Identification, characterization and analysis of expression of genes encoding arylalkylamine N-acetyltransferases in the pea aphid Acyrthosiphon pisum.

Most organisms exhibit some kind of rhythmicity in their behaviour and/or physiology as an adaptation to the cyclical movements of the Earth. In addition to circadian rhythms, many organisms have an annual rhythmicity in certain activities, such as reproduction, migration or induction of diapause. Current knowledge of the molecular basis controlling seasonal rhythmicity, especially in insects, is scarce. One element that seems to play an essential role in the maintenance of both circadian and seasonal rhythms in vertebrates is the hormone melatonin. In vertebrates, the limiting enzyme in its synthesis is the arylalkylamine N-acetyltransferase (AANAT). Melatonin is also present in insects but the precise biochemical pathway and the enzymes involved in its synthesis are unknown. Insects possess phylogenetically distant arylalkylamine N-acetyltransferases but their involvement in melatonin synthesis still needs to be fully demonstrated. Aphids have a seasonally rhythmical life cycle, reproducing parthenogenetically by viviparity in favourable seasons but, in unfavourable seasons, they produce a single generation of sexual individuals. The length of the photoperiod is the main environmental factor that controls the mode of reproduction in aphids. Taking advantage of the availability of the genome of the aphid Acyrthosiphon pisum, we searched for genes encoding aphid arylalkylamine N-acetyltransferase homologues that could be candidates for participation in seasonal rhythmicity. We identified four AANAT genes, of which at least two (Ap-AANAT1 and Ap-AANAT3) showed highly significant variation in transcription levels depending on the photoperiod conditions. These results are discussed in the context of how seasonality can be controlled in aphids.

Strengthening primary health care in low- and middle-income countries: furthering structural changes in the post-pandemic era.

Strengthening primary health care (PHC) is the most cost-effective approach in low- and middle-income countries (LMICs) to achieve sustainable universal health coverage (UHC), protect against health shocks, and promote health and wellbeing for all people. It has been 45 years since PHC was put on the global agenda followed by multiple efforts to advocate for more funding and improved performance of PHC. Yet, investment in PHC is still insufficient and overall performance of PHC systems is weak in LMICs, resulting in increased vulnerability and poor health outcomes especially among marginalized populations. As countries recover from the COVID-19 pandemic, which exposed the fragility of PHC platforms, it is imperative to go beyond advocacy for PHC investments and make systemic changes to strengthen PHC as the foundation of resilient and equitable health systems. We propose five gamechangers to facilitate structural changes for strengthening PHC through a focused health systems approach: (i) integration of client-centered health services at PHC level; (ii) digitization of PHC services; (iii) efficiency gains invested in essential health services; (iv) strengthening management practices for PHC at district and facility levels; and (v) advancing community engagement for PHC. To be successful, the implementation of the gamechangers must be contextualized and focus on achieving sustainable health outcomes, and therefore use implementation approaches that link essential health services to health outcomes. Through this way countries will maximize the possibility of achieving UHC and attaining the ambitious health targets of the Sustainable Development Goals (SDGs) by 2030.

[Chronic fatigue syndrome: study of a consecutive series of 824 cases assessed in two specialized units]. / Síndrome de fatiga crónica: estudio de una serie consecutiva de 824 casos evaluados en dos unidades especializadas.

BACKGROUND AND OBJECTIVE: The chronic fatigue syndrome (CFS) is a disabling disorder. Few studies are available in our area on the prevalence and characteristics of CFS. Therefore, we carried out a study of a consecutive series of 824 cases diagnosed in two specialized units. PATIENTS AND METHODS: We evaluated all of the CFS patients seen from January 2008 to June 2010. We analyzed social and demographic data, employment status, time of clinical evolution, trigger factors and onset, Fukuda and Canadian criteria, associated comorbidities and treatment. RESULTS: A total of 824 patients were included, 748 (91%) woman, mean age 48±9 years. Average age of onset of symptoms was 35±11 years, time to diagnosis 108±88 month. A precipitating factor was identified in 481 (58%) patients, the onset was gradual in 517 (63%) and 515 (62.5%) were not employed. The most outstanding diagnostic criteria of Fukuda were prolonged generalized fatigue after exercise, sleep disturbance and impairments in concentration and short-term memory. The different groups of symptoms defined by the Canadian consensus showed that CFS is a homogeneous entity. Accompanying comorbidity phenomena were anxiety 691 (83%), sicca syndrome 678 (82%), fibromyalgia 450 (55%). A total of 63% of patients (520) received pharmacological treatment. CONCLUSIONS: CFS is an illness that preferentially affects young women and results in employment absenteeism. The most relevant clinical features were prolonged generalized fatigue after exercise, neurocognitive impairment and sleep disturbance. In the evaluation of the patient, it is very important to apply the Canadian criteria and to assess comorbidity.

