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BACKGROUND: The value of myeloperoxidase (MPO) and proteinase 3 (PR3) antibody titres in the assessment of renal disease activity and flare prediction in patients with anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) is not well known. METHODS: We performed a retrospective study including 113 AVV patients with renal biopsy-proven pauci-immune necrotizing glomerulonephritis from seven Spanish hospitals. The main inclusion criteria were assessment of MPO antibodies using multiplex flow immunoassay and PR3 antibody measurements using immunoassay chemiluminescence with an identical range of values for all participating centres. RESULTS: Serum MPO antibodies 3 ± 1.2 months before relapse were higher in patients who relapsed [19.2 ± 12.2 versus 3.2 ± 5.1 antibody index (AI); P < 0.001]. The discrimination value of MPO antibodies 3 months before renal relapse had an area under the receiver operating characteristics curve (AUC) of 0.82 [95% confidence interval (CI) 0.73-0.92; P < 0.001]. ΔMPO antibodies (change in antibodies titration 6 months before relapse) were higher in patients who relapsed (8.3 ± 12 versus 0.9 ± 3.1 AI; P = 0.001). The discrimination value of ΔMPO had an AUC of 0.76 (95% CI 0.63-0.88; P < 0.001). The positive predictive value of renal relapse in PR3 patients is 100% and the negative predictive value of renal relapse in patients with PR3-positive titres is 57.1%. Serum PR3 antibodies were higher in patients who relapsed 2.8 ± 1.4 months before relapse (58.6 ± 24.6 versus 2.0 ± 0.6 AI; P < 0.001). CONCLUSIONS: MPO level monitoring using multiplex flow immunoassay and PR3 measurements using immunoassay chemiluminescence are useful and sensitive tools for the prediction of renal relapse in the follow-up of AAV patients with renal disease and relevant surrogate markers of renal disease activity.
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Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos , Enfermedades Renales , Nefritis , Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/complicaciones , Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/diagnóstico , Anticuerpos Anticitoplasma de Neutrófilos , Femenino , Humanos , Masculino , Mieloblastina , Peroxidasa , Recurrencia , Estudios RetrospectivosRESUMEN
A cyclical corticosteroid-cyclophosphamide regimen is recommended for patients with primary membranous nephropathy at high risk of progression. We hypothesized that sequential therapy with tacrolimus and rituximab is superior to cyclical alternating treatment with corticosteroids and cyclophosphamide in inducing persistent remission in these patients. This was tested in a randomized, open-label controlled trial of 86 patients with primary membranous nephropathy and persistent nephrotic syndrome after six-months observation and assigned 43 each to receive six-month cyclical treatment with corticosteroid and cyclophosphamide or sequential treatment with tacrolimus (full-dose for six months and tapering for another three months) and rituximab (one gram at month six). The primary outcome was complete or partial remission of nephrotic syndrome at 24 months. This composite outcome occurred in 36 patients (83.7%) in the corticosteroid-cyclophosphamide group and in 25 patients (58.1%) in the tacrolimus-rituximab group (relative risk 1.44; 95% confidence interval 1.08 to 1.92). Complete remission at 24 months occurred in 26 patients (60%) in the corticosteroid-cyclophosphamide group and in 11 patients (26%) in the tacrolimus-rituximab group (2.36; 1.34 to 4.16). Anti-PLA2R titers showed a significant decrease in both groups but the proportion of anti-PLA2R-positive patients who achieved immunological response (depletion of anti-PLA2R antibodies) was significantly higher at three and six months in the corticosteroid-cyclophosphamide group (77% and 92%, respectively), as compared to the tacrolimus-rituximab group (45% and 70%, respectively). Relapses occurred in one patient in the corticosteroid-cyclophosphamide group, and three patients in the tacrolimus-rituximab group. Serious adverse events were similar in both groups. Thus, treatment with corticosteroid-cyclophosphamide induced remission in a significantly greater number of patients with primary membranous nephropathy than tacrolimus-rituximab.
