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1.
J Neurosci ; 44(28)2024 Jul 10.
Artículo en Inglés | MEDLINE | ID: mdl-38830759

RESUMEN

Congenital single-sided deafness (SSD) leads to an aural preference syndrome that is characterized by overrepresentation of the hearing ear in the auditory system. Cochlear implantation (CI) of the deaf ear is an effective treatment for SSD. However, the newly introduced auditory input in congenital SSD often does not reach expectations in late-implanted CI recipients with respect to binaural hearing and speech perception. In a previous study, a reduction of the interaural time difference (ITD) sensitivity has been shown in unilaterally congenitally deaf cats (uCDCs). In the present study, we focused on the interaural level difference (ILD) processing in the primary auditory cortex. The uCDC group was compared with hearing cats (HCs) and bilaterally congenitally deaf cats (CDCs). The ILD representation was reorganized, replacing the preference for the contralateral ear with a preference for the hearing ear, regardless of the cortical hemisphere. In accordance with the previous study, uCDCs were less sensitive to interaural time differences than HCs, resulting in unmodulated ITD responses, thus lacking directional information. Such incongruent ITDs and ILDs cannot be integrated for binaural sound source localization. In normal hearing, the predominant effect of each ear is excitation of the auditory cortex in the contralateral cortical hemisphere and inhibition in the ipsilateral hemisphere. In SSD, however, auditory pathways reorganized such that the hearing ear produced greater excitation in both cortical hemispheres and the deaf ear produced weaker excitation and preserved inhibition in both cortical hemispheres.


Asunto(s)
Corteza Auditiva , Implantación Coclear , Señales (Psicología) , Pérdida Auditiva Unilateral , Localización de Sonidos , Gatos , Animales , Localización de Sonidos/fisiología , Pérdida Auditiva Unilateral/fisiopatología , Implantación Coclear/métodos , Corteza Auditiva/fisiopatología , Femenino , Masculino , Estimulación Acústica/métodos , Lateralidad Funcional/fisiología , Sordera/fisiopatología , Sordera/congénito , Sordera/cirugía
2.
J Cell Mol Med ; 28(14): e18532, 2024 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-39039705

RESUMEN

Long non-coding RNAs (lncRNAs) have emerged as crucial regulators in the central nervous system, yet their role in vestibular compensation remains elusive. To address this knowledge gap, we employed unilateral labyrinthectomy (UL) in rats to establish animal models of peripheral vestibular dysfunction. Utilizing ribonucleic acid sequencing (RNA-seq), we comprehensively analysed the expression profiles of genes dysregulated in the medial vestibular nucleus (MVN) of these rats at distinct time points: 4 h, 4 days, and 14 days post-UL. Through trans-target prediction analysis integrating differentially co-expressed messenger RNAs (mRNAs) and lncRNAs, we constructed lncRNA-mRNA regulatory networks. Validation of selected mRNAs and lncRNAs was performed using RT-qPCR. Our RNA-seq analysis revealed significant aberrant expression of 3054 lncRNAs and 1135 mRNAs compared to control samples. By applying weighted gene co-expression network analysis (WGCNA), we identified 11 co-expressed modules encompassing all genes. Notably, within the MEmagenta module, we observed an initial upregulation of differentially expressed genes (DEGs) at 4 h, followed by downregulation at 4- and 14-days post-UL. Our findings indicated that 3068 lncRNAs positively regulated 1259 DEGs, while 1482 lncRNAs negatively regulated 433 DEGs in the MVN. The RT-qPCR results corroborated the RNA-seq data, validating our findings. This study offers novel insights into the lncRNA-mRNA expression landscape during vestibular compensation, paving the way for further exploration of lncRNA functions in this context.


Asunto(s)
Perfilación de la Expresión Génica , Redes Reguladoras de Genes , ARN Largo no Codificante , ARN Mensajero , Núcleos Vestibulares , Vestíbulo del Laberinto , Animales , Núcleos Vestibulares/metabolismo , ARN Largo no Codificante/genética , ARN Largo no Codificante/metabolismo , ARN Mensajero/genética , ARN Mensajero/metabolismo , Ratas , Masculino , Vestíbulo del Laberinto/cirugía , Vestíbulo del Laberinto/metabolismo , Regulación de la Expresión Génica , Ratas Sprague-Dawley , Transcriptoma/genética
3.
J Transl Med ; 22(1): 75, 2024 01 19.
Artículo en Inglés | MEDLINE | ID: mdl-38243264

