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A NEW LIVER DISEASE
1178 considerable amount of routine work is necessary to keep a consultant’s opinion and technique up to scratch. Hence the schizophrenic attitude of many consultants to routine hospital work. Thirdly, much is said about general practitioners working in hospitals. The present set-up in general practice, together with scant regard for high professional competence in modern medicine (to be achieved nowadays only after a number of years in hospital), has resulted in more and more work being passed on to the hospital service. The senior registrars have already been treated with gross injustice, and it is already apparent that they may well be the victims again to satisfy the established general practitioner’s desire to enter hospital. Many plans are put forward to enable the general practitioner to enter his local hospital; but nothing is done to help the senior registrar to enter general practice, and even less to allow the senior registrar to establish a base in hospital (for which he should have prior consideration) and then to enter general practice locally. There can be no solution to the staffing of hospitals until justice is done, and outmoded and unworkable ideas discarded. REGISTRAR. a
A NEW LIVER DISEASE
SIR,-Your interesting leading article last week reminds me (comparison and contrast) of the case of a man whom I first saw in 1917 in connection with a life-assurance question. persistent jaundice of variable degree, dating from early childhood or birth, but had been able to live a normal life. I have fully described his case elsswhere,’ and I demonstrated the case on several occasions at the Royal Society of Medicine. His blood always gave a negative direct but positive indirect Hijmans van den Bergh reaction. His urine was always found free from bilirubin and from excess of urobilin and urobilinogen. There was never any anaemia or enlargement of liver or spleen or lymphadenopathy noted ; nor were any abnormal erythrocytes present. There was no other example of jaundice in his family ; the jaundice had not been transmitted to any of his four children. I saw the patient at intervals until his death in October, 1946. He would have been 80 in November, 1946. He had considerable cerebral atheromatosis, and he died from pneumonia and thrombotic softening in the basal ganglia. A careful post-mortem examination was made at the Archway He had
apparently
Hospital, Highgate. I have not been able to discover any record of a case exactly similar to this one, but I would now suggest that the condition may possibly have been due to an infective hepatitis contracted in childhood, or perhaps even before birth, and followed by life-long jaundice of the type I have described. Imust add, however, that in my original account of the case I suggested a somewhat different pathological
explanation : " The negative post-mortem findings in regard to the cause of the life-long jaundice were, of course, what would have been expected. His icteric symptoms had been proved to be due simply to excess of blood bilirubin in his blood. Excepting for this, and the history of leg ulcers, he has none of the following features ordinarily associated with familial acholuric (haemolytio) jaundice, namely ; chronic splenomegaly, excess (until the end) of urobilin in the urine, negative (until the end) direct van den Bergh’s reaction in the blood-serum, excess fragility of erythrocytes towards graduated hypotonic sodium chloride solutions. One cannot now ascertain whether his production of blood bilirubin had been excessive or not, nor whether there was a damming-up of blood bilirubin in the blood (high threshold for its elimination or catabolism). It seems probable, however, that there was such a damming-up of blood bilirubin, as the essential cause of the life-long jaundice. If so, one would not expect to find any microscopical structural peculiarity in the "
1. Parkes Weber, F. Rare Diseases and Some Debatable Subjects. 2nd ed., London, 1947 ; p. 118.
Thus, analogously, in cases o: organs to account for it. familial renal glycosuria there is no evidence by microscopical examination of the kidneys, pointing to the existence of a low threshold for the excretion of sugar. Neither, by thf; histological examination of the excretory organs of rabbit, as compared to those of men, could one ascertain that lipom feeding causes relatively more accumulation of cholestera. in rabbits than in men." To clear up the true pathogenesis of my case one must. think, await the occurrence of similar cases, vhen more exact methods of examination will have to be made use of, and the family history more carefully I
-
searched. London, N.W.I.
F. PARKES WEBER.
POISONING IN CHILDREN : A NEW HAZARD
SIR,-The long list of drugs and therapeutic substance
dangerous when swallowed by children has been by the arrival of attractively covered granule containing long-acting phenobarbitone. These granule, seem indistinguishable from the " hundreds and thousands of iced cakes. The range of brightly coloured and often sugar-coated pills, including ferrous sulphate.
which are increased
"
the anti-histamines, and many others, now form an ever-growing hazard in the home. It is time that thi problem was effectively tackled. It would seem that the manufacturers should be required to put public safety before " sales appeal." The former traditional ness of remedies, while having obvious advantages, need not be reverted to ;absence of attractive appearance and taste are now essential.
unpleasant-
Oldchurch Hospital, Romford, Essex.
THOMAS SAVAGE.
CARE AND DISPOSAL OF MONGOLIAN DEFECTIVES
SiR,-The- article by Mrs. Evans and Dr. Carter in your issue of Nov. 6 was interesting and their survey had clearly been carried out with care ; but may I, as a physician-superintendent responsible for the care of 800 mental defectives and in charge of a subregional admission bureau, make some comments on their deductions and recommendations. It is necessary to remember that mongolian defectives represent less than one in ten of all ascertained defectives, and it is of limited value to consider the care and disposal of this special class of defective unless we consider it against the background of the care and disposal of all defectives of every type and grade. I imagine that, if they had investigated the first 224 defective children with red hair, their result would have been much the same. Further criticisms can be made of their recommendations under the heading Further Provision Needed: Institutional Accommodation Mrs. Evans and Dr. Carter state that more places are needed for mongols in an institution. This is, of course, true. but must be considered in relation to the other types of defective also on the waiting-list whose need for admission is probably more urgent. It is well known that the mongol defective ’usually very easy to manage at home. They say that local authorities do not make sufficient use of their permissive view to pay for short-term care ;; in fact, if short-term care is given in a mental-defective institution, no fees are payable by the local authority.
