scholarly journals Takayasu Arteritis – A Systematic Review

2019 ◽  
Vol 46 (3) ◽  
pp. 56-64
Author(s):  
D. Hrisrova ◽  
S. Marchev
Keyword(s):  
Takayasu Arteritis ◽  
Clinical Presentation ◽  
Current Knowledge ◽  
Pulmonary Arteries ◽  
Clinical Manifestations ◽  
Asian Women ◽  
Close Monitoring ◽  
Laboratory Markers ◽  
Laboratory Biomarkers ◽  
Specific Symptoms

Abstract Takayasu arteritis is a chronic, progressive, idiopathic, large-vessel vasculitis that affects the aorta, its main branches and the pulmonary arteries. It typically occurs in young Asian women but can be found in any ethnic group and in men. The disease is named after Mikito Takayasu, a Japanese ophthalmologist, who first described the arteriovenous anomalies in the retina of a patient with the disease in 1908. The etiopathogenesis is not known, but studies are being conducted regarding the immunological, infection and genetic aspects of the disease. Early during the course of the disease, inflammation of the involved arteries progresses, resulting in segmental stenosis, occlusion, dilatation and/or aneurysm. The clinical presentation of Takayasu arteritis varies depending on the blood vessels involved. Early symptoms are nonspecific, making the diagnosis difficult. Subsequently, arterial occlusions occur, producing more specific ischemic symptoms. Paucity of specific symptoms and laboratory biomarkers, as well as difficulties in assessing the disease activity and progression, make it often unrecognized at onset, and its activity is frequently underestimated. The diagnosis is usually confirmed by a combination of clinical manifestations, laboratory markers, diagnostic criteria and imaging methods. The purpose of this review is to address the current knowledge on pathogenesis, investigations, classification and management, and to emphasize the need for timely diagnosis, effective therapeutic intervention, and close monitoring of this disease.

Atypical Presentation of Congenital Yellow Nail Syndrome in a 2-Year-Old Female

2013 ◽  
Vol 17 (1) ◽  
pp. 66-68 ◽  
Author(s):  
Michael Cecchini ◽  
Joseph Doumit ◽  
Nordau Kanigsberg
Keyword(s):  
Rare Case ◽  
Pediatric Patients ◽  
Clinical Presentation ◽  
Positive Family History ◽  
Clinical Manifestations ◽  
Clinical Entity ◽  
Unknown Etiology ◽  
Close Monitoring ◽  
Yellow Nail Syndrome ◽  
Atypical Clinical Presentation

Background: Yellow nail syndrome (YNS) is a rare clinical entity of unknown etiology that is characterized by a triad of yellow nails, respiratory manifestations, and lymphedema. The condition appears in the mid- to later years of life and only rarely in childhood. We describe a rare case of YNS with an atypical clinical presentation consisting of only yellow and dystrophic nails in a 2- year-old female since birth. Objective: A case of congenital YNS with only dystrophic and yellow nails is reported. Methods and Results: A 2-year-old female presented with yellow nails since birth. There was no positive family history. Physical examination revealed 20 thickened, dystrophic, yellow nails with onycholysis. There was no evidence of respiratory manifestations or lymphedema. Conclusion: Although rare, YNS can present as a congenital clinical entity and persist after birth. Pediatric patients with YNS show different clinical manifestations than the classic adult patient. The presence of yellow and dystrophic nails in the absence of respiratory and lymphatic manifestations may be the only sign of pathology and warrants close monitoring as progression to more serious complications can occur.

scholarly journals Takayasu’s Arteritis: Rare Cause of Hypertension in Children

2019 ◽  
Vol 38 (2) ◽  
pp. 140-142
Author(s):  
Reshma Dhakal Lamichhane ◽  
Ram Hari Chapagain
Keyword(s):  
Acute Phase ◽  
Takayasu Arteritis ◽  
Oral Corticosteroid ◽  
Takayasu’S Arteritis ◽  
Pulmonary Arteries ◽  
Large Vessel Vasculitis ◽  
Large Vessel ◽  
Acute Phase Reactants ◽  
Specific Symptoms ◽  
Hypertension In Children

