Síndrome de Herlyn-Werner Wünderlich: reporte de dos casos / Herlyn-Werner-Wünderlich syndrome: Two case report

El síndrome de Herlyn-Werner Wünderlich, también llamado OHVIRA por sus siglas en inglés (obstructed hemivagina and ipsilateral renal anomaly), es una anomalía congénita mülleriana poco frecuente que se caracteriza por la asociación entre útero didelfo, hemivagina obstruida y agenesia renal ipsilateral. La presentación clínica más común es la masa abdominal secundaria a hematocolpos, dolor y dismenorrea. Se asocia a infertilidad, endometriosis, alteraciones menstruales y obstétricas. La ecografía es la técnica de elección para la evaluación inicial, mientras que la resonancia magnética sigue siendo el método más exacto para el diagnóstico. La septotomía vaginal es el tratamiento recomendado. Se describen 2 casos clínicos con el objetivo de destacar la importancia del diagnóstico temprano para evitar las posibles complicaciones futuras.

Herlyn-Werner-Wunderlich syndrome, also known as obstructed hemivagina and ipsilateral renal anomaly (OHVIRA), is a rare, congenital Müllerian duct anomaly characterized by the association of septate uterus, obstructed hemivagina, and ipsilateral renal agenesis. The most common clinical presentation is an abdominal mass secondary to hematocolpos, pain, and dysmenorrhea. It is associated with infertility, endometriosis, and menstrual and obstetric alterations. The ultrasound is the technique of choice for the initial assessment, while the magnetic resonance imaging remains the most accurate method for diagnosis. The resection of the vaginal septum is the recommended treatment. Here we describe 2 clinical cases to highlight the importance of an early diagnosis to prevent potential complications in the future.

Involvement of SIRT1 and SIRT3 in Effects of Hypoxic Hypobaric Preconditioning on Hippocampal Damage Induced by Chronic Cerebral Hypoperfusion in C57/6j and a7nAchRs(-/-) Mice / Participación de SIRT1 y SIRT3 en los Efectos del Precondicionamiento Hipobárico Hipóxico sobre el Daño del Hipocampo Inducido por Hipoperfusión Cerebral Crónica en Ratones C57/6j y a7nAchRs (-/-)

SUMMARY: Hypoxic preconditioning is known to induce neuroprotection, but its effects and pathways in chronic brain pathology still unknown. The aim was to establish an involvement of a7 subunit of nicotinic acetylcholine receptors (a7nAchRs), and sirtuins of 1 (SIRT1) and 3 (SIRT3) types in the effects of hypoxic hypobaric preconditioning on brain damage in mice with chronic cerebral hypoperfusion caused by the left common carotid artery occlusion. The male C57/6j (C57, wild type) and a7nAchRs(-/-) mice were divided to six experimental groups (10 mice per group): sham-operated C57, C57 with chronic cerebral hypoperfusion, C57 with hypoxic hypobaric preconditioning and chronic cerebral hypoperfusion, sham-operated a7nAchRs(-/-) mice, a7nAchRs(-/-) with chronic cerebral hypoperfusion, a7nAchRs(-/-) with hypoxic hypobaric preconditioning and chronic cerebral hypoperfusion. For preconditioning, mice were exposed to hypoxia by "lifting" in barochamber to simulated altitude of 5600 m a.s.l. for 1 h/day on 3 consecutive days before surgical manipulation. Expressions of SIRT1, SIRT3 in brain tissue, and histopathological changes of the hippocampi were examined. It was shown that 8-week chronic hypoperfusion of the brain, caused by unilateral occlusion of the common carotid artery, was accompanied by injury to the neurons of the hippocampi of both hemispheres, which was more pronounced on the side of the occlusion. This damage, as well as the mechanisms of neuroprotection induced by hypoxic preconditioning, were maintained for at least 8 weeks by mechanisms mediated through a7nAChRs. Deficite of a7nAChRs was accompanied with reduction of neuronal damage caused CCH in 8 weeks, as well as preconditioning effects, and lead to compensatory activation of regulatory and protective mechanisms mediated by SIRT1, in normal conditions and in CCH. In wild-type (C57) mice, protective mechanisms in CCH were realized to a greater extent by increased expression of SIRT3 in both hemispheres of the brain.

Se sabe que el precondicionamiento hipóxico induce neuroprotección, pero aún se desconocen sus efectos y vías en la patología cerebral crónica. El objetivo fue establecer la participación de la subunidad a7 de los receptores nicotínicos de acetilcolina (a7nAchR) y las sirtuinas de tipo 1 (SIRT1) y 3 (SIRT3) en los efectos del precondicionamiento hipóxico hipobárico sobre el daño cerebral en ratones con hipoperfusión cerebral crónica causada por la oclusión de la arteria carótida común izquierda. Los ratones macho C57/6j (C57, tipo salvaje) y a7nAchRs(-/-) se dividieron en seis grupos experimentales (10 ratones por grupo): C57 con operación simulada, C57 con hipoperfusión cerebral crónica, C57 con precondicionamiento hipobárico hipóxico y crónica. hipoperfusión cerebral, ratones a7nAchRs(-/-) operados de forma simulada, a7nAchRs(-/-) con hipoperfusión cerebral crónica, a7nAchRs(-/-) con precondicionamiento hipobárico hipóxico e hipoperfusión cerebral crónica. Para el preacondicionamiento, los ratones fueron expuestos a hipoxia "levantándolos" en una cámara de barro a una altitud simulada de 5600 m s.n.m. durante 1 h/día durante 3 días consecutivos antes de la manipulación quirúrgica. Se examinaron las expresiones de SIRT1, SIRT3 en tejido cerebral y los cambios histopatológicos de los hipocampos. Se demostró que la hipoperfusión cerebral crónica de 8 semanas, causada por la oclusión unilateral de la arteria carótida común, se acompañaba de lesión de las neuronas del hipocampo de ambos hemisferios y que era más pronunciada en el lado de la oclusión. Este daño, así como los mecanismos de neuroprotección inducidos por el precondicionamiento hipóxico, se mantuvieron durante al menos 8 semanas mediante mecanismos mediados por a7nAChR. El déficit de a7nAChR se acompañó de una reducción del daño neuronal causado por CCH en 8 semanas, así como de efectos de precondicionamiento, y condujo a una activación compensatoria de mecanismos reguladores y protectores mediados por SIRT1, en condiciones normales y en CCH. En ratones de tipo salvaje (C57), los mecanismos de protección en CCH se realizaron en mayor medida mediante una mayor expresión de SIRT3 en ambos hemisfe- rios del cerebro.

