Pediatric Celiac Disease Patients Research Articles

Volumetric Analysis of Motor Cortex and Basal Ganglia in Pediatric Celiac Disease Patients Using volBrain: Implications for Neurological Dysfunction-Preliminary Results.

Celiac disease (CD) is a common immune-mediated, chronic systemic disorder that is treated with a strict, life-long gluten-free diet (GFD). In addition to gastrointestinal manifestations, CD also presents with a variety of extraintestinal symptoms, including significant neurological and neuropsychiatric symptoms. Among these neurological manifestations, motor dysfunctions are particularly notable. The aim of this study is to investigate the potential volumetric differences in brain structures, particularly the motor cortex and basal ganglia, between pediatric CD patients and healthy controls using the volBrain software AssemblyNet version 1.0. This prospective study included pediatric patients with CD who complained of neurological symptoms and were scheduled for brain magnetic resonance imaging (MRI). All children had been previously diagnosed with CD and their adherence to GFD was evaluated using the Biagi score. Brain MRIs were performed on all included patients to obtain volumetry at the onset of the disease. For volumetric and segmentation data, the volBrain software was used. In total, 12 pediatric patients with CD were included, with a median duration of a GFD of 5.3 years at the time of the MRI examination. There were no statistically significant differences between patients compliant with the GFD and those non-compliant in terms of age or duration of GFD. Volumetric analysis revealed deviations in all patients analyzed, which involved either a decrease or increase in the volume of the structures studied. Despite the limited number of patients in this study, the initial findings support previously described neurological manifestations in patients with CD. Newly developed MRI tools have the potential to enable a more detailed analysis of disease progression and its impact on the motor cortex.

The Association Between Iron Deficiency at Diagnosis, Female Sex, and Tissue Transglutaminase Antibody Normalization in Pediatric Celiac Disease.

Iron deficiency (ID) is one of the most common manifestations of Celiac disease (CD). We aimed to determine whether ID at CD diagnosis affects tissue transglutaminase antibody (TTG) normalization rate among pediatric CD patients adhering to a gluten-free diet (GFD). We conducted a retrospective, observational cohort study that enrolled CD subjects aged 2-18y, diagnosed between Jan 2016 and Dec 2020. Demographic and laboratory data were collected at diagnosis and one year after adherence to GFD. ID was determined according to hemoglobin and ferritin levels. We compared CD subjects with and without ID at CD diagnosis in relation to TTG normalization at one year. Our cohort included 118 pediatric CD subjects. At diagnosis, 61 (51.7%) of CD subjects had ID, of whom 27 (44.3%) were female, compared to 46 (80.7%) females in the non-ID group (p<0.001). Median age at CD diagnosis was 5.7y (IQR 4-8.4, range 2-14) and 7.2y (IQR 4.7-10.8, range 0.9-16), and among those with and without ID, respectively (p=0.1). After one year of adherence to GFD, TTG normalization was achieved in 38 (65.5%) and 28 (53.8%) of those with and without ID at CD diagnosis, respectively (p=0.21). However, TTG normalization was achieved in 38 (79.2%) of males compared to 42 (49.4%) of females (p=0.001). ID at CD diagnosis was not associated with lower rates of TTG normalization at one year among pediatric patients adhering to GFD. However, TTG normalization at one year was significantly more frequent among male subjects compared to females.

Helicobacter pylori infection in pediatric patients with celiac disease: A single-center experience

Aim: There are numerous studies investigating the relationship between celiac disease (CD) and Helicobacter pylori (H. pylori). In this study, we aimed to compare the prevalence of coexisting H. pylori infection and CD in pediatric patients diagnosed with celiac disease at our clinic versus an age-matched control group. Materials and Methods: A total of 198 patients with celiac disease and 131 sex and age -matched controls undergoing upper gastrointestinal endoscopy due to dyspeptic complaints were included in this study. The prevalence of H. pylori was compared between the groups. Results: Of the 198 patients with CD included in the study, 66 (33.3%) were male and 132 (66.7%) were female, while in the control group, 36 (27.5%) were male, and 95 (72.5%) were female. 89 (44%) of the patients with CD and 70 (53.4%) of the controls were H. pylori-positive. There was no statistically significant difference in the distribution of Marsh scores between the H. pylori-negative and -positive groups. Conclusion: The association between celiac disease and H. pylori remains controversial. Thus, multicenter studies are warranted to evaluate the role of H. pylori in the pathogenesis of celiac disease.

