Living domino liver transplantation in an adult with congenital absence of portal vein.

Congenital absence of the portal vein (CAPV) is a rare malformation of the splanchnic venous system. Although CAPV is usually detected in the pediatric age group, our patient was a 35-year-old woman. She had been diagnosed with CAPV in 1996 when she was 27 years old. In 1998, she was placed on hemodialysis due to chronic renal failure. After several episodes of encephalopathy in 2002, liver transplantation (LT) was recommended to her and her family. Since there was no suitable living donor candidate, she was put on the waiting list for a deceased donor liver transplant in Japan. In 2004, her ammonia level increased to around 300 microg/dl, and she went into a coma lasting for three days. After recovering from this event, she underwent a living domino transplantation using a whole liver donated by a familial amyloid polyneuropathy (FAP) patient. Her portal vein, which had drained directly into the inferior vena cava (IVC), was transected together with a cuff of the IVC wall and anastomosed to the graft liver portal vein in an end-to-end fashion. In conclusion, liver transplantation proved to be a safe and effective way to save this patient and improve her quality of life.     

Relationship Between Exhaled Carbon Monoxide and Airway Hyperresponsiveness in Asthmatic Patients

The study objectives were to analyze the changes in exhaled carbon monoxide (COex) induced by histamine provocation challenge in asthmatic patients and to evaluate the relationship between COex and airway sensitivity and reactivity. Levels of COex were measured in 105 nonsmoking mildly asthmatic subjects before and after histamine provocation challenge. Dose-response curves were characterized by their sensitivity (PD₂₀) and reactivity. Dose-response slope (DRS), continuous index of responsiveness (CIR), and bronchial reactivity index (BRI) were determined as reactivity indices. Bronchial challenge was positive for 47 subjects and negative for 58. The COex levels rose significantly after bronchial challenge in the positive response group (4.49 ± 0.4 vs. 5.74 ± 0.57 ppm, p = 0.025) and in the negative response group (2.84 ± 0.25 vs 4.00 ± 0.41 ppm, p = 0.000). An inverse relation between basal COex and PD₂₀ was found (r = - 0.318, p = 0.030). In all subjects, a proportional direct relationship between COex and DRS (r = 0.214, p = 0.015), CIR (r = 0.401, p = 0.000), and BRI (r = 0.208, p = 0.012) was observed. On stepwise multiple linear regression analysis, COex only significantly correlated with CIR (multiple r² = 0.174, p = 0.000). In conclusion, exhaled CO determination is a noninvasive inflammatory marker of the respiratory tract, which shows an acceptable association with airway hyperresponsiveness.     

Hypernephroma associated with multiple endocrine neoplasia type I: a case report

We report a case of multiple endocrine neoplasia type I and hypernephroma. Parathyroid hyperplasia, adrenocortical hyperplasia, a nodular goiter, multiple lipomas, a chromophobe adenoma of the pituitary and hypernephroma had all been diagnosed previously. All but the last are features consistent with the diagnosis of multiple endocrine neoplasia type I (Wermer's syndrome). The association of multiple endocrine neoplasia type I and hypernephroma may represent a new manifestation of this pleiotropic syndrome.     

Modulatory effects of progesterone upon dopamine release from the corpus striatum of ovariectomized estrogen-treated rats are stereo-specific

We have shown previously that progesterone linked with bovine serum albumin at position 3 of the progesterone molecule (P-3-BSA) was effective in modulating (both stimulating and inhibiting) amphetamine (AMPH)-evoked dopamine (DA) release in vitro from superfused corpus striatal (CS) tissue fragments of ovariectomized estrogen-primed rats. To examine the stereospecificity of this effect, progesterone linked with bovine serum albumin at position 11 (P-11-BSA) and deoxycorticosterone linked with bovine serum albumin at position 21 (DOC-BSA) were tested for their ability to modulate amphetamine-stimulated DA release in vitro when administered in either a pulsatile or a continuous infusion mode at different doses. The results demonstrate that a specific orientation of the P-BSA molecule is required for differential modulation of AMPH stimulated DA release from the CS of ovariectomized estrogen-treated rats, since DOC-BSA was ineffective and P-11-BSA showed only a stimulatory effect.     

