Hepatomegaly and jaundice as the presenting symptoms of systemic light-chain amyloidosis: A case report.

BACKGROUND: Light chain (AL) amyloidosis is a plasma cell dyscrasia characterized by the pathologic production and extracellular tissue deposition of fibrillar proteins derived from immunoglobulin AL fragments secreted by a clone of plasma cells, which leads to progressive dysfunction of the affected organs. The two most commonly affected organs are the heart and kidneys, and liver is rarely the dominant affected organ with only 3.9% of cases, making them prone to misdiagnosis and missed diagnosis. CASE SUMMARY: A 65-year-old woman was admitted with a 3-mo history of progressive jaundice and marked hepatomegaly. Initially, based on enhanced computed tomography scan and angiography, Budd-Chiari syndrome was considered and balloon dilatation of significant hepatic vein stenoses was performed. However, additional diagnostic procedures, including liver biopsy and bone marrow-examination, revealed immunoglobulin kapa AL amyloidosis with extensive liver involvement and hepatic vascular compression. The disease course was progressive and fatal, and the patient eventually died 5 mo after initial presentation of symptoms. CONCLUSION: AL amyloidosis with isolated liver involvement is very rare, and can be easily misdiagnosed as a vascular disease.

Non-cirrhotic portal hypertension secondary to cholangiointestinal anastomotic stricture after choledochal cyst excision: a case report.

Background: Cystectomy accompanied by biliary system reconstruction is an important treatment option for choledochal cysts, but the risk of post-operative complications is high. The most famous long-term complication is anastomotic stricture, whereas non-cirrhotic portal hypertension secondary to cholangiointestinal anastomotic stricture is rare. Case summary: Here we report the case of a 33-year-old female patient with a type I choledochal cyst who underwent choledochal cyst excision with Roux-en-Y hepaticojejunostomy. Thirteen years later, the patient presented with severe esophageal and gastric variceal bleeding, splenomegaly, and hypersplenism. Furthermore, cholangiointestinal anastomotic stricture with cholangiectasis was identified on imaging. A pathological examination of the liver suggested intrahepatic cholestasis, but the fibrosis was mild and inconsistent with severe portal hypertension. Therefore, the final diagnosis was portal hypertension secondary to a cholangiointestinal anastomotic stricture after choledochal cyst surgery. Fortunately, the patient recovered well after endoscopic treatment and dilated cholangiointestinal anastomotic stricture. Conclusion: Choledochal cyst excision with Roux-en-Y hepaticojejunostomy is the recommended standard of care for type I choledochal cysts; however, the long-term risk of cholangiointestinal anastomotic stricture requires consideration. Moreover, cholangiointestinal anastomotic stricture can lead to portal hypertension, and the degree of elevated portal pressure may be inconsistent with the degree of intrahepatic fibrosis.

Isolated gastric variceal bleeding related to non-cirrhotic portal hypertension following oxaliplatin-based chemotherapy: A case report.

BACKGROUND: Sinusoidal obstruction syndrome has been reported after oxaliplatin-based chemotherapy, but liver fibrosis and non-cirrhotic portal hypertension (NCPH) are rarely reported. CASE SUMMARY: Here, we describe the case of a 64-year-old woman who developed isolated gastric variceal bleeding 16 mo after completing eight cycles of oxaliplatin combined with capecitabine chemotherapy after colon cancer resection. Surprisingly, splenomegaly and thrombocytopenia were not accompanied by variceal bleeding, which has been reported to have predictive value for gastric variceal formation. However, a liver biopsy showed fibrosis in the portal area, suggesting NCPH. The patient underwent endoscopic treatment and experienced no further symptoms. CONCLUSION: It is necessary to guard against long-term complications after oxaliplatin-based chemotherapy. Sometimes splenic size and platelet level may not always accurately predict the occurrence of portal hypertension.

[Influence of pulse indicator continuous cardiac output in monitoring on the volume parameters of different tidal volume under pressure control ventilation mode and volume-controlled ventilation mode in sheep].

