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Sample records for change nephrotic syndrome

  1. Sagittal sinus thrombosis in adult minimal change nephrotic syndrome.

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    Urch, C; Pusey, C D

    1996-02-01

    Nephrotic syndrome causes a hypercoagulable state, leading to both venous and arterial thrombosis. The mechanisms are as yet unclear, but numerous alterations in coagulant and anti-coagulant factors have been reported [Llach et al. 1985, Harris and Ismail 1994]. The most common clinical features in adults are renal vein thrombosis, femoral vein thrombosis and pulmonary embolism, although thrombosis in numerous other arterial and venous sites has been described [Cameron 1984, Llach et al. 1985]. Intracranial thrombosis is rare, although in adult nephrotic syndrome arterial thrombosis is well recognized [Fuh et al. 1991]. We report a patient with minimal change nephrotic syndrome who developed venous sinus thrombosis detected by magnetic resonance (MR) scanning.

  2. [Cerebral venous thrombosis in minimal change nephrotic syndrome].

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    Hirata, M; Kuroda, M; Koni, I

    1999-06-01

    A 46-year old man presented with an eight-day history of edema and was found to be nephrotic, with a plasma albumin level of 1.1 g/dl and urine protein excretion of 13.3 g/24 hrs. The level of plasma creatinine was normal at 1.0 mg/dl. A finding of renal biopsy was consistent with minimal change glomerulopathy. On the 6th hospital day, he suddenly developed a severe headache and was noted to have bilateral papilledema. Lumbar puncture revealed an opening pressure of 250 mm of water. Magnetic resonance venography showed an irregular flow in the superior sagittal sinus and right transverse sinus, a finding consistent with thrombus. The diagnosis of cerebral venous thrombosis was made, and the patient was given both Warfarin 2 mg/day and prednisolone 60 mg/day. A complete recovery from nephrotic syndrome was achieved within eight weeks. Nephrotic syndrome causes a hypercoagulable state, leading to both venous and arterial thrombosis. The most common clinical features are renal vein thrombosis, femoral vein thrombosis, and pulmonary embolism, however, cerebral venous sinus thrombosis is rare in patients with nephrotic syndrome. It is important to be aware of this complication, since prompt treatment with anticoagulation and control of nephrotic syndrome can lead to a successful outcome.

  3. Complications of nephrotic syndrome

    OpenAIRE

    Se Jin Park; Jae Il Shin

    2011-01-01

    Nephrotic syndrome (NS) is one of the most common glomerular diseases that affect children. Renal histology reveals the presence of minimal change nephrotic syndrome (MCNS) in more than 80% of these patients. Most patients with MCNS have favorable outcomes without complications. However, a few of these children have lesions of focal segmental glomerulosclerosis, suffer from severe and prolonged proteinuria, and are at high risk for complications. Complications of NS are divided into two c...

  4. Concurrence of Bartter syndrome and minimal change nephrotic syndrome

    Institute of Scientific and Technical Information of China (English)

    SHEN Hui-jun; DAI Yu-wen; MAO Jian-hua; LIU Ai-min

    2009-01-01

    @@ Nephrotic syndrome(NS)is a common disease in children with a group of symptoms including heavy proteinuria(≥50 mg/kg per 24 hours),hypoalbuminaemia,hypercholesterolaemia and edema.Bartter syndrome(BS)is a clinically and genetically heterogenous kidney disease characterized by hypokalemia,hypochloremic metabolic alkalosis,obvious increase of rennin,angiotesin II,and normal blood pressure.

  5. Complications of nephrotic syndrome.

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    Park, Se Jin; Shin, Jae Il

    2011-08-01

    Nephrotic syndrome (NS) is one of the most common glomerular diseases that affect children. Renal histology reveals the presence of minimal change nephrotic syndrome (MCNS) in more than 80% of these patients. Most patients with MCNS have favorable outcomes without complications. However, a few of these children have lesions of focal segmental glomerulosclerosis, suffer from severe and prolonged proteinuria, and are at high risk for complications. Complications of NS are divided into two categories: disease-associated and drug-related complications. Disease-associated complications include infections (e.g., peritonitis, sepsis, cellulitis, and chicken pox), thromboembolism (e.g., venous thromboembolism and pulmonary embolism), hypovolemic crisis (e.g., abdominal pain, tachycardia, and hypotension), cardiovascular problems (e.g., hyperlipidemia), acute renal failure, anemia, and others (e.g., hypothyroidism, hypocalcemia, bone disease, and intussusception). The main pathomechanism of disease-associated complications originates from the large loss of plasma proteins in the urine of nephrotic children. The majority of children with MCNS who respond to treatment with corticosteroids or cytotoxic agents have smaller and milder complications than those with steroid-resistant NS. Corticosteroids, alkylating agents, cyclosporin A, and mycophenolate mofetil have often been used to treat NS, and these drugs have treatment-related complications. Early detection and appropriate treatment of these complications will improve outcomes for patients with NS. PMID:22087198

  6. Complications of nephrotic syndrome

    Directory of Open Access Journals (Sweden)

    Se Jin Park

    2011-08-01

    Full Text Available Nephrotic syndrome (NS is one of the most common glomerular diseases that affect children. Renal histology reveals the presence of minimal change nephrotic syndrome (MCNS in more than 80% of these patients. Most patients with MCNS have favorable outcomes without complications. However, a few of these children have lesions of focal segmental glomerulosclerosis, suffer from severe and prolonged proteinuria, and are at high risk for complications. Complications of NS are divided into two categories: disease-associated and drug-related complications. Disease-associated complications include infections (e.g., peritonitis, sepsis, cellulitis, and chicken pox, thromboembolism (e.g., venous thromboembolism and pulmonary embolism, hypovolemic crisis (e.g., abdominal pain, tachycardia, and hypotension, cardiovascular problems (e.g., hyperlipidemia, acute renal failure, anemia, and others (e.g., hypothyroidism, hypocalcemia, bone disease, and intussusception. The main pathomechanism of disease-associated complications originates from the large loss of plasma proteins in the urine of nephrotic children. The majority of children with MCNS who respond to treatment with corticosteroids or cytotoxic agents have smaller and milder complications than those with steroid-resistant NS. Corticosteroids, alkylating agents, cyclosporin A, and mycophenolate mofetil have often been used to treat NS, and these drugs have treatment-related complications. Early detection and appropriate treatment of these complications will improve outcomes for patients with NS.

  7. Minimal change nephrotic syndrome in an 82 year old patient following a tetanus-diphteria-poliomyelitis-vaccination

    OpenAIRE

    Clajus Christian; Spiegel Janine; Bröcker Verena; Chatzikyrkou Christos; Kielstein Jan T

    2009-01-01

    Abstract Background The most common cause of idiopathic nephrotic syndrome in children and younger adults is the minimal change nephrotic syndrome (MCNS). In the elderly MCNS is relatively uncommon. Over the last decade some reports suggest a rare but possible association with the administration of various vaccines. Case presentation A 82-year old Caucasian female presented with pronounced nephrotic syndrome (proteinuria of 7.1 g/d, hypoproteinemia of 47 g/l). About six weeks prior to admissi...

  8. Congenital nephrotic syndrome

    Science.gov (United States)

    The disorder often leads to infection, malnutrition, and kidney failure. It can lead to death by age 5, and many children die within the first year. Congenital nephrotic syndrome may be controlled in some cases with early ...

  9. [Nephrotic syndrome associated with ampulloma].

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    López-Navidad, A; Colomina, J; Domingo, P; Franco, M; Algaba, F

    1989-06-01

    A patient is reported who presented simultaneously classic nephrotic syndrome and adenocarcinoma of the Vater ampulla. Morphological study of the renal biopsy revealed changes characteristic of membranous glomerulonephritis and subepithelial deposits. These deposits stained specifically for IgG and C'3 with a granular pattern, but deposits of CEA-antiCEA immune complexes were not found in glomerular capillaries. The association of nephrotic syndrome with lymphoproliferative diseases and a large variety of solid tumors, like carcinoma of the breast, bronchogenic, colon and stomach has been communicated, but the present case constitutes the first known association with adenocarcinoma of the Vater ampulla. PMID:2772382

  10. Glomerular involution in children with frequently relapsing minimal change nephrotic syndrome: an unrecognized form of glomerulosclerosis?

    NARCIS (Netherlands)

    Dijkman, H.B.P.M.; Wetzels, J.F.M.; Gemmink, J.H.; Baede, J.; Levtchenko, E.N.; Steenbergen, E.

    2007-01-01

    Global glomerulosclerosis can be divided in the vascular (obsolescent) type and the glomerulopathic (solidified) type. In biopsies from children with recurrent nephrotic syndrome owing to minimal change nephropathy (MCN), we noticed small, globally sclerosed glomeruli that appeared to be distinct fr

  11. Hypercoagulability and nephrotic syndrome.

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    Gigante, Antonietta; Barbano, Biagio; Sardo, Liborio; Martina, Paola; Gasperini, Maria L; Labbadia, Raffaella; Liberatori, Marta; Amoroso, Antonio; Cianci, Rosario

    2014-05-01

    Patients with nephrotic syndrome are at increased risk for thromboembolic events such as deep venous and arterial thrombosis, renal vein thrombosis and pulmonary embolism. This thrombophilic phenomenon has been attributed to a "hypercoagulable" state in which an imbalance between naturally occurring pro-coagulant/pro-thrombotic factors and anti-coagulant/antithrombotic factors promotes in situ thrombosis in deep veins or arteries. Management of thromboembolic events may be divided in prophylactic and therapeutic strategies. Hypoalbuminemia is the most significant independent predictor factor of thrombotic risk, especially for values thrombosis. Reviewing the recent literature, we suggest the best therapeutic management of anticoagulation for patients with nephrotic syndrome, focusing on prophylactic strategies.

  12. Radiation nephritis causing nephrotic syndrome

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    Jennette, J.C.; Ordonez, N.G.

    1983-12-01

    Clinical symptoms of acute radiation nephritis with nephrotic syndrome developed in a fifty-six-year-old woman after abdominal radiation therapy for an astrocytoma of the spinal cord. The diagnosis of radiation nephritis was confirmed by renal biopsy. To our knowledge, this is the first documented case of radiation nephritis associated with nephrotic syndrome.

  13. [Secondary nephrotic syndrome due to cardiovascular disease].

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    Hirayama, Tomoya; Takahashi, Fumihiko; Kikuchi, Kenjiro

    2004-10-01

    Cardiovascular diseases ralely evoke nephrotic syndrome. Especially hypertensive renal disease (nephroscrelosis) and renovascular hypertension occasionally may lead to nephrotic syndrome. We reported a case of nephrotic syndrome with renovascular hypertension successfully treated with candesartan. In eldery patients cardiovascular diseases are appeared. It is very important for clinicians to detect the mechanism of nephrotic syndrome caused by cardiovascular diseases. PMID:15500142

  14. Long-term outcome after cyclophosphamide treatment in children with steroid-dependent and frequently relapsing minimal change nephrotic syndrome.

    NARCIS (Netherlands)

    Kyrieleis, H.A.; Levtchenko, E.N.; Wetzels, J.F.M.

    2007-01-01

    BACKGROUND: Seventy percent of children with minimal change nephrotic syndrome (MCNS) have a steroid-dependent or frequent relapsing course of the disease, and most are treated with cyclophosphamide. We describe the clinical course of children with biopsy-proven MCNS treated with cyclophosphamide fo

  15. Minimal change nephrotic syndrome in an 82 year old patient following a tetanus-diphteria-poliomyelitis-vaccination

    Directory of Open Access Journals (Sweden)

    Clajus Christian

    2009-08-01

    Full Text Available Abstract Background The most common cause of idiopathic nephrotic syndrome in children and younger adults is the minimal change nephrotic syndrome (MCNS. In the elderly MCNS is relatively uncommon. Over the last decade some reports suggest a rare but possible association with the administration of various vaccines. Case presentation A 82-year old Caucasian female presented with pronounced nephrotic syndrome (proteinuria of 7.1 g/d, hypoproteinemia of 47 g/l. About six weeks prior to admission, she had received a combination vaccination for tetanus, diphtheria and poliomyelitis as a booster-vaccination from her general practitioner. The renal biopsy revealed typical minimal change lesions. She responded well to the initiated steroid treatment. As through physical examination as well as extensive laboratory and imaging studies did neither find any evidence for malignancies nor infections we suggest that the minimal change nephrotic syndrome in this patient might be related to the activation of the immune system triggered by the vaccination. Conclusion Our case as well as previous anecdotal reports suggests that vaccination and the resulting stimulations of the immune system might cause MCNS and other severe immune-reactions. Increased awareness in that regard might help to expand the database of those cases.

  16. Thrombosis in nephrotic syndrome.

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    Barbano, Biagio; Gigante, Antonietta; Amoroso, Antonio; Cianci, Rosario

    2013-07-01

    Nephrotic syndrome (NS) is characterized by heavy proteinuria, edema, hypoalbuminemia, and hyperlipidemia and the most frequent causes are glomerular diseases. An uncommon presentation is iatrogenic NS, an adverse effect of some drugs administration. In the clinical course of NS, a typical feature is dysregulated coagulation state, promoted by the breakdown of permselectivity barrier of the glomerular capillary wall, resulting in the leakage of high-molecular-mass proteins, at least the size of albumin. This hypercoagulable condition is supported by several factors, such as abnormalities in platelet activation and an imbalance between anticoagulation/antithrombosis and procoagulant/prothrombotic mechanisms. Thus, NS and the risk of developing thromboses are strictly related. Thrombotic events affect the venous system rather than arterial vessels with different features and frequencies. Deep venous system of the lower extremities and renal veins are the most frequent source of pulmonary embolism, the most dangerous NS complication. Prophylactic anticoagulation and thrombosis treatment are not clearly established because large randomized trials and guidelines are lacking. The management of NS and the decision of when and how to anticoagulate the patient represent a teamwork challenge for physicians.

  17. The Genetics of Nephrotic Syndrome.

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    Rheault, Michelle N; Gbadegesin, Rasheed A

    2016-03-01

    Nephrotic syndrome (NS) is a common pediatric kidney disease and is defined as massive proteinuria, hypoalbuminemia, and edema. Dysfunction of the glomerular filtration barrier, which is made up of endothelial cells, glomerular basement membrane, and visceral epithelial cells known as podocytes, is evident in children with NS. While most children have steroid-responsive nephrotic syndrome (SSNS), approximately 20% have steroid-resistant nephrotic syndrome (SRNS) and are at risk for progressive kidney dysfunction. While the cause of SSNS is still not well understood, there has been an explosion of research into the genetic causes of SRNS in the past 15 years. More than 30 proteins regulating the function of the glomerular filtration barrier have been associated with SRNS including podocyte slit diaphragm proteins, podocyte actin cytoskeletal proteins, mitochondrial proteins, adhesion and glomerular basement membrane proteins, transcription factors, and others. A genetic cause of SRNS can be found in approximately 70% of infants presenting in the first 3 months of life and 50% of infants presenting between 4 and 12 months, with much lower likelihood for older patients. Identification of the underlying genetic etiology of SRNS is important in children because it allows for counseling of other family members who may be at risk, predicts risk of recurrent disease after kidney transplant, and predicts response to immunosuppressive therapy. Correlations between genetic mutation and clinical phenotype as well as genetic risk factors for SSNS and SRNS are reviewed in this article. PMID:27617138

  18. Extrarenal complications of the nephrotic syndrome.

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    Harris, R C; Ismail, N

    1994-04-01

    The systemic complications of nephrotic syndrome are responsible for much of the morbidity and mortality seen with this condition. This review discusses the causes for the hypoalbuminemia and the associated metabolic abnormalities of the nephrotic syndrome. No unifying hypothesis exists for the induction, maintenance, and resolution of nephrotic edema. In view of the wide spectrum of renal diseases leading to the nephrotic syndrome, more than a single mechanism may be responsible for the renal salt retention in these diverse conditions. Although hypoalbuminemia may be important, especially when plasma oncotic pressure is very low (serum albumin nephrotic kidney appears to be a major factor in pathogenesis of the edema. However, the decreased serum albumin and/or oncotic pressure seen with nephrotic syndrome is a major contributing factor to the development of the hyperlipidemia of nephrotic syndrome. Patients with unremitting nephrotic syndrome should be considered for combined dietary and lipid-lowering drug therapy. Urinary losses of binding proteins lead to the observed abnormalities in the endocrine system and in trace metals, and urinary losses of coagulation factors contribute to the hypercoagulable state. At present, selective renal venography is recommended when the suspicion of renal vein thrombosis is justified by clinical presentation. The impact on renal function caused by treating asymptomatic chronic renal vein thrombosis is undetermined, but anticoagulation for chronic renal vein thrombosis is associated with relatively few complications.

  19. [Nephrotic syndrome revealed by pulmonary embolism: about four cases].

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    Chaudesaygues, E; Grasse, M; Marchand, L; Villar, E; Aupetit, J-F

    2014-11-01

    Nephrotic syndrom is an association of proteinuria>3g/d or 50mg/kg/d, an hypoalbuminemiadiabetes, high blood pressure and amyloidosis. We present four cases about nephrotic syndrome after thromboembolic disease. In every case, patients show a pulmonary embolism symptomatic of a nephrotic syndrom, whose diagnostic could be delayed up to six months after first pulmonary symptoms. This raised the problem of renal biopsy in these patients who need anticoagulation. In minimal change nephrosis, without hematuria, high blood pressure or renal dysfonction, a corticosteroid therapy test could be done assuming that is corticosensitive minimal glomerular injury. In every case, anticoagulation course must be completed and maintained in case of patent nephrotic syndrom with an albuminemia under 20g/L. In case of pulmonary embolism or deep vein thrombosis, idiopathic-looking, a nephrotic syndrome must be sought-after. The two diagnosis ways are the proteinuria on the urine dipstick and the hypoproteinemia on usual biology. The main mechanism is the coagulation factor leak, side effect of the nephrotic syndrom, notably because of the antithrombin III. PMID:25281996

  20. Clinical significance of changes of plasma concentrations of activators of platelet origin in patients with primary nephrotic syndrome (PNS)

    International Nuclear Information System (INIS)

    Objective: To study the relationship between plasma concentrations of thromboxane (TXB2 ), platelet α-granule membrane protein (GMP-140) and progression of the disease process in patients with primary nephrotic syndrome. Methods: Plasma TXB2 (with RIA) and GMP-140 (with IRMA) concentrations were determined in 31 patients with PNS both before and after prednisone treatment as well as in 31 controls. Results: Plasma contents of TXB2 in patients with PNS were significantly higher than those in the controls (P2 concentrations dropped markedly to near normal levels. However, those who were prednisone-dependent or non-responsive had little changes in TXB2 levels (P>0.05). Plasma GMP-140 concentrations were significantly positively correlated with plasma TXB2 concentrations (r=0.6945, t=3.6134, P2 and GMP-140 concentrations after prednisone therapy is an indicator of treatment success with good prognosis. (authors)

  1. Antithrombin III and the nephrotic syndrome.

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    Jørgensen, K A; Stoffersen, E

    1979-05-01

    Plasma and urinary antithrombin III (AT-III) was measured in 15 cases of nephrotic syndrome. Plasma AT-III correlated well with serum albumin, but poorly with proteinuria, whereas urinary AT-III correlated well to proteinuria. The plasma AT-III level had a mean similar to 25 healthy controls, but the range was significantly wider. A case with nephrotic syndrome and left renal vein thrombosis is reported. The urinary output of AT-III rose and the plasma level fell with the activity of the disease. Although AT-III and albumin have similar molecule weight, their renal clearance was found to be different. It is suggested that urinary loss of AT-III plays a role in the hypercoagulable state sometimes found in the nephrotic syndrome.

  2. Thromboembolic complications in the nephrotic syndrome: pathophysiology and clinical management.

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    Singhal, Rajni; Brimble, K Scott

    2006-01-01

    Patients with the nephrotic syndrome are at increased risk of developing venous and arterial thromboembolism, the most common of which is renal vein thrombosis. There are several unanswered or controversial issues relating to the nephrotic syndrome and thromboembolism, which include the mechanism of thromboembolism, and optimal diagnostic and anticoagulant management strategies. This review will discuss several of these issues: the epidemiology and clinical spectrum of thromboembolic disease occurring in patients with the nephrotic syndrome; the pathophysiology of the hypercoagulable state associated with the nephrotic syndrome; the diagnosis of renal vein thrombosis in the nephrotic syndrome; and the evidence for prophylactic and therapeutic anticoagulation strategies in such patients.

  3. Glucocorticoid pharmacogenetics in pediatric idiopathic nephrotic syndrome.

    Science.gov (United States)

    Cuzzoni, Eva; De Iudicibus, Sara; Franca, Raffaella; Stocco, Gabriele; Lucafò, Marianna; Pelin, Marco; Favretto, Diego; Pasini, Andrea; Montini, Giovanni; Decorti, Giuliana

    2015-01-01

    Idiopathic nephrotic syndrome represents the most common type of primary glomerular disease in children: glucocorticoids (GCs) are the first-line therapy, even if considerable interindividual differences in their efficacy and side effects have been reported. Immunosuppressive and anti-inflammatory effects of these drugs are mainly due to the GC-mediated transcription regulation of pro- and anti-inflammatory genes. This mechanism of action is the result of a complex multistep pathway that involves the glucocorticoid receptor and several other proteins, encoded by polymorphic genes. Aim of this review is to highlight the current knowledge on genetic variants that could affect GC response, particularly focusing on children with idiopathic nephrotic syndrome.

  4. Rheumatoid disease presenting as a nephrotic syndrome.

    OpenAIRE

    Short, C. D.; Solomon, L R; Mallick, N. P.; Mackay, J. D.

    1988-01-01

    A 62 year old man with no relevant previous history presented with a nephrotic syndrome. Renal biopsy showed a membranous glomerulopathy and coincident investigation showed high serum titres of rheumatoid factors. It was not until some months later that he developed articular and extra-articular manifestations of rheumatoid arthritis.

  5. Neph1 Is Reduced in Primary Focal Segmental Glomerulosclerosis, Minimal Change Nephrotic Syndrome, and Corresponding Experimental Animal Models of Adriamycin-Induced Nephropathy and Puromycin Aminonucleoside Nephrosis

    OpenAIRE

    Hulkko, Jenny; Patrakka, Jaakko; Lal, Mark; Tryggvason, Karl; Hultenby, Kjell; Wernerson, Annika

    2014-01-01

    Background/Aims The transmembrane proteins Neph1 and nephrin form a complex in the slit diaphragm (SD) of podocytes. As recent studies indicate an involvement of this complex in the polymerization of the actin cytoskeleton and proteinuria, we wanted to study the subcellular localization of Neph1 in the normal human kidney and its expression in focal segmental glomerulosclerosis (FSGS), minimal change nephrotic syndrome (MCNS), and the corresponding experimental models of Adriamycin-induced ne...

  6. Nephrotic syndrome due to immunoglobulin M mesangial glomerulonephritis preceding juvenile idiopathic arthritis.

    Science.gov (United States)

    Voyer, Luis E; Alvarado, Caupolican; Cuttica, Rubén J; Balestracci, Alejandro; Zardini, Marta; Lago, Néstor

    2013-05-21

    The association between nephrotic syndrome and juvenile idiopathic arthritis have rarely been described in pediatric patients. We report a child with steroid-responsive nephrotic syndrome, with frequent relapses, who presented with a new relapse of nephrotic syndrome associated with arthritis and uveitis at 21 months in remission after treatment with chlorambucil. Juvenile idiopathic arthritis was diagnosed and kidney biopsy examination showed mesangial glomerulonephritis with immunoglobulin M deposits. To our knowledge, only 2 cases of nephrotic syndrome preceding juvenile idiopathic arthritis have been reported, one without histopathology assessment and the other with minimal change disease. Although mesangial glomerulonephritis with nephrotic syndrome and juvenile idiopathic arthritis could have been coincidental, the immune pathogenic mechanism accepted for both diseases suggests they could be related.

  7. Abdominal aortic thrombosis in a patient with nephrotic syndrome.

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    Nakamura, M; Ohnishi, T; Okamoto, S; Yamakado, T; Isaka, N; Nakano, T

    1998-01-01

    We report a patient who presented with severe nephrotic syndrome complicated with infrarenal aortic and right renal arterial thrombosis. The nephrotic syndrome frequently causes thromboembolic complications in veins, but arterial thrombosis is relatively rare, especially in the aorta. Various predisposing factors leading to thromboembolic complications are discussed. In this case, the thromboembolic complication may have some clinical association with the hypercoagulable state in nephrotic syndrome.

  8. Renal artery embolization in severe nephrotic syndrome.

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    Solak, Yalcin; Koc, Osman; Ucar, Ramazan; Ozbek, Orhan; Ergenc, Hasan; Gaipov, Abduzhappar; Turk, Suleyman

    2016-07-01

    Introduction Severe nephrotic syndrome is associated with increased morbidity and mortality. Renal artery embolization (RAE) has been used in a number of renal diseases such as renal tumors, arteriovenous fistulas etc. However, data regarding benefits of RAE in patients with symptomatic severe proteinuria is limited. We decided to evaluate role of RAE in the setting of severe symptomatic nephrotic syndrome. Methods Eight patients who had undergone transcatheter renal artery embolization with polyvinyl alcohol (PVA) were included. Clinico-demographic characteristics as well as baseline laboratory data including level of proteinuria, serum albumin, C-reactive protein and LDL cholesterol levels were recorded for each patient. After RAE, outpatient clinic control laboratory values were also assessed. Findings All patients except one underwent bilateral RAE (four simultaneous or three sequential). Two patients experienced postembolization syndrome characterized by flank pain, fever, and leukocytosis, which was self-limited and responded to analgesics in all patients. There was no technical complications associated with RAE procedure. All patients became anuric except one. Serum albumin levels increased and serum LDL-cholesterol levels decreased considerably in treated patients. Discussion Renal artery embolization with the purpose of amelioration in nephrotic syndrome complications was effective and free of major technical complications in our patients. PMID:26833695

  9. [Effectiveness of dietetic treatment in nephrotic syndrome].

    Science.gov (United States)

    Calleja Fernández, A; López Gómez, J J; Vidal Casariego, A; Cano Rodríguez, I; Ballesteros Pomar, M D

    2009-01-01

    We present the case of a male patient suffering from a primary amyloidosis and a nephrotic syndrome who came to dietotherapy clinic. In the first visit we made a nutritional record including an anthropometric evaluation, body composition, biochemistry, and food intake. The patient had an excess of body water, proteinuria, low plasma protein, albumin, prealbumin and HDL cholesterol levels, and high concentrations of total cholesterol, LDL and triglycerides. The consumption of protein and sodium was higher than the recommendation. An individualized diet was made. Six months later, his weight and the excess of body water decreased, but the fat free mass remained unchanged. The levels of albumin and prealbumin increased, the proteinuria decreased. Total cholesterol, LDL and triglycerides decreased until normal levels. Dietetary treatment in nephrotic syndrome is effective to decrease proteinuria, improve cholesterol and triglycerides levels, and to prevent malnutrition. PMID:20049380

  10. Nephrotic Syndrome and The TCM Treatment

    Institute of Scientific and Technical Information of China (English)

    王巍; 王淑琴

    2004-01-01

    @@ Nephrotic syndrome is a symptom complex characterized by severe albuminuria (+++ to ++++ in qualitative test and > 3.5 g/L in quantitative test),hypoproteinemia (< 3 g/L), edema and hyperlipemia(apparently increased plasma cholesterol and triglyceride and increased low density lipoprotein,LDL) due to abnormally increased permeability of glomerular capillary wall against plasma proteins.There may be lipoiduria with normal or abnormal level of high density lipoprotein (HDL).

  11. Management of idiopathic nephrotic syndrome in childhood

    Directory of Open Access Journals (Sweden)

    Peco-Antić Amira

    2004-01-01

    Full Text Available The management of idiopathic nephrotic syndrome (INS in children includes immunosuppressive and symptomatic treatment. The response to corticosteroid therapy is the best prognostic marker of the disease. The majority of children with INS (about 85% are steroid-sensitive as they normalize proteinuria within 4 weeks of daily, oral prednisone administration. The most of steroid-sensitive patients (94% has minimal change of nephrotic syndrome, while the majority (80.5%-94.4% of those who are steroid-resistant has focal segmental glomerulosderosis or mesangioproliferative glomerulonephritis. Initial therapy of INS consists of 60 mg/m2/day prednisone daily for 4 weeks followed by 40 mg/m2 on alternate days for 4 weeks, thereafter decreasing alternate day therapy every month by 25% over the next 4 months. Thus, the overall duration of the initial cortico-steroids course is 6 months that may be significantly protective against the future development of frequent relapses. Approximately 30% of patients experience only one attack and are cured after the first course of therapy; 10-20% have only 3 or 4 steroid-responsive episodes before permanent cure; the remaining 40-50% of patients are frequent relapsers, or steroid-dependent. Standard relapse therapy consists of 60 mg/m2/ day prednisone until urine is protein free for at least 3 days, followed by 40 mg/m2 on alternate days for 4 weeks. The treatment of frequent-relapses and steroid-dependent INS includes several different regimens: maintenance (6 months alternate steroid therapy just above steroid threshold (0.1-0.5 mg/kg/ 48h, levamisole, alkylating agents (cyclophosphamide or chlorambucil or cyclosporine. The worse prognosis is expected in steroid-resistant patients who are the most difficult to treat. Renal biopsy should be performed in them. At present, there is no consensus on therapeutic regimen for steroid-resistant patients. The following immunosuppressive drugs have been used with varying

  12. Histopathological types in adult nephrotic syndrome

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    Md. Ghulam Yusuf

    2016-01-01

    Full Text Available In Bangladesh, there are very few studies about biopsy proven adult Nephrotic syndrome (NS with histological types and their clinical findings. To determine the histological types of glomerulonephritis (GN in adult NS and correlate them with the clinical presentations and biochemical parameters, we studied 100 biopsies in 87 patients who underwent ultrasonography- guided renal biopsy in Rangpur Medical College and Hospital from July 2010 to June 2012. The mean age of the patients was 32.8 ± 13.2 years; male was preponderance (72.4% and most of the patients (67.8% came from rural areas. Membranoproliferative GN (MPGN was the most common underlying cause that was found in 32 (36.8% patients followed by mesangial prolife- rative GN in 27 (31% patients, membranous GN in 16 (18.4% cases, minimal change disease in four (4.6% patients, diffuse proliferative GN in four (4.6% patients, focal segmental GN, and focal proliferative GN in two (2.4% patients each. High proteinuria level was found in minimal change disease, which was 7.59 ± 0.24 g/24 h (mean ± standard deviation. The most common symptoms were oliguria (92% and edema (86.2% followed by hematuria (dark urine (72.4% and hypertension (35.6%. MPGN was the most common histological type of adult NS in Rangpur.

  13. Treatment of anemia of nephrotic syndrome with recombinant erythropoietin

    NARCIS (Netherlands)

    Gansevoort, RT; Vaziri, ND; deJong, PE

    1996-01-01

    Nephrotic syndrome has been recently shown to cause erythropoietin (EPO) deficiency in humans and experimental models. However, efficacy and safety of recombinant EPO (rEPO) in the treatment of the associated anemia has not been previously investigated. We report a patient with nephrotic syndrome an

  14. Nephrotic Syndrome in Children: From Bench to Treatment

    Directory of Open Access Journals (Sweden)

    J.-C. Davin

    2011-01-01

    Full Text Available Idiopathic nephrotic syndrome (INS is the most frequent form of NS in children. INS is defined by the association of the clinical features of NS with renal biopsy findings of minimal changes, focal segmental glomerulosclerosis (FSGS, or mesangial proliferation (MP on light microscopy and effacement of foot processes on electron microscopy. Actually the podocyte has become the favourite candidate for constituting the main part of the glomerular filtration barrier. Most cases are steroid sensitive (SSINS. Fifty percents of the latter recur frequently and necessitate a prevention of relapses by nonsteroid drugs. On the contrary to SSINS, steroid resistant nephrotic syndrome (SRINS leads often to end-stage renal failure. Thirty to forty percents of the latter are associated with mutations of genes coding for podocyte proteins. The rest is due to one or several different circulating factors. New strategies are in development to antagonize the effect of the latter.

  15. Nephrotic syndrome complicated with portal, splenic, and superior mesenteric vein thrombosis.

    Science.gov (United States)

    Park, Bong Soo; Park, Sihyung; Jin, Kyubok; Choi, Gibok; Park, Kang Min; Jo, Kyeong Min; Kim, Yang Wook

    2014-09-01

    Thromboembolism is a major complication of nephrotic syndrome. Renal vein thrombosis and deep vein thrombosis are relatively common, especially in membranous nephropathy. However, the incidence of portal vein and superior mesenteric vein (SMV) thrombosis in patients with nephrotic syndrome is very rare. To date, several cases of portal vein thrombosis treated by anticoagulation therapy, not by thrombolytic therapy, have been reported as a complication of nephrotic syndrome. Here, we report a case of portal, splenic, and SMV thrombosis in a patient with a relapsed steroid dependent minimal change disease who was treated successfully with anticoagulation and thrombolytic therapy using urokinase. Radiologic findings and his clinical conditions gradually improved. Six months later, a complete remission of the nephrotic syndrome was observed and the follow-up computed tomography scan showed the disappearance of all portal vein, splenic vein, and SMV thrombi.

  16. Serum lipid profile abnormalities among patients with nephrotic syndrome

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    Adu E.M

    2013-01-01

    Full Text Available Background: Nephrotic syndrome is a clinical entity with multiple causes characterized by increased glomerular permeability and manifested by massive proteinuria. Hyperlipidaemia has been found to be one of the cardinal manifestations of nephrotic syndrome. Aim: The present study was conducted to determine the lipid profile and cardiovascular risk of nephrotics in this locality. Methods: Serum total cholesterol (TC, triglycerides (TG, high density lipoprotein (HDL, low density lipoprotein (LDL, very low density lipoprotein (VLDL as well as atherogenic index (AI, coronary risk index (CRI and non-HDL cholesterol were determined in ninety-six subjects. Forty-eight were nephrotic patients while others were apparently healthy individuals used as controls. Result: TC, TG, and LDL-C of nephrotics was observed to be significantly higher (P0.05. HDL-C of nephrotics was observed to be significantly lower (P<0.05 when compared with control subjects. Conclusion: The result indicates apparent lipid derangement in nephrotic syndrome which may lead to cardiovascular disease. We therefore recommend that full lipid panel should be included in the investigation of suspected nephrotics to complement early diagnosis of the syndrome and to prevent further complications that could arise from the syndrome.

  17. Restless Legs Syndrome in Pediatric Patients With Nephrotic Syndrome

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    Victoria Cheung BA

    2015-05-01

    Full Text Available Background. Restless legs syndrome (RLS is a sleep disorder characterized by an urge to move or the presence of unpleasant sensations in the extremities. The prevalence of RLS is higher in children and adults with chronic kidney disease and in adults with glomerular disease. Objective. To determine the prevalence of RLS in children with nephrotic syndrome. Methods. We studied 50 children with nephrotic syndrome and 22 controls. The following surveys were administered: Pediatric Emory RLS questionnaire, Pediatric Daytime Sleepiness Scale, and Pediatric Sleep Questionnaire. Results. Children with nephrotic syndrome were 9.0 ± 4.4 years old, 27 were male, and 27 were in remission. The prevalence of RLS was similar in the nephrotic syndrome cases and controls, whether or not indeterminate cases were considered positive: 14.0% versus 13.6% including indeterminate cases, and 8.0% versus 9.1% excluding indeterminate cases. Conclusion. RLS is not more common in children with glomerular disease compared to healthy controls.

  18. High Steroid Sensitivity among Children with Nephrotic Syndrome in Southwestern Nigeria

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    Taiwo Augustina Ladapo

    2014-01-01

    Full Text Available Recent reports from both Caucasian and black populations suggest changes in steroid responsiveness of childhood nephrotic syndrome. This study was therefore undertaken to determine the features and steroid sensitivity pattern of a cohort of black children with nephrotic syndrome. Records of children managed for nephrotic syndrome from January 2008 to April 2013 were reviewed. Details including age, response to treatment, and renal histology were analysed. There were 108 children (median age: 5.9 years, peak: 1-2 years, 90.2% of whom had idiopathic nephrotic syndrome. Steroid sensitivity was 82.8% among children with idiopathic nephrotic syndrome but 75.9% overall. Median time to remission was 7 days. Median age was significantly lower in steroid sensitive compared with resistant patients. The predominant histologic finding in resistant cases was focal segmental glomerulosclerosis (53.3%. No cases of quartan malaria nephropathy or hepatitis B virus nephropathy were diagnosed. Overall mortality was 6.5%. In conclusion, unusually high steroid sensitivity is reported among a cohort of black children. This is likely attributable to the lower age structure of our cohort as well as possible changing epidemiology of some other childhood diseases. Surveillance of the epidemiology of childhood nephrotic syndrome and corresponding modifications in practice are therefore recommended.

  19. [Thrombotic complications in the nephrotic syndrome].

    Science.gov (United States)

    Keusch, G

    1989-08-01

    Thromboembolic episodes are one of the most serious complications in patients with nephrotic syndrome, with an overall incidence of 25%. The most frequent site of thrombosis is the renal vein, with a reported incidence varying from 2-42%. Arterial thromboses are much less common than venous thromboses, with an overall incidence of 3%. Clinical course of renal vein thrombosis may be acute or chronic. Renal venography is the method of choice in its diagnosis. Duplex scanning, computed tomography and magnetic resonance imaging may be as accurate as venography. Once the diagnosis of renal vein thrombosis is established, anticoagulation therapy should be started. Thrombectomy or thrombolytic therapy seem to have little to offer over oral anticoagulation. The increased incidence of thrombotic complications in nephrotic syndrome may be due to a hypercoagulable state distinguished by an increase in coagulation factors (V, VIII, X and fibrinogen); a decrease in the levels of coagulation inhibitors (antithrombin III, protein S); an increase in alpha 2-antiplasmin activity; and exaggerated platelet adhesiveness and aggregation. This pre-thrombotic state may be aggravated by additional rheological factors (immobilization, diuretic therapy etc.). Serum albumin has been found to be an appropriate parameter to assess the risk of thrombosis development in these patients. A serum albumin level below 20 g/l carries a high risk of thromboembolic complications. Prophylactic anticoagulation therapy is therefore indicated in patients with serum albumin below 20 g/l.

  20. New insight into the pathogenesis of minimal change nephrotic syndrome: Role of the persistence of respiratory tract virus in immune disorders.

    Science.gov (United States)

    Zhang, Hui; Wang, Zheng; Dong, Liqun; Guo, Yannan; Wu, Jin; Zhai, Songhui

    2016-07-01

    The pathogenesis of minimal change nephrotic syndrome (MCNS) is a complex clinical problem which, unfortunately, has been in need of significant breakthroughs for decades. Improved understanding of the mechanisms is important to develop effective treatment strategies. To our knowledge, the pathogenesis of MCNS is multifactorial, involving both intrinsic and extrinsic factors, reasonable to be regarded as a "long chain" cascade reaction. Current studies implicating that the disease could probably be caused by immune disorders, however, have focused merely on the middle or terminal of this "long chain". It remains unclear what really triggers the immune disorders. It is noteworthy that the close association of respiratory tract infection with the occurrence, relapse and aggravation of nephrotic syndrome has been confirmed for over two decades. Derived from what we demonstrated in earlier studies, that the persistence of respiratory tract virus may contribute to the onset and development of MCNS, this review summarizes current evidence investigating the possible mechanisms of viral persistence, and discusses the role of viral persistence in the pathogenesis of MCNS. The key point is: whether the persistence of respiratory tract virus results in immune disorders. The available evidence under review also highlight the fact that the background of genetic susceptibility to the disease was found in many patients, which could be triggered by extrinsic factors, e.g. by the infection of respiratory tract virus.

  1. New insights into lipid metabolism in the nephrotic syndrome

    NARCIS (Netherlands)

    Kaysen, GA; de Sain-van der Velden, MGM

    1999-01-01

    Hyperlipidemia in the nephrotic syndrome results from increased synthesis and decreased catabolism of lipoproteins. The contribution of each to establishing blood lipid levels is unknown. Increased triglyceride rich lipoprotein concentration, very low density lipoprotein (VLDL) and intermediate dens

  2. A PROSPECTIVE STUDY OF NEPHROTIC SYNDROME IN CHILDREN

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    Vijayalakshmi

    2015-05-01

    Full Text Available AIM OF THE STUDY: To study the incidence, age and sex related demographics in children with Nephrotic Syndrome (NS and to find the aetiology in atypical cases of NS by renal biopsy. MATERIALS AND METHOD S: The present study was a single centre, descriptive, prospective and observational cross sectional study. All patients who were admitted in the department of paediatrics with Nephrotic syndrome during the study period of 2 years from July 2010 to June 2012 were included. The demographics and renal biopsy results were analys ed further. RESULTS : Total number of cases of NS was 44 against total Paediatric admissions of 4827. Incidence of NS was 0.91% (44/4827 among all paediatric admissions. Male to female ratio was 2.14:1. Majority of NS cases were 3 - 5 years old (36.36%, n=44 , followed by 5 - 7 years (29.55%, n=44, 7 - 9 years (18.18%, n=44 and 1 - 3 years (15.91%, n=44. Total number of renal biopsies done was 18, out of which 14 (77.78%, n=18 had minimal change disease (MCNS and 3 (16.67%, n=18 had Focal Segmental Glomerulos clerosis (FSGS and 1 (5.55%, n=18 had anti Glomerular Basement Membrane disease. CONCLUSION: The incidence of Nephrotic syndrome in children was 0.91% (44/4827 among total paediatric admissions, had male preponderance with ratio of males to females of 2 .14:1, most commonly affecting children of 3 - 5 years of age and the most common aetiology being Minimal Change Disease (MCD.

  3. 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.

  4. Secondary Syphilis With Hepatitis and Nephrotic Syndrome: A Rare Concurrence

    OpenAIRE

    Makker, Jasbir; Bajantri, Bharat; Nayudu, Suresh Kumar

    2016-01-01

    Syphilis, a chronic multisystem disease, is caused by a spirochete, Treponema pallidum. Clinical presentation may expand to several stages including primary, secondary and latent syphilis, which may present as early or late syphilis. Nephrotic syndrome and acute hepatitis are well-known complications of secondary syphilis. To the best of our knowledge, secondary syphilis with coexisting renal and hepatic complications has rarely been reported. Here we present a rare case of concurrent nephrot...

  5. Nephrotic syndrome in hand, foot and mouth disease caused by coxsackievirus A16: a case report

    OpenAIRE

    Hong-Tao Zhou; Bing Wang; Xiao-Yan Che

    2014-01-01

    Some viruses, including certain members of the enterovirus genus, have been reported to cause nephrotic syndrome. However, no case of coxsackievirus A16 (CVA16)-related nephrotic syndrome has been reported so far. We describe a case of CVA16-related hand, foot and mouth disease presenting with nephrotic syndrome in a 3-year-old boy. This is the first report of CVA16-related nephrotic syndrome.

  6. Nephrotic syndrome and Guillan-Barré Syndrome: a rare association in child.

    Science.gov (United States)

    Bouyahia, Olfa; Khelifi, Ibtissem; Gharsallah, Lamia; Harzallah, Kais; Mrad, Sonia Mazigh; Ghargah, Tahar; Boukthir, Samir; Azza, Sammoud El Gharbi

    2010-01-01

    Only few cases of nephrotic syndrome associated with Guillain-Barre Syndrome (GBS) have been reported in the adult and pediatric literature. A 3-year-old boy was initially admitted to our hospital following five days of progressive weakness of his extremities, fatigue, right leg pain and numbness. There was no past history of renal or neurological disease. Cerebro-spinal fluid studies showed a protein level of 92 mg/dL and a white cell count of 1 per high-power field. The diagnosis of GBS was verified with a nerve conduction velocity test as well as. The GBS symptoms improved gradually on intravenous immunoglobulin. Three weeks later, he developed severe proteinuria and edema; laboratory investigation showed nephrotic syndrome which responded to steroid therapy. Renal biopsy showed minimal change glomerulonephritis. He remained free of proteinuria during his 20 months of follow-up.

  7. Nephrotic syndrome and Guillan-barre syndrome: A rare association in child

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    Bouyahia Olfa

    2010-01-01

    Full Text Available Only few cases of nephrotic syndrome associated with Guillain-Barre Syndrome (GBS have been reported in the adult and pediatric literature. A 3-year-old boy was initially admitted to our hospital following five days of progressive weakness of his extremities, fatigue, right leg pain and numbness. There was no past history of renal or neurological disease. Cerebro-spinal fluid studies showed a protein level of 92 mg/dL and a white cell count of 1 per high-power field. The diagnosis of GBS was verified with a nerve conduction velocity test as well as. The GBS symptoms improved gradually on intravenous immunoglobulin. Three weeks later, he deve-loped severe proteinuria and edema; laboratory investigation showed nephrotic syndrome which responded to steroid therapy. Renal biopsy showed minimal change glomerulonephritis. He re-mained free of proteinuria during his 20 months of follow-up.

  8. Splenic hypofunction in the nephrotic syndrome of childhood

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    McVicar, M.I.; Chandra, M.; Margouleff, D.; Zanzi, I.

    1986-05-01

    The reticuloendothelial system, including the spleen, subserves important immunologic functions. Loss of splenic function results in an increased incidence of severe bacterial infections and is accompanied by thrombocytosis. Several nephrotic children were noted to have remarkably high platelet counts and predisposition to bacterial infection with encapsulated organisms. We, therefore, investigated the splenic function of nine children with primary nephrotic syndrome and measured the phagocytic function of the spleen by sequestration of Technetium-99-labelled heat-treated autologous RBC, administered intravenously. Four children had decreased splenic function. Repeat studies performed in two of these children after remission of the nephrotic syndrome gave normal results. There were six episodes of bacterial infection (3 peritonitis, 1 septic arthritis, 1 cellulitis, and 1 Escherichia coli urinary tract infection) among the four patients with decreased splenic function. There were no episodes of bacterial infection among the five nephrotic children with normal splenic function. Nephrotic patients with decreased splenic function had significantly increased platelet counts (921,000 +/- 196,000; mean +/- SEM) compared to those with normal function (435,000 +/- 46,000; P less than 0.001). Our findings suggest the possibility that some nephrotic children may have decreased splenic function in association with increased susceptibility to bacterial infections.

  9. Long-term Outcomes of Childhood Onset Nephrotic Syndrome

    Science.gov (United States)

    Hjorten, Rebecca; Anwar, Zohra; Reidy, Kimberly Jean

    2016-01-01

    There are limited studies on long-term outcomes of childhood onset nephrotic syndrome (NS). A majority of children with NS have steroid-sensitive nephrotic syndrome (SSNS). Steroid-resistant nephrotic syndrome (SRNS) is associated with a high risk of developing end-stage renal disease. Biomarkers and analysis of genetic mutations may provide new information for prognosis in SRNS. Frequently relapsing and steroid-dependent NS is associated with long-term complications, including dyslipidemia, cataracts, osteoporosis and fractures, obesity, impaired growth, and infertility. Long-term complications of SSNS are likely to be under-recognized. There remain many gaps in our knowledge of long-term outcomes of childhood NS, and further study is indicated. PMID:27252935

  10. Fulminant intracranial hypertension due to cryptococcal meningitis in a child with nephrotic syndrome

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    Leena Nagotkar

    2011-01-01

    Full Text Available Cryptococcus neoformans has a worldwide distribution. Meningoencephalitis is the most common manifestation of cryptococcosis. The outcome of a patient with cryptococcal infection depends on the immune status of the host. Patients with nephrotic syndrome are particularly susceptible to cryptococcal infection not only due to innate changes in their immune system but also because of the immunosuppressive agents used in the treatment. We report an 8-year-old boy with nephrotic syndrome, who developed cryptococcal meningitis and died of fulminant intracranial hypertension.

  11. CLINICAL, BIOCHEMICAL AND HISTOPATHOLOGICAL PROFILE OF ADULT NEPHROTIC SYNDROME PATIENTS IN A TERTIARY CARE HOSPITAL

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    Krishnamoorthy

    2015-09-01

    Full Text Available BACKGROUND: The nephrotic syndrome is recognized as an independent entity of renal disease for over half a century . 1 Causes of nephrotic syndrome varies with age, time period, geographical location and race. In children, minimal change nephrotic syndrome is the commonest 2 ; however, membranous nephropathy is most frequent in adults . 3 As it commonly affects the younger age group and is associated with high morbidity and mortality, there is a need to understand and diagnose the disease at an early stage. Hence, this study has been done to identify the clinical presentation, biochemical parameters and histopathology associated with nephrotic syndrome in adults and its subtypes. OBJECTIVE: To study the clinical, biochemical and histopathological profile of patients with Adult Nephrotic Syndrome admitted in our tertiary care hospital. METHODS: Prospective study of 100 patients with Adult Nephrotic Syndrome admitt ed in our tertiary care hospital were screened with facial puffiness and pedal edema. They were tested for urine proteinuria, urine protein creatinine ratio or 24 hour urine protein estimation. Later renal biopsy was done for all patients to stratify the subtypes. RESULTS: In this study, males were predominantly affected. Most common presenting complaints were facial puffiness and pedal edema. Systolic BP was increased in 96% of patients and diastolic BP was elevated in 50% of patients. Serum LDL and TGL were elevated in nephrotic syndrome. In young patients less than 40 years Focal Segmental Glomerulosclerosis (FSGS is the commonest type, then Membrano Proliferative Glomerulo Nephritis (MPGN and Minimal Change Disease (MCD. In individuals more than 40 years, membranous nephropathy was predominantly seen followed by FSGS. CONCLUSION: There is a changing trend in primary nephrotic syndrome and FSGS was found to be the commonest subtype. Male preponderance was noticed and also FSGS was found to be more common in younger adults. Most

  12. Secondary Syphilis With Hepatitis and Nephrotic Syndrome: A Rare Concurrence.

    Science.gov (United States)

    Makker, Jasbir; Bajantri, Bharat; Nayudu, Suresh Kumar

    2016-07-01

    Syphilis, a chronic multisystem disease, is caused by a spirochete, Treponema pallidum. Clinical presentation may expand to several stages including primary, secondary and latent syphilis, which may present as early or late syphilis. Nephrotic syndrome and acute hepatitis are well-known complications of secondary syphilis. To the best of our knowledge, secondary syphilis with coexisting renal and hepatic complications has rarely been reported. Here we present a rare case of concurrent nephrotic syndrome and acute hepatitis in a patient with secondary syphilis. PMID:27298666

  13. Glucocorticoid Treatment in Childhood Nephrotic Syndrome : weighting the cornerstone

    NARCIS (Netherlands)

    N. Teeninga (Nynke)

    2013-01-01

    textabstractUnderstanding which factors influence relapse patterns in childhood nephrotic syndrome is clinically very relevant and could aid in developing new treatment strategies. Clinicians are continuously challenged to reduce relapse rates and at the same time to avoid glucocorticoid toxicity. B

  14. A CASE REPORT ON SICKLE CELL DISEASE WITH HEMOLYTIC ANEMIA, NEPHROTIC SYNDROME AND ACUTE CHEST SYNDROME

    OpenAIRE

    Putta; Yamini Devi

    2015-01-01

    Sickle cell disease is an autoimmune hemolytic anemia due to abnormal hemoglobin. Sickling of RBCs occur due to abnormal hemoglobin which leads to vaso - occlusive crisis. This disease manifests as hemolytic anemia, acute chest syndrome, stroke, ischemic leg ulcers and nephrotic syndrome. This patient presented with hemolytic anemia, nephrotic syndrome and acute chest syndrome. This case was diagnosed by electrophoresis of h emoglobin and peripheral smear. Thi...

  15. Paediatric T cell Lymphoma with Nephrotic Syndrome: A Rare Association

    OpenAIRE

    Joseph, Deepa; Biswajit, Dubashi; Ganesh, Rajesh Nachiappa; Parameswaran, Sreejith; Jain, Ankit

    2012-01-01

    Renal involvement is frequent in hematologic malignancies especially Hodgkin’s lymphoma. Renal complications in children with malignancies primarily arise from tumour lysis syndrome, malignant infiltration or obstruction of the urinary tract, deposits of immunoglobulin fractions or crystals, renal infiltration by malignant cells, paraneoplastic or storage glomerulopathies. Nephrotic syndrome has been described in B cell type Non Hodgkin’s lymphomas. There are very few reports of association o...

  16. An Asymptomatic Young Female with Chronic Hepatitis-B Presenting as Minimal Change Glomerulonephritis with Nephrotic Syndrome

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    A.K.Gupta ,A.Rohatgi, V.Pardasani, S.K.Sharma, A.k. Dinda, P.Shakhuja

    2003-10-01

    Full Text Available Hepatitis B is a worldwide problem with high prevalence rate in our country. Kidney involvement iscommon in chronic hepatitis B patients. The usual pattern is membranous glomerulonephritis inchildren and Type I membranoproliferative glomerulonephritis in adults. A previously asymptomaticyoung female suffering from chronic hepatitis B with portal hypertension presented with nephroticsyndrome. Kidney biopsy revealed minimal change glomerulonephritis which is rarely seen inassociation with hepatitis B. Patient recovered following administration of oral steroids. Wereconunend screening ofall patients ofnephrotic syndrome for chronic hepatitis B with viral markersin addition to HBsAg.

  17. Steroid-sensitive mechanism of soluble immune response suppressor production in steroid-responsive nephrotic syndrome.

    OpenAIRE

    Schnaper, H W; Aune, T M

    1987-01-01

    Soluble immune response suppressor (SIRS), a lymphokine that suppresses antibody production and delayed type hypersensitivity in vivo, has been detected in urine and serum from certain patients with nephrotic syndrome. In the present paper, the relationship between SIRS production and nephrotic syndrome is further characterized. A striking correlation was found between detection of SIRS and the presence of steroid-responsive nephrotic syndrome (SRNS). A potential mechanism of SIRS production ...

  18. Adult nephrotic syndrome: non-specific strategies for treatment.

    Science.gov (United States)

    Charlesworth, John A; Gracey, David M; Pussell, Bruce A

    2008-02-01

    Irrespective of aetiology, the nephrotic syndrome presents a range of potentially serious complications. These include thrombo-embolism, infection and hyperlipidaemia. Despite the prevalence of the nephrotic state among renal patients, there has been little prospective analysis of the therapeutic approach to these potentially life-threatening events even though their pathogenesis has been examined in some detail. Most of these complications are more prevalent once the albumin concentration falls below 20 g/L and it is recognized that restoration of serum albumin significantly diminishes their frequency. However, this may be difficult to achieve, especially in adults. The problems of thrombo-embolism and infection are of immediate concern but, in persistent cases, the additional issues of hyperlipidaemia and loss of bone density also require consideration for therapy. Thus, in addition to specific attempts to reduce proteinuria, it is recommended that high-risk nephrotic patients receive anticoagulation, pneumococcal vaccination and lipid lowering therapy. Strategies for the preservation of bone density should also be considered, particularly in patients who receive high-dose corticosteroids. Among a range of non-specific treatments for proteinuria, angiotensin-converting enzyme inhibitors appear best in terms of efficacy and safety. Prospective trials are required to clarify the longitudinal impact of these generic strategies on the protection of the persistently nephrotic patient.

  19. Molecular and Genetic Basis of Inherited Nephrotic Syndrome

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    Maddalena Gigante

    2011-01-01

    Full Text Available Nephrotic syndrome is an heterogeneous disease characterized by increased permeability of the glomerular filtration barrier for macromolecules. Podocytes, the visceral epithelial cells of glomerulus, play critical role in ultrafiltration of plasma and are involved in a wide number of inherited and acquired glomerular diseases. The identification of mutations in nephrin and other podocyte genes as causes of genetic forms of nephrotic syndrome has revealed new important aspects of the pathogenesis of proteinuric kidney diseases and expanded our knowledge of the glomerular biology. Moreover, a novel concept of a highly dynamic slit diaphragm proteins is emerging. The most significant discoveries in our understanding of the structure and function of the glomerular filtration barrier are reviewed in this paper.

  20. [Thrombosis and disorders of hemostasis in nephrotic syndrome].

    Science.gov (United States)

    Kanfer, A

    1992-01-01

    Thromboses and disorders of hemostasis in nephrotic syndrome. Thromboembolic complications are common in nephrotic syndrome (NS). This article reviews the factors of thrombogenesis in NS, including: 1) a hypercoagulable state with platelet hyperaggregability, hyperfibrinogenemia and elevated factor VIII, decrease in plasma levels of coagulation inhibitors antithrombin III and free protein S, reduced fibrinolytic activity; 2) excessive intravascular thrombin formation marked by increased plasma levels of fibrinopeptide A. The intensity of hemostasis disorders coincides with that of NS. Most disorders are related to hypoalbuminemia and proteinuria. In agreement with experimental data, the role of intraglomerular activation of coagulation during active phases of glomerulopathies has to be considered. This could explain the predominance of renal vein thrombosis in several glomerulopathies with NS. Several coagulation disorders in SN have implications for therapy.

  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. Defining nephrotic syndrome from an integrative genomics perspective

    OpenAIRE

    Sampson, Matthew G.; Hodgin, Jeffrey B.; Kretzler, Matthias

    2014-01-01

    Nephrotic syndrome (NS) is a clinical condition with a high degree of morbidity and mortality, caused by failure of the glomerular filtration barrier, resulting in massive proteinuria. Our current diagnostic, prognostic and therapeutic decisions in NS are largely based upon clinical or histological patterns such as “focal segmental glomerulosclerosis” or “steroid sensitive”. Yet these descriptive classifications lack the precision to explain the physiologic origins and clinical heterogeneity ...

  3. Bioimpedance for assessing volume status in children with nephrotic syndrome

    OpenAIRE

    ÖZDEMİR, KADRİYE; MİR, MAKBULE SEVGİ; DİNÇEL, NİDA; BOZABALI, SİBEL; BULUT, İPEK KAPLAN; YILMAZ, Ebru; SÖZERİ, BETÜL

    2015-01-01

    Background/aim: The effectiveness of assessing volume load via bioimpedance in pediatric patients with nephrotic syndrome (NS) was investigated. Materials and methods: Patients with NS (n = 34) were compared with healthy controls (n = 20). The inferior vena cava index and inferior vena cava collapsibility index (IVCCI) scores were determined for all subjects. Bioimpedance measurements were used to directly determine volume load. Clinical findings, relative fluid load, and echocardiographic m...

  4. Spontaneous Remission of Nephrotic Syndrome in Idiopathic Membranous Nephropathy

    Science.gov (United States)

    Polanco, Natalia; Gutiérrez, Elena; Covarsí, Adelardo; Ariza, Francisco; Carreño, Agustín; Vigil, Ana; Baltar, José; Fernández-Fresnedo, Gema; Martín, Carmen; Pons, Salvador; Lorenzo, Dolores; Bernis, Carmen; Arrizabalaga, Pilar; Fernández-Juárez, Gema; Barrio, Vicente; Sierra, Milagros; Castellanos, Ines; Espinosa, Mario; Rivera, Francisco; Oliet, Aniana; Fernández-Vega, Francisco

    2010-01-01

    Spontaneous remission is a well known characteristic of idiopathic membranous nephropathy, but contemporary studies describing predictors of remission and long-term outcomes are lacking. We conducted a retrospective, multicenter cohort study of 328 patients with nephrotic syndrome resulting from idiopathic membranous nephropathy that initially received conservative therapy. Spontaneous remission occurred in 104 (32%) patients: proteinuria progressively declined after diagnosis until remission of disease at 14.7 ± 11.4 months. Although spontaneous remission was more frequent with lower levels of baseline proteinuria, it also frequently occurred in patients with massive proteinuria: 26% among those with baseline proteinuria 8 to 12 g/24 h and 22% among those with proteinuria >12 g/24 h. Baseline serum creatinine and proteinuria, treatment with angiotensin-converting enzyme inhibitors or angiotensin receptor antagonists, and a >50% decline of proteinuria from baseline during the first year of follow-up were significant independent predictors for spontaneous remission. Only six patients (5.7%) experienced a relapse of nephrotic syndrome. The incidence of death and ESRD were significantly lower among patients with spontaneous remission. In conclusion, spontaneous remission is common among patients with nephrotic syndrome resulting from membranous nephropathy and carries a favorable long-term outcome with a low incidence of relapse. A decrease in proteinuria >50% from baseline during the first year predicts spontaneous remission. PMID:20110379

  5. Lupus podocytopathy: An important differential diagnosis of nephrotic syndrome in systemic lupus erythematosus.

    Science.gov (United States)

    Chaudhury, A R; Rajarajan, T; Yousuf, R; Fernando, E; Kurien, A A

    2016-01-01

    Some patients with systemic lupus erythematosus (SLE) present with sudden onset of nephrotic syndrome and biopsy findings may be of minimal change disease or focal segmental glomerulosclerosis with diffuse foot process effacement on electron microscopy but without significant immune deposits. This entity is termed lupus podocytopathy. Clinicians and renal pathologists need to be aware of this condition. Though steroid sensitive, it needs follow-up to recognize flare and class change, thereby optimizing therapy. PMID:27512302

  6. Plasma Histamine And Serotonin Levels In Children With Nephrotic Syndrome And Acute Poststreptococcal Glomerulonephritis

    Directory of Open Access Journals (Sweden)

    Nagwa Mohamed and Talaat El sayed

    2005-12-01

    Full Text Available Plasma histamine and serotonin concentrations were measured using fluorimeteric assay in 40 children with renal diseases. Minimal change nephrotic syndrome (15 focal segmental glomerulosclerosis(10 and acute poststreptococcal glomerulonephritis(15 to determine the relation between plasma levels of histamine and serotonin and these various types of renal diseases in children. Plasma histamine level was significantly increased in group of children with acute poststreptococcal glomerulonephritis. Plasma serotonin levels were significantly increased in all 3 groups of patient, when compared with those of controls. Raised plasma histamine in acute poststreptococcal glomerulonephritis group may be evidence of the acute immunological inflammation and defective renal excretion due to mild renal impairment in these children. Raised plasma serotonin in all 3 groups of patients may be due to diminished uptake and release of serotonin from platelets in children with minimal change nephrotic syndrome and focal segmental glomerulosclerosis and due to defective renal execretion in children with acute poststreptococcal glomerulo-nephritis.

  7. Budd-Chiari syndrome during nephrotic relapse in a patient with resistance to activated protein C clotting inhibitor.

    Science.gov (United States)

    Gambaro, G; Patrassi, G; Pittarello, F; Nardellotto, A; Checchetto, S; D'Angelo, A

    1998-10-01

    It has long been known that patients with nephrotic syndrome have a hypercoagulable state, which explains the association between nephrotic syndrome, renal vein thrombosis, and thromboembolism. However, the Budd-Chiari syndrome has never been reported in nephrotic patients. This is the first report of such an association that, most likely, depended on a primary resistance to activated protein C.

  8. Serum D-dimer concentrations in nephrotic syndrome track with albuminuria, not estimated glomerular filtration rate.

    LENUS (Irish Health Repository)

    Sexton, D J

    2012-01-01

    The nephrotic syndrome is associated with an increased risk of venous and arterial thrombosis. There are little published data on the distribution, interpretation or determinants of serum D-dimer levels in patients with the nephrotic syndrome. We aimed to describe this relationship.

  9. Copper and zinc metabolism in aminonucleoside-induced nephrotic syndrome.

    Science.gov (United States)

    Pedraza-Chaverrí, J; Torres-Rodríguez, G A; Cruz, C; Mainero, A; Tapia, E; Ibarra-Rubio, M E; Silencio, J L

    1994-01-01

    Copper (Cu) and zinc (Zn) were measured in urine, serum and tissues from rats with nephrotic syndrome (NS) induced with a single subcutaneous dose of puromycin aminonucleoside (PAN; 15 mg/100 g BW). Control animals were pair-fed. Urine was collected daily, and the rats were sacrificed on day 10. PAN-nephrotic rats had proteinuria (days 3-10), high urinary Cu (days 1, 2, 4-10) and Zn (days 3-10) excretion. On day 10, nephrotic rats had: (a) albuminuria, hypoalbuminemia, hypoproteinemia, high urine and low serum levels of ceruloplasmin; (b) low Cu and Zn serum levels; (c) high clearance and fractional excretion of Cu and Zn, and (d) low kidney and liver Cu content and essentially normal tissue Zn levels. The alterations in Cu metabolism were more intense than those in Zn metabolism. Urine Cu and Zn showed a positive correlation with urine total protein on days 3-10 which suggests that high urinary excretion of Cu and Zn may be due to the excretion of its carrier proteins. In conclusion, these rats did not show a typical Zn deficiency but a clear decrease in Cu in the liver and kidney.

  10. Risk of Nephrotic Syndrome following Enteroviral Infection in Children: A Nationwide Retrospective Cohort Study

    Science.gov (United States)

    Lin, Jiun-Nong; Lin, Cheng-Li; Yang, Chi-Hui; Lin, Ming-Chia; Lai, Chung-Hsu; Lin, Hsi-Hsun; Kao, Chia-Hung

    2016-01-01

    Purpose Nephrotic syndrome is a common chronic illness encountered during childhood. Infections have been identified as a cause of nephrotic syndrome. The aim of this study was to evaluate the association between enteroviral infection and nephrotic syndrome. Methods A nationwide retrospective cohort study was conducted by analyzing data from the National Health Insurance Research Database in Taiwan. Children aged enterovirus-infected children were randomly selected as the comparison cohort. The primary endpoint was the occurrence of nephrotic syndrome. Methods This study included 280,087 enterovirus-infected children and 280,085 non-enterovirus-infected children. The mean age of the enterovirus-infected children was 2.38 years, and 53.7% of these children were boys. The overall incidence densities of nephrotic syndrome for enterovirus- and non-enterovirus-infected children were 2.65 and 2.21 per 10,000 person-years, respectively. The enterovirus-infected cohort had a higher cumulative incidence of nephrotic syndrome than did the non-enterovirus-infected cohort (log-rank test, p = 0.01). Multivariable analyses revealed that children with enteroviral infection were significantly associated with an increased risk of nephrotic syndrome compared with those without enteroviral infection (adjusted hazard ratio, 1.20; 95% confidence interval, 1.04–1.39; p = 0.01), particularly in children infected with coxsackievirus. Subgroup analyses revealed that enterovirus-infected girls, children of blue-collar workers, and children without allergies had a higher risk of nephrotic syndrome than did children in the non-enterovirus-infected cohort. Conclusion This study revealed a significant association between enteroviral infection and nephrotic syndrome. Additional studies elucidating the role and pathogenesis of enterovirus in nephrotic syndrome are warranted. PMID:27508414

  11. 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.

  12. Chronic graft versus host disease and nephrotic syndrome

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    Samia Barbouch

    2014-01-01

    Full Text Available Disturbed kidney function is a common complication after bone marrow transplantation. Recently, attention has been given to immune-mediated glomerular damage related to graft versus host disease (GVHD. We describe a 19-year-old woman who developed membranous glomerulonephritis after bone marrow transplantation (BMT. Six months later, she developed soft palate, skin and liver lesions considered to be chronic GVHD. Fifteen months after undergoing BMT, this patient presented with nephrotic syndrome. A renal biopsy showed mem-branous glomerulonephritis associated with a focal segmental glomerulosclerosis. She was started on corticosteroid treatment with good outcome.

  13. Chronic graft versus host disease and nephrotic syndrome.

    Science.gov (United States)

    Barbouch, Samia; Gaied, Hanene; Abdelghani, Khaoula Ben; Goucha, Rim; Lakhal, Amel; Torjemen, Lamia; Hamida, Fethi Ben; Abderrahim, Ezzedine; Maiz, Hedi Ben; Adel, Khedher

    2014-09-01

    Disturbed kidney function is a common complication after bone marrow transplantation. Recently, attention has been given to immune-mediated glomerular damage related to graft versus host disease (GVHD). We describe a 19-year-old woman who developed membranous glomerulonephritis after bone marrow transplantation (BMT). Six months later, she developed soft palate, skin and liver lesions considered to be chronic GVHD. Fifteen months after undergoing BMT, this patient presented with nephrotic syndrome. A renal biopsy showed membranous glomerulonephritis associated with a focal segmental glomerulosclerosis. She was started on corticosteroid treatment with good outcome. PMID:25193909

  14. History of Nephrotic Syndrome and Evolution of its Treatment

    Science.gov (United States)

    Pal, Abhijeet; Kaskel, Frederick

    2016-01-01

    The recognition, evaluation, and early treatment of nephrotic syndrome in infants and children originate from physicians dating back to Hippocrates. It took nearly another 1000 years before the condition was described for its massive edema requiring treatment with herbs and other remedies. A rich history of observations and interpretations followed over the course of centuries until the recognition of the combination of clinical findings of foamy urine and swelling of the body, and measurements of urinary protein and blood analyses showed the phenotypic characteristics of the syndrome that were eventually linked to the early anatomic descriptions from first kidney autopsies and then renal biopsy analyses. Coincident with these findings were a series of treatment modalities involving the use of natural compounds to a host of immunosuppressive agents that are applied today. With the advent of molecular and precision medicine, the field is poised to make major advances in our understanding and effective treatment of nephrotic syndrome and prevent its long-term sequelae. PMID:27303658

  15. History of Nephrotic Syndrome and Evolution of its Treatment

    Directory of Open Access Journals (Sweden)

    Abhijeet ePal

    2016-05-01

    Full Text Available The recognition, evaluation, and early treatment of nephrotic syndrome in infants and children originates from physicians dating back to Hippocrates. It took nearly another thousand years before the condition was described for its massive edema requiring treatment with herbs and other remedies. A rich history of observations and interpretations followed over the course of centuries until the recognition of the combination of clinical findings of foamy urine, swelling of the body, and measurements of urinary protein and blood analyses showed the phenotypic characteristics of the syndrome that were eventually linked to the early anatomic descriptions from first kidney autopsies and then renal biopsy analyses. Coincident with these findings were a series of treatment modalities involving the use of natural compounds to a host of immunosuppressive agents that are applied today. With the advent of molecular and precision medicine, the field is poised to make major advances in our understanding and effective treatment of nephrotic syndrome and prevent its long-term sequelae.

  16. History of Nephrotic Syndrome and Evolution of its Treatment.

    Science.gov (United States)

    Pal, Abhijeet; Kaskel, Frederick

    2016-01-01

    The recognition, evaluation, and early treatment of nephrotic syndrome in infants and children originate from physicians dating back to Hippocrates. It took nearly another 1000 years before the condition was described for its massive edema requiring treatment with herbs and other remedies. A rich history of observations and interpretations followed over the course of centuries until the recognition of the combination of clinical findings of foamy urine and swelling of the body, and measurements of urinary protein and blood analyses showed the phenotypic characteristics of the syndrome that were eventually linked to the early anatomic descriptions from first kidney autopsies and then renal biopsy analyses. Coincident with these findings were a series of treatment modalities involving the use of natural compounds to a host of immunosuppressive agents that are applied today. With the advent of molecular and precision medicine, the field is poised to make major advances in our understanding and effective treatment of nephrotic syndrome and prevent its long-term sequelae. PMID:27303658

  17. Neural deficits, intelligence, maladjustment and family response in children with the nephrotic syndrome.

    Science.gov (United States)

    Naidoo, L R; Moodley, T Y; Coovadia, H M; Adhikari, M

    1987-01-10

    An intensive psychomedical study of a small number of children suffering from the nephrotic syndrome is reported. Significant organic and psychological sequelae exist in the nephrotic child. The patient's family also appears to need specialist psychological attention. The urgent need for well-organised liaison psychiatric services in non-psychiatric medical departments is emphasised. PMID:2432669

  18. [Hodgkin disease revealed by a nephrotic syndrome: A case report].

    Science.gov (United States)

    Cheptou, M; Pichault, V; Campagni, R; Vodoff, M-V; Fischbach, M; Paillard, C

    2015-12-01

    Pediatric nephrotic syndrome (NS) is most often idiopathic or primary but in rare cases, it can be secondary to neoplasia. We report on a case of steroid-resistant NS revealing as a paraneoplastic syndrome of Hodgkin disease (HD) in a 12-year-old boy. The onset of the NS can be earlier, later, or simultaneous to the HD. Treatment of the lymphoma allows the disappearance of the NS. In the case we observed, the diagnosis of HD was delayed because HD presented with an isolated, hilar adenopathy in the absence of retroperitoneal or peripheral locations. In children aged 10 years or more presenting with NS, steroid-resistant or otherwise, a possible paraneoplastic origin such as Hodgkin lymphoma should always be taken into consideration and eventually eliminated. PMID:26598043

  19. A CASE REPORT ON SICKLE CELL DISEASE WITH HEMOLYTIC ANEMIA, NEPHROTIC SYNDROME AND ACUTE CHEST SYNDROME

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    Putta

    2015-03-01

    Full Text Available Sickle cell disease is an autoimmune hemolytic anemia due to abnormal hemoglobin. Sickling of RBCs occur due to abnormal hemoglobin which leads to vaso - occlusive crisis. This disease manifests as hemolytic anemia, acute chest syndrome, stroke, ischemic leg ulcers and nephrotic syndrome. This patient presented with hemolytic anemia, nephrotic syndrome and acute chest syndrome. This case was diagnosed by electrophoresis of h emoglobin and peripheral smear. This patient recovered with blood transfusion, antibiotics, steroids, diuretics and oxygen inhalation. Sickle cell patients have a known predisposition to bacterial infection, particularly pneumococcal infection. The most si gnificant advance in the therapy of sickle cell anemia has been the introduction of hydroxyurea, but hydroxyurea should be considered in patients experiencing repeated episodes of acute chest syndrome. But in this patient as this is first episode, hydroxyu rea was not given and he recovered well.

  20. A rare association of Castleman′s disease and nephrotic syndrome

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    I Tazi

    2011-01-01

    Full Text Available Castleman′s Disease (CD is an uncommon and poorly understood disorder of lymph node hyperplasia of unknown etiology. This entity belongs to the atypical lymphoproliferative disorders, a heterogeneous group of diseases characterized by a hyperplastic reactive process involving the immune system. The association of the nephrotic syndrome and CD is extremely rare and their interrelation remains enigmatic. We report a case of CD of the hyaline-vascular type with unicentric localization complicated by nephrotic syndrome.

  1. Invasive Bladder Cancer after Cyclophosphamide Administration for Nephrotic Syndrome : A Case Report

    OpenAIRE

    Nakamoto, Takahisa; Kasaoka, Yoshinobu; Ikegami, Yoshihiko; Usui, Tsuguru

    2000-01-01

    We report a case of invasive bladder cancer after cyclophosphamide administration for nephrotic syndrome, and briefly discuss the association of bladder cancer and cyclophosphamide.  A 6-year-old boy, who was diagnosed as having nephrotic syndrome, was treated with oral administration of prednisolone and cyclophosphamide for 4 years, receiving a total dose of 49.5 g cyclophosphamide. At age 27, a gross hematuria with bloody clots appeared and he presented with postrenal renal failure. He unde...

  2. Expression of Interleukin- 13 inPeripheral Blood Mononuclear Cells from Patients with Nephrotic Syndrome

    Institute of Scientific and Technical Information of China (English)

    2000-01-01

    Objective Nephrotic syndrome (NS) is a kind of renal diseases characterized mainly by proteinuria and hypoalbuminemia. The disturbance of cellular immunity plays a major role in the pathogenesis of NS. The change of interleukin (IL)-13 produced by Th2 cells was investigated in 50 children with NS. Methods All the patients were selected during their nephrotic stage and remission stage. The expression of IL-13 protein and mRNA was determined by ELISA and reverse transcription-polymerase chain reaction ( RT-PCR ) respectively. Results ①The production of IL-13 inphytoheamagglutinin (PHA)- stimulated monouuclear cells and the expression of IL-13 mRNA were siguificantly increased in patients in nephrotic stage as compared with controls. ②The expression of IL13 protein and mRNA in patients in remission stage was similar to that in the normal controls. ③Th_ere was no correlation between IL-13 and laboratory parameters. Conclusion The results suggest that the disturbance of cellulr immunity plays an important role in the pathogenesis of NS and they also demonstrate a protective role for IL-13 in the children uith NS.

  3. Treatment of steroid resistant nephrotic syndrome in children

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    Kari Jameela

    2010-01-01

    Full Text Available Achieving remission in children with Steroid-Resistant Nephrotic Syndrome (SRNS could be difficult. Many immunosuppressive drugs are used with variable success rates. We have studied the response of children with SRNS who presented to our pediatric′s renal unit between 2002 and 2007 to various modalities of therapy. We included patients with no response to pred-nisolone (60 mg/M 2 /day after four weeks of therapy; all the patients had renal biopsy and follow-up duration for at least one year. We excluded patients with congenital nephrotic syndrome, lupus, or sickle cell disease. There were 31 (23 girls and 8 boys with F: M= 2.9:1; the mean age at presentation was 4.2 ± 3.2 children who fulfilled the inclusion criteria. The mean duration of follow up was 3.1 ± 1.6 years. Twenty children (65% achieved partial (6 children or complete (14 children remission. There were 16 children treated with cyclophosphamide either oral or intra-venous, and only 4 of them (25% achieved remission. Seven children received oral chlorambucil, and only2 of them (28.5% achieved remission; none of the children experienced side effects. Fifteen children received cyclosporine, and only eight of them (53% achieved remission. Six children developed gum hypertrophy and one had renal impairment, which was reversible after discontinuing the drug. Mycophonelate mofetil (MMF was used as the last option in 5 children, and 2 of them achieved complete remission. One child developed a systemic cytomegalovirus (CMV infection which indicated discontinuing the drug. Fourteen (45% children needed more than one immunosuppressive therapy. Three children progressed to end stage renal failure and required dialysis. We conclude that SRNS in children is a difficult disease with significant morbidity. However, remission is achievable with cyclosporine and other immunosuppressive agents. Treatment should be individualized according to the underlying histopathology, and clinical and social

  4. Children with Steroid-resistant Nephrotic Syndrome: a Single-Center Study

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    Rahime Renda

    2016-01-01

    Full Text Available Background and Aim: Steroid-resistant nephrotic syndrome (SRNS accounts for 10%-20% of all cases of idiopathic nephrotic syndrome. These patients are at risk of developing end-stage renal disease. The aim of this study was to determine the demographic characteristics, renal biopsy findings, response to immunosuppressive treatment, and prognosis in pediatric patients with SRNS.Materials and Methods: This retrospective study included 31 patients diagnosed as primary SRNS. Age at first episode, gender, parental consanguinity, and familial history of nephrotic syndrome were recorded. Demographic characteristics, renal biopsy findings, response to immunosuppressive treatment, and prognosis were analyzed, as were the number of and treatment of relapses, extra-renal manifestations, and complications of disease and treatment.Results: Mean age at first episode of nephrotic syndrome was 4,1±2,9 years. At the end of the first immunosuppressive treatment cycle, 14 (51.8% patients achieved complete remission, 4 (14.8% patients achieved partial remission, and 9 patients (33.3% did not achieve remission. Analysis of the final status of the patients showed that 16 patients (51.6% developed remission, 5 patients (16% continued to have nephrotic range proteinuria and 10 patients (32% developed chronic renal failure (CRF.Conclusion: The treatment of SRNS remains controversial. Early genetic testing can help the inevitable immunosuppressive treatments which may not be effective and have several side effects. Calcineurin inhibitors and mycophenolate mofetil are known to be effective immunosuppressive drugs for treating steroid resistant nephrotic syndrome .

