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Ectrodactyly

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857:(687 adults and 1259 larvae) were captured from a large wetland complex and evaluated for malformations. Among the 687 adults, 54 (7.9%) were malformed. Of these 54 adults, 46 (85%) had missing (ectrodactyly), extra (polyphalangy) or dwarfed digits (brachydactyly). Among the 1259 larvae, 102 were malformed, with 94 (92%) of the malformations involving ectrodactyly, polyphalangy, and brachydactyly. Results showed few differences in the frequency of malformations among life-history changes, suggesting that malformed larvae do not have substantially higher mortality than their adult conspecifics. 332:). The exact chromosomal defect in isolated cleft hand is not yet defined. However, the genetic causes of cleft hand related to syndromes have more clarity. The identified mutation for SHSF syndrome (split-hand/split-foot syndrome) a duplication on 10q24, and not a mutation of the tp63 gene as in families affected by EEC syndrome (ectrodactyly–ectodermal dysplasia–cleft syndrome). The p63 gene plays a critical role in the development of the apical ectodermal ridge (AER), this was found in mutant mice with dactylaplasia. 78: 140:
cleft hand appears as a V-shaped cleft situated in the centre of the hand. The digits at the borders of the cleft might be syndactilyzed, and one or more digits can be absent. In most types, the thumb, ring finger and little finger are the less affected parts of the hand. The incidence of cleft hand varies from 1 in 90,000 to 1 in 10,000 births depending on the used classification. Cleft hand can appear unilateral or bilateral, and can appear isolated or associated with a syndrome.
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between these morphogens, the involvement of modifier genes, and the presumed involvement of multiple gene or long-range regulatory elements in some cases of ectrodactyly. In the clinical setting these genetic characteristics can become problematic and making predictions of carrier status and severity of the disease impossible to predict.
148:(SHFM1 at 7q21-q22), SHFM2 (Xq26), SHFM3 (FBXW4/DACTYLIN at 10q24), SHFM4 (TP63 at 3q27), and SHFM5 (DLX1 and DLX 2 at 2q31). SHFM3 is unique in that it is caused by submicroscopic tandem chromosome duplications of FBXW4/DACTYLIN. SHFM3 is considered 'isolated' ectrodactyly and does not show a mutation of the tp63 gene. 642:
in 1575. Hartsink (1770) wrote the first report of true cleft hand. In 1896, the first operation of the cleft hand was performed by Doctor Charles N. Dowed of New York City. However, the first certain description of what we know as a cleft hand as we know it today was described at the end of the 19th
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Ueba described a less complicated surgery. Transverse flaps are used to resurface the palm, the dorsal side of the transposed digit and the ulnar part of the first web space. A tendon graft is used to connect the common extensor tendons of the border digits of the cleft to prevent digital separation
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The goal of this procedure is to create a wide first web space and to minimise the cleft in the hand. The index digit will be transferred to the ulnar side of the cleft. Simultaneously a correction of index malrotation and deviation is performed. To minimise the cleft, it is necessary to fix together
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The social and stigmatising aspects of a cleft hand require more attention. The hand is a part of the body which is usually shown during communication. When this hand is obviously different and deformed, stigmatisation or rejection can occur. Sometimes, in families with cleft hand with good function,
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in which the development of the hand is disturbed. It is a type I failure of formation – longitudinal arrest. The central ray of the hand is affected and usually appears without proximal deficiencies of nerves, vessels, tendons, muscles and bones in contrast to the radial and ulnar deficiencies. The
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The Department of Biological Sciences at the University of Alberta in Edmonton, Alberta performed a study to estimate deformity levels in wood frogs in areas of relatively low disturbance. After roughly 22,733 individuals were examined during field studies, it was found that only 49 wood frogs had
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Ectrodactyly is not only a genetic characteristic in humans, but can also occur in frogs and toads, mice, salamanders, cows, chickens, rabbits, marmosets, cats and dogs, and even West Indian manatees. The following examples are studies showing the natural occurrence of ectrodactyly in animals,
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When surgery is indicated, the choice of treatment is based on the classification. Table 4 shows the treatment of cleft hand divided into the classification of Manske and Halikis. Techniques described by Ueba, Miura and Komada and the procedure of Snow-Littler are guidelines; since clinical and
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The timing of surgical interventions is debatable. Parents have to decide about their child in a very vulnerable time of their parenthood. Indications for early treatment are progressive deformities, such as syndactyly between index and thumb or transverse bones between the digital rays. Other
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A number of factors make the identification of the genetic defects underlying human ectrodactyly a complicated process: the limited number of families linked to each split hand/foot malformation (SHFM) locus, the large number of morphogens involved in limb development, the complex interactions
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Ectrodactyly can be caused by various changes to 7q. When 7q is altered by a deletion or a translocation, ectrodactyly can sometimes be associated with hearing loss. Ectrodactyly, or Split hand/split foot malformation (SHFM) type 1 is the only form of split hand/ malformation associated with
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Split hand/foot malformation (SHFM) is characterized by underdeveloped or absent central digital rays, clefts of hands and feet, and variable syndactyly of the remaining digits. SHFM is a heterogeneous condition caused by abnormalities at one of multiple loci, including
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Historically, a U-type cleft hand was also known as atypical cleft hand. The classification in which typical and atypical cleft hand are described was mostly used for clinical aspects and is shown in table 1. Nowadays, this "atypical cleft hand" is referred to as
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Ectrodactyly is frequently seen with other congenital anomalies. Syndromes in which ectrodactyly is associated with other abnormalities can occur when two or more genes are affected by a chromosomal rearrangement. Disorders associated with ectrodactyly include
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There are several classifications for cleft hand, but the most used classification is described by Manske and Halikis see table 3. This classification is based on the first web space. The first web space is the space between the thumb and the index finger.