Clinical characteristics and prognostic analysis of metachronous bilateral breast carcinoma: a retrospective study based on propensity score matching.

BACKGROUND: To investigate clinical characteristics, treatment, outcomes, and prognostic risk factors of metachronous bilateral breast carcinoma (MBBC) and provide a theoretical basis for clinical management of MBBC. METHODS: This was a retrospective study. From January 1, 2010 to March 31, 2022, a total of 23,010 patients with breast cancer underwent surgical treatment at the Breast Center of the Fourth Hospital of Hebei Medical University, including 386 patients with MBBC. Propensity score matching (PSM) was performed on MBBC patients and unilateral breast cancer (UBC) patients in a 1:1 ratio, and 210 UBC patients and 210 MBBC patients were finally matched. Clinical medical records of all patients were collected, including age of onset, family history of breast cancer, tumor size, lymph node status, TNM stage, mode of surgery, menstruation, pathological type, immunohistochemical (IHC) typing, treatment, disease-free survival (DFS), and overall survival (OS). RESULTS: The result showed that age of onset of the second primary cancer (SPC) was significantly older than that of the first primary cancer (FPC) (P = 0.024). Baseline data from MPPC patients showed that the tumor size of FPC was significantly larger than that of SPC (P = 0.043), and the proportion of PR ( +) in FPC is significantly higher than that in SPC (P = 0.045). Among MBBC patients with FPC for estrogen receptor (ER) or progesterone receptor (PR) ( +) and Her-2 (-), clinical characteristics and treatment results showed that the proportion of PR ( +) in the drug-resistant group was significantly lower than that in the non-drug-resistant group. The 2-year OS rate of SPC in the drug-resistant group was significantly shorter than those of the non-drug-resistant group (78.9% vs 100%, P < 0.05). The result of PSM-based comparison between MBBC patients and UBC patients showed significantly lower proportion of MBBC patients with SPC received chemotherapy compared to UBC patients (P = 0.026), and there was no significant difference in OS and DFS between SPC course of MBBC patients and UBC patients (P > 0.05). The univariate analysis showed that high TNM stage was a risk factor for death and disease progression in MBBC patients, with the risk of death in stage III MBBC patients being about 5 times higher than that in stage I MBBC patients (HR = 4.97, 95%CI = 1.42-17.31, P = 0.012), and the risk of disease recurrence being about 3.5 times higher than that in stage I MBBC patients (HR = 3.55, 95%CI = 1.07-11.81, P = 0.039). CONCLUSION: In summary, this study presented clinical characteristics, treatment options, and outcomes of MBBC patients and patients with MBBC who were resistant to endocrine therapy have a worse SPC survival prognosis. The course of SPC in MBBC patients was similar to that of UBC in terms of prognosis and survival, which suggested that SPC can be treated according to UBC treatment regimen. High TNM stage was a prognostic risk factor for SPC patients.

Endoscopic Treatment of Lumbar Degenerative Disc Disease: A Narrative Review of Full-Endoscopic and Unilateral Biportal Endoscopic Spine Surgery.

BACKGROUND: Degenerative lumbar spine disease is the leading cause of disability and work absenteeism worldwide. Lumbar microdiscectomy became the standard treatment for herniated discs and stenotic disease. With the evolution of different techniques, endoscopic spinal surgery emerged to minimize the surgical footprint while providing at least non-inferior results. Currently, two different types of endoscopic spine procedures are dominating the surgical scenario: "Full-Endoscopic" (FE) and Unilateral Biportal Endoscopic" (UBE) Spine Surgery. The aim of this study is to describe and analyze their indications, their technical characteristicswithitsadvantagesanddisadvantagesofbothtechniquesandtheirfuture trends. METHODS: We performed a narrative review of the most relevant articles published up to August 2023 through a Pub Med search. The search terms " FE Spine Surgery" and " UBE Spine Surgery" were used. The articles selected, were independently reviewed by 3 authors and 55 full text articles were reviewed. RESULTS: The FE and UBE Spine Surgery techniques were described. The FE technique is performed with a monoportal access under constant saline irrigation. The FE comprises the transforaminal and the interlaminar approaches, and the indication depends from the pathology to treat, and still remains controversial. UBE can approach also the spine from a posterior, postero lateral,and para spinal route. It uses two different ports addressed to a target with continuous irrigation. The process of establishing these two portals is called triangulation. CONCLUSIONS: FE and UBE spine surgery have demonstrated outcomes comparable to open surgery, minimizing complications and surgical footprint.

Herlyn-Werner-Wünderlich syndrome: Two case report. / Síndrome de Herlyn-Werner Wünderlich:reporte de dos casos.

