Efficacy of a cartoon and photograph montage storybook in preparing children for voiding cystourethrogram.

BACKGROUND: Undergoing voiding cystourethrogram (VCUG) can be distressing for children. OBJECTIVE: To assess the efficacy of a cartoon and photograph montage storybook in preparing children for VCUG. MATERIALS AND METHODS: Outpatient children (ages 2-14 years) who had VCUGs between December 2011 and June 2012 were randomly assigned to two groups; one group received the storybook a week before the procedure. Parents and guardians were asked to complete an anonymous survey rating their child's tolerance of the exam from 1 to 5, worst to best, immediately after VCUG. The VCUG technologist also rated the child's tolerance. RESULTS: Children prepared for VCUG with the storybook had less distress than those without. Results were analyzed by Cochran-Mantel-Haenszel and Cochran-Armitage Trend exact tests, a P value of both tests of 0.0092 indicating a statistically significant difference between the tolerance scores of children prepared with the storybook and those without. Effects of gender and history of VCUG were not statistically significant. Two-thirds of all children had no other source of information. CONCLUSION: The cartoon and photograph montage storybook format of preparing children for VCUG was effective in increasing their tolerance for the procedure. The storybook should be mailed out in advance because the majority of families did not pursue information on preparing their children for VCUG.

Unexpected MRI findings in clinically suspected Legg-Calvé-Perthes disease.

BACKGROUND: In the setting of clinically suspected Legg-Calvé-Perthes (LCP) disease and negative/equivocal radiographs, contrast-enhanced MRI can be performed to confirm the diagnosis. OBJECTIVE: To determine the frequency of unexpected causes of hip pain as identified by MRI in children with clinically suspected LCP disease and negative/equivocal radiographs. MATERIALS AND METHODS: All pediatric contrast-enhanced MRI examinations of the pelvis and hips performed between January 2000 and February 2009 to evaluate for possible LCP disease in the setting of negative/equivocal radiographs were identified. MRI examinations performed to evaluate for secondary avascular necrosis were excluded. Imaging reports were retrospectively reviewed for unexpected clinically important causes of hip pain. RESULTS: Thirty-six pediatric patients underwent contrast-enhanced MRI examinations for clinically suspected LCP disease in the setting of negative/equivocal radiographs. Twenty-two (61%) imaging studies were normal, while four (11%) imaging studies demonstrated findings consistent with LCP disease. Ten (28%) imaging studies revealed unexpected clinically important causes of hip pain, including nonspecific unilateral joint effusion and synovitis (n=7, juvenile chronic arthritis was eventually diagnosed in 3 patients), sacral fracture (n=1), apophyseal injury (n=1), and femoral head subluxation (n=1). CONCLUSION: MRI frequently reveals unexpected clinically important causes of hip pain in children with suspected LCP disease and negative/equivocal radiographs.

Common and uncommon vascular rings and slings: a multi-modality review.

Vascular rings and pulmonary slings are congenital anomalies of the aortic arch/great vessels and pulmonary arteries, respectively, that commonly present early during infancy and childhood with respiratory and/or feeding difficulties. The diagnosis of these conditions frequently utilizes a multi-modality radiological approach, commonly utilizing some combination of radiography, esophagography, CT angiography and MR angiography. The purpose of this pictorial review is to illustrate the radiological findings of common and uncommon vascular rings and pulmonary slings in children using a state-of-the-art multi-modality imaging approach.

Cardiovascular magnetic resonance imaging of hypoplastic left heart syndrome in children.

Cardiovascular magnetic resonance imaging (CMR) plays an important complementary role to echocardiography and conventional angiography in the evaluation of hypoplastic left heart syndrome. This imaging modality is particularly useful for assessing cardiovascular postsurgical changes, extracardiac vascular anatomy, ventricular and valvular function, and a variety of complications. The purpose of this article is to provide a contemporary review of the role of CMR in the management of untreated and surgically palliated hypoplastic left heart syndrome in children.

Role of CT in the evaluation of congenital cardiovascular disease in children.

