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INTRODUCTION: In recent years, major findings on concomitant procedures and anticoagulation management have occurred in Mitral Valve (MV) surgery. Therefore, we sought to evaluate the current practices in MV interventions across Europe. METHODS: In October 2021, all national cardio-thoracic societies in the European region were identified following an electronic search and sent an online survey of 14 questions to distribute among their member consultant/attending cardiac surgeons. RESULTS: The survey was completed by 91 consultant/attending cardiac surgeons across 12 European countries, with 78% indicating MV repair as their specialty area. 57.1% performed >150 operations/year and 71.4% had 10+ years of experience.Concomitant tricuspid valve repair is performed for moderate tricuspid regurgitation (TR) by 69% of surgeons and for mild TR by 26.3%, both with annular diameter >40 mm. 50.6% indicated ischaemic MV surgery in patients undergoing CABG if moderate mitral regurgitation with ERO >20 mm2 and regurgitant volume >30 mL, and 45.1% perform it if severe MR with ERO >40 mm2 and regurgitant volume >60 mL. For these patients the preferred management was: MVR if predictors of repair failure identified (47.2%) and downsizing annuloplasty ring only (34.1%).For atrial fibrillation (AF) in cardiac surgery, 34.1% perform ablation with biatrial lesion and 20% with left sided only. 62.6% perform concomitant Left Atrial Appendage (LAA) Occlusion irrespective of AF ablation with a left atrial clip. A wide variability in anticoagulation strategies for MV repair and bioprosthetic MV valve was reported both for patients in sinus rhythm and AF. CONCLUSION: These results demonstrate a variable practice for MV surgery, and a degree of lack of compliance with surgical intervention guidelines and anticoagulation strategy.
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BACKGROUND: Given the variety in mitral valve (MV) pathology and associated surgical techniques, extended reality (XR) holds great potential to assist MV surgeons. This review aims to systematically evaluate the currently available evidence investigating the use of XR and associated technologies in MV surgery. METHODS: A systematic database search was conducted of original articles and case reports that explored the use of XR and MV surgery in EMBASE, MEDLINE, Cochrane database and Google Scholar, from inception to February 2022. RESULTS: Our search yielded 171 articles, of which 15 studies were included in this review, featuring 328 patients. Two main areas of application were identified: (i) pre-operative planning and (ii) predicting post-operative outcomes. The articles reporting outcomes relating to pre-operative planning were further categorised as exploring themes relevant to (i) mitral annular assessment; (ii) training; (iii) evaluation of surgical technique; (iv) surgical approach or plan and (v) selecting ring size or type. Preoperatively, XR has been shown to evaluate mitral annular pathology more accurately than echocardiography, informing the surgeon about the optimal surgical technique, approach and plan for a particular patient's MV pathology. Furthermore, XR could simulate and aid ring size/type selection for MV annuloplasty, creating a personalized surgical plan. Additionally, XR could estimate the postoperative MV biomechanical and physiological characteristics, predicting and pre-empting post-operative complications. CONCLUSION: XR demonstrated promising applications for assisting MV surgery, enhancing outcomes and patient-centred care, nevertheless, there remain the need for randomized studies to ascertain its feasibility, safety, and validity in clinical practice.
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OBJECTIVES: Pediatric patients implanted with a durable ventricular assist device are initially managed in the pediatric cardiac ICU but are eligible for discharge to the ward. Our objectives were to characterize discharge and readmission of ventricular assist device patients to the pediatric cardiac ICU, identify risk factors for readmission, and determine whether discharge or readmission is associated with mortality. DESIGN: Retrospective study. SETTING: Stollery Children's Hospital. PATIENTS: Patients implanted with a durable ventricular assist device at less than 18 years old between 2005 and 2016. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: There were 44 patients who underwent ventricular assist device implantation at a median age of 3.7 years (interquartile range, 0.6-9.0 yr), with the most common etiology being noncongenital heart disease (76.7%). Median time of total ventricular assist device support was 110.0 days (interquartile range, 42.3-212.3 d) with the median index pediatric cardiac ICU stay being 34.0 days (interquartile range, 19.8-81.0 d). Thirty patients (68.0%) were discharged to the ward with 18 (60.0%) having at least one readmission. The median time to first readmission was 18.0 days (interquartile range, 14.8-109.8 d) with a median of two readmissions per patient (interquartile range, 1.0-3.0). The most common reason for readmission was pump thrombosis (30.4%), followed by neurologic dysfunction (23.9%). There were no statistically significant pre- or post-implant factors associated with readmission, and readmission was not associated with mortality (p = 0.600). Univariate Kaplan-Meier survival analysis indicated that use of pre-implant extracorporeal membrane oxygenation, post-implant continuous renal replacement therapy, as well as failure to be discharged from the index pediatric cardiac ICU stay were associated with mortality. CONCLUSIONS: Readmissions to the pediatric cardiac ICU occurred in 60.0% of pediatric patients on durable ventricular assist devices with the first readmission occurring within a month of discharge from the index pediatric cardiac ICU stay. While readmission was not associated with mortality, lack of discharge from index pediatric cardiac ICU stay was likely due to a worse pre-implant clinical status.
