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Multiple Organ Systems (multiple + organ_system)
Selected AbstractsPlanar cell polarity effector gene Fuzzy regulates cilia formation and Hedgehog signal transduction in mouseDEVELOPMENTAL DYNAMICS, Issue 12 2009Westley Heydeck Abstract Precise planar cell polarity (PCP) is critical for the development of multiple organ systems in animals. A group of core-PCP proteins are recognized to play crucial roles in convergent extension and other PCP-related processes in mammals. However, the functions of another group of PCP-regulating proteins, the PCP-effector proteins, are yet to be fully studied. In this study, the generation and characterization of a mouse mutant for the PCP effector gene Fuzzy (Fuz) is reported. Fuz homozygous mutants are embryonically lethal, with multiple defects including neural tube defects, abnormal dorsal/ventral patterning of the spinal cord, and defective anterior/posterior patterning of the limb buds. Fuz mutants also exhibit abnormal Hedgehog (Hh) signaling and inefficient proteolytic processing of Gli3. Finally, a significant decrease in cilia was found in Fuz homozygous mutants. In conclusion, Fuz plays an important role in cilia formation, Hh signal transduction, and embryonic development in mammals. Developmental Dynamics 238:3035,3042, 2009. © 2009 Wiley-Liss, Inc. [source] Cardiovascular effects of the thiazolidinedionesDIABETES/METABOLISM: RESEARCH AND REVIEWS, Issue 2 2006Rehan Qayyum Abstract Thiazolidinediones, used for the treatment of diabetes mellitus type 2, modulate gene expression by binding to nuclear transcription factor, peroxisome proliferator-activated receptor-gamma. Peroxisome proliferator,activated receptor-gamma is expressed in several tissues, therefore, thiazolidinediones have biological effects on multiple organ systems. Here, we describe evidence that thiazolidinediones have beneficial effects on the cardiovascular system independent of their antidiabetic effect. Studies in animals have clearly shown that thiazolidinediones decrease blood pressure, left ventricular hypertrophy, development of atherosclerotic lesions, and protect myocardium from ischemia/reperfusion injury. Although relatively few studies in humans have been reported, the preponderance of available evidence suggests a beneficial effect of thiazolidinediones. Thus, by modulating gene expression, thiazolidinediones may provide a novel method for the prevention and treatment of cardiovascular diseases. Copyright © 2005 John Wiley & Sons, Ltd. [source] Bile duct proliferation in liver-specific Jag1 conditional knockout mice: Effects of gene dosage,HEPATOLOGY, Issue 2 2007Kathleen M. Loomes The Notch signaling pathway is involved in determination of cell fate and control of cell proliferation in multiple organ systems. Jag1 encodes a ligand in the Notch pathway and has been identified as the disease-causing gene for the developmental disorder Alagille syndrome. Evidence from the study of human disease and mouse models has implicated Jag1 as having an important role in the development of bile ducts. We have derived a conditional knockout allele (Jag1loxP) to study the role of Jag1 and Notch signaling in liver and bile duct development. We crossed Jag1loxP mice with a transgenic line carrying Cre recombinase under the control of the albumin promoter and ,-fetoprotein enhancer to ablate Jag1 in hepatoblasts. The liver-specific Jag1 conditional knockout mice showed normal bile duct development. To further decrease Notch pathway function, we crossed the Jag1 conditional knockout mice with mice carrying the hypomorphic Notch2 allele, and bile duct anatomy remained normal. When Jag1 conditional mice were crossed with mice carrying the Jag1 null allele, the adult progeny exhibited striking bile duct proliferation. Conclusion: These results indicate that Notch signaling in the liver is sensitive to Jag1 gene dosage and suggest a role for the Notch pathway in postnatal growth and morphogenesis of bile ducts. (HEPATOLOGY 2007.) [source] The ubiquitous role of zinc in health and diseaseJOURNAL OF VETERINARY EMERGENCY AND CRITICAL CARE, Issue 3 2009DACVIM, Julia E. Cummings DVM Abstract Objective , To review zinc physiology and pathophysiology and the importance of zinc toxicity and deficiency in veterinary patients. Data Sources , A review of human and veterinary medical literature. Human Data Synthesis , There is a significant amount of original research in humans and animals on the role of zinc in multiple organ systems. There is also significant data available on human patients with zinc abnormalities. Veterinary Data Synthesis , Zinc deficiency has been studied in dogs with genetic