Chase, HP

Fennell, RH

Nora, JJ

Porter, KA

Putnam, CW

Starzl, TE

English

D-Scholarship@Pitt

ORIGINAL ARTICLES \ , . , Obesity, Diet, and Type-II Hyperlipidremia \ B. Leelarthaepin, 'J.sc., J. M. Woodhill, D.SC., A. J. Palmet, F.R.A.C.P., Prof. R. B. Blacket, F.R.C.P. \ Left-ventricular Endomyocardial Biopsy '" 1. A. B. Brooksby, M.R.C.P., B. S. Jenkins, M.R.C.P., D. J. Co~artI M.R.C.P., M. M. Webb-Peploe, M.R.C.P., M. J. Davies, M.R.C.PATR. ' Assessment of the Apgar Score Geoffrey Chamberlain, F.R.C.S., James Banks, F.R.A. Sinus Histiocytosis in British and Japanese Patients with Breast Cancer G. H. Friedel!, M.D., E. A. Soto, M.D., S. Kumaoka" M.D., O. Abe, M.D., J. L. Hayward, F.R.C.S., R. D. Bulbrook, PH.D. PRELIMINARY COMMUNICATION 1225 1228 The Neuropathic Urethra III. .. _ ..... B;.,. Ji.;.; .. :Abel, F.R.C.S., . .-K., Gibbon, F.R.C.S., R. M. Jari:telion, F.R.C.S., K. R. Krishnan, F.R.C.S.E. 1229 The Pineal 1235 The Consultants' Contract 1237 Hot Spots or Hot Lungs? 1238 , \ . t !', HYPOTHESIS . B-cell Neoplasia in Man Prof. S. E. Salmon, M.D., Prof. Maxime Seligmann, M.D. REVIEWS Notices of Books MEDICAL ALLIANCE Substantial or Decorative ? W. J. Bicknell, M.D., Diana Walsh, M.S., Marsha Tanner, M.P.H. SCREENING FOR DISEASE PsYchiatric Disorders Prof. David Goldberg, F.R.C.P. OCCASIONAL SURVEY Nitroblue-tetrazolium Tests A. W. Segal, M.R.C.P. POINTS OF VIEW Your Money or Your Life ? M. S. Rose, M.R.C.PATH. 1230 1234 1241 1245 1248 1253 NAnONAL HEALTH SERVICE The Consultants' Contract 1254 -Genetics of Schizophrenia 1239 Spectinomycin for Gonorrhrea 1239 Eking Out 1240 INTERCEPTED LEITER The Worker's Voice TALKING POLITICS Pegging Public Spending SPECIAL ARTICLE Doctors in Chile LETTERS TO THE EDITOR Research on Dietary Fibre Dr G. A. Spiller, Dr R. J. Amen Urinary Reflux and Chronic Pyelonephritis Dr E. J. Evans Computer-assisted Tomography of the Brain Dr A. S. Bligh and others Minocyc1ine: Possihle Vestibular Side-effects Dr C. S. Nicol, Dr J. D. Oriel Antigenicity of Monocomponent Insulins J. Schlichtkrull Private Beds Prof. David Kerr, F.R.C.P.; Mr Roger Hole, F.R.C.S. The NurseJs Reward-and the Doctor's Dr J. J. Misiewicz The Nurse in the Community Dr Anthony Eustace Jack Becomes Master? Dr Anthony Radford; Dr F. M. Shattock Epidemiology of Crohn's Disease Dr D. S. Miller and others Portacaval Shunt in HyperJipidzmia Prof. T. E. Starzl, M.D., and others PortacBval Shunt in FaDlilial Hypercholesterohemia Shiela Mitchell, Dr R. 1. Levy 1256 1257 1258 1259 1259 1260 1260 1260 1261 1261 1262 1262 1263 1263 1263 The Vulnerable Myocardium. Mr Chnstopher Hassell, B.A • Ischzmic Heart .. disease and Diet Dr E. Bowers Epsilon .. aminocaproic Acid Dr M. D. M. Shaw, Dr J. D. Miller Benign Prostatic Hyperplasia and Cancer of the Prostate Dr R. D. Williams, Dr C. E. Blackard Effects of Hysterectomy Dr D. J. S. Hunter Rauwolfia Derivatives and Breast Cancer Dr E. C. Venezian; Dr G. Cosirola and others Health Services in Chile Prof. Gustavo Molina G., M.D. Respiratory Depression after 1264 1264 1264 1265 1265 1266 1266 Tranquillisers 1267 Dr Renate Huch, Prof. Albert Huch, M.D. Rapid Diagnosis of Arbovirus Infections by Counterelectrophoresis 1267 Dr H. H. Balfour, Jr., Dr C. K. Edelman Frequency of Hepatitis-B Antigen or Antibody in Household Contacts of HBAg Carriers Dr E. G. de la Concha and others Thallium Poisoning Dr Erling Gerdts <Estrogen Synthesis by Human Breast Carcinomas Dr L. de Thibault de Boesinghe and others What is Burkitt's Lymphoma? Dr R. H. Morrow, Jr., and others Cervix Cancer: a Clarification Prof. 1. D. Rotkin, PH.D. Boxers' Brain Damage Mr J. M. Poner, F.R.C.S.; Mr Tony Van den Bergh Nuclear Projections and ChroDlosome 1267 1268 1268 1268 1269 1270 Abnormalities 1270 Prof. J. F. Jackson, M.D., E. G. Clement Grey-scale Ultrasonography . 