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1.
Biol Psychiatry ; 2019 Jun 29.
Artigo em Inglês | MEDLINE | ID: mdl-31443933

RESUMO

BACKGROUND: The X-chromosome gene USP9X encodes a deubiquitylating enzyme that has been associated with neurodevelopmental disorders primarily in female subjects. USP9X escapes X inactivation, and in female subjects de novo heterozygous copy number loss or truncating mutations cause haploinsufficiency culminating in a recognizable syndrome with intellectual disability and signature brain and congenital abnormalities. In contrast, the involvement of USP9X in male neurodevelopmental disorders remains tentative. METHODS: We used clinically recommended guidelines to collect and interrogate the pathogenicity of 44 USP9X variants associated with neurodevelopmental disorders in males. Functional studies in patient-derived cell lines and mice were used to determine mechanisms of pathology. RESULTS: Twelve missense variants showed strong evidence of pathogenicity. We define a characteristic phenotype of the central nervous system (white matter disturbances, thin corpus callosum, and widened ventricles); global delay with significant alteration of speech, language, and behavior; hypotonia; joint hypermobility; visual system defects; and other common congenital and dysmorphic features. Comparison of in silico and phenotypical features align additional variants of unknown significance with likely pathogenicity. In support of partial loss-of-function mechanisms, using patient-derived cell lines, we show loss of only specific USP9X substrates that regulate neurodevelopmental signaling pathways and a united defect in transforming growth factor ß signaling. In addition, we find correlates of the male phenotype in Usp9x brain-specific knockout mice, and further resolve loss of hippocampal-dependent learning and memory. CONCLUSIONS: Our data demonstrate the involvement of USP9X variants in a distinctive neurodevelopmental and behavioral syndrome in male subjects and identify plausible mechanisms of pathogenesis centered on disrupted transforming growth factor ß signaling and hippocampal function.

2.
Mol Biol Rep ; 2019 Aug 07.
Artigo em Inglês | MEDLINE | ID: mdl-31392538

RESUMO

Pediococcus acidilactici NCDC 252 is a facultative anaerobe of dairy origin that possessed all studied in vitro probiotic attributes and several useful enzyme activities. Its whole genome was sequenced and analysed for its evolutionary relationship with other lactic acid bacteria (LAB). This is a novel sequence and first report of genome sequence of P. acidilactici of dairy origin. Its genome is relatively larger than other studied genomes of P. acidilactici and is comprised of 40 scaffolds that totals to 3,243,337 bases and 44.5% GC content. A total of 3054 coding sequences (CDS) were identified by RAST and DIAMOND servers. The genome also encoded different enzyme activities required for utilization of various carbohydrates. This was also confirmed by carbohydrate utilization studies. The genome also encoded genes for probiotics properties. The phylogenetic analysis of P. acidilactici NCDC 252 genome was done using Maximum Parsimony and Maximum Likelihood methods to study its evolution and relatedness to other LABs based upon their 16S rDNA sequences. The strain exhibited highest resemblance to Lactobacillus plantarum WCFS1 and is also much close to P. acidilactici based on similarity of ribosomal protein. The strain seems to have acquired some genes for its adaptation in dairy/environmental niche. This genome sequence is novel with genome more similar to L. plantarum and biochemical and phenotypic characteristics of P. acidilactici.

3.
Pediatr Cardiol ; 40(6): 1284-1288, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31317219

RESUMO

Junctional ectopic tachycardia (JET) is the commonest tachyarrhythmia in the early post-operative period in children undergoing open-heart surgery. It frequently leads to hemodynamic instability and needs to be managed aggressively. Amiodarone is the first-line agent along with non-pharmacological interventions. We report our initial experience with the use of Ivabradine in post-operative JET. A retrospective case records review of children with post-operative JET during the period from June 2018 to May 2019 was performed. Eight patients with post-operative JET were treated with Ivabradine during this period. The first patient was initially treated with Amiodarone. All eight patients responded to Ivabradine. The initial response was rate control permitting overdrive pacing. One patient had recurrence of JET 10 h after Ivabradine and after return to sinus rhythm. Amiodarone was administered along with the second dose of Ivabradine resulting in remission to sinus rhythm. Ivabradine appears to be an effective alternative to Amiodarone in children with post-operative JET based on our initial clinical experience.

