Sinovitis villonodular pigmentada focal: reporte de un caso / Focal pigmented villonodular synovitis: a case report

Pigmented villonodular synovitis represents a benign synovial proliferation associated to hemosiderin deposits, which originates in bone joints, bursae or tendinous sheaths. The knee articulation is the most commonly involved joint (80 percent of the cases), followed by the hip joint. Normally, this pathology has a diffuse presentation, and a small percentage shows a localized form. PVNS displays very specific imaging features, which allows a good diagnostic approach when aided by different radiological techniques, particularly magnetic resonance imaging (MRI). In this report we describe an infrequent case of focal PVNS localized in the hip, which was treated at "Clínica Alemana", Santiago, Chile.

La sinovitis villonodular pigmentada (SVNP) es una proliferación sinovial benigna asociada a depósitos de hemosiderina, que se origina en articulaciones, bursas o vainas tendíneas. La articulación más comprometida es la rodilla (80 por ciento de los casos), seguida por la cadera. Normalmente, esta patología es de presentación difusa y en un menor porcentaje se da en forma focal. La SVNP posee características imaginológicas muy particulares, lo que permite una buena aproximación diagnóstica de la mano de los diferentes métodos radiológicos, en particular la resonancia magnética. En este reporte se describe un caso poco frecuente de SVNP focal en la cadera, que se presentó en la Clínica Alemana de Santiago.

Actualización de la evaluación radiológica de la escoliosis / Update on radiologic assessment of scoliosis

Spinal deformities affect a considerable number of individuals of all ages. The etiological spectrum is broad and in cases of severe deformities a surgical treatment may be required. In addition to clinical evaluation, full spine radiographs constitute a fundamental tool, both in the diagnostic process as well as in the therapeutic follow-up of these patients. The aim of this paper is to provide a comprehensive overview on radiological assessment of spinal deformities, with an emphasis on scoliosis, by summarizing classical concepts and incorporating new ideas being developed in recent years, mainly due to the improvement and increased complexity of surgical management.

Las deformidades de columna vertebral afectan a un considerable número de individuos de todas las edades. Su espectro etiológico es amplio y en casos de deformidades severas, el manejo de éstas puede llegar a ser quirúrgico. Además de la evaluación clínica, la radiografía de columna total es un pilar fundamental, tanto en el proceso diagnóstico así como también en el control terapéutico de estos pacientes. El objetivo de este artículo es dar una visión integral de la evaluación radiológica de las deformidades de columna, con énfasis en las escoliosis, recapitulando conceptos clásicos como también incorporando nociones nuevas que se han desarrollado en los últimos años, debido principalmente al progreso y aumento de la complejidad de su manejo quirúrgico.

Tumores sólidos en niños / Solid tumors in children

El pronóstico y la posibilidad de sobrevida y curación en el cáncer han mejorado progresivamente en los últimos 20 a 30 años. En Chile, el avance y mejores resultados de sobrevida han sido evidentes desde la creación del grupo cooperativo nacional (PINDA) en 1988, siendo la sobrevida total de los niños oncológicos alrededor de 65 por ciento. El médico general juega un rol fundamental en la sospecha y diagnóstico precoz, pilares fundamentales para el tratamiento oportuno y buenos resultados. Revisaremos algunos conceptos generales de etiopatogenia, diagnóstico y tratamiento de los tumores sólidos en niños.

Síndrome de Williams: estudio clínico, citogenético, neurofisiológico y neuroanatómico / Williams syndrome: clinical, cytogenetic, neurophysiological and neuroanatomic features in 44 patients

Background: Williams syndrome (WS) is a genetically based disorder caused by deletion of elastin and contiguous genes on chromosome 7q11.23. This syndrome is characterized by multiorganic involvement with dysmorphic facial features and a distinctive cognitive profile. It is an interesting model for elucidation of relationships between brain, cognition and genes. Patients have a visual-spatial cognition impaired with relative strengths in social and language abilities. Aim: To report clinical, cytogenetic, neurophysiological and neuroanatomic features in 44 patients referred as WS. Patients and methods: Forty four patients, aged 2 to 17 years, with the clinical diagnosis of Williams syndrome were studied with fluorescence in situ hybridization (FISH). In three cases, electrophysiological and neuroimaging studies were performed. Result: The deletion was confirmed in 23 patients. In three patients with neurophysiological studies, event related potentials suggested a cognitive difficulty in detecting and processing visual stimuli. Magnetic resonance imaging showed normal brain morphology. SPECT showed hypoperfusion of the right frontal lobe and bilateral anterior cingulum hyperperfusion. Conclusions: There are functional alterations in the brains of patients with Williams, which may be related to the cognitive deficits

Myocardial high-energy phosphate metabolism is altered after treatment with anthracycline in childhood.