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Glomerulonefritis Membranosa , Tacrolimus , Corticoesteroides/efectos adversos , Ciclofosfamida/efectos adversos , Glomerulonefritis Membranosa/tratamiento farmacológico , Humanos , Inmunosupresores/efectos adversos , Rituximab/efectos adversos , Tacrolimus/efectos adversos , Resultado del TratamientoRESUMEN
Background: Complement dysregulation occurs in thrombotic microangiopathies (TMAs) other than primary atypical haemolytic uraemic syndrome (aHUS). A few of these patients have been reported previously to be successfully treated with eculizumab. Methods: We identified 29 patients with so-called secondary aHUS who had received eculizumab at 11 Spanish nephrology centres. Primary outcome was TMA resolution, defined by a normalization of platelet count (>150 × 10 9 /L) and haemoglobin, disappearance of all the markers of microangiopathic haemolytic anaemia (MAHA), and improvement of renal function, with a ≥25% reduction of serum creatinine from the onset of eculizumab administration. Results: Twenty-nine patients with secondary aHUS (15 drug-induced, 8 associated with systemic diseases, 2 with postpartum, 2 with cancer-related, 1 associated with acute humoral rejection and 1 with intestinal lymphangiectasia) were included in this study. The reason to initiate eculizumab treatment was worsening of renal function and persistence of TMA despite treatment of the TMA cause and plasmapheresis. All patients showed severe MAHA and renal function impairment (14 requiring dialysis) prior to eculizumab treatment and 11 presented severe extrarenal manifestations. A rapid resolution of the TMA was observed in 20 patients (68%), 15 of them showing a ≥50% serum creatinine reduction at the last follow-up. Comprehensive genetic and molecular studies in 22 patients identified complement pathogenic variants in only 2 patients. With these two exceptions, eculizumab was discontinued, after a median of 8 weeks of treatment, without the occurrence of aHUS relapses. Conclusion: Short treatment with eculizumab can result in a rapid improvement of patients with secondary aHUS in whom TMA has persisted and renal function worsened despite treatment of the TMA-inducing condition.
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Anticuerpos Monoclonales Humanizados/uso terapéutico , Síndrome Hemolítico Urémico Atípico/tratamiento farmacológico , Inactivadores del Complemento/uso terapéutico , Adulto , Síndrome Hemolítico Urémico Atípico/etiología , Síndrome Hemolítico Urémico Atípico/metabolismo , Síndrome de Churg-Strauss/complicaciones , Creatinina/metabolismo , Femenino , Humanos , Inmunosupresores/efectos adversos , Pruebas de Función Renal , Lupus Eritematoso Sistémico/complicaciones , Masculino , Persona de Mediana Edad , Plasmaféresis , Recuento de Plaquetas , Recurrencia , Insuficiencia Renal/etiología , Insuficiencia Renal/metabolismo , Esclerodermia Sistémica/complicaciones , Microangiopatías Trombóticas/tratamiento farmacológico , Microangiopatías Trombóticas/metabolismoRESUMEN
PURPOSE: To analyse pre-operative and post-operative changes of cervical sagittal alignment (CSA) in Scheuermann's kyphosis (SK) patients. METHODS: 64 SK and 33 control patients were retrospectively reviewed. Whole spine X-rays obtained at pre-op, 3 months post-op and at latest follow-up were reviewed and ten separate CSA radiological parameters were measured. Patients were divided in three groups: thoracic SK (TK group, apex T6-T9, n = 40), thoracolumbar SK (TLK group, apex T10-T12, n = 24), and controls. RESULTS: Pre-operative C2-C7 lordosis was 21.1° ± 8.1°(TK), 6.1° ± 5.0°(TLK), and 11.4° ± 8.3° in control group and correlated significantly with T1 slope in both SK groups (r = 0.640, r = 0.772). Pre-operative T1 slope was dependent on deformity type, thoracic kyphosis (TK, ß = 0.445), and lumbar lordosis (LL, ß = -0.354). At final follow-up C2-C7 lordosis decreased to 15.7° ± 5.5° in TK, and increased to 12.1° ± 4.1° in TLK group. C2-C7 lordosis changes linearly correlated with T1 slope changes post-operatively (r = 0.721). Post-operative T1 slope showed linear correlation with post-operative changes in TK (ß = 0.728) and pelvic tilt (PT, ß = 0.539) in TK, and LL (ß = -0.669), thoracolumbar kyphosis (TLK, ß = -0.434), and PT (ß = 0.760) in TLK group. CONCLUSIONS: Our study suggests that SK is not a homogenous group of patients. Two patterns of pre- and post-operative CSA are demonstrated in TK and TLK groups. T1 slope is the most important parameter in determining pre-operative CSA and correlates with other regional anatomical parameters (TK and LL). Post-operative CSA adaptations also correlate with T1 slope post-operative changes. However, post-surgical T1 slope correlates with different parameters in the two SK groups (TK and PT in TK group; TLK, LL, and PT in TLK group).