Early and late results of orthotopic liver transplantation

Between 4/1986 to 1/1989, 74 orthotopic liver transplantation were performed in 62 patients (62 first liver transplants, 10 as second graft and two as a third graft); 57 in adults and 17 in children. The main indication for the operation was liver cirrhosis (61.4%) (the most frequent etiology was alcoholic cirrhosis, 28.5%). Six cirrhotic patients had a hepatocarcinoma (9.6%). Two received a liver and kidney transplant due to terminal renal insufficiency and hemodialysis. The most frequent indication in children was biliary atresia (33.3%). Six patients had a fulminal liver failure (9.6%). AB0 blood group compatibility was identical in 87.5%, compatible in six and incompatible in three patients. Total orthotopic liver transplantation was performed in 67 patients, and size-reduced liver was indicated in 7 patients. Extracorporeal veno-venous bypass was used in adults but never in children. In 93.1% of the transplants a single hepatic artery was anastomosed to the recipient and in 6.9% a double anastomosis was performed. In 62.5% of the patients a end-to-end choledocho-choledochostomy was performed and in 34.8% hepatico-jejunostomy was indicated. Three months postoperative mortality rate was 12.9%. Arterial stenosis and thrombosis were the most frequent complication.     

The bioavailability and nonlinear pharmacokinetics of MK-679 in humans

MK-679 (R(?)-3-((3-(2-(7-chloro-2-quinolinyl)ethenyl)phenyl)(3-(dimethylamino)-3-oxopropyl)thio)methyl)thio(propanoic acid) is a potent and specific LTD₄-receptor antagonist. The disposition of MK-679 was investigated in a three-way crossover study in 12 healthy males receiving single intravenous doses of 75, 250, and 500 mg of MK-679. A greater than proportional increase in the area under the plasma concentration—time curve of MK-679 was observed with increase in dose. The plasma concentration data for each subject fitted well to the differential equations for a two-compartment model with linear tissue distribution and Michaelis-Menten elimination from the central compartment, indicating that the elimination of MK-679 in humans is saturable. In a previous study, the disposition of MK-679 in humans was also dose-dependent when given together with its S(+)-isomer, L-668,018. Thus, the disposition of MK-679 in humans is dose-dependent regardless of the presence of its stereoisomer. Also, the bioavailability of MK-679 was determined in six healthy males receiving simultaneously an oral dose of 250 mg of MK-679 and intravenous infusion of 1 mg ¹⁴C-MK-679. Results of this study indicate that the oral bioavailability of MK-679 is nearly quantitative.     

Stimulation of energy intake and growth by saccharin in rats

Rats fed wet diets containing saccharin consumed 12-14% more energy and gained 24-55% more weight than rats fed the same diets without saccharin. Saccharin-induced stimulation of intake was usually not so pronounced during the first week as during subsequent weeks of each experiment. Similar results were obtained using diets high in starch and fat. However, these effects could be obliterated by simply exposing the rats to unsweetened (plain) diet or to saccharin in water for several days before the sweetened diets were introduced. Furthermore, although stimulation of intake by saccharin was observed with diets containing 80% water, no such effect was observed with a diet containing 60% water. Rats given low-energy sweetened water in addition to their 80% water diet consumed substantially more fluid but not more or less energy than rats given unsweetened water. Preference tests suggest that saccharin increases diet palatability only very slightly; this finding is one of several observations suggesting that stimulation of intake by saccharin cannot be interpreted in terms of increased diet palatability. These results suggest that dietary hyperphagia results from the interaction between innate and learned responses to the taste of foods. Osmotic factors did not seem to exert a major influence in these experiments.     

Terodiline in the treatment of women with urgency and motor urge incontinence. A clinical and urodynamic double-blind cross-over study

In a consecutive double-blind cross-over study, 18 females with detrusor instability were treated with an anticholinergic and calcium antagonistic agent terodiline 25 mg bid or placebo for 3 weeks; cross-over took place after a wash-out period of 1 week. The efficacy of the drug was investigated at the end of each treatment period by means of drug preference, micturition charts, pad usage, bladder and urethral mucosal perception threshold and bulbocavernosus reflex latency measurement. Serum levels of terodiline were estimated at the end of each treatment period and all side effects were reported. The preference for the drug was statistically significant (P less than 0.01): 14 patients preferred the drug, one preferred the placebo and three had no preference. A small but statistically significant reduction was found in 24-h micturition frequency (P less than 0.05). Cystometry showed an increase in volume at first sensation, an increase in volume at detrusor contraction and a tendency towards an increase in bladder capacity, whereas detrusor contraction pressure was unchanged. Median serum levels of the drug were 559 ng/ml (range 203-1117). No serious side effects were reported. It was concluded that terodiline should be considered as an alternative drug in the treatment of motor urge incontinence.