4.
BMJ Open ; 9(6): e028307, 2019 Jun 22.
Artigo em Inglês | MEDLINE | ID: mdl-31230022

RESUMO

OBJECTIVE: Many low-income and middle-income countries (LMICs) struggle to provide the health services investment required for life-saving congenital heart disease (CHD) surgery. We explored associations between risk-adjusted CHD surgical mortality from 17 LMICs and global development indices to identify patterns that might inform investment strategies. DESIGN: Retrospective analysis: country-specific standardised mortality ratios were graphed against global development indices reflective of wealth and healthcare investment. Spearman correlation coefficients were calculated. SETTING AND PARTICIPANTS: The International Quality Improvement Collaborative (IQIC) keeps a volunteer registry of outcomes of CHD surgery programmes in low-resource settings. Inclusion in the IQIC is voluntary enrolment by hospital sites. Patients in the registry underwent congenital heart surgery. Sites that actively participated in IQIC in 2013, 2014 or 2015 and passed a 10% data audit were asked for permission to share data for this study. 31 sites in 17 countries are included. OUTCOME MEASURES: In-hospital mortality: standardised mortality ratios were calculated. Risk adjustment for in-hospital mortality uses the Risk Adjustment for Congenital Heart Surgery method, a model including surgical risk category, age group, prematurity, presence of a major non-cardiac structural anomaly and multiple congenital heart procedures during admission. RESULTS: The IQIC registry includes 24 917 congenital heart surgeries performed in children<18 years of age. The overall in-hospital mortality rate was 5.0%. Country-level congenital heart surgery standardised mortality ratios were negatively correlated with gross domestic product (GDP) per capita (r=-0.34, p=0.18), and health expenditure per capita (r=-0.23, p=0.37) and positively correlated with under-five mortality (r=0.60, p=0.01) and undernourishment (r=0.39, p=0.17). Countries with lower development had wider variation in mortality. GDP per capita is a driver of the association between some other measures and mortality. CONCLUSIONS: Results display a moderate relationship among wealth, healthcare investment and malnutrition, with significant variation, including superior results in many countries with low GDP per capita. These findings provide context and optimism for investment in CHD procedures in low-resource settings.

5.
Acta Trop ; 195: 103-114, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-31039335

RESUMO

Malaria, one of the major infectious disease-causing sizeable morbidity, mortality and economic loss worldwide. The main drawback for the failure to eradicate malaria is the spread of multiple drug resistance to the majority of currently available chemotherapy. At present nanotechnology offers an advanced opportunity in the delivery of drugs and vaccines to the desired targeted site in the body following oral and systemic administration. It confers the major advantages like improving drug pharmacokinetic profiles, reduce dose frequency and reduction in drug toxicity. Hence, Nano-based drug delivery system can provide a promising prospect in the way of malaria treatment. This paper is a review of recent researches highlighting includes nanocarriers loaded antimalarial drugs for better therapeutic efficacy and future perspective in the treatment of malaria.


Assuntos
Antimaláricos/administração & dosagem , Antimaláricos/uso terapêutico , Sistemas de Liberação de Medicamentos/métodos , Malária/tratamento farmacológico , Nanotecnologia/métodos , Humanos
6.
Artigo em Inglês | MEDLINE | ID: mdl-30851076

RESUMO

We report pseudoaneurysm of the axillary artery following stenting of the arterial duct in two infants. They were both successfully managed by ultrasound guided thrombin injection with preservation of native arterial flow. We also review the relevant literature.

