, are also available.

Hale and Lindy Boggs had three children: [[journalist]] [[Cokie Roberts]] (wife of journalist [[Steven V. Roberts]]), [[Tommy Boggs]], a prominent Washington, D.C.-based [[attorney]] and [[lobbyist]], and the late [[Barbara Boggs Sigmund]], who served as [[mayor]] of [[Princeton, New Jersey]]. In 1982, Mrs. Sigmund lost a bid for the Democratic nomination for the U.S. Senate to [[Frank Lautenberg]].

Treatment of a spontaneous [[pneumothorax]] is dependant on the volume of air in the pleural space and the natural progression of the individual's condition. A small pneumothorax might resolve without active treatment in 1 to 2 weeks. Recurrent pneumothoraxes might require chest surgery. Moderately sized pneumothoraxes might need [[Chest tube|chest drain]] management for several days in hospital. Large pneumothoraxes are likely to be medical emergencies requiring emergency decompression.

==Quote==

''"I wish I could stand here as a man who loves his state, born and reared in [[Southern United States|the South]], who has spent every year of his life in [[Louisiana]] since he was 5 years old, and say there has not been [[discrimination]]. But, unfortunately, it is not so."''

Research in laboratory [[mouse|mice]] has suggested that the [[angiotensin II receptor antagonist]] [[losartan]], which appears to block TGF-beta activity, can slow or halt the formation of aortic aneurysms in Marfan syndrome.<ref name="scimag">{{Cite journal | last = Habashi | first = Jennifer P. | coauthors = Daniel P. Judge, Tammy M. Holm, Ronald D. Cohn, Bart L. Loeys, Timothy K. Cooper, Loretha Myers, Erin C. Klein, Guosheng Liu, Carla Calvi, Megan Podowski, Enid R. Neptune, Marc K. Halushka, Djahida Bedja, Kathleen Gabrielson, Daniel B. Rifkin, Luca Carta, Francesco Ramirez, David L. Huso, and Harry C. Dietz | date = [[April 7]], [[2006]] | title = Losartan, an AT1 Antagonist, Prevents Aortic Aneurysm in a Mouse Model of Marfan Syndrome | volume = 312 | issue = 5770 | pages = 117 - 121 | doi = 10.1126/science.1124287 | url = http://www.sciencemag.org/cgi/content/full/312/5770/117 | abstract = http://www.sciencemag.org/cgi/content/abstract/sci;312/5770/117 | news = http://www.news-medical.net/?id=17249}}</ref> A large [[clinical trial]] sponsored by the [[National Institutes of Health]] comparing the effects of losartan and [[atenolol]] on the aortas of Marfan patients is scheduled to begin in early 2007, coordinated by Johns Hopkins.<ref name="trial">{{Cite web|url=http://www.marfan.org/nmf/GetSubContentRequestHandler.do?sub_menu_item_content_id=147&menu_item_id=91|title=Atenolol vs. Losartan in Individuals with Marfan Syndrome Clinial Trial|accessdate=2007-01-12|publisher=National Marfan Foundation}}</ref>

==Tributes==

The [[Hale Boggs Memorial Bridge]], which spans the [[Mississippi River]] in [[St. Charles Parish]], is named in memory of the former congressman.

Genetic counseling and specialized clinics are available at many academic medical centers for affected persons and family members.

==See also==

* [[Lindy Boggs]]

* [[Hale Boggs Memorial Bridge]]

* [[Nick Begich]]

* [[List of people who have disappeared]]

==Well known people==

==Notes==

Below is a list of prominent figures known or believed to have had Marfan syndrome:

{{reflist}}

* [[Euell Gibbons]], outdoorsman & health food proponent.

* [[Flo Hyman]], silver medal in Women's Volleyball (1984 Olympics)<ref name="flo">{{Cite web|url=http://www.volleyhall.org/hyman.html|title=Flo Hyman|accessdate=2007-01-11|publisher=Volleyball Hall of Fame}}</ref>

* [[Jonathan Larson]], Tony Award-winning playwright ([[Rent (musical)|Rent]]); he died in 1996 of [[aortic dissection]] on the eve of his musical's premiere<ref name="larson">{{Cite web|url=http://www.wnbc.com/drmaxgomez/5421112/detail.html|title=Marfan's Syndrome Is Deadly, Elusive|accessdate=2007-01-11|publisher=WNBC.com}}</ref>

* [[Robert Johnson (musician)|Robert Johnson]], blues singer and guitarist<ref name="robertj">{{Cite journal | last = Connel | first = David | date = [[September 2]], [[2006]] | title=Retrospective blues: Robert Johnson—an open letter to Eric Clapton | journal = British Medical Journal | volume = 333 | issue = 7566 | pages = 489 | url=http://www.pubmedcentral.nih.gov/articlerender.fcgi?artid=1557967|accessdate=2007-01-11}}</ref>

*[[Mike Dunleavy]], professional basketball player for the [[Portland Trailblazers]]

