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More on prescribing medication for pediatric patients
722
April, 1971 The Journal of P E D I A T R I C S
Letters to the Editor
Extra posterior cervical skin To the Editor: The recent publication in the JOURNAL by Drs. Shapiro, Hsu, and Hirschhorn 1 was of great interest to us. Their report described 3 infants with excess posterior cervical skin, but not webbing, who proved to have chromosomal abnormalities not previously described. In their fine discussion of the association of this physicaI finding with various cytogenetic aberrations, they did not specifically mention Turner's (XO) syndrome. An infant, recently referred to The Children's Hospital Medical Center because of progressive edema of the dorsmn of the hands and feet, was found to have excess posterior cervical skin, a shield chest with increased interareotar distance, and decreased femoral pulses. Chromosomal studies by Dr. Park Gerald revealed 45 chromosomes and XO karyotype. The finding of excess posterior cervical skin without webbing has been previously reported in Turner's syndrome. 2 We would llke to be certain Turner's (XO) syndrome is included in the list of chromosomal aberrations associated with this interesting physical finding. S. Charles Bean, M.D. Martha A. Lusser, M.D. Children's Hospital Medical Center Boston, Mass. 02155 REFERENCES
I. Shapiro, L. R., Hsu, L , and Hirschhorn, K.: Extra posterior cervical skln: A possible sign of chromosomal aberration in infancy, J. PEmAT. 77: 690, 1970. 2. Smith, D. W.: Recognizable patterns of human malformations, Philadelphia, 1970, W. B. Saunders Company, p. 57.
Reply To the Editor: In their letter concerning our report, "Extra posterior eervlcal skin: A possible sign of chromosomal aberration in infancy, TM Drs. Bean and Lusser indicate that excess posterior cervical skin without webbing has been previously reported in Turner's syndrome, and they believe VoL 78, No. 4, pp. 722-726
that Turner's syndrome should be "included in the list of chromosomal aberrations associated with this interesting physical finding." In our paper, we reported 3 different examples of chromosomal aberration, one of which was X O / X Y mosaicism (45,X/45,XY), in which extra posterior cervical skin was noted, and we also referred to a nonchromosomal disorder (cerebrohepatorenal syndrome) with the same finding. The purpose of our paper was simply to suggest that the finding of excess posterior cervical skin in an infant may be of value in raising suspicion of a nonspecified chromosomal aberration. We expect that as time goes on, additional eases of chromosomal aberrations will be observed in association with extra posterior cervical skin.
Lawrence R. Shapiro, M.D., F,A.A.P. L. Y. F. Hsu, M.D. Kurt Hirschhorn, M.D. Departments o/Pediatrics and Genetics Division o[ Medical Genetics Mr. Sinai School o[ Medicine New York, N. Y. and Letchworth Village ThielIs, N. Y. REFERENCE
1. Shapiro, L. R., Hsu, L. Y. F., and Hirschhorn, K.: Extra posterior cervical skin: A possible sign of chromosomal aberration in infancy, J. PEDIAT. 77" 690, 1970.
More on prescribing medication for pediatric patients To the Editor: Unfortunately I can only share Dr. Angella's concern regarding the well-meaning but often misguided drug abuse perpetrated upon pediatric patients by their physicians (J. PEDIAT. 77: 1092, 1970). I have recently been called to active duty in the United States Army and the problem there seems to be as rampant among army dependents as anywhere else. The progressive psychiatric programs that the army has recently initiated at certain bases to deal with the military
Volume 78 Number 4
Letters to the Editor
drug problems would do well to concern themselves early with their future soldiers, wives, and citizens. One side of the problem was outlined by G. B. Shaw in his preface to "The Doctor's Dilemma." "The demands of this poor public are not reasonable, but they are quite simple. It dreads disease and desires to be protected against it . . . what the public wants, therefore, is a cheap magic charm to prevent, and a cheap pill or potion to cure all disease. It forces all such charms on the doctors." On the other side however, we, the physicians, not only nurture and foster the demands of this poor public for potentially harmful or worthless drugs, we actually create that very demand. Infants are not born with an appetite to take a drug for every symptom--they acquire it. The most recent example of this continuing abuse, I believe, is the prescribing information currently being detailed for cyproheptadine HC1 (Periactin, Merck, Sharp & Dohme) as an appetite stimulant. The advertising material shows a distraught mother hovering over Johnny's full plate lying uneaten (Johnny incidentally looks the picture of health--within the normal percentiles for height and weight). Later, following the drugging, Johnny's plate is clean and anxious mother is ecstatic. Are we fostering and creating in this instance, at any rate, not only future drug takers and pill droppers but fat ones at that? The time has come.
Frederic W. Bruhn, M.D. Pediatric Service Ireland Army Hospital Fort Knox, Ky. 40121
Neonatal myastloenia gravis and antimuscle antibodies To the Editor: Antibodies reacting with skeletal muscular constituents are found in 30 to 75 per cent of adult patients with myasthenia gravis. 1, ~ The significance of these antibodies, as well as their role in the pathogenesis of myasthenia, is obscure. 1-s We have observed a child with transient neonatal myasthenia gravis who had high titers of antimuscle antibodies.