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Oxigenación por Membrana Extracorpórea , Corazón Auxiliar , Adolescente , Niño , Preescolar , Humanos , Lactante , Alta del Paciente , Readmisión del Paciente , Estudios RetrospectivosRESUMEN
INTRODUCTION: In the management of cloaca, there is concern that dissection of the urogenital sinus in early childhood with the aim of total anatomical correction is hazardous. Avoiding such mobilization and providing mitrofanoff channel, when needed, till peripubertal period reduces complications and is technically easier. MATERIALS AND METHODS: Forty-three cases of cloaca were managed in the period 2004-2016. Case records and radiology were reviewed retrospectively. The follow-up evaluation was done by looking into voiding history, bowel movements, and menstruation history. RESULTS: There were three groups of children, namely, those with no reconstruction done elsewhere except a diverting fecal stoma (Group I, n = 25), those who had undergone anorectal correction elsewhere with no attempt at urogenital reconstruction (Group IIA, n = 13), and those with attempted bowel and genitourinary reconstruction elsewhere (Group IIB, n = 5). The Group I children (one still awaiting reconstruction) underwent early rectal reconstruction followed by expectant management of the urogenital apparatus. The 18 referred cases had multiple problems, chiefly urogenital, of congenital or iatrogenic origin. While urinary reconstruction included bladder augmentation, ileal neobladder, bladder neck closure, and ureteric reimplantation, the foundation of urinary management was intermittent catheterization through mitrofanoff stoma and the avoidance of any dissection of the cloacal common channel. Surgery on the genital tracts included drainage of hydrocolpos, perineal surgery for low vaginae and abdominoperineal vaginoplasty for high vaginae in the peripubertal period with or without bowel supplementation. Spontaneous voiding was maintained in 17 of 25 (68%) Group I girls (including one death later from intestinal complications), 7 of 13 (54%), Group IIA girls, and 1 of 5 (20%) Group IIB girls. Painless menstruation was noted in eight postpubertal girls, three through the cloacal channel (awaiting reconstruction) and five through the reconstructed vagina. Most of the children are on a bowel management program for fecal cleanliness with washouts through the neoanus or Malone's stoma. CONCLUSION: We report a nonconventional approach to cloaca based on avoiding dissection of or around the common channel for urethrovaginal reconstruction, opting for mitrofanoff stoma for intermittent catheterization, when needed, and late vaginal reconstruction. We believe this approach has reduced the overall need for intermittent catheterization.
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OBJECTIVE: To compare the efficacy of topical voriconazole and topical natamycin with that of intrastromal voriconazole and topical natamycin in patients with recalcitrant fungal keratitis. DESIGN: Randomized clinical trial. PARTICIPANTS: Forty eyes of 40 patients with fungal keratitis (positive smear or culture results or both) larger than 2 mm, involving up to two thirds of the stromal depth, and not responding to topical natamycin therapy for 2 weeks were recruited. INTERVENTION: The patients were randomized to receive either topical 1% voriconazole therapy (n = 20) or intrastromal injections of voriconazole 50 µg/0.1 ml (n = 20). The patients in both groups continued topical natamycin 5% every 4 hours until the ulcer healed. MAIN OUTCOME MEASURES: Primary outcome measure was best spectacle-corrected visual acuity (BSCVA) 3 months after intervention, and secondary outcome measures were time to healing and the size of the scar. RESULTS: The patients in both groups had comparable baseline parameters. The mean BSCVA after treatment was 1.295 ± 0.5 logarithm of the minimum angle of resolution (logMAR) units in the topical group and 1.692 ± 0.29 logMAR units in the intrastromal group. The visual acuity after treatment was significantly better in the topical voriconazole group (P = 0.008). Nineteen patients receiving topical voriconazole and 16 patients who were given intrastromal voriconazole healed with therapy. CONCLUSIONS: Topical voriconazole seems to be a useful adjunct to natamycin in fungal keratitis not responding to topical natamycin. Intrastromal injections did not offer any beneficial effect over topical therapy.
