Hereditary spherocytosis historical perspective
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Overview
- Towards the end of the nineteenth century Vanlair and Masius described the case of a young woman who developed icterus, recurrent attacks of left upper quadrant abdominal pain and splenomegaly shortly after giving birth. The stools were not light coloured, but rather deeply pigmented. The patient's mother and sister were also icteric, and the sister's spleen was enlarged.
Historical Perspective
- Hereditary spherocytosis was first described in 1871 by Vanlair and Masius, where they described chronically icteric patients who had no bile in the urine, no evidence of liver disease and often splenomegaly and family history of jaundice.[1]
- It is the commonest cause of inherited chronic hemolysis in the northern europe and north america.[2]
- With remarkable discernment, Vanlair and Masius stated, ‘The jaundice of our patient appears to be a peculiar type of icterus. The fact that the patient’s mother and sister had a slight jaundice and that the sister had an enlarged spleen may indicate that this condition is one disease entity.'.[3]
References
- ↑ Sayeeda Huq, Mark A. C. Pietroni, Hafizur Rahman & Mohammad Tariqul Alam (2010). "Hereditary spherocytosis". Journal of health, population, and nutrition. 28 (1): 107–109. PMID 20214092. Unknown parameter
|month=ignored (help) - ↑ Sayeeda Huq, Mark A. C. Pietroni, Hafizur Rahman & Mohammad Tariqul Alam (2010). "Hereditary spherocytosis". Journal of health, population, and nutrition. 28 (1): 107–109. PMID 20214092. Unknown parameter
|month=ignored (help) - ↑ Packman, Charles H. (2001). "The spherocytic Haemolytic Anaemias". British Journal of Haematology. 112 (4): 888–899. doi:10.1046/j.1365-2141.2001.02440.x. ISSN 0007-1048.