Magnetic resonance imaging and BMB score in the evaluation of bone involvement in Gaucher’s disease patients

AbstractObjective:To evaluate by magnetic resonance imaging changes in bone marrow of patients undergoing treatment for type I Gaucher’s disease.Materials and Methods:Descriptive, cross-sectional study of Gaucher’s disease patients submitted to 3 T magnetic resonance imaging of femurs and lumbar spine. The images were blindly reviewed and the findings were classified according to the semiquantitative bone marrow burden (BMB) scoring system.Results:All of the seven evaluated patients (three men and four women) presented signs of bone marrow infiltration. Osteonecrosis of the femoral head was found in three patients, Erlenmeyer flask deformity in five, and no patient had vertebral body collapse. The mean BMB score was 11, ranging from 9 to 14.Conclusion:Magnetic resonance imaging is currently the method of choice for assessing bone involvement in Gaucher’s disease in adults due to its high sensitivity to detect both focal and diffuse bone marrow changes, and the BMB score is a simplified method for semiquantitative analysis, without depending on advanced sequences or sophisticated hardware, allowing for the classification of the disease extent and assisting in the treatment monitoring.

Circumsolar energetic particle distribution on 2011 November 3

Late on 2011 November 3, STEREO-A, STEREO-B, MESSENGER, and near-Earth spacecraft observed an energetic particle flux enhancement. Based on the analysis of in situ plasma and particle observations, their correlation with remote sensing observations, and an interplanetary transport model, we conclude that the particle increases observed at multiple locations had a common single source active region and the energetic particles filled a very broad region around the Sun. The active region was located at the solar backside (as seen from Earth) and was the source of a large flare, a fast and wide coronal mass ejection, and an EIT wave, accompanied by type II and type III radio-emission. In contrast to previous solar energetic particle events showing broad longitudinal spread, this event showed clear particle anisotropies at three widely separated observation points at 1AU, suggesting direct particle injection close to the magnetic footpoint of each spacecraft, lasting for several hours.We discuss these observations and the possible scenarios explaining the extremely broad particle spread for this event.

Multi-Eruption Solar Energetic Particle Events Observed by SOHO/ERNE

In the last two decades of studying the Solar Energetic Particle (SEP) phenomenon, intensive emphasis has been put on how and when and where these SEPs are injected into interplanetary space. It is well known that SEPs are related to solar flares and CMEs. However, the role of each in the acceleration of SEPs has been under debate since the major role was taken from flares ascribed to CMEs step by step after the skylab mission, which started the era of CME spaceborn observations. Since then, the shock wave generated by powerful CMEs in between 2-5 solar radii is considered the major accelerator. The current paradigm interprets the prolonged proton intensity-time profile in gradual SEP events as a direct effect of accelerated SEPs by shock wave propagating in the interplanetary medium. Thus the powerful CME is thought of as a starter for the acceleration and its shock wave as a continuing accelerator to result in such an intensity-time profile. Generally it is believed that a single powerful CME which might or might not be associated with a flare is always the reason behind such gradual events.

In this work we use the Energetic and Relativistic Nucleus and Electrons ERNE instrument on board Solar and Heliospheric Observatory SOHO to present an empirical study to show the possibility of multiple accelerations in SEP events. In the beginning we found 18 double-peaked SEP events by examining 88 SEP events. The peaks in the intensity-time profile were separated by 3-24 hours. We divided the SEP events according to possible multiple acceleration into four groups and in one of these groups we find evidence for multiple acceleration in velocity dispersion and change in the abundance ratio associated at transition to the second peak. Then we explored the intensity-time profiles of all SEP events during solar cycle 23 and found that most of the SEP events are associated with multiple eruptions at the Sun and we call those events as Multi-Eruption Solar Energetic Particles (MESEP) events. We use the data available by Large Angle and Spectrometric Coronograph LASCO on board SOHO to determine the CME associated with such events and YOHKOH and GOES satellites data to determine the flare associated with such events. We found four types of MESEP according to the appearance of the peaks in the intensity-time profile in large variation of energy levels. We found that it is not possible to determine whether the peaks are related to an eruption at the Sun or not, only by examining the anisotropy flux, He/p ratio and velocity dispersion. Then we chose a rare event in which there is evidence of SEP acceleration from behind previous CME. This work resulted in a conclusion which is inconsistent with the current SEP paradigm. Then we discovered through examining another MESEP event, that energetic particles accelerated by a second CME can penetrate a previous CME-driven decelerating shock. Finally, we report the previous two MESEP events with new two events and find a common basis for second CME SEPs penetrating previous decelerating shocks. This phenomenon is reported for the first time and expected to have significant impact on modification of the current paradigm of the solar energetic particle events.

