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Morane-Saulnier Type H
The Morane-Saulnier Type H was a successful pre-First World War single seat sports aircraft that served with limited success early in the war. By the time of the 1913 Paris Salon Morane-Saulnier had three types of monoplane aircraft on sale. The Type L was a parasol wing aircraft. The Type G was a shoulder winged two-seater. The Type H was a slightly smaller single-seat version of the Type G. All had a simple flat sided fabric covered fuselage, used wing warping controls and had straight wings with a tapered end.
The Type H was used by a number of famous pre-war pilots and achieved some significant successes. Between 10 June and 2 July Marcel Brindejonc flew one on a round-trip of the capitals of Europe, flying 3,000 miles. On 12 September 1913 Roland Garros made the first crossing of the Mediterranean in a Type H, flying from St-Raphael to Bizerte, a distance of 455 miles, in 7 hrs 53. The Type H was also built under licence in Britain by Graham-White, and one of his aircraft won the London-Paris-London contest of 11 July 1914.
The French War Ministry ordered twenty six Type Hs, and they were used during the first year of the First World War. Some were used during the defence of Paris in 1914 (serving with the Escadrille de Protection du Camp Retranché de Paris), despite only being armed with a pistol or carbine carried by the pilot. A small number of British built examples were used by the RFC early in the war, but in both cases the Type H had limited military capability.
Engine: Gnome or Le Rhône Rotary engine
Wing span: 29ft 11in
Max Speed: just above 84mph
Armament: Pistol or carbine carried by the pilot.
Books on the First World War |Subject Index: First World War
Type H blood vessels in bone modeling and remodeling
In the mammalian skeletal system, osteogenesis and angiogenesis are intimately linked during bone growth and regeneration in bone modeling and during bone homeostasis in bone remodeling. Recent studies have expanded our knowledge about the molecular and cellular mechanisms responsible for coupling angiogenesis and bone formation. Type H vessels, termed such because of high expression of Endomucin (Emcn) and CD31, have recently been identified and have the ability to induce bone formation. Factors including platelet-derived growth factor type BB (PDGF-BB), slit guidance ligand 3 (SLIT3), hypoxia-inducible factor 1-alpha (HIF-1α), Notch, and vascular endothelial growth factor (VEGF) are involved in the coupling of angiogenesis and osteogenesis. This review summarizes the current understanding of signaling pathways that regulate type H vessels and how type H vessels modulate osteogenesis. Further studies dissecting the regulation and function of type H vessels will provide new insights into the role of bone vasculature in the metabolism of the skeleton. We also discuss considerations for therapeutic approaches targeting type H vessels to promote fracture healing, prevent pathological bone loss, osteonecrosis, osteoarthritis, and bone metastases.
Keywords: Angiogenesis Bone formation CD31hiEmcnhi endothelial cells H-type vessels Osteogenesis Type H vessels.
Conflict of interest statement
Competing Interests: The authors have declared that no competing interest exists.
Human type H vessels are a sensitive biomarker of bone mass
Vascularization is fundamental for bone formation and bone tissue homeostasis. However, in human subjects, a direct molecular relationship has not been identified between angiogenesis and agents that promote bone disease or factors related to age. Osteopenia is a condition in which bone mineral density is lower than normal, and it represents a sign of normal aging. Here we tested whether the type H vessel, which was recently identified as strongly positive for CD31 and Endomucin (CD31 hi Emcn hi ) in mice, is an important indicator of aging and osteopenia in human subjects. We found that age-dependent losses of type H vessels in human bone sections conform to the observations in aged mice. The abundance of human type H vessels and osteoprogenitors may be relevant to changes in the skeletal microarchitecture and advanced osteopenia. Furthermore, ovariectomized mice, a widely used model for postmenopausal osteoporosis, exhibited significantly reduced type H vessels accompanied by reduced osteoprogenitors, which is consistent with impaired bone microarchitecture and osteoporosis, suggesting that this feature is an indicator of bone mass independent of aging. More importantly, administration of desferrioxamine led to significantly increased bone mass via enhanced angiogenesis and increased type H vessels in ovariectomized mice. Altogether, these data represent a novel finding that type H vessels are regulated in aged and osteopenia subjects. The abundance of human type H vessels is an early marker of bone loss and represents a potential target for improving bone quality via the induction of type H vessels.
