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. 1991 Apr;67(786):396–398. doi: 10.1136/pgmj.67.786.396

Primary shunt hyperbilirubinaemia: a variant of the congenital dyserythropoietic anaemias.

A R Bird 1, E Knottenbelt 1, P Jacobs 1, J Maigrot 1
PMCID: PMC2398831  PMID: 2068039

Abstract

A 19 year old Mauritian male presented with episodic nausea, abdominal discomfort and jaundice. Unconjugated hyperbilirubinaemia and erythroid hyperplasia without dyserythropoiesis led to the diagnosis of primary shunt hyperbilirubinaemia. The similarity between congenital dyserythropoietic anaemia and this entity suggests that patients with these lesions can be considered within a single spectrum of disorders, characterized as congenital ineffective erythropoiesis.

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Anselstetter V., Horstmann H. J., Heimpel H. Congenital dyserythropoietic anaemia, types I and II: aberrant pattern of erythrocyte membrane proteins in CDA II, as revealed by two-dimensional polyacrylamide gel electrophoresis. Br J Haematol. 1977 Feb;35(2):209–215. doi: 10.1111/j.1365-2141.1977.tb00577.x. [DOI] [PubMed] [Google Scholar]
  2. Benjamin J. T., Rosse W. F., Daldorf F. G., McMillan C. W. Congential dyserythropoietic anemia--type IV. J Pediatr. 1975 Aug;87(2):210–216. doi: 10.1016/s0022-3476(75)80581-6. [DOI] [PubMed] [Google Scholar]
  3. Bird A. R., Karabus C. D., Hartley P. S. Type IV congenital dyserythropoietic anemia with an unusual response to splenectomy. Am J Pediatr Hematol Oncol. 1985 Summer;7(2):196–199. [PubMed] [Google Scholar]
  4. Cavill I., Worwood M., Jacobs A. Internal regulation of iron absorption. Nature. 1975 Jul 24;256(5515):328–329. doi: 10.1038/256328a0. [DOI] [PubMed] [Google Scholar]
  5. Cazzola M., Barosi G., Bergamaschi G., Dezza L., Palestra P., Polino G., Ramella S., Spriano P., Ascari E. Iron loading in congenital dyserythropoietic anaemias and congenital sideroblastic anaemias. Br J Haematol. 1983 Aug;54(4):649–654. doi: 10.1111/j.1365-2141.1983.tb02145.x. [DOI] [PubMed] [Google Scholar]
  6. Frank D. J., Dusol M., Jr, Schiff E. R. Primary shunt hyperbilirubinemia with secondary iron overload: a case report. Gastroenterology. 1979 Oct;77(4 Pt 1):754–757. [PubMed] [Google Scholar]
  7. Howe R. B., Branda R. F., Douglas S. D., Brunning R. D. Hereditary dyserythropoiesis with abnormal membrane folate transport. Blood. 1979 Nov;54(5):1080–1090. [PubMed] [Google Scholar]
  8. Lewis S. M., Verwilghen R. I. Dyserythropoiesis and dyserythropoietic anemias. Prog Hematol. 1973;8:99–129. [PubMed] [Google Scholar]
  9. Lowenthal R. M., Marsden K. A., Dewar C. L., Thompson G. R. Congenital dyserythropoietic anaemia (CDA) with severe gout, rare Kell phenotype and erythrocyte, granulocyte and platelet membrane reduplication: a new variant of CDA type II. Br J Haematol. 1980 Feb;44(2):211–220. doi: 10.1111/j.1365-2141.1980.tb01203.x. [DOI] [PubMed] [Google Scholar]
  10. Metreau J. M., Yvart J., Dhumeaux D., Berthelot P. Role of bilirubin overproduction in revealing Gilbert's syndrome: is dyserythropoiesis an important factor? Gut. 1978 Sep;19(9):838–843. doi: 10.1136/gut.19.9.838. [DOI] [PMC free article] [PubMed] [Google Scholar]
  11. Samson D., Halliday D., Chanarin I. Congenital dyserythropoietic anaemia: response to splenectomy and quantitation of ineffective erythropoiesis. J Clin Pathol. 1977 Feb;30(2):184–190. doi: 10.1136/jcp.30.2.184. [DOI] [PMC free article] [PubMed] [Google Scholar]
  12. Sansone G. A new type of congenital dyserythropoietic anaemia. Br J Haematol. 1978 Aug;39(4):537–543. doi: 10.1111/j.1365-2141.1978.tb03623.x. [DOI] [PubMed] [Google Scholar]

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