{
  "abstract": "Seronegative coeliac disease (SNCD) is a rare condition and there are no agreed published paediatric criteria for diagnosis.Our study aimed to evaluate whether the diagnostic and management pathway for SNCD in our centre aligned with current best practice standards.Retrospective case notes review of children diagnosed with coeliac disease over a 5 year period (July 2019–July 2024) was undertaken and those diagnosed with SNCD identified. Data were extracted from electronic records including demographic information, laboratory results, endoscopy and histology reports and entered into Excel database. A comprehensive literature search, supported by the Illingworth Library, was undertaken to identify diagnostic criteria for SNCD. Parameters for data collection were defined based on the findings of the literature review and descriptive statistical analysis was performed.According to adult literature, diagnostic criteria for SNCD1–4 6 typically include, Exclusion of IgA deficiency and other causes of enteropathyNegative IgA tTG and IgA EMADuodenal histology consistent with coeliac enteropathyPresence of HLA-DQ2 or HLA-DQ8 haplotypesClinical and histological improvement following a GFD, with resolution of enteropathy on repeat endoscopy at one year337 children were diagnosed with coeliac disease during the study period. Of these, 10 were identified as SNCD (2.96%), consistent with the published frequency of 1.7–5%.3 5 None were IgA deficient. One had a positive family history of SNCD, and one had dermatitis herpetiformis. No sex predilection was observed.3 4The median age at diagnosis was 7.5 years (range 15 months–14 years).Endoscopic macroscopic findings were documented in all, and 6 had scalloping noted in the duodenum. Histological examination revealed villous atrophy in all patients (table 1). Three children underwent repeat endoscopy following a GFD and all demonstrated mucosal healing. Although repeat endoscopy was routinely offered, most families declined, as clinical improvement was observed on GFD.Clinical symptoms in our cohort mirrored those typically seen in coeliac disease5 (table 1).When comparing against the adult criteria:All children fulfilled criteria such as - IgA deficiency ruled out, IgA tTG neg, IgA EMA neg, Positive histology.70% did not undergo repeat endoscopy.HLA typing was not performed in 6 out of 10 cases.The frequency of SNCD in our cohort aligns with published literature. This study highlights the absence of agreed paediatric diagnostic criteria for SNCD, leading to variability in clinical practice. This is an important gap that needs addressing and paediatric best practice consensus criteria for diagnosing SNCD are required to guide optimal management.References Zanini B, Magni A, Caselani F, et al. Diagnosis of seronegative and ultrashort celiac disease. Gastroenterology 2024;167(1):104–115.Aziz I, Evans KE, Hadjivassiliou M. Evaluation and management of seronegative enteropathies. Gastroenterology 2021;160(2):437–444.Cárdenas-Rodríguez N, Donat E, Ribes-Koninckx C. Seronegative coeliac disease: clearing the diagnostic dilemma. Curr Opin Gastroenterol. 2018;34(3):154–158.Biagi F, Marchese A, Trotta L, et al. Clinical classification and long-term outcomes of seronegative coeliac disease: a 20-year multicenter follow-up study. Aliment Pharmacol Ther. 2021;54(10):1278–1289. doi:10.1111/apt.16599.Singh P, Arora S, Lal SB, et al. Clinical features of children with serology negative, biopsy positive celiac disease. J Pediatr Gastroenterol Nutr. 2023;77(2):240–243.Sadeghi A, Ebrahimi Daryani N, Asadzadeh Aghdaei H, et al. Clinical profile of patients with seronegative celiac disease. Gastroenterol Hepatol Bed Bench. 2023;16(2):203–209.Oliveira R, De Castro L, Brandão A, et al. Seronegative celiac disease in Brazilian patients: a series of cases. Arq Gastroenterol. 2021;58(2):214–216.Schiepatti A, Sanders DS, Baiardi P. Nomenclature and diagnosis of seronegative coeliac disease and chronic non-coeliac enteropathies in adults: the Paris consensus. Gut 2022 Nov;71(11):2218–2225. doi: 10.1136/gutjnl-2021-326645. Epub 2022 Jun 8. PMID: 35676085; PMCID: PMC9554081.Abstract OC11 Table 1NumberMarsh grading D1Marsh Grading D3Symptomatology before diagnosis13a3aFamily h/o SNCD, Symptomatic on Gluten diet23c3bSignificant reflux and previous h/o of h/pylori33b3bReflux and improvement on GF diet43c3cReflux and dysphagia53cNormalFood refusal and abdominal pain6Normal3areflux, vomiting, abdominal pain, poor weight gain73b1Abdominal pain and vomiting83b3bnausea and vomiting, weight loss93aNormalBloating, intermittent abdominal pain103a3areflux, poor weight gain, lethargy",
  "authors": [
    {
      "affiliations": [
        "Sheffield children’s hospital"
      ],
      "name": "Nitin Reddy Patlolla"
    },
    {
      "affiliations": [
        "Sheffield children’s hospital"
      ],
      "name": "Rayna Alamurova"
    },
    {
      "affiliations": [
        "Sheffield children’s hospital"
      ],
      "name": "Mike Thomson"
    },
    {
      "affiliations": [
        "Sheffield children’s hospital"
      ],
      "name": "Akshay Kapoor"
    },
    {
      "affiliations": [
        "Sheffield children’s hospital"
      ],
      "name": "Prithviraj Rao"
    },
    {
      "affiliations": [
        "Sheffield children’s hospital"
      ],
      "name": "Arun U"
    },
    {
      "affiliations": [
        "Sheffield children’s hospital"
      ],
      "name": "Zuzana Londt"
    },
    {
      "affiliations": [
        "Sheffield children’s hospital"
      ],
      "name": "Sishu Sharma"
    },
    {
      "affiliations": [
        "Sheffield children’s hospital"
      ],
      "name": "Dominique Schluckebier"
    },
    {
      "affiliations": [
        "Sheffield children’s hospital"
      ],
      "name": "Valeria Dipasquale"
    },
    {
      "affiliations": [
        "Sheffield children’s hospital"
      ],
      "name": "Lizzie Griffiths"
    },
    {
      "affiliations": [
        "Sheffield children’s hospital"
      ],
      "name": "Sunita Rajani"
    },
    {
      "affiliations": [
        "Sheffield children’s hospital"
      ],
      "name": "Priya Narula"
    }
  ],
  "title": "OC11 Seronegative coeliac disease: a 5-year single centre retrospective review",
  "uid": "b483a34f-dd89-56cf-9b35-7e7616fbac98"
}