Assistência de Enfermagem ao Doente com Cirrose Hepática

Este trabalho cujo título é a Assistência de Enfermagem ao doente com cirrose hepática realça a necessidade de dotar cada vez mais os profissionais de enfermagem de conhecimentos à cerca dessa patologia e de como cuidar da melhor forma dos utentes afectados. O objectivo principal desse trabalho é identificar a influência dos valores pessoais dos enfermeiros na prestação de cuidados ao doente com Cirrose Hepática. A cirrose é uma patologia crônica que afecta milhares de pessoas em todo o mundo, sendo responsável por uma grande percentagem de mortes no mundo inteiro, contribuindo significativamente pelo aumento das taxas de mortalidade, principalmente na Europa. O enfermeiro é o profissional de saúde que está 24 horas com o utente pelo que se revela pertinente saber até que ponto os valores pessoais desses profissionais podem influenciar na prática clínica, junto a esses doentes, sabendo que é uma patologia muitas vezes provocada pelo próprio quando ingere em demasia e por tempo indeterminado certas quantidades de álcool, estando também, por este motivo, sujeito a outros factores de risco. Sendo este um problema evitável e prevenível. Trata-se de um estudo qualitativo, descritivo e exploratório, de abordagem fenomenológica cujo método de recolha de dados elegido é a entrevista estruturada. A população alvo foi constituída por 5 enfermeiros do serviço de medicina com mais de 10 anos de experiência. Os resultados evidenciaram que os valores pessoais dos enfermeiros influenciam de forma positiva na prestação dos cuidados ao doente com cirrose hepática no serviço de Medicina. A maioria dos participantes aponta que os sentimentos na prática clínica são orientados pelo lado positivo e que não afectam negativamente no desempenho das suas funções. E foi também evidenciado que conhecem a realidade Cabo-verdiana, frequentemente acolhem esses utentes no serviço e sabem como lidar com estes e não sentem nenhum tipo de preconceito ou discriminação.

Humanização dos cuidados de enfermagem no atendimento ao doente etílico

O alcoolismo é considerado um problema a nível mundial, em Cabo Verde é a 3ª causa de morte e é responsável por uma alta taxa de mortalidade que deve ser encarada como tal, com profundidade, merecendo atenção adequada, por parte da sociedade, particularmente do Estado no que concerne à adopção de medidas visando o seu combate. O aumento do consumo e do número de usuários tem levado a agravos decorrentes da dependência desta substância, havendo maior procura pelos serviços de Banco de Urgência. Os cuidados às pessoas com problemas relacionados ao consumo do álcool, no entanto, ainda estão pautados em modelos tradicionais, punitivos e segregadores que dificultam o acesso humanizado aos serviços de saúde. Constata-se que a humanização é a característica fundamental de uma administração eficiente, pois deve estar presente em todos os cuidados de saúde prestados aos doentes, com a finalidade de garantir o bem-estar físico, psíquico, social e moral do doente. A humanização enfatiza a prestação de cuidados nos serviços de saúde, neste sentido considera importante desenvolver um estudo intitulado humanização assistência de enfermagem no atendimento ao doente etílico, tendo como objetivo geral conhecer a perceção dos enfermeiros de BUA acerca da humanização do cuidado prestado ao doente etílico. Para melhor compreender os objetivos do trabalho optou-se por uma abordagem qualitativa, com um estudo de caráter descritivo e exploratório utilizando como método de colheita de dados uma entrevista semi-estruturada com perguntas abertas, feita a 9 enfermeiros que trabalham no serviço. Relativamente aos dados obtidos, constata-se que os profissionais de enfermagem do Serviço, têm alguma noção da Humanização dos Cuidados embora não de forma técnica e científica. Os enfermeiros afirmam ainda que existem dificuldades na implementação deste conceito por ser um serviço de urgência o tempo é pouco, à falta de recursos materiais, humanos e físicos para implementação deste cuidado neste serviço. Deste modo, os resultados desta pesquisa fornecem um contributo enorme, servindo como fonte de informação para o serviço, de modo a que os profissionais de saúde possam implementar novas metas que visem a melhorar as condições de saúde do doente, e os próprios profissionais desse Serviço.

