Clubfoot: Congenital Talipes Equinovarus.

Congenital talipes equinovarus (CTEV), also known as clubfoot, is a common musculoskeletal entity that affects one to two per 1000 live births worldwide. Imaging modalities including radiography, US, and MRI have emerged as valuable tools for the diagnosis, treatment, and monitoring of CTEV. The deformity is characterized by midfoot cavus, forefoot adductus, and hindfoot varus and equinus. The Ponseti method of manipulation and serial casting is the standard treatment of CTEV. Radiography shows the anatomy, position, and relationships of the different bones of the foot. US allows accurate assessment of cartilaginous and bony structures, in addition to its inherent advantages such as absence of ionizing radiation exposure. One of the indications for US is to monitor the response to Ponseti method treatment. MRI enables visualization of bones, cartilage, and soft tissues and allows multiplanar evaluation of deformities, providing a comprehensive imaging analysis of CTEV. An integrated approach that combines clinical examination and imaging findings is essential for effective management of CTEV. The authors provide a comprehensive overview of CTEV with a review of imaging modalities to help evaluate CTEV, focusing on radiography, US, and MRI. Using this article as a guide, radiologists involved in the assessment and treatment of CTEV can contribute to the management of the condition. ©RSNA, 2024 Supplemental material is available for this article.

Botulinum toxin type A in the treatment of lower limb spasticity in children with cerebral palsy.

BACKGROUND: Cerebral palsy (CP) is the most common cause of physical disabilities in children in high-income countries. Spasticity is the most common motor disturbance in CP. Botulinum toxin type A (BoNT-A) is considered the first-line treatment for focal spasticity in people with CP. OBJECTIVES: To evaluate the effectiveness and safety of BoNT-A compared to other treatments used in the management of lower limb spasticity in children with CP. SEARCH METHODS: We searched CENTRAL, PubMed, four other databases, and two trial registers in October 2018. We also searched the reference lists of relevant studies and reviews and contacted experts in the field. We did not apply any date or language restrictions. SELECTION CRITERIA: Randomised controlled trials of children with CP, aged between birth and 19 years, treated with BoNT-A injections in the lower limb muscles compared to other interventions. The primary outcomes were gait analysis and function. The secondary outcomes were joint range of motion, quality of life, satisfaction, spasticity, and adverse events. DATA COLLECTION AND ANALYSIS: Two review authors independently selected studies, extracted data, assessed risk of bias, and rated the quality of the evidence using GRADE. A third review author arbitrated in case of disagreements. We conducted meta-analyses of available data whenever possible, analysing dichotomous data with risk ratios (RR), and continuous data with mean differences (MD) or standardised mean differences (SMD), with 95% confidence intervals (CI). We considered a 5% significance level for all analyses.Whenever possible, we analysed outcomes at the time points at which they were assessed: short term (2 to 8 weeks); medium term (12 to 16 weeks); and long term (> 24 weeks). MAIN RESULTS: We included 31 randomised controlled trials assessing 1508 participants. Most studies included ambulatory patients with more than one motor type of CP, and with a mean age of between three and seven years. There was a slight predominance of males.Studies compared BoNT-A in the lower limb muscles to usual care or physiotherapy (14 studies), placebo or sham (12 studies), serial casting (4 studies), or orthoses (1 study).We rated studies as at high or unclear risk of bias mainly due to random sequence generation, allocation concealment, blinding