The Shwachman Award of the North American Society for Pediatric Gastroenterology and Nutrition, 1999: Presentation : Journal of Pediatric Gastroenterology and Nutrition

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The Shwachman Award of the North American Society for Pediatric Gastroenterology and Nutrition, 1999: Presentation

Cohen, Mitchell B.

Journal of Pediatric Gastroenterology and Nutrition : April 2000 - Volume 30 - Issue 4 - p 355-358
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I am privileged to be able to share with you some stories, reflections, and insights on this year's recipient of the NASPGN Shwachman Award, William F. Balistreri. Bill has been my teacher and friend for the past 16 years. Many of you know him, as I do, as an extraordinary individual with an outstanding ability to teach, to advocate, and to inspire. Bill has an uncanny capacity to stay abreast of the medical literature, to recall all that he has read, and to conceptualize the principles of physiology and disease, so as to make them easily understandable and relevant to clinical medicine. He is completely devoted to advancing the field of pediatric gastroenterology and hepatology. His devotion is both to making the care we give better by advancing new knowledge and to making the discipline better by beginning new endeavors and by encouraging new disciples. As I will outline, Bill has done just about everything to advance our subspecialty. He always steps forward when asked to help. At the same time he has remained incredibly humble and true to his purpose: the advancement of the interests of our patients and the health of children.

How did Bill get to this honored moment? Bill was born on June 24, 1944, in Geneva, New York, located in the Finger Lakes region. His parents were of extremely modest means. In fact, because of his ethnic background and economic status, Bill often felt discriminated against. In response to this, Bill has always judged others by what they have accomplished rather than by their heritage. Despite his well-developed academic and athletic competitive skills, which I will also address, fair play is more important to Bill than is winning.

In high school, Bill was senior class president. He was also interested in sports and played baseball, basketball, and football. Unfortunately, he separated his shoulder playing football, and this injury ended his playing career. However, Bill was and remains an intellectual and enterprising jock. Since he continued to travel with the team, he convinced the editor of the local newspaper to allow him to write stories about the games. Bill was to be paid 10 cents per column inch for everything that was published. Coming from a family in which money was valued, he learned to write long and detailed stories. This successful endeavor continued through basketball season. Although Bill's family was not planning on his going to college, his sports writing led to his attending St. Bonaventure University on an endowed scholarship for journalism. This vignette provides an important paradigm for Bill's subsequent academic life. In transitioning from a player to a sports reporter, he was successful because he knew his subject well, was highly enthusiastic, had the respect of his teammates and colleagues, and was able to effectively communicate to a wide audience.

Despite his obvious talent, after a year in journalism school Bill made a change in his career path. He reflects that he was looking for a profession with more collegiality and fewer deadlines (sic). He finished college at the State University of New York (SUNY) at Buffalo where he subsequently attended medical school.

In his last year of medical school, Bill made a driving tour to investigate pediatric internships. One evening, he made an unplanned stop in Cincinnati where he ran into the chief resident at Children's Hospital Medical Center (CHMC) who talked with him in the cafeteria and gave him a recruiting tour. Bill liked CHMC and felt that it was right for him. In addition to showing how serendipity played a role in Bill's long-term career in Cincinnati, this story is also quite telling about Bill in other ways. He is keenly analytical; however, he also is remarkably intuitive. He has always had an ability to think with his heart as well as his head.

Even as a pediatric resident, Bill assumed the role of clinical investigator. As a second year resident, he cared for a patient with enigmatic and persistent hypoglycemia. He had read about the differential work-up of such patients in Cornblath and Schwarz's metabolic disease textbook. Among other considerations was the entity of leucine-sensitive hypoglycemia. He therefore proceeded with the evaluation including giving a leucine challenge to this infant on the pediatric ward! William K. Schubert, the patient's attending physician, looked on from a distance and shook his head. Bill Schubert was one of Balistreri's earliest role models and has been one of his most enduring mentors. Schubert also had outstanding instincts and diagnostic skills, and he recognized those same qualities in Bill Balistreri. Schubert is a man of few words. By the third day of evaluation of this patient with hypoglycemia, he gave Bill a pat on the back and acknowledged that he was “doing a good job.” Later in the year, Bill decided that he, “liked thinking about these sorts of patients,” so he approached Dr. Schubert about a fellowship in GI. Schubert's response was memorable, “What's that? We don't have a fellowship in GI.” Bill recognized that in Schubert-speak that wasn't a no, merely a challenge.

