1000-2000 5 ьшò

– Little to no increased risk in latent celiac disease (seropositive but normal biopsy)

Associated autoimmune conditions (type 1 diabetes, autoimmune thyroiditis, primary biliary cirrhosis, autoimmune hepatitis, psoriasis, Sjögren disease)

Associated genetic conditions (Down syndrome, IgAdeficiency, Turner syndrome, Williams syndrome)

Pregnancy Considerations

Prevalence of celiac disease: 2.5 to 3.5 times higher in women with unexplained infertility

Up to 19% of men with celiac disease have androgen resistance. Semen quality and likelihood of pregnancy increase with gluten-free diet.

Higher rates of low birth weight, prematurity, spontaneous abortions, intrauterine growth restriction, and stillbirths

Pediatric Considerations

Children with CD at higher risk for type 1 diabetes, Down syndrome, Turner syndrome, Williams syndrome, IgAdeficiency, and autoimmune thyroid disease (5)[C]

DIAGNOSIS

HISTORY

Diarrhea, cramping are the most common GI symptoms.

Steatorrhea (fatty stools)

Abdominal pain or distension

Nausea, vomiting, flatulence

Weight loss, weakness, fatigue

Delayed puberty

Iron deficiency anemia

Recurrent aphthous stomatitis

Dental enamel hyperplasia

Muscle cramps

Bone or joint pain

Anxiety, depression

Tingling numbness in hands, feet

Migraines

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In children, malabsorption may manifest as failure to thrive, short stature, or chronic fatigue (5).

Anorexia

Constipation or encopresis

PHYSICALEXAM

Often normal; look for specific findings with:

Oropharynx: aphthous stomatitis

Skin: dermatitis herpetiformis (symmetric erythematous papules and blisters on elbows, knees, buttocks, and back), signs of anemia

Abdomen: distention

DIFFERENTIALDIAGNOSIS

Gluten allergy–type II allergic reaction with signs of anaphylaxis

Nonceliac gluten sensitivity—GI symptoms and/or systemic symptoms improved by gluten-free diet but without biomarkers characteristic of celiac disease

Short bowel syndrome, small intestinal bacterial overgrowth

Lactose intolerance

Dyspepsia

Gastroesophageal reflux disease (GERD)

Pancreatic exocrine insufficiency

Crohn disease

Whipple disease

Tropical sprue

Hypogammaglobulinemia

Intestinal lymphoma

Microscopic colitis

Autoimmune enteropathy

HIV enteropathy

Acute enteritis; radiation enteritis

Eosinophilic gastroenteritis

Giardiasis

IBS

DIAGNOSTIC TESTS & INTERPRETATION

Initial Tests (lab, imaging)

Do not base diagnosis on serology alone. Patients with symptoms highly

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suggestive of celiac disease or those with positive serologies should undergo endoscopy for small bowel biopsy while on a gluten-containing diet. Tissue biopsy is the gold standard for diagnosis.

IgAanti-tTG is the preferred serologic test in patients >2 years (1)[C].

Total serum IgAto screen for IgAdeficiency

ALERT

Positive IgAtTG has high sensitivity and specificity (sensitivity, 95–98%; specificity, 95%) if on normal (non–gluten-free) diet for at least 4 weeks.

IgA-deficient patients have false-negative IgAanti-tTG antibodies.

IgAdeficiency is 10 to 15 times more prevalent in patients with celiac disease.

The tTG antibody test is the preferred test (over the deamidated gliadin peptide [DGP] antibody).

Follow-Up Tests & Special Considerations

If patient is IgAdeficient OR if IgAanti-tTG are negative, follow up with anti-DGPIgAand IgG.

– Sensitivity, 94%; specificity, 99% (~anti-TTG)

Do not use HLADQ serotyping for initial diagnosis. Consider if discrepant serology–histology results in patients unable to test on gluten-free diet and children with Down syndrome (1)[C].

Consider bone mineral density testing at diagnosis and after 1 year if osteopenia/osteoporosis on initial testing or 2 years if normal initially and patient still symptomatic or noncompliant.

Pediatric Considerations

Screen symptomatic pediatric patients with serologic testing as above; may screen asymptomatic patients in at-risk category after 3 years of age, after a year of regular exposure to gluten

–IgAantibody to endomysium (EMA) can be used but adds cost and risk of interpretation error.

–Antigliadin antibody tests (AGAIgAand AGAIgG) are inferior and not recommended.

In IgA-deficient individuals, compare TTG IgAwith quantitative serum IgA and obtain TTG IgG.

Confirm suspected diagnoses with intestinal biopsy.

Biopsy seronegative patients with chronic diarrhea if at risk for false negative, including children <2 years, positive family history of CD, or IgAdeficiency

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(5).

