bluuebunny/crossref_metadata_2025_split huggingface dataset:
Hepatitis C in Hiv–Infected Patients With and Without Aids: Prevalence and Relationship to Patient Survival
Authors: Wright Teresa L. , Hollander Harry , Pu Xiang , Held Michael J. , Lipson Peter , Quan Stella , Polito Alan , Thaler Michael M. , Bacchetti Peter , Scharschmidt Bruce F. 
Published: March 2007

Background:Limited information is available about the prevalence of hepatitis C virus in patients with human immunodeficiency virus in relation to specific risk factors or about the influence of hepatitis C virus coinfection on survival. This retrospective study addressed these questions.Methods:The study population consisted of 512 predominantly non–intravenous drug–using male homosexuals, 224 of whom had AIDS. Samples positive for hepatitis C virus antibody by second–generation enzyme immunoassay were further tested by means of strip immunoblot assay, and for hepatitis C virus RNA by means of polymerase chain reaction amplification. A randomly selected set of enzyme immunoassay–negative samples was also tested for hepatitis C virus RNA and, if hepatitis C virus RNA positive, by a second–generation recombinant immunoblot assay.Results:The prevalence of hepatitis C virus infection unaccounted for by intravenous drug use or transfusion was 11.7% by enzyme immunoassay, and 87% of sera positive by enzyme immunoassay were also positive by second–generation recombinant immunoblot assay or hepatitis C virus RNA analysis. Hepatitis C virus RNA was detectable in 53% of enzyme immunoassay–positive samples but in only about 1% of enzyme immunoassay–negative samples. Hepatitis C virus coinfection did not influence survival of HIV–infected patients with or without manifestations of AIDS.Conclusions:Hepatitis C virus infection in nontransfused, non–intravenous drug–using patients with HIV infection is several times more prevalent than in volunteer blood donors, suggesting homosexual transmission of hepatitis C virus. About half of patients seropositive for hepatitis C virus antibody have detectable hepatitis C virus RNA, and serologically occult hepatitis C virus viremia is rare. Hepatitis C virus coinfection does not appear to adversely influence survival. (Hepatology 1994;20:1152-1155).
Shedding light into the black box of childhood cholestatic liver disease
Authors: D'Agata Lan D. A. , Jonas Maureen M. 
Published: March 2007

This work details the histologic findings in 84 liver biopsy specimens from 28 patients with progressive familial intrahepatic cholestasis (PFIC), who met the clinical criteria of early onset of chronic unremitting cholestasis, exclusion of any known metabolic or anatomic etiology, and low serum γ-glutamyl transpeptidase (GGTP) values. Hepato-canalicular cholestasis and disruption of the liver cell plate arrangement were early, uniform findings, and giant cell transformation was found in 56% of initial biopsies. Duct loss was a prominent finding; 70% of patients had ductal paucity, and many had abnormal bile duct epithelium, suggesting degeneration. Fibrosis was seen in the samples from 16 patients, including bridging fibrosis in specimens obtained from six patients during the first 2 years of life. Proliferating ductules at the margins of portal tracts increased as fibrosis progressed and were especially prominent in end-stage histology. Cirrhosis developed in nine of these patients and had a characteristic histologic pattern, consisting of biliary cirrhosis with diffuse stellate lobular fibrosis associated with severe cholestasis and pseudoacinar transformation. Mallory hyalin and hepatocellular carcinoma were observed in materials from some patients with advanced cirrhosis. The constellation of histologic findings in PFIC forms a recognizable pattern, and the liver histology appears to have a predictable progression. Key Words: Familial cholestasis—Byler disease—Giant cell hepatitis—Ductal paucity—Cirrhosis—Hepatocellular carcinoma.
The clinical findings in 33 patients with progressive familial intrahepatic cholestasis (PFIC) are presented. Symptoms developed almost invariably before 6 months of age with severe pruritus and moderate jaundice. Other clinical findings included wheezing and nosebleeds, fat-soluble vitamin deficiency states, and cholelithiasis. Lower values for γglutamyl transpeptidase, averaging 15 IU/L before the administration of phenobarbital, and cholesterol, which averaged 156 mg/dl, are helpful in distinguishing PFIC from other pediatric cholestatic liver diseases. Autosomal recessive inheritance is probable. Twenty-six patients are alive at 12.9± 6.7 years of age, all having had successful surgical treatment, either partial biliary diversion (n = 17) or orthotopic liver transplantation (n = 10). Seven patients died at a mean age of 3.9 ± 2.4 years, as a result of liver failure in two, hepatocellular carcinoma in two, and complications of liver transplantation in three. Key Words: Byler disease-Ductal paucity-Surgical therapy for cholestatic liver disease-γGlutamy1 transpeptidase.
Walking the ethical highwire: Genetic screening and hereditary tyrosinemia
Authors: Romero René , Lavine Joel E. 
Published: March 2007

