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The blanket notion that preadolescent age-group should be excluded from awake mapping needs to be challenged, rather curated on a case basis.
Our 2 cases illustrate the first instance of successful use of awake IONM for maximal safe resection of ERG in preadolescent age-group. We believe, with proper preoperative planning and careful titration of anesthetics, it is safe and feasible. The blanket notion that preadolescent age-group should be excluded from awake mapping needs to be challenged, rather curated on a case basis.
Patients with chemosensory dysfunction frequently report symptoms of depression. BMS-1 inhibitor The current study aims to clarify whether the type (smell dysfunction, taste dysfunction, and mixed smell and taste dysfunction), severity, duration, or cause of dysfunction have differential impacts on the symptoms of depression.
899 patients with chemosensory disorders and 62 controls were included. Following a structured interview and an otorhinolaryngological examination, subjects underwent olfactory tests (Sniffin' Sticks), gustatory tests (taste sprays) and an assessment of depressive symptoms (Beck Depression Inventory). Information on the cause and duration of disorders was also collected.
Patients with combined olfactory/gustatory dysfunction had higher depression scores than patients with smell dysfunction only and controls, and no significant difference was found between the smell dysfunction and controls. Anosmia patients, but not hyposmia patients, exhibited higher depression scores than controls. Among variousin clinical practice.
Semantic dementia (SD) is characterized by fluent speech, anomia, and loss of word and object knowledge with varying degrees of right and left anterior-medial temporal lobe hypometabolism on [18F] fluorodeoxyglucose (FDG)-PET. We assessed neurobehavioral features in SD patients across 3 FDG-PET-defined metabolic patterns and investigated progression over time.
Thirty-four patients with SD who completed FDG-PET were classified into a left- and right-dominant group based on the degree of hypometabolism in each temporal lobe. The left-dominant group was further subdivided depending on whether hypometabolism in the right temporal lobe was more or less than 2 standard deviations from controls (left+ group). Neurobehavioral characteristics determined using the Neuropsychiatric Inventory Questionnaire (NPI-Q) were compared across groups. Progression of NPI-Q scores and FDG-PET hypometabolism was assessed in 14 patients with longitudinal follow-up.
The right-dominant group performed worse on the NPI-Q and had aal temporal lobe hypometabolism and commonly develop over time even in patients that present with left-dominant patterns of hypometabolism.
Natural history (NH) studies, using observational methods, are common in rare and orphan diseases (80% of which have a genetic component). There is profound interest in identifying genetic mutations driving these diseases in these studies to support the formulation of targeted precision medicines. The global regulatory classification of NH studies with novel molecular biomarker collection has not been clearly delineated, presenting researchers with the challenge of determining how these studies are classified and regulated across multiple geographies.
The aim of this investigation was to conduct a review of regulations related to NH studies and genetic testing to elucidate regulatory pathways to inform clinical researchers in the field.
Regulatory provisions for NH studies and genetic testing were obtained from Pharmaceutical Product Development (PPD)'s propriety regulatory intelligence database and by surveying the company's country-specific regulatory experts. A literature search was conducted in the in orphan and rare diseases. A set of harmonized international guidelines for these studies would improve efficiency, and this may be on the horizon with the recent adaption of International Conference on Harmonisation (ICH) guideline E18.To accurately determine the space use of animals, we need to follow animal movements over prolonged periods, which is especially challenging for the critically endangered Grauer's gorillas (Gorilla beringei graueri) in eastern Democratic Republic of the Congo (DRC). As a consequence, we know little about Grauer's gorillas, particularly from the lower elevational parts of their range. Between 2016 and 2018, we tracked unhabituated Grauer's gorillas in lowland forests (500-1,000 m a.s.l.), at the community-managed Nkuba Conservation Area in Nord Kivu (DRC) to provide estimates of daily travel distances (DTD), daily displacement distances (DDD), and the linearity of recorded paths expressed as the Linearity Index (LI) DDD/DTD. We found an average DTD of ∼1.3 km (range 0.05-5.0 km), with temporal variation among monthly averages; specifically, an increase in travel distance over the June-August dry season resulting in peak travel distances at the beginning of the September-December wet season. Daily displacements showed similar temporal variation, which resulted in a lack of obvious temporal patterns in LI. We conclude that the movement patterns of Grauer's gorillas in lowland forests, which are characterized by larger DTD than those of Grauer's gorillas that inhabit highland habitats, show similarity to travel distances of other predominantly frugivorous gorillas. Moreover, the observed temporal patterns in space use may be tentatively linked to temporal changes in fruit availability or consumption. These observations have consequences for our understanding of the ecological role that Grauer's gorillas play and provide baseline data to estimate current and future distributions, abundances, and carrying capacities of this highly threatened animal.
The objective of our study was to assess the utility of sequential fetal urine analysis in severe lower urinary tract obstruction (LUTO) when selecting cases suitable for vesicoamniotic shunting.
This was a retrospective cohort study of cases of severe LUTO treated in our fetal medicine center from 1994 to 2013. Two fetal bladder samples were taken 24-48 h apart to assess renal function. A vesicoamniotic shunt was inserted in case of improvement in urinary biochemistry between the 2 samples. We assessed perinatal morbidity and mortality and renal function at 5 years.
Among a total of 26 LUTO cases with sequential urine analysis, 5 showed normal urinary biochemistry, 13 were abnormal, and 8 improved between the 2 samples. These 8 cases underwent vesicoamniotic shunt placement, leading to the birth of 6/8 (75%) live infants, 5/6 (83%) of whom had normal renal function at 5 years. The 5 cases with normal biochemistry occasioned 2 neonatal deaths and 3 children with normal renal function at 5 years. Elective termination of pregnancy was requested by parents for the fetuses exhibiting abnormal biochemistry.