* [[Vincent Schiavelli]], actor<ref name="schiavelli">{{Cite web|url=http://www.marfan.org/nmf/PreviewPressReleaseInfoRequestHandler.do?press_release_id=24|title=NMF Mourns the Loss of its Honorary Co-Chair, Vincent Schiavelli|accessdate=2007-01-11|publisher=National Marfan Foundation}}</ref>

* [[Sir John Tavener]],<ref name="bbc">Richard Morrison, ''99 Names for God: John Tavener turns his back on Orthodoxy'', BBC Music, November 2004, page 30</ref> contemporary British composer

* Bradford Cox, frontman of the punk rock band [[Deerhunter]]<ref name="deerhunter">{{Cite web|url=http://www.pitchforkmedia.com/article/feature/43085-interview-deerhunter|title=Pitchfork Feature: Interview: Deerhunter}}</ref>

* [[Joey Ramone]], of the punk rock band [[The Ramones]].

===Spurious or conjectural claims===

==References==

There are a number of historical persons believed to have suffered from Marfan's syndrome, but as proper Marfan diagnosis was not available before well into the 20th century most such claims can only be considered as speculation based on sparse medical records and pictures.

* Maney, Patrick J. "Hale Boggs: The Southerner as National Democrat" in Raymond W Smock and Susan W Hammond, eds. ''Masters of the House: Congressional Leadership Over Two Centuries'' (1998) pp 33-62.

* Strahan, Randall. "Thomas Brackett Reed and the Rise of Party Government" in Raymond W Smock and Susan W Hammond, eds. ''Masters of the House: Congressional Leadership Over Two Centuries'' (1998) pp 223-259.

*{{cite web|accessdate=2007-04-15|url=http://bioguide.congress.gov/scripts/biodisplay.pl?index=b000594

|title=Boggs, Thomas Hale, Sr., (1914 - 1972)

|work=Biographical Directory of the United States Congress}}

* [[Akhenaten]], Egyptian Pharaoh, who was the father of King [[Tutankhamun]] (spurious claim based on early Amarna art style. Tutankhamun, who didn't have syndrome, was also portrayed this way)<ref name="pharaoh">{{Cite web|url=http://www.marfan.ca/pharaoh.html|title=Did Akhenaten Suffer from Marfan's Syndrome?|accessdate=2007-01-11|publisher=Canadian Marfan Association}}</ref>

== External links ==

* [http://www.lbjlib.utexas.edu/johnson/archives.hom/oralhistory.hom/boggsh/boggsh.asp Transcript, Hale Boggs Oral History Interview], 3/13/69, by T. H. Baker, Internet Copy, LBJ Library. Accessed April 3, 2005.

* {{cite web|url=http://foia.fbi.gov/foiaindex/boggs.htm

|title=Hale Boggs - Freedom of Information Privacy Act page

|publisher=Federal Bureau of Investigation}}

* [[Osama bin Laden]] may suffer from Marfan Syndrome (speculation based on tall size, use of cane and rumoured heart-disease; almost certainly in error)<ref>http://archive.salon.com/people/feature/2001/11/09/marfan/index.html</ref>

* [http://www.check-six.com/lib/Famous_Missing/Boggs.htm Hale Boggs Telex - Debunked]

* [[Charles de Gaulle]] (conjectural)<ref>http://archive.salon.com/people/feature/2001/11/09/marfan/index.html</ref>

{{start box}}

{{USRepSuccessionBox|

* [[Nicollo Paganini]] (Conjectural) <ref>[http://tafkac.org/celebrities/paganini_stories_myths.html], main reference being an article in the AMA journal by Dr. Myron R. Shoenfeld dated 2 January, 1978.</ref>

state= Louisiana |

district= 2 |

* It was once believed that [[Abraham Lincoln]] suffered from Marfan Sydrome, although recent research has argued that he probably didn't.

before= [[Paul H. Maloney]]|

years= 1941 – 1943|

* [[Johnny Appleseed]], a pioneer nurseryman in America living during the 18th century; he has become an almost mythical popular culture icon in America.

after=[[Paul H. Maloney]] }}

{{USRepSuccessionBox|

==Related disorders==

state= Louisiana |

The following disorders have similar signs and symptoms of Marfan syndrome:

district= 2 |

district_ord=2nd |

*[[Arachnodactyly|Congenital Contractural Arachnodactyly (CCA) or Beals Syndrome]]

before= [[Paul H. Maloney]]|

*[[Ehlers-Danlos syndrome]]

years= 1947 &ndash; 1972|

*[[Homocystinuria]]

after=[[Lindy Boggs]] }}

*[[Loeys-Dietz syndrome]]

{{succession box

*[[MASS phenotype]]

| title=[[Majority Leader of the United States House of Representatives]]

*[[Stickler syndrome]]

| before=[[Carl Albert]]

| after=[[Tip O'Neill]]

The following conditions that can result from having Marfan syndrome may also occur in people without any known underlying disorder:

| years=1971-1972

}}

<div style="width:30%; float:left; padding:0 3% 0 0; border:none; overflow:hidden; clear:left;">