  5. URINARY TRACT INFECTION IN CHILDREN WITH NEPHROTIC SYNDROME: A PROSPECTIVE OPEN LABELED STUDY

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    Rajendra

    2014-09-01

    Full Text Available : BACKGROUND: Nephrotic syndrome is an important chronic disorder in children in which prevalence of urinary tract infection (UTI is high. UTI may also be responsible for steroid resistance and relapse. UTI is an important but often undiagnosed condition in children with nephrotic syndrome. Hence the present study is being conducted. AIMS AND OBJECTIVES: The study is conducted to determine the clinical features of nephrotic syndrome and to evaluate the incidence, etiological agents, clinical features and the antibiotic sensitivity pattern of Urinary Tract Infections in children with Nephrotic Syndrome. MATERIAL & METHODS: The study was a Prospective hospital based study done by Stratified random sampling conducted on all the paediatric patients with a diagnosis of nephrotic syndrome attending the OPD and admitted to Paediatric hospital. Fifty children with diagnosis of Nephrotic Syndrome were studied. The specimen for urine culture was obtained carefully to prevent contamination by periurethral flora. A clean-catch midstream urine specimen was used. Contamination by periurethral and prepucial organisms was minimized by washing the genitalia. The specimen was directly collected in a sterile container. Prompt plating of the urine specimen, within one hour of collection was ensured. Identification of the organism to species level was done and antibiotic sensitivity pattern was also studied. RESULTS: Among the fifty children studied, boys were affected more than girls by Nephrotic syndrome with a ratio of 1.5:1. The mean age was 4.75 years. Urinary Tract Infection was detected in seventeen children (34%. The present study was statistically significant with p<0.001. The commonest Micro-organism isolated was Escherichia coli, followed by Staphylococcus aureus. Most of the Micro-organisms were sensitive to cephalosporins. CONCLUSION: Urinary Tract Infection is a significant infection detected in cases of Nephrotic Syndrome affecting one third of the

  6. The correlation between attention deficit hyperactivity disorder and steroid-dependent nephrotic syndrome

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    Parsa Yousefichaijan

    2015-01-01

    Full Text Available Nephrotic syndrome (NS is characterized by nephritic-range proteinuria and the triad of clinical findings associated with large urinary losses of protein, hypoalbuminemia, edema and hyperlipidemia. More than 80% of children below 13 years of age with primary NS have steroid-responsive forms. There is no identifiable cause of attention-deficit hyperactivity disorder (ADHD. It is likely that the symptoms of ADHD represent a final common pathway of diverse causes, including genetic, organic and environmental etiologies. This case-control study was performed on 130 children aged between 5 and 13 years who were followed-up for two years. Sixty-five children with steroid-dependent nephrotic syndrome (SDNS as the case group and 65 healthy children as the control group were included in the study. Patients with minimal change NS were treated with prednisolone for at least six months. Conner′s Parent Rating Scale - 48 (CPRS-48 was completed by the parents and the children were identified with any form of ADHD. Then, children were referred to an expert psychiatrist. The collected data were analyzed with SPSS software. The result showed that there was no significant relationship between different types of ADHD in both groups. Thus, based on current study, one may conclude that there are no significant differences between prevalence of ADHD in children with SDNS and the control group.

  7. The non-immunosuppressive management of childhood nephrotic syndrome.

    Science.gov (United States)

    McCaffrey, James; Lennon, Rachel; Webb, Nicholas J A

    2016-09-01

    Idiopathic nephrotic syndrome (INS) is one of the most common renal diseases found in the paediatric population and is associated with significant complications, including infection and thrombosis. A high proportion of children enter sustained remission before adulthood, and therapy must therefore mitigate the childhood complications, while minimising the long-term risk to health. Here we address the main complications of INS and summarise the available evidence and guidance to aid the clinician in determining the appropriate treatment for children with INS under their care. Additionally, we highlight areas where no consensus regarding appropriate management has been reached. In this review, we detail the reasons why routine prophylactic antimicrobial and antithrombotic therapy are not warranted in INS and emphasise the conservative management of oedema. When pharmacological intervention is required for the treatment of oedema, we provide guidance to aid the clinician in determining the appropriate therapy. Additionally, we discuss obesity and growth, fracture risk, dyslipidaemia and thyroid dysfunction associated with INS. Where appropriate, we describe how recent developments in research have identified potential novel therapeutic targets. PMID:26556028

  8. [Nephrotic syndrome as the first manifestation of juvenile systemic scleroderma.

    Science.gov (United States)

    Couto, Saulo B; Sallum, Adriana M; Henriques, Luciana S; Malheiros, Denise M; Silva, Clovis A; Vaisbich, Maria H

    2014-10-22

    Renal involvement occurs in 1%-12% in juvenile systemic sclerosis (JSSc) patients, mainly with arterial hypertension, proteinuria and scleroderma renal crisis. We report herein a patient who presented nephrotic syndrome (NS) as the first manifestation of JSSc with focal segmental glomerulosclerosis (FSGS). A female patient presented steroid-sensitive NS at the age of 12 years. At 14 years, she had orbital and lower limbs edema, arterial hypertension, sclerodactyly and proximal skin sclerosis. Moderate capillary dilation and mild focal devascularization were observed in nailfold capillaroscopy, compatible with early stage of scleroderma (scleroderma pattern). Percutaneous renal biopsy guided by ultrasound revealed focal segmental glomerulosclerosis and direct immunofluorescence were negative. Therefore, she fullfilled the provisional classification criteria for JSSc. Patient was treated with oral 25-hydroxyvitamin D (800 IU/day), methotrexate (0.5mg/kg/week) and amlodipin (0.15 mg/kg). Prednisone (60 mg/m(2)/day) was administered for 4 consecutive weeks, followed by alternate-day (40mg/m(2)) for 2 consecutive months, with tapering for 4 months and then stopping this medication. Currently she is being treated with methotrexate 15 mg/week, without edema and proteinuria. In conclusion, we reported a rare case of NS with FSGS as the first manifestation of scleroderma. Therefore, renal biopsy is mandatory in JSSc patients with sustained proteinuria or NS.

  9. Lessons from genetics: is it time to revise the therapeutic approach to children with steroid-resistant nephrotic syndrome?

    Science.gov (United States)

    Becherucci, Francesca; Mazzinghi, Benedetta; Provenzano, Aldesia; Murer, Luisa; Giglio, Sabrina; Romagnani, Paola

    2016-08-01

    Primitive nephrotic syndrome is one of the most common glomerular diseases in childhood and represents the clinical manifestation of various pathologic changes in the kidney. In children, nephrotic syndrome is classified based on the initial response to empiric corticosteroid treatment, which is considered as the best predictor of patients' final outcome. The advent of next-generation sequencing technology showed that genetic alterations in structural genes of the podocyte can be recognized in a significant proportion of not only familial or syndromic patients with steroid-resistant nephrotic syndrome (SRNS), but also of sporadic cases, raising the question of whether it is time to update current protocols of patient care. In this review, we discuss the implications derived from several studies describing a high prevalence in children with SRNS of pathogenic mutations in a group of genes and their unresponsiveness to immunosuppressive therapy. We propose a diagnostic and therapeutic algorithm to reduce the exposure to immunosuppressants in individuals with unresponsive forms of the disease, sparing patients the untoward side effects of prolonged ineffective treatments, and at the same time guaranteeing the optimal immunosuppressive or other new therapy in potentially responsive patients. PMID:27209298

  10. Nephrotic syndrome and multiple tubular defects in children: an early sign of focal segmental glomerulosclerosis.

    Science.gov (United States)

    McVicar, M; Exeni, R; Susin, M

    1980-12-01

    The nephrotic syndrome is rarely associated with renal tubular defects, and the combination has been reported only in association with advanced renal insufficiency. We report here five children with nephrotic syndrome and multiple tubular defects which evolved when glomular filtration rate ranged between 56 and 90 ml/minute/1.73 m2. The tubular defects were first noted at 3, 4, 4, 7, and 22 months after the onset of the nephrotic syndrome, and renal glycosuria was the first sign in all five children. Glycosuria was intermittent in three patients, constant in two, and ceased with loss of kidney function. Four patients had hyperaminoaciduria and renal tubular acidosis (two of four tested had distal renal tubular acidosis). Three patients had decreased tubular reabsorption of phosphorus and defective maximum concentrating capacity. All five had focal segmental glomerulosclerosis proven by renal biopsy. Over a follow-up period of seven years, all of the children have developed advanced renal insufficiency, four of the five have required dialysis or transplantation within 21 to 72 months after onset, and one has stabilized renal function at 35 ml/minute/1.73 m2. The one patient receiving a kidney transplant has had recurrence of focal segmental glomerulosclerosis in the transplanted kidney and became nephrotic with three subsequent transplants. Our experience suggests that the nephrotic syndrome associated with tubular defects in children forms a subgroup of focal segmental glomerulosclerosis, with rapid progression to renal insufficiency and the potential for recurrence of the lesion in the transplanted kidney.

  11. Correlation of fractional excretion of magnesium with steroid responsiveness in children with nephrotic syndrome

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    Jubaida Rumana

    2014-01-01

    Full Text Available Steroid-resistant nephrotic syndrome (SRNS patients are candidates for other alter-native drug regimes, and the non-responsiveness to steroid is more common among glomerulo-nephritides other than minimal change disease. Without performing biopsy and proper renal histology, progression of the disease cannot be assessed. Fractional excretion of magnesium (FE Mg has been found to correlate directly with various renal histologies. The aim of this study is to evaluate the relationship of FE Mg in children with the histological pattern in SRNS. In this prospective observational study, 40 children of nephrotic syndrome, both with the first episode as well as relapse, aged 1-12 years were included in the study. Of them, 20 were steroid-responsive cases and 20 were steroid-resistant cases. FE Mg was determined in all the patients and renal histology was performed in the steroid-resistant cases. A correlation was found between FE Mg and renal histology. Data were analyzed in SPSS program version-16. Comparison of two groups was performed by the Fisher exact test and unpaired t test. P-value less than 0.05 were considered to be significant. The results of histo-pathology showed that the mean difference in FE Mg was significant (P <0.001, as FE Mg was 7.0 ± 2.3% in mesangiocapillary glomerulonephritis, 6.9 ± 1.3% in focal segmental glomerulosclerosis, 4.7 ± 0.6% in immunoglobulin M nephropathy, 4.5 ± 1.2% in focal segmental proliferative glomerulo-nephritis, 4.4 ± 1.6% in minimal change disease, 4.2 ± 0.4% in diffuse mesangial proliferative glome-rulonephritis and 3.8 ± 1.3% in mesangial proliferative glomerulonephritis. There was a statistically significant difference between FE Mg in steroid-resistant nephrotic syndrome (4.9 ± 1.9 and steroid-responsive syndrome (1.2 ± 0.3. FE Mg is a simple, minimally invasive screening marker for SRNS, and is an early predictor of clinical outcome. It can be considered as an initial investigation where biopsy

  12. Correlation of fractional excretion of magnesium with steroid responsiveness in children with nephrotic syndrome.

    Science.gov (United States)

    Rumana, Jubaida; Hanif, Mohammed; Muinuddin, Golam; Maruf-Ul-Quader, Mohammed

    2014-07-01

    Steroid-resistant nephrotic syndrome (SRNS) patients are candidates for other alter-native drug regimes, and the non-responsiveness to steroid is more common among glomerulo-nephritides other than minimal change disease. Without performing biopsy and proper renal histology, progression of the disease cannot be assessed. Fractional excretion of magnesium (FE Mg) has been found to correlate directly with various renal histologies. The aim of this study is to evaluate the relationship of FE Mg in children with the histological pattern in SRNS. In this prospective observational study, 40 children of nephrotic syndrome, both with the first episode as well as relapse, aged 1-12 years were included in the study. Of them, 20 were steroid-responsive cases and 20 were steroid-resistant cases. FE Mg was determined in all the patients and renal histology was performed in the steroid-resistant cases. A correlation was found between FE Mg and renal histology. Data were analyzed in SPSS program version-16. Comparison of two groups was performed by the Fisher exact test and unpaired t test. P-value less than 0.05 were considered to be significant. The results of histo-pathology showed that the mean difference in FE Mg was significant (P <0.001), as FE Mg was 7.0 ± 2.3% in mesangiocapillary glomerulonephritis, 6.9 ± 1.3% in focal segmental glomerulosclerosis, 4.7 ± 0.6% in immunoglobulin M nephropathy, 4.5 ± 1.2% in focal segmental proliferative glomerulo-nephritis, 4.4 ± 1.6% in minimal change disease, 4.2 ± 0.4% in diffuse mesangial proliferative glome-rulonephritis and 3.8 ± 1.3% in mesangial proliferative glomerulonephritis. There was a statistically significant difference between FE Mg in steroid-resistant nephrotic syndrome (4.9 ± 1.9) and steroid-responsive syndrome (1.2 ± 0.3). FE Mg is a simple, minimally invasive screening marker for SRNS, and is an early predictor of clinical outcome. It can be considered as an initial investigation where biopsy cannot be

  13. Renal amyloidosis in leprosy, an infrequent cause of nephrotic syndrome in Europe.

    Science.gov (United States)

    Sanz-Martín, Noelia; Samillán-Sosa, Kelly Del Rocío; De Miguel, Julio; Martínez-Miguel, Patricia

    2016-01-01

    Leprosy is a chronic infectious disease caused by Mycobacterium leprae The main clinical manifestations involve the skin and the peripheral nervous system. Several types of nephropathy have been described in leprosy. One frequent form of renal involvement is amyloidosis, especially in patients with lepromatous leprosy. In these patients, end-stage renal disease is an important contributor to morbidity and mortality. Here, we present the case of a patient with nephrotic syndrome caused by secondary amyloidosis, chronic peripheral neuropathy and a history of leprosy. The patient was correctly treated in her youth, which is the best way to avoid renal pathology, but she developed a nephrotic syndrome years later. PMID:27489069

  14. Clinical Analysis of Pediatric Clinical Nephrotic Syndrome%小儿肾病综合征的临床分析

    Institute of Scientific and Technical Information of China (English)

    张丽丽

    2015-01-01

    ObjectiveTo investigate the clinical treatment of children with nephrotic syndrome.Methods 40 cases of children with nephrotic syndrome clinical treatment data were analyzed.Results 40 cases of children with nephrotic syndrome in patients with clinical remission in 24 cases, 14 cases improved, no change in two cases.Conclusion The disease mainly adopt a glucocorticoid , recurrent or steroid -resistant with the application of immunosuppressive drugs , the cure rate is high, small adverse reactions.%目的:探讨小儿肾病综合征患者临床治疗。方法对40例小儿肾病综合征患者临床治疗方法资料进行分析。结果40例小儿肾病综合征患者经治疗临床缓解24例,好转14例,无变化2例。结论本病采用以糖皮质激素为主,反复发作或对激素耐药者配合应用免疫抑制药,治愈率高,不良反应小。

  15. Long-term treatment of childhood refractory and steroid dependent nephrotic syndrome with Cyclosporin A

    Directory of Open Access Journals (Sweden)

    Madani A

    2002-07-01

    Full Text Available Background: Cyclosporin A (CsA is now commonly used in the management of children with steroid-dependent and steroid resistant nephoitic syndrome. It has been reported to be effective in maintaining remission in 70-100 percent of patients with SDNS but somewhat SRNS 0-100 percent. The aim of this study was to evaluate the efficacy of long-term (CsA in children with refractory nephrotic syndrome (RNS and steroid dependent nephrotic syndrome (SDNS. Materials and Methods: The long-term effect of (CsA in 91 Iranian children aged 3 months to 11 years (54 with RNS and 37 with SDNS was assessed between 1984 and 1999. Eighty of 91 children received renal biopsy prior to introduction of (CsA, and the other 11 patients had not consent for kidney biopsy. If the patients did not show remission aftre receiving 3-6 months of (CsA, the medication was discontinued. Results: All patient were treated with (CsA in combination with low dose alternate day prednisolone. In children with RNS and SDNS, therapy with (CsA induced, remission in 25 of 54 (46.2 percent and 27 of 37 (73 percent respectively (P<0.02. Of the 32 patients with minimal change disease (MCD, 23 (72 percent responded to therapy, compared with 4 of 18 (22 percent with focal segmental glomerulosclerosis (FSGS (P<0.005. Twenty-four (48 percent of 50 who entered complete remission, had relapse 1-12 months after cessation of (CsA. The duration between the onset of nephrotic syndrome (NS and administration of (CsA and sexuality of patients had no effect in result of treatment. Side effects occurred in 25 patients (27.4 percent. No patients exhibited raised transaminases, 8 (8.7 percent of the children developed hirsutism, 7 (7.6 percent hypertension, 7 (7.6 percent gingival hyperplasia, (2.2 percent neurological toxicity and 1 (1 percent increase in serum creatinine. Conclusion: Our findings suggest that (CsA can be used to induce a complete remission in a significant proportion of patients with RNS and

  16. Membranous glomerulonephropathy and nephrotic syndrome associated with iatrogenic metallic mercury poisoning in a cat.

    Science.gov (United States)

    Shull, R M; Stowe, C M; Osborne, C A; O'Leary, T P; Vernier, R L; Hammer, R F

    1981-02-01

    The nephrotic syndrome, characterized by nonselective proteinuria, hypoproteinemia, hypoalbuminemia, and ascites, was observed in a 10-month-old male cat. Profound glomerular changes and renal tubular changes appear to have been induced by iatrogenic chronic exposure to metallic mercury originally contained in a rectal thermometer. Large concentrations of mercury were present in the kidneys, liver, spleen, and urine. Evaluation of glomeruli by immunofluorescent microscopy revealed interrupted granular deposition of immuno-globulin G and the third component of complement in glomerular capillary walls and the mesangium. Electron microscopic evaluation of glomeruli revealed diffuse alterations in glomerular basement membranes and visceral epithelial cells. Small electron dense deposits were observed in capillary walls, but they were not characteristic of immune complexes. The mechanism(s) responsible for the mercury induced glomerulonephropathy in this patient could not be determined on the basis of available data. PMID:7257162

  17. Vincristine treatment in steroid-dependent nephrotic syndrome.

    Science.gov (United States)

    Kausman, Joshua Yehuda; Yin, Lei; Jones, Colin Lindsay; Johnstone, Lillian; Powell, Harley Robert

    2005-10-01

    Treatment of children with steroid-dependent nephrotic syndrome (SDNS) continues to be a challenge when relapses recur after treatment with cyclophosphamide and side effects or non-compliance make steroids and cyclosporin unsatisfactory. We treated 12 patients with intravenous vincristine for SDNS in a regime of 1-1.5 mg/m2 weekly for 4 weeks then monthly for 4 months. Four of the 5 patients in relapse when commencing vincristine remitted within 2 doses. Comparing relapse frequency in the 12 months before and after vincristine, there was a reduction from 4 to 1.5 (p=0.004) relapses per year. Median sustained remission was 5 months, but 1 frequently relapsing patient remains in remission 4 years after vincristine. Vincristine was also successfully used in 1 or 2 doses at weekly intervals for subsequent relapses in 5 patients. Side effects were minimal in most cases. Abdominal pain occurred in 2 patients who commenced vincristine at 1.5 mg/m2, but resolved when continued at 1 mg/m2. We felt vincristine had a role in a subset of children with challenging SDNS administered as 1 mg/m2 weekly for 4 weeks then 1.5 mg/m2 monthly for 4 months. Vincristine allowed steroid- and cyclosporin-sparing, contributed to long-term remission in some patients, and was especially valuable in children with poor compliance with oral medication. Many patients expressed a preference for a few doses of vincristine rather than a standard course of oral prednisolone or cyclosporin. PMID:15977025

  18. Nephroprotective effect of heparanase in experimental nephrotic syndrome.

    Directory of Open Access Journals (Sweden)

    Suheir Assady

    Full Text Available Heparanase, an endoglycosidase that cleaves heparan sulfate (HS, is involved in various biologic processes. Recently, an association between heparanase and glomerular injury was suggested. The present study examines the involvement of heparanase in the pathogenesis of Adriamycin-induced nephrotic syndrome (ADR-NS in a mouse model.BALB/c wild-type (wt mice and heparanase overexpressing transgenic mice (hpa-TG were tail-vein injected with either Adriamycin (ADR, 10 mg/kg or vehicle. Albuminuria was investigated at days 0, 7, and 14 thereafter. Mice were sacrificed at day 15, and kidneys were harvested for various analyses: structure and ultrastructure alterations, podocyte proteins expression, and heparanase enzymatic activity.ADR-injected wt mice developed severe albuminuria, while ADR-hpa-TG mice showed only a mild elevation in urinary albumin excretion. In parallel, light microscopy of stained cross sections of kidneys from ADR-injected wt mice, but not hpa-TG mice, showed mild to severe glomerular and tubular damage. Western blot and immunofluorescence analyses revealed significant reduction in nephrin and podocin protein expression in ADR-wt mice, but not in ADR-hpa-TG mice. These results were substantiated by electron-microscopy findings showing massive foot process effacement in injected ADR-wt mice, in contrast to largely preserved integrity of podocyte architecture in ADR-hpa-TG mice.Our results suggest that heparanase may play a nephroprotective role in ADR-NS, most likely independently of HS degradation. Moreover, hpa-TG mice comprise an invaluable in vivo platform to investigate the interplay between heparanase and glomerular injury.

  19. Serum osteoprotegerin (OPG in children with primary nephrotic syndrome

    Directory of Open Access Journals (Sweden)

    Gamal B Mohamed

    2011-01-01

    Full Text Available A novel cytokine system secreted by osteoblast, osteoprotegerin (OPG and its ligand (OPGL regulates osteoclastogenesis. To determine the relation of the serum OPG levels in children with nephrotic syndrome (NS to the renal disease, we studied 30 patients with NS in comparison with 30 healthy children serving as controls. The study patients were divided into three equal groups: group 1 included newly diagnosed patients who were studied before and after a short course (one month of steroid therapy for the first time, group 2 included frequent relapsers (FR, and group 3 included infrequent relapsers (IFR. In addition to serum OPG (ELISA, osteocalcin (OC, parathormone (PTH, alkaline phosphatase (ALP, and 24- hour urinary Ca and proteins were measured. The NS patients revealed a significantly lower serum OPG and parameters of bone formation (ALP and OC and a significantly higher 24- hour urinary Ca than controls. A short course of glucocorticoids therapy for one month resulted in a significant decrease of serum OPG, ALP and OC levels and a significant increase of 24- hour urinary Ca, while serum PTH levels were not significantly affected by this the- rapy; the FR revealed a significantly lower serum level and a significantly higher 24- hour urinary Ca and serum PTH than the IFR. OPG had significant negative correlations with markers of disease activity and severity (ESR, serum cholesterol, 24- hour urinary protein and cumulative steroid dose, PTH and 24- hour urinary Ca. On the other hand, OPG had significant positive correlations with ALP, OC, and serum albumin. Low serum OPG, which is attributed to the renal disease and/or steroid therapy, may be an important factor contributing to bone resorption in NS. Studies of the protective effect of OPG administration against bone loss in NS are warranted.

  20. Statins in nephrotic syndrome: a new weapon against tissue injury.

    Science.gov (United States)

    Buemi, Michele; Nostro, Lorena; Crascì, Eleonora; Barillà, Antonio; Cosentini, Vincenzo; Aloisi, Carmela; Sofi, Tito; Campo, Susanna; Frisina, Nicola

    2005-11-01

    The nephrotic syndrome is characterized by metabolic disorders leading to an increase in circulating lipoproteins levels. Hypertriglyceridemia and hypercholesterolemia in this case may depend on a reduction in triglyceride-rich lipoproteins catabolism and on an increase in hepatic synthesis of Apo B-containing lipoproteins. These alterations are the starting point of a self-maintaining mechanism, which can accelerate the progression of chronic renal failure. Indeed, hyperlipidemia can affect renal function, increase proteinuria and speed glomerulosclerosis, thus determining a higher risk of progression to dialysis. 3-hydroxy-3-methylglutaryl-coenzyme A (HMG-CoA) reductase is the rate-limiting enzyme in cholesterol synthesis from mevalonate and its inhibitors, or statins, can therefore interfere with the above-mentioned consequences of hyperlipidemia. Statins are already well known for their effectiveness on primary cardiovascular prevention, which cannot be explained only through their hypolipemic effect. As far as kidney diseases are concerned, statin therapy has been shown to prevent creatinine clearance decline and to slow renal function loss, particularly in case of proteinuria, and its favorable effect may depend only partially on the attenuation of hyperlipidemia. Statins may therefore confer tissue protection through lipid-independent mechanisms, which can be triggered by other mediators, such as angiotensin receptor blockers. Possible pathways for the protective action of statins, other than any hypocholesterolemic effect, are: cellular apoptosis/proliferation balance, inflammatory cytokines production, and signal transduction regulation. Statins also play a role in the regulation of the inflammatory and immune response, coagulation process, bone turnover, neovascularization, vascular tone, and arterial pressure. In this study, we would like to provide scientific evidences for the pleiotropic effects of statins, which could be the starting point for the

  1. 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.

  2. Serum peptidome profiling for identifying pathological patterns in patients with primary nephrotic syndrome

    Institute of Scientific and Technical Information of China (English)

    HUANG Lan-ting; WEN Qiong; ZHAO Ming-zhe; LI Zhi-bin; LUO Ning; WANG Yong-tao; DONG Xiu-qing; YU Xue-qing

    2012-01-01

    Background Renal biopsy is necessary for diagnosing the pathological changes of primary nephrotic syndrome (NS).However,it is invasive,time-consuming and can not be performed frequent on the same patient.Thus,development of a non-invasive and rapid diagnostic method may improve clinical patient management.Methods Proteomic tool magnetic bead-based matrix-assisted laser desorption/ionization time-of-flight mass spectrometry (MB-based MALDI TOF MS) was applied to serum to determine peptidome patterns that are characteristic of different pathological changes.Results Serum specimen from 114 patients with NS (62 were minimal change disease (MCD),30 were membranous nephropathy (MN),and 22 were focal segmental glomerulosclerosis (FSGS)) and 60 normal individuals were analyzed using MB-based MALDI TOF MS.The peptidome pattern was generated by genetic algorithms using a training set of 31 MCD,15 MN,11 FSGS and 30 normal individuals and was validated by an independent testing set of the remaining samples.The serum peptidome pattern,based on a panel of 14 peaks,accurately recognized samples from MCD,MN,FSGS and healthy control with sensitivities of 93.5%,86.7%,63.6% and 90.0%,and specificities of 98.2%,94.4%,100%and 89.5%,respectively.Moreover,one peptide from peptidome pattern was identified by liquid chromatography tandem mass spectrometry (LC MS/MS) as fibrinogen A.Conclusion Detection of the serum peptidome pattern is a rapid,non-invasive,high-throughout,and reproducible method for identifying the pathological patterns of patients with nephrotic syndrome.

  3. Infant Boy with Microcephaly Gastroesophageal Refl ux and Nephrotic Syndrome (Galloway-Mowat Syndrome): A Case Report.

    Science.gov (United States)

    Malaki, Majid; Rafeey, Mandana

    2012-01-01

    In this case report, we present the first diagnosed case of Galloway-Mowat syndrome in Iran. A 7 month old infant boy withmicrocephaly that had prominently stunted head growth afterbirth, gastroesophageal reflux, multiple craniofascial characters,hypothyroidism and nephrotic syndrome diagnosed at 5 monthsof age associated with rapid decline in renal function and heavyproteinuria in 2 months .

  4. Vitamin D status is insufficient in the majority of children at diagnosis of nephrotic syndrome

    DEFF Research Database (Denmark)

    Nielsen, Cecilie Ane; Jensen, Jens-Erik Bech; Cortes, Dina

    2015-01-01

    INTRODUCTION: Children with nephrotic syndrome (NS) are treated for at least 12 weeks with high doses of prednisolone, which may be harmful to the bones. Vitamin D deficiency is also harmful to the bones. METHODS: This was a prospective study of consecutive children with first episode of NS at th...

  5. MAC-2BP is increased in plasma during nephrotic syndrome and is produced by PBMC

    DEFF Research Database (Denmark)

    Andersen, René Frydensbjerg; Kamperis, Konstantinos; Rittig, Søren

    OBJECTIVES: Edema is a hallmark of nephrotic syndrome (NS) and has largely been attributed to sodium retention. Yet low plasma sodium is frequently observed in children with acute NS suggesting that a disturbance in renal water handling may coexist together with sodium retention. The aim of the s...

  6. Cortical sinovenous thrombosis in a child with nephrotic syndrome and iron deficiency anaemia.

    Directory of Open Access Journals (Sweden)

    Meena A

    2000-07-01

    Full Text Available Cortical sinovenous thrombosis in a child with nephrotic syndrome and iron deficiency anaemia is described. The most probable mechanism for the hypercoagulable state was thrombocytosis associated with iron deficiency anaemia. The other possible contributing factor might have been the diuretic therapy during the phase of relapse.

  7. Familial Mediterranean fever, Inflammation and Nephrotic Syndrome: Fibrillary Glomerulopathy and the M680I Missense Mutation

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    Semerdjian Ronald J

    2003-08-01

    Full Text Available Abstract Background Familial Mediterranean fever (FMF is an autosomal recessive disease characterized by inflammatory serositis (fever, peritonitis, synovitis and pleuritis. The gene locus responsible for FMF was identified in 1992 and localized to the short arm of chromosome 16. In 1997, a specific FMF gene locus, MEFV, was discovered to encode for a protein, pyrin that mediates inflammation. To date, more than forty missense mutations are known to exist. The diversity of mutations identified has provided insight into the variability of clinical presentation and disease progression. Case Report We report an individual heterozygous for the M680I gene mutation with a clinical diagnosis of FMF using the Tel-Hashomer criteria. Subsequently, the patient developed nephrotic syndrome with biopsy-confirmed fibrillary glomerulonephritis (FGN. Further diagnostic studies were unremarkable with clinical workup negative for amyloidosis or other secondary causes of nephrotic syndrome. Discussion Individuals with FMF are at greater risk for developing nephrotic syndrome. The most serious etiology is amyloidosis (AA variant with renal involvement, ultimately progressing to end-stage renal disease. Other known renal diseases in the FMF population include IgA nephropathy, IgM nephropathy, Henoch-Schönlein purpura as well as polyarteritis nodosa. Conclusion To our knowledge, this is the first association between FMF and the M680I mutation later complicated by nephrotic syndrome and fibrillary glomerulonephritis.

  8. Steroid-dependent nephrotic syndrome in lupus nephritis. Response to chlorambucil.

    Science.gov (United States)

    Abuelo, J G; Esparza, A R; Garella, S

    1984-12-01

    Nephrotic syndrome associated with mesangial lupus nephritis developed in a young woman. The heavy proteinuria exhibited a striking steroid-dependent course during a three-year period of time, with ten relapses occurring whenever attempts were made to withdraw prednisone therapy. A prolonged remission was induced by the administration of chlorambucil.

  9. High absolute risks and predictors of venous and arterial thromboembolic events in patients with nephrotic syndrome

    NARCIS (Netherlands)

    Mahmoodi, Bakhtawar K.; ten Kate, Min Ki; Waanders, Femke; Veeger, Nic J. G. M.; Brouwer, Jan-Leendert P.; Vogt, Liffert; Navis, Gerjan; van der Meer, Jan

    2008-01-01

    Background-No data are available on the absolute risk of either venous thromboembolism (VTE) or arterial thromboembolism (ATE) in patients with nephrotic syndrome. Reported risks are based on multiple case reports and small studies with mostly short-term follow-up. We assessed the absolute risk of V

  10. Statin use in patients with nephrotic syndrome is associated with a lower risk of venous thromboembolism

    NARCIS (Netherlands)

    Resh, Mohammad; Mahmoodi, Bakhtawar K.; Navis, Gerjan J.; Veeger, Nic J. G. M.; Lijfering, Willem M.

    2011-01-01

    Background: Nephrotic syndrome (NS) is a well-known risk factor for venous thromboembolism (VTE), however preventive measures are not routinely taken. In non-renal populations, statins are associated with lower risk of VTE. Hence, we set up this single-center retrospective cohort study to assess whe

  11. Nephrotic Syndrome Associated with Lung Cancer: A Rare Case of Malignancy Associated with AA Amyloidosis

    Science.gov (United States)

    Gueutin, Victor; Langlois, Anne-Lyse; Shehwaro, Nathalie; Elharraqui, Ryme; Rouvier, Philippe; Izzedine, Hassane

    2013-01-01

    Nonhematologic malignancies are rarely reported to be associated with AA amyloidosis. Although the association between renal cell carcinoma and systemic AA amyloidosis has been established, the evidence linking pulmonary cancer to AA amyloidosis is scarce. Here, a case of biopsy-proven renal AA amyloidosis complicated with nephrotic syndrome associated with lung carcinoma is reported. PMID:24558629

  12. Nephrotic Syndrome Associated with Lung Cancer: A Rare Case of Malignancy Associated with AA Amyloidosis

    OpenAIRE

    Victor Gueutin; Anne-Lyse Langlois; Nathalie Shehwaro; Ryme Elharraqui; Philippe Rouvier; Hassane Izzedine

    2013-01-01

    Nonhematologic malignancies are rarely reported to be associated with AA amyloidosis. Although the association between renal cell carcinoma and systemic AA amyloidosis has been established, the evidence linking pulmonary cancer to AA amyloidosis is scarce. Here, a case of biopsy-proven renal AA amyloidosis complicated with nephrotic syndrome associated with lung carcinoma is reported.

  13. Nephrotic Syndrome Associated with Lung Cancer: A Rare Case of Malignancy Associated with AA Amyloidosis

    Directory of Open Access Journals (Sweden)

    Victor Gueutin

    2013-01-01

    Full Text Available Nonhematologic malignancies are rarely reported to be associated with AA amyloidosis. Although the association between renal cell carcinoma and systemic AA amyloidosis has been established, the evidence linking pulmonary cancer to AA amyloidosis is scarce. Here, a case of biopsy-proven renal AA amyloidosis complicated with nephrotic syndrome associated with lung carcinoma is reported.

  14. A Case of Newly Diagnosed Klippel Trenaunay Weber Syndrome Presenting with Nephrotic Syndrome

    Directory of Open Access Journals (Sweden)

    Egemen Cebeci

    2015-01-01

    Full Text Available Klippel Trenaunay Weber syndrome (KTWS is a rare disease characterized by hemihypertrophy, variceal enlargement of the veins, and arteriovenous (AV malformations. Renal involvement in KTWS is not known except in rare case reports. Herein, we present a case of KTWS with nephrotic syndrome. A 52-year-old male was admitted due to dyspnea and swelling of the body for the last three months. The pathological physical findings were diffuse edema, decreased lung sounds at the right basal site, increased diameter and decreased length of the left leg compared with the right one, diffuse variceal enlargements, and a few hemangiomatous lesions on the left leg. The pathological laboratory findings were hypoalbuminemia, hyperlipidemia, increased creatinine level (1.23 mg/dL, and proteinuria (7.6 g/day. Radiographic pathological findings were cystic lesions in the liver, spleen, and kidneys, splenomegaly, AV malformation on the left posterolateral thigh, and hypertrophy of the soft tissues of the proximal left leg. He was diagnosed to have KTWS with these findings. Renal biopsy was performed to determine the cause of nephrotic syndrome. The pathologic examination was consistent with focal segmental sclerosis (FSGS. He was started on oral methylprednisolone at the dosage of 1 mg/kg and began to be followedup in the nephrology outpatient clinic.

  15. Corticosteroids and obesity in steroid-sensitive and steroid-resistant nephrotic syndrome

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    Nina Lestari

    2015-07-01

    Full Text Available Background Children with nephrotic syndrome need high-dose corticosteroids to achieve remission. Studies have estimated a 35-43% risk of obesity in these patients after corticosteroid treatment. Objective To determine the prevalence of obesity in children who received corticosteroids for nephrotic syndrome, and to compare the risk of obesity in children with steroid-sensitive nephrotic syndrome (SSNS and steroid-resistant nephrotic syndrome (SRNS. Methods We performed a retrospective cohort study in 50 children with SSNS or SRNS who received corticosteroid treatment. Obesity was defined to be a BMI-for-age Z-score above +2.0 SD, according to the WHO Growth Reference 2007. Central obesity was defined to be a waist-to-height ratio > 0.50. Results The overall prevalence of obesity was 22%, with 29% and 14% in the SSNS and SRNS groups, respectively. The overall prevalence of central obesity was 50%, with 54% and 46% in the SSNS and SRNS groups, respectively. The cumulative steroid doses in this study were not significantly different between the SSNS and SRNS groups. There were also no significant differences between groups for risk of obesity (RR 2.53; 95%CI 0.58 to 10.99 or central obesity (RR 1.39; 95%CI 0.45 to 4.25. Conclusion In children with nephrotic syndrome who received corticosteroids, the prevalence of obesity is 22% and of central obesity is 50%. In a comparison of SSNS and SRNS groups, cumulative steroid dose as well as risks of obesity and central obesity do not significantly differ between groups.

  16. Intracranial hypertension presenting with severe visual failure, without concurrent headache, in a child with nephrotic syndrome

    OpenAIRE

    Barnett, Madeleine; Sinha, Manish D.; Morrison, Danny; Lim, Ming

    2013-01-01

    Background Idiopathic intracranial hypertension is a condition typically characterised by headache, normal level of consciousness, papilloedema and raised cerebrospinal fluid pressure. Children often present with visual loss and atypical features of raised pressure, posing a diagnostic and management challenge. A range of renal disorders can predispose to developing this raised intracranial pressure syndrome. We present a case of severe visual failure in a child with nephrotic syndrome, with ...

  17. The Effect of a Gluten-Free Diet in Children With Difficult-to-Manage Nephrotic Syndrome

    Science.gov (United States)

    Lemley, Kevin V.; Faul, Christian; Schramm, Karla; Meyers, Kevin; Kaskel, Frederick; Dell, Katherine M.; Gipson, Debbie S.; Gibson, Keisha; Trachtman, Howard

    2016-01-01

    Case reports have linked childhood nephrotic syndrome to food sensitivity, including gluten. We report our experience with 8 children (6 boys, 2 girls; age at implementation of special diet 2–14 years) with difficult-to-manage nephrotic syndrome who were placed on a gluten-free diet for 3.4 ± 4.3 years (range, 0.6–14 years) and who had clinical improvement enabling reduction or discontinuation in steroid dosage. PMID:27338701

  18. Study of oxidative stress, homocysteine, copper & zinc in nephrotic syndrome: therapy with antioxidants, minerals and B-complex vitamins

    OpenAIRE

    Jyoti Dwivedi; Purnima Dey Sarkar

    2009-01-01

    Oxidative damage has been proposed as one of the possible mechanism involved in the nephrotic syndrome. Strengthening the defense system by antioxidants may provide protection against oxidative damage. Therefore, this study was carried out to investigate oxidant and antioxidant status with copper, zinc and homocysteine in nephrotic syndrome patients and the effect of antioxidants, minerals and B-complex vitamins on oxidant and antioxidant status. The blood samples were analyzed for quantitati...

  19. Experimental immune complex glomerulonephritis and the nephrotic syndrome in cats immunised with cationised bovine serum albumin.

    Science.gov (United States)

    Nash, A S; Mohammed, N A; Wright, N G

    1990-11-01

    Membranous nephropathy was induced in four cats by repeated intravenous injections of 120 mg cationic bovine serum albumin (BSA, pI 9.5). All four cats developed diffuse granular deposits of IgG and C3 along the glomerular capillary walls as early as five weeks which persisted until the end of the experiment at 17 weeks. Ultrastructural studies revealed many subepithelial electron dense deposits. Two cats developed severe proteinuria and the nephrotic syndrome characterised by hypoalbuminaemia and oedema. An additional four cats received repeated injections of unmodified native BSA (pI 4.5) and remained basically normal. This is the first report of membranous nephropathy and the nephrotic syndrome in an experimental animal model which, unlike other animal models, is subject to the spontaneously occurring disease. PMID:2148430

  20. Clinical Use of Diuretics in Heart Failure, Cirrhosis, and Nephrotic Syndrome

    Directory of Open Access Journals (Sweden)

    Ahmed Hassaan Qavi

    2015-01-01

    Full Text Available Diuretics play significant role in pharmacology and treatment options in medicine. This paper aims to review and evaluate the clinical use of diuretics in conditions that lead to fluid overload in the body such as cardiac failure, cirrhosis, and nephrotic syndrome. To know the principles of treatment it is essential to understand the underlying pathophysiological mechanisms that cause the need of diuresis in the human body. Various classes of diuretics exist, each having a unique mode of action. A systemic approach for management is recommended based on the current guidelines, starting from thiazides and proceeding to loop diuretics. The first condition for discussion in the paper is cardiac failure. Treatment of ascites in liver cirrhosis with spironolactone as the primary agent is highlighted with further therapeutic options. Lastly, management choices for nephrotic syndrome are discussed and recommended beginning from basic sodium restriction to combined diuretic therapies. Major side effects are discussed.

  1. Rare case of spot diagnosis of nephrotic syndrome in a Saudi male

    Directory of Open Access Journals (Sweden)

    A Askar

    2011-01-01

    Full Text Available A 26- year- old Saudi male school teacher presented with clinical features of nephrotic syndrome. Physical examination revealed loss of subcutaneous fat over the face and upper arms (partial lipodystrophy and drusen in both eyes. The serum level of complement C3 was low, with normal C4. Renal biopsy revealed features consistent with membranoproliferative glomerulo- nephritis (MPGN type II [Dense Deposit Disease (DDD]. He was treated with prednisolone and mycophenolate mofetil with partial remission. He also received lipitor and lisinopril. The occurrence of partial lipo- dystrophy and drusen in a patient with nephrotic syndrome provides a rare clinical opportunity of making a bedside diagnosis of type II MPGN even before kidney biopsy.

  2. Juvenile idiopathic arthritis complicated by amyloidosis with secondary nephrotic syndrome - effective treatment with tocilizumab.

    Science.gov (United States)

    Kwiatkowska, Małgorzata; Jednacz, Ewa; Rutkowska-Sak, Lidia

    2015-01-01

    A case report of a boy with juvenile idiopathic arthritis since the age of 2 years, generalized onset, complicated by nephrotic syndrome due to secondary type A amyloidosis is presented. In the patient the disease had an especially severe course, complicated by frequent infections, making routine treatment difficult. Amyloidosis was diagnosed in the 5(th) year of the disease based on a rectal biopsy. Since the disease onset the boy has been taking prednisolone and sequentially cyclosporine A, methotrexate, chlorambucil, etanercept, and cyclophosphamide. Clinical and laboratory remission was observed after treatment with tocilizumab. After 42 months of treatment with tocilizumab the boy's condition is good. There is no pain or joint edema, and no signs of nephrotic syndrome.

  3. Acute liver failure due to primary amyloidosis in a nephrotic syndrome: a swiftly progressive course.

    Science.gov (United States)

    Cardoso, Brigite Aguiar; Leal, Rita; Sá, Helena; Campos, Mário

    2016-01-01

    AL amyloidosis is a clonal plasma cell proliferative disorder characterised by extracellular tissue deposits of insoluble fibrils derived from κ or λ immunoglobulin light chains. The most common organs affected by AL amyloidosis are the kidney, presenting with nephrotic syndrome and/or progressive renal dysfunction, and the heart, with restrictive cardiomyopathy. Hepatic deposition of fibrils occurs in half the cases but the liver is rarely the predominantly affected organ. The most common presentation of hepatic amyloidosis is hepatomegaly with elevated alkaline phosphatase. Acute liver failure with cholestasis and jaundice is a rare complication, with a prevalence of approximately 5%, and is usually associated with a worse prognosis. We report a case of a 39-year-old man admitted to our nephrology department with an unusual presentation of primary amyloidosis with nephrotic syndrome and acute liver failure, complicated by obstructive cholestasis resulting in death 2 months after diagnosis. PMID:26965175

  4. Genetic basis of congenital nephrotic syndrome and generation of an animal model

    OpenAIRE

    Putaala, Heli

    2001-01-01

    Congenital nephrotic syndrome, NPHS1, is a disease highly enriched in Finland with incidence of 1:82oo births.The disease is characterized by massive proteinuria, go % of which is albumin, starting in utero, large placenta, which weighs over 25 % of the child's birth weight, and manifestation of proteinuria soon after birth. Historically, NPHS1 children have died before the age Of 2, but an aggressive nutritional therapy together with kidney transplantation can today save th...

  5. 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.

  6. Nephrotic Syndrome Following H1N1 Influenza in a 3-Year-Old Boy

    OpenAIRE

    Pio Liberatore; Francesca del Bufalo; Giorgia Bottaro; Pietro Ferrara; Antonio Gatto; Ottavio Vitelli

    2012-01-01

    Background: The pandemic influenza A/H1N1, spread through the world in 2009, producing a serious epidemic in Italy. Complications are generally limited to patients at the extremes of age (65 years) and those with comorbid medical illness. The most frequent complications of influenza involve the respiratory system.Case Presentation: A 3-year-old boy with a recent history of upper respiratory tract infection developed a nephrotic syndrome. Together with prednisone, furosemide and albumin bolus,...

  7. Colchicine therapy in amyloidosis related with plasmacytic castleman disease presenting with nephrotic syndrome

    Directory of Open Access Journals (Sweden)

    Saime Paydas

    2015-01-01

    Full Text Available Castleman disease (CD is a neoplasm that presents with single or multiple lymphadenopathy. The disease is characterized by fever, weight loss, anemia, polyclonal hyperglobulinemia, splenomegaly, thrombocytosis and peripheral lymphadenopathy. In this paper, we report a young man with plasmacytic type CD and amyloid A (AA deposition who presented with intra-abdominal mass and nephrotic syndrome. He was successfully treated with colchicine following surgery.

  8. Management of steroid resistant nephrotic syndrome in children with cyclosporine - a tertiary care centre experience

    International Nuclear Information System (INIS)

    Objective: To observe the response and adverse effects of cyclosporine in combination with oral steroids for management of idiopathic steroid resistant nephrotic syndrome in pediatric patients. Methodology: It was an observational study conducted at Children Hospital, Lahore, Pakistan from March 2014 to June 2015. Forty normotensive patients of idiopathic steroid resistant nephrotic syndrome between one and twelve years of age with normal renal function were included in the study. Patients were prescribed cyclosporine with prednisolone and were followed to see the response and adverse effects of drugs. Results: Out of 40 patients, 20(50%) were males and 20(50%) females. Mesangioproliferative glomerulonephritis was found in 27(67.5%) patients followed by Focal segmental glomerulosclerosis in 9(22.5%) patients. Complete response was observed in 32(80%) children while partial response in 8(20%) patients at the end of six months. The most common adverse effects were cushingoid features seen in 26(65%) and cyclosporine related hypertrichosis in 34(85%). Conclusion: Management of idiopathic steroid resistant nephrotic syndrome in children with a combination of cyclosporine and prednisolone provided good results as response to treatment was seen in 80% patients. (author)

  9. Do Not Forget Nephrotic Syndrome as a Cause of Increased Requirement of Levothyroxine Replacement Therapy.

    Science.gov (United States)

    Benvenga, Salvatore; Vita, Roberto; Di Bari, Flavia; Fallahi, Poupak; Antonelli, Alessandro

    2015-06-01

    Nephrotic syndrome increases L-thyroxine requirements because of urinary loss of free and protein-bound thyroid hormones. We report 2 hypothyroid patients referred to us because of high serum TSH, even though the L-thyroxine daily dose was maintained at appropriate levels or was increased. The cause of nephrotic syndrome was multiple myeloma in one patient and diabetic glomerulosclerosis in the other patient. As part of the periodic controls for diabetes, urinalysis was requested only in the second patient so that proteinuria could be detected. However, as in the first patient, facial puffiness and body weight increase were initially attributed to hypothyroidism, which was poorly compensated by L-thyroxine therapy. In the first patient, the pitting nature of the pedal edema was missed at the initial examination. An endocrinologist consulted over the phone by the practitioner hypothesized some causes of intestinal malabsorption of L-thyroxine. This diagnosis would have been accepted had the patient continued taking a known sequestrant of L-thyroxine, i.e. calcium carbonate. The diagnostic workup of patients with increasing requirements of L-thyroxine replacement therapy should not be concentrated on the digestive system alone. Careful history taking and physical examination need to be thorough. Endocrinologists should not forget nephrotic syndrome that, in turn, can be secondary to serious diseases.

  10. Nephrotic syndrome in dogs: clinical features and evidence-based treatment considerations.

    Science.gov (United States)

    Klosterman, Emily S; Pressler, Barrak M

    2011-08-01

    Nephrotic syndrome (NS), defined as the concurrent presence of hypoalbuminemia, proteinuria, hyperlipidemia, and fluid accumulation in interstitial spaces and/or body cavities, is a rare complication of glomerular disease in dogs, cats, and people. Affected animals frequently have markedly abnormal urine protein:creatinine ratios because of urinary loss of large amounts of protein; however, hypoalbuminemia-associated decreased plasma oncotic pressure is insufficient to explain fluid extravasation in most laboratory models, and, instead, either aberrant renal tubule retention of sodium with resultant increase in hydrostatic pressure or a systemic increase in vascular permeability may be the primary defects responsible for development of NS. Factors associated with NS in people (including "nephrotic-range" serum albumin concentration and urine protein concentration, and particular glomerular disease subtypes) have been assumed previously to also be important in dogs, although descriptions were limited to those patients included in case series of glomerular disease, and sporadic case reports. However, case-control comparison of larger cohorts of dogs with nephrotic versus nonnephrotic glomerular disease more recently suggests that predisposing factors and concurrent clinicopathologic abnormalities differ from those typically encountered in people with nephrotic syndrome, although case progression and negative effect on patient outcome are similar. This article briefly reviews major current theories and supporting evidence on the pathogenesis of NS, followed by an overview on the clinical features of this syndrome in dogs with glomerular disease. The authors also offer evidence-based and experience-based treatment recommendations that are based on minimizing the suspected dysregulation of the renin-angiotensin-aldosterone axis in affected dogs. PMID:21782144

  11. Protein abundance of urea transporters and aquaporin 2 change differently in nephrotic pair-fed vs. non-pair-fed rats

    OpenAIRE

    Bou Matar, Raed N.; Malik, Bela; Wang, Xiaonan H.; Martin, Christopher F; Eaton, Douglas C.; Sands, Jeff M.; Klein, Janet D.

    2012-01-01

    Salt and water retention is a hallmark of nephrotic syndrome (NS). In this study, we test for changes in the abundance of urea transporters, aquaporin 2 (AQP2), Na-K-2Cl cotransporter 2 (NKCC2), and Na-Cl cotransporter (NCC), in non-pair-fed and pair-fed nephrotic animals. Doxorubicin-injected male Sprague-Dawley rats (n = 10) were followed in metabolism cages. Urinary excretion of protein, sodium, and urea was measured periodically. Kidney inner medulla (IM), outer medulla, and cortex tissue...

  12. Systematic biomarker discovery and coordinative validation for different primary nephrotic syndromes using gas chromatography-mass spectrometry.

    Science.gov (United States)

    Lee, Jung-Eun; Lee, Yu Ho; Kim, Se-Yun; Kim, Yang Gyun; Moon, Ju-Young; Jeong, Kyung-Hwan; Lee, Tae Won; Ihm, Chun-Gyoo; Kim, Sooah; Kim, Kyoung Heon; Kim, Dong Ki; Kim, Yon Su; Kim, Chan-Duck; Park, Cheol Whee; Lee, Do Yup; Lee, Sang-Ho

    2016-07-01

    The goal of this study is to identify systematic biomarker panel for primary nephrotic syndromes from urine samples by applying a non-target metabolite profiling, and to validate their utility in independent sampling and analysis by multiplex statistical approaches. Nephrotic syndrome (NS) is a nonspecific kidney disorder, which is mostly represented by minimal change disease (MCD), focal segmental glomerulosclerosis (FSGS), and membranous glomerulonephritis (MGN). Since urine metabolites may mirror disease-specific functional perturbations in kidney injury, we examined urine samples for distinctive metabolic changes to identify biomarkers for clinical applications. We developed unbiased multi-component covarianced models from a discovery set with 48 samples (12 healthy controls, 12 MCD, 12 FSGS, and 12 MGN). To extensively validate their diagnostic potential, new batch from 54 patients with primary NS were independently examined a year after. In the independent validation set, the model including citric acid, pyruvic acid, fructose, ethanolamine, and cysteine effectively discriminated each NS using receiver operating characteristic (ROC) analysis except MCD-MGN comparison; nonetheless an additional metabolite multi-composite greatly improved the discrimination power between MCD and MGN. Finally, we proposed the re-constructed metabolic network distinctively dysregulated by the different NSs that may deepen comprehensive understanding of the disease mechanistic, and help the enhanced identification of NS and therapeutic plans for future. PMID:27247212

  13. Urinary retinol-binding protein as a prognostic marker in the treatment of nephrotic syndrome.

    Science.gov (United States)

    Mastroianni Kirsztajn, G; Nishida, S K; Silva, M S; Ajzen, H; Pereira, A B

    2000-10-01

    We studied the urinary levels of retinol-binding protein (urRBP), an index of proximal tubular dysfunction, in patients with nephrotic syndrome before and approximately 2 months after the beginning of steroid therapy as a predictor of response to therapy which included for some patients courses of immunosuppressive drugs. Those patients with minimal-change disease, mesangial proliferative glomerulonephritis, and focal-segmental glomerulosclerosis who had normal pretreatment urRBP levels were responsive to treatment; occasionally, responsive patients had an initially elevated urRBP level which normalized during treatment. Contrariwise, those patients with abnormally high levels of urRBP which did not normalize during treatment did not respond to treatment. The chance of a patient with minimal-change disease, mesangial proliferative glomerulonephritis, or focal-segmental glomerulosclerosis and a pretreatment urRBP level equal to or >1.0 mg/l being resistant to steroid treatment is 30 times that of a patient with a urRBP level <1.0 mg/l and even higher, if we consider the levels obtained during treatment. PMID:11014978

  14. [Longitudinal study in children with the nephrotic syndrome and minimal glomerular lesion].

    Science.gov (United States)

    Martini, R J; Gomila, A; Solanas, G

    1977-01-01

    Twenty-one children with idiopathic nephrotic syndrome and minimal changes on renal biopsy were followed during all the disease. The average of follow-up was 37 months, with a range from 12 to 124 months. Recurrent proteinuria was the most important feature during the follow-up; 14 out of the patients showed frequent relapses, but only 2 patients showed major complication (peritonitis, septicemia) during relapses. Frequent relapses appeared most frequently in patients who began the disease before their fourth birthday, showed allergic history, had hypertension and red blood cells in urine, or had recurrent infections and finally, in those where proteinuria reappeared soon after prednisone therapy was ended. Prednisone alone was successful to induce remission, but it did not prevent frequent relapses. The association clorambucil-prednisone allowed lengthening of the period of remission and possibly for this reason the rate of relapses fell during the first 37 months of the follow-up. There are no signs which permit to predict the length of the disease and the frequent relapses can occur even after many years from the beginning of the disease. Special care of these patients avoids major complications. PMID:758027

  15. Primary Nephrotic Syndrome in Adults as a Risk Factor for Pulmonary Embolism: An Up-to-Date Review of the Literature

    Directory of Open Access Journals (Sweden)

    Aibek E. Mirrakhimov

    2014-01-01

    Full Text Available Patients with nephrotic syndrome are at an increased risk for thrombotic events; deep venous thrombosis, renal vein thrombosis, and pulmonary embolism are quite common in patients with nephrotic syndrome. It is important to note that nephrotic syndrome secondary to membranous nephropathy may impose a greater thrombotic risk for unclear reasons. Increased platelet activation, enhanced red blood cell aggregation, and an imbalance between procoagulant and anticoagulant factors are thought to underlie the excessive thrombotic risk in patients with nephrotic syndrome. The current scientific literature suggests that patients with low serum albumin levels and membranous nephropathy may benefit from primary prophylactic anticoagulation. A thorough approach which includes accounting for all additional thrombotic risk factors is, therefore, essential. Patient counseling regarding the pros and cons of anticoagulation is of paramount importance. Future prospective randomized studies should address the question regarding the utility of primary thromboprophylaxis in patients with nephrotic syndrome.

  16. Comparison of three methods for isolation of urinary microvesicles to identify biomarkers of nephrotic syndrome.

    Science.gov (United States)

    Rood, Ilse M; Deegens, Jeroen K J; Merchant, Michael L; Tamboer, Wim P M; Wilkey, Daniel W; Wetzels, Jack F M; Klein, Jon B

    2010-10-01

    Urinary microvesicles, such as 40-100 nm exosomes and 100-1000 nm microparticles, contain many proteins that may serve as biomarkers of renal disease. Microvesicles have been isolated by ultracentrifugation or nanomembrane ultrafiltration from normal urine; however, little is known about the efficiency of these methods in isolating microvesicles from patients with nephrotic-range proteinuria. Here we compared three techniques to isolate microvesicles from nephrotic urine: nanomembrane ultrafiltration, ultracentrifugation, and ultracentrifugation followed by size-exclusion chromatography (UC-SEC). Highly abundant urinary proteins were still present in sufficient quantity after ultrafiltration or ultracentrifugation to blunt detection of less abundant microvesicular proteins by MALDI-TOF-TOF mass spectrometry. The microvesicular markers neprilysin, aquaporin-2, and podocalyxin were highly enriched following UC-SEC compared with preparations by ultrafiltration or ultracentrifugation alone. Electron microscopy of the UC-SEC fractions found microvesicles of varying size, compatible with the presence of both exosomes and microparticles. Thus, UC-SEC following ultracentrifugation to further enrich and purify microparticles facilitates the search for prognostic biomarkers that might be used to predict the clinical course of nephrotic syndrome. PMID:20686450

  17. Oxidative Stress and Nuclear Factor κB (NF-κB) Increase Peritoneal Filtration and Contribute to Ascites Formation in Nephrotic Syndrome.

    Science.gov (United States)

    Udwan, Khalil; Brideau, Gaëlle; Fila, Marc; Edwards, Aurélie; Vogt, Bruno; Doucet, Alain

    2016-05-20

    Water accumulation in the interstitium (edema) and the peritoneum (ascites) of nephrotic patients is classically thought to stem from the prevailing low plasma albumin concentration and the decreased transcapillary oncotic pressure gradient. However, several clinical and experimental observations suggest that it might also stem from changes in capillary permeability. We addressed this hypothesis by studying the peritoneum permeability of rats with puromycin aminonucleoside-induced nephrotic syndrome. The peritoneum of puromycin aminonucleoside rats displayed an increase in the water filtration coefficient of paracellular and transcellular pathways, and a decrease in the reflection coefficient to proteins. It also displayed oxidative stress and subsequent activation of NF-κB. Scavenging of reactive oxygen species and inhibition of NF-κB prevented the changes in the water permeability and reflection coefficient to proteins and reduced the volume of ascites by over 50%. Changes in water permeability were associated with the overexpression of the water channel aquaporin 1, which was prevented by reactive oxygen species scavenging and inhibition of NF-κB. In conclusion, nephrotic syndrome is associated with an increased filtration coefficient of the peritoneum and a decreased reflection coefficient to proteins. These changes, which account for over half of ascite volume, are triggered by oxidative stress and subsequent activation of NF-κB. PMID:27033704

  18. Giant Cell Arteritis in a 12-Year-Old Girl Presenting with Nephrotic Syndrome

    Directory of Open Access Journals (Sweden)

    Zeinab A. El-Sayed

    2014-01-01

    Full Text Available Giant cell arteritis (GCA is rare in children. The kidneys are generally spared. We present a case of GCA in a 12-year-old girl with severe headache and tender scalp especially over the right temporal area. The right superficial temporal artery was cord like and nodular and the pulsations were barely felt. Several small tender nodular swellings were felt in the occipital area. She had been previously diagnosed as a case of nephrotic syndrome due to underlying membranoproliferative glomerulonephritis. This report is aimed at drawing attention to this rare form of vasculitis in children aiming at decreasing its morbidities.

  19. Remission of nephrotic syndrome diminishes urinary plasmin content and abolishes activation of ENaC

    DEFF Research Database (Denmark)

    Andersen, René F; Buhl, Kristian Bergholt; Jensen, Boye L.;

    2013-01-01

    BACKGROUND: Urinary plasmin activates the epithelial Na(+) channel (ENaC) in vitro and may possibly be a mechanism of sodium retention in nephrotic syndrome (NS). This study used a paired design to test the hypothesis that remission of NS is associated with a decreased content of urinary plasmin...... and reduced ability of patients' urine to activate ENaC. METHODS: Samples were collected during active NS and at stable remission from 20 patients with idiopathic NS, aged 9.1 ± 3.2 years. Plasminogen-plasmin concentration was measured with an enzyme-linked immunosorbent assay. Western immunoblotting...

  20. Long-term Effect of TCM Decoctions in Treatment of Nephrotic Syndrome

    Institute of Scientific and Technical Information of China (English)

    魏连波; 叶任高; 栾图; 吕瑞和; 陈保田

    2002-01-01

    Fifty-seven cases of nephrotic syndrome were treated with TCM decoctions as accessory treatment for prednisone and cyclophosphamide, and the effects were observed in a follow-up period of 5-15 years. The long-term complete remission rate of 68.4% and recurrence rate of 26.3% in the treatment group were respectively higher and lower than those in the control group (P<0.01, and P<0.01). The results suggested that the TCM decoctions were very helpful in treating this condition.

  1. 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.

  2. Selected thrombosis and atherosclerosis risk factors in children with idiopathic nephrotic syndrome

    Directory of Open Access Journals (Sweden)

    Beata Bieniaś

    2012-04-01

    Full Text Available The purpose of our study was to evaluate selected thrombosis and atherosclerosis risk factors in children with idiopathic nephrotic syndrome (INS at three  stages of the disease (I – in acute phase before steroid therapy, II – during steroid therapy after resolution of proteinuria, III – in remission after completion of steroid therapy.In all children, serum total homocysteine, lipoprotein (a, total cholesterol, low-density lipoprotein cholesterol, high-density lipoprotein cholesterol and triglycerides levels were measured at three stages of the disease. Plasma antithrombin III, fibrinogen and D-dimer levels were also determined.  At all stages of INS, the serum t-HCY levels were similar and significantly higher than in controls.  Serum lipoprotein (a level, plasma antithrombin III, fibrinogen and D-dimer levels were significantly higher at stage I than at stages II, III and controls.In conclusion, children with INS are at high risk of thrombosis and atherosclerosis. Keywords: Idiopathic nephrotic syndrome, Homocysteine, Lipoprotein (a, Antithrombin III, fibrinogen, D-dimer

  3. Aberrant glomerular filtration of urokinase-plasminogen activator in nephrotic syndrome leads to amiloride-sensitive plasminogen activation in urine

    DEFF Research Database (Denmark)

    Stæhr, Mette; Buhl, Kristian Bergholt; Andersen, René F;

    2015-01-01

    In nephrotic syndrome, aberrant glomerular filtration of plasminogen and conversion to active plasmin in pre-urine is thought to activate proteolytically ENaC and contribute to sodium retention and edema. The ENaC blocker amiloride is an off-target inhibitor of urokinase-type plasminogen activator...

  4. Clinical Observation of Tiaojining (调激宁) Granule Combined with Corticosterone in Treating Infantile Primary Nephrotic Syndrome

    Institute of Scientific and Technical Information of China (English)

    2000-01-01

    In order to observe the effect of Tiaojining granule (调激宁, TJNG) combined with corticosteroids (CS) to enhance the efficacy and alleviate the side-effects, the authors used the combination therapy of TJNG and CS in treating infantile primary nephrotic syndrome (IPNS) in 1990-1996, and the following is the summary of clinical data.

  5. The treatment of relapsing primary nephrotic syndrome in children

    Institute of Scientific and Technical Information of China (English)

    WANG Ya-ping; LIU Ai-min; DAI Yu-wen; YANG Cheng; TANG Hong-feng

    2005-01-01

    Objective: To explore better therapy and reduce the rate of re-relapse of primary nephritic syndrome in children who had been treated with corticosteroids but relapsed. Methods: Eighty relapsers were enrolled from Jan. 1994 to Apr. 2000, who were randomly divided into two groups. The treatment group (n=39) had been treated with tripterysium glucosides for three months,with the control group (n=41) members were treated with cyclophosphmide (CTX) by intermission intravenous pulse, with total dose of CTX not being more than 150 mg/kg. Prednisone, meanwhile, was given to both groups. The total treatment period of prednisone was prolonged by 12-18 months. Results: After following up for 3-7 years, the re-relapse rates of both groups were observed. The re-relapse rate of the treatment group was 28.2% to 29.3% in the CTX-controlled group. The re-relapse rates between two groups were almost similar, and with no observed significant difference (P>0.05). The side effect of tripterysium glucosides was less than that of CTX. Conclusion: For the treatment of relapsing nephritic syndrome in children, the combination of tripterysium glucosides and prolonged corticosteroid therapy is as effective as the regimen of CTX plus prolonged use of prednisone.

  6. Hydroxyurea for Treatment of Nephrotic Syndrome Associated With Polycythemia Vera.

    Science.gov (United States)

    Hundemer, Gregory L; Rosales, Ivy A; Chen, Yi-Bin; Colvin, Robert B; Tolkoff-Rubin, Nina E

    2016-09-01

    Myeloproliferative disorders are a rare cause of focal segmental glomerulosclerosis (FSGS), although the mechanism is unclear. Hydroxyurea is commonly used in these disorders for its cytoreductive properties; however, the effect of this treatment on proteinuria or kidney function remains unclear in cases of myeloproliferative disorder-associated FSGS. We describe the clinical course of a patient with polycythemia vera and nephrotic-range proteinuria, demonstrated to have FSGS on biopsy. The patient had a distant history of granulomatosis with polyangiitis (Wegener's), for which he routinely had his kidney function and proteinuria measured, allowing for early detection of nephrotic syndrome soon after being diagnosed with polycythemia vera. Treatment with hydroxyurea resulted in rapid improvement in proteinuria that correlated with a decrease in hematocrit. This response was replicated 2 additional times when the patient was taken off and then restarted on hydroxyurea therapy. He now maintains a steady dose of hydroxyurea with favorable kidney measures (proteinuria with <1g/d of protein excretion and serum creatinine of 1.27mg/dL [corresponding to estimated glomerular filtration rate of 56mL/min/1.73 m(2)]). This case suggests that early screening and treatment for myeloproliferative disorder-associated FSGS may lead to improved long-standing kidney function. PMID:27133437

  7. Expression of glomerular ecto-ATPase in idiopathic nephrotic syndrome

    NARCIS (Netherlands)

    Cheung, PK; Baller, JFW; vanderHorst, MLC; Bakker, WW; Plesner, L; Kirley, TL; Knowles, AF

    1997-01-01

    The pathogenesis of glomerular alterations leading to increased glomerular permeability in disorders like Minimal Change Disease (MCD) is obscure. One of the preliminary observed glomerular alterations in MCD involves diminished expression of glomerular ecto-ATP-diphosphohydrolase (denoted as ecto-A

  8. Unusual pediatric co-morbility: autoimmune thyroiditis and cortico-resistant nephrotic syndrome in a 6-month-old Italian patient

    Directory of Open Access Journals (Sweden)

    Urbano Flavia

    2012-10-01

    Full Text Available Abstract We report on a case of autoimmune thyroiditis in a 6-month-old patient with cortico-resistant nephrotic syndrome. Normal serum levels of thyroid hormons and thyroid-stimulating hormone were detected with high titers of circulant antithyroid antibodies and a dysomogeneous ultrasound appearance of the gland, typical of autoimmune thyroiditis. The research of maternal thyroid antibodies was negative. This is the first case of autoimmune thyroiditis found in such a young patient with pre-existing nephrotic syndrome ever described in literature. This association is random because nephrotic syndrome does not have an autoimmune pathogenesis and the genes involved in autoimmune thyroiditis are not related to those of nephrotic syndrome.

  9. Albumin and Furosemide Combination for Management of Edema in Nephrotic Syndrome: A Review of Clinical Studies

    Directory of Open Access Journals (Sweden)

    Margaret Duffy

    2015-10-01

    Full Text Available The treatment of edema in patients with nephrotic syndrome is generally managed by dietary sodium restriction and loop diuretics. However, edema does not improve in some patients despite adequate sodium restriction and maximal dose of diuretics. In such patients, combination of albumin and a loop diuretic may improve edema by diuresis and natriuresis. The response to this combination of albumin and a diuretic has not been observed in all studies. The purpose of this review is to discuss the physiology of diuresis and natriuresis of this combination therapy, and provide a brief summary of various studies that have used albumin and a loop diuretic to improve diuretic-resistant edema. Also, the review suggests various reasons for not observing similar results by various investigators.

  10. Nephrotic Syndrome Following H1N1 Influenza in a 3-Year-Old Boy

    Directory of Open Access Journals (Sweden)

    Pio Liberatore

    2012-06-01

    Full Text Available Background: The pandemic influenza A/H1N1, spread through the world in 2009, producing a serious epidemic in Italy. Complications are generally limited to patients at the extremes of age (65years and those with comorbid medical illness. The most frequent complications of influenza involve the respiratory system.Case Presentation: A 3-year-old boy with a recent history of upper respiratory tract infection developed a nephrotic syndrome. Together with prednisone, furosemide and albumin bolus, a therapy with oseltamivir was started since the nasopharyngeal swab resulted positive for influenza A/H1N1. Clinical conditions andlaboratory findings progressively improved during hospitalization, becoming normal during a 2 month follow up.Conclusion: The possibility of a renal involvement after influenza A/H1N1 infection should be considered.

  11. Clinical Study of Gushen Tablet(固肾片) in Reducing Children's Nephrotic Syndrome Relapse

    Institute of Scientific and Technical Information of China (English)

    云鹰; 高雅; 马玉宏; 刘望乐; 赵蒙; 高智铭

    2003-01-01

    Objective: To explore the effect of Gushen tablet (固肾片, GST) in reducing the relapse of children′s nephrotic syndrome and the possible mechanism of drugs used. Methods: Fifty children with primary nephrotic syndrome who had been induced and alleviated with regular glucocorticoid (GC) were randomly divided into two groups: the GST group used GST and standard middle-long term course of GC,and the control group adopted standard middle-long term course of GC and immunoinhibitory or immunomodulatory agents for treatment. The 0.5, 1 and 2 years after the treatment the relapse episodes, time for urinary protein negative conversion after relapse, the episodes of patienfs infection and relapse after infection were evaluated. Before and after treatment the plasma cortisol and T lymphocyte subpopulation were determined. Results: The relapse rate of GST group: the rates after 0.5, 1, 2 years were 20.0 %, 30.0 %and 40.9%, and the frequent relapse rate were 0, 6.7% and 9.2% respectively, which were lower than those of control group (60.0%, 70. 0%, 69.2% and 25.0%, 15.0%, 15.4% respectively) ; in the GST group no relapse occurred within 0. 5 year, the relapse rate after 1 and 2 years reduced by 40. 0% and 28.3%, compared with those of the control group (all P<0.05) ; during the observation period, the mean infection/every child patient was 1.86 episodes in GST group, after infection the nephrotic relapse rate was 28. 3%, which was lower than that of the control group (2.25 episodes, 71. 1%, P<0. 05); the relapse per patient in GST group was 0.8 episodes, time for urinary protein negative conversion was 12.00±8.98 days, lower than those of control group (1.6 episodes, 20.75±11.95 days, P<0.05) ; 3 months after GST treatment the plasma cortisol level normalized, and the CD4/CD8 ratio elevated (P<0. 05). Conclusion: GST could possibly reduce the relapse of children nephrosis, and the frequent relapse and relapse episodes, and the time for post-relaptic urinary

  12. TRPC6 gene promoter polymorphisms in steroid resistant nephrotic syndrome children

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    Mahesh Kumar Kempanahalli Basappa

    2015-04-01

    Full Text Available Introduction: Nephrotic syndrome (NS is the most frequent cause of proteinuria in children and is emerging as a leading cause of uremia. Among idiopathic NS, 10% of children do not respond to steroids or to any other immunosuppressive therapy, and progress to end-stage renal disease (ESRD. Several studies have investigated the mutations in genes encoding podocyte proteins and their possible associations with several forms of hereditary NS. Objectives: The present study aimed to determine the distribution of the TRPC6 gene promoter polymorphisms in subjects with features of steroid resistant nephrotic syndrome (SRNS and controls. Patients and Methods: About 49 unrelated patients with SRNS and 45 age matched controls no renal or other disorders were included in the study. PCR-RFLP was used for genotyping rs3824934 (-254C>G and rs56134796 (-218C>T polymorphisms located in TRPC6 gene promoter region. Results: Both -254C>G and -218C>T are polymorphic in both SRNS patients and controls. No statistically significant differences in genotypes or allele frequencies between SRNS patients and controls were observed. Linkage disequilibrium was not strong and significant and haplotypes were not associated with SRNS. Interaction analysis by multifactor dimensionality reduction (MDR revealed a significant interaction between -254G>C and -218C>T in <10 years age group. Conclusion: The results demonstrate that the TRPC6 polymorphisms do not affect susceptibility of SRNS in Indian population. Further replications, preferably a systematic search for TRPC6 functional variants that affect gene expression are desirable for validation of our findings.

  13. Correlation between lipid profile and C-reactive protein in children with nephrotic syndrome

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    Kurnia Dwi Astuti

    2016-07-01

    Full Text Available Background Nephrotic syndrome (NS causes dyslipidemia in children, which can be long term or intermittent. Dyslipidemia has long been established as a risk factor for atherosclerosis. An early sign of atherosclerosis is elevated high sensitivity C-reactive protien (hsCRP. Atherosclerosis early in life, especially in childhood, warrants an assessment for NS. Study on a correlation between lipid profile and hsCRP, as a marker of atherosclerosis, in pediatric NS patients has been limited. Objective To assess for a correlation between lipid profile and hsCRP in childhood nephrotic syndrome. Methods This cross-sectional study was undertaken on 29 children with NS in Dr. Kariadi Hospital. Serum hsCRP, total cholesterol, low-density lipoprotein (LDL, and high-density lipoprotein (HDL were examined in the active phase. Spearman’s test was used to analyze a possible correlation between total cholesterol, LDL, HDL and hsCRP levels. Results Mean levels of total cholesterol (454 mg/dL and LDL (288 mg/dL in this study were high, while the HDL level (55 mg/dL was normal, according to US Department of Health and Human Services classifications. The median hsCRP level was 0.33 mg/L and 9 (31% subjects had high hsCRP levels of more than 1 mg/L. There was a positive correlation between LDL level and hsCRP (r=0.423; P<0.05. Conclusions There is a weak positive correlation between LDL and hsCRP levels in children with NS.

  14. Correlation between lipid profile and C-reactive protein in children with nephrotic syndrome

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    Kurnia Dwi Astuti, Mohammad Heru Muryawan, Omega Mellyana

    2015-01-01

    Full Text Available Background Nephrotic syndrome (NS causes dyslipidemia in children, which can be long term or intermittent. Dyslipidemia has long been established as a risk factor for atherosclerosis. An early sign of atherosclerosis is elevated high sensitivity C-reactive protien (hsCRP. Atherosclerosis early in life, especially in childhood, warrants an assessment for NS. Study on a correlation between lipid profile and hsCRP, as a marker of atherosclerosis, in pediatric NS patients has been limited. Objective To assess for a correlation between lipid profile and hsCRP in childhood nephrotic syndrome. Methods This cross-sectional study was undertaken on 29 children with NS in Dr. Kariadi Hospital. Serum hsCRP, total cholesterol, low-density lipoprotein (LDL, and high-density lipoprotein (HDL were examined in the active phase. Spearman’s test was used to analyze a possible correlation between total cholesterol, LDL, HDL and hsCRP levels. Results Mean levels of total cholesterol (454 mg/dL and LDL (288 mg/dL in this study were high, while the HDL level (55 mg/dL was normal, according to US Department of Health and Human Services classifications. The median hsCRP level was 0.33 mg/L and 9 (31% subjects had high hsCRP levels of more than 1 mg/L. There was a positive correlation between LDL level and hsCRP (r=0.423; P<0.05. Conclusions There is a weak positive correlation between LDL and hsCRP levels in children with NS. [Paediatr Indones. 2015;55:1-6.].

  15. Nephrotic syndrome

    Science.gov (United States)

    ... the following: Antinuclear antibody Cryoglobulins Complement levels Glucose tolerance test Hepatitis B and C antibodies HIV test ... PhD, and the A.D.A.M. Editorial team. Related MedlinePlus Health Topics Kidney Diseases Browse the ...

  16. Study of oxidative stress, homocysteine, copper & zinc in nephrotic syndrome: therapy with antioxidants, minerals and B-complex vitamins

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    Jyoti Dwivedi*

    2009-09-01

    Full Text Available Oxidative damage has been proposed as one of the possiblemechanism involved in the nephrotic syndrome. Strengthening thedefense system by antioxidants may provide protection againstoxidative damage. Therefore, this study was carried out toinvestigate oxidant and antioxidant status with copper, zinc andhomocysteine in nephrotic syndrome patients and the effect ofantioxidants, minerals and B-complex vitamins on oxidant andantioxidant status. The blood samples were analyzed for quantitationof malondialdehyde as index of lipid peroxide, vitamin C, totalantioxidant capacity, copper, zinc, and homocysteine. Significantlyincreased levels of serum lipid peroxide, homocysteine anddecreased levels of serum total antioxidant capacity, copper, zincand plasma vitamin C were noticed in the patients with nephroticsyndrome as compared to control subjects. However, significantreduction in lipid peroxide, homocysteine and improvement invitamin C, total antioxidant capacity, copper, and zinc activity wereobserved after treatment of antioxidants and minerals with Bcomplexvitamins.

  17. Sustained Remission of Antineutrophil Cytoplasmic Antibody-Mediated Glomerulonephritis and Nephrotic Syndrome in Mixed Connective Tissue Disease

    OpenAIRE

    Konstantinov, Konstantin N.; Harris, Alexis A.; Barry, Marc; Murata, Glen H; Tzamaloukas, Antonios H.