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Davis and Barry 1977 tested allele frequencies in domestic cats. Among the 265 cats observed, there were 101 males and 164 females. Only one cat was recorded to have the ectrodactyly abnormality, illustrating this rare disease.
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Eaton, Brian R.; Eaves, Sara; Stevens, Cameron; Puchniak, Allison; Paszkowski, Cynthia A.; et al. (2004). "Deformity Levels in Wild Populations of the Wood Frog (Rana sylvatica) in Three Ecoregions of Western Canada".
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Some studies have postulated that polydactyly, syndactyly and cleft hand have the same teratogenic mechanism. In vivo tests showed that limb anomalies were found alone or in combination with cleft hand when they were given
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and X-linked forms occur more rarely. Ectrodactyly can also be caused by a duplication on 10q24. Detailed studies of a number of mouse models for ectrodactyly have also revealed that a failure to maintain median
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Naruse, Takuji; Takahara, Masatoshi; Takagi, Michiaki; Ogino, Toshihiko (2007). "Early Morphological Changes Leading to Central Polydactyly, Syndactyly, and Central Deficiencies: An Experimental Study in Rats".
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dog. In another study, Carrig and co-workers also reported a series of 14 dogs with this abnormality proving that although ectrodactyly is an uncommon occurrence for dogs, it is not entirely unheard of.
128:). The hands and feet of people with ectrodactyly (ectrodactyls) are often described as "claw-like" and may include only the thumb and one finger (usually either the little finger, ring finger, or a 1342:
Shamseldin, Hanan E; Faden Maha A; Alashram Walid; Alkuraya Fowzan S (Nov 2011). "Identification of a novel DLX5 mutation in a family with autosomal recessive split hand and foot malformation".
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The release of the first webspace has the same principle as the Snow-Littler procedure. The difference is the closure of the first webspace; this is done by simple closure or closure with
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Carrig, ColinB.; Wortman, JeffreyA.; Morris, EarlL.; Blevins, WilliamE.; Root, CharlesR.; Hanlon, GriseldaF.; Suter, PeterF. (1981). "Ectrodactyly (Split-hand deformity) in the dog".
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Durowaye, Mathew; Adegboye, Abdulrasheed; Mokuolu, Olugbenga Ayodeji; Adeboye, Muhammed; Yahaya-Kongoila, Sefiyah; Adaje, Adeline; Adesiyun, Omotayo; Ernest, Samuel Kolade (2011).
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and cleft hand can occur within the same hand, therefore some investigators suggest that these entities occur from the same mechanism. This mechanism is not yet defined.
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Moerman, P.; Fryns, J.P. (1998). "Ectodermal dysplasia, Rapp–Hodgkin type in a mother and severe ectrodactyly-ectodermal dysplasia-clefting syndrome (EEC) in her child".
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of the condition. The function of a cleft hand is mostly not restricted, yet improving the function is one of the goals when the thumb or first webspace is absent.
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Kay, Simon P.; McCombe, David (2005). "Central hand deficiencies". In Green, David P.; Hotchkiss, Robert N.; Pederson, William C.; et al. (eds.).
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without the disease being reproduced and tested in a laboratory. In all three examples we see how rare the actual occurrence of ectrodactyly is.
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Manson, Jeanne; Dourson, Michael L.; Smith, Carl C. (1977). "Effects of Cytosine Arabinoside on In vivo and In vitro Mouse limb Development".
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Scherer, Stephen; Cheung, J; MacDonald, JR; Osborne, LR; Nakabayashi, K; Herbrick, JA; Carson, AR; Parker-Katiraee, L; et al. (2003).
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Suppression progresses in a more ulnar direction; therefore in the monodactylous form the thumb is usually the last remaining digit
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Atypical hand had a more severe manifestation in which there was varying absence of the central index, middle and ring finger rays
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The pathophysiology of cleft hand is thought to be a result of a wedge-shaped defect of the apical ectoderm of the limb bud (AER:
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during extension. The closure is simpler, but has cosmetic disadvantage because of the switch between palmar and dorsal skin.
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There is no cleft or web space and the thumb is very deficient. This hand requires consideration of creating a radial digit
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Manske, Paul R.; Halikis, Mark N. (1995). "Surgical classification of central deficiency according to the thumb web".
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as an inspirational character who battles her disease and counsels another family who have children with ectrodactyly
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In a study performed by the Department of Forestry and Natural Resources at Purdue University, approximately 2000
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Suppression progresses in a radial direction so that in the monodactylous form the most ulnar finger is preserved
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there is a man whose hands are crescent-shaped, and have only two fingers each, and his feet similarly formed.
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anatomical presentation within the types differ, the actual treatment is based on the individual abnormality.
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Peterson-Falzone, Sally J.; Hardin-Jones, Mary A.; Karnell, Michael P.; McWilliams, Betty Jane (2001).
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operations for cosmetic aspects are considered marginal and the families choose not to have surgery.
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The cause of cleft hand lies, for what is known, partly in genetics. The inheritance of cleft hand is
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Falliner AA., Analysis of anatomic variations in cleft hands, J Hand Surg Am. 2004 Nov;29(6):994-1001
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A large number of human gene defects can cause ectrodactyly. The most common mode of inheritance is
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Manske, Paul R.; Goldfarb, Charles A. (2009). "Congenital Failure of Formation of the Upper Limb".
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Wieland, I.; Muschke, P; Jakubiczka, S; Volleth, M; Freigang, B; Wieacker, PF; et al. (2004).
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Naruse, Takuji; Takahara, Masatoshi; Takagi, Michiaki; Oberg, Kerby C.; Ogino, Toshihiko (2007).
