PEaRLT h e P a n c r e a s E d u c a t i o n a n d R e s e a r c h L e t t e r

On the Internet:www.pancreas.org

Toll-Free Phone:1-888-PITT-DNA(1-888-748-8362)

T he North American Pancreatitis Group withcollaborators from England and Germany reported

the discovery of a genetic risk for chronic pancreatitisin men who are heavy drinkers. The report appeared as anadvanced on-line report in Nature Geneticson November 12, 2012. The discovery wasidentified as a genetic variant located on theX chromosome near the claudin-2 gene(CLDN2). This breakthrough will now helpdoctors caring for patients with pancreatitisto know how likely a patient will be toprogress to chronic pancreatitis in the future.It will also allow doctors to counsel specifichigh risk patients about preventing theprogression of disease.

The factor on chromosome X does notappear to cause pancreatitis but if pancreaticinjury occurs for any reason, such as gall-stone pancreatitis or abdominal trauma, it is more likelythat the person will develop chronic pancreatitis –especially if they also drink alcohol.

“The discovery that chronic pancreatitis has a geneticbasis solves a major mystery about why some peopledevelop chronic pancreatitis and others do not,” saidDavid C. Whitcomb, MD, PhD, the study’s leading author.“We also knew there was an unexpected higher risk of mendeveloping pancreatitis with alcohol consumption, but until

now we weren’t sure why. Our discovery of this new geneticvariant on chromosome X helps solve part of this mystery.”

The link between men, alcohol and the factor on the Xchromosome is very interesting. Researchers discovered

that the high-risk DNA variant on the Xchromosome was common, and presentin 26 percent of men without pancreatitis,but jumps to nearly 50 percent of mendiagnosed with alcoholic pancreatitis.Women have two X chromosomes, so mostwomen with the high-risk DNA variant onone X chromosome appear to be protectedfrom alcoholic chronic pancreatitis by theother X chromosome, if it is normal. Men haveone X chromosome and one Y chromosome,so if they inherit a high-risk X chromosome,there is no protection.

The finding, among the many others weshare with you in the PEaRL, was conducted on studysamples from participants in the North American Pancre-atitis Study II, or NAPS2. Samples from the HereditaryPancreatitis study, PAGER study and PROOF study werealso included. We thank you for your participation! �

This research was supported by the National Institutesof Health (NIH) (grants DK061451, DK054709, DK063922,MH057881, CA117926, UL1RR024153 andUL1TR000005).

Volume Six

Number Four

Fall / Winter 2012

~P E a R L is a

publication of the

Pancreatic Studies Office in the

Division of Gastroenterology,

Hepatology and Nutrition

at the University of Pittsburgh

~Editors

David C. Whitcomb, MD, PhD

Sheila Solomon, MS

~Publication of P E a R L

is supported, in part,

with support

from Abbott

Genetic Discovery Links Alcoholic Pancreatitiswith Chromosome X Factor

H A P P E N I N G S

Sheila Solomon, MS, Certified Genetic Counselor at the Universityof Pittsburgh, Division of Gastroenterology, Hepatology and

Nutrition, was invited to speak on the “Complexities of HereditaryPancreatitis: Clinical Implications for Your Practice”at the National Society of Genetic Counselors’ AnnualEducation Meeting in Boston, MA this October.

Ms. Solomon explained the details of this rarecondition to more than 1,600 genetic counselors fromacross the country and the world in attendance.“Regardless of the rarity of the disease, it is critical

to share this information with health care providers of all types,”Solomon explained. “Many genetic counselors approached me afterthe talk with questions and comments about how they can nowguide patient care based on this new information.”

The learning never ends. �

Sheila Solomon, MS, CGC speaks to the National Societyof Genetic Counselors about Hereditary Pancreatitis.

Turkey Winter Vegetable Stew

Wondering what to do with your extra turkeymeat from the holidays? This richly flavored

stew is full of vitamins, minerals, and protein. It can beserved as soup or over a whole grain for a heartier meal.