OBJECTIVES: We aimed to investigate whether changes in high-energy phosphate metabolism after treatment of children and young adults with anthracycline can be demonstrated non-invasively by 31P magnetic resonance spectroscopy. BACKGROUND: Abnormal myocardial energy metabolism has been suggested as a mechanism for anthracycline-induced cardiotoxicity. Deterioration in such has been shown in animal studies by resonance spectroscopy. METHODS: We studied 62 patients, with a mean age of 13.5+/-5 years, 3.7+/-4.3 years after a cumulative anthracycline dose of 270+/-137 mg/m2. Normal echocardiographic findings had been elicited in 54 patients. The control group consisted of 28 healthy subjects aged 20+/-7 years. Resonance spectrums of the anterior left ventricular myocardium were obtained at 1.5 Tesla using an image-selected in vivo spectroscopy localization technique. RESULTS: The ratio of phosphocreatine to adenosine triphosphate after blood correction was 1.09+/-0.43 for the patients, and 1.36+/-0.36 (mean+/-SD) for controls (p=0.005), with a significantly reduced mean ratio even in the subgroup of patients with normal echocardiographic results (1.11+/-0.44 versus 1.36+/-0.36, p=0.01). The ratio did not correlate with the cumulative dose of anthracycline. The ratio of phosphodiester to adenosine triphosphate was similar in patients and controls (0.90+/-0.56 versus 0.88+/-0.62). CONCLUSIONS: In patients treated with anthracyclines in childhood, myocardial high-energy phosphate metabolism may be impaired even in the absence of cardiomyopathy. Our data support the concept that anthracycline-induced cardiotoxicity is not clearly dose dependent.

[Diffusion-weighted magnetic resonance tomography in diagnosis of encephalitis disseminata]. / Diffusionsgewichtete Magnetresonanztomographie in der Diagnostik der Encephalitis disseminata.

Magnetic resonance (MR) imaging is one of the best methods in diagnosis of multiple sclerosis, particularly in disclosure of active demyelinating lesions. Aim of this study was to compare diffusion weighted imaging and contrast enhancement in the detection of active lesions. A MR study with a contrast enhanced T1-weighted pulse sequence with magnetization transfer presaturation and a diffusion weighted echoplanar pulse sequence (b = 1000 s/mm2) was performed in 17 patients (11 women, 6 men) with multiple sclerosis. 29 of 239 lesions showed an increased signal intensity in diffusion weighted imaging, 24 lesions a contrast enhancement, but only 16 lesions were visible in both pulse sequences. In patients with short clinical symptomatology significant more lesions could be detected with diffusion-weighted pulse sequence in comparison to patients with long standing symptomatology showing more lesions with contrast enhancement. Hence it is likely, that both pulse sequences detect different histopathologic changes. The early detection of demyelinating lesions in diffusion weighted imaging is attributed to the extracellular edema, however the contrast enhancement is caused by a blood brain barrier abnormality. It can be expected that diffusion weighted imaging will have a high impact on imaging of multiple sclerosis not only in therapeutic trials, but also in clinical routine.

Myocardial technetium-99m-tetrofosmin and technetium-99m-sestamibi kinetics in normal subjects and patients with coronary artery disease.

UNLABELLED: This study was designed to compare the tracer kinetics between 99mTc-sestamibi and 99mTc-tetrofosmin in a heterogeneous group of 24 patients admitted for routine perfusion imaging. METHODS: Twelve patients were studied with 99mTc-tetrofosmin and 12 with 99mTc-sestamibi. In each group, six patients had a low likelihood for coronary artery disease, and six patients had angiographically proven coronary artery stenoses of > 75% or previous myocardial infarction. Analysis of myocardial and liver uptake and clearance as well as target-to-organ contrasts were performed with planar stress images. RESULTS: Myocardial uptake of 99mTc-tetrofosmin was higher from 5 min (0.37 +/- 0.12 counts/pixel x MBq-1, p = 0.008) to 60 min (0.32 +/- 0.10 counts/pixel x MBq-1, p = 0.04) compared to 99mTc-sestamibi. Biological half-life for 99mTc-tetrofosmin (278 +/- 32 min) in normal myocardium was significantly shorter (p = 0.008) than for 99mTc-sestamibi (680 +/- 45 min). Biological liver half-life for 99mTc-tetrofosmin (67 +/- 16 min) was also significantly shorter (p = 0.02) than for 99mTc-sestamibi (136 +/- 18 min). Heart-to-lung ratios for 99mTc-tetrofosmin (2.49 +/- 0.43 at 5 min to 2.66 +/- 0.55 at 60 min) and 99mTc-sestamibi (2.52 +/- 0.37 at 5 min to 2.95 +/- 0.50 at 60 min) were similar. Whereas heart-to-liver ratios for 99mTc-tetrofosmin (1.04 +/- 0.24 at 5 min, increasing to 1.51 +/- 0.44 at 60 min) were significantly higher from 30-60 min postinjection (p = 0.05 at 30 min to p = 0.02 at 60 min) compared to the 99mTc-sestamibi (0.83 + 0.16 at 5 min to 1.08 +/- 0.27 at 60 min). CONCLUSION: Technetium-99m-tetrofosmin displays a shorter myocardial half-life compared to 99mTc-sestamibi. The rapid liver clearance of 99mTc-tetrofosmin, combined with comparable myocardial retention, resulted in higher heart-to-liver ratios but similar heart-to-lung contrasts compared to 99mTc-sestamibi from 30-60 min.

