PMCCPMCCPMCC

Search tips
Search criteria 

Advanced

 
Logo of archdischArchives of Disease in ChildhoodVisit this articleSubmit a manuscriptReceive email alertsContact usBMJ
 
Arch Dis Child. 1991 May; 66(5): 636–637.
PMCID: PMC1792930

Septicaemia and adrenal haemorrhage in congenital asplenia.

Abstract

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.

Full text

Full text is available as a scanned copy of the original print version. Get a printable copy (PDF file) of the complete article (392K), or click on a page image below to browse page by page. Links to PubMed are also available for Selected References.
 
 

Selected References

These references are in PubMed. This may not be the complete list of references from this article.
  • IVEMARK BI. Implications of agenesis of the spleen on the pathogenesis of conotruncus anomalies in childhood; an analysis of the heart malformations in the splenic agenesis syndrome, with fourteen new cases. Acta Paediatr Suppl. 1955 Nov;44(Suppl 104):7–110. [PubMed]
  • GILBERT EF, NISHIMURA K, WEDUM BG. Congenital malformations of the heart associated with splenic agenesis; with a report of five cases. Circulation. 1958 Jan;17(1):72–86. [PubMed]
  • Freedom RM. The asplenia syndrome: a review of significant extracardiac structural abnormalities in 29 necropsied patients. J Pediatr. 1972 Dec;81(6):1130–1133. [PubMed]
  • Rose V, Izukawa T, Moës CA. Syndromes of asplenia and polysplenia. A review of cardiac and non-cardiac malformations in 60 cases withspecial reference to diagnosis and prognosis. Br Heart J. 1975 Aug;37(8):840–852. [PMC free article] [PubMed]
  • RUTTENBERG HD, NEUFELD HN, LUCAS RV, Jr, CAREY LS, ADAMS P, Jr, ANDERSON RC, EDWARDS JE. SYNDROME OF CONGENITAL CARDIAC DISEASE WITH ASPLENIA. DISTINCTION FROM OTHER FORMS OF CONGENITAL CYANOTIC CARDIAC DISEASE. Am J Cardiol. 1964 Mar;13:387–406. [PubMed]
  • Marcelletti C, Di Donato R, Nijveld A, Squitieri C, Bulterijs AH, Naeff M, Schuller J, Becker AE. Right and left isomerism: the cardiac surgeon's view. Ann Thorac Surg. 1983 Apr;35(4):400–405. [PubMed]
  • Waldman JD, Rosenthal A, Smith AL, Shurin S, Nadas AS. Sepsis and congenital asplenia. J Pediatr. 1977 Apr;90(4):555–559. [PubMed]
  • Di Donato R, di Carlo D, Squitieri C, Rossi E, Ammirati A, Marino B, Marcelletti C. Palliation of cardiac malformations associated with right isomerism (asplenia syndrome) in infancy. Ann Thorac Surg. 1987 Jul;44(1):35–39. [PubMed]
  • Dickerman JD. Bacterial infection and the asplenic host: a review. J Trauma. 1976 Aug;16(08):662–668. [PubMed]
  • KING H, SHUMACKER HB., Jr Splenic studies. I. Susceptibility to infection after splenectomy performed in infancy. Ann Surg. 1952 Aug;136(2):239–242. [PubMed]
  • LUCAS RV, Jr, KRIVIT W. Overwhelming infection in children following splenectomy. J Pediatr. 1960 Aug;57:185–191. [PubMed]
  • Gopal V, Bisno AL. Fulminant pneumococcal infections in 'normal' asplenic hosts. Arch Intern Med. 1977 Nov;137(11):1526–1530. [PubMed]
  • Padmanabhan J, Risemberg HM, Rowe RD. Howell-Jolly bodies in the peripheral blood of full-term and premature neonates. Johns Hopkins Med J. 1973 Mar;132(3):146–150. [PubMed]
  • Biggar WD, Ramirez RA, Rose V. Congenital asplenia: immunologic assessment and a clinical review of eight surviving patients. Pediatrics. 1981 Apr;67(4):548–551. [PubMed]

Articles from Archives of Disease in Childhood are provided here courtesy of BMJ Group