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Immunologic Responsiveness in Phenylketonuria FREE

Abba I. Terr, MD; Richard J. Allen, MD; Neal A. Vanselow, MD
[+] Author Affiliations

Reprint requests to 1405 E Ann St, Ann Arbor, Mich 48104 (Dr. Vanselow).


From the departments of internal medicine (Drs. Terr and Vanselow) and pediatrics (Dr. Allen), University of Michigan Medical Center, Ann Arbor. Dr. Terr is recipient of Public Health Service Research Career Development award 9-K3-AM-25428 and is now in the Department of Medicine, Western Reserve University School of Medicine, Cleveland.; John C. Heffelfinger, MD, and the staff of the Coldwater (Mich) State Home and Training School gave assistance to this study.


JAMA. 1966;198(11):1185-1188. doi:10.1001/jama.1966.03110240093033
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Recent studies have demonstrated that administration of the amino acid phenylalanine (L-phenylalanine) to experimental animals will result in partial suppression of antibody responses to diptheria toxoid and sheep red blood cells and will also cause partial suppression of homograft rejection.1,2 Since patients with phenylketonuria may have markedly elevated serum phenylalanine levels, the present study was undertaken to determine if immunologic responsiveness is impaired in humans with this disorder. Antibody responses following immunization with diphtheria toxoid and Escherichia coli Vi antigen, the development of delayed hypersensitivity following administration of BCG vaccine, and serum immunoglobulin levels were studied in phenylketonuric children on normal diets and on standard low-phenylalanine diets. Similar studies were performed in nonphenylketonuric controls.

Materials and Methods 

Subjects.—  Four groups of subjects (Table 1) were used for this study:

Group A.—  Children with phenylketonuria (PKU currently on a standard low-phenylalanine diet with biochemical controls.

Group B.—  Control

REFERENCES

Ryan, W., and Carver, M.:  Inhibition of Antibody Synthesis by L-Phenylalanine , Science 143:479-480 ( (Jan) ) 1964;.
Ryan, W.:  Inhibition of the Immune Response by Phenylalanine , JAMA 191:295-296 ( (Jan 25) ) 1965;.
Stavitsky, A.:  Micromethods for the Study of Proteins and Antibodies: I. Procedure and General Applications of Hemagglutination and Hemagglutination-Inhibition Reactions With Tanic Acid and Protein-Treated Red Blood Cells , J Immunol 72:360-367 ( (May) ) 1954;.
Landy, M., and Lamb, E.:  Estimation of Vi Antibody Employing Erythrocytes Treated With Purified Vi Antigen , Proc Soc Exp Biol Med 82:593-598 ( (April) ) 1953;.
LaDu, B., and Michael, P.:  An Enzymatic Spectrophotometric Method for the Determination of Phenylalanine in Blood , J Lab Clin Med 55:491-496 ( (March) ) 1960;.
Fahey, J., and McKelvey, E.:  Quantitative Determination of Serum Immunoglobulins in Antibody-Agar Plates , J Immunol 94:84-90 ( (Jan) ) 1965;.
Landy, M.:  Studies on Vi Antigen: VI. Immunization of Human Beings With Purified Vi Antigen , Amer J Hyg 60:52-62 ( (July) ) 1954;.
Neame, K.:  Phenylalanine as Inhibitor of Transport of Amino Acids in Brain , Nature 192:173 ( (Oct 14) ) 1961;.
Oates, J., et al:  Conversion of Phenylalanine to Penethylamine in Patients With Phenylketonuria , Proc Soc Exp Biol Med 112:1078-1081 ( (May) ) 1963;.
Umbarger, B.; Berry, H.; and Sutherland, B.:  Advances in the Management of Patients With Phenylketonuria , JAMA 193: 784-790 ( (Sept 6) ) 1965;.
Krebs, E.:  Depression of Gamma Globulin in Hypoproteinemia Due to Malnutrition , J Lab Clin Med 31:85-89 ( (Jan) ) 1946;.
Bieler, M.; Ecker, E.; and Spies, T.:  Serum Proteins in Hypoproteinemia Due to Nutritional Deficiency , J Lab Clin Med 32:130-138 ( (Feb) ) 1947;.
Metcoff, J., et al:  Nutritional Status and Infection Response , J Lab Clin Med 33:47-66 ( (Jan) ) 1948;.

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Ryan, W., and Carver, M.:  Inhibition of Antibody Synthesis by L-Phenylalanine , Science 143:479-480 ( (Jan) ) 1964;.
Ryan, W.:  Inhibition of the Immune Response by Phenylalanine , JAMA 191:295-296 ( (Jan 25) ) 1965;.
Stavitsky, A.:  Micromethods for the Study of Proteins and Antibodies: I. Procedure and General Applications of Hemagglutination and Hemagglutination-Inhibition Reactions With Tanic Acid and Protein-Treated Red Blood Cells , J Immunol 72:360-367 ( (May) ) 1954;.
Landy, M., and Lamb, E.:  Estimation of Vi Antibody Employing Erythrocytes Treated With Purified Vi Antigen , Proc Soc Exp Biol Med 82:593-598 ( (April) ) 1953;.
LaDu, B., and Michael, P.:  An Enzymatic Spectrophotometric Method for the Determination of Phenylalanine in Blood , J Lab Clin Med 55:491-496 ( (March) ) 1960;.
Fahey, J., and McKelvey, E.:  Quantitative Determination of Serum Immunoglobulins in Antibody-Agar Plates , J Immunol 94:84-90 ( (Jan) ) 1965;.
Landy, M.:  Studies on Vi Antigen: VI. Immunization of Human Beings With Purified Vi Antigen , Amer J Hyg 60:52-62 ( (July) ) 1954;.
Neame, K.:  Phenylalanine as Inhibitor of Transport of Amino Acids in Brain , Nature 192:173 ( (Oct 14) ) 1961;.
Oates, J., et al:  Conversion of Phenylalanine to Penethylamine in Patients With Phenylketonuria , Proc Soc Exp Biol Med 112:1078-1081 ( (May) ) 1963;.
Umbarger, B.; Berry, H.; and Sutherland, B.:  Advances in the Management of Patients With Phenylketonuria , JAMA 193: 784-790 ( (Sept 6) ) 1965;.
Krebs, E.:  Depression of Gamma Globulin in Hypoproteinemia Due to Malnutrition , J Lab Clin Med 31:85-89 ( (Jan) ) 1946;.
Bieler, M.; Ecker, E.; and Spies, T.:  Serum Proteins in Hypoproteinemia Due to Nutritional Deficiency , J Lab Clin Med 32:130-138 ( (Feb) ) 1947;.
Metcoff, J., et al:  Nutritional Status and Infection Response , J Lab Clin Med 33:47-66 ( (Jan) ) 1948;.
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