Involvement Of Different Organs Research Articles

Non-invasive cardiovascular imaging for evaluating subclinical target organ damage in hypertensive patients: A consensus paper from the European Association of Cardiovascular Imaging (EACVI), the European Society of Cardiology Council on Hypertension, and the European Society of Hypertension (ESH).

Arterial hypertension (HTN) accounts for the largest amount of attributable cardiovascular (CV) mortality worldwide, and risk stratification in hypertensive patients is of crucial importance to manage treatment and prevent adverse events. Asymptomatic involvement of different organs in patients affected by HTN represents an independent determinant of CV risk and the identification of target organ damage (TOD) is recommended to further reclassify patients' risk. Non-invasive CV imaging is progressively being used and continues to provide new technological opportunities to TOD evaluation at early stage. The aim of this article is to provide the community of cardiology with an update on appropriate and justified use of non-invasive imaging tests in the growing population of hypertensive patients.

Abdominal tuberculosis: a radiological review with emphasis on computed tomography and magnetic resonance imaging findings.

Tuberculosis is a disease whose incidence has increased principally as a consequence of HIV infection and use of immunosuppressive drugs. The abdomen is the most common site of extrapulmonary tuberculosis. It may be confused with several different conditions such as inflammatory bowel disease, cancer and other infectious diseases. Delay in the diagnosis may result in significantly increased morbidity, and therefore an early recognition of the condition is essential for proper treatment. In the present essay, cases with confirmed diagnosis of abdominal tuberculosis were assessed by means of computed tomography and magnetic resonance imaging, demonstrating the involvement of different organs and systems, and presentations which frequently lead radiologists to a diagnostic dilemma. A brief literature review was focused on imaging findings and their respective prevalence.

Recombinant Enzyme Replacement Therapy in Hypophosphatasia.

Hypophosphatasia (HPP) is a rare monogenetic and multisystemic disease with involvement of different organs, including bone, muscle, kidney, lung, gastrointestinal tract and the nervous system. The exact metabolic mechanisms of the effects of TNAP deficiency in different tissues are not understood in detail. There is no approved specific treatment for HPP; therefore symptomatic treatment in order to improve the clinical features is of major interest. Enzyme replacement therapy (ERT) is a relatively new type of treatment based on the principle of administering a medical treatment replacing a defective or absent enzyme. Recently ERT with a bone targeted recombinant human TNAP molecule has been reported to be efficient in ten severely affected patients and improved survival of life threatening forms. These results are very promising especially with regard to the skeletal phenotype but it is unclear whether ERT also has beneficial effects for craniosynostosis and in other affected tissues in HPP such as brain and kidney. Long-term data are not yet available and further systematic clinical trials are needed. It is also necessary to establish therapeutic approaches to help patients who are affected by less severe forms of HPP but also suffer from a significant reduction in quality of life. Further basic research on TNAP function and role in different tissues and on its physiological substrates is critical to gain a better insight in the pathogenesis in HPP. This and further experiences in new therapeutic strategies may improve the prognosis and quality of life of patients with all forms of HPP.

Role of Serum TNFα Level in Atherosclerosis in Egyptian Systemic Lupus Erythematosus Patients

Systemic lupus erythematosus (SLE) is an autoimmune rheumatic disease that principally affects women in their childbearing years. Many cytokines have been implicated in regulating disease activity and the involvement of different organs in patients with SLE. The study was conducted to determine serum tumor necrosis factor alpha (TNFα) levels in SLE patients and its relation to clinically active disease and to assess the possible role of serum TNFα level in atherosclerosis. Thirty Egyptian female patients with SLE and 15 matched healthy control subjects had serum TNFα levels measured. In addition, the relation of common carotid artery intima-media thickness (CIMT) to serum TNFα was evaluated. Mean serum TNFα level of SLE patients was 48.36 ± 58.27 pg/ml, significantly different from the control group (17.62 ± 16.63 pg/ml; P = .014). There was a statistically significant difference in right carotid CIMT between SLE patients (0.06 ± 0.01 cm) and the control group (0.054 ± 0.009; P = .002). There was also a statistically significant difference in left carotid CIMT between lupus nephritis patients (0.07 ± 0.01) and non–lupus nephritis patients (0.06 ± 0.01; P = .027). These data indicate the activation of immune response in SLE patients suggested by their high levels of TNFα. Increased CITM in lupus patients was not related to TNFα and the lipid profile, suggesting an underlying etiology for atherosclerosis in lupus patients other than those factors. High CIMT in nephritis patients denote the role of nephritis in atherosclerosis.

