1008
Letters to the Editor
mentally retarded." We apologize for overlooking his subsequent statement that at the time o f examination she appeared to be normally intelligent, though unable to read or write, presumably of inadequate education. He states that Case 3 "was considered mentally retarded" without subsequent clarification o f her intelligence. That patient, however, was not available for biochemical study and the diagnosis of galactokinase deficiency was made on probability in view o f the family gene. In regard to Dr. Gitzelmann's second point, we did not catalogue a "normal human trait" in a large number o f subjects. We compiled a list of three biochemical and three clinical findings in the small group of 12 previously published patients with galactokinase deficiency, and suggest that their frequency suggests these may be manifestations o f the spectrum of this disorder. Clearly, only when many more patients are identified will this be known. For the present, it would not be wise to assume that galactokinase deficiency is a benign disorder other than involvement o f the lens. Nathan Litman, M.D. Montefiore Hospital and Medical Center Albert Einstein College o f Medicine Bronx, hr. Y. REFERENCES
1. Litman N, Kanter A, and Finberg L: Galactokinase deficiency presenting as pseudotumor cerebri, J PEDIATR 86:410, 1975. 2. Gitzelmann R: Hereditary galactokinase deficiency, a newly recognized cause of juvenile cataracts, Pediatr Res 1:14, 1967.
The Journal of Pediatrics December 1975
REFERENCES
1. Swick HM: Calcification of intervertebral discs in childhood, J PEDIATR 86:364, 1975. 2. Sutton TJ, and Turcotte B: Posterior herniation of calcified 9intervertebral discs in children, J Can Assoc Radiol 24:131, 1973. 3. Peck FC: A calcified thoracic intervertebral disk with herniation and spinal cord compression in a child, J Neurosurg 14:105, 1957.
Reply To the Editor: I appreciate the opportunity to reply to the letter from Tudor Sutton, M.D., regarding my paper, "Calcification of intervertebral discs in childhood." Dr. Sutton is quite correct in stating that most children with disc calcification do not require specific therapy, particularly neurosurgical intervention. When a child presents, however, with signs of acute spinal cord compression, it is necessary to establish a diagnosis quickly, since permanent spinal cord damage may ensue from significant extrinsic compression of any etiology within only a few hours. If neuologic signs are minimal and if myelography shows only minimal compression of the spinal cord, conservative therapy with careful observation of the child's neurologic condition might be warranted. If signs of advancing deficit become apparent, however, surgery may well become necessary. H. M. Swick, M.D., F.A.A.P. Assistant Professor of Neurology and Pediatrics University of Kentucky Lexington, Ky. 40506
Calcification of intervertebral discs in childhood To the Editor: I was very interested by the article "Calcification of intervertebral discs in childhood, TM describing two children who underwent neurosurgical intervention for signs of nerve root or spinal cord compression. We have published two similar cases-' with documented regression of clinical and myelographic signs of compression under conservative management. This stands to reason when one considers the description at surgery given bS~ Swick and by Peck:' of soft disc material. We therefore do not feel neurosurgical intervention is recommended even in patients with signs of nerve root or spinal cord compression. Of course, if compression with severe neurological deficit were present, neurosurgery would be indicated. This, however, does not seem to occur in this condition. Tudor J. Sutton, M.D., F.R.C.P.(C) 185, rue du Chevaleret 75013 Paris, France
Determination of bilirubin-binding capacity of serum albumin To the Editor: I should like to comment about the paper "Fluorescent dye method for the determination of the bilirubin binding capacity of serum albumin" by Lee, Gartner, and Zarafu ~which appeared in the February issue of TH~ JOURNAL OF PEDIATRICS. A basic assumption is made both in the presentation of the results and the discussion that the decrease in fluorescence of the Duct Yellow 7 (DY7) dye is due to its displacement from the albumin molecule. No direct proof is given that this is the case, nor is there any proof that the bilirubin displaces the dye from binding to the same site on the albumin. It appears to be another indirect technique for determining bilirubin-binding capacity similar to that of the HABBA dye mentioned in their discussion. No direct correlation is given between the AF and serum