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Infecciones Fúngicas del Ojo/tratamiento farmacológico , Queratitis/tratamiento farmacológico , Natamicina/administración & dosificación , Pirimidinas/administración & dosificación , Triazoles/administración & dosificación , Adulto , Antifúngicos/administración & dosificación , Sustancia Propia , Relación Dosis-Respuesta a Droga , Quimioterapia Combinada , Infecciones Fúngicas del Ojo/microbiología , Femenino , Estudios de Seguimiento , Humanos , Inyecciones , Queratitis/microbiología , Masculino , Persona de Mediana Edad , Soluciones Oftálmicas , Resultado del Tratamiento , VoriconazolRESUMEN
OBJECTIVE: Redo mitral valve surgery has traditionally been performed via a median sternotomy. It is often challenging and is associated with increased perioperative mortality. Advances in cardiac surgical techniques over the last two decades have led to an increase in the use of a minimally invasive approach via a right anterolateral mini-thoracotomy as opposed to a repeat median sternotomy. However, despite these advances, there is no general consensus on the best form of entry, and as of yet, there are no randomized controlled trials. We performed a meta-analysis of observational studies to aid in determining the best approach for redo mitral valve surgery. METHOD: The MEDLINE and EMBASE databases were conducted up until 1 June 2020. Data regarding mortality, stroke, reoperation for bleeding and length of hospital stay, wound infection and cardiopulmonary bypass time were extracted and submitted to a meta-analysis using random effects modelling and the I2-test for heterogeneity. Seven retrospective observational studies were included, enrolling a total of 1070 patients. RESULTS: There were a total of 1070 patients. Of these 364 had non-sternotomy approach compared with 707 patients who had median sternotomy. Further subgroup analysis revealed that 327 of the 364 patients had a mini-thoracotomy approach while the remaining 37 patients had a full thoracotomy approach. In-hospital mortality and length of stay were less in non-sternotomy group compared to median sternotomy group. There were no differences in stroke, CPB time and wound infections between the two groups. CONCLUSION: Redo mitral valve surgery can be performed safely with satisfactory outcomes via a mini-thoracotomy approach. This meta-analysis shows comparable results with reduced in-hospital mortality and hospital length of stay with a mini-thoracotomy approach.
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Enfermedades de las Válvulas Cardíacas , Esternotomía , Enfermedades de las Válvulas Cardíacas/cirugía , Humanos , Válvula Mitral/diagnóstico por imagen , Válvula Mitral/cirugía , Estudios Retrospectivos , Esternotomía/efectos adversos , Toracotomía/efectos adversosRESUMEN
PURPOSE: To determine the degree of stress in parents of children operated for intermediate anorectal malformations, and their quality of life (QOL) at follow-up. METHODS: Forty-two of the 166 children who had undergone a sacroperineal pullthrough operation for an intermediate type of anorectal malformation, between 1996 and 2005, in the department of paediatric surgery at Christian Medical College, Vellore, responded to follow-up. The psychosocial well-being of the parents and the QOL of the children were assessed by an independent observer. RESULTS: The main factor which aggravated the stress and caused dissatisfaction with the final outcome was fecal soiling. Mothers bore the brunt of the care of these children, with some help from the fathers and grandparents. The QOL was also significantly affected by soiling, and improvement in soiling resulted in a dramatic improvement in the QOL. CONCLUSION: Managing fecal soiling aggressively in the child with anorectal malformation, and providing social support to the family, are crucial for achieving a better QOL in these children and their families.
