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Marfan syndrome, arachnodactyly, and cardiac abnormalities
EDITORIAL CORRESPONDENCE
Editorial correspondence or letters to the Editor relative to articles published in THE JOURNAL or to topics of current interest are subject to critical review and to current editorial policy in respect to publication in part or in full.
Marfan syndrome, arachnodactyly, and cardiac abnormalities To the Editor: I read Gruber and associates' article? "Marfan syndrome with contractural arachnodactyly and severe mitral regurgitation in a premature infant," with interest. In 1954 we ~ reported a 9-month-old boy with Marfan syndrome and contractures of the fingers, arthrogryposis, and signs of Werdnig-Hoffman disease. This infant died and pertinent cardiac findings were medial cystic necrosis of the aorta with aneurysm formation, and fenestrated and nodular mitral and tricuspid valves which consisted of fibrous tissue. Perhaps a review of the pathologic reports of Marfan syndrome with congenital contractures may reveal similar cardiac malformations. Testing for homocystinuria was not available to us in 1954. Howard S. Traisman, M.D. Professor of Pediatrics Northwestern University Medical School Children's Memorial Hospital 2300 N. Childrens Plaza Chicago, 1L 60614 REFERENCES 1. Gruber MA, Graham TP Jr, Engel E, and Smith C: Marfan syndrome with contractural arachnodactyly and severe mitral regurgitation in a premature infant, J PEDIATR93:80, 1978. Traisman HS, and Johnson FR: Arachnodactyly associated with aneurysm of the aorta, Am J Dis Child 87:156, 1954.
Immunoglobulins of human colostrum and milk To the Editor: In the April, 1978, issue of THE JOURNAL, Ogra and Ogra l described the immunologic aspects of human colostrum and
milk. I have similar studies on human colostrum and transitional milk in Turkey Thirty-two pregnant women were studied at the time of delivery. The subjects ranged from 16 to 40 years and were in good health with a mean weight of 60.37 kg _ 8.74. All were Caucasian and Turkish women, 21 being nulliparous and 11 multiparous. Serum specimens were taken at admission; colostrum samples were collected on the first day of delivery and milk samples on the sixth postpartum day. Specimens of serum were also taken from 16 nonpregnant women not receiving contraceptive pills; the mean age of this group was 25.8 years. All samples of colostrum and milk were defatted by centrifugation. Their total protein content was determined by the nesslerization method. ~ Immunogtobulin, ceruloplasmin, and transferrin determinations were done by the radial immunodiffusion method of Mancini et al? The secretory piece antisera (kindly supplied by Dr. D. S. Rowe from Switzerland and by Dr. L. A. Hanson from Sweden) were used in the Ouchterlony test for each specimen? The antikappa and antilambda L chains, the IgG anti-Fab, anti-Fd, and anti-Fc antisera were used in the Ouchterlony test for each colostrum or milk specimen. The results are summarized in the Tables. Serum levels of IgG and IgM levels were significantly higher in nonpregnant women. Serum ceruloplasmin and transferrin levels were higher in pregnant women. The highest levels of IgM and IgA were observed in the colostrum, IgD was detected in only nine colostrum specimens. Transferrin levels were below 50 mg/dl in colostrum and diminished considerably in the milk on the sixth day. The IgA and IgM continued to predominate in the milk. Ceruloplasmin levels were at the same concentration in colostrum and in transitional milk. The secretory piece of IgA was present in both colostrum and milk specimens. The IgG Fab and Fc fragments decreased in the milk, but the Fd was found in trace in both colostrum and milk specimens. The kappa and lambda chains did not change. These results reveal that colostrum is rich in IgM, IgA, IgG, and IgD. The levels of IgA and IgM continue to remain high in milk, suggesting local production or selective concentration. Similarly to IgA, lgM in the mammary gland may be from immunocompetent B-lymphocytes migrating from the Peyer patches in the intestine to the stroma of the mammary glands." ~-' Breast-feeding during the first week may provide the infant high
The Journal of PEDIATRICS
49 7
Editorial correspondence or letters to the Editor relative to articles published in THE JOURNAL or to topics of current interest are subject to critical review and to current editorial policy in respect to publication in part or in full.