Path Towards Biopsy-Free Diagnosis of Celiac Disease in Pediatric Patients

BackgroundLaboratory testing for celiac disease in pediatric patients integrates serology, genetic susceptibility and duodenal biopsy examination. The 2023 American College of Gastroenterology guidelines recommend a biopsy-free approach in pediatric patients utilizing tissue transglutaminase antibody titers >10 times upper limit of normal and subsequent endomysial antibody seropositivity as sufficient for diagnosis. The objective of this study is to assess the diagnostic accuracy of biopsy-free approach at our pediatric hospital. MethodsWe conducted a retrospective study involving pediatric patients who underwent biopsy for diagnostic confirmation of celiac disease between May 2019 and May 2023. For these patients, the tissue transglutaminase and endomysial antibody test results were retrieved and performance of biopsy-free approach was assessed using the duodenal histology as the gold standard for celiac disease diagnosis. ResultsTissue transglutaminase antibody titers >10 times upper limit of normal alone demonstrated a positive predictive value of 99% for identifying celiac disease in children. Although endomysial antibody testing is underutilized at our center, its inclusion further improved the predictability to 100 %. ConclusionPositive predictive value of tissue transglutaminase antibody titers >10 times upper limit of normal is sufficiently high for celiac disease diagnosis in children and may allow for deferral of duodenal biopsy at diagnosis.

Relationship between vitamin D levels and pediatric celiac disease: a systematic review and meta-analysis

BackgroundThe relationship between Vitamin D levels and pediatric celiac disease (CD) remains controversial. In this study, we conducted a systematic review and meta-analysis to examine the relationship between Vitamin D and pediatric CD. Methods: We screened relevant studies from PubMed, EMBASE, and Web of Science published in English from January 1, 2000, to August 1, 2023. The included studies were assessed according to the STROBE checklist. Heterogeneity was quantified by Cochran’s Q test and the I2 statistic. Publication bias was estimated by Begg’s test and Egger’s test. Meta‐regression was used to detect potential sources of heterogeneity. Results: A total of 26 studies were included in the meta-analysis. Nineteen articles compared 25(OH)D3 levels between CD patients and control groups, average 25-hydroxyvitamin D3 [25(OH)D3 or calcidiol], and 1,25-dihydroxyvitamin D3 [1,25(OH)2D3 or calcitriol] levels, as the main forms of Vitamin D, there was a significant difference in CD patients and healthy controls (weighted mean difference (WMD) = − 5.77, 95% confidence interval (CI) = [− 10.86, − 0.69] nmol/L). Meanwhile, eleven articles reported the numbers of patients and controls with Vitamin D deficiency, there was a significant difference in the incidence of 25(OH)D3 deficiency between CD patients and healthy controls (odds ratio 2.20, 95% CI= [1.19, 4.08]). Nine articles reported changes in 25(OH)D3 levels before and after administering a GFD in patients with CD, the result of this study revealed the increase of 25(OH)D3 levels in CD patients after a gluten-free diet (GFD) (WMD = − 6.74, 95% CI = [− 9.78, − 3.70] nmol/L). Conclusions: Vitamin D levels in pediatric CD patients were lower than in healthy controls, and 25(OH)D3 deficiency was more prevalent in CD patients. We found that 25(OH)D3 levels were elevated in CD patients after GFD, which is consistent with previous research. Further well-designed, longitudinal, prospective cohort studies focusing on the role of Vitamin D in the pathogenesis of CD are therefore needed.

Autoimmunity-related LINC01934 and AP002954.4 lncRNA polymorphisms may be effective in pediatric celiac disease: a case-control study.

