Influence of ßS-Globin Haplotypes and Hydroxyurea on Arginase I Levels in Sickle Cell Disease.

Introduction. Sickle cell disease (SCD) is characterized by hemoglobin S homozygosity, leading to hemolysis and vasoocclusion. The hemolysis releases arginase I, an enzyme that decreases the bioavailability of nitric oxide, worsening the symptoms. The different SCD haplotypes are related to clinical symptoms and varied hemoglobin F (HbF) concentration. The aim of this study was to evaluate the impact of the ßS gene haplotypes and HbF concentration on arginase I levels in SCD patients. Methods. Fifty SCD adult patients were enrolled in the study and 20 blood donors composed the control group. Arginase I was measured by ELISA. The ßS haplotypes were identified by polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP). Statistical analyses were performed with GraphPad Prism program and the significance level was p < 0.05. Results. Significant increase was observed in the arginase I levels in SCD patients compared to the control group (p < 0.0001). The comparison between the levels of arginase I in three haplotypes groups showed a difference between the Bantu/Bantu × Bantu/Benin groups; Bantu/Bantu × Benin/Benin, independent of HU dosage. An inverse correlation with the arginase I levels and HbF concentration was observed. Conclusion. The results support the hypothesis that arginase I is associated with HbF concentration, also measured indirectly by the association with haplotypes.

Micro-reactors for characterization of nanostructure-based sensors.

Fabrication and testing of micro-reactors for the characterization of nanosensors is presented in this work. The reactors have a small volume (100 µl) and are equipped with gas input/output channels. They were machined from a single piece of kovar in order to avoid leaks in the system due to additional welding. The contact pins were electrically insulated from the body of the reactor using a borosilicate sealing glass and the reactor was hermetically sealed using a lid and an elastomeric o-ring. One of the advantages of the reactor lies in its simple assembly and ease of use with any vacuum/gas system, allowing the connection of more than one device. Moreover, the lid can be modified in order to fit a window for in situ optical characterization. In order to prove its versatility, carbon nanotube-based sensors were tested using this micro-reactor. The devices were fabricated by depositing carbon nanotubes over 1 µm thick gold electrodes patterned onto Si/SiO(2) substrates. The sensors were tested using oxygen and nitrogen atmospheres, in the pressure range between 10(-5) and 10(-1) mbar. The small chamber volume allowed the measurement of fast sensor characteristic times, with the sensors showing good sensitivity towards gas and pressure as well as high reproducibility.

[Primary sclerosing cholangitis in children and adolescents]. / Colangite esclerosante primária em crianças e adolescentes.

Primary sclerosing cholangitis is a disease usually with a poor prognosis with a mean survival of 10-12 years, starting from the time of diagnosis. Among the different populations studied, males predominate in a ratio 2:1 with ages ranging from 20-78 (mean 42 years). This article describes four patients with this syndrome, a child of eight and three adolescents, thirteen and fourteen years old. It is possibly of autoimmune etiology, characterized by stenosis, dilatation and fibrosis involving the intrahepatic and extrahepatic biliary tree. We analyzed laboratory assays, clinical, immunological, laboratory assays, histological and cholangiographic aspects. In conclusion, in this age group perhaps of the small population sample there was no sex predominance, and 50% progressed without jaundice and without hepatomegaly. The serum levels of gamma glutamyltransferases and especially alkaline phosphatase were elevated compared to aminotransferases without increase of the serum auto antibodies. Only one patients displayed the typical histological appearance of primary sclerosing cholangitis. The diagnosis in al was confirmed by typical cholangiographic signs, restricted only to the intrahepatic biliary tree, identified in all those included in this study.

[Treatment of hemorrhagic congestive gastropathy with TIPS implantation. Pilot study]. / Tratamento da gastropatia congestiva hemorrágica através do implante do TIPS. Estudo piloto.

Minimally invasive percutaneous procedures has an important place in internal medicine. In this concept is included a transjugular intrahepatic portosystemic shunts (TIPS). In this pilot study we describe the indication of this therapeutic modality in three cirrhotic patients with hemorrhagic congestive gastropathy unresponsive to infusion of drugs that reduce the portal pressure. All of them were treated by TIPS and control of gastric bleeding occurred in all. One of these showed partial stenosis of the wallstent, treated by prosthesis dilatation. Another developed portosystemic encephalopathy controlled by usual therapeutic measures in the other we implanted two TIPS to obtain a low pressure level of the hepatic-portal gradient. They had no further necessity of blood transfusion, with better hemodynamic conditions and biochemical tests with reduction of the values of the GHP. We conclude that TIPS may be useful to treat hemorrhagic congestive gastropathy acting like a bridge for subsequent elective liver transplantation under good clinical and hemodynamic conditions.

[Reversal of hepatorenal syndrome after liver transplantation. Considerations about 3 patients]. / Reversão da síndrome hepatorrenal após transplante de fígado. Considerações sobre três pacientes.