OBJECTIVE: To compare the influence of pulse indicator continuous cardiac output (PiCCO) in monitoring tidal volume (V(T)) under pressure control ventilation mode and volume-controlled ventilation mode in sheep. METHODS: After anesthesia and tracheotomy, 5 sheep, which were apneic and receiving mechanical ventilation. Twenty minutes later, central venous pressure (CVP) and cardiac function were monitored with different selected V(T) levels of 6, 10, 15, 20 ml/kg under bi-level positive airway pressure (BiPAP) mode by changing the pressure of inspiration, or under the synchronized intermittent mandatory ventilation (SIMV) mode with the same ventilation conditions. RESULTS: In both modes, the increase in V(T) led to an decrease of cardiac index (CI) and intrathoracic blood volume index (ITBVI), reaching a statistically significant difference at 15 ml/kg [SIMV mode: CI (3.94 + or - 1.03) L x min(-1) x m(-2), ITBVI (707 + or - 105) ml/m(2); BiPAP mode: CI (4.11 + or - 1.11) L x mi(-1) x m(-2), ITBVI (715 + or - 122) ml/m(2)] and 20 ml/kg [SIMV mode: CI (3.87 + or - 1.04) L x min(-1) x m(-2), ITBVI (705 + or - 116) ml/m(2); BiPAP mode: CI (3.64 + or - 0.96) L x min(-1) x m(-2), ITBVI (694 + or - 114) ml/m(2)] compared with 6 ml/kg [SIMV mode: CI (4.96 + or - 1.58) L x min(-1) x m(-2),ITBVI(811 + or - 169) ml/m(2); BiPAP mode:CI(5.67 + or - 1.96) L x min(-1) x m(-2), ITBVI (823 + or - 182) ml/m(2), all P<0.05]; an increase in systemic vascular resistance index (SVRI) and mean airway pressure (Pmean) at 15 ml/kg [SIMV mode: SVRI (237.6 + or - 56.2) kPaxs(-1) x L(-1), Pmean (14.0 + or - 3.2) cm H(2)O (1 cm H(2)O=0.098 kPa); BiPAP mode: SVRI (230.8 + or - 32.9) kPaxs(-1) x L(-1), Pmean (13.0 + or - 2.2) cm H(2)O] and 20 ml/kg [SIMV mode: SVRI (253.1 + or - 76.7) kPaxs(-1) x L(-1), Pmean (18.2 + or - 4.8) cm H(2)O ; BiPAP mode: SVRI (246.7 + or - 48.8) kPaxs(-1) x L(-1), Pmean (16.8 + or - 3.3) cm H(2)O] compared with 6 ml/kg [SIMV mode: SVRI (184.8 + or - 47.5) kPaxs(-1) x L(-1); Pmean (8.8 + or - 1.6) cm H(2)O; BiPAP mode: SVRI (184.5 + or - 51.5) kPaxs(-1) x L(-1), Pmean (8.6 + or - 0.5) cm H(2)O, all P<0.05]; but there was no significant effects on CVP, heart rate (HR), mean blood pressure (MBP). There was no significant difference of CI, ITBVI, SVRI and Pmean between the two ventilation modes with various V(T) levels. CONCLUSION: When the cardiac function was normal, the increase in V(T) led to a decrease of CI and ITBVI, but it had no significant effects on CVP. There was no significant difference of CI and ITBVI in the two ventilation modes, both were decreased. So a relatively constant V(T) should be maintained in determining ITBVI.

[Clinical study of survival time for chronic liver failure.].

OBJECTIVE: To identify independent risk factors influencing the survival time of patients with chronic liver failure and construct a predictive model. METHODS: Retrospective analysis was applied to clinical data of 362 patients with chronic liver failure treated with artificial liver in Tianjin third centre hospital between May 2002 and May 2007. Data were analyzed with SPSS 13.0 statistic software, t test and rank test were used on quantitative data, chi-square test was used on qualitative data, Cox regression analysis was used to select the independent risk factors influencing the survival time. According to independent risk factors from Cox regression model, a prognostic model was established. RESULTS: 1. Independent risk factors (P less than 0.05) influencing the survival time were: Child-Pugh score, bilirubin separation ALT, ascites, arginine, age, tyrosine and serum sodium. 2. By receiver operating characteristic curves (ROC) analysis, the area under ROC (AUR) to predict the outcome of chronic liver failure patients was 0.782, and the cutoff score was 27.69. CONCLUSIONS: 1. Child-Pugh score, bilirubin separation ALT, ascites, arginine, age, tyrosine and serum sodium are independent risk factors affecting survival time of patients with chronic liver failure. 2. Cox model we constructed can reliably predict the survival time of patients with chronic liver failure.