scholarly journals Fabry disease: Guidelines for the evaluation and management of multi-organ system involvement

2006 ◽  
Vol 8 (9) ◽  
pp. 539-548 ◽  
Author(s):  
Christine M Eng ◽  
Dominique P Germain ◽  
Maryam Banikazemi ◽  
David G Warnock ◽  
Christoph Wanner ◽  
...  
Keyword(s):  
Fabry Disease ◽  
Organ System ◽  
System Involvement

Clinical Characteristics and Organ System Involvement in Sarcoidosis: Comparison of the University of Minnesota Cohort and Other Cohorts

2020 ◽  
Author(s):  
Hok Sreng ◽  
David M Perlman ◽  
Chetan Shenoy ◽  
Daniel J Steinberger ◽  
Rebecca J Cogswell ◽  
...  
Keyword(s):  
Clinical Characteristics ◽  
High Variability ◽  
Organ System ◽  
Assessment Instrument ◽  
Genomic Research ◽  
Unknown Etiology ◽  
University Of Minnesota ◽  
System Involvement ◽  
Tertiary Center ◽  
The University

Abstract Background: Sarcoidosis is a systemic granulomatous disease of unknown etiology. Clinical cohort studies of different populations are important to understand the high variability in clinical presentation and disease course of sarcoidosis. The aim of the study is to evaluate clinical characteristics, including organ involvement, pulmonary function tests, and laboratory parameters, in a sarcoidosis cohort at the University of Minnesota. We compare the organ system involvement of this cohort with other available cohorts. Methods: We conducted a retrospective data collection and analysis of 187 subjects with biopsy-proven sarcoidosis seen at a tertiary center. Organ system involvement was determined using the WASOG sarcoidosis organ assessment instrument. Clinical phenotype groups were classified using the Genomic Research in Alpha-1 Antitrypsin Deficiency and Sarcoidosis criteria. Results: Mean subject age at diagnosis was 45.8 ± 12.4, with a higher proportion of males (55.1%), and a higher proportion of blacks (17.1%) compared to the racial distribution of Minnesota residents (5.95%). The majority (71.1%) of subjects required anti-inflammatory therapy for at least 1 month. Compared to the A Case Control Etiologic Study of Sarcoidosis cohort, there was a higher frequency of extra-thoracic lymph node (34.2% vs. 15.2%), eye (20.9% vs. 11.8%), liver (17.6% vs. 11.5%), spleen (20.9% vs. 6.7%), musculoskeletal (9.6% vs. 0.5%), and cardiac (10.7% vs. 2.3%) involvement in our cohort. A multisystem disease with at least five different organs involved was identified in 13.4% of subjects. A restrictive physiological pattern was observed in 21.6% of subjects, followed by an obstructive pattern in 17.3% and mixed obstructive and restrictive pattern in 2.2%. Almost half (49.2%) were Scadding stages II/III. Commonly employed disease activity markers, including soluble interleukin-2 receptor and angiotensin-converting enzyme, did not differ between treated and untreated groups. Conclusions: This cohort features a relatively high frequency of high-risk sarcoidosis phenotypes including cardiac and multiorgan disease. Commonly-utilized serum biomarkers do not identify subpopulations that require or do better with treatment. Findings from this study present further the high-variability nature of sarcoidosis and the need for a more reliable biomarker to predict and measure disease severity and outcomes for better clinical management for sarcoidosis patients.

scholarly journals Animal Virus Ecology and Evolution Are Shaped by the Virus Host-Body Infiltration and Colonization Pattern

Pathogens ◽  
2019 ◽  
Vol 8 (2) ◽  
pp. 72
Author(s):  
Jan Slingenbergh
Keyword(s):  
Virus Transmission ◽  
Mass Rearing ◽  
Virus Genome ◽  
Organ System ◽  
Emerging Viruses ◽  
Cell Infection ◽  
Host Environment ◽  
Animal Virus ◽  
Host Tropism ◽  
System Involvement

The current classification of animal viruses is largely based on the virus molecular world. Less attention is given to why and how virus fitness results from the success of virus transmission. Virus transmission reflects the infection-shedding-transmission dynamics, and with it, the organ system involvement and other, macroscopic dimensions of the host environment. This study describes the transmission ecology of the world main livestock viruses, 36 in total, a mix of RNA, DNA and retroviruses. Following an iterative process, the viruses are virtually ranked in an outer- to inner-body fashion, by organ system, on ecological grounds. Also portrayed are the shifts in virus host tropism and virus genome. The synthesis of the findings reveals a predictive virus evolution framework, based on the outer- to inner-body changes in the interplay of host environment-transmission modes-organ system involvement-host cell infection cycle-virus genome. Outer-body viruses opportunistically respond to the variation in the external environment. For example, respiratory and enteric viruses tend to be associated with poultry and pig mass rearing. Ruminant and equine viruses tend to be more deep-rooted and host-specific, and also establish themselves in the vital inner-body systems. It is concluded that the framework may assist the study of new emerging viruses and pandemic risks.

scholarly journals Clinical characteristics and organ system involvement in sarcoidosis: comparison of the University of Minnesota Cohort with other cohorts

2020 ◽  
Vol 20 (1) ◽  
Author(s):  
Hok Sreng Te ◽  
David M. Perlman ◽  
Chetan Shenoy ◽  
Daniel J. Steinberger ◽  
Rebecca J. Cogswell ◽  
...  
Keyword(s):  
Clinical Characteristics ◽  
Organ System ◽  
University Of Minnesota ◽  
System Involvement ◽  
The University

A Fatal Case of Pyoderma Gangrenosum with Multiple Organ System Involvement: Unexpected Progression: Case Report