OBJECTIVE: The purpose of this review is to describe the role of CT in the evaluation of congenital cardiovascular disease in children. Clinical indications, imaging techniques, and illustrations of relevant conditions are presented. CONCLUSION: CT accurately depicts many forms of congenital cardiovascular disease in children. It is particularly valuable in the assessment of extracardiac vascular anomalies and in the evaluation of postoperative complications.

MRI of Legg-Calve-Perthes disease.

OBJECTIVE: Legg-Calvé-Perthes disease is a common cause of hip pain in children that may be initially clinically and radiographically difficult to diagnose and stage. The purpose of this article is to describe and illustrate the various MRI appearances of this condition. CONCLUSION: MRI may show proximal femoral abnormalities before radiography in the setting of Legg-Calvé-Perthes disease, allowing appropriate diagnosis and prompt treatment. MRI can also assess for revascularization, healing, and multiple complications.

Imaging of pulmonary venous developmental anomalies.

OBJECTIVE: The purpose of this article is to review pulmonary venous embryology and to present the imaging findings of a variety of pulmonary venous developmental anomalies, including total anomalous pulmonary venous connection, partial anomalous pulmonary venous connection, pulmonary vein stenosis and hypoplasia/atresia, and cor triatriatum. CONCLUSION: There are numerous developmental pulmonary venous anomalies. Although these conditions have traditionally been evaluated with echocardiography and angiography, they can be accurately diagnosed using both MRI and MDCT.

Interrupted aortic arch: spectrum of MRI findings.

OBJECTIVE: The objective of this article is to review the types, pathogenesis, MRI appearance, treatment, and prognosis of interrupted aortic arch (IAA). CONCLUSION: IAA is a rare congenital vascular anomaly. Although this entity has been evaluated traditionally with echocardiography and angiography, MRI can accurately diagnose and characterize the various forms of IAA and associated congenital heart defects. MRI can also be used to evaluate for postoperative complications after repair.

Current state of biosimilars in Mexico: The position of the Mexican College of Rheumatology, 2016. / Escenario actual de los medicamentos biocomparables en México: posicionamiento del Colegio Mexicano de Reumatología, 2016.

The present document is a position statement of the Mexican College of Rheumatology on the use of biosimilars in rheumatic diseases. This position considers that biosimilars should be considered as interchangeable, that automatic substitution without previous notice in stable patients during follow-up is not ethical, that the approval of a biosimilar should only be given after exhaustive review of preclinical and clinical data marked by Mexican regulations, that it should be clearly stated in the nomenclature of biologic drugs which is the innovator and which is the biosimilar, that it is not correct to choose a biosimilar as treatment based only on economic reasons or extrapolate indications based only on the approval of the innovator and in the absence of safety and efficacy data for the biosimilar.

Magnetic resonance imaging of mediastinal vessels.

Advances in technology have led to a changing role for MRI in the evaluation of the thoracic vasculature in children. MRI, especially with 3D gadolinium-enhanced MR angiography, can clearly demonstrate the morphology of the aortic and pulmonary vascular supply. In patients with nonvalvar obstructive lesions of the aorta (i.e., coarctation, interruption of the aortic arch, and supravalvar stenosis), MRI can reliably assess the site and extent of the obstruction. Similarly, MRI can depict the morphology of the central pulmonary arteries and aortopulmonary collateral vessels in patients with obstructive lesions of the pulmonary artery. MRI is also useful in assessing the course of the aorta and pulmonary arteries in patients with suspected vascular rings. The result is that MRI can supplement information obtained from echocardiography and angiography and reduce the need for angiography.

Escenario actual de los medicamentos biocomparables en México: posicionamiento del Colegio Mexicano de Reumatología, 2016 / Current state of biosimilars in Mexico: The position of the Mexican College of Rheumatology, 2016

El presente documento refleja el posicionamiento del Colegio Mexicano de Reumatología y de expertos sobre el uso de medicamentos biocomparables (conocidos como biosimilares en otros países) en enfermedades reumáticas. En resumen, este posicionamiento considera que si bien los biocomparables deben considerarse como intercambiables, no es ética la sustitución automática de medicamentos sin previo aviso en pacientes estables durante el seguimiento; que la aprobación de un biocomparable debe llevarse a cabo solo después de revisar exhaustivamente las pruebas preclínicas y clínicas señaladas por la ley mexicana; que debe modificarse la forma de enfatizar en su nomenclatura que se trata de un medicamento biotecnológico innovador o biocomparable de manera clara; que no es adecuado elegir como tratamiento un biocomparable basándose únicamente en aspectos económicos ni realizarse la extrapolación de indicaciones basándose únicamente en la aprobación obtenida por el innovador y en ausencia de datos de seguridad y eficacia para el biocomparable

