Amino acids L-phenylalanine and L-lysine involvement in trans and cis piperamides biosynthesis in two Piper species.

Several Piper species accumulate piperamides as secondary metabolites, and although they have relevant biological importance, many details of their biosynthetic pathways have not yet been described experimentally. Experiments involving enzymatic reactions and labeled precursor feeding were performed using the species Piper tuberculatum and Piper arboreum. The activities of the phenylalanine ammonia lyase (PAL) enzymes, which are involved in the general phenylpropanoid pathway, were monitored by the conversion of the amino acid L-phenylalanine to cinnamic acid. The activity of the 4-hydroxylase (C4H) enzyme was also observed in P. tuberculatum by converting cinnamic acid to p-coumaric acid. L-[UL-14C]-phenylalanine was fed into the leaves of P. tuberculatum and incorporated into piperine (1), 4,5-dihydropiperine (2), fagaramide (4), trans-piplartine (7), and dihydropiplartine (9). In P. arboreum, it was only incorporated into the piperamide 4,5-dihydropiperiline (3). L-[UL-14C]-lysine was successfully incorporated into the 4,5-dihydropiperine piperidine group (2), dihydropyridinone, and trans- (7) and cis-piplartine (8). These data corroborate the proposal of mixed biosynthetic origin of piperamides with the aromatic moiety originating from cinnamic acid (shikimic acid pathway) and key amide construction with amino acids as precursors.

Robot-assisted Partial Nephrectomy and Bilateral Pyelolithotomy in Ectopic Pelvic Kidneys.

OBJECTIVE: To show how to perform a robot-assisted partial nephrectomy and bilateral pyelolithotomy in ectopic pelvic kidneys. This is a congenital abnormality of position and rotation1 frequently associated with urolithiasis.2 Renal cell carcinoma is a very rare event in pelvic kidneys.3,4 These 2 findings in the same patient could be a surgical challenge and whenever possible a "one stage" treatment is preferred. MATERIALS AND METHODS: A 44-year-old male with bilateral pelvic kidneys admitted because of left back pain. Abdominal CT scan showed a 17 mm stone in the left renal pelvis, a 12 mm stones in the right pelvis and a 34 × 27 mm right lower pole renal mass. A robotic surgery was indicated. Patient was placed in Trendelenburg position with ports configuration as for transperitoneal radical prostatectomy. The right kidney was firstly approached: after isolation of the ureter and suspension of the renal artery, a clampless partial nephrectomy was performed; then through a longitudinal pyelotomy the stone was extracted. To minimize the opening of the posterior peritoneum covering the left kidney, the site of the stone was identified by intraoperative ultrasound; then, through a longitudinal pyelotomy the stone was extracted. Given the watertight sutures and the lack of ureteral obstructions no pigtails ureteral catheters were inserted. A Jackson-Pratt drainage was placed through the inferior port. RESULTS: Consolle time was 190 minutes. Estimated Blood Loss (EBL) was 50 ml. No complications were reported. The drain was removed on the second postoperative day, assessed that creatinine dosage was equal to serum. The length of stay was 4 days. Histopathology showed a pT1a G2 clear cell renal cell carcinoma with negative surgical margins, while stones analysis was calcium oxalate. CONCLUSION: With the availability of robotic technology, the indications for minimally invasive surgery may be safely expanded to include concomitant morbidities in uncommon presentations.

INFN muon tomography demonstrator: past and recent results with an eye to near-future activities.

A short description of the muon tomography demonstrator at the INFN Laboratori Nazionali di Legnaro near Padua, Italy, is given and the principal achievements owing to the data collected at that experimental facility are presented. In particular, the feasibility studies for several applications based on the muon-tomographic technology, within national and European projects, are discussed. The experimental problems and the procedures used to improve the performance are underlined. In addition, new activities and the related detector optimization are illustrated.This article is part of the Theo Murphy meeting issue 'Cosmic-ray muography'.

