Asplenia syndrome and isolated total anomalous pulmonary venous connection in siblings

We report on a family with asplenia syndrome in one and total anomalous pulmonary venous connection (TAPVC) in the other sib. Both conditions are rare, may have a genetic cause and belong to a spectrum of laterality disorders. This suggests that both asplenia syndrome and TAPVC in this family are th...

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Veröffentlicht in:	European journal of pediatrics 1994-10, Vol.153 (10), p.712-714
Hauptverfasser:	DEVRIENDT, K, CASAER, A, VAN CAUTER, A, DE ZEGHER, F, DUMOULIN, M, GEWILLIG, M, DEVLIEGER, H
Format:	Artikel
Sprache:	eng
Schlagworte:	Biological and medical sciences Complex syndromes Heart Defects, Congenital - genetics Humans Infant Infant, Newborn Lung - abnormalities Lung - blood supply Male Medical genetics Medical sciences Pedigree Situs Inversus - complications Situs Inversus - genetics Spleen - abnormalities Syndrome
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Zusammenfassung:	We report on a family with asplenia syndrome in one and total anomalous pulmonary venous connection (TAPVC) in the other sib. Both conditions are rare, may have a genetic cause and belong to a spectrum of laterality disorders. This suggests that both asplenia syndrome and TAPVC in this family are the clinical expression of a single genetic disorder.
ISSN:	0340-6199 1432-1076
DOI:	10.1007/BF01954484