7.
J Cancer Res Clin Oncol ; 145(6): 1601-1611, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30891618

RESUMO

PURPOSE: Multiple myeloma (MM) is a hematological malignancy marked by uncontrolled proliferation and accumulation of plasma cells in bone marrow. Despite presence of numerous diagnostic markers for MM, their invasive and non-specific nature demands identification of some effective biomarker. Small non-coding RNAs, i.e., microRNAs being secreted out in circulation could depict the change in homeostasis. Earlier, we reported diagnostic potential of a proteoglycan, Versican (VCAN) in MM, hence, VCAN linked cell-free microRNAs have been explored to study their diagnostic involvement in MM. METHODS: Biopsy proven MM patients and controls were recruited. The relative microRNA expression of VCAN linked microRNAs (miR-143, miR-144, miR-199, and miR-203) along with levels of VCAN have been investigated in bone marrow supernatant fluid (BMSF) and blood serum and their correlation were done with clinico-pathological parameters. The diagnostic potential was assessed using ROC curve. RESULTS: Relative microRNA expression of all microRNAs was found significantly lower in MM patients in both BMSF and serum while VCAN levels were substantially higher in patients. VCAN levels showed positive trend while microRNAs expression showed negative trend with severity of disease. miR-203 showed significant correlation with myeloma-associated parameters and also showed optimum sensitivity and specificity for diagnosis of MM in serum. CONCLUSIONS: Downregulation of cell-free microRNAs illustrates their importance in MM. The negative trend of microRNAs with disease progression suggests their diagnostic significance. Correlation of miR-203 with myeloma clinical parameters along with optimum sensitivity and specificity affirms its non-invasive diagnostic potential in MM which could further be validated in larger patient cohort.


Assuntos
Biomarcadores Tumorais/sangue , MicroRNA Circulante/sangue , MicroRNAs/sangue , Mieloma Múltiplo/sangue , Adulto , Idoso , Biomarcadores Tumorais/biossíntese , Biomarcadores Tumorais/genética , Estudos de Casos e Controles , MicroRNA Circulante/biossíntese , MicroRNA Circulante/genética , Regulação para Baixo , Feminino , Humanos , Masculino , MicroRNAs/biossíntese , MicroRNAs/genética , Pessoa de Meia-Idade , Mieloma Múltiplo/genética , Mieloma Múltiplo/patologia , Estadiamento de Neoplasias , Versicanas/biossíntese , Versicanas/sangue , Versicanas/genética
8.
Hum Mol Genet ; 2018 Nov 21.
Artigo em Inglês | MEDLINE | ID: mdl-30476144

RESUMO

THOC6 encodes a subunit of the THO complex that is part of a highly-conserved TREX complex, known to have roles in mRNA processing and export. Few homozygous or compound heterozygote variants have been identified in the THOC6 gene in patients with a syndromic form of intellectual disability (ID) (Beaulieu-Boycott-Innes syndrome, BBIS MIM# 613680). Here we report two additional individuals affected with BBIS originating from the north of Europe and sharing an haplotype composed by threevery rare missense changes in the THOC6gene: p.(Trp100Arg; Val234Leu; Gly275Asp). The first individual is a boy who is homozygous for the three-variant haplotype, due to a maternal uniparental disomy event. The second is a girl, who is compound heterozygote for this haplotype and a previously reported p.(Gly190Glu) missense variant. We analyzed the impact of these different amino acid changes on THOC6 protein expression,cellular localization, and interaction with the other THO complex subunits. We show that the different THOC6 variants alter the physiological nuclear localization of the protein and its interaction with at least two THO subunits, THOC1 and THOC5. Two amino acid changes from the three-variant-haplotype have alone specific effects and might contribute to the pathogenicity of the haplotype. Overall, we expanded the cohort of currently known BBIS affected individuals by reporting two individuals carrying the same recurrent European haplotype composed of three amino acid changes, affecting THOC6 localization and interaction with THO protein partners.