[Nicotinamide adenine dinucleotide (NADH) in patients with chronic fatigue syndrome]. / Nicotinamida adenina dinucleótido (NADH) en pacientes con síndrome de fatiga crónica.

BACKGROUND: Nicotinamide adenine dinucleotide (NADH) may be depleted in chronic fatigue syndrome (SFC). The purpose of the study was to evaluate the efficacy of supplementation with NADH in these patients. MATERIAL AND METHODS: A double blind, placebo controlled, 3 month long clinical trial was conducted. The patients were randomized to oral NADH oral 20mg or placebo during the first two months. The intensity of the fatigue, functional performance, mood state, functional impact of the fatigue, quality of life, sleep quality, exercise capacity and functional reserve as well as the investigator's and patient's opinion on the efficacy of the intervention prior to and at 30, 60 and 90 days of the onset of the treatment were evaluated. A stress test was performed in the baseline visit and at 60 days (last day of the double blind treatment). RESULTS: A total of 86 patients, 77 of whom completed the study (mean age, 47 years, 72 women) were enrolled. No significant differences were found in most of the variable studied at the end of the study. Administration of NADH was associated to a decrease in anxiety condition of -1.0 points (p<0.05) and of -0.2 points (p=NS) in the placebo assigned group. Maximum heart rate after the stress test decreased a mean of -8.1l/min (p<0.05) in the NADH group and increased by +1.7l/min in the placebo group (p=0.73). No differences were found in the perception of efficacy with NADH and placebo, by the investigator and patients. CONCLUSIONS: Administration of oral NADH was associated to a decrease in anxiety and maximum heart rate, after a stress test in patients with CFS. On the contrary, this treatment did not modify other clinical variables and the global functional performance.

[A single-case research on the impact of spending cuts on the quality of care of a regional health service].

BACKGROUND: To apply single-case research methods to evaluate the impact of the Regional Government of Galicia's spending cuts on certain aspects of the quality of healthcare. METHODS: In our study, we consider average surgical wait indicators, a Generic Healthcare Deficiency Index (GHDI) of our own design, and rates of perceived improvement based on the Healthcare Barometer. Our analysis combines bottom-up approaches (both visual inspection and non-overlapping measures) with top-down approaches (parametric techniques). RESULTS: In the quinquennial period of cutbacks, we detect several adverse impacts on the quality of healthcare. The tendency to shorten the average surgical wait is reversed and this effect is statistically significant (p<0.01), according to the three estimated ARIMA models. The GHDI increases more than before, with a total effect inferred from regression analyses by both generalized least squares, using the Cochran-Orcutt procedure with the Prais-Winsten adjustment (p<0.1), and non-linear least squares (p<0.05). Indices of perceived improvement fall in primary and specialized care, as well as in hospitalization, with the effect being moderate (NAP, non-overlap of all pairs) or large (SMD, standardized mean difference). The C statistic confirms a significant change. CONCLUSIONS: In this empirical application with single-case research techniques, we notice a certain deterioration in the analyzed indicators, which primarily affect aspects of quality that are not shielded by the doctor-patient relationship, aspects more connected to the conditions of service provision and its overall perception.

Identification of a multifunctional, cell-binding peptide sequence from the a1(NC1) of type IV collagen.