After convincing Schubert, Bill then convinced the American Board of Pediatrics to let him do his third year of residency as a GI fellow. During this year, Bill cared for a patient with intractable diarrhea. After an extensive evaluation, Bill suspected a defect in the enterohepatic circulation of bile. Eventually, he determined that this patient had primary bile acid malabsorption (1). Alan Hofmann at the Mayo Clinic had written about the use of labeled bile acids for tracer studies, so Bill called him to discuss the investigative approach in this novel clinical situation. This led to a collaboration with Hofmann to validate the use of deuterium-labeled bile acids for measurement of bile acid kinetics in adults (2) and a 1-year research fellowship at Mayo to learn more about bile acids. Later, Jim Heubi was to further study and characterize the physiologic defect in this patient (3,4).

The bile acid story represents another important lesson that Bill has frequently retaught me. It is important to know the literature, to be rigorous when approaching novel paradigms, and to collaborate with others. Bill has always facilitated these new collaborations by guiding and encouraging his trainees and by inspiring them to bring the bedside to the bench and the bench back to the bedside.

In addition to the first description of a congenital defect in ileal bile acid transport (1), Bill has also made a number of other sentinel observations. These include the first description of reductase deficiency, a specific inborn error of bile acid metabolism (5) and the use of bile acid replacement therapy as treatment for this otherwise fatal disease (6,7). He was among the first to describe the use of a specific bile acid as a therapeutic agent in children with intrahepatic cholestasis (8). This is now an accepted form of therapy. Bill helped to unravel the E-Ferol mystery, a cause of toxic liver failure in newborns (9,10). In addition, he helped to elucidate the concept of physiologic cholestasis (11,12) and to pioneer the use of serum bile acid measurements in the diagnosis of liver disease (14).

The Berry Plan had enabled Bill's deferment until he had completed his training, but after a year at Mayo, he entered the military. Alan Hofmann had arranged for Bill to work with Don Castell in Philadelphia while serving in the Navy. Unfortunately, Bill's commander didn't like this idea and required him to be on site from 8:30 A.M. to 3:30 P.M. Instead of lamenting his fate, Bill again took the initiative, and after his duty hours, he went to the Children's Hospital of Philadelphia (CHOP) to sit in on Dr. Phil Holtzapple's GI clinics. In addition, Bill and several other outstanding pediatric generalists who were in the Navy, including Basil Zitelli, Ed Charney, and Warren Sweberg, developed an academic teaching program. This program was a significant milestone in the development of the core teaching style Bill subsequently infused into the Fellowship Program in Cincinnati. It was a pediatric teaching program—nothing in pediatrics was outside the purview of discourse; it was evidenced based—a practice instituted before the term had been coined—and it was case oriented—again, a prescient concept.

While he served in the Navy, several papers Bill had written with Hofmann were published (2,14). Jean Cortner, then director of pediatrics at CHOP, gave one of these papers to Holtzapple and suggested that he try to recruit Bill to the developing GI division. Cortner asked, “Do you know where to find Balistreri?” Holtzapple, replied, “Yes, he is in the Navy stationed in Philadelphia, and he comes to my clinic!” Bill joined the faculty at the University of Pennsylvania where, in addition to Holtzapple, he interacted with the Internal Medicine group including Roger Soloway, Sid Cohen, Donald Ostrow, Bill Snape, and Otakar Koldovsky (a former Shwachman awardee). However, in less than 2 years, Holtzapple left, and Bill was offered the opportunity to run the now one-person division at CHOP. Despite much encouragement, Bill felt he was too junior to do this, and in 1978, he accepted an offer to return to Cincinnati where Bill Schubert and John Partin already had established a much larger two-person division. By 1980, Bill Schubert had become Chair of Pediatrics in Cincinnati, and John Partin had become Chair of Pediatrics at SUNY Stony Brook. Bill was again offered the opportunity to be Director of Pediatric Gastroenterology and Nutrition; this time he accepted and stayed in Cincinnati. Jim Heubi and Mike Farrell, who had been fellows under Schubert, were his partners. Fred Suchy who had been chief resident in 1978 and subsequently a fellow in gastroenterology, was Bill's first faculty hire. A long list of highly successful trainees came to Cincinnati over the next 20 years (Table 1). Bill has always spoken of these trainees as his family.

T1-1
TABLE 1:
William F. Balistreri's trainees at Children's Hospital Medical Center, Cincinnati, Ohio, U.S.A.

In 1984, Bill was named the Dorothy M. M. Kersten Professor of Pediatrics. He probably also holds a named Chair on Delta Airlines as well, since he has been invited to give more than 350 lectures throughout the world in the past 25 years. In addition, he has authored approximately 350 manuscripts or book chapters and has received dozens of honors and awards. Among the most important to Bill, like the Shwachman, are those in memory of important early leaders in pediatric GI—the Andrew Sass-Kortsak Award of the Canadian Liver Foundation/Canadian Association for the Study of Liver, and the Murray Davidson Award of the American Academy of Pediatrics. Bill was especially pleased to win the Shwachman award. I find it notable that he has also been recognized by awards from the four medical institutions to which he has so significantly contributed, the SUNY School of Medicine at Buffalo, the University of Cincinnati School of Medicine, CHOP, and the Mayo School of Medicine.