Diagnostic Procedures/Other

Endoscopy with a minimum of four biopsies of distal duodenum and two of duodenal bulb at time of initial evaluation correctly diagnosed 95% of children (1)[C].

Video capsule endoscopy is a promising alternative with a sensitivity and specificity of 80% and 95%; particularly helpful if antibody screening and clinical picture are consistent with celiac disease despite nondiagnostic duodenal biopsies

Test Interpretation

Small-bowel biopsy

Villous atrophy, hyperplasia and lengthening of crypts, infiltration of plasma cells, and intraepithelial lymphocytosis in lamina propria

Villous atrophy also caused by Crohn disease, radiation enteritis, giardia, and other food intolerances

TREATMENT

GENERALMEASURES

Remove gluten from the diet.

–Rice, corn, and nut flour are safe and palatable substitutes (1)[C].

–Grains: uncontaminated oats, rice, corn, tapioca, quinoa, amaranth, sorghum

Levels of IgAantigliadin normalize with gluten abstinence.

Lifelong abstinence is required; immune response to gluten will recur with resumption of gluten intake.

MEDICATION

First Line

Usually no medications: Gluten-free diet is the treatment.

Second Line

In refractory disease, consider

– Steroids (prednisone (1)[C] or budesonide (1)[B])

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–Azathioprine (used with caution; use may lead to lymphoma) (1)[C]

–Cyclosporine

–Infliximab

–Cladribine

Depending on disease severity, patients may develop nutritional deficiencies that require appropriate supplementation.

ISSUES FOR REFERRAL

Additional nutritional support with qualified dietitian

Refractory celiac disease

Child with positive celiac serology

COMPLEMENTARY& ALTERNATIVE MEDICINE

Many alternative therapies are in development, and future treatment may include predigestion of gluten with peptidase, tight junction blockade (zonulin), transglutaminase 2 or HLADQ2/DQ8 blockers, and immune tolerance induction (NexVax) (4).

ONGOING CARE

FOLLOW-UPRECOMMENDATIONS

Consultation with registered dietitian

Screen for osteoporosis and treat accordingly.

Follow-up with GI at 3 to 6 months for serology and 12 months for repeat biopsy if indicated

Patient Monitoring

Repeat EGD if no clinical response to gluten-free diet or relapse in symptoms (1)[C].

Follow anti-tTG IgAor deaminated antigliadin antibodies as a measure of response/compliance with diet (vs. antigliadin IgAor IgG).

DIET

Remove gluten: wheat, rye, barley, and products with gluten additives (processed food/meat, medications, hygiene products).

Dietary change is challenging (especially learning sources of “hidden” gluten)

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and should be coordinated with a skilled registered dietitian.

PATIENT EDUCATION

Discuss how to recognize gluten in various products.

Highlight potential complications and outcomes of failing to follow a glutenfree diet.

Suggest support groups and self-education.

Celiac Disease Foundation: https://www.celiac.org/; Quick start gluten-free diet guide for celiac disease and non-celiac gluten sensitivity. http://celiac.org/wp-content/uploads/2013/12/quick-start-guide.pdf

Celiac Sprue Association (CSA): http://www.csa.celiacs.org

PROGNOSIS

Good prognosis if adherent to gluten-free diet

Patients should see improvement within 7 days of dietary modification.

Symptoms usually resolve in 4 to 6 weeks.

It is unknown whether strict dietary adherence decreases cancer risk.

COMPLICATIONS

Malignancy: Untreated and refractory patients have increased cancer risk, but successful treatment decreases risk to population baseline (1)[C]. Refractory disease (rare ~1–2% of all patients)

–May respond to prednisone

–May need total parenteral nutrition

Osteoporosis

Dehydration

Electrolyte depletion

REFERENCES

1.Rubio-Tapia A, Hill ID, Kelly CP, et al; for American College of Gastroenterology. ACG clinical guidelines: diagnosis and management of celiac disease. Am J Gastroenterol. 2013;108(5):656–676.

2.Riddle MS, Murray JA, Porter CK. The incidence and risk of celiac disease in a healthy US adult population. Am J Gastroenterol. 2012;107(8):1248–1255.

3.Rubio-Tapia A, Ludvigsson JF, Brantner TL, et al. The prevalence of celiac disease in the United States. Am J Gastroenterol. 2012;107(10):1538–1544.

4.Freeman H. Celiac disease: a disorder emerging from antiquity, its evolving classification and risk, and potential new treatment paradigms. Gut Liver.

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2015;9(1):28–37.

5.Hill ID, Dirks MH, Liptak GS, et al; for North American Society for Pediatric Gastroenterology, Hepatology and Nutrition. Guideline for the diagnosis and treatment of celiac disease in children: recommendations of the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition. J Pediatr Gastroenterol Nutr. 2005;40(1):1–19.

