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Sample records for childhood nephrotic syndrome

  1. Long-term Outcomes of Childhood Onset Nephrotic Syndrome

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    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, ...

  2. Long-term Outcomes of Childhood Onset Nephrotic Syndrome.

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    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.

  3. Management of idiopathic nephrotic syndrome in childhood

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    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

  4. Levamisole for steroid-dependent nephrotic syndrome of childhood

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    Hadadi M

    2008-06-01

    Full Text Available Background: Childhood nephrotic syndrome is frequently characterized by a relapsing course. Due to their adverse effects, the use of corticosteroids for the management of frequently relapsing nephrotic syndrome is limited. Levamisole, a steroid sparing agent, has been found to have low toxicity. This study was conducted to evaluate the efficacy of levamisole in steroid-sensitive nephrotic syndrome (SDNS.  Methods: In this retrospective study from January 1988 to September 2006, we included data from 305 pediatric SDNS patients at the Children's Medical Center clinics in Tehran, Iran. Nephrotic syndrome was diagnosed using classic criteria. None of the patients had any signs or symptoms of secondary causes of nephrotic syndrome. All had received prednisolone 60 mg/m2/day. After remission, prednisolone administration was reduced to every other day and the steroid was tapered over the next three months. With every recurrence, prednisolone was prescribed with the same dosage, but after remission it was continued at a lower dosage for another six months or longer if there was risk of recurrence. Levamisole was administered to all patients at a dose of 2 mg/kg every other day.          Results: Patients ranged in age from 1 to 20 years (mean±SD: 4.84 ±3.1 and 70.8% were male. At the last follow up, 84 (27.5% were in remission, while 220 (72.1% patients had relapsed or needed a low dose of steroid. Levamisole was effective in reducing the prednisolone dosage and long-term remission in 68 (22.3% and 90 (29.5% cases, respectively. A comparison of before vs. after levamisole treatment revealed a had significant decrease in the number of relapses (2.05±0.88 vs. 1.1±1.23; P<0.0001 and the prednisolone dosage (0.74±0.39 vs. 0.32±0.38 mg/kg/day; P<0.0001. Only one patient developed levamisole-induced neutropenia. Conclusions: In childhood steroid-dependent nephrotic syndrome, levamisole is an efficacious, safe initial therapy in

  5. Genetics of childhood steroid-sensitive nephrotic syndrome.

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    Karp, Alana M; Gbadegesin, Rasheed A

    2016-07-29

    The pathogenesis of childhood-onset nephrotic syndrome (NS), disparity in incidence of NS among races, and variable responses to therapies in children with NS have defied explanation to date. In the last 20 years over 50 genetic causes of steroid-resistant nephrotic syndrome (SRNS) have been identified, and at least two disease loci for two pathologic variants of SRNS (focal segmental glomerulosclerosis and membranous nephropathy) have been defined. However, the genetic causes and risk loci for steroid-sensitive nephrotic syndrome (SSNS) remain elusive, partly because SSNS is relatively rare and also because cases of SSNS vary widely in phenotypic expression over time. A recent study of a well-defined modest cohort of children with SSNS identified variants in HLA-DQA1 as a risk factor for SSNS. Here we review what is currently known about the genetics of SSNS and also discuss how recent careful phenotypic and genomic studies reinforce the role of adaptive immunity in the molecular mechanisms of SSNS.

  6. Reciprocal Regulation of 11β-HSDs May Predict Steroid Sensitivity in Childhood Nephrotic Syndrome.

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    Sai, Shuji; Yamamoto, Masaki; Yamaguchi, Rie; Chapman, Karen E; Hongo, Teruaki

    2016-09-01

    Childhood nephrotic syndrome, in which steroid-dependence occurs concurrently with steroid-resistance, requires aggressive therapy to prevent relapse. Predictive biomarkers that can be used to stratify treatment are urgently needed. Here we report that reciprocal regulation of the glucocorticoid metabolizing enzymes, 11β-hydroxysteroid dehydrogenase types 1 and 2, is associated with steroid-responsiveness and disease remission in childhood nephrotic syndrome, potentially providing a marker to identify patients in which aggressive therapy is required.

  7. 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.

  8. Nephrotic Syndrome in Adults

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    ... Disease Chronic Kidney Disease (CKD) What Is Chronic Kidney Disease? Causes of CKD Tests & Diagnosis Managing CKD Eating Right Preventing CKD What If My Kidneys Fail? Clinical Trials Anemia High Blood Pressure Heart ... Nephropathy Kidney Disease in Children Childhood Nephrotic Syndrome Hemolytic ...

  9. Disruption of PTPRO Causes Childhood-Onset Nephrotic Syndrome

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    Ozaltin, Fatih; Ibsirlioglu, Tulin; Taskiran, Ekim Z.; Baydar, Dilek Ertoy; Kaymaz, Figen; Buyukcelik, Mithat; Kilic, Beltinge Demircioglu; Balat, Ayse; Iatropoulos, Paraskevas; Asan, Esin; Akarsu, Nurten A.; Schaefer, Franz; Yilmaz, Engin; Bakkaloglu, Ayşin

    2011-01-01

    Idiopathic nephrotic syndrome (INS) is a genetically heterogeneous group of disorders characterized by proteinuria, hypoalbuminemia, and edema. Because it typically results in end-stage kidney disease, the steroid-resistant subtype (SRNS) of INS is especially important when it occurs in children. The present study included 29 affected and 22 normal individuals from 17 SRNS families; genome-wide analysis was performed with Affymetrix 250K SNP arrays followed by homozygosity mapping. A large homozygous stretch on chromosomal region 12p12 was identified in one consanguineous family with two affected siblings. Direct sequencing of protein tyrosine phosphatase receptor type O (PTPRO; also known as glomerular epithelial protein-1 [GLEPP1]) showed homozygous c.2627+1G>T donor splice-site mutation. This mutation causes skipping of the evolutionarily conserved exon 16 (p.Glu854_Trp876del) at the RNA level. Immunohistochemistry with GLEPP1 antibody showed a similar staining pattern in the podocytes of the diseased and control kidney tissues. We used a highly polymorphic intragenic DNA marker—D12S1303—to search for homozygosity in 120 Turkish and 13 non-Turkish individuals in the PodoNet registry. This analysis yielded 17 candidate families, and a distinct homozygous c.2745+1G>A donor splice-site mutation in PTPRO was further identified via DNA sequencing in a second Turkish family. This mutation causes skipping of exon 19, and this introduces a premature stop codon at the very beginning of exon 20 (p.Asn888Lysfs∗3) and causes degradation of mRNA via nonsense-mediated decay. Immunohistochemical analysis showed complete absence of immunoreactive PTPRO. Ultrastructural alterations, such as diffuse foot process fusion and extensive microvillus transformation of podocytes, were observed via electron microscopy in both families. The present study introduces mutations in PTPRO as another cause of autosomal-recessive nephrotic syndrome. PMID:21722858

  10. Second-Line Immunosuppressive Treatment of Childhood Nephrotic Syndrome: A Single-Center Experience

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    J. Kim

    2014-01-01

    Full Text Available Objective: Most cases of idiopathic nephrotic syndrome in childhood are responsive to corticosteroids. However, there is a small group of children that demonstrate steroid resistance (steroid-resistant nephrotic syndrome; SRNS, steroid dependence, or that frequently relapse (frequent-relapse steroid-sensitive nephrotic syndrome; FR-SSNS which are more clinically difficult to treat. Therefore, second-line immunosuppressants, such as alkylating agents, calcineurin inhibitors, antimetabolites and, more recently, rituximab, have been used with varying success. The objective was to evaluate the response rates of various second-line therapies in the treatment of childhood nephrotic syndrome. Study Design: A retrospective chart review of pediatric subjects with idiopathic nephrotic syndrome was conducted at a single tertiary care center (2007-2012. Drug responses were classified as complete response, partial response, and no response. Results: Of the 188 charts reviewed, 121 children were classified as SSNS and 67 children as SRNS; 58% were classified as FR-SSNS. Sixty-five subjects were diagnosed with focal segmental glomerulosclerosis via biopsy. Follow-up ranged from 6 months to 21 years. The combined rate of complete and partial response for mycophenolate mofetil (MMF was 65% (33/51 in SSNS and 67% (6/9 in SRNS. For tacrolimus, the response rate was 96% (22/23 for SSNS and 77% (17/22 for SRNS. Eighty-three percent (5/6 of SSNS subjects treated with rituximab went into complete remission; 60% relapsed after B-cell repletion. Eight refractory subjects were treated with combined MMF/tacrolimus/corticosteroid therapy with a 75% response rate. Conclusion: Our experience demonstrates that older medications can be replaced with newer ones such as MMF, tacrolimus, and rituximab with good outcomes and better side effect profiles. The treatment of refractory cases with combination therapy is promising.

  11. 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.

  12. Elevation of plasma-soluble HLA-G in childhood nephrotic syndrome is associated with IgE.

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    Liu, Yanqing; Lai, Meimei; Lou, Yunyan; Han, Qiuyue; Yang, Qing; Chen, Minguang; Li, Jingbo; Wang, Huiyan; Yan, Weihua; Zheng, Xiaoqun

    2017-01-01

    Background Nephrotic syndrome is related to immune system dysfunction. Soluble human leukocyte antigen-G has been suggested to have an immunomodulatory role. Additionally, human leukocyte antigen-G expression may be influenced by the 14-base pair insertion/deletion polymorphism. However, this molecule has not been investigated in nephrotic syndrome. Methods Fifty-five children with nephrotic syndrome were enrolled: 24 primary nephrotic syndrome patients and 31 recurrent nephrotic syndrome patients. A group of 120 healthy subjects were included as reference controls. Additionally, 22 patients in nephrotic syndrome remission after treatments were also included. Both nephrotic syndrome patients and healthy subjects were genotyped for the 14-base pair insertion/deletion polymorphism. Plasma soluble human leukocyte antigen-G concentrations and serum immunoglobulin concentrations were determined. Results Nephrotic syndrome patients showed significantly higher levels of both soluble human leukocyte antigen-G and immunoglobulin E compared to normal controls. Nephrotic syndrome patients presented a higher frequency of the -14-base pair allele than did normal controls. Soluble human leukocyte antigen-G concentrations in remission patients were dramatically lower compared to in nephrotic syndrome patients. Moreover, soluble human leukocyte antigen-G and immunoglobulin E were moderately correlated in nephrotic syndrome patients. Conclusions The present study demonstrated that plasma soluble human leukocyte antigen-G concentrations were significantly elevated and that a relationship between serum total immunoglobulin E in nephrotic syndrome patients and the human leukocyte antigen-G -14-base pair allele may be a risk factor for nephrotic syndrome. These findings suggest that soluble human leukocyte antigen-G may be used as a monitoring marker for nephrotic syndrome patients' condition.

  13. 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.

  14. Congenital nephrotic syndrome

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    Claudia Fanni

    2014-06-01

    Full Text Available CNS (Congenital nephrotic syndrome is a disorder characterized by the presence of a nephrotic syndrome in the first three months of life. Different pathologies can cause this syndrome. In general, we can distinguish primary forms (sporadic and hereditary and secondary forms (acquired and associated with other syndromes. The most common form is the Finnish CNS (CNF, congenital nephrotic syndrome of the Finnish type, a hereditary form whose name derives from the fact that the highest incidence is described in that country (1.2:10,000. The pathogenesis, the clinical picture, the diagnostic criteria, the therapy and the outcome are described in details.  Proceedings of the International Course on Perinatal Pathology (part of the 10th International Workshop on Neonatology · October 22nd-25th, 2014 · Cagliari (Italy · October 25th, 2014 · The role of the clinical pathological dialogue in problem solving Guest Editors: Gavino Faa, Vassilios Fanos, Peter Van Eyken

  15. Effects of gluten-free, dairy-free diet on childhood nephrotic syndrome and gut microbiota.

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    Uy, Natalie; Graf, Lauren; Lemley, Kevin V; Kaskel, Frederick

    2015-01-01

    Emerging evidence suggests an association between food sensitivity and gut microbiota in children with nephrotic syndrome. Diminished proteinuria resulted from eliminating cow's milk and the use of an oligoantigenic diet which excluded gluten, especially in patients with immune-related conditions, i.e., celiac disease and nephrotic syndrome. The mechanisms underlying the association of diet, gut microbiota, and dysregulation of the immune system are unknown. Gut microbiota is influenced by a number of factors including diet composition and other environmental epigenetic exposures. The imbalance in gut microbiota may be ameliorated by gluten-free and dairy-free diets. Gluten-free diet increased the number of unhealthy bacteria while reducing bacterial-induced cytokine production of IL-10. Thus, gluten-free diet may influence the composition and immune function of gut microbiota and should be considered a possible environmental factor associated with immune-related disease, including nephrotic syndrome. Furthermore, the imbalance of gut microbiota may be related to the development of cow's milk protein allergy. Investigations are needed to fill the gaps in our knowledge concerning the associations between the gut microbiome, environmental exposures, epigenetics, racial influences, and the propensity for immune dysregulation with its inherent risk to the developing individual.

  16. Complications of nephrotic syndrome

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    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.

  17. 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.

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

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    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

  19. Glomerular Glucocorticoid Receptors Expression and Clinicopathological Types of Childhood Nephrotic Syndrome.

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    Gamal, Yasser; Badawy, Ahlam; Swelam, Salwa; Tawfeek, Mostafa S K; Gad, Eman Fathalla

    2017-02-01

    Glucocorticoids are primary therapy of idiopathic nephrotic syndrome (INS). However, not all children respond to steroid therapy. We assessed glomerular glucocorticoid receptor expression in fifty-one children with INS and its relation to response to steroid therapy and to histopathological type. Clinical, laboratory and glomerular expression of glucocorticoid receptors were compared between groups with different steroid response. Glomerular glucocorticoid expression was slightly higher in controls than in minimal change early responders, which in turn was significantly higher than in minimal change late responders. There was significantly lower glomerular glucocorticoid receptor expression in steroid-resistance compared to early responders, late responders and controls. Glomerular glucocorticoid expression was significantly higher in all minimal change disease (MCD) compared to focal segmental glomerulosclerosis. In INS, response to glucocorticoid is dependent on glomerular expression of receptors and peripheral expression. Evaluation of glomerular glucocorticoid receptor expression at time of diagnosis of NS can predict response to steroid therapy.

  20. Nephrotic Syndrome Associated with Thymoma

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    Sheng-Yen Hsiao

    2014-12-01

    Full Text Available Thymoma is associated with a wide variety of paraneoplastic syndromes such as myasthenia gravis, pure red cell aplasia, and hypogammaglobulinemia. Paraneoplastic glomerulonephritis is a rare clinical presentation of malignancy. This condition often goes undetected as it has no specific clinical symptoms and signs. Approximately 2% of thymoma patients have been reported to have paraneoplastic glomerulonephritis, and the nephrotic syndrome has been shown to be a clinical manifestation of the disorder. We report two cases diagnosed to have thymoma and nephrotic syndrome. Renal biopsy showed that one case had focal segmental glomerulosclerosis, whereas the other had minimal change disease. In case 1, the nephrotic syndrome was diagnosed before thymoma was detected, while in case 2, the symptomatic nephrotic syndrome occurred after thymoma treatment. Because parathymic nephropathy often remains undiagnosed and interferes with treatment, the possibility of the nephrotic syndrome should always be considered throughout the course of thymoma management, particularly in patients who also present with anasarca or hypoalbuminemia. A multidisciplinary approach is needed. Besides, it is to be noted that the nephrotic syndrome may be the initial presentation of thymoma.

  1. Pulmonary carcinoid tumor associated with nephrotic syndrome.

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    DePace, N L; Elquezabal, A; Hardenburg, H C

    1980-04-01

    A patient with carcinoid tumor of the lung associated with nephrotic syndrome was treated. Excision of the tumor resulted in remission of marked proteinuria, hypoalbuminemia, and edema. A review of the literature disclosed many neoplasms associated with the nephrotic syndrome; however, no association of the nephrotic syndrome and a carcinoid tumor of the lung has previously been reported, to our knowledge.

  2. 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.

  3. 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.

  4. Childhood Nephrotic Syndrome

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    ... Ira Davis, M.D.; Ann Guillott, M.D.; Steve Alexander, M.D.; Deborah Kees-Folts, M.D.; Alicia Neu, M.D.; Steve Wassner, M.D.; John Brandt, M.D.; and ... Advisory & Coordinating Committees Strategic Plans & Reports Research Areas Jobs at NIDDK FAQs Visit Us News NIDDK News ...

