Back, Joint, and Extremity Pain - Case 5-4: 16-Year-Old Girl
I. History of Present Illness
A 16-year-old girl was admitted with joint pain and a 35-pound weight loss over
the preceding 7 months. After completion of her gymnastics season 7 months
before admission, she had noticed decreased energy and stiff, slightly swollen
peripheral joints bilaterally, including elbows, wrists, knees, and ankles. She
was diagnosed with juvenile rheumatoid arthritis and treated with naproxen, a
nonsteroidal antiinflammatory drug (NSAID). Her pain improved slightly. Shortly
after starting naproxen, she began having daily episodes of epistaxis that
required four to five facial tissues to control the bleeding. Five months
before admission, she changed from naproxen to ibuprofen without significant
change in the degree of joint pain.
Three months before admission, she noticed a change in her bowel habits, from
two to three stools per week to daily stools that were frequently mixed with
blood. One month before admission, she developed intermittent cramping
abdominal pain. She continued to have episodes of epistaxis and was treated
with fluticasone nasal spray and an oral antihistamine for presumed allergic
rhinosinusitis. Her weight decreased from 148 pounds to 113 pounds. She
complained of decreased appetite and decreased activity level over the
preceding few months. There were no fevers, flank tenderness, dysuria, urgency,
or frequency. There was no change in mood or intentional weight loss. There was
no change in her menstrual cycle. She had not traveled recently.
II. Past Medical History
She had not previously required hospitalization. Menarche occurred at 11 years
of age. Her periods were regular. There were no other medical problems. Her
only medications were ibuprofen, fluticasone nasal spray, and oral
antihistamines as previously mentioned. There was a family history of
hypertension in older relatives.
III. Physical Examination
T, 35.8°C; RR, 18/min; HR, 93 bpm; BP, 123/66 mm Hg
Weight, 40 kg; Height, 162 cm (50th percentile); weight-for-height ratio, less
than 5th percentile
Physical examination revealed a thin girl. Her palpebral conjunctivae were
slightly pale. There were several superficial but actively bleeding erosions on
the left medial nasal septum. There were no oral ulcers. Heart and lung sounds
were normal. The abdomen was soft with mild right lower-quadrant tenderness to
palpation. There were no peritoneal signs. Bright red blood mixed with stool
was detected on rectal examination. There was a small left knee effusion and
bilateral ankle effusions. All joints had a normal range of motion.
IV. Diagnostic Studies
Complete blood count revealed 8,900 WBCs/mm3; hemoglobin, 9.6 mg/dL; and 463,000 platelets/mm3. MCV was 70 fL. The reticulocyte count was 1.5%. ESR was 89 mm/hour.
Prothrombin time, partial thromboplastin time, and serum transaminases were
normal. Serum albumin was 3.0 mg/dL. Urine pregnancy test was negative. There
were no red blood cells (RBCs) or WBCs on urinanalysis. Stool was sent for
bacterial culture, ova and parasite examination, and
Clostridium difficile toxin detection. Abdominal radiography revealed stool in the rectal vault.
V. Course of Illness
An upper gastrointestinal barium study with small-bowel follow-through of
contrast revealed the diagnosis (Fig. 5-5).
Discussion: Case 5-4
I. Differential Diagnosis
In an adolescent, hematochezia with cramping abdominal pain has several
potential causes. Patients with chronic NSAID use often develop
gastrointestinal tract ulceration, although gastric and duodenal ulcers
typically result in melena rather than bright red blood. Infectious
enterocolitis may be caused by
Salmonella spp., Shigella spp., Campylobacter jejuni, enteroinvasive and enterohemorrhagic E. coli (including E. coli O157:H7), Yersinia enterocolitica, and C. difficile. Parasitic causes include Entamoeba histolytica, Cryptosporidium parvum, Schistosoma, and Strongyloides stercoralis. Exposure to undercooked meat and clusters of patients with similar symptoms
suggest a common infectious source. Although 35% to 90% of patients with
E. coli O157:H7 infection develop bloody diarrhea, only 10% progress to
hemolytic-uremic syndrome. The absence of a pertinent travel history makes some
of the parasitic diseases less likely. Proctitis can be caused by
Neisseria gonorrhoeae, herpes simplex virus, or Treponema pallidum. Henoch-Schönlein purpura (HSP) may manifest with bloody diarrhea. Vascular malformations of
the gastrointestinal tract often manifest with recurrent melena or
hematochezia. Eosinophilic gastroenteropathy, a chronic, relapsing disorder
characterized by eosinophilic inflammatory gastrointestinal tract infiltrate,
often manifests with abdominal pain and rectal bleeding. Abdominal complaints,
including abdominal pain due to ileocecal ulcerations, are seen in up to 15% of
patients with Beh
çet's disease. Both Crohn's disease and ulcerative colitis commonly present with abdominal pain and lower
gastrointestinal tract bleeding.
