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Hardtop

A hardtop is a rigid form of automobile roof, typically metal, and integral to the vehicle's design, strength, and style. The term typically applies to a pillarless hardtop, a car body style without a B-pillar. The term "pillared hardtop" was used in the 1970s to refer to cars that had a B-pillar but had frameless door glass like a pillarless hardtop.

In limited cases, a hardtop roof can be detachable (often designed to store in the trunk), or retractable within the vehicle itself.

Pillarless hardtop

The pillarless hardtop (abbreviated as "hardtop") is a post-World War II car body designed with no center or B-pillar or glass frames. If window glass frames are present, they are designed to retract with the window when lowered. This creates an impression of uninterrupted glass along the side of the car. Even the smaller automakers like Packard introduced two-door hardtops in 1952 "as a response to America's newly discovered fondness for sportier looking cars that resembled a convertible, but had the comfort and convenience of a two-door sedan."

In one instance, stylists added faux ribbing to a hardtop roof to make it appear to be like a convertible top,

By the mid-1960s, optional vinyl roofs became widely available in virtually all model lines, reinforcing the convertible look in hardtops.

A pillarless hardtop is inherently less rigid than a pillared body, requiring extra underbody strength to prevent shaking. Production hardtops commonly share the frame or reinforced body structure of the contemporary convertible model, which is reinforced to compensate for the lack of a fixed roof. The hardtop design received criticism that its roof structure may not provide adequate protection during a rollover crash. However, subsequent research reported that rates of serious or fatal injury in hardtop models (both two- and four-door versions), as well as four-door station wagons, were significantly lower than sedans while the rates of any degree of injury for those same body styles were also significantly lower. A 1980 study for evaluation of rollover test devices reported that a greater degree of roof crush was associated with hardtop body styles, but severe passenger injury was not more frequent than in the other car body styles.

The hardtop body style began to disappear along with convertibles in the mid-1970s, partly out of a concern that U.S. federal safety regulations would be onerous for pillarless models to pass. The ascendancy of monocoque construction also made the pillarless design less practical. Some models adopted modified roof styling, placing the B-pillars behind the tinted side window glass and painting or molding the outer side of each pillar in black to make them less visible, creating a hardtop look without actually omitting the pillar. Some mid- to late-1970s models continued their previous two-door hardtop bodies, but with fixed rear windows or a variety of vinyl roof and opera window treatments.

By the end of the 1990s, almost all hardtop designs were discontinued as structural integrity standards increased.

Hardtops were typically more expensive than regular sedan models when new. They are more collectible than standard models of the same vehicle, which have a B-pillar.

Origins
Early automobiles had no roof or sides, but by 1900, several cars were offered with fabric roofs and primitive folding tops. However, cars with fully closed bodies (i.e., with a rigid roof and sides) grew in popularity and soon became the norm.

In 1915–1918, the first pillarless hardtop cars were produced, then called "convertible cars" (or "touring sedans" or "Springfields"). The Springfield design featured folding upper frames on the doors and the rear glass frames are removable and stored under or behind the seats. In the late teens, Cadillac offered a sedan with removable "B" pillars.

Another form of early pillarless hardtop is the "California top", originating in Los Angeles and most popular from 1917 to 1927. These were designed to replace the folding roofs of touring cars, to enclose the sides of the car for better weather protection. One objective of these aftermarket tops was to bring the cost of the closed car nearer to the prices of corresponding open cars. Automobile dealers were encouraged to equip an open car with a California top to demonstrate that they were "cool and clean in summer, and warm and dry in winter." The hard tops were frequently equipped with celluloid windows that retracted like a roller blind for open sided motoring offering a low-cost compromise between an open and closed car.

United States
There were a variety of hardtop-like body styles dating back to 1916. Chrysler Corporation built seven pillarless Town and Country hardtop coupes as concept vehicles in 1946, and even included the body style in its advertising that year called the Town and Country Custom Club Coupe. In 1951, Plymouth offered the Cranbrook Belvedere as a low priced hardtop two-door until 1953.

Mass-production of hardtops began with General Motors, which launched two-door, pillarless hardtops in 1949 as the Buick Roadmaster Riviera, Oldsmobile 98 Holiday, and Cadillac Coupe de Ville. They were purportedly inspired by the wife of a Buick executive who always drove convertibles, but never lowered the top.

The Kaiser-Frazer 1949 Virginian was an early example of a four-door hardtop albeit with a removable thin, chrome- and-glass 'B' pillar held on by five screws. The car was designed to have a convertible look and padded nylon or cotton was applied over the roof contributing to the soft-top appearance.

Two-door hardtops became popular with consumers in the 1950s, while the two-door sedan body design fell out of favor among buyers.

In 1955, General Motors introduced the first four-door hardtops. Following the pattern established by the two-door variants, GM utilized the same special sub-designations for the pillarless four-door body types within all their brands in North America. The term de Ville was used for Cadillac, Riviera was used for Buick, Holiday was used for Oldsmobile, Catalina was used for Pontiac, and Bel Air was used for Chevrolet.

Other manufacturers also designated unique names for their pillarless models. Ford called them Victoria, Chrysler used Newport, and their luxury division Imperial used the name Southampton, Packard named them Mayfair, and Hudson's were Hollywoods. Nash used the Country Club moniker while pillarless Studebakers were Starliners, a name that was later used by Ford for its Galaxie hardtop.

By 1956, every major U.S. automaker offered two- and four-door hardtops in a particular model lineup. General Motors restyled their new models and offered four-door hardtops from every division and nearly every series except the lowest-priced lines. Chrysler also offered two- and four-door hardtops for every brand, from Imperial, Chrysler, DeSoto, Dodge, and Plymouth.

In 1956, the first four-door hardtop station wagons were introduced to the Rambler line by American Motors Corporation. The following year, the Mercury Commuter hardtop wagons became available in both two- and four-door body styles. Chrysler built four-door hardtop station wagons through 1964 in both the Chrysler and Dodge 880 lines.

Throughout the 1960s, the two-door pillarless hardtop was the most popular body style in most lines where such a model was offered. Even on family-type vehicles like the Chevrolet Impala, the two-door hardtop regularly outsold four-door sedans. Some car lines (such as the 1957-64 Cadillac and 1965–69 Corvair) only offered pillarless models with no pillared sedans. So prevalent were true hardtops that Popular Mechanics had to describe that the new full-sized 1967 Oldsmobile Delmont models even included a "pillar" sedan.

The U.S. industry's last pillarless two-door and four-door hardtops were in the 1978 Chrysler Newport and New Yorker lines. Since then, no U.S. manufacturer has offered a true hardtop in regular production.

Europe

Various European manufacturers have produced hardtops without B-pillars (usually coupes). However, they are rarely marketed as pillarless hardtops. Examples include the current Bentley Continental GT, the 2008 Bentley Brooklands, the 2001-2003 Renault Avantime, the Rolls-Royce Wraith, and the 2012-2017 Ford B-Max. The 1958-1964 Facel Vega Excellence is one of few four-door hardtops produced in Europe.

German pillarless hardtops included cars from Taunus, a Ford subbrand. Namely the Ford Taunus P5 and Ford P7 including both P7A and P7B versions. They had regular coupe versions with B pillars, but the Hardtop Taunus models had no B pillar and provided an open-air feel inside the car when all the windows were rolled down. DKW was the only German manufacturer that offered pillarless two-door windows, as well as wrap-around windows in the back of the glasshouse from 1953 (3=6 F91) on and with a wrap-around windscreen since 1959 (1000 Coupe).

British pillarless hardtops included the Sunbeam Rapier and the Ford Consul Capri (355) which, unlike American models, sold fewer cars than their regular center pillar saloon versions.

A New Mini two-door sedan has been marketed as a hardtop in the U.S. includes a structural B-pillar on the inside that is disguised on the exterior by a black border on the fixed rear windows.

The Mercedes E-class coupe W213 from 2016 is an example of a modern pillarless design.

Japan
In July 1965, Toyota introduced Japan's first two-door hardtop in the third generation Toyota Corona line. This was followed by several manufacturers offering the popular body style as a luxury car appearance. During the 1970s, Toyota produced the Toyota Crown in a genuine two-door hardtop, while offering a pillared four-door hardtop sedan.

Nissan followed suit with the Nissan Cedric and Nissan Gloria but offered a genuine pillarless four-door hardtop along with a two-door hardtop body styles, with the latter "rendered as a premium quality personal car." Subaru introduced a new compact coupe as a genuine two-door hardtop with the Subaru Leone in 1971. The pillarless hardtop models were more expensive and luxurious than the sedan versions.

In the 1980s, Toyota continued the design with a pillared four-door hardtop Mark II, while Nissan again offered a genuine hardtop sedan with its Laurel. Toyota introduced a genuine four-door hardtop with the Toyota Carina ED followed by the Toyota Corona EXiV. Mazda marketed its Luce and Subaru from 1989 until 2009 with their Legacy/Outback sedans and station wagons as a pillared hardtop.

Detachable hardtops

A detachable hardtop is a rigid, removable roof panel that is often stored in a car's trunk/boot.

Retractable hardtops

A retractable hardtop (also known as coupé convertible or coupé cabriolet) is a type of convertible that forgoes a folding textile roof in favor of an automatically operated, multi-part, self-storing roof where the rigid roof sections are opaque, translucent, or independently operable.

title: Hardtop


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Osteogenesis imperfecta

Osteogenesis imperfecta (OI), colloquially known as brittle bone disease, is a group of genetic disorders that all result in bones that break easily. The range of symptoms—on the skeleton as well as on the body's other organs—may be mild to severe. Symptoms found in various types of OI include whites of the eye (sclerae) that are blue instead, short stature, loose joints, hearing loss, breathing problems and problems with the teeth (dentinogenesis imperfecta). Potentially life-threatening complications, all of which become more common in more severe OI, include: tearing (dissection) of the major arteries, such as the aorta; pulmonary valve insufficiency secondary to distortion of the ribcage;  and basilar invagination.

