Scedosporium apiospermum and its teleomorph (sexual form) Pseudallescheria boydii are ubiquitous saprophytic fungi, which under specific conditions, such as near-drowning, may cause therapy-refractory and life-threatening infections. We reviewed 22 cases (eight children and 14 adults) of S. apiospermum infection after near-drowning reported in the literature including an additional paediatric case from our institution. Scedosporiosis after near-drowning was associated with high mortality (16/23, 70%) even in immunocompetent hosts. It affected mainly young (mean age 24 years) and immunocompetent (83% with no apparent immune defect) males (male to female ratio 2.5 : 1). Scedosporiosis after near-drowning was a slow progressive disease (mean survival time 87 days) involving virtually all body organs. However, central nervous system (CNS) dissemination predominated (21/23, 91%) presenting mainly as multiple brain abscesses (15/23, 65%). All 23 patients showed preceding clinical and/or radiological evidence of lung disease indicating the mode of invasion. Diagnosis was delayed (median time to diagnosis 28 days) and was made by culture (16/23, 69.5%) or culture and tissue examination (7/23, 30.5%). The majority of the patients (20/23, 87%) received antifungal treatment and underwent neurosurgery. While the optimal treatment remains undefined, the most recent reports indicated voriconazole as a potentially effective option. Better knowledge of scedosporiosis after near-drowning could lead to improved intervention and ultimately to more favourable outcome.
Aim: The complex pathobiology of traumatic brain injury (TBI) offers numerous targets for potential neuroprotective agents. We evaluate the clinical benefit after creatine (Cr) administration in children and adolescents Methods: A prospective, randomized, comparative, open-labelled pilot study of the possible neuroprotective effect of Cr was carried out on 39 children and adolescents, aged between 1 and 18 years of age, with TBI. The Cr was administered for 6 months, at a dose of 0.4 g/kg in an oral suspension form every day. For categorical variables, we used the Chi-square test to identify differences between controls and cases. Statistical significance was defined as a p-value < 0.05 and not statistically significant if p-value > 0.1. Results: The administration of Cr to children and adolescents with TBI improved results in several parameters, including duration of post traumatic amnesia (PTA), duration of intubation, intensive care unit stay. Significant improvement was recorded in the categories of headache (p < 0.001), dizziness (p = 0.005) and fatigue (p < 0.001), aspects in all patients. No side effects were seen due to Cr administration. Conclusion: More specific examinations including brain spectroscopy for in vivo evaluation of Cr can be done, in order to draw conclusions for the optimal duration and manner of Cr supply, as well as its possible role for the prevention of TBI complications, in double blind studies.
Ethylmalonic encephalopathy (EE) is a rare, recently defined inborn error of metabolism which affects the brain, gastrointestinal system and peripheral blood vessels and is characterized by a unique constellation of clinical and biochemical features. A 7-month-old male, who presented with psychomotor retardation, chronic diarrhea and relapsing petechiae is described with the objective of highlighting the biochemical and neuroradiological features of this disorder as well as the effect of high-dose riboflavin therapy. Urinary organic acid analysis revealed markedly increased excretion of ethylmalonic acid, isobutyrylglycine, 2-methylbutyrylglycine and isovalerylglycine. Acylcarnitine analysis in dried blood spots showed increased butyrylcarnitine. Short-chain acyl-CoA dehydrogenase (SCAD) activity in muscle was normal as were mitochondrial OXPHOS enzyme activities in cultured skin fibroblasts. In skeletal muscle the catalytic activity of complex II was decreased. Brain MRI revealed bilateral and symmetrical atrophy in the fronto-temporal areas, massive enlargement of the subarachnoid spaces and hyperdensities on T (2) sequences of the basal ganglia. Mutation analysis of the ETHE1 gene demonstrated homozygosity for the Arg163Gly mutation, confirming the diagnosis of EE at a molecular level. On repeat MRI, a significant deterioration was seen, correlating well with the clinical deterioration of the patient.
Acquisition of IRAB by PICU patients was independently associated with aminoglycoside use and prolonged stay in the unit. Studies of evaluation of infection control policies need to be pursued.
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