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Disease
Symptom
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Enzyme
Compound
Pivot Concepts:
Gene/Protein
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Target Concepts:
Gene/Protein
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Query: UMLS:C0038379 (
strabismus
)
9,317
document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)
Following renal transplantation in
nephropathic cystinosis
, an extension of retinopathy associated with a variety of subtle functional ocular abnormalities may occur. However, these abnormalities have not (except for photophobia) been of subjective significance to cystinotic patients. Our data, gathered from patients transplanted as long as nine years ago, indicate that cystinotic patients should not have renal transplant withheld because of the fear of subsequent progressive ocular deterioration.
J Pediatr Ophthalmol
Strabismus
PMID:Long-term ocular changes in cystinosis: observations in renal transplant recipients. 37 2
We studied the usefulness of topical cysteamine ophthalmic drops in reducing crystal formation within the corneas of patients affected by
nephropathic cystinosis
. A dosage of 0.3% cysteamine given four times daily was chosen as a more manageable method than a previously reported protocol of hourly administration of 0.11% drops. No reduction in crystal formation was observed in our patients; they were followed for seven months.
J Pediatr Ophthalmol
Strabismus
PMID:The effect of topical cysteamine drops on reducing crystal formation within the cornea of patients affected by nephropathic cystinosis. 224 44
An 11 year old boy with
nephropathic cystinosis
developed moderate to severe bilateral optic disc edema two months after he received a deceased donor renal allograft. The bilateral optic disc edema was found to be a result of intracranial hypertension diagnosed by lumbar puncture. No etiology was found. He was treated with acetazolamide and his optic dis edema resolved over a period of eight months and did not recur after acetazolamide was discontinued. The mechanism of intracranial hypertension in patients with
nephropathic cystinosis
is not well understood, but may involve obstruction of cerebrospinal fluid outflow due to deposition of cystine crystals in arachnoid villi.
Binocul Vis
Strabismus
Q 2008
PMID:A case of intracranial hypertension and papilledema associated with nephropathic cystinosis and ocular involvement. 1839 38