RESUMEN

BACKGROUNDS: Unilateral high myopia (uHM), commonly observed in patients with retinal diseases or only with high myopia, is frequently associated with amblyopia with poor prognosis. This study aims to reveal the clinical and genetic spectrum of uHM in a large Chinese cohort. METHODS: A total of 75 probands with simplex uHM were included in our Pediatric and Genetic Eye Clinic. Patients with significant posterior anomalies other than myopic fundus changes were excluded. Variants were detected by exome sequencing and then analyzed through multiple-step bioinformatic and co-segregation analysis and finally confirmed by Sanger sequencing. Genetic findings were correlated with associated clinical data for analysis. RESULTS: Among the 75 probands with a mean age of 6.21 ± 4.70 years at the presentation, myopic fundus of C1 and C2 was observed in 73 (97.3%) probands. Surprisingly, specific peripheral changes were identified in 63 eyes involving 36 (48.0%) probands after extensive examination, including peripheral retinal avascular zone (74.6%, 47/63 eyes), neovascularization (54.0%), fluorescein leakage (31.7%), peripheral pigmentary changes (31.7%), and others. Exome sequencing identified 21 potential pathogenic variants of 13 genes in 20 of 75 (26.7%) probands, including genes for Stickler syndrome (COL11A1 and COL2A1; 6/20), FEVR (FZD4, LRP5, and TSPAN12; 5/20), and others (FBN1, GPR179, ZEB2, PAX6, GPR143, OPN1LW, FRMD7, and CACNA1F; 9/20). For the peripheral retinal changes in the 20 probands, variants in Stickler syndrome-related genes were predominantly associated with retinal pigmentary changes, lattice degeneration, and retinal avascular region, while variants in genes related to FEVR were mainly associated with the avascular zone, neovascularization, and fluorescein leakage. CONCLUSIONS: Genetic defects were identified in about one-fourth of simplex uHM patients in which significant consequences may be hidden under a classic myopic fundus in up to half. To our knowledge, this is the first systematic genetic study on simplex uHM to date. In addition to routine care of strabismus and amblyopia, careful examination of the peripheral retina and genetic screening is warranted for patients with uHM in order to identify signs of risk for retinal detachment and other complications and provide meaningful genetic counseling.


Asunto(s)
Ambliopía , Artritis , Enfermedades del Tejido Conjuntivo , Pérdida Auditiva Sensorineural , Miopía , Desprendimiento de Retina , Humanos , Niño , Lactante , Preescolar , Ambliopía/complicaciones , Mutación , Linaje , Miopía/genética , Fluoresceínas , Factores de Riesgo , Análisis Mutacional de ADN , Receptores Frizzled/genética , Proteínas del Citoesqueleto/genética , Proteínas de la Membrana/genética , Tetraspaninas/genética
4.
Am J Physiol Regul Integr Comp Physiol ; 326(6): R472-R483, 2024 Jun 01.
Artículo en Inglés | MEDLINE | ID: mdl-38557152

RESUMEN

The role of muscle mass in modulating performance and perceived fatigability across the entire intensity spectrum during cycling remains unexplored. We hypothesized that at task failure (Tlim), muscle contractile function would decline more following single- (SL) versus double-leg (DL) cycling within severe and extreme intensities, but not moderate and heavy intensities. After DL and SL ramp-incremental tests, on separate days, 11 recreationally active males (V̇o2max: 49.5 ± 7.7 mL·kg-1·min-1) completed SL and DL cycling until Tlim within each intensity domain. Power output for SL trials was set at 60% of the corresponding DL trial. Before and immediately after Tlim, participants performed an isometric maximal voluntary contraction (MVC) coupled with one superimposed and three resting femoral nerve stimulations [100 Hz; 10 Hz; single twitch (Qtw)] to measure performance fatigability. Perceived fatigue, leg pain, dyspnea, and effort were collected during trials. Tlim within each intensity domain was not different between SL and DL (all P > 0.05). MVC declined more for SL versus DL following heavy- (-42 ± 16% vs. -30 ± 18%; P = 0.011) and severe-intensity cycling (-41 ± 12% vs. -31 ± 15%; P = 0.036). Similarly, peak Qtw force declined more for SL following heavy- (-31 ± 12% vs. -22 ± 10%; P = 0.007) and severe-intensity cycling (-49 ± 13% vs. -40 ± 7%; P = 0.048). Except for heavy intensity, voluntary activation reductions were similar between modes. Similarly, except for dyspnea, which was lower for SL versus DL across all domains, ratings of fatigue, pain, and effort were similar at Tlim between exercise modes. Thus, the amount of muscle mass modulates the extent of contractile function impairment in an intensity-dependent manner.NEW & NOTEWORTHY We investigated the modulatory role of muscle mass on performance and perceived fatigability across the entire intensity spectrum. Despite similar time-to-task failure, single-leg cycling resulted in greater impairments in muscle contractile function within the heavy- and severe-intensity domains, but not the moderate- and extreme-intensity domains. Perceived fatigue, pain, and effort were similar between cycling modes. This indicates that the modulatory role of muscle mass on the extent of performance fatigability is intensity domain-dependent.


Asunto(s)
Ciclismo , Fatiga Muscular , Músculo Esquelético , Humanos , Masculino , Músculo Esquelético/inervación , Músculo Esquelético/fisiología , Adulto Joven , Adulto , Percepción/fisiología , Contracción Muscular , Contracción Isométrica , Estimulación Eléctrica , Esfuerzo Físico
5.
J Vasc Surg ; 80(3): 685-692, 2024 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-38677659

