Nasim Sabery Khavari

All Publications

• Association between lichen sclerosus and celiac disease: a report of three pediatric cases. Pediatric dermatology Jacobs, L., Gilliam, A., Khavari, N., Bass, D. 2014; 31 (6): e128-31

  Abstract

  The prevalence of celiac disease (CD) is increasing and may be as high as 1% of the US population. The typical presentation of CD generally includes gastrointestinal symptoms, but more individuals are presenting with extraintestinal manifestations. A wide variety of dermatologic associations have been described with CD, including alopecia, dermatitis herpetiformis, and enamel hypoplasia. In this report we describe three girls with CD who presented with hypopigmented skin lesions and pruritus in the perivaginal and perianal areas, consistent with the diagnosis of lichen sclerosus (LS). All three presented within 1 year to the same practitioner. To our knowledge, this association has not previously been explored in the literature. These cases elucidate a possible relationship between CD and LS.

  View details for DOI 10.1111/pde.12402

  View details for PubMedID 25382799

• Association Between Lichen Sclerosus and Celiac Disease: A Report of Three Pediatric Cases PEDIATRIC DERMATOLOGY Jacobs, L., Gilliam, A., Khavari, N., Bass, D. 2014; 31 (6): E128-E131

  Abstract

  The prevalence of celiac disease (CD) is increasing and may be as high as 1% of the US population. The typical presentation of CD generally includes gastrointestinal symptoms, but more individuals are presenting with extraintestinal manifestations. A wide variety of dermatologic associations have been described with CD, including alopecia, dermatitis herpetiformis, and enamel hypoplasia. In this report we describe three girls with CD who presented with hypopigmented skin lesions and pruritus in the perivaginal and perianal areas, consistent with the diagnosis of lichen sclerosus (LS). All three presented within 1 year to the same practitioner. To our knowledge, this association has not previously been explored in the literature. These cases elucidate a possible relationship between CD and LS.

  View details for DOI 10.1111/pde.12402

  View details for Web of Science ID 000345694100008

  View details for PubMedID 25382799

• Cost-effectiveness of Universal Serologic Screening to Prevent Nontraumatic Hip and Vertebral Fractures in Patients With Celiac Disease. Clinical gastroenterology and hepatology Park, K. T., Tsai, R., Wang, L., Khavari, N., Bachrach, L., Bass, D. 2013; 11 (6): 645-653

  Abstract

  Patients with asymptomatic or poorly managed celiac disease can experience bone loss, placing them at risk for hip and vertebral fractures. We analyzed the cost-effectiveness of universal serologic screening (USS) vs symptomatic at-risk screening (SAS) strategies for celiac disease because of the risk of nontraumatic hip and vertebral fractures if untreated or undiagnosed.We developed a lifetime Markov model of the screening strategies, each with male or female cohorts of 1000 patients who were 12 years old when screening began. We screened serum samples for levels of immunoglobulin A, compared with tissue transglutaminase and total immunoglobulin A, and findings were confirmed by mucosal biopsy. Transition probabilities and quality of life estimates were obtained from the literature. We used generalizable cost estimates and Medicare reimbursement rates and ran deterministic and probabilistic sensitivity analyses.For men, the average lifetime costs were $8532 and $8472 for USS and SAS strategies, respectively, corresponding to average quality-adjusted life year gains of 25.511 and 25.515. Similarly for women, costs were $11,383 and $11,328 for USS and SAS strategies, respectively, corresponding to quality-adjusted life year gains of 25.74 and 25.75. Compared with the current standard of care (SAS), USS produced higher average lifetime costs and lower quality of life for each sex. Deterministic and probabilistic sensitivity analyses showed that the model was robust to realistic changes in all the variables, making USS cost-ineffective on the basis of these outcomes.USS and SAS are similar in lifetime costs and quality of life, although the current SAS strategy was overall more cost-effective in preventing bone loss and fractures among patients with undiagnosed or subclinical disease. On the basis of best available supportive evidence, it is more cost-effective to maintain the standard celiac screening practices, although future robust population-based evidence in other health outcomes could be leveraged to reevaluate current screening guidelines.