  5. METHYLPREDNISOLONE PULSE THERAPY IN MANAGEMENT OF NON RESPONDER NEPHROTIC SYNDROME

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    A. Madani

    1999-08-01

    Full Text Available Some patients with the diagnosis of childhood nephrotic syndrome are unresponsive to conventional treatment regimens. Recent studies of more aggressive therapies have provided strong evidence of the benefit of high dose methylprednisolonc (MP protocol with alternate - day prednisone alone or with alternate - day prednisone plus an alkylating agent (I in these patients."nFrom May 1996 to May 1997 we have treated 14 patients with non-responder nephrotic syndrome with mcthyprcdnisolone protocol. Eight patients had histologic diagnosis of focal segmental glomerulosclerosis, 3 diffuse mesangial proliferation and 3 has minimal change disease. C'ylosporin was added in two patients to methylprcdnisotonc at the beginning of the second course of therapy. Tfie patients were observed for an average of 8 months (range 4-12 months. In the last follow up there were no patients in remission and all remained nephrotic. Seven patients had persistent massive proteinuria with normal creatinine clearance (CrCI. Two had decreased CrCl. Five progressed to end-stage renal disease. Tlicsc observations suggest that "Puke" methy{prednisolone is not effective in patients with non respondcr nephrotic syndrome.

  6. 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.

  7. 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.

  8. Glucocorticoid pharmacogenetics in pediatric idiopathic nephrotic syndrome.

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    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.

  9. 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.

  10. Genetic testing in nephrotic syndrome--challenges and opportunities.

    Science.gov (United States)

    Gbadegesin, Rasheed A; Winn, Michelle P; Smoyer, William E

    2013-03-01

    Monogenic nephrotic syndrome (nephrotic syndrome caused by a single gene defect) is responsible for only a small percentage of cases of nephrotic syndrome, but information from studies of the unique cohort of patients with this form of the disease has dramatically improved our understanding of the disease pathogenesis. The use of genetic testing in the management of children and adults with nephrotic syndrome poses unique challenges for clinicians in terms of who to test and how to use the information obtained from testing in the clinical setting. In our view, not enough data exist at present to justify the routine genetic testing of all patients with nephrotic syndrome. Testing is warranted, however, in patients with congenital nephrotic syndrome (onset at 0-3 months), infantile nephrotic syndrome (onset at 3-12 months), a family history of nephrotic syndrome, and those in whom nephrotic syndrome is associated with other congenital malformations. The family and/or the patient should be given complete and unbiased information on the potential benefits and risks associated with therapy, including the reported outcomes of treatment in patients with similar mutations. Based on the data available in the literature so far, intensive immunosuppressive treatment is probably not indicated in monogenic nephrotic syndrome if complete or partial remission has not been achieved within 6 weeks of starting treatment. We advocate that family members of individuals with genetic forms of nephrotic syndrome undergo routine genetic testing prior to living-related kidney transplantation. Prospective, multicentre studies are needed to more completely determine the burden of disease caused by monogenic nephrotic syndrome, and randomized controlled trials are needed to clarify the presence or absence of clinical responses of monogenic nephrotic syndrome to available therapies.

  11. The nephrotic syndrome and its complications.

    Science.gov (United States)

    Cameron, J S

    1987-09-01

    Modern views of the pathogenesis and natural history of nephrotic syndrome have changed substantially since the early studies by Cotugno and Bright. Contrary to beliefs held 20 years ago, we do not possess a unique satisfying explanation for the induction, maintenance, and resolution of nephrotic edema, and many concepts firmly established as "classic" are now being revised or reconsidered. These include the relationship between urinary protein losses and hypoalbuminemia, which is complicated by several factors such as daily protein intake, albumin catabolism, and the possible role of albumin loss at extrarenal sites. The influence of lowered plasma albumin on the decrease in plasma volume is also quite complex, due to technical difficulties in measuring plasma volume and turnover of radio-labeled albumin. The most contentious areas are how sodium and water retention are initiated and maintained and the relationship between hypoalbuminemia, plasma oncotic pressure, and edema. While aldosterone excretion and plasma concentrations are elevated in nephrotic patients, data on the renin-angiotensin system are controversial and the renal handling of sodium is related to a host of factors including glomerular filtration rate, altered proximal tubular reabsorption, and the role of vasodilators or vasoconstrictors. The complications of nephrotic syndrome are protean and relatively common. Among those are acute renal failure, thrombosis, infections, and hyperlipidemia. Since the introduction of percutaneous biopsy, the spectrum of lesions underlying nephrotic syndrome has widened considerably, the most common being minimal change, especially in children. There are very few prognostic indicators by which response to treatment may be predicted and these include persistent microscopic hematuria.

  12. Two cases of nephrotic syndrome with different etiologies

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    Sanjay K Mandal

    2013-01-01

    Full Text Available There are various causes of secondary nephrotic syndrome. Finding an underlying etiology in a case of nephrotic syndrome or subnephrotic range proteinuria can markedly alter the therapeutic options and disease course. We describe two cases of secondary nephrotic syndrome. The first case was a 22-year-old male with pulmonary tuberculosis with nephrotic syndrome secondary to renal amyloidosis, whereas the second case was a 17-year-old male with chronic hepatitis B-associated nephrotic syndrome. It is important, especially in developing countries, to be aware that tuberculosis and infections like hepatitis B, C, etc. continue to be part of the differential diagnosis of secondary nephrotic syndrome in adolescents and young adults.

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

    Science.gov (United States)

    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.

  14. 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).

  15. Diuretics and its use in nephrotic syndrome

    OpenAIRE

    2012-01-01

    Nephrotic syndrome is a clinical condition characterized by an urinary massive lossof proteins, specially albumin, which leads to the development of hypoalbuminemiaand secondary edema. Other alterations, like dyslipidemia, accompany to thesemanifestations in a compensatory way to the loss of oncotic pressure, but thesemanifestations aren´t part of the definition. The treatment is based on the use ofcorticoids and cytotoxic drugs, however other measures for the control of edema, as theuse of d...

  16. 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

  17. Cerebral Sinovenous Thrombosis in a Child with Idiopathic Nephrotic Syndrome

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    L. Ghedira Besbes

    2011-01-01

    Full Text Available Nephrotic syndrome (NS is a renal disorder characterized by heavy proteinuria, hypoalbuninemia, edema and hypercholesterolemia. Nephrotic syndrome in children is known to be associated with an hypercoagulable state and thromboembolic complications. However cerebral sinovenous thrombosis (CSVT is very rare. Here we report a seven-year-old child with steroid-dependent idopathic nephrotic syndrome resulting from a minimal change disease, developed multiple cerebral sinovenous thrombosis, presenting with headache, left sixth nerve palsy, and papilledema. The diagnosis of CSVT was established by cranial computed tomography, magnetic resonance imaging, and magnetic resonance angiography. He gradually recovered after anticoagulant therapy. CSVT is very rare in nephrotic children. The diagnosis of CSVT should be considered in any patient with nephrotic syndrome who develops neurologic symptoms. This report highlights the importance of suspecting and recognizing this potentially life threatening complication and initiating early treatment.

  18. 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.

  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. Sagittal sinus thrombosis in adult minimal change nephrotic syndrome.

    Science.gov (United States)

    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.

  1. 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.

  2. 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

  3. Cerebral infarction in patient with minimal change nephrotic syndrome.

    Science.gov (United States)

    Babu, A; Boddana, P; Robson, S; Ludeman, L

    2013-01-01

    We report a case of 68-year-old Caucasian man who presented with cerebral infarcts secondary to arterial thrombosis associated with nephrotic syndrome. His initial presentation included edema of legs, left hemiparesis, and right-sided cerebellar signs. Investigations with computed tomography and magnetic resonance imaging of brain showed multiple cerebral infarcts in middle cerebral and posterior cerebral artery territory. Blood and urine investigations also showed impaired renal function, hypercholesterolemia, hypoalbuminaemia, and nephrotic range proteinuria. Renal biopsy showed minimal change disease. Cerebral infarcts were treated with antiplatelet agents and nephrotic syndrome was treated with high dose steroids. Patient responded well to the treatment and is all well till date.

  4. 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.

  5. Mesothelioma of the testis and nephrotic syndrome: a case report

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    Bacchetta Justine

    2009-06-01

    Full Text Available Abstract Introduction Paraneoplastic glomerulopathies are rare manifestations of neoplastic disease to be distinguished from iatrogenic renal damage. Solid tumors are preferentially associated with membranous nephropathy, whereas Hodgkin's lymphomas are associated with minimal change disease. Case presentation We report a 63-year-old Caucasian male diagnosed with a mesothelioma of the tunica vaginalis testis who, secondary to this, also presented with a nephrotic syndrome due to minimal change disease. In the present case, the paraneoplastic etiology of the nephrotic syndrome can be discussed on four unusual elements: minimal change lesions were found; the glomerulopathy was very sensitive to corticosteroids; the nephrotic syndrome occurred 11 months after the diagnosis of the primary malignancy, but concomitantly with the recurrence; and the nephrotic syndrome did not decrease with tumor control and did not recur when the mesothelioma escaped treatment. No other etiologies could nevertheless explain this phenomenon. Conclusion Paraneoplastic nephrotic syndrome is often associated with membranous nephropathy in patients with solid tumors, especially in patients with lung and gastrointestinal tract neoplasia. The management of these patients is associated with a symptomatic treatment such as sodium and water restriction, diuretics and ACE inhibitors and a prophylaxis of specific complications of nephrotic syndrome including thromboembolism, infections and lipid abnormalities. Treatment of neoplasia must be undertaken rapidly, treatments must be regularly analyzed and drugs binding to albumin may be used with precaution.

  6. Calcium and Vitamin D Metabolism in Pediatric Nephrotic Syndrome; An Update on the Existing Literature

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    Mohammad Esmaeeili

    2015-03-01

    Full Text Available  Minimal Change Disease (MCD is the leading cause of childhood Nephrotic Syndrome (NS. Therefore in pediatrics nephrotic syndrome, most children beyond the first year of life will be treated with corticosteroids without an initial biopsy. Children with NS often display a number of calcium homeostasis disturbances causing abnormal bone histology, including hypocalcemia, reduced serum vitamin D metabolites, impaired intestinal absorption of calcium, and elevated levels of immunoreactive parathyroid hormone (iPTH. These are mainly attributed to the loss of a variety of plasma proteins and minerals in the urine as well as steroid therapy. Early diagnosis and management of these abnormalities, could prevent the growth retardation and renal osteodystrophy that affects children with nephrotic syndrome. Here we reviewed the literature for changes of calcium and vitamin D metabolism in nephrotic syndrome and its consequences on bones, also the effect of corticosteroid and possible preventive strategies that could be done to avoid long term outcomes in children. Although the exact biochemical basis for Changes in levels of calcium and vitamin D metabolites in patients with NS remains speculative; Because of the potential adverse effects of these changes among growing children, widespread screening for vitamin D deficiency or routine vitamin D supplementation should be considered.

  7. Cerebral venous thrombosis and secondary polycythemia in a case of nephrotic syndrome

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    Shankar Prasad Nagaraju

    2016-01-01

    Full Text Available Cerebral venous thrombosis (CVT and polycythemia are considered as rare and life threatening complications of nephrotic syndrome. We report an unusual combination of both these complications in a case of nephrotic syndrome due to minimal change disease that was treated successfully. There was prompt and complete remission of nephrotic syndrome with steroid therapy, concurrent with complete resolution of polycythemia and CVT.

  8. 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.

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

    Science.gov (United States)

    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.

  10. Mammary-type myofibroblastoma with the nephrotic syndrome.

    Science.gov (United States)

    Colbert, Gates B; Vankawala, Preksha; Kuperman, Michael B; Mennel, Robert G

    2016-07-01

    We describe a 23-year-old white man who presented with anasarca and a new periumbilical mass. He had preserved kidney function and laboratory findings consistent with nephrotic syndrome, including 9.7 g/day albuminuria. Serum serologies were positive for anti-SSa and anti-SSb and low complements but were negative for antinuclear antibody. Pathologic findings of the abdominal mass showed a mammary-type myofibroblastoma. A kidney biopsy revealed a diffuse proliferative and membranous immune-mediated glomerulonephritis with 10% interstitial fibrosis. This is a novel case of mammary-type myofibroblastoma associated with nephrotic syndrome mimicking a proliferative lupus pattern.

  11. Serum immunoglobulin E and interleukin-13 levels in children with idiopathic nephrotic syndrome.

    Science.gov (United States)

    Mishra, Om P; Teli, Akkatai S; Singh, Usha; Abhinay, Abhishek; Prasad, Rajniti

    2014-12-01

    Serum IgE and IL-13 levels were estimated in 40 idiopathic nephrotic syndrome and 16 controls. There were 15 first episode nephrotic syndrome (FENS), 15 infrequent relapsing nephrotic syndrome (IRNS) and 10 patients belonged to frequent relapsing nephrotic syndrome (FRNS). Serum IgE and IL-13 levels were significantly increased in active nephrotic syndrome and its sub-groups as compared to controls and remission (p Il-13 was significantly higher in FRNS in comparison with FENS (p = 0.041). Both IgE and IL-13 levels were comparable in nephrotic patients with and without bronchial asthma. Serum IL-13 had significant positive correlation with IgE (r = 0.605, p IL-13 are found in nephrotic syndrome and could have a role in the pathogenesis of disease.

  12. Mycophenolate mofetil versus cyclosporine for remission maintenance in nephrotic syndrome

    NARCIS (Netherlands)

    E.M. Dorresteijn (Eiske); J.E. Kist-Van Holthe (Joana); E.N. Levtchenko (Elena); J. Nauta (Jeroen); W.C.J. Hop (Wim); A.J. van der Heijden (Bert)

    2008-01-01

    textabstractWe performed a multi-centre randomized controlled trial to compare the efficacy of mycophenolate mofetil (MMF) to that of cyclosporine A (CsA) in treating children with frequently relapsing nephrotic syndrome and biopsy-proven minimal change disease. Of the 31 randomized initially select

  13. [Nephrotic syndrome. What is new since the 1988 study?].

    Science.gov (United States)

    Seves, M G; Brito, M J; Lamy, S; Luiz, P V; Bastos, G; Faleiro, M; Batista, J; De Sousa, J F

    1998-07-01

    The authors make a retrospective review of 53 new cases of Nephrotic Syndrome followed up in the Nephrology Unit from November 1988 to March 1994, bearing in mind the evaluation of casual changes of the disease standard regarding a previous study of 1988. Epidemiological, clinical, therapeutical and evolutional aspects were studied. Forty-four cases of primary Nephrotic Syndrome (83%) were identified, 61.4% of which behaved as cortico-sensitive, 25% as cortico-dependent, and 13.6% as cortico-resistant; 8 cases (15%) of Nephrotic Syndrome secondary to infection, Systemic Lupus Erythematosus and Amyloidosis, and 1 case of congenital Nephrotic Syndrome (2%). The theory that the high number of cortico-dependent is, probably, related with a higher severity in the relapse diagnosis and/or changes in the children's standard of living is admissible. It was also observed that at present there is a lower number of hospital discharges, related to more careful attitudes adopted regarding the evolution of the disease.

  14. Steroid Resistant Nephrotic Syndrome-Genetic Consideration.

    Science.gov (United States)

    Tasic, Velibor; Gucev, Zoran; Polenakovic, Momir

    2015-01-01

    Nephrotic syndrome is defined as the association of massive proteinuria, hypoalbuminaemia, edema, and hyperlipidemia. It is separated to steroid-sensitive or steroid-resistant (SRNS) forms in respect to the response to intensive steroid therapy. SRNS usually progresses to end-stage renal failure. According to the North American Pediatric Renal Trials and Collaborative Studies SRNS constitutes the second most frequent cause of ESRD in the first two decades of life. Unfortunately, there is no curative treatment for majority of patients. Majority of the SRNS patients have the histologic picture of focal segmental glomerulosclerosis. Interestingly, the risk of recurrence in the kidney graft in patients with hereditary SRNS is lower than in those who do not have genetic background. The etiology and pathogenesis of SRSN has remained enigma for decades. The discovery of 39 dominant or recessive SRNS genes enabled better understanding of the function of the glomerular podocytes and slit membrane. Hildebrandt's group has shown that 85% of the SRNS cases with onset by 3 months of age and 66% with onset by 1 year of age can be explained by recessive mutations in one of four genes only (NPHS1, NPHS2, LAMB2, or WT1). The same group used modern diagnostic techniques such as the next generation sequencing and tested a large international cohort of SRNS patients (n = 1783 families). The diagnostic panel included 21 genes with a recessive mode of inheritance and 6 genes with a dominant mode of inheritance. Single-gene cause was detected in 29.5% (526 of 1783) of the families with SRNS that manifested before 25 years of age. The identification of causative single-gene mutations may have important therapeutic consequences in some cases. This is very important for patients who carry mutations in a gene of coenzyme Q10 biosynthesis (COQ2, COQ6, ADCK4, or PDSS2). In these patients the treatment with coenzyme Q10 may be indicated. Also, patients with recessive mutations in PLCE1 may

  15. Hypovolemia and hypovolemic shock in children with nephrotic syndrome.

    Science.gov (United States)

    Wang, S J; Tsau, Y K; Lu, F L; Chen, C H

    2000-01-01

    Hypovolemic shock is not an uncommon presentation in nephrotic syndrome, yet it is seldom mentioned in the literature. This study was performed to investigate the prevalence of hypovolemia and hypovolemic shock in the acute nephrotic stage, and the association of hemoconcentration and abdominal pain with hypovolemic status. Two hundred and twenty-five patients with a total of 328 admissions to the pediatric ward of our hospital during 1983 to 1996 were retrospectively reviewed for hypovolemic episodes. Clinical presentation and laboratory data including hemoglobin, serum sodium, albumin, cholesterol, and triglyceride levels were investigated. Thirteen patients with 19 episodes (5.8%) of hypovolemic shock were found, and had more severe hemoconcentration (hemoglobin 19.6 +/- 2.2 g/dL) and hyponatremia (127.3 +/- 7.2 mEq/L). Another 33 patients with 41 symptomatic hypovolemic episodes without hypotension (12.5%) were found, and their hemoglobin levels were higher compared to patients without hypovolemic symptoms. Among 61 episodes of abdominal pain and hemoconcentration, 58 were responsive to albumin infusion. This suggested the presence of hypovolemia. Twenty patients had abdominal pain without hemoconcentration, and 18 of them had primary peritonitis. Hypovolemia was found in patients at the acute nephrotic stage, and was usually associated with hemoconcentration and abdominal pain. A combined examination of hemoglobin and serum sodium is the best indicator of hypovolemic status. Both primary peritonitis and hypovolemic episodes should be taken into consideration when managing abdominal pain in children with nephrotic syndrome.

