Aural tuberculosis at the start of the 21st century. Literature review according to SWiM guidelines. Part 2: Treatment

ObjectivesSystematic review of the scientific literature dedicated to treatment modalities and results for aural tuberculosis published since the start of the 21^st century.Material and methodsSearch of the Medline, Cochrane and Embase databases for the period 2000 - 2020. Selection of articles in English, French and Spanish devoted to clinical cases and series documenting treatment of auricular tuberculosis. Extraction of data on pre-established files documenting treatment modalities and results. Reading of articles by two authors. Analysis performed according to SWiM guidelines, evaluating cure, tuberculosis-related death, treatment-related complications, improvement in facial palsy, and hearing sequelae rates.ResultsOne hundred and twenty eight articles: 118 case reports (159 patients) and 10 cohorts (177 patients) from 42 countries were analyzed. Female/male sex ratio was 1.2 with ages ranging from 1 month to 87 years. Medical treatment consisted in 5 to 24 months’ antitubercular antibiotic treatment using 2 to 8 antibiotics. Mastoidectomy, tympanoplasty and facial nerve decompression were associated to medical treatment in 64.7%, 17.4% and 6.2% of cases, respectively. Overall rates of cure, death, treatment-related complications, facial sequelae and hearing sequelae were 96.8%, 2%, 9.5%, 35.8% and 75.5%. In case reports, BCG vaccination did not appear to protect against facial palsy and severe intracranial complications (P > 0.6). There was no significant correlation (P > 0.3) between death and the clinical variables tested, and facial nerve decompression did not appear to influence outcome for facial function (P = 0.4).ConclusionMedical treatment is very effective but not without risk of death, complications and sequelae. It is the same as for pulmonary tuberculosis. Indications for and benefit of major auricular surgery during medical treatment deserve further studies.     

Assessing the Effects of Geniculate Artery Embolization in a Nonsurgical Animal Model of Osteoarthritis

PurposeTo create a nonsurgical animal model of osteoarthritis (OA) to evaluate the effects of embolotherapy during geniculate artery embolization (GAE).Materials and MethodsFluoroscopy-guided injections of 700 mg of sodium monoiodoacetate were performed into the left stifle in 6 rams. Kinematic data were collected before and after induction. At 10 weeks after induction, Subjects 1 and 4–6 underwent magnetic resonance (MR) imaging with dynamic contrast enhancement (DCE) and Subjects 1, 3, and 4–6 underwent angiography with angiographic scoring to identify regions with greatest disease severity for superselective embolization (75–250-μm microspheres). Target vessel size was measured. At 24 weeks after angiography, DCE-MR imaging, angiography, and euthanasia were performed, and bilateral stifles were harvested. Medial/lateral tibial and femoral condylar, patellar, and synovial samples were cut, preserved, decalcified, and scored using the Osteoarthritis Research Society International criteria. The stifle and synovium Whole-Organ Magnetic Resonance Imaging Score and Multicenter Osteoarthritis Study score were determined. The volume transfer constant (K_trans) and extracellular volume fraction (v_e) were calculated from DCE-MR imaging along the lateral synovial regions of interest.ResultsThe mean gross and microscopic pathological scores were elevated at 38 and 61, respectively. Mean synovitis score was elevated at 9.2. Mean pre-embolization and postembolization angiographic scores were 5 and 3.8, respectively. Mean superior, transverse, and inferior geniculate artery diameters were 3.1 mm ± 1.21, 2.0 mm ± 0.50, and 1.6 mm ± 0.41 mm, respectively. Mean pre-embolization and postembolization cartilage and synovitis scores were elevated at 35.13 and 73.3 and 5.5 and 9.2, respectively. The K_trans/v_e values of Subjects 4, 5, and 6 were elevated at 0.049/0.38, 0.074/0.53, and 0.065/0.51, respectively. Altered gait of the hind limb was observed in all subjects after induction, with reduced joint mobility. No skin necrosis or osteonecrosis was observed.ConclusionsA nonsurgical ovine animal knee OA model was created, which allowed the collection of angiographic, histopathological, MR imaging, and kinematic data to study the effects of GAE.     

