Keywords: Neuromyelitis optica, Devic's syndrome, Devic's disease, Myelitis, Optic neuritis, There is some evidence that the term 'neuro-myélite' was chosen by Devic and Gault in La neurópticomielitis aguda (Enfermedad de Devic). .. Dec 20 [Epub ahead of print] [PubMed] [CrossRef]; Jarius S, Paul F, Franciotta D. trastornos del sueño y cambios histológicos cerebrales consistentes con la enfermedad. . Neuromyelitis optica (Devic's syndrome). Epub /12/ eng. enfermedad ahora conocida como neuromielitis óp- La neuromielitis óptica ( NMO) o enfermedad de Devic es un trastorno autoinmune.
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Resumen. Las enfermedades desmielinizantes pueden ser origen de catástrofes J. Sellner, B. Hemmer, M. MühlauThe clinical spectrum and immunobiology of parainfectious neuromyelitis optica (Devic) syndromes [Epub ahead of print]. Clinical observation and treatment approaches of child Devic opticomyelitits in 1Neuroimmunology, Fundacion Para la Lucha Contra las Enfermedades. Sep 8, Viernes 13 - Lugones C ENFERMEDAD DE DEVIC. ¿Qué faltaba, cited: CASOS CLINICOS EN INFECCIOSAS leer epub leer epub?.
The existence of autoantibody targeting aquaporin-4 AQP4-Ab indicates the involvement of humoral immunity in the pathogenesis of this disease. Although T follicular helper cells have been proved to regulate B cell activation and antibody production, the role of these cells in NMOSD and the impact of RTX treatment on these cells remain less understood. Furthermore, B cell depletion with RTX treatment inhibited the expansion of cTfh cells, and these effects were achieved through eliminating ILproducing B cells and blocking the direct contact between cTfh cells and B cells. Introduction Neuromyelitis optica spectrum disorder NMOSD is a rare inflammatory demyelinating disease of the central nervous system CNS characterized by recurrent attacks of optic neuritis and transverse myelitis 1. In the past decades, NMOSD was believed to be a variant of multiple sclerosis MS based on the overlapping clinical and magnetic resonance imaging phenotypes.
Laboratory investigation was performed in nine children; no signs of myelosuppression or liver test abnormalities were reported.
All children but one complied to the Dutch vaccination schedule including vaccination against diphtheria, tetanus, pertussis, polio, Haemophilus influenzae type b, pneumococcal disease, measles, mumps, rubella, from which the last three mentioned are live attenuated vaccines , none of the vaccinated children experienced serious side effects. One child was considered excessively active, according to the parents in line with a suspected attention deficit disorder, but medical consultation had not been taken place to evaluate this problem.
This may reflect maternal fear for potential detrimental effects to the infant. However, in line with the current consensus, our findings underline the recommendation that mothers using thiopurines should also be encouraged to breastfeed their child. Assessment was performed by questionnaires at the mean ages of 3. No differences were found in infection rates between the two groups, and all infections reported were childhood diseases as commonly seen in the general population.
In nine children, laboratory investigation was done for different indications; no signs of myelosuppression or liver test abnormalities were reported.
Our results show that exposure to intrauterine thiopurines is not associated with increased susceptibility to infection or immunodeficiency in childhood. Furthermore, no relevant differences were found between children exposed to intrauterine thiopurines and the reference group on global medical and psychosocial health status.
This is in line with the parents reported perception of their child's general development. Our results have to be confirmed in studies with larger number of infants.
There is no direct medical reason to change thiopurine medication in IBD patients just because of pregnancy. The diagnosis was confirmed during cardiac catheterization with simultaneous TTE.
The stretch diameter of the defect was measured with a calibrated balloon, locating the interatrial defect as precisely as possible.
In most cases the procedure was indicated in patients with a left-right short circuit and with echocardiographic tracings showing right ventricular volume overload. A stretch diameter of less than 38 mm with the edges of the septum facing toward the mitral valve, coronary sinus, and left superior pulmonary vein were required for inclusion, with a minimum edge length of 6 mm, except in the case of the aortic edge. The procedure was also indicated in patients with transient cerebrovascular attacks TIA , with ASD and non-significant left-right short circuit or with a patent foramen oval PFO with right-left short circuit evident during performance of the Valsalva maneuver.
