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X-linked lymphoproliferative disease; Other names: Duncan disease, Purtilo syndrome: Specialty: Hematology : Symptoms: Reduced resistance to the Epstein-Barr virus (EBV), leading to infectious mononucleosis, hemophagocytic lymphohistiocytosis (HLH), dysgammaglobulinemia, non-Hodgkin lymphoma, aplastic anemia, vasculitis, chronic gastritis, skin lesions
Cell growth refers to an increase in the total mass of a cell, including both cytoplasmic, nuclear and organelle volume. [1] Cell growth occurs when the overall rate of cellular biosynthesis (production of biomolecules or anabolism) is greater than the overall rate of cellular degradation (the destruction of biomolecules via the proteasome, lysosome or autophagy, or catabolism).
Conventionally, a leukocytosis exceeding 50,000 WBC/mm 3 with a significant increase in early neutrophil precursors is referred to as a leukemoid reaction. [2] The peripheral blood smear may show myelocytes, metamyelocytes, promyelocytes, and rarely myeloblasts; however, there is a mixture of early mature neutrophil precursors, in contrast to the immature forms typically seen in acute leukemia.
Growth factors are usually secreted by either specialized or non-specialized cells to induce cell proliferation in themselves, nearby cells, or distant cells. An oncogene may cause a cell to secrete growth factors even though it does not normally do so.
However, defective cell division is unlikely to be the main defect leading to progeria, particularly because children develop normally without any signs of disease until about one year of age. Farnesylated prelamin A variants also lead to defective DNA repair, which may play a role in the development of progeria. [ 22 ]
It is the result of increased cell mitosis or division (also referred to as cell proliferation). The two types of physiologic hyperplasia are compensatory and hormonal. Compensatory hyperplasia permits tissue and organ regeneration. It is common in epithelial cells of the epidermis and intestine, liver hepatocytes, bone marrow cells, and ...
Latitudinal and longitudinal growth may be affected. [1] [2] [3] Nevertheless, the musculoskeletal features are central to the diagnosis of some syndromes such as Proteus syndrome. [2] The time of presentation of children with overgrowth syndromes is an important contributor to the differential diagnosis.
Laron syndrome (LS), also known as growth hormone insensitivity or growth hormone receptor deficiency (GHRD), is an autosomal recessive disorder characterized by a lack of insulin-like growth factor 1 (IGF-1; somatomedin-C) production in response to growth hormone (GH; hGH; somatotropin). [6]