1271 Dr A. R. Leeds and others Respiratory-distress Syndrome of the Newborn, Reye's Syndrome, and Fatty Acids Dr R. E. Brown, Dr R. M. Drake The Law of the Heart Dr Fulvia Pezza Prophylaxis for Postoperative Venous 1271 1272 Thrombosis :272 Dr V. C.Roberts, Dr L. T. Canon Glaucoma and HL-A An~enK Prof. W. L. Henley and others Hzmophilia-B Carriers Dr Susan EIOdi. The Great Protein Fiasco N. W. Pirie, F.R.S. Renal Activity and Renal Filtration Fraction Prof. cran~ois Reubi, M.D., and others Liver-specific Bile Canalicular Antibodies Dr E. Linder and others 1273 1273 1274 1274 1275 Drugs Safety and Children 1275 Kay Monram Mortality and Natural Fluoride Content of Drinking-water 1275 MrH.A. Cook Mumps Vaccine Dr A. J. Steigman PARLIAMENT The Budget and Social Security Benefits NOTES AND NEWS Approved Names University of Leicester University of Liverpool University of Southampton 1276 1276 1277 1277 1277 1278 !. is Vi IC ~y Ie ot le le le as ed to he 1 a :b-his for nic h? ;t a :an ley, 11 is :e a ;ore t to jea! !pes The \,·tlo \lich :oJ1l-r THE LANCET, NOVEMBER 23, 1974 vou ask in your editorial-forms the kernel of the argument for both interdisciplinary students and interdisciplinary staff in the teaching of community health. In my opinion, a health professional is a person whose work has some effect on the health of the community, either directly. or indirectly (i.e., economists, school teachers, veterinarians, and so on), and who undertakes further training in the health-related aspects of his work. All such people, if suitably qualified, would be welcome on the M.COMM.H. course. Department of Tropical Community Health, Liverpool School of Tropical Medicine, Pembroke Place, Liverpool L3 5QA. FRANCIS M. SHATTOCK. EPIDEMIOLOGY OF CROHN'S DISEASE SIR,-Dr Barker and Dr Gardner (Nov. 9, p. 1140) were right to draw attention to the regional variation in discharge-rates for Crohn's disease and ulcerative colitis in England and Wales. We decided to use national rather than regional data to compare with our own and that from the specified countries in Northern Europe, where prevalence of the disease is comparable with our own figure. Con-siderable caution is necessary in interpreting regional data because it is not collected with the same care as that given to special studies. Departments of Community Health and Therapeutics, Medical School, Nottingham University, University Park, Nottingham NG7 2RD. D. S. MILLER A. C. KEIGHLEY M. J. S. LANGMAN. PORTACAVAL SHUNT IN HYPERLIPIDlEMIA Sm,-Twice previously in this journal we described amelioration of symptoms in an ll-year-old girl with homozygous rype-I1a hyperlipoproteina:mia following end-to-side portacaval shunt. 1,' Preoperatively, the child had suffered severe myocardial infarction. After operation, serum cholesterol and low-density-lipoprotein concentra-tions were reduced. Cutaneous and tendinous xanthomas underwent resorption. There was apparent regression of cardiovascular disease with relief of angina and reversal of ao~c stenosis. However, coronary angiograms 16 months after portacaval shunt showed three residual tight stenoses: one at the bifurcation of the left coronary artery into the circumflex and left anterior descending arteries; a second more distal in the circumflex artery; and the third in the right coronary artery. A coronary bypass procedure was decided against because of the belief that these lesions might resolve spontaneously. The patient was last seen by us on Sept. 14, 1974, as the subject of a Grand Rounds at the University. She felt well and had normal liver-function tests. On Sept. 23, while ~~g home from school, she collapsed in the street and died Immediately. At necropsy, 18t months after portacaval shunt, the most important findings were in the cardio-~ascular system. The heart was greatly enlarged, weighing 10 g. (normal 200 g.). There was a large left ventricular ~eurysm which was the result of the . old myocardial ~nfarcti?nK The three coronary-artery stenoses identified J angIography 3 months earlier were still present but nre we.re no thromboses or areas of fresh infarction. art e .aomc valve easily admitted the tip of a finger. Many d enc:s, as well as the aorta, had residual xanthomatous r:lplt~K The right and left lungs weighed 415 and 340 g., pectlvely, and had histological features consistent with ---lKp~~q------------------------------------1973 .: b~I Chase, H. P., Putnam, C. W., Poner, K. A. Lancet, 2 S ' ll, 940. • ~41I T. E., Chase, H. P., Putnam, C. W., Nora,].J. ibid. Sept. 21, , p. 714. 