Trial registration: ClinicalTrials.gov NCT02750046.
Conflict of interest statement
The authors declare no conflict of interest.
Age series of mouse tibia…
Age series of mouse tibia and femur immunostaining. ( a – c )…
Decreased type H vessels in…
Decreased type H vessels in aged patients. ( a ), Schematic illustration of…
Synergistic decrease of type H…
Synergistic decrease of type H vessel and bone mineral density in human bone.…
Early Type H Rotor with long shaft output pinion
Bottom view showing mounting feet used only on early examples of type H
Side view showing long shaft output pinion
The other side of the rotor is labeled: Telechron, Inc.
This example may be a later replacement for an early rotor, as the date code 095 means September 1950, if I am interpreting it correctly (first digit is the year, second digit is the month, and third digit is the decade - the code used from 1950 to 1990.)
Depron indoor Morane Saulnier Type H
I've already started this in a different thread re. a profile SE5a, but figured it should really be in its own spot. It's based on Emanuel Fillon's FF rubber powered version, enlarged to 160% and built from depron instead of stick and tissue. The WS is 20" and wing area is 90 sg in. I'm probably going to use a V911 brick. Motor to be determined from what I have on hand. Probably an 8mm gearbox WLtoys combo from Banggood. Weight so far is 12 grams.
I've just started working on the cowl.
You might well find that's too much motor for a sub 25 gram model. Even the Hisky 7 mm unit is pretty potent and needs minimal throttle in 15", 18 gram biplanes. I know you've mentioned restricting throttle but smaller is lighter and lighter flies slower.
Looking forward to seeing how this one works out.
Yes, guys, I too am sure it is too powerful as-is (though I do expect the model will finish up closer to 40 grams than 25, Peter).
I do have a couple of 7 gram motors and gearboxes. One is an old style HobbyKing GPS-7, and the other is a new Banggood combo:
The motors are different -- the 7mm HK motor is 17 mm long and the 7mm BG motor is 20 mm long and includes a prop.
If I were to go with one of the 7mm motors/gearboxes, do you think either of those would work with this plane?
I was planning to use an 8mm set with a TX adjustment to limit max power to 50% using the throttle curve function in the Devo7e.
I've been playing around with Deviation's "5 point throttle curve" on the SE5a and I've got a really nice low throttle response now, reduced max, with a broad range low down, and I think this could work out really well for that model, even though it has an 8.5mm motor.
The weight of the motor did make the plane balance with no ballast, and the SE5A has a long nose, unlike the Morane -- as you mentioned epilot. I does seem silly to use a too large motor just to balance the Morane, but I do have a lot of those size motors and at lower speeds they and their larger propellers are also likely more efficient, longer lasting, and would probably give longer flight times than the smaller motors.
H.D. Smith 660-6 "Perfect Handle" Screwdriver
Fig. 1 shows an H.D. Smith 660-6 flat-tipped screwdriver, marked with the S-Circle logo forged into one side, with "Improved" and "660" plus "Pat Ap'l'd For" forged into the reverse.
The overall length is 11.6 inches, and the finish is plain steel.
H.D. Smith "Perfect Handle" 8 Inch Monkey Wrench
Fig. 2 shows an H.D. Smith 8 inch monkey wrench, stamped on the shank with "The H.D. Smith & Co." and "Perfect Handle" in circular arcs, with patent dates and "Made in U.S.A." in the center. (The markings are partially obscured by the moving jaw.)
The overall length is 8.6 inches, and the finish is plain steel.
The patent dates are marked "Pat'd Oct. 30-1900 Jan. 15-1901 Feb. 26-1901", with the first date corresponding to design patent #D33,468, issued to Amos Shepard on October 30, 1900. This patent shows a design for the body and handle of a wrench very similar to the present item.
H.D. Smith "Perfect Handle" 14 Inch Pipe Wrench
Fig. 3 shows an H.D. Smith 14 inch pipe wrench, marked with "Perfect Handle" and "Patented" forged into the shank, with "The H.D. Smith & Co." and "14 In." forged into the reverse.
The overall length is 13.4 inches closed and 15.4 inches fully opened. The finish is plain steel.