Occupation Centres They declare that
more places at occupation centres are needed and that these should include places for aged five years. I have yet to visit an occupation centre which does not provide places for mongols aged five. I cannot agree that occupation centres should be brought under the educatilrdepartment ;these centres should always be under the sup?t’ vision of a medical officer experienced in the work. The preference of parents for a school for educationally subnorms (E.s.N.) is quite irrelevant. The mental status of the patientis the important factor.
mongols
A NEW LIVER DISEASE
SIR,-Your interesting leading article last week reminds me (comparison and contrast) of the case of a man whom I first saw in 1917 in connection with a life-assurance question. persistent jaundice of variable degree, dating from early childhood or birth, but had been able to live a normal life. I have fully described his case elsswhere,’ and I demonstrated the case on several occasions at the Royal Society of Medicine. His blood always gave a negative direct but positive indirect Hijmans van den Bergh reaction. His urine was always found free from bilirubin and from excess of urobilin and urobilinogen. There was never any anaemia or enlargement of liver or spleen or lymphadenopathy noted ; nor were any abnormal erythrocytes present. There was no other example of jaundice in his family ; the jaundice had not been transmitted to any of his four children. I saw the patient at intervals until his death in October, 1946. He would have been 80 in November, 1946. He had considerable cerebral atheromatosis, and he died from pneumonia and thrombotic softening in the basal ganglia. A careful post-mortem examination was made at the Archway He had
apparently
Hospital, Highgate. I have not been able to discover any record of a case exactly similar to this one, but I would now suggest that the condition may possibly have been due to an infective hepatitis contracted in childhood, or perhaps even before birth, and followed by life-long jaundice of the type I have described. Imust add, however, that in my original account of the case I suggested a somewhat different pathological
explanation : " The negative post-mortem findings in regard to the cause of the life-long jaundice were, of course, what would have been expected. His icteric symptoms had been proved to be due simply to excess of blood bilirubin in his blood. Excepting for this, and the history of leg ulcers, he has none of the following features ordinarily associated with familial acholuric (haemolytio) jaundice, namely ; chronic splenomegaly, excess (until the end) of urobilin in the urine, negative (until the end) direct van den Bergh’s reaction in the blood-serum, excess fragility of erythrocytes towards graduated hypotonic sodium chloride solutions. One cannot now ascertain whether his production of blood bilirubin had been excessive or not, nor whether there was a damming-up of blood bilirubin in the blood (high threshold for its elimination or catabolism). It seems probable, however, that there was such a damming-up of blood bilirubin, as the essential cause of the life-long jaundice. If so, one would not expect to find any microscopical structural peculiarity in the "
1. Parkes Weber, F. Rare Diseases and Some Debatable Subjects. 2nd ed., London, 1947 ; p. 118.
Thus, analogously, in cases o: organs to account for it. familial renal glycosuria there is no evidence by microscopical examination of the kidneys, pointing to the existence of a low threshold for the excretion of sugar. Neither, by thf; histological examination of the excretory organs of rabbit, as compared to those of men, could one ascertain that lipom feeding causes relatively more accumulation of cholestera. in rabbits than in men." To clear up the true pathogenesis of my case one must. think, await the occurrence of similar cases, vhen more exact methods of examination will have to be made use of, and the family history more carefully I
-
searched. London, N.W.I.
F. PARKES WEBER.
POISONING IN CHILDREN : A NEW HAZARD
SIR,-The long list of drugs and therapeutic substance
dangerous when swallowed by children has been by the arrival of attractively covered granule containing long-acting phenobarbitone. These granule, seem indistinguishable from the " hundreds and thousands of iced cakes. The range of brightly coloured and often sugar-coated pills, including ferrous sulphate.
which are increased
"
the anti-histamines, and many others, now form an ever-growing hazard in the home. It is time that thi problem was effectively tackled. It would seem that the manufacturers should be required to put public safety before " sales appeal." The former traditional ness of remedies, while having obvious advantages, need not be reverted to ;absence of attractive appearance and taste are now essential.
unpleasant-
Oldchurch Hospital, Romford, Essex.
THOMAS SAVAGE.
CARE AND DISPOSAL OF MONGOLIAN DEFECTIVES
SiR,-The- article by Mrs. Evans and Dr. Carter in your issue of Nov. 6 was interesting and their survey had clearly been carried out with care ; but may I, as a physician-superintendent responsible for the care of 800 mental defectives and in charge of a subregional admission bureau, make some comments on their deductions and recommendations. It is necessary to remember that mongolian defectives represent less than one in ten of all ascertained defectives, and it is of limited value to consider the care and disposal of this special class of defective unless we consider it against the background of the care and disposal of all defectives of every type and grade. I imagine that, if they had investigated the first 224 defective children with red hair, their result would have been much the same. Further criticisms can be made of their recommendations under the heading Further Provision Needed: Institutional Accommodation Mrs. Evans and Dr. Carter state that more places are needed for mongols in an institution. This is, of course, true. but must be considered in relation to the other types of defective also on the waiting-list whose need for admission is probably more urgent. It is well known that the mongol defective ’usually very easy to manage at home. They say that local authorities do not make sufficient use of their permissive view to pay for short-term care ;; in fact, if short-term care is given in a mental-defective institution, no fees are payable by the local authority.
Occupation Centres They declare that
more places at occupation centres are needed and that these should include places for aged five years. I have yet to visit an occupation centre which does not provide places for mongols aged five. I cannot agree that occupation centres should be brought under the educatilrdepartment ;these centres should always be under the sup?t’ vision of a medical officer experienced in the work. The preference of parents for a school for educationally subnorms (E.s.N.) is quite irrelevant. The mental status of the patientis the important factor.
mongols