Neuropsychological long-term sequelae after posterior fossa tumour resection during childhood

The importance of the cerebellum for non‐motor functions is becoming more and more evident. The influence on cognitive functions from acquired cerebellar lesions during childhood, however, is not well known. We present follow‐up data from 24 patients, who were operated upon during childhood for benign cerebellar tumours. The benign histology of these tumours required neither radiotherapy nor chemotherapy. Post‐operatively, these children were of normal intelligence with a mean IQ of 99.1, performance intelligence quotient (PIQ) of 101.3 and verbal intelligence quotient (VIQ) of 96.8. However, 57% of patients showed abnormalities in subtesting. In addition, more extensive neuropsychological testing revealed significant problems for attention, memory, processing speed and interference. Visuo‐constructive problems were marked for copying the Rey figure, but less pronounced for recall of the figure. Verbal fluency was more affected than design fluency. Behavioural deficits could be detected in 33% of patients. Attention deficit problems were marked in 12.5%, whereas others demonstrated psychiatric symptoms such as mutism, addiction problems, anorexia, uncontrolled temper tantrums and phobia. Age at tumour operation and size of tumour had no influence on outcome. Vermis involvement was related to an increase in neuropsychological and psychiatric problems. The observation that patients with left‐sided cerebellar tumours were more affected than patients with right‐sided tumours is probably also influenced by a more pronounced vermian involvement in the former group. In summary, this study confirms the importance of the cerebellum for cognitive development and points to the necessity of careful follow‐up for these children to provide them with the necessary help to achieve full integration into professional life.

As compreensões do construtivismo de Ernst Von Glasersfeld e John Fossa: intermediando um diálogo em busca de novas significações

The present thesis, orientated by a letter sent by Ernst von Glasersfeld to John Fossa, is the product of a theoretical investigation of radical constructivism. In this letter, von Glasersfeld made three observations about Fossa’s understanding of radical constructivism. However, we limited our study to the second of these considerations since it de als with some of the core issues of constructivism. Consequently, we investigated what issues are raised by von Glasersfeld’s observation and whether these issues are relevant to a better understanding of constructivism and its implications for the mathema tics classroom . In order to realize the investigation, it was necessary to characterize von Glasersfeld’s epistemological approach to constructivism, to identify which questions about radical constructivism are raised by von Glasersfeld’s observation, to i nvestigate whether these issues are relevant to a better understanding of constructivism and to analyze the implications of these issues for the mathematics classroom. Upon making a hermeneutic study of radical constructivism, we found that what is central to it is its radicalism, in the sense that it breaks with tradition by its absence of an ontology. Thus, we defend the thesis that the absence of an ontology, although it has advantages for radical constructivism, incurs serious problems not only for the theory itself, but also for its implications for the mathematics classroom. The advantages that we were able to identify include a change from the usual philosophical paths to a very different rational view of the world, an overcoming of a naive way of thi nking, an understanding of the subject as active in the construction of his/her experiential reality, an interpretation of cognition as an instrument of adaptation, a new concept of knowledge and a vision of knowledge as fallible (or provisional). The prob lems are associated with the impossibility of radical constructivism to explain adequately why the reality that we build up is regular, stable, non - arbitrary and publicly shared. With regard to the educational implications of radical constructivism, the ab sence of an ontology brings to the mathematics classroom not only certain relevant aspects (or favorable points) that make teaching a process of researching student learning, empowering the student to learn and changing the classroom design, but also certa in weaknesses or limitations. These weaknesses or limitations of constructivism in the classroom are due to its conception of knowledge as being essentially subjective. This requires it to work with one - on - one situations and, likewise, makes the success of teaching dependent on the teacher’s individual skills. Perhaps the most important weakness or limitation, in this sense, is that it makes teaching orientated by constructivist principles unable to reach the goal of the formation of a community. We conclud e that issues raised by von Glasersfeld’s observation are absolutely relevant to the context of a better understanding of radical constructivism and its implications for education, especially for Mathematics Education.