Ingredients:1-1/2 tablespoons olive oil1 pound cooked turkey white meat, diced2-1/3 cups reduced-sodium chicken

or vegetable broth2/3 cup dry red wine2 cloves garlic, minced2 shallots, thinly sliced1 tablespoon fresh thyme, chopped1/4 teaspoon each ground sea salt

and freshly ground black pepper1 bay leaf2 cups butternut squash, peeled, seeded, and diced1 cup parsnips, peeled and sliced1 medium-large yam, peeled and chopped3 ribs celery, chopped1 medium yellow onion, thinly sliced1/2 cup reduced-fat sour cream3 tablespoons whole wheat flour

Instructions:Place a large saucepan over medium heat. Add oil

and heat about 1 minute. Add chicken and brown on allsides. Stir in broth and wine. Add garlic, shallots, thyme,salt, pepper, and bay leaf. Bring mixture to a boil.Reduce heat, cover, and simmer 20 minutes. Mix insquash, parsnips, yam, celery, and onion. Bring to a boil;reduce heat to low and simmer 30 minutes, or untilvegetables are tender. In a small bowl, blend togethersour cream and flour. Gradually stir 1/2 cup of the hotstew into the sour cream/flour mixture. Add the sourcream mixture to the saucepan. Remove bay leaf andcontinue to cook, stirring occasionally, until soup hasthickened, about 6–8 minutes. Makes 6 servings.

Nutritional informationper serving:

Calories: 298Fat: 4 gSaturated fat: 1 gCarbohydrate: 20 gTotal sugars: 3 gProtein: 20 gSodium: 260 mgCholesterol: 50 mgDietary fiber: 3 g

Page 2 ~ Fall / Winter 2012 www.pancreas.org

K i d s ’ C o r n e r

Be INSPPIRED This Holiday Season!

INSPPIRE (IInnternational SStudy Group of PPediatricPPancreatitis: IIn search for a curree) is a group comprised

of 21 pediatric gastroenterology centers in the UnitedStates, Canada, Israel and Australia. INSPPIRE is the first-ever initiative to create a multi-center approach to sys-

tematically characterize pancreatitisin children, a poorly understood

problem for which there are noclear diagnostic guidelines ortherapeutic alternatives.

The group is led by Dr. Aliye Uc,(photo, above right) an Associate

Professor of Pediatrics and a pediatricgastroenterologist at the University of Iowa

Children’s Hospital. Dr. Uc recently received a grant fromthe National Institutes of Health (R21 grant, $275,000 in2 years) to build a consortium that includes 15 academicinstitutions, with centers in the US, Canada, Australia, andIsrael. The University of Iowa is the coordinating center.

The INSPPIRE group created task forces to definepediatric acute pancreatitis, acute recurrent pancreatitis

and chronic pancreatitis; conducteda survey of INSPPIRE members toexamine the number of patients fol-lowed at each institution, the scope ofthe problem and the current practice;identified the most important ques-tions in pediatric pancreatitis; andformed a subcommittee to developstandardized patient and physician questionnaires.

The immediate goal of INSPPIRE is to develop a data-base of children with acute recurrent pancreatitis andchronic pancreatitis and to understand the epidemiology,etiologies, natural history and outcome in a well-studiedgroup of children with these disorders. The initial projectwill define pediatric acute recurrent pancreatitis andchronic pancreatitis, understand its epidemiology andbuild a patient registry and sample repository. The group’slong-term goal is to develop new, effective therapies forthese diseases in children.

We wish them the best as they make an importanteffort to help children with pancreatitis. �

www.pancreas.org Fall / Winter 2012 ~ Page 3

A team of University of Pittsburgh Medical Center pancreatic cancer surgeons led by Herbert Zeh, MD

and transplantation surgeons led by Abhinav Humar, MDperformed the first total pancreatectomy with isletautotrans plantation (TPIAT) using minimally invasiverobot-assisted surgery. This highly advanced procedure fortotal pancreatectomy was designed for pancreatic cancersurgery because the traumatic effect on the body is muchless, leading to a shorter recovery time and more strengthto combat the cancer and cancer therapy. The surgeonsuse the Da Vinci robot, which gives the surgeon a moreclear vision of the tissue and high magnification to dovery delicate cutting and suturing.

