Overview

Inflammatory bowel disease (IBD) is a chronic immune-mediated disorder of the digestive tract in which the body inappropriately attacks its intestines. Symptoms vary depending on the location and extent of inflamed bowel but usually include chronic diarrhea that is sometimes bloody, abdominal pain, and weight loss. Extraintestinal conditions may involve the mouth, skin, joints, liver, or eyes. IBD has the potential to affect nutrition, growth, and puberty. This resource provides guidance to primary care clinicians to care for children and teens with IBD.

The term IBD covers 2 disease entities that can have significant clinical overlap: ulcerative colitis (UC), in which inflammation affects mostly the large intestine, and Crohn’s disease (CD), where any portion of the digestive tract from mouth to anus can be affected. Children with IBD who do not fit either category (≈15%) are diagnosed with “indeterminate colitis” or inflammatory bowel disease unclassified type (IBD-U).

Prevalence

The prevalence of ulcerative colitis and Crohn’s disease in children younger than 20 years is 1:2,326 and 1:3,571, respectively. [Kappelman: 2007] IBD peaks around age 20 and again at age 60; nearly 25% of patients with IBD present before the age of 20. [Wong: 2008] The peak incidence of IBD occurs in patients between 15 and 30 years. [Johnston: 2008]

There is a very early-onset form of IBD (VEO-IBD) in children ≤6 years of age and constituting 4-10% of pediatric IBD, also known as monogenic IBD. [Moran: 2017] Approximately 5-10% of patients develop IBD during childhood or adolescence; the incidence of pediatric IBD appears to be increasing.

Genetics

Though the DNA of patients with IBD has been extensively studied, no causative genes have been found. However, twin studies suggest a genetic component: [Halme: 2006]

• In identical twins, 20-50% will both get Crohn’s disease; 15% will both get ulcerative colitis.
• In non-identical twins, 5-10% will both get Crohn’s; 5% will both get ulcerative colitis
• In non-twin siblings, 5-10% will both get IBD.

Prognosis

The inability to absorb adequate calories and nutrients can lead to malnutrition, growth retardation, abnormal sexual maturation, and vitamin and micronutrient deficiencies. Chronic inflammation leads to anemia and, in Crohn's disease, to abscess, stricture, and fistula formation. Both Crohn’s disease and ulcerative colitis carry an increased risk for colorectal cancers, particularly when disease has been present for many years. Significant limitations in school, sports, and other activities occur with flares or with poorly controlled disease. The social stigma associated with fecal urgency or surgical ostomy can be extreme. Some children develop comorbid anxiety and depression.

Practice Guidelines

Turner D, Levine A, Escher JC, Griffiths AM, Russell RK, Dignass A, Dias JA, Bronsky J, Braegger CP, Cucchiara S, de Ridder L, Fagerberg UL, Hussey S, Hugot JP, Kolacek S, Kolho KL, Lionetti P, Paerregaard A, Potapov A, Rintala R, Serban DE, Staiano A, Sweeny B, Veerman G, Veres G, Wilson DC, Ruemmele FM.
Management of pediatric ulcerative colitis: joint ECCO and ESPGHAN evidence-based consensus guidelines.
J Pediatr Gastroenterol Nutr. 2012;55(3):340-61. PubMed abstract

Ruemmele FM, Veres G, Kolho KL, Griffiths A, Levine A, Escher JC, Amil Dias J, Barabino A, Braegger CP, Bronsky J, Buderus S, Martín-de-Carpi J, De Ridder L, Fagerberg UL, Hugot JP, Kierkus J, Kolacek S, Koletzko S, Lionetti P, Miele E, Navas López VM, Paerregaard A, Russell RK, Serban DE, Shaoul R, Van Rheenen P, Veereman G, Weiss B, Wilson D, Dignass A, Eliakim A, Winter H, Turner D.
Consensus guidelines of ECCO/ESPGHAN on the medical management of pediatric Crohn's disease.
J Crohns Colitis. 2014;8(10):1179-207. PubMed abstract / Full Text

Critch J, Day AS, Otley A, King-Moore C, Teitelbaum JE, Shashidhar H.
Use of enteral nutrition for the control of intestinal inflammation in pediatric Crohn disease.
J Pediatr Gastroenterol Nutr. 2012;54(2):298-305. PubMed abstract

Roles of the Medical Home

The medical home should:

• Monitor for signs of ongoing disease and exacerbation.
• Monitor for side effects of medications.
• Help ensure adequate catch-up growth.
• Monitor for appropriate pubertal development.
• Screen for physical findings of vitamin and mineral deficiencies.
• Screen for comorbid depression and anxiety.
• Evaluate illnesses in patients on immune-modulating medications.
• Work with the patient and family to encourage therapeutic compliance.
• Perform routine health screening and immunizations.
• Assist family in establishing care with a behavioral therapist.
• Assist family with eventual transition to adult care.

Ongoing collaboration with pediatric gastroenterology may optimize outcomes for patients. Follow-up with the subspecialist varies from patient to patient and with response to medications but generally occurs every 1–2 months following initial diagnosis and every 6–12 months in patients with remission. Development of a 504 Plan may include immediate/discreet access to a bathroom, stop-the-clock testing, access to healthy snacks and beverages, plans for unintended late arrivals to school, and alternative activities for physical education class. School Accommodations: IEPs & 504s has tips for collaborating with school personnel to help ensure appropriate education-related services.