CASE R E P O R T A 2,400 Gm., 47 era. male infant was delivered 3 weeks before term to a 26-year-old primipara
723
who had severe myasthenia gravls and thymoma. The mother's illness began abruptly in the twentysecond week of pregnancy. Radiographic examination suggested a thymoma. Despite treatment with appropriate doses of pyridostigmin bromide (Mestinon), she had to be maintained on artificial respiration for the last few months of pregnancy and for several weeks thereafter. The clinical state then improved gradually, but the mother died 4 months after delivery, in an acute myasthenic crisis. No autopsy was performed. The infant's symptoms became evident shortly after birth and included hypotonia, inertia, diminished or absent reflexes, cyanosis, abundant pharyngeal mucous, and ptosis. Despite therapy with edrophonium chloride (Tensilon, 1 rag. twice a day) and increasing doses of Mestinon (up to 4.5 rag. per day), he rapidly developed serious respiratory difficulties. Endotracheal intubation was performed and maintained for 10 days, artificial respiration being maintained for 5 days. Feeding through a nasogastric tube was initiated 6 days after birth and maintained for 17 days. The symptoms disappeared gradually so that all medication could be stopped after 22 days. Thereafter no further treatment was necessary, and the child developed normally without any signs of disease.
LABORATORY
RESULTS
Binding of immunoglobulin from the patient's serum to constituents of skeletal muscle was measured by the indirect immunofluorescent technique, using human muscle obtained at surgery as substrate and fluorescein isothiocyanatelabeled rabbit antihuman IgG and antihuman IgM. It is referred to as antimuscle antibody. The antibody titer is expressed as the last reciprocal dilution of serum yielding the characteristic striated immunofluorescence pattern as described by others3, 4 As summarized in Table I the sera of both mother and child contained high titers of antimuscle antibodies of the IgG type 3 months after delivery. The antibody titer in the child diminished gradually, and tests were negative at 8 months. Whereas the mother had no significant titer of antibodies of the IgM type, a titer of 320 was found in the child 3 months after birth. Immunoglobulin levels were normal in the mother but were rather high in the child 1 month after birth for IgM and IgA, when measured by the radial immunodiffusion technique; they returned to normal after 3 months (Table I). DISCUSSION So far 82 children with transient neonatal myasthenia have been described? But only in 20 instances were sera of such patients assayed by indirect immunofluorescence, and most in-
April, 1971 The Journal of P E D I A T R I C S
Letters to the Editor
Extra posterior cervical skin To the Editor: The recent publication in the JOURNAL by Drs. Shapiro, Hsu, and Hirschhorn 1 was of great interest to us. Their report described 3 infants with excess posterior cervical skin, but not webbing, who proved to have chromosomal abnormalities not previously described. In their fine discussion of the association of this physicaI finding with various cytogenetic aberrations, they did not specifically mention Turner's (XO) syndrome. An infant, recently referred to The Children's Hospital Medical Center because of progressive edema of the dorsmn of the hands and feet, was found to have excess posterior cervical skin, a shield chest with increased interareotar distance, and decreased femoral pulses. Chromosomal studies by Dr. Park Gerald revealed 45 chromosomes and XO karyotype. The finding of excess posterior cervical skin without webbing has been previously reported in Turner's syndrome. 2 We would llke to be certain Turner's (XO) syndrome is included in the list of chromosomal aberrations associated with this interesting physical finding. S. Charles Bean, M.D. Martha A. Lusser, M.D. Children's Hospital Medical Center Boston, Mass. 02155 REFERENCES
I. Shapiro, L. R., Hsu, L , and Hirschhorn, K.: Extra posterior cervical skln: A possible sign of chromosomal aberration in infancy, J. PEmAT. 77: 690, 1970. 2. Smith, D. W.: Recognizable patterns of human malformations, Philadelphia, 1970, W. B. Saunders Company, p. 57.
Reply To the Editor: In their letter concerning our report, "Extra posterior eervlcal skin: A possible sign of chromosomal aberration in infancy, TM Drs. Bean and Lusser indicate that excess posterior cervical skin without webbing has been previously reported in Turner's syndrome, and they believe VoL 78, No. 4, pp. 722-726
that Turner's syndrome should be "included in the list of chromosomal aberrations associated with this interesting physical finding." In our paper, we reported 3 different examples of chromosomal aberration, one of which was X O / X Y mosaicism (45,X/45,XY), in which extra posterior cervical skin was noted, and we also referred to a nonchromosomal disorder (cerebrohepatorenal syndrome) with the same finding. The purpose of our paper was simply to suggest that the finding of excess posterior cervical skin in an infant may be of value in raising suspicion of a nonspecified chromosomal aberration. We expect that as time goes on, additional eases of chromosomal aberrations will be observed in association with extra posterior cervical skin.