Marfan syndrome, arachnodactyly, and cardiac abnormalities To the Editor: I read Gruber and associates' article? "Marfan syndrome with contractural arachnodactyly and severe mitral regurgitation in a premature infant," with interest. In 1954 we ~ reported a 9-month-old boy with Marfan syndrome and contractures of the fingers, arthrogryposis, and signs of Werdnig-Hoffman disease. This infant died and pertinent cardiac findings were medial cystic necrosis of the aorta with aneurysm formation, and fenestrated and nodular mitral and tricuspid valves which consisted of fibrous tissue. Perhaps a review of the pathologic reports of Marfan syndrome with congenital contractures may reveal similar cardiac malformations. Testing for homocystinuria was not available to us in 1954. Howard S. Traisman, M.D. Professor of Pediatrics Northwestern University Medical School Children's Memorial Hospital 2300 N. Childrens Plaza Chicago, 1L 60614 REFERENCES 1. Gruber MA, Graham TP Jr, Engel E, and Smith C: Marfan syndrome with contractural arachnodactyly and severe mitral regurgitation in a premature infant, J PEDIATR93:80, 1978. Traisman HS, and Johnson FR: Arachnodactyly associated with aneurysm of the aorta, Am J Dis Child 87:156, 1954.
Immunoglobulins of human colostrum and milk To the Editor: In the April, 1978, issue of THE JOURNAL, Ogra and Ogra l described the immunologic aspects of human colostrum and
milk. I have similar studies on human colostrum and transitional milk in Turkey Thirty-two pregnant women were studied at the time of delivery. The subjects ranged from 16 to 40 years and were in good health with a mean weight of 60.37 kg _ 8.74. All were Caucasian and Turkish women, 21 being nulliparous and 11 multiparous. Serum specimens were taken at admission; colostrum samples were collected on the first day of delivery and milk samples on the sixth postpartum day. Specimens of serum were also taken from 16 nonpregnant women not receiving contraceptive pills; the mean age of this group was 25.8 years. All samples of colostrum and milk were defatted by centrifugation. Their total protein content was determined by the nesslerization method. ~ Immunogtobulin, ceruloplasmin, and transferrin determinations were done by the radial immunodiffusion method of Mancini et al? The secretory piece antisera (kindly supplied by Dr. D. S. Rowe from Switzerland and by Dr. L. A. Hanson from Sweden) were used in the Ouchterlony test for each specimen? The antikappa and antilambda L chains, the IgG anti-Fab, anti-Fd, and anti-Fc antisera were used in the Ouchterlony test for each colostrum or milk specimen. The results are summarized in the Tables. Serum levels of IgG and IgM levels were significantly higher in nonpregnant women. Serum ceruloplasmin and transferrin levels were higher in pregnant women. The highest levels of IgM and IgA were observed in the colostrum, IgD was detected in only nine colostrum specimens. Transferrin levels were below 50 mg/dl in colostrum and diminished considerably in the milk on the sixth day. The IgA and IgM continued to predominate in the milk. Ceruloplasmin levels were at the same concentration in colostrum and in transitional milk. The secretory piece of IgA was present in both colostrum and milk specimens. The IgG Fab and Fc fragments decreased in the milk, but the Fd was found in trace in both colostrum and milk specimens. The kappa and lambda chains did not change. These results reveal that colostrum is rich in IgM, IgA, IgG, and IgD. The levels of IgA and IgM continue to remain high in milk, suggesting local production or selective concentration. Similarly to IgA, lgM in the mammary gland may be from immunocompetent B-lymphocytes migrating from the Peyer patches in the intestine to the stroma of the mammary glands." ~-' Breast-feeding during the first week may provide the infant high
The Journal of PEDIATRICS
49 7