Pearls & Alerts for Assessment

Most abdominal pain in children is not IBD

The child with gassy abdominal pains and bloating, non-focal or periumbilical pain, no diarrhea, no fecal urgency, no nocturnal stooling, and normal growth is unlikely to have IBD, especially if routine bloodwork, including an inflammatory marker (ESR or CRP), is normal. For patients who present with common symptoms of abdominal pain, the most common diagnoses for these are functional abdominal pain (FAP) or constipation. Most hematochezia in children is from constipation, causing external or internal anal fissure, hemorrhoids, etc. [Tabbers: 2014]

For the Condition

For patients with clinically concerning symptoms (weight loss, diarrhea, blood in stool), screening is performed with serologic testing: CBC, CMP, CRP, ESR. For diarrhea, a fecal calprotectin may be useful for screening as it is a marker of ongoing inflammation in the intestines. The main utility of this test is to aid in diagnosing and monitoring IBD and differentiating it from functional gastrointestinal disorders (FGIDs). [Koninckx: 2021] A recent study shows that it may not be as sensitive a marker for Crohn’s as for UC. [Degraeuwe: 2015] [Henderson: 2014]

Fecal calprotectin levels are better non-invasive screening markers than serology, such as elevated CRP or ESR, at the time of diagnosis. [Levine: 2014]

Of Family Members

Studies have shown that between 1.5% and 28% of people with IBD have a first-degree relative, such as a parent, child, or sibling, who also has one of the diseases. [Crohn’s: 2021]

The genetic risk is greater with Crohn’s disease than ulcerative colitis.

The risk of Crohn’s disease or ulcerative colitis is substantially higher when both parents have IBD.

There is no official recommendation for screening family members, but clinical suspicion should be increased for IBD if family members develop red flag symptoms as discussed in the history and physical section.

For Complications

Patients with IBD on immunosuppressive medications should have bloodwork monitored by their gastroenterologist no less than every 3 months to look for liver damage, pancreatitis, low white blood cell count, and other complications of immunosuppression, including lymphoma. For dysplasia screening, consider a colonoscopy every 5 years after diagnosis.

Presentations

Presentation depends, in part, upon the location and extent of the inflamed bowel. Inflammation in Crohn’s disease may occur anywhere along the GI tract. Most individuals with Crohn’s disease have inflammation in the distal ileum and cecum (~70%). Ulcerative colitis can have variable severity. By definition, the inflammation in those with ulcerative colitis always involves only the large bowel. Either disorder can have associated extraintestinal symptoms. In both conditions, the inflammation manifests primarily as abdominal pain and diarrhea. For this review, patient presentations will be divided into mild intestinal, moderate/severe intestinal, and primarily extraintestinal patterns.

Mild intestinal presentation: Approximately 60% of children with IBD present, typically in an outpatient setting, with mild colonic and distal small bowel symptoms, chronic diarrhea, cramps, and abdominal pains. Mild blood in the stools is more characteristic of ulcerative colitis, but it can present in ~30% of those with Crohn’s disease. Children with mild presentation have normal vital signs, look well, and have unremarkable physical exams.

Moderate/severe intestinal presentation: Approximately 30% of children with IBD present with moderate or severe colonic and distal small bowel symptoms that may include frequent and/or grossly bloody stools, cramps, fecal urgency, fevers, weight loss, and some degree of general systemic illness. Vital sign instability, dehydration, anemia, or pain not controlled by over-the-counter medications may lead to hospitalization. A distended or tender abdomen, particularly with any signs of peritonitis, requires urgent referral for imaging and evaluation by the most experienced team available—ideally, a tertiary care center with a pediatric surgeon. Complications of IBD, such as perforation, obstructive strictures, and toxic megacolon, are surgical emergencies.

Primarily extraintestinal presentation: About 10-50% of children with IBD present with extracolonic symptoms. The most common is growth failure. IBD deserves consideration in an appropriately aged patient with otherwise idiopathic weight loss meeting malnutrition parameters (low weight for age with or without low height for age, or low weight for height) as well as delayed puberty. A pattern of chronic nausea, vomiting, and anorexia is seen in Crohn’s disease with stomach or proximal small bowel involvement. Some children with Crohn’s disease present with just perianal disease. Additional symptoms may include any combination of fatigue, malaise, oligoarthritis arthritis or arthralgia, recurrent oral aphthous ulceration, erythema nodosum, pyoderma gangrenosum, anemia, hepatitis, and digital clubbing.

Patients with other primary autoimmune disorders such as celiac disease, juvenile inflammatory arthritis, vasculitis, for example, can have an increased lifetime risk of developing IBD as well.

Diagnostic Criteria

Diagnosis is usually made by a pediatric gastroenterologist and pathologist. It is based on endoscopic and histologic data, with a suggestive history and physical exam, and after exclusion of infectious etiology. Definitive diagnosis requires all 3 of the following:

1. Clinical symptoms (diarrhea, rectal bleeding, abdominal pain, weight loss or growth disturbance, complicated perianal disease, and/or fevers)
2. Appropriate time course (symptoms on 2 or more occasions separated by at least 8 weeks, or ongoing symptoms for at least 6 weeks)
3. Objective evidence of inflammation on endoscopy, radiology, and/or histology

Abnormal CBC, CRP, ESR, and albumin can suggest the need for further workup but cannot alone make or exclude the diagnosis (see Laboratory Testing, below). The best radiographic study to support diagnosis is magnetic resonance enterography (MRE) because it provides real-time information on luminal inflammation and peristalsis and can better characterize strictures in addition to detecting bowel wall inflammation with additional benefit of no radiation. If MRE is not available, computed tomography enterography with contrast (CTE) is the next best option to evaluate for bowel wall inflammation. [Minordi: 2021]

Capsule endoscopy, in which the patient swallows a camera pill that can image the entire GI tract, is available at some centers.

Genome-wide association studies (GWAS) can be used to confirm monogenic IBD genes, which can help differentiate other conditions causing IBD-like symptoms in infancy, such as severe combined immunodeficiency (SCID) and immunodysregulation polyendocrinopathy enteropathy X-linked syndrome (IPEX). The large majority of GWAS-associated loci involve non-coding variation, many of which modulate levels of gene expression. [McGovern: 2015] Meta-analyses of (genome-wide) association studies of adolescent and adult-onset IBD identified 163 IBD-associated genetic loci encompassing about 300 potential candidate genes. However, it is very important to consider that these 163 loci individually contribute only a small percentage of the expected heritability in IBD. This suggests that IBD, including Crohn’s disease and ulcerative colitis, can be regarded as a classical polygenic disorder. [Uhlig: 2014]

More discrete laboratory workups (i.e., ANCA and ASCA antibody panels) are used only in select cases to distinguish between ulcerative colitis and Crohn’s disease. They lack sufficient accuracy to belong in the routine diagnostic workup, are quite expensive, and should only be ordered by specialists. [Wong: 2008]

Clinical Classification

Classification of IBD is divided into remission, mild, moderate, and severe disease. For both Crohn’s disease and UC, pediatric activity index scores are calculated using patient’s growth, symptoms, most recent labs: PUCAI, pediatric ulcerative colitis activity index; PCDAI, pediatric Crohn disease activity index.

