Secondary Hemophagocytic Lymphohistiocytosis Causing Hemorrhagic Encephalomyelitis and Spinal Subarachnoid Hemorrhage: A Case Report.

We are writing to present an interesting and novel case from our practice of a patient who presented with altered mental status and a rapidly progressive paraplegia as well as high fevers and pancytopenia. A bone marrow biopsy was diagnostic of hemophagocytic lymphohistiocytosis (HLH) and MRI showed hemorrhagic encephalitis and spinal subarachnoid hemorrhage. This case demonstrates the diverse neurological symptoms with which HLH presents, including spinal cord pathology.

Cytokine exposure mediates transcriptional activation of the orphan nuclear receptor Nur77 in hematopoietic cells.

The orphan nuclear receptor Nur77 is an immediate-early response gene that based on tissue and cell context is implicated in a plethora of cellular processes, including proliferation, differentiation, apoptosis, metabolism, and inflammation. Nur77 has a ligand-binding pocket that is obstructed by hydrophobic side groups. Naturally occurring, cell-endogenous ligands have not been identified, and Nur77 transcriptional activity is thought to be regulated through posttranslational modification and modulation of protein levels.

RXRA DT448/9PP generates a dominant active variant capable of inducing maturation in acute myeloid leukemia cells.

RARA and RXRA contribute to myeloid maturation in both mice and humans, and deletion of Rxra and Rxrb augments leukemic growth in mice. While defining the domains of RXRA that are required for anti-leukemic effects in murine KMT2A-MLLT3 leukemia cells, we unexpectedly identified RXRA DT448/9PP as a constitutively active variant capable of inducing maturation and loss of their proliferative phenotype. RXRA DT448/9PP was associated with ligand-independent activity in reporter assays, with enhanced co-activator interactions, reduced engraftment in vivo, and activation of myeloid maturation transcriptional signatures that overlapped with those of cells treated with the potent RXRA agonist bexarotene, suggestive of constitutive activity that leads to leukemic maturation.

Endogenous and combination retinoids are active in myelomonocytic leukemias.

Retinoid therapy transformed response and survival outcomes in acute promyelocytic leukemia (APL), but has demonstrated only modest activity in non-APL forms of acute myeloid leukemia (AML). The presence of natural retinoids in vivo could influence the efficacy of pharmacologic agonists and antagonists. We found that natural RXRA ligands, but not RARA ligands, were present in murine MLL-AF9-derived myelomonocytic leukemias in vivo and that the concurrent presence of receptors and ligands acted as tumor suppressors.

CDK1 and CDK2 regulate NICD1 turnover and the periodicity of the segmentation clock.

All vertebrates share a segmented body axis. Segments form from the rostral end of the presomitic mesoderm (PSM) with a periodicity that is regulated by the segmentation clock. The segmentation clock is a molecular oscillator that exhibits dynamic clock gene expression across the PSM with a periodicity that matches somite formation.

The Pediatric AIDS Corps: a 5-year evaluation.

Background: The Baylor College of Medicine International Pediatric AIDS Initiative at Texas Children's Hospital created a global health corps named the Pediatric AIDS Corps (PAC) in June 2005. This report provides descriptive details and outputs for PAC over its first 5 years.

Methods: Demographic data were gathered about PAC physicians employed from July 2006 to June 2011.

A prospective newborn screening and treatment program for sickle cell anemia in Luanda, Angola.

Over 300,000 infants are born annually with sickle cell anemia (SCA) in sub-Saharan Africa, and >50% die young from infection or anemia, usually without diagnosis of SCA. Early identification by newborn screening (NBS), followed by simple interventions dramatically reduced the mortality of SCA in the United States, but this strategy is not yet established in Africa. We designed and implemented a proof-of-principle NBS and treatment program for SCA in Angola, with focus on capacity building and local ownership.

The genome of a songbird.

The zebra finch is an important model organism in several fields with unique relevance to human neuroscience. Like other songbirds, the zebra finch communicates through learned vocalizations, an ability otherwise documented only in humans and a few other animals and lacking in the chicken-the only bird with a sequenced genome until now. Here we present a structural, functional and comparative analysis of the genome sequence of the zebra finch (Taeniopygia guttata), which is a songbird belonging to the large avian order Passeriformes.

The Pediatric AIDS Corps: responding to the African HIV/AIDS health professional resource crisis.

Health professional capacity for delivery of HIV/AIDS care and treatment is severely constrained across sub-Saharan Africa. African health professional expertise in pediatrics is in particularly short supply. Here we describe a Pediatric AIDS Corps program that was designed to place pediatricians and other physicians in Africa on a long-term basis to expand existing health professional capacity for pediatric and family HIV/AIDS care and treatment.

Education and preparation of physicians entering an international pediatric AIDS program: the Pediatric AIDS Corps.

The Pediatric AIDS Corps (PAC) are a group of physicians that were hired to provide clinical care and treatment to children and their families infected with HIV/AIDS and to help educate local health care professionals in the management of children with HIV/AIDS located in the high prevalence areas of sub-Saharan Africa. Prior to their departure the PAC were required to participate in a 4-week educational training program that included travel and tropical medicine and HIV infections in children, teaching skills, bioethics, and good clinical practice in human research training. Evaluation of the program was done using a 50-question pretest/posttest design, a standard postcourse evaluation, and a PAC focus group follow-up.

The Songbird Neurogenomics (SoNG) Initiative: community-based tools and strategies for study of brain gene function and evolution.

Background: Songbirds hold great promise for biomedical, environmental and evolutionary research. A complete draft sequence of the zebra finch genome is imminent, yet a need remains for application of genomic resources within a research community traditionally focused on ethology and neurobiological methods. In response, we developed a core set of genomic tools and a novel collaborative strategy to probe gene expression in diverse songbird species and natural contexts.

Influence of institutionalization on time to HIV disease progression in a cohort of Romanian children and teens.

Objectives: The purpose of this work was to investigate the effect of institutionalization on death and CD4 decline in a cohort of 325 HIV-infected Romanian children.

Methods: A retrospective database analysis was conducted. Data from a nearly 3-year period were examined with Kaplan-Meier survival analysis and Cox regression analysis models.