RESUMEN

OBJECTIVE: There is a lack of data on the role of characteristics of injured vessels on the outcomes of patients with blunt cerebrovascular injuries (BCVIs). The aim of this study was to assess the effect of the number (single vs multiple) of injured vessels on outcomes. METHODS: This is a retrospective study at two American College of Surgeons Level I trauma centers (2017-2021). Adult (>16 years) trauma patients with BCVIs are included. Injuries were graded by the Denver Scale based on the initial computed tomography angiography (CTA). Early repeat CTA was performed 7 to 10 days after diagnosis. Patients were stratified by the number (single vs multiple) of the involved vessels. Outcomes included progression of BCVIs on repeat CTA, stroke, and in-hospital mortality attributable to BCVIs. Multivariable regression analyses were performed to identify the association between the number of injured vessels and outcomes. RESULTS: A total of 491 patients with 591 injured vessels (285 carotid and 306 vertebral arteries) were identified. Sixty percent were male, the mean age was 44 years, and the median Injury Severity Score was 18 (interquartile range, 11-25). Overall, 18% had multiple-vessel injuries, 16% had bilateral vessel injuries, and 3% had multiple injuries on the same side. The overall rates of progression to higher-grade injuries, stroke, and mortality were 23%, 7.7%, and 8.8%, respectively. On uni- and multivariable analyses, multiple BCVIs were associated with progression to higher-grade injuries on repeat imaging, stroke, and mortality compared with single-vessel injuries. CONCLUSIONS: BCVIs with multiple injured vessels are more likely to progress to higher grades on repeat CTA, with multiple injuries independently associated with worse clinical outcomes, compared with those with single injuries. These findings highlight the importance of incorporating the number of injured vessels in clinical decision-making and in defining protocols for repeat imaging.


Asunto(s)
Traumatismos Cerebrovasculares , Angiografía por Tomografía Computarizada , Mortalidad Hospitalaria , Heridas no Penetrantes , Humanos , Masculino , Femenino , Estudios Retrospectivos , Adulto , Persona de Mediana Edad , Heridas no Penetrantes/diagnóstico por imagen , Heridas no Penetrantes/mortalidad , Traumatismos Cerebrovasculares/diagnóstico por imagen , Traumatismos Cerebrovasculares/mortalidad , Factores de Riesgo , Puntaje de Gravedad del Traumatismo , Medición de Riesgo , Angiografía Cerebral/métodos , Arteria Vertebral/lesiones , Arteria Vertebral/diagnóstico por imagen , Traumatismos de las Arterias Carótidas/diagnóstico por imagen , Traumatismos de las Arterias Carótidas/mortalidad , Factores de Tiempo , Valor Predictivo de las Pruebas , Accidente Cerebrovascular/diagnóstico por imagen , Accidente Cerebrovascular/etiología , Accidente Cerebrovascular/mortalidad , Centros Traumatológicos , Pronóstico
6.
Cephalalgia ; 44(2): 3331024241232256, 2024 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-38415675

RESUMEN

BACKGROUND: Short-lasting unilateral neuralgiform headache attacks (SUNHA) have the features of both short-lasting unilateral neuralgiform pain, such as trigeminal neuralgia or stabbing headache, and associated trigeminal autonomic symptoms, such as paroxysmal hemicrania or cluster headache. Recognizing and adequately treating SUNHA is essential but current treatment methods are ineffective in treating SUNHA. METHODS: We reviewed the changes in the concept of short-lasting unilateral neuralgiform headache attacks and provide a narrative review of the current medical and surgical treatment options, from the first choice of treatment for patients to treatments for selective intractable cases. RESULTS: Unlike the initial impression of an intractable primary headache disorder affecting older men, SUNHA affects both sexes throughout their lifespan. One striking feature of SUNHA is that the attacks are triggered by cutaneous or intraoral stimulation. The efficacy of conventional treatments is disappointing and challenging, and preventive therapy is the mainstay of treatment because of highly frequent attacks of a very brief duration. Amongst them, lamotrigine is effective in approximately two-third of the patients with SUNHA, and intravenous lidocaine is essential for the management of acute exacerbation of intractable pain. Topiramate, oxcarbazepine and gabapentin are considered good secondary options for SUNHA, and botulinum toxin can be used in selective cases. Neurovascular compression is commonly observed in SUNHA, and surgical approaches, such as neurovascular compression, have been reported to be effective for intractable cases. CONCLUSIONS: Recent advances in the understanding of SUNHA have improved the recognition and treatment approaches for this unique condition.


Asunto(s)
Neuralgia , Síndrome SUNCT , Cefalalgia Autónoma del Trigémino , Masculino , Femenino , Humanos , Anciano , Síndrome SUNCT/terapia , Síndrome SUNCT/tratamiento farmacológico , Cefalea , Anticonvulsivantes/uso terapéutico , Gabapentina/uso terapéutico , Lamotrigina/uso terapéutico , Cefalalgia Autónoma del Trigémino/diagnóstico , Cefalalgia Autónoma del Trigémino/terapia
7.
Am J Bot ; 111(4): e16309, 2024 04.
Artículo en Inglés | MEDLINE | ID: mdl-38584339

RESUMEN

PREMISE: Barriers at different reproductive stages contribute to reproductive isolation. Self-incompatibility (SI) systems that prevent self-pollination could also act to control interspecific pollination and contribute to reproductive isolation, preventing hybridization. Here we evaluated whether SI contributes to reproductive isolation among four co-occurring Opuntia species that flower at similar times and may hybridize with each other. METHODS: We assessed whether Opuntia cantabrigiensis, O. robusta, O. streptacantha, and O. tomentosa, were self-compatible and formed hybrid seeds in five manipulation treatments to achieve self-pollination, intraspecific cross-pollination, open pollination (control), interspecific crosses or apomixis, then recorded flowering phenology and synchrony. RESULTS: All species flowered in the spring with a degree of synchrony, so that two pairs of species were predisposed to interspecific pollination (O. cantabrigiensis with O. robusta, O. streptacantha with O. tomentosa). All species had distinct reproductive systems: Opuntia cantabrigiensis is self-incompatible and did not produce hybrid seeds as an interspecific pollen recipient; O. robusta is a dioecious species, which formed a low proportion of hybrid seeds; O. streptacantha and O. tomentosa are self-compatible and produced hybrid seeds. CONCLUSIONS: Opuntia cantabrigiensis had a strong pollen-pistil barrier, likely due to its self-incompatibility. Opuntia robusta, the dioecious species, is an obligate outcrosser and probably partially lost its ability to prevent interspecific pollen germination. Given that the self-compatible species can set hybrid seeds, we conclude that pollen-pistil interaction and high flowering synchrony represent weak barriers; whether reproductive isolation occurs later in their life cycle (e.g., germination or seedling survival) needs to be determined.


