Inner ear abnormalities in a Kcnq1 (Kvlqt1) knockout mouse: a model of Jervell and Lange-Nielsen syndrome.

HYPOTHESIS: Mice lacking functional KCNQ1 (previously known as KvLQT1) channels exhibit functional and structural abnormalities that indicate disturbed production of endolymph. BACKGROUND: Congenital deafness associated with cardiac conduction abnormalities (Jervell and Lange-Nielsen syndrome) is associated with dysfunctional KCNQ1/KCNE1 channel complex. This potassium channel plays a critical role in the production and homeostasis of endolymph by the stria vascularis. A preliminary report documented severe abnormalities of the scala media and vestibular compartments of a single mouse lacking functional KCNQ1 alleles. METHODS: Hearing thresholds were measured in three Kcnq1 knockout mice, two heterozygous mice, and one wild-type mouse by auditory brainstem response recordings using clicks, after which the temporal bones were removed. After fixation and dehydration, the ears were embedded in araldite, sectioned at 20-microm thickness, stained with toluidine blue on glass slides, and examined with the light microscope. RESULTS: Kcnq1 knockout mice were deaf and demonstrated circling behavior. They exhibited a marked atrophy of the stria vascularis, contraction of the endolymphatic compartments, and collapse and adhesion of surrounding membranes. There was a complete degeneration of the organ of Corti and an associated degeneration of the spiral ganglion. CONCLUSION: Kcnq1 knockout mice exhibit histopathologic findings that are comparable to those reported in human temporal bone cases of Jervell and Lange-Nielsen syndrome, and provide further evidence that KCNQ1 channel dysfunction can lead to congenital deafness in this syndrome.     

Proof of concept of a method that assesses the spread of microbial infections with spatially explicit and non-spatially explicit data

Background  

A method that assesses bacterial spatial dissemination was explored. It measures microbial genotypes (defined by electrophoretic patterns or EP), host, location (farm), interfarm Euclidean distance, and time. Its proof of concept (construct and internal validity) was evaluated using a dataset that included 113 Staphylococcus aureus EPs from 1126 bovine milk isolates collected on 23 farms between 1988 and 2005.     

Primary Pleomorphic T-cell Lymphoma of the Spleen

We report a case of primary splenic T-cell lymphoma that posed difficult problems in differential diagnosis with erythrophagocytic T-gamma lymphoma and inflammatory pseudotumor of the spleen. The need for immunophenotypic and molecular studies for establishing the correct diagnosis and the importance of early detection and treatment, is emphasized in the light of the relatively good prognosis of splenic lymphoma, diagnosed in the early stages of disease.     

Absence of in vitro or in vivo bystander effects in a thymidine kinase-transduced murine T lymphoma

Among the goals of an optimal gene transfer system are a predictably high efficiency of transfer and the ability to confer stable gene expression. An additional benefit of strategies designed to target tumor or effector cells could be the induction of a bystander effect. Although tumor killing by the bystander effect in vivo has been obtained in several types of malignant tumors, it has not been reported for T lymphomas. The goals of this work were to determine the stability of the expression of the herpes simplex virus type-1 thymidine kinase and the low-affinity receptor for nerve growth factor truncated of its intracellular domain (deltaLNGFR) genes inserted in a murine T lymphoma; in addition, we sought to determine whether a bystander effect (direct or indirect) was present after treatment of the transduced tumor with ganciclovir. This study demonstrates a high level of stable expression of both genes in the T lymphoma in vitro and in vivo. However, we could not detect direct or indirect bystander effects in vivo mediated by the herpes simplex virus thymidine kinase/ganciclovir system in this tumor of lymphocyte origin. This is the first report to investigate bystander effects in vivo on a T-cell lineage tumor; in addition, this report has implications for the therapeutic transfer of non-transformed, antigen-specific T cells in vivo.     

Eficacia,seguridad y satisfacción de los procedimientos de sedoanalgesia en las urgencias españolas

Objectives

To offer analgesia and sedation should be a priority in paediatric emergency departments. The aim of this study was to determine the effectiveness and safety of the sedation-analgesia procedure, as well as the satisfaction of the doctors, patients and parents.

Reduction of mouth opening due to mandibular coronoid progess enlargement. Report of a case.

A case of unilateral mandibular coronoid enlargement associated with facial asymmetry and severe limitation of mandibular movement in a 19-year-old male patient is described. The indications for treatment and the types of surgical approach for removal of the enlarged process are discussed. The dual surgical approach required for resection of the coronoid in this case is described.     

Body wall dysplasia: a rare case of abdominal hernia

Malformations of the fetal abdominal wall include a broad spectrum of anomalies, and prenatal sonography provides the possibility of detecting most of them. Omphalocele and gastroschisis are the most common conditions, but there are other rare forms. We describe here a rare case of body wall dysplasia that we called abdominal hernia that appeared upon prenatal sonography as an omphalocele-like defect.