Reliability and validity of the gait classification system in children with cerebral palsy (GCS-CP)

BackgroundGait classification systems (GCS) may enable clinicians to differentiate gait patterns into clinically significant categories that assist in clinical decision-making and assessment of outcomes. Davids and Bagley in 2014 [1] described a GCS for children with cerebral palsy (GCS-CP). The purpose of our study was to use the GCS-CP for the first time on a sample of patients with CP and to evaluate the reliability and utility of the classification system.MethodsThe gait of 131 children with CP was retrospectively reviewed and classified according to Davids and Bagley’s classification using two-dimensional (2D) video and three-dimensional (3D) lower limb kinematics and kinetics. Gross Motor Function Classification System (GMFCS) levels were determined, and the Gait Profile Scores (GPS) calculated to characterize the sample concerning gait classification. The comparison between the groups was performed using the Kruskal-Wallis test with respect to the non-normal distribution of the data. The intrarater and interrater reliability was determined using the Kappa index (k) statistics with 95% CI.ResultsAll GCS-CP groups were represented within the evaluated sample. Of the 131 cases evaluated, 127 (96.95%) were able to be classified with respect to sagittal plane stance phase gait deviations. All patients in the sample were able to be classified with respect to sagittal plane swing phase and transverse plane gait deviations. The interrater reliability was 0.596 and 0.485 for the first and second levels of the classification, respectively, according to the Fleiss’s Kappa statistics. Intrarater reliability was 0.776 and 0.714 for the raters one and two, respectively, according to the Cohen’s Kappa statistics.SignificanceThe GCS-CP exhibited clinical utility, successfully classifying almost all subjects with CP in two planes, based upon kinematic and kinetic data. The classification is valid and has moderate interrater and moderate to substantial intrarater reliability.     

Reliability and validity of the Turkish version of Fullerton Advanced Balance Scale in cerebral palsy

BackgroundThe Fullerton Advanced Balance Scale (FAB) is a multi-item balance assessment test designed to measure balance in relatively higher functioning individuals. The aim of this study was to examine the reliability and validity of the Turkish version of the FAB (FAB-T) in children with cerebral palsy (CP).Research questionIs the Turkish version of the Fullerton Advance Balance Scale valid and reliable in determining balance problems in children with cerebral palsy and determining the underlying cause of this condition?MethodsForty-six children with CP participated in this study. Rasch analysis was used to investigate item adherence. Internal consistency of the FAB-T was established using Cronbach's alpha coefficient. Test-retest reliability was also evaluated. In addition, to assess concurrent validity, FAB-T scores were compared with the Pediatric Balance Scale (PBS) using the Spearman correlation coefficient.ResultsThe FAB-T showed satisfactory internal consistency (Cronbach's alpha value=0.94) and excellent test-retest reliability (ICC=0.99). The FAB and the PBS exhibited concurrent positive validity (r = 0.913; p < 0.001). All items of the FAB-T were found to fit the Rasch Model (Chi-square 16.01(df=20), p = 0.716).SignificanceThe FAB-T is a reliable and valid tool that can be used to measure balance skills and to identify the source of the problem in children with CP.     

脑瘫患儿发生癫痫持续状态的急救护理体会

目的对脑瘫患儿发生癫痫持续状态的急救护理方法和效果进行探讨。方法选择2018年4月—2018年9月在医院收治的50例脑瘫发生癫痫持续状态的患儿作为对照组,对其实施常规急救护理,同时选择2018年10月—2019年3月在医院收治的50例脑瘫发生癫痫持续状态的患儿作为观察组,对其实施强化急救护理,对比两组护理满意度、生命体征评分、护理有效率。结果观察组的总体满意率、护理有效率、生命体征评分均优于对照组,两组差异明显,有统计学意义,P<0.05。结论对脑瘫发生癫痫持续状态的患儿实施强化急救护理能够提升治护效果,提高患儿家长满意度,更好的改善患儿生命体征。     

作业治疗对不随意运动型脑瘫手眼协调能力的影响

目的:探讨作业治疗对不随意运动型脑瘫患儿手眼协调能力的临床疗效。方法:选取2017年5月~2018年9月在广州中医药大学附属南海妇产儿童医院儿童康复科就诊的不随意运动型脑瘫患儿30例,进行作业治疗手眼协调训练,采用Peabody精细运动发育量表(PDMS-FM)及FMFM(手眼协调E项)评定临床疗效。结果:患儿FMQ评分、FMFM(E区)评分随治疗时间推移呈逐渐增加趋势,第二次评估与第一次评估比较有统计学意义(P<0.05),第三次评估和第二次评估有统计学意义(P<0.01)。结论:作业治疗手眼协调训练对不随意运动型脑瘫精细运动及手技巧有明显改善作用。     

