Kernicterus Springer/ Humana Press 2011 9781441965547 MedicalScienceBooks Review

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Kernicterus
Author: David W. McCandless
Pages: 286 Hard Cover
ISBN 13: 978-1-4419-6554-7
Humana Press 2011
List Price: $189

Medical Science Books .com Medical Book Review:

     Hyperbilirubinemia and the prevention of development of neurological pathology and/ or death due to kernicterus in newborns is a significant concern for physicians and allied healthcare workers worldwide. Although a number of clinical publications dealing with neonatal medicine and pediatrics cover the essential information regarding patient management, often little more than phototherapy, none to date can claim the comprehensive survey of all things “bilirubin” as Kernicterus written by David W. McCandles. Even publications such as Emery and Rimoin’s Principles and Practice of Medical Genetics and The Metabolic and Molecular Bases of Inherited Disease, volumes which exemplify the combination of basic science and clinical practice in relation to metabolic disease, don’t give the reader as rich a perspective on defective bilirubin handling in the body as can be found in this title.
     Much of the additional perspective gained by reading Kernicterus derives from the intellectually stimulating manner in which it is written. For example, the chapter on the history of bilirubin can be included as a reading for a course dealing with the history of medicine. Due respect and credit is given to the pioneers of the description and treatment of hyperbilirubinemia and the reader can retrace all the major milestones of our current understanding of this disease. The author has done an outstanding job of incorporating the collective knowledge of an impressive number of journal articles written over the last two centuries since the first modern descriptions of jaundice and kernicterus were reported in the early 19^th century. The majority of articles are discussed succinctly and put into context within the chapters according to the major findings of the research. In fact a minor criticism of this style might be that too many individual articles are discussed rather than more general trends being discussed from the consolidation of several studies together. However, to the author’s credit, the individual discussion of articles flows quite nicely in most instances because he takes on the role of attempting to reconcile some of the discrepancies that arise for example with epidemiological analysis of disease prevalence in a subset of the population.
     Little information regarding bilirubin is left out of the discussion in Kernicterus which examines not only the biochemistry and physiology of this molecule but a thorough analysis of the major conditions leading to the development of kernicterus and of course the neuropathology and sequelae associated with untreated hyperbilirubinemia. Those readers with an interest in gene therapy techniques and other “hot” topics in the genetics field will particularly find the chapter on the Gunn rat model of kernicterus useful as few animal models of a human metabolic condition so closely reflect the gene level control of a physiological process. The fact that inroads to successful treatment in this model have been made using viral vector gene therapy is significant to researchers in a variety of fields.
     Finally, some medical textbooks are better than others in making us consider the global impact of certain diseases or conditions. Kernicterus is one of those titles which belong in a select class which highlight the difficulties, particularly in underdeveloped countries, of treating relatively amenable conditions. Many readers from the industrialized world will have their eyes opened by the prologue which details the full impact of kernicterus which is rarely seen in the modern medical center. However, as the author points out, current global immigration trends make it likely that significant numbers of cases of hyperbilirubinemia will be brought to the attention of physicians in western countries as ethnic populations with genetic propensities toward metabolic syndromes such as glucose-6 phosphate dehydrogenase deficiency (G6PDH) resettle in new areas.
     Kernicterus is a worthy addition to the clinical neuroscience literature as a treatise on a condition that preferentially attacks critical brain regions. It is also a worthy addition to the larger collection of titles pertaining to global health. As such, it should be read widely by clinicians and public health officers confronted by issues related to the early diagnosis, treatment and rehabilitation of individuals suffering from kernicterus.

Ratings (1-4 , 4 being the highest):

Organization of information: 3

Usefulness of book: 3.5

Suitable for intended audience: 3.5

Author’s objectives met: 4

Significant number of illustrations: 3

Quality of illustrations: 3

Description:

Kernicterus is an excellent example of a metabolic encephalopathy. It clearly produces a biochemical lesion early when bilirubin first enters the brain. If initial staining can be minimized or reversed, permanent damage can be avoided. This takes strict attention to detail, and a keen ability for timely and accurate diagnosis. The potential for improvement/reversal argues strongly for increased awareness by all health care workers.

Kernicterus will examine the history, biochemistry, physiology, neuropathology, etc., of bilirubin. New concepts of how bilirubin is toxic, and damages the acoustic system will be thoroughly examined. The promising new treatment possibility using gene therapy will be examined in detail. This therapy has been successfully used in the excellent animal model, the Gunn rat. Translation of these exciting results to newborn infants is well underway.

Table of Contents:

Prologue: World Health Concerns . . . . . . . . . . . . . . . . . . . . . 1

History of Bilirubin . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 11

Biochemistry and Physiology of Bilirubin . . . . . . . . . . . . . . . . . 19

Premature Birth . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 33

Erythroblastosis Fetalis . . . . . . . . . . . . . . . . . . . . . . . . . . . 43

The Gunn Rat Model . . . . . . . . . . . . . . . . . . . . . . . . . . . . 51

Crigler–Najjar Syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . 65

Neuropathology of Kernicterus . . . . . . . . . . . . . . . . . . . . . . . 81

Bilirubin and Energy Metabolism . . . . . . . . . . . . . . . . . . . . . 95

Jaundice and Other Biochemical Changes . . . . . . . . . . . . . . . . . 109

Breast Milk Jaundice . . . . . . . . . . . . . . . . . . . . . . . . . . . . 115

Jaundice in Malaria . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 121

Jaundice in Congenital Hypertrophic

Pyloric Stenosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 133

Phototherapy for Hyperbilirubinemia . . . . . . . . . . . . . . . . . . . 141

Non-phototherapy Treatment . . . . . . . . . . . . . . . . . . . . . . . . 153

Hyperbilirubinemia Revisited . . . . . . . . . . . . . . . . . . . . . . . . 161

Auditory Brainstem Response . . . . . . . . . . . . . . . . . . . . . . . 175

Progressive Familial Intrahepatic Cholestasis . . . . . . . . . . . . . . . 189

Kernicterus in Older Children and Adults . . . . . . . . . . . . . . . . . 203

Cerebral Palsy and Counseling . . . . . . . . . . . . . . . . . . . . . . . 209

Neurological Sequelae from Jaundice . . . . . . . . . . . . . . . . . . . 219

Neurobehavioral Teratology . . . . . . . . . . . . . . . . . . . . . . . . 227

xiii

xiv Contents

Gene Therapy for Hyperbilirubinemia . . . . . . . . . . . . . . . . . . . 235

Epilogue: Comments and Future Directions . . . . . . . . . . . . . . . . 245

Appendix – Sources . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 251

References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 253

Index . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 273