94

9. Dean, G., Barnes, H. D. (1958) Brit. med. J. i, 298.10. Dean, G., Barnes, H. D. (1959) S. Afr. med. J., 33, 246.

In the past decade reports have come from SouthAfrica which suggest that the prevalence of por-phyria there is the highest in the world. Of thetwo types, one is seen in the white populationwhile the other is confined to the Bantus-a remar-kable example of intracellular apartheid. DEAN- 9 has

diagnosed 564 cases of porphyria in 54 family groupsfrom European stock and he holds that all these

patients are descendants of a Dutch settler-a por-phyric-who married at the Cape in 1688. Further-more, after investigating the urines and faeces of1253 whites, DEAN and BARNES 9 found 15 porphyries,a frequency of 1 /c. This type of porphyria in whiteSouth Africans is apparently inherited as a men-

delian dominant character. Acute attacks, similarto these of acute intermittent porphyria, are com-

monest in affected females, whereas light sensitivityis usually found in male members of the same familyand may be very slight or absent in the females.DEAN and BARNES 10 have now compared the por-phyrin excretion of these patients with that of a

group of Swedish patients from WALDENSTRÖM’Sseries of acute intermittent porphyria. In whiteSouth African porphyria they find a high faecal

porphyrin during remission, with an increase in

urinary porphyrin, porphobilinogen, and &dgr;-amino-

11. Schmid, R., Schwartz, S., Watson, C. J. (1951) Arch. intern.Med. 93, 167.

12. Barnes, H. D. (1959) S. Afr med. J. 33, 274.

laevulic acid during the acute phases of the disease.In the Swedish type, contrary to the observation ofScHwD et al. 11, the fsecal porphyrin remains normalduring remission. There has been some confusionover the relation of white South African porphyriato the other porphyrias, and a new title (variegateporphyria) has been coined. But this seems a need-less complication if, as we think, the condition canbe fitted genetically, clinically, and biochemicallyinto the group porphyria cutanea tarda hereditaria(protocoproporphyria). Of the porphyria seen inBantus light sensitivity is almost the only manifes-tation. The great majority of patients are adultsin towns; there is inconclusive evidence of a hered-

itary factor; and the condition may be related to

the hepatic effect of adulterated drinks. The urineusually contains excessive quantities of porphyrinsand occasionally increased amounts of porphobil-inogen and &dgr;-aminolævulic acid, but the faecal por-phyrins are not significantly increased I’. Thus theBantu porphyria, of which hundreds of cases have

already been found, may be readily assigned to thegroup porphyria cutanea tarda symptomatica.

These studies in South Africa will no doubt encou-rage attempts to determine the true prevalence ofthe porphyria diseases in other parts of the world.

Annotations

FIRST DECADE OF N.H.S.

1. Report of the Ministry of Health for the Year ended Dec. 31,1958. Part I. 1. The National Health Service. 2. Welfare,Food and Drugs. Civ il Defence. Cmmd, 806, H.M. StationeryOffice. 1959. Pp. 365. 15s. 6d.

THE report of the Ministry of Health for 1958 1

is largely concerned with the development of theNational Health Service in its first ten years. In hisforeword, Mr. Derek Walker-Smith, the Minister ofHealth, insists that the record of the N.H.S. mustbe judged in the light of contemporary circum-stances : the proportion of the national resources

to be devoted to the service at any particular timemust be decided by the Government of the day.1’ndcr the pressure of economic stringency greatprogress has been made in using resources ef-

fectively. Thus in the hospitals a million more

inpatients were treated in 1958 than ten years before- an increase of over 30 %, whereas the availablebeds had increased by little more than 6 %. Like-wise the number of visits by home nurses was 41 %greater in 1958 than in 1919, although the numberof nurses had risen by only 20 % ; and, althoughthe number of dentists had grown by only 81c, thetotal number of courses of treatment had increasedbv 46 % and courses of conservative treatment hy102 %. The increasing cost of the service duringthese ten years was mainlv due to inflation : some70 % of the increase was accounted for by higherprices and higher remuneration. ,

Looking to the future, the Minister declares:’The battle for health must be fought more and more

in the community, with the family doctor as the leaderof the clinical team and the local health authority sup-porting him... In this battle the hospitals will firstbring reinforcement to the family doctor by the faci-lities, diagnostic aids and consultant services they pro-vide, and then, if necessary, will constitute the lastline of defence, treating the casualties and rehabilitat-ing them to enable their speedy return to active life."

