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CHALLENGES IN

PEDIATRIC ULCERATIVE COLITIS

ADVICE TO THE PRACTITIONER

Diagnosisand Management of

Ulcerative Colitis

therefore, are generally safe even in individuals withulcerative colitis who are on immunosuppressive ther-apy. All patients with ulcerative colitis should receivean annual vaccine for influenza. All patients with ul-cerative colitis should be considered for an annualvaccine for influenza.

Live viral vaccines should be avoided in immune com-promised children. This includes children on steroids(prednisone >20mg/d or 2mg/kg/day for 2 weeksor more), 6 MP/azathioprine, or methotrexate.Whenever possible, serologic conversion should bedocumented in children being immunized while im-mune compromised.

When should I be concerned that the ulcerativecolitis is flaring?

It is an old adage that patients flare true. The symp-toms the child had at first presentation are generallythe same symptoms at time of flare. Rectal bleedingwould be the most common symptom of a UC flare.Other potential symptoms include diarrhea, fever,persistent abdominal pain or extra-intestinal symp-

toms including a rash, arthralgias/arthritis, jaundiceor eye pain.

When is it not a disease flare? It is not always easy to distinguish between a viral

illness and a flare of the ulcerative colitis. Time,patience and resolution of symptoms are often thedetermining factors. It is important to consider othersources for an illness prior to ascribing symptomsto the ulcerative colitis. Stool cultures and screeningblood work looking for anemia, hypoalbuminemiaor elevation of acute phase reactants can be helpfulas well. Patients and their families can often tell thedifference between an intercurrent illness and a flare

of the ulcerative colitis.

First line anti-pyretic and analgesic therapy (for com-mon fever, headaches and the like) should probablybe acetaminophen since there is some data to sug-gest that chronic NSAID therapy may adversely af-fect UC.

What is the role of surgery?

While medical therapy is the mainstay of treatment, sur-gery is performed in as many as 25% of pediatric UCpatients. Steroid dependence and lack of response tomedical therapy are the most common indications for

colectomy in UC.Advances in surgical techniques have made it possibleto remove the colon and use the terminal ileum as a neo-

rectum or pouch. The ultimate result allows for continuityof the gastrointestinal tract without a permanent stoma. Aninflammation of the pouch can develop (pouchitis) and thiscan usually be t reated medically.

How important is cancer surveillancein a UC patient?

It has been well established that patients with UC have anincreased risk of colo-rectal cancer. Regular colonoscopyfor cancer surveillance is recommended for all patients whohave had UC more than 8-10 years including pediatric andadolescent patients. Surveillance of the pouch in patientswho have undergone colectomy is usually recommendedas well.

www.KidsIBD.orgwww.CDHNF.orgwww.NASPGHAN.org

CDHNF National Office:P.O. Box 6, FlourtownPA 19031

Phone: 215-233-0808

Educational support for The CDHNFPediatric Ulcerative Colitis Campaign

was provided by Procter&Gamble

Physician Notes

CHILDRENS DIGESTIVE

HEALTH & NUTRITION

FOUNDATION

NORTH AMERICAN SOCIETY FORPEDIATRIC GASTROENTEROLOGY,HEPATOLOGY AND NUTRITION

CHILDRENS DIGESTIVE

HEALTH & NUTRITION

FOUNDATION

NORTH AMERICAN SOCIETY FORPEDIATRIC GASTROENTEROLOGY,HEPATOLOGY AND NUTRITION

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What is pediatric ulcerative colitis?

Ulcerative Colitis (UC) is an auto-inflammatory process thatresults in chronic inflammation of the large intestine. UCinvolves only the mucosa and extends proximally from therectum. In children and adolescents, the area of involve-ment often extends beyond the splenic flexure. The extentof involvement does not correlate well with clinical disease

activity. The mucosal depth and continuous distribution ofthe inflammation distinguishes UC from Crohns disease inmost cases.

Most researchers believe that UC is caused by a combi-nation of genetic and environmental factors. Although UCmay occur at any pediatric age, it is most often diagnosedin early adolescence. It affects males and females equally.

How does ulcerative colitis present?

The most common presentation of a patient with UC isbloody diarrhea and abdominal cramping. Extra-intestinalsymptoms may also be present such as fever, skin rash, joint

symptoms including frank arthritis, and liver disease.

What evaluation should be offered bythe primary health care provider?

