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THE VULNERABLE ŒSOPHAGUS
779
by histological examination. I have always chosen biopsy, preferably with frozen section. If a doubtful report is given on the frozen section, then the lesser operation is performed. If a paraffin section later proves malignancy, then and only then is an extensive resection carried out. Some surgeons prefer to excise the ulcer for histological examination. nancy is
"
West London Hospital, London W.6.
"
HAROLD BURGE.
THE VULNERABLE ŒSOPHAGUS
SIR,-With regard to oesophageal reflux and hiatus hernia recently discussed in your columns (Aug. 3, p. 267), is it not a great pity that this condition is not more closely related to eating when there is no real desire for food, or for that particular food ? This is set out at some length in the joint work by myself and Campbel1,1 in which the instinct of appetite is shown to be grossly thwarted under modern conditions, by the eating of arbitrary meals and arbitrary food mixtures. Ivy and Farley,2 confirming the work of earlier investigators, showed how closely the emptying-time of the stomach is determined by the amount of hunger present. Speaking personally, I have never had acid eructations into the back of the throat without being able to relate them to eating a meal I did not fancy, and very rarely have I found with patients that I was not able to effect a striking improvement in this symptom by directing their attention to this point. The matter is being elaborated in the new edition of our work. Catisfield, Fareham, Hants.
T. L. CLEAVE.
AND PARTIAL DELETIONS OF CHROMOSOME 18 SIR,-Several investigators have recently reported abnormalities of serum-immunoglobulins associated with a deletion of chromosome 18. A deficiency of serum-IgA was reported by Dr. Finley and her colleagues in a patient with a ring-18 chromosome,3 and by Feingold et al. in a girl with a long-arm deletion and a boy with a ring-18 chromosome.4 Daentle and Smith have made a similar observation in a child with deletion of the short arm of chromosome 18 who also had rheumatoid arthritis.5 On the other hand, Dr. Richards and Dr. Hobbs have reported a patient with a ring-18 chromosome, normal serum-IgA level, and borderline low salivary IgA.Among the above patients, two had a history of frequent infections. We have recently studied the immunoglobulins in two patients with abnormalities of chromosome 18. Case 1.-This is a 31/2-year-old Caucasian girl with small stature, mental retardation (i.Q. 42), and many of the features of a syndrome associated with deletion of chromosome 18 first described by de Grouchyand Lejeune 8-e.g., maxillary hypoplasia, stenotic ear-canals, and hearing loss. She has not had frequent infections. Chromosomal analysis of leucocytes and fibroblasts showed deletion of the long arm of chromosome 18 (46XX, 18q-) in all cells studied. Immunoglobulin assay of serum and parotid secretions showed an absence of IgA in both; other family members were normal. Case 2.-This is an 18-month-old Caucasian boy with mental retardation (I.Q. 43), microcephaly, and multiple congenital anomalies including a carp-shaped mouth and congenital heartdisease. Chromosomal analysis showed a ring-18 chromosome
IgA
1.
Cleave,
T. L., Campbell, G. D. The Saccharine Disease; p. 99. Bristol, 1966. 2. Ivy, A. C., Farley, G. B. Am. J. Physiol. 1929, 91, 205. 3. Finley, S. C., Finley, W. H., Nato, T. A., Uchida, I. A., Roddam, R. F. Lancet, 1968, i, 1095. 4. Feingold, M., Schwartz, R. S., Atkins, L., Anderson, R., Bartsocas, C. S. Page, D. L., Littlefield, J. W. J. clin. Invest. 1968, 47, 34. 5. Daentle, D., Smith, D. Personal communication. 6. Richards, B. W., Hobbs, J. R. Lancet, 1968, i, 1426. 7. de Grouchy, J. J. Pediat. 1965, 66, 414. 8. Lejeune, J., Berger, R. E., Lafourcade, J. A., Rethore, M. Annls Génét.
1966, 9,
32.
