Hypohidrotic Ectodermal Dysplasia Associated with Glucose-6-Phosphate Dehydrogenase Deficiency

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Hypohidrotic Ectodermal Dysplasia Associated with Glucose-6-Phosphate Dehydrogenase Deficiency

Hypohidrotic ectodermal dysplasia (HED) is a syndrome characterized by hypodontia, hypotrichosis, and partial or total ecrine sweat gland deficiency. The most prevalent form of HED is inherited as an X linked pattern. Glucose-6-phosphate dehydrogenase (G-6-PD) deficiency is an X-linked recessive disease, which leads to hemolytic anemia and jaundice. It is expressed in males, while heterozygous ...

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Glucose-6-Phosphate Dehydrogenase Deficiency Associated with Thalassemia and Sickle Cell Anemia in an Iranian Family

Sickle cell-thalassemia associated with glucose- 6-phosphate dehydrogenese deficiency is re­ported in an Iranian family. The father had sickle trait and G.6.P.D. deficiency, the mother minor thalassemia, one of the sons siclc:le ceH-thailasemiia., the oth-er sickle cell trait; the daughter had sickle cell-thalassemia and was carrier of G.6.P.D. deficiellcy. 

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Glucose-6-Phosphate Dehydrogenase Deficiency and Neonatal Hyperbilirubinemia

Background: Jaundice is affecting over 60-80 percent of neonates in the first week of life. Glucose-6-phosphate dehydrogenase (G6PD) deficiency, which is an important cause of pathologic hyperbilirubinemia, can lead to hemolytic anemia, jaundice and kernicterus. The present study was performed to determine the prevalence of G6PD deficiency among icteric neonates in Shirvan, Iran. Methods: This...

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Hypohidrotic ectodermal dysplasia.

X linked hypohidrotic ectodermal dysplasia was studied in the dentition of both affected males and carrier females. Hypodontia was more severe in males than females and there were differences in the pattern of tooth absence between the sexes. Abnormal crown form, with the maximum diameter of the teeth being apically displaced, was noted particulady in the anterior teeth. Taurodontism was common...

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[glucose-6-phosphate Dehydrogenase Deficiency].

LABORATORY DATA biochemical markers metabolic defect chromosomal assignment chromosome X localization chromosome Xq localization chromosome Xq28 localization gene, structural-functional anomalies G6PD (G6PD1) glucose-6-phosphate dehydrogenase, gene chr.Xq28 gene analysis-DNA analysis myelo-erythropoietic disorders glucose-6-phosphate dehydrogenase deficiency haemolytic anaemia isolated hematopo...

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ژورنال

عنوان ژورنال: Annals of Dermatology

سال: 2011

ISSN: 1013-9087

DOI: 10.5021/ad.2011.23.s1.s8