Effect of Media Modified To Mimic Cystic Fibrosis Sputum on the Susceptibility of Aspergillus fumigatus, and the Frequency of Resistance at One Center.

Studies of cystic fibrosis (CF) patient exacerbations attributed toPseudomonas aeruginosainfection have indicated a lack of correlation of outcome within vitrosusceptibility results. One explanation is that the media used for testing do not mimic the airway milieu, resulting in incorrect conclusions. Therefore, media have been devised to mimic CF sputum.Aspergillus fumigatusis the leading fungal pathogen in CF, and susceptibility testing is also used to decide therapeutic choices. We assessed whether media designed to mimic CF sputa would give different fungal susceptibility results than those of classical methods, assaying voriconazole, the most utilized anti-Aspergillusdrug in this setting, and 30 CFAspergillusisolates. The frequency of marked resistance (defined as an MIC of >4 µg/ml) in our CF unit by classical methods is 7%. Studies performed with classical methods and with digested sputum medium, synthetic sputum medium, and artificial sputum medium revealed prominent differences inAspergillussusceptibility results, as well as growth rate, with each medium. Clinical correlative studies are required to determine which results are most useful in predicting outcome. Comparison of MICs with non-CF isolates also indicated the CF isolates were generally more resistant.

Hereditary inclusion body myopathy: single patient response to intravenous dosing of GNE gene lipoplex.

Hereditary inclusion body myopathy (HIBM) is an autosomal recessive adult-onset myopathy due to mutations in the GNE (UDP-N-acetylglucosamine 2-epimerase/N-acetylmannosamine kinase) gene. Affected patients have no therapeutic options. We have previously demonstrated in preclinical testing the ability to safely correct GNE gene function through liposomal delivery of the wild-type GNE gene. Results were verified in a single patient treated by intravenous infusion of GNE gene lipoplex. A single patient (patient 001) with severe HIBM treated with a compassionate investigational new drug received seven doses of GNE gene lipoplex via intravenous infusion at the following doses: 0.4, 0.4, 1.0, 4.0, 5.0, 6.0, and 7.0 mg of DNA. GNE transgene expression, downstream induction of sialic acid, safety, and muscle function were evaluated. Transient low-grade fever, myalgia, tachycardia, transaminase elevation, hyponatremia, and hypotension were observed after infusion of each dose of GNE gene lipoplex. Quadriceps muscle expression of the delivered GNE, plasmid, and RNA was observed 24 hr after the 5.0-mg dose and at significantly greater levels 72 hr after the 7.0-mg infusion in comparison with expression in quadriceps muscle immediately before infusion. Sialic acid-related proteins were increased and stabilization in the decline of muscle strength was observed. We conclude that clinical safety and activity have been demonstrated with intravenous infusion of GNE gene lipoplex. Further assessment will involve a phase I trial of intravenous administration of GNE gene lipoplex in individuals with less advanced HIBM with more muscle function.

¿ Metastasis o implantes de tumores serosos limitrofes (borderline) del ovario a ganglios linfaticos? revisión de la literatura a propósito de un caso / Metastasis or implant of bounding serous tumors? rervition of the literature a propuse of an case

Aproximadamente el 15 a 20 por ciento de todos los tumores epiteliales del ovario son limítrofes (borderline) o de bajo potencial de malignidad. De estos, el 30 al 5o por ceinto presentan "implantes peritoneales" para el momento del diagnóstico. En la s ultimas décadas se ha informado el compromiso de ganglios linfáticos asociados a tumores serosos lilmítrofes, este fenómeno es mucho más dificil de interpretar ya que establece la disyuntiva de sí se tratan de verdaderas metástasis o de una transformación secundaria de restos del epitelio mulleriano en el ganglio linfático. Tratando de aclarar un poco este controversial tema, presentamos una revisión de la literatura existente sobre el mismo así como los hallazgos clínicos, histológicos e inmumohistoquímicos de un caso de tumor seroso limítrofe (TSL) con compromiso de ganglios linfáticos

Anemia drepanocítica, lesiones hepáticas: estudio clínico, morfológico y ultraestructura de 21 casos / Sickle cell anemia hepatic lesions: clinical morphologic and ultrastructural study of 21 cases

La afectación hepática en pacientes con anemia drepanocítica, para algunos autores, es un hecho común y no tiene relación con la severidad de la anemia. El grado de disfunción hepática ésta relacionada con la isquemia y, existe probablemente, una disminución de la circulación intrahepática, secundaria a la obstrucción sinusoidal, debida a las masas de glóbulos rojos falciformes acumuladas y a la hipertrofia de las células de Kupffer. En este trabajo, se presentan los hallazgos clínicos, morfológicos y estructurales de 21 casos de hemoglobinopatías SS y SA. El 60% de los pacientes eran del sexo femenino el 40% del masculino, con edades comprendidas entre los 18 y 46 años. El hallazgo microscópico, más constante, fue la distensión sinusoidal, seguido de la hipertrofia de las células de Kupffer y la inflamación, observándose también en áreas de necrosis, esteatosis y la presencia de hierro

Aneurismas del septum membranoso interventricular / Aneurysms of the membranous septum

Se reportan ocho autopsias de adultos (F/M: 6/2 con aneurismas del septum membranoso, uno de los cuales se complicó con perforación sin consecuencia hemodinámica importante. Ninguno fue diagnósticado en vida ni presentaron malformaciones cardíacas. Uno solo se asoció a Síndrome de Down