of participants and personnel, and blinding of outcome assessment.BoNT-A versus usual care or physiotherapyBoNT-A might improve overall gait scores at medium-term follow-up (MD 2.80, 95% CI 1.55 to 4.05; 1 study, 40 children; very low-quality evidence) and is moderately effective at improving function at short-term (SMD 0.59, 95% CI 0.23 to 0.95; 2 studies, 123 children) and medium-term (SMD 1.04, 95% CI 0.16 to 1.91; 4 studies, 191 children) follow-up (all very low-quality evidence).BoNT-A improves ankle range of motion, satisfaction, and ankle plantarflexors spasticity at one or more time points (very low-quality evidence).The proportion of adverse events in the BoNT-A group was 0.37 (95% CI 0.08 to 0.66; I2 = 95%; very low-quality evidence). No adverse events were reported in the control group.BoNT-A versus placebo or shamBoNT-A improves overall gait scores at short-term (RR 1.66, 95% CI 1.16 to 2.37, P = 0.006; 4 studies, 261 assessments) and medium-term (RR 1.90, 95% CI 1.32 to 2.74, P < 0.001; 3 studies, 248 assessments) follow-up, and may improve peak ankle dorsiflexion in stance (MD 15.90 degrees, 95% CI 4.87 to 26.93, P = 0.005; 1 study, 19 children) and in swing (MD 10.20 degrees, 95% CI 4.01 to 16.39, P = 0.001; 1 study, 19 children) at short-term follow-up (all moderate-quality evidence).BoNT-A is not more effective than placebo or sham at improving function at short-term (SMD 0.24, 95% CI -0.35 to 0.83, P = 0.42; 4 studies, 305 children) or long-term (SMD -0.07, 95% CI -0.48 to 0.35, P = 0.76; 2 studies, 91 children) follow-up, but has a small positive effect at medium-term follow-up (SMD 0.28, 95% CI 0.06 to 0.49, P = 0.01; 5 studies, 327 children) (all moderate-quality evidence).BoNT-A improves passive ankle range of motion, satisfaction, and ankle plantarflexors spasticity at one or more time points (moderate-quality evidence).There was no difference between groups in the rate of adverse events at short-term follow-up (RR 1.29, 95% CI 0.87 to 1.93, P = 0.21; 12 studies, 918 children; moderate-quality evidence).BoNT-A versus serial castingThere was no difference between groups for overall gait scores at short-term (MD 0.00, 95% CI -1.66 to 1.66); medium-term (MD 0.65, 95% CI -1.21 to 2.51); or long-term (MD 0.46, 95% CI -1.33 to 2.25) follow-up in one study with 18 children (moderate-quality evidence).BoNT-A improved instrumented gait analysis only in terms of ankle dorsiflexion at initial contact (MD 6.59 degrees, 95% CI 1.39 to 11.78, P = 0.01; 2 studies, 47 children). There was no difference between groups for peak ankle dorsiflexion in stance and swing, and gait speed at any time point (moderate- and low-quality evidence).BoNT-A is not more effective than serial casting at improving function, ankle range of motion, and spasticity at any time point (moderate- and low-quality evidence).BoNT-A is not associated with a higher risk of adverse events than serial casting (RR 0.59, 95% CI 0.03 to 11.03; 3 studies, 64 children; low-quality evidence).BoNT-A versus orthosesThere was no difference between groups for function at medium-term follow-up (MD 11.14, 95% CI -0.05 to 22.33; 1 study, 43 children), but BoNT-A is more effective than orthoses at improving hip range of motion and hip adductors spasticity (all very low-quality evidence). AUTHORS' CONCLUSIONS: The quality of the evidence was low or very low for most of the outcomes analysed. We found limited evidence that BoNT-A is more effective than placebo or a non-placebo control at improving gait, joint range of motion, satisfaction, and lower limb spasticity in children with CP, whereas the results for function were contradictory. The rate of adverse events with BoNT-A is similar to placebo. BoNT-A is not more effective than ankle serial casting to treat ankle contractures for any of the assessed outcomes, but is more effective than orthotics at improving range of motion and spasticity.