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Vértebras Cervicales/patología , Enfermedad de Scheuermann/cirugía , Adolescente , Adulto , Estudios de Casos y Controles , Vértebras Cervicales/diagnóstico por imagen , Femenino , Estudios de Seguimiento , Humanos , Masculino , Radiografía , Estudios Retrospectivos , Enfermedad de Scheuermann/diagnóstico por imagen , Enfermedad de Scheuermann/patología , Resultado del Tratamiento , Adulto JovenRESUMEN
BACKGROUND: Standard laminectomy for treatment of cervical myelopathy is associated with secondary instability and kyphosis, while laminectomy combined with fusion puts adjacent segments at risk of degeneration. Single- and double-door laminoplasty techniques have been developed to overcome these limitations. More recently, complications related to bone graft dislodgment have fostered development of hardware-augmented laminoplasty techniques. The aim of this study is to review the clinical safety and effectiveness of a newly developed technique of instrumented French-door laminoplasty for treatment of cervical myelopathy. MATERIALS AND METHODS: A series of 25 consecutive myelopathic patients were treated with a novel instrumented cervical French-door laminoplasty technique, whereby the enlarged posterior arch was held open with maxillofacial plates and screws. Patients had pre- and postoperative assessments with the Neck Disability Index (NDI), Japanese Orthopaedic Association (JOA) Score, Visual Analogue Score and radiographs. Minimum follow-up was 40 months, with regular interval assessments. RESULTS: There were 18 males with a mean age of 45 years. The mean operative time was 145 min. The average hospital stay was 2.4 days and the mean follow-up was 56.5 months (40-72). All patients reported neurological improvements and there was a 35% improvement in NDI, and JOA score improved by 4.8 points. No postoperative hardware-related complications were noted and only one case of temporary C5 palsy, which completely resolved by the one-year follow-up. CONCLUSIONS: Our data and clinical experience demonstrate that this hardware-augmented laminoplasty technique is safe and effective. We observed no hardware-related complications in our series. The use of readily available maxillofacial titanium miniplates and ease of surgical procedure means that this technique can be easily adopted into clinical practice. LEVEL OF EVIDENCE: Level IV.
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Placas Óseas , Trasplante Óseo , Laminoplastia/instrumentación , Enfermedades de la Médula Espinal/cirugía , Adulto , Vértebras Cervicales , Diseño de Equipo , Femenino , Estudios de Seguimiento , Humanos , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Enfermedades de la Médula Espinal/diagnóstico , Factores de Tiempo , Tomografía Computarizada por Rayos X , Resultado del TratamientoRESUMEN
BACKGROUND: Although tacrolimus is recommended by KDIGO Clinical Practice Guideline for Glomerulonephritis for the treatment of idiopathic membranous nephropathy (MN), little is known about factors that influence response and relapse of the disease after tacrolimus therapy. METHODS: Multicentre study that collected 122 MN patients with nephrotic syndrome and stable renal function treated with tacrolimus. Duration of treatment was 17.6 ± 7.2 months, including a full-dose and a tapering period. RESULTS: The percentage of remission was 60, 78 and 84% after 6, 12 and 18 months of treatment, respectively. The amount of proteinuria at baseline significantly predicted remission, the lower the baseline proteinuria the higher the probability of remission. Only 10 patients (8%) received concomitantly corticosteroids, and their rate of remission was similar (80% at 18 months). Among responders, 42% achieved complete remission (CR) and 58% partial remission (PR). Almost half (44%) of the responder patients relapsed. The amount of proteinuria at the onset of tacrolimus tapering was significantly higher in relapsing patients. By multivariable analysis, the presence of a PR versus CR at the onset of tacrolimus tapering and a shorter duration of the tapering period significantly predicted relapses. Tolerance was good and the number of adverse events low. CONCLUSIONS: Tacrolimus monotherapy is an effective and safe option for the treatment of MN with stable renal function. Relapses are frequent in patients with PR and can be partially prevented by a longer tapering period.