1263 the chronic right-heart failure from which she had suffered since the original myocardial infarction. The liver, which weighed 618 g., was grossly normal, and microscopically it was unchanged from the biopsy obtained 6 months postoperatively. 1 The portacaval shunt was widely patent. From the clinical course and the necropsy findings, the conclusions of the clinicians and pathologists was that death was by an acute cardiac arrhythmia related to the residual coronary-artery disease and/or the earlier myo-cardial infarction. Departments of Surgery, Pediatrics, and Pathology, University of Colorado Medical . Center and Veterans Administration Hospital, Denver, Colorado 80220, U.S.A., and Department of Pathology, St. Mary's Hospital and Medical School, London W2 1NY. England. THOMAS E. STARZL H. PETER CHASE CHARLES W. PUTNAM JAMES J. NORA ROBERT H. FENNELL, JR. KENDRICK A. PORTER. PORTACAVAL SHUNT IN FAMIUAL HYPERCHOLESTEROLffiMIA SIR,-The reports 1.2 by Dr Thomas Starzl and his colleagues of the successful use in one patient with homo-zygous familial hypercholesterolremia of an end-to-side portacaval shunt has engendered much discussion. The effects of the procedure, and the needs and opportunities for further clinical research that it suggests~ were lately commented on in a paper and an editorial. 3 ,-We should like to report briefly on a workshop on this subject held at the National Heart and Lung Institute, Bethesda, Maryland, on Aug. 1 and 2, 1974. The work-shop was prompted by the implic;.ations the initial observa-tion has both for therapy and for the better understanding of lipid metabolism and atherogenesis. The small group of discussants considered the definition and natural history, the pathophysiology of hypercholesterolremia, treatment by various means, and the portacaval shunt. Some recom-mendations on the applicability of the shunt for homo-zygous familial hypercholesterolremia and for research needs arose from this discussion * : Conclusions and Recommendations (a) The diagnostic criteria for homozygous familial hyper-cholesterola:mia include serum-cholesterol levels in excess of 500 mg. per 100 mi., hypercholesterola:mia in both parents, or, in the absence of a parent, in both family lines; and the appearance of xanthomas in the first decade of life. While these diagnostic criteria may be expanded and usefully include tissue-culture criteria, the above descriptors constitute a minimum character-isation for this diagnosis. (b) This inborn error of metabolism so limits life expectancy that it justifies prompt and intensive treatment. including inno-vative measures. (cl The medical treatment of choice is a combined regimen of a low-fat, low-cholesterol diet with a high PIS fatty-acid ratio; a bile-acid sequestrant such as cholestyramine (up to 32 g. per day); and nicotinic acid (up to 3·0 g. per day) to the limits of tolerance, but monitored to avoid abnormally high serum-transaminase levels. Successful medical management includes both a major (40% or more) and fairly prompt reduction in serum-cholesterol and the slower resolution of xanthomas. It is unproven at present whether these attributes of management are also accompanied by a regression of atherosclerotic vascular disease and a significant prolongation of life. Medical management may have little or no effect on some patients. Cd) The portacaval shunt is an innovation for homozygous * A more complete summary of the workshop, including a selected bibliography, is available on request from the Atherogenesis Branch, Division of Heart and Vascular Diseases, National Heart and Lung Institute, Bethesda, Maryland 20014, U.S.A. I. Starzl, T. E., Chase, H. P., Putnam, C. W., Poner, K. A. Lancet, 1973, ii, 940. 