Background:
Hereditary tyrosinemia type I is an autosomal recessive inborn error of metabolism caused by a deficiency of the enzyme fumarylacetoacetate hydrolase. The disorder clusters in the Saguenay-Lac-St.-Jean area of Quebec. In this region, 1 of 1846 newborns is affected and 1 of every 22 persons is thought to be a carrier. Recently, we identified a splice mutation and two nonsense mutations in the fumarylacetoacetate hydrolase gene in two patients from Quebec with tyrosinemia type I.

Methods:
We used allele-specific-oligonucleotide hybridization to examine the frequency of these three candidate mutations in patients with tyrosinemia type I and in the population of Quebec.

Results:
The splice mutation was found in 100 percent of patients from the Saguenay-Lac-St.-Jean area and in 28 percent of patients from other regions of the world. Of 25 patients from the Saguenay-Lac-St.-Jean region, 20 (80 percent) were homozygous for this mutation, a guanine-to-adenine change in the splice-donor sequence in intron 12 of the gene, indicating that it causes most cases of tyrosinemia type I in the region. The frequency of carrier status, based on screening of blood spots from newborns, was about 1 per 25 in the Saguenay-Lac-St.-Jean population and about 1 per 66 overall in Quebec.

Conclusions:
This study identified the most prevalent mutation causing hereditary tyrosinemia in French Canada; it also showed the feasibility of DNA-based testing for carriers in the population at risk. (N Engl J Med 1994;331:353-7.)
Nonalcoholic steatohepatitis: Tightening the morphological screws on a hepatic rambler
Authors: Lee Randall G. 
Published: March 2007

Background/Aims:
In the past, nonalcoholic steatohepatitis has been described mostly in obese women with diabetes. The aim of this study was to describe a series of patients with nonalcoholic steatohepatitis with a different clinical profile.

Methods:
The clinical, biochemical, and histological features of 33 patients with nonalcoholic steatohepatitis seen from July 1990 to June 1993 were analyzed.

Results:
The mean age was 47 years. All patients were antibody to hepatitis C virus-negative. Nineteen of 33 (58%) were men, 20 of 33 (61%) were non-obese, 26 of 33 (79%) had normal glucose levels, and 26 of 33 (79%) had normal lipid levels. Fourteen of 33 (42%) had normal glucose and lipid levels and were not obese. Thirteen of 33 (39%) had pathological increases in fibrosis, 5 of whom had micronodular cirrhosis. Of these 13 with severe, progressive disease, 8 (62%) were women, 8 (62%) were obese, 4 (31%) were diabetic or had an elevated glucose level, and 3 (23%) had hyperlipidemia. Although serum iron studies (transferrin saturation and ferritin) were abnormal in 18 of 31 (58%), no patient had hemochromatosis.

Conclusions:
Nonalcoholic steatohepatitis can be a severe, progressive liver disease leading to the development of cirrhosis. It should no longer be considered a disease predominantly seen in obese women with diabetes.
Hepatitis C: IIb (IV) or not IIb (IV) that is the question
Authors: Sallie Richard 
Published: March 2007

Objective:
To analyze the distribution of hepatitis C virus (HCV) genotypes among patients positive for antibody to HCV (anti-HCV) according to age, severity of liver disease, and duration of infection; to investigate the influence of HCV genotypes on response to interferon-α therapy; and to study HCV viremia levels in relation to genotypes and severity of liver disease.

Design:
Cross-sectional study.

Setting:
3 university hospitals and 2 research units.

