{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,30]],"date-time":"2026-04-30T09:49:06Z","timestamp":1777542546753,"version":"3.51.4"},"reference-count":11,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2017,9,14]],"date-time":"2017-09-14T00:00:00Z","timestamp":1505347200000},"content-version":"vor","delay-in-days":19949,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Australasian Annals of Medicine"],"published-print":{"date-parts":[[1963,2]]},"abstract":"<jats:title>SUMMARY<\/jats:title><jats:p>The integrity and normal survival of the red cell are dependent on ATP (energy) production from glycolysis. Destruction at the end of normal life span is considered due to metabolic exhaustion.<\/jats:p><jats:p>In the congenital h\u00e6molytic an\u00e6mias defects of glycolysis are present. The defects demonstrated vary in the different disorders. The premature destruction of the cells is related, at least in part, to the defective energy production.<\/jats:p><jats:p>In non\u2010spherocytic congenital h\u00e6molytic an\u00e6mia Type II, there is defective glycolysis and ATP production due to a specific enzyme defect, namely, pyruvate kinase deficiency.<\/jats:p><jats:p>Non\u2010spherocytic congenital h\u00e6molytic an\u00e6mia Type I appears to be biochemically heterogeneous; glucose\u20106 phosphate dehydrogenase (G\u20106PD) is deficient in some cases and normal in others. Deficiency of ATP has been demonstrated in our cases, but the mechanism of the defect is unknown.<\/jats:p><jats:p>In hereditary spherocytosis two abnormalities exist, of shape and of metabolism. The abnormality of glycolytic metabolism is obvious <jats:italic>in vitro<\/jats:italic> only on incubation. It is possible that passage through the spleen makes this latent defect overt and \u201cconditions\u201d the cells for their premature destruction.<\/jats:p><jats:p>In hereditary elliptocytosis, preliminary evidence suggests that the defect is similar in some respects to that of hereditary spherocytosis.<\/jats:p>","DOI":"10.1111\/imj.1963.12.1.6","type":"journal-article","created":{"date-parts":[[2017,9,14]],"date-time":"2017-09-14T14:31:50Z","timestamp":1505399510000},"page":"6-10","source":"Crossref","is-referenced-by-count":10,"title":["RED\u2010CELL METABOLISM IN CONGENITAL H\u00c6MOLYTIC AN\u00c6MIAS"],"prefix":"10.1111","volume":"12","author":[{"given":"G. C.","family":"DE GRUCHY","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2017,9,14]]},"reference":[{"key":"e_1_2_1_2_1","doi-asserted-by":"publisher","DOI":"10.1111\/imj.1960.9.3.237"},{"key":"e_1_2_1_3_1","doi-asserted-by":"crossref","first-page":"1371","DOI":"10.1182\/blood.V16.4.1371.1371","article-title":"Nonspherocytic Congenital Hmolytic Anaemia","volume":"16","author":"DE GRUCHY G. C.","year":"1960","journal-title":"Blood"},{"key":"e_1_2_1_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.1962.tb06509.x"},{"key":"e_1_2_1_5_1","unstructured":"LODER P. B. andDE GRUCHY G. C.(1962) unpublished observations."},{"key":"e_1_2_1_6_1","first-page":"286","article-title":"Erythrocyte Metabolism. II. Glucose Metabolism and Pathways","volume":"55","author":"MURPHY J. R.","year":"1960","journal-title":"J. Lab. clin. Med."},{"key":"e_1_2_1_7_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.1955.tb05495.x"},{"key":"e_1_2_1_8_1","first-page":"1019","article-title":"Abnormalities of In Vitro Behaviour of Structural Lipids of Red Blood Cells from Patients with Hereditary Spherocytosis","volume":"39","author":"REED C. F.","year":"1960","journal-title":"J. clin. Invest."},{"key":"e_1_2_1_9_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.1961.tb00343.x"},{"key":"e_1_2_1_10_1","doi-asserted-by":"crossref","first-page":"414","DOI":"10.1182\/blood.V9.5.414.414","article-title":"Autohaemolysis and Other Changes Resulting from the Incubation In Vitro of Red Cells from Patients with Congenital Hmolytic An\u00e6mias","volume":"9","author":"SELWYN J. G.","year":"1954","journal-title":"Blood"},{"key":"e_1_2_1_11_1","doi-asserted-by":"crossref","first-page":"267","DOI":"10.1182\/blood.V19.3.267.267","article-title":"Pyruvate Kinase (PK) Deficiency Hereditary Nonspherocytic H\u00e6molytic An\u00e6mia","volume":"19","author":"TANAKA K. R.","year":"1962","journal-title":"Blood"},{"key":"e_1_2_1_12_1","doi-asserted-by":"publisher","DOI":"10.1001\/archinte.1962.03620140081013"}],"container-title":["Australasian Annals of Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fimj.1963.12.1.6","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/imj.1963.12.1.6","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,10]],"date-time":"2023-10-10T16:10:57Z","timestamp":1696954257000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/imj.1963.12.1.6"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1963,2]]},"references-count":11,"journal-issue":{"issue":"1","published-print":{"date-parts":[[1963,2]]}},"alternative-id":["10.1111\/imj.1963.12.1.6"],"URL":"https:\/\/doi.org\/10.1111\/imj.1963.12.1.6","archive":["Portico"],"relation":{},"ISSN":["0571-9283"],"issn-type":[{"value":"0571-9283","type":"print"}],"subject":[],"published":{"date-parts":[[1963,2]]}}}