부산대학교 도서관

Purpose of the study Idiopathic non-cirrhotic portal hypertension (INCPH) has been reported increasingly in patients with chronic HIV infection. However, several aspects of this disorder remain to be elucidated. The aim of our study was to evaluate the prevalence and risk factors of HIV associated INCPH. Methods All adult HIV patients attending the outpatient clinic between February and September 2011 underwent sonographical spleen size determination and assessment of portosystemic collaterals. Patients with splenomegaly underwent an extensive ultrasound examination. Gastroscopy was performed when additional signs of portal hypertension or collaterals were observed. All children with HIV infection underwent extensive ultrasound examination. INCPH was diagnosed according to the general definition. Differences between INCPH cases (group 1) and HIV patients treated with didanosine (ddI) without portal hypertension (group 2) were assessed at HIV diagnosis, start of ddI and INCPH diagnosis. Summary of results Four out of 1010 screened adult HIV patients were diagnosed with INCPH (prevalence of 4‰). Hundred of the 1010 screened patients were treated with ddI. All INCPH patients were treated with ddI, representing an INCPH prevalence of 4% in patients exposed to ddI. In The Netherlands, 7000 patients were treated with ddI and only 17 are diagnosed with INCPH, suggesting underdiagnosis in 260 patients. No differences in clinical characteristics predictive for the development of INCPH could be demonstrated between group 1 and group 2 at HIV infection or start of ddI treatment. INCPH patients were treated longer with ddI [86 vs. 51 months, p<0.01] and concomitant treatment with ddI and stavudine [21 vs. 4 months, p<0.01]. Corrected for age and duration of follow-up of HIV, active protein c [0.64 vs. 1.13, p<0.01] and active protein s [0.67 vs. 1.01, p<0.01] levels were lower in the INCPH group. None of the 38 children with HIV infection were diagnosed with INCPH. None of these children were ever treated with ddI. Conclusions In our study HIV-associated INCPH only occurred in patients exposed to ddI. Awareness for this disorder is warranted considering the suggested underdiagnosis based on these study results. Risk factors are long term treatment with ddI and concomitant treatment with stavudine and screening for signs of portal hypertension in this subgroup may be recommended. A possible pathophysiological role for thrombophilia remains to be elucidated. [ABSTRACT FROM AUTHOR]