Non-Progressive Leukoencephalopathy with Bilateral Temporal Cysts

We report two cases of a peculiar leukoencephalopathy with temporal cysts. Both patients have a non-progressive neurological disorder with mental retardation, microcephaly and sensorineural deafness although clinical differences between them may reflect a different aetiology. The metabolic disorders with white matter involvement and the recently described leukoencephalopathies (Van Der Knaap disease, 'vanishing white matter disease') were excluded based on clinical, biologic and imaging findings. Cytomegalovirus infection is a likely possibility in the first case although the magnetic resonance imaging picture is only partially similar to previously reported cases. Our patients are strikingly similar to the patients reported by Deonna et al. and Olivier et al. We discuss the clinical and imaging findings in our patients and the differential diagnosis considering the known disorders of the white matter in childhood.

Vitamimos: Children take control over nutrition

A Work Project, presented as part of the requirements for the Award of a Masters Degree in Management from the NOVA – School of Business and Economics

Late onset sepsis and intestinal bacterial colonization in very low birth weight infants receiving long-term parenteral nutrition

INTRODUCTION: The purpose of this study was to establish the late onset sepsis (LOS) rate of our service, characterize the intestinal microbiota and evaluate a possible association between gut flora and sepsis in surgical infants who were receiving parenteral nutrition (PN). METHODS: Surveillance cultures of the gut were taken at the start of PN and thereafter once a week. Specimens for blood culture were collected based on clinical criteria established by the medical staff. The central venous catheter (CVC) tip was removed under aseptic conditions. Standard laboratory methods were used to identify the microorganisms that grew on cultures of gut, blood and CVC tip. RESULTS: 74 very low birth weight infants were analyzed. All the infants were receiving PN and antibiotics when the gut culture was started. In total, 21 (28.4%) infants experienced 28 episodes of LOS with no identified source. Coagulase negative staphylococci were the most common bacteria identified, both in the intestine (74.2%) and blood (67.8%). All infections occurred in patients who received PN through a central venous catheter. Six infants experienced episodes of microbial translocation. CONCLUSIONS: In this study, LOS was the most frequent episode in neonates receiving parenteral nutrition who had been submitted to surgery; 28.6% of this infection was probably a gut-derived phenomenon and requires novel strategies for prevention.

Advanced megaesophagus (Group III) secondary to vector-borne Chagas disease in a 20-month-old infant

The authors report the case of a female infant with Group III (or Grade III) megaesophagus secondary to vector-borne Chagas disease, resulting in severe malnutrition that reversed after surgery (Heller technique). The infant was then treated with the antiparasitic drug benznidazole, and the infection was cured, as demonstrated serologically and parasitologically. After follow-up of several years without evidence of disease, with satisfactory weight and height development, the patient had her first child at age 23, in whom serological tests for Chagas disease yielded negative results. Thirty years after the initial examination, the patient's electrocardiogram, echocardiogram, and chest radiography remained normal.

Hemophagocytic lymphohistiocytosis associated with H1N1 virus infection and visceral leishmaniasis in a 4.5-month-old infant

We present a case of a 4.5-month-old boy from Turkey with hemophagocytic lymphohistiocytosis (HLH) associated with H1N1 virus and Leishmania spp. coinfection. Because visceral leishmaniasis can mimic hematologic disorders like HLH, it is important to rule out this clinical condition before starting immunosuppressive therapy. In our case, treatment with liposomal amphotericin B resulted in a dramatic resolution of clinical and laboratory abnormalities.

Modulation of electrical stimulation applied to human physiology and clinical diagnostic