PUCAI scores are interpreted as follows:

• 0 to 9 – Remission
• 10 to 34 – Mild disease
• 35 to 64 – Moderate disease
• 65 to 85 – Severe disease

PCDAI scores are interpreted as follows:

• 0 to 10 -- inactive disease
• 11 to 30 --mild disease activity
• >30 -- moderate to severe disease activity.

A decrease in PCDAI of ≥12.5 points reflects a clinical response (improvement from moderate/severe to mild/inactive disease).

Differential Diagnosis

Acute symptoms: Infection is the primary alternative diagnosis, particularly when symptoms present acutely in an otherwise healthy child who has no growth disturbance. Since IBD and acute infection cannot be distinguished by clinical criteria alone, stool studies should be performed to rule out infection prior to initiation of immunosuppressive medications or further (and more expensive) workups.

• Alternative considerations: Bacteria (Clostridium difficile, Salmonella, Shigella, Campylobacter, Yersinia, Aeromonas, Enterohemorrhagic E. coli, and Aeromonas) and protozoa (including Entamoeba histolytica, Blastocystis hominis, Cryptosporidium, and Giardia species), which can present similarly to IBD

Abdominal pain: Recurrent or chronic abdominal pain is common in school-age children; non-organic causes predominate and include the spectrum of functional abdominal pain disorders. IBD almost never presents as pain without diarrhea or growth failure. The child with gassy abdominal pains and bloating, or with primarily periumbilical pain, who has no diarrhea, no fecal urgency, and normal growth is unlikely to have IBD. A normal CBC, CRP, ESR, CMP, and low to normal fecal calprotectin for an otherwise well child with chronic abdominal pain nearly always excludes IBD.

• Alternative considerations: Irritable bowel syndrome, constipation, H. pylori infection, abdominal migraine, and celiac sprue

Rectal bleeding: Chronic constipation is a likely source of mild intermittent rectal bleeding. Hematochezia or melena can arise from intestinal polyps or a Meckel diverticulum; this is usually painless bleeding. Henoch-Schonlein purpura and hemolytic uremic syndrome can cause cramping, abdominal pain, and blood in the stool. Most often, though, it is acute in onset and has other features atypical of IBD. Intestinal lymphoma in its earlier stages may mimic IBD.

Growth failure: Although unusual, the first presentation of IBD may be growth failure alone. The differential diagnosis for a child presenting primarily with poor growth is quite broad.

• Alternative considerations: Celiac disease, thyroid dysfunction, cystic fibrosis, giardia infection, anorexia or other eating disorders, adrenal insufficiency, chronic kidney disease, and malignancy can manifest in this way.

Medical Conditions Causing Condition

IBD that becomes symptomatic or is diagnosed before 6 years of age is termed "very early-onset IBD" (VEO-IBD). Compared with children whose IBD develops later in life, those with VEO-IBD, and particularly those with onset before 2 years of age (sometimes termed "infantile IBD") are more likely to have single gene defects that alter immune function or disturb epithelial barrier function, and they often have a more severe disease course. The disorders include defects in interleukin-10 (IL-10) signaling and can be seen in additional immunodysregulation conditions, such as atypical severe combined immunodeficiency, common variable immunodeficiency, chronic granulomatous disease and other neutrophil defects, Wiskott-Aldrich syndrome, agammaglobulinemia, hyperimmunoglobulin M syndrome, familial hemophagocytic lymphohistiocytosis, and IPEX (immune dysregulation, polyendocrinopathy, enteropathy, X-linked) or other autoimmune enteropathy. [Uhlig: 2014]

Case reports describe apparent ulcerative colitis presenting in infants with genetic mutations of familial Mediterranean fever. [Sari: 2008] Presenting symptoms include bloody diarrhea, failure to thrive, anemia, and leukocytosis. These reports and epidemiologic studies in populations of adults suggest that the genes responsible for familial Mediterranean fever may have a disease-modifying effect on IBD. [Cattan: 2000]

Comorbid & Secondary Conditions

One of the most common extraintestinal manifestations with IBD is musculoskeletal conditions, particularly juvenile arthritis or polyarticular arthritis. These can occur in up to 40-50% of patients with IBD and can often signal an intestinal flare or poor medication response; they sometimes occur as a separate condition even in the presence of intestinal remission. [Malik: 2021] [Levine: 2011] Sacroileitis is present in up to 25% of patients with IBD, particularly those with HLA-B27 positivity.

Hepatobiliary disease can occur in up to 50% of patients with IBD. The most common of these is primary sclerosing cholangitis and conditions that cause liver inflammation and biliary tract fibrosis and scarring. Studies have shown this occurs in >10% of pediatric patients with ulcerative colitis. [Deneau: 2013]

Patients with IBD, particularly UC, can develop primary sclerosing cholangitis (PSC) and are at risk for developing cirrhosis and end-stage liver disease. PSC children have high rates of autoimmune hepatitis. Patients with PSC have higher rates of liver and colon cancer. [Deneau: 2017] For liver cancer, screening with annual CA 19-9 at age 18 and US is recommended.

Other less common secondary conditions (<15%) include cutaneous, ocular as well as vascular inflammatory conditions. Practitioners should have a low threshold for monitoring and screening for any systemic condition in patients with IBD.