Proteomic profile of Piper tuberculatum (Piperaceae) / Perfil proteômico e metabólico de Piper tuberculatum (Piperaceae)

Abstract Piper tuberculatum (Piperaceae) is a species that accumulates especially amides as secondary metabolites and several biological activities was previously reported. In this article, we report a proteomic study of P. tuberculatum. Bidimensional electrophoresis (2D SDS-PAGE) and mass spectrometry (ESI-Q-TOF) were used in this study. Over a hundred spots and various peptides were identified in this species and the putative functions of these peptides related to defense mechanism as biotic and abiotic stress were assigned. The information presented extend the range of molecular information of P. tuberculatum.

Resumo Piper tuberculatum (Piperaceae) é uma espécie que acumula especialmente amidas como metabólitos secundários e diversas atividades biológicas dessa espécie foram relatadas anteriormente. No presente artigo, relatamos um estudo proteômico dessa espécie. Eletroforese bidimensional (2D SDS-PAGE) e espectrometria de massas (ESI-Q-TOF) foram utilizadas nesse estudos. Mais de cem spots e vários peptídeos foram identificados nesta espécie e as funções putativas desses peptídeos relacionadas a mecanismo de defesa como estresse biótico e abiótico foram atribuídos. As informações apresentadas ampliam a gama de informações moleculares dessa espécie.

Proteomic profile of Piper tuberculatum (Piperaceae).

Piper tuberculatum (Piperaceae) is a species that accumulates especially amides as secondary metabolites and several biological activities was previously reported. In this article, we report a proteomic study of P. tuberculatum. Bidimensional electrophoresis (2D SDS-PAGE) and mass spectrometry (ESI-Q-TOF) were used in this study. Over a hundred spots and various peptides were identified in this species and the putative functions of these peptides related to defense mechanism as biotic and abiotic stress were assigned. The information presented extend the range of molecular information of P. tuberculatum.

Predictive factors of overall and major postoperative complications after partial nephrectomy: Results from a multicenter prospective study (The RECORd 1 project).

INTRODUCTION AND OBJECTIVES: To analyze postoperative complications and to assess for significant predictive factors during partial nephrectomy (PN) using a large multicenter dataset. METHODS: Patients who underwent PN for clinical T1 renal tumors at 19 urological Italian centers (Registry of Conservative Renal Surgery [RECORd] project) were evaluated between 2009 and 2012. Anthropometric data, comorbidities and perioperative outcomes were analyzed. Complications were divided as intra- and postoperative, medical and surgical, as appropriate. The severity of postoperative complications was graded according to the modified Clavien classification system. Patients who experienced intraoperative complications were excluded from the analyses for the potential confounding effect in the evaluation of predicting factors for postoperative complications. RESULTS: Overall, 979 patients were analyzed: open, laparoscopic and robot-assisted (available since 2011) surgical approaches were used in 522 (56.4%), 286 (30.9%) and 117 (12.6%) cases, respectively. Surgical postoperative complications were reported in 121 (13.1%) cases (32 (3.5%) were Clavien 3), medical were reported in 52 (5.6%) cases (3 (0.3%) were Clavien 3). No Clavien 4 complications were reported. At multivariable analysis, ECOG score ≥1 (OR 1.98; p = 0.002), lower preoperative hemoglobin (OR 0.71; p < 0.0001) and open surgical approach (2.91; p = 0.02) were significant predictive factors of overall surgical postoperative complications, ECOG score ≥1 (OR 1.93; p = 0.04) and surgical approach (p = 0.05) were significant predictive factors of Clavien 3 either surgical or medical postoperative complications. CONCLUSIONS: Comorbidities and surgical approach should be considered in preoperative evaluation of patients undergoing PN, as they resulted to play a significant role in the occurrence of postoperative complications.