QUALITY-OF-LIFE ISSUES IN THE COMPREHENSIVE EXAMINATION OF PATIENTS WITH SYSTEMIC LUPUS ERYTHEMATOSUS

Система комплексной поддержки и сопровождения научного журнала Elpub позволяет запустить двуязычный сайт журнала со встроенной системой электронной редакции в соответствии с лучшими издательскими практиками, а так же предусматривает техническую и методическую поддержку со стороны специалистов НЭИКОН.

Therapeutic competence of dried garlic powder (Allium sativum) on biochemical parameters in lead (Pb) exposed broiler chickens

The study was conducted to assess the therapeutic competence of garlic (Allium sativum) in lead (Pb) exposed chickens. The experimental birds (n=350) were grouped into T0 (as control), T1, T2, T3 and T4. The birds of group T1 was provided with lead acetate at 100 mg/kg body weight. Group T2 had lead acetate at 100 mg/kg b.wt. + 1% garlic supplement, whereas group T3 was fed with lead acetate at 100 mg/kg b.wt. + 2% garlic supplement, and group T4 had lead acetate at 100 mg/kg b.wt. + 4% garlic supplement. The mean values (mg/dL) of uric acid, blood urea nitrogen, creatinine, cholesterol, triglycerides, low density lipoprotein, high density lipoprotein, and blood glucose in the birds of group T1 were significantly increased (p<0.01) on day 42 of post-treatment. Elevation of these parameters was suggestive for the pathological involvement of different organs like liver, kidney, muscles. Statistical analysis of variance indicated that lead acetate at 100 mg/kg b.wt. + 2% garlic supplement (T3) resulted significant (p<0.01) ameliorative effect on the biochemical parameters as compared to the group T2 and T4. In conclusion, potency of garlic in reversion of the values of the biochemical properties in Pb exposed chickens was close to the normal levels of the values. http://dx.doi.org/10.5455/javar.2014.a34

Behçet’s disease in clinical practice

Behçet’s disease is a chronic relapsing multisystemic vasculitis involving vessels of different diameter. The disease is characterized by involvement of skin and mucosa, eyes, gastrointestinal tract, joints, vessels, genitourinary system and neurological symptoms. According to data, male/female ratio is 2-10:1 The etiology of the disease is still unclear. Diagnosis is mainly based on thorough analysis of clinical manifestations. According to the International Study Group for Behçet’s Disease Diagnostic Criteria, combination of recurrent oral aphthosis and at least any two of the following: severe or scarring genital aphthosis, eye lesions (posterior uveitis, retinal vasculitis), skin lesions (nodular erythema, pseudofolliculitis, acne-like lesions) and positive pathergy test is needed to classify the patient as a patient with Behçet’s disease. Several clinical subtypes of Behçet’s disease are distinguished depending on predominant involvement of different organs and systems, including skin and mucosa, joints, eyes, or neurological manifestations. There are no specific laboratory tests for Behçet’s disease. The treatment approaches are variable depending on clinical manifestations. The effect of systemic corticosteroids, colchicine, azathioprine, chlorambucil, cyclophosphamide, sulfasalazine, methotrexate, cyclosporine, infliximab was proven. All the abovementioned requires the good clinical knowledge on Behçet’s disease from practicing doctors of different specialties, particularly - physicians. A case of highly active acute Behçet’s disease with nose, pharynx, eye and oral cavity mucosa, scrotum skin involvement is presented in the article. Questions of differential diagnosis are reviewed, specific treatment program with efficacy analysis is presented.

