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MECONIUM ILEUS
177
open to them if they become statf nurses or unfortunately survival after operation is not the same as to to them a is attract, hard What cure. As nearly all these infants have fibrocystic disease sisters. there, then, and harassment of the of the pancreas the alimentary and respiratory changes with examinations, long hours, job when they could do the same work for nearly the same characteristic of this condition almost inevitably develop later on and eventually prove fatal, T’he management pay and without the threat of the examiner, or—by of those who survive operation requires close coöperation becoming either a domestic worker or a laundrymaid
prospects
---
do easier work pay?A handy and exceptionally an institution for mental defectives, in maid intelligent when asked why she did not transfer to the nursing statf, replied without hesitation : "I’m better off as I am ; and I get my evenings and weekends." It was the reply of common sense, and deserves attention. Young women choosing to look after the mentally sick cannot, and do not, expect to be paid at excessive rates : for But it is one thing the country could not afford it. surely time we recognised, however modestly, that the work they do is more personal, responsible, and exacting than that of a laundry or domestic maid. for
more
MECONIUM ILEUS MECONIUM ileus is one of the less common causes of intestinal obstruction in the newborn baby. The obstruction arises from the presence in the small bowel of firm, putty-like, extremely sticky meconium which clings to the mucosa and clogs the lumen. The abnormal qualities of the meconium are due to the absence of pancreatic secretions-a relationship first recognised by Landsteiner1 in 1905, although he mistakenly concluded that the obstruction. pancreatic changes were due to the intestinal The work of Andersen2 and Farber 3 has shown that the absence of pancreatic secretion is the primary disturbance, and the condition is nearly alwaysassociated with fibrocystic disease of the pancreas-Bodian4 has estimated that meconium ileus occurs in one out of five cases of this disease. Occasionally meconium ileus is associated with atresia or stenosis of the main pancreatic duct.66 The usual history is that of any form of intestinal obstruction in the newborn-abdominal distension, vomiting, and failure to pass meconium. The abdomen may feel rather doughy or lumpy ; but otherwise there are no characteristic features, and respiratory symptoms, which are a distinct feature of fibrocystic disease in older infants and children, are not present at birth. Some help in making a preoperative diagnosis may be had from the family history, since more than one child is often affected, and from a plain radiograph of the abdomen, which may show the presence of small bubbles of gas in 7 The radiograph also amass of inspissated meconium. of that termination of the intesshows absence abrupt tinal gas-shadows which is usually seen in other forms of obstruction. At laparotomy the small intestine is distended and filled with a hard rubbery mass. Areas of the distended bowel are discoloured, and one or more perforations may be present. Volvulus of the intestine is occasionally found. The ileum tapers to a narrow string-like colon which is small, not because it has failed to develop, but because it has remained completely empty. As lately as 1945, the condition was regarded as uniformly fatal,8 but since then successfully treated cases have been recorded.4 9-11 The first case in which surgery was attempted was reported in 1929.6 Montgomery 11 has recently reported a further case, in which the baby continued to thrive for a year after operation. The mortality from operation is gradually falling ; but 1. Landsteiner, K. Zbl. allg. Path. path. Anat. 1905, 16, 903. 2. Andersen, D. H. Amer. J. Dis. Child. 1938, 56, 344. 3. Farber, S. J. Pediat. 1944, 24, 387. 4. Bodian, M. Fibrocystic Disease of the Pancreas. London, 1952. 5. Hurwitt, E. S., Arnheim, E. E. Amer. J. Dis. Child. 1942, 64, 443. 6. Kornblith, B. A., Otani, S. Amer. J Path. 1929, 5, 249. 7. Nuihauser, E. B. D. Radiology, 1946, 46, 319. 8. Swenson, O., Ladd, W. E. New Engl. J. Med. 1945, 233, 660. 9. Hiatt, R. B., Wilson, P. E. Surg. Gynec. Obstet. 1948, 87, 317. 10. May, C. D., Lowe, C. U. J. Pediat. 1949, 34, 663. 11. Montgomery, W. F. J. Amer. med. Ass. 1953, 152, 225,
between the surgeon and the
pædiatrician.
