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35-437: The webbed-fingered Karl Stromberg is a successful businessman as head of his own shipping firm and chain of laboratories. Stromberg's obsession and passion is the ocean where he lives in his palace, named Atlantis, that could submerge itself underwater so as not to be seen or detected. Located off the coast of Sardinia , Italy, Atlantis has everything to support life above and below water for any length of time. In fact, Atlantis

70-432: A Soviet nuclear submarine and a British submarine. Stromberg's specially adapted tanker, Liparus , sneaks up on the subs, forces them to the surface through methods using some kind of high frequencies to disrupt the sub's electrical system and captures them inside the tanker. His plan calls for the firing of nuclear missiles from these subs at Moscow and New York City , thus framing each other's government and starting

105-575: A comic relief after cancelling the payment to the professor and doctor he has his secretary inform the two men's families that they have met with an accident and are "buried at sea".) This scheme is similar to that of an earlier Bond film, You Only Live Twice , which posited stealing space capsules to start a war between the Soviets and the Americans. The idea of commandeering two nuclear missiles and attempting to fire them at two major cities likewise recalls

140-433: A higher incidence of about 1 in 65,000 live births. The cranial malformations are the most apparent effects of acrocephalosyndactyly. Craniosynostosis occurs, in which the cranial sutures close too soon, though the child's brain is still growing and expanding. Brachycephaly is the common pattern of growth, where the coronal sutures close prematurely, preventing the skull from expanding frontward or backward and causing

175-460: A nuclear war, which would wipe out every last human being on Earth. Among Stromberg's many minions were an assistant who betrayed him by trying to sell the plans for the submarine tracking system, and a professor and a doctor who were to help him operate the tracking system after it was complete. He killed his assistant by feeding her to a shark, and killed the professor and the doctor, having outlived their usefulness, by blowing up their helicopter. (In

210-445: A protein named sonic hedgehog , also known as SHH, which dissolves the tissue between the fingers and toes, and the webbing disappears. In some fetuses, this process does not occur completely between all fingers or toes and some residual webbing remains. Five types of syndactyly have been identified in humans. The corresponding loci associated with these types and their common phenotypical expression are as follows: Syndactyly of

245-413: A typical characteristic of branchial arch syndromes. All acrocephalosyndactyly syndromes show some level of limb anomalies, so it can be hard to tell them apart. However, the typical hand deformities in patients with Apert syndrome distinguish it from the other syndromes. The hands in patients with Apert syndrome always show four common features: The deformity of the space between the index finger and

280-526: Is a birth defect in which an intestine or other abdominal organs are outside of the body of an infant because of a hole in the bellybutton area. However, the association between omphalocele and Apert syndrome is not confirmed yet, so additional studies are necessary. Acrocephalosyndactyly may be an autosomal dominant disorder. Males and females are affected equally; however research is yet to determine an exact cause. Nonetheless, almost all cases are sporadic, signifying fresh mutations or environmental insult to

315-420: Is from Ancient Greek σύν (syn)  'together' and δάκτυλος (daktulos)  'finger'. Syndactyly can be simple or complex. Syndactyly can be complete or incomplete. Complex syndactyly occurs as part of a syndrome (such as Apert syndrome ) and typically involves more digits than simple syndactyly. Fenestrated syndactyly, also known as acrosyndactyly or terminal syndactyly, means

350-430: Is more like a city, able to support dozens if not hundreds of people. Stromberg also owns a huge tanker , named Liparus , that serves as his headquarters away from Atlantis. Aboard the tanker he has a small army of red-liveried soldiers. In Christopher Wood's novelisation of the film, Stromberg is Swedish and his first name is "Sigmund". Although Stromberg has a passion for the ocean and its various species , he despises

385-415: Is that the grafts darken in the years after surgery and become more noticeable. Also, if the skin grafts are harvested from the groin area, the skin may grow hair. Finally, the fingers may deviate after surgery. This is most commonly seen in complex syndactyly (when there has been a bone joining of the fingers). The earliest appreciation of syndactyly as a birth anomaly or burn-trauma can be traced back to

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420-485: Is typically by the apparent physical characteristics and can be aided by skull X-ray or head CT examination . Molecular genetic testing can confirm the diagnosis. Surgery is needed to prevent the closing of the coronal sutures from damaging brain development. In particular, surgeries for the LeFort III or monobloc midface distraction osteogenesis which detaches the midface or the entire upper face, respectively, from

