Abnormal Band Research Articles

RPGR: Deep Phenotyping and Genetic Characterization With Findings Specific to the 3'-end of ORF15.

To describe a group of patients with retinitis pigmentosa GTPase regulator (RPGR)-related retinopathy with a tapetal-like retinal sheen and corresponding changes in the reflectivity of the ellipsoid zone on optical coherence tomography (OCT) imaging. A retrospective case series of 66 patients with a disease-causing variant in RPGR was performed. An expert examiner, masked to patient demographics, clinical evaluations, and specific RPGR variant, analyzed color fundus photographs for the presence of a tapetal-like retinal sheen and assessed OCT images for the presence of an abnormally broad hyper-reflective band in the outer retina. Longitudinal reflectivity profiles were generated and compared with healthy controls. Twelve patients (18.2%) had a retinal sheen on color images that cosegregated with an abnormally broad hyper-reflective ellipsoid zone band on OCT imaging. Three-fourths of these patients were male, had a cone-rod dystrophy, and had pathogenic RPGR variants located toward the 3'-end of ORF15. This group had a different longitudinal reflectivity profile signature compared with controls. After a period of prolonged dark adaptation, the abnormal hyper-reflective band on OCT became less apparent, and the outer retinal layers adopted a more normal appearance. RPGR-related retinopathy should be considered for males presenting with retinal sheen, abnormal ellipsoid zone hyper-reflectivity, and cone or cone-rod dysfunction on ERG, and pursued with molecular testing. Our results have implications for understanding the role of the C-terminal domain encoded by RPGR ORF15 in the phototransduction cascade. Further, the findings may be important to incorporate into both inclusion criteria and outcome measure developments in future RPGR-related cone or cone-rod dystrophy clinical trials.

Behavioral and Emotional Problems among School Children in Tripoli, Libya: What Impact does it have on Community Public Health?

Objective: to detect the prevalence of mental health problems, including behavioral and emotional difficulties among school children (6-15 years) in Tripoli, Libya as observed by their parents and teachers. Methods: The study took place in Tripoli, Libya. Data was collected over three months period between January and March 2022 from parents and teachers. The Arabic version of Strengths and Difficulties Questionnaire (SDQ. Goodman 2002) were used. The sample size was 300 children aged between 6 and 15 years who were attending compulsory education. Four schools were randomly selected and then children were randomly sampled from each class. Parents were asked to hand over the questionnaire to their child’s teacher and collect them later. Data was analyzed to describe normative scores, bandings and cut-offs for normal, borderline and abnormal scores. Only public schools were included in the study. Results: When it came to the perception of teachers and parents, it was found that the response rate for parents and teachers were almost the same only 245 questionnaires were returned (134 male and 111 female) giving a response rate of 81.6%. out of these, 208, both the parents and teacher questionnaires were returned (84.8%),26 filled in only the parents' questionnaires (10.6%), and 11 returned only the teacher’s questionnaire (4.4%). TEACHERS rated their pupils consistently as showing difficulties in all areas with (23.0 %) of the children considered to be displaying abnormal behavior (Total Difficulties Score). The highest proportion of abnormal behavior was for peer relation (17.0%) and conduct problems (18.0%). PARENTS rated their children as having fewer problems than rated by teachers, but rates were still high with (15%) of the children receiving total difficulty scores in the abnormal band. The most problematic areas as assessed by parents were peer relations (29%) followed by emotion problems (22%). Conclusion: Parent SDQs revealed high rates of children with conduct, emotional and peer problems falling above the 90th centile established in the UK sample. Teachers SDQs revealed higher rates of children with conduct problems. Comparison with UK and Egypt data showed abnormal total difficulties score of 11.9% which is a bit higher than UK (10.1%) but lower than that of Egypt (20.6%). Much greater investment in child mental health care is needed. More efforts are needed to identify those children and decide what we can usefully do to help the many-not only the few. Keywords: Tripoli, Libya, behavioral problems, school children, SDQ, mental health