We have previously identified three distinctive amino acid sequences from type IV collagen which specifically bound to heparin and also inhibited the binding of heparin to intact type IV collagen. One of these chemically synthesized domains, peptide Hep-I, has the sequence TAGSCLRKFSTM and originates from the a1(noncollagenous [NC1]) chain of type IV collagen (Koliakos, G. G., K. K. Koliakos, L. T. Furcht, L. A. Reger, and E. C. Tsilibary. 1989. J. Biol. Chem. 264:2313-2323). We describe in this report that this same peptide also bound to intact type IV collagen in solid-phase assays, in a dose-dependent and specific manner. Interactions between peptide Hep-I and type IV collagen in solution resulted in inhibition of the assembly process of this basement membrane glycoprotein. Therefore, peptide Hep-I should represent a major recognition site in type IV collagen when this protein polymerizes to form a network. In addition, solid phase-immobilized peptide Hep-I was able to promote the adhesion and spreading of bovine aortic endothelial cells. When present in solution, peptide Hep-I competed for the binding of these cells to type IV collagen- and NC1 domain-coated substrata in a dose-dependent manner. Furthermore, radiolabeled peptide Hep-I in solution also bound to endothelial cells in a dose-dependent and specific manner. The binding of radiolabeled Hep-I to endothelial cells could be inhibited by an excess of unlabeled peptide. Finally, in the presence of heparin or chondroitin/dermatan sulfate glycosaminoglycan side chains, the binding of endothelial cells to peptide Hep-I and NC1 domain-coated substrates was also inhibited. We conclude that peptide Hep-I should have a number of functions. The role of this type IV collagen-derived sequence in such diverse phenomena as self-association, heparin binding and cell binding and adhesion makes Hep-I a crucial domain involved in the determination of basement membrane ultrastructure and cellular interactions with type IV collagen-containing matrices.

Sexual dysfunction as related to severity of fatigue in women with CFS.

To assess sexual function in women with chronic fatigue syndrome. The study included 27 women, aged 20 to 45 years, with chronic fatigue syndrome (CFS) and 15 healthy female controls. Sexual function was measured with the Golombok Rust Inventory of Sexual Satisfaction (GRISS) questionnaire and five clinical questions. In the patient group, total fatigue impact scale (FIS) score correlated with the GRISS satisfaction (r:-0.471, P < .005), avoidance (r: 0.632, P < .001) and sensuality (r: -0.445, P = .008) subscales. The GRISS satisfaction, avoidance, and sensuality subscale results and the fact of seeing the sexual act as a negative experience correlated with the intensity of fatigue in women with CFS.

Differentiating between malignant and idiopathic pleural effusions: the value of diagnostic procedures.

BACKGROUND: Which diagnostic procedures should be used to differentiate between idiopathic and malignant pleural effusions, is not well established. AIM: To identify which parameters allow differentiation between idiopathic and malignant pleural effusions. DESIGN: Case-note review. METHODS: Over a 12-year period, we treated 1014 consecutive pleural effusion patients, of whom 346 were diagnosed as having an idiopathic or malignant aetiology. We analysed medical history, chest X-ray, pleural fluid analysis (biochemical, microbiological and cytological), chest CT scan and additional examinations that were used according to clinical findings, and compared them with the eventual diagnosis and outcome. RESULTS: Eighty-three patients with idiopathic effusions and 263 with malignant effusions were included. Idiopathic pleural effusion resolved in 47 patients, improved in 20 and persisted in 16. Biochemical pleural fluid analysis did not predict these outcomes. A history of neoplasm, chest X-ray and CT features, as well as additional examinations according to clinical findings, established a diagnosis or suspicion of malignancy in 256 (97.7%) of the 263 patients who received a diagnosis of malignant effusion. Diagnostic thoracoscopy was helpful in seven patients in whom malignant disease was strongly suspected, despite the absence of other pathological findings. DISCUSSION: Non-invasive complementary examinations generally allowed accurate differentiation between malignant and idiopathic effusions. Patients with idiopathic pleural effusions generally had favourable outcomes.

Cytoreductive surgery and hyperthermic intraperitoneal chemotherapy for pseudomyxoma peritonei and appendix tumours in elderly patients: Is it justified?

BACKGROUND: Cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (HIPEC) for pseudomyxoma peritonei and appendix tumours are widespread in the world. It is unclear what should be the attitude in elderly patients. METHODS: This retrospective multicenter study collected the database from ten Spanish centers from Spanish Group of Peritoneal Cancer Surgery. The study period was between November 2002 and March 2014. Seventeen patients with age greater than or equal to 75 years with peritoneal carcinomatosis from pseudomyxoma peritonei and appendix tumours met the selection criteria for the study. Outcomes in terms of morbidity and mortality such as disease-free and overall survival were analyzed. RESULTS: Median PCI was 16 (range 6-39). Ten postoperative adverse events were detected in nine patients (44.4%). 28% were grade I-II and 17% were grade III-IV. Disease-free survival at 1 and 3 years was 67 and 44%, respectively. Overall survival at 1 and 3 years was 100 and 88%, respectively. Only cytoreduction was related to worst disease free survival after univariate (p = 0.007) and multivariate (OR 11.639, 95% CI 1.24-109.74, p = 0.03) analyses. Cytoreduction was related to the worst overall survival after univariate analysis (p = 0.046). CONCLUSION: Cytoreductive surgery and HIPEC for pseudomyxoma peritonei and appendix tumours in elderly patients it is a procedure with feasible postoperative morbi-mortality and survival outcomes. TRIAL REGISTRATION: researchregistry1587 (retrospectively registered).