Patients on anti-TNF alpha biologics (infliximab, adalimumab) can also have medication-induced autoimmune conditions or complications such as vasculitis, peripheral neuropathy, psoriasis, and temporary joint pains. Many patients have to discontinue medication use due to these conditions, even if their intestines are in remission.

Current & Past Medical History

Current and past history:

• Fecal urgency, especially if child wakes from sleep to have an urgent bowel movement
• Blood or mucus in stools (after constipation has been excluded)
• Growth disturbance
• Pubertal delay
• Fevers, anorexia, malaise, fatigue (signs of systemic inflammation)
• Recurrent oral or genital ulcerations (extraintestinal manifestations)
• Arthritis, especially oligoarticular, large joint, and primarily early-morning (extraintestinal manifestations)
• Rashes (pyoderma gangrenosum or erythema nodosum)
• Eye inflammation (anterior uveitis)

Ask about any side effects of medications. Risk factors for developing IBD may include a history of too many antibiotic prescriptions in youth, whether a patient was delivered via cesarean or vaginal method, chemicals and additives to foods, and the types of bacteria inside the gut.

Family History

Ask about family history; affected relatives increase the likelihood of IBD.

Pregnancy/Perinatal History

The risks of IBD to pregnancy are significant and manifold, including miscarriage, delivery of a small-for-gestational-age infant, premature delivery, poor maternal weight gain, and complications of labor and delivery (e.g., preeclampsia, placental abruption, increased probability of cesarean delivery). [Mahadevan: 2019]

Ideally, a pregnant patient with IBD is monitored by both a gastroenterologist specializing in IBD and an MFM specialist with assistance from nutritionists, lactation counselors, and colorectal surgeons, as needed.

Developmental & Educational Progress

Monitor school progress, which may reflect symptom control—those who miss more than a little school due to exacerbations may have difficulty keeping up. Behavior problems may reflect depression or problems with peers (perhaps due to symptom manifestations at school). Many patients report experiencing “brain fog” or fatigue prior to diagnosis, as they have a high-calorie demand to keep up with ongoing intestinal inflammation. Therefore, an unexpected decrease in grades or poor school performance may be a sign of new-onset IBD.

Maturational Progress

Delayed pubertal development and/or primary amenorrhea can result from chronic inflammation.

Social & Family Functioning

Ask about support from family members. Establishing care with a behavioral health specialist is encouraged as a chronic illness diagnosis can lead to adjustment disorder, anxiety, depressive episode, or evolve into long-term depression for some patients.

Physical Exam

Laboratory Testing

Exclusion of infectious etiology is the first step in the workup for any patient with prolonged diarrhea. GI PCR film array including Clostridium difficile toxin assay, routine stool culture, and a microscopic examination for ova and parasites are generally sufficient in covering the most common organisms with presentations similar to IBD. More cultures may be indicated if there is supportive history.

In children with IBD, hemoglobin and ESR and CRP are the most likely routine blood tests to be abnormal. Elevated white count, and high or low platelets, can be seen. Serum albumin (half-life is about 3 weeks) may be low as a result of prolonged protein-losing enteropathy. A subset of patients with mild to moderate IBD will have normal labs.

Fecal calprotectin has adequate sensitivity and specificity to identify those patients most likely to have organic bowel disease and can help differentiate IBD from functional disease, such as irritable bowel syndrome (IBS). [Walsham: 2016] Recent studies show this test may have less sensitivity in Crohn’s disease than in UC. [Quail: 2009]

Current serologies such as the Prometheus IBD-7 panel lack the sensitivity and specificity to make them useful to distinguish between IBD and other disorders. Antibody panels such as ANCA/ASCA are expensive, lack sufficient positive and negative predictive value, and are not recommended.

Imaging

Imaging of the small bowel may identify lesions suggestive of Crohn's disease. The best radiographic studies are magnetic resonance enterography or CT enterography, which requires NJ tube placement for contrast infusion directly into the small bowel. Magnetic resonance enterography (MRE) is an MRI of the small bowel. It is preferred over CT enterography because there is no radiation exposure to the pediatric patient, and MRE provides real-time visualization of peristalsis, which helps evaluate for possible strictures. Capsule endoscopy, in which the patient swallows a camera pill that can image the GI tract not able to be directly visualized via endoscopy, is available at some centers.

Genetic Testing

Next-generation DNA-sequencing technologies are available to diagnose monogenic causes of IBD. Routine genetic screening is not recommended for all IBD patients. Genetic testing should be considered depending on age of IBD-onset (infantile IBD, very early-onset IBD, pediatric or young adult IBD), and further criteria, such as family history, relevant comorbidities, and extraintestinal manifestations. Genetic testing is also recommended in advance of hematopoietic stem cell transplantation. [Uhlig: 2021]

Other Testing

Antibody testing has limited utility for discriminating between ulcerative colitis and Crohn's disease, and practice guidelines for children and adults are equivocal regarding its use. [Bousvaros: 2007] The most common antibody tests are perinuclear antineutrophil cytoplasmic antibodies (P-ANCA) and anti-saccharomyces cerevisiae antibodies (ASCA). When combined into panels, the antibody tests have reasonably high sensitivities for detecting IBD (>90 percent in populations with symptoms), but their ability to distinguish between ulcerative colitis and Crohn’s disease is not well validated. Thus, antibody testing for IBD does not generally contribute useful information to management decisions.

The anti-OmpC antibody has been identified as a potential serologic marker of IBD. In a study of 198 children, anti-OmpC was detected in 25 percent of patients with Crohn's disease, 11 percent of patients with UC, and 5 percent of controls. Because anti-OmpC was positive in nine children with IBD who were not detected by ASCA (IgA and IgG) or P-ANCA, the addition of anti-OmpC to these antibody assays increased the sensitivity from 63 to 70 percent but decreased the specificity from 97 to 94 percent. [Zholudev: 2004]

Antibodies to the bacterial flagellin CBir1 are found in approximately 50 percent of individuals with Crohn’s disease and have been associated with small bowel, internal-penetrating, and fibrostenosing patterns. [Papadakis: 2007]

Pearls & Alerts for Treatment & Management

Test for tuberculosis before use of biologic agents

Children should be tested for tuberculosis prior to receiving biologic agents (Infliximab and adalimumab monoclonal antibodies), and they should be monitored for opportunistic infections.