ADDITIONALREADING

Husby S, Koletzko S, Korponay-Szabó IR, et al; for ESPGHAN Gastroenterology Committee, European Society for Pediatric Gastroenterology, Hepatology, and Nutrition. European Society for Pediatric Gastroenterology, Hepatology, and Nutrition guidelines for the diagnosis of coeliac disease. J Pediatr Gastroenterol Nutr. 2012;54(1):136–160.

Pinto-Sánchez MI, Verdu EF, Liu E, et al. Gluten introduction to infant feeding and risk of celiac disease: systematic review and meta-analysis. J Pediatr. 2016;168:132–143.

Rozenberg O, Lerner A, Pacht A, et al. Anovel algorithm for the diagnosis of celiac disease and a comprehensive review of celiac disease diagnostics. Clin Rev Allergy Immunol. 2012;42(3):331–341.

SEE ALSO

Algorithms: Diarrhea, Chronic; Malabsorption Syndrome

CODES

ICD10

K90.0 Celiac disease

CLINICALPEARLS

Screen for celiac disease in patients with non-specific GI symptoms, presumed IBS, dermatitis herpetiformis, unexplained transaminitis, or unexplained iron deficiency anemia.

Test total IgAlevels along with IgAanti-tTG antibodies in patients >2 years of age. Positive serology is not definitive.

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Diagnostic testing must be completed while on a gluten-containing diet.

Endoscopic biopsy is the gold standard for diagnosis.

Standard of treatment is a gluten-free diet. Patient symptoms should improve in 7 days if fully compliant.

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CELLULITIS

Jowhara Al-Qahtani, MD Amer Homsi, MD Bakr

Nour, MD, PhD, FACS

BASICS

Acommon global health burden with >650,000 admissions per year in the United States alone (1)[A]

DESCRIPTION

An acute bacterial infection of the dermis and subcutaneous (SC) tissue Types and locations:

–Periorbital cellulitis: bacterial infection of the eyelid and surrounding tissues (anterior compartment)

–Orbital cellulitis: infection of the eye posterior to the septum; sinusitis is the most common risk factor.

–Facial cellulitis: preceded by upper respiratory infection or otitis media

–Buccal cellulitis: infection of cheek in children associated with bacteremia (common before Haemophilus influenzae type B vaccine)

–Peritonsillar cellulitis: common in children; associated with fever, sore throat, and “hot potato” speech

–Abdominal wall cellulitis: common in morbidly obese patients

–Perianal cellulitis: sharply demarcated, bright, perianal erythema

–Necrotizing cellulitis: gas-producing bacteria in the lower extremities; common in diabetics

EPIDEMIOLOGY

Predominant sex: male = female

All-cause mortality for patient admitted with cellulitis is 7%. Recurrence rate of cellulitis is 8–20% (2)[A].

Incidence

200/100,000 patient/years

Prevalence

The exact prevalence is uncertain as cellulitis is common and not reportable. It

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affects all age groups and all races; however, certain types of cellulitis/microorganisms occur in certain populations.

In the United States, ~14.5 million annual cases of cellulitis account for $3.7 billion in ambulatory costs (1)[A].

ETIOLOGYAND PATHOPHYSIOLOGY

Cellulitis is caused by bacterial penetration through a break in the skin. Hyaluronidase mediates SC spread.

Microbiology

–β-Hemolytic streptococci (groups A, B, C, G, and F), Staphylococcus aureus, including MRSAand gram-negative aerobic bacilli, are the most common.

–S. aureus seen in periorbital and orbital cellulitis and IV drug users

–Pseudomonas aeruginosa seen in diabetics and other immunocompromised patients

–H. influenza causes buccal cellulitis.

–Clostridia and non–spore-forming anaerobes: necrotizing cellulitis (crepitant/gangrenous)

–Streptococcus agalactiae: cellulitis following lymph node dissection

–Pasteurella multocida and Capnocytophaga canimorsus: cellulitis preceded by bites

–Streptococcus iniae: immunocompromised hosts

–Rare causes: Mycobacterium, fungal (mucormycosis, aspergillosis, syphilis)

Genetics

No genetic pattern

RISK FACTORS

Disruption of skin barrier: trauma, infection, insect bites, injection drug use, body piercing

Inflammation: eczema or radiation therapy

Edema due to venous insufficiency; lymphatic obstruction due to surgery or congestive heart failure

Elderly, diabetes, hypertension, obesity Recurrent cellulitis:

–Cellulitis recurrence score (predicts recurrence of lower extremity cellulitis based on presence of lymphedema, chronic venous insufficiency, peripheral vascular disease, and deep venous thrombosis) (3)[A]

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