ABSTRACT
Here, a study of NMOSD in Central America and the Caribbean with a multinational collaborative, multicentric and descriptive approach involving 25 institutions from 9 countries is presented. Demographics, clinical manifestations, expanded disability scale status (EDSS), brain and spinal cord MRI, serological anti-AQP4-IgG and anti-MOG-IgG antibodies, and cerebrospinal fluid (CSF) oligoclonal bands were included. A central serological repository utilized the cell-based assay. The specimens outside of this network employed diverse methodologies. Data were collected at the Gorgas Commemorative Institute of Health Studies (ICGES), Panama, and included 186 subjects, of which 84% were females (sex ratio of 5.6:1). Mestizos constituted 72% of the study group. The median age was 42.5 years (IQR: 32.0-52.0). Associated autoimmune diseases (8.1%) were myasthenia gravis, Sjögren's syndrome and systemic lupus erythematosus. The most common manifestation was optic neuritis-transverse myelitis (42.5%). A relapsing course was described in 72.3% of cases. EDSS scores of 0-3.5 were reported in 57.2% of cases and higher than 7.0 in 14.5%. Positive anti-AQP4-IgG antibody occurred in 59.8% and anti-MOG-IgG antibody in 11.5% of individuals. Antibody testing was lacking for 13.4% of patients. The estimated crude prevalence of NMOSD from Panama and the Dominican Republic was 1.62/100,000 (incidence of 0.08-0.41) and 0.73/100,000 (incidence 0.02-0.14), respectively. This multinational study contributes additional insights and data on the understanding of NMOSD in this Latin American region.
ABSTRACT
BACKGROUND/OBJECTIVES: Acute graft-versus-host disease (aGVHD) is a serious condition after allogeneic hematopoietic stem cell transplantation (HSCT), frequently involving skin, gut, and liver. It can be difficult to diagnose early, yet this is vital for adequate management. We sought to identify initial clinical and histopathological features in children with suspected GVHD and the association with clinical course and outcomes. METHODS: Retrospective study of patients with skin biopsies for suspected aGVHD from 2006 to 2016. We collected demographic and clinical information, histologic, and immunohistochemical (IHC) findings, and outcomes during follow-up. Bivariate and multivariate analyses were done to identify risk factors associated with remission, development of severe/life-threatening aGVHD, and mortality. RESULTS: We included 42 patients, 15 females. Skin manifestations occurred 51 days (median) after HSCT. On biopsy, 76.2% had mild (stage 1-2) skin aGVHD; during the course of the disease, severity and systemic involvement increased to global grade III/IV in 66.6%. All patients received treatment; 15 are in remission from aGVHD and 23 have died. Histologic features were diagnostic in 83.3%. On bivariate and multivariate analysis, we identified initial clinical and histologic findings that were associated with the measured outcomes: odds of remission from aGVHD were increased when focal vacuolar changes were found on skin biopsy (OR 6.028; 95%CI:1.253-28.992) but decreased by initial hepatic aGVHD (OR 0.112; 95%CI: 0.017-0.748); severe/life-threatening aGVHD was associated with initial gastrointestinal aGVHD (OR 6.054; 95%CI:1.257-29.159); and odds of mortality were decreased with male donor (OR 0.056; 95%CI:0.004-0.804), nulliparous female donor (OR 0.076; 95%CI:0.009-0.669), and focal vacuolar changes on skin biopsy (OR 0.113; 95%CI:0.017-0.770). CONCLUSIONS: We found novel indicators predictive of remission, severity, and mortality in children with aGVHD. Further studies of this condition in children are needed.
Subject(s)
Graft vs Host Disease , Hematopoietic Stem Cell Transplantation , Acute Disease , Child , Female , Graft vs Host Disease/diagnosis , Hematopoietic Stem Cell Transplantation/adverse effects , Humans , Male , Retrospective Studies , Risk FactorsABSTRACT
La Enfermedad Renal Crónica Terminal (ERCT) se ha convertido en un problema de Salud Pública, debido al número elevado de pacientes que son diagnosticados, día a día. Las complicaciones respiratorias, son comunes en los pacientes con ERCT, y pueden ser detectados hasta en el 70% de los casos, siendo el Derrame Pleural una de las más frecuentes. El objetivo del estudio fue determinar la incidencia de derrame pleural en los pacientes ERCT estadio V, que ingresaban por primera vez a diálisis peritoneal intermitente. Se realizó un estudio observacional, descriptivo, retrospectivo, longitudinal, de pacientes de nacionalidad salvadoreña, mayores de 12 años con diagnóstico de Enfermedad Renal Crónica Terminal de primera vez, en el período comprendido del 1 de Junio 2016 al 30 de Junio 2017, que recibieron tratamiento dialítico a través de catéter rígido y que desarrollaron Derrame Pleural; se determinó el perfil clínico, así como las características bioquímicas del líquido pleural. Se incluyeron 489 pacientes, 306 hombres (62.57%), y 183 mujeres (37.43%), con una edad media de 55.18 ± 13.78 años. 56 (11.5%) pacientes desarrollaron Derrame pleural, se realizó toracocentesis diagnóstica al 8.92%, de los cuales 60% era compatible con trasudado y 40 % con exudado (según criterios de Light). Los síntomas más frecuentes fueron: disnea y edema de miembros inferiores. Las comorbilidades concomitantes encontradas fueron HTA, Anemia y sobrecarga hidrica. Además niveles de albumina por debajo de 3.4 gr/dl se registraron en 68% de estos pacientes. Muchos de los pacientes en estudio presentaron múltiples comorbilidades, y desarrollaron derrame pleural en los primeros 3 meses de inicio de terapia dialítica, el hemitórax derecho fue el más afectado; en el perfil bioquímico predominó el trasudado. Los síntomas más frecuentemente presentados fueron: disnea (33%), seguido del edema de miembros inferiores (26%) y tos 20.5%. Los mayores de 50 años tuvieron más predisposición. La incidencia de derrame pleural en un año de seguimiento fue de 11.5%