Causes of Abnormal Hemoglobin Electrophoresis

To investigate the possible causes of abnormal hemoglobin electrophoresis results. The hemoglobin electrophoresis results of 5 696 patients in the First Affiliated Hospital of Chengdu Medical College from September 2018 to July 2021 were collected, and the abnormal results and clinical significance were analyzed. The results of 486 patients (accounting for 8.53%) were abnormal, of which 300 cases had increased HbA2, 135 cases had decreased HbA2, 44 cases had increased F alone, and 7 cases had abnormal hemoglobin bands. Among the 486 patients, 246 patients were thalassemia gene positive (the positive rate was 50.62%), including 29 cases of α thalassemia, 208 cases of β thalassemia and 9 cases of αβ thalassemia. Among the patients with elevated HbA2, 68.67% were detected β thalassemia, 3.00% αβ thalassemia, 9.33% were suspected to be caused by macrocytosis, 6.33% by thyroid dysfunction, and 12.67% by uncertainty of the method. Among the patients with reduced HbA2, 21.48% were detected α thalassemia, 60.00% iron deficiency anemia, 8.15% were suspected to be caused by thyroid dysfunction, and 10.37% by uncertainty of the method. Among the patients with elevated F alone, the results of thalassemia gene detection were negative, 40.91% of them were suspected to be caused by macrocytosis, 27.27% by hereditary persistence of fetal hemoglobin, 29.55% by special physiological condition of pregnant women, and 2.27% by hyperthyroidism. Abnormal hemoglobin bands were detected in 7 patients, including 4 cases of hemoglobin D, 2 cases of hemoglobin E, and 1 case of hemoglobin J. Thalassemia, iron deficiency anemia, macrocytosis such as megaloblastic anemia and non-severe aplastic anemia, thyroid dysfunction, hereditary persistence of fetal hemoglobin, abnormal hemoglobin diseases, the uncertainty of the method are all important causes of abnormal hemoglobin electrophoresis results. In clinical work, the patient's indicators should be comprehensively analyzed to determine the possible cause.

A novel compound heterozygous of β-thalassemia with HbG-Coushatta: case report of Iran

A 30-year-old male couple from Ardabil city, Iran, were admitted for premarital screening. An abnormal band in HbS/D regions with high levels of HbF and HbA 2 led us to suspect the possibility of a compound heterozygous state of β-thalassemia in our affected proband. Therefore, beta globin chain sequencing of proband discovered a heterozygote combination of the Hb G-Coushatta [b22 (B4) Glu>Ala, HBB: c.68A>C) with HBB: IVS-II-1 (G>A) mutation as a compound heterozygote.

Ixekizumab-associated transverse myelopathy in a patient with psoriatic arthritis (P14-3.017)

<h3>Objective:</h3> We present the case of a woman with acute-onset myelopathy who presented with sensory symptoms and ataxia. We discuss her diagnosis of monoclonal antibody-induced short-segment transverse myelopathy and the potential mechanisms mediating this adverse reaction. <h3>Background:</h3> Monoclonal antibodies have provided novel therapeutic avenues for several disorders in oncology, rheumatology, and neurology. These treatments, however, have been noted to carry a rapidly growing host of adverse reactions, many of which involve the nervous system. <h3>Design/Methods:</h3> N/A <h3>Results:</h3> A 57-year-old right-handed woman with psoriatic arthritis on monthly ixekizumab injections (a humanized monoclonal antibody directed against interleukin 17A) was admitted for a two-day history of progressive dysesthesias, which ascended from her feet to the lower abdomen. Her neurologic examination showed patellar and ankle hyperreflexia with large-fiber modality length-dependent sensory loss and preserved small-fiber modalities. The workup was notable for normal B12, homocysteine, copper, and zinc levels. No abnormal protein bands were identified on serum protein electrophoresis. A lumbar puncture yielded a bland CSF with two white cells, glucose of 65, protein of 43.5, and 0 oligoclonal bands. Serum ANA was positive with homogeneous with a 1:320 titer, reflecting to positive dsDNA and Scl70. A broad infectious workup was negative for HSV, VZV, CMV West Nile virus, Lyme, syphilis, HIV, and HTLV-2. MRI of the neuraxis was remarkable for a short-segment T2 hyperintense lesion of the dorsal column spanning T7–9. In the context of unrevealing etiological work-up, she was diagnosed with ixekizumab-induced transverse myelopathy and the biologic drug was stopped. She continued to improve during the following year without recurring CNS symptoms. <h3>Conclusions:</h3> We report the first case of ixekizumab-induced thoracic transverse myelopathy which we stipulate may result from dysregulation of the T-helper pathways given the antagonism of IL-17A. As shown by this clinical presentation, neurologists need to be aware of the adverse reactions of monoclonal antibodies. <b>Disclosure:</b> Dr. Rosso has nothing to disclose. Dr. Milano has received personal compensation in the range of $500-$4,999 for serving as an Expert Witness for Jackson kelly. Dr. Gleichgerrcht has nothing to disclose.

