Mottled chest radiograph and gas transfer defect

Transcription

1 Thorx (1972), 27, 315. Mottled chest rdiogrph nd gs trnsfer defect in chronic liver disese N. N. STANLEY1 nd D. J. WOODGATE2 Deprtments of Medicine nd Crdiology, The Royl Free Hospitl, London N.W.3 In prospective study of 17 ptients with vrious types of chronic liver disese, pulmonry gs trnsfer defect ws found in 2% nd mottled shdowing in the chest rdiogrph in 6%. The presence of these bnormlities ws not relted to the cuse of the liver disese. Reduction of trnsfer fctor in liver disese ws not ccompnied by restrictive ventiltory defect nd ws found in most cses wit-h mottled rdiogrphs. The incidence of finger clubbing nd the levels of both venorteril dmixture nd crdic output were higher in ptients with mottling thn in those with norml rdiogrphs. Mottling ws lso seen in the chest rdiogrphs of five other ptients with heptic cirrhosis who hd previously been investigted for cynosis due to intrpulmonry shunting. Despite erlier reports of ctive chronic heptitis nd primry biliry cirrhosis occurring in ptients with fibrosing lveolitis, we suggest tht these rdiogrphic chnges in liver disese re usully cused by pulmonry vsculr disorder, rther thn by coincidentl lung disese, nd re locl mnifesttion of generlized vsodilted stte. The low incidence of diffuse lung disese complicting chronic liver disese ws confirmed by reviewing the hospitl necropsy records. In one ptient the rdiogrphic mottling disppered nd the physiologicl evidence of circultory disorder becme less mrked during period of improved heptocellulr function. Some of the crdiorespirtory disorders in chronic liver disese (CLD) hve received considerble ttention. They include hypoxemi due to intrpulmonry rteriovenous shunts (Rydell nd Hoffbuer, 1956), incresed crdic output (Kowlski nd Abelmnn, 1953), respirtory lklosis (Vnmee et l., 1956), nd pulmonry hypertension (Senior et l., 1968). It is less well known tht mottled shdows my sometimes be seen t the lung bses on chest rdiogrphs (Chrispin nd Lessof, 1965); post-mortem studies hve suggested tht these re due to dilted precpillry pulmonry blood vessels (Berthelot, Wlker, Sherlock, nd Reid, 1966). Isolted cses of CLD hve lso been reported with reduced pulmonry trnsfer fctor (Krlish, Mrshll, Reid, nd Sherlock, 1967; Cotes, Field, Brown, nd Red, 1968; El Gml, Stoker, Spiers, nd Whitker, 197). Rdiogrphic mottling nd gs trnsfer defect re both chrcteristic fetures of diffuse lung disese nd it hs recently been reported tht fibrosing lveolitis is occsionlly ssocited with both ctive chronic lpresent ddress: Hospitl of the University of Pennsylvni, Phildelphi. P U.S.A. 2Present ddress: St. Andrew's Hospitl, Billericy, Essex. 315 heptitis (Turner-Wrwick, 1968) nd primry biliry cirrhosis (Mson, Mclllmurry, Golding, nd Hughes, 197). We present in this pper the results of pulmonry investigtions in 17 ptients with CLD. Specil ttention is given to the physiologicl findings in CLD with mottled chest rdiogrphs nd these re compred with the physiologicl pttern seen in diffuse lung disese. The incidence of diffuse lung disese in CLD ws lso determined by use of hospitl necropsy records. MATERIALS AND METHODS We investigted 17 ptients with CLD who hd not been ssessed from respirtory viewpoint beforehnd (the prospective study). The dignosis of the liver disorder hd been bsed on the clinicl picture, liver biopsy, nd immunologicl sttus. Seventy-three hd ctive chronic heptitis, 37 primry biliry cirrhosis, nd 27 lcoholic cirrhosis; 33 hd cirrhosis which ws cryptogenic or of other etiology. Cses with severely decompensted liver disese, scites or chronic bronchitis were excluded. Clinicl evidence of pulmonry disturbnce ws sought by full history nd physicl exmintion of the chest. Dyspnoe ws grded ccording to the revised Medicl Reserch Thorx: first published s /thx on 1 My Downloded from on 26 October 218 by guest. Protected by copyright.