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Once a popular television anchor woman in Los Angeles, she has appeared in the television drama
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Constitutional Disorders of the Skeleton in Dogs and Cats: Textbook of Small Animal Orthopedics
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Transverse bones (this will progress the deformity; growth of these bones will widen the cleft)
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According to M.P. Ferreira, a case of ectrodactyly was found in a two-month-old male mixed
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Davis, Brian; Davis, Barry (1977). "Allele frequencies in a cat population in Budapest".
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Ferreira, M.P.; Alievi, M.M.; Beck, C.A.C.; Voll, J.; Muccillo, M.S.; Gomes, C. (2007).
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The treatment of cleft hand is usually invasive and can differ each time because of the
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which used to border the cleft. Through repositioning flaps, the wound can be closed.
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Typical hand was manifest in the complete or incomplete absence of the middle finger
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Deforming syndactyly (mostly between digits of unequal length like index and thumb)
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This article is about the congenital disorder. For the neurological syndrome, see
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Upton, Joseph; Taghinia, Amir H. (2010). "Correction of the Typical Cleft Hand".
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Mobility and/or position of the thumb of ulnar digit to promote pinch and grasp
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Transposition of the index metacarpal with reconstruction of the thumb webspace
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Thumb elements suppressed, ulnar rays remain, thumb web space no longer present
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Farrell HB (1984). "The two-toed Wadoma--familial ectrodactyly in Zimbabwe".
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10.1002/(SICI)1096-8628(19960614)63:3<479::AID-AJMG12>3.0.CO;2-J
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Williams, Rod N; Bos, David H; Gopurenko, David; DeWoody, J Andrew (2008).
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Jones, Neil F; Kono, Michiyuki (2004). "Cleft hands with six metacarpals".
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The first modern reference to what might be considered a cleft hand was by
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and Limb-mammary (LMS) syndrome, Ectrodactyly-Cleft Palate (ECP) syndrome,
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Table 3: Classification for cleft hand described by Manske and Halikis
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Ectrodactyly in all extremities; only eight total digits present, 1870
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surgical interventions are less urgent and can wait for 1 or 2 years.
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Surgical treatment of the cleft hand is based on several indications:
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Table 4: Treatment based on the classification of Manske and Halikis
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of 70%. Cleft hand can be a spontaneous mutation during pregnancy (
1787: 938:"Familial Ectrodactyly Syndrome in a Nigerian Child: A Case Report" 723: 613: 605: 145: 1914:. Ithaca, New York: International Veterinary Information Service. 2340: 2118: 2060: 2051: 622:
Literature shows that cleft hand is described centuries ago. In
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Index ray suppressed, thumb web space is merged with the cleft
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Ectrodactyly-Ectodermal Dysplasia-Macular Dystrophy syndrome
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showing his right profile and right hand with ectrodactyly
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Thumb and index rays syndactylized, web space obliterated
