An Unusual Cause of Pediatric Dysphagia: Bronchogenic Cyst
Kaistha, Abha; Levine, Jeremiah
Inflammatory bowel disease: the classic gastrointestinal autoimmune disease
Kaistha, Abha; Levine, Jeremiah
Inflammatory bowel disease (IBD) is an idiopathic disease thought to be caused by a dysregulated immune response to host intestinal microflora. The role of genetic factors is indicated by familial clustering of cases and higher incidence in monozygotic twins. An interaction between genetic and environmental factors is thought to play a role in the pathogenesis of these disorders. Changes in diet, antibiotic use and intestinal colonization have likely contributed to increased prevalence of inflammatory bowel disease in the past century. Environmental factors or infections are thought to alter the barrier function of the epithelium, leading to loss of immune tolerance to intestinal antigens. This loss of tolerance activates dendritic cells, triggering their transport to mesenteric lymph nodes, where they promote differentiation of naive T cells to TH-1, TH-2, TH-17 cells or T regulatory cells. Production of proinflammatory cytokines and chemokines then follows. Circulating effector and regulatory cells enter the intestine through a highly selective mechanism that involves interaction with the vascular endothelium, diapedesis through the vessel wall and migration to the lamina propria. There are several genes implicated in IBD. Mutations in certain genes can cause defective down regulation of the innate immune response, ineffective clearance of intracellular bacteria and proliferation of both luminal and mucosal-adherent commensal bacteria. IBD is a chronic relapsing inflammatory condition that is immune mediated. Results from research in animal models, human genetics, basic science and clinical trials provide evidence that it is heterogeneous, characterized by various genetic abnormalities, leading to a dysregulated and overly aggressive T cell response to commensal enteric bacteria. Different genetic abnormalities can be characterized as causing defects in mucosal barrier function, immunoregulation or bacterial clearance. Advances in our understanding of the interplay between components of innate and adaptive immune response will be central to future progress.
A case of menetrier disease in a child
Williamson, Kristy; Park, Hyunsoo K; Schacht, Robert; Kaistha, Abha
ABSTRACT: Menetrier disease is a protein-losing gastroenteropathy often misdiagnosed in the pediatric population. The disease is characterized by hypoalbuminemia secondary to protein loss through the gastrointestinal mucosa and resultant peripheral edema. It is important for emergency department practitioners to consider this diagnosis in the differential diagnosis for edema and low albumin levels in pediatric patients. We present a case report of Menetrier disease in an edematous child and a brief review.
Celiac disease in African American children with type 1 diabetes mellitus in inner city Brooklyn
Kaistha, Abha; Castells, Salvador
OBJECTIVE: There is a strong evidence for association between type 1 diabetes and Celiac Disease. Up to 8% of patients with type1 diabetes have characteristic features of CD on small intestinal biopsy. Type 1 diabetics who have HLA DQ2 or DQ8 are at risk for CD. However most of this data is from studies conducted in Europe, with mostly Caucasian population. This study aims to identify the prevalence of celiac disease in African American children with Type1 diabetes in inner city Brooklyn, New York. METHODS: IgA and IgG Antigliadin antibodies, IgA tissue transglutaminase and HLA typing was measured in blood collected from 34 children with type1 diabetes mellitus. Patients with positive anti tissue transglutaminase antibody underwent small intestinal biopsy. RESULTS: 17 patients had elevated IgG AGA, none showed elevated IgA AGA. Only one patient had elevated IgA and anti tTG levels, and a normal small intestinal biopsy. 28 patients had HLA DQ2 or DQ8 present. CONCLUSIONS: 94% of the African American children with type1 diabetes were serology negative for celiac disease inspite of having the predisposing HLA haplotype. Only one patient was positive for anti tTG antibody, with a negative small intestinal biopsy, the prevalence of celiac disease in this population may not be similar to the other populations. Pediatric Endocrinology in review 990,2008.
Jejunal adenocarcinoma: a rare cause of chronic anemia in children [Case Report]
Pasternak, Brad A; Kaistha, Abha