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Adaptación Psicológica , Colostomía/psicología , Familia/psicología , Relaciones Padres-Hijo , Calidad de Vida/psicología , Adolescente , Adulto , Malformaciones Anorrectales , Ano Imperforado/diagnóstico , Ano Imperforado/psicología , Ano Imperforado/cirugía , Niño , Preescolar , Colostomía/métodos , Padre/psicología , Femenino , Estudios de Seguimiento , Humanos , Masculino , Madres/psicología , Padres/psicología , Estudios Retrospectivos , Índice de Severidad de la Enfermedad , Factores de Tiempo , Adulto JovenRESUMEN
Distribution and ecological risks of Phthalic acid esters (PAEs) are poorly studied in estuarine environments in India. An attempt is made to chart the sources and assess the ecological risk of six PAE congeners (∑6PAEs), present in dissolved and particulate forms in a tropical ecosystem (Cochin Estuary, India). Terrestrial input, as attested by a clear seasonality with substantial enrichment during monsoon (2-28 µg/L and 31-1203 µg/g; dissolved and particulate PAEs respectively) and post-monsoon (1-7 µg/L and 7-321 µg/g; dissolved and particulate PAEs respectively), was identified as the primary source. DnBP (di-n-butyl phthalate) and DEHP (diethylhexyl phthalate) were found to be the dominant species except for dissolved PAEs at pre-monsoon season. Statistical analysis identified two major clusters, in the ∑6PAEs, composed of medium to high molecular weight PAEs (derived from plastic products) and low molecular weight PAEs (derived from cosmetic products). Calculated Risk Quotient (RQ) indicated values indicated moderate to high ecological risk for DnBP and DEHP congeners is a grim pointer to their detrimental effects on human health through consumption of contaminated organisms. Although substantial enrichment of suspended matter gets flushed out of the estuary during monsoon, there is a net PAE accumulation in the estuary during post-monsoon following an increased sedimentary restitution. Data of PAEs generated herein raises a challenge for immediate enactment of statutory legislation to curb and regulate hazardous contamination of estuaries by phthalic acid esters.
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Estuarios , Ácidos Ftálicos/análisis , China , Dibutil Ftalato/análisis , Ecosistema , Ésteres/análisis , Humanos , IndiaRESUMEN
To facilitate the assessment of the safety profile of rotavirus vaccines effectively, baseline data on intussusception are important for comparison with intussusception rates following the introduction of vaccine. The aim of the study was to describe epidemiological and clinical features of intussusception in children aged less than five years in an Indian medical facility. Hospital data on intussusception for children discharged during 1 January 2001-30 June 2004 from the Christian Medical College Hospital, Vellore, India, were reviewed. Relevant information was extracted from medical records to classify cases according to the criteria of the Brighton Collaboration Intussusception Working Group. Complete review of medical records for clinical and demographic information was only performed for those cases fulfilling level 1 diagnostic certainty (definite intussusception) (Study ID 101245). During the surveillance period, 31 infants and children with definite intussusception were identified. The majority (61.2%) of the cases occurred in the first year of life. The male : female ratio was 3.4 : 1. Intussusception cases occurred round the year with no distinct seasonality. No intussusception-associated death was recorded. This study provides baseline data on intussusception in South India. Cases identified in the study were similar in presentation and demographics as those observed in other Asian settings. Prospective surveillance systems, using standardized case definitions will further increase the understanding of the aetiology and epidemiology of intussusception, especially as new rotavirus vaccines are made available.
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Intususcepción/epidemiología , Preescolar , Femenino , Humanos , Incidencia , India/epidemiología , Lactante , Intususcepción/diagnóstico , Intususcepción/mortalidad , Masculino , Estudios Retrospectivos , Vacunas contra Rotavirus/efectos adversos , Factores SexualesRESUMEN
AIM: Cervicovaginal or vaginal agenesis with functioning endometrial tissue is rare. We report the construction of a colon conduit which is anastomosed to posterior uterine wall or upper vaginal pouch to allow menstruation. MATERIALS AND METHODS: We report seven girls with cervicovaginal agenesis and four with lower vaginal agenesis (aged 12-20 years) who presented with painful cryptomenorrheoa. All the girls wanted to conserve their uterus and menstruate normally. A colon conduit was constructed for the egress of menstrual blood. The colon conduit was anastomosed to the posterior uterine wall in the seven girls with cervicovaginal agenesis and to the distended upper vaginal pouch in the four girls with vaginal agenesis. Utero-colonic neovaginal anastomosis was performed only after excising a circular portion of the posterior myometrium to prevent stenosis. RESULTS: The colon conduit functioned effectively, providing an egress for regular painless menstruation. One patient had stenosis of the perineal neovaginal orifice for which dilations were done. One girl has married and reports satisfactory intercourse. The mean follow up is 2.2 years. CONCLUSIONS: This group of patients forms a separate subgroup needing a conduit not only for sexual function but also for menstruation. However, if treated by the method described herein, they should be cautioned against pregnancy if they have cervicovaginal agenesis and against vaginal delivery if they have vaginal agenesis.