Various studies have reported that certain long non-coding RNA levels are unusually low in the intestines of celiac disease patients, suggesting that this may be associated with the inflammation observed in celiac disease. Despite these studies, the research aimed at uncovering the potential role of long non-coding RNAs in the pathogenesis of autoimmune diseases like celiac disease remains insufficient. Therefore, in this study, we plan to assess long non-coding RNA polymorphisms associated with autoimmunity in children diagnosed with celiac disease according to the European Society for Paediatric Gastroenterology Hepatology and Nutrition criteria. DNA was isolated from paraffin tissue samples of 88 pediatric celiac disease patients and 74 healthy pediatric individuals. Single-nucleotide polymorphism genotyping of five long non-coding RNA polymorphisms associated with autoimmunity (LINC01934-rs1018326, IL18RAP-rs917997, AP002954.4-rs10892258, UQCRC2P1-rs6441961, and HCG14 rs3135316) was conducted using the TaqMan single-nucleotide polymorphism genotyping assays with the LightCycler 480. In our study, the genotypic and allelic frequency distribution of LINC01934-rs1018326 and AP002954.4-rs10892258 polymorphisms was found to be statistically significant in the comparison between the two groups (p<0.05). According to the multiple genetic model analyses, the LINC01934-rs1018326 polymorphism was observed to confer a 1.14-fold risk in the recessive model and a 1.2-fold risk in the additive model for pediatric celiac disease. Similarly, the AP002954.4-rs10892258 polymorphism was found to pose a 1.40-fold risk in the dominant model and a 1.7-fold risk in the additive model. Our study results draw attention to the LINC01934-rs1018326 and AP002954.4-rs10892258 polymorphisms in celiac disease and suggest that these polymorphisms may be associated with inflammation in autoimmune diseases like celiac disease.

Relation between Type 1 Diabetes and Celiac Disease in Children

Objective: This retrospective study aimed to investigate the association between Type 1 diabetes (T1D) and celiac disease in pediatric patients, examining the prevalence, clinical presentation, and laboratory findings in a cohort of children with both conditions. Methods: Medical records of 155 pediatric patients diagnosed with T1D at Nawaz Sharif Medical College in Gujrat were reviewed. Patients diagnosed with celiac disease either through serological tests or duodenal biopsies were included. Data on demographic characteristics, clinical symptoms, laboratory findings, and family history were collected and analyzed. Subgroup analysis was conducted to investigate associations between variables. Results: Among the 155 pediatric patients with T1D, 25 (16.1%) were found to have coexisting celiac disease. The clinical presentation included gastrointestinal symptoms such as abdominal pain and diarrhea, along with failure to thrive. Serological tests for anti-tissue transglutaminase antibodies were positive in all confirmed cases, and duodenal biopsies revealed characteristic histopathological changes. No significant correlations were observed between age at T1D diagnosis, family history of T1D or celiac disease, and the co-occurrence of both conditions. Practical Implication: This study will be helpful in finding the association of diabetes and celiac diseases in pediatric patients. Conclusion: This retrospective study highlights the significant association between T1D and celiac disease in pediatric patients, emphasizing the importance of early screening and detection for celiac disease in children with T1D. Healthcare professionals should remain vigilant in assessing gastrointestinal symptoms and growth patterns to optimize early diagnosis and appropriate management. Keywords: Pediatric, Patients, Diseases, TID

The value of mid-upper arm circumference for malnutrition screening in pediatric celiac disease.