    2013-01-01

    A woman diagnosed with mixed connective tissue disease (MCTD) developed an anti-myeloperoxidase (MPO) antineutrophil cytoplasmic antibody (ANCA) and nephrotic syndrome with normal serum creatinine. Percutaneous kidney biopsy showed pauci-immune glomerulonephritis with superimposed immune complex deposition. After treatment with cyclophophamide and prednisone, proteinuria decreased progressively to a level of 0.4 g/g creatinine, ANCA became undetectable, while serum creatinine remained normal ...

  18. Prediction of Negative Conversion Days of Childhood Nephrotic Syndrome Based on the Improved Backpropagation Neural Network with Momentum

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    Yi-jun Liu

    2015-12-01

    Full Text Available Childhood nephrotic syndrome is a chronic disease harmful to growth of children. Scientific and accurate prediction of negative conversion days for children with nephrotic syndrome offers potential benefits for treatment of patients and helps achieve better cure effect. In this study, the improved backpropagation neural network with momentum is used for prediction. Momentum speeds up convergence and maintains the generalization performance of the neural network, and therefore overcomes weaknesses of the standard backpropagation algorithm. The three-tier network structure is constructed. Eight indicators including age, lgG, lgA and lgM, etc. are selected for network inputs. The scientific computing software of MATLAB and its neural network tools are used to create model and predict. The training sample of twenty-eight cases is used to train the neural network. The test sample of six typical cases belonging to six different age groups respectively is used to test the predictive model. The low mean absolute error of predictive results is achieved at 0.83. The experimental results of the small-size sample show that the proposed approach is to some degree applicable for the prediction of negative conversion days of childhood nephrotic syndrome.

  19. Pattern of steroid resistant nephrotic syndrome in children living in the kingdom of Saudi Arabia: A single center study

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    Kari Jameela

    2009-01-01

    Full Text Available Steroid resistant nephrotic syndrome (SRNS remains a challenge facing pediatric nephrologists. The underlying histopathology usually affects the course of the disease and the response to treatment. We studied the pattern of histopathology in children with SRNS who presented to the King Abdul Aziz University Hospital (KAUH, Jeddah, Saudi Arabia. The records of all children with primary SRNS, who were seen between 2002 and 2007 were reviewed. Only patients who had undergone a renal biopsy were included in the study. The histopathology slides were reviewed by two renal pathologists independently. Patients with congenital nephrotic syndrome, lupus or sickle cell disease, were excluded from the study. Thirty-six children fulfilled the inclusion criteria, and included 25 girls and 11 boys with female to male ratio of 2.3:1. Fifty percent of the children (n=18 were Saudi and the remaining 50% were from various other racial backgrounds (9 Asians, 4 Arabs, 2 Africans and 3 from the Far East. Their mean age at presentation was 4.3 ± 3.0 years (range 1-12 years. The mean serum albumin at presentation was 15.6 ± 7.1 g/L and all of them had 4+ proteinuria on urinalysis. Five children had elevated serum creatinine at presentation while the mean serum creatinine was 50.4 ± 45.6 µmol/L. Three children had low serum complement levels at presentation and none were positive for hepatitis B surface antigen or antinuclear antibody (ANA. The renal histopathology was compatible with focal and segmental glomerulosclerosis (FSGS in 39% (n=14, IgM nephro-pathy in 28% (n=10, mesengioproliferative glomerulonephritis (MesPGN in 17% (n=6, mini-mal change disease (MCD and C1q nephropathy (C1qNP in 8% each (n=3 + 3 and IgA nephro-pathy in 3% (n=1. Our retrospective review shows that FSGS was the commonest underlying histopathology in children who presented with SRNS followed by IgM nephropathy and other variants of MCD such as MesPGN. C1qNP was the underlying cause in some

  20. [Pefloxacin as a first-line treatment for nephrotic syndrome in minimal glomerular lesions in the adult. Multicenter study of 32 patients].

    Science.gov (United States)

    Pruna, A; Barka, A; Nochy, D; Hauet, T; Boulanger, H; Landais, P

    1997-01-01

    Minimal change nephrotic syndrome (MCNS) is the most frequent single cause of nephrotic syndrome occurring both in adults and children. Although it appears to be a self-limiting disorder (10% spontaneous remissions within the fortnight following the initial flare), MCNS displays a high rate of complications during the nephrotic period (10 to 15% cases) and prompts one to treat patients as early as possible. Corticosteroids are currently used as first-line treatment. A 16 weeks full-dose steroid course (1 mg/kg/day) usually induces remission in 75% MCNS in adults. Nevertheless, duration of treatment (9 months) and occurrence of relapses despite a slowly tapering dosage schedule, expose patients to steroids side-effects. Immunosuppressive drugs are recommended in case of steroid resistance and their side-effects are not harmless. Therefore, an alternative to steroids or immunosuppressives would lend a serious helping hand in MCNS management. The present work is dealing with pefloxacin efficacy in 40% MCNS in adults. Thirty-two MCNS adult patients were treated in a national multicenter study. A short-duration pefloxacin course (4 to 6 weeks) allowed partial or complete remission in 13 out of 32 cases. Thus far, this effect was undescribed for this class of drugs. Pefloxacin belongs to antibacterial agents of the fluoroquinolone family and is active against Gram negative Enterobacteria species. Fluoroquinolones also act on eukaryotic cells as lymphocytes and chondrocytes and alter IL2, gamma IFN and integrin expression. Although their precise mode of action is unknown in this kind of immunological disorder, fluoroquinolones might represent an alternative to steroids in some adult form of MCNS. However, predictive criteria for sensitivity to fluoroquinolones are currently not available and further controlled studies would be helpful using fluoroquinolones as first-line treatment in all the MCNS. PMID:9297136

  1. A Prospective Observational Survey on the Long-Term Effect of LDL Apheresis on Drug-Resistant Nephrotic Syndrome

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    Eri Muso

    2015-08-01

    Full Text Available Background/Aims: LDL apheresis (LDL-A is used for drug-resistant nephrotic syndrome (NS as an alternative therapy to induce remission by improvement of hyperlipidemia. Several clinical studies have suggested the efficacy of LDL-A for refractory NS, but the level of evidence remains insufficient. A multicenter prospective study, POLARIS (Prospective Observational Survey on the Long-Term Effects of LDL Apheresis on Drug-Resistant Nephrotic Syndrome, was conducted to evaluate its clinical efficacy with high-level evidence. Methods: Patients with NS who showed resistance to primary medication for at least 4 weeks were prospectively recruited to the study and treated with LDL-A. The long-term outcome was evaluated based on the rate of remission of NS 2 years after treatment. Factors affecting the outcome were also examined. Results: A total of 58 refractory NS patients from 40 facilities were recruited and enrolled as subjects of the POLARIS study. Of the 44 subjects followed for 2 years, 21 (47.7% showed remission of NS based on a urinary protein (UP level Conclusions: Almost half of the cases of drug-resistant NS showed remission 2 years after LDL-A. Improvement of nephrotic parameters at termination of the LDL-A treatment was a predictor of a favorable outcome.

  2. Polimiosite associada à síndrome nefrótica Polymyositis associated with nephrotic syndrome

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    Renato Oliveira Freire

    2010-08-01

    Full Text Available A polimiosite (PM é uma doença sistêmica do grupo das miopatias inflamatórias idiopáticas, clinicamente caracterizada por fraqueza muscular proximal e simétrica. Há relatos na literatura de PM associada a neoplasias, doenças autoimunes e infecções virais. Entretanto, a associação entre PM e nefropatia não é frequente. Descrevemos o caso de um paciente com polimiosite que evoluiu com síndrome nefrótica devido à presença de glomerulonefrite mesangialPolymyositis (PM is a systemic disease of the idiopathic inflammatory myopathy group, clinically characterized by symmetric and proximal muscle weakness. There are reports in literature of PM associated with malignancies, autoimmune diseases, and viral infections. However, the association between PM and nephropathy is not common. We describe a case report of a patient with polymyositis who developed nephrotic syndrome due to mesangial glomerulonephritis

  3. Relations of nuclear factor-kappa B activity in the kidney of children with primary nephrotic syndrome to clinical manifestations, pathological types, and urinary protein excretion

    Institute of Scientific and Technical Information of China (English)

    ZHAO Hong-yang; SUN Ruo-peng; DONG Jun-hua; ZHEN Jun-hui

    2005-01-01

    @@ The pathogenesis of childhood primary nephrotic syndrome (PNS) is unclear. However, an immune mechanism has generally been accepted as a cause. Imbalance of T lymphocyte and a variety of inflammatory cytokines, chemotactic and transcription factors are involved in the pathophysiology and manifestations of PNS,1,2 and nuclear factor kappa B (NF-κB) transcriptionally regulates the expression of these factors.3 Research has been focused on NF-κB and inflammatory regulated mediators of renal diseases, but seldom on different clinical manifestations and histopathological changes. In order to explore a potential mechanism for the pathogenesis of PNS in children and a basis for preventing its advance, we determined NF-κB activity in the kidney of children with PNS in vitro using immunohistochemical staining and the multimedia coloured pathological image analysis system and its relations to clinical manifestations, histopathological changes and 24-hour urinary protein excretion.

  4. Treatment of Severe Edema in Children with Nephrotic Syndrome with Diuretics Alone — A Prospective Study

    Science.gov (United States)

    Kapur, Gaurav; Valentini, Rudolph P.; Imam, Abubakr A.; Mattoo, Tej K.

    2009-01-01

    Background and objective: Severe edema in children with nephrotic syndrome (NS) may be associated with volume contraction (VC) or volume expansion (VE). Usually, severe edema in children is treated with intravenous (IV) albumin and diuretics, which is appropriate for VC patients. However, in VE patients, this can precipitate fluid overload. The objective of this study was to evaluate treatment of severe edema in NS with diuretics alone. Design, setting, participants, & measurements: Thirty NS patients with severe edema were enrolled in this prospective study in two phases. VC was diagnosed based on fractional excretion of sodium (FeNa) <1%. VC patients received IV albumin and furosemide. VE patients received IV furosemide and oral spironolactone. On the basis of phase 1 observations, FeNa <0.2% identified VC in 20 phase 2 patients. Results: All phase 1 patients had FeNa <1%. Phase 1 patients when reanalyzed based on a FeNa cutoff of 0.2%; it was noted that VC patients had higher BUN, BUN/creatinine ratio, urine osmolality, and lower FeNa and urine sodium compared with VE patients. Similar results were observed in phase 2. VC patients had significantly higher renin, aldosterone, and antidiuretic hormone levels. In phase 2, 11 VE patients received diuretics alone and 9 VC patients received albumin and furosemide. There was no difference in hospital stay and weight loss in VC and VE groups after treatment. Conclusions: FeNa is useful in distinguishing VC versus VE in NS children with severe edema. The use of diuretics alone in VE patients is safe and effective. PMID:19406963

  5. Recurrence of proteinuria following renal transplantation in congenital nephrotic syndrome of the Finnish type.

    Science.gov (United States)

    Srivastava, Tarak; Garola, Robert E; Kestila, Marjo; Tryggvason, Karl; Ruotsalainen, Vesa; Sharma, Mukut; Savin, Virginia J; Jalanko, Hannu; Warady, Bradley A

    2006-05-01

    We report a Caucasian boy of Italian descent with congenital nephrotic syndrome of the Finnish type (NPHS1, CNF, MIM 256300) who developed recurrence of proteinuria and hypoalbuminemia on the seventh post-operative day following living related renal transplantation from his paternal aunt. The allograft biopsy was normal except for effacement of podocyte foot processes on electron microscopy. He was treated by the substitution of mycophenolate mofetil with cyclophosphamide for 12 weeks, in addition to cyclosporine, prednisone and daclizumab. His proteinuria resolved quickly following the initiation of cyclophosphamide treatment, and he remains in remission 4 years after receiving his transplant. His native and allograft kidneys were evaluated for nephrin expression by immunohistochemistry, DNA analysis for the NPHS1 mutation, serum for the presence of auto-antibodies to nephrin by both enzyme-linked immunosorbent assay (ELISA) and fetal glomeruli immunofluorescence assay, and serum for glomerular permeability to albumin (Palb) activity using a functional in vitro assay for Palb. Nephrin expression was completely absent in the native kidney, while it was decreased in the allograft compared with normal. DNA analysis of the NPHS1 gene revealed mutations 3248G>T and 3250delG in exon 24, causing G1083V and 1084Vfs, respectively, inherited from his father, and 3478C>T in exon 27, that leads to R1160X, inherited from his mother. Serum was negative for auto-antibodies to nephrin. Interestingly, the Palb activity was increased at the time of recurrence of proteinuria following transplantation (Palb 0.73+/-0.10) and remained elevated when retested more than 3 years later (Palb 0.54+/-0.09). This is the first report of increased Palb activity in recurrence of proteinuria following transplantation in NPHS1. We speculate the role of increased Palb activity in the recurrence of proteinuria following transplantation in NPHS1. PMID:16518627

  6. Calcium and vitamin D supplementation in children with frequently relapsing and steroid-dependent nephrotic syndrome

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    Ayi Dilla Septarini

    2012-01-01

    Full Text Available Background Children with frequently relapsing and steroid-dependent nephrotic syndrome (FRNS/SDNS are at risk for osteoporosis due to impaired metabolism of calcium and vitamin D. Objective To determine the effect of calcium and vitamin D supplementation on bone mineral density, serum ionized calcium levels and serum 25-hydroxy-vitamin D levels in children with FRNS and SDNS. Methods A clinical trial with a before and after design was performed. Subjects were SDNS or FRNS pediatric patients ≥ 5 years of age. Subjects received 800 mg elemental calcium and 400 IU vitamin D supplementation for 8 weeks. Serum ionized calcium, serum 25-hydroxy-vitamin D [25(OHD], and bone mineral density (BMD were determined before and after the supplementation. Results Of the 30 subjects, 28 completed the study. However, only 20 subjects underwent BMD determination before and after supplementation. Oof the 28 subjects, 22 had hypocalcemia and 26 had low vitamin D levels. Osteopenia was found in 14/20 subjects and osteoporosis was in 2/20 subjects. After 8 weeks of supplementation, mean serum ionized calcium increased from low [1.15 mmol/L (SD 0.03] to normal [1.18 mmol/L (SD 0.04] (P<0.001 levels, but mean serum 25(OHD only increased from vitamin D deficiency category [20 ng/mL (SD 7.7] to vitamin D insufficiency category [25.5 ng/mL (7.7] (P=0.010. Mean z-score BMD increased from -1.1 (SD 0.9 to -0.7 (SD 0.2 after supplementation (P<0.001. Conclusion Calcium vitamin D supplementation effectively increased serum ionized calcium, serum 25(OHD, and BMD in subjects with FRNS and SDNS. [Paediatr Indones. 2012;52:16-21].

  7. A relationship between proteinuria and acute tubulointerstitial disease in rats with experimental nephrotic syndrome

    International Nuclear Information System (INIS)

    The relationship between tubulointerstitial nephritis and proteinuria was characterized in experimental nephrosis in rats. In one group, proteinuria induced by aminonucleoside of puromycin (PAN) was reduced by using an 8% protein diet and adding the angiotensin I-converting enzyme (ACE) inhibitor enalapril to the drinking water. Two control groups were injected with saline and PAN, respectively, and fed a 27% protein diet. The first group had significantly reduced albuminuria and a definite attenuation of tubular cell injury. There was a strong positive correlation between the number of interstitial macrophages and albuminuria. The beneficial effect was reproduced by dietary-protein restriction alone, whereas ACE inhibition alone had an insignificant effect on the degree of proteinuria. Depletion of circulating T lymphocytes in one group of nephrotic rats eliminated interstitial lymphocytes but did not affect interstitial macrophage influx. Inhibition of the in situ proliferation of resident interstitial macrophages by unilateral kidney irradiation failed to change the intensity of the macrophage infiltration. Treatment of rats with sodium maleate produced proximal tubular cell toxicity but interstitial inflammation did not develop, suggesting that the latter is not a nonspecific response to tubular injury. These studies demonstrate a strong relationship between tubulointerstitial nephritis and the severity of proteinuria in experimental nephrosis

  8. High absolute risks and predictors of venous and arterial thromboembolic events in patients with nephrotic syndrome: results from a large retrospective cohort study.

    NARCIS (Netherlands)

    Mahmoodi, B.K.; Kate, M.K. ten; Waanders, F.; Veeger, N.J.; Brouwer, J.L.; Vogt, L.; Navis, G.; Meer, J.W.M. van der

    2008-01-01

    BACKGROUND: No data are available on the absolute risk of either venous thromboembolism (VTE) or arterial thromboembolism (ATE) in patients with nephrotic syndrome. Reported risks are based on multiple case reports and small studies with mostly short-term follow-up. We assessed the absolute risk of

  9. Gene-level integrated metric of negative selection (GIMS) prioritizes candidate genes for nephrotic syndrome.

    Science.gov (United States)

    Sampson, Matthew G; Gillies, Christopher E; Ju, Wenjun; Kretzler, Matthias; Kang, Hyun Min

    2013-01-01

    Nephrotic syndrome (NS) gene discovery efforts are now occurring in small kindreds and cohorts of sporadic cases. Power to identify causal variants in these groups beyond a statistical significance threshold is challenging due to small sample size and/or lack of family information. There is a need to develop novel methods to identify NS-associated variants. One way to determine putative functional relevance of a gene is to measure its strength of negative selection, as variants in genes under strong negative selection are more likely to be deleterious. We created a gene-level, integrated metric of negative selection (GIMS) score for 20,079 genes by combining multiple comparative genomics and population genetics measures. To understand the utility of GIMS for NS gene discovery, we examined this score in a diverse set of NS-relevant gene sets. These included genes known to cause monogenic forms of NS in humans as well as genes expressed in the cells of the glomerulus and, particularly, the podocyte. We found strong negative selection in the following NS-relevant gene sets: (1) autosomal-dominant Mendelian focal segmental glomerulosclerosis (FSGS) genes (p = 0.03 compared to reference), (2) glomerular expressed genes (p = 4×10(-23)), and (3) predicted podocyte genes (p = 3×10(-9)). Eight genes causing autosomal dominant forms of FSGS had a stronger combined score of negative selection and podocyte enrichment as compared to all other genes (p = 1 x 10(-3)). As a whole, recessive FSGS genes were not enriched for negative selection. Thus, we also created a transcript-level, integrated metric of negative selection (TIMS) to quantify negative selection on an isoform level. These revealed transcripts of known autosomal recessive disease-causing genes that were nonetheless under strong selection. We suggest that a filtering strategy that includes measuring negative selection on a gene or isoform level could aid in identifying NS-related genes. Our GIMS and TIMS scores are

  10. Cyclosporine treatment in idiopathic membranous nephropathy nephrotic syndrome in adults: a retrospective study spanning 15 years

    Institute of Scientific and Technical Information of China (English)

    TAO Jian-ling; LIU Li-li; WEN Yu-bing; GAO Rui-tong; LI Hang; LI Ming-xi; LI Xue-mei; LI Xue-wang

    2011-01-01

    Background Cyclosporine is effective in treating nephrotic syndrome (NS) with idiopathic membranous nephropathy (IMN) in adults.But high relapse rate remains a major concern.The way to manipulate cyclosporine is inconclusive.The aim of this study was to introduce the way how to titrate the cyclosporine to maintain complete remission without relapse.Methods Patients with biopsy-proven IMN with NS treated with cyclosporine for at least 1 month from 1996 to 2011 at Peking Union Medical College Hospital were reviewed.Results Mean age of the 51 eligible patients was 52 years,with 39 men.Mean proteinuria was (7.47+3.14) g/d,serum albumin (24.50±6.29) g/L,and serum creatinine (82.62+21.18) μmol/L.Cyclosporine was commenced at a mean dose of (3.46±0.63) mg.kg1.d-1.Ora1 prednisone (0.40±0.29) mg·kg1·d-1 was given concomitantly in 38 patients.Cyclosporine was administered for a median of 16 months (range 1-93 months) and stopped in non-responders by month six.By month 3 (n=47),the number in complete remission (CR) and partial remission (PR) was 3 and 24,which shifted to 12 and 17 by month 6 (n=41).Male gender,heavy proteinuria,low serum albumin level,and high serum creatinine level were significant determinants in poor response by month six (P <0.05 in all variables compared with responders).There was a significant reversible serum creatinine increase within 25% during month 3 to 12 (P <0.05 in all variables compared with baseline value).Eleven patients maintained cyclosporine for more than 24 months with a cyclosporine dose of (1.04±1.06)mg·kg1·d-1.Nine patients were in CR.Renal function,systolic and diastolic blood pressure remained stable.Renal impairment (>30% rise of serum creatinine),secondary infection,hypertension,gingival hyperplasia and liver impairment occurred in 6,4,10,4,and 1 patients,respectively.Conclusions The observation time for cyclosporine to effectively induce CR of NS in IMN adults should be at least six months.Long-term and low

  11. An intriguing association of Turner syndrome with severe nephrotic syndrome: searching for a diagnosis.

    Science.gov (United States)

    Minzala, G; Ismail, G

    2016-10-01

    Systemic lupus erythematosus (SLE) is a chronic disease caused by an aberrant autoimmune response, with a large spectrum of clinical manifestations. It strikingly affects women. Recent papers reveal that the men with Klinefelter syndrome (47, XXY) have a higher incidence of lupus than the men in the general population, similar with that of genotypic females. On the other hand, there is a great lack of information regarding the association of SLE with Turner syndrome, but it seems to be a lower risk for females with Turner to develop SLE. We present a rare association of a Turner syndrome with SLE, with negative immunology for SLE and with diagnosis made on renal biopsy. These data suggest that the presence of two X chromosomes may predispose to SLE, the ligand (CD40 ligand) for one of the genes that contributes to the pathogenesis of SLE being located on the X chromosome.

  12. Canadian Society of Nephrology Commentary on the 2012 KDIGO clinical practice guideline for glomerulonephritis: management of nephrotic syndrome in children.

    Science.gov (United States)

    Samuel, Susan; Bitzan, Martin; Zappitelli, Michael; Dart, Allison; Mammen, Cherry; Pinsk, Maury; Cybulsky, Andrey V; Walsh, Michael; Knoll, Greg; Hladunewich, Michelle; Bargman, Joanne; Reich, Heather; Humar, Atul; Muirhead, Norman

    2014-03-01

    The KDIGO (Kidney Disease: Improving Global Outcomes) clinical practice guideline for management of glomerulonephritis was recently released. The Canadian Society of Nephrology convened a working group to review the recommendations and comment on their relevancy and applicability to the Canadian context. A subgroup of pediatric nephrologists reviewed the guideline statements for management of childhood nephrotic syndrome and agreed with most of the guideline statements developed by KDIGO. This commentary highlights areas in which there is lack of evidence and areas in need of translation of evidence into clinical practice. Areas of controversy or uncertainty, including the length of corticosteroid therapy for the initial presentation and relapses, definitions of steroid resistance, and choice of second-line agents, are discussed in more detail. Existing practice variation is also addressed.

  13. SOCS3 and SOCS5 mRNA expressions may predict initial steroid response in nephrotic syndrome children

    Directory of Open Access Journals (Sweden)

    Witold Szaflarski

    2012-01-01

    Full Text Available Suppressors of Cytokine Signaling (SOCS inhibit Signal Transducers and Activators of Transcription (STATs phosphorylation by binding and inhibiting Janus Kinases (JaKs. The aim of the present study was to evaluate the influence of glucocorticosteroids on the JaK/STAT signaling pathway in the leukocytes of nephrotic syndrome (NS patients. The study group was composed of 34 steroid sensitive NS (SSNS children and 20 steroid resistant NS (SRNS subjects. Gene expression was assessed by real-time PCR using pre-designed human JaK/STAT PCR array. Protein expression was evaluated using ELISA assay (plasma concentration and immunofluorescence (in situ protein expression. In SSNS children, the initial increased expression of JaK1, JaK2, JaK3, STAT1, STAT2, STAT6, TYK2, SOCS1, SOCS2, SOCS3, SOCS4 and SOCS5 was reduced back to the control limits. Similarly, in SRNS patients the increased levels of almost all mRNA expressions for the abovementioned genes were decreased, with the exceptions of SOCS3 and SOCS5 expressions. These mRNA expressions were still significantly increased and correlated with early unfavorable course of nephrotic syndrome in children. Plasma levels of SOCS3, SOCS5, IL-6 and IL-20 were significantly increased in SRNS subjects after six weeks of steroids medication compared to SSNS and control participants. We conclude that SOCS3 and SOCS5 increased mRNA expressions might predict initial resistance to steroids in NS patients. (Folia Histochemica et Cytobiologica 2011; Vol. 49, No. 4, pp. 719–728

  14. The role of ventilation-perfusion lung scintigraphy in the diagnosis of pulmonary embolism in nephrotic syndrome patients

    International Nuclear Information System (INIS)

    Purpose: Patients with nephrotic syndrome (NS) have a high risk to develop thrombosis and even to progress to pulmonary embolism (PE). This study was performed to investigate the possible role of ventilafion-perfusion (V/Q) lung scans to evaluate PE in NS patients. Methods: 194 patients with NS (8 cases of minimal change NS (MCNS), 33 of mesangial proliferative (MsPGN), 19 of Mesangiocapillary glomerulonephritis (MCGN), 69 of membranous nephropathy, 56 of focal and segmental glomerular sclerosis (FSGS), and 9 of NS induced from purpura, SLE, diabetes mellitus or amyloidosis) were studied. In all patients, the development probability of PE was assessed based on the results of V/Q lung scans (Technegas for ventilation and Tc-99m MAA for perfusion imaging). The findings of V/Q lung scans were interrupted into high, intermediate, low or no probability of PE. The patients' clinical symptoms and signs were observed. Additional examinations included chest radiography, and serum biochemical tests such as albumin, blood urea nitrogen (BUN), creatinine (Cr), plasma fibrinogen (Fg), antithrombin III (AT III), prothrombin time (PT), and activated partial thromboplastin time (APTT). Results: Based on the findings of V/Q lung scans, 39 (20%) of the patients were categorized as having a high probability of PE and 56 (29%) as intermediate or low probability of PE. The occurrence of PE in patients with membranous nephropathy (23 cases, 33%) was significantly higher than that in those other pathological types. In the 86 patients with severe hypoalbuminemia (serum albumin concentration = 20g/L. The Fg and AT III levels were found to be correlated with the occurrence of PE. The clinical symptoms and signs, chest radiograph results and values of BUN, Cr, PT and APTT were not consistent with the occurrence of PE. Conclusion: Though usually clinically silent, PE is not a rare complication in patients with NS, especially in those with membranous nephropathy. In this study, the occurrence

  15. Relapse or Worsening of Nephrotic Syndrome in Idiopathic Membranous Nephropathy Can Occur even though the Glomerular Immune Deposits Have Been Eradicated

    OpenAIRE

    Barnes, Chadwick E.; Wilmer, William A.; Hernandez, Jr., Raul A.; Valentine, Christopher; Hiremath, Leena S.; Nadasdy, Tibor; Satoskar, Anjali A.; Shim, Rose L.; Rovin, Brad H; Hebert, Lee A.

    2011-01-01

    Background: Relapse or worsening of nephrotic syndrome (NS) in idiopathic membranous nephropathy (IMN) is generally assumed to be due to recurrent disease. Here we document that often that may not be the case. Subjects and Methods: This is a prospective study of 7 consecutive IMN patients whose renal status improved, then worsened after completing a course of immunosuppressive therapy. Each underwent detailed testing and repeat kidney biopsy. Results: In 4 patients (group A), the biopsy showe...

  16. 肾病综合征患儿行为问题探讨%An investigation of related behavior problems in children with nephrotic syndrome

    Institute of Scientific and Technical Information of China (English)

    俞杰; 周辉; 赵建美; 徐美玉; 杨丽丽

    2011-01-01

    Objective To study the behavior problems in children with nephrotic syndrome.Methods The state of behavior problems in 52 children with nephrotic syndrome (treatment group) was investigated with Conners children behavior questionnaires (parents questionnaires).Fifty-two healthy children with the same sex and age in treatment group were taken as the control group.Results The behavior problems such as study, heart, and anxiety were significantly higher in the children with nephrotic syndrome than those before tratment and in control group (P<0.05).Conclusion The children with nephrotic syndrome have behavior problems in study, heart, and anxiety.%目的 探讨肾病综合征患儿行为问题.方法 随访52例肾病综合征患儿,应用Conners儿童行为问卷(父母问卷)调查其治疗前后的行为问题状况.按1:1配比性别、年龄相同健康幼儿为对照组.结果 肾病综合征患儿的学习问题、心身问题和焦虑明显高于对照组(P<0.05).治疗后的学习、心身问题和焦虑明显高于治疗前(P<0.05).结论 肾病综合征患儿存在学习问题、心身问题和焦虑等行为问题.

  17. Elevated Urinary Levels of 8-Hydroxy-2'-deoxyguanosine in a Japanese Child of Xeroderma Pigmentosum/Cockayne Syndrome Complex with Infantile Onset of Nephrotic Syndrome.

    Science.gov (United States)

    Kondo, Daiki; Noguchi, Atsuko; Tamura, Hiroaki; Tsuchida, Satoko; Takahashi, Ikuko; Kubota, Hiroki; Yano, Tamami; Oyama, Chikako; Sawaishi, Yukio; Moriwaki, Shinichi; Takahashi, Tsutomu

    2016-01-01

    Nucleotide excision repair (NER) is an essential biological pathway protecting against ultraviolet light-induced DNA damage. Deficient NER causes a group of rare genetic disorders including two autosomal recessive diseases, xeroderma pigmentosum (XP) and Cockayne syndrome (CS). In addition to the cutaneous photosensitivity shared in XP and CS, CS is featured by growth failure, neurological deterioration, microcephaly, and deep sunken eyes. XP/CS complex is an extremely rare type of NER disorder with a distinct phenotype that is characterized by the skin and eye pathology of XP and the somatic and neurological abnormalities of CS. Some of CS cases have been reported to be complicated with renal failure, but the genetic background or the etiology of the renal failure has not been reported. We herein report a 1-year-old Japanese boy with XP/CS complex, complicated by nephrotic syndrome. Diagnosis was confirmed by the presence of compound heterozygous mutations, G47R (c.139G>A) and R616G (c.1846C>G), in the excision repair cross-complementation group 2 (ERCC2) gene. The kidney biopsies, performed at the age of 1 year and 2 months, revealed diffuse expansion of the mesangial matrix and segmental glomerulosclerosis under light microscopy, and diffused thin capillary walls with partially lamellated regions under electron microscopy. Notably, high levels of urinary 8-hydroxy-2'-deoxyguanosin, known as an oxidative stress marker, were observed during the clinical course. The patient died at the age of 1 year and 11 months because of renal failure. We suggest the involvement of oxidative stress in the pathogenesis of nephrotic syndrome in NER disorders. PMID:27396511

  18. Nephrotic syndrome associated with hepatointestinal schistosomiasis Síndrome nefrótica associada à esquistossomose hepatointestinal

    Directory of Open Access Journals (Sweden)

    H. Abensur

    1992-08-01

    Full Text Available Schistosomal nephropathy has long been related to the hepatosplenic form of schistosomiasis. In the last few years, 24 patients with hepatointestinal schistosomiasis and the nephrotic syndrome were studied. Aiming at evaluating a possible etiologic participation of schistosomiasis in the development of the nephropathy, this group was comparatively studied with a group of 37 patients with idiopathic nephrotic syndrome. Both groups had a different distribution of the histologic lesions. In the group with schistosomiasis there was a statistically significant prevalence of proliferative mesangial glomerulonephritis (33.3%, whereas in the control group there was prevalence of membranous glomerulonephritis (32.4%. On immunofluorescence, IgM was positive in 94.4% of the patients with schistosomiasis versus 55.0% in the control group (pA nefropatia esquistossomótica está classicamente vinculada à fornia hepatoesplênica da esquistossomose. Ao longo dos últimos anos 24 casos de pacientes esquistossomóticos hepato-intestinais e portadores de síndrome nefrótica foram estudados. Com o objetivo de verificar a possível participação etiológica da esquistossomose na gênese da nefropatia, analisamos este grupo comparativamente ao grupo de 37 doentes portadores de síndrome nefrótica idiopática. Ambos os grupos apresentaram distribuição distinta dos tipos histológicos de glomerulopatia. No grupo de esquistossomóticos houve predomínio estatisticamente significante de glomerulonefrite proliferativa mesangial (33.3%, enquanto no grupo controle houve predomínio da glomerulonefrite membranosa (32.4%. A positividade para IgM à imunofluorescência foi de 94.4% nos doentes esquistossomóticos versus 55.0% no grupo controle (p<0.01. No grupo de esquistossomóticos 8 pacientes evidenciaram glomerulonefrite proliferativa mesangial e 5, glomerulonefrite membranoproliferativa. Em ambos os tipos histológicos a imunofluorescência mostrou dep

  19. Vitamin D receptor gene TaqI and Apal polymorphisms and steroid responsiveness in childhood idiopathic nephrotic syndrome

    Directory of Open Access Journals (Sweden)

    Al-Eisa AA

    2016-08-01

    Full Text Available Amal A Al-Eisa, Mohammad Z Haider Department of Pediatrics, Faculty of Medicine, Kuwait University, Safat, Kuwait Background: Vitamin D activity is controlled by vitamin D receptors (VDRs, which are affected by different genetic polymorphisms, including TaqI and Apal restriction fragment length polymorphisms (RFLPs, which have been reported to be associated with several diseases. The aim of this study was to determine the frequency and the association of VDR gene polymorphisms with idiopathic nephrotic syndrome (INS and steroid responsiveness in Kuwaiti children. Subjects and methods: Genotypes of the VDR TaqI gene polymorphism and the Apal gene polymorphism were analyzed using polymerase chain reaction-RFLP in 78 INS patients and 56 matched controls. Results: A total of 78 INS (62 steroid sensitive [SS] and 16 steroid resistant [SR] patients with a mean age of 6.5±3.1 years were studied. Male:female ratio was 2:1. The TT genotype of VDR–TaqI polymorphism was detected in 41% of the INS patients compared to 42% of the controls (P=0.816. The heterozygous TC genotype was detected in 33% of INS patients compared to 46% of the controls (P=0.462. The CC genotype was detected in 25.6% of INS patients and 21% of the controls (P=0.719. The C-allele frequency, in its homozygous and heterozygous forms, was 71% in INS patients compared to 63% in the controls (P=0.342. Similarly, no significant difference was detected in terms of VDR–Apal polymorphism in INS patients compared to the controls for all the three genotypes (P=0.76, P=0.207, and P=0.364, respectively, for GG, GT, and TT genotypes. The T-allele frequency, in its homozygous and heterozygous forms, was 89% in INS patients compared to 93% in the controls (P=0.076. No significant difference was found in any of the allele frequencies between SS and SR subgroups when compared with each other or when compared to the controls. Conclusion: Our data do not support the use of VDR–TaqI or

  20. Relationship of Tumor Necrosis Factor-α and Nitrogen Oxide with Treatment of Frequent Relapse Nephrotic Syndrome by Shenkangling(肾康灵)Granule in Children

    Institute of Scientific and Technical Information of China (English)

    王莉玲; 郑健; 曾章超; 董飞侠; 吴群励

    2003-01-01

    Objective: To observe the relationship of tumor necrosis factor-α (TNF-α) and nitrogen oxide (NO) with the treatment of frequent relapse nephrotic syndrome (FRNS) and to explore the pathogenesis of FRNS and the therapeutic mechanism of Shenkangling(肾康灵,SKL) Granule in children.Methods: Sixty children suffering from FRNS were randomly divided into the treated group and control group, 30 in each, and the other 30 healthy children were taken as healthy group. The patients were treated with prednisone for a long-term course, and those with no effect or partial effect shown were treated with additional Tripterygium or Cytoxan in the control group, while in the treated group patients were treated with prednisone and additional SKL. The two groups were compared as to their changes of TNF-α,NO before and after treatment, and the relapses after treatment. Results: The levels of TNF-α and NO in the sick children before treatment were markedly higher than those after treatment and normal group (P<0. 01). The positive correlation between TNF-α of FRNS cases and relapse risk displayed more significance than that between the relapse of FRNS and NO. The difference between treated group and control group was significant (P<0. 01). Conclusion: TNF-α can be regarded as the monitoring parameter of the active phase in FRNS, and the higher the level, the more possible the relapse would occur. SKL could markedly reduce the relapse rate of FRNS in children.

  1. The Change-Over of Yin-yang and Gene Regulation in Kidney Deficiency Syndromes

    Institute of Scientific and Technical Information of China (English)

    DONG Fei-xia; HE Li-qun

    2009-01-01

    The present paper studies gene regulation in kidney deficiency syndromes from the simple Nephrotic Syndrome and with the principle of positive-negative regulation to control the change-over ofyin-yang, the modern molecular biological techniques can be used, such as gene chip, representational difference analysis (RDA) and gene sequence analysis, so as to investigate the inner relationship between the genes and kidney deficiency syndromes and prove the effect given by these genes on the pathophysiological status of change-over ofyin-yang in kidney deficiency syndromes.This philosophical approach and method can also be adopted for studies of the related genes in other TCM syndromes.

  2. Changes of Serum and Urinary IFN-γ, IL-13 and TGF-β1 in Children with Primary Nephrotic Syndrome and Effect of Astragalus%原发性肾病综合征患儿血尿IFN-γ、IL-13、TGF-β1的变化及黄芪的作用

    Institute of Scientific and Technical Information of China (English)

    康国贵; 周江瑾; 张琦; 陈惠琴; 康友群

    2011-01-01

    目的 探讨原性肾病综合征(PNS)患儿血、尿IFN-γ、IL-13、TGF-β1水平的变化及黄芪的干预作用.方法 46例PNS患儿随机分为黄芪治疗组(Ⅰ组,n=24),PNS对照组(Ⅱ组,n=22),观察用药前后血清及尿液IFN-γ、IL-13、 TGF-β1水平及临床转归.健康儿童20例为正常对照组(Ⅲ组).结果 血清 IFN-γ、IL-13水平,入院时Ⅰ、Ⅱ组均显著高于Ⅲ组(P均<0.01);缓解后其水平均虽显著下降(P均<0.01),但I组的下降明显高于Ⅱ组(P均<0.01),与Ⅲ组比较已无显著差异(P均>0.05),而Ⅱ组仍显著高于Ⅲ组(P均>0.05).血清TGF-β1水平入院时Ⅰ、Ⅱ组均显著高于Ⅲ组(P均<0.01);缓解后均显著下降,与Ⅲ组比均已无显著差别(P均>0.05),Ⅰ组与Ⅱ组比较亦无显著差别(P>0.05).尿液IFN-γ、IL-13、TGF-β1水平与血清 IFN-γ、IL-13、TGF-β1水平一样用药前后也发生相同的变化.继发感染率和反复或复发率Ⅰ组均显著低于Ⅱ组(分别为50%比81.8%和33.3%比63.6%,分别χ2=5.123,4.224,P均<0.05).感染后治愈天数Ⅰ组显著少于Ⅱ组(5.0±1 6天比8.0±2天,t=3.066,P<0.005).结论 IFN-γ、IL-13、TGF-β1各细胞因子可能均参与PNS的发病;黄芪对血、尿IFN-γ、IL-13有一定的调节作用.黄芪在佐治SNS中具有预防和降低感染、减少和预防反复或复发、避免反复大量应用激素的作用.%Objective To investigate the changes of interferon - gamma (IFN - γ) , interleukin -13 (IL - 13) and transforming growth factor beta 1 ( TGF - β1 ) in serum and urinary in children with primary nephrotic syndrome ( PNS) and study the effect of astragalus. Methods Forty-six children with PNS were randomly divided into two groups : astragalus granula treatment group( Ⅰ group,n = 24) and prednison control group ( Ⅱ group,n =22) . In addition, 20 healthy children were normal control group( Ⅲ group). The treatment protocol of prednison used for 6 months was administered in two groups, while 15g

  3. Activation of the IL-2 Receptor in Podocytes: A Potential Mechanism for Podocyte Injury in Idiopathic Nephrotic Syndrome?

    Science.gov (United States)

    Zea, Arnold H.; Stewart, Tyrus; Ascani, Jeannine; Tate, David J.; Finkel-Jimenez, Beatriz; Wilk, Anna; Reiss, Krzysztof; Smoyer, William E.; Aviles, Diego H.

    2016-01-01

    The renal podocyte plays an important role in maintaining the structural integrity of the glomerular basement membrane. We have previously reported that patients with idiopathic nephrotic syndrome (INS) have increased IL-2 production. We hypothesized that podocytes express an IL-2 receptor (IL-2R) and signaling through this receptor can result in podocyte injury. To confirm the presence of the IL-2R, we tested a conditionally immortalized murine podocyte cell line by flow cytometry, qPCR, and Western blot. To test for the presence of the IL-2R in vivo, immunohistochemical staining was performed on human renal biopsies in children with FSGS and control. Podocytes were stimulated with IL-2 in vitro, to study signaling events via the JAK/STAT pathway. The results showed that stimulation with IL-2 resulted in increased mRNA and protein expression of STAT 5a, phosphorylated STAT 5, JAK 3, and phosphorylated JAK 3. We then investigated for signs of cellular injury and the data showed that pro-apoptotic markers Bax and cFLIP were significantly increased following IL-2 exposure, whereas LC3 II was decreased. Furthermore, mitochondrial depolarization and apoptosis were both significantly increased following activation of the IL-2R. We used a paracellular permeability assay to monitor the structural integrity of a podocyte monolayer following IL-2 exposure. The results showed that podocytes exposed to IL-2 have increased albumin leakage across the monolayer. We conclude that murine podocytes express the IL-2R, and that activation through the IL-2R results in podocyte injury. PMID:27389192

  4. Activation of the IL-2 Receptor in Podocytes: A Potential Mechanism for Podocyte Injury in Idiopathic Nephrotic Syndrome?

    Directory of Open Access Journals (Sweden)

    Arnold H Zea

    Full Text Available The renal podocyte plays an important role in maintaining the structural integrity of the glomerular basement membrane. We have previously reported that patients with idiopathic nephrotic syndrome (INS have increased IL-2 production. We hypothesized that podocytes express an IL-2 receptor (IL-2R and signaling through this receptor can result in podocyte injury. To confirm the presence of the IL-2R, we tested a conditionally immortalized murine podocyte cell line by flow cytometry, qPCR, and Western blot. To test for the presence of the IL-2R in vivo, immunohistochemical staining was performed on human renal biopsies in children with FSGS and control. Podocytes were stimulated with IL-2 in vitro, to study signaling events via the JAK/STAT pathway. The results showed that stimulation with IL-2 resulted in increased mRNA and protein expression of STAT 5a, phosphorylated STAT 5, JAK 3, and phosphorylated JAK 3. We then investigated for signs of cellular injury and the data showed that pro-apoptotic markers Bax and cFLIP were significantly increased following IL-2 exposure, whereas LC3 II was decreased. Furthermore, mitochondrial depolarization and apoptosis were both significantly increased following activation of the IL-2R. We used a paracellular permeability assay to monitor the structural integrity of a podocyte monolayer following IL-2 exposure. The results showed that podocytes exposed to IL-2 have increased albumin leakage across the monolayer. We conclude that murine podocytes express the IL-2R, and that activation through the IL-2R results in podocyte injury.

  5. Effect of calcium and vitamin D supplementation on serum calcium level in children with idiopathic nephrotic syndrome

    Directory of Open Access Journals (Sweden)

    Vaya Dasitania

    2014-05-01

    Full Text Available Background Patients with idiopathic nephrotic syndrome (NS may develop hypocalcemia caused by low levels of albumin and vitamin D-binding protein, which subsequently decreases calcium absorption in the intestine. Hypocalcemia may result in neuromuscular manifestations, such as Chvostek’s and Trosseau’s signs. Objectives To evaluate the effect of calcium and vitamin D supplementation on hypocalcemia and its clinical manifestations in idiopathic NS children. Methods A randomized, single-blind, controlled trial was performed in idiopathic NS patients aged 1−14 years. Subjects were divided into treatment and placebo groups. Subjects in the treatment group received 800 mg elemental calcium and 400 IU vitamin D supplementation, while they in control group received placebo syrup, both for 8 weeks. Serum calcium and manifestations of hypocalcemia were examined before and after supplementation. Results Thirty subjects completed the study (15 in each group. Seventeen subjects experienced hypocalcemia. Chvostek’s and Trosseau’s signs were observed in 6 subjects in the treatment group and 2 subjects in the placebo group (P= 0.427. After 8 weeks of intervention, Chvostek’s and Trosseau’s signs disappeared in both groups, and calcium levels were significantly increased in both groups compared to the levels before intervention. However, there was no significant difference in serum calcium levels after 8 weeks between the treatment and placebo groups (P =0.707. Conclusion Normalization of serum calcium levels and improved clinical manifestations of hypocalcemia occur both in NS patients who receive calcium and vitamin D supplementation and those who do not.

  6. 单纯超滤治疗肾病综合征高度水肿40例疗效分析%Curative Effect of Pure Ultrafiltration on Severe Edema in 40 Cases with Nephrotic Syndrome

    Institute of Scientific and Technical Information of China (English)

    刘红平; 雷方; 姜苗苗

    2015-01-01

    目的:观察单纯超滤脱水治疗肾病综合征高度水肿的临床效果。方法选取40例肾病综合征伴重度水肿或严重浮肿的病例,所有病例均行单纯超滤脱水,平均透析3~5次,时间2.0~2.5h,每周透析2~3次,脱水量2.0~3.5kg/次。观察水肿和肌酐、白蛋白、尿蛋白等指标的变化。结果所有病例胸腹水减少明显,下肢水肿明显消退,尿蛋白、血白蛋白等指标均有改善,睡眠、食欲、气促、腹胀均有好转,未发现明显副作用。结论单纯超滤治疗肾病综合征合并重度水肿或严重浮肿是可行、有效和安全的。%Objective To observe the clinical effect of pure ultrafiltration dehydration on severe edema in cases with nephrotic syn-drome. Methods 40 cases with severe edema or serious dropsy of nephrotic syndrome were selected and received pure ultrafiltration de-hydration for an average of 3 to 5 times, 2 to 2.5 hours each time, 2 to 3 times per week and dehydration amount of 2.0kg to 3.5kg each time;an observation was made to the changes of edema and the changes in the index of creatinine, albumin, urine protein and blood al-bumin. Results Hydrothorax and ascites in all the cases decreased greatly and the leg edema extinction was obvious;the index of creati-nine, albumin, urine protein and blood albumin were significantly improved as well as sleep, appetite and the symptoms of shortness of breath and abdominal distention;no obvious side effects were found. Conclusions Pure ultrafiltration is feasible, effective and safe for severe edema or serious dropsy in patients with nephrotic syndrome.

  7. Correlation between Resilience and Depression in Children with Nephrotic Syndrome%肾病综合征患儿的韧性与抑郁的相关性

    Institute of Scientific and Technical Information of China (English)

    杨红霞; 刘化侠; 李蕾; 万学英; 崔文香

    2012-01-01

    Objective To investigate the resilience and depression in children with nephrotic syndrome and the correlation between them. Methods From April 2009 to February 2010,104 children with nephrotic syndrome from 5 Grade-HI Class-A hospitals were surveyed with personal information questionnaire, chronic illness children's resilience scale(CICRS) ,children's depression inventory (CDI). Results The average score of resilience in children with nephrotic syndrome was 99. 53±14. 33. There were 20 cases with poor state of mental resilience, accounting for 19. 23%; 60 cases were observed with good mental resilience, accounting for 57. 69% s 24 cases with excellent mental resilience, accounting for 23. 08%. The average depression score in children with nephrotic syndrome as 11. 83 ±7. 83. There were 18 cases with symptoms of depression,accounting for 17. 31%; 86 cases showed no' symptoms of depression at all, accounting for 82. 69%. As for children suffering from nephrotic syndrome, there was a moderately negative correlation between the scores of resilience and depression(r= -0. 582,P<0. 01). The correlation coefficients of all the dimensions and the depression score were negatively correlated(P<0. 01). Conclusion The medical staff should carry out the resilience intervention to enhance disease management behavior of children and promote their mental health.%目的 调查肾病综合征患儿的韧性和抑郁状况,并探讨两者之间的相关性.方法 便利抽样法选取2009年4月至2010年2月在山东省5所三级甲等医院进行治疗的104名肾病综合征患儿为研究对象,采用自制的一般情况调查表、慢性病儿童韧性量表(chronic illness children's resilience scale,CICRS)、儿童抑郁量表(children's depression inventory,CDI)对其进行调查.结果 肾病综合征患儿韧性水平均分为(99.53±14.33)分,韧性水平差者20例(19.23%);韧性水平较好者60例(57.69%);韧性水平很好者24例(23.08%).肾病综

  8. Vitamin D receptor gene TaqI and Apal polymorphisms and steroid responsiveness in childhood idiopathic nephrotic syndrome

    Science.gov (United States)

    Al-Eisa, Amal A; Haider, Mohammad Z

    2016-01-01

    Background Vitamin D activity is controlled by vitamin D receptors (VDRs), which are affected by different genetic polymorphisms, including TaqI and Apal restriction fragment length polymorphisms (RFLPs), which have been reported to be associated with several diseases. The aim of this study was to determine the frequency and the association of VDR gene polymorphisms with idiopathic nephrotic syndrome (INS) and steroid responsiveness in Kuwaiti children. Subjects and methods Genotypes of the VDR TaqI gene polymorphism and the Apal gene polymorphism were analyzed using polymerase chain reaction-RFLP in 78 INS patients and 56 matched controls. Results A total of 78 INS (62 steroid sensitive [SS] and 16 steroid resistant [SR]) patients with a mean age of 6.5±3.1 years were studied. Male:female ratio was 2:1. The TT genotype of VDR–TaqI polymorphism was detected in 41% of the INS patients compared to 42% of the controls (P=0.816). The heterozygous TC genotype was detected in 33% of INS patients compared to 46% of the controls (P=0.462). The CC genotype was detected in 25.6% of INS patients and 21% of the controls (P=0.719). The C-allele frequency, in its homozygous and heterozygous forms, was 71% in INS patients compared to 63% in the controls (P=0.342). Similarly, no significant difference was detected in terms of VDR–Apal polymorphism in INS patients compared to the controls for all the three genotypes (P=0.76, P=0.207, and P=0.364, respectively, for GG, GT, and TT genotypes). The T-allele frequency, in its homozygous and heterozygous forms, was 89% in INS patients compared to 93% in the controls (P=0.076). No significant difference was found in any of the allele frequencies between SS and SR subgroups when compared with each other or when compared to the controls. Conclusion Our data do not support the use of VDR–TaqI or –Apal gene polymorphisms as genetic markers of INS nor do they predict steroid responsiveness in children with the disease.

  9. Is biopsy required prior to cyclophosphamide in steroid-sensitive nephrotic syndrome?

    NARCIS (Netherlands)

    Stadermann, M.B.; Lilien, M.R.; Kar, N.C.A.J. van de; Monnens, L.A.H.; Schröder, C.H.

    2003-01-01

    AIM: The present studywas designed to retrospectively evaluate the use of renal biopsies prior to cyclophosphamide therapy. The aim of the study was to determine in how many cases histological outcome of the biopsies had subsequently changed the decision to treat or refrain from treatment. PATIENTS

  10. Progression of glomerulonephritis to end-stage kidney disease in a cat with nephrotic syndrome.

    Science.gov (United States)

    Kamiie, Junichi; Haishima, Atsuko; Inoue, Kaoru; Ogihara, Kikumi; Ono, Mihoko; Yasuno, Kyohei; Kobayashi, Ryosuke; Aihara, Naoyuki; Ohmuro, Tamio; Shirota, Kinji

    2011-01-01

    A percutaneous renal biopsy was performed on a 3-year-old female Japanese domestic cat with pleural effusion, mild azotemia, hypoalbuminemia, hypercholesterolemia, and proteinuria. Glomerular lesions included mild diffuse hypercellularity and numerous capsular adhesions with segmental sclerosis/hyalinosis of glomerular tufts. Electron microscopy revealed many subendothelial dense deposits with characteristic outer protrusion of glomerular basement membrane. Diffuse and global granular deposits of IgG and C3 were detected along the capillary walls. Tubulo-interstitial changes were mild at the time of biopsy, but progression of the disease was predicted because of the many capsular adhesions of the glomerular tufts. The cat was fed a prescription diet without any other specific or symptomatic therapy after renal biopsy, and died 43 weeks after the biopsy. At necropsy, extensive tubulo-interstitial fibrosis and mononuclear cell infiltration had developed throughout the cortex and outer medulla, and most glomeruli had extensive global sclerosis or obsolescence with less prominent depositions of IgG and C3. PMID:20823662

  11. A 17-Year-Old With Steroid-Resistant Nephrotic Syndrome.

    Science.gov (United States)

    Band, Molly E; Sheldon, Candice; Brancato, John; Parikh, Nehal S; D'Alessandri-Silva, Cynthia

    2016-05-01

    A 17-year-old girl presented with facial swelling and shortness of breath to an outside emergency department. She was treated for an allergic reaction with steroids and antihistamines, and discharged from the hospital. Subsequently, she was referred as an outpatient to pediatric nephrology for recurrent edema and proteinuria. Initial laboratory workup by nephrology was significant for a normal complete blood count and reassuring electrolyte panel. Pertinent laboratories were a creatinine of 0.5 mg/dL (0.4-1.1 mg/dL) and an albumin 2.3 g/dL (3.5-5.0 g/dL). The urine protein-to-creatinine ratio was >7 (<0.2). A renal ultrasound showed symmetrically sized kidneys with normal echotexture. The patient's renal biopsy results were consistent with minimal change disease. Based on the biopsy results, prednisone was started. Due to a poor response to prednisone, an alternate immunomodulator therapy was selected. Her subsequent complete blood counts showed a downward trend of all cell lines and an elevated serum uric acid. Concurrently, she reported worsening fatigue, low back pain, nausea, vomiting, night sweats, and pruritus. More details of her case and the outcome are presented. PMID:27244796

  12. A case of rheumatic fever with acute post-streptococcal glomerulonephritis and nephrotic syndrome caused by a cutaneous infection with beta-hemolytic streptococci

    Directory of Open Access Journals (Sweden)

    Carsten Sauer Mikkelsen

    2010-01-01

    Full Text Available A middle-aged patient of Greenlandic origin was referred for skin infection of the leg. An initial minor trauma of the skin of the distal right lower extremity was complicated by bullous erysipelas which cultured positive for group A β-hemolytic streptococci (GABHS. The clinical condition deteriorated and necrotizing fasciitis developed despite relevant surgical and antibiotic treatment. Approximately 3 weeks later, the patient developed arthralgia, impaired renal function with azotemia, hypertension and severe nephrotic syndrome with periorbital and peripheral edema. A kidney biopsy demonstrated endocapillary glomerulonephritis. Concomitantly, carditis with chest pain, moderately reduced left ventricular ejection fraction and mitral regurgitation were noted. The patient had no signs of pharyngitis in the whole period. The patient thus contracted poststreptococ glomerulonephritis and furthermore she fulfilled the criteria of acute rheumatic fever following a GABHS skin infection. We suggest a possible relation between a virulent GABHS clone causing NF and ARF.

  13. Childhood Nephrotic Syndrome

    Science.gov (United States)

    ... Research Training & Career Development Grant programs for students, postdocs, and faculty Research at NIDDK Labs, faculty, and ... diabetes, digestive and liver diseases, kidney diseases, weight control and nutrition, urologic diseases, endocrine and metabolic diseases, ...

  14. Childhood Nephrotic Syndrome

    Science.gov (United States)

    ... specially trained technician performs the procedure in a health care provider’s office, an outpatient center, or a hospital. A radiologist—a doctor who specializes in medical imaging—interprets ...

  15. Plasmin in nephrotic urine activates the epithelial sodium channel

    DEFF Research Database (Denmark)

    Svenningsen, Per; Bistrup, Claus; Friis, Ulla G;

    2008-01-01

    Proteinuria and increased renal reabsorption of NaCl characterize the nephrotic syndrome. Here, we show that protein-rich urine from nephrotic rats and from patients with nephrotic syndrome activate the epithelial sodium channel (ENaC) in cultured M-1 mouse collecting duct cells and in Xenopus...... laevis oocytes heterologously expressing ENaC. The activation depended on urinary serine protease activity. We identified plasmin as a urinary serine protease by matrix-assisted laser desorption/ionization time of-flight mass spectrometry. Purified plasmin activated ENaC currents, and inhibitors...... of plasmin abolished urinary protease activity and the ability to activate ENaC. In nephrotic syndrome, tubular urokinase-type plasminogen activator likely converts filtered plasminogen to plasmin. Consistent with this, the combined application of urokinase-type plasminogen activator and plasminogen...

  16. Eficacia del tratamiento dietético en el síndrome nefrótico Effectiveness of dietetic treatment in nephrotic syndrome

    Directory of Open Access Journals (Sweden)

    A. Calleja Fernández

    2009-12-01

    Full Text Available Presentamos el caso de un paciente diagnosticado de amiloidosis primaria y síndrome nefrótico que acude a la consulta de dietoterapia. En la consulta inicial se realizó una historia nutricional que incluyó una valoración antropométrica completa, composición corporal, bioquímica completa y análisis de la ingesta. El paciente presentó un exceso de agua corporal, proteinuria, niveles disminuidos de proteínas totales, albúmina, prealbúmina y colesterol HDL y concentraciones elevadas de colesterol total, de LDL y de triglicéridos. El consumo de proteínas y sodio era superior a la recomendación. Se elaboró una dieta personalizada. Tras seis meses de dieta el paciente presentó una pérdida de peso a partir de agua, manteniendo el estado nutricional, una disminución de la proteinuria, manteniéndose la función renal y una mejora del perfil lipídico. El tratamiento dietético del síndrome nefrótico es eficaz para disminuir la proteinuria recuperar y mejorar el perfil lipídico y prevenir la desnutrición del paciente.We present the case of a male patient suffering from a primary amyloidosis and a nephrotic syndrome who came to dietotherapy clinic. In the first visit we made a nutritional record including an anthropometric evaluation, body composition, biochemistry, and food intake. The patient had an excess of body water, proteinuria, low plasma protein, albumin, prealbumin and HDL cholesterol levels, and high concentrations of total cholesterol, LDL and triglycerides. The consumption of protein and sodium was higher than the recommendation. An individualized diet was made. Six months later, his weight and the excess of body water decreased, but the fat free mass remained unchanged. The levels of albumin and prealbumin increased, the proteinuria decreased. Total cholesterol, LDL and triglycerides decreased until normal levels. Dietetary treatment in nephrotic syndrome is effective to decrease proteinuria, improve cholesterol and

  17. Identification of the lymphokine soluble immune response suppressor in urine of nephrotic children.

    OpenAIRE

    Schnaper, H W; Aune, T M

    1985-01-01

    Patients with minimal change nephrotic syndrome (MCNS) frequently have suppressed in vivo and in vitro immune responsiveness of uncertain etiology. Because increased suppressor cell activity has been associated with this disease, urines from MCNS patients were screened for activity of the lymphokine soluble immune response suppressor (SIRS), a product of concanavalin A- or interferon-activated suppressor T cells. Urines from untreated MCNS patients suppressed polyclonal plaque-forming cell re...

  18. 原发性肾病综合征与血小板活化因子水解酶的相关性分析%The Correlation Analysis of Primary Nephrotic Syndrome with Platelet Activating Factor Acetylhydrolase

    Institute of Scientific and Technical Information of China (English)

    田鲁; 胡亚琳; 操轩

    2015-01-01

    Objective:To observe the correlation of primary nephrotic syndrome ( primary nephritic syn-drome,PNS) with platelet activating factor acetylhydrolase (platelet activating factor cetylhydrolase ,PAF-AH).Method:48 patients with primary nephrotic syndrome treated in the hospital from Jan .2013 to Jun. 2013 were selected as the research object , all were initial treatment patients .They were divided into simple nephrotic syndrome group (STNS group with 31 cases) and nephritic type nephrotic syndrome group (NTNS group with 17 cases);and according to the difference in effects of hormone treatment all patients were divided sensitive nephrotic syndrome ( steroid-sensitive nephritic syndrome , SSNS ) group ( 19 cases ) , steroid re-sistant nephrotic syndrome (steroid-resistent nephritic syndrome, SRNS) group (15 cases), steroid depend-ent nephrotic syndrome group ( steroid-dependent nephritic syndrome , SDNS ) group ( 14 cases ) , then se-lected healthy persons with 30 cases as control group , 78 cases were determined the activity of PAF-AH, then compared differences between two groups .Result:PAF-AH activity in STNS group was(51.9 ±8.3)μmoL· min-1 · L-1 and was higher than those in NTNS group and healthy control group , all P<0.05; and NTNS activity in PAF-AH group were higher than that in healthy control group , all P<0.05.The activity of PAF-AH in SSNS group were higher than those in SRNS group , SDNS group and the control group , P<0.05 group, SDNS group, SRNS and PAF-AH activity were higher than the control group , all P<0.05.By the a-nalysis of perason , PAF-AH activity and primary nephrotic syndrome had obvious correlation analysis ,rSTNS=0.618;rNTNS=0.524; rSSNS=0.717; rSRNS=0.567.; rSDNS=0.327, P<0.05.Conclusion: Platelet activating factor acetylhydrolase activity of patients with nephrotic syndrome is significantly higher than the normal population, and the different types of PNS patients ’ PAF-AH activity are different.%目的:观察

  19. The Correlation Analysis of Primary Nephrotic Syndrome with Platelet Activating Factor Acetylhydrolase%原发性肾病综合征与血小板活化因子水解酶的相关性分析

    Institute of Scientific and Technical Information of China (English)

    田鲁; 胡亚琳; 操轩

    2015-01-01

    Objective:To observe the correlation of primary nephrotic syndrome ( primary nephritic syn-drome,PNS) with platelet activating factor acetylhydrolase (platelet activating factor cetylhydrolase ,PAF-AH).Method:48 patients with primary nephrotic syndrome treated in the hospital from Jan .2013 to Jun. 2013 were selected as the research object , all were initial treatment patients .They were divided into simple nephrotic syndrome group (STNS group with 31 cases) and nephritic type nephrotic syndrome group (NTNS group with 17 cases);and according to the difference in effects of hormone treatment all patients were divided sensitive nephrotic syndrome ( steroid-sensitive nephritic syndrome , SSNS ) group ( 19 cases ) , steroid re-sistant nephrotic syndrome (steroid-resistent nephritic syndrome, SRNS) group (15 cases), steroid depend-ent nephrotic syndrome group ( steroid-dependent nephritic syndrome , SDNS ) group ( 14 cases ) , then se-lected healthy persons with 30 cases as control group , 78 cases were determined the activity of PAF-AH, then compared differences between two groups .Result:PAF-AH activity in STNS group was(51.9 ±8.3)μmoL· min-1 · L-1 and was higher than those in NTNS group and healthy control group , all P<0.05; and NTNS activity in PAF-AH group were higher than that in healthy control group , all P<0.05.The activity of PAF-AH in SSNS group were higher than those in SRNS group , SDNS group and the control group , P<0.05 group, SDNS group, SRNS and PAF-AH activity were higher than the control group , all P<0.05.By the a-nalysis of perason , PAF-AH activity and primary nephrotic syndrome had obvious correlation analysis ,rSTNS=0.618;rNTNS=0.524; rSSNS=0.717; rSRNS=0.567.; rSDNS=0.327, P<0.05.Conclusion: Platelet activating factor acetylhydrolase activity of patients with nephrotic syndrome is significantly higher than the normal population, and the different types of PNS patients ’ PAF-AH activity are different.%目的:观察

  20. Long-term follow-up of pediatric patients with nephrotic syndrome treated at Hospital Universitario San Vicente de Paúl (Medellín (HUSVP, Colombia, between January 1960 and December 2009 = Evolución a largo plazo de los niños con diagnóstico de síndrome nefrótico atendidos en el Hospital Universitario San Vicente de Paúl, Medellín, entre enero de 1960 y diciembre de 2009

    Directory of Open Access Journals (Sweden)

    Atehortúa Baena, Paola Shyrley

    2013-04-01

    Full Text Available Introduction: Nephrotic syndrome (NS is a glomerular disease that frequently affects children. There have been few studies on it in Colombia.Objective: To describe the clinical and epidemiological features of children with SN treated at HUSVP between 1960 and 2009.Methodology: Retrospective and descriptive study.Results: Steroid-sensitive nephrotic syndrome was diagnosed in 87.9% of the patients, and between 1.7%-5.4% turned steroid-resistant. Biopsies revealed disease with minimal changes in 43.6% and focal segmental glomerulosclerosis in 37.3%. Additional immunosuppressive therapy was required by 40% of the patients; in 88.8% of these, cyclophosphamide was used, and remission was achieved in 85.7%. In 56% of the cases there were complications that were infectious in 52%. Nine percent of the patients progressed to end-stage renal disease. Mortality rate was 5.7%.Discussion: The large number of patients with nephrotic syndrome studied in this series and the long period of follow-up (up to 35 years provide valuable information about the clinical behavior of this syndrome in Colombia and on its response to immunosuppressive therapy.

  1. Effect of extended care on self-care abilities in patients with nephrotic syndrome%延续护理对肾病综合征自我护理能力的影响

    Institute of Scientific and Technical Information of China (English)

    阮柳红; 黎华; 伍慧贤

    2012-01-01

    Objective To observe the effect of extended care on self-care ability in patients with nephrotic syndrome.Methods 82 discharged patients with nephrotic syndrome were randomly divided into study group and control group.The study group received extended care.Rate of awareness of recurrence prevention,self-care abilities,and recurrence rate were compared between the two groups.All the patients were followed up for one year.Results The rate of awareness of recurrence prevention and self-care abilities were significantly greater and the recurrence rate was markedly lower in the study group than in the control group.Conclusions Extended care has a marked effect on self-care abilities in patients with nephrotic syndrome,resulting in a decrease in the recurrence rate.%目的 观察延续护理对肾病综合征自我护理能力的影响.方法 选择出院的肾病综合征患者82例并随机分为实验组和对照组,实验组增加出院后延续护理,比较两组的预防复发知识知晓率、自我护理能力及复发率,调查时间为1年.结果 实验组的预防复发知识知晓率及自我护理能力显著高于对照组(P< 0.01),复发率明显低于对照组(P<0.01),两组比较差异有显著性.结论 延续护理对出院肾病综合征患者的自我护理能力有显著影响,从而减低复发率.

  2. The influence of health education on anxiety emotion of parents of primary nephrotic syndrome children%健康教育对肾病综合征患儿家长焦虑情绪的影响

    Institute of Scientific and Technical Information of China (English)

    姚霞; 李小洁; 姚志红

    2009-01-01

    目的 探讨健康教育对肾病综合征患儿家长焦虑情绪的影响.方法 98例确诊为肾病综合征患儿的家长随机分为实验组48例及对照组50例,对照组患儿给予常规治疗及一般护理.实验组在对照组基础上给予健康教育.观察两组患儿家长焦虑情绪改变.结果 实验组肾病综合征息儿家长经健康教育后其焦虑情绪较对照组明显减轻P<0.01.结论 健康教育可有效减轻肾病综合征患儿家长的焦虑情绪,有利于肾病综合征患儿病情康复.%Objective To investigate the influence of health education on anxiety emotion of parents of primary nephrotic syndrome children.Methods 98 cases were randomly divided into two groups.The control group was given routine treatment and nursing,and the experimental group was additional given health education.The anxiety emtion of the two groups were observed.Results The anxiety emtion in the experimental group was distinctly decreased than that of control group(P <0.01).Conclusions Health education could efficiently decrease the anxiety emotion of parents of primary nephrotic syndrome children and is beneficial for the rehabilitation of the children with primary nephrotic syndrome.

  3. 环磷酰胺冲击疗法联合激素在肾病综合征治疗中的价值分析%Value Analysis of Cyclophosphamide Pulse Therapy Combined With Hormone Treatment of Nephrotic Syndrome

    Institute of Scientific and Technical Information of China (English)

    龙京花

    2015-01-01

    目的:分析环磷酰胺冲击疗法联合激素在肾病综合征治疗中的价值。方法将肾病综合征患者68例随机分为观察组与对照组,观察组给予环磷酰胺冲击疗法联合激素治疗,对照组给予常规激素治疗。结果观察组总缓解率高于对照组,有统计学意义(P <0.05);不良反应发生率低于对照组,有统计学意义(P <0.01)。结论环磷酰胺冲击疗法联合激素在肾病综合征治疗中的价值较高。%Objective To analyze cyclophosphamide pulse therapy combined with hormone therapy in nephrotic syndrome value. Methods 68 cases of nephrotic syndrome were randomly divided into observation group and control group. The observation group was treated with cyclophosphamide pulse therapy combined with hormone therapy, and the control group was treated with routine hormone therapy. Results The overall response rate was higher, with statistical significance (P<0.05), incidence of adverse events was significantly lower than the control group, there was statistically significant (P<0.01). Conclusion Cyclophosphamide pulse therapy combined with hormone higher value in the treatment of nephrotic syndrome.

  4. The Discussion of Genetic Research and Diagnosis of Nephrotic Syndrome%对遗传性肾病综合征的基因诊断研究与讨论

    Institute of Scientific and Technical Information of China (English)

    刘俊泽

    2015-01-01

    Study and discuss genetic diagnosis of hereditary nephrotic syndrome. Target sequence capture using genetic sequencing technology nephropathy diagnosis and treatment of disease. Regimen hereditary nephrotic syndrome is more complex, while the conventional regimen no significant effects, such as hormone therapy and immunosuppressive hair hair, so the definition of the class of diseases, molecular genetics classification, clinical features, and kidneys pathology and other related developments need to be understood and clear. Target sequence capture using sequencing techniques, can make a one-time NS for genetic testing, diagnosis and treatment. Using molecular biological methods for the diagnosis of hereditary kidney disease, help biology, clinical and pathological features and other common molecular genetic study of nephrotic syndrome, to establish genetic nephrotic syndrome diagnostic thinking.%研究讨论遗传性肾病综合征的基因诊断。采用靶序列捕获测序技术对遗传性肾病的病症进行诊断与治疗。遗传性肾病综合征的治疗方案较为复杂,而常规的治疗方案没有明显的效果,如激素和免疫抑制剂。所以,对该类疾病的定义、分子遗传学分类、临床特征以及肾脏病理等相关进展需要进行理解和明确。采用靶序列捕获测序技术,可以一次性对遗传性NS进行检测、诊断和治疗。采用靶序列捕获测序技术对遗传性肾病进行诊断,可以帮助对遗传性肾病综合征的常见分子生物学、临床及病理特征等进行研究,并且为建立遗传性肾病综合征的基因提供诊断依据。

  5. In silico analysis of functional nsSNPs in human TRPC6 gene associated with steroid resistant nephrotic syndrome.

    Science.gov (United States)

    Joshi, Bhoomi B; Koringa, Prakash G; Mistry, Kinnari N; Patel, Amrut K; Gang, Sishir; Joshi, Chaitanya G

    2015-11-01

    The aim of the present study is to identify functional non-synonymous SNPs of TRPC6 gene using various in silico approaches. These SNPs are believed to have a direct impact on protein stability through conformation changes. Transient receptor potential cation channel-6 (TRPC6) is one of the proteins that plays a key role causing focal segmental glomerulosclerosis (FSGS) associated with the steroid-resistant nephritic syndrome (SRNS). Data of TRPC6 was collected from dbSNP and further used to investigate a damaging effect using SIFT, PolyPhen, PROVEAN, and PANTHER. The comparative analysis predicted that two functional SNPs "rs35857503 at position N157T and rs36111323 at position A404V" showed a damaging effect (score of 0.096-1.00).We modeled the 3D structure of TRPC6 using a SWISS-MODEL workspace and validated it via PROCHECK to get a Ramachandran plot (83.0% residues in the most favored region, 12.7% in additionally allowed regions, 2.3% in a generously allowed region and 2.0% were in a disallowed region). QMEAN (0.311) and MUSTER (10.06) scores were under acceptable limits. Putative functional SNPs that may possibly undergo post-translation modifications were also identified in TRPC6 protein. It was found that mutation at N157T can lead to alteration in glycation whereas mutation at A404V was present at a ligand binding site. Additionally, I-Mutant showed a decrease in stability for these nsSNPs upon mutation, thus suggesting that the N157T and A404V variants of TRPC6 could directly or indirectly destabilize the amino acid interactions causing functional deviations of protein to some extent.

  6. Hipotiroidismo, miocardiopatía dilatada y síndrome nefrótico durante el embarazo Hypothyroidism, dilated cardiomyopathy and nephrotic syndrome during pregnancy

    Directory of Open Access Journals (Sweden)

    Ariel K. Saad

    2011-02-01

    Full Text Available El hipotiroidismo en el embarazo es infrecuente, pero cuando ocurre suele asociarse con complicaciones maternas y fetales. Se presenta el caso de una mujer joven sin antecedentes de enfermedad cardiovascular que consulta por ortopnea, dolor torácico y edema de miembros inferiores. Los exámenes pusieron en evidencia la existencia de insuficiencia cardíaca, hipotiroidismo, síndrome nefrótico e insuficiencia renal. El eco-Doppler mostró dilatación de las cuatro cavidades cardíacas con deterioro grave de la función sistólica. El tratamiento con levotiroxina por vía intravenosa mejoró el cuadro clínico y los parámetros de laboratorio. Se analizan los efectos de la hormona tiroidea sobre el aparato cardiovascular y se comentan los mecanismos fisiopatológicos de la insuficiencia cardíaca en el embarazo.Hypothyroidism during pregnancy is infrequent, but its presence is associated with maternal and fetal complications. We present the case of a young pregnant woman with no previous history of cardiovascular disease, who consulted for orthopnea, chest pain and edema in both legs. Laboratory tests demonstrated a hypothyroid condition and a nephrotic syndrome with renal failure. The echo-Doppler exam showed a four chamber dilatation with systolic dysfunction. Treatment with intravenous levothyroxine improved her medical condition. We analyze the effects of thyroid hormone on the heart and vascular system and discuss the pathophysiologic mechanisms of heart failure during pregnancy.

  7. Urinary IgG and α2-Macroglobulin Are Powerful Predictors of Outcome and Responsiveness to Steroids and Cyclophosphamide in Idiopathic Focal Segmental Glomerulosclerosis with Nephrotic Syndrome

    Directory of Open Access Journals (Sweden)

    Claudio Bazzi

    2013-01-01

    Full Text Available Objective. To assess whether high-molecular-weight proteins excretion predicts outcome and therapy-responsiveness in patients with FSGS and nephrotic syndrome. Research Design and Methods. Thirty-eight patients measured at biopsy fractional excretion of IgG (FEIgG and urinary α2-macroglobulin/creatinine ratio (α2m/C. Low and high risk groups were defined by cutoffs assessed by ROC analysis. In all patients first-line therapy was with steroids alone or in combination with cyclophosphamide. Results. α2m/C and FEIgG were correlated with segmental sclerosis (r=0.546; r=0.522. Twenty-three patients (61% entered Remission and 9 (24% progressed to ESRD. Comparing low and high risk groups, by univariate analysis remission was predicted by FEIgG (77% versus 25%, P=0.016 and α2m/C (81% versus 17%, P=0.007 and ESRD at best by FEIgG (0% versus 75%, P<0.0001 and α2m/C (4% versus 67%, P<0.0001. By multivariate analysis FEIgG was the only independent predictor of remission and α2m/C the most powerful predictor of ESRD. Low and high risk groups of FEIgG and α2m/C in combination had very high predictive value of sustained remission and ESRD in response to therapy. Conclusions. FEIgG and α2m/C are powerful predictors of outcome and responsiveness to steroids and cyclophosphamide; their predictive value, if validated in prospective studies, may be useful in clinical practice suggesting first-line alternative treatments in high risk patients.

  8. Steroid-resistant idiopathic nephrotic syndrome in children: long-term follow-up and risk factors for end-stage renal disease

    Directory of Open Access Journals (Sweden)

    Alberto Zagury

    2013-09-01

    Full Text Available INTRODUTION: Steroid resistant idiopathic nephrotic syndrome (SRINS in children is one of the leading causes of progression to chronic kidney disease stage V (CKD V/end stage renal disease (ESRD. OBJECTIVE: The aim of this retrospective study is to evaluate the efficacy of immunosuppressive drugs (IS and to identify risk factors for progression to ESRD in this population. METHODS: Clinical and biochemical variables at presentation, early or late steroid resistance, histological pattern and response to cyclosporine A (CsA and cyclophosfamide (CP were reviewed in 136 children with SRINS. The analyzed outcome was the progression to ESRD. Univariate as well as multivariate Cox-regression analysis were performed. RESULTS: Median age at onset was 5.54 years (0.67-17.22 and median follow up time was 6.1 years (0.25-30.83. Early steroid-resistance was observed in 114 patients and late resistance in 22. Resistance to CP and CsA was 62.9% and 35% respectively. At last follow-up 57 patients reached ESRD. The renal survival rate was 71.5%, 58.4%, 55.3%, 35.6% and 28.5% at 5, 10, 15, 20 and 25 years respectively. Univariate analysis demonstrated that older age at onset, early steroid-resistance, hematuria, hypertension, focal segmental glomerulosclerosis (FSGS, and resistance to IS were risk factors for ESRD. The Cox proportional-hazards regression identified CsAresistance and FSGS as the only predictors for ESRD. CONCLUSION: Our findings showed that CsA-resistance and FSGS were risk factors for ESRD.

