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Sample records for renal syndrome virus

  1. Virus-Associated Hemophagocytic Syndrome in Renal Transplant Recipients: Report of 2 Cases from a Single Center

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    Koji Nanmoku

    2015-01-01

    Full Text Available Virus-associated hemophagocytic syndrome (HPS is a potentially fatal complication of immunosuppression for transplantation. However, it presents with heterogeneous clinical symptoms (fever, disturbed consciousness, and hepatosplenomegaly and laboratory findings (pancytopenia, elevated hepatic enzyme levels, abnormal coagulation, and hyperferritinemia, impeding diagnosis. Case 1: A 39-year-old female developed fever 4 years after ABO-incompatible living-related renal transplantation. Laboratory findings revealed thrombocytopenia, elevated hepatic enzymes, Epstein-Barr virus (EBV DNA seropositivity, and hyperferritinemia. EBV-associated HPS was confirmed by bone marrow aspiration. Steroid pulse therapy and etoposide were ineffective. Disseminated intravascular coagulation resulted in multiple organ failure, and the patient died 32 days after disease onset. Case 2: A 67-year-old male was admitted with rotavirus enteritis a month after living-unrelated renal transplantation. He developed sudden-onset high fever, disturbance of consciousness, and tachypnea 8 days after admission. Laboratory findings revealed elevated hepatic enzyme levels, hyperkalemia, and hyperferritinemia. Emergency continuous hemodiafiltration ameliorated the fever, and steroid pulse therapy improved abnormal laboratory values. Varicella-zoster virus meningitis was confirmed by spinal tap. Acyclovir improved consciousness, and he was discharged 87 days after admission. Fatal virus-associated HPS may develop in organ transplant patients receiving immunosuppressive therapy. Pathognomonic hyperferritinemia is useful for differential diagnosis.

  2. Branchio-oto-renal syndrome.

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    Jalil, Jawad; Basheer, Faisal; Shafique, Mobeen

    2014-05-01

    The association of branchial arch anomalies (branchial cysts, branchial fistulas), hearing loss and renal anomalies constitutes the branchio-oto-renal (BOR) syndrome also known as Melnick Fraser syndrome. We present a case of this rare disorder in a girl child who presented with profound deafness, preauricular pits, branchial sinuses and renal hypoplasia.

  3. OCULO-CEREBRO-RENAL SYNDROME (LOWE'S SYNDROME)

    Institute of Scientific and Technical Information of China (English)

    1991-01-01

    Oculo-cerebro-renal syndrome (Lowe's syndrome) is characterized by mental and motor retardation, cataract, glaucoma and renal abnormalities. It is an X-linked recessive metabolic disease. Two brothers suffering from Lowe's syndrome are reported. Their mother with lenticular opacities and peculiar facial appearance is in concordance with the obligate carrier. The ocular changes and heridity are discussed.

  4. [Pulmonary-renal syndrome].

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    Risso, Jorge A; Mazzocchi, Octavio; De All, Jorge; Gnocchi, César A

    2009-01-01

    The pulmonary-renal syndrome is defined as a combination of diffuse alveolar hemorrhage and glomerulonephritis. The coexistence of these two clinical conditions is due to diseases with different pathogenic mechanisms. Primary systemic vasculitis and Goodpasture syndrome are the most frequent etiologies. Systemic lupus erythematosus, connective tissue diseases, negative anti neutrophil cytoplasmic antibody vasculitis and those secondary to drugs are far less common causes. An early diagnosis based on clinical, radiologic, laboratory and histologic criteria enables early treatment, thus diminishing its high morbidity-mortality rate. Therapy is based on high doses of corticosteroids, immunosuppressants, tumor necrosis factor inhibitors and plasmapheresis.

  5. Hemorrhagic Fever with Renal Syndrome (HFRS)

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    ... this page: About CDC.gov . Share Compartir Hemorrhagic Fever with Renal Syndrome (HFRS) On this Page What ... is HFRS prevented? Suggested Reading What is hemorrhagic fever with renal syndrome? Hemorrhagic fever with renal syndrome ( ...

  6. Dobrava virus carried by the yellow-necked field mouse Apodemus flavicollis, causing hemorrhagic fever with renal syndrome in Romania.

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    Panculescu-Gatej, Raluca Ioana; Sirbu, Anca; Dinu, Sorin; Waldstrom, Maria; Heyman, Paul; Murariu, Dimitru; Petrescu, Angela; Szmal, Camelia; Oprisan, Gabriela; Lundkvist, Ake; Ceianu, Cornelia S

    2014-05-01

    Hemorrhagic fever with renal syndrome (HFRS) has been confirmed by serological methods during recent years in Romania. In the present study, focus-reduction neutralization tests (FRNT) confirmed Dobrava hantavirus (DOBV) as the causative agent in some HFRS cases, but could not distinguish between DOBV and Saaremaa virus (SAAV) infections in other cases. DOBV was detected by a DOBV-specific TaqMan assay in sera of nine patients out of 22 tested. Partial sequences of the M genomic segment of DOBV were obtained from sera of three patients and revealed the circulation of two DOBV lineages in Romania. Investigation of rodents trapped in Romania found three DOBV-positive Apodemus flavicollis out of 83 rodents tested. Two different DOBV lineages were also detected in A. flavicollis as determined from partial sequences of the M and S genomic segments. Sequences of DOBV in A. flavicollis were either identical or closely related to the sequences obtained from the HFRS patients. The DOBV strains circulating in Romania clustered in two monophyletic groups, together with strains from Slovenia and the north of Greece. This is the first evidence for the circulation of DOBV in wild rodents and for a DOBV etiology of HFRS in Romania.

  7. Renal involvement in antiphospholipid syndrome.

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    Pons-Estel, Guillermo J; Cervera, Ricard

    2014-02-01

    Renal involvement can be a serious problem for patients with antiphospholipid syndrome (APS). However, this complication has been poorly recognized and studied. It can be present in patients who have either primary or systemic lupus erythematosus-associated APS. Clinical and laboratory features of renal involvement in APS include hypertension, hematuria, acute renal failure, and progressive chronic renal insufficiency with mild levels of proteinuria that can progress to nephrotic-range proteinuria. The main lesions are renal artery stenosis, venous renal thrombosis, and glomerular lesions (APS nephropathy) that may be acute (thrombotic microangiopathy) and/or chronic (arteriosclerosis, arterial fibrous intimal hyperplasia, tubular thyroidization, arteriolar occlusions, and focal cortical atrophy). APS can also cause end-stage renal disease and allograft vascular thrombosis. This article reviews the range of renal abnormalities associated with APS, and their diagnosis and treatment options.

  8. Enamel renal syndrome: A rare case report

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    S V Kala Vani

    2012-01-01

    Full Text Available Enamel renal syndrome is a very rare disorder associating amelogenesis imperfecta with nephrocalcinosis. It is known by various synonyms such as amelogenesis imperfecta nephrocalcinosis syndrome, MacGibbon syndrome, Lubinsky syndrome, and Lubinsky-MacGibbon syndrome. It is characterized by enamel agenesis and medullary nephrocalcinosis. This paper describes enamel renal syndrome in a female patient born in a consanguineous family.

  9. Scimitar syndrome with renal agenesis

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    Hasan Kahraman

    2012-01-01

    Full Text Available Partial pulmonary venous connection anomaly is relatively uncommon form of congenital heart diseases. The quite rare combination of this anomaly with hypoplasia of the right lung and dextroposition of the heart is designated as scimitar syndrome. Most cases are presented in infantile period and adult presentation is exceedingly rare. Our patient, a 38-year-old man, was admitted to a doctor with flu-like complaint and because of abnormalities on chest X-ray he was sent to our clinic. He did not have any chronic complaints such as shortness of breath and fatigue. After investigation, scimitar syndrome was diagnosed. Left renal agenesis was determined with abdominal examination. Best of our knowledge in literature we did not detect any case both with Scimitar syndrome and renal agenesis, and we wanted to report the asymptomatic adult Scimitar syndrome case with left renal agenesis.

  10. Hemorrhagic Fever with Renal Syndrome Complicated by Orchitis

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    Edmond Puca; Arben Pilaca; Pellumb Pipero; Silva Bino; Majlinda Kote; Elton Rogozi; Entela Puca; Dhimiter Kraja

    2011-01-01

    Hemorrhagic fever with renal syndrome (HFRS) is a disease caused by viruses of the family Bunyaviridae,genus Hantavirus.HFRS from Dobrava virus (DOBV) is a seldom reported disease in Albania.Clinically HFRS is manifested as mild,moderate,or severe.Therefore,the number of cases of Hantavirus'infection may be underestimated,and should be included in the differential diagnosis of many acute infections,hematologic diseases,acute abdominal diseases and renal diseases complicated by acute renal failure.We report here an atypical presentation of HFRS from Dobrava virus complicated by orchitis with a positive outcome.

  11. Study on the Cytotoxic T Lymphocytes Clone Specific for the Nucleocapsid Protein of Hantaan Virus from Peripheral Blood in Patients with Hemorrhagic Fever with Renal Syndrome

    Institute of Scientific and Technical Information of China (English)

    潘蕾; 白雪帆; 黄长形; 李光玉

    2003-01-01

    In order to elucidate the molecular and immunological mechanisms as well as the pathogenesis of hemorrhagic fever with renal syndrome (HFRS), the CD8+ cytotoxic T lymphecytes (CTL) clone was established directly from peripheral blood mononuclear cells (PBMC) of patients with HFRS. The activities of CTL were detected as usual with EBV-transformed lymphoblastoid cell line (BLCL) as target cells. The results showed that the CTL clone could recognized and killed the targetcells with specificity of nucleocapsid protein of Hantaan virus (HTNVNP) with the cytotoxicity percentages of 50.2%,25.4% and 39.0% respectively. These results demonstrated that the antigenic epitopes of HTNVNP mainly located on the C-temainal of the viral nucleocapsid protein.

  12. Renal involvement in antiphospholipid syndrome.

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    Sciascia, Savino; Cuadrado, Maria José; Khamashta, Munther; Roccatello, Dario

    2014-05-01

    Antiphospholipid syndrome (APS) is an autoimmune disease defined by the presence of arterial or venous thrombotic events and/or pregnancy morbidity in patients who test positive for antiphospholipid antibodies (aPLs). APS can be isolated (known as primary APS) or associated with other autoimmune diseases, such as systemic lupus erythematosus (SLE; known as secondary APS). The kidney is a major target organ in APS and renal thrombosis can occur at any level within the vasculature of the kidney (renal arteries, intrarenal arteries, glomerular capillaries and renal veins); events reflect the site and size of the involved vessels. Histological findings vary widely, including ischaemic glomeruli and thrombotic lesions without glomerular or arterial immune deposits on immunofluorescence. Renal prognosis is affected by the presence of aPLs in patients with lupus nephritis and can be poor. In patients with SLE and aPLs, biopsy should be performed because inflammatory and thrombotic lesions require different therapeutic approaches. Renal involvement in patients with definite APS is treated by anticoagulation with long-term warfarin. The range of renal manifestations associated with APS is broadening and, therefore, aPLs have increasing relevance in end-stage renal disease, transplantation and pregnancy.

  13. Interferon-Induced Transmembrane Protein 3 Inhibits Hantaan Virus Infection, and Its Single Nucleotide Polymorphism rs12252 Influences the Severity of Hemorrhagic Fever with Renal Syndrome

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    Xu-yang, Zheng; Pei-yu, Bian; Chuan-tao, Ye; Wei, Ye; Hong-wei, Ma; Kang, Tang; Chun-mei, Zhang; Ying-feng, Lei; Xin, Wei; Ping-zhong, Wang; Chang-xing, Huang; Xue-fan, Bai; Ying, Zhang; Zhan-sheng, Jia

    2017-01-01

    Hantaan virus (HTNV) causes hemorrhagic fever with renal syndrome (HFRS). Previous studies have identified interferon-induced transmembrane proteins (IFITMs) as an interferon-stimulated gene family. However, the role of IFITMs in HTNV infection is unclear. In this study, we observed that IFITM3 single nucleotide polymorphisms (SNP) rs12252 C allele and CC genotype associated with the disease severity and HTNV load in the plasma of HFRS patients. In vitro experiments showed that the truncated protein produced by the rs12252 C allele exhibited an impaired anti-HTNV activity. We also proved that IFITM3 was able to inhibit HTNV infection in both HUVEC and A549 cells by overexpression and RNAi assays, likely via a mechanism of inhibiting virus entry demonstrated by binding and entry assay. Localization of IFITM3 in late endosomes was also observed. In addition, we demonstrated that the transcription of IFITM3 is negatively regulated by an lncRNA negative regulator of interferon response (NRIR). Taken together, we conclude that IFITM3, negatively regulated by NRIR, inhibits HTNV infection, and its SNP rs12252 correlates with the plasma HTNV load and the disease severity of patients with HFRS. PMID:28096800

  14. RENAL FUNCTION DERANGEMENT IN HELLP SYNDROME

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    Anusha Harihara Gopalan

    2017-04-01

    Full Text Available BACKGROUND HELLP is the acronym for haemolysis, elevated liver enzymes and low platelet count. HELLP syndrome is a form of severe preeclampsia with an incidence of 0.2-0.6% of all pregnancies. The aim of our study is to evaluate the trend of renal dysfunction in HELLP syndrome patients. MATERIALS AND METHODS This is a cross-sectional observational study conducted in Department of Obstetrics and Gynaecology, Institute of Maternal and Child Health, Government Medical College, Kozhikode, during the period of 12 months from January 2013 to December 2013. All patients with HELLP syndrome were included in the study and their renal function tests monitored. RESULTS Results were renal dysfunction prevalence in HELLP syndrome patients is 77%. The renal impairment recovered by 6 days on an average. All had normal renal function tests at the time of discharge. Patients with renal function derangement had a mean duration of hospital stay and transfusion requirements more than the patients without renal function derangement, which was statistically significant (p value 0.036. Haemodialysis required for one out of 60 patients. One maternal mortality due to acute renal failure. CONCLUSION As the prevalence of renal dysfunction is high, some amount of renal impairment maybe a part of the HELLP syndrome disease spectrum itself. Though the incidence of progression to acute renal failure and dialysis is less, this is the important cause of morbidity and mortality in HELLP syndrome patients.

  15. Hemorrhagic Fever with Renal Syndrome: Pathogenesis and Clinical Picture

    OpenAIRE

    2016-01-01

    Hantaan virus (HTNV) causes hemorrhagic fever with renal syndrome (HFRS), which is a zoonosis endemic in eastern Asia, especially in China. The reservoir host of HTNV is field mouse (Apodemus agraricus). The main manifestation of HFRS, including acute kidney injury, increases vascular permeability, and coagulation abnormalities. In this paper, we review the current knowledge of the pathogenesis of HFRS including virus factor, immunity factor and host genetic factors. Furthermore, the treatmen...

  16. A novel Sin Nombre virus DNA vaccine and its inclusion in a candidate pan-hantavirus vaccine against hantavirus pulmonary syndrome (HPS) and hemorrhagic fever with renal syndrome (HFRS).

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    Hooper, Jay W; Josleyn, Matthew; Ballantyne, John; Brocato, Rebecca

    2013-09-13

    Sin Nombre virus (SNV; family Bunyaviridae, genus Hantavirus) causes a hemorrhagic fever known as hantavirus pulmonary syndrome (HPS) in North America. There have been approximately 200 fatal cases of HPS in the United States since 1993, predominantly in healthy working-age males (case fatality rate 35%). There are no FDA-approved vaccines or drugs to prevent or treat HPS. Previously, we reported that hantavirus vaccines based on the full-length M gene segment of Andes virus (ANDV) for HPS in South America, and Hantaan virus (HTNV) and Puumala virus (PUUV) for hemorrhagic fever with renal syndrome (HFRS) in Eurasia, all elicited high-titer neutralizing antibodies in animal models. HFRS is more prevalent than HPS (>20,000 cases per year) but less pathogenic (case fatality rate 1-15%). Here, we report the construction and testing of a SNV full-length M gene-based DNA vaccine to prevent HPS. Rabbits vaccinated with the SNV DNA vaccine by muscle electroporation (mEP) developed high titers of neutralizing antibodies. Furthermore, hamsters vaccinated three times with the SNV DNA vaccine using a gene gun were completely protected against SNV infection. This is the first vaccine of any kind that specifically elicits high-titer neutralizing antibodies against SNV. To test the possibility of producing a pan-hantavirus vaccine, rabbits were vaccinated by mEP with an HPS mix (ANDV and SNV plasmids), or HFRS mix (HTNV and PUUV plasmids), or HPS/HFRS mix (all four plasmids). The HPS mix and HFRS mix elicited neutralizing antibodies predominantly against ANDV/SNV and HTNV/PUUV, respectively. Furthermore, the HPS/HFRS mix elicited neutralizing antibodies against all four viruses. These findings demonstrate a pan-hantavirus vaccine using a mixed-plasmid DNA vaccine approach is feasible and warrants further development. Copyright © 2013 The Authors. Published by Elsevier Ltd.. All rights reserved.

  17. A novel Sin Nombre virus DNA vaccine and its inclusion in a candidate pan-hantavirus vaccine against hantavirus pulmonary syndrome (HPS) and hemorrhagic fever with renal syndrome (HFRS)☆

    Science.gov (United States)

    Hooper, Jay W.; Josleyn, Matthew; Ballantyne, John; Brocato, Rebecca

    2014-01-01

    Sin Nombre virus (SNV; family Bunyaviridae, genus Hantavirus) causes a hemorrhagic fever known as hantavirus pulmonary syndrome (HPS) in North America. There have been approximately 200 fatal cases of HPS in the United States since 1993, predominantly in healthy working-age males (case fatality rate 35%). There are no FDA-approved vaccines or drugs to prevent or treat HPS. Previously, we reported that hantavirus vaccines based on the full-length M gene segment of Andes virus (ANDV) for HPS in South America, and Hantaan virus (HTNV) and Puumala virus (PUUV) for hemorrhagic fever with renal syndrome (HFRS) in Eurasia, all elicited high-titer neutralizing antibodies in animal models. HFRS is more prevalent than HPS (>20,000 cases per year) but less pathogenic (case fatality rate 1–15%). Here, we report the construction and testing of a SNV full-length M gene-based DNA vaccine to prevent HPS. Rabbits vaccinated with the SNV DNA vaccine by muscle electroporation (mEP) developed high titers of neutralizing antibodies. Furthermore, hamsters vaccinated three times with the SNV DNA vaccine using a gene gun were completely protected against SNV infection. This is the first vaccine of any kind that specifically elicits high-titer neutralizing antibodies against SNV. To test the possibility of producing a pan-hantavirus vaccine, rabbits were vaccinated by mEP with an HPS mix (ANDV and SNV plasmids), or HFRS mix (HTNV and PUUV plasmids), or HPS/HFRS mix (all four plasmids). The HPS mix and HFRS mix elicited neutralizing antibodies predominantly against ANDV/SNV and HTNV/PUUV, respectively. Furthermore, the HPS/HFRS mix elicited neutralizing antibodies against all four viruses. These findings demonstrate a pan-hantavirus vaccine using a mixed-plasmid DNA vaccine approach is feasible and warrants further development. PMID:23892100

  18. Hemorrhagic Fever with renal syndrome and its history in Iran.

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    Ardalan, Mohammadreza; Chinikar, Sadegh; Mohajel Shoja, Mohammadali

    2014-11-01

    Hemorrhagic fever with renal syndrome (HFRS) is a serious human disease of zoonotic viral origin. A group of different viruses that belong to the family of hemorrhagic fever could represent with HFRS. The basic pathophysiologic feature is virus-induced leaky microcirculation. There is no effective antiviral treatment against them. Because of rapid environmental changes, global warming, and increased global traveling, different hemorrhagic fever syndromes could be found anywhere in the world and beyond their old endemic borders. This review is a brief overview of HFRS in Iran during the early and mid-twentieth century.

  19. Hemorrhagic fever with renal syndrome and coexisting hantavirus pulmonary syndrome

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    Young Min Hong

    2012-06-01

    Full Text Available Hemorrhagic fever with renal syndrome (HFRS is an acute viral disease with fever, hemorrhage and renal failure caused by hantavirus infection. Hantavirus induces HFRS or hantavirus pulmonary syndrome (HPS. HPS progression to a life-threatening pulmonary disease is found primarily in the USA and very rarely in South Korea. Here, we report a case of HFRS and coexisting HPS.

  20. Genetic detection of Dobrava/Belgrade virus in a Czech patient with Haemorrhagic fever with renal syndrome.

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    Papa, A; Zelená, H; Barnetová, D; Petrousová, L

    2010-08-01

    In the summer of 2008, a 15-year-old boy was hospitalized in a paediatric intensive care unit in the Czech Republic. Laboratory diagnosis of hantavirus infection was established by serological and molecular methods. Sequence and phylogenetic analyses showed that the causative strain was Dobrava/Belgrade virus, which is genetically closer to strains associated with Apodemus flavicollis rodents.

  1. [Hereditary cerebro-oculo-renal syndromes].

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    Sessa, Galina; Hjortshøj, Tina Duelund; Egfjord, Martin

    2014-02-17

    Although many congenital diseases present disturbances of the central nervous system, eyes and renal function, only few of these have a defined genetic basis. The first clinical features of cerebro-oculo-renal diseases usually develop in early childhood and deterioration of kidney function and even end-stage kidney disease may occur in a young age. The syndromes should be considered in patients with retarded growth and development, central nervous system abnormalities, impaired vision or blindness and progressive renal failure.

  2. Renal involvement in primary antiphospholipid syndrome.

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    Marcantoni, Carmelita; Emmanuele, Carmela; Scolari, Francesco

    2016-08-01

    Antiphospholipid syndrome is an autoimmune disorder characterized by recurrent venous or arterial thrombosis and/or pregnancy-related problems associated with persistently elevated levels of antiphospholipid antibodies. The kidney is a major target organ in both primary and secondary antiphospholipid syndrome. This review describes several aspects of the renal involvement in the primary form of the syndrome, in particular the histological pattern of the so-called antiphospholipid syndrome nephropathy (APSN). APSN is a vascular nephropathy characterized by small vessel vaso-occlusive lesions associated with fibrous intimal hyperplasia of interlobular arteries, recanalizing thrombi in arteries and arterioles, and focal atrophy, a constellation of morphological lesions suggestive of primary antiphospholipid syndrome.

  3. Renal Alterations in Feline Immunodeficiency Virus (FIV-Infected Cats: A Natural Model of Lentivirus-Induced Renal Disease Changes

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    Mauro Pistello

    2012-08-01

    Full Text Available Human immunodeficiency virus (HIV is associated with several renal syndromes including acute and chronic renal failures, but the underlying pathogenic mechanisms are unclear. HIV and feline immunodeficiency virus (FIV share numerous biological and pathological features, including renal alterations. We investigated and compared the morphological changes of renal tissue of 51 experimentally and 21 naturally infected cats. Compared to the latter, the experimentally infected cats exhibited some mesangial widening and glomerulonephritis, milder proteinuria, and lower tubular and interstitial alterations. The numbers of giant protein tubular casts and tubular microcysts were also lower. In contrast, diffuse interstitial infiltrates and glomerular and interstitial amyloidosis were detected only in naturally infected cats. Similar alterations are found in HIV infected patients, thus supporting the idea of a causative role of FIV infection in renal disease, and underlining the relevance of the FIV and its natural host as an animal model for investigating lentivirus-associated nephropathy.

  4. Acute renal failure in neuroleptic malignant syndrome.

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    Nishioka, Yoshiaki; Miyazaki, Masanobu; Kubo, Susumu; Ozono, Yoshiyuki; Harada, Takashi; Kohno, Shigeru

    2002-07-01

    We report a patient with neuroleptic malignant syndrome (NMS) who developed acute renal failure and was successfully treated by hemodialysis. A 60-year-old man with a 26-year history of schizophrenia had been treated with thioridazine and sulpiride. He was admitted to our hospital for muscular rigidity and oliguria. After the diagnosis of NMS with acute renal failure was established, thioridazine and sulpiride were discontinued and hemodialysis was instituted. Renal function gradually improved and hemodialysis was discontinued after 17th treatment. We also reviewed 57 cases of NMS with acute renal failure reported in the literature. To our knowledge, 26 years is the longest latency between initiation of neuroleptic drug treatment and onset of NMS. Our review of reported NMS cases with acute renal failure identified those risks for poor prognosis as high level of BUN, age and female gender.

  5. Metabolic Syndrome and Outcomes after Renal Intervention

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    Daynene Vykoukal

    2011-01-01

    Full Text Available Metabolic syndrome significantly increases the risk for cardiovascular disease and chronic kidney disease. The increased risk for cardiovascular diseases can partly be caused by a prothrombotic state that exists because of abdominal obesity. Multiple observational studies have consistently shown that increased body mass index as well as insulin resistance and increased fasting insulin levels is associated with chronic kidney disease, even after adjustment for related disorders. Metabolic syndrome appears to be a risk factor for chronic kidney disease, likely due to the combination of dysglycemia and high blood pressure. Metabolic syndrome is associated with markedly reduced renal clinical benefit and increased progression to hemodialysis following endovascular intervention for atherosclerotic renal artery stenosis. Metabolic syndrome is associated with inferior early outcomes for dialysis access procedures.

  6. Metabolic syndrome and outcomes after renal intervention.

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    Vykoukal, Daynene; Davies, Mark G

    2010-12-27

    Metabolic syndrome significantly increases the risk for cardiovascular disease and chronic kidney disease. The increased risk for cardiovascular diseases can partly be caused by a prothrombotic state that exists because of abdominal obesity. Multiple observational studies have consistently shown that increased body mass index as well as insulin resistance and increased fasting insulin levels is associated with chronic kidney disease, even after adjustment for related disorders. Metabolic syndrome appears to be a risk factor for chronic kidney disease, likely due to the combination of dysglycemia and high blood pressure. Metabolic syndrome is associated with markedly reduced renal clinical benefit and increased progression to hemodialysis following endovascular intervention for atherosclerotic renal artery stenosis. Metabolic syndrome is associated with inferior early outcomes for dialysis access procedures.

  7. Saethre-Chotzen syndrome presenting with incomplete renal Fanconi syndrome.

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    Oktenli, Cagatay; Saglam, Mutlu; Zafer, Emre; Gül, Davut

    2002-10-01

    Here we report on a patient with findings of acrocephaly, craniosynostosis, low frontal hairline, ptosis of eyelids, deviated nasal septum, broad great toes, moderate hallux valgus, bilateral symmetrical complete soft tissue syndactyly of toes 2 and 3, and partial soft tissue syndactyly of toes 4 and 5 consistent with the diagnosis of Saethre-Chotzen syndrome. Additionally, the patient had some unusual findings as part of generalized dysfunction of the renal tubules including hypophosphatemia with renal phosphate wasting, normocalcemic hypercalciuria, hypomagnesemia with renal magnesium wasting, low-molecular-weight proteinuria, decreased serum PTH levels, osteopenia, and nephrolithiasis. In the light of these findings, the diagnosis of incomplete renal Fanconi syndrome was made. In conclusion, on the basis of the present findings, it is difficult to say whether renal tubular dysfunction are somehow connected to the Saethre-Chotzen syndrome or not. Therefore, we consider that this is probably just a coincident. However, further studies may show the connection between renal tubular dysfunction and Saethre-Chotzen syndrome.

  8. Renal abnormalities in patients with Kallmann syndrome.

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    Zenteno, J C; Méndez, J P; Maya-Núñez, G; Ulloa-Aguirre, A; Kofman-Alfaro, S

    1999-03-01

    To report experience in patients with Kallmann syndrome (KS) in whom urography was used to establish the type and frequency of renal anomalies associated with the disorder. Of 19 patients with KS, 15 had the X-linked recessive form of the disease, whereas the remaining four were sporadic. Each patient underwent intravenous pyelography (IVP) using a non-ionic, low osmolarity contrast medium. Of the 19 patients with KS, 10 had kidney abnormalities; four presented with unilateral renal agenesis and six had less severe forms of renal abnormality (renal malrotation in four and bilateral dilatation of the calyces and pelves in two). One of the patients with unilateral renal agenesis carried a deletion in KAL, the gene responsible for the X-linked type of KS. Three of the four patients with renal malrotation had a confirmed X-linked recessive form and one carried a point mutation in KAL. These results suggest that kidney abnormalities are more frequent and diverse in patients with KS than previously reported. They also indicate that defects in the KAL gene may contribute to abnormal renal development. However, a review of the literature revealed no close correlation between KAL mutations and kidney anomalies in the X-linked type of disease. Taken together, these data suggest that KAL mutations are not invariably associated with failure of renal development and that additional factors (epigenetic or local) may compensate for defects in the KAL protein.

  9. Nutcracker Syndrome Complicated by Left Renal Vein Thrombosis

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    Faouzi Mallat

    2013-01-01

    Full Text Available Isolated renal vein thrombosis is a rare entity. We present a patient whose complaint of flank pain led to the diagnosis of a renal vein thrombosis. In this case, abdominal computed tomography angiography was helpful in diagnosing the nutcracker syndrome complicated by the renal vein thrombosis. Anticoagulation was started and three weeks later, CTA showed complete disappearance of the renal vein thrombosis. To treat the Nutcracker syndrome, we proposed left renal vein transposition that the patient consented to.

  10. Nutcracker syndrome complicated by left renal vein thrombosis.

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    Mallat, Faouzi; Hmida, Wissem; Jaidane, Mehdi; Mama, Nadia; Mosbah, Faouzi

    2013-01-01

    Isolated renal vein thrombosis is a rare entity. We present a patient whose complaint of flank pain led to the diagnosis of a renal vein thrombosis. In this case, abdominal computed tomography angiography was helpful in diagnosing the nutcracker syndrome complicated by the renal vein thrombosis. Anticoagulation was started and three weeks later, CTA showed complete disappearance of the renal vein thrombosis. To treat the Nutcracker syndrome, we proposed left renal vein transposition that the patient consented to.

  11. The cardio-renal anemia syndrome

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    Dimković Siniša

    2007-01-01

    Full Text Available Introduction. The problem of anemia in congestive heart failure and chronic kidney disease was thought to be insignificant for a long period of time. Recent investigations pointed out that the problem of anemia should be defined in the context of the cardio-renal anemia syndrome. A positive feedback mechanism indicates that cardio-renal anemia syndrome is due to an interaction between congestive heart failure, chronic renal failure and anemia. The aim of the study was to present the possible pathophysiological mechanisms of this syndrome, epidemiological characteristics and therapeutic results of the former investigations. Results. The results of the retrospective and prospective controlled trails have shown that management of anemia with subcutaneous administration of recombinant human erythropoietin together with intravenous iron infusion for at least 3-6 months lead to: relief of symptoms (improved NYHA functional class; increased left ventricular ejection fraction; reduced cardiovascular morbidity and mortality; reduced number of rehospitalizations; reduced requirements for usual therapeutic agents (especially diuretics; and improved renal function. Conclusion. In patients with heart and kidney disease anemia should be routinely identified and appropriately treated. Subcutaneous recombinant erythropoietin and intravenous iron may significantly improve overall survival and quality of life of these patients. .

  12. Hemorrhagic Fever with Renal Syndrome Associated with Acute Pancreatitis

    Institute of Scientific and Technical Information of China (English)

    Edmond Puca; Arben Pilaca; Pellumb Pipero; Dhimiter Kraja; Entela Y Puca

    2012-01-01

    Hemorrhagic fever with renal syndrome (HFRS) is a systemic infectious disease caused by Hantaviruses and characterized by fevers,bleeding tendencies,gastrointestinal symptoms and renal failure.It encompasses a broad spectrum of clinical presentations,ranging from unapparent or mild illnesses to fulminant hemorrhagic processes.Among the various complications of HFRS,acute pancreatitis is a rare find.In this report,based on clinical data,laboratory and radiologic examination findings,we describe a clinical case,with HFRS from Dobrava virus,associated with acute pancreatitis.The patient was successfully treated by supportive management.Clinicians should be alert to the possibility of HFRS when examining patients with epidemiological data and symptoms of acute pancreatitis.

  13. [Acute renal failure in neuroleptic malignant syndrome].

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    Wagner, G; Rónai, L

    1995-10-01

    The neuroleptic malignant syndrome is characterized by hyperpyrexia, muscle rigidity, extrapyramidal motion disorder, vegetative symptoms and mental disorientation. This group of symptoms develops abruptly and may lead to serious complications. One of these complications is the acute renal failure. Permanent muscle rigidity causes the damage of muscle cells which result in myoglobinaemia, myoglobinuria and elevations in muscle related creatine phosphokinase. The authors report the case of a young man who underwent neuroleptic medication because of hebephrenia. During the medication the following symptoms were developed: extrapyramidal symptoms, restlessness, muscle rigidity, high fever. These symptoms eventually lead to acute renal failure caused by rhabdomyolysis (characteristic urine finding, significant elevations in serum creatine phosphokinase). With regards to the neuroleptic malignant syndrome dantrolenum and bromocriptin treatment were applied with the discontinuation of neuroleptic medication. As a part of the complex therapy a massive volumen-supplementing and alkalizing treatment was used but haemodialysis had also become necessary. During the above mentioned treatment symptoms referring to the neuroleptic malignant syndrome resolved and the acute renal failure was cured. The case report calls attention to a specific group of symptoms and the possibilities for prevention and treatment. The above case report is the first observation in Hungary.

  14. Hemolytic uremic syndrome as a primary manifestation of acute human immunodeficiency virus infection.

    Science.gov (United States)

    Gomes, A M; Ventura, A; Almeida, C; Correia, M; Tavares, V; Mota, M; Seabra, J

    2009-05-01

    Hemolytic uremic syndrome may be associated with human immunodeficiency virus infection but it occurs in advanced stages of human immunodeficiency virus disease. As in other forms of hemolytic uremic syndrome plasmapheresis seems to be the treatment of choice. The authors present an unusual case of hemolytic uremic syndrome associated with acute human immunodeficiency virus infection in a 38 year-old black male. The patient was admitted with fever, asthenia, nausea, diarrhea, and reduced urinary output. He was found to have anemia, thrombocytopenia and severe renal failure. Hemolytic uremic syndrome was diagnosed and he was started on plasmapheresis and hemodialysis. Serological tests were consistent with acute human immunodeficiency virus infection: the enzyme linked immunosorbent assay for human immunodeficiency virus was weakly positive, Western Blot test was negative and human immunodeficiency virus RNA quantification was positive, with > 1,000,000 copies/microl. After 4 daily treatment sessions, patient's clinical condition improved and hemoglobin, platelets, lactic dehydrogenase and renal function normalized.

  15. Role of viruses in renal cell carcinoma

    Directory of Open Access Journals (Sweden)

    Mehdi Salehipoor

    2012-01-01

    Full Text Available To determine whether viral infections are related to renal cell carcinoma (RCC, we studied 49 patients with RCC (29 patients were males with age ranging from 30 to 81 years and a mean of 57.5 years; 20 patients were females with age ranging from 36 to 70 years with a mean of 58.4 years and 16 non-neoplastic kidney patients as controls. Tissues specimens from study patients and controls were examined by nested polymerase chain reaction (PCR to determine the presence of DNA of several viruses including human papilloma virus (HPV, Epstein-Barr virus (EBV, and polyoma viruses (BKV and JCV. Our results revealed that 7 of 49 (14.29% RCC tissue specimens had HPV DNA compared with none of 16 non-cancer control subjects. Regarding the HPV types, all the positive results were high-risk HPV types (type 16 in three and 18 in four patients. The present study suggests that HPV infection, especially high-risk types, is associated with RCC. However, more studies are necessary to demonstrate the molecular oncogenic processes involved in this association.

  16. Hemorrhagic Fever with Renal Syndrome in 4 US Soldiers, South Korea, 2005

    OpenAIRE

    Song, Jin-Won; Moon, Sung-Sil; Gu, Se Hun; Song, Ki-Joon; Baek, Luck Ju; Kim, Heung Chul; Kijek, Todd; O’Guinn, Monica L.; Lee, John S.; Turell, Michael J.; Klein, Terry A.

    2009-01-01

    Four US soldiers acquired hemorrhagic fever with renal syndrome while training near the Demilitarized Zone, South Korea, in 2005. Hantaan virus sequences were amplified by reverse transcription–PCR from patient serum samples and from lung tissues of striped field mice (Apodemus agrarius) captured at training sites. Epidemiologic investigations specified the ecology of possible sites of patient infection.

  17. Tenofovir-related nephrotoxicity in human immunodeficiency virus-infected patients: three cases of renal failure, Fanconi syndrome, and nephrogenic diabetes insipidus.

    Science.gov (United States)

    Karras, Alexandre; Lafaurie, Matthieu; Furco, André; Bourgarit, Anne; Droz, Dominique; Sereni, Daniel; Legendre, Christophe; Martinez, Frank; Molina, Jean-Michel

    2003-04-15

    We report 3 cases of renal toxicity associated with use of the antiviral agent tenofovir. Renal failure, proximal tubular dysfunction, and nephrogenic diabetes insipidus were observed, and, in 2 cases, renal biopsy revealed severe tubular necrosis with characteristic nuclear changes. Patients receiving tenofovir must be monitored closely for early signs of tubulopathy (glycosuria, acidosis, mild increase in the plasma creatinine level, and proteinuria).

  18. Branchio-oto-renal syndrome with obstructive sleep apnoea.

    Science.gov (United States)

    Chavan, Arjun; Shastri, Aravind R T; Ross-Russell, Robert I

    2012-08-13

    Branchio-oto-renal (BOR) syndrome is a rare autosomal dominant disorder characterised by branchial arch anomalies, otological and renal abnormalities. To the best of our knowledge, upper airway obstruction has not been hitherto reported in BOR. The authors report a 19-month-old girl with BOR syndrome with features of severe airway obstruction needing tracheostomy.

  19. A Challenging Twist in Pulmonary Renal Syndrome

    Directory of Open Access Journals (Sweden)

    Rajaie Namas

    2014-01-01

    Full Text Available Case. We report a rare case of hydralazine-induced anti-neutrophil cytoplasmic antibody-associated vasculitis. A 75-year-old African American woman with history of high blood pressure on hydralazine for 3 years presented with acute onset of shortness of breath and hemoptysis. Lab workup revealed a severe normocytic anemia and a serum creatinine of 5.09 mg/dL (baseline 0.9. Bronchoscopy demonstrated active pulmonary hemorrhage. A urine sample revealed red cell casts and a renal biopsy demonstrated pauci-immune, focally necrotizing glomerulonephritis with small crescents consistent with possible anti-neutrophil cytoplasmic antibody-positive renal vasculitis. Serologies showed high-titer MPO-ANCA and high-titer anti-histone antibodies. She was treated with intravenous steroids and subsequently with immunosuppression after cessation of hydralazine. The patient was subsequently discharged from hospital after a rapid clinical improvement. Conclusion. Hydralazine-induced anti-neutrophil cytoplasmic antibody-positive renal vasculitis is a rare adverse effect and can present as a severe vasculitic syndrome with multiple organ involvement. Features of this association include the presence of high titer of anti-myeloperoxidase anti-neutrophil cytoplasmic antibody with multiantigenicity, positive anti-histone antibodies, and the lack of immunoglobulin and complement deposition. Prompt cessation of hydralazine may be sufficient to reverse disease activity but immunosuppression may be needed.

  20. Pulmonary Renal Syndrome After Streptococcal Pharyngitis

    Directory of Open Access Journals (Sweden)

    Gopi Mara-Koosham PhD

    2016-04-01

    Full Text Available Pulmonary renal syndrome is a class of small vessel vasculitides that are characterized by the dual presentation of diffuse alveolar hemorrhage (DAH and glomerulonephritis. Pulmonary renal syndrome has multiple etiologies, but its development has been rarely reported following infection with group A streptococcus. We present the case of a 36-year-old Native American male who was transferred to our facility due to refractory hypoxic respiratory failure. He had been diagnosed with streptococcal pharyngitis 2 weeks prior to admission. Given the presence of hemoptysis, bronchoscopy was performed and was consistent with DAH. Urinalysis demonstrated hematuria and proteinuria, in the setting of elevated creatinine and blood urea nitrogen. Additionally, antistreptolysin O titer was positive. Given the constellation of laboratory findings and history of streptococcal pharyngitis, the patient was diagnosed with PRS secondary to streptococcal infection. High-dose methylprednisolone was initiated with concomitant plasmapheresis. He was extubated successfully after his respiratory status improved and was eventually discharged home after making a full recovery within 2 weeks after admission. This case illustrates the importance of clinically relevant sequelae of streptococcal infection as well as the appropriate treatment of PRS secondary to streptococcal pharyngitis with plasmapheresis and intravenous corticosteroids.

  1. [Treatment of renal vein thrombosis associated with nephrotic syndrome].

    Science.gov (United States)

    Funami, M; Takaba, T; Tanaka, H; Murakami, A; Kadokura, M; Hori, G; Ishii, J

    1988-06-01

    Renal vein thrombosis is a rare entity in which true incidence is unknown. The disease occurs most frequently in patients with nephrotic syndrome, but it also can occur in the presence of other hypercoagulable state. Two cases of renal vein thrombosis with nephrotic syndrome which were treated by thrombectomy are reported here. One patient was successfully treated by renal vein and inferior vena cava thrombectomy before developing severe pulmonary embolism. The other was treated by renal vein thrombectomy by which fatal shock was able to be prevented. In those cases, immediate operation was indicated, primarily to prevent additional, possibly fatal, pulmonary embolism and also to improve perfusion of the kidney. In the hope of salvaging the kidney, thrombectomy may be the treatment of choice for acute renal vein thrombosis, complication of pulmonary embolism and inferior vena cava thrombosis, right renal vein thrombosis without collateral flow and acute renal vein thrombosis with shock.

  2. Effect of Renal Transplantation in Restless Legs Syndrome.

    Science.gov (United States)

    Kahvecioglu, Serdar; Yildiz, Demet; Buyukkoyuncu, Nilufer; Celik, Huseyin; Tufan, Fatih; Kılıç, Ahmet Kasım; Gul, Bulent; Yildiz, Abdulmecid

    2016-02-01

    Restless legs syndrome is a disorder in which patients have irresistible urge to move legs during rest. Restless legs syndrome seems to be common in end-stage renal disease. After a successful renal transplant, symptoms ameliorate with renal function improvement and restless legs syndrome is seen less in this population. Here, we aimed to investigate restless legs syndrome frequency and associated factors in renal transplant patients. In a cross-sectional study with 193 patients (116 hemodialysis patients, 45 transplant patients, and 32 controls), the presence of restless legs syndrome was assessed using the Restless Legs Syndrome Questionnaire. Medical history, demographic, and laboratory data were collected from the patients' medical records. Patients were questioned about the presence of restless legs syndrome using the Restless Legs Syndrome Questionnaire. Patients were evaluated with Beck Depression Scale for depression and Pittsburgh tests for sleep disturbances. While the rate of restless legs syndrome was similar between transplants and controls, it was significantly greater in hemodialysis patients. Hemodialysis patients and controls had similar depression scores that were higher compared with transplant patients. Pittsburgh score was similar in transplant patients and controls and significantly increased in the hemodialysis patients. The rate of insomnia was significantly higher in the hemodialysis patients compared with the other 2 groups. Logistic regression analysis revealed independent correlates of restless legs syndrome as insomnia, Beck depression score, and being on hemodialysis. Linear regression analysis showed that independent correlates of higher Pittsburgh score were higher depression score, higher age, and presence of restless legs syndrome. The prevalence of restless legs syndrome is significantly lower in transplant patients than it is in patients on maintenance dialysis. In renal transplant patients, restless legs syndrome frequency was

  3. Hemorrhagic fever with renal syndrome during pregnancy: Case report

    Directory of Open Access Journals (Sweden)

    Todorović Zoran

    2010-01-01

    Full Text Available Introduction Hemorrhagic fever with renal syndrome (HFWRS is an acute infectious disease with abrupt onset, high fever, renal failure and frequent hemorrhages. HFWRS during the pregnancy has relatively low occurrence, with only few described cases mostly in the region of the former Soviet Union. Although, according to the data from the literature, the disease is less severe during the pregnancy, the eclampsy, stillbirth and fetal infection were also described. Material and methods During the summer period, June-September 2002 in the area of nort-heast Montenegro, and mainly among people whose job was connected with rural areas, the HFRS was observed more frequently. In that area during the stated period , 12 patients underwent treatment whose disease was confirmed by the I I F method at the Torlak Institute of Virology in Belgrade. Among the patients there were two pregnant women aged 23 and 29 (both in their second pregnancies, in the 23rd and 26th gestational week, respectively. The aim of this study is to present 2 case studies and to show the disease impact on fetal development. Results The patients aged 23 and 29 yrs, were previously healthy women in their second pregnancies (23rd and 26th gestational week, respectively. The disease had typical beginning. The predictions made according to the laboratory abnormalities and the level of oliguria were that the disease would have a less severe course. Hemorrhagic syndrome was less prominent, and the laboratory analyses were normalized after 15 to 18 days. The monitoring of pregnancy during the disease period and after recovering showed no fetal suffering. The patients had PV delivery, in 39-th an 40-th, respectively. The newborns blood testing on Hantan viruses was done by ELISA method in Torlak Institute, Belgrade. Only the IgG antibodies (1/ 512, were detected, the IgM antibodies were not found. Two years later the tests were repeated, and showed negative results.

  4. Hyponatremic Hypertensive Syndrome in an Obese Man with Renal Ischemia

    Directory of Open Access Journals (Sweden)

    Saeed Khawer

    2006-01-01

    Full Text Available Renovascular hypertension occasionally manifests as an electrolyte disorder. The combination of hyponatremia and renovascular hypertension is known as hyponatremic-hypertensive syndrome. This syndrome was initially reported in children. Here, we describe a 45 year-old Saudi man who was admitted to the hospital with generalized body weakness and inability to walk. He was confused and was noted to have severe hypertension and very low serum sodium and potassium. The patient was recently started on captopril for blood pressure control, which was discontinued because of deterioration of renal function. Color Doppler renal ultrasound, and magnetic resonance angiography confirmed the diagnosis of renal artery stenosis.

  5. Gastrointestinal and renal abnormalities in cardio-facio-cutaneous syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Herman, Thomas E.; McAlister, William H. [Washington University School of Medicine, St. Louis Children' s Hospital, Mallinckrodt Institute of Radiology, St. Louis, MO (United States)

    2005-02-01

    Cardio-facio-cutaneous syndrome (CFC) is an uncommon autosomal recessive condition recently distinguished from Noonan syndrome but with more marked growth failure and ectodermal dysplasia. Abdominal symptoms are frequently described but anatomic lesions in CFC have rarely been described. We have found significant anatomic abnormalities in CFC patients including antral foveolar hyperplasia, severe constipation with fecal impaction, nephrocalcinosis and renal cysts. (orig.)

  6. Hemorrhagic fever with renal syndrome in Montenegro.

    Science.gov (United States)

    Gledovic, Z B; Jeknic, A S; Grgurevic, A D; Rakocevic, B B; Bozovic, B R; Mugosa, B V

    2008-09-01

    The objective of the study was to analyze the epidemiological features of hemorrhagic fever with renal syndrome (HFRS) in Montenegro. The study included 169 cases of HFRS diagnosed in the period between 1995 and 2005 according to the clinical symptoms and serological confirmation. For the analysis of the demographic characteristics of the cases, as well as of the chronological and topographical features of the disease, a descriptive epidemiological method was employed. The average incidence rate in the observed period was 2.6 per 100,000. In the observed period, 8 people died; the average case fatality rate was 4.8% (range: 0.1-15%). Among the diseased persons, 116 were males and 53 were females; most of the cases were adults. The greatest number of HFRS cases occurred during the summer months. The highest incidence rates were registered in the northeastern, rural part of the country. The most frequent type of hantaviruses in Montenegro were Dobrava-Belgrade and Hantaan, carried by rodent species, i.e., the yellow-neck mouse and the striped-field mouse. It is likely that HFRS in Montenegro will become more common in the near future, unless public health control measures are taken.

  7. Klinefelter's syndrome with renal tubular acidosis: impact on height.

    Science.gov (United States)

    Jebasingh, F; Paul, T V; Spurgeon, R; Abraham, S; Jacob, J J

    2010-02-01

    A 19-year-old Indian man presented with a history of proximal muscle weakness, knock knees and gynaecomastia. On examination he had features of rickets and bilateral small testes. Karyotyping revealed a chromosomal pattern of 47,XXX, confirming the diagnosis of Klinefelter's syndrome. He was also found to have hyperchloraemic metabolic acidosis with hypokalaemia, hypophosphataemia, phosphaturia and glycosuria, which favoured a diagnosis of proximal renal tubular acidosis. Patients with Klinefelter's syndrome typically have a tall stature due to androgen deficiency, resulting in unfused epiphyses and an additional X chromosome. However, this patient had a short stature due to associated proximal renal tubular acidosis. To the best of our knowledge, this is the second case of Klinefelter's syndrome with short stature due to associated renal tubular acidosis reported in the literature. This report highlights the need to consider other causes when patients with Klinefelter's syndrome present with a short stature.

  8. BK polyoma virus infection and renal disease in non-renal solid organ transplantation.

    Science.gov (United States)

    Kuppachi, Sarat; Kaur, Deepkamal; Holanda, Danniele G; Thomas, Christie P

    2016-04-01

    BK virus (BKV) is a non-enveloped DNA virus of the polyomaviridae family that causes an interstitial nephritis in immunosuppressed patients. BKV nephropathy is now a leading cause of chronic kidney disease and early allograft failure following kidney transplantation. It is also known to cause renal disease with a progressive decline in kidney function in non-renal solid organ transplant (NRSOT) recipients, although the disease may not be recognized nor its impact appreciated in this patient population. In this report, we review the existing literature to highlight our current understanding of its incidence in NRSOT populations, the approaches to diagnosis and the potential treatment options.

  9. Pulmonary-Renal Syndrome with a Focus on Anti-GBM Disease

    NARCIS (Netherlands)

    Sanders, Jan-Stephan F.; Rutgers, Abraham; Stegeman, Coen A; Kallenberg, Cees G.M.

    Pulmonary-renal syndrome is a potentially life-threatening combination of pulmonary hemorrhage and acute renal failure. Several pathological entities can cause this syndrome. This review discusses the diagnostic strategy required to initiate appropriate therapy. Rapid serological testing and

  10. Cardio-renal-anemia syndrome: A report of three cases

    Directory of Open Access Journals (Sweden)

    U H Okafor

    2012-01-01

    Full Text Available Patients with chronic kidney diseases (CKDs and cardiovascular diseases (CVDs present with various degree of anemia. Anemia has been associated with poor outcome in patients with CKD and CVD. CVD is the commonest cause of morbidity and mortality in patients with CKD. CKD causes anemia and CVD, and this rapidly deteriorates when anemia is not corrected. This triad of CVD, CKD, and anemia has been termed cardio-renal-anemia syndrome. The objec-tive of this study is to highlight the importance of cardio-renal-anemia syndrome, their relation-ship, and management. Three patients with various stages of CKD who presented with anemia and cardiovascular abnormalities are reported. The patients responded well to various interventional measures, with improvement in their clinical and laboratory parameters. Cardio-renal-anemia syndrome is an entity that should be identified. Early and appropriate intervention leads to better outcome.

  11. Respiratory Syncytial Virus Aggravates Renal Injury through Cytokines and Direct Renal Injury

    Directory of Open Access Journals (Sweden)

    Songhui Zhai

    2016-09-01

    Full Text Available The purpose of this study was to investigate the relationship between renal injury and reinfection that is caused by respiratory syncytial virus (RSV and to analyze the mechanism of renal injury. Rats were repeatedly infected with RSV on days 4, 8, 14, and 28, then sacrificed and examined on day 56 after the primary infection. Renal injury was examined by transmission electron microscopy and histopathology. The F protein of RSV was detected in the renal tissue by indirect immunofluorescence. Proteinuria and urinary glycosaminoglycans (GAGs, serum levels of albumin, urea nitrogen, and creatinine, secretion of cytokines, T lymphocyte population and subsets, and dendritic cell (DC activation state were examined. The results showed that renal injury was more serious in the reinfection group than in the primary infection group. At a higher infection dose, 6×106 PFU, the renal injury was more severe, accompanied by higher levels of proteinuria and urinary GAGs excretion, and lower levels of serum albumin. Podocyte foot effacement was more extensive, and hyperplasia of mesangial cells and proliferation of mesangial matrix were observed. The maturation state of DCs was specific, compared with the primary infection. There was also a decrease in the ratio of CD4+ to CD8+T lymphocytes, due to an increase in the percentage of CD8+T lymphocytes and a decrease in the percentage of CD4+T lymphocytes, and a dramatic increase in the levels of IL-6 and IL-17. In terms of the different reinfection times, the day 14 reinfection group yielded the most serious renal injury and the most significant change in immune function. RSV F protein was still expressed in the glomeruli 56 days after RSV infection. Altogether, these results reveal that RSV infection could aggravate renal injury, which might be due to direct renal injury caused by RSV and the inflammatory lesions caused by the anti-virus response induced by RSV.

  12. Porcine Reproductive and Respiratory Syndrome Virus (PRRSV)

    DEFF Research Database (Denmark)

    Kvisgaard, Lise Kirstine

    This PhD thesis presents the diversity of Porcine Reproductive and Respiratory Syndrome viruses (PRRSV) circulating in the Danish pig population. PRRS is a disease in pigs caused by the PRRS virus resulting in reproductive failures in sows and gilts and respiratory diseases in pigs . Due to genetic...... heterogeneity, PRRSV is divided into two genotypes, Type 1 and Type 2. Type 1 PRRS viruses are further divided into at least 3 subtypes. The virus evolves rapidly and reports of high pathogenic variants of both Type 1 and Type 2 appearing in Europe, North America, and Asia have been reported within recent years...... confirmed that only Type 1 subtype 1 PRRSV is circulating in the Danish pig population. The examination of the Danish PRRS field viruses confirmed that there is a high overall diversity among Type 1 viruses in Europe. The phylogenetic study also indicated the presence of two Danish virus clusters, one...

  13. Renal histology of mucocutaneous lymph node syndrome (Kawasaki disease).

    Science.gov (United States)

    Salcedo, J R; Greenberg, L; Kapur, S

    1988-01-01

    Renal involvement is well described in patients with mucocutaneous lymph node syndrome (MCLNS), or Kawasaki disease and is manifested by mild azotemia, hematuria, pyuria or cylinduria, and more often, proteinuria. Renal morphology during the acute stages of the illness has never been reported. In this paper we describe the renal histopathologic changes in a child with MCLNS. The glomerular histopathologic findings suggest immune complex damage to the kidney as a possible mechanism of nephrotoxicity in MCLNS. Presence of kidney lesions, which speak in favor of the injurious role of immune complexes in MLCNS may be relevant to the understanding of the pathogenesis of the vascular lesions that are characteristic of this disease.

  14. Campylobacter-Associated Hemolytic Uremic Syndrome Associated with Pulmonary-Renal Syndrome.

    Science.gov (United States)

    Bowen, Emily Elizabeth; Hangartner, Robert; Macdougall, Iain

    2016-03-01

    Common causes of pulmonary-renal syndrome include anti-glomerular basement membrane (anti-GBM) disease anti-neutrophil cytoplasmic antibody (ANCA) positive vasculitis, and systemic lupus erythematosus. We describe a case of life-threatening pulmonary hemorrhage associated with Campylobacter hemolytic uremic syndrome (HUS), which we believe is a new disease entity. We hypothesize that the cause of this pulmonary-renal syndrome was an immunological reaction to Campylobacter; and that the initiation of high-dose steroids was responsible for the rapid reversal of the patient's pulmonary and renal impairment. The aim of this article is to raise awareness of this unusual cause of a pulmonary-renal syndrome, guiding physicians to recognize it as a potential complication, and to consider high-dose steroids in managing the condition.

  15. [BK virus infection in a pediatric renal transplant recipient].

    Science.gov (United States)

    Bonaventura, R; Vázquez, A; Exeni, A; Rivero, K; Freire, M C

    2005-01-01

    BK Human Polyomavirus causes an asymptomatic primary infection in children, then establishing latency mainly in the urinary tratt. Viral reactivation can lead to renal pathology in individuals with impaired cellular immune response. This is particularly important in pediatric transplant recipients, who can suffer a primary infection when immunosupressed. We followed up the case of a 5 years old patient who received a renal transplant in October 2003, and presented damaged graft 45 days after the intervention. The patient suffered 3 episodes of renal function failure between October 2003 and June 2004. Blood, urine, renal biopsy and lymphocele liquid samples were analyzed. A differential diagnosis between acute rejection and infectious causes was established by testing for BK, CMV and ADV viruses, and the cytological study of renal tissue. Laboratory findings together with clinical signs suggest the patient was infected by BK virus. As a final consideration, the great importance of differentiating between acute rejection and BK infection is emphasized, since immunosuppressant management is opposite in each case.

  16. Canine renal failure syndrome in three dogs.

    Science.gov (United States)

    Jeong, Won Il; Do, Sun Hee; Jeong, Da Hee; Chung, Jae Yong; Yang, Hai Jie; Yuan, Dong Wei; Hong, Il Hwa; Park, Jin Kyu; Goo, Moon Jung; Jeong, Kyu Shik

    2006-09-01

    Three dead dogs were brought to the College of Veterinary Medicine, Kyungpook National University for study. Clinically, all the dogs showed emaciation, anorexia, depression, hemorrhagic vomiting and diarrhea for 7-10 days before death. All the clinical signs were first noted for about one month after feeding the dogs with commercial diets. At necropsy, all 3 dogs had severe renal damage with the same green-yellowish colored nephroliths in the renal pelvis. They also showed systemic hemorrhage and calcification of several organs, which might have been induced by uremia. Microscopically, necrosis, calcification and calculi were detected in the renal tubules, and especially in the proximal convoluted tubules and collecting ducts of the kidney. These findings were supportive of a mycotoxic effect, and especially on their kidneys. However, the precise cause of the toxic effect in these cases of canine renal failure could not be determined.

  17. Pregnancy in a patient with Goodpasture syndrome and renal transplantation.

    Science.gov (United States)

    Wells, S R; Kuller, J A; Thorp, J M

    1996-02-01

    Patients with Goodpasture syndrome have classically had decreased fertility and associated pregnancy wastage. Renal transplantation can increase the likelihood of successful pregnancy. We describe a patient who carried a pregnancy into the third trimester and had a good neonatal outcome. However, she developed superimposed preeclampsia with subsequent graft rejection.

  18. Renal Artery Embolization of Perirenal Hematoma in Hemorrhagic Fever with Renal Syndrome: A Case Report

    Energy Technology Data Exchange (ETDEWEB)

    Choi, Hee Seok; Lee, Yong Seok; Lim, Ji Hyon; Kim, Kyung Soo; Yoon, Yup [Dongguk University College of Medicine, Goyang (Korea, Republic of); Hwang, Jae Cheol [Ulsan University Hospital, University of Ulsan College of Medicine, Ulsan (Korea, Republic of)

    2007-08-15

    Hemorrhagic fever with renal syndrome (HFRS) is an acute viral disease characterized by fever, hemorrhage and renal failure. Among the various hemorrhagic complications of HFRS, spontaneous rupture of the kidney and perirenal hematoma are very rare findings. We report here on a case of HFRS complicated by massive perirenal hematoma, and this was treated with transcatheter arterial embolization. Hemorrhagic fever with renal syndrome (HFRS) is an acute infectious disease caused by hantavirus. HFRS is clinically characterized by fever, renal failure and hemorrhage in organs such as lung, kidney, spleen and the pituitary gland. Renal medullary hemorrhage is a well-known complication in the kidney, but spontaneous rupture of the kidney and perirenal hematoma in HFRS is rare, and patients showing continuous bleeding and massive perirenal hematoma have often been surgically treated. We report here on a case of HFRS complicated by massive perirenal hematoma, and the patient was treated with transcatheter arterial embolization. In summary, spontaneous rupture of the kidney and perirenal hematoma is a rare complication of HFRS. We report here on a case of HFRS that caused massive perirenal hematoma, and this was treated with superselective renal artery embolization.

  19. Nephrotic Syndrome and Acute Renal Failure Apparently Induced by Sunitinib

    Directory of Open Access Journals (Sweden)

    Ying-Shou Chen

    2009-10-01

    Full Text Available We report a case of nephrotic syndrome and acute renal failure apparently induced by sunitinib. A 67-year-old man with a history of metastatic renal cell carcinoma presented with progressive kidney dysfunction with proteinuria, general edema, and body weight gain of 21 kg after undergoing 3 weeks of sunitinib therapy. The patient had taken no other over-the-counter medications, and all other possible causes of nephrotic syndrome were excluded. The Naranjo Adverse Drug Reaction Probability Scale score for this event was 6, indicating a high probability that the observed presentations were associated with use of the drug. However, despite the discontinuation of sunitinib, his condition deteriorated, and hemodialysis was initiated for respiratory distress. A renal biopsy was performed, which revealed ischemic acute tubular necrosis with minimal change nephropathy. In conclusion, nephrologists and oncologists should be aware that nephrotic syndrome with ischemic acute tubular necrosis is a possible adverse effect of sunitinib. For early diagnosis of this condition and to avoid renal damage, we recommend differential diagnosis of serum creatinine and proteinuria in patients undergoing sunitinib therapy.

  20. Renal fibroblast-like cells in Goodpasture syndrome rats.

    Science.gov (United States)

    Okada, H; Inoue, T; Kanno, Y; Kobayashi, T; Ban, S; Kalluri, R; Suzuki, H

    2001-08-01

    The extent of renal fibrosis is the best predictor for functional outcomes in a variety of progressive renal diseases. Interstitial fibroblast-like cells (FbLCs) are presumably involved in the fibrotic process. However, such FbLCs have never been well characterized in the kidney. We characterized renal FbLCs in the nephritic kidney (in which the number of FbLCs and extracellular matrix accumulation were significantly increased) with regards to their expression of phenotypic and functional markers using day 49 Goodpasture syndrome (GPS) rats. Within the renal cortical interstitium, there were a number of alpha-smooth muscle actin(+) (alpha-SMA(+)) FbLCs, negative for vimentin (VIM) and transforming growth factor-beta 1, and not equipped with well-developed rough endoplasmic reticulum and actin-stress fibers. All of these findings were incompatible with the typical features of granulation tissue alpha-SMA(+) myofibroblasts. On the other hand, FbLCs negative for alpha-SMA and VIM produced alpha1(I) procollagen in the nephritic kidney. A number of FbLC populations reside within the cortical interstitium of the kidney in GPS rats, each of which is likely to have developed independently in response to the local conditions of the nephritic kidney, contributing to renal fibrogenesis. Further studies are needed to clarify the key type of FbLC that orchestrates other members to produce renal fibrosis.

  1. Amelogenesis imperfecta with distal renal tubular acidosis: A novel syndrome?

    Directory of Open Access Journals (Sweden)

    R A Misgar

    2017-01-01

    Full Text Available Amelogenesis imperfecta (AI is a heterogeneous group of inherited dental enamel defects. It has rarely been reported in association with multiorgan syndromes and metabolic disorders. The metabolic disorders that have been reported in association with AI include hypocalciuria, impaired urinary concentrating ability, and Bartter-like syndrome. In literature, only three cases of AI and distal renal tubular acidosis (dRTA have been described: two cases in adults and a solitary case in the pediatric age group. Here, we report a child with AI presenting with dRTA; to the best of our knowledge, our reported case is the only second such case in pediatric age group. Our case highlights the importance of recognizing the possibility of renal abnormalities in patients with AI as it will affect the long-term prognosis.

  2. Meckel-Gruber Syndrome with unilateral renal agenesis.

    Science.gov (United States)

    Uysal, Fatma; Uysal, Ahmet

    2015-04-01

    Meckel-Gruber Syndrome (MKS) is an autosomal recessive disorder, characterized by a combination of central nervous system malformation (occipital encephalocele), post-axial polydactyly, and enlarged polycystic kidney dysplasia. With a recurrence risk of 25% this lethal syndrome can be detected in early screening by ultrasound. However, to the authors' knowledge, association of MKS with unilateral renal agenesis was reported only once until now. Here, we describe a case of 26-year primigravida with 23 weeks pregnancy whose detailed sonographical examination of the fetus revealed large encephalocele through the posterior fontanelle microcephaly, anhydramnios, unilateral left enlarged polycystic kidney and right sided renal agenesis. The pregnancy was wilfully terminated on medical grounds. Risk for subsequent pregnancies was explained to the parents.

  3. Mucormycosis with Orbital Apex Syndrome in a Renal Transplant Recipient

    Directory of Open Access Journals (Sweden)

    Ebru Kursun

    2015-06-01

    Full Text Available Mucormycosis is a rarely encountered invasive fungal infection with high mortality.Solid organ transplantation is one of the risk factors for mucormycosis. Mucormycosis can be classified in six different groups according to the anatomical localization; rhinocerebral, pulmonary, cutaneous, gastrointestinal, disseminated, and other less common involvements. This paper presented a mucormycosis case with rhinoorbitocerebral involvementin a renal transplantation receiver, which manifested with orbital apex syndrome. [Cukurova Med J 2015; 40(2.000: 384-389

  4. Behcet's syndrome and renal involvement: a histological and immunofluorescent study of eleven renal biopsies.

    Science.gov (United States)

    Herreman, G; Beaufils, H; Godeau, P; Cassou, B; Wechsler, B; Boujeau, J; Chomette, G

    1982-01-01

    The finding of focal glomerulonephritis in a patient with Behcet's syndrome led us to perform systematic renal biopsies in ten other patients with the disease. None of the patients had symptoms of renal disease. Proteinuria was found in five, two of whom had associated leukocyturia. By light microscopy mesangial and extramembranous glomerular deposits were observed in eight patients. Arterioles in ten patients showed subendothelial and medial hyaline deposits. A granular pattern of fluorescent staining identified the presence of the third component of complement in these deposits. Circulating immune deposits were sought and found in six out of seven patients. The finding of circulating immune complexes and deposition of complement in glomerular and arteriolar tissues supports an immune complex mediated nephropathy and is consistent with the hypothesis of an immunological pathogenesis in Behcet's syndrome.

  5. An update on renal involvement in hemophagocytic syndrome (macrophage activation syndrome).

    Science.gov (United States)

    Esmaili, Haydarali; Mostafidi, Elmira; Mehramuz, Bahareh; Ardalan, Mohammadreza; Mohajel-Shoja, Mohammadali

    2016-01-01

    Hemophagocytic syndrome (HPS) is mainly characterized by massive infiltration of bone marrow by activated macrophages and often presents with pancytopenia. Thrombotic microangiopathy (TMA) is also present with thrombocytopenia and renal involvement. Both conditions could coexist with each other and complicate the condition. Directory of Open Access Journals (DOAJ), EMBASE, Google Scholar, PubMed, EBSCO, and Web of Science with keywords relevant to; Hemophagocytic syndrome, macrophage activation syndrome, interferon-gamma and thrombotic microangiopathy, have been searched. Viral infection, rheumatologic disease and malignancies are the main underlying causes for secondary HPS. calcineurin inhibitors and viral infections are also the main underlying causes of TMA in transplant recipients. In this review, we discussed a 39-year-old male who presented with pancytopenia and renal allograft dysfunction. With the diagnosis of HPS induced TMA his renal condition and pancytopenia improved after receiving intravenous immunoglobulin (IVIG) and plasmapheresis therapy. HPS is an increasingly recognized disorder in the realm of different medical specialties. Renal involvement complicates the clinical picture of the disease, and this condition even is more complex in renal transplant recipients. We should consider the possibility of HPS in any renal transplant recipient with pancytopenia and allograft dysfunction. The combination of HPS with TMA future increases the complexity of the situation.

  6. [The acute renal and cerebral toxicity of lithium: a cerebro-renal syndrome? A case report].

    Science.gov (United States)

    Prencipe, M; Cicchella, A; Del Giudice, A; Di Giorgio, A; Scarlatella, A; Vergura, M; Aucella, F

    2013-01-01

    This descriptive report describes the case of a 50 year-old woman with bipolar disorder, whose maintenance therapy comprised risperidone, sodium valproato and lithium carbonate without any past occurrence of toxicity. Her past medical history was significant for hypertension, cardiopathy and obesity. She presented with a 1-week history of fever, increasing confusion and slurred speech. At presentation, the patient was somnolent. Laboratory investigations revealed a serum creatinine of 3,6 mg/dl, BUN 45 mg/dl serum lithium 3,0 mEq/L with polyuria defined as more than 3 litres a day. EEG and ECG were abnormal. CT brain scanning and lumbar puncture were negative for brain haemorrage or infection. Lithium toxicity causes impairment of renal concentration and encephalopathy due to lithium recirculation, a mechanism responsible for the so-called cerebro-renal syndrome, where dialysis plays an important role in treatment.The patient was treated with continous veno-venous haemodiafiltration (CVVHDF) over 35 hours with gradual improvement of her general condition and efficacy of renal concentration. Our case highlights a few important points. Lithium nefrotoxicity and neurotoxicity can cause a cerebro-renal syndrome even when serum lithium levels are not particularly raised (2,5-3,5 mEq/L). Haemodialysis is the treatment of choice to reduce the molecular mechanisms of lithium-related changes in urinary concentration and reinstate dopaminergic activity in the brain.

  7. Observation on the Efficiency of the Mongolian Gerbil Kidney Tissue Culture Inactivated Bivalent Vaccine for Hemorrhagic Fever with Renal Syndrome

    Institute of Scientific and Technical Information of China (English)

    董关木; 朱智勇; 安祺; 朱凤才; 刘文雪; 孔艳; 杨立宏; 俞永新

    2004-01-01

    The Z10 and Z37 strains of hemorrhagic fever with renal syndrome (HFRS) virus and the Mongolian gerbil (Merions unguiculatus ) kidney cells were used to prepare the inactivated bivalent vaccine. A phase Ⅱ clinical trial use of this vaccine was made in 750 Chinese volunteers. The results showed that the side reaction rate was 2.5% and the sero-conversion rate of neutralizing antibodies against Hantaan and Seoul viruses in the inoculated volunteers were 87.6% and 96.3% respectively.

  8. INTERMEDIATE SYNDROME: A TYPICAL PATTERN OF PRE-RENAL ACUTE RENAL FAILURE IN THE ELDERLY

    Directory of Open Access Journals (Sweden)

    Greloni G

    2004-01-01

    Full Text Available Acute renal failure is a frequent entity in the elderly. This is due on one hand to the structural and physiological changes of the aged kidney, and on the other hand to the exposure of this population to polypharmacy and their reduced capability to metabolize drugs. In the present report we present a case of a seventy year-old woman who developed acute renal failure secondary to severe dehydration with a clinical and laboratory pattern of intermediate syndrome: laboratory results compatible with parenchymal renal insufficiency (elevated urinary sodium, plasma urea and creatinine, but with a positive response to hydration. The main characteristics of the aged kidney that predispose to the development of an intermediate syndrome are: the vascular dysautonomy and reduced capability of sodium and water reabsorption. The intermediate syndrome is a typical pattern of pre-renal insufficiency in the elderly. RESUMEN: La insuficiencia renal aguda es frecuente en el anciano. Esto se debe por un lado a los cambios estructurales y funcionales propios del riñón senil, y por otro a la gran exposición que esta población tiene a la polifarmacia, y su reducida capacidad para metabolizar los medicamentos. En este reporte presentamos el caso de una mujer de 70 años que desarrolló una insuficiencia renal aguda secundaria a severa deshidratación, mostrando un patrón clínico y de laboratorio propio de un sindrome intermedio: laboratorios compatibles con una insuficiencia renal parenquimatosa (sodio urinario, uremia y creatininemia elevadas, pero con una respuesta favorable a la hidratación. Las principales características del riñón senil que predisponen al desarrollo del sindrome intermedio: son la disautonomía vascular y la reducida capacidad en la recuperación de sodio y agua El sindrome intermedio es un patrón típico de insuficiencia prerrenal en el anciano.

