Asplenia and functional hyposplenism in autoimmune polyglandular syndrome type 1.
Asplenia was diagnosed in four patients with autoimmune polyendocrine syndrome type-I (APS-I): two children, aged 2-4 years, from the same family and two adults, the father of the two children and his cousin. We have observed a worsening in splenic function in the children during a follow-up of a few years. Patients with APS-I should be evaluated for splenic function, since splenic dysfunction has important therapeutic implications, especially in children.
Pollak
Uri
U
Department of Pediatrics B, Schneider Children's Medical Center of Israel, Petah Tiqwa 49202, Israel.
Bar-Sever
Zvi
Z
Hoffer
Vered
V
Marcus
Nufar
N
Scheuerman
Oded
O
Garty
Ben Zion
BZ
D000328:Adult; D000900:Anti-Bacterial Agents / Q000008:administration & dosage; D002648:Child; D002675:Child, Preschool; D003241:Consanguinity; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007492:Iran / Q000208:ethnology; D007557:Israel; D007585:Jews / Q000235:genetics; D008134:Long-Term Care; D008297:Male; D010641:Phenotype; D022242:Pneumococcal Vaccines / Q000008:administration & dosage; D016884:Polyendocrinopathies, Autoimmune / Q000175:diagnosis / Q000235:genetics* / Q000628:therapy; D013154:Spleen / Q000002:abnormalities*
D000900:Anti-Bacterial Agents; D022242:Pneumococcal Vaccines