TOP BRANCHES OF THE VASCULAR TREE

1. Ustvedt, H. J. Acta med. scand. 1959, 163, 305.

THE causes of cerebrovascular disease have beenless closely studied than those of other types of ,cardiovascular disease, particularly insufficiency of the coronary arteries, and it cannot be assumedthat the two sets of predisposing factors are ident-ical. Indeed there is some statistical evidencethat they are not, for while deaths from cardiovas-cular disease have increased since the war in cer-tain groups of patients, those due to cerebrovas-cular disease have not shown anything like a cor-responding increase in the same group. Interpret-ation is made difficult because some statisticalrecords showing this trend demonstrated a fall inboth types of disease during the last war. Ustvedt’carefully analysed the apparent changes in Nor-

way, but he was unable to reach a dogmatic con-clusion about their causes. In the age-group 40-59years, both sexes show a declining death-rate forcardiovascular-renal disease from 1920 to 1945. Inmales the decline accelerated during the war, butafter the war the death-rate for men showed animmediate steep rise, whereas the rate for femalesremained steady at its wartime low level. Fatalcerebrovascular lesions in females remained at a

stable level from 1927 to 1939, while there was aslight decrease in males during the same period.During the war years the rate for females fell steep.ly while the male rate fell slightly more quicklythan before the war. After the war the rate forboth females and males increased only slightly.Interpretation of these Norwegian figures is com.plicated because changes in lists of nomenclaturehave cast doubt on the strict correspondence ofgroups from different periods. Although Ustvedtconcludes that the figures he derives show " real

"

trends some doubt must remain on this point. Itis unlikely, however, to account for such a big dis-

’

crepancy as appears in the rates between the twosexes. Similarly the interaction of multiple causesof death in diffuse disease of the vascular system

. is unlikely to react selectively on one sex rather, than the other.

95

2, Elkington, J. St. C. Lancet, 1958, li, 275.3. Denny-Brown, D. Med. Clin. N. Amer. 1951, 35, 1457.4. Wilson, G., Rupp, C., Riggs, H. F. Wilson, W. W. J. Amer.

med. Ass. 1951, 145, 1227.5. Ström, A., Jensen, A. R. Lancet, 1951, i, 126.6. Robinson, R. W., Cohen, W. D. Higano, N., Meyer, R.,

Lukowski, G. H., McLaughlin, R. B., MacGilpin, H.H.J. Amer. med. Ass. 1959, 169, 1149.

Of the exogenous influences that may be invol-

ved, changes in food are likely to affect bothsexes, but smoking is more common in Norwegianmales. No difference in physical or mental stresswas demonstrated. Even if all are subject to the

same external factors, females may be protectedagainst the consequences of these by some endogen-cular-renal disease. Such external factors themsel-ves seem to act on cardiovascular-renal disease,even in males ousdifferer.ce, which applies onlyto cardiouas. Of great importance in cerebro-

vascular disease is atheromatosis, though no

thrombosis of the vessel is demonstrated in morethan half the cases with actual cerebral infarc-

tion. Temporarily lowered blood-pressure or

ansemia may contribute to symptoms and even toinfarction2-3, as may cardiac insufficiency4. Embol-ism accounts for comparatively few cerebrovas-cular accidents, and the significance of spasm ofcerebral vessels (in the absence of other locallesion) remains debatable.

Both cardiovascular-renal and cerebrovasculardisease in Norway declined during the war. A com-mon factor ust therefore be soughtperhaps the

lowering of blood-pressure-3. Another explanation ofthe declines before and during the war might bea continued fall in rheumatic heart-disease, thoughthis explanation seems unlikely to explain complete-ly the decrease in cerebrovascular disease. Thediscrepancy after the war might be due to diff-erent latent periods in the action of exogenous fact-ors on cerebral and cardiac vascular disease.Atheromatosis may develop more slowly in cerebralthan in myocardial vessels but this has not beendemonstrated.

This discrepancy between rates for heart-diseaseand vascular lesions of the nervous system hasbeen found elsewhere, for example, in Scotland andin America, where the war had no apparent effecton cerebrovascular disease nor, in the United Sta-tes, on cardiovascular disease, which makes thefall in Norway even more interesting.In a study of the prognosis in cerebral thrombo-

sis Robinson et al6 found that the initial attackwas fatal in 21 % of 1018 cases. Of the survivors50 % died within 4.1 years (as compared with18 % of a comparable sample of the general popu-lation), recurrent vascular disease accounting for85 % of the deaths. Severe initial attacks, earlyrecurrence, and congestive cardiac failure, indic-ated a poor prognosis. There were no importantdifferences in the incidence of coronary disease,diabetes mellitus, or hypertension between thosewho survived and those who died in the first attack.