Takayasu arteritis (TA) is a large vessel vasculitis that involves the aorta, its major branches and pulmonary arteries. Diagnosis of TA during childhood remains challenging due to the non-specific symptoms. We report a six years age boy with unresolved hypertension who was later diagnosed as childhood TA. Oral corticosteroid was started 2mg/kg/day. TA is rare in children; Childhood TA must be considered in children who present with, hypertension and increased acute phase reactants.

scholarly journals Leptospiral Infection, Pathogenesis and Its Diagnosis—A Review

Pathogens ◽  
2021 ◽  
Vol 10 (2) ◽  
pp. 145
Author(s):  
Antony V. Samrot ◽  
Tan Chuan Sean ◽  
Karanam Sai Bhavya ◽  
Chamarthy Sai Sahithya ◽  
SaiPriya Chan-drasekaran ◽  
...  
Keyword(s):  
Risk Factors ◽  
Current Knowledge ◽  
Outer Membrane Proteins ◽  
Clinical Manifestations ◽  
Diagnostic Techniques ◽  
Severe Form ◽  
Research Progress ◽  
Systemic Complications ◽  
Pros And Cons ◽  
Specific Symptoms

Leptospirosis is a perplexing conundrum for many. In the existing literature, the pathophysiological mechanisms pertaining to leptospirosis is still not understood in full. Considered as a neglected tropical zoonotic disease, leptospirosis is culminating as a serious problem worldwide, seemingly existing as co-infections with various other unrelated diseases, including dengue and malaria. Misdiagnosis is also common as non-specific symptoms are documented extensively in the literature. This can easily lead to death, as the severe form of leptospirosis (Weil’s disease) manifests as a complex of systemic complications, especially renal failure. The virulence of Leptospira sp. is usually attributed to the outer membrane proteins, including LipL32. With an armament of virulence factors at their disposal, their ability to easily adhere, invade and replicate within cells calls for a swift refinement in research progress to establish their exact pathophysiological framework. As an effort to reconstitute the current knowledge on leptospirosis, the basis of leptospiral infection, including its risk factors, classification, morphology, transmission, pathogenesis, co-infections and clinical manifestations are highlighted in this review. The various diagnostic techniques are also outlined with emphasis on their respective pros and cons.

scholarly journals Late Diagnosis of Takayasu Arteritis with Cardiac Involvement: Case Report

2015 ◽  
Vol 2015 ◽  
pp. 1 ◽  
Author(s):  
Maja Stojanović ◽  
Aleksandra Perić-Popadić ◽  
Sanvila Rašković ◽  
Jasna Bolpačić ◽  
Maja Vučković ◽  
...  
Keyword(s):  
Young Women ◽  
Takayasu Arteritis ◽  
Cardiac Involvement ◽  
Clinical Presentation ◽  
Immunosuppressive Treatment ◽  
Pulmonary Arteries ◽  
Left Ventricular ◽  
Heart Failure Therapy ◽  
Vascular Abnormalities ◽  
Granulomatous Vasculitis

Takayasu arteritis (TA) is an idiopathic chronic granulomatous vasculitis that affects aorta, its main branches and occasionally pulmonary arteries. It is more common in Asian persons, affecting predominantly young women. Clinical presentation is nonspecific at the beginning of the disease, while in the ischemic disease’s stage it depends on the territories affected. We present the case of a 26-year-old woman who was diagnosed as having TA. Multiple vascular abnormalities of aorta and its branches and severely reduced left ventricular function were present at the time of diagnosis. Immunosuppressive treatment consisting of prednisone and azathyoprine along with conventional heart failure therapy significantly improved her cardiac function.

scholarly journals A Reappraisal of Clinical Characteristics of Typhoid Fever

2012 ◽  
Vol 34 (3) ◽  
pp. 80-85 ◽  
Author(s):  
ABM Shahidul Alam ◽  
Sanjana Zaman ◽  
Farhana Chaiti ◽  
Naveen Sheikh ◽  
Gopen Kumar Kundu
Keyword(s):  
Typhoid Fever ◽  
Clinical Presentation ◽  
Current Knowledge ◽  
Clinical Manifestations ◽  
Health Resources ◽  
Signs And Symptoms ◽  
Salmonella Typhi ◽  
Widal Test ◽  
Missed Diagnosis ◽  
The Mean

