Summary
Close liaison with ICU‐trained dietitians and early initiation of nutrition is a fundamental principle of care of critically ill patients– this should be done while monitoring closely for ...refeeding syndrome. Enteral nutrition delivered by volumetric pumps should be used where possible, though parenteral nutrition should be started early in patients with high nutritional risk factors. Malnutrition and loss of muscle mass are common in patients who are admitted to ICUs and are prognostic for patient‐centred outcomes including complications and mortality. Obesity is part of that story, and isocaloric and high‐protein provision of nutrition is important in this group of patients who comprise a growing proportion of people treated. Assessing protein stores and appropriate dosing is, however, challenging in all groups of patients. It would be beneficial to develop strategies to reduce muscle wasting as well; various strategies including amino acid supplementation, ketogenic nutrition and exercise have been trialled, but the quality of data has been inadequate to address this phenomenon. Nutritional targets are rarely achieved in practice, and all ICUs should incorporate clear guidelines to help address this. These should include local nutritional and fasting guidelines and for the management of feed intolerance, early access to post‐pyloric feeding and a multidisciplinary framework to support the importance of nutritional education.
In-situ EXAFS combined with a Paris-Edinburgh press (PEP) is an outstanding tool to investigate the local environment of trace elements in melts at high pressure and temperature. A novel design of ...the pressure assembly ensures a highly stable experimental setup (reaching temperatures of up to 2000 K at 2.5 GPa) while permitting the necessary level of X- ray transmission. This study focuses on the structural incorporation of the geochemically important trace elements Y and Sr in sodium-rich silicate-carbonate melts. Y and Sr K edge EXAFS were collected in transmission mode of the melt (at ∼2.5 GPa, 1600 K) and its respective quench products. Distinct changes in the XANES region suggest a change in site symmetry during the cooling process.
Presented is a pedigree with infancy-onset benign hereditary chorea (BHC) caused by a novel nonsense mutation in exon 3 (523G-->T, E175X) of the TITF-1 (Nkx2.1) gene. Four confirmed mutation carriers ...showed the typical movement disorder of BHC and congenital hypothyroidism. Surprisingly, treatment with levodopa improved gait dramatically and reduced chorea in two patients. Dopaminergic drugs should be considered a useful therapeutic option in BHC.
Pediatric Graves’ orbitopathy is rare, thus its clinical picture is less well defined in comparison with the adult population. Therefore it is our aim to describe the clinical spectrum at our ...tertiary referral center. We identified 11 patients under the age of 18 years (3♂, 8♀; range 3–16 years, mean age 14.5 years) with Graves’ disease and signs of orbitopathy. Seven of them where reevaluated (mean follow-up 25 months, range 3–66 months). Eyelid retraction and proptosis were the predominant signs in 10/11 of our patients. In six patients, Hertel readings ranged from 22 mm or above. Mild ocular motility impairment was seen in four children. Active orbitopathy or severe impairment of visual acuity/ocular motility, corneal or optic nerve involvement was not observed in our study. Our series confirms that pediatric Graves’ orbitopathy lacks significant inflammatory features; however, proptosis is common and may be marked. All seven patients who were reevaluated did not show any clinically significant change of ocular signs during the observation period. In particular, there was no improvement of proptosis despite restoration of euthyroidism.
Congenital hypothyroidism occurs with a prevalence of approximately 1:3 500. Defects in thyroid hormone synthesis which lead to goitrous hypothyroidism account for 10-15% of these cases. Several ...genetic defects have been characterized and mutations in the thyroid peroxidase (TPO) gene are the most common cause for dyshormonogenesis.So far, more than 80 mutations in the TPO gene have been described, resulting in a variable decrease in TPO bioactivity. Clinically TPO defects manifest with congenital primary goitrous hypothyroidism.We here present 2 children with congenital primary hypothyroidism, who were identified to have compound heterozygous TPO mutations. They both shared the same novel mutation in the TPO gene (C756R) in exon 13. One case presented with an apparently dominant inheritance of thyroid dyshormonogenesis.