The use, manipulation and application of electrical currents, as a controlled interference mechanism in the human body system, is currently a strong source of motivation to researchers in areas such as clinical, sports, neuroscience, amongst others. In electrical stimulation (ES), the current applied to tissue is traditionally controlled concerning stimulation amplitude, frequency and pulse-width. The main drawbacks of the transcutaneous ES are the rapid fatigue induction and the high discomfort induced by the non-selective activation of nervous fibers. There are, however, electrophysiological parameters whose response, like the response to different stimulation waveforms, polarity or a personalized charge control, is still unknown. The study of the following questions is of great importance: What is the physiological effect of the electric pulse parametrization concerning charge, waveform and polarity? Does the effect change with the clinical condition of the subjects? The parametrization influence on muscle recruitment can retard fatigue onset? Can parametrization enable fiber selectivity, optimizing the motor fibers recruitment rather than the nervous fibers, reducing contraction discomfort? Current hardware solutions lack flexibility at the level of stimulation control and physiological response assessment. To answer these questions, a miniaturized, portable and wireless controlled device with ES functions and full integration with a generic biosignals acquisition platform has been created. Hardware was also developed to provide complete freedom for controlling the applied current with respect to the waveform, polarity, frequency, amplitude, pulse-width and duration. The impact of the methodologies developed is successfully applied and evaluated in the contexts of fundamental electrophysiology, psycho-motor rehabilitation and neuromuscular disorders diagnosis. This PhD project was carried out in the Physics Department of Faculty of Sciences and Technology (FCT-UNL), in straight collaboration with PLUX - Wireless Biosignals S.A. company and co-funded by the Foundation for Science and Technology.

Knowledge management framework based on brain models and human physiology

The life of humans and most living beings depend on sensation and perception for the best assessment of the surrounding world. Sensorial organs acquire a variety of stimuli that are interpreted and integrated in our brain for immediate use or stored in memory for later recall. Among the reasoning aspects, a person has to decide what to do with available information. Emotions are classifiers of collected information, assigning a personal meaning to objects, events and individuals, making part of our own identity. Emotions play a decisive role in cognitive processes as reasoning, decision and memory by assigning relevance to collected information. The access to pervasive computing devices, empowered by the ability to sense and perceive the world, provides new forms of acquiring and integrating information. But prior to data assessment on its usefulness, systems must capture and ensure that data is properly managed for diverse possible goals. Portable and wearable devices are now able to gather and store information, from the environment and from our body, using cloud based services and Internet connections. Systems limitations in handling sensorial data, compared with our sensorial capabilities constitute an identified problem. Another problem is the lack of interoperability between humans and devices, as they do not properly understand human’s emotional states and human needs. Addressing those problems is a motivation for the present research work. The mission hereby assumed is to include sensorial and physiological data into a Framework that will be able to manage collected data towards human cognitive functions, supported by a new data model. By learning from selected human functional and behavioural models and reasoning over collected data, the Framework aims at providing evaluation on a person’s emotional state, for empowering human centric applications, along with the capability of storing episodic information on a person’s life with physiologic indicators on emotional states to be used by new generation applications.

Nutritional follow-up of critically ill infants receiving short term parenteral nutrition

Few studies have tried to characterize the efficacy of parenteral support of critically ill infants during short period of intensive care. We studied seventeen infants during five days of total parenteral hyperalimentation. Subsequently, according to the clinical conditions, the patients received nutritional support by parenteral, enteral route or both up to the 10th day. Evaluations were performed on the 1st, 5th, and 10th days. These included: clinical data (food intake and anthropometric measurements), haematological data (lymphocyte count), biochemical tests (albumin, transferrin, fibronectin, prealbumin, retinol-binding protein) and hormone assays (cortisol, insulin, glucagon). Anthropometric measurements revealed no significant difference between the first and second evaluations. Serum albumin and transferrin did not change significantly, but mean values of fibronectin (8.9 to 16 mg/dL), prealbumin (7.7 to 18 mg/dL), and retinol-binding protein (2.4 to 3.7 mg/dL) increased significantly (p < 0.05) from the 1st to the 10th day. The hormonal study showed no difference for insulin, glucagon, and cortisol when the three evaluations were compared. The mean value of the glucose/insulin ratio was of 25.7 in the 1st day and 15.5 in the 5th day, revealing a transitory supression of this hormone. Cortisol showed values above normal in the beginning of the study. We conclude that the anthropometric parameters were not useful due to the short time of the study; serum proteins, fibronectin, prealbumin, and retinol-binding protein were very sensitive indicators of nutritional status, and an elevated glucose/insulin ratio, associated with a slight tendency for increased cortisol levels suggest hypercatabolic state. The critically ill patient can benefit from an early metabolic support.

