| congenital hypoplastic anaemia | Congenital nonregenerative, familial hypoplastic, or pure red cell anaemia; erythrogenesis imperfecta; Diamond-Blackfan syndrome; autosomal recessive normocytic normochromic anaemia resulting from congenital hypoplasia of the bone marrow, which is grossly deficient in erythroid precursors while other elements are normal; anaemia is progressive and severe, but leukocyte and platelet counts are normal or slightly reduced; survival of transfused erythrocytes is normal; minor congenital anomalies are found in some patients. Synonym: congenital nonregenerative anaemia, Diamond-Blackfan anaemia, Diamond-Blackfan syndrome, erythrogenesis imperfecta, familial hypoplastic anaemia, pure red cell anaemia. (05 Mar 2000) |
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| hypoplastic | Pertaining to or characterised by hypoplasia. (05 Mar 2000) |
| hypoplastic anaemia | <haematology> A low red blood cell count that results from the underproduction of red blood cells by the bone marrow. This is often secondary to a drug (chemotherapy) side effect. (27 Sep 1997) |
| hypoplastic foetal chondrodystrophy | A developmental error of the epiphyses characterised by severe deformities, epiphyses ossified from several discrete centres and with a stippled appearance, and thickened shafts of the long bones; congenital cataract and mental retardation are often present. There is an autosomal dominant form and an autosomal recessive form. Synonym: chondrodysplasia punctata, chondrodystrophia calcificans congenita, hypoplastic foetal chondrodystrophy, stippled epiphysis. (05 Mar 2000) |
| hypoplastic heart | A small heart, as seen in Addison's disease. (05 Mar 2000) |
| hypoplastic left heart syndrome | <syndrome> Underdevelopment of the left side of the heart characterised by: aortic valve atresia, hypoplastic ascending aorta, hypoplastic/atretic mitral valve, endocardial fibroelastosis most common cause of congestive heart failure in neonate, 25% of cardiac deaths in 1st week of life, prognosis: 100% fatal by 6 weeks haemodynamics: pulmonary venous return is diverted from LA to RA through atrial septal defect, RV supplies pulmonary artery, ductus arteriosus, descending aorta (antegrade flow), aortic arch, ascending aorta, coronary circulation (retrograde flow), leads to RV work overload and congestive heart failure Treatment: Norwood procedure (palliative), transplant (12 Dec 1998) |
| familial hypoplastic anaemia | Congenital nonregenerative, familial hypoplastic, or pure red cell anaemia; erythrogenesis imperfecta; Diamond-Blackfan syndrome; autosomal recessive normocytic normochromic anaemia resulting from congenital hypoplasia of the bone marrow, which is grossly deficient in erythroid precursors while other elements are normal; anaemia is progressive and severe, but leukocyte and platelet counts are normal or slightly reduced; survival of transfused erythrocytes is normal; minor congenital anomalies are found in some patients. Synonym: congenital nonregenerative anaemia, Diamond-Blackfan anaemia, Diamond-Blackfan syndrome, erythrogenesis imperfecta, familial hypoplastic anaemia, pure red cell anaemia. (05 Mar 2000) |
| reduced enamel epithelium | The several layers of the enamel organ remaining on the enamel surface after formation of enamel is completed. Synonym: reduced enamel epithelium. (05 Mar 2000) |
| mottled enamel | Alterations in enamel structure due to excessive fluoride ingestion during tooth formation; varies in appearance from small white opacities to yellow and black spotting. Nanoid enamel, a condition of abnormal thinness of the enamel. Synonym: dwarfed enamel. Whorled enamel, enamel in which the rods assume a spiral or twisting course. (05 Mar 2000) |
| dental enamel | <dentistry> A hard thin translucent layer of calcified substance which envelops and protects the dentin of the crown of the tooth. It is the hardest substance in the body and is almost entirely composed of calcium salts. Under the microscope, it is composed of thin rods (enamel prisms) held together by cementing substance, and surrounded by an enamel sheath. (12 Dec 1998) |
| dental enamel hypoplasia | <dentistry> A form of amelogenesis imperfecta characterised by incomplete formation of the dental enamel and transmitted as an x-linked or autosomal dominant trait. It is also associated with vitamin a, c, or d deficiency, infectious disease, prematurity, birth injury, rh incompatibility, trauma, or local infection. Small grooves, pits, and fissures are seen in mild cases, deep horizontal rows of pits in severe cases, or absence of enamel in extreme cases. (12 Dec 1998) |
| dental enamel permeability | <dentistry> The property of dental enamel to permit passage of light, heat, gases, liquids, metabolites, mineral ions and other substances. It does not include the penetration of the dental enamel by microorganisms. (12 Dec 1998) |
| dental enamel proteins | <dentistry> The proteins that are part of the dental enamel matrix. (12 Dec 1998) |
| dwarfed enamel | nanoid enamel |
| enamel | <dentistry> A hard ceramic which covers the exposed part of your teeth. (08 Jan 1998) |