  16. Nephrotic Syndrome in Children: From Bench to Treatment

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    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.

  17. 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.

  18. [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.

  19. [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.

  20. 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; Praga, Manuel

    2010-04-01

    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.

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

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    Ying-Shou Chen

    2009-10-01

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

  2. 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

  3. 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.

  4. 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.

  5. Acute lymphoblastic leukaemia after treatment of nephrotic syndrome with immunosuppressive drugs.

    Science.gov (United States)

    Kuis, W; de Kraker, J; Kuijten, R H; Donckerwolcke, R A; Voûte, P A

    1976-06-01

    The authors present a 4-year-old girl with nephrotic syndrome who developed actue lymphoblastic leukaemia 5 months after the start of a combined treatment of alternate day prednisone and daily cyclophosphamide during 3 months. The nephrotic syndrome was due to focal segmental glomerulosclerosis. The occurrence of leukaemia might be related to the preceeding treatment with cyclophosphamide.

  6. Meningitis and intracranial bleed in a child with steroid-resistant nephrotic syndrome

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    Kanika Kapoor

    2015-01-01

    Full Text Available Meningitis and associated intracranial bleeding have been rarely reported in patients with steroid-resistant nephrotic syndrome. We present such a case with raised intracranial tension in a 13-year-old child and discuss the management issues. Prompt recognition and appropriate treatment of these complications can be life saving in a child with nephrotic syndrome.

  7. Meningitis and intracranial bleed in a child with steroid-resistant nephrotic syndrome.

    Science.gov (United States)

    Kapoor, Kanika; Saha, Abhijeet; Thakkar, Dhwanee; Dubey, N K; Vani, Kavita

    2015-11-01

    Meningitis and associated intracranial bleeding have been rarely reported in patients with steroid-resistant nephrotic syndrome. We present such a case with raised intracranial tension in a 13-year-old child and discuss the management issues. Prompt recognition and appropriate treatment of these complications can be life saving in a child with nephrotic syndrome.

  8. Familial steroid-sensitive idiopathic nephrotic syndrome: seven cases from three families in China

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    Yonghui Xia

    2013-05-01

    Full Text Available OBJECTIVES: Familial steroid-sensitive idiopathic nephrotic syndrome is rare, and only approximately 3% of patients have affected siblings. METHODS: Herein, we report seven cases of patients with steroid-sensitive idiopathic nephrotic syndrome from three Chinese families. Mutational screening of the Nphs2 gene was performed in all the patients. RESULTS: All seven of the familial steroid-sensitive idiopathic nephrotic syndrome cases in our sample exhibited minimal change disease, and one case also presented with mesangial proliferative glomerulonephritis, according to the renal pathology. No significant was associations were found between Nphs2 gene mutations and the onset of proteinuria and nephrotic syndrome in these familial cases. CONCLUSIONS: The presence of minimal change disease is important, but it is not an unusual finding in patients with familial steroid-sensitive idiopathic nephrotic syndrome, which appears to be clinically benign and genetically distinct from other types of nephrosis.

  9. 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.

  10. 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.

  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. 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.

  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.

  14. 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.

  15. Pulmonary embolism as the primary presenting feature of nephrotic syndrome

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    Pallavi Periwal

    2016-01-01

    Full Text Available A 36-year-old previously healthy male presented with subacute onset of shortness of breath and chest pain. He was diagnosed with bilateral extensive pulmonary embolism (PE. In the absence of any predisposing factors, an extensive workup for unprovoked thrombophilia was done. During the course of his illness, the patient developed anasarca and was diagnosed to be suffering from nephrotic syndrome (NS, secondary to membranous glomerulopathy. Although, thrombotic complications are commonly associated with NS, it is unusual for PE to be the primary presenting feature in these patients.

  16. Should we consider MMF therapy after rituximab for nephrotic syndrome?

    Science.gov (United States)

    Filler, Guido; Huang, Shih-Han Susan; Sharma, Ajay P

    2011-10-01

    The management of steroid-dependent nephrotic syndrome, especially in patients who have failed to respond to cytotoxic drugs, such as cyclophosphamide, remains challenging. Rituximab represents a new (off-label) therapeutic option. In a significant portion of patients, it has a short serum half-life following the recovery of CD20-positive cells. The addition of mycophenolate mofetil (MMF) as a maintenance therapy is also an attractive option, but one which requires testing in a prospective randomized clinical trial with therapeutic drug monitoring and mechanistic ancillary studies.

  17. History of Nephrotic Syndrome and Evolution of its Treatment

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    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.

  18. Influence of nephrotic state on the infectious profile in childhood idiopathic nephrotic syndrome Pacientes com síndrome nefrótica idiopática apresentam alterações na imunidade celular e humoral que predispõem a processos infecciosos

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    Emilia Maria Dantas Soeiro

    2004-01-01

    Full Text Available Patients with idiopathic nephrotic syndrome present alterations in their cellular and humoral immune reactions that predispose them to the development of infectious processes. PURPOSE: To characterize the infectious processes in patients with idiopathic nephrotic syndrome. PATIENTS AND METHODS: Ninety-two children and adolescents with idiopathic nephrotic syndrome were assessed retrospectively. The types of infection were grouped as follows: upper respiratory tract infections; pneumonia; skin infections; peritonitis; diarrhea; urinary tract infection ; herpes virus; and others. The patients were divided into 2 groups: Group I (steroid-responsive n = 75, with 4 subgroups-IA (single episode n = 10, IB (infrequent relapsers n = 5, IC (frequent relapsers n = 14, and ID (steroid-dependent n = 46; and Group II (steroid-resistant n = 17. The incidence-density of infection among the patients was assessed throughout the follow-up period. Comparisons for each group and subgroup were done during the periods of negative and nephrotic proteinuria. RESULTS: The analysis revealed a greater incidence-density of infections during the period of nephrotic proteinuria in all the groups and subgroups, with the exception of subgroup IA. During the period of nephrotic proteinuria, subgroups IC, ID, and Group II presented a greater incidence-density of infections as compared to subgroup IA. For the period of negative proteinuria, there was no difference in the incidence-density of infections between the groups and subgroups. Upper respiratory tract infections were the most frequent infectious processes. CONCLUSION: The nephrotic condition, whether as part of a course of frequent relapses, steroid dependence, or steroid resistance, conferred greater susceptibility to infection among the patients with idiopathic nephrotic syndrome. The results of this study suggest that the best preventive action against infection in this disease is to control the nephrotic state

  19. [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.

  20. 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.

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

    Directory of Open Access Journals (Sweden)

    Bong Soo Park

    2014-09-01

    Full Text Available 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.

  2. Skin grafting for necrotizing fasciitis in a child with nephrotic syndrome.

    Science.gov (United States)

    Bagri, Narendra; Saha, Abhijeet; Dubey, Nandkishore K; Rai, Ashish; Bhattacharya, Sameek

    2013-11-01

    Necrotizing fasciitis is a rare complication of nephrotic syndrome in children, with a high mortality rate. We report a case with successful outcome with judicious intravenous antibiotics and skin grafting of the bilateral lower thighs.

  3. Dysregulation of ENaC in Animal Models of Nephrotic Syndrome and Liver Cirrhosis

    OpenAIRE

    Kim,Soo Wan

    2006-01-01

    Nephrotic syndrome and liver cirrhosis are common clinical manifestations, and are associated with avid sodium retention leading to the development of edema and ascites. However, the mechanism for the sodium retention is still incompletely understood and the molecular basis remains undefined. We examined the changes of sodium (co)transporters and epithelial sodium channels (ENaCs) in the kidneys of experimental nephrotic syndrome and liver cirrhosis. The results demonstrated that puromycin- o...

  4. [Kimura's disease: an unrecognized cause of adult-onset nephrotic syndrome with minimal change disease].

    Science.gov (United States)

    Shehwaro, N; Langlois, A-L; Gueutin, V; Debchi, L; Charlotte, F; Rouvier, P; Rottembourg, J; Izzedine, H

    2014-02-01

    Kimura's disease (KD) is an angiolymphoid proliferative disorder of soft tissue with eosinophilia, with a predilection for head and neck regions in young Oriental men. Kidney disease is thought to be rare in KD. About a case of adult-onset nephrotic syndrome with minimal change disease, we comment Kimura's disease and its associated kidney damage. Kimura disease should be suspected and included in the diagnosis of adult-onset nephrotic syndrome with minimal change disease.

  5. 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.

  6. Pneumococcal sepsis, peritonitis, and cellulitis at the first episode of nephrotic syndrome.

    Science.gov (United States)

    Naseri, Mitra

    2013-09-01

    Bacterial infections are common in patients with nephrotic syndrome, including peritonitis, sepsis, meningitis, urinary tract infection, and cellulitis. An 8-year-old boy presented with colicky abdominal pain, vomiting, swollen and painful erythematous lesions around the umbilicus and in anterior surface of left thigh (cellulitis), mild generalized edema, and ascites. The microorganism isolated from peritoneal fluid and blood cultures was Pneumococcus. Association of pneumococcal sepsis, peritonitis, and cellulitis has been rarely reported in nephrotic syndrome.

  7. Profound nephrotic syndrome in a patient with ovarian teratoma

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    Abdallah Jeroudi

    2013-01-01

    Full Text Available The nephrotic syndrome (NS has been associated with a variety of malignancies in a number of reports in the literature, but has been reported in only nine cases associated with ovarian neoplasms. Membranous nephropathy is the most common glomerular pathology causing the NS in patients with solid tumors. There has been only one report of an ovarian neoplasm associated with minimal change disease (MCD. We describe the case of a 36-year-old woman who presented with the NS secondary to biopsy-proven MCD, likely secondary to mature ovarian teratoma. Treatment by tumor removal and prednisone led to remission of the NS. To the best of our knowledge, this is the first report of an ovarian teratoma and the second report of an ovarian neoplasm associated with MCD.

  8. 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 .

  9. 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.

  10. 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.

  11. 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

  12. 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.

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

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    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.

  14. Myocardial Infarction in a Young Man With Nephrotic Syndrome.

    Science.gov (United States)

    Chu, Zhenliang; Zhu, Hang; Zhang, Bin; Jiang, Liqin

    2017-03-17

    A 26-year-old man diagnosed with nephrotic syndrome (NS) 2 years previously presented with chest pain. An electrocardiogram (ECG) performed at a local hospital showed ST-elevation in chest leads. Cardiac troponin-I was significantly positive. Echocardiography revealed mild regional wall-motion abnormalities in the heart apex. Seven days later, angiography (CAG) revealed a thrombus in the left anterior descending branch (LAD). Tirofiban was injected into the LAD for thromboclasis. ECG after CAG showed the ST-segment was much lower than before. The diagnosis after CAG was ST-segment elevation myocardial infarction (MI) and thrombogenesis in the LAD. He continued to receive antiplatelet and anticoagulation medication and atorvastatin after CAG, and was discharged 3 days later. MI is very rare in young males, but the incidence of MI is 8 times higher than normal in patients with NS. For young patients with MI, clinicians should pay more attention to the history of previous diseases with high risk of thromboembolism and they should actively promote prevention and the treatment of renal disease patients to reduce the incidence of complications of thromboembolism.

  15. 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.

  16. Neuromyelitis optica accompanied by nephrotic syndrome and autoimmune-related pancytopenia.

    Science.gov (United States)

    ZhangBao, Jingzi; Zhou, Lei; Lu, Jiahong; Xi, Jianying; Zhao, Chongbo; Quan, Chao

    2016-05-01

    Neuromyelitis optica (NMO) associated with nephrotic syndrome and autoimmune-related pancytopenia has not been reported previously. We report herein a young woman who initially presented with bilateral blurring of vision and numbness in her hands. MRI disclosed multiple white matter lesions and a long cervical spinal cord lesion extending to the medulla oblongata. Serum aquaporin-4 antibody was positive and the patient was diagnosed with NMO. While in the hospital, she presented with hypoproteinemia and heavy proteinuria, meeting the diagnostic criteria of nephrotic syndrome. After high-dose methylprednisolone treatment, her vision improved significantly and urine protein quantity decreased. However, the patient subsequently developed severe pancytopenia with a positive Coombs' test. Thrombocytopenia finally led to uncontrollable gastrointestinal bleeding as the direct cause of the patient's death. This case illustrates the extremely rare condition of concurrence of NMO, nephrotic syndrome, and autoimmune pancytopenia in one patient, which suggests the involvement of organs beyond the central nervous system in NMO spectrum disorders.

  17. Nephrotic syndrome of minimal change disease following exposure to mercury-containing skin-lightening cream.

    Science.gov (United States)

    Zhang, Lin; Liu, Fuyou; Peng, Youming; Sun, Lin; Chen, Chunguo

    2014-01-01

    A 28-year-old female suffered from nephrotic syndrome after a long-term use of mercury-containing, skin-lightening cream. The blood and urinary mercury content of this patient increased with use. Renal biopsy showed minimal change disease. Her symptoms were relieved 6 months after discontinuing use of the cream and receiving sodium dimercaptosulfonate and glucocorticosteroid treatments. Proteinuria disappeared, and blood and urinary mercury levels returned to normal. Previous reports of nephrotic syndrome caused by mercury-containing, skin-lightening creams have mostly been identified as be.ing due to membranous nephropathy. Minimal change disease has been reported in a few case reports published in the English language. Here we report a case of nephrotic syndrome with minimal change disease following exposure to a mercury-containing, skin-lightening cream. We also reviewed relevant published reports to summarize clinical features and treatments and to explore the possible mechanisms involved.

  18. Assessment on Application of Chinese Herbal Medicine in the Process of Corticosterone Reduction in Nephrotic Syndrome

    Institute of Scientific and Technical Information of China (English)

    2001-01-01

    Objective: To evaluate the effect of Chinese herbal medicine (CHM) in the process of corticosterone (CS) reduction in nephrotic syndrome. Methods: One hundred and twenty patients of idiopathic nephrotic syndrome sensitive to prednisone treatment were divided into two groups randomly. The dosage of prednisone used was conventionally reduced in both groups but CHM herbal treatment was added to the treated group in the period of prednisone reduction. The effect of CHM was estimated by observing the recurrence rate of disease and side-effects of prednisone. Results: The recurrence rate of disease and the occurrence rate of side-effects of prednisone in the treated group were less than those in the control group significantly (P<0.01). Conclusion: Additional CHM in the process of CS reduction in treating CS sensitive nephrotic syndrome could markedly abate the recurrence rate of disease and the side-effect of CS reduction.

  19. 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.

  20. 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

  1. Nephroprotective effect of heparanase in experimental nephrotic syndrome.

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    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.

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

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    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.

