1
404 about histidinuria and histaminuria; and furthermore, we know nothing at all about the excretion of all those other amino-acids which we cannot readily detect and estimate. Taking the normal range of histidine excretion to be up to 80 mg. per 100 c.cm. of urine, this amounts to more than 1 g. per day-quite a large quantity of a valuable and essential bodily constituent. We know of no syndrome due to histidine deficiency, but this rate of loss suggests that such a thing is not inconceivable. FRACTURED MANDIBLE IN the last war and up to now in the present one the treatment of fractures of the mandible at or posterior to the angle has presented insurmountable difficulties when the patient has no teeth. Here, as in other fractures, correct apposition of the ends of the broken bone is the first essential to bony union, and such apposition is very difficult to attain because when the fracture is far back in the region of the last molar the small posterior frag- ment is drawn upwards and inwards towards the midline by the pterygoid and temporal muscles. The many ingenious appliances so far devised have mostly failed to keep the ends together, so that the patient has been left with fibrous union and deformity. Moreover, they usually entailed immobilisation of the jaw leading to difficulties in feeding and delayed union from prolonged lack of function. Last November Vaughan Tomey suggested to the central counties branch of the British Dental Association in Birmingham that external splinting might be feasible, and the two papers published in this issue demonstrate that it is. Dr. Pohl grips the frag- ments with hook-screws of his own design and welds the screws together by an external metftl bar. Mr. Rainsford Mowlem and his colleagues at Hill End maxillo-facial unit use crossed Kirschner wires inserted into each fragment, and fix them by an external clamp. Pohl has applied his technique to one case ; the other has been used in 19. Both have the advantage of allowing some measure of mastication as soon as the splint is applied. A SOCIAL DIAGNOSTICIAN DiA&osis must come before treatment, and in his second examination 1 of the symptoms of social ill in York, Mr. Seebohm Rowntree once more, as in 1899, diagnoses poverty as their cause. In his preface he disclaims any wish to prescribe for the disease, contenting himself with a masterly picture of the syndrome. He discusses the remedies that lie to hand, but leaves the choice of treatment to others. He has made his diag- nosis on the result of a house-to-house visitation of over 16,000 families-almost the whole working-class popula- tion of York-and in a chapter, which he modestly assumes will interest only the statistician, he compares his figures with those which might have been obtained by the more usual sampling methods. Mr. Rowntree’s technique gives an objective picture of the economic situation of the city, of its housing, of its public-health and education services, and of the leisure-time habits of its inhabitants. He found that the proportion of the working-class population living in abject poverty has been reduced by more than half since 1899, but that rather more than half of the working-class children under one year of age were still living below the poverty line ; 47% of them would probably remain in poverty for five years or more, and 31 % for ten years or more. Yet Mr. Rowntree’s standards of poverty are not indulgent. He has taken as his criterion for a man and wife with three children a minimum income of 43s. 6d. after rent and rates are paid. But during the last forty years there has been progress as well as poverty. Housing in York shows a persistent upward trend, and both social and public-health services have increased to a remarkable extent. The chapter 1. Poverty and Progress. London: Longmans, Green and Co. Pp. 540. 15s. on health records a fall in the general death-rate of a half, and in the infant mortality of two-thirds, but even so the infant death-rate was nearly twice as high in the poorest class as in the highest class of those investigated. Reduced working hours combined with increased facilities for recreation have led to widened activities . and interests for workers of all ages. Less money is spent on drink, but the enormous growth of gambling has created a new " Public Enemy No. I," and in these circensian days Mr. Rowntree urges that leisure should be a time of re-creation and not escapism. Andrew Lang is reputed to have said " Many use statistics as a drunken man uses a lamp-post-for support rather than for illumination," but Mr. Rowntree has used them as a searchlight on the murky limitations which poverty imposes on the life and health of our people. RETINAL DYSTROPHY THE Lawrence-Moon-Biedl syndrome is a rare in- herited condition in which small stature, hypogenitalism, obesity, retinitis pigmentosa, polydactyly and mental defect are associated. The genetics of its inheritance are highly involved, and the evidence available is not enough to establish the gene abnormality that underlies it. R. W. B. Ellis and F. W. Law record an incomplete example of the full syndrome-an obese undersized child of normal intelligence, with hypogenitalism and retinal changes Mental defect and polydactyly were lacking in her case, but a maternal uncle had a supernumerary digit and a sister was mentally defective. These observations alone will hardly make the genetic picture clearer, though they may be set alongside Sorsby, Avery and Cockayne’s observation 2 of -" the frequent occur- rence of one or more components of the Lawrence-Biedl syndrome in the ascendants of patients showing the full syndrome." Ellis and Law describe the retinal defect in their case in some detail. The retinae appeared thin, especially at the maculae, whith were atrophic.. The choroidal vessels were easily seen. There was general deficiency of retinal pigment, but a number of plaques or veils of pigment, varying in size up to twice the diameter of a disc, were scattered over the retinae, lying at about the level of the retinal vessels, and there was a peppering of the macular area with pigmentary dots. These appearances bear no resemblance to what is com- monly called retinitis pigmentosa, although they agree fairly closely with Lawrence and Moon’s description of their original cases. In*’other published records con- siderable variation in the retinal appearances is found, but few of the accounts approximate to that of typical retinitis pigmentosa. Ellis and Law therefore suggest that, for the sake of precision in terminology, the term retinitis pigmentosa, applied to a component of the Lawrence-Moon-Biedl syndrome, be dropped and " re- . tinal dystrophy " be used instead. They quote Lawrence and Moon who said (in 1886) /’ In calling these cases by the name of retinitis pigmentosa, we have been guided rather by usage than by the intimate nature of the cases." Sir GiRLiNCr BALL has been appointed a member of the Shakespeare Committee in place of Sir Alfred Webb-Johnson, who retains his membership in the capacity of president of the Royal College of Surgeons. A LUNCHEON party at Grosvenor House on Monday celebrated the 21st anniversary of the opening of the Tavistock Clinic, where the first patient was received on Sept. 27; 1920. Since that day 14,700 patients have been treated at the clinic in Tavistock Square, in Malet Street, and since the outbreak of war at Westfield College, Hampstead. Colonel J. R. Rees, the medical director, said that these patients had come from every county in Britain. 1. Arch. Dis. Childh. 1941, 16, 105. 2. Sorsby, A., Avery, H. and Cockayne, E. A. Quart. J. Med. 1939, 8, 51.