Inhaled steroids do not decrease bone mineral density but increase risk of fractures: data from the GIUMO Study Group.

Although the negative effect of systemic steroids on bone is well documented, there is not clear evidence about possible adverse effects of inhaled steroids on bone metabolism and fractures. A cross-sectional study was performed on 105 women suffering from bronchial asthma treated with inhaled steroids and 133 controls. Bone mineral density (BMD) was measured by quantitative ultrasonography (QUS) at the calcaneus and by dual X-ray absorptiometry (DXA), at both the lumbar spine and proximal femur. Patients suffering from bronchial asthma showed no statistically significant changes in BMD as measured by DXA or QUS, compared with controls. A higher prevalence of fractures was found in the group of women with bronchial asthma, with an age-adjusted odds ratio of 2.79 (95% CI: 1.19-6.54). Inhaled steroids do not appear to decrease BMD, but are associated with an increased risk of fracture in women.

Morbidity and mortality outcomes after cytoreductive surgery and hyperthermic intraperitoneal chemotherapy in patients aged 75 years and over: Spanish group of peritoneal cancer surgery (GECOP) multicenter study.

BACKGROUND: The aim of this study is to report the perioperative outcomes of cytoreductive surgery and hyperthermic intraoperative intraperitoneal chemotherapy (HIPEC) in patients ≥75 years from a Spanish multi-institutional experience. METHODS: This multi-institutional retrospectively analyzed a prospectively collected clinical data from 10 Spanish hospitals that are part of the Spanish Group Peritoneal Cancer Surgery (GECOP). We assessed postoperative morbidity rates and performed univariate and multivariate analyses of factors associated with overall (grade I-IV) and major (grade III-IV) postoperative morbidity. RESULTS: A total of 85 patients aged ≥75 years were included. Forty six postoperative adverse events were detected in 37 patients (43.5%). Twenty five complications in 20 patients (23.5%) were mild (grade I-II) and 16 complications in 12 patients (14.1%) were moderate-severe (grade III-IV). Five patients died in the first 90 days after the procedure (5.9%). After multivariate analysis, independent factors associated with postoperative complications were: PCI> 12 (OR: 4.14, 95% CI 1.22-14.12, p = 0.043) and the need for perioperative blood transfusion (OR: 14.91, 95% CI 3.87-57.46, p < 0.001). Regarding grade III-IV complications, after multivariate analysis, the presence of preoperative albumin levels <3.5 mgr/dl (OR: 9.15, 95% CI 1.38-60.57, p = 0.017), need for diaphragmatic peritonectomy procedures (OR: 11.32, 95% CI 1.40-91.32, p = 0.023) and perioperative blood transfusion (OR: 8.58, 95% CI 1.44-51.16, p = 0.018) were independent factors. CONCLUSIONS: Cytoreductive surgery and performing HIPEC by experienced groups in selected patients aged ≥75 years can be performed with morbidity and mortality similar to that described in the literature.

Molecular genetic characterization of a locus that contains duplicate Adh genes in Drosophila mojavensis and related species.

Drosophila mojavensis and other species of the mulleri subgroup contain a duplicate gene encoding the enzyme alcohol dehydrogenase (ADH). Studies on the genetic relationship of the two genes using electrophoretic variants show them to be closely linked. We have cloned a 13.5-kb fragment of D. mojavensis DNA into the lambda vector, Charon 30. This fragment contains both Adh genes separated by approximately 2 kb of DNA. The clone hybridized to a single position on chromosome 3 in D. mojavensis following in situ hybridization. It is likely that the genes are tandemly arranged in the genome. One of the two genes shows a complexity in its structure that suggests the close linkage of a pseudogene or part of a gene. The structure of the Adh locus in five species of the mulleri subgroup have been compared by constructing restriction maps of genomic DNA. Two of these species D. arizonensis and D. mojavensis express Adh-1 in the ovaries; the others do not. In comparing these species it is evident that there has been one or two insertions into the region between the Adh genes. It is possible that one of these structural changes is related to the change in Adh tissue-specific expression that has occurred during the evolution of these species.

Postmenopausal women with colles' fracture have lower values of bone mineral density than controls as measured by quantitative ultrasound and densitometry.