Asunto(s)
Flores , Hibridación Genética , Opuntia , Polinización , Aislamiento Reproductivo , Semillas , Autoincompatibilidad en las Plantas con Flores , Simpatría , Autoincompatibilidad en las Plantas con Flores/fisiología , Flores/fisiología , Semillas/fisiología , Opuntia/fisiología , Reproducción , Polen/fisiología , Especificidad de la Especie , Apomixis/fisiología
8.
BMC Neurol ; 24(1): 243, 2024 Jul 15.
Artículo en Inglés | MEDLINE | ID: mdl-39009960

RESUMEN

Rheumatoid meningitis (RM) is a rare extra-articular manifestation of rheumatoid arthritis (RA) that has been increasingly recognized by neurologists. However, the diversity of its clinical manifestations makes its diagnosis difficult. RM does not have a unified diagnostic standard, and its link with RA needs to be studied further. Here we report two cases of RM without a history of RA. The first patient, an 80-year-old woman, presented with sudden unilateral limb weakness, with brain MR showing abnormal signals in the leptomeningeal of the right frontal parietal. Subarachnoid hemorrhage was excluded after imaging examination, and infectious meningitis was ruled out after cerebrospinal fluid (CSF) examination. The patient was diagnosed as having RM, she had increased levels of CCP and AKA, the markers of RA, but no history of the disease or other clinical manifestations of it. Another case, a 65-year-old man, was hospitalized with Bell's palsy. We found that he had intracranial imaging changes highly consistent with those characteristic of RM during his routine examination. Except for the left peripheral facial palsy, the patient had no other neurological signs or symptoms and no RA history. After a careful physical examination, we found no joint or other manifestations or serological abnormalities consistent with RA (RF, CCP, AKA, etc.). However, after excluding infection meningitis and considering the patient's unique imaging results, we diagnosed him as having RM. We report these two cases as references for clinical diagnosis and treatment of RM, providing a discussion of our rationale.


Asunto(s)
Artritis Reumatoide , Meningitis , Humanos , Femenino , Artritis Reumatoide/complicaciones , Artritis Reumatoide/diagnóstico , Anciano de 80 o más Años , Meningitis/diagnóstico , Meningitis/complicaciones , Anciano , Masculino , Imagen por Resonancia Magnética
9.
BMC Neurol ; 24(1): 247, 2024 Jul 17.
Artículo en Inglés | MEDLINE | ID: mdl-39020271

RESUMEN

BACKGROUND: The harlequin syndrome is a rare disorder of the autonomic nervous system characterized by unilateral diminished flushing and sweating of the face following exposure to heat or physical activity. It results from sympathetic dysfunction and most commonly occurs idiopathically. A secondary development due to an underlying pathology (e.g., carotid artery dissection, tumors) must be excluded at first appearance. There is evidence that the cranial autonomic system is involved in the pathophysiology of trigeminal autonomic headaches like hemicrania continua. Therefore, an overlap in the pathophysiology of harlequin syndrome and trigeminal autonomic headache disorders seems plausible. However, the association of a harlequin syndrome with hemicrania continua was never reported. CASE PRESENTATION: This work describes the case of a 42-year-old female patient presenting to our headache unit. The patient reported persisting unilateral headache of the right side of dragging or squeezing character accompanied by trigeminal autonomic symptoms, including lacrimation, nasal congestion, conjunctival injection and Horner's syndrome, and was responsive to treatment with 75mg/d indomethacin. Five months after the initial consultation, the patient noted that the upper right quadrant of her face was pale after jogging. A harlequin syndrome was diagnosed. Further, she developed a short-lasting, bilateral headache of pulsatile character during strenuous exercise consistent with exertional headache. Comprehensive diagnostic evaluations, encompassing cranial and cervical MRI scans, laboratory tests, and biopsies, culminated in the diagnosis of Sjögren's syndrome. This finding suggests that the trigemino-autonomic dysfunction may either be idiopathic or a direct manifestation of Sjögren's syndrome. CONCLUSIONS: This report documents the case of a rare combination of a headache resembling probable hemicrania continua and the harlequin syndrome (and even exertional headache). It illustrates the underlying anatomy of the autonomic nervous system in a clinical context and emphasizes the hypothesis of a pathophysiological link between abnormal sympathetic activity and trigeminal autonomic headaches.