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Glomerulonefritis Membranosa/tratamiento farmacológico , Inmunosupresores/uso terapéutico , Proteinuria/diagnóstico , Proteinuria/epidemiología , Tacrolimus/uso terapéutico , Estudios de Casos y Controles , Femenino , Humanos , Incidencia , Estudios Longitudinales , Masculino , Persona de Mediana Edad , Pronóstico , Recurrencia , Inducción de Remisión , Estudios RetrospectivosRESUMEN
BACKGROUND: A standing X-ray is recommended for decision-making relative to the therapy for a traumatic thoracolumbar fracture (TLF). However, standing X-ray management can be demanding in elderly patients because of pain. The goal of this study was to determine whether supine radiograph is sufficient for proper therapeutic decision-making in patients older than 65 years with acute stable traumatic TLF. METHODS: Patients older than 65 years who came to the emergency department diagnosed with an acute and stable traumatic vertebral fracture between T10 and L3 (both included) were included in the study. Initially, all the patients were studied with a supine radiograph and computed tomography (CT) scan. If the TLF was stable, a standing radiograph was performed. Segmental kyphosis (SK) and visual analog scale (VAS) score were collected and compared in both the supine and standing X-ray projections. RESULTS: Twenty-seven patients with a mean age of 76.39 (range: 65-93) years were included; most were females. The mean supine SK was 10.14degrees (SD±7.22degrees). It increased to 12.97 (SD±8.61degrees) in the standing projection (p<0.001). In 37.1% of the patients, the SK increased from 13.22degrees (SD±7.21degrees) in supine X-ray to 19.96degrees (SD±5.34degrees) in the standing position in this group. When the initial supine projection showed an SK of ≥10degrees, the mean SK observed in the standing X-ray increased to 20.5degrees (SD±5.30, p=0.321). CONCLUSION: Stable traumatic TLF in patients older than 65 years showing ≥10degrees of SK in supine radiography may benefit from a standing radiography to make a proper therapeutic decision.
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Columna Vertebral , Posición de Pie , Femenino , Humanos , Anciano , Masculino , Posición Supina , Radiografía , Tomografía Computarizada por Rayos XRESUMEN
Background: Genetic causes are increasingly recognized in patients with focal segmental glomerulosclerosis (FSGS), but it remains unclear which patients should undergo genetic study. Our objective was to determine the frequency and distribution of genetic variants in steroid-resistant nephrotic syndrome FSGS (SRNS-FSGS) and in FSGS of undetermined cause (FSGS-UC). Methods: We performed targeted exome sequencing of 84 genes associated with glomerulopathy in patients with adult-onset SRNS-FSGS or FSGS-UC after ruling out secondary causes. Results: Seventy-six patients met the study criteria; 24 presented with SRNS-FSGS and 52 with FSGS-UC. We detected FSGS-related disease-causing variants in 27/76 patients (35.5%). There were no differences between genetic and non-genetic causes in age, proteinuria, glomerular filtration rate, serum albumin, body mass index, hypertension, diabetes or family history. Hematuria was more prevalent among patients with genetic causes. We found 19 pathogenic variants in COL4A3-5 genes in 16 (29.3%) patients. NPHS2 mutations were identified in 6 (16.2%) patients. The remaining cases had variants affecting INF2, OCRL, ACTN4 genes or APOL1 high-risk alleles. FSGS-related genetic variants were more common in SRNS-FSGS than in FSGS-UC (41.7% vs 32.7%). Four SRNS-FSGS patients presented with NPHS2 disease-causing variants. COL4A variants were the most prevalent finding in FSGS-UC patients, with 12 patients carrying disease-causing variants in these genes. Conclusions: FSGS-related variants were detected in a substantial number of patients with SRNS-FSGS or FSGS-UC, regardless of age of onset of disease or the patient's family history. In our experience, genetic testing should be performed in routine clinical practice for the diagnosis of this group of patients.
