Abstract:
BACKGROUND:Idiopathic portal hypertension (IPH) is a disorder of unknown etiology and is characterized clinically by portal hypertension, splenomegaly, and hypersplenism accompanied by pancytopenia. This study evaluates the pathogenic concept of the disease by a systematic review of the literature and illustrates novel pathologic and laboratory findings. CASE PRESENTATION:We report the first case of uncontrolled splenic hyperperfusion and enlargement with subsequent hypersplenism leading to life-threatening complications of IPH in infancy and emergent splenectomy. CONCLUSIONS:Our results suggest that splenic NO and VCAM-1, rather than ET-1, have a significant impact on the development of IPH, even at a very early stage of disease. The success of surgical interventions targeting the splenic hyperperfusion suggests that the primary defect in the regulation of splenic blood flow seems to be crucial for the development of IPH. Thus, beside other treatment options splenectomy needs to be considered as a prime therapeutic option for IPH.
journal_name
BMC Gastroenteroljournal_title
BMC gastroenterologyauthors
Däbritz J,Worch J,Materna U,Koch B,Koehler G,Duck C,Frühwald MC,Foell Ddoi
10.1186/1471-230X-10-122subject
Has Abstractpub_date
2010-10-20 00:00:00pages
122issn
1471-230Xpii
1471-230X-10-122journal_volume
10pub_type
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