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Monodactyly of both hands; only two fingers present, 1897
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Ectrodactyly-Ectodermal Dysplasia-Clefting (EEC) syndrome
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Malformation of the central digit(s) of the hand or foot
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Arquivo Brasileiro de Medicina VeterinĂĄria e Zootecnia
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Reconstruction of the transverse metacarpal ligament
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of the two) with similar abnormalities of the feet.
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(August 1987). 988:American Journal of Medical Genetics Part A 2769: 2599: 2590: 2451: 2299: 2221: 2212: 2175: 2161: 2153: 2066: 76: 48: 39: 1989: 1979: 1886: 1594: 1592: 1543: 1541: 1539: 1537: 1535: 1533: 1531: 1511: 1500:Development, Growth & Differentiation 1412: 1318: 1259: 1197: 1165: 1163: 961: 120:of the hand or foot and is also known as 1863:"Amphibian malformations and inbreeding" 1766: 1764: 1762: 1681: 1679: 1235: 1233: 1231: 1229: 1227: 1225: 1223: 1221: 1219: 1217: 164: 901: 657:Atypical cleft hand (symbrachydactyly) 223:Ectrodactyly/mandibulofacial dysostosis 1964:"Ectrodactilia em cĂŁo: relato de caso" 1102: 1100: 1098: 1096: 1094: 1092: 1090: 1088: 1086: 1084: 216:Ectrodactyly and cleft palate syndrome 1941:10.1093/oxfordjournals.jhered.a108768 1554:The Journal of Bone and Joint Surgery 1082: 1080: 1078: 1076: 1074: 1072: 1070: 1068: 1066: 1064: 909:Giele, Henk; Cassell, Oliver (2008). 276:, which is closely correlated to the 181:Split-Hand-Foot Malformation Syndrome 7: 2696:Greig cephalopolysyndactyly syndrome 716:and is not a subtype of cleft hand. 2057:Online Mendelian Inheritance in Man 2048:Online Mendelian Inheritance in Man 1387:"The London Dysmorphology Database" 626:(426 A.D.), St. Augustine remarks: 135:It is a substantial rare form of a 61:on the hand of a one-year-old child 2186:malformations and deformations of 2019:10.1111/j.1740-8261.1981.tb01363.x 1240:Pascal, H.G.; et al. (2003). 912:Plastic and Reconstructive Surgery 411:Thumb web space severely narrowed 268:was found to be involved in SHFM. 230:Ectrodactyly and macular dystrophy 122:split hand/split foot malformation 25: 2035: 1672:City of God, Book XVI, Chapter 8 1566:10.2106/00004623-196446080-00006 1513:10.1111/j.1440-169X.2007.00949.x 503:Timing of surgical interventions 400:Thumb web space mildly narrowed 109: 'miscarriage' and 2369:Congenital patellar dislocation 2248:Wallis–Zieff–Goldblatt syndrome 1981:10.1590/S0102-09352007000400015 1722:"Medical Mystery: Ectrodactyly" 248:with reduced penetrance, while 1109:Green's Operative Hand Surgery 1: 1613:10.1016/S0363-5023(05)80293-X 1443:Katarincic, Julia A. (2003). 1356:10.1136/jmedgenet-2011-100556 389:Thumb web space not narrowed 264:In 2011, a novel mutation in 845:the ectrodactyly deformity. 681:One limb involved (no feet) 512:Classification and treatment 157:sensorineural hearing loss. 2655:Oto-palato-digital syndrome 2650:Hallermann–Streiff syndrome 2374:Congenital knee dislocation 2192:musculoskeletal abnormality 1688:The Journal of Hand Surgery 1601:The Journal of Hand Surgery 1461:The Journal of Hand Surgery 1392:Journal of Medical Genetics 1344:Journal of Medical Genetics 1177:Journal of Medical Genetics 1134:The Journal of Hand Surgery 1052:Congenital Hand Deformities 2878: 2686:Craniodiaphyseal dysplasia 1700:10.1016/j.jhsa.2004.04.002 1473:10.1016/j.jhsa.2007.06.017 1146:10.1016/j.jhsa.2009.12.021 814:2020 US Open Quad Champion 678:One to four limbs involved 195:Cornelia de Lange syndrome 29: 2660:Treacher Collins syndrome 2511:reduction deficits / limb 1651:10.1016/j.hcl.2008.10.005 1548:Barsky, Arthur J (1964). 56: 47: 2485:Cenani–Lenz syndactylism 2238:Cleidocranial dysostosis 1247:Human Molecular Genetics 731:people with ectrodactyly 286:Ectrodactyly-Polydactyly 2632:Craniofacial dysostosis 1311:10.1126/science.1083423 1190:10.1136/jmg.2003.010587 806:, freak show performer. 