Malnutrition rates in pediatric celiac disease (CD) patients range from 20.2% to 67.3%. To investigate the prevalence of malnutrition in pediatric CD patients in Turkey using different anthropometric measurements, including mid-upper arm circumference (MUAC). This prospective study included 124 patients aged 1-18 years with a diagnosis of CD, admitted to the Pediatric Gastroenterology Outpatient Clinic of Adana City Training and Research Hospital, Turkey. The anthropometric measurements, including weight-for-age (WFA) Z-score, height-for-age (HFA) Z-score, body mass index (BMI)-for-age Z-score, MUAC [cm], and MUAC Z-score were calculated. The study analyzed 75 female (60.5%) and 49 male (39.5%) patients with a mean age of 9.83 ±4.1 years. While 44 patients (35.5%) had malnutrition according to their BMI Z-scores, 60 patients (48.4%) had malnutrition based on their MUAC Z-scores. The number of patients with stunting (HFA value below -2) was 24 (19.4%), and the WFA value was below -2 in 27 (21.8%) patients. Furthermore, the BMI Z-score failed to identify chronic malnutrition in 70.9% of patients. There was a positive linear correlation (r = 0.396) between the BMI value and the MUAC value (p < 0.001). However, the degree of agreement between the BMI Z-scores and MUAC Z-scores was weak (κ: 0.300). The MUAC Z-score successfully detected acute and chronic malnutrition and should be included in standard anthropometric measurements at follow-up nutritional assessments in CD patients.

Nutrient intakes in adult and pediatric coeliac disease patients on gluten-free diet: a systematic review and meta-analysis.

Celiac Disease (CD) continues to require a strict lifetime gluten-free diet (GFD) to maintain healthy status. Many studies have assessed the GFD nutritional adequacy in their cohorts, but an overall picture in adults and children would offer a lifetime vision to identify actionable areas of change. We aimed at assessing the nutrient intakes of adult and pediatric CD patients following a GFD diet and identifying potential areas of improvement. Systematic review was carried out across PubMed, Scopus and Scholar up to October 2022, including full-text studies that assessed the nutrient intakes of CD patients on GFD, in terms of macro- and/or micronutrients (absolute or percentage daily average). Random-effect meta-analysis and univariable meta-regression were applied to obtain pooled estimates for proportions and influencing variables on the outcome, respectively. Thirty-eight studies with a total of 2114 patients were included. Overall, the daily energy intake was 1995 (CI 1884-2106) Kcal with 47.8% (CI 45.7-49.8%) from carbohydrates, 15.5% (CI 14.8-16.2%) from proteins, and 35.8% (CI 34.5-37.0%) from fats. Of total fats, 13.2% (CI 12.4-14.0%) were saturated fats. Teenagers had the highest consumption of fats (94.9, CI 54.8-134.9 g/day), and adults presented insufficient dietary fiber intake (18.9 g, CI 16.5-21.4 g). Calcium, magnesium, and iron intakes were particularly insufficient in adolescence, whereas vitamin D was insufficient in all age groups. In conclusion, GFD may expose CD patients to high fat and low essential micronutrient intakes. Given GFD is a lifelong therapy, to prevent the occurrence of diseases (e.g. cardiovascular or bone disorders) dietary intakes need to be assessed on long-term follow-ups.

Peroxiredoxins and Hypoxia-Inducible Factor-1α in Duodenal Tissue: Emerging Factors in the Pathophysiology of Pediatric Celiac Disease Patients.

Celiac disease (CD) is an autoimmune enteropathy. Peroxiredoxins (PRDXs) are powerful antioxidant enzymes having an important role in significant cellular pathways including cell survival, apoptosis, and inflammation. This study aimed at investigating the expression levels of all PRDX isoforms (1-6) and their possible relationships with a transcription factor, HIF-1α, in the small intestinal tissue samples of pediatric CD patients. The study groups consisted of first-diagnosed CD patients (n = 7) and non-CD patients with functional gastrointestinal tract disorders as the controls (n = 7). The PRDXs and HIF-1α expression levels were determined by using real-time PCR and Western blotting in duodenal biopsy samples. It was observed that the mRNA and protein expression levels of PRDX 5 were significantly higher in the CD patients, whereas the PRDX 1, -2, and -4 expressions were decreased in each case compared to the control group. No significant differences were detected in the PRDX 3 and PRDX 6 expressions. The expression of HIF-1α was also significantly elevated in CD patients. These findings indicate, for the first time, that PRDXs, particularly PRDX 5, may play a significant role in the pathogenesis of CD. Furthermore, our results suggest that HIF-1α may upregulate PRDX-5 transcription in the duodenal tissue of CD.