Herlyn-Werner-Wunderlich syndrome, also known as obstructed hemivagina and ipsilateral renal anomaly (OHVIRA), is a rare, congenital Müllerian duct anomaly characterized by the association of septate uterus, obstructed hemivagina, and ipsilateral renal agenesis. The most common clinical presentation is an abdominal mass secondary to hematocolpos, pain, and dysmenorrhea. It is associated with infertility, endometriosis, and menstrual and obstetric alterations. The ultrasound is the technique of choice for the initial assessment, while the magnetic resonance imaging remains the most accurate method for diagnosis. The resection of the vaginal septum is the recommended treatment. Here we describe 2 clinical cases to highlight the importance of an early diagnosis to prevent potential complications in the future.

El síndrome de Herlyn-Werner Wünderlich, también llamado OHVIRA por sus siglas en inglés (obstructed hemivagina and ipsilateral renal anomaly), es una anomalía congénita mülleriana poco frecuente que se caracteriza por la asociación entre útero didelfo, hemivagina obstruida y agenesia renal ipsilateral. La presentación clínica más común es la masa abdominal secundaria a hematocolpos, dolor y dismenorrea. Se asocia a infertilidad, endometriosis, alteraciones menstruales y obstétricas. La ecografía es la técnica de elección para la evaluación inicial, mientras que la resonancia magnética sigue siendo el método más exacto para el diagnóstico. La septotomía vaginal es el tratamiento recomendado. Se describen 2 casos clínicos con el objetivo de destacar la importancia del diagnóstico temprano para evitar las posibles complicaciones futuras.

Variables Associated With Knee Valgus in Male Professional Soccer Players During a Single-Leg Vertical Landing Task.

Prior studies have explored the relationship between knee valgus and musculoskeletal variables to formulate injury prevention programs, primarily for females. Nonetheless, there is insufficient evidence pertaining to professional male soccer players. Here, the aim was to test the correlation of lateral trunk inclination, hip adduction, hip internal rotation, ankle dorsiflexion range of motion, and hip isometric strength with knee valgus during the single-leg vertical jump test. Twenty-four professional male soccer players performed a single-leg vertical hop test, hip strength assessments, and an ankle dorsiflexion range of motion test. A motion analysis system was employed for kinematic analysis. Maximal isometric hip strength and ankle dorsiflexion range of motion were tested using a handheld dynamometer and a digital inclinometer, respectively. The correlation of peak knee valgus with peak lateral trunk inclination was .43 during the landing phase (P = .04) and with peak hip internal rotation was -.68 (P < .001). For knee valgus angular displacement, only peak lateral trunk inclination presented a moderate positive correlation (r = .40, P = .05). This study showed that trunk and hip kinematics are associated with knee valgus, which could consequently lead to increased knee overload in male professional soccer players following a unilateral vertical landing test.

Continuous Unilateral Cephalalgia Due to Systemic Lupus Erythematosus: A Case Report / Cefaleia unilateral contínua por lúpus eritematoso sistêmico: relato de caso

BACKGROUND: Hemicrania continua is a rare form of cephalalgia featuring a chronic and persistent headache in only one side of the head. OBJECTIVES: In this report, we present a case of a patient with hemicrania continua and systemic lupus erythematosus (SLE). METHODS: We collected patient data through the electronic medical record. Afterward, we reviewed the literature regarding hemicrania continua and its pathophysiology and correlation with neurovascular alterations, inflammation, and SLE. RESULTS: A 42-year-old woman visited the emergency department due to worsening constant unilateral cephalalgia that had been present for the past 6 months. The patient reported a highly intense (10/10) headache in the entire left hemicrania that radiated to the left shoulder. During physical examination, she presented with nystagmus, vertigo, and aggravated cephalalgia associated to body movement and, despite having no optic nerve thickening. In addition, she had jaundice, tachycardia, and splenomegaly. Complimentary exams found deep anemia, depletion in complement system and anti-nuclear factors, suggesting a possible hemolytic anemia (AIHA) due to SLE. Treatment was initiated with hydrocortisone and prednisone, associated with amitriptyline, fluoxetine and diazepam, reaching full remission. CONCLUSION: These syndromes have aggravated each other, and possibly the explanation for the cephalalgia remission was the control of AIHA and SLE. It features a rare case in literature and thus warrants discussion.

INTRODUÇÃO: Hemicrania contínua é uma forma rara de cefaléia caracterizada por cefaleia crônica e persistente em apenas um lado da cabeça. OBJETIVOS: Neste relato apresentamos o caso de um paciente com hemicrania contínua e lúpus eritematoso sistêmico (LES). MÉTODOS: Coletamos dados dos pacientes por meio do prontuário eletrônico. Posteriormente, revisamos a literatura sobre a hemicrania contínua e sua fisiopatologia e correlação com alterações neurovasculares, inflamação e LES. RESULTADOS: Uma mulher de 42 anos recorreu ao serviço de urgência devido ao agravamento da cefaleia unilateral constante, presente nos últimos 6 meses. O paciente relatou cefaleia de alta intensidade (10/10) em toda a hemicrânia esquerda com irradiação para o ombro esquerdo. Ao exame físico apresentava nistagmo, vertigem e cefaléia agravada associada à movimentação corporal e, apesar de não apresentar espessamento do nervo óptico. Além disso, ela apresentava icterícia, taquicardia e esplenomegalia. Os exames complementares evidenciaram anemia profunda, depleção do sistema complemento e fatores antinucleares, sugerindo uma possível anemia hemolítica (AIHA) por LES. Iniciou-se tratamento com hidrocortisona e prednisona, associadas a amitriptilina, fluoxetina e diazepam, atingindo remissão completa. CONCLUSÃO: Essas síndromes agravaram-se mutuamente e possivelmente a explicação para a remissão da cefaléia foi o controle da AIHA e do LES. Apresenta um caso raro na literatura e, portanto, merece discussão.

