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CASE REPORT: The patient was a boy born in June, 1990. The proband's father had a history of nonspherocytic hemolytic anemia. The patient was anemic at birth (Hb 11.9 g/dl) and had a hemolytic attack on postnatal day 2. His hemolysis became well compensated, and his second hemolytic episode occurred at three years of age. CLINICAL AND LABORATORY FINDINGS: The patient's mental development had so far been normal and he has no neurological symptoms. His only clinical manifestation has been compensated hemolytic anemia with a hemoglobin concentration of about 11.0 g/dl and a reticulocyte count of 3-6%. He was positive on the Heinz body formation test, and target cells were seen on his peripheral blood smear. The osmotic fragility test yielded slightly increased value. Decreased reduced glutathione (GSH) was observed (4.4 mg/dlRBC) (normal range: 63.9 +/- 9.6), and he also had decreased glutathione synthetase (GS) activity of 0.03 U/gHb (0.38 +/- 0.08 U/gHb). A diagnosis of GS deficiency was made. Decreased glutathione S-transferase (GST) activity was also found (0.57 U/gHb) (normal range: 6.65 +/- 1.20). DISCUSSION: GS deficiency has been reported in about 30 families all over the world. This patient was the first Japanese patient with red cell GS deficiency. 相似文献
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Pea and Broad Bean Pods as a Natural Source of Dietary Fiber: The Impact on Texture and Sensory Properties of Cake
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Lilia Belghith‐Fendri Fatma Chaari Fatma Kallel Soumaya Zouari‐Ellouzi Raoudha Ghorbel Souhail Besbes Semia Ellouz‐Chaabouni Dhouha Ghribi‐Aydi 《Journal of food science》2016,81(10):C2360-C2366
Attention has focused on bakery products such as cake which is one of the most common bakery products consumed by people in the world. Legume by‐products, pea pods (PPs) (Pisum sativum L.) and broad bean pods (BBPs) (Vicia faba L.) mediterranean (Tunisian), has been studied for its high dietary fiber content (PP: 43.87 g/100 g; BBP: 53.01 g/100 g). Protein content was also a considerable component for both by‐products. We investigated the effect of substituted of 5%, 10%, 15%, 20%, 25%, and 30% of PP and BBP flours on the sensory and technological properties in cake. Cakes hardness increased whereas L* and a* color values decreased. The overall acceptability rate showed that a maximum of 15% of PP and BBP flours can be added to prepare acceptable quality cakes. 相似文献
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