FREEDOM TO PRESCRIBE THE maintenance of professional freedom against the incursions of central control remains a matter of deep coiieerit to us all. But we must recognise that there will always be two sides to the question when the freedom we claim implies freedom for the individual doctor ’td In the letter on proprietary spenci public money. medicines which he has sent to all National Health Service doctors this week (see p. 194) Sir John Charles points out that the practitioner may be called on to justify the cost of his prescribing to his colleagues on the local medical committee." As everyone knows, some members of our profession acquire a particular affection for certain remedies. If they happen to put their faith in the excellent mist. gent. cum rheo. alk. nobody is likely to raise objections ; but if, on the other hand, they persistently prescribe expensive drugs on official forms, sooner or later they will be asked to justify their beliefs. Such a case has recently attracted considerable public attention in Belfast, where a practitioner prescribed ’Anahaemin ’ very freely, particularly for patients with neoplastic In support of this practice he instanced the diseased case of a cancer patient who had been declared inoperable by the Royal Victoria Hospital but after treatment with anahæmin recovered sufficiently to return to work. Obviously in such a case there are three possibilities : (1) that the anahsemin checked the progress of the disease ; (2) that, without affecting the cancer, the anahæmin, or its administration, had a tonic effect on the patient ; and (3) that this particular patient would have had a remission of symptoms even if nothing had been done for him. Important contributions have been made in the past by practitioners who noticed therapeutic effects which had escaped other observers, and who stuck to their unorthodox opinions. The same thing will happen again ; and it is important that in the National Health Service, as elsewhere, a doctor who believes that a remedy is effective should be able to continue to use it unless or until an adequate investigation by independent observers shows that he is mistaken, or at least that the advantage gained is insufficient. The Belfast case was particularly unsatisfactory. First, the newspapers gave prominence to it, basing their initial account on the minutes of the Northern Ireland General Health Services Board ; but the report of a " special subcommittee to review the doctor’s prescribing of Anahæmin and consider in general the question of prescribing in incurable disease " was heard in camera. It seems to us desirable that when matters of public interest and matters affecting the public purse are involved, the cases should be freely presented to the public. Secondly, though it was clear from the previous proceedings that there was considerable sympathy with the doctor concerned, he thought it necessary to resign from the health service while his case was under consideration. As a matter of principle was involved, it would have been far better to await the decision of the board. If the decision had been such as would threaten professional freedom, powerful support for the doctor would certainly have been forthcoming from the professional associations. If on the other hand the board had clearly established that it was merely curbing an expensive and unfounded whim, its reasons should have been clearly presented to the public. 1.
Belfast News-Letter, June 25, 1953.
open to them if they become statf nurses or unfortunately survival after operation is not the same as to to them a is attract, hard What cure. As nearly all these infants have fibrocystic disease sisters. there, then, and harassment of the of the pancreas the alimentary and respiratory changes with examinations, long hours, job when they could do the same work for nearly the same characteristic of this condition almost inevitably develop later on and eventually prove fatal, T’he management pay and without the threat of the examiner, or—by of those who survive operation requires close coöperation becoming either a domestic worker or a laundrymaid
prospects
---
do easier work pay?A handy and exceptionally an institution for mental defectives, in maid intelligent when asked why she did not transfer to the nursing statf, replied without hesitation : "I’m better off as I am ; and I get my evenings and weekends." It was the reply of common sense, and deserves attention. Young women choosing to look after the mentally sick cannot, and do not, expect to be paid at excessive rates : for But it is one thing the country could not afford it. surely time we recognised, however modestly, that the work they do is more personal, responsible, and exacting than that of a laundry or domestic maid. for
more
MECONIUM ILEUS MECONIUM ileus is one of the less common causes of intestinal obstruction in the newborn baby. The obstruction arises from the presence in the small bowel of firm, putty-like, extremely sticky meconium which clings to the mucosa and clogs the lumen. The abnormal qualities of the meconium are due to the absence of pancreatic secretions-a relationship first recognised by Landsteiner1 in 1905, although he mistakenly concluded that the obstruction. pancreatic changes were due to the intestinal The work of Andersen2 and Farber 3 has shown that the absence of pancreatic secretion is the primary disturbance, and the condition is nearly alwaysassociated with fibrocystic disease of the pancreas-Bodian4 has estimated that meconium ileus occurs in one out of five cases of this disease. Occasionally meconium ileus is associated with atresia or stenosis of the main pancreatic duct.66 