Early detection of injuries in leaves of Clusia hilariana Schltdl. (Clusiaceae) caused by particulate deposition of iron

This study aims to evaluate the prognostic value of microscopic parameters of asymptomatic leaves of Clusia hilariana Schltdl. subjected to particulate deposition of iron (2.14 mg cm-2 day-1) for 45 consecutive days. Samples of young and expanded leaves without symptoms were collected and subjected to light and scanning electron microscopy techniques. The height of the epidermal cells on both surfaces of the leaf and the thickness of the hypodermis, the chlorophyll parenchyma, and the leaf blade were measured. Micromorphological injury occurred in the abaxial surface of young leaves and on both surfaces of expanded leaves. Erosion of the epicuticular wax and cuticle rupture were frequent on the adaxial surface, while on the abaxial surface of both leaves there was a loss of sinuosity on the anticlinal wall of the epidermal cells, stomatal deformity and obstruction. Micromorphometric alterations were seen in all leaf tissues except in the height of epidermic cells, probably due to the thick cuticle and prominent cuticular flanges. The highest difference in thickness of the leaf blade was seen in young leaves of plants subjected to SPMFe, indicating greater sensibility to particulate iron in comparison to the expanded leaves. The micromorphological and micromorphometric alterations in the leaf blade of Clusia hilariana Schltdl. showed the prognostic potential of these tools on the evaluation of impacts caused by the deposition of particulate matter, especially in the 'Restinga' natural vegetation, where the exposure is increasing due to the presence of iron ore industry in their surroundings.

Use of surgical mesh of different compositions in the correction of the abdominal wall defect in rats

Objective: To analyze the performance of two surgical meshes of different compositions during the defect healing process of the abdominal wall of rats. Methods: thirty-three adult Wistar rats were anesthetized and subjected to removal of an area of 1.5 cm x 2 cm of the anterior abdominal wall, except for the skin; 17 animals had the defect corrected by edge-to-edge surgical suture of a mesh made of polypropylene + poliglecaprone (Group U - UltraproTM); 16 animals had the defect corrected with a surgical mesh made of polypropylene + polidioxanone + cellulose (Group P - ProceedTM). Each group was divided into two subgroups, according to the euthanasia moment (seven days or 28 days after the operation). Parameters analyzed were macroscopic (adherence), microscopic (quantification of mature and immature collagen) and tensiometric (maximum tension and maximum rupture strength). Results : there was an increase in collagen type I in the ProceedTM group from seven to 28 days, p = 0.047. Also, there was an increase in the rupture tension on both groups when comparing the two periods. There was a lower rupture tension and tissue deformity with ProceedTM mesh in seven days, becoming equal at day 28. Conclusion : the meshes retain similarities in the final result and more studies with larger numbers of animals must be carried for better assessment.

Comprimento do colo uterino e dilatação do orifício interno avaliados pela ultra-sonografia bidimensional e tridimensional

OBJETIVO: comparar as medidas do comprimento da cérvice e afunilamento, obtidas por ultra-sonografia transvaginal bidimensional e tridimensional na gestação. MÉTODOS: estudo descritivo, prospectivo, com comparação de grupos realizado no período de abril de 2004 a fevereiro de 2005. Foram incluídas 74 gestantes com idade gestacional entre a 19ª e a 24ª semana, independente da presença ou não de fatores de risco para parto prematuro. O exame ultra-sonográfico da cérvice foi efetuado uma única vez na mesma paciente e por único observador. As medidas aferidas por ultra-sonografia bidimensional foram feitas no momento da realização dos exames, e pela tridimensional, com intervalo de 7 a 15 dias do exame bidimensional. A medição do comprimento da cérvice, diâmetro e comprimento do funil cervical foi realizada por ultra-sonografia bidimensional no plano sagital e tridimensional nos planos sagital e coronal. Para testar a diferença estatística entre os resultados médios das medidas do comprimento cervical obtidas nos exames bi e tridimensional, foi utilizado o teste t pareado. Também foi calculado para essas medidas o coeficiente de correlação de Pearson. RESULTADOS: objetivando-se determinar associação linear entre elas encontramos diferença significante entre as medidas aferidas no plano sagital por meio da ultra-sonografia bidimensional e plano coronal da USG 3D (p=0,009), e na ultra-sonografia tridimensional entre os cortes sagital e coronal (p=0,001), sendo as médias das medidas do comprimento cervical (±desvio padrão) de 3,9±0,92c; 4,1±0,97 e 3,9±0,88 cm, respectivamente. O número de afunilamento em nossa casuística foi de 9/74 exames (12,1%). As ultra-sonogafias bi- e tridimensional visualizaram o mesmo número de afunilamento (8/9). Na ultra-sonografia tridimensional, o corte coronal detectou número maior de funil cervical que o corte sagital (8/9). Não houve diferença significativa entre as médias das medidas do comprimento do colo aferidas por ultra-sonografia bidimensional e tridimensional no plano sagital (p=0,23), entretanto houve diferença entre as médias das medidas da cérvice mensuradas por ultra-sonografia bidimensional no corte sagital e tridimensional no coronal (p=0,009), e na ultra-sonografia tridimensional entre os planos sagital e coronal (p=0,001). Os exames bidimensional e tridimensional visualizaram o mesmo número de funil cervical, demonstrando concordância entre os métodos (teste kappa=0,86). Na comparação das médias das medidas do afunilamento, não houve diferença estatística (p>0,05). CONCLUSÃO: existem diferenças entre as medidas do comprimento cervical, obtidas por ultra-sonografia bidimensional e tridimensional, testando o plano coronal do exame tridimensional.