(46XY, 18r) in a portion of leucocytes and fibroblasts. Serum and salivary immunoglobulin estimations on the patient and his family (father not available) were normal. There was no history of repeated infections. Comment.-In patient 1, in whom mosaicism has not been demonstrated, there is an absence of salivary and serum IgA. On the other hand, IgA levels are normal in patient 2, though he, too, has a presumed deletion of chromosomal material from the long arm of 18. This may be explained by the mosaic state, with a sufficient number of normal cells being present to provide adequate levels of IgA. However, in view of the reports by Dr. Richards and Dr. Hobbs6 and by Daentle and Smith,5 one consider the possibility that the effect of the chromosomal deletion on IgA may be non-specific. must
A more extensive discussion of our findings and their implications is being prepared for publication in the near future. This work was aided by a grant from the National Foundation, and is contribution no. 339 of this Department of Biophysics. Department of Pediatrics, University of Colorado Medical Center, JANET STEWART. SUMIO GO University of Colorado Medical Center ELLIOT ELLIS. and National Jewish Hospital, Department of Biophysics, ARTHUR ROBINSON. University of Colorado Medical Center, Colorado 80220. Deliver,
THE YY SYNDROME letter of Dr. Borgaonkar and his colleagues (Aug. SiR,ŅThe 24, p. 461) prompts us to report a recent case of a boy of 16 with the chromosome constitution 47, XYY. We examined this boy mainly because of his tall stature-205 cm. (6 ft. 10 in.). He gets good marks at his secondary school and has an i.Q. of 116 on the Stanford-Binet scale. He has an undoubtedly deviant personality, showing obsessive and compulsive traits, difficulty in establishing natural relations with other people, and abnormal shyness. One can discern a tendency to overaggressiveness at times, but so far he has shown no signs of an antisocial attitude. We agree with Dr. Borgaonkar and his colleagues that one must guard against jumping to conclusions about the mental qualities associated with the YY abnormality. To learn about these, in our opinion, we must follow a series of YY males, uncovered in an unselected population, old enough for us to be able to judge their mental capacity and ability to adjust. We have no doubt, nevertheless, that there is some form of link between an extra Y chromosome and antisocial conduct. This has been clearly indicated by the work of Price and Whatmore1 and others. HANS FORSSMAN Psychiatric Research Centre, HANS OLOF ÅKESSON St. Jörgen Hospital, LEIF WALLIN. Sweden.
BASAL INSULIN AND OBESITY SIR,-I read with interest the paper by Dr. Bagdade (Sept. 14, p. 630), and should like to present some of my own findings. By measuring the insulin response to oral glucose-tolerance tests in four obese patients with hyperinsulinism, I was able to show that the insulin levels had fallen to within normal limits after successful weight reduction in all four patients.2 The result of this study, however, did not establish whether hyperinsulinism was the cause or effect of obesity. Further work with volunteers showed that it was possible to create hyperinsulinism experimentally, in response to a longcontinued and artificially high sucrose diet, in six out of nineteen healthy subjects.3 In addition to hyperinsulinism, these same six subjects showed a significant increase in weight after their consumption of the high-sucrose diet, although there was no significant increase in caloric intake. These findings are in line with the work of Butterfield,4 who Price, W. H., Whatmore, P. B. Br. med. J. 1967, i, 533. Szanto, S. Proc. Nutr. Soc. 1968, 27, 11A. 3. Szanto, S., Yudkin, J. Unpublished. 4. Butterfield, W. J. H. J. R. Coll. Physns Lond. 1967, 1, 276. 1. 2.
by histological examination. I have always chosen biopsy, preferably with frozen section. If a doubtful report is given on the frozen section, then the lesser operation is performed. If a paraffin section later proves malignancy, then and only then is an extensive resection carried out. Some surgeons prefer to excise the ulcer for histological examination. nancy is
"
West London Hospital, London W.6.
"
HAROLD BURGE.
THE VULNERABLE ŒSOPHAGUS
SIR,-With regard to oesophageal reflux and hiatus hernia recently discussed in your columns (Aug. 3, p. 267), is it not a great pity that this condition is not more closely related to eating when there is no real desire for food, or for that particular food ? This is set out at some length in the joint work by myself and Campbel1,1 in which the instinct of appetite is shown to be grossly thwarted under modern conditions, by the eating of arbitrary meals and arbitrary food mixtures. Ivy and Farley,2 confirming the work of earlier investigators, showed how closely the emptying-time of the stomach is determined by the amount of hunger present. Speaking personally, I have never had acid eructations into the back of the throat without being able to relate them to eating a meal I did not fancy, and very rarely have I found with patients that I was not able to effect a striking improvement in this symptom by directing their attention to this point. The matter is being elaborated in the new edition of our work. Catisfield, Fareham, Hants.
T. L. CLEAVE.
AND PARTIAL DELETIONS OF CHROMOSOME 18 SIR,-Several investigators have recently reported abnormalities of serum-immunoglobulins associated with a deletion of chromosome 18. A deficiency of serum-IgA was reported by Dr. Finley and her colleagues in a patient with a ring-18 chromosome,3 and by Feingold et al. in a girl with a long-arm deletion and a boy with a ring-18 chromosome.4 Daentle and Smith have made a similar observation in a child with deletion of the short arm of chromosome 18 who also had rheumatoid arthritis.5 On the other hand, Dr. Richards and Dr. Hobbs have reported a patient with a ring-18 chromosome, normal serum-IgA level, and borderline low salivary IgA.Among the above patients, two had a history of frequent infections. We have recently studied the immunoglobulins in two patients with abnormalities of chromosome 18. Case 1.-This is a 31/2-year-old Caucasian girl with small stature, mental retardation (i.Q. 42), and many of the features of a syndrome associated with deletion of chromosome 18 first described by de Grouchyand Lejeune 8-e.g., maxillary hypoplasia, stenotic ear-canals, and hearing loss. She has not had frequent infections. Chromosomal analysis of leucocytes and fibroblasts showed deletion of the long arm of chromosome 18 (46XX, 18q-) in all cells studied. Immunoglobulin assay of serum and parotid secretions showed an absence of IgA in both; other family members were normal. Case 2.-This is an 18-month-old Caucasian boy with mental retardation (I.Q. 43), microcephaly, and multiple congenital anomalies including a carp-shaped mouth and congenital heartdisease. Chromosomal analysis showed a ring-18 chromosome
IgA
1.