Intrafamilial phenotypic variability in four families with Anderson‐Fabry disease

We analysed the clinical history of 16 hemizygous males affected by Anderson-Fabry Disease, from four families, to verify their intrafamilial phenotypic variability. Seven male patients, ranging from 26 to 61 years of age, died, whereas nine (age range 23-55) are alive. Eleven patients have undergone enzyme replacement therapy (ERT) for a period of 5-10 years. We have found a wide range of intrafamilial phenotypic variability in these families, both in terms of target-organs and severity of the disease. Overall, our findings confirm previous data from the literature showing a high degree of intrafamilial phenotypic variability in patients carrying the same mutation. Furthermore, our results underscore the difficulty in giving accurate prognostic information to patients during genetic counselling, both in terms of rate of disease progression and involvement of different organs, when such prognosis is solely based on the patient's family history.

AB0676 The clinical relevance of anti-RO52 and anti-RO60 in patients in the united kingdom primary sjogren’s syndrome registry (UKPSSR)

BackgroundPrimary Sjögren’s syndrome (pSS) is a multi-system autoimmune disease. Anti-Ro is the most commonly associated antibody. This can be differentiated into two subtypes, anti-Ro52 and anti-Ro60, each specific to different...

Clinical and biochemical features associated with BCS1L mutation

Our study describes a novel phenotype in a series of nine Saudi patients with lactic acidosis, from four consanguineous families three of which are related. Detailed genetic studies including linkage, homozygosity mapping and targeted sequencing identified a causative mutation in the BCS1L gene. All affected members of the families have an identical mutation in this gene, mutations of which are recognized causes of Björnstad syndrome, GRACILE syndrome and a syndrome of neonatal tubulopathy, encephalopathy, and liver failure (MIM 606104) leading to isolated mitochondrial respiratory chain complex III deficiency. Here we report the appearance of a novel behavioral (five patients) and psychiatric (two patients) phenotype associated with a p.Gly129Arg BCS1L mutation, differing from the phenotype in a previously reported singleton patient with this mutation. The psychiatric symptoms emanated after childhood, initially as hypomania later evolving into intermittent psychosis. Neuroradiological findings included subtle white matter abnormalities, whilst muscle histopathology and respiratory chain studies confirmed respiratory chain dysfunction. The variable neuro-psychiatric manifestations and cortical visual dysfunction are most unusual and not reported associated with other BCS1L mutations. This report emphasizes the clinical heterogeneity associated with the mutation in BCS1L gene, even within the same family and we recommend that defects in this gene should be considered in the differential diagnosis of lactic acidosis with variable involvement of different organs.

Total serum IgE levels and atopic status in patients with sarcoidosis

To date, two studies have reported lower total serum immunoglobulin E (IgE) levels and lower prevalence of atopy in patients with sarcoidosis compared with healthy subjects. However, those reports did not consider age or gender differences between cases and controls. In addition, the association between total serum IgE levels and clinical manifestations of sarcoidosis has not been clarified. This study assessed total serum IgE levels and prevalence of atopy in patients with sarcoidosis after taking age and sex differences into account and evaluated associations between total serum IgE levels and clinical manifestations of sarcoidosis. Total serum IgE levels and prevalence of atopy on initial visits were compared between 189 patients with sarcoidosis and 378 age- and sex-matched controls. Associations between total serum IgE levels and involvement of each affected organ were evaluated. Changes in total serum IgE levels during the clinical course of sarcoidosis were also evaluated. Total serum IgE levels were significantly lower in patients with sarcoidosis than in controls, independent of atopic status (atopic subjects, p = 0.025; nonatopic subjects, p < 0.001). Total serum IgE levels did not differ according to the involvement of different organs. Total serum IgE levels decreased further, albeit only slightly, after disease remission (p < 0.001). Increased susceptibility to sarcoidosis may be attributable to several underlying genetic or environmental factors that result in lower total serum IgE levels.