Valor da ressonância magnética no diagnóstico antenatal do acretismo placentário

OBJETIVOS: estabelecer os principais sinais de acretismo placentário na ressonância magnética (RM) em gestantes com suspeita clínica e avaliar a utilidade do método. MÉTODOS: estudo prospectivo, transversal em 15 pacientes com suspeita de acretismo placentário. O período compreendido foi de março de 2003 a fevereiro de 2006. A idade gestacional variou de 20 a 31 semanas. Todas as pacientes realizaram RM com estudo dirigido para placenta e haviam sido submetidas à ultra-sonografia (US) prévia ao exame. Todas as peças foram encaminhadas para estudo anátomo-patológico (AP). Os exames foram realizados nos equipamentos Magnetom Impact e Sonata Maestro Class Siemens®, adquiridas as seqüências HASTE, TURBO FISP, nos planos axial, sagital e coronal e gradiente echo (GE®) pré- e pós- contraste dinâmico no melhor plano de aquisição. A análise das imagens foi realizada por dois radiologistas em consenso. RESULTADOS: a idade gestacional média das pacientes foi de 24,3 semanas. Foram estudadas sete placentas prévias centro-totais (47%), seis placentas corporais anteriores (40%) e duas placentas corporais posteriores (13%). A US foi positiva em 80% dos casos e a RM em 53% dos casos. No entanto, a US apresentou concordância fraca com o AP pelo teste de kappa (11%), com sensibilidade de 75%, especificidade de 14%, valor preditivo positivo (VPP) de 50% e valor preditivo negativo (VPN) de 33%. Já a RM teve concordância excelente com o AP (0,86), com sensibilidade de 100%, especificidade de 86% , VPP de 89% e VPN de 100%. CONCLUSÃO: a RM é útil na identificação do acretismo placentário. Os principais sinais na RM do acretismo placentário são: o hipersinal transmural, a descontinuidade da parede miometrial nas seqüências rápidas e a identificação dos vasos invadindo o miométrio nas seqüências dinâmicas.

Desenvolvimento das fissuras cerebrais fetais: avaliação com ultrassonografia tridimensional

OBJETIVO: avaliar a distância das fissuras cerebrais fetais à borda interna da calota craniana por meio da ultrassonografia tridimensional (US3D). MÉTODOS: realizou-se um estudo de corte transversal em 80 gestantes normais entre a 21ª e 34ª semanas de gestação. Avaliou-se a distância entre a tábua óssea interna da calota craniana fetal e as fissuras de Sylvius, parieto-occipital, hipocampo e calcarina. Para a obtenção desta distância para as três primeiras fissuras, realizou-se uma varredura tridimensional através do plano axial (nível dos ventrículos laterais). Para a obtenção da distância da fissura calcarina utilizou-se uma varredura coronal (nível dos lobos occiptais). Para avaliar a correlação entre as fissuras e a idade gestacional foram realizadas regressões de primeiro grau, sendo os ajustes calculados pelo coeficiente de determinação (R²). Foram determinados percentis 5, 50 e 95 para cada fissura. Avaliou-se ainda a correlação entre a distância destas fissuras com os diâmetros biparietal (DBP) e circunferência craniana (CC) utilizando o coeficiente de correlação de Pearson (r). RESULTADOS: todas as medidas das fissuras apresentaram correlação linear com a idade gestacional (Sylvius: R²=0,5; parieto-occipital: R²=0,7; hipocampo: R²=0,3 e calcarina: R²=0,3). A média da distância das fissuras variou de 7,0 a 14,0 mm, 15,9 a 28,7 mm, 15,4 a 25,4 mm e 15,7 a 24,8 mm para as fissuras de Sylvius, parieto-occipital, hipocampo e calcarina, respectivamente. As fissuras de Sylvius e parieto-occipital apresentaram as maiores correlações com o DBP (r=0,8 e 0,7, respectivamente) e a CC (r=0,7 e 0,8, respectivamente). CONCLUSÕES: a distância das fissuras cerebrais fetais à borda interna da calota craniana por meio da US3D apresentou correlação positiva com a idade gestacional.