Polyarticular lipoma arborescens--a clinical and aesthetical case.

Lipoma arborescens is a benign tumor, but it may be a reactive process to other disorders, and its clinical, analytical, radiological and ultrasound presentation may be redundant to any synovial tumor. Despite the characteristic feature on magnetic resonance imaging (MRI), the correct differential diagnosis in atypical presentation, and the need for timely removal of the lesion to prevent joint damage, forces, ultimately, to invasive procedures. The clinical case reported here, fourth described in English language publications on the polyarticular form, also presented other specificities related to one of the swellings, in the knee. Because of its atypical location in the popliteal fossa, recurrent episodes of joint effusion, personal history of knee trauma, pulmonary tuberculosis, and family history of rheumatoid arthritis required particular attention. This process was hampered by the refusal of knee (and ankle) surgery by the patient. He accepted surgical removal of the swellings of the wrists, for aesthetical reasons, with pathologic confirmation of the diagnosis, and clinical success in that location. MRI of the knee showed the typical image of lipoma arborescens, but also other changes that compromise the prognosis.

Resources and strategies for the optimal care of patients with axial spondyloarthritis: The CREA project.

BACKGROUND AND OBJECTIVE: Axial spondyloarthritis (axSpA) are musculoskeletal diseases with different manifestations. In clinical practice, variability, and limitations in the collection of the outcomes required for follow-up have been observed. The objective of the CREA project was to agree on improvement strategies for the initial assessment and follow-up of patients with axSpA in Spain. MATERIALS AND METHODS: A survey with 33 questions was conducted by a representative sample of rheumatologists on clinical practice, resources, and present limitations in the follow-up of patients with axSpA. The results of the survey were discussed in 10 regional meetings, and 105 strategies were proposed and evaluated through a Delphi consensus in which 85 experts participated. RESULTS: The lack of time for clinical visits, the lack of nurses and/or support staff and the delay in performing the imaging tests were the most prominent limitations in the follow-up of patients with axSpA. One hundred and five strategies were proposed related to the evaluation of disease activity, physical function, quality of life and disease impact, to the evaluation of comorbidities and extra-articular manifestations, laboratory tests; imaging tests, physical examination and metrology. Of the total, 85 were considered highly advisable. No regional differences were found. CONCLUSIONS: The proposals agreed upon as highly advisable in the present study are applicable to the entire national territory, allow tighter and more homogeneous monitoring of the patients with axSpA, facilitate more comprehensive management of the disease, and respond to the unmet needs detected in the initial survey.

Strategies and resources to optimise the management of Psoriatic Arthritis patients: The CREA Project.

BACKGROUND AND AIM: Psoriatic arthritis (PsA) is a chronic immune-mediated inflammatory disease that affects the musculoskeletal system and skin, and manifests heterogeneously, with a variable course. In current clinical practice, variability and limitations in its follow-up have been observed. The aim of the CREA project was to agree on strategies to improve the initial assessment and follow-up of patients with PsA in Spain. MATERIALS AND METHODS: A survey was conducted among a representative sample of expert rheumatologists in Spain, containing 33 questions on current clinical practice, available resources, and current limitations in the follow-up of patients with PsA. The results were discussed in regional meetings and 105 strategies were proposed and finally evaluated by 85 experts in a Delphi consensus. RESULTS: The most important limitations in the follow-up of PsA were lack of consultation time, lack of nursing staff, and delays in performing imaging tests. A total of 108 strategies were proposed related to the assessment of quality of life and disease-impact indices; comorbidities and extra-articular manifestations; laboratory tests; imaging tests; physical examination and metrology; and activity and function indices. Of the total, 53 were considered highly advisable, with no regional differences in consensus values. DISCUSSION AND CONCLUSIONS: The proposals offered in the current study are applicable to the entire country, respond to the unmet needs detected in the initial survey, form a minimum action framework, and ensure optimal follow-up of patients with PsA.

Clinical significance of high levels of soluble tumour necrosis factor-α receptor-2 produced by alternative splicing in rheumatoid arthritis: a longitudinal prospective cohort study.

OBJECTIVES: We investigated whether serum levels of an alternatively spliced soluble (s)TNF receptor-2 (DS-TNFR2) affected the clinical response to anti-TNF-α therapy, classical DMARDs or radiological evidence of disease progression in patients with RA. METHODS: We included 116 patients with RA. Cohort 1: 52 DMARD-naïve early RA patients [mean (s.d.) disease duration 8.5 (6.2) months] who started gold salts and MTX therapies. Cohort 2: 64 MTX-resistant established RA patients [144 (107) months] who started infliximab therapy. We evaluated the European League Against Rheumatism (EULAR) response to therapy and the serum levels of DS-TNFR2, sTNFR2 and ACPAs at baseline and at 12 months. In Cohort 1, radiological progression and levels of MMP-1 were also determined. RESULTS: In Cohort 1, 40% of patients had high baseline levels (HL > 50 ng/ml) of DS-TNFR2 with significantly higher RF and ACPA levels than patients with normal levels (NL ≤ 50 ng/ml) of DS-TNFR2. The EULAR response to DMARDs was similar in HL and NL patients. Radiographic progression was observed in 23.5% of all patients after 12 months. In Cohort 2, 26.6% of patients had HL of DS-TNFR2 with significantly higher RF and ACPA levels than patients with NLs. The EULAR response from 6 to 30 weeks was prolonged in the HL group compared with the NL group. CONCLUSIONS: Patients with HL of DS-TNFR2 maintained a prolonged therapeutic response to anti-TNF-α therapy and had proportionally less radiographic progression compared with patients with NLs.

Micromechanics modelling for mineral volume fraction determination: application on a terrigenous formation.

