| ID |
http://id.who.int/icd/entity/353005375 |
| Preferred Name |
complexe de la paroi latérale du corps |
| Definitions |
Limb body wall complex (LBWC) is characterized by severe multiple congenital anomalies in the fetus with exencephaly/encephalocele, thoraco- and/or abdominoschisis (anterior body wall defects) and limb defects, with or without facial clefts. Approximately 250 cases have been reported in the literature so far. Clinical manifestations vary widely and include limb defects and visceral malformations (95% of cases), spinal abnormalities, absent diaphragm, bowel atresia and renal agenesis. LBWC generally presents as a short umbilical cord, abdominal placental attachment, persistence of an extraembryonic celom, anorectal malformations, urogenital abnormalities, lumbosacral meningomyelocele and kyphoscoliosis. A spectrum of LBWC manifestations is included in the amniotic band sequence (see this term), presenting mostly with craniofacial defects, facial clefts, amniotic bands and/or adhesions. At present, it remains unclear whether these two entities represent a single disorder. The etiology of LBWC remains unknown. Karyotypes have been reported as normal and no correlations with gender, parental age and teratogenic agents have been found. The principal theories are an extrinsic origin by early amniotic rupture, or a vascular origin due to an early vascular accident during embryological development. Single cases of familial occurrence have been documented. Diagnosis is based on the presenting features. Early antenatal diagnosis is feasible by ultrasound examination and may be followed by medical termination of the pregnancy. LBWC should be differentiated from gastroschisis (see this term), which has better prognosis. LBWC is fatal, with death occurring during antenatally or early in the neonatal period. |
| Type |
http://www.w3.org/2002/07/owl#Class |
| comment | Extremitätenwandkomplex (lbwc) ist durch schwere multiple angeborene Anomalien im Fötus mit Exenzephalie / Enzephalozele, Thorako- und / oder Abdominoschisis (Defekte der vorderen Körperwand) und Gliedmaße mit oder ohne Gesichtsspalten gekennzeichnet. ca. 250 Fälle wurden bisher in der Literatur berichtet. klinische Manifestationen variieren stark und umfassen Extremitätendefekte und viszerale Fehlbildungen (95% der Fälle), Wirbelsäulenanomalien, fehlendes Zwerchfell, Darmatresistenz und Nierenagenese. lbwc präsentiert sich in der Regel als kurze Nabelschnur, abdominale Plazenta-Attachment, Persistenz eines extraembryonalen Zeloms, anorektale Fehlbildungen, η πρώιμη προγεννητική διάγνωση, η οποία με την πρωτοπαθή ή περιθωριοποίηση (βλ. αυτό το όρο), η οποία έχει ως επί το πλείστον με υπερεγκεφαλικές ανωμαλίες, υπερηχοκαρδιογράφημα (βλ. αυτό το όρο), η οποία έχει ως επί το πλείστον με κρανιοεγκεφαλικές ανωμαλίες, την επίμονη θωρακική προσκόλληση, επίμονο εξόγκωμα, επίμονο ανωμαλίες, ουρογεννητικές ανωμαλίες, οσφυαλγίες της σπονδυλικής στήλης (95% των περιπτώσεων), spinal abnormalities, absent diaphragm, atresia on atresia και renal agenesis. lbwc γενικά παρουσιάζει ως ένα σύντομο ομφάλιο λώρο, κοιλιακή θωμερική προσκόλληση, επίμονο extraembryonic celom, anorectal malformations, urogenital abnormalities, lumbosacr le diagnostic précoce de l'apparition du cancer du col de l'utérus (aip) peut être associé à des anomalies congénitales multiples graves chez le foetus avec exencéphalie / encéphalocèle, thoraco- et / ou abdominoschisis (anomalies de la paroi antérieure du corps) et des malformations des membres, avec ou sans fissures faciales. jusqu'à présent, environ 250 cas ont été signalés dans la littérature. les manifestations cliniques varient considérablement et comprennent des anomalies d'un membre et des malformations viscérales (95 % des cas), des anomalies médullaires, l'absence de diaphragme, d'atrésie de l'intestin et d'agenèse rénale. de façon générale, il n'est pas clair si ces deux entités représentent un seul trouble. l "étiologie des manifestations de la tour du côlon extra-embryonnaire, des malformations anorectales, urogénitales, Limb body wall complex (LBWC) is characterized by severe multiple congenital anomalies in the fetus with exencephaly/encephalocele, thoraco- and/or abdominoschisis (anterior body wall defects) and limb defects, with or without facial clefts. Approximately 250 cases have been reported in the literature so far. Clinical manifestations vary widely and include limb defects and visceral malformations (95% of cases), spinal abnormalities, absent diaphragm, bowel atresia and renal agenesis. LBWC generally presents as a short umbilical cord, abdominal placental attachment, persistence of an extraembryonic celom, anorectal malformations, urogenital abnormalities, lumbosacral meningomyelocele and kyphoscoliosis. A spectrum of LBWC manifestations is included in the amniotic band sequence (see this term), presenting mostly with craniofacial defects, facial clefts, amniotic bands and/or adhesions. At present, it remains unclear whether these two entities represent a single disorder. The etiology of LBWC remains unknown. Karyotypes have been reported as normal and no correlations with gender, parental age and teratogenic agents have been found. The principal theories are an extrinsic origin by early amniotic rupture, or a vascular origin due to an early vascular accident during embryological development. Single cases of familial occurrence have been documented. Diagnosis is based on the presenting features. Early antenatal diagnosis is feasible by ultrasound examination and may be followed by medical termination of the pregnancy. LBWC should be differentiated from gastroschisis (see this term), which has better prognosis. LBWC is fatal, with death occurring during antenatally or early in the neonatal period. rozpoznano jednoznaczne przypadki rozpoznania gruczołu krokowego w okresie pierwotnym (patrz to określenie), z których rozpoznano wczesne rozpoznanie gruczołu krokowego (b) u noworodków, u których rozpoznano niepłodność gruczołu krokowego, nieżyt kończyn, nieprawidłowości w obrębie narządów moczowych, zaburzenia urogenitalne, nieprawidłowości żylaków, wady kończyn mózgowych i wady owrzoczne (95% przypadków), nieprawidłowości kręgosłupa, nieobecna przeponę, bowel wrezji i nerek. lbwc ogólnie przedstawia jako krótki pępowinę pępkową, brzusznej załącznika, wytrwałość pozaembrionalny celom, anorektalne nieprawidłowości, nieprawidłowości urogenitalne, nieprawidłowości lumboacral, wady kończyn kończyn i wady kończyn (95% przypadków), nieprawidłowości kręgosłupa, przeponowy przeponowy i nerki nerkowoka. lb |
| broaderTransitive | |
| prefLabel | complexe de la paroi latérale du corps |
| browserUrl | NA |
| label | complexe de la paroi latérale du corps Seitenwand-Komplex boki boczne boczne kompleks Lateral body wall complex πολυμερές συγκρότημα τοίχων σώματος |
| subClassOf |
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