SYSTEMIC CAPILLARY LEAK SYNDROME

330 DESTITUTION AT THE FESTIVE SEASON

RHEUMATOID ARTHRITIS AND COELIAC DISEASE

SIR,-Dr Weller and colleagues (Jan 28, p 220) report preliminary findings from their Christmas and New Year survey. We add our experience. Over five days around Christmas, 1988, about 1000 of London’s homeless people spent some time at a shelter provided by the charity Crisis at Christmas and 375 visited the on-site medical centre, which was open all day. Most of the patients attending the medical centre answered a

SIR,-Dr O’Farrelly and colleagues (Oct 8, p 819) highlight the complex relation between bowel and joint disease. Professor Corbeel and colleagues’ case-report (Dec 17 p 1420) complicates the issue. They describe a girl who failed to thrive and had daily

questionnaire about their health and

access to

medical

care.

Not all

patients answered all questions. 179 (61 %) of 294 patients admitted to having an alcohol problem and 73 (37%) of 200 had had psychiatric care. Review of medical notes showed 106 (28%) of the 375 patients to have significant illness requiring investigation and/or treatment. (For this analysis alcoholism or psychiatric illness were not regarded as significant, unless there was an acute problem.) 61 (58%) of the 106 patients with significant illness had an acute problem including 11 (10%) with alcohol withdrawal, 11 with trauma, and 11 with respiratory infections or asthma; 6 (6%) had dental problems, and a 51-year-old man had a two week history of dysphagia to solids. 45 cases (42%) had chronic problems, including lice/scabies in 25 (24%), probable duodenal ulceration in 5 (5%), angina in 3 (3%), and a young man with a six month history of fits and headache. Only 144 (43%) of 333 had a general practitioner in London. Patients with or without a general practitioner in London were equally likely to have significant acute (15% vs 19%, respectively) or chronic (14% vs 12%, respectively) illness. The lack of benefit of having a local general practitioner might be because patients ignore their doctor’s advice or because their doctors do not meet the needs of the homeless. We attribute the popularity of the Crisis at Christmas medical centre to the fact that it is open on a walk-in basis. Long opening hours are not essential as 70% of patients came between 0900 and 1800. In addition to the high prevalence of alcoholism and psychiatric illness in this population which Weller and colleagues report (and have reported previously), at least 10% of those attending Crisis at Christmas had significant illness. Similar clinics are needed throughout the year to alleviate the medical problems of the homeless. Central Birmingham Health Authority, Birmingham B15 2TZ

MICHAEL R. BRADDICK

Crisis at Christmas, London E1 1BJ

MIKE TOMSON

BGA, Weller MPI, Coker E, Mahomed S. Crisis at Christmas 1986. Lancet 1987, i: 553-54.

1 Weller

recurrent

fever,

a

rash, and diarrhoea at the age of 1

further flare with arthritis at 8 years. This child clearly has systemic-onset juvenile chronic arthritis (JCA),1 a disease very different from and with a different prognosis from the adult rheumatoid arthritis that O’Farrelly and colleagues describe. The nearest equivalent childhood arthritis is seropositive juvenile rheumatoid arthritis, which is always seropositive within the first year, causes erosive change early, and is unusual at this age of onset.2 Systemic JCA often relapses and remits2 and systemic features usually appear before the arthritis. Follow-up series, at 10-15 years after onset, show no or slight physical limitation in about half the patients; active disease that remits within 5 years has a better prognosis.3 The association of coeliac disease and JCA is not common in childhood although transient episodic polyarthritis has been seen.’ Pinals’ described a girl who had a pauciarticular-type JCA with arthritis of the knees and ankles at the age of 12 years and who was later found to have the small bowel changes of coeliac disease; both conditions responded to a gluten-free diet. This type of arthritis resembles the seronegative spondylarthritis described by Bourne et al,5 who reported 6 patients (youngest 17 years at onset of arthritis) who remitted totally or in part on such a diet. Lumbar spine, hips, knees, and shoulders were commonly affected. 3 similar cases were seen in a series of 314 adult patients with coeliac disease.6 A further 2 had true rheumatoid arthritis. Diet did not appear to affect the arthritis. Seropositive rheumatoid arthritis has been reported by others.7 In only 1 of the 3 cases did the arthritis respond to diet. Thus Corbeel and colleagues’ case is different from the patients in the original study who had no bowel or malabsorption signs, arthritis previously described in coeliac disease, or true rheumatoid arthritis. It is possible that Corbeel and colleagues’ case is a chance association of an often naturally remitting disease and coeliac disease. Whilst the association is of interest, it is difficult to draw conclusions that would be applicable to adults. Department of Rheumatology and Rehabilitation, Northwick Park Hospital, Harrow, Middlesex HA1 3UJ 1. Munthe senes

