Guillain-Barré Syndrome and Hydrocephalus in an infant with Wiskott-Aldrich Syndrome. / Síndrome de Guillain Barré e hidrocefalia en un lactante con Síndrome de Wiskott Aldrich.

INTRODUCTION: Guillain-Barre Syndrome (GBS) is rarely diagnosed in the first year of life. The association of GBS with Wiskott-Aldrich syndrome (WAS) is even less frequent and has been previously reported in only two children to our knowledge. Hydrocephalus is a known but rare complication of GBS. OBJECTIVE: To describe the case of an infant in which GBS, WAS and hydrocephalus appear clinically associated. CLINICAL CASE: A nine-months-old male infant with a history of WAS was admitted to our ICU with acute hypotonia and poor general condition. He developed flaccid paralysis, absent deep tendon reflexes, and respiratory failure. A lumbar puncture showed albuminocytologic dissociation. GBS was suspected and an electromyography was performed, showing a demyelinating polyneuropathy. He was successfully treated with intravenous immunoglobulins. During hospitalization, he presented intermittent bradycardia, so a brain CT scan was performed, showing acute hydrocephalus which was managed through an external ventricular drain achieving favorable response. In the long term, the patient underwent bone marrow transplant and had to be reoperated due to valve-related complications. However, his psychomotor development is normal, with no obvious neurological sequelae. CONCLUSION: We present the third case of GBS in a patient with WAS, which is the first infant younger than one year. Additionally, he presented acute hydrocephalus as a complication of GBS. We suggest considering these three comorbidities since their early diagnosis and prompt management allow bet ter neurological recovery and avoid potentially lethal complications.

Síndrome de Guillain Barré e hidrocefalia en un lactante con Síndrome de Wiskott Aldrich / Guillain-Barré Syndrome and Hydrocephalus in an infant with Wiskott-Aldrich Syndrome

Resumen: Introducción: El Síndrome de Guillain-Barré (SGB) es raramente diagnosticado en lactantes menores de 1 año. Su asociación con el Síndrome de Wiskott Aldrich (SWA), es aún menos frecuente, y ha sido previa mente reportada sólo en dos pacientes a nuestro conocimiento. La hidrocefalia, es una complicación conocida, pero infrecuente del SGB. Objetivo: presentar el caso clínico de un lactante en el que se asocian las patologías de SGB, SWA e hidrocefalia. Caso Clínico: varon de 9 meses, portador de SWA hospitalizado en unidad de cuidados intensivos por hipotonía aguda y compromiso del estado gene ral. Evolucionó con parálisis fláccida, falla ventilatoria y arreflexia generalizada. Una punción lumbar mostró disociación albuminocitológica, y el estudio electrofisiológico mostró signos de polineuropatía desmielinizante severa. Se trató con inmunoglobulina, evolucionando en forma satisfactoria. Por bradicardia intermitente, se realizó tomografla axial computada cerebral (TAC), que mostró signos de una hidrocefalia aguda, manejada mediante válvula derivativa ventrículo peritoneal con favorable respuesta. En el largo plazo, se sometió a trasplante de médula ósea y debió ser reintervenido por complicaciones valvulares, sin embargo, su desarrollo psicomotor es normal sin secuelas neurológi cas evidentes hasta los 3 años. Conclusión: Presentamos el tercer caso de SGB en un paciente porta dor de SWA, destacando ser el primero de ellos en un lactante menor de 1 año. Adicionalmente, este niño presentó una hidrocefalia aguda como complicación del SGB. Consideramos relevante tener presente estas comorbilidades, debido a que su pronto diagnóstico y manejo oportuno, permiten una mejor recuperación neurológica y evitan complicaciones potencialmente letales.