  9. Altered renal distal tubule structure and renal Na(+) and Ca(2+) handling in a mouse model for Gitelman's syndrome.

    NARCIS (Netherlands)

    Loffing, J.; Vallon, V.; Loffing-Cueni, D.; Aregger, F.; Richter, K.H.; Pietri, L.; Bloch-Faure, M.; Hoenderop, J.G.J.; Shull, G.E.; Meneton, P.; Kaissling, B.

    2004-01-01

    Gitelman's syndrome, an autosomal recessive renal tubulopathy caused by loss-of-function mutations in the thiazide-sensitive NaCl co-transporter (NCC) of the distal convoluted tubule (DCT), is characterized by mild renal Na(+) wasting, hypocalciuria, hypomagnesemia, and hypokalemic alkalosis. For ga

  10. Novel EYA1 variants causing Branchio-oto-renal syndrome.

    Science.gov (United States)

    Klingbeil, Kyle D; Greenland, Christopher M; Arslan, Selcuk; Llamos Paneque, Arianne; Gurkan, Hakan; Demir Ulusal, Selma; Maroofian, Reza; Carrera-Gonzalez, Andrea; Montufar-Armendariz, Stefany; Paredes, Rosario; Elcioglu, Nursel; Menendez, Ibis; Behnam, Mahdiyeh; Foster, Joseph; Guo, Shengru; Escarfuller, Sebastian; Cengiz, Filiz Basak; Duman, Duygu; Bademci, Guney; Tekin, Mustafa

    2017-07-01

    Branchio-oto-renal (BOR) syndrome is an autosomal dominant genetic disorder characterized by second branchial arch anomalies, hearing impairment, and renal malformations. Pathogenic mutations have been discovered in several genes such as EYA1, SIX5, and SIX1. However, nearly half of those affected reveal no pathogenic variant by traditional genetic testing. Whole Exome sequencing and/or Sanger sequencing performed in 10 unrelated families from Turkey, Iran, Ecuador, and USA with BOR syndrome in this study. We identified causative DNA variants in six families including novel c.525delT, c.979T > C, and c.1768delG and a previously reported c.1779A > T variants in EYA1. Two large heterozygous deletions involving EYA1 were detected in additional two families. Whole exome sequencing did not reveal a causative variant in the remaining four families. A variety of DNA changes including large deletions underlie BOR syndrome in different populations, which can be detected with comprehensive genetic testing. Copyright © 2017 Elsevier B.V. All rights reserved.

  11. Living donor renal transplantation in patients with antiphospholipid syndrome

    Science.gov (United States)

    Choi, Ji Yoon; Jung, Joo Hee; Shin, Sung; Kim, Young Hoon; Han, Duck Jong

    2016-01-01

    Abstract Introduction: Antiphospholipid syndrome (APS), autoantibodies directed against phospholipid-binding proteins are associated with cause vascular thrombosis. Patients with APS requiring renal transplantation are at risk of early graft loss due to arterial or venous thrombosis, or thrombotic microangiopathy (TMA). Here, we report 3 cases of successful renal transplantation in patients with APS. Clinical Findings: A 53-year-old man with end-stage renal disease (ESRD) had experienced bilateral deep venous thrombosis (DVT) in the lower extremities 16 years ago and was administered warfarin. However, he frequently experienced recurrent DVT despite of anticoagulation therapy. Before the surgery, APS was confirmed based on positive results lupus anticoagulant in serological tests. A 40-year-old man with polycystic kidney disease and a history recurrent DVT tested positive for lupus anticoagulant and anticardiolipin antibodies. Lastly, a 42-year-old woman with ESRD was diagnosed with APS 7 years ago. She also developed DVT and tested positive for lupus anticoagulant and anti-B2-glycoprotein 1. The anticoagulation protocol was as follows in all cases: Warfarin was stopped 5 days before living donor renal transplantation and intravenous heparin therapy was started. During surgery, bolus heparin injections (3000 U) were administered to prevent arterial or venous thrombosis. Heparin was substituted with warfarin on postoperative day 4. The third patient (42/F) developed clinical rejection indicated by increased serum creatinine levels and donor-specific antibodies (DSA) and received steroid pulse therapy, plasmapheresis, and rituximab. This treatment restored graft function to within the normal range. The latest graft function in all patients was maintained at normal levels in the outpatient clinic. Conclusions: Living donor renal transplantation may be successful in patients with APS following perioperative anticoagulation therapy. However, because of the high risk of

  12. Pulmonary-Renal Syndrome with a Focus on Anti-GBM Disease

    NARCIS (Netherlands)

    Sanders, Jan-Stephan F.; Rutgers, Abraham; Stegeman, Coen A; Kallenberg, Cees G.M.

    2011-01-01

    Pulmonary-renal syndrome is a potentially life-threatening combination of pulmonary hemorrhage and acute renal failure. Several pathological entities can cause this syndrome. This review discusses the diagnostic strategy required to initiate appropriate therapy. Rapid serological testing and appropr

  13. Outcomes of male patients with Alport syndrome undergoing renal replacement therapy

    DEFF Research Database (Denmark)

    Temme, Johanna; Kramer, Anneke; Jager, Kitty J;

    2012-01-01

    Patients with the hereditary disease Alport syndrome commonly require renal replacement therapy (RRT) in the second or third decade of life. This study compared age at onset of RRT, renal allograft, and patient survival in men with Alport syndrome receiving various forms of RRT (peritoneal dialys...

  14. Ectopic germinal center and megalin defect in primary Sjogren syndrome with renal Fanconi syndrome.

    Science.gov (United States)

    Wang, Jing; Wen, Yubing; Zhou, Mengyu; Shi, Xiaoxiao; Jiang, Lanping; Li, Mingxi; Yu, Yang; Li, Xuemei; Li, Xuewang; Zhang, Wen; Lundquist, Andrew L; Chen, Limeng

    2017-06-02

    This study reports the clinical and pathological features of 12 cases of primary Sjogren syndrome (pSS) with renal involvement presenting with proximal tubular dysfunction in a single center, and investigates the possible correlation of ectopic germinal center formation and megalin/cubilin down-expression. Clinical and pathological records were reviewed. Immunohistochemistry was carried out to detect megalin, cubilin, CD21 and IL-17 expression. Patients presented with different degrees of proximal renal tubule lesion and decreased estimated glomerular filtration rate (eGFR). Renal biopsy revealed tubulointerstitial nephritis, with tubular epithelial cell degeneration, tubular atrophy, interstitial inflammation and focal fibrosis. Immunohistochemistry revealed decreased expression of megalin and cubilin, two important multiligand protein receptors on the brush border of proximal tubular epithelial cells. IL-17 secreted by Th17 subtype effector T cells was diffusely detected in the renal proximal tubule, with a negative correlation of IL-17 and megalin expression. In addition, ectopic germinal centers characterized by CD21(+) follicular dendritic cells were present in the renal interstitium. In patients with a decreased eGFR, treatment with 4 weeks of glucocorticoid therapy resulted in an improved eGFR in 75% of patients. We report 12 cases of pSS characterized by Fanconi syndrome. The decreased megalin and cubilin expression may contribute to the proximal tubular reabsorption defect, possibly secondary to Th17 infiltration and formation of ectopic germinal centers.

  15. Atypical Hemolytic Uremic Syndrome Recurrence after Renal Transplantation

    Science.gov (United States)

    Bouatou, Yassine; Bacchi, Véronique Frémeaux; Villard, Jean; Moll, Solange; Martin, Pierre-Yves; Hadaya, Karine

    2015-01-01

    Abstract Risk for atypical hemolytic uremic syndrome (aHUS) recurrence after renal transplantation is low with an isolated membrane cofactor protein mutation (MCP). We report the case of a 32-year-old woman with a MCP who underwent kidney transplantation with a good evolution at 12 months. At 15 and 35 months, 2 episodes of thrombotic microangiopathy (TMA), after a miscarriage and a preeclampsia, were misinterpreted as triggered by tacrolimus. After each episode however serum creatinine returned to baseline. Five years after transplantation, she had a self-limited rhinosinusitis followed 3 weeks later by an oliguric renal failure. Her complement profile was normal. Graft biopsy showed C3 glomerulonephritis with no “humps” on electron microscopy. No significant renal function improvement followed methylprednisolone pulsing. A second biopsy showed severe acute TMA lesions with C3 glomerular deposits. Despite weekly eculizumab for 1 month, dialysis was resumed. A new workup identified the “at-risk” complement factor H haplotype. Thus, aHUS recurrence should be ruled out in aHUS patients considered at low recurrence risk when a TMA is found in graft biopsy. Prompt eculizumab therapy should be considered to avoid graft loss as aHUS recurrence can first present as a C3 glomerulonephritis. PMID:27500215

  16. Renal salt-wasting syndrome in children with intracranial disorders.

    Science.gov (United States)

    Bettinelli, Alberto; Longoni, Laura; Tammaro, Fabiana; Faré, Pietro B; Garzoni, Luca; Bianchetti, Mario G

    2012-05-01

    Hypotonic hyponatremia, a serious and recognized complication of any intracranial disorder, results from extra-cellular fluid volume depletion, inappropriate anti-diuresis or renal salt-wasting. The putative mechanisms by which intracranial disorders might lead to renal salt-wasting are either a disrupted neural input to the kidney or the elaboration of a circulating natriuretic factor. The key to diagnosis of renal salt-wasting lies in the assessment of extra-cellular volume status: the central venous pressure is currently considered the yardstick for measuring fluid volume status in subjects with intracranial disorders and hyponatremia. Approximately 110 cases have been reported so far in subjects ≤18 years of age (male: 63%; female: 37%): intracranial surgery, meningo-encephalitis (most frequently tuberculous) or head injury were the most common underlying disorders. Volume and sodium repletion are the goals of treatment, and this can be performed using some combination of isotonic saline, hypertonic saline, and mineralocorticoids (fludrocortisone). It is worthy of a mention, however, that some authorities contend that cerebral salt wasting syndrome does not exist, since this diagnosis requires evidence of a reduced arterial blood volume, a concept but not a measurable variable.

  17. A case of Klippel-Feil syndrome with renal agenesis

    Directory of Open Access Journals (Sweden)

    Mehmet Karakoç

    2010-05-01

    Full Text Available Klippel-Feil syndrome (KFS is is a rare syndrome that characterized by fusion of at least two congenital vertebrae in the cervical region. The most common characteristics of KFS are short neck, low nuchal hair line and restricted joint mobility of the cervical region.Addititonal features are scoliosis, sipina bifida, cervical rib, Sprengel deformity and facial asymmetry, and renal and cardiac anomalies. In this report a 10 years old girl was admitted with complaints of short stature and abnormal neck curvature. Physical examination of the patient revealed short neck, low nuchal hair line, and Sprengel’s deformity, height hard palate, facial asymmetry and torticollis. Cervical paravertebral muscle spasm was found on the left as (+ / right (+ +. Muscle tone, muscle strength, reflexes and sensory examination were found to be normal. Radiological examination of the C4-5 and C6-7 cervical region revealed the loss of inteverebral disc spaces and block vertebra. Also there was hemivertebra anomaly and aplasia of odontoid process. Abdominal and pelvic ultrasound examination revelaed absence of right kidney. Echocardiographical examination gave no abnormality. The case was presented in order to draw attention to KFS with renal agenesia in the light of current literature.

  18. Osteomalacia complicating renal tubular acidosis in association with Sjogren's syndrome.

    Science.gov (United States)

    El Ati, Zohra; Fatma, Lilia Ben; Boulahya, Ghada; Rais, Lamia; Krid, Madiha; Smaoui, Wided; Maiz, Hedi Ben; Beji, Soumaya; Zouaghi, Karim; Moussa, Fatma Ben

    2014-09-01

    Renal involvement in Sjogren's syndrome (SS) is not uncommon and may precede other complaints. Tubulointerstitial nephritis is the most common renal disease in SS and may lead to renal tubular acidosis (RTA), which in turn may cause osteomalacia. Nevertheless, osteomalacia rarely occurs as the first manifestation of a renal tubule disorder due to SS. We herewith describe a 43-year-old woman who was admitted to our hospital for weakness, lumbago and inability to walk. X-ray of the long bones showed extensive demineralization of the bones. Laboratory investigations revealed chronic kidney disease with serum creatinine of 2.3 mg/dL and creatinine clearance of 40 mL/min, hypokalemia (3.2 mmol/L), hypophosphatemia (0.4 mmol/L), hypocalcemia (2.14 mmol/L) and hyperchloremic metabolic acidosis (chlorine: 114 mmol/L; alkaline reserve: 14 mmol/L). The serum alkaline phosphatase levels were elevated. The serum levels of 25-hydroxyvitamin D and 1,25-dihydroxy vitamin D were low and borderline low, respectively, and the parathyroid hormone level was 70 pg/L. Urinalysis showed inappropriate alkaline urine (urinary PH: 7), glycosuria with normal blood glucose, phosphaturia and uricosuria. These values indicated the presence of both distal and proximal RTA. Our patient reported dryness of the mouth and eyes and Schirmer's test showed xerophthalmia. An accessory salivary gland biopsy showed changes corresponding to stage IV of Chisholm and Masson score. Kidney biopsy showed diffuse and severe tubulo-interstitial nephritis with dense lymphoplasmocyte infiltrates. Sicca syndrome and renal interstitial infiltrates indicated SS as the underlying cause of the RTA and osteomalacia. The patient received alkalinization, vitamin D (Sterogyl ®), calcium supplements and steroids in an initial dose of 1 mg/kg/day, tapered to 10 mg daily. The prognosis was favorable and the serum creatinine level was 1.7 mg/dL, calcium was 2.2 mmol/L and serum phosphate was 0.9 mmol/L.

  19. Computed tomographic findings in a case of renal vein thrombosis with nephrotic syndrome.

    Science.gov (United States)

    Adler, J; Greweldinger, J; Hallac, R; Frier, S

    1981-01-01

    Renal vein thrombosis is a complication of the nephrotic syndrome presumably related to compression of renal veins by edematous parenchyma and a concomitant hypercoagulable state. The diagnosis has been made by demonstrating marked widening of the left renal vein as it crosses horizontally anterior to the aorta on computed tomography. Inferior venacavography confirmed the presence of thrombosis within the vessels. CT is suggested as a method for noninvasive imaging of the renal veins which might eliminate the need for venography.

  20. Renal anomalies in patients with turner syndrome: Is scintigraphy superior to ultrasound?

    Science.gov (United States)

    Hamza, Rasha T; Shalaby, Mennatallah H; Hamed, Laith S; Abdulla, Dunya B A; Elfekky, Sahar M; Sultan, Omar M

    2016-02-01

    Renal anomalies are present in up to 30% of patients with Turner syndrome (TS). Renal ultrasound (U/S) detects anatomical renal anomalies only while renal scintigraphy detects anomalies, detects early renal malfunction, and estimates glomerular filtration rate (GFR). Thus, we aimed to assess frequency of renal abnormalities detected by scintigraphy in comparison to renal U/S in TS patients. Ninety TS patients were subjected to auxological assessment, measurement of serum creatinine; and renal U/S and scintigraphy. Renal U/S detected renal anomalies in 22.22% of patients versus 17.78 % detected by scintigraphy (P = 0.035). Scintigraphy detected renal functional abnormalities in 44.44% of patients in the form of subnormal total GFR, abnormal renogram curve pattern, improper tracer handling and perfusion; and difference in split renal function >10% between both kidneys. Patients with a 45,X karyotype had more renal functional abnormalities (56%) than those with mosaic karyotype (33.33%), P = 0.04. In conclusion, renal scintigraphy is not superior to U/S in detection of renal anomalies but is a reliable method for early detection of renal malfunction in TS patients especially those with 45,X to ensure early management to offer a better quality of life.

  1. Cochlear implantation in branchio-oto-renal syndrome — A surgical challenge

    OpenAIRE

    Kameswaran, Mohan; Kumar, R. S. Anand; Murali, Sathiya; Raghunandhan, S.; Karthikeyan, K.

    2007-01-01

    Branchio-oto-renal syndrome (Melnick-Fraser Syndrome) is a rare Autosomal Dominant disorder characterized by the syndromic association of branchial cysts or fistulae along with external, middle & inner malformations and renal anomalies. Incomplete penetrance and variable expressivity are common with the phenotypic variation ranging from mild to severe forms & consisting of various eye, ear, oral and craniofacial abnormalities. Mutations in the EYA1 gene on chromosomal site 8q13.3 are identifi...

  2. Cochlear implantation in branchio-oto-renal syndrome — A surgical challenge

    OpenAIRE

    Kameswaran, Mohan; Kumar, R. S. Anand; Murali, Sathiya; Raghunandhan, S.; K.KARTHIKEYAN

    2007-01-01

    Branchio-oto-renal syndrome (Melnick-Fraser Syndrome) is a rare Autosomal Dominant disorder characterized by the syndromic association of branchial cysts or fistulae along with external, middle & inner malformations and renal anomalies. Incomplete penetrance and variable expressivity are common with the phenotypic variation ranging from mild to severe forms & consisting of various eye, ear, oral and craniofacial abnormalities. Mutations in the EYA1 gene on chromosomal site 8q13.3 are identifi...

  3. Acute Renal Failure: Unusual Complication of Epstein-Barr Virus-Induced Infectious Mononucleosis

    National Research Council Canada - National Science Library

    Paul S. Lei; Amy Lowichik; Wain Allen; Teri Jo Mauch

    2000-01-01

    A 17-year-old boy with juvenile rheumatoid arthritis presented with jaundice, confusion, hemolytic anemia, thrombocytopenia, and acute renal failure secondary to titer-confirmed acute Epstein-Barr virus (EBV...

  4. Analysis of incidence and related factors of hemorrhagic fever with renal syndrome in Hebei Province, China.

    Directory of Open Access Journals (Sweden)

    Qi Li

    Full Text Available BACKGROUND: Hemorrhagic fever with renal syndrome (HFRS is an important infectious disease in Hebei province, China. The average annual incidence is 3.10/100000. Although the incidence of HFRS is stable with a general decline in trend since 2004, an increase in the trend was observed in 2011. Few studies have been conducted to investigate the underlying risk factors for this disease. METHODS: The epidemiological data of HFRS and the population data, meteorology, and remote sensing aspects of cities in Hebei province from 1999 to 2011 were collected. The epidemiological data included the population density in the region, seasonal data and rat density and rat's virus carriage rate. The data were analyzed by descriptive study, correlation analysis and multivariate linear analysis. RESULTS: There were 26837 cases of HFRS was reported from 1999 to 2011. The infection occurred mainly in males, aged 20∼49 years, who were farmers. Rattus norvegicus was the main host animal. The incidence of HFRS was related to NDVI value, rat density and rat's virus carriage rate by multiple linear regression (F = 25.936, P<0.01. The pseudo- R2value for the model was 0.644. CONCLUSION: The incidence of HFRS was related to NDVI value, rat density and rat's virus carriage rate. Control of these factors should be used to prevent HFRS in Hebei province.

  5. [Rhabdomyolysis with acute renal failure and malignant neuroleptic syndrome].

    Science.gov (United States)

    Kleinknecht, D; Parent, A; Blot, P; Bochereau, G; Lallement, P Y; Pourriat, J L

    1982-01-01

    Seven episodes of rhabdomyolysis with acute renal failure (ARF) have been observed in 6 patients treated with various short-acting tranquilizers and antidepressants. Clinical features usually included severe hyperthermia, diffuse hypertonicity with or without coma, circulatory failure or unstable blood pressure, and often acute respiratory failure. Serum CPK were always elevated. The type of ARF was prerenal failure without oliguria in 5/7 episodes, and acute tubular necrosis in 2/7 episodes, requiring hemodialyses in one patient. Three patients died. In any case, the tranquilizers and antidepressants responsible for this syndrome were stopped, and electrolyte disorders and acidosis were corrected. Associated acute circulatory failure, septicemia and/or acute hepatic failure required prompt therapy, and artificial ventilation was required in 4 instances. The further use of phenothiazines, butyrophenones, sulpiride and their derivatives should be avoided in any patient having developed such an accident, whose pathophysiology is similar to that described in malignant hyperthermia of various origin.

  6. Exosomes: a potential key target in cardio-renal syndrome

    Directory of Open Access Journals (Sweden)

    Laura eGonzalez-Calero

    2014-10-01

    Full Text Available Exosomes have proven roles in regulating immune response, antigen presentation, RNA and protein transfer, and cell–cell (organ–organ interaction/signaling. These microvesicles can be considered a mechanism of non-classical secretion of proteins, and they represent a sub-proteome, thus assisting in the difficult task of biomarker discovery in a biological fluid as urine, plasma or serum. A potential role of exosomes in the cardio-renal syndrome is currently underexplored. Cardiovascular disease (CVD continues to be the leading cause of morbidity and mortality worldwide and, particularly, rates of cardiovascular events and death consistently increase as kidney function worsens. In other words, chronic kidney disease acts as a risk multiplier. Unfortunately, the relationship between markers of cardiovascular risk in kidney pathology often differs from that in the general population. Efforts in the search for novel action mechanisms simultaneously operating in both pathologies are thus of maximum interest.This article focuses to the role of exosomes in cardiovascular and renal diseases, in the search for novel key targets of interaction between heart and kidneys.

  7. Treatment of compartment syndrome of the thigh associated with acute renal failure after the Wenchuan earthquake.

    Science.gov (United States)

    Duan, Xin; Zhang, Kaiwei; Zhong, Gang; Cen, Shiqiang; Huang, Fuguo; Lv, Jingtong; Xiang, Zhou

    2012-04-01

    Compartment syndrome of the thigh is a rare emergency often treated operatively. The purpose of this study was to evaluate the effects of nonoperative treatment for compartment syndrome of the thigh associated with acute renal failure after the 2008 Wenchuan earthquake. Nonoperative treatment, which primarily involves continuous renal replacement therapy, was performed in 6 patients (3 men and 3 women) who presented with compartment syndrome of the thigh associated with acute renal failure. The mean mangled extremity severity score (MESS) and laboratory data regarding renal function were analyzed before and after treatment, and the clinical outcome was evaluated at 17-month follow-up. Laboratory data regarding renal function showed improvements. All 6 patients survived with the affected lower limbs intact after nonoperative treatment. Follow-up revealed active knee range of motion and increased muscle strength, as well as a recovery of sensation. A positive linear correlation was found between MESS and the time required to achieve a reduction in swelling, as well as the time required for the recovery of sensation and knee range of motion (r>0.8; P<.05). Satisfactory clinical outcomes were obtained in patients with compartment syndrome of the thigh associated with acute renal failure.Urine alkalization, electrolyte and water balance, and continuous renal replacement therapy have played an important role in saving lives and extremities. Nonoperative treatment should be considered in the treatment of compartment syndrome of the thigh associated with acute renal failure.

  8. Pulmonary renal syndrome in childhood: a report of twenty-one cases and a review of the literature.

    Science.gov (United States)

    von Vigier, R O; Trummler, S A; Laux-End, R; Sauvain, M J; Truttmann, A C; Bianchetti, M G

    2000-05-01

    In adults, the term specific pulmonary renal syndrome describes disorders with pulmonary and glomerular manifestations and includes Wegener's granulomatosis, Goodpasture disease, and systemic lupus erythematosus. Nonspecific pulmonary renal syndrome refers to either pulmonary disease complicating glomerular disease, or glomerular diseases following pulmonary disease. Since little is known regarding pulmonary renal syndrome in childhood, we reviewed the charts of 21 pediatric patients with pulmonary renal syndromes treated by the Department of Pediatrics, University of Bern between 1991 and 1998; we also reviewed the pediatric literature that deals with specific pulmonary renal syndromes. Specific pulmonary renal syndrome was noted in 3 children with systemic vasculitis (Wegener granulomatosis, N = 2; microscopic polyangiitis, N = 1) and 2 with systemic lupus erythematosus. Nonspecific pulmonary renal syndrome was observed in 12 patients with pulmonary edema (N = 9), pulmonary thromboembolism (N = 2), and pulmonary infection (N = 1) complicating the course of a glomerular disease, and in 4 children with a pulmonary disease followed by a glomerular disease. Review of the literature disclosed 52 cases of specific pulmonary renal syndrome other than systemic lupus erythematosus: Wegener granulomatosis (N = 28), Goodpasture disease (N = 13), and Henoch-Schönlein purpura (N = 11). In addition, hemolytic uremic syndrome complicated pneumococcal pneumonia in 32 cases. We conclude that pulmonary renal syndromes need to be looked for in childhood. Apart from Wegener granulomatosis, Goodpasture disease, and systemic lupus erythematosus, Henoch-Schönlein purpura and hemolytic-uremic syndrome occasionally have both pulmonary and renal features. Copyright 2000 Wiley-Liss, Inc.

  9. BK virus in solid organ transplant recipients: an emerging syndrome.

    Science.gov (United States)

    Mylonakis, E; Goes, N; Rubin, R H; Cosimi, A B; Colvin, R B; Fishman, J A

    2001-11-27

    BK virus is a human polyomavirus associated with a range of clinical presentations from asymptomatic viruria with pyuria to ureteral ulceration with ureteral stenosis in renal transplant patients or hemorrhagic cystitis in bone marrow transplant recipients. Infection of renal allografts has been associated with diminished graft function in some individuals. Fortunately, however, the majority of patients with BK virus infections are asymptomatic. The type, duration, and intensity of immunosuppression are major contributors to susceptibility to the activation of BK virus infection. Histopathology is required for the demonstration of renal parenchymal involvement; urine cytology and viral polymerase chain reaction methods are useful adjunctive diagnostic tools. Current, treatment of immunosuppressed patients with polyomavirus viruria is largely supportive and directed toward minimizing immunosuppression. Improved diagnostic tools and antiviral therapies are needed for polyomavirus infections.

  10. Enamel renal syndrome with associated amelogenesis imperfecta, nephrolithiasis, and hypocitraturia: A case report.

    Science.gov (United States)

    Bhesania, Dhvani; Arora, Ankit; Kapoor, Sonali

    2015-09-01

    Numerous cases of enamel renal syndrome have been previously reported. Various terms, such as enamel renal syndrome, amelogenesis imperfecta and gingival fibromatosis syndrome, and enamel-renal-gingival syndrome, have been used for patients presenting with the dental phenotype characteristic of this condition, nephrocalcinosis or nephrolithiasis, and gingival findings. This report describes a case of amelogenesis imperfecta of the enamel agenesis variety with nephrolithiasis in a 21-year-old male patient who complained of small teeth. The imaging modalities employed were conventional radiography, cone-beam computed tomography, and renal sonography. Such cases are first encountered by dentists, as other organ or metabolic diseases are generally hidden. Hence, cases of amelogenesis imperfecta should be subjected to advanced diagnostic modalities, incorporating both dental and medical criteria, in order to facilitate comprehensive long-term management.

  11. Enamel renal syndrome with associated amelogenesis imperfecta, nephrolithiasis, and hypocitraturia: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Bhesania, Dhvani; Arora, Ankit; Kapoor, Sonali [Dept. of Conservative Dentistry and Endodontics, Manubhai Patel Dental College, Maharaja Krishnakumarsinhji Bhavnagar University, Vadodara (India)

    2015-09-15

    Numerous cases of enamel renal syndrome have been previously reported. Various terms, such as enamel renal syndrome, amelogenesis imperfecta and gingival fibromatosis syndrome, and enamel-renal-gingival syndrome, have been used for patients presenting with the dental phenotype characteristic of this condition, nephrocalcinosis or nephrolithiasis, and gingival findings. This report describes a case of amelogenesis imperfecta of the enamel agenesis variety with nephrolithiasis in a 21-year-old male patient who complained of small teeth. The imaging modalities employed were conventional radiography, cone-beam computed tomography, and renal sonography. Such cases are first encountered by dentists, as other organ or metabolic diseases are generally hidden. Hence, cases of amelogenesis imperfecta should be subjected to advanced diagnostic modalities, incorporating both dental and medical criteria, in order to facilitate comprehensive long-term management.

  12. The cardio-renal-anaemia syndrome predicts survival in peritoneally dialyzed patients

    National Research Council Canada - National Science Library

    Malyszko, Jolanta; Zbroch, Edyta; Malyszko, Jacek; Mysliwiec, Michal; Iaina, Adrian

    2010-01-01

    .... The correction of anaemia was effective in the amelioration of both cardiac and renal failure. We studied the relationship between the severity of CRA syndrome in peritoneally dialyzed patients and their survival probability...

  13. Branchio-oto-renal syndrome: comprehensive review based on nationwide surveillance in Japan.

    Science.gov (United States)

    Morisada, Naoya; Nozu, Kandai; Iijima, Kazumoto

    2014-06-01

    Branchio-oto-renal (BOR) syndrome is an autosomal dominant disorder characterized by branchiogenic malformation, hearing loss and renal anomalies. The prevalence of BOR syndrome is 1/40,000 in Western countries, and nationwide surveillance in 2009-2010 identified approximately 250 BOR patients in Japan. Three causative genes for BOR syndrome have been reported thus far: EYA1, SIX1, and SIX5, but the causative genes for approximately half of all BOR patients remain unknown. This review article discusses the epidemiology, clinical symptoms, genetic background and management of BOR syndrome. © 2014 Japan Pediatric Society.

  14. Tubulointerstitial Nephritis Complicated by Fanconi Syndrome and Renal Tubular Acidosis Associated with three autoimmune diseases

    OpenAIRE

    Io, Kumiko; Obata, Yoko; Nishino, Tomoya; Hirose, Misaki; Yamashita, Hiroshi; Uramatsu, Tadashi; Ichikawa, Tatsuki; Hayashi, Tomayoshi; Kawakami, Atsushi; Taguchi, Takashi; Kohno, Shigeru

    2013-01-01

    A 45-year-old woman experiencing back pain showed signs of metabolic acidosis and electrolyte imbalances. The results of blood and urine tests indicated Fanconi syndrome and renal tubular acidosis. An x-ray showed vertebral fractures, which were thought to responsible for the back pain. In addition, the patient had proteinuria and renal dysfunction; therefore, renal biopsy was performed, and tubulointerstitial nephritis (TIN) was diagnosed. While investigating TIN, primary biliary cirrhosis a...

  15. Branchio-oto-renal syndrome caused by partial EYA1 deletion due to LINE-1 insertion

    DEFF Research Database (Denmark)

    Morisada, Naoya; Rendtorff, Nanna Dahl; Nozu, Kandai

    2010-01-01

    A 7-year-old Japanese girl with conductive deafness and preauricular fistulae developed proteinuria. She had renal insufficiency, and ultrasound revealed bilateral small kidneys. These findings indicated that she had branchio-oto-renal (BOR) syndrome. In the present patient, we identified, by using...

  16. Post-partum bilateral renal cortical necrosis in antiphospholipid syndrome and systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    Venkat Sainaresh Vellanki

    2013-01-01

    Full Text Available In the presence of systemic lupus erythematosus or related autoimmune disorders, antiphospholipid syndrome (APS is termed secondary APS. Pregnancy-related renal failure due to SAPS is rarely reported in the literature. We present the case of a young primgravida woman with bilateral renal cortical necrosis due to secondary APS in late pregnancy.

  17. Renal failure in a patient with postpolio syndrome and a normal creatinine level.

    Science.gov (United States)

    Leming, Melissa K; Breyer, Michael J

    2012-01-01

    Patients with renal failure who are taking trimethoprim have an increased risk of developing hyperkalemia, which can cause muscle weakness. In patients with postpolio syndrome, a normal creatinine level could be abnormally high, renal failure is possible because of lack of creatinine production, and the muscle weakness from resultant hyperkalemia could be more severe because of their underlying condition. This abnormally high creatinine level has been termed from this point relative renal failure. The objective of the study was to review a case in which relative renal failure and hyperkalemia caused muscle weakness that manifested as shortness of breath and confusion with electrocardiographic changes. A dehydrated patient with relative renal failure and postpolio syndrome had taken trimethoprim-sulfamethoxazole that caused symptomatic hyperkalemia. The patient presented with muscle weakness, shortness of breath, and confusion, with her postpolio syndrome compounding the situation and likely making the muscle weakness more severe. A patient on trimethoprim with renal failure is at an increased risk of developing hyperkalemia. Patients with postpolio syndrome could have severe muscle weakness from the hyperkalemia and could have renal failure even with a normal creatinine level. This case report will remind treating physicians to evaluate such patients for hyperkalemia if they present with muscle weakness, especially if the patient has renal failure and is on trimethoprim.

  18. Infección por virus BK en paciente pediátrico trasplantado renal BK virus infection in a pediatric renal transplant recipient

    Directory of Open Access Journals (Sweden)

    R. Bonaventura

    2005-09-01

    Full Text Available El poliomavirus humano BK causa infección primaria asintomática en la niñez, estableciendo latencia principalmente en el tracto urinario. En individuos con alteración en la inmunidad celular se puede producir su reactivación desencadenando patología a nivel renal. Por estas razones es particularmente importante en la población pediátrica trasplantada renal, en la que puede producir la infección primaria cuando el paciente está inmunosuprimido. En nuestro trabajo se realizó el seguimiento de un paciente de 5 años trasplantado renal en octubre de 2003 que 45 días post-trasplante sufrió un deterioro del órgano injertado. Desde la fecha del trasplante hasta junio de 2004 se produjeron 3 episodios de alteración en la función renal, durante los cuales se analizaron muestras de sangre, orina, biopsia renal y líquido de linfocele. Para el diagnóstico difererencial entre rechazo agudo versus causa infecciosa se emplearon técnicas de detección para los virus BK, CMV y ADV, además del estudio citológico del tejido renal. Los resultados obtenidos junto con la clínica del paciente indican un probable caso de infección por BK. La importancia de realizar el diagnóstico diferencial entre rechazo agudo y la infección por BK radica en que la conducta en cuanto a la terapia inmunosupresora es opuesta en cada caso.BK Human Polyomavirus causes an asymptomatic primary infection in children, then establishing latency mainly in the urinary tract. Viral reactivation can lead to renal pathology in individuals with impaired cellular immune response. This is particularly important in pediatric transplant recipients, who can suffer a primary infection when immunosupressed. We followed up the case of a 5 years old patient who received a renal transplant in October 2003, and presented damaged graft 45 days after the intervention. The patient suffered 3 episodes of renal function failure between October 2003 and June 2004. Blood, urine, renal biopsy

  19. Prenatal diagnosis and follow-up of a case of branchio-oto-renal syndrome displays renal growth impairment after the second trimester.

    Science.gov (United States)

    Bertucci, Emma; Mazza, Vincenzo; Lugli, Licia; Ferrari, Fabrizio; Stanghellini, Ilaria; Percesepe, Antonio

    2015-11-01

    Branchio-oto-renal syndrome combines branchial arch defects, hearing impairment and renal malformations or hypoplasia. Due to the high phenotypic variability, prenatal diagnosis has a limited prognostic value in mutation-positive cases. We report the first branchio-oto-renal syndrome molecular prenatal diagnosis and ultrasonographic follow-up, showing a normal renal growth until the 24th week of pregnancy, a growth deceleration during the third trimester and a renal volume recovery during the first months of life. © 2015 Japan Society of Obstetrics and Gynecology.

  20. Polyoma (BK) virus associated urothelial carcinoma originating within a renal allograft five years following resolution of polyoma virus nephropathy.

    Science.gov (United States)

    Salvatore, Steven P; Myers-Gurevitch, Patricia M; Chu, Stacy; Robinson, Brian D; Dadhania, Darshana; Seshan, Surya V

    2016-03-01

    A direct role for BK polyomavirus infection in malignant tumors of renal allografts and urinary tract is emerging. Case reports suggest a link between BK virus (BKV) reactivation and development of malignancy in renal allograft recipients. Herein we describe the first case of BKV positive invasive urothelial carcinoma within the renal allograft, presenting with chronic diarrhea and weight loss 5 years following resolution of BK viremia/nephropathy (BKVN). Unique to our case was the remote history of BK viremia/BKVN, rising titer of anti-HLA antibody and presence of renal limited urothelial carcinoma with microinvasion of malignant cells staining positive for SV40 large T antigen (T-Ag). These findings suggest that persistence of subclinical BKV infection within the renal allograft may play a role in the malignant transformation of epithelial cells. Patients with history of BKVN may be at risk for kidney and urinary tract malignancy despite resolution of BK viremia/BKVN.

  1. Genomics and transcriptomics of White spot syndrome virus

    NARCIS (Netherlands)

    Marks, H.

    2005-01-01

    White Spot Syndrome Virus (WSSV) is a large enveloped DNA virus that infects shrimp and other crustaceans. The virions are approximately 275 x 120 nm in size and have an ovoid to bacilliform shape and a tail-like appendage at one end. Sequencing revealed that the circular, double stranded (ds) DNA g

  2. Immune defence White Spot Syndrome Virus infected shrimp, Penaeus monodon

    NARCIS (Netherlands)

    Arts, J.A.J.

    2006-01-01

    White spot syndrome virus (WSSV) is the most important viral pathogen of cultured penaeid shrimp worldwide. Since the initial discovery of the virus inTaiwanin 1992, it has spread to shrimp farming regions in Southeast Asia, the

  3. Genomics and transcriptomics of White spot syndrome virus

    NARCIS (Netherlands)

    Marks, H.

    2005-01-01

    White Spot Syndrome Virus (WSSV) is a large enveloped DNA virus that infects shrimp and other crustaceans. The virions are approximately 275 x 120 nm in size and have an ovoid to bacilliform shape and a tail-like appendage at one end. Sequencing revealed that the circular, double stranded (ds) DNA

  4. Nephritic-nephrotic syndrome as a presentation of BK virus infection

    Directory of Open Access Journals (Sweden)

    Nima Derakhshan

    2011-01-01

    Full Text Available BK virus (BKV is increasingly found as an important cause of allograft nephro-pathy. Nephrotic syndrome is not a usual manifestation of BKV nephropathy. Here, we report a 12-year-old boy, a case of end-stage renal disease due to nephronophthisis, who got the kidney trans-planted from a 16-year-old cadaver, and after 18 months of uneventful transplantation on triple immunosuppressive therapy (Mycophenolate mofetil (MMF, cyclosporin and prednisolone, pre-sented with nephrotic feature (edema, heavy proteinuria, hypoalbuminemia and hyperlipidema. Kidney biopsy was in favor of BKV infection and eventually ended in graft failure.

  5. Preventing acute renal failure is crucial during acute tumor lysis syndrome

    Directory of Open Access Journals (Sweden)

    Darmon Michael

    2007-01-01

    Full Text Available Tumour Lysis syndrome (TLS is characterized by the massive destruction of tumoral cells and the release in the extracellular space of their content. While TLS may occur spontaneously before treatment, it usually develops shortly after the initiation of cytotoxic chemotherapy. These metabolites can overwhelm the homeostatic mechanisms and cause hyperuricemia, hyperkalemia, hyperphosphatemia and hypocalcemia. Moreover, TLS may lead to an acute renal failure (ARF. In addition to the hospital mortality induced by the acute renal failure itself, development of an ARF may preclude optimal cancer treatment. Therefore, prevention of the acute renal failure during acute tumor lysis syndrome is mandatory. The objective of this review is to describe pathophysiological mechanisms leading to acute tumor lysis syndrome, clinical and biological consequences of this syndrome and to provide up-to-date guidelines to ensure prevention and prompt management of this syndrome.

  6. Hemorrhagic Fever with Renal Syndrome in the New, and Hantavirus Pulmonary Syndrome in the Old World: paradi(se)gm lost or regained?

    Science.gov (United States)

    Clement, Jan; Maes, Piet; Van Ranst, Marc

    2014-07-17

    Since the first clinical description in 1994 of the so-called "Hantavirus Pulmonary Syndrome" (HPS) as a "newly recognized disease", hantavirus infections have always been characterized as presenting in two distinct syndromes, the so-called "Hemorrhagic Fever with Renal Syndrome" (HFRS) in the Old World, with the kidney as main target organ, in contrast to HPS in the New World, with the lung as main target organ. However, European literature mentions already since 1934 a mostly milder local HFRS form, aptly named "nephropathia epidemica" (NE), and caused by the prototype European hantavirus species Puumala virus (PUUV). Several NE reports dating from the 1980s and early 1990s described already non-cardiogenic HPS-like lung involvement, prior to any kidney involvement, and increasing evidence is now mounting that a considerable clinical overlap exists between HPS and HFRS. Moreover, growing immunologic insights point to common pathologic mechanisms, leading to capillary hyperpermeability, the cardinal feature of all hantavirus infections, both of the New and Old World. It is now perhaps time to reconsider the paradigm of two "different" syndromes caused by viruses of the same Hantavirus genus in the same Bunyaviridae family, and to agree on a common, more logical disease denomination, such as simply and briefly "Hantavirus fever".

  7. Acute Cholestatic Hepatitis A Virus Infection Presenting with Hemolytic Anemia and Renal Failure: A Case Report

    Science.gov (United States)

    Rochling, Fedja

    2013-01-01

    Hepatitis A virus is the most common acute viral hepatitis worldwide with approximately 1.5 million cases annually. Hepatitis A virus infection in general is self-limited. In rare cases, hepatitis A virus infection may cause renal failure, hemolytic anemia, and/or cholestasis. We report the first case of acute cholestatic hepatitis A virus infection complicated by hemolytic anemia, and renal failure in one patient. A 42-year-old Caucasian male presented with cholestasis, hemolytic anemia and renal failure after consuming street tacos in Central and South America while on a business trip. His protracted course required corticosteroid therapy, multiple sessions of plasma exchange, and numerous units of packed red blood cells. This case demonstrates the importance of vaccination in high-risk adults. A prompt diagnosis of acute hepatitis A virus infection is essential, as uncommon presentations may delay diagnosis leading to permanent morbidity and potentially death in fulminant cases. We also demonstrate the efficacy of treatment of cholestatic hepatitis A virus infection, hemolytic anemia, and renal failure with corticosteroids and plasma exchange. PMID:25431704

  8. Acute Cholestatic Hepatitis A Virus Infection Presenting with Hemolytic Anemia and Renal Failure: A Case Report

    Directory of Open Access Journals (Sweden)

    Robert T. Lapp

    2013-01-01

    Full Text Available Hepatitis A virus is the most common acute viral hepatitis worldwide with approximately 1.5 million cases annually. Hepatitis A virus infection in general is self-limited. In rare cases, hepatitis A virus infection may cause renal failure, hemolytic anemia, and/or cholestasis. We report the first case of acute cholestatic hepatitis A virus infection complicated by hemolytic anemia, and renal failure in one patient. A 42-year-old Caucasian male presented with cholestasis, hemolytic anemia and renal failure after consuming street tacos in Central and South America while on a business trip. His protracted course required corticosteroid therapy, multiple sessions of plasma exchange, and numerous units of packed red blood cells. This case demonstrates the importance of vaccination in high-risk adults. A prompt diagnosis of acute hepatitis A virus infection is essential, as uncommon presentations may delay diagnosis leading to permanent morbidity and potentially death in fulminant cases. We also demonstrate the efficacy of treatment of cholestatic hepatitis A virus infection, hemolytic anemia, and renal failure with corticosteroids and plasma exchange.

  9. Pathophysiology of the cardio-renal syndromes types 1-5: An uptodate.

    Science.gov (United States)

    Di Lullo, L; Bellasi, A; Barbera, V; Russo, D; Russo, L; Di Iorio, B; Cozzolino, M; Ronco, C

    According to the recent definition proposed by the Consensus conference on Acute Dialysis Quality Initiative Group, the term cardio-renal syndrome (CRS) has been used to define different clinical conditions in which heart and kidney dysfunction overlap. Type 1 CRS (acute cardio- renal syndrome) is characterized by acute worsening of cardiac function leading to AKI (5, 6) in the setting of active cardiac disease such as ADHF, while type - 2 CRS occurs in a setting of chronic heart disease. Type 3 CRS is closely link to acute kidney injury (AKI), while type 4 represent cardiovascular involvement in chronic kidney disese (CKD) patients. Type 5 CRS represent cardiac and renal involvement in several diseases such as sepsis, hepato - renal syndrome and immune - mediated diseases. Copyright © 2017 Cardiological Society of India. Published by Elsevier B.V. All rights reserved.

  10. Analyis of changes in the serum concentration of cystatin c, creatinine and renal neutrophil gelatinase-associated lipocalin in patients with hemorrhagic fever with renal syndrome

    Directory of Open Access Journals (Sweden)

    E. M. Mingazova

    2015-01-01

    Full Text Available Introduction. Acute kidney injury is a frequent complication of hemorrhagic fever with renal syndrome. The objective evaluation of аcute kidney injury severity degree is significant in determining the amount of medical actions at hemorrhagic fever with renal syndrome.Objective. Тhe shifts of acute kidney injury biomarkers’ levels (urine neutrophil gelatinase-associated lipocalin, serum cystatin C and serum creatinine at different periods of hemorrhagic fever with renal syndrome were evaluated.Methods. Depending to hemorrhagic fever with renal syndrome severity the patients were divided into groups with severe (n=16 and moderate form of hemorrhagic fever with renal syndrome (n=10; the control group included 10 healthy individuals. The levels of biomarkers were measured by ELISA.Results. Тhe serum concentration of creatinine and cystatin C – markers of glomerular pathology – increased significantly in hemorrhagic fever with renal syndrome, peaking at oligouric period; while changes of cystatin C were more rapid. Urine neutrophil gelatinase-associated lipocalin level – marker of renal tubular damage – increased 30 to 96 times compared to the control group in fever period of hemorrhagic fever with renal syndrome and gradually decreased thereafter.Conclusion. Тhe use of modern biochemical markers of renal pathology (sCystatin C, urine neutrophil gelatinaseassociated lipocalin in hemorrhagic fever with renal syndrome, along with traditional indicators, allows a more differentiated approach to the assessment of renal pathology and gives additional evidence to highlight stage and severity of the disease.

  11. Anatomic and Quantitative Temporal Bone CT for Preoperative Assessment of Branchio-Oto-Renal Syndrome.

    Science.gov (United States)

    Ginat, D T; Ferro, L; Gluth, M B

    2016-12-01

    We describe the temporal bone computed tomography (CT) findings of an unusual case of branchio-oto-renal syndrome with ectopic ossicles that are partially located in the middle cranial fossa. We also describe quantitative temporal bone CT assessment pertaining to cochlear implantation in the setting of anomalous cochlear anatomy associated with this syndrome.

  12. Compartment syndrome, rhabdomyolysis and risk of acute renal failure as complications of the lithotomy position.

    NARCIS (Netherlands)

    Bocca, G.; Moorselaar, R.J.A. van; Feitz, W.F.J.; Staak, F.H.J.M. van der; Monnens, L.A.H.

    2002-01-01

    Compartment syndrome, rhabdomyolysis and the risk of acute renal failure are potential complications of the lithotomy position. A six-year-old girl is described who developed a compartment syndrome with rhabdomyolysis after prolonged surgery in the lithotomy position. This complication occurred thre

  13. Hemorrhagic fever with renal syndrome complicated with pregnancy: a case report.

    Science.gov (United States)

    Kim, Baek-Nam; Choi, Byung-Don

    2006-06-01

    Hantaviruses cause two forms of human disease: hemorrhagic fever with renal syndrome (HFRS) and hantavirus pulmonary syndrome. Hantavirus infection can occur in pregnant women and it can have an influence on the maternal and fetal outcomes, although this is a rare finding even in endemic areas. We describe here a recent case of HFRS complicating pregnancy.

  14. Hemorrhagic Fever with Renal Syndrome Complicated with Pregnancy: A Case Report

    OpenAIRE

    2006-01-01

    Hantaviruses cause two forms of human disease: hemorrhagic fever with renal syndrome (HFRS) and hantavirus pulmonary syndrome. Hantavirus infection can occur in pregnant women and it can have an influence on the maternal and fetal outcomes, although this is a rare finding even in endemic areas. We describe here a recent case of HFRS complicating pregnancy.

  15. Compartment syndrome, rhabdomyolysis and risk of acute renal failure as complications of the lithotomy position.

    NARCIS (Netherlands)

    Bocca, G.; Moorselaar, R.J.A. van; Feitz, W.F.J.; Staak, F.H.J.M. van der; Monnens, L.A.H.

    2002-01-01

    Compartment syndrome, rhabdomyolysis and the risk of acute renal failure are potential complications of the lithotomy position. A six-year-old girl is described who developed a compartment syndrome with rhabdomyolysis after prolonged surgery in the lithotomy position. This complication occurred

  16. Spatial analysis of hemorrhagic fever with renal syndrome in China

    Directory of Open Access Journals (Sweden)

    Yang Hong

    2006-04-01

    Full Text Available Abstract Background Hemorrhagic fever with renal syndrome (HFRS is endemic in many provinces with high incidence in mainland China, although integrated intervention measures including rodent control, environment management and vaccination have been implemented for over ten years. In this study, we conducted a geographic information system (GIS-based spatial analysis on distribution of HFRS cases for the whole country with an objective to inform priority areas for public health planning and resource allocation. Methods Annualized average incidence at a county level was calculated using HFRS cases reported during 1994–1998 in mainland China. GIS-based spatial analyses were conducted to detect spatial autocorrelation and clusters of HFRS incidence at the county level throughout the country. Results Spatial distribution of HFRS cases in mainland China from 1994 to 1998 was mapped at county level in the aspects of crude incidence, excess hazard and spatial smoothed incidence. The spatial distribution of HFRS cases was nonrandom and clustered with a Moran's I = 0.5044 (p = 0.001. Spatial cluster analyses suggested that 26 and 39 areas were at increased risks of HFRS (p Conclusion The application of GIS, together with spatial statistical techniques, provide a means to quantify explicit HFRS risks and to further identify environmental factors responsible for the increasing disease risks. We demonstrate a new perspective of integrating such spatial analysis tools into the epidemiologic study and risk assessment of HFRS.

  17. Recurrence of hemolytic uremic syndrome after renal transplantation.

    Science.gov (United States)

    Seitz, B; Albano, L; Vocila, F; Mzoughi, S; Aoudia, R; Guitard, J; Ribes, D; Vachet-Copponat, H; Mourad, G; Bienaimé, F; Dahan, P; Frémeaux-Bacchi, V; Cassuto, E

    2007-10-01

    Non-Shiga toxin-associated hemolytic uremic syndrome (non-Stx-HUS) is a rare disease. The clinical outcome is often unfavorable: 50% of patients progress to end-stage renal failure. Several mutations in complement regulatory genes predispose to non-Stx-HUS. Transplantation outcomes are poor among patients with either mutation in the genes encoding complement H or I factors, with 80% graft loss due to HUS recurrence. In contrast, patients with mutation in the gene encoding MCP have no disease relapse after transplantation. There are no treatment guidelines for non-Stx-HUS recurrence. Herein we have presented 8 patients with non-Stx-HUS recurrence after transplantation during the last 10 years in the South of France. HUS recurrence, which occurred early after transplantation in all but 1 patient, was treated by plasma exchange (PE) with substitution by fresh frozen plasma (FFP). Three patients still treated with long-term plasma therapy have no recurrence at 15, 19, or 24 months. An international registry would help to define new guidelines.

  18. The syndrome of renal tubular acidosis with nerve deafness.

    Science.gov (United States)

    Donckerwolcke, R A; Van Biervliet, J P; Koorevaar, G; Kuijten, R H; Van Stekelenburg, G J

    1976-01-01

    Two brothers with renal tubular acidosis and nerve deafness are described. Studies of the physiopathological characteristics of the renal acidification defect show that the defect is limited to the distal tubule. Renal tubular acidosis with nerve deafness is a distinct nosologic entity that is determined by an autosomal recessive trait.

  19. Syndrome of inappropriate antidiuretic hormone secretion and cerebral/renal salt wasting syndrome: similarities and differences.

    Science.gov (United States)

    Oh, Ji Young; Shin, Jae Il

    2014-01-01

    Hyponatremia (sodium levels of cerebral edema and brain herniation; therefore, prompt diagnosis and proper treatment is important in preventing morbidity and mortality. Among various causes of hyponatremia, diagnosing syndrome of inappropriate antidiuretic hormone secretion (SIADH) and cerebral/renal salt wasting syndrome (C/RSW) is difficult due to many similarities. SIADH is caused by excess of renal water reabsorption through inappropriate antidiuretic hormone secretion, and fluid restriction is the treatment of choice. On the other hand, C/RSW is caused by natriuresis, which is followed by volume depletion and negative sodium balance and replacement of water and sodium is the mainstay of treatment. Determinating volume status in hyponatremic patients is the key point in differential between SIADH and C/RSW. However, in most situations, differential diagnosis of these two diseases is difficult because they overlap in many clinical and laboratory aspects, especially to assess differences in volume status of these patients. Although distinction between the SIADH and C/RSW is difficult, improvement of hypouricemia and an increased fractional excretion of uric acid after the correction of hyponatremia in SIADH, not in C/RSW, may be one of the helpful points in discriminating the two diseases. In this review, we compare these two diseases regarding the pathophysiologic mechanisms, diagnosis, and therapeutic point of view.

  20. An adolescent with 48,xxyy syndrome with hypergonadotrophic hypogonadism, attention deficit hyperactive disorder and renal malformations

    Directory of Open Access Journals (Sweden)

    Prasad Katulanda

    2012-01-01

    Full Text Available 48,XXYY is a rare sex chromosome aneuploidy affecting 1 in 18,000 to 50,000 male births. They present with developmental delay, hypogonadism, gynecomastia, intention tremors, and a spectrum of neurodevelopmental and psychiatric disorders. At one time this condition was considered a variant of Klinefelter syndrome. In clinically suspected cases, 48,XXYY syndrome can be diagnosed by chromosome culture and karyotyping. This patient presented with hypergonadotrophic hypogonadism, attention deficit hyperactive disorder, and renal malformatons. Klinefelter syndrome was clinically suspected. The karyotype confirmed the diagnosis of 48,XXYY syndrome. This is the first reported case of 48,XXYY syndrome from Sri Lanka.

  1. An adolescent with 48,XXYY syndrome with hypergonadotrophic hypogonadism, attention deficit hyperactive disorder and renal malformations.

    Science.gov (United States)

    Katulanda, Prasad; Rajapakse, J Rasika D K; Kariyawasam, Jayani; Jayasekara, Rohan; Dissanayake, Vajira H W

    2012-09-01

    48,XXYY is a rare sex chromosome aneuploidy affecting 1 in 18,000 to 50,000 male births. They present with developmental delay, hypogonadism, gynecomastia, intention tremors, and a spectrum of neurodevelopmental and psychiatric disorders. At one time this condition was considered a variant of Klinefelter syndrome. In clinically suspected cases, 48,XXYY syndrome can be diagnosed by chromosome culture and karyotyping. This patient presented with hypergonadotrophic hypogonadism, attention deficit hyperactive disorder, and renal malformatons. Klinefelter syndrome was clinically suspected. The karyotype confirmed the diagnosis of 48,XXYY syndrome. This is the first reported case of 48,XXYY syndrome from Sri Lanka.

  2. Smoking and renal function in people living with human immunodeficiency virus

    DEFF Research Database (Denmark)

    Ahlström, Magnus Glindvad; Feldt-Rasmussen, Bo; Legarth, Rebecca

    2015-01-01

    INTRODUCTION: Smoking is a main risk factor for morbidity and mortality in people living with human immunodeficiency virus (PLHIV), but its potential association with renal impairment remains to be established. METHODS: We did a nationwide population-based cohort study in Danish PLHIV to evaluate...... the association between smoking status and 1) overall renal function and risk of chronic kidney disease (CKD), 2) risk of any renal replacement therapy (aRRT), and 3) mortality following aRRT. We calculated estimated creatinine clearance using the Cockcroft-Gault equation (CG-CrCl), and evaluated renal function...... Cohort Study, we identified 1,475 never smokers, 768 previous smokers, and 2,272 current smokers. During study period, we observed no association of smoking status with overall renal function. Previous and current smoking was not associated with increased risk of CKD (adjusted IRR: 1.1, 95% confidence...

  3. A Case of Pulmonary-Renal Syndrome Leading to the Diagnosis of Legionnaires' Disease.

    Science.gov (United States)

    Sabani, Erasmia; Sarafidis, Pantelis A; Lazaridis, Antonios; Kouloukourgiotou, Theodora; Stylianou, Konstantinos; Pantzaki, Afroditi; Papagianni, Aikaterini; Efstratiadis, Georgios

    2016-01-01

    We report a case of a 51-year-old Caucasian man referred at our department due to acute renal failure (ARF) complicating respiratory failure during hospitalization in a regional hospital. The patient was previously started on steroids due to the suspicion of rapidly progressive glomerulonephritis (RPGN) in the context of Goodpasture syndrome. However, clinical and laboratory findings did not support this diagnosis; instead a careful evaluation limited differential diagnosis of the renal insult to acute tubular necrosis or acute interstitial nephritis (AIN) following respiratory infection. With lung function fully improved but renal function not recovering, a renal biopsy revealed AIN, a finding leading to further diagnostic testing and finally to the diagnosis of Legionnaires' disease as a cause of this patient's pulmonary-renal syndrome. The management consisted of progressive tapering of oral steroids associated with full recovery of the patient's renal function. This is a rare case of Legionnaires' disease causing immune-mediated AIN and highlights the possibility of Legionella infection as a cause of pulmonary-renal syndrome.

  4. A Case of Pulmonary-Renal Syndrome Leading to the Diagnosis of Legionnaires’ Disease

    Directory of Open Access Journals (Sweden)

    Erasmia Sabani

    2016-01-01

    Full Text Available We report a case of a 51-year-old Caucasian man referred at our department due to acute renal failure (ARF complicating respiratory failure during hospitalization in a regional hospital. The patient was previously started on steroids due to the suspicion of rapidly progressive glomerulonephritis (RPGN in the context of Goodpasture syndrome. However, clinical and laboratory findings did not support this diagnosis; instead a careful evaluation limited differential diagnosis of the renal insult to acute tubular necrosis or acute interstitial nephritis (AIN following respiratory infection. With lung function fully improved but renal function not recovering, a renal biopsy revealed AIN, a finding leading to further diagnostic testing and finally to the diagnosis of Legionnaires’ disease as a cause of this patient’s pulmonary-renal syndrome. The management consisted of progressive tapering of oral steroids associated with full recovery of the patient’s renal function. This is a rare case of Legionnaires’ disease causing immune-mediated AIN and highlights the possibility of Legionella infection as a cause of pulmonary-renal syndrome.

  5. Medullary nephrocalcinosis, distal renal tubular acidosis and polycythaemia in a patient with nephrotic syndrome

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    Karunarathne Suneth

    2012-07-01

    Full Text Available Abstract Background Medullary nephrocalcinosis and distal renal tubular acidosis are closely associated and each can lead to the other. These clinical entities are rare in patients with nephrotic syndrome and polycythaemia is an unusual finding in such patients. We describe the presence of medullary nephrocalcinosis, distal renal tubular acidosis and polycythaemia in a patient with nephrotic syndrome due to minimal change disease. Proposed mechanisms of polycythaemia in patients with nephrotic syndrome and distal renal tubular acidosis include, increased erythropoietin production and secretion of interleukin 8 which in turn stimulate erythropoiesis. Case presentation A 22 year old Sri Lankan Sinhala male with nephrotic syndrome due to minimal change disease was investigated for incidentally detected polycythaemia. Investigations revealed the presence of renal tubular acidosis type I and medullary nephrocalcinosis. Despite extensive investigation, a definite cause for polycythaemia was not found in this patient. Treatment with potassium and bicarbonate supplementation with potassium citrate led to correction of acidosis thereby avoiding the progression of nephrocalcinosis and harmful effects of chronic acidosis. Conclusion The constellation of clinical and biochemical findings in this patient is unique but the pathogenesis of erythrocytosis is not clearly explained. The proposed mechanisms for erythrocytosis in other patients with proteinuria include increased erythropoietin secretion due to renal hypoxia and increased secretion of interleukin 8 from the kidney. This case illustrates that there may exist hitherto unknown connections between tubular and glomerular dysfunction in patients with nephrotic syndrome.

  6. [The pulmonary-renal syndrome: a diagnostic and therapeutic emergency for the internist and the intensivist].

    Science.gov (United States)

    Hié, M; Costedoat-Chalumeau, N; Saadoun, D; Azoulay, E

    2013-11-01

    The pulmonary-renal syndrome is a rare and life-threatening condition. It is defined as the association of a diffuse alveolar hemorrhage and a rapidly progressive glomerulonephritis. The characteristic histological lesion common to all underlying diseases is a necrotizing and crescentic glomerulonephritis. The pulmonary-renal syndrome is a diagnostic and therapeutic emergency: any delay in its management will lead to death or serious functional damage as pulmonary and renal impairment. ANCA-associated vasculitis and Goodpasture's disease are the main disorders associated to pulmonary-renal syndrome. More rarely systemic lupus, cryoglobulinaemia, Henoch-Schonlein purpura or subacute endocarditis may induce a pulmonary-renal syndrome. Differential diagnosis can sometimes be difficult, highlighting some ambiguity in the definition of the syndrome. Initial treatment usually associates systemic corticosteroid, cyclophosphamide and plasma exchange. The role of biotherapy as first line therapy remains to be determined. Copyright © 2013 Société nationale française de médecine interne (SNFMI). Published by Elsevier SAS. All rights reserved.

  7. Severe Renal Hemorrhage in a Pregnant Woman Complicated with Antiphospholipid Syndrome: A Case Report

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    Shohei Kawaguchi

    2011-01-01

    Full Text Available Antiphospholipid syndrome is a systemic autoimmune disease with thrombotic tendency. Consensus guidelines for pregnancy with antiphospholipid syndrome recommend low-dose aspirin combined with unfractionated or low-molecular-weight heparin because antiphospholipid syndrome causes habitual abortion. We report a 36-year-old pregnant woman diagnosed with antiphospholipid syndrome receiving anticoagulation treatment. The patient developed left abdominal pain and gross hematuria at week 20 of pregnancy. An initial diagnosis of left ureteral calculus was made. Subsequently abdominal-pelvic computed tomography was required for diagnosis because of the appearance of severe contralateral pain. Computed tomography revealed serious renal hemorrhage, and ureteral stent placement and pain control by patient-controlled analgesia were required. After treatment, continuance of pregnancy was possible and vaginal delivery was performed safely. This is the first case report of serious renal hemorrhage in a pregnant woman with antiphospholipid syndrome receiving anticoagulation treatment and is an instructive case for urological and obstetrical practitioners.

  8. STUDY ON AEROSOL TRANSMISSION ROUTES OF HEMORRHAGIC FEVER WITH RENAL SYNDROME (HFRS)

    Institute of Scientific and Technical Information of China (English)

    Fengxiang Che; Lingying Meng; Jianchun Lu; Jinsong Li; Junbao Li; Minxia Liu

    2003-01-01

    The present paper reviews our studies on transmission routes and prophylactic measures of Hemorrhagic Fever with Renal Syndrome (HFRS), including aerosol inhalation, skin injury/mucosa, insect vectors, peroral infection and vertical transmission. The results show that HFRS is transmitted by Hantaan virus via multi-routes. One of them, the aerosol transmission, is perhaps the main route transmitting HFRS from mouse to human beings. The injury and mucosa is the main transmission route from mouse to mouse and also an important agent from mouse to human beings.The peroral infection may occur in very serious pollution of foods. The insect vectors may play an important role in the focus of HFRS and in the transmission of HFRS from mouse to human. The epidemiological significance of vertical transmission is yet uncertain. According to the results, four proposals should be emphasized including killing insects and mice, long lasting and combined prophylactics, intensive studies on prophylactic measures on viral aerosols and the blocking of transmission of HFRS from mouse to human via aerosols.

  9. Developmental Abnormalities, Blood Pressure Variability and Renal Disease In Riley Day Syndrome

    OpenAIRE

    Norcliffe-Kaufmann, Lucy; Axelrod, Felicia B.; Kaufmann, Horacio

    2011-01-01

    Riley Day syndrome, commonly referred to as familial dysautonomia (FD), is a genetic disease with extremely labile blood pressure due to baroreflex deafferenation. Chronic renal disease is very frequent in these patients and was attributed to recurrent arterial hypotension and renal hypoperfusion. Aggressive treatment of hypotension, however, has not reduced its prevalence. We evaluated the frequency of kidney malformations as well as the impact of hypertension, hypotension and blood pressure...

  10. RENOVASCULAR HYPERTENSION DUE TO RENAL ARTERY STENOSIS IN KLIPPEL-FEIL SYNDROME

    OpenAIRE

    Foyaca-Sibat H. MD.; Ibañez-Valdés LdeF. MD,

    2003-01-01

    ABSTRACT We report one patient with Klippel-Feil (KFS) syndrome, other associated anomalies, uncontrolled arterial hypertension, and renal artery stenosis. Because this patient underwent for surgical revascularization with unsuccessful result, all proposed way of treatments are revised, and we have hypothesized that probably for patients with KFS and unilateral renal artery stenosis, medical treatment with ACE inhibitors can provide more benefits than surgical revascularization or percutan...

  11. Experience With Intravenous Ribavirin in the Treatment of Hemorrhagic Fever With Renal Syndrome in Korea

    Science.gov (United States)

    2009-01-01

    baseline pregnancy test was obtained in women. Baseline and daily laboratory tests included complete blood count, serum electrolytes, renal and liver...used to confirmHFRS diagnosis . Sero- logical tests were obtained at baseline and the subsequent 2 days. Tests were performed at the 121st General...J.C.,Moussinga, N., 1992. Bloc auriculo-ventriculaire chez deux patients atteints de fievre hemorragique avec syndrome renal ( Nephropathies a Hantaan

  12. Implications of Von Hippel-Lindau Syndrome and Renal Cell Carcinoma

    Science.gov (United States)

    Ashouri, Kenan; Mohseni, Sophia; Tourtelot, John; Sharma, Pranav

    2015-01-01

    Von Hippel-Lindau syndrome (VHLS) is a rare hereditary neoplastic disorder caused by mutations in the vhl gene leading to the development of tumors in several organs including the central nervous system, pancreas, kidneys, and reproductive organs. Manifestations of VHLS can present at different ages based on the affected organ and subclass of disease. In the subclasses of VHLS that cause renal disease, renal involvement typically begins closer to the end of the second decade of life and can present in different ways ranging from simple cystic lesions to solid tumors. Mutations in vhl are most often associated with clear cell renal carcinoma, the most common type of renal cancer, and also play a major role in sporadic cases of clear cell renal carcinoma. The recurrent, multifocal nature of this disease presents difficult challenges in the long-term management of patients with VHLS. Optimization of renal function warrants the use of several different approaches common to the management of renal carcinoma such as nephron sparing surgery, enucleation, ablation, and targeted therapies. In VHLS, renal lesions of 3 cm or bigger are considered to have metastatic potential and even small lesions often harbor malignancy. Many of the aspects of management revolve around optimizing both oncologic outcome and long-term renal function. As new surgical strategies and targeted therapies develop, the management of this complex disease evolves. This review will discuss the key aspects of the current management of VHLS.

  13. Implications of Von Hippel-Lindau Syndrome and Renal Cell Carcinoma

    Directory of Open Access Journals (Sweden)

    Kenan Ashouri

    2015-09-01

    Full Text Available Von Hippel-Lindau syndrome (VHLS is a rare hereditary neoplastic disorder caused by mutations in the vhl gene leading to the development of tumors in several organs including the central nervous system, pancreas, kidneys, and reproductive organs. Manifestations of VHLS can present at different ages based on the affected organ and subclass of disease. In the subclasses of VHLS that cause renal disease, renal involvement typically begins closer to the end of the second decade of life and can present in different ways ranging from simple cystic lesions to solid tumors. Mutations in vhl are most often associated with clear cell renal carcinoma, the most common type of renal cancer, and also play a major role in sporadic cases of clear cell renal carcinoma. The recurrent, multifocal nature of this disease presents difficult challenges in the long-term management of patients with VHLS. Optimization of renal function warrants the use of several different approaches common to the management of renal carcinoma such as nephron sparing surgery, enucleation, ablation, and targeted therapies. In VHLS, renal lesions of 3 cm or bigger are considered to have metastatic potential and even small lesions often harbor malignancy. Many of the aspects of management revolve around optimizing both oncologic outcome and long-term renal function. As new surgical strategies and targeted therapies develop, the management of this complex disease evolves.  This review will discuss the key aspects of the current management of VHLS.

  14. Spatial analysis of hemorrhagic fever with renal syndrome in Zibo City, China, 2009-2012.

    Directory of Open Access Journals (Sweden)

    Feng Cui

    Full Text Available BACKGROUND: Hemorrhagic fever with renal syndrome (HFRS is highly endemic in mainland China, where human cases account for 90% of the total global cases. Zibo City is one of the most serious affected areas in Shandong Province China with the HFRS incidence increasing sharply from 2009 to 2012. However, the hotspots of HFRS in Zibo remained unclear. Thus, a spatial analysis was conducted with the aim to explore the spatial, spatial-temporal and seasonal patterns of HFRS in Zibo from 2009 to 2012, and to provide guidance for formulating regional prevention and control strategies. METHODS: The study was based on the reported cases of HFRS from the National Notifiable Disease Surveillance System. Annualized incidence maps and seasonal incidence maps were produced to analyze the spatial and seasonal distribution of HFRS in Zibo City. Then spatial scan statistics and space-time scan statistics were conducted to identify clusters of HFRS. RESULTS: There were 200 cases reported in Zibo City during the 4-year study period. One most likely cluster and one secondary cluster for high incidence of HFRS were identified by the space-time analysis. And the most likely cluster was found to exist at Yiyuan County in October to December 2012. The human infections in the fall and winter reflected a seasonal characteristic pattern of Hantaan virus (HTNV transmission. The secondary cluster was detected at the center of Zibo in May to June 2009, presenting a seasonal characteristic of Seoul virus (SEOV transmission. CONCLUSION: To control and prevent HFRS in Zibo city, the comprehensive preventive strategy should be implemented in the southern areas of Zibo in autumn and in the northern areas of Zibo in spring.

  15. Renal manifestations of severe Rabson-Mendenhall syndrome: a case report

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    Chong Yih Harng

    2013-01-01

    Full Text Available Abstract Introduction Rabson-Mendenhall Syndrome (RMS is a rare form of severe insulin resistance due to a recessive mutation of the insulin receptor. Associated manifestations include facial dysmorphism, skin abnormalities, and renal anomalies. Case presentation We report a case of a 13 year old African female with RMS, severe insulin resistance, and a cluster of renal pathologies including nephromegaly, nephrolithiasis, hydronephrosis, and medullary sponge kidney. Conclusion This is the first case of severe insulin resistance associated with the collection of renal conditions described. We postulate that renal conditions present in RMS may be under recognised, and recommend screening for the above conditions. This case adds to the scarce body of literature of associated renal manifestations with RMS, including medullary sponge kidney, across the spectrum of insulin resistance.

  16. [Acute renal failure after dengue virus infection: A pediatric case report].

