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Oesophageal and severe gut involvement in the haemolytic uraemic syndrome
M N de la Hunt1, K P Morris, M G Coulthard
1Department of Paediatric Surgery, Royal Victoria Infirmary, Newcastle upon Tyne, UK.
Insights
Diarrhoea-associated haemolytic uraemic syndrome (HUS) can cause severe gastrointestinal issues, including bowel perforation. Early diagnostic awareness is crucial to prevent unnecessary appendicectomies in children with HUS.
Area of Science:
- Pediatrics
- Gastroenterology
- Nephrology
Background:
- Hemolytic Uremic Syndrome (HUS) is a serious condition often following diarrheal illness in children.
- Gastrointestinal complications are a significant concern in pediatric HUS cases.
Observation:
- A study reviewed 78 children with HUS, identifying seven with severe gastrointestinal involvement.
- Complications included bowel perforation, necrosis, and a rare esophageal stricture.
- Associated systemic issues like hypertension and cerebral/pancreatic involvement were noted.
Findings:
- Severe gastrointestinal involvement did not significantly impact long-term outcomes.
- Hematological indices showed potential for predicting severe gut involvement.
- Four children underwent appendectomy prior to HUS diagnosis, with findings atypical for primary appendicitis.
Implications:
- Increased clinical awareness of HUS can aid in earlier diagnosis and prevent misdiagnosis as appendicitis.
- Understanding predictive hematological markers may guide management strategies for HUS.
- Recognizing HUS-specific gastrointestinal presentations is vital for appropriate pediatric care.
Abstract:
Between 1982 and 1989, 78 children with diarrhoea-associated haemolytic uraemic syndrome (HUS) were referred to this hospital. Most presented with abdominal pain, bloody diarrhoea and vomiting. Seven had severe gastrointestinal involvement, four of whom required resection for bowel perforation or necrosis. One also developed an oesophageal stricture, a previously unreported complication of HUS. These seven children had a high incidence of other complications including hypertension, and cerebral and pancreatic involvement. One died from severe cerebral involvement, one has a residual neurological deficit and one has residual renal impairment. Severe gastrointestinal involvement did not significantly affect the long-term outcome. Simple haematological indices helped predict severe gut involvement. Four of the 78 children had undergone appendicectomy before the diagnosis of HUS was made. The operative findings were in no case typical of primary acute appendicitis, although histological examination did confirm inflammation of the appendix in two patients. Diagnosis is difficult in early disease, but increased awareness may help prevent unnecessary appendicectomy.