A Enfermagem Orto-pediátrica e o pé boto

A realização da presente investigação constituiu um grande desafio visto que o fenómeno abordado trata-se de um tema ainda pouco explorado na área de enfermagem a nível mundial e consequentemente em Cabo Verde. A escolha deste tema deve-se ao fato do pé boto ser uma patologia congénita, de causas ainda indeterminadas e portanto exige certos cuidados por parte da enfermagem, sendo necessário conhecimentos sólidos nessa área, que permitam prestar os cuidados adequados, ajudando desse modo na identificação e diagnóstico precoce da doença, uma vez que, o pé boto quando não tratado ou negligenciado resulta em limitações e incapacidades físicas para a criança doente. Seguindo esses pressupostos elaborou-se o presente trabalho que tem por objetivo, conhecer a perceção das mães sobre os cuidados de Enfermagem prestados às crianças com pé boto, no Serviço de Orto-Traumatologia do Hospital Baptista de Sousa. Para alcançar o objetivo preconizado utilizou-se como abordagem metodológica a metodologia qualitativa de caráter descritivo e fenomenológico e como instrumento de recolha de dados a entrevista estruturada que foi aplicado a seis (6) mães de crianças com pé boto. Desta pesquisa constata-se que o enfermeiro detém uma importante função na assistência e prestação de cuidados às crianças com pé boto e suas famílias, visto ser o profissional de saúde que está mais próximo dos utentes prestando os cuidados adequados as suas necessidades. Os enfermeiros também possuem grande importância na assistência aos familiares das crianças com pé boto, oferecendo-lhes as informações adequadas sobre a patologia e capacitando-os a cuidar da criança durante o longo tratamento. Com a análise dos dados colhidos pode-se concluir que as mães das crianças com pé boto, não estão satisfeitas com as intervenções de enfermagem prestadas aos filhos.

Neonatal treatment of CINCA syndrome.

ABSTRACT: Chronic Infantile Neurological Cutaneous Articular (CINCA) syndrome, also called Neonatal Onset Multisystem Inflammatory Disease (NOMID) is a chronic disease with early onset affecting mainly the central nervous system, bones and joints and may lead to permanent damage. We report two preterm infants with severe CINCA syndrome treated by anti-interleukin-1 in the neonatal period, although, so far, no experience with this treatment in infants younger than three months of age has been reported. A review of the literature was performed with focus on treatment and neonatal features of CINCA syndrome. CASE REPORT: Two cases suspected to have CINCA syndrome were put on treatment with anakinra in the early neonatal period due to severe clinical presentation. We observed a rapid and persistent decline of clinical signs and systemic inflammation and good drug tolerance. Diagnosis was confirmed in both cases by mutations in the NLRP3/CIAS1-gene coding for cryopyrin. As particular neonatal clinical signs polyhydramnios and endocardial overgrowth are to be mentioned. CONCLUSION: We strongly suggest that specific treatment targeting interleukin-1 activity should be started early. Being well tolerated, it can be introduced already in neonates presenting clinical signs of severe CINCA syndrome in order to rapidly control inflammation and to prevent life-long disability.

Hipoglucèmia neonatal en fills de mares que han presentat diabetis gestacional

La hipoglucèmia neonatal (HN) és un problema de salut freqüent en les primeres hores de vida del recent nascut (RN) especialment de mares que pateixen diabetis mellitus (DM) durant l’embaràs, més coneguda com a diabetis gestacional (DG). La diabetis gestacional és un dels principals factors de risc que condiciona a un nadó a presentar hipoglucèmia en les hores posteriors al naixement, per això, els professionals d’infermeria necessiten conèixer la millor manera d’atendre a la dona embarassada que pateix DG amb l’objectiu d’ensenyar-li unes cures personals especifiques per intentar disminuir la incidència de recent nascuts (RN) que presenten hipoglucèmia.

SOST expression is restricted to the great arteries during embryonic and neonatal cardiovascular development.

Spatial-temporal regulation of bone morphogenetic protein (BMP) and Wnt activity is essential for normal cardiovascular development, and altered activity of these growth factors causes maldevelopment of the cardiac outflow tract and great arteries. In the present study, we show that SOST, a Dan family member reported to antagonize BMP and Wnt activity, is expressed within the medial vessel wall of the great arteries containing smooth muscle cells. The ascending aorta, aortic arch, brachiocephalic artery, common carotids, and pulmonary trunk were all associated with SOST expressing smooth muscle cells, while the heart itself, including the valves, and more distal arteries, that is, pulmonary arteries, subclavian arteries, and descending aorta, were negative. SOST was expressed from embryonic day 15.5 up to the neonatal period. SOST expression, however, did not correspond with inhibition of Smad-dependent BMP activity or beta-catenin-dependent Wnt activity in the great arteries. Activity of both signaling pathways was already down-regulated before induction of SOST expression.