This work presents a non-linear Self-Consistent (SC) micromechanics method to model the observed physical elastic properties of a terrigenous formation with the purpose to obtain its depth mineral volume fractions profile. In this approach, it is first assumed that the observed physical elastic properties obtained from well logs, such as the density [Formula: see text] and the elastic compressional [Formula: see text] and shear [Formula: see text] velocities, are a non-linear relationship of the unknown mineral volume fractions [Formula: see text]. Then, a gradient descent algorithm is implemented to seek for those volume fractions [Formula: see text] for which differences between modelled and observed physical elastic properties are minimum. It is assumed that quartz, calcite and clay are the main comprising minerals of the formation. Obtained volume fractions profile follow the same general trends to those estimated by implementing the Linear Least-Squares Inversion LLSI method which is widely used in petrophysical analysis to obtain mineral concentrations from density [Formula: see text], photoelectric effect [Formula: see text] and compressional slowness [Formula: see text] well logs. Results also show that calcite and clay volume fractions from these two methods are highly correlated while quartz volume fractions show low correlation. Further comparison between clay concentrations from SC method with clay concentrations calculated from direct measurements of gamma ray GR well logs used as a guideline also exhibits high correlation. These results suggest that the SC method is better suited to obtain clay and calcite volume fractions rather than quartz volume fractions. However, SC method can provide with insights about the general distribution of quartz along the borehole.

Cardiac function after CPAP therapy in patients with chronic heart failure and sleep apnea: a multicenter study.

BACKGROUND AND PURPOSE: Continuous positive airway pressure (CPAP) is an effective treatment for sleep apnea (SA), although the evidence for improving chronic heart failure (CHF) is inconclusive. Our aim was to evaluate the effect of CPAP treatment on the left ventricle ejection fraction (LVEF) among other cardiological variables in a randomized, multicenter, placebo (sham-CPAP)-controlled study. METHODS: After the selection procedure, 60 patients with CHF with LVEF<45% and SA with an apnea-hypopnea index (AHI)>10/h were evaluated at baseline, and after 3 months of treatment with optimal CPAP or sham-CPAP. The assessment was based on the LVEF, hypertension, daytime sleepiness (Epworth sleepiness scale [ESS]), quality of life (SF-36), New York Heart Scale (NYHA score), dyspnea (by using the Borg scale) and exercise tolerance (6-min walk test). RESULTS: The mean AHI was normalized in the optimal CPAP group but not in the sham-CPAP group. The LVEF showed a significant improvement in the group of patients treated with CPAP (2.5; 95% CI: 0.6 to 4.3), which was not observed in the sham-CPAP group (0.0; 95% CI: -2.1 to 2.1). However, the change in the LVEF from baseline to 3 months was not significantly greater in the whole group (obstructive and Cheyne-Stokes events) treated with CPAP than in the control group (p: 0.07). In patients with only obstructive sleep apnea (OSA), who account for 83% of the total population, there was a significant improvement in the LVEF in the group of patients treated with CPAP but no such improvement in the sham-CPAP group. In this OSA group, the change in the LVEF from baseline to 3 months was significantly greater in the group treated with CPAP than in the sham-CPAP group (p: 0.03). The other variables studied were not modified. When the patients were divided according to the severity of the LVEF (a LVEF cut-off of 30%), improvement was observed in those with a LVEF>30. No changes were found in the other cardiological variables. CONCLUSIONS: CPAP therapy proved to be useful in patients with associated sleep-disordered breathing and CHF. The improvement was more marked in patients with a LVEF>30%. However, the increased LVEF in the CPAP group was not accompanied by changes in the other cardiological variables.

Recommendations for the use of methotrexate in psoriatic arthritis. / Recomendaciones sobre el uso de metotrexato en pacientes con artritis psoriásica.

OBJECTIVES: To develop recommendations for the management of methotrexate (MTX) in psoriatic arthritis (PsA), based on best evidence and experience. METHODS: A group of 12 experts on MTX use was selected. The coordinators formulated 14 questions about the use of MTX in PsA patients (indications, efficacy, safety and cost-effectiveness). A systematic review was conducted to answer the questions. Using this information, inclusion and exclusion criteria were established, as were the search strategies (Medline, EMBASE and the Cochrane Library were searched). Two different reviewers selected the articles. Evidence tables were created. At the same time, European League Against Rheumatism and American College of Rheumatology abstracts were evaluated. Based on this evidence, the coordinators proposed 12 preliminary recommendations that the experts discussed and voted on in a nominal group meeting. The level of evidence and grade of recommendation were established using the Oxford Centre for Evidence Based Medicine and the level of agreement with the Delphi technique (2 rounds). Agreement was established if at least 80% of the experts voted yes (yes/no). RESULTS: A total of 12 preliminary recommendations on the use of MTX were proposed, 9 of which were accepted. One was included in a different recommendation and another 2 were not voted on and were thereafter clarified in the main text. CONCLUSIONS: These recommendations aim to answer frequent questions and help in decision making strategies when treating PsA patients with MTX.

ISLES 2016 and 2017-Benchmarking Ischemic Stroke Lesion Outcome Prediction Based on Multispectral MRI.