Asunto(s)
Enfermedades del Sistema Nervioso Autónomo , Rubor , Hipohidrosis , Humanos , Femenino , Adulto , Rubor/diagnóstico , Rubor/etiología , Hipohidrosis/diagnóstico , Hipohidrosis/complicaciones , Hipohidrosis/fisiopatología , Enfermedades del Sistema Nervioso Autónomo/diagnóstico , Enfermedades del Sistema Nervioso Autónomo/complicaciones , Enfermedades del Sistema Nervioso Autónomo/fisiopatología , Cefalea/etiología , Cefalea/diagnóstico , Cefalea/fisiopatología
10.
Headache ; 64(3): 259-265, 2024 03.
Artículo en Inglés | MEDLINE | ID: mdl-38433351

RESUMEN

OBJECTIVE: The goal of this study was to clarify whether clinical differences exist between patients with migraine who experience headache that is typically left-sided ("left-migraine") versus right-sided ("right-migraine") during attacks. BACKGROUND: Migraine has been associated with unilateral headache for millennia and remains a supportive trait for the clinical diagnosis of migraine of the International Classification of Headache Disorders. It is currently unknown why headache in migraine is commonly unilateral, and whether headache-sidedness is associated with other clinical features. METHODS: This is a cross-sectional study comparing left- versus right-migraine using all available intake questionnaires of new patients evaluated at an academic tertiary headache center over a 20-year period. Eligibility was based on patient written responses indicating the typical location of headache during attacks. In our analyses, the side of headache (left or right) was the predictor variable. The outcomes included various migraine characteristics and psychiatric comorbidities. RESULTS: We identified 6527 patients with migraine, of which 340 met study eligibility criteria. Of these, 48.8% (166/340) had left migraine, and 51.2% (174/340) had right migraine. When comparing patients with left- versus right-migraine, patients with left migraine experienced 3.6 fewer headache-free days (95% confidence interval [CI] 1.3-5.9; p = 0.002) and 2.4 more severe headache days (95% CI 0.8-4.1; p = 0.004) in the previous 4 weeks. No significant differences in age, sex, handedness, migraine characteristics, or psychiatric comorbidities were identified between the two groups. CONCLUSIONS: Patients with migraine with typically left-sided headache during attacks reported a higher burden of headache frequency and severity than those with typically right-sided headache during attacks. These findings may have implications for our understanding of migraine pathophysiology, treatment, and clinical trial design.


Asunto(s)
Trastornos Migrañosos , Humanos , Estudios Transversales , Trastornos Migrañosos/tratamiento farmacológico , Cefalea , Lateralidad Funcional/fisiología , Encuestas y Cuestionarios
11.
Audiol Neurootol ; : 1-9, 2024 May 29.
Artículo en Inglés | MEDLINE | ID: mdl-38810609

RESUMEN

BACKGROUND: Many studies have shown increased academic problems in children with unilateral hearing loss (UHL). However, whether hearing devices can ameliorate the educational difficulties associated with UHL is not well studied. Therefore, the objective of the current systematic review was to answer the question: do nonsurgical amplification devices, bone-anchored hearing aids, and/or cochlear implants improve academic outcomes in school-aged children and adolescents with UHL? METHODS: Embase, MEDLINE, Scopus, CINAHL, APA PsycInfo, ClinicalTrials.gov, and Cochrane databases were searched from inception to December 21, 2022. Published, peer-reviewed studies comparing academic outcomes in patients with UHL aged ≥5 and ≤19 years with and without hearing devices (nonsurgical amplification devices, bone-anchored hearing aids, or cochlear implants) were included. Results of studies were qualitatively synthesized, and the risk of bias was evaluated with the Effective Public Health Practice Project (EPHPP) Quality Assessment Tool. RESULTS: A total of 5,644 non-duplicate publications were identified by the search, and four studies were included for synthesis, every one of which was investigating nonsurgical amplification. One small, single-arm study demonstrated significant improvement in subjective classroom listening difficulties after a 3- to 4-month trial with a behind-the-ear hearing aid. The other three studies of nonsurgical amplification devices showed no benefit across multiple academic outcomes with FM systems and conventional and CROS-style hearing aids. DISCUSSION: The small sample sizes, heterogeneous and/or ill-defined study samples, and overall low quality of the available literature ultimately make it hard to draw definitive conclusions regarding nonsurgical amplification devices' effectiveness in improving academic outcomes in children with UHL. No articles were identified that studied cochlear implants or bone-anchored hearing aids. Further studies with high-quality study design, large sample sizes, and long-term follow-up are needed to answer this clinically important question.

