Celiac disease in Saudi children with isolated short stature: is it rare or are we not screening rigorously enough?

2020 ◽  
Vol 33 (1) ◽  
pp. 89-93 ◽  
Author(s):  
Omar I. Saadah ◽  
Nouf M. ALNosani

AbstractBackgroundCeliac disease (CeD) is an immune-mediated enteropathy induced by gluten exposure in individuals with genetic susceptibility. Short stature (SS) can be the sole clinical manifestation of CeD, in the absence of gastrointestinal (GI) symptoms. This study aimed to determine the prevalence of CeD in Saudi Arabian children with SS.Patients and methodsMedical records were reviewed in a total number of 275 retrospective cases (during the period 2002–2014) of children with isolated SS from King Abdulaziz University Hospital, Jeddah. Their serum samples were tested with tissue transglutaminase (tTG) antibodies. Patients with a positive serology were scheduled for an upper endoscopy and intestinal biopsy to confirm CeD diagnosis before starting a gluten-free diet (GFD). Clinical, anthropometric and laboratory data were recorded for all patients.ResultsA total of 275 children with SS were included. The mean age ± standard deviation (SD) was 9.4 ± 4.0 years (range, 2.6–16.9 years) and males constituted the predominant gender group (151/275; 54.9%) over females (124/275; 45.1%). The mean ± SD height for age z score (HAZ) was −2.9 ± 1.0.Thirty-eight (13.8%) had positive serology, and 16 (5.8%) had biopsy-proven CeD. Apart from the difference in duration of delayed bone age between CeD patients and CeD-negative serology subjects (mean ± SD, 39.6 ± 10.5 vs. 18.6 ± 16.8, p = 0.02), no other major difference in other clinical or laboratory parameters was evident.ConclusionsThe prevalence rate of CeD in Saudi Arabian SS children was 5.8%, which is comparable to published reports of a number of other countries. Regular screening of children with SS is therefore justifiable.

2018 ◽  
Vol 55 (2) ◽  
pp. 175-178
Author(s):  
Yasin ŞAHIN ◽  
Kenan BARUT ◽  
Tufan KUTLU ◽  
Fugen Cullu COKUGRAS ◽  
Amra ADROVIC ◽  
...  

ABSTRACT BACKGROUND: Familial Mediterranean fever and celiac disease share some common clinical features such as abdominal pain, diarrhea, arthralgia and arthritis. Also, both of the diseases are associated with many inflammatory and autoimmune diseases. Previous studies have shown the association between familial Mediterranean fever (FMF) and different clinical conditions. OBJECTIVE: We aimed to investigate the relationship between celiac disease and colchicine-resistant familial Mediterranean fever (crFMF) disease. METHODS: This prospective study was conducted at the Department of Pediatric Gastroenterology and Pediatric Rheumatology from October 2015 to August 2016. A total of 24 patients with crFMF were included in the study. We used 60 sex- and age-matched healthy subjects as a control group. Levels of total IgA and tissue transglutaminase (tTG) IgA antibody were measured in both groups. Those with increased level of tTG IgA were tested for anti-endomysium IgA antibodies (EMA). Gastroduodenoscopy and intestinal biopsy were planned for a definite diagnosis of celiac disease in patients with positive EMA. RESULTS: Of the 24 patients in this study, 18 (75.0%) were female. Only 4 (16.6%) of 24 patients were positive for tTG IgA. Patients with positive tTG IgA were then tested for EMA IgA antibodies and none of them had a positive result. Only one (1.6%) subject from the control group was positive for tTG IgA but EMA positivity was not detected. CONCLUSION: We did not found celiac disease in 24 children with crFMF. Since crFMF disease is rarely seen in general population, further studies with more patients are needed to provide more precise interpretation.


2022 ◽  
Author(s):  
Elham Rahmanipour ◽  
fahimeh attarian ◽  
Mohammad Ghorbani ◽  
Bijan Shahbazkhani ◽  
Vahid Ghavami ◽  
...  