The underlying mechanism is usually a problem with connective tissue due to a lack of, or poorly formed, type I collagen. In more than 90% of cases, OI occurs due to mutations in the COL1A1 or COL1A2 genes. These mutations may be hereditary in an autosomal dominant manner but may also occur spontaneously (de novo). There are four clinically defined types: type I, the least severe; type IV, moderately severe; type III, severe and progressively deforming; and type II, perinatally lethal. , 19 different genes are known to cause the 21 documented genetically defined types of OI, many of which are extremely rare and have only been documented in a few individuals. Diagnosis is often based on symptoms and may be confirmed by collagen biopsy or DNA sequencing.

Although there is no cure, most cases of OI do not have a major effect on life expectancy, death during childhood from it is rare, and many adults with OI can achieve a significant degree of autonomy despite disability. Maintaining a healthy lifestyle by exercising, eating a balanced diet sufficient in vitamin D and calcium, and avoiding smoking can help prevent fractures. Genetic counseling may be sought by those with OI to prevent their children from inheriting the disorder from them. Treatment may include acute care of broken bones, pain medication, physical therapy, mobility aids such as leg braces and wheelchairs, vitamin D supplementation, and, especially in childhood, rodding surgery. Rodding is an implantation of metal intramedullary rods along the long bones (such as the femur) in an attempt to strengthen them. Medical research also supports the use of medications of the bisphosphonate class, such as pamidronate, to increase bone density. Bisphosphonates are especially effective in children; however, it is unclear if they either increase quality of life or decrease the rate of fracture incidence.

OI affects only about one in 15,000 to 20,000 people, making it a rare genetic disease. Outcomes depend on the genetic cause of the disorder (its type). Type I (the least severe) is the most common, with other types comprising a minority of cases. Moderate-to-severe OI primarily affects mobility; if rodding surgery is performed during childhood, some of those with more severe types of OI may gain the ability to walk. The condition has been described since ancient history. The Latinate term osteogenesis imperfecta was coined by Dutch anatomist Willem Vrolik in 1849; translated literally, it means "imperfect bone formation".

Orthopedic
The main symptom of osteogenesis imperfecta is fragile, low mineral density bones; all types of OI have some bone involvement. In moderate and especially severe OI, the long bones may be bowed, sometimes extremely so. The weakness of the bones causes them to fracture easily—a study at the Endocrine Unit at the National Institute of Child Health in Karachi, Pakistan found an average of 5.8 fractures per year in untreated children. Fractures typically occur much less after puberty, but begin to increase again in women after menopause and in men between the ages of 60 and 80.

Joint hypermobility is also a common sign of OI, thought to be because the affected genes are the same as those that cause some types of Ehlers–Danlos syndrome.

Otologic
By the age of 50, about 50% of adults with OI experience significant hearing loss, much earlier as compared to the general population. Hearing loss in OI may or may not be associated with visible deformities of the ossicles and inner ear. Hearing loss frequently begins during the second, third, and fourth decades of life, and may be conductive, sensorineural, or a combination of both ("mixed"). If hearing loss does not occur by age 50, it is significantly less likely to occur in the years afterwards. Mixed hearing loss is most common among those with OI of all age groups, while conductive hearing loss is most likely to affect older people, with sensorineural hearing loss most likely to affect children.

Although relatively rare, OI-related hearing loss can also begin in childhood; in a study of forty-five children aged four to sixteen, two were found to be affected, aged 11 and 15. In a different 2008 study, the hearing of 41 people with OI was checked. The results showed that 88% of those over 20 years of age had some form of hearing loss, while only 38% of those under 20 did.

Hearing loss is most common in type I OI; it is less common in types III and IV. Other parts of the inner ear may also be affected by OI. causing balance issues; however, only small studies have found links between vertigo and OI. OI may worsen the outcome of medical treatments which correct hearing loss.

Besides OI's association with sensorineural hearing loss, OI is associated with a number of neurological abnormalities, usually involving the central nervous system, due to deformities in the skeletal structures surrounding it. Neurological complications, especially basilar invagination, may adversely affect life expectancy. In OI, this is most often due to upwards migration of the dens,  a feature of the C2 vertebra. Neurosurgery may be needed to correct severe abnormalities when they risk the patient's life or cause either great suffering or intolerable neurological deficits.

Systemic
As its biological causes have been more precisely determined, it has become more widely recognized that while the primary disease process of OI happens in the bones, the most common types of OI—those caused by type I collagen gene mutations—affect virtually all of the human body's organs in some way.

Type I collagen is present throughout the circulatory and respiratory systems: from the ventricles of the heart itself, to the heart valves, to the vasculature, it is an integral part of the connective tissue of the lungs. As such, cardiovascular complications, among them aortic insufficiency, aortic aneurysm, and arterial dissections, are sometimes comorbid with OI, but not as frequently as they are comorbid with Marfan syndrome.

Respiratory illnesses are a major cause of death in OI. The most obvious source of respiratory problems in OI is pulmonary insufficiency caused by problems in the architecture of the thoracic wall. However, respiratory tract infections, such as pneumonia, are also more fatal among those with OI than the general population. Those with more severe ribcage deformities were found to have worse lung restriction in a small-scale 2012 study involving 22 Italian patients with OI types III and IV, plus 26 non-affected controls.

OI—especially its severe form type III—also has effects on the gastrointestinal system. It was found to be associated with recurrent abdominal pain and chronic constipation in two studies on patients affected by OI. Chronic constipation is especially common, and is thought to be aggravated by an asymmetric pelvis (acetabular protrusion). Especially in childhood, OI-associated constipation may cause a feeling of fullness and associated food refusal, leading to malnutrition.

Classification
There are two typing systems for OI in modern use. The first, created by David Sillence in 1979, classifies patients into four types, or syndromes, according to their clinical presentation, without taking into account the genetic cause of their disease. The second system expands on the Sillence model, but assigns new numbered types genetically as they are found. Therefore, people with OI can be described as having both a clinical type and a genetic type, which may or may not be equivalent.

Type I is the most common, and 90% of cases result from mutations to either COL1A1 or COL1A2. Symptoms vary widely between types, as well as vary from person to person, even in the same family.

As of 2021, 21 types of OI have been defined:

Sillence types
Sillence's four types have both a clinical and a genetic meaning; the descriptions below are clinical and can be applied to several genetic types of OI. When used to refer to a genetic as well as a clinical type, it indicates that the clinical symptoms are indeed caused by mutations in the COL1A1 or COL1A2 genes which are inherited in an autosomal dominant fashion.

Type I
Collagen is of normal quality but is produced in insufficient quantities. Bones fracture more easily than in the general public, but not as easily as more severe types of OI; there might be scoliosis, albeit mild compared to OI types III and IV, with a lower Cobb angle; the joints may be loose; blue sclerae may be apparent; hearing loss is likely to occur; and there might be a slight decrease in height. Because cases exist missing one or more of these symptoms, OI type I in some cases goes undetected into adulthood.

Some further split type I into types I–A and I–B, defined as being distinguished by the absence (I–A) or presence (I–B) of dentinogenesis imperfecta (opalescent teeth). People with type I generally have a normal lifespan.

Type II
Collagen is fatally defective at its C-terminus. Most cases result in death shortly after birth, or within the first year of life, due to respiratory failure. Another common cause of death is intracranial bleeds from skull fractures present at, or sustained during or shortly after, birth. In many cases, the newborn already has multiple broken bones at the time of birth. Type II infants also exhibit severe respiratory problems, and have severely deformed bones. Sixty percent of infants die less than 24 hours after being born, and survival after the first year is extremely unlikely and normally requires mechanical ventilation. In the rare cases of infants who survive their first year of life, severe developmental and motor delays are seen; neither of two infants studied in 2019, both aged around two years, had achieved head control, and both required a ventilator to breathe.

Type II is also known as the "lethal perinatal" form of OI, and is not compatible with survival into adulthood. Due to similarly severely deformed bones, sometimes infants with severe type III are wrongly initially classified as type II; once long-term survival is shown, they are considered as having type III instead.

Type III
Collagen quantity is sufficient, but is not of a high enough quality. Clinical differentiation between types III and IV is not always simple, and is further confounded by the fact that an untreated adult with type IV may have worse symptoms than a treated adult with type III; features only found in type III are its progressively deforming nature  and the presence of a face with a "triangular" appearance. Another differentiating factor between type III and IV is blue sclerae; in type III, infants commonly have blue sclerae that gradually turn white with age, but blue sclerae are not commonly seen in type IV, although they are seen in 10% of cases.

OI type III causes osteopenic bones that fracture very easily, sometimes even in utero, often leading to hundreds of fractures during a lifetime; early scoliosis that progresses until puberty; dwarfism (a final adult height frequently less than 4 ft); loose joints; and possible respiratory problems due to low rib cage volume causing low lung volumes.

Due to the severity of the issues with the bones, neurological and seizure disorders are more likely to develop in type III. Basilar invagination, which puts pressure on the brainstem, may cause or contribute to early death; surgical treatment of it is more complex in OI cases.

Type IV
Collagen quantity is sufficient, but is not of a high enough quality. Type IV is for cases of variable severity, which do not fit into either types III or I. While one of Sillence's required characteristics for type IV was having normal sclerae,  modern classification allows even those with blue sclerae to fit the criteria for type IV if they meet the other clinical requirements of the type.

In type IV, bone deformity can be mild to severe, bones fracture easily (especially before puberty), dwarfism is common, vertebral collapse and scoliosis are evident, and hearing loss is possible, although uncommon. Type IV OI is mostly defined in contrast to type III and type I, being the clinical classification for patients somewhere in the middle ground between the two. As such, type IV OI is often termed "variable" OI, with the severity of even those in the same family (so, with the same genetic mutation) differing.

