Escorbuto una Enfermedad del Pasado en Nuestros Tiempos. A Propósito de un Caso / Scurvy a Disease of the Past in Our Times. About a case

El término escorbuto procede de una palabra del antiguo escandinavo, scorbruck, que significa "hinchazones ulceradas". Es una entidad infrecuente en pediatría, causada por deficiencia nutricional exógena de ácido ascórbico. Los seres humanos, a diferencia de otros seres vivos, no pueden sintetizar la vitamina C y, en consecuencia, dependen estrictamente de su aporte exógeno. El escorbuto puede simular varios desórdenes reumatológicos. Aunque es poco común, puede presentarse como pseudovasculitis o artritis crónica. Para el diagnóstico en pacientes que presentan síntomas músculo esqueléticos se requiere un alto índice de sospecha. Se reporta el caso de un paciente masculino de 9 años que desarrolló la enfermedad por un hábito alimentario selectivo de larga evolución, sin ingesta de frutas ni verduras. El objetivo del presente reporte es alertar al pediatra acerca del desarrollo de escorbuto como potencial consecuencia de las dietas restrictivas, y la importancia de realizar una anamnesis alimentaria completa ante la sospecha diagnóstica

The term scurvy comes from a word from the old Scandinavian, scorbruck, which means ulcerated swellings. Scurvy is an infrequent entity in pediatrics caused by exogenous nutritional deficiency of ascorbic acid. Human beings, unlike other living beings, can not synthesize vitamin C and, consequently, they depend strictly on their exogenous contribution. Scurvy can simulate several rheumatological disorders. Although it is uncommon, it can present as pseudovasculitis or chronic arthritis. A high index of suspicion is required to diagnose patients with musculoskeletal symptoms. We report a case of a 9-year-old male patient who developed the disease due to a long-term selective eating habit, without fruits or vegetables intake. The objective of this report is to alert the pediatrician about the development of scurvy as a potential consequence of restrictive diets, and the importance of performing a complete dietary history to suspect diagnosis

A revision of the bee genus Ancyloscelis (Hymenoptera: Apidae) in Argentina.

A revision of the emphorine bee genus Ancyloscelis in Argentina is presented. The genus extends from the United States to the center of Argentina, but the maximum diversity of morphological structures is found among South American species. Its species form two distinctive groups regarding their mouthparts: one of them with hooked setae on the proboscis, and the other one with simple or plumose setae on the proboscis. To the first group belong in Argentina, A. bonariensis Brèthes, A. halictoides (Holmberg), A. mesopotamica (Holmberg), and the new species A. holmergi. To the second group belong in Argentina A. apiformis (Fabricius), A. romeroi (Holmberg), A. saltensis Rodríguez Roig-Alsina, and A. nigricornis Rodríguez Roig-Alsina. Lectotype designations are made for Leptergatis bonariensis Brèthes and Leptergatis mesopotamica Holmberg. Ancyloscelis turmalis Vachal is a junior new synonym of Ancyloscelis mesopotamica. The synonymy of Ancyloscelis gigas Friese as junior synonym of Ancyloscelis halictoides is confirmed. Descriptions, illustrations, and a key to the species are provided.

Economic analysis of the treatment of end-stage renal disease treatment: living-donor kidney transplantation versus hemodialysis.