    Science.gov (United States)

    Nicolon, C; Broustal, E

    2016-01-01

    Dengue is an emerging, rapidly expanding disease, whose clinical and biological manifestations vary. Kidney injury is not usual but can be severe, and it is most often associated with dengue hemorrhagic fever or shock. Guadeloupe, which is located in an endemic area, experienced an epidemic from 2013 to 2014. During this outbreak, a case of renal failure during dengue was observed in a 10-year-old child. No evidence of dengue hemorrhagic fever or shock syndrome was found. The clinical and biological course improved with symptomatic treatment. The association of acute renal failure with hemolytic anemia suggested a diagnosis of hemolytic uremic syndrome. However, this could not be confirmed in the absence of thrombocytopenia and cytopathologic evidence. This case illustrates the diversity of clinical presentations of dengue, and the possibility of severe renal impairment unrelated to the usual factors encountered in dengue.

  17. POEMS syndrome with vascular lesions and renal carcinoma - possible role of cytokines.

    Science.gov (United States)

    Pasqui, A L; Bova, G; Saletti, M; Bruni, F; Di Renzo, M; Auteri, A

    1998-06-17

    We describe here the case of a 60 years man with POEMS syndrome associated with renal tumor and vascular lesions. The patient had osteosclerotic myeloma IgA-lambda, polyneuropathy, endocrinopathy and skin changes. In addition, he developed renal clear cell carcinoma and gangrena of lower limbs. The humoral study showed thrombocytosis, high levels of IL-1beta and IL-6 and of some coagulative/fibrinolytic and endothelial factors (von Willebrand factor, plasmin-antiplasmine complexes, plasminogen activator). We suggest the hypothesis that these factors are capable of determining some manifestations of POEMS syndrome.

  18. [Renal cell carcinoma in patient with situs inversus and Kartagener syndrome].

    Science.gov (United States)

    Jiménez Verdejo, A; Martínez Torres, J L; Palao Yago, F; Tinaut Ranera, J; Arrabal Martín, M; Miján Ortiz, J L; Zuluaga Gómez, A

    2000-02-01

    A case of a adenocarcinoma renal in a patient with situs inversus complete to which was associated bronchiectasis and chronic sinusitis (kartagener's syndrome) is reported. It is the third case described in the international literature of a renal cells carcinoma in a patient with situs inversus totalis and the first in patient the one which has the triada classic of the kartagener's syndrome plus sterility. The tumor was discovered in a way incidental upon accomplishing a TAC toracoabdominal and was solved through nefrectomia for lumbotomy approach.

  19. Primary antiphospholipid syndrome presenting as renal vein thrombosis and membranous nephropathy.

    Science.gov (United States)

    Chaturvedi, Swasti; Brandao, Leonardo; Geary, Denis; Licht, Christoph

    2011-06-01

    Antiphospholipid syndrome is a multisystem auto-immune disorder characterized by thrombotic events and the presence of circulating antiphospholipid antibodies. Large vessel involvement in the form of thrombosis/stenosis and thrombotic microangiopathy is a commonly described renal finding. However, non-thrombotic glomerulopathies are increasingly being recognized in patients with antiphospholipid syndrome. We report a rare occurrence of both renal vein thrombosis and membranous nephropathy in a previously healthy adolescent male. Investigations revealed persistently positive antiphospholipid antibodies in the absence of an underlying systemic autoimmune disorder or malignancy. Our patient responded favourably to anti-proteinuric therapy and anticoagulation with complete resolution of proteinuria and a nearly occlusive thrombus.

  20. New Line of Investigation in Rehabilitation of Patients Suffered from Hemorrhagic Fever with Renal Syndrome

    Directory of Open Access Journals (Sweden)

    G. M. Khasanova

    2011-01-01

    Full Text Available A new educational program for patients suffered from hemorrhagic fever with renal syndrome was worked out and applied into practice for the first time. It was revealed that patient teaching increased patient complianceand improved rehabilitation results for sure. Asthenia symptoms, arterial hypertension and inflammatory processes in uringenital system were recorded more rarely with such patients for certain. The efficiency of this educational program in combined rehabilitation after cases of hemorrhagic fever with renal syndrome proves its advisability of wide application in medical and preventive treatment institutions.

  1. Renal disease in human immunodeficiency virus - Not just HIV-associated nephropathy.

    Science.gov (United States)

    Vali, P S; Ismal, K; Gowrishankar, S; Sahay, M

    2012-03-01

    The aim of the study was to determine the various histopathological lesions in human immunodeficiency virus (HIV) patients with renal dysfunction and to establish clinicopathological correlation. Over a period of two years from January 2008 to March 2010, 27 HIV positive patients with renal dysfunction were subjected to renal biopsy. Of the 27 patients, 23 were males and four were females (85.2% males, 14.8% females). Mean age was 38.2 ± 10.36 (range 20 - 60) years. The probable mode of acquisition of HIV infection was sexual in 22 patients (81.5%). Thirteen patients (48%) had nephrotic proteinuria. The CD4 count ranged from 77 to 633/microliter. The kidneys were of normal size in 19 (70.4%) and bulky in eight (29.6%) patients. Thirteen patients required renal replacement therapy. Eleven patients had acute tubule-interstitial lesions (40.7%) while 15 (55.5%) had glomerular lesions. The various glomerular lesions were, focal segmental glomerulosclerosis in five, amyloidosis in three, diffuse proliferative GN in two, and membranoproliferative glomerulonephritis (GN), membranous GN, minimal change disease, diabetic nephropathy, crescentic GN, and thrombotic microangiopathy were seen in one each. None of the clinical or laboratory variables, except hypertension, was found to predict glomerular versus non-glomerular lesions on biopsy. In conclusion we show that a variety of glomerular and tubulointerstitial lesions can be seen on renal histology. Hence, renal biopsy is indicated in renal dysfunction associated with HIV for making proper diagnosis and therapy.

  2. [Goodpasture Syndrome: a Rare Cause of Acute Anuric Renal Failure].

    Science.gov (United States)

    Bareiss, Dirk; Hopfer, Helmut; Menter, Thomas; Jehle, Andreas; Burkhalter, Felix

    2016-06-08

    We report a case of a 68 year old male who presented with an acute onset of anuric renal failure. Investigations revealed a histologically confirmed “double-positive” anti-GBM disease with initially undetectably high antibody values. An induction therapy with plasma exchange, cyclophosphamide and initially high dose steroids and further maintenance therapy for three months was initiated. The patient remained dialysis-dependent despite partial recovering of renal function. Without pulmonary involvement there were no clues for Goodpasture’s disease. Renal prognosis is unfavourable.

  3. On the vaccination of shrimp against white spot syndrome virus

    NARCIS (Netherlands)

    Witteveldt, J.

    2006-01-01

    More than a decade after its discovery inSouth-East Asia, White Spot Syndrome Virus (WSSV) is still the most important (viral) pathogen in the shrimp culture industry. Despite the shift from culturingPenaeusmonodon

  4. Successful Salvage of a Renal Allograft after Acute Renal Vein Thrombosis due to May-Thurner Syndrome

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    Omkar U. Vaidya

    2012-01-01

    Full Text Available A 68-year-old Caucasian female with a past medical history of a deceased donor kidney transplant four months prior was admitted with a two-day history of anuria and acute kidney injury. A renal ultrasound demonstrated thrombus in the transplanted kidney's renal vein that extended into the left iliac vein as well as into the left femoral venous system. Catheter-guided tissue thrombolytics were infused directly into the clot. Within twelve hours of initiating thrombolytic infusion, there was brisk urine output. Interval venography demonstrated decreasing clot burden. At the time of discharge her creatinine was 0.78 mg/dL, similar to her baseline value prior to presentation. The patient was noted to have May-Thurner syndrome on intravascular ultrasound (IVUS. Angioplasty followed by stent placement was done. Unique to our case report was the timing of the presentation of renal vein thrombosis (four months after transplant and the predisposing anatomy consistent with May-Thurner syndrome, which was diagnosed with IVUS and successfully treated with local thrombolytics.

  5. Fatal outcome after renal transplant in a pediatric patient with Noonan syndrome.

    Science.gov (United States)

    Araz, Coskun; Kaval, Ebru; Torgay, Adnan; Moray, Gokhan; Haberal, Mehmet

    2015-04-01

    Noonan syndrome is a congenital, common, hereditary disorder. Facial dysmorphism, growth retardation, and various heart defects are typical clinical features. In patients with minor cardiac pathology, life expectancy is normal. We report a case of renal transplant in a pediatric patient with Noonan syndrome that ended with death of the patient. Our patient presented with unexpected and refractory postoperative neurological complications that were unresponsive to intensive therapy, and the patient died because of secondary complications.

  6. Hemorrhagic Fever with Renal Syndrome: Diagnostic Problems with a Known Disease

    OpenAIRE

    Wichmann, Dominic; Slenczka, Werner; Alter, Peter; Boehm, Stephan; Feldmann, Heinz

    2001-01-01

    Hemorrhagic fever with renal syndrome (HFRS), caused by different hantaviruses, is a distinct clinical syndrome endemic in several parts of Asia and Europe. However, the clinical picture can sometimes be indistinguishable from that of other infectious or noninfectious diseases. In this report we describe a clinical case, which is a rare occurrence but is a prime example of the difficulties in the diagnosis of HFRS in areas with a low prevalence of the disease.

  7. Acute renal failure in a patient with Sheehan syndrome and rhabdomyolysis.

    Science.gov (United States)

    Soltani, Parvin; Rezvanfar, Mohammad Reza; Pirasteh, Shadi

    2008-01-01

    We report a case of acute renal failure related to rhabdomyolysis in a patient with Sheehan syndrome, while other diseases that could cause rhabdomyolysis were excluded. The patient's kidney function completely recovered with 3 sessions of intermittent hemodialysis. After thyroxine replacement therapy, musculoskeletal symptoms disappeared and creatine kinase concentrations decreased. Steroid replacement therapy was also administered. The present case suggests that rhabdomyolysis could occur in a patient with Sheehan syndrome without other precipitating factors.

  8. Polycystic Ovary Syndrome: The Correlation Between Renal Doppler Ultrasound and Laboratory Parameters

    Directory of Open Access Journals (Sweden)

    Elif Karadeli

    2014-12-01

    Full Text Available Aim: To investigate whether there is alteration both right and left kidney lenght, parenchymal thickness, renal arterial,venous blood flow measurements in normotensive reproductive age women with polycystic ovary syndrome (PCOS. Material and Method: Forty women with PCOS according to Rotterdam criteria and thirty-six healthy volunteers women were included in our study. Hormonal, biochemical analysis, renal Doppler ultrasonography were performed and were investigated in terms of both left and right renal lenght, parenchymal thickness, peak systolic velocity (PSV, resistive index (RI, venous impedance index (VI, metabolic characteristics having insulin resistance, impaired glucose tolerance, serum lipid concentration. The student t test and pearson corelation test were used for statistical analysis.Results: The measurements for kidneys were not different between women with PCOS and healthy women. The peak systolic velocity of mean renal artery was lower in PCOS group. The mean renal venous impedance also was higher in PCOS group than control group. The mean renal resistive index was slightly higher in PCOS but not statistical significant. In bivariate corelation analyse including all patients, it was seen that BMI, WHR, level of serum fasting glucose, insulin, LDL, trigliserides were positively related with mean renal length and mean parenchymal thickness measurements. Discussion: We found that there was alterations kidney blood flow in normotensive reproductive age women with PCOS. This findings may indicate results of long term renal and cardiovascular complications of PCOS.

  9. BK virus as a potential oncovirus for bladder cancer in a renal transplant patient.

    Science.gov (United States)

    Yin, Wen-Yao; Lee, Ming-Che; Lai, Ning-Sheng; Lu, Ming-Chi

    2015-04-01

    Renal transplant patients have high risk for bladder cancer. The reactivation of BK virus is common in renal transplant patients especially in the urinary tract. There was some evidence suggesting that the reactivation of BK virus (BKV) in renal transplant patients may associate with the development of bladder cancer. Here we demonstrated that a patient that had persistent elevated BKV viruria (urine BKV DNA concentration more than 10(11) copies/ml) after renal transplantation. Then, bladder cancer was found in 13 months after kidney transplantation. The urine BKV DNA concentration was detected by real-time PCR and the BKV DNA in the bladder tumor was detected by PCR. BKV DNA was found in the marginal and central part of the bladder tumor. After removal of the bladder cancer, the urine BKV viral load in this patients dropped dramatically to <10(2) copies/ml. However, the urine viral load had increased modestly to 10(6) copies/ml in 3 months after surgery. Since there is a close correlation between the urine BK viral load and the presence of bladder cancer, we suggested that there might be a causal relationship between the reactivation of BKV and the development of bladder cancer in renal transplant patient.

  10. IgM AUTOANTIBODIES TO DNA IN BLOOD SERUM OF THE PATIENTS WITH HEMORRHAGIC FEVER WITH RENAL SYNDROME

    Directory of Open Access Journals (Sweden)

    D. G. Ishmukhametova

    2010-01-01

    Full Text Available Levels of IgM autoantibodies (AAbs to native (double-stranded and denaturated (single-stranded DNA were studied in blood serum of sixty patients with hemorrhagic fever with renal syndrome (HFRS and twenty-five healthy persons, using an ELISA technique. The median levels of IgM AAbs to double-stranded DNA in blood serum of healthy persons and HFRS patients corresponded to 0.41 and 0.53 arbitrary units, respectively. Thus, the difference between the samples from HFRS and healthy persons proved to be non-significant. The median level of IgM AAbs to single-stranded DNA in blood sera of HFRS patients (0.71 arbitrary units did significantly exceed serum values of healthy persons (0.57 arbitrary units. A probable involvement of IgM AAbs into regulation of IgG AAbs' production during virus-induced activation of autoimmune events in HFRS patients is discussed.

  11. Renal thrombotic microangiopathy and FIP1L1/PDGFRα-associated myeloproliferative variant of hypereosinophilic syndrome.

    Science.gov (United States)

    Langlois, Anne Lyse; Shehwaro, Nathalie; Rondet, Claire; Benbrik, Youssef; Maloum, Karim; Gueutin, Victor; Rouvier, Philippe; Izzedine, Hassane

    2013-08-01

    We report a case of renal thrombotic microangiopathy (TMA) in a myeloproliferative variant of hypereosinophilic syndrome (HES) in a 24-year-old man which resolved with imatinib therapy. This is one of a few cases in the literature to date describing TMA in HES, suggesting that the pathogenesis of thrombosis is at least in part related to damage from activated eosinophils.

  12. Renal dysfunction indicative of outcomes in hospitalized patients with takotsubo syndrome.

    Science.gov (United States)

    Ando, Kaoru; Sukekawa, Hiroyasu; Takahata, Aoi; Kobari, Yusuke; Tsuchiya, Hayato; Ishigaki, Daisuke; Tamabuchi, Toshiaki; Koyama, Yo

    2017-06-01

    Left ventricular dysfunction as part of takotsubo syndrome is reversible, and the long-term prognosis appears favorable. However, life-threatening complications are not uncommon during the acute phase, and it remains unclear whether renal dysfunction is a factor in complications suffered by hospitalized patients with takotsubo syndrome. The present study was conducted to investigate the implications of renal dysfunction in this setting. Data from 61 consecutive patients (male, 21; female, 40) diagnosed with takotsubo syndrome at our hospital between years 2010 and 2016 were evaluated retrospectively. In-hospital complications by definition were all-cause deaths and severe pump failure (Killip class ≥III). Overall, 30 patients (49%) developed renal dysfunction. In the 32 patients (52%) who suffered in-hospital complications (mortality, 10; severe pump failure, 22), estimated glomerular filtration rate (eGFR) was significantly lower by comparison (51.3±29.8 vs. 69.5±29.0; p=0.019). Low eGFR (60 ml/min per 1.73m(2)). Renal dysfunction is a simple but useful means of predicting complications in hospitalized patients with takotsubo syndrome, especially those with chronic kidney disease.

  13. The predictive value of mild renal insufficiency on the prognosis of patients with acute coronary syndrome

    Institute of Scientific and Technical Information of China (English)

    张建华

    2014-01-01

    Objective To investigate the predictive value of mild renal insufficiency on the endpoint events in patients with acute coronary syndrome(ACS).Methods A total of 552 patients with ACS were enrolled in the present study.According to the levels of estimated glomerular filtration rate(eGFR),patients were divided into two groups,normal

  14. Cardio-renal syndromes : report from the consensus conference of the Acute Dialysis Quality Initiative

    NARCIS (Netherlands)

    Ronco, Claudio; McCullough, Peter; Anker, Stefan D.; Anand, Inder; Aspromonte, Nadia; Bagshaw, Sean M.; Bellomo, Rinaldo; Berl, Tomas; Bobek, Ilona; Cruz, Dinna N.; Daliento, Luciano; Davenport, Andrew; Haapio, Mikko; Hillege, Hans; House, Andrew A.; Katz, Nevin; Maisel, Alan; Mankad, Sunil; Zanco, Pierluigi; Mebazaa, Alexandre; Palazzuoli, Alberto; Ronco, Federico; Shaw, Andrew; Sheinfeld, Geoff; Soni, Sachin; Vescovo, Giorgio; Zamperetti, Nereo; Ponikowski, Piotr

    2010-01-01

    A consensus conference on cardio-renal syndromes (CRS) was held in Venice Italy, in September 2008 under the auspices of the Acute Dialysis Quality Initiative (ADQI). The following topics were matter of discussion after a systematic literature review and the appraisal of the best available evidence:

  15. Progressive fluctuant hearing loss, enlarged vestibular aqueduct, and cochlear hypoplasia in branchio-oto-renal syndrome.

    NARCIS (Netherlands)

    Kemperman, M.H.; Stinckens, C.I.C.; Kumar, S.; Huygen, P.L.M.; Joosten, F.B.M.; Cremers, C.W.R.J.

    2001-01-01

    OBJECTIVE: To study the results of petrosal bone imaging and audiometric long-term follow-up of two patients with branchio-oto-renal (BOR) syndrome and relate them to the clinical features, including caloric responses. STUDY DESIGN: Longitudinal case study. SETTING: Tertiary referral center. PATIENT

  16. Hypokalemic periodic paralysis in Sjogren's syndrome secondary to distal renal tubular acidosis.

    Science.gov (United States)

    Yılmaz, Hakkı; Kaya, Mustafa; Özbek, Mustafa; ÜUreten, Kemal; Safa Yıldırım, İ

    2013-07-01

    We report a 53-year-old Turkish female presented with progressive weakness and mild dyspnea. Laboratory results demonstrated severe hypokalemia with hyperchloremic metabolic acidosis. The urinary anion gap was positive in the presence of acidemia, thus she was diagnosed with hypokalemic paralysis from a severe distal renal tubular acidosis (RTA). Immunologic work-up showed a strongly positive ANA of 1:3,200 and positive antibodies to SSA and SSB. Schirmer's test was abnormal. Autoimmune and other tests revealed Sjögren syndrome as the underlying cause of the distal renal tubular acidosis. Renal involvement in Sjogren's syndrome (SS) is not uncommon and may precede sicca complaints. The pathology in most cases is a tubulointerstitial nephritis causing among other things, distal RTA, and, rarely, hypokalemic paralysis. Treatment consists of potassium repletion, alkali therapy, and corticosteroids. Primary SS could be a differential in women with acute weakness and hypokalemia.

  17. Acute renal failure: A rare presentation of Sheehan's syndrome.

    Science.gov (United States)

    Bhat, Manzoor A; Laway, Bashir A; Allaqaband, Faheem A; Kotwal, Suman K; Wani, Imtiyaz A; Banday, Khursheed A

    2012-03-01

    Sheehan's syndrome occurs as a result of ischemic pituitary necrosis secondary to severe postpartum bleeding. It is one of the most common causes of hypopituitarism, characterized by variable clinical presentation. Acute kidney injury occurs rarely in Sheehan's syndrome and most of the cases have been found to be precipitated by rhabdomyolysis. We here present a case of Sheehan's syndrome with acute kidney injury where theprecipitating cause was chronic hypocortisolemia. We believe this is the first reported case of Sheehan's syndrome in which acute kidney injury was precipitated by adrenal insufficiency.

  18. Zika Virus Infection of the Human Glomerular Cells: Implications for Viral Reservoirs and Renal Pathogenesis.

    Science.gov (United States)

    Alcendor, Donald J

    2017-07-15

    Zika virus (ZIKV) infection in the human renal compartment has not been reported. Several clinical reports have describe high-level persistent viral shedding in the urine of infected patients, but the associated mechanisms have not been explored until now. The current study examined cellular components of the glomerulus of the human kidney for ZIKV infectivity. I infected primary human podocytes, renal glomerular endothelial cells (GECs), and mesangial cells with ZIKV. Viral infectivity was analyzed by means of microscopy, immunofluorescence, real-time reverse-transcription polymerase chain reaction (RT-PCR), and quantitative RT-PCR (qRT-PCR), and the proinflammatory cytokines interleukin 1β, interferon β, and RANTES (regulated on activation of normal T cells expressed and secreted) were assessed using qRT-PCR. I show that glomerular podocytes, renal GECs, and mesangial cells are permissive for ZIKV infection. ZIKV infectivity was confirmed in all 3 cell types by means of immunofluorescence staining, RT-PCR, and qRT-PCR, and qRT-PCR analysis revealed increased transcriptional induction of interleukin 1β, interferon β, and RANTES in ZIKV-infected podocytes at 72 hours, compared with renal GECs and mesangial cells. The findings of this study support the notion that the glomerulus may serve as an amplification reservoir for ZIKV in the renal compartment. The impact of ZIKV infection in the human renal compartment is unknown and will require further study.

  19. Risk Factors for Severe Renal Disease in Bardet-Biedl Syndrome.

    Science.gov (United States)

    Forsythe, Elizabeth; Sparks, Kathryn; Best, Sunayna; Borrows, Sarah; Hoskins, Bethan; Sabir, Ataf; Barrett, Timothy; Williams, Denise; Mohammed, Shehla; Goldsmith, David; Milford, David V; Bockenhauer, Detlef; Foggensteiner, Lukas; Beales, Philip L

    2017-03-01

    Bardet-Biedl syndrome is a rare autosomal recessive, multisystem disease characterized by retinal dystrophy, renal malformation, obesity, intellectual disability, polydactyly, and hypogonadism. Nineteen disease-causing genes (BBS1-19) have been identified, of which mutations in BBS1 are most common in North America and Europe. A hallmark of the disease, renal malformation is heterogeneous and is a cause of morbidity and mortality through the development of CKD. We studied the prevalence and severity of CKD in 350 patients with Bardet-Biedl syndrome-related renal disease attending the United Kingdom national Bardet-Biedl syndrome clinics to further elucidate the phenotype and identify risk indicators of CKD. Overall, 31% of children and 42% of adults had CKD; 6% of children and 8% of adults had stage 4-5 CKD. In children, renal disease was often detected within the first year of life. Analysis of the most commonly mutated disease-associated genes revealed that, compared with two truncating mutations, two missense mutations associated with less severe CKD in adults. Moreover, compared with mutations in BBS10, mutations in BBS1 associated with less severe CKD or lack of CKD in adults. Finally, 51% of patients with available ultrasounds had structural renal abnormalities, and 35% of adults were hypertensive. The presence of structural abnormalities or antihypertensive medication also correlated statistically with stage 3b-5 CKD. This study describes the largest reported cohort of patients with renal disease in Bardet-Biedl syndrome and identifies risk factors to be considered in genetic counseling.

  20. Transient renal Fanconi syndrome in a Chihuahua exposed to Chinese chicken jerky treats.

    Science.gov (United States)

    Hooijberg, E H; Furman, E; Leidinger, J; Brandstetter, D; Hochleithner, C; Sewell, A C; Leidinger, E; Giger, U

    2015-01-01

    Transient Fanconi syndrome without azotemia was diagnosed in a dog and was associated with ingestion of Chinese chicken jerky treats. Fanconi syndrome is a proximal renal tubular defect and a diagnosis was made based upon severe glucosuria with normoglycemia, and severe generalized aminoaciduria. The clinical signs of polyuria and polydipsia as well as the massive urinary metabolic abnormalities resolved after jerky treat withdrawal. While frequently seen in North America and Australia, this is the first report of jerky treat induced Fanconi syndrome in continental Europe. Clinicians should be aware of this potential intoxication and be vigilant for a history of jerky treat consumption in a dog with glucosuria.

  1. A reversible bilateral renal artery stenosis in association with antiphospholipid syndrome.

    Science.gov (United States)

    Remondino, G I; Mysler, E; Pissano, M N; Furattini, M C; Basta, M C; Presas, J L; Allievi, A

    2000-01-01

    We describe a 26-year-old white female with a history of Raynaud phenomenon, erythema nodosum, polyarthralgias, migraine, vertigo, seizures, transient ischemic attacks, one fetal loss, and false positive VDRL, who developed milk hypertension without overt lupus nephritis. She had positive antinuclear antibodies (ANA) and double-stranded deoxyribonucleic acid (dsDNA) antibodies. The lupus anticoagulant test (LAC) and cardiolipins antibodies (aCL) were positive. She was diagnosed as having a Systemic Lupus Erythematosus-like illness (SLE-like) with 'secondary' antiphospholipid syndrome (APS). Renal spiral computed tomography (CT) with intravenous (IV) contrast showed bilateral renal artery stenosis. Anticoagulation with acenocumarol was started. She became normotensive without antihypertensive drugs five months later. A follow-up renal spiral CT showed complete recanalization of both renal arteries, making thrombosis the more likely culprit pathology in the stenosis. After two years follow up the patient is normotensive. She remains on acenocumarol.

  2. Living related donor renal transplant in human immunodeficiency virus infected patient: Case reports from tertiary care hospital in western India

    Directory of Open Access Journals (Sweden)

    Sonal Dalal

    2014-01-01

    Full Text Available Renal transplantation (TX in human immunodeficiency virus (HIV infected patients with end stage renal disease (ESRD is increasingly performed in developed countries in the era of antiretroviral therapy (ART. Management of HIV infected patients during and post-transplant is very complex and challenging due to drug interaction, infection risk and associated co-infections. We described our experience with living related donor renal TX in three HIV infected patients.

  3. Pharmacokinetics, Efficacy, and Safety of Hepatitis C Virus Drugs in Patients with Liver and/or Renal Impairment

    NARCIS (Netherlands)

    Smolders, Elise J; de Kanter, Clara T M M; van Hoek, Bart; Arends, Joop E; Drenth, Joost P H; Burger, David M

    2016-01-01

    Hepatitis C virus (HCV)-infected patients often suffer from liver cirrhosis, which can be complicated by renal impairment. Therefore, in this review we describe the treatment possibilities in HCV patients with hepatic and renal impairment. Cirrhosis alters the structure of the liver, which affects d

  4. A case of neuroleptic malignant syndrome with acute renal failure after the discontinuation of sulpiride and maprotiline.

    Science.gov (United States)

    Kiyatake, I; Yamaji, K; Shirato, I; Kubota, M; Nakayama, S; Tomino, Y; Koide, H

    1991-01-01

    A 46-year-old man developed neuroleptic malignant syndrome with acute myoglobinuric renal failure after the discontinuation of sulpiride and maprotiline treatment. He showed the characteristic features of hyperpyrexia, altered consciousness, muscle rigidity, and autonomic dysfunction. Laboratory data showed lysis of skeletal muscle cells and renal impairment. Muscle biopsy revealed necrosis and regenerative changes in muscle fibers. Renal biopsy showed focal tubulitis and interstitial infiltration of small inflammatory cells. The combination of sulpiride and maprotiline has not previously been reported to be the cause of neuroleptic malignant syndrome and acute myoglobinuric renal failure.

  5. Is there an association of hepatitis B virus infection with minimal change disease of nephrotic syndrome? A clinical observational report.

    Science.gov (United States)

    Zhou, Tian-Biao; Jiang, Zong-Pei

    2015-04-01

    The rate of hepatitis B virus (HBV) infection is high in the Chinese population, and the implications of HBV infection are widely recognized, and membranous nephropathy is the most common renal lesion to be associated with HBV infection. Minimal change disease (MCD) is one of the most important histopathological characteristics in patients with nephrotic syndrome. There is no any study to report that HBV infection is associated with the etiology of MCD. Herein, we report four MCD patients with HBV infection and speculate that there is an association of HBV infection with the pathological type of MCD. In this study, we also reported the treatment schedule for these four MCD patients, and found that the anti-virus alone and combination of anti-virus with immunosuppressive agent could obtain a benefit for MCD patients with HBV infection. However, a well-designed study should be performed to confirm this association.

  6. Renal involvement in MELAS syndrome - a series of 5 cases and review of the literature.

    Science.gov (United States)

    Seidowsky, Alexandre; Hoffmann, Maxime; Glowacki, François; Dhaenens, Claire-Marie; Devaux, Jean-Philippe; de Sainte Foy, Celia Lessore; Provot, François; Gheerbrant, Jean-Dominique; Hummel, Aurelie; Hazzan, Marc; Dracon, Michel; Dieux-Coeslier, Anne; Copin, Marie-Christine; Noël, Christian; Buob, David

    2013-12-01

    Renal dysfunction is increasingly recognized as a potential clinical feature of mitochondrial cytopathies such as mitochondrial encephalomyopathy, lacticacidosis and stroke-like episodes (MELAS) syndrome. Five cases of MELAS syndrome with renal involvement from 4 unrelated families are presented in this case series. Three of the 5 patients had a history of maternally-inherited diabetes and/or deafness. Focal and segmental glomerulosclerosis and arteriolar hyaline thickening were the most striking findings on renal biopsy. In addition to clinical presentation with the typical symptoms of MELAS syndrome, genetic testing in these patients identified the A3243G point mutation in the tRNALeu gene of the mitochondrial DNA (mtDNA). The diagnosis of MELAS syndrome was thus considered to be unequivocal. The incidence of kidney disease in MELAS syndrome may be underestimated although a study is required to investigate this hypothesis. As the A3243G mtDNA mutation leads to a progressive adult-onset form of focal segmental glomerulosclerosis (FSGS), screening for the MELAS A3243G mtDNA mutation should therefore be performed especially in patients with maternally-inherited diabetes or hearing loss presenting with FSGS.

  7. De Novo collapsing glomerulopathy in renal allograft in association with BK virus nephropathy in a child and stabilization of renal function by elimination of viremia

    Directory of Open Access Journals (Sweden)

    D N Gera

    2017-01-01

    Full Text Available Well-recognized association between HIV 1 infection and collapsing glomerulopathy (CG raises the possibility that intrarenal infection by other viruses may also contribute to the development of this lesion in native or post-transplant kidneys. There is evidence in literature about association of these lesions with cytomegalovirus, Epstein–Barr virus, hepatitis C virus, and parvovirus B19 infections. Here, we present a case report of post-transplant BK virus nephropathy in a male child who was found to have CG in subsequent biopsy 2 months later. His renal function and proteinuria were stabilized on elimination of viremia.

  8. New syndrome with ocular, skeletal and renal involvement

    Energy Technology Data Exchange (ETDEWEB)

    Cirillo Silengo, M.; Lopez Bell, G.; Biagioli, M.; Guala, A.; Franceschini, P.; Porcellini, G.

    1987-03-01

    A patient with retinitis pigmentosa, hypertension with interstitial nephropathy, short limb dwarfism with Madelung deformity of the forearms and an unclassified type of brachydactyly is described. Such bone dysplasia has never been reported to date either as a single entity or associated with renal and retinal diseases.

  9. [Urolithiasis due to renal tubular acidosis associated with Sjögren's syndrome].

    Science.gov (United States)

    Umekawa, T; Esa, A; Uemura, T; Kohri, K; Kurita, T; Ishikawa, Y; Iguchi, M; Kataoka, K

    1990-03-01

    We encountered 4 patients with urolithiasis due to renal tubular acidosis (RTA) associated with Sjögren's syndrome. Laboratory results about RTA in 4 patients with Sjögrenhs syndrome were not significantly different from those in patients who suffered from urolithiasis due to RTA without Sjögren's syndrome. The incidence of urolitiasis in these cases was suspected to be higher than that in RTA patients without Sjögren's syndrome, because all 4 patients in this study had urolithiasis. When we examine patients with bilateral and multiple urolithiasis, particularly in middle-aged women, we should bear in mind that RTA and Sjögren's syndrome may exist in the background.

  10. Dynamics of hormonal status in women of different age groups in hemorrhagic fever with renal syndrome

    Directory of Open Access Journals (Sweden)

    Murzabaeva R.Т.

    2012-06-01

    Full Text Available Objective: To study the hormonal parameters in women of different age groups in hemorrhagic fever with renal syndrome. Materials and methods: We have studied the content of cortisol, thyrotropic hormone (TTH, triiodothyro-nine (T3, free thyroxin (FT4, luteinizing hormone (LH, follicle-stimulating hormone (FSH, testosterone, estradiol, progesterone, prolactin in blood serum of 62 women with moderate (33 and severe (29 HFRS forms age (17-62. They were divided into 2 groups: the first group (33 patients women with the normal menstrual cycle, the second group (29 women consisted of patients in climacteric period. Results: TTH secretion increase, T3 and FT4 — decrease with their normalization to the recovery period were registered in the thyroid system of the compared groups. Blood cortisol level was high during the illness. Gonadotropic hypophysis function study demonstrated that LH and blood prolactin concentrations were increased since oliguria period; FSH was authentic reduced. The indices of these hormones were restored to the normal level by the reconvalescence period. LH and FSH contents were authentic higher in women of the second group in comparison with the first group. The hyperprolactinemia was observed in both women groups during the whole period of disease. The increased progesterone and testosterone concentrations have been manifested in blood serum. The estradiol concentration had different direction tendencies. Conclusion: Thus, the complex study of hypophysic- thyreoid and gonadotropic hormone state of adrenal system and the sexual hormone levels in women of different age groups in HFRS revealed the hormone status indces changes due to the period and severity of the disease, connected with the virus action, intoxication, the general inflammation reactions and their age.

  11. Evidence of hepatitis E virus transmission by renal graft.

    Science.gov (United States)

    Pourbaix, Annabelle; Ouali, Nacera; Soussan, Patrick; Roque Afonso, Anne Marie; Péraldi, Marie-Noelle; Rondeau, Eric; Peltier, Julie

    2017-02-01

    Hepatitis E virus (HEV) can cause chronic infection among immunocompromised patients, especially solid organ transplant recipients, and can evolve to cirrhosis. Several modes of transmission are known. Here we describe the first two cases, to our knowledge, of HEV infection transmitted by a kidney graft from the same infected donor that led to chronic hepatitis. Consequently, systematic screening of donors by HEV serology and HEV RNA detection by polymerase chain reaction, particularly in endemic regions, should be considered.

  12. Eculizumab prevents recurrent antiphospholipid antibody syndrome and enables successful renal transplantation.

    Science.gov (United States)

    Lonze, B E; Zachary, A A; Magro, C M; Desai, N M; Orandi, B J; Dagher, N N; Singer, A L; Carter-Monroe, N; Nazarian, S M; Segev, D L; Streiff, M B; Montgomery, R A

    2014-02-01

    Renal transplantation in patients with antiphospholipid antibodies has historically proven challenging due to increased risk for thrombosis and allograft failure. This is especially true for patients with antiphospholipid antibody syndrome (APS) and its rare subtype, the catastrophic antiphospholipid antibody syndrome (CAPS). Since a critical mechanism of thrombosis in APS/CAPS is one mediated by complement activation, we hypothesized that preemptive treatment with the terminal complement inhibitor, eculizumab, would reduce the extent of vascular injury and thrombosis, enabling renal transplantation for patients in whom it would otherwise be contraindicated. Three patients with APS, two with a history of CAPS, were treated with continuous systemic anticoagulation together with eculizumab prior to and following live donor renal transplantation. Two patients were also sensitized to human leukocyte antigens (HLA) and required plasmapheresis for reduction of donor-specific antibodies. After follow-up ranging from 4 months to 4 years, all patients have functioning renal allografts. No systemic thrombotic events or early graft losses were observed. While the appropriate duration of treatment remains to be determined, this case series suggests that complement inhibitors such as eculizumab may prove to be effective in preventing the recurrence of APS after renal transplantation.

  13. Endocytic receptor LRP2/megalin-of holoprosencephaly and renal Fanconi syndrome.

    Science.gov (United States)

    Willnow, Thomas E; Christ, Annabel

    2017-08-01

    Megalin (or LRP2) is an endocytic receptor that plays a central role in embryonic development and adult tissue homeostasis. Loss of this receptor in congenital or acquired diseases results in multiple organ dysfunctions, including forebrain malformation (holoprosencephaly) and renal reabsorption defects (renal Fanconi syndrome). Here, we describe current concepts of the mode of receptor action that include co-receptors and a repertoire of different ligands, and we discuss how these interactions govern functional integrity of the kidney and the brain, and cause disease when defective.

  14. Sorafenib-induced hand-foot syndrome in a patient of renal cell carcinoma

    Directory of Open Access Journals (Sweden)

    Amrita Sil

    2014-01-01

    Full Text Available Sorafenib, a multikinase inhibitor, is approved for treatment of renal cell cancer and hepatocellular cancer. Hand-foot syndrome (HFD is a condition where erythema, scaling, and bullous lesion affect the hand and feet. In this case, a post-nephrectomy renal carcinoma patient prescribed sorafenib developed HFD 1 week after the drug usage. All laboratory parameters were within normal limits. The dose of sorafenib was reduced and topical corticosteroids, antihistamines, and emollients were prescribed. The reaction reduced after 2 weeks of therapy, only to reappear again when the second cycle of sorafenib-targeted therapy was started. The case was diagnosed as sorafenib-induced HFD.

  15. Posterior reversible encephalopathy syndrome in a hypertensive patient with renal failure

    Directory of Open Access Journals (Sweden)

    T Aatif

    2016-01-01

    Full Text Available Posterior reversible encephalopathy syndrome (PRES is a clinical and neuroimaging entity characterized by headache, visual field deficits, changes in mentation and seizures, and by typical neuro-imaging features such as areas of sub-cortical edema, occasionally cortical, involving predominantly the occipital and parietal lobes of both hemispheres. Hypertension, uremia, immunosuppressive drugs neurotoxicity, preeclampsia or eclampsia, renal disease, and sepsis are the most common etiologies of PRES. Less common, it has been described in the setting of autoimmune disease. We report a case of PRES which was associated with hypertensive crisis in a patient with renal failure. Antihypertensive therapy and hemodialysis resulted in complete recovery.

  16. [Sjögren syndrome associated with renal tubular acidosis type I].

    Science.gov (United States)

    Górriz, L; Molino, R; Arjona, D; Estripeaut, D

    2000-01-01

    Primary Sjögren's Syndrome complicated with a renal tubular acidosis type 1 and hypocalcemic paralysis, as the principal clinical manifestation, is uncommon. Although the initial manifestations of the nephropathy are not well understood, it is believed that the invasion of mononuclear cells and the high level of circulating antibodies, play an important role in the pathogenesis of the disease. We present a patient with hypocalcemic paralysis as an initial manifestation of a latent Sjögren's disease. The glandular biopsy was normal, suggesting a mayor participation of an immunological humoral factor in the renal lesion.

  17. Anatomical Changes and Audiological Profile in Branchio-oto-renal Syndrome: A Literature Review

    Directory of Open Access Journals (Sweden)

    Lindau, Tâmara Andrade

    2014-01-01

    Full Text Available Introduction Branchio-oto-renal (BOR syndrome is an autosomal-dominant genetic condition with high penetrance and variable expressivity, with an estimated prevalence of 1 in 40,000. Approximately 40% of the patients with the syndrome have mutations in the gene EYA1, located at chromosomal region 8q13.3, and 5% have mutations in the gene SIX5 in chromosome region 19q13. The phenotype of this syndrome is characterized by preauricular fistulas; structural malformations of the external, middle, and inner ears; branchial fistulas; renal disorders; cleft palate; and variable type and degree of hearing loss. Aim Hearing loss is part of BOR syndrome phenotype. The aim of this study was to present a literature review on the anatomical aspects and audiological profile of BOR syndrome. Data Synthesis Thirty-four studies were selected for analysis. Some aspects when specifying the phenotype of BOR syndrome are controversial, especially those issues related to the audiological profile in which there was variability on auditory standard, hearing loss progression, and type and degree of the hearing loss. Mixed loss was the most common type of hearing loss among the studies; however, there was no consensus among studies regarding the degree of the hearing loss.

  18. Anatomical Changes and Audiological Profile in Branchio-oto-renal Syndrome: A Literature Review.

    Science.gov (United States)

    Lindau, Tâmara Andrade; Cardoso, Ana Cláudia Vieira; Rossi, Natalia Freitas; Giacheti, Célia Maria

    2014-01-01

    Introduction Branchio-oto-renal (BOR) syndrome is an autosomal-dominant genetic condition with high penetrance and variable expressivity, with an estimated prevalence of 1 in 40,000. Approximately 40% of the patients with the syndrome have mutations in the gene EYA1, located at chromosomal region 8q13.3, and 5% have mutations in the gene SIX5 in chromosome region 19q13. The phenotype of this syndrome is characterized by preauricular fistulas; structural malformations of the external, middle, and inner ears; branchial fistulas; renal disorders; cleft palate; and variable type and degree of hearing loss. Aim Hearing loss is part of BOR syndrome phenotype. The aim of this study was to present a literature review on the anatomical aspects and audiological profile of BOR syndrome. Data Synthesis Thirty-four studies were selected for analysis. Some aspects when specifying the phenotype of BOR syndrome are controversial, especially those issues related to the audiological profile in which there was variability on auditory standard, hearing loss progression, and type and degree of the hearing loss. Mixed loss was the most common type of hearing loss among the studies; however, there was no consensus among studies regarding the degree of the hearing loss.

  19. ADQI 7 : the clinical management of the Cardio-Renal syndromes: work group statements from the 7th ADQI consensus conference

    NARCIS (Netherlands)

    Davenport, A.; Anker, S. D.; Mebazaa, A.; Palazzuoli, A.; Vescovo, G.; Bellomo, R.; Ponikowski, P.; Anand, I.; Aspromonte, N.; Bagshaw, S.; Berl, T.; Bobek, I.; Cruz, D. N.; Daliento, L.; Haapio, M.; Hillege, H.; House, A.; Katz, N.; Maisel, A.; Mankad, S.; McCullough, P.; Ronco, F.; Shaw, A.; Sheinfeld, G.; Soni, S.; Zamperetti, N.; Zanco, P.; Ronco, C.

    2010-01-01

    Many patients with heart failure have underlying renal dysfunction, and similarly, patients with kidney failure are prone to cardiac failure. This has led to the concept of cardio-renal syndromes, which can be an acute or chronic cardio-renal syndrome, when cardiac failure causes deterioration in re

  20. White spot syndrome virus inactivation study by using gamma irradiation

    Science.gov (United States)

    Heidareh, Marzieh; Sedeh, Farahnaz Motamedi; Soltani, Mehdi; Rajabifar, Saeed; Afsharnasab, Mohammad; Dashtiannasab, Aghil

    2014-09-01

    The present study was conducted to investigate the effect of gamma irradiation on white spot syndrome virus (WSSV). White spot syndrome virus is a pathogen of major economic importance in cultured penaeid shrimp industries. White spot disease can cause mortalities reaching 100% within 3-10 days of gross signs appearing. During the period of culture, immunostimulant agents and vaccines may provide potential methods to protect shrimps from opportunistic and pathogenic microrganisms. In this study, firstly, WSSV was isolated from infected shrimp and then multiplied in crayfish. WSSV was purified from the infected crayfish haemolymph by sucrose gradient and confirmed by transmission electron microscopy. In vivo virus titration was performed in shrimp, Penaeus semisulcatus. The LD50 of live virus stock was calculated 10 5.4/mL. Shrimp post-larvae (1-2 g) were treated with gamma-irradiated (different doses) WSSV (100 to 10-4 dilutions) for a period of 10 days. The dose/survival curve for irradiated and un-irradiated WSSV was drawn; the optimum dose range for inactivation of WSSV and unaltered antigenicity was obtained 14-15 kGy. This preliminary information suggests that shrimp appear to benefit from treatment with gammairradiated WSSV especially at 14-15 KGy.

  1. Renal dysfunction and anemia in patients with heart failure — the cardio-renal anemia syndrome

    OpenAIRE

    Malić, Daniela; Matijević, Sanja; Bubić, Ivan; Rački, Sanjin; Zaputović, Luka

    2011-01-01

    Cardiovascular diseases are the leading cause of morbidity and mortality in patients with chronic kidney disease. The appearance of cardiovascular complications is strongly in positive correlation with the severity of kidney disease. About 40% of patients with moderate or severe kidney disease and even 60% of patients in the terminal phase have some degree of chronic heart failure. “The Cardio-Renal Syndrome” represents a variety of pathophysiological abnormalities of ...

  2. Hemorrhagic syndrome and Acute renal failure in a pregnant woman after contact with Lonomia caterpillars: a case report

    Directory of Open Access Journals (Sweden)

    FAN Hui Wen

    1998-01-01

    Full Text Available A case of a 37-week pregnant woman who developed a hemorrhagic syndrome and acute renal failure after contact with Lonomia caterpillars is reported. The accident also initiated labour and the patient gave birth to an alive child. Some pathophysiological aspects of the genital bleeding and of the acute renal failure are discussed.

  3. Identification of a novel EYA1 splice-site mutation in a Danish branchio-oto-renal syndrome family

    DEFF Research Database (Denmark)

    Henriksen, Ann Marie; Tümer, Zeynep; Tommerup, Niels

    2004-01-01

    Branchio-oto-renal (BOR) syndrome is an autosomal dominant disorder characterized by variable clinical manifestations including branchial fistulae, preauricular pits, ear malformations, hearing impairment, and renal anomalies. BOR is caused by mutations in the genes EYA1 and SIX1. A Danish BOR...

  4. Association between the glomerular filtration rate of renal dysfunction and metabolic syndrome: an age-stratified analysis

    Institute of Scientific and Technical Information of China (English)

    宋慧

    2014-01-01

    Objective To explore the relationship between the renal dysfunction rate and metabolic syndrome(MS),stratified by age.Methods People took part in physical check-up in a certain tertiary hospital from March 2010to September 2012,were enrolled in this study.Estimated glomerular filtration rate(e GFR),—a renal dysfunction indicator,was calculated by modified MDRD

  5. Hereditary Leiomyomatosis and Renal Cell Carcinoma Syndrome Combined With Adrenocortical Carcinoma on 18F-FDG PET/CT.

    Science.gov (United States)

    Guo, Xiuyu; Chen, Haojun; Fu, Hao; Wu, Hua

    2017-09-01

    Hereditary leiomyomatosis and renal cell carcinoma (HLRCC) syndrome is a recognized distinct phenotypic variant of multiple cutaneous and uterine leiomyomatosis. The present case reports an extremely rare case of HLRCC syndrome combined with adrenocortical carcinoma. The case suggests that HLRCC should be considered in any young patient with bulky uterine leiomyomas and renal cell cancer, and F-FDG PET/CT can help detect unexpected additional primary malignancy in a patient with known cancer.

  6. The Perlman syndrome: familial renal dysplasia with Wilms tumor, fetal gigantism and multiple congenital anomalies. 1984.

    Science.gov (United States)

    Neri, Giovanni; Martini-Neri, Maria Enrica; Katz, Ben E; Opitz, John M

    2013-11-01

    The ensuing paper by Professor Giovanni Neri and colleagues was originally published in 1984, American Journal of Medical Genetics 19:195–207. The original article described a new family with a condition that the authors designated as the Perlman syndrome. This disorder, while uncommon, is an important multiple congenital anomaly and dysplasia syndrome; the causative gene was recently identified. This paper is a seminal work and is graciously republished by Wiley-Blackwell in the Special Festschrift issue honoring Professor Neri. We describe a familial syndrome of renal dysplasia, Wilms tumor, hyperplasia of the endocrine pancreas, fetal gigantism, multiple congenital anomalies and mental retardation. This condition was previously described by Perlman et al. [1973, 1975] and we propose to call it the "Perlman syndrome." It appears to be transmitted as an autosomal recessive trait. The possible relationships between dysplasia, neoplasia and malformation are discussed. © 2013 Wiley Periodicals, Inc.

  7. Anterior pituitary lobe atrophy as late complication of hemorrhagic fever with renal syndrome

    Directory of Open Access Journals (Sweden)

    Jovanović Dragan

    2009-01-01

    Full Text Available Introduction. Hemorrhagic fever with renal syndrome (HFRS is acute infective multisystemic disease followed by febrility, hemorrhages and acute renal insufficiency. Bleeding in the anterior pituitary lobe leading to tissue necrosis occurs in acute stage of severe clinical forms of HFRS, while atrophy of the anterior pituitary lobe with diminution of the gland function occurs after recovery stage. Case report. We presented a patient with the development of chronic renal insufficiency and hypopituitarism as complication that had been diagnosed six years after Hantavirus infection. Magnetic resonance of the pituitary gland revealed atrophy and empty sella turcica. Conclusion. Regarding frequency of this viral infection and its endemic character in some parts of our country partial and/or complete loss of pituitary function should be considered during the late stage of HFRS.

  8. Prevalance of anemia and its association with cardio-renal syndrome.

    Science.gov (United States)

    Silva, Ricardo P; Barbosa, Paulo H U; Kimura, Osamu S; Sobrinho, Carlos Roberto M R; Sousa Neto, João David; Silva, Frederico A L; Silva Júnior, Geraldo B; Mota, Rosa M S; Daher, Elizabeth F

    2007-08-21

    Anemia is common in cardio-renal syndrome and may contribute to increase mortality. To examine the prevalence of anemia and its relationship with cardio-renal syndrome, and to evaluate the risk factors for death. Retrospective study with all patients admitted with congestive heart failure (CHF). The parameters as age, gender, hemoglobin (Hb), estimated glomerular filtration rate (eGFR), New York Heart Association (NYHA) functional class, ejection fraction (EF%), hospital stay, hypertension, diabetes, smoking and CHF etiology were analyzed. Anemia was defined as Hbrenal failure was stratified according to K-DOQI classification. Statistical analysis was done by the programs EpiInfo and SPSS for windows. A total of 174 patients were studied. The average age was 63+/-16 years, 65.5% were males, and 18 of them (11%) were non-survivors. Anemia was observed in 45% of patients, and 82% presented some degree of renal failure. The majority of patients (87%) were classified as NYHA functional class III or IV. The average ejection fraction was 43.9+/-16.6%, and there was no difference between survivors and non-survivors (p>0.05). Mortality was not significantly higher among patients with anemia (12.4%) when comparing to those without anemia (8.3%, p=0.31). There was a progressive decrease in the level of hemoglobin as renal function decreased (pprevalence of anemia is high among patients with cardio-renal syndrome but was not associated with increased mortality. Increased serum creatinine and low EF% were variables associated with death.

  9. Acute forearm compressive myopathy syndrome secondary to upper limb entrapment: an unusual cause of renal failure.

    Science.gov (United States)

    Tachtsi, Maria D; Kalogirou, Thomas E; Atmatzidis, Stefanos K; Papadimitriou, Dimitrios K; Atmatzidis, Konstantinos S

    2011-05-01

    Compressive myopathy syndrome (SCM) is a syndrome characterized by the lesion of skeletal muscle resulting in subsequent release of intracellular contents (myoglobin, creatine phosphokinase, potassium, etc.) into the circulatory system, which can cause potentially lethal complications. There are numerous causes that can lead to SCM resulting to acute rhabdomyolysis, and many patients present with multiple causes. The most common potentially lethal complication is acute renal failure. The occurrence of acute rhabdomyolysis should be considered as a possibility in any patient who can remain stationary for long periods, or is in a coma, or is intoxicated in any form. We report the rare case of a 26-year-old patient who developed SCM caused by ischemia reperfusion, with subsequent acute rhabdomyolysis and acute renal failure after prolonged compression of the right upper extremity.

  10. Herlyn-Werner-Wunderlich Syndrome with Unilateral Hemivaginal Obstruction, Ipsilateral Renal Agenesis, and Contralateral Renal Thin GBM Disease: A Case Report with Radiological Follow Up

    Energy Technology Data Exchange (ETDEWEB)

    Park, Noh Hyuck; Park, Hee Jin; Park, Chan Sup [Myongji Hospital, Kwandong University, Koyang (Korea, Republic of); Park, Sung Il [Bucheon Hospital, Soonchunhyang University, Bucheon (Korea, Republic of)

    2010-08-15

    Herlyn-Werner-Wunderlich syndrome is a rare Mullerian ductal anomaly that is characterized by the presence of a hemivaginal septum, a didelphic uterus and ipsilateral renal agenesis. It is generally difficult to diagnose the uterine malformation before menarche owing to its small size. Therefore, a follow-up study is very important for confirming the uterine malformation in girls with renal agenesis. We report a patient with renal agenesis and microscopic hematuria, who showed symptoms before menarche. A follow-up study eventually revealed uterine didelphys with a hemivaginal obstruction. A biopsy proved that the microscopic hematuria was caused by thin glomerular basement membrane disease of the contralateral kidney

  11. Ocular, Ear and Renal Manifestations of Alport Syndrome in Three Iranian Families

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    Mohammad Hossein Davari

    2014-12-01

    Full Text Available Alport syndrome is a genetic disorder of basement membranes caused by mutations in type IV collagen network. It was first identified by Dr. Alport in 1927. Its major clinical manifestations are included: glomerulopathy, sensory hearing loss, anterior lenticonus, and the prevalence of Alports’ gene in general population is about 1 in 5000 and the disease prevalence is 1 in 10000 [1-3]. Here we report ocular, ear and renal manifestations of Alport syndrome in 3 families that living in the east city of Iran, Birjand, and we have followed all of this family for 6 years so far.

  12. A Case of Leser-Trélat Syndrome Associated with a Renal Cell Carcinoma

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    Dietrich Barth

    2015-09-01

    Full Text Available Seborrheic keratoses can often be found in elderly people. In general, they appear gradually. In cases of a sudden eruption with itching it might be paraneoplastic. Although some authors doubt the existence of the paraneoplastic Leser-Trélat syndrome, we present a case of sudden eruption of seborrheic keratoses connected with a newly diagnosed renal cell carcinoma. As far as we know, this is the first case report of a Leser-Trélat syndrome with a malignancy of the kidney.

  13. Evaluation of metabolic syndrome and associations with inflammation and graft function in renal transplant recipients

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    Mariana Gascue de Alencastro

    2013-12-01

    Full Text Available INTRODUCTION: Cardiovascular disease (CVD is a major determinant of mortality in renal transplant recipients (RTR. Metabolic syndrome (MS and chronic inflammation are currently considered non traditional risk factors for cardiovascular disease. This study evaluates the frequency of these conditions their associations with graft function. OBJECTIVE: To evaluate the prevalence of metabolic syndrome (MS and inflammation and their associations with graft function in renal transplant recipients. METHODS: A cross-sectional study was carried out with 200 RTR. MS was defined by the NCEP-ATP III criteria. Inflammation was assessed by CRP levels. Renal function was assessed by GFR estimation using the MDRD equation. RESULTS: MS occurred in 71 patients (35.5%. Patients with MS had higher CPR and decreased GFR levels. Inflammation was present in 99 patients (49.5%. Mean waist perimeter, body mass index, triglycerides and serum total cholesterol were significantly higher in inflamed patients. An association between MS and inflammation was demonstrated, 48 (67.6% patients with MS were inflamed and among those without MS the rate of inflamed patients was 39.5% (51 patients (p < 0.001. A significantly higher percentage of patients with MS in the group of patients in chronic renal disease stages III and IV was observed. CONCLUSION: In RTR there is a significant association among MS and inflammation. MS is negatively associated with graft function. The clinical implications of these findings must be evaluated in longitudinal studies.

  14. Hypertension, Chronic Kidney Disease, and Renal Pathology in a Child with Hermansky-Pudlak Syndrome

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    Roberto Gordillo

    2011-01-01

    Full Text Available We report a child with Hermansky-Pudlak Syndrome (HPS and chronic kidney disease (stage II with histological diagnosis of focal segmental glomerulosclerosis (FSGS. A 15-year-old male of Puerto Rico ancestry with history of HPS, hypertension (HTN, asthma, obesity, and chronic kidney disease (CKD stage II presented with new-onset proteinuria without edema. His blood pressure had been controlled, serum creatinine had been 0.9–1.4 mg/dL, and first morning urine protein/creatinine ratio (UPC ranged from 0.2 to 0.38. Due to persistent nonorthostatic proteinuria with CKD, renal biopsy was performed and FSGS (not otherwise specified with chronic diffuse tubulopathy (tubular cytoplasmic droplets and acute tubular injury was reported. Ceroid-like material is known to infiltrate tissues (i.e., lungs, colon, and kidney in HPS, but the reason for the renal insufficiency is unknown. Nonspecific kidney disease and in one adult case IgA nephropathy with ANCA-positive glomerulonephritis have previously been reported in patients with Hermansky-Pudlak syndrome. To our knowledge, we report the first pediatric renal pathology case of HPS associated with CKD. This paper discusses presentation and management of renal disease in HPS.

  15. Hypertension, chronic kidney disease, and renal pathology in a child with hermansky-pudlak syndrome.

    Science.gov (United States)

    Gordillo, Roberto; Del Rio, Marcela; Thomas, David B; Flynn, Joseph T; Woroniecki, Robert P

    2011-01-01

    We report a child with Hermansky-Pudlak Syndrome (HPS) and chronic kidney disease (stage II) with histological diagnosis of focal segmental glomerulosclerosis (FSGS). A 15-year-old male of Puerto Rico ancestry with history of HPS, hypertension (HTN), asthma, obesity, and chronic kidney disease (CKD) stage II presented with new-onset proteinuria without edema. His blood pressure had been controlled, serum creatinine had been 0.9-1.4 mg/dL, and first morning urine protein/creatinine ratio (UPC) ranged from 0.2 to 0.38. Due to persistent nonorthostatic proteinuria with CKD, renal biopsy was performed and FSGS (not otherwise specified) with chronic diffuse tubulopathy (tubular cytoplasmic droplets) and acute tubular injury was reported. Ceroid-like material is known to infiltrate tissues (i.e., lungs, colon, and kidney) in HPS, but the reason for the renal insufficiency is unknown. Nonspecific kidney disease and in one adult case IgA nephropathy with ANCA-positive glomerulonephritis have previously been reported in patients with Hermansky-Pudlak syndrome. To our knowledge, we report the first pediatric renal pathology case of HPS associated with CKD. This paper discusses presentation and management of renal disease in HPS.

  16. The association of nephrotic syndrome and renal vein thrombosis: a clinicopathological analysis of eight pediatric patients.

    Science.gov (United States)

    Tinaztepe, K; Buyan, N; Tinaztepe, B; Akkök, N

    1989-01-01

    Cases with a pathological diagnosis of renal venous thrombosis (RVT) associated with nephrotic syndrome (NS) were studied retrospectively for clinicopathological evaluation. The material consisted of 21 RVT cases which were diagnosed in 2000 consecutive pediatric necropsies, with an overall incidence of about one percent. Eight of the 21 RVT cases were associated with nephrotic syndrome (34%), and this group formed 0.4 percent of the total necropsies in our pediatric center. The glomerulopathies of these nephrotic patients consisted of three cases of Finnish-type congenital NS (FCNS), three cases of renal amyloidosis secondary to familial Mediterranean fever, and two cases of membranoproliferative glomerulonephritis (MPGN). The presence of sepsis associated with disseminated intravascular coagulation, and the morphological age of the thrombi suggested that the RVT was secondary to sepsis in the FCNS cases. In the MPGN and secondary renal amyloidosis cases, the long duration of both the nephrotic state and the administration of diuretics along with glucocorticoid treatment and also the newly formed thrombi without infarction are strong evidences, although not definite, that the RVT developed as a complication of the glomerulopathy. Even though there were no definite clinical criteria for the diagnosis of most of the RVT cases, we would like to emphasize the importance of flank pain, the rapid deterioration of renal functions in a stable nephrotic patient, as well as the hypercoagulable state in the consideration of the development of RVT which indicate the need for appropriate radiological studies for confirmation of this condition during life.

  17. Fanconi syndrome and chronic renal failure in a chronic hepatitis B monoinfected patient treated with tenofovir

    Directory of Open Access Journals (Sweden)

    Pedro Magalhães-Costa

    Full Text Available Tenofovir disoproxil fumarate (TDF is one of the first-line treatment options in chronic hepatitis B (CHB. Despite its efficacy in suppressing viral load and a high resistance barrier, long life maintenance therapy is required. Registration studies demonstrated TDF to be a safe drug. However, post-marketing experience reported cases of serious nephrotoxicity associated with hypophosphatemia, osteomalacia and, even more recently, Fanconi syndrome associated with TDF therapy in CHB monoinfected patients. Here the authors report a case of a 40 year-old male, with a CHB monoinfection, that, three years after TDF therapy, developed a progressive chronic kidney disease with a serious hypophosphatemia and a secondary osteomalacia that was manifested by bone pain and multiple bone fractures. Further investigational analyses unveiled a proximal renal tubular dysfunction, which fulfilled most of the diagnostic criteria for a Fanconi syndrome. After TDF withdrawal and oral supplementation with phosphate and calcitriol, his renal function stabilized (despite not returning to normal, proximal renal tubular dysfunction abnormalities resolved as well as osteomalacia. In conclusion, physicians should be aware that, in CHB monoinfected patients under TDF therapy, serious renal damage is possible and preventable by timely monitoring serum creatinine and phosphate.

  18. Climate Variability and Hemorrhagic Fever with Renal Syndrome Transmission in Northeastern China

    OpenAIRE

    Zhang, Wen-yi; Guo, Wei-Dong; Fang, Li-Qun; Li, Chang-Ping; Bi, Peng; Gregory E Glass; Jiang, Jia-Fu; Sun, Shan-Hua; Qian, Quan; Liu, Wei; Yan, Lei; Yang, Hong; Tong, Shi-Lu; Cao, Wu-Chun

    2010-01-01

    Background The transmission of hemorrhagic fever with renal syndrome (HFRS) is influenced by climatic variables. However, few studies have examined the quantitative relationship between climate variation and HFRS transmission. Objective We examined the potential impact of climate variability on HFRS transmission and developed climate-based forecasting models for HFRS in northeastern China. Methods We obtained data on monthly counts of reported HFRS cases in Elunchun and Molidawahaner counties...

  19. Yohimbine-induced cutaneous drug eruption, progressive renal failure, and lupus-like syndrome.

    Science.gov (United States)

    Sandler, B; Aronson, P

    1993-04-01

    Yohimbine is an indole alkaloid obtained from the yohimbe tree, a common tree in West Africa. We describe a forty-two-year black man in whom a generalized erythrodermic skin eruption, progressive renal failure, and lupus-like syndrome developed following treatment with the drug, yohimbine. A literature review failed to reveal any reported association of these side effects. We review current information on yohimbine's use in male impotence, reported side effects, and its role as a drug allergen.

  20. Thromboembolic complications in nephrotic syndrome. Coagulation abnormalities, renal vein thrombosis, and other conditions.

    Science.gov (United States)

    Llach, F

    1984-11-01

    In patients with nephrotic syndrome, the presence of a hypercoagulable state is thought to give rise to a high incidence of thromboembolic phenomena. Renal vein thrombosis is a common complication in nephrotic patients, mainly in those with membranous nephropathy, and many other types of thromboembolic complications also occur. The mortality rate in nephrotic patients with thromboembolic complications may be significantly increased, with pulmonary emboli likely being the most common cause of death.

  1. Carnitine Profile and Effect of Suppletion in Children with Renal Fanconi Syndrome due to Cystinosis

    OpenAIRE

    Besouw, M.; Cornelissen, E.; Cassiman, D.; Kluijtmans, L.; L. van den Heuvel; Levtchenko, E

    2014-01-01

    Background: Cystinosis is an autosomal recessive disorder marked by intralysosomal cystine accumulation. Patients present with generalized proximal tubular dysfunction called renal Fanconi syndrome. Urinary carnitine loss results in plasma and muscle carnitine deficiency, but no clinical signs of carnitine deficiency have been described. Also, the optimal dose of carnitine supplementation is undefined. This study aimed to determine whether currently recommended carnitine doses result in adequ...

  2. Spectrum of Clinical Presentations in Human Immunodeficiency Virus (HIV) Infected Patients with Renal Disease.

    Science.gov (United States)

    Okafor, U H; Unuigbe, E I; Wokoma, F S

    2011-01-01

    HIV infection is a multiorgan disease with the kidney not spared. A variety of renal syndromes with varying clinical presentations has been reported amongst HIV infected patients. This study aims to highlight the spectrum of clinical presentations in HIV infected patients with renal disease. HIV infected patients presenting at University of Benin Teaching Hospital (UBTH) Benin City were the study population. A total of 383 patients were studied. Their biodata, clinical presentations and laboratory investigations including serum urea, creatinine and albumin, urine protein and creatinine were assessed. Their glomerular filtration rate (GFR) and protein urine excretion were calculated using six equations of modification of diet in renal disease (MDRD) and protein: creatinine ratio respectively. Patients were stratified according to their renal functions into normal, mild, moderate and severe renal function impairment. The data was analysed using statistical software program SPSS Vs 15.0. 53.3% of 383 patients screened had renal function impairment, 40.2% mild, 37.7% moderate and 22.2% severe impairment. Mean age was 35.6±8.3, 36.0±9.9 and 36.3±8.3 years for mild, moderate and severe renal function impairment (RFI) respectively. Easy fatigability was the commonest symptoms occurring in 47.5%, 30.0%, 37.5% and 22.5% of control, mild RFI, moderate RFI and severe RFI subjects respectively (p = 0.568). Oliguria, facial and body swelling occurred more in patients with RFI especially in patients with severe renal impairment. The difference is statistically significant (p = 0.046, 0.041, and 0.033 respectively). Pallor was the commonest clinical sign occurring in 32.5%, 50.0%, 35.0% and 62.5% of control and patients with mild, moderate, and severe RFI respectively; the difference was not statistically significant (p = 0.459). Ascites, facial puffiness and pedal oedema were commoner in patients with RFI especially those with severe RFI. The differences were statistically

  3. Primary retroperitoneal teratoma and crossed fused renal ectopia with turner's syndrome -a case report-

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Yun Jung; Hong, Ki Ung [St. Francisco General Hospital, New York (United States)

    1988-02-15

    In 1938, Turner described a clinical entity in phenotype females characterized by sexual infantilism, congenital webbed neck and cubitus valgus. After then, the occurrence of renal anomalies in patients with Turner's syndrome has been recognized. Associated crossed fused renal ectopia is very rare. Primary retroperitoneal teratoma is also rare and usually during childhood. The authors report a case of primary retroperitoneal teratoma and crossed fused renal ectopia with Turner's syndrome (mosaic type). The clinical, pathological and radiographical findings are reviewed.

  4. Serum TNF-α, sTNFR1, IL-6, IL-8 and IL-10 levels in hemorrhagic fever with renal syndrome.

    Science.gov (United States)

    Kyriakidis, Ioannis; Papa, Anna

    2013-07-01

    It is generally accepted that the pathogenesis of hantavirus infections is the result of virus-mediated host immune response. Hantaviruses, and mainly Dobrava-Belgrade virus, are present in Greece, and cause to humans hemorrhagic fever with renal syndrome (HFRS). Serum IL-6, IL-8, IL-10, TNF-α and sTNFR1 levels were measured in 29 HFRS Greek patients. Significant higher sTNFR1, IL-6, IL-8 and IL-10 levels were observed in severe than in mild/moderate cases, while TNF-α did not seem to be associated with disease severity. Correlations between cytokine levels and their fluctuation over time after onset of the illness, along with comparisons from previously published data on the field, led in building an immune response pattern for HFRS.

  5. Detection of Herpes Simplex Virus DNA in Pseudoexfoliation Syndrome

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    Masoomeh Eghtedari

    2009-06-01

    Full Text Available Background: Pseudoexfoliation syndrome is one of the mostcommon identifiable causes of open angle glaucoma. It hasunknown etiology and pathogenesis. Infection, possibly viral,is one of the proposed pathogenic mechanisms in this condition.In the present study the presence of herpes simplex virus(HSV in specimens of anterior lens capsule of patients withpseudoexfoliation syndrome has been assessed.Methods: The presence of HSV- DNA was searched by usingpolymerase chain reaction method in specimens of anteriorlens capsule (5 mm diameter of 50 patients with pseudoexfoliationsyndrome (study group and 50 age-matchedpatients without the disease (control group who underwentcataract or combined cataract and glaucoma surgery duringa one-year (2006-2007 period in Khalili Hospital, Shiraz,Iran. The results were compared statistically with Chisquaretest and independent samples t test using SPSS software(version 11.5.Results: HSV type I DNA was detected in 18% of the patientsin the study group compared with 2% in the control group (Chisquare test, P = 0.008. The difference between the ranges ofintraocular pressure in the two groups was not statistically significant.Conclusion: The presence of HSV type I DNA suggests thepossible relationship between the virus and pseudoexfoliationsyndrome. It may be a treatable etiology in this multi-factorialdisorder and may help to future management of patients; especiallyto prevent some of the complications in this syndrome.

  6. Early diagnosis of the urofacial syndrome is essential to prevent irreversible renal failure

    Directory of Open Access Journals (Sweden)

    Francisco A. Nicanor

    2005-10-01

    Full Text Available INTRODUCTION: The urofacial or Ochoa syndrome is a rare disease characterized by the presence of functional obstructive uropathy associated with peculiar facial features when patients attempt to smile or laugh. Unfortunately, many of these patients remain without proper diagnosis or adequate treatment due to lack of recognition of the disease. This can ultimately result in upper tract deterioration and eventual renal failure. We present our experience with this rare syndrome. MATERIALS AND METHODS: We identified 3 patients who presented initially with acute renal failure, urinary tract infection (UTI and severe dysfunctional elimination. All patients were thoroughly evaluated, including screening for spinal cord anomalies, and were subsequently diagnosed with urofacial syndrome. RESULTS: At the outset, the two older patients (aged 4 and 9 years presented with the typical facial features when attempting to smile or laugh. One patient in the newborn period presented with urinary and fecal retention and septicemia and, to our knowledge, represents the youngest case of urofacial syndrome reported so far. All patients were evaluated with ultrasonography, renal scan, voiding cystourethrogram (VCUG and urodynamics. Findings included hydronephrosis and a thick-walled, trabeculated bladder with poor compliance and detrusor hypereflexia respectively in each patient. All were subsequently treated with clean intermittent catheterization (CIC, antibiotic prophylaxis and anticholinergic therapy. One patient required appendicovesicostomy for CIC due to discomfort secondary to a sensate urethra. CONCLUSIONS: Our series demonstrates that early recognition of this rare syndrome is necessary to adequately treat and prevent upper tract deterioration in these unique individuals. Although the urofacial is difficult to diagnose in infants, cognizance must be maintained in order to prevent severe subsequent sequalae.

  7. Temporal bone anomalies in the branchio-oto-renal syndrome: detailed computed tomographic and magnetic resonance imaging findings.

    NARCIS (Netherlands)

    Ceruti, S.; Stinckens, C.I.C.; Cremers, C.W.R.J.; Casselman, J.W.

    2002-01-01

    OBJECTIVE: To inventory computed tomographic and magnetic resonance imaging findings in the branchio-oto-renal (BOR) syndrome. STUDY DESIGN: A prospective computed tomographic and magnetic resonance imaging study on a family with the BOR syndrome. SETTING: Department of medical imaging and magnetic

  8. Temporal bone anomalies in the branchio-oto-renal syndrome: detailed computed tomographic and magnetic resonance imaging findings.

    NARCIS (Netherlands)

    Ceruti, S.; Stinckens, C.I.C.; Cremers, C.W.R.J.; Casselman, J.W.