Background: Recent reports from developing countries show that the clinical presentation, diagnosis and treatment of typhoid have significantly altered often leading to missed diagnosis. The incidence of complications is also reported to be variable. The consequence of missed diagnosis is immense in terms of burden on limited health resources and patients’ suffering. Therefore, its clinical spectrum requires constant reappraisal to update our physicians with current knowledge. This study was carried out to determine the changes in clinical pattern of typhoid fever. Patients & Methods: A total of 106 children, aged up to 14 years, diagnosed primarily as typhoid fever, were included as study population. The diagnostic criteria were either positive blood culture for Salmonella typhi or Salmonella paratyphi or at least a four-fold rise in antibody titre on Widal test. The study included mode of clinical presentations, treatment received before admission, Widal test findings and culture and sensitivity to antibiotics. Results: The mean age of the patients was 5.2 years and males were a little than the females. The mean duration of illness was 11.2±3.3 days. Majority of the patients presented with classical signs and symptoms like step ladder pattern of fever (nearly 70%) coated tongue (69.8%), diarrhoea (49.1%), toxemia (68.9%), relative leucopenia (71.7%), hepatomegaly (55.7%), pain in the right hypochondrium (41.5%) and splennomegaly (18.9%). Very few cases had a typical manifestations. Over 85% of the patients had raised SGPT (>40 IU/L) and 13.8% had detectable jaundice (serum billirubin >3 mg/dl). Widal test demonstrated that about 45% of the patients’ ‘O’ antibody titer increased to 4-fold, 27.4% to 8-fold or more. In case of antibody ‘H’, 35.8% exhibited 4-fold and 39.7% 8-fold or more increase. Of the 103 cases, 68(66%) were positive for Salmonella typhi. Majority of the isolated organisms was sensitive to cefixime, ceftriaxone and gentamycin (83%, 84% and 82% respectively). The second line of sensitivity was obtained to amikacin (64.2%), meropenem (50%), ciprofloxacin (46.2%), imepenem (46.2%) and azithromycin (43.4%). The least sensitive drugs were amoxicillin (28.3%), cotrimoxazole (27.4%) and chloramphenicol (22.6%). Onethird (33.8%) of the patients had multidrug resistant (MDR) strains. However, No significant association was found between multi-drug resistant (MDR) strains and atypical clinical manifestations. Conclusion: Clinical presentation of most typhoid fever still conforms with the classic pattern. High fever, anorexia, coated tongue, diarrhoea, relative leucopenia and hepatosplenomegaly are still common manifestations of typhoid fever. So, majority of the patients could be treated blindly based on clinical diagnosis. However, treatment should be given with first line of drugs like cefexime or ceftriaxone. DOI: http://dx.doi.org/10.3357/bjch.v34i3.10357 BJCH 2010; 34(3): 80-85

scholarly journals AUTOIMMUNE/INFLAMMATORYSYNDROMEINDUCEDBYADJUVANTS (ASIA) - REVISION

2021 ◽  
Vol 9 (5) ◽  
pp. 798-804
Author(s):  
Adriana Novaes Rodrigues
Keyword(s):  
Current Knowledge ◽  
Clinical Manifestations ◽  
Adjuvant Activity ◽  
Autoantibody Production ◽  
Disease Spectrum ◽  
Autoimmune Conditions ◽  
Asia Syndrome ◽  
Specific Symptoms ◽  
Inflammatory Syndrome ◽  
The Relationship

The autoimmune/inflammatory syndrome induced by adjuvants (ASIA), also known as Shoenfeld’s syndrome, encompasses several autoimmune conditions andphenomena that are induced following the exposure to substances with adjuvant activity. The disease spectrum is heterogeneous in respect to clinical presentation as well as severity of the clinical manifestations. Some genetically predisposed individuals can develop generalized non-specific symptoms, autoantibody production, new onset, or worsening of disease presentation. In this review, we focus on the current knowledge presented in the literature on ASIA syndrome, increasing awareness about the basic concepts and highlight the amount of data accumulated in the last few years concerning the relationship between various adjuvants and autoimmunity.

