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Long-term follow-up of young patients with chronic hereditary or idiopathic pancreatitis
K M Konzen1, J Perrault, C Moir
1Department of Pediatric and Adolescent Medicine, Mayo Clinic Rochester, MN 55905.
Insights
Hereditary pancreatitis (HP) in children is a more severe condition than idiopathic pancreatitis (IP). HP patients experienced more frequent complications and required more surgeries compared to IP patients.
Area of Science:
- Gastroenterology
- Pediatric Medicine
- Genetics
Background:
- Chronic pancreatitis in pediatric patients presents diagnostic and therapeutic challenges.
- Distinguishing between hereditary and idiopathic forms is crucial for prognosis.
- Previous studies have not fully elucidated the comparative clinical course of these subtypes in young patients.
Purpose of the Study:
- To compare the clinical course and outcomes of hereditary pancreatitis (HP) versus idiopathic pancreatitis (IP) in patients under 20 years of age.
- To identify differences in symptom presentation, complication rates, and need for surgical intervention between HP and IP.
- To assess the overall severity of HP in comparison to IP in a pediatric cohort.
Main Methods:
- Retrospective study of pediatric patients diagnosed with chronic pancreatitis between 1960 and 1990 at the Mayo Clinic.
- Exclusion of patients with known secondary causes of pancreatitis (viral, traumatic, alcohol-induced, hyperlipidemia).
- Comparison of clinical data between 42 patients with HP (≥2 affected family members) and 28 patients with IP.
Main Results:
- The mean age at initial assessment was lower for HP (7 years) than for IP (12 years).
- Abdominal pain was universal; vomiting was more frequent in HP.
- HP patients had significantly higher rates of complications including pseudocysts, steatorrhea, ascites, portal hypertension, and diabetes compared to IP patients.
- Surgical intervention was required in 23/42 HP patients versus 4/28 IP patients.
Conclusions:
- Hereditary pancreatitis represents a more severe variant of chronic pancreatitis in children compared to idiopathic pancreatitis.
- HP is associated with a higher burden of complications and a greater need for surgical management.
- Early identification and aggressive management strategies may be warranted for pediatric patients with hereditary pancreatitis.
Abstract:
We conducted a retrospective study of patients younger than 20 years of age who had a diagnosis of chronic pancreatitis and underwent assessment at the Mayo Clinic between 1960 and 1990. Those with a known etiologic factor for the pancreatitis (such as a virus, trauma, alcohol, or hyperlipidemia) were excluded from the study. We compared the clinical course of the 42 patients who had hereditary pancreatitis (HP)--defined as at least two family members affected by the condition--with that of the 28 patients who had idiopathic pancreatitis (IP). The mean age at initial assessment was 7 years for those with HP and 12 years for those with IP. All patients in both groups had abdominal pain. Vomiting was more frequent in patients with HP than in those with IP; otherwise the initial symptoms were similar in both groups. Patients with HP, however, had more complications, including pseudocysts (seven patients), steatorrhea (four), ascites (three), portal hypertension (two), and diabetes (one), than did patients with IP (one each had diabetes, steatorrhea, and a pseudocyst). Complications or pain necessitated surgical intervention in 23 of 42 patients with HP versus 4 of 28 patients with IP. Overall in comparison with IP, HP seems to be a more severe variant of chronic pancreatitis, inasmuch as it is associated with more frequent complications and need for surgical intervention.