Abstract Background Celiac disease (1) mostly diagnosed base on positive serology and duodenal mucosal atrophy, but some patients have negative serology and their diagnosis have some limitation, it delay in diagnosis likely accompanied a poor prognosis and high risk of developing complications of CD. The aim of this study was determent clinical profile of patients with Seronegative CD (SNCD). Methods in this retrospective study, 1115+8 patients, that evaluated for CD with mucosal atrophy included between 2010 to2020. All patients with IgA deficiency other IgG based serology for diagnosis of celiac was done and if these antibodies were negative consider as possible SNCD. If they had positive DQ2-DQ8, and clinical symptoms or had positive challenge test after12 months of GFD were considered as SNCD. Results of total 1115 patients 27 (2.4%) had seronegative mucosal atrophy of duodenum and diagnosed as a SNCD (96.2% marsh3), the mean age and BMI in SNCD patients were significantly higher than other CD patients (p<0.05). Conclusion The prevalence of SNCD was 2.4% that likely related to over weighting, so clinicians should be considered high possible of seronegative CD in patients with over weighting and mucosal atrophy of duodenum.


2022 ◽  
Vol 22 (1) ◽  
Author(s):  
Zahra Akbari Namvar ◽  
Reza Mahdavi ◽  
Masood Shirmohammadi ◽  
Zeinab Nikniaz

Abstract Background In this trial, we investigated the effect of a group-based education program on gastrointestinal (GI) symptoms and quality of life (QOL) in patients with celiac disease (CD). Method In the present study, 130 patients with CD who were on a GFD for at least 3 months, randomly assigned to receive group-based education (n = 66) or routine education in the celiac clinic (n = 64) for 3 months. We assessed gastrointestinal symptoms and quality of life using the gastrointestinal symptom rating scale (GSRS) questionnaire and SF-36 questionnaire at baseline and 3 months after interventions. Results The mean age of the participants was 37.57 ± 9.59 years. There were no significant differences between the two groups regarding the baseline values. Results showed that the mean score of total GSRS score in the intervention group was significantly lower compared with the control group 3 months post-intervention (p = 0.04). Also, there was a significant difference in the mean score of SF-36 between the two groups 3 months post-intervention (p = 0.02). Conclusion Results showed that group-based education was an effective intervention in patients with celiac disease to improve gastrointestinal symptoms and quality of life. Trial registration IRCT code: IRCT20080904001197N21; registration date: 5/23/2019.


2008 ◽  
Vol 45 (1) ◽  
pp. 69-72 ◽  
Author(s):  
Patrícia Lopes de Almeida ◽  
Lenora Gandolfi ◽  
Inês Cristina Modelli ◽  
Rita de Cássia Martins ◽  
Rodrigo Coutinho de Almeida ◽  
...  

BACKGROUND: Several studies have shown that celiac disease, an autoimmune disorder that occurs in genetically susceptible individuals, is highly prevalent among relatives of celiac patients. AIM: To determine the prevalence of celiac disease in a group of first degree relatives of Brazilian celiac patients. METHODS: First degree relatives of celiac patients attending the Brasilia University Hospital Pediatric Gastroenterology Outpatient Clinic or the Celiac Disease Investigation Center, Brasília, DF, Brazil, between March 2001 and November 2004 were invited to undergo serological screening for celiac disease applying the IgA anti-endomysium antibody test (IgA-EMA). All positive IgA-EMA sera underwent a second screening using the IgA anti-tissue transglutaminase antibodies test. Duodenal or small intestinal biopsies were performed in all subjects positive to serological testing. Biopsy samples were classified as type (O) normal, (I) infiltrative, (II) infiltrative hyperplastic, (III) flat destructive, and (IV) atrophic hypoplastic. The final diagnosis was ascertained in subjects showing positive serological tests and a grade I to III small intestinal lesion. RESULTS: Nine new cases of celiac disease were found among the 188 first degree relatives tested (4.8%). CONCLUSION: The present study confirms the high prevalence of celiac disease among first degree celiac patients’ relatives and reinforces the need of extensive diagnostic screening in this specific group.


