choroideremia
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sensitivity developed after only twenty-four hours’treatment. The ointment was not tolerated by acute oroozing lesions, possibly because of the nature of thevehicle.
Topical applications are unexpectedly difficult to assess,for no two skin conditions are identical and all are
subject to changes independent of treatment. Moreover,symptoms cannot be measured objectively, and theycan be influenced- by the enthusiasm of the doctor armedwith any new preparation. Treating a symmetricaleruption on one side only might seem to be the answer,but it has been found in various skin diseases that theuntreated side may respond as well as the treated.The effect of the vehicle must be considered : -: for
example, Reiss and Kern,16 when testing an anti-histamineointment, found that over 28% of their patients wererelieved of itching by the base alone. In these investi-
gations the main control has been the patients’ experiencewith previous preparations, which may have had quitedissimilar bases. The reports are therefore open to
criticism, but they can at least be taken as an indica-tion that the substance is useful and safe. It seemsunwise to use it combined in an ointment with ananti-histamine (as is the case in ’Teevex’), for anti-histamines can also cause sensitisation dermatitis after
repeated local applications. On the other hand, it wouldbe helpful to have the pure substance available for
incorporation in different media.
16. Reiss, F., Kern, B. B. J. Allergy, 1950, 21, 160.17. McCulloch, C., McCulloch. R. J. P. Trans. Amer. Acad. Ophthal.
Oto-laryng. 1948, 52. 160.18. Sorsby, A., Francheschetti, A., Joseph, R., Davey, J. B. Brit.
J. Ophthal. 1952, 36, 547.
CHOROIDEREMIA
PROGRESSIVE degenerative lesions of the retina thathave been grouped as
"
abiotrophies " include thedifferent kinds of retinitis pigmentosa, gyrate atrophyof the retina, choroideremia, and several types of maculardystrophy. In choroideremia the earliest symptom is
night-blindness ; then peripheral vision is gradually lostand finally central vision, so that the patient is blind. Inadvanced cases ophthalmoscopic examination shows thatthe choroidal vessels have disappeared and there isextensive retinal atrophy, affecting the central area least.At necropsy the choroidal vessels are either sclerosed orabsent, and the outer layer of the retina is extremelydegenerate. Earlier workers thought that this conditionmight be a variant of retinitis pigmentosa, with which itshares several features-the strong genetic determination,the progressive nature of the lesion, and in some instancespigmentation of the retina. Furthermore some of thefemale relations of patients with choroideremia werethought to have retinitis pigmentosa.A few years ago American workers
17 described a familyof some 600 of whom 33 had choroideremia. Sorsby andhis colleagues 18 have now given an account of threefurther affected families. These studies show clearly thatthe condition is distinct from retinitis pigmentosa, whichdid not occur in these families. They also show that thecondition is progressive ; and in different members ofthe family at different ages all stages can be seen fromsymptomless pepper-and-salt changes in the peripheryof the retina to the unmasking, sclerosis, and finallydisappearance of the retinal vessels. Professor Sorsbyfound advanced choroideremia in a boy aged only 41/2’and one man was blind at 25 ; but blindness is rarebefore the age of 40, central vision being well retained’while peripheral vision is gradually lost.
Only males have symptoms of choroideremia, but
many of the females in these families had retinal
changes typical of the earliest stages of the disease inmales. There is thus no doubt that the condition is dueto a sex-linked gene which is intermediate between adominant and a recessive ; it produces a severe lesion in
the male, but in the heterozygous " carrier " state in the
female it produces only a mild and apparently non-progressive disturbance. Sorsby and his colleagues showthat the exceptions to this mode of inheritance describedin earlier reports are due to errors in diagnosis, and thatthe carrier state in the female relations of men withchoroideremia was mistaken for retinitis pigmentosa.With any genetically determined disorder the ability
to recognise the carrier state is most helpful in advisingparents on the chance that their children will be affected.The sisters of men with choroideremia have an evenchance of being normal or of being carriers. It isimportant to them to know which they are, since halfthe sons of carriers on the average will go blind. Thosewith normal retinae can be reassured that there is norisk that their sons and grandsons will be affected.
NEW YEAR HONOURS
IN the select company of the members of the Order ofMerit the place left vacant by the death of Sir CharlesSherrington is filled by the appointment of a surgeon,Prof. Wilder Penfield, F.R.S., of the Montreal Neuro-logical Institute. A leader in neurosurgery, he has doneclassical work on cerebral localisation and the relatedproblems of epilepsy, and the profession in this country,as in Canada, will welcome his new distinction. Of thedoctors to be created knights bachelor, three are
Australians-Mr. N. M. Gregg, the Sydney ophthalmicsurgeon who first drew attention to the relation of
congenital cataract to maternal rubella ; Dr. E. BrittenJones, of Adelaide, who has been vice-president of theRoyal Australasian College of Physicians and presidentof the Medical Board of South Australia ; and Dr. PeterMacCallum, professor of pathology at Melbourne for26 years and chairman of the Australian NationalResearch Council and of the Australian National RedCross. Nearer home it is a pleasure to see a knighthoodconferred on Mr. Zachary Cope, of St. Mary’s Hospital,who has so well earned this honour as a teacher, as aformer vice-president of the Royal College of Surgeons,as chairman of the Cope Committees-and simply as
Zachary Cope. Another welcome name is that of Prof.Arthur Ellis, once of Toronto but afterwards of theLondon Hospital and the regius chair at Oxford, whoseuncommon sense-whether directed to the kidney or thecurriculum-has done so much, in quiet ways, formedicine and its students. We are happy to note thatDr. Pridie’s notable and far-flung work as chief medicalofficer of the Colonial Office since 1948 has been recog-nised by promotion in the Order of St. Michael andSt. George, and that the distinguished medical headsof the Navy and Army, Admiral MacKenzie and GeneralHarris, receive knighthoods in the Order of the BritishEmpire. Many other familiar and respected names willbe found in the list we publish oh p. 87 ; and to thesewe would add that of Mr. A. Landsborough Thomson.D.sc., now knighted, whose long service as secondsecretary of the Medical Research Council makes usalmost able to claim him as a member of our profession.
VACANCIES
READERS who reached the concluding pages of oW’last issue will have noticed the analysis of vacant
appointments which was printed so conspicuously there.As we explained in an annotation, this analysis is toserve as a key, and will be published weekly. But nowthat we have prominently drawn attention to its exis-tence, we are’ transferring it to our advertisementcolumns, where it will be found this week on p. 30.
Dr. G. F. ABERCROMBiE has been elected chairmanof the foundation council of the College of GeneralPractitioners.