2 316 N. N. Stnley nd D. J. Woodgte Council's questionnire on respirtory symptoms'. Recent chest rdiogrphs were studied with the ptients' identities conceled from the exminer. Vitl cpcity (VC) ws mesured by dry spirometer (Vitlogrph). Pulmonry trnsfer fctor for crbon monoxide (TF) ws mesured by the single breth method of Ogilvie, Forster, Blkemore, nd Morton (1957) modified by using the clcultion of brethholding time proposed by Jones nd Mede (1961) nd of lveolr volume from the dilution of helium by the test breth. Mesured vlues of TF were corrected for the effect of nemi by the regression formul of Dinkr et l. (197). Twenty-six ptients with CLD consented to further physiologicl investigtions fter full explntion of their nture nd purpose. Detils of these procedures re given elsewhere (Stnley nd Woodgte, 1971). In brief, simultneous collections were mde of rteril blood, mixed venous blood, nd expired ir while brething ir nd oxygen successively in the supine position. Gsometric nlysis of these smples llowed determintion of the lveolr-rteril 2 differences (A-Do2) by the lveolr ir eqution, crdic output (Qt) by the Fick principle, nd venorteril dmixture (Qv) by the stndrd shunt formul. For comprison VC, TF, A-Do2, nd Qv were determined in eight successive ptients ttending this hospitl with diffuse lung disese; four hd fibrosing lveolitis nd four hd pulmonry srcoidosis. Mixed venous blood ws not smpled in these subjects, but 'Obtinble from W. J. Holmn, Ltd., Dwlish, Devon TABLE I FINDINGS IN THE PROSPECTIVE STUDY n rteriovenous 2 difference of 4 5 vol % ws ssumed for the clcultion of Qv. VC nd TF were expressed s percentges of the predicted vlues for the ptients' heights nd ges given by Cotes (1968). Approximte 95% confidence limits for these mesurements were set by the use of Cotes' estimtes of their stndrd devitions in helthy subjects. Thus, norml rnges for VC nd TF were ssumed to be % nd 7-13% of the predicted vlues respectively. The men VC nd TF of 2 norml control subjects studied in this lbortory were 99% nd 11% of these predicted vlues. Gsometric dt obtined in 14 helthy volunteers, whose men ge ws 42 yers, were lso used to estblish the following norml rnges: rteril Po mmhg, A-Do2 brething mbient ir less thn 25 mmhg, nd Qv less thn 3% of the crdic output. We lso reviewed the rdiogrphs nd physiologicl dt of five ptients with heptic cirrhosis previously seen t our hospitl who hd been investigted for severe rteril unsturtion (the retrospective study). The necropsy records provided informtion on the pulmonry histology of 12 ptients with CLD who died during the period They included 32 with ctive chronic heptitis nd 18 with primry biliry cirrhosis: their lung sections were re-exmined by light microscopy nd evidence of fibrosing lveolitis ws sought. These ptients lso included 11 whose lung function hd been ssessed in life, 1 in the prospective study nd one in the retrospective study. Low Trnsfer Fctor Cuse of CLD No. of Men Dyspnoe Rles Mottled Cses Age (uncorrected (corrected Chest (yr) for nemi) for nemi) Rdiogrph Active chronic heptitis (18%) 2 (27%) 15 (21 %) 3 Primry biliry cirrhosis (32%) 1 (27%) 7 (19%) 2 Alcoholic cirrhosis (19%) 6 (22%) 5 (18 %) 1 Other cuses of cirrhosis (3%) 2 13 (39 %) 8 (24%) 3 Totl (23%) 2 49 (29%) 35 (2%) 9 TABLE II FINDINGS IN CASES OF CHRONIC LIVER DISEASE WITH MOTTLED CHEST RADIOGRAPHS PRESENTING IN THE PROSPECTIVE STUDY Liver Disese Alv.-rt. Venorteril Cse Age - Dyspnoe Clubbing Vitl Trnsfer Arteril,, Diff. Crdic Admixture No. (yr) Cuse Durtion Grde Cpcity Fctor1 Tension Brething Index (Y. crdic (yr) (% predicted) (% predicted) (mmhg) Air (mmhg) (1/min/m') output) 1 17 Fsciol ACH Crypt Crypt PBC * Alc ACH _ ACH PBC Fsciol.=heptic fsciolisis; ACH=ctive chronic heptitis; Crypt.=cryptogenic cirrhosis; PBC=primry biliry cirrhosis; Aic.=lcoholic cirrhosis 'Corrected for effect of nemi Thorx: first published s /thx on 1 My Downloded from on 26 October 218 by guest. Protected by copyright.

