| Sinusoidal obstruction syndrome ( SOS ) is a syndrome of portalhypertension mostly resulted from sinusoidal outflow tract obstruction followed sinusoidal endothelial cell injury. The originally described cases of SOS were due to ingestion of senecio, its classic clinical features were hyperbilirubinemia, right upper quadrant pain of liver orgin or hepatomegaly, and weight gain, the most striking histological feature was occlusion of the central veins, an it was subsequently named hepatic venoocclusive disease. Clinical studies have demonstrated that involvement of the central veins is not essential to the disease, and experimental studies have shown that the disease process originates in the sinusoids. Based onthese findings, investigators in this field have proposed renaming this sinusoidal obstruction syndrome. SOS is infrequent and its clinical symptoms are non-specific, so it is difficult to diagnose SOS and to distinguish it from other diseases. Studies for diagnosis and differential diagnosis of SOS is insufficient, this study is to investigate the means for diagnosis and treatment of SOS.METHODSThe data of 13 patients, including etiopathogenisis, clinical manifestation, laboratory result, imaging, pathology, and the course of diagnosis and treatment were reviewed. All cases were followed up.RESULTSNine cases were resulted from gynura segetum and 2 cases were complicated from bone marrow transplantation. The chief symptoms included abdominal distention(13/13), hypodynamia(ll/13), anorexia( 11/13), hepatalgia(ll/13), diarrhea(6/13), and fever(2/13). The findings from physical inspection included signs of ascites(13/13), hepatomegaly with tenderness(ll/13), splenomegaly(7/13), edema of lower limbs(6/13), ictericsclera(6/13), weight gain(5/13), and subcutaneous varicos vein of abdominal wall(4/13). There were 6 patients occurred hepatalgia, abdominal distention, and jaundice(hyperbilirubinemia) in order. There were mild or midrange hepatic function damage in 11 patients and heavy damage in 2. All patients' serum aspartate aminotransferase level was higher than alanine aminotransferase and serum-ascites albumin gradient(SAAG) exceeded ll.lg/L. The CA125 levels of 9/10 patients' serum and ascites elevated significantly. All patients' ultrasonography(US) showed hepatomegaly and portal hypertension, 10 cases showed hepatic veins attenuated and 6/11 cases showed reverse blood flow in portal vein. 9/9 patients' magnetic resonance imaging(MRJ) showed that contrast agent accumulated unevenly in parenchyma of liver in both portal period and lag period, but filled poorly in hepatic veins. Per cutem liver biopsy showed that 8/8 patients' hepatic sinusoids were congested and only 3/8 cases had venular occlusion. 10/13 cases had been misdiagnosised. One patient healed after transplantation of liver. Six patients recovered gradually by treatments of Heparin, PGE1, and so on. One patient has not been recovered yet and five patients died.CONCLUSIONSGynura segetum and bone marrow transplantation are common causes of SOS. Outstanding signs of portal hypertension and lighter liver functionallesion should be the clinical characteristics of SOS, occurrence of hepatalgia, abdominal distention, and jaundice(hyperbilirubinemia) in order is the typical manifestation of SOS. The CA125 level of SOS patients' serum and ascites often elevates significantly, and the ascites mostly results from portal hypertension. Sonogram features of SOS are portal hypertension and attenuating of hepatic vein without stenosis, accumulating of contrast agent unevenly in liver but poor filling in hepatic veins are features on MRI. The misdiagnosis rate of SOS is quite high, US and MRI have significant value in differential diagnosis, but the value of per cutem liver biopsy is limited, combination of imaging and pathology should contribute to the correct diagnosis of SOS. Application of anticoagulant in early course is very important, live transplantation should be considered in severe cases.
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