The usual history is that of any form of intestinal obstruction in the newborn-abdominal distension, vomiting, and failure to pass meconium. The abdomen may feel rather doughy or lumpy ; but otherwise there are no characteristic features, and respiratory symptoms, which are a distinct feature of fibrocystic disease in older infants and children, are not present at birth. Some help in making a preoperative diagnosis may be had from the family history, since more than one child is often affected, and from a plain radiograph of the abdomen, which may show the presence of small bubbles of gas in 7 The radiograph also amass of inspissated meconium. of that termination of the intesshows absence abrupt tinal gas-shadows which is usually seen in other forms of obstruction. At laparotomy the small intestine is distended and filled with a hard rubbery mass. Areas of the distended bowel are discoloured, and one or more perforations may be present. Volvulus of the intestine is occasionally found. The ileum tapers to a narrow string-like colon which is small, not because it has failed to develop, but because it has remained completely empty. As lately as 1945, the condition was regarded as uniformly fatal,8 but since then successfully treated cases have been recorded.4 9-11 The first case in which surgery was attempted was reported in 1929.6 Montgomery 11 has recently reported a further case, in which the baby continued to thrive for a year after operation. The mortality from operation is gradually falling ; but 1. Landsteiner, K. Zbl. allg. Path. path. Anat. 1905, 16, 903. 2. Andersen, D. H. Amer. J. Dis. Child. 1938, 56, 344. 3. Farber, S. J. Pediat. 1944, 24, 387. 4. Bodian, M. Fibrocystic Disease of the Pancreas. London, 1952. 5. Hurwitt, E. S., Arnheim, E. E. Amer. J. Dis. Child. 1942, 64, 443. 6. Kornblith, B. A., Otani, S. Amer. J Path. 1929, 5, 249. 7. Nuihauser, E. B. D. Radiology, 1946, 46, 319. 8. Swenson, O., Ladd, W. E. New Engl. J. Med. 1945, 233, 660. 9. Hiatt, R. B., Wilson, P. E. Surg. Gynec. Obstet. 1948, 87, 317. 10. May, C. D., Lowe, C. U. J. Pediat. 1949, 34, 663. 11. Montgomery, W. F. J. Amer. med. Ass. 1953, 152, 225,
between the surgeon and the
pædiatrician.
FREEDOM TO PRESCRIBE THE maintenance of professional freedom against the incursions of central control remains a matter of deep coiieerit to us all. But we must recognise that there will always be two sides to the question when the freedom we claim implies freedom for the individual doctor ’td In the letter on proprietary spenci public money. medicines which he has sent to all National Health Service doctors this week (see p. 194) Sir John Charles points out that the practitioner may be called on to justify the cost of his prescribing to his colleagues on the local medical committee." As everyone knows, some members of our profession acquire a particular affection for certain remedies. If they happen to put their faith in the excellent mist. gent. cum rheo. alk. nobody is likely to raise objections ; but if, on the other hand, they persistently prescribe expensive drugs on official forms, sooner or later they will be asked to justify their beliefs. Such a case has recently attracted considerable public attention in Belfast, where a practitioner prescribed ’Anahaemin ’ very freely, particularly for patients with neoplastic In support of this practice he instanced the diseased case of a cancer patient who had been declared inoperable by the Royal Victoria Hospital but after treatment with anahæmin recovered sufficiently to return to work. Obviously in such a case there are three possibilities : (1) that the anahsemin checked the progress of the disease ; (2) that, without affecting the cancer, the anahæmin, or its administration, had a tonic effect on the patient ; and (3) that this particular patient would have had a remission of symptoms even if nothing had been done for him. Important contributions have been made in the past by practitioners who noticed therapeutic effects which had escaped other observers, and who stuck to their unorthodox opinions. The same thing will happen again ; and it is important that in the National Health Service, as elsewhere, a doctor who believes that a remedy is effective should be able to continue to use it unless or until an adequate investigation by independent observers shows that he is mistaken, or at least that the advantage gained is insufficient. The Belfast case was particularly unsatisfactory. First, the newspapers gave prominence to it, basing their initial account on the minutes of the Northern Ireland General Health Services Board ; but the report of a " special subcommittee to review the doctor’s prescribing of Anahæmin and consider in general the question of prescribing in incurable disease " was heard in camera. It seems to us desirable that when matters of public interest and matters affecting the public purse are involved, the cases should be freely presented to the public. Secondly, though it was clear from the previous proceedings that there was considerable sympathy with the doctor concerned, he thought it necessary to resign from the health service while his case was under consideration. As a matter of principle was involved, it would have been far better to await the decision of the board. If the decision had been such as would threaten professional freedom, powerful support for the doctor would certainly have been forthcoming from the professional associations. If on the other hand the board had clearly established that it was merely curbing an expensive and unfounded whim, its reasons should have been clearly presented to the public. 1.
Belfast News-Letter, June 25, 1953.