  3. Atrial natriuretic factor in the acute nephritic and nephrotic syndromes.

    Science.gov (United States)

    Rodríguez-Iturbe, B; Colic, D; Parra, G; Gutkowska, J

    1990-09-01

    Because the role of systemic hormones in the pathophysiology of edema in acute renal disease remains incompletely understood, we compared the levels of atrial natriuretic factor (ANF) and plasma renin activity (PRA) in patients with acute glomerulonephritis (AGN), nephrotic syndrome (NS), and normal individuals during salt deprivation and salt loading. Sixteen patients with AGN (10 males) and nine patients with NS and hypoalbuminemia (7 males) were studied on admission, and after recovery (12 AGN patients) or remission (4 NS patients). Eighteen normal controls were each studied after five days on a low (20 mEq Na/day), regular (120 mEq Na/day) and high (300 mEq Na/day) dietary salt intake. Patients with AGN and NS had comparable edema (AGN 2.8 +/- 0.53 kg; NS 3.36 +/- 0.47 kg; SE) and urinary Na excretion (mean +/- SEM: AGN 0.97 +/- 0.11 mEq/hr; NS 1.06 +/- 0.16 mEq/hr), but AGN patients had five times higher ANF (AGN 27.2 +/- 4.06 fmol/ml; NS 5.51 +/- 1.02 fmol/ml; P less than 0.001) and six times lower PRA ng/liter.sec levels (AGN 0.187 +/- 0.047; NS 1.144 +/- 0.222; P less than 0.001) than NS patients. The degree of edema was correlated with ANF levels in AGN patients (P less than 0.001) but not in NS patients. There was a strong exponential negative correlation (r = -0.773, P less than 0.0001) between ANF and PRA, in which AGN patients and Na-restricted controls were located in the opposite ends of the volume sensing-response, and NS patients in the middle, alongside controls with regular Na intake.(ABSTRACT TRUNCATED AT 250 WORDS)

  4. 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

  5. Nephrotic Syndrome and Idiopathic Membranous Nephropathy Associated with Autosomal-Dominant Polycystic Kidney Disease

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    Ramón Peces

    2011-01-01

    Full Text Available We report the case of a 38-year-old male with autosomal-dominant polycystic kidney disease (ADPKD and concomitant nephrotic syndrome secondary to membranous nephropathy (MN. A 3-month course of prednisone 60 mg daily and losartan 100 mg daily resulted in resistance. Treatment with chlorambucil 0.2 mg/kg daily, low-dose prednisone, plus an angiotensin-converting enzyme inhibitor (ACEI and an angiotensin II receptor blocker (ARB for 6 weeks resulted in partial remission of his nephrotic syndrome for a duration of 10 months. After relapse of the nephrotic syndrome, a 13-month course of mycophenolate mofetil (MFM 2 g daily and low-dose prednisone produced complete remission for 44 months. After a new relapse, a second 24-month course of MFM and low-dose prednisone produced partial to complete remission of proteinuria with preservation of renal function. Thirty-six months after MFM withdrawal, complete remission of nephrotic-range proteinuria was maintained and renal function was preserved. This case supports the idea that renal biopsy is needed for ADPKD patients with nephrotic-range proteinuria in order to exclude coexisting glomerular disease and for appropriate treatment/prevention of renal function deterioration. To the best of our knowledge, this is the first reported case of nephrotic syndrome due to MN in a patient with ADPKD treated with MFM, with remission of proteinuria and preservation of renal function after more than 10 years. Findings in this patient also suggest that MFM might reduce cystic cell proliferation and fibrosis, preventing progressive renal scarring with preservation of renal function.

  6. Nephrotic syndrome and idiopathic membranous nephropathy associated with autosomal-dominant polycystic kidney disease.

    Science.gov (United States)

    Peces, Ramón; Martínez-Ara, Jorge; Peces, Carlos; Picazo, Mariluz; Cuesta-López, Emilio; Vega, Cristina; Azorín, Sebastián; Selgas, Rafael

    2011-05-05

    We report the case of a 38-year-old male with autosomal-dominant polycystic kidney disease (ADPKD) and concomitant nephrotic syndrome secondary to membranous nephropathy (MN). A 3-month course of prednisone 60 mg daily and losartan 100 mg daily resulted in resistance. Treatment with chlorambucil 0.2 mg/kg daily, low-dose prednisone, plus an angiotensin-converting enzyme inhibitor (ACEI) and an angiotensin II receptor blocker (ARB) for 6 weeks resulted in partial remission of his nephrotic syndrome for a duration of 10 months. After relapse of the nephrotic syndrome, a 13-month course of mycophenolate mofetil (MFM) 2 g daily and low-dose prednisone produced complete remission for 44 months. After a new relapse, a second 24-month course of MFM and low-dose prednisone produced partial to complete remission of proteinuria with preservation of renal function. Thirty-six months after MFM withdrawal, complete remission of nephrotic-range proteinuria was maintained and renal function was preserved. This case supports the idea that renal biopsy is needed for ADPKD patients with nephrotic-range proteinuria in order to exclude coexisting glomerular disease and for appropriate treatment/prevention of renal function deterioration. To the best of our knowledge, this is the first reported case of nephrotic syndrome due to MN in a patient with ADPKD treated with MFM, with remission of proteinuria and preservation of renal function after more than 10 years. Findings in this patient also suggest that MFM might reduce cystic cell proliferation and fibrosis, preventing progressive renal scarring with preservation of renal function.

  7. 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.

  8. Childhood myelodysplastic syndrome.

    Science.gov (United States)

    Chatterjee, Tathagata; Choudhry, V P

    2013-09-01

    Myelodysplastic syndrome (MDS) comprises of a heterogeneous group of bone marrow disorders resulting from a clonal stem cell defect characterised by cytopenias despite a relatively hypercellular marrow, ineffective hematopoiesis, morphological dysplasia in the marrow elements, no response to hematinics such as iron, B12 or folic acid and risk of progression to leukemia. Myelodysplastic syndrome in childhood is extremely rare and accounts for less than 5% of all hematopoietic neoplasms in children below the age of 14 y. The primary MDS in children, also known as de novo MDS differs from secondary MDS which generally follows congenital or acquired bone marrow (BM) failure syndromes as well as from therapy related MDS, commonly resulting from cytotoxic therapy. MDS associated with Down syndrome which accounts for approximately one-fourth of cases of childhood MDS is now considered a unique biologic entity synonymous with Down syndrome-related myeloid leukemia and is biologically distinct from other cases of childhood MDS. Refractory cytopenia of childhood (RCC) is the commonest type of MDS. Genetic changes predisposing to MDS in childhood remain largely obscure. Monosomy 7 is by-far the commonest cytogenetic abnormality associated with childhood MDS; however most cases of RCC show a normal karyotype. Complex cytogenetic abnormalities and trisomy 8 and trisomy 21 are also occasionally observed. The most effective and curative treatment is Hematopoietic stem cell transplantation and this is particularly effective in children with the monosomy 7 genetic defect as well as those displaying complex karyotype abnormalities provided it is instituted early in the course of the disease.

  9. 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 .

  10. 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.

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

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    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.

  12. 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...

  13. Maternal Environment Interacts with Modifier Genes to Influence Progression of Nephrotic Syndrome

    Science.gov (United States)

    Ratelade, Julien; Lavin, Tiphaine Aguirre; Muda, Andrea Onetti; Morisset, Ludivine; Mollet, Géraldine; Boyer, Olivia; Chen, Deborah S.; Henger, Anna; Kretzler, Matthias; Hubner, Norbert; Théry, Clotilde; Gubler, Marie-Claire; Montagutelli, Xavier; Antignac, Corinne; Esquivel, Ernie L.

    2008-01-01

    Mutations in the NPHS2 gene, which encodes podocin, are responsible for some cases of sporadic and familial autosomal recessive steroid-resistant nephrotic syndrome. Inter- and intrafamilial variability in the progression of renal disease among patients bearing NPHS2 mutations suggests a potential role for modifier genes. Using a mouse model in which the podocin gene is constitutively inactivated, we sought to identify genetic determinants of the development and progression of renal disease as a result of the nephrotic syndrome. We report that the evolution of renal disease as a result of nephrotic syndrome in Nphs2-null mice depends on genetic background. Furthermore, the maternal environment significantly interacts with genetic determinants to modify survival and progression of renal disease. Quantitative trait locus mapping suggested that these genetic determinants may be encoded for by genes on the distal end of chromosome 3, which are linked to proteinuria, and on the distal end of chromosome 7, which are linked to a composite trait of urea, creatinine, and potassium. These loci demonstrate epistatic interactions with other chromosomal regions, highlighting the complex genetics of renal disease progression. In summary, constitutive inactivation of podocin models the complex interactions between maternal and genetically determined factors on the progression of renal disease as a result of nephrotic syndrome in mice. PMID:18385421

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

    Directory of Open Access Journals (Sweden)

    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.

  15. 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

  16. 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

  17. 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...

  18. 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.

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

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    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.

  20. Population pharmacokinetics of levamisole in children with steroid-sensitive nephrotic syndrome

    NARCIS (Netherlands)

    Kreeftmeijer-Vegter, A.R.; Dorlo, T.P.C.; Gruppen, M.P.; De Boer, A.; De Vries, P.J.

    2015-01-01

    Aim The aim was to investigate the population pharmacokinetics of levamisole in children with steroid-sensitive nephrotic syndrome. Methods Non-linear mixed effects modelling was performed on samples collected during a randomized controlled trial. Samples were collected from children who were receiv

  1. 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

  2. [Ocular toxocariasis in a boy with idiopathic nephrotic syndrome - a case report].

    Science.gov (United States)

    Antonowicz, Agnieszka; Skrzypczyk, Piotr; Kępa, Beata; Pańczyk-Tomaszewska, Małgorzata

    2016-10-19

    Toxocariasis is a common zoonosis caused by infection with Toxocara canis or cati larvae. Ocular toxocariasis is one of the forms of infestation found in 1/1,000 - 1/10,000 children. Children with idiopathic nephrotic syndrome (INS) are at high risk of infections, also parasitic infestations, which can, in turn, cause relapses of the disease.

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

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    Nima Derakhshan

    2011-01-01

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

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

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    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.

  5. Dysregulation of ENaC in Animal Models of Nephrotic Syndrome and Liver Cirrhosis.

    Science.gov (United States)

    Kim, Soo Wan

    2006-03-01

    Nephrotic syndrome and liver cirrhosis are common clinical manifestations, and are associated with avid sodium retention leading to the development of edema and ascites. However, the mechanism for the sodium retention is still incompletely understood and the molecular basis remains undefined. We examined the changes of sodium (co)transporters and epithelial sodium channels (ENaCs) in the kidneys of experimental nephrotic syndrome and liver cirrhosis. The results demonstrated that puromycin- or HgCl2-induced nephrotic syndrome was associated with 1) sodium retention, decreased urinary sodium excretion, development of ascites, and increased plasma aldosterone level; 2) increased apical targeting of ENaC subunits in connecting tubule and collecting duct segments; and 3) decreased protein abundance of type 2 11β-hydroxysteroid dehydrogenase (11βHSD2). Experimental liver cirrhosis was induced in rats by CCl4 treatment or common bile duct ligation. An increased apical targeting of alpha-, beta-, and gamma-ENaC subunits in connecting tubule, and cortical and medullary collecting duct segments in sodium retaining phase of liver cirhosis but not in escape phase of sodium retention. Immunolabeling intensity of 11βHSD2 in the connecting tubule and cortical collecting duct was significantly reduced in sodium retaining phase of liver cirrhosis, and this was confirmed by immunoblotting. These observations therefore strongly support the view that the renal sodium retention associated with nephrotic syndrome and liver cirrhosis is caused by increased sodium reabsorption in the aldosterone sensitive distal nephron including the connecting tubule and collecting duct, and increased apical targeting of ENaC subunits plays a role in the development of sodium retention in nephrotic syndrome and liver cirrhosis.

  6. 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.

  7. Posterior reversible encephalopathy syndrome in a 5-year-old boy with steroid-dependent nephrotic syndrome

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    Md. Habibur Rahman

    2016-01-01

    Full Text Available Posterior reversible encephalopathy syndrome (PRES is a rare clinical and radiological phenomenon is encountered in children compared to adults. In our center, a 5-yearold boy with steroid-dependent nephrotic syndrome (SDNS presented with headache and blurring of vision during relapse after a long course of immunosuppressive therapy. Evaluation by computed tomography scan of the brain showed that the child had hypodense areas throughout the occipital region of the brain. All signs of PRES, except papilledema, resolved after seven days of supportive treatment evidenced by subsequent radiological evaluation. PRES should be kept in mind in any nephrotic child who is on prolonged immunosuppressive therapy.

  8. 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.

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

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    H. Abensur; Nussenzveig,I.; L.B. Saldanha; M.S.C. Petalozzi; Barros,M.T.; M. Marcondes; R.T. Barros

    1992-01-01

    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 ...

  10. Increased fasting plasma acylation-stimulating protein concentrations in nephrotic syndrome.

    Science.gov (United States)

    Ozata, Metin; Oktenli, Cagatay; Gulec, Mustafa; Ozgurtas, Taner; Bulucu, Fatih; Caglar, Kayser; Bingol, Necati; Vural, Abdulgaffar; Ozdemir, I Caglayan

    2002-02-01

    Acylation-stimulating protein (ASP) is an adipocyte-derived protein that has recently been suggested to play an important role in the regulation of lipoprotein metabolism and triglyceride (TG) storage. ASP also appears to have a role in the regulation of energy balance. In addition to its role as a hormonal regulator of body weight and energy expenditure, leptin is now implicated as a regulatory molecule in lipid metabolism. However, little is known about the alterations in fasting plasma ASP and leptin concentrations in the nephrotic syndrome. As hyperlipidemia is one of the most striking manifestations of the nephrotic syndrome, we have investigated fasting plasma ASP and leptin levels and their relation to lipid levels in this syndrome. Twenty-five patients with untreated nephrotic syndrome and 25 age-, sex-, and body mass index-matched healthy controls were included in the study. Fasting plasma lipoproteins, TG, total cholesterol, lipoprotein(a), apolipoprotein AI (apoAI), apoB, urinary protein, plasma albumin, third component of complement (C3), ASP, and leptin levels were measured in both groups. Total cholesterol, TG, low and very low density lipoproteins, lipoprotein(a), apoB, and urinary protein levels were increased in the patient group, whereas plasma albumin, high density lipoprotein cholesterol, and apoAI levels were decreased compared with those in the control group (P Fasting ASP concentrations showed no correlation with body mass index, proteinuria, plasma albumin, leptin, or any lipid parameter in either group, but C3 levels (in patient group: r(s) = 0.92; P < 0.001; in control group: r(s) = 0.68; P < 0.001). Our findings showed that plasma ASP levels were significantly elevated, whereas leptin levels were normal in the nephrotic syndrome. Increased ASP levels in the setting of dyslipidemia in the nephrotic syndrome raise the possibility of an ASP receptor defect in adipocytes, which also suggests the existence of so-called ASP resistance. Moreover

  11. A RETROSPECTIVE STUDY ON CLINICAL PRESENTATION OF STEROID SENSITIVE NEPHROTIC SYNDROME

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    Sosamma M. M

    2016-09-01

    Full Text Available BACKGROUND Nephrotic syndrome is a disease affecting the renal system. Most paediatricians will invariably encounter children with nephrotic syndrome in their clinic. The disease is characterised by the presence of oedema, persistent heavy proteinuria, hypoproteinaemia and hypercholesterolaemia. The disease is influenced by factors like age, geography, race and also has certain genetic influence related to HLA (DR7, B12, B8. In children, minimal change nephrotic syndrome is the most common variant of primary nephrotic syndrome. It accounts to more than eighty per cent of the cases seen children under seven years whereas it has a chance of fifty per cent in the age group of seven to sixteen years. Males are affected two times higher compared to females. The parents usually bring the child to the hospital with signs of oedema. Usually, the child recovers with treatment, but in some cases, there can be relapse. MATERIALS AND METHODS  The study was conducted in the Department of Paediatrics, Travancore Medical College, Kollam.  The study was done from January 2015 to January 2016.  Sixty cases were identified and were chosen for the study. INCLUSION CRITERIA 1. Steroid sensitive cases of nephrotic syndrome. 2. Age less than twelve years. 3. Admitted cases. EXCLUSION CRITERIA 1. Steroid-resistant and steroid-dependent cases. 2. Age more than twelve years. 3. Outpatient cases. RESULTS Out of the sixty cases studied, forty one cases belonged to male sex and nineteen cases belonged to female sex. Based on the age group, maximum number of cases belonged to age group four to eight years, which amounted to thirty four cases followed by age group eight to twelve years, which amounted to eighteen cases. Age group zero to four years had the least number of cases, which amounted to eight in number. Based on clinical signs and symptoms, fifty five cases presented with oedema either periorbital, scrotal or pedal oedema. Ten cases presented with fever

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

    Science.gov (United States)

    Urbano, Flavia; Acquafredda, Angelo; Aceto, Gabriella; Penza, Rosa; Cavallo, Luciano

    2012-10-23

    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.