Hepatorenal syndrome was observed in 25% of the patients with end stage liver disease. In this situation they stayed longer time in intensive unit treatment, hemodialysis. This evolution was observed in 67% of them with this complication after surgery. In this report we describe three patients with hepatorenal syndrome reversible after liver transplant. We define the form of management of these patients after surgery, regard treatment with cyclosporine A, only 72 h after surgery, without administration of aminoglycosides and amphoterycin. This good evaluation also depends of absence of arterial hypotension or infection episodes during and after surgery. It also depends of the good conditions of the implanted liver.

[Transjugular intrahepatic portosystemic shunts (TIPS) as a bridge for liver transplantation]. / Anastomose portossistêmica intra-hepática (TIPS) como ponte para o transplante de fígado.

Transjugular intrahepatic portosystemic shunts (TIPS) represents a new surgical technique minimally invasive utilized in the treatment of portal hypertension. Such technique avoid the risks of general anesthesia, and major surgery like portocava anastomosis, reducing the hepatic-portal gradient, and help bleeding esophagogastric varices, hemorrhagic congestive gastropathy and refractary ascites. Certainly diminishes the intensity of intraperitoneal colorectal circulation and the necessity of blood transfusion during surgery for liver transplantation. In this report we reported the first case in Brazil of the implant of TIPS like preparation for liver transplantation in cirrhotic alcoholic patient. We made consideration about techniques aspects and surgery evolution. This new interesting technique applied for this type of patients is indicated as a bridge for candidates for liver transplantation.

[Ultrasonically guided needle biopsy in malignant focal solid nodules of the liver]. / Biopsia percutânea guiada por ultra-som em lesões nodulares sólidas focais malignas do fígado.

In the most of the cases, the diagnosis of focal solid hepatic lesions are performed by ultrasonography, computed tomography, magnetic resonance and hepatic angiography imagig. However, the distinction between benign and malignant neoplasias, sometimes is made after liver biopsy. This report is about 32 of these lesions, diagnosed after guided liver biopsy by ultrasonography. The efficacy of this propedeutic method, minimally invasive, is defined emphasizing that there are no mortality and low levels of morbidity.

Cholelithiasis in cirrhotic patients. (Analysis of cholelithiasis among patients with liver cirrhosis in São Paulo, Brazil).

During the past 20 years, several authors have reported increased prevalence of cholelithiasis in liver cirrhosis. This biliary disease has been implicated with the deterioration of liver function, liver disease of alcoholic origin or even the presence of hypersplenism in this patient population. This study analyzes the incidence and possible factors which are responsible for promoting cholelithiasis in cirrhosis. The study included 110 cirrhotic patients of a private center specialized in treating liver diseases. The incidence of cholelithiasis was 27.3% (25.3% in males and 33.3% in females). There was no correlation between liver function defined by Child's classification or through the laboratory examinations (AST, ALT, AP, GGT, PT, Alb, TB, DB, PA) and the presence of gallstones. No evidence was found that the etiology of cirrhosis or the presence/absence of hypersplenism affected the prevalence of cholelithiasis in this population. In conclusion, an increased prevalence of cholelithiasis was verified in this population of cirrhotics but the pathogenesis is still obscure.

[Hepatic hemangioma. Analysis of 103 cases. Clinical considerations and imaging methods]. / Hemangiomas hepáticos. Análise de 103 casos. Considerações clínicas e dos métodos de imagem.

Hemangiomas are the most common benign vascular tumor found in the liver. In this study, 103 cases of hepatic hemangiomas were analysed in a retrospective manner. Image aspects such as those seen in ultrassonography, computed tomography, nuclear magnetic resonance and arteriography are discussed. The incidence was higher amongst the female sex, between individuals from 40 to 60 years old, which presented predominantly without symptoms (66%) and localized mainly in the right hepatic lobe (74%). The meaning of those aspects, when adopted, permits the clinicians to differentiate benign from malignant nodular lesions in the liver.

Abscessos piogênicos do fígado: análise de 36 casos tratados por punçäo percutânea / Pyogenic liver abscesses: analysis of 36 cases treated by percutaneous drainage

Analisaram-se 36 casos de abscessos piogênicos do fígado tratados por drenagem percutânea associada à antibioticoterapia. Os sintomas predominantes forma febre (75%) e dor (72,2%), e os sinais clínicos mais freqüentes, hepatomegalia (58,3%) e icterícia (33,3%). O tempo médio entre o início dos sintomas e o diagnóstico foi de 16 dias, e a tomografia computadorizada e a ultra-sonografia eficazes em todos os casos. Para a drenagem percutânea realizou-se apenas uma punçäo em 19 casos, duas em 8, três em 8 e mais de quatro em apenas um paciente. Observaram-se três casos de hemoperitônio (8,3%), um deles submetido a laparotomia. O período médio de internaçäo foi de 24,8 dias, com mortalidade de 5,5%. Conclui-se ser a drenagem percutânea associada à antibioticoterapia terapêutica pouco invasiva e eficaz no tratamento dos abscessos piogênicos do fígado

[Pyogenic liver abscesses: analysis of 36 cases treated by percutaneous drainage]. / Abscessos piogênicos do fígado: análise de 36 casos tratados por punção percutânea.