    2002-01-01

    OBJECTIVE: To inventory computed tomographic and magnetic resonance imaging findings in the branchio-oto-renal (BOR) syndrome. STUDY DESIGN: A prospective computed tomographic and magnetic resonance imaging study on a family with the BOR syndrome. SETTING: Department of medical imaging and magnetic

  9. Hemorrhagic Fever with Renal Syndrome (Korean Hemorrhagic Fever).

    Science.gov (United States)

    1986-07-23

    tration. Necropsy tissues include lungs, liver, spleen, kidneys, - b and parotid glands. A portion of each organ was examined immediately by FA for...gens were persisted for 90 days until examined in lung tissues and for about 30 days in liver, kidney spleen and parotid gland. Some amounts of viral...8217 Seoul Spleen -0/4 1/4 :+ /3 0/4 +2/4 +2/4 0/4 1/4 % virus, _ _ _ +++ +++ _ _ HR80/39 - WR-P6, ....... ... ..- 10% Lung + - susp. Parotid

  10. Draft Genome Sequence of White Spot Syndrome Virus Isolated from Cultured Litopenaeus vannamei in Mexico

    Science.gov (United States)

    Rodriguez-Anaya, Libia Zulema; Gonzalez-Galaviz, Jose Reyes; Casillas-Hernandez, Ramón; Lares-Villa, Fernando; Estrada, Karel

    2016-01-01

    The first genome sequence of a Mexican white spot syndrome virus is presented here. White spot syndrome is a shrimp pandemic virus that has devastated production in Mexico for more than 10 years. The availability of this genome will greatly aid epidemiological studies worldwide, contributing to the molecular diagnostic and disease prevention in shrimp farming. PMID:26966222

  11. Burning mouth syndrome associated with varicella zoster virus.

    Science.gov (United States)

    Nagel, Maria A; Gilden, Don

    2016-07-05

    We present two cases of burning mouth syndrome (BMS)-of 8-month duration in a 61-year-old woman and of 2-year duration in a 63-year-old woman-both associated with increased levels of antivaricella zoster virus (VZV) IgM antibodies in serum and with pain that improved with antiviral treatment. Combined with our previous finding of BMS due to herpes simplex virus type 1 (HSV-1) infection, we recommend evaluation of patients with BMS not only for VZV or HSV-1 DNA in the saliva, but also for serum anti-VZV and anti-HSV-1 IgM antibodies. Both infections are treatable with oral antiviral agents.

  12. Clinical study of critical patients with hemorrhagic fever with renal syndrome complicated by acute respiratory distress syndrome.

    Directory of Open Access Journals (Sweden)

    Hong Du

    Full Text Available OBJECTIVES: The aim of this study was to investigate the clinical characteristics and outcomes of critical patients with hemorrhagic fever with renal syndrome (HFRS complicated by acute respiratory distress syndrome (ARDS. MATERIALS AND METHODS: To observe the demographic, epidemiological and clinical characteristics, and to explore the predictive effects for prognosis in laboratory findings, we conducted a detailed retrospective analysis of clinical records for critical patients with HFRS complicated by ARDS, treated at the center for infectious diseases, Tangdu Hospital, between January 2008 and December 2012. RESULTS: A total of 48 critical patients with laboratory confirmed HFRS accompanied by ARDS were enrolled in the study, including 27 survivors and 21 non-survivors, with a fatality rate of 43.75%. Thirty-one individuals (64.6% contracted HFRS between the months of September and December. The non-survivors tended to have lower incidence of overlapping phase (P = 0.025. There were no obvious differences in the needs for mechanical ventilation (MV and renal replacement therapy (RRT, except for the need for vasoactive drugs between the survivors and non-survivors (P = 0.001. The non-survivors were found to have higher frequencies of encephalopathy, refractory shock and multiple organ dysfunction syndrome (MODS, lower incidences of acute renal failure (ARF and secondary hypertension (P<0.05. The non-survivors tended to have lower levels of serum creatinine (Scr (P<0.001 and fibrinogen (Fib (P = 0.003, higher incidences of prolonged prothrombin time (PT (P = 0.006 and activated partial thromboplastin time (APTT (P = 0.020 and higher levels of aspartate aminotransferase (AST (P = 0.015, and the laboratory parameters mentioned above reached statistical significance for predicting prognosis (P<0.05. CONCLUSION: The high mortality rate of critical patients with HFRS complicated by ARDS emphasizes the importance of

  13. Eradication of hepatitis C virus and non-liver-related non-acquired immune deficiency syndrome-related events in human immunodeficiency virus/hepatitis C virus coinfection.

    Science.gov (United States)

    Berenguer, Juan; Rodríguez-Castellano, Elena; Carrero, Ana; Von Wichmann, Miguel A; Montero, Marta; Galindo, María J; Mallolas, Josep; Crespo, Manuel; Téllez, María J; Quereda, Carmen; Sanz, José; Barros, Carlos; Tural, Cristina; Santos, Ignacio; Pulido, Federico; Guardiola, Josep M; Rubio, Rafael; Ortega, Enrique; Montes, María L; Jusdado, Juan J; Gaspar, Gabriel; Esteban, Herminia; Bellón, José M; González-García, Juan

    2017-08-01

    We assessed non-liver-related non-acquired immunodeficiency syndrome (AIDS)-related (NLR-NAR) events and mortality in a cohort of human immunodeficiency virus (HIV)/hepatitis C virus (HCV)-coinfected patients treated with interferon (IFN) and ribavirin (RBV), between 2000 and 2008. The censoring date was May 31, 2014. Cox regression analysis was performed to assess the adjusted hazard rate (HR) of overall death in responders and nonresponders. Fine and Gray regression analysis was conducted to determine the adjusted subhazard rate (sHR) of NLR deaths and NLR-NAR events considering death as the competing risk. The NLR-NAR events analyzed included diabetes mellitus, chronic renal failure, cardiovascular events, NLR-NAR cancer, bone events, and non-AIDS-related infections. The variables for adjustment were age, sex, past AIDS, HIV transmission category, nadir CD4(+) T-cell count, antiretroviral therapy, HIV RNA, liver fibrosis, HCV genotype, and exposure to specific anti-HIV drugs. Of the 1,625 patients included, 592 (36%) had a sustained viral response (SVR). After a median 5-year follow-up, SVR was found to be associated with a significant decrease in the hazard of diabetes mellitus (sHR, 0.57; 95% confidence interval [CI], 0.35-0.93; P = 0.024) and decline in the hazard of chronic renal failure close to the threshold of significance (sHR, 0.43; 95% CI, 0.17-1.09; P = 0.075). Our data suggest that eradication of HCV in coinfected patients is associated not only with a reduction in the frequency of death, HIV progression, and liver-related events, but also with a reduced hazard of diabetes mellitus and possibly of chronic renal failure. These findings argue for the prescription of HCV therapy in coinfected patients regardless of fibrosis stage. (Hepatology 2017;66:344-356). © 2017 The Authors. Hepatology published by Wiley Periodicals, Inc., on behalf of the American Association for the Study of Liver Diseases.

  14. Anterior opercular syndrome induced by Epstein-Barr virus encephalitis.

    Science.gov (United States)

    Matsushima, Takashi; Nishioka, Kenya; Tanaka, Ryota; Yokoyama, Kazumasa; Hattori, Nobutaka

    2016-01-01

    We report a 19-year-old female presenting with fever, drooling, anarthria, and voluntary facial movement disruption, characteristic of anterior opercular syndrome (AOS). Serological examination revealed Epstein-Barr virus (EBV) infection following acute encephalitis with severe ataxia. A single-photon emission computerized tomography (SPECT) examination indicated hypoperfusion in the left perisylvian region, bilateral thalamus, occipital lobe, and cerebellum. This is the first report of AOS related to EBV encephalitis. SPECT was a useful method for detecting the damaged region of the operculum. In addition, AOS is a clinically distinct entity that may help us understand the mechanisms of language circuits within the operculum.

  15. Restless legs syndrome among Saudi end-stage renal disease patients on hemodialysis

    Science.gov (United States)

    Wali, Siraj O.; Alkhouli, Abeer F.

    2015-01-01

    Objectives: To determine the prevalence of restless legs syndrome (RLS) in Saudi patients with end-stage renal disease (ESRD) on hemodialysis. Methods: A cross-sectional study was carried out in 3 hemodialysis centers in Jeddah, Saudi Arabia, between June 2012 and September 2013. All patients were individually interviewed and data was collected on the following demographic features, medical history, laboratory test, the International Restless Legs Syndrome Study Group questionnaire, Epworth Sleepiness Scale (ESS), and Berlin Questionnaire. Results: Three hundred and fifty-five patients were recruited. The prevalence of RLS among ESRD patients was 19.4%, with most patients having moderate to severe disease. The RLS was significantly associated with obstructive sleep apnea (pRestless legs syndrome is common in ESRD patients on hemodialysis, and it is an important source of sleep disruption. In addition to body mass index, Aspirin and anticoagulants may be important risk factors. PMID:25719586

  16. Posterior reversible encephalopathy syndrome in an AIDS patient with acute renal failure and hypertension

    Directory of Open Access Journals (Sweden)

    Olivia Bargiacchi

    2013-03-01

    Full Text Available Introduction: The posterior reversible encephalopathy syndrome (PRES is a neurological entity characterized by magnetic resonance imaging (MRI evidence of bilateral subcortical edema in the occipital regions of the brain. Case report: We report the case of a female patient with AIDS, pulmonary aspergillosis, CMV infection, and acute renal failure due to Clostridium difficile diarrhea. Her clinical course was complicated by seizures and hypertension. MRI findings were consistent with PRES. The patient was treated with anticonvulsants and antihypertensive agents with clinical improvement. Discussion and conclusions: Few cases of PRES in HIV-infected patients have been described, and it is not clear whether HIV infection is a predisposing factor for this syndrome. The article reviews the literature on PRES in HIV and discusses the role of HIV-associated endothelial damage in the pathogenesis of this syndrome.

  17. Renal salt wasting as part of dysautonomia in Guillain-Barre syndrome.

    Science.gov (United States)

    Lenhard, T; Grimm, C; Ringleb, P A

    2011-09-01

    Cerebral salt-wasting syndrome and the syndrome of inappropriate antidiuresis (SIAD) are the most important causes of non-iatrogenic hyponatraemia that can significantly complicate various brain diseases. Salt wasting without an underlying CNS disease may have been disregarded so far by clinicians and has been described as renal salt-wasting (RSW) in patients as drug side effect (eg, cisplatin), in older people with various common diseases (eg, hip fracture, pulmonary infections) and other sporadic conditions. In Guillain-Barré Syndrome (GBS), however, hyponatraemia has been described mainly as SIAD. However, symptoms of hyponatraemia rarely develop in GBS. Here, we report on a woman with GBS with dominant symptoms of dysautonomia and moderate severe hyponatraemia. We could identify RSW as part of the autonomic dysfunction that significantly contributed to disease worsening.

  18. Serotonin syndrome due to fluoxetine and tramadol in renal impaired patient

    Directory of Open Access Journals (Sweden)

    Rajnish Raj

    2014-02-01

    Full Text Available Serotonin syndrome causes confusion or altered mental status; other symptoms include myoclonus, shivering, tremors, diaphoresis, hyperreflexia, incoordination, fever and diarrhoea. Tramadol possesses dual pharmacological effects i.e., a weak opiate agonist at mu, kappa and delta opiate receptors along with reuptake inhibition of norepinephrine and serotonin. Risk associated with tramadol increases when co-administered with serotonergic antidepressants or MAOIs (monoamine oxidase inhibitors and in renal impaired. The incidence of this syndrome is less than 1% as most of the cases remain unreported. The case highlights the fact that interaction between serotonergic agents like fluoxetine and tramadol especially in the presence of co-morbid medical illness can lead to serotonin syndrome. [Int J Basic Clin Pharmacol 2014; 3(1.000: 227-229

  19. Sjogren's syndrome with distal renal tubular acidosis presenting as hypokalaemic paralysis.

    Science.gov (United States)

    Vaidya, Gaurang; Ganeshpure, Swapnil

    2012-10-19

    A young lady with a history of repeated episodes of generalised weakness and fatigue presented to our hospital with similar symptoms and was found to have severe hypokalaemia. She had been previously diagnosed as hypokalaemic periodic paralysis but during this presentation she had also started complaining of the classic sicca-complex of Sjogren's syndrome, which was not present previously. On subsequent investigations she was found to have normal anion-gap metabolic acidosis with positive urine anion gap consistent with the diagnosis of distal renal tubular acidosis (RTA). It was thus concluded that the distal RTA secondary to Sjogren's syndrome was the cause of severe hypokalaemia in our patient. By presenting this case we aim to not only highlight one of the rare presentations of Sjogren's syndrome but also the favourable response of our patient to potassium replacement alone.

  20. Pharmacokinetics of netivudine, a potent anti-varicella zoster virus drug, in patients with renal impairment.

    Science.gov (United States)

    Fillastre, J P; Godin, M; Legallicier, B; Chretien, P; Bidault, R; Gillotin, C; Wooton, R; Posner, J; Peck, R W

    1996-05-01

    The pharmacokinetics of a single oral 200 mg dose of netivudine (1-(beta-D-arabinofuranosyl)-5-(1-propynyl)uracil), a nucleoside analogue under development for use in varicella zoster virus infections, were studied in 12 renal failure (RF) subjects (creatinine clearance 15 +/- 7 mL/min) and 12 age-matched healthy subjects with normal creatinine clearance. Blood and urine samples were collected up to nine days after drug administration. Concentrations of netivudine and of its main metabolite, the pyrimidine base 5-(1-propynyl)uracil (5 PU), were determined by a specific high performance liquid chromatography assay. The mean peak plasma concentrations of netivudine, Tmax, and volume of distribution were not significantly affected by RF. The elimination half-life of netivudine was approximately 15 h in subjects with normal renal function and 60 h in RF patients. Plasma and renal clearances of netivudine were significantly reduced in RF patients and AUC was three to four times higher in these patients. Cmax and AUC of 5 PU were higher in RF patients, and the half-life was also significantly longer. However, the half-life of this metabolite was much lower than that of the parent compound. Tmax and the lag time were similar in the two groups. There were highly significant correlations for netivudine and 5 PU between half-life and creatinine clearance and between renal clearance and creatinine clearance. These findings suggest that netivudine dosage may need to be reduced in patients with severe renal failure, and confirm that formation of the 5 PU is independent of the elimination of netivudine from plasma.

  1. Recurrent atypical hemolytic uremic syndrome associated with factor I mutation in a living related renal transplant recipient.

    Science.gov (United States)

    Chan, Micah R; Thomas, Christie P; Torrealba, Jose R; Djamali, Arjang; Fernandez, Luis A; Nishimura, Carla J; Smith, Richard J H; Samaniego, Millie D

    2009-02-01

    Atypical hemolytic uremic syndrome, or the nondiarrheal form of hemolytic uremic syndrome, is a rare disorder typically classified as familial or sporadic. Recent literature has suggested that approximately 50% of patients have mutations in factor H (CFH), factor I (CFI), or membrane cofactor protein (encoded by CD46). Importantly, results of renal transplantation in patients with mutations in either CFH or CFI are dismal, with recurrent disease leading to graft loss in the majority of cases. We describe an adult renal transplant recipient who developed recurrent hemolytic uremic syndrome 1 month after transplantation. Bidirectional sequencing of CFH, CFI, and CD46 confirmed that the patient was heterozygous for a novel missense mutation, a substitution of a serine reside for a tyrosine residue at amino acid 369, in CFI. This report reemphasizes the importance of screening patients with atypical hemolytic uremic syndrome for mutations in these genes before renal transplantation and shows the challenges in the management of these patients.

  2. Collective immunity of the population from endemic zones of hemorrhagic fever with renal syndrome in Kosovo.

    Science.gov (United States)

    Muçaj, Sefedin; Kabashi, Serbeze; Ahmeti, Salih; Dedushaj, Isuf; Ramadani, Naser; Avsic-Zupanc, Tatjana

    2009-01-01

    Hemorrhagic fever with renal syndrome (HFRS), also known as mice fever is an acute viral zoonosis and it appears in the natural focus after the human contact with Hantaan virus infected mice. The objective (purpose) of this study was to investigate the prevalence of specific antibodies in HFRS, in convalescent persons (collective immunity in endemic hearths). In this project we applied the epidemiological method of studying with retrospective-perspective, the serological method for determination and detecting antibodies from the persons of epidemical focus and statistical methods. The disease diagnosis is based on the epidemiological, clinical and serological records. The collected samples have been sent to referral laboratory in Medical Faculty-Institute of Microbiology Ljubljana for laboratory confirmation. From the results we came to conclusion that in the territory of Republic of Kosovo, the HFRS is still a serious health, economic and biological problem. The lethality rate from HFRS in 1986 was 15.4%, 1986-89 10.8%, from 1995-2006 8.70%. The lowest rates of morbidity, mortality and lethality of HFRS compared with the previous periods of time, prove collective immunity growth in Dukagjini valley. For collective immunity research and to conduct the persistence of antibodies for viral corresponding (relative) antigen, after the disease, the samples were collected in the time period of May-June 2008, with 203 persons that were tested with serological method IIF (Indirect immune fluorescence) from which 187 cases (92.1%) resulted sero-negative and 16 cases (7.9%) resulted sero-positive with HFRS. This proves the collective immunity increase for HFRS. From 13 recovered patients previously diagnosed with HFRS (1986-1989-1995), levels of antibodies were screened in 2008 with IIF. Out of 13 persons, positive antibodies were found in 10 cases, while 3 cases were negative for antibodies (HTN, PUU, and DOB). After 13, 19 and 22 years HTN, PUU and DOB antibodies persisted

  3. Porcine reproductive and respiratory syndrome virus nonstructural protein 2 (nsp2) topology and selective isoform integration in artificial membranes

    Science.gov (United States)

    Membrane modification of host subcellular compartments is critical to the replication of many RNA viruses. Enveloped viruses additionally require the ability to requisition cellular membranes during egress for the development of infectious progeny. Porcine reproductive and respiratory syndrome virus...

  4. 肾综合征出血热和汉坦病毒肺综合征研究进展%Hantavirus pulmonary syndrome and hemorrhagic fever with renal syndrome

    Institute of Scientific and Technical Information of China (English)

    白雪帆; 王平忠

    2011-01-01

    肾综合征出血热(hemorrhagic fever with renal syndrome,HFRS)和汉坦病毒肺综合征(hantavirus pulmonary syndrome,HPS)均是由汉坦病毒属病毒(hantaviruses)引起的急性传染病.HFRS的病原体——汉滩病毒(Hantaan virus,HTNV)、汉城病毒(SEOV)、普马拉病毒(PUUV)和多布拉伐病毒(DOBV)等主要分布于有着几千年文明历史的欧亚大陆,国际上称为旧世界汉坦病毒(hantavirus in old world);

  5. Smoking and renal function in people living with human immunodeficiency virus: a Danish nationwide cohort study

    Directory of Open Access Journals (Sweden)

    Ahlström MG

    2015-08-01

    Full Text Available Magnus Glindvad Ahlström,1 Bo Feldt-Rasmussen,2 Rebecca Legarth,1 Gitte Kronborg,3 Court Pedersen,4 Carsten Schade Larsen,5 Jan Gerstoft,1 Niels Obel1 1Department of Infectious Diseases, 2Department of Nephrology, Copenhagen University Hospital, Rigshospitalet, Copenhagen, 3Department of Infectious Diseases, Copenhagen University Hospital, Hvidovre, 4Department of Infectious Diseases, Odense University Hospital, Odense, 5Department of Infectious Diseases, Aarhus University Hospital, Skejby, Aarhus, Denmark Introduction: Smoking is a main risk factor for morbidity and mortality in people living with human immunodeficiency virus (PLHIV, but its potential association with renal impairment remains to be established. Methods: We did a nationwide population-based cohort study in Danish PLHIV to evaluate the association between smoking status and 1 overall renal function and risk of chronic kidney disease (CKD, 2 risk of any renal replacement therapy (aRRT, and 3 mortality following aRRT. We calculated estimated creatinine clearance using the Cockcroft–Gault equation (CG-CrCl, and evaluated renal function graphically. We calculated cumulative incidence of CKD (defined as two consecutive CG-CrCls of ≤60 mL/min, ≥3 months apart and aRRT and used Cox regression models to calculate incidence rate ratios (IRRs for risk of CKD, aRRT, and mortality rate ratios (MRRs following aRRT. Results: From the Danish HIV Cohort Study, we identified 1,475 never smokers, 768 previous smokers, and 2,272 current smokers. During study period, we observed no association of smoking status with overall renal function. Previous and current smoking was not associated with increased risk of CKD (adjusted IRR: 1.1, 95% confidence interval [CI]: 0.7–1.7; adjusted IRR: 1.3, 95% CI: 0.9–1.8 or aRRT (adjusted IRR: 0.8, 95% CI: 0.4–1.7; adjusted IRR: 0.9, 95% CI: 0.5–1.7. Mortality following aRRT was high in PLHIV and increased in smokers vs never smokers (adjusted MRR: 3

  6. Thrombo-embolic renal infarction in a case of mid-ventricular takotsubo syndrome.

    Science.gov (United States)

    Y-Hassan, Shams; Shahgaldi, Kambiz

    2011-01-01

    Thrombo-embolism is one of the serious complications of takotsubo syndrome (TS). It typically occurs in the classical mid-apical left ventricular ballooning form of TS. This complication has not been reported in cases of left mid-ventricular ballooning type of TS. We describe a 67-year-old woman who presented with 2-3 days of increasing signs and symptoms of heart failure. Echocardiography showed marked hypokinesia/akinesia in the mid-anterior, mid-anterolateral, and mid-inferior wall of the left ventricle and mild hypokinesia in the apical region. There was also hypokinesia of the mid and apical parts of the right ventricle. One day after admission, she developed acute left-sided renal infarction. Left ventriculography and coronary angiography 3 days after admission showed typical left mid-ventricular ballooning with no identifiable coronary lesion. Follow-up echocardiography showed complete resolution of left and right ventricular dysfunction. Takotsubo syndrome with right ventricular involvement complicated with heart failure and left renal embolic infarction was diagnosed. The mechanism of left renal embolic infarction is discussed.

  7. Renal infarction as a presentation of Austrian syndrome: thromboembolic phenomenon of pneumococcal endocarditis.

    Science.gov (United States)

    Mankongpaisarnrung, Charoen; Soontrapa, Suthipong; Nantsupawat, Teerapat; Desai, Vipul; Nugent, Kenneth

    2012-09-01

    A 52-year-old unvaccinated and splenectomized man presented with fever, altered sensorium, bilateral flank pain and chest discomfort accompanied with paroxysmal atrial fibrillation with a rapid ventricular response. An abdominal computed tomography scan was performed, which revealed a right renal infarct and splenosis. Transthoracic echocardiography was performed, which demonstrated an echodense structure on the mitral valve with mitral regurgitation and a vegetation on the aortic valve with aortic regurgitation. Subsequently, he was found to have pneumococcal infective endocarditis, pneumococcal pneumonia and bacterial meningitis, namely Austrian syndrome. He underwent an early aortic valve and mitral valve repair but still had a poor clinical outcome. Renal infarction has a mortality of approximately 13.2%, which is strongly influenced by the underlying diseases and infectious complications. Medical and surgical treatment initiated in a timely manner is often inadequate. The authors report the first case of Austrian syndrome presenting with renal infarction as a clue to an embolic event associated with infective endocarditis in this study.

  8. Pediatric Sjogren syndrome with distal renal tubular acidosis and autoimmune hypothyroidism: an uncommon association.

    Science.gov (United States)

    Agarwal, Amit; Kumar, Pradeep; Gupta, Nomeeta

    2015-11-01

    A 14-year-old female came with the history of sudden onset weakness; during work up, she was found to have hyperchloremic metabolic acidosis with normal anion gap and normal renal function suggesting the possibility of renal tubular acidosis (RTA). On further evaluation of RTA, she had positive antinuclear antibody, anti-Ro, and anti-La antibodies. On nuclear scan of salivary glands, her left parotid gland was nonfunctional. Her parotid biopsy revealed dilated interlobular ducts engulfed by lymphoid cells. She also had autoimmune hypothyroidism as suggested by raised TSH and positive anti-TPO antibodies. At admission, her serum potassium levels were low and she was treated with intravenous potassium chloride. After she recovered from acute hypokalemic paralysis, she was started on oral potassium citrate along with phosphate supplements, hydroxychloroquine, oral prednisolone and thyroxine supplements. Over the next 6 months, she has significant reduction in the dosage of potassium, bicarbonate and phosphate and gained 3 kg of weight and 3.5 cm of height. As primary Sjogren syndrome itself is rare in pediatric population and its association with renal tubular acidosis is even rarer, we suggest considering Sjogren syndrome as a differential diagnosis during the RTA work-up is worth trying.

  9. Risk factors for infection of sow herds with porcine reproductive and respiratory syndrome (PRRS) virus

    DEFF Research Database (Denmark)

    Mortensen, Sten; Stryhn, Henrik; Søgaard, Rikke

    2002-01-01

    In 1992, the porcine reproductive and respiratory syndrome virus (PRRSV) of European type (PRRSV-EU) was introduced in Denmark. By 1996, the virus had spread to approximately 25% of the Danish herds. In January 1996, a modified-live vaccine based on the American type of the virus (PRRSV-US) was u...

  10. Effects of a porcine reproductive and respiratory syndrome virus infection on the development of the immune response against pseudorabies virus

    NARCIS (Netherlands)

    Bruin, de M.G.M.; Samsom, J.N.; Voermans, J.J.M.; Rooij, van E.M.A.; Visser, de Y.E.; Bianchi, A.T.J.

    2000-01-01

    The aim of this study was to investigate the effects of a porcine reproductive and respiratory syndrome virus (PRRSV) infection on the development of the immune response after pseudorabies virus (PRV) vaccination in pigs. Pigs were intranasally inoculated with the European PRRSV strain, Lelystad vir

  11. Pulmonary-renal syndrome in systemic sclerosis: a report of three cases and review of the literature.

    Science.gov (United States)

    Naniwa, Taio; Banno, Shogo; Sugiura, Yoshiki; Yokota, Kaori; Oosawa, Tomoyo; Maeda, Shinji; Hayami, Yoshihito; Takahashi, Nobuyuki; Ueda, Ryuzo; Matsumoto, Yoshifuji

    2007-01-01

    We describe three cases of acute renal failure with diffuse alveolar hemorrhage, which is designated pulmonary-renal syndrome (PRS), in systemic sclerosis (SSc) and review the literature to better define this rare but severe complication of SSc. The clinical course of three SSc patients with acute renal failure and concomitant diffuse alveolar hemorrhage are reported, and the literature published between 1967 and 2005 is reviewed following a PubMed search. Including our cases, a total of 19 SSc patients with acute renal failure and concomitant diffuse alveolar hemorrhage have been reported. Pulmonary-renal syndrome developing in SSc patients can be categorized clinicopathologically into three entities: PRS with thrombotic microangiopathy, PRS with small vessel vasculitides accompanied with SSc, and d-penicillamine-induced Goodpasture-like syndrome. Patients with scleroderma PRS with thrombotic microangiopathy, to which group our all patients belong, often developed diffuse alveolar hemorrhage after receiving high-dose corticosteroid therapy. Pulmonary-renal syndrome is a fatal complication of SSc and results from different pathogenic processes. Prompt differential diagnosis between the subsets is critical, because therapeutic strategy may differ in the use of high-dose corticosteroid and plasma exchange between the subsets of PRS. Clinical courses of the patients with PRS with thrombotic microangiopathy suggest that high-dose corticosteroid therapy is a trigger of diffuse alveolar hemorrhage in patients with diffuse SSc with signs of thrombotic microangiopathy.

  12. Review of renal tumors associated with Birt-Hogg-Dubé syndrome with focus on clinical and pathobiological aspects.

    Science.gov (United States)

    Kuroda, N; Furuya, M; Nagashima, Y; Gotohda, H; Kawakami, F; Moritani, S; Ota, S; Hora, M; Michal, M; Hes, O; Nakatani, Y

    2014-06-01

    Birt-Hogg-Dubé syndrome (BHDS) is an autosomal dominant inherited disorder characterized by clinical features of skin lesions, pulmonary lesions and renal tumor. The gene responsible for this syndrome is located on chromosome 17p11.2 and designated as FLCN. In this article, we review renal tumors associated with BHDS with a focus on clinical and pathobiological aspects. Renal tumors often occur multifocally or bilaterally in the imaging analyses or gross examination. Histological examination of renal tumors includes a variety of subtypes such as hybrid oncocytic tumor, chromophobe renal cell carcinoma (RCC), oncocytoma, clear cell RCC and papillary RCC. The histologic discordance in multiple tumors seems to be characteristic of this syndrome. Oncocytosis is observed histologically in about half of the cases. Several investigations have elucidated that folliculin may be involved in the mammalian target of rapamycin (mTOR) pathway recently. Renal tumors composed of clear cells may behave in an aggressive fashion. However, renal tumors including hybrid oncocytic tumor, chromophobe RCC and oncocytoma behave mostly in an indolent fashion.

  13. [Hepatorenal syndrome in decompensated cirrhosis : A special form of acute renal failure].

    Science.gov (United States)

    Lenz, K; Buder, R; Lohr, G; Piringer, P; Voglmayr, M

    2016-06-01

    Renal failure is a serious complication in patients with advanced cirrhosis. It occurs in about 20 % of patients hospitalized with cirrhosis. In about 70 % it is caused by prerenal failure, and in 30 % it is due to intrarenal causes. In about 70 % of patients with rperenal failure, renal function can be restored with fluid replacement, but the remaining 30 % are unresponsive to volume expansion. Minor increase in serum creatinine have been shown to be clinically relevant and can adversely affect survival. Therefore early efforts should be made to avoid precipitation of renal failure.Hepatorenal syndrome (HRS) is a  fully reversible impairment of renal function in patients with cirrhosis unresponsive to volume expansion characterized by an acute progressive decrease in kidney function (serumcreatinin > 1,5 mg/dl) - type 1 HRS, whereas type 2 HRS features a decrease in kidney function over a long time, mostly in patients with refractory ascites. Therapy with vasoconstrictors like terlipressin to reverse splanchnic vasodilation, together with albumin is effective in 30-50 % of patients with HRS 1 and improves survival. The only effective longterm therapy is livertransplantation. An improvement of kidney fuction before transplantation is associated with a better outcome and posttransplant kidney function.

  14. Risk factors for poor renal prognosis in children with hemolytic uremic syndrome.

    Science.gov (United States)

    Gianviti, Alessandra; Tozzi, Alberto E; De Petris, Laura; Caprioli, Alfredo; Ravà, Lucilla; Edefonti, Alberto; Ardissino, Gianluigi; Montini, Giovanni; Zacchello, Graziella; Ferretti, Alfonso; Pecoraro, Carmine; De Palo, Tommaso; Caringella, Angela; Gaido, Maurizio; Coppo, Rosanna; Perfumo, Francesco; Miglietti, Nunzia; Ratsche, Ilse; Penza, Rosa; Capasso, Giovambattista; Maringhini, Silvio; Li Volti, Salvatore; Setzu, Carmen; Pennesi, Marco; Bettinelli, Alberto; Peratoner, Leopoldo; Pela, Ivana; Salvaggio, Elio; Lama, Giuliana; Maffei, Salvatore; Rizzoni, Gianfranco

    2003-12-01

    Many factors have been proposed as predictors of poor renal prognosis in children with hemolytic uremic syndrome (HUS), but their role is still controversial. Our aim was to detect the most reliable early predictors of poor renal prognosis to promptly identify children at major risk of bad outcome who could eventually benefit from early specific treatments, such as plasmapheresis. Prognostic factors identifiable at onset of HUS were evaluated by survival analysis and a proportional hazard model. These included age at onset, prodromal diarrhea (D), leukocyte count, central nervous system (CNS) involvement, and evidence of Shiga toxin-producing Escherichia coli (STEC) infection. Three hundred and eighty-seven HUS cases were reported; 276 were investigated for STEC infection and 189 (68%) proved positive. Age at onset, leukocyte count, and CNS involvement were not associated with the time to recovery. Absence of prodromal D and lack of evidence of STEC infection were independently associated with a poor renal prognosis; only 34% of patients D(-)STEC(- )recovered normal renal function compared with 65%-76% of D(+)STEC(+), D(+)STEC(-) and D(-)STEC(+ )patients. In conclusion, absence of both D and evidence of STEC infection are needed to identify patients with HUS and worst prognosis, while D(-) but STEC(+) patients have a significantly better prognosis.

  15. Lowe Syndrome (Oculo-cerebro-renal Syndrome of Lowe: A Case Report from Eastern India

    Directory of Open Access Journals (Sweden)

    Dipankar Das

    2014-01-01

    Full Text Available Lowe syndrome (the oculocerebrorenal syndrome of Lowe, OCRL is a rare X-linked recessive metabolic disorder that primarily affects eyes, kidneys and brain. It is caused by the deficiency of enzyme phosphatidylinositol 4, 5-bisphosphate 5-phosphatase. The gene coding for this enzyme, OCRL1 and mutations in it are responsible to cause Lowe Syndrome. We report a 6 years old boy from Eastern India, with Lowe Syndrome. Diagnosis was suggested by typical features in the MRI of the brain along with other clinical feature and investigation.

  16. Obesity, metabolic syndrome and diabetes mellitus after renal transplantation: prevention and treatment.

    Science.gov (United States)

    Wissing, Karl Martin; Pipeleers, Lissa

    2014-04-01

    The prevalence of the metabolic syndrome in dialysis patients is high and further increases after transplantation due to weight gain and the detrimental metabolic effects of immunosuppressive drugs. Corticosteroids cause insulin resistance, hyperlipidemia, abnormal glucose metabolism and arterial hypertension. The calcineurin inhibitor tacrolimus is diabetogenic by inhibiting insulin secretion, whereas cyclosporine causes hypertension and increases cholesterol levels. Mtor antagonists are responsible for hyperlipidemia and abnormal glucose metabolism by mechanisms that also implicate insulin resistance. The metabolic syndrome in transplant recipients has numerous detrimental effects such as increasing the risk of new onset diabetes, cardiovascular disease events and patient death. In addition, it has also been linked with accelerated loss of graft function, proteinuria and ultimately graft loss. Prevention and management of the metabolic syndrome are based on increasing physical activity, promotion of weight loss and control of cardiovascular risk factors. Bariatric surgery before or after renal transplantation in patients with body mass index >35 kg/m(2) is an option but its long term effects on graft and patient survival have not been investigated. Steroid withdrawal and replacement of tacrolimus with cyclosporine facilitate control of diabetes, whereas replacement of cyclosporine and mtor antagonists can improve hyperlipidemia. The new costimulation inhibitor belatacept has potent immunosuppressive properties without metabolic adverse effects and will be an important component of immunosuppressive regimens with better metabolic risk profile. Medical treatment of cardiovascular risk factors has to take potential drug interactions with immunosuppressive medication and drug accumulation due to renal insufficiency into account. Copyright © 2014 Elsevier Inc. All rights reserved.

  17. Spontaneous Pelvic Rupture as a Result of Renal Colic in a Patient with Klinefelter Syndrome

    Directory of Open Access Journals (Sweden)

    Sergey Reva

    2013-01-01

    Full Text Available We present the case of a young man with Klinefelter syndrome, who was admitted to our clinic with renal colic. Shortly after admittance, spontaneous decrease in pain has occurred. Ultrasound and intravenous contrast computed tomography were performed, which showed the evidence of urine extravasation at the level of left renal pelvis and a 4 mm stone in the lower third of the left ureter. The management with a double-J ureteric stent for three weeks was successful. Then, the stent was removed and computed tomography confirmed the absence of urine extravasation. We also analyze the literature related to this case and discuss the main mechanisms of collecting system rupture.

  18. RENOVASCULAR HYPERTENSION DUE TO RENAL ARTERY STENOSIS IN KLIPPEL-FEIL SYNDROME

    Directory of Open Access Journals (Sweden)

    Foyaca-Sibat H. MD.

    2003-01-01

    Full Text Available ABSTRACT We report one patient with Klippel-Feil (KFS syndrome, other associated anomalies, uncontrolled arterial hypertension, and renal artery stenosis. Because this patient underwent for surgical revascularization with unsuccessful result, all proposed way of treatments are revised, and we have hypothesized that probably for patients with KFS and unilateral renal artery stenosis, medical treatment with ACE inhibitors can provide more benefits than surgical revascularization or percutaneous transluminal angioplasty. We considered that those patients should be manage by a team of medical doctors being aware of their common associated anomalies, identifying all of them when it is possible then, making an integral evaluation of the each individual situation for establishing their medical priorities in order, and then address its treatments accordingly. If at this stage any surgical treatment is required, is important to bring those problems to the anesthesiologist’s attention for a very careful manipulation of the neck and head during induction of anesthesia. The final results will be strongly related with the capacity of management of the underlying cardio-respiratory, renal, skeletal, urogenital, and nervous system problems. . We also propose the term of Klippel-Feil syndrome "Plus" for those patients with cervical vertebral fusion and many other associated deformities rather than to add new eponyms to the long list that already exist. _____________ RESUMEN: HIPERTENSION RENOVASCULAR DEBIDO A ESTENOSIS DE LA ARTERIA RENAL EN EL SÍNDROME DE KLIPPEL-FEIL Reportamos un paciente afectado por un syndrome de Klippel-Feil, otras anormalias congenitas, hipertension arterial incontrolada y una estenosis unilateral de la arteria renal. Este paciente fue sometido a un tratamiento quirÚrgico de la estenosis de la arteria renal, cuyos resultados fueron no satisfactorios por lo que revisamos todas las alternativas de tratamiento para la estenosis

  19. Distal renal tubular acidosis and quadriparaesis in Sjögren′s syndrome: A cunning congregate

    Directory of Open Access Journals (Sweden)

    Arundhati G Diwan

    2014-01-01

    Full Text Available Sjögren′s syndrome (SS is a chronic autoimmune disease, chiefly affecting the exocrine glandular function of salivary glands and lacrimal glands. Rarely, it involves the kidneys, central and peripheral nervous system, muscloskeletal apparatus and lungs. We report a rare constellation of SS with distal renal tubular acidosis and quadriparaesis in a young female. History of quadriparaesis was acute, with rapid progression. Supplementary treatment for severe hypokalemia was instituted at the earliest, lest the patient develop respiratory muscle weakness. Concomitantly, metabolic acidosis with alkaline urine was suspected and subsequently investigated. Eventually, this was attributed to impaired renal acidification of urine in the distal tubules. History of dryness of eyes and mouth since 6 months justified salivary gland biopsy. The results yielded a lymphocytic infiltrative pathology strongly favoring SS. The patient benefited from prompt potassium replacement therapy and had complete resolution over the next week. Supportive treatment for predictable manifestations was continued along with potassium supplements.

  20. [Meningoencephalitis caused by West Nile virus in a renal transplant recipient].

    Science.gov (United States)

    Ertilav, Muhittin; Ozkul, Aykut; Zeytinoğlu, Ayşın; Sen, Sait; Sipahi, Savaş; Töz, Hüseyin; Kitiş, Omer; Eraslan, Cenk

    2014-10-01

    West Nile virus (WNV) infection which is asymptomatic or mild in normal population, it may cause serious clinical conditions leading to death in eldery and immunosupressed patients. The virus is mainly transmitted by mosquito bites, however transfusion, transplantation, transplasental and nosocomial ways have also been reported to be responsible for viral transmission. It is known that WNV may cause life-threatining conditions such as central nervous system (CNS) infections especially in bone marrow and solid organ transplant recipients. In this report, the first case of WNV encephalitis in an immunosuppressed patient with renal transplant in Turkey was presented. A 25-year-old male patient admitted to our hospital with the complaints of generalized myalgia, nausea and vomiting, after the 24. day of renal transplantation from a live donor. Since he developed diffuse tonic clonic seizures during his follow up, he was diagnosed as meningoencephalitis with the results of cranial magnetic resonance imaging (MR) and cerebrospinal fluid (CSF) biochemistry. Bacterial and fungal cultures of blood and CSF yielded negative results. CMV antigenemia test and CMV IgM in blood, and nucleic acid tests for CMV, EBV, HSV-1/2, VZV, HHV-6, enterovirus and parvovirus in CSF were also negative. However, WNV RNA was detected in CSF by an in-house reverse transcriptase (RT) nested PCR method. The sequence analysis (GenBank BLAST) of the virus showed that it had 99% similarity with Lineage-1 WNV strains. To define the transmission way of the virus to the recipient, WNV-RNA was searched in the renal biopsy sample and found negative by RT nested PCR. The clinical condition of the patient was improved with supportive therapy and by the de-escalation of immunosuppressive drugs [Mycophenolate mofetil (MMF; 1 g/day), cyclosporin (1 mg/kg/day)]. However WNV meningoencephalitis recurred one month later. The patient presented with fever, myalgia, confusions, leukocytosis, anemia, and repeating WNV

  1. Valsartan attenuates cardiac and renal hypertrophy in rats with experimental cardiorenal syndrome possibly through down-regulating galectin-3 signaling.

    Science.gov (United States)

    Zhang, M-J; Gu, Y; Wang, H; Zhu, P-F; Liu, X-Y; Wu, J

    2016-01-01

    Aortocaval fistula (AV) induced chronic volume overload in rats with preexisting mild renal dysfunction (right kidney remove: UNX) could mimic the type 4 cardiorenal syndrome (CRS): chronic renocardiac syndrome. Galectin-3, a β-galactoside binding lectin, is an emerging biomarker in cardiovascular as well as renal diseases. We observed the impact of valsartan on cardiac and renal hypertrophy and galectin-3 changes in this model. Adult male Sprague-Dawley (SD) rats (200-250 g) were divided into S (Sham, n = 7), M (UNX+AV, n = 7) and M+V (UNX+AV+valsartan, n = 7) groups. Eight weeks later, cardiac function was measured by echocardiography. Renal outcome was measured by glomerular filtration rate, effective renal plasma flow, renal blood flow and 24 hours albuminuria. Immunohistochemistry and real-time PCR were used to evaluate the expressions of galectin-3 in heart and renal. Cardiac hypertrophy and renal hypertrophy as well as cardiac enlargement were evidenced in this AV shunt induced chronic volume overload rat model with preexisting mild renal dysfunction. Cardiac and renal hypertrophy were significantly attenuated but cardiac enlargement was unaffected by valsartan independent of its blood pressure lowering effect. 24 hours urine albumin was significantly increased, which was significantly reduced by valsartan in this model. Immunohistochemistry and real-time PCR evidenced significantly up-regulated galectin-3 expression in heart and kidney and borderline increased myocardial collagen I expression, which tended to be lower post valsartan treatment. Up-regulated galectin-3 signaling might also be involved in the pathogenesis in this CRS model. The beneficial effects of valsartan in terms of attenuating cardiac and renal hypertrophy and reducing 24 hours albumin in this model might partly be mediated through down-regulating galectin-3 signal pathway.

  2. Hepatitis B virus X protein promotes renal epithelial-mesenchymal transition in human renal proximal tubule epithelial cells through the activation of NF-κB.

    Science.gov (United States)

    Li, Mei; Hu, Liping; Zhu, Fengxin; Zhou, Zhangmei; Tian, Jianwei; Ai, Jun

    2016-08-01

    Hepatitis B virus (HBV)-associated glomerulo-nephritis is the most common extra-hepatic disorder occurring with hepatitis B virus infection. In the present study, we hypothesized that HBV X protein (HBx) may play a critical role in renal interstitial fibrosis, as HBx has been shown to induce epithelial-mesenchymal transition (EMT) in renal cells. For this purpose, we successfully transfected HBx plasmid into human renal proximal tubule epithelial cells (HK-2 cells). We found that transfection with HBx plasmid significantly downregulated E-cadherin expression and upregulated α-smooth muscle actin, collagen I and fibronectin expression in a time- and concentration-dependent manner (at the lower concentrations and earlier time points). HBx also increased nuclear factor-κB (NF-κB) phosphorylation in a time- and concentration-dependent manner (again at the lower concentrations and earlier time points); however, it did not alter the phosphorylation of Smad2, Smad3, p38, phosphoinositide 3-kinase (PI3K) or extracellular signal-regulated kinase (ERK). Thus, the findings of this study demonstrate that HBx promotes EMT in renal HK-2 cells, and the potential underlying mechanisms may involve the activation of the NF-κB signaling pathway.

  3. Immune reconstitution syndrome in a human immunodeficiency virus infected child due to giardiasis leading to shock

    Directory of Open Access Journals (Sweden)

    Sneha Nandy

    2015-01-01

    Full Text Available Human immunodeficiency virus (HIV-associated immune reconstitution inflammatory syndrome has been reported in association with tuberculosis, herpes zoster (shingles, Cryptococcus neoformans, Kaposi′s sarcoma, Pneumocystis pneumonia, hepatitis B virus, hepatitis C virus, herpes simplex virus, Histoplasma capsulatum, human papillomavirus, and Cytomegalovirus. However, it has never been documented with giardiasis. We present a 7-year-old HIV infected girl who developed diarrhea and shock following the initiation of antiretroviral therapy, and her stool showed the presence of giardiasis.

  4. Uterus didelphys with obstructed hemivagina and ipsilateral renal anomaly (OHVIRA syndrome: A case report

    Directory of Open Access Journals (Sweden)

    Mohamed Amin El-Gohary

    2014-09-01

    Full Text Available Uterus didelphys with obstructed hemivagina and ipsilateral renal anomaly (OHVIRA syndrome is a rare congenital urogenital anomaly. We report a case of a 14-year-old female who presented with acute lower abdominal pain and tender pelvic mass. The initial diagnosis of twisted left ovarian cyst was confirmed by abdominal ultrasonography (US, which showed an associated absent left kidney. The findings at laparoscopy were that of uterus didelphys with obstructed left hemivagina. Parents were concerned about hymnal integrity and refused hemivaginal septal resection.

  5. Central Diabetes Insipidus and Cisplatin-Induced Renal Salt Wasting Syndrome: A Challenging Combination.

    Science.gov (United States)

    Cortina, Gerard; Hansford, Jordan R; Duke, Trevor

    2016-05-01

    We describe a 2-year-old female with a suprasellar primitive neuroectodermal tumor and central diabetes insipidus (DI) who developed polyuria with natriuresis and subsequent hyponatremia 36 hr after cisplatin administration. The marked urinary losses of sodium in combination with a negative sodium balance led to the diagnosis of cisplatin-induced renal salt wasting syndrome (RSWS). The subsequent clinical management is very challenging. Four weeks later she was discharged from ICU without neurological sequela. The combination of cisplatin-induced RSWS with DI can be confusing and needs careful clinical assessment as inaccurate diagnosis and management can result in increased neurological injury.

  6. Renal artery thrombosis and hypertension in a 13 year old girl with antiphospholipid syndrome.

    Science.gov (United States)

    Ostuni, P A; Lazzarin, P; Pengo, V; Ruffatti, A; Schiavon, F; Gambari, P

    1990-01-01

    The case of a 13 year old girl with renal artery thrombosis and hypertension is described. A cerebrovascular accident and a probable occlusion of the superior mesenteric artery also occurred. Very high levels of 'lupus anticoagulant', anticardiolipin antibodies as well as false positive Venereal Disease Research Laboratory tests were repeatedly shown. Moreover, the patient fulfilled at least four classification criteria for systemic lupus erythematosus, but only a slight positivity for antinucleolar antibodies was present. The striking relation between antiphospholipid antibody levels and clinical events and the treatment of this complex syndrome are discussed. Images PMID:2108619

  7. Severe hypophosphatemic osteomalacia with Fanconi syndrome, renal tubular acidosis, vitamin D deficiency and primary biliary cirrhosis.

    Science.gov (United States)

    Bando, Hironori; Hashimoto, Naoko; Hirota, Yushi; Sakaguchi, Kazuhiko; Hisa, Itoko; Inoue, Yoshifumi; Imanishi, Yasuo; Seino, Susumu; Kaji, Hiroshi

    2009-01-01

    A 49-year-old woman was admitted to our hospital for back pain with marked thoracic and extremity deformities leading to bed-rest for three years. She was diagnosed with hypophosphatemic osteomalacia based on her symptoms, X-ray and bone scintigram, high serum alkaline phosphatase level, and low serum levels of both phosphorus and 1,25 dihydroxyvitamin D(3) with inhibition of phosphorus reabsorption. Fanconi syndrome with renal tubular acidosis, vitamin D deficiency and primary biliary cirrhosis were related to the pathogenesis of osteomalacia in this case. Several causal diseases may be concomitantly responsible for acceleration of the severity of osteomalacia in this patient.

  8. Molecular-genetic risk assessement of determining angiotensin-converting enzyme hyperactivity in hemorrhagic fever with renal syndrome

    Directory of Open Access Journals (Sweden)

    Ildar R. Minniakhmetov

    2012-09-01

    Full Text Available The present study was designed to investigate changes in angiotensin-converting enzyme (ACE blood activity and angiotensin II type 1 receptor gene polymorphism as a possible disease predictor in hemorrhagic fever with renal syndrome (HFRS. Four hundred and nine patients (346 males and 63 females with HFRS serologic confirmation were enrolled in the study. Their age ranged from 15 to 65 years. ACE blood activity was assessed kinetically using the Bühlmann (Switzerland kit. Peripheral blood genomic DNA was isolated by a phenol-chloroform extraction. The genotyping of DNA loci was done using a polymerase chain reaction of DNA synthesis. Statistically, ACE blood activity was significantly higher throughout the entire HFRS course with diverse severity apart from the feverish phase of moderate-to-severe uncomplicated disease forms. *A1166 and *C1166 alleles, *A1166/*A1166 and *C1166/*C1166 genotypes of angiotensin II type 1 receptor gene were not associated with HFRS severity. The results of this study indicate that high ACE activity has not adaptive characteristics due to abnormalities in angiotensin II reception. It is an adequate metabolic response of the body to endotheliotropic virus activity.

  9. Shock Wave Lithotripsy Does Not Impair Renal Function in a Swine Model of Metabolic Syndrome

    Science.gov (United States)

    Johnson, Cynthia D.; Connors, Bret A.; Evan, Andrew P.; Phillips, Carrie L.; Liu, Ziyue

    2015-01-01

    Abstract Purpose: To determine whether shock wave lithotripsy (SWL) may be a risk factor for renal functional impairment in a swine model of metabolic syndrome (MetS). Materials and Methods: Nine-month-old female Ossabaw pigs were fed an excess calorie atherogenic diet to induce MetS. At 15 months of age, the MetS pigs were treated with 2000 SWs or an overtreatment dose of 4000 SWs targeted at the upper pole calyx of the left kidney (24 kV at 120 SWs/min using the unmodified Dornier HM3 lithotripter; n=5–6 per treatment group). Serum creatinine (Cr) and blood urea nitrogen (BUN) levels were measured in conscious pigs before and ∼60 days after SWL to provide a qualitative assessment of how well both kidneys were filtering (glomerular filtration rate [GFR]). Bilateral renal function was assessed at ∼65 days post-SWL in anesthetized pigs with GFR and effective renal plasma flow (ERPF) quantified by the renal clearance of inulin and para-amino hippurate, respectively. Results: Cr and BUN values were within normal limits before SWL and remained unchanged after lithotripsy in both the 2000 SW- and 4000 SW-treated pigs. GFR and ERPF of kidneys treated with SWL at either SW dose were similar to the contralateral nontreated kidney. Chronic histological changes in the SW-treated pole of the kidney included interstitial fibrosis, sclerotic glomeruli, and dilated and atrophic tubules. Conclusions: Our results are consistent with the view that a single SWL session does not result in renal impairment, even in the presence of MetS. PMID:25285417

  10. Posterior Nutcracker Syndrome Associated with Interrupted Left Inferior Vena Cava with Azygos Continuation and Retroaortic Right Renal Vein

    Energy Technology Data Exchange (ETDEWEB)

    Luo, Xiao Li; Zhou, Xiao Dong [Xijing Hospital, Fourth Military Medical University, Shaanxi (China); Qian, Gen Nian; Xiao, Hui; Zhao, Chun Lei [Fuzhou General Hospital, Fujian (China)

    2012-06-15

    Various anatomic anomalies have been considered the causes of nutcracker syndrome (NCS). Posterior NCS refers to the condition, in which vascular narrowing was secondary to the compression of the retroaortic left renal vein while it is crossing between the aorta and the vertebral column. Here, we report an unusual case of posterior NCS associated with a complicated malformation of the interrupted left inferior vena cava with azygos continuation and retroaortic right renal vein, diagnosed by both color Doppler ultrasonography and CT angiography.

  11. White spot syndrome virus envelope protein VP124 involved in the virus infection

    Institute of Scientific and Technical Information of China (English)

    ZHU Yanbing; WU Chenglin; YANG Feng

    2008-01-01

    White spot syndrome virus(WSSV)is one of the major shrimp pathogens causing large economic losses to shrimp farming.In an attempt to identify the envelope proteins involved in the virus infection,purified WSSV virions were mixed with three antisera against WSSV envelope proteins(VP39,VP124 and VP187),individually.And then they were injected intramuscularly into crayfish (Procambarus clarkii) to conduct in vivo neutralization assays.The results showed that for groups injected with virions only and groups injected with the mixture of virions and antiserum against VP124,the crayfish mortalities were 100% and 60% on the 8th day postinfection,individually.The virus infection could be delayed or neutralized by antibody against the envelope pro-tein VP124.Quantitative PCR was used to further investigate the influence of three antisera described above on the virus infec-tion.The results showed that the antiserum against VP124 could restrain the propagation of WSSV in crayfish.All of the results suggested that the viral envelope protein VP124 played a role in WSSV infection.

  12. Effect of hepatitis B virus X gene on apoptosis and immune molecules of renal tubular epithelial cells

    Institute of Scientific and Technical Information of China (English)

    王轩

    2013-01-01

    Objective To investigate the effect of hepatitis B virus X(HBX)gene on apoptosis and immune moleculesof human proximal renal tubular epithelial cell line(HK-2).Methods The eukaryotic vector pcDNA3.1-myc-HBX containing HBX gene was transiently transfected into

  13. [From the molecular genetics of Alport's syndrome to principles of organo-protection in chronic renal diseases].

    Science.gov (United States)

    Gross, Oliver; Weber, Manfred

    2005-12-15

    Scarring is known to be the endpoint of most chronic kidney diseases. Therefore, prevention of renal fibrosis is a very important topic. The hereditary type IV collagen disease Alport's syndrome is a rare, but challenging cause of chronic renal fibrosis. Increasing knowledge about the pathogenesis of Alport's syndrome may help to find principles of nephro-protection in chronic renal diseases. The defect gene in Alport's syndrome causes an altered assembly of extracellular matrix leading to a defect cell-matrix interaction and fibrosis. This scarring is regulated by comparable mechanisms as in diabetic nephropathy or chronic inflammatory renal diseases. NEPHRO-PROTECTION IN ANIMAL MODELS: By using an Alport animal model of chronic renal fibrosis, principles of nephro-protective therapies such as blockade of the renin-angiotensin system or the effect of HMG-CoA reductase inhibitors can be investigated. CURRENT AND FUTURE NEPHRO-PROTECTION IN HUMANS: The same model serves for evaluation of new organo-protective therapies such as vasopeptidase inhibitors, blockade of endothelin, chemokine and collagen receptors as well as stem cell therapy and their potential benefit for patients with chronic renal diseases.

  14. GATA3 mutation in a family with hypoparathyroidism, deafness and renal dysplasia syndrome

    Institute of Scientific and Technical Information of China (English)

    Zi-Yang Zhu; Qiao-Li Zhou; Shi-Ning Ni; Wei Gu

    2014-01-01

    Background: The hypoparathyroidism, deafness and renal dysplasia (HDR) syndrome is an autosomal dominant disorder primarily caused by GATA3 gene mutation. We report here a case that both of a Chinese boy and his father had HDR syndrome which caused by a novel mutation of GATA3. Methods: Polymerase chain reaction and DNA sequencing was performed to detect the exons of the GATA3 gene for mutation analysis. Results: Sequence analysis of GATA3 revealed a heterozygous nonsense mutation in this family: a mutation of GATA3 at exon 2 (c.515C >A) that resulted in a premature stop at codon 172 (p.S172X) with a loss of two zinc fi nger domains. Conclusion: We identifi ed a novel nonsense mutation which will expand the spectrum of HDR-associated GATA3 mutations.

  15. HERV-mediated genomic rearrangement of EYA1 in an individual with branchio-oto-renal syndrome.

    Science.gov (United States)

    Sanchez-Valle, Amarilis; Wang, Xueqing; Potocki, Lorraine; Xia, Zhilian; Kang, Sung-Hae L; Carlin, Mary E; Michel, Donnice; Williams, Patricia; Cabrera-Meza, Gerardo; Brundage, Ellen K; Eifert, Anna L; Stankiewicz, Pawel; Cheung, Sau Wai; Lalani, Seema R

    2010-11-01

    Branchio-oto-renal syndrome is characterized by branchial defects, hearing loss, preauricular pits, and renal anomalies. Mutations in EYA1 are the most common cause of branchio-oto-renal and branchio-otic syndromes. Large chromosomal aberrations of 8q13, including complex rearrangements occur in about 20% of these individuals. However, submicroscopic deletions and the molecular characterization of genomic rearrangements involving the EYA1 gene have rarely been reported. Using the array-comparative genomic hybridization, we identified non-recurrent genomic deletions including the EYA1 gene in three patients with branchio-oto-renal syndrome, short stature, and developmental delay. One of these deletions was mediated by two human endogenous retroviral sequence blocks, analogous to the AZFa microdeletion on Yq11, responsible for male infertility. This report describes the expanded phenotype of individuals, resulting from contiguous gene deletion involving the EYA1 gene and provides a molecular description of the genomic rearrangements involving this gene in branchio-oto-renal syndrome. © 2010 Wiley-Liss, Inc.

  16. Concurrent nephrotic syndrome and acute renal failure caused by chronic lymphocytic leukemia (CLL): a case report and literature review.

    Science.gov (United States)

    Dou, Xianrui; Hu, Haitang; Ju, Yongle; Liu, Yongdong; Kang, Kaifu; Zhou, Shufeng; Chen, Wenfang

    2011-10-13

    Kidney injury associated with lymphocytic leukemia (CLL) is typically caused by direct tumor infiltration which occasionally results in acute renal failure. Glomerular involvement presenting as proteinuria or even nephrotic syndrome is exceptionally rare. Here we report a case of 54-year-old male CLL patient with nephrotic syndrome and renal failure. The lymph node biopsy confirmed that the patients had CLL with remarkable immunoglobulin light chain amyloid deposition. The renal biopsy demonstrated the concurrence of AL amyloidosis and neoplastic infiltration. Combined treatment of fludarabine, cyclophosphamide and rituximab resulted in remission of CLL, as well as the renal disfunction and nephrotic syndrome, without recurrence during a 12-month follow-up. To our knowledge, this is the first case of CLL patient showing the nephrotic syndrome and acute renal failure caused by AL amyloidosis and neoplastic infiltration. Though AL amyloidosis caused by plasma cell dyscrasia usually responses poorly to chemotherapy, this patient exhibited a satisfactory clinical outcome due to successful inhibition of the production of amylodogenic light chains by combined chemotherapy.

  17. Oral carnitine therapy in children with cystinosis and renal Fanconi syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Gahl, W.A.; Bernardini, I.; Dalakas, M.; Rizzo, W.B.; Harper, G.S.; Hoeg, J.M.; Hurko, O.; Bernar, J.

    1988-02-01

    11 children with either cystinosis or Lowe's syndrome had a reduced content of plasma and muscle carnitine due to renal Fanconi syndrome. After treatment with oral L-carnitine, 100 mg/kg per d divided every 6 h, plasma carnitine concentrations became normal in all subjects within 2 d. Initial plasma free fatty acid concentrations, inversely related to free carnitine concentrations, were reduced after 7-20 mo of carnitine therapy. Muscle lipid accumulation, which varied directly with duration of carnitine deficiency (r = 0.73), improved significantly in three of seven rebiopsied patients after carnitine therapy. One Lowe's syndrome patient achieved a normal muscle carnitine level after therapy. Muscle carnitine levels remained low in all cystinosis patients, even though cystinotic muscle cells in culture took up L-(/sup 3/H)carnitine normally. The half-life of plasma carnitine for cystinotic children given a single oral dose approximated 6.3 h; 14% of ingested L-carnitine was excreted within 24 h. Studies in a uremic patient with cystinosis showed that her plasma carnitine was in equilibrium with some larger compartment and may have been maintained by release of carnitine from the muscle during dialysis. Because oral L-carnitine corrects plasma carnitine deficiency, lowers plasma free fatty acid concentrations, and reverses muscle lipid accumulation in some patients, its use as therapy in renal Fanconi syndrome should be considered. However, its efficacy in restoring muscle carnitine to normal, and the optimal dosage regimen, have yet to be determined.

  18. Herlyn-Werner-Wunderlich Syndrome Consisting of Uterine Didelphys, Obstructed Hemivagina and Ipsilateral Renal Agenesis in a Newborn

    Directory of Open Access Journals (Sweden)

    Tsung-Hsin Wu

    2012-02-01

    Full Text Available Herlyn-Werner-Wunderlich (HWW syndrome is a rare variant of Müllerian duct anomalies consisting of uterine didelphys, obstructed hemivagina, and ipsilateral renal agenesis. Patients with HWW syndrome are usually asymptomatic until menarche, when they present with acute lower abdominal pain. Here we report a case of a female newborn with right renal agenesis diagnosed during the pregnancy. The patient presented with a protruding mass over the vaginal introitus that was associated with an obstructed hemivagina and uterine didelphys.

  19. Bilateral renal tumors in an adult man with Smith-Magenis syndrome: The role of the FLCN gene.

    Science.gov (United States)

    Dardour, Leila; Verleyen, Pieter; Lesage, Karl; Holvoet, Maureen; Devriendt, Koen

    2016-10-01

    Smith-Magenis syndrome (SMS) is a contiguous-gene disorder most commonly caused by a deletion of chromosome 17p11.2. We report a 57 year-old man with SMS who presents bilateral renal tumors. This is most likely related to haploinsufficiency of FLCN gene, located in the deleted region, and a known tumor suppressor gene. Haploinsufficiency of FLCN causes Birt-Hogg-Dubé syndrome (BHDS), characterized by pulmonary cysts, renal and skin tumors. The present observation suggests that the follow-up of patients with SMS should also focus on possible manifestations of BHDS.

  20. Recurrent Posterior Reversible Encephalopathy Syndrome Potentially Related to AIDS and End-Stage Renal Disease: A Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Olivia Hui-Chiun Chang

    2012-01-01

    Full Text Available Posterior reversible encephalopathy syndrome (PRES is a clinicoradiological syndrome that is characterized by clinical features including headache, altered mental status, cortical blindness, seizures, and other focal neurological signs as well as subcortical edema without infarction on neuroimaging. Under the umbrella of hypertensive encephalopathy, PRES is defined by reversible cerebral edema due to dysfunction of the cerebrovascular blood-brain barrier unit. The pathophysiology of PRES is thought to result from abnormalities in the transmembrane flow of intravascular fluid and proteins caused by two phenomena: one, cerebral autoregulatory failure and two, loss of integrity of the blood-brain barrier. PRES is not a common disease in patients with human immunodeficiency virus (HIV and AIDS with only three previously reported cases. Both the HIV and end-stage renal disease appear to further compromise the blood brain barrier. Although uncommon, PRES recurrence has been described. To the best of our knowledge, this is the first report demonstrating recurrent PRES in a HIV patient on hemodialysis for end-stage renal disease.

  1. Peroxisome proliferator-activated receptor family and its relationship to renal complications of the metabolic syndrome.

    Science.gov (United States)

    Guan, Youfei

    2004-11-01

    Peroxisome proliferator-activated receptors (PPAR) are members of the nuclear hormone receptor superfamily of ligand-activated transcription factors. Three PPAR isoforms, designated PPARalpha, -beta/delta, and -gamma, have been identified and attracted enormous attention as a result of the key role that these receptors play in regulating adipogenesis, lipid metabolism, insulin sensitivity, inflammation, and BP. Growing evidence points to a causative relationship between PPAR activity and the metabolic syndrome, including insulin resistance, glucose intolerance or type 2 diabetes, obesity, dyslipidemia, hypertension, atherosclerosis, and albuminuria. Importantly, both PPAR-alpha activators, such as the fibric acid class of hypolipidemic drugs, and PPAR-gamma agonists, including antidiabetic thiazolidinediones, have been proved to be effective for improving diverse aspects of the metabolic syndrome. All three PPAR isoforms seem to play important roles in the development of diabetic nephropathy in type 2 diabetes. Accumulating data suggesting that PPAR may serve as potential therapeutic targets for treating the metabolic syndrome and its related renal complications have begun to emerge. This article reviews the literature pertaining to the action, ligand selectivity, and physiologic role of PPAR. Particular emphasis is placed on their pathogenic roles in the metabolic syndrome and the therapeutic utility of PPAR modulators in the treatment of diabetic nephropathy.

  2. Alport syndrome: significance of gingival biopsy in the initial diagnosis and periodontal evaluation after renal transplantation.

    Science.gov (United States)

    Toygar, Hilal Uslu; Toygar, Okan; Guzeldemir, Esra; Cilasun, Ulkem; Nacar, Ahmet; Bal, Nebil

    2009-01-01

    Alport Syndrome (AS) is an important hereditary disorder affecting the glomerular basement membrane. Diagnosis of AS is based on the presence of hematuric nephropathy, renal failure, hearing loss, ocular abnormalities and changes in the glomerular basement membrane of the lamina densa. The aims of this case report were to show the changes in the gingival tissues in a patient with AS under therapy with cyclosporin-A after renal transplantation and to discuss the possible role of type IV collagen in gingival basal lamina as an alternative approach for the diagnosis of AS. A 20-year-old male patient with AS underwent periodontal therapy including a series of gingivectomy surgeries. Gingival samples obtained during the second surgery were examined histopathologically and by transmission electron microscopy for further pathological examination. Gingivectomy procedures have been performed every 6 months over the last 4 years. The excessive and fibrous gingival enlargements resulted in migration of the anterior teeth, but no alveolar bone loss occurred. This is the first report to demonstrate the possible changes in the gingival tissues caused by AS. It is suggested that gingival biopsy can be an initial diagnostic tool instead of renal or skin biopsies. Proper dental and periodontal care and regular visits to the dentist could provide limited gingival hyperplasia to patients with AS.

  3. The roles of the liver and pancreas in renal nutcracker syndrome.

    Science.gov (United States)

    Yun, Seong Jong; Nam, Deok Ho; Ryu, Jung Kyu; Kim, Ji Su

    2014-10-01

    To assess the frequency and significance of presence of the liver and pancreas at the left renal vein (LRV) level in patients with suspected renal nutcracker syndrome (NCS). We included 101 patients with hematuria who underwent urography three-dimensional CT between April 2009 and November 2013. These patients were divided into NCS (n=25) and non-NCS (n=76) patients according to the following CT criteria: (1) the presence of beak sign and (2) hilar-aortomesenteric left renal vein diameter ratio >4. Patients were grouped according to the presence of the liver and pancreas at the LRV: group LP (both liver and pancreas), group L (only liver), group P (only pancreas), and group O (neither liver nor pancreas). The difference in the frequencies of groups was analyzed between NCS and non-NCS patients. Multivariate analysis was used to determine the independent factors between NCS and non-NCS patients. The frequencies of group LP, group L, group P, and group O in NCS vs. non-NCS were 88% vs. 5.3% (ppancreas at the level of the LRV was frequently found in NCS and was the independent factor for NCS. Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.

  4. Alport syndrome: significance of gingival biopsy in the initial diagnosis and periodontal evaluation after renal transplantation

    Directory of Open Access Journals (Sweden)

    Hilal Uslu Toygar

    2009-12-01

    Full Text Available Alport Syndrome (AS is an important hereditary disorder affecting the glomerular basement membrane. Diagnosis of AS is based on the presence of hematuric nephropathy, renal failure, hearing loss, ocular abnormalities and changes in the glomerular basement membrane of the lamina densa. The aims of this case report were to show the changes in the gingival tissues in a patient with AS under therapy with cyclosporin-A after renal transplantation and to discuss the possible role of type IV collagen in gingival basal lamina as an alternative approach for the diagnosis of AS. A 20-year-old male patient with AS underwent periodontal therapy including a series of gingivectomy surgeries. Gingival samples obtained during the second surgery were examined histopathologically and by transmission electron microscopy for further pathological examination. Gingivectomy procedures have been performed every 6 months over the last 4 years. The excessive and fibrous gingival enlargements resulted in migration of the anterior teeth, but no alveolar bone loss occurred. This is the first report to demonstrate the possible changes in the gingival tissues caused by AS. It is suggested that gingival biopsy can be an initial diagnostic tool instead of renal or skin biopsies. Proper dental and periodontal care and regular visits to the dentist could provide limited gingival hyperplasia to patients with AS.

  5. [A case of cryptococcal meningitis with nephrotic syndrome and renal insufficiency under immunosuppressive therapy].

    Science.gov (United States)

    Tsuchida, Hiroki; Ichikawa, Daisuke; Shima, Yoshinori; Yasuda, Takashi; Sato, Takeo; Kimura, Kenjiro

    2007-01-01

    A 76 year-old woman was admitted to our hospital because of pyrexia and fatigue. One year earlier, she was diagnosed as nephrotic syndrome(NS) caused by focal segmental glomerulosclerosis and immunosuppressive therapy was started with marked amelioration of proteinuria. Thereafter, her renal function worsened, but only supportive treatment was continued. After admission, a cerebrospinal fluid (CSF) examination revealed Cryptococcus neoformans (C. neoformans) by india ink staining and a subsequent CSF culture confirmed C. neoformans infection. Accordingly, we made the diagnosis of cryptococcal meningitis and immediately started multiple anti fungal drugs with dosage modification according to her impaired renal function. Immunosuppressive therapy for NS was temporarily terminated. The inflammatory signs and symptoms soon were markedly improved, but the anti cryptococcal antibody titer in the serum and CSF remained high. Immunosuppressive therapy was started again at a low dosage because urinary protein had increased again. One hundred and eight days from admission, she was discharged with a regimen of multiple anti fungal drugs. Proteinuria and renal insufficiency was almost stable during hospitalization. Most fungal infection develops in patients in an immunosuppressive state induced by immunosuppressive drugs, HIV infection and so on. Patients with NS are frequently in an immunosuppressive state because of urinary loss of immunoglobulins and the use of immunosuppressive drugs. Therefore, it should be remembered that patients with NS are at a high risk of suffering from fungal infection.

  6. Renal recovery with eculizumab in atypical hemolytic uremic syndrome following prolonged dialysis.

    Science.gov (United States)

    Povey, Hannah; Vundru, Rahul; Junglee, Naushad; Jibani, Mahdi

    2014-11-01

    Hemolytic uremic syndrome (HUS) is a thrombotic microangiopathy (TMA) which encompasses hemolytic anemia, thrombocytopenia, and organ impairment. Around 10% of cases are atypical HUS (aHUS), a rare disease with poor outcomes caused by uncontrolled activation of the alternative complement pathway. This case describes a young woman with clinical manifestations compatible with TMA during childhood and adolescence who was formally diagnosed with aHUS at the age of 21. She was managed with intensive plasma exchange and hemodialysis, which failed to improve her severe acute kidney injury and other hematological manifestations of aHUS. This was further compounded by several episodes of flash pulmonary edema and the posterior reversible encephalopathy syndrome (PRES). Treatment with the monoclonal anti-C5 inhibitor, eculizumab, improved all hematological parameters with almost full renal recovery following 3.5 months of dialysis. So far, long-term use of eculizumab (> 11 months) continues to be effective and without complication. Our case illustrates the difficulty but importance of early consideration of aHUS in patients presenting with TMA. More importantly, we highlight that near-normal renal recovery may be attained with eculizumab in adults even after a long dependence on dialysis - an observation that has not been reported in the literature so far.