The patent notice refers to patent #1,122,335, filed by W.S. Thomson in 1913 and issued in 1914. The patent illustration shows a pipe wrench closely resembling the present example.
H.D. Smith "Perfect Handle" [No. 650] Valve Spring Lifter
Fig. 4 shows an H.D. Smith valve spring lifter of a unique design, employing a hook and chain to provide the fulcrum for the lever. The lever is marked with "The H.D. Smith Co." and "Made in Plantsville, Conn, U.S.A." forged into the shank, with "Perfect Handle" and "Patented" forged into the reverse. The forged hook is also marked, with the same company information on one side and "Drop Forged" on the reverse (not shown).
The overall length of the lever is 12.7 inches, and the maximum length of the chain and hook is 17.5 inches. The finish is plain steel.
Although not marked with a model number, this tool is listed as No. 650 in a 1916 J.M. Waterston tool catalog.
The operation of this tool may not be immediately apparent due to its unusual design. The point of the large hook is first placed in a suitable depression on the top of the engine block, with the chain hanging over the side near the valves. With the small "S" hook removed from the end, the chain is then passed through one of the holes in the lever arm, and the "S" hook is inserted below to set the fulcrum at a suitable point. The valve spring can then be lifted using the lever handle.
A Short History of English Springer Spaniels
Histories hint that dogs of the Spaniel type have populated the civilized world for many centuries. The Spaniel is thought to have originated in Spain and was perhaps introduced to ancient Britons by the Roman legions. The Spaniel was known in pre-Christian Britain, and is mentioned by name in an ancient law of Wales as early as 300 AD.
Prints and paintings from the 16th and 17th centuries show dogs of similar type to today's English Springer Spaniel, some with docked tails. The dog was used to spring (flush) or start game, both feathered and fur bearing, for hawks, coursing hounds, and nets. The invention of the wheel lock firearm in the 17th century made "flying shooting" possible, and the Spaniel was highly successful at flushing game for this style of hunting.
Throughout the 19th and early 20th century in Britain, smaller dogs in the same litter would be used to hunt woodcock they were called "cockers." Larger littermates, used to flush (or "spring") game, were called "Springers." The Sporting Spaniel Society of Britain decided upon the name "Springer" in 1902.
The first English Springer Spaniel in North America of traceable lineage was imported from England to Canada in 1913. In little more than a decade, the breed had risen from the ranks of the unknown to become numbered among the most popular of the breeds eligible for American Kennel Club registration. Early importers and breeders were both careful and selective in their breeding practices, and many field trialers enjoyed exhibiting their Springers in conformation competition. The English Springer Spaniel Field Trial Association, the parent club of the breed in the United States, was founded in 1924. In those days, dogs might emerge from a day of heavy field work to be presented in conformation competition the next day.
"Dual type" Springers - those capable of competing in both conformation and field trial competition - became a part of history by the early 1940's, when the last dual championship was earned. Field trial enthusiasts began selecting those qualities in their dogs which produced top caliber performance, while the show-minded endeavored just as earnestly to breed dogs consistent with the breed's written standard, successful as conformation competitors.
The true beauty of the English Springer Spaniel can be found in its original purpose, that of a companion gundog. The diverse appearance of today's show and field bred Springers is due to specialization, and to the choices breeders make to achieve success in their endeavors. Competitive field trial Springers are the ultimate in athleticism and performance. Competitive conformation Springers are breathtaking examples of breed type, soundness, and symmetry.
Thinly sourced research from a small nonprofit, The Rebecca Project, suggested a massive international conspiracy, led by the Gates Foundation, to push dangerous contraceptives on poor black women as a means of decreasing African births.
As black feminists pointed out, these groups cared little for women&rsquos or civil rights in general, or black women&rsquos well-being in particular. (A 2009 U.S. House bill titled the &ldquoSusan B. Anthony and Frederick Douglass Prenatal Nondiscrimination Act&rdquo was co-sponsored by a champion of the Confederate flag.)
