Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0038002 (splenomegaly)
 9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

NCPH is a heterogeneous group of liver disorders of vascular origin, leading to PHT with near normal HVPG. NCPF/IPH is a disorder of young adults or middle aged women, whereas EHPVO is a disorder of childhood. Early age acute or recurrent infections in an individual with thrombotic predisposition constitute the likely pathogenesis. Both disorders present with clinically significant PHT with preserved liver functions. Diagnosis is easy and can often be made clinically with support from imaging modalities. Management centers on control and prophylaxis of variceal bleeding. In EHPVO, there are additional concerns of growth faltering, portal biliopathy, MHE and parenchymal dysfunction. Surgical shunts are indicated in patients with failure of endotherapy, bleeding from sites not amenable to endotherapy, symptomatic hypersplenism or symptomatic biliopathy. Persistent growth failure, symptomatic and recurrent hepatic encephalopathy, impaired quality of life or massive splenomegaly that interferes with daily activities are other surgical indications. Rex-shunt or MLPVB is the recommended shunt for EHPVO, but needs proper pre-operative radiological assessment and surgical expertise. Both disorders have otherwise a fairly good prognosis, but need regular and careful surveillance. Hepatic schistosomiasis, CHF and NRH have similar presentation and comparable prognosis.
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PMID:Non-cirrhotic portal hypertension - diagnosis and management. 2397 14

Surgical resection is not indicated in patients with portal hypertension in the current guideline of Barcelona Clinic Liver Cancer (BCLC) stage. We report a systematic review and meta-analysis to determine the impact of clinically significant portal hypertension on survival in patients with hepatocellular carcinoma (HCC) following hepatectomy. Searched data in PubMed, EMBASE, and the Cochrane Library were reviewed and 11 publications were included in the meta-analysis. The inclusion criteria of clinically significant portal hypertension were esophageal varices and/or thrombocytopenia with splenomegaly. Pooled data were extracted and computed into odds ratios (ORs) for clinical outcome and hazard ratios (HRs) for overall survival. The final pooled data were composed of 2,285 patients. There were 775 patients with clinically significant portal hypertension (PHT group) and 1,510 patients without clinically significant portal hypertension (non-PHT group). Pooled proportion of mortality was 6.1% (95% confidence interval [CI] 0.032-0.116) in PHT group and 2.8% (95% CI 0.014-0.054) in the non-PHT group. The pooled proportion of morbidity was 41.7% (95% CI 0.274-0.575) in PHT group and 34.7% (95% CI 0.243-0.467) in non-PHT group. Pooled data confirmed a significantly higher postoperative mortality in the PHT group, with OR 3.02 (P < 0.001). The PHT group also demonstrated significantly higher occurrence of postoperative complications (OR 1.39, P = 0.008), liver-related morbidity (OR 3.10, P < 0.00001), and liver failure (OR 2.14, P = 0.0005) compared to the non-PHT group. According to the overall survival, pooled analysis demonstrated that the PHT group demonstrated poorer survival than the non-PHT group (HR 1.48, P = 0.007). The analyses support significantly higher rates of postoperative mortality, complications, liver-related morbidity, liver failure, and poorer overall survival in PHT group compared with the non-PHT group. Surgical resection should be selected carefully with strict surgical strategy in patients with clinically significant portal hypertension when surgical resection is planned.
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PMID:Influence of clinically significant portal hypertension on surgical outcomes and survival following hepatectomy for hepatocellular carcinoma: a systematic review and meta-analysis. 2486 54