  9. Proportionate increase of fibrinogen and albumin synthesis in nephrotic patients : Measurements with stable isotopes

    NARCIS (Netherlands)

    de Sain-van der Velden, MGM; Kaysen, GA; de Meer, K; Stellaard, F; Voorbij, HAM; Reijngoud, DJ; Rabelink, TJ; Koomans, HA

    1998-01-01

    Hyperfibrinogenemia is a common feature of the nephrotic syndrome, and contributes to increased tendency for thrombosis and atherosclerosis. Its genesis is not certain, but the increase in liver fibrinogen mRNA in nephrotic rats indicates increased synthesis. Data in humans are scarce. We presently

  10. The Practice of Clinical Pharmacists'Participation in the Treatment of One Case with Nephrotic Syndrome%临床药师参与1例肾病综合征患者的治疗实践

    Institute of Scientific and Technical Information of China (English)

    马文明

    2015-01-01

    OBJECTIVE:To explore the effect of the clinic participation of pharmacists in the treatment of the patient with nephrotic syndrome .METHODS:Through participating in the treatment of a certain patient with nephrotic syndrome , pharmacists assisted the doctor to adjust the drug use program and analyze the rationality of the application of diuretics .RESULTS:The suggestions provided by clinical pharmacists were accepted by doctors , and the patient got better and discharged from the hospital .CONCLUSIONS:Clinical participation of pharmacists in the establishment of clinic treatment program can effectively solve the clinical problems and promoted rational drug use .%目的:探讨临床药师在临床药物治疗中所发挥的作用。方法:临床药师参与1例肾内科肾病综合征患者的临床治疗过程,参与药物治疗方案的调整,分析利尿剂应用的合理性。结果:临床药师的建议被医师采纳,患者病情好转出院。结论:临床药师参与临床药物治疗方案的制订,可有效解决临床问题,有利于促进合理用药。

  11. Mechanisms of hypercoagulability and thromboembolism in pediatric primary nephrotic syndrome%儿童原发性肾病综合征并发高凝状态与血栓栓塞的机制

    Institute of Scientific and Technical Information of China (English)

    张莹

    2012-01-01

    高凝状态和血栓栓塞在原发性肾病综合征(PNS)中发生率很高,且其形成机制颇有争议,包括内皮损伤,血小板活化,凝血功能和抗凝血功能失衡,以及肾病综合征(NS)导致的蛋白尿、低蛋白血症、高胆固醇血症等.近年来,大规模临床病例对照研究进一步阐释了NS患者高凝状态与血栓栓塞的机制.%Patients with the nephrotic syndrome are at increased risk of developing hypercoagulable states and thromboembolism, the most common of which is renal vein thrombosis. There are several unanswered or controversial issues relating to the mechanisms of the hypercoagulability and thromboembolism in the nephrotic syndrome which include endothelial dysfunction, platelet activation, functional disequilibrium between coagulation and anticoagulation, and proteinuria, hypoproteinemia, hypercholesterolemia caused by NS. In the recent years, the mechanisms have been updated by many case-control studies.

  12. Rituximab in treatment of children with frequently relapsed nephrotic syndrome%利妥昔单抗在小儿频复发肾病综合征中的应用

    Institute of Scientific and Technical Information of China (English)

    都娟; 黄建萍; 王硕; 赵晓艳; 肖丽丽

    2014-01-01

    Objective To explore the effect and safety of rituximab (RTX) in children with frequently relapsed nephrotic syndrome.Methods Twelve children (8 male and 4 female) with frequently relapse nephrotic syndrome were treated with RTX.They all showed resistance to various immunosuppressive agents and relapsed when steroid was reduced at 1.5-2.0 mg/kg.All immunosuppressive agents were stopped.Steroid was prescribed at doses of 2 mg/(kg · d),followed by alternate-day dosing (2 mg/kg) after proteinuria was negative for 5 days,and then tapered by 5 mg every 2 weeks until to low dose (< 0.5 mg/kg on alternate day) or discontinued.RTX was administered at a dose of 375 mg/m2 once every week for 3 weeks.Results The patients were followed up for 4-19 months [median (7.79 ± 3.00) months].Proteinuria turned negative on 1-14 d after first RTX treatment.Mean steroid dosages were significantly reduced than before treatment [alternate-day dosing (1.83 ± 1.02) mg/kg vs alternate-day dosing (0.34 ± 0.16) mg/kg,t =3.78,P =0.002].Gingival hyperplasia and Cushing appearance were significantly improved and steroid-induced glaucoma disappeared.The height was significantly increased compared with before treatment [(2.21 ± 1.40) cm/year vs (8.27 ±2.10) cm/year].Six cases had no relapse during follow-up.Another 6 cases relapsed in 5.5-19.0 months.All patients showed CD19 + B lymphocytes depletion after RTX treatment.However,CD19 + recovery could be seen in 5.5-12.0 months.Serum immunoglobulin did not significantly change.None of the patients was found with adverse events.Conclusions RTX can significantly reduce the dosage of steroid or even stop medication in children free of immunosuppressive agents,maintain remission for (7.79 ± 3.00) months,significantly improve the adverse reaction induced by steroid and immune-suppression.Thus,for children with frequently relapsed nephritic syndrome/steroid-dependent nephrotic syndrome who show resistance to immunosuppressive therapies

  13. 178例儿童肾病综合征免疫抑制剂用药分析%Analysis of Immunosuppressive Agents Application in 178 Cases with Pediatric Nephrotic Syndrome

    Institute of Scientific and Technical Information of China (English)

    高茗; 张征; 何旭

    2012-01-01

    Objective To understand the drug use trend of immunosuppressive agents in pediatric nephrotic syndrome and to evaluate the rationality of drug use for promoting the clinical pharmaciststo better participate in pediatric rational drug use. Methods The rational medication PASS software system was adopted to access the medication information of the pediatric patients diagnosed with nephrotic syndrome from July to September 2010. The Clinical Application Guidelines of Gglucocorticosteroid' and the 'Evidence Based Guideline of Common Renal Diseases in Children formulated' by the Ministry of Public Health were used as the evaluation standard. Results The main drug for treating nephrotic syndrome was glucocorticoid, its dosage and course conformed to the requirements. Because the disease is easy to generate glucocorticoid resistance,other immunosuppressants are often used alone or combined with glucocorticoid. The data analysis showed that pediatric immunosuppressant application adopted the individuation treatment according to different stages of the disease. Combination of immunosuppressants also choose drugs affecting different locus. Conclusion From the drug use trend and experience, besides glucocorticoid,tacrolimus and tripterygium glucoeides are common immunosuppressants in pediatric nephrotic syndrome and have become the promising effective ways to treat nephrotic syndrome.%目的 了解医院儿科肾病综合征免疫抑制剂用药趋势,评价用药合理性,促进临床药师更好地参与儿科合理用药.方法 利用合理用药(PASS)软件系统,调出医院儿科2010年7至9月确诊为肾病综合征入院患者的疾病及用药信息,以国家卫生部《糖皮质激素类药物临床应用指导原则》《儿童常见肾脏疾病诊治循证指南》为评价标准.结果 医院治疗肾病综合征的主要药物为糖皮质激素,且用药剂量和疗程均符合要求.由于该疾病易产生激素耐药,因此临床常将激素与其他免疫

  14. Class Ⅱ lupus nephritis with nephrotic syndrome%伴肾病综合征Ⅱ型狼疮性肾炎的临床病理特征

    Institute of Scientific and Technical Information of China (English)

    刘瑾; 陈惠萍; 陈浩; 曾彩虹; 刘正钊; 章海涛; 刘志红; 胡伟新

    2012-01-01

    目的:比较临床表现肾病综合征(NS)和非NS的Ⅱ型狼疮性肾炎(LN)患者的临床、病理特征和预后. 方法:回顾性分析经肾活检病理确诊为Ⅱ型LN患者124例,其中女性112例,男性12例,平均年龄29.2±11.4岁,根据尿蛋白定量分为NS组(尿蛋白定量≥3 g/d,血清白蛋白<30 g/L)和非NS组(尿蛋白定量<3g/d,血清白蛋白≥30 g/L,伴或不伴镜下血尿).比较两组患者的临床、免疫学指标、肾脏病理特征及预后. 结果:124例Ⅱ型LN中NS组27例(21.8%),非NS组97例(78.2%),两组患者性别、发病年龄和病程无差异.NS组以肾损害为首发症状(77.8%vs15.5%,P<0.01)及病程中出现急性肾损伤的比例(29.6%vs0,P<0.01)均显著高于非NS组,而皮疹(40.7% vs 69.1%,P<0.01)、发热(14.8%vs62.9%,P<0.01)和关节炎(29.6%vs75.3%,P<0.01)的发生率及血清抗-dsDNA阳性率(29.6%vs52.6%,P<0.05)明显低于非NS组.肾活检病理NS组患者肾小球中重度系膜增生的比例显著低于非NS组(7.4%vs59.8%,P<0.01),电镜下肾小球足细胞足突广泛融合的比例显著高于非NS组(88.9%vs0,P<0.01).NS组和非NS组激素治疗均获得高缓解率(100%vs98.4%,P=0.882),但NS组复发率显著高于非NS组(69.9%vs33.3%,P<0.01),两组分别随访8~125月(中位时间55月)和6~274月(中位时间57月),均未发生终末期肾病.7例复发者重复肾活检,NS组2例病理类型均未转型,非NS组5例均发生转型. 结论:表现为NS的Ⅱ型LN其本质为足细胞病,非NS的Ⅱ型LN为系膜增生性病变,两者临床表现、免疫学异常及病理转型的显著差异表明两者的发生机制不同,狼疮足细胞损伤的机制有待进一步研究.%Objective: To investigate the clinical characteristics, renal histological features and the prognosis in patients of Class Ⅱ LN with nephrotic syndrome. Methodology;One hundred twenty four cases(112 females, 12 males, mean age 29. 22 ± 11

  15. 家庭护理干预对肾病综合症儿童疗效及心理适应行为的影响%nephrotic syndrome

    Institute of Scientific and Technical Information of China (English)

    骆成珠; 银青梅; 周月琼; 周琼花

    2014-01-01

    目的:探讨家庭护理干预对肾病综合症儿童疗效及心理适应行为的影响。方法对100名肾病综合症儿童随机分为家庭干预组和对照组,家庭干预组实施家庭护理干预后,比较两组儿童疗效和心理适应行为差异。结果家庭干预组儿童的疗效和心理适应行为和对照组的相比均有明显改善。结论家庭护理干预能降低肾病综合症儿童复发率,提高治疗疗效,并且能改善其心理适应行为。%Objective To investigate the impact of family nursing intervention on efficacy and psychological adaptive behavior of children with nephrotic syndrome.Methods 100 children with nephrotic syndrome were randomly divided into control group and family intervention group, the control group accepted routine nursing intervention and drug treatment, while the intervention accepted family nursing intervention and drug treatment, observing children's efficacy and psychological adaptation behavior difference.Results The efficacy and psychological adaptive behavior of children with family nursing intervention were advanced comparing to the control group children.Conclusions Family nursing intervention can effectively reduce relapse rates and improve the efficacy and their psychological adaptive behavior of children with nephritic syndrome.

  16. 护理干预在小儿肾病综合征中的应用效果观察%Effect of nursing intervention on nephrotic syndrome in children

    Institute of Scientific and Technical Information of China (English)

    刘春妍

    2011-01-01

    Objective To investigate the effect of nursing intervention on nephrotic syndrome in children. Methods 64 cases with nephrotic syndrome were randomly divided into the study group and the control group, with 32 cases in each group. Non-discriminatory treatment of two groups, both were given diet, hormone therapy, control high blood pressure, blood lipids and adjust to correct water and electrolyte disorders treatment. Patients in the control group received routine care, while those in the study group were given nursing interventions during hospitalization, in cluding psychological care, guidance of diet and medication, health education, medication guidance. Compliance be havior of children, the rates of cure and recurrence were observed. Results The compliance behavior and clinical ef ficacy of children in the study group were significantly better than that in the control group (P<0.01, P<0.05); The re currence rate in the study group was lower than that in the control group (P<0.05). Conclusion Intervention can im prove care of children with nephrotic syndrome in compliance behavior and reduce relapse.%目的 探讨护理干预在小儿肾病综合征中的应用效果.方法 将我院收治的肾病综合征患儿64例随机分为研究组和对照组,每组32例.对照组患儿给予常规护理,研究组患儿在常规护理的基础上给予护理干预,包括住院期间的心理护理,指导饮食及用药;出院前的健康教育,用药指导;出院后的定期随访等方法.观察两组患儿遵医行为、治愈及复发情况.结果 ①研究组患儿遵医行为较对照组显著提高(P<0.01);临床疗效较对照组明显改善(P<0.05).②出院后随访1年,研究组患儿复发率较对照组低(P<0.05).结论 护理干预可以提高小儿肾病综合征的遵医行为,提高治疗效果,降低复发率.

  17. Investigation and analysis of subjective quality of life in children with primary nephrotic syndrome%原发性肾病综合征患儿主观生活质量的调查分析

    Institute of Scientific and Technical Information of China (English)

    李君丽; 韦琴

    2013-01-01

    Objective To evaluate the quality of life in children with primary nephrotic syndrome and analyze the influencing factors of the quality of life of the population.Methods The quality of life in children with primary nephrotic syndrome was assessed with Inventory of Subjective Life Quality for Children and Adolescent(ISLQ) edited by CHENG Zao-huo and compared to 55 normal children.The influencing factors were analyzed with multi-linear Logistic regression analysis.Results The total degree of satisfaction,recognition and emotion satisfaction of life of quality in patient children were significantly lower than those in healthy children.The data in each satisfaction degree in patient children were lower than those in healthy children,thereinto,the degree of satisfaction in score of home life,school life,depression practice and anxiety practice,self-cognition in patient children were significantly lower than those in healthy children.Conclusions The quality of life in patient children were significantly lower than that in healthy children,they should accept the comprehensive treatment intervention.%目的 评估原发性肾病综合征(primary nephrotic syndrome,PNS)患儿的生活质量,并分析该人群生活质量的影响因素.方法 采用程灶火等编制的儿少主观生活质量问卷(ISLQ)对58例PNS患儿(病例组)的生活质量进行评定,并与55名正常同龄儿童(对照组)进行对比,采用多元线性回归分析其影响因素.结果 病例组患儿的生活质量总体满意度、认知满意度和情感满意度均明显低于对照组,差异有统计学意义.各维度满意水平均低于对照组,其中家庭生活、学校生活、自我认识、焦虑体验、抑郁体验维度得分比较差异有统计学意义.家庭经济状况影响患儿的生活状况,二者成正相关关系.结论 PNS患儿的生活质量明显低于正常儿童,应对PNS患儿进行综合的干预治疗和护理.

  18. 肾病综合征并发深静脉血栓和(或)肺栓塞临床分析%Clinical analysis of deep venous thrombosis and (or) pulmonary embolism in nephrotic syndrome

    Institute of Scientific and Technical Information of China (English)

    王亚芬; 孙东; 柳彩侠; 冯锦红; 崔爱东

    2011-01-01

    Objective To explore the related risk factors, diagnosis and treatment of nephrotic syndrome (NS)complicated with deep venous thrombosis (DVT) and (or) pulmonary embolism (PE). Methods A retrospective study was conducted on the clinical data of 10 cases of nephrotic syndrome complicated with DVT and (or) PE, which was compared with 50 synchronous cases of nephrotic syndrome with no thrombosis as to age (AGE) , plasma albumin ( ALB ), 24 - hour urine protein ( 24 - UP), total cholesterol ( CHOL), triglyceride ( TG), platelet (PLT) and fibrinogen (FIB). Results In the thrombosis group, 6 patients underwent renal biopsy , 3 of whom had membranous nephropathy. There were significant differences in the indicators such as AGE , ALB, 24 - UP, FIB between the thrombosis group and the non -thrombosis group (P< 0.05 ); the differences in CHOL, TG and PLT had no statistical significance (P>0.05 ). The thrombi disappeared in the thrombosis group following a series of therapies including anticoagulation , thrombolysis, implantation of inferior vena cava filter (IVCF) , etc. Conclusion AGE, ALB, 24 - UP and FIB play very important roles in the formation off DVT and (or) PE in NS. Membranous nephropathy is the most common pathological type in NS with thromboembolism. In cases of NS complicated with DVT and (or) PE, anticoagulation, thrombolysis or interventional therapy should be conducted as required. Implantation of IVCF is an effective method to prevent DVT leading to PE .%目的 探讨肾病综合征(nephrotic syndrome,NS)并发深静脉血栓形成 (DVT)和(或)肺栓塞(PE)的相关危险因素及诊治体会.方法 对10例确诊的NS并发DVT和(或)PE患者(血栓组)的临床资料进行回顾性分析,并与同期50例NS无血栓患者(无血栓组)的年龄(AGE)、血浆白蛋白(ALB)、24小时尿蛋白定量(24-UP)、总胆固醇(CHOL)、三酰甘油(TG)、血小板(PLT)、纤维蛋白原(FIB)等指标进行比较.结果 血栓组10例,肾活检6

  19. Genetic Aspects of Nephrotic Syndrome

    DEFF Research Database (Denmark)

    Joshi, Shivani

    glomerulosclerosis (FSGS) in a renal biopsy, and a high risk of developing end stage renal disease. Variants in several NS genes like NPHS1, NPHS2, PLCE1, ACTN4, TRPC6, and INF2 have improved our understanding of the pathogenesis of SRNS. Patients with SSNS generally have a good renal prognosis but often develop...

  20. Increased VLDL in nephrotic patients results from a decreased catabolism while increased LDL results from increased synthesis

    NARCIS (Netherlands)

    de Sain-van der Velden, M; Kaysen, GA; Barrett, HA; Stellaard, F; Gadellaa, MM; Voorbij, HA; Reijngoud, DJ; Rabelink, TJ

    1998-01-01

    Increased very low density lipoprotein (VLDL) in nephrotic patients results from a decreased catabolism while increased low density lipoprotein (LDL) results from increased synthesis. Hyperlipidemias a hallmark of nephrotic syndrome that has been associated with increased risk for ischemic heart dis

  1. 肾病综合征、糖尿病、癫痫、预激综合征和神经肌源性损害%Recurrent nephrotic syndrome with diabetes,epilepsy,pre-excitation syndrome and neuromyopathy-case report

    Institute of Scientific and Technical Information of China (English)

    全军肾脏病研究所学术委员会; 许书添; 谢红浪; 刘志红

    2012-01-01

    A 19-year-old female patient with mitochondria] disease manifested primarily nephrotic syndrome, following diabetes, epilepsy, pre-excitation syndrome and neuromyopathy was reported. A novel change 8969G > A mutation in mitochondrial-DNA was found in peripheral blood. This case may be helpful for clinicians to pay attention on mitochondrial disease,which usually manifest multisystemie dysfunction with a broad clinical spectrum,not simply confined to ihe diagnosis of kidney, to avoid errors and missions of diagnosis. When the tests for common mitochondrial DNA (mtDNA) point mutations are negative and the mtDNA defects are still hypothesized, it is necessary to screen the entire mitochondrial genome for unknown mutations in order to confirm the diagnosis.%青年女性患者,肾病综合征起病,病程中逐渐出现糖尿病、癫痫、预激综合征及神经肌源性损害等多系统病变;经外周血细胞线粒体基因组检测证实线粒体DNA 8969位点G→A发生点突变,它是线粒体病的一个新基因位点突变.提示一旦高度疑似线粒体病,虽未发现经典位点基因突变,仍需检测整个线粒体基因组.警示临床医师应根据患者肾外多系统受累并结合相关实验室检查综合分析,不要仅限于肾活检病理诊断,以免误诊、漏诊.

  2. Clinical significance of determining urinary podocalyxin level in children with primary nephrotic syndrome%检测原发性肾病综合征患儿尿足细胞标志蛋白的临床意义

    Institute of Scientific and Technical Information of China (English)

    刘涛; 张碧丽; 李莉

    2012-01-01

    Objective To study the clinical significance of urinary podoeyte marker protein podocalyxin (PCX) in the diagnosis of primary nephrotic syndrome (PNS) in children and the evaluation of disease severity. Methods PCX levels in the first morning urine were measured by turhidimetric immunoassay (TIA) in 175 children, including 53 children with acute PNS[36 cases of simple nephrotic syndrome (NS) and 17 cases of nephritic NS], 56 children with PNS in the remission stage (relapsed: 42 cases) and 66 healthy children ( control group). Twenty-four hour urinary protein was measured in the 53 children with acute PNS. The optimal operating points for the diagnosis of acute PNS and nephritic NS were determined using the receiver-operating characteristic curve ( ROC carve). Results Significant increasd levels of urinary PCX were found in children with acute PNS compared with those in the remission stage and the control group ( P < 0.01 ). A positive correlation was found between urinary PCX and 24 hour urinary protein in children with acute PNS (r = 0.39,P<0.01). In children with acute PNS, urinary PCX levels were significantly higher in the nephritic NS group than in the simple NS group (P < 0.05). In children in the remission stage, a significant increase in levels of urinary PCX was found in children who had relapsed compared with those who had not (P <0.05). The area under the ROC curve for the diagnosis of acute PNS and nephritic NS was 0.915 and 0.784 respectively. The optimal operating point for the diagnosis of acute PNS and nephritic NS was 7.97 and 10. 2S ng/mL respectively, with a sensitivity and specificity of 81.1% and 93.4% respectively for acut? PNS and of 94.1% and52.8% respectively for nephritic NS. Conclusions The quantitative detection of urinary PCX is useful in the evaluation of podoeyte dynamic changes. It is helpful in the diagnosis of acute PNS and in the differentiation of nephritic NS and simple NS.%目的 探讨检测尿液中足细胞标志蛋

  3. Síndrome nefrótica córtico-sensível e diabetes mellitus tipo 1 de início simultâneo Simultaneous onset of steroid-sensitive nephrotic syndrome and type 1 diabetes

    Directory of Open Access Journals (Sweden)

    Eduardo A. Rego Filho

    2003-11-01

    with steroid-sensitive nephrotic syndrome coexisting with type-1 diabetes mellitus. The interest to this clinical case is due to the unusual association of these diseases, the clinical symptoms and laboratory tests used to confirm diagnosis and the difficulties on corticotherapy. DESCRIPTION: Nephrotic syndrome was diagnosed in a boy (age 3 years and 11 months with generalized edema. Marked weight loss (23 to 16 kg, polyuria, polydipsia and weakness were observed after three weeks of treatment with prednisone 2 mg/kg/day. Diabetic ketoacidosis was confirmed by laboratory tests: hyperglycemia (glucose 657 mg/dl, glycosuria without proteinuria, acidosis and ketonuria. Therapy with insulin and prednisone was started. He was then maintained on a daily dose of NPH insulin. At age 4 years and 1 month a new episode of ketoacidosis without proteinuria occurred in association with a viral infection of the upper airways. At age 4 years and 4 months nephrotic syndrome relapsed, but the child responded well to steroid therapy. There was another relapse three months later, when prednisone treatment was interrupted. This led to the introduction of cyclophosphamide, with good results. Since then, the patient (now 5 years and 6 months old has been taking insulin daily and nephrotic syndrome has not relapsed. Plasma levels of C3 and C4 and renal function are normal. Hematuria is occasionally present. Anti-GAD antibodies (glutamic decarboxilase are normal and anti-islet cell antibodies are positive. HLA antigens: A2; B44; B52; DR4; DR8; DR53. COMMENTS: The simultaneous occurrence of steroid-sensitive nephrotic syndrome and type-1 diabetes mellitus is rare. The literature data, the familiar pattern and studies on HLA antigens are discussed.

  4. 原发性肾病综合征继发甲状腺功能异常的机制及治疗%Mechanism and treatment of thyroid dysfunction secondary to primary nephrotic syndrome

    Institute of Scientific and Technical Information of China (English)

    周维燕; 潘斌斌; 杜新

    2012-01-01

    临床上原发性肾病综合征(PNS)继发甲状腺功能异常的病例并不少见.近年来多项研究对于该现象进行了深入的探讨,提出了各种可能的病理生理机制,目前尚无统一定论.早期行甲状腺激素补充及抗氧化应激等治疗,可以帮助患者维持甲状腺功能正常,保护肾小球结构、改善内皮功能障碍、利于原发疾病的缓解等.儿童PNS患者外源摄入甲状腺激素联合依那普利还可促进其自身机体发育、骨骼生长及智力成熟.动态观察患者血清甲状腺激素水平可作为观察病情、判断疗效以及预后的敏感指标.%It's not uncommon that thyroid dysfunction is secondary to primary nephrotic syndrome.Recently a number of studies have explored this phenomenon and several possible pathophysiological mechanisms have been put forward.However,at present there is no unified conclusion.If thyroid hormone supplement and anti-oxidative stress treatment are given at early stages,patients may benefit a lot such as maintaining normal thyroid function,protecting glomerular structure,improving endothelial dysfunction and easing the primary diseases,etc.Intaking exogenous thyroid hormone as well as enalaprilat also can help children with primary nephrotic syndrome to promote their own body development,bone growth and intellectual maturity.Dynamic monitor of serum thyroid hormone level can served as a sensitive index for illness evaluation,response judgment and prognosis.

  5. Nephrotic syndrome of intracranial venous sinus thrombosis nursing care%肾病综合征并发颅内静脉窦血栓的护理效果观察

    Institute of Scientific and Technical Information of China (English)

    徐敏; 张青; 夏正坤; 王慧; 贾丽丽; 吴婷婷; 胡蝶; 杨红萍; 杨波; 滕彦

    2011-01-01

    目的 探讨应用尿激酶与低分子肝素等联合治疗肾病综合征患儿并发颅内静脉窦血栓后的护理效果.方法 采用尿激酶与低分子肝素等联合治疗,观察血压、神志、尿量、肢体色泽、温度及有无皮下出血情况.结果 观察发现肾病综合者患儿在激素治疗的过程中易合并高凝状态,肾病综合征的高凝状态是静脉血栓形成的基础, 临床上一旦患儿血压升高并发头痛、头昏、嗜睡、呕吐等症状时,应尽早进行头颅CT或MRI检查以便了解有无头颅静脉血栓,并常规运用尿激酶与低分子肝素联合治疗.结论 早期应用尿激酶抗纤溶、低分子肝素抗凝及抗血小板凝聚药物综合治疗,其溶栓疗效较好.本文对儿童肾病综合征并发颅内静脉窦血栓的早期护理观察具有推广价值.%Objective To explore the effect of the application of urokinase and low molecular heparin in nephrotic syndrome children with the complication of intracranial venous thrombosis.Methods To apply urokinase and low molecular heparin unitedly, and observe blood pressure, consciousness, urine output, body color, temperature and the presence of subcutaneous bleeding. Resuits We observed that children with nephrotic syndrome easily have hypercoagulation, on which basis venous thrombosis forms. Once headache, dizziness, drowsiness, vomiting occur, head CI or MRI examination should be applied in order to know whether the intracranial vein thrombosis is complicated. Conclusion Early application of urokinase and low molecular heparin and anti-platelet coagulation drugs has good effect.

  6. 频复发肾病综合征患儿家庭功能状况调查%Survey of family function condition of children with frequently relapsing nephrotic syndrome

    Institute of Scientific and Technical Information of China (English)

    吕林华; 李智英; 刘晓红

    2016-01-01

    [目的]探讨频复发肾病综合征患儿家庭功能现状及其影响因素。[方法]采用家庭功能评定量表(FAD)及自行设计的一般资料问卷,对广州市某三级甲等医院180例频复发肾病综合征住院患儿的家长进行调查。[结果]与广州市普通儿童家庭相比,频复发肾病综合征患儿的家庭功能在角色、行为控制、沟通及总体功能方面水平偏低(P<0.05),问题解决能力优于普通儿童家庭(P<0.05);主要照顾者为母亲的家庭情感介入功能较好(F=4.653,P=0.018),照顾者职业对问题解决功能存在影响(F=3.107,P=0.044),家庭居住地在城市者其家庭角色功能优于农村家庭(t=-3.114,P=0.004),确诊时间对家庭问题解决(F=3.587,P=0.042)及行为控制(F=5.506,P=0.010)均存在影响。[结论]频复发肾病综合征患儿家庭功能存在一定的功能障碍,主要照顾者、照顾者职业、家庭居住地、确诊时间均为家庭功能的影响因素。在护理工作中应当根据家庭及患儿情况,为家庭成员提供针对性的健康教育及心理护理。%Objective:To probe into the family function status quo of children with frequently relapsing nephrotic syndrome and its influencing factors.Methods:A total of 180 parents of children with frequently relapsing ne-phrotic syndrome from a third grade A hospital in Guangzhou were analyzed by using the Family Assessment Device(FAD)and self designed general information questionnaires.Results:Comparing with the families of normal children in Guangzhou,the family function of children with frequently relapsing nephrotic syndrome showed a low level in dimensions of role,behavior control,communication and overall function(P<0.05);but the problem solving ability showed a higher level than normal children's families(P<0.05).Emotional involve-ment showed a better function while mothers acting as the main caregivers for children(F=4.653,P=0.018), the job of caregivers affected the function of

  7. 前列腺素E1对原发性肾病综合征作用初探%EFFECT OF PROSTAGLANDIN E1 ONPRIMARY NEPHROTIC SYNDROME

    Institute of Scientific and Technical Information of China (English)

    周巧玲; 成小苗; 欧阳春; 解勤之; 陈立平; 胡杨清

    2002-01-01

    Objective:To investigate the effect of prostaglandin E1 (PGE1) (Alprostadii injection) on patients with primary nephrotic syndrome(PNS). Methods: 37 patients with PNS were recruited to study the effect of prostaglandin E1 on platelet aggregation function [ PAG (5,) PAG( m ) ], serum total protein (TP) , albumin (Al),blood urea nitrogen(BUN) ,serum creatinine(Scr) ,cholesterol(CHO), triglyceride(TG), protein in 24-hour urine (Pr/24h) and platelet account (PLT). Results: TP, Al, CHO, TG, BUN, Scr, Pr/24h, PAG(5) and PAG(m) in PNS group before treatment were significantly different from those in control group(P<0.05, P<0.01) while no significant difference was found for PLT. When treated with PGE1 , TP,Al,CHO, TG, Pr/24h, ADP- induced PAG(5) ,and Adr- induced PAG(5) and PAG(m) were significantly different from those before treatment (P<0.05). Adr- induced PAG(5) and PAG(m) were significantly different. Adr- induced PAG(5) was xsitively correlated with BUN and Scr in PNS(P<0.01). Similar correlation was found between ADP-induced PAG(5) and Al ,BUN,Scr,Pr/24h(P<0.05), AD- induced PAG(m) and TP,CHO(P<0.05). Conclusions: PGE1 may be an effective drug for the treatment for hypercoagulation in patients with PNS.

  8. Multiple-factor analysis of proteinuria elimination time in children with primary nephrotic syndrome%儿童原发性肾病综合征尿蛋白转阴时间的多因素分析

    Institute of Scientific and Technical Information of China (English)

    元君辉; 王启林

    2014-01-01

    目的:分析儿童原发性肾病综合征激素治疗后尿蛋白转阴时间的影响因素。方法146例原发性肾病综合征患儿行激素治疗后,可评价疗效136例。按照尿蛋白转阴时间分为A组(尿蛋白转阴时间≤1周)和B组(1周1周的危险因素,与患儿的不良预后相关,临床应予以高度重视。%Objective To analyze the influencing factors of proteinuria elimination time after hormone therapy in chil-dren with primary nephrotic syndrome (PNS). Methods 146 cases of children with PNS were treated by hormone, and a-mong them 136 patients were evaluated for efficacy and tolerability. The patients were divided into two groups accord-ing to proteinuria elimination time. The proteinuria elimination time≤1 week of patients were in group A, while 1 week 1 week in children with PNS, which also relate to the worse prognosis and need to be paid more atten-tion in clinic.

  9. Clinic research of pediatric primary nephrotic syndrome of treatment of Huaiqihuang puls glucocorticoid%槐杞黄颗粒联合糖皮质激素治疗小儿原发性肾病综合征的临床研究

    Institute of Scientific and Technical Information of China (English)

    冯仕品; 谢敏; 王莉; 罗苇; 张伟; 翟洋

    2011-01-01

    normal, the infected numbers and the change of cellular immunity ( CD3, CD4,CD8,CD4/CD8) and humoral immunity(IgA,IgM,IgG) before and after treatment. Observation period was twelve weeks. Results In the treatment group, the time of edema regression and prourine normal was shorter than that in the contrast group(P 0. 05). Tow groups had nausea, vomit,Diarrhea,no rash and the abnormal of liver and kidney function, there were no Statistically significant difference. Conclusion The adjuvant treatment of Huaiqihuang granule on primary nephrotic syndrome can shorten the time of edema regression and prourine normal, enhance the immulogical regulation function, and decrease the chance of infection. Also the adjuvant treatment of Huaiqihuang granule enhance the curative effect of glucocorticoid and improve prognosis of the disease. There is no side effect in the process,which confirm that Huaiqihuang granule is safety and effective adjuvant drug of primary nephrotic syndrome.

  10. Late de novo minimal change disease in a renal allograft

    Directory of Open Access Journals (Sweden)

    Madhan Krishan

    2009-01-01

    Full Text Available Among the causes of the nephrotic syndrome in renal allografts, minimal change disease is a rarity with only few cases described in the medical literature. Most cases described have occurred early in the post-transplant course. There is no established treatment for the condition but prognosis is favorable. We describe a case of minimal change disease that developed 8 years after a successful transplantation of a renal allograft in a middle-aged woman. The nephrotic syndrome was accompanied by deterioration of allograft function. Treatment with mycophenolate mofetil was successful in inducing remission and stabilizing allograft function.

  11. Multicenter study on present status of diagnosis and treatment of steroid-resistant nephrotic syndrome in children%激素耐药型肾病综合征患儿诊治现状多中心调研报告

    Institute of Scientific and Technical Information of China (English)

    中华医学会儿科学分会肾脏病学组

    2014-01-01

    Objective To investigate the treatment of multi-center hospitalized children with steroid resistant nephrotic syndrome (SRNS) retrospectively.Method The Subspecialty Group of Nephrology,The Society of Pediatrics,Chinese Medical Association organized 35 hospitals to investigate children with primary SRNS from 2008 to 2011.By filling in the questionnaire,data including age,gender,clinical type,related examination,pathology and treatment in local hospitals were collected,and then statistical analysis was performed.Result A total of 35 hospitals participated in this survey,577 patients were enrolled,their age was from 8 months to 18 years.The male to female ratio was 2∶ 1.School-age children accounted for 33.3% (n =194).Clinical types were mostly simple nephrotic syndrome,which accounted for 60.5% (n =349) and nephritis nephrotic syndrome accounted for 39.5% (n =228).The incidences of different types of NS were significantly different between 2008 and 2011 (x2 =10.751,P =0.001).There were 306 cases of infants who received renal biopsy.Renal pathology showed that 40 of them had mesangial proliferative glomerulonephritis (MsPNG),17 focal segmental glomerulo-sclerosis (FSGS),9 membranous nephropathy (MN),9 minimal change disease (MCD),8 minor lesions,3 endocapillary proliferative glomerulonephritis (EnPGN),2 membrano-proliferative glomerulonephritis (MPGN),2 sclerotic glomerulonephritis and 2 podocyte lesions in 2008.Otherwise there were 53 MsPGN,35 FSGS,25 minor lesions,23 MCD,13 MN,5 MPGN,3 podocyte lesions,2 EnPGN,1 sclerotic glomerulonephritis.Since the evidence-based guidelines had been issued in 2011,209 cases were treated with adequate-dose prednisone therapy > 4 weeks program.Eighty-seven children with nephrotic syndrome whose urinary protein could not be turned negative after 4 weeks' glucocorticoid treatment were treated with high-dose methylprednisolone for 1 course.Thirtyeight of these children got negative urinary protein after the pulse therapy

  12. Clinical experience for treating infantile nephrotic syndrome by asthenia in origin and asthenia in superficiality and sequenced differentiation of symptoms and signs%从本虚标实、序贯辨证论治小儿肾病综合征临床体会

    Institute of Scientific and Technical Information of China (English)

    张汶娟; 刘素云; 任献青

    2013-01-01

      全国名老中医丁樱教授、知名专家翟文生教授以及任献青博士在临床中积累了丰富的治疗小儿肾病的经验。本文对其进行了总结整理。%The national distinguished veteran TCM doctors DING Ying, the famous expert ZHAI Wen-sheng and Doctor REN Xian-qing have abundant experiences on treating infantile nephrotic syndrome. In this article, their experiences were summarized.

  13. 探讨尿N-乙酰-β-D-氨基葡萄糖苷酶检测对原发性肾病综合征的临床价值%Discusses the urine N-acetyl-β-D-amino grape glycosidase to test the application value of the primary nephrotic syndrome

    Institute of Scientific and Technical Information of China (English)

    刘红; 伊力夏提; 岳华; 陆晨; 刘颖; 赵红娟

    2013-01-01

    Objectives To investigate the clinical value of urine NAG in Primary nephrotic syndrome patients.Methods 52 patients with primary nephrotic syndrome were enrolled who were in-patients,dated from December 2008 to May 2011,in patients before and after the use of hormone by colorimetric method to detect the urine NAG,and the detection results compare and statistical analysis.Results Primary nephrotic syndrome patients use hormone therapy,the detection of positive urine NAG for 9.62%,urinary protein qualitative results for (-) ~ +,a statistically significant difference(P < 0.05).Conclusions Urine NAG enzyme detect can as a primary nephrotic syndrome sensitive indexes,the curative judgment and delay the progress of kidney disease has an important clinical significance.%目的 探讨尿N-乙酰-β-D-氨基葡萄糖苷酶(N-acetyl-β-D-glucosa minidase,NAG)检测对原发性肾病综合征患者的临床价值.方法 选择2008年12月~2011年5月在新疆维吾尔自治区人民医院肾病科住院的原发性肾病综合征患者52例为病例组,对患者使用激素前、后采用对硝基苯酚(PNP)比色法检测尿NAG,并对检测结果进行比较及统计学分析.结果 原发性肾病综合征患者使用激素治疗后,检测尿NAG阳性率为9.62%,尿蛋白定性结果为(-)~+,差异具有统计学意义(P<0.05).结论 尿NAG酶检测可以作为原发性肾病综合征的敏感指标,对疗效判断及延缓肾脏疾病的进展具有重要的临床意义.

  14. 小儿肾病综合征相关骨代谢异常及防治%Prevention and treatment of nephrotic syndrome associated bone metabolic abnormality in children

    Institute of Scientific and Technical Information of China (English)

    徐海霞; 姚勇

    2015-01-01

    Metabolic bone disease in nephrotic syndrome(NS) are increasingly being renal physician's attention.As calcium binding protein and VitD binding protein losing with a large number of protenuria, the bone metabolic biochemical abnormalities had happened at the beginning of the onset of the nephrotic syndrome, and is further exacerbated by therapeutic high-dose or long course of glucocorticoids (GC) application.The main mechanism of the glucocorticoid-induced osteoporosis (GIOP) is for GC to inhibit the activity of osteoblasts and promote apoptosis of osteoblasts and formation of osteoclasts, resulting in secondary hyperparathyroidism,leading to increasing the risk of osteoporosis,slow growth and fracture,seriously harm to children's physical and mental health.The biomarkers of bone transform can prompt the NS with bone metabolic abnormalities early;vertebral body bone dual-energy X-ray absorptiometry bone mineral density detection is the best method and position to determine GIOP.As the most commonly used and effective means to prevent and control metabolic bone disease, calcium supplements and VitD were always taken when GC was treated for NS, even the dosage of GC was very low.So far, it is still lack of guideline of prevention and treatment of bone metabolic abnormalities in NS in children.%肾病综合征(NS)时的代谢性骨病正日益受到儿肾科医师的关注.在NS起病之初因钙结合蛋白、维生素D结合蛋白自尿中丢失,骨代谢生化异常即已发生.治疗性糖皮质激素(GC)大剂量、长疗程的应用则进一步加剧了骨代谢异常,其主要机制为GC抑制成骨细胞的活性、促进成骨细胞的凋亡以及促进破骨细胞的生成、引起继发性甲状旁腺功能亢进,导致骨质疏松、生长迟缓、骨折风险增加,严重危及儿童身心健康.骨转换的生物标志物可以早期反映NS患儿骨代谢的异常;椎体骨双能X线吸收法骨密度检测是判断GC相关性骨质疏松症的最佳

  15. 呈肾病综合征的乙型肝炎病毒相关性肾炎临床病理特点%Clinicopathological Features of Hepatitis B Virus-associated Glomerulonephritis Patients with Nephrotic Syndrome

    Institute of Scientific and Technical Information of China (English)

    刘波; 庄永泽; 余英豪

    2013-01-01

    目的:探讨呈肾病综合征(NS)的乙型肝炎病毒相关性肾炎(hepatitis B virus-associated glomerulonephritis,HBV-GN)的临床病理特点.方法:回顾性分析呈NS且肾穿组织内小动脉≥5条的HBV-GN患者152例的临床病理资料,光镜下观察其肾内小动脉病变,比较伴与不伴肾内小动脉病变患者的临床病理资料.结果:平均年龄26.3岁,男:女为3.6:1;伴高血压者36例(23.7%)、贫血52例(34.2%)、肾衰竭者33例(21.7%)、肝功能异常者20例(13.2%).乙肝大三阳者87例(57.2%);血清和肾组织内HBV-DNA载量呈正相关(P<0.01).病理表现以膜性肾性(MN)、系膜增生性肾炎(MsPGN)、膜增生性肾炎(MPGN)为主;未成年组与成年组MN、MPGN所占比例不同(P<0.05).伴有肾内小动脉病变者84例(55.2%),与无肾内小动脉病变者相比,其高血压发生率较高(P<0.05),24 h尿蛋白定量显著较高(P<0.01),贫血、肾间质纤维化的发生率显著较高(P<0.01).结论:呈NS的HBV-GN患者绝大多数为HBV携带者且伴有病毒复制,病理以MN、MsPGN及MPGN为主,半数以上伴有肾内小动脉病变,伴有肾内小血管病变者高血压、贫血及肾小管间质纤维化发生率较高,蛋白尿更严重,为预后不良的病理学指标.%Objective:To Investigate the clinicopathological features of hepatitis B virus -associated glomerulonephritis patients with nephrotic syndrome. Methods:The clinical and pathological data of 152 cases with nephrotic syndrome and renal biopsy tissue small artery cross - sectional ≥ 5 of HBV - GN were analyzed retrospectively. Small intrarenal arterial lesions( SRAL ) were observed, and the clinical and pathological data in patients with or without SRAL were compared. Results: Among them, there was a 3. 6:1 predominance of males, mean age was 26. 3 years,36( 23. 7% ) patients broken out with hypertension, 52( 34. 2% ) with anemia, 33( 21. 7% ) suffered renal dysfunction, 20( 13. 2% ) liver function were impaired, 87

  16. Padrões morfológicos de lesão glomerular e correlação com achados clinicolaboratoriais de 43 crianças com síndrome nefrótica Morphologic patterns of glomerular lesion and correlation with clinical and laboratory findings of 43 children with nephrotic syndrome

    Directory of Open Access Journals (Sweden)

    Márcia Camegaçava Riyuzo

    2004-10-01

    Full Text Available OBJETIVOS: Avaliar a associação entre os parâmetros clinicolaboratoriais e alteração morfológica de biópsias renais em crianças com síndrome nefrótica. MÉTODOS: Os dados foram obtidos dos prontuários médicos de 43 crianças com síndrome nefrótica submetidas a biópsia renal. RESULTADOS: Vinte e oito pacientes eram do sexo masculino (65,1%, idades entre 1,4 a 12 anos (média de 4,7±3,2. Quarenta e dois pacientes (97,7% apresentaram edema; 83,7%, oligúria e 32,5%, hipertensão arterial. A média de proteinúria foi 15,3g/1,73m²SC/dia e 55,8% apresentaram hematúria microscópica. As biópsias renais mostraram: glomerulonefrite proliferativa mesangial (GNPM em 37,2%, glomeruloesclerose segmentar e focal (GESF em 27,9%, alterações glomerulares mínimas (LM em 25,6%, glomerulonefrite membranoproliferativa (GNMP em 7% e glomerulonefrite membranosa (GNM em 2,3%. Vinte e seis pacientes (60,5% apresentaram resistência ao corticosteróide. Idade, sexo, hipertensão arterial, oligúria, uréia e creatinina séricas não mostraram diferenças estatísticas significativas entre os pacientes com GNPM, GESF e LM. Os pacientes com GNPM e GESF apresentaram maior freqüência de hematúria microscópica (p OBJECTIVES: To evaluate the association between clinical features and laboratory findings with the morphological changes in children with nephrotic syndrome. METHODS: The data were obtained from medical records of 43 children with nephrotic syndrome submitted to renal biopsy. RESULTS: Twenty-eight patients were male (65.1%, aged 1.4-12 years (mean 4.7 ± 3,2. Forty-two patients (97,7% presented edema, 83.7% oliguria and 32.5% hypertension. The mean of proteinuria was 15.3g/1.73m² BSA per day and 55.8% presented microscopic hematuria. Renal biopsies showed: proliferative mesangial glomerulonephritis (PMGN in 37.2%, focal segmental glomerulosclerosis (FSGS in 27.9%, minimal change disease (MCD in 25.6%, membranoproliferative

  17. 黄芪注射液对儿童原发性肾病综合征高凝状态的影响%Effect of Astragalus Inj ection on Hypercoagulation State in Children with Primary Nephrotic Syndrome

    Institute of Scientific and Technical Information of China (English)

    陈文波; 张经; 吴学典

    2014-01-01

    目的:探讨黄芪注射液对儿童原发性肾病综合征(primary nephrotic syndrome,PNS)高凝状态的影响。方法将41例PNS患儿随机分为治疗组21例、对照组20例。2组均给予泼尼松片1.5~2 mg·kg-1·d-1口服治疗,在常规激素治疗基础上治疗组给予黄芪注射液1 mL · kg-1· d-1静脉滴注治疗,对照组给予阿司匹林1~3 mg· kg-1· d-1口服治疗。15 d 为1个疗程,2组均治疗2个疗程。结果2组治疗后血生化指标血尿素氮(BUN)、肌酐清除率(Ccr)、血肌酐(Scr)、血浆清蛋白(A1b)、总胆固醇(TC)、三酰甘油(TG),24 h尿蛋白定量及血液高凝状态指标血纤维蛋白原降解产物(FDP)、血浆纤维蛋白原(FIB)浓度、血 D-二聚体(D-D)较治疗前均显著改善(P<0.01),且治疗组改善效果明显优于对照组,差异有统计学意义(P<0.05);2组凝血酶原时间(PT)指标治疗前后比较差异无统计学意义(P>0.05)。结论黄芪注射液对PNS患儿高凝状态有显著疗效,同时可降低蛋白尿,保护肾功能。%Objective To investigate the effect of astragalus injection on hypercoagulation state in children with primary nephrotic syndrome(PNS).Methods Forty-one children were randomly divided into treatment group(n=21)and control group(n=20).All patients were treated with o-ral prednisone tablets (1 .5-2 mg · kg-1 · d-1 ).In addition,treatment group and control group were given two 15-day cycles of intravenous infusion of astragalus injection(1 mL·kg-1·d-1) and oral aspirin(1-3 mg·kg-1 ·d-1 ),respectively.Results Blood urea nitrogen,creatinine clear-ance rate,serum creatinine,plasma albumin,total cholesterol,triacylglycerol,24-hour protein ex-cretion,fibrinogen degradation products,plasma fibrinogen and D-dimer were significantly im-proved after treatment in both group(P0.05).Conclusion Astragalus injection is effec-tive for hypercoagulation state

  18. Clinicopathological features of cosmetics mercury poisoning-related nephrotic syndrome: A case report%1例化妆品汞中毒相关肾病综合征临床病理分析

    Institute of Scientific and Technical Information of China (English)

    李红艳; 魏日胞; 王远大; 杨勇

    2011-01-01

    目的 探讨化妆品汞中毒相关肾病的临床病理特点及治疗方法.方法 解放军总医院肾科2010年1 1月收治的1例女性化妆品汞中毒病例,分析其临床病理特点及对驱汞治疗的反应.结果 该病例用含汞美白化妆品半年,临床表现为肾病综合征,血压及肾功能正常,血清自身抗体系列正常.病理特点表现为膜性肾病Ⅰ期,患者未用免疫抑制剂,驱汞治疗效果较好,尿蛋白逐渐减少,全身症状改善.结论 本例化妆品汞中毒相关肾病表现为肾病综合征,病理为膜性肾病Ⅰ期,对驱汞治疗反应较好.%Objective To study the clinicopathological features of cosmetics mercury poisoning-related nephropathy and its treatment methods. Methods Clinicopathological features of a female patient with cosmetics mercury poisoning-related nephropathy admitted to Department of Nephrology, Chinese PL A General Hospital, in November 2010 and her response to antimercurialism were analyzed. Results The patient developed nephritic syndrome after using whiten cosmetics that contains mercury for half a year. Her blood pressure, renal function, and serum autoantibody were normal. Pathologic examination showed stage 1 membranous nephropathy. The patient did receive any immunosuppressive agent. After antimercurialism, her urinary protein gradually decreased and systemic symptoms improved. Conclusion Cosmetics mercury-related nephropathy is manifested as nephrotic syndrome (which was diagnosed as stage I membranous nephropathy in this patient who had a rather good response to antimercurialism).

  19. Neuroblastoma Presenting with Acute Kidney Injury, Hyponatremic-Hypertensive-Like Syndrome and Nephrotic Proteinuria in a 10-Month-Old Child

    Directory of Open Access Journals (Sweden)

    Giovanni Maria Poggi

    2011-08-01

    Full Text Available Neuroblastoma is the most common extracranial solid tumor in childhood. Its presenting signs and symptoms may be highly variable, depending on the location of the primary tumor and its local or metastatic diffusion and, rarely, with paraneoplastic syndrome such as opsoclonus-myoclonus-ataxia syndrome and gastrointestinal disturbances, due to autoantibodies or to aberrant secretion of vasoactive intestinal peptide. Herein we describe a 10-month-old child with neuroblastoma presenting with a complex clinical picture characterized by acute kidney injury manifested by renal insufficiency and signs and symptoms of tubulointerstitial damage, with polyuria, polydipsia, glucosuria, aminoaciduria and hypochloremic metabolic alkalosis, and of glomerular damage with heavy proteinuria. Imaging study documented a suprarenal mass enveloping the aorta and its abdominal and renal ramifications and bilaterally renal veins. This clinical picture shows some analogies with the hyponatremic-hypertensive syndrome concerning the renovascular disease; however, in absence of systemic arterial hypertension, the heavy proteinuria and the polyuria could be explained by sectional increased intraglomerular pressure, due to local renal blood vessels constriction. Hypochloremic metabolic alkalosis probably developed because of local production of renin, responsible of renin-angiotensin-aldosterone system activation, but above all because of chloride loss through sweating. The long lasting dehydration, due to vomiting, sweating and polyuria, caused prolonged prerenal failure evolving in proximal tubular damage manifestations.

  20. Hemostatic problems and thromboembolic complications in nephrotic children.

    Science.gov (United States)

    Citak, A; Emre, S; Sâirin, A; Bilge, I; Nayir, A

    2000-02-01

    A hypercoagulable state and the risk of thromboembolism in both arterial and venous circulation is a relatively frequent and serious feature of nephrotic syndrome (NS) in children and adults. The aim of this study was to evaluate the coagulation states of children with NS before and after corticosteroid (CS) therapy and to compare the results with a healthy control group. The first group consisted of 49 nephrotic children (30 boys and 19 girls) with a mean age of 6. 5+/-4.9 years (range 1-16 years). The control group included 17 healthy children (9 boys and 8 girls). At the time of admission, all patients were evaluated for the presence of clinical thromboembolism, hematological and biochemical indicators of a hypercoagulative state, and renal disease. This was repeated after CS treatment. Deep vein thrombosis was observed in 2 nephrotic patients who had very low plasma antithrombin III (AT III) levels and fibrinogen levels above 750 mg/dl. Thus, the prevalence of thromboembolism was 4% in our pediatric nephrotic population. The mean AT III level of the study group was 68.2+/-23.4% at the onset of the disease, which was significantly lower than the level of the control group (84.0+/-7. 6%). Plasma AT III levels increased to 74.4+/-15.3% after CS treatment, which correlated with the serum albumin levels. However, there was no correlation with urinary protein excretion. Protein C levels were higher than controls during all stages of the disease in both steroid-responsive and -unresponsive patients. The mean protein S level was similar in both groups. Plasma fibrinogen and cholesterol levels were significantly higher in the study group but decreased to within normal limits with remission. Our study suggests that thromboembolic complications are not infrequent in children with NS, and may be related to low plasma AT III and albumin and high fibrinogen and cholesterol levels.

  1. Plasma alpha(2) macroglobulin is increased in nephrotic patients as a result of increased synthesis alone

    NARCIS (Netherlands)

    Sain-van der Velden, MGM; Rabelink, TJ; Reijngoud, DJ; Gadellaa, MM; Voorbij, HAM; Stellaard, F; Kaysen, GA

    1998-01-01

    Background. alpha(2) Macroglobulin (alpha(2)M), a protease inhibitor, is often increased in plasma of patients with the nephrotic syndrome. Although it has been speculated that synthesis is increased, no direct measurements have been performed. Methods. alpha(2)M synthesis in both normal subjects (N

  2. 肾病综合征的临床治疗预后分析%Analysis of prognosis of patients with nephrotic syndrome

    Institute of Scientific and Technical Information of China (English)

    贺建军

    2014-01-01

    目的:探讨肾病综合征的临床中西药结合治疗预后。方法:肾病综合征成人患者60例根据随机抽签法分为观察组与对照组各30例,所有患者都采用激素治疗,在此基础上观察组加用自拟中药汤治疗,疗程为4周。结果:观察组与对照组总有效率分别为93.3%和73.3%,2组对比差异明显(P<0.05)。治疗后2组的SCr和BUN值都明显下降,与治疗前对比差异明显,同时上述值在治疗后的组间对比差异明显(P<0.05)。结论:肾病综合征在临床上采用中西药结合治疗能改善肾功能,提高预后疗效。%Objective:To discuss the prognosis of patients with nephritic syndrome treated by combination of Chinese traditional and western medicine .Methods:60 adults with nephritic syndrome were divided randomly into observation group and control group with 30 pa-tients each group.All the patients were treated by hormonotherapy .Observation group were treated by self -made Chinese medicine .The course was 4 weeks.Results:The total effective rates of observation group and control group were 93.3%and 73.3%respectively.The difference between two groups was obviously (P<0.05).The SCr and BUN in two groups were obviously reduced after treatment .The differences between before and after treatment in each group were obviously .The differences of SCr and BUN between two groups after treatment were obviously(P<0.05).Conclusion:Combination of Chinese traditional and western medicine for patients with nephritic syn -drome could improve the renal function and prognosis .

  3. Analysis of prognosis of patients with nephrotic syndrome%肾病综合征的临床治疗预后分析

    Institute of Scientific and Technical Information of China (English)

    贺建军

    2014-01-01

    Objective:To discuss the prognosis of patients with nephritic syndrome treated by combination of Chinese traditional and western medicine .Methods:60 adults with nephritic syndrome were divided randomly into observation group and control group with 30 pa-tients each group.All the patients were treated by hormonotherapy .Observation group were treated by self -made Chinese medicine .The course was 4 weeks.Results:The total effective rates of observation group and control group were 93.3%and 73.3%respectively.The difference between two groups was obviously (P<0.05).The SCr and BUN in two groups were obviously reduced after treatment .The differences between before and after treatment in each group were obviously .The differences of SCr and BUN between two groups after treatment were obviously(P<0.05).Conclusion:Combination of Chinese traditional and western medicine for patients with nephritic syn -drome could improve the renal function and prognosis .%目的:探讨肾病综合征的临床中西药结合治疗预后。方法:肾病综合征成人患者60例根据随机抽签法分为观察组与对照组各30例,所有患者都采用激素治疗,在此基础上观察组加用自拟中药汤治疗,疗程为4周。结果:观察组与对照组总有效率分别为93.3%和73.3%,2组对比差异明显(P<0.05)。治疗后2组的SCr和BUN值都明显下降,与治疗前对比差异明显,同时上述值在治疗后的组间对比差异明显(P<0.05)。结论:肾病综合征在临床上采用中西药结合治疗能改善肾功能,提高预后疗效。

  4. 1例难治性肾病综合征患儿免疫抑制药物选择的药学实践%Pharmaceutical Practice in Selection of Immunosuppressive Drugs for One Case of Refractory Nephrotic Syndrome

    Institute of Scientific and Technical Information of China (English)

    孙会静; 陈文颖

    2015-01-01

    ABSTRACTThrough the analysis and intervention conducted by clinical pharmacist in the selection of immuno-suppressive drugs for a child patient with refractory nephrotic syndrome, a safe and effective treatment regime with immunosuppressive drugs for the child was made with the assistance of clinical pharmacist, and the role played by the pharmacist in the treatment was explored.%通过临床药师对1例难治性肾病综合征患儿选择免疫抑制药物进行的分析和干预,协助医师制定免疫抑制药物治疗方案,探讨药师在临床治疗中发挥的作用。

  5. 桂西地区壮族人肾素-血管紧张素系统基因多态与原发性肾病综合征的相关分析%Correlation analysis between rennin-angiotensin system gene polymorphism and primary nephrotic syndrome in Zhuang population of West of Guangxi Zhuang Autonomous Region

    Institute of Scientific and Technical Information of China (English)

    尤燕舞; 林栩; 王洁; 杨发奋

    2012-01-01

    Objective To analysis the correlation between three key genes of angiotensin II type 1 receptor (AT,R) gene Al 166C polymorphism, angiotensin I converting enzyme (ACE) gene insertion/deletion (I/D) polymorphism and angiotensinogen (AGT) gene M235T polymorphism in rennin-angiotensin system (RAS) and primary nephrotic syndrome in Zhuang population of West of Guangxi Zhuang Autonomous Region. Methods 112 cases of patients with primary nephrotic syndrome in Zhuang population of West of Guangxi Zhuang Autonomous Region (nephropathy group) and 150 cases of normal controls (control group) were selected for case-control study. Polymerase chain reaction-restriction fragment length polymorphism technique, direct polymerase chain reaction technique and DNA sequencing method were used to detect the RAS ACE gene I/D polymorphism, AT(R gene A1166C polymorphism and AGT gene M235T polymorphism. The distributions of 3 genes polymorphism in the two group were statistically compared. Results The ACE gene I/D polymorphism in the nephropathy group was different from the normal control group, D genotype and D allele in the nephropathy group were accounted for a significant advantage (P 0.05). Conclusion D allele of ACE I/D polymorphism is one of the predisposing factors of primary nephrotic syndrome in Zhuang population of West of Guangxi Zhuang Autonomous Region. This study has not found the relevance between AT,R gene A1166C polymorphisms, AGT gene M235T polymorphisms and primary nephrotic syndrome.%目的 分析桂西地区壮族人肾素-血管紧张素系统中血管紧张素Ⅱ1型受体(AT1R)基因A1166C多态、血管紧张素Ⅰ转化酶(ACE)基因插入/缺失(I/D)多态及血管紧张素原(AGT)基因M235T多态与原发性肾病综合征的相关性.方法 选取112例桂西地区壮族原发性肾病综合征患者(肾病组)及150例正常对照者(对照组)进行病例对照研究,采用聚合酶链反应-限制性片段长度多态性技术、直接聚合酶链反应

  6. Relationship between epstein-barr virus and children primary nephrotic syndrome%EB病毒感染与儿童原发性肾病综合征关系的研究

    Institute of Scientific and Technical Information of China (English)

    李素娥; 唐骏; 吴乐天

    2014-01-01

    Objective To explore the relationship between epstein-barr virus (EBV)infection and children primary nephrotic syndrome. Methods Blood samples and renal biopsy tissue of 66 children with PNS were analyzed as PNS group,the EBV DNA、VCA antibodies (VCA-IgM、VCA-IgG),atypical lympho-cyte were detected.Samples of 68 children without kidney disease were enrolled as control group. The differ-ence between two groups about EBV DNA,VCA-IgM、VCA-IgG,atypical lymphocyte in blood were com-pared. EBV DNA、hemic-renal EBV DNA in renal tissue were also compared between simple type and nephrit-ic type of PNS. Results The positive rates of VCA-IgM and VCA-IgG in PNS group were higher than those of control group (P<0.05). And compared with simple type of PNS,the positive rates of VCA-IgMand VCA-IgG in nephritic type of PNS were significantly higher (P<0.05 ). The positive rate of hemic-renal EBV DNA expression in nephritic type of PNS was also higher than that of simple type of PNS (P<0.05 ). Conclusion There may be some relations between EBV and children PNS,especially nephritic type of PNS.%目的:探讨EB病毒感染与儿童原发性肾病综合征(PNS)的关系。方法收集66例PNS患儿血液和肾组织标本行EB病毒DNA和EB病毒壳抗原抗体VCA-IgM、VCA-IgG以及异形淋巴细胞检测,并将同期住院的68例非肾脏疾病患儿作为对照,比较两组患儿血EB病毒DNA、VCA-IgM、VCA-IgG以及异形淋巴细胞的差异,并比较单纯型及肾炎型PNS患儿的肾EB病毒DNA、血-肾EB病毒DNA表达差异。结果 PNS 患儿血 VCA-IgM及 VCA-IgG 的阳性率高于对照组(P 均<0.05)。与单纯型 PNS 相对比,肾炎型 PNS 患儿血 VCA-IgM及 VCA-IgG 的阳性率更高(P 均<0.05);且肾炎型PNS患儿血、肾组织均有EB病毒DNA表达者的阳性率高于单纯型PNS患儿(P<0.05)。结论 EB病毒与儿童PNS可能有一定关联,特别是与肾炎型PNS。

  7. 我院肾病综合征超说明书用药分析%Analysis of Off-label Drug Use in Nephrotic Syndrome in Our Hospital

    Institute of Scientific and Technical Information of China (English)

    黄婧; 于西全; 陈威

    2016-01-01

    Objective:To analyze the rationality of off-label drug use in nephritic syndrome prescriptions to provide scientific basis for clinical rational drug use and further regulate the off-label drug use in our hospital. Methods:Totally 1 908 outpatient prescriptions of nephritic syndrome selected from our hospital during November 2014 to April 2015 were analyzed, and all the off-label drugs were listed . The rationality of the off-label drug use was analyzed and evaluated by searching the related kidney disease guidelines and litera-tures. Results:The off-label drug use of tacrolimus capsules, mycophenolate mofetil capsules, cyclophosphamide for injection, ciclos-porin soft capsules, dipyridamole tablets, hydroxychloroquine sulfate tablets and warfarin sodium tablets were recommended by domes-tic and overseas guidelines with better evidence of evidence-based medicines. Tripterygium glycosides tablets and leflunomide tablets were supported by the literatures on clinical studies at home and abroad. The above 9 kinds of off-label drugs were rational drug use. Lumbrokinase enteric-coated capsules, salvia miltiorrhiza and ligustrazine injection and pidotimod dispersible tablets were reported only by a handful of journals. Bacteria lysate capsules and boric acid powder had no related information support, which belonged to the em-pirical prescriptions of physicians. The above 5 kinds of off-label drugs were not rational drug use. Conclusion:It is a widely existing phenomenon that the medication in nephrotic syndrome is beyond the instruction, the most of off-label drug use are reasonable, and cli-nicians should prescribe medicines carefully. Our hospital needs to further standardize the management of off-label drug use supported by higher evidence in order to improve the level of clinical rational drug use and the reasonable rate of prescriptions.%目的::分析我院治疗肾病综合征超说明书用药的合理性,为临床合理用药提供科学依据,并进一

  8. Serum osteoprotegerin level in children with nephrotic syndrome and the effect of glucocorticoid on it%肾病综合征患儿血清骨保护素改变及糖皮质激素对其的影响

    Institute of Scientific and Technical Information of China (English)

    李玉柳; 王华

    2012-01-01

    目的 观察肾病综合征( nephrotic syndrome,NS)患儿骨保护素改变及糖皮质激素治疗对骨保护素的影响,探讨骨保护素在NS骨代谢改变中的应用价值.方法 随机选取NS患儿44例,其中NS初发患儿24例,糖皮质激素治疗减量过程中复发患儿20例,其糖皮质激素累积剂量为28327±5879 mg/m2;23例年龄性别匹配的正常儿童设为对照组.采用酶联免疫吸附法测定血清骨保护素浓度,采用电化学发光免疫法测定血清骨钙素N端中分子片段(骨钙素N-MID)含量.结果 初发NS组血清骨保护素、骨钙素N-MID含量分别为211±55 ng/L、46±14 ng/mL,均明显低于对照组(分别为470±57 ng/L、73±9 ng/mL)(P<0.05).NS复发组血清骨保护素、骨钙素N-MID含量均较NS初发组及对照组低,分别为176±42 ng,/L、29±10 ng/mL(P<0.05).结论 NS患儿本身即存在骨代谢异常,大剂量应用糖皮质激素后可进一步加重骨代谢改变,提示NS患儿骨保护素改变受疾病本身及糖皮质激素治疗的双重影响,骨保护素有望成为预测NS患儿骨代谢改变的新型生化指标.%Objective To observe serum osteoprotegerin ( OPG) level in children with nephrotic syndrome ( NS) and changes in serum OPG level after glucocorticoid therapy, with the aim of studying the role of OPG in the bone metabolism of children with NS. Methods Forty-four children with idiopathic NS were randomly selected as the study group, including 24 newly diagnosed, untreated patients and 20 who had relapsed during the process of glucocorticoid reduction (cumulative dose of glucocorticoid 28327 + 5879 mg/m2). Twenty-three age- and sex-matched healthy children served as the control group. Serum osteoprotegerin ( OPG) level was measured using ELISA. Serum N-terminal midfragment of osteocalcin (N-MID osteocalcin) was determined using electrochemical luminescence immunoassays (ECLJA). Results Serum levels of OPG (211 ± 55 ng/L) and N-MID osteocalcin (46 ± 14 ng

  9. PLA2R antibody levels and clinical outcome in patients with membranous nephropathy and non-nephrotic range proteinuria under treatment with inhibitors of the renin-angiotensin system.

    Directory of Open Access Journals (Sweden)

    Elion Hoxha

    Full Text Available Patients with primary membranous nephropathy (MN who experience spontaneous remission of proteinuria generally have an excellent outcome without need of immunosuppressive therapy. It is, however, unclear whether non-nephrotic proteinuria at the time of diagnosis is also associated with good prognosis since a reasonable number of these patients develop nephrotic syndrome despite blockade of the renin-angiotensin system. No clinical or laboratory parameters are available, which allow the assessment of risk for development of nephrotic proteinuria. Phospholipase A2 Receptor antibodies (PLA2R-Ab play a prominent role in the pathogenesis of primary MN and are associated with persistence of nephrotic proteinuria. In this study we analysed whether PLA2R-Ab levels might predict development of nephrotic syndrome and the clinical outcome in 33 patients with biopsy-proven primary MN and non-nephrotic proteinuria under treatment with blockers of the renin-angiotensin system. PLA2R-Ab levels, proteinuria and serum creatinine were measured every three months. Nephrotic-range proteinuria developed in 18 (55% patients. At study start (1.2±1.5 months after renal biopsy and time of diagnosis, 16 (48% patients were positive for PLA2R-Ab. A multivariate analysis showed that PLA2R-Ab levels were associated with an increased risk for development of nephrotic proteinuria (HR = 3.66; 95%CI: 1.39-9.64; p = 0.009. Immunosuppressive therapy was initiated more frequently in PLA2R-Ab positive patients (13 of 16 patients, 81% compared to PLA2R-Ab negative patients (2 of 17 patients, 12%. PLA2R-Ab levels are associated with higher risk for development of nephrotic-range proteinuria in this cohort of non-nephrotic patients at the time of diagnosis and should be closely monitored in the clinical management.

  10. PLA2R antibody levels and clinical outcome in patients with membranous nephropathy and non-nephrotic range proteinuria under treatment with inhibitors of the renin-angiotensin system.

    Science.gov (United States)

    Hoxha, Elion; Harendza, Sigrid; Pinnschmidt, Hans; Panzer, Ulf; Stahl, Rolf A K

    2014-01-01

    Patients with primary membranous nephropathy (MN) who experience spontaneous remission of proteinuria generally have an excellent outcome without need of immunosuppressive therapy. It is, however, unclear whether non-nephrotic proteinuria at the time of diagnosis is also associated with good prognosis since a reasonable number of these patients develop nephrotic syndrome despite blockade of the renin-angiotensin system. No clinical or laboratory parameters are available, which allow the assessment of risk for development of nephrotic proteinuria. Phospholipase A2 Receptor antibodies (PLA2R-Ab) play a prominent role in the pathogenesis of primary MN and are associated with persistence of nephrotic proteinuria. In this study we analysed whether PLA2R-Ab levels might predict development of nephrotic syndrome and the clinical outcome in 33 patients with biopsy-proven primary MN and non-nephrotic proteinuria under treatment with blockers of the renin-angiotensin system. PLA2R-Ab levels, proteinuria and serum creatinine were measured every three months. Nephrotic-range proteinuria developed in 18 (55%) patients. At study start (1.2±1.5 months after renal biopsy and time of diagnosis), 16 (48%) patients were positive for PLA2R-Ab. A multivariate analysis showed that PLA2R-Ab levels were associated with an increased risk for development of nephrotic proteinuria (HR = 3.66; 95%CI: 1.39-9.64; p = 0.009). Immunosuppressive therapy was initiated more frequently in PLA2R-Ab positive patients (13 of 16 patients, 81%) compared to PLA2R-Ab negative patients (2 of 17 patients, 12%). PLA2R-Ab levels are associated with higher risk for development of nephrotic-range proteinuria in this cohort of non-nephrotic patients at the time of diagnosis and should be closely monitored in the clinical management.

  11. Effect of Shenkang injection combined with low molecular weight heparin sodium injection on a hypercoagulable state of the primary nephrotic syndrome%肾康注射液联合低分子量肝素钠注射液对原发性肾病综合征高凝状态的影响

    Institute of Scientific and Technical Information of China (English)

    牛凯; 冯珍; 刘冰; 史亚男; 董春霞

    2011-01-01

    Objective To investigate the effect of Shenkang injection combined with low molecular weight heparin sodium injection on a hypercoagulable state of the primary nephrotic syndrome. Methods 88 patients with primary nephrotic syndrome (PNS) were randomly divided into four groups, Dipyridamole tablets group, Shenkang injection group, low molecular weight heparin sodium injection group and combination therapy group. Patients in four groups were treated by conventional blood pressure, lipid lowering therapy, and oral methylprednisolone. Dipyridamole tablets group ( n =21) received Dipyridamole tablets. Shenkang injection group ( n = 22) and low molecular weight heparin sodium injection group ( n = 22) separately received Shenkang injection and low molecular weight heparin sodium injection. Combination therapy group ( n =23) received Shenkang injection combined with low molecular weight heparin sodium injection. The course was four weeks in four groups. The changes of 24 h urinary protein excretion, serum albumin, prothrombin time (PT) , activated partial thromboplastin enzyme time (APTT) and fibrinogen ( FIB) were measured before and after treatment. Results 24 h urinary protein excretion and FIB in Shenkang injection group and low molecular weight heparin sodium injection group were decreased as compared with those in Dipyridamole tablets group ( P <0. 05). Plasma albumin, PT and APTT in Shenkang injection group and low molecular weight heparin sodium injection group were increased as compared with those in control group ( P < 0. 05 ). 24 h urine protein excretion in Shenkang injection group was decreased as compared with that in low molecular weight heparin sodium injection group ( P <0. 05) , plasma albumin was increased ( P <0. 05). 24 h urinary protein excretion and FIB in combination therapy group were significantly decreased as compared with those in other three treatment groups ( P < 0. 05 ). Plasma albumin, PT and APTT were obviously increased in comparison

  12. Clinical significance of retinol-binding protein detection for diagnosis of nephrotic syndrome in children%视黄醇结合蛋白检测在肾病综合征患儿诊断中的临床意义

    Institute of Scientific and Technical Information of China (English)

    李浩军; 孟秀荣; 董晓妮

    2014-01-01

    Objective To explore the clinical significance of retinol‐binding protein detection for diagnosis of nephrotic syndrome in children .Methods A total of 60 cases of children with nephrotic syndrome were enrolled in the experimental group and divided into two groups (33 cases for the simple type nephrotic syndrome group and 27 cases for the nephritic type nephrotic syndrome group) .And other 30 cases of healthy children were selected as the healthy control group .The levels of retinol‐binding protein ,urea and creatinine were detected and analyzed .Results The levels of serum retinol‐binding protein ,urea and creatinine were higher in two experimental groups than those in healthy control group ,and in the two experimental groups the positive detectable rate of serum retinol‐binding protein was higher than that of urea and creatinine ,all with significant difference(P< 0 .05) .The levels of serum retinol‐binding protein detected after treatment were evidently higher than those detected before treatment(P<0 .05) ,and a certain correlation was found between levels of serum retinol‐binding protein and the clinical feature (r=0 .799 3 ,P<0 .05) .The diagnostic efficiency of retinol‐binding protein was the highest ,followed by urea and creatinine .Conclu‐sion The retinol binding protein detection could be with positive clinical value for the clinical diagnosis and thera‐peutic morniteration of children with nephrotic syndrome .%目的:探讨视黄醇结合蛋白检测在肾病综合征患儿诊断中的临床意义。方法选择2013年1~12月在涿州市中医院确诊为肾病综合征的患儿60例为试验组(单纯型肾病综合征组33例,肾炎型肾病综合征组27例),另选择30名健康儿童设为健康对照组,分别测定其血清视黄醇结合蛋白、尿素和肌酐水平并进行比较分析。结果试验组两组患儿的血清视黄醇结合蛋白、尿素和肌酐浓度水平均高于健康对照组,且试验组

  13. Characteristics of Mood Status in School-Aged Children with Nephrotic Syndrome and Their Primary Caregivers%学龄期肾病综合征患儿及其主要照顾者的心境现状

    Institute of Scientific and Technical Information of China (English)

    张大华; 张凡; 贾玉静; 张臻

    2012-01-01

    Objective To investigate the prevalence and characteristics of mood status in children with nephrotic syndrome ( NS ) and their primary caregivers. The correlative factors of the mood status of school - aged children with NS and their primary caregivers were analyzed. Methods There were 43 cases with NS. General information from enrolled subjects was collected by using self - designed questionnaires used in this study. Screening for Child Anxiety Related Emotional Disorders (SCARED) , Depression Self- Rating Scale for Children ( DSRSC), Self - rating Anxiety Scale (SAS) and Self - rating Depression Scale (SDS) were performed on the subjects. Statistical analysis was performed by using SPSS 14.0 software. Results 1. The total positive rates were 32.6% (14/43 cases) and 44.2% (19/43 cases) in children with NS and their primary caregivers, respectively. The scores of anxiety and depression in children with NS and their primary caregivers were higher than those in the general population,and there was significant statistical difference between the primary caregivers and the general population. 2. There was no correlation between sex, age, mothers' educational level, family finance status, family residence, disease relapses during the past 6 months, the primary caregivers' type, the primary caregivers' mood status and the mood status in children with NS. There was correlation among mood status of the primary caregivers, the primary caregivers' type, family residence, and disease relapses during the past 6 months. Conclusions Medical staff should pay attention to the mood status in children with NS and their primary caregivers. It should be recognized and treated as early as possible. The life quality of patients and their family might be improved through individual intervention.%目的 探讨学龄期肾病综合征(NS)患儿及其主要照顾者的心境现状,并分析影响学龄期NS患儿及其主要照顾者心境现状的相关因素.方法 NS患儿43例.采用

  14. Clinical analysis of spontaneous bacterial peritonitis in children with nephrotic syndrome%儿童肾病综合征并自发性细菌性腹膜炎临床分析

    Institute of Scientific and Technical Information of China (English)

    樊剑锋; 刘小荣; 孟群; 张桂菊; 周楠; 陈植; 蒋也平

    2015-01-01

    目的 探讨肾病综合征(NS)并自发性细菌性腹膜炎(SBP)患儿的临床特征.方法 回顾性总结2010年1月至2014年6月在首都医科大学附属北京儿童医院肾内科住院的11例NS合并SBP患儿的临床表现、实验室资料和治疗情况.结果 11例NS患儿共发生12例次SBP,其中1例患儿发生2次.男9例,女2例;发病年龄2岁11个月~14岁11个月.9例患儿接受糖皮质激素和/或免疫抑制剂治疗.11例患儿均有不同程度的腹痛,其中7例起病缓,全腹胀痛不伴反跳痛和腹肌紧张.出现发热10例,腹胀8例,腹泻5例,呕吐4例.2例患儿病程中突然出现休克.全部患儿存在高度水肿,其中5例表现为腹水迅速增加,利尿剂治疗无效.11例患儿外周血白细胞计数和C反应蛋白均升高.血清清蛋白水平明显降低.CD4辅助细胞比例和血IgG水平降低.腹水外观混浊呈脓样,中性粒细胞计数升高.2例患儿腹水培养和血培养肺炎链球菌阳性,均应用抗生素治疗.9例患儿应用单一抗生素治疗,疗程7~10 d.2例血培养阳性的危重患儿抗生素疗程4周.5例患儿采用腹腔穿刺放液治疗.1 1例患儿腹膜炎全部治愈.2例肾病复发患儿在腹膜炎治愈后尿蛋白转阴.结论 SBP在NS患儿表现不典型,甚至缺少腹膜激惹征,容易漏诊导致病情突然恶化,需要临床医师予以重视.SBP亦会导致NS复发,腹膜炎的治愈有助于原发病的缓解.%Objective To explore the clinical feature of spontaneous bacterial peritonitis (SBP) in children with nephrotic syndrome (NS).Methods Eleven cases of SBP in children with NS from Department of Nephrology,Beijing Children's Hospital Affiliated to Capital University of Medical Science between January 2010 and June 2014 were analyzed retrospectively.The clinical features,laboratory data and efficacy of therapy were reviewed.Results In this study,12 episodes of primary peritonitis were detected in 11 patients.One patient had 2 attacks

  15. Association between angiotensin-converting enzyme 2 gene polymorphisms and childhood primary nephrotic syndrome%ACE2基因多态性与儿童原发性肾病综合征的相关性研究

    Institute of Scientific and Technical Information of China (English)

    邱明瑜; 谢琴芳; 王丽娜; 于力

    2015-01-01

    ObjectiveAngiotensin-converting enzyme 2 (ACE2) gene polymorphisms have been shown to be implicated in hypertension, diabetic nephropathy, and other diseases. However, it remains unclear whether ACE2 gene polymorphisms are involved in the development of primary nephrotic syndrome (PNS) in children. The aim of this study was to assess the association between A9570G polymorphisms of ACE2 gene and PNS in a group of Han children in Guangdong Province, China.MethodsThe genotype distribution and allele frequency of ACE2 gene A9570G in 66 children with PNS and 60 healthy subjects (control group) were analyzed by polymerase chain reaction and restriction fragment length polymorphism.ResultsAllele frequency and genotype distribution showed no signiifcant difference between the PNS and control groups whether in female or in male children (P>0.05). The PNS group was classiifed into the glucocorticoid-sensitive and glucocorticoid-resistant subgroups according to glucocorticoid treatment response. Subgroup analysis revealed that in female children, the frequency of GG genotype was 17% in the glucocorticoid-sensitive group vs 45% in the glucocorticoid-sensitive group (P=0.018); the frequency of G allele was 31% in the glucocorticoid-sensitive group vs 61% in the glucocorticoid-resistant group (P=0.023). In male children, the frequency of G genotype/G allele was 36% in the glucocorticoid-sensitive group vs 64% in the glucocorticoid-resistant group (P=0.017).ConclusionsThere is no clear association between ACE2 gene A9570G polymorphisms and childhood PNS, but ACE2 gene A9570G polymorphisms might be associated with glucocorticoid treatment response in children with PNS. The G allele might be a genetic susceptibility factor of glucocorticoid resistance in children with PNS.%目的:血管紧张素转换酶2(ACE2)基因多态性与高血压病、糖尿病肾病等多种疾病相关,是否参与儿童原发性肾病综合征(PNS)的发病尚不明确,该研

  16. Peculiaridades da terapia trombolítica na síndrome nefrótica pediátrica: monitorização do fator anti-Xa Particularities of thrombolytic therapy in pediatric nephrotic syndrome: anti-factor Xa monitoring

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    Patrícia Zambi Meirelles

    2008-06-01

    Full Text Available OBJETIVO: Descrever a importância do tromboembolismo pulmonar (TEP na síndrome nefrótica pediátrica e o uso da heparina de baixo peso molecular como opção terapêutica segura e eficaz. DESCRIÇÃO DO CASO: Menino de 5,7 anos com síndrome nefrótica córtico-resistente e glomérulo-esclerose segmentar e focal foi internado devido à diarréia, distúrbios eletrolíticos e anasarca. No 11º dia de internação, evoluiu com desconforto respiratório súbito, cuja investigação mostrou área de alta probabilidade de TEP na cintilografia pulmonar ventilação/perfusão e obstrução em veia jugular interna esquerda ao ultra-som doppler. Iniciado suporte ventilatório com nebulização de oxigênio e anticoagulação com enoxaparina (2mg/kg/dia. Após seis dias, evoluiu com sintomas neurológicos compatíveis com episódio isquêmico transitório, sem alteração na tomografia computadorizada de crânio. A monitorização do fator anti-Xa no soro demonstrou nível subterapêutico e a dose de enoxaparina foi ajustada para 3mg/kg/dia. O edema e os sintomas pulmonares melhoraram e o paciente recebeu alta hospitalar após 33 dias. COMENTÁRIOS: Embora o TEP seja raro em crianças, a síndrome nefrótica é uma condição pró-trombótica que favorece a complicação. A heparina de baixo peso molecular pode ser considerada no tratamento e na profilaxia secundária do TEP, sendo importante monitorizar o nível sérico do fator anti-Xa para ajustar sua dose e promover tratamento seguro e eficaz.OBJECTIVE: Report the importance of pulmonary thromboembolism (PTE in pediatric nephrotic syndrome and the use of low molecular weight heparin (LMWH as an effective and secure therapeutic option. CASE DESCRIPTION: A 5.7 year-old boy with steroid-resistant nephrotic syndrome and focal segmental glomerulosclerosis was admitted to the pediatric unit with diarrhea, electrolyte disturbances and anasarca. On the 11th day of hospital stay, he developed a sudden

  17. 自我效能对肾病综合征患者饮食自我管理水平的影响%Study on the effect of self-efficacy on the level of dietary self-management of patients with nephrotic syndrome

    Institute of Scientific and Technical Information of China (English)

    林剑珊; 黄燕林

    2010-01-01

    Objective To study the effect of self-efficacy on the level of dietary self-management of patients with nephrotic syndrome. Methods Data collected from 128 patients with nephrotic syndrome measured by Self-Management Scale and General Self-Efficacy Scale. 65 patients in the experimental group were given self-efficacy training, and the other 63 patients in the control group were given clinical general nursing. After 6months, data were collected again. Results Before intervention, two groups had no significant difference both in self-management and self-efficacy( P > 0.05 ). After intervention, two groups had significant differences both in self-management and self-efficacy ( P < 0.05 ). There was correlation between the scores of self-efficacy and the level of dietary self-management. Conclusions The higher the scores of self-efficacy, the better the level of dietary self-management the patients with nephrotic syndrome got. The self-efficacy training had more advantages in improving the patients'dietary self-management level than clinical general nursing.%目的 探讨自我效能对肾病综合征患者饮食自我管理水平的影响.方法 将128例肾病综合征患者分为实验组(n=65)和对照组(n=63),实验组给予自我效能训练,对照组给予常规指导,干预前后采用肾病综合征患者自我管理行为问卷和一般自我效能感量表对f两组患者进行调查评估.结果 干预前两组患者在饮食自我管理、自我效能的得分情况方面的比较均无统计学意义(P>0.05);干预后实验组患者在饮食自我管理水平方面高于对照组,差异统计学意义(P<0.05);干预后两组患者在自我效能得分情况方面的比较差异有统计学意义(P<0.05);患者自我效能得分值与因子饮食自我管理得分值呈正相关.结论 肾病综合征患者自我效能越高,其饮食自我管理水平就越高.自我效能训练在提高肾病综合征患者饮食自我管理水平方面比传统的常规指导更具有优势.

