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Cystinosis
T H E BOOK S H E L F
L. Emmettnolt, Jr.,
Editor
Book reviews C l i n i c a l p e d i a t r i c o n t o l o g y W a t a r u W. Sutow, T e r e s a J. Vietti, a n d D o n a l d J. F e r n b a c h , editors, St. Louis, 1973, T h e C. V. M o s b y C o m p a n y , 602 pages. $35.00. This is a well-written and much needed textbook on the treatment of cancer in children. The editors have compiled the text from thirty-three authors, the majority of whom are members of the Pediatric Division of the Southwest Cancer Chemotherapy Study Group. With few exceptions, they are pediatricians, and the multiplicity of authors provides a balanced point of view regarding the merits of various treatment regimens. Most of the chapters are clearly written and include the background and rationale for the treatment suggested. Even though the therapy of cancer is a rapidly changing field, much of the content is basic information regarding the disease and will remain relevant in the years to come. The volume should be valuable to a wide range of readers. The general pediatrician will find it a useful source of reference regarding current treatment and prognosis of a specific tumor. Paramedical personnel such as nurses and social workers interested in oncology would benefit from reading the general chapters on childhood cancer, etiology, and principles of psychological support. The medical student should be interested in the chapters devoted to etiology, cell kinetics, chemotherapy, and the natural history of leukemia; the pediatric oncologists would benefit from the chapters on surgical considerations and implications of radiation therapy. The volume comprises 28 chapters; the first eight deal with general aspects of pediatric cancer and its treatment and the rest are devoted to tumors arising in specific sites. No attempt is made to include extensive discussions of histopathology and radiology, except as they influence treatment. The authors major objective is to re-emphasize the need for collaboration between the therapeutic disciplines and highlight the current progress in each area. In a brief review, it is not possible to comment on the various chapters. The discussion of etiology is clearly written and should be of interest to pediatricians as a whole and other physicians involved with cancer, since Dr. Miller details those clues which currently exist. The 42 pages on radiation therapy are rather technical, but fill a distinct void. It is not easy to find a short, concise explanation of radiation physics and the technical aspects of treatment. Dr. Perez includes a detailed discussion of the effects of x-ray on normal tissues, which is particularly appropriate in these days of increased survival into adult life. The pediatrician must now discuss with parents the long-term effects of treatment on health, growth, and reproduction. The discussion of cellular kinetics in relationship to chemotherapy is a good summary, Although this information is available elsewhere, it should be readily on hand for the pediatrician considering the pros and cons of chemotherapy. Two important chapters are devoted to supportive care divided
i n t o physiologic and psychological measures. As their introduction states, improved techniques in the treatment of infection and bleeding have probably contributed as much to increased survival as have the advances in chemotherapy. Dr. Lane presents a balanced discussion on the emotional support of the child and his family, mentioning the all too often forgotten factor, which is the expense of an illness such as cancer. Although he makes reference at the end to the team involved in care, more discussion of the roleof social worker, nurse, and chaplain would be appropriate. A chapter on the natural history of leukemia provides a wealth of valuable data. Dr. Fernbach is fortunate in having a large number of carefully monitored patients with leukemia and also one of few population-based tumor registries. This type of information provides an excellent basis for planning and evaluation of various treatment regimens. The references included in this and other chapters are copious and serve as a valuable source of additional information for the interested reader.
