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RAYNAUD'S PHENOMENON

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388 clinical syndrome. While these are not the first records of the excretion of cestrin in the urine in such cases they throw this feature into stronger relief and indicate an important line for further investigation. It is of interest that Graef and his co-workers 3 inde- pendently observed the hyaline cytoplasmic change in the basophil cells of the pituitary gland in their case ; their coloured plate clearly shows it to be the same as that described by Crooke. Graef regards it as a selective degenerative change, but it is by no means certain that the accompanying nuclear degenerations described by him are not attributable to the terminal sepsis from which the patient died. RAYNAUD’S PHENOMENON To the July number of the Quarterly Journal of Medicine Dr. John Hunt contributes a "critical review" of "the Raynaud phenomena," which will be welcome to all who are interested in the clinical manifestations of peripheral vascular spasm. He deplores the confusion that for fifty years has attended the term " Raynaud’s disease," and resolves a great deal of it by returning to Jonathan Hutchinson’s term. Raynaud’s phenomenon is a symptom which may occur in more diseases than one. It is defined, on the basis of Raynaud’s description, as " inter- mittent pallor or cyanosis of the extremities, pre- cipitated by exposure to cold, without clinical evidence of blockage of the large peripheral vessels and with nutritional lesions, if present at all, limited to the skin," which more shortly put is " attacks of ’dead’ fingers or toes brought on by cold." This is a clinical definition, which Dr. Hunt equates with Lewis and Pickering’s physiological definition-" active and intermittent closure of small arteries of the order of digital arteries, supplying the extremities." Attacks of this kind may be observed in many clinical associations, and the bulk of Dr. Hunt’s paper is devoted to classification and description of these, with illustrative records from Raynaud’s writings and from his own experience. The phenomenon is common in the form of the " dead fingers" that many normal people experience on exposure to cold air or cold water ; these occur in both sexes, appear first in childhood, and are apt to run in families, for which reasons they are grouped separately under the heading " Hereditary cold fingers," and dis- tinguished from the rare true " Raynaud’s disease," where the onset is in early adult life and the patient almost always a woman, the attacks being usually symmetrical and in general much more frequent and severe. To this latter group only, now well defined and much studied, the term " Raynaud’s disease " may with advantage be restricted. Its aetiology is quite obscure. In a further group of patients, on the other hand, the phenomenon seems to have a direct relation to the use of vibrating tools, such as pneumatic drills and riveting machines ; it begins in the fingers nearest to the vibrating end of the tool, and spreads to the others, but the exciting cause of each attack is not vibration but cold. In those three groups Raynaud’s phenomenon occurs unasso- ciated with known structural lesions of the vessels involved or of adjacent parts, though admittedly evidence of the histological appearances of those vessels is scanty. In the remaining groups structural lesions are present. Raynaud’s phenomenon is often associated with the form of scleroderma which affects hands, feet, and face ; it may precede the skin changes ; intimal thickening and medial fibrosis are found in the vessels. Raynaud’s phenomenon in the feet is a feature of about one-third of all cases of thrombo-angiitis obliterans in their earliest stages, but is no longer evident by the time claudication and rest-pain are prominent. It may also occur in feet whose vessels are moderately narrowed by arterio- sclerosis. An infrequent but interesting association is with syphilitic arteritis, and Dr. Hunt emphasises that it is in syphilitic patients only that Raynaud’s phenomenon in the extremities is accompanied by either hsemoglobinuria or necrotic lesions of nose and ears. He describes also a rarer form of arteritis in the limbs, which proceeds in the space of days to almost complete obstruction and then recedes, Raynaud’s phenomenon appearing in the inter- mediate stages ; this sequence of events follows a streptococcal tonsillitis. Dr. Hunt’s final group includes a number of miscellaneous conditions, like leukaemia, advanced phthisis, and polycythsemia, vera, in which Raynaud’s phenomenon may occasionally be seen, but where its basis is still quite obscure. The mechanism of Raynaud’s phenomenon is only briefly discussed. While it is generally agreed that in an attack the digital arteries close completely, there is still doubt how far sympathetic vasoconstrictor impulses contribute to that closure. Dr. Hunt accepts Lewis’s position-namely, that vasoconstrictor tone or an increase in vasoconstrictor tone may certainly contribute, but that there is no satisfactory evidence that vasoconstrictor tone in patients showing Raynaud’s phenomenon varies beyond normal physio- logical limits. The vessels in question also react by constriction to the effect of direct cooling, but even this combined with sympathetic vasoconstriction will not bring about complete closure of normal vessels (unless perhaps in the peculiar circumstances of Dr. Hunt’s experiments on himself, where the tem- perature of the whole body and of the blood was lowered to 94°-95°F.). An abnormality of the vessels must therefore be postulated to explain Raynaud’s phenomenon. In the later groups of Dr. Hunt’s classification this is presumably the structural change known to be present; the vessel is already so much narrowed thereby that moderate contraction of its media can close it. The familiar method of recording the rise in skin temperature during anaesthesia of the sympathetic supply to the vessels will demonstrate the organic narrowing. In the earlier groups however-hereditary cold fingers, " Raynaud’s disease " proper, and Raynaud’s phenomenon in vibratory tool workers-no structural change is known and sympathetic paralysis will produce full vasodilatation. Here the abnormality must be "functional," an abnormal reactivity to local cold. There the matter stands. From the practical aspect these considerations indicate what may be expected of sympathetic ganglionectomy as a therapeutic measure. It cannot remove the local abnormality in vessels that display Raynaud’s phenomenon, but it does remove the normal vaso- constrictor impulses which, added to the local abnormality, contribute to the occlusive spasm. Where the local abnormality is structural (and usually progressive), this seldom benefits the patient much or for very long, but in the case of " Raynaud’s disease " proper, where the local abnormality is apparently functional, ganglionectomy diminishes the frequency and severity of the attacks to an extent for which most severely affected patients are grateful. The operation is worth trying in some cases of sclero- derma with Raynaud’s phenomenon, but it is then less promising, while in the people with hereditary cold fingers and the workers with vibrating tools the severity of their symptoms rarely justifies it. These conclusions, like all his arguments, Dr. Hunt
Transcript