12.
Kidney Blood Press Res ; 49(1): 69-80, 2024.
Artículo en Inglés | MEDLINE | ID: mdl-38185105

RESUMEN

INTRODUCTION: Renal fibrosis is a critical event in the development and progression of chronic kidney disease (CKD), and it is considered the final common pathway for all types of CKD. The prevalence of CKD is higher in females; however, males have a greater prevalence of end-stage renal disease. In addition, low birth weight and low nephron number are associated with increased risk for CKD. This study examined the development and severity of unilateral ureter obstruction (UUO)-induced renal fibrosis in male and female wild-type (ROP +/+) and mutant (ROP Os/+) mice, a mouse model of low nephron number. METHODS: Male and female ROP +/+ and ROP Os/+ mice were subjected to UUO, and kidney tissue was collected at the end of the 10-day experimental period. Kidney histological analysis and mRNA expression determined renal fibrosis, tubular injury, collagen deposition, extracellular matrix proteins, and immune cell infiltration. RESULTS: Male and female UUO mice demonstrated marked renal injury, kidney fibrosis, and renal extracellular matrix production. Renal fibrosis and α-smooth muscle actin were increased to a similar degree in ROP +/+ and ROP Os/+ mice with UUO of either sex. There were also no sex differences in renal tubular cast formation or renal infiltration of macrophage in ROP +/+ and ROP Os/+ UUO mice. Interestingly, renal fibrosis and α-smooth muscle actin were 1.5-3-fold greater in UUO-ROP +/+ compared to UUO-ROP Os/+ mice. Renal inflammation phenotypes following UUO were also 30-45% greater in ROP +/+ compared to ROP Os/+ mice. Likewise, expression of extracellular matrix and renal fibrotic genes was greater in UUO-ROP +/+ mice compared to UUO-ROP Os/+ mice. In contrast to these findings, ROP Os/+ mice with UUO demonstrated glomerular hypertrophy with 50% greater glomerular tuft area compared to ROP +/+ with UUO. Glomerular hypertrophy was not sex-dependent in any of the genotypes of ROP mice. These findings provide evidence that low nephron number contributes to UUO-induced glomerular hypertrophy in ROP Os/+ mice but does not enhance renal fibrosis, inflammation, and renal tubular injury. CONCLUSION: Taken together, we demonstrate that low nephron number contributes to enhanced glomerular hypertrophy but not kidney fibrosis and tubular injury. We also demonstrate that none of the changes caused by UUO was affected by sex in any of the ROP mice genotypes.


Asunto(s)
Insuficiencia Renal Crónica , Obstrucción Ureteral , Femenino , Masculino , Animales , Ratones , Obstrucción Ureteral/complicaciones , Obstrucción Ureteral/metabolismo , Actinas/metabolismo , Caracteres Sexuales , Riñón/patología , Insuficiencia Renal Crónica/complicaciones , Inflamación/patología , Fibrosis , Hipertrofia/patología , Ratones Endogámicos C57BL , Modelos Animales de Enfermedad
13.
Pediatr Nephrol ; 39(9): 2645-2654, 2024 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-38622348

RESUMEN

BACKGROUND: Individuals with congenital solitary functioning kidney (SFK) are at an increased risk of kidney damage. According to some studies, the risk is higher in unilateral kidney agenesis (UKA) than in unilateral multicystic dysplastic kidney (UMCDK). We hypothesized that with early detection of children with UKA and UMCDK, there would be no difference in the presence of hypertension, proteinuria, and reduced glomerular filtration rate (GFR) between UKA and UMCDK. METHODS: Based on a long-term follow-up protocol, we evaluated a cohort of 160 children followed from birth for SFK (84 with UKA and 76 with UMCDK) detected by prenatal or routine neonatal ultrasound screening. Hypertension, proteinuria, and reduced GFR were monitored as markers of kidney damage. We compared the characteristics and outcomes of the subgroups of children with UKA and UMCDK. RESULTS: GFR was reduced in 42 (26.2%) children, of whom 41 showed only mild reduction. Hypertension and proteinuria were found in 22 (13.8%) and 14 (8.8%) children, respectively. Combined kidney damage was present in 57 (35.6%) children. The UMCDK and UKA subgroups differed in GFR at final examination, with UMCDK patients being significantly more likely to have normal GFR compared to UKA patients (82% vs. 67%; p = 0.039). CONCLUSIONS: One third of the children showed signs of SFK damage, albeit mild. Patients with UKA had reduced GFR significantly more often than those with UMCDK, but did not differ in the rates of hyperfiltration injury or congenital anomalies of the kidneys and urinary tract (CAKUT) in SFK.


Asunto(s)
Diagnóstico Precoz , Tasa de Filtración Glomerular , Riñón , Riñón Displástico Multiquístico , Proteinuria , Riñón Único , Humanos , Femenino , Riñón Displástico Multiquístico/diagnóstico , Riñón Displástico Multiquístico/complicaciones , Riñón Displástico Multiquístico/fisiopatología , Masculino , Riñón Único/complicaciones , Riñón Único/diagnóstico , Riñón Único/fisiopatología , Riñón/anomalías , Riñón/fisiopatología , Riñón/diagnóstico por imagen , Recién Nacido , Pronóstico , Proteinuria/etiología , Proteinuria/diagnóstico , Lactante , Preescolar , Niño , Hipertensión/diagnóstico , Hipertensión/etiología , Hipertensión/epidemiología , Hipertensión/fisiopatología , Estudios de Seguimiento , Anomalías Congénitas/diagnóstico , Anomalías Congénitas/diagnóstico por imagen , Tamizaje Neonatal/métodos , Enfermedades Renales/congénito
14.
Pediatr Nephrol ; 39(6): 1783-1789, 2024 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-38197956