INTRODUCTION: End-stage renal disease (ESRD) is a major public health problem in the Spanish health system. Kidney transplantation is the treatment of choice, offering better survival and cost-effectiveness than other alternatives. This study aimed to compare the cost of living-donor kidney transplantation (LDKT) during the first year after transplantation with that of hemodialysis (HD). METHOD: A prospective, descriptive study of cost and efficacy was performed in the Hospital Clinic in Barcelona from January to December 2011. We included 106 patients (57 undergoing HD and 49 receiving a LDKT). The costs of LDKT (donor and recipient) and HD were calculated based on our economic database program. RESULTS: The mean age of recipients and donors was 46 ± 15 and 52 ± 10 years, respectively, and 67% of the recipients were men. In HD patients, the mean age was 67 ± 11 years and 62% were men. The total cost of LDKT was €29,897.91 (€8,128.44 for donors and €21,769.47 for recipients). The total cost of HD was €43,000.88 (€37,917 for HD and related procedures plus €5,082 for transport). LDKT represented a savings of €13,102.97 per patient/year and the payback period was less than 1 year. Quality-adjusted life years were higher in LDKT than in HD patients. CONCLUSION: LDKT is cost effective during the first year after transplantation and is associated with enhanced quality of life. From both the medical and economic points of view, pre-emptive LDKD should be encouraged in Spain to reduce the health budget for ESRD.

Polyclonal versus monoclonal induction therapy in a calcineurin inhibitor-free immunosuppressive therapy in renal transplantation: a comparison of efficacy and costs.

BACKGROUND: Induction therapy in renal transplantation reduces the incidence of acute rejection (AR) in expanded criteria donation (ECD) and donation after cardiac death (DCD). We compared the efficacy of Thymoglobulin (Sanofi-Aventis, Spain), ATG Fresenius (ATG-Fresenius, Spain), and Simulect (Novartis Farm, Spain) in a calcineurin-free protocol in ECD and DCD renal transplantation by evaluating patient survival, graft survival, and AR at 1 year and overall costs. METHODS: An observational retrospective study was performed using our database of 289 consecutive cadaveric ECD renal transplant recipients (n = 178) and DCD recipients (n = 111) from April 1999 to December 2011. Induction therapy consisted of Simulect, Thymoglobulin, and ATG Fresenius. Calcineurin-inhibitor (CNI)-free maintenance therapy consisted of mycophenolate mofetil or sodium and steroids. RESULTS: There were no differences in the patients' demographic characteristics or patient and graft survival. One-year AR rates were equivalent (ECD: 10%, 19.1%, 17.7% versus DCD: 14.3%, 7.1%, 16.7%). Leukopenia and thrombopenia were significantly more frequent in the ECD group treated with polyclonal induction. The average total cost of transplantation was higher in the ECD group but there were no significant differences in the average total cost between ECD and DCD: 39,970.31 ± 7,732€ versus 35,058.34 ± 6,801€ (P = NS). CONCLUSION: Our study shows the same efficacy with polyclonal and monoclonal antibody induction and a CNI-free treatment regimen in ECD and DCD renal transplantation with no differences in overall costs at 1 year after transplantation.

Social determinants of orthotopic liver transplantation candidacy: role of patient-related factors.

INTRODUCTION: Eligibility for orthotopic liver transplantation (OLT) requires careful selection of the best possible candidate. The aim of this study was to identify factors associated with transplantation ineligibility. METHOD: This was a retrospective cohort study of all patients evaluated for OLT at our center (2004-2006) and deemed not eligible. We identified all patients who were evaluated using information from our transplantation database. We extracted demographic data, insurance status, laboratory data, and clinical information including psychosocial evaluations. RESULTS: During the study period 242 evaluated candidates were not listed for transplantation. The most common reason for ineligibility for transplantation listing was early referral (n=59; 24.4%), followed by psychosocial (18.6%), medical contraindications (17.3%), death during evaluation (n=32; 13.2%), malignancy (n=22; 9.1%), declined evaluation or transfer to other transplantation center (n=21; 8.7%), and other reasons (8.7%). In contrast to whites, psychosocial factors were the most common reason among African American candidates. CONCLUSION: This study provides insight into factors contributing to OLT ineligibility among candidates of various ethnic backgrounds.

Orthotopic liver transplantation in a multiethnic population: role of spatial accessibility.