ed. The 3: 43.

E,

care

3. 4.

SiR,—In the UK there are now many one-person-operated buses with driver-controlled exit doors. Most of these doors close with considerable force and do not spring back or give a warning to the driver if they encounter an obstruction. Arms, legs, coats, and shoulder-bags have been trapped in this way. If the driver is unaware of what has happened, he may move off dragging his ex-passenger along, to be injured or even killed. This horrific form of accident is not rare. Inquiries that I have made since my mother was disabled in this way a few months ago suggest that the figure is likely to be many hundreds a year. The tragedy is that these accidents need never occur. It would be easy for the Government to insist that bus-doors were fitted with safety devices in the same way as underground trains or lifts. Although the problem has been recognised for some time, nothing has been done. I appeal to colleagues to join me in urging action through their Members of Parliament and to send me, in confidence, information about any such accidents they have encountered. Department of Child Health, Southmead Hospital, Bristol BS10 5NB

PETER M. DUNN

5. 6. 7.

RICHARD HULL

of the rheumatic child. Basle: EULAR, 1978:

2. Ansell BM Rheumatic disorders in childhood. In Apley J, ed.

BUS-DOOR ACCIDENTS

year and who

diagnosed as having coeliac disease. There was a further with raised immunoglobulins at 2 years before the flare systemic onset of arthritis 6 months later. She was well by 5 years but had a had been

monograph

Postgraduate paediatric

series. London: Butterworths, 1980: 41-151. Hull RG. Outcome in juvenile arthritis. Br J Rheumatol 1988; 27 (suppl 1): 66-71 Pinals RS Arthritis associated with gluten sensitive enteropathy. J Rheumatol 1986, 13: 201-04. Bourne JT, Kumar P, Huskisson EC, Mageed R, Unsworth DJ, Wojtuleski JA Arthritis and coeliac disease. Ann Rheum Dis 1985; 44: 592-98. Cooper BT, Holmes GKT, Cooke WT. Coeliac disease and immunological disorders. Br Med J 1978; i: 537-39. Parke AL, Fagan EA, Chadwick VF, Hughes GRB. Coeliac disease and rheumatoid arthritis. Ann Rheum Dis 1984; 43: 378-80.

SYSTEMIC CAPILLARY LEAK SYNDROME

SIR,-Dr Fellows (Nov 12, p 1143) and Dr Ewan and colleagues and Dr Doorenbos (Dec 24/31, p 1496) report their experience with the systemic capillary leak syndrome. We too have managed a patient with this condition. Our patient, a man aged 46, had symptoms, signs, and investigative findings typical of the systemic capillary leak syndrome, which ran a rapid and fatal course. On eight occasions over 18 months the patient required resuscitation for severe hypovolaemic shock. He died during a particularly severe attack. The characteristic reciprocal relation of elevated haematocrit and reduced serum albumin was observed during attacks. Cl esterase inhibitor activity during and between attacks was normal. An IgG-kappa paraprotein was demonstrated but skeletal survey and