3 Mottled chest rdiogrph nd gs trrnsfer defect in chronic liver disese 317 RESULTS THE PROSPECTIVE STUDY The findings re summrized in Tble I. Dyspnoe ws common, lthough usully mild (grde I). More severe dyspnoe (grdes II to IV) ws present in 5%. Crepittions were herd in only two ptients nd on clinicl grounds were ttributed to hert filure rther thn to lung disese. The mesured levels of TF were low in 29 % nd in 2% fter correction for nemi. Mottled rdiogrphs were seen in 6%. The incidence of reduced TF nd bnorml rdiogrphs ws not relted to the type of liver disese: in prticulr, these disturbnces were not more frequent in cses of ctive chronic heptitis or primry biliry cirrhosis. Findings in cses with mottled rdiogrphs (Tble II) A striking spect of the liver disese ws its long durtion in most cses. Eight of these nine ptients hd clubbing, compred with n incidence of only 8% in the 161 ptients with norml rdiogrphs. Four were mildly dyspnoeic, but none hd clinicl evidence of lung disese. Erythrocyte sedimenttion rte ws vrible but often norml. Abnorml immunologicl tests were found only when pproprite to the underlying liver disese. Physiologicl comprisons between CLD nd diffuse lung disese Reduction of TF in CLD ws often mrked in those with rdiogrphic mottling (Fig. 1). Indeed, cses of CLD with bnorml rdiogrphs could not be distinguished -6 ) I- 15 -,5- I chronic liver disese diffuse norml X-ry mottled X-ry lung disese./x. lw v %O FIG. 1. Pulmonry trnsfer fctor (TF) in cses ofchronic liver disese, with norml or mottled chest rdiogrphs, nd in cses ofdiffuse lung disese. TF vlues re correctedfor nemi nd their rnge is indicted by the men vlue 2 S.D. in the cses of liver disese with norml rdiogrphs. 4 cr O EE.n rnorml rnge < from cses of diffuse lung disese by mesurement of TF. By contrst, VC ws virtully norml in CLD irrespective of the rdiogrphic ppernce but tended to be low in cses of lung disese (Fig. 2). vs 15 E._ U o rnge FIG. 2. Vitl cpcity (VC) in cses of chronic liver disese with norml or mottled chest rdiogrphs, nd in cses of diffuse lung disese. The rnge of VC in cses of liver disese with norml rdiogrphs is indicted by the men vlue +2 S.D. The levels of rteril P2 nd A-Do2 in cses of CLD with mottled rdiogrphs nd ptients with diffuse lung disese were similr (Fig. 3). Crdic u - IV (v, -"=E > ' E FIG. 3. Arteril 2 tensions nd lveolr-rteril 2 differences brething room ir in cses of chronic liver disese with norml or mottled chest rdiogrphs, nd in cses ofdiffuse lung disese. indices (Fig. 4) were usully higher in cses of CLD with mottled shdowing thn in ptients with Thorx: first published s /thx on 1 My Downloded from on 26 October 218 by guest. Protected by copyright.

