Biomaterial Database

Hemimegalencephaly with hemihypertrophy (Klippel-Trénaunay-Weber syndrome). 1983

O Matsubara, and M Tanaka, and T Ida, and R Okeda

A 17-year-old Japanese girl had right hemihypertrophy, vascular nevus of the right side of the body, right hemimegalencephaly, multiple haemangiomatosis, varicosis and chronic proliferative glomerulonephritis. The hypertrophied side of the cerebrum showed no malformations. Quantitative studies showed no significant differences between the two cerebral hemispheres in size, form, density or DNA content of nerve and glial cells. The hemimegalencephaly seemed to be due to an increase in the absolute number of nerve and glial cells in the ipsilateral cerebral hemisphere. Eight cases of hemihypertrophy with hemimegalencephaly have been reported to date, in all of them hemihypertrophy and hemimegalencephaly were on the same side. This condition seems to be due to unilateral overproduction of neuroblasts and glioblasts or hindrance of the normal loss of excess neurons, which may be induced by hemihypertrophy of the mesenchyme surrounding the CNS.

UI	MeSH Term	Description	Entries
D007715	Klippel-Trenaunay-Weber Syndrome	A congenital disorder that is characterized by a triad of capillary malformations (HEMANGIOMA), venous malformations (ARTERIOVENOUS FISTULA), and soft tissue or bony hypertrophy of the limb. This syndrome is caused by mutations in the VG5Q gene which encodes a strong angiogenesis stimulator.	Klippel-Trenaunay Disease,Angio-Osteohypertrophy Syndrome,Angioosteohypertrophy Syndrome,Congenital Dysplastic Angiopathy,KTW Syndrome,Klippel Trenaunay Syndrome,Klippel-Trenaunay Syndrome,Klippel-Trénaunay-Weber Syndrome,Angio Osteohypertrophy Syndrome,Angio-Osteohypertrophy Syndromes,Angioosteohypertrophy Syndromes,Angiopathies, Congenital Dysplastic,Angiopathy, Congenital Dysplastic,Congenital Dysplastic Angiopathies,Disease, Klippel-Trenaunay,Dysplastic Angiopathies, Congenital,Dysplastic Angiopathy, Congenital,KTW Syndromes,Klippel Trenaunay Disease,Klippel Trenaunay Weber Syndrome,Klippel Trénaunay Weber Syndrome,Klippel-Trenaunay Syndromes,Syndrome, Angio-Osteohypertrophy,Syndrome, Angioosteohypertrophy,Syndrome, KTW,Syndrome, Klippel Trenaunay,Syndrome, Klippel-Trenaunay,Syndrome, Klippel-Trenaunay-Weber,Syndrome, Klippel-Trénaunay-Weber,Syndromes, Angio-Osteohypertrophy,Syndromes, Angioosteohypertrophy,Syndromes, KTW,Syndromes, Klippel-Trenaunay
D009457	Neuroglia	The non-neuronal cells of the nervous system. They not only provide physical support, but also respond to injury, regulate the ionic and chemical composition of the extracellular milieu, participate in the BLOOD-BRAIN BARRIER and BLOOD-RETINAL BARRIER, form the myelin insulation of nervous pathways, guide neuronal migration during development, and exchange metabolites with neurons. Neuroglia have high-affinity transmitter uptake systems, voltage-dependent and transmitter-gated ion channels, and can release transmitters, but their role in signaling (as in many other functions) is unclear.	Bergmann Glia,Bergmann Glia Cells,Bergmann Glial Cells,Glia,Glia Cells,Satellite Glia,Satellite Glia Cells,Satellite Glial Cells,Glial Cells,Neuroglial Cells,Bergmann Glia Cell,Bergmann Glial Cell,Cell, Bergmann Glia,Cell, Bergmann Glial,Cell, Glia,Cell, Glial,Cell, Neuroglial,Cell, Satellite Glia,Cell, Satellite Glial,Glia Cell,Glia Cell, Bergmann,Glia Cell, Satellite,Glia, Bergmann,Glia, Satellite,Glial Cell,Glial Cell, Bergmann,Glial Cell, Satellite,Glias,Neuroglial Cell,Neuroglias,Satellite Glia Cell,Satellite Glial Cell,Satellite Glias
D009474	Neurons	The basic cellular units of nervous tissue. Each neuron consists of a body, an axon, and dendrites. Their purpose is to receive, conduct, and transmit impulses in the NERVOUS SYSTEM.	Nerve Cells,Cell, Nerve,Cells, Nerve,Nerve Cell,Neuron
D001921	Brain	The part of CENTRAL NERVOUS SYSTEM that is contained within the skull (CRANIUM). Arising from the NEURAL TUBE, the embryonic brain is comprised of three major parts including PROSENCEPHALON (the forebrain); MESENCEPHALON (the midbrain); and RHOMBENCEPHALON (the hindbrain). The developed brain consists of CEREBRUM; CEREBELLUM; and other structures in the BRAIN STEM.	Encephalon
D002452	Cell Count	The number of CELLS of a specific kind, usually measured per unit volume or area of sample.	Cell Density,Cell Number,Cell Counts,Cell Densities,Cell Numbers,Count, Cell,Counts, Cell,Densities, Cell,Density, Cell,Number, Cell,Numbers, Cell
D005260	Female		Females
D005921	Glomerulonephritis	Inflammation of the renal glomeruli (KIDNEY GLOMERULUS) that can be classified by the type of glomerular injuries including antibody deposition, complement activation, cellular proliferation, and glomerulosclerosis. These structural and functional abnormalities usually lead to HEMATURIA; PROTEINURIA; HYPERTENSION; and RENAL INSUFFICIENCY.	Bright Disease,Kidney Scarring,Glomerulonephritides,Scarring, Kidney
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000293	Adolescent	A person 13 to 18 years of age.	Adolescence,Youth,Adolescents,Adolescents, Female,Adolescents, Male,Teenagers,Teens,Adolescent, Female,Adolescent, Male,Female Adolescent,Female Adolescents,Male Adolescent,Male Adolescents,Teen,Teenager,Youths
D000798	Angiomatosis	A condition with multiple tumor-like lesions caused either by congenital or developmental malformations of BLOOD VESSELS, or reactive vascular proliferations, such as in bacillary angiomatosis. Angiomatosis is considered non-neoplastic.	Angiomatoses