  13. Kinetics of Rituximab Excretion into Urine and Peritoneal Fluid in Two Patients with Nephrotic Syndrome

    Science.gov (United States)

    Schwarz, Anke; Wagner, A. D.; Haller, Hermann; Schiffer, Mario

    2017-01-01

    Clinical observations suggest that treatment of Rituximab might be less effective in patients with nephrotic range proteinuria when compared to nonnephrotic patients. It is conceivable that the reason for this is that significant amounts of Rituximab might be lost in the urine in a nephrotic patient and that these patients require a repeated or higher dosage. However, this has not been systematically studied. In this case report we describe two different patients with nephrotic range proteinuria receiving Rituximab. The first patient received Rituximab for therapy resistant cryoglobulinemic membranoproliferative glomerulonephritis and the other for second line treatment of Felty's syndrome. We employed flow cytometry to determine the amount of Rituximab excretion in both urine and peritoneal fluid specimens in these patients following administration of Rituximab. We found that a significant amount of Rituximab is lost from the circulation by excretion into the urine. Furthermore we saw a close correlation of the excretion of Rituximab to the excretion of IgG molecules suggesting selectivity of proteinuria as the determining factor of Rituximab excretion. Further larger scale clinical studies could have the potential to evaluate an optimal cut-off value of IgG urinary loss before a possible administration of Rituximab therefore contributing to a more individualized treatment approach in patients with nonselective and nephrotic range proteinuria.

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

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    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.

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

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    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.

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

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    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.

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

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    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.

  18. 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.

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

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    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.

  20. Nephrotic Syndrome without Hematuria due to Infection-Related Glomerulonephritis Mimicking Minimal-Change Disease in a Child.

    Science.gov (United States)

    Iwafuchi, Yoichi; Morioka, Tetsuo; Morita, Takashi; Watanabe, Kanako; Oyama, Yuko; Narita, Ichiei

    2016-01-01

    Nephrotic syndrome without hematuria due to infection-related glomerulonephritis is uncommon. The present report describes a case of nephrotic syndrome due to infection-related glomerulonephritis without hematuria and hypertension in an older child. A 14-year-old boy was referred to our hospital because of a 5-day history of fever, nausea, weight gain and recent leg edema without hypertension. Laboratory data showed nephrotic-range proteinuria, hypoalbuminemia, mild hypocomplementemia and acute renal injury without hematuria. Although, due to the clinical presentation, minimal-change nephrotic syndrome was mostly suspected, a renal biopsy showed endocapillary hypercellularity mainly of mononuclear cells with segmental mesangiolytic changes. Fine granular IgG and C3 deposits were noted by an immunofluorescent study; many relatively small electron-dense deposits were observed electron-microscopically. These findings led to the diagnosis of nephrotic syndrome due to infection-related endocapillary proliferative glomerulonephritis, although the causative organism of his nephritis was not detected. He recovered with rest and dietary cure. When we examine an acute nephrotic child, infection-related glomerulonephritis should be considered as the differential diagnosis to avoid unnecessary use of corticosteroids.

  1. The risk of urinary tract infection in children with nephrotic syndrome

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    Patricia Y. Gunawan

    2016-08-01

    Full Text Available Background Urinary tract infections (UTI may affect any part of the urinary system: the kidneys, ureters, bladder, or urethra. Nephrotic syndrome (NS is the most common glomerular disorder in childhood, comprising a group of symptoms that include proteinuria, hypoalbuminemia, hypercholesterolemia, and edema. The prevalence of UTI in NS patients is high, around 25-66.7%. The increased prevalence of UTI in NS is due to immunoglobulin loss, defective T cell function, the presence of ascites, and relative malnutrition. Objective To study the risk of UTI in children with NS. Methods We performed a retrospective study of NS and UTI patients from January 2004 to December 2013 in the Division of Nephrology at Prof. Dr. R.D. Kandou Hospital, Manado. Data was collected from medical records. Diagnosis of UTI was made based on urine culture results. Diagnosis of NS was made based on the group of symptoms mentioned above. Analysis was done using Chi-square test with SPSS version 22 software. Results Of 74 NS patients, 34 (46% had UTIs. During the same study period, 117 patients had UTIs. NS was more common in boys (64.9%, while NS with UTI was more common in girls (67.6%. The most common organisms causing UTI in NS patients were Eschericia coli and Citrobacter diversus (23% each. Imipenem and amikacin were most commonly used antibiotics to which the bacteria were sensitive. Increased risk of UTI was significant in children with NS (OR 1.8; P=0.03. Conclusion Children with NS are at significantly increased risk of UTIs.

  2. Podocin inactivation in mature kidneys causes focal segmental glomerulosclerosis and nephrotic syndrome.

    Science.gov (United States)

    Mollet, Géraldine; Ratelade, Julien; Boyer, Olivia; Muda, Andrea Onetti; Morisset, Ludivine; Lavin, Tiphaine Aguirre; Kitzis, David; Dallman, Margaret J; Bugeon, Laurence; Hubner, Norbert; Gubler, Marie-Claire; Antignac, Corinne; Esquivel, Ernie L

    2009-10-01

    Podocin is a critical component of the glomerular slit diaphragm, and genetic mutations lead to both familial and sporadic forms of steroid-resistant nephrotic syndrome. In mice, constitutive absence of podocin leads to rapidly progressive renal disease characterized by mesangiolysis and/or mesangial sclerosis and nephrotic syndrome. Using established Cre-loxP technology, we inactivated podocin in the adult mouse kidney in a podocyte-specific manner. Progressive loss of podocin in the glomerulus recapitulated albuminuria, hypercholesterolemia, hypertension, and renal failure seen in nephrotic syndrome in humans. Lesions of FSGS appeared after 4 wk, with subsequent development of diffuse glomerulosclerosis and tubulointerstitial damage. Interestingly, conditional inactivation of podocin at birth resulted in a gradient of glomerular lesions, including mesangial proliferation, demonstrating a developmental stage dependence of renal histologic patterns of injury. The development of significant albuminuria in this model occurred only after early and focal foot process effacement had progressed to diffuse involvement, with complete absence of podocin immunolabeling at the slit diaphragm. Finally, we identified novel potential mediators and perturbed molecular pathways, including cellular proliferation, in the course of progression of renal disease leading to glomerulosclerosis, using global gene expression profiling.

  3. Effect of Poria cocos on Puromycin Aminonucleoside-Induced Nephrotic Syndrome in Rats.

    Science.gov (United States)

    Lee, So Min; Lee, Yun Jung; Yoon, Jung Joo; Kang, Dae Gill; Lee, Ho Sub

    2014-01-01

    Nephrotic syndrome is associated with altered renal handling of water and sodium and changes in the levels of aquaporins (AQPs) and epithelial Na channels (ENaCs). The dried sclerotia of Poria cocos Wolf (WPC) have been used for treating chronic edema and nephrosis. We evaluated the effects of WPC on puromycin aminonucleoside- (PAN-) induced renal functional derangement and altered renal AQP2 and ENaC expression. In the nephrotic syndrome rat model, animals were injected with 75 mg/kg PAN and then treated with Losartan (30 mg·kg(-1) ·day(-1)) or WPC (200 mg·kg(-1) ·day(-1)) for 7 days. In the WPC group, proteinuria and ascites improved significantly. Plasma levels of triglyceride, total cholesterol, and low-density lipoprotein- (LDL-) cholesterol reduced significantly in the WPC group. In addition, the WPC group exhibited attenuation of the PAN-induced increase in AQP2 and ENaC α/β subunit protein and mRNA levels. WPC suppressed significantly PAN-induced organic osmolyte regulators, reducing serum- and glucocorticoid-inducible protein kinase (Sgk1) and sodium-myo-inositol cotransporter (SMIT) mRNA expression. Our results show that WPC improves nephrotic syndrome, including proteinuria and ascites, through inhibition of AQP2 and ENaC expression. Therefore, WPC influences body-fluid regulation via inhibition of water and sodium channels, thereby, improving renal disorders such as edema or nephrosis.

  4. Podocin Inactivation in Mature Kidneys Causes Focal Segmental Glomerulosclerosis and Nephrotic Syndrome

    Science.gov (United States)

    Mollet, Géraldine; Ratelade, Julien; Boyer, Olivia; Muda, Andrea Onetti; Morisset, Ludivine; Lavin, Tiphaine Aguirre; Kitzis, David; Dallman, Margaret J.; Bugeon, Laurence; Hubner, Norbert; Gubler, Marie-Claire; Esquivel, Ernie L.

    2009-01-01

    Podocin is a critical component of the glomerular slit diaphragm, and genetic mutations lead to both familial and sporadic forms of steroid-resistant nephrotic syndrome. In mice, constitutive absence of podocin leads to rapidly progressive renal disease characterized by mesangiolysis and/or mesangial sclerosis and nephrotic syndrome. Using established Cre-loxP technology, we inactivated podocin in the adult mouse kidney in a podocyte-specific manner. Progressive loss of podocin in the glomerulus recapitulated albuminuria, hypercholesterolemia, hypertension, and renal failure seen in nephrotic syndrome in humans. Lesions of FSGS appeared after 4 wk, with subsequent development of diffuse glomerulosclerosis and tubulointerstitial damage. Interestingly, conditional inactivation of podocin at birth resulted in a gradient of glomerular lesions, including mesangial proliferation, demonstrating a developmental stage dependence of renal histologic patterns of injury. The development of significant albuminuria in this model occurred only after early and focal foot process effacement had progressed to diffuse involvement, with complete absence of podocin immunolabeling at the slit diaphragm. Finally, we identified novel potential mediators and perturbed molecular pathways, including cellular proliferation, in the course of progression of renal disease leading to glomerulosclerosis, using global gene expression profiling. PMID:19713307

  5. Minimal change nephrotic syndrome after stem cell transplantation: a case report and literature review

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    Silva Sandra

    2007-10-01

    Full Text Available Abstract Graft-versus-host disease is one of the most frequent complications occurring after haematopoietic stem cell transplantation. Recently, renal involvement has been described as a manifestation of chronic graft-versus-host disease. Immunosuppression seems to play a major role: clinical disease is triggered by its tapering and resolution is achieved with the resumption of the immunosuppressive therapy. Prognosis is apparently favourable, but long term follow up data are lacking. We report a case of a 53-year-old man who developed nephrotic syndrome 142 days after allogeneic stem cell transplantation for acute myeloid leukaemia. Onset of nephrotic syndrome occurred after reduction of immunosuppressants and was accompanied by manifestations of chronic graft-versus-host disease. Histological examination of the kidney was consistent with Minimal Change Disease. After treatment with prednisolone and mycophenolate mofetil he had complete remission of proteinuria and improvement of graft-versus-host disease. Eighteen months after transplantation the patient keeps haematological remission and normal renal function, without proteinuria. Since patients with chronic graft-versus-host disease might be considered at risk for development of nephrotic syndrome, careful monitoring of renal parameters, namely proteinuria, is advisable.

  6. Minimal change nephrotic syndrome after stem cell transplantation: a case report and literature review.

    Science.gov (United States)

    Silva, Sandra; Maximino, José; Henrique, Rui; Paiva, Ana; Baldaia, Jorge; Campilho, Fernando; Pimentel, Pedro; Loureiro, Alfredo

    2007-10-30

    Graft-versus-host disease is one of the most frequent complications occurring after haematopoietic stem cell transplantation. Recently, renal involvement has been described as a manifestation of chronic graft-versus-host disease. Immunosuppression seems to play a major role: clinical disease is triggered by its tapering and resolution is achieved with the resumption of the immunosuppressive therapy. Prognosis is apparently favourable, but long term follow up data are lacking.We report a case of a 53-year-old man who developed nephrotic syndrome 142 days after allogeneic stem cell transplantation for acute myeloid leukaemia. Onset of nephrotic syndrome occurred after reduction of immunosuppressants and was accompanied by manifestations of chronic graft-versus-host disease. Histological examination of the kidney was consistent with Minimal Change Disease. After treatment with prednisolone and mycophenolate mofetil he had complete remission of proteinuria and improvement of graft-versus-host disease. Eighteen months after transplantation the patient keeps haematological remission and normal renal function, without proteinuria.Since patients with chronic graft-versus-host disease might be considered at risk for development of nephrotic syndrome, careful monitoring of renal parameters, namely proteinuria, is advisable.

  7. Use of Rituximab in Children with Steroid- and Calcineurin-Inhibitor-Dependent Idiopathic Nephrotic Syndrome

    Science.gov (United States)

    Ravani, Pietro; Ponticelli, Alessandro; Siciliano, Chiara; Fornoni, Alessia; Magnasco, Alberto; Sica, Felice; Bodria, Monica; Caridi, Gianluca; Wei, Changli; Belingheri, Mirco; Ghio, Luciana; Merscher-Gomez, Sandra; Edefonti, Alberto; Pasini, Andrea; Montini, Giovanni; Murtas, Corrado; Wang, Xiangyu; Muruve, Daniel; Vaglio, Augusto; Martorana, Davide; Pani, Antonello; Scolari, Francesco; Reiser, Jochen; Ghiggeri, Gian Marco

    2013-01-01

    In children with idiopathic nephrotic syndrome rituximab can maintain short-term remission with withdrawal of prednisone and calcineurin-inhibitors. Long-term effects including number of repeated infusions to maintain remission are unknown. We treated with rituximab 46 consecutive children with idiopathic nephrotic syndrome lasting for at least one year (6.3±4.1 years), who were maintained in remission with oral prednisone and calcineurin inhibitors. They received 1–5 rituximab courses during a median follow-up of three years (range 1–5). Oral agents were tapered after each infusion, and completely withdrawn within 45 days. Rituximab was well tolerated. Six-month probabilities of remission were 48% after the first infusion and 37% after subsequent infusions. One- and two-year-remission probabilities were respectively 20% and 10%. Median time intervals between complete oral-agent withdrawal and relapse were 5.6 and 8.5 months respectively following the first and subsequent courses. Time to reconstitution of CD20 cells correlated with the duration of remission, but was not associated with variation in FcyR, CD20 or SMPDL-3B polymorphisms. Podocyte Src phosphorylation was normal. Rituximab can be safely and repeatedly used as prednisone and calcineurin-inhibitor-sparing therapy in a considerable proportion of children with dependent forms of idiopathic nephrotic syndrome. Further research is needed to identify patients who will benefit most from rituximab therapy. PMID:23739238

  8. [Childhood periodic syndromes].

    Science.gov (United States)

    Cuvellier, J-C; Lépine, A

    2010-01-01

    This review focuses on the so-called "periodic syndromes of childhood that are precursors to migraine", as included in the Second Edition of the International Classification of Headache Disorders. Three periodic syndromes of childhood are included in the Second Edition of the International Classification of Headache Disorders: abdominal migraine, cyclic vomiting syndrome and benign paroxysmal vertigo, and a fourth, benign paroxysmal torticollis is presented in the Appendix. The key clinical features of this group of disorders are the episodic pattern and intervals of complete health. Episodes of benign paroxysmal torticollis begin between 2 and 8 months of age. Attacks are characterized by an abnormal inclination and/or rotation of the head to one side, due to cervical dystonia. They usually resolve by 5 years. Benign paroxysmal vertigo presents as sudden attacks of vertigo, accompanied by inability to stand without support, and lasting seconds to minutes. Age at onset is between 2 and 4 years, and the symptoms disappear by the age of 5. Cyclic vomiting syndrome is characterized in young infants and children by repeated stereotyped episodes of pernicious vomiting, at times to the point of dehydration, and impacting quality of life. Mean age of onset is 5 years. Abdominal migraine remains a controversial issue and presents in childhood with repeated stereotyped episodes of unexplained abdominal pain, nausea and vomiting occurring in the absence of headache. Mean age of onset is 7 years. Both cyclic vomiting syndrome and abdominal migraine are noted for the absence of pathognomonic clinical features but also for the large number of other conditions to be considered in their differential diagnoses. Diagnostic criteria, such as those of the Second Edition of the International Classification of Headache Disorders and the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition, have made diagnostic approach and management easier. Their diagnosis

  9. 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.

  10. 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.

  11. 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...

  12. Minimal Change Nephrotic Syndrome Which Was Most Likely Caused by Chronic Sinusitis.

    Science.gov (United States)

    Iwatani, Hirotsugu; Mori, Daisuke; Yamamoto, Satoko; Nakano, Chikako; Yamamoto, Ryohei; Masumura, Chisako; Shikina, Takashi; Imai, Takao; Inohara, Hidenori; Rakugi, Hiromi; Isaka, Yoshitaka

    2015-01-01

    A 33-year-old Japanese man was admitted with severe edema, and a renal biopsy confirmed minimal change nephrotic syndrome (MCNS). CT revealed his severe chronic sinusitis, and he first received antimicrobial therapy, which resulted in decreased proteinuria. The surgical operation for sinusitis resulted in the complete disappearance of proteinuria without corticosteroid or immunosuppressant therapy within one week. MCNS may be triggered by infection, but there are no previously reported cases of MCNS that is completely remitted by infection control alone. Therefore, we herein report the first case of MCNS that attained complete remission following therapy for chronic sinusitis alone, which suggests a strong etiology of chronic sinusitis for MCNS.