Audrey Evans, M.D. Children "s Hospital of Philadelphia Philadelphia, Pa. 19146
C y s t i n o s i s J o s e p h D. S c h u l m a n , M.D., W a s h i n g t o n , 1973, D e p a r t m e n t of H e a l t h , E d u c a t i o n , a n d W e l f a r e Publication, 258 pages. $3.85. The book is a compilation of contributions from several physicians and scientists on various aspects of cytinosis, including comprehensive reviews of the literature on historical, clinical, and biochemical aspects of the disease as well as some results not previously mentioned in the literature. The historical review is short and interestingly written. T h e clinical description is quite useful and gives a brief discussion of the two more recently described variants--benign cystinosis and late onset cystinosis. The section on diagnostic techniques is quite useful. The ophthalmologic findings are treated in detail and represent information not generally appreciated. The pathologic review is comprehensive and the interesting observation is noted that in renal tissue there is evidence suggesting that cytoplasmic cystine stores may not always be in membrane-lined structures i.e., nonlysosomal. The discussion of the complex pattern of proteinuria in cystinosis and the pattern of change during the course of the illness was interesting. The effect of the tubular damage of cystinosis on immunoglobulin L-chain metabolism is discussed. The various aspects and facets of cystine and sulfer metabolism as they relate to the disease are quite well covered and include the evidence for including cystinosis among the lysosomal dis'eases. The review of the enzymologic studies that have been performed on cystinosis cells was somewhat truncated but the in vitro transport data are adequately covered. The section on
Vol. 84, No. Zpp. 297-300
298
The book shelf
The Journal of Pedtatric~ February 1974
treatment reviews the disappointments of the different modalities tried. Renal transplantation was essentially supported as a viable consideration with the judgment that the problems and results are comparable to renal transplantation in noncystinotic individuals. The book is a well-written and up-to-date summary of the current knowledge in the field. It should be of interest to human biochemical geneticists, those interested in sulfur metabolism, and the many pediatricians who are caring for patients with cystinosis. In general it is fascinating to note how much information has been generated about this disease in such a relatively short period of time. As good as the book is, its most unique feature is the price. J. B. Sidbury, Jr., M.D. Department of Pediatrics Duke University Medical School Durham, N. C. 27710
New books received The CIBA collection of medical illustrations. Vol. 6. Kidneys, ureters, and urinary bladder Prepared by Frank H. Netter, M.D., edited by Robert K. Shapter, M.D., Summit, N. J.. 1973, CIBA Pharmaceutical Company, 312 pages. No price listed. Self-discovery through serf-expression. Use of art in psychotherapy with children and adults Mala Betensky, Springfield, I11., 1973, Charles C Thomas, Publisher, 399 pages. $13.95. Textbook of pediatric nursing, ed. 4 Dorothy R. Marlow, R. N., Philadelphia, 1973, W. B. Saunders Company, 776 pages. $10.75. Pathobiology of development--or ontogeny revisited Edited by E. V. D. Perrin, M.D., and Milton J. Finegold, M.D., Baltimore, 1973, The Williams & Wilkins Company, 151 pages. $19.75. All about Jimmy and his friend. An ostomy coloring book Carol Norris, Los Angeles, 1973, United Ostomy Association, Inc., 28 pages. 75r The effects of hospitalization" on children. Models for their care Edited by Evelyn K. Oremland, M. S. W., and Jerome D. Oremland, M.D., Springfield , Ill., 1973, Charles C Thomas, Publisher, 341 pages. $12.75. Nutrition for developing countries with special reference to the maize, cassava and millet areas of Africa Maurice H. King, Felicity M. A. King, David C. Morley, H. J. Leslie Burgess, and Ann P. Burgess,
New York, 1972, Oxford University Press. $5.95. Review of medical physiology, ed. 6 W,F. Ganong, M.D., Los Altos, Calif., 1973, Lange Medical Publications, 577 pages. $9.00. Review of physiological chemistry, ed. 14 H. A. Harper, Los Altos, Calif., 1973, Lange Medical Publications, 545 pages. $8.50. Practical pediatric electrocardiography Arthur J. Moss, M.D., and George C. Emmanouilides, M.D., Philadelphia, 1973, J. B. Lippincott Company, 148 pages. No price listed. Manual of skin diseases, ed. 3 Gordon C. Sauer, M.D., Philadelphia, 1973, J. B. Lippincott Company, 357 pages. $24.00. Retrolental fibroplasia and autisitic symptomatology. An investigation into some relationships among nenonatal, environmental, developmental and affective variables in blind prematures Joan B. Chase, Ed.D., New York, 1972, American Foundation for the Blind, Inc., 215 pages. No price listed. Sickle cell hemoglobin. Molecule to man Makio Murayama, Ph.D,, and Robert N. Nalbandian, M.D., Boston, 1973, Little, Brown & Company, 198 pages. $16.50. Square pages, round holes. The learning disabled child in the classroom and at home Harold B. Levy, M.D., Boston, 1973, Little, Brown & Company, 250 pages. $7.50. Child abuse in Nova Scotia. A research project about battered and maternally deprived children F. Murray Fraser, John P. Anderson, and Kevin Burns, Halifax, Nova Scotia, 1973, Dalhousie University, 295 pages. No price listed. Disease in infancy and childhood, ed. 7 Ross G. Mitchell, M.D., Baltimore, 1973, The Williams & Wilkins Company, 426 pages. $20.50. A bibliography of Chinese sources on medicine and public health in the People's Republic of China: 1960-1970 Department of Health, Education and Welfare publication No. (NIH) 73-439, Washington, 1973, United States Government Printing Office, 486 pages. $5.55. The prenatal diagnosis of hereditary disorders Aubrey Milunsky, Springfield, Ill., 1973, Charles C Thomas, Publisher, 253 pages. $11.75. Atherogenesis: Initiating factors Edited by Ruth Porter and Julie Knight, CIBA Foundation Symposium 12 (new series); New York, 1973, American Elsevier Publishing Company, Inc., 288 pages. No price listed.