  7. Clinical and immunological characteristics of hemorrhagic fever with renal syndrome in women of different age groups

    Directory of Open Access Journals (Sweden)

    Kutdusova A.M.

    2012-09-01

    Full Text Available Objective: To evaluate the clinical and immunological features of the hemorrhagic fever with renal syndrome in women of different age groups. Materials and methods: Clinical and laboratory characteristics of hemorrhagic fever with renal syndrome in 148 women aged 17 to 65 years old have been investigated. Patients have been divided into two groups: group I included 101 patients with normal menstrual rhythm, group II included 47 female patients with menopause. In 57 women (36 from group I, 21 — from group II the content of CD3+, CD4+, CD8+, CD16+, CD19+ — sub-populations of peripheral blood lymphocytes has been determined. Results: In compared groups significant differences in structure and frequency of complications of the disease have been revealed. Unidirectional tendency to increase significantly reduced absolute rates of investigated lymphocyte subpopulations in dynamics of the disease has been identified. It also has been stated that by the time of early convalescence in case of severe form of HFRS the indices did not reach the standard level. In an older group of women deeper damage and long-term recovery of immune system have been marked. Conclusion: According to the results of clinical and immunological studies the research work has revealed that in young women the response of the immune system to HFRS has developed faster and stronger than that in patients during the menopause period.

  8. Osteomalacia complicating renal tubular acidosis in association with Sjogren′s syndrome

    Directory of Open Access Journals (Sweden)

    Zohra El Ati

    2014-01-01

    Full Text Available Renal involvement in Sjogren′s syndrome (SS is not uncommon and may precede other complaints. Tubulointerstitial nephritis is the most common renal disease in SS and may lead to renal tubular acidosis (RTA, which in turn may cause osteomalacia. Nevertheless, osteomalacia rarely occurs as the first manifestation of a renal tubule disorder due to SS. We herewith describe a 43-year-old woman who was admitted to our hospital for weakness, lumbago and inability to walk. X-ray of the long bones showed extensive demineralization of the bones. Laboratory investigations revealed chronic kidney disease with serum creatinine of 2.3 mg/dL and creatinine clearance of 40 mL/min, hypokalemia (3.2 mmol/L, hypophosphatemia (0.4 mmol/L, hypocalcemia (2.14 mmol/L and hyperchloremic metabolic acidosis (chlorine: 114 mmol/L; alkaline reserve: 14 mmol/L. The serum alkaline phosphatase levels were elevated. The serum levels of 25-hydroxyvitamin D and 1,25-dihydroxy vitamin D were low and borderline low, respectively, and the parathyroid hormone level was 70 pg/L. Urinalysis showed inappropriate alkaline urine (urinary PH: 7, glycosuria with normal blood glucose, phosphaturia and uricosuria. These values indicated the presence of both distal and proximal RTA. Our patient reported dryness of the mouth and eyes and Schirmer′s test showed xerophthalmia. An accessory salivary gland biopsy showed changes corresponding to stage IV of Chisholm and Masson score. Kidney biopsy showed diffuse and severe tubulo-interstitial nephritis with dense lymphoplasmocyte infiltrates. Sicca syndrome and renal interstitial infiltrates indicated SS as the underlying cause of the RTA and osteomalacia. The patient received alkalinization, vitamin D (Sterogyl ®, calcium supplements and steroids in an initial dose of 1 mg/kg/day, tapered to 10 mg daily. The prognosis was favorable and the serum creatinine level was 1.7 mg/dL, calcium was 2.2 mmol/L and serum phosphate was 0.9 mmol/L.

  9. Blue toe syndrome treated with sympathectomy in a patient with acute renal failure caused by cholesterol embolization

    Directory of Open Access Journals (Sweden)

    Min-Gang Kim

    2013-12-01

    Full Text Available Blue toe syndrome is the most frequent manifestation of tissue ischemia caused by cholesterol embolization (CE, which can lead to amputation of affected lower extremities, if severe. However, any effective treatment is lacking. We experienced a case of spontaneously presenting blue toe syndrome and concomitant acute renal failure in a patient with multiple atherosclerotic risk factors. CE was confirmed by renal biopsy. Despite medical treatment including prostaglandin therapy and narcotics, the toe lesion progressed to gangrene with worsening ischemic pain. Therefore, we performed lumbar sympathectomy, which provided dramatic pain relief as well as an adequate blood flow to the ischemic lower extremities, resulting in healing of the gangrenous lesion and avoiding toe amputation. This is the first reported case of a patient with intractable ischemic toe syndrome caused by CE that was treated successfully by sympathectomy. Our observations suggest that sympathectomy may be beneficial in some patients with CE-associated blue toe syndrome.

  10. [Executive summary of the recommendations on the evaluation and management of renal disease in human immunodeficiency virus-infected patients].

    Science.gov (United States)

    Gorriz, José L; Gutiérrez, Félix; Trullàs, Joan C; Arazo, Piedad; Arribas, Jose R; Barril, Guillermina; Cervero, Miguel; Cofán, Frederic; Domingo, Pere; Estrada, Vicente; Fulladosa, Xavier; Galindo, María J; Gràcia, Sílvia; Iribarren, José A; Knobel, Hernando; López-Aldeguer, José; Lozano, Fernando; Martínez-Castelao, Alberto; Martínez, Esteban; Mazuecos, Maria A; Miralles, Celia; Montañés, Rosario; Negredo, Eugenia; Palacios, Rosario; Pérez-Elías, María J; Portilla, Joaquín; Praga, Manuel; Quereda, Carlos; Rivero, Antonio; Santamaría, Juan M; Sanz, José; Sanz, Jesús; Miró, José M

    2014-11-01

    The aim of this article is to update the 2010 recommendations on the evaluation and management of renal disease in human immunodeficiency virus (HIV)-infected patients. Renal function should be monitored in all HIV-infected patients. The basic renal work-up should include measurements of serum creatinine, estimated glomerular filtration rate by CKD-EPI, urine protein-to-creatinine ratio, and urinary sediment. Tubular function tests should include determination of serum phosphate levels and urine dipstick for glycosuria. In the absence of abnormal values, renal screening should be performed annually. In patients treated with tenofovir, or with risk factors for chronic kidney disease (CKD), more frequent renal screening is recommended. In order to prevent disease progression, potentially nephrotoxic antiretroviral drugs are not recommended in patients with CKD or risk factors for CKD. The document provides indications for renal biopsy and advises on the optimal time for referral of a patient to the nephrologist. The indications for and evaluation and management of dialysis and renal transplantation are also addressed. Copyright © 2014 Elsevier España, S.L.U. y Sociedad Española de Enfermedades Infecciosas y Microbiología Clínica. All rights reserved.

  11. Porites white patch syndrome: associated viruses and disease physiology

    Science.gov (United States)

    Lawrence, S. A.; Davy, J. E.; Wilson, W. H.; Hoegh-Guldberg, O.; Davy, S. K.

    2015-03-01

    In recent decades, coral reefs worldwide have undergone significant changes in response to various environmental and anthropogenic impacts. Among the numerous causes of reef degradation, coral disease is one factor that is to a large extent still poorly understood. Here, we characterize the physiology of white patch syndrome (WPS), a disease affecting poritid corals on the Great Barrier Reef. WPS manifests as small, generally discrete patches of tissue discolouration. Physiological analysis revealed that chlorophyll a content was significantly lower in lesions than in healthy tissues, while host protein content remained constant, suggesting that host tissue is not affected by WPS. This was confirmed by transmission electron microscope (TEM) examination, which showed intact host tissue within lesions. TEM also revealed that Symbiodinium cells are lost from the host gastrodermis with no apparent harm caused to the surrounding host tissue. Also present in the electron micrographs were numerous virus-like particles (VLPs), in both coral and Symbiodinium cells. Small (<50 nm diameter) icosahedral VLPs were significantly more abundant in coral tissue taken from diseased colonies, and there was an apparent, but not statistically significant, increase in abundance of filamentous VLPs in Symbiodinium cells from diseased colonies. There was no apparent increase in prokaryotic or eukaryotic microbial abundance in diseased colonies. Taken together, these results suggest that viruses infecting the coral and/or its resident Symbiodinium cells may be the causative agents of WPS.

  12. An Intriguing Relationship between Type 2 Diabetes Mellitus and Hepatitis C Virus Infection: The Renal Perspective

    Directory of Open Access Journals (Sweden)

    Raman B. Suresh

    2009-06-01

    Full Text Available Type 2 diabetes mellitus has emerged as the commonest cause of end-stage renal disease (ESRD over the past three decades. It accounts for 30% to 50% of patients on renal replacement therapy (RRT; nearly 80% of patients are managed with hospital-based hemodialysis. Chronic hepatitis C virus (HCV infection is endemic among patients on RRT, especially among those undergoing maintenance hemodialysis (HD (1, 2. Therefore, both the disorders are prevalent and coexist among patients with ESRD and frequently cause complications in a considerable majority of the affected patients resulting in poor outcomes.A number of studies have demonstrated a higher prevalence of type 2 diabetes mellitus in patients with HCV in comparison to general population (2-6. Mehta et al., in a recent epidemiological study based on data from the Third National Health and Nutrition Examination Survey (NHANES III, provided compelling data for the distinctive association between chronic HCV infection and type 2 diabetes mellitus (3. In this report, anti-HCV positive subjects (>40 years old had a significantly higher prevalence (OR=3.77 of type 2 diabetes mellitus compared with HCV-negative subjects. Likewise, Egyptian investigators observed a two-fold higher incidence of type 2 diabetes mellitus in patients who had HCV infection compared with those who did not (4.

  13. Proteomic Analyses of the Shrimp White Spot Syndrome Virus

    Institute of Scientific and Technical Information of China (English)

    Yan-wei TAN; Zheng-li SHI

    2008-01-01

    White spot syndrome virus (WSSV), a unique member within the virus family Nimaviridae, is the most notorious aquatic virus infecting shrimp and other crustaceans and has caused enormous economic losses in the shrimp farming industry worldwide. Therefore, a comprehensive understanding of WSSV morphogenesis, structural proteins, and replication is essential for developing prevention measures of this serious parasite. The viral genome is approximately 300kb and contains more than 180 open reading frames (ORF). However, most of proteins encoded by these ORF have not been characterized. Due to the importance of WSSV structural proteins in the composition of the virion structure, infection process and interaction with host cells, knowledge of structural proteins is essential to understanding WSSV entry and infection as well as for exploring effective prevention measures. This review article summarizes mainly current investigations on WSSV structural proteins including the relative quantities, localization, function and protein-protein interactions. Traditional proteomic studies of 1D or 2D gel electrophoresis separations and mass spectrometry (MS) followed by database searches have identified a total of 39 structural proteins. Shotgun proteomics and iTRAQ were initiated to identify more structural proteins. To date, it is estimated that WSSV is assembled by at least 59 structural proteins, among them 35 are defined as the envelope fraction (including tegument proteins) and 9 as nucleocapsid proteins. Furthermore, the interaction within several major structural proteins has also been investigated. This identitification and characterization of WSSV protein components should help in the understanding of the viral assembly process and elucidate the roles of several major structural proteins.

  14. Accelerated renal disease is associated with the development of metabolic syndrome in a glucolipotoxic mouse model.

    Science.gov (United States)

    Martínez-García, Cristina; Izquierdo, Adriana; Velagapudi, Vidya; Vivas, Yurena; Velasco, Ismael; Campbell, Mark; Burling, Keith; Cava, Fernando; Ros, Manuel; Oresic, Matej; Vidal-Puig, Antonio; Medina-Gomez, Gema

    2012-09-01

    Individuals with metabolic syndrome are at high risk of developing chronic kidney disease (CKD) through unclear pathogenic mechanisms. Obesity and diabetes are known to induce glucolipotoxic effects in metabolically relevant organs. However, the pathogenic role of glucolipotoxicity in the aetiology of diabetic nephropathy is debated. We generated a murine model, the POKO mouse, obtained by crossing the peroxisome proliferator-activated receptor gamma 2 (PPARγ2) knockout (KO) mouse into a genetically obese ob/ob background. We have previously shown that the POKO mice showed: hyperphagia, insulin resistance, hyperglycaemia and dyslipidaemia as early as 4 weeks of age, and developed a complete loss of normal β-cell function by 16 weeks of age. Metabolic phenotyping of the POKO model has led to investigation of the structural and functional changes in the kidney and changes in blood pressure in these mice. Here we demonstrate that the POKO mouse is a model of renal disease that is accelerated by high levels of glucose and lipid accumulation. Similar to ob/ob mice, at 4 weeks of age these animals exhibited an increased urinary albumin:creatinine ratio and significantly increased blood pressure, but in contrast showed a significant increase in the renal hypertrophy index and an associated increase in p27(Kip1) expression compared with their obese littermates. Moreover, at 4 weeks of age POKO mice showed insulin resistance, an alteration of lipid metabolism and glomeruli damage associated with increased transforming growth factor beta (TGFβ) and parathyroid hormone-related protein (PTHrP) expression. At this age, levels of proinflammatory molecules, such as monocyte chemoattractant protein-1 (MCP-1), and fibrotic factors were also increased at the glomerular level compared with levels in ob/ob mice. At 12 weeks of age, renal damage was fully established. These data suggest an accelerated lesion through glucolipotoxic effects in the renal pathogenesis in POKO mice.

  15. Accelerated renal disease is associated with the development of metabolic syndrome in a glucolipotoxic mouse model

    Directory of Open Access Journals (Sweden)

    Cristina Martínez-García

    2012-09-01

    Individuals with metabolic syndrome are at high risk of developing chronic kidney disease (CKD through unclear pathogenic mechanisms. Obesity and diabetes are known to induce glucolipotoxic effects in metabolically relevant organs. However, the pathogenic role of glucolipotoxicity in the aetiology of diabetic nephropathy is debated. We generated a murine model, the POKO mouse, obtained by crossing the peroxisome proliferator-activated receptor gamma 2 (PPARγ2 knockout (KO mouse into a genetically obese ob/ob background. We have previously shown that the POKO mice showed: hyperphagia, insulin resistance, hyperglycaemia and dyslipidaemia as early as 4 weeks of age, and developed a complete loss of normal β-cell function by 16 weeks of age. Metabolic phenotyping of the POKO model has led to investigation of the structural and functional changes in the kidney and changes in blood pressure in these mice. Here we demonstrate that the POKO mouse is a model of renal disease that is accelerated by high levels of glucose and lipid accumulation. Similar to ob/ob mice, at 4 weeks of age these animals exhibited an increased urinary albumin:creatinine ratio and significantly increased blood pressure, but in contrast showed a significant increase in the renal hypertrophy index and an associated increase in p27Kip1 expression compared with their obese littermates. Moreover, at 4 weeks of age POKO mice showed insulin resistance, an alteration of lipid metabolism and glomeruli damage associated with increased transforming growth factor beta (TGFβ and parathyroid hormone-related protein (PTHrP expression. At this age, levels of proinflammatory molecules, such as monocyte chemoattractant protein-1 (MCP-1, and fibrotic factors were also increased at the glomerular level compared with levels in ob/ob mice. At 12 weeks of age, renal damage was fully established. These data suggest an accelerated lesion through glucolipotoxic effects in the renal pathogenesis in POKO mice.

  16. Sensitivitas Berbagai Stadia Kepiting Bakau (Scylla paramamosain Estampador) terhadap White Spot Syndrome Virus

    National Research Council Canada - National Science Library

    ZAFRAN; RIA IKA MAHARANI; SURANTO

    2005-01-01

    .... One of those is the lower survival rate of larva stage due to the diseases. The objective of this research was to observe the sensitivity of mud crab from various stages to White Spot Syndrome Virus (WSSV...

  17. Porcine reproductive and respiratory syndrome virus (PRRSV): pathogenesis and interaction with the immune System

    Science.gov (United States)

    This review addresses important issues of porcine reproductive and respiratory syndrome virus (PRRSV) infection, immunity, pathogenesis and control. Worldwide PRRS is the most economically important infectious disease of pigs. We highlight the latest information on viral genome structure, pathogenic...

  18. A case of minimal change nephrotic syndrome with acute renal failure complicating Hashimotoâs disease.

    Science.gov (United States)

    Iwazu, Y; Nemoto, J; Okuda, K; Nakazawa, E; Hashimoto, A; Fujio, Y; Sakamoto, M; Ando, Y; Muto, S; Kusano, E

    2008-01-01

    A 63-year-old man was admitted to our hospital for evaluation of generalized edema. Coexistence of severe hypothyroidism and nephrotic syndrome was detected by laboratory examination. High titer of both antimicrosomal antibody and antithyroid peroxidase antibody indicated Hashimotoâs disease. Renal biopsy showed minimal change glomerular abnormality, but no findings of membranous nephropathy. A series of medical treatments, including steroid therapy, thyroid hormone and human albumin replacement therapy, were administered. However, acute renal failure accompanied by hypotension, was not sufficiently prevented. After 9 sessions of plasmapheresis therapy, the severe proteinuria and low serum albumin levels were improved. Even after resting hypotension was normalized, neither renal function nor thyroid function were fully recovered. After discharge, renal function gradually returned to normal, and the blood pressure developed into a hypertensive state concomitant with the normalization of thyroid function. This report is a rare case of autoimmune thyroid disease complicated with minimal change nephrotic syndrome. In most cases of nephritic syndrome, acute renal failure (ARF) has been reported to coexist with hypertension. Although pseudohypothyroidism is well-known in nephrotic pathophysiology, complications of actual hypothyroidism are uncommon. It is suggested that the development of hypotension and ARF could be enhanced not only by hypoproteinemia, but also by severe hypothyroidism.

  19. Case report of a novel mutation of the EYA1 gene in a patient with branchio-oto-renal syndrome.

    Science.gov (United States)

    Spahiu, L; Merovci, B; Ismaili Jaha, V; Batalli Këpuska, A; Jashari, H

    2016-12-01

    Branchio-oto-renal (BOR) syndrome is an autosomal dominant disorder characterized by the coexistence of branchial cysts or fistulae, external ear malformation with pre-auricular pits or tags, hearing impairment and renal malformations. However, the presence of the main features varies in affected families. Here, we present a 16-year-old boy admitted to the Department of Nephrology at the Pediatric Clinic, University Clinical Center of Kosovo, Pristina, Republic of Kosovo because of severe renal insufficiency diagnosed 6 years ago, which progressed to end-stage renal failure. Clinical examination on readmission showed a pale, lethargic and edematous child, with auricular deformity, pre-auricular tags and pits as well as bilateral branchial fistulae. Laboratory tests revealed high blood urea nitrogen (BUN) 15.96 mmol/L and serum creatinine 633.0 µmol/L; low glomerular filtration rate (GFR) 12 mL/min./ 1.73 m(2) and massive proteinuria 4+. Abdominal ultrasound showed bilateral kidney hypoplasia. A novel mutation of the EYA1 gene was confirmed. Daily hemodialysis is continuing until renal transplantation is done. This case is presented to increase awareness among general practitioners to consider BOR syndrome or other renal abnormalities in patients with branchial fistula and/ or external ear anomalies or similar findings in other family members.

  20. Neuropatía por virus BK post trasplante renal diagnostico y seguimiento por PCR en tiempo real BK virus nephropathy after renal transplantation: Diagnosis and prognosis by real time PCR

    Directory of Open Access Journals (Sweden)

    Marcela Echavarria

    2007-12-01

    Full Text Available La nefropatía producida por el virus BK puede llevar a la pérdida del trasplante renal. El diagnóstico etiológico es importante debido a que la clínica no permite diferenciar entre nefropatía por virus BK y rechazo agudo, en donde los tratamientos de estas dos entidades son diametralmente opuestos. El desarrollo reciente de métodos moleculares muy sensibles y específicos como PCR y PCR en tiempo real para virus BK permiten un diagnóstico de certeza en forma rápida y cuantificar la carga viral presente. El diagnóstico de nefropatía por virus BK se realiza por inmunohistoquímica en una biopsia renal, pero dada la naturaleza multifocal de las lesiones, la sensibilidad no siempre es del 100%. Los nuevos métodos de PCR para detectar virus BK en sangre y orina contribuyen al diagnóstico de nefropatía de una manera más normatizada y menos invasiva. Más aún, la cuantificación del virus BK en sangre por PCR en tiempo real, ha demostrado ser útil en el diagnóstico y monitoreo de esta enfermedad. En este trabajo se presenta el caso de una paciente transplantada renal con nefropatía por virus BK y el desarrollo de un método de PCR en tiempo real para la detección de virus BK en sangre y orina. Esta nueva metodología confirmó el diagnóstico de nefropatía por virus BK lo que permitió un cambio en el esquema de inmunosupresión y la instauración de un tratamiento que pudo ser monitorizado utilizando la carga viral.BK virus nephropathy may lead to kidney transplant failure. BK infection and acute rejection are clinically undistinguishable, therefore diagnosis of these entities is critical to establish the correct treatment. The new molecular methods using PCR and real time PCR have significantly contributed to the rapid and sensitive diagnosis of BK virus. Furthermore, viral load determination in plasma has significantly been associated with BK virus nephropathy. Definite diagnosis of nephropathy requires renal biopsy, although

  1. Vasculitic syndromes in hepatitis C virus: A review

    Directory of Open Access Journals (Sweden)

    Gaafar Ragab

    2017-03-01

    Full Text Available Vasculitis is a remarkable presentation of the extrahepatic manifestations of HCV. According to the presence or absence of cryoglobulins it is subdivided into two main types: cryoglobulinemic vasculitis and non cryoglobulinemic vasculitis based on the attribution of vasculitis to serum cryoglobulins as a pathogenic factor. The attribution of cryoglobulinemia to HCV represents a success story in the history of immunology, microbiology, and clinical medicine. HCV can bind to and invade lymphocytes, consequently triggering an immune response through different mechanisms. The epidemiology of the disease is well described and the clinical picture describes cutaneous, pulmonary, musculoskeletal, neurological, renal, endocrine, gastrointestinal, hepatic and cardiovascular manifestations. It may also be associated with sicca symptoms, an increased risk of lymphoma and serious catastrophic events. The pathology is well characterized. A classification criteria of the syndrome that was validated in 2014 is discussed. Management of CV is decided according to the presence and severity of its clinical presentation. It is divided into asymptomatic, mild, moderate, severe and life threatening disease. Recently introduced direct antiviral agents are proving safe and effective in the management of cryoglobulinemic vasculitis, and it is advocated that the two types of vasculitis be given prioritization in the Egyptian mass campaign to eradicate HCV.

  2. Seroprevalence of avian hepatitis E virus and avian leucosis virus subgroup J in chicken flocks with hepatitis syndrome, China

    OpenAIRE

    Sun, Yani; Du, Taofeng; Liu, Baoyuan; Syed, Shahid Faraz; Chen, Yiyang; Li, Huixia; Wang, Xinjie; Zhang, Gaiping; Zhou, En-Min; Zhao, Qin

    2016-01-01

    Background From 2014 to 2015 in China, many broiler breeder and layer hen flocks exhibited a decrease in egg production and some chickens developed hepatitis syndrome including hepatomegaly, hepatic necrosis and hemorrhage. Avian hepatitis E virus (HEV) and avian leucosis virus subgroup J (ALV-J) both cause decreasing in egg production, hepatomegaly and hepatic hemorrhage in broiler breeder and layer hens. In the study, the seroprevalence of avian HEV and ALV-J in these flocks emerging the di...

  3. Cognitive, Affective Problems and Renal Cross Ectopy in a Patient with 48,XXYY/47,XYY Syndrome

    Directory of Open Access Journals (Sweden)

    Sefa Resim

    2015-01-01

    Full Text Available Klinefelter syndrome is the most common sex chromosome abnormality (SCA in infertile patients and 47,XXY genomic configuration constitutes most of the cases. However, additional Xs and/or Y such as 48,XXYY, 48,XXXY, and 47,XYY can occur less frequently than 47,XXY. Those configurations were considered as variants of Klinefelter syndrome. In this report, we present an infertile man with tall stature and decreased testicular volume. Semen analysis and hormonal evaluation supported the diagnosis of nonobstructive azoospermia. Genetic investigation demonstrated an abnormal male karyotype with two X chromosomes and two Y chromosomes consistent with 48,XXYY(17/47,XYY (13. Additionally, the patient expressed cognitive and affective problems which were documented by psychomotor retardation and borderline intelligence measured by an IQ value between 70 and 80. Systemic evaluation also revealed cross ectopy and malrotation of the right kidney in the patient. The couple was referred to microtesticular sperm extraction (micro-TESE/intracytoplasmic sperm injection (ICSI cycles and preimplantation genetic diagnosis (PGD. To the best of our knowledge, this is the first report of combination of XYY and XXYY syndromes associated with cognitive, affective dysfunction and renal malrotation.

  4. Cognitive, Affective Problems and Renal Cross Ectopy in a Patient with 48,XXYY/47,XYY Syndrome

    Science.gov (United States)

    Resim, Sefa; Kucukdurmaz, Faruk; Kankılıc, Nazım; Altunoren, Ozlem; Efe, Erkan; Benlioglu, Can

    2015-01-01

    Klinefelter syndrome is the most common sex chromosome abnormality (SCA) in infertile patients and 47,XXY genomic configuration constitutes most of the cases. However, additional Xs and/or Y such as 48,XXYY, 48,XXXY, and 47,XYY can occur less frequently than 47,XXY. Those configurations were considered as variants of Klinefelter syndrome. In this report, we present an infertile man with tall stature and decreased testicular volume. Semen analysis and hormonal evaluation supported the diagnosis of nonobstructive azoospermia. Genetic investigation demonstrated an abnormal male karyotype with two X chromosomes and two Y chromosomes consistent with 48,XXYY(17)/47,XYY (13). Additionally, the patient expressed cognitive and affective problems which were documented by psychomotor retardation and borderline intelligence measured by an IQ value between 70 and 80. Systemic evaluation also revealed cross ectopy and malrotation of the right kidney in the patient. The couple was referred to microtesticular sperm extraction (micro-TESE)/intracytoplasmic sperm injection (ICSI) cycles and preimplantation genetic diagnosis (PGD). To the best of our knowledge, this is the first report of combination of XYY and XXYY syndromes associated with cognitive, affective dysfunction and renal malrotation. PMID:26075116

  5. Reversible Posterior Leukoencephalopathy Syndrome Developing After Restart of Sunitinib Therapy for Metastatic Renal Cell Carcinoma

    Directory of Open Access Journals (Sweden)

    Shinji Fukui

    2016-01-01

    Full Text Available A 64-year-old Japanese man had started molecular-targeted therapy with sunitinib for lymph node metastasis 5 years after nephrectomy for left renal cell carcinoma (clear cell carcinoma, G2, pT2N0M0. He was transported to our emergency department because of generalized tonic-clonic seizure, vision loss, and impaired consciousness with acute hypertension after 8 cycles of treatment (2 years after the initiation of sunitinib therapy, including a drug withdrawal period for one year. MRI of the brain (FLAIR images showed multiple high-intensity lesions in the white matter of the occipital and cerebellar lobes, dorsal brain stem, and left thalamus. Reversible posterior leukoencephalopathy syndrome caused by sunitinib was suspected. In addition to the immediate discontinuation of sunitinib therapy, the administration of antihypertensive agents and anticonvulsants improved the clinical symptoms without neurological damage. Physicians should be aware that sunitinib causes reversible posterior leukoencephalopathy syndrome. The early recognition of reversible posterior leukoencephalopathy syndrome is critical to avoid irreversible neurological damage.

  6. Virion composition and genomics of white spot syndrome virus of shrimp

    NARCIS (Netherlands)

    Hulten, van M.C.W.

    2001-01-01


    Since its first discovery in Taiwan in 1992, White spot syndrome virus (WSSV) has caused major economic damage to shrimp culture. The virus has spread rapidly through Asia and reached the Western Hemisphere in 1995 (Texas), where it continued its devastating effect fu

  7. Virion composition and genomics of white spot syndrome virus of shrimp

    NARCIS (Netherlands)

    Hulten, van M.C.W.

    2001-01-01


    Since its first discovery in Taiwan in 1992, White spot syndrome virus (WSSV) has caused major economic damage to shrimp culture. The virus has spread rapidly through Asia and reached the Western Hemisphere in 1995 (Texas), where it continued its devastating effect

  8. Three functionally diverged major White Spot Syndrome Virus structural proteins evolved by gene duplication

    NARCIS (Netherlands)

    Hulten, van M.C.W.; Goldbach, R.W.; Vlak, J.M.

    2000-01-01

    White spot syndrome virus (WSSV) is an invertebrate virus causing considerable mortality in penaeid shrimp. The oval-to-bacilliform shaped virions, isolated from infected Penaeus monodon, contain four major proteins: VP28, VP26, VP24 and VP19 (28, 26, 24 and 19 kDa, respectively). VP26 and VP24 are

  9. Virion composition and genomics of white spot syndrome virus of shrimp

    NARCIS (Netherlands)

    Hulten, van M.C.W.

    2001-01-01

    Since its first discovery in Taiwan in 1992, White spot syndrome virus (WSSV) has caused major economic damage to shrimp culture. The virus has spread rapidly through Asia and reached the Western Hemisphere in 1995 (Texas), where it continued its devastating effect further into Central- and South-Am

  10. Severe fever with thrombocytopenia syndrome virus in ticks collected from humans, South Korea, 2013.

    Science.gov (United States)

    Yun, Seok-Min; Lee, Wook-Gyo; Ryou, Jungsang; Yang, Sung-Chan; Park, Sun-Whan; Roh, Jong Yeol; Lee, Ye-Ji; Park, Chan; Han, Myung Guk

    2014-08-01

    We investigated the infection rate for severe fever with thrombocytopenia syndrome virus (SFTSV) among ticks collected from humans during May-October 2013 in South Korea. Haemaphysalis longicornis ticks have been considered the SFTSV vector. However, we detected the virus in H. longicornis, Amblyomma testudinarium, and Ixodes nipponensis ticks, indicating additional potential SFTSV vectors.

  11. Unusual Presentation of Gianotti-Crosti Syndrome due to Epstein-Barr Virus Infection.

    Science.gov (United States)

    Al Dhaheri, Hind Saif; Al Kaabi, Amani; Kara Hamo, Yasmin; Al Kaabi, Aysha; Al Kaabi, Salwa; Al Tatari, Hossam

    2016-01-01

    Gianotti-Crosti syndrome (GCS) is viral exanthema of childhood. It typically presents with a symmetric erythematous papular and papulovesicular eruption. It has been classically associated with hepatitis B virus, as well as rarely with Epstein-Barr virus (EBV). We report a case of GCS related to EBV infection without the classical systemic symptoms in a five-year-old male patient.

  12. Two White Spot Syndrome Virus MicroRNAs Target the Dorsal Gene To Promote Virus Infection in Marsupenaeus japonicus Shrimp

    Science.gov (United States)

    Ren, Qian; Huang, Xin; Cui, Yalei; Sun, Jiejie; Wang, Wen

    2017-01-01

    ABSTRACT In eukaryotes, microRNAs (miRNAs) serve as regulators of many biological processes, including virus infection. An miRNA can generally target diverse genes during virus-host interactions. However, the regulation of gene expression by multiple miRNAs has not yet been extensively explored during virus infection. This study found that the Spaztle (Spz)-Toll-Dorsal-antilipopolysaccharide factor (ALF) signaling pathway plays a very important role in antiviral immunity against invasion of white spot syndrome virus (WSSV) in shrimp (Marsupenaeus japonicus). Dorsal, the central gene in the Toll pathway, was targeted by two viral miRNAs (WSSV-miR-N13 and WSSV-miR-N23) during WSSV infection. The regulation of Dorsal expression by viral miRNAs suppressed the Spz-Toll-Dorsal-ALF signaling pathway in shrimp in vivo, leading to virus infection. Our study contributes novel insights into the viral miRNA-mediated Toll signaling pathway during the virus-host interaction. IMPORTANCE An miRNA can target diverse genes during virus-host interactions. However, the regulation of gene expression by multiple miRNAs during virus infection has not yet been extensively explored. The results of this study indicated that the shrimp Dorsal gene, the central gene in the Toll pathway, was targeted by two viral miRNAs during infection with white spot syndrome virus. Regulation of Dorsal expression by viral miRNAs suppressed the Spz-Toll-Dorsal-ALF signaling pathway in shrimp in vivo, leading to virus infection. Our study provides new insight into the viral miRNA-mediated Toll signaling pathway in virus-host interactions. PMID:28179524

  13. Distal renal tubular acidosis without renal impairment after use of tenofovir: a case report

    OpenAIRE

    2016-01-01

    Background Tenofovir, one of antiretroviral medication to treat human immunodeficiency virus (HIV) infection, is known to cause proximal renal tubular acidosis such as Fanconi syndrome, but cases of distal renal tubular acidosis had never been reported. Case presentation A 20-year-old man with HIV infection developed nausea and vomiting without diarrhea after starting antiretroviral therapy. Arterial blood gas revealed non-anion-gap metabolic acidosis and urine test showed positive urine anio...

  14. Spontaneous Dissection of the Renal Artery in Vascular Ehlers-Danlos Syndrome

    Science.gov (United States)

    Pereira, Filipa; Cardoso, Teresa; Sá, Paula

    2015-01-01

    Ehlers-Danlos syndrome (EDS) is a rare heterogeneous group of connective tissue disorders. The vascular type (vEDS) is an autosomal dominant disorder caused by heterozygous mutations in the COL3A1 gene predisposing to premature arterial, intestinal, or uterine rupture. We report a case of a 38-year-old woman with a recent diagnosis of vEDS admitted in the Emergency Department with a suspicion of a pyelonephritis that evolved to a cardiopulmonary arrest. A fatal retroperitoneal hematoma related with a haemorrhagic dissection of the right renal artery was found after emergency surgery. This case highlights the need to be aware of the particular characteristics of vEDS, such as a severe vascular complication that can lead to a fatal outcome. PMID:26175915

  15. Nephrocalcinosis (Enamel Renal Syndrome) Caused by Autosomal Recessive FAM20A Mutations

    Science.gov (United States)

    Jaureguiberry, Graciana; De la Dure-Molla, Muriel; Parry, David; Quentric, Mickael; Himmerkus, Nina; Koike, Toshiyasu; Poulter, James; Klootwijk, Enriko; Robinette, Steven L.; Howie, Alexander J.; Patel, Vaksha; Figueres, Marie-Lucile; Stanescu, Horia C.; Issler, Naomi; Nicholson, Jeremy K.; Bockenhauer, Detlef; Laing, Christopher; Walsh, Stephen B.; McCredie, David A.; Povey, Sue; Asselin, Audrey; Picard, Arnaud; Coulomb, Aurore; Medlar, Alan J.; Bailleul-Forestier, Isabelle; Verloes, Alain; Le Caignec, Cedric; Roussey, Gwenaelle; Guiol, Julien; Isidor, Bertrand; Logan, Clare; Shore, Roger; Johnson, Colin; Inglehearn, Christopher; Al-Bahlani, Suhaila; Schmittbuhl, Matthieu; Clauss, François; Huckert, Mathilde; Laugel, Virginie; Ginglinger, Emmanuelle; Pajarola, Sandra; Spartà, Giuseppina; Bartholdi, Deborah; Rauch, Anita; Addor, Marie-Claude; Yamaguti, Paulo M.; Safatle, Heloisa P.; Acevedo, Ana Carolina; Martelli-Júnior, Hercílio; dos Santos Netos, Pedro E.; Coletta, Ricardo D.; Gruessel, Sandra; Sandmann, Carolin; Ruehmann, Denise; Langman, Craig B.; Scheinman, Steven J.; Ozdemir-Ozenen, Didem; Hart, Thomas C.; Hart, P. Suzanne; Neugebauer, Ute; Schlatter, Eberhard; Houillier, Pascal; Gahl, William A.; Vikkula, Miikka; Bloch-Zupan, Agnès; Bleich, Markus; Kitagawa, Hiroshi; Unwin, Robert J.; Mighell, Alan; Berdal, Ariane; Kleta, Robert

    2013-01-01

    Background/Aims Calcium homeostasis requires regulated cellular and interstitial systems interacting to modulate the activity and movement of this ion. Disruption of these systems in the kidney results in nephrocalcinosis and nephrolithiasis, important medical problems whose pathogenesis is incompletely understood. Methods We investigated 25 patients from 16 families with unexplained nephrocalcinosis and characteristic dental defects (amelogenesis imperfecta, gingival hyperplasia, impaired tooth eruption). To identify the causative gene, we performed genome-wide linkage analysis, exome capture, next-generation sequencing, and Sanger sequencing. Results All patients had bi-allelic FAM20A mutations segregating with the disease; 20 different mutations were identified. Conclusions This au-tosomal recessive disorder, also known as enamel renal syndrome, of FAM20A causes nephrocalcinosis and amelogenesis imperfecta. We speculate that all individuals with biallelic FAM20A mutations will eventually show nephrocalcinosis. PMID:23434854

  16. Antithymocyte globulin-induced acute respiratory distress syndrome after renal transplantation: a case report

    Institute of Scientific and Technical Information of China (English)

    TU Guo-wei; JU Min-jie; XU Ming; RONG Rui-ming; ZHU Tong-yu; LUO Zhe

    2012-01-01

    Antithymocyte globulin (ATG) has long been used for immune-induction and anti-rejection treatments for solid organ transplantations.To date,few cases of ATG-induced acute respiratory distress syndrome (ARDS) have been published.Here,we present a case of ARDS caused by a single low-dose of ATG in a renal transplant recipient and the subsequent treatments administered.Although the patient suffered from ARDS and delayed graft function,he was successfully treated.We emphasize that the presence of such complications should be considered when unexplained respiratory distress occurs.Early use of corticosteroids,adjustment of immunosuppressive regimens,and conservative fluid management,as well as empiric antimicrobial therapies,may be effective strategies for the treatment of ARDS caused by ATG.

  17. Spontaneous Dissection of the Renal Artery in Vascular Ehlers-Danlos Syndrome

    Directory of Open Access Journals (Sweden)

    Filipa Pereira

    2015-01-01

    Full Text Available Ehlers-Danlos syndrome (EDS is a rare heterogeneous group of connective tissue disorders. The vascular type (vEDS is an autosomal dominant disorder caused by heterozygous mutations in the COL3A1 gene predisposing to premature arterial, intestinal, or uterine rupture. We report a case of a 38-year-old woman with a recent diagnosis of vEDS admitted in the Emergency Department with a suspicion of a pyelonephritis that evolved to a cardiopulmonary arrest. A fatal retroperitoneal hematoma related with a haemorrhagic dissection of the right renal artery was found after emergency surgery. This case highlights the need to be aware of the particular characteristics of vEDS, such as a severe vascular complication that can lead to a fatal outcome.

  18. Metabolic acidosis and malnutrition-inflammation complex syndrome in chronic renal failure.

    Science.gov (United States)

    Kalantar-Zadeh, Kamyar; Mehrotra, Rajnish; Fouque, Denis; Kopple, Joel D

    2004-01-01

    Metabolic acidosis, a common condition in patients with renal failure, may be linked to protein-energy malnutrition (PEM) and inflammation, together also known as malnutrition-inflammation complex syndrome (MICS). Methods of serum bicarbonate measurement may misrepresent the true bicarbonate level, since the total serum carbon dioxide measurement usually overestimates the serum bicarbonate concentration. Moreover, the air transportation of blood samples to distant laboratories may lead to erroneous readings. In patients with chronic kidney disease (CKD) or end-stage renal disease (ESRD), a significant number of endocrine, musculoskeletal, and metabolic abnormalities are believed to result from acidemia. Metabolic acidosis may be related to PEM and MICS due to an increased protein catabolism, decreased protein synthesis, endocrine abnormalities including insulin resistance, decreased serum leptin level, and inflammation among individuals with renal failure. Evidence suggests that the catabolic effects of metabolic acidosis may result from an increased activity of the adenosine triphosphate (ATP)-dependent ubiquitin-proteasome and branched-chain keto acid dehydrogenase. In contrast to the metabolic studies, many epidemiologic studies in maintenance dialysis patients have indicated a paradoxically inverse association between mildly decreased serum bicarbonate and improved markers of protein-energy nutritional state. Hence metabolic acidosis may be considered as yet another element of the reverse epidemiology in ESRD patients. Interventional studies have yielded inconsistent results in CKD and ESRD patients, although in peritoneal dialysis patients, mitigating acidemia appears to more consistently improve nutritional status and reduce hospitalizations. Large-scale, prospective randomized interventional studies are needed to ascertain the potential benefits of correcting acidemia in malnourished and/or inflamed CKD and maintenance hemodialysis patients. Until then, all

  19. Syndrome of rapid onset end stage renal disease in incident Mayo Clinic chronic hemodialysis patient

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    M. A. C. Onuigbo

    2014-01-01

    Full Text Available Despite decades of research, a full understanding of chronic kidney disease (CKD-end stage renal disease (ESRD progression remains elusive. The common consensus is a predictable, linear, progressive and time-dependent decline of CKD to ESRD. Acute kidney injury (AKI on CKD is usually assumed to be transient, with recovery as the expected outcome. AKI-ESRD association in current nephrology literature is blamed on the so-called "residual confounding." We had previously described a relationship between AKI events and rapid onset yet irreversible ESRD happening in a continuum in a high-risk CKD cohort. However, the contribution of the syndrome of rapid onset-ESRD (SORO-ESRD to incident United States ESRD population remained conjectural. In this retrospective analysis, we analyzed serum creatinine trajectories of the last 100 consecutive ESRD patients in 4 Mayo Clinic chronic hemodialysis units to determine the incidence of SORO-ESRD. Excluding 9 patients, 31 (34% patients, including two renal transplant recipients, had SORO-ESRD: 18 males and 13 females age 72 (range 50-92 years. Precipitating AKI followed pneumonia (8, acutely decompensated heart failure (7, pyelonephritis (4, post-operative (5, sepsis (3, contrast-induced nephropathy (2, and others (2. Time to dialysis was shortest following surgical procedures. Concurrent renin angiotensin aldosterone system blockade was higher with SORO-ESRD - 23% versus 5%, P = 0.0113. In conclusion, SORO-ESRD is not uncommon among the incident general US ESRD population. The implications for ESRD care planning, AV-fistula-first programs, general CKD care and any associations with renal ageing/senescence warrant further study.

  20. Hepatitis B Virus Infection and the Kidney: Renal Abnormalities in HBV Patients, Antiviral Drugs Handling, and Specific Follow-Up

    Directory of Open Access Journals (Sweden)

    Gilbert Deray

    2015-01-01

    Full Text Available Chronic hepatitis B virus (CHB infection is one of the most common causes of chronic liver disease, cirrhosis, and hepatocellular carcinoma (HCC worldwide. Many patients with CHB have variable degrees of functional renal impairment, and approximately 2 to 15% of patients on hemodialysis have CHB. Several therapeutic regimens have been developed in the past years, among which oral nucleoside and nucleotide analogues have been demonstrated to be efficient and well tolerated. However, they all are excreted in the urine and may thus require dosage adjustment in patients with decreased renal function. Furthermore, a number of them may in addition be toxic to the kidneys, especially in those patients presenting with renal insufficiency.

  1. Parainfluenza virus infection associated with posterior reversible encephalopathy syndrome: a case report

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    Ogunneye Owolabi

    2012-03-01

    Full Text Available Abstract Introduction Posterior reversible encephalopathy syndrome is a clinical and radiological entity. The most accepted theory of posterior reversible encephalopathy syndrome is a loss of autoregulation in cerebral blood flow with a subsequent increase in vascular permeability and leakage of blood plasma and erythrocytes, producing vasogenic edema. In infection-associated posterior reversible encephalopathy syndrome, a clinical pattern consistent with systemic inflammatory response syndrome develops. Parainfluenza virus has not been reported in the medical literature to be associated with posterior reversible encephalopathy syndrome. Case presentation We report herein the case of a 54-year-old Caucasian woman with posterior reversible encephalopathy syndrome associated with parainfluenza virus infection who presented with generalized headache, blurring of vision, new-onset seizure and flu-like symptoms. Conclusion Infection-associated posterior reversible encephalopathy syndrome as well as hypertension-associated posterior reversible encephalopathy syndrome favor the contribution of endothelial dysfunction to the pathophysiology of this clinicoradiological syndrome. In view of the reversible nature of this clinical entity, it is important that all physicians are well aware of posterior reversible encephalopathy syndrome in patients presenting with headache and seizure activity. A detailed clinical assessment leading to the recognition of precipitant factors in posterior reversible encephalopathy syndrome is paramount.

  2. Clinical management of restless legs syndrome in end-stage renal disease patients.

    Science.gov (United States)

    Sahli, Zeyad T; Jo, Jae; Mousa, Shaker A; Tarazi, Frank I

    2017-02-01

    Restless legs syndrome (RLS) is a common neurological movement disorder, characterized by restless and unpleasant sensations in the deep inside of legs. The symptoms of RLS are less noticeable during daytime, but more prevalent at night. Therefore, the disorder can induce low quality of life, insomnia, and impairment of daytime activity. RLS in end-stage renal disease (ESRD) patients is especially problematic due to premature discontinuation of dialysis and increased mortality. The prevalence of RLS among dialysis patients is much higher compared to the prevalence of the same disorder in patients with normal renal functions. Even though there are recommended treatment guidelines for the general population established by Medical Advisory Board of the RLS foundation, which include the use of dopamine agonists, levodopa, gabapentin, benzodiazepines, and opioids, limited information is available on the effects of these therapies in ESRD patients. Since the existing clinical data were extrapolated from small sample sizes in short-term clinical trials, further clinical studies are still needed to better assess the efficacy, safety, and tolerability of these medications in patients with ESRD.

  3. Acute respiratory distress syndrome and acute renal failure from Plasmodium ovale infection with fatal outcome.

    Science.gov (United States)

    Lau, Yee-Ling; Lee, Wenn-Chyau; Tan, Lian-Huat; Kamarulzaman, Adeeba; Syed Omar, Sharifah Faridah; Fong, Mun-Yik; Cheong, Fei-Wen; Mahmud, Rohela

    2013-11-04

    Plasmodium ovale is one of the causative agents of human malaria. Plasmodium ovale infection has long been thought to be non-fatal. Due to its lower morbidity, P. ovale receives little attention in malaria research. Two Malaysians went to Nigeria for two weeks. After returning to Malaysia, they fell sick and were admitted to different hospitals. Plasmodium ovale parasites were identified from blood smears of these patients. The species identification was further confirmed with nested PCR. One of them was successfully treated with no incident of relapse within 12-month medical follow-up. The other patient came down with malaria-induced respiratory complication during the course of treatment. Although parasites were cleared off the circulation, the patient's condition worsened. He succumbed to multiple complications including acute respiratory distress syndrome and acute renal failure. Sequencing of the malaria parasite DNA from both cases, followed by multiple sequence alignment and phylogenetic tree construction suggested that the causative agent for both malaria cases was P. ovale curtisi. In this report, the differences between both cases were discussed, and the potential capability of P. ovale in causing severe complications and death as seen in this case report was highlighted. Plasmodium ovale is potentially capable of causing severe complications, if not death. Complete travel and clinical history of malaria patient are vital for successful diagnoses and treatment. Monitoring of respiratory and renal function of malaria patients, regardless of the species of malaria parasites involved is crucial during the course of hospital admission.

  4. Immunohistochemical diagnosis of Alport's syndrome in paraffin-embedded renal sections: antigen retrieval with autoclave heating.

    Science.gov (United States)

    Naito, Ichiro; Ninomiya, Yoshifumi; Nomura, Shinsuke

    2003-03-01

    Alport's syndrome (AS) is a hereditary renal disease caused by mutations in the genes encoding collagen type IV. Immunohistochemical analysis of the alpha chains of collagen type IV has been found to be useful for the diagnosis of this disease. The monoclonal antibodies (mAbs) generated by us recognize alpha 1(IV) through alpha 6(IV) chains of collagen type IV on fresh-frozen sections but not on paraffin-embedded sections. Antigen retrieval by autoclave heating has been found to restore the epitopes recognized by the mAbs; however the heating conditions had not been well established. In this study, the heating conditions were carefully examined using renal sections obtained from AS and non-AS patients. The heating was performed in an autoclave, at 105 degrees -127 degrees C for 6-8 min. During the heating, the sections were immersed in 0.2 N HCl solution (pH 0.9). Then, the mAbs were applied for 30 min, and the bound mAbs were detected using the LSAB kit. The optimal temperature for the antigen retrieval varied among specimens, and was dependent on the type of basement membrane examined. Thus, it was considered that heating at two or three different temperatures could be helpful for the precise diagnosis of AS. Adopting the antigen retrieval method could extend the possibility of immunohistochemical diagnosis of AS to cases without using fresh-frozen sections.

  5. FAM20A mutations can cause enamel-renal syndrome (ERS.

    Directory of Open Access Journals (Sweden)

    Shih-Kai Wang

    Full Text Available Enamel-renal syndrome (ERS is an autosomal recessive disorder characterized by severe enamel hypoplasia, failed tooth eruption, intrapulpal calcifications, enlarged gingiva, and nephrocalcinosis. Recently, mutations in FAM20A were reported to cause amelogenesis imperfecta and gingival fibromatosis syndrome (AIGFS, which closely resembles ERS except for the renal calcifications. We characterized three families with AIGFS and identified, in each case, recessive FAM20A mutations: family 1 (c.992G>A; g.63853G>A; p.Gly331Asp, family 2 (c.720-2A>G; g.62232A>G; p.Gln241_Arg271del, and family 3 (c.406C>T; g.50213C>T; p.Arg136* and c.1432C>T; g.68284C>T; p.Arg478*. Significantly, a kidney ultrasound of the family 2 proband revealed nephrocalcinosis, revising the diagnosis from AIGFS to ERS. By characterizing teeth extracted from the family 3 proband, we demonstrated that FAM20A(-/- molars lacked true enamel, showed extensive crown and root resorption, hypercementosis, and partial replacement of resorbed mineral with bone or coalesced mineral spheres. Supported by the observation of severe ectopic calcifications in the kidneys of Fam20a null mice, we conclude that FAM20A, which has a kinase homology domain and localizes to the Golgi, is a putative Golgi kinase that plays a significant role in the regulation of biomineralization processes, and that mutations in FAM20A cause both AIGFS and ERS.

  6. Intrahepatic cytokine profile in renal-transplant patients infected by hepatitis C virus.

    Science.gov (United States)

    Kamar, Nassim; Rostaing, Lionel; Sandres-Saune, Karine; Selves, Janick; Barthe, Carole; Dubois, Martine; Alric, Laurent; Durand, Dominique; Izopet, Jacques

    2005-08-01

    In order to examine the immunopathogenesis of hepatitis C virus (HCV)-related liver injury in renal-transplant patients, intra-hepatic cytokine profiles were examined in 38 liver biopsies from 38 patients by measuring messenger RNA (mRNA) concentrations by a real-time PCR method of a Th1 cytokine (i.e., interferon (IFN)-gamma), a Th2 cytokines (i.e., interleukine (IL)-10), a proinflammatory cytokine (i.e., IL-8), and a potent fibrogenic factor (transforming growth factor [TGF]-beta). There was no significant difference in TGF-beta, IFN-gamma, IL-10, or IL-8 levels of expression according to liver-activity grade, liver-fibrosis stage, the concentration of HCV RNA at liver biopsies, or the HCV genotype. However, IFN-gamma/beta-actin mRNA concentration was higher than the IL-10/beta-actin mRNA concentration in patients with F3 Metavir score. Median IFN-gamma/beta-actin mRNA concentration tended to be higher in patients with A3 and A4 Metavir activity grades compared with those with A0 and A1 activity grades. There was a significant correlation between the duration of HCV infection and both TGF-beta/beta-actin (r(2)=0.19, P=0.04) and IL-8/beta-actin mRNA concentrations (r(2)=0.19, P=0.03). IFN-gamma/beta-actin mRNA concentration also increased according to the duration of HCV (r(2)=0.19, P=0.07). Finally, there was a significant correlation between the duration of HCV infection and liver fibrosis stage (r(2)=0.17, P=0.045). Intrahepatic Th1 cytokine profile seems to be predominant in patients with extensive fibrosis and activity scores, suggesting that it might be responsible for liver injury in renal transplant patients.

  7. [Hepatitis B virus in patients with renal transplant. Report of 52 cases].

    Science.gov (United States)

    Duncan, C R; Virginillo, M B; Palmitano, J B; Tani, R D; Aguirre, C

    1990-01-01

    Hepatitis B virus (HBV) is a high risk factor in the frequently found liver involvement of renal transplant recipients. As in other immunosuppressed patients, these often follow a course of slight jaundice, with a progressive tendency and great replicative and infectious power. Also, in addition to an increased incidence of chronic hepatitis (CH) in transplanted when compared with hemodialized patients, specially when HBsAg is present, it is surprising the poor correlation between enzyme levels and the grade of activity of the hepatic lesion. In a retro and prospective study, we present 52 patients of the 73 transplant reviewed. There are 32 men and 20 females, with and average age of 34 years, minimum time on dialysis of 2 months and maximum of 7 years, time of renal transplant from 6 moth to 15 years (average 4.9 years). There were 31 cadaver transplants and 21 live donors. The HBsAg was + in 20 (9 seroconverted), HBeAg was + in 4 (with 2 seroconvertions), hyperbilirrubinemia in 5, hyperalkaline phosphatasemia (2 or more times) in 11 and elevated serum transaminases (SGPT) (3 times or more) in 20 cases. Positive HBsAg plus SGPT x 3 was found on 9 occasions and positive HBsAg with SGPT x 3 in 3 cases. liver biopsy (LB), in those with enzymatic changes and/or positive antigenemia, was performed in 15 instances and there were 5 autopsies. The most important histological findings were: 5 acute viral hepatitis, 2 active chronic hepatitis (CAH), 2 persistent chronic hepatitis (CPH), 5 with fat infiltration and 4 with colestasis.(ABSTRACT TRUNCATED AT 250 WORDS)

  8. Elevated soluble CD163 plasma levels are associated with disease severity in patients with hemorrhagic fever with renal syndrome.

    Directory of Open Access Journals (Sweden)

    Junning Wang

    Full Text Available Hantaan virus is a major zoonotic pathogen that causesing hemorrhagic fever with renal syndrome (HFRS. Although HFRS pathogenesis has not been entirely elucidated, the importance of host-related immune responses in HFRS pathogenesis has been widely recognized. CD163, a monocyte and macrophage-specific scavenger receptor that plays a vital function in the hosts can reduce inflammation, is shed during activation as soluble CD163 (sCD163. The aim of this study was to investigate the pathological significance of sCD163 in patients with HFRS.Blood samples were collected from 81 hospitalized patients in Tangdu Hospital from October 2011 to January 2014 and from 15 healthy controls. The sCD163 plasma levels were measured using a sandwich ELISA, and the relationship between sCD163 and disease severity was analyzed. Furthermore, CD163 expression in 3 monocytes subset was analyzed by flow cytometry.The results demonstrated that sCD163 plasma levels during the HFRS acute phase were significantly higher in patients than during the convalescent stage and the levels in the healthy controls (P<0.0001. The sCD163 plasma levels in the severe/critical group were higher than those in the mild/moderate group during the acute (P<0.0001. A Spearman correlation analysis indicated that the sCD163 levels were positively correlated with white blood cell, serum creatine, blood urea nitrogen levels, while they were negatively correlated with blood platelet levels in the HFRS patients. The monocyte subsets were significantly altered during the acute stage. Though the CD163 expression levels within the monocyte subsets were increased during the acute stage, the highest CD163 expression level was observed in the CD14++CD16+ monocytes when compared with the other monocyte subsets.sCD163 may be correlated with disease severity and the disease progression in HFRS patients; however, the underlying mechanisms should be explored further.

  9. Xenotropic Murine Leukemia Virus-Related Virus in Monozygotic Twins Discordant for Chronic Fatigue Syndrome

    Science.gov (United States)

    Jerome, Keith R.; Diem, Kurt; Huang, Meei-Li; Selke, Stacy; Corey, Lawrence; Buchwald, Dedra

    2011-01-01

    A recent report suggested an association between xenotropic murine leukemia virus-related virus (XMRV) and chronic fatigue syndrome (CFS). If confirmed, this would suggest that antiretroviral therapy might benefit patients suffering from CFS. We validated a set of assays for XMRV, and evaluated the prevalence of XMRV in a cohort of monozygotic twins discordant for CFS. Stored PBMC were tested with 3 separate PCR assays (one of which was nested) for XMRV DNA, and serum/plasma was tested for XMRV RNA by reverse transcription (RT)-PCR. None of the PBMC samples from the twins with CFS or their unaffected co-twins were positive for XMRV, by any of the assays. One plasma sample, from an unaffected co-twin, was reproducibly positive by RT-PCR. However, serum from the same day was negative, as was a followup plasma sample obtained 2 days after the positive specimen. These data do not support an association of XMRV with CFS. PMID:21795004

  10. Middle east respiratory syndrome coronavirus spike protein delivered by modified vaccinia virus ankara efficiently induces virus-neutralizing antibodies

    NARCIS (Netherlands)

    F. Song (Fei); R. Fux (Robert); L.B.V. Provacia (Lisette); A. Volz (Asisa); M. Eickmann; S. Becker (Stephan); A.D.M.E. Osterhaus (Albert); B.L. Haagmans (Bart); G. Suttera (Gerd)

    2013-01-01

    textabstractMiddle East respiratory syndrome coronavirus (MERS-CoV) has recently emerged as a causative agent of severe respiratory disease in humans. Here, we constructed recombinant modified vaccinia virus Ankara (MVA) expressing full-length MERS-CoV spike (S) protein (MVA-MERS-S). The genetic sta

  11. Posterior Nutcracker Syndrome with Left Renal Vein Duplication: A Rare Cause of Haematuria in a 12-Year-Old Boy

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    J. Preza Fernandes

    2012-01-01

    Full Text Available The nutcracker syndrome (NCS is a rare cause of haematuria. It embraces an extended nonpathognomonic spectrum of symptoms that imply a difficult diagnosis. Ultimately it may be associated with substantial morbidity and even life-threatening events. We report a rare cause if a 12-year-old boy who presented with a history of frequent intermittent episodes of painless constant haematuria. The cystoscopy showed a bloody urine ejaculate from the left ureter meatus. The Doppler ultrasonography showed turbulent pattern of venous blood flow of the posterior renal vein branch behind the aorta. The abdominopelvic computer tomography (apCT revealed left renal vein (LRV duplication with a dilated retroaortic branch, entrapped between the aorta and the vertebral column, promoting the renal nutcracker syndrome. The patient was initially hospitalized and managed with oral iron supplements and continuous saline bladder irrigation, not requiring additional treatment. The child is currently asymptomatic, with haemoglobin value returning to normal and therefore proposed to conservative management with close followup. The authors present a case report of episodic haematuria caused by a rare entity—posterior nutcracker syndrome with renal vein duplication.

  12. Clinical features of acute renal failure associated with hepatitis A virus infection.

    Science.gov (United States)

    Jung, Y J; Kim, W; Jeong, J B; Kim, B G; Lee, K L; Oh, K-H; Yoon, J-H; Lee, H-S; Kim, Y J

    2010-09-01

    Acute hepatitis A (AHA) is one of the most common infectious diseases; it is usually a self-limiting disease affecting the liver. Although extrahepatic manifestations are not common, some cases have been reported associated with acute renal failure. We reviewed the clinical features of patients with AHA complicated by acute renal failure (ARF group) and compared them with patients with noncomplicated AHA (non-ARF group). The medical records of 208 consecutive patients with AHA who were diagnosed between January 2003 and October 2008 were reviewed. We identified 15 patients (7.2%) with ARF associated with AHA. There were no differences between the ARF and non-ARF group with regard to gender and age. The peak value of alanine aminotransferase (ALT) (median: 6060 IU/L vs 1792 IU/L, P hepatic failure, and two patients died because of fulminant hepatic failure. There were no deaths among patients with noncomplicated AHA in the non-ARF group. Five patients underwent kidney biopsy; two patients were diagnosed with acute tubular necrosis, two patients with acute interstitial nephritis with IgA nephropathy and one patient with acute tubulointerstitial nephritis. All patients in the ARF group had microscopic haematuria and proteinuria (100%vs 31.1%, P < 0.001). Urine sodium levels were more than 10 mEq/L in 10 patients. The findings of high urinary sodium concentrations, microscopic haematuria and proteinuria did not support the diagnosis of hepatorenal syndrome (HRS). Patients with AHA with ARF had higher ALT levels, more prolonged PTs, and higher total bilirubin levels. The prognosis for these patients was poorer than for those without ARF. However, the patients with ARF and nonfulminant AHA had recovered with proper treatment and should not be confused with patients that have HRS.

  13. Birt-Hogg-Dubé syndrome with a renal angiomyolipoma: further evidence of a relationship between Birt-Hogg-Dubé syndrome and tuberous sclerosis complex.

    Science.gov (United States)

    Byrne, Marguerite; Mallipeddi, Raj; Pichert, Gabriella; Whittaker, Sean

    2012-05-01

    Birt-Hogg-Dubé syndrome (BHDS) is an autosomal dominant condition caused by mutations in the gene which codes for folliculin (FLCN). It is characterised clinically by fibrofolliculomas, trichodiscomas, pulmonary cysts, spontaneous pneumothoraces and renal cancers. This case illustrates a patient with BHDS and a renal angiomyolipoma. Angiomyolipomas are not described as a feature of BHDS, but rather they can occur sporadically or in tuberous sclerosis complex (TSC). Recent studies suggest that clinical similarities between BHDS and TSC may be explained by FLCN and TSC proteins functioning on a common pathway, mammalian target of rapamycin. This case adds to the literature of cases with clinical similarities.

  14. [Middle ear salivary gland choristoma related to branchio-oto-renal syndrome diagnosed by array-CGH].

    Science.gov (United States)

    Amrhein, P; Sittel, C; Spaich, C; Kohlhase, J; Boppert, R; Kohlhof, P; Koitschev, A

    2014-05-01

    Branchio-oto-renal (BOR) syndrome is characterized by ear malformations associated with sensorineural or mixed hearing loss. In addition, preauricular tags, preauricular pits, branchial cleft fistulas and cysts, as well as renal dysplasia are seen. A genetic mutation on chromosome 8, either autosomal dominantly inherited or occuring as a spontaneous mutation, is the cause in the majority of cases. Using array-based comparative genomic hybridization (CGH), it is possible to detect even the smallest genetic changes. Salivary gland choristoma in the middle ear is very rare. Surgical removal and histological clarification are required.

  15. Delusional Infestation in a Patient with Renal Failure, Metabolic Syndrome, and Chronic Cerebrovascular Disease Treated with Aripiprazole: A Case Report

    Science.gov (United States)

    Carpiniello, Bernardo; Pinna, Federica; Tuveri, Raffaella

    2011-01-01

    Delusional infestation is an aspecific psychiatric condition manifested either as a primary psychotic disorder or a secondary disorder induced by a wide range of very different medical conditions. Both primary and secondary delusional infestations seem to respond to typical and atypical antipsychotics. The latter are considered the first-line treatment although the use of second-generation antipsychotics featuring a higher metabolic, cardiovascular, and renal tolerability is preferable in secondary cases, which often occur in patients with multiple, severe medical conditions. We report a case of a 72-year-old patient affected by delusional infestation associated with severe renal failure, metabolic syndrome, hypertensive cardiopathy, and chronic cerebrovascular disease. PMID:22174718

  16. Delusional Infestation in a Patient with Renal Failure, Metabolic Syndrome, and Chronic Cerebrovascular Disease Treated with Aripiprazole: A Case Report

    Directory of Open Access Journals (Sweden)

    Bernardo Carpiniello

    2011-01-01

    Full Text Available Delusional infestation is an aspecific psychiatric condition manifested either as a primary psychotic disorder or a secondary disorder induced by a wide range of very different medical conditions. Both primary and secondary delusional infestations seem to respond to typical and atypical antipsychotics. The latter are considered the first-line treatment although the use of second-generation antipsychotics featuring a higher metabolic, cardiovascular, and renal tolerability is preferable in secondary cases, which often occur in patients with multiple, severe medical conditions. We report a case of a 72-year-old patient affected by delusional infestation associated with severe renal failure, metabolic syndrome, hypertensive cardiopathy, and chronic cerebrovascular disease.

  17. Can Kallmann syndrome be occasionally diagnosed during childhood? Genetic diagnosis in a child with associated renal agenesis and mirror movements

    Institute of Scientific and Technical Information of China (English)

    Neoklis A. Georgopoulos; Vasiliki Koika; Petros Varmavas; Alexandra Efthymiadou; Dimitra J. Marioli; Stefanos Mantagos; Dionysios Chrysis

    2009-01-01

    @@ Dear Sir, I am Neoklis A. Georgopoulos, from Department of Obstetrics and Gynaecology, Division of Reproductive Endocrinology, University of Patras Medical School,Greece. We present an early prepubertal diagnosis of Kallmann syndrome (KS) in a child with anosmia, renal agenesis and mirror movements. KS is a congenital defect characterized by the presence of hypogonadotropic hypogonadism and anosmia or hyposmia [1].Besides hypogonadism and anosmia, the clinical phenotype is characterized by the presence of other associated features, including, among others, mirror movements (synkinesis), cleft palate and renal agenesis/dysgenesis.

  18. A case of Noonan`s syndrome with terminal renal failure and neoplasmic disease; Przypadek zespolu Noonan z niewydolnoscia nerek i rozrostem nowotworowym

    Energy Technology Data Exchange (ETDEWEB)

    Wierzbowska-Lange, B.; Sieniawska, M.; Polanski, J.; Kisiel, M. [Akademia Medyczna, Warsaw (Poland)

    1993-12-31

    A 16 year old boy with Noonan syndrome diagnosed in the 3rd month of life was treated for renal insufficiency and hepatic tumor. Hemodialisis was started in preparation for surgery. Exacerbation of renal insufficiency occurred after surgical removal of the hepatic tumor together with the right hepatic lobe. Histopathologic examination showed adenoma hepatocellulare. Cysts in the extraperitoneal space appeared in the following months and were identified as cystes dysontogenetici benigni ``hamartia``. Renal function deteriorated during this time to end stage renal disease. The authors emphasize the possibility of good mental development in a patients with Noonan syndrome as well as the presence of urinary tract abnormalities and renal insufficiency of unknown origin - a phenomenon that has not yet been reported in other cases of Noonan syndrome. (author) 11 refs, 1 fig

  19. Posterior Reversible Encephalopathy Syndrome in Two Omani Children with Underlying Renal Diseases

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    Mohamed A. El-Naggari

    2015-08-01

    Full Text Available Posterior reversible encephalopathy syndrome (PRES is a neurological condition with a combination of clinical and radiological features. Clinical symptoms include headaches, confusion, seizures, disturbed vision or an altered level of consciousness. Classic magnetic resonance imaging (MRI findings indicate subcortical and cortical oedema, affecting mainly the posterior cerebral region. We report two paediatric cases of PRES with underlying renal diseases presenting at the Sultan Qaboos University Hospital in Muscat, Oman, in April 2010 and August 2011. The first case was an 11-year-old girl diagnosed with systemic lupus erythematosus and the second was a six-and-a-half-year-old boy on peritoneal dialysis due to multi-drug-resistant nephrotic syndrome. Both patients were hypertensive and treated with blood pressure control medications. No residual neurological dysfunction was noted in the patients at a one-year follow-up and at discharge, respectively. The role of hypertension in paediatric PRES cases, among other important risk factors, is emphasised. Additionally, MRI is an important diagnostic and prognostic tool. Prompt diagnosis and aggressive management is fundamental to preventing permanent neurological damage.

  20. Development of a Liquid Chip Technique to Simultaneously Detect Taura Syndrome Virus( TSV) and Yellow Head Disease Virus( YHDV)

    Institute of Scientific and Technical Information of China (English)

    Yin; Weili; Zhang; Sihua; Yue; Zhiqin; Zheng; Xiaolong; Liu; Hong

    2014-01-01

    The aim is to develop a liquid chip technique to detect Taura syndrome virus( TSV) and yellow head disease virus( YHDV) on Penaeus orientalis simultaneously. The CP2 gene of TSV and N gene of YHDV in Gen Bank was analysed by using the software DNAStar 7. 0 to design the TSV-and YHDV-specific primers. The primers were labeled with biotin and subjected to amination modification. They were then coupled with fluorescence-coded microspheres and then used for hybridization with RT- PCR products of TSV and YHDV. The liquid chip detection technique for detection of TSV and YHDV was established by using BD FACSArray to detect fluorescence signal in the reaction system. This assay system had a high sensitivity to TSV and YHDV,with the detection of limit of 100 pg. Moreover,the assay was specific for the detection of TSV,YHDV and was not susceptible to cross with other viruses,including white spot syndrome virus( WSSV),spring viremia of carp virus( SVCV),infectious haematopoietic necrosis virus( IHNV). In conclusion,the liquid chip assay technique established in this study is highly sensitive and specific to TSV and YHDV detection. Moreover,it provides a novel,convenient and rapid approach for the detection of TSV and YHDV.

  1. Intratumoral peripheral small papillary tufts: a diagnostic clue of renal tumors associated with Birt-Hogg-Dubé syndrome.

    Science.gov (United States)

    Kuroda, Naoto; Furuya, Mitsuko; Nagashima, Yoji; Gotohda, Hiroko; Moritani, Suzuko; Kawakami, Fumi; Imamura, Yoshiaki; Bando, Yoshimi; Takahashi, Masayuki; Kanayama, Hiro-omi; Ota, Satoshi; Michal, Michal; Hes, Ondrej; Nakatani, Yukio

    2014-06-01

    In this article, we searched for the common histologic characteristic of renal tumors in patients with Birt-Hogg-Dubé syndrome (BHDS). We selected 6 patients with histologically confirmed renal tumor in BHDS. Germline FLCN gene mutation has been identified in 5 patients. Multifocality and bilaterality of the renal tumors were pathologically or radiologically confirmed in 5 and 2 cases, respectively. Histologic subtypes of the dominant tumor included 3 previously described hybrid oncocytic tumors, one composite chromophobe/papillary/clear cell renal cell carcinoma (RCC) and one unclassified RCC resembling hybrid chromophobe/clear cell RCC. In one case, chromophobe RCC and clear cell RCC were separately observed. Small papillary lesions located in the peripheral area of the tumor, which we designated as intratumoral peripheral small papillary tufts, were identified in all patients. In conclusion, multifocality/bilaterality of renal tumors, discordance of histologic subtypes, and the presence of intratumoral peripheral small papillary tufts may be important clues to identify BHDS-associated renal tumors.