But the strategy exploited the real and painful history of medical abuses against people of color in the U.S., from compulsory or coercive sterilization campaigns from the 1910s to &rsquo60s (including the sterilization of a third of all Puerto Rican mothers between 20 and 49 years old by 1965) to unsafe contraceptives marketed to poor women of color from the 1970s to &rsquo90s. And the legacy of those abuses could be profound. One 2016 study found that the notorious Tuskegee Study, wherein hundreds of black men were left with untreated syphilis so U.S. government researchers could track the progress of the disease, led to such mistrust of the medical establishment that it reduced the life expectancy of a generation of black men by more than a year.
The Rebecca Project, a small, Washington-based nonprofit focused on issues disproportionately affecting women of color, hadn&rsquot been involved on either side of the abortion fight. But in 2011, the group released a thinly-sourced report titled &ldquoNon-Consensual Research in Africa: The Outsourcing of Tuskegee,&rdquo outlining what it claimed was a series of unethical U.S.-backed medical experiments in Africa.
Some of the examples were documented stories of legitimate concern &mdash for instance, HIV-positive women in southern Africa had been pressured into sterilization procedures by local health care entities. The report attempted to link them to shakier allegations of USAID funding being used for coercive sterilization campaigns in other countries. But the report&rsquos real target, it seemed, was the Gates-backed health initiative in Navrongo. Later, the report&rsquos lead author would suggest that people involved with the project should be charged with attempted genocide.
The report had numerous factual problems. Its author ― the Rebecca Project&rsquos chief financial officer, Kwame Fosu ― also hadn&rsquot disclosed a significant conflict of interest: The employee who&rsquod leveled the charges against the Ghana project was the mother of his child. The fallout wound up splitting the organization, as one of its founders and several staff departed, taking with them all the Rebecca Project&rsquos funding. Left with the group&rsquos name, Fosu doubled down on his conspiratorial claims.
In 2013, Fosu published another report, &ldquoDepo-Provera: Deadly Reproductive Violence Against Women.&rdquo Drawing heavily on unnamed sources, paranoid accusations and the rhetoric of right-wing anti-abortion groups, this report used the Ghana story to anchor claims of a massive international conspiracy, led by the Gates Foundation, to push dangerous contraceptives on poor black women as a means of decreasing African births and advancing &ldquopopulation control ideology.&rdquo Fosu brought the Rebecca Project into alliance with a network of conservative Catholic nonprofits, like C-Fam and the Population Research Institute (PRI), that had long focused on fighting reproductive rights in developing nations or at the United Nations.
A portion of the title page from The Rebecca Project for Justice’s 2013 report.
His new allies began publicizing Fosu&rsquos claims to a large audience of conservative activists, arguing that he had uncovered the smoking gun confirming their long-held suspicions. As the head of PRI put it, &ldquoThe population controllers will stop at nothing to stop African women from having children.&rdquo By 2014, the Rebecca Project was focusing full-time on the scourge of Depo-Provera. At the same time, the Gates Foundation was undertaking a new mission to radically expand contraceptive access to women in Africa, including with a new, low-dose adaptation of Depo-Provera.
The foundation&rsquos family planning campaign had already drawn predictable backlash from religious groups. But as U.S. anti-abortion groups and websites circulated the Rebecca Project&rsquos allegations, the opposition was no longer dominated by complaints that Gates was tempting African women to defy their faith. The new narrative was that Gates was waging &ldquochemical warfare on poor women&rdquo in a neocolonial effort to suppress African births.
Soon, powerful figures across Africa were making similar claims, undermining vital public health projects in the process. In 2014, Zimbabwe&rsquos Registrar General, Tobaiwa Mudede &mdash the official responsible for overseeing the country&rsquos dubious elections &mdash warned women to avoid modern contraceptives because they caused cancer and were a Western ploy to limit African population growth. In 2015, Mudede told parliamentarians, &ldquoWestern countries are bent on curtailing the population of the darker races of the world.&rdquo According to a parliamentary committee, Mudede&rsquos campaign panicked Zimbabwean women, who flooded into clinics to have contraceptive implants removed.
Hypermobile Ehlers-Danlos Syndrome: Clinical Description and Natural History (for Non-experts)
Hypermobile type Ehlers-Danlos syndrome (hEDS) is the most common subtype of the Ehlers-Danlos syndromes (EDS) and possibly the most common of all hereditary disorders of connective tissue (HDCT). The new EDS classification system replaces the diagnosis of Ehlers&ndashDanlos syndrome Type III/ Ehlers&ndashDanlos syndrome Hypermobility Type (EDS-III / EDS-HT) and joint hypermobility syndrome (JHS).