Proteomic profile of Piper tuberculatum (Piperaceae)

Abstract Piper tuberculatum (Piperaceae) is a species that accumulates especially amides as secondary metabolites and several biological activities was previously reported. In this article, we report a proteomic study of P. tuberculatum. Bidimensional electrophoresis (2D SDS-PAGE) and mass spectrometry (ESI-Q-TOF) were used in this study. Over a hundred spots and various peptides were identified in this species and the putative functions of these peptides related to defense mechanism as biotic and abiotic stress were assigned. The information presented extend the range of molecular information of P. tuberculatum.

Resumo Piper tuberculatum (Piperaceae) é uma espécie que acumula especialmente amidas como metabólitos secundários e diversas atividades biológicas dessa espécie foram relatadas anteriormente. No presente artigo, relatamos um estudo proteômico dessa espécie. Eletroforese bidimensional (2D SDS-PAGE) e espectrometria de massas (ESI-Q-TOF) foram utilizadas nesse estudos. Mais de cem spots e vários peptídeos foram identificados nesta espécie e as funções putativas desses peptídeos relacionadas a mecanismo de defesa como estresse biótico e abiótico foram atribuídos. As informações apresentadas ampliam a gama de informações moleculares dessa espécie.

Head and neck mucosal melanoma: clinicopathological analysis of 51 cases treated in a single cancer centre and review of the literature.

Head and neck mucosal melanoma (HNMM) is a rare and aggressive malignancy. The objective of this study was to describe the outcomes of patients with HNMM. Clinical and pathological data from 51 patients with primary HNMM were reviewed. All patients were treated at a single cancer centre between 1954 and 2012. Most tumours involved the nasal cavity (35.3%) and upper gingiva (29.4%). The majority of lesions were ulcerated (54.9%) and pigmented (84.3%). Forty-three patients underwent surgical treatment and 21 (41.2%) underwent adjuvant chemotherapy and/or radiotherapy. Eight patients (15.7%) received palliative treatment. The median follow-up period was 21 months. During this period, 30 (58.8%) patients had tumour recurrences. At the last clinical evaluation, only seven (13.7%) patients were alive with no evidence of disease and three (5.9%) were alive with HNMM. There were significant differences in overall survival probability according to the presence of ulceration (P=0.004), metastatic lymph nodes (P=0.003), and treatment including a radical surgical procedure (P<0.001). On multivariate analysis, ulceration was the only variable associated with an increased risk of death. Despite the poor prognosis, there was significant improvement in overall survival in the most recent years in this sample, mainly due to advances in diagnosis and reconstruction techniques.

Reproductive features of the swimming crab Callinectes danae(Crustacea, Portunoidea) on the subtropical coast of Brazil: a sampling outside the estuary.

The life cycle of the crab Callinectes danae is estuarine-dependent, and studies on aspects of their biology should also cover marine areas. The present study investigated the sexual maturity, as well as habitat preference by adults in different gonadal stages, and the crabs' reproductive periodicity outside the estuary. Three bays on the subtropical southeastern coast of Brazil were sampled monthly for two years. For each bay, six transects were established, four of them parallel to the beach line (5, 10, 15 and 20 m depth), as well as one transect in an exposed area, and another sheltered from the action of waves. The results showed that the pattern of spatio-temporal distribution of adults C. danae was similar in three bays, although the highest abundance was found in Ubatumirim. Females with developed gonads/ovigerous females were found in greater abundance than females with rudimentary/developing gonads, mainly in deeper transects. Although the areas sampled have different environmental characteristics, the reproductive pattern of the species did not change, showing continuous reproduction throughout, with more abundance of reproductive females on spring and summer. Males reached maturity at larger sizes than females in all three bays.