  18. A prospective, randomized, controlled clinical study of Huai Qi Huang granules in treatment of childhood primary nephrotic syndrome%槐杞黄颗粒治疗儿童原发性肾病综合征的前瞻性随机对照临床研究

    Institute of Scientific and Technical Information of China (English)

    耿海云; 李建国; 李华荣; 杜培玮; 曹力; 王薇; 陈大坤; 陈朝英; 初梅; 季丽娜; 王京晶; 涂娟

    2015-01-01

    Objective To study the efifcacy of Huai Qi Huang granules in the treatment of childhood primary nephrotic syndrome. Methods Between July 2009 and December 2011, patients who were admitted and diagnosed for the ifrst time as childhood primary nephrotic syndrome were randomized into a treatment group (Huai Qi Huang granules plus glucocorticoid;n=23) and a control group (glucocorticoid alone;n=19) for a prospective study. The two groups were compared for regression time of edema, time to urinary protein clearance, relapse rate, incidence of infection, dosage of glucocorticoid, and humoral and cellular immunological indicators. Results There were no signiifcant differences in regression time of edema, time to urinary protein clearance, and relapse rate between the treatment and control groups (P>0.05). The treatment group had signiifcantly lower incidence of infection and daily dose of glucocorticoid (at month 6) than the control group (P0.05). No Huai Qi Huang-related adverse events were observed in this study. Conclusions Huai Qi Huang granules treatment can reduce the dose of glucocorticoid and the incidence of infection in children with primary nephrotic syndrome and has a favourable safety.%目的:观察槐杞黄颗粒对儿童原发性肾病综合征的治疗作用。方法采用前瞻性研究法,2009年7月至2011年12月住院并初诊为原发性肾病综合征的患儿随机分为糖皮质激素联用槐杞黄颗粒治疗组(观察组,n=23)和单用糖皮质激素治疗组(对照组,n=19),比较两组患儿浮肿消退时间、尿蛋白阴转时间、感染发生率、复发率、糖皮质激素用量及细胞、体液免疫功能。结果观察组与对照组浮肿消退时间、尿蛋白阴转时间、复发率差异无统计学意义(P>0.05)。观察组感染发生率低于对照组(P0.05)。未观察到槐杞黄相关不良反应的发生。结论槐杞黄可减少肾病综合征患儿糖皮质激素用量及感染合并症的

  19. Impact of group psychology guidance combined with conductive education on social anxiety of children with primary nephrotic syndrome%团体心理辅导联合引导式教育对原发性肾病综合征患儿社交焦虑的影响

    Institute of Scientific and Technical Information of China (English)

    王爱虹; 邹春杰; 张井兰; 姚丽; 黄慧桃

    2016-01-01

    目的:探讨团体心理辅导联合引导式教育对原发性肾病综合征( PNS)患儿的影响。方法选取临床确诊为PNS的60例患儿,按入院时间前后分为研究组和对照组,每组各30例,两组均给予泼尼松治疗,对照组仅给予常规健康教育,研究组给予团体心理辅导联合引导式教育,采用儿童社交焦虑量表于激素治疗前(T1)、诱导缓解结束时(T2)、泼尼松1 mg/(kg.d)隔日时(T3)、泼尼松0.5 mg/(kg.d)隔日时( T4)评估患儿社交焦虑水平,比较两组各时间点的社交焦虑水平。结果两组患儿4个时点社交焦虑水平比较差异有统计学意义( P<0.01);两组T2、T3、T4社交焦虑水平均高于同组T1焦虑水平,差异有统计学意义(P<0.05);研究组T1、T2、T3、T4的社交焦虑水平均低于对照组,差异有统计学意义(P<0.05)。结论 PNS患儿长期应用糖皮质激素治疗可导致社交焦虑水平升高,团体心理辅导联合引导式教育可有效缓解其焦虑情绪。%Objective To discuss the impact of group psychology guidance combined with conductive education on social anxiety of children with primary nephrotic syndrome. Methods A total of 60 children with primary nephrotic syndrome were selected, and divided into research group ( 30 cases ) and control group ( 30 cases ) according to the sequence of admission. The patients of two groups were given treatment of metacortandracin. The patients of control group received routine health education while the patients of research group underwent conductive education who was used social anxiety scale for children to evaluate the children anxiety level on the time of before treatment ( T1 ) , induced remission ( T2 ) , alternate-day-treated metacortandracin 1 mg/(kg.d)(T3), alternate-day-treated metacortandracin 0.5 mg/(kg.d)(T4), and the anxiety level of two groups were compared at different time point.Results There were significant differences on anxiety level of 4

  20. Electrocardiographic Changes in Mitral Valve Prolapse Syndrome

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    Mohamad Mehdi Peighambari

    2014-03-01

    Full Text Available Background- Mitral valve prolapse syndrome (MVP is most common valvular abnormality in young and is correlated with increased frequency of cardiac dysrhythmias and sudden death. The aim of this study was to compare frequency of "early repolarization" in electrocardiogram (ECG between MVP patients and healthy adults. Methods- In this cross-sectional study, we compared ECG presentations of early repolarization including notch in descending arm of QRS and J-point and/or ST segment changes in 100 patients with MVP with 100 healthy individuals. MVP patients were referred to cardiology clinic with symptoms of palpitation, chest pain or anxiety. Results-The mean age in patients with MVP was significantly less than healthy subjects (29.5 ± 9.3 years versus 31.0 ± 6.9 years in control group, p=0.1967. We detected an early repolarization as a prevalent sign in ECG of patients, which was a notch in descending arm of QRS and/or ST segment or J-point elevation seen in 74% of patients ( 51% in inferior leads and 23% in I and aVL leads , whilst the same findings was seen in 8 men (8% in control group (p=0.0001. Conclusion- Early repolarization in ECG presented as a notch in descending arm of QRS and/or ST segment or J-point elevation is more frequent in in young patients with MVP syndrome.

  1. 评价中医益气固摄法治疗原发性肾病综合征的疗效与安全性%Evaluation on the Efficacy and Safety of Traditional Chinese Medicine in the Treatment of Primary Nephrotic Syndrome

    Institute of Scientific and Technical Information of China (English)

    刘蕊

    2015-01-01

    ObjectiveTo study the clinical treatment effect of primary nephrotic syndrome with the method of Supplementing Qi and nourishing qi in the treatment.Methods 68 patients with primary renal disease treated in our hospital from January 2014 to January 2015, who were divided into control group and observation group. The control group was treated by using the method of Supplementing Qi and nourishing therapy. The clinical efficacy of the two groups were compared.Results The total effective rate was 91.18% in the observation group and 70.41% in the control group(P<0.05),while the patients in the observation group were significantly different from the control group(24 h). 3 cases in the observation group and 8 cases in the control group. Conclusion The treatment of patients with primary nephrotic syndrome with traditional Chinese medicine therapy can improve the clinical effect.%目的:研究分析中医益气固摄法在治疗原发性肾病综合征的临床疗效。方法选择我院2014年1月~2015年1月收治的原发性肾病68例,将其按照治疗方法分为对照组和观察组,对照组患者给予西药治疗,观察组在对照组基础上应用益气固摄法进行治疗,比较两组患者的临床疗效。结果观察组总有效率为91.18%,对照组为70.41%,比较差异有统计学意义(P<0.05);同时观察组患者经治疗后血白蛋白、24 h尿蛋白定量改善情况均优于对照组,比较差异有统计学意义(P<0.05);观察组与对照组中分别有3例、8例出现不良反应。结论给予原发性肾病综合征患者应用中医益气固摄法进行治疗,可提升临床疗效。

  2. Síndrome nefrótica primária grave em crianças: descrição clínica e dos padrões histológicos renais de seis casos Severe primary nephrotic syndrome in children: description of clinical aspects and of the renal histological patterns of six cases

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    Márcia Camegaçava Riyuzo

    2006-10-01

    Full Text Available Os autores relatam os casos de seis crianças com síndrome nefrótica primária grave de padrão histológico renal incomum na rotina cotidiana dos nefrologistas e patologistas. O diagnóstico da doença foi realizado nas faixas etárias de 3 a 9 meses de idade (n = 4, aos 2 anos e 4 meses (n = 1 e aos 11 anos (n = 1. Um paciente foi prematuro, duas pacientes eram irmãs e seus pais eram primos de primeiro grau. Todos apresentavam edema generalizado; dois pacientes apresentavam desnutrição e hipotireoidismo e dois apresentavam hipertensão arterial e insuficiência renal. A histologia renal mostrou esclerose mesangial difusa (n = 3, proliferação mesangial (n = 2 e síndrome nefrótica do tipo finlandês (n = 1. Quatro pacientes faleceram, as causas de óbito foram infecção (n = 2, insuficiência renal (n = 1 e acidose metabólica (n = 1. Entre os sobreviventes, um paciente foi tratado com vitaminas, tiroxina, captopril e indometacina, apresentando aumento da albumina sérica e melhora do crescimento. O outro paciente apresentava insuficiência renal terminal, sendo tratado com diálise e transplante renal.The authors report six children with severe primary nephrotic syndrome with unusual renal histological patterns in the daily routine of nephrologists and pathologists. The diagnosis of the disease was made at the age between 3 to 9 months (n = 4, at 2 years and 4 months (n = 1 and at 11 years (n = 1. One patient was born prematurely; two patients were sisters and their parents were first-degree cousins. All patients presented generalized edema, two patients presented malnutrition and hypothyroidism; two patients presented hypertension and renal failure. The renal histology showed diffuse mesangial sclerosis (n = 3; diffuse mesangial hypercellularity (n = 2 and nephrotic syndrome of the Finnish type (n = 1. Four patients died, causes of death were infection (n = 2, renal failure (n = 1 and metabolic acidosis (n = 1. Among the survivors

  3. Observation on the clinical effects of cyclosporine A combined with glucocorticoids in children with primary nephrotic syndrome from a single center%环孢素A联合激素治疗儿童原发性肾病综合征的单中心临床疗效观察

    Institute of Scientific and Technical Information of China (English)

    宁文慧; 韩子明; 韩玫瑰

    2016-01-01

    ObjectiveTo observe the clinical effects and safe of cyclosporine A (CsA) in the treatment of primary nephrotic syndrome in children.MethodsThere were 40 child patients with nephrotic syndrome treated with CsA (3-5 mg²kg-1²d-1) combined with glucocorticoids, in which including steroid-resistant nephritic syndrome (SRNS) 13 cases, steroid-dependent nephrotic syndrome (SDNS) 18 cases, frequent-relapses nephrotic syndrome (FRNS) 9 cases. The concentration of CsA maintained 100-200μg/L. Total course of treatment was two years, the dose was tapered gradually in 9-12 months after onset, the remission rate of child patients, 24 h urine protein, plasma cholesterol, CD4+, CD8+, the ratio of CD4+/CD8+, and the occurrence of adverse drug reactions were observed closely.Results24 cases (60%) were completely remission, 7 cases (17.5%) were partially remission, whose short-term complete remission rate gradually increased along with the duration of treatment. 9 cases (22.5%) were ineffective who were given to adjust other immunosuppressants to further treat after the 3 months follow-up. After the 6, 9, 12 months follow-up, the 31 cases’ clinical biochemical parameters were significantly improved, serum albumin increased, 24 h urine protein, serum cholesterol, CD4+ and the ratio of CD4+/CD8+ decreased, there was significant difference after the treatment of CsA and before (P0.05). There was no significant difference in the efficacy of different pathological types. The main adverse effects of CsA included hirsutism, gingival hyperplasia, gastrointestinal reactions, mild hypertension, liver and renal function impairment, meanwhile, one case of reversible encephalopathy syndrome was in back of the brain, and all were acceptable.ConclusionIn small samples of clinical study, the application of CsA combined with hormone to treat children with NS were relatively safe and effective, a better efficacy for SDNS, adverse reactions should be paid attention to.%目的:观察环孢素 A

  4. Serum level of 25-hydroxyvitamin D in children with nephrotic syndrome and determinants analysis%肾病综合征患儿血清25-羟维生素D的影响因素分析

    Institute of Scientific and Technical Information of China (English)

    梁慧开; 周建华; 仇丽茹; 程黎明; 鲍春; 李辉军

    2014-01-01

    [25(OH)D]in children with nephrotic syndrome (NS)and relevant factors. Methods Clinical data of NS paitients hospitalized at Tongji Hospital affiliated to Tongji Medical College of Huazhong University of Science & Technology from January 2012 to December 2012 were retrospectively collected,and healthy children coming for physical examination were enrolled as normal control group. The serum levels of 25( OH )D were defined as deficiency(≤20 ng · mL-1 ). Univariate analysis and multivariable logistic regression analysis were performed to explore the possible influencing factors related to vitamin D deficiency. Meanwhile,multi-regression analysis was used to investigate the potential factors affecting serum 25( OH)D level. Results A total of 222 subjects were enrolled,including 139 patients with NS and 83 healthy children. The serum 25(OH)D level of NS group was siginificantly higher than control group,(10. 3 ± 7. 5)vs (29. 42 ± 9. 85)ng·mL-1 ,P<0. 000 1. The prevalence rates of vitamin D deficiency of NS and control groups were 86. 3% and 19. 3%(P<0. 000 1). The multivariate logistic regression showed that patients with higher weight Z-score(0. 151,95%CI:0. 035-0. 649),longer duation of NS(0. 143,95%CI:0. 035-0. 592),greater 24 h protein amount(28. 177,95%CI:3. 295-240. 970)were significantly assocaited with vitamin D deficiency. Multiple linear regression determined that serum 25( OH)D levels were associated with male,24 h urinary protein amount,cumulative glucocorticoid dosage and serum calcium level( P<0. 05). Serum levels of 25(OH)D were significantly increased after treatment with alfacalcidol for 3 months,(7. 2 ± 5. 0)to (14. 9 ± 11. 0)ng·mL-1 ,P=0. 001. Conclusion Vitamin D deficiency was significantly associated with urine protein amount. Alfacalcidol supplements may improve vitamin D deficiency in nephritic patients.

  5. 同时接种b型流行性感冒嗜血杆菌结合疫苗和流行性乙型脑炎减毒活疫苗偶合肾病综合征1例报告%1 Cases of Nephrotic Syndrome Caused by B Type Haemophilus Influenzae Vaccine and Japanese Encephalitis Vaccine at the Same Time

    Institute of Scientific and Technical Information of China (English)

    李彩霞

    2015-01-01

    报告1例同时接种b型流行性感冒嗜血杆菌结合疫苗和流行性乙型脑炎减毒活疫苗偶合肾病综合征患者,判断其是否是异常接种反应。通过本例提示预防接种人员在实施预防接种前,要详细询问接种对象的健康状况和观察其精神状态,严格把握接种禁忌证,以减少异常反应发生或减少偶合疾病,化解医患之间矛盾,确保免疫规划工作正常开展。%Report one patient who inoculated B type of influenza Haemophilus Conjugate Vaccine and Japanese encephalitis at enuated live vaccine coupled with nephrotic syndrome,to determine whether it is abnornal vaccination reaction.Vaccination staf s should ask the vaccinated people about health in detail and look into their mental condtion and control strictly vaccination contraindication to reduce abnormal reactions or diseases and resolve conficts between doctors and patients to ensure immunazation programs proceeded wel .

  6. Variation of GH-IGF Axis in Children with the Refractory Nephrotic Syndrome%难治性肾病综合征患儿生长激素-胰岛素样 生长因子轴的变化及意义

    Institute of Scientific and Technical Information of China (English)

    蒋小云; 莫樱; 陈述枚; 朱志红; 赖峰; 朱春浓

    2001-01-01

    Objective To observe the variation of GH-IGF axis in children with the refractory nephrotic syndrome (RNS). Methods Serum and urine levels of IGF-I and IGFBP-3 and baseline serum levels of GH were assayed using RIA and IRMA in 26 patients with RNS, and hight standard deviation score (HtSDS) was calculated. Eighteen healthy children of similar ages were used as the control group (NC group). Results Serum IGF-I [(152.68±120.95) ng/ml] and IGFBP-3 [(2 183.33±1 711.33) ng/ml] levels in the RNS group were significantly lower than those of the NC group [(255.68±46.92) ng/ml, 4 333.87±1 122.00) ng/ml] (P0.05。RNS组身高标准差积分(HtSDS)(-0.42±0.75)低于NC组(0.30±0.17),(P<0.05)。结论 RNS患儿存在GH-IGF轴的变化,此变化为RNS患儿生长障碍的主要原因之一。

  7. Progressive anticonvulsant hypersensitivity syndrome associated with change of drug product

    DEFF Research Database (Denmark)

    Sabroe, T.P.; Sabers, A.

    2008-01-01

    This report describes the laboratory and physical manifestations of lamotrigine-like toxicity in a young man with refractory epilepsy receiving lamotrigine presenting as anticonvulsant hypersensitivity syndrome (AHS) associated with an abrupt change of drug product Udgivelsesdato: 2008/6...

  8. Propofol Infusion Syndrome Heralded by ECG Changes

    NARCIS (Netherlands)

    Mijzen, Elsbeth J.; Jacobs, Bram; Aslan, Adnan; Rodgers, Michael G. G.

    2012-01-01

    Propofol infusion syndrome (PRIS) is well known, often associated with, lethal complication of sedation with propofol. PRIS seems to be associated with young age, traumatic brain injury (TBI), higher cumulative doses of propofol, and the concomitant use of catecholamines. Known manifestations of PRI

  9. 糖皮质激素对原发性肾病综合征患儿社交焦虑与攻击行为的影响%Effects on Social Anxiety and Aggressive Behavior of Glucocorticoids in Children with Primary Nephrotic Syndrome

    Institute of Scientific and Technical Information of China (English)

    蒋新苗; 杨松萍

    2014-01-01

    Objective To assess the characteristics of social anxiety and aggressive behavior in receiving hormone therapy children with nephrotic syndrome(NS).Methods By convenience sampling,38 cases of children with NS were selected and their social anxiety and aggressive behavior were assessed by using Children’s Social Anxiety Questionnaire and Buss-Perry Aggression Questionnaire were applied to assess social anxiety levels and aggressive behavior before treatment(T1),the end of remission phase(T2),the day after using 1 mg/kg/d prednisone(T3)and the day after using 0.5 mg/kg/d prednisone(T4).Results Social anxiety score and aggressive behavior score were significantly different between the four measures (all P<0.05),and T2,T3,T4 scores were higher than the baseline of T1 score.Linear correlation analysis showed that social anxiety score and aggressive behavior score were positively correlated with the dose of prednisone(all P<0.05).Conclusion Levels of social anxiety and aggressive behavior in NS children trea-ted with glucocorticoids for a long time were enhanced,which requires nurses and parents to raise aware-ness and care in order to promote a better physical and psychological rehabilitation of children.%评估肾病综合征(nephrotic syndrome,NS)患儿在应用糖皮质激素后的社交焦虑和攻击性行为特点.方法采用便利抽样法选取2011年6月至2012年6月在东阳市人民医院治疗的 NS 患儿38例,应用儿童社交焦虑问卷、Buss-Perry攻击问卷于患儿激素治疗前(T1)、诱导缓解结束时(T2)、泼尼松1 mg/(kg��d)隔日时(T3)、泼尼松0.5 mg/(kg��d)隔日时(T4)进行社交焦虑水平和攻击性行为评估.结果4个时点的社交焦虑评分、攻击性行为评分差异具有统计学意义(均P<0.05),其中T2、T3、T4时点的评分均高于基线T1的评分.直线相关分析表明,社交焦虑评分、攻击性行为评分与泼尼松剂量呈正相关(均P<0.05).结论 NS患儿在长期应

  10. Preliminary observation of low-dose cyclosporine A combined with low-dose prednisone in treatment of patients with primary nephrotic syndrome%小剂量环孢素A联合小剂量激素治疗原发性肾病综合征疗效观察

    Institute of Scientific and Technical Information of China (English)

    李忠心; 陈向东; 李新

    2013-01-01

    Objective To evaluate the efficacy and safety of combining low-dose cyclosporine A(CsA) and prednisone in induction therapy for patients with primary nephrotic syndrome (PNS),and their comparison with furl amount of prednisone.Methods 67 cases of PNS were confirmed by renal biopsy examination and prospective nonrandomized analyzed for more than 18 months.Treatment group:low-dose CsA combined low-dose prednisone,41 cases,including 8 cases of refractory nephrotic syndrome.Patients were treated with CsA 2.5 mg · kg-1 · d-1 combined with prednisone 0.5 mg · kg-1 · d-1 (maximum dose of prednisone 30 mg/d).After 2 to 3 months,dose of prednisone and CsA turn down 10% every 2 to 4 weeks or continuous application of the original dose.Control group:enough prednisone,26 patients,patients were treated with prednisone 1.0 mg · kg-1 · d-1 (maximum dose of prednisone 60 mg/d).After 2 to 3 months,dose of prednisone turn down 10% every 2 to 4 weeks.10 cases combined with cyclophosphamide(CTX) treatment(CTX 0.8 g/month in 500 ml normal saline intravenous or oral 0.1 g/d,CTX total 6.0-8.0 g).Clinical parameters (MTP,ALB,ALT,CR,UA,GLU,TC and LDL) and adverse effects were estimated before and after therapy.Results MTP and ALB levels were significantly improved in both groups (P <0.01) after treatment.The total effective rates were 80.5% and 84.6% after 18 months(P >0.05).The creatinine in treatment group tended to increase after 12 months of treatment (P < 0.01),but returned to normal after 18 months.5 cases (17.9%) recurrened in 28 cured cases after stopping CsA up for 3 years.Some adverse effects (gingival hyperplasia,hypertension,increase of serum uric acid increased) occurred during CsA therapy,but could be easily controlled.Conclusions Low-dose CsA combined low-dose prednisone is adequate efficient for treatment of primary nephrotic syndrome compared to that with the enough prednisone.At the same time reducing the side effects of CsA and corticosteroids which

  11. Clinical and pathological characteristics and the expression of interleukin-6, interleukin-17 and tumor necrosis factor-α in elderly patients with primary nephrotic syndrome%老年原发性肾病综合征患者临床病理特征及血清中炎性因子的表达

    Institute of Scientific and Technical Information of China (English)

    黄静蓉

    2015-01-01

    目的 探讨原发性肾病综合征老年患者的临床病理特征及血清中白细胞介素-6(IL-6)、白细胞介素-17(IL-17)和肿瘤坏死因子-α(TNF-α)的表达. 方法 选择原发性肾病综合征的老年患者75例为观察组,另选择无明显器质性疾病的老年人40例为对照组,抽取空腹静脉血检测血清中IL-6、IL-17和TNF-α的表达. 结果 75例患者中,有病理资料者67例,其中膜性肾小球肾炎46例,局灶节段性病变5例,系膜毛细血管性肾小球肾炎5例,系膜增生性肾小球肾炎和IgA肾病各3例,肾小球轻微及微小病变、硬化性肾小球肾炎、弥漫性增生性肾小球肾炎、节段增生性肾小球肾炎、轻微肾小球病变各1例.观察组和对照组IL-6、IL-17和TNF-α的表达差异有统计学意义;观察组不同病理特征患者中,IL-6和IL-17的表达差异无统计学意义,TNF-α表达在膜性肾小球肾炎患者中明显高于其他病理类型的患者. 结论 原发性肾病综合征老年患者的病理类型主要是膜性肾小球肾炎;老年肾病综合征患者血清中IL-6、IL-17和TNF-α的表达明显升高,TNF-α在膜性肾小球肾炎中的表达升高更为明显.%Objective To investigate clinical and pathological characteristics and the expression of interleukin (IL)-6, IL-17 and tumor necrosis factor (TNF)-α in elderly patients with primary nephrotic syndrome.Methods A total of 75 elderly cases with primary nephrotic syndrome were chosen as the observation group, and 40 healthy elderly persons served as the control group.Fasting serum IL-6, IL-17 and TNF-α levels were measured by enzyme-linked immunosorbent assay (ELISA) for both groups.Results 67 of the 75 patients received pathological diagnoses, of whom 46 had membranous glomerulonephritis, 5 had focal segmental glomerulosclerosis, 5 had mesangiocapillary glomerulonephritis, 3 had mesangial proliferative glomerulonephritis, 3 had IgA nephropathy, and the rest included one case for each

  12. 骨髓间充质干细胞对肾病综合征大鼠CD4+CD25+调节性T细胞的影响%Effects of bone marrow mesenchymal stem cell transplantation on CD4+CD25+regulatory T cells in rats with primary nephrotic syndrome

    Institute of Scientific and Technical Information of China (English)

    杨焕丹; 张锐锋; 封东进; 朱冰冰; 吕娟

    2014-01-01

    BACKGROUND:Decreased function and reduced number of CD4+CD25+regulatory T cells have been considered the major manifestation of immunity dysfunction in children with primary nephrotic syndrome. Bone marrow mesenchymal stem cells have immunoregulation effects, which up-regulate CD4+CD25+regulatory T cells, inhibit proliferation of lymphocytes, and have been widely used in many immune diseases. OBJECTIVE:To investigate the effects of bone marrow mesenchymal stem celltransplantation on the CD4+CD25+regulatory T cells of peripheral blood in rats with primary nephrotic syndrome. METHODS:Bone marrow mesenchymal stem cells from six Sprague-Dawley rats were isolated, passaged and utilized for cellsuspension preparation. At the third passage, bone marrow mesenchymal stem cells were used for transplantation. The remaining 30 rats were randomly and equal y divided into three groups:normal group, normal saline infusion group, and bone marrow mesenchymal stem cells group. The rat models of primary nephrotic syndrome were established by single injection of adriamycin intravenously through tail vein in the latter two groups. Rats were then treated with bone marrow mesenchymal stem cells (1×10 7 ) (bone marrow mesenchymal stem cells group) or normal saline (normal saline infusion group) through tail vein at the same time after adriamycin administration. The normal group received no treatment. RESULTS AND CONCLUSION:Compared with the normal group, rats in the normal saline infusion group developed nephropathy characterized by ascites, proteinuria, hypoalbuminemia, hypercholastero-lnemia, and progressive renal injury. However, the proteinurine and clinical severity in bone marrow mesenchymal stem cells group were significantly ameliorated after treatment with bone marrow mesenchymal stem cells. CD4+CD25+Treg/CD4+Treg in the peripheral blood in the bone marrow mesenchymal stem cells group and normal saline infusion group were significantly higher than that in the normal group at 28

  13. Urinary Peptide Profiling to Differentiate between Minimal Change Disease and Focal Segmental Glomerulosclerosis

    OpenAIRE

    Pérez, Vanessa; Ibernón, Meritxell; López, Dolores; Pastor, María Cruz; Navarro, Maruja; Navarro-Muñoz, Maribel; Bonet, Josep; Romero, Ramón

    2014-01-01

    Background Minimal change disease (MCD) and primary focal segmental glomerulosclerosis (FSGS) are the main causes of primary idiopathic nephrotic syndrome in children and adults, with diagnosis being essential for the appropriate choice of therapy and requiring renal biopsy. However, the presence of only normal glomeruli on renal biopsy of FSGS patients may lead to the misclassification of these patients as having MCD. The aim of this study was to (i) compare the peptide profile of MCD and FS...

  14. 强的松治疗对肾病综合征儿童血清IGF-Ⅰ及IGFBPs的影响%Effect of glucocorticoid treatment on insulin like growth factor-Ⅰ and its binding proteins in children with nephrotic syndrome

    Institute of Scientific and Technical Information of China (English)

    董峰; 周湘; 庞宁; 魏珉

    2002-01-01

    目的了解儿童肾病综合征(NS)及糖皮质激素(GC)治疗对血清胰岛素样生长因子-Ⅰ(IGF-Ⅰ)及其结合蛋白(IGFBPs)的影响.方法采用免疫放射法对36名NS患儿,即未治疗的活动期组(ANS,12例),强的松治疗中活动期组(GNS,12例)和缓解期组(RE,12例),进行了血清IGF-Ⅰ及其IGFBPs水平测定,并以同年龄组正常儿童作对照(NC,10例).结果 (1)与正常对照比较,NS活动期血清IGF-Ⅰ和IGFBP-3明显降低(P<0.01),而IGFBP-1和IGFBP-2均明显升高(P<0.01);(2)NS缓解期血清IGF-Ⅰ和IGFBP-3明显高于活动期 (P<0.01),而IGFBP-1和IGFBP-2明显低于活动期(P<0.01);(3)经激素治疗的活动期组血清IGF-Ⅰ和IGFBP-3均明显高于未治疗活动期组(P<0.01),IGFBP-1和IGFBP-2水平也明显低于ANS组(P<0.01);(4)NS活动期血清IGFBP-3与ALB成正相关(r=0.76,P<0.01),血清IGF-Ⅰ与IGFBP-3成正相关, 而与IGFBP-1、IGFBP-2成负相关.在缓解期及激素治疗中的活动期血清IGF-Ⅰ及IGFBPs与ALB及CHO没有相关关系.结论 1)儿童肾病综合征活动期血清IGF-Ⅰ和IGFBPs发生明显改变,这种改变在缓解期可恢复正常.2)糖皮质激素治疗对肾病综合征儿童血清IGF-Ⅰ和IGFBPs有明显影响.3)肾病综合征儿童生长障碍可能与血清IGF-Ⅰ和IGFBPs变化有关.%Objective To identify the changes in serum insulin like growth factor-Ⅰ (IGF-Ⅰ) and IGF binding proteins (IGFBPs) in children with nephrotic syndrome (NS) and the effect of glucocorticoid on serum IGF-Ⅰ and IGFBPs. Methods We measured serum IGF-Ⅰ and IGFBPs levels by radioimmune assay and immune radiomagnetic assay in 36 children with NS, consisting of an active stage group (ANS, n=12), a remission stage group (RE, n=12), an active stage group with glucocorticoid treatment (GNS, n=12), and a normal control group (NC, n=10). Results 1) Compared to NC, serum levels of IGF-Ⅰ and IGFBP-3 were decreased (P<0.01); serum levels of IGFBP-1 and IGFBP-2 were increased (P<0.01) in

  15. Cardiovascular Changes in Animal Models of Metabolic Syndrome

    Directory of Open Access Journals (Sweden)

    Alexandre M. Lehnen

    2013-01-01

    Full Text Available Metabolic syndrome has been defined as a group of risk factors that directly contribute to the development of cardiovascular disease and/or type 2 diabetes. Insulin resistance seems to have a fundamental role in the genesis of this syndrome. Over the past years to the present day, basic and translational research has used small animal models to explore the pathophysiology of metabolic syndrome and to develop novel therapies that might slow the progression of this prevalent condition. In this paper we discuss the animal models used for the study of metabolic syndrome, with particular focus on cardiovascular changes, since they are the main cause of death associated with the condition in humans.

  16. Lymphocyte Subpopulation, Interleukin-2 and High Affinity Interleukin-2 Receptor Expression in Primary Nephrotic Syndrome%原发性肾病综合征患者外周血淋巴细胞亚群、白介素2及其高亲和力受体表达的研究

    Institute of Scientific and Technical Information of China (English)

    付平; 许国章; 黄颂敏; 余庭龙

    2001-01-01

    目的 探讨原发性肾病综合征(PNS)患者发病期及缓解期的细胞免疫状态。方法 分别采用单克隆抗体致敏红细胞检测法、白介素2(IL-2)生物活性检测法和受体的放射性配体结合法,对39例PNS患者及25例正常对照的外周血淋巴细胞亚群、IL-2活性及高亲和力白介素2受体(IL-2R)的表达进行检测。结果 PNS组22例发病期患者的T淋巴细胞(CD3)、辅助淋巴细胞(CD4)、抑制淋巴细胞(CD8)以及IL-2、IL-2R均显著低于对照组(P<0.05);17例缓解期患者的CD3、CD4、IL-2、IL-2R高于发病期组,但低于对照组,且与后两组的上述各项指标均有显著性差异(P<0.05)。结论 PNS患者发病期细胞免疫功能低下,缓解期有所恢复,但仍未达正常水平。缓解期细胞免疫功能低下可能是PNS易于复发的原因之一。%Objective To understand the cellular immune response duringrelapse and remission stage in primary nephrotic syndrome(PNS). Methods We applied the radioligand binding assay (RBA),the bioactivity measurement of IL-2,and monoclonal antibody sensitized red blood cell method to evaluate the expression of IL-2R, the production of IL-2 and T lymphocyte subsets of PBMC from 22 patients suffering from PNS, 17 patients with PNS in remission, and 25 normal subjects matched age and sex. Results CD3,CD4,CD8,IL-2,IL-2R in relapse were significantly lower than those in control (P<0.05); all above except CD8 in remission patients were significantly higher than those in relapse, but lower than those in control (P<0.05).Conclusion This study suggested that the cell mediated immunity (CMI) during acute nephrotic phase decreased; the deficiency of CMI of PNS in remission improved a lot when compared with the acute phase, but still not recovered completely; the deficiency of CMI with PNS in remission might be one of the causes of easily recurring of PNS.

  17. Conformational Transitions and Glycation of Serum Albumin in Patients with Minimal-Change Glomerulopathy

    Science.gov (United States)

    Hong, Sae Yong; Lee, Eun Young; Yang, Jong Oh; Kim, Tae Yeong; Kim, Eun Hee; Cheong, Mi Young; Kim, Soo Hyun; Cheong, Chae Joon

    2004-01-01

    Background There has been a lack of study on the structural changes of serum albumin in patients with minimal change disease (MCD). To determine whether glycation and/or conformational transitions of albumin are involved in the pathogenesis of albuminuria, nine patients with MCD were enrolled in a prospective follow-up study for comparison of these parameters in serum albumin during the remission and relapse of nephrotic syndrome. Methods Circular dichroism measurements were made with purified albumin. Ellipticities at each wavelength were transformed to mean residue ellipticity. Monosaccharide composition was analyzed by high-pH anion-exchange chromatography with pulsed amperometric detection. Results There was no difference in the proportions of α-helix, β-conformation, and β-turn of albumin between the sera of control patients and those with nephrotic syndrome. However, the proportion of the random configuration was slightly higher in the plasma albumin of patients in relapse than in those in remission. The proportion of the random configuration was lower in the albumin of the serum than in the urine of patients with nephrotic syndrome, but there was no difference in the proportions of α-helix, β-conformation, and β-turn of albumin between their plasma and urine. Conclusion Our results suggest that conformational changes in albumin are involved in albuminuria in patients with MCD. PMID:15481604

  18. Changing face of irritable bowel syndrome

    Institute of Scientific and Technical Information of China (English)

    Eamonn MM Quigley

    2006-01-01

    Recent years have witnessed tremendous progress in our understanding of irritable bowel syndrome (IBS). It is evident that this is a truly global disease associated with significant symptoms and impairments in personal and social functioning for afflicted individuals. Advances in our understanding of gut flora-mucosal interactions, the enteric nervous system and the brain-gut axis have led to substantial progress in the pathogenesis of symptoms in IBS and have provided some hints towards the basic etiology of this disorder, in some subpopulations, at the very least. We look forward to a time when therapy will be addressed to pathophysiology and perhaps, even to primary etiology. In the meantime, a model based on a primary role for intestinal inflammation serves to integrate the various strands, which contribute to the presentation of IBS

  19. Expression of L-FABP in serum, urine and renal tissues in the patients with primary nephrotic syndrome%原发性肾病综合征患者血清、尿肝型脂肪酸结合蛋白的变化及其在肾组织表达的意义

    Institute of Scientific and Technical Information of China (English)

    施婧; 尹忠诚; 唐敏; 杨晶; 张颖; 李胜开

    2014-01-01

    Objective By detecting liver-type fatty acid binding protein (L-FABP) levels in serum, urine and L-FABP expression in renal tissues in patients with primary nephrotic syndrome ( PNS), to investigate its relationship with pathological type and PNS with acute kidney injury (AKI).Methods 45 patients with PNS were divided into 2 groups according to whether they had acute tubular necrosis ( ATN) .There were 37 cases of PNS without ATN and AKI ( PNS without AKI group ) .According to the pathological types , they were further divided into mesangial proliferative glomerolonephritis (MsPGN) group (13 cases), minimal change disease (MCD) group (5 cases), focal segmental glo-merulosclerosis (FSGS) group (9 cases), and membranous nephropathy (MN) group (10 cases).There were 8 cases of PNS with ATN and AKI (PNS with AKI group ) , in which all cases were MCD .Serum and urine of 10 healthy subject who received routine physical checkup and 10 normal renal tissues located far from renal tumor in patients with nephritic tumor served as control groups .The levels of L-FABP in serum and urine were detected by enzyme -linked immunosor-bent assay ( ELISA) .Immunohistochemical staining was used to detect the expression of L -FABP in renal tissues .Re-sults ①The levels of L-FABP in serum and urine and expression of L -FABP in renal tissues in patients with PNS were significantly higher than those of the control group (P0.05).②The serum and urine levels of L-FABP and the expression of L -FABP in renal tissures were enhanced in the PNS with AKI group compared with the PNS without AKI group (P<0.05).③The receiver operator characteristic curve′s area under the curve (ROC-AUC) of serum, urine L-FABP and serum creatinine (SCr) for diagnosis of AKI were 0.910, 0.973, 0.812, respectively.④The serum and urine levels of L -FABP were positively correlated with SCr , blood urea nitrogen (BUN), 24 h urine pro-tein (24hUpro) and expression of L -FABP in renal tissures (r=0.331~0.764, P<0

  20. Long-term effects of cyclosporine A in children with steroid-resistant idiopathic nephrotic syndrome and outcomes of the patients%长疗程环孢素A治疗儿童激素耐药型肾病综合征的疗效和预后

    Institute of Scientific and Technical Information of China (English)

    曹琦; 黄文彦; 徐虹; 周利军

    2008-01-01

    Objective To observe the effects of long-term cyclosparine A(CsA)treatment in 20 children with steroid-resistant idiopathic nephrotic syndrome(SRNS)and analyse the relevant influencing factom of CsA therapy.Methods Twenty children with SRNS received CsA therapy for 2 years between February 2001 and October 2006 in the Department of Nephmlogy.The mean age of children at initiation of CsA therapy was 5.5 years(30 months to 12 years).The initial renal histology showed minimal change (MCNS)in 15 patients,focal segmental glomerulosclerosis(FSGS)in 4 patients and mesangial proliferative with a trough level of 40-70 μg/L wfs applied to mainrain remission for 1 year.Liver function,serum albumin,serum cholesterol,serum creatinine,urinary NAG/Cr,24 h urinary protein excretion and CsA whole blood trough level of the patients were monitoted every one or three months.Results (1)Complete remission(proteinuria≤0.1 g/d or negative by dipstick for 3 consecutive days),partial remission of 100μg/L)were observed in 65%,20% and 15%,respectively.Eleven patients who had complete remission discontinued CsA,in 5(45%)patients the disease relapsed,and resumption of CsA thempy was foilowed by remission in th.ree of them.(2)MCNS showed a 93%response to CsA therapy while non-MCNS showed a 60% response,but the difference was not significant(P>0.05).(3)Hypertrichosis,gingival hyperplasia and hypertension occurred in 75%,25%and 10%of the patients,respectively.Two patients were found to have renal impairment(>30% rise of serum creatinine)and recovered in 2 weeks. Four patients showed a rise of urinary NAG/Cr.The central nervous system adverse event occurred in 2 cages.Post-thempy biopsies performed in 3 patients(2 with FSGS and one with MCNS)did not show any relevant tubulointerstitial fibrosis.Two patients with FSGs ofthe twenty cases progressed into end-stage renal failure.Conclusions CsA treatment was confirmed to be effective in children with SRNS.Rehal fibrosis was rare in patients

  1. Frequency and clinicopathological correlations of histopathological variants of idiopathic focal segmental glomerulosclerosis in nephrotic adolescents

    International Nuclear Information System (INIS)

    Objective: To determine the frequency and clinicopathological correlations of focal segmental glomerulosclerosis variants in adolescents with idiopathic nephrotic syndrome. Methods: All consecutive adolescents (12 to 18 years) who presented with idiopathic nephrotic syndrome in the period, January 2009 to December 2012, and in whom the histological diagnosis of focal segmental glomerulosclerosis was made on renal biopsies, were included in this prospective study. Their clinical, laboratory and histopathological features at the time of presentation or biopsy were noted from the case files and the biopsy reports. Results: Among 50 adolescents, 34 (68%) were males and 16 (32%) females. The mean age was 15.14+-2.3 years. The mean duration of disease was 6.3+-11.2 months. The mean serum creatinine was 0.96+-0.82 mg/dl. The mean 24-hour urinary protein excretion was 3.8+-0.68 grams. Biopsy indications were steroid-resistant nephritic syndrome in 15 (30%), steroid-dependant nephritic syndrome in 19 (38%) and adolescent nephritic syndrome in 16 (32%) cases. Among the focal segmental glomerulosclerosis variants, 40 (80%) were not otherwise specified, followed by the collapsing variant, which accounted for 8 (16%) cases. The tip and cellular variants, both were found in one (2%) case each. Among the histological features, global glomerulosclerosis was found in 23 (46%) cases, and segmental scarring/collapse in all (100%). A variable degree of tubular atrophy and interstitial fibrosis was noted in 44 (88%) cases. Conclusion: The results from this study indicate that the pattern of focal segmental glomerulosclerosis variants differs markedly in adolescents compared with younger children. (author)

  2. 原发性肾病综合征患儿外周血GRα、GRβ的表达及临床意义%Expression of GRα, GRβ in peripheral blood mononuclear cells in patients with primary nephrotic syndrome and its clinical significance

    Institute of Scientific and Technical Information of China (English)

    邱玲; 封其华; 赵惠君

    2012-01-01

    目的 探讨糖皮质激素受体α(GRα)和β(GRβ)在原发性肾病综合征(PNS)中的作用及其介导耐药的可能机制.方法 选择15例糖皮质激素(GC)敏感型PNS(SSNS)患儿和15例GC耐药型PNS(SRNS)患儿,以及10例健康对照儿童,应用逆转录-聚合酶链式反应(RT-PCR)检测各组外周血单个核细胞(PBMC)中GRα和GRβ mRNA的表达,并分析其与24h尿蛋白定量(24 hUTP)及肾脏病理积分的关系.结果 PNS患儿PBMC中GRα和GRβ均有表达,并以GRα为主;三组间GRα mRNA的表达无差异(P>0.05);而SRNS组GRβ mRNA、GRα/GRβ水平均高于SSNS和对照组(P均<0.05).GRβ mRNA的表达与24 hUTP和病理积分呈正相关(P<0.05),即GRβ mRNA的表达越高,病理积分越高,病理损伤越大.结论 GRβ mRNA可以作为监测PNS患儿病情以及预后的指标.针对SRNS患儿PBMC中GRβ升高,增加激素受体敏感性,有望为SRNS的治疗提供新的思路.%Objective To investigate the expression of glucocorticoid receptor (GR)α, GFβ in peripheral blood mononuclear cells in patients with primary nephrotic syndrome and the possible mechanism of GRα and GRβ mediated the steroid resistance. Methods Fifteen children with glucoeorticoid-sensitive primary nephritic syndrome (SSNS group) , 15 children with glucocortieoid-resistant primary nephritic syndrome (SRNS group) , and 10 healthy children (control group) were recruited. The expression of GRα and GRβ Mrna in peripheral blood mononuclear cells (PMBC) was detected by reverse transcription polymerase chain reaction. The relationship of the expression of GRα and GRβ with 24 h urinary protein and renal pathological score were analyzed. Results Both GRα and GRβ, mainly GRα, were expressed in PMBC in children with primary nephritic syndrome. The expression of GRα Mrna were not significant different among SRNS, SSNS and control groups (P > 0.05). The expression of GRp Mrna and GRα/GRβ Mrna was significantly higher in SRNS group than that

  3. Relationship between Angiotensin-Converting Enzyme Insertion/Deletion Gene Polymorphism and Susceptibility of Minimal Change Nephrotic Syndrome: A Meta-Analysis

    Directory of Open Access Journals (Sweden)

    Tian-Biao Zhou

    2011-01-01

    Africans: D: =.81, DD: =.49. Furthermore, the II genotype seemed not to play a protective role against MCNS risk for Asians, Caucasians and Africans (=.12, =.09, =.76, resp.. Interestingly, there was also significant association between ACE I/D gene polymorphism and MCNS susceptibility in overall populations (D: =.007, DD: =.04, II: =.03. Conclusion. D allele or DD genotype might be a significant genetic molecular marker for MCNS susceptibility in Asians and overall populations, but not for Caucasians and Africans. More larger and rigorous genetic epidemiological investigations are required to further explore this association.

  4. Failure Analysis of Nephrotic Syndrome Pattern Identification by TCM for 23 cases%原发性肾病综合征23例中医辨证失误分析

    Institute of Scientific and Technical Information of China (English)

    邓立武; 欧阳龙

    2010-01-01

    @@ 原发性肾病综合征(Primordial Nephritic Syndrome,PNS)常简称"肾综",中西医结合治疗本病,获得了较好的临床疗效.临床中,常常遇见因中医辨证失误,导致疗效欠佳的情况.兹将我们2003年3月至2008年12月间资料完整的23例病人,就中医辨证失误分析如下.

  5. Erlotinib preserves renal function and prevents salt retention in doxorubicin treated nephrotic rats.

    Directory of Open Access Journals (Sweden)

    Raed N Bou Matar

    Full Text Available Nephrotic syndrome is associated with up-regulation of the heparin-binding epidermal growth factor (HB-EGF. Erlotinib blocks the activation of the epidermal growth factor receptor (EGFR in response to HB-EGF. This study investigates the effect of Erlotinib on the progression of proteinuria, renal dysfunction, and salt retention in doxorubicin treated nephrotic rats. Male rats were divided into 3 pair-fed groups (n = 13/group as follows: Control rats (Ctrl; rats receiving intravenous doxorubicin (Dox; and rats receiving intravenous doxorubicin followed by daily oral Erlotinib (Dox + Erl. Upon establishment of high grade proteinuria, urine sodium and creatinine clearance were measured. Kidney tissue was dissected and analyzed for γ-epithelial sodium channel (γENaC, sodium-potassium -chloride co-transporter 2 (NKCC2, sodium chloride co-transporter (NCC, aquaporin 2 (AQP2, and EGFR abundances using western blot. Creatinine clearance was preserved in the Dox + Erl rats as compared to the Dox group (in ml/min: Ctrl: 5.2±.5, Dox: 1.9±0.3, Dox + Erl: 3.6±0.5. Despite a minimal effect on the degree of proteinuria, Erlotinib prevented salt retention (Urinary Na in mEq/d: Ctrl: 2.2±0.2, Dox: 1.8±0.3, Dox + Erl: 2.2±0.2. The cleaved/uncleaved γENaC ratio was increased by 41±16% in the Dox group but unchanged in the Dox + Erl group when compared to Ctrl. The phosphorylated EGFR/total EGFR ratio was reduced by 74±7% in the Dox group and by 77±4% in the Dox + Erl group. In conclusion, Erlotinib preserved renal function and prevented salt retention in nephrotic rats. The observed effects do not appear to be mediated by direct blockade of EGFR.

  6. Focal Segmental Glomerular Sclerosis Type Nephrotic Syndrome to Line the Kidney in the Therapeutic Effect of huoxue Soup%局灶性节段性肾小球硬化型肾病综合征行自拟肾综活血汤治疗的疗效

    Institute of Scientific and Technical Information of China (English)

    海江

    2015-01-01

    Objective:To explore the renal head huoxue decoction in the treatment of focal segmental glomerular sclerosis type ne-phrotic syndrome ( FSGSNS) clinical curative effect.Methods:Randomly selected data in June 2011 -June 2014 in our hospital treated 90 cases of patients with FSGSNS, were randomly divided into research group and the control group, 45 cases in each group.The control group was conventional symptomatic treatment, the team on the basis of upward renal head huoxue soup treatment, analysis of two groups of therapeutic effect.Results:the treatment of 6 months, 1 year team 24 hualb, BUN, SCr and PLT level are lower than the control group, GFR, IgG, Propagated, Hb level were higher than control group (P <0.05).Conclusoi n:the patients with renal heald huoxue soup can effectively improve FSGSNS renal function, control level of proteinuria, improve their quality of life.%目的:探究肾综活血汤治疗局灶性节段性肾小球硬化型肾病综合征( FSGSNS)的临床疗效。方法:资料随机选取2011年6月~2014年6月本院收治的90例FSGSNS患者,随机分为研究组与对照组,每组各45例。对照组行常规对症治疗,研究组在此基础上行肾综活血汤治疗,分析2组治疗效果。结果:治疗后半年、1年研究组24小时UAlb、BUN、SCr、PLT水平均低于对照组,GFR、IgG、ALB、Hb水平均高于对照组(P<0.05)。结果:肾综活血汤可有效改善FSGSNS患者肾功能,控制蛋白尿水平,提高其生活质量。

  7. Integrative medicine " three wide" mode effects on patients with nephrotic syndrome disease the extent and effects of anxiety and depression intervention%中西医结合“三全”干预对肾病综合征患者疾病影响程度和焦虑抑郁干预效应

    Institute of Scientific and Technical Information of China (English)

    付百灵

    2014-01-01

    Objective: To observe the effect ofimplementation of " intervention effect Sanquan"intervention on patients of nephrotic syndrome andadverse emotional state. Methods: 200 subjects, randomly divided into observation group and control group with 100 cases in each group, group used the nursing programcontrol to give a systematic holistic nursing care,observation group with integrated traditional Chinese and Western medicine " three all" mode that " all human intervention, and mental intervention and Quan Chengji disease intervention" mode of intervention. Two groups of patients before intervention (the first day of admission) andintervention (after 1 years) scale, self rating Anxiety Scale( SAS) and the disease affects the self rating Depression Scale (SDS) [into the group and post implementationevaluation. The 2 evaluation results were treated statistically (t test)]. Results:the two groups of patients with different mode Before intervention the influence degree of anxiety,depression, disease evaluation scores had no significant difference between the effects of the disease, the impact of different patterns of physical function after intervention(SD-Ⅰ), social and psychological influence (SD-Ⅱ), the 3 dimensions of evaluation score and the anxiety and depression, emotional state, the observation group were lower than those in the control group decreased, compared with the control group and the observation group before and after compared, all have very significant difference(P<0.01) and very significant difference (P < 0. 001). Conclusion: combining traditional Chinese and Western medicine " three" of the intervention model can effectively alleviate the patients with nephrotic syndrome of the emotional state, reduce the disease effect, promote the rehabilitation.%目的:观察实施“三全”干预模式对肾病综合征患者疾病影响程度和不良情绪状态的干预效应。方法:研究对象200例,随机分为观察组和对照组各100例,

  8. 广西西部地区壮族难治性肾病综合征患儿肾素-血管紧张素系统基因多态性研究%Study on Gene Polymorphism of Renin Angiotensin System in Refractory Nephrotic Syndrome Children in Zhuang Population of West Guangxi Zhuang Autonomous Region

    Institute of Scientific and Technical Information of China (English)

    尤燕舞; 林栩; 王洁; 杨发奋

    2012-01-01

    目的 研究肾素-血管紧张素(RAS)系统3个关键基因血管紧张素Ⅱ1型受体(AT1R)基因A1166C多态性、血管紧张素Ⅰ转化酶(ACE)基因插入/缺失(I/D)多态性和血管紧张素原(AGT)基因M235T多态性在广西西部地区壮族原发性肾病综合征(PNS)患儿激素敏感组和难治组中的分布,探讨RAS基因多态性在儿童难治性肾病综合征(NS)中的作用.方法 选取原籍广西西部地区的壮族PNS患儿62例(肾病组),根据其对激素的治疗反应分为显效组42例(激素敏感型NS组)和难治组20例(难治性NS组),并选取50例健康儿童作为健康对照组.采用直接PCR和PCR-限制性片段长度多态性技术检测RAS ACE基因I/D多态性、AT1R基因A1166C多态性和AGT基因M235T多态性在各组中的分布,并进行统计学比较.结果 肾病组ACE基因I/D多态性与健康对照组比较差异有统计学意义,D等位基因在肾病组中占显著优势(P<0.05),AT1R基因A1166C、AGT基因M235T多态性在2组中分布的差异无统计学意义.难治性NS组与激素敏感型NS组比较,AT1R A1166C、ACE 1/D和AGT M235T基因型与等位基因分布频率差异均无统计学意义.结论 ACE I/D多态性的D等位基因是广西西部地区壮族PNS患儿易感因素之一,未能发现RAS基因多态性在广西西部地区壮族儿童难治性NS中的作用.%Objective To study the distributions of angiotensin D type 1 receptor( ATI R) gene A1166C polymorphism, angiotensin I converting enzyme( ACE) gene insertion/deletion( I/D) polymorphism and angiotensinogen( AGT) gene M235T polymorphism in Zhuang children of West Guangxi Zhuang Autonomous Region with primary nephrotic syndrome(PNS) .which included steroid sensitive group and refractory group. To explore the effect of renin angiotensin system ( RAS) gene polymorphism in children with refractory nephrotic syndrome (NS). Methods Sixty — two cases of West Guangxi Zhuang Autonomous Region with PNS( nephropathy group) were

  9. Screening of Effective Chemical Fractions of Rhizoma Atractylodis Macrocephalae and Its Optimal Proportion in Combination with Cortex Mori and Their Effects on Nephrotic Syndrome%白术各化学拆分组分及其配伍对大鼠肾病综合征的影响

    Institute of Scientific and Technical Information of China (English)

    郑晓珂; 于洋; 周静; 吴广操; 王小兰; 牛艳; 黄岩杰; 匡海学; 冯卫生

    2016-01-01

    compatibility proportion for the treatment of the nephrotic syndrome.MethodsAdriamycin-induced nephrotic syndrome rat model was adopted. With 24 h urine protein,and the levels of serum albumin(Alb),triglycerides(TG),total cholesterol(TCH),serum creatinine(Cre-S)and bloodurea nitrogen(BUN)as the evaluation indexes,we screened the optimal effective fractions and the best compatibility ratio ofBaizhu. ELISA was applied to detect the serum levels of interleukin 6(IL-6),tumor necrosis factor alpha (TNF-α),superoxide dismutase(SOD),malondialdehyde(MDA)for the evaluation of the effects of the optimal effective fractions. Renal histology of rats in effective fraction groups was observed after HE staining. The therapeutic mechanism of the effective fractions was evaluated by examining the urine volume,arginine vasopressin(AVP)and serum levels of aquaporin 2(AQP2)and aquaporin 1(AQP1).ResultsAfter medication for 3 weeks,levels of 24 h urine protein,Cre-S,BUN,TG,TCH,and Alb of the low dose group(2.8 g·kg-1)were improved significantly(P< 0.05),indicating that 2.8 g·kg-1 was the optimal dose. The levels of 24 h urine protein,Cre-S,BUN,TG,and TCH of the polysaccharide fraction group were improved significantly(P< 0.05),IL-6 and TNF-αwere decreased, SOD was increased,and renal injury was relieved,indicating that polysaccharide fraction was optimal effective fraction. The optimal compatibility proportion ofBaizhutoSangbaipiwas 1∶1,and the combination of the two herbs had better effect on increasing rat urine volume and on decreasing AVP and AQP2 than the single herb ofSangbaipi (P< 0.05).ConclusionThe optimal effective chemical fractions ofBaizhuis polysaccharide fraction,which has anti-oxidative and anti-inflammation actions and can relieve renal injury. The optimal compatibility ratio ofBaizhuto Sangbaipiis 1∶1,and their combination at the ratio of 1∶1 can decrease the level of AVP and enhance the diuretic action.

  10. EFFECT OF HIGH—LIPID DIET ON GLOMERULAR MESANGIAL MATRIX IN ADRIAMYCIN—INDUCED NEPHROTIC RATS

    Institute of Scientific and Technical Information of China (English)

    宋红梅; 李学旺; 等

    2002-01-01

    Objective:To determine the effect of hypercholesterolemia induced by a high-lipid diet on glomerulosclerosis.Methods:Twenty nephrotic syndrome (NS) Wistar rats administrated adriamycin(ADR) with a single intravenous dose of 5mg/kg body weight,were divided into the standard and high-lipid chow groups.Another 20 weight-matched non-NS rats that received a vehicle alone were grouped as control.Urinary protein excretion and serum cholesterol were assayed;image analysis and techniques of pathology,immunohistochemistry,and molecular biology were used to determine morphological changes in glomeruli and the production of glomerular mesangial matrices in different groups.Results:The total cholesterol level was significantly higher in rats with high-lipid chow in both non-NS[(2.2±0.3)g/L vs.(0.9±0.1)g/L,P<0.01] and NS[(9.5±0.2)g/L vs.(2.3±0.3)g/L,p<0.01].The urinary protein excretion was significantly higher in the high-lipid diet rats than in standard chow rats[(76.2±24.2)mg/24h vs.(44.8±13.6)mg/24h,P<0.05]in NS rats.Although increases in the mesangial matrix and mesangial cells were observed in rats with high-lipid diet in both NS and non-NS group,more obvious pathological changes were found in NS group,such as lipid deposits and foam cell formation in mesangial areas,and progressing to focal and segmental glomerulosclerosis in someglomeruli.Theimmunohistochemical assay showed that the production of 3 major components (collagen IV,fibronectin,and laminin)was increased in NS group,especially in the rats with high-lipid chow.The increased expression of laminin mRNA was also detected with slot blotting in bloth NS and non-NS rats with high-lipid chow,and it was more obvious in the rats with NS.Conclusion:Our findings indicated that diet-induced hyperlipidemia can lead to over-production of mesangial matrix components,and further aggravate glomerulosclerosis in ADR-induced nephrosis.

  11. EFFECT OF HIGH-LIPID DIET ON GLOMERULAR MESANGIAL MATRIX IN ADRIAMYCIN-INDUCED NEPHROTIC RATS

    Institute of Scientific and Technical Information of China (English)

    宋红梅; 李学旺; 魏珉; 朱传酉

    2002-01-01

    Objective. To determine the effect of hypercholesterolemia induced by a high-lipid diet on glomerulosclerosis. Methods. Twenty nephrotic syndrome (NS) Wistar rats administrated adriamycin (ADR) with a single intravenous dose of 5 mg/kg body weight, were divided into the standard and high-lipid chow groups. Another 20 weight-matched non-NS rats that received a vehicle alone were grouped as control. Urinary protein excretion and serum cholesterol were assayed; image analysis and techniques of pathology, immunohistochemistry, and molecular biology were used to determine morphological changes in glomeruli and the production of glomerular mesangial matrices in different groups. Results. The serum total cholesterol level was significantly higher in rats with high-lipid chow in both non-NS [(2.2 ± 0.3) g/L vs. (0.9 ± 0.1) g/L, P<0.01] and NS [(9.5± 0.2) g/L vs. (2.3 ± 0.3) g/L, P<0.01]. The urinary protein excretion was significantly higher in the high-lipid diet rats than in standard chow rats[(76.2± 24.2) mg/24 h vs. (44.8 ± 13.6) mg/24 h, P<0.05] in NS rats. Although increases in the mesangial matrix and mesangial cells were observed in rats with high-lipid diet in both NS and non-NS group, more obvious pathological changes were found in NS group, such as lipid deposits and foam cell formation in mesangial areas, and progressing to focal and segmental glomerulosclerosis in some glomeruli. The immunohistochemical assay showed that the production of 3 major components (collagen IV, fibronectin, and laminin) was increased in NS group, especially in the rats with high-lipid chow. The increased expression of laminin mRNA was also detected with slot blotting in both NS and non-NS rats with high-lipid chow, and it was more obvious in the rats with NS. Conclusion. Our findings indicated that diet-induced hyperlipidemia can lead to over-production of mesangial matrix components, and further aggravate glomerulosclerosis in ADR-induced nephrosis.

  12. A Unique Cause of Proteinuria in Pregnancy: Class II Lupus Nephritis with Concomitant Minimal Change Disease

    Science.gov (United States)

    Kunjal, Ryan; Adam-Eldien, Rabie; Makary, Raafat; Jo-Hoy, Francois; Heilig, Charles W.

    2016-01-01

    We report the case of a 22-year-old African American female who presented to another facility for routine follow-up in the 34th week of pregnancy with lower extremity swelling and nephrotic-range proteinuria. Although she was normotensive, it was initially thought that she had preeclampsia. She was monitored carefully and delivery was induced at 37 weeks of gestation. She was transferred to our hospital, where she was diagnosed with systemic lupus erythematosus (SLE) based on clinical and laboratory criteria. Renal biopsy revealed a surprising finding of minimal change disease (MCD) concomitant with class II lupus nephritis (LN). She was managed with pulses and then tapering doses of steroid therapy with dramatic resolution of the nephrotic syndrome. This case demonstrates not only the rare de novo occurrence of SLE in pregnancy, but the unique finding of MCD coexisting with class II LN. We propose that altered T cell activity may be the link between these seemingly distinct entities.

  13. Collapsing Glomerulopathy in a Child with Galloway-Mowat Syndrome.

    Science.gov (United States)

    Zeybek, Cengiz; Basbozkurt, Gokalp; Hamcan, Salih; Ozcan, Ayhan; Gul, Davut; Gok, Faysal

    2016-01-01

    Galloway-Mowat syndrome (GMS) is an autosomal recessive disorder with a poor prognosis that was first defined as a triad of central nervous system involvement, hiatal hernia, and nephrotic syndrome. However, this syndrome is now known to have a heterogeneous clinical presentation. The nephrotic syndrome is steroid resistant and is responsible for the outcome. The combination of collapsing glomerulopathy and GMS is very rare. A 26-month-old boy presented with steroid-resistant nephrotic syndrome associated with neurologic findings, including microcephaly, psychomotor retardation, and nystagmus. Magnetic resonance imaging showed marked cerebral atrophy, optic atrophy, and hypomyelination. A renal biopsy was consistent with collapsing glomerulopathy. If collapsing glomerulopathy is associated with neurological abnormalities, especially with microcephaly, clinicians should consider GMS as a possible underlying cause. PMID:27403357

  14. Collapsing Glomerulopathy in a Child with Galloway-Mowat Syndrome

    Science.gov (United States)

    Zeybek, Cengiz; Basbozkurt, Gokalp; Hamcan, Salih; Ozcan, Ayhan; Gul, Davut; Gok, Faysal

    2016-01-01

    Galloway-Mowat syndrome (GMS) is an autosomal recessive disorder with a poor prognosis that was first defined as a triad of central nervous system involvement, hiatal hernia, and nephrotic syndrome. However, this syndrome is now known to have a heterogeneous clinical presentation. The nephrotic syndrome is steroid resistant and is responsible for the outcome. The combination of collapsing glomerulopathy and GMS is very rare. A 26-month-old boy presented with steroid-resistant nephrotic syndrome associated with neurologic findings, including microcephaly, psychomotor retardation, and nystagmus. Magnetic resonance imaging showed marked cerebral atrophy, optic atrophy, and hypomyelination. A renal biopsy was consistent with collapsing glomerulopathy. If collapsing glomerulopathy is associated with neurological abnormalities, especially with microcephaly, clinicians should consider GMS as a possible underlying cause. PMID:27403357

  15. [Cardiovascular risk factors in children with primary nephrotic syndrome].

    Science.gov (United States)

    Alegría-Torres, Gabriela Alejandra; Aguilar-Kitsu, María Alejandra; Estrada-Loza, María Jesús; Villasís-Keever, Miguel Ángel

    2015-01-01

    Introducción: se ha propuesto que los pacientes con síndrome nefrótico (SN) tienen con mayor frecuencia factores de riesgo cardiovascular. El objetivo de este estudio fue determinar la frecuencia de factores de riesgo cardiovascular en niños con SN primario. Métodos: estudio transversal, descriptivo y prospectivo. Se incluyeron 55 pacientes con SN primario. Se evaluó la presencia de sobrepeso/obesidad, hipertensión, dislipidemia, hiperglicemia, elevación de proteína C reactiva (PCR) de alta sensibilidad y el incremento en el grosor intimo-medial carotídeo (GIM). Se analizó el tipo de SN, el tiempo de evolución, el tratamiento actual y el número de recaídas. Para el análisis estadístico se utilizó chi cuadrada y rho de Spearman. Resultados: el factor de riesgo cardiovascular más frecuente fue el incremento del GIM carotídeo (98.1 %), seguido de hipertrigliceridemia (54.4 %) y de hipercolesterolemia total (40 %). Los pacientes con SN corticosensible tuvieron menor número de factores de riesgo comparados con los corticorresistentes. Además hubo una correlación positiva de mayor tiempo de evolución y número de recaídas con el incremento de factores. Conclusiones: el 98 % de los niños con SN primario tuvieron al menos un factor de riesgo cardiovascular. Ciertas características clínicas como ser corticorresistentes y el tiempo de evolución y tratamiento parecen estar relacionadas con la presencia de un mayor número de factores de riesgo.

  16. Morphological changes of gastric mucosa depending on functional dyspepsia syndrome

    Directory of Open Access Journals (Sweden)

    Svintsitskyy A.

    2014-06-01

    Full Text Available Background. Chronic gastritis process of cell renewal in the mucosa is disturbed, leading to rapid movement of the generative cell zone without full differentiation into mature specialized area accommodation epithelial cells. The result of this process is the inability to fully function gastric glands. Crucial in the diagnosis of gastritis given the nature of the morphological changes of the gastric mucosa and preferential localization of these changes. Objective. To assess histological changes of gastric mucosa in patients with clinically different types of functional dyspepsia. Methods. Adult patients (18-65 years with confirmed diagnosis of functional dyspepsia were eligible to participate. Biopsy specimens were taken from stomach due to the Houston-updated gastric biopsy sampling protocol for the next histological examination. One expert gastrointestinal pathologist assessed all tissue samples. Atrophy was assessed due to Operative Link for Gastritis Assessment (OLGA staging system. Results. 75 patients were recruited, 42 of which had epigastric pain (I group and 33 – postprandial distress syndrome (II group due to Rome III criteria (2006. Antral and corpus atrophy were detected at the same frequency in both groups (p>0.05, however the stage of atrophy didn’t exceed I in all cases. Complete antral metaplasia was revealed in 11 (26.2% patients of the I group and 11 (33.3% patients of the II one. Incomplete antral metaplasia was seen in 2 (4.7% patients of the I group and 2 (6.1% patients of the II one. No cases of corpus metaplasia or dysplasia were found. Conclusion. Our study didn’t reveal statistically significant correlation between stage of gastritis, atrophic or metaplastic changes and clinical symptoms of functional dyspepsia. Citation: Svintsitskyy A, Korendovych I, Kuryk O, Solovyova G. [Morphological changes of gastric mucosa depending on functional dyspepsia syndrome]. Morphologia. 2014;8(2:50-5. Ukrainian.

  17. Exercise Induced Adipokine Changes and the Metabolic Syndrome

    Directory of Open Access Journals (Sweden)

    Saeid Golbidi

    2014-01-01

    Full Text Available The lack of adequate physical activity and obesity created a worldwide pandemic. Obesity is characterized by the deposition of adipose tissue in various parts of the body; it is now evident that adipose tissue also acts as an endocrine organ capable of secreting many cytokines that are though to be involved in the pathophysiology of obesity, insulin resistance, and metabolic syndrome. Adipokines, or adipose tissue-derived proteins, play a pivotal role in this scenario. Increased secretion of proinflammatory adipokines leads to a chronic inflammatory state that is accompanied by insulin resistance and glucose intolerance. Lifestyle change in terms of increased physical activity and exercise is the best nonpharmacological treatment for obesity since these can reduce insulin resistance, counteract the inflammatory state, and improve the lipid profile. There is growing evidence that exercise exerts its beneficial effects partly through alterations in the adipokine profile; that is, exercise increases secretion of anti-inflammatory adipokines and reduces proinflammatory cytokines. In this paper we briefly describe the pathophysiologic role of four important adipokines (adiponectin, leptin, TNF-α, and IL-6 in the metabolic syndrome and review some of the clinical trials that monitored these adipokines as a clinical outcome before and after exercise.

  18. A Framework for Modelling Connective Tissue Changes in VIIP Syndrome

    Science.gov (United States)

    Ethier, C. R.; Best, L.; Gleason, R.; Mulugeta, L.; Myers, J. G.; Nelson, E. S.; Samuels, B. C.

    2014-01-01

    Insertion of astronauts into microgravity induces a cascade of physiological adaptations, notably including a cephalad fluid shift. Longer-duration flights carry an increased risk of developing Visual Impairment and Intracranial Pressure (VIIP) syndrome, a spectrum of ophthalmic changes including posterior globe flattening, choroidal folds, distension of the optic nerve sheath, kinking of the optic nerve and potentially permanent degradation of visual function. The slow onset of changes in VIIP, their chronic nature, and the similarity of certain clinical features of VIIP to ophthalmic findings in patients with raised intracranial pressure strongly suggest that: (i) biomechanical factors play a role in VIIP, and (ii) connective tissue remodeling must be accounted for if we wish to understand the pathology of VIIP. Our goal is to elucidate the pathophysiology of VIIP and suggest countermeasures based on biomechanical modeling of ocular tissues, suitably informed by experimental data, and followed by validation and verification. We specifically seek to understand the quasi-homeostatic state that evolves over weeks to months in space, during which ocular tissue remodeling occurs. This effort is informed by three bodies of work: (i) modeling of cephalad fluid shifts; (ii) modeling of ophthalmic tissue biomechanics in glaucoma; and (iii) modeling of connective tissue changes in response to biomechanical loading.

  19. P选择素在肾病综合征并发深静脉血栓中的作用及犬血栓模型磁共振分子成像观察%Effect of P-selectin on deep vein thrombosis in nephrotic syndrome and molecular magnetic resonance imaging targeting P-selectin in a dog model of venous thrombosis

    Institute of Scientific and Technical Information of China (English)

    周同; 王鸿利; 李晓; 赵亚鹏; 金佩佩; 王学锋; 钟高仁; 汪登斌; 张明钧; 陈楠

    2008-01-01

    Objectives To detect the effects of P-selectin on deep venous thrombosis (DVT) in nephrotic syndrome (NS). and to evaluate the molecular magnetic resonance imaging (MRI) with a P-selectin targeted conlrost agent in diagnosis of thrombosis in the early phase. Methods (1) Forty-one patients with NS hospitalized in our department from 2005 to 2006 were enrolled in this study. They were assigned into DVT group and non-DVT group according to lower limbs radionuclide imaging (RNV) with 99mTc MAA. Blood P-selectin level was measured by ELISA method. (2) P-selectin was detected both in injured vein and blood immediately, 1 h and 3 h after the dog DVT model was established. (3) The P-selectin-targeted contrast agent was developed by conjugating anti-P-selectin lectin-EGF domain monoclonal antibody (PsL-EGFmAb) which was prepared by our lab. The potential of this contrast agent used in vitro molecular imaging experiment as well as in vivo experiment in dog DVT model was investigated. Results (1) Blood P-selectin level was elevated in patients with NS. It was much higher in DVT group than that in non-DVT group. (2) Blood P-selectin level was also elevated in DVT dogs and P-selectin expressed immediately in tunica intima of injured vein and subsequently in thrombus after the model established. (3) Mural thrombus showed higher signal visualization than surrounding muscle in 30 rain after contrast agent injection. These enhanced signals exhibited P-selectin specificity and persisted from the initiation of intima lesions to 3 h after development of thrombosis. There was signficant Differences in contrast-to-noise ratio (CNR) of the experiment group and the control group (11.50±2.32 vs 2.71±0.86, P<0.01). The same results were derived from 30 rain to 1 hafter contrast agent being injected in distal to heart part of the injured vessel, and the signal decreased 24 h later. Differences in CNR of the experiment group and the control group were also statistically significant (10

  20. 双重血浆置换联合他汀治疗肾病综合征严重高胆固醇血症的疗效%Treatment of severe hypercholesterolemia in resistant nephrotic syndrome: the effect of combined therapy using double filtration plasmapheresis and statins

    Institute of Scientific and Technical Information of China (English)

    龚德华; 徐斌; 张丽华; 朱冬冬; 季大玺; 刘志红

    2013-01-01

    by a case-controlled design, to investigate the effect of combined therapy using double filtration plasmapheresis (DFPP) and oral statins on severe hypercholesterolemia in resistant nephrotic syndrome (NS). Methodology; Twenty two patients with severe hypercholesterolemia caused by resistant NS were enrolled in this study. They were divided into three groups as follow: 2 of them in the group receiving a single session of DFPP alone ( DFPP group) , 8 in the group receiving a single session of DFPP combined with oral Atorvastatin (Combination group) , and 12 in the group receiving oral Atorvastatin alone (Statins group). Other treatments for primary disease and symptom-control remained unchanged. All patients were followed-up to 4 weeks. Results: The baseline levels of serum total cholesterol (TC) were (12. 7 ±1.40) mmol/L in DFPP group, (17. 1 ±4. 70) mmol/L in combination group,and (15. 4 ±5. 10) mmol/L in statins group ( P > 0. 05 ). Single session of DFPP decreased serum TC by (85. 8% ±7. 20% ) ( P < 0. 01) , accompanied with a decline of plasma immunoglobulin A (IgA) , M and fibrinogen (P < 0. 01) but not albumin or IgG. No remission was found in the all groups during 4 weeks follow-up. During the follow-up period, compared with baselinedata,serum TC was 85. 9% at the 1 week, 100% at the 2 week, and 134% at the end in DFPP group, serum TC was below 50% in the first 2 weeks,and 70. 2% at the end in combination group,and serum TC was around 81. 1% through the whole period in the statins group. The levels of serum TC were significantly lower than that in the other groups at the first two weeks ( P < 0. 01). Conclusion; Oral statin alone can only decline lipids slightly in patients with severe hypercholesterolemia due to resistant nephrosis, while combined therapy using DFPP plus oral statin can decrease lipids to a larger extent in these patients.%目的:观察双重血浆置换(DFPP)联合他汀类降脂药治疗肾病综合征(NS)严重高胆固醇血症的

  1. STUDY OF DEPRESSION AND RELEVANT FACTORS IN PATIENTS WITH NEPHROTIC SYN-DROME%肾病综合征患者抑郁状态及其相关因素分析

    Institute of Scientific and Technical Information of China (English)

    汤艳兰; 郑黎; 李玲

    2012-01-01

    [Objective] To investigate the depression status and relevant factors in patents with nephrotic syndrome. [Methods] The depression status of 180 patients with nephrotic syndrome were investigated through Zung self-rating depression scale. [Results] The total incidence rate of depression was 46.5% and relevant factors were degree of education, pecuniary condition, medical insurance, curative effect, course of disease and occurrence of complications. [Conclusion] Depression exists in most patients with nephrotic syndrome. Psychotherapy should be strengthened besides clinical treatment.%[目的]了解肾病综合征患者抑郁现况及影响因素.[方法]采用Zung自评抑郁量表(SDS)对180例肾病综合征患者进行调查.[结果]抑郁症患病率为46.5%.影响患者抑郁得分值的因素有文化程度、经济状况、医保情况、激素效果、治疗时间、复发情况、并发症.[结论]对肾病综合征患者应及时采取针对性的措施进行心理状况干预,以改善患者的心理状态,提高患者的生活质量.