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Introduction: Anti-glomerular basement membrane (anti-GBM) disease is a severe entity with few therapeutic options including plasma exchange and immunosuppressive agents. The aim of this study was to analyze the clinical and pathological features that predict the evolution of end-stage kidney disease (ESKD) and the kidney survival in a cohort of patients with anti-GBM disease with renal involvement in real life. Methods: A retrospective multicentre observational study including 72 patients from 18 nephrology departments with biopsy-proven anti-GBM disease from 1999 to 2019 was performed. Progression to ESKD in relation to clinical and histological variables was evaluated. Results: Creatinine at admission was 8.6 (± 4) mg/dL and 61 patients (84.7%) required dialysis. Sixty-five patients (90.3%) underwent plasma exchange. Twenty-two patients (30.6%) presented pulmonary hemorrhage. Kidney survival was worse in patients with creatinine levels > 4.7 mg/dL (3 vs. 44% p < 0.01) and in patients with > 50% crescents (6 vs. 49%; p = 0.03). Dialysis dependence at admission and creatinine levels > 4.7 mg/dL remained independent significant predictors of ESKD in the multivariable analysis [HR (hazard ratio) 3.13 (1.25-7.84); HR 3 (1.01-9.14); p < 0.01]. The discrimination value for a creatinine level > 4.7 mg/dL and 50.5% crescents had an area under the curve (AUC) of 0.9 (95% CI 0.82-0.97; p < 0.001) and 0.77 (95% CI 0.56-0.98; p = 0.008), respectively. Kidney survival at 1 and 2 years was 13.5 and 11%, respectively. Patient survival at 5 years was 81%. Conclusion: In real life, patients with severe anti-GBM disease (creatinine > 4.7 mg/dL and > 50% crescents) remained with devastating renal prognosis despite plasma exchange and immunosuppressive treatment. New therapies for the treatment of this rare renal disease are urgently needed.
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BACKGROUND AND OBJECTIVES: Atypical hemolytic uremic syndrome is a form of thrombotic microangiopathy caused by dysregulation of the alternative complement pathway. There is evidence showing complement activation in other thrombotic microangiopathies. The aim of this study was to evaluate complement activation in different thrombotic microangiopathies and to monitor treatment response. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: Complement activation was assessed by exposing endothelial cells to sera or activated-patient plasma-citrated plasma mixed with a control sera pool (1:1)-to analyze C5b-9 deposits by immunofluorescence. Patients with atypical hemolytic uremic syndrome (n=34) at different stages of the disease, HELLP syndrome (a pregnancy complication characterized by hemolysis, elevated liver enzymes, and low platelet count) or severe preeclampsia (n=10), and malignant hypertension (n=5) were included. RESULTS: Acute phase atypical hemolytic uremic syndrome-activated plasma induced an increased C5b-9 deposition on endothelial cells. Standard and lower doses of eculizumab inhibited C5b-9 deposition in all patients with atypical hemolytic uremic syndrome, except in two who showed partial remission and clinical relapse. Significant fibrin formation was observed together with C5b-9 deposition. Results obtained using activated-plasma samples were more marked and reproducible than those obtained with sera. C5b-9 deposition was also increased with samples from patients with HELLP (all cases) and preeclampsia (90%) at disease onset. This increase was sustained in those with HELLP after 40 days, and levels normalized in patients with both HELLP and preeclampsia after 6-9 months. Complement activation in those with malignant hypertension was at control levels. CONCLUSIONS: The proposed methodology identifies complement overactivation in patients with atypical hemolytic uremic syndrome at acute phase and in other diseases such as HELLP syndrome and preeclampsia. Moreover, it is sensitive enough to individually assess the efficiency of the C5 inhibition treatment.