Prednisone withdrawal late after adult liver transplantation reduces diabetes, hypertension, and hypercholesterolemia without causing graft loss.

We prospectively withdrew prednisone in 28 adult patients who had stable graft function more than 2 years after orthotopic liver transplantation (OLTx) and had been on 5 mg/d prednisone for at least 6 months. Prednisone was decreased from 5 mg/d to 2.5 mg/d for 1 month then stopped completely. Cyclosporine monotherapy was maintained at a level of approximately 200 ng/mL (TDX). Nineteen patients had prednisone withdrawn without complications. Four (14.2%) had modest elevations in liver function tests (two biopsy proven mild rejections and two were not biopsied). These four were treated with methylprednisolone boluses and then withdrawal of steroids again. Prednisone was restarted in five patients because of generalized fatigue and body aches (n = 4) and colitis (n = 1). Steroids later were successfully withdrawn in two of these patients. After prednisone withdrawal, three of five insulin-dependent diabetic patients were able to discontinue insulin therapy and their glycosylated hemoglobin levels improved. Four of fourteen hypertensive patients were able to discontinue antihypertensive medicines. Mean serum cholesterol decreased from 222.6 +/- 43.3 to 188.3 +/- 33.3 mg/dL (P < .001). The number of patients with serum cholesterol levels > 220 mg/dL decreased from 13 to 4. A control group of 24 patients maintained on 5 mg/d prednisone at least 2 years after liver transplantation also was studied. In this group during the study period, no diabetic became normoglycemic, no patient decreased their antihypertensive medicine, and the mean serum cholesterol levels did not change significantly. We conclude that prednisone withdrawal using cyclosporine monotherapy late after liver transplantation does not lead to graft loss and decreases the prevalence of diabetes, hypertension, and hypercholesterolemia. Symptoms occurring during withdrawal may be minimized by earlier or slower tapering.

A comparison of sevoflurane with halothane in outpatient adenotomy in children with mild upper respiratory tract infections.

STUDY OBJECTIVE: To investigate the efficacy and safety of sevoflurane compared with halothane in pediatric outpatient ear-nose-throat (ENT) surgery during the induction, maintenance, emergence, and recovery of anesthesia. DESIGN: Prospective, randomized, comparative, open-label study. SETTING: ENT operating room and postoperative recovery room at a university medical center. PATIENTS: 41 ASA status I and II children between the ages of 2 to 10 years, with mild upper respiratory tract infection (URI). INTERVENTIONS: Induction and maintenance of anesthesia with either sevoflurane or halothane for outpatient adenotomy, otomicroscopy, and myringotomy. MEASUREMENTS AND MAIN RESULTS: Induction (means +/- SEM) was significantly shorter in the sevoflurane group (2.6 +/- 0.2 minutes) than in the halothane group (3.2 +/- 0.2 minutes). There was no difference between the two groups with regard to complications that occurred during the induction and maintenance period. The time to emergence and recovery was significantly shorter with sevoflurane than with halothane (means +/- SEM; time to extubation 9.9 +/- 0.98 minutes vs. 13.4 +/- 1.06 minutes, time to eye opening 12.9 +/- 1.6 minutes vs. 24.5 +/- 1.8 minutes, command response time 20.7 +/- 2.5 minutes vs. 36.4 +/- 2.8 minutes). No difference in the incidence of complications during emergence and recovery was found. Evaluation of recovery as assessed by a modified Aldrete score showed that children who had received sevoflurane reached higher scores in the first 30 minutes following the discontinuation of the anesthetic. The Pain/Discomfort Scale demonstrated a difference in the sevoflurane group, with more children being agitated and restless. CONCLUSION: Sevoflurane provides a safe and rapid anesthetic induction with no differences in complications during the induction, maintenance, and emergence period. With sevoflurane, the time of emergence and recovery was significantly shorter. The characteristics of sevoflurane as evaluated in the present study make it a suitable anesthetic in pediatric outpatient surgery even in the presence of mild URI.