Reproductive features of the swimming crab Callinectes danae(Crustacea, Portunoidea) on the subtropical coast of Brazil: a sampling outside the estuary / Características reprodutivas do siri Callinectes danae(Crustacea, Portunoidea) na região subtropical da costa do Brasil: uma amostragem fora do estuário

AbstractThe life cycle of the crab Callinectes danae is estuarine-dependent, and studies on aspects of their biology should also cover marine areas. The present study investigated the sexual maturity, as well as habitat preference by adults in different gonadal stages, and the crabs’ reproductive periodicity outside the estuary. Three bays on the subtropical southeastern coast of Brazil were sampled monthly for two years. For each bay, six transects were established, four of them parallel to the beach line (5, 10, 15 and 20 m depth), as well as one transect in an exposed area, and another sheltered from the action of waves. The results showed that the pattern of spatio-temporal distribution of adults C. danae was similar in three bays, although the highest abundance was found in Ubatumirim. Females with developed gonads/ovigerous females were found in greater abundance than females with rudimentary/developing gonads, mainly in deeper transects. Although the areas sampled have different environmental characteristics, the reproductive pattern of the species did not change, showing continuous reproduction throughout, with more abundance of reproductive females on spring and summer. Males reached maturity at larger sizes than females in all three bays.

ResumoO siri Callinectes danae é estuarino dependente em seu ciclo de vida, sendo que os estudos que levantam informações sobre os aspectos da sua biologia devem também abranger regiões marinhas. Assim, o presente estudo teve como objetivo investigar a maturidade sexual, bem como a preferência ocupacional por adultos em diferentes estágios gonadais e sua periodicidade reprodutiva fora da região estuarina. Para tanto, três enseadas da porção subtropical do litoral sudeste brasileiro foram amostradas mensalmente, durante dois anos. Para cada enseada, seis transectos foram estabelecidos, quatro deles paralelos à linha de praia (5, 10, 15 e 20m de profundidade), um transecto exposto e outro abrigado à ação de ondas. Os resultados mostraram que o padrão de distribuição espaço-temporal de adultos de C. danae foi similar nas três enseadas, embora uma maior abundância tenha sido encontrada em Ubatumirim. Fêmeas ovígeras e com gônadas desenvolvidas foram encontradas em maior abundância que fêmeas com gônadas rudimentares e em desenvolvimento, principalmente nos transectos mais profundos. Apesar das regiões amostradas possuírem características ambientais diferenciadas, o padrão reprodutivo da espécie não sofreu alterações, apresentando reprodução contínua, com maior abundância de fêmeas reprodutivas na primavera e verão. Machos atingiram maturidade em tamanhos maiores que as fêmeas nas três enseadas.

Investigation of urban ethnoveterinary in three veterinary clinics at east zone of São Paulo city, Brazil.

ETHNOPHARMACOLOGICAL RELEVANCE: Urban Ethnoveterinary should be further explored because of its value as it resists conventional medical care. In addition, the comparison between the resources used by Ethnoveterinary and Ethnopharmacology should be investigated in depth, increasing the availability of new bioactive potential in human and veterinary medicines. This project aimed to determine whether plants are used in the health care of dogs and/or cats in urban area and to compare its uses with other ethnoveterinary and ethnopharmacological data. MATERIALS AND METHODS: Three veterinary clinics from east area of São Paulo city were selected, in order to record the offering of plants to pets by their owners. Individual interviews were conducted from May to November 2012 and consisted of application of semi-structured interviews and questionnaires. A literature search was performed to compare our findings with other ethnopharmacological and ethnoveterinarian data from the literature. RESULTS: A total of 273 people were interviewed and 46 (16.84%) of them utilize medicinal plants for their pets. Most plant species are exclusively home grown (57.9%). The plants most cited were Plectranthus barbatus Andrews, Matricaria chamomilla L. and Foeniculum vulgare Mill. The most frequent route of administration, part utilized and mode of preparation was oral (80.35%), leaves (89.47%) and infusion (61.90%), respectively. From 19 cited plants, 14 (73.7%) are mentioned in ethnoveterinary literature, whereas 11 (57.9%) are used for the same purposes. All plants reported in our study have at least one common use with ethnopharmacology. CONCLUSION: The survey provided evidence of ethnoveterinary use of medicinal plants for dogs and/or cats in urban area of São Paulo, complementarily with the official veterinary, and showed that the plants used in pets are also used in humans with the same purposes and routes of administration, as well as in other animals.