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Cuello del Útero/anomalías , Colon/trasplante , Vagina/anomalías , Adolescente , Anastomosis Quirúrgica , Cuello del Útero/cirugía , Coito , Colon/cirugía , Femenino , Humanos , Menstruación , Procedimientos de Cirugía Plástica/métodos , Recuperación de la Función , Vagina/cirugía , Adulto JovenRESUMEN
AIM: To review cases of congenital ureteric stenosis treated in the period between 1999 and 2007. We propose to analyze the type of presentation, management and results. MATERIAL AND METHODS: We report 17 children aged 20 days to 8 years with obstructive uropathy due to congenital stenosis of the ureter at one or more levels. This condition could be mistaken for the more common pelviureteric junction obstruction (PUJO) or primary megaureter, but it is a distinct and more serious anomaly. 13 of the 17 children had one or more associated anomalies, the most significant of which was a contralateral multicystic dysplastic kidney. Other associated anomalies included PUJO, megacalyx, vesicoureteric reflux, urogenital sinus, duplicate vagina, anorectal malformation and agenesis of the bladder. 16 children were symptomatic at presentation, with uremia (serum creatinine >1 mg/dl) in 5, while 1 was diagnosed antenatally. The correct preoperative diagnosis was made in only three children. Reconstruction included ureteroureteral anastomosis, ureteric reimplantation or ureteral substitution. RESULTS: There is follow up for 15 of the 17 patients. Length of follow up ranges from 1 to 7 years (average 2.7 years). There was satisfactory urinary drainage established in all 17 cases and uremia has resolved 3 of the 5 children. The children with solitary functioning kidney are at risk of uremia in later life. CONCLUSION: Congenital ureteric stenosis is a rare condition, but distinct anomaly with possible grave consequence and has been distinguished from other causes of congenital ureteric obstruction.
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Obstrucción Ureteral/diagnóstico , Obstrucción Ureteral/cirugía , Anomalías Múltiples , Niño , Preescolar , Constricción Patológica/congénito , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Obstrucción Ureteral/etiologíaRESUMEN
Artistic creativity can emerge in patients with Parkinson's disease. Here we describe two patients who had creative awakening while on levodopa treatment for Parkinson's disease and discuss its implications.
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Creatividad , Dopaminérgicos/farmacología , Levodopa/farmacología , Enfermedad de Parkinson/tratamiento farmacológico , Anciano , Humanos , Masculino , Persona de Mediana Edad , Investigación CualitativaRESUMEN
Phthalic acid esters (PAEs) are a group of endocrine-disrupting chemicals listed as priority pollutants by United States Environmental Protection Agency (USEPA, 2009). This study provides baseline information on seasonal distribution and contamination status of six phthalic acid esters (∑6PAEs) in sediments of a tropical estuary (Cochin-India). In general, the sediments accumulated more PAEs during the post monsoon (mean 2325 ngg-1; between 1402 and 3121 ngg-1) and monsoon (mean 1372 ngg-1; between 331 and 4015 ngg-1) periods indicating land run off as the major transport pathway. Moderate run off and comparatively high residence time lead to effective sorption and settling of PAEs in the surface sediments during post monsoon season. Despite a high discharge of PAEs in to the water column, their deposition on to the sediments occurs at a lower rate during monsoon than that post monsoon season. PAEs were (mean 810 ngg-1; between 44 and 1722 ngg-1) lowest in pre monsoon season. The pre monsoon season is characterized by a minimal runoff consequent to the trapping of these organic pollutants in the river catchment area. The mid and high molecular PAEs (DEHP-Di ethylhexyl phthalate, BBP-Benzyl butyl phthalate and DnBP-Di-n-butyl phthalate) were the dominant congeners relative to the low molecular weight congeners (DMP-Dimethyl phthalate and DEP-Diethyl phthalate). DEHP and BBP levels exceeded permissible risk levels indicating a serious ecological hazard to the estuarine ecosystem.