9.
Ann Pediatr Cardiol ; 11(3): 275-277, 2018 Sep-Dec.
Artigo em Inglês | MEDLINE | ID: mdl-30271017

RESUMO

A true presubclavian coarctation of the aorta with arch hypoplasia without major intracardiac anomaly can result in a surgical dilemma. Theoretically, one can avoid repair through median sternotomy using selective cerebral perfusion and its sequelae since no major intracardiac repair is needed. Repair through thoracotomy is technically challenging if arch reconstruction is required and precise surgical planning is required to avoid spinal hypoperfusion and to maintain cerebral perfusion. Moreover, inadequate repair often has resulted in hypoplastic aortic arch or residual coarctation on follow-up requiring future intervention. We have employed a modified surgical repair through thoracotomy avoiding cardiopulmonary bypass to address presubclavian coarctation of the aorta with diffuse arch hypoplasia with theoretically less chance for future stenosis.

10.
Mol Biol Rep ; 2018 Oct 11.
Artigo em Inglês | MEDLINE | ID: mdl-30311125

RESUMO

Shelterin complex and its associated molecules are imperative for proper functioning and maintenance of human telomeres. These molecules in association with human telomerase have been found altered in most cancers including multiple myeloma thereby proposed them as suitable therapeutic targets. Further, due to aggressive and recurring behavior of myeloma novel, efficacious and safe therapeutic agents for disease prevention are primary requirements for treatment of this disease. This maiden attempt evaluated the anti-proliferative properties of tanshinone I (TanI) alone or in combination with lenalidomide (Len) on myeloma cancer cell lines (RPMI8226 and U226). Further, after drug treatment levels of telomerase activity (TA) and molecular expression (mRNA & protein) of shelterin complex and its associated molecules have also been investigated. Results demonstrated that, TanI significantly inhibited proliferation of myeloma cells in dose and time dependent manner as observed through cytotoxicity assay. Additionally, induction of apoptosis by TanI and in combination with Len was observed in myeloma cells through propidium iodide (PI) staining, annexin V-FITC/PI staining, TUNEL and caspase-3/7 activity assays. Further, drug treatment significantly decreased (p < 0.01) TA and molecular expression of ACD, TERF2IP and TANK1 in comparison to vehicle control (0.1% DMSO) myeloma cells. Thus, this maiden in-vitro study provided initial evidences of therapeutic potential of TanI alone or in combination with chemotherapeutic agent Len as novel anticancer agents in myeloma cells which need further evaluation in future. Lastly, down-regulation of TA and decreased expression of these molecules underscores their potential as plausible therapeutic targets.

11.
Artigo em Inglês | MEDLINE | ID: mdl-30208249

RESUMO

OBJECTIVE: We sought to evaluate the feasibility, technical aspects, and outcome of transcatheter perimembranous ventricular septal defect (pmVSD) closure using duct occluder devices with a single retention disc. BACKGROUND: Use of duct occluder devices to close pmVSD seems a promising alternative therapy. However, limited data exist on this technique. METHODS: From 2010 to 2016, 222 patients (female 47.7%) were identified from databases of five participating institutions in whom pmVSD closure was attempted using an Amplatzer Duct Occluder I or Lifetech duct occluder device. RESULTS: Patients ranged in age from 0.7 to 52 years (median, 7.0 years) and in weight from 4.0 to 70 kg (median, 18.0 kg). The mean size of the VSD was 6.8 ± 2.2 mm. A large defect (> 6 mm) was present in 137 patients (61.7%). Device closure was successful in 218 patients (98.2%). The 10/8 mm device was used in most patients (n = 85, 38.3%), and the vascular approach was from the femoral vein in 169 patients (76.1%). There were 18 early complications in 17/218 patients (7.8%). Three patients (1.4%) developed complete heart block (transient n = 2; requiring permanent pacing n = 1). Median follow-up was 6 months (6 months-6 years). A mild residual shunt was seen in 10 patients at 6 months follow-up. CONCLUSIONS: The immediate results of transcatheter pmVSD closure using a duct occluder device with a single retention disc are promising. It is an effective technique with a lower rate of complications than for other currently available devices.