Many people who were previously assigned a diagnosis of EDS-III, EDS-HT, or JHS will meet the criteria for hEDS some will instead be classed as having Hypermobility Spectrum Disorders (HSD). The new criteria for hEDS are stricter now, intended for a more consistent and targeted identification, in the hopes of aiding a greater understanding of the cause(s) and course (natural history) of the disorder. With stricter criteria there is a greater chance of finding the specific reasons why and how disease comes about. With greater understanding comes a better chance of positive changes for those with hEDS: fostering highly targeted research, achieving successful disease management, and ultimately maximizing quality of life for people with hEDS.
hEDS is mainly identified by generalized joint hypermobility (GJH), additional joint issues, and obvious skin signs, which are less severe than those seen in Classic EDS (cEDS) or Vascular EDS (vEDS). hEDS is more than &ldquojust GJH&rdquo plus a few items from a checklist of findings. In order to best understand the nature of hEDS, people with hEDS and those caring for them must become familiar with the disorder.
Prevalence of hEDS: EDS occurs in at least 1 in 5000 of the population, 80&ndash90% of which could be cases of hEDS.
Accurate estimates for the occurrence of hEDS are lacking, but a minimum of 1 in 5,000 people are thought to have EDS, 80&ndash90% of which are cases of hEDS. High estimates under the previous classification system give figures suggesting up to 2 million people in the UK, 10 million in the USA, 17 million in Europe, and 255 million worldwide have hEDS. The new classification of hEDS is more selective, so figures will be lower than previously reported.
Genetics: We have a lot to learn about the underlying genetics of hEDS.
At present, no single gene mutation causing hEDS has been identified. hEDS is likely to be caused by many different genetic changes. With the stricter classification of hEDS and more in-depth genetic studies, additional hEDS-related genes should be identified. Identification of genetic causes for hEDS may lead to it being further broken down into specific named hEDS subtypes, or perhaps lead to hEDS being redefined or replaced by multiple new specific EDS types, in a manner similar to how classical-like EDS (clEDS) has been defined as a new EDS type due to the genetic cause being identified.
hEDS has a roughly 50% chance of being passed on to each child (autosomal dominance), but other patterns of inheritance may explain this disorder in certain families. hEDS inheritance is somewhat difficult to analyze, as hEDS may be mild during much of life, or compared to close relatives with the disorder, or may even appear to &ldquoskip&rdquo a generation. This may be in part because JH is heavily influenced by age, gender, and weight. The reason for a perceived excess of females remains poorly understood, but may come down to sex hormones having a greater influence upon JH. The best way to describe hEDS is as an autosomal dominant disorder influenced by age and gender, with symptoms more common in females.
Natural History: What happens to people with hEDS?
If a person has hEDS, it will likely affect them in different ways throughout their lifetime, and the person may be diagnosed with many other conditions known to occur in those with hEDS. For example, three disease phases were proposed in a 2010 study: a &ldquohypermobility&rdquo phase, a &ldquopain&rdquo phase, and a &ldquostiffness&rdquo phase. Alternatively, existing studies have led to speculation that there is a natural transition from EDS-HT to GJH with age.
Existing studies show that children with hEDS who experience pain will be more likely to have pain limited to lower limbs (e.g., &ldquogrowing pains&rdquo) and pain caused by repetitive tasks such as handwriting in the school setting. Children with hEDS may have poor coordination. The &ldquopain&rdquo phase is often accompanied by diagnosis with fibromyalgia or other long-term (chronic) pain conditions and perhaps chronic fatigue, typically starting in the second to fourth decade and accompanied by chronic pain, headaches, digestive system disorders, among others. The &ldquostiffness&rdquo phase is seen in only a few persons, and, unfortunately for them, the symptoms of the &ldquopain&rdquo phase may persist and escalate, functionality may overall be significantly reduced.
Conditions Often Occurring in Persons with hEDS
Pain: Yes, hEDS CAN cause significant pain!