INTRODUCTION: Access to orthotopic liver transplantation (OLT) varies among different ethnic groups. The aim of this study was to determine if distance from transplantation center (DT) impedes referral pattern and accessibility to OLT among ethnic groups. METHOD: This is a retrospective cohort study of all patients evaluated for OLT at our center (2002-2007). The ZipCode Basic software was used to compute distance between the candidate's residence and transplantation center. RESULTS: Five hundred one patients were evaluated during the study period and there were 439 (87.6%) whites 43 (8.6%) African Americans (AA), and others (3.8%). The median DT was 36.8 miles (range, 0.5-231), and there was no significant correlation with the Model for End-Stage Liver Disease (MELD) at presentation (P=.87). Although AA had a higher likelihood of residing closer to a transplantation center they were more likely to have a higher MELD at presentation (20 vs 15.4; P<.001) and less likely to be referred early to initiate OLT evaluation (11.6% vs 26.4%; P=.04). Additionally, type of insurance correlated with higher MELD at presentation. CONCLUSION: DT was not a contributory factor to the observed access disparity in our patient population, rather the insurance type and disease severity as determined using MELD differed significantly among ethnic groups.

Role of lymphadenectomy in endometrioid endometrial cancer.

OBJECTIVE: To assess the risk factors associated with node involvement. STUDY DESIGN: In the period 1990-2008 a total of 265 endometrial cancers were treated in the Institut Universitari Dexeus. We analysed the rate of myometrial invasion, tumour grade, histological type and node involvement. RESULTS: Overall, 86% of tumours were endometrioid, 5.3% papillary serous, 4.9% mixed and 2.6% endometrial stroma sarcoma. Among those with endometrioid histology, lymphadenectomy was not performed (NL) in 85 cases (37.2%), whereas pelvic lymphadenectomy (PL) or pelvic and aortic lymphadenectomy (PAL) was carried out in 84 (36.84%) and 59 patients (25.87%), respectively. In NL patients the overall disease-free survival (DFS) rate at five years was 92.8%. In the PL group, node involvement was observed in 2.4% of cases and the five-year DFS rate was 92.3%. Among PAL patients, 18.6% showed node involvement (72.7% positive pelvic nodes and 63.6% aortic). Aortic involvement was present in 5.9% of cases when there was no pelvic disease, whereas in the presence of positive pelvic nodes the rate of aortic involvement was 50%. The DFS rate at five years was 93.6%. Referring to the risk factors, when infiltration was > 50% of the myometrium, lymph node involvement occurred in 37% of cases and G3 tumors in 45.5%. CONCLUSIONS: Node involvement is more commonly observed in cases with > 50% myometrial invasion and G3, accounting for 25% of cases that can be considered as at-risk patients. When node involvement is present it is equally distributed between the pelvic and aortic levels. As node involvement is a predictive factor for distant metastasis, the 25% of patients considered to be at risk should undergo pelvic and aortic lymphadenectomy

Improving access to liver transplantation: the University of Puerto Rico experience

Liver transplantation is the only treatment for end-stage liver disease. It is costly, difficult, and not performed in Puerto Rico. For these reasons, it has been a limited option for Puerto Ricans with advanced cirrhosis, especially for those with no medical insurance to cover for the procedure. In an effort to improve access to the procedure and offer this chance of life to more Puerto Ricans facing death from complications of advanced liver disease, the Gastroenterology and Liver Diseases Division of the University of Puerto Rico, in collaboration with LifeLink Transplant Institute in Tampa, Florida and the Office of Catastrophic Funds of the Commonwealth of Puerto Rico, opened a clinic for liver transplant evaluation at the Medical Sciences Campus. The purpose of this clinic is to coordinate the pre-transplant evaluation of candidates for this therapy, provide the evaluation by the transplant surgeon in Puerto Rico, expedite the process in seriously ill patients, and offer post-transplant follow-up upon the patient's return to Puerto Rico. The purpose of this article is to describe the experience in this clinic from 1999 to 2003. One hundred ninety-three patients were seen from September 1999 to January 2003. The most common causes for liver disease were hepatitis C and alcohol, alone or in combination. One hundred thirty four were accepted as candidates for evaluation. Of these, 63 had completed the process, 33 were listed for transplantation and 21 had been transplanted by January 2003. Neither education level, marital status, health insurance nor Child score were associated with successful outcome. This clinic offers Puerto Ricans, especially those with limited resources, with a viable access to liver transplantation.