388

clinical syndrome. While these are not the firstrecords of the excretion of cestrin in the urine in suchcases they throw this feature into stronger relief andindicate an important line for further investigation.It is of interest that Graef and his co-workers 3 inde-pendently observed the hyaline cytoplasmic change inthe basophil cells of the pituitary gland in their case ;their coloured plate clearly shows it to be the sameas that described by Crooke. Graef regards it as aselective degenerative change, but it is by no meanscertain that the accompanying nuclear degenerationsdescribed by him are not attributable to the terminalsepsis from which the patient died.

RAYNAUD’S PHENOMENON

To the July number of the Quarterly Journal ofMedicine Dr. John Hunt contributes a "criticalreview" of "the Raynaud phenomena," which will bewelcome to all who are interested in the clinicalmanifestations of peripheral vascular spasm. He

deplores the confusion that for fifty years has attendedthe term " Raynaud’s disease," and resolves a greatdeal of it by returning to Jonathan Hutchinson’sterm. Raynaud’s phenomenon is a symptom whichmay occur in more diseases than one. It is defined,on the basis of Raynaud’s description, as " inter-mittent pallor or cyanosis of the extremities, pre-cipitated by exposure to cold, without clinical evidenceof blockage of the large peripheral vessels and withnutritional lesions, if present at all, limited to theskin," which more shortly put is " attacks of ’dead’fingers or toes brought on by cold." This is a clinicaldefinition, which Dr. Hunt equates with Lewis andPickering’s physiological definition-" active andintermittent closure of small arteries of the order ofdigital arteries, supplying the extremities."Attacks of this kind may be observed in many clinical

associations, and the bulk of Dr. Hunt’s paper isdevoted to classification and description of these,with illustrative records from Raynaud’s writingsand from his own experience. The phenomenon iscommon in the form of the " dead fingers" thatmany normal people experience on exposure to coldair or cold water ; these occur in both sexes, appearfirst in childhood, and are apt to run in families,for which reasons they are grouped separately underthe heading " Hereditary cold fingers," and dis-

tinguished from the rare true " Raynaud’s disease,"where the onset is in early adult life and the patientalmost always a woman, the attacks being usuallysymmetrical and in general much more frequent andsevere. To this latter group only, now well definedand much studied, the term " Raynaud’s disease "may with advantage be restricted. Its aetiology is

quite obscure. In a further group of patients, onthe other hand, the phenomenon seems to have adirect relation to the use of vibrating tools, such aspneumatic drills and riveting machines ; it beginsin the fingers nearest to the vibrating end of thetool, and spreads to the others, but the exciting causeof each attack is not vibration but cold. In thosethree groups Raynaud’s phenomenon occurs unasso-ciated with known structural lesions of the vesselsinvolved or of adjacent parts, though admittedlyevidence of the histological appearances of thosevessels is scanty. In the remaining groups structurallesions are present. Raynaud’s phenomenon is oftenassociated with the form of scleroderma which affectshands, feet, and face ; it may precede the skin

changes ; intimal thickening and medial fibrosis arefound in the vessels. Raynaud’s phenomenon in thefeet is a feature of about one-third of all cases of