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Contaminación de Medicamentos , Monitoreo del Ambiente/métodos , Ésteres/análisis , Estuarios , Sedimentos Geológicos/análisis , Ácidos Ftálicos/análisis , Estaciones del Año , IndiaRESUMEN
Methods@#Obese patients (body mass index [BMI] ≥30.0 kg/m2) who underwent single-level MIS TLIF or ALIF at L5/S1 were included in the study. Demographic/perioperative variables, presenting patient pathology, and 1-year arthrodesis statistics were collected. PROM scores for Visual Analog Scale (VAS) back/leg, Oswestry Disability Index, 12-item Short Form Physical Composite Scale, and Patient-Reported Outcome Measurement Information System Physical Function (PROMIS-PF) were collected from preoperative and postoperative (6 weeks, 12 weeks, 6 months, 1 year, 2 years) PROMIS-PF. The obese patients were classified based on the procedure they underwent (MIS TLIF vs. ALIF). @*Results@#The criteria were met by 210 patients in total. After coarsened exact matching for Charlson comorbidity index score, degenerative spondylolisthesis, isthmic spondylolisthesis, degenerative scoliosis, foraminal stenosis, insurance, male, and ethnicity, 94 obese patients were included in the total cohort, with 59 receiving MIS TLIF and 35 receiving ALIF. ALIF recipients had higher PROMIS-PF scores at 6 weeks (p=0.014) and 12 weeks (p=0.030), as well as a higher VAS leg at 2 years (p=0.017). Following multiple regression accounting for differences in baseline BMI, only the 6-week PROMIS-PF significantly differed (p=0.028), with no other intergroup differences in mean PROMs between fusion types. Aside from a significantly higher 6-week MCID achievement rate for PROMIS-PF among ALIF recipients (p=0.006), no differences in attainment were observed. @*Conclusions@#There were no statistically significant differences in perioperative characteristics, fusion rates, PROMs, or MCID achievement between obese patients receiving MIS TLIF vs. ALIF. As a result, our findings indicate that MIS TLIF and ALIF at L5/S1 are equally effective in an obese patient population.
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Methods@#WC recipients undergoing single-level MIS TLIF were identified. PROMs of Visual Analog Scale (VAS) for back and leg pain, Oswestry Disability Index (ODI), 12-item Short Form Physical and Mental Composite Scale (SF-12 PCS/MCS), and Patient-Reported Outcomes Measurement Information System Physical Function evaluated subjects preoperatively/postoperatively. Subjects were grouped according to preoperative SF-12 MCS: <41 vs. ≥41. Demographic/perioperative variables, PROMs, and MCID were compared using inferential statistics. Multiple regression was used to account for differences in spinal pathology. @*Results@#The SF-12 MCS <41 and SF-12 MCS ≥41 groups included 48 and 45 patients, respectively. Significant differences in ΔPROMs were observed at SF-12 MCS at all timepoints, except at 6 months (p≤0.041, all). The SF-12 MCS <41 group had worse preoperative to 6-months SF-12 MCS, 12-weeks/6-months VAS back, 12-week VAS leg, and preoperative to 6-months ODI (p≤0.029, all). The SF-12 MCS <41 group had greater MCID achievement for overall ODI and 6-weeks/1-year/overall SF-12 MCS (p≤0.043, all); the SF-12 MCS ≥41 group had greater attainment for 6-month VAS back (p=0.004). @*Conclusions@#Poorer mental functioning adversely affected the baseline and intermediate postoperative quality-of-life outcomes pertaining to mental health, back pain, and disability among WC recipients undergoing lumbar fusion. However, outcomes did not differ 1–2 years after surgery. While MCID achievement for pain and physical function was largely unaffected by preoperative mental health score, WC recipients with poorer baseline mental health demonstrated higher rates of overall clinically meaningful improvements for disability and mental health
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Three siblings (one boy and two girls) with Brown-Vialetto-van Laere syndrome are reported. A peculiar feature of onset with hearing loss in a patient with multiple cranial nerve palsies and a positive family history suggests this diagnosis. In our family, an autosomal recessive mode of inheritance was seen. In addition, we observed that early onset was associated with rapid deterioration and death. Optic nerve involvement and hyperintensity of the brainstem nuclei on magnetic resonance imaging (MRI) are two new features described in this report.
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Tronco Encefálico/patología , Parálisis Bulbar Progresiva/patología , Pérdida Auditiva Sensorineural/patología , Nervio Óptico/patología , Adolescente , Niño , Femenino , Humanos , Imagen por Resonancia Magnética , Masculino , Linaje , SíndromeRESUMEN
We report a case of pulmonary arteriovenous malformation (PAVM) and associated pulmonary tuberculosis in a young girl who presented with hemoptysis. As intra-arterial coil embolization did not result in improvement of symptoms, surgical resection of the PAVM was performed, resulting in dramatic clinical improvement. Histopathology revealed a large PAVM, and in addition, caseous necrotizing granulomatous inflammation, suggestive of pulmonary tuberculosis. This case is being reported since the association of large PAVM and tuberculosis is very rare, and both could present with hemoptysis.