The present document is a position statement of the Mexican College of Rheumatology on the use of biosimilars in rheumatic diseases. This position considers that biosimilars should be considered as interchangeable, that automatic substitution without previous notice in stable patients during follow-up is not ethical, that the approval of a biosimilar should only be given after exhaustive review of preclinical and clinical data marked by Mexican regulations, that it should be clearly stated in the nomenclature of biologic drugs which is the innovator and which is the biosimilar, that it is not correct to choose a biosimilar as treatment based only on economic reasons or extrapolate indications based only on the approval of the innovator and in the absence of safety and efficacy data for the biosimilar

Constitutive phosphorylation of interferon receptor A-associated signaling proteins in systemic lupus erythematosus.

BACKGROUND: Overexpression of type I interferon (IFN-I)-induced genes is a common feature of systemic lupus erythematosus (SLE) and its experimental models, but the participation of endogenous overproduction of IFN-I on it is not clear. To explore the possibility that abnormally increased IFN-I receptor (IFNAR) signaling could participate in IFN-I-induced gene overexpression of SLE, we examined the phosphorylation status of the IFNAR-associated signaling partners Jak1 and STAT2, and its relation with expression of its physiologic inhibitor SOCS1 and with plasma levels of IFNα and IFN-like activity. METHODOLOGY/PRINCIPAL FINDINGS: Peripheral blood mononuclear cells (PBMC) from SLE patients with or without disease activity and healthy controls cultured in the presence or in the absence of IFNß were examined by immunoprecipitation and/or western blotting for expression of the two IFNAR chains, Jak1, Tyk2, and STAT2 and their phosphorylated forms. In SLE but not in healthy control PBMC, Jak1 and STAT2 were constitutively phosphorylated, even in the absence of disease activity (basal pJak1: controls vs. active SLE p<0.0001 and controls vs. inactive SLE p = 0.0006; basal pSTAT2: controls vs. active and inactive SLE p<0.0001). Although SOCS1 protein was slightly but significantly decreased in SLE in the absence or in the presence of IFNß (p = 0.0096 to p<0.0001), in SOCS1 mRNA levels were markedly decreased (p = 0.036 to p<0.0001). IFNß induced higher levels of the IFN-I-dependent MxA protein mRNA in SLE than in healthy controls, whereas the opposite was observed for SOCS1. Although there was no relation to increased serum IFNα, active SLE plasma could induce expression of IFN-dependent genes by normal PBMC. CONCLUSIONS/SIGNIFICANCE: These findings suggest that in some SLE patients IFN-I dependent gene expression could be the result of a low IFNAR signaling threshold.

Design and function of triplex hairpin ribozymes.

Triplex ribozymes allow for the individual activity of multiple trans-acting ribozymes producing higher target cleavage relative to tandem-expressed RZs. A triplex expression system based on a single hairpin ribozyme for the multiple expression (multiplex) vectors can be engineered to target RNAs with single or multiple antisense-accessible sites. System construction relies on triplex expression modules consisting of hairpin ribozyme cassettes flanked by ribozymes lacking catalytic domains. Multiplex vectors can be generated with single or multiple specificity by tandem cloning of triplex expression modules. Triplex ribozymes are initially tested in vitro using cis- and trans-cleavage assays against radioactive-labeled targets. In addition, triplex ribozymes are tested for cis and trans cleavage in vivo by transfection in cultured cells followed by ribonuclease protection assays (RPAs) and RT-PCR. The use of triplex configurations with multiplex ribozymes will provide the basis for the development of future RZ-based therapies and technologies.

MR findings of endocardial fibroelastosis in children.