Uneducated doctors all-too-often make emphatic statements such as, &ldquoEhlers-Danlos syndrome cannot cause pain!&rdquo In fact, while hEDS does not necessarily cause significant pain for every person meeting diagnostic criteria, many with hEDS will develop significant pain for some portion of their lives. Any doctor who invalidates the fact that hEDS can cause significant pain may have a profoundly negative impact, not only upon the success of the relationship between clinician and patient, but also upon the quality of life of those with hEDS.
Health practitioners need to understand that the occurrence of significant pain for many persons with hEDS is well-supported in the literature and has logically proposed or proven cause. In fact, joint/muscle pain in two or more limbs (recurring daily, &ge 3 months) and long-term widespread pain (&ge 3 months) are specifically considered as a part of the criteria for diagnosis of hEDS.
Skin and Connective Tissue: People with hEDS do NOT have to have profoundly stretchy skin!
Most notably, in hEDS, the degree of softness, stretchiness, fragility, bruisability, and poor wound healing of skin differs from &ldquonormal&rdquo subjects but is mild in comparison to other types of EDS. Clinicians who are not up to date on the classification of EDS types often expect that all EDS types demonstrate severe skin changes, such as those observed in persons with cEDS and vEDS. Mild skin stretchiness (rather than severe) is clearly considered as a systemic manifestation in the criteria for clinical diagnosis of hEDS.
Stretch marks are not inevitable in hEDS, however, they often appear in persons with hEDS during adolescent growth spurts and are not necessarily due to rapid weight gain. The absence of stretch marks should not argue against a diagnosis of hEDS. Other tissues which may fail in hEDS include the protective coverings around organs. Weakness in this connective tissue in hEDS often results in hernia (tissues or organs pushing through). Hernias may also be more likely in persons with hEDS who undergo abdominal surgery, such as laparotomy or C-section.
Fatigue: Chronic fatigue is one of the most common complications among persons with hEDS.
Chronic, debilitating fatigue is common in hEDS, and such fatigue has significant impact on mental and physical function and ultimately on quality of life. Those with hEDS will often meet the criteria for chronic fatigue syndrome (CFS). Under no circumstances should a diagnosis of CFS to a person who also meets criteria for hEDS mean that hEDS should be disregarded. hEDS is considered to be a specific cause of chronic fatigue, while CFS is considered a syndrome with unknown cause in this case the diagnosis of CFS would more appropriately called into question.
Cardiovascular: While conditions of the heart and blood vessels can occur with hEDS, they are not usually life-threatening, but deserve individual consideration.
Heart and blood vessel conditions occurring in hEDS include heart valve and vessel dysfunction including mitral valve prolapse (MVP) and aortic root dilation. Problems with blood pressure and heart rate can also occur including postural orthostatic tachycardia syndrome (POTS), neurally-mediated hypotension (NMH), and orthostatic intolerance. Those having blood pressure changes may also experience near-fainting or fainting episodes.
Gastrointestinal (GI) Disorders: Digestive system problems occur with high frequency in hEDS.
As many as 75% of people with hEDS are likely to encounter problems with the function of their digestive system in their lifetime. Problems can occur anywhere along the digestive system, including the mouth and throat (e.g., chewing and swallowing) in addition to the functions of the rest of the GI tract. Disorders include poor movement of material along the tract, reflux, heartburn, abdominal pain, bloating, irritable bowel, diarrhea, constipation, or incontinence. Structural problems occur at a higher rate in those with hEDS than in the general population including hernias, internal organ displacement, and rectal prolapse. The relationship between all structural abnormalities and hEDS requires further study.
Dysautonomia: &ldquoFight or Flight&rdquo and &ldquoRest and Digest&rdquo function poorly for a majority with hEDS.
An extensive body of literature clearly indicates frequent problems with involuntary bodily functions (dysautonomia) in hEDS. Problems with involuntary body functions can lead to fatigue, dizziness, fainting, memory changes, poor concentration, reduced sweat production, changes in gut movement, bladder dysfunction, and/or certain psychological traits. Dysautonomia can be a devastating manifestation of hEDS, and, for some patients, it affects their quality of life more profoundly than joint instability, pain, or any other part of the disorder.
Bone Density: Existing evidence does not clearly indicate that hEDS is expected to cause low bone density or increased risk for fragility fractures. Carefully designed research is needed.