{{end box}}

*[[Aortic aneurysm|Aortic aneurysm or dilitation]]

{{WarrenCommission}}

*[[Arachnodactyly]]

{{USHouseMajLead}}

*[[Bicuspid aortic valve]]

{{USSenMajWhip}}

*[[Cysts]]

*[[Craniosynostosis]]

*[[Cystic medial necrosis]]

*[[Dural ectasia]]

*[[Ectopia lentis]]

</div>

<div style="width:30%; float:left; padding:0 3% 0 0; border:none; overflow:hidden; ">

*[[Flat feet]]

*[[Gigantism]]

*[[Glaucoma]]

*[[Hernias]]

*[[Hyperflex|Hypermobility of the joints]]

*[[Malocclusion]]

*[[Mitral valve prolapse]]

*[[Myopia]]

</div>

<div class="editmode" style="width:30%; float:left; padding:0 3% 0 0; border:none; overflow:hidden; ">

*[[COPD|Obstructive lung disease]]

*[[Osteoarthritis]]

*[[Pectus carinatum]] or [[pectus excavatum|excavatum]]

*[[Pneumothorax]]

*[[Retinal detachment]]

*[[Scoliosis]]

*[[Sleep apnea]]

*[[Stretch marks]]

</div><br clear="left" />

==References==

{{reflist|2}}

==External links==

*[http://marfanworld.org/ International Federation of Marfan Syndrome Organisations]

*[http://www.marfan.org/ National Marfan Foundation (USA)]

*[http://www.marfan.org.za/diagnosis.html Marfan diagnosis criteria]

*[http://www.ncbi.nlm.nih.gov/disease/Marfan.html National Institute for Health Marfan syndrome page (USA)]

*[http://www.medicinenet.com/marfan_syndrome/index.htm Marfan Syndrome Center at medicinenet.com]

*[http://marfansyndrome.researchtoday.net/ Marfan Syndrome Research] - recent literature on Marfan Syndrome

*[http://www.supportmarfan.com Marfan support]

*[http://www.marfan.ca/ Canadian Marfan Association]

*[http://www.marfan.org.uk/ Marfan Association UK]

*[http://www.marfan.org.mx/ Marfan de Mexico]

*[http://www.marfan.no/ Norwegian Marfan Organization]

*[http://www.marfanlife.net Marfan Life blog] - mostly links to news articles about Marfan Syndrome

*[http://www.marfanlife.net/lists/ Marfan-List] - email discussion list for people and families with Marfan Syndrome

*[http://www.marfan.org.za/ South African Marfan Syndrome Organisation] - support group for Africa

*[http://www.medstudents.com.br/original/revisao/marfan/marfan.htm Eye Findings in Marfan's syndrome]

[[Category:1914 births|Boggs, HaleCardiology]]

[[Category:1972Diseases deaths|Boggs,involving Halethe fasciae]]

[[Category:AccidentalGenetic deaths|Boggs, Haledisorders]]

[[Category:Syndromes]]

[[Category:American military personnel of World War II|Boggs, Hale]]

[[Category:Disappeared people|Boggs, Hale]]

[[Category:Unexplained disappearances]]

[[Category:John F. Kennedy assassination|Boggs, Thomas]]

[[Category:Louisiana lawyers|Boggs, Hale]]

[[Category:Louisiana politicians|Boggs, Thomas Hale]]

[[Category:Majority Leaders of the United States House of Representatives|Boggs, Thomas Hale]]

[[Category:Members of the United States House of Representatives from Louisiana|Boggs, Thomas Hale]]

[[Category:Plane crash victims|Boggs, Hale]]

[[Category:Roman Catholic politicians|Boggs, Hale]]

[[Category:Southern Manifesto|Boggs, Hale]]

[[Category:United States Navy officers|Boggs, Hale]]

[[Category:Warren Commission|Boggs, Thomas]]

[[Category:Watergate figures|Boggs, Hale]]

[[Category:Candidates for governor of Louisiana|Boggs, Hale]]

[[Category:People from Louisiana|Boggs, Hale]]

[[Category:Tulane University alumni|Boggs, Hale]]

[[ar:متلازمة مارفان]]

[[de:Hale Boggs]]

[[de:Marfan-Syndrom]]

[[es:Síndrome de Marfan]]

[[fr:Syndrome de Marfan]]

[[ko:마르팡 증후군]]

[[it:Sindrome di Marfan]]

[[he:תסמונת מרפן]]

[[nl:Syndroom van Marfan]]

[[ja:マルファン症候群]]

[[nn:Marfans syndrom]]

[[pl:Zespół Marfana]]

[[pt:Síndrome de Marfan]]

[[ru:Синдром Марфана]]

[[sr:Марфанов синдром]]

[[fi:Marfanin oireyhtymä]]

[[sv:Marfans syndrom]]

[[uk:Синдром Марфана]]