thrombo-angiitis obliterans in their earliest stages,but is no longer evident by the time claudication andrest-pain are prominent. It may also occur in feetwhose vessels are moderately narrowed by arterio-sclerosis. An infrequent but interesting associationis with syphilitic arteritis, and Dr. Hunt emphasisesthat it is in syphilitic patients only that Raynaud’sphenomenon in the extremities is accompanied byeither hsemoglobinuria or necrotic lesions of nose andears. He describes also a rarer form of arteritis inthe limbs, which proceeds in the space of days toalmost complete obstruction and then recedes,Raynaud’s phenomenon appearing in the inter-mediate stages ; this sequence of events follows astreptococcal tonsillitis. Dr. Hunt’s final groupincludes a number of miscellaneous conditions, likeleukaemia, advanced phthisis, and polycythsemia, vera,in which Raynaud’s phenomenon may occasionallybe seen, but where its basis is still quite obscure.The mechanism of Raynaud’s phenomenon is only

briefly discussed. While it is generally agreed thatin an attack the digital arteries close completely,there is still doubt how far sympathetic vasoconstrictorimpulses contribute to that closure. Dr. Hunt acceptsLewis’s position-namely, that vasoconstrictor toneor an increase in vasoconstrictor tone may certainlycontribute, but that there is no satisfactory evidencethat vasoconstrictor tone in patients showingRaynaud’s phenomenon varies beyond normal physio-logical limits. The vessels in question also react byconstriction to the effect of direct cooling, but eventhis combined with sympathetic vasoconstriction willnot bring about complete closure of normal vessels(unless perhaps in the peculiar circumstances ofDr. Hunt’s experiments on himself, where the tem-perature of the whole body and of the blood waslowered to 94°-95°F.). An abnormality of thevessels must therefore be postulated to explainRaynaud’s phenomenon. In the later groups ofDr. Hunt’s classification this is presumably thestructural change known to be present; the vesselis already so much narrowed thereby that moderatecontraction of its media can close it. The familiarmethod of recording the rise in skin temperatureduring anaesthesia of the sympathetic supply to thevessels will demonstrate the organic narrowing. Inthe earlier groups however-hereditary cold fingers,"

Raynaud’s disease " proper, and Raynaud’sphenomenon in vibratory tool workers-no structuralchange is known and sympathetic paralysis willproduce full vasodilatation. Here the abnormalitymust be "functional," an abnormal reactivity tolocal cold. There the matter stands. From the

practical aspect these considerations indicate whatmay be expected of sympathetic ganglionectomy as atherapeutic measure. It cannot remove the local

abnormality in vessels that display Raynaud’sphenomenon, but it does remove the normal vaso-constrictor impulses which, added to the localabnormality, contribute to the occlusive spasm.Where the local abnormality is structural (andusually progressive), this seldom benefits the patientmuch or for very long, but in the case of

"

Raynaud’sdisease " proper, where the local abnormality is

apparently functional, ganglionectomy diminishes thefrequency and severity of the attacks to an extentfor which most severely affected patients are grateful.The operation is worth trying in some cases of sclero-derma with Raynaud’s phenomenon, but it is thenless promising, while in the people with hereditarycold fingers and the workers with vibrating tools theseverity of their symptoms rarely justifies it.These conclusions, like all his arguments, Dr. Hunt

389

bases on a very extensive study of the literature anda considerable personal experience. His surveyshould do much to bring about a wider and clearerrecognition of Raynaud’s phenomenon and its

significance, as well as a more accurate diagnosis ofthe disease process in patients who present it.

REACTIONS AFTER DIPHTHERIA TESTS

ATTENTION has lately been drawn to two rare

types of reaction that may follow the Moloney andSchick tests, and it is desirable that workers usingthese tests should have a clear picture of thesereactions so that they will not be surprised or undulyalarmed if they meet them. In 1934 A. Taylor, of £Dandenong, Australia, reported a peculiar reactionwhich in about 1 in 1000 cases follows the injectionof formol toxoid used in the Moloney test, and ithas now been described in some detail by C. R.MerriHees.1 The Taylor reaction develops in personswho would be passed as Moloney-negative if the testwere read at the usual time. It appears about48 hours later than the Moloney reaction and differsfrom it by having a raised shiny surface and a

mottled red colour. It develops later than the trueMoloney reaction, being best read about the fifthday, and its discoloration is much more persistent.It seems to occur only in Schick-positive persons,and according to Merrillees it indicates that the

