Childhood acute pyelonephritis: comparison of power Doppler sonography and Tc-DMSA scintigraphy

Background Tc 99m DMSA scintigraphy is regarded as the gold standard for the detection and localization of acute pyelonephritis (APN) in children. Power Doppler sonography (PD US) is a radiation-free and cost-effective technique that could be useful in the diagnosis of APN in children. Objective To compare the predictive value of PD US with DMSA scintigraphy in the diagnosis of APN in children. Materials and methods A total of 74 neonates and children with clinical findings consistent with possible upper urinary tract infection were evaluated with PD US and DMSA scintigraphy. Children with anatomic (grey-scale) abnormalities were excluded. A total of 147 kidneys were examined within the first 48 h after the onset of symptoms. Each kidney was divided into three zones (upper, middle, and lower third). Results APN was diagnosed by PD US in 46 kidneys. Sensitivity and specificity for detecting APN using DMSA scintigraphy as the reference standard were 73.8% and 85.7%, respectively. There was good agreement between PD US and DMSA scintigraphy in the localization of lesions. Conclusion In clinically suspected APN, PD US has acceptable specificity and sensitivity, if performed within the first 48 h and could be helpful in neonates and children under 3 months of age in whom the use of scintigraphy is generally discouraged.     

Longitudinal Evaluation of Changes in Retinal Architecture Using Optical Coherence Tomography in Achromatopsia

PurposeThis prospective study investigates longitudinal changes in retinal structure in patients with achromatopsia (ACHM) using optical coherence tomography (OCT).MethodsSeventeen patients (five adults, 12 children) with genetically confirmed CNGA3- or CNGB3-associated ACHM underwent ocular examination and OCT over a follow-up period of between 2 and 9.33 years (mean = 5.7 years). Foveal tomograms were qualitatively graded and were segmented for quantitative analysis: central macular thickness (CMt), outer nuclear layer thickness (ONLt), and size of the foveal hyporeflective zone (vertical HRZ thickness: HRZt and horizontal HRZ width: HRZw) were measured. Data were analyzed using linear mixed regression models. Both age and visit were included into the models, to explore the possibility that the rate of disease progression depends on patient age.ResultsFifteen of 17 patients (88%) showed longitudinal changes in retinal structure over the follow-up period. The most common patterns of progression was development of ellipsoid zone (EZ) disruption and HRZ. There was a significant increase in HRZt (P = 0.01) and HRZw (P = 0.001) between visits and no significant change in CMt and ONLt. Retinal parameters showed no difference in changes by genetic mutation (CNGA3 (n = 11), CNGB3 (n = 6)).ConclusionsThis study demonstrates clear longitudinal changes in foveal structure mainly in children, but also in adults with ACHM, over a long follow-up period. The longitudinal foveal changes suggest that treatment at an earlier age should be favored.     

Imaging of filtering blebs after implantation of the Ex-PRESS shunt with the use of the Visante optical coherence tomography

AIM:To analyze the features of the filtering blebs following implantation of the Ex-PRESS glaucoma device (model P-50) with the aid of the Visante anterior segment optical coherence tomography (AS-OCT)METHODS: Five patients with open angle glaucomas were included in the study. They all underwent implantation of the Ex-PRESS device under a scleral flap. The surgical procedure was augmented with the use of mitomycin C subconjunctivally. The filtering blebs were analyzed with the Visante AS-OCT with the scans taken along the axis of the implantation of the glaucoma device.RESULTS: All filtering blebs were graded as diffuse functioning. The morphological characteristics of the blebs were similar to those of the trabeculectomy. However the use of the Ex-PRESS implant tend to form a characteristic episcleral lake at the site of the plate of the implant.CONCLUSION: The use of the Ex-PRESS implant produces filtering blebs similar to those of the trabeculectomy with the formation of a characteristic episcleral lake at the site of the plate of the implant.     