Joint involvement occurs in some of the previously mentioned conditions.
Reactive arthritis can be associated with
Campylobacter enteritis as well as with Salmonella, Shigella, and Yersinia enteritis. However, the arthritis usually begins 1 to 6 weeks after the onset of
diarrhea and resolves within 3 weeks. Arthritis or arthralgias occur in 65% to
85% of children with HSP. Though HSP may recur, prolonged, unremitting symptoms
without rash or nephritis are unusual. Children with Beh
çet's disease usually have recurrent oral ulcers, genital ulcers, and iritis or
uveitis in addition to the joint findings. Arthritis may be seen in 10% to 15%
of children with Crohn
's disease or ulcerative colitis.
II. Diagnosis
Colonoscopy revealed linear ulcerations and luminal edema in the ascending
colon. During the gastrointestinal barium study, the contrast agent pursued a
normal course through the duodenum, jejunum, and proximal ileum. However, only
a thin line of barium connected the ileum to the cecum (Kantor string sign),
indicating significant terminal ileal edema (see Fig. 5-5). Severe mucosal
irregularity was noted in the distal ileum and ascending colon.
These radiologic findings, combined with the presence of bloody stool,
arthritis, nasal ulceration, anemia, and elevated ESR, strongly suggested Crohn
's disease. She was treated with oral sulfasalazine, intravenous methylprednisolone, bowel
rest, and parenteral nutrition support. Her symptoms improved over the course
of 1 week. Three months later, her weight had increased to 140 pounds.
III. Incidence and Epidemiology of Crohn's Disease.
Crohn's disease, a major form of chronic intestinal inflammation, can segmentally
involve any part of the gastrointestinal tract, from the esophagus to the
colon. The inflammation involves the terminal portion of the ileum in
approximately 90% of cases. Inflammation occurs in the ileum and colon together
in 60% of cases, and the upper portion of the gastrointestinal tract is
involved in approximately 30% of cases. In contrast, inflammation in ulcerative
colitis begins in the rectum and extends continuously into the colon but does
not involve more proximal portions of the gastrointestinal tract. Isolated
colonic involvement occurs in 10% of cases of Crohn
's disease, making distinction from ulcerative colitis difficult in some cases.
The prevalence of Crohn's disease in North America ranges from 26 to 198 cases per 100,000 persons, with
higher rates occurring in the more northern latitudes. Crohn
's disease is most common among Caucasians and least common among Hispanics and
Asian-Americans. Peak incidence occurs in young adulthood, and a second,
smaller peak occurs during the sixth decade of life. Approximately 15% of
patients with Crohn
's disease are diagnosed during childhood. The etiology of Crohn's disease is not known but probably involves a combination of environmental,
genetic, and immunoregulatory factors. When the diagnosis of either Crohn
's disease or ulcerative colitis is made, the likelihood of finding inflammatory
bowel disease in a first-degree relative is 10% to 25%.
IV. Clinical Presentation
Approximately 80% of children with Crohn's disease present with abdominal pain, diarrhea, anorexia, and weight loss with
or without extraintestinal manifestations. Recurrent oral ulcers are common.