subject is likely to react severely when given theusual immunising doses of formol toxoid. The otherreaction was described in our own coloumns lastweek by Dr. Parish. It is a rare sequel to the Schicktest, and the symptoms, which may come on a fewminutes after the injection, are those that may beexpected on injecting foreign protein into a highlysensitive person-local weals, oedema of eyelids andlips, faintness, and dyspnoea. Most of the peoplewho reacted in this way had had previous injectionsof diphtheria antigens or antitoxin, and two wereknown to be sensitive to Witte peptone injectedintracutaneously. Of 14 patients 4 were adults andthe others children ranging in age from 11 monthsto 15 years. It is hard to estimate how often one

may expect to encounter such reactions after theSchick test, but Monroe, Volk, and Park 2 observedonly 2 cases in 7000 immunisations. Parish pointsout that there seem to be no records of this type of £accident before 1934, and he makes the interestingsuggestion that they are due to the peptone whichhas in recent years been added to many preparationsof Schick toxin dilutions with a view to stabilisingthem. Two of the patients in his own series wereknown to be sensitive to Witte peptone and hequotes a similar case of Bousfield’s. Though the possi-bility of general reactions after the Schick test mustnot be forgotten, they are neither common enoughnor dangerous enough to be considered a seriousdrawback.

NUTRITION ABROAD

A FORTNIGHT ago we reviewed two new reportsissued by the League of Nations under the headingof the Problem of Nutrition. They are now joinedby a third,3 which is concerned not with the staticsubject of adequacy or inadequacy of diets but withthe dynamic one of what is being done (for better orfor worse) by nutritional policies, both national andprivate, in various countries. Of course the policiesare almost entirely for the better and to read the

1 Med. Jour. Australia, 1936, i., 503.2 Amer. Jour. Pub. Health, 1934, xxiv., 342.

3 League of Nations. Problem of Nutrition. Vol. III. Nutritionin Various Countries. Obtainable from George Allen and UnwinLtd., London. 1936. Pp. 271. 5s. 6d.

volume is to have a glimpse of the big and wide-spread changes the " newer knowledge of nutrition "has made. Each country mentioned now makessome provision of food for needy children, for

expectant mothers, and for unemployed. But inspite of this common ground the methods of tacklingnutritional problems are intriguingly different.Czechoslovakia regards the lowness of the diet ofemployed persons as a loss to the nation, not so

much because it may lead to ill-health as because itcertainly represents small expenditure, and thuscontributes to the depression. In France the Statedoes little to control the diets of the insured and theunemployed, but it is now being suggested that theinsurance companies that have to pay for sicknessshould be able to take some action in connexionwith the nutrition of their subscribers. In Francethe distribution of unemployment relief in kind is

discouraged for fear that the employed should berelatively worse off. The State of South Australia

goes to the other extreme : a complete ration,including three loaves and 31 lb. of meat per week, isprovided for the unemployed adult.The fitting of nutritional -requirements to the local

agricultural produce or to the Government’s policy isconsidered in several of the countries-notably theArgentine, Latvia, and Italy. In Latvia, because ofa State monopoly, the Government is encouraginggreater consumption of sugar. In Great Britain, onthe other hand, the view is taken, on the basis of theOrr Report, that much money spent on sugar werebetter spent on vegetables. In this report the variousstatements about each country are written fromwithin that country, and each reflects in some measureits form of national pride. To take two examples :the Italians, making a virtue of necessity, give anaccount of their extensive diet propaganda issued onaccount of sanctions. Their instructions, amongothers, are that a portion of meat, fish, dairy products,fruit, or vegetables should be included in the day’smenu. But Finland, the country with the highestconsumption of dairy produce per person, by deplor-ing the present nutrition of its poorest inhabitants,shows its keen sense of how much more can be done.

RELIEF OF INTESTINAL DISTENSION

EXPERIMENT and experience alike testify to thedisastrous results of suddenly releasing pressure inobstructed bowel. Sir William Wheeler, on p. 361,describes the case of the patient who dies soon aftera comparatively simple operation for the relief ofobstruction, although the utmost care has beentaken to combat shock. Such a patient dies withsymptoms of acute toxaemia; the operation thatwas designed to save him has almost certainly hastenedhis end. The fault, according to Wheeler’s observa-tion, is that the release of distension has been toosudden. Many operations for relieving obstructioninclude the insertion of a tube into the lumen of the

gut for drainage purposes, and in acute cases, Wheelersays, it is important that this tube should be of smallcalibre, and the drainage slow and gradual. He

suggests that the cause of the collapse followingsudden decompression is sudden vascular dilatationin the wall of the bowel; like those of the over-distended bladder or gall-bladder the damagedtissues cannot respond to the rapid increase of

circulation, whereas gradual release of tension wouldbe followed by gradual recovery. Aird andHenderson’s experiments, reported on p. 373, provideequally cogent evidence of the danger, though notquite the same explanation of it. In animals they


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