Periventricular nodular heterotopia associated with a "transmantle band sign" in patients with epilepsy.

Previous studies using advanced magnetic resonance imaging (MRI) techniques have documented abnormal transmantle bands connecting ectopic nodules to overlying cortex in patients with periventricular nodular heterotopia (PNH). We describe a similar finding using conventional MRI techniques. Patients were identified by means of a full-text search of radiological reports. All scanning was performed using conventional sequences at 3 Tesla (3T). Scans were reviewed by three neuroradiologists, and we characterized imaging features based on type of PNH and cortical irregularities associated with the transmantle band. A total 57 PNH patients were reviewed, of whom 41 demonstrated a "transmantle band" connecting the nodule to the overlying cortex. One or more periventricular heterotopic nodules was present in all 41 patients-this was bilateral in 29 of 41 (71%) and unilateral in the remaining 29%. In many cases there was more than one such band, and in some cases this band was nodular. In 19 of the cases, the cortex to which the band connected was abnormal, showing thinning in 4 cases, thickening in 5 cases, and polymicrogyria in another 10. The transmantle band can be seen frequently in both unilateral and bilateral cases of PNH and can be visualized with conventional 3T MRI sequences. The band highlights the underlying neuronal migration issues at play in the pathogenesis of this disorder, but its underlying role in the complex, patient-specific epileptogenic networks in this cohort has yet to be determined and warrants further investigation.

Analysis of electromagnetic characteristics of typical faults in permanent magnet wind generators

Due to the harsh actual operating environment of the permanent magnet wind turbine, it is easy to break down and difficult to monitor. Therefore, the electromagnetic characteristics identification of major fault types of large-scale permanent magnet wind turbines is studied in this paper. The typical faults of rotor eccentricity, stator winding short circuit and permanent magnet demagnetization of permanent magnet wind turbines are analyzed theoretically. The wavelet analysis algorithm is used to decompose and reconstruct the abnormal electromagnetic signal waveform band, and the characteristic frequency of the electromagnetic signal is obtained when the fault occurs. In order to verify the effectiveness of the proposed method, a 3.680MW permanent magnet wind turbine was taken as the research object. Its physical simulation model was established, and an external circuit was built to carry out field co-simulation. The results show that the motor fault type can be determined by detecting the change rule of fault characteristic frequency in the spectrum diagram, and the electromagnetic characteristic analysis can be applied to the early monitoring of the permanent magnet wind turbine fault.

Charge transfer effect: a new assignment of the abnormal optical absorption band of gold nanoparticles

As a significant accompanying phenomenon of surface-enhanced Raman scattering (SERS), the addition of foreign molecules to colloidal gold or silver nanoparticles results in a new abnormal optical absorption (AOA) band, which usually appears in the long-wavelength region. The assignment of this AOA band has long been debated as an important issue that is desired to be addressed in the SERS field, which is crucial for a clear understanding of the SERS enhancement mechanism and beneficial to surface plasmonics. In this study, both the calculated and measured optical absorptions of gold nanoparticle monomers and dimers as well as their interactions with adsorbed molecules, showed that the AOA band in the long-wavelength region which was assigned to the characteristic longitudinal localized surface plasmon resonance (LSPR) effect of gold nanoparticle chain aggregates in conventional SERS electromagnetic theory, should be attributed to the charge-transfer resonance absorption from gold nanoparticles to adsorbed molecules. This was further confirmed by the corresponding SERS effects. As the excitation wavelength at 785 nm was resonant with the broad AOA band centered at 750 nm, the SERS peaks of the adsorbed pyridine molecules could be dramatically enhanced due to the charge-transfer resonance effect. In contrast, under an excitation wavelength of 532 nm, the SERS peaks appeared very weak, although the excitation wavelength was resonant with the LSPR absorption band of the individual gold nanoparticles.

