Septicaemia and adrenal haemorrhage in congenital asplenia.
Five patients developed overwhelming infection as a result of congenital asplenia, which was previously unsuspected in all cases. Each illness followed a fulminant course resulting in death within 24 hours. They illustrate the respective roles of Haemophilus influenzae infection (n = 4) and adrenal haemorrhage (n = 4) in this condition. We suggest a management protocol for screening infants with abnormalities of the atria or viscera with splenic ultrasound and examination of a blood film for Howell-Jolly bodies. Vaccination and prophylactic antibiotics should be considered for those at risk. Vigorous use of parenteral antibiotics and steroids in suspected infection is recommended.
Dyke
M P
MP
Royal Hospital for Sick Children, Bristol.
Martin
R P
RP
Berry
P J
PJ
D000015:Abnormalities, Multiple; D000307:Adrenal Gland Diseases / Q000209:etiology*; D002675:Child, Preschool; D003645:Death, Sudden / Q000209:etiology; D005260:Female; D006192:Haemophilus Infections / Q000150:complications; D006193:Haemophilus influenzae; D006330:Heart Defects, Congenital / Q000150:complications; D006470:Hemorrhage / Q000209:etiology*; D006801:Humans; D007223:Infant; D008297:Male; D018805:Sepsis / Q000209:etiology*; D013154:Spleen / Q000002:abnormalities*