Prepubertal bone fracture rates are another way of clinically assessing type IV OI—those with it tend to have fracture rates of ≈1 per year, compared to ≈3 per year for severe OI (type III).

As in type I, some further split type IV into types IV–A and IV–B, defined again by the absence (IV–A) or presence (IV–B) of dentinogenesis imperfecta.

Genetically defined types (types V–XXI)
As of 2020, fifteen types of OI are defined genetically:

 * Type V – Having the same clinical features as type IV, it can be clinically distinguished by observing a "mesh-like" appearance to a bone biopsy under a microscope. Type V can be further distinguished from other types of OI by the "V triad": an opaque band (visible on X-ray) adjacent to the growth plates; hypertrophic calluses (abnormally large masses of bony repair tissue) which form at fracture sites during the healing process; and calcification of the interosseous membrane of the forearm, which may make it difficult to turn the wrist.  Other features of this condition may include pulled elbow, and, as in other types of OI, long bone bowing and hearing loss.  Cases of this type are caused by mutations in the IFITM5 gene on chromosome 11 p15.5. The separation of type V from type IV OI, its clinical type, was initially suggested even before its genetic cause was known, by Glorieux et al. in 2000. Type V is relatively common compared to other genetically defined types of OI—4% of OI patients at the genetics department of the Brazilian Hospital de Clínicas de Porto Alegre were found to have it.
 * Type VI – With the same clinical features as type III, it is distinguished by bones which have an appearance similar to that seen in osteomalacia. Type VI is caused by a loss-of-function mutation in the SERPINF1 gene on chromosome 17 p13.3.
 * Type VII – OI caused by a mutation in the gene CRTAP on chromosome 3 p22.3; clinically similar to OI types II and III, depending on affected individual. Type VII was the first recessive OI type confirmed, initially found among First Nations people in Quebec.
 * Type VIII – OI caused by a mutation in the gene LEPRE1 on chromosome 1 p34.2; clinically similar to OI types II and III, depending on affected individual.
 * Type IX – OI caused by homozygous or compound heterozygous mutation in the PPIB gene on chromosome 15 q22.31.
 * Type X – OI caused by homozygous mutation in the SERPINH1 gene on chromosome 11q13.
 * Type XI – OI caused by mutations in FKBP10 on chromosome 17q21. The mutations cause a decrease in secretion of trimeric procollagen molecules. Other mutations in this gene can cause autosomal recessive Bruck syndrome, which is similar to OI.
 * Type XII – OI caused by a frameshift mutation in SP7 on chromosome 12 q13.13. This mutation causes bone deformities, fractures, and delayed tooth eruption.
 * Type XIII – OI caused by a mutation in the bone morphogenetic protein 1 (BMP1) gene on chromosome 8 p21.3. This mutation causes recurrent fractures, high bone mass, and hypermobile joints.
 * Type XIV – OI caused by mutations in the TMEM38B gene on chromosome 9 q31.2. This mutation causes recurrent fractures and osteopenia, although the disease trajectory is highly variable.
 * Type XV – OI caused by homozygous or compound heterozygous mutations in the WNT1 gene on chromosome 12q13.12. It is autosomal recessive.
 * Type XVI – OI caused by mutations in the CREB3L1 gene on chromosome 11p11.2. The homozygous mutation causes prenatal onset of recurrent fractures of the ribs and long bones, demineralization, decreased ossification of the skull, and blue sclerae; it is clinically type II or type III. Family members who are heterozygous for OI XVI may have recurrent fractures, osteopenia and blue sclerae.
 * Type XVII – OI caused by homozygous mutation in the SPARC gene on chromosome 5 q33, causing a defect in the protein osteonectin, which leads to severe disease characterized by generalized platyspondyly, dependence on a wheelchair, and recurrent fractures.
 * Type XVIII – OI caused by homozygous mutation in the FAM46A gene on chromosome 6 q14.1. Characterized by congenital bowing of the long bones, Wormian bones, blue sclerae, vertebral collapse, and multiple fractures in the first years of life.
 * Type XIX – OI caused by hemizygous mutation in the MBTPS2 gene on chromosome X p22.12. Thus far, OI type XIX is the only known type of OI with an X-linked recessive pattern of inheritance, making it the only type that is more common in males than females. OI type XIX disrupts regulated intramembrane proteolysis, which is critical for healthy bone formation.
 * Type XX – OI caused by homozygous mutation in the MESD gene on chromosome 15q25.1. Initial studies of type XX indicate that it may cause global developmental delay, a first among OI types. OI type XX disrupts the Wnt signaling pathway, which is thought to have a role in bone development.
 * Type XXI – OI caused by homozygous mutation in the KDELR2 gene on chromosome 7 p22.1. Causes disease clinically similar to types II and III, thought to be related to inability of chaperone protein HP47 to unbind from collagen type I, as to do so it needs to bind to the missing ER lumen protein retaining receptor 2 protein encoded by KDELR2.

Given the rapid rate of type discovery, it is extremely likely that there are other genes associated with OI that have yet to be reported.

Genetics
Osteogenesis imperfecta is a group of genetic disorders, all of which cause bone fragility. OI has high genetic heterogeneity, that is, many different genetic mutations lead to the same or similar sets of observable symptoms (phenotypes).

The main causes for developing the disorder are a result of mutations in the COL1A1 and/or COL1A2 genes which are jointly responsible for the production of collagen type I. Approximately 90% of people with OI are heterozygous for mutations in either the COL1A1 or COL1A2 genes. There are several biological factors that are results of the dominant form of OI. These factors include: intracellular stress; abnormal tissue mineralization; abnormal cell to cell interactions; abnormal cell-matrix interactions; a compromised cell matrix structure; and, abnormal interaction between non-collagenous proteins and collagen.

Previous research led to the belief that OI was an autosomal dominant disorder with few other variations in genomes. However, with the lowering of the cost of DNA sequencing in the wake of 2003's Human Genome Project, autosomal recessive forms of the disorder have been identified. Recessive forms of OI relate heavily to defects in the collagen chaperones responsible for production of procollagen and the assembly of the related proteins. Examples of collagen chaperones that are defective in patients with recessive forms of OI include chaperone HSP47 (Cole-Carpenter syndrome) and FKBP65. Mutations in these chaperones result in an improper folding pattern in the collagen 1 proteins which causes the recessive form of the disorder. There are three significant types of OI that are a result of mutations in the collagen prolyl 3-hydroxylation complex (components CRTAP, P3H1, and CyPB). These components are responsible for the modification of collagen α1(l)Pro986. Mutations in other genes such as SP7, SERPINF1, TMEM38B and BMP1 can also lead to irregularly formed proteins and enzymes that result in other recessive types of osteogenesis imperfecta.

Defects in the proteins pigment epithelium-derived factor (PEDF) and bone-restricted interferon-induced transmembrane protein (BRIL) are the causes of type V and VI osteogenesis imperfecta. Defects in these proteins lead to defective bone mineralization which causes the characteristic brittle bones of osteogenesis imperfecta. A single point mutation in the 5′ untranslated region (5′ UTR) of the IFITM5 gene, which encodes BRIL, is linked directly to OI type V.

In the rare case of type XIX, first discovered in 2016, OI is inherited as an X-linked genetic disorder, with its detrimental effects resulting ultimately from a mutation in the gene MBTPS2. Genetic research is ongoing, and it is uncertain when all the genetic causes of OI will be identified, as the number of genes that need to be tested to rule out the disorder continue to increase.

In a study of 37 families, a 1.3% chance was found that OI recurs in multiple siblings born to two unaffected parents—this is a much higher rate than would be expected if all such recurrences were de novo. The cause is genetic mosaicism; that is, some of, or most of, the germ cells of one parent have a dominant form of OI, but not enough of their somatic cells do to cause symptoms or obvious disability in the parent—the parent's different cells have two (or more) sets of slightly different DNA. It has been clinically observed that ≈5–10% of cases of OI types II and III are attributable to genetic mosaicism.

Pathophysiology
People with OI are either born with defective connective tissue, born without the ability to make it in sufficient quantities, or, in the rarest genetic types, born with deficiencies in other aspects of bone formation such as chaperone proteins, the Wnt signaling pathway, the BRIL protein, et cetera. In type I the collagen's structure itself is normal, it is just its quantity that is low. Types II, III and IV are usually, but not always, related to a deficiency of type I collagen. One possible deficiency arises from an amino acid substitution of glycine to a bulkier amino acid, such as alanine, in the collagen protein's triple helix structure. The larger amino acid side-chains lead to steric effects that creates a bulge in the collagen complex, which in turn influences both the molecular nanomechanics and the interaction between molecules, which are both compromised. Depending on both the location of the substitution and the amino acid being used instead, different effects are seen which account for the type diversity in OI despite the same two collagen genes being responsible for most cases. Replacements of glycine with serine or cysteine are seen less often in fatal type II OI, while replacements with valine, aspartic acid, glutamic acid, or arginine are seen more often.

At a larger scale, the relationship between the collagen fibrils and hydroxyapatite crystals to form bone is altered, causing brittleness. Bone fractures occur because the stress state within collagen fibrils is altered at the locations of mutations, where locally larger shear forces lead to rapid failure of fibrils even at moderate loads because the homogeneous stress state normally found in healthy collagen fibrils is lost. OI is therefore a multi-scale phenomenon, where defects at the smallest levels of tissues (genetic, nano, micro) domino to affect the macro level of tissues.

Diagnosis
Diagnosis is typically based on medical imaging, including plain X-rays, and symptoms. In severe OI, signs on medical imaging include abnormalities in all extremities and in the spine. As X-rays are often insensitive to the comparatively smaller bone density loss associated with type I OI, DEXA scans may be needed.

