asplenia
Image Gallery
[ (||image_reduire{0,60}|inserer_attribut{alt,Medial liver and asplenia in heterotaxy with asplenia/polysplenia (Ivemark (...)}) ] [ (||image_reduire{0,60}|inserer_attribut{alt,Dextrocardia with pulmonary artery hypoplasia in heterotaxy with (...)}) ]
Synopsis
recurrent invasive bacterial infections
Howell-Jolly bodies in the blood smear
relative with congenital isolated asplenia.
Etiology
isolated asplenia
- isolated congenital asplenia
- vanishing spleen and hyposplenism
syndromic asplenia
- asplenia with situs ambiguus (heterotaxia)
- asplenia/polysplenia syndrome (Ivemark syndrome) (MIM.208530)
- deleted ring chromosome 4 (#8933346#)
Associations
cardiovascular malformations
- dextrocardia (#11105625#)
- transposition of the great vessels (TGV) (#11105625#)
- stenosis of the pulmonary artery (#11105625#)
- common atrioventricular canal (CAVC) (#11105625#)
- patent ductus arteriosus (#11105625#)
situs anomalies (heterotaxy)
renal dysplasia
- cystic dilated collecting ducts of the kidney (#11105625#)
cystic dilated collecting ducts of the kidney (#11105625#)
hepatopancreaticorenal dysplasia
- Crawfurd syndrome (hepatopancreaticorenal dysplasia with asplenia)