Spontaneous orbitofacial neurofibroma in a sow / Neurofibroma orbitofacial espontâneo em uma matriz suína

Neurofibromas are neoplasms derived from nerve bundles and are frequent in humans but not common in animals. This report described the macroscopic, histologic, and immunohistochemistry findings of an orbitofacial neurofibroma in a sow. The sow presented left eyelids with marked expansion, associated with severe ectropion and reddening of both conjunctival mucosae. The mass on the cutting surface was homogeneous, with a light tan, and shiny, measuring 9.0 x 7.0 x 7.0 cm in width, compressing the eyeball. Microscopically, there was paucicellular neoplastic proliferation of elongated cells amid the accentuated myxoid matrix. There were multifocal areas where well-differentiated nervous fascicles and skeletal musculature were seen. Neoplastic cells were immunostained positive for GFAP, neurofilament, S-100, Sox-10, and vimentin. Cytokeratin showed immunolabeling around primitive nerve bundles and desmin around neoformed vessels and muscle bundles within the tumor. Orbitopalpebral and orbitofacial neurofibroma, despite being common in humans, have not been described in domestic pigs and should be considered as a differential diagnosis in eyelid tumors in pigs.

Neurofibromas são neoplasias derivadas dos feixes nervosos e são frequentes em humanos, mas incomuns em espécies animais domésticos. Esse relato tem como objetivo descrever os achados macroscópicos, histológicos e imuno-histoquímicos de um neurofibroma orbitofacial em uma matriz suína. O animal apresentava as pálpebras esquerdas acentuadamente expandidas, associada a severo ectrópio e avermelhamento de ambas as mucosas conjuntivais. A tumoração palpebral, na superfície de corte, era homogênea, levemente acastanhada e brilhante, medindo 9,0 x 7,0 x 7,0 cm, e comprimia o globo ocular. Microscopicamente foi observada uma proliferação neoplásica pobremente celular de células alongadas no meio de uma acentuada quantidade de matriz mixoide. Havia áreas intratumorias multifocais onde fascículos nervosos bem diferenciados e musculatura esquelética foram observados. As células neoplásicas foram positivas na imuno-histoquímica para GFAP, neurofilamento, S-100, Sox-10 e vimentina. Citoqueratina mostrou uma imunomarcação ao redor de feixes nervosos primitivos e desmina ao redor de vasos neoformados e feixes musculares dentro do tumor. O neurofibroma orbitofacial, apesar de comum em humanos, nunca foi descrito em suínos domésticos e deve ser considerado um diagnóstico diferencial para neoplasias palpebrais em suínos.

Is it Always Laryngopharyngeal Reflux? How Voice Disorders Can Manifest as LPR

Introduction: Laryngopharyngeal reflux (LPR) manifests with a constellation of common throat symptoms and inconclusive signs on laryngoscopic exam. It is a diagnosis, often made clinically, that can lead to prescriptions of proton pump inhibitors that are unnecessary and potentially harmful. Glottic insufficiency (GI) and the accompanying hyperfunctional laryngeal behaviors can also present with similar, common throat complaints that may or may not include a qualitative change to the voice. Methods: This is a reflection article. It is written to summarize, explain, and support with evidence the opinion of the author on the topic of how symptoms of voice disorders can easily be mistaken for symptoms of LPR. The offered reflection is based on his experience, research and the available literature. Reflection: This article intends to explore the similarities between GI and LPR, how to ultimately differentiate them and how to approach treatment with a broader differential diagnosis. Conclusion: LPR and GI can present with identical, vague throat, and voice symptoms. Empiric medication trials, behavioral interventions and objective laryngovideostroboscopy, impedance-based reflux, and esophageal motility testing may all be needed, sometimes in a trial and error fashion, to correctly diagnose and treat a patient's symptoms.

Introducción: El reflujo laríngeo-faríngeo (LPR, por sus siglas en inglés) se manifiesta con una serie de síntomas comunes en la garganta y signos no concluyentes en el examen larinoscópico. Es un diagnóstico que a menudo se realiza clínicamente y que puede llevar a la prescripción de inhibidores de la bomba de protones que son innecesarios y potencialmente perjudiciales. La insuficiencia glótica (IG) y los comportamientos laríngeos hiperfuncionales que la acompañan también pueden presentar síntomas de garganta comunes similares, que pueden o no incluir un cambio cualitativo en la voz. Métodos: Este es un artículo de reflexión. Está escrito para resumir, explicar y respaldar con evidencia la opinión del autor sobre cómo los síntomas de los trastornos de la voz pueden confundirse fácilmente con los síntomas del LPR. La reflexión ofrecida se basa en su experiencia, investigación y la literatura disponible. Reflexión: Este artículo tiene la intención de explorar las similitudes entre la IG y el LPR, cómo diferenciarlos finalmente y cómo abordar el tratamiento con un diagnóstico diferencial más amplio. Conclusión: El LPR y la IG pueden presentar síntomas idénticos y vagos en la garganta y la voz. Puede ser necesario realizar ensayos de medicación empírica, intervenciones conductuales y pruebas objetivas de laringovideostroboscopia, reflujo basado en impedancia y motilidad esofágica, a veces de manera experimental, para diagnosticar y tratar correctamente los síntomas de un paciente.

Middle Ear Cholesteatoma and Vestibular Schwannoma Resection Followed by Cochlear Implant: Surgical Challenges and Audiological Outcomes.