487:Narrowed first webspace 302:apical ectodermal ridge 255:apical ectodermal ridge 209:Focal dermal hypoplasia 188:Silver–Russell syndrome 2188:musculoskeletal system 1879:10.1098/rsbl.2008.0233 1775:Journal of Herpetology 732: 636: 619: 611: 237:Buttien-Fryns syndrome 2740:Klippel–Feil syndrome 748:Grady Stiles Sr. and 727: 617: 609: 408:Severely narrowed web 2750:Spina bifida occulta 2266:Madelung's deformity 2243:Sprengel's deformity 2044:at Wikimedia Commons 2007:Veterinary Radiology 1910:Jezyk, P.F. (1985). 1405:10.1136/jmg.24.8.509 942:Oman Medical Journal 784:World Chess Champion 494:Aesthetical aspects 2568:RAPADILINO syndrome 1928:Journal of Heredity 1728:. January 27, 2007. 1303:2003Sci...300..767S 1026:Cleft Palate Speech 954:10.5001/omj.2011.67 397:Mildly narrowed web 326:variable penetrance 250:autosomal recessive 137:congenital disorder 32:split hand syndrome 2430:Rocker bottom foot 1823:10.1007/BF02615104 1261:10.1093/hmg/ddg090 769:tribe in northern 733: 694:Autosomal dominant 654:Typical cleft hand 620: 612: 498:Reducing deformity 475:Improving function 322:autosomal dominant 246:autosomal dominant 202:Acrorenal syndrome 2844: 2843: 2840: 2839: 2836: 2835: 2765:Thoracic skeleton 2745:Spondylolisthesis 2714: 2713: 2580: 2579: 2576: 2575: 2443: 2442: 2289: 2288: 2150: 2149: 2040:Media related to 1297:(5620): 767–772. 1118:978-0-443-06626-9 1037:978-0-8151-3153-3 922:978-0-19-263222-7 709: 708: 565: 564: 448: 447: 419:Syndactylized web 242: 241: 85: 84: 57:Ectrodactyly and 37:Medical condition 16:(Redirected from 2869: 2812:Pectus carinatum 2807:Pectus excavatum 2770: 2728:Spinal curvature 2720:Vertebral column 2640:Crouzon syndrome 2604:Craniosynostosis 2600: 2591: 2456:fingers and toes 2452: 2364:Discoid meniscus 2321:Upington disease 2300: 2222: 2213: 2177: 2170: 2163: 2154: 2067: 2039: 2023: 2022: 2002: 1996: 1995: 1993: 1983: 1959: 1953: 1952: 1922: 1916: 1915: 1907: 1901: 1900: 1890: 1858: 1843: 1842: 1806: 1800: 1799: 1768: 1757: 1756: 1736: 1730: 1729: 1718: 1712: 1711: 1683: 1674: 1669: 1663: 1662: 1634: 1625: 1624: 1596: 1587: 1584: 1578: 1577: 1545: 1526: 1525: 1515: 1491: 1485: 1484: 1455: 1449: 1448: 1440: 1427: 1426: 1416: 1382: 1376: 1375: 1339: 1333: 1332: 1322: 1280: 1274: 1273: 1263: 1237: 1212: 1211: 1201: 1167: 1158: 1157: 1129: 1123: 1122: 1104: 1059: 1048: 1042: 1041: 1029: 1019: 1013: 1012: 982: 976: 975: 965: 933: 927: 926: 906: 750:Grady Stiles Jr. 714:symbrachydactyly 689:Lower incidence 686:Higher incidence 651: 647:Symbrachydactyly 632:Hippo-Diarrhytus 590:Miura and Komada 523: 378:Characteristics 369: 330:de novo mutation 165: 81: 80: 72:Medical genetics 52: 40: 21: 2877: 2876: 2872: 2871: 2870: 2868: 2867: 2866: 2847: 2846: 2845: 2832: 2828:Poland syndrome 2816: 2792: 2759: 2710: 2664: 2626: 2572: 2563:Larsen syndrome 2551:multiple joints 2546: 2506: 2439: 2378: 2359:Genu recurvatum 2335: 2312:Hip dislocation 2285: 2252: 2202: 2194: 2181: 2151: 2146: 2145: 2078: 2032: 2027: 2026: 2004: 2003: 1999: 1961: 1960: 1956: 1924: 1923: 1919: 1909: 1908: 1904: 1867:Biology Letters 1860: 1859: 1846: 1808: 1807: 1803: 1770: 1769: 1760: 1738: 1737: 1733: 1720: 1719: 1715: 1685: 1684: 1677: 1670: 1666: 1636: 1635: 1628: 1598: 1597: 1590: 1585: 1581: 1547: 1546: 1529: 1493: 1492: 1488: 1457: 1456: 1452: 1442: 1441: 1430: 1384: 1383: 1379: 1341: 1340: 1336: 1282: 1281: 1277: 1239: 1238: 1215: 1169: 1168: 1161: 1131: 1130: 1126: 1119: 1106: 1105: 1062: 1049: 1045: 1038: 1021: 1020: 1016: 984: 983: 979: 935: 934: 930: 923: 908: 907: 903: 898: 880: 863: 851: 842: 833: 820:Francesca Jones 722: 673:U-shaped cleft 649: 604: 592: 583: 570: 514: 505: 469: 453: 360: 355: 338: 318: 298: 296:Pathophysiology 163: 154: 75: 38: 35: 28: 23: 22: 15: 12: 11: 5: 2875: 2873: 2865: 2864: 2859: 2849: 2848: 2842: 2841: 2838: 2837: 2834: 2833: 2831: 2830: 2824: 2822: 2818: 2817: 2815: 2814: 2809: 2803: 2801: 2794: 2793: 2791: 2790: 2785: 2779: 2777: 2767: 2761: 2760: 2758: 2757: 2752: 2747: 2742: 2737: 2736: 2735: 2724: 2722: 2716: 2715: 2712: 2711: 2709: 2708: 2703: 2698: 2693: 2691:Dolichocephaly 2688: 2683: 2678: 2672: 2670: 2666: 2665: 2663: 2662: 2657: 2652: 2647: 2642: 2636: 2634: 2628: 2627: 2625: 2624: 2622:Trigonocephaly 2619: 2614: 2608: 2606: 2597: 2595:Skull and face 2588: 2582: 2581: 2578: 2577: 2574: 2573: 2571: 2570: 2565: 2560: 2558:Arthrogryposis 2554: 2552: 2548: 2547: 2545: 2544: 2543: 2542: 2537: 2532: 2520: 2514: 2512: 2508: 2507: 