  2. A single-center epidemiological study of BK virus infection and analysis of risk factors in patients with renal transplantation

    Directory of Open Access Journals (Sweden)

    Ji-gang LI

    2014-10-01

    Full Text Available Objective To investigate the epidemiological characteristics of BK virus (BKV infection in living renal transplantation patients, and analyze the risk factors of BKV infection and BKV nephropathy (BKVN. Methods The BKV DNA load in urine and blood samples of 43 renal transplant recipients, who had received renal transplantation in 309 Hospital from Feb. 2012 to Feb. 2013, was determined at preoperative period and 0.5, 1, 3, 6, 9, 12 and 15 months after transplantation. Meanwhile, the biopsy of grafted kidney was performed in those patients with continuously elevated serum creatinine and those with higher BKV DNA load. Patients were divided into 3 groups as follows according to the test results: BK viruria group, BK viremia group and pathologically diagnosed BKVN group. Data of each group were then recorded, including gender, age, postoperative diabetes (PTDM, acute rejection (AR, delayed recovery of graft function (DGF, postoperative pulmonary infection, preoperative immune induction therapy, postoperative immunosuppressive regimen, and other information. The risk factors for postoperative BKV infection and BKVN were analyzed. Results After an average of 15-month follow-up, it was found that the incidence of BKV viruria was 46.5%, that of BKV viremia was 14.0%, and that of BKVN was 2.3%. Sixth month after transplantation was found to be the peak time of viruria and viremia. FK506 was significantly associated with viremia in living donor renal transplantation. The immunosuppressive regimen was the immune related independent risk factor for BK viremia developing BKVN after living renal transplantation. Conclusion The incidence of BK viremia and BKVN is lower in living donor renal transplantation than in cadaver renal transplantation, but that of viruria is similar in both groups. Immunosuppressive scheme based on FK506 is an immune related independent risk factor leading to BK viremia proceeding to BKVN in living donor kidney

  3. Establishment of a novel one-step reverse transcription loop-mediated isothermal amplification assay for rapid identification of RNA from the severe fever with thrombocytopenia syndrome virus.

    Science.gov (United States)

    Xu, Haihong; Zhang, Lei; Shen, Guangqiang; Feng, Cen; Wang, Xinying; Yan, Jie; Zhang, Yanjun

    2013-12-01

    As an emerging infectious disease, severe fever with thrombocytopenia syndrome virus (SFTSV) infection has been found in many areas of China. Suitable laboratory diagnostic method is urgently needed in clinical detections and epidemiological investigations. In this study, a modified, low-cost and rapid visualized one-step reverse transcription loop-mediated isothermal amplification (RT-LAMP) method for the detection of RNA from the SFTSV has been established. In order to avoid the risk of aerosol contamination and facilitate the naked eye to observe, a microcrystalline wax-dye capsule wrapping the highly sensitive DNA fluorescence dye SYBR Green I was added to the RT-LAMP reaction tube before the initiation of the assay. The detection limit of the established RT-LAMP assay was 10 fg template RNA per reaction mixture. The RT-LAMP assay was confirmed to be high specific to SFTSV, and no cross-reaction was found with the detection of the Chikungunya fever virus, Hemorrhagic Fever with Renal Syndrome virus (HFRSV), and Dengue fever virus. The assay was then applied for the detection of SFTSV RNA in 32 clinical serum samples and showed 94.4% consistence with the detection results of the real-time RT-PCR. The whole process, from sample preparation to result reporting, can be completed within 2h. This adapted, cost efficient and quick visualized RT-LAMP method is feasible for SFTSV field diagnosis in resource-limited field settings.

  4. Branchio-oto-renal syndrome plus; a contiguous gene constellation of congenital anomalies?

    Energy Technology Data Exchange (ETDEWEB)

    Kelly, T.E. [Univ. of Virginia Health Sciences Center, Charlottesville, VA (United States)

    1994-09-01

    A term female infant was referred to the University Hospital because of respiratory distress secondary to bilateral choanal stenosis. Her examination revealed bilateral pre-auricular pits, branchial fistulae, cupped shaped ears, and bilateral athelia. She failed ABR screening; her creatinine was elevated to 1.5 mgs% and renal ultra-sonography showed reduced kidney size bilaterally. She was the product of her mother`s third pregnancy. The first produced a now normal 5 year old son. The second pregnancy was complicated by oligohydramnios and resulted in a premature delivery at 27 weeks gestation. The infant expired secondary to pulmonary hypoplasia. The mother had bilateral neurosensory deafness, pre-auricular pits, cupped shaped ears, lacrimal stenois and bilateral athelia. She wore dentures having earlier been diagnosed with dentogeneis imperfecta. She was shorter than her three normal sisters and had experienced academic problems throughout her school years. The maternal grandfather had an adult onset neurosensory hearing loss, but he and the maternal grandmother exhibited no other features of the BOR syndrome. Althelia was present only in the mother and daughter. The mother clearly has BOR syndrome transmitted to one, and possibly two, of her three offspring. The additional features of athelia, choanal stenosis and dentogenesis imperfecta are thought to represent additional autosomal dominant traits. Greenberg described an infant with athelia and choanal atresia. By family linkage studies, the BOR syndrome has been mapped to 8q13-21 with no recombination observed with loci D8S530 and D8S279. Given a normal prophase karyotype in the proband, it is speculated that a sub-microscopic deletion at 8q13-21 is the likely basis for the constellation of birth defects seen in this mother and daughter. Analysis of D8S530 and D8S279 is currently underway in this family.

  5. Pulmonary-renal syndromes: Experience from an Indian Intensive Care Unit

    Directory of Open Access Journals (Sweden)

    Srinivas Rajagopala

    2015-01-01

    Full Text Available Background: The etiology of patients presenting with pulmonary-renal syndrome (PRS to Intensive Care Units (ICUs in India is not previously reported. Aims: The aim was to describe the prevalence, etiology, clinical manifestations, and outcomes of PRS in an Indian ICU and identify variables that differentiate immunologic causes of PRS from tropical syndromes presenting with PRS. Materials and Methods: We conducted a prospective observational study of all patients presenting with PRS over 1-year. Clinical characteristics of patients with "definite PRS" were compared with those with "PRS mimics." Results: We saw 27 patients with "provisional PRS" over the said duration; this included 13 patients with "definite PRS" and 14 with "PRS mimics." The clinical symptoms were similar, but patients with PRS were younger and presented with longer symptom duration. Ninety-two percent of the PRS cohort required mechanical ventilation, 77% required vasopressors and 61.5% required dialysis within 48 h of ICU admission. The etiologic diagnosis of PRS was made after ICU admission in 61.5%. Systemic lupus erythrematosus (54% was the most common diagnosis. A combination of biopsy and serology was needed in the majority (69%, 9/13. Pulse methylprednisolone (92% and cyclophosphamide (61.5% was the most common protocol employed. Patients with PRS had more alveolar hemorrhage, hypoxemia and higher mortality (69% when compared to "PRS mimics." Conclusion: The spectrum of PRS is different in the tropics and tropical syndromes presenting with PRS are not uncommon. Multicentric studies are needed to further characterize the burden, etiology, treatment protocols, and outcomes of PRS in India.

  6. Epidemic transmission of human immunodeficiency virus in renal dialysis centers in Egypt.

    Science.gov (United States)

    El Sayed, N M; Gomatos, P J; Beck-Sagué, C M; Dietrich, U; von Briesen, H; Osmanov, S; Esparza, J; Arthur, R R; Wahdan, M H; Jarvis, W R

    2000-01-01

    In 1993 an epidemic of human immunodeficiency virus (HIV) infection occurred among 39 patients at 2 renal dialysis centers in Egypt. The centers, private center A (PCA) and university center A (UCA) were visited, HIV-infected patients were interviewed, seroconversion rates at UCA were calculated, and relatedness of HIV strains was determined by sequence analysis; 34 (62%) of 55 patients from UCA and 5 (42%) of 12 patients from PCA were HIV-infected. The HIV seroconversion risk at UCA varied significantly with day and shift of dialysis session. Practices that resulted in sharing of syringes among patients were observed at both centers. The analyzed V3 loop sequences of the HIV strain of 12 outbreak patients were >96% related to each other. V3 loop sequences from each of 8 HIV-infected Egyptians unrelated to the 1993 epidemic were only 76%-89% related to those from outbreak strains. Dialysis patients may be at risk for HIV infection if infection control guidelines are not followed.

  7. Genetic diversity of hepatitis C virus quasispecies in chronic renal failure patients in Midwest Brazil.

    Science.gov (United States)

    de Amorim, Regina Maria Santos; Coelho, Alexandre; Lampe, Elisabeth; Raiol, Tainá; Martins, Regina Maria Bringel

    2014-08-01

    Hepatitis C virus (HCV) quasispecies constitute a dynamic population in a continuous process of variation and selection. To investigate effect of the immune system on the genetic variability of HCV, we compared the hypervariable region 1 (HVR1) of immunosuppressed patients with chronic renal failure (CRF group) to immunocompetent patients with HCV chronic infection (control group). The HVR1 from ten samples of each group was amplified, cloned and sequenced. The HCV quasispecies from the control group had a higher frequency of variable sites in HVR1 (83.9 % vs 59.3 %, p quasispecies of the CRF group in the phylogenetic tree also showed the limited diversity of the quasispecies in immunosuppressed patients. Moreover, a higher variability of amino acids at positions 384, 386, 391, 394, 397, 398, 400, 405 and 410 was observed in the control group than in the CRF group, which showed a greater variability only at position 388 (p < 0.05). These data corroborates the hypothesis that the major selective pressure factor is the immune system, which promotes a high degree of diversity in the viral progeny and contributes to a constant evolution of HCV.

  8. The role of genetic nephropathies in the formation of chronic renal failure in children (a clinical observation of a child with acrorenal syndrome

    Directory of Open Access Journals (Sweden)

    M.A. Gonchar

    2017-08-01

    Full Text Available In the article, the authors analyze the literature, as well as the results of their own long-term experience in the diagnosis of genetically determined renal diseases accompanied by the development of chronic renal failure. The main causes of diseases, the principles of their diagnosis and treatment were outlined. Clinical observation of a patient with acrorenal syndrome with complicated development of chronic renal failure is given.

  9. Parámetros inmunitaríos en camarones juveniles lv en piscinas camaroneras infectadas con white spot syndrom virus

    OpenAIRE

    Montesdeoca, Bélgica Mercedes; Ibarra, Eulalia; Rodríguez, Jenny

    2002-01-01

    Parámetros inmunitarios en camarones juveniles LV en piscinas camaroneras infectadas con White Spot Syndrom Virus Parámetros inmunitarios en camarones juveniles LV en piscinas camaroneras infectadas con White Spot Syndrom Virus

  10. Síndrome pulmonar por hantavirus Andes en Chile CARDIOPULMONARY SYNDROME DUE TO ANDES VIRUS IN CHILE

    Directory of Open Access Journals (Sweden)

    CONSTANZA CASTILLO H.

    2002-01-01

    Full Text Available Desde 1993 han ocurrido 204 casos de Síndrome Cardiopulmonar por Hantavirus (SCPH en Chile. Los brotes epidémicos comenzaron en el sur y avanzan hacia el norte del país. Los más afectados son varones jóvenes, obreros agrícolas o forestales. En Chile, el SCPH es causado por el virus Andes, cuyo reservorio es el Oligorizomys longicaudatus (ratón de cola larga, que se distribuye desde la III Región al sur. El cuadro clínico es similar al descrito en EE.UU., caracterizado por una fase prodrómica que simula un estado gripal o cuadro gastrointestinal febril y que agrava por la aparición de edema pulmonar agudo e inestabilidad hemodinámica (fase cardiopulmonar. Sin embargo, cursa con mayores alteraciones hemorragí-paras y compromiso renal. La mortalidad inicial fue sobre 50% y actualmente es de alrededor del 33,3%. La presente revisión incluye: historia de la enfermedad, reservorio, modos de transmisión, patogenia, cuadro clínico, diagnóstico, tratamiento y medidas de prevenciónSince 1993, 204 cases of Hantavirus cardiopulmonary syndrome (HCPS occurred in Chile. Epidemic began in the south and moved thereafter to the northern regions. The disease affected predominantly young males, who worked in agricultural labours or as timber workers. The HCPS in Chile is caused by the Andes virus. The reservoir is the wild rat Oligoryzomis longicaudatus distributed from the III to the XII Region. The clinical features are similar to those described for Sin Nombre Virus. The disease has a prodromal stage characterised by fever, muscular pain, with or without gastrointestinal manifestations, followed by the rapid onset of respiratory insufficiency and haemodynamic unstability. Andes virus courses more often with haemorrhagic disorders and overt renal failure, than Sin Nombre Virus. The initial mortality was over 50% and declined to 33,3% in the last year. History of hantavirus-diseases, reservoir, and mode of contagion, pathogenesis, clinical

  11. Diagnosis of nutcracker syndrome of the left renal vein. Value of the corticomedullary phase of helical CT

    Energy Technology Data Exchange (ETDEWEB)

    Igari, Hidenori [Yokohama Comprehensive Care Continuum (Japan)

    2001-05-01

    The clinical significance of the corticomedullary phase of helical CT in the diagnosis of nutcracker syndrome of the left renal vein (LRV) was evaluated. A total of 38 patients with hematuria of unknown origin (12 men and 26 women, mean age 24 years [range: 18-32 years]) were examined by helical CT of the kidneys, retrograde left renal venography, and measurement of renocaval pressure gradients. The sensitivity and specificity of the corticomedullary and nephrographic phases of helical CT for detecting the nutcracker syndrome were determined. The nutcracker syndrome was diagnosed on the basis of the renography findings combined with pressure measurements in 19 patients: 12 with LRV hypertension and collateral veins, 1 with hypertension and no collateral veins, and 6 with collateral veins and normal pressure gradients or borderline hypertension. Both the corticomedullary- and nephrographic-phase images revealed distended LRVs in 26 (68%) of the 38 patients. The LRV mean attenuation values in the corticomedullary phase (164{+-}22 Hounsfield units [H], range: 138-209 H) was significantly greater (p<0.001) than in the nephrographic phase (80{+-}14 H, range: 62-100 H). During the corticomedullary phase, opacified blood from the renal vein was mixed with unopacified blood in the IVC. In the nephrographic phase, however, the IVC was enhanced homogeneously, but only slightly. Corticomedullary-phase imaging detected collateral veins that exhibited early enhancement, indicating retrograde flow from the LRVs in 15 out of 18 patients (83% of those with collateral veins), while the nephrographic-phase imaging revealed collateral veins in 8 patients (44% of those with collateral veins). The sensitivity and specificity of the corticomedullary-phase scan for detecting the nutcracker syndrome were 79% and 100%, respectively, as opposed to 42% and 100%, respectively, for the nephrographic phase. Retrograde flow from the LRV into the collateral veins in the corticomedullary phase is

  12. Renal cancer and pneumothorax risk in Birt-Hogg-Dubé syndrome; an analysis of 115 FLCN mutation carriers from 35 BHD families

    NARCIS (Netherlands)

    A.C. Houweling; L.M. Gijezen; M.A. Jonker; M.B.A. van Doorn; R.A. Oldenburg; K.Y. van Spaendonck-Zwarts; E.M. Leter; T.A. van Os; N.C.T. van Grieken; E.H. Jaspars; M.M. de Jong; E.M.H.F. Bongers; P.C. Johannesma; P.E. Postmus; R.J.A. van Moorselaar; J.H.T.M. van Waesberghe; T.M. Starink; M.A.M. van Steensel; J.J.P. Gille; F.H. Menko

    2011-01-01

    Birt-Hogg-Dubé (BHD) syndrome is an autosomal dominant condition caused by germline FLCN mutations, and characterised by fibrofolliculomas, pneumothorax and renal cancer. The renal cancer risk, cancer phenotype and pneumothorax risk of BHD have not yet been fully clarified. The main focus of this st

  13. Renal cancer and pneumothorax risk in Birt-Hogg-Dubé syndrome; An analysis of 115 FLCN mutation carriers from 35 BHD families

    NARCIS (Netherlands)

    A.C. Houweling (Arjan); L.M. Gijezen (L.); M.A. Jonker (Marianne); M.B. van Doorn (Martijn); R.A. Oldenburg (Rogier); K.Y. van Spaendonck-Zwarts (Karin); E.M. Leter (Edward); T.A.M. van Os (Theo); N.C.T. Grieken (Nicole); J.J. Jaspars (Joris); M.M. de Jong (Mirjam); E. Bongers (Ernie); P.C. Johannesma (P.); D. Postmus (Douwe); R.J.A. van Moorselaar; J.-H. van Waesberghe (J.); T.M. Starink; M.A.M. van Steensel; J.J. Gille (Johan); F. Menko

    2011-01-01

    textabstractBackground: Birt-Hogg-Dubé (BHD) syndrome is an autosomal dominant condition caused by germline FLCN mutations, and characterised by fibrofolliculomas, pneumothorax and renal cancer. The renal cancer risk, cancer phenotype and pneumothorax risk of BHD have not yet been fully clarified. T

  14. Renal cancer and pneumothorax risk in Birt-Hogg-Dube syndrome; an analysis of 115 FLCN mutation carriers from 35 BHD families

    NARCIS (Netherlands)

    Houweling, A. C.; Gijezen, L. M.; Jonker, M. A.; van Doorn, M. B. A.; Oldenburg, R. A.; van Spaendonck-Zwarts, K. Y.; Leter, E. M.; van Os, T. A.; van Grieken, N. C. T.; Jaspars, E. H.; de Jong, M. M.; Johannesma, P. C.; Postmus, P. E.; van Moorselaar, R. J. A.; van Waesberghe, J-H T. M.; Starink, T. M.; van Steensel, M. A. M.; Gille, J. J. P.; Menko, F. H.; Bongers, Ernie M. H. F.

    2011-01-01

    BACKGROUND: Birt-Hogg-Dube (BHD) syndrome is an autosomal dominant condition caused by germline FLCN mutations, and characterised by fibrofolliculomas, pneumothorax and renal cancer. The renal cancer risk, cancer phenotype and pneumothorax risk of BHD have not yet been fully clarified. The main focu

  15. Branchio-Oto-Renal Syndrome: Detection of EYA1 and SIX1 mutations in five out of six Danish families by combining linkage, sequencing and MLPA analyses

    DEFF Research Database (Denmark)

    Sanggard, Kirsten Marie; Rendtorff, Nanna Dahl; Kjaer, Klaus Wilbrandt

    2007-01-01

    The branchio-oto-renal (BOR) syndrome is an autosomal-dominant disorder characterized by hearing loss, branchial and renal anomalies. BOR is genetically heterogeneous and caused by mutations in EYA1 (8q13.3), SIX1 (14q23.1), SIX5 (19q13.3) and in an unidentified gene on 1q31. We examined six Danish...

  16. Thyroid, Renal, and Breast Carcinomas, Chondrosarcoma, Colon Adenomas, and Ganglioneuroma: A New Cancer Syndrome, FAP, or Just Coincidence

    Directory of Open Access Journals (Sweden)

    Ihab Shafek Atta

    2016-01-01

    Full Text Available We are presenting a case associated with papillary thyroid carcinoma, renal cell carcinoma, invasive mammary carcinoma, chondrosarcoma, benign ganglioneuroma, and numerous colon adenomas. The patient had a family history of colon cancer, kidney and bladder cancers, lung cancer, thyroid cancer, leukemia, and throat and mouth cancers. She was diagnosed with colonic villous adenoma at the age of 41 followed by thyroid, renal, and breast cancers and chondrosarcoma at the ages of 48, 64, 71, and 74, respectively. Additionally, we included a table with the most common familial cancer syndromes with one or more benign or malignant tumors diagnosed in our case, namely, FAP, HNPCC, Cowden, Peutz-Jeghers, renal cancer, tuberous sclerosis, VHL, breast/other, breast/ovarian, Carney, Werner’s, Bloom, Li-Fraumeni, xeroderma pigmentosum, ataxia-telangiectasia, osteochondromatosis, retinoblastoma, and MEN2A.

  17. Gemcitabine-induced hemolytic uremic syndrome mimicking scleroderma renal crisis presenting with Raynaud's phenomenon, positive antinuclear antibodies and hypertensive emergency.

    Science.gov (United States)

    Yamada, Yuichiro; Suzuki, Keisuke; Nobata, Hironobu; Kawai, Hirohisa; Wakamatsu, Ryo; Miura, Naoto; Banno, Shogo; Imai, Hirokazu

    2014-01-01

    A 58-year-old woman who received gemcitabine for advanced gallbladder cancer developed an impaired renal function, thrombocytopenia, Raynaud's phenomenon, digital ischemic changes, a high antinuclear antibody titer and hypertensive emergency that mimicked a scleroderma renal crisis. A kidney biopsy specimen demonstrated onion-skin lesions in the arterioles and small arteries along with ischemic changes in the glomeruli, compatible with a diagnosis of hypertensive emergency (malignant hypertension). The intravenous administration of a calcium channel blocker, the oral administration of an angiotensin-converting enzyme inhibitor and angiotensin II receptor blocker and the transfusion of fresh frozen plasma were effective for treating the thrombocytopenia and progressive kidney dysfunction. Gemcitabine induces hemolytic uremic syndrome with accelerated hypertension and Raynaud's phenomenon, mimicking scleroderma renal crisis.

  18. Duck egg-drop syndrome caused by BYD virus, a new Tembusu-related flavivirus.

    Directory of Open Access Journals (Sweden)

    Jingliang Su

    Full Text Available Since April 2010, a severe outbreak of duck viral infection, with egg drop, feed uptake decline and ovary-oviduct disease, has spread around the major duck-producing regions in China. A new virus, named BYD virus, was isolated in different areas, and a similar disease was reproduced in healthy egg-producing ducks, infecting with the isolated virus. The virus was re-isolated from the affected ducks and replicated well in primary duck embryo fibroblasts and Vero cells, causing the cytopathic effect. The virus was identified as an enveloped positive-stranded RNA virus with a size of approximately 55 nm in diameter. Genomic sequencing of the isolated virus revealed that it is closely related to Tembusu virus (a mosquito-borne Ntaya group flavivirus, with 87-91% nucleotide identity of the partial E (envelope proteins to that of Tembusu virus and 72% of the entire genome coding sequence with Bagaza virus, the most closely related flavivirus with an entirely sequenced genome. Collectively our systematic studies fulfill Koch's postulates, and therefore, the causative agent of the duck egg drop syndrome occurring in China is a new flavivirus. Flavivirus is an emerging and re-emerging zoonotic pathogen and BYD virus that causes severe egg-drop, could be disastrous for the duck industry. More importantly its public health concerns should also be evaluated, and its epidemiology should be closely watched due to the zoonotic nature of flaviviruses.

  19. Duck egg-drop syndrome caused by BYD virus, a new Tembusu-related flavivirus.

    Science.gov (United States)

    Su, Jingliang; Li, Shuang; Hu, Xudong; Yu, Xiuling; Wang, Yongyue; Liu, Peipei; Lu, Xishan; Zhang, Guozhong; Hu, Xueying; Liu, Di; Li, Xiaoxia; Su, Wenliang; Lu, Hao; Mok, Ngai Shing; Wang, Peiyi; Wang, Ming; Tian, Kegong; Gao, George F

    2011-03-24

    Since April 2010, a severe outbreak of duck viral infection, with egg drop, feed uptake decline and ovary-oviduct disease, has spread around the major duck-producing regions in China. A new virus, named BYD virus, was isolated in different areas, and a similar disease was reproduced in healthy egg-producing ducks, infecting with the isolated virus. The virus was re-isolated from the affected ducks and replicated well in primary duck embryo fibroblasts and Vero cells, causing the cytopathic effect. The virus was identified as an enveloped positive-stranded RNA virus with a size of approximately 55 nm in diameter. Genomic sequencing of the isolated virus revealed that it is closely related to Tembusu virus (a mosquito-borne Ntaya group flavivirus), with 87-91% nucleotide identity of the partial E (envelope) proteins to that of Tembusu virus and 72% of the entire genome coding sequence with Bagaza virus, the most closely related flavivirus with an entirely sequenced genome. Collectively our systematic studies fulfill Koch's postulates, and therefore, the causative agent of the duck egg drop syndrome occurring in China is a new flavivirus. Flavivirus is an emerging and re-emerging zoonotic pathogen and BYD virus that causes severe egg-drop, could be disastrous for the duck industry. More importantly its public health concerns should also be evaluated, and its epidemiology should be closely watched due to the zoonotic nature of flaviviruses.

  20. Suppression of Shrimp Melanization during White Spot Syndrome Virus Infection*

    Science.gov (United States)

    Sutthangkul, Jantiwan; Amparyup, Piti; Charoensapsri, Walaiporn; Senapin, Saengchan; Phiwsaiya, Kornsunee; Tassanakajon, Anchalee

    2015-01-01

    The melanization cascade, activated by the prophenoloxidase (proPO) system, plays a key role in the production of cytotoxic intermediates, as well as melanin products for microbial sequestration in invertebrates. Here, we show that the proPO system is an important component of the Penaeus monodon shrimp immune defense toward a major viral pathogen, white spot syndrome virus (WSSV). Gene silencing of PmproPO(s) resulted in increased cumulative shrimp mortality after WSSV infection, whereas incubation of WSSV with an in vitro melanization reaction prior to injection into shrimp significantly increased the shrimp survival rate. The hemolymph phenoloxidase (PO) activity of WSSV-infected shrimp was extremely reduced at days 2 and 3 post-injection compared with uninfected shrimp but was fully restored after the addition of exogenous trypsin, suggesting that WSSV probably inhibits the activity of some proteinases in the proPO cascade. Using yeast two-hybrid screening and co-immunoprecipitation assays, the viral protein WSSV453 was found to interact with the proPO-activating enzyme 2 (PmPPAE2) of P. monodon. Gene silencing of WSSV453 showed a significant increase of PO activity in WSSV-infected shrimp, whereas co-silencing of WSSV453 and PmPPAE2 did not, suggesting that silencing of WSSV453 partially restored the PO activity via PmPPAE2 in WSSV-infected shrimp. Moreover, the activation of PO activity in shrimp plasma by PmPPAE2 was significantly decreased by preincubation with recombinant WSSV453. These results suggest that the inhibition of the shrimp proPO system by WSSV partly occurs via the PmPPAE2-inhibiting activity of WSSV453. PMID:25572398

  1. Metabolic syndrome in human immunodeficiency virus positive patients

    Directory of Open Access Journals (Sweden)

    Sarita Bajaj

    2013-01-01

    Full Text Available Aims and Objectives : To assess the prevalence of metabolic syndrome (MetS in human immunodeficiency virus (HIV positive patients. Prevalence of MetS was compared in patients who were not on highly active antiretroviral therapy (HAART to patients who were on HAART. Materials and Methods: Seventy HIV positive cases were studied. Pregnant and lactating women, patients on drugs other than HAART known to cause metabolic abnormalities and those having diabetes or hypertension were excluded. Cases were evaluated for MetS by using National Cholesterol Education Program Adult Treatment Panel-III. Results: 47 cases were on HAART and 23 cases were not on HAART. Fasting Blood Glucose ≥100 mg/dl was present in 28.6% cases, out of whom 27.7% were on HAART and 30.4% were not on HAART (P = 0.8089. 12.9% cases had BP ≥130/≥85 mm Hg, out of whom 14.9% were on HAART and 8.7% were not on HAART (P = 0.4666. 42.9% cases had TG ≥150 mg/dl, out of whom 44.7% were on HAART and 39.1% were not on HAART (P = 0.6894. HDL cholesterol was low (males <40 mg/dl, females <50 mg/dl in 50% cases, out of whom 55.3% were on HAART and 39.1% were not on HAART (P = 0.2035. Conclusions: Prevalence of MetS was 20%. Majority of patients had only one component of MetS (32.9%. Low HDL was present in 50%, followed by raised triglycerides in 42.9%. Waist circumference was not increased in any of the patients. There was no statistically significant difference between those on HAART and those not on HAART in distribution of risk factors and individual components of MetS.

  2. Suppression of shrimp melanization during white spot syndrome virus infection.

    Science.gov (United States)

    Sutthangkul, Jantiwan; Amparyup, Piti; Charoensapsri, Walaiporn; Senapin, Saengchan; Phiwsaiya, Kornsunee; Tassanakajon, Anchalee

    2015-03-06

    The melanization cascade, activated by the prophenoloxidase (proPO) system, plays a key role in the production of cytotoxic intermediates, as well as melanin products for microbial sequestration in invertebrates. Here, we show that the proPO system is an important component of the Penaeus monodon shrimp immune defense toward a major viral pathogen, white spot syndrome virus (WSSV). Gene silencing of PmproPO(s) resulted in increased cumulative shrimp mortality after WSSV infection, whereas incubation of WSSV with an in vitro melanization reaction prior to injection into shrimp significantly increased the shrimp survival rate. The hemolymph phenoloxidase (PO) activity of WSSV-infected shrimp was extremely reduced at days 2 and 3 post-injection compared with uninfected shrimp but was fully restored after the addition of exogenous trypsin, suggesting that WSSV probably inhibits the activity of some proteinases in the proPO cascade. Using yeast two-hybrid screening and co-immunoprecipitation assays, the viral protein WSSV453 was found to interact with the proPO-activating enzyme 2 (PmPPAE2) of P. monodon. Gene silencing of WSSV453 showed a significant increase of PO activity in WSSV-infected shrimp, whereas co-silencing of WSSV453 and PmPPAE2 did not, suggesting that silencing of WSSV453 partially restored the PO activity via PmPPAE2 in WSSV-infected shrimp. Moreover, the activation of PO activity in shrimp plasma by PmPPAE2 was significantly decreased by preincubation with recombinant WSSV453. These results suggest that the inhibition of the shrimp proPO system by WSSV partly occurs via the PmPPAE2-inhibiting activity of WSSV453.

  3. Nephrocalcinosis and Renal Failure in Lesch-Nyhan Syndrome: Report of Two Familial Cases and Review of the Literature.

    Science.gov (United States)

    Vargiami, Euthymia; Printza, Nikoleta; Papadimiditriou, Eleni; Batzios, Spyros; Kyriazi, Maria; Papachristou, Fotios; Zafeiriou, Dimitrios I

    2016-11-01

    Lesch-Nyhan syndrome is an X-linked recessive inborn error of purine metabolism, due to deficiency of the enzyme HPRT (hypoxanthine-guanine phosphoribosyl transferase) and underlying HPRT gene mutations (over 300 mutations identified up to date). It is characterized by a wide range of neurological symptoms and signs (mainly a combination of spastic diplegia with choreoathetosis and an overall psychomotor redardation). Herein, we report of two cousins with Lesch-Nyhan syndrome and a confirmed novel HPRT gene mutation: c.65T>C, who both developed nephrocalcinosis and renal failure, findings not been previously published in children with HPRT deficiency. Copyright © 2016 Elsevier Inc. All rights reserved.

  4. [Acute necrotizing capillaritis in an adolescent dying from a a Goodpasture-like pulmonary-renal syndrome].

    Science.gov (United States)

    Maresi, E; Becchina, G; Orlando, E; Ottoveggio, G

    1995-12-01

    In this report the authors describe a rare case of pulmonary/renal syndrome like-Goodpasture's disease characterized by a necrotizing acute capillaritis in the lung, kidney, liver, spleen and heart. In the heart the capillaritis was associated with a contiguous acute ischemic lesion. The clinical and pathologic findings of the case suggest that the presence of vasculitis should not exclude a diagnosis of Goodpasture's Syndrome and that the multisystemic capillaritis would demonstrate the organ-non-specificity of anti-glomerular/alveolar basement membrane antibody.

  5. Efficacy and safety of treatment of hepatitis C virus infection in renal transplant recipients

    Institute of Scientific and Technical Information of China (English)

    Abdulrahman A Aljumah; Abdulla A Al Sayyari; Mohamed A Saeed; Ahmed I Al Flaiw; Ibrahim H Al Traif; Abduljaleel M Al Alwan; Salem H Al Qurashi; Ghormallah A Al Ghamdi; Fayez F Al Hejaili; Mohammed A Al Balwi

    2012-01-01

    AIM: To assess the efficacy and safety of combined pegylated interferon and ribavirin therapy in hepatitis C virus (HCV) infection in renal transplant recipients. METHODS: This is a retrospective chart review of post renal transplant patients who were positive for anti-HCV and HCV-RNA, and who have received treatment with combination of pegylated interferon and ribavirin between October 2003 and December 2008. Only patients with stable graft function and absence of evidence of cirrhosis and who received the therapy for continuous 48 wk were included. Nineteen patients (13 male and 6 female) were identified and included. The patient's complete blood count, liver and kidney profile, and calculated glomerular filtration rate (GFR) were monitored every 6-8 wk while on treatment. HCV-RNA was tested at 12 wk for early virological response, at 48 wk for end of treatment response (ETR), and then retested at 24, and 48 wk after completion of therapy for sustained virological response (SVR). Liver biopsies were obtained before treatment from all patients and graft kidney biopsies were performed as required. RESULTS: Of the entire cohort, 9 patients (47.4%) showed an ETR and 8 had SVR (42.1%). Of the 8 patients with abnormal alanine aminotransferase (ALT) levels at baseline, 78.9% had their ALT normalized (including the virological non responders). ALT was normal in all responders at the end of therapy and at 24 wk post therapy (100%). Only one patient (5.3%) developed an increase in creatinine and decline in GFR from baseline towards the end of treatment. This patient's kidney biopsy revealed borderline rejection. There was no impact on response by HCV-genotype, initial HCV RNA load, age or sex of the patient or duration post transplant before commencement of therapy. All patients tolerated treatment in the same way as non-transplant with no unusual or increased occurrence of side effects. CONCLUSION: The combination of pegylated interferon and ribavirin is effective in

  6. VITAMIN D RECEPTOR ACTIVATION AND A NOVEL CLASSIFICATION OF CARDIO-RENAL SYNDROME

    Directory of Open Access Journals (Sweden)

    Sirous Darabian

    2012-06-01

    Full Text Available Due to increasing survival rate of cardiac and renal disease patients, Cardiorenal syndrome (CRS, a combination of these two is becoming an important problem. Some classifications have been proposed for CRS but for clinical approach, it would be more appropriate to emphasize the pathophysiologic pathways to classify CRS into: 1\\ hemodynamic, 2\\ atherosclerotic, 3\\ uremic, 4\\ neurohumoral, 5\\ anemic-hematologic, 6\\ inflammatory-oxidative, 7\\ vitamin D receptor (VDR related, and 8\\ multi-factorial CRS. Recently, it has been revealed that vitamin D and its receptor play an important role in the CRS. Decreased 1-α-hydroxylase activity, nutritional deficiency, decreased Megalin receptors, and increased 1-24-hydroxylase activity are major causes for vitamin D depletion in CKD. Decrease in GFR, renal mass and 1-α-hydroxylase expression along with phosphate retention, increased FGF-23, and loss of both 1-α-hydroxylase and 25(OHvitamin D are important factors for decreased 1-α-hydroxylase activity. Suboptimal or defective VDR activation may play a role in causing or aggravating CRS. Newer VDR activators such as vitamin D mimetics (e.g. paricalcitol and maxacalcitol are promising agents. Some studies have confirmed the survival advantages of D-mimetics as compared to non-selective VDR activators. Higher doses of D-mimetic per unit of PTH (paricalcitol to PTH ratio are associated with greater survival, and the survival advantages of African American dialysis patients could be explained by higher received doses of paricalcitol. More studies are needed to verify these data and to explore additional avenues for CRS management via modulating VDR pathway.

  7. In an Ovine Model of Polycystic Ovary Syndrome (PCOS) Prenatal Androgens Suppress Female Fetal Renal Gluconeogenesis.

    Science.gov (United States)

    Connolly, Fiona; Rae, Michael T; Späth, Katharina; Boswell, Lyndsey; McNeilly, Alan S; Duncan, W Colin

    2015-01-01

    Increased maternal androgen exposure during pregnancy programmes a polycystic ovary syndrome (PCOS)-like condition, with metabolic dysfunction, in adult female offspring. Other in utero exposures associated with the development of insulin resistance, such as intrauterine growth restriction and exposure to prenatal glucocorticoids, are associated with altered fetal gluconeogenesis. We therefore aimed to assess the effect of maternal androgenisation on the expression of PEPCK and G6PC in the ovine fetus. Pregnant Scottish Greyface sheep were treated with twice weekly testosterone propionate (TP; 100mg) or vehicle control from day 62 to day 102 of gestation. At day 90 and day 112 fetal plasma and liver and kidney tissue was collected for analysis. PEPCK and G6PC expression were analysed by quantitative RT-PCR, immunohistochemistry and western blotting. PEPCK and G6PC were localised to fetal hepatocytes but maternal androgens had no effect on female or male fetuses. PEPCK and G6PC were also localised to the renal tubules and renal PEPCK (P<0.01) and G6PC (P = 0.057) were lower in females after prenatal androgenisation with no change in male fetuses. These tissue and sex specific observations could not be explained by alterations in fetal insulin or cortisol. The sexual dimorphism may be related to the increase in circulating estrogen (P<0.01) and testosterone (P<0.001) in females but not males. The tissue specific effects may be related to the increased expression of ESR1 (P<0.01) and AR (P<0.05) in the kidney when compared to the fetal liver. After discontinuation of maternal androgenisation female fetal kidney PEPCK expression normalised. These data further highlight the fetal and sexual dimorphic effects of maternal androgenisation, an antecedent to adult disease and the plasticity of fetal development.

  8. In an Ovine Model of Polycystic Ovary Syndrome (PCOS Prenatal Androgens Suppress Female Fetal Renal Gluconeogenesis.

    Directory of Open Access Journals (Sweden)

    Fiona Connolly

    Full Text Available Increased maternal androgen exposure during pregnancy programmes a polycystic ovary syndrome (PCOS-like condition, with metabolic dysfunction, in adult female offspring. Other in utero exposures associated with the development of insulin resistance, such as intrauterine growth restriction and exposure to prenatal glucocorticoids, are associated with altered fetal gluconeogenesis. We therefore aimed to assess the effect of maternal androgenisation on the expression of PEPCK and G6PC in the ovine fetus. Pregnant Scottish Greyface sheep were treated with twice weekly testosterone propionate (TP; 100mg or vehicle control from day 62 to day 102 of gestation. At day 90 and day 112 fetal plasma and liver and kidney tissue was collected for analysis. PEPCK and G6PC expression were analysed by quantitative RT-PCR, immunohistochemistry and western blotting. PEPCK and G6PC were localised to fetal hepatocytes but maternal androgens had no effect on female or male fetuses. PEPCK and G6PC were also localised to the renal tubules and renal PEPCK (P<0.01 and G6PC (P = 0.057 were lower in females after prenatal androgenisation with no change in male fetuses. These tissue and sex specific observations could not be explained by alterations in fetal insulin or cortisol. The sexual dimorphism may be related to the increase in circulating estrogen (P<0.01 and testosterone (P<0.001 in females but not males. The tissue specific effects may be related to the increased expression of ESR1 (P<0.01 and AR (P<0.05 in the kidney when compared to the fetal liver. After discontinuation of maternal androgenisation female fetal kidney PEPCK expression normalised. These data further highlight the fetal and sexual dimorphic effects of maternal androgenisation, an antecedent to adult disease and the plasticity of fetal development.

  9. Manifestações renais na síndrome de Joubert Renal symptoms in the Joubert syndrome

    Directory of Open Access Journals (Sweden)

    Ana Paula Weiss

    2009-06-01

    Full Text Available OBJETIVO:Descrever o caso clínico de paciente com Síndrome de Joubert associada a alterações renais. DESCRIÇÃO DO CASO: Paciente de dois meses de idade admitida com quadro hipotonia e hiperpneia. Ao exame físico, observaram-se, além da respiração irregular, movimentos oculares anormais e hipertensão arterial; não se evidenciaram alterações na ausculta cardíaca e pulmonar. Durante investigação clínico-laboratorial inicial, as causas cardíacas e pulmonares foram descartadas. Aventaram-se hipóteses diagnósticas de patologias neurológicas com doença renal. Os exames laboratoriais mostraram presença de alcalose respiratória, acidose metabólica e hipercalemia, com função renal normal. A ressonância magnética evidenciou alterações neurológicas compatíveis com "sinal do dente molar", quadro frequentemente associada à Sindrome de Joubert. Levando-se em consideração a associação dessa síndrome com alterações renais, a investigação nefrológica demonstrou imagens císticas em parênquima renal. COMENTÁRIOS: Patologias cardíacas e pulmonares estão frequentemente associadas a manifestações clínicas como taquipneia e distúrbios metabólicos. Entretanto, pode ser necessária uma investigação neurológica porque diversas doenças que acometem o sistema nervoso central apresentam tais alterações. A associação entre alterações renais e malformações de sistema nervoso central é frequente em diversos processos sindrômicos, justificando-se a sua investigação. A Síndrome de Joubert e as desordens a ela relacionadas caracterizam-se por aplasia do vermix cerebelar, ataxia, movimentos oculares anormais, respiração irregular e retardo do desenvolvimento neuropsicomotor. As alterações renais mais comuns são os cistos renais e a nefronoftise, que pode progredir para doença renal terminal.OBJECTIVE:To describe the case of a patient with Joubert syndrome associated with renal impairments. CASE

  10. Acute Renal Replacement Therapy in Children with Diarrhea-Associated Hemolytic Uremic Syndrome: A Single Center 16 Years of Experience

    OpenAIRE

    Silviu Grisaru; Morgunov, Melissa A.; Samuel, Susan M.; Julian P Midgley; Wade, Andrew W; Tee, James B.; Hamiwka, Lorraine A.

    2011-01-01

    Acute kidney injury (AKI) is becoming more prevalent among hospitalized children, its etiologies are shifting, and new treatment modalities are evolving; however, diarrhea-associated hemolytic uremic syndrome (D+HUS) remains the most common primary disease causing AKI in young children. Little has been published about acute renal replacement therapy (ARRT) and its challenges in this population. We describe our single center's experience managing 134 pediatric patients with D+HUS out of whom 5...

  11. Immunization effect of purified bivalent vaccine to haemorrhagic fever with renal syndrome manufactured from primary cultured hamster kidney cells

    Institute of Scientific and Technical Information of China (English)

    DONG Guan-mu; HAN Liang; AN Qi; LIU Wen-xue; KONG Yan; YANG Li-hong

    2005-01-01

    @@ Haemorrhagic fever with renal syndrome (HFRS) is a worldwide epidemic plaguing over thirty nations. This disease has spread across 26 provinces, cities and autonomous regions of China with an annual onsets number of more than a hundred thousand and a mortality rate of 5% to 15%, accounting for over 80% of cases in the world, and threatening the safety and health of Chinese people.1 Analysis of serum samples over recent years indicates that the plagued areas are expanding.

  12. Urine alkalinization may be enough for the treatment of bilateral renal pelvis stones associated with Lesch-Nyhan syndrome.

    Science.gov (United States)

    Oh, Mi Mi; Ham, Byeong Kuk; Kang, Seok Ho; Bae, Jae Hyun; Kim, Je Jong; Yoo, Ki Hwan; Yoon, Duck Ki; Moon, Du Geon

    2011-10-01

    Lesch-Nyhan syndrome is a rare sex-linked disorder of purine metabolism that is caused by a mutation in the hypoxanthine-guanine phosphoribosyltransferase (HPRT) gene which causes marked hyperuricemia and hyperuricosuria, with signs of gouty arthritis and uric acid stone disease in early childhood. We report a case of renal pelvis calculi which was dissolved within 10 days of urine alkalinization and hydration.

  13. Rapidly progressive antineutrophil cytoplasm antibodies associated with pulmonary-renal syndrome in a 10-year-old girl

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    Fermin Blanco Filho

    2001-01-01

    Full Text Available CONTEXT: The term pulmonary-renal syndrome has been used frequently to describe the clinical manifestations of a great number of diseases in which pulmonary hemorrhage and glomerulonephritis coexist. The classic example of this type of vasculitis is Goodpasture´s syndrome, a term used to describe the association of pulmonary hemorrhage, glomerulonephritis and the presence of circulating antiglomerular basement membrane antibodies (anti-GBM. Among the several types of systemic vasculitides that can present clinical manifestations of the pulmonary-renal syndrome, we focus the discussion on two types more frequently associated with antineutrophil cytoplasm antibodies (ANCA, microscopic polyangiitis and Wegener´s granulomatosis, concerning a 10 year old girl with clinical signs and symptoms of pulmonary-renal syndrome, with positive ANCA and rapidly progressive evolution. CASE REPORT: We describe the case of a 10-year-old girl referred to our hospital for evaluation of profound anemia detected in a primary health center. Five days before entry she had experienced malaise, pallor and began to cough up blood-tinged sputum that was at first attributed to dental bleeding. She was admitted to the infirmary with hemoglobin = 4 mg/dL, hematocrit = 14%, platelets = 260,000, white blood cells = 8300, 74% segmented, 4% eosinophils, 19% lymphocytes and 3% monocytes. Radiographs of the chest revealed bilateral diffuse interstitial alveolar infiltrates. There was progressive worsening of cough and respiratory distress during the admission day, when she began to cough up large quantities of blood and hematuria was noted. There was rapid and progressive loss of renal function and massive lung hemorrhage. The antineutrophil cytoplasm antibody (ANCA test with antigen specificity for myeloperoxidase (anti-MPO was positive and the circulating anti-GBM showed an indeterminate result.

  14. The roles of the liver and pancreas in renal nutcracker syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Yun, Seong Jong, E-mail: zoomknight@naver.com [Department of Radiology, Kyung Hee University Hospital at Gangdong, Kyung Hee University School of Medicine, 149 Sangil-dong, Kangdong-gu, Seoul 134-727 (Korea, Republic of); Department of Radiology, Graduate School of Medicine, Kyung Hee University, Hoegi-dong, Dongdaemun-gu, Seoul 130-701 (Korea, Republic of); Nam, Deok Ho, E-mail: namjindan@daum.net [Department of Radiology, Kyung Hee University Hospital at Gangdong, Kyung Hee University School of Medicine, 149 Sangil-dong, Kangdong-gu, Seoul 134-727 (Korea, Republic of); Ryu, Jung Kyu, E-mail: oddie2@naver.com [Department of Radiology, Kyung Hee University Hospital at Gangdong, Kyung Hee University School of Medicine, 149 Sangil-dong, Kangdong-gu, Seoul 134-727 (Korea, Republic of); Kim, Ji Su, E-mail: js830808@hanmail.net [Department of Radiology, Kyung Hee University Hospital at Gangdong, Kyung Hee University School of Medicine, 149 Sangil-dong, Kangdong-gu, Seoul 134-727 (Korea, Republic of)

    2014-10-15

    Graphical abstract: - Highlights: • The presence of the liver and pancreas may influence NCS by compressing SMA against the aorta. • The presence of the liver and pancreas at the level of the LRV is not yet recognized as an independent factor for NCS but should be. • The presence of the liver and the pancreas may allow clinicians to identify NCS patients and may influence the choice of treatment options. - Abstract: Introduction: To assess the frequency and significance of presence of the liver and pancreas at the left renal vein (LRV) level in patients with suspected renal nutcracker syndrome (NCS). Materials and methods: We included 101 patients with hematuria who underwent urography three-dimensional CT between April 2009 and November 2013. These patients were divided into NCS (n = 25) and non-NCS (n = 76) patients according to the following CT criteria: (1) the presence of beak sign and (2) hilar-aortomesenteric left renal vein diameter ratio >4. Patients were grouped according to the presence of the liver and pancreas at the LRV: group LP (both liver and pancreas), group L (only liver), group P (only pancreas), and group O (neither liver nor pancreas). The difference in the frequencies of groups was analyzed between NCS and non-NCS patients. Multivariate analysis was used to determine the independent factors between NCS and non-NCS patients. Results: The frequencies of group LP, group L, group P, and group O in NCS vs. non-NCS were 88% vs. 5.3% (p < 0.001), 4.0% vs. 2.6% (p = 0.75), 4.0% vs. 11.8% (p = 0.45), 4.0% vs. 80.3% (p < 0.001), respectively. Multivariate analysis demonstrated that group was a predictor for differential diagnosis between NCS and non-NCS (p = 0.022), and group LP was an independent factor for the presence of NCS (odds ratio, 43.8; 95% confidence interval, 3.8–500.3; p < 0.002; reference, group O). Conclusion: The presence of the liver and pancreas at the level of the LRV was frequently found in NCS and was the independent factor

  15. Renal transplantation in human immunodeficiency virus-infected recipients: a case-control study from the Brazilian experience.

    Science.gov (United States)

    Vicari, A R; Spuldaro, F; Sandes-Freitas, T V; Cristelli, M P; Requião-Moura, L R; Reusing, J O; Pierrotti, L C; Oliveira, M L; Girão, C M; Gadonski, G; Kroth, L V; Deboni, L M; Ferreira, G F; Tedesco-Silva, H; Esmeraldo, R; David-Neto, E; Saitovitch, D; Keitel, E; Garcia, V D; Pacheco-Silva, A; Medina-Pestana, J O; Manfro, R C

    2016-10-01

    Highly active antiretroviral therapy has turned human immunodeficiency virus (HIV)-infected patients with end-stage renal disease into suitable candidates for renal transplantation. We present the Brazilian experience with kidney transplantation in HIV-infected recipients observed in a multicenter study. HIV-infected kidney transplant recipients and matched controls were evaluated for the incidence of delayed graft function (DGF), acute rejection (AR), infections, graft function, and survival of patients and renal grafts. Fifty-three HIV-infected recipients and 106 controls were enrolled. Baseline characteristics were similar, but a higher frequency of pre-transplant positivity for hepatitis C virus and cytomegalovirus infections was found in the HIV group. Immunosuppressive regimens did not differ, but a trend was observed toward lower use of anti-thymocyte globulin in the group of HIV-infected recipients (P = 0.079). The HIV-positive recipient group presented a higher incidence of treated AR (P = 0.036) and DGF (P = 0.044). Chronic Kidney Disease Epidemiology Collaboration estimated that glomerular filtration rate was similar at 6 months (P = 0.374) and at 12 months (P = 0.957). The median number of infections per patient was higher in the HIV-infected group (P = 0.018). The 1-year patient survival (P experience, despite somewhat inferior outcomes, kidney transplantation is an adequate therapy for selected HIV-infected recipients. © 2016 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  16. Spontaneous intracranial hemorrhage in a patient with Middle East respiratory syndrome corona virus

    Science.gov (United States)

    Al-Hameed, Fahad M.

    2017-01-01

    The Middle East respiratory syndrome corona virus (MERS-CoV) is a novel positive sense singlestranded ribonucleic acid virus of the genus Beta corona virus. This virus was first isolated from a patient who died from severe respiratory illness in June 2012 in Jeddah, Kingdom of Saudi Arabia. We describe an unusual case of a 42 year old healthcare worker who was admitted to our Intensive Care Unit (ICU) King Abdul-Aziz Medical City, with MERS-CoV and severe acute respiratory distress Syndrome and developed a sudden-onset diabetes insipidus and spontaneous massive intracranial hemorrhage with intra-ventricular extension and tonsillar herniation. Computed angiogram of the brain did not reveal any aneurysm or structural defects. She never had uncontrolled hypertension, or coagulopathy, nor she received antiplatelets. We are reporting a rare case of structural neurological damage associated with MERS-CoV infection. PMID:28133694

  17. Distal renal tubular acidosis as a cause of osteomalacia in a patient with primary Sjögren's syndrome

    Directory of Open Access Journals (Sweden)

    Jovelić Aleksandra

    2005-01-01

    Full Text Available Background. One half of the patients with primary Sjögren’s syndrome has extraglandular manifestations, including renal involvement. The most frequent renal lesion is tubulo-interstitial nephritis, which manifests clinically as distal tubular acidosis and may result in the development of osteomalacia. Case report. In a 29 - year-old female patient, with bilateral nephrolithiasis, the diagnosis of primary Sjögren’s syndrome, tubulo-interstitial nephritis, distal renal tubular acidosis, and hypokalemia were established. She was treated for hypokalemia. Two years later she developed bone pains and muscle weakness, she wasn’t able to walk, her proximal muscles and pelvic bones were painful, with radiological signs of pelvic bones osteopenia and pubic bones fractures. The diagnosis of osteomalacia was established and the treatment started with Schol’s solution, vitamin D and calcium. In the following two months, acidosis was corrected, and the patient started walking. Conclusion. In our patient with primary Sjögren’s syndrome and interstitial nephritis, osteomalacia was a result of the long time decompensate acidosis, so the correction of acidosis, and the supplementation of vitamin D and calcium were the integral part of the therapy.

  18. Renal angiomyolipoma bleeding in a patient with TSC2/PKD1 contiguous gene syndrome after 17 years of renal replacement therapy.

    Science.gov (United States)

    Furlano, Mónica; Barreiro, Yaima; Martí, Teresa; Facundo, Carme; Ruiz-García, César; DaSilva, Iara; Ayasreh, Nadia; Cabrera-López, Cristina; Ballarín, José; Ars, Elisabet; Torra, Roser

    We report the case of a 32-year-old male diagnosed with TSC2/PKD1 contiguous gene syndrome, presenting with tuberous sclerosis (TS) and autosomal dominant polycystic kidney disease simultaneously. He progressed to end-stage renal disease and received a kidney transplant at the age of 12. The native kidneys presented angiomyolipomas (AML), which are common benign tumours in patients with TS. Seventeen years after transplantation, he presented with abdominal pain, anaemia and a retroperitoneal haematoma, the latter caused by renal AML bleeding. Selective embolisation was performed. Our patient could have benefited from the administration of mTOR inhibitors at transplant. This therapy is immunosuppressive and reduces the size of benign tumours in TS as well as the risk of rupture and bleeding. This patient did not receive mTOR inhibitors at the time of the transplant because the relationship between mTOR inhibitors and TS was unknown at that time. This case confirms the persistent risk of renal AML bleeding for both transplanted patients and patients on dialysis. As a result, we would recommend routine check-ups of native kidneys and nephrectomy assessment.

  19. Renal angiomyolipoma bleeding in a patient with TSC2/PKD1 contiguous gene syndrome after 17 years of renal replacement therapy

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    Mónica Furlano

    2017-01-01

    Full Text Available We report the case of a 32-year-old male diagnosed with TSC2/PKD1 contiguous gene syndrome, presenting with tuberous sclerosis complex (TSC and autosomal dominant polycystic kidney disease simultaneously. He progressed to end-stage renal disease and received a kidney transplant at the age of 12. The native kidneys presented angiomyolipomas (AML, which are common benign tumours in patients with TSC. Seventeen years after transplantation, he presented with abdominal pain, anaemia and a retroperitoneal haematoma, the latter caused by renal AML bleeding. Selective embolisation was performed. Our patient could have benefited from the administration of mTOR inhibitors at transplant. This therapy is immunosuppressive and reduces the size of benign tumours in TSC as well as the risk of rupture and bleeding. This patient did not receive mTOR inhibitors at the time of the transplant because the relationship between mTOR inhibitors and TSC was unknown at that time. This case confirms the persistent risk of renal AML bleeding for both transplanted patients and patients on dialysis. As a result, we would recommend routine check-ups of native kidneys and nephrectomy assessment.

  20. Eculizumab: safety and efficacy after 17 months of treatment in a renal transplant patient with recurrent atypical hemolytic-uremic syndrome: case report.

    Science.gov (United States)

    Châtelet, V; Lobbedez, T; Frémeaux-Bacchi, V; Ficheux, M; Ryckelynck, J Ph; Hurault de Ligny, B

    2010-12-01

    In a recent study, eculizumab, a humanized monoclonal antibody which targets complement factor C5, appeared to resolve hemolysis and thrombocytopenia leading to recovery of renal function in a transplant patient during an episode of an atypical hemolytic uremic syndrome. We report the efficacy of eculizumab in a patient who presented with a recurrence of atypical hemolytic syndrome at 3 years after renal transplantation. After 17 months of eculizumab treatment, and without concomitant plasma therapy, renal function was maintained, the need for blood transfusions reduced, and acute thrombotic microangiopathy and hemolysis controlled. These data suggested that eculizumab should be considered to be a permanent treatment for this patient.

  1. Chikungunya virus infection amongst the acute encephalitis syndrome cases in West Bengal, India

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    D Taraphdar

    2015-01-01

    Full Text Available Chikungunya virus (CHIKV infection from the acute encephalitis syndrome cases is an uncommon form and has been observed in the year 2010-11 from West Bengal, India. The case-1 and case-2 had the acute encephalitis syndrome; case-3 was of acute disseminated encephalomyelitis whereas the case-4 had the symptoms of meningo-encephalopathy with bulbar involvement. We are reporting four cases with neurological complications involving central nervous system (CNS due to CHIKV infection from this state for the first time. The virus has spread almost every districts of this state rapidly. At this stage, these cases are public health threat.

  2. Zika virus infection complicated by Guillain-Barre syndrome--case report, French Polynesia, December 2013.

    Science.gov (United States)

    Oehler, E; Watrin, L; Larre, P; Leparc-Goffart, I; Lastere, S; Valour, F; Baudouin, L; Mallet, Hp; Musso, D; Ghawche, F

    2014-03-06

    Zika fever, considered as an emerging disease of arboviral origin, because of its expanding geographic area, is known as a benign infection usually presenting as an influenza-like illness with cutaneous rash. So far, Zika virus infection has never led to hospitalisation. We describe the first case of Guillain-Barré syndrome (GBS) occurring immediately after a Zika virus infection, during the current Zika and type 1 and 3 dengue fever co-epidemics in French Polynesia.

  3. Unusual Presentation of Gianotti-Crosti Syndrome due to Epstein-Barr Virus Infection

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    Hind Saif Al Dhaheri

    2016-01-01

    Full Text Available Gianotti-Crosti syndrome (GCS is viral exanthema of childhood. It typically presents with a symmetric erythematous papular and papulovesicular eruption. It has been classically associated with hepatitis B virus, as well as rarely with Epstein-Barr virus (EBV. We report a case of GCS related to EBV infection without the classical systemic symptoms in a five-year-old male patient.

  4. Unusual Presentation of Gianotti-Crosti Syndrome due to Epstein-Barr Virus Infection

    Science.gov (United States)

    Al Dhaheri, Hind Saif; Al Kaabi, Amani; Kara Hamo, Yasmin

    2016-01-01

    Gianotti-Crosti syndrome (GCS) is viral exanthema of childhood. It typically presents with a symmetric erythematous papular and papulovesicular eruption. It has been classically associated with hepatitis B virus, as well as rarely with Epstein-Barr virus (EBV). We report a case of GCS related to EBV infection without the classical systemic symptoms in a five-year-old male patient. PMID:28050291

  5. [Effect of Astragali Radix in improving early renal damage in metabolic syndrome rats through ACE2/Mas pathway].

    Science.gov (United States)

    Wang, Qiong-ying; Liang, Wei; Jiang, Cheng; Li, Ning-yin; Xu, Han; Yang, Mi-na; Lin, Xin; Yu, Heng; Chang, Peng; Yu, Jing

    2015-11-01

    To study the expression of angiotensin converting enzyme 2 (ACE2) and angiotensin (Ang) 1-7 specific receptor Mas protain in renal blood vessels of metabolic syndrome ( MS) rats and its anti-oxidative effect. A total of 80 male SD rats were divided into four groups: the normal control group (NC, the same volume of normal saline), the MS group (high fat diet), the MS + Astragali Radix group (MS + HQ, 6 g x kg(-1) x d(-1) in gavage) and the MS + Valsartan group (MS + XST, 30 mg x kg(-1) x d(-1) in gavage). After four weeks of intervention, their general indexes, biochemical indexes and blood pressure were measured; plasma and renal tissue Ang II, malondialdehyde (MDA) and superoxide demutase (SOD) levels were measured with radioimmunoassay. The protein expressions of Mas receptor, AT1R, ACE and ACE2 were detected by western blot analysis. According to the result, compared with the NC group, the MS group and the MS + HQ group showed significant increases in systolic and diastolic pressures, body weight, fasting glucose, fasting insulin, triglycerides, free fatty acid and Ang II level of MS rats (P Mas receptor expressions (all P Mas receptor expression in renal tissues, whereas the MS + XST group showed notable decrease in AT1R (all P Mas receptor expressions in renal tissues, decrease ACE expression and change local Ang II, MDA, NO and SOD in kidneys, so as to protect early damages in renal tissues.

  6. Birt-Hogg-Dubé syndrome: novel FLCN frameshift deletion in daughter and father with renal cell carcinomas.

    Science.gov (United States)

    Näf, Ernst; Laubscher, Dominik; Hopfer, Helmut; Streit, Markus; Matyas, Gabor

    2016-01-01

    Germline mutation of the FLCN gene causes Birt-Hogg-Dubé syndrome (BHD), a rare autosomal dominant condition characterized by skin fibrofolliculomas, lung cysts, spontaneous pneumothorax and renal tumours. We identified a hitherto unreported pathogenic FLCN frameshift deletion c.563delT (p.Phe188Serfs*35) in a family of a 46-year-old woman presented with macrohematuria due to bilateral chromophobe renal carcinomas. A heritable renal cancer was suspected due to the bilaterality of the tumour and as the father of this woman had suffered from renal cancer. Initially, however, BHD was overlooked by the medical team despite the highly suggestive clinical presentation. We assume that BHD is underdiagnosed, at least partially, due to low awareness of this variable condition and to insufficient use of appropriate genetic testing. Our study indicates that BHD and FLCN testing should be routinely considered in patients with positive family or personal history of renal tumours. In addition, we demonstrate how patients and their families can play a driving role in initiating genetic diagnosis, presymptomatic testing of at-risk relatives, targeted disease management, and genetic counselling of rare diseases such as BHD.

  7. Angiopoietin-2 Is an Early Indicator of Acute Pancreatic-Renal Syndrome in Patients with Acute Pancreatitis

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    Mateusz Sporek

    2016-01-01

    Full Text Available Within the first week of the disease, acute kidney injury (AKI is among the most common causes of mortality in acute pancreatitis (AP. Recently, serum angiopoietin-2 (Ang-2 has been associated with hyperdynamic state of the systemic circulation. The aim of this study was to examine the associations between Ang-2 and the clinical AP severity during the first 72 hours of the disease, and organ disfunction, including AKI. Methods. Study included patients admitted to the surgery ward, diagnosed with AP. AKI was diagnosed according to KDIGO guidelines and renal failure according to modified Marshall scoring system. Ang-2 was determined in serum with ELISA. Results. AP was classified as mild (MAP in 71% of patients, moderately severe (MSAP in 22%, and severe (SAP in 8%. During the first 72 hours of AP, 11 patients developed AKI and 6 developed renal failure. Ang-2 at 24, 48, and 72 hours following the onset of AP symptoms significantly predicted SAP and MSAP, as well as AKI and renal failure. Also, Ang-2 significantly correlated with acute phase proteins as well as with the indicators of renal disfunction. Conclusions. Serum Ang-2 may be a relevant predictor of AP severity, in particular of the development of AP-renal syndrome.

  8. Kidney disease in heart failure: the importance of novel biomarkers for type 1 cardio-renal syndrome detection.

    Science.gov (United States)

    Palazzuoli, Alberto; McCullough, Peter A; Ronco, Claudio; Nuti, Ranuccio

    2015-08-01

    Chronic kidney disease (CKD) in heart failure (HF) has been recognized as an independent risk factor for adverse outcome, although the most important clinical trials tend to exclude patients with moderate and severe renal insufficiency. Despite this common association, the precise pathophysiological connection and liaison between heart and kidney is partially understood. Moreover, is it not enough considering how much cardio-renal syndrome type 1 is attributable to previous CKD, and how much to new-onset acute kidney injury (AKI). Neither development of AKI, its progression and time nor duration is related to an adverse outcome. An AKI definition is not universally recognized, and many confounding terms have been used in literature: "worsening renal function", "renal impairment", "renal dysfunction", etc., are all names that contribute to misunderstanding, and do not facilitate an universal classification. Therefore, AKI development should be the consequence of the basal clinical characteristics of patients, different primitive kidney disease and hemodynamic status. AKI could also be the mirror of several underlying associated diseases poorly controlled. Finally, it is not clear which is the optimal laboratory tool for identifying patients with an increased risk of AKI. In the current report, we review the different kidney diseases' impact in HF, and we analyze the modalities for AKI recognition during HF focusing our attention about some new biomarkers with potential application in the current setting.

  9. Biopsy-proven renal involvement and prognosis in 13 hispanic patients with primary Sjögren syndrome.

    Science.gov (United States)

    Carrillo-Pérez, Diego Luis; Tejeda-Maldonado, Javier; Garza-García, Carlos; Soto-Abraham, Virgilia; Hernández-Molina, Gabriela; Molina-Paredes, Giovanni Arnoldo; Uribe-Uribe, Norma O; Morales-Buenrostro, Luis E

    2017-08-29

    The aim of this study was to describe a case series of 13 Hispanic patients with primary Sjögren syndrome (pSS) and biopsy-proven renal involvement. We describe the clinical, serological and histological characteristics as well as the prognosis in a group of patients with pSS and biopsy-proven renal involvement, treated in 2 referral nephrology units in Mexico City. Thirteen patients with pSS underwent kidney biopsy (KB) over a period of 27 years. The median duration from pSS diagnosis to KB was 13.9 months. Seven patients (54%) had glomerulonephritis and 6 patients (46%) had tubulointerstitial nephritis. All patients were treated with corticosteroids and/or immunosuppressants. Eight patients (62%) remained stable or their renal function improved after a median follow-up of 12 months. This case series reflects the broad spectrum of renal involvement in pSS. We observed that in our Hispanic population, glomerular involvement was the most frequent abnormality, mainly membranous glomerulopathy, followed by tubulointerstitial disease. Tubular atrophy and interstitial fibrosis were also common biopsy findings. Treatment with corticosteroids or other immunosuppressive agents appear to slow renal disease progression. Copyright © 2017 Elsevier España, S.L.U. All rights reserved.

  10. Angiopoietin-2 Is an Early Indicator of Acute Pancreatic-Renal Syndrome in Patients with Acute Pancreatitis.

    Science.gov (United States)

    Sporek, Mateusz; Dumnicka, Paulina; Gala-Bladzinska, Agnieszka; Ceranowicz, Piotr; Warzecha, Zygmunt; Dembinski, Artur; Stepien, Ewa; Walocha, Jerzy; Drozdz, Ryszard; Kuzniewski, Marek; Kusnierz-Cabala, Beata

    2016-01-01

    Within the first week of the disease, acute kidney injury (AKI) is among the most common causes of mortality in acute pancreatitis (AP). Recently, serum angiopoietin-2 (Ang-2) has been associated with hyperdynamic state of the systemic circulation. The aim of this study was to examine the associations between Ang-2 and the clinical AP severity during the first 72 hours of the disease, and organ disfunction, including AKI. Methods. Study included patients admitted to the surgery ward, diagnosed with AP. AKI was diagnosed according to KDIGO guidelines and renal failure according to modified Marshall scoring system. Ang-2 was determined in serum with ELISA. Results. AP was classified as mild (MAP) in 71% of patients, moderately severe (MSAP) in 22%, and severe (SAP) in 8%. During the first 72 hours of AP, 11 patients developed AKI and 6 developed renal failure. Ang-2 at 24, 48, and 72 hours following the onset of AP symptoms significantly predicted SAP and MSAP, as well as AKI and renal failure. Also, Ang-2 significantly correlated with acute phase proteins as well as with the indicators of renal disfunction. Conclusions. Serum Ang-2 may be a relevant predictor of AP severity, in particular of the development of AP-renal syndrome.

  11. Effects of tacrolimus and cyclosporine treatment on metabolic syndrome and cardiovascular risk factors after renal transplantation: a meta-analysis.

    Science.gov (United States)

    Xue, Wenrui; Zhang, Qiang; Xu, Yue; Wang, Wei; Zhang, Xiaodong; Hu, Xiaopeng

    2014-01-01

    The therapeutic success of renal transplantation has been largely attributable to the development of effective and balanced immunosuppressive treatment regimens. This study provides a meta-analysis of a series of randomized controlled trials that compared the effects of tacrolimus and cyclosporine on metabolic syndrome (MetS) and cardiovascular risk factors after renal transplantation. We searched various electronic databases and bibliographies, including MEDLINE, the Cochrane Central Register of Controlled Trials, and EMBASE, for relevant studies published prior to October 2012. Our meta-analysis included five randomized controlled trials that examined a total of 923 patients. The tacrolimus group and the cyclosporine group exhibited no significant differences in MetS incidence after renal transplantation; risk ratio (RR): 1.06, 95% confidence interval (CI): 0.73-1.55, P = 0.76. Cyclosporine treatment was associated with a higher incidence of hyperlipidemia (RR: 0.50, 95% CI: 0.39-0.64, P cyclosporine treatment was associated with a higher incidence of hypertension (RR: 0.91, 95% CI: 0.83-1.00, P = 0.06) after renal transplantation compared to tacrolimus treatment, and tacrolimus treatment was associated with a higher incidence of diabetes after renal transplantation (RR: 1.79, 95% CI: 0.98-3.27, P = 0.06) compared to cyclosporine treatment. Compared to tacrolimus treatment, cyclosporine treatment was associated with a higher incidence of hyperlipidemia. Future large-scale studies are expected to be conducted to further confirm our findings.

  12. Effects of Entecavir and Tenofovir on Renal Function in Patients with Hepatitis B Virus-Related Compensated and Decompensated Cirrhosis.

    Science.gov (United States)

    Park, Jihye; Jung, Kyu Sik; Lee, Hye Won; Kim, Beom Kyung; Kim, Seung Up; Kim, Do Young; Ahn, Sang Hoon; Han, Kwang-Hyub; Park, Jun Yong

    2017-06-27

    The renal effects of nucleos(t)ide analogs in patients with chronic hepatitis B are controversial. We aimed to compare the impact of entecavir (ETV) and tenofovir (TDF) on renal function in patients with hepatitis B virus (HBV)-related cirrhosis. We performed a retrospective cohort study of 235 consecutive treatment-naïve patients with HBV-related cirrhosis who were treated with ETV or TDF between December 2012 and November 2013 at Severance Hospital, Seoul, Korea. Compensated cirrhosis was noted in 183 patients (ETV 130, TDF 53), and decompensated cirrhosis was noted in 52 patients (ETV 32, TDF 20). There were no significant changes in estimated glomerular filtration rates (eGFR) from baseline in either the ETV- or TDF-treated groups at week 96 (CKD-EPI, ETV -1.68% and TDF -5.03%, p=0.358). Using a multivariate analysis, the significant factors associated with a decrease in eGFR >20% were baseline eGFR, diabetes mellitus (DM), and the use of diuretics. The use of antiviral agents and baseline decompensation were not determined to be significant factors. In patients with HBV-related cirrhosis, TDF has shown similar renal safety to that of ETV over a two year period. Renal function should be closely monitored, especially in patients who exhibit decreasing eGFR, DM, and the use of diuretics.

  13. Síndrome pulmón-riñón Pulmonary-renal syndrome

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    Jorge A. Risso

    2009-12-01

    Full Text Available El síndrome pulmón-riñón se define como una combinación de hemorragia alveolar difusa y glomerulonefritis. La coexistencia de estas dos afecciones clínicas se produce por enfermedades con distintos mecanismos patogénicos. Las vasculitis sistémicas primarias y el síndrome de Goodpasture son las etiologías más frecuentes. El lupus eritematoso sistémico, otras colagenopatías, las vasculitis con anticuerpos anticitoplasma de los neutrófilos negativos y las secundarias a drogas son causas mucho menos comunes. El diagnóstico temprano basado en criterios clínicos, radiológicos, de laboratorio e histológicos, permite iniciar el tratamiento disminuyendo su elevada morbi-mortalidad. La terapéutica se basa en altas dosis de corticoides, inmunosupresores, inhibidores del factor de necrosis tumoral y plasmaféresis.The pulmonary-renal syndrome is defined as a combination of diffuse alveolar hemorrhage and glomerulonephritis. The coexistence of these two clinical conditions is due to diseases with different pathogenic mechanisms. Primary systemic vasculitis and Goodpasture syndrome are the most frequent etiologies. Systemic lupus erythematosus, connective tissue diseases, negative anti neutrophil cytoplasmic antibody vasculitis and those secondary to drugs are far less common causes. An early diagnosis based on clinical, radiologic, laboratory and histologic criteria enables early treatment, thus diminishing its high morbility-mortality rate. Therapy is based on high doses of corticosteroids, immunosuppressants, tumor necrosis factor inhibitors and plasmapheresis.