(1) Background: The occurrence of vestibular schwannoma (VS) associated with cholesteatoma is rare. A hearing impairment is one of the most significant issues in such cases. Moreover, the presence of middle and inner ear pathologies combined may represent a surgical challenge. No studies have described a combined surgical approach for these coexisting conditions (VS and cholesteatoma), nor the hearing rehabilitation outcomes of using cochlear implants for these patients. (2) Case Report: This paper is on a female patient who underwent simultaneous surgical treatments for VS and middle ear cholesteatoma in the right ear followed by a cochlear implant, describing the technique and the audiological results. (3) Conclusions: The surgical approach was successful and enabled the resection of lesions with the auditory nerve and cochlea preservation. Cochlear implantation in the right ear showed positive postoperative results, with an improvement in the results with the CI in silent and noisy environments.

Popcorn (Zea mays L. var. Everta) haploids identified by Navajo phenotype and ploidy level.

For popcorn, obtaining and identifying haploids are still challenging steps. We aimed to induce and screen haploids in popcorn using the Navajo phenotype, seedling vigor, and ploidy level. We used the Krasnodar Haploid Inducer (KHI) in crosses with 20 popcorn source germplasms and five maize controls. The field trial design was completely randomized, with three replications. We assessed the efficacy of induction and identification of haploids based on the haploidy induction rate (HIR) and false positive and negative rates (FPR and FNR). Additionally, we also measured the penetrance of the Navajo marker gene (R1-nj). All putative haploids classified by the R1-nj were germinated together with a diploid sample and evaluated for false positives and negatives based on vigor. Seedlings from 14 females were submitted to flow cytometry to determine the ploidy level. The HIR and penetrance were analyzed by fitting a generalized linear model with a logit link function. The HIR of the KHI, adjusted by cytometry, ranged from 0.0 to 1.2%, with a mean of 0.34%. The average FPR from screening based on the Navajo phenotype was 26.2% and 76.4% for vigor and ploidy, respectively. The FNR was zero. The penetrance of R1-nj ranged from 30.8 to 98.6%. The average number of seeds per ear in temperate germplasm (76) was lower than that obtained in tropical germplasm (98). There is an induction of haploids in germplasm of tropical and temperate origin. We recommend the selection of haploids associated with the Navajo phenotype with a direct method of confirming the ploidy level, such as flow cytometry. We also show that haploid screening based on Navajo phenotype and seedling vigor reduces misclassification. The origin and genetic background of the source germplasm influence the R1-nj penetrance. Because the known inducers are maize, developing doubled haploid technology for popcorn hybrid breeding requires overcoming unilateral cross-incompatibility.

Early Treatment of Unilateral Condylar Hyperplasia in Adolescents: Preliminary Results.

Facial asymmetry associated with unilateral condylar hyperplasia (UCH) is a rare disease. The aim of this study was to evaluate the clinical conditions of progressive facial asymmetry in young subjects treated with high condylectomy. A retrospective study was performed including nine subjects diagnosed with UCH type 1B and progressive facial asymmetry around 12 years old with an upper canine progressing towards dental occlusion. After an analysis and a decision of treatment, orthodontics began one to two weeks prior to the condylectomy (with a mean vertical reduction of 4.83 ± 0.44 mm). Facial and dental asymmetry, dental occlusion, TMJ status and an open/closing mouth were analyzed before surgery and in the final stage of treatment, almost 3 years after surgery. Statistical analyses were performed using the Shapiro-Wilk test and a Student's t-test considering a p value of <0.05. Comparing T1 (before surgery) and T2 (once orthodontic treatment was finalized), the operated condyle showed a similar height to that observed in stage 1 with a 0.12 mm difference in height (p = 0.8), whereas the non-operated condyle showed greater height increase with an average of 3.88 mm of vertical growth (p = 0.0001). This indicated that the non-operated condyle remained steady and that the operative condyle did not register significant growth. In terms of facial asymmetry in the preoperative stage, a chin deviation of 7.55 mm (±2.57 mm) was observed; in the final stage, there was a significant reduction in the chin deviation with an average of 1.55 mm (±1.26 mm) (p = 0.0001). Given the small number of patients in the sample, we can conclude that high condylectomy (approx. 5 mm), if performed early, especially in the mixed-dentition stage before full canine eruption, is beneficial for the early resolution of asymmetry and thus the avoidance of future orthognathic surgery. However, further follow-up until the end of facial growth is required.

Laparoscopic right adrenalectomy in a patient with Carney syndrome: A case report.

INTRODUCTION AND IMPORTANCE: Carney complex (CNC) is an extremely infrequent multiple endocrine neoplasia syndrome characterized by distinctive pigmented skin and mucosal lesions, cardiac and noncardiac myxomatous tumors, and multiple endocrine tumors. We herein report a case of CNC and surgical and history of laparoscopic left adrenalectomy complicated with a primary pigmented nodular adrenocortical disease (PPNAD). PRESENTATION OF CASE: We present the case of a 38-year-old woman with a previous diagnosis of CNC and history of laparoscopic left adrenalectomy who consulted for severe depression refractory to medical treatment. In the laboratory tests performed, altered ACTH, prolactin, Somatomedin C-IGF-1 and estradiol. An abdomen and pelvis C/T scan was requested, where an 8 mm lesion was found at the level of the right adrenal gland. Laparoscopic right adrenalectomy was performed. Histopathology of the surgical resection specimen revealed PPNAD. DISCUSSION: CNC is an infrequent syndrome with autosomal dominant inheritance and genetically heterogeneous. PPNAD is a consistent feature in CNC patients, however, reports of Cushing's syndrome in the literature indicate that only 25-45 % of CNC patients have PPNAD. CONCLUSION: PPNAD can be present in patients with Carney complex, with surgical adrenalectomy history. With an adequate selection of patients, laparoscopic adrenalectomy with subsequent hormone replacement therapy should be performed.

