Lim
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*Tuberous sclerosis; (originally not starts from neuron)
hamartoma :tumor like...NO TUMOR!!!!(qbank distractor was Hemangioblastoma)
A benign tumorlike malformation resulting from faulty development in an organ and composed of an abnormal mixture of tissue elements that develop and grow at the same rate as normal elements but are not likely to compress adjacent tissue.
1. adenoma sebaceum(fibrovascular tissue,nothing with neuron)
2. hypopigmented"ash leaf spots" on skin
3. cortical(qbank q,Subepndymal giant cell astrocytoma) and retinal hamartoma ->seizure,MR ,
-kinds of neronal?
4. renal cyst,cardiac rhabdomyomas
TS is just abnormal growh..of something tissue ... or somwhere in the body... NO neuronal involvement...
------------------------------------------------------------------------- ------------------------------------*Neurofibromatosis
NF-I(Von Recklinghausen's disease;ch17) 90%
0. Cafe-au-lait spots.
1. Lisch nodules(pigmented iris hamartomas; this name is why two things can be confused... except this,all things about neurons..)
2.meningioma pheochromocytoma
NF-II(ch22) ; 10%
0. Cafe-au-lait spots.
1. bilateral acoustic neuroma
2. ependymoma,meningioma NO LISCH(-)
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i marked a wrong answer between two..i'm reading FA now...i guess it's because Iris hamartoma
(qbank correct rate was 48%)
if i'm wrong above or there're things to add...please write..
Easy q now..
where do 'periungual fibroma,shagreen patch,renal angiomyolipoma' go in?
Edited by Lim on 09/08/06 - 08:36 AM
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| vanshita
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Non traumatic ungual or periungual fibroma
Shagreen patch (connective tissue nevus)
seen in tuberous sclerosis
i think renal angiomyolipoma' in von hippes lindu syndrome
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| Lim
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All goes in TS.
it's Multiple bilateral renal cell carcinoma for VHL.
renal angiomyolipoma for Tuberous Sclerosis ( extremely rare but coexistence recognized )
i also leant at school that's the reason Tuberous sclerosis patients have to checked their kidneys by abdominal sono.
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| docarchana
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neurological symptoms in TS r hydrocephalus,infantile spasms,epilepsy,autism,mental retardation.
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