Multi-modal assessment of long-term erythropoietin treatment after neonatal hypoxic-ischemic injury in rat brain.

Erythropoietin (EPO) has been recognized as a neuroprotective agent. In animal models of neonatal brain injury, exogenous EPO has been shown to reduce lesion size, improve structure and function. Experimental studies have focused on short course treatment after injury. Timing, dose and length of treatment in preterm brain damage remain to be defined. We have evaluated the effects of high dose and long-term EPO treatment in hypoxic-ischemic (HI) injury in 3 days old (P3) rat pups using histopathology, magnetic resonance imaging (MRI) and spectroscopy (MRS) as well as functional assessment with somatosensory-evoked potentials (SEP). After HI, rat pups were assessed by MRI for initial damage and were randomized to receive EPO or vehicle. At the end of treatment period (P25) the size of resulting cortical damage and white matter (WM) microstructure integrity were assessed by MRI and cortical metabolism by MRS. Whisker elicited SEP were recorded to evaluate somatosensory function. Brains were collected for neuropathological assessment. The EPO treated animals did not show significant decrease of the HI induced cortical loss at P25. WM microstructure measured by diffusion tensor imaging was improved and SEP response in the injured cortex was recovered in the EPO treated animals compared to vehicle treated animals. In addition, the metabolic profile was less altered in the EPO group. Long-term treatment with high dose EPO after HI injury in the very immature rat brain induced recovery of WM microstructure and connectivity as well as somatosensory cortical function despite no effects on volume of cortical damage. This indicates that long-term high-dose EPO induces recovery of structural and functional connectivity despite persisting gross anatomical cortical alteration resulting from HI.

Composition of weight gain during the neonatal period and longitudinal growth follow-up in premature babies.

Changes in the rate of growth and adiposity index (Quetelet index), calculated as weight/(length)2, kg/m2, were monitored from birth to 3 years in 19 premature babies (post-conceptional age 31.2 +/- 2 weeks) who were subjected during rapid growth (16 +/- 4 g/kg.day) to initial metabolic balance studies in the first weeks of life. These studies showed that the rate of fat accretion in these infants (3.3 +/- 0.9 g/kg.day) was substantially greater than that observed in fetuses of the same gestational age (2 g/kg.day) but the adiposity index was lower (9.6 +/- 1 kg/m2) than intrauterine values (11 kg/m2). Since at 6 months of age (corrected for gestational age at birth) the adiposity index was close to normality (103% of standard), the greater rate of fat accretion in early life contributed to progressively restore total body fat in premature babies. It is concluded that despite substantial fat deposition during the first weeks of life, the future evolution of these premature babies is favourable as judged from the normalization of adiposity index within the first 2 years of life.

Maturation of the lymphoid system. III. Induction of selective unresponsiveness by injection of a haptenated carbohydrate into neonatal mice.

Neonatal treatment of A/J mice with DNP-Ficoll reduced or eliminated indirect anti-DNP PFC normally produced in response to adult challenge with DNP-keyhole limpet hemocyanin. The remaining direct anti-DNP PFC response was of low avidity. Spleen cells from neonatal A/J mice inhibited the in vitro but not the in vivo response of adult spleen cells to DNP-Ficoll.

Neonatal steroids induce a down-regulation of tenascin-C and elastin and cause a deceleration of the first phase and an acceleration of the second phase of lung alveolarization.

Pre- and postnatal corticosteroids are often used in perinatal medicine to improve pulmonary function in preterm infants. To mimic this clinical situation, newborn rats were treated systemically with dexamethasone (Dex), 0.1-0.01 mg/kg/day on days P1-P4. We hypothesized that postnatal Dex may have an impact on alveolarization by interfering with extracellular matrix proteins and cellular differentiation. Morphological alterations were observed on 3D images obtained by high-resolution synchrotron radiation X-ray tomographic microscopy. Alveolarization was quantified stereologically by estimating the formation of new septa between days P4 and P60. The parenchymal expression of tenascin-C (TNC), smooth muscle actin (SMA), and elastin was measured by immunofluorescence and gene expression for TNC by qRT-PCR. After Dex treatment, the first phase of alveolarization was significantly delayed between days P6 and P10, whereas the second phase was accelerated. Elastin and SMA expressions were delayed by Dex treatment, whereas TNC expression was delayed and prolonged. A short course of neonatal steroids impairs the first phase of alveolarization, most likely by altering the TNC and elastin expression. Due to an overshooting catch-up during the second phase of alveolarization, the differences disappear when the animals reach adulthood.

Method for retinal gene repair in neonatal mouse.

