Arterial embolization for ruptured adrenal pheochromocytoma.

BACKGROUND: Spontaneous rupture of adrenal pheochromocytoma is an extremely rare condition that can be lethal. Optimal treatment in these cases is still unclear. METHODS: We conducted a comprehensive review of medline articles on ruptured pheochromocytomas to locate all research done on this topic. Based on the literature review and one additional case at European Gaza Hospital, we analyzed clinical presentations, hemorrhage site, tumour side, mortality rate, and treatment options. RESULTS: In addition to our case, we identified 53 cases of ruptured pheochromocytoma. A review of all 53 cases revealed that 17 (32%) of the patients died, and that no mortality occurred among the 12 patients who received an alpha-blocker (to control high blood pressure) and fluid infusion therapy before surgery. Only 3 patients, including our case, underwent elective surgery after transcatheter arterial embolization (tae). CONCLUSIONS: Surgical treatment should be considered for ruptured pheochromocytoma. Surgical approaches involve either emergency or elective surgery. It has been reported that emergency surgery is commonly associated with a high mortality rate; no deaths were reported in patients who underwent elective surgery. We therefore consider that, if a patient has hemodynamic instability, tae can be an effective and a safe procedure for achieving hemostasis and maintaining the patient in good condition until surgery can be performed.

Highly simplified method for gas-liquid chromatographic quantitation of bile acids and sterols in human stool.

A simple method for the gas-liquid chromatographic quantitation of human fecal bile acids and sterols is described where bile acids are subjected to n-butyl ester derivatization, without prior isolation from the stool, followed by trimethylsilylation of the sterols and bile acids. Under these conditions, bile acid derivatives are well resolved from each other and from the trimethylsilyl ether derivatives of fecal sterols and no overlap occurs. The method was shown to be highly reproducible and recoveries were similar to those obtained with other methods used for fecal bile acid analysis. Application of the method for bile acid and sterol analysis in human stool is described.

Experimental studies of chlamydiosis in Japanese quails.

Two-week-old Japanese quails were infected intratracheally with six strains of Chlamydia psittaci isolated from calf pneumonia, swine pneumonia, goat abortion, sheep abortion, kid enteritis, and calf conjunctivitis, respectively. The Japanese quails from infected and control groups were closely observed for clinical symptoms. In order to examine the gross and microscopic lesions, quails in each group were sacrificed at 2, 5, 7, 10, 14, 21 and 30 days post infection. Alterations induced by pneumonic strains were more severe than those found in abortion isolates whereas the isolate of conjunctivitis failed to induce any lesion in the quail. Chlamydia psittaci was successfully recovered from lungs, spleen and intestinal contents of sacrificed quails. Calf pneumonia and goat abortion strains could be propagated in quails for a longer period (30 days), than in swine pneumonia, sheep abortion, kid enteritis (15 days each) and calf conjunctivitis isolates (7 days). The control quails were normal and no isolation could be made from them.

Capillary gas chromatographic analysis of serum bile acids as the n-butyl ester-trimethylsilyl ether derivatives.

Gas chromatographic separations of n-butyl ester-trimethylsilyl ether derivatives of several common bile acids were compared with those of the corresponding methyl ester-trimethylsilyl ether derivatives on a CP-Sil-5 CB capillary column. Both types of derivatives were similarly resolved from each other. However, the n-butyl ester-trimethylsilyl ether derivatives of the bile acids showed longer retention times than the corresponding methyl ester-trimethylsilyl ethers and unlike the methyl ester-trimethylsilyl ether derivatives, were completely resolved from and eluted later than the trimethylsilyl ethers of common plasma sterols including sitosterol. A simplified method of plasma work-up for quantitation of bile acids and application of the above method in quantification of plasma bile acids in humans is described.

Capillary gas-liquid chromatography of acetate-methyl esters of bile acids.

Gas-liquid chromatographic separations of acetate-methyl esters of several common bile acids with and without a hydroxyl group at C-6 are compared with those of the corresponding trimethylsilyl ether-methyl esters on a CP-Sil-5 CB capillary column. Unlike the trimethylsilyl ether derivatives, the retention indices of the corresponding acetates were greatly influenced by the number of hydroxyl groups in the ring system. Epimeric hydroxyl groups at carbons 6, 7 as well as 12 increased retention index of the acetate-methyl esters of the bile acids, the effect of the 7 beta-hydroxyl group being most prominent. The 6 beta-acetoxyl group increased the retention index more than the 6 alpha-acetoxy group and contrary to the trimethylsilyl ether derivatives, a 6 beta, 7 beta-diacetoxy group showed larger increase in the retention index than the corresponding 6 alpha, 7 beta-diacetoxy group. The acetate derivatives of bile acid-methyl esters show larger retention times and reduced sensitivity than the corresponding trimethylsilyl ether derivatives. However, gas chromatography of bile acid acetate-methyl esters can be very useful for the characterization of bile acids and for bile acid analysis in the rat where muricholic acids and hyodeoxycholic acid are in abundance, since these bile acids are difficult to resolve from each other and from other common bile acids as the trimethylsilyl ether derivatives.