RETINAL DYSTROPHY

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about histidinuria and histaminuria; and furthermore, weknow nothing at all about the excretion of all those otheramino-acids which we cannot readily detect and estimate.Taking the normal range of histidine excretion to be upto 80 mg. per 100 c.cm. of urine, this amounts to morethan 1 g. per day-quite a large quantity of a valuableand essential bodily constituent. We know of no

syndrome due to histidine deficiency, but this rate of losssuggests that such a thing is not inconceivable.

FRACTURED MANDIBLEIN the last war and up to now in the present one the

treatment of fractures of the mandible at or posterior tothe angle has presented insurmountable difficulties whenthe patient has no teeth. Here, as in other fractures,correct apposition of the ends of the broken bone is thefirst essential to bony union, and such apposition is verydifficult to attain because when the fracture is far backin the region of the last molar the small posterior frag-ment is drawn upwards and inwards towards the midlineby the pterygoid and temporal muscles. The manyingenious appliances so far devised have mostly failedto keep the ends together, so that the patient has beenleft with fibrous union and deformity. Moreover, theyusually entailed immobilisation of the jaw leading todifficulties in feeding and delayed union from prolongedlack of function. Last November Vaughan Tomeysuggested to the central counties branch of the BritishDental Association in Birmingham that external splintingmight be feasible, and the two papers published in thisissue demonstrate that it is. Dr. Pohl grips the frag-ments with hook-screws of his own design and weldsthe screws together by an external metftl bar. Mr.Rainsford Mowlem and his colleagues at Hill Endmaxillo-facial unit use crossed Kirschner wires insertedinto each fragment, and fix them by an external clamp.Pohl has applied his technique to one case ; the otherhas been used in 19. Both have the advantage of

allowing some measure of mastication as soon as thesplint is applied.