RESUMEN

BACKGROUND: The prevalence of Müllerian anomalies (MA) among patients with congenital solitary functioning kidney (SFK) is not well defined. A delay in diagnosis of obstructive MA can increase the risk of poor clinical outcomes. This study describes the prevalence of MA in patients with congenital SFK. METHODS: A retrospective review was performed of patients within the Nationwide Children's Hospital system with ICD9 or ICD10 diagnostic codes for congenital SFK defined as either unilateral renal agenesis (URA) or multicystic dysplastic kidney (MCDK) and confirmed by chart review. Patients with complex urogenital pathology were excluded. Renal anomaly, MA, reason for and type of pelvic evaluation, and age of diagnosis of anomalies were evaluated. RESULTS: Congenital SFK occurred in 431 girls due to URA (209) or MCDK (222). Pelvic evaluation, most commonly by ultrasound for evaluation of abdominal pain or dysmenorrhea, occurred in 115 patients leading to MA diagnosis in 60 instances. Among 221 patients ages 10 years and older, 104 underwent pelvic evaluation and 52 were diagnosed with an MA of which 20 were obstructive. Isolated uterine or combined uterine and vaginal anomalies were the most common MA. MA were five-fold more common in patients with URA compared to MCDK. In 75% of patients, the SFK was diagnosed prior to the MA. CONCLUSIONS: The prevalence of MA in patients with congenital SFK was 24% among those age 10 years or older, and 38% were obstructive. This justifies routine screening pelvic ultrasound in girls with congenital SFK to improve early diagnosis.


Asunto(s)
Enfermedades Renales , Riñón Displástico Multiquístico , Riñón Único , Sistema Urinario , Niño , Femenino , Humanos , Riñón Único/epidemiología , Riñón/anomalías , Enfermedades Renales/diagnóstico , Estudios Retrospectivos
15.
Cereb Cortex ; 33(14): 9165-9174, 2023 07 05.
Artículo en Inglés | MEDLINE | ID: mdl-37310142

RESUMEN

It has been proved that unilateral hearing loss (UHL) can cause functional connectivity alterations in adults. However, the mechanism of the human brain coping with the challenge of unilateral hearing deprivation at very early developmental phases remains poorly understood. Here, we performed a resting-state functional near-infrared spectroscopy (fNIRS) study on 3- to 10-month-old infants with varying degrees of unilateral hearing loss to investigate the effect of unilateral auditory deprivation in infants. Using network-based statistics, increased functional connectivity was observed in single-sided deafness (SSD) compared with normal hearing infants, and the right middle temporal gyrus was the most involved nodes. In addition, changes in cortical function in infants were related to the degree of hearing loss, with significantly increased functional connectivity in infants with severe to profound unilateral hearing loss compared with the ones with mild to moderate. Moreover, more significant cortical functional recombination changes were found in right-SSD than in left-SSD infants. For the first time, our study provides evidence for the effects of unilateral hearing deprivation on the early cortical development of the human brain, which would also act as a reference for intervention decisions in children with unilateral hearing loss in clinical settings.


Asunto(s)
Pérdida Auditiva Unilateral , Pérdida Auditiva , Adulto , Niño , Humanos , Lactante , Pérdida Auditiva Unilateral/diagnóstico por imagen , Encéfalo , Mapeo Encefálico , Tiempo
16.
Cereb Cortex ; 33(11): 7221-7236, 2023 05 24.
Artículo en Inglés | MEDLINE | ID: mdl-36806394

RESUMEN

The ability to localize sounds in patients with Unilateral Hearing Loss (UHL) is usually disrupted due to alteration in the integration of binaural cues. Nonetheless, some patients are able to compensate deficit using adaptive strategies. In this study, we explored the neural correlates underlying this adaptation. Twenty-one patients with UHL were separated into 3 groups using cluster analysis based on their binaural performance. The resulting clusters were referred to as better, moderate, and poorer performers cluster (BPC, MPC, and PPC). We measured the mismatch negativity (MMN) elicited by deviant sounds located at 10°, 20°, and 100° from a standard positioned at 50° ipsilateral to the deaf ear. The BPC exhibited significant MMN for all 3 deviants, similar to normal hearing (NH) subjects. In contrast, there was no significant MMN for 10° and 20° deviants for the PPC and for NH when one ear was plugged and muffed. Scalp distribution was maximal over central regions in BPC, while PPC showed more frontal MMN distribution. Thus, the BPC exhibited a contralateral activation pattern, similar to NH, while the PPC exhibited more symmetrical hemispheric activation. MMN can be used as a neural marker to reflect spatial adaptation in patients with UHL.


Asunto(s)
Pérdida Auditiva Unilateral , Localización de Sonidos , Humanos , Localización de Sonidos/fisiología , Pruebas Auditivas , Sonido , Plasticidad Neuronal
17.
Clin Oral Implants Res ; 35(5): 526-533, 2024 May.
Artículo en Inglés | MEDLINE | ID: mdl-38363047

RESUMEN

OBJECTIVES: This retrospective study aimed to investigate the differences in tooth loss rate between fixed implant-supported prostheses (FISPs) and removable partial dentures (RPDs) in cases of unilateral free-end missing teeth. MATERIALS AND METHODS: The data of 324 patients who underwent treatment with FISPs or RPDs for unilateral free-end missing teeth and satisfied the applicable criteria, were evaluated (47 in the FISPs group and 277 in the RPDs group). After propensity score (PS) matching, which was used to extract patients with similar background factors related to prosthetic selection at baseline, survival time analyses were performed with tooth loss as the endpoint. The adjusted variables were age, sex, number of restored teeth, periodontal status, and the practicing dentist's experience in years. The remaining teeth were classified into subcategories in relation to the missing molars. RESULTS: Overall, 58 patients (29 in each group) selected by PS matching were evaluated in the final analysis. The total number of lost teeth was 35 (FISPs group: n = 10; RPDs group: n = 25). The mean (±SD) period to tooth loss and the 10-year survival rates in the FISPs and RPDs groups were 51.6 (±30.1) months and 42.3 (±29.7) months, 70.5% and 16.4%, respectively. The log-rank test showed that significantly longer survival time in FISPs compared with RPDs. CONCLUSIONS: After adjustments for confounding factors using PS matching, replacing unilateral free-end missing teeth with FISPs may exhibit a lower tooth loss rate in adjacent and contralateral teeth compared to replacing with RPDs.