Metabolic complications after liver transplantation. Diabetes, hypercholesterolemia, hypertension, and obesity.

We retrospectively studied the incidence of diabetes, hypercholesterolemia, hypertension, and obesity in 123 consecutive adult liver transplant recipients (61 men and 62 women) who were alive at least 1 year after transplantation. We also studied the change in these metabolic complications in 61 patients who subsequently were able to be tapered to 5 mg prednisone per day. One year after transplantation--a point at which almost all patients were on maintenance immunosuppression and had stable graft function--the incidence of diabetes was 13% and hypertension was 69.1%. The overall incidence of hypercholesterolemia (serum cholesterol > 240 ng/ml) was 31% and was more frequent in women than in men (38.7% vs. 23.0%, P < 0.06). The incidence of obesity at 1 year was 41.9% in women and 39.3% in men. With tapering of prednisone from 10 mg to 5 mg per day in 61 patients, the mean serum cholesterol decreased from 224.6 +/- 65.2 mg/dl to 203.3 +/- 65.5 mg/dl, P < 0.005. With steroid tapering, 8 patients were able to discontinue antihypertensive medications and 4 were able to discontinue insulin treatment for diabetes. Five patients became obese during the steroid-tapering period. No patient developed irreversible rejection with steroid tapering and no immunologic graft losses occurred more than a year after transplantation. Nine patients who lived a year subsequently died. Of these, 7 patients were diabetic and 2 died of cardiac disease. We conclude that metabolic complications such as diabetes, hypertension, and hypercholesterolemia are common later after liver transplantation and that these may contribute to patient morbidity and mortality. In addition, we conclude steroid tapering to 5 mg/day does not lead to graft loss and may decrease the incidence and severity of late metabolic complications.

Quinapril therapy in patients with chronic mitral regurgitation.

Pre- and afterload reduction is known to have beneficial effects in patients with chronic mitral regurgitation. To date, no controlled study has been reported analyzing the long term influence of angiotensin-converting enzyme inhibitor treatment on patients with chronic mitral regurgitation. Therefore the aim of this study was to assess the effects of one year angiotensin-converting enzyme inhibition with quinapril on myocardial performance in patients with chronic mitral regurgitation. Twelve patients with moderate to severe isolated chronic mitral regurgitation and no coronary disease on coronary angiography were studied under control conditions and followed up until one year of quinapril therapy (10-20mg/day) using echocardiography and simultaneous right heart catheterization, and radionuclide ventriculography at rest and exercise. As the result of a significant pre- and afterload reduction after one year quinapril treatment regurgitant fraction fell from 0.43 +/- 0.10 at control before therapy to 0.25 +/- 0.08 (p = 0.0001), left ventricular end-diastolic volume was reduced from 146 +/- 26 to 109 +/- 24 ml/m2 (p = 0.0001) and end-systolic volume decreased from 63 +/- 43 to 47 +/- 29 ml/m2 (p = 0.02). Left ventricular ejection fraction at control averaged 0.59 +/- 0.20 at rest, increased to 0.65 +/- 0.21 with maximum exercise and was unchanged after one year quinapril therapy. After one year treatment left ventricular mass was reduced by 15% (p = 0.0004) and septal wall thickness decreased from 11.8 +/- 0.7 to 10.8 +/- 0.8 mm (p = 0.0006). Moreover, there was significant functional improvement of nearly one NYHA class after one year quinapril therapy. In conclusion, in patients with chronic mitral regurgitation long term angiotensin-converting enzyme inhibition with quinapril reduces regurgitation and decreases left ventricular size and mass thereby demonstrating functional improvement. In addition, these data suggest that angiotensin-converting enzyme inhibition might have the potential of delaying mitral valve surgery.

Beneficial effects of a single dose of quinapril on left ventricular performance in chronic mitral regurgitation.