Arterial entrapment syndrome in the cubital fossa: a rare cause of acute stress-related arterial thrombosis in a patient with brachial artery duplication

Arterial entrapment syndrome (AES) at elbow level is very rare and to our knowledge no case of AES by lacertus fibrosus in the cubital fossa in presence of brachial artery duplication has been described to date. We describe a rare case of acute arterial thrombosis of one of two brachial arteries highlighted in the cubital fossa which developed after strenuous right elbow flexor muscle activity and hyper-extensions presumably related to AES by lacertus fibrosus at elbow level. A 43-year-old right-handed woman, experienced paleness, coldness and numbness of the right hand, after 8 consecutive hours of gardening. As she worked, her ipsilateral flexor elbow muscles remained in prolonged and inappropriate tension. Clinical examination evidenced the absence of radial artery pulse in the wrist and mild hypothermia in the second and third finger. During surgical exploration two anastomosed brachial arteries were detected in the cubital fossa under the lacertus fibrosus. The lateral superficial brachial artery was occluded. Intraoperative arteriography evidenced brachial artery duplication at the third superior of the arm and normal vascular pattern at the forearm level. In cases of unexplained atypical intermittent upper extremity claudication or acute ischemic symptoms an AES should always be ruled out, particularly when symptoms are exacerbated by strenuous upper extremity activity or when upper limb muscular hypertrophy is evident. In these cases a thorough dynamic clinical and instrumental examination is mandatory to confirm a diagnosis of AES and to avoid possible future ischemic complications.

Lavage treatment of distal intestinal obstruction syndrome in children with cystic fibrosis

The efficacy, adverse reactions, and long-term effects of intestinal lavage treatment with a balanced electrolyte solution (Golytely) was evaluated in patients with cystic fibrosis and distal intestinal obstruction syndrome. Twenty-two patients with cystic fibrosis (mean age 21.8 years, range 14 to 34 years, 15 boys or men) who sough medical attention because of abdominal pain and a mass in the right iliac fossa received Golytely, 5.6 ± 1.9 L (mean ± 1 SD), either orally (n = 14) or via nasogastric tube (n = 8) during 5.6 ± 2.4 hours. No serious side effects occurred. Serum electrolyte values remained within normal limits. Body weight did not change significantly. Minor adverse reactions included bloating (n = 12), nausea (n = 8), vomiting (n = 1), and chills (n = 3). All but one patient reported impressive relief of symptoms and remained pain free for an average of 3 months (range 1 to 19 months). Symptoms of abdominal pain and radiologic signs of fecal impaction assessed before and after lavage both decreased significantly (P < .0001). During follow-up (mean 15.2 months, range 4 to 26 months), 11 patients required a total of 38 (range one to nine) additional doses of Golytely. Seven patients drank the solution at home (21 treatments); only two patients chose a nasogastric tube. In ten patients with symptoms of recurrent distal intestinal obstruction syndrome prior to institution of therapy, duration of hospitalization was significantly reduced by this treatment (5.1 ± 7.6 v 2.3 ± 6.3 hospital days per annum, P < .02). It is concluded that intestinal lavage is a well-accepted, safe, and effective therapy for distal intestinal obstruction syndrome in patients with cystic fibrosis.

Atrial Electric Signal During Sinus Rhythm in Lone Paroxysmal Atrial Fibrillation

Atrial fibrillation (AF) is the most common tachyarrhythmia and is associated with substantial morbidity, increased mortality and cost. The treatment modalities of AF have increased, but results are still far from optimal. More individualized therapy may be beneficial. Aiming for this calls improved diagnostics. Aim of this study was to find non-invasive parameters obtained during sinus rhythm reflecting electrophysiological patterns related to propensity to AF and particularly to AF occurring without any associated heart disease, lone AF. Overall 240 subjects were enrolled, 136 patients with paroxysmal lone AF and 104 controls (mean age 45 years, 75% males). Signal measurements were performed by non-invasive magnetocardiography (MCG) and by invasive electroanatomic mapping (EAM). High-pass filtering techniques and a new method based on a surface gradient technique were adapted to analyze atrial MCG signal. The EAM was used to elucidate atrial activation in patients and as a reference for MCG. The results showed that MCG mapping is an accurate method to detect atrial electrophysiologic properties. In lone paroxysmal AF, duration of the atrial depolarization complex was marginally prolonged. The difference was more obvious in women and was also related to interatrial conduction patterns. In the focal type of AF (75%), the root mean square (RMS) amplitudes of the atrial signal were normal, but in AF without demonstrable triggers the late atrial RMS amplitudes were reduced. In addition, the atrial characteristics tended to remain similar even when examined several years after the first AF episodes. The intra-atrial recordings confirmed the occurrence of three distinct sites of electrical connection from right to left atrium (LA): the Bachmann bundle (BB), the margin of the fossa ovalis (FO), and the coronary sinus ostial area (CS). The propagation of atrial signal could also be evaluated non-invasively. Three MCG atrial wave types were identified, each of which represented a distinct interatrial activation pattern. In conclusion, in paroxysmal lone AF, active focal triggers are common, atrial depolarization is slightly prolonged, but with a normal amplitude, and the arrhythmia does not necessarily lead to electrical or mechanical dysfunction of the atria. In women the prolongation of atrial depolarization is more obvious. This may be related to gender differences in presentation of AF. A significant minority of patients with lone AF lack frequent focal triggers, and in them, the late atrial signal amplitude is reduced, possibly signifying a wider degenerative process in the LA. In lone AF, natural impulse propagation to LA during sinus rhythm goes through one or more of the principal pathways described. The BB is the most common route, but in one-third, the earliest LA activation occurs outside the BB. Susceptibility to paroxysmal lone AF is associated with propagation of the atrial signal via the margin of the FO or via multiple pathways. When conduction occurs via the BB, it is related with prolonged atrial activation. Thus, altered and alternative conduction pathways may contribute to pathogenesis of lone AF. There is growing evidence of variability in genesis of AF also within lone paroxysmal AF. Present study suggests that this variation may be reflected in cardiac signal pattern. Recognizing the distinct signal profiles may assist in understanding the pathogenesis of AF and identifying subgroups for patient-tailored therapy.