disease and dietary deficiency leading to dermatological disease and immune deficiency. Zinc toxicity has been described after ingestion of metallic foreign bodies containing zinc. Conclusions , Historically, the role of zinc in health and disease has been studied through patients with toxicity or severe deficiency with obvious clinical signs. As the ubiquitous contribution of zinc to structure and function in biological systems was discovered, clinically significant but subtle deficiency states have been revealed. In human medicine, mild zinc deficiencies are currently thought to cause chronic metabolic derangement leading to or exacerbating immune deficiency, gastrointestinal problems, endocrine disorders, neurologic dysfunction, cancer, accelerated aging, degenerative disease, and more. Determining the causal relationships between mild zinc deficiency and concurrent disease is complicated by the lack of sensitive or specific tests for zinc deficiency. The prevalence of zinc deficiency and its contribution to disease in veterinary patients is not well known. Continued research is warranted to develop more sensitive and specific tests to assess zinc status, to determine which patients are at risk for deficiency, and to optimize supplementation in health and disease. [source] Manifestations of systemic sclerosis necessitate a holistic approach to patient care: a case reportMUSCULOSKELETAL CARE, Issue 3 2010Robert J. Moots Abstract This case describes a young woman with manifestations of systemic sclerosis in multiple organ systems and her guidance through two successful pregnancies. This case emphasizes that patients with severe diseases are not just clinical cases; they each have unique needs and concerns beyond the physical manifestations of their disease. Young patients can be a particular challenge due to their need for independence. Communication is the key. Textbook advice in this case was that the risks posed by pregnancy were too great; however, a holistic approach, including frank discussion and much practical support, enabled the patient to make informed choices about her life, with a successful outcome. Copyright © 2010 John Wiley & Sons, Ltd. [source] Anesthesia in HIV-infected childrenPEDIATRIC ANESTHESIA, Issue 6 2007RUENREONG LEELANUKROM Summary In 2005, it was estimated that 2.3 million children below15 years of age were living with human immunodeficiency virus (HIV)/AIDS and 570 000 children below 15 years died. Maternal-infant or vertical transmission is the most common mode of HIV infection in children. As transplacental passage of maternal anti-HIV antibodies, diagnosis of HIV infection in young infants relies on virologic assays. Infants older than 18 months of age can be diagnosed by serology alone. Pediatric HIV infections are classified according to Center for Disease Control and Prevention 1994 revised classification system. The understanding of viral pathogenesis, the development of highly active antiretroviral therapy, and the ability to quantitate viral burden have led to significant reduction in disease progression and morbidity in HIV-infected children. As survival improves, these children will require anesthesia care and pain treatment during the course of their illness. Considerations for the anesthesiologist include: possible involvement of multiple organ systems, adverse reactions and drug interactions of antiretroviral agents and adequate infection control to prevent HIV transmission in hospital and other infections to the immunocompromized patients. Finally, care should be taken not to violate confidentiality. [source] Interferon-regulated chemokines as biomarkers of systemic lupus erythematosus disease activity: A validation studyARTHRITIS & RHEUMATISM, Issue 10 2009Jason W. Bauer Objective Systemic lupus erythematosus (SLE) is a complex autoimmune disease characterized by unpredictable flares of disease activity and irreversible damage to multiple organ systems. An earlier study showed that SLE patients carrying an interferon (IFN) gene expression signature in blood have elevated serum levels of IFN-regulated chemokines. These chemokines were associated with more-severe and active disease and showed promise as SLE disease activity biomarkers. This study was designed to validate IFN-regulated chemokines as biomarkers of SLE disease activity in 267 SLE patients followed up longitudinally. Methods To validate the potential utility of serum chemokine levels as biomarkers of disease activity, we measured serum levels of CXCL10 (IFN,-inducible 10-kd protein), CCL2 (monocyte chemotactic protein 1), and CCL19 (macrophage inflammatory protein 3,) in an independent cohort of 267 SLE patients followed up longitudinally