Pediatric Annals

EDITORIAL 

Liver Transplantation and Recent Advances in Hepatology

Milton I Levine, MD

Abstract

My father was the son of a Civil War veteraiv - a soldier in the New York Fifth Regiment. As a child 1 was told the strategies of the battles of Bull Run and Gettysburg, with my Dad pointing out on a large map the various positions of the Union and Confederate Armies. Some years later the grey cloud of the first World War spread across Europe. This was the first war in which tanks, poison gas, submarines and airplanes were used.

I remember vividly one day when my father, reading the newspaper, turned to me and said "Who ever would have thought that man would fight battles in the skies?" The paper had a picture of a dog-fight between German and English planes - the German, with a black cross on their wings, the English, with circular design and a black dot in the center.

"You knew," my father said quietly, "I'm sure that anything Man can think of Man can do. "

I have often thought of that statement which I didn't believe so many years ago, but which I almost believe today.

I've thought of it so many times as I followed the advances in science and especially in medicine.

I was taught in medical school and during my residencies that the body would always reject foreign material. However, that restriction has been greatly modified by the use of certain plastics and metals. And, as for the use of human and animal tissue transplants, Cyclosporin A has been found most effective as an immunosuppressant. More recently there is the promising and innovative technology of artificial organ transplantation.

Two articles in this issue of Pediatric Annals, the one on liver transplants and the other on biliary atresia, brought my father's statement back to mind.

Our problem at present is in organ procurement, but I am sure that in the not-too-distant future scientists will develop a safe means of interspecies transplantation for I have come to believe that, with very few exceptions, "anything man can think of man can do."

This is the second issue of the magazine devoted to Hepatology, and as with the first is under the Guest Editorship of Dr. Mervin Silverberg, Jack Hausman Professor of Pediatrics, Cornell University Medical College, and Director of Pediatrics of the North Shore Hospital, Manhasset, Long Island.

The first contribution to the symposium is on "Liver Transplantation" and has been written by Dr. David H. Van Thiel of the Division of Gastroenterology at the University of Pittsburgh School of Medicine.

As is generally recognized, the unit at the University of Pittsburgh is the foremost in this recent field. Dr. Van Thiel writes on the basis of 5 years of experience.

His report should not only prove informative but exciting for pediatricians for he notes how much better the prognosis is for children than for adults who undergo transplants.

Step by step this article covers all aspects of the subject in line with present-day knowledge. What are the criteria for the selection for liver transplantation, what is the preoperative evaluation and management? The postoperative problems and their treatment are covered as they occur during specific periods following surgery - those that occur during the first 24 hours, those in the next 24 hours, those in 2 to 12 days, and those that occur 13 days and beyond.

Dr. Van Thiel states that most programs treating children can achieve a 75% to 80% survival rate after 1 year, one even reporting a survival at 1 year of 90%.

The second article discusses the present knowledge on biliary atresia and the results of…

My father was the son of a Civil War veteraiv - a soldier in the New York Fifth Regiment. As a child 1 was told the strategies of the battles of Bull Run and Gettysburg, with my Dad pointing out on a large map the various positions of the Union and Confederate Armies. Some years later the grey cloud of the first World War spread across Europe. This was the first war in which tanks, poison gas, submarines and airplanes were used.

I remember vividly one day when my father, reading the newspaper, turned to me and said "Who ever would have thought that man would fight battles in the skies?" The paper had a picture of a dog-fight between German and English planes - the German, with a black cross on their wings, the English, with circular design and a black dot in the center.

"You knew," my father said quietly, "I'm sure that anything Man can think of Man can do. "

I have often thought of that statement which I didn't believe so many years ago, but which I almost believe today.

I've thought of it so many times as I followed the advances in science and especially in medicine.

I was taught in medical school and during my residencies that the body would always reject foreign material. However, that restriction has been greatly modified by the use of certain plastics and metals. And, as for the use of human and animal tissue transplants, Cyclosporin A has been found most effective as an immunosuppressant. More recently there is the promising and innovative technology of artificial organ transplantation.

Two articles in this issue of Pediatric Annals, the one on liver transplants and the other on biliary atresia, brought my father's statement back to mind.

Our problem at present is in organ procurement, but I am sure that in the not-too-distant future scientists will develop a safe means of interspecies transplantation for I have come to believe that, with very few exceptions, "anything man can think of man can do."

This is the second issue of the magazine devoted to Hepatology, and as with the first is under the Guest Editorship of Dr. Mervin Silverberg, Jack Hausman Professor of Pediatrics, Cornell University Medical College, and Director of Pediatrics of the North Shore Hospital, Manhasset, Long Island.

The first contribution to the symposium is on "Liver Transplantation" and has been written by Dr. David H. Van Thiel of the Division of Gastroenterology at the University of Pittsburgh School of Medicine.

As is generally recognized, the unit at the University of Pittsburgh is the foremost in this recent field. Dr. Van Thiel writes on the basis of 5 years of experience.