Hydatidosis in human and slaughtered herbivores in Mazandaran province, northern Iran

Objective To determine the rate of hydatid cyst in hospitalized patients and slaughtered animals in Mazandaran province, northern Iran. Methods In this descriptive study, files of 34 hospitalized patients who underwent surgery for hydatid cysts during 2003-2008 in Mazandaran province were analyzed. Also, 1 678 343 sheep, 815 635 goats, 451 743 cattle and 1 130 buffaloes were examined carefully for the presence of hydatid cysts in slaughterhouse of Mazandaran province, during 2003-2008. Results In human, the highest rate of hydatidosis was related to 30-39 age group. Infection rate in females was higher than that in males (61.8% vs 31.2%). Cyst involvement of different organs was as follows: liver 76.5%, lung 13.5%, spleen 5.4%, gall bladder 2.7% and abdomen septum 2.9%. In slaughtered animals, overall, 6.38% had liver cyst and 12.70% had lung cyst. Involvement of liver and lung in sheep, goat, cattle and buffalo were 7.09% and 14.70%; 5.12% and 10.20%; 5.97% and 9.60%; and 14.90% and 13.18%, respectively. In sheep, goat and cattle the most infected organ was lung but in buffalo it was liver. Conclusions The results show that in human, all age groups and both sexes are exposed to hydatidosis. In livestock it causes economic loss to veterinary industries. Therefore, appropriate control method is needed to prevent this zoonotic disease.

Clinical and Imaging Findings in the Alveolar Echinococcosis

Aim: To review the imaging features of alveolar echinococcosis in eight patients with involvement of different organs. Material and Methods: Ultrasound (US) and computed tomography (CT) examinations were performed in all patients, and magnetic resonance imaging (MRI) and color Doppler ultrasound (CDUS) were performed for two patients. Three patients had involvement of only the liver; two had involvement of lung and liver; one had involvement of only the lung; one had involvement of liver and sternum; and one had lung, liver, and adrenal involvement. The diagnosis was confirmed by histopathologic examination in all patients. Results: The lesions located in the liver were generally seen as heterogeneous hypoechogenic on US, hypodense on CT, and hypointense solid masses on MRI. These masses included necrosis and calcifications. Vascular flow on CDUS and contrast enhancement on CT and MRI were not observed in any of the lesions. On CT, lung lesions were seen as multiple, nodular, well-defined or ill-defined opacities; some had cavitations caused by necrosis. Conclusion: Knowledge of imaging characteristics of alveolar echinococcosis makes it possible to provide an early diagnosis radiologically. In addition, radiologic findings have an important role in the follow-up. Ultrasound, CT, and MRI are complementary methods in the diagnosis of alveolar echinococcosis.

Antiphospholipid syndrome dilemmas still to be solved: 2008 status

Extensive investigation of antiphospholipid syndrome (APS) has improved our knowledge of the disease, but raised new questions. Although the pathogenic role of antiphospholipid antibodies (aPL) is widely accepted, the fact...

Treatment Options in Steroid-Refractory Acute Graft-Versus-Host Disease Following Hematopoietic Stem Cell Transplantation

To evaluate the treatment options in steroid-refractory acute graft-versus-host disease (GVHD) following hematopoietic stem cell transplantation. Literature was obtained by searching MEDLINE (1966-May 2007) and EMBASE (1980-May 2007). All pertinent clinical trials, retrospective studies, case reports, and compassionate use studies were identified and evaluated for safety and efficacy of the pharmacologic agents. Steroid-refractory acute GVHD is associated with high rates of morbidity and mortality. Although various pharmacologic agents have been studied in the treatment of steroid-refractory acute GVHD, no treatments have been established as a salvage therapy. Preliminary data on different pharmacologic agents have been identified and evaluated for their efficacy and tolerability in the treatment of steroid-refractory acute GVHD. The effects of the pharmacologic agents varied significantly among patients: severity of the disease, involvement of different organs, and the patient's age seem to be the major factors that affect an individual's response to drug therapy. In addition, the treatments are further challenged by the high incidence of potentially fatal opportunistic infections that occur during the therapy. Selection of pharmacologic agents for the treatment of steroid-refractory acute GVHD should be based on the target organs, adverse drug reactions, and economic factors. Further studies with larger sample sizes are warranted to better understand the roles of these agents in the treatment of steroid-refractory acute GVHD.

