Letter to the Editor
To the Editor:
The pathogenesis of juvenile polyposis syndrome (JPS) and associated manifestations (telangiectasias, congenital heart disease, cleft lip/palate) is unknown; however, genetics (BMPR1A and SMAD4) and lifestyle have been implicated (1). A Canadian report showed hyperplastic-inflammatory polyps in the esophagus of a patient with long-standing eosinophilic esophagitis (EoE) (2). We performed an upper endoscopy on a patient with JPS, which was revealing for EoE. Upon review of the literature, an association between these 2 entities may exist.
JPS and EoE share a predilection for boys (3) and those with a personal or family history of allergy (4). Because nasal polyps share many histological similarities with juvenile polyps and are believed to be allergic manifestations, studies have suggested that juvenile polyps represent a response of the gastrointestinal system to an allergic stimulus leading to increased eosinophil-dominant inflammation (4). This same mechanism is thought to occur in patients with EoE.
Our patient tested negative for BMPR1A and SMAD4 gene mutations; however, microarray revealed a deletion within the serine/threonine kinase 10 gene. Its gene product affects cellular functions such as growth, apoptosis, and morphogenesis (5) and is closely related to the serine/threonine kinase 11 gene seen in Peutz-Jeghers syndrome. Protein tyrosine kinases play an integral role in the activation of inflammatory and airway smooth muscle and epithelial cells in asthmatics. Inhibitors of tyrosine kinase have been analyzed to prevent airway hyperresponsiveness and eosinophilic infiltration (6).
At this time, we recommend that physicians consider taking esophageal biopsies of their patients with JPS to evaluate for EoE.
1. Roma-Giannikou ES, Papazoglou TA, Panayiotou JV, et al. Colon polyps in childhood: increased mucosal eosinophilia in juvenile polyps. Ann Gastroenterol
2. Mulder DJ, Gander S, Hurlbut DJ, et al. Multiple squamous hyperplastic-fibrous inflammatory polyps of the oesophagus: a new feature of eosinophilic oesophagitis? J Clin Pathol
3. Thakkar K, Alsarraj A, Fong E, et al. Prevalence of colorectal polyps in pediatric colonoscopy. Dig Dis Sci
4. Alexander R, Beckwith JB, Morgan A, et al. Juvenile polyps of the colon and their relationship to allergy. Am J Surg
5. Walter SA, Cutler RE Jr, Marinez R, et al. Stk10, a new member of the polo-like kinase kinase family highly expressed in hematopoietic tissue. J Biol Chem
6. Kim J-H, Park B-L, Cheong HS, et al. Variations in the STK10 gene and possible associations with aspirin-intolerant asthma in a Korean population. J Invest Allerg Clin