Ghosal hematodiaphyseal dysplasia, is a rare, autosomal recessive disease, characterized by diaphyseal dysplasia and metaphyseal dysplasia of the long bones and refractory anemia. [2][3][1] It is associated with a deficiency of Thromboxane-A …

2022年10月1日 · Ghosal hematodiaphyseal dysplasia syndrome (GHDD) is a rare authosomal ressesive disorder characterized by increased bone density and regenerative corticosteroid-sensitive anemia.We describe GHDD in an 11-year old Azerbaijani boy with refractory anemia,mild thrombocytopenia and radiological metadiaphyseal dysplasia.The diagnosis was made based o...

Ghosal hematodiaphyseal dysplasia syndrome (GHDD) is a rare disorder characterized by increased bone density (predominantly diaphyseal) and aregenerative corticosteroid-sensitive anemia. Summary.

2023年11月2日 · Ghosal Hematodiaphyseal Dysplasia (GHDD) is a rare autosomal recessive disorder characterized by bone modeling failure, resulting in thickening of bone cortices with predominant diaphyseal involvement and responsive anemia to steroids. Late diagnosis of GHDD in adulthood can lead to significant morbidity and suboptimal response to steroids.

2014年8月30日 · Ghosal hematodiaphyseal dysplasia is a rare autosomal recessive disorder characterized by metadiaphyseal dysplasia of long bones and defective hematopoiesis due to fibrosis or sclerosis of bone marrow. Approximately 15 cases of this entity have been reported in the literature so far.

Ghosal hematodiaphyseal dysplasia is a rare inherited condition characterized by abnormally thick bones and a shortage of red blood cells (anemia). Signs and symptoms of the condition become apparent in early childhood. In affected individuals, the long bones in the arms and legs are unusually dense and wide.

2010年10月25日 · Ghosal hematodiaphyseal dysplasia syndrome (GHDD) is a rare clinical syndrome characterized by increased bone density and a severe, myelophthisic anemia. Few cases have been reported worldwide and there are no detailed descriptions of the associated hematologic abnormalities and long‐term clinical outcomes after treatment.

2023年12月1日 · Ghosal hematodiaphyseal dysplasia (GHDD) is a rare autosomal recessive disorder characterized by corticosteroid-sensitive myelophthisic anemia and increased long bone density. This disease was first described in 1988 by Ghosal et al., who observed five children in early childhood with marked anemia and symmetrical thickening of long bones.

Ghosal hematodiaphyseal dysplasia (GHDD) is an autosomal recessive disorder characterized by increased bone density with predominant diaphyseal involvement and aregenerative corticosteroid-sensitive anemia (summary by Genevieve et al., 2008). [from OMIM]

Ghosal hematodiaphyseal dysplasia (GHDD) is an autosomal recessive disorder characterized by increased bone density with predominant diaphyseal involvement and aregenerative corticosteroid-sensitive anemia (summary by Genevieve et al., 2008). [from OMIM] 23 tests are in the database for this condition.