Engineering charge-transfer interactions for red-emitting SrLa(Sc,Ga)O4:Ce3+ phosphor with improved thermal stability

Blue-light-excitable red-emitting phosphors with high thermal stability are essential for fabricating white light-emitting diodes (WLEDs). Herein, Ce3+-doped SrLaScO4 (SLO:Ce3+) phosphor is discovered to have an abnormal red emission band centered at 640 nm when excited at 440 nm. Spectroscopy and structural analyses confirm that Ce3+ ions occupy the [LaO8] polyhedrons competitively, generating a strong crystal field splitting and a large Stokes shift to produce a red emission. To further restrict the thermal quenching of SLO:Ce3+, charge-transfer engineering is implemented by incorporating a large electronegative Ga3+ in the Sc3+ site, which can attract more charges from nearby coordinating groups to decrease the electronic occupation at the bottom of the conduction band and thereby enlarge the band gap. Sc/Ga substitution in SrLa(Sc,Ga)O4:Ce3+ enhances the thermal stability by increasing the intensity ratio from 15% to 31% at 150°C compared with 20°C. This is attributed to the efficient suppression of the thermally stimulated ionization process. This study presents a general design principle for discovering novel Ce3+-doped red phosphors with good thermal stability for WLED applications.

Malrotation and Midgut Volvulus in Children: Diagnostic Approach, Imaging Findings, and Pitfalls

Malrotation and midgut volvulus are surgical emergencies that commonly occur within the first month of life. The classic symptom is acute bilious vomiting, while nonspecific symptoms such as recurrent abdominal pain may be present in older children. Malrotation can be associated with duodenal obstruction caused by an abnormal peritoneal fibrous band or congenital anomalies, such as an annular pancreas or a preduodenal portal vein. Volvulus can lead to bowel ischemia and a life-threatening condition, thus prompt and accurate diagnosis is crucial. Diagnosis can be made through upper gastrointestinal series, ultrasonography, and CT, with ultrasonography being preferred as a screening tool due to its rapid and accurate diagnosis, without radiation exposure, in children. This pictorial essay discusses the key imaging findings and diagnostic approaches for malrotation and midgut volvulus, as well as diagnostic pitfalls based on actual cases.

One Case of POMES Syndrome with “Limb Pain” As the Main Presentation

Objective: To summarize the clinical manifestations, imaging features and gene mutation sites of a patient with pomes syndrome with “limb pain” as the main manifestation, so as to improve the understanding of this rare disease. Medical History: The patient was a 34 year old male with limb pain and fatigue for more than 20 days. Symptoms and Signs: The tongue is in the middle, the uvula is in the middle, the bilateral pharyngeal reflex disappears, the neck is soft, the muscle strength of both upper limbs is grade 5, the proximal muscle strength of both lower limbs is grade 4, and the distal muscle strength is grade 5. The muscle tension of the extremities is normal, the tendon reflex of the extremities disappears, and no obvious abnormalities are found in the examination of deep and shallow sensation and ataxia. The diagnostic method bone marrow puncture showed thrombocytosis. The pathological results showed that the hematopoietic cells of the three lines were proliferative, the oligoclonal bands in CSF were positive, and the oligoclonal bands in serum were positive. Serum immunofixation electrophoresis (March 30, 2018): IgG λ Abnormal monoclonal bands were found in the lane, and the type of monoclonal immunoglobulin was IgG- λ Type. Blood analysis suggested thrombocytosis (738 × 10°several L). There is no specific therapeutic drug, but hormone and hydroxyurea are effective. The clinical outcome was improved and the disease was gradually alleviated. The clinical conclusion is that poems syndrome is a rare clinical syndrome with various forms. Early intervention is effective for the prognosis of patients.