An OI diagnosis can be confirmed through DNA or collagen protein analysis, but in many cases, the occurrence of bone fractures with little trauma and the presence of other clinical features such as blue sclerae are sufficient for a diagnosis. A skin biopsy can be performed to determine the structure and quantity of type I collagen. While DNA testing can confirm the diagnosis, it cannot absolutely exclude it because not all mutations causing OI are yet known and/or tested for. OI type II is often diagnosed by ultrasound during pregnancy, where already multiple fractures and other characteristic features may be visible. Relative to control, OI cortical bone shows increased porosity, canal diameter, and connectivity in micro-computed tomography. OI can also be detected before birth by using an in vitro genetic testing technique such as amniocentresis.

Genetic testing
In order to determine whether osteogenesis imperfecta is present, genetic sequencing of the most common problematic genes, COL1A1, COL1A2, and IFITM5, may be done; if no mutation is found yet OI is still suspected, the other 10+ genes known to cause OI may be tested. Duplication and deletion testing is also suggested to parents who suspect their child has OI. The presence of frameshift mutations caused by duplications and deletions is generally the cause of increased severity of disease.

Differential diagnosis
An important differential diagnosis of OI is child abuse, as both may present to a clinician with multiple fractures in various stages of healing. Differentiating them can be difficult, especially when no other characteristic features of OI are present. This can become an issue in court; in the United States, several child abuse cases were resolved with a finding that osteogenesis imperfecta was the true cause of a child's fractures, leading to lawsuits seeking redress such as Alice Velasquez, et al. v. United States.

Other differential diagnoses include rickets and osteomalacia, both caused by malnutrition, as well as rare skeletal syndromes such as Bruck syndrome, hypophosphatasia, geroderma osteodysplasticum, and Ehlers–Danlos syndrome. Various forms of osteoporosis, such as iatrogenic osteoporosis, idiopathic juvenile osteoporosis, disuse osteoporosis and exercise-related osteoporosis should also be considered as explanations when OI is suspected.

Treatment
There is no cure for osteogenesis imperfecta. Maintaining a healthy lifestyle by exercising and avoiding smoking can help prevent fractures. Treatment may include care of broken bones, pain medication, physical therapy, mobility aids such as braces or wheelchairs, and surgery.

Judging the success or failure of treatment can be difficult in OI patients, as decreased bone fracture rates may just be coincidental. While these rates are often used in medical studies to judge treatment efficacy, a Norwegian study of fifteen people with OI emphasized that they feel doctors should consider the whole patient and not just fracture rates.

Acute bone fracture care
Bone fractures are treated in individuals with osteogenesis imperfecta in much the same way as they are treated in the general population—OI bone heals at the same rate as non-OI bone. A greater emphasis is placed on using lightweight materials to immobilize the fracture, as in moderate or severe types of OI, using heavy casts, such as hip spica casts, can cause fractures at the bones at the boundaries of the cast, as well as generalized osteopenia. The lightweight cast or splint is then replaced with a removable orthosis after a few weeks and once evidence of union is seen on X-ray. In order to prevent a nonunion or malunion, all fractures should be immobilized, even if the fracture seems trivial (microfracture), as people with OI are at greater risk of nonunion.

Bone infections secondary to fractures are treated as and when they occur with the appropriate antibiotics and antiseptics, as in the general population.

Bisphosphonates
In 1998, an initial observational trial demonstrated the effectiveness of intravenous pamidronate, a bisphosphonate which had previously been used in adults to treat osteoporosis. In severe OI, this trial showed that pamidronate reduced bone pain, prevented new vertebral fractures, reshaped previously fractured vertebral bodies, and reduced the number of long-bone fractures.

Although oral bisphosphonates are more convenient and cheaper, they are not absorbed as well, and intravenous bisphosphonates are generally more effective, although this is under study. Some studies have found oral and intravenous bisphosphonates, such as oral alendronate and intravenous pamidronate, equivalent. In a 2013 double-blind trial of children with mild OI, oral risedronate increased bone mineral densities, and reduced nonvertebral fractures. However, it did not decrease new vertebral fractures. A Cochrane review in 2016 concluded that though bisphosphonates seem to improve bone mineral density, it is uncertain whether this leads either to a reduction in bone fractures or improvement in the quality of life of individuals with osteogenesis imperfecta. Even in trials with as many as 125 children, no causal link has been found between bisphosphonates and decreased fracture rates; placebo controlled trials were also unable to prove that they brought about increased strength, motor control or lower pain levels.

Bisphosphonates are not as effective at increasing the bone mineral density of adults.

Nutritional supplements
OI is a genetic disorder and is not caused by insufficient intake of any vitamin or mineral; supplementation cannot cure OI. Nevertheless, people with OI tend to be severely deficient in vitamin D at much higher rates than the general population, and the cause of this is not well understood. The severity of the deficiency and the likelihood of its occurrence is thought to be related to severity of OI. Vitamin D supplementation may be recommended, at least until levels of 25(OH)D3 in a patient's blood return to normal. Vitamin D deficiency is also a concern as it may decrease the benefit of bisphosphanates.

Surgery
A surgery of any type inherently carries more risks when done on a patient who has (especially moderate to severe) OI. Skeletal deformities and dentinogenesis imperfecta may hinder access to the airway. Use of, and weaning off of, mechanical ventilation is also more challenging to carry out on patients with OI. During the procedure itself or the healing process, defective OI collagen may lead to bleeding diatheses.

The safety of anesthesia is also of more concern among patients with OI, with anesthetic complications 5.6x more likely to occur when the patient has OI type III. A unique concern of anesthesia in OI is perioperative fracture—fractures sustained due to patient transfer and airway access techniques that, while routine when a patient's bones are strong, may cause injury with brittle OI bones. As an example, due to a 1972 report of a humerus fracture from a sphygmomanometer cuff sustained in an OI patient during surgery, blood pressure monitoring protocols are often modified for patients with OI, with neonatal size cuffs and machine settings being used even in adults; further, the least deformed of the patient's limbs is preferred to receive the cuff.

Rodding
Metal rods can be surgically inserted in the long bones to improve strength, a procedure developed by Harold A. Sofield when he was Chief of Staff at Chicago's Shriners Hospitals for Children, a hospital that offers orthopedic care and surgery to children regardless of their family's ability to pay. Large numbers of children with OI came to Shriners, and Sofield experimented with various methods to strengthen their bones. In 1959, with Edward A. Millar [sic], Sofield wrote a seminal article describing a three-part surgery that seemed radical at the time: precisely breaking the bones ("fragmentation"), putting the resulting bone fragments in a straight line ("realignment"), then placing metal rods into the intramedullary canals of the long bones to stabilize and strengthen them ("rod fixation"). His treatment proved useful for increasing the mobility of people with OI, and it has been adopted throughout the world—it became standard surgical treatment for severe OI by 1979, in which year David Sillence found that ≈$2/3$ of the patients he surveyed with OI type III had undergone at least one rodding surgery.

Rodding surgery is often done with the hope that it will offer a path to ambulation, walking, to patients with moderate or severe OI. A 2020 review in The Journal of Bone and Joint Surgery (JB&JS) found it remains broadly popular: ≈$2/3$ of people with OI types III and IV (severe OI) have undergone some form of rodding surgery in their lives, at a mean age of $4 1/10$ and $7 1/2$ years respectively; one possible explanation for a tendency towards earlier intervention in type III is that one half of affected children could not walk at all without the surgery, as their limbs were more bowed, so surgery was sought sooner.

In those with type III OI who had undergone rodding surgery, 79.5% had the femurs and tibias of both legs rodded. The most common form of rods used are intramedullary (IM) rods, some of which, such as the Fassier–Duval IM rod, are telescoping, meaning that they are designed to grow as the child grows, in an attempt to avoid the necessity of revision surgeries. Telescoping IM rods are widely used, and the common Fassier–Duval IM rod is designed to be used to rod the femur, tibia, and humerus. The surgery involves breaking the long bones in between one and three (or more) places, then fixing the rod alongside the bone to keep it straight.

While telescoping IM rods are intended to grow along with both the femur and tibia in developing children; surgeons have a preference to use non-telescoping IM rods, such as Rush rods, in the tibia, which grows less comparatively—the JB&JS review found that while 69.7% of femurs were treated with telescoping IM rods, only 36.9% of tibiae were.

While the review in the JB&JS was able to correlate receiving rodding surgery with greater mobility across all types of OI, in patients with type IV, the surgery did not decrease the incidence of broken bones as compared to non-rodded patients—while type IV patients with rodded tibiae experienced 0.93 tibia fractures per year, patients with natural tibiae experienced only 0.81. However, in patients with type III, rodding surgery decreased the average number of tibia fractures per year from 0.84 to 0.57.

Spinal
Spinal fusion can be performed either as a preventative measure or to correct existing scoliosis, although the inherent fragility of OI bone makes this operation more complex in OI patients than it does with patients who have adolescent idiopathic scoliosis, but normal bone density. Despite the risks, however, three Nemours–duPont orthopedic surgeons who specialize in surgical intervention for osteogenesis imperfecta recommend operating if the curve is greater than 50° after a child is past peak height velocity, as the spine's curve can continue to worsen even into adulthood.

Due to the risk involved, the same surgeons recommend that surgery for basilar impressions and basilar invaginations should only be carried out if the pressure being exerted on the spinal cord and brain stem is causing actual neurological symptoms. Once basilar invagination has become symptomatic, only surgery can halt or reverse the progression of neurological deficits.

Physical therapy
Physical therapy is generally recommended, however individualized protocols are required due to the variability of OI. Physical therapy is used to strengthen muscles, improve motility, improve flexibility, and help with weight maintenance, although it must be done in a gentle manner to minimize the risk of bone fracture. In people with OI, exercise often involves water aerobics, light resistance exercises, and walking, if the patient is able. However, even in patients with mild OI, contact sports, as well as activities likely to put unnecessary stress on the joints, such as jumping, are contraindicated due to the risks they pose.