Auxiliary heterotopic partial liver transplantation in pigs with acute ischemic liver failure.

BACKGROUND/AIMS: Fulminant hepatic failure (FHF) is usually fatal without liver transplantation. Auxiliary heterotopic partial liver transplantation (AHPLT) may offer advantages over orthotopic liver transplantation (OLT) or any other heterotopic procedure for the treatment of patients with fulminant liver failure. We studied AHPLT in a severe acute hepatic failure model in pigs. METHODOLOGY: Group A (control: n = 5) underwent portal vein and hepatic artery ligation and side-to-side portocaval shunting. Group B (AHPLT: n = 15) underwent host portal vein and hepatic artery ligation and AHPLT. RESULTS: All of the pigs in group A died within 48 hours from massive liver necrosis. Ten of the 15 pigs (67%) in group B had well-functioning grafts. Five of these ten died between 8 and 17 days postoperatively due to various complications. The remaining five survived for sixty days postoperatively in healthy condition. At the time of sacrifice, four of these five had well-functioning grafts weighing 739 +/- 52 g (mean +/- SEM) and regenerated, but still atrophied, host livers weighing 262 +/- 23 g (p < 0.0002). On the other hand, the one remaining pig had an atrophied graft weighing 310 g and a well-regenerated host liver weighing 470 g, probably due to a late, poorly functioning graft associated with severe rejection. CONCLUSION: AHPLT may result in survival despite host hepatic failure, and the host liver may recover within two months, despite total interruption of blood inflow.

Extraction and purification of a catalase from Candida albicans.

Candida albicans yeast cells H317 were grown to mid-log phase, mechanically disrupted and the resulting crude extract clarified by centrifugation. This catalase rich fraction (1.26 x 10(-4) units/ml) was fractionated by liquid phase isoelectric focusing in a pH gradient ranging from 3 to 10 using the Rotofor Isoelectric Focusing Preparative Cell. After isoelectric separation, fractions containing catalase activity were focused between pH 6.7 and 9.3. Active fractions were pooled and re-focused. After the second fractionation, catalase activity increased to 1.52 x 10(-2) units/ml and was restricted to fractions ranging from pH 7.6 to 8.8. To this point a 121 fold purification was achieved. Native polyacrylamide gel electrophoresis analysis of active fractions revealed a band migrating between 272,000 and 132,000 daltons which showed catalase activity. Purification of C. albicans catalase will allow us to evaluate its potential role in protecting this opportunistic pathogen from products of the oxidative burst. Antibodies generated against the catalase provide means for the evaluation of neutralizing fungal defenses against products of the oxidative burst during phagocytosis.

Extraction and purification of a catalase from Candida albicans

Candida albicans yeast cells H317 were grown to mid-log phase, mechanically disrupted and the resulting crude extract clarified by centrifugation. This catalase rich fraction (1.26 x 10(-4) units/ml) was fractionated by liquid phase isoelectric focusing in a pH gradient ranging from 3 to 10 using the Rotofor Isoelectric Focusing Preparative Cell. After isoelectric separation, fractions containing catalase activity were focused between pH 6.7 and 9.3. Active fractions were pooled and re-focused. After the second fractionation, catalase activity increased to 1.52 x 10(-2) units/ml and was restricted to fractions ranging from pH 7.6 to 8.8. To this point a 121 fold purification was achieved. Native polyacrylamide gel electrophoresis analysis of active fractions revealed a band migrating between 272,000 and 132,000 daltons which showed catalase activity. Purification of C. albicans catalase will allow us to evaluate its potential role in protecting this opportunistic pathogen from products of the oxidative burst. Antibodies generated against the catalase provide means for the evaluation of neutralizing fungal defenses against products of the oxidative burst during phagocytosis

Liver transplantation for acute fulminant hepatic failure.