  13. A Case of Nephrotic Syndrome With Minimal-Change Disease and Waldenstrom's Macroglobulinemia.

    Science.gov (United States)

    Grabe, Darren W; Li, Bo; Haqqie, Syed S

    2013-12-01

    Kidney disease is a rare complication of Waldenstrom's macroglobulinemia. We report a case of nephrotic syndrome and minimal change disease in a patient with biopsy proven Waldenstrom's macroglobulinemia. The patient presented with over 12 grams of proteinuria and was successfully treated with oral prednisone over the course of 4 weeks. Repeat serum protein electrophoresis as well as serum immunoelectrophoresis revealed no paraproteins, urine analysis was negative for protein or blood by dipstick and spot urine protein was 9 mg/dL with creatinine of 101 mg/dL at time of last office visit. This case illustrates the successful treatment with corticosteroids alone with prolonged complete remission.

  14. 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.

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

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    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.

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

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    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

  17. [A case of AKI-caused minimal change nephrotic syndrome with concomitant pleuritis].

    Science.gov (United States)

    Watanabe, Renya; Abe, Yasuhiro; Sasaki, Masaru; Hamauchi, Aki; Yasunaga, Tomoe; Kurata, Satoshi; Yasuno, Tetsuhiko; Ito, Kenji; Sasatomi, Yoshie; Hisano, Satoshi; Nakashima, Hitoshi

    2016-01-01

    A twenty-year-old man complaining of chest pain was diagnosed as nephrotic syndrome complicated with pleural effusion and ascites. Despite treatment with antibiotics, his fever and high inflammatory reaction persisted. After hospitalization, his urine volume decreased and renal function had deteriorated. As he was suffering from dyspnea, hemodialysis was performed together with chest drainage. His pleural effusion was exudative, and IVIG treatment was added to the antibiotic treatment. He was diagnosed as suspected developed minimal change nephrotic syndrome (MCNS) and administered prednisolone intravenously. His renal function ameliorated as a result of this treatment, enabling him to withdraw from hemodialysis. Inflammatory reaction gradually decreased and his general condition improved. The result of a renal biopsy examination carried out after the hemodialysis treatment confirmed MCNS, which suggested that MCNS had induced acute kidney injury (AKI) atypically in this case. Generally AKI is not induced by MCNS in youth, but it may occur under severe inflammatory conditions. Physicians should be aware that MCNS in young patients may lead to the development of AKI requiring hemodialysis treatment.

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

    Science.gov (United States)

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

    2011-10-13

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

  19. 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.

  20. Influence of low birth weight on minimal change nephrotic syndrome in children, including a meta-analysis

    NARCIS (Netherlands)

    N. Teeninga (Nynke); M.F. Schreuder (Michiel); A. Bökenkamp (Arend); H.A.D.V.D. Waal; J.A.E.V. Wijk

    2008-01-01

    textabstractBackground. Low birth weight (LBW) has been shown to lead to a low nephron endowment with subsequent glomerular hyperfiltration. Additional renal disease can therefore be expected to have a more severe course. Minimal change nephrotic syndrome (MCNS) is a common chronic illness in childh

  1. D-Pencillamine induced nephrotic syndrome in 11-year old girl with wilson disease: A case report

    Directory of Open Access Journals (Sweden)

    Dr. Anil Kumar*

    2013-12-01

    Full Text Available D-pencillamine and zinc remains the first line of treatment for Wilson’s disease in India. Membranous glomerulopathy is most commonly associated with nephrotic syndrome secondary to d penicillamine but isolated cases of minimal change lesions are rarely reported.We report a pediatric patient with Wilson’s disease who developed nephrotic syndrome 9 months after starting D-pencillamine. After stopping D-pencillamine and with only zinc for maintanence, her proteinuria resolved within a week’s time with full dose of steroids for nephrotic syndrome.Wilson disease itself may have tubular dysfunction but glomerulopathy is rare Isolated minimal change disease can occur in a 11 – year old patient yet it is statistically more likely to occur in a much younger age group.The most likely cause of nephrotic syndrome in this child is due to the late complication of D-penicillamine. It also re – emphasizes the importance of early monitoring for proteinuria and the need to shift to an alternative agent if side effects develop

  2. Epithelioid pleural mesothelioma concurrently associated with miliary pulmonary metastases and minimal change nephrotic syndrome - A hitherto undescribed case.

    Science.gov (United States)

    Tsukamoto, Yoshitane; Otsuki, Taiichiro; Hao, Hiroyuki; Kuribayashi, Kozo; Nakano, Takashi; Kida, Aritoshi; Nakanishi, Takeshi; Funatsu, Eriko; Noguchi, Chihiro; Yoshihara, Shunya; Kaku, Koji; Hirota, Seiichi

    2015-12-01

    Malignant pleural mesothelioma (MPM) is the aggressive disease typically spreading along the pleural surface and encasing the lung, leading to respiratory failure or cachexia. Rare cases with atypical clinical manifestation or presentation have been reported in MPM. We experienced a unique case of MPM concurrently associated with miliary pulmonary metastases and nephrotic syndrome. A 73-year-old Japanese man with past history of asbestos exposure was referred to our hospital for the investigation of the left pleural effusion. Chest computed tomography showed thickening of the left parietal pleura. Biopsy specimen of the pleura showed proliferating epithelioid tumor cells, leading to the pathological diagnosis of epithelioid MPM with the aid of immunohistochemistry. After the diagnosis of MPM, chemotherapy was performed without effect. Soon after the clinical diagnosis of progressive disease with skull metastasis, edema and weight gain appeared. Laboratory data met the criteria of nephrotic syndrome, and renal biopsy with electron microscopic examination revealed the minimal change disease. Steroid therapy was started but showed no effect. Around the same time of onset of nephrotic syndrome, multiple miliary lung nodules appeared on chest CT. Transbronchial biopsy specimen of the nodules showed the metastatic MPM in the lung. The patient died because of the worsening of the general condition. To our knowledge, this is the first case of MPM concurrently associated with multiple miliary pulmonary metastases and nephrotic syndrome.

  3. 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

  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. Nephrotic syndrome due to minimal change disease secondary to spider bite: clinico-pathological case of a non-described complication of latrodectism.

    Science.gov (United States)

    Méndez, Gonzalo P; Enos, Daniel; Moreira, José Luis; Alvaredo, Fátima; Oddó, David

    2017-04-01

    The patient was an 18-year-old man who developed nephrotic syndrome after a 'wheat spider' bite (Latrodectus mactans). Due to this atypical manifestation of latrodectism, a renal biopsy was performed showing minimal change disease. The nephrotic syndrome subsided after 1 week without specific treatment. This self-limited evolution suggests that the mechanism of podocyte damage was temporary and potentially mediated by a secondary mechanism of hypersensitivity or direct effect of the α-latrotoxin. The patient did not show signs of relapse in subsequent checkup. This is the first reported case of nephrotic syndrome due to a minimal change lesion secondary to latrodectism.

  6. 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.

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

    Directory of Open Access Journals (Sweden)

    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

  8. 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

  9. 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.

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

    DEFF Research Database (Denmark)

    Staehr, Mette; Buhl, Kristian B; Andersen, René F

    2015-01-01

    (uPA) in vitro. It was hypothesized that uPA is abnormally filtered to pre-urine and is inhibited in urine by amiloride in nephrotic syndrome. This was tested by determination of Na+-balance, uPA protein and activity and amiloride concentration in urine from rats with puromycin aminonucleoside (PAN......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......) induced nephrotic syndrome. Urine samples from 6 adult and 18 pediatric patients with nephrotic syndrome were analyzed for uPA activity and protein. PAN-treatment induced significant proteinuria in rats which coincided with increased urine uPA protein and activity, increased urine protease activity...

  11. 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.

  12. [Treating the symptoms in nephrotic syndrome: which therapeutic strategies are evidence based in the treatment of proteinuria?].

    Science.gov (United States)

    Kisner, T; Müller, R-U; Benzing, T

    2011-02-01

    Glomerular diseases are among the most common renal pathologies leading frequently to end-stage renal disease. Clinical disease can be divided into five different groups the features of which are determined by the underlying pathophysiology. One of these five clinical syndromes is the nephrotic syndrome, which is characterized by proteinuria > 3.5 g/day accompanied by hypalbuminemia, hyperlipoproteinemia and pronounced edema. The nephrotic syndrome may be the clinical manifestation of a row of underlying diseases. The pathophysiological basics had remained elusive for decades, yet recently significant progress which allows for establishing new therapeutic strategies has been made. A major breakthrough in understanding the function of the glomerular filter unit has been possible in the last years through both genetic and cell biological studies, which have revealed a crucial role for the visceral epithelial cells of the glomerulus - the podocytes. By now various factors have been found causing podocyte damage, such as toxines, immunological phenomena or systemic disease like diabetes mellitus.

  13. A case of abdominal aortic thrombosis associated with the nephrotic syndrome

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    Jannet Labidi

    2017-01-01

    Full Text Available Thromboembolic disease is an important and frequent complication in patients with the nephrotic syndrome (NS, and the consequences are often severe. Usually, the venous system is affected. Arterial thrombosis has rarely been reported and occurs mainly in children. We report the case of a 27-year-old man with a history of NS due to focal and segmental glomerulosclerosis resistant to steroids and cyclosporine, admitted for bilateral pain in the calves. Aortogram revealed a suspended thrombus in the abdominal aorta just below the origin of the renal arteries with embolism into the left tibioperoneal trunk and the right anterior tibial artery. Endarterectomy was performed followed by systemic heparinization with a good outcome. Arterial thrombosis is rare and must be prevented.

  14. Distribution of pathological finding in the children with nephrotic syndrome from Guangxi

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    Tian-Biao Zhou

    2014-01-01

    Full Text Available To identify the variations in pediatric renal biopsy pathology and clinicopathological features in Guangxi, China, in the past ten years, we studied retrospectively the kidney biopsies performed to evaluate the primary nephrotic syndrome (PNS in 218 children at two main medical centers in Guangxi from January 1999 to January 2009. The major pathological finding was mesangial proliferative glomerulonephritis (48.2%, focal segmental glomerulosclerosis (16.5%, immunoglobulin A nephropathy (13.3% and minimal change disease (11.0%. Patients with different pathological types yielded different response rates to glucocorticoids (P <0.001. There were statistical significant differences between prognosis for the different pathological types (P <0.05. The pathological characteristics of PNS in children were diverse and significant for guiding the grade of glucocorticoid response and predicting the prognosis of the PNS disease.

  15. Minimal change nephrotic syndrome associated with invasive thymoma: a case report with literature review.

    Science.gov (United States)

    Long, Quan; Wu, Ping; Jiang, Gengru; Zhu, Chun

    2014-04-01

    The present report describes a case of nephrotic syndrome (NS) with invasive thymoma. A male patient was hospitalized for severe edema with reduced urine output. He had a history of thymectomy and radiotherapy because of invasive thymoma 4 years before the development of NS. Renal biopsy displayed minimal change disease (MCD). Although imaging study showed probably recurrent sign of invasive thymoma, the patient still received steroid monotherapy for ~ 9 months and he got partial remission of NS at the 8th week. Therefore, we suggest that MCD should be taken into account as a pathological lesion type in old NS patients with thymoma. In spite of longer remission time, steroid monotherapy and combination therapy with immunosuppressant are effective for thymoma-associated MCD.

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

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    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.

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

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    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.

  18. 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

  19. An Estimation of Steroid Responsiveness of Idiopathic Nephrotic Syndrome in Iranian Children

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    Niloofar Hajizadeh

    2010-06-01

    Full Text Available Objective:Idiopathic Nephrotic syndrome (INS is the most common form of nephrotic syndrome (NS in children with the potential of progression to end stage renal disease (ESRD. INS is steroid-responsive in most children, but not all patients respond to it. The aim of this study was to determine the rate of steroid responsiveness in children with INS that referred to Childrens Medical Center since 1995 to 2007. Methods:In as a cross sectional study, the medical records of all children with INS aged 1 to 15 years who were referred to our referral hospital was reviewed. All patients with onset of disease less than 1 year of age, spontaneous remission, secondary forms of NS associated with systemic diseases, and follow up duration of less than 12 months were excluded from the study. Patients were categorized into 6 groups: Group 1 needed biopsy prior to any treatment, group 2 non-relapsing NS, group 3 infrequently relapsing NS, Group 4 frequently relapsing NS, group 5& steroid dependent NS and group 6 steroid resistant NS. Findings:A total of 238 patients were enrolled in the study. Kidney biopsy was performed in 79 cases. Minimal change lesion (MCL was the most common (36.7% pathological diagnosis. Steroid responsiveness was found in 81.5% of all cases including: 96% of MCL (consisting of biopsy proven cases and presumed ones, 32% of focal and segmental glomerulosclerosis, 73% of diffuse mesangial proliferation and 58% of membranoproliferative glomerulonephritis& patients. During minimal follow up period of 12 months, there were 194 patients in remission, 32 patients with active NS, and 12 patients in ESRD. Conclusion:Our study results showed that 81.5% of all patients, 96.2% of MCL and 32% of FSGS patients initially responded to steroid therapy.

  20. 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.

  1. Nephrotic syndrome

    Science.gov (United States)

    ... by: Charles Silberberg, DO, private practice specializing in nephrology, affiliated with New York Medical College, Division of Nephrology, Valhalla, NY. Review provided by VeriMed Healthcare Network. ...

  2. Nephrotic Syndrome

    Science.gov (United States)

    ... kidney disease. Diabetes can lead to kidney damage (diabetic nephropathy) that affects the glomeruli. Systemic lupus erythematosus. This ... will I always have it? What are my treatment options? And which do you recommend for me? ...

  3. Mesangioproliferative glomerulonephritis: An important glomerulonephritis in nephrotic syndrome of young adult

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    Usha

    2008-07-01

    Full Text Available Mesangioproliferative glomerulonephritis (MesPGN consists 10% of the total renal biopsy of glomerulonephritis. Aim of the present study was to find out clinicopathological changes in MesPGN and differences between diffuse and focal variety. MesPGN was seen mostly in young adults with mean age of 28.63 years for males and 26.3 years for females. Male predominance was noted (M:F ratio - 1.4:1. About 70.83% patient presented with edema feet, followed by hypertension (29.19%, fever (16.66%, oliguria, nausea and vomiting (10.41%. Urine analysis in 50 patients revealed that 70% patients presented with nephrotic-range proteinuria, 36% patients with microscopic hematuria and 56% patients with leukocyturia. Statistically, no significant difference was found in clinical features of diffuse and focal MesPGN. Microscopic comparison between diffuse and focal variety showed that significant increase of focal glomerular basement membrane thickening, focal endothelial cell proliferation, focal smooth muscle hyperplasia, hyaline sclerosis and vasculitis was more common in diffuse variety. In focal variety, Capillary loop congestion, periglomerulitis, cloudy swelling and vacuolar degeneration in tubules were significantly more as compared to diffuse variety. Details of the clinical features, special laboratory tests and histological details revealed that diffuse variety had systemic diseases, which included Wegner′s granulomatosis, microscopic polyangitis, Henoch′s schonlein purpura, systemic lupus erythematosus (two cases and one case each of Kimura′s disease, pyelonephritis and tuberculosis. Only one case of focal MesPGN showed tuberculosis. Thus, our study concludes that MesPGN is an important cause of nephrotic syndrome among young adults. Secondly, search for some other diseases should be made and thirdly, if biopsy shows focal mesangial cell proliferations in minimal change glomerulonephritis (MCGN, it should be diagnosed as focal MesPGN rather than

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

    Science.gov (United States)

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

    2008-01-01

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

  5. Astragalus in the Prevention of Upper Respiratory Tract Infection in Children with Nephrotic Syndrome: Evidence-Based Clinical Practice

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    Chuan Zou

    2013-01-01

    Full Text Available Aims. To explore whether Astragalus or its formulations could prevent upper respiratory infection in children with nephrotic syndrome and how best to use it. Methods. We transformed a common clinical question in practice to an answerable question according to the PICO principle. Databases, including the Cochrane Library (Issue 5, 2012, PUBMED (1966–2012.8, CBM (1978–2012.8, VIP (1989–2012.8, and CNKI (1979–2012.8, were searched to identify Cochrane systematic reviews and clinical trials. Then, the quality of and recommendations from the clinical evidence were evaluated using the GRADEpro software. Results. The search yielded 537 papers. Only two studies with high validity were included for synthesis calculations. The results showed that Astragalus granules could effectively reduce URTI in children with nephrotic syndrome compared with prednisone treatment alone (23.9% versus 42.9%; RR = 0.56 and 95% CI = 0.33–0.93. The dose of Astragalus granules was 2.25 gram (equivalent to 15 gram crude Astragalus twice per day, at least for 3–6 months. The level of evidence quality was low, but we still recommended the evidence to the patient according to GRADEpro with the opinion of the expert. Followup showed the incidence of URTI in this child decreased significantly. Conclusions. Astragalus granules may reduce the incidence of URTI in children with nephrotic syndrome.

