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Paeds Casesgastroenterology-hepatology-and-nutrition

Paeds Cases · gastroenterology-hepatology-and-nutrition

Inflammatory bowel disease: Case

Clinical case of a fourteen-year-old girl with bloody diarrhoea, fatigue, and weight loss who develops acute severe ulcerative colitis, covering the PUCAI-driven approach to diagnosis, intravenous corticosteroids, and the decision for rescue therapy.

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Target exams

RACP DCEMRCPCH ClinicalRCPSC Pediatrics

Target exams

RACP DCEMRCPCH ClinicalRCPSC Pediatrics
Prompt
A 14-year-old girl presents with a two-month history of bloody diarrhoea, six to eight times a day including overnight, with urgency, tenesmus, and cramping lower abdominal pain. She has lost 5 kg, is fatigued, and her haemoglobin is 92 g per litre, C-reactive protein is 45 mg per litre, and albumin is 26 g per litre. Her PUCAI on admission is 70. Ileocolonoscopy shows continuous superficial inflammation from the rectum to the splenic flexure with mucosal friability and ulceration, and biopsies confirm chronic active colitis with crypt abscesses.

This girl presents the classic picture of acute severe ulcerative colitis. Profuse bloody diarrhoea with nocturnal waking, tenesmus, weight loss, iron deficiency anaemia, raised inflammatory markers, and hypoalbuminaemia are the expected features, and a PUCAI of 70 confirms the diagnosis of acute severe colitis. The continuous superficial inflammation from the rectum with crypt abscesses on histology distinguishes ulcerative colitis from Crohn disease. [2]

Clinical findings

The pattern is unequivocally that of acute severe colitis. A PUCAI of 65 or greater defines acute severe colitis in children and mandates hospital admission for intravenous corticosteroids. Her anaemia, raised C-reactive protein, and low albumin reflect the severity and systemic nature of the inflammation, and the nocturnal diarrhoea and tenesmus confirm organic disease rather than a functional disorder. The continuous rectal inflammation with crypt abscesses and the absence of skip lesions, perianal disease, or upper-gut involvement fit ulcerative colitis rather than Crohn disease. [3]

The differential at presentation included infectious colitis, which is excluded by the negative stool studies, and Crohn disease, which is excluded by the continuous rectal-origin superficial inflammation and the absence of transmural or perianal features. The initial workup followed the ESPGHAN revised Porto criteria with ileocolonoscopy and biopsies, and upper endoscopy would be performed to complete the evaluation and exclude co-existing upper-gut involvement. [1]

Investigations and diagnosis

The diagnosis is acute severe ulcerative colitis, confirmed endoscopically and histologically. The PUCAI of 70 quantifies the severity and, combined with the raised inflammatory markers and hypoalbuminaemia, identifies a child at high risk of colectomy if she does not respond promptly to medical therapy. A plain abdominal radiograph is performed to exclude toxic megacolon, and the paediatric gastroenterology and surgical teams are involved from admission because the colectomy rate rises steeply with delay. [2]

Blood tests are tracked daily for haemoglobin, C-reactive protein, albumin, and electrolytes, and the PUCAI is recalculated each day to guide the timing of rescue therapy. This structured, score-driven approach is the cornerstone of acute severe colitis management and prevents the dangerous scenario of waiting too long before escalating. [3]

Management and outcome

Management begins with intravenous corticosteroids, fluid and electrolyte correction, and thromboprophylaxis, with close daily monitoring. By day three to five, if the PUCAI remains high on maximal medical therapy, the guideline-driven trigger for second-line rescue therapy is reached. Rescue options are infliximab or ciclosporin, and the surgical team remains involved in case colectomy becomes necessary. The aim is to rescue the colon without compromising safety. [2]

If she responds to intravenous therapy or rescue, she transitions to maintenance with aminosalicylates and, depending on the disease course, an immunomodulator such as azathioprine or an anti-TNF agent. Colectomy remains curative for the colonic disease if medical therapy fails or dysplasia develops, and she is enrolled in colorectal cancer surveillance because of the cumulative risk associated with longstanding extensive colitis. Structured transition to adult care and multidisciplinary support for nutrition, mental health, and adherence complete the long-term plan. [1]

References

  1. [1]Turner D, Ruemmele FM, Orlanski-Meyer E, Griffiths AM, de Carpi JM, Bronsky J, et al Management of Paediatric Ulcerative Colitis, Part 1: Ambulatory Care-An Evidence-based Guideline From European Crohn's and Colitis Organization and European Society of Paediatric Gastroenterology, Hepatology and Nutrition J Pediatr Gastroenterol Nutr, 2018.PMID 30044357
  2. [2]Turner D, Ruemmele FM, Orlanski-Meyer E, Griffiths AM, de Carpi JM, Bronsky J, et al Management of Paediatric Ulcerative Colitis, Part 2: Acute Severe Colitis-An Evidence-based Consensus Guideline From the European Crohn's and Colitis Organization and the European Society of Paediatric Gastroenterology, Hepatology and Nutrition J Pediatr Gastroenterol Nutr, 2018.PMID 30044358
  3. [3]Turner D, Travis SP, Griffiths AM, Ruemmele FM, Levine A, Benchimol EI, et al Consensus for managing acute severe ulcerative colitis in children: a systematic review and joint statement from ECCO, ESPGHAN, and the Porto IBD Working Group of ESPGHAN Am J Gastroenterol, 2011.PMID 21224839