Orthotopic liver transplantation (OLT) remains the only option for progressive acute fulminant hepatic failure (FHF). The overall one-year survival is approximately 58% versus 75% for all patients undergoing OLT. Nevertheless, this is superior to the results obtained with medical management alone, which carries a mortality of approximately 60-85%. Seven of 32 patients at Hartford Hospital (22%) received liver transplants for acute FHF; the one-year survival was 57%. Four of seven patients are alive with complete neurologic recovery. One case is presented in detail.

Value of 111indium leukocyte scanning in febrile organ transplant patients.

Immunosuppressed febrile organ transplant patients present a diagnostic and therapeutic dilemma since symptomatology is often altered by immunosuppression, which also masks the location of infection. Fifty 111indium leukocyte ( 111In WBC) scans were performed to determine their usefulness in the organ transplant patient. The results were compared with computerized tomography (CT) and gallium 67-citrate (Ga) scanning. Eleven patients received both 111In WBC and Ga scans; 22 received both 111In WBC and CT scans. Ten 111In WBC scans had subtraction of 99m Tc sulfur or albumin colloid for liver evaluation and four 111In WBC scans had subtraction of 99m Tc DMSA for kidney evaluation. The overall sensitivity and specificity for 111In WBC scans was 90% and 90%, respectively. Lung uptake was sensitive (89%) and specific (97%) for pulmonary infections, including bacterial, fungal and cytomegalovirus pneumonias. Renal graft uptake occurred in 15 cases (41%), all except 2 being due to rejection, pyelonephritis, urinary tract infections, or cytomegalovirus infections. Pyelonephritis and renal abscesses were diagnosed in 3 cases with 99m Tc DMSA subtraction. Perihepatic abscesses (2), and infected liver cysts (4) were diagnosed with 99m Tc sulfur or albumin colloid subtraction. There were five false-negative CT scans and three false-negative Ga scans. Therefore, when compared with 111In: sensitivity = 88% vs 64% (CT), specificity = 80% vs 86% (CT); and sensitivity = 111In 90% vs 67% (Ga), specificity = 100% for both 111In WBC and Ga scans.(ABSTRACT TRUNCATED AT 250 WORDS)

Liver transplant for metastatic neuroendocrine tumor.

Generally, the results of liver transplantation for metastatic liver disease have not been favorable. One exception has been the unique group of neuroendocrine tumors, the slow growth of which allows liver transplantation to effectively palliate and control symptoms. We report two cases: (a) A 51-year-old man who underwent orthotopic liver transplantation and resection of the pancreatic primary tumor for a nonfunctioning malignant neuroendocrine tumor with features of both carcinoid and islet-cell glucagonoma remains symptom-free and without evidence of tumor recurrence at 13 months follow-up. (b) A 47-year-old man who underwent orthotopic liver transplantation and Whipple resection for a metastatic islet-cell tumor in the head of the pancreas is fully recovered at 5 months follow-up.

Catheter-associated Candida utilis fungemia in a patient with acquired immunodeficiency syndrome: species verification with a molecular probe.

Candida utilis was cultured from the blood of a patient with acquired immunodeficiency syndrome. The candidemia was apparently associated with catheter implantation. The isolate was identified initially by standard methods and verified by molecular probing. The pattern of actin-specific restriction fragments obtained from the DNA of the isolate, probed with C. albicans actin sequences, corresponded to that of C. utilis. This organism adds to the growing list of Candida species associated with human disease. Molecular probing offers a definitive identification when an unexpected etiological agent is found.