His report should not only prove informative but exciting for pediatricians for he notes how much better the prognosis is for children than for adults who undergo transplants.

Step by step this article covers all aspects of the subject in line with present-day knowledge. What are the criteria for the selection for liver transplantation, what is the preoperative evaluation and management? The postoperative problems and their treatment are covered as they occur during specific periods following surgery - those that occur during the first 24 hours, those in the next 24 hours, those in 2 to 12 days, and those that occur 13 days and beyond.

Dr. Van Thiel states that most programs treating children can achieve a 75% to 80% survival rate after 1 year, one even reporting a survival at 1 year of 90%.

The second article discusses the present knowledge on biliary atresia and the results of corrective surgery. It is authored by Dr. R. Peter Altman, Professor of Surgery and Pediatrics, and Director of Pediatric Surgery; and Dr. Joseph Levy, Assistant Professor of Pediatrics, and Director of the Clinical GI Service. Both are from the Columbia University College of Physicians and Surgeons, and from Babies Hospital, Columbia- Presbyterian Medical Center, New York.

The authors remind us that in an article by Dr. Altman 8 years ago he reported on a corrective procedure from Japan, the Kasai procedure, with early reports from that country of a "cure" for biliary atresia. Dr. Altman at that time stated that "guarded optimism is justified." In this present review the authors report on the surgical treatment of 100 patients with biliary atresia using this method of portoenterostomy. Unfortunately, the prediction of "cure" has not been routinely achieved. Cholangitis occurs in over 50% of the infants.

The article covers the three most important aspects in the postoperative management; maintenance of the nutritional state, stimulation of bile flow by pharmacologic means, and the prevention of cholangitis.

It is interesting to note that of the 100 patients with surgical correction, extended bile drainage was accomplished in 81. This is another condition where organ transplantation may be necessary.

The third paper in this symposium has been contributed by Doctors Donald A. Novak and William F. Balistreri of the Children's Hospital Medical Center in Cincinnati, Ohio. The authors discuss management of the child with chronic cholestasis.

This carefully presented study covers the numerous consequences of the lack of adequate bile flow and the optimal management of each of these conditions. Primarily the authors discuss the prurit is and xanthomata; the malnutrition; and the signs of hepatic failure including variceal hemorrhages and ascites. If treatment is started early and aggressively with proper diet and pharmacologic intervention, adequate growth and nutrition can be maintained in many children. In severe cases liver transplantation is necessary.

The next article deals with another difficult pediatric problem - portal hypertension. It is presented by Dr. Peter F. Whitington of the University of Chicago Pritzker School of Medicine in Chicago, Illinois.

The discussion opens with the diagnostic features and the methods used for measuring the portal pressure. The basic causes of the condition are reviewed, and then Dr. Whitington describes the various methods of diagnosis including liver biopsy and the recent techniques of imaging the portal system. An interesting section of this paper reports on prehepatic or extrahepatic portal hypertensions as compared to the intrahepatic type. The author notes that patients with signs of portal hypertension will probably be found to have the extrahepatic disease. It is reported that one of the most common causes of portal hypertension is one rarely seen in our part of the world - schistosomiasis.

Emphasis is placed on the clinical management which must be thorough since it dictates the therapy. Much of the prognosis depends on the adequacy of treatment. Patients with extrahepatic portal hypertension may be expected to survive indefinitely with little morbidity.

The next contribution describes the "Management of Metabolic Diseases of the Liver." It is written by Dr. Alex Mowat of the Kings College Hospital Medical Center, University of London, London, England.

This is a superb review of a multi-faceted subject, for, as the author states, there are already over 5,000 genetically determined metabolic pathways described in liver tissues. Dr. Mowat concentrates on those metabolic conditions where the primary feature is hepatic and where effective treatment is available. He emphasizes the need for the earliest possible diagnosis not only to prevent permanent damage to such organs as the brain and liver but, when no treatment is available, to minimize complications. The importance of genetic counseling is also discussed. This review brings to the reader the latest information on methods of diagnosis and treatment and the latest knowledge on prognosis. It is well worth reading carefully.

The final paper is on the "Management of Reye's Syndrome" and is contributed by Dr. John C. Partin, Professor and Chairman of the Department of Pediatrics at the State University of New York at Stony Brook, New York.

All pediatricians today are well aware of this condition which was first described in 1963. Originally it was noted that it almost always followed such conditions as influenza and chicken pox. The actual cause was unknown but treatment by glucose infusions or transfusions were advised. Without adequate treatment 75% of these children the within a few days. With treatment the mortality is still about 40%.

In 1982 the FDA reported the relationship of aspirin intake to the onset of this condition which involves the liver, and often leads to severe encephalopathy. Dr. Partin, who has studied this disease for over 10 years and published a number of papers on it, reviews the present knowledge not only on prevention but on modem therapy as well. He emphasizes the importance of early diagnosis and the prompt treatment with intravenous glucose. Once a child has become comatose there is no proven treatment - intensive care is a necessity.

10.3928/0090-4481-19850701-04

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