Scrub typhus (Orientia tsutsugamushi), A rapidly spreading epidemic in Chennai - A standalone lab experience

2016 ◽  
Vol 5 (09) ◽  
pp. 4896
Author(s):  
Sripriya C.S.* ◽  
Shanthi B. ◽  
Arockia Doss S. ◽  
Antonie Raj I. ◽  
Mohana Priya
Keyword(s):  
Scrub Typhus ◽  
Clinical Symptoms ◽  
Clinical Manifestations ◽  
Febrile Illness ◽  
Orientia Tsutsugamushi ◽  
The Past ◽  
Igm Elisa ◽  
The Mean ◽  
Specific Symptoms ◽  
Symptoms And Signs

Scrub typhus (Orientia tsutsugamushi), is a strict intracellular bacterium which is reported to be a recent threat to parts of southern India. There is re-emergence of scrub typhus during the past few years in Chennai. Scrub typhus is an acute febrile illness which generally causes non-specific symptoms and signs. The clinical manifestations of this disease range from sub-clinical disease to organ failure to fatal disease. This study documents our laboratory experience in diagnosis of scrub typhus in patients with fever and suspected clinical symptoms of scrub typhus infection for a period of two years from April 2014 to April 2016 using immunochromatography and IgM ELISA methods. The study was conducted on 648 patients out of whom 188 patients were found to be positive for scrub typhus. Results also showed that pediatric (0 -12 years) and young adults (20 – 39 years) were more exposed to scrub typhus infection and female patients were more infected compared to male. The study also showed that the rate of infection was higher between September to February which also suggested that the infection rate is proportional to the climatic condition. Statistical analysis showed that the mean age of the patients in this study was 37.6, standard deviation was 18.97, CV % was 50.45. 

scholarly journals Epilepsy in Down Syndrome: A Highly Prevalent Comorbidity

2021 ◽  
Vol 10 (13) ◽  
pp. 2776
Author(s):  
Miren Altuna ◽  
Sandra Giménez ◽  
Juan Fortea
Keyword(s):  
Down Syndrome ◽  
Functional Outcomes ◽  
Clinical Presentation ◽  
Late Onset ◽  
Current Knowledge ◽  
Epileptic Seizures ◽  
Myoclonic Epilepsy ◽  
Increased Risk ◽  
Number Of Individuals

Individuals with Down syndrome (DS) have an increased risk for epilepsy during the whole lifespan, but especially after age 40 years. The increase in the number of individuals with DS living into late middle age due to improved health care is resulting in an increase in epilepsy prevalence in this population. However, these epileptic seizures are probably underdiagnosed and inadequately treated. This late onset epilepsy is linked to the development of symptomatic Alzheimer’s disease (AD), which is the main comorbidity in adults with DS with a cumulative incidence of more than 90% of adults by the seventh decade. More than 50% of patients with DS and AD dementia will most likely develop epilepsy, which in this context has a specific clinical presentation in the form of generalized myoclonic epilepsy. This epilepsy, named late onset myoclonic epilepsy (LOMEDS) affects the quality of life, might be associated with worse cognitive and functional outcomes in patients with AD dementia and has an impact on mortality. This review aims to summarize the current knowledge about the clinical and electrophysiological characteristics, diagnosis and treatment of epileptic seizures in the DS population, with a special emphasis on LOMEDS. Raised awareness and a better understanding of epilepsy in DS from families, caregivers and clinicians could enable earlier diagnoses and better treatments for individuals with DS.

scholarly journals Pan-American Lancehead Pit-Vipers: Coagulotoxic Venom Effects and Antivenom Neutralisation of Bothrops asper and B. atrox Geographical Variants

Toxins ◽  
2021 ◽  
Vol 13 (2) ◽  
pp. 78
Author(s):  
Lachlan A. Bourke ◽  
Christina N. Zdenek ◽  
Edgar Neri-Castro ◽  
Melisa Bénard-Valle ◽  
Alejandro Alagón ◽  
...  
Keyword(s):  
Evolutionary Biology ◽  
Current Knowledge ◽  
Clinical Manifestations ◽  
In Vivo Studies ◽  
Factor X ◽  
Clotting Factors ◽  
Bothrops Asper ◽  
Species Specific