Author(s):  
Omar Ibrahim Saadah

Objective: To describe the growth pattern of children with celiac disease (CD) after introduction of a gluten-free diet (GFD). Methods: In this retrospective, children 2–16 years old with biopsy-proven CD in 2015–2018 were included in the study conducted at King Abdulaziz University Hospital, Jeddah, Saudi Arabia. Serial measurements of height-for-age z-score (HAZ) and weight-for-age z-score (WAZ), were recorded at 0, 4, 8, 12, and 16 months. Data on insulin-like growth hormone-1 (IGF-1) and insulin-like growth factor binding protein-3 (IGFBP-3) obtained at diagnosis and during follow-up were retrieved. Clinical, demographic, and laboratory data were extracted from patients' medical files. Results: The median age for the patients was 8.9 years (range, 2.4–16 years). Males constituted 53.2%. The mean WAZ at diagnosis was -2.8±1.9 and the mean HAZ was -3±0.99. Trend analysis indicated a significant time effect for WAZ (p<0.001) and for HAZ (p<0.001). The mean IGF-1 was 133.4±96.8 ng/ml and the mean IGFBP-3 was 3174±1081 ng/ml. There was significant increase in the secretion of IGF-1 (p=0.01) and IGFBP-3 (p=0.004) during the first 8 months of a GFD. Conclusion: The administration of a GFD for Saudi children with CD normalizes their growth parameters within 16 months of follow-up and improves the endogenous secretion of growth factors. Keywords: Celiac disease, growth, child, Continuous...


Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 1665-1665
Author(s):  
R.K. Marwaha ◽  
Deepak Bansal ◽  
Amita Trehan ◽  
Akash Patel

Abstract Celiac disease (CD) is a malabsorptive disorder wherein the proximal small bowel mucosa is damaged as a result of dietary exposure to gluten. Children with intractable diarrhea and failure to thrive are diagnosed with relative ease. Diagnosis can however be challenging and is often delayed when children present with ‘difficult to treat anemia’, without overt gastrointestinal manifestations. The case records of 77 patients with CD were scrutinized retrospectively. Diagnosis was established with serology (tissue transglutaminase-IgA assay) in 46 (59.7%), serology along with small bowel mucosal biopsy in 23 (29.9%) and with biopsy alone in the remaining 8 (10.4%). All children belonged to the predominantly wheat consuming northern Indian states. The mean age at presentation was 99.1±34.8 months (median: 102, range: 22–168). Males outnumbered females in a ratio of 1.96:1. The mean duration of symptoms was 41±31.2 months (median: 36, range: 1–132). The overwhelming majority, i.e., 75 (97.4%) children had anemia (Hemoglobin <11 g/dL). Mean hemoglobin (Hb) was 7.0±2.2 g/dL (median: 7.2, range: 2.3–12.5). 52 (67.5%) had received iron supplements for sufficient lengths, without benefit. The red cell morphology was microcytic hypochromic in 37 (48%) and dimorphic in 33 (42.9%). A history of diarrhea was not forthcoming in 32 (41.6%) cases. 59 (76.6%) were malnourished, with a weight less than 80 % of expected for the age and 30 (39 %) were stunted, with a height falling below the 90% of expected. Two children had skin bleeds secondary to coagulopathy, due to Vitamin K malabsorption. In another 2, recurrent anemia was attributed to pulmonary hemosiderosis; further investigations for secondary causes unearthed CD. All children were initiated on an austere gluten free diet, along with iron and folic acid supplements for the initial 6–9 months. Mean duration of follow was 17.7±20.9 months. Improvement was perceptible within days of initiating gluten free diet. Of the 38 (49.4%) children who had a follow up of a year or longer, the mean Hb at the last visit had risen to 12.9±1.2 g/dL. Conclusions: Hematologists need to be aware of the mono-symptomatic presentation of CD with anemia. The typical period of presentation of CD is described to be between 6 mo and 2 yr of age. Prolonged duration of symptoms and a diagnosis at a relatively older age is striking in the index study. In a suggestive clinical background, identification of CD with serodiagnosis alone, without resorting to small bowel biopsy is increasingly gaining acceptance, as the specificity of newer serological assays is 95–98%. This is particularly true in tropical countries, where some degree of flattening of villi may be attributed to malnutrition and or infections, such as rotavirus enteritis, Giardia lamblia, or tropical sprue. A biopsy may be misleading in such cases. Heightened awareness is essential to identify CD at an early age, especially, in children in whom anemia is the dominant manifestation. The benefits of gluten free diet are apparent with the rise in hemoglobin and the improvement in growth parameters are gratifying both for physicians and the caretakers.