To what extent is bruxism associated with musculoskeletal signs and symptoms? A systematic review

The aim of the present systematic review was to answer the overall research question: “To what extent is bruxism associated with musculoskeletal signs and symptoms?”. The review was performed in accordance with the PRISMA guidelines. A PubMed search of articles published until 23 November 2017 was conducted. The search string included both MeSH terms and text words. Results were presented in categories according to study design, study population (eg, adults, children), bruxism sub‐type (awake, sleep), assessment methods for bruxism and musculoskeletal symptoms (self‐report, validated test) and type of outcome (pain, non‐painful musculoskeletal symptoms). It could be concluded that bruxism is to some extent associated with musculoskeletal symptoms, even though the evidence is conflicting and seems to be dependent on many factors, such as age, whether the bruxism occurs during sleep or wakefulness, and also the quality of the diagnostic methodology regarding bruxism and musculoskeletal signs and symptoms. The literature does not support a direct linear causal relationship between bruxism and such symptoms, but points more in the direction of a multifaceted relationship dependent on the presence of other risk factors. Pain is by far the most commonly assessed symptom, whereas non‐painful musculoskeletal symptoms have generally not been systematically evaluated. In the light of recent findings indicating that non‐painful symptoms may precede TMD pain, it is suggested to increase the scientific focus on non‐painful musculoskeletal symptoms in future studies. Also, future studies should use validated methods for case definition and outcome assessments.     

High Frequency of Thyroid Disorders in Patients Presenting With Neutropenia to an Outpatient Hematology Clinic STROBE-Compliant Article

Granulopoiesis abnormalities have been described in association with thyroid disorders (TD). However, data regarding systematic evaluation of adult neutropenia and concurrent or prior TD are scarce.To investigate the frequency of TD among patients presenting with neutropenia, and the immunophenotypic and immunologic profile of neutropenic patients with concomitant thyroidopathy.Two hundred eighteen consecutive neutropenic patients were prospectively evaluated in our outpatient Hematology Clinic, with a detailed laboratory screen, including thyroid function tests, antineutrophil antibodies, blood lymphocytes immunophenotyping, and detection of T-cell clonality by PCR.Among 218 patients with neutropenia, 95 (43.6%) had TD, 65 chronic immunologic neutropenia, 20 clonal proliferation of T-large granular lymphocytes (T-LGL), 5 autoimmune disorders, and 33 other diagnoses. TD-patients had an increased frequency of recurrent infections compared with other patients (P = 0.045). The following correlations were found: negative correlation between FT3 and absolute neutrophil count (ANC) (r² = −0.274, P = 0.007), negative correlation between TPO-Abs/TG-Abs and C4 (r² = −0.16, P = 0.045; r² = −0.266, P = 0.001), and CD4⁺ counts were inversely correlated to T4 and positively to TSH (r² = −0.274, P = 0.024; r² = 0.16, P = 0.045). In addition, TD-patients had significantly higher percentages of CD4⁺ lymphocytes (P = 0.003). Among TD-patients, 23.4% had Hashimoto thyroiditis (HT), 4.1%, Graves disease (GD), 8.2% nontoxic multinodular goiter (NTMG), 5% subclinical hypothyroidism, and 2.8% had undergone total thyroidectomy associated with nodules (TTM). Thirteen TD-patients displayed T-LGL. Patients with autoimmune thyroidopathy had an increased frequency of concomitant autoimmune manifestations (P = 0.03). Significant differences between the different thyroidopathies included: HT-patients had higher percentages of B-lymphocytes, while the opposite was evident for the TTM-subgroup (P = 0.009, 0.02); GD-patients showed an increase of the proportion of NK cells and a decrease in the percentage of TCRγδ+ lymphocytes (P = 0.001, 0.045); and NTMG-patients had significantly higher ANC (P = 0.004) compared to other thyroidopathies. Antineutrophil antibodies were found in 37.2% of TD-patients tested. Anti-TPO titers were significantly higher in patients with positive antineutrophil antibodies (P = 0.04).The frequency of TD among neutropenic patients may be higher than previously reported. The existence of antineutrophil antibodies, as well as the different distribution of lymphocyte subsets among patients with different TD, suggests both humoral and cellular mechanisms in the pathophysiology of thyroid disease-associated neutropenia.     

