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Three cases of hereditary nonspherocytic hemolytic anemia associated with red blood cell glutathione deficiency
| Content Provider | Scilit |
|---|---|
| Author | Hirono, A. Iyori, H. Sekine, I. Ueyama, J. Chiba, H. Kanno, H. Fujii, H. Miwa, S. |
| Copyright Year | 1996 |
| Description | Three unrelated Japanese patients with chronic nonspherocytic hemolytic anemia wer found to have marked deficiency of red blood cell (RBC) reduced glutathoine (GSH) (4.4%, 13.1%, and 6.9% of normal, respectively). A panel of RBC enzyme assays showed that one patient had decreased glutathione synthetase activity and the other two were moderately deficient in gamma-glutamylcystine synthetase. Some family members of each patient showed mild deficiency of the respective enzymes. RBCs of these patients also showed a decreased level of glutathione-S-transferase as in previously described GSH-deficient cases. Hemolytic anemia was their only manifestation, and neither 5- oxoprolinemia nor 5-oxoprolinuria, which are usually associated with to generalized type of glutathione synthetase deficiency, was noted in our patients. |
| Related Links | https://ashpublications.org/blood/article-pdf/87/5/2071/623582/2071.pdf |
| Ending Page | 2074 |
| Page Count | 4 |
| Starting Page | 2071 |
| DOI | 10.1182/blood.v87.5.2071.2071 |
| Journal | Blood |
| Issue Number | 5 |
| Volume Number | 87 |
| Language | English |
| Publisher | American Society of Hematology |
| Publisher Date | 1996-03-01 |
| Access Restriction | Open |
| Subject Keyword | Medical Laboratory Technology Glutathione Deficiency Gsh Rbc Hemolytic Anemia Deficient Synthetase Nonspherocytic Journal: Blood (Vol- 83, Issue- 5) |
| Content Type | Text |
| Resource Type | Article |