455-505: The Andalusian Muslim surgeon Al-Zahrawi (d. 1013 CE), known in the West as Abulcasis. The French barber surgeon Ambroise Paré also described syndactyly in the sixteenth century. [REDACTED] Media related to Syndactyly at Wikimedia Commons Apert syndrome Apert syndrome is a form of acrocephalosyndactyly , a congenital disorder characterized by malformations of

490-409: The genome . The offspring of a parent with Apert syndrome has a 50% chance of inheriting the condition. In 1995, A.O.M. Wilkie published a paper showing evidence that acrocephalosyndactyly is caused by a defect on the fibroblast growth factor receptor 2 gene , on chromosome 10 . Apert syndrome is an autosomal dominant disorder; approximately two-thirds of the cases are due to a C to G mutation at

525-526: The border digits (thumb/index finger or ring/small fingers) is treated at early age to prevent the larger digit from curving towards the smaller digit with growth. Typically, syndactyly of these digits is treated at six months of age. The treatment of syndactyly of the other digits is elective and is more commonly performed when the digits have grown, at 18 months of age. Special situations, such as complex syndactyly and involvement of border digits, may warrant surgical intervention earlier than 6 months. Because

560-556: The brain to expand the skull to the sides and upwards. This results in another common characteristic, a high, prominent forehead with a flat back of the skull. Due to the premature closing of the coronal sutures, increased cranial pressure can develop, leading to mental deficiency. A flat or concave face may develop as a result of deficient growth in the mid-facial bones, leading to a condition known as pseudomandibular prognathism. Other features of acrocephalosyndactyly may include shallow bony orbits and broadly spaced eyes. Low-set ears are also

595-424: The case of acrocephalosyndactyly, selective cell death does not occur and skin, and rarely bone, between the fingers and toes fuses. The cranial bones are affected as well, similar to Crouzon syndrome and Pfeiffer syndrome . Craniosynostosis occurs when the fetal skull and facial bones fuse too soon in utero , disrupting normal bone growth. Fusion of different sutures leads to different patterns of growth on

630-400: The circumference of the conjoined fingers is smaller than the circumference of the two separated fingers, there is not enough skin to cover both digits once they are separated at the time of surgery. Therefore, the surgeon must bring new skin into the area at the time of surgery. This is most commonly done with a skin graft (from groin or anterior elbow). Skin can also be used from the back of

665-507: The crews from the submarines escape and take over the tanker. With the tanker under their control, Bond is able to order the stolen submarines, which were crewed by Stromberg's men, to fire their nuclear warheads at each other. Prior to this, however, Stromberg had abducted Bond's partner, the Russian agent Anya Amasova , and escaped to his city-ship, Atlantis. Bond pursues Stromberg, and after two failed attempts by Stromberg to kill him (including

700-426: The expression of three isoforms of FGFR2, the gene with the point mutations that causes the syndrome in 98% of the patients. KGFR, keratinocyte growth factor receptor, is an isoform active in the metaphysis and interphalangeal joints. FGFR1 is an isoform active in the diaphysis . FGFR2-Bek is active in the metaphysis, as well as the diaphysis, but also in the interdigital mesenchyme . The point mutation increases

735-412: The hand by mobilizing it (called a "graftless" syndactyly correction), which requires planning over a period of months prior to surgery. The most common problem with syndactyly correction is creeping of the skin towards the fingertip over time. This is likely due to tension at the site of the repair between the digits. Additional surgery may be required to correct this. One critique of using skin grafts

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770-524: The human race, not unlike Jules Verne 's Captain Nemo . Stromberg, however, has no interest in benefiting the world. He has a congenital condition in which his hands are webbed like those of aquatic birds or mammals. It is his personal mission to start over with a new civilisation underwater via complete anarchy against the "surface world". After contracting two scientists to create the technology to track nuclear submarines , Stromberg uses this technology to capture

805-508: The ligand-dependent activation of FGFR2 and thus of its isoforms. This means that FGFR2 loses its specificity, causing binding of FGFs that normally do not bind to the receptor. Since FGF suppresses apoptosis , the interdigital mesenchyme is maintained. FGF also increases replication and differentiation of osteoblasts , thus early fusion of several sutures of the skull. This may explain why both symptoms are always found in Apert syndrome. Diagnosis