Acidic layer-enhanced nanoconfinement of anions in cylindrical pore of single-walled carbon nanotube

The adsorption of the nitrate ion by the cylindrical pore of single-walled carbon nanotubes (SWCNT) was found to be aided by an acidic adsorbed layer. Adsorbed water in the vicinity of the pore wall can supply protons through ionization, forming the acidic layer, according to Raman spectra and results of solution pH fluctuations caused by ion species adsorption. Such an acidic adsorbed layer leads to surplus adsorption of anionic species where the adsorbed amount of nitrate ions is much larger than that of cations. Also, we could observe the Raman bands being assignable to the symmetrical stretching mode at an extremely high-frequency region for nano-restricted nitrate ions compared to any other bulk phases. The abnormal band shift of adsorbed nitrate ions indicates that the nitrate ions are confined in the pore under the effects of nanoconfinement by the pore and the strong interaction with the acidic layer in the pore. Our results warn that we have to construct the adsorption model of aqueous electrolytes confined in carbon pores by deliberating the acid layer formed by the adsorbed water.

Genetic relationship of microstructure and mechanical properties between ferrite/pearlite steel plate and billet

This paper proposed the double-pass compression thermal/force simulation experiment to in situ analyze the phase transformation of continuous casting billet during the controlled rolling and controlled cooling process. The genetic relationship between the central segregation of the billet and solute element distribution of the steel plate, and also the genetic relationship of microstructure and mechanical properties between them were experimentally studied. The results show that the microstructure and mechanical properties between the billet and corresponding batch of steel plate have the genetic relationship, and the link of the relationship is central segregation. The phase transformation of supercooling austenite will be affected by the central segregation of Mn and C elements, and the granular bainite abnormal segregation band is the transformation product of Mn and C element segregation region.

Dysfunction of Emotion Regulation in Mild Cognitive Impairment Individuals Combined With Depressive Disorder: A Neural Mechanism Study.

Depression increases the risk of progression from mild cognitive impairment (MCI) to dementia, where impaired emotion regulation is a core symptom of depression. However, the neural mechanisms underlying the decreased emotion regulation in individuals with MCI combined with depressive symptoms are not precise. We assessed the behavioral performance by emotion regulation tasks and recorded event-related electroencephalography (EEG) signals related to emotion regulation tasks simultaneously. EEG analysis, including event-related potential (ERP), event-related spectral perturbation (ERSP), functional connectivity and graph theory, was used to compare the difference between MCI individuals and MCI depressed individuals in behavioral performance, the late positive potential (LPP) amplitudes, neural oscillations and brain networks during the processing of emotional stimuli. We found that MCI depressed individuals have negative preferences and are prone to allocate more attentional resources to negative stimuli. Results suggested that theta and alpha oscillations activity is increased, and gamma oscillations activity is decreased during negative stimulus processing in MCI depressed individuals, thus indicating that the decreased emotion regulation in MCI depressed individuals may be associated with enhanced low-frequency and decreased high-frequency oscillations activity. Functional connectivity analysis revealed a decrease in functional connectivity in the left cerebral hemisphere of the alpha band and an increase in functional connectivity in the right cerebral hemisphere of the alpha band in MCI depressed individuals. Graph theory analysis suggested that global network metrics, including clustering coefficients and disassortative, decreased, while nodal and modular network metrics regarding local nodal efficiency, degree centrality, and betweenness centrality were significantly increased in the frontal lobe and decreased in the parieto-occipital lobe, which was observed in the alpha band, further suggesting that abnormal alpha band network connectivity may be a potential marker of depressive symptoms. Correlational analyses showed that depressive symptoms were closely related to emotion regulation, power oscillations and functional connectivity. In conclusion, the dominant processing of negative stimuli, the increased low-frequency oscillations activity and decreased high-frequency activity, so as the decrease in top-down information processing in the frontal parieto-occipital lobe, results in the abnormality of alpha-band network connectivity. It is suggested that these factors, in turn, contribute to the declined ability of MCI depressed individuals in emotion regulation.