over 1 year (1,166 total clinic visits). Results Serum chemokine levels correlated with lupus activity at the current visit (P = 2 × 10,10), rising at the time of SLE flare (P = 2 × 10,3) and decreasing as disease remitted (P = 1 × 10,3); they also performed better than the currently available laboratory tests. Chemokine levels measured at a single baseline visit in patients with a Systemic Lupus Erythematosus Disease Activity Index of ,4 were predictive of lupus flare over the ensuing year (P = 1 × 10,4). Conclusion Monitoring serum chemokine levels in SLE may improve the assessment of current disease activity, the prediction of future disease flares, and the overall clinical decision-making. [source] Simultaneous scrofuloderma and intracranial tuberculomas: A rare presentation of systemic tuberculosisAUSTRALASIAN JOURNAL OF DERMATOLOGY, Issue 1 2010Kenneth Kien Siang Wong ABSTRACT Tuberculosis can involve multiple organ systems concurrently. We report a case of simultaneous brain tuberculomas and scrofuloderma occurring in the same patient. Skin biopsies confirmed scrofuloderma and the patient was successfully treated for tuberculosis with resolution of the brain masses. This case illustrates the importance of dermatological manifestations of systemic disease as an accessible source for diagnosis and guidance in appropriate therapy. [source] Unusual case of subcutaneous panniculitis-like T-cell lymphomaAUSTRALASIAN JOURNAL OF DERMATOLOGY, Issue 2 2004Jillian Wells SUMMARY An unusual case of subcutaneous panniculitis-like T-cell lymphoma is presented involving multiple organ systems, which eventually culminated in rapid demise from the haemophagocytic syndrome, after an initial protracted course. A 44-year-old man presented in April 2001 with bronchiolitis obliterans organising pneumonia that initially responded well to corticosteroids. However, the condition relapsed on attempted prednisone withdrawal in January 2002 and the patient was noted to have developed truncal subcutaneous nodules. Initial skin biopsy revealed lobular panniculitis, with negative microbiological culture. In July 2002, mononeuritis multiplex was diagnosed after the patient presented with paresthesiae and was treated with pulse cyclophosphamide therapy. By November 2002 there was ulceration of the subcutaneous nodules. Repeat skin biopsy revealed subcutaneous panniculitis-like T-cell lymphoma. The clinical manifestations were supportive of an unifying diagnosis of malignancy involving pulmonary, cutaneous and nervous systems. Combination chemotherapy with fludarabine, mitoxantrone and dexamethasone was commenced. However, the patient deteriorated, with fevers, weight loss, pancytopenia and laboratory features consistent with the haemophagocytic syndrome. Despite maximal supportive therapy the patient succumbed to his disease. [source] Incidence and clinical characteristics of symptomatic choroidal metastasis from lung cancerACTA OPHTHALMOLOGICA, Issue 5 2008Klaus-Martin Kreusel Abstract. Purpose:, To determine the clinical characteristics of symptomatic choroidal metastasis (CM) resulting from metastatic lung cancer. Methods:, Twenty-two consecutive patients with symptomatic CM resulting from lung cancer were retrospectively reviewed for ocular findings, medical history and systemic disease. All patients underwent a complete screening for further organ metastasis by computed tomography (CT) and bone scintigraphy. Annual frequency of CM was determined and compared with the incidence predicted from ocular screening studies. Results:, In eight of 22 (36%; 95% confidence interval [CI] 17,59) patients, lung cancer had been diagnosed before occurrence of CM, with a median interval of 13 months. In 14 patients lung cancer was detected after diagnosis of CM, with a median interval of 1 month. Choroidal metastasis was unilateral, solitary and located close to or at the posterior pole in the majority of patients. Further organ metastasis with a median number of three affected organ systems was present in 19 (86%; 95% CI 65,97) patients. Median survival after diagnosis of symptomatic CM was 13 months, by contrast with 2 months in lung cancer patients with CM identified in an ocular screening study. The mean number of patients in Berlin diagnosed with symptomatic CM was 1.4 per year, which was two orders of magnitude less than predicted from screening studies. Conclusions:, Symptomatic choroidal lung cancer metastasis in the majority of patients presents as a solitary tumour before diagnosis of lung cancer in patients with multiple organ systems affected by metastatic disease. Contrary to predictions from ocular screening studies, it is a rare clinical entity. [source] | ||||||||||