Bill has been an innovator in pediatric gastroenterology and hepatology. In 1980, he organized the first midwest pediatric gastroenterology club meeting to help promote collegiality and discussion among the pioneers of pediatric GI in the hinterlands. In 1982, he served as cochair of the first Ross–NASPGN Conference on pediatric GI and helped to formulate the first Mead Johnson–NASPGN conference in 1983. In 1984, he cochaired the first Mead Johnson Neonatal GI Nutrition Symposium and has continued to organize this annual conference. Bill has regularly codirected the highly successful Aspen summer conferences in pediatric GI and hepatology since 1985. In all these diverse conferences, Bill has left his mark due to his ability to connect with the audience and to provide insight into pediatric hepatology. All we who have enjoyed them as fellows and faculty have benefited from the exchange of knowledge and the ability to meet positive role models.

In 1985, Bill organized the first postgraduate course at the first NASPGN-ESPGHAN joint meeting in New York. This meeting, which was planned during his term as President of NASPGN, paved the way for subsequent NASPGN-ESPGHAN joint meetings and the annual NASPGN postgraduate course and eventually the First World Congress to be held next year, where Bill will again direct the postgraduate course.

Bill coauthored one of the first textbooks of pediatric hepatology, a book resulting from the Aspen conference in 1987 (15). He also coauthored and eventually authored several revisions of the tome-like chapter on pediatric hepatology in Schiff's textbook of hepatology (16). He has served as an associate editor and reviewer for numerous specialty journals. In 1991, Bill became the first Western Hemisphere editor of the Journal of Pediatric Gastroenterology and Nutrition (JPGN). Under his leadership, JPGN became the official journal of NASPGN-ESPGHAN and the voice of our subspecialty. The journal's visibility increased, and it was indexed in Current Contents. Bill wanted all NASPGN members to experience ownership of JPGN, and he went to great lengths to obtain manuscript reviews from a wide cross-section of the membership. Bill's philosophy was to define peer review as democratically as possible. In part because of his talent and his success with JPGN, Bill subsequently became the editor of the Journal of Pediatrics in 1996. While of course showing no editorial preference, Bill has elevated the subspecialty of pediatric gastroenterology, hepatology, and nutrition within the specialty of pediatrics by his editorship of this prestigious journal. Similarly, Bill has elevated the stature of pediatric hepatology within the specialty of hepatology by his long-standing participation in the American Association for the Study of Liver Diseases (AASLD). He assumed the presidency of this organization in November 1999, and he is the first pediatrician to be president of the AASLD.

In addition to being highly innovative and giving of his time, Bill is a terrific humorist and can match wits with anyone. Laughter is very pleasing to his ear, and no one is spared the well-intentioned barb. He is also fond of using humor in his talks, both to entertain and to teach. Bill is also wont to teach by the use of pithy aphorisms such as, “If all you have is a hammer, everything looks like a nail.” Bill teaches that, “Uncommon manifestations of common diseases are more common than common manifestations of uncommon diseases.” Nonetheless, many of us go to him for his diagnostic acumen, especially when we suspect a rare or puzzling answer.

Some of his most important international role models in pediatric hepatology were Professors Alagille, Kasai, and Mowat. Possibly more than any other academic leader, Alec Mowat had the greatest influence on Bill. Bill was with him in Chile at the time of his premature death. In many ways, Bill shared Mowat's credo. They both thought and cared about children and had a common belief that children with liver disease needed a special focus distinct from that of internal medicine (17,18). They both spent a lot of time teaching. Like Mowat, when Bill is teaching, no one ever gets short shrift. No one is ever put down for asking a question. In fact, civility is his watchword. If the questioner is enthusiastic, Bill is more enthusiastic. He has a response for every student. For the wise student, he has a challenge. For the simple student, he has encouragement and information. For the wicked student, he has a kind word. And for the student who does not know to ask, Bill can draw out the question and get him or her to deliver the answer.

Another of Bill's top priorities is his family. He and his wife, Becky Balistreri, have raised three wonderful children. Tony is a senior resident in internal medicine and will enter a fellowship in gastroenterology at the Medical University of South Carolina next summer. Bill is very proud of the first review to be published shortly in Annals of Internal Medicine by Balistreri and Balistreri (19). Jenny is a graduate of the University of Indiana and is now a senior account executive with Federated Department Stores in Cincinnati. She is a special friend and running companion. Billy is a senior student at Wake Forest University and is a highly talented musician.

Bill's accomplishments are too many to enumerate. Rather, I have taken the opportunity to share a portion of them with you and to try to give some insight into why he is special and so clearly deserving of the Shwachman award.

