Plummer-Vinson Syndrome is a condition where iron deficiency is associated with difficulty swallowing due to the presence of an esophageal web. Deficiency of iron-dependent oxidative enzymes causes gradual degradation of the pharyngeal muscles which lead to mucosal atrophy and formation of webs. Although it is a very rare condition, an increased risk of esophageal squamous cell carcinoma makes its identification very important. Dilation of the esophageal web using a Savary dilator is a more effective and safer approach compared to conventional balloon dilation.
Objective: Noneislet cell tumor hypoglycemia (NICTH) is an uncommon paraneoplastic syndrome associated with mesenchymal neoplasms such as gastrointestinal stromal tumors (GISTs). We report the case of a patient with type 1 diabetes (T1D) and recurrent GIST who not only required discontinuation of insulin therapy but also required continuous parenteral glucose infusions to prevent hypoglycemia. Methods: A 59-year-old woman with a 24-year history of T1D and recurrent GIST presented with frequent episodes of symptomatic hypoglycemia despite continuous reductions in her insulin therapy. Laboratory workup revealed undetectable insulin and C-peptide, low insulin-like growth factor (IGF) 1, normal IGF-2, and an elevated IGF-2:IGF-1 ratio. Medical management with prednisone alone and, later, in combination with octreotide did not reduce hypoglycemic episodes. Eventually, during hospitalization for severe hypoglycemia, she was treated and discharged with continuous intravenous dextrose infusion. She ultimately required around-the-clock glucose infusions, which helped her maintain what she believed was an acceptable quality of life during her remaining weeks. Discussion: NICTH is characterized by excessive tumor production of IGF-2 or pro-IGF-2, leading to unrestricted glucose uptake in peripheral tissues and hypoglycemia. A diagnosis of NICTH can be made on the basis of low IGF-1 levels in the plasma with normal or elevated IGF-2. Tumor resection is the most definitive treatment for NICTH. Conclusion: This patient with T1D presented with resistant hypoglycemia due to recurrence of an enlarging GIST. She required discontinuation of all insulin therapy and continuous dextrose infusions to maintain euglycemia.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.