Natural compounds isolated from Brazilian plants are potent inhibitors of hepatitis C virus replication in vitro.

Compounds extracted from plants can provide an alternative approach to new therapies. They present characteristics such as high chemical diversity, lower cost of production and milder or inexistent side effects compared with conventional treatment. The Brazilian flora represents a vast, largely untapped, resource of potential antiviral compounds. In this study, we investigate the antiviral effects of a panel of natural compounds isolated from Brazilian plants species on hepatitis C virus (HCV) genome replication. To do this we used firefly luciferase-based HCV sub-genomic replicons of genotypes 2a (JFH-1), 1b and 3a and the compounds were assessed for their effects on both HCV replication and cellular toxicity. Initial screening of compounds was performed using the maximum non-toxic concentration and 4 compounds that exhibited a useful therapeutic index (favourable ratio of cytotoxicity to antiviral potency) were selected for extra analysis. The compounds APS (EC50=2.3µM), a natural alkaloid isolated from Maytrenus ilicifolia, and the lignans 3(∗)43 (EC50=4.0µM), 3(∗)20 (EC50=8.2µM) and 5(∗)362 (EC50=38.9µM) from Peperomia blanda dramatically inhibited HCV replication as judged by reductions in luciferase activity and HCV protein expression in both the subgenomic and infectious systems. We further show that these compounds are active against a daclatasvir resistance mutant subgenomic replicon. Consistent with inhibition of genome replication, production of infectious JFH-1 virus was significantly reduced by all 4 compounds. These data are the first description of Brazilian natural compounds possessing anti-HCV activity and further analyses are being performed in order to investigate the mode of action of those compounds.

Óleo de melaleuca (Melaleuca alternifolia Maiden & Betche, Cheel) no controle de cercosporiose em beterraba / Tea tree oil (Melaleuca alternifolia Maiden& Betche, Cheel) in control of Cercospora beticola Sacc. on Beet

RESUMO O óleo volátil da melaleuca (Melaleuca alternifolia Maiden & Betche, Cheel) possui atividade antimicrobiana podendo causar efeitos sobre as plantas. Avaliou-se a inibição do óleo em Cercospora beticolaSacc., e seu efeito no aumento da produção e qualidade de raízes de beterraba. As doses foram de 0,13; 0,67; 0,80 e 1,00% do óleo, além das testemunhas composta pelo meio de cultura Batata Dextrose Ágar (BDA) no experimento in vitro, e água no experimento in vivo. As plantas foram pulverizadas duas vezes por semana. O delineamento foi inteiramente casualizado, com 4 repetições, e as médias foram comparadas pelo teste Tukey a 5% de probabilidade. O índice de infecção das folhas foi determinado por escala diagramática além do peso e diâmetro das raízes. Os resultados de inibição do crescimento micelial para as doses do óleo foram 0; 56; 87; 83 e 99%, e os índices de infecção: 77,08; 35,62; 21,04; 19,37 e 20,00%, respectivamente, para a testemunha e as doses 0,13; 0,67; 0,80 e 1,00% do óleo. Somente na concentração de 0,80% o óleo proporcionou relação positiva entre o ganho de peso e o diâmetro das raízes. O óleo de Melaleuca foi eficaz no controle de C. beticola e, como consequência, houve produção de raízes de beterraba com melhor desenvolvimento.