Performance of models highly depend not only on the used algorithm but also the data set it was applied to. This makes the comparison of newly developed tools to previously published approaches difficult. Either researchers need to implement others' algorithms first, to establish an adequate benchmark on their data, or a direct comparison of new and old techniques is infeasible. The Ischemic Stroke Lesion Segmentation (ISLES) challenge, which has ran now consecutively for 3 years, aims to address this problem of comparability. ISLES 2016 and 2017 focused on lesion outcome prediction after ischemic stroke: By providing a uniformly pre-processed data set, researchers from all over the world could apply their algorithm directly. A total of nine teams participated in ISLES 2015, and 15 teams participated in ISLES 2016. Their performance was evaluated in a fair and transparent way to identify the state-of-the-art among all submissions. Top ranked teams almost always employed deep learning tools, which were predominately convolutional neural networks (CNNs). Despite the great efforts, lesion outcome prediction persists challenging. The annotated data set remains publicly available and new approaches can be compared directly via the online evaluation system, serving as a continuing benchmark (www.isles-challenge.org).

[Clinical and pathological features in Venezuelan children with IgA nephropathy]. / Características clínico patológicas y evolución de la nefropatía por IgA en una serie hospitalaria de niños venezolanos.

IgA nephropathy (IgAN) is the most common glomerulonephritis in humans worldwide; its prevalence and prognosis vary according with geographical areas. The incidence is higher in adults under 30 years of age and in children, it occurs more frequently in patients between 3 and 10 years. Hematuria is the predominant manifestation at presentation of the disease and 20-40% of the cases progress to terminal chronic renal disease. Renal biopsies were performed in 426 children during the period 1980-2002, of them, 12 cases corresponded to IgAN. The clinico-pathological characteristics and evolution of patients were evaluated during an average of 3.85 years. Mean age of patients was 6.2 years, and it was more frequent in males. Hematuria and proteinuria were found in 100% of cases and proteinuria of nephrotic range in 75%. Hypertriglyceridemia and hypercholesterolemia in 91%, arterial hypertension in 50% and acute renal failure at presentation in 25%. The predominant histopathological patterns (WHO) were II and III, deposits of mesangial IgA, IgG and C3 were observed in all cases and C4 deposits in 25%. 41.7% of cases had complete remission, 41.7% maintained normal renal function with persistent proteinuria and 16% progressed to terminal chronic renal failure. The actuarial survival of patients was 100% at 3 years, 87% at 4 years and 76% at 8 years. Two patients died during the period of study, at 3.5 and 8.5 years. The variability of presentation of IgA nephropathy was confirmed in this study, which could be attributable to geographical differences, racial influences and clinicopathological features related to sanitary conditions. Despite of the frequency of bad prognosis characteristics at presentation of IgAN in our series, the evolution was similar to reports of other groups.

[Enlarged vestibular aqueduct syndrome: report of 3 cases and literature review]. / Síndrome do aqueduto vestibular alargado: relato de 3 casos e revisão bibliográfica.

The Enlarged Vestibular Aqueduct Syndrome is characterized by a widening of the vestibular aqueduct, associated with sensorineural hearing loss, or sometimes with mixed hearing loss, which may be congenital or acquired during childhood. The sensorineural hearing loss may be classified into mild, moderate and severe, associated with sudden periods of improvement or aggravation. The enlargement of the vestibular aqueduct is the most common inner ear anomaly. This syndrome is admitted as a result of a genetic abnormality of the vestibular aqueduct development, previous to the fifth week of gestation. The incidence of this syndrome ranges from 1% to 1.3%, with the possibility of getting up to 7%, depending on the examined population. The aim of this study was to analyze three cases of LVAS seen at the Otorhinolaryngology and Radiology Department of Sao Camilo Hospital - Sao Paulo. Two of these three cases were of brothers, from the same mother but from different fathers. Two were male and one was female and the ages ranged from 9 to 30 years old. The diagnosed method of election was CT, Computerized Tomography of the temporal bones. The procedure for the cases was that of observation, with exception for those of cranial traumatisms, barotraumas and, when necessary, the use of auditive prosthesis.

Is there a gender difference in non-small cell lung cancer survival?