While existing studies have suggested a link between various EDS types and conditions such as osteoporosis (low bone density) and osteopenia (low bone mineralization), those with EDS often have a significantly reduced level of activity, and, rather than any alteration in bone density or mineralization being directly attributable to EDS, alteration in bone density or mineralization should be considered to be a direct result of reduced activity. Bone health should not be ignored in hEDS.
Osteoarthritis: Yes, hEDS increases the susceptibility of joints to osteoarthritis.
Osteoarthritis (a breakdown of joints) has been described in the literature as a possible long-term consequence of JH for decades. Since JH is a hallmark feature of hEDS, and JH is recognized to cause osteoarthritis, then hEDS would logically be expected to predispose many of those with the disorder to osteoarthritis. By the same token, osteoarthritis is by no means universal for all persons with JH, so it should NOT be expected to be inevitable for all people with hEDS.
Headaches: hEDS may lead to severe or debilitating headaches for a multitude of reasons.
Headaches in general, as well as migraines in particular, are known to occur more frequently in persons with EDS than in the general population. Headaches in hEDS are considered to have many causes, with potential mechanisms including instability, strain, or muscle spasms in the neck, jaw joint dysfunction, and/or medication side effects. The headaches vary in type and severity among all persons with hEDS who have headaches. Some find headaches to be the most disabling manifestation of hEDS.
Temporomandibular Joint and Dental Issues: Dentists, Orthodontists, and Oral Surgeons may be the first to consider a diagnosis of hEDS.
A hypermobile jaw joint is more likely to show dysfunction than a non-hypermobile jaw. Those with hEDS and jaw joint dysfunction often have sounds, locking, dislocation, grinding teeth, and headaches in the temple.
Mouth manifestations of hEDS may include fragile gums which bleed easily, gum recession, gum disease, small or absent frenula (small connective flaps between the front gums and lips), pointed and deep teeth, shortened roots, abnormal enamel, tooth fractures, ineffective dental anesthesia, and rapid orthodontic correction (and rapid return without orthodontia).
Spine: It is difficult at times to determine whether a person with hEDS would benefit from surgery, and, even when surgery is necessary, it doesn&rsquot always solve everything.
People with hEDS may have neck pain, difficulty walking, numbness and tingling of the hands and feet, dizziness, swallowing difficulties, and changes in speech. These people are more likely to have signs of looseness or instability around the head and neck. In many cases, these symptoms are not entirely attributable to head and neck dysfunction: symptoms may still persist after successful surgery.
Abnormal spine curvature is common in people with hEDS, in a large part due to a combination of structural and functional abnormalities in the supporting tissues of the spine. Conservative management will ideally allow avoidance of surgery.
Gynecologic Issues: Women with hEDS may experience heavy periods or painful intercourse.
Pelvic Dysfunction: It is unclear whether hEDS alone predisposes women to pelvic dysfunction or how much of a role childbirth plays.
While the existing literature suggests that pelvic floor problems including urinary incontinence or uterine, rectal, or bladder prolapse are common to hEDS, many studies did not control for childbirth history, and included various EDS types.
Pregnancy and Childbirth: No studies to date recommend against pregnancy and childbirth based simply upon a diagnosis of hEDS.
While some studies of women with EDS-III/EDS-HT and JHS suggest increased rates of infertility, pregnancy losses, and premature births, other studies did not. In the case of studies regarding premature birth, the study group included women with Classic EDS. One of the most thorough studies prior to the current EDS classifications suggested similar measures of fertility.
When it comes to how pregnancy affects symptoms of hEDS, it goes like this: some get worse, some don&rsquot change, and some get better during the pregnancy. When it comes to labor, rapid labor is thought to occur in more than 1/3 of deliveries. The most considerable complications related to labor and delivery thought to occur at a significant rate in women with hEDS include bleeding during or after birth, as well as abnormal scarring from C-section or episiotomy. In general, no studies to date recommend against pregnancy and childbirth based simply upon a diagnosis of hEDS in the absence of any other factors.
Urinary System: hEDS may predispose children to urinary incontinence, urinary tract infections, vesicoureteral reflux, and voiding dysfunction.