ABSTRACT The volatile oil from Melaleuca (Melaleuca alternifolia Maiden & Betche Cheel.) has antimicrobial properties and can promote several effects on plant cultivation. The aim of this study was to evaluate the inhibition of the oil in Cercospora beticola Sacc. and if it favors the growth and development of beet root. The doses were 0.13, 0.67, 0.8 and 1% of oil, besides the control PDA (potato-dextrose-agar) in vitro (laboratory condition) and with water as treatment control in vivo (field conditions). The plants were sprayed twice a week. The treatments were completely randomized and the averages were compared using the Tukey test at 5%. The infection rate of leaves was measured by diagrammatic scale besides the weight and diameter of tubers. The inhibition results of the radial growth by oil treatments were 0; 56, 87, 83 and 99%, while the infection rate showed: 77.08, 35.62, 21.04, 19.37 and 20% respectively to the control and to the oil concentration of 0,13; 0,67; 0,80 e 1,00%. Only at concentration of 0.8% the tea tree oil showed a positive relationship between tuber´s weight and tuber´s diameter gains. It can be concluded that tea tree oil is effective to controlling C. beticola, and also promotes an increase on development in beet tubers.

Fragmentation of sperm DNA using the TUNEL method.

OBJECTIVES: To establish the validity of the TUNEL assay in determining sperm DNA fragmentation, the relationship between the degree of fragmentation and the seminal parameters and the sample needed to conduct the test. MATERIAL AND METHODS: We used semen samples from healthy fertile men (n=33), patients who consulted for infertility with a prescription for the TUNEL assay (n=77) and patients with intracytoplasmic sperm injection failure (n=20), analyzed according to the 2010 WHO. The TUNEL/propidium iodide test was performed by flow cytometry, on baseline and post-swim-up samples. RESULTS: The cutoff value for the TUNEL assay (ROC curves) was 26%, with a sensitivity and specificity of 85% and 89%, respectively. The pre-swim-up and post-swim-up medians of the results from the TUNEL assay showed no significant differences (17.0% vs. 12.9%, respectively). However, 39.1% of the samples showed a difference greater than 15 in absolute value between the results of the baseline and post-swim-up TUNEL assays. The linear correlation study of the morphology, mobility and vitality using the post-swim-up TUNEL assay showed a greater correlation than preselection, with significant results (r: -0.394, P<.0001; r: -0.461, P<.0001; r: -0.526, P<.0001). CONCLUSIONS: The TUNEL assay is a valid test for clinical use. DNA fragmentation is a factor independent from traditional semen tests. We found a greater susceptibility to damage generated in the laboratory procedures in the samples with lower quality. The sample of choice for evaluating DNA fragmentation will depend on whether the clinician is treating a natural or assisted fertilization.

Avaliação da descontaminação fúngica de camomila [Chamomilla recutita (L. ) Rauschert] por meio de diferentes métodos caseiros em duas temperaturas / Evaluation of fungal decontamination of chamomile [Chamomilla recutita (L. ) Rauschert] through different home procedures at two temperatures

O aumento no consumo de plantas medicinais vem se transformando em problema de Saúde Pública, devido ao potencial de contaminação microbiana, principalmente por origem natural e condições inadequadas de uso e armazenamento. O presente trabalho teve como objetivo avaliar a descontaminação fúngica de camomila [Chamomilla recutita (L.) Rauschert] através de diferentes processos caseiros (decocção, infusão, água morna). Foram analisadas 10 amostras de camomila, procedentes de diferentes estabelecimentos comerciais. Os resultados das análises microbiológicas indicaram redução da contaminação fúngica na maioria das amostras, porém não atingindo os índices considerados satisfatórios, o que evidencia a necessidade de medidas regulatórias e educacionais que garantam a qualidade destes produtos, desde a produção até a colheita.

The increase in the consumption of medicinal plants has become a Public Health problem due to potential microbiological contamination, especially due to their natural origin and inadequate conditions of use and storage. The present study aimed to evaluate the fungal decontamination of chamomile [Chamomilla recutita (L.) Rauschert] through different home procedures (decoction, infusion, warm water). Ten chamomile samples from different commercial establishments were analyzed. The results of microbiological analyses indicated a reduction in fungal contamination in most samples which, however, did not reach the indexes considered satisfactory, evidencing the need of regulatory and educational measures to guarantee the quality of these products, from production to harvest.