Asunto(s)
Prótesis Dental de Soporte Implantado , Dentadura Parcial Removible , Pérdida de Diente , Humanos , Femenino , Masculino , Estudios Retrospectivos , Persona de Mediana Edad , Anciano , Puntaje de Propensión , Dentadura Parcial Fija , Adulto , Arcada Parcialmente Edéntula
18.
Demography ; 61(4): 1097-1116, 2024 Aug 01.
Artículo en Inglés | MEDLINE | ID: mdl-39012245

RESUMEN

The introduction of unilateral divorce legislation (UDL) starting in the late 1960s led to spikes in U.S. divorce rates. We ask whether making divorce easier affected the educational structure of marriage. Using marriage and divorce certificate data covering 1970-1988, we provide new evidence on the evolution of the educational structure of marriage inflows (newlyweds) and outflows (divorces). Next, we leverage the timing of UDL introduction across states to estimate its effects on both flows. We find that UDL affected the educational structure of divorce but not of new marriages: it made generally unstable hypogamous couples (women married to less educated partners) less likely to divorce and made homogamous couples more stable than hypergamous ones (women married to more educated partners).


Asunto(s)
Divorcio , Escolaridad , Matrimonio , Humanos , Divorcio/legislación & jurisprudencia , Femenino , Estados Unidos , Masculino , Matrimonio/legislación & jurisprudencia , Adulto , Factores Socioeconómicos , Persona de Mediana Edad , Adulto Joven
19.
J Endocrinol Invest ; 47(3): 749-756, 2024 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-37796369

RESUMEN

PURPOSE: Primary bilateral adrenal hyperplasia (PBMAH) is associated with hypercortisolism and a heterogeneous clinical expression in terms of cortisol secretion and related comorbidities. Historically, treatment of choice was bilateral adrenalectomy (B-Adx); however, recent data suggest that unilateral adrenalectomy (U-Adx) may be an effective alternative. For the latter, factors predicting the postsurgical outcome (e.g., biochemical control) have not been identified yet. METHODS: PBMAH patients undergoing U-Adx for overt Cushing's syndrome (CS) in two tertiary care centers were retrospectively analysed. Remission was defined as a normalization of urinary free cortisol (UFC) without the need for medical treatment. The potential of hCRH test as a predictor of U-Adx outcome was evaluated in a subgroup. RESULTS: 23 patients were evaluated (69% females, mean age 55 years). Remission rate after U-Adx was 74% at last follow up (median 115 months from UAdx). Before U-Adx, a positive ACTH response to hCRH (Δ%ACTH increase > 50% from baseline) was associated with higher remission rates. CONCLUSIONS: Three of four patients with PBMAH are surgically cured with U-Adx. Pre-operative hCRH testing can be useful to predict long-term remission rates.


Asunto(s)
Adrenalectomía , Síndrome de Cushing , Femenino , Humanos , Persona de Mediana Edad , Masculino , Hormona Liberadora de Corticotropina , Hidrocortisona , Hiperplasia/cirugía , Estudios Retrospectivos , Síndrome de Cushing/diagnóstico , Síndrome de Cushing/etiología , Síndrome de Cushing/cirugía , Hormona Adrenocorticotrópica
20.
Neurol Sci ; 45(4): 1419-1428, 2024 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-38102519

RESUMEN

In recent years, the stroke incidence has been increasing year by year, and the related sequelae after stroke, such as cognitive impairment, motor dysfunction, and post-stroke depression, seriously affect the patient's rehabilitation and daily activities. Repetitive transcranial magnetic stimulation (rTMS), as a safe, non-invasive, and effective new rehabilitation method, has been widely recognized in clinical practice. This article reviews the application and research progress of rTMS in treating different functional impairments (cognitive impairment, motor dysfunction, unilateral spatial neglect, depression) after stroke in recent years, and preliminary summarized the possible mechanisms. It has been found that the key parameters that determine the effectiveness of rTMS in improving post-stroke functional impairments include pulse number, stimulated brain areas, stimulation intensity and frequency, as well as duration. Generally, high-frequency stimulation is used to excite the ipsilateral cerebral cortex, while low-frequency stimulation is used to inhibit the contralateral cerebral cortex, thus achieving a balance of excitability between the two hemispheres. However, the specific mechanisms and the optimal stimulation mode for different functional impairments have not yet reached a consistent conclusion, and more research is needed to explore and clarify the best way to use rTMS. Furthermore, we will identify the issues and challenges in the current research, explore possible mechanisms to deepen understanding of rTMS, propose future research directions, and offer insightful insights for better clinical applications.


Asunto(s)
Agnosia , Rehabilitación de Accidente Cerebrovascular , Accidente Cerebrovascular , Humanos , Estimulación Magnética Transcraneal , Accidente Cerebrovascular/complicaciones , Accidente Cerebrovascular/terapia , Encéfalo , Corteza Cerebral
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