2015 ◽  
Vol 25 (2) ◽  
pp. 67-70
Author(s):  
Suzan D. PEKTAŞ ◽  
Neriman D. ALTINTAŞ ◽  
Seval İZDEŞ ◽  
Sibel YÜCEL ◽  
Hayriye T. DOĞAN
Keyword(s):  
Case Report ◽  
Pyoderma Gangrenosum ◽  
Fatal Case ◽  
Multiple Organ ◽  
Organ System ◽  
System Involvement ◽  
Multiple Organ System

scholarly journals Early diagnosis of peripheral nervous system involvement in Fabry disease and treatment of neuropathic pain: the report of an expert panel

BMC Neurology ◽  
2011 ◽  
Vol 11 (1) ◽  
Author(s):  
Alessandro P Burlina ◽  
Katherine B Sims ◽  
Juan M Politei ◽  
Gary J Bennett ◽  
Ralf Baron ◽  
...  
Keyword(s):  
Nervous System ◽  
Neuropathic Pain ◽  
Early Diagnosis ◽  
Fabry Disease ◽  
Peripheral Nervous System ◽  
Expert Panel ◽  
System Involvement

scholarly journals Spectrum of Multi-Organ System Involvement in Perinatal Asphyxia at a Tertiary Care Hospital in Southern India - A Descriptive Study

2021 ◽  
Vol 8 (35) ◽  
pp. 3230-3235
Author(s):  
Soumya Satna ◽  
Ramadevi Devagudi ◽  
Ramu Pedada ◽  
Narayana Lunavath
Keyword(s):  
Intensive Care Unit ◽  
Intensive Care ◽  
Birth Weight ◽  
Cardiovascular System ◽  
Perinatal Asphyxia ◽  
Descriptive Study ◽  
Organ System ◽  
Care Hospital ◽  
Maternal Risk ◽  
System Involvement

BACKGROUND Perinatal asphyxia (PA) is an important cause of neonatal morbidity, mortality, and neurologic handicap in children. Dysfunction of organs other than central nervous system is often recognised after perinatal asphyxia and manifests as hypoxic ischaemic insults to heart, lungs, kidneys and bowel. The purpose of this study was to assess the spectrum of multi-organ system involvement in perinatal asphyxia. METHODS This observational, descriptive study was conducted at SVRRGGH (Sri Venkateswara Ramnarayan Ruia Government General Hospital) - Tirupati from October 2010 to September 2011 and has Institutional Ethics Committee approval (Regd. No: 58647, Dt: 20 / 11 / 2010). After considering the inclusion and exclusion criteria, 204 neonates diagnosed with perinatal asphyxia who got admitted in our newborn intensive care unit (NICU) were included in this study. RESULTS In the present study, we had 118 (57.89 %) male babies and 86 (42.11 %) female babies. The mean birth weight was 2640 +/- 460 grams. Infants of birth weight 2500 - 4000 grams (appropriate for gestational age - AGA) accounted for 202 (98.96 %). Major maternal risk factors in this study were MSAF (meconium-stained amniotic fluid (66/204, 32.4 %), PIH (pregnancy induced hypertension) and Eclampsia (26/204, 12.7 %) and PROM (premature rupture of membranes) (26/204, 12.7 %). In the present study, we found higher mortality (19/117, 16.2 %) in babies born to multiparous mothers. Respiratory system involvement was seen in 80 (39.2 %) infants. Renal involvement was observed in 58 (27.5 %) infants. Acute renal failure was diagnosed in 22 (10.8 %) cases. CVS (cardiovascular system) involvement was seen in 56 (27.5 %) cases where as GIT (gastrointestinal tract) involvement was found in 32 (15.68 %) cases. CONCLUSIONS Epidemiological research is needed to accurately estimate the contribution of birth asphyxia to perinatal morbidity and mortality, especially in community settings where the burden of disease, due to high proportion of unattended deliveries, is likely to be larger than the hospital setting. KEYWORDS Perinatal Asphyxia, Neonatal Intensive Care Unit, Hypoxic Ischaemic Encephalopathy, Multi Organ Dysfunction, Cardiovascular System

Ventricular Tachyarrhythmia Due to Cardiac Sarcoidosis in a Child

PEDIATRICS ◽  
1978 ◽  
Vol 62 (3) ◽  
pp. 322-325
Author(s):  
Gerald A. Serwer ◽  
Sam B. Edwards ◽  
D. Woodrow Benson ◽  
Page A. W. Anderson ◽  
Madison Spach
Keyword(s):  
Heart Failure ◽  
Congestive Heart Failure ◽  
Ventricular Tachycardia ◽  
Cardiac Sarcoidosis ◽  
Diuretic Therapy ◽  
Ventricular Tachyarrhythmia ◽  
Disease Process ◽  
Cor Pulmonale ◽  
Organ System ◽  
System Involvement

Cardiac involvement by systemic sarcoidosis is well known, but occurs rarely. It usually manifests as either heart block, heart failure due to direct myocardial involvement, or cor pulmonale. We present the case of a patient with cardiac sarcoidosis who had ventricular tachycardia and congestive heart failure. Although there was other organ system involvement, the cardiac manifestation was the first to become clinically apparent. Therapy consisted of quinidine sulfate to control the arrhythmias and chronic diuretic therapy to control congestive heart failure. Steroid therapy was initially associated with recurrence of the ventricular tachycardia and was discontinued. It was reinstituted 18 months later when other organ system involvement developed with no recurrence of the ventricular tachyarrhythmia. The patient responded well to therapy and is currently doing well. This case is presented to illustrate a somewhat unusual, but nevertheless important, etiology of ventricular tachyarrhythmias. The recognition of underlying sarcoidosis is critical because of the propensity for other organ system involvement by this disease process.

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