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The Reticuloendothelial System

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Keeling’s Fetal and Neonatal Pathology

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Abstract

The development of the spleen and the thymus is described. Developmental abnormalities of the spleen include polyasplenia, a term that encompasses the spectrum of disorders of asplenia and polysplenia. Splenogonadal fusion and splenopancreatic fusion are two uncommon anomalies that are associated with other anomalies. Developmental abnormalities of the thymus include ectopia, aplasia, and hypoplasia, the last two of which may be related to DiGeorge syndrome, itself mainly due to deletion of chromosome 22q11. Intrauterine stress leads to involution of the thymus.

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References

  1. Koss M, Bolze A, Brendolan A, Saggese M, Capellini TD, Bojilova E, et al. Congenital asplenia in mice and humans with mutations in a PBX/NKX2-5/P15 MODULE. Dev Cell. 2012;22:913–26.

    Article  CAS  Google Scholar 

  2. Gruenwald P, Minh H. Evaluation of body and organ weights in perinatal pathology. I. Normal standards derived from autopsies. Am J Clin Pathol. 1960;34:247–53.

    Article  CAS  Google Scholar 

  3. Opitz JM. Editorial comment on the paper by de la monte and hutchins on familial polyasplenia. Am J Med Genet. 1985;21:175–6.

    Article  Google Scholar 

  4. 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. 1955;44:1–110.

    Article  Google Scholar 

  5. Mahlaoui N, Minard-Colin V, Picard C, Bolze A, Ku CL, Tournilhac O, Gilbert-Dussardier B, Pautard B, Durand P, Devictor D, Lachassinne E, Guillois B, Morin M, Gouraud F, Valensi F, Fischer A, Puel A, Abel L, Bonnet D, Casanova JL. Isolated congenital asplenia: a French nationwide retrospective survey of 20 cases. J Pediatr. 2011;158:142–8.

    Article  Google Scholar 

  6. Bolze A, Mahlaoui N, Byun M, Turner B, Trede N, Ellis SR, Abhyankar A, Itan Y, Patin E, Brebner S, Sackstein P, Puel A, Picard C, Abel L, Quintana-Murci L, Faust SN, Williams AP, Baretto R, Duddridge M, Kini U, Pollard AJ, Gaud C, Frange P, Orbach D, Emile JF, Stephan JL, Sorensen R, Plebani A, Hammarstrom L, Conley ME, Selleri L, Casanova JL. Ribosomal protein sa haploinsufficiency in humans with isolated congenital asplenia. Science. 2013;340:976–8.

    Article  CAS  Google Scholar 

  7. Strouse PJ, Haller JO, Berdon WE, Rosovsky MA, Bellah RD, Barr Jr M. Horseshoe adrenal gland in association with asplenia: presentation of six new cases and review of the literature. Pediatr Radiol. 2002;32:778–82.

    Article  Google Scholar 

  8. Ticho BS, Goldstein AM, Van Praagh R. Extracardiac anomalies in the heterotaxy syndromes with focus on anomalies of midline-associated structures. Am J Cardiol. 2000;85:729–34.

    Article  CAS  Google Scholar 

  9. Noack F, Sayk F, Ressel A, Berg C, Gembruch U, Reusche E. Ivemark syndrome with agenesis of the corpus callosum: a case report with a review of the literature. Prenat Diagn. 2002;22:1011–5.

    Article  CAS  Google Scholar 

  10. McPherson F, Frias JL, Spicer D, Opitz JM, Gilbert-Barness EF. Splenogonadal fusion-limb defect “syndrome” and associated malformations. Am J Med Genet A. 2003;120:518–22.

    Article  Google Scholar 

  11. Speare R, Roberts J, Cohen M, Wales J. Splenogonadal fusion and sex reversal. J Pediatr Endocrinol Metab. 2012;25:541–2.

