1. A Diagnosis of Eosinophilic Esophagitis in a Toddler with Cystic Fibrosis
Rebecca Wukovich, M.D. Fellow, Pediatric Pulmonology
University Hospitals Cleveland Medical Center

2. There are no relationships to disclose related to this presentation
Presenter Disclosure
There are no relationships to disclose related to this presentation

3. Case Presentation
16 mos. M with cystic fibrosis (F508del/G542X) admitted for cystic fibrosis exacerbation and weight loss (weight 11%ile)
Presenting History
Pancreatic insufficiency
Failure to thrive
Reflux, constipation, oral aversion
Throat cx: E. coli, Enterobacter, P. aeruginosa
Sweat chloride 111, 123
Initial visit weight at 6%ile. Increased to 33%ile at 6 months of age. Over next 10 months had a few measurements of weight loss, mainly poor weight gain. Weight %ile was teens-20s but ultimately at 11%ile at admission.
10mos weight was down to 19%ile and 11%ile at admission at 16mos of age.
Noted over time to have frequent spit-ups, vomiting, eventually developed gagging on foods and avoidance of solid foods around a year of age.
Pseudomonas was isolated on 3 consecutive throat cx starting at 13 mos of age (3/28, 5/9, 6/27)

4. Treatment timeline
-Started at initial visit on enzymes and salt supplementation
-Started on ranitidine just before 3 moths. When ranitidine started- consider Upper GI for vomiting/spit-up if not improved on meds. Did improve briefly but throughout first year continued to have recurrent symptoms of spit-up, abdominal distention, gassiness that would briefly respond to changing ranitidine doses.
-Started shortly after 3 months on duocal to add calories. At 5 months started adding butter. Rec starting solids at 5 months. 10 months of age switched to pediasure
-Around 13 months noted to be gagging and spitting up solid foods. Speech/OT referral. Also grew classic pseudomonas for the first time on throat cx.

5. Malnutrition in Cystic Fibrosis
Increased Losses
Inadequate Intake
Increased Needs
Inadequate intake- inappropriate food intake (juices), picky eater/dysfunctional feeding, GERD, Food scarcity, food allergy
Inadequate absorption/increased losses: malabsorption (CF), vomiting, celiac disease
Increased requirements- chronic diseases, chronic or recurrent system infection/chronic respiratory insufficiency, congenital heart disease, food allergies
CF infant nutritional guidelines recommend evaluation for co-morbid conditions in pts with failure to gain weight: GERD, CFRD, hepatobiliary disease, infectious enteritis, bacterial overgrowth, Crohn’s dz, celiac dz, ineffective pancreatic enzyme dosing; behavioral evaluation

6. Evaluation Labs: Bronchoscopy + Lavage
Electrolytes, LFTs, CRP, IgE- normal
Peripheral eosinophils: 10.6% (0.41x10E3/µL)
Bronchoscopy + Lavage
Cx: MRSA
Cytology: Neutrophils 18% and 41%. No Eos.

7. Evaluation
Endoscopy
Furrows
Up to 85 eos/HPF in mid esophagus

8. Eosinophilic Esophagitis
Immune-mediated reaction to allergens
First described as allergic disease in 1995
Increasing prevalence: cases/100,000
Associated with other disorders
Asthma
Atopic dermatitis
Celiac disease
Connective tissue disorder

9. Symptoms Infancy Childhood Adolescence Failure to thrive
Feeding dysfunction
Vomiting
Abdominal pain
Dysphagia
Odynophagia
Food Impaction
Failure to thrive- poor weight gain or weight loss
Feeding dysfunction: failure to develop normal eating patterns (not advancing past liquids) or adopting coping strategies (refusing solids after eating them previously)

10. Diagnosis Symptoms of esophageal dysfunction
Eosinophilic inflammation: ≥15 eos/HPF
limited to esophagus
persists despite PPI trial
Response to treatment supports diagnosis

11. Treatment Topical Steroids Systemic Steroids Dietary Adjustments
Fluticasone MDI, Swallowed
Oral viscous budesonide
Systemic Steroids
Dietary Adjustments
Targeted elimination diet
6 food elimination diet
Elemental or amino-acid based formula
Endpoints of therapy of EoE include improvements in clinical symptoms and esophageal eosinophilic inflammation- studies assessing treatment use variable definitions of response (1 eos/HPF to 0-6 eos/HPF
Systemic Steroids- typically reserved for times when topical steroids are not effective or patients need a rapid improvement in symptoms.
Targeted elimination- guide by allergy testing, typically skin prick or patch testing
6 food elimination- removing the six most common known food groups that are triggers of EoE: soy, egg, milk, wheat, nuts, and seafood.

12. Follow Up Brief improvement in PO intake
Weight loss following hospitalization (9%ile)
G-tube placed  weight increased to 26%ile
Improved endoscopic findings: peak 35 eos/HPF

13. Discussion Points
Eosinophilic disorders are not limited to children with atopy
Atypical symptoms may suggest alternate etiologies
Failure to respond to interventions should prompt further evaluation

14. Acknowledgements
Cystic Fibrosis Foundation for support from a Third Year Clinical Fellowship Grant

15. References Pathpedia.com
Dellon, ES, Gonsalves, N., Hirano, I., Furuta, GT, Liacouras, C., & Katzka, DA. ACG Clinical Guideline: Evidenced Based Approach to the Diagnosis and Management of Esophageal Eosinophilia and Eosinophilic Esophagitis. Am J Gastroenterol 2014; 108:
Dellon, ES. Epidemiology of Eosinophilic Esophagitis. Gastroenterol Clin N Am 2014; 43: 201–218
Contreras, EM, and Gupta, SK. Steroids in Pediatric Eosinophilic Esophagitis. Gastroenterol Clin N Am 2014; 43:
Akhondi, Hossein. Diagnostic approaches and treatment of eosinophilic esophagitis. A review article. Annals of Medicine and Surgery 2017; 20: 69-73
Bonis, PL, and Furuta, GT. “Clinical manifestations and diagnosis of eosinophilic esophagitis.” Oct. 2016, esophagitis&selectedTitle=1~150.
Soon IS, Butzner JD, Kaplan GG, deBruyn JC. Incidence and prevalence of eosinophilic esophagitis in children. J Pediatr Gastroenterol Nutr. 2013;57(1):72.
Borowitz D, Baker RD, Stallings V. Consensus Report on Nutrition for Pediatric Patients With Cystic Fibrosis. J Pediatr Gastroenterol Nutr 2002; 35:
Koletzko S and Reinhardt D. Nutritional challenges of infants with cystic fibrosis. Early Human Development 2001 S53–S61