Excitatory deep brain stimulation quenches beta oscillations arising in a computational model of the subthalamo-pallidal loop

Parkinson’s disease (PD) is associated with abnormal beta band oscillations (13–30 Hz) in the cortico-basal ganglia circuits. Abnormally increased striato-pallidal inhibition and strengthening the synaptic coupling between subthalamic nucleus (STN) and globus pallidus externa (GPe), due to the loss of dopamine, are considered as the potential sources of beta oscillations in the basal ganglia. Deep brain stimulation (DBS) of the basal ganglia subregions is known as a way to reduce the pathological beta oscillations and motor deficits related to PD. Despite the success of the DBS, its underlying mechanism is poorly understood and, there is controversy about the inhibitory or excitatory role of the DBS in the literature. Here, we utilized a computational network model of basal ganglia which consists of STN, GPe, globus pallidus interna, and thalamic neuronal population. This model can reproduce healthy and pathological beta oscillations similar to what has been observed in experimental studies. Using this model, we investigated the effect of DBS to understand whether its effect is excitatory or inhibitory. Our results show that the excitatory DBS is able to quench the pathological synchrony and beta oscillations, while, applying inhibitory DBS failed to quench the PD signs. In light of simulation results, we conclude that the effect of the DBS on its target is excitatory.

Abnormal Evolution of a Layered Structure and Band Gap in AgInP2S6 under Compression

van der Waals layered materials are extremely sensitive to the effect of pressure, which can lead to many novel properties. Hence, we apply ultraviolet (UV) optical absorbance, Raman spectroscopy, X-ray diffraction, and first-principles calculations to study the influence of pressure-induced interlayered structure, bandgap evolution of AgInP2S6. The results show the abnormal band gap evolution in compression, which is related to the bond stretching of sulfur in AgInP2S6. For the AgInP2S6 structure, the XRD and calculations reveal that there is no structure transition in compression until 50 GPa. Furthermore, we also find the layer angle has a peculiar rotation coincidence with the abnormal band gap evolution from 0 to 30 GPa because of the S–P–S bond. These observations not only provide the understanding of the mechanism by which chemical bond angles induced the changes in the interlayer structure but also expand the promising exploration of more [P2S6]4–-based functional ceramics photoelectric applications.

Bilaterally Reduced Rolandic Beta Band Activity in Minor Stroke Patients.

Stroke patients with hemiparesis display decreased beta band (13–25 Hz) rolandic activity, correlating to impaired motor function. However, clinically, patients without significant weakness, with small lesions far from sensorimotor cortex, exhibit bilateral decreased motor dexterity and slowed reaction times. We investigate whether these minor stroke patients also display abnormal beta band activity. Magnetoencephalographic (MEG) data were collected from nine minor stroke patients (NIHSS < 4) without significant hemiparesis, at ~1 and ~6 months postinfarct, and eight age-similar controls. Rolandic relative beta power during matching tasks and resting state, and Beta Event Related (De)Synchronization (ERD/ERS) during button press responses were analyzed. Regardless of lesion location, patients had significantly reduced relative beta power and ERS compared to controls. Abnormalities persisted over visits, and were present in both ipsi- and contra-lesional hemispheres, consistent with bilateral impairments in motor dexterity and speed. Minor stroke patients without severe weakness display reduced rolandic beta band activity in both hemispheres, which may be linked to bilaterally impaired dexterity and processing speed, implicating global connectivity dysfunction affecting sensorimotor cortex independent of lesion location. Findings not only illustrate global network disruption after minor stroke, but suggest rolandic beta band activity may be a potential biomarker and treatment target, even for minor stroke patients with small lesions far from sensorimotor areas.

Genetic Analysis of Thalassemia in 22 940 Pregnant Women in Xiangxi Tujia and Miao Autonomous Prefecture

To investigate the incidence and types of thalassemia in Xiangxi Tujia and Miao Autonomous Prefecture. Automatic capillary electrophoresis was used to screen the thalassemia phenotypes of 22 940 blood samples of pregnant women and puerperants collected in our hospital and some other medical institutions in the prefecture during 2017-2019, among which there were 3 356 cases of Tujia ethnicity, 2 821 cases of Miao ethnicity, and 2 233 cases of Han ethnicity included, whose ethnicity were indicated. The samples with positive result would undergo further genetic testing. There were 2 314 cases of suspicious thalassemia were screened from 22 940 cases by the electrophoresis, thus the positive rate was 10.1% (hematological phenotypes from some other institutions were not included). Specifically, there were 1 706 cases with HBA2 less than 2.5%, 255 cases with HBA2 ranged from 2.5% to 3.5%, which displayed abnormal hematology (MCV or/and MCH) or other abnormal bands, and 353 cases with HBA2>3.5%. There were 436 suspected positive patients in 2 314 suspicious samples received further thalassemia gene testing in our hospital, among them 48 cases were diagnosed with α-thalassemia, 85 cases with β-thalassemia, and 2 cases as compound type. The positive diagnosis rate of α-thalassemia gene test was 11.0%, β-thalassemia was 19.4%, and positive pregnant women was 31.0%. The positive rate of thalassemia screening in Xiangxi Autonomous Prefecture is roughly the same as that in other regions of Hunan. The positive predictive value of β-thalassemia screening is as high as 86%. Compared with the missed screening data, it is recommended to use hematology (MCV, MCH) method combined with capillary hemoglobin electrophoresis for thalassemia screening.