331 bone marrow were normal and urine

was

negative for Bence-Jones

protein. Ewan and colleagues report benefit from plasmapheresis but Cain et all found this unhelpful. There are similar conflicting data about the value of terbutaline. Using prophylactic terbutaline alone Cain et al reported benefit but Garcia-Bragado et al2 found this compound ineffective. After an initially good effect the patient reported by Doorenbos died while receiving prophylactic terbutaline. Doorenbos recommends terbutaline combined with corticosteroids to prevent (32-receptor down-regulation. In one patient in3 whom this combination was used severe attacks still occurred. The variable course of systemic capillary leak syndrome and the unpredictability and self-limiting nature of attacks cloud assessment of therapeutic intervention. Reports of benefit from therapy remain anecdotal; let us hope that through increased awareness, reporting, and collective experience, rational therapy will emerge and the cause of this condition will be determined. Department of Medicine, Western General Hospital, Edinburgh EH4 2XU

S. TEELUCKSINGH P. L. PADFIELD

I. Cain

RJ, Bourgoigme JJ, Yeonsa SA, Schulz DR. Systemic capillary leak syndrome (SCLS)’ a rare cause of systemic edema. Kzdney Int 1986; 29: 181. 2 Garcia-Bragado F, Lima J, Monteagudo M, Manez R, Martinez J, Villar M. Sindrome de hiperpermeabiliadad capilar generalizada: una rara causa de shock hipovolemico y edemas generalizamos ciclicos Apportacion de dos casos. Med Clm (Bare) 1987; 89: 426-28. 3. Lofdahl CG, Solvell L, Laurell AB, Johansson BR. Systemic capillary leak syndrome with monoclonal IgG and complement alterations (a case report on an episodic syndrome) Acta Med Scand 1979; 206: 405-12.

INFECTIVE CAUSE OF CHILDHOOD LEUKAEMIA

SIR,-Dr Kinlen (Dec 10, p 1323) proposes a possible infective for childhood leukaemia. The observation of a raised incidence of childhood (under 15-years-old) leukaemia and nonHodgkin’s lymphoma (NHL) at Seascale near Sellafield from the early 1950s onwards,’ and in the neighbourhood ofDounreay from 1968 onwards,2 led Kinlen to construct a hypothesis related to isolation and population influx. He tests this hypothesis at two locations for the period when the population influx was greatest (1951-67): at Thurso, near Dounreay (because neither registration nor mortality data for the period before 1968 had been examined previously2) and at Glenrothes New Town. The null hypothesis is accepted for childhood (0-14-years-old) leukaemia and NHL mortality at Thurso during 1951-67 (observed [0] =2, expected [E] = 1’ 14) but rejected at Glenrothes (0 = 9, E 3-45). However, as Dr Cartwright and colleagues (Jan 14, p 94) point out, the statistical power of the study must be considered. The number of children in Glenrothes during the period was three times greater than that in Thurso. The consequence of this may be seen by examination of the 95 % confidence intervals for the mortality ratios (O/E) for the two towns: Thurso 0’2-6’3and Glenrothes 1’2-5’C. Thus the mortality ratio for Thurso is not necessarily incompatible with that for Glenrothes and does not therefore invalidate Kinlen’s

which reflected improvements in case survival. In the latter half of the 1960s mortality rates fell less rapidly in the north and west Britain than in East Anglia, London, and the south east. One explanation is that higher radiation doses from fallout in the wetter regions led to a rise in the incidence of leukaemia in these areas and that this was sufficient to offset some of the downward trend in mortality resulting from improving case survival. However, another explanation is that improvements in case survival in the 1960s might have been greater in East Anglia, London, and south-east England. We are seeking data to test this hypothesis, but until this has been done it is too early to draw any conclusions about whether our results support a link between radiation from fallout and childhood leukaemia. Even if further work does indicate such a link this would not mean that the leukaemia excesses near nuclear reprocessing plants are necessarily the result of radiation. One central issue is the wide gap between the small number of leukaemia cases expected on the basis of conventional estimates of radiation risk and the larger number of observed cases. 1-3 We can see nothing in our findings that is likely to narrow this gap substantially. School of Environmental Sciences, University of East Anglia, Norwich NR4 7TJ

