Læknablaðið - 15.01.1987, Page 24

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LÆKNABLAÐIÐ
Flokkun þessi er alls ekki fullnægjandi, þar sem
einnig geta verið sjúklegar breytingar í sjóntaug,
æðum augnbotns, glerhlaupi og æðahimnu (9,
12).
Mikilvægt er að geta leiðbeint þessum
einstaklingum um námsval og vinnu og veitt
erfðafræðilega ráðgjöf.
Dæmi er um að einn úr þessari ætt valdi sér iðn er
krafðist góðrar sjónar en varð síðan að skipta um
starf um þrítugsaldur þar sem sjón hans hafði
versnað til muna.
Höfundum er kunnugt um eina aðra ætt með
þennan sjúkdóm hér á landi og hefur tveimur
einstaklingum hennar verið lýst áður (9).
SUMMARY
This study contains a pedigree of an Icelandic family of
102 members with 8 affected males, having hereditary
juvenile retinoschisis. The family has been known to
Icelandic ophthalmologists for several years and
members of the family have been examined in this
country and abroad. In this study we have examined 21
members of this family at the University Eye Clinic, St.
Josephs Hospital in Reykjavik and obtained
informations about 7 members of this family from other
ophthalmologists. Pathologic findings included as much
as microcystoid macular and periferal retinal
degeneration, retinoschisis of varying degree and
derangement of the pigment epithelium. The results of
this study are in agreement with previous reports from
other countries.
HEIMILDIR
1. Andersson JR. Anterior Dialysis of Retina.
Disinsertion or avulsion at the Ora Serrata. Br J
Ophthalmol 1932; 16: 705-26.
2. Tomson E. Memorandum Regarding a Family in
Which Neuro-Retinal Diseases of Unusual Kind
Occures Only in the Males. Br J Ophthalmol 1932;
16: 681-6.
3. Forsius H, Vainio-Mattila B, Eriksson A. X-linked
hereditary retinoschisis. Br J Ophthalmol 1967; 46:
678-81.
4. Krause U, Vainio-Mattila B, Eriksson A, Forsius
H. Fluorescein angiographic studies on
x-chromosomal retinoschisis. Acta Ophthalmol
(Kbh) 1970; 48: 794-807.
5. Vainio-Mattila B, Eriksson A, Forsius H.
X-chromosomal recessive retinoschisis in the
region of Pori. Acta Ophthalmol (Kbh) 1969; 47:
1135-48.
6. Constantares AA, Dobbie JG, Chromotos EA.
Juvenile sex linked recessive retinoschisis in black
family. Am J Ophthalmol 1972; 74: 1166-78.
7. Gieser EP, Falls HF. Hereditary retinoschisis. Am J
Ophthalmol 1961; 51: 1193-1200.
8. Lisch W, Ullerich K. Hereditare periphere
Retinoschisis. Klin Mbl Augenheilk 1979; 174:
351-8.
9. Bengtsson B, Linder B Sex -linked hereditary
juvenile retinoschisis. Acta Ophthalmol (Kbh)
1967; 45: 411-23.
10. Manschot WA. Pathology of hereditary juvenile
retinoschisis. Arch Ophthalmol 1972; 88: 131-38.
11. Yanhoff M, Rahn E K, Zimmerman LE.
Histophathology of juvenile retinoschisis. Arch
Ophthalmol 1968; 79: 49-53.
12. Lisch W, Feltrup FJ, Lisch C. Differentialdiagnose
hereditarer vitroretinaler Degenerationen. Klin Mbl
Augenheilk 1982; 181: 10-13.