Comportamiento de la maloclusión funcional e interferencias oclusales en niños con dentición mixta

Introducción: las interferencias oclusales no controladas precozmente pueden producir desviación de la mandíbula en sentido anteroposterior o transversal. El manejo de las mordidas cruzadas se debe iniciar en el momento del diagnóstico, preferiblemente en edades tempranas, con el fin de tratarlas en el nivel primario de prevención. Objetivo: determinar el comportamiento de la maloclusión funcional causada por interferencias oclusales en niños con dentición mixta de la Escuela Primaria Mártires del Corynthia, entre octubre de 2019 y junio de 2021. Materiales y métodos: se realizó un estudio observacional descriptivo de corte transversal en la Escuela Primaria Mártires del Corynthia, del Área de Salud José Jacinto Milanés, del municipio de Matanzas, entre octubre de 2019 y junio de 2021. El universo estuvo conformado por 66 niños. Se utilizó una planilla de recolección de datos y se solicitó el consentimiento informado a los tutores de los niños. Resultados: las edades donde los niños presentaron mayor afectación fueron de 6 a 7 años y de 8 a 9 años, ambos rangos con un 10,6 %. El 27,3 % presentó interferencias oclusales y mordida cruzada posterior unilateral. El 48,5 % fue del sexo femenino y el 30,3 % tenían edades de 6 a 7 años. El 71,2 % eran simétricos y presentaron mordida cruzada posterior unilateral. Los simétricos y con línea media coincidente representaron un 36,4 %. Conclusiones: la maloclusión funcional más frecuente en niños con dentición mixta fue la mordida cruzada posterior unilateral, que se relacionó de manera directa con las interferencias oclusales. Las asimetrías faciales y la línea media desviada estuvieron asociadas a dicha maloclusión.

Introduction: early uncontrolled occlusal interferences can produce anterior-posterior jaw deflection. Management of cross-bites should be initiated at the time of diagnosis, preferably at early ages, in order to treat them at the primary level of prevention. Objective: to determine the behavior of functional malocclusion caused by occlusal interferences in children with mixed dentition from the Martires del Corynthia primary school between October 2019 and June 2021. Materials and methods: a cross-sectional, observational, descriptive study was carried out at the Martires del Corynthia Primary School, of the Jose Jacinto Milanes Health Area, Matanzas municipality, between October 2019 and June 2021. The universe consisted of 66 children. A data collection form was used and informed consent was requested from the children's guardians. Results: the ages where the children presented more affectation were from 6 to 7 years and from 8 to 9 years, both ranges with 10.6%. 27.3% presented occlusal interferences and posterior unilateral cross-bite. 48.5% were female and 30.3% were 6 to 7 years old. 71.2% were symmetrical and presented unilateral posterior cross-bite. Symmetric patients and with a coincident midline represented 36.4%. Conclusions: the most frequent functional malocclusion in children with mixed dentition was the unilateral posterior cross-bite, which was related to occlusal interferences in a direct way. Facial asymmetries and a deviated midline were associated with the before mentioned malocclusion.

Síndrome de cascanueces y agenesia renal derecha. Una rara asociación / Nutcracker Syndrome and Right Renal Agenesis. A Rare Association

Introducción: El síndrome de cascanueces se considera una anomalía infrecuente y poco pensada en la práctica médica; su incidencia no está bien establecida debido a su sintomatología variada. La agenesia renal unilateral se estima entre 1/2500 y 1/4000 nacidos vivos. Objetivos: Describir una paciente de 18 años con sospecha prenatal de agenesia renal derecha confirmada por imágenes después del nacimiento, que ingresó por proteinuria. Presentación del caso: Paciente de 18 años con agenesia renal derecha conocida, en la que un examen de orina detectó proteinuria desde los 13 años. En su estudio se clasificó como proteinuria ortostática y evolutivamente refirió dolor lumbar izquierdo ligero y transitorio. En el ultrasonido renal, realizado para valorar crecimiento del riñón único, se detectó dilatación de la vena renal izquierda. Se repitió el estudio ecográfico para precisar ángulo aorto-mesentérico y dilatación de vena renal izquierda, y se confirmó el síndrome de cascanueces. Conclusiones: La asociación entre agenesia renal derecha y síndrome de cascanueces, resulta extremadamente rara, y, cuando se presenta con proteinuria ortostática y dolor lumbar ocasional, debe seguirse en forma expectante, pero no se necesita tratamiento quirúrgico en la mayoría de los casos(AU)

Introduction: Nutcracker syndrome is considered an infrequent and poorly thought out anomaly in medical practice; its incidence is not well established due to its varied symptomatology. Unilateral renal agenesis is estimated to be between 1/2500 and 1/4000 live births. Objectives: To describe an 18-year-old female patient with suspected pre-natal imaging-confirmed right renal agenesis after birth, who was admitted due to proteinuria. Case presentation: An 18-year-old female patient with known right renal agenesis, in whom a urine test detected proteinuria from the age of 13. In the study it was classified as orthostatic proteinuria and evolutionarily she referred mild and transient left low back pain. Renal ultrasound, performed to assess single kidney growth, showed dilation of the left renal vein. The ultrasound study was repeated to specify aorto-mesenteric angle and left renal vein dilation, and nutcracker syndrome was confirmed. Conclusions: The association between right renal agenesis and nutcracker syndrome is extremely rare, and, when it presents with orthostatic proteinuria and occasional low back pain, it should be followed expectantly, but surgical treatment is not needed in most cases(AU)