  2. The sopite syndrome revisited: Drowsiness and mood changes during real or apparent motion

    Science.gov (United States)

    Lawson, B. D.; Mead, A. M.

    The sopite syndrome is a poorly understood response to motion. Drowsiness and mood changes are the primary characteristics of the syndrome. The sopite syndrome can exist in isolation from more apparent symptoms such as nausea, can last long; after nausea has subsided, and can debilitate some individuals. It is most likely a distinct syndrome from "regular" motion sickness or common fatigue, and is of potential concern in a variety of situations. The syndrome may be particularly hazardous in transportation settings where other performance challenges (e.g., sleep deprivation) are already present. It is also a potential concern in cases where illnesses such as sleep disorders or depression may interact with the syndrome and confuse diagnosis.

  3. Complete Remission of Minimal Change Disease Following an Improvement of Lung Mycobacterium avium Infection.

    Science.gov (United States)

    Yamashiro, Aoi; Uchida, Takahiro; Ito, Seigo; Oshima, Naoki; Oda, Takashi; Kumagai, Hiroo

    2016-01-01

    A 46-year-old woman suddenly developed peripheral edema. Her massive proteinuria, hypoproteinemia, and renal biopsy findings yielded the diagnosis of minimal change disease (MCD). In addition, lung Mycobacterium avium infection was diagnosed according to a positive culture of her bronchoalveolar lavage fluid. The lung lesion was improved by anti-nontuberculous mycobacteria therapy. Surprisingly, her proteinuria also gradually decreased and she attained complete remission of MCD without any immunosuppressive therapy. She has subsequently remained in complete remission. We herein report an interesting case of MCD with lung Mycobacterium avium infection, suggesting a causal relationship among infection, immune system abnormality, and MCD/nephrotic syndrome. PMID:27629965

  4. Metabolic and functional connectivity changes in mal de debarquement syndrome.

    Directory of Open Access Journals (Sweden)

    Yoon-Hee Cha

    Full Text Available BACKGROUND: Individuals with mal de debarquement syndrome (MdDS experience a chronic illusion of self-motion triggered by prolonged exposure to passive motion, such as from sea or air travel. The experience is one of rocking dizziness similar to when the individual was originally on the motion trigger such as a boat or airplane. MdDS represents a prolonged version of a normal phenomenon familiar to most individuals but which persists for months or years in others. It represents a natural example of the neuroplasticity of motion adaptation. However, the localization of where that motion adaptation occurs is unknown. Our goal was to localize metabolic and functional connectivity changes associated with persistent MdDS. METHODS: Twenty subjects with MdDS lasting a median duration of 17.5 months were compared to 20 normal controls with (18F FDG PET and resting state fMRI. Resting state metabolism and functional connectivity were calculated using age, grey matter volume, and mood and anxiety scores as nuisance covariates. RESULTS: MdDS subjects showed increased metabolism in the left entorhinal cortex and amygdala (z>3.3. Areas of relative hypometabolism included the left superior medial gyrus, left middle frontal gyrus, right amygdala, right insula, and clusters in the left superior, middle, and inferior temporal gyri. MdDS subjects showed increased connectivity between the entorhinal cortex/amygdala cluster and posterior visual and vestibular processing areas including middle temporal gyrus, motion sensitive area MT/V5, superior parietal lobule, and primary visual cortex, while showing decreased connectivity to multiple prefrontal areas. CONCLUSION: These data show an association between resting state metabolic activity and functional connectivity between the entorhinal cortex and amygdala in a human disorder of abnormal motion perception. We propose a model for how these biological substrates can allow a limited period of motion exposure to lead

  5. MRI of white matter changes in the Sjoegren-Larsson syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Hussain, M.Z. [Dept. of Radiology, Yamanashi Medical Coll., Yamanashi (Japan); Oba, H. [Dept. of Radiology, Yamanashi Medical Coll., Yamanashi (Japan); Ohtomo, K. [Dept. of Radiology, Yamanashi Medical Coll., Yamanashi (Japan); Aihara, M. [Dept. of Paediatrics, Yamanashi Medical Coll., Tamahocho, Yamanashi (Japan); Hayashibe, H. [Dept. of Paediatrics, Yamanashi Medical Coll., Tamahocho, Yamanashi (Japan); Nakazawa, S. [Dept. of Paediatrics, Yamanashi Medical Coll., Tamahocho, Yamanashi (Japan); Uchiyama, G. [Dept. of Radiology, Yamanashi Medical Coll., Yamanashi (Japan)

    1995-10-01

    We report a case of Sjoegren-Larsson syndrome with spastic diplegia and conduction aphasia. MRI demonstrated the white matter changes deep in the cerebral hemispheres. We analyse the MRI findings and compare the results with neuropsychological signs. (orig.)

  6. Changing the Perspective on Early Development of Rett Syndrome

    Science.gov (United States)

    Marschik, Peter B.; Kaufmann, Walter E.; Sigafoos, Jeff; Wolin, Thomas; Zhang, Dajie; Bartl-Pokorny, Katrin D.; Pini, Giorgio; Zappella, Michele; Tager-Flusberg, Helen; Einspieler, Christa; Johnston, Michael V.

    2013-01-01

    We delineated the achievement of early speech-language milestones in 15 young children with Rett syndrome ("MECP2" positive) in the first two years of life using retrospective video analysis. By contrast to the commonly accepted concept that these children are normal in the pre-regression period, we found markedly atypical development of…

  7. CLINICAL AND ENCEPHALOGRAPHIC CHANGES AT LENNOX–GASTAUT SYNDROME

    Directory of Open Access Journals (Sweden)

    K. Yu. Mukhin

    2015-01-01

    Full Text Available The Lennox–Gastaut syndrome (LGS is an epileptic encephalopathy, starting in childhood and showing in often polymorphic seizures (including tonic axial ones, severe cognitive deficite, slow activity of the acute–slow wave in the interictal period at the electroencephalography (EEG, runs of fast activity of 10–20 Hz, often associated with tonic seizures, as well as with the resistance to therapy. According ILAE Classification of epilepsy syndromes and epilepsies 1989 LGS was referred to generalized cryptogenic or symptomatic forms of the epilepsy. According to Proposed Diagnostic Scheme for People with Epileptic Seizures and with Epilepsy (2001 LGS is a classic representative of the group of childhood epileptic encephalopathies. LGS is a rather rare form of the epilepsy. The syndrome frequency makes from 1–4 to 6.6 % among all forms of the childhood epilepsy. LGS is subdivided into the cryptogenic and the symptomatic variants. From our point of view the latter it will be more correct to refer to the symptomatic focal epilepsy with the secondary bilateral synchrony phenomena at EEG. The LGS can be caused by cortical development defects, by perinatal encephalopathies, by brain tumors, by inherited metabolism diseases, by chromosomal anomalies, as well as by other factors. In case of the classic cryptogenic variant the ethiology of the LGS remains unknown. The disease onset is at the age of 2–8 y. o. In 20–40 % of cases LGS is transformed from the West syndrome. The LGS attribute is the polymorphism of seizures. The syndrome structure can combine tonic seizures, epileptic drop seizures, atypical absences, generalized tonic-clonic seizures. Focal seizures at LGS are a matter of argument. The article gives details on the clinical EEG criteria of LGS, the semiology of epileptic seizures in the syndrome structure, diagnostic and treatment approaches. The main accent is made on EEG peculiarities of the disease. The author presents the

  8. A case of clinical Reye syndrome presenting characteristic CT changes

    International Nuclear Information System (INIS)

    A 9-month-old male infant was admitted to our hospital on the second day of cold like syndrome because of high fever, convulsion, coma, and decerebrate rigidity. Serum GOT, GPT, LDH, and CPK were markedly elevated. Serum ammonia was slightly increased, and hypoglycemia was present. The cerebrospinal fluid showed no pleocytosis, normal sugar content, but increased protein. Thus we made a diagnosis of clinical Reye syndrome according to the criteria by Yamashita, et al. A CT on the day of admission showed symmetrical low-density areas in the posterior fossa and the regions of thalamus. Ringed enhancements were seen around the areas of low density in the thalamus on the twenty-second hospital day. We consider that these lesions may represent the infarction due to obstruction of the thalamoperforant arteries caused by cerebral edema in the early stage of the disease. (author)

  9. Serum leptin changes with metformin treatment in polycystic ovarian syndrome

    OpenAIRE

    Rehan, H. S.; Seth, Vikas; Upadhyaya, Prerna

    2011-01-01

    The relationship between leptin, insulin resistance and other hormonal parameters in polycystic ovarian syndrome (PCOS) is controversial. We investigated the effect of metformin on leptin levels in women with PCOS. Thirty women with PCOS received metformin 500 mg thrice a day. After two months of metformin treatment the mean leptin levels reduced significantly (p < 0.001). Ovulation was achieved in 28 patients, who also had a significant reduction in leptin levels (p < 0.001). Leptin showe...

  10. CNS changes in Usher's syndrome with mental disorder: CT, MRI and PET findings.

    OpenAIRE

    Koizumi, J; Ofuku, K; Sakuma, K.; Shiraishi, H.; Iio, M.; Nawano, S

    1988-01-01

    CNS changes in a case of Usher's syndrome associated with schizophrenia-like mental disorder were observed by CT, MRI and PET. The neuro-radiological findings of the case demonstrate the degenerative and metabolic alterations in various regions of cortex, white matter and subcortical areas in the brain. Mental disorder of the case is almost indistinguishable from that of schizophrenia, but the psychotic feature is regarded as an atypical or mixed organic brain syndrome according to the classi...

  11. Age-related changes in plasma levels of BDNF in Down syndrome patients

    OpenAIRE

    Licastro Federico; Galliera Emanuela; Dogliotti Giada; Corsi Massimiliano M

    2010-01-01

    Abstract Background The prevalence of coronary artery diseases is low among Down Syndrome (DS) patients and they rarely die of atherosclerotic complications. Histopathological investigations showed no increase in atherosclerosis, or even a total lack of atherosclerotic changes, in DS Aim The aim of our study is to investigate the relationship between age and brain-derived neurotrophic factor (BDNF) levels in Down Syndrome (DS). Subjects and methods Three groups of DS patients were studied: th...

  12. The relationship between irritable bowel syndrome and psychiatric disorders: from molecular changes to clinical manifestations

    OpenAIRE

    Fadgyas-Stanculete, Mihaela; Buga, Ana-Maria; Popa-Wagner, Aurel; Dumitrascu, Dan L

    2014-01-01

    Irritable bowel syndrome (IBS) is a functional syndrome characterized by chronic abdominal pain accompanied by altered bowel habits. Although generally considered a functional disorder, there is now substantial evidence that IBS is associated with a poor quality of life and significant negative impact on work and social domains. Neuroimaging studies documented changes in the prefrontal cortex, ventro-lateral and posterior parietal cortex and thalami, and implicate alteration of brain circuits...

  13. "It's All Changed:" Carers' Experiences of Caring for Adults Who Have Down's Syndrome and Dementia

    Science.gov (United States)

    McLaughlin, Katrina; Jones, Aled

    2011-01-01

    A qualitative interview study was undertaken to determine the information and support needs of carers of adults who have Down's syndrome and dementia. The data were analysed thematically. Carers' information and support needs were seen to change at pre-diagnosis, diagnosis and post-diagnosis. Helping carers to manage the changing nature of the…

  14. Developmental Changes in Cognitive and Behavioural Functioning of Adolescents with Fragile-X Syndrome

    Science.gov (United States)

    Frolli, A.; Piscopo, S.; Conson, M.

    2015-01-01

    Background: Individuals with fragile-X syndrome exhibit developmental delay, hyperexcitation and social anxiety; they also show lack of attention and hyperactivity. Few studies have investigated whether levels of functioning change with increasing age. Here, we explored developmental changes across adolescence in the cognitive and behavioural…

  15. Ischemic Changes in a Case of Unilateral Pseudoexfoliation Syndrome

    Directory of Open Access Journals (Sweden)

    Tolga Kocaturk

    2016-05-01

    Full Text Available A 66 year old man with normotensive unilateral pseudoexfoliation syndrome associated with ipsilateral marked ischemia with nerve fiber layer thinning and nasal step on successive visual field tests mimicking glaucomatous visual field loss is presented. Although the optic disc appearance of the clinically visible pseudoexfoliative left eye was not suggestive of glaucomatous cupping the disc appeared much pale and retinal vessels narrowed compared to the right eye. Color Doppler imaging of the left ophthalmic artery showed extremely high resistivity index of 0.88. The case is discussed in light of recent literature underscoring the fact that pseudoexfoliation is a cause of ocular ischemia.

  16. Physiological Changes and Clinical Implications of Syndromic Craniosynostosis

    Science.gov (United States)

    Matsusaka, Yasuhiro; Kunihiro, Noritsugu; Imai, Keisuke

    2016-01-01

    Syndromic craniosynostosis has severe cranial stenosis and deformity, combined with hypoplastic maxillary bone and other developmental skeletal lesions. Among these various lesions, upper air way obstruction by hypoplastic maxillary bone could be the first life-threatening condition after birth. Aggressive cranial vault expansion for severely deformed cranial vaults due to multiple synostoses is necessary even in infancy, to normalize the intracranial pressure. Fronto-orbital advancement (FOA) is recommended for patients with hypoplastic anterior part of cranium induced by bicoronal and/or metopic synostoses, and posterior cranial vault expansion is recommended for those with flattening of the posterior part of the cranium by lambdoid synostosis. Although sufficient spontaneous reshaping of the cranium can be expected by expansive cranioplasty, keeping the cranial bone flap expanded sufficiently is often difficult when the initial expansion is performed during infancy. So far distraction osteogenesis (DO) is the only method to make it possible and to provide low rates of re-expansion of the cranial vault. DO is quite beneficial for both FOA and posterior cranial vault expansion, compared with the conventional methods. Associated hydrocephalus and chronic tonsillar herniation due to lambdoid synostosis can be surgically treatable. Abnormal venous drainages from the intracranial space and air way obstruction should be always considered at any surgical procedures. Neurosurgeons have to know well about the managements not only of the deformed cranial vault and the associated brain lesions but also of other multiple skeletal lesions associated with syndromic craniosynostosis, to improve treatment outcome. PMID:27226850

  17. 环磷酰胺在难治性肾病综合征治疗中的应用——30年文献Meta分析%Cyclophosphamide therapy in refractory nephrotic syndrome: a meta-analysis of clinical trials from 1970 to 2000

    Institute of Scientific and Technical Information of China (English)

    马祖祥; 易著文; 吴小川; 党西强; 何小解; 何庆南

    2002-01-01

    目的明确环磷酰胺能否有效治疗难治性肾病综合征,以及不同的环磷酰胺应用方案中何种最佳,为临床用药以及进一步大规模、多中心的临床流行病学研究提供指导.方法通过MEDLINE、AHRQ、Cochrane、ACP Journal Club计算机检索系统,以nephrotic 条件的参考文献,以有效率(肾病综合征完全缓解率+部分缓解率)作为统计指标,采用加权百分率法进行Meta分析。 结果应用环磷酰胺(口服)与泼尼松联合 治疗肾病综合征的有效率为49.1%,单纯应用泼尼松治疗的有效率为16.2%,两者比较差 异 有显著意义(P<0.05);大剂量环磷酰胺(累积剂量为168~192 mg/kg)与泼尼松联合口 服治疗 的有效率为44%,小剂量环磷酰胺(累积剂量为42~168 mg/kg) 与泼尼松联合口服治疗 的 有效率为25%,两者比较差异有显著意义(P<0.05);应用环磷酰胺冲击治疗的有 效率为76 %,应用环磷酰胺口服治疗的有效率为59%,两者比较差异无显著意义(P>0.05 )。结论 环磷酰胺能有效治疗难治性肾病综合征,且以大剂量环磷酰胺和泼尼松同时口服治疗的效果最佳。

  18. [Episodic manifestation of hemiparkinson syndrome with severe dementia personality change and precursors of paranoid hallucination symptoms].

    Science.gov (United States)

    Postrach, F

    1989-09-01

    A case of episodic manifestation of semiparalysis agitans is described, accompanied by severe demential personality change and precursory hallucinatory symptoms, which is made the basis for the discussion of aspects of mental disorders, notably dementia and symptoms resembling schizophrenia, in Parkinsonian patients. By way of allusion to Glass, a diagnosis including a very extensive, complex, symptomatology is made of a Parkinsonian syndrome.

  19. Carpal arch and median nerve changes during radioulnar wrist compression in carpal tunnel syndrome patients.

    Science.gov (United States)

    Marquardt, Tamara L; Evans, Peter J; Seitz, William H; Li, Zong-Ming

    2016-07-01

    The purpose of this study was to investigate the morphological changes of the carpal arch and median nerve during the application of radiounlarly directed compressive force across the wrist in patients with carpal tunnel syndrome. Radioulnar compressive forces of 10 N and 20 N were applied at the distal level of the carpal tunnel in 10 female patients diagnosed with carpal tunnel syndrome. Immediately prior to force application and after 3 min of application, ultrasound images of the distal carpal tunnel were obtained. It was found that applying force across the wrist decreased the carpal arch width (p 1234-1240, 2016. PMID:26662276

  20. Transgenic isolation of skeletal muscle and kidney defects in laminin beta2 mutant mice: Implications for Pierson syndrome

    OpenAIRE

    Miner, Jeffrey H.; Go, Gloriosa; Cunningham, Jeanette; Patton, Bruce L.; Jarad, George

    2006-01-01

    Pierson syndrome is a recently defined disease usually lethal within the first postnatal months and caused by mutations in the gene encoding laminin β2 (LAMB2). The hallmarks of Pierson syndrome are congenital nephrotic syndrome accompanied by ocular abnormalities, including microcoria (small pupils), with muscular and neurological developmental defects also present. Lamb2−/− mice are a model for Pierson syndrome; they exhibit defects in the kidney glomerular barrier, in the development and o...

  1. Sexual desire changes during menstrual cycle and relationship with premenstrual syndrome

    OpenAIRE

    Kiani Asiabar A.; Heidari M; Mohammady Tabar SH.; Faghihzadeh S

    2007-01-01

    Background: Sexual function in women may be affected by their menstrual cycle. Lack of sexual drive is a deficiency or absence of sexual fantasies and desire for sexual activity. This study aims at determining the changes in sexual desire during the menstrual cycle and those associated with premenstrual syndrome (PMS) and evaluates sexual desire during the menstrual cycle and the associated changes with PMS. Methods: The sample for this cross-sectional study includes 150 women employed in fac...

  2. Equine Assisted Therapy and Changes in Gait for a Young Adult Female with Down Syndrome

    Directory of Open Access Journals (Sweden)

    Katherine J. Coffey

    2015-10-01

    Full Text Available The purpose of this study was to examine the effects of equine assisted therapy on selected gait parameters in a person with Down syndrome. One female participant with Down syndrome completed two therapeutic horseback riding programs, each consisting of six riding sessions. Specific gait characteristics were analyzed with a trend analysis of the data by examining the means of the different variables. The trend analysis revealed a difference in stride length as well as hip and knee angle. These results indicate that over the course of the two therapeutic horseback riding programs, changes in gait occurred. Therefore, therapeutic horseback riding may have the potential to benefit gait characteristics and stability in young adult females with Down syndrome; however, further research is warranted.

  3. Chronic kidney disease, severe arterial and arteriolar sclerosis and kidney neoplasia: on the spectrum of kidney involvement in MELAS syndrome

    Directory of Open Access Journals (Sweden)

    Piccoli Giorgina

    2012-02-01

    Full Text Available Abstract Background MELAS syndrome (MIM ID#540000, an acronym for Mitochondrial Encephalopathy, Lactic Acidosis and Stroke-like episodes, is a genetically heterogeneous mitochondrial disorder with protean manifestations and occasional kidney involvement. Interest in the latter is rising due to the identification of cases with predominant kidney involvement and to the hypothesis of a link between mitochondrial DNA and kidney neoplasia. Case presentation We report the case of a 41-year-old male with full blown MELAS syndrome, with lactic acidosis and neurological impairment, affected by the "classic" 3243A > G mutation of mitochondrial DNA, with kidney cancer. After unilateral nephrectomy, he rapidly developed severe kidney functional impairment, with nephrotic proteinuria. Analysis of the kidney tissue at a distance from the two tumor lesions, sampled at the time of nephrectomy was performed in the context of normal blood pressure, recent onset of diabetes and before the appearance of proteinuria. The morphological examination revealed a widespread interstitial fibrosis with dense inflammatory infiltrate and tubular atrophy, mostly with thyroidization pattern. Vascular lesions were prominent: large vessels displayed marked intimal fibrosis and arterioles had hyaline deposits typical of hyaline arteriolosclerosis. These severe vascular lesions explained the different glomerular alterations including ischemic and obsolescent glomeruli, as is commonly observed in the so-called "benign" arteriolonephrosclerosis. Some rare glomeruli showed focal segmental glomerulosclerosis; as the patient subsequently developed nephrotic syndrome, these lesions suggest that silent ischemic changes may result in the development of focal segmental glomerulosclerosis secondary to nephron loss. Conclusions Nephron loss may trigger glomerular sclerosis, at least in some cases of MELAS-related nephropathy. Thus the incidence of kidney disease in the "survivors" of MELAS

  4. Handwriting changes due to aging and Parkinson's syndrome.

    Science.gov (United States)

    Walton, J

    1997-08-22

    Wills signed by elderly people are often contested on the grounds the the signature is different from their earlier specimen signatures. Neurological disease, which can affect handwriting, is very common and progressive amongst elderly people. Handwriting change due to old age and neurological disease is poorly understood. To better understand this subject, we carried out a large methodical study based on almost 200 handwriting specimens of Parkinson patients and age-matched controls. Interestingly, our findings indicate that some of the handwriting changes which occur in these populations tend to resemble forgery indicia although upon close inspection they are distinguishable from them. Thus, document examiners are urged to exercise caution in assessing purported forgeries on wills and other documents signed of written during older age or a writer suffering from neurological disease. PMID:9291592

  5. Elevated serum β2-GPI/ox-LDL complexes levels in children with nephrotic syndrome%肾病综合征患儿血清β2-糖蛋白I/氧化低密度脂蛋白复合物水平升高的研究

    Institute of Scientific and Technical Information of China (English)

    黄中伟; 张春妮; 季茳; 牛冬梅; 罗阳; 蔡晓懿; 夏正坤; 杨玉华; 钟天鹰; 汪俊军

    2013-01-01

    Objective Oxidized low density lipoprotein (ox-LDL) easily forms stable complex with β2 -glycoprotein I (β2 -GPI) in circulation, which is regarded as a novel risk factor for thrombosis and atherosclerosis. Children with nephritic syndrome (NS) suffer from hyperlipidemia and increased oxidative stress. NS is associated with increased risk of glomerulosclerosis and thromboembo-lism. The aim of this study is to measure the serum concentrations of β2 -GPI/ox-LDL complex and its correlation with childhood NS.Methods The concentrations of β2-GPI/ox-LDL complex wereexamined in 84 patients with NS and 84 age/sex-matched health)' controls by ELISA using rabbit anti-human β2 -GPI antibody as the capture antibody, and quantitated with anti-apo(B) polyclonal antibody-enzyme conjugate. The concentrations of ox-LDL in serum were examined by ELISA, and the levels of serum lipids and kidney parameters were determined simultaneously. Results The concentrations of serum β2 -GPI/ox-LDL complex in children with NS were significantly higher than those in controls ([ 1. 08±0.47 ]U/ml vs [0.54±0.29 ]U/ml, P<0.01). Ox-LDL levels were also markedly elevated ([ 143.76±64.55 ]mg/L vs [89.11±53. 80 ]mg/L, P<0.01). The concentrations of β2 -GPI/ox-LDL were positively correlated with ox-LDL (r = 0.721), LDL-C (r = 0.518), total cholesterol (r =0.503) and urine protein (r = 0.326) levels in children with NS (P<0.01), while negatively related with serum albumin (Alb) in children with NS (r = -0.417, P<0.01). Multiple linear regression analysis found the related change of ox-LDL (β = 0.527, P = 0.000), urine protein (β= 0.172,P = 0.036) and LDL-C (β = 0.203 , P = 0.037) accounted for 49. 5% of the variation in the change of (β -GPI/ox-LDL. Conclusion The levels of (β-GPI/ox-LDL in serum complexes levels significantly elevat influenced by the condition of NS patients in NS patients, which may have a potential link with the pathology of childhood NS.%目的 在血循环

  6. Pathomorphological cardial changes in the sudden infant death syndrome

    Directory of Open Access Journals (Sweden)

    Rozumny D.V.

    2014-03-01

    Full Text Available In order to establish the relationship between the timing of fetal obliteration of communication and expression remodeling of the heart chambers, as one of the possible causative factors of SIDS, the selected material has been based on macromorphometric study. Material included 43 hearts from infants who died of SIDS. The control group consisted of 48 hearts of children suddenly dead from aspiration of gastric contents and infectious disease. The methods composed a macroscopic examination of the heart by the method of separate weighing of the complex morphometric studies, histological examination (used the following stains: hematoxylin and eosin, picrofuchsin by Van Gieson, Nissl's stain, immunohistochemical method using markers of neurofilament. The results revealed that in children who died of SIDS, obliteration of the fetal communication occurs essentially in the late period —to the age of 8 months, and the mass of the left and right ventricles of the heart exceeds the age limit of normal due to the prevalence of ventricular muscle mass and hypertrophy of cardiomyocytes. In all parts of the heart subendocardial fibrosis in the ventricles — pronounced interstitial fibrosis has been observed. In the interatrial septum at the location at the oval window morphometric study resulted in significant predominance area percentage of connective tissue in relation to the muscle tissue. On histological sections elements of the nervous tissue — randomly arranged nerve ganglia with degenerative changes and nerve fibers were identified. Conclusion. Finally, it should be noted that the growth of cardiosclerosis in all parts of the heart in newborns and infants, who died suddenly from SIDS, extends the terms of obliteration of fetal communications, and degenerative changes in the nerve ganglia that lead to the disturbance of supply and distribution of nerve impulses that pass through them. These factors contribute to the development of hemodynamic

  7. Chest X ray changes in severe acute respiratory syndrome cases after discontinuation of glucocorticosteroids treatment

    Institute of Scientific and Technical Information of China (English)

    姚婉贞; 陈亚红; 张立强; 王筱宏; 孙永昌; 孙威; 韩江莉; 张福春; 郑亚安; 孙伯章; 贺蓓; 赵鸣武

    2004-01-01

    @@ Severe acute respiratory syndrome (SARS) is a disease identified in Asia, North America and Europe. The drugs for treatment and prevention of and vaccine for the disease are in research.1,2 There is still no agreement on glucocorticosteroid treatment of SARS. In treatment of SARS patients with glucocorticosteroids, we found 5 cases whose chest X ray changes were different from what the literature reported.

  8. Changes of monocyte subsets in patients with acute coronary syndrome and correlation with myocardial injury markers

    OpenAIRE

    Zhu, Li; Yin, Yigang; Zhou, Ruifang; Lin, Jie; Li, Jianming; Ye, Jun

    2015-01-01

    Objective: To explore the changes of peripheral blood monocytes subsets in acute coronary syndrome (ACS) and its clinical significance. Methods: A total of 68 ACS patients and 27 healthy subjects (HS) were enrolled. Monocyte subset analysis was performed using flow cytometry: CD14++CD16-(Mon1), CD14++CD16+ (Mon2), and CD14+CD16++ (Mon3). Results: 1. The number of Mon1 and Mon3 were significantly increased in ACS patients compared with HS (P

  9. Electrocardiographic changes following exercise in the congenitally deaf school children: relationship with Jervell Lange Neilsen syndrome (the Long QT syndrome).

    Science.gov (United States)

    Srivastava, R D; Pramod, J; Deep, J; Jaison, T M; Singh, S; Soni, K

    1998-10-01

    The present study was conducted to test the effects of exercise stress on the ECG of the congenitally deaf children from school for deaf, in view of the occurrence of the Jervell-Lange Neilsen (Surdo Cardiac) variant of the Long QT Syndrome (LQTS) in them. An ECG Lead II was recorded at rest and after two minutes of static jogging. For comparison, the same protocol was repeated in normal healthy children from another school. ECG were analysed for the calculation of corrected QT interval (QTc) by Bazett's equation QTc = QT/square root of R-R and also for the evidence for other abnormalities. Both in the normal and deaf children, exercise did not produce significant (P > 0.05) change in QTc from their resting values. However, when pre and post exercise QTc values of deaf children were compared with normal children, the female deaf had significantly longer QTc (P < 0.01) both at rest and after exercise than normal female children. Normal children did not show significant ECG abnormality either at rest or on exercise. On the contrary many of their counter part (deaf) exhibited occasional ECG abnormality at rest but plethora of abnormalities after exercise viz., sinus arrhythmias, sinus pauses, ST depression, T-inversion, biphasic-T, notched-T, T-alternans, nodal ectopics and junctional rhythm. These results lend credence to the hypothesis of sympathetic imbalance and repolarisation defects in deaf children's heart, which in more severe form could pass into frank Jervell-Lange Neilsen variant of the Long: QT Syndrome. PMID:10874353

  10. Changes in Socio-Emotional and Behavioral Functioning After Attending a Camp for Children with Tourette Syndrome: A Preliminary Investigation.

    Science.gov (United States)

    Eaton, Cyd K; LaMotte, Julia; Gutierrez-Colina, Ana M; Kardon, Patricia; Blount, Ronald L

    2016-08-01

    Children and adolescents with Tourette syndrome are at risk for social, emotional, and behavioral difficulties that may negatively influence feelings of self-competence and attitudes toward having this diagnosis. Attending a camp for children with Tourette syndrome may facilitate improvements in overall socio-emotional and behavioral functioning and self-perception. The current study obtained data from 37 campers (76 % male, 24 % female) and 47 caregivers of campers to investigate changes in children's emotional and behavioral functioning, self-perception (i.e., social and general), attitudes towards having Tourette syndrome, and how different they felt from peers after attending a 1-week summer camp for youth with Tourette syndrome using a pre-post design. Results indicated that campers endorsed a significantly improved sense of social self-competence and more favorable attitudes toward having Tourette syndrome post-camp. Campers also tended to endorse feeling less different than peers at camp versus peers in general. Caregivers endorsed significantly less severe symptoms associated with Tourette syndrome for campers after attending camp. No pre-to-post-camp changes were observed for campers' levels of anxiety or obsessive-compulsive symptoms. These preliminary findings suggest that attending camp or having other opportunities to interact with similar peers may promote better perceptions of social self-competence, more favorable attitudes toward having Tourette syndrome, and a stronger sense of affiliation with peers for children with Tourette syndrome. PMID:26589437

  11. Red blood cells in Rett syndrome: oxidative stress, morphological changes and altered membrane organization.

    Science.gov (United States)

    Ciccoli, Lucia; De Felice, Claudio; Leoncini, Silvia; Signorini, Cinzia; Cortelazzo, Alessio; Zollo, Gloria; Pecorelli, Alessandra; Rossi, Marcello; Hayek, Joussef

    2015-11-01

    In this review, we summarize the current evidence on the erythrocyte as a previously unrecognized target cell in Rett syndrome, a rare (1:10 000 females) and devastating neurodevelopmental disorder caused by loss-of-function mutations in a single gene (i.e. MeCP2, CDKL5, or rarely FOXG1). In particular, we focus on morphological changes, membrane oxidative damage, altered membrane fatty acid profile, and aberrant skeletal organization in erythrocytes from patients with typical Rett syndrome and MeCP2 gene mutations. The beneficial effects of ω-3 polyunsaturated fatty acids (PUFAs) are also summarized for this condition to be considered as a 'model' condition for autism spectrum disorders.

  12. Intraoperative "Kounis syndrome" that improved electrocardiography changes and hemodynamic situation afteradministering nitroglycerine

    Directory of Open Access Journals (Sweden)

    Victoria O. Sánchez

    2014-07-01

    Full Text Available A 58-year-old female without cardiovascular risk factors, was going to be operated to repair the rotator cuff. Induction and interscalene brachial plexus block were uneventful, but after her placement for surgery the patient started with severe bronchospasm, hypotension, cutaneous allergic reaction and ST elevation on the electrocardiogram. An anaphylactic shock was suspected and treated but until the perfusion of nitroglycerina was started no electrocardiographic changes resolved. After necessary diagnostic test the final diagnosis was variant I of Kounis syndrome due to cefazolin and rocuronium. Ephinephrine is the cornerstone of treatment for anaphylaxis but should we use it if the anaphylactic reaction is also accompanied by myocardial ischemia? The answer is that we should not use it because myocardial ischemia in this syndrome is caused by vasospasm, so it would be more useful drugs such as nitroglycerin. But what if we do not know if it is a Kounis syndrome or not? In this article we report our experience that maybe could help you in a similar situation.

  13. Clinical significance of changes of serum leptin and insulin levels in patients with polycystic ovary syndrome

    International Nuclear Information System (INIS)

    Objective: To explore the relationship between the serum leptin, insulin levels and development of polycystic ovary syndrome (PCOS). Methods: Serum leptin and insulin levels (with RIA) were determined in 34 patients with PCOS and 30 controls. Results: The serum leptin and insulin levels in the 34 PCOS patients were significantly higher than those in controls (P<0. 01), and those in obese patients (n=22) were significantly higher than those in non-obese ones (n=12) too(P<0.01). Conclusion: Changes of serum leptin and insulin levels were closely related to the development of PCOS and leptin might be used as a diagnostic indicator for PCOS. (authors)

  14. Changes in the epidemiology of sudden infant death syndrome in Sweden 1973-1996

    OpenAIRE

    Alm, B; Norvenius, S; Wennergren, G; Skjarven, R; Oyen, N; Milerad, J; Wennborg, M; Kjaerbeck, J; Helweg-Larsen, K.; Irgens, L.

    2001-01-01

    BACKGROUND—From the early 1970s to the early 1990s, there was a significant rise in the incidence of sudden infant death syndrome (SIDS) in Scandinavia. Following the risk reducing campaign, the incidence has fallen to about the same level as in 1973.
AIMS—To identify the changes that have occurred in the epidemiology of SIDS.
METHODS—We compared the Swedish part of the Nordic Epidemiological SIDS Study (NESS), covering the years 1992-1995, with two earlier, descriptive s...

  15. Diurnal changes of blood pressure values (24 h blood pressures in women with polycystic ovary syndrome

    Directory of Open Access Journals (Sweden)

    Barbara Grzechocińska

    2011-06-01

    Full Text Available Background: More attention is being paid lately to polycystic ovary syndrome (PCOS not only in the aspect of fertility but also according to long-term metabolic and cardiovascular abnormalities. Observations of women with PCOS show that the occurrence of blood hypertension is more frequent after the menopause. There are few articles concerning the predictable symptoms of blood hypertension. It seems that twenty-four hour blood pressure monitoring and assessment of changes of diurnal rhythm could be useful in this group of patients.Aim of the study: The purpose of this study was to assess diurnal changes of blood pressure in women with polycystic ovary syndrome.Material and methods: 26 women with PCOS diagnosed according to Rotterdam consensus criteria and Androgen Excess Society (AES criteria were included in our study. The mean age of the examined women was 29.5 years and the mean BMI (body mass index was 24.7 kg/m2. The control group consisted of 12 age-matched women, without PCOS. Twenty-four hour Holter monitoring of ambulatory blood pressure was performed during normal daily activity in all patients. Halberg’s cosinor method was used to analyse daily biorhythm.Results: Results show the diurnal changes of systolic blood pressure in the preclinical phase in 30% and diastolic in 15% of PCOS women. In this group no physiological decrease of blood pressure at night time was observed.

  16. Changes in illness perceptions mediated the effect of cognitive behavioural therapy in severe functional somatic syndromes

    DEFF Research Database (Denmark)

    Christensen, Sara Sletten; Frostholm, Lisbeth; Ørnbøl, Eva;

    2014-01-01

    . Methods We analysed additional data from a randomised controlled trial comparing completers of cognitive behavioural group therapy (46 patients) to an enhanced usual care group (66 patients). Proposed mediators (illness perceptions) and primary (physical health) and secondary (somatic symptoms and illness...... (primary analysis), and (2) whether changes in illness perceptions during the whole trial period were associated with improved outcome (secondary analysis). Results Improvements in illness perceptions during treatment partially mediated the effect of cognitive behavioural therapy on physical health one......Objective Although there is substantial evidence that cognitive behavioural therapy alleviates symptoms in functional somatic syndromes, the mechanisms of change are less investigated. This study examined whether changes in illness perceptions mediated the effect of cognitive behavioural therapy...

  17. Renal manifestations of human brucellosis: First report of minimal change disease.

    Science.gov (United States)

    Sabanis, Nikolaos; Gavriilaki, Eleni; Paschou, Eleni; Tsotsiou, Eleni; Kalaitzoglou, Asterios; Kavlakoudis, Christos; Vasileiou, Sotirios

    2016-05-01

    Human brucellosis is considered a great example of the complexity of clinical manifestations possibly affecting multiple organs or systems. Renal manifestations of human brucellosis have been documented in few case reports and one case series. Herein, we present a case of Nephrotic syndrome (NS) due to minimal change disease in the course of acute brucellosis. A 53-year-old male farmer was admitted to our department with acute brucellosis and NS. Renal biopsy revealed minimal change disease. Combined treatment with prednisone (1 mg/kg), rifampicin (600 mg/day), and doxycycline (200 mg/day) was initiated. Complete remission of NS was achieved at the end of the fourth week. One year later, the patient remained in complete remission of NS without any sign of relapse of brucellosis.

  18. Structural changes in the somatosensory system correlate with tic severity in Gilles de la Tourette syndrome

    DEFF Research Database (Denmark)

    Thomalla, Götz; Siebner, Hartwig R; Jonas, Melanie;

    2009-01-01

    Gilles de la Tourette syndrome (GTS) is a neuropsychiatric disorder characterized by multiple motor and vocal tics. Previous structural MRI studies have identified regional abnormalities in grey matter, especially in the basal ganglia. These findings are consistent with the assumption of alterati......Gilles de la Tourette syndrome (GTS) is a neuropsychiatric disorder characterized by multiple motor and vocal tics. Previous structural MRI studies have identified regional abnormalities in grey matter, especially in the basal ganglia. These findings are consistent with the assumption...... of white matter changes in GTS. In this study, we aimed to examine whether GTS is associated with abnormalities in white matter microstructure and whether these changes are correlated with tic severity. In a morphometric study based on diffusion tensor MRI of the whole brain, we compared brain tissue...... the groups. We also tested for a linear relationship between regional FA values and clinical scores of tic severity. Probabilistic fibre tracking was applied to characterize anatomical connectivity of those areas showing differences in regional FA. Compared with healthy controls, GTS patients showed...

  19. Cell biology and genetics of minimal change disease

    Science.gov (United States)

    Saleem, Moin A.; Kobayashi, Yasuko

    2016-01-01

    Minimal change disease (MCD) is an important cause of nephrotic syndrome and is characterized by massive proteinuria and hypoalbuminemia, resulting in edema and hypercholesterolemia. The podocyte plays a key role in filtration and its disruption results in a dramatic loss of function leading to proteinuria. Immunologic disturbance has been suggested in the pathogenesis of MCD. Because of its clinical features, such as recurrent relapse/remission course, steroid response in most patients, and rare familial cases, a genetic defect has been thought to be less likely in MCD. Recent progress in whole-exome sequencing reveals pathogenic mutations in familial cases in steroid-sensitive nephrotic syndrome (SSNS) and sheds light on possible mechanisms and key molecules in podocytes in MCD. On the other hand, in the majority of cases, the existence of circulating permeability factors has been implicated along with T lymphocyte dysfunction. Observations of benefit with rituximab added B cell involvement to the disease. Animal models are unsatisfactory, and the humanized mouse may be a good model that well reflects MCD pathophysiology to investigate suggested “T cell dysfunction” directly related to podocytes in vivo. Several candidate circulating factors and their effects on podocytes have been proposed but are still not sufficient to explain whole mechanisms and clinical features in MCD. Another circulating factor disease is focal segmental glomerulosclerosis (FSGS), and it is not clear if this is a distinct entity, or on the same spectrum, implicating the same circulating factor(s). These patients are mostly steroid resistant and often have a rapid relapse after transplantation. In clinical practice, predicting relapse or disease activity and response to steroids is important and is an area where novel biomarkers can be developed based on our growing knowledge of podocyte signaling pathways. In this review, we discuss recent findings in genetics and podocyte biology in

  20. A low-protein diet restricts albumin synthesis in nephrotic rats.

    OpenAIRE

    Kaysen, G A; Jones, H.; Martin, V.; Hutchison, F N

    1989-01-01

    High-protein diets increase albumin synthesis in rats with Heymann nephritis but albuminuria increases also, causing serum albumin concentration to be suppressed further than in nephrotic animals eating a low-protein diet. Experiments were designed to determine whether dietary protein augmentation directly stimulates albumin synthesis, or whether instead increased albumin synthesis is triggered by the decrease in serum albumin concentration. Evidence is presented that dietary protein augmenta...

  1. Parents’ Readiness to Change Affects BMI Reduction Outcomes in Adolescents with Polycystic Ovary Syndrome

    Directory of Open Access Journals (Sweden)

    Karen P. Jakubowski

    2012-01-01

    Full Text Available Evidence supports the importance of parental involvement for youth’s ability to manage weight. This study utilized the stages of change (SOC model to assess readiness to change weight control behaviors as well as the predictive value of SOC in determining BMI outcomes in forty adolescent-parent dyads (mean adolescent age = 15 ± 1.84 (13–20, BMI = 37 ± 8.60; 70% white participating in a weight management intervention for adolescent females with polycystic ovary syndrome (PCOS. Adolescents and parents completed a questionnaire assessing their SOC for the following four weight control domains: increasing dietary portion control, increasing fruit and vegetable consumption, decreasing dietary fat, and increasing usual physical activity. Linear regression analyses indicated that adolescent change in total SOC from baseline to treatment completion was not predictive of adolescent change in BMI from baseline to treatment completion. However, parent change in total SOC from baseline to treatment completion was predictive of adolescent change in BMI, (t(24 = 2.15, p=0.043. Findings support future research which carefully assesses adolescent and parent SOC and potentially develops interventions targeting adolescent and parental readiness to adopt healthy lifestyle goals.

  2. Hypertriglyceridemia Thalassemia Syndrome: A Report of 4 Cases

    OpenAIRE

    Khera, Rachna; Singh, Meeta; Goel, Garima; Gupta, Parul; Singh, Tejinder; A P Dubey

    2014-01-01

    Hypertriglyceridemia in children can be familial or acquired. Acquired forms of hypertriglyceridemia in children may be associated with several other diseases obesity, diabetes mellitus, uremia/dialysis, hypothyroidism, nephrotic syndrome, drugs etc. Hypertriglyceridemia with β-thalassemia major is an association of unknown pathogenesis which is rarely described in the literature but is important to recognize, for the prevention of complications and proper management of thalassemic children.

  3. Aerobic exercise training induces metabolic benefits in rats with metabolic syndrome independent of dietary changes

    Directory of Open Access Journals (Sweden)

    Paula Wesendonck Caponi

    2013-07-01

    Full Text Available OBJECTIVES: We evaluated the effects of aerobic exercise training without dietary changes on cardiovascular and metabolic variables and on the expression of glucose transporter Type 4 in rats with metabolic syndrome. METHODS: Twenty male spontaneously hypertensive rats received monosodium glutamate during the neonatal period. The animals were allocated to the following groups: MS (sedentary metabolic syndrome, MS-T (trained on a treadmill for 1 hour/day, 5 days/week for 10 weeks, H (sedentary spontaneously hypertensive rats and H-T (trained spontaneously hypertensive rats. The Lee index, blood pressure (tail-cuff system, insulin sensitivity (insulin tolerance test and functional capacity were evaluated before and after 10 weeks of training. Glucose transporter Type 4 expression was analyzed using Western blotting. The data were compared using analysis of variance (ANOVA (p<0.05. RESULTS: At baseline, the MS rats exhibited lower insulin sensitivity and increased Lee index compared with the H rats. Training decreased the body weight and Lee index of the MS rats (MS-T vs. MS, but not of the H rats (H-T vs. H. There were no differences in food intake between the groups. At the end of the experiments, the systolic blood pressure was lower in the two trained groups than in their sedentary controls. Whole-body insulin sensitivity increased in the trained groups. Glucose transporter Type 4 content increased in the heart, white adipose tissue and gastrocnemius muscle of the trained groups relative to their respective untrained groups. CONCLUSION: In conclusion, the present study shows that an isolated aerobic exercise training intervention is an efficient means of improving several components of metabolic syndrome, that is, training reduces obesity and hypertension and increases insulin sensitivity.

  4. Early Change of Extracellular Matrix and Diastolic Parameters in Metabolic Syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Santos, Angela B. S., E-mail: angelabssantos@yahoo.com.br [Hospital de Clínicas de Porto Alegre, Porto Alegre, RS (Brazil); Universidade Federal do Rio Grande do Sul, Porto Alegre, RS (Brazil); Junges, Mauricio; Silvello, Daiane; Macari, Adriana; Araújo, Bruno S. de [Universidade Federal do Rio Grande do Sul, Porto Alegre, RS (Brazil); Seligman, Beatriz G. [Hospital de Clínicas de Porto Alegre, Porto Alegre, RS (Brazil); Universidade Federal do Rio Grande do Sul, Porto Alegre, RS (Brazil); Duncan, Bruce B. [Universidade Federal do Rio Grande do Sul, Porto Alegre, RS (Brazil); Rohde, Luis Eduardo P.; Clausell, Nadine; Foppa, Murilo [Hospital de Clínicas de Porto Alegre, Porto Alegre, RS (Brazil); Universidade Federal do Rio Grande do Sul, Porto Alegre, RS (Brazil)

    2013-10-15

    Metabolic syndrome (MS) is associated with increased cardiovascular risk. It is not clear whether myocardial changes showed in this syndrome, such as diastolic dysfunction, are due to the systemic effects of the syndrome, or to specific myocardial effects. Compare diastolic function, biomarkers representing extracellular matrix activity (ECM), inflammation and cardiac hemodynamic stress in patients with the MS and healthy controls. MS patients (n = 76) and healthy controls (n=30) were submitted to a clinical assessment, echocardiographic study, and measurement of plasma levels of metalloproteinase-9 (MMP9), tissue inhibitor of metalloproteinase-1 (TIMP1), ultrasensitive-reactive-C-Protein (us-CRP), insulin resistance (HOMA-IR) and natriuretic peptide (NT-proBNP). MS group showed lower E' wave (10.1 ± 3.0 cm/s vs 11.9 ± 2.6 cm/s, p = 0.005), increased A wave (63.4 ± 14.1 cm/s vs. 53.1 ± 8.9 cm/s; p < 0.001), E/E' ratio (8.0 ± 2.2 vs. 6.3 ± 1.2; p < 0.001), MMP9 (502.9 ± 237.1 ng / mL vs. 330.4±162.7 ng/mL; p < 0.001), us-CRP (p = 0.001) and HOMA-IR (p < 0.001), but no difference for TIMP1 or NT-proBNP levels. In a multivariable analysis, only MMP9 was independently associated with MS. MS patients showed differences for echocardiographic measures of diastolic function, ECM activity, us-CRP and HOMA-IR when compared to controls. However, only MMP9 was independently associated with the MS. These findings suggest that there are early effects on ECM activity, which cannot be tracked by routine echocardiographic measures of diastolic function.

  5. Myelodysplastic syndrome complicated by central diabetes insipidus and cerebral salt wasting syndrome with peculiar change in magnetic resonance images.

    Science.gov (United States)

    Sano, Soichi; Yamagami, Keiko; Morikawa, Takashi; Yoshioka, Katsunobu

    2010-01-01

    Central diabetes insipidus (CDI) could occurs in patients with myelodysplastic syndrome (MDS) or acute myeloid leukemia (AML), because of infiltration of leukemic cells into the neurohypophysis or some other reason and it is closely associated with abnormalities of chromosome 7. We report a case of MDS with abnormalities of chromosome 7, presenting as CDI followed by deterioration of polyuria and hyponatremia with a decreased extracellular fluid volume. Magnetic resonance imaging (MRI) revealed symmetrically enhanced lesions in the hypothalamus. Fludrocortisone treatment normalized his serum sodium level and cerebral salt wasting syndrome (CSWS) was suspected.

  6. Significance of Experiments on the Relationship of Spleen Deficiency Syndrome with Gastrin and Somatostatin Changes

    Institute of Scientific and Technical Information of China (English)

    姚永莉; 徐波; 宋于刚; 张万岱

    2002-01-01

    Objective: To explore the relationship among gast ri n, somatostatin and pathogenesis of spleen deficiency syndrome (SDS). Me thods: The changes of gastrin and somatostatin in body fluid and tissues were measured by radioimmunoassay. G cells (gastrin cells) and D cells (s omatostatin cells) of gastroduodenal mucosa were analyzed with polyclonal antibo dy to gastrin and somatostatin, immunohistochemical technique and Quantimet 500 image analysis system. Sijunzi decoction (四君子汤, SJZD) was chosen for counter -co nfirmation of syndrome. Results: The levels of gastrin in plasma, gastric juice, intestinal juice, gastric antrum, duodenum and hypothalamus of e x perimental SDS were significantly lowered and somatostatin markedly higher than the control group, which was improved and even better than spontaneous recovery group after prevention and treatment with SJZD (P<0.05). The count of G cell s and D cells reduced (P<0.05), the area of D cells declined (P<0.05), but m ean grey of G cells elevated (P<0.05) and ratio of the count and area on G/D also in crea sed (P<0.05) in experimental SDS, which was improved and even better than sp onta neous recovery group after prevention and treatment with SJZD (P<0.05). Con clusion: The study confirmed that disturbance of relationship betwee n gastrin and somatostatin would lead to gastrointestinal disorder, which was on e of important causes for pathogenesis of SDS.

  7. The relationship between irritable bowel syndrome and psychiatric disorders: from molecular changes to clinical manifestations.

    Science.gov (United States)

    Fadgyas-Stanculete, Mihaela; Buga, Ana-Maria; Popa-Wagner, Aurel; Dumitrascu, Dan L

    2014-01-01

    Irritable bowel syndrome (IBS) is a functional syndrome characterized by chronic abdominal pain accompanied by altered bowel habits. Although generally considered a functional disorder, there is now substantial evidence that IBS is associated with a poor quality of life and significant negative impact on work and social domains. Neuroimaging studies documented changes in the prefrontal cortex, ventro-lateral and posterior parietal cortex and thalami, and implicate alteration of brain circuits involved in attention, emotion and pain modulation. Emerging data reveals the interaction between psychiatric disorders including generalized anxiety disorder, panic disorder, major depressive disorder, bipolar disorder, and schizophrenia and IBS, which suggests that this association should not be ignored when developing strategies for screening and treatment. Psychological, social and genetic factors appear to be important in the development of IBS symptomatology through several mechanisms: alteration of HPA axis modulation, enhanced perception of visceral stimuli or psychological vulnerability. Elucidating the molecular mechanisms of IBS with or without psychiatric comorbidities is crucial for elucidating the pathophysiology and for the identification of new therapeutical targets in IBS. PMID:25408914

  8. Reliability and sensitivity to change of the timed standing balance test in children with down syndrome

    Directory of Open Access Journals (Sweden)

    Vencita Priyanka Aranha

    2016-01-01

    Full Text Available Objective: To estimate the reliability and sensitivity to change of the timed standing balance test in children with Down syndrome (DS. Methods: It was a nonblinded, comparison study with a convenience sample of subjects consisting of children with DS (n = 9 aged 8–17 years. The main outcome measure was standing balance which was assessed using timed standing balance test, the time required to maintain in four conditions, eyes open static, eyes closed static, eyes open dynamic, and eyes closed dynamic. Results: Relative reliability was excellent for all four conditions with an Interclass Correlation Coefficient (ICC ranging from 0.91 to 0.93. The variation between repeated measurements for each condition was minimal with standard error of measurement (SEM of 0.21–0.59 s, suggestive of excellent absolute reliability. The sensitivity to change as measured by smallest real change (SRC was 1.27 s for eyes open static, 1.63 s for eyes closed static, 0.58 s for eyes open dynamic, and 0.61 s for eyes closed static. Conclusions: Timed standing balance test is an easy to administer test and sensitive to change with strong absolute and relative reliabilities, an important first step in establishing its utility as a clinical balance measure in children with DS.

  9. Grey matter changes of the pain matrix in patients with burning mouth syndrome.

    Science.gov (United States)

    Sinding, Charlotte; Gransjøen, Anne Mari; Schlumberger, Gina; Grushka, Miriam; Frasnelli, Johannes; Singh, Preet Bano

    2016-04-01

    Burning mouth syndrome (BMS) is characterized by a burning sensation in the mouth, usually in the absence of clinical and laboratory findings. Latest findings indicate that BMS could result from neuropathic trigeminal conditions. While many investigations have focused on the periphery, very few have examined possible central dysfunctions. To highlight changes of the central system of subjects with BMS, we analysed the grey matter concentration in 12 subjects using voxel-based morphometry. Data were compared with a control group (Ct). To better understand the brain mechanisms underlying BMS, the grey matter concentration of patients was also compared with those of dysgeusic patients (Dys). Dysgeusia is another oral dysfunction condition, characterized by a distorted sense of taste and accompanied by a reduced taste function. We found that a major part of the 'pain matrix' presented modifications of the grey matter concentration in subjects with BMS. Six regions out of eight were affected [anterior and posterior cingulate gyrus, lobules of the cerebellum, insula/frontal operculum, inferior temporal area, primary motor cortex, dorsolateral pre-frontal cortex (DLPFC)]. In the anterior cingulate gyrus, the lobules of the cerebellum, the inferior temporal lobe and the DLPFC, pain intensity correlated with grey matter concentration. Dys also presented changes in grey matter concentration but in different areas of the brain. Our results suggest that a deficiency in the control of pain could in part be a cause of BMS and that BMS and dysgeusia conditions are not linked to similar structural changes in the brain. PMID:26741696

  10. Challenges in identifying Lennox-Gastaut syndrome in adults: A case series illustrating its changing nature.

    Science.gov (United States)

    Piña-Garza, Jesus Eric; Chung, Steve; Montouris, Georgia D; Radtke, Rodney A; Resnick, Trevor; Wechsler, Robert T

    2016-01-01

    The variable presentation and progression of Lennox-Gastaut syndrome (LGS) can make it difficult to recognize, particularly in adults. To improve diagnosis, a retrospective chart review was conducted on patients who were diagnosed as adults and/or were followed for several years after diagnosis. We present 5 cases that illustrate changes in LGS features over time. Cases 1 and 2 were diagnosed by age 8 with intractable seizures, developmental delay, and abnormal EEGs with 1.5-2 Hz SSW discharges. However, seizure type and frequency changed over time for both patients, and the incidence of SSW discharges decreased. Cases 3, 4, and 5 were diagnosed with LGS as adults based on current and past features and symptoms, including treatment-resistant seizures, cognitive and motor impairment, and abnormal EEG findings. While incomplete, their records indicate that an earlier LGS diagnosis may have been missed or lost to history. These cases demonstrate the need to thoroughly and continuously evaluate all aspects of a patient's encephalopathy, bearing in mind the potential for LGS features to change over time.

  11. Challenges in identifying Lennox–Gastaut syndrome in adults: A case series illustrating its changing nature

    Directory of Open Access Journals (Sweden)

    Jesus Eric Piña-Garza

    2016-01-01

    Full Text Available The variable presentation and progression of Lennox–Gastaut syndrome (LGS can make it difficult to recognize, particularly in adults. To improve diagnosis, a retrospective chart review was conducted on patients who were diagnosed as adults and/or were followed for several years after diagnosis. We present 5 cases that illustrate changes in LGS features over time. Cases 1 and 2 were diagnosed by age 8 with intractable seizures, developmental delay, and abnormal EEGs with 1.5–2 Hz SSW discharges. However, seizure type and frequency changed over time for both patients, and the incidence of SSW discharges decreased. Cases 3, 4, and 5 were diagnosed with LGS as adults based on current and past features and symptoms, including treatment-resistant seizures, cognitive and motor impairment, and abnormal EEG findings. While incomplete, their records indicate that an earlier LGS diagnosis may have been missed or lost to history. These cases demonstrate the need to thoroughly and continuously evaluate all aspects of a patient's encephalopathy, bearing in mind the potential for LGS features to change over time.

  12. Kidney biopsy

    Science.gov (United States)

    ... Goodpasture syndrome IgA nephropathy Interstitial nephritis Lupus nephritis Medullary cystic kidney disease Membranoproliferative glomerulonephritis Membranous nephropathy Minimal change disease Nephrotic ...

  13. Correlation of skin changes with hormonal changes in polycystic ovarian syndrome: A cross-sectional study clinical study

    Directory of Open Access Journals (Sweden)

    B Vijaya Gowri

    2015-01-01

    Full Text Available Background: Polycystic ovarian syndrome (PCOS is a heterogenous collection of signs and symptoms that when gathered, form a spectrum of disorder with disturbance of reproductive, endocrine and metabolic functions. Aim: The aim of this study is to correlate the skin manifestations with hormonal changes and to know the incidence and prevalence of skin manifestations in patients with PCOS. Materials and Methods: A total of 40 patients with PCOS were examined during 1 year time period from May 2008 P to May 2009. Detailed clinical history was taken from each patient. PCOS was diagnosed on the basis of ultrasonography. Hormonal assays included fasting blood sugar, postprandial blood sugar, follicle-stimulating hormone, luteinizing hormone, thyroid stimulating hormone, dehydroepiandrostenedione, prolactin, free testosterone, fasting lipid profile and sex hormone binding globulin. The results obtained were statistically correlated. Results: In our study, the prevalence of cutaneous manifestations was 90%. Of all the cutaneous manifestations acne was seen in highest percentage (67.5%, followed by hirsutism (62.5%, seborrhea (52.5%, androgenetic alopecia (AGA (30%, acanthosis nigricans (22.5% and acrochordons (10%. Fasting insulin levels was the most common hormonal abnormality seen in both acne and hirsutism, whereas AGA was associated with high testosterone levels. Conclusion: The prevalence of cutaneous manifestations in PCOS was 90%. Hirsutism, acne, seborrhea, acanthosis nigricans and acrochordons were associated with increased levels of fasting insulin, whereas AGA showed higher levels of serum testosterone.

  14. The change and its clinical significance of serum cystatin C in elderly patients with obstructive sleep apnea hypopnea syndrome

    Institute of Scientific and Technical Information of China (English)

    张孝斌

    2013-01-01

    Objective To investigate the change of serum cystatin C (CC) level and its clinical significance in elderly patients with obstructive sleep apnea hypopnea syndrome (OSAHS) ,and to evaluate the diagnostic value of serum CC for renal impairment in elderly OSAHS patients.Methods Elderly snoring subjects (age≥60 years) un-

  15. Sexual desire changes during menstrual cycle and relationship with premenstrual syndrome

    Directory of Open Access Journals (Sweden)

    Kiani Asiabar A.

    2007-09-01

    Full Text Available Background: Sexual function in women may be affected by their menstrual cycle. Lack of sexual drive is a deficiency or absence of sexual fantasies and desire for sexual activity. This study aims at determining the changes in sexual desire during the menstrual cycle and those associated with premenstrual syndrome (PMS and evaluates sexual desire during the menstrual cycle and the associated changes with PMS. Methods: The sample for this cross-sectional study includes 150 women employed in factories in Tehran. The instruments for data collection were questionnaires and journals of premenstrual experiences.Results: Analysis of the data showed that the mean age of the subjects was 31 years standard deviation = 8.46(. The most frequent decrease in sexual desire was during the week prior to the start of menstrual bleeding (27.3% and the least frequent was from the end of bleeding to one week before the next period of menstrual bleeding (5.3%. In 24.7% of the cases, an increase in sexual desire occurred during the middle of the menstrual cycle and 27.3% during the course of menstrual bleeding. Moreover, 10.7% of the subjects had an increase in sexual desire during the week before bleeding. Furthermore, a positive correlation was found between changes in sexual desire and PMS (p<0.001. In addition, a positive correlation was found between changes in sexual desire and breast tenderness, joint and muscle pain. Conclusions: The sexual desire of women, with or without PMS, changes during the menstrual cycle. The greatest decrease in sexual desire occurs during the first week before menstrual bleeding in women with PMS. Such information can greatly help toward understanding and treatment in sexual therapy for couples.

  16. CT Manifestations of Lung Changes and Complications in Patients with Severe Acute Respiratory Syndrome

    Institute of Scientific and Technical Information of China (English)

    张雪哲; 王武; 卢延; 黄振国; 洪闻; 尚燕宁; 任安

    2003-01-01

    Objective:To investigate the role of CT scanning in diagnosing severe acute respiratory syndrome(SARS). Methods: One hundred and twelve times of spiral CT scanning, 106 times on the chest with standard pulmonary and mediastinal window, 5 on the brain and once on the abdomen, were performed in 82 patients (37 males and 45 females) of SARS. Results: Bilateral shadows showed in 66 patients (80.48%) and unilateral shadow in 16 (19.52%). The lung CT findings were sub-pleural focal consolidation in 26 patients (31.70%), flaky cloudy opacity in 53 (64.63%), large area consolidation in 9 (10.97%), ground-glass blurry shadow in 31 (37.80%), alveolar substantive shadow in 14 (17.07%) and interstitial changes in 16 (19.51%). The pulmonary CT signs of SARS were relatively characterized by: (1) The lesions tending to multiply occur, mostly to be bilaterally distributed and commonly involved in the lower lung field. (2) The lung shadows mostly showed as sub-pleural focal consolidation, flaky cloudy shadow, large area consolidation, ground-glass blurry shadow, and often accompanied with signs of broncho-inflation. (3) Having opacified nodular shadows in the alveolar cavities. (4) Rapid progressions or changes on the size, amount, and distribution of the lesions likely to be found in dynamic observation of chest X-ray and CT scanning, i.e., markedly dynamic changes found within 24 to 48 hrs. Lesions with these characteristics may be recognized as pulmonary changes possibly induced by SARS. Complications were found in 6 patients (7.31%), including tuberculosis of lung and brain accompanied with pneumomediastinum in one patient, secondary infection of lung in 2, pneumothorax in 1, pulmonary fungus in 1, and pyothorax in 1.Conclusion: CT scanning is a sensitive method for diagnosis of SARS, by which more accurate assessment of the abnormal changes of lung and occurrence of complications in SARS patients can be made.

  17. Early Markers of Tubulointerstitial Fibrosis in Children With Idiopathic Nephrotic Syndrome

    OpenAIRE

    Bieniaś, Beata; Zajączkowska, Małgorzata; Borzęcka, Halina; Sikora, Przemysław; Wieczorkiewicz-Płaza, Anna; Wilczyńska, Barbara

    2015-01-01

    Abstract Tubulointerstitial fibrosis and tubular atrophy play a crucial role in the pathogenesis of chronic kidney disease (CKD). They are also major determinants in chronic kidney disease development and progression in patients with primary renal diseases characterized by persistent or recurrent proteinuria. The purpose of the study was to assess urinary excretion of alpha-glutathione S-transferase (alpha-GST), pi-glutathione S-transferase (pi-GST), neutrophil gelatinase-associated lipocalin...

  18. The role of liver in leptin metabolism in experimental nephrotic syndrome

    OpenAIRE

    Ahmed, Mohamed Mahmoud; Amin, Ahmed Ibrahim; Fahmi, Abdelgawad Ali; Habib, Dawoud Fakhry; Kholousy, Naglaa Mohamed; Shalaby, Mostafa; Shanab, Asem Metwally Abo

    2012-01-01

    Leptin is a hormone influencing food intake, energy expenditure and body weight. It is pro-duced by adipocytes, exerts its effects on brain, endocrine pancreas and other organs by acti-vating trans-membrane receptors and is cleared from plasma mainly by the kidneys. Several studies have suggested that leptin's effects on metabolism are mediated by the liver. Our aim was to evaluate the role of the liver in the metabolism of leptin by comparing the serum leptin level in the portal vein with th...

  19. Vitamin D status is insufficient in the majority of children at diagnosis of nephrotic syndrome

    DEFF Research Database (Denmark)

    Nielsen, Cecilie Ane; Jensen, Jens-Erik Bech; Cortes, Dina

    2015-01-01

    at the time of their diagnosis before treatment with glucocorticoids. The following plasma levels were measured: 25-hydroxy-vitamin-D (25(OH)D), albumin, ionised calcium, phosphate, parathyroid hormone (PTH), alkaline phosphatase and creatinine. The glomerular filtration rate (GFR) was estimated from...

  20. [Dermatoglyphics differences among children with nephrotic syndrome according to steroid response].

    Science.gov (United States)

    Corona-Rivera, Jorge Román; Pérez-Cortés, Gustavo; Osuna-Osuna, Julieta; Garay-Cortés, Marcela Guadalupe; Pérez-Molina, J Jesús; Ramírez-Godínez, Santa; Peña-Padilla, Christian; Rivera-Vargas, Jehú; Bobadilla-Morales, Lucina

    2016-01-01

    Introducción: aunque la asociación entre el tipo de síndrome nefrótico idiopático (SNI) y algún patrón peculiar de huellas digitales sugeriría la presencia de factores genéticos relacionados a ambos, esto no ha sido previamente estudiado. Este estudio pretende evaluar si existen diferencias entre los patrones digitales de niños con SNI resistente a esteroides (SNRE) y aquellos con el SNI sensible a esteroides (SNSE). Métodos: se estudiaron las frecuencias de arcos, asas cubitales, asas radiales y rizos en 60 niños con SNRE y 60 niños con SNSE. Se realizó análisis bivariado para detectar la relación entre cada figura digital y los grupos de estudio mediante la prueba de Chi cuadrada y para evaluar su posible asociación se calcularon los odds ratio (OR) con sus intervalos de confianza del 95 %. Resultados: los pacientes con SNRE tuvieron una mayor frecuencia de rizos en comparación con pacientes con SNSE (46.7 % frente a 30.7 %, p = 0.005). Comparaciones adicionales utilizando una definición de «excesos de rizos» obtenida datos normativos previos de nuestra población (≥ 7 rizos en mujeres o ≥ 8 en varones), también se asoció a la presencia de SNRE (OR: 2.96, IC95 %: 1.15-7.61). Conclusiones: estos hallazgos indican que existen diferencias entre los niños con SNRE y SNSE a nivel de los dermatoglifos digitales, aunque son necesarios estudios adicionales para confirmar la presente asociación y sus posibles implicaciones.

  1. Comparison of three methods for isolation of urinary microvesicles to identify biomarkers of nephrotic syndrome.

    NARCIS (Netherlands)

    Rood, I.M.; Deegens, J.K.J.; Merchant, M.L.; Tamboer, W.P.M.; Wilkey, D.W.; Wetzels, J.F.M.; Klein, J.B.

    2010-01-01

    Urinary microvesicles, such as 40-100 nm exosomes and 100-1000 nm microparticles, contain many proteins that may serve as biomarkers of renal disease. Microvesicles have been isolated by ultracentrifugation or nanomembrane ultrafiltration from normal urine; however, little is known about the efficie

  2. Urinary AQP2 excretion is increased during nephrotic syndrome and is associated with reduced urine production

    DEFF Research Database (Denmark)

    Andersen, René Frydensbjerg; Kamperis, Konstantinos; Frøkjær, Jørgen;

    : The concentration of uAQP2 was significantly higher during active NS compared to remission, 9.9 ng/ml (CI95% 4.3-22.5 ng/ml) vs. 3.9 ng/ml (CI95% 1.5-10.5 ng/ml), p=0.003. Mean urine production was 1.3 ml/h/kg during active NS compared to 1.0 ml/h/kg at remission, p=0.25. A linear regression analysis revealed...... a significant negative relationship between uAQP2 and urine production (r2=-0.20, p=0.05). Urine osmolality and urine production was negatively correlated (r2=-0.44, p=0.009) though urine osmolality did not differ comparing NS to remission 671±58 mOsm/kg vs. 703±44 mOsm/kg, p=0.62. CONCLUSIONS: During active NS...

  3. Quercetin ameliorates cardiovascular, hepatic, and metabolic changes in diet-induced metabolic syndrome in rats.

    Science.gov (United States)

    Panchal, Sunil K; Poudyal, Hemant; Brown, Lindsay

    2012-06-01

    Metabolic syndrome is a risk factor for cardiovascular disease and nonalcoholic fatty liver disease (NAFLD). We investigated the responses to the flavonol, quercetin, in male Wistar rats (8-9 wk old) divided into 4 groups. Two groups were given either a corn starch-rich (C) or high-carbohydrate, high-fat (H) diet for 16 wk; the remaining 2 groups were given either a C or H diet for 8 wk followed by supplementation with 0.8 g/kg quercetin in the food for the following 8 wk (CQ and HQ, respectively). The H diet contained ~68% carbohydrates, mainly as fructose and sucrose, and ~24% fat from beef tallow; the C diet contained ~68% carbohydrates as polysaccharides and ~0.7% fat. Compared with the C rats, the H rats had greater body weight and abdominal obesity, dyslipidemia, higher systolic blood pressure, impaired glucose tolerance, cardiovascular remodeling, and NAFLD. The H rats had lower protein expressions of nuclear factor (erythroid-derived 2)-related factor-2 (Nrf2), heme oxygenase-1 (HO-1), and carnitine palmitoyltransferase 1 (CPT1) with greater expression of NF-κB in both the heart and the liver and less expression of caspase-3 in the liver than in C rats. HQ rats had higher expression of Nrf2, HO-1, and CPT1 and lower expression of NF-κB than H rats in both the heart and the liver. HQ rats had less abdominal fat and lower systolic blood pressure along with attenuation of changes in structure and function of the heart and the liver compared with H rats, although body weight and dyslipidemia did not differ between the H and HQ rats. Thus, quercetin treatment attenuated most of the symptoms of metabolic syndrome, including abdominal obesity, cardiovascular remodeling, and NAFLD, with the most likely mechanisms being decreases in oxidative stress and inflammation.

  4. A fulminant case of Guillain-Barré syndrome: topographic and fibre size related analysis of demyelinating changes.

    OpenAIRE

    Kanda, T; Hayashi, H.; H. Tanabe; Tsubaki, T; Oda, M.

    1989-01-01

    In a necropsy case of early fulminant Guillain-Barré syndrome, demyelinating changes were observed throughout the peripheral nervous system, most conspicuous in the spinal nerve roots. The central/peripheral nervous system transition regions and most proximal zones of the roots escaped damage. In some root areas there were widespread early changes in myelin sheaths in the absence of significant infiltrates of inflammatory cells. In the fibre size analytical study, small myelinated fibres were...

  5. Myopathological features in MELAS syndrome without significant changes in muscle biopsy pathology

    Institute of Scientific and Technical Information of China (English)

    Pu Fang; Chengsi Wu; Meihong Zhou; Renshi Xu; Xianhua Liu; Caixia Wei

    2010-01-01

    Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) are common types of mitochondrial encephalomyopathy. The involved muscular pathology is characterized by typical changes of mitochondrial abnormalities. Gene screening has been the gold diagnostic standard for MELAS diagnosis. This study presents three primary MELAS patients, with an age of onset from 13 to 18 years, including one patient with seizure, and two with headache and vomiting.All patients had a family history of disease, with matermal inheritance. Cerebral magnetic resonance imaging revealed abnormally high signals in T2-weighted images: temporal lobe in three cases,occipital lobe in two cases, and parietal lobe in one case. Migrating stroke-like lesions were confirmed in one patient. Muscle biopsy revealed several strongly succinate dehydrogenase-reactive vessels scattered in muscle sections of three patients, but ragged-red fibers and cytochrome c oxidase-negative/dense (COX-/+) fibers were not observed. Mitochondrial DNA A3243G mutation was identified in all three cases. MELAS syndrome has obvious clinical heterogeneity, and muscle weakness was not prominent in some of the cases. Muscle pathological changes did not accompany ragged-red fibers or COX-/+ fibers, but succinate dehydrogenasereactive vessels are important for MELAS diagnosis.

  6. Pseudomyopathic Changes in Needle Electromyography in Lambert-Eaton Myasthenic Syndrome

    Directory of Open Access Journals (Sweden)

    Teppei Komatsu

    2013-01-01

    Full Text Available Lambert-Eaton myasthenic syndrome (LEMS is a rare presynaptic disorder of the neuromuscular junction in association with cancer and subsequently in cases in which no neoplasm has been detected (O’Neill et al., 1988. The diagnosis of LEMS is based on the combination of fluctuating muscle weakness, diminished or absent reflexes, and a more than 60% increment of compound muscle action potential (CMAP amplitude after brief exercise or 50 Hz stimulation for 1 s in a repetitive nerve stimulation (RNS test (Oh et al., 2005. On the other hand, needle electromyography (EMG findings related to LEMS have not been well described. Here, we report a case of LEMS, which showed apparent myopathic changes in needle EMG findings. Furthermore, we retrospectively examined the needle EMG findings in 8 patients with LEMS. In six of the 8 patients, the EMG findings showed myopathy-like findings. Although the findings of needle EMG indicated myopathic changes at a glance, the motor unit potential (MUP returned to normal after a sustained strong muscle contraction. We propose the name “pseudomyopathic changes” for this phenomenon.

  7. Cell biology and genetics of minimal change disease [version 1; referees: 2 approved

    Directory of Open Access Journals (Sweden)

    Moin A. Saleem

    2016-03-01

    Full Text Available Minimal change disease (MCD is an important cause of nephrotic syndrome and is characterized by massive proteinuria and hypoalbuminemia, resulting in edema and hypercholesterolemia. The podocyte plays a key role in filtration and its disruption results in a dramatic loss of function leading to proteinuria. Immunologic disturbance has been suggested in the pathogenesis of MCD. Because of its clinical features, such as recurrent relapse/remission course, steroid response in most patients, and rare familial cases, a genetic defect has been thought to be less likely in MCD. Recent progress in whole-exome sequencing reveals pathogenic mutations in familial cases in steroid-sensitive nephrotic syndrome (SSNS and sheds light on possible mechanisms and key molecules in podocytes in MCD. On the other hand, in the majority of cases, the existence of circulating permeability factors has been implicated along with T lymphocyte dysfunction. Observations of benefit with rituximab added B cell involvement to the disease. Animal models are unsatisfactory, and the humanized mouse may be a good model that well reflects MCD pathophysiology to investigate suggested “T cell dysfunction” directly related to podocytes in vivo. Several candidate circulating factors and their effects on podocytes have been proposed but are still not sufficient to explain whole mechanisms and clinical features in MCD. Another circulating factor disease is focal segmental glomerulosclerosis (FSGS, and it is not clear if this is a distinct entity, or on the same spectrum, implicating the same circulating factor(s. These patients are mostly steroid resistant and often have a rapid relapse after transplantation. In clinical practice, predicting relapse or disease activity and response to steroids is important and is an area where novel biomarkers can be developed based on our growing knowledge of podocyte signaling pathways. In this review, we discuss recent findings in genetics and

  8. Membranous nephropathy and lupus-like syndrome after hematopoietic cell transplantation: a case report

    Directory of Open Access Journals (Sweden)

    Stylianou Kostas

    2010-09-01

    Full Text Available Abstract Introduction The kidney is increasingly recognised as a target organ of chronic graft-versus-host disease after hematopoietic cell transplantation in the context of the development of the nephrotic syndrome. Chronic graft-versus-host disease is associated with autoimmune phenomena similar, but not identical, to those observed in various rheumatologic disorders, implicating autoimmunity as an important component of the disease. Case presentation We report the case of a 57-year-old Caucasian man who developed the nephrotic syndrome due to membranous nephropathy in association with recurrent chronic graft-versus-host disease, along with a lupus-like syndrome manifested with pancytopenia, hair loss, positive anti-DNA antibodies and sub-epithelial and mesangial immune deposits. To the best of our knowledge, this is the first case reported in the literature. The nephrotic syndrome subsided soon after he was treated with a short course of cyclosporin with steroids. Unfortunately he died seven months later due to a relapse of leukemia. Conclusions Our case report confirms the notion that chronic graft-versus-host disease is characterized by the appearance of autoimmune phenomena similar, but not identical, to those seen in autoimmune diseases. The decision for more immunosuppression has to be weighed against the need for preservation of the graft versus leukemia phenomenon.

  9. Longitudinal Changes in Liver Aminotransferases Predict Metabolic Syndrome in Chinese Patients with Nonviral Hepatitis

    Institute of Scientific and Technical Information of China (English)

    CHEN Qi Cai; XIAO Juan; ZHANG Peng Peng; CHEN Li Li; CHEN Xiao Xiao; WANG Shu Mei

    2016-01-01

    ObjectiveThis study exploredthe correlation of longitudinal changes in serumalanine aminotransferase (ALT) and aspartate aminotransferase (AST)levels with the incidence of metabolic syndrome (Mets)based on a dynamic health examination cohort. MethodsA Mets-free dynamic cohortinvolving 4541 participants who underwent at leastthree health examinations from 2006 to 2011 was included in the study. Mets was defined according to the Chinese Medical Association Diabetes Branch definitionthat included hypertension, obesity, hyperlipidemia, and hyperglycemia. Generalized estimating equation (GEE) model was used to analyze multivariate relative risk (RR) of repeated observations ofALT and AST in quartiles for Mets or its components according to gender. ResultsIn all, 826Mets cases were reported. Adjustmentof relevant parameters indicated that time-varyingchanges in ALT and ASTlevels were positively associated with the incidenceof Mets in a dose-response manner. Positive association between high ALT levels and fatty liver was much stronger than that between high AST levels and fatty liver, particularly in maleparticipants. These associations were consistently observed in the following subgroups: participants with ALT and ASTlevels of ConclusionThese results suggested that elevated serum ALT and AST levels wereearly biomarkers of Mets or its components.