Abdominal pain in the right lower quadrant suggests ileocecal involvement,
epigastric pain suggests gastroduodenal involvement, and periumbilical pain
suggests generalized small-bowel disease. Fifty percent of children have gross
or microscopic blood in the stool. Perirectal disease (e.g., fissures,
fistulas, skin tags, abscesses) are present in up to 40% of patients.
Extraintestinal manifestations predominate in 8% to 10% of patients and are
likely to be associated with diagnostic confusion and delay (Table 5-3).
Although more than 100 localized extraintestinal manifestations have been
described, the most common are joint complaints (including arthritis), which
occur in 15% to 30% of cases. Approximately 50% of children with Crohn
's disease and peripheral arthritis develop ocular or skin findings (see Table
5-3). Sclerosing cholangitis develops in 1% of children with Crohn
's disease. Symptoms of sclerosing cholangitis include jaundice, generalized
pruritus, and abdominal pain. Pancreatitis occurs as an extraintestinal
manifestation of Crohn
's disease but can also occur as a complication of duodenal involvement,
sclerosing cholangitis, or drug therapy. Renal stones complicating Crohn
's disease may be due to calcium oxalate, calcium phosphate, or uric acid.
Erythema nodosum tends to occur when intestinal disease is active, but it does
not correlate with disease severity. Rashes due to trace mineral deficiencies
may occur as a consequence of malabsorption.
V. Diagnostic Approach
Initial screening tests should include a complete blood count, ESR, liver
function tests, and stool testing for blood, bacteria, and parasites. The
results of the initial screening determine the need for further testing.
Complete blood count. Anemia is present in 40% to 70% of patients. MCV may be low due to the
combination of iron deficiency and chronic inflammation. Vitamin B
12 and folate deficiencies also contribute to the anemia. The WBC count is
typically normal. Thrombocytosis occurs in more than half of patients.
Erythrocyte sedimentation rate. ESR, an acute phase reactant, is elevated in up to 70% of patients, but a normal
ESR does not preclude the diagnosis of Crohn
's disease.
Tests of liver function. Serum transaminases and γ-glutamyltransferase (GGT) may be mildly elevated in the absence of
complications. Crohn
's-related complications, such as sclerosing cholangitis and chronic active
hepatitis, lead to more significant elevation of the transaminases and GGT.
Hypoalbuminemia reflects compromised nutritional status and diffuse
inflammation.
Stool studies. Stool bacterial culture, ova and parasite examination, and C. difficile detection should be performed in all children with bloody diarrhea and suspected
Crohn
's disease, to exclude other causes.
Endoscopic evaluation. Endoscopic evaluation of the colon should precede barium radiography in the
presence of bloody diarrhea. Colonic mucosa biopsy, even if the mucosa appears
normal, is required, because microscopic inflammation with granuloma formation
may be present. If possible, the examination should extend to the terminal
ileum. Esophagogastroduodenoscopy may be required to assess upper
gastrointestinal tract symptoms.
Upper gastrointestinal tract barium study with small-bowel follow-through. This study is important early in the evaluation, because the terminal ileum is
involved in 90% of patients with Crohn
's disease. Features suggestive of Crohn's include terminal ileal thickening (Kantor string sign), nodularity, ulcers,
and fistulous connections.
Other studies. The following studies may be useful in assessing the patient's nutritional status: folic acid, vitamin B12, fat-soluble vitamins (especially vitamin D), prothrombin time, partial
thromboplastin time, zinc, iron, total iron-binding capacity, calcium,
magnesium, phosphorus, and prealbumin.
VI. Treatment
Therapeutic strategies include a combination of medical and surgical
interventions. Medical therapy includes 5-aminosalicylates, corticosteroids,
immunomodulators, and antibiotics. Oral sulfasalazine consists of
5-aminosalicylic acid (5-ASA) bound to sulfapyridine. The sulfa moiety
functions as a carrier, facilitating delivery of the agent to the colon, where
it is cleaved by resident bacteria into therapeutically active 5-ASA. The 5-ASA
decreases colon inflammation by inhibiting leukotriene synthesis via the
lipoxygenase pathway of arachidonic acid metabolism. It also decreases
neutrophil-mediated tissue damage by interfering with myeloperoxidase activity
and scavenging reactive oxygen species. Newer oral 5-ASA analogues (e.g.,
mesalamine) function by either pH-dependent or timed-release mechanisms that
allow the drug to be distributed throughout the small bowel.