2. Starz!, T. E., Chase, H. P., Putnam, C. W., Nora, J. J. ibid. Sept. 21, 1974, p. 714 • 3. Ahrens, E. H., Jr. ibid. Aug. 24, 1974, p. 449. 4. ibid. p. 444. , . ! ~ " ! I Ii " 1:1" 1264 familial hypercholesterolremia that deserves intensive evaluation and study. It is recommended that, for the present, its use should be restricted to the patient with severe homozygous familial hypercholesterolremia who has clinical vascular disease and who fails to respond to intensive medical therapy. (e) The procedure should be carried out in institutions with the necessary diagnostic, surgical, and investigative metabolic expenise to assure, as far as possible, a successful clinical outcome, an adequate medical and neuropsychiatric follow-up, and the capability to study important metabolic parameters. Intercentre cooperation will be helpful. Collaboration with reference laboratories that have specialised in the conduct of certain specific but uncommon tests is recommended, both in order to facilitate the performance of the tests and to standardise their quality. (f) The development of a central patient registry is recom-mended to facilitate the prompt collection of information about both medical and surgical managements of homozygous familial hypercholesterolremia, and to act as a responsive resource for investigators and others interested in this problem. Not only is homozygous familial hyperchoiesterolremia a rare disorder, but there are relatively few medical centres in the world capable of the sophisticated sterol, lipoprotein, and other studies that may advance our knowledge. Indeed, intercentre collaboration may be needed if some investiga-tions are to be performed economically and in the most appropriate way. In these circumstances it is advisable to facilitate scientific communication, to pool ideas and data, to make the most of such data as may be obtained, and to enhance the contri-bution of future investigations. To this end the National Heart and Lung Institute has offered to act as an initial focus or registry for information exchange on' current or planned shunt procedures for this condition. Investigators active in this area are urged to communicate with S. M. at the Institute. Division of Heart and Vascular Disease, National Heart and Lung Institute, National Institutes of Health, Landow Building, Room C.S08, Bethesda, Maryland 20014, U.S.A. SHIELA MITCHELL ROBERT 1. LEVY. THE VULNERABLE MYOCARDIUM SIR,-As you have rightly pointed out in your editorial (Oct. 26, p. 994) too little serious attention has been given to those proposing " unconventional " hypotheses for our modern epidemics of myocardial infarction, diabetes, &c. Anyone reading Cleave's latest work 1 must be struck by the strength of his arguments. Trowell 2 has added fuel to this fire with his survey of diabetes death-rates and their relation to diet. In addition to the epidemiological evidence advanced by Cleave and others, Anderson 3 postulated an attractive biochemical hypothesis related to a myocardium left vulnerable to insult because, through a lack of dietary antioxidants, the myocardial metabolic machinery is incapable of withstanding the dystrophic effect of per-oxides. He finds that the modern diet has been: (1) stripped of d-a-tocopherol; and (2) supplemented with polyun-saturated fatty acids. Using this sort of approach, one could go directly to a myocardial infarction without necessarily having atherosclerosis. Support for such a hypothesis comes from Richardson et a!.' who have found angina pectoris in patients with normal coronary arteries. We have grown to accept the view that atherosclerosis causes myocardial infarction, the pendulum having swung so far that many Governments are now officially in favour of increased polyunsaturates. In view of this counter 1. Cleave, T. L. The Saccharine Disease. Bristol, ~ 974. .... Trowell, H. Lancet, 1974, Ii, p. 998. 