hepatolenticular degeneration

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Related to hepatolenticular degeneration: Wilson's disease, Kayser-Fleischer rings


deterioration; change from a higher to a lower form, especially change of tissue to a lower or less functionally active form. When there is chemical change of the tissue itself, it is true degeneration; when the change consists in the deposit of abnormal matter in the tissues, it is infiltration. adj., adj degen´erative.
caseous degeneration caseation (def. 2).
cerebromacular degeneration (cerebroretinal degeneration)
1. degeneration of brain cells and of the macula retinae, as occurs in tay-sachs disease.
2. any lipidosis with cerebral lesions and degeneration of the retinal macula.
colloid degeneration degeneration with conversion of the tissues into a gelatinous or gumlike material.
cystic degeneration degeneration with formation of cysts.
fatty degeneration deposit of fat globules in a tissue.
fibroid degeneration degeneration of a leiomyoma with subsequent fibrosis.
hepatolenticular degeneration Wilson's disease.
hyaline degeneration a regressive change in cells in which the cytoplasm takes on a homogeneous, glassy appearance; also used loosely to describe the histologic appearance of tissues.
hydropic degeneration a form in which the epithelial cells absorb much water.
lattice degeneration of retina a frequently bilateral, usually benign asymptomatic condition, characterized by patches of fine gray or white lines that intersect at irregular intervals in the peripheral retina, usually associated with numerous, round, punched-out areas of retinal thinning or retinal holes.
macular degeneration see macular degeneration.
macular degeneration, congenital see stargardt's disease.
macular degeneration, Stargardt's stargardt's disease.
mucoid degeneration degeneration with deposit of myelin and lecithin in the cells.
mucous degeneration degeneration with accumulation of mucus in epithelial tissues.
myofibrillar degeneration damage to selective cardiac cells when surrounding interstitial cells, nerves, and capillaries remain viable.
myxomatous degeneration mucous degeneration.
spongy degeneration of central nervous system (spongy degeneration of white matter) Canavan disease.
subacute combined degeneration of spinal cord degeneration of both the posterior and lateral columns of the spinal cord, producing various motor and sensory disturbances; it is due to vitamin B12 deficiency and is usually associated with pernicious anemia. Called also Lichtheim's or Putnam-Dana syndrome.
wallerian degeneration fatty degeneration of a nerve fiber that has been severed from its nutritive source.
Zenker's degeneration Zenker's necrosis.

Wil·son dis·ease

(wil'sŏn), [MIM*277900]
1. A disorder of copper metabolism, characterized by liver cirrhosis, basal ganglia degeneration, neurologic manifestations, and deposition of green or golden brown pigmentation in the periphery of the cornea. The plasma levels of copper and ceruloplasmin are decreased; urinary excretion of copper is increased; and the amounts of copper in the liver, brain, kidneys, and lenticular nucleus are unusually high whereas cytochrome oxidase is reduced. Autosomal recessive inheritance caused by mutation in the copper-transporting ATPase gene (ATP7B) on chromosome 13q.
See also: Kayser-Fleischer ring. Synonym(s): hepatolenticular degeneration
2. Synonym(s): exfoliative dermatitis

hepatolenticular degeneration

Etymology: Gk, hēpar + L, lens, lentil
an abnormal autosomal-recessive condition associated with defective copper metabolism in the body, characterized by decreased serum ceruloplasmin and copper levels and increased secretion of urinary copper. In individuals with this condition, tissue deposits of copper associated with hepatic cirrhosis, deep marginal pigmentation of the cornea (known as Kayser-Fleischer rings), and extensive degeneration of the central nervous system, especially the basal ganglions, develop. Also called Wilson's disease.

hepatolenticular degeneration

Wilson's disease, see there.

he·pa·to·len·tic·u·lar de·gen·er·a·tion

(hep'ă-tō-len-tik'yū-lăr dĕ-jen'ĕr-ā'shŭn)
1. A familial disorder characterized by copper deposition in the liver, causing chronic hepatitisand eventually cirrhosis; degeneration of the lenticular (pallidal and putaminal) nuclei, and marked hyperplasia of astrocytes in the cerebral cortex, cerebellum, basal ganglia, and brainstem nuclei; plasma levels of ceruloplasmins and copper are decreased, urinary excretion of copper is increased, and the amounts of copper in the liver, brain, and kidneys is high; clinical features include deposition of golden brown pigment in the cornea (Kayser-Fleischer rings), dysphasia and dysarthria, rigidity, and a coarse resting tremor, which increases when the limbs are outstretched ("wing-beating" tremor).
2. Synonym(s): Wilson disease (1) .

hepatolenticular degeneration


hepatolenticular degeneration

Wilson's disease.

Patient discussion about hepatolenticular degeneration

Q. Hi this is Wilson; I have a daughter, who is 5. She has frequent nose bleeds when she is behaving badly. Hi this is Bell; I have a daughter, who is 5. She has frequent nose bleeds when she is behaving badly. She has been screened by her school for ADHD and I haven’t had a chance to consult a doctor for her screening and exam for ADHD. I thought that her nose bleeds cause due to her behavior? I need help.

A. I don’t have an idea about this. But I think that you should first discuss these issues with your doctor. Also mention to the doctor if she has any problems with sleeping, like only sleeping 6 hours a night. Try with occupational Therapists which help children and adults with small motor issues like handwriting and also with sensory issues like learning how to calm down. Early intervention is always best.

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