BACKGROUND: A possible association had previously been noted between gender and prognosis in non-small cell lung cancer (NSCLC), with a better survival rate for women. OBJECTIVE: The purpose of the current study was to further clarify the role of gender as a possible prognostic factor in NSCLC. METHODS: This retrospective cohort study examined the survival of NSCLC patients who underwent surgical curative treatment at the Hospital Sao Lucas da PUCRS, Porto Alegre, Brazil, between January 1, 1990, and December 31, 2000. Data were analyzed using Kaplan-Meier survival curves. In addition, a Cox proportional hazards regression model was used to adjust for potential confounding factors. RESULTS: In the period studied, lung resections were performed in 253 patients with NSCLC, of whom 114 had stage I tumors. Four patients were excluded because of death in the immediate postoperative period, and 1 committed suicide. The 5-year survival rate was 85.5% for women and 46.4% for men (P<0.0001). The median overall survival time was 44.7 months (interquartile range [IQR(25%-75%)], 17.4-72.3 months) and was longer in women than in men: 63.9 months (IQR(25%5%), 35.2-98.7 months) versus 32.3 months (IQR(25%-75%), 11.8-61.5 months), respectively (P<0.0001). Gender effect was still present after adjustment by Cox regression for several factors (age, smoking habits, hemoglobin, forced expiratory volume in 1 second, tumor size, tumor-node-metastasis stage, histology, postoperative complications, and surgery type). The adjusted hazard ratio in women was 0.23 (95% CI, 0.09-0.59; (P<0.0001) when compared with men. CONCLUSION: This study confirms previous findings that women live longer than men after surgery for stage I NSCLC. This effect persisted after adjusting for several factors. These results highlight the fact that analyses of long-term survival of NSCLC patients, usually generalized to men and women as a whole, may be an inadequate extrapolation. These results question whether analyses of long-term survival, which commonly group men and women together, provide an adequate prognosis of survival rates in women with NSCLC.

Role of gender in the survival of surgical patients with nonsmall cell lung cancer.

PURPOSE: There are reports of greater survival rates in nonsmall cell lung cancer (NSCLC) patients of female gender. The objective of this study was to evaluate the role of gender in survival of NSCLC patients treated surgically with curative intent (stage I/II). METHODS: In a retrospective cohort design, we screened 498 NSCLC patients submitted to thoracotomies at the hospital Sγo Lucas, in Porto Alegre, Brazil from 1990 to 2009. After exclusion of patients that did not fit to all the inclusion criteria, we analyzed survival rates of 385 subjects. Survival was analyzed using the Kaplan-Meier method. The Cox regression model was used to evaluate potential confounding factors. RESULTS: Survival rates at 5 and 10 years were 65.3% and 49.5% for women and 46.5% and 33.2% for men, respectively (P = 0.006). Considering only stage I patients, the survival rates at 5 and 10 years were 76.2% and 55.1% for women and 50.7% and 35.4% for men, respectively (P = 0.011). No significant differences in survival rates were found among stage II patients. CONCLUSIONS: Our results show female gender as a possible protective factor for better survival of stage I NSCLC patients, but not among stage II patients. This study adds data to the knowledge that combined both genders survival rates for NSCLC is not an adequate prognosis.

Biopsia renal en pediatría: análisis de la casuística de 28 años: Hospital de Niños de Valencia-Venezuela: [revisión] / Renal biopsy in pediatrics: casuistica analysis of 28 years: Hospital of Niños of Valencia-Venezuela: [review]

El análisis de los resultados de casuísticas de biopsias renales es importante con fines diagnósticos, terapéuticos y de pronóstico. Evaluar la serie de biopsias renales en el Hospital de Niños de Valencia, Venezuela, durante el período 1978-2007. Fueron analizadas 421 biopsias renales practicadas en 395 pacientes, de 2 meses a 20 años, 57% varones. El material fue procesado por microscopía óptica, inmunofluorescencia y microscopía electrónica en 98% de los casos. Se obtuvo muestra adecuada (más de 10 glomérulos) en 93% de los casos (n=392). Indicaciones clínicas: síndrome nefrótico 199(50%), síndrome nefrítico atípico 53(13%), otro: (hematuria/proteinuria, proteinuria, trasplante, enfermedades sistémicas 143(37%). Resultados Histopatológicos: A.-Glomerulonefrítis primaria (GNP) 302 casos (77%), B.-Nefropatías secundarias (NS) 68 casos (17%), C.-Riñones trasplantados 28 casos (7%).-Diagnósticos en GNP: 1) Lesión de cambios mínimos 140(46%), 2) Glomeruloesclerosis segmentaria y focal 79 (26%), 3) GN proliferativa y/o mesangial 67(22%), 4) GN Membranosa 16 (5%). -Diagnósticos en NS: Nefritis lúpica: 20 casos (32.25%), Nefropatía IgA: 22 casos (35.50%); Otras: 20 casos (32.25%). -Diagnósticos en riñones trasplantados: rechazo agudo 50%, necrosis tubular aguda 25%, rechazo crónico 20%, enfermedad recurrente en trasplante 5%. Complicaciones: Hematuria transitoria: 21 casos (5%), hematoma perirenal: 3(<1%), perforación intestinal: 2 (<0.5%), hemorragia importante: 2 (<0.5%), nefrectomía: 1(0.2%). La presente es una de las primeras casuísticas de biopsias renales reportadas en Latinoamérica y una de las más grandes en el mundo y, de acuerdo a nuestros resultados, es un procedimiento seguro con gran utilidad diagnóstica, pocas complicaciones, sin mortalidad.