Individuals with more limited mobility are encouraged to change positions regularly throughout the day; people who sit in a wheelchair most or all of the day are recommended to get out of it every two hours, as a form of exercise, to decrease stiffness, and to prevent pressure ulcers.

Individuals with moderate to severe OI, who require assistive mobility devices and adapted vehicles, face significant barriers to access wheelchair-accessible pools or gyms—they either may not have any in their area, nor the means to get there. Obesity may be more likely to present among those with severe OI, (especially after the age of 20,) and can, in some, cause further declines in mobility.

Tilt table whole body vibration may also be done to increase the mobility of long-term immobilized (bedridden) patients with OI; in at least two cases it helped bedridden children to be able to sit upright.

Teeth
More than 1 in 2 people with OI also have dentinogenesis imperfecta (DI)—a congenital abnormality in the formation of dentin, one of the four major components of the human tooth. Dental treatment may pose as a challenge as a result of the various deformities, skeletal and dental, due to OI. Children with OI should go for a dental check-up as soon as their teeth erupt; this may minimize tooth structure loss as a result of abnormal dentine, and they should be monitored regularly to preserve their teeth and oral health.

Many people with OI are treated with bisphosphonates, and there are several possible related complications with dental procedures, for example, medication-related osteonecrosis of the jaw (MRONJ). However, no report of bisphosphonate-related MRONJ in either a child or adult with OI was found in a 2016 Cochrane review of the safety and efficacy of bisphosphonates for OI.

Monoclonal antibodies
Monoclonal antibodies have long been considered for OI, but as of 2021, such therapy has not been approved for OI, neither in the European Union nor in the United States. Thus, it is unclear whether they are safe or effective. Among the monoclonal antibodies that have been studied are romosozumab (targets sclerostin, by Amgen), fresolimumab (TGF-β, Sanofi), blosozumab (sclerostin, Lilly), and setrusumab (sclerostin, begun by Novartis).

Setrusumab, formerly known as BPS-804, is a monoclonal antibody that targets sclerostin, and has been studied in OI specifically more than any of the others. In the body, sclerostin binds to the LRP5 and LRP6 receptors, resulting in inhibition of the Wnt signaling pathway. This decreases bone formation, and is not a problem when a person has healthy bones. It is thought, though, that decreasing the concentration of sclerostin in the body may lead to the formation of more bone, and that is the premise as to why monoclonal antibodies that reduce the concentrations of naturally occurring sclerostin may help strengthen OI bone. While setrusumab was first developed at the pharmaceutical company Novartis, Novartis sold its rights to patent the drug to Mereo Biopharma in 2015, who has continued its development in conjunction with Ultragenyx. In 2019, Mereo announced that it had concluded collecting data for its phase II-B trial of setrusumab; the study was completed on 12 November 2020. Despite the trial data failing to show improvements in bone density on QCT scans, its primary goal, there were improvements on DXA scans. In a September 2020 press release, Mereo said it was seeking to do a phase III trial in 2021, and had received a Rare Pediatric Disease (RPD) designation from the US Food and Drug Administration (FDA).

Romosozumab, which also is a monoclonal antibody targeting sclerostin, is an approved drug in the US and EU for the treatment of osteoporosis. The pharmaceutical industry analyst Evercore has remarked that "it could wipe out setrusumab's economics", as romosozumab is priced more cheaply than a drug for a rare disease would be, claiming that it will be "vital" to Ultragenyx's profit margins to prove its setrusumab is more efficacious than romosozumab for OI. A clinical trial evaluating romosozumab's efficacy in OI began in September 2020 and as of September 2021 is ongoing. Ultragenyx predicts that its phase 2/3 trials for setrusumab will be completed in 2026.

Prevention

As a genetic disorder, the mainstay of twenty-first century prevention of osteogenesis imperfecta is based on preventing affected individuals from being born in the first place. Genetic counseling can help patients and their families determine what types of screening, if any, are right for their situation. Patients can consider preimplantation genetic diagnosis after in vitro fertilization to select fertilized embryos which are not affected. Common mutations which cause OI may be caught by exome sequencing and whole genome sequencing. If a pregnancy is already in progress, the procedure of amniocentesis may be undergone to see if the fetus is affected. If affected, it is up to the family to consider whether or not they want to terminate the pregnancy and try again—raising questions of medical ethics and a woman's right to choose.

Without intervention, patients with the most common mutations causing osteogenesis imperfecta have a 50% chance per gestation of passing on the disorder, as these mutations are inherited in an autosomal dominant pattern of Mendelian inheritance. Those with the rare autosomal recessive forms of OI have a 25% chance of passing on the disorder. Genetic testing of the affected members of the family can be used to determine which inheritance pattern applies.

As OI type I may be difficult to detect in a newborn child, the cord blood of the child can be tested to determine if it has been passed on if the family has already rejected the more invasive genetic screening methods. In more severe cases, the diagnosis may be able to be done via ultrasound, especially if OI is already a possibility. An ethical concern with prenatal screening for OI often arises when parents inquire as to how severely affected their child will be—such questions are as yet difficult to answer conclusively.

If a non-affected person has already had a child with OI, there is a greater likelihood (although still quite remote), that their future children will have OI due to genetic mosaicism.

The disability rights critique of prenatal screening for OI, held by some bioethicists and some affected individuals, negatively compares it to eugenics, with even those not opposed to abortion opposing selective abortions on the ethical ground that their existence betrays the belief that the lives of those with OI are "less worth living [and] less valuable".

Prognosis
The prognosis of osteogenesis imperfecta depends entirely on its type (see ).

Life expectancy
In the mild form of the disorder, type I, the life expectancy of patients is near that of the general population. In type II, however, patients only very rarely live past the age of two, and typically die in their first weeks of life. Assessment of the life expectancy of patients with types III and IV is more complicated, as lifestyle choices can cause fatally traumatic injuries that would not have otherwise occurred, or not been fatal in the general population. Life expectancy in type IV OI is thought to be close to normal, but in type III it is lower than in the general population.

A 2016 study of data in Denmark's National Patient Register found that across all types of OI, all-cause mortality was three times higher, leading to a loss of around seven years in females and nine years in males. A 1996 study published in the British Medical Journal found that mortality in type III OI is significantly higher, with many patients dying in their 20's, 30's, and 40's; patients who survive to the age of 10 were further found to have longer life expectancy than newborns.

Mobility
People with mild (type I) OI as adults need few pieces of adaptive equipment, although in infancy they reach motor milestones at a significant delay compared to the general population.

With adaptive equipment such as crutches, motorized wheelchairs, splints, reach extenders, and/or modifications to the home, many individuals with moderate to severe OI can achieve or maintain a significant degree of independence. With treatment and physical therapy, the maximum levels of mobility are expected to be unassisted community walking for type I, household or exercise walking for type III, and household or community walking for type IV; due to the variability of OI between individuals, mobility achieved varies and may be below this expected maximum.

Epidemiology
In the United States, the incidence of osteogenesis imperfecta is estimated to be one per 20,000 live births. An estimated 20,000 to 50,000 people are affected by OI in the United States.

The most common types are I, II, III, and IV, while the rest are very rare. Type I is the most common and has been reported to be around three times more common than type II. The prevalence of types III and IV is less certain. In a 1989 study in Denmark, type I was found to comprise 71% of cases and type II 12% of cases, with other types comprising the other 17%. In a 2015 study in Sweden, type I was nearly six times more common than type III and nearly four times more common than type IV.

Most people with OI receive it from a parent, but in many cases, it is a brand new (de novo or "sporadic") mutation in a family. Among a study of patients with survivable types of OI, OI type III is most often de novo (85%), followed by type IV (50%) and type I (34%).

Some populations can have a higher incidence of OI than would be otherwise expected if they have a larger than average number of carriers of the recessive forms of the disease.

History
The condition, or types of it, has had various other names over the years and in different nations; "osteogenesis imperfecta" has, however, been the most widely accepted name for the condition since the late 20th century. Among some of the most common alternatives are "fragilitas ossium"; "Ekman–Lobstein syndrome", and "Vrolik syndrome", both eponyms; and, the colloquialism, "brittle bone disease".

Earliest recorded cases
OI has been identified in an ancient Egyptian infant mummified in around 1000 BC, originally dismissed by archaeologists as containing the remains of a monkey. The Norse king Ivar the Boneless, who lived c. 800 CE, is speculated to have had OI as well.

Nicolas de Malebranche is oft credited as being the first person to describe the physical characteristics of OI in his 1688 book The Search after Truth, in which he describes a man who has had his "bones broken in the places a murderer's would be" all his life. His confident description of the pathology of the disorder, however, which creates what he termed «enfants monstrueux» ("monstrous children"), is scientifically void—he wrote that it was due to the mother's antepartum viewership of a public execution by breaking wheel.

The earliest modern scientific studies of OI began in 1788 by Olof Jakob Ekman, who described the condition, which he termed "osteomalacia congenital", in his doctoral thesis and mentioned cases of it going back to 1678, all in the same family, through three generations. Ekman's description of the condition mentioned dwarfism, bone fragility, and bowing of the long bones. In 1831, Edmund Axmann gave a detailed description of it in himself and his two brothers, being the first to mention blue sclerae as a characteristic sign of OI. Jean Lobstein first described the mild form of the condition, today known as type I, in 1833, calling it "osteopsathyrosis idiopathica".

It was not until 1912 that hearing loss was positively recognized as a symptom of OI, first mentioned in a brief paper by the English physician Charles Allen Adair-Dighton.