    Google Scholar 

  12. Gomi K, Sato Y, Tanaka M, Ijiri R, Kato K, Aoki I, et al. Specificity of splenopancreatic field abnormality in trisomy 13 syndrome: macroscopic and histological analysis in 21 autopsy cases. Pathol Int. 2009;59:147–51.

    Article  Google Scholar 

  13. Lee CH. Intestinal obstruction caused by infarcted splenic hemangioma with renal vein thrombosis in a newborn: a case report. Int Surg. 2002;87:157–9.

    Google Scholar 

  14. Kahwash SB, Fung B, Savelli S, Bleesing JJ, Qualman SJ. Autoimmune lymphoproliferative syndrome (ALPS): a case with congenital onset. Pediatr Dev Pathol. 2007;10:315–9.

    Google Scholar 

  15. Felgentreff K, Schupp W, Otten JE, Rückauer KD, Uhl M, Jüttner E, et al. Inspiratory stridor and dysphagia in two newborn infants caused by ectopic thymus tissue. Eur J Pediatr. 2009;168:1141–5.

    Article  Google Scholar 

  16. Burn J. Closing time for CATCH22. J Med Genet. 1999;36:737–8.

    Article  CAS  Google Scholar 

  17. Botto LD, May K, Fernhoff PM, Correa A, Coleman K, Rasmussen SA, Merritt RK, O’Leary LA, Wong LY, Elixson EM, Mahle WT, Campbell RM. A population-based study of the 22q11.2 deletion: phenotype, incidence, and contribution to major birth defects in the population. Pediatrics. 2003;112:101–7.

    Article  Google Scholar 

  18. Oskarsdottir S, Vujic M, Fasth A. Incidence and prevalence of the 22q11 deletion syndrome: a population-based study in western Sweden. Arch Dis Child. 2004;89:148–51.

    Article  CAS  Google Scholar 

  19. Goldmuntz E. DiGeorge syndrome: new insights. Clin Perinatol. 2005;32:963–78.

    Article  Google Scholar 

  20. Yagi H, Furutani Y, Hamada H, Sasaki T, Asakawa S, Minoshima S, et al. Role of tbx1 in human del22q11.2 syndrome. Lancet. 2003;362:1366–73.

    Article  CAS  Google Scholar 

  21. van Baarlen J, Schuurmann H, Reitsma R, Huber J. Acute thymus involution during infancy and childhood: immunohistology of the thymus and peripheral lymphoid tissues after acute illness. Pediatr Pathol. 1989;9:261–75.

    Google Scholar 

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Author information

Authors and Affiliations

  1. Department of Pathology and Department of Obstetrics and Gynaecology, University of Adelaide, North Adelaide, SA, Australia

    T. Yee Khong MBChB, MSc, MD, FRCPath, FRCPA

  2. Department of Histopathology, Women’s & Children’s Hospital, North Adelaide, SA, Australia

    T. Yee Khong MBChB, MSc, MD, FRCPath, FRCPA

Authors
  1. T. Yee Khong MBChB, MSc, MD, FRCPath, FRCPA
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Correspondence to T. Yee Khong MBChB, MSc, MD, FRCPath, FRCPA .

Editor information

Editors and Affiliations

  1. Department of Pathology Department of Obstetrics and Gynaecology, University of Adelaide, North Adelaide, South Australia, Australia

    T. Yee Khong

  2. Department of Histopathology, Women’s & Children’s Hospital, North Adelaide, South Australia, Australia

    T. Yee Khong

  3. Department of Histopathology, University Hospitals of Leicester NHS Trust Leicester Royal Infirmary, Leicester, UK

    Roger D. G. Malcomson

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© 2015 Springer International Publishing

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Khong, T.Y. (2015). The Reticuloendothelial System. In: Khong, T.Y., Malcomson, R.D.G. (eds) Keeling’s Fetal and Neonatal Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-19207-9_26

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  • DOI: https://doi.org/10.1007/978-3-319-19207-9_26

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-19206-2

  • Online ISBN: 978-3-319-19207-9

  • eBook Packages: MedicineMedicine (R0)

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