  14. Budd-Chiari syndrome in urology: Impact on nephrectomy for advanced renal cell carcinoma

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    Samir P Shirodkar

    2011-01-01

    Full Text Available Objectives : Budd-Chiari syndrome (BCS is a poorly understood entity in urology. It results from obstruction of the hepatic veins and the subsequent complications. It has been infrequently reported to be secondary to hepatic venous obstruction from invasion by an inferior vena cava (IVC tumor thrombus in renal cell carcinoma (RCC. We report the largest known series of patients with RCC and BCS. Patients and Methods : Ten patients presented to a tertiary hospital with locally advanced RCC with IVC tumor thrombus. All were evaluated and had clinical or radiographic evidence of BCS. All underwent nephrectomy, IVC thrombectomy or ligation, and tumor removal from the hepatic veins. The perioperative and pathological factors were measured. These included estimated blood loss (EBL and transfusions. Inpatient factors including duration of intubation, length of intensive care unit (ICU stay, and overall length of stay (LOS were recorded. The tumor-free status was evaluated. Results : The average age was 59 years. No intraoperative deaths occurred. Two intraoperative complications were noted. The mean EBL was 4244 cc; mean surgery length was 8 hours 12 minutes; and the mean ICU stay was nine days. The overall LOS averaged 13.25 days. One patient died postoperatively of sepsis and multisystem organ failure. One patient required reoperation for an abdominal wall hematoma caused by subcutaneous enoxaparin administration. Average follow-up was 28 months. Five patients are alive with no evidence of disease. Conclusions : Budd-Chiari syndrome is a rare entity in urology, with a potential for significant morbidity and mortality. Surgical excision of the primary tumor along with thrombectomy results in alleviation of BCS and improvement in the patient.

  15. Horizontal transmission dynamics of White spot syndrome virus by cohabitation trials in juvenile Penaeus monodon and P. vannamei

    NARCIS (Netherlands)

    Ngo Xuan, T.; Verreth, J.A.J.; Vlak, J.M.; Jong, de M.C.M.

    2014-01-01

    White spot syndrome virus (WSSV), a rod-shaped double-stranded DNA virus, is an infectious agent causing fatal disease in shrimp farming around the globe. Within shrimp populations WSSV is transmitted very fast, however, the modes and dynamics of transmission of this virus are not well understood. I

  16. A Case with Mega Cisterna Magna Renal and Ear Anomalies: Is This a New Syndrome?

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    Çapan Konca

    2013-01-01

    Full Text Available Background. Extrarenal pathologies may be associated with renal position and fusion anomalies. According to the literature, our patient is the first horseshoe kidney case that had mega cisterna magna, arachnodactyly, and mild mental retardation. Case Report. A 9-year-old boy admitted because of the myoclonic jerks. He had a dysmorphic face, low-set and cup-shaped ears, arachnodactyly, and mild mental retardation. The patient’s laboratory findings were normal except for a mild leucocytosis and hypochromic microcytic anemia. His cerebrospinal fluid was cytologically and biochemically normal. Cranial MRI revealed 1.5 cm diametered mega cisterna magna in the retrocerebellar region. Although there were no significant epileptical discharges in the electroencephalography, there were slow wave discharges arising from the anterior regions of both hemispheres. Because he had stomachache, abdominal ultrasonography was performed, and horseshoe kidney was determined. Abdominal CT did not reveal any abnormalities except the horseshoe kidney. There were not any cardiac pathologies in echocardiography. He had normal 46XY karyotype and there were no repeated chromosomal derangements, but we could not evaluate for molecular and submicroscopic somatic changes. He was treated with valproic acid and myoclonic jerks did not repeat. Conclusion. We suggest that the presence of these novel findings may represent a newly recognized, separate syndrome.

  17. Nutcracker Syndrome: laparoscopic external stenting of the renal vein (“the shield technique”)

    Science.gov (United States)

    Korkes, Fernando; Silveira, Marcel; Neves-Neto, Oseas Castro; Brandão, Luiz Franco; Tobias-Machado, Marcos; Wolosker, Nelson; Nasser, Felipe; Maurano, Alexandre

    2017-01-01

    ABSTRACT Nutcracker syndrome refers to the complex of clinical symptoms caused by the compression of the left renal vein (LRV) between the abdominal aorta and the superior mesenteric artery, leading to stenosis of the aortomesenteric portion of the LRV and dilatation of the distal portion. Hematuria, proteinuria, flank pain, varicocele and pelvic congestion may occur, occurring more frequently in young adults. Conservative management, might be the option whenever it is possible. When surgical treatment is required, classically open surgery have been performed, with major surgeries as LRV transposition or bypass techniques. The main caveats regards the fact that these are large and risky surgeries. Endovascular surgery with venous stent placement has gained some space as it is minimally invasive alternative. However, venous stents are associated with a high number of trombotic complications and in many cases requirement of life-long anticoagulants. External stenting of the LRV with this “shield technique” is a minimally invasive alternative, with good medium term results. We herein demonstrate our second experience with the technique of this surgery in a patient with 12 months of follow up and excellent results. PMID:27649112

  18. Nutcracker Syndrome: laparoscopic external stenting of the renal vein (″the shield technique″).

    Science.gov (United States)

    Korkes, Fernando; Silveira, Marcel; Neves-Neto, Oseas Castro; Brandao, Luiz Franco; Tobias-Machado, Marcos; Wolosker, Nelson; Nasser, Felipe; Maurano, Alexandre

    2017-01-01

    Nutcracker syndrome refers to the complex of clinical symptoms caused by the compression of the left renal vein (LRV) between the abdominal aorta and the superior mesenteric artery, leading to stenosis of the aortomesenteric portion of the LRV and dilatation of the distal portion. Hematuria, proteinuria, flank pain, varicocele and pelvic congestion may occur, occurring more frequently in young adults. Conservative management, might be the option whenever it is possible. When surgical treatment is required, classically open surgery have been performed, with major surgeries as LRV transposition or bypass techniques. The main caveats regards the fact that these are large and risky surgeries. Endovascular surgery with venous stent placement has gained some space as it is minimally invasive alternative. However, venous stents are associated with a high number of trombotic complications and in many cases requirement of life-long anticoagulants. External stenting of the LRV with this "shield technique" is a minimally invasive alternative, with good medium term results. We herein demonstrate our second experience with the technique of this surgery in a patient with 12 months of follow up and excellent results. Copyright® by the International Brazilian Journal of Urology.

  19. Immunohistochemical characterization of renal tumors in patients with Birt-Hogg-Dubé syndrome.

    Science.gov (United States)

    Iribe, Yasuhiro; Kuroda, Naoto; Nagashima, Yoji; Yao, Masahiro; Tanaka, Reiko; Gotoda, Hiroko; Kawakami, Fumi; Imamura, Yoshiaki; Nakamura, Yasushi; Ando, Midori; Araki, Akinobu; Matsushima, Jun; Nakatani, Yukio; Furuya, Mitsuko

    2015-03-01

    Birt-Hogg-Dubé syndrome (BHD) is an autosomal dominant disorder associated with a germline mutation of folliculin (FLCN). The affected families are at a high risk for developing multiple renal cell carcinomas (RCC). Little is known about the immunostaining patterns of mutant FLCN-associated RCCs. We investigated 32 RCCs obtained from 17 BHD patients. The studied tumors included chromophobe RCCs (n = 15), hybrid oncocytic/chromophobe tumors (HOCT) (n = 14) and clear cell RCCs (n = 3). Almost all chromophobe RCCs and HOCTs revealed positive staining for S100A1, Ksp-cadherin and CD82. They stained either focally or diffusely for CK7, and were negative for CA-IX. All clear cell RCCs were positively stained for CA-IX and negative for CK7. These data confirmed that mutant FLCN-associated oncocytic and clear cell RCCs exhibited generally similar immunostaining patterns compared to their sporadic counterparts. Frequent positive staining for S100A1, Ksp-cadherin and CD82 in chromophobe RCCs and HOCTs indicated that these two types were relatively similar rather than distinctively different in their patterns of immunoreactivity. Characteristic peri-nuclear halos and polygonal cells with clear cytoplasm, which often misleads pathologists into the diagnosis of clear cell RCC, should be carefully examined using an immunohistochemical panel including CA-IX, Ksp-cadherin, CD82 and CK7.

  20. Climate variability and hemorrhagic fever with renal syndrome transmission in Northeastern China.

    Science.gov (United States)

    Zhang, Wen-Yi; Guo, Wei-Dong; Fang, Li-Qun; Li, Chang-Ping; Bi, Peng; Glass, Gregory E; Jiang, Jia-Fu; Sun, Shan-Hua; Qian, Quan; Liu, Wei; Yan, Lei; Yang, Hong; Tong, Shi-Lu; Cao, Wu-Chun

    2010-07-01

    The transmission of hemorrhagic fever with renal syndrome (HFRS) is influenced by climatic variables. However, few studies have examined the quantitative relationship between climate variation and HFRS transmission. We examined the potential impact of climate variability on HFRS transmission and developed climate-based forecasting models for HFRS in northeastern China. We obtained data on monthly counts of reported HFRS cases in Elunchun and Molidawahaner counties for 1997-2007 from the Inner Mongolia Center for Disease Control and Prevention and climate data from the Chinese Bureau of Meteorology. Cross-correlations assessed crude associations between climate variables, including rainfall, land surface temperature (LST), relative humidity (RH), and the multivariate El Niño Southern Oscillation (ENSO) index (MEI) and monthly HFRS cases over a range of lags. We used time-series Poisson regression models to examine the independent contribution of climatic variables to HFRS transmission. Cross-correlation analyses showed that rainfall, LST, RH, and MEI were significantly associated with monthly HFRS cases with lags of 3-5 months in both study areas. The results of Poisson regression indicated that after controlling for the autocorrelation, seasonality, and long-term trend, rainfall, LST, RH, and MEI with lags of 3-5 months were associated with HFRS in both study areas. The final model had good accuracy in forecasting the occurrence of HFRS. Climate variability plays a significant role in HFRS transmission in northeastern China. The model developed in this study has implications for HFRS control and prevention.

  1. Acute renal failure due to abdominal compartment syndrome: report on four cases and literature review

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    Cleva Roberto de

    2001-01-01

    Full Text Available We report on 4 cases of abdominal compartment syndrome complicated by acute renal failure that were promptly reversed by different abdominal decompression methods. Case 1: A 57-year-old obese woman in the post-operative period after giant incisional hernia correction with an intra-abdominal pressure of 24 mm Hg. She was sedated and curarized, and the intra-abdominal pressure fell to 15 mm Hg. Case 2: A 73-year-old woman with acute inflammatory abdomen was undergoing exploratory laparotomy when a hypertensive pneumoperitoneum was noticed. During the surgery, enhancement of urinary output was observed. Case 3: An 18-year-old man who underwent hepatectomy and developed coagulopathy and hepatic bleeding that required abdominal packing, developed oliguria with a transvesical intra-abdominal pressure of 22 mm Hg. During reoperation, the compresses were removed with a prompt improvement in urinary flow. Case 4: A 46-year-old man with hepatic cirrhosis was admitted after incisional hernia repair with intra-abdominal pressure of 16 mm Hg. After paracentesis, the intra-abdominal pressure fell to 11 mm Hg.

  2. Spatiotemporal heterogeneity analysis of hemorrhagic fever with renal syndrome in China using geographically weighted regression models.

    Science.gov (United States)

    Li, Shujuan; Ren, Hongyan; Hu, Wensheng; Lu, Liang; Xu, Xinliang; Zhuang, Dafang; Liu, Qiyong

    2014-11-25

    Hemorrhagic fever with renal syndrome (HFRS) is an important public health problem in China. The identification of the spatiotemporal pattern of HFRS will provide a foundation for the effective control of the disease. Based on the incidence of HFRS, as well as environmental factors, and social-economic factors of China from 2005-2012, this paper identified the spatiotemporal characteristics of HFRS distribution and the factors that impact this distribution. The results indicate that the spatial distribution of HFRS had a significant, positive spatial correlation. The spatiotemporal heterogeneity was affected by the temperature, precipitation, humidity, NDVI of January, NDVI of August for the previous year, land use, and elevation in 2005-2009. However, these factors did not explain the spatiotemporal heterogeneity of HFRS incidences in 2010-2012. Spatiotemporal heterogeneity of provincial HFRS incidences and its relation to environmental factors would provide valuable information for hygiene authorities to design and implement effective measures for the prevention and control of HFRS in China.

  3. Spatiotemporal Heterogeneity Analysis of Hemorrhagic Fever with Renal Syndrome in China Using Geographically Weighted Regression Models

    Directory of Open Access Journals (Sweden)

    Shujuan Li

    2014-11-01

    Full Text Available Hemorrhagic fever with renal syndrome (HFRS is an important public health problem in China. The identification of the spatiotemporal pattern of HFRS will provide a foundation for the effective control of the disease. Based on the incidence of HFRS, as well as environmental factors, and social-economic factors of China from 2005–2012, this paper identified the spatiotemporal characteristics of HFRS distribution and the factors that impact this distribution. The results indicate that the spatial distribution of HFRS had a significant, positive spatial correlation. The spatiotemporal heterogeneity was affected by the temperature, precipitation, humidity, NDVI of January, NDVI of August for the previous year, land use, and elevation in 2005–2009. However, these factors did not explain the spatiotemporal heterogeneity of HFRS incidences in 2010–2012. Spatiotemporal heterogeneity of provincial HFRS incidences and its relation to environmental factors would provide valuable information for hygiene authorities to design and implement effective measures for the prevention and control of HFRS in China.

  4. A case of passenger lymphocyte syndrome following minor ABO incompatible renal transplantation

    Directory of Open Access Journals (Sweden)

    Anju Dubey

    2014-01-01

    Full Text Available Immune hemolysis is one of the adverse effects that can occur following solid organ transplantation. Understanding the clinical settings and the various causes is necessary for prompt diagnosis and appropriate management. One such condition is passenger lymphocyte syndrome (PLS. This case report describes the case of a 27-year-old male renal allograft recipient of the B-positive blood group who received a kidney from an O-positive donor. Postoperatively, the patient showed declining hemoglobin (Hb level and was transfused with B-group packed RBCs (PRBCs, following which there was steep fall in Hb level. A request for PRBCs was sent to the blood bank and this time cross-match with B-group PRBCs showed incompatibility. The patient′s RBCs were found to be strongly DAT (direct anti-globulin test positive and the eluate showed the presence of anti-B with a titer of 32. Thus, diagnosis of probable PLS was made. The patient was managed with methylprednisolone, plasmapheresis and O-group PRBCs. Gradually his condition improved and was discharged in stable condition.

  5. A case of passenger lymphocyte syndrome following minor ABO incompatible renal transplantation.

    Science.gov (United States)

    Dubey, Anju; Pandey, Hemchandra; Sonker, Atul; Chaudhary, Rajendra K

    2014-01-01

    Immune hemolysis is one of the adverse effects that can occur following solid organ transplantation. Understanding the clinical settings and the various causes is necessary for prompt diagnosis and appropriate management. One such condition is passenger lymphocyte syndrome (PLS). This case report describes the case of a 27-year-old male renal allograft recipient of the B-positive blood group who received a kidney from an O-positive donor. Postoperatively, the patient showed declining hemoglobin (Hb) level and was transfused with B-group packed RBCs (PRBCs), following which there was steep fall in Hb level. A request for PRBCs was sent to the blood bank and this time cross-match with B-group PRBCs showed incompatibility. The patient's RBCs were found to be strongly DAT (direct anti-globulin test) positive and the eluate showed the presence of anti-B with a titer of 32. Thus, diagnosis of probable PLS was made. The patient was managed with methylprednisolone, plasmapheresis and O-group PRBCs. Gradually his condition improved and was discharged in stable condition.

  6. The impact of the vaccination program for hemorrhagic fever with renal syndrome in Hu County, China.

    Science.gov (United States)

    Xiao, Dan; Wu, Kejian; Tan, Xin; Yan, Tiecheng; Li, Haitao; Yan, Yongping

    2014-02-03

    The incidence of hemorrhagic fever with renal syndrome (HFRS) in Hu County ranked third of all counties in China in 2010. Although this county has provided a HFRS vaccination program freely since 1994, the impact of HFRS remains quite substantial. In order to continue the vaccination program effectively and control HFRS, a detailed understanding of the effect of the vaccination program should be undertaken. The Cochran-Armitage trend test was employed to examine the temporal trends of HFRS incidences, mortality rate and vaccination compliance. Temporal cluster analysis was performed to detect time periods of high HFRS risk. Cross correlation analysis was conducted to detect the correlation between HFRS incidence and vaccination compliance. Wavelet analysis was employed to detect the shift of the periodicity of HFRS. Between 1971 and 2011, the HFRS incidence and mortality rate ranged from 9.53/100,000 to 300.57/100,000 and 0 to 24.91/100,000, respectively, with a fluctuating but distinctly declining trend (incidence: Z=-34.38, P1994. In conclusion, the increase in vaccination compliance may play an important role in HFRS control and prevention in Hu County, China.

  7. DETECTION OF WHITE SPOT SYNDROME VIRUS(WSSV) OF PENAEUS CHINENSIS BY IN SITU HYBRIDIZATION

    Institute of Scientific and Technical Information of China (English)

    2000-01-01

    White Spot Syndrome Virus (WSSV) was purified from hemolymph of infected shrimp. After nucleic acid extraction from the purified virus particles, EcoR I-digested fragments of the WSSV genome were cloned; three of these fragments were used as non-radioactive probes labeled with DIG-11-dUTP. The probes hybridized in situ, with sections located in the nuclei of all WSSV-infected tissues. The virus was detected in the gill, stomach, epidermis, and connective tissue and so on, but not detected in healthy shrimp tissues and epithelial cells of hepatopancreatic tubules of diseased shrimp.

  8. Genomic characterization of Indian isolates of egg drop syndrome 1976 virus.

    Science.gov (United States)

    Raj, G D; Sivakumar, S; Sudharsan, S; Mohan, A C; Nachimuthu, K

    2001-02-01

    Five Indian isolates of egg drop syndrome (EDS) 1976 virus and the reference strain 127 were compared by restriction enzyme analysis of viral DNA, and the hexon gene amplified by polymerase chain reaction. Using these techniques, no differences were seen among these viruses. However, partial sequencing of the hexon gene revealed major differences (4.6%) in one of the isolates sequenced, EDS Kerala. Phylogenetic analysis also placed this isolate in a different lineage compared with the other isolates. The need for constant monitoring of the genetic nature of the field isolates of EDS viruses is emphasized.

  9. Egg drop syndrome 1976 (EDS-76) virus infection in inadequately vaccinated chickens.

    Science.gov (United States)

    Cook, J K

    1983-01-01

    Experiments were carried out to study the epizootiology of the egg drop syndrome 1976 virus infection in chickens that were not adequately protected by vaccination. Virus excretion was demonstrated when chickens vaccinated at the recommended level were challenged within 10 days of vaccination and when chickens were challenged 6 weeks after administration of low doses of vaccine. In the latter experiment, those chickens which shed virus had all shown a poor haemagglutination inhibition antibody response to vaccination but had high serum neutralisation antibody titres when challenged.

  10. Production and Characterization of Monoclonal Antibodies of Shrimp White Spot Syndrome Virus Envelope Protein VP28

    Institute of Scientific and Technical Information of China (English)

    Wan-gang GU; Jun-fa YUAN; Ge-lin XU; Li-juan LI; Ni LIU; Cong ZHANG; Jian-hong ZHANG; Zheng-li SHI

    2007-01-01

    BALB/c mice were immunized with purified White spot syndrome virus (WSSV).Six monoclonal antibody cell lines were selected by ELISA with VP28 protein expressed in E.coll in vitro neutralization experiments showed that 4 of them could inhibit the virus infection in crayfish.Westernblot suggested that all these monoclonal antibodies were against the conformational structure of VP28.The monoclonal antibody 7B4 was labeled with colloidal gold particles and used to locate the VP28 on virus envelope by immunogold labeling.These monoclonal antibodies could be used to develop immunological diagnosis methods for WSSV infection.

  11. Molecular Interactions of the Hantaan Virus Nucleocapsid Protein

    Science.gov (United States)

    1991-09-12

    Antoniades et, §J^., 1985/ Avsic- Zupanc et. al., 1989/ Brummer-Korvenkonto et. al., 1980). Several NE viruses have been isolated, the type strain...virus: Comparison to other causative agents of hemorrhagic fever with renal syndrome. Virology 176: 114-125. Avsic- Zupanc , T., B. Cizman, A. Gligic, G

  12. Long term metabolic syndrome induced by a high fat high fructose diet leads to minimal renal injury in C57BL/6 mice

    OpenAIRE

    Dissard, R.; Klein, J.; Caubet, C.; Breuil, B.; Siwy, J.; Hoffman, J.; Sicard, L.; Ducasse, L.; Rascalou, S.; Payre, B.; Buleon, M.; Mullen, W.; Mischak, H; Tack, I.; Bascands, J.

    2013-01-01

    Metabolic syndrome can induce chronic kidney disease in humans. Genetically engineered mice on a C57BL/6 background are highly used for mechanistic studies. Although it has been shown that metabolic syndrome induces cardiovascular lesions in C57BL/6 mice, in depth renal phenotyping has never been performed. Therefore in this study we characterized renal function and injury in C57BL/6 mice with long-term metabolic syndrome induced by a high fat and fructose diet (HFFD). C57BL/6 mice received a...

  13. Long Term Metabolic Syndrome Induced by a High Fat High Fructose Diet Leads to Minimal Renal Injury in C57BL/6 Mice

    OpenAIRE

    Dissard, Romain; Klein, Julie; Caubet, Cécile; Breuil, Benjamin; Siwy, Justyna; Hoffman, Janosch; Sicard, Laurent; Ducassé, Laure; Rascalou, Simon; Payre, Bruno; Buléon, Marie; Mullen, William; Mischak, Harald; Tack, Ivan; Bascands, Jean-Loup

    2013-01-01

    Metabolic syndrome can induce chronic kidney disease in humans. Genetically engineered mice on a C57BL/6 background are highly used for mechanistic studies. Although it has been shown that metabolic syndrome induces cardiovascular lesions in C57BL/6 mice, in depth renal phenotyping has never been performed. Therefore in this study we characterized renal function and injury in C57BL/6 mice with long-term metabolic syndrome induced by a high fat and fructose diet (HFFD). C57BL/6 mice received a...

  14. Duck egg drop syndrome virus: an emerging Tembusu-related flavivirus in China.

    Science.gov (United States)

    Liu, PeiPei; Lu, Hao; Li, Shuang; Wu, Ying; Gao, George Fu; Su, JingLiang

    2013-08-01

    Duck egg drop syndrome virus (DEDSV) is a newly emerging pathogenic flavivirus isolated from ducks in China. DEDSV infection mainly results in severe egg drop syndrome in domestic poultry, which leads to huge economic losses. Thus, the discovery of ways and means to combat DEDSV is urgent. Since 2010, a remarkable amount of progress concerning DEDSV research has been achieved. Here, we review current knowledge on the epidemiology, symptomatology, and pathology of DEDSV. A detailed dissection of the viral genome and polyprotein sequences, comparative analysis of viral antigenicity and the corresponding potential immunity against the virus are also summarized. Current findings indicate that DEDSV should be a distinct species from Tembusu virus. Moreover, the adaption of DEDSV in wildlife and its high homology to pathogenic flaviviruses (e.g., West Nile virus, Japanese encephalitis virus, and dengue virus), illustrate its reemergence and potential to become a zoonotic pathogen that should not be overlooked. Detailed insight into the antigenicity and corresponding immunity against the virus is of clear significance for the development of vaccines and antiviral drugs specific for DEDSV.

  15. Factors Associated with Death Due to 2009 Influenza A (H1N1) Virus Infection and Acute Respiratory Distress Syndrome in Beijing, 2009-2011

    Institute of Scientific and Technical Information of China (English)

    Jin-qian; Zhang; Li-cheng; Zhang; Na; Ren; Ming; Zhang; Li-min; Guo; Xing-wang; Li; Jun; Cheng

    2012-01-01

    Objective Patients with H1N1 virus infection were hospitalized and quarantined, and some of them developed into acute respiratory failure, and were transfered to the medical intensive care unit of Beijing Ditan Hospital, Capital Medical University in Beijing, China. Methods The clinical features and preliminary epidemiologic findings among 30 patients with confirmed H1N1 virus infection who developed into acute respiratory failure for ventilatory support were investigated. Results A total of 30 patients(37.43 ± 18.80 years old) with 2009 influenza A(H1N1) related acute respiratory distress syndrome(ARDS) received treatment with mechanical ventilation, 15 cases of whom were male and 17 cases died of ARDS. Fatal cases were significantly associated with an APACHE Ⅱ score(P = 0.016), but not with PaO 2 /FIO 2(P = 0.912) and chest radiograph(P = 0.333). The most common complication was acute renal failure(n = 9). Five patients received extracorporeal membrane oxygenation(ECMO), 3 of whom died and the others survived. The major causes of death were multiple organ dysfunction syndrome(MODS)(39%), intractable respiratory failure(27%) and sepsis(20%). Conclusions Most patients with respiratory failure due to influenza A(H1N1) virus infection were young, with a high mortality, particularly associated with APACHE Ⅱ score, secondary infection of lung or type 2 diabetes mellitus.

  16. The role of egg drop syndrome virus in acute respiratory disease of goslings.

    Science.gov (United States)

    Ivanics, E; Palya, V; Glavits, R; Dan, A; Palfi, V; Revesz, T; Benko, M

    2001-06-01

    An outbreak of severe acute respiratory disease characterized by tracheitis and bronchitis was observed in young goslings on a large-scale goose farm in Hungary. Histological examination revealed amphophilic intranuclear inclusion bodies in the superficial epithelial cells of the trachea and bronchi. Adenovirus-like particles were detected by electron microscopy, and the virus isolated from the trachea and the lungs was identified as egg drop syndrome (EDS) virus by serological and genomic examination. The clinical and pathological signs were reproduced by intratracheal administration of the virus isolate to 1-day-old goslings free of EDS antibodies. The presence of EDS virus DNA in different organs of the naturally and experimentally infected goslings was detected by polymerase chain reaction. This is the first report on the involvement of EDS virus in severe respiratory disease of geese.

  17. Renal Cell Carcinoma in A Patient with Kartagener Syndrome: First Case Report in English Language

    Directory of Open Access Journals (Sweden)

    Erkin Sağlam

    2015-03-01

    Full Text Available Cardiac and pulmonary anomalies are common among patients with situs inversus totalis. Renal anomalies, including renal agenesis, dysplasia, hypoplasia, ectopia, polycystic kidney, and horseshoe kidney have been reported. We report a case of renal cell carcinoma in a patient with situs inversus totalis (SIT. Our case represents the fourth case report of renal cell carcinoma in a patient with situs inversus totalis and to the best of our knowledge this is the first report in English language. Due to the higher frequency of cardiac, pulmonary, renal, and vascular anomalies the management of patients with situs inversus and urologic disease requires careful preoperative evaluation.

  18. Zika virus infection and Guillain-Barré syndrome in three patients from Suriname

    NARCIS (Netherlands)

    T. Langerak (Thomas); Yang, H. (Harvey); Baptista, M. (Mark); Doornekamp, L. (Laura); Kerkman, T. (Tessa); Codrington, J. (John); Roosblad, J. (Jimmy); Vreden, S.G.S. (Stephen G.S.); E.I. de Bruin (Esther); R. Mögling (Ramona); B.C. Jacobs (Bart); S.D. Pas (Suzan); C.H. Geurts van Kessel (Corine); C.B.E.M. Reusken (Chantal); M.P.G. Koopmans D.V.M. (Marion); E.C.M. van Gorp (Eric); Alberga, H. (Henk)

    2016-01-01

    textabstractWe present three patients from Suriname who were diagnosed with Guillain-Barré syndrome (GBS) during the Zika virus (ZIKV) outbreak in this country. One patient had a positive ZIKV urine real-time RT-PCR (qRT-PCR) result. The other two patients had a negative ZIKV urine qRT-PCR but a

  19. Zika virus infection and Guillain-Barré syndrome in three patients from Suriname

    NARCIS (Netherlands)

    T. Langerak (Thomas); Yang, H. (Harvey); Baptista, M. (Mark); Doornekamp, L. (Laura); Kerkman, T. (Tessa); Codrington, J. (John); Roosblad, J. (Jimmy); Vreden, S.G.S. (Stephen G.S.); E.I. de Bruin (Esther); R. Mögling (Ramona); B.C. Jacobs (Bart); S.D. Pas (Suzan); C.H. Geurts van Kessel (Corine); C.B.E.M. Reusken (Chantal); M.P.G. Koopmans D.V.M. (Marion); E.C.M. van Gorp (Eric); Alberga, H. (Henk)

    2016-01-01

    textabstractWe present three patients from Suriname who were diagnosed with Guillain-Barré syndrome (GBS) during the Zika virus (ZIKV) outbreak in this country. One patient had a positive ZIKV urine real-time RT-PCR (qRT-PCR) result. The other two patients had a negative ZIKV urine qRT-PCR but a pos

  20. Evolutionary trajectory of white spot syndrome virus (WSSV) genome shrinkage during spread in Asia

    NARCIS (Netherlands)

    Zwart, M.P.; Bui Thi Minh Dieu,; Hemerik, L.; Vlak, J.M.

    2010-01-01

    Background - White spot syndrome virus (WSSV) is the sole member of the novel Nimaviridae family, and the source of major economic problems in shrimp aquaculture. WSSV appears to have rapidly spread worldwide after the first reported outbreak in the early 1990s. Genomic deletions of various sizes oc

  1. Transmission of white spot syndrome virus (WSSV) from Dendronereis spp. (Peters) (Nereididae) to penaeid shrimp

    NARCIS (Netherlands)

    Haryadi, D.; Verreth, J.A.J.; Verdegem, M.C.J.; Vlak, J.M.

    2015-01-01

    Dendronereis spp. (Peters) (Nereididae) is a common polychaete in shrimp ponds built on intertidal land and is natural food for shrimp in traditionally managed ponds in Indonesia. White spot syndrome virus (WSSV), an important viral pathogen of the shrimp, can replicate in this polychaete (Desrina e

  2. Zika virus infection and Guillain-Barré syndrome in three patients from Suriname

    NARCIS (Netherlands)

    T. Langerak (Thomas); Yang, H. (Harvey); Baptista, M. (Mark); Doornekamp, L. (Laura); Kerkman, T. (Tessa); Codrington, J. (John); Roosblad, J. (Jimmy); Vreden, S.G.S. (Stephen G.S.); E.I. de Bruin (Esther); R. Mögling (Ramona); B.C. Jacobs (Bart); S.D. Pas (Suzan); C.H. Geurts van Kessel (Corine); C.B.E.M. Reusken (Chantal); M.P.G. Koopmans D.V.M. (Marion); E.C.M. van Gorp (Eric); Alberga, H. (Henk)

    2016-01-01

    textabstractWe present three patients from Suriname who were diagnosed with Guillain-Barré syndrome (GBS) during the Zika virus (ZIKV) outbreak in this country. One patient had a positive ZIKV urine real-time RT-PCR (qRT-PCR) result. The other two patients had a negative ZIKV urine qRT-PCR but a pos

  3. Low-dose growth hormone and human immunodeficiency virus-associated lipodystrophy syndrome: a pilot study

    DEFF Research Database (Denmark)

    Andersen, O; Haugaard, S B; Flyvbjerg, A

    2004-01-01

    BACKGROUND: Treatment with high doses (2-6 mg day(-1)) of human growth hormone (hGH) in patients with human immunodeficiency virus (HIV)-associated lipodystrophy syndrome (HALS) has been shown to increase concentrations of total insulin-like growth-factor-I (IGF-I) more than twofold greater than ...

  4. Transcriptional analysis of the ribonucleotide reductase genes in shrimp white spot syndrome virus

    NARCIS (Netherlands)

    Tsai, M.F.; Lo, C.F.; Hulten, van M.C.W.; Tzeng, H.F.; Chou, C.M.; Huang, C.J.; Wang, C.S.

    2000-01-01

    The causative agent of white spot syndrome (WSS) is a large double-stranded DNA virus, WSSV, which is probably a representative of a new genus, provisionally called Whispovirus. From previously constructed WSSV genomic libraries of a Taiwan WSSV isolate, clones with open reading frames (ORFs) that

  5. Transcriptional analysis of the ribonucleotide reductase genes in shrimp white spot syndrome virus

    NARCIS (Netherlands)

    Tsai, M.F.; Lo, C.F.; Hulten, van M.C.W.; Tzeng, H.F.; Chou, C.M.; Huang, C.J.; Wang, C.S.

    2000-01-01

    The causative agent of white spot syndrome (WSS) is a large double-stranded DNA virus, WSSV, which is probably a representative of a new genus, provisionally called Whispovirus. From previously constructed WSSV genomic libraries of a Taiwan WSSV isolate, clones with open reading frames (ORFs) that e

  6. Polarisation of Major Histocompatibility Complex II Host Genotype with Pathogenesis of European Brown Hare Syndrome Virus

    DEFF Research Database (Denmark)

    Iacovakis, Christos; Mamuris, Zissis; Moutou, Katerina A;

    2013-01-01

    A study was conducted in order to determine the occurrence of European Brown Hare Syndrome virus (EBHSV) in Denmark and possible relation between disease pathogenesis and Major Histocompatibility Complex (MHC) host genotype. Liver samples were examined from 170 brown hares (hunted, found sick or ...

  7. Early Growth and Neurologic Outcomes of Infants with Probable Congenital Zika Virus Syndrome

    Science.gov (United States)

    Ganz, Jucelia Sousa Santos; Sousa, Patricia da Silva; Doriqui, Maria Juliana Rodvalho; Ribeiro, Marizelia Rodrigues Costa; Branco, Maria dos Remédios Freitas Carvalho; Queiroz, Rejane Christine de Sousa; Pacheco, Maria de Jesus Torres; Vieira da Costa, Flavia Regina; Silva, Francelena de Sousa; Simões, Vanda Maria Ferreira; Pacheco, Marcos Antonio Barbosa; Lamy-Filho, Fernando; Lamy, Zeni Carvalho; Soares de Britto e Alves, Maria Teresa Seabra

    2016-01-01

    We report the early growth and neurologic findings of 48 infants in Brazil diagnosed with probable congenital Zika virus syndrome and followed to age 1–8 months. Most of these infants had microcephaly (86.7%) and craniofacial disproportion (95.8%). The clinical pattern included poor head growth with increasingly negative z-scores, pyramidal/extrapyramidal symptoms, and epilepsy. PMID:27767931

  8. SLC30A9 mutation affecting intracellular zinc homeostasis causes a novel cerebro-renal syndrome.

    Science.gov (United States)

    Perez, Yonatan; Shorer, Zamir; Liani-Leibson, Keren; Chabosseau, Pauline; Kadir, Rotem; Volodarsky, Michael; Halperin, Daniel; Barber-Zucker, Shiran; Shalev, Hanna; Schreiber, Ruth; Gradstein, Libe; Gurevich, Evgenia; Zarivach, Raz; Rutter, Guy A; Landau, Daniel; Birk, Ohad S

    2017-02-09

    A novel autosomal recessive cerebro-renal syndrome was identified in consanguineous Bedouin kindred: neurological deterioration was evident as of early age, progressing into severe intellectual disability, profound ataxia, camptocormia and oculomotor apraxia. Brain MRI was normal. Four of the six affected individuals also had early-onset nephropathy with features of tubulo-interstitial nephritis, hypertension and tendency for hyperkalemia, though none had rapid deterioration of renal function. Genome wide linkage analysis identified an ∼18 Mb disease-associated locus on chromosome 4 (maximal logarithm of odds score 4.4 at D4S2971; θ = 0). Whole exome sequencing identified a single mutation in SLC30A9 within this locus, segregating as expected within the kindred and not found in a homozygous state in 300 Bedouin controls. We showed that SLC30A9 (solute carrier family 30 member 9; also known as ZnT-9) is ubiquitously expressed with high levels in cerebellum, skeletal muscle, thymus and kidney. Confocal analysis of SH-SY5Y cells overexpressing SLC30A9 fused to enhanced green fluorescent protein demonstrated vesicular cytosolic localization associated with the endoplasmic reticulum, not co-localizing with endosomal or Golgi markers. SLC30A9 encodes a putative zinc transporter (by similarity) previously associated with Wnt signalling. However, using dual-luciferase reporter assay in SH-SY5Y cells we showed that Wnt signalling was not affected by the mutation. Based on protein modelling, the identified mutation is expected to affect SLC30A9's highly conserved cation efflux domain, putatively disrupting its transmembrane helix structure. Cytosolic Zn2+ measurements in HEK293 cells overexpressing wild-type and mutant SLC30A9 showed lower zinc concentration within mutant rather than wild-type SLC30A9 cells. This suggests that SLC30A9 has zinc transport properties affecting intracellular zinc homeostasis, and that the molecular mechanism of the disease is through

  9. A complicated case of antepartum eclamptic fit with HELLP syndrome, acute renal failure and multiple intracranial hemorrhages: A mortality report

    Directory of Open Access Journals (Sweden)

    Ahmed Samy El-agwany

    2016-04-01

    Full Text Available HELLP is an acronym for hemolysis, elevated liver enzymes and low platelets count, affecting 0.2–12% of all pregnancies or 4–12% of those with preeclampsia. The maternal mortality reported from the literature is up 4% due to disseminated intravascular coagulation, placental abruption, acute renal failure, eclampsia, and cerebral hemorrhage. A 20 year old, G2P1, at 36 weeks of gestation, was referred to our hospital because of postictal coma state with bilateral mydriasis and epistaxis due to repeated antepartum eclamptic fits. Elevated blood pressure level 170/110 mmHg was accompanied with massive proteinuria. Cesarean section was performed and female newborn were delivered. Laboratory findings were characteristic of preeclampsia, HELLP syndrome and renal failure. The patient developed an intraventricular hematoma and an intracerebral hemorrhage with subarachnoid one, which were not suitable to neurosurgical treatment. The patient died from refractory hemolytic anemia, spontaneous bleeding of multiple organs, renal failure and intracranial hemorrhage. Preeclampsia, HELLP syndrome, and acute fatty liver of pregnancy might overlap and be associated with potentially fatal complications, including intracranial hemorrhage, as in the present case. Early detection and diagnosis are crucial to ensure appropriate management and treatment success.

  10. Evidence of Uncoupling between Renal Dysfunction and Injury in Cardiorenal Syndrome: Insights from the BIONICS Study

    Science.gov (United States)

    Legrand, Matthieu; De Berardinis, Benedetta; Gaggin, Hanna K.; Magrini, Laura; Belcher, Arianna; Zancla, Benedetta; Femia, Alexandra; Simon, Mandy; Motiwala, Shweta; Sambhare, Rasika; Di Somma, Salvatore; Mebazaa, Alexandre; Vaidya, Vishal S.; Januzzi, James L.; (GREAT), from the Global Research on Acute Conditions Team

    2014-01-01

    Objective The objective of the study was to assess urinary biomarkers of renal injury for their individual or collective ability to predict Worsening renal function (WRF) in patients with acutely decompensated heart failure (ADHF). Methods In a prospective, blinded international study, 87 emergency department (ED) patients with ADHF were evaluated with biomarkers of cardiac stretch (B type natriuretic peptide [BNP] and its amino terminal equivalent [NT-proBNP], ST2), biomarkers of renal function (creatinine, estimated glomerular filtration rate [eGFR]) and biomarkers of renal injury (plasma neutrophil gelatinase associated lipocalin [pNGAL], urine kidney injury molecule-1 [KIM-1], urine N-acetyl-beta-D-glucosaminidase [NAG], urine Cystatin C, urine fibrinogen). The primary endpoint was WRF. Results 26% developed WRF; baseline characteristics of subjects who developed WRF were generally comparable to those who did not. Biomarkers of renal function and urine biomarkers of renal injury were not correlated, while urine biomarkers of renal injury correlated between each other. Biomarker concentrations were similar between patients with and without WRF except for baseline BNP. Although plasma NGAL was associated with the combined endpoint, none of the biomarker showed predictive accuracy for WRF. Conclusions In ED patients with ADHF, urine biomarkers of renal injury did not predict WRF. Our data suggest that a weak association exists between renal dysfunction and renal injury in this setting (Clinicaltrials.gov NCT#0150153). PMID:25386851

  11. Evidence of uncoupling between renal dysfunction and injury in cardiorenal syndrome: insights from the BIONICS study.

    Directory of Open Access Journals (Sweden)

    Matthieu Legrand

    Full Text Available The objective of the study was to assess urinary biomarkers of renal injury for their individual or collective ability to predict Worsening renal function (WRF in patients with acutely decompensated heart failure (ADHF.In a prospective, blinded international study, 87 emergency department (ED patients with ADHF were evaluated with biomarkers of cardiac stretch (B type natriuretic peptide [BNP] and its amino terminal equivalent [NT-proBNP], ST2, biomarkers of renal function (creatinine, estimated glomerular filtration rate [eGFR] and biomarkers of renal injury (plasma neutrophil gelatinase associated lipocalin [pNGAL], urine kidney injury molecule-1 [KIM-1], urine N-acetyl-beta-D-glucosaminidase [NAG], urine Cystatin C, urine fibrinogen. The primary endpoint was WRF.26% developed WRF; baseline characteristics of subjects who developed WRF were generally comparable to those who did not. Biomarkers of renal function and urine biomarkers of renal injury were not correlated, while urine biomarkers of renal injury correlated between each other. Biomarker concentrations were similar between patients with and without WRF except for baseline BNP. Although plasma NGAL was associated with the combined endpoint, none of the biomarker showed predictive accuracy for WRF.In ED patients with ADHF, urine biomarkers of renal injury did not predict WRF. Our data suggest that a weak association exists between renal dysfunction and renal injury in this setting (Clinicaltrials.gov NCT#0150153.

  12. Molecular Epidemiological Investigation of Infectious Hypodermal and Hematopoietic Necrosis Virus and Taura Syndrome Virus in Penaeus Vannamei Cultured in China

    Institute of Scientific and Technical Information of China (English)

    2007-01-01

    The Infectious hypodermal and hematopoietic necrosis virus (IHHNV) and Taura syndrome virus (TSV) are two important shrimp viruses in cultured shrimp in America. These two viruses were transmitted to China at the beginning of the 21st century. In this study, 214 shrimp samples of Penaeus vannamei were collected from seven different areas of China and tested by PCR for IHHNV and TSV infection. The results showed that there were a high prevalence of IHHNV (65.42%) and low prevalence of TSV (3.27%) in the tested samples. Several samples were found to be co-infected with these two viruses. A 3 kb fragment of 7 positive IHHNV samples and a structure protein region (ORF2) of three TSV positive samples were amplified and sequenced. The sequence comparison indicated that both IHHNV and TSV sequenced in China have a low genetic variations compared with the prototype IHHNV and TSV from Hawaii. Phylogenetic analysis showed that TSV isolates were clustered into two groups, Asia and America group, which was genetically correlated to geographic distribution.

  13. Heterologous challenge with porcine reproductive and respiratory syndrome (PRRS) vaccine virus: no evidence of reactivation of previous European-type PRRS virus infection

    DEFF Research Database (Denmark)

    Bøtner, Anette; Nielsen, Jens; Oleksiewicz, M.B.

    1999-01-01

    In Denmark, a porcine reproductive and respiratory syndrome virus (PRRSV) control programme, comprising vaccination of seropositive herds with a live American type PRRSV vaccine, was started in 1996. In several of these herds, spread of vaccine virus from vaccinated 3-18 week old pigs to non-vacc...

  14. Metabolic syndrome in hemodialysis patients as a risk factor for new-onset diabetes mellitus after renal transplant: a prospective observational study

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    Bonet J

    2013-09-01

    Full Text Available Josep Bonet1, Albert Martinez-Castelao2, Beatriz Bayés11Department of Nephrology, Hospital Universitari Germans Trias i Pujol, Badalona, Spain; 2Department of Nephrology, Hospital Universitari de Bellvitge, IDIBELL, Hospitalet de Llobregat, Barcelona, SpainPurpose: Metabolic syndrome is a cluster of biochemical abnormalities including cardiovascular and diabetes risk factors. The development of diabetes mellitus after renal transplant represents a major posttransplant complication that may adversely affect graft/patient survival. The aim of this study was to assess the role of metabolic syndrome in patients on hemodialysis as a risk factor for the incidence of new-onset diabetes mellitus after renal transplant.Patients and methods: This was a prospective observational epidemiologic study carried out in adult nondiabetic patients undergoing chronic hemodialysis and on the renal transplant waiting list between November 2008 and April 2009. Patients were followed up from Visit 1 (baseline to 6 months after the renal transplant. The analysis of the role of metabolic syndrome in hemodialysis patients as a risk factor for the incidence of new-onset diabetes mellitus after renal transplant included the estimation of relative risk and its 95% confidence interval (CI.Results: A total of 383 evaluable patients were entered into the study (mean age, 52.7 years; male, 57.7%; Caucasian, 90.1%. The prevalence of metabolic syndrome on hemodialysis was 30.4% (95% CI, 25.8%–35.4%. Hypertension was the most prevalent criterion for metabolic syndrome (65.0%, followed by low levels of high-density lipoprotein cholesterol (52.7%, abdominal obesity (36.2%, hypertriglyceridemia (32.4%, and impaired glucose (8.9%. After the renal transplant, the prevalence of metabolic syndrome was still 25.8%. During the posttransplant period, the incidence of new-onset diabetes mellitus reached 13.0% (95% CI, 7.8%–20.6% and patients with pretransplant metabolic syndrome were 2

  15. The association between renal stone disease and cholesterol gallstones: the easy to believe and not hard to retrieve theory of the metabolic syndrome.

    Science.gov (United States)

    Ahmed, Mohamed H; Barakat, Salma; Almobarak, Ahmed O

    2014-07-01

    Renal stone disease and gallstone disease are widely prevalent and costly disease across the globe. Both renal stone disease and gallstone disease are associated with a variety of diseases including obesity, metabolic syndrome, dyslipidemia, hypertension, insulin resistance diabetes and gout. Importantly, the presence of either renal stone disease or gallstone disease is associated with an increased risk of cardiovascular disease. In a recent study of the Atherosclerosis Risk in Communities (ARIC), individuals with a history of gallstones were 54% more likely to report a history of nephrolithiasis after adjusting for age, gender, body size and other factors. Furthermore, in three large cohorts including over 240,000 subjects: the Nurses' Health Studies (NHS) I and II and the Health Professionals Follow-up Study (HPFS), showed that gallstone disease is independently associated with nephrolithiasis. The mechanisms linking gallstone disease and renal stone disease are complex and not yet established. Insulin resistance, lithogenic diets, alterations of transporters in gallbladder and urinary system, and pH are possible potential mechanisms for future exploration. How the liver communicates with kidney in individuals with renal stone disease and gallstone disease is not well known and whether this communication is similar as in hepto-renal syndrome is subject for future research. Further research is needed to determine: (i) the underlying mechanisms of renal stone disease and gallstone disease; (ii) the potential treatment of renal stone disease and gallstone disease.

  16. Hemorrhagic fever with renal syndrome:pathogenesis and clinical picture

    OpenAIRE

    2016-01-01

    Hantaan virus (HTNV) causes hemorrhagic fever with renal syndrome (HFRS), which is a zoonosis endemic in eastern Asia, especially in China. The reservoir host of HTNV is field mouse (Apodemus agraricus). The main manifestation of HFRS, including acute kidney injury, increases vascular permeability and coagulation abnormalities. In this paper, we review the current knowledge of the pathogenesis of HFRS including virus factor, immunity factor and host genetic factors. Furthermore, the treatmen...

  17. Adeno-Associated Virus-Mediated Gene Transfer to Renal Tubule Cells via a Retrograde Ureteral Approach

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    Daniel C. Chung

    2011-11-01

    Full Text Available Background/Aims: Gene therapy involves delivery of exogenous DNA to provide a therapeutic protein. Ideally, a gene therapy vector should be non-toxic, non-immunogenic, easy to produce, and efficient in protecting and delivering DNA into target cells. Methods: Adeno-associated virus (AAV offers these advantages and few, if any, disadvantages, and over 100 isolates exist. We previously showed that AAV-mediated gene therapy can be used to restore vision to patients with Leber’s congenital amaurosis, a disease of childhood blindness. Results: Here we show that novel recombinant AAV2/8 and AAV2/9 transduce kidney tubule cells with high efficiency both in vitroin cell culture and in vivoin mice. In addition, we adapted and modified a retrograde approach to allow for optimal transgene delivery to renal tubular cells that further minimizes the risk of an immunogenic reaction. Conclusions: We believe that recombinant AAV2, especially AAV2/8, gene delivery to renal tubule cells via a retrograde approach represents a viable method for gene therapy for a multitude of renal disorders ranging from autosomal dominant polycystic kidney disease to acute kidney injury.

  18. Epidemiologic characteristics of haemorrhagic fever with renal syndrome in Mainland China from 2006 to 2010

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    Shiwen Wang

    2012-02-01

    Full Text Available Objective: To design effective prevention and control strategies for haemorrhagic fever with renal syndrome (HFRS in Mainland China, we evaluated the epidemiologic characteristics and trends of HFRS cases reported between 2006 and 2010.Methods: HFRS data from 1970 to 2010 were obtained from the China Notifiable Disease Reporting System (CNDRS. The cases analysed included clinical cases and laboratory-confirmed cases. Data was extracted for statistical analysis by time, region and profession; the incidence rate was obtained directly from CNDRS. In this study, we analysed the morbidity and mortality data of HFRS from 2006 to 2010.Results: HFRS cases trended downward from 2006 (15 098 to 2009 (8745, but exhibited a slight increase in 2010 (9526. Twenty-nine of 31 provinces reported HFRS cases between 2006 and 2010. Five provinces, namely, Heilongjiang, Jilin and Liaoning in the North-east, Shandong in the east, and Shaanxi in the central part of China, were characterized as high-endemic areas. Seasonal case distribution was bimodal, with peaks of cases in spring and winter. Young male farmers were the most susceptible population to HFRS. Early- to middle-aged adults (20–50 years old represented the largest groups of HFRS cases.Conclusion: The overall number of cases of HFRS in China has trended downward possibly due to national vaccine and rodent vector control programmes implemented in the past 25 years. However, this trend slowed down in the last five years. High-endemic regions and at-risk population groups still exist and will benefit from further targeted prevention strategies.

  19. Spatiotemporal transmission dynamics of hemorrhagic fever with renal syndrome in China, 2005-2012.

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    Wen-Yi Zhang

    2014-11-01

    Full Text Available Hemorrhagic fever with renal syndrome (HFRS is a rodent-borne disease caused by many serotypes of hantaviruses. In China, HFRS has been recognized as a severe public health problem with 90% of the total reported cases in the world. This study describes the spatiotemporal dynamics of HFRS cases in China and identifies the regions, time, and populations at highest risk, which could help the planning and implementation of key preventative measures.Data on all reported HFRS cases at the county level from January 2005 to December 2012 were collected from Chinese Center for Disease Control and Prevention. Geographic Information System-based spatiotemporal analyses including Local Indicators of Spatial Association and Kulldorff's space-time scan statistic were performed to detect local high-risk space-time clusters of HFRS in China. In addition, cases from high-risk and low-risk counties were compared to identify significant demographic differences.A total of 100,868 cases were reported during 2005-2012 in mainland China. There were significant variations in the spatiotemporal dynamics of HFRS. HFRS cases occurred most frequently in June, November, and December. There was a significant positive spatial autocorrelation of HFRS incidence during the study periods, with Moran's I values ranging from 0.46 to 0.56 (P<0.05. Several distinct HFRS cluster areas were identified, mainly concentrated in northeastern, central, and eastern of China. Compared with cases from low-risk areas, a higher proportion of cases were younger, non-farmer, and floating residents in high-risk counties.This study identified significant space-time clusters of HFRS in China during 2005-2012 indicating that preventative strategies for HFRS should be particularly focused on the northeastern, central, and eastern of China to achieve the most cost-effective outcomes.

  20. The epidemic characteristics and changing trend of hemorrhagic fever with renal syndrome in Hubei Province, China.

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    Yi-Hui Zhang

    Full Text Available BACKGROUND: Hemorrhagic fever with renal syndrome (HFRS is caused by different hantaviruses within the Bunyaviridae family. HFRS is a fulminant, infectious disease that occurs worldwide and is endemic in all 31 provinces of China. Since the first HFRS case in Hubei Province was reported in 1957, the disease has spread across the province and Hubei has become one of the seriously affected areas in China with the greatest number of reported HFRS cases in the 1980's. However, the epidemic characteristics of HFRS in Hubei are still not entirely clear and long-term, systematic investigations of this epidemic area have been very limited. METHODS: The spatiotemporal distribution of HFRS was investigated using data spanning the years 1980 to 2009. The annual HFRS incidence, fatality rate and seasonal incidence between 1980 and 2009 were calculated and plotted. GIS-based spatial analyses were conducted to detect the spatial distribution and seasonal pattern of HFRS. A spatial statistical analysis, using Kulldorff's spatial scan statistic, was performed to identify clustering of HFRS. RESULTS: A total of 104,467 HFRS cases were reported in Hubei Province between 1980 and 2009. Incidence of and mortality due to HFRS declined after the outbreak in 1980s and HFRS cases have been sporadic in recent years. The locations and scale of disease clusters have changed during the three decades. The seasonal epidemic pattern of HFRS was characterized by the shift from the unimodal type (autumn/winter peak to the bimodal type. CONCLUSIONS: Socioeconomic development has great influence on the transmission of hantaviruses to humans and new epidemic characteristics have emerged in Hubei Province. It is necessary to reinforce preventative measures against HFRS according to the newly-presented seasonal variation and to intensify these efforts especially in the urban areas of Hubei Province.

  1. Therapeutic effects of continuous renal replacement in patients with multiple organ dysfunction syndrome in ICU

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    Shuang-qing LIU

    2013-05-01

    Full Text Available Objective  To investigate the effects of continuous renal replacement therapy (CRRT on patients with multiple organ dysfunction syndrome (MODS. Methods  Forty-seven patients with MODS, admitted to the Department of SICU of First Affiliated Hospital of General Hospital of PLA, were divided into two groups according to the causative agents: the sepsis group (n=28 and the non-sepsis group (n=19. All the patients involved were treated with CRRT therapy. The systemic and brain hemodynamic parameters, and oxygenation and metabolic variables were assessed immediately before and 12 hours after CRRT. The expected mortality was obtained by APACHE Ⅱ score, and it was compared with the actual mortality to obtain the standard mortality, of which less than 0.9 was defined as an improved survival rate. Results  After 12 hours of CRRT treatment, all the variables (except mean arterial pressure and brain hemodynamics were significantly improved in the sepsis group (P<0.05. The survival rate in ICU was 60.7% with a standard mortality of 0.70. In the non-sepsis group, the survival rate in ICU was only 15.8% and the standard mortality was 1.77. Only the FiO2 was found to be significantly improved in the non-sepsis group. Conclusion  CRRT may significantly improve the clinical outcome and survival rate of patients with sepsis-caused MODS, but no obvious effect is found among the patients with MODS caused by non-sepsis factors.

  2. Renal angiomyolipoma in Birt-Hogg-Dube syndrome: A case study supporting overlap with tuberous sclerosis complex.

    Science.gov (United States)

    Dow, Eryn; Winship, Ingrid

    2016-12-01

    Birt-Hogg-Dube syndrome (BHD) is an autosomal dominant disease characterised by benign cutaneous lesions, pulmonary cysts, and an increased risk of renal tumors. This rare condition is due to a mutation in the folliculin (FLCN) gene on chromosome 17q11.2, which has a role in the mechanistic/mammalian target of rapamycin (mTOR) signaling pathway of tumorigenesis. This case illustrates a patient with BHD and a renal angiomyolipoma, a neoplastic lesion not usually associated with BHD but common in Tuberous Sclerosis Complex (TSC). There is both clinical and molecular overlap between BHD and TSC, which may arise from similarities in function of the TSC and FLCN proteins in the mTOR pathway; this case further demonstrates this potential correlation. © 2016 Wiley Periodicals, Inc.

  3. Vasculotoxic snake bite presenting with sepsis, acute renal failure, disseminated intravascular coagulation, and acute respiratory distress syndrome

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    Vikram Bhausaheb Vikhe

    2013-01-01

    Full Text Available Vasculotoxic snake bites are well known to cause local complications like necrosis and cellulitis and systemic complications such as coagulopathy, acute renal failure (ARF, and hemolysis. We report a case of young female patient who was bitten by a viper. She developed cellulitis, sepsis, ARF, and Disseminated Intravascular Coagulation. She was treated for the above complications and all her renal and hematological parameters returned to normal on seventh day. After this, on the same day, patient developed Acute Respiratory Distress Syndrome probably due to the direct toxic effect of venom on pulmonary vascular endothelium which has been reported as a late complication of snake venom. With close monitoring and proper management of complications, the patient recovered and walked out of the hospital on the twenty first day without any complications.

  4. Creatinine clearance, urinary excretion of glomerular basement membrane antigens and renal histology in congenital nephrotic syndrome of Finnish type.

    Science.gov (United States)

    Huttunen, N P

    1977-04-01

    The endogenous creatinine clearance and urinary excretion rate of glomerular basement membrane (GBM) antigens were followed from 2 to 19 months in fifteen patients with congenital nephrotic syndrome (CNF). The quantitative examination of renal morphology was made on fourteen of these patients. Creatinine clearance increased during the first few months of life and thereafter gradually decreased. The urinary excretion rate of GBM antigens rose during the course of the disease. The creatinine clearance did not correlate significantly with glomerular fibrosis but it did correlate with tubular atrophy and interstitial fibrosis. The urinary excretion of GBM antigens correlated significantly with glomerular and interstitial fibrosis and with tubular atrophy. It is concluded that there is a clear progress in the disease and the renal histological changes probably are caused by accumulation of GBM material in glomeruli.

  5. The Relation between Fructose-Induced Metabolic Syndrome and Altered Renal Haemodynamic and Excretory Function in the Rat

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    Mohammed H. Abdulla

    2011-01-01

    Full Text Available This paper explores the possible relationships between dietary fructose and altered neurohumoral regulation of renal haemodynamic and excretory function in this model of metabolic syndrome. Fructose consumption induces hyperinsulinemia, hypertriglyceridaemia, insulin resistance, and hypertension. The pathogenesis of fructose-induced hypertension is dubious and involves numerous pathways acting both singly and together. In addition, hyperinsulinemia and hypertension contribute significantly to progressive renal disease in fructose-fed rats. Moreover, increased activity of the renin-angiotensin and sympathetic nervous systems leading to downregulation of receptors may be responsible for the blunted vascular sensitivity to angiotensin II and catecholamines, respectively. Various approaches have been suggested to prevent the development of fructose-induced hypertension and/or metabolic alteration. In this paper, we address the role played by the renin-angiotensin and sympathetic nervous systems in the haemodynamic alterations that occur due to prolonged consumption of fructose.

  6. Fatal group A streptococcal necrotizing fasciitis and toxic shock syndrome in a patient with psoriasis and chronic renal impairment.

    Science.gov (United States)

    Chong, Alvin H; Burrows, Nigel P

    2002-08-01

    A 78-year-old woman presented with rapid onset of skin pain which evolved into oedema, discoloration and infarction. She was diagnosed with group A beta-haemolytic streptococcus (Streptococcus pyogenes) necrotizing fasciitis and streptococcal toxic shock syndrome. The patient had a past history of psoriasis and end-stage renal impairment. Despite treatment with multiple antibiotics in an intensive care unit, the skin infarction involving the upper trunk continued to expand and the patient died within 24 hours of hospital admission. Group A streptococcus and Staphylococcus aureus were cultured from a tissue biopsy. Renal failure and compromised skin barrier function are known to predispose to invasive streptococcal infections, but necrotizing fasciitis has only rarely been reported in association with psoriasis. This case illustrates the fulminant nature of the infection.

  7. Effect of renal shock wave lithotripsy on the development of metabolic syndrome in a juvenile swine model: a pilot study.

    Science.gov (United States)

    Handa, Rajash K; Liu, Ziyue; Connors, Bret A; Alloosh, Mouhamad; Basile, David P; Tune, Johnathan D; Sturek, Michael; Evan, Andrew P; Lingeman, James E

    2015-04-01

    We performed a pilot study to assess whether renal shock wave lithotripsy influences metabolic syndrome onset and severity. Three-month-old juvenile female Ossabaw miniature pigs were treated with shock wave lithotripsy (2,000 shock waves at 24 kV with 120 shock waves per minute in 2) or sham shock wave lithotripsy (no shock waves in 2). Shock waves were targeted to the upper pole of the left kidney to model treatment that would also expose the pancreatic tail to shock waves. Pigs were then instrumented to directly measure arterial blood pressure via an implanted radiotelemetry device. They later received a hypercaloric atherogenic diet for about 7 months. Metabolic syndrome development was assessed by the intravenous glucose tolerance test. Metabolic syndrome progression and severity were similar in the sham treated and lithotripsy groups. The only exception arterial blood pressure, which remained relatively constant in sham treated pigs but began to increase at about 2 months towards hypertensive levels in lithotripsy treated pigs. Metabolic data on the 2 groups were pooled to provide a more complete assessment of metabolic syndrome development and progression in this juvenile pig model. The intravenous glucose tolerance test revealed substantial insulin resistance with impaired glucose tolerance within 2 months on the hypercaloric atherogenic diet with signs of further metabolic impairment at 7 months. These preliminary results suggest that renal shock wave lithotripsy is not a risk factor for worsening glucose tolerance or diabetes mellitus onset. However, it appears to be a risk factor for early onset hypertension in metabolic syndrome. Copyright © 2015 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.

  8. Aerobic Exercise Improves Signs of Restless Leg Syndrome in End Stage Renal Disease Patients Suffering Chronic Hemodialysis

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    Mojgan Mortazavi

    2013-01-01

    Full Text Available Background. Restless leg syndrome (RLS is one of the prevalent complaints of patients with end stage renal diseases suffering chronic hemodialysis. Although there are some known pharmacological managements for this syndrome, the adverse effect of drugs causes a limitation for using them. In this randomized clinical trial we aimed to find a nonpharmacological way to improve signs of restless leg syndrome and patients’ quality of life. Material and Methods. Twenty-six patients were included in the study and divided into 2 groups of control and exercise. The exercise group used aerobic exercise during their hemodialysis for 16 weeks. The quality of life and severity of restless leg syndrome were assessed at the first week of study and final week. Data were analyzed using SPSS software. Results. The difference of means of RLS signs at the first week of study and final week was in exercise group and in control group. There was not any statistical difference between control group and exercise group in quality of life at the first week of study and final week. Conclusions. We suggest using aerobic exercise for improving signs of restless leg syndrome, but no evidence was found for its efficacy on patient’s quality of life.

  9. Assessment of the role of brine shrimp Artemia in white spot syndrome virus (WSSV) transmission.

    Science.gov (United States)

    Zhang, Jia-Song; Dong, Shuang-Lin; Dong, Yun-Wei; Tian, Xiang-Li; Cao, Yi-Cheng; Li, Zuo-Jia; Yan, Dong-Chun

    2010-01-01

    Challenge tests with Artemia four different development stages (nauplii, metanauplii, pseudoadults and adults) to white spot syndrome virus was carried out by immersion challenge and virus-phytoplankton adhesion route in order to asses the possibility of Artemia acting as a vector of WSSV to penaeid shrimp Litopenaeus vannamei postlarvae. The WSSV succeeded in infecting four stages Artemia, and nested-PCR detection for WSSV revealed positive results to virus-phytoplankton adhesion route. No mass mortalities were observed in penaeid shrimp postlarvae fed with WSSV-positive Artemia which exposed to WSSV by virus-phytoplankton adhesion route, whereas WSSV DNA detected in penaeid shrimp postlarvae by nested-PCR. By contrary, no WSSV-positive was detected in any animal fed with WSSV-negative Artemia. These results indicated that Artemia could serve as a vector in WSSV transmission.

  10. Persistence of secondary restless legs syndrome in a phantom limb caused by end-stage renal disease.

    Science.gov (United States)

    Nishida, Shingo; Hitsumoto, Akiko; Namba, Kazuyoshi; Usui, Akira; Inoue, Yuichi

    2013-01-01

    Our patient had secondary restless legs syndrome (RLS) in the left lower limb caused by end-stage renal disease (ESRD). Severe RLS symptoms persisted even after amputation of the affected limb. Considering that oral administration of a dopamine receptor agonist was effective in treating the RLS in the phantom limb in this case, dysfunction of the central dopaminergic system was thought to be involved in the phantom limb-RLS mechanism. The persistence of RLS symptoms even after amputation of the affected limb suggests that the area responsible for ESRD-related RLS symptoms exists at the spinal level or in the higher central nervous system.

  11. Managing Renal Cell Carcinoma Associated Paraneoplastic Syndrome with Nephron-sparing Surgery in a Patient with von Hippel-Lindau

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    John M. DiBianco

    2017-07-01

    Full Text Available A patient with germline von Hippel-Lindau (VHL gene alteration and history of multiple tumors present with classical paraneoplastic syndrome (PNS associated with renal cell carcinoma (RCC. She underwent open nephron sparing surgery with resolution of symptoms. She remained without recurrence of RCC for the initial 2 years of her follow-up. To the best of our knowledge, this case represents the first in which PNS was specifically resolved using a partial nephrectomy in a patient with VHL. This case report provides initial evidence for the potential role of nephron sparing surgery in the management of paraneoplastic symptoms associated with hereditary RCC.

  12. Changes in rodent abundance and weather conditions potentially drive hemorrhagic fever with renal syndrome outbreaks in Xi'an, China, 2005-2012.

    Directory of Open Access Journals (Sweden)

    Huai-Yu Tian

    2015-03-01

    Full Text Available Increased risks for hemorrhagic fever with renal syndrome (HFRS caused by Hantaan virus have been observed since 2005, in Xi'an, China. Despite increased vigilance and preparedness, HFRS outbreaks in 2010, 2011, and 2012 were larger than ever, with a total of 3,938 confirmed HFRS cases and 88 deaths in 2010 and 2011.Data on HFRS cases and weather were collected monthly from 2005 to 2012, along with active rodent monitoring. Wavelet analyses were performed to assess the temporal relationship between HFRS incidence, rodent density and climatic factors over the study period. Results showed that HFRS cases correlated to rodent density, rainfall, and temperature with 2, 3 and 4-month lags, respectively. Using a Bayesian time-series Poisson adjusted model, we fitted the HFRS outbreaks among humans for risk assessment in Xi'an. The best models included seasonality, autocorrelation, rodent density 2 months previously, and rainfall 2 to 3 months previously. Our models well reflected the epidemic characteristics by one step ahead prediction, out-of-sample.In addition to a strong seasonal pattern, HFRS incidence was correlated with rodent density and rainfall, indicating that they potentially drive the HFRS outbreaks. Future work should aim to determine the mechanism underlying the seasonal pattern and autocorrelation. However, this model can be useful in risk management to provide early warning of potential outbreaks of this disease.

  13. Severe acute respiratory syndrome coronavirus envelope protein ion channel activity promotes virus fitness and pathogenesis.

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    Jose L Nieto-Torres

    2014-05-01

    Full Text Available Deletion of Severe Acute Respiratory Syndrome Coronavirus (SARS-CoV envelope (E gene attenuates the virus. E gene encodes a small multifunctional protein that possesses ion channel (IC activity, an important function in virus-host interaction. To test the contribution of E protein IC activity in virus pathogenesis, two recombinant mouse-adapted SARS-CoVs, each containing one single amino acid mutation that suppressed ion conductivity, were engineered. After serial infections, mutant viruses, in general, incorporated compensatory mutations within E gene that rendered active ion channels. Furthermore, IC activity conferred better fitness in competition assays, suggesting that ion conductivity represents an advantage for the virus. Interestingly, mice infected with viruses displaying E protein IC activity, either with the wild-type E protein sequence or with the revertants that restored ion transport, rapidly lost weight and died. In contrast, mice infected with mutants lacking IC activity, which did not incorporate mutations within E gene during the experiment, recovered from disease and most survived. Knocking down E protein IC activity did not significantly affect virus growth in infected mice but decreased edema accumulation, the major determinant of acute respiratory distress syndrome (ARDS leading to death. Reduced edema correlated with lung epithelia integrity and proper localization of Na+/K+ ATPase, which participates in edema resolution. Levels of inflammasome-activated IL-1β were reduced in the lung airways of the animals infected with viruses lacking E protein IC activity, indicating that E protein IC function is required for inflammasome activation. Reduction of IL-1β was accompanied by diminished amounts of TNF and IL-6 in the absence of E protein ion conductivity. All these key cytokines promote the progression of lung damage and ARDS pathology. In conclusion, E protein IC activity represents a new determinant for SARS

  14. Arginine kinase of Litopenaeus vannamei involved in white spot syndrome virus infection.

    Science.gov (United States)

    Ma, Fang-fang; Liu, Qing-hui; Guan, Guang-kuo; Li, Chen; Huang, Jie

    2014-04-10

    Virus-host interaction is important for virus infection. White spot syndrome virus VP14 contains transmembrane and signal peptides domain, which is considered to be important for virus infection. Until now, the function of this protein remains undefined. In this study, we explored the interaction of VP14 with host cell. A new shrimp protein (arginine kinase of Litopenaeus vannamei, LvAK) is selected and its localization in shrimp cells is also confirmed. Cellular localization of LvAK protein in shrimp hemocytes showed that LvAK was primarily located at the periphery of hemocytes and was scarcely detectable in the nucleus. Tissue distribution indicated that arginine kinase gene was spread commonly in the tissues and was highly present in shrimp muscle tissue. The expression of LvAK mRNA in muscle was significantly up-regulated after WSSV stimulation. Indirect immunofluorescence assay showed that LvAK interacted with VP14 in WSSV-infected shrimp. Injection of LvAK protein enhanced the mortality of shrimp infected with white spot syndrome virus (WSSV). These results showed that LvAK is involved in WSSV infection. Future research on this topic will help to reveal the molecular mechanism of WSSV infection.

  15. Simultaneous pancreas and kidney transplantation with concurrent allograft nephrectomy for recipients with prior renal transplants lost to BK virus nephropathy: two case reports.

    Science.gov (United States)

    Kubal, S; Powelson, J A; Taber, T E; Goble, M L; Fridell, J A

    2010-01-01

    Candidacy for retransplantation after allograft loss due to BK virus-associated nephropathy (BKVN) with or without allograft nephrectomy is controversial. This report describes 2 renal transplant recipients who lost their grafts to BKVN and subsequently underwent simultaneous kidney and pancreas transplantation with allograft nephrectomy.

  16. Cysteine residues of the porcine reproductive and respiratory syndrome virus ORF5a protein are not essential for virus viability.