L. Emmettnolt, Jr.,
Editor
Book reviews C l i n i c a l p e d i a t r i c o n t o l o g y W a t a r u W. Sutow, T e r e s a J. Vietti, a n d D o n a l d J. F e r n b a c h , editors, St. Louis, 1973, T h e C. V. M o s b y C o m p a n y , 602 pages. $35.00. This is a well-written and much needed textbook on the treatment of cancer in children. The editors have compiled the text from thirty-three authors, the majority of whom are members of the Pediatric Division of the Southwest Cancer Chemotherapy Study Group. With few exceptions, they are pediatricians, and the multiplicity of authors provides a balanced point of view regarding the merits of various treatment regimens. Most of the chapters are clearly written and include the background and rationale for the treatment suggested. Even though the therapy of cancer is a rapidly changing field, much of the content is basic information regarding the disease and will remain relevant in the years to come. The volume should be valuable to a wide range of readers. The general pediatrician will find it a useful source of reference regarding current treatment and prognosis of a specific tumor. Paramedical personnel such as nurses and social workers interested in oncology would benefit from reading the general chapters on childhood cancer, etiology, and principles of psychological support. The medical student should be interested in the chapters devoted to etiology, cell kinetics, chemotherapy, and the natural history of leukemia; the pediatric oncologists would benefit from the chapters on surgical considerations and implications of radiation therapy. The volume comprises 28 chapters; the first eight deal with general aspects of pediatric cancer and its treatment and the rest are devoted to tumors arising in specific sites. No attempt is made to include extensive discussions of histopathology and radiology, except as they influence treatment. The authors major objective is to re-emphasize the need for collaboration between the therapeutic disciplines and highlight the current progress in each area. In a brief review, it is not possible to comment on the various chapters. The discussion of etiology is clearly written and should be of interest to pediatricians as a whole and other physicians involved with cancer, since Dr. Miller details those clues which currently exist. The 42 pages on radiation therapy are rather technical, but fill a distinct void. It is not easy to find a short, concise explanation of radiation physics and the technical aspects of treatment. Dr. Perez includes a detailed discussion of the effects of x-ray on normal tissues, which is particularly appropriate in these days of increased survival into adult life. The pediatrician must now discuss with parents the long-term effects of treatment on health, growth, and reproduction. The discussion of cellular kinetics in relationship to chemotherapy is a good summary, Although this information is available elsewhere, it should be readily on hand for the pediatrician considering the pros and cons of chemotherapy. Two important chapters are devoted to supportive care divided
i n t o physiologic and psychological measures. As their introduction states, improved techniques in the treatment of infection and bleeding have probably contributed as much to increased survival as have the advances in chemotherapy. Dr. Lane presents a balanced discussion on the emotional support of the child and his family, mentioning the all too often forgotten factor, which is the expense of an illness such as cancer. Although he makes reference at the end to the team involved in care, more discussion of the roleof social worker, nurse, and chaplain would be appropriate. A chapter on the natural history of leukemia provides a wealth of valuable data. Dr. Fernbach is fortunate in having a large number of carefully monitored patients with leukemia and also one of few population-based tumor registries. This type of information provides an excellent basis for planning and evaluation of various treatment regimens. The references included in this and other chapters are copious and serve as a valuable source of additional information for the interested reader.