Letters to the Editor
mentally retarded." We apologize for overlooking his subsequent statement that at the time o f examination she appeared to be normally intelligent, though unable to read or write, presumably of inadequate education. He states that Case 3 "was considered mentally retarded" without subsequent clarification o f her intelligence. That patient, however, was not available for biochemical study and the diagnosis of galactokinase deficiency was made on probability in view o f the family gene. In regard to Dr. Gitzelmann's second point, we did not catalogue a "normal human trait" in a large number o f subjects. We compiled a list of three biochemical and three clinical findings in the small group of 12 previously published patients with galactokinase deficiency, and suggest that their frequency suggests these may be manifestations o f the spectrum of this disorder. Clearly, only when many more patients are identified will this be known. For the present, it would not be wise to assume that galactokinase deficiency is a benign disorder other than involvement o f the lens. Nathan Litman, M.D. Montefiore Hospital and Medical Center Albert Einstein College o f Medicine Bronx, hr. Y. REFERENCES
1. Litman N, Kanter A, and Finberg L: Galactokinase deficiency presenting as pseudotumor cerebri, J PEDIATR 86:410, 1975. 2. Gitzelmann R: Hereditary galactokinase deficiency, a newly recognized cause of juvenile cataracts, Pediatr Res 1:14, 1967.
The Journal of Pediatrics December 1975
REFERENCES
1. Swick HM: Calcification of intervertebral discs in childhood, J PEDIATR 86:364, 1975. 2. Sutton TJ, and Turcotte B: Posterior herniation of calcified 9intervertebral discs in children, J Can Assoc Radiol 24:131, 1973. 3. Peck FC: A calcified thoracic intervertebral disk with herniation and spinal cord compression in a child, J Neurosurg 14:105, 1957.
Reply To the Editor: I appreciate the opportunity to reply to the letter from Tudor Sutton, M.D., regarding my paper, "Calcification of intervertebral discs in childhood." Dr. Sutton is quite correct in stating that most children with disc calcification do not require specific therapy, particularly neurosurgical intervention. When a child presents, however, with signs of acute spinal cord compression, it is necessary to establish a diagnosis quickly, since permanent spinal cord damage may ensue from significant extrinsic compression of any etiology within only a few hours. If neuologic signs are minimal and if myelography shows only minimal compression of the spinal cord, conservative therapy with careful observation of the child's neurologic condition might be warranted. If signs of advancing deficit become apparent, however, surgery may well become necessary. H. M. Swick, M.D., F.A.A.P. Assistant Professor of Neurology and Pediatrics University of Kentucky Lexington, Ky. 40506
Calcification of intervertebral discs in childhood To the Editor: I was very interested by the article "Calcification of intervertebral discs in childhood, TM describing two children who underwent neurosurgical intervention for signs of nerve root or spinal cord compression. We have published two similar cases-' with documented regression of clinical and myelographic signs of compression under conservative management. This stands to reason when one considers the description at surgery given bS~ Swick and by Peck:' of soft disc material. We therefore do not feel neurosurgical intervention is recommended even in patients with signs of nerve root or spinal cord compression. Of course, if compression with severe neurological deficit were present, neurosurgery would be indicated. This, however, does not seem to occur in this condition. Tudor J. Sutton, M.D., F.R.C.P.(C) 185, rue du Chevaleret 75013 Paris, France
Determination of bilirubin-binding capacity of serum albumin To the Editor: I should like to comment about the paper "Fluorescent dye method for the determination of the bilirubin binding capacity of serum albumin" by Lee, Gartner, and Zarafu ~which appeared in the February issue of TH~ JOURNAL OF PEDIATRICS. A basic assumption is made both in the presentation of the results and the discussion that the decrease in fluorescence of the Duct Yellow 7 (DY7) dye is due to its displacement from the albumin molecule. No direct proof is given that this is the case, nor is there any proof that the bilirubin displaces the dye from binding to the same site on the albumin. It appears to be another indirect technique for determining bilirubin-binding capacity similar to that of the HABBA dye mentioned in their discussion. No direct correlation is given between the AF and serum