Thirty-six patients with pyogenic liver abscess treated by percutaneous drainage plus antibiotic therapy were reviewed. The most frequent symptoms were fever (75%) and pain (72.2%), and the most common physical signs were liver enlargement (58.3%) and jaundice (33.3%). The mean time interval from admission to diagnosis was 16 days. CT scans and ultrasonography were diagnostic in 100% of the patients. The percutaneous drainage was effective in one attempt in 19 patients, two attempts in 8, three attempts in 8 patients and more than four attempts in only one patient. There were three patients with hemoperitoneum (8.7%) as a post-drainage complication, one of them needed to be treated surgically. Mean hospital stay was of 24.8 days and the mortality rate was 5.5%. Percutaneous drainage plus antibiotic therapy is then an efficient therapeutic approach to the treatment of pyogenic liver abscess.

[Immunological parameters in the differential diagnosis of ascites secondary to peritoneal carcinomatosis, hepatic cirrhosis, and congestive heart failure]. / Parâmetros imunológicos no diagnóstico differencial de ascite secundária à carcinomatose peritoneal, cirrose hepática e insuficiência cardíaca congestiva.

As a contribution to the study of ascites in patients with liver cirrhosis, congestive heart failure and peritoneal carcinomatosis evaluate in serum and ascites the concentrations of alphafetoprotein, carcinoembryonic antigen and fibronectin, they might suggest a diagnosis for the basic pathology. Forty-seven patients were studied, from whom 23 with cirrhosis, 17 peritoneal carcinomatosis and 7 with congestive heart failure. We conclude that: a) none of the tools usually employed in the analysis of ascitic fluid alone can make the base pathological process responsible for producing ascites; b) fibronectins were more useful for differential diagnosis between cirrhosis and carcinomatosis; c) alpha-fetoprotein and carcinoembryonic antigen were not useful for the definition for differential diagnosis.

[Hepatic hemangiomas. The importance of the image]. / Hemangiomas hepáticos. Importância da imagem.

In the authors' experience, 0.24% of the patients submitted to liver imaging (ultrasound or computerized tomography) have hemangiomas. These are shown as solid nodular lesions, mostly found by chance. Sometimes they do not appear as typical solid vascular lesions. The authors' experience and the literature are discussed. A clear and concise approach to this benign neoplasm is suggested.

[Chronic active hepatitis and possible relationship with immunosuppressive therapy]. / Hepatite crônica ativa e possível relação com terapia imunossupressora.

A 54-year-old man with Chagas cardiomyopathy that was submitted to a heart transplantation and five months later presented a progressive increase in liver enzymes. Diagnosis of chronic active hepatitis was confirmed by histopathologic studies. A transitory interruption of treatment with azathioprine and the posterior re-introduction of small doses, as well as the use of lesser amounts of cyclosporin-A improved the clinical and laboratory pictures. Despite several investigations the pathogenesis of hepatitis remained undetermined, although some type of drug injury was presumed to be involved. The eventual role of viruses as the primary inciting event could not be completely ruled out.

[Surgical treatment of ascites with Le Veen shunt in patients with alcoholic liver disease]. / Tratamento cirúrgico da ascite com o implante da válvula de Le Veen em pacientes com doença hepática alcoólica.

Studies were carried out on 16 alcoholic cirrhotics with clinically intractable ascites who underwent the implant of peritoneovenous (LeVeen) shunts. Our purpose was to find out how this group of patients responded to this kind of surgical treatment for ascites. Fifteen of these patients were male and one female, with a median age of 51.3 years. According to Child classification, 7 were Child B and 9 Child C. Thirty-three surgeries were conducted, in that 5 were performed for revision of the shunt positioning and 12 for the replacement of the valves. A total of 28 shunts were used. Postoperative immediate mortality occurred in 5 patients (4 Child C and one Child B). The median late postoperative follow-up of the 11 patients who survived was 25.8 months. We concluded that LeVeen shunt implantation is a valid palliative therapeutic resource for the treatment of ascites in alcoholic cirrhotics. The results obtained with this patient population are similar to those observed in cirrhotics who presented other etiologies. We also concluded that late mortality was not related to the implant of a LeVeen shunt but was due to the normal course of the disease.

[Diagnosis of hepatocellular carcinoma performed by searching for serologic tumor markers]. / O diagnóstico do carcinoma hepatocelular (CHC) realizado através da pesquisa de marcadores sorológicos tumorais.

Serum levels of alkaline phosphatase, gamma-glutamyltranspeptidase, -1 fucosidase and glutathione-S-transferase are increased in 60, 90, 75 and 64% of patients with hepatocellular carcinoma. In these patients the mean plasma fibrinogen levels is 461.78 mg/dl, while mean serum copper is 200.50 mg/dl. Serum levels of desgamma-carboxiprothrombin is over 900 mg/dl in 67% of the patients (60% of them have HB virus, mostly anti HBe positive). Forty to 95% of them have increased levels of -fetoprotein (AFP). The authors suggest that cirrhotic patients, with or without HB virus, specially those with increased AFP, should have ultrasound examination of the liver every 6 months. This method of imaging has been shown to be more sensitive than AFP (72% versus 25%) in the detection of hepatocellular carcinoma smaller than 2 cm in diameter.