Patients:
3 groups of French and Italian patients with chronic HCV infection and detectable serum HCV RNA: Group 1 included 35 patients with hepatocellular carcinoma; group 2, 71 patients with cirrhosis who did not have hepatocellular carcinoma; and group
THE physiology of jaundice: Molecular and functional characterization of the Crigler-Najjar syndromes
Authors: Berg Carl L. 
Published: March 2007

Crigler-Najjar (CN) disease is classified into two subtypes, type I and II. The molecular basis for the difference between these types is not well understood.
Several mutations in the bilirubin UDP-glucuronosyltransferase (B-UGT) gene of six CN type I and two CN type II patients were identified. Recombinant cDNAs containing these mutations were expressed in COS cells. B-UGT activity was measured using HPLC and the amount of expressed protein was quantitated using a sandwich ELISA. This enabled us to determine the specific activities of the expressed enzymes. All type I patients examined had mutations in the B-UGT1 gene that lead to completely inactive enzymes. The mutations in the B-UGT1 gene of patients with CN type II only partially inactivated the enzyme. At saturating concentrations of bilirubin (75 μM) CN type II patient A had 4.4 ± 2% residual activity and CN type II patient B had 38 ± 2% residual activity.
Kinetic constants for the glucuronidation of bilirubin were determined. The affinities for bilirubin of B-UGT1 expressed in COS cells and B-UGT from human liver microsomes were similar with K
m of 5.1 ± 0.9 μM and 7.9 ± 5.3 μM, respectively. B-UGT1 from patient B had a tenfold decreased affinity for bilirubin, K
m = 56 ± 23 μM.
At physiological concentrations of bilirubin both type II patients will have a strongly reduced conjugation capacity, whereas type I patients have no B-UGT activity. We conclude that CN type I is caused by a complete absence of functional B-UGT and that in CN type II B-UGT activity is reduced. (J. Clin. Invest. 1994. 94:2385-2391.) Key words: hereditary diseases · site directed mutagenesis · enzyme-linked immunosorbent assay · enzyme kinetics · enzyme activity. (Reproduced from The Journal of Clinical Investigation, 1994, vol. 94, pp. 2385-2391 by copyright permission of The Society for Clinical Investigation.
Endothelin and vascular reactivity in cirrhosis
Authors: Sieber Cornel C. 
Published: March 2007

Background/Aims:
Because the vasodilator nitric oxide is overproduced in cirrhosis, this substance may decrease pressor responses to the vasoconstrictor endothelin 1. This study aimed to examine the effects of a NO synthesis inhibitor (N
G-nitro-l-arginine methyl ester; l-NAME) on vascular responsiveness to endothelin 1 in normal and cirrhotic rats.

Methods:
Pressor dose-response curves to endothelin 1 (0.5, 1, 3, 6, and 10 μg/kg intravenously) were obtained in animals with or without pretreatment with l-NAME.

Results:
Pressor responses to endothelin 1 alone were significantly lower in cirrhotic than in normal rats. In cirrhotic animals, pressor responses to 3, 6, and 10 μg/kg of endothelin 1 were significantly higher in the presence than in the absence of l-NAME. The responses to the other doses of endothelin 1 were not affected by l-NAME. In normal rats, pressor responses to all doses of endothelin 1 were significantly higher in the presence than in the absence of l-NAME. In animals pretreated with l-NAME, pressor responses to 6 and 10 μg/kg of endothelin 1 did not differ between cirrhotic and normal rats, whereas responses to other doses remained lower in cirrhotic than in normal rats.

Conclusions:
In rats with cirrhosis, NO seems to contribute to vascular hyporeactivity to high doses but not to low doses of endothelin 1.
Closing in on a “familial cholestasis gene”
Authors: Wagstaff Joseph , Jonas Maureen M. 
Published: March 2007

It is now feasible to map disease genes by screening the genome for linkage disequilibrium between the disease and marker alleles. This report presents the first application of this approach for a previously unmapped locus. A gene for benign recurrent intrahepatic cholestasis (BRIC) was mapped to chromosome 18 by searching for chromosome segments shared by only three distantly related patients. The screening results were confirmed by identifying an extended haplotype conserved between the patients. Probability calculations indicate that such segment sharing is unlikely to arise by chance. Searching the genome for segments shared by patients is a powerful empirical method for mapping disease genes. Computer simulations suggest that, in appropriate populations, the approach may be used to localize genes for common diseases.
A locus for progressive familial intrahepatic cholestasis (PFIC), also known as Byler disease, has been mapped to a 19 cM region of chromosome 18 by a search for shared segments, using patients from the Amish kindred in which the disorder was originally described. A similar liver disease, benign recurrent intrahepatic cholestasis (BRIC), recently has been mapped to the same region, suggesting that these two diseases are caused by mutations in the same gene. Although PFIC and BRIC are clinically distinct diseases, episodic attacks of jaundice and pruritus, with elevated concentrations of bile acid in serum, are seen in both disorders. In PFIC patients, these attacks result in progressive liver damage and death. The clinical and biochemical features of PFIC and BRIC are suggestive of a defect in primary bile acid secretion. The biology of bile secretion is of great interest because of its vital importance in digestion of dietary fats as well as in secretion of xenobiotics and metabolic waste products. Cloning of the gene (or genes) responsible for PFIC and BRIC will likely provide important insights into this pathway. (Reprinted by permission of Oxford University Press).
Transjugular intrahepatic portosystemic shunt: Is it the ultimate solution for refractory ascites?
Authors: Wong Florence , Blendis Laurence 
Published: March 2007