Barriers to patient recruitment in a poststroke neurorehabilitation multicenter trial in Brazil

There is a high demand for stroke rehabilitation in the Brazilian public health system, but most studies that have addressed rehabilitation for unilateral spatial neglect (USN) after stroke have been performed in high-income countries. Therefore, the aim of this study was to analyze USN patient recruitment in a multicenter noninvasive brain stimulation clinical trial performed in Brazil and to provide study design recommendations for future studies. We evaluated the reasons for exclusion of patients from a multicenter, randomized, double-blinded clinical trial of rehabilitation of USN patients after stroke. Clinical and demographic variables were compared between the included and excluded patients. A descriptive statistical analysis was performed. Only 173 of the 1953 potential neglect patients (8.8%) passed the initial screening. After screening evaluation, 87/173 patients (50.3%) were excluded for clinical reasons. Cognitive impairment led to the exclusion of 21/87 patients (24.1%). Low socioeconomic status led to the exclusion of 37/173 patients (21.4%). Difficulty obtaining transportation to access treatment was the most common reason for their exclusion (16/37 patients, 43.3%). The analyzed Brazilian institutions have potential for conducting studies of USN. The recruitment of stroke survivors with USN was restricted by the study design and limited financial support. A history of cognitive impairment, intracranial stenting or craniectomy, and lack of transportation were the most common barriers to participating in a multicenter noninvasive brain stimulation trial among patients with USN after stroke.

Hearing rehabilitation of children and adolescents with unilateral hearing loss / Reabilitação auditiva de crianças e adolescentes com perda auditiva unilateral

ABSTRACT Purpose To describe the audiological characteristics and the type of intervention chosen on unilateral hearing loss cases in children and adolescents as well as to analyze correlations between the degree of hearing loss, the indication and the use of electronic devices. Methods Observational, descriptive and retrospective study, carried out with information of 34 medical records from children and adolescents with unilateral hearing loss, assessed by two auditory rehabilitation services of medium complexity, throughout 2016 to 2019. Descriptive and Inferential statistical analysis were performed with the data. Results A predominance of profound sensorineural unilateral hearing loss in the right ear, of pre-lingual character, with 20.6% of malformations. The most adopted intervention was the hearing aid indication, although its use is low, regardless of the degree of the hearing loss. An association was found between the degree of the hearing loss and the healthcare professionals in indicating the use of the devices. Conclusion The indication of hearing aids is the most frequent and this decision is influenced by the degree of the hearing loss, in which the devices are mostly indicated for mild to severe losses, with bigger divergence of conduct for profound hearing losses.

RESUMO Objetivo Descrever as características audiológicas e o tipo de intervenção tomada em casos de perda auditiva unilateral em crianças e adolescentes e analisar correlações entre o grau da deficiência auditiva, a indicação e o uso de auxiliares de audição. Método Estudo observacional, descritivo e retrospectivo, realizado com informações dos prontuários de 34 crianças e adolescentes com perda auditiva unilateral, atendidos em dois serviços de reabilitação auditiva de média complexidade, no período de 2016 a 2019. Realizou-se a análise estatística descritiva e inferencial dos dados. Resultados Predomínio de perda auditiva unilateral do tipo neurossensorial, de grau profundo à direita, pré-lingual com 20,6% de malformações. A intervenção mais adotada foi a indicação de prótese auditiva, porém o uso é baixo, independente do grau da perda. Houve associação entre o grau da perda auditiva e a decisão dos profissionais em indicar o uso dos dispositivos. Conclusão A indicação de próteses auditivas é a mais frequente e esta decisão é influenciada pelo grau da perda auditiva, sendo os aparelhos auditivos indicados para as perdas leves a severas, com uma maior divergência de condutas para as perdas profundas.

Sulforaphane Protects against Unilateral Ureteral Obstruction-Induced Renal Damage in Rats by Alleviating Mitochondrial and Lipid Metabolism Impairment.

Unilateral ureteral obstruction (UUO) is an animal rodent model that allows the study of obstructive nephropathy in an accelerated manner. During UUO, tubular damage is induced, and alterations such as oxidative stress, inflammation, lipid metabolism, and mitochondrial impairment favor fibrosis development, leading to chronic kidney disease progression. Sulforaphane (SFN), an isothiocyanate derived from green cruciferous vegetables, might improve mitochondrial functions and lipid metabolism; however, its role in UUO has been poorly explored. Therefore, we aimed to determine the protective effect of SFN related to mitochondria and lipid metabolism in UUO. Our results showed that in UUO SFN decreased renal damage, attributed to increased mitochondrial biogenesis. We showed that SFN augmented peroxisome proliferator-activated receptor γ co-activator 1α (PGC-1α) and nuclear respiratory factor 1 (NRF1). The increase in biogenesis augmented the mitochondrial mass marker voltage-dependent anion channel (VDAC) and improved mitochondrial structure, as well as complex III (CIII), aconitase 2 (ACO2) and citrate synthase activities in UUO. In addition, lipid metabolism was improved, observed by the downregulation of cluster of differentiation 36 (CD36), sterol regulatory-element binding protein 1 (SREBP1), fatty acid synthase (FASN), and diacylglycerol O-acyltransferase 1 (DGAT1), which reduces triglyceride (TG) accumulation. Finally, restoring the mitochondrial structure reduced excessive fission by decreasing the fission protein dynamin-related protein-1 (DRP1). Autophagy flux was further restored by reducing beclin and sequestosome (p62) and increasing B-cell lymphoma 2 (Bcl2) and the ratio of microtubule-associated proteins 1A/1B light chain 3 II and I (LC3II/LC3I). These results reveal that SFN confers protection against UUO-induced kidney injury by targeting mitochondrial biogenesis, which also improves lipid metabolism.