Gluten-free diet affects fecal small non-coding RNA profiles and microbiome composition in celiac disease supporting a host-gut microbiota crosstalk

ABSTRACT Current treatment for celiac disease (CD) is adhering to a gluten-free diet (GFD), although its long-term molecular effects are still undescribed. New molecular features detectable in stool may improve and facilitate noninvasive clinical management of CD. For this purpose, fecal small non-coding RNAs (sncRNAs) and gut microbiome profiles were concomitantly explored in CD subjects in relation to strict (or not) GFD adherence over time. In this observational study, we performed small RNA and shotgun metagenomic sequencing in stool from 63 treated CD (tCD) and 3 untreated subjects as well as 66 sex- and age-matched healthy controls. tCD included 51 individuals on strict GFD and with negative transglutaminase (TG) serology (tCD-TG-) and 12 symptomatic with not strict/short-time of GFD adherence and positive TG serology (tCD-TG+). Samples from additional 40 healthy adult individuals and a cohort of 19 untreated pediatric CD subjects and 19 sex/age matched controls were analyzed to further test the outcomes. Several miRNA and microbial profiles were altered in tCD subjects (adj. p < .05). Findings were validated in the external group of adult controls. In tCD-TG-, GFD duration correlated with five miRNA levels (p < .05): for miR-4533-3p and miR-2681-3p, the longer the diet adherence, the less the expression differed from controls. tCD-TG+ and untreated pediatric CD patients showed a similar miRNA dysregulation. Immune-response, trans-membrane transport and cell death pathways were enriched in targets of identified miRNAs. Bifidobacterium longum, Ruminococcus bicirculans, and Haemophilus parainfluenzae abundances shifted (adj. p < .05) with a progressive reduction of denitrification pathways with GFD length. Integrative analysis highlighted 121 miRNA-bacterial relationships (adj. p < .05). Specific molecular patterns in stool characterize CD subjects, reflecting either the long-term GFD effects or the gut inflammatory status, in case of a not strict/short-time adherence. Our findings suggest novel host-microbial interplays and could help the discovery of biomarkers for GFD monitoring over time.

Human Leukocyte Antigen-DQ Genotyping in Pediatric Celiac Disease

The purpose of this study was to determine the pattern of human leukocyte antigen (HLA)-DQ genotype in children diagnosed with celiac disease (CD) (biopsy proven), and to compare this with a control group; and secondarily, to correlate HLA genotypes with clinical profiles of CD. This cross-sectional comparative observational study included 26 controls and 52 patients diagnosed with CD who presented at Sir Padampat Mother and Child Health Institute, Jaipur, from May, 2017 to October, 2018. HLA DQ genotype was assessed for each patients and correlated with clinical profiles. HLA DQ2/DQ8 genotypes were significantly more common in CD (present in 100.0% cases) than in controls (23.1%) in Northern India (Rajasthan). When HLA DQ2.5 and DQ8 were present together, individuals had significantly more atypical presentations and severe findings on duodenal biopsy. Similarly, patients with the HLA DQ 2.5 genotype were also predisposed to more severe endoscopic findings, while HLA DQ2.2 predisposed them to less severe biopsy findings. HLA DQ8 was significantly associated with later age at diagnosis (>5 years) and shorter stature. The highest HLA DQ relative risk (RR) for CD development was associated with HLA DQ2.5 and DQ2.2 in combination, followed by HLA DQ2.5 and DQ8 in combination, while HLA DQx.5 and HLA DQ2.2 together had the lowest risk. HLA DQ2/DQ8 genotypes are strongly associated with pediatric CD patients in northern India. These genotypes and their combinations may be associated with different clinical presentations of CD, and may help predict severity of CD.

The role of serology in the diagnosis of coeliac disease.

