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AMYLOID DEGENERATION
1037
1961 in Massachusetts. This statistical analysis suggests that the concurrence of measles and chickenpox in a single patient is very uncommon. There have been other recorded examples of simultaneous virus infections, but’it is usually found that only In this elegant a single clinical picture has developed. disease states were two distinct clinically little study
period during
recognisable. DEGENERATION AMYLOID
DESPITE electron microscopy and new physical methods for the study of serum-proteins, the pathogenesis of amyloidosis is almost as much a mystery as ever. Possibly owing to the recognition in patients with Familial Mediterranean Fever of a genetically determined form of amyloidosis, the subject has received considerable attention in Israel; and two significant contributions have recently been made by Israeli workers. Amyloidosis has never been easy to diagnose clinically, and even the Congo-red test, introduced by Bennhold1 forty years ago, did little to ease diagnosis. Thus Calkins and Cohen2 reported false-negative results of a Congo-red test in half of a series of 24 patients, and Blum and Sohar3 found the test positive in only 11 of 83 patients in whom the disease was confirmed by biopsy or necropsy. The only satisfactory basis for diagnosis during life is some form of biopsy; but, of the sites commonly used for this purpose up to 1950, only the kidney and the liver gave sufficiently consistent results to be of real value. Since biopsy of these organs is not without risk, alternative sites were sought; and the gum margin was found to be fairly suitable. But the situation was transformed in 1960 when Gafni and Sohar4 reported a high incidence of amyloid infiltration in the rectum in all varieties of the disease. Their positive findings in 75% of rectal biopsies compare very favourably with their figures of 87% and 48% for renal and hepatic biopsies respectively. The widespread use of this diagnostic procedure throughout Israel since 1959 has resulted in a rise in the antemortem diagnosis from an average of 39% of cases in 1950-58
74% in 1959-60. The long-accepted classification of amyloid disease into the secondary variety associated with some other chronic disease, such as tuberculosis, and the primary form, which arises independently, has proved unsatisfactory since the distribution of the affected areas is not at all closely related to whether the disorder is primary or secondary. The so-called classical distribution of the lesions in the liver, spleen, intestines, and adrenals thought to be characteristic of the secondary variety is commonly found in the primary form. In 1959 Missmahl5 reported a correlation between the site of arterial deposition of amyloid and the clinical variety of the disease. He recognised two clearly differentiated types of arterial involvement. In the first the amyloid was deposited in the intima and inner portion of the media; in the second these areas were spared but deposition involved the adventitia, the outer media, and the intervascular connective tissue. In typical primary amyloidosis the deposition was invariably adventitial, in secondary amyloidosis invariably intimal. Gafni 6 has enlarged on these observato
1. 2. 3. 4. 5. 6.
Bennhold, H. Dtsch. Arch. klin. Med. 1923, 142, 32. Calkins, E., Cohen, A. S. Bull. rheum. Dis. 1960, 10, 215. Blum, A., Sohar, E. Lancet, 1962, i, 721. Gafni, J., Sohar, E. Amer. J. med. Sci. 1960, 240, 332. Missmahl, H. P. Verh. dtsch. Ges. inn. Med. 1959, 56, 439. Gafni, J. Proc. Tel-Hashomer Hosp. 1962, 1, 73.
tions and now claims that in the intimal type the deposition of amyloid is intimately related to reticulin, whereas in the adventitial type amyloid deposition is related to collagen fibres. According to Gafni, in the so-called classical distribution of amyloid, whether primary or secondary, the deposition is invariably the intimal-that is, of reticulin type. The atypical distribution of amyloid (i.e., in tongue, heart, skin, and nerves) invariably shows the adventitial-that is, collagen-type of deposition whether it arises spontaneously or secondarily to multiple myeloma. When we last considered amyloidosis,7 we suggested that amyloid itself was formed from a circulating precursor by polymerisation when it came in contact with extravascular connective tissue. If Gafni is correct there may be two forms of soluble amyloid precursor, the first requiring reticulin and the second collagen to initiate its
polymerisation. HOUSEBOUND
" GERIATRICS " is a new, ugly, and unscholarly word; but its coining is a welcome sign of obligations accepted, provided that it implies that medical care of the old is an essential province of medicine and not that yet another specialty has been created. Looking after the old has long been recognised as a family and social duty, but gerontology-the study of age and ageing, whether by the methods of sociology, clinical medicine, or physiology-is still in its scientific infancy with far to go and much to be done. When gerontology has gone further, geriatrics will have a larger fund of technical knowledge to learn and apply and a firmer claim to recognition as a specialty. But as things are, the crying needs of the old are for energetic and imaginative application of knowledge based on established but unpalatable facts. We have ample to go
on.