Beta band modulation by dopamine D2 receptors in the primary motor cortex and pedunculopontine nucleus in a rat model of Parkinson’s disease

Beta band (12–30 Hz) hypersynchrony within the basal ganglia-thalamocortical network has been suggested as a hallmark of Parkinson’s disease (PD) pathophysiology. Abnormal beta band oscillations are found in the pedunculopontine nucleus (PPN) and primary motor cortex (M1) and are correlated with dopamine depletion. Dopamine acts locomotion and motor performance mainly through dopamine receptors (D1 and D2). However, the precise mechanism by which dopamine receptors regulate beta band electrophysiological activities between the PPN and M1 is still unknown. Here, we recorded the neuronal activity of the PPN and M1 simultaneously by the administration of the drug (SCH23390 and raclopride), selectively blocking the dopamine D1 receptor and D2 receptor. We discovered that the increased coherent activity of the beta band (12–30 Hz) between M1 and PPN in the lesioned group could be reduced and restored by injecting raclopride in the resting and wheel running states. Our studies revealed the unique role of D2 dopamine receptor signaling in regulating β band oscillatory activity in M1 and PPN and their relationship after the loss of dopamine, which contributes to elucidating the underlying mechanism of the pathophysiology of PD.

The Prognostic Impact of Abnormal Protein Bands in Multiple Myeloma: A Systematic Review and Meta-Analysis

Introduction: The detection of abnormal protein bands (APB) different from the original monoclonal protein in patients with multiple myeloma (MM) who underwent stem cell transplantation and/or chemotherapy has been reported. These atypical serum immunofixation patterns may be monoclonal (also termed secondary monoclonal gammopathy of undetermined significance) or oligoclonal bands (OB). The prognostic significance of this phenomenon remains controversial. This systematic review and meta-analysis aimed to summarize and analyze the evidence for the association between APB with survival in MM patients.Methods: A systematic search of PubMed, Cochrane, Google Scholar, Medline-Ovid, CINAHL, and ERIC, was conducted using relevant search terms. All studies were screened using predefined selection criteria and critically appraised for quality assessment following the Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) guideline. All studies that described the presence of APB, defined as monoclonal spike with heavy or light chain immunoglobulin distinct from the original paraprotein at initial diagnosis of MM, and its associations with survival were included. Studies that reported multivariate adjusted hazard ratios (HR) were further included for meta-analysis. Random-effects model was used to synthesize the pooled HR estimate for the association of APB with survival. Cochran's Q statistics and I2 tests were used to evaluate statistical heterogeneity.Results: A total of 24 out of 181 eligible studies were included for qualitative synthesis. Four studies (including 1115 patients) that reported HR estimates were included in the final meta-analysis. The random-effect summary HR for the progression-free survival among patients with APB was 0.44 (95% CI, 0.31-0.65) with no statistical heterogeneity (I2=0%, p=0.93). The pooled HR for the association with overall survival was 0.31 (95% CI, 0.14-0.66) with moderate statistical heterogeneity (I2=45%; p=0.16) for patients with APB. One study (Silva et al, 2017) only included patients with at least very good partial response while the other three studies reported similar findings of higher occurrence of APB with complete response (Tovar et al, 2013; Jo et al, 2014; Zou et al, 2014). These results are also consistent with the presumed association of APB with complete response as suggested in other studies included in qualitative review.Conclusions: This study indicated a potential prognostic value of APB for favorable outcomes in the context of both overall and progression-free survival in MM patients after treatment. Further research is needed to evaluate the prognostic impact of the sole emergence of APB irrespective of treatment response. [Display omitted] DisclosuresJamshed: Takeda: Honoraria.