Serology has significantly revolutionized the knowledge of celiac disease (CD), leading to the identification of unsuspected patients in at-risk CD groups, thereby increasing the number of CD diagnoses compared to the pre-screening era. Several markers for CD with a progressive diagnostic accuracy have been identified over the years, but only three of them, i.e. anti-tissue transglutaminase (anti-tTG), anti-endomysial (EmA) and anti-deamidated gliadin antibodies (DGP) are currently assessed in the daily clinical practice. A thorough review of the literature identified 44 original studies published between 1998 to 2022 for a total of 5098 pediatric and adult CD patients (without selective IgA deficiency) and 11930 disease controls. The results highlighted that anti-tTG IgA exhibited a higher sensitivity for CD (93.4%) than EmA IgA (92.8%), DGP IgG (81.8%) and DGP IgA (83.8%). The specificity of EmA IgA (99%) resulted to be higher than those of anti-tTG IgA (95.8%), DGP IgG (96.4%) and DGP IgA (92.1%). In patients with selective IgA deficiency, a condition closely related to CD, serological screening should include one of the three antibodies of IgG class, since anti-tTG, DGP and EmA have a very similar diagnostic accuracy in this clinical setting. According to age, there are two main diagnostic strategies for CD detection. In children, the revised ESPGHAN 2020 guidelines established that CD could be diagnosed in both symptomatic and asymptomatic children by high anti-tTG IgA titers (>10 times the cut-off) and EmA positivity with no need to obtain duodenal biopsy and HLA typing. In adult patients, although high tTG IgA titers (confirmed by EmA IgA positivity) correlate with villous atrophy, an intestinal biopsy is still considered mandatory for confirming CD diagnosis. Currently, a case finding approach in at-risk groups is preferred to mass screening for CD detection.

Association Between Allergic Conditions and Celiac Disease in Pediatric Patients.

Association Between Allergic Conditions and Celiac Disease in Pediatric Patients.

Coexisting Type 1 Diabetes, Persistent Symptoms, and Financial Issues Associate With Poorer Adherence to a Gluten-Free Diet in Celiac Disease After Transition From Pediatrics to Adult Care.

PurposeWe evaluated adherence to a gluten-free diet and associated factors in adult celiac disease patients diagnosed in childhood.MethodsComprehensive medical data on 955 pediatric celiac disease patients was collected and study questionnaires sent to 559 who were now adults. All variables were compared between strictly adherent and non-adherent patients.ResultsAltogether 237 adults (median age 27 years, 69% women) responded to the questionnaires a median of 18 (range 3–51) years after the childhood diagnosis. Altogether 78% were reportedly adherent and 22% non-adherent. The non-adherent patients had more concomitant type 1 diabetes (18% vs. 4%, p = 0.003), whereas the groups did not differ in demographic data or clinical and histological features at diagnosis, or in short-term dietary adherence. In adulthood, non-adherent patients found gluten-free diet more challenging (39% vs. 17%, p < 0.001) and had higher prevalence (39% vs. 19%, p = 0.004) and severity of symptoms. The main motivation factors for dietary adherence were attempts to avoid symptoms and complications, but these were considered less important and price of gluten-free products more important among non-adherent patients. Adherent and non-adherent patients did not differ in socioeconomic or lifestyle factors, comorbidities other than type 1 diabetes, self-reported general health, health concerns, follow-up, or in quality of life.ConclusionMost originally pediatric celiac disease patients reported strict dietary adherence in adulthood. However, particularly those with concomitant type 1 diabetes, persistent symptoms or financial issues may require attention during the transition from pediatric to adult care.