4 318 norml chest rdiogrph. An increse of Qv ws common in CLD but ws present in only one ptient with diffuse lung disese (Fig. 5). However, it ws especilly striking tht Qv ws higher in six of the seven cses of CLD with mottled shdowing thn in ny of the other ptients. 4) E L- x cv ~ norml chest X-ry I~~~~~ evi IC 1,~~~~~~~~~~~~ N. N. Stnley nd D. J. Woodgte mottled chest X-ry FIG. 4. Crdic indices in cses of chronic liver disese with either norml or mottled chest rdiogrphs. ' 15 u 2O._ L.._.E -o - 4,~ 5 - norml rnqe FIG. 5. Levels ofvenorteril dmixture in cses ofchronic liver disese with norml or mottled chest rdiogrphs, nd in cses of diffuse lung disese. cses of CLD reveled wide vriety of common lung diseses, such s chronic bronchitis, emphysem, nd pneumoni, but no evidence of fibrosing lveolitis ws seen. Two cses of primry biliry TABLE III FINDINGS IN CASES OF CHRONIC LIVER DISEASE PRESENTING IN THE RETROSPECTIVE STUDY Liver Disese Venorteril Cse Age Dyspnoe Vitl Trnsfer Arteril 2 Admixture No. (yr) Cuse Durtion Grde Cpcity Fctor' Tension (Y. crdic (yr) (% predicted) (% predicted) (mmhg) output) 1 6 Alc Crypt (84 /) Crypt Crypt ACH 'Corrected for effect of nemi 2%, Arteril sturtion (mesured by mnometric Vn Slyke pprtus) ACH =ctive chronic heptitis; Alc. =lcoholic cirrhosis; Crypt. =cryptogenic cirrhosis C C chronic liver disese diffuse norml X-ry mottled X-ry lung S 2 ~~~ Thorx: first published s /thx on 1 My Downloded from THE RETROSPECTIVE STUDY The findings in the five ptients previously investigted becuse of rteril desturtion re shown in Tble III. The liver disese ws of no prticulr etiology but hd usully been of long durtion. Cse S hd chronic bronchitis nd bronchiectsis, but the remining four ptients hd no clinicl evidence of lung disese lthough they ll complined of dyspnoe. All hd cynosis, finger clubbing, nd high levels of venorteril dmixture. Retrospective exmintion of their chest rdiogrphs reveled mottling in every cse. Although none hd significnt reduction of vitl cpcity, ll hd considerble impirment of gs trnsfer. Histologicl study The necropsy findings of 12 cirrhosis hd widespred intrpulmonry grnuloms; mrked rdiogrphic mottling hd been observed in one of these ptients (cse 9 in Tble II) nd full detils will be reported elsewhere. Lung tissue ws lso vilble in second cse with mottling nd reduced TF (cse 1 in Tble III). This showed pncinr emphysem which hd not been suspected cliniclly. However, this emphysem could not hve produced the rdiogrphic chnges, lthough it ws possibly responsible for the gs trnsfer defect. The pulmonry vessels hd not been injected nd no useful comments could be mde bout their structure. No pulmonry disese ws found in two other cses in whom vlues of TF below 6% of predicted norml hd been mesured during life. on 26 October 218 by guest. Protected by copyright.

5 (b) rx _F z (). i... s w... _Mw.v.% zsr _Fj j :B. _b ::: r.s_7jv_ j ' x>-., ; ;,. R Wu e si X.X z_ e lt.s i:. 1 ; w Mottled chest rdiogrph nd gs trnsfer defect in chronic liver disese. j=-.v i_w! ::. l. i. _1: _- L i io Ej z w J i w x_ ; :- jb b s-b... 2) I._# F_ 8... H. w r w4ms =... _ o _f.' S _. n :. i. 2.: * X E x. 319 Thorx: first published s /thx on 1 My Downloded from z Fio. 6. Cse 1. Chest rdiogrphs () September There re corse nodulr shdows which re most mrked in the right lower zone. The hilr vsculr shdows re enlrged nd the peripherl vsculr mrkings re ccentuted. (b) Detil of the right lower zone showing the nodulr shdows. on 26 October 218 by guest. Protected by copyright.