  10. Redox Imbalance and Morphological Changes in Skin Fibroblasts in Typical Rett Syndrome

    Directory of Open Access Journals (Sweden)

    Cinzia Signorini

    2014-01-01

    Full Text Available Evidence of oxidative stress has been reported in the blood of patients with Rett syndrome (RTT, a neurodevelopmental disorder mainly caused by mutations in the gene encoding the Methyl-CpG-binding protein 2. Little is known regarding the redox status in RTT cellular systems and its relationship with the morphological phenotype. In RTT patients (n = 16 we investigated four different oxidative stress markers, F2-Isoprostanes (F2-IsoPs, F4-Neuroprostanes (F4-NeuroPs, nonprotein bound iron (NPBI, and (4-HNE PAs, and glutathione in one of the most accessible cells, that is, skin fibroblasts, and searched for possible changes in cellular/intracellular structure and qualitative modifications of synthesized collagen. Significantly increased F4-NeuroPs (12-folds, F2-IsoPs (7.5-folds NPBI (2.3-folds, 4-HNE PAs (1.48-folds, and GSSG (1.44-folds were detected, with significantly decreased GSH (−43.6% and GSH/GSSG ratio (−3.05 folds. A marked dilation of the rough endoplasmic reticulum cisternae, associated with several cytoplasmic multilamellar bodies, was detectable in RTT fibroblasts. Colocalization of collagen I and collagen III, as well as the percentage of type I collagen as derived by semiquantitative immunofluorescence staining analyses, appears to be significantly reduced in RTT cells. Our findings indicate the presence of a redox imbalance and previously unrecognized morphological skin fibroblast abnormalities in RTT patients.

  11. Morphological changes of an inflammatory myopathy in rhesus monkeys with simian acquired immunodeficiency syndrome.

    Science.gov (United States)

    Dalakas, M C; Gravell, M; London, W T; Cunningham, G; Sever, J L

    1987-09-01

    Eleven of 25 rhesus monkeys which died of simian acquired immunodeficiency syndrome (SAIDS) caused by infection with a type D retrovirus related to Mason-Pfizer monkey virus showed evidence of muscle weakness and atrophy and had elevated levels of muscle enzymes. Biopsies of affected muscle studied with enzyme histochemistry showed the characteristic features of polymyositis. Inflammatory cells consisting of lymphocytes, macrophages, and large vacuolated bizarre-shaped cells of undetermined type were surrounding or invading muscle fibers and were present in the perivascular spaces and endomysia septa. Within the perivascular infiltrates, lymphocytes were abundant but very few macrophages were present. Other myopathic features including profound proliferation of fibrous tissue, necrosis, and phagocytosis of muscle fibers were noted to a variable degree. The retrovirus was isolated from affected muscles. The clinical and historical features of polymyositis in rhesus monkeys with SAIDS are very similar to those of human polymyositis. The polymyositis in SAIDS induced by a type D retrovirus related to Mason-Pfizer monkey virus is an excellent primate model to study the mechanism and morphological changes of viral-induced muscle damage.

  12. Histopathologic changes of hypothalamus and pituitary in a rat model of polycystic ovary syndrome

    Institute of Scientific and Technical Information of China (English)

    Jiang Yan; Meng Fan-yu; Hu Zhen-hua; Liu Fang

    2010-01-01

    Objective: To investigate the histopathologic changes of hypothalamus and pituitary in a rat model of polycystic ovary syndrome (PCOS).Methods: Rat model of PCOS was established in 6 immature female SD rats and another 6 immature rats treated with placebo were as control. The tissues of hypothalamus and pituitary were obtained and observed by light microscope and transmission electron microscope.Results: Light microscopy revealed little difference in morphology of neurons in arcuate nucleus or basophilic cells in pituitary between PCOS rats and normal rats. Electron microscopic examination showed that, compared with those in normal rats, GnRH neurons in PCOS rats were larger and fuller, with Golgi complex and mitochondria increased. The mitochondria were small, round and swelling. More high-density secretory granules and bright vesicles were observed in the cytoplasm. The Golgi complex near nucleus in pituitary gonadotropin cell in PCOS rats was fractured and expanded, and there were increased number mitochondria and different sizes of the higher electron density secretory granules in the cytoplasm. Conclusion: The morphological alterations in hypothalamus and pituitary could play a very important role in the development of PCOS.

  13. Looking for sufficient change: Evaluation of counsellor training for STI syndromic management in India.

    Science.gov (United States)

    Vaz, Melita; Kadyan, Nisha; Chalil, Sumitha; Prasad, Turlapati L N; Singh, Aman Kumar

    2016-10-01

    In India, public health care of Sexually Transmitted Infections is delivered through Designated STI/RTI Clinics (DSRCs) using syndromic management. This paper describes efforts, over three years, to improve in-service training for counsellors positioned at DSRCs-using a data approach. The programme managers realised, through rigorous monitoring of initial induction training reports that, while knowledge and attitudes of most trainees had improved as evident from t-tests, at least one-quarter scored worse on post-training assessments (n=859). Therefore, they undertook a survey using a competency approach to diagnose what critical competencies are influenced through training: counselling skills, risk reduction suggestions, labelling male and female anatomy, record-keeping and STI patient education (n=132). Survey results demonstrated that trainees failed to pass a two-thirds cutoff score in most competencies. These findings led the programme managers to modify training and implement tighter quality measures. In the second round of training - refresher training - outcomes on competency assessments before and after training showed more acceptable performance (n=833). The paper describes how programme managers, after an acceptance of such initial short-comings, developed customized assessments when literature provided limited guidance and how they worked to achieve change that was acceptable for programme needs. PMID:27372031

  14. Infection and T lymphocyte subpopulations: changes associated with bacteremia and the acquired immunodeficiency syndrome.

    Science.gov (United States)

    Fishman, J A; Martell, K M; Rubin, R H

    1983-01-01

    Patients with bacteremia, bacterial endocarditis, or acquired immunodeficiency syndrome (AIDS) were prospectively studied using monoclonal antibody reagents to assess alterations in T-lymphocyte subpopulations. Patients with endocarditis had significantly higher ratios of T-helper (OKT4+) to T-suppressor-cytotoxic (OKT8+) cells than did patients with bacteremia alone. Staphylococcus aureus endocarditis patients had a mean ratio of 8.49 (range 4.73-22.36) while S aureus bacteremia had a mean ratio of 2.75 (range 2.15 to 3.21). Similar results were found with Staphylococcus epidermidis endocarditis (mean 1.62) and bacteremia (mean 1.23). Klebsiella pneumoniae endocarditis (5.10) and sepsis (4.32), and E coli bacteremia (2.15). Nine male patients with AIDS had markedly depressed ratios (mean 0.25, range 0.04 to 0.67) while eight male homosexuals with unexplained lymphadenopathy ("pre-AIDS") had normal or increased ratios. Bacteremic infections are associated with an increased OKT4+/OKT8+ ratio with the degree of increase dependent upon virulence, location, and duration of infection. The immunomodulating effects of infection are manifested in changes in T-cell subsets, and these measurements can be useful in clinical management. PMID:6094086

  15. Biliary Cast Syndrome: Hepatic Artery Resistance Index, Pathological Changes, Morphology and Endoscopic Therapy

    Directory of Open Access Journals (Sweden)

    Hu Tian

    2015-01-01

    Full Text Available Background: Biliary cast syndrome (BCS was a postoperative complication of orthotopic liver transplantation (OLT, and the reason for BSC was considered to relate with ischemic type biliary lesions. This study aimed to evaluate the relationship between BCS following OLT and the hepatic artery resistance index (HARI, and to observe pathological changes and morphology of biliary casts. Methods: Totally, 18 patients were diagnosed with BCS by cholangiography following OLT using choledochoscope or endoscopic retrograde cholangiopancreatography. In addition, 36 patients who did not present with BCS in the corresponding period had detectable postoperative HARI on weeks 1, 2, 3 shown by color Doppler flow imaging. The compositions of biliary casts were analyzed by pathological examination and scanning electron microscopy. Results: HARI values of the BCS group were significantly decreased as compared with the non-BCS group on postoperative weeks 2 and 3 (P 1 (OR = 1.300; 1.223; and 1.889, respectively. The OR of HARI 3 was statistically significant (OR = 1.889; 95% confidence interval = 1.166-7.490; P = 0.024. The compositions of biliary casts were different when bile duct stones were present. Furthermore, vascular epithelial cells were found by pathological examination in biliary casts. Conclusions: HARI may possibly serve as an independent risk factor and early predictive factor of BCS. Components and formation of biliary casts and bile duct stones are different.

  16. Age and body mass related changes of cardiovascular risk factors in women with polycystic ovary syndrome

    Directory of Open Access Journals (Sweden)

    Macut Đuro P.

    2002-01-01

    Full Text Available Polycystic ovary syndrome (PCOS is considered a metabolic disorder closely related to obesity, insulin resistance (IR, hyperinsulinemia and unfavorable lipid profile, all increasing the risk for the occurrence of cardiovascular diseases. The aim of this study was to assess age and body mass index (BMI related changes of cardiovascular risk factors in 90 women with PCOS. The cut-off age point was 30 years and for BMI 27.8 kg/m2. In all patients systolic and diastolic blood pressure (BP, metabolic parameters comprising values of glucose and insulin during oral glucose tolerance test (OGTT, and basal lipid values were determined. Significant increase in blood pressure (BP indices, basal insulin values and insulin resistance (IR assessed by HOMA model were observed with aging and the increase of BMI, while the parameters of glucose metabolism, total cholesterol and triglycerides were significantly elevated only with aging. However, the correlation between the indices of arterial blood pressure, and lipid and glucose metabolism parameters occurred only in patients over 30 years of age, pointing to the causative relation and the consequent deterioration of IR and lipid profile with aging, influencing cardiovascular function in women with PCOS.

  17. Association of global weather changes with acute coronary syndromes: gaining insights from clinical trials data

    Science.gov (United States)

    Bakal, Jeffrey A.; Ezekowitz, Justin A.; Westerhout, Cynthia M.; Boersma, Eric; Armstrong, Paul W.

    2013-05-01

    The aim of this study was to develop a method for the identification of global weather parameters and patient characteristics associated with a type of heart attack in which there is a sudden partial blockage of a coronary artery. This type of heart attack does not demonstrate an elevation of the ST segment on an electrocardiogram and is defined as a non-ST elevation acute coronary syndrome (NSTE-ACS). Data from the Global Summary of the Day database was linked with the enrollment and baseline data for a phase III international clinical trial in NSTE-ACS in four 48-h time periods covering the week prior to the clinical event that prompted enrollment in the study. Meteorological events were determined by standardizing the weather data from enrollment dates against an empirical distribution from the month prior. These meteorological events were then linked to the patients' geographic region, demographics and comorbidities to identify potential susceptible populations. After standardization, changes in temperature and humidity demonstrated an association with the enrollment event. Additionally there appeared to be an association with gender, region and a history of stroke. This methodology may provide a useful global insight into assessing the biometeorologic component of diseases from international data.

  18. Hematological Changes m White Spot Syndrome Virus-Infected Shrimp, Fenneropenaeus chinensis (Osbeck)

    Institute of Scientific and Technical Information of China (English)

    FENG Shouming; ZHAN Wenbin; XING Jing; LI Jun; YANG Kai; WANG Jing

    2008-01-01

    The pathological changes of hemocytes in the haemolymph and hepatopancreas were examined in experimentally and naturally WSSV (white spot syndrome virus) infected Fenneropenaeus chinensis. The results showed that the pathological manifesta- tions of hemocytes were similar among moribund shrimps infected via injection, feeding and by nature. Firstly, the total hemocyte counts (THCs) in WSSV-infected shrimp were significantly lower than those in healthy shrimp. Secondly, necrotic, broken and dis- integrated cells were often observed, and a typical hematolysis was present in the haemolymph smear of WSSV-infected shrimp. Thirdly, necrosis and typical apoptosis of hemocytes were detected with TEM in the peripheral haemolymph of WSSV-infected shrimp. Hyalinocytes and semi-granulocytes with masses of WSSVs in their nuclei often appeared, whereas no granular bemocytes with WSSV were found in the hepatopancreas of moribund infected shrimps. All our results supported that hemocytes were the main target cells of WSSV, and hyalinocytes and semigranular hemocytes seemed to be more favorable for WSSV infection in F. chinensis.

  19. Local cytokine changes in complex regional pain syndrome type I (CRPS I) resolve after 6 months.

    Science.gov (United States)

    Lenz, Melanie; Uçeyler, Nurcan; Frettlöh, Jule; Höffken, Oliver; Krumova, Elena K; Lissek, Silke; Reinersmann, Annika; Sommer, Claudia; Stude, Philipp; Waaga-Gasser, Ana M; Tegenthoff, Martin; Maier, Christoph

    2013-10-01

    There is evidence that inflammatory processes are involved in at least the early phase of complex regional pain syndrome (CRPS). We compared a panel of pro- and antiinflammatory cytokines in skin blister fluids and serum from patients with CRPS and patients with upper-limb pain of other origin (non-CRPS) in the early stage (< 1 year) and after 6 months of pain treatment. Blister fluid was collected from the affected and contralateral nonaffected side. We used a multiplex-10 bead array cytokine assay and Luminex technology to measure protein concentrations of the cytokines interleukin-1 receptor antagonist (IL-1RA), IL-2, IL-6, IL-8, IL-10, IL-12p40, and tumor necrosis factor-alpha (TNF-α) and the chemokines eotaxin, monocyte chemotactic protein-1 (MCP-1), and macrophage inflammatory protein-1β (MIP-1β). We found bilaterally increased proinflammatory TNF-α and MIP-1β and decreased antiinflammatory IL-1RA protein levels in CRPS patients compared to non-CRPS patients. Neither group showed side differences. After 6 months under analgesic treatment, protein levels of all measured cytokines in CRPS patients, except for IL-6, significantly changed bilaterally to the level of non-CRPS patients. These changes were not related to treatment outcome. In serum, only IL-8, TNF-α, eotaxin, MCP-1, and MIP-1β were detectable without intergroup differences. Blister fluid of CRPS patients showed a bilateral proinflammatory cytokine profile. This profile seems to be relevant only at the early stage of CRPS. Almost all measured cytokine levels were comparable to those of non-CRPS patients after 6 months of analgesic treatment and were not related to treatment outcome.

  20. Changes of Indocyanine Green and Fluorescein Angiography in Multiple Evanescent White-dot Syndrome: A Case Report

    Institute of Scientific and Technical Information of China (English)

    Changxian Yi; Guilin Zhao; Jiexiong Ou; Hong Yan

    2003-01-01

    Purpose: To study the clinicopathological change on "Multiple Evanescent White-dot Syndrome (MEWDS)"through fundus angiogrphy analyses.Methods:Examing the case with fluorescein angiography (FFA) and indocyanine green angiography(ICGA), visual field and following up for 15 months.Results :There exist multiple white dots in the fundus photo and angiography. The changes on ICGA maintain longer than fundus photo and FFA. Papille may be edematous and macula could present with special hypofluorescent flecks.Conclusions:MEWDS has specific angiographic features. Its pathological changes involve deep retinal pigment epithelium(RPE) as well as choroidal capillaries. The prognosis of the visual acuity for MEWDS is very good.

  1. Changes in Angiotensin Receptor Distribution and in Aortic Morphology Are Associated with Blood Pressure Control in Aged Metabolic Syndrome Rats

    OpenAIRE

    Verónica Guarner-Lans; Elizabeth Soria-Castro; Rocío Torrico-Lavayen; Araceli Patrón-Soberano; Karla G. Carvajal-Aguilera; Vicente Castrejón-Tellez; María Esther Rubio-Ruiz

    2016-01-01

    The role of the renin-angiotensin system (RAS) in blood pressure regulation in MS during aging is unknown. It participates in metabolic syndrome (MS) and aging regulating vascular tone and remodeling. RAS might participate in a compensatory mechanism decreasing blood pressure and allowing MS rats to reach 18 months of age and it might form part of therapeutical procedures to ameliorate MS. We studied histological changes and distribution of RAS receptors in aortas of MS aged rats. Electron mi...

  2. Changes in androgens and insulin sensitivity indexes throughout pregnancy in women with polycystic ovary syndrome (PCOS): relationships with adverse outcomes

    OpenAIRE

    Falbo Angela; Rocca Morena; Russo Tiziana; D'Ettore Antonietta; Tolino Achille; Zullo Fulvio; Orio Francesco; Palomba Stefano

    2010-01-01

    Abstract Background Given the high rate of pregnancy and perinatal complications recently observed in patients with polycystic ovary syndrome (PCOS) and the lack of data on the serum variations in androgens and insulin sensitivity indexes in pregnant women with PCOS, the current study was aimed to assess these changes and their potential effect on pregnancy outcomes in a population of women with PCOS. Methods Forty-five pregnant patients with ovulatory PCOS (PCOS group) and other 42 healthy p...

  3. Changes in Enteric Neurons of Small Intestine in a Rat Model of Irritable Bowel Syndrome with Diarrhea

    OpenAIRE

    Li, Shan; Fei, Guijun; Fang, Xiucai; Yang, Xilin; Sun, Xiaohong; Qian, Jiaming; Wood, Jackie D.; Ke, Meiyun

    2016-01-01

    Background/Aims Physical and/or emotional stresses are important factors in the exacerbation of symptoms in irritable bowel syndrome (IBS). Several lines of evidence support that a major impact of stress on the gastrointestinal tract occurs via the enteric nervous system. We aimed to evaluate histological changes in the submucosal plexus (SMP) and myenteric plexus (MP) of the distal ileum in concert with the intestinal motor function in a rat model of IBS with diarrhea. Methods The rat model ...

  4. Changes of atrial natriuretic peptide and antidiuretic hormone in children with postural tachycardia syndrome and orthostatic hypertension: a case control study

    Institute of Scientific and Technical Information of China (English)

    Zhao Juan; Yang Jinyan; Du Shuxu; Tang Chaoshu; Du Junbao; Jin Hongfang

    2014-01-01

    Background The abnormal blood volume regulation is one of the most important pathogenesis in postural tachycardia syndrome in children.This study was designed to investigate the plasma atrial natriuretic peptide and antidiuretic hormone levels in postural tachycardia syndrome children,and their associations with the changes in heart rate and blood pressure in head-up test.Methods Twenty-one postural tachycardia syndrome patients ((12±2) years) and 26 healthy children ((12±1) years) were included.According to blood pressure changes in head-up test,the postural tachycardia syndrome patients were divided into two subgroups:postural tachycardia syndrome with orthostatic hypertension and postural tachycardia syndrome without orthostatic hypertension.The plasma atrial natriuretic peptide and antidiuretic hormone levels were measured using enzyme-linked immunosorbent assay.Results The plasma atrial natriuretic peptide level in postural tachycardia syndrome patients was higher than the control (P=0.004),whereas the difference in plasma antidiuretic hormone level between postural tachycardia syndrome and controls was not significant (P=0.222).The plasma antidiuretic hormone level of patients suffering from postural tachycardia syndrome with orthostatic hypertension was much higher than that of children having postural tachycardia syndrome without orthostatic hypertension (P <0.05).In postural tachycardia syndrome patients,the updght max heart rate was positively correlated with the plasma atrial natriuretic peptide level (r=0.490,P<0.05) and the upright systolic blood pressure was positively correlated with the plasma antidiuretic hormone levels (r=0.472,P <0.05).Conclusions There was a disturbance of plasma atrial natriuretic peptide and antidiuretic hormone in postural tachycardia syndrome children.

  5. Equine Assisted Therapy and Changes in Gait for a Young Adult Female with Down Syndrome

    OpenAIRE

    Katherine J. Coffey; Adam C. Knight; Benjamin Wax

    2015-01-01

    The purpose of this study was to examine the effects of equine assisted therapy on selected gait parameters in a person with Down syndrome. One female participant with Down syndrome completed two therapeutic horseback riding programs, each consisting of six riding sessions. Specific gait characteristics were analyzed with a trend analysis of the data by examining the means of the different variables. The trend analysis revealed a difference in stride length as well as hip and knee angle. Thes...

  6. Change of International Restless Legs Syndrome Study Group Rating Scale subscales with treatment and placebo: a pilot study

    Directory of Open Access Journals (Sweden)

    Mitchell UH

    2014-02-01

    Full Text Available Ulrike H Mitchell,1 Sterling C Hilton2 1Brigham Young University, Department of Exercise Sciences, 2Department of Educational Leadership and Foundations, Provo, UT, USA Background: In 2003, the 10-question International Restless Legs Syndrome Study Group Rating Scale (IRLS was developed as a means of assessing the severity of restless legs syndrome. Two subscales were identified: symptom severity (SS 1 and symptom impact (SS 2. Only one study has investigated the subscales' responsiveness to a 12-week treatment with ropinirole. This current study was undertaken to assess the impact of a 4-week, non-pharmaceutical treatment on the two subscales and to explore whether or not both subscales were impacted by the observed placebo effect. Methods: The pooled data from questionnaires of 58 patients (41 from both treatment groups and 17 from the sham treatment control group, who participated in two clinical studies, were reviewed. Their change in score over a 4-week trial was computed. The average change in both subscales in both groups was computed and t-tests were performed. Results: In the treatment group, the average scores of both subscales changed significantly from baseline to week 4 (P<0.005 for both. Compared to the control, SS 1 changed (P<0.001, but not SS 2 (P=0.18. In the sham treatment group, the scores for SS 1 changed significantly (P=0.002, but not for SS 2 (P=0.2. Conclusion: This study corroborated findings from an earlier study in which both subscales changed with a 12-week drug treatment. It also showed that the observed placebo effect is attributed to a small but significant change in symptom severity, but not symptom impact. Keywords: restless legs syndrome, RLS severity scale, IRLS subscales, symptom impact, symptom severity

  7. Membranous nephropathy and lupus-like syndrome after hematopoietic cell transplantation: a case report

    OpenAIRE

    Stylianou Kostas; Stratakis Stavros; Mavroeidi Vasiliki; Petrakis Ioannis; Xydakis Dimitris; Vardaki Eleftheria; Stratigis Spyros; Perakis Kostas; Katsarou Theodora; Kanellou Peggy; Xylouri Irene; Petraki Constantina; Alexandrakis Michael; Daphnis Eugene

    2010-01-01

    Abstract Introduction The kidney is increasingly recognised as a target organ of chronic graft-versus-host disease after hematopoietic cell transplantation in the context of the development of the nephrotic syndrome. Chronic graft-versus-host disease is associated with autoimmune phenomena similar, but not identical, to those observed in various rheumatologic disorders, implicating autoimmunity as an important component of the disease. Case presentation We report the case of a 57-year-old Cau...

  8. NADPH oxidase 4 attenuates cerebral artery changes during the progression of Marfan syndrome.

    Science.gov (United States)

    Onetti, Yara; Meirelles, Thayna; Dantas, Ana P; Schröder, Katrin; Vila, Elisabet; Egea, Gustavo; Jiménez-Altayó, Francesc

    2016-05-01

    Marfan syndrome (MFS) is a connective tissue disorder that is often associated with the fibrillin-1 (Fbn1) gene mutation and characterized by cardiovascular alterations, predominantly ascending aortic aneurysms. Although neurovascular complications are uncommon in MFS, the improvement in Marfan patients' life expectancy is revealing other secondary alterations, potentially including neurovascular disorders. However, little is known about small-vessel pathophysiology in MFS. MFS is associated with hyperactivated transforming growth factor (TGF)-β signaling, which among numerous other downstream effectors, induces the NADPH oxidase 4 (Nox4) isoform of NADPH oxidase, a strong enzymatic source of H2O2 We hypothesized that MFS induces middle cerebral artery (MCA) alterations and that Nox4 contributes to them. MCA properties from 3-, 6-, or 9-mo-old Marfan (Fbn1(C1039G/+)) mice were compared with those from age/sex-matched wild-type littermates. At 6 mo, Marfan compared with wild-type mice developed higher MCA wall/lumen (wild-type: 0.081 ± 0.004; Marfan: 0.093 ± 0.002; 60 mmHg; P Marfan mice with Nox4 deficiency (Nox4(-/-)). Strikingly, Nox4 deletion in Marfan mice aggravated MCA wall thickening (cross-sectional area; Marfan: 6,660 ± 363 μm(2); Marfan Nox4(-/-): 8,795 ± 824 μm(2); 60 mmHg; P < 0.05), accompanied by decreased TGF-β expression and increased collagen deposition and Nox1 expression. These findings provide the first evidence that Nox4 mitigates cerebral artery structural changes in a murine model of MFS. PMID:26945079

  9. Morphological changes of carotid bodies in acute respiratory distress syndrome: a morphometric study in humans

    Directory of Open Access Journals (Sweden)

    Vinhaes E.N.G.

    2002-01-01

    Full Text Available Carotid bodies are chemoreceptors sensitive to a fall of partial oxygen pressure in blood (hypoxia. The morphological alterations of these organs in patients with chronic obstructive pulmonary disease (COPD and in people living at high altitude are well known. However, it is not known whether the histological profile of human carotid bodies is changed in acute clinical conditions such as acute respiratory distress syndrome (ARDS. The objective of the present study was to perform a quantitative analysis of the histology of carotid bodies collected from patients who died of ARDS. A morphometric study of carotid bodies collected during routine autopsies was carried out on three groups: patients that died of non-respiratory diseases (controls, N = 8, patients that presented COPD and died of its complications or associated diseases (N = 7, and patients that died of ARDS (N = 7. Morphometric measurements of the volume fraction of clusters of chief cells were performed in five fields on each slide at 40X magnification. The numerical proportion of the four main histological cell types (light, dark, progenitor and sustentacular cells was determined analyzing 10 fields on each slide at 400X magnification. The proportion of dark cells was 0.22 in ARDS patients, 0.12 in controls (P<0.001, and 0.08 in the COPD group. The proportion of light cells was 0.33 (ARDS, 0.44 (controls (P<0.001, and 0.36 (COPD. These findings suggest that chronic and acute hypoxia have different effects on the histology of glomic tissue.

  10. Changes in lung parenchyma after acute respiratory distress syndrome (ARDS): assessment with high-resolution computed tomography

    International Nuclear Information System (INIS)

    The aim of this study was to evaluate the appearance, extent, and distribution of parenchymal changes in the lung after acute respiratory distress syndrome (ARDS) as a function of disease severity and therapeutic procedures. High-resolution computed tomography (HRCT), clinical examination, and lung function tests were performed in 15 patients, 6-10 months after ARDS. The appearance and extent of parenchymal changes were compared with the severity of ARDS, as well as with clinical and therapeutic data. Lung parenchymal changes resembling those found in the presence of pulmonary fibrosis were observed in 13 of 15 patients (87%). The changes were significantly more frequent and more pronounced in the ventral than in the dorsal portions of the lung (p<0.01). A significant correlation was observed between the extent of lung alterations and the severity of ARDS (p<0.01), and the duration in which patients had received mechanical ventilation either with a peak inspiratory pressure greater than 30 mmHg (p<0.05), or with more than 70% oxygen (p<0.01). Acute respiratory distress syndrome frequently is followed by fibrotic changes in lung parenchyma. The predominantly ventral distribution of these changes indicates that they may be caused by the ventilation regimen and the oxygen therapy rather than by the ARDS. (orig.)

  11. Sensory changes,C-and A-fiber function,and shoulder-hand syndrome in hemiplegic patients after stroke

    Institute of Scientific and Technical Information of China (English)

    Yi Yuan; Xiaohong Zi; Xian Huang

    2008-01-01

    BACKGROUND:Clinical diagnosis of various neurological disorders involving the sensory nerves depends primarily on subjective description.which cannot be quantitatively evaluated,and is also less reproducible and specific.Quantitative sensory testing mcthods can overcome these shortcomings and is currently used to identify the function of the C-and A-fibers.OBJECTIVE:To apply the quantitative sensory testing method for analyzing changes in temperature sensation,cryalgesia,thermalgesia,and vibration sense on the skin surface of hemiplegic patients with post-stroke shoulder-hand syndrome,and to analyze the relationship between these changes and shoulder-hand syndrome.DESIGN,TIME AND SETTING:A non-randomized,concurrent,control study was performed at the Clinic and Inpatient Department of the Third Xiangya Hospital,Central South University,between June 2000 and April 2001.PARTICIPANTS:Thirty post-stroke,hemiplegic patients were divided into shoulder-hand syndrome and control groups,according to whether patients exhibited shoulder-hand syndrome,with 15 patients in each group.METHODS:A TSA2001 quantitative sensory testing device(Medoc,Israel)was used for quantitative sensory testing.All sensory testing employed limits,testing temperature sense on the palm thenar eminence and vibration sense on the thumb metacarpal.Cold threshold was≤28℃.warmth threshold was≥36℃,cold-evoked pain threshold was≤5℃.heat-evoked pain threshold was≥51℃,vibration threshold was≥5 μ m/s;if a patient met one of these items,he/she was considered to be hypoanesthesia.MAIN OUTCOME MEASURES:Cold,warm,cold-evoked pain,heat-evoked pain and vibration threshold changes on skin from the paralyzed upper extremity was measured in the shoulder-hand syndrome and control groups.RESULTS:Incidence of sensory disability in the shoulder-hand syndrome group increased more significantly than in the control group(P<0.05),with the primary manifestations being decreased cold threshold(P<0.05)and

  12. Intermittent Fever, Progressive Weight Gain, and Personality Changes in a Five-Year-Old Girl: Unusual Paraneoplastic Syndrome due to Presacral Ganglioneuroma

    Directory of Open Access Journals (Sweden)

    Chao Yang

    2016-01-01

    Full Text Available Ganglioneuromas are rare tumors in the neuroblastoma group. Paraneoplastic syndrome (PNS due to presacral ganglioneuromas was hardly reported in previous literature. Here, we reported that a case of a 5-year-old girl with a presacral ganglioneuroma presented with PNS, who presented with intermittent fever, progressive weight gain, and personality changes. Our report revealed intermittent fever, progressive weight gain, and personality changes may represent rare paraneoplastic syndromes in ganglioneuromas.

  13. Structural Changes in the Somatosensory System Correlate with Tic Severity in Gilles de la Tourette Syndrome

    Science.gov (United States)

    Thomalla, Gotz; Siebner, Hartwig R.; Jonas, Melanie; Baumer, Tobias; Biermann-Ruben, Katja; Hummel, Friedhelm; Gerloff, Christian; Muller-Vahl, Kirsten; Schnitzler, Alfons; Orth, Michael; Munchau, Alexander

    2009-01-01

    Gilles de la Tourette syndrome (GTS) is a neuropsychiatric disorder characterized by multiple motor and vocal tics. Previous structural MRI studies have identified regional abnormalities in grey matter, especially in the basal ganglia. These findings are consistent with the assumption of alterations in cortico-striato-thalamo-cortical circuits and…

  14. Pilot observational study on haemodynamic changes after surfactant administration in preterm newborns with respiratory distress syndrome

    OpenAIRE

    Vitali, Francesca; Galletti, Silvia; Aceti, Arianna; Aquilano, Giulia; Fabi, Marianna; Balducci, Anna; Faldella, Giacomo

    2014-01-01

    Background Surfactant treatment reduces respiratory morbidity and mortality in preterm infants. Data on its haemodynamic consequences are conflicting. The aim was to characterise the haemodynamic effects of surfactant treatment on cardiac function in preterm newborns with respiratory distress syndrome (RDS). Methods Preterm infants (gestational age

  15. Ultrastructural changes in fibroblast mitochondria of a patient with HHH-syndrome.

    Science.gov (United States)

    Metoki, K; Hommes, F A; Dyken, P; Kelloes, C; Trefz, J

    1984-01-01

    Electron micrographs of fibroblasts of an HHH-syndrome patient showed abnormal structures, similar, but not identical, to those observed in the liver of such patients. It is suggested that incorporation of a mutated protein into the inner mitochondrial membrane gives rise to a rearrangement of that membrane, resulting in unusual structures.

  16. Activity-Dependent Changes in MAPK Activation in the Angelman Syndrome Mouse Model

    Science.gov (United States)

    Filonova, Irina; Trotter, Justin H.; Banko, Jessica L.; Weeber, Edwin J.

    2014-01-01

    Angelman Syndrome (AS) is a devastating neurological disorder caused by disruption of the maternal "UBE3A" gene. Ube3a protein is identified as an E3 ubiquitin ligase that shows neuron-specific imprinting. Despite extensive research evaluating the localization and basal expression profiles of Ube3a in mouse models, the molecular…

  17. Temporal changes in serum creatine kinase concentration and degree of muscle rigidity in 24 patients with neuroleptic malignant syndrome

    Directory of Open Access Journals (Sweden)

    Nisijima K

    2013-06-01

    Full Text Available Koichi Nisijima, Katutoshi ShiodaDepartment of Psychiatry, Jichi Medical University, Tochigi, JapanAbstract: Neuroleptic malignant syndrome (NMS is a dangerous adverse response to antipsychotic drugs. It is characterized by the four major clinical symptoms of hyperthermia, severe muscle rigidity, autonomic dysfunction, and altered mental state. Serum creatine kinase (CK elevation occurs in over 90% of NMS cases. In the present study, the detailed temporal changes in serum CK and degree of muscle rigidity, and the relationship between CK concentration and degree of muscle rigidity over the time course from fever onset, were evaluated in 24 affected patients. The results showed that serum CK peaked on day 2 after onset of fever and returned to within normal limits at day 12. Mild muscle rigidity was observed before the onset of fever in 17 of 24 cases (71%. Muscle rigidity was gradually exacerbated and worsened until day 4 after onset of fever. These findings confirm physicians' empirical understanding of serum CK concentrations and muscle rigidity in NMS based on data accumulated from numerous patients with the syndrome, and they indicate that serum CK may contribute to the early detection of NMS.Keywords: neuroleptic malignant syndrome, creatine kinase, muscle rigidity

  18. Metformin Lowers Serum Cobalamin without Changing Other Markers of Cobalamin Status: A Study on Women with Polycystic Ovary Syndrome

    DEFF Research Database (Denmark)

    Greibe, Eva; Trolle, G. Birgitta; Bor, Mustafa Vakur;

    2013-01-01

    Treatment with the anti-diabetic drug metformin is followed by a decline in plasma cobalamin, but it is unsettled whether this denotes an impaired cobalamin status. This study has explored changes in the markers of cobalamin status in women with Polycystic Ovary Syndrome treated with metformin (1...... have two implications: The data questions whether metformin treatment induces an impaired cobalamin status in PCOS patients, and further suggests that serum cobalamin is a futile marker for judging cobalamin status in metformin-treated patients....

  19. Analysis of clinic and osteal change in Turner syndrome (report of 15 cases)

    International Nuclear Information System (INIS)

    Objective: To research the clinical and osteal radiology imaging features in Turner's syndrome. Methods: Analyzed the clinical and osteal radiology imaging features of 15 Turner's syndrome. Results: 15 patients were Karyotyped into 3 groups I, 45, X, n=8; II, 46, XX, n=5; III, 46, XX/45, X, n = 2. Radiology imaging features: osteo-porosis, n=14; metacarpal sign, n=8; phahanx, n=9; cubitus valgus, n=10; vertebra steodystrophia, n=5; brachy-dactylya, n=9; basilar impression malformation, n=11. Conclusion: The terminal heights of Turner' s patients were marked lower than normal growth, hormone was deficiency, learning ability declined and the sex gland was hypoplastic. The typical imaging feature do main reference value to diagnosis. (authors)

  20. Reproductive and biochemical changes in obese and non obese polycystic ovary syndrome women

    OpenAIRE

    Manal Ibrahim Mahmoud; Fawzia Habeeb; Khaled Kasim

    2015-01-01

    Background: Reproductive, clinical and laboratory implication varies in polycystic ovary syndrome (PCOS) according to body weight. Objective: To compare reproductive, clinical and laboratory data between obese and non obese women with PCOS. Methods: A cohort of 180 women with PCOS who attended outpatient clinic of Taibah University from January to September 2012 was included. Studied women were classified according to body mass index (BMI) into overweight/obese (BMI >25 kg/m2) and norma...

  1. [Refeeding syndrome].

    Science.gov (United States)

    Ševela, Stanislav; Novák, František; Kazda, Antonín; Brodská, Helena

    2016-01-01

    Despite being known more than 60 years, refeeding syndrome (RS) still bears many uncertainties. For example, its definition is not clear and definite, and the attitude to it varies from the complete neglect to over-prevention.The term "refeeding syndrome" refers to electrolyte and metabolic changes occurring in malnourished patients after the readministration of nutrition. These changes concern especially to phosphates and ions. Potassium, magnesium, naturism and fluids balance are involved. The changes lead to cell energetic metabolism and electric potential disturbances, with related clinical symptoms.Fully developed refeeding syndrome is quite rare; nevertheless it can be fatal for the patient. However, even its development can lead to many complications increasing the patient's morbidity and the length of stay in the hospital. Yet the refeeding syndrome is more or less predictable and if kept in mind also preventable.The aim of this article is to get the reader to know more about this metabolic phenomenon and possible attitudes towards it.

  2. From armchair to wheelchair: how patients with a locked-in syndrome integrate bodily changes in experienced identity.

    Science.gov (United States)

    Nizzi, Marie-Christine; Demertzi, Athena; Gosseries, Olivia; Bruno, Marie-Aurélie; Jouen, François; Laureys, Steven

    2012-03-01

    Different sort of people are interested in personal identity. Philosophers frequently ask what it takes to remain oneself. Caregivers imagine their patients' experience. But both philosophers and caregivers think from the armchair: they can only make assumptions about what it would be like to wake up with massive bodily changes. Patients with a locked-in syndrome (LIS) suffer a full body paralysis without cognitive impairment. They can tell us what it is like. Forty-four chronic LIS patients and 20 age-matched healthy medical professionals answered a 15-items questionnaire targeting: (A) global evaluation of identity, (B) body representation and (C) experienced meaning in life. In patients, self-reported identity was correlated with B and C. Patients differed with controls in C. These results suggest that the paralyzed body remains a strong component of patients' experienced identity, that patients can adjust to objectives changes perceived as meaningful and that caregivers fail in predicting patients' experience. PMID:22100276

  3. Age-Related Changes of Adaptive and Neuropsychological Features in Persons with Down Syndrome

    OpenAIRE

    Ghezzo, Alessandro; Salvioli, Stefano; Solimando, Maria Caterina; Palmieri, Alice; Chiostergi, Chiara; Scurti, Maria; Lomartire, Laura; Bedetti, Federica; Cocchi, Guido; Follo, Daniela; Pipitone, Emanuela; Rovatti, Paolo; Zamberletti, Jessica; Gomiero, Tiziano; Castellani, Gastone

    2014-01-01

    Down Syndrome (DS) is characterised by premature aging and an accelerated decline of cognitive functions in the vast majority of cases. As the life expectancy of DS persons is rapidly increasing, this decline is becoming a dramatic health problem. The aim of this study was to thoroughly evaluate a group of 67 non-demented persons with DS of different ages (11 to 66 years), from a neuropsychological, neuropsychiatric and psychomotor point of view in order to evaluate in a cross-sectional study...

  4. 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.

  5. Clinical significance of changes of serum hs-CRP, IL-6 and TNF-α levels after treatment in patients with polycystic ovary syndrome

    International Nuclear Information System (INIS)

    Objective: To explore the clinical significance of changes of serum hs-CRP, IL-6 and TNF-α levels in patients with polycystic ovary syndrome. Methods: Serum hs-CRP (with immuno turbidity method), IL-6, TNF-α (with RIA) levels were determined in 31 patients with polycystic ovary syndrome both before and after six, month's treatment as well as 35 controls. Results: Before treatment, the serum hs-CRP, IL-6 and TNF-α levels in the 31 patients with polycystic ovary syndrome were significantly higher than those in controls (P0.05). Serum hs-CRP levels were positive correlate with serum IL-6, TNF-α levels (r=0.6014, 0.5982, P<0.01). Conclusion: Serum hs-CRP, IL-6 and TNF-α levels were correlated to the development of polycystic ovary syndrome (PCOS). (authors)

  6. The Effect of Changing Serum 25-Hydroxyvitamin D Concentrations on Metabolic Syndrome: A Longitudinal Analysis of Participants of a Preventive Health Program

    Directory of Open Access Journals (Sweden)

    Truong-Minh Pham

    2015-08-01

    Full Text Available Several studies have shown that a poor vitamin D status may increase the risk of developing metabolic syndrome, which leaves the question whether improving one’s vitamin D status may reduce the risk for the syndrome. Here we investigate the effect of temporal changes in serum 25-hydroxyvitamin D (25(OHD concentrations on metabolic syndrome among Canadians enrolled in a preventive health program that promotes vitamin D supplementation. We accessed and analyzed data of 6682 volunteer participants with repeated observations on serum 25(OHD concentrations and metabolic syndrome. We applied logistic regression to quantify the independent contribution of baseline serum 25(OHD and temporal increases in serum 25(OHD to the development of metabolic syndrome. In the first year in the program, participants, on average, increased their serum 25(OHD concentrations by 37 nmol/L. We observed a statistical significant inverse relationship of increases in serum 25(OHD with risk for metabolic syndrome. Relative to those without improvements, those who improved their serum 25(OHD concentrations with less 25 nmol/L, 25 to 50 nmol/L, 50 to 75 nmol/L, and more 75 nmol/L had respectively 0.76, 0.64, 0.59, 0.56 times the risk for metabolic syndrome at follow up. These estimates were independent of the effect of baseline serum 25(OHD concentrations on metabolic syndrome. Improvement of vitamin D status may help reduce the public health burden of metabolic syndrome, and potential subsequent health conditions including type 2 diabetes and cardiovascular disease.

  7. Extending the mutation spectrum for Galloway-Mowat syndrome to include homozygous missense mutations in the WDR73 gene.

    Science.gov (United States)

    Rosti, Rasim O; Dikoglu, Esra; Zaki, Maha S; Abdel-Salam, Ghada; Makhseed, Nawal; Sese, Jordan C; Musaev, Damir; Rosti, Basak; Harbert, Mary J; Jones, Marilyn C; Vaux, Keith K; Gleeson, Joseph G

    2016-04-01

    Galloway-Mowat syndrome is a rare autosomal-recessive disorder classically described as the combination of microcephaly and nephrotic syndrome. Recently, homozygous truncating mutations in WDR73 (WD repeat domain 73) were described in two of 31 unrelated families with Galloway-Mowat syndrome which was followed by a report of two sibs in an Egyptian consanguineous family. In this report, seven affecteds from four families showing biallelic missense mutations in WDR73 were identified by exome sequencing and confirmed to follow a recessive model of inheritance. Three-dimensional modeling predicted conformational alterations as a result of the mutation, supporting pathogenicity. An additional 13 families with microcephaly and renal phenotype were negative for WDR73 mutations. Missense mutations in the WDR73 gene are reported for the first time in Galloway-Mowat syndrome. A detailed phenotypic comparison of all reported WDR73-linked Galloway-Mowat syndrome patients with WDR73 negative patients showed that WDR73 mutations are limited to those with classical Galloway-Mowat syndrome features, in addition to cerebellar atrophy, thin corpus callosum, brain stem hypoplasia, occasional coarse face, late-onset and mostly slow progressive nephrotic syndrome, and frequent epilepsy. PMID:27001912

  8. Ferulic Acid Alleviates Changes in a Rat Model of Metabolic Syndrome Induced by High-Carbohydrate, High-Fat Diet

    Directory of Open Access Journals (Sweden)

    Ketmanee Senaphan

    2015-08-01

    Full Text Available Metabolic syndrome is a cluster of metabolic abnormalities characterized by obesity, insulin resistance, hypertension and dyslipidemia. Ferulic acid (FA is the major phenolic compound found in rice oil and various fruits and vegetables. In this study, we examined the beneficial effects of FA in minimizing insulin resistance, vascular dysfunction and remodeling in a rat model of high-carbohydrate, high-fat diet-induced metabolic changes, which is regarded as an analogue of metabolic syndrome (MS in man. Male Sprague-Dawley rats were fed a high carbohydrate, high fat (HCHF diet and 15% fructose in drinking water for 16 weeks, where control rats were fed with standard chow diet and tap water. FA (30 or 60 mg/kg was orally administered to the HCHF and control rats during the last six weeks of the study. We observed that FA significantly improved insulin sensitivity and lipid profiles, and reduced elevated blood pressure, compared to untreated controls (p < 0.05. Moreover, FA also improved vascular function and prevented vascular remodeling of mesenteric arteries. The effects of FA in HCHF-induced MS may be realized through suppression of oxidative stress by down-regulation of p47phox, increased nitric oxide (NO bioavailability with up-regulation of endothelial nitric oxide synthase (eNOS and suppression of tumor necrosis factor-α (TNF-α. Our results suggest that supplementation of FA may have health benefits by minimizing the cardiovascular complications of MS and alleviating its symptoms.

  9. Ferulic Acid Alleviates Changes in a Rat Model of Metabolic Syndrome Induced by High-Carbohydrate, High-Fat Diet.

    Science.gov (United States)

    Senaphan, Ketmanee; Kukongviriyapan, Upa; Sangartit, Weerapon; Pakdeechote, Poungrat; Pannangpetch, Patchareewan; Prachaney, Parichat; Greenwald, Stephen E; Kukongviriyapan, Veerapol

    2015-08-04

    Metabolic syndrome is a cluster of metabolic abnormalities characterized by obesity, insulin resistance, hypertension and dyslipidemia. Ferulic acid (FA) is the major phenolic compound found in rice oil and various fruits and vegetables. In this study, we examined the beneficial effects of FA in minimizing insulin resistance, vascular dysfunction and remodeling in a rat model of high-carbohydrate, high-fat diet-induced metabolic changes, which is regarded as an analogue of metabolic syndrome (MS) in man. Male Sprague-Dawley rats were fed a high carbohydrate, high fat (HCHF) diet and 15% fructose in drinking water for 16 weeks, where control rats were fed with standard chow diet and tap water. FA (30 or 60 mg/kg) was orally administered to the HCHF and control rats during the last six weeks of the study. We observed that FA significantly improved insulin sensitivity and lipid profiles, and reduced elevated blood pressure, compared to untreated controls (p < 0.05). Moreover, FA also improved vascular function and prevented vascular remodeling of mesenteric arteries. The effects of FA in HCHF-induced MS may be realized through suppression of oxidative stress by down-regulation of p47phox, increased nitric oxide (NO) bioavailability with up-regulation of endothelial nitric oxide synthase (eNOS) and suppression of tumor necrosis factor-α (TNF-α). Our results suggest that supplementation of FA may have health benefits by minimizing the cardiovascular complications of MS and alleviating its symptoms.

  10. Turner Syndrome

    OpenAIRE

    Ramachandran Sudarshan; G Sree Vijayabala; KS Prem Kumar

    2012-01-01

    Turner syndrome is a genetic disorder that affects mostly females. Affected females have characteristic features such as short stature, premature ovarian failure, and several other features. Oral manifestations of this condition are not much discussed in the literature. But reported literature includes teeth, palate, periodontal and salivary changes. So the aim of this review is to illustrate the general manifestations, and especially the oral manifestations of Turner syndrome and evaluate th...

  11. Changes of pregnancy-associated plasma protein-A in patients with acute coronary syndrome

    Institute of Scientific and Technical Information of China (English)

    LIU Jin-lai; ZHANG Hui; XIE Xu-jing; CHEN Lin; ZHAO Chang-lin

    2005-01-01

    @@ The term vulnerable patient has been proposed to define subjects susceptible to an acute coronary syndrome (ACS) or sudden cardiac death based on plaque characteristics, blood abnorma-lities, or myocardial vulnerability.1 It will be important in the future to identify both vulnerable patients and vulnerable plaques. Atherosclerotic arteries obtained at autopsy from patients who died suddenly of cardiac causes indicate that pregnancy-associated plasma protein-A (PAPP-A) was abundantly expressed in plaque cells and in the extracellular matrix of ruptured and eroded unstable plaques, but not in stable plaques.2 Here we examined circulating PAPP-A levels in patients with ACS in order to evaluate its potential use in identifying vulnerable patients.

  12. [Ultrastructural changes in the lung in acute adult respiratory distress syndrome].

    Science.gov (United States)

    Szemenyei, K; Széll, K; Kádas, L

    1980-04-01

    Morphological alterations of the lung in respiratory distress syndrome of adults (ARDS) were analyzed in 10 cases with traumatic-and septic shock, laryngitis subglottica descendens and bronchopneumonia. For the better understanding of the pathomechanism of the disease in addition to the standard methods, first of all ultrastructural alterations were studied. Two phases of the morphologic alterations could be distinguished, the phase of the destruction and the phase of the repair. These two processes are not sharply distinguishable. Genesis of the characteristic histological alterations (damage to the epithelial and endothelial cells, formation of hyaline membranes, microcoagulation, proliferation of the type II pneumocytes and fibroblasts, fibrosis) is discussed, with regard to the data of the literature.

  13. Post-traumatic fat embolism syndrome--a 10 year retrospective study in Chang Gung Memorial Hospital.

    Science.gov (United States)

    Hsu, D T; Chao, E K; Shih, C H

    1990-06-20

    Forty patients with post-traumatic fat embolism syndrome (FES) from January 1977 through December 1986 were retrospectively analyzed. Diagnosis was made according to the criteria modified by Guard. All 40 patients had at least two major criteria, namely change in consciousness and hypoxia. Twenty patients (50%) presented with full-blown clinical features. Prompt respiratory support with oxygen mask or nasal prongs was the first line of treatment. Forty per cent of patients responded well, whereas the others had to be advanced to endotracheal intubation and mechanical ventilation with positive end expiratory pressure. Steroids were given to 80% of patients and better results were achieved than in the group not treated with steroids. The mortality rate was low (2.5%) and only four patients suffered prolonged cerebral sequelae. The clinical course and prognosis cannot be predicted from the severity of the fracture. PMID:2224610

  14. Dynamic Changes in Serum Estradiol and Progesterone levels in Patients of Premenstrual Syndrome with Adverse Flow of Liver-qi

    Institute of Scientific and Technical Information of China (English)

    Qiao Mingqi; Zhang Huiyun; Yu Yanhong; Ci Yuzhen; Xu Xujie; Ye Qing; Chen Yuzhen; Wang Xinzhong

    2008-01-01

    Objective:To probe into the influence of changes of ovarian hormones on the pathogenesis of the specific sub-type premenstrual syndrome(PMS)and reveal partial microcosmic mechanisms of adverse flow of liver-qi.Methods:Estradiol(E2)and progesterone(P)levels in serum were determined at different phases of menstrual cycle by radioimmunoassay.Results:In the group of PMS with adverse flow of liver-qi.the secretive peak value Of E2 and P at the follicular phase significantly decreased,and the secretive peak value at the luteal phase did not come into being.Conclusions:Low E2 and P secretive peak at the follicular phase and absence of secretive peak at the luteal phase is one of the microcosmic mechanisms of PMS with adverse flow of liver-qi.One of the pathophysiologic mechanisms of specific sub-type PMS is probably the continuous low level of E2and P.

  15. Assessment of Metformin as an Additional Treatment to Therapeutic Lifestyle Changes in Pediatric Patients with Metabolic Syndrome

    Directory of Open Access Journals (Sweden)

    Rebecca M. Raub

    2012-01-01

    Full Text Available Objective. To assess the effectiveness of metformin and therapeutic lifestyle changes (TLCs in a clinical setting, compared to TLC alone in adolescents with metabolic syndrome (MS. Methodology. This study was a retrospective trial consisting of 60 patients, aged 8–18 years, who were treated for MS at an outpatient clinic. Two groups were formed: the metformin group (M group and the control group (C group. The M group had been given metformin along with TLC, and the C group had been given TLC alone. Several outcome measures were obtained; the main outcome measure was measuring the change in percentile and z-score of weight and BMI. Results. There were no significant differences between the two groups at the conclusion of the study, except for height percentile (P=0.02 and z-score (P=0.03. Both groups showed promising significant intragroup decreases in weight z-score but BMI percentile and z-score were only significantly decreased in the M group. Conclusion. Metformin at an average dose of 1033 mg, when added to TLC, did not show any clinically important efficacy compared to TLC alone in a pediatric population with MS. However, both groups made significant changes in a positive direction, which may be solely due to TLC.

  16. Bone mineral density and changes in bone metabolism in patients with obstructive sleep apnea syndrome.

    Science.gov (United States)

    Terzi, Rabia; Yılmaz, Zahide

    2016-07-01

    The aim of this study was to evaluate the differences between patients with obstructive sleep apnea syndrome (OSAS) and phenotypically similar subjects without OSAS in terms of bone mineral density (BMD) and bone turnover markers. The study was conducted on 30 males diagnosed with OSAS and 20 healthy males. All subjects underwent polysomnographic testing. Calcium, phosphorus parathyroid hormone, thyroid stimulating hormone, bone-specific alkaline phosphatase, 25-hydroxyvitamin D3, osteocalcin, and beta-CrossLaps (β-CTx) were measured. BMD in the lumbar spine (L1-L4) and femoral neck was measured by dual energy X-ray absorptiometry. There was no statistically significant difference between the two groups in terms of demographic data with the exception of bone mass index and waist circumference. (p < 0.05). Analyses showed significantly lower BMD measurements in the femoral neck and T-scores in the femoral neck in patients diagnosed with OSAS. Serum β-CTx levels were found to be statistically significantly higher in the OSAS group (p = 0.017). In multivariate assessments performed for apnea/hypopnea index values, mean saturation O2 levels were found to be significantly associated with osteocalcin levels and neck BMD. OSAS patients might represent a risk group with respect to loss of BMD and bone resorption. It is important to evaluate bone loss in these patients. Further studies should be carried out on larger study populations to evaluate the effects of chronic hypoxia on BMD in detail. PMID:26204846

  17. Age-related changes of adaptive and neuropsychological features in persons with Down Syndrome.

    Directory of Open Access Journals (Sweden)

    Alessandro Ghezzo

    Full Text Available Down Syndrome (DS is characterised by premature aging and an accelerated decline of cognitive functions in the vast majority of cases. As the life expectancy of DS persons is rapidly increasing, this decline is becoming a dramatic health problem. The aim of this study was to thoroughly evaluate a group of 67 non-demented persons with DS of different ages (11 to 66 years, from a neuropsychological, neuropsychiatric and psychomotor point of view in order to evaluate in a cross-sectional study the age-related adaptive and neuropsychological features, and to possibly identify early signs predictive of cognitive decline. The main finding of this study is that both neuropsychological functions and adaptive skills are lower in adult DS persons over 40 years old, compared to younger ones. In particular, language and short memory skills, frontal lobe functions, visuo-spatial abilities and adaptive behaviour appear to be the more affected domains. A growing deficit in verbal comprehension, along with social isolation, loss of interest and greater fatigue in daily tasks, are the main features found in older, non demented DS persons evaluated in our study. It is proposed that these signs can be alarm bells for incipient dementia, and that neuro-cognitive rehabilitation and psycho-pharmacological interventions must start as soon as the fourth decade (or even earlier in DS persons, i.e. at an age where interventions can have the greatest efficacy.

  18. [Antioxidative status changes in golden syrian hamsters with experimental metabolic syndrome].

    Science.gov (United States)

    Zahaĭko, A L; Voronina, L M; Kaliman, P A; Strel'chenko, K V

    2008-01-01

    Some indices of the antioxidant status (content of the alpha-tocopherol, reduced glutathione and ascorbic acid, activity of the glutathione reductase and aryl-esterase) and lipid peroxidation processes in the liver, blood serum, and some blood serum lipoprotein fractions of the Golden Syrian hamsters of different sex and age status under high-caloric diet were investigated. It has been shown that the hypercaloric diet leads to a decreaseng of reduced glutathione content and increase of the level of lipid peroxidation products in the liver of experimental animals. The ascorbic acids content in male liver is decreased and in female liver is increased. In the blood serum under hypercaloric nutrition the accumulation of lipid peroxidation products and alpha-tocopherol content a decrease in ApoB-lipoproteins and HDL is observed. Simultaneously the ascorbic acid content is increased in the blood serum of all experimental animals. Activation of free-radical oxidation both in the liver, and blood serum is more significant in males compared with females. The data obtained allow to suppose that atherosclerotic complications of metabolic syndrome development may be connected to the lipoprotein oxidant status infringement. PMID:18959034

  19. Changes in Coagulation and Fibrinolytic Indices in Women with Polycystic Ovarian Syndrome Undergoing Controlled Ovarian Hyperstimulation

    Directory of Open Access Journals (Sweden)

    Ying Huang

    2014-01-01

    Full Text Available Background. Polycystic ovarian syndrome (PCOS women undergoing in vitro fertilization and embryo transfer (IVF-ET treatment always attain a low cumulative pregnancy rate disaccording with the satisfactory number of oocytes. Objective. We aim to evaluate the status of coagulation and fibrinolytic system in PCOS patients undergoing controlled ovarian hyperstimulation (COH process. Method. Of the 97 women, 30 patients with PCOS composed the study group; 67 women of child-bearing age with normal endocrine function composed the control group. All participants underwent GnRH agonist standard long protocol, and plasma HCY, FVIII, FX, and D-dimer levels as well as hormone parameters were measured at day of full downregulation, hCG priming, and embryos transfer. Results. On day of full downregulation, FX levels were significantly higher in PCOS group (P<0.01. On hCG priming day, FX and estrogen levels in PCOS group were higher than in the control group and FVIII levels were significantly lower on day of embryos transfer whereas FX and E2 levels were significantly higher in PCOS group. Conclusion. Hypercoagulable state during peri-implantation phase would probably lead to poor microcirculation of endometrium and be one of the most important disadvantages of successful implantation and subsequent clinical pregnancy.

  20. [Heptopulmonary syndrome].

    Science.gov (United States)

    Cuadrado, Antonio; Díaz, Ainhoa; Iruzubieta, Paula; Salcines, José Ramón; Crespo, Javier

    2015-01-01

    Hepatopulmonary syndrome is characterized by the presence of liver disease, pulmonary vascular dilatations, and arterial hypoxemia. It is usually associated with cirrhosis of any origin, but has been described in other liver diseases, both acute and chronic, and not always associated with portal hypertension. The gold standard method to detect pulmonary vascular dilations is contrast enhancement echocardiography with saline and is essential for the diagnosis of hepatopulmonary syndrome. These dilatations reflect changes in the pulmonary microvasculature (vasodilatation, intravascular monocyte accumulation, and angiogenesis) and induce a ventilation/perfusion mismatch, or even true intrapulmonary shunts, which eventually trigger hypoxemia. This syndrome worsens patients' prognosis and impairs their quality of life and may lead to the need for liver transplantation, which is the only effective and definitive treatment. In this article, we review the etiological, pathophysiological, clinical and therapeutic features of this syndrome. PMID:25840463

  1. [MORPHOLOGICAL CHANGES OF SKIN IN OPERATIVE WOUND IN SYNDROME OF OPIOID-INDUCED HYPERALGESIA].

    Science.gov (United States)

    Dmytriyev, D V; Konoplytskyi, V S

    2015-10-01

    Morphological changes of skin in region of operative wound were investigated. There was established, that while application of fentanyl in high doses, using constant infusion for anesthesia in early postoperative period in children, operated for abdominal cavity tumors, the opioid-induced hyperalgesia occurrence is possible, what is accompanied by morphological changes in skin around operative wound, necrosis in centre of focus, pronounced perifocal reactive changes in a kind of significant inflammation and essential disorder of microcirculation with formation of small neural fibers on the 14th day. Pronounced fibrosis of derma, formation of big quantity of collagen fibers with edema, stratification.

  2. Ultrastructural Changes of Airway in Murine Models of Allergy and Diet-Induced Metabolic Syndrome

    OpenAIRE

    Leishangthem, Geeta Devi; Mabalirajan, Ulaganathan; Singh, Vijay Pal; Agrawal, Anurag; Ghosh, Balaram; Dinda, Amit Kumar

    2013-01-01

    Studying ultrastructural changes could reveal novel pathophysiology of obese-asthmatic condition as existing concepts in asthma pathogenesis are based on the histological changes of the diseased airway. While asthma is defined in functional terms, the potential of electron microscopy (EM) in providing cellular and subcellular detail is underutilized. With this view, we have performed transmission EM in the lungs from allergic mice that show key features of asthma and high-fat- or high-fructos...

  3. The Change of Interleukin-6 and Tumor Necrosis Factor in Patients with Obstructive Sleep Apnea Syndrome

    Institute of Scientific and Technical Information of China (English)

    2000-01-01

    The levels of lipopolysaccharide (LPS)-induced interleukin-6 (IL-6) and tumor necrosis factor-α (TNF-α) expression in culture of peripheral blood mononuclear cells (PBMC) and the plasma levels of IL-6 and TNF-α in the patients with obstructive sleep apnea syndrome (OSAS) were measured and the relationship between OSAS and IL-6 or TNF-α expression studied. Both IL-6 and TNF-α were detected by using ELISA in 22 patients with OSAS and 16 normal controls. The levels of LPS-induced IL-6 (787.82±151.97 pg/ml) and TNF-α (4165.45±1501.43 pg/ml) expression in the supernatant of the culture of PBMC and plasma level of IL-6 (50.67±4.70 pg/ml) and TNF-α (299.09±43.57 pg/ml) in the patients with OSAS were significantly higher than those in the normal controls (in the supernatant of the culture of PBMC: 562.69±197.54 pg/ml and 1596.25±403.08 pg/ml respectively; in the plasma: 12.69±2.75 pg/ml and 101.88±21.27 pg/ml respectively). There were significantly positive correlation between the levels of IL-6 and TNF-α and the percentage of time of apnea and hyponea, as well as the percentage of time spending at SaO2 below 90 % in the total sleep time. It was concluded that LPS-induced IL-6 and TNF-α levels as well as plasma IL-6 and TNF-α levels in the patients with OSAS were up-regulated, which may be associated with the pathogenesis of OSAS.

  4. Metabolic Syndrome Is Associated with Increased Oxo-Nitrative Stress and Asthma-Like Changes in Lungs.

    Science.gov (United States)

    Singh, Vijay Pal; Aggarwal, Rangoli; Singh, Suchita; Banik, Arpita; Ahmad, Tanveer; Patnaik, Bijay Ranjan; Nappanveettil, Giridharan; Singh, Kunal Pratap; Aggarwal, Madan Lal; Ghosh, Balaram; Agrawal, Anurag

    2015-01-01

    Epidemiological studies have shown an increased obesity-related risk of asthma. In support, obese mice develop airway hyperresponsiveness (AHR). However, it remains unclear whether the increased risk is a consequence of obesity, adipogenic diet, or the metabolic syndrome (MetS). Altered L-arginine and nitric oxide (NO) metabolism is a common feature between asthma and metabolic syndrome that appears independent of body mass. Increased asthma risk resulting from such metabolic changes would have important consequences in global health. Since high-sugar diets can induce MetS, without necessarily causing obesity, studies of their effect on arginine/NO metabolism and airway function could clarify this aspect. We investigated whether normal-weight mice with MetS, due to high-fructose diet, had dysfunctional arginine/NO metabolism and features of asthma. Mice were fed chow-diet, high-fat-diet, or high-fructose-diet for 18 weeks. Only the high-fat-diet group developed obesity or adiposity. Hyperinsulinemia, hyperglycaemia, and hyperlipidaemia were common to both high-fat-diet and high-fructose-diet groups and the high-fructose-diet group additionally developed hypertension. At 18 weeks, airway hyperresponsiveness (AHR) could be seen in obese high-fat-diet mice as well as non-obese high-fructose-diet mice, when compared to standard chow-diet mice. No inflammatory cell infiltrate or goblet cell metaplasia was seen in either high-fat-diet or high-fructose-diet mice. Exhaled NO was reduced in both these groups. This reduction in exhaled NO correlated with reduced arginine bioavailability in lungs. In summary, mice with normal weight but metabolic obesity show reduced arginine bioavailability, reduced NO production, and asthma-like features. Reduced NO related bronchodilation and increased oxo-nitrosative stress may contribute to the pathogenesis.

  5. Acute mental status change as the presenting feature of adrenal insufficiency in a patient with autoimmune polyglandular syndrome type II and stroke

    OpenAIRE

    Watson, Sara; Raj, Shekar; Eugster, Erica; Sanchez, Juan

    2014-01-01

    Primary adrenal insufficiency (AI) in children usually presents with non-specific symptoms such as fatigue, nausea, vomiting, and anorexia. Here, we report an unusual case of a 15 year old girl who presented with acute mental status change and was ultimately diagnosed with AI due to autoimmune polyglandular syndrome type II (APS2). Central nervous system imaging revealed a cerebral infarction. To our knowledge, the constellation of APS2, stroke and acute mental status change has not been prev...

  6. How Is Metabolic Syndrome Treated?

    Science.gov (United States)

    ... page from the NHLBI on Twitter. How Is Metabolic Syndrome Treated? Heart-healthy lifestyle changes are the first line of treatment for metabolic syndrome. If heart-healthy lifestyle changes aren’t enough, ...

  7. Returning to work after suffering from burnout syndrome: Perceived changes in personality, views, values, and behaviors connected with work

    Directory of Open Access Journals (Sweden)

    Boštjančič Eva

    2014-01-01

    Full Text Available To date only a few studies have focused on returning to work after suffering from burnout syndrome. Participants were asked about their perceived work effectiveness, changes in their personal values, and obstacles and support factors that they encountered when they returned to work. Among the 27 individuals of various professions included in the study, 18 achieved an average or a high score on the Maslach Burnout Inventory, which was used to conduct a semi-structured interview. The answers were later processed by analyzing the content. The results showed that burned-out individuals only slowly return to work after recovery. When they return to work, they encounter changes in personality, personal values, and work effectiveness, and they only receive partial support from the environment. The results draw attention to insufficient detection of the disease by medical staff and employers in Slovenia. Recovering from burnout is a long-term process, which depends most on individuals themselves. At the same time, they can receive the necessary support from their family and coworkers, especially in terms of understanding them and partially adapting their responsibilities at work when they return. This study draws attention to a number of factors that can influence an individual’s process of returning to work and can be used as a basis for developing systematic rehabilitation programs.

  8. Corneal changes in ectrodactyly-ectodermal dysplasia-cleft lip and palate syndrome: case series and literature review.

    Science.gov (United States)

    Felipe, Anthony F; Abazari, Azin; Hammersmith, Kristin M; Rapuano, Christopher J; Nagra, Parveen K; Peiro, Baltasar Moratal

    2012-10-01

    The aim of this study is to describe the corneal changes in three unrelated patients with ectrodactyly-ectodermal dysplasia-cleft lip and palate (EEC) syndrome and review the literature on the possible etiology and clinical presentation of similar cases. Case 1 is an 18-year-old female with cleft lip and palate, syndactyly, and bilateral corneal pannus superiorly and inferiorly. She was initially diagnosed and treated as herpes simplex virus keratitis. Case 2 is a 3-year-old female born with cleft lip and palate, absent radial digits in both hands, and bilateral lacrimal stenosis. She developed progressive stromal scarring and neovascularization in both eyes. Her cornea perforated after developing infectious ulceration. Case 3 is a 49-year-old male with cleft palate, claw-hand deformities, absent meibomian glands and lacrimal duct, right ankyloblepharon, and a superior wedge-shaped opacity in the left cornea. The clinical findings demonstrated the different spectrum of keratopathy seen in patients with EEC. All patients were treated medically and without any surgical intervention. Limbal stem cell deficiency (LSCD) is presumed to be the cause in all three cases. Corneal changes in EEC can have variable presentation. LSCD seems to be the etiology of such keratopathy. Recurrent infection from lacrimal drainage obstruction and tear film instability are other risk factors for disease severity and progression. PMID:22618129

  9. Hyperornithinemia-hyperammonemia-homocitrullinuria (HHH)-syndrome. Ultrastructural changes of mitochondria in cultured dermal fibroblasts of three patients.

    Science.gov (United States)

    Haust, M D; Dewar, R A; Gatfield, D P; Gordon, B A

    1996-03-01

    Mitochondria of fibroblasts cultured from the skin obtained at biopsy from three patients with the hyperornithinemia-hyperammonemia-homocitrullinuria (HHH)-syndrome, one of the autosomal recessive, heritable urea cycle disorders, were studied with appropriate controls ultrastructurally. The patients were two severely retarded 10- and 12-year-old boys, and a 22-year-old sister of the former whose mental status was at the low normal range; she never had motor impairments or seizures. The mitochondria, similar in all three patients, were increased in number, very long, branching and/or "looping," and tortuous. "Spurs" or "buddings" extended from their lateral surfaces and the terminal segments were often bulbous. Other unusual configurations were also present. In addition, giant forms with large diameter contained innumerable closely-packed and parallel cristae which traversed the entire width of these mitochondria; at times they assumed a "whirled" pattern. The mitochondrial matrix was usually of high electron density. These changes were not a feature of fibroblastic mitochondria of controls. Several changes resembled those of hepatic mitochondria in this disorder. All features are interpreted as an attempt at expanding the mitochondrial volume (via structural substratum) to compensate for the metabolic incompetence of these organelles (a block in transmembranous transfer of ornithine from hyaloplasm into mitochondria for conversion to citrulline).

  10. White spot syndrome virus induces metabolic changes resembling the warburg effect in shrimp hemocytes in the early stage of infection.

    Science.gov (United States)

    Chen, I-Tung; Aoki, Takashi; Huang, Yun-Tzu; Hirono, Ikuo; Chen, Tsan-Chi; Huang, Jiun-Yan; Chang, Geen-Dong; Lo, Chu-Fang; Wang, Han-Ching

    2011-12-01

    The Warburg effect is an abnormal glycolysis response that is associated with cancer cells. Here we present evidence that metabolic changes resembling the Warburg effect are induced by a nonmammalian virus. When shrimp were infected with white spot syndrome virus (WSSV), changes were induced in several metabolic pathways related to the mitochondria. At the viral genome replication stage (12 h postinfection [hpi]), glucose consumption and plasma lactate concentration were both increased in WSSV-infected shrimp, and the key enzyme of the pentose phosphate pathway, glucose-6-phosphate dehydrogenase (G6PDH), showed increased activity. We also found that at 12 hpi there was no alteration in the ADP/ATP ratio and that oxidative stress was lower than that in uninfected controls. All of these results are characteristic of the Warburg effect as it is present in mammals. There was also a significant decrease in triglyceride concentration starting at 12 hpi. At the late stage of the infection cycle (24 hpi), hemocytes of WSSV-infected shrimp showed several changes associated with cell death. These included the induction of mitochondrial membrane permeabilization (MMP), increased oxidative stress, decreased glucose consumption, and disrupted energy production. A previous study showed that WSSV infection led to upregulation of the voltage-dependent anion channel (VDAC), which is known to be involved in both the Warburg effect and MMP. Here we show that double-stranded RNA (dsRNA) silencing of the VDAC reduces WSSV-induced mortality and virion copy number. For these results, we hypothesize a model depicting the metabolic changes in host cells at the early and late stages of WSSV infection.

  11. Volumetric Brain Morphometry Changes in patients with Obstructive Sleep Apnea Syndrome : effects of CPAP treatment and literature review.

    Directory of Open Access Journals (Sweden)

    Nelly T Huynh

    2014-04-01

    Full Text Available Introduction: Obstructive sleep apnea syndrome (OSAS is a frequent breathing disorder occurring during sleep that is characterized by recurrent hypoxic episodes and sleep fragmentation. It remains unclear whether OSAS leads to structural brain changes, and if so, in which brain regions. Brain region-specific gray and white matter volume (GMV and WMV changes can be measured with voxel-based morphometry (VBM. The aims of this study were to use VBM to analyze GMV and WMV in untreated OSAS patients compared to healthy controls (HC; examine the impact of OSAS-related variables (nocturnal hypoxemia duration and sleep fragmentation index on GMV and WMV; and assess the effects of therapeutic versus sham continuous positive airway pressure (CPAP. We discuss our results in light of previous findings and provide a comprehensive literature review. Methods: Twenty-seven treatment-naïve male patients with moderate to severe OSAS and seven healthy age- and education-matched control subjects (HC were recruited. After a baseline fMRI scan, patients randomly received either active (therapeutic, n=14 or sham (subtherapeutic, n=13 nasal CPAP treatment for 2 months. Results: Significant negative correlations were observed between nocturnal hypoxemia duration and GMV in bilateral lateral temporal regions. No differences in GMV or WMV were found between OSAS patients and HC, and no differences between CPAP versus sham CPAP treatment effects in OSAS patients. Conclusion: It appears that considering VBM GMV changes there is little difference between OSAS patients and HC. The largest VBM study to date indicates structural changes in the lateral aspect of the temporal lobe, which also showed a significant negative correlation with nocturnal hypoxemia duration in our study. This finding suggests an association between the effect of nocturnal hypoxemia and decreased GMV in OSAS patients.

  12. Thyroid Malignancy Association with Cortical and Subcortical Brain SPECT Changes In Patients Presenting with a Myalgic Encephalomyelitis / Chronic Fatigue Syndrome

    International Nuclear Information System (INIS)

    Thyroid malignancy in ME/CFS patients greatly exceeds the normal incidence of thyroid malignancy in any known subgroup. The thyroid malignancy incidence in the ME/CFS group may exceed 6,000 / 100,000. As part of their investigation, Myalgic Encephalomyelitis / Chronic Fatigue Syndrome (ME/CFS) patients should be examined by thyroid ultrasound for evidence of thyroid pathology and malignancy. Thyroid pathology may be missed in this group of patients if investigation relies only upon serum testing for TSH, FT3, FT4, microsomal and thyroglobulin antibodies, which are usually normal. Thyroid uptake scans tend also to be normal and may also miss malignant lesions. A newly recognized syndrome may exist in ME/CFS patients characterized by: (a) thyroid malignancy, (b) persistent abnormal cortical and subcortical SPECT brain scans (NeuroSPECT), (c) failure of thyroidectomy surgery and hormone replacement to correct the fatigue syndrome, and (d) an unusual high incidence of cervical vertebrae osteoarthritic changes. ME/CFS patients with treated non-malignant thyroid disease and abnormal NeuroSPECT scans may also fail to improve despite adequate thyroid hormone replacement. A brief summary of the differences between ME and CFS is discussed. Lee, Hur and Ahn [1] stated that thyroid malignancy is said to be an infrequent occurrence found in 0.5 to 3 patients per 100,000 in the general population. They noted that in a subgroup of patients booked for mammography, a thyroid ultrasound was also performed. In this group, they found thyroid malignancy frequency was as high as 3 per 100,000. It is not known if their subgroup was at a higher risk for malignancy. Mittelstaedt [2] in the Globe and Mail states that thyroid malignancy was 15 per 100,000. In the past 100 patients whom I have investigated for (ME/CFS)[3], with or without associated Fibromyalgia Syndrome (FS), I have found that 6% of these patients had thyroid malignancy. In each of these patients the diagnosis was made by

  13. The detection of aberrantly glycosylated serum IgA1 in minimal change nephrotic syndrome with mesangial IgA deposition patients%伴IgA沉积的微小病变肾病综合征患者血清IgA1糖基化缺陷的检测

    Institute of Scientific and Technical Information of China (English)

    陈玮; 燕宇; 陈幸; 陈欢; 王梅

    2012-01-01

    目的 通过对伴IgA沉积的微小病变肾病综合征(MCNS-IgA)患者血清中IgA1分子糖基化缺陷程度的检测,探讨MCNS-IgA的可能的病理分类归属. 方法 选择北京大学人民医院肾内科MCNS-IgA患者10例,微小病变肾病综合症(MCNS)患者10例、大量蛋白尿IgAN (H-IgAN)患者10例为对照.用双抗体夹心ELISA法检测各组患者血清IgA1的相对浓度,用黑木凝集素检测IgA1分子的α2,6唾液酸水平,花生凝集素检测IgA1分子的半乳糖水平,蜗牛凝集素检测IgA1分子的N-乙酰氨基半乳糖水平,计算经血清IgA1浓度校正的各糖基水平.观察MCNS-IgA组患者血清IgA1分子糖基化缺陷情况,并且与H-IgAN及MCNS组进行比较.结果 与MCNS组相比,MCNS-IgA肾病患者血清IgA1的α2,6唾液酸(1.232±0.250比较1.379±0.623,P=0.455)、半乳糖缺失(0.204±0.053 vs 0.229±0.088,P=0.454)水平及N-乙酰氨基半乳糖暴露(0.191±0.039 vs0.205±0.068,P=0.626)水半无明显差异.但其血清IgA1分子α2,6唾液酸缺失(1.232±0.250vs 0.756±0.243,P=0.015)及N-乙酰氨基半乳糖的暴露(0.191±0.039比0.258±0.066,P=0.025)显著低于H-IgAN组,半乳糖缺失少,但未达统计学差异(0.204±0.053比0.139±0.038,P=0.052). 结论 MCNS-IgA组患者血清IgA1糖基化水平上显示了与IgA肾病不同的特点,提示其可能是微小病变伴IgA分子的非特异性沉积.%Objective To test the different degree of glycosylation of serum IgAl among MCNS-IgA, H-IgAN, MCNS, in order to investigate the relationship between MCNS-IgA and IgAN. Methods Sera from 30 patients were collected. 10 of them were MCNS-IgA, 10 patients were H-IgA and the rest were MCNS used as controls. Biotinylated lectins were used in ELISAto examine different glycans on serum IgAl molecules. The a 2,6 sialic acid was detected by elderberry bark lectin (SNA), the exposure of terminal galactose (Gal) and N-acetylgalactosamine (GalNAc) were detected by arachis hypogaea (PNA) and Helix asperas agglutinin (HAA), respectively. The IgAl glycans levels, corrected by IgAl concentrations, were compared between patients and controls. Results Reduced terminal a 2,6 sialic acid (1.232 ± 0.250 vs 0.756 ± 0.243, P=0.015), and increased exposure of N-acetylgalactosamine (0.191 ± 0.039 vs 0.258 ± 0.066, P=0.025) were demonstrated in serum IgAl from patients with H-IgAN as compared in that from MCNS-IgA. However, MNCS and MCNS-IgA had no significant differences among the levels of glycosylation (P > 0.05). Conclusion MCNS-IgA shows the characteristics of IgA nephropathy in serum IgAl glycosylation level, suggesting the presence of MCD with non-specific mesangial IgA molecule deposition.

  14. 儿童肾病综合征及泼尼松治疗对血清IGF-1及IGFBPs的影响%Change of insulin like growth factor-1(IGF-1) and IGF binding proteins during glucocorticoid treatment in children with nephrotic syndrome

    Institute of Scientific and Technical Information of China (English)

    董峰; 周湘; 庞宁; 魏珉

    2001-01-01

    目的了解儿童肾病综合征(NS)及糖皮质激素(GC)治疗对血清胰岛素样生长因子-1(IGF-1)及其结合蛋白(IGFBPs)的影响.方法采用免疫放射法对36名NS患儿,即未治疗的活动期组(ANS,12例),泼尼松治疗中活动期组(GNS,12例)和缓解期组(RE,12例),进行了血清IGF-1及其IGFBPs水平测定,并以同年龄组正常儿童作对照(NC,10例).结果 (1)与正常对照比较,NS活动期血清IGF-1和IGFBP-3明显降低(P<0.01),而IGFBP-1和IGFBP-2均明显升高(P<0.01);(2)NS缓解期血清IGF-1和IGFBP-3明显高于活动期 (P<0.01),而IGFBP-1和IGFBP-2明显低于活动期(P<0.01);(3)经激素治疗的活动期组血清IGF-1和IGFBP-3均明显高于未治疗活动期组 (P<0.01),IGFBP-1和IGFBP-2水平也明显低于ANS组(P<0.01);(4)NS活动期血清IGFBP-3与ALB成正相关(r=0.76,P<0.01),血清IGF-1与IGFBP-3成正相关,而与IGFBP-1、IGFBP-2成负相关.在缓解期及激素治疗中的活动期血清IGF-1及IGFBPs与ALB及CHO没有相关关系.结论肾病综合征及糖皮质激素治疗对NS儿童血清IGF-1和IGFBPs有明显影响,此影响可能与NS儿童生长障碍有关.

