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Ocular manifestations of Wilson's disease in Iran.
Trans Ophthalmol Soc U K. 1977 Apr; 97(1):18-9.TO

Abstract

Within the last 5 years, 25 proved cases of hepato-lenticular degeneration (Wilson's disease) have been seen at the Children's Hospital Medical Center affiliated to Tehran University. The disease manifested abnormal copper metabolism in the following respects: (1) Hypoceruloplasminaemia was present: (2) 24-hr urinary copper excretion was low; (3) Tissue concentration of copper was high; (4) Treatment with penicillamine led to increased copper excretion in the urine and usually to relief of symptoms. The ophthalmologist cannot always assist the paediatrician in diagnosis, but fourteen of the 25 patients showed a Kayser-Fleischer ring and these were all in the older age groups. The following conclusions were drawn: (1) The incidence of Wilson's disease in Iran is high; (2) Penicillamine treatment may be successful; (3) Any young person with kidney, liver, or neurological disease of uncertain aetiology should have a detailed ophthalmological examination.

Authors

No affiliation info available

Pub Type(s)

Journal Article

Language

eng

PubMed ID

271391

Citation

Kashani, A A.. "Ocular Manifestations of Wilson's Disease in Iran." Transactions of the Ophthalmological Societies of the United Kingdom, vol. 97, no. 1, 1977, pp. 18-9.
Kashani AA. Ocular manifestations of Wilson's disease in Iran. Trans Ophthalmol Soc U K. 1977;97(1):18-9.
Kashani, A. A. (1977). Ocular manifestations of Wilson's disease in Iran. Transactions of the Ophthalmological Societies of the United Kingdom, 97(1), 18-9.
Kashani AA. Ocular Manifestations of Wilson's Disease in Iran. Trans Ophthalmol Soc U K. 1977;97(1):18-9. PubMed PMID: 271391.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Ocular manifestations of Wilson's disease in Iran. A1 - Kashani,A A, PY - 1977/4/1/pubmed PY - 1977/4/1/medline PY - 1977/4/1/entrez SP - 18 EP - 9 JF - Transactions of the ophthalmological societies of the United Kingdom JO - Trans Ophthalmol Soc U K VL - 97 IS - 1 N2 - Within the last 5 years, 25 proved cases of hepato-lenticular degeneration (Wilson's disease) have been seen at the Children's Hospital Medical Center affiliated to Tehran University. The disease manifested abnormal copper metabolism in the following respects: (1) Hypoceruloplasminaemia was present: (2) 24-hr urinary copper excretion was low; (3) Tissue concentration of copper was high; (4) Treatment with penicillamine led to increased copper excretion in the urine and usually to relief of symptoms. The ophthalmologist cannot always assist the paediatrician in diagnosis, but fourteen of the 25 patients showed a Kayser-Fleischer ring and these were all in the older age groups. The following conclusions were drawn: (1) The incidence of Wilson's disease in Iran is high; (2) Penicillamine treatment may be successful; (3) Any young person with kidney, liver, or neurological disease of uncertain aetiology should have a detailed ophthalmological examination. SN - 0078-5334 UR - https://www.unboundmedicine.com/medline/citation/271391/Ocular_manifestations_of_Wilson's_disease_in_Iran_ L2 - https://medlineplus.gov/wilsondisease.html DB - PRIME DP - Unbound Medicine ER -