6 32 N. N. Stnley nd D. J. Woodgte Chest rdiogrphs The mottling ws lwys bilterl with nodulr shdows which were most pronounced in the lower lung zones, but in detil there were two distinct ppernces. Usully the nodules were soft, corse (1-4 mm), irregulr, nd superimposed on cler bckground. An ssocited feture ws ccentution of the pulmonry vessels both in the hilr regions nd peripherlly. The smll vessels were especilly prominent t the costophrenic ngles where they often resembled septl lines, but liner shdows in this region could lwys be trced centrlly to join other vessel mrkings nd never extended lterlly to touch the lung mrgin. These ppernces were noted in cses 1 to 8 in the prospective study nd in ll the cses in the retrospective study. The most obvious chnges of this type were seen in cse 1 (Fig. 6, b). A second type of shdowing ws seen in cse 9 of the prospective study. The nodules were much finer nd in the right lower zone colesced to form ground-glss hze. The vsculr mrkings were norml. She ws the only cse of CLD with nodulr shdowing who hd norml level of crdic index nd venorteril dmixture. At necropsy she ws found to hve diffuse intrpulmonry grnuloms. CASE REPORT Cse 1 is reported in detil primrily to demonstrte tht the rdiogrphic shdowing nd some of the ssocited physiologicl chnges my resolve during clinicl remission of the underlying liver disese. The cse lso shows tht incresed venorteril dmixture is not the sole cuse of rteril hypoxemi in CLD. This young mn hd been dmitted to hospitl in 1959 when ged 7 yers. He hd heptosplenomegly nd blood eosinophili of 15,/mm3. Liver biopsy showed cirrhosis nd infiltrtion of the portl trcts with eosinophils nd lymphocytes. Investigtions for prsitic infesttion including Fsciol heptic were negtive. A portcvl shunt ws performed in 1961 for recurrent gstrointestinl bleeding. Finger clubbing nd incresed vsculr mrkings on chest rdiogrphy were noted in The next yer he developed precom; he responded initilly to low protein diet, but in July 1968 he required dditionl neomycin therpy to prevent recurrent encephlopthy. Over this period ( ) his effort tolernce slowly deteriorted until he ws unble to hurry on level ground. In September 1968 he ws trnsferred to the Royl Free Hospitl for further ssessment. Physicl exmintion showed smll, icteric, nd ill-looking youth. There ws gross clubbing of the fingers nd toes. His pulse (76/minute) ws bounding. Blood pressure (11/65 mmhg) nd venous pressure were norml. The second hert sound in the pulmonry re ws ccentuted but normlly split. An ejection systolic murmur ws udible over the whole precordium. There ws no peripherl oedem. Exmintion of the lungs ws norml. The liver ws enlrged, but the spleen ws implpble nd there ws no scites. Lbortory studies included the following: Hemoglobin 1-8 g/1 ml; WBC 17,5/mm3 with polymorphs 11,55, lymphocytes 4,9, monocytes 875, nd eosinophils 175; nd ESR 116 mm/hour; totl serum bilirubin 14 mg/1 ml, lkline phosphtse 9 iu/l, sprtte trnsminse 9 iu/l, lbumin 1-8 g/1 ml nd globulin 5-6 g/1 ml. In the serum the Fsciol heptic complement fixtion test hd become strongly positive (dilution 1:32). Chest rdiogrphy showed prominent hilr nd peripherl vsculr mrkings with widespred nodulrity in both lower nd middle lung zones (Figs. 6, b). The electrocrdiogrm ws norml. Crdiorespirtory dt re given in Tble IV. Vitl cpcity ws within norml limits, but trnsfer fctor TABLE IV CASE 1: CARDIOPULMONARY DATA Norml September 1968 My 197 Vlues Chest rdiogrph Hevy mottling No mottling Trnsfer fctor' 12 (16) 12 (16) (ml/min/mmhg) Vitl cpcity 3,9 4,2 4,1-4,72 (Ml) Arteril oxygen tension > 78 (mmhg) Alv.-rt. 2 difference <25 brething ir (mmhg) Venorteril dmixture 11 2 < 3 (% crdic output) Crdic index (1/min/m2) Pulmonry rteril 54/16 48/16 < 32, 13 pressure (mmhg) 'Vlues in prentheses re corrected for effect of nemi 2Lower vlues for 1968, higher vlues for 197 ws very low even fter correction for nemi (54% predicted norml). A-Do2 brething room ir ws 41 mmhg. It ws clculted tht the venorteril dmixture ws 11% of the crdic output, which must hve contributed lrge prt of the rteril hypoxemi. Mesurements of pressure nd sturtion t different sites during right hert ctheteriztion reveled no evidence of congenitl hert disese nd the systolic murmur ws ttributed to the high crdic index (6-2 1/min/m2). Although dignosis of heptic fsciolisis hd not been fully substntited, his deteriorting condition seemed to wrrnt tril of specific therpy for this condition nd he ws given course of emetine hydrochloride. Over the next 18 months the heptic filure lessened nd his effort tolernce improved. Dietry restrictions nd neomycin therpy were withdrwn nd he hd no recurrence of encephlopthy. The totl serum bilirubin fell to 7 5 mg/1 ml nd the serum lbumin level rose, lthough vrying widely from month to month (2-3 5). Thorx: first published s /thx on 1 My Downloded from on 26 October 218 by guest. Protected by copyright.