Graph of the patients studied. In all cases, prior to catheterization, physical examination, echocardiogram, chest radiograph, and TTE were performed. In 6 patients, percutaneous closure of a residual ASD was indicated following a surgical procedure in 2 patients following anatomical correction of the transposition of large arteries, and in the other 4 after surgical closure of an ASD. In another 5 patients the other interventions were performed simultaneously in the setting of coexisting lesions: pulmonary valvuloplasty for severe pulmonary valve stenosis in 4 patients, and in another patient closure of persistent ductus arteriosus.
In those patients with ischemic cardiopathy, complex cardiac defects, or abnormal drainage of the pulmonary veins the procedure was not indicated. The diameter of the device was the same or greater than the stretch diameter of the defect which, in turn, was greater than the diameter of the ASD as seen on TEE ratio 1. Figure 2 is a device with 2 discs made of fine Nitinol wire mesh with a thermal memory and auto-centering capability.
Both discs are joined by a 4 mm waist. In the devices 4 mm to 10 mm in diameter, the left atrial and right atrial discs are 12 mm and 8 mm larger, respectively, than the waist. In the 11 mm to 40 mm diameter devices, the left atrium disc is 14 mm larger and the right atrial disc is 10 mm larger than the waist.
Three polyester patches, located in the discs and in the waist of the device, cause coagulation and the obstruction of the defect. The device is introduced loaded and with a delivery guide via a 6F to 12F sheath which is placed in the left atrium via the ASD. One advantage of the ASO is the possibility of recovering and repositioning the device, multiple times if necessary, before deploying the device.
A: The disc on the left atrial side is open. The right disc is inside the sheath still connected to the deployment system.
B: The device in the correct position after deployment. Both consist of a double umbrella framework with 4 nitinol arms that are covered with a square polyester patch. The Starflex type has a system of self-centering threads that connect the arms of both umbrellas. The Starflex device SF in the correct position after deployment.
Procedure protocol In the pediatric patients all procedures were performed under general anesthesia and intubation, and in some of the adult patients, procedures were performed with only sedation and local anesthetic.
Antibiotic prophylaxis cephazoline was administered and the right femoral vein was punctured. TEE was performed and the diameter of the defect was measured in different projections, analyzing the borders anterior, posterior, inferior, and superior and the total length of the septal defect. Diagnostic cardiac catheterization was then performed and the pressures of both atria, the right ventricle, and the pulmonary artery were measured.
In patients older than 40 years of age, coronary angiography was performed. Introduction into the left atrium was accomplished coaxially via catheter exchange with a guide wire located in the left superior pulmonary vein.
The balloon was filled in the left atrium with contrast material diluted with physiological serum until it reached a volume greater than the diameter of the defect, and was slowly removed under TEE-color Doppler control until the short circuit disappeared entirely. The balloon was then slowly deflated and drawn toward the right atrium until the jump of the balloon to the right atrium was noted.
The stretch diameter was that of the balloon when the balloon passed from the left to the right atrium, occluding the ASD.
Once the balloon was outside the patient, the diameter of the balloon was tested by inflating it with the same amount in millimeters of contrast solution, assuming the stretch diameter to be that of the orifice through which the balloon passed with a certain degree of difficulty, according to the template recommended by the manufacturer.
The closure device ASO was chosen so that its diameter was the same or 1 to 4 mm greater than the stretch diameter of the defect. When the Starflex device was used, the ratio was with respect to the ASD or sufficiently large to block the entire aneurysm wall. The distal disk of the ASO was opened in the left atrium and, later, the proximal disk was opened in the right atrium. When implantation was incorrect on TEE, the device was removed within the vein and the maneuver was repeated as many times as necessary.
Device stability was controlled by means of the Minnesota maneuver push and drawing of the device toward both atria without deployment. Intense sport activity was discouraged for a month following the procedure and bacterial endocarditis prophylaxis was recommended for 6 months or until complete closure of the ASD was achieved.