Macroscopic description of teeth of Azara's agouti (Dasyprocta azarae)

The teeth of Azara's agouti (Dasyprocta azarae) were described macroscopically in order to provide biological data on one of the largest wild rodents of the Americas. Radiography was taken on six heads and the teeth were described. Enamel surrounds the coronal dentin, projects to the roots and is present as parallel inner laminae in buccolingual direction. The dentin is located among the enamel laminae and surrounds the pulp horns. The cementum is located internally to the enamel laminae. On the lingual surface, the cementum and dentin are the outer elements.

NADPH-diaphorase activity in area 17 of the squirrel monkey visual cortex: neuropil pattern, cell morphology and laminar distribution

We studied the distribution of NADPH-diaphorase activity in the visual cortex of normal adult New World monkeys (Saimiri sciureus) using the malic enzyme "indirect" method. NADPH-diaphorase neuropil activity had a heterogeneous distribution. In coronal sections, it had a clear laminar pattern that was coincident with Nissl-stained layers. In tangential sections, we observed blobs in supragranular layers of V1 and stripes throughout the entire V2. We quantified and compared the tangential distribution of NADPH-diaphorase and cytochrome oxidase blobs in adjacent sections of the supragranular layers of V1. Although their spatial distributions were rather similar, the two enzymes did not always overlap. The histochemical reaction also revealed two different types of stained cells: a slightly stained subpopulation and a subgroup of deeply stained neurons resembling a Golgi impregnation. These neurons were sparsely spined non-pyramidal cells. Their dendritic arbors were very well stained but their axons were not always evident. In the gray matter, heavily stained neurons showed different dendritic arbor morphologies. However, most of the strongly reactive cells lay in the subjacent white matter, where they presented a more homogenous morphology. Our results demonstrate that the pattern of NADPH-diaphorase activity is similar to that previously described in Old World monkeys

The size and prevalence of the cavum septum pellucidum are normal in subjects with panic disorder

Panic disorder is thought to involve dysfunction in the septohippocampal system, and the presence of a cavum septum pellucidum might indicate the aberrant development of this system. We compared the prevalence and size of cavum septum pellucidum in 21 patients with panic disorder and in 21 healthy controls by magnetic resonance imaging. The length of the cavum septum pellucidum was measured by counting the number of consecutive 1-mm coronal slices in which it appeared. A cavum septum pellucidum of >6 mm in length was rated as large. There was no significant difference in the proportion of patients (16 of 21 or 76.2%) and controls (18 of 21 or 85.7%) with a cavum septum pellucidum (P = 0.35, Fisher's exact test, one-tailed), and no members of either group had a large cavum septum pellucidum. The mean cavum septum pellucidum rating in the patient and control groups was 1.81 (SD = 1.50) and 2.09 (SD = 1.51), respectively. There were also no significant differences between groups when we analyzed cavum septum pellucidum ratings as a continuous variable (U = 196.5; P = 0.54). Across all subjects there was a trend towards a higher prevalence of cavum septum pellucidum in males (100%, 10 of 10) than females (75%, 24 of 32; P = 0.09, Fisher's exact test, one-tailed). Thus, we conclude that, while panic disorder may involve septo-hippocampal dysfunction, it is not associated with an increased prevalence or size of the cavum septum pellucidum.

Abnormalities of hippocampal signal intensity in patients with familial mesial temporal lobe epilepsy