Background.
Previous studies have suggested that the transjugular placement of an intrahepatic stent to establish a portosystemic shunt is an effective treatment of uncomplicated ascites accompanying variceal bleeding. We studied the stent shunt for use in patients with liver cirrhosis and ascites refractory to medical treatment.

Methods.
Fifty of 62 consecutive patients with cirrhosis and refractory ascites were treated with the stent shunt-an expandable stent of metallic mesh placed between a major branch of the portal vein and one of the hepatic veins. Patients were followed for a mean (±SD) of 426 ± 333 days.

Results.
The stent shunt was successfully placed in all patients and reduced the pressure gradient between the portal vein and the inferior vena cava by an average of 63 percent. Thirty-seven patients (74 percent) had complete responses (total remission of ascites within three months), and nine patients (18 percent) had partial responses. Four patients did not respond, including two who died within two weeks of shunt placement. After the procedure, 25 patients had hepatic encephalopathy, as compared with 20 patients before the procedure; although encephalopathy improved in 3 patients, new encephalopathy developed in 8 patients. In the 28 of the 33 patients followed for more than six months who were evaluated, the mean serum creatinine concentration was 1.5 ± 0.09 mg per deciliter (133 ± 8 μmol per liter) before placement of the stent shunt, 1.5 ± 1.6 mg per deciliter (133 ± 141 μmol per liter) one week after the procedure, and 0.9 ± 0.3 mg per deciliter (80 ± 27 μmol per liter) after six months (P = 0.008 for the comparison of concentrations before and six months after the procedure). Renal function did not improve in the six patients with organic kidney disease. Procedure-related complications developed in 16 patients. During follow-up, an additional 29 patients died-10 of progressive liver disease and 19 of other causes. Survival for at least one year was associated with a patient’s being under 60 years of age, having a serum bilirubin level before placement of the stent shunt of less than 1.3 mg per deciliter (22 μmol per liter), and having a complete response.

Conclusions.
Our findings in an uncontrolled prospective study suggest that the transjugular intrahepatic porto-systemic stent-shunt procedure was an effective treatment for many patients with liver cirrhosis and refractory ascites, but mortality from underlying diseases was substantial. (N Engl J Med 1995; 332:1192-7. Copyright 1995. Massachusetts Medical Society. All rights reserved).
Thrombogenesis in experimental microvascular anastomosis
Authors: Shimizu Yukio , Yasargil M. G. , Smith R. D. 
Published: March 2007

AbstractA large number of microvascular anastomoses, involving both arteriovenous and artery‐artery end‐to‐side and end‐to‐end anastomoses, were carried out in the rat to develop a temporal and morphologic profile of thrombogenesis. While excellent patency rates were obtained in purely arterial preparations, significant thrombus formation occurred in the arteriovenous preparations, both at the suture line and more distally in the vein. Factors contributing to thrombogenesis in these microvascular anastomoses are discussed.
Microsurgical revascularization of the canine corpus cavernosum penis
Authors: Gross Peter L. , Geltzeiler Jules , Kwart Arnold M. 
Published: March 2007

AbstractThe concept of vasculogenic impotence in humans is addressed by attempting to surgically create and correct vasculogenic impotence in healthy dogs. Initial experience reveals that only by extensive pelvic vascular interruption can such impotence be created and that, at best, it is only temporary because of collateralization. Microsurgical revascularization using both direct femoral artery implantation and femoral vein interposition into the corpus cavernosum penis yielded low patency rates. Further studies on this technique are needed before it can be recommended for widespread use in human beings.
Microsurgery for tubal reconstruction following falope‐ring® sterilization in swine
Authors: Rock John A. , Rosenwaks Zev , Adashi Eli Y. , Jones Howard W. , King Theodore M. 
Published: March 2007