3 .• 'Uloerson, T. Wf. ,hid. 1973, ii, 298. .1, Richardson. P. J.} Livesley, B., Oram, S., Olsen, E. G. J., Armstrong, ~FK loid. :'974. ii. :]T!. THE LANCET, NOVEMBER 23, 1974 hypothesis, such action could potentially exacerbate the problem. Royal College of Surgeons in Ireland, St. pteph~nDs Green, Dublin. CHRISTOPHER HASSELL. ISCHlEMIC HEART -DISEASE AND DIET SIR,-Your leader (Aug. 24, p. 444) on portacaval anastomosis for metabolic disease drew attention to the remarka ble case of a child reported by S tarzl et aJ.1·2 with xanthomas, myocardial infarction, and serum-cholesterol level of 900 mg. per 100 ml. (32·2 mmole per 1.). Diet and drugs were ineffective, but intravenous feeding halved the serum-cholesterol for a few weeks. Portacaval anastomosis was followed by a fall to 240 mg. per 100 ml. (6'3 mmole per 1.), resolution of xanthomas, and relief of angina. The endoplasmic reticulum of the liver cells was reduced after operation, suggesting that cholesterol production may have decreased. An enzyme system responding by an increase when exposed to high concentrations of substrates would fit the observations, the high concentrations of digestion products reaching the liver after each meal being reduced by intravenous feeding or portacaval shunt. The implications of this theory are: (1) intragastric drip feed should be as effective as intravenous .. feeding"; numerous small feeds spread over the day may lower the serum-lipids without a change in total intake; (2) the restriction of cholesterol, saturated fats, and simple sugars may owe some of their failure to the custom of one large meal per day. The Guest Hospital, Dudley DYl 4SE. E. BOWERS. EPSILON-AMINO CAPROIC ACID SIR,-We were very pleased to learn. that Mr Uttley and Mr Richardson (Nov. 2, p. 1080) have not seen serious complications resulting from the administration of anti-fibrinolytic drugs in a large series of patients with sub-arachnoid hremorrhage. We would, however, wish to question their assertion that the re-bleeding-rate of 12% which they found in their treated patients is " much superior" to the natural history of the condition. In a large cooperative study, to which the authors were themselves major contributors, the re-bleeding rate in cases of aneurysms of the anterior part of the circle of Willis was 10% in the first week, 12% in the second, 7% in the third, and 8% in the fourth." These figures cannot be simply summated to obtain the re-bleeding rate over 2 or 3 weeks because the population of patients at risk is diminishing with time. The figure of 12% given in the trial of antifibrinolytic agents would seem to be an underestimate of the risk of re-bleeding, since some patients will have undergone surgical occlusion of their aneurysms or will have died. It is a matter of considerable importance to establish statistically whether antifibrinolytic therapy offers significant protection against re-bleeding from aneurysms. We and many other neurosurgeons would be immensely grateful to Mr Uttley and Mr Richardson if they could present firm evidence. University Department of Neurosurgery, Institute of Neurological Sciences, Glasgow G51 4TF. M. D. M. SHAW J. DOUGAS MILLER. 1. Starz!, T. E., Chase, H. P., Putnam, C. \v/'1 Porter, Ie A. Lancet, 1973, ii. <140 . ,., Starzi, T. E., Chase, H. P., mutnam~ C. W., }.fora, J. J. ibid. Sept. 21, 1974. ,J. 715 . LOd,-siey. H .. J).]. ~se!EqlsuqgK ~9SSI 25, _'21. 11' ( I J r l /. f \ i· f ( 

Portacaval shunt in hyperlipidemia

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Portacaval shunt in hyperlipidemia

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