12.
J Family Med Prim Care ; 7(4): 649-651, 2018 Jul-Aug.
Artigo em Inglês | MEDLINE | ID: mdl-30234032

RESUMO

In the 40th anniversary year of Alma Ata: Health for All by 2000 conference, Academy of Family Physicians of India organized the 15th WONCA World Rural Health conference in New Delhi from 26 to 29th April 2018. More than a thousand delegates (1044) from 40 countries participated in this international conference. The conference attracted a multidisciplinary participation from various backgrounds with an interest in rural primary healthcare. Theme of this conference was "Healing the Heart of Healthcare-Leaving No one behind". The conference aspired to bring rural healthcare on global agenda. This was the first ever World Rural Health Conference organized in South Asia, incidentally a quarter of human population lives in South Asia, majority of which is based in rural habitat. The conference culminated with the unanimous adoption of the Delhi Declaration; calling for people living in rural and isolated parts of the to be given special priority, if nations are to achieve universal health coverage. The declaration has been cited at the website of the World Health Organization and is available in six languages (Chinese, English, French, Japanese, Portuguese, and Spanish.

13.
Pediatr Cardiol ; 2018 Sep 03.
Artigo em Inglês | MEDLINE | ID: mdl-30178189

RESUMO

The long-term outcome after repair of tetralogy of Fallot (TOF) is critically dependent pulmonary valve competence that is compromised by trans-annular patch (TAP). We compared a new echocardiographic index [pulmonary annulus index (PAI)] to conventional methods of predicting need for TAP in infants undergoing TOF repair. Consecutive infants undergoing TOF repair were prospectively studied. Pre-operative aortic and pulmonary annuli and main pulmonary artery (MPA) diameters were measured and z scores determined. PAI was a ratio of observed to expected pulmonary annulus (PA) diameter. TAP was based on intra-operative sizing by surgeons blinded to PAI values. Receiver operator curves (ROC) were generated for all PAI, MPA z scores and pulmonary annulus z scores. Of 84 infants (8.6 ± 2.6 months; 7.5 ± 1.3 kg), 36 needed TAP (43%). All the three indices viz. PAI, Pulmonary annulus and MPA z scores performed similarly in predicting need for TAP (ROC curves ~ 80%). Combining cut-offs of MPA z scores (> - 3.83) with either PAI (> 0.73) or PA z score (> - 1.83) predicted avoidance of TAP with ~ 90% accuracy. When both PAI and MPA z scores were below the cut-offs there was an 80% likelihood of TAP. Failure to predict TAP was associated with unicommisural pulmonary valves. PAI was equal to PA z scores in predicting need for TAP during repair of TOF. Combining either with MPA z scores was the most accurate method of prediction. Failure of prediction was mainly due to presence of a unicommissural pulmonary valve.

14.
J Family Med Prim Care ; 7(3): 487-488, 2018 May-Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30112293

RESUMO

The traditional "medical model" of health care has a paternalistic approach where patients are reassured and not decision makers, but some individuals and families prefer it. But there are some other confusing terminologies in health-care delivery and quality improvement namely patient-centered, person-centered, and patient-directed care. By changing the language, it is emphasizing that people are more than diseases and their socioeconomic determinants can affect their disease trajectories and care choices. Patient-centered denotes more holism and empowerment as it promotes and increases the access to personal health information through various modes using updated technology such as computers or mobile phones and keeping them informed through their active participation. Whereas person-centered care would focus on the whole person not just the medical conditions. In patient-directed care model, again the approach puts individuals in control of decisions about their care. One of the reasons why family medicine fraternity sprang up was to provide an antidote for high-tech specialty care that did not respect patients' values and concerns which is seen by their emphasis on the patient as a whole.