To evaluate the effect of a single dose of the angiotensin-converting enzyme inhibitor quinapril on left ventricular (LV) performance and size in patients with moderate to severe chronic mitral regurgitation (MR), 12 patients with angiographically proven isolated MR grade II to III and no evidence of coronary artery disease were studied. In all patients a baseline right heart catheterization and simultaneous radionuclide angiogram were performed at rest and during supine exercise (maximum 100 W) as well as 2 hours after oral administration of 10 mg of quinapril. Quinapril reduced heart rate slightly and lowered mean blood pressure at rest and during maximal exercise (p < 0.05). Systemic vascular resistance at rest was decreased from 1,484 +/- 505 to 1,150 +/- 427 dynes s cm-5 and with maximal exercise from 999 +/- 455 to 734 +/- 395 dynes s cm-5 (p < 0.005). Pulmonary capillary arterial pressure at rest decreased from 13 +/- 6 to 10 +/- 4 mm Hg (p = 0.01) and during maximal exercise from 29 +/- 10 to 20 +/- 7 mm Hg (p = 0.001). LV end-diastolic volume at rest (146 +/- 26 ml/m2) decreased after administration of quinapril to 128 +/- 24 ml/m2 (p = 0.001) and was also reduced during exercise (p = 0.001). LV end-systolic volume decreased from 63 +/- 43 to 49 +/- 35 ml/m2 at rest (p = 0.001) and with maximal exercise from 56 +/- 49 to 44 +/- 39 ml/m2 (p < 0.01).(ABSTRACT TRUNCATED AT 250 WORDS)

Assessment of tissue viability with fluorine-18-fluoro-2-deoxyglucose (FDG) and carbon-11-acetate PET imaging.

Positron emission tomography allows noninvasive assessment of myocardial metabolic processes and perfusion. Myocardial F-18-2 deoxyglucose (FDG) uptake as assessed by PET imaging has been established as a metabolic tracer indicating myocardial viability, whereas N-13 ammonia uptake reflects myocardial perfusion. Furthermore, it has been shown that the clearance of C-11-acetate in normal and ischemic myocardium correlates closely with overall myocardial oxygen consumption (MVO2). Therefore, C-11-acetate PET imaging has been proposed as a tracer for myocardial viability. In contrast with myocardial FDG uptake, the clearance of C-11-acetate is independent from overall substrate utilization. In the present time the highly expensive PET studies should only be performed in patients with chronic advanced coronary artery disease resulting in severely impaired left ventricular function. Moreover, coronary anatomy has to be suitable for revascularization and there has to be an absence of reversible perfusion abnormalities on Tl-201-chloride reinjection SPECT scans. This review focuses on clinical studies employing C-11-acetate and FDG PET imaging in patients with advanced chronic coronary artery disease, acute myocardial infarction and after heart transplantation. Clinical applications of FDG and C-11-acetate PET imaging and prognosis of PET flow-metabolic imaging regarding recovery of myocardial function and clinical outcome after revascularization and thrombolytic therapy are summarized. Furthermore, PET results are compared to SPECT imaging with Tl-201 and Tc-99m-MIBI.

Metastatic renal cell carcinoma associated with acquired cystic kidney disease 15 years after successful renal transplantation.

Renal cell carcinoma (RCC) is a relatively uncommon cancer in renal transplant patients. From 1968 to 1987, 101 cases of RCC of native kidneys have been reported to the Cincinnati Transplant Tumor Registry. We describe here a case of metastatic RCC associated with acquired cystic kidney disease (ACKD) 15 years after successful renal transplantation. The patient presented with a subcutaneous nodule, which led to discovery of a large primary tumor in the left kidney. ACKD was present in the atrophic right kidney. The reported cases of ACKD-associated RCC in renal transplant recipients were reviewed. Most of these cases are middle-aged men with a long posttransplant course, good graft function, and usage of azathioprine and prednisone as immunosuppressive agents. ACKD can develop or persist and progress to RCC many years after successful renal transplantation. Transplant patients with flank pain, hematuria, or other suspicious symptoms should have imaging studies of their native kidneys.

Frequency and spectrum of microorganisms isolated from biopsy specimens in chronic colitis.

In 109 patients with chronic diarrhea colonic biopsies were examined for the presence of specific microorganisms. A positive result was obtained in 48% of patients with ulcerative colitis, 50% with Crohn's disease, 21% with non-specific colitis and 36% with non-specific proctitis. Chlamydiae were found most frequently in all groups. Obligate enteropathogenic bacteria were isolated in only three cases of nonspecific colitis. Of the facultatively enteropathogenic organisms Klebsiella and Pseudomonas aeruginosa were isolated in 31% and 24%, respectively, of patients with ulcerative colitis, in 21% and 7% of patients with Crohn's disease, and in 10% and 6% of patients with non-specific colitis. Whereas chlamydial proctitis is a well-known disease, the results of this study point also to a possible pathogenic role of chlamydiae in the proximal colon. Facultatively enteropathogenic organisms causing acute diarrhea might have aetiologic relevance in some cases of chronic non-specific colitis.