Sleep Disturbance: Patients with hEDS may feel particularly tired.
People with hEDS often experience significant sleep deprivation. Going without healthy, restorative sleep can lead to any combination of significant health problems such as impaired immune response, poor muscle coordination, and higher perception of pain, as well as problems with memory and thought processes (e.g. &ldquobrain fog&rdquo), moodiness, depression, and anxiety. Some with hEDS carry formal diagnoses of additional conditions such as restless leg syndrome or sleep apnea.
Mast Cell Activation Disorder: Current understanding of mast cell activation syndrome (MCAS) in hEDS is limited.
Mast cell activation syndrome is an immune condition that can create allergy-like symptoms, up to and including anaphylaxis. More research is needed to see whether MCAS is a condition that occurs with greater frequency in hEDS than in the general population and how it affects a person with hEDS and the management.
Psychiatric: hEDS is not in the psyche, it is in the connective tissue, but it can affect the psyche.
Many with hEDS are assigned psychiatric diagnoses or frankly ignored when clinicians fail to recognize that they meet diagnostic criteria for hEDS. Clearly, this leads to a failure in attempts to effectively care for the person with hEDS. It is important to realize that psychological conditions (e.g. anxiety or depression) are common in chronic conditions including hEDS. Ignoring significant coexisting psychological problems will lead to suboptimal treatment.
Quality of Life: Studies to date consistently suggest that hEDS adversely affects quality of life.
Carefully designed studies have clearly demonstrated that quality of life is often measured or reported as lower in people with EDS than in the general population&thinsp&mdash&thinspin particular, people with EDS and associated secondary issues such as digestive system disorders, anxiety, depression, physical pain.
Recognizing hEDS is only half of the battle. Diagnosis alone is not enough. As with any disorder, effective management of hEDS includes recognizing its complexity. A body of evidence-based standards of care exists and includes the efforts of multiple healthcare practitioners. Management of hEDS must follow established standards of care and include treatment of both immediate and long-term issues as well as focusing on preventative care.
Clinicians, particularly surgeons and anesthesiologists, caring for a patient with hEDS should familiarize themselves with the management standards as they appear in the full publication (http://bit.ly/2017hEDS).
Key points for management of conditions in hEDS include:
- The approach should be holistic focusing on the complications, the desire(s) of the patient, quality of life and functionality, as well as psychological aspects.
- Results should not be expected overnight: It often takes months of routine toning exercise to stop deterioration, and it may take years before substantial reduction in pain is recognized. Fatigue, like pain, often responds to treatment such as exercise therapy but only very slowly.
- Use of multiple medications together, physical therapy, and complementary medicine is often more effective than as-needed use of one or two medications at a time. Some patients who continue to struggle to cope with their pain may need a broad pain management program. The overall goal should be to maintain adequate control of pain to a tolerable level, not to completely eliminate it.
- Management of psychiatric issues should include consideration of counseling and cognitive behavioral therapy, in addition to consideration of techniques such as distraction, hypnosis, and careful consideration of drug therapy.
While refinement of the diagnostic criteria of hEDS has been important, the search for genetic causes is crucial to identify, study, and ultimately treat patients with the disorder. hEDS is the only type of Ehlers&ndashDanlos syndrome without a known molecular defect. In order to solve the puzzle and identify how hEDS comes about, tools such as a database registry will be imperative. Many further studies are urgently needed to better guide therapy. Studies could investigate physical therapy, pain management, imaging and measurements, and MCAS in the hEDS population.
Every clinician should expect to encounter a number of patients with hEDS during the course of their practice. Further research is desperately needed, and hEDS must be on every clinician&rsquos radar. It is nothing short of negligent for clinicians to ignore these established standards of care for this disorder, as it has significant potential for negative impact on health-related quality of life.
Acknowledgments. We thank Gary Resnick and John C. Detter, Bioscience Division, Los Alamos National Laboratory, for their project contributions and helpful discussions.
Financial support. This work was supported by the Infant Botulism Treatment and Prevention Fund of the California Department of Public Health.
Potential conflicts of interest. All authors: No reported conflicts.
All authors have submitted the ICMJE Form for Disclosure of Potential Conflicts of Interest. Conflicts that the editors consider relevant to the content of the manuscript have been disclosed.