GRAHAM BENTHAM ROBIN HAYNES

Investigation of the possible increased incidence of cancer in West Cumbria: report of the Independent Advisory Group. London: HM Stationery Office, 1984. 2. Committee on Medical Aspects of Radiation in the Environment (COMARE). The implications of the new data on the releases from Sellafield in the 1950s for the conclusions of the Report on the Investigation of the Possible Increased Incidence of Cancer in West Cumbna. London: HM Stationery Office, 1986. 3. Committee on Medical Aspects of Radiation in the Environment (COMARE). Investigation of the possible increased incidence of leukaemia in young people near the Dounreay nuclear establishment, Caithness, Scotland. London: HM Stationery Office, 1988. 1.

aetiology

=

hypothesis. Health and Safety Directorate, British Nuclear Fuels plc,

Risley, Warrington,

RICHARD WAKEFORD

Cheshire WA3 6AS 1.

Sir Douglas Black) Investigation of the in West Cumbna. London: HM Stationery

Independent Advisory Group (chairman:

possible increased incidence of cancer Office, 1984. 2. Committee on Medical Aspects of Radiation in the Environment (COMARE) (chairman: Prof M. Bobrow) Second report investigation of the possible increased incidence of leukaemia in young people near the Dounreay nuclear establishment, Caithness, Scotland. London: HM Stationery Office, 1988.

SIR,-We do not agree with Dr Russell Jones’ claim (Jan 14, p 94) that our data remove the remaining scientific obstacle to accepting that radioactive discharges from Sellafield and Dounreay are responsible for the excess cases of leukaemia and lymphoma in the surrounding population. He cites the paper we presented in Coventry on Jan 6, at a meeting of the Institute of British Geographers. We did a regional comparison of trends in childhood leukaemia mortality from 1950 to 1985. For the 0-4 age group from about 1960 onwards there was a downward trend in mortality rates,

SHORT STATURE AND MALNUTRITION IN CYSTIC FIBROSIS

SIR,-We write to clarify some issues raised by your Nov 5 editorial and Dr Tedeschi and colleagues’ letter (Jan 14, p 100) which referred to a chapter written by one of us.’ The term "wasting" in everyday language, and as used with reference to short-term malnutrition and cachectic diseases, describes a dynamic catabolic process which may be regarded as the converse of growth (except that stature is not lost) and thus requires serial measurements. Wasting is also used, as in your editorial, as shorthand for the condition of low body mass for height, which can be shown by a single measurement. The inference of weight loss in these circumstances is not always justified, because in some growth disturbances (such as juvenile hypothyroidism and hyperthyroidism) height increments may proceed at a different rate from accretion of muscle and fat. At the time of diagnosis of infants with cystic fibrosis (CF) low weight for height, and often evidence of weight loss, are easy to observe from clinical appearances, but stunting is less readily demonstrated because of inaccuracies in routine length measurement of newborn and young infants. Tedeschi et al have reported that stunting is present when CF is diagnosed in older children and in 10 out of 14 aged less than 2 years. Most CF children in the UK are diagnosed in the first year of life (British Paediatric Association, unpublished). In a recent report, significant stunting was reported in 20 newly diagnosed infants aged less than 8 months, but the weight deficit was relatively greater than the height deficit.2 There is clear evidence that in advanced CF a chronic catabolic state exists.3 There is also evidence for increased resting energy expenditure in CF which is proportional to impairment of lung function, but appears to be present even when puhnonary function is normal."* Recent studies in young infants with CF and apparently normal lungs also showed increased energy expenditures although this work has not been universally accepted.6 If increased resting energy expenditure is indeed an intrinsic metabolic feature of CF, it might help to explain not only the discrepancy between height and body mass but also the low birthweight in these infants.7,8There are few reliable published data on length at birth of CF babies, but whether they are wasted, stunted, or both, the fact remains that as a