A Review of Pediatric Celiac Patients in Southeastern Turkey: A Single-Center Experience

Objective: Celiac disease is an autoimmune enteropathy that primarily affects the small intestine. Celiac disease occurs with the ingestion of foods containing gluten and is characterized by malabsorption in individuals with a genetic predisposition. This study aimed to review the clinical, laboratory, radiological, and pathological findings of pediatric celiac patients who were followed in our clinic and to compare the data with previous reports in the literature. Methods: A total of 509 patients who were diagnosed with celiac disease in the Pediatric Gastroenterology Clinic between 2010 and 2013 were included in this study. Medical records of the patients were reviewed retrospectively to collect their demo-graphic characteristics, anthropometric and bone mineral density measurements, laboratory results, radiological imaging, endoscopic examinations, and pathology reports of the biopsy materials. Results: Of the 509 patients enrolled in the study, 290 (57%) were females and 219 (43%) males. Among these patients, 441 (86.6%) presented with typical symptoms and 68 (13.4%) presented with atypical symptoms. A total of 479 (94.1%) patients were compliant with the gluten-free diet, whereas 30 (5.9%) patients were not. The chief complaint was growth retardation in the patient groups aged 61 to 144 months (44.6%) and &gt;145 months (59%) and diarrhea (26.1%) in the patient group aged 0-60 months. In all patients, the most common physical examination findings at the time of presentation were normal (57.4%), and the most common comorbidities were iron deficiency anemia (35%) and osteoporosis (33%). While Marsh-Oberhuber stage 3c (52.5%) was most common in patients aged 0-60 months, Marsh-Oberhuber stage 3b was most common in patients aged 61-144 months and patients aged &gt;145 months (51.9% and 63.8%, respectively). Conclusions: Although growth and developmental retardation and chronic diarrhea are cautionary for celiac disease, patients may also present with extra-gastrointestinal (atypical) findings. The mainstay of celiac disease treatment is strict compliance with a gluten-free diet. Reviewing a relatively large number of cases, this study sheds some light on the current status of pedi-atric celiac disease patients in the southeastern part of Turkey. Keywords: Celiac disease, child, Marsh

Association between idiopathic pulmonary hemosiderosis and celiac disease in pediatric patients: A scoping review of the literature over the past 50 years.

Idiopathic pulmonary hemosiderosis (IPH) is a rare cause of diffuse alveolar hemorrhage, the mechanism of which is currently unknown. Nearly one-third of pediatric patients with IPH test positive for Celiac disease (CD) serology. Several hypothetical mechanisms have been proposed to unify the coexistence of these two entities, also referred to as Lane-Hamilton syndrome (LHS). This manuscript is a scoping review of the medical literature. Medline, Embase, and PubMed Central databases were searched between 1971 and 2021 with appropriate search words to identify all cases of pediatric LHS. A total of 20 manuscripts with 23 pediatric patients with LHS were identified. The mean age was 11 years, and 13/23 (56.5%) of the children were boys. Hemoptysis was present in 57% of patients during diagnosis. Bronchoscopy with bronchoalveolar lavage demonstrating hemosiderin laden macrophages was the primary mode of diagnostic confirmation. Only three patients underwent lung biopsy. Any significant GI symptom was reported in a minority of patients (22%). Iron deficiency anemia on presentation was described in 83% of children. The majority of patients were malnourished. Serology for CD was positive in all patients, as was the histopathologic analysis of the small bowel biopsy. No patients had any other autoantibody positivity. The introduction of gluten free diet (GFD) was associated with a positive response in 20/23 patients. All pediatric patients with IPH should undergo screening for CD. Low serum ferritin in patients with IPH could be suggestive of coexisting CD. Strict GFD should be tried as the initial therapy.

Clinical Presentation of Pediatric Celiac Disease Patients in the Qassim Region Over Recent Years.

IntroductionCeliac disease is an immune-mediated systemic disease. It is prevalent and has diverse clinical manifestations; gastrointestinal symptoms are more common in children, including failure to thrive, chronic diarrhea, vomiting, and abdominal distention. The diagnosis should be made at a precise time to evade severe irreversible complications, especially for pediatric patients. This study aimed to determine the clinical presentation and diagnosis, including laboratory, serological tests, and histopathological findings, in pediatric celiac disease patients.Patients and methods From January 2019 to August 2021, all children with a confirmed celiac disease diagnosis at Maternity and Children's Hospital in Buraydah, Qassim region, Saudi Arabia, were studied retrospectively. Information was collected, including demographics, clinical presentation, and diagnostic modalities with serology and small intestinal histology reported by Marsh grading. ResultsFourteen patients were reviewed, with a mean age of 8.64 years. Marsh grading of those who underwent biopsy revealed that half of the patients had type 3a, and the rest had either type 1 or 3b celiac disease. Clinical manifestations included abdominal distention and chronic diarrhea, and some patients were asymptomatic.ConclusionAbdominal distention, chronic diarrhea, constipation, and nausea were the most common clinical features. Patients with a family history of celiac disease, longer symptom duration, and higher tissue transglutaminase immunoglobulin A (tTG-IgA) levels are more symptomatic.