BACKGROUND: Endocardial fibroelastosis (EFE) is characterized by a diffuse white fibrous tissue lining the endocardium. The diagnosis is difficult to establish because clinical symptoms and electrocardiographic findings are nonspecific. Surgical resection of EFE requires the establishment of the diagnosis and delineation of the extent of the fibrotic changes. OBJECTIVE: To describe the use of MRI in the assessment of EFE in children. MATERIALS AND METHODS: Three children after surgery for aortic stenosis who were suspected of having EFE were evaluated by echocardiography and MRI. The MR evaluation consisted of black-blood, triple IR, bright-blood, perfusion and myocardial delayed-enhancement sequences. EFE was confirmed at surgery in all patients. RESULTS: Echocardiograms demonstrated vigorous systolic function but substantial diastolic dysfunction of the left ventricle in all. Mild endocardial brightening of the anterior septum, anterior wall, or papillary muscles was present in two. No study was thought to be diagnostic of endocardial fibrosis. On MRI EFE manifested at the endocardial surface as a rim of hypointense signal in the perfusion sequences and as a rim of hyperintense signal in the myocardial delayed-enhancement sequences. The black-blood, triple IR, and bright-blood sequences were not diagnostic. CONCLUSION: The diagnosis of EFE is difficult to establish by echocardiography. MRI using perfusion and myocardial delayed enhancement can be useful in establishing the diagnosis.

Markedly hypoplastic circumflex retroesophageal right aortic arch: MR imaging and surgical implications.

BACKGROUND: Circumflex retroesophageal right aortic arch with a markedly hypoplastic retroesophageal segment is a rare anomaly of the aortic arch. Adequate surgical management relies on precise diagnosis, which might not be feasible with echocardiography. OBJECTIVE: To demonstrate the utility of MR imaging in establishing the diagnosis and to describe potential pitfalls in making the diagnosis. MATERIALS AND METHODS: Three patients with a circumflex retroesophageal right aortic arch with a markedly hypoplastic retroesophageal segment were retrospectively evaluated. All patients underwent evaluation by echocardiography and MR imaging. The MR imaging consisted of 3-D MR angiography in two patients and fast gradient recalled echo with cardiac-triggered segmented acquisition in one patient. Surgical confirmation was obtained on all three patients. RESULTS: The arch anatomy was accurately depicted in all three patients by MR imaging and in none of the patients by echocardiography. CONCLUSION: MR imaging is extremely useful in establishing the diagnosis of markedly hypoplastic retroesophageal circumflex right aortic arch and thus helps in surgical planning.

CT of tracheal agenesis.

BACKGROUND: Tracheal agenesis is a rare and usually lethal anomaly. In the past, opaque contrast medium was injected via the esophagus to demonstrate the anatomy. OBJECTIVE: To demonstrate the utility of helical and multidetector CT in delineating the aberrant anatomy in newborns with tracheal agenesis. MATERIALS AND METHODS: Four newborns with tracheal agenesis were identified from three institutions. Imaging studies and medical records were reviewed. Each child was imaged with chest radiography. One child was imaged on a single-detector helical CT scanner and the other three on multidetector scanners. RESULTS: Helical and multidetector CT with 2D and 3D reconstructions clearly delineated the aberrant tracheobronchial and esophageal anatomy in each infant. Minimum intensity projection reformatted CT images were particularly helpful. One infant each had type I and type II tracheal agenesis. Two infants had type III tracheal agenesis. All four infants died. CONCLUSION: CT is a useful tool for delineating the aberrant anatomy of newborns with tracheal agenesis and thus helps in making rational clinical decisions.

Stage-I osteochondritis dissecans versus normal variants of ossification in the knee in children.