Even the daunting catalogue of Ecclesiastes 8-the edentulous jaws, darkened eyesight, feeble, shaking hands, useless legs, fear of falling, and the relapses after hardfought recoveries-is not much dated; we have our palliatives, but hardly more, and to it our civilisation has added the stairs, the lonely tenement or room, the terrifying traffic, the frequent neglect of the busy bustling world, and compulsory retirement at a fixed age. Medicine has lengthened the span of life, and, as an unplanned sideeffect, has created a growing disproportion between old and young and a large and perhaps increasing number of old people who wish their lives would end, or had been allowed to end. Gerontology and geriatrics apart, doctors have collective professional obligations to the old and ageing as a class, and these obligations are of more manageable size than those to the population explosion in the world at large. They are also perhaps better recognised. A symposium on the family doctor and the care of the aged, with expert and imaginative contributions from many sources, has lately been published as a supplement to the Journal of the College of General Practitionersand makes, in every sense, important reading. From Luton Dr. D. R. Snellgrove IQ reports on his survey of some 330 elderly housebound. Too often he has to record loneliness, the frustration of social uselessness, malnutrition-especially lack of vitamins-unsuitable environments, many remediable or easable disabilities. Though much, in bulk, is being done, 7. 8. 9. 10.
Lancet, 1961, i, 1215. Ecclesiastes, XII, 1-5. J. Coll. gen. Pract. 1963, 6, suppl.
no.
1.
Snellgrove, D. R. Elderly Housebound. (Crescent Road, Luton). 10s.
1963. White Crescent Press
1961 in Massachusetts. This statistical analysis suggests that the concurrence of measles and chickenpox in a single patient is very uncommon. There have been other recorded examples of simultaneous virus infections, but’it is usually found that only In this elegant a single clinical picture has developed. disease states were two distinct clinically little study
period during
recognisable. DEGENERATION AMYLOID
DESPITE electron microscopy and new physical methods for the study of serum-proteins, the pathogenesis of amyloidosis is almost as much a mystery as ever. Possibly owing to the recognition in patients with Familial Mediterranean Fever of a genetically determined form of amyloidosis, the subject has received considerable attention in Israel; and two significant contributions have recently been made by Israeli workers. Amyloidosis has never been easy to diagnose clinically, and even the Congo-red test, introduced by Bennhold1 forty years ago, did little to ease diagnosis. Thus Calkins and Cohen2 reported false-negative results of a Congo-red test in half of a series of 24 patients, and Blum and Sohar3 found the test positive in only 11 of 83 patients in whom the disease was confirmed by biopsy or necropsy. The only satisfactory basis for diagnosis during life is some form of biopsy; but, of the sites commonly used for this purpose up to 1950, only the kidney and the liver gave sufficiently consistent results to be of real value. Since biopsy of these organs is not without risk, alternative sites were sought; and the gum margin was found to be fairly suitable. But the situation was transformed in 1960 when Gafni and Sohar4 reported a high incidence of amyloid infiltration in the rectum in all varieties of the disease. Their positive findings in 75% of rectal biopsies compare very favourably with their figures of 87% and 48% for renal and hepatic biopsies respectively. The widespread use of this diagnostic procedure throughout Israel since 1959 has resulted in a rise in the antemortem diagnosis from an average of 39% of cases in 1950-58
74% in 1959-60. The long-accepted classification of amyloid disease into the secondary variety associated with some other chronic disease, such as tuberculosis, and the primary form, which arises independently, has proved unsatisfactory since the distribution of the affected areas is not at all closely related to whether the disorder is primary or secondary. The so-called classical distribution of the lesions in the liver, spleen, intestines, and adrenals thought to be characteristic of the secondary variety is commonly found in the primary form. In 1959 Missmahl5 reported a correlation between the site of arterial deposition of amyloid and the clinical variety of the disease. He recognised two clearly differentiated types of arterial involvement. In the first the amyloid was deposited in the intima and inner portion of the media; in the second these areas were spared but deposition involved the adventitia, the outer media, and the intervascular connective tissue. In typical primary amyloidosis the deposition was invariably adventitial, in secondary amyloidosis invariably intimal. Gafni 6 has enlarged on these observato
1. 2. 3. 4. 5. 6.