All of us at Cincinnati and all of us in NASPGN are very proud of you and grateful for your leadership. In conclusion, it is a great honor to introduce to you, the 1999 Shwachman award winner of NASPGN, William F. Balistreri, M.D.

REFERENCES

1. Balistreri WF, Partin JC, Schubert WK. Bile acid malabsorption a consequence of terminal ileal dysfunction in protracted diarrhea of infancy. J Pediatr 1977; 90:2128.
2. Balistreri WF, Cowan AEC, Hofmann AF, Szczepanik PA, Klein PJ. Validation of use of 11,122H labeled chenodeoxycholic acid in isotope dilution measurements of bile acid kinetics in man. Pediatr Res 1975; 9:757–60.
3. Heubi JE, Balistreri WF, Partin JC, Schubert WK. Refractory infantile diarrhea due to primary bile acid malabsorption. J Pediatr 1979; 94:546–51.
4. Heubi JE, Balistreri WF, Fondacaro JD, Partin JC, Schubert WK. Primary bile acid malabsorption: Defective in vitro ileal active bile acid transport. Gastroenterology 1982; 83:804–11.
5. Setchell KDR, Suchy FJ, Welsh, MB, Zimmer-Nechemias L, Heubi J, Balistreri WF. Δ43oxosteroid 5β-reductase deficiency described in identical twins with neonatal hepatitis a new inborn error in bile acid synthesis. J Clin Invest 1988; 82:2148–57.
6. Balistreri WF, A-Kader HH, Setchell KDR, Ryckman FC. Ursodeoxycholic acid therapy in patients with chronic intrahepatic cholestasis and extrahepatic biliary atresia: Preliminary results of an open-label, multi-center trial. Proceedings of the Fifth International Sendai Symposium on Biliary Atresia, In: Ohi R, ed. Biliary Atresia. Tokyo: Icom Associates, Inc., 1991: 167–72.
7. Balistreri WF, A-Kader HH, Setchell KDR and the UDCA Study Group. Ursodeoxycholic acid therapy in pediatric patients with chronic cholestasis. In: Lentze, Reichen, eds. Pediatric Cholestasis: Approaches to Treatment. London: Kluwer Academic Publishers, 1992:333–44.
8. Daugherty CC, Setchell KD, Heubi JE, Balistreri WF. Resolution of liver biopsy alterations in three siblings with bile acid treatment of an inborn error of bile acid metabolism (delta 4-3-oxosteroid 5 beta-reductase deficiency). Hepatology 1993; 18:1096–101.
9. Bove KE, Kosmetatos N, Wedig KE, et al. Vasculopathic hepatotoxicity associated with E-Ferol syndrome in low-birth-weight infants. JAMA 1985; 254:2422–30.
10. Balistreri WF, Farrell MK, Bove KE. Lessons from the E-Ferol tragedy. Pediatrics 1986; 78:503—6.
11. Balistreri WF, Heubi JE, Suchy FJ. Immaturity of the enterohepatic circulation in early life: Factors predisposing to “physiologic” maldigestion and cholestasis. J Pediatr Gastroenterol Nutr 1983; 2:346–54.
12. Balistreri WF. Fetal bile acid synthesis and metabolism: Clinical implications. J Inherit Metab Dis 1991; 14:459–77.
13. Suchy FJ, Balistreri WF, Heubi JE, Searcy JE, Levin RS. Physiologic cholestasis: Elevation of the primary serum bile acid concentrations in normal infants. Gastroenterology 1981; 80:1037—41.
14. Balistreri WF, Korman MD, Cowan AE, Hofmann AF. Sulfated lithocholic acid in Reye's syndrome. In: Pollack JD. Reye's Syndrome. New York: Grune and Stratton, 1975: 269–71.
15. Balistreri WF, Stocker JT, eds. Pediatric Hepatology. New York: Hemisphere Publishing Co., 1990.
16. Balistreri WF. Liver disease in infancy and childhood. In: Schiff ER, Sorrell MF, Maddrey WC, eds. Schiff's diseases of the liver. 8th ed. Philadelphia: Lippincott–Raven, 1999: 1357–512.
17. Balistreri WF. Patients/patience: Alex P. Mowat in a word. Pregnancy, sex hormones, and the liver. In: Reyes HB, Leuschner I, Arias I, eds. Proceedings of the 89th Falk Symposium. Lancaster, UK: Kluwer Academic Publishers, 1996: xiv–xvi.
18. Mowat A, Balistreri WF. Biliary atresia into the 21st century: A historical perspective. Hepatology 1996; 23:1693–5.
19. Balistreri TM, Balistreri WF. Review of “Clinical Practice of Gastroenterology, Volume One and Volume Two.”Ann Intern Med 1999. In press.
© 2000 Lippincott Williams & Wilkins, Inc.