[Haematopoietic stem cell transplantation in pyruvate kinase deficiency: When is it indicated?]
نویسندگان
چکیده
منابع مشابه
Erythrocyte pyruvate kinase deficiency among anemic individuals in Bandar Abbas, Iran
Introduction: In addition to G6PD deficiency, human erythrocyte pyruvate kinase (PK-R) deficiency is one of the most common causes of non-spherocytic hemolytic anemia. Clinical severity of this disorder is not the same in homozygote form of this disease and ranges from mild to chronic and anemia; so it has a wide variation. Severely effected individuals require blood transfusions or splenectomy...
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Background: The frequency of pyruvate kinase (PK) deficiency, an autosomal recessive defect, is approximately 3 per 10,000 individuals in Shiraz and surrounding areas, and is increased due to high consanguinity marriage frequency. The purpose of this study is to obtain data on the frequency and spectrum of gene mutation of PK in newborns, from Shiraz and surrounding areas. Materials and Methods...
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Purpose: To evaluate the long-term outcome of limbal stem cell transplantation (LSCT) for the management of total limbal stem cell deficiency (LSCD) due to chemical burn in Labbafinejad Medical Center (LMC). Methods: Records of patients with a history of severe (i.e. grade III and IV) chemical burns, who were referred to LMC and underwent LSCT, were reviewed and data including demographic chara...
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BACKGROUND Hematopoietic stem cell (HSC) gene therapy has cured immunodeficiencies including X-linked severe combined immunodeficiency (SCID-X1) and adenine deaminase deficiency (ADA). For these immunodeficiencies corrected cells have a selective advantage in vivo, and low numbers of gene-modified cells are sufficient to provide therapeutic benefit. Strategies to efficiently transduce and/or ex...
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Background Stem cell transplantation has become as a novel treatment for end-stage kidney, lung, heart , liver diseases and several hematologic disorders. Improved survival of transplant recipients has raised awareness of post-transplant complications. One of these complications is transplant-related osteoporosis. Methods In this manuscript we review prevention methods for transplant-relat...
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ورودعنوان ژورنال:
- Anales de pediatria
دوره 88 2 شماره
صفحات -
تاریخ انتشار 2018