Measurement of ultrasonographic parameters provides information concerning not only bone density but also bone architecture. We investigated the usefulness of ultrasonographic parameters and bone mineral density (BMD) to evaluate the probability of Colles' fracture. Two-hundred eighty-nine postmenopausal women (62.3 +/- 8.7 yr) with (n = 76) and without (n = 213) Colles' fracture were studied. BMD of lumbar spine and proximal femur was evaluated in all women by dual-energy X-ray absorptiometry (DXA) and speed of sound (SOS), broadband ultrasound attenuation (BUA), and stiffness in the calcaneus were measured by a Sahara ultrasonometer (Hologic). Patients suffering from Colles' fracture had lower values of BMD adjusted by height at the lumbar spine, L2-L4 (0.797 g/cm2 vs 0.860 g/cm2), femoral neck (0.685 g/cm2 vs 0.712 g/cm2 ), SOS (1518 m/sg vs 1525 m/sg), and stiffness (74.6 vs 77.7) (p < 0.05). Nevertheless, BUA values were similar in both groups. After stepwise logistic regression analysis, the area found under receiver operating characteristic (ROC) curves was 0.60 for L2L4 and 0.63 for a formula combining L2L4 and height. Our data suggest that patients suffering from Colles' fracture have lower values of BMD by DXA, SOS, and stiffness. However, the ability of these techniques to discriminate is low because the values for the area under ROC curve are 0.60 for L2-L4 and 0.63 for a formula derived of the combination of L2-L4 and height.

Postmenopausal women with Colles' fracture have bone mineral density values similar to those of controls when measured with calcaneus quantitative ultrasound.

BACKGROUND: It is a matter of controversy whether or not Colles' fracture is an osteoporotic fracture. Indeed, the usefulness of quantitative ultrasound in distinguishing Colles' fracture from normal fractures is also unclear. METHODS: A cross-sectional case-control study was done on 469 postmenopausal Spanish women, 121 with Colles' fracture and 348 controls. Assessment of risk factors for osteoporosis and measurement of calcaneus quantitative ultrasound were carried out using a Sahara, Hologic device. RESULTS: Patients with Colles' fracture had BUA, SOS, and QUI values that were similar to those of controls, and no statistically significant differences were found. We estimated ROC curves for SOS and a score based on a linear combination of height and SOS (SH-Score). The areas under both curves were 0.56 and 0.61, respectively, which was statistically significant. To obtain 5% false-negative and 10% false-positive figures, the T-score cut-off for SOS was -2.45 and -0.045, respectively. Of these, only 9.2% were classified as high risk and 11% as low risk with 79.8% undetermined. CONCLUSIONS: Patients with Colles' fracture had BUA, SOS, and QUI values that were similar to those of controls. Nevertheless, ROC curves calculated by a combination of height and SOS showed that quantitative calcaneus ultrasound may be a useful tool for identifying postmenopausal women with Colles' fracture. These results indicate that measuring bone mineral density with ultrasound only captures limited aspects of the pathophysiology of Colles' fractures.

The value of chest roentgenography in the diagnosis of pneumothorax after thoracentesis.

PURPOSE: We sought to assess the yield of chest roentgenography for the detection of pneumothorax among hospitalized patients with pleural effusion who have undergone diagnostic or therapeutic thoracentesis. SUBJECTS AND METHODS: We performed a prospective study of 506 thoracentesis procedures in 370 patients. After the procedure, each operator filled out a note recording patient data and the characteristics of the thoracentesis. A chest radiograph was performed within 12 hours after the procedure in all patients. RESULTS: Eighteen (4%) pneumothoraces occurred in 17 patients, 9 (2%) of which required chest tube drainage. Of the 488 patients without symptoms, only 5 (1%) developed a pneumothorax, only 1 of which required chest tube drainage. By contrast, of the 18 patients with symptoms, 13 developed a pneumothorax, 8 of which required chest tubes. There were two independent predictors of pneumothorax: presence of symptoms (odds ratio [OR] = 250; 95% confidence interval [CI]: 65 to 980) and male gender (OR = 5.4; 95% CI: 1.9 to 69). CONCLUSIONS: Among the symptom-free patients in our sample, the risk of developing pneumothorax with clinical consequences was so low that the practice of routine chest roentgenography may not be justified.

Legionella pneumophila. A cause of severe community-acquired pneumonia.