Urinary tract infection in full-term newborn infants: risk factor analysis

OBJECTIVE: To analyze the correlation of risk factors to the occurrence of urinary tract infection in full-term newborn infants. PATIENTS AND METHODS: Retrospective study (1997) including full-term infants having a positive urine culture by bag specimen. Urine collection was based on: fever, weight loss > 10% of birth weight, nonspecific symptoms (feeding intolerance, failure to thrive, hypoactivity, debilitate suction, irritability), or renal and urinary tract malformations. In these cases, another urine culture by suprapubic bladder aspiration was collected to confirm the diagnosis. To compare and validate the risk factors in each group, the selected cases were divided into two groups: Group I - positive urine culture by bag specimen collection and negative urine culture by suprapubic aspiration, and Group II - positive urine culture by bag specimen collection and positive urine culture by suprapubic aspiration . RESULTS: Sixty one infants were studied, Group I, n = 42 (68.9%) and Group II, n = 19 (31.1%). The selected risk factors (associated infectious diseases, use of broad-spectrum antibiotics, renal and urinary tract malformations, mechanical ventilation, parenteral nutrition and intravascular catheter) were more frequent in Group II (p<0.05). Through relative risk analysis, risk factors were, in decreasing importance: parenteral nutrition, intravascular catheter, associated infectious diseases, use of broad-spectrum antibiotics, mechanical ventilation, and renal and urinary tract malformations. CONCLUSION: The results showed that parenteral nutrition, intravascular catheter, and associated infectious diseases contributed to increase the frequency of neonatal urinary tract infection, and in the presence of more than one risk factor, the occurrence of urinary tract infection rose up to 11 times.

Necrotizing fasciitis in a newborn infant: a case report

We report the case of a one-day-old newborn infant, female, birth weight 1900 g, gestational age 36 weeks presenting with necrotizing fasciitis caused by E. coli and Morganella morganii. The newborn was allowed to fall into the toilet bowl during a domestic delivery. The initial lesion was observed at 24 hours of life on the left leg at the site of the venipuncture for the administration of hypertonic glucose solution. Despite early treatment, a rapid progression occurred resulting in a fatal outcome. We call attention to the risk presented by this serious complication in newborns with a contaminated delivery, and highlight the site of the lesion and causal agents.

Nutrition for the pediatric surgical patient: approach in the peri-operative period

Nutrition is essential for maintenance of physiologic homeostasis and growth. Hypermetabolic states lead to a depletion of body stores, with decreased immunocompetence and increased morbidity and mortality. The purpose of this paper is to provide an update regarding the provision of appropriate nutrition for the pediatric surgical patient, emphasizing the preoperative and postoperative periods. Modern nutritional support for the surgical patient comprises numerous stages, including assessment of nutritional status, nutritional requirements, and nutritional therapy. Nutritional assessment is performed utilizing the clinical history, clinical examination, anthropometry, and biochemical evaluation. Anthropometric parameters include body weight, height, arm and head circumference, and skinfold thickness measurements. The biochemical evaluation is conducted using determinations of plasma levels of proteins, including album, pre-albumin, transferrin, and retinol-binding protein. These parameters are subject to error and are influenced by the rapid changes in body composition in the peri-operative period. Nutritional therapy includes enteral and/or parenteral nutrition. Enteral feeding is the first choice for nutritional therapy. If enteral feeding is not indicated, parenteral nutrition must be utilized. In all cases, an individualized, adequate diet (enteral formula or parenteral solution) is obligatory to decrease the occurrence of overfeeding and its undesirable consequences.

Effects of continuous versus bolus infusion of enteral nutrition in critical patients

PURPOSE: Enteral alimentation is the preferred modality of support in critical patients who have acceptable digestive function and are unable to eat orally, but the advantages of continuous versus intermittent administration are surrounded by controversy. With the purpose of identifying the benefits and complications of each technique, a prospective controlled study with matched subjects was conducted. PATIENTS AND METHODS: Twenty-eight consecutive candidates for enteral feeding were divided into 2 groups (n = 14 each) that were matched for diagnosis and APACHE II score. A commercial immune-stimulating polymeric diet was administered via nasogastric tube by electronic pump in the proportion of 25 kcal/kg/day, either as a 1-hour bolus every 3 hours (Group I), or continuously for 24 hours (Group II), over a 3-day period. Anthropometrics, biochemical measurements, recording of administered drugs and other therapies, thorax X-ray, measurement of abdominal circumference, monitoring of gastric residue, and clinical and nutritional assessments were performed at least once daily. The principal measured outcomes of this protocol were frequency of abdominal distention and pulmonary aspiration, and efficacy in supplying the desired amount of nutrients. RESULTS: Nearly half of the total population (46.4%) exhibited high gastric residues on at least 1 occasion, but only 1 confirmed episode of pulmonary aspiration occurred (3.6%). Both groups displayed a moderate number of complications, without differences. Food input during the first day was greater in Group II (approximately 20% difference), but by the third day, both groups displayed similarly small deficits in total furnished volume of about 10%, when compared with the prescribed diet. CONCLUSIONS: Both administration modalities permitted practical and effective administration of the diet with frequent registered abnormalities but few clinically significant problems. The two groups were similar in this regard, without statistical differences, probably because of meticulous technique, careful monitoring, strict patient matching, and conservative amounts of diet employed in both situations. Further studies with additional populations, diagnostic groups, and dietetic prescriptions should be performed in order to elucidate the differences between these commonly used feeding modalities.