Corticosteroids continue to be the mainstay of treatment. They exert
antiinflammatory effects by decreasing cytokine release, capillary
permeability, and neutrophil and monocyte function. Newer corticosteroids have
a strong affinity for intestinal steroid receptors, leading to high topical
antiinflammatory potency. Because they are rapidly transformed into inactivated
metabolites by the liver after absorption, they cause fewer systemic side
effects. For example, budesonide binds to intestinal receptors 15 times more
efficiently than prednisolone but undergoes rapid hepatic metabolism, so
systemic bioavailability is only 10%, compared with 80% for prednisolone.
Delayed-release (time- and pH-dependent) formulations of budesonide permit more
effective delivery of the drug to the terminal ileum and proximal colon.
Immunomodulators are being used to target the inflammatory response more
selectively. For example, tumor necrosis factor-
α (TNF-α), a cytokine, activates components of the immune system involved in Crohn's disease. Infliximab, a chimeric (mouse-human) anti-TNF-α immunoglobulin G antibody, binds to TNF-α and neutralizes its activity. Infliximab infusions administered at 4- to 12-week
intervals induce remissions in patients with moderate to severe Crohn
's disease and facilitate healing of fistulas. Infliximab also permits
significant reduction in steroid use in children.
The antibiotic metronidazole may reduce Crohn's disease activity. It has also been used to treat perianal fistulas and
abscesses. Indications for bowel resection include intractable disease, severe
fistula formation, uncontrolled hemorrhage, and bowel perforation. More than
50% of children with Crohn
's disease require intestinal surgery within 10 to 15 years after diagnosis. The
disease is not usually limited to one portion of the gastrointestinal tract, so
surgery is not curative. Recurrent disease after bowel resection is common.
The course of Crohn's disease is characterized by periods of symptom exacerbation and remission.
Only 1% of children with well-documented Crohn
's disease will have no relapses after diagnosis and initial therapy. Many
children with Crohn
's disease also suffer from medication- and central venous catheter–related complications. In the long term, impaired height velocity is common.
Approximately 8% of patients with severe Crohn
's disease develop colorectal cancer. Death from Crohn's disease in childhood is rare; however, the risk of death is 1.5 times higher
in affected adults compared with age-matched controls.
VII. References
1. Hyams JS. Inflammatory bowel disease. Pediatr Rev 2000;21:291–295.
2. Hyams JS. Extraintestinal manifestations of inflammatory bowel disease in
children.
J Pediatr Gastroenterol Nutr 1994;19:7–21.
3. Hyams JS. Inflammatory bowel disease. In: Altschuler SM, Liacouras CA, eds. Clinical pediatric gastroenterology. Philadelphia: Churchill Livingstone, 1998:213–221.
4. Mamula P, Telega GW, Markowitz JE, et al. Inflammatory bowel disease in
children 5 years of age and younger.
Am J Gastroenterol 2002;97:2005–2010.
5. Stephens MC, Shepanski MA, Mamula P, et al. Safety and steroid-sparing
experience using infliximab for Crohn
's disease at a pediatric inflammatory bowel disease center. Am J Gastroenterol 2003;98:104–111.
6. Thomas DW, Sinatra FR. Screening laboratory tests for Crohn's disease. West J Med 1989;150:163–164.
Pictures
Book Source Details
- Book Title: Pediatric Complaints and Diagnostic Dilemmas
- Author(s): Samir S Shah MD; Stephen Ludwig MD
- Year of Publication: 2003
- Copyright Details: Pediatric Complaints and Diagnostic Dilemmas, Copyright © 2003 Lippincott Williams & Wilkins.
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Copyright Details: Pediatric Complaints and Diagnostic Dilemmas, Copyright © 2008 Williams & Wilkins.
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