Live virus vaccinations

Patients with IBD on immunosuppressive medications are considered to have secondary immunodeficiency due to treatments that can include human immune mediators like interleukins and colony-stimulating factors, immune modulators, and medicines like tumor necrosis factor-alpha inhibitors and anti-B cell agents. The CDC recommends that non-live and live vaccines be administered 2 or more weeks before initiating such therapies. Live vaccines should be withheld 3 months following such therapies, and both non-live and live vaccines should be withheld at least 6 months following therapy with anti-B cell agents. Risks and benefits should always be discussed with families of patients on immunosuppression therapy prior to administration of live vaccines, especially in the context of possible travel outside the United States.

Pneumonia vaccination

Children with IBD should be immunized with 2 doses of pneumococcal polysaccharide vaccine (PPSV23) in addition to pneumococcal conjugate vaccine (PCV13). [Centers: 2022]

COVID-19 vaccination

The COVID vaccinations are safe and effective for patients with IBD, and patients should follow CDC guidelines on immunization booster schedule for immunosuppressed individuals, as their antibody response to vaccines may be altered by their medications.

Surgical emergencies

Complications of IBD, such as perforation, obstructive strictures, and toxic megacolon, are surgical emergencies.

Growth or Weight Gain

Growth failure (subnormal gains in height or weight, or weight loss) and/or delayed puberty are common, especially in those with Crohn's disease. Nonetheless, a substantial number of children are overweight when they present with IBD because overweight and obesity are common in most populations.

Malabsorption is common in IBD, making it difficult to absorb necessary nutrients in the small intestine, such as proteins, fats, sugars, vitamins, and minerals. It can be caused by inflammation in the intestines. The degree of malabsorption depends on how much of the small intestine is affected. Malabsorption and nutrient deficiencies are often more significant if larger sections of the small intestine are inflamed or have been surgically removed. If a significant portion of the ileum or the terminal ileum / ileo-cecal valve is inflamed or removed, the absorption of fat-soluble vitamins A, D, E, K, and B12 will likely be affected. [Crohn’s: 2021]

Therefore, some patients may require both enteral nutrition via a nasogastric (NG) or nasojejunal (NJ) tube and parenteral nutrition at onset of diagnosis or during flares.

Common supplements recommended for IBD patients are a multivitamin that contains calcium, folic acid, iron, zinc, B12, fat-soluble vitamins A, D, E, K.

Patients with ulcerative colitis may have less significant nutrient deficiencies, though severe diarrhea and blood loss can cause weight loss.

A known risk for reduced height in IBD patients includes exposure to 3 months or greater of corticosteroids before puberty. [Wyllie: 2006]

It is encouraged that families have the support of a registered dietician to help with growth and weight gain.

Development (Cognitive, Motor, Language, Social-Emotional)

Studies on development outcomes of patients with IBD are limited. One study compared a Danish population of children exposed to IBD in utero to an unexposed population. At 18 months, exposed children were significantly less likely to "use word-like sounds," but there was no difference between the use of "sentences of 2 words." At 6 and 18 months, there were no other obvious differences in language and motor development. At 7 years, cognitive scores (emotional, conduct, hyperactivity, peer and social scores) and motor development (gross/fine motor skills and general coordination) were similar between the 2 groups. [Friedman: 2020]

Viral Infections

Most cases of viral respiratory and gastrointestinal infections pass normally in children with IBD, even in patients on immunosuppressive medications. Yet, care must be taken with chickenpox or zoster rash exposures in patients on biologic medications: some patients require prophylactic antiviral medicines. Care must also be taken with EBV (mono) infections, which can evolve into serious systemic inflammatory responses in the spectrum of hemophagocytic lymphohistiocytosis.

Gastroenterology should be contacted immediately for exposure to a virus, and patients on immunosuppression should be treated with antivirals for documented positive tests of influenza viruses.

Bacterial Infections

Patients on immunosuppression who develop a chronic cough should be worked up for tuberculosis, histoplasmosis, coccidiomycosis, and blastomycosis, depending on exposure history.

Over the Counter Medications

There is mixed evidence that non-steroidal anti-inflammatory medicines (NSAIDs) like ibuprofen (Advil, Motrin) should be avoided in people with IBD since they may cause disease flares or worsen bleeding symptoms.

Patients with IBD also have an increased incidence of inflammatory bowel syndrome (IBS) symptoms. Many of these patients can treat their dyspepsia or IBS with peppermint oil or ginger extract capsules, stool softeners, and probiotic supplements, for example.

Prescription Medications

Some IBD patients can develop IBS like symptoms, including abdominal pain and nausea, and may benefit from the prescribed medications below:

• Hyoscyamine (Levsin)—0.125 – 0.25mg up to 4 times per day as needed for abdominal pain symptoms
• Cyproheptadine—for morning nausea and appetite stimulation. Can cause or worsen constipation as a side effect

Common Complaints

• Gastrointestinal symptoms—Loose stools or bloody diarrhea, abdominal pain, or cramping
• Systemic symptoms—Fatigue is common at presentation and during flares of disease
• Extraintestinal manifestations—Oral ulcerations (aphthous stomatitis), swollen digits (clubbing), rash (erythema nodosum or pyoderma gangrenosum), eye pain due to eye inflammation (uveitis), yellowing eyes or skin (jaundice), joint pain in one or multiple joints (arthritis)

Systems

On the Web

Inflammatory Bowel Disease (OMIM)
Extensive review of literature that provides technical information on genetic disorders; Online Mendelian Inheritance in Man site, hosted by Johns Hopkins University.

Helpful Articles

PubMed search for inflammatory bowel diseases in children, last 2 years.