A SOCIAL DIAGNOSTICIAN

DiA&osis must come before treatment, and in hissecond examination 1 of the symptoms of social ill inYork, Mr. Seebohm Rowntree once more, as in 1899,diagnoses poverty as their cause. In his preface hedisclaims any wish to prescribe for the disease, contentinghimself with a masterly picture of the syndrome. Hediscusses the remedies that lie to hand, but leaves thechoice of treatment to others. He has made his diag-nosis on the result of a house-to-house visitation of over16,000 families-almost the whole working-class popula-tion of York-and in a chapter, which he modestlyassumes will interest only the statistician, he compareshis figures with those which might have been obtainedby the more usual sampling methods. Mr. Rowntree’s

technique gives an objective picture of the economicsituation of the city, of its housing, of its public-healthand education services, and of the leisure-time habitsof its inhabitants. He found that the proportion of theworking-class population living in abject poverty hasbeen reduced by more than half since 1899, but thatrather more than half of the working-class children underone year of age were still living below the poverty line ;47% of them would probably remain in poverty for fiveyears or more, and 31 % for ten years or more. Yet Mr.Rowntree’s standards of poverty are not indulgent.He has taken as his criterion for a man and wife withthree children a minimum income of 43s. 6d. after rentand rates are paid.But during the last forty years there has been progress

as well as poverty. Housing in York shows a persistentupward trend, and both social and public-health serviceshave increased to a remarkable extent. The chapter1. Poverty and Progress. London: Longmans, Green and Co.

Pp. 540. 15s.

on health records a fall in the general death-rate of a half,and in the infant mortality of two-thirds, but even so theinfant death-rate was nearly twice as high in the poorestclass as in the highest class of those investigated.Reduced working hours combined with increasedfacilities for recreation have led to widened activities

. and interests for workers of all ages. Less money isspent on drink, but the enormous growth of gamblinghas created a new " Public Enemy No. I," and in thesecircensian days Mr. Rowntree urges that leisure shouldbe a time of re-creation and not escapism. Andrew Langis reputed to have said " Many use statistics as a drunkenman uses a lamp-post-for support rather than forillumination," but Mr. Rowntree has used them as a ’

searchlight on the murky limitations which povertyimposes on the life and health of our people.

RETINAL DYSTROPHY

THE Lawrence-Moon-Biedl syndrome is a rare in-herited condition in which small stature, hypogenitalism,obesity, retinitis pigmentosa, polydactyly and mentaldefect are associated. The genetics of its inheritance arehighly involved, and the evidence available is not

enough to establish the gene abnormality that underliesit. R. W. B. Ellis and F. W. Law record an incompleteexample of the full syndrome-an obese undersized childof normal intelligence, with hypogenitalism and retinalchanges Mental defect and polydactyly were lackingin her case, but a maternal uncle had a supernumerarydigit and a sister was mentally defective. Theseobservations alone will hardly make the genetic pictureclearer, though they may be set alongside Sorsby, Averyand Cockayne’s observation 2 of -" the frequent occur-rence of one or more components of the Lawrence-Biedlsyndrome in the ascendants of patients showing the fullsyndrome." Ellis and Law describe the retinal defectin their case in some detail. The retinae appeared thin,especially at the maculae, whith were atrophic.. Thechoroidal vessels were easily seen. There was generaldeficiency of retinal pigment, but a number of plaquesor veils of pigment, varying in size up to twice thediameter of a disc, were scattered over the retinae, lyingat about the level of the retinal vessels, and there was apeppering of the macular area with pigmentary dots.These appearances bear no resemblance to what is com-monly called retinitis pigmentosa, although they agreefairly closely with Lawrence and Moon’s description oftheir original cases. In*’other published records con-siderable variation in the retinal appearances is found,but few of the accounts approximate to that of typicalretinitis pigmentosa. Ellis and Law therefore suggestthat, for the sake of precision in terminology, the termretinitis pigmentosa, applied to a component of theLawrence-Moon-Biedl syndrome, be dropped and " re- .

tinal dystrophy " be used instead. They quote Lawrenceand Moon who said (in 1886) /’ In calling these cases bythe name of retinitis pigmentosa, we have been guidedrather by usage than by the intimate nature of the cases."

Sir GiRLiNCr BALL has been appointed a member ofthe Shakespeare Committee in place of Sir AlfredWebb-Johnson, who retains his membership in thecapacity of president of the Royal College of Surgeons.A LUNCHEON party at Grosvenor House on Monday

celebrated the 21st anniversary of the opening of theTavistock Clinic, where the first patient was receivedon Sept. 27; 1920. Since that day 14,700 patients havebeen treated at the clinic in Tavistock Square, in MaletStreet, and since the outbreak of war at WestfieldCollege, Hampstead. Colonel J. R. Rees, the medicaldirector, said that these patients had come from everycounty in Britain.

1. Arch. Dis. Childh. 1941, 16, 105.2. Sorsby, A., Avery, H. and Cockayne, E. A. Quart. J. Med.

1939, 8, 51.