{
  "abstract": "Coeliac crisis is a medical emergency, rarely encountered in clinical practice. It may be a consequence of a delayed diagnosis of coeliac disease (CD). Diagnostic criteria for coeliac crisis include severe dehydration including orthostatic changes, renal dysfunction, neurological dysfunction, metabolic acidosis, abnormal electrolyte levels, hypoproteinaemia with Albumin below 3 g/dl and weight loss more than 4.5 kg. A diagnosis is made if more than 2 criteria are present. 1 We describe the case of a 4-year-old girl who presented with coeliac crisis as the first manifestation of undiagnosed coeliac disease, to raise awareness of the diagnostic criteria for coeliac crisis and consideration of this in unwell patients. Our patient presented with chronic diarrhoea, fatigue, and exhaustion. On admission she was not clinically dehydrated, had peripheral oedema, mildly distended abdomen with no signs of ascites. She had 20% weight loss, and her BMI was 13.23 (on 2d centile). A no-biopsy diagnosis of coeliac disease was made as she had IgA anti-tissue transglutaminase antibodies more than ten times the upper limit of normal and positive endomysium antibodies.5 Bloods showed compensated metabolic acidosis, with bicarbonate of 13, normal glucose at 4.1, low magnesium at 0.39 mmol/L, low albumin at 13 g/L. She required magnesium and albumin infusions for deficit correction. In addition, she had deranged PT/INR which normalised after two doses of intravenous Vitamin K. She also had transaminitis, and an extensive hepatitis screen. Autoimmune hepatitis was ruled out. An abdominal ultrasound showed fatty liver and normal elastography. Metabolic screen was normal. The levels of Vitamin D, folic acid, copper and zinc were low, and she had supplementation.Our patient met 4 out of the 7 criteria for coeliac crisis. She was commenced on gluten free diet, initially via nasogastric tube, then orally from day 4. She was monitored for refeeding syndrome and discharged following nutritional rehabilitation after 11 days. At her 2 months clinic follow up her bloods were normal; she has gained weight. Four months after her presentation her BMI improved to 17.7 (on 91st centile). Coeliac crisis was first described by Andersen and Di Sant’ Agnese in 1953 when they reported 35 patients with coeliac crisis, among which 3 were complicated by fatality.3 4 It is rarely encountered, probably due to better screening and early diagnosis; however, this can occasionally be the initial presentation of CD. The only effective treatment is a life-long gluten free diet. In coeliac crisis, the emergency treatment of electrolyte abnormalities is followed by gluten free diet and monitoring for refeeding syndrome. The role of corticosteroids in coeliac crisis is controversial but there are case reports of coeliac crisis with a good response to steroids, as they can reduce intestinal inflammation and restore the brush border enzymes.2 Our patient did not require steroids and responded well to gluten free diet. This case highlights the importance of considering coeliac crisis in an acutely unwell patient with chronic diarrhoea, weight loss and metabolic derangements.References Mauro A, Casini F. Celiac crisis as the life-threatening onset of celiac disease in children: a case report. Frontiers in Pediatrcs. DOI 10.3389/fped.2023.1163765.Gupta S, Kapoor K. Steroids in celiac crisis: doubtful role. Indian Pediatrics 2014;51(9):756–757.Di Sant’ Agnese PA. Idiopathic coeliac disease. II. Course and prognosis. Pediatrics 1953 Mar;11(3):224–37.Andersen DH, Di Sant’ Agnese PA. Idiopathic coeliac disease. I. Mode of onset and diagnosis. Pediatrics 1953 Mar;11(3):207–23.Husby S, Koletzko S, Wessels M, et al. European society paediatric gastroenterology, hepatology and nutrition guidelines for diagnosing coeliac disease 2020. JPGN 2020;70:141–157.",
  "authors": [
    {
      "affiliations": [
        "Sheffield Children’s Hospital"
      ],
      "name": "Rayna Alamurova"
    },
    {
      "affiliations": [
        "Sheffield Children’s Hospital"
      ],
      "name": "Natalia Nedelkopoulou"
    },
    {
      "affiliations": [
        "Sheffield Children’s Hospital"
      ],
      "name": "Prithviraj Rao"
    },
    {
      "affiliations": [
        "Sheffield Children’s Hospital"
      ],
      "name": "Arun Urs"
    },
    {
      "affiliations": [
        "Sheffield Children’s Hospital"
      ],
      "name": "Shishu Sharma"
    },
    {
      "affiliations": [
        "Sheffield Children’s Hospital"
      ],
      "name": "Dominique Schluckebier"
    },
    {
      "affiliations": [
        "Sheffield Children’s Hospital"
      ],
      "name": "Zuzana Londt"
    },
    {
      "affiliations": [
        "Sheffield Children’s Hospital"
      ],
      "name": "Akshay Kapoor"
    },
    {
      "affiliations": [
        "Sheffield Children’s Hospital"
      ],
      "name": "Mike Thomson"
    },
    {
      "affiliations": [
        "Sheffield Children’s Hospital"
      ],
      "name": "Priya Narula"
    }
  ],
  "title": "OC93 Coeliac crisis: case report",
  "uid": "731a01e1-058e-5444-8795-fc26d17813d0"
}