Book Review

Infants and Children

Liver Disease in Children

N Engl J Med 1994; 331:410-411August 11, 1994

Article

Liver Disease in Children
Edited by Frederick J. Suchy. 1013 pp., illustrated. St. Louis, Mosby, 1994. $149. ISBN: 1-55664-377-2

Hepatology has enjoyed remarkable advances in the past 10 to 15 years because of tremendous progress in diagnostic capabilities, knowledge of the molecular basis of liver disease, and medical and surgical therapies. Somewhat less obvious but equally profound progress has been made against the hepatobiliary disorders of childhood. In addition, attempts continue to adapt and extrapolate technologic advances in treating adults to the special circumstances of infants and children. Thus, pediatric hepatology has emerged as an established subspecialty. The book under review is a compendium of the knowledge base and the ongoing investigations in this new subspecialty.

The stated goal of Liver Disease in Children is to provide a framework for understanding the pathophysiology, manifestations, and management of pediatric hepatobiliary disorders. The intended audience is a somewhat diverse group of clinicians, such as pediatricians, pediatric gastroenterologists, and pediatric and transplantation surgeons. There is no attempt to recapitulate all of the material in internal-medicine textbooks on hepatology that may apply to children, but rather an effort to build on that foundation a discussion of aspects of hepatology that are pertinent or unique to pediatrics.

The book has four sections. The first, “Structure and Function of the Developing Liver,” covers in great detail not only the traditional topic of the embryology of the liver and more recent concepts of the biliary tree but also hepatic growth and regeneration and the development of hepatic biochemical functions. This section alone is a valuable resource because it contains a wealth of information in a compact and readable format.

The second section, “Mechanisms and Manifestations of Liver Disease,” represents a transition between the basic physiology already presented and the clinical material to come. It covers the pathophysiology of cholestasis, as well as mechanisms underlying the generic conditions, giant-cell hepatitis, cirrhosis, and portal hypertension. It also has a comprehensive and valuable discussion of hepatic failure in childhood. Section III, “Evaluation of Liver Function and Injury,” includes various aspects of laboratory, radiographic, and procedure-based diagnostic evaluation. The chapter “Laboratory Diagnosis of Inborn Errors of Metabolism” makes a complex and rather intimidating topic more user-friendly by incorporating clear and simple tables.

Section IV, “Clinical Aspects and Management of Specific Disorders,” makes up the bulk of the text. Some discussions, such as those of acute and chronic viral hepatitis and drug-induced liver disease, rely heavily on information derived from studies of adults, yet the authors consistently emphasize pediatric issues and concerns. Others present disorders seen primarily in infants and children, such as inborn errors in amino acid, fatty-acid, and bile-acid metabolic pathways and extrahepatic biliary atresia. Still other discussions include entities that are usually seen in children but may occasionally be diagnosed and treated in older patients, such as cystic disease of the liver, alpha₁-antitrypsin deficiency, and the hepatobiliary abnormalities of cystic fibrosis. The authors cover pathogenetic mechanisms, including the results of molecular biologic studies and ultrastructural correlation, whenever possible.

Because of this somewhat artificial organizational framework, the reader often encounters repetition, such as duplicative discussions of bilirubin metabolism and hepatic drug metabolism. At other times, to get the big picture one must jump from section to section: the reader will learn about the embryology of the biliary tree in section I, cholangiopathies in section II, and the specific disorders resulting from disturbances in these processes in section IV. Yet this overlap and disruption seem a fairly small price to pay for a book that strives for so much detail and thoroughness. Only rarely is the reader shortchanged by this inconvenience. For example, after reading “Immune Mechanisms in Pediatric Liver Disease,” one finds only a brief and rather cursory discussion of autoimmune chronic active hepatitis in the chapter entitled “Chronic Hepatitis,” which focuses instead on mechanisms of fibrogenesis.

In general, the writing styles are clear, the discussions are exhaustive, and new and controversial topics are included. The bibliographies are extensive, and many authors refer to recent reviews and other sources. The editor's goal of presenting aspects pertinent to children has been achieved, since the authors consistently highlight pediatric issues and concerns.

To be able to read a chapter dedicated to a specific entity or illness that would usually be mentioned in a short paragraph in any other hepatology textbook was indeed a welcome pleasure. This book will be a valuable resource for its intended readership, as well as internal-medicine hepatologists who want to expand their understanding of the development and pathophysiologic repertoire of the liver.

Maureen M. Jonas, M.D.
Children's Hospital Medical Center, Boston, MA 02115