Epidemiological, Clinical, Histological, Serological and Therapeutic Study of Children Celiac Disease in Western Algeria Region

Objectives: To determine the epidemiological, clinical, histological, serological and therapeutic profile of celiac disease in children in Western Algeria region.Methods: 250 patients over a period of three years (2016 - 2019) from the pediatric department of the University Hospital of Oran (West Algeria) were included in our retrospective study.Results: We noticed a female predominance with a sex ratio F/M = 1.57. Symptoms were defined by typical (73.2%) and atypical (26.8%) forms, digestive disorders (47.2%), extra digestive disorders (14.4%). Anemia was noted at 73.6%, and the association with autoimmune diseases at 14.8%. Seropositivity was present in 77.6% of cases and histology revealed partial grade villous atrophy at 54%. We noticed a significant link between the histological grade and the serology (p = 0.001), between age and histological grade (p<10-3), between bone age and body mass index (p = 0.017), between the age of onset of the disease and the age of food diversification (p = 0.030), and with the age of breastfeeding (p = 0.026). In addition, we found an excellent correlation between anti-transglutaminase and anti-endomysium autoantibodies during diagnosis and under diet (p<10-3), and between serology during diagnosis and serology under diet (p = 0.002)Conclusion: Celiac disease (CD) of children in western Algeria is characterized by a variety of clinical symptoms. The gluten-free diet remains the only therapy for these affected children. Keywords: celiac disease, children, epidemiology, serology, histology, associated diseases, treatment.

Download Full-text

Celiac Disease—The Relative Importance of Wheat Gluten

PEDIATRICS ◽

10.1542/peds.16.4.503 ◽

1955 ◽

Vol 16 (4) ◽

pp. 503-503

Keyword(s):

Celiac Disease ◽

Bile Salts ◽

Clinical Symptoms ◽

Wheat Gluten ◽

Significant Advance ◽

Fat Absorption ◽

Digestive Disorders ◽

The University ◽

Harmful Effects

This report is a continuation of an exploration by the group at the University of Birmingham of the suggestions proposed by the Dutch workers, Dicke, Weijers, and van de Kamer, reported in 1950 that children with celiac disease recover when wheat and rye flours are excluded from the diet. The gluten fraction of wheat and of rye appears to contain the harmful substance. The present study reports on the proportion of children with active celiac disease responding to a diet free from wheat or rye gluten. All but 2 of 30 children with celiac disease responded to diets free from wheat gluten. Improvement was manifested by elimination of clinical symptoms and coincident improvement of the indices of fat absorption. One of the children who did not respond to the diet was found to improve by the administration of bile salts; and a deficiency of bile salts was demonstrated in the duodenal contents. The other child who failed to improve was considered an example of fat intolerance which persisted after an attack of diarrhea. The series of observations concerning the role of gluten in the pathogenesis of celiac is the most significant advance in the understanding of this disorder which has been made for many years. The references provided will enable one to trace the development of this intersting concept. Further elucidation of the mechanism involved in the harmful effects produced by gluten should provide interesting chapters in the long search for understanding and treatment of one of the most troublesome and intriguing digestive disorders affecting infants and children.

Download Full-text

A161 MACROSCOPIC AND HISTOLOGY FINDINGS DURING UPPER DIGESTIVE ENDOSCOPY IN CHILDREN WITH SUSPICION OF CELIAC DISEASE AND HIGH LEVELS OF TRANSGLUTAMINASE IGA-ANTIBODY

Journal of the Canadian Association of Gastroenterology ◽

10.1093/jcag/gwz047.160 ◽

2020 ◽

Vol 3 (Supplement_1) ◽

pp. 25-26

Author(s):

P Jantchou ◽

M Dirks ◽

K Grzywacz ◽

V Marchand ◽

U Halac ◽

...

Keyword(s):