Clearly much more attention must be paid to anal-ysis of factors contributing to cerebrovasculardisease as distinct from cardiovascular disease in

general. The fact that the causes of both are atpresent obscure need be no deterrent to clear think-ing during the period of elucidation : indeed it isan inducement to eschew prejudice. It is a com

monplace that vascular disease may ravage thebrain or the heart in one individual to differentdegrees. It may not be surprising that statisticsreflect this fact : perhaps they may even one dayexplain it.

PUERPERAL SEPSIS

1. Colebrook, L., Kenny, M. Lancet, 1936, ii, 1319.2. Gibberd, G. F. J. Irish med. Ass. 1959, 44, 126.3. Gibson, J., Calman, R.M. Lancet, 1953, i, 320.4. Boissard, J. M., Eton, B. Brit. med. J. 1956, ii, 574.5. Kwantes, W., James, J. R.E., ibid. 1956, ii, 576.6. Pollard, B.R., Perry, S.E. Mon. Bull. Minist. Hlth Lab, Serv.

1959, 18, 46.7. Hare R.. Ridley, M. Brit, med. J. 1958, i. 69.8. Calman, R. M., Gibson, J, Lancet, 1953, ii, 649.

DEATHS from puerperal sepsis are now veryuncommon: there have been none in the past ten

years in two London hospitals where, in the earlythirties, it was responsible for a maternal mortalityof 1 in 1000. The most obvious reasons are theintroduction of sulphonamides in 1936, penicillinin 1945, and other antibiotics since then. Theirchief effect was to remove the killing power ofthe Lancefield group-A streptococcus, which hadbeen responsible for 75 % of deaths from puerperalsepsis. But there were other causes for the impro-vement. Colebrook and Kenny I, for example, showedthat in one London hospital the fatality-rate frompuerperal sepsis due to this organism fell from 25 %to 5 % in the three years before sulphonamides werebrought into use. This may have been partly theresult of a decrease in the virulence of the organism,but an important cause was the researches into thebehaviour of streptococci and into air hygiene ingeneral. As Gibberd has said 2, "This new knowledgeand its practical application produced, in the bestdepartments, a standard of organisation and bact-eriological control that is not always seen in thesecarefrec (or careless) days of antibiotics". In thesame way, there was a fall in the mortality frominfection due to organisms which were not affectedby the sulphonamides, before the introduction ofpenicillin. These bacteria (anaerobic and non-hæmo-lytic streptococci, Clostridium welchii, and Esche-richia coli) may all be present in the normal vaginain the puerperium and become invasive only in thepresence of devitalised tissue. The decrease in theincidence of puerperal infection by these organismsis, in Gibberd’s opinion, due to improvements inobstetric technique. Deliveries are less traumaticthan they were twenty years ago and special atten-tion is now paid to the danger of such proceduresas instrumental induction of labour when the foetusis dead.

The fall in mortality from puerperal sepsis is thusattributable to real advances in preventive methods.both bacteriological and obstetric, and to the use

of antibacterial substances. These substances do notprevent the spread of potentially dangerous organ-isms round a maternity unit, although they decreasethe severity of the disease. Indeed, virulent group-Astreptococci were shown to have spread widely inmaternity hospitals 3-6, and there is also the present-day plague of the antibiotic-resistant staphylococcus.The staphylococcus used to be much less dangerousthan the streptococcus, but now our hospitals containstrains which are highly virulent and fully invasive.Hare and Ridley 7 showed that the perineum is, inthe male, a not uncommon site for symptomlesscarriage of Staphylococcus aureus. Although thecomparable carriage-rate in the female has not beenrecorded, it seems likely that this organism cannotbe far from the birth canal of women confined in

hospital. In 1951 Calman and Gibson 8 found that16.5 (/0 genital-tract infections were caused by Staph.aureus: more up-to-date figures are not available. Itmay be that conditions in the uterus after a normalconfinement are not suitable for the growth of thisorganism; but if Staph. aureus is not responsible foran increasing amount of puerperal uterine infection,the reason does not lie in its scarcity in the envi-ronment.