Capillary gas-liquid chromatography of 6-hydroxylated bile acids.

Gas-liquid chromatographic separation of several bile acids with a hydroxyl group at C-6 is described on two capillary columns, CP-Sil-19 CB and CP-Sil-5 CB. The gas-liquid chromatographic retention indices of bile acids with 6 alpha- and 6 beta-hydroxyl groups are compared with those of bile acids without the C-6 hydroxyl group and the effect of the C-6 hydroxyl group on the retention indices of bile acids is determined. Both 6 alpha- and 6 beta-hydroxyl groups increase the retention index of a bile acid. The retention indices of 6 alpha- or 6 beta-hydroxyl derivatives of chenodeoxycholic acid were found to be higher than those of the corresponding 6-hydroxy derivatives of cholic acid on the CP-Sil-19 CB column but lower on the CP-Sil-5 CB column. Although all 6-hydroxylated derivatives of lithocholic, chenodeoxycholic and cholic acids were not completely resolved on either column alone, combining the two columns resulted in the complete separation of all these compounds.

Effect of long-term treatment with ursodiol on clinical and biochemical features and biliary bile acid metabolism in patients with primary biliary cirrhosis.

The effect of ursodiol on the clinical and biochemical features, serum, urinary, and biliary bile acids was investigated over a 2-yr treatment period in 14 patients with primary biliary cirrhosis (stages II-IV). Pruritus and fatigue improved, and alkaline phosphatase and liver transferases declined significantly in all patients during therapy. In four patients, less inflammation was noted by liver biopsy after 2 yr, but histology of disease did not change. Serum and urinary bile acids were increased several-fold before treatment, with cholic acid predominating. Ursodiol accounted for 30% of biliary bile acids after administration (gallstone subjects approximately 50%), and was conjugated with glycine and taurine in a ratio of 7.3:1. However, in the endogenous bile acids, the ratio increased from 1.2:1 to only 2.1:1. About 6% unconjugated bile acids were secreted into the bile (healthy controls < 1%). Thus, in patients with primary biliary cirrhosis, a larger fraction of free bile acids and a higher proportion of taurine-conjugated bile acids are secreted into the bile, compared with healthy controls. Ursodiol improves symptoms and histology with lower biliary enrichment with this bile acid.

High-performance liquid chromatographic separation of bile acids and bile alcohols diastereoisomeric at C-25.

The high-performance liquid chromatographic separation of the 25R and 25S diastereoisomers of the bile alcohols 5 beta-cholestane-3 alpha,7 alpha,26-triol and 5 beta-cholestane-3 alpha,7 alpha, 12 alpha, 26-tetrol and the bile acids, 3 alpha,7 alpha-dihydroxy-5 beta-cholestane-26-oic acid and 3 alpha,7 alpha,12 alpha-trihydroxy-5 beta-cholestane-26-oic acid is described. A Radial-Pak microBondapak C18 reversed-phase cartridge was used for the separations and elutions were carried out with acetonitrile-water-methanol-acetic acid mixtures. All eight diastereoisomeric compounds showed baseline separation when up to 200 micrograms of the isomeric mixtures were injected into the column and the method can be used for isolation of pure diastereoisomers of these bile acids and bile alcohols.

Side chain conjugation prevents bacterial 7-dehydroxylation of bile acids.

The effect of side chain conjugation on 7-dehydroxylation of bile acids has been investigated. C24-bile acids and their glycine and taurine conjugates and keto bile acids were incubated with pure strains of Eubacterium sp. VPI 12708. Bile acids of the 5 alpha- or 5 beta-series with a free terminal carboxyl group and a 3 alpha, 7 alpha-dihydroxy system were very effectively 7 alpha-dehydroxylated, whereas 7 beta-hydroxy bile acids resisted 7-dehydroxylation. Oxo bile acids were metabolized at the oxygen function also. Glycine- and taurine-conjugated bile acids were neither deamidated nor 7-dehydroxylated by the bacteria. Thus, side chain conjugation prevents 7-dehydroxylation of bile acids by Eubacterium sp. VPI 12708.

Increased plasma bile alcohol glucuronides in patients with cerebrotendinous xanthomatosis: effect of chenodeoxycholic acid.