  View details for DOI 10.1016/j.cgh.2012.12.037

  View details for PubMedID 23357490

• ASPEN Clinical Guidelines: Nutrition Support of Children With Human Immunodeficiency Virus Infection JOURNAL OF PARENTERAL AND ENTERAL NUTRITION Sabery, N., Duggan, C. 2009; 33 (6): 588-606

  View details for DOI 10.1177/0148607109346276

  View details for Web of Science ID 000271393400001

  View details for PubMedID 19892900

• Desquamative Enteropathy and Pyloric Atresia Without Skin Disease Caused by a Novel Intracellular beta 4 Integrin Mutation JOURNAL OF PEDIATRIC GASTROENTEROLOGY AND NUTRITION Salvestrini, C., McGrath, J. A., Ozoemena, L., Husain, K., Buhamrah, E., Sabrey, N., Leichtner, A., Rufo, P. A., Perez-Atayde, A., Orteu, C. H., Torrente, F., Heuschkel, R. B., Thomson, M. A., Murch, S. H. 2008; 47 (5): 585-591

  Abstract

  Mutations in alpha6 or beta4 integrins (ITGA6, ITGB4) are known to cause junctional epidermolysis bullosa with pyloric atresia (JEB-PA), often lethal in infancy through skin desquamation. There is 1 report of pyloric atresia associated with a desquamatory enteropathy but without skin disease, of unknown molecular basis.We report 2 Kuwaiti siblings with pyloric atresia and life-threatening intestinal desquamation without significant skin abnormality. The older sibling died of intractable diarrhoea, and the younger sibling suffered episodes of massive protein-losing enteropathy, triggered by viral infections, in addition to obstructive uropathy. Mutation analysis was performed for ITGA6 and ITGB4 and expression of ITGA6 and ITGB4 protein was examined in skin and intestinal biopsies. Her serum also was incubated with normal intestine.We identified a novel mutation in ITGB4, with homozygous deletion of a single residue (isoleucine 1314) within the intracellular plectin-binding domain. Expression of ITGA6 and ITGB4 within skin, duodenal, and colonic epithelium was normal or minimally reduced, in contrast to previous reports. Biopsies taken during relapse showed accumulation of immunoglobulin G and C1q within intestinal basement membrane, whereas immunoglobulin G from her serum bound to basement membrane of normal small intestine. Immunomodulatory therapy induced significant improvement following relapses.ITGB4 mutation may induce a desquamative enteropathy in infancy without significant skin disease. A history of pyloric atresia is important in infants with severe chronic diarrhoeal disease and should prompt investigation for JEB-PA associated mutations. Acquired immune responses may exacerbate primary genetic disorders of epithelial adhesion and immunomodulatory therapy may be beneficial.

  View details for Web of Science ID 000260519400009

  View details for PubMedID 18955862

• Use of serologic markers as a screening tool in inflammatory bowel disease compared with elevated erythrocyte sedimentation rate and anemia PEDIATRICS Sabery, N., Bass, D. 2007; 119 (1): E193-E199

  Abstract

  The purpose of this work was to evaluate the use of serologic testing as a screening test for inflammatory bowel disease compared with erythrocyte sedimentation rate and hemoglobin in a referred patient population with suspected inflammatory bowel disease.A retrospective study was performed, reviewing medical charts of patients who had inflammatory bowel disease serology performed at Prometheus Laboratories from September 2002 to September 2004. Patients were divided into 4 categories: ulcerative colitis, Crohn disease, indeterminate colitis, and noninflammatory bowel disease groups. Patients were categorized based on clinical evaluation by board-certified pediatric gastroenterologists.A total of 227 patients seen at the Lucile Packard Children's Hospital Gastroenterology Clinic had inflammatory bowel disease serology performed at or before the time of diagnosis. Seventeen charts were excluded secondary to inadequate information. Forty children were found to have inflammatory bowel disease, a prevalence of 19%. Overall, serologic testing for inflammatory bowel disease had 60% sensitivity and 92% specificity. A positive laboratory test for anemia or an elevated erythrocyte sedimentation rate had 83% sensitivity, whereas the combination of anemia and elevated erythrocyte sedimentation rate had 96% specificity. The positive predictive value of serologic testing was 60% compared with 79% in patients with anemia and elevated erythrocyte sedimentation rate. The positive predictive value of serologic testing in the subgroup of subjects without rectal bleeding (139 subjects) was only 35% compared with 60% using routine tests. Almost one third of all positive serologic tests were in patients with no demonstrable inflammatory bowel disease.As a pediatric inflammatory bowel disease screening strategy for the general pediatrician or gastroenterologist, the measurement of the combination of erythrocyte sedimentation rate and hemoglobin has a higher positive predictive value and is more sensitive, more specific, and less costly than commercial serologic testing.

  View details for DOI 10.1542/peds.2006-1361

  View details for PubMedID 17158948