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Intractable diarrhoea in infancy in the 1990s: a survey in Italy
1Istituto di Clinica Pediatrica, Policlinico Santa Chiara, University of Pisa, Italy.
Insights
Intractable diarrhea is rare in Italy, affecting few children. Autoimmune enteropathy is a common cause, but many cases lack a clear diagnosis, often requiring long-term parenteral nutrition (PN).
Area of Science:
- Pediatric Gastroenterology
- Clinical Research
Background:
- Intractable diarrhea, defined as diarrhea lasting over 3 weeks and requiring over 50% of caloric intake from parenteral nutrition (PN), is a significant clinical challenge.
- Understanding the prevalence and causes of this condition is crucial for effective management.
Purpose of the Study:
- To determine the prevalence of intractable diarrhea in Italy.
- To identify the common etiologies and diagnostic challenges associated with this condition.
Main Methods:
- A rapid prevalence study utilizing a FAX-based questionnaire distributed to 35 pediatric gastroenterology services across Italy.
- Data collection focused on identifying cases of intractable diarrhea, treatment modalities (inpatient vs. home PN), diagnostic outcomes, and specific etiologies.
Main Results:
- Twenty cases of intractable diarrhea were identified in 9 centers, indicating a low prevalence.
- Autoimmune enteropathy was the most frequent diagnosis (5 cases), followed by congenital microvillous atrophy (3 cases).
- In 11 out of 20 cases, a diagnosis was established; 9 cases showed intestinal mucosal atrophy, and 9/20 had undefined etiologies.
Conclusions:
- Intractable diarrhea is a rare but complex condition in Italy, often necessitating long-term PN.
- While autoimmune enteropathy is a leading cause, a significant proportion of cases remain without a defined etiopathogenetic diagnosis.
Unlabelled:
A "quick" prevalence study of intractable diarrhoea (defined as diarrhoea lasting more than 3 weeks and dependent on parenteral nutrition [PN] for more than 50% of daily caloric intake) was conducted by FAX. All 35 paediatric gastroenterology services which had been contacted answered questionnaire sent by FAX. 20 cases of intractable diarrhoea were identified in 9 centres. In 12 cases PN was administered at home, the other 8 cases being treated as inpatients for an average duration of 9.5 months. A diagnosis had been established in 11 out of 20 cases. Auto-immune enteropathy was the most frequent diagnosis (5 cases); congenital microvillous atrophy (3 cases); chronic pseudo-obstruction (2 cases) and multiple food intolerance (1 case). Undefined 9/20 cases presented atrophy of intestinal mucosa. The age of the beginning of diarrhoea varied from 2 days to 12 years, but was more than 16 months only in some cases with auto-immune enteropathy.
Conclusion:
Intractable diarrhoea has a low prevalence in Italy and remains a rare but very intricating problem. Long-term PN is recommended in most cases: autoimmune enteropathy is the most frequent cause but in about half of the cases the aetiopathogenetic diagnosis is still not defined.