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Activación de Complemento , Microangiopatías Trombóticas/inmunología , Adulto , Anticuerpos Monoclonales Humanizados/uso terapéutico , Síndrome Hemolítico Urémico Atípico/tratamiento farmacológico , Síndrome Hemolítico Urémico Atípico/inmunología , Complejo de Ataque a Membrana del Sistema Complemento/metabolismo , Femenino , Síndrome HELLP/inmunología , Humanos , Masculino , Preeclampsia/tratamiento farmacológico , Preeclampsia/inmunología , Embarazo , Microangiopatías Trombóticas/tratamiento farmacológicoRESUMEN
STUDY DESIGN: Retrospective, blinded analysis of imaging studies. OBJECTIVE: The aim of this study is compare the use of magnetic resonance imaging (MRI) to lateral radiograph using bolster in the evaluation of Scheuermann kyphosis (SK) curve flexibility measurement. SUMMARY AND BACKGROUND DATA: The flexibility of the thoracic curve [thoracic kyphosis (TK)] in SK is of primary importance in its preoperative planning. Several methods have been described for SK curve flexibility measurement. The most commonly used method is lateral hyperextension radiography on hard bolster [hyperextension radiograph (HE)]. No current methods use MRI for flexibility assessment. MATERIALS AND METHODS: Flexibility of TK in SK patients was measured as a difference between standing radiograph and bolster-assisted lateral HE or supine MRI. The sagittal Cobb angle of the TK was measured between the superior endplate of T4 and the inferior endplate of T12 vertebral body. Flexibilities measured by these 2 methods were compared and analyzed using the generalized estimating equation analysis and the correlation analysis. RESULTS: We assessed 18 SK patients (14 males and 4 females) with mean age of 20.06±6.03 years. The standing TK x-rays showed 83.8±6.1 degrees. On HE, TK curve reduced by 39.3 degrees (95% confidence interval, 35.8-42.9) to 44.5±6.2 degrees (P<0.001). Preoperative MRI images showed TK of 53.8±5.9 degrees which means reduction by 30 degrees (95% confidence interval, 26.6-33.4) from the standing radiographs (P<0.001). Linear dependency between HE and MRI flexibility with a mean difference of 9.3 degrees was found (R=0.61, P<0.001). CONCLUSIONS: Our study shows that preoperative MRI can be used for SK flexibility assessment with similar predictive value as routinely used bolster-assisted hyperextension lateral radiograph. Consequently, patient exposure to preoperative hyperextension ionizing radiation may be reduced.
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Imagen por Resonancia Magnética , Enfermedad de Scheuermann/diagnóstico , Enfermedad de Scheuermann/fisiopatología , Adolescente , Adulto , Fenómenos Biomecánicos , Femenino , Humanos , Masculino , Docilidad , Adulto JovenRESUMEN
BACKGROUND CONTEXT: Surgical correction of Scheuermann kyphosis (SK) is challenging and plagued by relatively high rates of proximal junctional kyphosis and failure (PJK and PJF). Normal sagittal alignment of the spine is determined by pelvic geometric parameters. How these parameters correlate with the risk of developing PJK in SK is not known. PURPOSE: The study aimed to investigate the relationship between preoperative and postoperative spinopelvic alignment and occurrence of PJK and PJF. STUDY DESIGN/SETTING: This is a retrospective observational cohort study. PATIENT SAMPLE: The sample included 37 patients who underwent posterior correction of SK from January 2006 to December 2012. OUTCOME MEASURES: The outcome measure was correlation analysis between preoperative and postoperative spinopelvic alignment parameters and the development of PJK over the course of the study period. METHODS: Whole spine x-rays obtained before surgery, 3 months after surgery, and at the latest follow-up were analyzed. The following parameters were measured: thoracic kyphosis (TK), lumbar lordosis (LL), sagittal vertical axis (SVA), pelvic incidence (PI), pelvic tilt (PT), and sacral slope (SS). The development of PJK was considered the primary end point of the study. Patient population was split into a control and a PJK group; repeated-measures analysis of variance was used to assess group and time differences. RESULTS: Seven patients developed PJK over the study period. Although the severity of the preoperative deformity (TK) did not differ significantly between the two groups, preoperative PI was significantly higher in the PJK group (51.9°C±8.6°C vs. 42.7°C±8.8°C, p=.018). Postoperative correction of TK was similar between the two groups (39.3% and 41.2%, p=.678) and final LL did not differ as well (53.6°C±9.2°C vs. 51.3°C±11.5°C). However, because PJK patients had larger preoperative PI values, a significant deficit of LL was observed at final follow-up in this group compared with the control group (ΔLL -10.5°C±9.8°C vs. 0.6°C±10.5°C, p=.013). CONCLUSIONS: Scheuermann kyphosis patients who developed PJK appeared to have a significant postoperative deficit of LL (lumbopelvic mismatch). Lumbar lordosis decreases after surgery following correction of TK; therefore, TK correction should be planned according to preoperative PI values to avoid excessive reduction of LL in patients with higher PI values.