    Science.gov (United States)

    Sun, Lichang; Zhou, Yan; Liu, Runxia; Li, Yanhua; Gao, Fei; Wang, Xiaomin; Fan, Hongjie; Yuan, Shishan; Wei, Zuzhang; Tong, Guangzhi

    2015-02-02

    ORF5a protein was recently identified as a novel structural protein in porcine reproductive and respiratory syndrome virus (PRRSV). The ORF5a protein possesses two cysteines at positions 29 and 30 that are highly conserved among type 2 PRRSV. In this study, the significance of the ORF5a protein cysteine residues on virus replication was determined based on a type 2 PRRSV cDNA clone (pAJXM). Each cysteine was substituted by serine or glycine and the mutations were introduced into pAJXM. We found that the replacement of cysteine to glycine at position 30 was lethal for virus viability, but all serine mutant clones produced infectious progeny viruses. This data indicated that cysteine residues in the ORF5a protein were not essential for replication of type 2 PRRSV. The bimolecular fluorescence complementation (BiFC) and Co-immunoprecipitation (Co-IP) assay were used to study ORF5a protein interacted with other enveloped proteins. These results showed that ORF5a protein interacted non-covalently with itself and interacted with GP4 and 2b protein. The replacement of cysteine to glycine at position 30 affected the ORF5a protein interacted non-covalently with itself, which may account for the lethal phenotype of mutants carrying substitution of cysteine to glycine at position 30.

  17. Evaluation of the Genetic Variation of Non Coding Control Region of BK Virus Using Nested-PCR Sequencing Method in Renal Graft Patients

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    A Emami

    2015-05-01

    Full Text Available Background & aim: Polyomaviruses (BK is a comprehensive infection with more than of 80% prevalence in the world. One of the most important reasons of BK virus nephropathy is in the renal transplant recipients and rejection of transplanted tissue between them. Non Coding region of this virus play a regulatory role in replication and amplification of the virus. The aim of this study was to evaluate the genetic patterns of this area in renal graft at Namazi Transplantation Center, Shiraz, Iran. Methods: In the present experimental study, 380 renal allograft serums were collected. DNAs of 129 eligible samples were extracted and evaluated using a virus genome. The presence of the virus was determined by qualitative and sequencing. Of these, 129 samples were tested for the presence of virus according to the condition study, using quantitative, qualitative genomic amplification and sequencing. Results: The study showed symptoms of nephropathy, 76 (58.9% of them were males and 46 (35.7% were females with the mean age 38.0±.089 years of age. In general, 46 patients (35.7% percent were positive for BK Polyomaviruses. After comparing the genomic sequence with applications of molecular they were categorized in three groups and then recorded in gene bank. Conclusion: About 35% of renal transplant recipients with high creatinine levels were positive for the presence of BK virus. Non-coding region of respondents in the sample survey revealed that among patients with the most common genotypes were rearranged the entire transplant patients were observed at this tranplant center. Examination of these sequences indicated that this rearrangments had a specific pattern, different from the standard strain of archaea type.

  18. Zika Virus Infection and Guillain-Barré Syndrome in Three Patients from Suriname.

    Science.gov (United States)

    Langerak, Thomas; Yang, Harvey; Baptista, Mark; Doornekamp, Laura; Kerkman, Tessa; Codrington, John; Roosblad, Jimmy; Vreden, Stephen G S; De Bruin, Erwin; Mögling, Ramona; Jacobs, Bart C; Pas, Suzan D; GeurtsvanKessel, Corine H; Reusken, Chantal B E M; Koopmans, Marion P; Van Gorp, Eric C M; Alberga, Henk

    2016-01-01

    We present three patients from Suriname who were diagnosed with Guillain-Barré syndrome (GBS) during the Zika virus (ZIKV) outbreak in this country. One patient had a positive ZIKV urine real-time RT-PCR (qRT-PCR) result. The other two patients had a negative ZIKV urine qRT-PCR but a positive virus neutralization test and presence of IgG antibodies against ZIKV in the serum. Considering the evidence of a past ZIKV infection and absence of evidence for recent infections with the most common preceding infections of GBS, it is very likely that these GBS cases were triggered by ZIKV.

  19. Zika Virus Infection and Guillain–Barré Syndrome in Three Patients from Suriname

    Science.gov (United States)

    Langerak, Thomas; Yang, Harvey; Baptista, Mark; Doornekamp, Laura; Kerkman, Tessa; Codrington, John; Roosblad, Jimmy; Vreden, Stephen G. S.; De Bruin, Erwin; Mögling, Ramona; Jacobs, Bart C.; Pas, Suzan D.; GeurtsvanKessel, Corine H.; Reusken, Chantal B. E. M.; Koopmans, Marion P.; Van Gorp, Eric C. M.; Alberga, Henk

    2016-01-01

    We present three patients from Suriname who were diagnosed with Guillain–Barré syndrome (GBS) during the Zika virus (ZIKV) outbreak in this country. One patient had a positive ZIKV urine real-time RT-PCR (qRT-PCR) result. The other two patients had a negative ZIKV urine qRT-PCR but a positive virus neutralization test and presence of IgG antibodies against ZIKV in the serum. Considering the evidence of a past ZIKV infection and absence of evidence for recent infections with the most common preceding infections of GBS, it is very likely that these GBS cases were triggered by ZIKV. PMID:28066317

  20. Lewis-Sumner syndrome in hepatitis C virus infection: a possible pathogenetic association with therapeutic problems.

    Science.gov (United States)

    Caporale, Christina M; Capasso, Margherita; Ragno, Michele; Di Muzio, Antonio; Uncini, Antonino

    2006-07-01

    A patient with chronic hepatitis from hepatitis C virus (HCV) infection developed Lewis-Sumner syndrome (LSS). The neuropathy worsened after intravenous immunoglobulins, remitted after intravenous methylprednisolone, relapsed during interferon-alpha, but responded again to steroids continued for 68 weeks with clinical remission and without worsening of hepatitis. We are not aware of other reports of HCV infection and LSS. This association may be coincidental or related to a virus-triggered immune-mediated process. Although the coexistence of a dysimmune neuropathy with hepatitis makes problematic the choice of treatment, we emphasize that the patient's condition during treatment with steroids and the 46 following weeks without therapy has been excellent.

  1. Full Genome Sequence of Egg Drop Syndrome Virus Strain FJ12025 Isolated from Muscovy Duckling.

    Science.gov (United States)

    Fu, Guanghua; Chen, Hongmei; Huang, Yu; Cheng, Longfei; Fu, Qiuling; Shi, Shaohua; Wan, Chunhe; Chen, Cuiteng; Lin, Jiansheng

    2013-08-22

    Egg drop syndrome virus (EDSV) strain FJ12025 was isolated from a 9-day-old Muscovy duckling. The results of the sequence showed that the genome of strain FJ12025 is 33,213 bp in length, with a G+C content of 43.03%. When comparing the genome sequence of strain FJ12025 to that of laying duck original strain AV-127, we found 50 single-nucleotide polymorphisms (SNPs) between the two viral genome sequences. A genomic sequence comparison of FJ12025 and AV-127 will help to understand the phenotypic differences between the two viruses.

  2. Renoscintigraphy in assessment of renal lesions in children after hemolytic-uremic syndrome; Renoscyntygrafia w ocenie skutkow uszkodzenia nerek u dzieci po przebytym zespole hemolityczno-mocznicowym

    Energy Technology Data Exchange (ETDEWEB)

    Lass, P.; Marczak, E.; Romanowicz, G. [Akademia Medyczna, Gdansk (Poland); and others

    1994-12-31

    The aim of the study was to assess the role of renoscintigraphic examination in monitoring of patients after the hemolytic-uremic syndrome. 27 children mean 9 years the hemolytic-uremic syndrome underwent the complex of biochemical, ultrasound and renoscintigraphic examinations. The abnormal renoscintigraphic was seen in 85.1% of children, while the alternative test described the renal lesion in 29-66%. Renoscintigraphic examination seems to be the most sensitive in monitoring of remote sequel in patients after HUS. Those patients should undergone long-lasting observation, for the sake of possibility of development of renal insufficiency. (author). 14 refs.

  3. Guillain-Barré Syndrome Associated with Zika Virus Infection in Colombia.

    Science.gov (United States)

    Parra, Beatriz; Lizarazo, Jairo; Jiménez-Arango, Jorge A; Zea-Vera, Andrés F; González-Manrique, Guillermo; Vargas, José; Angarita, Jorge A; Zuñiga, Gonzalo; Lopez-Gonzalez, Reydmar; Beltran, Cindy L; Rizcala, Karen H; Morales, Maria T; Pacheco, Oscar; Ospina, Martha L; Kumar, Anupama; Cornblath, David R; Muñoz, Laura S; Osorio, Lyda; Barreras, Paula; Pardo, Carlos A

    2016-10-20

    Background Zika virus (ZIKV) infection has been linked to the Guillain-Barré syndrome. From November 2015 through March 2016, clusters of cases of the Guillain-Barré syndrome were observed during the outbreak of ZIKV infection in Colombia. We characterized the clinical features of cases of Guillain-Barré syndrome in the context of this ZIKV infection outbreak and investigated their relationship with ZIKV infection. Methods A total of 68 patients with the Guillain-Barré syndrome at six Colombian hospitals were evaluated clinically, and virologic studies were completed for 42 of the patients. We performed reverse-transcriptase-polymerase-chain-reaction (RT-PCR) assays for ZIKV in blood, cerebrospinal fluid, and urine, as well as antiflavivirus antibody assays. Results A total of 66 patients (97%) had symptoms compatible with ZIKV infection before the onset of the Guillain-Barré syndrome. The median period between the onset of symptoms of ZIKV infection and symptoms of the Guillain-Barré syndrome was 7 days (interquartile range, 3 to 10). Among the 68 patients with the Guillain-Barré syndrome, 50% were found to have bilateral facial paralysis on examination. Among 46 patients in whom nerve-conduction studies and electromyography were performed, the results in 36 patients (78%) were consistent with the acute inflammatory demyelinating polyneuropathy subtype of the Guillain-Barré syndrome. Among the 42 patients who had samples tested for ZIKV by RT-PCR, the results were positive in 17 patients (40%). Most of the positive RT-PCR results were in urine samples (in 16 of the 17 patients with positive RT-PCR results), although 3 samples of cerebrospinal fluid were also positive. In 18 of 42 patients (43%) with the Guillain-Barré syndrome who underwent laboratory testing, the presence of ZIKV infection was supported by clinical and immunologic findings. In 20 of these 42 patients (48%), the Guillain-Barré syndrome had a parainfectious onset. All patients tested were

  4. Bullous Variant of Sweet’s Syndrome after Herpes Zoster Virus Infection

    OpenAIRE

    2011-01-01

    Aim: Cutaneous manifestations of Sweet’s syndrome (SS) are typically painful plaque-forming erythematous papules, while bullae are quite uncommon. We present a case of bullous variant of SS in acute myeloid leukaemia. In this case, herpes infection of the left mandible had preceded the development of SS. Case Report: A 75-year-old male with myelodysplastic syndrome first presented with herpes zoster virus infection-like bullae and erosive plaques on the left side of the face and neck. Treatme...

  5. Clinical features and treatment of hepatitis B virus and hepatitis C virus co-infection among patients with acquired immune deficiency syndrome

    Institute of Scientific and Technical Information of China (English)

    杨蓉蓉

    2014-01-01

    Objective To estimate the clinical features of hepatitis B virus(HBV)and hepatitis C virus(HCV)co-infection among acquired immune deficiency syndrome(AIDS)patients and the interaction of lamivudine(3 TC)contained antiretroviral therapy(ART)with hepatitis virus replication.Methods From 2004 to 2010,199human immunodeficiency virus(HIV)/HBV coinfected patients admitted to Zhongnan Hospital of Wuhan University were enrolled,including 76 cases of HIV/HBV/HCV triple infection and 123 cases of

  6. A Retrospective Study of Continuous Renal Therapy and Anticoagulation in Patients with Hemorrhagic Fever with Renal Syndrome

    Institute of Scientific and Technical Information of China (English)

    2014-01-01

    Objective To observe the application of continuous renal replacement therapy(CRRT) and heparin anticoagulation in patients with HFRS, and to explore a more suitable anticoagulant strategy. MethodsEighty-five severe-type patients (severe group) and 71 critical-type patients (critical group) were enrolled in this study. The frequency of CRRT was compared between the two groups; the frequency of CRRT treated with and without heparin anticoagulation and the frequency of hemorrhage and channel blood clotting induced by the two anticoagulant strategies were observed. ResultsThe frequency of CRRT in the critical group was higher than thatin the severe group (P<0.001). The frequency of CRRT initiated during the overlapping phases in the critical group was signiifcantly higher than that of the severe group (P=0.032). The total times of CRRT was 103, and 70 of them were treated with heparin anticoagulation. The frequencies of hemorrhage induced by heparin anticoagulation and no heparinization were 16 and 0, respectively, and the frequencies of channel blood clotting were 2 and 4, respectively. Conclusions CRRT has been used extensively in the critical-type patients with HFRS. The heparin anticoagulation and no anticoagulant strategies should be used more rationally in patients treated with CRRT, according to the clinical characteristics of the disease.

  7. Vesicourethral reflux-induced renal failure in a patient with ICF syndrome due to a novel DNMT3B mutation.

    Science.gov (United States)

    Kutluğ, Seyhan; Ogur, Gönül; Yilmaz, Aysegül; Thijssen, Peter E; Abur, Ummet; Yildiran, Alisan

    2016-12-01

    ICF syndrome is a primary immunodeficiency disease characterized by hypo- or agammaglobulinemia, centromeric instability mainly on chromosomes 1, 9, and 16 and facial anomalies. ICF syndrome presents with frequent respiratory tract infections in infancy. A 20-month-old female patient was referred to our clinic due to frequent lower respiratory tract infections. ICF syndrome was considered because of comorbidity of hypogammaglobulinemia, facial anomalies, and neuromotor growth retardation. Metaphase chromosome analysis revealed centromeric instability on chromosomes 1, 9, and 16 and through Sanger a previously unreported homozygous missense mutation (c.1805T>C; [p.V602A]) was identified in the DNMT3B, confirming ICF1. The patient was found to have a breakdown in renal function 1 year later; the urinary system was examined and bilateral vesicoureteral reflux was found, warranting the need for dialysis in time. This report expands the mutation spectrum of ICF1 and is the first to describe bilateral vesicoureteral reflux accompanying ICF syndrome. © 2016 Wiley Periodicals, Inc. © 2016 Wiley Periodicals, Inc.

  8. Temporal trend and climate factors of hemorrhagic fever with renal syndrome epidemic in Shenyang City, China

    Directory of Open Access Journals (Sweden)

    Liu Xiaodong

    2011-12-01

    Full Text Available Abstract Background Hemorrhagic fever with renal syndrome (HFRS is an important infectious disease caused by different species of hantaviruses. As a rodent-borne disease with a seasonal distribution, external environmental factors including climate factors may play a significant role in its transmission. The city of Shenyang is one of the most seriously endemic areas for HFRS. Here, we characterized the dynamic temporal trend of HFRS, and identified climate-related risk factors and their roles in HFRS transmission in Shenyang, China. Methods The annual and monthly cumulative numbers of HFRS cases from 2004 to 2009 were calculated and plotted to show the annual and seasonal fluctuation in Shenyang. Cross-correlation and autocorrelation analyses were performed to detect the lagged effect of climate factors on HFRS transmission and the autocorrelation of monthly HFRS cases. Principal component analysis was constructed by using climate data from 2004 to 2009 to extract principal components of climate factors to reduce co-linearity. The extracted principal components and autocorrelation terms of monthly HFRS cases were added into a multiple regression model called principal components regression model (PCR to quantify the relationship between climate factors, autocorrelation terms and transmission of HFRS. The PCR model was compared to a general multiple regression model conducted only with climate factors as independent variables. Results A distinctly declining temporal trend of annual HFRS incidence was identified. HFRS cases were reported every month, and the two peak periods occurred in spring (March to May and winter (November to January, during which, nearly 75% of the HFRS cases were reported. Three principal components were extracted with a cumulative contribution rate of 86.06%. Component 1 represented MinRH0, MT1, RH1, and MWV1; component 2 represented RH2, MaxT3, and MAP3; and component 3 represented MaxT2, MAP2, and MWV2. The PCR model

  9. The functional state of neutrophils correlates with the severity of renal dysfunction in children with hemolytic uremic syndrome.

    Science.gov (United States)

    Fernandez, Gabriela C; Gomez, Sonia A; Ramos, Maria V; Bentancor, Leticia V; Fernandez-Brando, Romina J; Landoni, Veronica I; Lopez, Laura; Ramirez, Flavia; Diaz, Mario; Alduncin, Marta; Grimoldi, Irene; Exeni, Ramon; Isturiz, Martin A; Palermo, Marina S

    2007-01-01

    Hemolytic Uremic Syndrome (HUS) is the main cause of acute renal failure in children. The high percentage of patients who develop long-term sequelae constitutes an important medical concern. The identification of parameters that correlate with the degree of renal failure may be useful to plan the best treatment soon after hospitalization. Here, we investigated the functional state of neutrophils (PMN) from HUS patients on admission, before dialysis and/or transfusion, in relation to the severity of renal impairment reached during the acute period (AP). We found that all PMN activation parameters measured in severe cases of HUS (HUS AP3) were statistically lower comparing to children with mild cases of HUS (HUS AP1). As HUS PMN phenotype and dysfunction is compatible with that of cells undergoing cell death, we also studied spontaneous apoptosis. Not only were HUS PMN not apoptotic, but HUS AP3 PMN showed an increased survival. Almost all phenotypic and functional parameters measured on PMN correlated with severity. Our results revealed a marked deactivation of PMN in severe cases of HUS, and suggest that studying the functional state of PMN could be of prognostic value.

  10. Fulminant Buddchiari syndrome caused by renal primitive neuroectodermal tumor with inferior vena cava thrombus extending to atrium.

    Science.gov (United States)

    Mete, Uttam K; Singh, Dig Vijay; Bhattacharya, Anish; Kakkar, Nandita

    2015-01-01

    Primitive neuroectodermal tumors (PNET) of the kidney are rare, the diagnosis usually being made at histopathology. A young female presented with a massive right renal mass with features of hepatic dysfunction. Computed tomography scan of the abdomen revealed a large tumor of right kidney with tumor thrombus extending from inferior vena cava (IVC) to right atrium with features suggesting Buddchiari syndrome (BCS). Needle biopsy of mass showed a round cell neoplasm and positive staining for neuron specific enolase and minimum inhibitory concentration-2 on immunohistochemistry. She was managed with neo-adjuvant chemotherapy, surgery and adjuvant chemotherapy. To the best of our knowledge this is the first case of renal PNET with inferior IVC tumor thrombus extending to right atrium with BCS. We suggest that renal PNET should be kept in mind as a differential diagnosis in young adults presenting with a large kidney mass extending to IVC that shows evidence of necrosis on imaging, which may be associated with BCS as in index case.

  11. Acute Renal Failure in Association with Community-Acquired Clostridium difficile Infection and McKittrick-Wheelock Syndrome

    Directory of Open Access Journals (Sweden)

    Robert M. Learney

    2011-08-01

    Full Text Available We report the case of a 65-year-old Caucasian woman who experienced two separate episodes of acute renal failure within an 18-month period, both requiring emergency admission and complicated treatment. Each episode was precipitated by hypovolaemia from intestinal fluid losses, but from two rare and independent pathologies. Her first admission was attributed to community-acquired Clostridium difficile-associated diarrhoea (CDAD and was treated in the intensive therapy unit. She returned 18 months later with volume depletion and electrolyte disturbances, but on this occasion a giant hypersecretory villous adenoma of the rectum (McKittrick-Wheelock syndrome was diagnosed following initial abnormal findings on digital rectal examination by a junior physician. Unlike hospital-acquired C. difficile, community-acquired infection is not common, although increasing numbers are being reported. Whilst community-acquired CDAD can be severe, it rarely causes acute renal failure. This case report highlights the pathological mechanisms whereby C. difficile toxin and hypersecretory villous adenoma of the rectum can predispose to acute renal failure, as well as the values of thorough clinical examination in the emergency room, and early communication with intensivist colleagues in dire situations.

  12. Association of urothelial carcinoma of the renal pelvis with papillary and medullary thyroid carcinomas. A new sporadic neoplastic syndrome?

    Science.gov (United States)

    Albores-Saavedra, Jorge; Dorantes-Heredia, Rita; Chablé-Montero, Fredy; Córdova-Ramón, Juan Carlos; Henson, Donald E

    2014-10-01

    We describe 2 adult women (72 and 54 years), 1 with a low-grade noninvasive papillary urothelial carcinoma of the renal pelvis, who 14 years later developed a papillary carcinoma in 1 thyroid lobe and a medullary carcinoma in the contralateral lobe. Both neoplasms were similar in size and appeared symmetrical. Despite its small size, the medullary carcinoma metastasized in multiple cervical lymph nodes. The second patient had a high-grade invasive papillary urothelial carcinoma of the renal pelvis that infiltrated the renal parenchyma and metastasized in one of the lungs. Five months later, a papillary carcinoma was discovered in the thyroid gland. The 2 papillary thyroid carcinomas were of the follicular variant. Adjacent to 1 papillary carcinoma, there was a dominant nodule of a colloid and adenomatous goiter. The medullary carcinoma contained stromal amyloid and was immunoreactive for calcitonin and carcinoembryonic antigen. There was no C-cell hyperplasia (medullary carcinoma in situ). The 2 patients are alive, 1 is living with pulmonary metastasis from the high-grade urothelial carcinoma. Twelve cases of this neoplastic association were registered in the Survey, Epidemiology, and End Results Program from 1980 to 2009. We believe that the combination of these unusual neoplasms in the same patient may represent a new sporadic neoplastic syndrome.

  13. Diagnosis and management of fluid overload in heart failure and cardio-renal syndrome: the "5B" approach.

    Science.gov (United States)

    Ronco, Claudio; Kaushik, Manish; Valle, Roberto; Aspromonte, Nadia; Peacock, W Frank

    2012-01-01

    Cardio-Renal syndrome may occur as a result of either primarily renal or cardiac dysfunction. This complex interaction requires a tailored approach to manage the underlying pathophysiology while optimizing the patient's symptoms and thus providing the best outcomes. Patients often are admitted to the hospital for signs and symptoms of congestion and fluid overload is the most frequent cause of subsequent re-admission. Fluid management is of paramount importance in the strategy of treatment for heart failure patients. Adequate fluid status should be obtained but a target value should be set according to objective indicators and biomarkers. Once the fluid excess is identified, a careful prescription of fluid removal by diuretics or extracorporeal therapies must be made. While delivering these therapies, adequate monitoring should be performed to prevent unwanted effects such as worsening of renal function or other complications. There is a very narrow window of optimal hydration for heart failure patients. Overhydration can result in myocardial stretching and potential decompensation. Inappropriate dehydration or relative reduction of circulating blood volume may result in distant organ damage caused by inadequate perfusion. We suggest consideration of the "5B" approach. This stands for balance of fluids (reflected by body weight), blood pressure, biomarkers, bioimpedance vector analysis, and blood volume. Addressing these parameters ensures that the most important issues affecting symptoms and outcomes are addressed. Furthermore, the patient is receiving the best possible care while avoiding unwanted side effects of the treatment. Copyright © 2012 Elsevier Inc. All rights reserved.

  14. Severe Renal Hemorrhage in a Pregnant Woman Complicated with Antiphospholipid Syndrome: A Case Report

    OpenAIRE

    Shohei Kawaguchi; Kouji Izumi; Takahiro Nohara; Tohru Miyagi; Hiroyuki Konaka; Atsushi Mizokami; Eitetsu Koh; Mikio Namiki

    2011-01-01

    Antiphospholipid syndrome is a systemic autoimmune disease with thrombotic tendency. Consensus guidelines for pregnancy with antiphospholipid syndrome recommend low-dose aspirin combined with unfractionated or low-molecular-weight heparin because antiphospholipid syndrome causes habitual abortion. We report a 36-year-old pregnant woman diagnosed with antiphospholipid syndrome receiving anticoagulation treatment. The patient developed left abdominal pain and gross hematuria at week 20 of pregn...

  15. Atmospheric moisture variability and transmission of hemorrhagic fever with renal syndrome in Changsha City, Mainland China, 1991-2010.

    Directory of Open Access Journals (Sweden)

    Hong Xiao

    Full Text Available BACKGROUND: The transmission of hemorrhagic fever with renal syndrome (HFRS is influenced by environmental determinants. This study aimed to explore the association between atmospheric moisture variability and the transmission of hemorrhagic fever with renal syndrome (HFRS for the period of 1991-2010 in Changsha, China. METHODS AND FINDINGS: Wavelet analyses were performed by using monthly reported time series data of HFRS cases to detect and quantify the periodicity of HFRS. A generalized linear model with a Poisson distribution and a log link model were used to quantify the relationship between climate and HFRS cases, highlighting the importance of moisture conditions. There was a continuous annual oscillation mode and multi-annual cycle around 3-4 years from 1994 to 1999. There was a significant association of HFRS incidence with moisture conditions and the Multivariate El Niño-Southern Oscillation Index (MEI. Particularly, atmospheric moisture has a significant effect on the propagation of HFRS; annual incidence of HFRS was positively correlated with annual precipitation and annual mean absolute humidity. CONCLUSIONS: The final model had good accuracy in forecasting the occurrence of HFRS and moisture condition can be used in disease surveillance and risk management to provide early warning of potential epidemics of this disease.

  16. Posterior Reversible Encephalopathy Syndrome in a Patient with Newly Diagnosed HIV Infection and End Stage Renal Disease.

    Science.gov (United States)

    Kurukumbi, Mohankumar; Castellanos, Maria I; Crawford, Amanda K; Gowdar, Shreyas D; Jayam-Trouth, Annapurni

    2013-01-01

    Posterior reversible encephalopathy syndrome (PRES) is a clinicoradiological syndrome in which patients present with an acute or subacute clinical presentation of seizures, visual disturbances, headache, and altered mental status. The pathophysiology of PRES may be explained by endothelial dysfunction that leads to transudation of fluids and protein, resulting in vasogenic cerebral edema. PRES is typically associated with many conditions such as hypertension, uremia, immunosuppressive drugs, and sepsis. This is a case report of a 39-year-old woman with untreated HIV infection and end-stage renal disease (ESRD) who developed PRES with a normal blood pressure and no other known causes of PRES. Untreated HIV is associated with known endothelial dysfunction and we believe that this, in combination with her untreated end-stage renal disease, contributed to her unique presentation of PRES. Although uncommon in HIV-infected patients and challenging to diagnose, prompt recognition of PRES is critical to provide appropriate care and ensure reversibility of the vasogenic edema seen in PRES.

  17. Posterior Reversible Encephalopathy Syndrome in a Patient with Newly Diagnosed HIV Infection and End Stage Renal Disease

    Directory of Open Access Journals (Sweden)

    Mohankumar Kurukumbi

    2013-01-01

    Full Text Available Posterior reversible encephalopathy syndrome (PRES is a clinicoradiological syndrome in which patients present with an acute or subacute clinical presentation of seizures, visual disturbances, headache, and altered mental status. The pathophysiology of PRES may be explained by endothelial dysfunction that leads to transudation of fluids and protein, resulting in vasogenic cerebral edema. PRES is typically associated with many conditions such as hypertension, uremia, immunosuppressive drugs, and sepsis. This is a case report of a 39-year-old woman with untreated HIV infection and end-stage renal disease (ESRD who developed PRES with a normal blood pressure and no other known causes of PRES. Untreated HIV is associated with known endothelial dysfunction and we believe that this, in combination with her untreated end-stage renal disease, contributed to her unique presentation of PRES. Although uncommon in HIV-infected patients and challenging to diagnose, prompt recognition of PRES is critical to provide appropriate care and ensure reversibility of the vasogenic edema seen in PRES.

  18. Renal dysfunction due to hydronephrosis by SAPHO syndrome: a case report

    OpenAIRE

    Kakoki, Katsura; Miyata, Yasuyoshi; Enokizono, Mikako; Uetani, Masataka; Sakai , Hideki

    2015-01-01

    Key Clinical Message Synovitis, Acne, Pustulosis, Hyperostosis, Osteitis (SAPHO) syndrome shows varied pathological symptoms. This is the first report of hydronephrosis due to the mechanical compression of bilateral ureters as a result of SAPHO syndrome. From our experience, MRI is the most useful imaging examination to check the upper urinary tract in SAPHO syndrome.

  19. Renal dysfunction due to hydronephrosis by SAPHO syndrome: a case report.

    Science.gov (United States)

    Kakoki, Katsura; Miyata, Yasuyoshi; Enokizono, Mikako; Uetani, Masataka; Sakai, Hideki

    2015-08-01

    Synovitis, Acne, Pustulosis, Hyperostosis, Osteitis (SAPHO) syndrome shows varied pathological symptoms. This is the first report of hydronephrosis due to the mechanical compression of bilateral ureters as a result of SAPHO syndrome. From our experience, MRI is the most useful imaging examination to check the upper urinary tract in SAPHO syndrome.

  20. On the role of the polychaete Dendronereis spp. i the transmission of white spot syndrome virus in shrimp ponds

    NARCIS (Netherlands)

    Desrina, Haryadi

    2014-01-01

    White spot syndrome virus (WSSV) is by far the most devastating shrimp virus. Control measures have lowered the WSSV incidence to various degrees, but the pathogen remains plaguing shrimp culture worldwide. Continuous exposure may cause WSSV to adapt and infect non-crustacean benthic fauna in ponds

  1. Down syndrome : A novel risk factor for respiratory syncytial virus bronchiolitis - A prospective birth-cohort study

    NARCIS (Netherlands)

    Bloemers, Beatrijs L. P.; van Furth, Marceline; Weijerman, Michel E.; Gemke, Reinoud J. B. J.; Broers, Chantal J. M.; van den Ende, Kimberly; Kimpen, Jan L. L.; Strengers, Jan L. M.; Bont, Louis J.

    2007-01-01

    OBJECTIVES. Respiratory syncytial virus is the single-most important cause of lower respiratory tract infections in children. Preterm birth and congenital heart disease are known risk factors for severe respiratory syncytial virus infections. Although Down syndrome is associated with a high risk of

  2. Protein profiling in the gut of Penaeus monodon gavaged with oral WSSV-vaccines and live white spot syndrom virus

    NARCIS (Netherlands)

    Kulkarni, A.D.; Kiron, V.; Rombout, J.H.W.M.; Brinchmann, M.; Fernandes, J.M.O.; Sudheer, N.S.; Singh, B.I.S.

    2014-01-01

    White spot syndrome virus (WSSV) is a pathogen that causes considerable mortality of the farmed shrimp, Penaeus monodon. Candidate ‘vaccines’, WSSV envelope protein VP28 and formalin-inactivated WSSV, can provide short-lived protection against the virus. In this study, P. monodon was orally intubate

  3. Zika virus detection in urine from patients with Guillain-Barré syndrome on Martinique, January 2016.

    Science.gov (United States)

    Rozé, Benoît; Najioullah, Fatiha; Fergé, Jean-Louis; Apetse, Kossivi; Brouste, Yannick; Cesaire, Raymond; Fagour, Cédric; Fagour, Laurence; Hochedez, Patrick; Jeannin, Séverine; Joux, Julien; Mehdaoui, Hossein; Valentino, Ruddy; Signate, Aïssatou; Cabié, André

    2016-01-01

    We report two cases of Guillain-Barré syndrome who had concomitant Zika virus viruria. This viruria persisted for longer than 15 days after symptom onset. The cases occurred on Martinique in January 2016, at the beginning of the Zika virus outbreak. Awareness of this possible neurological complication of ZikV infection is needed.

  4. Meningitis and stridor in advanced Human immunodeficiency virus/acquired immune deficiency syndrome

    Directory of Open Access Journals (Sweden)

    Naidoo P

    2013-09-01

    Full Text Available P Naidoo, D Pillay, S SamanDepartment of Internal Medicine, Port Shepstone Regional Hospital, University of KwaZulu-Natal, South AfricaAbstract: A 37-year-old female presented confused with a preceding history of severe headache. After clinical examination and investigations, she was diagnosed with disseminated tuberculosis (including central nervous system involvement, and Human immunodeficiency virus/acquired immune deficiency syndrome. Her hospital stay was complicated. She developed stridor and a cerebrovascular accident with left hemiplegia. She died approximately 2 weeks after admission. The potential causes of her stridor included a mediastinal mass or a central mechanism secondary to tuberculosis meningitis. Limited resources precluded definitive imaging of the chest to rule out a mediastinal mass. Further, an autopsy was not done. Despite these limitations, this case is unique because it reports the presence of both stridor and tuberculosis meningitis in an adult patient.Keywords: Human immunodeficiency virus, acquired immune deficiency syndrome, meningitis, stridor, tuberculosis

  5. Avian hepatitis E virus, vaccines and methods of protecting against avian hepatitis-splenomegaly syndrome and mammalian hepatitis E

    OpenAIRE

    2009-01-01

    The present invention relates to a novel isolated avian hepatitis E virus having a nucleotide sequence set forth in SEQ ID NO:1 or its complementary strand. The invention further concerns immunogenic compositions comprising this new virus or recombinant products such as the nucleic acid and vaccines that protect an avian or mammalian species from viral infection or hepatitis-splenomegaly syndrome caused by the hepatitis E virus. Also included in the scope of the invention is a method for prop...

  6. Down's syndrome is a risk factor for severe lower respiratory tract infection due to respiratory syncytial virus

    NARCIS (Netherlands)

    Galleguillos, Constanza; Galleguillos, Bárbara; Larios, Guillermo; Menchaca, Gonzalo; Bont, Louis; Castro-Rodriguez, Jose A

    2016-01-01

    AIM: Previous studies have suggested that Down's syndrome is an independent risk factor for severe respiratory infection due to respiratory syncytial virus (RSV). We compared the clinical characteristics of children with and without Down's syndrome hospitalised due to RSV. METHODS: This retrospectiv

  7. Angiographic Features and Cardiovascular Risk Factors in Human Immunodeficiency Virus-Infected Patients With First-Time Acute Coronary Syndrome

    DEFF Research Database (Denmark)

    Knudsen, Andreas; Mathiasen, Anders B; Worck, R.H.

    2013-01-01

    A matched cohort study was conducted comparing patients with first-time acute coronary syndromes infected with human immunodeficiency virus (HIV) to non-HIV-infected patients with and without diabetes matched for smoking, gender, and type of acute coronary syndrome who underwent first-time coronary...

  8. Down's syndrome is a risk factor for severe lower respiratory tract infection due to respiratory syncytial virus

    NARCIS (Netherlands)

    Galleguillos, Constanza; Galleguillos, Bárbara; Larios, Guillermo; Menchaca, Gonzalo; Bont, Louis|info:eu-repo/dai/nl/304813370; Castro-Rodriguez, Jose A

    2016-01-01

    AIM: Previous studies have suggested that Down's syndrome is an independent risk factor for severe respiratory infection due to respiratory syncytial virus (RSV). We compared the clinical characteristics of children with and without Down's syndrome hospitalised due to RSV. METHODS: This retrospectiv

  9. Comparison of hemagglutination inhibition test and ELISA in quantification of antibodies to egg drop syndrome virus.

    Science.gov (United States)

    Raj, G Dhinakar; Ratnapraba, S; Matheswaran, K; Nachimuthu, K

    2004-01-01

    A single-serum dilution ELISA for egg drop syndrome (EDS) virus-specific antibodies was developed. In testing 425 chicken sera it was found to have a 93.6% sensitivity and 98.7% specificity relative to a hemagglutination inhibition (HI) test. The correlation coefficient for ELISA and HI titers was 0.793. The ELISA was efficacious in quantification of both vaccinal and infection antibodies and could routinely be used for screening large numbers of field sera.

  10. Meningitis and stridor in advanced Human immunodeficiency virus/acquired immune deficiency syndrome

    OpenAIRE

    Naidoo P; Pillay D; Saman S

    2013-01-01

    P Naidoo, D Pillay, S SamanDepartment of Internal Medicine, Port Shepstone Regional Hospital, University of KwaZulu-Natal, South AfricaAbstract: A 37-year-old female presented confused with a preceding history of severe headache. After clinical examination and investigations, she was diagnosed with disseminated tuberculosis (including central nervous system involvement), and Human immunodeficiency virus/acquired immune deficiency syndrome. Her hospital stay was complicated. She developed stri...

  11. Effect of porcine reproductive and respiratory syndrome virus (PRRSV) on alveolar lung macrophage survival and function

    DEFF Research Database (Denmark)

    Oleksiewicz, Martin B.; Nielsen, Jens

    1999-01-01

    Porcine reproductive and respiratory syndrome virus (PRRSV) recently emerged as an important cause of reproductive disorders and pneumonia in domestic pigs throughout the world. Acute cytocidal replication of PRRSV in alveolar lung macrophages causes the acute pneumonia; however, it remains largely...... infection in this system. In short, in our minimal system containing only a single cell type, phagocytosis-suppressive effects of PRRSV infection were detected, that acted at the culture level by reducing the total number of alveolar lung macrophages....

  12. Vanishing bile duct syndrome in human immunodeficiency virus: Nevirapine hepatotoxicity revisited

    Institute of Scientific and Technical Information of China (English)

    Rajan; Kochar; Moises; I; Nevah; Frank; J; Lukens; Michael; B; Fallon; Victor; I; Machicao

    2010-01-01

    Vanishing bile duct syndrome (VBDS) refers to a group of disorders characterized by prolonged cholestasis as a result of destruction and disappearance ofintrahepatic bile ducts. Multiple etiologies have been indentifi ed including infections, neoplastic disorders, autoimmune conditions and drugs. The natural history of this condition is variable and may involve resolution of cholestasis or progression with irreversible damage. VBDS is extremely rare in human immunodeficiency virus (HIV)-infected patients an...

  13. Haemaphysalis longicornis Ticks as Reservoir and Vector of Severe Fever with Thrombocytopenia Syndrome Virus in China

    OpenAIRE

    Luo, Li-mei; Zhao, Li; Wen, Hong-Ling; Zhang, Zhen-Tang; Liu, Jian-Wei; Fang, Li-Zhu; Xue, Zai-Feng; Ma, Dong-Qiang; Zhang, Xiao-Shuang; Ding, Shu-Jun; Lei, Xiao-Ying; Yu, Xue-jie

    2015-01-01

    Severe fever with thrombocytopenia syndrome (SFTS) is an emerging hemorrhagic fever in East Asia caused by SFTS virus (SFTSV), a newly discovered phlebovirus. The Haemaphysalis longicornis tick has been suspected to be the vector of SFTSV. To determine whether SFTSV can be transmitted among ticks, from ticks to animals, and from animals to ticks, we conducted transmission studies between developmental stages of H. longicornis ticks and between ticks and mice. Using reverse transcription PCR, ...

  14. Renal leishmaniasis as unusual cause of nephrotic syndrome in an HIV patient.

    Science.gov (United States)

    Amann, Kerstin; Bogdan, Christian; Harrer, Thomas; Rech, Juergen

    2012-04-01

    Renal involvement is a rare complication in HIV-1-infected patients leading to various pathologies and clinical symptoms. In addition to the classic HIV-1-associated nephropathy with collapsing-type focal segmental glomerulosclerosis and characteristic tubulocystic changes, which is more common in Afro-American than in Caucasian HIV-1 patients, immune complex GNs such as membranous GN and membranoproliferative GN are particularly common renal manifestations. Besides HIV-1 itself, a number of opportunistic infections may cause renal disease in HIV-1-infected patients. In this study, we report an unusual case of HIV-1 infection with a severe renal manifestation of systemic leishmaniasis that developed years after repeated visits to Mediterranean countries. The case presents several remarkable clinical, pathologic, and therapeutic aspects that may be important for daily clinical practice.

  15. Recombinant egg drop syndrome subunit vaccine offers an alternative to virus propagation in duck eggs.

    Science.gov (United States)

    Gutter, B; Fingerut, E; Gallili, G; Eliahu, D; Perelman, B; Finger, A; Pitcovski, J

    2008-02-01

    Egg drop syndrome (EDS) virus vaccines are routinely produced in embryonated duck eggs (Solyom et al., 1982). This procedure poses the risk of dissemination of pathogens, such as avian influenza virus, as the eggs used are not from specific pathogen free birds. To address this problem, the knob and part of the shaft domain of the fibre protein of the EDS virus (termed knob-s) were expressed in Escherichia coli and assessed as a subunit vaccine. A single vaccination with the recombinant protein induced the production of anti-EDS virus antibodies, as detected by haemagglutination inhibition, enzyme-linked immunosorbent assay and virus neutralization tests, for at least 20 weeks. A positive correlation was demonstrated between these three assays. A dose-response assessment showed that the vaccine was effective over the range of 2 to 64 microg protein per dose. Two vaccinations with the recombinant protein, administered before the onset of lay, induced high haemagglutination inhibition antibody titres, comparable with those induced by an inactivated whole-virus vaccine. The vaccine did not have any adverse effects on egg production, quality or weight. The present study has shown that two vaccinations with the recombinant knob-s protein elicited high neutralizing antibody titres that persisted for more than 50 weeks of lay.

  16. The role of white spot syndrome virus (WSSV) VP466 protein in shrimp antiviral phagocytosis.

    Science.gov (United States)

    Ye, Ting; Zong, Rongrong; Zhang, Xiaobo

    2012-08-01

    Widespread evidence indicates that the structural proteins of virus play very important roles in virus-host interactions. However, the effect of viral proteins on host immunity has not been addressed. Our previous studies revealed that the host shrimp Rab6 (termed as PjRab previously), tropomyosin, β-actin and the white spot syndrome virus (WSSV) envelope protein VP466 formed a complex. In this study, the VP466 protein was shown to be able to bind host Rab6 protein and increase its GTPase activity in vivo and vitro. Thus, VP466 could function as a GTPase-activating protein (GAP) of Rab6. In the VP466-Rab-actin pathway, the increase of the Rab6 activity induced rearrangements of the actin cytoskeleton, resulting in the formation of actin stress fibers which promoted the phagocytosis against virus. Therefore our findings revealed that a viral protein could be employed by host to initiate the host immunity, representing a novel molecular mechanism in the virus-host interaction. Our study would help to better understand the molecular events in immune response against virus infection in invertebrates.

  17. Renal Leishmaniasis as Unusual Cause of Nephrotic Syndrome in an HIV Patient

    OpenAIRE

    2012-01-01

    Renal involvement is a rare complication in HIV-1–infected patients leading to various pathologies and clinical symptoms. In addition to the classic HIV-1–associated nephropathy with collapsing-type focal segmental glomerulosclerosis and characteristic tubulocystic changes, which is more common in Afro-American than in Caucasian HIV-1 patients, immune complex GNs such as membranous GN and membranoproliferative GN are particularly common renal manifestations. Besides HIV-1 itself, a number of ...

  18. Flash pulmonary edema in patients with renal artery stenosis--the Pickering Syndrome

    DEFF Research Database (Denmark)

    Pelta, Anna; Andersen, Ulrik B; Just, Sven

    2010-01-01

    We report the prevalence of flash pulmonary edema in patients consecutively referred for balloon angioplasty of uni- or bilateral renal artery stenosis (PTRA), and describe the characteristics of this special fraction of the patients. We further report two unusual cases.......We report the prevalence of flash pulmonary edema in patients consecutively referred for balloon angioplasty of uni- or bilateral renal artery stenosis (PTRA), and describe the characteristics of this special fraction of the patients. We further report two unusual cases....

  19. Efficacy of intravenous immunoglobulin in the treatment of persistent BK viremia and BK virus nephropathy in renal transplant recipients.

    Science.gov (United States)

    Vu, D; Shah, T; Ansari, J; Naraghi, R; Min, D

    2015-03-01

    BK virus-associated nephropathy (BKVN) can cause clinically significant viral infection in renal transplant recipients, leading to allograft dysfunction and loss. The usual management of BKVN involves the reduction of immunosuppression and the addition of leflunomide, quinolones, and cidofovir, but the rate of graft loss remains high. The aim of this study was to assess the impact of treatment with intravenous human immunoglobulin (IVIG) on the outcome of BKVN in renal transplant recipients. Upon diagnosis of BKVN, patients remained on anti-polyomavirus treatment, consisting of the reduction of immunosuppression and the use of leflunomide therapy. Treatment with IVIG was given only to patients who did not respond to 8 weeks of the adjustment of immunosuppression and leflunomide. All 30 patients had persistent BKV viremia and BKVN with their mean BK viral loads higher than the baseline (range, 15,000-2 million copies/mL). Mean peak BK load was 205,314 copies/mL compared with 697 copies/mL after 1 year of follow-up. Twenty-seven patients (90%) had a positive response in clearing viremia. The actuarial patient and graft survival rates after 12 months were 100% and 96.7%, respectively. IVIG administration appeared to be safe and effective in treating BKV viremia and BKVN and preventing graft loss in patients who had inadequate response to immunosuppression reduction and leflunomide therapy. Copyright © 2015. Published by Elsevier Inc.

  20. Comparative analysis of signature genes in porcine reproductive and respiratory syndrome virus (PRRSV)-infected porcine monocyte-derived dendritic cells at differential activation statuses

    Science.gov (United States)

    Activation statuses of monocytic cells, e.g. monocytes, macrophages and dendritic cells (DCs), are critically important for antiviral immunity. In particular, some devastating viruses, including porcine reproductive and respiratory syndrome virus (PRRSV), are capable of directly infecting these cell...

  1. Pseudo-Cushing's syndrome in human immunodeficiency virus-infected patients.

    Science.gov (United States)

    Miller, K K; Daly, P A; Sentochnik, D; Doweiko, J; Samore, M; Basgoz, N O; Grinspoon, S K

    1998-07-01

    To our knowledge, an association between human immunodeficiency virus infection and pseudo-Cushing's syndrome has not previously been described. We describe four HIV-infected patients with pseudo-Cushing's syndrome, characterized by striking dorsocervical and submandibular fat accumulation and central obesity. In each case, cortisol levels were either normal or suppressed adequately with administration of dexamethasone, excluding the diagnosis of true Cushing's syndrome. Immune function and weight improved significantly preceding the development of pseudo-Cushing's syndrome. Three of the four patients were taking a common protease inhibitor at the onset of symptoms, and the fourth reported the exacerbation of his symptoms with the addition of a protease inhibitor. The observed characteristic pattern of fat deposition may be attributable to a specific effect of new antiretroviral therapies or may relate to recovery independent of medication usage. Distinguishing between pseudo-Cushing's syndrome and true Cushing's syndrome is critical for preventing the unnecessary and potentially harmful treatment of such patients. Further research into the mechanisms of this novel phenomenon is needed.

  2. Emergence of Epidemic Zika Virus Transmission and Congenital Zika Syndrome: Are Recently Evolved Traits to Blame?

    Science.gov (United States)

    2017-01-01

    ABSTRACT The mechanisms responsible for the dramatic emergence of Zika virus (ZIKV), accompanied by congenital Zika syndrome and Guillain-Barré syndrome (GBS), remain unclear. However, two hypotheses are prominent: (i) evolution for enhanced urban transmission via adaptation to mosquito vectors, or for enhanced human infection to increase amplification, or (ii) the stochastic introduction of ZIKV into large, naive human populations in regions with abundant Aedes aegypti populations, leading to enough rare, severe infection outcomes for their first recognition. Advances in animal models for human infection combined with improvements in serodiagnostics, better surveillance, and reverse genetic approaches should provide more conclusive evidence of whether mosquito transmission or human pathogenesis changed coincidentally with emergence in the South Pacific and the Americas. Ultimately, understanding the mechanisms of epidemic ZIKV emergence, and its associated syndromes, is critical to predict future risks as well as to target surveillance and control measures in key locations. PMID:28074023

  3. Emergence of Epidemic Zika Virus Transmission and Congenital Zika Syndrome: Are Recently Evolved Traits to Blame?

    Science.gov (United States)

    Weaver, Scott C

    2017-01-10

    The mechanisms responsible for the dramatic emergence of Zika virus (ZIKV), accompanied by congenital Zika syndrome and Guillain-Barré syndrome (GBS), remain unclear. However, two hypotheses are prominent: (i) evolution for enhanced urban transmission via adaptation to mosquito vectors, or for enhanced human infection to increase amplification, or (ii) the stochastic introduction of ZIKV into large, naive human populations in regions with abundant Aedes aegypti populations, leading to enough rare, severe infection outcomes for their first recognition. Advances in animal models for human infection combined with improvements in serodiagnostics, better surveillance, and reverse genetic approaches should provide more conclusive evidence of whether mosquito transmission or human pathogenesis changed coincidentally with emergence in the South Pacific and the Americas. Ultimately, understanding the mechanisms of epidemic ZIKV emergence, and its associated syndromes, is critical to predict future risks as well as to target surveillance and control measures in key locations. Copyright © 2017 Weaver.

  4. METODE DETEKSI CEPAT WHITE SPOT SYNDROME VIRUS (WSSV DAN INFECTIUOS MYONECROSIS VIRUS (IMNV MENGGUNAKAN PORTABEL/MOBILE POLYMERASE CHAIN REACTION

    Directory of Open Access Journals (Sweden)

    Isti Koesharyani

    2015-06-01

    Full Text Available Budidaya udang Penaeus monodon dan Litopenaeus vannamei di Indonesia merupakan komoditas primadona untuk ekspor dan merupakan salah satu spesies unggulan dalam program budidaya pada Kementerian Kelautan dan Perikanan. Kendala pada budidaya udang tersebut adalah penyakit yang disebabkan oleh infeksi virus, terutama white spot syndrome virus (WSSV dan infectiuos myonecrosis virus (IMNV. Deteksi secara molekuler yang berbasis DNA merupakan metode deteksi sangat akurat dan tepat. Saat ini sudah ada metode deteksi PCR sederhana yang dapat diaplikasi langsung di lapangan, yaitu dengan menggunakan alat Portable Polymerase Chain Reaction Pockit (iiPCR. Tujuan penelitian ini adalah untuk melakukan deteksi cepat WSSV dan IMNV pada udang P. monodon dan L. vannamei dengan menggunakan portabel-PCR. Aplikasi deteksi ini diujicobakan pada sampel udang yang diawetkan dan sudah diketahui kondisinya. Hasil analisis memperlihatkan hasil yang sesuai dengan hasil analisis sebelumnya dengan menggunakan PCR konvensional. Metode ini relatif lebih sederhana, cepat (hanya memerlukan waktu kurang dari 90 menit dan dapat diketahui hasilnya tanpa adanya proses elektroforesis. Metode deteksi ini sangat membantu pembudidaya dalam monitoring penyakit di lapangan secara on the spot, serta cepat dan tepat.

  5. Production and Evaluation of Virus-Like Particles Displaying Immunogenic Epitopes of Porcine Reproductive and Respiratory Syndrome Virus (PRRSV

    Directory of Open Access Journals (Sweden)

    Ambika Mosale Venkatesh Murthy

    2015-04-01

    Full Text Available Porcine reproductive and respiratory syndrome (PRRS is the most significant infectious disease currently affecting the swine industry worldwide. Several inactivated and modified live vaccines (MLV have been developed to curb PRRSV infections. However, the efficacy and safety of these vaccines are unsatisfactory, and hence, there is a strong demand for the development of new PRRS universal vaccines. Virus-like particle (VLP-based vaccines are gaining increasing acceptance compared to subunit vaccines, as they present the antigens in a more veritable conformation and are readily recognized by the immune system. Hepatitis B virus core antigen (HBcAg has been successfully used as a carrier for more than 100 viral sequences. In this study, hybrid HBcAg VLPs were generated by fusion of the conserved protective epitopes of PRRSV and expressed in E. coli. An optimized purification protocol was developed to obtain hybrid HBcAg VLP protein from the inclusion bodies. This hybrid HBcAg VLP protein self-assembled to 23-nm VLPs that were shown to block virus infection of susceptible cells when tested on MARC 145 cells. Together with the safety of non-infectious and non-replicable VLPs and the low cost of production through E. coli fermentation, this hybrid VLP could be a promising vaccine candidate for PRRS.

  6. Porcine reproductive and respiratory syndrome virus ORF5a protein is essential for virus viability.

    Science.gov (United States)

    Sun, Lichang; Li, Yanhua; Liu, Runxia; Wang, Xiaomin; Gao, Fei; Lin, Tao; Huang, Ting; Yao, Huochun; Tong, Guangzhi; Fan, Hongjie; Wei, Zuzhang; Yuan, Shishan

    2013-01-01

    It has been shown that ORF5a protein in EAV is important but not essential for virus infectivity. In this study, we found that RNA changes in the overlapping region (1-104 nucleotide, nt) between ORF5 and ORF5a introduced by codon-optimized GP5 was lethal for virus viability, suggesting that the nt changes or amino acid (aa) mutations in the GP5 or ORF5a protein did not permit the production of infectious virus. Furthermore, inactivation of ORF5a expression in the context of type 1 (pSHE) and type 2 (pAJXM and pAPRRS) full-length PRRSV cDNA clones was lethal for the production of infectious virus, while viable PRRSV could be recovered by expressing ORF5a protein in trans, suggesting that ORF5a protein was essential for virus viability. Finally, ORF5a protein could be putatively extended to 63 aas by inactivation of the downstream stop codon candidates, thereby demonstrating that the C-terminus of ORF5a may be variable.

  7. Rapidly progressive renal disease as part of Wolfram syndrome in a large inbred Turkish family due to a novel WFS1 mutation (p.Leu511Pro)

    DEFF Research Database (Denmark)

    Yuca, Sevil Ari; Rendtorff, Nanna Dahl; Boulahbel, Houda

    2012-01-01

    Wolfram syndrome, also named "DIDMOAD" (diabetes insipidus, diabetes mellitus, optic atrophy, and deafness), is an inherited association of juvenile-onset diabetes mellitus and optic atrophy as key diagnostic criteria. Renal tract abnormalities and neurodegenerative disorder may occur in the thir...

  8. Acute renal failure secondary to drug-related crystalluria and/or drug reaction with eosinophilia and systemic symptom syndrome in a patient with metastatic lung cancer

    Directory of Open Access Journals (Sweden)

    Saime Paydas

    2017-01-01

    Full Text Available Drug reaction with eosinophilia and systemic symptoms (DRESS or drug-induced hypersensitivity is a severe adverse drug-induced reaction. Aromatic anticonvulsants, such as phenytoin, phenobarbital, and carbamazepine, and some drugs, can induce DRESS. Atypical crystalluria can be seen in patients treated with amoxycillin or some drugs and can cause acute renal failure. We describe a 66-year-old man who presented fever and rash and acute renal failure three days after starting amoxycillin. He was also using phenytoin because of cerebral metastatic lung cancer. Investigation revealed eosinophilia and atypical crystalluria. The diagnosis of DRESS syndrome was made, amoxicillin was stopped, and dose of phenytoin was reduced. No systemic corticosteroid therapy was prescribed. Symptoms began to resolve within three to four days. The aim of this paper is to highlight the importance of microscopic examination of urine in a case with acute renal failure and skin lesions to suspect DRESS syndrome.

  9. 肾移植术后BK病毒相关性肾病的研究现状%Study on BK virus-associated nephropathy after renal transplantation

    Institute of Scientific and Technical Information of China (English)

    肖荆; 田野

    2008-01-01

    BACKGROUND: BK viral infection after renal transplantation influences the prognosis of BK virus-associated nephropathy in renal transplant recipients. The disease has been widely studied in foreign countries.OBJECTIVE: This study was designed to sum up the diagnosis and treatment of BK virus-associated nephropathy after renal transplantation.RETRIVAL STRATEGY: Using the terms "renal transplantation; BK virus" in the English language, manuscripts responsible for BK virus-associated nephropathy after renal transplantation that were published from January 2000 to January 2007 were retrieved from the PubMed database. A total of 206 manuscripts were obtained and primarily screened. Inclusion criteria: studies addressing BK virus-associated nephropathy after renal transplantation. Exclusion criteria: repetitive studies.LITERATURE EVALUATION: The included manuscripts were primarily from PubMed database. Manuscripts were primarily original and review studies.DATA SYNTHESIS: BK virus can be found in the urine of 3%-40% of adult renal transplant recipients. BK virus reactivation rate is very high, but the histological manifestations of BK virus associated nephropathy are found only in a small number of renal transplant recipients. The prognosis of BK virus associated nephropathy is very poor. BK virus associated nephropathy develops into renal failure, leading to transplant loss, in 30%-50% patients. BK virus-caused renal transplant disease must be diagnosed according to histological manifestations. Viral infection should be primarily confirmed, but serological measurements have no predominant effects. Electron microscopy should be involved in the assessment of renal graft biopsy, especially when renal failure factors are unknown, as through the use of electron microscope, viral particles in the nucleus, cytoplasm and outside of the cells could be detectable. Viral antigen in the urine sample of patients with BK viruria can be detected by nucleic acid hybridization method

  10. Prevalence of renal disease in Nigerian children infected with the human immunodeficiency virus and on highly active anti-retroviral therapy.

    Science.gov (United States)

    Iduoriyekemwen, Nosakhare J; Sadoh, Wilson E; Sadoh, Ayebo E

    2013-01-01

    Access to highly active anti-retroviral therapy (HAART) has improved the prognosis of Nigerian children infected with the human immunodeficiency virus (HIV); thus, more children are surviving. Long-term exposure to HAART is potentially nephrotoxic. We therefore aimed at assessing the prevalence of renal disease in Nigerian children infected with HIV, who are on HAART. In this cross-sectional study, we studied children, aged ten months to 17 years, infected with HIV, attending the pediatric HIV clinics of the University of Benin Teaching Hospital. Demographic and clinical data were obtained by parental interview as well as from the medical records. Each child's urine was tested for albumin and microalbuminuria using multi test strips and mitral test strips, respectively. The serum creatinine level of each child was also estimated and used in calculating the glomerular filtration rate (GFR). Renal disease was defined as the presence of significant proteinuria of 1+ and above on dipstick or the presence of microalbuminuria of ≥20 mg and/or GFR renal disease was 16.2%. Microalbuminuria was seen in 11 children with renal disease (11.1%); 3 of them had significant proteinuria. GFR of less than 60 mL/min/1.73 m 2 was seen in five children (5.1%) with renal disease, but none had end-stage renal disease (GFR less than 15 mL/min/1.73 m 2 ). Renal disease was found to be significantly associated with advanced stage of HIV infection (P renal disease in HAART-treated Nigerian children is high and majority of them are asymptomatic of renal disease, but in the advanced stages of HIV infection.

  11. Extrahepatic manifestations of hepatitis E virus.

    Science.gov (United States)

    Kamar, Nassim; Marion, Olivier; Abravanel, Florence; Izopet, Jacques; Dalton, Harry R

    2016-04-01

    Hepatitis E virus can cause acute, fulminant and chronic hepatitis and has been associated with a range of extrahepatic manifestations. Guillain-Barré syndrome, neuralgic amyotrophy and encephalitis are the main neurological manifestations associated with acute and chronic hepatitis E virus infection. Renal injuries have been also reported, including membranoproliferative glomerulonephritis with or without cryoglobulinemia and membranous glomerulonephritis. Acute pancreatitis, haematological disorders and other autoimmune extrahepatic manifestations of hepatitis E virus, such as myocarditis and thyroiditis, have been also reported. In this comprehensive article, we review all published reports describing hepatitis E virus-associated extrahepatic manifestations.

  12. Rapid Response to Cyclosporin A and Favorable Renal Outcome in Nongenetic Versus Genetic Steroid-Resistant Nephrotic Syndrome.

    Science.gov (United States)

    Büscher, Anja K; Beck, Bodo B; Melk, Anette; Hoefele, Julia; Kranz, Birgitta; Bamborschke, Daniel; Baig, Sabrina; Lange-Sperandio, Bärbel; Jungraithmayr, Theresa; Weber, Lutz T; Kemper, Markus J; Tönshoff, Burkhard; Hoyer, Peter F; Konrad, Martin; Weber, Stefanie

    2016-02-05

    Treatment of congenital nephrotic syndrome (CNS) and steroid-resistant nephrotic syndrome (SRNS) is demanding, and renal prognosis is poor. Numerous causative gene mutations have been identified in SRNS that affect the renal podocyte. In the era of high-throughput sequencing techniques, patients with nongenetic SRNS frequently escape the scientific interest. We here present the long-term data of the German CNS/SRNS Follow-Up Study, focusing on the response to cyclosporin A (CsA) in patients with nongenetic versus genetic disease. Cross-sectional and longitudinal clinical data were collected from 231 patients with CNS/SRNS treated at eight university pediatric nephrology units with a median observation time of 113 months (interquartile range, 50-178). Genotyping was performed systematically in all patients. The overall mutation detection rate was high at 57% (97% in CNS and 41% in SRNS); 85% of all mutations were identified by the analysis of three single genes only (NPHS1, NPHS2, and WT1), accounting for 92% of all mutations in patients with CNS and 79% of all mutations in patients with SRNS. Remission of the disease in nongenetic SRNS was observed in 78% of patients after a median treatment period of 2.5 months; 82% of nongenetic patients responded within 6 months of therapy, and 98% of patients with nongenetic SRNS and CsA-induced complete remission (normalbuminemia and no proteinuria) maintained a normal renal function. Genetic SRNS, on the contrary, is associated with a high rate of ESRD in 66% of patients. Only 3% of patients with genetic SRNS experienced a complete remission and 16% of patients with genetic SRNS experienced a partial remission after CsA therapy. The efficacy of CsA is high in nonhereditary SRNS, with an excellent prognosis of renal function in the large majority of patients. CsA should be given for a minimum period of 6 months in these patients with nongenetic SRNS. In genetic SRNS, response to CsA was low and restricted to exceptional

  13. Acute renal failure: A rare presentation of Sheehan′s syndrome

    Directory of Open Access Journals (Sweden)

    Manzoor A Bhat

    2012-01-01

    Full Text Available Sheehan′s syndrome occurs as a result of ischemic pituitary necrosis secondary to severe postpartum bleeding. It is one of the most common causes of hypopituitarism, characterized by variable clinical presentation. Acute kidney injury occurs rarely in Sheehan′s syndrome and most of the cases have been found to be precipitated by rhabdomyolysis. We here present a case of Sheehan′s syndrome with acute kidney injury where theprecipitating cause was chronic hypocortisolemia. We believe this is the first reported case of Sheehan′s syndrome in which acute kidney injury was precipitated by adrenal insufficiency.

  14. Primary EBV infection induces an expression profile distinct from other viruses but similar to hemophagocytic syndromes.

    Science.gov (United States)

    Dunmire, Samantha K; Odumade, Oludare A; Porter, Jean L; Reyes-Genere, Juan; Schmeling, David O; Bilgic, Hatice; Fan, Danhua; Baechler, Emily C; Balfour, Henry H; Hogquist, Kristin A

    2014-01-01

    Epstein-Barr Virus (EBV) causes infectious mononucleosis and establishes lifelong infection associated with cancer and autoimmune disease. To better understand immunity to EBV, we performed a prospective study of natural infection in healthy humans. Transcriptome analysis defined a striking and reproducible expression profile during acute infection but no lasting gene changes were apparent during latent infection. Comparing the EBV response profile to multiple other acute viral infections, including influenza A (influenza), respiratory syncytial virus (RSV), human rhinovirus (HRV), attenuated yellow fever virus (YFV), and Dengue fever virus (DENV), revealed similarity only to DENV. The signature shared by EBV and DENV was also present in patients with hemophagocytic syndromes, suggesting these two viruses cause uncontrolled inflammatory responses. Interestingly, while EBV induced a strong type I interferon response, a subset of interferon induced genes, including MX1, HERC5, and OAS1, were not upregulated, suggesting a mechanism by which viral antagonism of immunity results in a profound inflammatory response. These data provide an important first description of the response to a natural herpesvirus infection in humans.

  15. Comparative Pathogenicity of Liver Homogenate and Cell Culture Propagated Hydropericardium Syndrome Virus in Broiler Birds

    Directory of Open Access Journals (Sweden)

    M. D. Ahmad, S. Zaman1, M. H. Mushtaq*, A. A. Anjum1 and M. Akram1

    2011-10-01

    Full Text Available Comparative pathogenicity of liver homogenate and cell culture propagated agents of hydropericardium syndrome was studied in broiler birds. In Experiment I, 25-day-old while in experiment II, broiler birds at different ages were inoculated through different routes. In Experiment I, liver homogenate caused 64% mortality through intramuscular route and 33.33% mortality through oral route. The cell culture propagated HPS virus caused 60 and 13.33% mortality in broiler birds through intramuscular and oral routes, respectively. In Experiment II, none of the day-old-chicks died when challenged with liver homogenate and cell culture propagated HPS virus through S/C and oral route. The liver homogenate and cell culture propagated HPS virus caused higher mortality in different age groups of broiler birds through s/c route compared to oral route. The values of hemoglobin (Hb and packed cell volume (PCV showed highly significant (P<0.05 reduction indicating anemia. The values of Hb and PCV of the broiler birds inoculated with infectious liver homogenate were significantly lower as compared to birds inoculated with cell culture propagated HPS virus. The results indicated that the liver homogenate is more pathogenic than cell culture propagated HPS virus. These changes may be due to adoptability of the original FAdVs (fowl adenovirus after continued passages in the culture of chicken embryo liver cells. Importance of this study in vaccine production is also discussed.

  16. The Role of Severe Acute Respiratory Syndrome (SARS)-Coronavirus Accessory Proteins in Virus Pathogenesis

    Science.gov (United States)

    McBride, Ruth; Fielding, Burtram C.

    2012-01-01

    A respiratory disease caused by a novel coronavirus, termed the severe acute respiratory syndrome coronavirus (SARS-CoV), was first reported in China in late 2002. The subsequent efficient human-to-human transmission of this virus eventually affected more than 30 countries worldwide, resulting in a mortality rate of ~10% of infected individuals. The spread of the virus was ultimately controlled by isolation of infected individuals and there has been no infections reported since April 2004. However, the natural reservoir of the virus was never identified and it is not known if this virus will re-emerge and, therefore, research on this virus continues. The SARS-CoV genome is about 30 kb in length and is predicted to contain 14 functional open reading frames (ORFs). The genome encodes for proteins that are homologous to known coronavirus proteins, such as the replicase proteins (ORFs 1a and 1b) and the four major structural proteins: nucleocapsid (N), spike (S), membrane (M) and envelope (E). SARS-CoV also encodes for eight unique proteins, called accessory proteins, with no known homologues. This review will summarize the current knowledge on SARS-CoV accessory proteins and will include: (i) expression and processing; (ii) the effects on cellular processes; and (iii) functional studies. PMID:23202509

  17. Primary EBV infection induces an expression profile distinct from other viruses but similar to hemophagocytic syndromes.

    Directory of Open Access Journals (Sweden)

    Samantha K Dunmire

    Full Text Available Epstein-Barr Virus (EBV causes infectious mononucleosis and establishes lifelong infection associated with cancer and autoimmune disease. To better understand immunity to EBV, we performed a prospective study of natural infection in healthy humans. Transcriptome analysis defined a striking and reproducible expression profile during acute infection but no lasting gene changes were apparent during latent infection. Comparing the EBV response profile to multiple other acute viral infections, including influenza A (influenza, respiratory syncytial virus (RSV, human rhinovirus (HRV, attenuated yellow fever virus (YFV, and Dengue fever virus (DENV, revealed similarity only to DENV. The signature shared by EBV and DENV was also present in patients with hemophagocytic syndromes, suggesting these two viruses cause uncontrolled inflammatory responses. Interestingly, while EBV induced a strong type I interferon response, a subset of interferon induced genes, including MX1, HERC5, and OAS1, were not upregulated, suggesting a mechanism by which viral antagonism of immunity results in a profound inflammatory response. These data provide an important first description of the response to a natural herpesvirus infection in humans.

  18. Long term prognosis of acute coronary syndrome with chronic renal dysfunction treated in different therapy units at department of cardiology: a retrospective cohort study.

    Science.gov (United States)

    Fu, Cong; Sheng, Zulong; Yao, Yuyu; Wang, Xin; Yu, Chaojun; Ma, Genshan

    2015-01-01

    Coronary care unit is common in hospitals and clinical centers which offer intensive care and therapy for severe coronary artery disease patients. However, if coronary care unit could improve the long term prognosis of acute coronary syndrome patients with renal dysfunction remain unknown. Accordingly, we designed this study to evaluate the differences of incidence of major adverse cardiovascular events for acute coronary syndromes patients with renal dysfunction who treated in coronary care unit or normal unit. The primary end point was all cause mortality. A total of 414 acute coronary syndromes patients with renal dysfunction involved in the study. The results showed that during 12-48 months follow-up, death of any cause occurred in 1.8% patients (4 of 247) in coronary care unit group, as compared with 1.8% in the normal group (3 of 167) (hazard ratio, 1.098; 95% confidence interval, 0.246 to 4.904; P=0.903). Kaplan-Meier survival analysis showed that there were no significant differences between the two groups with respect to the risk of death (P=0.903), revascularization (P=0.948), stroke (P=0.542), heart failure (P=0.198). This trial firstly revealed that acute coronary syndromes patients with renal dysfunction treated in coronary care unit and normal units. Our study showed that acute coronary syndromes patients with renal dysfunction treated in coronary care unit obtained no significant benefits compared with patients in normal units, although there was a declining tendency of the risk of major adverse cardiovascular effectswith patients in coronary care unit.

  19. Long term prognosis of acute coronary syndrome with chronic renal dysfunction treated in different therapy units at department of cardiology: a retrospective cohort study

    Science.gov (United States)

    Fu, Cong; Sheng, Zulong; Yao, Yuyu; Wang, Xin; Yu, Chaojun; Ma, Genshan

    2015-01-01

    Coronary care unit is common in hospitals and clinical centers which offer intensive care and therapy for severe coronary artery disease patients. However, if coronary care unit could improve the long term prognosis of acute coronary syndrome patients with renal dysfunction remain unknown. Accordingly, we designed this study to evaluate the differences of incidence of major adverse cardiovascular events for acute coronary syndromes patients with renal dysfunction who treated in coronary care unit or normal unit. The primary end point was all cause mortality. A total of 414 acute coronary syndromes patients with renal dysfunction involved in the study. The results showed that during 12-48 months follow-up, death of any cause occurred in 1.8% patients (4 of 247) in coronary care unit group, as compared with 1.8% in the normal group (3 of 167) (hazard ratio, 1.098; 95% confidence interval, 0.246 to 4.904; P=0.903). Kaplan-Meier survival analysis showed that there were no significant differences between the two groups with respect to the risk of death (P=0.903), revascularization (P=0.948), stroke (P=0.542), heart failure (P=0.198). This trial firstly revealed that acute coronary syndromes patients with renal dysfunction treated in coronary care unit and normal units. Our study showed that acute coronary syndromes patients with renal dysfunction treated in coronary care unit obtained no significant benefits compared with patients in normal units, although there was a declining tendency of the risk of major adverse cardiovascular effectswith patients in coronary care unit. PMID:26770436

  20. Postpartum renal vein thrombosis.

    Science.gov (United States)

    Rubens, D; Sterns, R H; Segal, A J

    1985-01-01

    Renal vein thrombosis in adults is usually a complication of the nephrotic syndrome. Rarely, it has been reported in nonnephrotic women postpartum. The thrombosis may be a complication of the hypercoagulable state associated with both the nephrotic syndrome and pregnancy. Two postpartum patients with renal vein thrombosis and no prior history of renal disease are reported here. Neither patient had heavy proteinuria. In both cases, pyelonephritis was suspected clinically and the diagnosis of renal vein thrombosis was first suggested and confirmed by radiologic examination. Renal vein thrombosis should be considered in women presenting postpartum with flank pain.