Shwachman-Diamond syndrome : Clinical, genetic and radiological study

Shwachman-Diamond syndrome (SDS) is a rare autosomal recessive disorder in which the cardinal symptoms arise from exocrine pancreatic insufficiency and bone marrow dysfunction. Previous studies have suggested increased risk of fatal complications among Finnish SDS infants. The genetic defect responsible for the disease was recently identified; the SBDS gene is located at chromosome 7q11 and encodes a protein that is involved in ribosome biosynthesis. The discovery of the SBDS gene has opened new insights into the pathogenesis of this multi-organ disease. This study aimed to assess phenotypic and genotypic features of Finnish patients with SDS. Seventeen Finnish patients with a clinical diagnosis of SDS were included in the study cohort. Extensive clinical, biochemical and imaging assessments were performed to elucidate the phenotypic features, and the findings were correlated with the SBDS genotype. Imaging studies included abdominal magnetic reso-nance imaging (MRI), brain MRI, cardiac echocardiography including tissue Doppler examination, and cardiac MRI. The skeletal phenotype was assessed by dual-energy X-ray absorptiometry and bone histomorphometry. Twelve patients had mutations in the SBDS gene. In MRI, a characteristic pattern of fat-replaced pancreas with occasional enhancement of scattered parenchymal foci and of pancreatic duct was noted in the SBDS mutation-positive patients while the mutation-negative patients did not have pancreatic fat accumulation. The patients with SBDS mutations had significantly reduced bone mineral density associated with low-energy peripheral fractures and vertebral compression fractures. Bone histomorphometry confirmed low-turnover osteoporosis. The patients with SBDS mutations had learning difficulties and smaller head size and brain volume than control subjects. Corpus callosum, cerebellar vermis, and pos-terior fossa structures were significantly smaller in SDS patients than in controls. Patients with SDS did not have evidence of clinical heart disease or myocardial fibrosis. However, subtle diastolic changes in the right ventricle and exercise-induced changes in the left ventricle contractile reserve were observed. This study expanded the phenotypic features of SDS to include primary low-turnover osteoporosis and structural alterations in the brain. Pancreatic MRI showed characteristic changes in the SBDS mutation-positive patients while these were absent in the mutation-negative patients, suggesting that MRI can be used to differentiate patients harbouring SBDS mutations from those without mutations. No evidence for clinical cardiac manifestations was found, but imaging studies revealed slightly altered myocardial function that may have clinical implications. These findings confirm the pleiotropic nature of SDS and underscore the importance of careful multidisciplinary follow-up of the affected individuals.