Of the term
Willem Vrolik, a Dutch anatomist who was also curator of the "Museum Vrolikianum", which made him privy to many specimens of bodies having birth defects, coined the term "osteogenesis imperfecta" in his bilingual Latin and Dutch language book on teratology, Illustrations of Human and Mammalian Embryogenesis, first published in 1849.

Included is a description of the remains of an infant who had what is now known as perinatally fatal OI type II  (as verified in a 1998 re-examination of the remains by Baljet et al.). The remains were first given to Vrolik's father, who could not make sense of them. Vrolik described poorly mineralized bones, bowed long bones, and fractures in various states of healing. Vrolik correctly determined that what he termed OI in the infant was not caused by secondary rickets, but a congenital abnormality causing primary osteopenia; he theorized this was due to a lack of "intrinsic generative energy".

Of its classification
Classification of OI has also evolved as scientific understanding of it has improved. Before the advent of modern genetic testing, OI was classified in two broad groups: osteogenesis imperfecta congenita, and osteogenesis imperfecta tarda, a division first proposed by the German physician E. Looser in 1906. Congenita was used to describe the modern clinical types II, III, and some cases of IV, where upon birth the condition was obvious, either due to bowing of the limbs or due to fractures sustained in utero. Tarda was used to classify the modern OI type I and some cases of type IV, where the inherent fragility of the bones did not become clear until long after birth. The idea that these "late" and "prenatal" forms were manifestations of the same disorder was first proposed in 1897 by Martin Benno Schmidt; by the 1950s this fact was well accepted.

The modern system of four types (I, II, III, IV), meanwhile, were introduced in a paper by David Sillence, Alison Senn, and David Danks in the Journal of Medical Genetics in 1979, and have since become standard terms among doctors, patients, and researchers. The modern genetic types, (those with numbers greater than IV,) have come into use as more and more recessively inherited forms of OI have been discovered since the discovery of the first one by Roy Morello et al. in 2006. In 2010, the International Nomenclature Group for Constitutional Disorders of the Skeleton (INCDS) "freed" the Sillence types from molecular reference, acquiescing to their new clinical-first role in the wake of what was to them a "surprising" increase in the number of genetic causes of OI.

Writing for the Annual Review of Genetics in 2012, Drs. Peter Byers and Shawna M. Pyott lamented how the expansion of the number of types to include genetic types has created a system that "grew like Topsy". They suggest that it may indeed be impossible to create a system which is useful for clinicians and which accurately describes the genetic cause of a person's OI, with attempts always prioritizing one use at the expense of the other.

Society and culture
Much medical research has been done into the causes of osteogenesis imperfecta, benefiting not only those with OI but medicine more broadly; in the ten years between 2006 and 2016, the many discoveries of non-collagen related recessive gene mutations, which still led in those who have them to the clinical signs of OI, led to numerous breakthroughs in medical understanding of the process of healthy bone development.

Other animals
In dogs, OI is an autosomal recessive condition, meaning that dogs with two copies of the allele will be affected. Many breed organizations and veterinarians offer OI tests to tell if a dog is a carrier of OI. To prevent OI, dogs who are heterozygous for OI should only be bred to non-carriers.

Naturally occurring mutations causing OI have been found in Golden Retrievers, Dachshunds, and Beagles. OI has also been identified in zebrafish and mice.

Although dogs, mice, fish, and humans are not genetically identical, some of these animal models have been officially recognized to represent the varying types of OI in humans. For example, homozygous oim/oim mice experience spontaneous bone fractures, small body size, and kyphosis, making them a model of OI type III. Meanwhile, heterozygous oim/+ mice appear normal but have bones which are quite a bit weaker than wild mice, making them a model for OI type I. As in human OI, the location on the gene which is mutated affects the severity of resulting disease—the G859C Col1a1 mouse is a model for OI type II as affected mice all die in the perinatal period.

Animal testing on identified animal models may lead to human therapies for OI.

license: Creative Commons - Attribution Share-Alike - https://creativecommons.org/licenses/by-sa/4.0/

title: Osteogenesis imperfecta


text:
Talk:Internal Macedonian Revolutionary Organization

Splitting the IMRO/VMRO article in two separate articles 1) Internal Macedonian Revolutionary Organization (IMRO/VMRO) and 2) Bulgarian Internal Macedonian Revolutionary Organization (BIMRO/BVMRO)
If someone is reading this article he or she might think that the people from IMRO/VMRO were a bit confused if they were Bulgarian IMRO or just IMRO. That was not the case. It was two separate antagonist organizations (federalist/socialist/center left versus fascist/far right). Both wings had and have a very strong pro-Macedonian or pro-Bulgarian standpoints. We can see a historical development of two streams that are ideologically completely different, but having the same name. Maybe it is good to clear a bit in this article by splitting it in two.

1) IMRO/VMRO is a revolutionary organization for liberation of Macedonia and autonomy within the Ottoman empire (or federation of Balkan states). It became leftist and socialist before WW2 and organized the Macedonian Liberation War against fascist Bulgaria and Germany (it dissolved because Republic of Macedonia became an autonomous part of Yugoslavia, a partial federation of Balkan states). VMRO-DPMNE came out in the stage in the 1990s taking the name of a dissolved organization. Despite its proclamation as a "right" party, VMRO-DPMNE is still center-leftist in its politics of subsidies, populism, etc. For the ethnic Macedonians, the socialism and nationalism went hand in hand. The Macedonian nationalism was historically and is non xenophobic, but cosmopolitan (ethnic Macedonian living jointly with other ethnicities) as written in the Krushevo Maniphesto.

2) BIMRO/BVMRO is a wing of IMRO/VMRO that fought for liberation of Macedonia as part of San Stefano's Bulgaria. It was led by Macedonian intelligentsia in Bulgaria and fought for national Bulgarian interests. This wing became fascist before WW2 and kept its far right position until today. Today's IMRO-SMD or IMRO-BNM are Bulgarian nationalist far right party like the Party of the Swedes or Golden Dawn in Greece. These parties are often considered as xenophobic.

Toci (talk) 21:15, 10 May 2015 (UTC)

First name of the organisation
"After Hristo Tatarchev's "Memoirs" its first name since 1894 was Macedonian Revolutionary Organization (MRO). Ivan Hadzhinikolov in his memoirs underlines the five basic principles of the MRO's foundation ..."

In fact, Tatarchev did not point out that this has been the first name - he wrote why and how a Macedonian revolutionary organization has been established but the name itself is not explicitly indicated in his memories. The only reliable source regarding the initial name is the Statute of the Bulgarian Macedonian-Adrianople Revolutionary Committees, where the organisation is clearly named. The first registered name is therefore BMARC, rather than MRO! —Preceding unsigned comment added by 212.73.146.73 (talk) 23:03, 23 February 2011 (UTC)

Keep calm and read more. Regs! Jingby (talk) 08:13, 24 February 2011 (UTC)

The name of IMRO in Macedonian
Please, provide reference or source that the name of the organisation was written in Macedonian language since it was established in 1893 to its ban in 1934. If no I will remove this later transcription as inappropriate. Thanks. Jingby (talk) 18:34, 16 March 2009 (UTC)

 * You know well that the modern Macedonian language standards were setup later, but it is not a reason for removal of the Macedonian transcription. IMRO was an organization that fought under the slogan "Macedonia for Macedonians" and the organization has importance for both modern Macedonia and Bulgaria, so the name of the organization written in Macedonian language is relevant and appropriate. If we follow your logic, what would be the next step, removing Macedonian transcription in Goce Delcev and Gjorche Petrov articles?MatriX (talk) 19:12, 16 March 2009 (UTC)

"Macedonia for the Macedonians"- that's right but we shouldn't miss the part "all Macedonians regardless nationality and religion". The original IM(A)RO used Bulgarian as only official language in the documentation because the leaders were Macedonian Bulgarians which is shown in the article and could be found in detail in their memoirs. The organisation which could be renamed in accordance to the Macedonian spelling is IMRO-United. The original I. Mac.-Adr. R. O. operated not only on the territory of Macedonia but in Thrace too. Therefore using the Macedonian spelling is quite inaccurate because even not all Macedonians from that time have Macedonian identity today, especially the diaspora in Bulgaria. --Ivo (talk) 21:43, 16 March 2009 (UTC)

 * As I said, the Macedonian naming is appropriate and it cannot be excluded because we are talking here about an organization that was fighting for Macedonian autonomy or independence in the Balkan region and it this organization played a key importance for the ethnic Macedonian struggle for independence and national awakening, therefore I'll reinsert the Macedonian transcription (I'm wondering why it is so irritating to a few Bulgarian editors).MatriX (talk) 13:54, 17 March 2009 (UTC)

This is only your POV. We need scietific proofs. Show them. Jingby (talk) 13:59, 17 March 2009 (UTC)

The Organisation was indisputably fighting for autonomous Macedonia but being classified as important for the ethnic Macedonian struggle is preposterously. Nobody from the leadership of IMARO had ethnic Macedonian identity and the autonomy was seen by many as the first step towards unification with Bulgaria(words of IMARO's founder, dr. Hristo Tatarchev). --Ivo (talk) 22:28, 18 March 2009 (UTC)

 * The organization and its activities inspired and accelerated the movement to build (or strengthen) a separate Macedonian identity and state and from the current perspective it is significant to have the name of the organization written in Macedonian language. MatriX (talk) 09:27, 19 March 2009 (UTC)

Krste Misirkov, a highly controversial writer who alternated between pan-Bulgarian and pan-Macedonian nationalism throughout his lifetime described the IMARO in his pro-Macedonian publication On Macedonian Matters written in the wake of the Ilinden-Preobrazhenie uprising as Bulgarian officials working for Bulgarian interests and linked in name, and in church and school matters, to the people of Bulgaria, their country and their interests. Misirkov wrote:

