Fifteen years of follow-up of a liver transplant recipient with glycogen storage disease type Ia (Von Gierke disease).

Von Gierke's disease or glycogen storage disease type Ia (GSD-Ia) is an infrequent metabolic disease caused by an atypical accumulation of glycogen. The principal cause of this pathology is deficiency of the glucose-6-phosphatase enzyme. Herein we have reported a case of a young man with a history of Von Gierke's disease (GSD-Ia) since childhood who developed hepatocellular adenomatosis brought to light by ultrasounds and TACs.

Study of liver transplant rejection in alcoholism-induced cirrhosis.

Rejection is the most usual cause of primary dysfunction of hepatic allograft transplants. Acute rejection (AR) often occurs in the early post-transplantation weeks, with an incidence of 12%-19%. Chronic rejection (CR) is less usual (2.

Rituximab for the treatment of lupus erythematosus panniculitis.

Lupus erythematosus panniculitis (LEP) or lupus profundus (LP) is a clinical variant of lupus eryhematosus that involves the deep dermis and the subcutaneous fat and is associated with tender subcutaneous nodules or plaques, with occasional ulceration, atrophy, and scarring. The management of this entity can be difficult because of the lack of response to conventional treatments, such as systemic steroids and antimalarials. The two patients of this study presented LP refractory to several therapies that demonstrated a remarkable improve to the infusion of the anti-CD20 monoclonal antibody, rituximab at a dosage of 375 mg/m²/week.

Kidney failure after heart transplantation.

Background And Purpose: Heart transplantation is a procedure with a high mortality rate. Altered kidney function (AKF) after a heart transplant is common. The purpose of this study was to determine the incidence of and associated factors for renal dysfunction among patients who underwent heart transplantation in our hospital between January 2006 and November 2008.

Severe rhabdomyolysis after allogeneic transplantation of facial structures: a case report.

We present a patient with panfacial neurofibromatosis type 1 who underwent allogeneic transplantation of facial structures, which was complicated by severe rhabdomyolysis and temporary oligoanuria. Because of his underlying disease, this 35 year-old man, weighing 68 kg and with a body mass index (BMI) of 27, had undergone 17 operations for resection modeling of hypertrophied tissues, either alone or combined with static suspension techniques. He finally underwent allogeneic transplantation of facial structures.

Initial experience involving treatment and retreatment with carmustine wafers in combination with oral temozolomide: long-term survival in a child with relapsed glioblastoma multiforme.

Background: Glioblastomas occur infrequently in children, and the prognosis is better than for glioblastomas seen in adults. Aggressive treatment is justified in pediatric patients.

Observations: We present the case of a 6-year-old child with malignant posterior temporal glioma treated with surgery, radiotherapy, local chemotherapy with carmustine wafers, and oral therapy with temozolomide, both at initial diagnosis and at relapse 18 months later.

Computed tomographic angiography with VirSSPA three-dimensional software for perforator navigation improves perioperative outcomes in DIEP flap breast reconstruction.

Background: Vascular anatomy of the abdominal wall varies greatly, especially in the case of the perforator branches of the deep inferior epigastric artery. Preoperative three-dimensional reconstructions with VirSSPA software have been used in the authors' center since the year 2007 for the planning of perforator flaps in breast reconstruction. The main objectives were to reduce surgery time and the number of complications.

Volume and composition of emboli in neuroprotected stenting of the carotid artery.

Background And Purpose: Periprocedural microembolization is a major and permanent risk for patients treated by angioplasty and stent placement of high-grade carotid stenoses. Little is known however about the characteristics and significance of these embolized particles. Our aim was to assess the volume and composition of debris captured by filters during carotid angioplasty and stent placement (CAS) of severe internal carotid artery (ICA) stenoses.

Efficacy and safety of mycophenolate mofetil monotherapy in liver transplant patients with renal failure induced by calcineurin inhibitors.

Objective: To assess the efficacy and safety of mycophenolate mofetil (MMF) monotherapy in liver transplant recipients with renal failure secondary to the use of calcineurin inhibitors (CNIs).

Materials And Methods: Thirty-one patients on MMF monotherapy with creatinine levels >1.3 mg/dL, previously immunosuppressed with CNIs and MMF, were analyzed.

Haemostatic effects of adrenaline-lidocaine subcutaneous infiltration at donor sites.

This study sought methods in burn surgery to reduce postoperative pain and blood loss at donor sites. A prospective, randomised, controlled, blinded trial included 56 people undergoing burn surgery, divided into two groups. Both groups received subcutaneous infiltration at donor sites, with either 1:500,000 adrenaline solution containing added lidocaine or with 0.

Application of Business Process Management to drive the deployment of a speech recognition system in a healthcare organization.

We present a methodology based on Business Process Management to guide the development of a speech recognition system in a hospital in Spain. The methodology eases the deployment of the system by 1) involving the clinical staff in the process, 2) providing the IT professionals with a description of the process and its requirements, 3) assessing advantages and disadvantages of the speech recognition system, as well as its impact in the organisation, and 4) help reorganising the healthcare process before implementing the new technology in order to identify how it can better contribute to the overall objective of the organisation.

Effect of age, birth cohort and period of death on skin melanoma mortality in Spain, 1975 through 2004.