Evaluation and analysis of the results of renal biopsy are important for diagnostic, therapeutic and prognostic matters. To evaluate a series of renal biopsies performed during the period 1978-2007 in the Hospital de Niños de Valencia, Venezuela. All patients had history of either primary or secondary nephropathies. 421 biopsies were done in 377 patients, ages 2 months-20 years; 57% boys. 26 patients were re-biopsed. Percutaneous needle biopsy (PNB) was performed in all the patients, except in one who underwent open biopsy because of a solitary kidney. Renal tissue was processed for optical, inmunofluorescence and electronic microscopy in 98% of cases. The biopsy technique, clinical syndromes at presentation, hystopathological pattern, effectiveness and complications are described. Adequate sample was obtained in 392 cases (93%) (more than 10 glomeruli) and inadequate or failed biopsy in 29(7%). Clinical syndromes at presentation were: nephrotic syndrome: 199 cases (50%), atypical acute nephritic syndrome: 53 (13%), others: hematuria and proteinuria, isolated proteinuria, kidney transplant biopsy or systemic diseases: 143(37%). The hystopathological pattern obtained was as follows. A.-Primary glomerulonephritis (PG): 302 cases, 77%, B.-Secondary nephropathies: 68 cases, 17%, C.- Kidney transplant biopsies: 28 cases, 5 %. Primary Glomerulonephritis diagnosis: minimal change disease: 140 cases, 46%, Focal Segmental Glomerulosclerosis: 79(26%), diffuse proliferative glomerulonephritis/mesangial: 67(22%), membranous glomerulonephritis: 16(5%). Secondary nephropathies: lupus nephritis: 20 cases (32.25%), IgA Nephropathy: 22 cases (35.50%), others: 20 cases (32.25%).Transplant biopsies: rejection 50%, acute tubular necrosis 25%, chronic rejection 20%, and recurrent disease 5%. Complications: transient hematuria: 21(5%), perirenal hematoma: 3(<1%), gut perforation (<0.2%), bleeding which required blood transfusion: 2(<0.5%) and nephrectomy because of incontrollable...

Síndrome do aqueduto vestibular alargado: relato de 3 casos e revisão bibliográfica

A Síndrome do Aqueduto Vestibular Alargado (SAVA) é caracterizada por um alargamento do aqueduto vestibular associado a uma perda auditiva neurossensorial, algumas vezes mista, que pode ser congênita ou adquirida na infância. A disacusia pode ser dividida em leve, moderada ou profunda, associada com períodos de melhora ou de piora súbita. O alargamento do aqueduto vestibular é a anomalia da orelha interna mais comum. A SAVA é admitida como resultado de uma anormalidade genética no desenvolvimento do aqueduto vestibular anterior à quinta semana de gestação. A incidência de SAVA está entre 1 por cento e 1,3 por cento, podendo chegar a 7 por cento dependendo da população examinada. O objetivo deste estudo é relatar 3 casos de SAVA atendidos no Núcleo de Otorrinolaringologia e Cirurgia de Cabeça e Pescoço de São Paulo e no serviço de Radiologia do Hospital São Camilo - São Paulo, sendo que dois pacientes são irmãos somente por parte materna. Dois pacientes eram do sexo feminino, um do sexo masculino e a idade variou de 9 a 30 anos. O método diagnóstico de eleição é a TC de osso temporais. A conduta para os casos foi o tratamento conservador com ressalva a cuidados como traumatismos cranianos, barotraumas e, quando necessário, uso de próteses auditivas.

Paraglanglima de corpo carotídeo: apresentação de caso e revisão da bibliográfica / Carotid body tumor: a case report and literature review

Os paragangliomas são tumores que se desenvolvem a partir dos paragânglios. São raros, têm predominância familiar e malignizam-se em poucos casos. O mais comum é o de corpo carotídeo (0,012% de todos os tumores de cabeça e pescoço). A manifestação clínica mais comum é a presença de massa cervical lateral. Angiografia é o exame de escolha para o diagnóstico do tumor. O tratamento é cirúrgico. Apresentamos o caso de uma paciente com 67 anos de idade, com massa cervical bilateral, assintomática. São descritos a abordagem diagnóstica, os detalhes cirúrgicos e o resultado pós-operatório.