Gene correction at the site of the mutation in the chromosome is the absolute way to really cure a genetic disease. The oligonucleotide (ODN)-mediated gene repair technology uses an ODN perfectly complementary to the genomic sequence except for a mismatch at the base that is mutated. The endogenous repair machinery of the targeted cell then mediates substitution of the desired base in the gene, resulting in a completely normal sequence. Theoretically, it avoids potential gene silencing or random integration associated with common viral gene augmentation approaches and allows an intact regulation of expression of the therapeutic protein. The eye is a particularly attractive target for gene repair because of its unique features (small organ, easily accessible, low diffusion into systemic circulation). Moreover therapeutic effects on visual impairment could be obtained with modest levels of repair. This chapter describes in details the optimized method to target active ODNs to the nuclei of photoreceptors in neonatal mouse using (1) an electric current application at the eye surface (saline transpalpebral iontophoresis), (2) combined with an intravitreous injection of ODNs, as well as the experimental methods for (3) the dissection of adult neural retinas, (4) their immuno-labelling, and (5) flat-mounting for direct observation of photoreceptor survival, a relevant criteria of treatment outcomes for retinal degeneration.

Pédiatrie [Perinatal asphyxia and neonatal hydronephrosis]

Perinatal asphyxia. Perinatal asphyxia remains one of the most important causes for high mortality and morbidity in the neonatal population. Despite intensive animal and clinical research in this field, no pharmocological strategy has been shown neuroprotective in humans. Moderate hypothermia for severely and moderately asphyctic babies has been aknowledged since a few years as therapeutical approach to improve the outcome of these infants, specifically the long-term follow up (18 months). Neonatal hydronephrosis. Neonatal hydronephrosis is a pathology that requires regular and efficient follow up by a multidisciplinary team. One of the causes of neonatal hydronephrosis is obstructive pathologies which may endanger the kidney. We have developed a strategy that allows a rapid diagnosis of obstructive pathologies with minimal radiological exams. Moreover, this strategy assures the coordination between obstetricians, neonatologists, pediatric urologists, and pediatric nephrologists.

A homozygous missense mutation in SCNN1A is responsible for a transient neonatal form of pseudohypoaldosteronism type 1.

Pseudohypoaldosteronism type 1 (PHA1) is a monogenic disorder of mineralocorticoid resistance characterized by salt wasting, hyperkalemia, high aldosterone levels, and failure to thrive. An autosomal recessive form (AR-PHA1) is caused by mutations in the epithelial sodium channel ENaC with usually severe and persisting multiorgan symptoms. The autosomal dominant form of PHA1 (AD-PHA1) is due to mutations in the mineralocorticoid receptor causing milder and transient symptoms restricted to the kidney. We identified a homozygous missense mutation in the SCNN1A gene (c.727T>C/p.Ser(243)Pro), encoding α-subunit of ENaC (α-ENaC) in a prematurely born boy with a severe salt-losing syndrome. The patient improved rapidly under treatment, and dietary salt supplementation could be stopped after 6 mo. Interestingly, the patient's sibling born at term and harboring the same homozygous Ser(243)Pro mutation showed no symptom of salt-losing nephropathy. In vitro expression of the αSer(243)Pro ENaC mutant revealed a slight but significant decrease in ENaC activity that is exacerbated in the presence of high Na(+) load. Our study provides the first evidence that ENaC activity is critical for the maintenance of salt balance in the immature kidney of preterm babies. Together with previous studies, it shows that, when the kidney is fully mature, the severity of the symptoms of AR-PHA1 is related to the degree of the ENaC loss of function. Finally, this study identifies a novel functional domain in the extracellular loop of ENaC.

The neonatal progeroid syndrome (Wiedemann-Rautenstrauch) and its relationship to Pelizaeus-Merzbacher's disease.

The neuropathology of a clinically well-documented case of the neonatal progeroid syndrome Wiedemann-Rautenstrauch is described. The most striking feature was a nearly complete absence of mature myelin in the brain. When immunohistochemistry for myelin basic protein was applied, some subcortical nerve fibres were accompanied by immature myelin sheaths. The neuropathology corresponds exactly to that of Pelizaeus-Merzbacher disease (Seitelberger type). Furthermore, this morphology, with the presence of myelin basic protein in the absence of mature myelin sheaths is reminiscent of the early stages of myelination in the newborn. From a brief review of the literature on Wiedemann-Rautenstrauch syndrome, we conclude, that the neuropathology of the syndrome is heterogeneous, and that there is relationship between the progeroid aspect and pathological myelination.