TPAIT is a procedure used for severe pain and disabilityin patients with recurrent acute pancreatitis and chronicpancreatitis to remove the source of pain while retaining thespecialized islet cells that make insulin and prevent diabetes.Dr. Humar’s team are experts at autotrans plan tation, butin the past the procedure required a major incision acrossthe abdomen and resulted in a long recovery time.

Combining the robotic-assisted approach to thepancreas with the methods of islet autotransplantationis a win-win. Dr. Zeh’s team was able to complete theprocedure with no complications. Dr. Humar’s teamassured that the islet cells had minimal damage duringthe removal phase, and retained the maximal number ofislet cells. Both teams of surgeons confirmed that thenew approach in this case was a total success. �

Each year physician-scientistsmeet in Pittsburgh to discuss

advances and ways to work toge -ther as national and internationalteams of scientists to bring scientificadvances to the patients as fast aspossible. PancreasFest 2012,held on July 26, 27, 2012 onthe campus of the Universityof Pittsburgh, boasted oneof the largest and mostwidely successful meetingsof its history. The focus forPancreasFest 2012 was tohighlight pancreatic cancerand chronic pancreatitisadvancements ranging fromcancer prevention and earlydetection strategies, totreatment advancementimplications for chronic andacute pancreatitis care as well asapplying concepts of personalizedmedicine to pancreas disease treat-ment. New this year was a discus-sion of treatment implications and options through case discussion,

whereby participants used a handheld wireless device to measureattendee education as well as tovote on recommendations forchronic pancreatitis care options.Integrating this high tech function

into the program was well receivedand results will be published soon.

One of the highlights ofPancreasFest 2012 includedhonoring Ralph Hruban, MD,Professor of Pathology and

Oncology at The Johns HopkinsUniversity School of Medicine. He is currently the Director ofthe Sol Goldman PancreaticCancer Research Center, andDirector of the Division of Gastro -

intestinal/Liver Pathology.Dr. Hruban has dedicated hiscareer to understanding thedevelopment of pancreaticcancer and has been hugelyinflu ential in the progressof identifying at riskpatients and clarifying thepathology and genetics ofpancreatic cancer.

At PancreasFest, Dr. Hrubanreceived the Ruth C. BrufskyAward for Excellence inClinical Research onPancre atic Cancer and spoke

to the group on the “PancreaticPathology and Whole GenomeSequencing: Implications for Diagnostics and Therapeutics”.

Congratulations and thank you,Dr. Hruban. �

PancreasFest 2012 Highlights

From left to right: David Whitcomb, MD, PhD, Ralph Hruban, MD,and Randall Brand, MD

Dr. Zeh using the Da Vinci robot during a pancreatic surgery.

First Total Pancreatectomy for Islet Autotransplantation

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Fall / Winter 2012

For more information, contact Sheila Solomon, MS at 1-888-PITT-DNA or solomonsr@upmc.edu

Each year the Pittsburgh and Boston chapters of theNational Pancreas Foundation (NPF) identify and

honor physician-scientists who have dedicated their livesto treating patients with pancreatic diseases.

On October 26, 2012 the Pittsburgh chapter of theNPF honored Herbert Zeh, MD, a pancreatic surgeon atthe University of Pittsburgh who has pioneered the useof minimally invasive pancreatic surgery for pancreaticdiseases using robotic-assisted methods (see featurearticle on page 3). Dr. Zeh also directs a basic sciencelaboratory focused on pancreatic inflammation.

On November 29, 2012 the Boston chapter of the NPFhonored Darwin Conwell, MD, a clinical pancreatologistat Harvard University who practices at the Brigham andWoman’s Medical Center in Boston, MA. Dr. Conwell haspioneered better methods of evaluating the function ofpancreas as a diagnostic test. He is also recognized as ateacher and mentor of physicians who are interested inadvanced training in managing pancreatic diseases. �

Pancreatic Clinicians Honored by National Pancreas Foundation

Darwin Conwell, MD (right) with his senior partner, Peter Banks, MDat the NPF Gala in Boston, MA.