Fell JM.
Update of the management of inflammatory bowel disease.
Arch Dis Child. 2012;97(1):78-83. PubMed abstract / Full Text

Hommel KA, Greenley RN, Maddux MH, Gray WN, Mackner LM.
Self-management in pediatric inflammatory bowel disease: A clinical report of the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.
J Pediatr Gastroenterol Nutr. 2013;57(2):250-7. PubMed abstract / Full Text

Leung Y, Heyman MB, Mahadevan U.
Transitioning the adolescent inflammatory bowel disease patient: guidelines for the adult and pediatric gastroenterologist.
Inflamm Bowel Dis. 2011;17(10):2169-73. PubMed abstract / Full Text

Letters of Medical Necessity

Appeal Letters (CCFA)
Templates for requesting school accommodations and appealing denials of funding for medications and procedures; Crohn's & Colitis Foundation of America.

Patient Education & Instructions

Parents' Guide to Inflammatory Bowel Disease (CCFA)
Provides information about diagnosis and treatment, helpful tips for lifestyle changes, and resources for emotional support; Crohn's and Colitis Foundation of America.

Information on the Web

A Guide for Teachers to Inflammatory Bowel Disease (CCFA)
Information for school personnel about the diagnosis, sports participation, and planning for potential school absences; Crohn's and Colitis Foundation of America.

Ulcerative Colitis (MedlinePlus)
Diagnosis and management information; sponsored by the U.S. National Library of Medicine.

Crohn's Disease (MedlinePlus)
Diagnosis and management information; sponsored by the U.S. National Library of Medicine.

Inflammatory Bowel Disease (KidsHealth.com)
Family-focused information about IBD; from the Nemours Foundation.

Caring for your Child with IBD (Johns Hopkins Health Book)
A 304-page resource for the family living with IBD; by the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.

Just Like Me! Teens with IBD (CCFA)
Information for teens with IBD including an ask-the-expert section, a chat room, and "Hot Topics" related to dating, family, friends, and school; Crohn's & Colitis Foundation of America.

National & Local Support

Crohn's & Colitis Foundation of America (CCFA)
Credible disease information with an extensive “kids and teens” section, information about summer camps, and lists of support groups at the local level.

Studies/Registries

Inflammatory Bowel Disease (clincialtrials.gov)
A listing of registries and clinical trials for children with inflammatory bowel disease; National Institutes of Health.

Services for Patients & Families in Rhode Island (RI)

Service Categories	# of providers* in:	RI	NW	Other states (4)  (show)		NM	NV	OH	UT
Developmental - Behavioral Pediatrics		12	1		2	2	2	9
General Pediatric Surgery		4			4	5		2
Nutrition Assessment Services		2				3	1	7
Pediatric Dentistry		41	2		6	31	3	59
Pediatric Gastroenterology		19	1		3	6	1	4
Pediatric Ophthalmology		8	1		6	6	1	5

For services not listed above, browse our Services categories or search our database.

* number of provider listings may vary by how states categorize services, whether providers are listed by organization or individual, how services are organized in the state, and other factors; Nationwide (NW) providers are generally limited to web-based services, provider locator services, and organizations that serve children from across the nation.

Bousvaros A, Antonioli DA, Colletti RB, Dubinsky MC, Glickman JN, Gold BD, Griffiths AM, Jevon GP, Higuchi LM, Hyams JS, Kirschner BS, Kugathasan S, Baldassano RN, Russo PA.
Differentiating ulcerative colitis from Crohn disease in children and young adults: report of a working group of the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition and the Crohn's and Colitis Foundation of America.
J Pediatr Gastroenterol Nutr. 2007;44(5):653-74. PubMed abstract

Cattan D, Notarnicola C, Molinari N, Touitou I.
Inflammatory bowel disease in non-Ashkenazi Jews with familial Mediterranean fever.
Lancet. 2000;355(9201):378-9. PubMed abstract

Centers for Disease Control and Prevention.
Pneumococcal Vaccination: Summary of Who and When to Vaccinate.
National Center for Immunization and Respiratory Diseases; (2022) https://www.cdc.gov/vaccines/vpd/pneumo/hcp/who-when-to-vaccinate.html. Accessed on 2022.

Conklin LS, Oliva-Hemker M.
Nutritional considerations in pediatric inflammatory bowel disease.
Expert Rev Gastroenterol Hepatol. 2010;4(3):305-17. PubMed abstract

Critch J, Day AS, Otley A, King-Moore C, Teitelbaum JE, Shashidhar H.
Use of enteral nutrition for the control of intestinal inflammation in pediatric Crohn disease.
J Pediatr Gastroenterol Nutr. 2012;54(2):298-305. PubMed abstract

Crohn’s & Colitis Foundation.
Crohn’s & Colitis Foundation.
(2021) https://www.crohnscolitisfoundation.org/.

Degraeuwe PL, Beld MP, Ashorn M, Canani RB, Day AS, Diamanti A, Fagerberg UL, Henderson P, Kolho KL, Van de Vijver E, van Rheenen PF, Wilson DC, Kessels AG.
Faecal calprotectin in suspected paediatric inflammatory bowel disease.
J Pediatr Gastroenterol Nutr. 2015;60(3):339-46. PubMed abstract

Deneau M, Jensen MK, Holmen J, Williams MS, Book LS, Guthery SL.
Primary sclerosing cholangitis, autoimmune hepatitis, and overlap in Utah children: epidemiology and natural history.
Hepatology. 2013;58(4):1392-400. PubMed abstract

Deneau MR, El-Matary W, Valentino PL, Abdou R, Alqoaer K, Amin M, Amir AZ, Auth M, Bazerbachi F, Broderick A, Chan A, Cotter J, Doan S, El-Youssef M, Ferrari F, Furuya KN, Gottrand M, Gottrand F, Gupta N, Homan M, Kamath BM, Kim KM, Kolho KL, Konidari A, Koot B, Iorio R, Ledder O, Mack C, Martinez M, Miloh T, Mohan P, O'Cathain N, Papadopoulou A, Ricciuto A, Saubermann L, Sathya P, Shteyer E, Smolka V, Tanaka A, Varier R, Venkat V, Vitola B, Vos MB, Woynarowski M, Yap J, Jensen MK.
The natural history of primary sclerosing cholangitis in 781 children: A multicenter, international collaboration.
Hepatology. 2017;66(2):518-527. PubMed abstract