The effects of dual tasks on gait in children with cerebral palsy

AimTo assess the gait and cognitive performances of children with cerebral palsy (CP) during dual tasks (DT) in comparison to typically developing (TD) children.MethodThis prospective, observational, case-control study included 18 children with CP (7 girls, 11 boys; median age 12 [10:13] years and 19 controls (9 girls, 10 boys; median age 12 [10:13y6mo] years). Performances were recorded during a simple walking task, 5 DT (walking + cognitive tasks with increasing cognitive load), and 5 simple cognitive tasks (while sitting). Gait parameters were computed using an optoelectronic system during walking tasks. Six parameters were selected for analysis by a principal component analysis. Cognitive performance was measured for each cognitive task. The dual-task cost (DTC) was calculated for each DT.ResultsGait performance decreased in both groups as DT cognitive load increased (e.g., walking speed normalized by leg length, in simple task: 1.25 [1.15:1.46] s⁻¹ for CP, 1.53 [1.38:1.62] s⁻¹ for TD; DT with highest load: 0.64 [0.53:0.80] s⁻¹ for CP, 0.95 [0.75:1.08] s⁻¹ for TD). The CP group performed significantly worse than TD group in every task (including the simple task), but DTC were similar in both groups. A task effect was found for the majority of the gait parameters.InterpretationThe reduced gait performance induced by DT may generate underestimated difficulties for children with CP in daily-life situations, where DT are common. This should be considered in clinical assessments.     

Superior trans‐septal approach for minimally invasive mitral valve surgery via right small thoracotomy

Minimally invasive mitral valve surgery (MIMVS), despite its challenges, is not a rare procedure. However, MIMVS via a right small thoracotomy must be performed using long‐shafted surgical instruments and thoracotomy instruments specialized for minimally invasive cardiac surgeries. We have performed 12 cases of MIMVS via right small thoracotomy using the superior trans‐septal approach and secured a surgical visual field that easily allows a finger to reach the mitral valve annulus without using special instruments for minimally invasive cardiac surgery. We named this technique the “drawer‐case technique.” In conclusion, MIMVS via right thoracotomy using the superior trans‐septal approach can be performed easily and safely, similar to mitral valve surgery performed via median sternotomy.     

Flavin adenine dinucleotide synthase deficiency due to FLAD1 mutation presenting as multiple acyl-CoA dehydrogenation deficiency-like disease: A case report

Multiple acyl-CoA dehydrogenase deficiency (MADD), also known as glutaric acidemia type II, is classically caused by a congenital defect in electron transfer flavoprotein (ETF) or ETF dehydrogenase (ETFDH). Flavin adenine dinucleotide synthase (FADS) deficiency caused by mutations in FLAD1 was recently reported as a novel riboflavin metabolism disorder resembling MADD. Here, we describe a Japanese boy with FADS deficiency due to a novel mutation (p.R249*) in FLAD1. In the asymptomatic male infant born at full term, newborn screening showed positive results with elevated C5 and C14:1 acylcarnitine levels and an increased C14:1/C2 ratio. Biochemical studies were unremarkable except for lactic acidosis (pH 7.197, lactate 61 mg/dL). A diagnosis of MADD was suspected because of mild abnormalities of the acylcarnitine profile and apparent abnormalities of urinary organic acids, although mutations in the ETFA, ETFB, ETFDH, and riboflavin transporter genes (SLC52A1, SLC52A2, and SLC52A3) were not detected. Administration of riboflavin and L-carnitine was initiated at one month of age based on the diagnosis of “biochemical MADD” despite a lack of symptoms. Nevertheless, the acylcarnitine profile was not normalized. Symptoms resembling bulbar palsy, such as vocal cord paralysis and dyspnea with stridor, were present from 3 months of age. At 4 months of age, he became bedridden because of hypoxic-ischemic encephalopathy due to fulminant respiratory failure with aspiration pneumonia. At 2 years and 5 months of age, a homozygous c.745C > T (p.R249*) mutation in the FLAD1 gene was identified, confirming the diagnosis of FADS deficiency. His severe clinical course may be caused by this nonsense mutation associated with poor responsiveness to riboflavin. Persistent lactic acidosis and neuropathy, such as bulbar palsy, may be important for diagnosing FADS deficiency. Although the biochemical findings in FADS deficiency are similar to those in MADD, their clinical symptoms and severity may not be identical.