The toxin composition of snake venoms and, thus, their functional activity, can vary between and within species. Intraspecific venom variation across a species’ geographic range is a major concern for antivenom treatment of envenomations, particularly for countries like French Guiana that lack a locally produced antivenom. Bothrops asper and Bothrops atrox are the most medically significant species of snakes in Latin America, both producing a variety of clinical manifestations, including systemic bleeding. These pathophysiological actions are due to the activation by the venom of the blood clotting factors Factor X and prothrombin, thereby causing severe consumptive coagulopathy. Both species are extremely wide-ranging, and previous studies have shown their venoms to exhibit regional venom variation. In this study, we investigate the differential coagulotoxic effects on human plasma of six venoms (four B. asper and two B. atrox samples) from different geographic locations, spanning from Mexico to Peru. We assessed how the venom variation of these venom samples affects neutralisation by five regionally available antivenoms: Antivipmyn, Antivipmyn-Tri, PoliVal-ICP, Bothrofav, and Soro Antibotrópico (SAB). The results revealed both inter- and intraspecific variations in the clotting activity of the venoms. These variations in turn resulted in significant variation in antivenom efficacy against the coagulotoxic effects of these venoms. Due to variations in the venoms used in the antivenom production process, antivenoms differed in their species-specific or geographical neutralisation capacity. Some antivenoms (PoliVal-ICP, Bothrofav, and SAB) showed species-specific patterns of neutralisation, while another antivenom (Antivipmyn) showed geographic-specific patterns of neutralisation. This study adds to current knowledge of Bothrops venoms and also illustrates the importance of considering evolutionary biology when developing antivenoms. Therefore, these results have tangible, real-world implications by aiding evidence-based design of antivenoms for treatment of the envenomed patient. We stress that these in vitro studies must be backed by future in vivo studies and clinical trials before therapeutic guidelines are issued regarding specific antivenom use in a clinical setting.

scholarly journals The Role of JAK/STAT Molecular Pathway in Vascular Remodeling Associated with Pulmonary Hypertension

2021 ◽  
Vol 22 (9) ◽  
pp. 4980
Author(s):  
Inés Roger ◽  
Javier Milara ◽  
Paula Montero ◽  
Julio Cortijo
Keyword(s):  
Pulmonary Hypertension ◽  
Smooth Muscle ◽  
Pulmonary Artery ◽  
Vascular Remodeling ◽  
Pulmonary Arteries ◽  
Clinical Manifestations ◽  
Different Types ◽  
Artery Remodeling ◽  
Stat Pathway ◽  
Pulmonary Artery Remodeling

Pulmonary hypertension is defined as a group of diseases characterized by a progressive increase in pulmonary vascular resistance (PVR), which leads to right ventricular failure and premature death. There are multiple clinical manifestations that can be grouped into five different types. Pulmonary artery remodeling is a common feature in pulmonary hypertension (PH) characterized by endothelial dysfunction and smooth muscle pulmonary artery cell proliferation. The current treatments for PH are limited to vasodilatory agents that do not stop the progression of the disease. Therefore, there is a need for new agents that inhibit pulmonary artery remodeling targeting the main genetic, molecular, and cellular processes involved in PH. Chronic inflammation contributes to pulmonary artery remodeling and PH, among other vascular disorders, and many inflammatory mediators signal through the JAK/STAT pathway. Recent evidence indicates that the JAK/STAT pathway is overactivated in the pulmonary arteries of patients with PH of different types. In addition, different profibrotic cytokines such as IL-6, IL-13, and IL-11 and growth factors such as PDGF, VEGF, and TGFβ1 are activators of the JAK/STAT pathway and inducers of pulmonary remodeling, thus participating in the development of PH. The understanding of the participation and modulation of the JAK/STAT pathway in PH could be an attractive strategy for developing future treatments. There have been no studies to date focused on the JAK/STAT pathway and PH. In this review, we focus on the analysis of the expression and distribution of different JAK/STAT isoforms in the pulmonary arteries of patients with different types of PH. Furthermore, molecular canonical and noncanonical JAK/STAT pathway transactivation will be discussed in the context of vascular remodeling and PH. The consequences of JAK/STAT activation for endothelial cells and pulmonary artery smooth muscle cells’ proliferation, migration, senescence, and transformation into mesenchymal/myofibroblast cells will be described and discussed, together with different promising drugs targeting the JAK/STAT pathway in vitro and in vivo.

Sign in / Sign up

Export Citation Format

Share Document