  6. 用尿脱氧吡啶啉及羟脯氨酸排泄率评价肾病综合征患儿骨吸收功能状况%Evaluation bone resorption activity of childhood nephrotic syndrome with urinary deoxypyridinoline and hydroxyproline

    Institute of Scientific and Technical Information of China (English)

    窦志艳; 张会丰; 田秀巧; 耿佩琛; 刘艳平; 王丽芳

    2001-01-01

    目的 观察糖皮质激素(激素)对肾病综合征(肾病)患儿骨吸收功能的影响。方法 采用酶联免疫竞争法和分光光度比色法,对68例泼尼松治疗不同阶段的肾病患儿及同龄健康儿童16例尿脱氧吡啶啉(DPD)及羟脯氨酸(HOP)进行检测。结果 (1)激素足量治疗组DPD/肌酐(Cr)为(30±17) nmol/mmol,较正常对照组[(21±5) nmol/mmol]、初发激素治疗前组[(20±8) nmol/mmol]及激素减量治疗组[(20±11) nmol/mmol]均有升高(P均<0.05);(2)与DPD/Cr值变化趋势一致,激素足量治疗组HOP/Cr为[(5.3±2.7)mg/mmol],与正常对照组[(3.2±1.2)mg/mmol]、初发激素治疗前组[(3.5±0.9)mg/mmol]及激素减量治疗组[(3.7±1.7)mg/mmol]比较差异均有显著意义(P<0.05, <0.01, <0.05);(3)DPD/Cr与HOP/Cr两指标在正常对照组及肾病各组均呈明显正相关(r=0.64、0.65、0.76、0.78,P均<0. 01)。结论 超生理剂量的泼尼松治疗能使肾病患儿骨吸收功能增强,易导致骨质疏松。%Osteoporosis is one of the major side -effects in the treatment of nephritic syndrome with glucocorticoids, but the exact mechanism of osteopenia is not completely understood. Excess glucocorticoids will induce a reduction of bone formation as well as an increase of bone resorption on the other hand. Objective  To analyze the effect of glucocorticoid on the bone resorption activity of childhood nephrotic syndrome (CNS). Methods The bone resorption activities in 68 cases of CNS were evaluated by measuring urinary deoxypyridinoline (DPD) and urinary hydroxyproline (HOP). The patients were divided into three groups. The prednisone-pretreatment group included 25 children (19 boys and 6 girls) aged 4-11 years old without the prednisone treatment. The full dose prednisone group included 25 children (17 boys and 8 girls) aged 4-12 years old with the prednisone treatment [2 mg/(kg*d)] of 4-6 weeks. The

  7. Sphingosine-1-phosphate lyase mutations cause primary adrenal insufficiency and steroid-resistant nephrotic syndrome

    Science.gov (United States)

    Prasad, Rathi; Hadjidemetriou, Irene; Meimaridou, Eirini; Buonocore, Federica; Saleem, Moin; Hurcombe, Jenny; Bierzynska, Agnieszka; Barbagelata, Eliana; Bergadá, Ignacio; Cassinelli, Hamilton; Das, Urmi; Krone, Ruth; Hacihamdioglu, Bulent; Sari, Erkan; Yesilkaya, Ediz; Storr, Helen L.; Clemente, Maria; Fernandez-Cancio, Monica; Camats, Nuria; Ram, Nanik; Achermann, John C.; Van Veldhoven, Paul P.; Guasti, Leonardo; Braslavsky, Debora; Guran, Tulay; Metherell, Louise A.

    2017-01-01

    Primary adrenal insufficiency is life threatening and can present alone or in combination with other comorbidities. Here, we have described a primary adrenal insufficiency syndrome and steroid-resistant nephrotic syndrome caused by loss-of-function mutations in sphingosine-1-phosphate lyase (SGPL1). SGPL1 executes the final decisive step of the sphingolipid breakdown pathway, mediating the irreversible cleavage of the lipid-signaling molecule sphingosine-1-phosphate (S1P). Mutations in other upstream components of the pathway lead to harmful accumulation of lysosomal sphingolipid species, which are associated with a series of conditions known as the sphingolipidoses. In this work, we have identified 4 different homozygous mutations, c.665G>A (p.R222Q), c.1633_1635delTTC (p.F545del), c.261+1G>A (p.S65Rfs*6), and c.7dupA (p.S3Kfs*11), in 5 families with the condition. In total, 8 patients were investigated, some of whom also manifested other features, including ichthyosis, primary hypothyroidism, neurological symptoms, and cryptorchidism. Sgpl1–/– mice recapitulated the main characteristics of the human disease with abnormal adrenal and renal morphology. Sgpl1–/– mice displayed disrupted adrenocortical zonation and defective expression of steroidogenic enzymes as well as renal histology in keeping with a glomerular phenotype. In summary, we have identified SGPL1 mutations in humans that perhaps represent a distinct multisystemic disorder of sphingolipid metabolism. PMID:28165343

  8. 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.

  9. Nephrotic syndrome after allogeneic hematopoietic stem cell transplantation: etiology and pathogenesis.

    Science.gov (United States)

    Luo, Xiao-dan; Liu, Qi-fa; Zhang, Yu; Sun, Jing; Wang, Guo-bao; Fan, Zhi-ping; Yi, Zheng-shan; Ling, Yi-wen; Wei, Yong-qiang; Liu, Xiao-li; Xu, Bing

    2011-02-15

    In this study we investigated the etiology and pathogenesis of nephrotic syndrome (NS) after allogeneic hematopoietic stem cell transplantation (allo-HSCT) in 257 patients with hematopoietic malignancies who survived more than 2 months post allo-HSCT. Associations of NS with the conditioning regimen, graft versus host disease (GVHD), and other variables were analyzed. Pathologic features of the kidney, regulatory T cells (Tregs), interferon-γ (IFN-γ), and tumor necrosis factor-α (TNF-α) were studied. NS was identified in 9 patients. The number of Tregs at day+30, 60, 90, and 180 was lower in NS patients than non-NS patients (P=0.001, 0.001, 0.007, 0.003). Serum levels of IFN-γ and TNF-α were higher in NS patients (P=0.032, 0.001, respectively). NS post allo-HSCT was associated with the occurrence of chronic GVHD (P=0.02). NS post-HSCT is an immune disorder that may involve immune complex deposition, Th1 cytokines, and Tregs.

  10. Drug-induced interstitial nephritis in a child with idiopathic nephrotic syndrome

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    Printza Nikoleta

    2009-01-01

    Full Text Available Acute renal failure (ARF is a rare but severe complication of active idiopathic nephrotic syndrome (INS in children. It may be due to several causes with different outcomes. Both the clinical picture of the patient as well as laboratory, imaging and histopathological findings may help in the diagnosis. We present a case of drug-induced acute interstitial nephritis (AIN, complicated with ARF, in a 2½ -year-old girl with active INS. The child was referred to the Hippokration General Hospital, Thessaloniki, Greece hospital with steroid-resistant NS; renal biopsy was performed, which did not show any remarkable findings and cyclosporine was admi-nistered in addition to steroid therapy. The first day after biopsy, the child developed gross hematuria and abdominal pain and an antibiotic was added to her treatment. In the following days, fever, vomiting, hypertension and ARF occurred. Ultrasound study revealed enlarged kidneys with increased echogenity and loss of corticomedullary differentiation. The antibiotic and cyclos-porine were stopped and the child was managed with furosemide, nifedipine and steroids. A second renal biopsy was performed, which confirmed the diagnosis of acute interstitial nephritis. The child did not require dialysis therapy. Her urine output improved gradually and the serum creatinine normalized one month after the initial episode. Our case re-emphasizes the need for investigation of factors precipitating ARF in children with idiopathic NS.

  11. Concerted Action of ANP and Dopamine D1-Receptor to Regulate Sodium Homeostasis in Nephrotic Syndrome

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    Cátia Fernandes-Cerqueira

    2013-01-01

    Full Text Available The edema formation in nephrotic syndrome (NS is associated with a blunted response to atrial natriuretic peptide (ANP. The natriuretic effects of ANP have been related to renal dopamine D1-receptors (D1R. We examined the interaction between ANP and renal D1R in rats with puromycin aminonucleoside-induced NS (PAN-NS. Urinary sodium, cyclic guanosine monophosphate (cGMP excretion, and D1R protein expression and localization in renal tubules were evaluated in PAN-NS and control rats before and during volume expansion (VE. The effects of zaprinast (phosphodiesterase type 5 inhibitor, alone or in combination with Sch-23390 (D1R antagonist, were examined in both groups. The increased natriuresis and urinary cGMP excretion evoked by acute VE were blunted in PAN-NS despite increased levels of circulating ANP. This was accompanied in PAN-NS by a marked decrease of D1R expression in the renal tubules. Infusion of zaprinast in PAN-NS resulted in increased urinary excretion of cGMP and sodium to similar levels of control rats and increased expression of D1R in the plasma membrane of renal tubular cells. Combined administration of Sch-23390 and zaprinast prevented natriuresis and increased cGMP excretion induced by zaprinast alone. We conclude that D1R may play a major role in the ANP resistance observed in PAN-NS.

  12. Reduced 11beta-hydroxysteroid dehydrogenase activity in patients with the nephrotic syndrome.

    Science.gov (United States)

    Vogt, B; Dick, B; N'Gankam, V; Frey, F J; Frey, B M

    1999-02-01

    Patients with the nephrotic syndrome (NS) exhibit abnormal renal sodium retention which cannot completely explained by a secondary hyperaldosteronism due to reduced renal perfusion. As an alternative mechanism to explain this phenomenon we postulate a cortisol-mediated mineralocorticoid effect as a consequence of a reduced activity of 11beta-hydroxysteroid dehydrogenase (11beta-HSD). A down-regulation of 11beta-HSD, i.e. of the shuttle of active to inactive glucocorticosteroids, has been shown to cause mineralocorticoid effects. Therefore we investigated the activity of 11beta-HSD by measuring the urinary ratio of (tetrahydrocortisol + 5alpha-tetrahydrocortisol)/tetrahydrocortisone [(THF+5alpha-THF)/THE] by gas-chromatography in 29 NS patients with biopsy-proven glomerulonephritis and 29 healthy control subjects. The ratio of (THF+5alpha-THF)/THE was higher in NS patients (median 1.49, range 0.45-4.07) than in the control subjects (0.98, 0.60-1.36; pnew mechanism contributing to the exaggerated sodium retention in patients with the NS.

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

    Science.gov (United States)

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

    2007-01-01

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

  14. 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

  15. 儿童难治性肾病综合征的治疗进展%Progress in treatment of refractory nephrotic syndrome

    Institute of Scientific and Technical Information of China (English)

    许云鹏; 郭艳芳

    2014-01-01

    难治性肾病综合征(RNS)是临床上原发型肾病综合征(PNS)中频复发型(FRNS)、激素依赖型(SDNS)和激素耐药型(SRNS)肾病综合征的总称。按其对激素治疗是否敏感及尿蛋白转阴情况,可将原发性肾病综合征分为频复发型、激素依赖型和激素耐药型。临床观察发现80%~90%的PNS患儿经初始激素治疗即可获完全缓解,但有76%~93%的患儿复发,并超过半数的激素耐药型肾病儿童在10年内发展为终末期肾病(ESRD)。该文章就近年来关于儿童RNS临床治疗的方法作一综述。%Refractory nephrotic syndrome (RNS) is the generic terms of recurrence of intermediate frequency (frequently relapse nephrotic syndrome, FRNS), steroid dependence (steroid-dependent nephrotic syndrome, SDNS) and prednisone resistant type (steroid-resistant nephrotic syndrome, SRNS) in clinical nephrotic syndrome (primary nephrotic syndrome, PNS). According to the sensitivity to prednisone therapy and whether urinary protein is negative, recurrence of primary nephrotic syndrome can be divided into frequency, prednisone dependent and prednisone drug resistance. Clinical observation has found that 80%-90% of children with PNS after initial prednisone therapy can relieve completely, but have a relapse in 76%-93% in later days, and more than half of the prednisone resistant type kidney disease in children in ten years can progress end-stage renal disease (ESRD). The article summarized the clinical treatment of refractory nephrotic syndrome in children.

  16. 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

  17. Long term outcomes in children with steroid resistant nephrotic syndrome treated with calcineurin inhibitors

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    Nathan T Beins

    2015-11-01

    Full Text Available Background: Steroid resistant nephrotic syndrome (SRNS is an important cause of chronic kidney disease (CKD in children that often progresses to end-stage renal disease (ESRD. Calcineurin inhibitors (CNIs have been shown to be effective in inducing short-term remission in some patients with SRNS. However, there are little data examining their long-term impact on ESRD progression rates.Methods: We performed a retrospective chart review of all patients treated for SRNS with CNIs at our institution from 1995-2013. Data collected including demographics, initial response to medical therapy, number of relapses, progression to ESRD, and treatment complications.Results: A total of 16 patients met inclusion criteria with a mean follow-up of 6.6 years (range 0.6-17.6 years. Histopathological diagnoses were focal segmental glomerulosclerosis (8, mesangial proliferative glomerulonephritis (4, IgM nephropathy (3, and minimal change disease (1. Three patients (18.8% were unresponsive to CNIs while the remaining 13 (81.2% achieved remission with CNI therapy. Six patients (37.5% progressed to ESRD during the study period, three of whom did so after initially responding to CNI therapy. Renal survival rates were 87%, 71%, and 57% at 2 years, 5 years, and 10 years respectively. Non-Caucasian ethnicity was associated with progression to ESRD. Finally, a higher number of acute kidney injury episodes was associated with a lower final estimated glomerular filtration rate.Discussion: Despite the majority of SRNS patients initially responding to CNI therapy, a significant percentage still progressed to ESRD despite achieving short-term remission. Recurrent episodes of AKI may be associated with progression of CKD in SRNS patients with.

  18. [The Hypereosinophilic Syndromes in Childhood].

    Science.gov (United States)

    Leu, T; Simon, H-U; Hebestreit, H; Kunzmann, S

    2015-11-01

    The hypereosinophilic syndromes are rare disorders in childhood and require extensive differential diagnostic considerations. In the last years the earlier "idiopathic HES" called syndromes could be differentiated into molecular biologically, immunophenotypically and clinically more characterized heterogeneous diseases with high therapeutic and prognostic relevance. Nowadays the term HES summarizes diseases, which go hand in hand with a local or systemic hypereosinophilia (HE) connected with an organ damage. Depending on the cause of the HE one differentiates primary/neoplastic HES (HESN) from secondary/reactive HES (HESR). The latter develops reactively in connection with allergies, parasitosis, medications, neoplasia or a clonal increase of T-lymphocytes among others. With HESN the HE results from a clonal increase of eosinophilic granulocytes. While for some subgroups of the HESN (among others FIP1L1-PDGFRA fusion gene) the administration of a tyrosine kinase inhibitor is a new and effective therapy option, glucocorticoids still represent the medication of first choice for many not PDGFRA associated variants. Different immunomodulatory drugs or cytostatic agents are necessary to allow dose reduction of glucocorticoids. The promising therapy with anti-IL-5 antibodies is still not approved in infancy, could however become a treatment option in the future. Due to the present lack of knowledge about the HES in infancy the establishment of a register should be aimed for the treatment of HES in infancy.

  19. An unusual case of anti-glomerular basement membrane disease presenting with nephrotic syndrome.

    Science.gov (United States)

    Okafor, Chidi C; Balogun, Rasheed A; Bourne, David T; Alhussain, Turki O; Abdel-Rahman, E M

    2011-12-01

    Anti-glomerular basement membrane (anti-GBM) disease is a vasculitic disease characterized by acute kidney injury, oliguria, hematuria and proteinuria. Proteinuria is rarely in the nephrotic range. A case of anti-GBM disease with proteinuria of 22.5 g/day is discussed. Immunofluorescence showed strong linear IgG deposits while electron microscopy showed widespread visceral epithelial cell foot cell process effacement. No electron dense immune complex-type deposits were identified. Pathology findings were not suggestive of simultaneous presentation of anti-GBM disease and other diseases associated with nephrotic range proteinuria. Anti-GBM disease should be considered in a comprehensive differential diagnosis of severe proteinuria.