AbstractThirteen domestic swine underwent laparotomy and sterilization using the Falope‐Ring®. The silastic band was applied to the isthmus in 6 swine, and to the isthmic‐ampullary junction in 7 swine. Tubal reconstruction was performed using the OPMI–6 Zeiss operating microscope. Pregnancy was observed in 5 (83%) of 6 swine following isthmic‐isthmic anastomosis, and in 5 (71%) of 7 swine following isthmic‐ampullary anastomosis. Overall, 10 (77%) of 13 swine conceived following tubal anastomosis. An advantage to the placement of the Falope‐Ring® in the isthmus of the swine oviduct could not be demonstrated by chi‐square analysis.
Microsurgical replantation of an incompletely amputated penis
Authors: Franklin John D. , Kirchner Fred K. 
Published: March 2007

AbstractThe use of microvascular techniques in the replantation of one partially amputated penis is presented. The advantages of these techniques are discussed. A review of the literature is included; penis‐replantation results prior to the introduction of microvascular techniques are compared to results achieved since the advent of these techniques. The superior results associated with microsurgical techniques are demonstrated, and it is concluded that microsurgical replantation is the procedure of choice in the repair of penis amputations.
Microsurgery in otology and laryngology: A review of developments during the past thirty years
Authors: Smyth G. D. L. 
Published: March 2007

AbstractThe operating microscope was originally introduced to provide better visualization and illumination for otologic surgery. The successful use of this instrument in the fenestration operation for otosclerosis rapidly led to its application to reconstructive procedures in chronic suppurative otitis media. Later, it became evident that the operating microscope also provided advantages in the treatment of inner ear disorders and in the removal of acoustic Schwannomas; a considerable reduction in morbidity and an improved prognosis have resulted from application of microsurgery to these conditions. In addition, new photographic and television equipment, used in combination with microsurgery, has greatly improved the standards of teaching and the recording of pathologic information.
Microsurgical operations in the treatment of trigeminal neuralgia
Authors: Rand Robert W. 
Published: March 2007

AbstractThree neurosurgical procedures to aid patients suffering from intractable, medically resistant tic douloureux are described: (1) the Sweet‐Nugent transcutaneous radiofrequency neurolysis of the gasserian ganglion, (2) the Gardner‐Rand suboccipital arterial decompression or rhizotomy of the trigeminal root, and (3) the Rand subtemporal transtentorial retrogasserian arterial decompression or rhizotomy of the trigeminal root. Each procedure has its indications, and the neurosurgeon should adjust his approach to the circumstances of the individual case. The clinical results with follow‐up evaluations over a 10‐year period for each of these operations are approximately the same. Ninety percent or more of patients suffering from intractable tic douloureux were completely relieved. Anesthesia dolorosa was not observed in more than 300 patients treated by these three operations, even though on rare occasions total anesthesia occurred following radiofrequency neurolysis. There was no operative mortality in the radiofrequency neurolysis series, and the operative morbidity and mortality rates were less than 4% for both the suboccipital and subtemporal craniotomy procedures.
Anatomic description of the free retroauricular flap
Authors: Wada Mitchitaka , Fujino Toyomi , Terashima Toshio 
Published: March 2007

AbstractTwenty‐five human cadavers were examined to study the anatomy of the closed vascular system between the retroauricular artery and the retroauricular vein in the skin on the posterior aspect of the auricle and the mastoid process. The mean external diameters of the vessels supplying this region are 1.2 mm (artery) and 1.4 mm (vein). A flap averaging 5 × 7 cm may be taken from this area for use as facial cover.
Autogenous artery and vein grafts in common carotid‐supraclinoid carotid anastomoses
Authors: Shields Frcs(C) Christopher B. 
Published: March 2007

AbstractMicrosurgical techniques employing either autogenous saphenous arterial grafts or venous grafts were used for common carotid‐supraclinoid carotid anastomoses (CC‐SCAs) in dogs. The CC‐SCA provides a large volume of blood to a major vessel supplying the brain. Within 10 to 14 days postoperatively, angiography and postmortem examination showed arterial patency in 9 (69%) of 13 dogs and venous patency in 2 (25%) of 8 dogs. The discrepancy in patency between the arterial and venous grafts may be the result of the differences in construction of arteries and veins, technical failure, shear stress, turbulence, and/or boundary layer separation. The application of autogenous arterial grafts (i.e., radial, hypogastric) in humans seems justified.