Gastrointestinal Involvement in Pediatric Polyarteritis Nodosa

Gastrointestinal Involvement in Pediatric Polyarteritis Nodosa

Systemic amyloidosis: a clinical challenge

This study reports a case of an 82-year-old white woman with a history of congestive heart failure refractory to diuretic therapy who was found to have systemic amyloidosis, confirmed by a rectal biopsy. Cardiac involvement by amyloid should be considered in the differential diagnosis of any patient with heart failure with preserved ventricular systolic function, who does not have evidence of ischaemic heart disease or hypertension. However, the rarity of this disease and the various involvement of different organs and tissues are often responsible for missed or delayed diagnosis. Systemic amyloidosis is a life-threatening disease but an early diagnosis may modify its course; therefore it is important to maintain a high clinical suspicion and to increase the awareness of this overlooked condition among clinicians.

Síndrome de Marfán

Marfan Syndrome is an autosomic dominant genetic disorder of the elastic fibers of connective tissue. Although neonatal and infant forms of the disease exist, the classic Marfan Syndrome is the most frequent form of presentation in childhood and adolescence, with a hereditary background in 70 to 85% of cases. Due to the natural evolution of the disease, there is a progressive involvement of different organs or systems such as skeletal, cardiovascular, dura, ocular, skin-integument and lungs. However, the suspicion must arise on skeletal clinical aspects which are first evident signs. The cardiovascular involvement appears later but is the major life threatening complication. When suspecting Marfan phenotype, it is mandatory to apply Ghent criteria based on family history and clinical findings to establish the diagnosis. If diagnosis is confirmed, the severity of organ involvement must be assessed, to take preventive and/or therapeutic measures, including the search of new cases among relatives. When patients do not fulfill the diagnostic criteria, they must have a yearly evaluation considering the natural progressive evolution of the disease. The aim of this review is to spread and unify criteria on this disease whose diagnosis is eminently clinical, that requires early integral and updated management by a multidisciplinary group, to obtain the best quality of life and survival.

Amyloidosis

Amyloidosis is a clinical disorder caused by extracellular deposition of insoluble abnormal fibrils, derived from aggregation of misfolded, normally soluble, protein. About 23 different unrelated proteins are known to form amyloid fibrils in vivo, which share a pathognomonic structure although they are associated with clinically distinct conditions. Systemic amyloidosis, with amyloid deposits in the viscera, blood vessel walls, and connective tissue, is usually fatal and is the cause of about one per thousand deaths in developed countries. This rarity and the variable involvement of different organs and tissues are often responsible for missed or delayed diagnosis, and amyloidosis remains a considerable clinical challenge. However, recent elucidation of important aspects of pathogenesis, as well as developments in diagnosis, monitoring, and treatment, have greatly improved outcomes, especially when patients are managed in specialist centers.

QUANTIFICATION OF PRION GENE EXPRESSION IN BRAIN AND PERIPHERAL ORGANS OF GOLDEN HAMSTER BY REAL-TIME RT-PCR

ABSTRACT Determination of tissue-specific expression of cellular prion protein (PrPc) is essential for understanding its poorly explained role in organisms. Herein we report on quantification of PrP mRNA in golden hamsters, a popular experimental model for studying mechanisms of transmissible spongiform encephalopathies (TSE), by real-time RT-PCR. Total RNA was isolated from four different regions of the brain and six peripheral organs of eight golden hamsters. PrP mRNA copy numbers were determined using absolute standard curve method with real-time quantitative PCR instrument. It was found that high mRNA levels were present in all four regions of the brain examined, including obex, neocortex, cerebellum, and thalamus. In peripheral organs examined, inguinal lymph node showed high level of the expression similar to that in overall brain; spleen, heart, liver, and lung showed moderate levels of the expression; and kidney showed the lowest expression. Our result is consistent with the potential involvement of different organs in prion diseases and offers essential data for further study of TSE mechanism in this animal model.