El-Matary W, Sikora S, Spady D.
Bone Mineral Density, Vitamin D, and Disease Activity in Children Newly Diagnosed with Inflammatory Bowel Disease.
Dig Dis Sci. 2010. PubMed abstract

Fell JM.
Update of the management of inflammatory bowel disease.
Arch Dis Child. 2012;97(1):78-83. PubMed abstract / Full Text

Friedman S, Nielsen J, Jølving LR, Nohr EA, Nørgård BM.
Long-term motor and cognitive function in the children of women with inflammatory bowel disease.
J Crohns Colitis. 2020. PubMed abstract

Halme L, Paavola-Sakki P, Turunen U, Lappalainen M, Farkkila M, Kontula K.
Family and twin studies in inflammatory bowel disease.
World J Gastroenterol. 2006;12(23):3668-72. PubMed abstract / Full Text

Henderson P, Anderson NH, Wilson DC.
The diagnostic accuracy of fecal calprotectin during the investigation of suspected pediatric inflammatory bowel disease: a systematic review and meta-analysis.
Am J Gastroenterol. 2014;109(5):637-45. PubMed abstract

Hommel KA, Greenley RN, Maddux MH, Gray WN, Mackner LM.
Self-management in pediatric inflammatory bowel disease: A clinical report of the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.
J Pediatr Gastroenterol Nutr. 2013;57(2):250-7. PubMed abstract / Full Text

Johnston RD, Logan RF.
What is the peak age for onset of IBD?.
Inflamm Bowel Dis. 2008;14 Suppl 2:S4-5. PubMed abstract

Kappelman MD, Rifas-Shiman SL, Kleinman K, Ollendorf D, Bousvaros A, Grand RJ, Finkelstein JA.
The prevalence and geographic distribution of Crohn's disease and ulcerative colitis in the United States.
Clin Gastroenterol Hepatol. 2007;5(12):1424-9. PubMed abstract

Koninckx CR, Donat E, Benninga MA, Broekaert IJ, Gottrand F, Kolho KL, Lionetti P, Miele E, Orel R, Papadopoulou A, Pienar C, Schäppi MG, Wilschanski M, Thapar N.
The Use of Fecal Calprotectin Testing in Paediatric Disorders: A Position Paper of the European Society for Paediatric Gastroenterology and Nutrition Gastroenterology Committee.
J Pediatr Gastroenterol Nutr. 2021;72(4):617-640. PubMed abstract

Leung Y, Heyman MB, Mahadevan U.
Transitioning the adolescent inflammatory bowel disease patient: guidelines for the adult and pediatric gastroenterologist.
Inflamm Bowel Dis. 2011;17(10):2169-73. PubMed abstract / Full Text

Levine A, Turner D, Pfeffer Gik T, Amil Dias J, Veres G, Shaoul R, Staiano A, Escher J, Kolho KL, Paerregaard A, Martin de Carpi J, Veereman Wauters G, Koletzko S, Shevah O, Finnby L, Sladek M.
Comparison of outcomes parameters for induction of remission in new onset pediatric Crohn's disease: evaluation of the porto IBD group "growth relapse and outcomes with therapy" (GROWTH CD) study.
Inflamm Bowel Dis. 2014;20(2):278-85. PubMed abstract

Levine JS, Burakoff R.
Extraintestinal manifestations of inflammatory bowel disease.
Gastroenterol Hepatol (N Y). 2011;7(4):235-41. PubMed abstract / Full Text

Mackner LM, Whitaker BN, Maddux MH, Thompson S, Hughes-Reid C, Drovetta M, Reed B.
Depression Screening in Pediatric Inflammatory Bowel Disease Clinics: Recommendations and a Toolkit for Implementation.
J Pediatr Gastroenterol Nutr. 2020;70(1):42-47. PubMed abstract / Full Text

Mahadevan U, Robinson C, Bernasko N, Boland B, Chambers C, Dubinsky M, Friedman S, Kane S, Manthey J, Sauberan J, Stone J, Jain R.
Inflammatory Bowel Disease in Pregnancy Clinical Care Pathway: A Report From the American Gastroenterological Association IBD Parenthood Project Working Group.
Gastroenterology. 2019;156(5):1508-1524. PubMed abstract

Malik TF, Aurelio DM.
Extraintestinal Manifestations of Inflammatory Bowel Disease.
StatPearls [Internet]. 2021. PubMed abstract

McGovern DP, Kugathasan S, Cho JH.
Genetics of Inflammatory Bowel Diseases.
Gastroenterology. 2015;149(5):1163-1176.e2. PubMed abstract / Full Text

Minordi LM, Bevere A, Papa A, Larosa L, Manfredi R.
CT and MRI Evaluations in Crohn's Complications: A Guide for the Radiologist.
Acad Radiol. 2021. PubMed abstract

Moran CJ.
Very early onset inflammatory bowel disease.
Semin Pediatr Surg. 2017;26(6):356-359. PubMed abstract

Moriczi M, Pujol-Muncunill G, Martín-Masot R, et al.
Predictors of Response to Exclusive Enteral Nutrition in Newly Diagnosed Crohn´s Disease in Children: PRESENCE Study from SEGHNP.
Nutrients. 2020;12(4). PubMed abstract / Full Text

Papadakis KA, Yang H, Ippoliti A, Mei L, Elson CO, Hershberg RM, Vasiliauskas EA, Fleshner PR, Abreu MT, Taylor K, Landers CJ, Rotter JI, Targan SR.
Anti-flagellin (CBir1) phenotypic and genetic Crohn's disease associations.
Inflamm Bowel Dis. 2007;13(5):524-30. PubMed abstract

