A method for analysis of 7a-hydroxy-4-cholesten-3-one in plasma is described. Following solid-phase extraction/purification the compound is determined by high-performance liquid chromatography using a UV detector. The median concentration in healthy subjects was 12 ng/ml (range 3-W). The levels were lower in diseases associated with a low bile acid production: extrahepatic cholestasis, < 1.5 ng/ml (range < 0.9-3); liver cirrhosis < 1.5 ng/ml (range < 0.9-38), and higher in diseases associated with a high bile acid production: cholestyramine treatment, 188 ng/ml (range 54477); ileal resection 397 ng/ml (range 128-750). The levels were essentially normal in patients with colon resection. The results are consistent with a strong positive correlation between the levels of 7a-hydroxy-4-cholesten-3-one in plasma and the rate of bile acid synthesis.
In twelve females with early forms of primary biliary cirrhosis (PBC) serum lipoproteins, the intravenous fat tolerance test ( IVFTT ) and the lecithin: cholesterol acyl transferase (LCAT) rate were determined and compared to healthy controls. The cholestatic LDL (LP-X) test was negative in all cases. PBC patients had lower very low density lipoprotein TG concentrations than controls and had levels of (high-density lipoprotein) HDL-TG, -cholesterol and -phospholipids that were about 50% higher than in controls. In PBC the HDL2-cholesterol concentration was double but the HDL3-cholesterol concentration was 60% of control values (P less than 0.001 for both). The LCAT rate and the IVFTT value did not differ between the groups. A typical finding on agarose gel electrophoresis was the appearance of a slow-moving alpha-band. Several interpretations of these results are possible. In PBC the hepatic lipase activity may be impaired leading to a shift of the HDL2/HDL3 relation. The transport of HDL2 to the liver lipase site may also be affected and HDL3 production reduced due to malabsorption in the intestine.
Takotsubo cardiomyopathy (TCM) is a unique type of cardiomyopathy characterised by left ventricular systolic dysfunction in association with stressful conditions. Patients with this condition usually present with chest pain and dyspnoea, and the presentation can mimic acute coronary syndrome. We present a case of a woman aged 58 years who presented with progressive dyspnoea and cough. Her initial evaluation was suggestive of acute myocardial infarction with elevated serum troponin T and ST segment elevation. Her chest radiograph showed a large right-sided pneumothorax, which was treated with chest tube insertion. Coronary angiography and echocardiogram did not show any evidence of obstructive coronary artery disease but did show a large area of akinesis consistent with TCM. The patient was managed medically with supportive care. Her pneumothorax resolved, and her follow-up echocardiogram also showed improvement. The association between pneumothorax and TCM is rare, and only four other cases have been reported so far in the English literature.
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