Audrey Evans, M.D. Children "s Hospital of Philadelphia Philadelphia, Pa. 19146
C y s t i n o s i s J o s e p h D. S c h u l m a n , M.D., W a s h i n g t o n , 1973, D e p a r t m e n t of H e a l t h , E d u c a t i o n , a n d W e l f a r e Publication, 258 pages. $3.85. The book is a compilation of contributions from several physicians and scientists on various aspects of cytinosis, including comprehensive reviews of the literature on historical, clinical, and biochemical aspects of the disease as well as some results not previously mentioned in the literature. The historical review is short and interestingly written. T h e clinical description is quite useful and gives a brief discussion of the two more recently described variants--benign cystinosis and late onset cystinosis. The section on diagnostic techniques is quite useful. The ophthalmologic findings are treated in detail and represent information not generally appreciated. The pathologic review is comprehensive and the interesting observation is noted that in renal tissue there is evidence suggesting that cytoplasmic cystine stores may not always be in membrane-lined structures i.e., nonlysosomal. The discussion of the complex pattern of proteinuria in cystinosis and the pattern of change during the course of the illness was interesting. The effect of the tubular damage of cystinosis on immunoglobulin L-chain metabolism is discussed. The various aspects and facets of cystine and sulfer metabolism as they relate to the disease are quite well covered and include the evidence for including cystinosis among the lysosomal dis'eases. The review of the enzymologic studies that have been performed on cystinosis cells was somewhat truncated but the in vitro transport data are adequately covered. The section on
Vol. 84, No. Zpp. 297-300
298
The book shelf
The Journal of Pedtatric~ February 1974
treatment reviews the disappointments of the different modalities tried. Renal transplantation was essentially supported as a viable consideration with the judgment that the problems and results are comparable to renal transplantation in noncystinotic individuals. The book is a well-written and up-to-date summary of the current knowledge in the field. It should be of interest to human biochemical geneticists, those interested in sulfur metabolism, and the many pediatricians who are caring for patients with cystinosis. In general it is fascinating to note how much information has been generated about this disease in such a relatively short period of time. As good as the book is, its most unique feature is the price. J. B. Sidbury, Jr., M.D. Department of Pediatrics Duke University Medical School Durham, N. C. 27710
New books received The CIBA collection of medical illustrations. Vol. 6. Kidneys, ureters, and urinary bladder Prepared by Frank H. Netter, M.D., edited by Robert K. Shapter, M.D., Summit, N. J.. 1973, CIBA Pharmaceutical Company, 312 pages. No price listed. Self-discovery through serf-expression. Use of art in psychotherapy with children and adults Mala Betensky, Springfield, I11., 1973, Charles C Thomas, Publisher, 399 pages. $13.95. Textbook of pediatric nursing, ed. 4 Dorothy R. Marlow, R. N., Philadelphia, 1973, W. B. Saunders Company, 776 pages. $10.75. Pathobiology of development--or ontogeny revisited Edited by E. V. D. Perrin, M.D., and Milton J. Finegold, M.D., Baltimore, 1973, The Williams & Wilkins Company, 151 pages. $19.75. All about Jimmy and his friend. An ostomy coloring book Carol Norris, Los Angeles, 1973, United Ostomy Association, Inc., 28 pages. 75r The effects of hospitalization" on children. Models for their care Edited by Evelyn K. Oremland, M. S. W., and Jerome D. Oremland, M.D., Springfield , Ill., 1973, Charles C Thomas, Publisher, 341 pages. $12.75. Nutrition for developing countries with special reference to the maize, cassava and millet areas of Africa Maurice H. King, Felicity M. A. King, David C. Morley, H. J. Leslie Burgess, and Ann P. Burgess,
New York, 1972, Oxford University Press. $5.95. Review of medical physiology, ed. 6 W,F. Ganong, M.D., Los Altos, Calif., 1973, Lange Medical Publications, 577 pages. $9.00. Review of physiological chemistry, ed. 14 H. A. Harper, Los Altos, Calif., 1973, Lange Medical Publications, 545 pages. $8.50. Practical pediatric electrocardiography Arthur J. Moss, M.D., and George C. Emmanouilides, M.D., Philadelphia, 1973, J. B. Lippincott Company, 148 pages. No price listed. Manual of skin diseases, ed. 3 Gordon C. Sauer, M.D., Philadelphia, 1973, J. B. Lippincott Company, 357 pages. $24.00. Retrolental fibroplasia and autisitic symptomatology. An investigation into some relationships among nenonatal, environmental, developmental and affective variables in blind prematures Joan B. Chase, Ed.D., New York, 1972, American Foundation for the Blind, Inc., 215 pages. No price listed. Sickle cell hemoglobin. Molecule to man Makio Murayama, Ph.D,, and Robert N. Nalbandian, M.D., Boston, 1973, Little, Brown & Company, 198 pages. $16.50. Square pages, round holes. The learning disabled child in the classroom and at home Harold B. Levy, M.D., Boston, 1973, Little, Brown & Company, 250 pages. $7.50. Child abuse in Nova Scotia. A research project about battered and maternally deprived children F. Murray Fraser, John P. Anderson, and Kevin Burns, Halifax, Nova Scotia, 1973, Dalhousie University, 295 pages. No price listed. Disease in infancy and childhood, ed. 7 Ross G. Mitchell, M.D., Baltimore, 1973, The Williams & Wilkins Company, 426 pages. $20.50. A bibliography of Chinese sources on medicine and public health in the People's Republic of China: 1960-1970 Department of Health, Education and Welfare publication No. (NIH) 73-439, Washington, 1973, United States Government Printing Office, 486 pages. $5.55. The prenatal diagnosis of hereditary disorders Aubrey Milunsky, Springfield, Ill., 1973, Charles C Thomas, Publisher, 253 pages. $11.75. Atherogenesis: Initiating factors Edited by Ruth Porter and Julie Knight, CIBA Foundation Symposium 12 (new series); New York, 1973, American Elsevier Publishing Company, Inc., 288 pages. No price listed.