2015 ◽  
Vol 52 (4) ◽  
pp. 278-282 ◽  
Author(s):  
Mahmud BAGHBANIAN ◽  
Ali FARAHAT ◽  
Hasan Ali VAHEDIAN ◽  
Elham SHEYDA ◽  
Mohamad Reza ZARE-KHORMIZI

Background - Celiac disease is an immune-mediated enteropathy due to a permanent sensitivity to gluten in genetically susceptible people. Iron-deficiency anemia is the most widely experienced anemia in humans. Iron-deficiency anemia additionally is a common extra intestinal manifestation of celiac disease. Objective - To investigate correlation between tTg levels and histological alterations and then to determine the prevalence of celiac disease in Center and South area patients of Iran with iron deficiency anemia. Methods - A total of 402 patients aged 12-78 years who presented with iron-deficiency anemia were included in this study. Hemoglobin, mean corpuscular volume and serum ferritin were determined. Venous blood samples for anti-tissue transglutaminase antibody immunoglobuline A and G were obtained from these patients. Upper gastrointestinal endoscopy was recommended to patients who had positive serology. Results - Of 402 patients with iron-deficiency anemia, 42 (10.4%) had positive serology for celiac disease. The small intestine biopsy of all patients with positive serology showed pathological changes (Marsh I, II & III). There was not significant difference in the mean hemoglobin level between iron-deficiency anemia patients with celiac disease and without celiac disease, duodenal biopsy results did not show significant relationship between the severity of pathological changes and levels of anti-tTG IgG (P -value: 0/869) but significant relationship was discovered between pathological changes and levels of anti-tTG IgA (P -value: 0/004). Conclusion - Screening of celiac disease by anti-tissue transglutaminase antibody should be completed as a routine investigation in patients with iron-deficiency anemia. Also physicians must consider celiac disease as a possible reason of anemia in all patients with iron deficiency anemia.


2008 ◽  
Vol 54 (7) ◽  
pp. 1203-1209 ◽  
Author(s):  
Kelly E McGowan ◽  
Martha E Lyon ◽  
J Decker Butzner

AbstractBackground: IgA deficiency causes false-negative IgA-based celiac serology results in patients with celiac disease. Using a case-finding strategy, we examined the prevalence of IgA deficiency, physician evaluation, and management of IgA deficiency during serological testing for celiac disease.Methods: We reviewed consecutive IgA-endomysial antibody (EMA) and serum IgA results from the laboratory database over 17 months. We cross-referenced seronegative patients with IgA deficiency (IgA &lt;0.06 g/L) to the pathology database to evaluate intestinal biopsy results. Ordering physicians received a questionnaire regarding the management of seronegative patients with IgA deficiency who had no biopsy record.Results: Among the 9533 patients tested for IgA-EMA, 4698 (49%) were tested for IgA deficiency. IgA deficiency occurred in 35 of 4698 (0.75%) patients screened for IgA deficiency. Only 19 of 35 (54%) IgA-deficient patients were diagnosed appropriately with either intestinal biopsy (17 patients) or measurement of IgG-tissue transglutaminase (2 patients). Thirteen (76%) of the 17 IgA-deficient patients who underwent upper endoscopy with or without colonoscopy displayed gastrointestinal pathology on biopsies, including 3 (18%) with celiac disease. No further evaluation to exclude celiac disease was performed for the remaining 16 of 35 (46%) IgA-deficient, EMA-negative patients because of inappropriate management (6 patients), administrative error (7 patients), or patient/physician refusal (3 patients).Conclusions: IgA deficiency occurred in 1:131 patients tested for celiac disease, and celiac disease occurred in 1:6 of those properly evaluated. Inadequate evaluation of IgA deficiency while testing for celiac disease occurred frequently and resulted in the underdiagnosis of both. Changes in testing algorithms and reporting of results were made to improve testing for celiac disease and IgA deficiency.


Author(s):  
Amita Surana ◽  
Vaishali Chaudhari ◽  
Shefali Patel

In this article we report a case of 17 years old male with short stature, severe anaemia and delayed puberty. After extensive workup and in-spite of being seronegative for celiac antibodies, he was diagnosed as an atypical case of celiac disease based on duodenal biopsy report suggestive of CD. The patient is put on gluten free diet along with other nutritional supplements. Long term consequences like short stature, infertility can be prevented by higher clinical suspicion and early diagnosis. Intestinal biopsy should be performed if there is strong clinical suspicion. The relevant literature has been reviewed and discussed in brief.


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