The grumpy stump - ultrasound and stump appendicitis

BACKGROUND: Stump appendicitis, the inflammation of an incompletely removed appendix, is a rare clinical presentation. Sonography can be useful in the diagnosis of the condition; by either directly visualising the inflamed stump, or by identifying signs of peri-caecal inflammation that can raise suspicion of the condition. SUMMARY OF WORK: A potential case of stump appendicitis was identified. This prompted a review of literature focused on the incidence of stump appendicitis, utility of ultrasound to identify an inflamed appendiceal stump, and surgical techniques used in appendectomy. SUMMARY OF RESULTS: Stump appendicitis is rare, with as few as 61 cases identified in literature during the last 60 years1. Of the two common techniques of appendectomy, which are ligation and invagination, the former can sometimes leave a residual stump that acts as a potential lumen for the pathophysiological process of appendicitis to recur. Established sonographic criteria for appendicitis also apply to the residual stump. Sonographic secondary signs that suggest the presence of acute appendicitis2 have also been demonstrated in cases of stump appendicitis3, even in the absence of an identifiable stump. DISCUSSION AND CONCLUSIONS: Appendicitis is usually dismissed in patients with a history of appendectomy. Though uncommon, sonographers should be aware of stump appendicitis in post-appendectomy patients that present with right iliac fossa pain from months to decades later. REFERENCE(S) 1. Subramanian A, Liang MK. A 60-year literature review of stump appendicitis: The need for a critical view. Am J Surg; 2012;203(4):503–7. 2. Reddan T, Corness J, Mengersen K, Harden F. Ultrasound of paediatric appendicitis and its secondary sonographic signs: providing a more meaningful finding. J Med Radiat Sci. 2015;DOI: 10.1002/jmrs.154 3. Martínez Chamorro E, Merina Castilla A, Muñoz Fraile B, Koren Fernández L, Borruel Nacenta S. Stump appendicitis: Preoperative imaging findings in four cases. Abdom Imaging. 2013;38(6):1214–9.

Distribuição de tensões em próteses parciais fixas posteriores livres de metal com retentores intracoronários: análise em elementos finitos

Para reabilitar a ausência de um elemento dentário posterior, as próteses parciais fixas (PPF) com retentores intracoronários são uma alternativa aos implantes osseointegrados. O objetivo deste estudo foi avaliar a distribuição de tensões nessas próteses com três combinações de materiais: cerâmica de zircônia parcialmente estabilizada por ítria (ZPEI) revestida por cerâmica de fluorapatita (α), cerâmica de dissilicato de lítio (β) ou compósito fibrorreforçado (γ). Na composição α, foram analisadas a presença ou ausência da cerâmica de revestimento na parede cervical das caixas proximais e três variações na área total da seção transversal dos conectores (4 mm de largura x 3,2, 4,2 ou 5,2 mm de altura). Em 8 modelos bidimensionais de elementos finitos, uma carga vertical de 500 N foi aplicada na fossa central do pôntico e as tensões principais máximas (tração) e mínimas (compressão) foram apontadas em MPa. Inicialmente foram avaliados os 6 modelos com PPF de ZPEI e suas variações. Os maiores valores das tensões de tração foram encontrados no terço cervical dos conectores. Quando presente nestas regiões, a cerâmica de revestimento recebeu tensões acima do limite de sua resistência à flexão. Na comparação entre os modelos sem cerâmica de revestimento na parede cervical das caixas proximais, mesmo aquele com conectores de 3,2 x 4 mm, cuja infraestrutura apresentava 2,5 x 3 mm, poderia ser recomendado para uso clínico. Altos valores de tensões de compressão foram registrados entre o terço oclusal e médio dos conectores, correspondente à união entre as cerâmicas, o que poderia ocasionar, devido à flexão, falhas adesivas. Posteriormente, o modelo de ZPEI com a cerâmica de fluorapatita ausente da parede cervical das caixas proximais e área total dos conectores de 4,2 x 4 mm foi comparado aos dois outros materiais com conectores de mesma área. Na PPF de dissilicato de lítio, os valores representaram uma provável violação do limite de sua resistência à flexão. A PPF de compósito fibrorreforçado apresentou tensões bem abaixo do limite de resistência à flexão de sua infraestrutura, mas, como no modelo de ZPEI, tensões compressivas se concentraram com alto valor entre o terço oclusal e médio dos conectores, local de união entre a resina composta e a infraestrutura de fibras. Os resultados mostraram que a cerâmica de dissilicato de lítio e a presença da cerâmica de fluorapatita na parede cervical das caixas proximais deveriam ser contraindicadas para a condição proposta. Parece viável uma área de conectores na infraestrutura de ZPEI com no mínimo 2,5 x 3 mm. A PPF de compósito fibrorreforçado apresenta resistência estrutural para a situação estudada, mas, como também aquelas compostas de ZPEI, aparenta ter como pontos fracos a adesão entre a infraestrutura e o material de cobertura e a própria resistência deste último.