Bennhold, H. Dtsch. Arch. klin. Med. 1923, 142, 32. Calkins, E., Cohen, A. S. Bull. rheum. Dis. 1960, 10, 215. Blum, A., Sohar, E. Lancet, 1962, i, 721. Gafni, J., Sohar, E. Amer. J. med. Sci. 1960, 240, 332. Missmahl, H. P. Verh. dtsch. Ges. inn. Med. 1959, 56, 439. Gafni, J. Proc. Tel-Hashomer Hosp. 1962, 1, 73.
tions and now claims that in the intimal type the deposition of amyloid is intimately related to reticulin, whereas in the adventitial type amyloid deposition is related to collagen fibres. According to Gafni, in the so-called classical distribution of amyloid, whether primary or secondary, the deposition is invariably the intimal-that is, of reticulin type. The atypical distribution of amyloid (i.e., in tongue, heart, skin, and nerves) invariably shows the adventitial-that is, collagen-type of deposition whether it arises spontaneously or secondarily to multiple myeloma. When we last considered amyloidosis,7 we suggested that amyloid itself was formed from a circulating precursor by polymerisation when it came in contact with extravascular connective tissue. If Gafni is correct there may be two forms of soluble amyloid precursor, the first requiring reticulin and the second collagen to initiate its
polymerisation. HOUSEBOUND
" GERIATRICS " is a new, ugly, and unscholarly word; but its coining is a welcome sign of obligations accepted, provided that it implies that medical care of the old is an essential province of medicine and not that yet another specialty has been created. Looking after the old has long been recognised as a family and social duty, but gerontology-the study of age and ageing, whether by the methods of sociology, clinical medicine, or physiology-is still in its scientific infancy with far to go and much to be done. When gerontology has gone further, geriatrics will have a larger fund of technical knowledge to learn and apply and a firmer claim to recognition as a specialty. But as things are, the crying needs of the old are for energetic and imaginative application of knowledge based on established but unpalatable facts. We have ample to go
on.
Even the daunting catalogue of Ecclesiastes 8-the edentulous jaws, darkened eyesight, feeble, shaking hands, useless legs, fear of falling, and the relapses after hardfought recoveries-is not much dated; we have our palliatives, but hardly more, and to it our civilisation has added the stairs, the lonely tenement or room, the terrifying traffic, the frequent neglect of the busy bustling world, and compulsory retirement at a fixed age. Medicine has lengthened the span of life, and, as an unplanned sideeffect, has created a growing disproportion between old and young and a large and perhaps increasing number of old people who wish their lives would end, or had been allowed to end. Gerontology and geriatrics apart, doctors have collective professional obligations to the old and ageing as a class, and these obligations are of more manageable size than those to the population explosion in the world at large. They are also perhaps better recognised. A symposium on the family doctor and the care of the aged, with expert and imaginative contributions from many sources, has lately been published as a supplement to the Journal of the College of General Practitionersand makes, in every sense, important reading. From Luton Dr. D. R. Snellgrove IQ reports on his survey of some 330 elderly housebound. Too often he has to record loneliness, the frustration of social uselessness, malnutrition-especially lack of vitamins-unsuitable environments, many remediable or easable disabilities. Though much, in bulk, is being done, 7. 8. 9. 10.
Lancet, 1961, i, 1215. Ecclesiastes, XII, 1-5. J. Coll. gen. Pract. 1963, 6, suppl.
no.
1.
Snellgrove, D. R. Elderly Housebound. (Crescent Road, Luton). 10s.
1963. White Crescent Press