15.
J Family Med Prim Care ; 7(3): 489-494, 2018 May-Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30112294

RESUMO

Despite the stated aim of Medical Council of India (body regulating medical education in India) to produce an Indian Medical Graduate with requisite knowledge, skills, attitudes, values and responsiveness, so that he or she may function appropriately and effectively as a doctor of first contact of the community while being globally relevant, it appears that we failed. The joint working group extensively consisting of medical teachers have come up with suggestions which may work as the game changer in Indian Health care system. The key is to dedicate medical education towards primary care.

16.
Sleep Sci ; 11(2): 2017 Apr, 2018 Mar-Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30083291

RESUMO

Over the last three to four decades, it has been observed that the average total number of hours of sleep obtained per night by normal individuals have decreased. Concomitantly, global figures indicate that insufficient sleep is associated with serious adverse health and social outcomes. Moreover, insufficient sleep has been linked to seven of the fifteen leading causes of death. Additionally, current evidence suggests that sleep plays a significant role in determining cognitive performance and workplace productivity. There is a great need for a systematic analysis of the economic impact of insufficient sleep, particularly given current evidence that this phenomenon, as well as the poor sleep hygiene practices which produce it, is increasing worldwide. This paper takes the view that health authorities around the world need to raise the general awareness of benefits of sleep. There is considerable scope for research into both the public health impact as well as the macroeconomic consequences of insufficient sleep syndrome (ISS). Additionally, various models which estimate the undiagnosed burden of ISS on the GDP (gross domestic product) are needed to prioritize health issues and to highlight the national policies that are necessary to combat this medical problem. Sleep insufficiency has been declared to be a 'public health epidemic'; therefore, we propose ISS as a potential noncommunicable disease. This review elaborates on this topic further, exploring the causes and consequences of insufficient sleep, and thus providing a perspective on the policies that are needed as well as the research that will be required to support and justify these policies.

17.
J Family Med Prim Care ; 7(2): 275-283, 2018 Mar-Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30090764

RESUMO

Nipah virus (NiV) encephalitis first reported in "Sungai Nipah" in Malaysia in 1999 has emerged as a global public health threat in the Southeast Asia region. From 1998 to 2018, more than 630 cases of NiV human infections were reported. NiV is transmitted by zoonotic (from bats to humans, or from bats to pigs, and then to humans) as well as human-to-human routes. Deforestation and urbanization of some areas have contributed to greater overlap between human and bat habitats resulting in NiV outbreaks. Common symptoms of NiV infection in humans are similar to that of influenza such as fever and muscle pain and in some cases, the inflammation of the brain occurs leading to encephalitis. The recent epidemic in May 2018 in Kerala for the first time has killed over 17 people in 7 days with high case fatality and highlighted the importance of One Health approach. The diagnosis is often not suspected at the time of presentation and creates challenges in outbreak detection, timely control measures, and outbreak response activities. Currently, there are no drugs or vaccines specific for NiV infection although this is a priority disease on the World Health Organization's agenda. Antivirals (Ribavirin, HR2-based fusion inhibitor), biologicals (convalescent plasma, monoclonal antibodies), immunomodulators, and intensive supportive care are the mainstay to treat severe respiratory and neurologic complications. There is a great need for strengthening animal health surveillance system, using a One Health approach, to detect new cases and provide early warning for veterinary and human public health authorities.