  15. 微小病变性肾病合并门静脉脾静脉肠系膜上静脉血栓1例并文献复习%Minimal change disease of nephrotic syndrome complicated with portal,splenic and superior mesenteric vein thrombosis

    Institute of Scientific and Technical Information of China (English)

    王军; 田百玲; 范秋灵

    2009-01-01

    原发性肾病综合征(NS)由多种疾病组成,主要包括微小病变性肾病、局灶节段性肾小球硬化、膜性肾病、IgA肾病、膜增生性肾小球肾炎等。NS易形成静脉血栓,多为肾静脉血栓、下肢静脉血栓等,但合并门静脉、脾静脉、肠系膜上静脉血栓形成十分罕见。现将1例NS合并门静脉、脾静脉、肠系膜上静脉血栓形成的病例报道如下。

  16. Beals Syndrome

    Science.gov (United States)

    ... Boards & Staff Annual Report & Financials Contact Us Donate Marfan & Related Disorders What is Marfan Syndrome? What are ... the syndrome. How does Beals syndrome compare with Marfan syndrome? People with Beals syndrome have many of ...

  17. VASCULAR ENDOTHELIAL INJURIES AND CHANGES OF BLOOD COAGULATION AND FIBRINOLYSIS INDEXES IN PATIENTS WITH ACUTE RESPIRATORY DISTRESS SYNDROME

    Institute of Scientific and Technical Information of China (English)

    Xiao-lin He; Zhi Liu; Shu-yue Xia

    2004-01-01

    Objective To study endothelial damage by observing changes of circulating endothelial cells (CECs) in blood, coagulation and fibrinolysis index in patients with acute respiratory distress syndrome.Methods CECs were separated by isopycnic centrifugation method in 14 patients with acute lung injury (ALI), 7patients with acute respiratory distress syndrome (ARDS), 10 intensive care unit (ICU) controls, and 15 healthy controls.Plasma prothrombin time (PT), activated partial thromboplastin time (APTT), fibrinogen (FG), fibrin degradation products (FDP), and D-dimer were examined simultaneously. Acute physiology and chronic health evaluation (APACHE) Ⅱ and lung injury score (LIS) were recorded to evaluate severity of illness and lung injury.Results (1) The number of CECs in ALI (10.4 ± 2.3 ) and ARDS groups ( 16.1 ± 2.7) was higher than that in the healthy (1.9 ± 0.5) (P < 0.01). In both ALI and ARDS, the number of CECs correlated with APACHE Ⅱ (r = 0.55, P < 0.05 and r =0.62, P < 0.05, respectively) and LIS (r = 0.60, P < 0.05 and r = 0.53, P < 0.05, respectively). CEC number was negatively correlated with PaO2 in ALI and ARDS (r=-0.49, P< 0.05 and r=-0.64, P< 0.05, respectively). (2) The level of FDP and D-dirmer were higher in ALI and ARDS patients than that in ICU and healthy control groups (P<0.05). The level of FG in ARDS group was significantly higher than in the ICU and healthy control groups (P < 0.05). But in ALI group, the level of FG was significantly higher than only healthy control group (P < 0.05).Conclusions Endothelial cell damage occurs in ARDS patients, which may play a major role in the pathophysiology of ARDS. Changes of endothelial cell activation and damage markers, such as CECs, plasma coagulation and fibrinolysis index,to some extent reflect severity of illness and lung injury in ARDS.

  18. The expression of glucose transporter 4 in endometrium of polycystic ovary syndrome women and its change after metformin treatment

    Institute of Scientific and Technical Information of China (English)

    Lin Xian-hua; Ye Bi-lu; Xu Bing-seng; Zhao Jun-zhao; Lin Jin-ju; Yang Hai-yan

    2007-01-01

    Objective: To investigate the protein and messenger RNA expression of glucose transporter 4 in endometrium of women with polycystic ovary syndrome (PCOS), and to evaluate its change after three months treatment of metformin.Methods.Twenty-two patients with polycystic ovary syndrome (group A) and six non-PCOS infertile women (group B) were recruited in our hospital.The consent form was obtained from each patient.Endometrium and blood samples were obtained during the proliferative phase of the menstrual cycle.Reverse transeriptase-polymerase chain reaction (RT-PCR) and immunohistochemical method were applied to detect the expression of glucose transporter 4 (GLUT4) in endometrium.All PCOS patients received monotherapy of metformin after endometrium biopsy.Seven un-conceived patients (group A1) from group A who completed three months of metformin treatment were selected to perform the second time biopsy during proliferative phase.The expression of GLUT4 were remeasured as well.Results.There were no significant differences of the levels of E2, P and endometrium thickness on the biopsy day between group A and group B.But the basal levels of LH, T, LH/FSH ratio, and the ovarian volume were significantly higher in group A as compared with group B (P<0.001).The expression of GLUT4 in group A was significantly lower than that of group B (1.05±-0.13 vs 1.50±0.21, P<0.001).In group A1,the expression of GLUT4 in endometrium were changed from 1.08±0.08 to 1.27±0.16 before and after treatment (P<0.05).The results of immunohistochemical staining of GLUT4 in endometrium were coincident with the results of RT-PCR.The fast insulin level and the insulin sensitivity index were also improved obviously after three months of metformin therapy (P<0.05).Conclusions: Insulin-resistance status was proved existing in endometrium of PCOS women; A significant improvement of GLUT4 expression was observed in endometrium after metformin treatment, supporting that metformin can relief

  19. Retinal nerve fiber layer thickness changes in obstructive sleep apnea syndrome: one year follow-up results

    Institute of Scientific and Technical Information of China (English)

    Mehmet; Ozgur; Zengin; Ibrahim; Tuncer; Eyyup; Karahan

    2014-01-01

    ·AIM: To investigate the retinal nerve fiber layer(RNFL)thickness changes in patients with obstructive sleep apnea syndrome(OSAS) for one year follow-up. To discuss the possibility of detecting tendency of glaucoma in this population by using spectral domain optical coherence tomography(3D-OCT-2000 Spectral domain).·METHODS: After polysomnographic study, all subjects(64 OSAS patients and 40 controls) underwent detailed ophthalmological examination. After these examinations,patients with glaucoma and patients who had ophthalmological and/or systemic disease were excluded from the study.Totally, 20 patients in OSAS group and five patients in controls were excluded from the study in the first examination and follow-up period. The RNFL thickness was assessed with OCT. Forty-four OSAS patients and 35 control subjects were followed up for12 mo. RNFL thickness change and OSAS patients were evaluated for severity of disease by Apnea-Hypopnea Index(AHI).·RESULTS: Forty-four OSAS patients and 35 controls were enrolled in the study. Statistically significance was found between OSAS patients and controls at the 12 thmo.Average RNFL thickness was found to be significantly lower in last measurements in OSAS patients when compared with first measurements and control subjects(P <0.001, 0.002, respectively). There was a statistically significant correlation among AHI, and RNFL thickness(P <0.05).·CONCLUSION: The results suggest that the patients with OSAS were related with a proportional decrease in the RNFL thickness. These patients should be followed up regularly for glaucomatous changes. Detecting more RNFL thinning in severe OSAS was important.

  20. Metabolic Syndrome

    Science.gov (United States)

    ... If you already have metabolic syndrome, making these healthy lifestyle choices can help reduce your risk of heart disease and other health problems. If lifestyle changes alone can’t control your ... to help. Maintain a healthy weight Your doctor can measure your body mass ...

  1. Baseline brain activity changes in patients with clinically isolated syndrome revealed by resting-state functional MRI

    Energy Technology Data Exchange (ETDEWEB)

    Liu, Yaou; Duan, Yunyun; Liang, Peipeng; Jia, Xiuqin; Yu, Chunshui [Dept. of Radiology, Xuanwu Hospital, Capital Medical Univ., Beijing (China); Ye, Jing [Dept. of Neurology, Xuanwu Hospital, Capital Medical Univ., Beijing (China); Butzkueven, Helmut [Dept. of Medicine, Univ. of Melbourne, Melbourne (Australia); Dong, Huiqing [Dept. of Neurology, Xuanwu Hospital, Capital Medical Univ., Beijing (China); Li, Kuncheng [Dept. of Radiology, Xuanwu Hospital, Capital Medical Univ., Beijing (China); Beijing Key Laboratory of MRI and Brain Informatics, Beijing (China)], E-mail: likuncheng1955@yahoo.com.cn

    2012-11-15

    Background A clinically isolated syndrome (CIS) is the first manifestation of multiple sclerosis (MS). Previous task-related functional MRI studies demonstrate functional reorganization in patients with CIS. Purpose To assess baseline brain activity changes in patients with CIS by using the technique of regional amplitude of low frequency fluctuation (ALFF) as an index in resting-state fMRI. Material and Methods Resting-state fMRIs data acquired from 37 patients with CIS and 37 age- and sex-matched normal controls were compared to investigate ALFF differences. The relationships between ALFF in regions with significant group differences and the EDSS (Expanded Disability Status Scale), disease duration, and T2 lesion volume (T2LV) were further explored. Results Patients with CIS had significantly decreased ALFF in the right anterior cingulate cortex, right caudate, right lingual gyrus, and right cuneus (P < 0.05 corrected for multiple comparisons using Monte Carlo simulation) compared to normal controls, while no significantly increased ALFF were observed in CIS. No significant correlation was found between the EDSS, disease duration, T2LV, and ALFF in regions with significant group differences. Conclusion In patients with CIS, resting-state fMRI demonstrates decreased activity in several brain regions. These results are in contrast to patients with established MS, in whom ALFF demonstrates several regions of increased activity. It is possible that this shift from decreased activity in CIS to increased activity in MS could reflect the dynamics of cortical reorganization.

  2. Changes in Angiotensin Receptor Distribution and in Aortic Morphology Are Associated with Blood Pressure Control in Aged Metabolic Syndrome Rats

    Directory of Open Access Journals (Sweden)

    Verónica Guarner-Lans

    2016-01-01

    Full Text Available The role of the renin-angiotensin system (RAS in blood pressure regulation in MS during aging is unknown. It participates in metabolic syndrome (MS and aging regulating vascular tone and remodeling. RAS might participate in a compensatory mechanism decreasing blood pressure and allowing MS rats to reach 18 months of age and it might form part of therapeutical procedures to ameliorate MS. We studied histological changes and distribution of RAS receptors in aortas of MS aged rats. Electron microscopy images showed premature aging in MS since the increased fibrosis, enlarged endothelium, and invasion of this layer by muscle cells that was present in control 18-month-old aortas were also found in 6-month-old aortas from MS rats. AT1, AT2, and Mas receptors mediate the effects of Ang II and Ang 1-7, respectively. Fluorescence from AT2 decreased with age in control and MS aortas, while fluorescence of AT1 increased in aortas from MS rats at 6 months and diminished during aging. Mas expression increased in MS rats and remained unchanged in control rats. In conclusion, there is premature aging in the aortas from MS rats and the elevated expression of Mas receptor might contribute to decrease blood pressure during aging in MS.

  3. Changes in Angiotensin Receptor Distribution and in Aortic Morphology Are Associated with Blood Pressure Control in Aged Metabolic Syndrome Rats

    Science.gov (United States)

    Guarner-Lans, Verónica; Soria-Castro, Elizabeth; Torrico-Lavayen, Rocío; Patrón-Soberano, Araceli; Carvajal-Aguilera, Karla G.; Castrejón-Tellez, Vicente; Rubio-Ruiz, María Esther

    2016-01-01

    The role of the renin-angiotensin system (RAS) in blood pressure regulation in MS during aging is unknown. It participates in metabolic syndrome (MS) and aging regulating vascular tone and remodeling. RAS might participate in a compensatory mechanism decreasing blood pressure and allowing MS rats to reach 18 months of age and it might form part of therapeutical procedures to ameliorate MS. We studied histological changes and distribution of RAS receptors in aortas of MS aged rats. Electron microscopy images showed premature aging in MS since the increased fibrosis, enlarged endothelium, and invasion of this layer by muscle cells that was present in control 18-month-old aortas were also found in 6-month-old aortas from MS rats. AT1, AT2, and Mas receptors mediate the effects of Ang II and Ang 1-7, respectively. Fluorescence from AT2 decreased with age in control and MS aortas, while fluorescence of AT1 increased in aortas from MS rats at 6 months and diminished during aging. Mas expression increased in MS rats and remained unchanged in control rats. In conclusion, there is premature aging in the aortas from MS rats and the elevated expression of Mas receptor might contribute to decrease blood pressure during aging in MS. PMID:27293881

  4. Saliva amylase as a measure of sympathetic change elicited by autogenic training in patients with functional somatic syndromes.

    Science.gov (United States)

    Kiba, Tadashi; Kanbara, Kenji; Ban, Ikumi; Kato, Fumie; Kawashima, Sadanobu; Saka, Yukie; Yamamoto, Kazumi; Nishiyama, Junji; Mizuno, Yasuyuki; Abe, Tetsuya; Fukunaga, Mikihiko

    2015-12-01

    The aim of this study was to discuss the effect of autogenic training (AT) on patients with functional somatic syndrome (FSS) using salivary amylase, the skin temperature of the finger, subjective severity of symptoms, and psychological characteristics as measures. We assessed 20 patients with FSS and 23 healthy controls before and after AT. Baseline levels of salivary amylase prior to an AT session were significantly higher in the FSS group than in the control group. However, this difference was not significant after AT. The skin temperature of the finger increased after AT in both the FSS and control groups. AT contributed to the improvement of somatic symptoms in patients with FSS. Our results regarding psychological characteristics suggest that mood disturbances are deeply involved in the pathology of FSS. Individuals with FSS exhibited elevated levels of sympathetic activity compared with healthy controls. Our data indicates that AT eased dysregulation of the autonomic nervous system in patients with FSS. Thus, salivary amylase may be a useful index of change induced by AT in patients with FSS. PMID:26219656

  5. Effect of weight loss, independent of change in diet composition, on apolipoprotein AI kinetic in men with metabolic syndrome.

    Science.gov (United States)

    Richard, Caroline; Couture, Patrick; Desroches, Sophie; Lichtenstein, Alice H; Lamarche, Benoît

    2013-01-01

    We investigated the effect of weight loss, independent of change in diet composition, on HDL and apoAI metabolism in men with metabolic syndrome (MetS). Subjects (19 men with MetS [NCEP-ATPIII]) were fed an isoenergetic Mediterranean-style diet for 5 weeks (all foods provided). Participants then underwent a 20-week free-living period during which they were counseled to restrict energy intake, after which they were again fed an isoenergetic Mediterranean-style diet for 5 weeks. At the end of the two controlled diets, participants received a single bolus of [5,5,5-(2)H(3)] (L)-leucine, and fasting blood samples were collected over a 96 h period. ApoAI kinetic was assessed using multicompartmental modeling of the tracer enrichment data. Participants achieved a 9.1 ± 2.8% reduction in body weight (P diet composition, increases plasma HDL primarily by delaying the catabolism of apoAI.

  6. Rapunzel syndrome

    International Nuclear Information System (INIS)

    An 18-year-old single female patient, presented with non specific gastrointestinal symptoms of anorexia, abdominal pain, and change in bowel habit. Clinically she was anemic, cachectic, and depressed. Abdominal examination revealed mobile epigastric mass. The scalp alopecia and endoscopy coupled by computed tomography scan, confirmed the diagnoses of trichobezoar, but it was not diagnosed as Rapunzel syndrome except after laparotomy, gastrotomy, and enterotomy. There are less than 16 cases of Rapunzel syndrome described worldwide, and this is the first case to be described in the middle east. (author)

  7. Turner Syndrome

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    Ramachandran Sudarshan

    2012-08-01

    Full Text Available Turner syndrome is a genetic disorder that affects mostly females. Affected females have characteristic features such as short stature, premature ovarian failure, and several other features. Oral manifestations of this condition are not much discussed in the literature. But reported literature includes teeth, palate, periodontal and salivary changes. So the aim of this review is to illustrate the general manifestations, and especially the oral manifestations of Turner syndrome and evaluate their possible management. [Archives Medical Review Journal 2012; 21(4.000: 246-252

  8. Deciphering the metabolic changes associated with diapause syndrome and cold acclimation in the two-spotted spider mite Tetranychus urticae.

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    Samira Khodayari

    Full Text Available Diapause is a common feature in several arthropod species that are subject to unfavorable growing seasons. The range of environmental cues that trigger the onset and termination of diapause, in addition to associated hormonal, biochemical, and molecular changes, have been studied extensively in recent years; however, such information is only available for a few insect species. Diapause and cold hardening usually occur together in overwintering arthropods, and can be characterized by recording changes to the wealth of molecules present in the tissue, hemolymph, or whole body of organisms. Recent technological advances, such as high throughput screening and quantification of metabolites via chromatographic analyses, are able to identify such molecules. In the present work, we examined the survival ability of diapausing and non-diapausing females of the two-spotted spider mite, Tetranychus urticae, in the presence (0 or 5°C or absence of cold acclimation. Furthermore, we examined the metabolic fingerprints of these specimens via gas chromatography-mass spectrophotometry (GC-MS. Partial Least Square Discriminant Analysis (PLS-DA of metabolites revealed that major metabolic variations were related to diapause, indicating in a clear cut-off between diapausing and non-diapausing females, regardless of acclimation state. Signs of metabolic depression were evident in diapausing females, with most amino acids and TCA cycle intermediates being significantly reduced. Out of the 40 accurately quantified metabolites, seven metabolites remained elevated or were accumulated in diapausing mites, i.e. cadaverine, gluconolactone, glucose, inositol, maltose, mannitol and sorbitol. The capacity to accumulate winter polyols during cold-acclimation was restricted to diapausing females. We conclude that the induction of increased cold hardiness in this species is associated with the diapause syndrome, rather than being a direct effect of low temperature. Our results

  9. [Refeeding syndrome].

    Science.gov (United States)

    Ševela, Stanislav; Novák, František; Kazda, Antonín; Brodská, Helena

    2016-01-01

    Despite being known more than 60 years, refeeding syndrome (RS) still bears many uncertainties. For example, its definition is not clear and definite, and the attitude to it varies from the complete neglect to over-prevention.The term "refeeding syndrome" refers to electrolyte and metabolic changes occurring in malnourished patients after the readministration of nutrition. These changes concern especially to phosphates and ions. Potassium, magnesium, naturism and fluids balance are involved. The changes lead to cell energetic metabolism and electric potential disturbances, with related clinical symptoms.Fully developed refeeding syndrome is quite rare; nevertheless it can be fatal for the patient. However, even its development can lead to many complications increasing the patient's morbidity and the length of stay in the hospital. Yet the refeeding syndrome is more or less predictable and if kept in mind also preventable.The aim of this article is to get the reader to know more about this metabolic phenomenon and possible attitudes towards it. PMID:27088791

  10. Hyponatremic-Hypertensive Syndrome in an 18-Month-Old Male Child

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    Dilek Yilmaz

    2016-04-01

    Full Text Available An eighteen-month-old boy presented with polyuria, polydipsia, hypertension, severe hyponatremia, metabolic alkalosis and nephrotic-range proteinuria. Hypertension was drug resistant. Renal artery angiogram revealed right renal artery occlusion. Nonfunctional right kidney was also detected on the nuclear renal scan. As percutaneous transluminal renal artery angioplasty was not appropriate for our patient, nephrectomy was performed. Right nephrectomy resulted in the resolution of all the symptoms. Rarity of hyponatremic hypertensive syndrome in children may project its under-recognition. We aimed to increase awareness of early diagnosis of HHS among polyuric, hypertensive and hyponatremic children in order to prevent renal damage and life-threatening complications.

  11. A prospective study of the psychobehavioral factors responsible for a change from non-patient irritable bowel syndrome to IBS patient status

    OpenAIRE

    Nomura Shinobu; Fujii Yasushi

    2008-01-01

    Abstract Background To investigate non-patient irritable bowel syndrome (IBS) change to IBS and to determine factors predictive of the onset of IBS, individual biological factors, psychological factors, behavioral factors, and environmental factors were examined. Methods The subjects were 105 non-patient IBS (male = 59, female = 46, average age:21.49 ± 2.37), including 68 of the diarrhea-predominant type and 37 of the constipation-predominant type selected from 1,409 university and technical ...

  12. The effect of dietary changes on distinct components of the metabolic syndrome in a young Sri Lankan population at high risk of CVD.

    Science.gov (United States)

    Guess, Nicola; Wijesuriya, Mahen; Vasantharajah, Laksha; Gulliford, Martin; Viberti, Giancarlo; Gnudi, Luigi; Karalliedde, Janaka

    2016-08-01

    South Asian populations are predisposed to early onset of the metabolic syndrome. Lifestyle intervention programmes have demonstrated a reduction in the metabolic syndrome and CVD risk; however, the most effective components of the multi-faceted lifestyle interventions are unknown. We studied 2637 Sri Lankan males (n 1237) and females (n 1380), with a mean BMI of 23·9 (sd 4·2) kg/m2, aged 22·5 (sd 10·0) years, who had participated in a 5-year lifestyle-modification programme to examine the effect of dietary changes on distinct components of the metabolic syndrome. The dietary intervention comprised advice to replace polished starches with unpolished starches, high-fat meat and dairy products with low-fat products and high-sugar beverages and snacks with low-sugar varieties. For the purposes of this analysis, data from the control and intensive lifestyle groups were combined. Anthropometric and biochemical data were recorded, and a FFQ was completed annually. Multiple regression was used to determine the effect of the dietary changes on distinct components of the metabolic syndrome. The ratio unpolished:polished rice was inversely related to change in fasting glucose (β=-0·084, P=0·007) and TAG (β=-0·084, P=0·005) and positively associated with change in HDL-cholesterol (β=0·066, P=0·031) at the 5-year follow-up after controlling for relevant confounders. Red meat intake was positively associated with fasting glucose concentrations (β=0·05, P=0·017), whereas low-fat (β=-0·046, P=0·018) but not high-fat dairy products (β=0·003, P=0·853) was inversely related to glucose tolerance at the follow-up visit. Replacement of polished with unpolished rice may be a particularly effective dietary advice in this and similar populations. PMID:27358019

  13. Functional and metabolic changes in the brain in neuropathic pain syndrome against the background of chronic epidural electrostimulation of the spinal cord.

    Science.gov (United States)

    Sufianov, A A; Shapkin, A G; Sufianova, G Z; Elishev, V G; Barashin, D A; Berdichevskii, V B; Churkin, S V

    2014-08-01

    Changes in functional and metabolic activities of the brain were evaluated by EEG and positron-emission/computer tomography with 18F-fluorodeoxyglucose in patients with neuropathic pain syndrome previous to and 3 months after implantation of a system for chronic epidural spinal cord stimulation. In most cases, the use of a nerve stimulator was followed by alleviation of neuropathic pain and partial normalization of functional and metabolic activities of brain structures responsible for pain perception, emotiogenic, behavioral, and autonomic responses.

  14. Morphological changes of intestinal mucosa in patients with different clinical variants of irritable bowel syndrome using tetracyclic antidepressants and selective serotonin reuptake inhibitor

    OpenAIRE

    Nagieva S.; Svintsitskyy A.; Kuryk O.; Korendovych I.

    2015-01-01

    Objective. To assess histological changes of colonic mucosa in patients with clinically different types of irritable bowel syndrome (IBS) before and after the treatment with tetracyclic antidepressant and selective serotonin reuptake inhibitor. Methods. Adult patients (over 18 years) with confirmed diagnosis of IBS were examined. Biopsy specimens were taken from colon during colonoscopy for the next histological examination. One expert gastrointestinal pathologist assessed all tissue samples....

  15. Country, sex, EDSS change and therapy choice independently predict treatment discontinuation in multiple sclerosis and clinically isolated syndrome.

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    Claire Meyniel

    Full Text Available OBJECTIVES: We conducted a prospective study, MSBASIS, to assess factors leading to first treatment discontinuation in patients with a clinically isolated syndrome (CIS and early relapsing-remitting multiple sclerosis (RRMS. METHODS: The MSBASIS Study, conducted by MSBase Study Group members, enrols patients seen from CIS onset, reporting baseline demographics, cerebral magnetic resonance imaging (MRI features and Expanded Disability Status Scale (EDSS scores. Follow-up visits report relapses, EDSS scores, and the start and end dates of MS-specific therapies. We performed a multivariable survival analysis to determine factors within this dataset that predict first treatment discontinuation. RESULTS: A total of 2314 CIS patients from 44 centres were followed for a median of 2.7 years, during which time 1247 commenced immunomodulatory drug (IMD treatment. Ninety percent initiated IMD after a diagnosis of MS was confirmed, and 10% while still in CIS status. Over 40% of these patients stopped their first IMD during the observation period. Females were more likely to cease medication than males (HR 1.36, p = 0.003. Patients treated in Australia were twice as likely to cease their first IMD than patients treated in Spain (HR 1.98, p = 0.001. Increasing EDSS was associated with higher rate of IMD cessation (HR 1.21 per EDSS unit, p<0.001, and intramuscular interferon-β-1a (HR 1.38, p = 0.028 and subcutaneous interferon-β-1a (HR 1.45, p = 0.012 had higher rates of discontinuation than glatiramer acetate, although this varied widely in different countries. Onset cerebral MRI features, age, time to treatment initiation or relapse on treatment were not associated with IMD cessation. CONCLUSION: In this multivariable survival analysis, female sex, country of residence, EDSS change and IMD choice independently predicted time to first IMD cessation.

  16. Relation of EEG alpha background to cognitive fuction, brain atrophy, and cerebral metabolism in Down's syndrome. Age-specific changes

    International Nuclear Information System (INIS)

    We studied 19 young adults (19 to 37 years old) and 9 older patients (42 to 66 years old) with Down's syndrome (DS) and a control group of 13 healthy adults (22 to 38 years old) to investigate the relation of electroencephalographic (EEG) alpha background to cognitive function and cerebral metabolism. Four of the older patients with DS had a history of mental deterioration, disorientation, and memory loss and were demented. Patients and control subjects had EEGs, psychometric testing, quantitative computed tomography, and positron emission tomography with fludeoxyglucose F 18. A blinded reader classified the EEGs into two groups--those with normal alpha background or those with abnormal background. All the control subjects, the 13 young adult patients with DS, and the 5 older patients with DS had normal EEG backgrounds. In comparison with the age-matched patients with DS with normal alpha background, older patients with DS with decreased alpha background had dementia, fewer visuospatial skills, decreased attention span, larger third ventricles, and a global decrease in cerebral glucose utilization with parietal hypometabolism. In the young patients with DS, the EEG background did not correlate with psychometric or positron emission tomographic findings, but the third ventricles were significantly larger in those with abnormal EEG background. The young patients with DS, with or without normal EEG background, had positron emission tomographic findings similar to those of the control subjects. The mechanism underlying the abnormal EEG background may be the neuropathologic changes of Alzheimer's disease in older patients with DS and may be cerebral immaturity in younger patients with DS

  17. Auriculotemporal Syndrome (Frey Syndrome).

    Science.gov (United States)

    Motz, Kevin M; Kim, Young J

    2016-04-01

    Frey syndrome is a common sequela of parotidectomy, and although it is not frequently manifested clinically, it can cause significant morbidity for those affected. Frey syndrome results from synkinetic autonomic reinnervation by transected postganglionic parasympathetic nerve fiber within the parotid gland to the overlying sweat glands of the skin. Many surgical techniques have been proposed to prevent the development of Frey syndrome. For those who develop clinical symptoms of Frey syndrome, objective testing can be performed with a Minor starch-iodine test. Some of the current methods to prevent and treat symptomatic Frey syndrome are reviewed. PMID:26902982

  18. The therapeutic effect of monocyte chemoattractant protein-1 delivered by an electrospun scaffold for hyperglycemia and nephrotic disorders

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    Yong C

    2014-02-01

    Full Text Available Cai Yong,2,* Zhengxin Wang,1,* Xing Zhang,3 Xiaomin Shi,1 Zhijia Ni,1 Hong Fu,1 Guoshan Ding,1 Zhiren Fu,1 Hao Yin1,3 1Department of Surgery, Organ Transplant Center, Shanghai Changzheng Hospital, Second Military Medical University, Shanghai, People's Republic of China; 2Department of Transplantation, First Affiliated Hospital of Wenzhou Medical College, Wenzhou, People's Republic of China; 3Department of Surgery, University of Chicago, Chicago, IL, USA *These authors contributed equally to this article Abstract: Here, we investigated in diabetic mice the therapeutic effect of monocyte chemoattractant protein-1 (MCP-1, locally delivered by an electrospun scaffold, on transplanted islets. This therapeutic scheme is expected to exert a synergistic effect to ameliorate hyperglycemia and its associated nephrotic disorders. The cumulative amount of MCP-1 released from the scaffold in vitro within a 3-week window was 267.77±32.18 ng, without a compromise in bioactivity. After 8 weeks following the transplantation, the islet population stimulated by MCP-1 was 35.14%±7.23% larger than the non-stimulated islet population. Moreover, MCP-1 increased concentrations of blood insulin and C-peptide 2 by 49.83%±5.29% and 43.49%±9.21%, respectively. Consequently, the blood glucose concentration in the MCP-1 group was significantly lower than that in the control group at week 2 post-surgery. MCP-1 also enhanced the tolerance of sudden oral glucose challenge. The rapid decrease of blood creatinine, urine creatinine, and blood urea nitrogen suggested that the recovery of renal functions compromised by hyperglycemia could also be attributed to MCP-1. Our study shed new light on a synergistic strategy to alleviate hyperglycemia and nephrotic disorders in diabetic patients. Keywords: MCP-1, electrospinning, islet transplantation, diabetes

  19. Changes in small intestinal chromogranin A-immunoreactive cell densities in patients with irritable bowel syndrome after receiving dietary guidance.

    Science.gov (United States)

    Mazzawi, Tarek; El-Salhy, Magdy

    2016-05-01

    Chromogranin A (CgA) is a common marker for enteroendocrine cells in the gut, and CgA-immunoreactive cell densities are abnormal in patients with irritable bowel syndrome (IBS). The majority of patients with IBS report that their symptoms develop after consuming certain foodstuffs. In the present study, we investigated the effects of dietary guidance on the total enteroendocrine cell densities in the small intestine, as detected by CgA. A total of 14 patients with IBS underwent a gastroscopy with duodenal biopsies and 11 of them also underwent a colonoscopy, with biopsy samples obtained from the ileum. Fourteen control subjects were also included. Each patient received 3 sessions of dietary guidance. Gastroscopies and colonoscopies were performed on both the controls and patients with IBS (at baseline and at 3-9 months after receiving guidance). Biopsy samples obtained from the duodenum and ileum were immunostained for CgA using the avidin-biotin complex (ABC) method and were quantified using computerized image analysis. The density of CgA-immunoreactive cells in the duodenum (mean ± SEM values) in the control subjects was 235.9 ± 31.9 cells/mm2; in the patients with IBS, the density was 36.9 ± 9.8 and 103.7 ± 16.9 cells/mm2 before and after they received dietary guidance, respectively (P=0.007). The density of CgA-immunoreactive cells in the ileum in the control subjects was 47.4 ± 8.3 cells/mm2; in the patients with IBS, the density was 48.4 ± 8.1 and 17.9 ± 4.4 cells/mm2, before and after they received dietary guidance, respectively (P=0.0006). These data indicate that changes in CgA-immunoreactive cell densities in patients with IBS after receiving dietary guidance may reflect a change in the densities of the small intestinal enteroendocrine cells, which may contribute to an improvement in the IBS symptoms. PMID:26987104

  20. "Scleroderma linearis: hemiatrophia faciei progressiva (Parry-Romberg syndrom without any changes in CNS and linear scleroderma "en coup de sabre" with CNS tumor

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    Brzezińska-Wcisło Ligia

    2009-07-01

    Full Text Available Abstract Background Hemifacial atrophy (Parry-Romberg syndrome is a relatively rare disease. The etiology of the disease is not clear. Some authors postulate its relation with limited scleroderma linearis. Linear scleroderma "en coup de sabre" is characterized by clinical presence of most commonly one-sided linear syndrome. In a number of patients, neurological affection is the medium of the disease. The treatment of both scleroderma varieties is similar to the treatment of limited systemic sclerosis. Case presentation We present two cases of a disease: a case of a 49-year-old woman with a typical image of hemifacial atrophy, without any changes of the nervous system and a case of a 33-year-old patient with an "en coup de sabre" scleroderma and with CNS tumor. Conclusion We described typical cases of a rare diseases, hemifacial atrophy and "en coup de sabre" scleroderma. In the patient diagnosed with Parry-Romberg syndrome, with Borrelia burgdoferi infection and with minor neurological symptoms, despite a four-year case history, there was a lack of proper diagnosis and treatment. In the second patient only skin changes without any neurological symptoms could be observed and only a precise neurological diagnosis revealed the presence of CNS tumor.

  1. Clinical significance of changes of plasma leptin and serum T, E2, FSH, LH, PRL levels in patients with polycystic ovary syndrome (PCOS)

    International Nuclear Information System (INIS)

    Objective: To explore the clinical significance of changes of plasma leptin and serum T, E2, FSH, LH, PRL levels in patients with polycystic ovary syndrome(PCOS). Methods: Plasma leptin (with RIA), serum T, E2, FSH, LH, PRL (with ECLIA) levels were detected in 31 patients with polycystic ovary syndrome(PCOS) as well as in 35 controls. Results: The plasma leptin and serum T, LH, PRL levels in the patients with PCOS were significantly higher than those in controls (P2, FSH levels were not significantly different from those in controls (P>0.05). Plasma leptin levels were positively correlated with those of serum T, LH, PRL (r=0.5784, 0.5411, 0.6082, P<0.01). Conclusion: Development of PCOS was closely related to the plasma leptin and serum T, LH, PRL levels. (authors)

  2. Minimal Change Disease and IgA Deposition: Separate Entities or Common Pathophysiology?

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    Brandon S. Oberweis

    2013-01-01

    Full Text Available Introduction. Minimal Change Disease (MCD is the most common cause of nephrotic syndrome in children, while IgA nephropathy is the most common cause of glomerulonephritis worldwide. MCD is responsive to glucocorticoids, while the role of steroids in IgA nephropathy remains unclear. We describe a case of two distinct clinical and pathological findings, raising the question of whether MCD and IgA nephropathy are separate entities or if there is a common pathophysiology. Case Report. A 19-year old man with no medical history presented to the Emergency Department with a 20-day history of anasarca and frothy urine, BUN 68 mg/dL, Cr 2.3 mg/dL, urinalysis 3+ RBCs, 3+ protein, and urine protein : creatinine ratio 6.4. Renal biopsy revealed hypertrophic podocytes on light microscopy, podocyte foot process effacement on electron microscopy, and immunofluorescent mesangial staining for IgA. The patient was started on prednisone and exhibited dramatic improvement. Discussion. MCD typically has an overwhelming improvement with glucocorticoids, while the resolution of IgA nephropathy is rare. Our patient presented with MCD with the uncharacteristic finding of hematuria. Given the improvement with glucocorticoids, we raise the question of whether there is a shared pathophysiologic component of these two distinct clinical diseases that represents a clinical variant.

  3. Schistosomal glomerulopathy and changes in the distribution of histological patterns of glomerular diseases in Bahia, Brazil

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    Washington Luis Conrado dos-Santos

    2011-11-01

    Full Text Available Distinct patterns of glomerular lesions, including membranoproliferative glomerulonephritis and focal segmental glomerulosclerosis, are associated with infection by Schistosoma mansoni or Schistosoma japonicum. Evidence suggests that immune complex deposition is the main mechanism underlying the different forms of schistosomal glomerulonephritis and that immune complex deposition may be intensified by portal hypertension. The relationship between focal segmental glomerulosclerosis and schistosomiasis remains poorly understood. A clinicopathologic classification of schistosomal glomerulopathies was proposed in 1992 by the African Association of Nephrology. In Brazil, mass treatment with oral medications has led to a decrease in the occurrence of schistosomal glomerulopathy. In a survey of renal biopsies performed in Salvador, Brazil, from 2003-2009, only 24 (4% patients were identified as positive for S. mansoni infection. Among these patients, only one had the hepatosplenic form of the disease. Focal segmental glomerulosclerosis was found in seven patients and membranoproliferative glomerulonephritis was found in four patients. Although retrospective studies on the prevalence of renal diseases based on kidney biopsies may be influenced by many patient selection biases, a change in the distribution of glomerulopathies associated with nephrotic syndrome was observed along with a decline in the occurrence of severe forms of schistosomiasis.

  4. Changing the facial features of patients with Treacher Collins syndrome: protocol for 3-stage treatment of hard and soft tissue hypoplasia in the upper half of the face.

    Science.gov (United States)

    Mitsukawa, Nobuyuki; Saiga, Atsuomi; Satoh, Kaneshige

    2014-07-01

    Treacher Collins syndrome is a disorder characterized by various congenital soft tissue anomalies involving hypoplasia of the zygoma, maxilla, and mandible. A variety of treatments have been reported to date. These treatments can be classified into 2 major types. The first type involves osteotomy for hard tissue such as the zygoma and mandible. The second type involves plastic surgery using bone grafting in the malar region and soft tissue repair of eyelid deformities. We devised a new treatment to comprehensively correct hard and soft tissue deformities in the upper half of the face of Treacher Collins patients. The aim was to "change facial features and make it difficult to tell that the patients have this disorder." This innovative treatment strategy consists of 3 stages: (1) placement of dermal fat graft from the lower eyelid to the malar subcutaneous area, (2) custom-made synthetic zygomatic bone grafting, and (3) Z-plasty flap transposition from the upper to the lower eyelid and superior repositioning and fixation of the lateral canthal tendon using a Mitek anchor system. This method was used on 4 patients with Treacher Collins syndrome who had moderate to severe hypoplasia of the zygomas and the lower eyelids. Facial features of these patients were markedly improved and very good results were obtained. There were no major complications intraoperatively or postoperatively in any of the patients during the series of treatments. In synthetic bone grafting in the second stage, the implant in some patients was in the way of the infraorbital nerve. Thus, the nerve was detached and then sutured under the microscope. Postoperatively, patients had almost full restoration of sensory nerve torpor within 5 to 6 months. We devised a 3-stage treatment to "change facial features" of patients with hypoplasia of the upper half of the face due to Treacher Collins syndrome. The treatment protocol provided a very effective way to treat deformities of the upper half of the face

  5. Contribution of regional cerebral blood flow changes on the evaluation of neuropsychological impairment in patients with Tourette syndrome

    International Nuclear Information System (INIS)

    Aims: The literature to date has suggested that intellectual ability is normally distributed in Tourette syndrome (TS). However, patients with TS have significant discrepancies between their verbal and non verbal abilities. Specific cognitive deficits in TS include visuomotor integration problems, impaired fine motor skill, and executive dysfunction. The presence of a learning disability or cognitive deficit may lead to a greater obstacle for patients with TS than the tic disorder itself. It would be important that patients with TS will be evaluated to having neuropsychological difficulties as soon as possible. The aim of this study was to evaluate the correlation between the clinical severity and neuropsychological impairment (especially memory deficit) of patients with TS and rCBF abnormalities. Methods: The assessment included neurological, psychiatrical examination (Yale Scale [YS], staging of the severity of disease), CT/MRI, and a specific test of explicit and implicit memory (Probabilistic Classification Learning test, PCL). 99mTc-HMPAO-SPECT studies were carried out with a standard technique for each patient. The data were analyzed visually and by a special ROIs program. RCBF data were correlated to the results of the PCL test and the YS. Results: The SPECT measurements showed significant (p40 in the temporal medial and lateral regions, and also in the cerebellum, compared to the patients with YS<40. Also, an increase of rCBF was observed in the left striatal, temporal medial and lateral regions, compared to the contralateral homologous regions (p<0.05). The YS (disease severity) demonstrated significant (p<0.05) and strong correlation with the rCBF in the right temporal region (r= -0.83) and in the thalamus (r=-0.69). The performance in the explicit memory test showed significant (p<0.05) and strong correlation with the perfusion in both temporopolar (r=0.9) and left temporomedial region (r=0.92). Conclusion: RCBF SPECT proved to be concordant with disease

  6. Changes in Gut and Plasma Microbiome following Exercise Challenge in Myalgic Encephalomyelitis/Chronic Fatigue Syndrome (ME/CFS.

    Directory of Open Access Journals (Sweden)

    Sanjay K Shukla

    Full Text Available Myalgic encephalomyelitis/chronic fatigue syndrome (ME/CFS is a disease characterized by intense and debilitating fatigue not due to physical activity that has persisted for at least 6 months, post-exertional malaise, unrefreshing sleep, and accompanied by a number of secondary symptoms, including sore throat, memory and concentration impairment, headache, and muscle/joint pain. In patients with post-exertional malaise, significant worsening of symptoms occurs following physical exertion and exercise challenge serves as a useful method for identifying biomarkers for exertion intolerance. Evidence suggests that intestinal dysbiosis and systemic responses to gut microorganisms may play a role in the symptomology of ME/CFS. As such, we hypothesized that post-exertion worsening of ME/CFS symptoms could be due to increased bacterial translocation from the intestine into the systemic circulation. To test this hypothesis, we collected symptom reports and blood and stool samples from ten clinically characterized ME/CFS patients and ten matched healthy controls before and 15 minutes, 48 hours, and 72 hours after a maximal exercise challenge. Microbiomes of blood and stool samples were examined. Stool sample microbiomes differed between ME/CFS patients and healthy controls in the abundance of several major bacterial phyla. Following maximal exercise challenge, there was an increase in relative abundance of 6 of the 9 major bacterial phyla/genera in ME/CFS patients from baseline to 72 hours post-exercise compared to only 2 of the 9 phyla/genera in controls (p = 0.005. There was also a significant difference in clearance of specific bacterial phyla from blood following exercise with high levels of bacterial sequences maintained at 72 hours post-exercise in ME/CFS patients versus clearance in the controls. These results provide evidence for a systemic effect of an altered gut microbiome in ME/CFS patients compared to controls. Upon exercise challenge, there

  7. Change of teicoplanin loading dose requirement for incremental increases of systemic inflammatory response syndrome score in the setting of sepsis.

    Science.gov (United States)

    Nakano, Takafumi; Nakamura, Yoshihiko; Takata, Tohru; Irie, Keiichi; Sano, Kazunori; Imakyure, Osamu; Mishima, Kenichi; Futagami, Koujiro

    2016-08-01

    Background Target trough concentrations are recommended for teicoplanin (TEIC) to minimize its adverse effects and to maximize efficacy in sepsis caused by grampositive cocci, including methicillin-resistant Staphylococcus aureus infection. However, optimal doses to attain proper trough values in patients with sepsis have not yet been well established for TEIC. Objective This study investigated whether the systemic inflammatory response syndrome (SIRS) score could predict the pharmacokinetics of TEIC in patients with sepsis. Setting This study was conducted at Fukuoka University Hospital in Japan. Methods We retrospectively reviewed the records of patients using TEIC between April 2012 and March 2015. SIRS positive was defined as infection with a SIRS score ≥2. Estimates of pharmacokinetic parameters were calculated using a Bayesian method. Creatinine clearance rates were estimated by the Cockcroft-Gault formula (eCcr). Main outcome measure Change of TEIC loading dose requirement for incremental increases of SIRS score. Results In total, 133 patients were enrolled: 50 non-SIRS patients and 83 patients with SIRS. The TEIC plasma trough concentration was significantly lower in SIRS than non-SIRS patients (15.7 ± 7.1 vs. 20.1 ± 8.6 μg/mL; P < 0.01), although there was no significant difference in the loading dose administered. Moreover, SIRS scores were increasingly predictive of eCcr and TEIC clearance in a stepwise manner. To achieve the target trough concentration (15-30 μg/mL), the optimal doses required in non-SIRS versus SIRS patients were 12-24 versus 18-30 mg/kg/day, respectively, during the first 48 h. Conclusions These findings suggest that the pharmacokinetics of TEIC are altered in SIRS patients, who required higher doses than non-SIRS patients to achieve the target trough concentration. We suggest that the SIRS score can become a new modality to determine the initial TEIC loading dose. PMID:27125378

  8. Robinow Syndrome

    Directory of Open Access Journals (Sweden)

    Gökhan Gökalp

    2010-05-01

    Full Text Available Introduction: Robinow syndrome is characterized by dwarfism demonstrating short-limbed extremities, vertebral malsegmentation/malformation (hemivertebra, costal dysplasia, genital hypoplasia, and fetal facial appearance (wide and prominent forehead, hypertelorism, small and wide nose, molar hypoplasia, and retrognathia. It is a rare genetic disease which may present with either mild autosomal dominant form or severe recessive form. Vertebral and costal abnormalities are common diagnostic signs that may be severe. The disease presents with kyphoscoliosis and chest abnormalities along with thoracic vertebral fusion and hemivertebral appearance. Ribs may demonstrate fusion. Based on those involvements, the disease can be categorized as spondylothoracic, spondylocostal, ischiovertebral dysplasia, and cervicofaciothoracic syndrome.Diagnosis is established by the help of clinical characteristics. Radiography might contribute to the diagnosis by revealing changes in the skeletal system. Case Report: A three-year-old male patient presented with operated left undescendent testis and buried penis. On physical examination, he also had a dysmorphic face characterized by macrocephaly, hypertelorism, prominent eyes, a flattened nasal bridge, triangular-fish mouth, gingival hypertrophy and left hand clinodactyly. Radiographic examination documented mesomelic shortening of the radius-ulna, malsegmentation of the thoracal spine and the ribs fusion.Conclusion: Robinow syndrome is a rare syndrome which can be diagnosed by typical facial appearance and radiologic findings. (Journal of Current Pediatrics 2010; 8: 44-7

  9. Language Development in Infants and Toddlers with Fragile X Syndrome: Change over Time and the Role of Attention

    Science.gov (United States)

    Kover, Sara T.; McCary, Lindsay M.; Ingram, Alexandra M.; Hatton, Deborah D.; Roberts, Jane E.

    2015-01-01

    Fragile X syndrome (FXS) is associated with significant language and communication delays, as well as problems with attention. This study investigated early language abilities in infants and toddlers with FXS (n = 13) and considered visual attention as a predictor of those skills. We found that language abilities increased over the study period of…

  10. Does Cardiac Rehabilitation After an Acute Cardiac Syndrome Lead to Changes in Physical Activity Habits? Systematic Review

    NARCIS (Netherlands)

    ter Hoeve, Nienke; Huisstede, Bionka M. A.; Stam, Henk J.; van Domburg, Ron T.; Sunamura, Madoka; van den Berg-Emons, Rita J. G.

    2015-01-01

    Background. Optimal physical activity levels have health benefits for patients with acute coronary syndrome (ACS) and are an important goal of cardiac rehabilitation (CR). Purpose. The purpose of this study was to systematically review literature regarding short-term effects (= 6 months after comple

  11. Country, Sex, EDSS Change and Therapy Choice Independently Predict Treatment Discontinuation in Multiple Sclerosis and Clinically Isolated Syndrome

    NARCIS (Netherlands)

    Meyniel, Claire; Spelman, Timothy; Jokubaitis, Vilija G.; Trojano, Maria; Izquierdo, Guillermo; Grand'Maison, Francois; Oreja-Guevara, Celia; Boz, Cavit; Lugaresi, Alessandra; Girard, Marc; Grammond, Pierre; Iuliano, Gerardo; Fiol, Marcela; Antonio Cabrera-Gomez, Jose; Fernandez-Bolanos, Ricardo; Giuliani, Giorgio; Lechner-Scott, Jeannette; Cristiano, Edgardo; Herbert, Joseph; Petkovska-Boskova, Tatjana; Bergamaschi, Roberto; van Pesch, Vincent; Moore, Fraser; Vella, Norbert; Slee, Mark; Santiago, Vetere; Barnett, Michael; Havrdova, Eva; Young, Carolyn; Sirbu, Carmen-Adella; Tanner, Mary; Rutherford, Michelle; Butzkueven, Helmut

    2012-01-01

    Objectives: We conducted a prospective study, MSBASIS, to assess factors leading to first treatment discontinuation in patients with a clinically isolated syndrome (CIS) and early relapsing-remitting multiple sclerosis (RRMS). Methods: The MSBASIS Study, conducted by MSBase Study Group members, enro

  12. Changes in yearly birth prevalence rates of children with Down syndrome in the period 1986-2007 in the Netherlands

    NARCIS (Netherlands)

    de Graaf, G.; Hochstenbach, R.; Engelen, J.; Gerssen-Schoorl, K.; Poddighe, P.; Smeets, D.; van Hove, G.; Haveman, M.

    2011-01-01

    Background The Netherlands are lacking reliable national empirical data in relation to the development of birth prevalence of Down syndrome. Our study aims at assessing valid national live birth prevalence rates for the period 1986-2007. Method On the basis of the annual child/adult ratio of Down sy

  13. Urodynamic changes in a female case of obstructive sleep apnea syndrome with enuresis: 7 years' follow-up

    Institute of Scientific and Technical Information of China (English)

    CAO Xia; HU Ke; CHEN Xue-qin; XIANYU Yun-yan; Lü Sheng-qi; LI Qing-quan

    2010-01-01

    @@ Obstructive sleep apnea syndrome (OSAS) is characterized by repetitive upper airway occlusion resulting in apnea lasting 10 seconds or more. Clinical manifestations include snoring, daytime somnolence,intellectual deficiency, sexual impotence, and nocturnal polyuria. Enuresis associated with OSAS is suggested to be more common in children than in adults.1

  14. DSM-5 Changes and the Prevalence of Parent-Reported Autism Spectrum Symptoms in Fragile X Syndrome

    Science.gov (United States)

    Wheeler, Anne C.; Mussey, Joanna; Villagomez, Adrienne; Bishop, Ellen; Raspa, Melissa; Edwards, Anne; Bodfish, James; Bann, Carla; Bailey, Donald B.

    2015-01-01

    We used survey methodology to assess parent-reported autism symptomology in 758 individuals (639 males; 119 females) with fragile X syndrome (FXS). Caregivers reported whether their child with FXS had been diagnosed with an autism spectrum disorder (ASD) and endorsed symptoms based on a list of observable behaviors related to ASD diagnoses.…

  15. [Bilateral tibial compartment syndrome with crush symptoms after cannabis abuse].

    Science.gov (United States)

    Huber, F X; Eckstein, H H; Allenberg, J R

    1997-08-01

    Acute compartmental syndrome (CS) is a surgical emergency. Different conditions in which high non-physiological pressure appears within a closed fascial space reduce the necessary blood perfusion. CS is caused by trauma, burns, bleeding in patients with coagulopathies arterial injuries, nephrotic syndrome or unusual physical exercise with secondary compartmental swelling. When decompression occurs too late, permanent loss of function and limb contracture may result. In the following paper we report on a case of four-compartmental syndrome in the lower legs of a patient with drug intoxication. After cannabis consumption, the patient fell asleep sitting cross-legged. During our first examination several hours later, the signs of compartmental syndrome with spontaneous pain, turgid swelling and paresis were present. Bilateral skin incision technique was used to gain entrance into the four compartments in both lower legs. Immediately after the operation, the patient showed crush syndrome with high serum creatine kinase activity 140.501 U/l and acute renal failure caused by rhabdomyolysis. Within 2 weeks of haemofiltration and dialysis, a full recovery to a normal serum creatinine level of 0.7 mg/dl was achieved. After emergency treatment and rehabilitation, the patient showed neither vascular nor neural defects. PMID:9377997

  16. Natural dipeptidyl peptidase-IV inhibitor mangiferin mitigates diabetes- and metabolic syndrome-induced changes in experimental rats

    Science.gov (United States)

    Suman, Rajesh Kumar; Mohanty, Ipseeta Ray; Maheshwari, Ujwala; Borde, Manjusha K; Deshmukh, YA

    2016-01-01

    Background Mangiferin (MNG) is known to possess antidiabetic and antioxidant activity. However, there is no experimental evidence presently available in the literature with regard to its ameliorating effects on diabetes mellitus coexisting with metabolic syndrome. Objective The present study was designed to evaluate the protective effects of MNG on various components of metabolic syndrome with diabetes as an essential component. Material and methods Adult Wistar rats were fed high-fat diets for 10 weeks and challenged with streptozotocin (40 mg/kg) at week three (high-fat diabetic control group). After the confirmation of metabolic syndrome in the setting of diabetes, MNG 40 mg/kg was orally fed to these rats from the fourth to tenth week. Results The treatment with MNG showed beneficial effects on various components of metabolic syndrome, such as reduced dyslipidemia (decreased triglyceride, total cholesterol, low-density lipoprotein cholesterol, and increased high-density lipoprotein cholesterol) and diabetes mellitus (reduced blood glucose and glycosylated hemoglobin). In addition, an increase in serum insulin, C-peptide, and homeostasis model assessment-β and a reduction in homeostasis model assessment of insulin resistance-IR were observed in MNG-treated group compared with high-fat diabetic control group. MNG was also found to be cardioprotective (reduction in creatine phosphokinase-MB levels, atherogenic index, high-sensitivity C-reactive protein). Reduction in serum dipeptidyl peptidase–IV levels in the MNG-treated group correlated with improvement in insulin resistance and enhanced β-cell function. Conclusion The present study has demonstrated antidiabetic, hypolipidemic, and cardioprotective effects of MNG in the setting of diabetes with metabolic syndrome. Thus, MNG has the potential to be developed as a natural alternative to synthetic dipeptidyl peptidase-IV inhibitors beneficial in this comorbid condition. PMID:27621658

  17. Progressive cerebellar degenerative changes in the severe mental retardation syndrome caused by duplication of MECP2 and adjacent loci on Xq28.

    LENUS (Irish Health Repository)

    Reardon, William

    2010-08-01

    Localised duplications, involving the MECP2 locus, at Xq28 have been associated with a syndrome comprising X-linked mental retardation, hypotonia and recurrent infections in males. We now present neuroradiological evidence that progressive cerebellar degenerative changes may also be a consistent feature of this syndrome, emerging in the second decade of life. We report seven affected males, from three different families who, in addition to the previously described clinical findings, have a reduction in the volume of the white matter and mild dilatation of the lateral ventricles. Three of the older patients show a consistent cerebellar degenerative phenotype. Furthermore, we describe the first female affected with the disorder. The female was mildly affected and shows X-inactivation in the ratio of 70:30, demonstrating that X-inactivation cannot be exclusively relied upon to spare the female carriers from symptoms. In conclusion, there is a radiological phenotype associated with Xq28 duplication which clearly demonstrates progressive degenerative cerebellar disease as part of the syndrome.

  18. Bi Syndrome (Arthralgia Syndrome)

    Institute of Scientific and Technical Information of China (English)

    ZHANG En-qin

    2010-01-01

    @@ The word 'Bi' (痹) in Chinese means an obstruction.Bi Syndrome refers the syndrome characterized by the obstruction of qi and blood in the meridians due to the invasion of external pathogenic wind, cold and dampness, manifested as soreness, pain, numbness,heavy sensation, swelling of joints and limbs, limitation of movements and so on.

  19. Changes in the epidemiological pattern of sudden infant death syndrome in southeast Norway, 1984-1998: implications for future prevention and research

    OpenAIRE

    Arnestad, M; Andersen, M.; Vege, A; Rognum, T

    2001-01-01

    AIM—To look for changes in risk factors for sudden infant death syndrome (SIDS) after decrease and stabilisation of the SIDS rate.
METHODS—Questionnaires were distributed to parents of 174 SIDS infants, dying between 1984 and 1998, and 375 age and sex matched controls in southeast Norway.
RESULTS—The proportion of infants sleeping prone has decreased, along with the decrease in SIDS rate for the region during the periods studied, but over half of the SIDS victims are stil...

  20. Changes in dead space/tidal volume ratio and pulmonary mechanics after surfactant replacement therapy in respiratory distress syndrome of the newborn infants.

    OpenAIRE

    Chung, E. H.; Ko, S. Y.; Kim, I Y; Chang, Y. S.; Park, W. S.

    2001-01-01

    This study was performed to elucidate the mechanism of improved oxygenation after surfactant replacement therapy in respiratory distress syndrome (RDS) of the newborn infants. In 26 newborns with RDS, end tidal-CO2 tension (PetCO2), arterial blood gas analysis and pulmonary function tests were measured at baseline, 30 min, 2 hr and 6 hr after surfactant administration. The changes in dead space/tidal volume ratio (VD/VT ratio=(PaCO2-PetCO2)/PaCO2), oxygenation index and arterial-alveolar part...

  1. An Exploratory Analysis of Dynamic Change of Metabolic Syndrome in Relation to the Risk of Developing Cardiovascular Disease in a Chinese Cohort

    Directory of Open Access Journals (Sweden)

    C Yang

    2012-04-01

    Full Text Available Background: Metabolic syndrome (MS is the syndrome closely related to cardiovascular disease (CVD risk factors. Few prospective studies have compared the impact of dynamic changes of MS on the development of cardiovascular diseases (CVD.Methods: Overall, 3461 subjects were recruited from a cohort study on Prevention of Multiple Metabolic disorders and MS in Jiangsu of China (PMMJS with a follow up of 3.8 years. The associations between the dynamic changes (Difference, the value at first follow-up subtract the value at baseline of MS, component numbers, components and relative risk (RR of CVD were analyzed by using Cox regression model.Results: The total incidence standardized rate of CVD was 2.58%,and the incidence standardized rates of CVD in MS-/follow-up MS-,baseline MS-/follow-up MS+, baseline MS+/follow-up MS- and baseline MS+/follow-up groups were 2.05%,5.01%,1.65% and 4.39% separately. After adjustment confounding factors Difference in FPG, BP and TG have significantly effects on the incidence of CVD.Conclusion: Difference of MS component numbers had the prediction ability of CVD, but MS groups based on baseline and first follow-up MS and/or non-MS had not. In Chinese, the dynamic change of MS component numbers was a useful predict factor for CVD.

  2. Natural dipeptidyl peptidase-IV inhibitor mangiferin mitigates diabetes- and metabolic syndrome-induced changes in experimental rats

    Directory of Open Access Journals (Sweden)

    Suman RK

    2016-08-01

    Full Text Available Rajesh Kumar Suman,1 Ipseeta Ray Mohanty,1 Ujwala Maheshwari,2 Manjusha K Borde,1 YA Deshmukh1 1Department of Pharmacology, 2Department of Pathology, MGM Medical College, Kamothe, Navi Mumbai, India Background: Mangiferin (MNG is known to possess antidiabetic and antioxidant activity. However, there is no experimental evidence presently available in the literature with regard to its ameliorating effects on diabetes mellitus coexisting with metabolic syndrome.Objective: The present study was designed to evaluate the protective effects of MNG on various components of metabolic syndrome with diabetes as an essential component.Material and methods: Adult Wistar rats were fed high-fat diets for 10 weeks and challenged with streptozotocin (40 mg/kg at week three (high-fat diabetic control group. After the confirmation of metabolic syndrome in the setting of diabetes, MNG 40 mg/kg was orally fed to these rats from the fourth to tenth week.Results: The treatment with MNG showed beneficial effects on various components of metabolic syndrome, such as reduced dyslipidemia (decreased triglyceride, total cholesterol, low-density lipoprotein cholesterol, and increased high-density lipoprotein cholesterol and diabetes mellitus (reduced blood glucose and glycosylated hemoglobin. In addition, an increase in serum insulin, C-peptide, and homeostasis model assessment-β and a reduction in homeostasis model assessment of insulin resistance-IR were observed in MNG-treated group compared with high-fat diabetic control group. MNG was also found to be cardioprotective (reduction in creatine phosphokinase-MB levels, atherogenic index, high-sensitivity C-reactive protein. Reduction in serum dipeptidyl peptidase--IV levels in the MNG-treated group correlated with improvement in insulin resistance and enhanced β-cell function.Conclusion: The present study has demonstrated antidiabetic, hypolipidemic, and cardioprotective effects of MNG in the setting of diabetes with

  3. Syndrome in question*

    Science.gov (United States)

    Peruzzo, Juliano; Nazar, Fernanda Luca; Tubone, Mariana Quirino; Escobar, Gabriela Fortes; Cestari, Tania Ferreira

    2015-01-01

    Waardenburg syndrome is an inherited disease characterized by sensorineural hearing loss, pigmentation changes and minor facial malformations. It has four clinical variants. We report the case of a girl who, like her mother, was affected by this syndrome. The diagnosis was made after detection and treatment of deafness. PMID:26375234

  4. Syndrome in Question.

    Science.gov (United States)

    Peruzzo, Juliano; Nazar, Fernanda Luca; Tubone, Mariana Quirino; Escobar, Gabriela Fortes; Cestari, Tania Ferreira

    2015-01-01

    Waardenburg syndrome is an inherited disease characterized by sensorineural hearing loss, pigmentation changes and minor facial malformations. It has four clinical variants. We report the case of a girl who, like her mother, was affected by this syndrome. The diagnosis was made after detection and treatment of deafness. PMID:26375234

  5. Syndrome in question*

    OpenAIRE

    Peruzzo, Juliano; Nazar, Fernanda Luca; Tubone, Mariana Quirino; Escobar, Gabriela Fortes; Cestari, Tania Ferreira

    2015-01-01

    Waardenburg syndrome is an inherited disease characterized by sensorineural hearing loss, pigmentation changes and minor facial malformations. It has four clinical variants. We report the case of a girl who, like her mother, was affected by this syndrome. The diagnosis was made after detection and treatment of deafness.

  6. Rothmund - Thomson Syndrome

    Directory of Open Access Journals (Sweden)

    Sharma N. L

    2003-01-01

    Full Text Available Rothmund-Thomson syndrome is a rare geno-photodermatosis of children. Poikilodermatous cutaneous changes, growth retardation, juvenile cataract and high incidence of malignancy are its classical features. A Thomson type of Rothmund-Thomson syndrome with characteristic poikiloderma congenitale, growth retardation, absence of juvenile cataract and parental non-consanguinity is described in an 8 year old Indian girl.

  7. Changes in small intestinal chromogranin A-immunoreactive cell densities in patients with irritable bowel syndrome after receiving dietary guidance

    OpenAIRE

    Mazzawi, Tarek; El-Salhy, Magdy

    2016-01-01

    Chromogranin A (CgA) is a common marker for enteroendocrine cells in the gut, and CgA-immunoreactive cell densities are abnormal in patients with irritable bowel syndrome (IBS). The majority of patients with IBS report that their symptoms develop after consuming certain foodstuffs. In the present study, we investigated the effects of dietary guidance on the total enteroendocrine cell densities in the small intestine, as detected by CgA. A total of 14 patients with IBS underwent a gastroscopy ...

  8. Histopathological changes in exocrine glands of murine transplantation chimeras. II: Sjögren's syndrome-like exocrinopathy in mice without lupus nephritis. A model of primary Sjögren's syndrome

    DEFF Research Database (Denmark)

    Ussing, Anne Phaff; Prause, J.U.; Sørensen, Inger;

    1992-01-01

    Autoimmune disease, primary Sjögren's syndrome, transplantation chimeras, experimental model, exocrinopathy, inbred mouse strains......Autoimmune disease, primary Sjögren's syndrome, transplantation chimeras, experimental model, exocrinopathy, inbred mouse strains...

  9. Membranous nephropathy in the cat: a clinical and pathological study.

    Science.gov (United States)

    Nash, A S; Wright, N G; Spencer, A J; Thompson, H; Fisher, E W

    1979-07-28

    A series of 13 cases of feline membranous nephropathy is presented. Two groups were distinguished clinically; eight cats had the nephrotic syndrome and five others were in renal failure but not nephrotic. The definitive diagnosis was based on histological, immunofluorescence and ultrastructural examinations of renal tissue obtained at renal biopsy or necropsy. Glomerular lesions were classified according to the degree of glomerular change into three distinct groups; mild, moderately severe and advanced. A relationship was established between the mild and moderately severe groups and cats with the nephrotic syndrome, and the advanced group and cats in renal failure. Diuretic therapy was satisfactory in initial control of oedema in the nephrotic cases. Monitoring of previously nephrotic cats for up to three years indicated that the disease is progressive, although in some cases it is sufficiently slow for a cat to live a relatively normal life without continuing treatment. The prognosis for cats presented in renal failure is hopeless. PMID:552741

  10. Marfan Syndrome

    Science.gov (United States)

    Marfan syndrome is a disorder that affects connective tissue. Connective tissues are proteins that support skin, bones, ... fibrillin. A problem with the fibrillin gene causes Marfan syndrome. Marfan syndrome can be mild to severe, ...

  11. Gallium-68 DOTA-TATE Positron Emission Tomography/Computed Tomography: Scintigraphic Changes of Adrenal Glands Following Management of Ectopic Cushing's Syndrome by Steroidogenesis Inhibitors.

    Science.gov (United States)

    Huang, Yu-Ting; Aziz, Shaikh Irfan; Ravi Kumar, Aravind S

    2014-09-01

    In the era of emerging functional imaging techniques, an understanding of the effects of hormonal therapies on the scintigraphic appearance of endocrine organs is desirable to minimize the erroneous scan interpretation. The mechanisms by which changes in the scintigraphic appearance of endocrine organs occur however sometimes remain ambiguous. This case demonstrates the gallium-68 (Ga-68) DOTA-TATE positron emission tomography/computed tomography (CT) appearance of adrenal glands following management with steroidogenesis inhibitors. The potential mechanisms underlying this change are discussed. A 17-year-old boy with adrenocorticotropic hormone (ACTH) dependent Cushing's syndrome secondary to ectopic ACTH secretion underwent pre- and post-metyrapone and dexamethasone treatment Ga-68 DOTA-TATE scans 4 months apart. Pretreatment, both adrenals demonstrated normal symmetrical prominent Ga-68 DOTA-TATE uptake and normal CT appearance. The posttherapy scan revealed marked symmetrical suppression of Ga-68 DOTA-TATE uptake, but with bilateral adrenal hypertrophy on CT.

  12. Crystal structure of the Bloom's syndrome helicase indicates a role for the HRDC domain in conformational changes

    DEFF Research Database (Denmark)

    Newman, Joseph A; Savitsky, Pavel; Allerston, Charles K;

    2015-01-01

    Bloom's syndrome helicase (BLM) is a member of the RecQ family of DNA helicases, which play key roles in the maintenance of genome integrity in all organism groups. We describe crystal structures of the BLM helicase domain in complex with DNA and with an antibody fragment, as well as SAXS...... core domains. Comparison with other crystal structures of RecQ helicases permits the definition of structural transitions underlying ATP-driven helicase action, and the identification of a nucleotide-regulated tunnel that may play a role in interactions with complex DNA substrates....

  13. Minimal change disease caused by mercury-containing cosmetics: 7 cases report%七例化妆品汞中毒相关肾小球微小病变病报告

    Institute of Scientific and Technical Information of China (English)

    王艳艳; 谌贻璞; 王国勤; 董鸿瑞; 孙丽君; 王长江; 唐艳玲; 程虹

    2013-01-01

    目的 探讨化妆品汞中毒相关微小病变病(MCD)的临床病理表现及治疗方法.方法 回顾性分析首都医科大学附属北京安贞医院肾内科确诊的7例化妆品汞中毒相关MCD患者的临床及病理资料.结果 7例患者均为女性,平均年龄37.2岁,均曾间断使用美白祛斑化妆品.7例患者尿汞均明显升高,为正常值的2.2~ 59.1倍.其中2例患者化妆品的汞检测,汞浓度为国家规定化妆品汞限量的1000多倍.7例患者均表现为肾病综合征,肾组织病理检查符合MCD诊断.经停用含汞化妆品,并予糖皮质激素及驱汞治疗,7例患者肾病综合征均在应用激素后1~2个月完全缓解.结论 对非少年儿童及老年的MCD女性患者,要注意询问美白祛斑化妆品的应用史,检测尿和血汞浓度,以早期发现化妆品汞中毒相关MCD.%Objective To enhance the understanding of mercury poisoning related minimal change disease (MP-MCD).Methods A retrospective analysis about the clinical,laboratory and pathological manifestations,diagnosis,treatment and outcome of 7 patients with MP-MCD caused by skin-lightening cosmetics were conducted.Results All of the 7 patients were female with mean age 37.2 years.One patient had intermittently used skin-lightening cosmetics for 15 years and the other 6 patients continuously used the cosmetics for 2 ~ 6 months.Urine mercury levels of the 7 patients were significantly increased,reaching to 2.2 ~ 59.1 times of the normal value.The mercury content of 2 cosmetics used by patients was 1200 to 1560 times the mercury limit in cosmetics set by the government in 1987.Seven patients all presented as nephrotic syndrome,and all had MCD on renal biopsy.After the diagnosis of MP-MCD was confirmed,all the patients stopped using the mercurycontain cosmetics and accepted corticosteroid and chelation therapy.The nephrotic syndrome underwent complete remission after 1 ~ 2 months of therapy.Urine mercury levels in the 5 patients

  14. Impairments in motor coordination without major changes in cerebellar plasticity in the Tc1 mouse model of Down syndrome.

    Science.gov (United States)

    Galante, Micaela; Jani, Harsha; Vanes, Lesley; Daniel, Hervé; Fisher, Elizabeth M C; Tybulewicz, Victor L J; Bliss, Timothy V P; Morice, Elise

    2009-04-15

    Down syndrome (DS) is a genetic disorder arising from the presence of a third copy of human chromosome 21 (Hsa21). Recently, O'Doherty et al. [An aneuploid mouse strain carrying human chromosome 21 with Down syndrome phenotypes. Science 309 (2005) 2033-2037] generated a trans-species aneuploid mouse line (Tc1) that carries an almost complete Hsa21. The Tc1 mouse is the most complete animal model for DS currently available. Tc1 mice show many features that relate to human DS, including alterations in memory, synaptic plasticity, cerebellar neuronal number, heart development and mandible size. Because motor deficits are one of the most frequently occurring features of DS, we have undertaken a detailed analysis of motor behaviour in cerebellum-dependent learning tasks that require high motor coordination and balance. In addition, basic electrophysiological properties of cerebellar circuitry and synaptic plasticity have been investigated. Our results reveal that, compared with controls, Tc1 mice exhibit a higher spontaneous locomotor activity, a reduced ability to habituate to their environments, a different gait and major deficits on several measures of motor coordination and balance in the rota rod and static rod tests. Moreover, cerebellar long-term depression is essentially normal in Tc1 mice, with only a slight difference in time course. Our observations provide further evidence that support the validity of the Tc1 mouse as a model for DS, which will help us to provide insights into the causal factors responsible for motor deficits observed in persons with DS.

  15. Refeeding syndrome.

    Science.gov (United States)

    Fuentebella, Judy; Kerner, John A

    2009-10-01

    Refeeding syndrome (RFS) is the result of aggressive enteral or parenteral feeding in a malnourished patient, with hypophosphatemia being the hallmark of this phenomenon. Other metabolic abnormalities, such as hypokalemia and hypomagnesemia, may also occur, along with sodium and fluid retention. The metabolic changes that occur in RFS can be severe enough to cause cardiorespiratory failure and death. This article reviews the pathophysiology, the clinical manifestations, and the management of RFS. The key to prevention is identifying patients at risk and being aware of the potential complications involved in rapidly reintroducing feeds to a malnourished patient.

  16. Dynamic changes of pulmonary lesions on CT in patients with severe acute respiratory syndrome treated by non-invasive positive pressure ventilation

    International Nuclear Information System (INIS)

    Objective: To study the dynamic changes of pulmonary lesions of severe acute respiratory syndrome (SARS) treated by non-invasive positive pressure ventilation (NIPPV) on CT. Methods: Serial chest CT images in 18 patients with SARS were retrospectively analyzed before and after NIPPV (4-12 days), and a long-term follow-up (3-36 months) examination by CT was carried out. And 27 SARS cases without NIPPV were enrolled as the control group. Results: (1) There were dynamic changes in 15 cases of 18 cases: partial multilobar consolidations of bilateral lungs changed into ground-glass opacities in 7 of 18 cases, a circular pulmonary emphysema around bronchial vascular strains of the lower lobes was seen in 5 of 18, subpleural pulmonary emphysema was seen in 8 of 18, pulmonary barotraumas were showed in 2 of 18. Residual of subpleural curvilinear shadow related to NIPPV was found in 2 eases on the follow-up scan. (2) Above changes on CT were not found in 27 cases without NIPPV. Conclusion: There were changes of pulmonary lesions of SARS on CT related to NIPPV treatment, It is beneficial to assess the CT images in the NIPPV treatment of the related pneumonia in SARS. (authors)

  17. Multidimensional ultrasound imaging of the wrist: Changes of shape and displacement of the median nerve and tendons in carpal tunnel syndrome.

    Science.gov (United States)

    Filius, Anika; Scheltens, Marjan; Bosch, Hans G; van Doorn, Pieter A; Stam, Henk J; Hovius, Steven E R; Amadio, Peter C; Selles, Ruud W

    2015-09-01

    Dynamics of structures within the carpal tunnel may alter in carpal tunnel syndrome (CTS) due to fibrotic changes and increased carpal tunnel pressure. Ultrasound can visualize these potential changes, making ultrasound potentially an accurate diagnostic tool. To study this, we imaged the carpal tunnel of 113 patients and 42 controls. CTS severity was classified according to validated clinical and nerve conduction study (NCS) classifications. Transversal and longitudinal displacement and shape (changes) were calculated for the median nerve, tendons and surrounding tissue. To predict diagnostic value binary logistic regression modeling was applied. Reduced longitudinal nerve displacement (p≤ 0.019), increased nerve cross-sectional area (p≤ 0.006) and perimeter (p≤ 0.007), and a trend of relatively changed tendon displacements were seen in patients. Changes were more convincing when CTS was classified as more severe. Binary logistic modeling to diagnose CTS using ultrasound showed a sensitivity of 70-71% and specificity of 80-84%. In conclusion, CTS patients have altered dynamics of structures within the carpal tunnel.

  18. Targeted exome sequencing integrated with clinicopathological information reveals novel and rare mutations in atypical, suspected and unknown cases of Alport syndrome or proteinuria.

    Directory of Open Access Journals (Sweden)

    Rajshekhar Chatterjee

    Full Text Available We applied customized targeted next-generation exome sequencing (NGS to determine if mutations in genes associated with renal malformations, Alport syndrome (AS or nephrotic syndrome are a potential cause of renal abnormalities in patients with equivocal or atypical presentation. We first sequenced 4,041 exons representing 292 kidney disease genes in a Caucasian woman with a history of congenital vesicoureteral reflux (VUR, recurrent urinary tract infections and hydronephrosis who presented with nephrotic range proteinuria at the age of 45. Her biopsy was remarkable for focal segmental glomerulosclerosis (FSGS, a potential complication of longstanding VUR. She had no family history of renal disease. Her proteinuria improved initially, however, several years later she presented with worsening proteinuria and microhematuria. NGS analysis revealed two deleterious COL4A3 mutations, one novel and the other previously reported in AS, and a novel deleterious SALL2 mutation, a gene linked to renal malformations. Pedigree analysis confirmed that COL4A3 mutations were nonallelic and compound heterozygous. The genomic results in conjunction with subsequent abnormal electron microscopy, Collagen IV minor chain immunohistochemistry and progressive sensorineural hearing loss confirmed AS. We then modified our NGS approach to enable more efficient discovery of variants associated with AS or a subset of FSGS by multiplexing targeted exome sequencing of 19 genes associated with AS or FSGS in 14 patients. Using this approach, we found novel or known COL4A3 or COL4A5 mutations in a subset of patients with clinically diagnosed or suspected AS, APOL1 variants associated with FSGS in African Americans and novel mutations in genes associated with nephrotic syndrome. These studies demonstrate the successful application of targeted capture-based exome sequencing to simultaneously evaluate genetic variations in many genes in patients with complex renal phenotypes and

  19. Electroacupuncture at Tianshu(ST 25)for diarrhea-predominant irritable bowel syndrome using positron emission tomography Changes in visceral sensation center

    Institute of Scientific and Technical Information of China (English)

    Huirong Liu; Enhua Zhou; Huangan Wu; Li Qi; Xiaolong Wang; Yihui Guan; Chuantao Zuo; Linying Tan; Lingsong Yuan; Xiaopeng Ma; Xiaomei Wang

    2010-01-01

    Previous studies have demonstrated that electroacupuncture therapy is effective in the treatment of irritable bowel syndrome.However,the precise mechanism of this therapy is unknown.The present study served to investigate the effects of electroacupuncture therapy on treatment of patients with diarrhea-predominant irritable bowel syndrome(IBS).We compared brain activation maps based on the changes of cerebral glucose metabolism obtained by 18-fluorodeoxyglucose positron emission tomography scanning under three conditions: resting,rectal balloon distension and rectal balloon distension plus electroacupuncture.Under the resting condition,compared with healthy controls,IBS patients displayed an increasing regional cerebral metabolic rate of glucose over a wide range:bilateral superior temporal gyrus,right middle occipital gyrus,superior frontal gyrus and bilateral middle frontal gyrus.However,there was no significant activity in the visceral pain center.Compared with the resting condition,under the rectal balloon distension condition,patients with IBS had a greater regional cerebral metabolic rate of glucose in the prefrontal cortex,left anterior cingulate cortex,postcentral gyrus,precentral gyrus and temporal gyrus.Under the rectal balloon distension plus electroacupuncture condition,stimulation by electroacupuncture at Tianshu(ST 25)manifested a decreased regional cerebral metabolic rate of glucose in the left cingulate gyrus,right insula,right caudate nucleus,fusiform gyrus and hippocampal gyrus.Electroacupuncture therapy relieved abdominal pain,distension or discomfort by decreasing glucose metabolism in the brain.

  20. Changes in left ventricular systolic function in patients with chronic heart failure with preserved ejection fraction and cardiorenal anemic syndrome

    Directory of Open Access Journals (Sweden)

    Vasylenko V.A.

    2015-09-01

    Full Text Available The feature of chronic heart failure (CHF in elderly people is increasing incidence of heart failure with preserved left ventricular ejection fraction (LVEF which is associated with age. Such patients account for almost half of the total number of patients with heart failure. Cardiorenal syndrome (CRS is associated with an increased risk of mortality in patients with CHF. The impact of CRS on the structural and functional condition of the heart in these patients is studied insufficiently. The study involved 103 patients with CHF II-IV NYHA with preserved LVEF (>45% and CRS (hemoglobin <120 g/l and