Expression Profiles of Kidney Mitochondrial Proteome during the Progression of the Unilateral Ureteral Obstruction: Focus on Energy Metabolism Adaptions.

Kidney diseases encompass many pathologies, including obstructive nephropathy (ON), a common clinical condition caused by different etiologies such as urolithiasis, prostatic hyperplasia in males, tumors, congenital stenosis, and others. Unilateral ureteral obstruction (UUO) in rodents is an experimental model widely used to explore the pathophysiology of ON, replicating vascular alterations, tubular atrophy, inflammation, and fibrosis development. In addition, due to the kidney's high energetic demand, mitochondrial function has gained great attention, as morphological and functional alterations have been demonstrated in kidney diseases. Here we explore the kidney mitochondrial proteome differences during a time course of 7, 14, and 21 days after the UUO in rats, revealing changes in proteins involved in three main metabolic pathways, oxidative phosphorylation (OXPHOS), the tricarboxylic acid cycle (TCA), and the fatty acid (FA) metabolism, all of them related to bioenergetics. Our results provide new insight into the mechanisms involved in metabolic adaptations triggered by the alterations in kidney mitochondrial proteome during the ON.

A retrospective analysis of revision framework surgeries for unilateral vocal fold paralysis / Análise retrospectiva das cirurgias de revisão do arcabouço laríngeo para paralisia unilateral de prega vocal

Abstract Introduction Revision framework surgeries might be required for unilateral vocal fold paralyses. However, outcomes and indications of revision surgeries have not been adequately documented. For a better understanding of indications for the procedure and to help in achieving better vocal outcomes, we performed a retrospective chart review of patients who underwent revision framework surgeries for unilateral vocal fold paralysis. Objectives This study aimed to present clinical features of patients who underwent revision framework surgeries for the treatment of unilateral vocal fold paralysis. Methods Of the 149 framework surgeries performed between October 2004 and October 2019, 21 revision framework surgeries were performed in 19 patients. Self-assessments by patients using the voice handicap index-10 questionnaire, and objective aerodynamic and acoustic assessments performed pre- and post-operatively were analyzed using the Wilcoxon's signed-rank test for paired comparisons. Results Undercorrection was indicated as reasons for revision surgeries in all cases. The revision techniques included type I thyroplasty, type IV thyroplasty, and arytenoid adduction, and revision surgeries were completed without any severe complication in all cases. Pre- and post-operative voice handicap index-10 scores were obtained in 12 cases, and other parameters were evaluated in 18 cases. Significant improvements were observed in voice handicap index-10 scores, maximum phonation time, mean flow rate, Current/Direct Current ratio, and pitch perturbation quotient. Conclusion Undercorrection was observed in all patients who underwent revision framework surgeries for unilateral vocal fold paralysis, and the initial assessment and planning are thought to be important in order to avoid revision surgeries. Revision surgeries were performed safely in all cases, and significantly improved vocal outcomes were observed, even after multiple procedures. Revision surgery should be considered for patients with unsatisfactory vocal functions after primary framework surgeries for unilateral vocal fold paralysis.

Resumo Introdução As cirurgias de revisão do arcabouço laríngeo podem ser necessárias em casos de paralisia unilateral de prega vocal. Entretanto, os resultados e as indicações das cirurgias de revisão não têm sido documentados de forma adequada. Para melhor compreensão das indicações do procedimento e para auxiliar na obtenção de melhores resultados vocais, fizemos uma revisão retrospectiva dos prontuários de pacientes submetidos a cirurgias de revisão do arcabouço laríngeo em paralisia unilateral de prega vocal. Objetivos Apresentar as características clínicas de pacientes submetidos a cirurgias de revisão do arcabouço laríngeo para tratamento de paralisia unilateral de prega vocal. Método Das 149 cirurgias de revisão do arcabouço laríngeo feitas entre outubro de 2004 e outubro de 2019, 21 cirurgias de revisão do arcabouço laríngeo foram feitas em 19 pacientes. As autoavaliações feitas pelos pacientes com o questionário voice handicap index‐10 e avaliações aerodinâmicas e acústicas objetivas feitas no pré e pós‐operatório foram analisadas com o teste de postos sinalizados de Wilcoxon para comparações pareadas. Resultados A hipocorreção foi apontada como o motivo das cirurgias de revisão em todos os casos. As técnicas de revisão incluíram tireoplastia tipo I, tireoplastia tipo IV e adução de aritenoide. As cirurgias de revisão foram feitas sem qualquer complicação grave em todos os casos. Os escores do questionário voice handicap index‐10 pré e pós‐operatórios foram obtidos em 12 casos e outros parâmetros foram avaliados em 18 casos. Melhorias significativas foram observadas nos escores do questionário, no tempo máximo de fonação, taxa de fluxo médio, relação antes/depois e no quociente de perturbação do pitch. Conclusão Hipocorreção foi observada em todos os pacientes submetidos a cirurgias de revisão do arcabouço laríngeo para paralisia unilateral de prega vocal e a avaliação inicial e o planejamento são considerados importantes para evitar cirurgias de revisão. As cirurgias de revisão foram feitas com segurança em todos os casos e melhoria significativa dos resultados vocais foi observada mesmo após múltiplos procedimentos. A cirurgia de revisão deve ser considerada para pacientes com funções vocais insatisfatórias após cirurgia primária do arcabouço laríngeo para paralisia unilateral de prega vocal.