Tuesday, June 4, 2019

Celiac Disease Presenting as Unexplained Infertility

coeliac Disease Presenting as Unexplained InfertilityTitle- coeliac disease takeing as unexplained sterility and pancytopenia- A Case ReportAbstract Celiac disease is an autoimmune disorder of the small intestine significant tie beam with several extraintestinal features, such as reproductive disorders in women with undiagnosed celiac disease. Worldwide, CD prevalence is approximately 1%. Several studies suggest a high prevalence of undiagnosed CD in patients with infertility. A 21 years old Indian woman who presented with history of primary amenorrhea, diminished secondary versed character, asthenia, history of weight loss and chronic diarrhea. On admission her hemoglobin (Hgb) was 4.2 g/dL. Laboratory investigations confirmed a Immunoglobulin A (IgA) anti-tissue transglutaminase antibodies (IgA-tTG) was markedly grand to more than 300 u/ml, colonoscopy was normal, Bone flock densitometry revealed osteopenia and hypercellular bone marrow on bone marrow biopsy. Celiac disease diagnosis was made multivitamins, mineral, endovenous straighten out replacement and gluten free diet replacement treatment was initiated. In order trilineage hematopoiesis, iron deficiency anemia, granulocytic hyperplasia and mild megakaryocytic hypoplasia. Both her thrombocyte counts and white blood cell recovered uneventfully with continuing iron, calcium, vitmine D3 and folic acid supplementation. The possible mechanism for this phenomenon is discussed in this report.Introduction Celiac disease (CD) is a multifactorial chronic autoimmune general disease, triggered by gluten consumption in geneticalally predisposed individuals 1. Classically the disease is manifested by symptoms of diarrhea, flatulence and malabsorption, however, it is also associated with variable systemic manifestations, including metabolic bone disease, diabetes, thyroid dysfunction and lympho-proliferative malignancies 2. Worldwide, the prevalence of CD in the general population is approximately 1% femal e male ratio is 2 1 3-7. Several studies have confirmed the implications of celiac disease on the reproductive health of women. Celiac disease Development in cases of acute leukemia after(prenominal) allogeneic bone marrow transplantation (BMT) from Human leukocyte antigen identical siblings who suffered from celiac disease 11,12. We report the first case of celiac disease presented as pancytopenia with unexplained infertility in womanCase report A 21 year old Indian woman presented with primary amenorrhea, diminished secondary familiar character, asthenia, history of weight loss and chronic diarrhea. On admission her hemoglobin (Hgb) was 4.2 g/dL (normal range 11.015.1 gm/dL), red cell distribution width (RDW) was 39% (normal range 11.315.5%) and mean corpuscular volume was 63.6 fL (normal range 7997 fL). Reticulocyte count was low at 9000/mm3(normal range 25100 103/mm3), total iron binding capacity of 486 g/dL (normal range 250450 g/DL), percentage saturation of 5 and a ferri tin level of 3/mcL (normal range 150 four hundred 103/L), white blood cell (WBC) count was 3.2 103/L (normal range 411 103/L) with 4-6 segmented neutrophils. On the bone marrow core sections ring sideroblasts was non found. Bone marrow iron store showed passd amounts of storage iron. Total colonoscopy was normal. Upper gastrointestinal endoscopy showed a loss of folds in the second part of the duodenum and a biopsy from the second part of duodenum showed intraepithelial lymphocytes, flattening of duodenal mucosa, lymphoplasmacytic infiltration in lamina propria, and crypt hyperplasia (Marsh class 3). Immunoglobulin A (IgA) anti-tissue transglutaminase antibodies (IgA-tTG) was markedly elevated to more than 300 u/ml. Bone mass densitometry revealed osteopenia. He was under observation in short intervals at the Oncology clinic with abnormal finding as hypercellular bone marrow on bone marrow biopsy. Celiac disease diagnosis was made multivitamins, mineral, intravenous iron sucro se complex given at a dose of 100 mg twice weekly. successor and gluten free diet replacement treatment was initiated. On day 14th of treatment hemoglobin increase upto 6.1 g/dL , WBC count to 6.4 103/mcL, and platelets were 195 103/mcL. We present the first case of celiac disease that presented as primary amenorrhea with pancytopenia.Discussion A patient came to medicine OPD presenting primary amenorrhea, diminished secondary sexual character, asthenia, history of weight loss and chronic diarrhea. However, celiac disease is also associated with variable systemic manifestations, including metabolic bone disease, diabetes, thyroid dysfunction and lympho-proliferative malignancies 2. Given the constitutional anemia and the marginal thrombocytopenia and leucopenia. In to improve erythropoiesis she was initiated on iron replacement therapy. Initially which led decrease in WBC counts and platelet. Even though iron deficiency is related with a reactive thrombocytosis 13, when the seve rity of the iron deficiency increases lead to normalization 14,15 and infrequently even decrease in platelet counts 16,17,18. Still The precise mechanism of this is indistinct. Cause of pancytopenia in our patient may be related to decrease compactness of bone and increase free original damage to hemopoetic stem cell in bone marrow. Extremely low levels of estrogen hormone observed in our patient which is associated with bone demineralization and infertility, unretentive secondary sexual character in female.Conclusion Undiagnosed celiac disease is a jeopardy of infertility and pancytopenia. Pancytopenia may be due to decrease bone density result of that more exposure bone more ( haemopoetic stem cell) to free radical. Due to lack of proper nutrition, ovarian capsule could non protect newly growing follicle which lead to poorly development of estrogen hormone dependent secondary sexual character. Women seeking medical advice for this meticulous role should be screened for celiac disease and bone density (DEXA scan). Adoption of a gluten-free diet could have a positive knock on fertility in such kind of patients. It may be a potentially modifiable (and treatable) risk factor. Futher, a large scale study prospectively study for bone density and ovarian capsule composition to evaluate association between celiac disease and infertility in woman, pancytopenia to clarify these hypothesis.References Snchez E, de Palma G, Capilla A, et al. Influence of environmental and genetic factors linked to celiac disease risk on infant gut colonization by Bacteroides species. Applied and Environmental Microbiology. 201177(15)53165323.1b. Green PH, Cellier C. Celiac disease. 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