Lawrence R. Shapiro, M.D., F,A.A.P. L. Y. F. Hsu, M.D. Kurt Hirschhorn, M.D. Departments o/Pediatrics and Genetics Division o[ Medical Genetics Mr. Sinai School o[ Medicine New York, N. Y. and Letchworth Village ThielIs, N. Y. REFERENCE
1. Shapiro, L. R., Hsu, L. Y. F., and Hirschhorn, K.: Extra posterior cervical skin: A possible sign of chromosomal aberration in infancy, J. PEDIAT. 77" 690, 1970.
More on prescribing medication for pediatric patients To the Editor: Unfortunately I can only share Dr. Angella's concern regarding the well-meaning but often misguided drug abuse perpetrated upon pediatric patients by their physicians (J. PEDIAT. 77: 1092, 1970). I have recently been called to active duty in the United States Army and the problem there seems to be as rampant among army dependents as anywhere else. The progressive psychiatric programs that the army has recently initiated at certain bases to deal with the military
Volume 78 Number 4
Letters to the Editor
drug problems would do well to concern themselves early with their future soldiers, wives, and citizens. One side of the problem was outlined by G. B. Shaw in his preface to "The Doctor's Dilemma." "The demands of this poor public are not reasonable, but they are quite simple. It dreads disease and desires to be protected against it . . . what the public wants, therefore, is a cheap magic charm to prevent, and a cheap pill or potion to cure all disease. It forces all such charms on the doctors." On the other side however, we, the physicians, not only nurture and foster the demands of this poor public for potentially harmful or worthless drugs, we actually create that very demand. Infants are not born with an appetite to take a drug for every symptom--they acquire it. The most recent example of this continuing abuse, I believe, is the prescribing information currently being detailed for cyproheptadine HC1 (Periactin, Merck, Sharp & Dohme) as an appetite stimulant. The advertising material shows a distraught mother hovering over Johnny's full plate lying uneaten (Johnny incidentally looks the picture of health--within the normal percentiles for height and weight). Later, following the drugging, Johnny's plate is clean and anxious mother is ecstatic. Are we fostering and creating in this instance, at any rate, not only future drug takers and pill droppers but fat ones at that? The time has come.
Frederic W. Bruhn, M.D. Pediatric Service Ireland Army Hospital Fort Knox, Ky. 40121
Neonatal myastloenia gravis and antimuscle antibodies To the Editor: Antibodies reacting with skeletal muscular constituents are found in 30 to 75 per cent of adult patients with myasthenia gravis. 1, ~ The significance of these antibodies, as well as their role in the pathogenesis of myasthenia, is obscure. 1-s We have observed a child with transient neonatal myasthenia gravis who had high titers of antimuscle antibodies.
CASE R E P O R T A 2,400 Gm., 47 era. male infant was delivered 3 weeks before term to a 26-year-old primipara
723
who had severe myasthenia gravls and thymoma. The mother's illness began abruptly in the twentysecond week of pregnancy. Radiographic examination suggested a thymoma. Despite treatment with appropriate doses of pyridostigmin bromide (Mestinon), she had to be maintained on artificial respiration for the last few months of pregnancy and for several weeks thereafter. The clinical state then improved gradually, but the mother died 4 months after delivery, in an acute myasthenic crisis. No autopsy was performed. The infant's symptoms became evident shortly after birth and included hypotonia, inertia, diminished or absent reflexes, cyanosis, abundant pharyngeal mucous, and ptosis. Despite therapy with edrophonium chloride (Tensilon, 1 rag. twice a day) and increasing doses of Mestinon (up to 4.5 rag. per day), he rapidly developed serious respiratory difficulties. Endotracheal intubation was performed and maintained for 10 days, artificial respiration being maintained for 5 days. Feeding through a nasogastric tube was initiated 6 days after birth and maintained for 17 days. The symptoms disappeared gradually so that all medication could be stopped after 22 days. Thereafter no further treatment was necessary, and the child developed normally without any signs of disease.
LABORATORY
RESULTS
Binding of immunoglobulin from the patient's serum to constituents of skeletal muscle was measured by the indirect immunofluorescent technique, using human muscle obtained at surgery as substrate and fluorescein isothiocyanatelabeled rabbit antihuman IgG and antihuman IgM. It is referred to as antimuscle antibody. The antibody titer is expressed as the last reciprocal dilution of serum yielding the characteristic striated immunofluorescence pattern as described by others3, 4 As summarized in Table I the sera of both mother and child contained high titers of antimuscle antibodies of the IgG type 3 months after delivery. The antibody titer in the child diminished gradually, and tests were negative at 8 months. Whereas the mother had no significant titer of antibodies of the IgM type, a titer of 320 was found in the child 3 months after birth. Immunoglobulin levels were normal in the mother but were rather high in the child 1 month after birth for IgM and IgA, when measured by the radial immunodiffusion technique; they returned to normal after 3 months (Table I). DISCUSSION So far 82 children with transient neonatal myasthenia have been described? But only in 20 instances were sera of such patients assayed by indirect immunofluorescence, and most in-