2505: 2504: 2503: 2502: 2492: 2487: 2482: 2480:Arachnodactyly 2477: 2476: 2475: 2460: 2458: 2449: 2445: 2444: 2441: 2440: 2438: 2437: 2432: 2427: 2422: 2421: 2420: 2408: 2407: 2406: 2401: 2388: 2386: 2384:foot deformity 2380: 2379: 2377: 2376: 2371: 2366: 2361: 2356: 2351: 2345: 2343: 2337: 2336: 2334: 2333: 2328: 2323: 2318: 2308: 2306: 2297: 2291: 2290: 2287: 2286: 2284: 2283: 2278: 2273: 2268: 2262: 2260: 2258:hand deformity 2254: 2253: 2251: 2250: 2245: 2240: 2234: 2232: 2219: 2210: 2196: 2195: 2182: 2180: 2179: 2172: 2165: 2157: 2148: 2147: 2144: 2143: 2132: 2121: 2110: 2095: 2079: 2074: 2073: 2071: 2070:Classification 2064: 2063: 2054: 2045: 2031: 2030:External links 2028: 2025: 2024: 1997: 1954: 1917: 1902: 1844: 1817:(7): 434–442. 1801: 1788:10.1670/95-03N 1782:(2): 283–287. 1758: 1741:S. Afr. Med. J 1731: 1713: 1675: 1664: 1626: 1588: 1579: 1527: 1486: 1450: 1428: 1399:(8): 509–510. 1377: 1334: 1275: 1254:(1): R51–R60. 1213: 1159: 1124: 1117: 1060: 1043: 1036: 1014: 977: 928: 921: 900: 899: 897: 894: 893: 892: 889:Sonic hedgehog 886: 879: 876: 862: 859: 850: 847: 841: 838: 832: 829: 828: 827: 817: 807: 804:Black Scorpion 801: 795: 794: 793: 782:chess player, 773: 763: 756:reality show, 746: 721: 718: 707: 706: 703: 699: 698: 695: 691: 690: 687: 683: 682: 679: 675: 674: 671: 670:V-shaped cleft 667: 666: 663: 659: 658: 655: 648: 645: 603: 600: 591: 588: 582: 579: 569: 566: 563: 562: 559: 555: 554: 551: 547: 546: 543: 539: 538: 535: 531: 530: 527: 513: 510: 504: 501: 500: 499: 492: 491: 488: 485: 482: 479: 476: 468: 465: 452: 449: 446: 445: 442: 439: 435: 434: 431: 428: 424: 423: 420: 417: 413: 412: 409: 406: 402: 401: 398: 395: 391: 390: 387: 384: 380: 379: 376: 373: 359: 358:Classification 356: 354: 351: 337: 334: 317: 314: 297: 294: 278:ADULT syndrome 240: 239: 233: 232: 226: 225: 219: 218: 212: 211: 205: 204: 198: 197: 191: 190: 184: 183: 177: 176: 170: 169: 162: 159: 153: 150: 83: 82: 69: 63: 62: 54: 53: 45: 44: 36: 26: 24: 14: 13: 10: 9: 6: 4: 3: 2: 2874: 2863: 2860: 2858: 2857:Rare diseases 2855: 2854: 2852: 2829: 2826: 2825: 2823: 2819: 2813: 2810: 2808: 2805: 2804: 2802: 2799: 2795: 2789: 2786: 2784: 2781: 2780: 2778: 2775: 2771: 2768: 2766: 2762: 2756: 2755:Sacralization 2753: 2751: 2748: 2746: 2743: 2741: 2738: 2734: 2731: 2730: 2729: 2726: 2725: 2723: 2721: 2717: 2707: 2704: 2702: 2701:Plagiocephaly 2699: 2697: 2694: 2692: 2689: 2687: 2684: 2682: 2679: 2677: 2674: 2673: 2671: 2667: 2661: 2658: 2656: 2653: 2651: 2648: 2646: 2645:Hypertelorism 2643: 2641: 2638: 2637: 2635: 2633: 2629: 2623: 2620: 2618: 2615: 2613: 2612:Scaphocephaly 2610: 2609: 2607: 2605: 2601: 2598: 2596: 2592: 2589: 2587: 2583: 2569: 2566: 2564: 2561: 2559: 2556: 2555: 2553: 2549: 2541: 2538: 2536: 2533: 2531: 2528: 2527: 2526: 2525: 2521: 2519: 2516: 2515: 2513: 2509: 2501: 2498: 2497: 2496: 2495:Brachydactyly 2493: 2491: 2488: 2486: 2483: 2481: 2478: 2474: 2471: 2470: 2469: 2465: 2462: 2461: 2459: 2457: 2453: 2450: 2448:Either / both 2446: 2436: 2433: 2431: 2428: 2426: 2423: 2419: 2416: 2415: 2414: 2413: 2409: 2405: 2402: 2400: 2397: 2396: 2395: 2394: 2390: 2389: 2387: 2385: 2381: 2375: 2372: 2370: 2367: 2365: 2362: 2360: 2357: 2355: 2352: 2350: 2347: 2346: 2344: 2342: 2338: 2332: 2329: 2327: 2324: 2322: 2319: 2317: 2316:Hip dysplasia 2313: 2310: 2309: 2307: 2305: 2301: 2298: 2296: 2292: 2282: 2279: 2277: 2274: 2272: 2269: 2267: 2264: 2263: 2261: 2259: 2255: 2249: 2246: 2244: 2241: 2239: 2236: 2235: 2233: 2231: 2227: 2223: 2220: 2218: 2214: 2211: 2209: 2205: 2201: 2197: 2193: 2189: 2185: 2178: 2173: 2171: 2166: 2164: 2159: 2158: 2155: 2142: 2138: 2137: 2133: 2131: 2127: 2126: 2122: 2120: 2116: 2115: 2111: 2109: 2105: 2104: 2100: 2096: 2094: 2090: 2089: 2085: 2081: 2080: 2077: 2072: 2068: 2062: 2058: 2055: 2053: 2049: 2046: 2043: 2038: 2034: 2033: 2029: 2020: 2016: 2013:(3): 123–44. 2012: 2008: 2001: 1998: 1992: 1987: 1982: 1977: 1973: 1969: 1965: 1958: 1955: 1950: 1946: 1942: 1938: 1934: 1930: 1929: 1921: 1918: 1913: 1906: 1903: 1898: 1894: 1889: 1884: 1880: 1876: 1873:(5): 549–52. 1872: 1868: 1864: 1857: 1855: 1853: 1851: 1849: 1845: 1840: 1836: 1832: 1828: 1824: 1820: 1816: 1812: 1805: 1802: 1797: 1793: 1789: 1785: 1781: 1777: 1776: 1767: 1765: 1763: 1759: 1754: 1750: 1747:(13): 531–3. 