Celiac Disease ◽

Clinical Symptoms ◽

Diagnostic Yield ◽

Villous Atrophy ◽

Duodenal Bulb ◽

Analysis Data ◽

The United States ◽

Predictive Test ◽

Histological Findings ◽

Total Villous Atrophy

Abstract Background The European Society of Pediatric Gastroenterology Hepatology and Nutrition suggests that the diagnosis of Celiac disease (CD) can be confirmed solely on the basis of clinical symptoms and bloodwork including a level of transglutaminase IgA-antibodies (TGA) ≥ 10 times the upper limit of normal (10XN). In Canada and the United States, this recommendation has not been endorsed. We recently demonstrated that TGA ≥ 10XN performed at our institution (INOVA Diagnostics’ Quanta Lite) was a reliable predictive test of villous atrophy in patients with suspicion of CD. Aims The aim of the present study was to investigate the rate of supplemental endoscopic or histological findings in a cohort of children with TGA ≥ 10XN and the association of these findings with clinical symptoms. Methods Consecutive children with suspected CD who had an endoscopy between 2011 and 2018 were included in this analysis. Data was extracted from our CD database. The macroscopic and histological findings were reported. We compared these diagnoses to the clinical symptoms. Results From 2011 to 2018, 405 new cases of CD were identified in our pediatric center. In total, 238 (58.7%) patients had baseline TGA levels ≥ 10XN (67.2% females, median (IQR) age 8.4 (4.8–12.2)). The median interval between the first visit to the gastroenterology unit and the endoscopy was 43.0 (21.0–78.0) days. In total, 58% of the endoscopies had macroscopic findings in the bulb (37.8 %) or the duodenum (41.5%) including a mosaic pattern, mucosal fissuring, or erythema. Seven cases (2.8%) of esophagitis were identified during endoscopy; histological analysis confirmed eosinophilic esophagitis in 3 cases (1.2%) and peptic esophagitis in 4 cases. Non-specific gastritis was present in 58 patients (24.4%) and 2 cases of Helicobacter pylori infection were identified. The biopsies showed subtotal/total villous atrophy of duodenum in 171 (71.8%) or partial villous atrophy in 51 patients (29.8 %). Ten patients (4,3%) had villous atrophy in the duodenal bulb alone, with normal biopsies of the second part of the duodenum. Abdominal pain did not correlate with gastritis or duodenitis. However, children with diarrhea had a greater prevalence of visible endoscopic inflammation in the duodenum than those without diarrhea: 53.5% vs 38.9% respectively; P= 0,037. Conclusions Apart from the classical features associated with CD, the supplementary diagnostic yield of endoscopy was low. There were only a few cases of additional diseases identified by the endoscopic procedure in a large cohort of children and adolescents with suspicion of CD. Therefore, these results support the no-biopsy approach in the settings of TGA ≥ 10XN using a reliable diagnostic kit. Funding Agencies None

Download Full-text

CLINICAL SYMPTOMS, ULTRASOUND AND HISTOPATHOLOGY OF TUMORLIKE LESIONS OF THE BLADDER.

Journal of Medicine and Pharmacy ◽

10.34071/jmp.2017.1.6 ◽

2017 ◽

pp. 41-46

Author(s):

Van Mao Nguyen ◽

Thi Bich Chi Nguyen

Keyword(s):

Bladder Cancer ◽

Bladder Tumor ◽

Clinical Symptoms ◽

Bladder Wall ◽

Lower Abdominal Pain ◽

University Hospital ◽

Wall Thickening ◽

Health Examination ◽

Ultrasound Images ◽

Key Words Bladder Cancer

Background: Bladder cancer is one of the most frequent type of urinary cancer which has been ever increasing. For the better treatment, the early discovery and definite diagnosis of this disease played an important role. Objective: To describe some clinical symptoms and ultrasound features of tumorlike lesions of the bladder. To diagnose and classify the histopathology of tumorlike lesions of the bladder. Materials, method: cross - sectional study on 64 cases in Hue University Hospital and Hue central hospital from April, 2016 to February, 2017. Results: Hematuria was the most common reason that patients went to hospital (79.7%). Lower abdominal pain and irritation during urination accounting for 9.4% and 6.2% respectively. Only 3 patients with bladder cancer were accidentally discovered through periodic health examination (4.7%). The characteristics of hematuria in bladder tumor was flesh red urine (62.5%) and total hematuria (60.7%). With ultrasonography, the results of 64 patients were divided in 3 groups as follow: bladder tumor, which was the highest rate 87.5%, bladder polyp was 3.1% and focal bladder wall thickening was 9.4%. Of which, the vast majority of these ultrasound images was tumor - like lesions protruding in the lumen of the bladder (75%), the rest was wall thickening lesions (25%). Tumors were different in size, the biggest tumor was 7cm in diameter and the smallest was 0.6cm. Those with the diameter 3cm or bigger accounting for 42.2%, the smaller was 57.8%. Most cases have only one lesion (62.5%) and at lateral wall (46.6%). Histopathologically, cancer was 59/64 case (92.2%): urothelial carcinoma was 98.3 %, squamous cell carcinomawas 1.7% and 5 cases (7.8%) were benign. Most cancerous cases were poorly differentiated, grade II (50.9%) and grade III (32.2%). The stage T1NxMx was 20.3% and worse than T2MxNx was 79.7%. Conclusion: hematuria was the most popular symptom, suggesting bladder cancer. Clinical diagnosing bladder cancer was not high sensitive (61.01%). Ultrasound could detect bladder tumor with high sensitive (89.8%). These patients also needed histopathology classification to diagnose and finally choose the best method for the appropriate treatment. Key words: bladder cancer, histopathology, ultrasound, uroepithelial carcinoma, hematuria