Cleave,
T. L., Campbell, G. D. The Saccharine Disease; p. 99. Bristol, 1966. 2. Ivy, A. C., Farley, G. B. Am. J. Physiol. 1929, 91, 205. 3. Finley, S. C., Finley, W. H., Nato, T. A., Uchida, I. A., Roddam, R. F. Lancet, 1968, i, 1095. 4. Feingold, M., Schwartz, R. S., Atkins, L., Anderson, R., Bartsocas, C. S. Page, D. L., Littlefield, J. W. J. clin. Invest. 1968, 47, 34. 5. Daentle, D., Smith, D. Personal communication. 6. Richards, B. W., Hobbs, J. R. Lancet, 1968, i, 1426. 7. de Grouchy, J. J. Pediat. 1965, 66, 414. 8. Lejeune, J., Berger, R. E., Lafourcade, J. A., Rethore, M. Annls Génét.
1966, 9,
32.
(46XY, 18r) in a portion of leucocytes and fibroblasts. Serum and salivary immunoglobulin estimations on the patient and his family (father not available) were normal. There was no history of repeated infections. Comment.-In patient 1, in whom mosaicism has not been demonstrated, there is an absence of salivary and serum IgA. On the other hand, IgA levels are normal in patient 2, though he, too, has a presumed deletion of chromosomal material from the long arm of 18. This may be explained by the mosaic state, with a sufficient number of normal cells being present to provide adequate levels of IgA. However, in view of the reports by Dr. Richards and Dr. Hobbs6 and by Daentle and Smith,5 one consider the possibility that the effect of the chromosomal deletion on IgA may be non-specific. must
A more extensive discussion of our findings and their implications is being prepared for publication in the near future. This work was aided by a grant from the National Foundation, and is contribution no. 339 of this Department of Biophysics. Department of Pediatrics, University of Colorado Medical Center, JANET STEWART. SUMIO GO University of Colorado Medical Center ELLIOT ELLIS. and National Jewish Hospital, Department of Biophysics, ARTHUR ROBINSON. University of Colorado Medical Center, Colorado 80220. Deliver,
THE YY SYNDROME letter of Dr. Borgaonkar and his colleagues (Aug. SiR,ŅThe 24, p. 461) prompts us to report a recent case of a boy of 16 with the chromosome constitution 47, XYY. We examined this boy mainly because of his tall stature-205 cm. (6 ft. 10 in.). He gets good marks at his secondary school and has an i.Q. of 116 on the Stanford-Binet scale. He has an undoubtedly deviant personality, showing obsessive and compulsive traits, difficulty in establishing natural relations with other people, and abnormal shyness. One can discern a tendency to overaggressiveness at times, but so far he has shown no signs of an antisocial attitude. We agree with Dr. Borgaonkar and his colleagues that one must guard against jumping to conclusions about the mental qualities associated with the YY abnormality. To learn about these, in our opinion, we must follow a series of YY males, uncovered in an unselected population, old enough for us to be able to judge their mental capacity and ability to adjust. We have no doubt, nevertheless, that there is some form of link between an extra Y chromosome and antisocial conduct. This has been clearly indicated by the work of Price and Whatmore1 and others. HANS FORSSMAN Psychiatric Research Centre, HANS OLOF ÅKESSON St. Jörgen Hospital, LEIF WALLIN. Sweden.
BASAL INSULIN AND OBESITY SIR,-I read with interest the paper by Dr. Bagdade (Sept. 14, p. 630), and should like to present some of my own findings. By measuring the insulin response to oral glucose-tolerance tests in four obese patients with hyperinsulinism, I was able to show that the insulin levels had fallen to within normal limits after successful weight reduction in all four patients.2 The result of this study, however, did not establish whether hyperinsulinism was the cause or effect of obesity. Further work with volunteers showed that it was possible to create hyperinsulinism experimentally, in response to a longcontinued and artificially high sucrose diet, in six out of nineteen healthy subjects.3 In addition to hyperinsulinism, these same six subjects showed a significant increase in weight after their consumption of the high-sucrose diet, although there was no significant increase in caloric intake. These findings are in line with the work of Butterfield,4 who Price, W. H., Whatmore, P. B. Br. med. J. 1967, i, 533. Szanto, S. Proc. Nutr. Soc. 1968, 27, 11A. 3. Szanto, S., Yudkin, J. Unpublished. 4. Butterfield, W. J. H. J. R. Coll. Physns Lond. 1967, 1, 276. 1. 2.