1746: 1742: 1735: 1732: 1727: 1723: 1717: 1714: 1709: 1705: 1701: 1697: 1693: 1689: 1682: 1680: 1676: 1673: 1668: 1665: 1660: 1656: 1652: 1648: 1645:(2): 157–70. 1644: 1640: 1633: 1631: 1627: 1622: 1618: 1614: 1610: 1607:(4): 687–97. 1606: 1602: 1595: 1593: 1589: 1583: 1580: 1575: 1571: 1567: 1563: 1559: 1555: 1551: 1544: 1542: 1540: 1538: 1536: 1534: 1532: 1528: 1523: 1519: 1514: 1509: 1506:(6): 533–41. 1505: 1501: 1497: 1490: 1487: 1482: 1478: 1474: 1470: 1467:(9): 1413–7. 1466: 1462: 1454: 1451: 1446: 1439: 1437: 1435: 1433: 1429: 1424: 1420: 1415: 1410: 1406: 1402: 1398: 1394: 1393: 1388: 1381: 1378: 1373: 1369: 1365: 1361: 1357: 1353: 1349: 1345: 1338: 1335: 1330: 1326: 1321: 1316: 1312: 1308: 1304: 1300: 1296: 1292: 1291: 1286: 1279: 1276: 1271: 1267: 1262: 1257: 1253: 1249: 1248: 1243: 1236: 1234: 1232: 1230: 1228: 1226: 1224: 1222: 1220: 1218: 1214: 1209: 1205: 1200: 1195: 1191: 1187: 1183: 1179: 1178: 1173: 1166: 1164: 1160: 1155: 1151: 1147: 1143: 1139: 1135: 1128: 1125: 1120: 1114: 1110: 1103: 1101: 1099: 1097: 1095: 1093: 1091: 1089: 1087: 1085: 1083: 1081: 1079: 1077: 1075: 1073: 1071: 1069: 1067: 1065: 1061: 1058: 1054: 1053: 1047: 1044: 1039: 1033: 1028: 1027: 1018: 1015: 1010: 1006: 1002: 998: 995:(3): 479–81. 994: 990: 989: 981: 978: 973: 969: 964: 959: 955: 951: 947: 943: 939: 932: 929: 924: 918: 914: 913: 905: 902: 895: 890: 887: 885: 882: 881: 877: 875: 872: 867: 861:Cats and dogs 860: 858: 856: 848: 846: 839: 837: 830: 825: 821: 818: 815: 811: 808: 805: 802: 799: 796: 791: 788: 787: 785: 781: 777: 774: 772: 768: 764: 761: 760: 755: 751: 747: 744: 743: 738: 735: 734: 730: 726: 720:Notable cases 719: 717: 715: 704: 701: 700: 696: 693: 692: 688: 685: 684: 680: 677: 676: 672: 669: 668: 664: 661: 660: 656: 653: 652: 646: 644: 641: 640:Ambroise ParĂ© 635: 633: 627: 625: 616: 608: 601: 599: 597: 589: 587: 580: 578: 576: 567: 560: 557: 556: 552: 549: 548: 544: 541: 540: 536: 533: 532: 528: 525: 524: 521: 518: 511: 509: 502: 497: 496: 495: 489: 486: 483: 480: 477: 474: 473: 472: 466: 464: 460: 458: 457:heterogeneity 450: 443: 440: 437: 436: 432: 429: 426: 425: 421: 418: 415: 414: 410: 407: 404: 403: 399: 396: 393: 392: 388: 385: 382: 381: 377: 374: 371: 370: 367: 364: 357: 352: 350: 348: 344: 335: 333: 331: 327: 323: 315: 313: 311: 307: 303: 295: 293: 291: 290:Dysmorphology 287: 283: 279: 275: 269: 267: 262: 258: 256: 251: 247: 238: 235: 234: 231: 228: 227: 224: 221: 220: 217: 214: 213: 210: 207: 206: 203: 200: 199: 196: 193: 192: 189: 186: 185: 182: 179: 178: 175: 172: 171: 167: 166: 160: 158: 151: 149: 147: 141: 138: 133: 131: 127: 123: 119: 115: 111: 108: 104: 101: 100:Ancient Greek 97: 93: 89: 79: 73: 70: 68: 64: 60: 55: 51: 46: 41: 33: 19: 2676:Macrocephaly 2522: 2518:Acheiropodia 2490:Ectrodactyly 2489: 2410: 2391: 2276:Oligodactyly 2271:Clinodactyly 2200:Appendicular 2134: 2123: 2112: 2097: 2082: 2042:Ectrodactyly 2010: 2006: 2000: 1974:(4): 910–3. 1971: 1967: 1957: 1935:(1): 31–34. 1932: 1926: 1920: 1911: 1905: 1870: 1866: 1814: 1810: 1804: 1779: 1773: 1744: 1740: 1734: 1725: 1716: 1694:(4): 720–6. 1691: 1687: 1667: 1642: 1639:Hand Clinics 1638: 1604: 1600: 1582: 1557: 1553: 1503: 1499: 1489: 1464: 1460: 1453: 1444: 1396: 1390: 1380: 1350:(1): 16–20. 1347: 1343: 1337: 1294: 1288: 1278: 1251: 1245: 1181: 1175: 1140:(3): 480–5. 1137: 1133: 1127: 1108: 1050: 1046: 1025: 1017: 992: 986: 980: 948:(4): 275–8. 945: 941: 931: 911: 904: 884:Oligodactyly 868: 864: 852: 843: 834: 824:WTA rankings 810:Sam Schröder 757: 740: 710: 637: 629: 623: 621: 593: 584: 571: 568:Snow-Littler 519: 515: 506: 493: 478:Absent thumb 470: 461: 454: 365: 361: 339: 319: 299: 270: 263: 259: 243: 155: 152:Presentation 142: 134: 125: 121: 113: 110: 106: 103: 95: 91: 88:Ectrodactyly 87: 86: 43:Ectrodactyly 2706:Saddle nose 2473:Webbed toes 2464:Polydactyly 2349:Genu valgum 2281:Polydactyly 1991:10183/76981 1560:: 1707–20. 855:salamanders 849:Salamanders 776:Mikhail Tal 737:Bree Walker 624:City of God 575:metacarpals 467:Indications 375:Description 306:Polydactyly 2851:Categories 2681:Platybasia 2617:Oxycephaly 2530:Phocomelia 2524:Ectromelia 2500:Stub thumb 2468:Syndactyly 2435:Hammer toe 2404:Pigeon toe 2354:Genu varum 2326:Coxa valga 2184:Congenital 2136:DiseasesDB 1445:Cleft Hand 1184:(5): e54. 