18.
J Family Med Prim Care ; 7(2): 291-302, 2018 Mar-Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30090767

RESUMO

Purpose: This joint position statement, by the Indian Association of Palliative Care (IAPC) and Academy of Family Physicians of India (AFPI), proposes to address gaps in palliative care provision in the country by developing a community-based palliative care model that will empower primary care physicians to provide basic palliative care. Evidence: India ranks very poorly, 67th of 80 countries in the quality of death index. Two-thirds of patients who die need palliative care and many such patients spend the last hours of life in the Intensive care unit. The Indian National Health Policy (NHP) 2017 and other international bodies endorse palliative care as an essential health-care service component. NHP 2017 also recommends development of distance and continuing education options for general practitioners to upgrade their skills to provide timely interventions and avoid unnecessary referrals. Methods: A taskforce was formed with Indian and International expertise in palliative care and family medicine to develop this paper including an open conference at the IAPC conference 2017, agreement of a formal liaison between IAPC and AFPI and wide consultation leading to the development of this position paper aimed at supporting integration, networking, and joint working between palliative care specialists and generalists. The WHO model of taking a public health approach to palliative care was used as a framework for potential developments; policy support, education and training, service development, and availability of appropriate medicines. Recommendations: This taskforce recommends the following (1) Palliative care should be integrated into all levels of care including primary care with clear referral pathways, networking between palliative care specialist centers and family medicine physicians and generalists in community settings, to support education and clinical services. (2) Implement the recommendations of NHP 2017 to develop services and training programs for upskilling of primary care doctors in public and private sector. (3) Include palliative care as a mandatory component in the undergraduate (MBBS) and postgraduate curriculum of family physicians. (4) Improve access to necessary medications in urban and rural areas. (5) Provide relevant in-service training and support for palliative care to all levels of service providers including primary care and community staff. (6) Generate public awareness about palliative care and empower the community to identify those with chronic disease and provide support for those choosing to die at home.

19.
Pediatric Health Med Ther ; 9: 67-71, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-29985487

RESUMO

Subacute sclerosing panencephalitis is a progressive neurodegenerative disease. It usually occurs 7-10 years after measles infection. The clinical course is characterized by progressive cognitive decline and behavior changes followed by focal or generalized seizures as well as myoclonus, ataxia, visual disturbance, and later vegetative state, eventually leading to death. It is diagnosed on the basis of Dyken's criteria. There is no known cure for subacute sclerosing panencephalitis to date, but it is preventable by ensuring that an effective vaccine program for measles is made compulsory for all children younger than 5 years in endemic countries.

20.
Int J Cardiol ; 265: 212-217, 2018 Aug 15.
Artigo em Inglês | MEDLINE | ID: mdl-29885688

RESUMO

BACKGROUND: Epidemiological data on pulmonary hypertension (PH) are scarce from developing countries including India. METHODS: We established a multi-center registry of PH, the PRO-KERALA registry, in Kerala, India. Fifty hospitals enrolled consecutive adult (>18 years) patients for one year. Echocardiographic criteria (right ventricular systolic pressure - RVSP > 50 mmHg) or invasively obtained mean pulmonary artery pressure > 25 mmHg was the criteria for entry. RESULTS: There were 2003 patients (52% Women, mean age 56 ±â€¯16.1 years) enrolled. The mean RVSP was 68.2 (SD = 17.9) mmHg. Majority of the study participants (59%) belonged to group 2 of the WHO Nice Classification 2013 (PH secondary to left heart disease). One-fifth (21.2%) belonged to group 1, while 13.3%, 3.8% and 2.4% of the study population belonged to groups 3, 4 and 5 respectively. More than a quarter (27%) reported PH due to left heart disease with valvular disease etiology; while 20.7% had coronary artery disease. The other common etiological factors were chronic obstructive pulmonary disease (10.6%), congenital heart disease (14.6%), idiopathic pulmonary hypertension (5.8%), and chronic thromboembolic pulmonary hypertension (3.8%). Only one of two patients with pulmonary artery hypertension was receiving PH specific therapies. The use of combination therapy was negligible and PH-specific therapies were prescribed off-label to a small proportion of patients too. CONCLUSION: PRO-KERALA is the first PH registry from South Asia and the second largest globally. Left heart diseases attribute to three fifths of patients with PH. Utilization rates of PH specific drug therapies are remarkably lower than the Western population.

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