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Portal hypertension in north Indian children
1Department of Paediatrics, All India Institute of Medical Sciences, New Delhi.
Insights
Extrahepatic portal hypertension (EHPH) is common in children and presents earlier with more frequent bleeding than intrahepatic causes. Understanding its natural history is key for effective treatment and prognosis.
Area of Science:
- Pediatric Gastroenterology
- Hepatology
- Clinical Research
Background:
- Portal hypertension in children necessitates understanding etiological factors for effective therapy and prognosis.
- Extrahepatic portal hypertension (EHPH) is a significant cause of portal hypertension in pediatric populations.
Purpose of the Study:
- To determine the etiology and clinical profile of portal hypertension in children.
- To compare the clinical presentation and outcomes of EHPH versus intrahepatic portal hypertension.
Main Methods:
- Cross-sectional observational study of 115 children under 14 years with suspected portal hypertension.
- Prospective cohort assembly to analyze etiology and clinical features.
- Assessment of splenoportal axis block and liver biopsy in chronic liver disease (CLD) patients.
Main Results:
- 76.5% of patients had EHPH; 23.5% had intrahepatic or post-hepatic causes.
- EHPH cases showed significantly earlier symptom onset (p=0.002) and more frequent bleeding (p=0.00) than intrahepatic cases.
- Commonest block site was portal vein formation; cirrhosis found in 60% of CLD patients with biopsy.
Conclusions:
- EHPH is the predominant cause of portal hypertension in this pediatric cohort.
- Clinical differences between EHPH and intrahepatic portal hypertension impact management.
- Further understanding of natural history is crucial for optimizing interventions and predicting outcomes in pediatric portal hypertension.
Abstract:
Etiological factors associated with portal hypertension in children influence the decision about therapy and the prognosis. This cross-sectional observational study was performed at a tertiary care centre in northern India from January, 1990 to December, 1994. Children below the age of 14 years with suspected portal hypertension were prospectively assembled into a cohort to determine the etiology and clinical profile of portal hypertension. Of the 115 patients with portal hypertension, 76.5% had extrahepatic portal hypertension (EHPH). Remaining 23.5% of the cases had intrahepatic and post-hepatic causes of portal hypertension. Children with EHPH had a significantly earlier onset of symptoms as compared to those with intrahepatic portal hypertension (p = 0.002) and bled significantly more frequently (p = 0.00). Forty per cent of patients with chronic liver disease (CLD) never had jaundice. History suggestive of potential etiological factors could be elicited in only 7% of EHPH patients. The commonest site of block in splenoportal axis was at the formation of the portal vein. An inverse relation of bleeding rates with duration of illness was seen in EHPH. Of the 10 CLD patients in whom liver biopsy could be done, cirrhosis was present in 6 patients. Understanding the natural history of EHPH and portal hypertension due to other etiologies may have significant implications in choosing the appropriate intervention and predicting the outcome.