Large quantities of C27 bile alcohols hydroxylated at C-25 are excreted in the bile and urine of patients with cerebrotendinous xanthomatosis, a lipid storage disease that results from defective bile acid synthesis. The presence of both biliary and urinary bile alcohols reflects impaired bile acid synthesis. After treatment of samples with beta-glucuronidase, plasma bile alcohols were quantitated by gas-liquid chromatography-mass spectrometry. 5 beta-Cholestane-3 alpha,7 alpha,12 alpha,25-tetrol (334 micrograms/dl) was found to be the major bile alcohol, followed by 5 beta-cholestane-3 alpha,7 alpha,12 alpha,23R,25-pentol (65 micrograms/dl), and 5 beta-cholestane-3 alpha,7 alpha,12 alpha,24(R and S),25-pentols (62.5 micrograms/dl and 64.5 micrograms/dl, respectively) in the plasma of these patients. When compared to biliary and urinary bile alcohol excretions, the plasma pattern resembled bile where 5 beta-cholestane-3 alpha,7 alpha,12 alpha,25-tetrol glucuronide predominated. In contrast, urinary bile alcohols were composed chiefly of 5 beta-cholestanepentol glucuronides with only small amounts of 5 beta-cholestane-3 alpha,7 alpha,12 alpha,25-tetrol glucuronide. Treatment with chenodeoxycholic acid, which suppresses abnormal bile acid synthesis in these patients, reduced plasma bile alcohol concentrations dramatically. These results show that large quantities of bile alcohol glucuronides, particularly 5 beta-cholestane-3 alpha,7 alpha,12 alpha,25-tetrolglucuronide, circulate in plasma of patients with cerebrotendinous xanthomatosis. The plasma bile alcohols closely resemble biliary bile alcohols which indicates their hepatic origin. The large quantities of polyhydroxylated bile alcohols in the urine may suggest their formation, at least in part, from 5 beta-cholestane-3 alpha,7 alpha,12 alpha,25-tetrol by renal hydroxylating mechanisms.(ABSTRACT TRUNCATED AT 250 WORDS)

Effect of chenodeoxycholic acid on biliary and urinary bile acids and bile alcohols in cerebrotendinous xanthomatosis; monitoring by high performance liquid chromatography.

Biliary and urinary bile alcohol and bile acid composition has been determined by high performance liquid chromatography in patients with cerebrotendinous xanthomatosis before and after treatment with chenodeoxycholic acid. Most of the bile acids and bile alcohols in the bile and urine were separated in less than 30 min using a radial pack C18 muBondapak 5 micron particle size column with a mobile phase of acetonitrile-water-methanol-acetic acid 70:70:20:1 (v/v/v/v) at a flow rate of 2 ml/min, and a refractive index detector. Before treatment, cholic acid (49%) and 5 beta-cholestane-3 alpha, 7 alpha, 12 alpha, 25-tetrol (27%) were the major biliary bile acid and bile alcohol, respectively, but were not detected in the urine of five patients. 5 beta-Cholestane-pentols were, instead, the major urinary bile alcohols with 5 beta-cholestane-3 alpha, 7 alpha, 12 alpha, 23 xi, 25-pentol (56%) predominating. Whereas 5 beta-cholestane-3 alpha, 7 alpha, 12 alpha, 24S,25-pentol was not detected in the bile, it was isolated in the urine of all patients (27%). The only urinary bile acid isolated by high performance liquid chromatography was nor-cholic acid. After 1 month of treatment with chenodeoxycholic acid, 0.75 g/day, chenodeoxycholic acid became the major bile acid in the bile of all patients (71%) along with its metabolite, ursodeoxycholic acid (21%). Cholic acid and 5 beta-cholestane-3 alpha, 7 alpha, 12 alpha, 25-tetrol were drastically reduced and were only 3% each. The excretion of 5 beta-cholestane-pentols in the urine was also drastically reduced from 130 mg/day to 15 mg/day.

Evaluation of a radioreceptor assay for beta-adrenoceptor antagonists in plasma.

A radioreceptor assay (RRA) recently developed in this laboratory for beta-adrenoceptor antagonists in plasma was evaluated in normal volunteers and compared with a radioimmunoassay (RIA) for propranolol. The RRA depends upon the ability of beta-adrenoceptor antagonists to complete with a radiolabelled ligand for beta-adrenoceptor binding sites on lung membranes. Unlike other assays, it measures biologically active drugs including active metabolites of the parent compound. In volunteers given a single oral dose of (+/-)-propranolol, considerable differences between the two assay methods were demonstrated. In other experiments this difference was shown to relate to the RIA's sensitivity to the inactive (+)-isomer of propranolol and possibly to inactive metabolites. The facility of the RRA in measuring plasma levels of several other non-selective beta-adrenoceptor antagonists was also demonstrated. By employing (-)-propranolol as the standard in the RRA, all of these drugs can be directly compared with a single and relatively simple assay technique.

Measurement of beta-adrenoceptor antagonists in biological fluids using a radioreceptor assay.

A new assay for plasma levels of beta-adrenoceptor antagonists is described. The assay depends upon the ability of the drug to compete with a labelled beta-adrenoceptor antagonist (-)-[3H]dihydroalprenolol for beta-adrenoceptor binding sites on lung membranes. The assay is simple to perform, very sensitive and does not require prior extraction of plasma. The assay can also detect bioactive metabolites of these agents and is clearly not limited to a single beta-adrenoceptor antagonist.