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Laurence_Moon_Biedl Syndrome 3例를 包含한 網膜色素變性症 43例에 對한 臨床的 觀察

Clinical Survey of 43 Cases of Pigmentary Degeneration of the Retina Including 3 Cases of Laurens-Moon-Bie이 Syndrome

대한안과학회지 1964년 5권 2호 p.55 ~ 62
김상민, 尹炳珠,
소속 상세정보
김상민 (  ) - 國立醫療院 眼科
尹炳珠 (  ) - 國立醫療院 眼科

Abstract


Forty three cases of the pigmentary degeneration of the retina (P.D.R.) visited N.M.C. from Nov. 1958 to Sept. 1964 were analysed clinically in regard to various factors which were also based on a review of litrature. The results of the study include following findings.
1. Among 43 cases, retinitis pigmentosa binocular were the largest (25), Lauren-Moon-Biedl Syndrome (3), Macular Involvement (2), Retinitis Pigmentosa sine pigment,(2), Retinitis Pigmentosa monocular (1).
2. 6-15 years group is exceedingly largest in regard to the age of the onset.
3. Male is exceedingly. prevalent (32) than female(11).
4. 51-60 years old group is the largest in regard to the duration of illness.
5. The known cases through the family history was only 10. The mode of heredity seems to be rather prevalent as recessive than dominant but the group is too small to make a definite decision.
6. Pigmentary deposits in the retina was the largest in regard to the ophthalmoscopic findings. Concentric contraction is the largest as to the visual field changes.
7. Cataract is the largest (24 cases), myopia(12 cases) in the next, as to the complications.
8. As to the symptoms of the Laurens-Moon-Biedl Syndrome, Obecity, Retinitis Pigmentosa, and Mental retardation is found in all cases, but Polydactly in 2 cases, and Genital dystrophy in 1 case. None of cases were specifically related to this disease through the family history.

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