In a prospective study of community-acquired pneumonias, 30 patients were diagnosed with Legionnaires' disease in 15 months. Clinical, laboratory and radiologic features of these patients are reviewed and compared with those who have pneumococcal pneumonia. Alcoholism, history of smoking, previous antimicrobial therapy, gastrointestinal and neurologic manifestations, elevations of serum transaminases, alkaline phosphatase and creatinine levels were more frequent in pneumonia due to Legionella pneumophila than in pneumococcal pneumonia. The presence of respiratory failure and radiologic progression were common findings that suggested L pneumophila as the etiologic agent of a community-acquired pneumonia. Development of respiratory failure was associated with involvement of several lobes and isolation of L pneumophila in any specimen. In 21 of 30 patients with Legionnaires' disease, L pneumophila was isolated from respiratory specimens. Overall mortality was 10 percent, but it increased to 27 percent in patients not treated with erythromycin initially.

Respiratory symptoms and pulmonary function of workers exposed to cork dust, toluene diisocyanate and conidia.

A cross-sectional study on suberosis was conducted to determine the prevalence of respiratory symptoms and the level of pulmonary function, and their relationships within job categories of exposure to cork dust, toluene diisocyanate (TDI) resin bonding and conidia, among cork workers. Exposure-response relationships, with confounders taken into account, showed specific associations between cork dust and chronic bronchitis, TDI and asthma, and conidia and symptoms of hypersensitivity pneumonitis. Workers exposed to these risk factors had reduced mean spirometric values. A significant correlation between a decrease in pulmonary function and length of exposure was found for the workers exposed to cork dust. These results strongly suggest that suberosis, as hitherto described, might in fact be comprised of three different diseases with different etiologic risk factors, ie, respiratory hypersensitivity from exposure to conidia, asthma from exposure to TDI, and chronic obstructive pulmonary disease from exposure cork dust.

[Residual pleural thickening in tuberculous pleuritis. Associated factors]]. / Engrosamiento pleural residual en la pleuritis tuberculosa. Factores asociados.

BACKGROUND: To study the factors related to the development of residual pleural thickening in pleural tuberculosis. PATIENTS AND METHODS: We studied 39 patients with tuberculous pleural effusion. A chest X-ray was taken of each patient at the end of treatment. The patients' medical histories, pleural fluid findings and diagnostic chest films were evaluated. Residual pleural thickening was defined as thickening that was visibly greater than 2 mm in the lower side portion of the chest film. RESULTS: Residual pleural thickening developed in 26% of patients and was found mainly in men (RR = 3.86). In no patients with Löwenstein-Jensen cultures positive for Mycobacterium tuberculosis did pleural complications develop. CONCLUSION: Residual pleural thickening is a common complication of tuberculous pleural effusion. Residual pleural thickening in tuberculous pleurisy occurs more often in men and older patients, and in cases in which pleural liquid culture is negative for M. tuberculosis.

[Tumors diagnosed in 65 patients with type I neurofibromatosis]. / Tumoraciones diagnosticadas en 65 pacientes afectados de neurofibromatosis tipo I.

BACKGROUND: Type 1 neurofibromatosis patients develop malignant neoplasm more frequently than general population. PATIENTS AND METHOD: We have studied 65 patients with neurofibromatosis type 1 because we intend to know the kind of neoplasms that they have presented. RESULTS: 65 patients (48 men and 17 women) fulfilling type 1 neurofibromatosis criteria have been studied. Globally 67 tumors appeared, from which 47 (70%) were benign and 20 (30%) were malignant. Benign tumors included 25 neurofibromas, 11 central nervous system tumors and 11 extraneurological tumors. Malignant tumors accounted for 6 soft tissue sarcomas, 6 carcinomas, 3 malignant CNS tumors and 2 acute lymphoblastic leukemias. CONCLUSION: Presentation of neoplasms is frequent in type 1 neurofibromatosis and constitutes main death and morbility cause.

[Acute myelofibrosis: apropos of a case with immunocytochemical and ultrastructural studies]. / Mielofibrosis aguda: a propósito de un caso con estudios inmunocitoquímicos y ultraestructurales.

We report a case of acute myelofibrosis. This is a rare myeloproliferative disorder characterized by pancytopenia, minimal or absent anisopoikilocytosis, bone marrow fibrosis with hyperplasia and immaturity of the three main cellular lines with megakaryocyte predominance, absence of splenomegaly and rapidly fatal course. We discuss its relationship with acute megakaryocytic leukemia, as its blast elements correspond to megakaryocytes when ultrastructural and antifactor VIII immunoperoxidase techniques are used; these techniques disclose alpha granules and cell demarcating membranes.