Aged ; Female ; Hemophilia A ; complications ; Hemorrhage ; etiology ; Humans ; Mediastinal Diseases ; etiology

The X-linked dystonia-parkinsonism (XDP) is a severe progressive, adult-onset X-linked endemic disorder in Filipinos, which is characterized by dystonic movements that start in the third of fourth decade, and replaced by parkinsonism beyond the 10th year of illness. Understanding the pathophysiology of XDP and development of rational therapies will depend on observations from imaging pathological and genetic studies. In this paper we summarize the results of these studies on patients with XDP. The cranial magnetic resonance imaging shows hy-perintense putaminal rim in both dystonic and parkinsonian stages, and atrophy of the caudate head or putamen in the parkinsonian stage. Neuropathological findings show atrophy of the caudate nucleus and putamen, with mild to severe neuronal loss and gliosis. In the neostriatum, the dystonic phase of XDP shows the involvement of striosomes and matrix sparing, while the later, i.e., p[arkinsonian phase, shows matrix involvement as well. In the dystonic phase, the loss of striosomal inhibitory projections lead to disinhibition of nigral dopaminergic neurons, perhaps resulting in a hyperkinetic state; while in the parkinsonian phase, severe and critical reduction of matrix-based projection may result in extranigral parkinsonism. Genetic sequencing of the XDP critical region in Xq13.1 has revealed an SVA retronsposon insertion in an intron of TAF1. This may reduce neuron-specific expression of the TAF1 isoform in the caudade nucleus, and subsequently interfere with the transcription of many neuronal genes, including DRD2. Findings from imaging, pahtology, and genetics studies are gradually shedding light on the pathophysiology of XDP, which hopefully will lead to mare rational and directed therapies.

Human ; Adult ; Atrophy ; Caudate Nucleus ; Dopaminergic Neurons ; Dystonic Disorders ; Genetic Diseases, X-linked ; Gliosis ; Introns ; Parkinsonian Disorders ; Protein Isoforms ; Putamen

In March 2020, the Philippine Department of Health (DOH) designed and rapidly implemented a national surveillance system for the utilization of hospital beds by patients with coronavirus disease (COVID-19) to produce complete and timely data for use by various levels of governance in response to the COVID-19 pandemic. The DOH launched the DOH DataCollect (DDC) Bed Tracker system, a web-based application that collects information from all 1906 public and private hospitals and infirmaries across the country using a modular data collection tool. Data on the maximum number of occupied COVID-19-designated beds (n = 28 261), hospital bed utilization rate (71.7%), and mechanical ventilator number (n = 1846) and utilization rate (58.5%) were recorded in September 2021 during the Delta surge of cases in the Philippines. Data on human resources, personal protective equipment and supplies, and other operational indicators were added to the system during various modifications. Information from the DDC was used to inform the COVID-19 response and operations at national and local levels and facilitated research at academic and nongovernmental agencies. The development of the DDC system demonstrates that an effective surveillance system for use by all health-care facilities is achievable through strong national leadership, the use of available technology and adaptive information systems, and the establishment of networks across different health facilities and stakeholders.