Consensus-based clinical guidelines for ambulatory electromyography and contingent electrical stimulation in sleep bruxism

As yet, there are still no evidence-based clinical diagnostic and management guidelines for ambulatory single-channel EMG devices, like the BUTLER^® GrindCare^® (GrindCare), that are used in patients with sleep bruxism. Therefore, a consensus meeting was organised with GrindCare developers, researchers, and academic and non-academic clinicians experienced with the use of ambulatory EMG devices. The aim of the meeting was to discuss and develop recommendations for clinical guidelines for GrindCare usage, based on the existing clinical and research experience of the consensus meeting's participants. As an important outcome of the consensus meeting, clinical guidelines were proposed in which an initial 2-week baseline phase with the device in its inactive (non-stimulus) mode for habituation and assessment of the number of jaw-muscle activities is followed by a 4-week active phase with contingent electrical stimuli suppressing the jaw-muscle activities. As to avoid the commonly reported reduction in sensitivity to the stimuli, a 2-week inactive phase is subsequently installed, followed by a repetition of active and inactive phases until a lasting reduction in the number of jaw-muscle activities and/or associated complaints has been achieved. This proposal has the characteristics of a single-patient clinical trial. From a research point of view, adoption of this approach by large numbers of GrindCare users creates a great opportunity to recruit relatively large numbers of study participants that follow the same protocol.     

The role of SOAT-1 polymorphisms in cognitive decline and delirium after bypass heart surgery

Background Cognitive decline (CD) and delirium (PD) are commonly observed complications after bypass heart surgery. In this study we aimed to investigate whether certain genetic factors (alleles of the SOAT-1 gene) play a role in their appearance. Patients and methods We examined 137 patients receiving coronary bypass surgery with a neuropsychiatric test battery consisting of the Mini Mental State Examination (MMSE), the Brief Psychiatric Rating Scale (BPRS), the Wechsler's Memory Scale-Revised (WMS-R) on admission and one month after surgery, and the Delirium Rating Scale postoperatively, when indicated, and genotyped them in relation to the SOAT–1 genotypes (AA positive group with augmented protection of the nerve cells against stress and the AA negative group – AC and CC subgroups – with diminished protection against stress). Results We noted a significant decline in test results postoperatively and a high frequency of delirium (29.92% of the patients). None of these complications could be associated to the SOAT-1 genotypes. Conclusions Our study confirmed the expected cognitive decline and highly frequent delirium after bypass heart surgery and excluded the possible role of SOAT-1 genotype polymorphisms in their genesis.     

The POM Monoclonals: A Comprehensive Set of Antibodies to Non-Overlapping Prion Protein Epitopes

PrP^Sc, a misfolded and aggregated form of the cellular prion protein PrP^C, is the only defined constituent of the transmissible agent causing prion diseases. Expression of PrP^C in the host organism is necessary for prion replication and for prion neurotoxicity. Understanding prion diseases necessitates detailed structural insights into PrP^C and PrP^Sc. Towards this goal, we have developed a comprehensive collection of monoclonal antibodies denoted POM1 to POM19 and directed against many different epitopes of mouse PrP^C. Three epitopes are located within the N-terminal octarepeat region, one is situated within the central unstructured region, and four epitopes are discontinuous within the globular C-proximal domain of PrP^C. Some of these antibodies recognize epitopes that are resilient to protease digestion in PrP^Sc. Other antibodies immunoprecipitate PrP^C, but not PrP^Sc. A third group was found to immunoprecipitate both PrP isoforms. Some of the latter antibodies could be blocked with epitope-mimicking peptides, and incubation with an excess of these peptides allowed for immunochromatography of PrP^C and PrP^Sc. Amino-proximal antibodies were found to react with repetitive PrP^C epitopes, thereby vastly increasing their avidity. We have also created functional single-chain miniantibodies from selected POMs, which retained the binding characteristics despite their low molecular mass. The POM collection, thus, represents a unique set of reagents allowing for studies with a variety of techniques, including western blotting, ELISA, immunoprecipitation, conformation-dependent immunoassays, and plasmon surface plasmon resonance-based assays.