Desempenho de tanques vegetados em um ecossistema engenheirado para tratamento descentralizado de esgotos domiciliares na Ilha Grande, RJ

A falta de infraestrutura adequada para a coleta e o tratamento de esgotos atinge várias comunidades costeiras e de sistemas insulares do Estado do Rio de Janeiro, que vivem em condições de insalubridade. O objetivo do presente estudo foi avaliar o desempenho de um ecossistema engenheirado para tratamento descentralizado de esgoto domiciliar implantado no Centro de Estudos Ambientais e Desenvolvimento Sustentável (CEADS) da UERJ, na Vila Dois Rios, Ilha Grande - RJ, com ênfase na contribuição de macrófitas e algas em tanques vegetados como tratamento terciário para a melhoria geral da qualidade do efluente e em especial, para remoção de nutrientes. O sistema é composto por caixa controladora de vazão, caixa de gordura, fossa séptica, filtro aerado submerso, decantador secundário e o conjunto de tanques vegetados contendo as macrófitas aquáticas Eichhornia crassipes, Schoenoplectus sp., Panicum cf. racemosum + Cyperus ligularis, além de um tanque de algas entre as 2 primeiras macrófitas. Os principais parâmetros monitorados em amostras quinzenais obtidas durante o período de 09/2009 a 07/2010 em 10 pontos de coleta equivalentes à saída do efluente de cada unidade de tratamento foram: OD, pH, Cond. Elétrica, Temperatura, DBO5, DQO, SST, SDT, Nitrogênio amoniacal, Nitrito, Nitrato e Fósforo total. A vazão média do sistema foi 52 L/h. A produção primária de biomassa das macrófitas e o metabolismo no tanque com algas também foram estimados. A eficiência média do ecossistema engenheirado foi de 93% de redução dos SST, 93% de DBO, 90% da DQO, 30% de N amoniacal e, 38% de Fósforo. O conjunto de tanques vegetados promoveu um aumento médio de oxigênio dissolvido (OD) em 182% comparados ao valor de saída do decantador. Pode-se concluir que esse sistema contribuiu para uma melhoria geral da qualidade do efluente final com relação a praticamente todos os parâmetros, ainda que em alguns casos, apenas de forma incremental. Houve maior produção média de biomassa (peso seco.dia-1) e retenção média de água na espécie E. crassipes (aguapé) em comparação com as espécies Schoenoplectus sp, P. cf. racemosum e C. ligularis. A baixa produção primária no tanque de algas em relação à respiração, pelo menos no período monitorado, indica condições desfavoráveis à remoção de nutrientes (N e P) nesse tanque através da incorporação na biomassa. Uma investigação mais detalhada acerca dos parâmetros de projeto que inclua um eventual redimensionamento do decantador e do tanque de algas é sugerida.

Fatores associados à positividade da intradermorreação de Montenegro em Teresina, Piauí

A leishmaniose visceral americana (LVA) ou Calazar é um exemplo de doença infecciosa reemergente, cuja urbanização a uma das principais marcas da alteração de seu padrão epidemiológico de ocorrência. No Brasil, a infecção por L. chagasi tem se comportado de forma epidêmica em diversas regiões e as estratégias de controle não tem sido efetivas na contenção da propagação da doença. Para melhor compreensão deste processo, estudaram-se associações da imunidade tardia, medida por meio do teste de intradermorreação de Montenegro (IDRM), com fatores demográficos, sociais, econômicos e ambientais em área urbana com alta forca de transmissão. A análise dos dados foi feita tendo como base o modelo de regressão de Poisson ajustado pelo qui-quadrado, onde foram calculadas as razões de prevalência (RP) e seus respectivos intervalos de 95% de confiança (IC). Foi detectada maior prevalência da positividade a IDRM para o sexo masculino (RP = 1,32 IC: 1,11 1,56), com o aumento da idade (RP = 1,18 IC: 1,10 1,26), com a posse de cao por período de três anos ou mais (RP = 1,26 IC: 1,01 1,56) e com presença de fossa rudimentar no domicilio (RP = 1,33 IC: 0,99 1,77). Indivíduos com maior escolaridade (RP = 0,81 IC; 0,66 0,99), convivendo com três ou mais pessoas no domicilio (RP = 0,66 IC; 0,47 0,93), com história de Calazar no domicílio (RP = 0,40 IC: 0,16 0,99) e com parede com tijolo sem reboco (RP= 0,79 IC: 0,64 0,98) apresentaram prevalências mais baixas mostrando possível proteção infecção anterior. Os resultados obtidos podem contribuir para o melhor entendimento do dinamismo da doença em contexto urbano, permitindo melhor direcionamento das estratégias de intervenção.