CHRONIC HEMOLYTIC ANEMIA DUE TO GLUCOSE-6-PHOSPHATE DEHYDROGENASE DEFICIENCY OR GLUTATHIONE SYNTHETASE DEFICIENCY: THE ROLE OF VITAMIN E IN ITS TREATMENT

1982 ◽  
Vol 393 (1 Vitamin E) ◽  
pp. 348-360 ◽  
Author(s):  
Laurence M. Corash ◽  
Michael Sheetz ◽  
John G. Bieri ◽  
Christos Bartsocas ◽  
Shimon Moses ◽  
...  
Blood ◽  
1983 ◽  
Vol 62 (4) ◽  
pp. 754-757 ◽  
Author(s):  
JT Prchal ◽  
WM Crist ◽  
M Roper ◽  
VP Wellner

Abstract The clinical and laboratory features of a 3-mo-old black male infant with glutathione (GSH) synthetase deficiency of the generalized type was evaluated. Partial albinism, brisk hemolytic anemia, recurrent febrile episodes, and mental retardation were noted. Also, severe recurrent metabolic acidosis and marked oxoprolinemia and oxoprolinuria were found in the proband but not in his first-degree relatives. The relationship of these disease manifestations to the underlying metabolic defect is discussed.


Blood ◽  
1983 ◽  
Vol 62 (4) ◽  
pp. 754-757
Author(s):  
JT Prchal ◽  
WM Crist ◽  
M Roper ◽  
VP Wellner

The clinical and laboratory features of a 3-mo-old black male infant with glutathione (GSH) synthetase deficiency of the generalized type was evaluated. Partial albinism, brisk hemolytic anemia, recurrent febrile episodes, and mental retardation were noted. Also, severe recurrent metabolic acidosis and marked oxoprolinemia and oxoprolinuria were found in the proband but not in his first-degree relatives. The relationship of these disease manifestations to the underlying metabolic defect is discussed.


PEDIATRICS ◽  
1980 ◽  
Vol 66 (2) ◽  
pp. 330-331
Author(s):  
Sinasi Ozoylu

I would like to add my short comments to the concise commentary of Phelps1 on vitamin E. In addition to the mentioned several conditions related to vitamin E metabolism, probable vitamin E deficiency in the pathogenesis of thrombocytosis in Caffey's disease2 should be remembered by the pediatricians. We have seen such a case recently.3 Improvement of erythrocyte survival in chronic hemolyzing G-6 PD and glutathione synthetase deficiency with high doses of vitamin E seems to be promising approach in the treatment of these patients.4


2014 ◽  
Vol 34 (1) ◽  
pp. 18-20 ◽  
Author(s):  
Salma Ben Ameur ◽  
Hajer Aloulou ◽  
Fehmi Nasrallah ◽  
Thouraya Kamoun ◽  
Naziha Kaabachi ◽  
...  

Author(s):  
Nayma Sultana ◽  
Noorzahan Begum ◽  
Shelina Begum ◽  
Sultana Ferdousi ◽  
Taskina Ali

Vitamin E works within the cell membrane as a biological antioxidant and may prevent premature destruction of RBC in Glucose 6-phosphate dehydrogenase (G6PD) deficient hemolytic anemia. Changes in some of the hematological variables like hemoglobin (Hb) concentration, total count (TC) of RBC, packed cell volume (PCV) and reticulocyte counts may occur due to hemolysis of RBC in G6PD deficiency In the present study the role of vitamin E supplementation on these changes were observed in reducing chronic hemolysis in anemic patients with glucose 6-phosphate dehydrogenase (G6PD) deficiency For this, a total number of 102 subjects with age ranged from 5 to 40 years of both sexes were included in the study Among them 68 were G6PD enzyme deficient patients, of whom 34 were in supplemented group (experimental group) and 34 were nonsupplemented group (control group). The supplemented group received vitamin E supplementation for 60 consecutive days at a dose of 800 IU/day for adult and 400 IU/day for children 5. 12 years (in a divided dose i,e. 4 times daily). Age and sex matched 34 apparently healthy subjects with normal blood G6PD level were taken to observe the baseline data (healthy control) and also for comparison. All the G6PD deficient patients were selected from Out Patient Department (OPD) of hematology, Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh during the period of July 2005 to June 2006 and all the healthy subjects were selected from personal contact. Blood G6PD level, Hb%, TC of RBC, PCV, reticulocyte count and serum bilirubin level of all subjects were measured by standard laboratory techniques. All the parameters were measured on day 1(one) of their 1st visit forall the groups and also were on day 60 in deficient group. Data were compared among the different groups, also in supplemented group just before and after supplementation. Analysis of data was done by appropriate statistical method. Mean blood Hb%, TC of RBC and PCV were significantly lower but reticulocyte count and serum bilirubin levels were significantly higher in patients suffering from hemolytic anemia due to G6PD deficiency in comparison to those of the healthy control. After supplementation with vitamin E (i.e. on day-60) Hb concentration, total count of RBC, PCV were significantly increased whereas, reticulocyte count and serum bilirubin levels were significantly decreased towards those of healthy control in supplemented group of patients in comparison to those of their pre-supplemented (day-1) and non-supplemented groups both on day-1 and day-60. Therefore, from this study it may be concluded that, deterioration of some of the hematological parameters occur in G6PD deficient hemolytic anemic patients, improvement of which occur following vitamin E supplementation, which clearly indicates the role of this antioxidant vitamin in reducing the rate of hemolysis in this group of patients. So, vitamin E supplementation can be considered along with other drugs to treat this group of patients. DOI: 10.3329/bjpp.v22i1.3563 Bangladesh J Physiol Pharmacol 2006; 22(1/2) : 12-17


1979 ◽  
Vol 301 (17) ◽  
pp. 901-905 ◽  
Author(s):  
Laurence A. Boxer ◽  
Janet M. Oliver ◽  
Stephen P. Spielberg ◽  
John M. Allen ◽  
Joseph D. Schulman

PEDIATRICS ◽  
2016 ◽  
Vol 138 (3) ◽  
pp. e20154324-e20154324 ◽  
Author(s):  
I. Signolet ◽  
R. Chenouard ◽  
F. Oca ◽  
M. Barth ◽  
P. Reynier ◽  
...  

1977 ◽  
Vol 145 (4) ◽  
pp. 983-998 ◽  
Author(s):  
S J Klebanoff

Estradiol binds covalently to normal leukocytes during phagocytosis. The binding involves three cell types, neutrophils, eosinophils, and monocytes and at least two reaction mechanisms, one involving the peroxidase of neutrophils and monocytes (myeloperoxidase [MPO]) and possibly the eosinophil peroxidase, and the second involving catalase. Binding is markedly reduced when leukocytes from patients with chronic granulomatous disease (CGD), severe leukocytic glucose 6-phosphate dehydrogenase deficiency, and familial lipochrome histiocytosis are employed and two populations of neutrophils, one which binds estradiol and one which does not, can be demonstrated in the blood of a CGD carrier. Leukocytes from patients with hereditary MPO deficiency also bind estradiol poorly although the defect is not as severe as in CGD. These findings are discussed in relation to the inactivation of estrogens during infection and the possible role of estrogens in neutrophil function.


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