896:References 798:Lee Hee-ah 790:Photograph 596:Z-plasties 529:Treatment 441:Absent web 430:Merged web 386:Normal web 336:Embryology 324:and has a 310:syndactyly 292:Database. 130:syndactyly 114:(daktylos) 98:(from 96:cleft hand 92:split hand 59:syndactyly 18:Cleft hand 2733:Scoliosis 2540:Hemimelia 2425:Pes cavus 2418:Flat feet 2399:Club foot 2331:Coxa vara 1057:eMedicine 1030:. Mosby. 840:Wood frog 759:Freakshow 697:Sporadic 643:century. 451:Treatment 353:Diagnosis 347:gestation 168:Syndrome 107:(ektroma) 67:Specialty 2783:Cervical 2230:shoulder 2226:clavicle 2208:dysmelia 2059:(OMIM): 2050:(OMIM): 1897:18593670 1839:10311465 1811:In Vitro 1796:86275852 1726:ABC News 1708:15249100 1659:19380058 1574:14239859 1522:17661743 1481:17996777 1372:25692622 1364:22121204 1329:12690205 1270:12668597 1208:15121782 1154:20138711 972:22043435 878:See also 786:1960–61 771:Zimbabwe 742:Nip/Tuck 490:The feet 316:Genetics 161:Genetics 112:ÎŽÎŹÎșÏ„Ï…Î»ÎżÏ‚ 2798:sternum 2130:C574275 1888:2610075 1753:6710256 1621:7594304 1423:3656376 1414:1050214 1320:2882961 1299:Bibcode 1290:Science 1199:1735762 1009:8737656 963:3191709 871:Terrier 831:Animals 602:History 542:IIB/III 343:Myleran 105:ጔÎșτρωΌα 2821:other: 2535:Amelia 2412:valgus 2119:225300 2061:183800 2052:183600 1949:864231 1947:  1895:  1885:  1837:  1831:885563 1829:  1794:  1751:  1706:  1657:  1619:  1572:  1520:  1479:  1421:  1411:  1370:  1362:  1327:  1317:  1268:  1206:  1196:  1152:  1115:  1034:  1007:  970:  960:  919:  780:Soviet 767:Vadoma 729:Vadoma 118:digits 74:  2788:Bifid 2669:other 2586:Axial 2393:varus 2141:32141 2108:755.4 2093:Q71.6 1835:S2CID 1792:S2CID 1368:S2CID 534:I/IIA 146:SHFM1 102: 94:, or 2774:ribs 2341:knee 2217:Arms 2204:limb 2125:MeSH 2114:OMIM 2103:9-CM 1945:PMID 1893:PMID 1827:PMID 1749:PMID 1704:PMID 1655:PMID 1617:PMID 1570:PMID 1518:PMID 1477:PMID 1419:PMID 1360:PMID 1325:PMID 1266:PMID 1204:PMID 1150:PMID 1113:ISBN 1032:ISBN 1005:PMID 968:PMID 917:ISBN 765:The 581:Ueba 573:the 526:Type 372:Type 266:DLX5 126:SHFM 2304:hip 2295:Leg 2099:ICD 2084:ICD 2015:doi 1986:hdl 1976:doi 1937:doi 1883:PMC 1875:doi 1819:doi 1784:doi 1696:doi 1647:doi 1609:doi 1562:doi 1508:doi 1469:doi 1409:PMC 1401:doi 1352:doi 1315:PMC 1307:doi 1295:300 1256:doi 1194:PMC 1186:doi 1142:doi 1055:at 997:doi 958:PMC 950:doi 754:AMC 630:At 416:III 405:IIB 394:IIA 304:). 2853:: 2466:/ 2314:/ 2228:/ 2206:/ 2190:/ 2139:: 2128:: 2117:: 2106:: 2091:: 2088:10 2011:22 2009:. 1984:. 1972:59 1970:. 1966:. 1943:. 1933:68 1931:. 1891:. 1881:. 1869:. 1865:. 1847:^ 1833:. 1825:. 1815:13 1813:. 1790:. 1780:38 1778:. 1761:^ 1745:65 1743:. 1724:. 1702:. 1692:29 1690:. 1678:^ 1653:. 1643:25 1641:. 1629:^ 1615:. 1605:20 1603:. 1591:^ 1568:. 1558:46 1556:. 1552:. 1530:^ 1516:. 1504:49 1502:. 1498:. 1475:. 1465:32 1463:. 1431:^ 1417:. 1407:. 1397:24 1395:. 1389:. 1366:. 1358:. 1348:49 1346:. 1323:. 1313:. 1305:. 1293:. 1287:. 1264:. 1252:12 1250:. 1244:. 1216:^ 1202:. 1192:. 1182:41 1180:. 1174:. 1162:^ 1148:. 1138:35 1136:. 1063:^ 1003:. 993:63 991:. 966:. 956:. 946:26 944:. 940:. 812:, 778:, 598:. 550:IV 427:IV 349:. 308:, 90:, 2800:: 2776:: 2176:e 2169:t 2162:v 2101:- 2086:- 2076:D 2021:. 2017:: 1994:. 1988:: 1978:: 1951:. 1939:: 1899:. 1877:: 1871:4 1841:. 1821:: 1798:. 1786:: 1755:. 1710:. 1698:: 1661:. 1649:: 1623:. 1611:: 1576:. 1564:: 1524:. 1510:: 1483:. 1471:: 1425:. 1403:: 1374:. 1354:: 1331:. 1309:: 1301:: 1272:. 1258:: 1210:. 1188:: 1156:. 1144:: 1121:. 1040:. 1011:. 999:: 974:. 952:: 925:. 826:. 816:. 762:. 558:V 438:V 383:I 124:( 34:. 20:)

Index

Cleft hand
split hand syndrome

syndactyly
Specialty
Medical genetics
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Ancient Greek
digits
syndactyly
congenital disorder
SHFM1
Ectrodactyly–ectodermal dysplasia–cleft syndrome
Split-Hand-Foot Malformation Syndrome
Silver–Russell syndrome
Cornelia de Lange syndrome
Acrorenal syndrome
Focal dermal hypoplasia
Ectrodactyly and cleft palate syndrome
Ectrodactyly/mandibulofacial dysostosis
Ectrodactyly and macular dystrophy
Buttien-Fryns syndrome
autosomal dominant
autosomal recessive
apical ectodermal ridge
DLX5
Ectrodactyly-Ectodermal Dysplasia-Clefting (EEC) syndrome
ADULT syndrome
Ectrodactyly-Ectodermal Dysplasia-Macular Dystrophy syndrome
Ectrodactyly-Polydactyly

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