Background Each year many children suffer from respiratory infections caused by Influenza A virus, but only a limited number experiences severe complications. One of these serious complications is an ...acute necrotizing encephalopathy (ANEC). Methods We report two cases of patients with ANEC following upper airway infection. Both children were referred to our paediatric intensive care unit (PICU) due to rapid neurological deterioration and respiratory failure. None had received vaccination against influenza. A 16-year old boy presented with difficulties in speech and motor deficits. A 14-months old girl had multiple focal seizures. Her Glasgow-Coma-Scale was 4 on admittance to PICU. Both patients received a MRI (Magnetic Resonance Imaging) of the brain. The MR imaging findings demonstrated abnormal signal intensity bilaterally in multiple cortical and subcortical regions. In both cases Influenza A was detected on a nasopharyngeal swab by using polymerase chain reaction assay. Results Both patients were treated with oseltamivir without notable clinical improvement. The boy showed complete reconvalescence. The girl suffered from increased intracranial pressure within hours after hospital admittance needing urgent external cerebrospinal fluid drainage. Her condition was complicated by an acute respiratory distress syndrome requiring mechanical ventilation. Meanwhile her consciousness has dramatically improved, but we still expect neurological sequelae. Conclusion To our knowledge severe complications following an infection with influenza are rare but have to be kept in mind when treating a child with respiratory infection and neurological impairment. The insufficient response to oseltamivir underlines the need to re-evaluate the benefit of vaccination against influenza in children.
We review recent progress in studying structural properties of liquids using X-ray absorption spectroscopy coupled with the Paris-Edinburgh press at third-generation synchrotron facilities. This ...experimental method allows for detecting subtle changes in atomic arrangements of melts over a wide pressure-temperature range. It has been also employed to monitor variations of the local coordination environment of diluted species contained in glasses, liquids and crystalline phases as a function of the pressure and temperature. Such information is of great importance for gaining deeper insights into the physico-chemical properties of liquids at extreme condition, including the understanding of such phenomena as liquid-liquid phase transitions, viscosity drops and various transport properties of geological melts. Here, we describe the experimental approach and discuss its potential in structural characterization on selected scientific highlights. Finally, the current ongoing instrumental developments and future scientific opportunities are discussed.
Celotno besedilo
Dostopno za:
BFBNIB, DOBA, GIS, IJS, IZUM, KILJ, KISLJ, NUK, PILJ, PNG, SAZU, UILJ, UKNU, UL, UM, UPUK
Aim: To analyse the urinary steroid metabolome in a boy who had true precocious puberty after a Leydig cell tumour.
Method: Case report and detailed description of clinical and metabolic findings ...in a 7‐year‐old‐boy with a Leydig cell tumour.
Results: Before surgery, the urinary steroid metabolome showed an activation of an alternative route to gonadal androgens independent of dehydroepiandrosterone (DHEA). After surgery, the boy entered true precocious puberty. Under leuprolide acetate treatment, clinical and laboratory findings normalized.
Conclusion: Central precocious puberty after precocious pseudopuberty may be more common than expected and should be considered in children with persistent or recurrent symptoms after initial treatment of precocious pseudopuberty. Patients with a Leydig cell tumour seem to reactivate the so‐called ‘back door pathway’ of androgen production, which is independent of the classical route via DHEA.
Kidney lesions similar to those in humans with hemolytic-uremic syndrome were observed histologically in 82 of 122 piglets inoculated intragastrically with Shiga-toxigenic Escherichia coli but not in ...29 controls. The locations of lesions matched locations where Stx-2 binding and Gb3 (globotriasylceramide receptors for Stx) were identified immunohistochemically.
The role of colostral immunoglobulins for the protection of newborn calves has been studied extensively, but little is known about the importance of colostral leukocytes. To study the uptake of ...colostral leukocytes in the intestine of calves and to determine preferential sites for this uptake, FITC-labelled colostral cells derived from the respective dams were injected into intestinal loops with/without Peyer’s patches of three male Holstein Frisian calves about 5
h post natum. In adjacent loops, PBS was injected as control. Loops were excised after an exposure of 1.5–2
h. FITC-labelled material and cells were detected by the direct immunoperoxidase method in paraplast sections. Twenty-five consecutive sections were evaluated from each localization. Uptake of labelled material and cells was observed in all three calves in the jejunal Peyer’s patch and in two calves in the ileal Peyer’s patch as well. In the jejunal Peyer’s patch, labelled material and cells were present in epithelium, domes and sinuses around lymphoid follicles, whereas in the ileal Peyer’s patch, they were found in the sinuses only. These findings confirm that uptake of colostral leukocytes through the intestinal barrier is possible and that the preferential route of uptake is through follicle-associated epithelium of Peyer’s patches.
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