BACKGROUND: Juvenile osteochondritis dissecans (OCD) has a better prognosis than the adult type. OBJECTIVE: We postulated that the excellent prognosis of juvenile OCD could be explained, at least in part, by the erroneous diagnosis of some developmental variants of ossification as stage-I OCD. MATERIALS AND METHODS: Knee MRIs of 38 children, ages 7.5-17.7 years (mean and median age 13 years), were retrospectively reviewed to look for features that might separate normal variants of ossification from stage-I OCD. These included age, gender, site, configuration of the lesion, residual cartilaginous model and presence of edema. RESULTS: Twenty-three patients (32 condyles) had ossification defects with intact articular cartilage suggestive of stage-I lesions. No stage-II lesions were seen in the posterior femoral condyles. Accessory ossification centers were seen in 11/16 posterior condyles and 3/16 central condyles. Spiculation of existing ossification was seen in 12/16 posterior condylar lesions and 1/16 central condyles. There was a predominance of accessory ossifications and spiculations in the patients with 10% or greater residual cartilaginous model. No edema signal greater than diaphyseal red-marrow signal was seen in the posterior condyles. Clinical follow-up ranged from 0.5 to 38 months, with clinical improvement in 22 out of 23 patients. CONCLUSION: Inclusion of normal variants in the stage-I OCD category might explain, in part, the marked difference in published outcome between the juvenile and adult forms of OCD. Ossification defects in the posterior femoral condyles with intact overlying articular cartilage, accessory ossification centers, spiculation, residual cartilaginous model, and lack of bone-marrow edema are features of developmental variants rather than OCD.

Myofibromatosis: imaging characteristics.

BACKGROUND: Infantile myofibromatosis is the most common fibrous tumor of infancy. It can involve the skin, muscle, bone, and viscera. This uncommon entity is subdivided into solitary and multicentric forms, with or without visceral involvement. OBJECTIVE: To describe the imaging characteristics of extracranial myofibromatosis. MATERIALS AND METHODS: Six infants, aged 1 day-1 week, were evaluated by imaging. All six patients had evaluation of one of the masses by US; four patients had CT evaluation of at least one of the masses; and five patients had evaluation by MRI. RESULTS: The US appearance of the myofibromas included a mass with a purely anechoic center with a thick wall, a mass with a partially anechoic center, and a mass without anechoic components. On enhanced CT, the masses had lower or similar attenuation compared to adjacent muscle, with some masses exhibiting peripheral enhancement. The MR appearance consisted of low signal on T1-weighted imaging. On T2-weighted imaging, two had low signal of the center and the other three had high signal. All masses showed peripheral enhancement after gadolinium administration. CONCLUSIONS: Myofibromas have variable appearance on US, with a mass with an anechoic center being the most common feature. On CT, the mass can exhibit peripheral enhancement, calcifications, and erosion of adjacent bone. The MR appearance consisted of low signal on T1-weighted imaging and high or low signal of the center on T2-weighted imaging. All masses showed peripheral enhancement after gadolinium administration.

Pigmented villonodular synovitis: MR imaging in pediatric patients.

OBJECTIVES: To describe the usefulness of gradient-echo imaging and contrast-enhanced MR imaging in diagnosing and assessing the extent of pigmented villonodular synovitis (PVNS) in pediatric patients. PATIENTS AND METHODS: Retrospective analysis of five pediatric patients (median age 14 years) with PVNS of the knee examined with a 1.5-T unit. Signal characteristics were obtained comparing T1- and proton-density (PD)-weighted imaging with gradient-echo imaging and enhanced imaging. The extent of the disease was assessed using the arthroscopic approach. Signal intensity ratios were statistically analyzed. RESULTS: Compared to PD- and T1-weighted imaging sequences, gradient-echo imaging provides superior depiction of the extent of the disease due to signal decay (T2*-effect) of hemosiderin-laden thickened synovium and masses. Inflamed synovium with low hemosiderin deposition was identified on enhanced imaging. Beside the suprapatellar bursa and Hoffa's fat pad, the area posterior to the cruciate ligaments is commonly involved. No bony abnormalities were present. CONCLUSION: Gradient-echo imaging together with enhanced imaging is useful in diagnosing and assessing the extent of PVNS in pediatric patients. A low signal mass behind the cruciate ligaments may represent an important diagnostic feature. Bony abnormalities were always absent.

Focal pyomyositis of the perisciatic muscles in children.

OBJECTIVE: The purpose of this report is to describe the role of MR imaging in the diagnosis of focal pyomyositis surrounding the sciatic nerve in children. CONCLUSION: In the absence of joint effusion on sonography, MR imaging should be considered in pediatric patients who present with a febrile illness and incapacitating pelvic pain.