Download Full-text

Non-Celiac Gluten Sensitivity: A Challenging Diagnosis in Children with Abdominal Pain

Annals of Nutrition and Metabolism ◽

10.1159/000493929 ◽

2018 ◽

Vol 73 (Suppl. 4) ◽

pp. 39-46 ◽

Cited By ~ 2

Author(s):

Frank M. Ruemmele

Keyword(s):

Abdominal Pain ◽

Celiac Disease ◽

Clinical Symptoms ◽

Gluten Sensitivity ◽

Gluten Free ◽

Clear Cut ◽

Clinical Presentations ◽

New Findings ◽

Three Phase ◽

Gi Symptoms

Several disorders related to the ingestion of gluten are well recognized despite overlapping clinical presentations: celiac disease, an autoimmune enteropathy triggered by gluten ingestions in susceptible individuals, allergy to wheat, and more recently non-celiac gluten sensitivity (NCGS). While celiac disease and wheat allergy are well-known disorders with a clear-cut diagnosis based on clinical tests and biological parameters, NCGS is a more difficult diagnosis, especially in children with functional gastrointestinal (GI) complaints. NCGS is considered a syndrome of intestinal but also extraintestinal symptoms occurring within hours, but sometimes even after several days of gluten ingestion. In children, the leading symptoms of NCGS are abdominal pain and diarrhea, while extraintestinal symptoms are rare, in contrast to adult patients. No precise diagnostic test nor specific biomarkers exist, except a rather cumbersome three-phase gluten-exposure, gluten-free diet, followed by a blinded placebo-controlled gluten challenge with crossover to provoke symptoms elicited by gluten in a reproducible manner that disappear on gluten-free alimentation. Recent data indicate that the peptide part of wheat proteins is not necessarily the sole trigger of clinical symptoms. Mono- or oligosaccharides, such as fructan and other constituents of wheat, were able to provoke GI symptoms in clinical trials. These new findings indicate that the term gluten sensitivity is probably too restrictive. The incidence of NCGS was reported in the range of 1–10% in the general population and to increase steadily; however, most data are based on patients’ self-reported gluten intolerance or avoidance without a medically confirmed diagnosis. Treatment consists of gluten avoidance for at least several weeks or months. Patients with NCGS require regular reassessment for gluten tolerance allowing with time the reintroduction of increasing amounts of gluten.

Download Full-text

Variable clinical characteristics and laboratory results in five patients with Chinese Good's syndrome (thymoma and hypogammaglobulinemia): an 8-year retrospective analysis in a university hospital in China

BMC Immunology ◽

10.1186/s12865-021-00441-9 ◽

2021 ◽

Vol 22 (1) ◽

Author(s):

Jinyao Ni ◽

Junwu Zhang ◽

Yanxia Chen ◽

Weizhong Wang ◽

Jinlin Liu

Keyword(s):

Information System ◽

Lichen Planus ◽

Hospital Information System ◽

Clinical Characteristics ◽

Opportunistic Infections ◽

Clinical Symptoms ◽

University Hospital ◽

Ivig Treatment ◽

Good’S Syndrome ◽

Laboratory Results

Abstract Background Good's syndrome (GS) is a rare secondary immunodeficiency disease presenting as thymoma and hypogammaglobulinemia. Due to its rarity, the diagnosis of GS is often missed. Methods We used the hospital information system to retrospectively screen thymoma and hypogammaglobulinemia patients at the First Affiliated Hospital of Wenzhou Medical University from Apr 2012 to Apr 2020. The clinical, laboratory, treatment, and outcome data for these patients were collected and analyzed. Results Among the 181 screened thymoma patients, 5 thymoma patients with hypogammaglobulinemia were identified; 3 patients had confirmed diagnoses of GS, and the other 2 did not have a diagnosis of GS recorded in the hospital information system. A retrospective review of the clinical characteristics, laboratory results, and follow-up data for these 2 undiagnosed patients confirmed the diagnosis of GS. All 5 GS patients presented with pneumonia, 2 patients presented with recurrent skin abscesses, 2 patients presented with recurrent cough and expectoration, 1 patient presented with recurrent oral lichen planus and diarrhea, and 1 patient presented with tuberculosis and granulomatous epididymitis. In the years after the diagnosis of hypogammaglobulinemia with mild symptoms, all 5 patients had received irregular intravenous immunoglobulin (IVIG) treatment. As the course of the disease progressed, the clinical symptoms of all patients worsened, but the symptoms were partly resolved with IVIG in these patients. However, 4 patients died due to comorbidities. Conclusion GS should be investigated as a possible diagnosis in thymoma patients who present with hypogammaglobulinemia, especially those with recurrent opportunistic infections, recurrent skin abscesses, chronic diarrhea, or recurrent lichen planus.