Quail MA, Russell RK, Van Limbergen JE, Rogers P, Drummond HE, Wilson DC, Gillett PM.
Fecal calprotectin complements routine laboratory investigations in diagnosing childhood inflammatory bowel disease.
Inflamm Bowel Dis. 2009;15(5):756-9. PubMed abstract

Ruemmele FM, Veres G, Kolho KL, Griffiths A, Levine A, Escher JC, Amil Dias J, Barabino A, Braegger CP, Bronsky J, Buderus S, Martín-de-Carpi J, De Ridder L, Fagerberg UL, Hugot JP, Kierkus J, Kolacek S, Koletzko S, Lionetti P, Miele E, Navas López VM, Paerregaard A, Russell RK, Serban DE, Shaoul R, Van Rheenen P, Veereman G, Weiss B, Wilson D, Dignass A, Eliakim A, Winter H, Turner D.
Consensus guidelines of ECCO/ESPGHAN on the medical management of pediatric Crohn's disease.
J Crohns Colitis. 2014;8(10):1179-207. PubMed abstract / Full Text
Intended to give practical (whenever possible evidence-based) answers to (pediatric) gastroenterologists who take care of children and adolescents with CD; 2014 European Crohn's and Colitis Organisation.

Sari S, Egritas O, Dalgic B.
The familial Mediterranean fever (MEFV) gene may be a modifier factor of inflammatory bowel disease in infancy.
Eur J Pediatr. 2008;167(4):391-3. PubMed abstract

Schaefer ME, Machan JT, Kawatu D, Langton CR, Markowitz J, Crandall W, Mack DR, Evans JS, Pfefferkorn MD, Griffiths AM, Otley AR, Bousvaros A, Kugathasan S, Rosh JR, Keljo DJ, Carvalho RS, Tomer G, Mamula P, Kay MH, Kerzner B, Oliva-Hemker M, Kappelman MD, Saeed SA, Hyams JS, Leleiko NS.
Factors that determine risk for surgery in pediatric patients with crohn's disease.
Clin Gastroenterol Hepatol. 2010;8(9):789-794.e2. PubMed abstract

Schurman JV, Cushing CC, Carpenter E, Christenson K.
Volitional and Accidental Nonadherence to Pediatric Inflammatory Bowel Disease Treatment Plans: Initial Investigation of Associations with Quality of Life and Disease Activity.
J Pediatr Psychol. 2010. PubMed abstract

Szigethy E, McLafferty L, Goyal A.
Inflammatory bowel disease.
Child Adolesc Psychiatr Clin N Am. 2010;19(2):301-18, ix. PubMed abstract

Tabbers MM, DiLorenzo C, Berger MY, Faure C, Langendam MW, Nurko S, Staiano A, Vandenplas Y, Benninga MA.
Evaluation and treatment of functional constipation in infants and children: evidence-based recommendations from ESPGHAN and NASPGHAN.
J Pediatr Gastroenterol Nutr. 2014;58(2):258-74. PubMed abstract / Full Text

Turner D, Levine A, Escher JC, Griffiths AM, Russell RK, Dignass A, Dias JA, Bronsky J, Braegger CP, Cucchiara S, de Ridder L, Fagerberg UL, Hussey S, Hugot JP, Kolacek S, Kolho KL, Lionetti P, Paerregaard A, Potapov A, Rintala R, Serban DE, Staiano A, Sweeny B, Veerman G, Veres G, Wilson DC, Ruemmele FM.
Management of pediatric ulcerative colitis: joint ECCO and ESPGHAN evidence-based consensus guidelines.
J Pediatr Gastroenterol Nutr. 2012;55(3):340-61. PubMed abstract
Forty recommendations regarding initial evaluation, how to monitor disease activity, the role of endoscopic evaluation, medical and surgical therapy, timing and choice of each medication, the role of combined therapy, and when to stop medications. Includes a management flowchart.

Uhlig HH, Charbit-Henrion F, Kotlarz D, Shouval DS, Schwerd T, Strisciuglio C, de Ridder L, van Limbergen J, Macchi M, Snapper SB, Ruemmele FM, Wilson DC, Travis SPL, Griffiths AM, Turner D, Klein C, Muise AM, Russell RK.
Clinical Genomics for the Diagnosis of Monogenic Forms of Inflammatory Bowel Disease: A Position Paper From the Paediatric IBD Porto Group of European Society of Paediatric Gastroenterology, Hepatology and Nutrition.
J Pediatr Gastroenterol Nutr. 2021;72(3):456-473. PubMed abstract / Full Text

Uhlig HH, Schwerd T, Koletzko S, Shah N, Kammermeier J, Elkadri A, Ouahed J, Wilson DC, Travis SP, Turner D, Klein C, Snapper SB, Muise AM.
The diagnostic approach to monogenic very early onset inflammatory bowel disease.
Gastroenterology. 2014;147(5):990-1007.e3. PubMed abstract / Full Text

Walsham NE, Sherwood RA.
Fecal calprotectin in inflammatory bowel disease.
Clin Exp Gastroenterol. 2016;9:21-9. PubMed abstract / Full Text

Wiskin AE, Wootton SA, Hunt TM, Cornelius VR, Afzal NA, Jackson AA, Beattie RM.
Body composition in childhood inflammatory bowel disease.
Clin Nutr. 2010. PubMed abstract

Wong A, Bass D.
Laboratory evaluation of inflammatory bowel disease.
Curr Opin Pediatr. 2008;20(5):566-70. PubMed abstract

Wyllie R, Hyams JS, Kay M.
Pediatric Gastrointestinal and Liver Disease.
3rd ed. Saunders Elsevier; 2006. 0721639240

Zholudev A, Zurakowski D, Young W, Leichtner A, Bousvaros A.
Serologic testing with ANCA, ASCA, and anti-OmpC in children and young adults with Crohn's disease and ulcerative colitis: diagnostic value and correlation with disease phenotype.
Am J Gastroenterol. 2004;99(11):2235-41. PubMed abstract