Burkitt-like lymphoma in an infant: a case report

Childhood non-Hodgkin's lymphomas, including Burkitt and Burkitt-like, are rarely diagnosed in infants. A case of B-cell lymphoma in a 13-month-old girl with extensive abdominal disease, ascites, pleural effusion, and tumor lysis syndrome is reported. Phenotypic analysis showed a germinal center B-cell phenotype, and a B-cell clonality was confirmed by polymerase chain reaction. There was no evidence of Epstein-Barr and HIV infection. The case herein reported emphasizes the need for considering the diagnosis of lymphoma even in very young children.

Fertilization and cover crop effects on soil nitrogen and plant nutrition in a young guarana plantation

Fruit tree production is gaining an increasing importance in the central Amazon and elsewhere in the humid tropics, but very little is known about the nutrient dynamics in the soil-plant system. The present study quantified the effects of fertilization and cover cropping with a legume (Pueraria phaseoloides (Roxb.) Benth.) on soil nitrogen (N) dynamics and plant nutrition in a young guarana plantation (Paullinia cupana Kunth. (H.B. and K.) var. sorbilis (Mart.) Ducke) on a highly weathered Xanthic Ferralsol. Large subsoil nitrate (NO3-) accumulation at 0.3-3 m below the guarana plantation indicated N leaching from the topsoil. The NO3- contents to a depth of 2 m were 2.4 times greater between the trees than underneath unfertilized trees (P<0.05). The legume cover crop between the trees increased soil N availability as shown by elevated aerobic N mineralization and lower N immobilization in microbial biomass. The guarana N nutrition and yield did not benefit from the N input by biological fixation of atmospheric N2 by the legume cover (P>0.05). Even without a legume intercrop, large amounts of NO3- were found in the subsoil between unfertilized trees. Subsoil NO3- between the trees could be utilized, however, by fertilized guarana. This can be explained by a more vigorous growth of fertilized trees which had a larger nutrient demand and exploited a larger soil volume. With a legume cover crop, however, more mineral N was available at the topsoil which was leached into the subsoil and consequently accumulated at 0.3-3 m depth. Fertilizer additions of P and K were needed to increase subsoil NO3- use between trees.

Goblet cell density association with tear function and ocular surface physiology

Purpose: To determine the relationship of goblet cell density (GCD) with tear function and ocular surface physiology. Methods: This was a cross-sectional study conducted in 35 asymptomatic subjects with mean age 23.8±3.6 years. Tear film assessment, conjunctiva and cornea examination were done in each subject. Conjunctival impression cytology was performed by applying Nitrocellulose Millipore MFTM-Membrane filter over the superior bulbar conjunctiva. The filter paper was than fixed with 96% ethanol and stained with Periodic Acid Schiff, Hematoxylin and Eosin. GCD was determined by optical microscopy. Relation between GCD and Schirmer score, tear break-up time (TBUT), bulbar redness, limbal redness and corneal staining was determined. Results: The mean GCD was 151±122 cells/mm2. GCD was found higher in eyes with higher Schirmer score but it was not significant (p = 0.75). There was a significant relationship ofGCDwith TBUT (p = 0.042). GCD was not correlated with bulbar redness (p = 0.126), and limbal redness (p = 0.054) as well as corneal staining (p = 0.079). No relationship of GCD with age and gender of the subjects (p > 0.05) was observed. Conclusion: GCD was found correlated with TBUT but no significant correlation was found with the aqueous portion of the tear, limbal as well as bulbar redness and corneal staining.