840-544: The plot of Thunderball . The scheme in which the villain wishes to destroy mankind to create a new race or new civilisation was also used in Moonraker , the next film after The Spy Who Loved Me . The film Moonraker was also written by Christopher Wood and featured Jaws as a henchman. Stromberg's scheme is foiled after Bond is taken aboard the Liparus as a prisoner from a recently captured American submarine. With Bond's help,

875-549: The population have varied, with estimates as low as 1 birth in 200,000 provided and 160,000 given as an average by older studies. A study conducted in 1997, however, by the California Birth Defects Monitoring Program found an incidence rate of 1 in 80,645 out of almost 2.5 million live births. Another study conducted in 2002 by the Craniofacial Center, North Texas Hospital for Children, found

910-446: The position 755 in the FGFR2 gene, which causes a Ser to Trp change in the protein. This is a male-specific mutation hotspot: in a study of 57 cases, the mutation always occurred on the paternally derived allele. On the basis of the observed birth prevalence of the disease (1 in 70,000), the apparent rate of C to G mutations at this site is about .00005, which is 200- to 800-fold higher than

945-550: The rest of the skull, are performed in order to reposition them in the correct plane. These surgeries are performed by both plastic and oral and maxillofacial (OMS) surgeons, often in collaboration. There is no standard treatment for the hand malformations in Apert due to the differences and severity in clinical manifestations in different patients. Every patient should therefore be individually approached and treated, aiming at an adequate balance between hand functionality and aesthetics. However, some guidelines can be given depending on

980-432: The severity of the deformities. In general it is initially recommended to release the first and fourth interdigital spaces, thus releasing the border rays. This makes it possible for the child to grasp things by hand, a very important function for the child's development. Later the second and third interdigital spaces have to be released. Because there are three handtypes in Apert, all with their own deformities, they all need

1015-411: The skin is joined for most of the digit but in a proximal area there are gaps in the syndactyly with normal skin. This type of syndactyly is found in amniotic band syndrome . Simple syndactyly can be full or partial, and is present at birth (congenital). In early human fetal development, webbing (syndactyly) of the toes and fingers is normal. At about 6 weeks of gestation, apoptosis takes place due to

1050-433: The skull, face, hands and feet. It is classified as a branchial arch syndrome, affecting the first branchial (or pharyngeal) arch , the precursor of the maxilla and mandible . Disturbances in the development of the branchial arches in fetal development create lasting and widespread effects. In 1906, Eugène Apert , a French physician, described nine people sharing similar attributes and characteristics. Linguistically, in

1085-414: The skull. Examples include: trigonocephaly (fusion of the metopic suture ), brachycephaly (fusion of the coronal suture and lambdoid suture bilaterally), dolichocephaly (fusion of the sagittal suture ), plagiocephaly (fusion of coronal and lambdoidal sutures unilaterally) and oxycephaly or turricephaly (fusion of coronal and lambdoid sutures). Findings for the incidence of the syndrome in

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1120-410: The term "acrocephalosyndactyly", acro is Greek for "peak", referring to the "peaked" head that is common in the syndrome; cephalo , also from Greek, is a combining form meaning "head"; syndactyly refers to webbing of fingers and toes. In embryology , the hands and feet have selective cells that die in a process called selective cell death, or apoptosis , causing separation of the digits. In

1155-475: The thumb may be variable. Based on this first webspace, three different types of hand deformation can be diffentiated: Common relevant features of acrocephalosyndactyly are a high-arched palate, pseudomandibular prognathism (appearing as mandibular prognathism ), a narrow palate and crowding of the teeth. Omphalocele has been described in two patients with Apert syndrome by Herman T.E. et al. (USA, 2010) and by Ercoli G. et al. (Argentina, 2014). An omphalocele

1190-431: The use of an explosive harpoon which ran the length of a dinner table), Bond exercises his licence to kill by shooting Stromberg multiple times in the groin and chest. Atlantis is later torpedoed and sunk, giving Stromberg a burial at sea. Syndactyly Syndactyly is a condition wherein two or more digits are fused together. It occurs normally in some mammals , but is an unusual condition in humans. The term

1225-536: The usual rate for mutations at CG dinucleotides. Moreover, the incidence rises sharply with the age of the father . Goriely et al. (2003) analyzed the allelic distribution of mutations in sperm samples from men of different ages and concluded that the simplest explanation for the data is that the C to G mutation gives the cell an advantage in the male germline. It is still not very clear why people with Apert syndrome have both craniosynostosis and syndactyly. There has been one study that suggests it has something to do with

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