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Cifosis/etiología , Lordosis/etiología , Procedimientos Ortopédicos/efectos adversos , Pelvis/fisiopatología , Enfermedad de Scheuermann/fisiopatología , Enfermedad de Scheuermann/cirugía , Adolescente , Adulto , Anciano , Femenino , Humanos , Lordosis/diagnóstico por imagen , Vértebras Lumbares/fisiopatología , Masculino , Pelvis/diagnóstico por imagen , Radiografía , Estudios Retrospectivos , Factores de Riesgo , Sacro/diagnóstico por imagen , Sacro/fisiopatología , Enfermedad de Scheuermann/diagnóstico por imagen , Vértebras Torácicas/fisiopatología , Insuficiencia del TratamientoRESUMEN
BACKGROUND CONTEXT: Proteus syndrome (PS) is an extremely rare congenital disorder causing asymmetric overgrowth of different tissues. The etiology remains unclear. Limb deformities are common and often necessitate amputations. Only a few cases associated with spinal deformities have been described. PURPOSE: The aim was to report a rare case of PS associated with spinal deformity and its surgical management. STUDY DESIGN: A case of young boy with PS causing vertebral hypertrophy and kyphoscoliotic deformity, which was surgically corrected, is presented. METHODS: The patient was assessed clinically and with whole spine plain radiographs, computed tomography, and magnetic resonance imaging. Surgical correction was performed. RESULTS: Satisfactory correction of the deformity was achieved by posterior spinal fusion with instrumentation from T4-L5, five Ponte osteotomies T8-L1, and an L2 pedicle subtraction osteotomy. The kyphosis was corrected from 87° to 55°; there was improvement in all spinopelvic parameters. One year after surgery, there was maintenance of the deformity correction with no deterioration of the sagittal balance, and the patient was free of pain and had no loss of neurologic function. CONCLUSIONS: Proteus syndrome can be associated with spinal stenosis and deformity. Although the syndrome can be progressive in nature, the symptomatic spinal pathology should be treated appropriately.
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Cifosis/cirugía , Osteotomía/métodos , Síndrome de Proteo/cirugía , Fusión Vertebral/métodos , Adolescente , Niño , Humanos , Cifosis/diagnóstico por imagen , Masculino , Síndrome de Proteo/diagnóstico por imagen , Radiografía , Resultado del TratamientoRESUMEN
Tercera y última etapa de una investigación (5, 6) cuya finalidad fue la construcción de escalas adecuadas para la evaluación subjetiva de estímulos sonoros, utilizando la Técnica del diferencial semántico. El objetivo de esta etapa fue caracterizar 40 estímulos sonoros a través de un grupo de las escalas antes mencionadas y teniendo en cuenta las dimensiones psicológicas subyacentes obtenidas por el análisis factorial de las mismas. Para ello se aplicó un programa especial de diferencial semántico obteniéndose los siguientes índices: puntaje escalar, polaridad, familiaridad y distancia inter-conceptos. Se determinaron los estímulos sonoros mejor caracterizados y las escalas más adecuadas para evaluar cada concepto