Recent statistics cohort analysis of mortality reveals that skin melanoma rates are dropping in the younger cohorts. Therefore, the aim of this study is to provide up-to-date information and to analyze recent changes in skin melanoma mortality trends in Spain during the period of 1975-2004 using joinpoint regression and age-period-cohort models. Between 1975 and 2004, the age-standardized (overall) mortality rates for skin melanoma increased from 0.

Vesicoureteral reflux after kidney transplantation in children.

We analyzed the frequency of vesicoureteral reflux and the factors that favor its appearance after kidney transplantation in pediatric patients. This retrospective analysis examined the prevalence of post-transplant vesicoureteral reflux in a total of 181 kidney transplants performed in children at our center between 1978 and 2004. In patients who required corrective surgery for this problem, we analyzed pretransplant residual diuresis, pretransplant pathology and post-transplant problems related to vesicoureteral reflux.

Pharmacokinetic evaluation of mycophenolic acid profiles during the period immediately following an orthotopic liver transplant.

Background: Area under the curve (AUC) limited sampling strategies have been proposed to improve the efficiency of mycophenolic acid (MPA), treatment of the transplanted patient. Our objective was to develop a model in the initial phase of the transplantation that explains the variability in the pharmacokinetic behavior of MPA in the immediate posttransplant period, following treatment with mycophenolate mofetil (MMF) in adult liver transplantation.

Methods: One hundred ten pharmacokinetic simplified sampling profiles were collected, including four samples over a 6-hour postdose interval, in over 60 patients treated with cyclosporine or tacrolimus, MMF, and steroids, combining Daclizumab in more than a third of the patients.

Scoring guide when deciding to accept an organ for a liver transplant.

Unlabelled: Our objective was establish a scoring system that allows a donor to be evaluated quickly and easily using a set of variables that are evaluated prior to the donation and another set that are evaluated during surgery.

Materials And Methods: Prior to the donation we analyzed age, medication requirements, natremia, hepatic biochemistry, gas levels, days in ICU, history of hypertension, and weight. A value of 40% was allocated to this group of factors.

Clinical and ethical implications of genetic counselling in familial adenomatous polyposis.

The association of specific genetic disturbances with the development of hereditary cancer helps us to understand the risk of suffering from it, the possibility of an earlier diagnosis, and the treatment and prevention of this disease. Familial adenomatous polyposis (FAP) is a pre-neoplastic syndrome characterized by the presence of hundreds of adenomatous polyps in the colon, which develop into a carcinoma. FAP can be diagnosed using sequencing techniques to detect mutations in the germinal line of the APC (adenomatous polyposis coli) gene.

Efficacy of beta-lactams against experimental pneumococcal endocarditis caused by strains with different susceptibilities to penicillin.

Objectives: To compare the in vitro and in vivo activity of penicillin, cefotaxime and ceftriaxone, using three strains of Streptococcus pneumoniae with different susceptibilities to penicillin (MICs of 0.015, 0.25 and 2 mg/L, respectively).

Has mortality from malignant melanoma stopped rising in Spain? Analysis of trends between 1975 and 2001.

Background: There have been suggestions that mortality from cutaneous malignant melanoma (CMM) is starting to level off in the European Union.

Objectives: To analyse changes in CMM mortality trends in Spain during 1975-2001 using joinpoint regression models.

Methods: Mortality data were obtained from the National Statistics Institute.

Long-term outcome of patients with vasovagal syncope.

Background: After abnormal head-up tilt test (HUT), several trials have evaluated treatment strategies for vasovagal syncope (VVS). However, few unequivocal results have been obtained. The aim of the study was to prospectively analyze the natural history of patients with VVS who did not undergo specific treatment but received education for avoiding syncope after an abnormal HUT.

Joinpoint regression analysis of lung cancer mortality, Andalusia 1975-2000.

Background: Previous analyses of lung cancer mortality were based on models of death rates within one time period, assuming that rates increase or decrease with time at a constant rate. The aim of this work is to analyse recent changes in lung cancer mortality trends in Andalusia (Spain) during the period of 1975-2000 using joinpoint regression models.

Patients And Methods: Mortality data were obtained from the Death Registry of Andalusia.

Immature granulocyte detection by the SE-9000 haematology analyser during pregnancy.

The objective of this study was to determine the nature of the alarm for immature granulocytes appearing in haemograms from pregnant women, as detected by the immature cell information channel (IMI) of the SE-9000 automated haematology analyser. Of all tests run on pregnant women in a 4-month period (n = 698), the first 100 haemograms with immature granulocyte alarms (14.33%) were collected.

Comparison of a repetitive extragenic palindromic sequence-based PCR method and clinical and microbiological methods for determining strain sources in cases of nosocomial Acinetobacter baumannii bacteremia.

Using a repetitive extragenic palindromic PCR (REP-PCR), we genotypically characterized strains causing nosocomial Acinetobacter baumannii infections and analyzed the source of bacteremia in 67 patients from an institution in which infections by this bacterium were endemic. Six different genotypes were found, including 21, 27, 3, 9, 3, and 4 strains. The probable source of bacteremia, according to clinical and/or microbiological criteria, was known in 42 patients (63%): respiratory tract (n = 19), surgical sites (n = 12), intravascular catheters (n = 5), burns (n = 3), and urinary tract (n = 3).