Jingby (talk) 10:22, 19 March 2009 (UTC)

Bulgarian awakener, publicist and poet Petko Slaveikov in February 1874 reported from Salonika: MatriX (talk) 10:41, 19 March 2009 (UTC) Dimitar Vlahov, another extremely controvercial politician and revolutionary, who also alternated between pan-Bulgarian and pan-Macedonian  nationalism, member of the left wing of the Macedonian-Adrianople revolutionary movement, later  Bulgarian deputy in Ottoman Parliament, afterwards one of the main leaders of IMRO (United) - de facto extension of the Bulgarian Communist Party, but in 1946 elected as vice-president of the Praesidium of Communist Yugoslavia Parliament, wrote in his book "The strugles of Macedonian people for freedom", published in Vienna in 1925:

This article is about Bulgarian organization IMRO. For macedonists, see: Macedonism. You can edit the second article. Jingby (talk) 11:03, 19 March 2009 (UTC)

Please, annonimous IP, i.e. sock of Kubura, stop vandalising the article. Read the source bevore POV-ing your Yugoslav propaganda. It clearly says: ...In addition, the long arc of IMRO’s 40-year evolution—from a Christian national liberation movement in the Ottoman Empire, to a transnational terror network seeking to change state frontiers—offers lessons of wider relevance for understanding terrorist violence and ways to combat it.... I f you do not provide reference about your statements I am going to revert you. Jingby (talk) 16:23, 17 May 2009 (UTC)

I am removing the Macedonian name because it is not in accordance with Wikipedia's rules and principles(objectivity, neutrality etc). The inclusion of Macedonian name implies a certain political view, adopted in former Yugoslavia after WW2, without being accepted by the independent international historiography. IMRO wasn't a multinational Medieval settlement with different names but a political organisation that had various aims and rules. All of them are mentioned in its Statute which is exclusively in Bulgarian- the only language used by the members of the Organisation. Unless a reliable source, indisputably showing that another language was used alongside Bulgarian, is provided, the attempts for 'Macedonisation' of the name remain pure politization of the article. I will keep an eye on the article because my sixth Wikipedian sense whispers me that the chances of being vandalized in the next 60 minutes are fairly high. This time I'm really not going to wage an edit war and my reaction will be to report the situation(in full details). Ivo (talk) 22:20, 3 January 2010 (UTC)

VMRO meant only one thing - Вътрешна македонска революционна организация (ВМРО) Vatreshna makedonska revoliutsionna organizatsia (VMRO)). When you find anthentic documents using the (neo)-Macedonian spelling you will add it. Until then we will stick to history. --Ivo (talk) 07:52, 18 April 2011 (UTC)

Semiprotected
Semiprotected for one month due to edit-warring. The article is being rapidly reverted by IPs with few edits who have never commented on the Talk page. I take note that User:Laveol, though he's an experienced editor, ought to participate on the talk page as well. People seem to be very concerned about which tags should go on the article, but I see no discussion about tags here. Two things usually go together: an article tag plus the explanation (on the talk page) of what problem ought to be fixed. EdJohnston (talk) 02:43, 6 September 2010 (UTC)
 * Ummm, but there is not a number of editors concerned with this, instead just one vandal with a floating IP address who was already blocked for spamming the same tags on at least a hundred articles. Of course when he got blocked he just changed his IP and continued to do the same. his edits and block log. There has not been a line dropped on the talkpage on this and since I know this annon for quite a time, he will not try to write here. I've tried to contact him at least a dozen times before, but he just refuses to take part in any discussion. As for the language: that's the stable version of the article with the names in Bulgarian and Macedonian. It's been here forever and their usage makes sense. The annon (that's why I called him a vandal) just ges around and removes the Bulgarian name. Again, no explanations (not that there could be any). -- L a v e o l  T 09:14, 6 September 2010 (UTC)
 * Great, now we have the attention of a sock-puppet as well. User 8MA8 is a sockpuppet of a single-purpose account User:Micoapostolov. I'm not sure why the case was closed without a resulting block, but it was more than clear. If anyone has any objections to the removal of the tags, please, post them here. If not, I'll consider it as disruptive behaviour (which it actually is since we got two sockpuppeteers involved). -- L a v e o l  T 16:11, 6 September 2010 (UTC)
 * Coming to think more about it, I do suppose that the sockpuppet that was last to edit is actually a sock of the same person that does the annon edits. I'll be filling a checkuser request once I dig up all the clues. -- L a v e o l  T 16:14, 6 September 2010 (UTC)
 * Here's the case. -- L a v e o l  T 16:20, 6 September 2010 (UTC)
 * Ed, is it legit to use check user in this case? The guy editing has already used sockpuppets. I also noticed that the IP and the registered user edit those pages in almost the same time (with only two minutes between them - just the time to log off and load the other page). -- L a v e o l  T 19:05, 6 September 2010 (UTC)

Treaty of Niš (1923)
I think that Treaty of Niš is obviously very important for activities of IMRO and should have its place in the article. I propose to add below sentence after "IMRO began sending armed bands called cheti into Greek and Yugoslav Macedonia and Thrace to assassinate officials and stir up the spirit of the oppressed population.":

Оn March 23, 1923 Aleksandar Stamboliyski signed the Treaty of Niš with the Kingdom of Serbs, Croats and Slovenes and undertook the obligation to suppress the operations of the Internal Macedonian Revolutionary Organization (IMRO) carried out from Bulgarian territory. --Antidiskriminator (talk) 23:10, 13 March 2011 (UTC)

Done. Jingby (talk) 12:16, 14 March 2011 (UTC)

This article needs to be semi-protected
This article needs again semi-protection to prevents edits from unregistered user, as well as edits from accounts that are not autoconfirmed. A sock of a banned who is a spam-tags puser is here again under. Before that he was active here under multiple IP-s and nick-names as   and others and has been trying to insert different tags as a spam. Jingby (talk) 11:02, 20 May 2011 (UTC)

Please, stop reverting the following text: Initially its aim was to gain autonomy for Macedonia and Adrianople regions in Ottoman Empire, but later it became an agent serving Bulgarian interests in Balkan politics. It is taken from Encyclopedia Britannica and objectively reflects the real facts at the time in brief. Thank you. Jingby (talk) 06:24, 8 September 2011 (UTC)

The photo of the statute of the organization says that the organization is Bulgarian and its aim is to revive the Bulgarian self-consciousness of the population in the regions of Macedonia and Adrianople - regions with dominating Bulgarian population at the time. Also, as said in Chapter 2, membership in the organization is open only to Bulgarians. So, it's not a Macedonian organization fighting for Macedonian independence, but rather a Bulgarian one. — Preceding unsigned comment added by Nicksss93 (talk • contribs) 13:50, 8 September 2011 (UTC)

Which from the seven statutes? If I know, despite its pro-Bulgarian character, every one from the Organization's seven statutes aimed authonomy or even independence for Macedonia (Southern Thrace). Wikipedia does not check as reliable primary sources, i.e. Britannica is much more reliable, then one statute. You can look on W:RS. Jingby (talk) 14:00, 8 September 2011 (UTC)

Hristo Tatarchev, one of the founders of the organization, wrote in his memoirs:

"We talked a long time about the goal of this organization and at last we fixed it on autonomy of Macedonia with the priority of the Bulgarian element. We couldn't accept the position for "direct joining to Bulgaria" because we saw that it would meet big difficulties by reason of confrontation of the Great powers and the aspirations of the neighbouring small countries and Turkey. It passed through our thoughts that one autonomous Macedonia could easier unite with Bulgaria subsequently and if the worst comes to the worst, that it could play a role as a unificating link of a federation of Balkan people. The region of Adrianople, as far as I remember, didn't take part in our program, and I think the idea to add it to the autonomous Macedonia came later."

This is another proof that the organization aimed at unification with Bulgaria since the very beginning. Nicksss93 (talk) 16:27, 9 September 2011 (UTC)

This is the personal opinion of one of the founders of IMARO cited in his memoires, but anywhere and in anytime, in any official document, such formulation appeared. Only the autonomy was proclaimed officially as aim of the organization in its statutes, later the independence. Jingby (talk) 13:39, 9 September 2011 (UTC)

So - membership in the organization is open only to Bulgarians, the organization operates in regions with significant Bulgarian population. And these Bulgarians fight for Macedonian independence - does it make sense? Everything in this organization - from its creation to its members, is associated entirely with Bulgaria. And everything Tatarchev says is from the name of all the founders - "We couldn't accept that..", not "I couldn't...", so it's not personal opinion. Nicksss93 (talk) 20:16, 9 September 2011 (UTC)

And now the referenced dangerous sentence you have deleted several times without any reason: Initially its aim was to gain autonomy for Macedonia and Adrianople regions in Ottoman Empire, but later it became an agent serving Bulgarian interests in Balkan politics. Lol! Jingby (talk) 17:33, 9 September 2011 (UTC)
 * The Encyclopedia Britannica is usually accepted as a reliable source. Editors who can locate good work by historians may be able to expand the article to supply a better-balanced picture. EdJohnston (talk) 18:15, 9 September 2011 (UTC)

Statues bar
I suggest that the long vertical sidebar be transformed into a horizontal gallery. They would take less space that way, and most readers of the enwiki can't read Bulgarian anyway, so a clickable thumbnail with English under it should suffice. See for example Siege of Rhodes (1522); I chose this particular example to show that you can have plenty of text under each photo. Have mörser, will travel (talk) 16:15, 19 September 2011 (UTC)

No thanks. 17:47, 19 September 2011 (UTC)

Blatant POV
Please, stop reverting this page and adding incorrecrt info. Neither Macedonian language, nor Macedonian nation existed at the time of the IMRO (1893-1934). Thank you. Jingiby (talk) 11:23, 7 August 2013 (UTC)