Mesial temporal lobe epilepsy (MTLE) is associated with hippocampal atrophy and hippocampal signal abnormalities. In our series of familial MTLE (FMTLE), we found a high proportion of hippocampal abnormalities. To quantify signal abnormalities in patients with FMTLE we studied 152 individuals (46 of them asymptomatic) with FMTLE. We used NIH-Image® for volumetry and signal quantification in coronal T1 inversion recovery and T2 for all cross-sections of the hippocampus. Values diverging by 2 or more SD from the control mean were considered abnormal. T2 hippocampal signal abnormalities were found in 52% of all individuals: 54% of affected subjects and 48% of asymptomatic subjects. T1 hippocampal signal changes were found in 34% of all individuals: 42.5% of affected subjects and 15% of asymptomatic subjects. Analysis of the hippocampal head (first three slices) revealed T2 abnormalities in 73% of all individuals (74% of affected subjects and 72% of asymptomatic subjects) and T1 abnormalities in 59% (67% of affected subjects and 41% of asymptomatic subjects). Affected individuals had smaller volumes than controls (P < 0.0001). There was no difference in hippocampal volumes between asymptomatic subjects and controls, although 39% of asymptomatic patients had hippocampal atrophy. Patients with an abnormal hippocampal signal (133 individuals) had smaller ipsilateral volume, but no linear correlation could be determined. Hippocampal signal abnormalities in FMTLE were more frequently found in the hippocampal head in both affected and asymptomatic family members, including those with normal volumes. These results indicate that subtle abnormalities leading to an abnormal hippocampal signal in FMTLE are not necessarily related to seizures and may be determined by genetic factors.

RANK, RANKL and osteoprotegerin in arthritic bone loss

Rheumatoid arthritis is characterized by the presence of inflammatory synovitis and destruction of joint cartilage and bone. Tissue proteinases released by synovia, chondrocytes and pannus can cause cartilage destruction and cytokine-activated osteoclasts have been implicated in bone erosions. Rheumatoid arthritis synovial tissues produce a variety of cytokines and growth factors that induce monocyte differentiation to osteoclasts and their proliferation, activation and longer survival in tissues. More recently, a major role in bone erosion has been attributed to the receptor activator of nuclear factor kappa B ligand (RANKL) released by activated lymphocytes and osteoblasts. In fact, osteoclasts are markedly activated after RANKL binding to the cognate RANK expressed on the surface of these cells. RANKL expression can be upregulated by bone-resorbing factors such as glucocorticoids, vitamin D3, interleukin 1 (IL-1), IL-6, IL-11, IL-17, tumor necrosis factor-alpha, prostaglandin E2, or parathyroid hormone-related peptide. Supporting this idea, inhibition of RANKL by osteoprotegerin, a natural soluble RANKL receptor, prevents bone loss in experimental models. Tumor growth factor-ß released from bone during active bone resorption has been suggested as one feedback mechanism for upregulating osteoprotegerin and estrogen can increase its production on osteoblasts. Modulation of these systems provides the opportunity to inhibit bone loss and deformity in chronic arthritis.

The importance of accurate anatomic assessment for the volumetric analysis of the amygdala

There is a wide range of values reported in volumetric studies of the amygdala. The use of single plane thick magnetic resonance imaging (MRI) may prevent the correct visualization of anatomic landmarks and yield imprecise results. To assess whether there is a difference between volumetric analysis of the amygdala performed with single plane MRI 3-mm slices and with multiplanar analysis of MRI 1-mm slices, we studied healthy subjects and patients with temporal lobe epilepsy. We performed manual delineation of the amygdala on T1-weighted inversion recovery, 3-mm coronal slices and manual delineation of the amygdala on three-dimensional volumetric T1-weighted images with 1-mm slice thickness. The data were compared using a dependent t-test. There was a significant difference between the volumes obtained by the coronal plane-based measurements and the volumes obtained by three-dimensional analysis (P < 0.001). An incorrect estimate of the amygdala volume may preclude a correct analysis of the biological effects of alterations in amygdala volume. Three-dimensional analysis is preferred because it is based on more extensive anatomical assessment and the results are similar to those obtained in post-mortem studies.

Differential patterns of myosin Va expression during the ontogenesis of the rat hippocampus

Myosin Va is an actin-based, processive molecular motor protein highly enriched in the nervous tissue of vertebrates. It has been associated with processes of cellular motility, which include organelle transport and neurite outgrowth. The in vivo expression of myosin Va protein in the developing nervous system of mammals has not yet been reported. We describe here the immunolocalization of myosin Va in the developing rat hippocampus. Coronal sections of the embryonic and postnatal rat hippocampus were probed with an affinity-purified, polyclonal anti-myosin Va antibody. Myosin Va was localized in the cytoplasm of granule cells in the dentate gyrus and of pyramidal cells in Ammon's horn formation. Myosin Va expression changed during development, being higher in differentiating rather than already differentiated granule and pyramidal cells. Some of these cells presented a typical migratory profile, while others resembled neurons that were in the process of differentiation. Myosin Va was also transiently expressed in fibers present in the fimbria. Myosin Va was not detected in germinative matrices of the hippocampus proper or of the dentate gyrus. In conclusion, myosin Va expression in both granule and pyramidal cells showed both position and time dependency during hippocampal development, indicating that this motor protein is under developmental regulation.