Evaluation of psychiatric symptoms of children with Celiac Disease and their relationship with gluten-free diet and maternal factors

Background. The purpose was to evaluate the quality of life scores and the adherence of gluten-free diet (GFD) in children with celiac disease (CD). The other objective was to determine the relevance of the maternal emotional status between the psychological adjustments and GFD compliance of the patients with CD. Material and methods. Children’s depression inventory (CDI), Screen for Child Anxiety and Related Disorders (SCARED), Strength and Difficulties Questionnaire (SDQ), KINDer Lebensqualitätsfragebogen Questionnaire, Beck Depression Inventory (BDI) and Beck Anxiety Inventory (BAI) were administered to both children with CD healthy controls. Furthmore, the results were compared between the CD patients ant the healthy ones. Results. A total of 47 patients with CD, 33 healthy children and their mothers were enrolled. GFD-compliance rate, which was confirmed by antibody tests, was found to be 41.7 %. The scores of CDI, SCARED, and SDQ were significantly higher in CD patients than the healthy children. Moreover, the total scores of KINDL was significantly lower in CD group. Higher scores of BDI and BAI were found in the CD patients’ mothers than the healthy group. In patients group there were positive-moderate statistically significant correlation detected between score of BDI, BAI of mothers and CDI, SCARED, SDQ scores of children. There were also negative statistically significant correlation between scores of BDI, BAI of mothers and KINDL scores of children. Conclusions. Increased prevalence of psychopathology and reduced quality of life have been clearly demonstrated in children with CD. Worse maternal psychosocial adjustment significantly associated with depressive symptoms in pediatric CD patients.

Assessment of Duodenal Intraepithelial Lymphocyte Composition (Lymphogram) for Accurate and Prompt Diagnosis of Celiac Disease in Pediatric Patients.

INTRODUCTION:Quantitative and phenotypic analyses of duodenal intraepithelial lymphocytes (IELs) by flow cytometry (IEL lymphogram) confer specificity and enable the diagnosis even in unconventional presentations of celiac disease (CD). To evaluate the validity of the IEL lymphograms in the pediatric population for new insights into their use as biomarkers in the natural history of CD.METHODS:We retrospectively included 1,211 children (602 with active CD, 92 on a gluten-free diet, 47 with potential CD, and 470 nonceliac controls) who required duodenal biopsies in this study. The cutoff values for IEL subsets were established to calculate the probability of disease according to the lymphogram.RESULTS:A celiac lymphogram (a ≥15% increase in gamma-delta T-cell receptor IELs and a simultaneous ≤6% decrease in CD3 surface-negative [sCD3−]) IELs was strongly associated with the diagnosis of active CD, which was present in 89.7% of the confirmed patients. The remaining 10% of the celiac patients had a partial celiac lymphogram (≥15% increase gamma-delta T-cell receptor IELs or ≤6% decrease in sCD3− IELs), with lower diagnostic certainty. On a gluten-free diet, nearly 20% of the patients were indistinguishable from nonceliac subjects based on the lymphogram. In potential CD, a decrease in sCD3− IELs was a risk marker of progression to villous atrophy and a diagnosis of active CD.DISCUSSION:If a biopsy is clinically indicated, the IEL lymphogram adds specificity to the histological findings, reducing diagnostic delays and misdiagnoses. The lymphogram is useful for monitoring the natural progression of the disease and predicting the transition from potential celiac to overt CD.