Download Full-text

Celiac disease: Understandings in diagnostic, nutritional, and medicinal aspects

International Journal of Immunopathology and Pharmacology ◽

10.1177/20587384211008709 ◽

2021 ◽

Vol 35 ◽

pp. 205873842110087

Author(s):

Taoufik Ben Houmich ◽

Brahim Admou

Keyword(s):

Celiac Disease ◽

Villous Atrophy ◽

Current Trend ◽

Diagnostic Delay ◽

General Medicine ◽

Iga Deficiency ◽

Health Concern ◽

Intestinal Biopsy ◽

Antibody Testing ◽

Main Challenge

Celiac disease (CD) is characterized by clinical polymorphism, with classic, asymptomatic or oligosymptomatic, and extra-intestinal forms, which may lead to diagnostic delay and exposure to serious complications. CD is a multidisciplinary health concern involving general medicine, pediatric, and adult gastroenterology, among other disciplines. Immunology and pathology laboratories have a fundamental role in diagnosing and monitoring CD. The diagnosis consists of serological testing based on IgA anti-transglutaminase (TG2) antibodies combined with IgA quantification to rule out IgA deficiency, a potential misleading factor of CD diagnosis. Positive TG2 serology should be corroborated by anti-endomysium antibody testing before considering an intestinal biopsy. Owing to multiple differential diagnoses, celiac disease cannot be confirmed based on serological positivity alone, nor on isolated villous atrophy. In children with classical signs or even when asymptomatic, with high levels of CD-linked markers and positive HLA DQ2 and/or DQ8 molecules, the current trend is to confirm the diagnosis on basis of the non-systematic use of the biopsy, which remains obligatory in adults. The main challenge in managing CD is the implementation and compliance with a gluten-free diet (GFD). This explains the key role of the dietitian and the active participation of patients and their families throughout the disease-management process. The presence of the gluten in several forms of medicine requires the sensitization of physicians when prescribing, and particularly when dispensing gluten-containing formulations by pharmacists. This underlines the importance of the contribution of the pharmacist in the care of patients with CD within the framework of close collaboration with physicians and nutritionists.

Download Full-text

Novel mutation in the TGFBI gene in a Moroccan family with atypical corneal dystrophy: a case report

BMC Medical Genomics ◽

10.1186/s12920-020-00861-3 ◽

2021 ◽

Vol 14 (1) ◽

Author(s):

Yahya Benbouchta ◽

Imane Cherkaoui Jaouad ◽

Habiba Tazi ◽

Hamza Elorch ◽

Mouna Ouhenach ◽

...

Keyword(s):

Exome Sequencing ◽

Whole Exome Sequencing ◽

Clinical Symptoms ◽

Age Of Onset ◽

Novel Mutation ◽

Corneal Dystrophy ◽

Heterozygous Mutation ◽

Large Variability ◽

Whole Exome ◽

Moroccan Family

Abstract Background Corneal dystrophies (CDs) are a heterogeneous group of bilateral, genetically determined, noninflammatory bilateral corneal diseases that are usually limited to the cornea. CD is characterized by a large variability in the age of onset, evolution and visual impact and the accumulation of insoluble deposits at different depths in the cornea. Clinical symptoms revealed bilateral multiple superficial, epithelial, and stromal anterior granular opacities in different stages of severity among three patients of this family. A total of 99 genes are involved in CDs. The aim of this study was to identify pathogenic variants causing atypical corneal dystrophy in a large Moroccan family and to describe the clinical phenotype with severely different stages of evolution. Case presentation In this study, we report a large Moroccan family with CD. Whole-exome sequencing (WES) was performed in the three affected members who shared a phenotype of corneal dystrophy in different stages of severity. Variant validation and familial segregation were performed by Sanger sequencing in affected sisters and mothers and in two unaffected brothers. Whole-exome sequencing showed a novel heterozygous mutation (c.1772C > A; p.Ser591Tyr) in the TGFBI gene. Clinical examinations demonstrated bilaterally multiple superficial, epithelial and stromal anterior granular opacities in different stages of severity among three patients in this family. Conclusions This report describes a novel mutation in the TGFBI gene found in three family members affected by different phenotypic aspects. This mutation is associated with Thiel-Behnke corneal dystrophy; therefore, it could be considered a novel phenotype genotype correlation, which will help in genetic counselling for this family.

Download Full-text