Paraganglioma are tumors that arise from paraganglia. They are rare, there is inheritability and malignancy is also rare. The most common one is carotid body paraganglioma (0,012% of all head and neck tumors). The most common feature is lateral cervical mass. Angiography is gold-standard for diagnoses. Surgical treatment is emphasised. We report the case of a 67 year-old woman with asimptomatic bilateral neck mass. Diagnostic and therapeutic approaches as outcome are described.

Estudo comparativo histológico na prega vocal após incisão com instrumental a frio e com laser de CO2 em modelo animal / Comparative histology study of the vocal folds after incision with cold instruments and CO2 laser in an animal model

Dentre os vários tipos de laser usados em Medicina, o laser de CO2 é o mais usado na Otorrinolaringologia e Cirurgia de Cabeça e Pescoço. As vantagens de seu uso são a diminuição do sangramento, a diminuição do edema no pós-operatório e a facilidade de acesso ao campo operatório, entre outras. Desde os trabalhos de Jako e Strong em 1972(1,2), quando o laser de CO2 passou a ser usado no tratamento de papilomatose laríngea e de lesões malignas glóticas iniciais, suas indicações têm aumetado, principalmente em lesões benignas, a partir da alta tecnologia desenvolvida dos últimos anos como, por exemplo, a diminuição do microspot e o uso do superpulso, reduzindo conseqüentemente seu efeito térmico sobre os tecidos. MÉTODOS: Neste trabalho foram realizadas incisões com instrumental a frio e com laser de CO2 1 watt de modo contínuo e superpulso, em pregas vocais caninas e observado, através de cortes histológicos corados pelo método de Sirius Red, a quantidade de colágeno depositada sobre as mesmas. RESULTADOS: A quantidade de colágeno das pregas vocais foi maior do que no grupo controle, e estatisticamente maior no grupo de animais submetidos a procedimentos com instrumental a frio do que com laser de CO2. Não houve diferença estatística entre o grupo controle e o grupo submetido a incisões com instrumentos a frio. CONCLUSÃO: A microcirurgia de laringe com o laser de CO2, quando este é usado em baixa potência, com pequeno "microspot" e com superpulso, é um método seguro em relação à deposição de colágeno, quando comparado com instrumentos com lâmina a frio, obedecendo os princípios da fonomicrocirurgia.

Sobrevida de longo prazo em carcinoma brônquico após tratamento cirúrgico: sexo é fator prognóstico? / Long time survival rate in lung carcinoma after surgical treatment: is gender a prognostic factor?

INTRODUÇAO: A semelhança de resultados relatados por outros autores, anteriormente havíamos encontrado possível associação entre sexo e prognóstico em carcinoma brônquico não-pequenas células (CBNPC) em estágio I, com melhores taxas de sobrevida em mulheres. OBJETIVO: O objetivo do presente trabalho foi o de ampliar o estudo dos possíveis fatores prognósticos em CBNPC. MÉTODO: Em estudo de coorte retrospectivo, foi avaliada a sobrevida de 163 pacientes com CBNPC tratados cirurgicamente, com intenção curativa, no Hospital São Lucas da Pontifícia Universidade Católica do Rio Grande do Sul (PUCRS), entre 1990 e 1997, até o óbito ou o seguimento por cinco anos. Os dados foram analisados através de curvas de Kaplan-Meier e pelo teste de Mann-Whitney para comparar os tempos de sobrevida e pelo modelo de regressão de Cox para avaliar possíveis fatores de confusão. RESULTADOS: Foram incluídos 124 (76,07 por cento) homens e 39 (23,93 por cento) mulheres. A sobrevida mediana foi 32,3 e 60,6 meses e a sobrevida em cinco anos de 38,0 por cento e 55,4 por cento, para homens e mulheres, respectivamente (p=0,030). Considerando apenas pacientes em Estágio I, as taxas de sobrevida foram 44,4 por cento e 81,8 por cento para homens e mulheres, respectivamente (p=0,009). O efeito do sexo persistiu após ajuste para vários fatores (idade, hemoglobina, histologia, tamanho do tumor, extensão da cirurgia e complicações pós-operatórias), realizado através da regressão de Cox. O risco relativo em mulheres foi 0,09 (IC90 por cento:0,03-0,25, p<0,001) quando comparado com o de homens. CONCLUSAO: Este estudo confirmou achados prévios de que as mulheres vivem por mais tempo após cirurgia para tratamento de CBNPC, quando comparadas aos homens. Esse efeito é observado apenas em estágio precoce e persiste após ajuste de vários fatores.