7 Mottled chest rdiogrph nd gs trnsfer defect in chronic liver disese r FIG. 7. Cse 1. Chest rdiogrph My 197. The nodulr shdows nd incresed peripherl vsculr mrkings re no longer present. The hilr vsculr shdows re less prominent thn previously. He ws redmitted in My 197 for further crdiopulmonry investigtion nd the findings re shown in Tble IV. The rteril 2 tension hd risen somewht, together with reduction of the elevted A-mxs brething ir. Furthermore, the level of venorteril dmixture hd fllen to within norml limits. Both crdic index nd pulmonry rtery pressure were lower thn previously. The chest rdiogrph (Fig. 7) no longer showed bsl nodulr shdowing or incresed peripherl vsculr mrkings nd the hilr vsculr shdows were less prominent. The TF hd not improved nd it seemed likely tht the remining hypoxemi ws lrgely due to ventiltion-perfusion inequlity, which my lso occur in cirrhosis, s described by Cotes et l. (1968). The pulmonry hypertension, lthough extensively documented in liver cirrhosis (Senior et l., 1968), remins unexplined. DISCUSSION Since pulmonry gs trnsfer hs often been regrded s norml in liver disese, we hve reviewed the mesurement of trnsfer fctor in 321 heptic cirrhosis given by other workers. Reduced TF hs been found in five previous cses studied by the single breth method (Krlish et l., 1967; Cotes et l., 1968; El Gml et l., 197). The stedy stte TF ws lso very low in one out of five cses reported by Blckburn et l. (196). Using the hypoxic brething procedure the trnsfer fctor for oxygen hs been clculted in 27 cses (Willims, 196; Heinemnn, Emirgil nd Mijnssen, 196; Abelmnn, Krmer, Verstreten, Grvllese, nd McNeely, 1961; Chies, Cippi, Blbi, nd Chindussi, 1969): mong these were eight with vlues below 15 ml/min/mmhg, which hs been given s the lower limit of norml (Comroe et l., 1962). Thus our vlues of trnsfer fctor gree with erlier dt, nd the occurrence of impired gs trnsfer in CLD seems well estblished. In cses of CLD without scites decresed TF is not usully ssocited with ny restrictive ventiltory defect. This contrsts with most lung diseses in which decresed TF tends to be ccompnied by smll vitl cpcity. The Thorx: first published s /thx on 1 My Downloded from on 26 October 218 by guest. Protected by copyright.