Abstract: Introduction: Guillain-Barre Syndrome (GBS) is rarely diagnosed in the first year of life. The association of GBS with Wiskott-Aldrich syndrome (WAS) is even less frequent and has been previously reported in only two children to our knowledge. Hydrocephalus is a known but rare complication of GBS. Objective: To describe the case of an infant in which GBS, WAS and hydrocephalus appear clinically associated. Clinical Case: A nine-months-old male infant with a history of WAS was admitted to our ICU with acute hypotonia and poor general condition. He developed flaccid paralysis, absent deep tendon reflexes, and respiratory failure. A lumbar puncture showed albuminocytologic dissociation. GBS was suspected and an electromyography was performed, showing a demyelinating polyneuropathy. He was successfully treated with intravenous immunoglobulins. During hospitalization, he presented intermittent bradycardia, so a brain CT scan was performed, showing acute hydrocephalus which was managed through an external ventricular drain achieving favorable response. In the long term, the patient underwent bone marrow transplant and had to be reoperated due to valve-related complications. However, his psychomotor development is normal, with no obvious neurological sequelae. Conclusion: We present the third case of GBS in a patient with WAS, which is the first infant younger than one year. Additionally, he presented acute hydrocephalus as a complication of GBS. We suggest considering these three comorbidities since their early diagnosis and prompt management allow bet ter neurological recovery and avoid potentially lethal complications.

Intratumoral CD56bright natural killer cells are associated with improved survival in bladder cancer.

BACKGROUND: Natural killer (NK) cells are effective at killing tumors in a non-MHC restricted manner and are emerging targets for cancer therapy but their importance in bladder cancer (BC) is poorly defined. NK cells are commonly subdivided into populations based on relative surface expression of CD56. Two major subsets are CD56bright and CD56dim NK cells. METHODS: The prevalence of intratumoral lymphocytes was examined via flow cytometric analysis of bladder tissue from a local cohort of patients with non-invasive and invasive BC (n=28). The association of NK cell subsets with cancer-specific survival (CSS) and overall survival (OS) was examined in 50 patients with BC using Cox regression. Fluorescence-activated cell sorting (FACS) of intratumoral lymphocytes isolated CD56 NK cell subsets were used for examination of function, including cytokine production and in vitro cytotoxicity. RESULTS: NK cells predominated among bladder intratumoral lymphocytes. Intratumoral CD56bright NK cells showed increased cytokine production and cytotoxicity compared to their CD56dim counterparts and were associated with improved CSS and OS independent of pathologic tumor stage. On the other hand, CD56dim NK cells were not associated with improved outcomes but were associated with higher pathologic stage. CONCLUSIONS: NK cells are frequent among intratumoral lymphocytes in BC. Bladder intratumoral CD56bright NK cells are functional and prognostically relevant whereas CD56dim NK cells are dysfunctional and prevalent in higher stage tumors. Thus, CD56bright NK cells are promising targets in BC.

Molecular characterization of pestiviruses isolated from bovines in Chile.

Thirty-three pestiviruses isolated from bovines on different farms in Chile were characterized at the molecular level. The 5'-untranslated region (5'UTR) of the isolates was amplified by reverse transcription-polymerase chain reaction (RT-PCR) and subsequently digested with restriction enzymes (RE) Bgl I, Xho I and Pst I. Furthermore, the isolates were amplified by differential RT-PCR, which selectively amplified bovine viral diarrhea virus type 2 (BVDV-2) but not bovine viral diarrhea virus type 1 (BVDV-1). Of the 33 isolates, 16 were classified as BVDV-1 and 17 as BVDV-2. Phylogenetic analysis of the PCR-amplified fragments from 14 isolates showed the presence of viruses belonging to the BVDV-1a, BVDV-1b, BVDV-1c, and BVDV-2 types. Antigenicity of some viruses belonging to both genotypes was studied by cross-seroneutralization, revealing great antigenic differences among them. It is concluded that BVDV viruses circulating in Chile are genetically and antigenically heterogeneous, comprising isolates of genogroups 1 and 2.