lipofuscinosis


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lipofuscinosis

 [lip″o-fu″sin-o´sis]
any disorder due to abnormal storage of lipofuscins.
neuronal ceroid-lipofuscinosis any of several genetic lipidoses characterized by progressive neurodegeneration, loss of vision, and a fatal course; included are janský-bielschowsky disease, vogt-spielmeyer disease, and kufs' disease. Formerly known as amaurotic familial idiocy.

lip·o·fus·ci·no·sis

(lip'ō-fyūs'i-nō'sis),
Abnormal storage of any one of a group of fatty pigments; best viewed using fluorescent microscopy.

lipofuscinosis

/lipo·fus·cin·o·sis/ (-fu″sin-o´sis) any disorder due to abnormal storage of lipofuscins.
neuronal ceroid-lipofuscinosis  any of several genetic lipidoses characterized by progressive neurodegeneration, loss of vision, and a fatal course; included are Janský-Bielschowsky disease, Vogt-Spielmeyer disease, and Kufs' disease.

lip·o·fus·ci·no·sis

(lip'ō-fyūs'i-nō'sis)
Abnormal storage of any one of a group of fatty pigments.

lip·o·fus·ci·no·sis

(lip'ō-fyūs'i-nō'sis)
Abnormal storage of any one of a group of fatty pigments.

lipofuscinosis

any disorder due to abnormal storage of lipofuscins.

ceroid lipofuscinosis
an inherited disease in sheep, cattle, dogs and cats, characterized by blindness, mental dullness and abnormal behavior. There is atrophy of the brain with extensive accumulation of lipopigments ('ceroid bodies') in nervous tissue and later viscera as well. The comparable, though possibly not identical, disease in humans is also called Batten disease.
hepatic lipofuscinosis
a deposit of lipofuscin in partly oxidized form in the livers of sheep, which gives them a deep black color and makes them unsaleable. The condition is common in areas where the mulga tree (Acacia aneura) grows and the leaves are fed to sheep as fodder.
References in periodicals archive ?
has been granted Orphan Drug Designation for PLX-200 for the treatment of neuronal ceroid lipofuscinosis (NCL) by the European Medicines Agency (EMA), the company said.
Infantile neuronal ceroid lipofuscinosis (INCL) is a severe lysosomal disease caused by mutations in the CLN1 gene, which encodes the soluble lysosomal enzyme Palmitoyl- Protein-Thioesterase-1 (PPT1) and result in osmiophilic granules accumulating in lysosomes and leading to neuroinflammation, neurodegeneration and death.
Melanosis coli (pseudomelanosis or lipofuscinosis coli) partly endogenous and exogenous from laxatives Black thyroid (also minocycline deposition) Age-related macular degeneration Some Leydig cell tumors;other tumors?
Presentacion de caso clinico: Paciente masculino de 12 1/12 anos con diagnostico de lipofuscinosis infantil aguda (Figura 1).
The efficiency of diet has been reported in seizures related with Rett syndrome, Lafora body disease, Landau Kleffner syndrome which is known as epileptic aphasia and neuronal seroid lipofuscinosis which is a lysosomal storage disease (26).
October 21, 2013 -- Patients with neuronal ceroid lipofuscinosis (NCL, or Batten disease) transplanted with StemCells, Inc.
A secondary form of MPO deficiency has been described in various disorders, including lead poisoning, severe infections, neuronal lipofuscinosis, obstructive jaundice, diabetes mellitus, and such disseminated cancers as acute and chronic myeloid leukemia, myelodysplastic syndrome, and Hodgkin's lymphoma (1), (2), (4).
Thus, were observed damage of total architectonics of liver, microcirculation disturbances, cellular polymorphism, destruction of border plane; incompletion of hepatocytes, fat, hydropic and balloon dystrophy of hepatocytes, lipofuscinosis of hepatocytes, nidus hepatonecrosis, hyperchromatism and polychromatism, degeneration (vacuolization) of hepatocytes' nuclei, intralobular lymphoplasmocytic infiltration, pericentral lymphoid infiltration.
The data were derived from postmortem examinations of three patients in the company's Phase I clinical trial of HuCNS-SC cells in neuronal ceroid lipofuscinosis (NCL, also referred to as Batten disease), a rare and fatal neurode-generative disorder in children.
It is the most common form of a group of disorders called neuronal ceroid lipofuscinosis (or NCLs).
Though rare, it is the most common form of a group of disorders called neuronal ceroid lipofuscinosis.
En los alcoholicos humanos es corriente la deposicion de la lipofuscina del miocardio (Jaatinen, Saukko y Hervonen, 1993) y la lipofuscinosis ceroidea en la fibrosis hepatica alcoholica (Kishi, Maeyama, Koike, Aida, Yoshida y Uchikoshi, 1996).