Cultures for bacterial pathogens (Salmonella, Shigella,Campylobacter, Yersinia, E. Coli including 0157:H7,Clostridium difficile toxins A & B) should be completed.Lab testing for parasites including serology for Entamebahistolytica should be considered.

Additional testing (Complete blood counts with differential,sedimentation rate, C-reactive protein and serum chemis-tries including albumin) should be part of the primary evalu-

ation of the patient with persistent rectal bleeding. Anemia,elevation of acute phase reactants and hypoalbuminemiamay be present indicating inflammation and chronicity. Ifthe cultures are negative and the symptoms persist beyondtwo weeks, then referral to a pediatric gastroenterologist isappropriate.

When should I refer to a pediatricgastroenterologist?

Patients presenting with more insidious intermittent rectalbleeding, additional factors such as weight loss; extra-intes-tinal manifestations of inflammation and a family history of

IBD should prompt early evaluation and referral.In patients where the infectious evaluation is negative butbleeding persists with or without pain, referral is indicated.

How is the diagnosis made?

The diagnostic gold standard is a colonoscopy with bi-opsy. Grossly, a continuous colitis that begins in the rectumis the most common appearance of UC. Biopsies are takento confirm the diagnosis and further exclude infections.It is not unusual for a pediatric gastroenterologist to alsoperform esophagogastroduodenoscopy (EGD) at thetime of the colonoscopy since the EGD can help classify thediagnosis as one of Crohns disease or UC. Additonally,acontrast small bowel series is frequently performedto image the rest of the bowel and to confirm that all theinvolvement is restricted to the colon and does not involvethe small intestine, as is seen in Crohns disease.

More recently, it has been noted that there are various anti-bodies that are seen in a majority of individuals with inflam-matory bowel disease (IBD). While the diagnosis of IBDcannot be made serologically, antibody testing can helpto confirm the diagnosis. Peri-nuclear anti-neutrophil cyto-plasmic antibody (pANCA) is an auto-antibody that is mostassociated with UC and may help predict some potentiallong-term complications.

What treatments should I expect mypatient to receive?

As with any chronic condition, the first goal of medical ther-apy in UC is to induce a clinical remission. Once remission

is achieved, therapy is directed to maintain that remission.Corticosteroids have been the mainstay of induction thera-py for many years. The rapid onset of action makes steroids

an attractive choice especially in patients who are ill andhave a poor quality of life at the time of diagnosis. Theimmunosuppressive, cosmetic and metabolic side effectsof steroids make them inappropriate to use as mainte-nance agents.

5-aminosalicylic acid (5-ASA) preparations are ef-fective anti-inflammatory agents and have been dem-onstrated to both induce and maintain remission in UC.

Clinical response can be somewhat slower than steroidsand, therefore, patients with severe UC continue to re-ceive steroids in most cases. Once remission is achieved,either with 5-ASA or steroids, maintenance therapy with5-ASA is the most common therapeutic approach. Thereare oral and rectal preparations available.

Recurrent use of steroids becomes a concern in patientswho are intolerant or unresponsive to 5-ASA. Since agoal of therapy is to eliminate long-term steroid use, im-munomodulator therapy may be introduced. 6-mercapto-purine (6MP) and its parent compound Azathioprine arethe immunomodulators most commonly used to steroid-spare in pediatric UC.

Most recently, biologic therapy in the form of infliximab(Remicade) has been approved for the treatment of re-fractory UC in adults.

In addition to medical therapy, psychosocial support ofthe pediatric patient and the affected family is critical inUC or any chronic pediatric illness. Implementation of apsychosocial support plan is a critical component of thehealth care plan. Often, the primary care provider hasa unique and long term relationship with the family andchild. This relationship can facilitate the execution of acoordinated psychosocial support plan

What are primary care issuesin ulcerative colitis

How important are vaccines? It is important to recognize that children with ulcer-

ative colitis continue to be at risk for routine childhoodillnesses. Immunizations are an important protectivemechanism and children and adults with ulcerativecolitis should be maintained on the recommended im-munization schedule.

At diagnosis, it is important that the immunization his-tory be reviewed so that catch-up immunizations canbe given if needed. Immunity to varicella should beconfirmed by history or serology so that those whorequire varicella vaccine can receive the appropriatedoses prior to receiving immunosuppressive therapy.The majority of vaccines do not contain live virus and,