Where are the sources regarding "troops being greeted as liberators" in 1941? This is a blatantly biased statement implying a widespread support for the organization in particular, and Bulgarian occupation of parts of Yugoslavia in general. Are there any non-nationalist sources available? In general, sources which are quoted tend to be from a Bulgarian nationalist point of view. If there are no "neutral" points of view (i.e. focused on facts) shouldnt there be other nationalist sources, for the sake of balance? — Preceding unsigned comment added by 87.116.165.85 (talk) 11:40, 17 February 2021 (UTC)

Foreign office record 78/4951
This record is supposedly mentioned in Документи за борбата на македонскиот народ за самостојност и за национална држава (Skopje, 1981) in relation to the naming controversy, but it doesn't seem to exist (see ). It could be that someone copied the wrong record number from the book (Документи за борбата...). If anyone has access to it, it would be nice to verify. Tropcho (talk) 13:19, 18 July 2015 (UTC)

Internal
What does "internal" mean in this case? --YOMAL SIDOROFF-BIARMSKII (talk) 02:11, 10 June 2016 (UTC)

External links modified
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 * Added archive https://web.archive.org/web/20111006202334/http://www.macedoniainfo.com/Independent_Macedonia.htm to http://www.macedoniainfo.com/Independent_Macedonia.htm
 * Added tag to http://www.angelfire.com/super2/vmro-istorija/Knigi/spomht03.htm
 * Added tag to http://library.ferris.edu/~cochranr/stone/stone2c3.htm
 * Added archive https://web.archive.org/web/20040210185155/http://www.vmro-dpmne.org.mk/index.asp to http://www.vmro-dpmne.org.mk/index.asp
 * Added archive https://web.archive.org/web/20061022043326/http://www.makedonskaistorija.com/media/1/20050517-UstavBMORKw.jpg to http://www.makedonskaistorija.com/media/1/20050517-UstavBMORKw.jpg
 * Added archive https://web.archive.org/web/20050210165854/http://www.bulgaria.com/VMRO/bmork.htm to http://www.bulgaria.com/VMRO/bmork.htm

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Bulgarian Organization
It is a Bulgarian organization, why isn't that written in the introduction?

Short description
The first name of the IMRO was "Bulgarian Macedonian-Adrianople Revolutionary Committees", which was later changed several times. Initially its membership was restricted only for Bulgarians. It was active not only in Macedonia but also in Thrace (the Vilayet of Adrianople). Since its early name emphasized the Bulgarian nature of the organisation by linking the inhabitants of Thrace and Macedonia to Bulgaria, these facts are still difficult to be explained from the Macedonian historiography. They suggest that IMRO revolutionaries in the Ottoman period did not differentiate between ‘Macedonians’ and ‘Bulgarians’. Moreover, as their own writings attest, they often saw themselves and their compatriots as ‘Bulgarians’ and wrote in Bulgarian standard language. After the WWI the organization split in Macedonian and Thracian parts. For more see: Brunnbauer, Ulf (2004) Historiography, Myths and the Nation in the Republic of Macedonia. In: Brunnbauer, Ulf, (ed.) (Re)Writing History. Historiography in Southeast Europe after Socialism. Studies on South East Europe, vol. 4. LIT, Münster, pp. 165-200 ISBN 382587365X. Jingiby (talk) 12:17, 2 June 2021 (UTC)

Semi-protected edit request on 6 May 2022
The official language was Macedonian. 46.164.24.198 (talk) 12:43, 6 May 2022 (UTC)
 * Red information icon with gradient background.svg Not done: please provide reliable sources that support the change you want to be made. ScottishFinnishRadish (talk) 12:54, 6 May 2022 (UTC)

Semi-protected edit request on 31 July 2022
Wrong flag colours and Macedonia was also a large speaking language within the IMRO members 92.53.35.232 (talk) 18:43, 31 July 2022 (UTC)
 * Red question icon with gradient background.svg Not done: it's not clear what changes you want to be made. Please mention the specific changes in a "change X to Y" format and provide a reliable source if appropriate. ScottishFinnishRadish (talk) 18:50, 31 July 2022 (UTC)


text:
Paul Forseth

Paul Eugene Forseth (born December 10, 1946) is a Canadian politician, who was a member of Parliament for British Columbia from 1993 to 2006.

He was first elected as the Member of Parliament for New Westminster—Burnaby in the 1993 federal election, as a member of the Reform Party of Canada, defeating New Democrat member of Parliament (MP) Dawn Black. He won reelection in 1997 as a Reform Party Member, in 2000 as a member of the Canadian Alliance and in 2004 as a member of the Conservative Party, until Black defeated him in the 2006 election.

During his 12 years of elected office, he was not part of the government, but served from the 'Opposition Benches'.

In the Reform Party, he was instrumental to sponsor major planks of the Reform Party platform published in the Reform "Blue Sheet" concerning 'Justice' and 'Environment' policy, and led House of Commons debates on those subjects.

During his Canadian Alliance tenure, he was part of the 'shadow cabinet' under Stockwell Day, leader of the Official Opposition.

During the Conservative Party term, he was his Party's lead representative on the Government Operations and Estimates Committee, which investigated the administrative record of Privacy Commissioner George Radwanski. Forseth co-chaired the sub-committee which held in-camera investigative hearings. Radwanski was accused of falsifying documents and other administrative excesses by the Auditor General, (Ottawa Citizen Oct. 01, 2003) After Parliamentary hearings, the G.O.E.Committee tabled a report in the House of Commons. (37th PARLIAMENT, 2nd SESSION HANSARD • NUMBER 150 Tuesday, November 4, 2003) As a consequence, the House of Commons declared George Radwanski to be in contempt of Parliament. (37th PARLIAMENT, 2nd SESSION HANSARD • NUMBER 152 Thursday, November 6, 2003)  “That this House find George Radwanski to have been in contempt of this House, and acknowledge receipt of his letter of apology, tabled and read to the House earlier today.”

Paul Forseth was also among the very few MPs in Parliamentary history to ever have his Private Members Bill, coming from the Opposition side of the House, cooperatively taken over by the government and incorporated into a larger piece Government Legislation. (Bankruptcy and Insolvency Act - discharge list). (Hansard Tuesday, October 22, 1996)

As of September 2009, Forseth is again a Conservative candidate. He said “I have been nominated as the Conservative Party candidate in the district of Burnaby-New Westminster. I am offering myself for service, as our community needs a Conservative voice at the national level.” (source - paulforseth - blogspot)

May 2, 2011, there was the national federal election, and Prime Minister Stephen Harper consolidated his government standing by achieving a majority by winning 167 out of 308 seats in the House of Commons. Paul Forseth was a candidate in the election but was not successful to unseat the NDP incumbent Peter Julian who has held the district of Burnaby-New Westminster since 2004. Mr. Forseth came in second place with the Liberal candidate Garth Evans achieving third place.

Forseth was the BC-Conservative Candidate (Provincial) for the local district of New Westminster. Voting Day was May 14, 2013, and he was unsuccessful.

He is retired and lives in Powell River, British Columbia.

title: Paul Forseth


text:
User talk:Pnorman

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text:
Sachertorte

Sachertorte is a chocolate cake, or torte, of Austrian origin,  invented by Franz Sacher, supposedly in 1832 for Prince Metternich in Vienna. It is one of the most famous Viennese culinary specialties.

History
According to Franz Sacher's son Eduard, Sachertorte was invented by Sacher when he was an apprentice under Metternich's chef Chambellier. The story goes that Sacher was required to create a novel cake when the chef was ill. The Metternich connection was probably invented by Eduard many years later, to appeal to "Viennese nostalgia for their imperial past".

Sachertorte remains popular in Austria and worldwide.

Composition
The cake consists of a dense chocolate cake with a thin layer of apricot jam in between two halves, coated in dark chocolate icing on the top and sides. It is traditionally served with unsweetened whipped cream.

Variations
The "Original" Sacher Torte has two layers of apricot jam between the outer layer of chocolate icing and the sponge base, while Demel's "Eduard-Sacher-Torte" has only one. Demel's cake is denser and smoother.

Some of the various recipes for cakes similar to the "Original" are listed below. For example, at "Graz-Kulturhauptstadt 2003", a festival marking the city of Graz being declared cultural capital that year, "Wanda Sacher-Masoch-Torte" was presented (its name alluding to Wanda von Sacher-Masoch, the wife of Leopold von Sacher-Masoch), using redcurrant jam and marzipan.

Production and sale of the "Original Sacher-Torte"
Hotel Sacher's "Original Sacher Torte" is sold at the Vienna and Salzburg locations of the Hotel Sacher, at Cafe Sacher branches in Innsbruck and Graz, at the Sacher Shop in Bolzano, in the Duty Free area of Vienna airport, and via the Hotel Sacher's online shop.

The recipe of the Hotel Sacher's version of the cake is a closely guarded secret. Those privy to it claim that the secret to the Sacher Torte's desirability lies not in the ingredients of the cake itself, but rather those of the chocolate icing. According to widely available information, the icing consists of three special types of chocolate, which are produced exclusively by different manufacturers for this sole purpose. The hotel obtains these products from Lübeck in Germany and from Belgium.

The Hotel Sacher has gone to great lengths to distinguish the Original Sacher Torte from other variations. This includes four golden corners on the wooden box, the wood engraving of the Hotel Sacher Wien as well as "Das Original" and "Hotel Sacher Wien" in writing in the inside of the lid, and bordeaux red wrapping paper with a Biedermeier motif.

Legal dispute with Demel's
In 1934, the Demel pastry shop started selling "Eduard Sacher-Torte", while the Sacher Hotel sold the "Original Sacher-Torte". The hotel's owners sued Demel for trademark infringement, and won in 1938. The lawsuit was appealed after the war, and the hotel was eventually given the exclusive right to call its version "the original".

title: Sachertorte