8 322 level of TF provided useful index of the overll efficiency of pulmonry gs exchnge in CLD s cses with diminished crbon monoxide uptke usully hd n incresed A-Do2 brething room ir. Severl possible mechnisms my limit gs trnsfer in CLD. In some ptients nemi reduces the mss of hemoglobin in the pulmonry cpillries vilble for combintion with crbon monoxide. Coincidentl lung disese my be present in others. However, the level of TF corrected for the effect of nemi ws often low when lung disese ws not pprent cliniclly or, in two cses, t subsequent necropsy. Cotes nd his collegues (1968) suggested tht trnsfer limittion in their ptients might hve reflected decresed surfce re for gs exchnge produced by uneven pulmonry blood flow. Although more recent work with rdioctive xenon hs shown tht the predominnt regionl inequlity of lung function in cirrhosis resides in the distribution of ventiltion rther thn blood flow (Ruff et l., 1971), it remins possible tht some pttern of pulmonry inhomogeneity my be responsible for the reduction of TF in liver disese. The bsl nodulrity sometimes pprent on chest rdiogrphy hs been ttributed to dilttion of smll pulmonry blood vessels (Berthelot et l., 1966); this would lso be in keeping with the observed ccentution of vsculr mrkings. The high crdic outputs giving incresed pulmonry flow rtes in the cses with nodulr shdows provided n dditionl reson for their incresed vsculr plethor. A physiologicl consequence of this pulmonry vsodilttion ws incresed venorteril dmixture, presumbly due to opening of multiple intrpulmonry rteriovenous nstomoses. The incresed venorteril dmixture, hyperkinetic circultion, nd finger clubbing often seen in the cses with rdiogrphic shdows my be concurrent mnifesttions of generlized vsodilted stte in CLD (Stnley nd Woodgte, 1971). It is thus of specil interest tht ll these signs regressed in one ptient during period of clinicl improvement. Remission of cynosis due to intrpulmonry shunting in liver disese hs previously been reported in child (Silvermn, Cooper, Moller, nd Good, 1968) nd it ppers tht t lest some of the circultory chnges cused by CLD re not immutble. Alterntively, the rdiogrphic nodulrity could be relted to interstitil oedem; this hs been suggested s the cuse for hypoventiltion of the dependent lung zones found with rdioctive xenon in heptic cirrhosis (Ruff et l., 1971). However, true Kerley B lines were not seen in ny of our cses with nodulrity. N. N. Stnley nd D. J. Woodgte Finlly, one clinicl spect must be stressed. Fibrosing lveolitis is occsionlly ccompnied by ctive chronic heptitis or primry biliry cirrhosis, nd in cses of CLD with both rdiogrphic mottling nd reduced TF dignostic problem my sometimes rise. For exmple, the first cse report of CLD with low single breth TF (Krlish et l., 1967) concerned young mn with ctive chronic heptitis: his chest rdiogrph showed nodulr shdowing which ws initilly ttributed to fibrosing lveolitis, but precpillry vsodilttion ws the only pulmonry disorder found t necropsy. In our cses with this combintion of physiologicl nd rdiogrphic disturbnces we hve considered the overll picture to be unlike fibrosing lveolitis, but without histologicl proof this opinion must be gurded. Although differences between CLD with rdiogrphic mottling nd diffuse lung disese in the mesurements of vitl cpcity nd venorteril dmixture were observed, their clinicl importnce is probbly limited. The vitl cpcity in CLD my be reduced by scites (Abelmnn, Frnk, Gensler, nd Cugell, 1954) nd incresed venorteril dmixture my occur in dvnced diffuse lung disese (Arndt, King, nd Briscoe, 197). Indeed, the exclusion of pulmonry disese in some cses of heptic disese my not be possible without lung biopsy, which my be hzrdous due to cogultion deficiencies. It is thus fortunte tht our necropsy mteril confirms tht the incidence of fibrosing lveolitis in CLD is truly rre. We wish to thnk Professor Sheil Sherlock for permission to study ptients under her cre nd Dr. Frnces Grdner for llowing us to use the equipment of the Deprtment of Crdiology. Also we grtefully cknowledge the skilled technicl ssistnce given by Mr. Donld Wgstff, Mrs. Crol Shw, nd Miss Hrriet Coppermn. REFERENCES Abelmnn, W. H., Frnk, N. R., Gensler, E. A., nd Cugell, D. W. (1954). Effects of bdominl distention by scites on lung volumes nd ventiltion. Arch. intern. Med., 93, 528. Krmer, G. E., Verstreten, J. M., Grvllese, M. A., nd McNeely, W. F. (1961). Cirrhosis of the liver nd decresed rteril oxygen sturtion. Arch. intern. Med., 18, 34. Arndt, H., King, T. K. C., nd Briscoe, W. A. (197). Diffusing cpcities nd ventiltion: perfusion rtios in ptients with the clinicl syndrome of lveolr cpillry block. J. clin. Invest., 49, 48. Berthelot, P., Wlker, J. G., Sherlock, S., nd Reid, L. (1966). Arteril chnges in the lungs in cirrhosis of the liverlung spider nevi. New Engi. J. Med., 274, 291. Thorx: first published s /thx on 1 My Downloded from on 26 October 218 by guest. Protected by copyright.

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