Antimicrobial efficacy of alcohol-based hand gels.

In recent years, several commercial alcohol-based hand gels have appeared on the market to improve the hand-cleansing compliance of healthcare workers. Although the antimicrobial efficacy of these products has been reported in different countries, few studies have investigated this subject in Brazil. In this study, we assessed the antimicrobial efficacy of 12 alcohol-based hand gels produced in Brazil, containing 70% w/w or v/v ethyl alcohol as the active ingredient, according to the European Standard EN 1500 (EN 1500). The following alcohol gels were tested: Hand Gel, Voga Gel, Solumax Solugel, Doctor Clean, Rio Gel, Clear Gel, Sevengel, Hand CHC, Gel Bac, WBL-50 Gel, Sanigel and Soft Care Gel. In addition, 70% w/w ethyl alcohol and three alcohol-based hand rubs (Sterillium, Sterillium Gel, and Spitaderm), commonly used in Europe and effective according to EN 1500, were also tested. All the products tested, except for two, were approved by the EN 1500 test protocol with a 60s application. The results confirmed the antimicrobial efficacy of the majority of the alcohol gels produced in Brazil for hand hygiene of healthcare workers.

[Managing the hospitalizations for older patients in University Hospital of Pisa]. / L'andamento dei ricoveri ordinari relativi ai pazienti anziani in un' Azienda Ospedaliera di Alta Specializzazione.

We have analyzed hospitalizations of older people (> 64) from 2002 to 2005. Patients, discharges and stay in hospital have increased, the variation has been statistically significant. We have noticed the same trend about the rehospitalizations. Patients and discharges coming from ASL 5 and zone 4 have decreased in relation with all the elderly people. The reduction of stay in hospital and the decrease of rehospitalizations have been statistically significant.

Antioxidant activity of Agaricus blazei.

The ethanolic extract of Agaricus blazei and ethyl acetate and hydroalcoholic fractions were evaluated for their antioxidant activity.

Antioxidant activity of Maytenus ilicifolia root bark.

Maytenus ilicifolia is an important plant with potential on cancer treatment and has been largely used in Brazil and other countries. We have evaluated the crude ethanolic extract of M. ilicifolia as a potential antioxidant source using an assay based on the bleaching of the radical monocation 2,2'-azinobis (3-ethylbenzothiazoline-6-sulfonic acid) (ABTS(*+)) and by HOCl scavenger capacity. Trolox and uric acid were used as positive controls. The results indicated M. ilicifolia root bark as a great source of antioxidants based on its potential as scavenger of radicals.

Remission of thrombotic thrombocytopenic purpura in a patient with compound heterozygous deficiency of von Willebrand factor-cleaving protease by infusion of solvent/detergent plasma.

UNLABELLED: Plasma exchange or plasma infusion is considered to be the therapy of choice in patients with thrombotic thrombocytopenic purpura (TTP) who are deficient in von Willebrand factor-cleaving protease (VWF-CP). Recently, mutations in the ADAMTS 13 gene were identified as being responsible for VWF-CP deficiency in patients with familial TTP (VWF-CP deficiency in the absence of an inhibitor). Here we report on a girl who presented with recurrent thrombocytopenia and anaemia since birth, developing the full pentad of characteristic TTP at the age of 16 y. Congenital TTP was confirmed on the basis of severe VWF-CP deficiency in the absence of an acquired inhibitor. The patient was found to be compound heterozygous for two hitherto undescribed mutations in the ADAMTS 13 gene: a truncating frame shift mutation, 4143insA in exon 29, and the nonsense mutation 3100A >T in exon 24 (R1034X). After infusion of solvent/detergent plasma, the patient went into remission and remained asymptomatic under regular plasma therapy at 2-wk intervals for over two years. CONCLUSION: TTP in childhood may be mild and oligosymptomatic. Determination of VWF-CP activity is helpful in the differential diagnosis of thrombocytopenia.