  20. ANCA-positive vasculitis induced by levamisole-adulterated cocaine and nephrotic syndrome: The kidney as an unusual target

    Science.gov (United States)

    Álvarez Díaz, Hortensia; Marińo Callejo, Ana Isabel; García Rodríguez, José Francisco; Rodríguez Pazos, Laura; Gómez Buela, Inmaculada; Bermejo Barrera, Ana María

    2013-01-01

    Patient: Male, 36 Final Diagnosis: Levamisole-induced vasculopathy Symptoms: Purpuric skin lesions Medication: Levamisole Clinical Procedure: — Specialty: Internal Medicine Objective: Unusual clinical course Background: Levamisole has been detected in seized cocaine samples and a levamisole-induced vasculopathy (LIV) has been described, mainly focused on skin. Case Report: A 36-year-old Caucasian man with history of antibodies to hepatitis C infection (negative hepatitis C virus RNA and negative HIV serology), smoking, and intravenous use of cocaine and brown heroin, presented to the hospital with purpuric skin lesions on extremities and earlobes. One month before the current presentation, a skin punch biopsy of one of these lesions was performed, showing histopathologic findings suggestive of mixed cryoglobulinemia. Laboratory testing revealed leukopenia, renal failure, and nephrotic syndrome. Antimyeloperoxidase antineutrophil cytoplasmic antibodies (MPO-ANCA) were positive. The previous skin punch biopsy was revised and demonstrated pathologic findings consistent with leukocytoclastic vasculitis. An analysis of a cocaine sample for personal use, provided by the patient, was performed using mass spectrometry-gas chromatography and levamisole was detected. Three boluses of intravenous methylprednisolone were administered, followed by oral prednisone 1 mg/Kg per day. Skin lesions and renal function improved. Conclusions: To our knowledge, this is the first report of nephrotic syndrome induced by levamisole-adulterated cocaine, proven by cocaine sample toxicology. Lack of renal biopsy is a limitation of this report. PMID:24478818

  1. Nephrotic syndrome; is rituximab the light at the end of the tunnel in the treatment of adult steroid-dependent minimal change disease and focal segmental glomerulosclerosis?

    Science.gov (United States)

    Kronbichler, Andreas; Mayer, Gert

    2014-01-01

    Implication for health policy/practice/research/medical education: Reports on patients with steroid-dependent nephrotic syndrome and underlying minimal change disease or focal segmental glomerulosclerosis have shown promising results. There is a strong need for more trials conducted in a prospective, controlled manner to clearly recommend rituximab therapy in this indication on a regular basis.

  2. A novel fibrillin-1 mutation in an egyptian marfan family: A proband showing nephrotic syndrome due to focal segmental glomerulosclerosis

    Directory of Open Access Journals (Sweden)

    Mohammad Al-Haggar

    2017-01-01

    Full Text Available Marfan syndrome (MFS, the founding member of connective tissue disorder, is an autosomal dominant disease; it is caused by a deficiency of the microfibrillar protein fibrillin-1 ( FBN1 and characterized by involvement of three main systems; skeletal, ocular, and cardiovascular. More than one thousand mutations in FBN1 gene on chromosome 15 were found to cause MFS. Nephrotic syndrome (NS had been described in very few patients with MFS being attributed to membranoproliferative glomerulonephritis secondary to infective endocarditis. Focal segmental glomerulosclerosis (FSGS had been reported in NS in conjunction with MFS without confirming the diagnosis by mutational analysis of FBN1. We hereby present an Egyptian family with MFS documented at the molecular level; it showed a male proband with NS secondary to FSGS, unfortunately, we failed to make any causal link between FBN dysfunction and FSGS. In this context, we review the spectrum of renal involvements occurring in MFS patients.

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

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    Matthew G Sampson

    Full Text Available 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

  4. 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

  5. The treatment of relapse in adults with minimal change nephrotic syndrome: Myths and facts

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    Mazin M.T. Shigidi

    2011-01-01

    Full Text Available Few controlled trials have studied the treatment of relapse in adults with minimal change disease. Repeated courses of steroids, cyclophosphamide, cyclosporine and even mycophe-nolate mofetil (MMF, all seem to play a role. The aim of this study was to review and critically analyze the literature regarding the use of immunosuppressive therapy for the treatment of relapse in adults with minimal change nephrotic syndrome (MCNS. An intensive search was done for pub-lished trials in the general medical database. Retrieved studies were further sorted according to spe-cific inclusion and exclusion criteria. Selected trials were critically analyzed and evaluated using the Oxford Centre for Evidence-based Medicine Levels of Evidence, 2009 rating. Six studies were selected and systematically reviewed. One randomized controlled trial compared the use of cyclo-phosphamide versus cyclosporine (11 adults and showed that both drugs are effective in the treatment of frequent relapses [level 1b evidence (grade B]. Three trials (total of 20 patients tested the use of cyclosporine therapy and showed that cyclosporine, though effective in the treatment of relapse, is associated with an extremely high incidence of subsequent relapses following drug with-drawal. A long-term follow-up cross-sectional study of 95 patients, with 69 relapsers, supported the use of steroids in the treatment of occasional relapses [level 2c evidence (grade C]. One case series described the benefits of MMF [level 4 evidence (grade C]. Most of the the clinical trials studied were heterogeneous, underpowered by small adult populations, open-labelled, non-randomized, with poor statistical analysis, validity and utility. We conclude that there is poor evidence that successful treatment of the first relapse of adult MCNS can be achieved with a second course of steroids or cyclosporine. Also, there is weak evidence that frequent relapses can be treated using cyclophosphamide, cyclosporine or MMF

  6. 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.

  7. Genomic and clinical profiling of a national nephrotic syndrome cohort advocates a precision medicine approach to disease management.

    Science.gov (United States)

    Bierzynska, Agnieszka; McCarthy, Hugh J; Soderquest, Katrina; Sen, Ethan S; Colby, Elizabeth; Ding, Wen Y; Nabhan, Marwa M; Kerecuk, Larissa; Hegde, Shivram; Hughes, David; Marks, Stephen; Feather, Sally; Jones, Caroline; Webb, Nicholas J A; Ognjanovic, Milos; Christian, Martin; Gilbert, Rodney D; Sinha, Manish D; Lord, Graham M; Simpson, Michael; Koziell, Ania B; Welsh, Gavin I; Saleem, Moin A

    2017-04-01

    Steroid Resistant Nephrotic Syndrome (SRNS) in children and young adults has differing etiologies with monogenic disease accounting for 2.9-30% in selected series. Using whole exome sequencing we sought to stratify a national population of children with SRNS into monogenic and non-monogenic forms, and further define those groups by detailed phenotypic analysis. Pediatric patients with SRNS were identified via a national United Kingdom Renal Registry. Whole exome sequencing was performed on 187 patients, of which 12% have a positive family history with a focus on the 53 genes currently known to be associated with nephrotic syndrome. Genetic findings were correlated with individual case disease characteristics. Disease causing variants were detected in 26.2% of patients. Most often this occurred in the three most common SRNS-associated genes: NPHS1, NPHS2, and WT1 but also in 14 other genes. The genotype did not always correlate with expected phenotype since mutations in OCRL, COL4A3, and DGKE associated with specific syndromes were detected in patients with isolated renal disease. Analysis by primary/presumed compared with secondary steroid resistance found 30.8% monogenic disease in primary compared with none in secondary SRNS permitting further mechanistic stratification. Genetic SRNS progressed faster to end stage renal failure, with no documented disease recurrence post-transplantation within this cohort. Primary steroid resistance in which no gene mutation was identified had a 47.8% risk of recurrence. In this unbiased pediatric population, whole exome sequencing allowed screening of all current candidate genes. Thus, deep phenotyping combined with whole exome sequencing is an effective tool for early identification of SRNS etiology, yielding an evidence-based algorithm for clinical management.

  8. Therapeutic Dilemmas Regarding Anticoagulation: An Experience in a Patient with Nephrotic Syndrome, Pulmonary Embolism, and Traumatic Brain Injury

    Science.gov (United States)

    Akimoto, Tetsu; Yamazaki, Tomoyuki; Kusano, Eiji; Nagata, Daisuke

    2016-01-01

    Patients with active bleeding complications who concomitantly develop overt pulmonary embolism (PE) present distinct therapeutic dilemmas, since they are perceived to be at substantial risk for the progression of the embolism in the absence of treatment and for aggravation of the hemorrhagic lesions if treated with anticoagulants. A 76-year-old patient with nephrotic syndrome, which is associated with an increased risk of thromboembolism, concurrently developed acute PE and intracranial bleeding because of traumatic brain injury. In this case, we prioritized the treatment for PE with the intravenous unfractionated heparin followed by warfarinization. Despite the transient hemorrhagic progression of the brain contusion after the institution of anticoagulation, our patient recovered favorably from the disease without any signs of neurological compromise. Several conundrums regarding anticoagulation that emerged in this case are also discussed. PMID:27840582

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

    Science.gov (United States)

    Zhou, Tian-Biao; Jiang, Zong-Pei

    2015-04-01

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

  10. 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.

  11. Spironolactone Plus Full-Dose ACE Inhibition in Patients with Idiopathic Membranous Nephropathy and Nephrotic Syndrome: Does It Really Work?

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    Giuseppe Remuzzi

    2010-01-01

    Full Text Available We have studied the effects of add-on spironolactone treatment (100 mg/day in 11 patients with idiopathic membranous nephropathy (IMN and > 3 gm proteinuria/day despite angiotensin converting enzyme (ACE inhibitor therapy titrated to a systolic/diastolic blood pressure < 120/80 mmHg. Blood pressure, 24-hour urinary protein excretion, and creatinine clearance were measured prior to, after two months of combined therapy, and after a 2-month withdrawal period of spironolactone. While systolic and diastolic blood pressure decreased significantly after spironolactone therapy, proteinuria did not improve. Serum potassium increased significantly as well, with three patients requiring resin-binding therapy. Thus, spironolactone seems to have no additional antiproteinuric effects over ACE inhibitor therapy in patients with IMN and nephrotic syndrome and carries the risk of significant hyperkalemia.

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

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    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

  13. IER3 Expression in Childhood Myelodysplastic Syndrome

    DEFF Research Database (Denmark)

    de Vries, Andrica; Zwaan, Christian M.; Danen van Ooschot, Astrid

    Background: Childhood myelodysplastic syndrome (MDS) is a rare disease accounting for less than 5% of all hematological malignancies. In about 50% of the MDS cases an abnormal karyotype is found by conventional karyotyping, of which chromosome 6 is involved in 10%. The immediate-early-response 3 ...

  14. 小儿肾病综合征合并低钠血症的护理体会%Nursing Experience of Hyponatremia in Children with Nephrotic Syndrome

    Institute of Scientific and Technical Information of China (English)

    饶萍

    2014-01-01

    目的分析原发性肾病综合征的观察和护理经验,探讨低钠血症的儿童的护理方法。方法对小儿原发性肾病综合征患者低钠血症的系统观察,积极采取干预措施。结果儿童原发性肾病综合征264例,低钠血症176例,其中轻度72例,中度64例,重度32例,极8例。经过仔细观察,积极治疗后有所改善。结论在原发性肾病综合征并发低钠血症的临床症状的儿童,及时了解,早期发现,早期诊断,早期治疗和护理,可减少严重后果的发生。%Objective To analysis, discussion of children with primary nephrotic syndrome observation. Methods The children with primary nephrotic syndrome patients comprehensive system of hyponatremia observed, active intervention measures. Results Children with primary nephrotic syndrome in 264 cases, 176 cases of hyponatremia, including 72 mild cases, 64 moderate cases and 32 severe cases, 8. After careful observation, active treatment improved. Conclusion In children with primary nephrotic syndrome complicated with hyponatremia, clinical symptoms of malaise, timely understanding of the disease, early detection, early diagnosis, early treatment and nursing, can reduce the occur ence of serious consequences.

  15. 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.

  16. A case of steroid-induced psychosis in a child having nephrotic syndrome with toxic epidermal necrolysis

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    Sae Yoon Kim

    2010-03-01

    Full Text Available Toxic epidermal necrolysis (TEN and Stevens&#8211;Johnson syndrome (SJS are rare, life-threatening mucocutaneous diseases, usually attributable to drugs and infections. Corticosteroids have been used in the management of TEN for the last 30 years. This remains controversial and is still much debated. TEN can occur despite administration of high doses of systemic corticosteroids. The psychiatric side effects of corticosteroids can include headache, insomnia, depression, and mood disorders with or without psychotic episodes. Steroid-induced psychosis is dealt with by tapering or discontinuing the steroid; antipsychotics are also sometimes used. We report a case of an 11-year-old boy who was admitted with TEN. He had also been diagnosed as having nephrotic syndrome in the past. Remission was achieved through induction therapy and by maintaining the use of steroids. After a full-dose intravenous dexamethasone for TEN, he showed psychotic symptoms. We diagnosed him as having steroid-induced psychosis. We tapered the steroid use and initiated an atypical antipsychotic medication, olazapine and intravenous immunoglobulin (IV-IG. His symptoms dramatically improved and he was discharged.

  17. [Idiopathic nephrotic syndrome with minimal glomerular lesions in children (author's transl)].

    Science.gov (United States)

    Scharer, K; Gilli, G

    1975-01-01

    Minimal glomerular changes were found in 80% of the cases of nephrotic sindromes in children. 5 groups can be separated under light microscopy. Electron microscopy always shows foot processes fusion. 80% of the children show edema, one third has transient hematuria, proteinuria is selective for small molecules, plasma B 1 C globuline levels are normal. According with the International Study of kidney disease in children, prednisone therapy gives positive results in 84% of all cases (Early responders) but many of them relapse (Frequent relapsers); the long-term prognosis with steroid is poor in the majority of cases. Cyclophosphamide therapy (2,5-3 mg/kg/die for 6 week and for a maximum of 60-80 days) gives better results and lesser relapses, but caution is needed because of an higher toxicity.

  18. 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

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

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    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

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

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    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.

  1. Nephrotic syndrome as a clinical manifestation of graft-versus-host disease (GVHD) in a marrow transplant recipient after cyclosporine withdrawal.

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    Oliveira, J S; Bahia, D; Franco, M; Balda, C; Stella, S; Kerbauy, J

    1999-01-01

    GVHD is one of the most frequent complications of BMT and recently nephrotic syndrome (NS) has been described as a manifestation of chronic GVHD. Here, we present an AA patient who developed NS 1 year after BMT when cyclosporine was stopped. Renal biopsy showed focal sclerosis associated with membranous deposits. He also had other clinical manifestations of chronic GVHD: sicca-like syndrome and colestasis. After 15 days of CsA therapy, he experienced a remarkable improvement in the NS and GVHD as a whole. We comment on immunological mechanisms that could be involved in the pathogenesis of this manifestation.

  2. DD genotype of ACE gene in boys: may it be a risk factor for minimal change nephrotic syndrome?

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    Alasehirli, Belgin; Balat, Ayşe; Büyükçelik, Mithat

    2012-01-01

    It has been shown that angiotensin-converting enzyme (ACE) gene insertion/deletion (I/D) polymorphism affects the circulating and cellular levels of ACE and may be a risk factor in several renal diseases. We analyzed the association of ACE gene I/D polymorphism with the clinical presentation of minimal change nephrotic syndrome (MCNS) in a Turkish child population. This study consisted of 97 children with MCNS and 144 healthy controls. Genotyping of ACE gene was performed using polymerase chain reaction (PCR). The distributions of ACE genotypes were II in 13%, ID in 49%, and DD in 38% in patient group, and 9%, 49%, and 42% in control group, respectively. The frequency of the D allele was 63% and that of the I allele was 37% in patients. There were no relevant differences in the allele frequencies and genotypes of ACE I/D polymorphism between patients and controls. However, DD genotype was higher in boys in children with MCNS (78.4%. vs. 50.0%, p = 0.004). The frequencies of DD genotype and D allele in boys were 7.25 and 2.56 times higher than II genotype and I allele in the patient group, respectively. We suggest that DD genotype in boys may be one of the risk factors for MCNS.

  3. Impact of the 4G/5G polymorphism in the plasminogen activator inhibitor-1 gene on primary nephrotic syndrome.

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    Luo, Yuezhong; Wang, Chao; Tu, Haitao

    2014-03-01

    The aim of the present study was to investigate whether the four guanosines (4G)/five guanosines (5G) polymorphism in the gene coding for plasminogen activator inhibitor-1 (PAI-1) affects the clinical features of primary nephrotic syndrome (PNS). A cohort of 200 biopsy-diagnosed PNS patients was studied, with 40 healthy subjects as controls. The PAI-1 gene polymorphism was detected by polymerase chain reaction and DNA sequencing. Associations between the PAI-1 4G/5G polymorphism and clinical features and pathological types of PNS were analyzed. The results indicated that the PAI-1 genotype distribution is significantly different between patients with PNS and healthy controls, with significantly higher numbers of the 4G/4G genotype and lower numbers of the 5G5G genotype detected in PNS patients compared to controls (both P5G genotypes, as well as of the 4G allele. The increased 4G frequency was also detected in patients with minimal change disease (MCD). Significantly increased international normalized ratio (INR) and prolonged activated partial thromboplastin time (APTT) were observed in 4G/4G compared to 5G/5G PNS subjects. The response to steroids was not significantly different among the three genotypes. In conclusion, the 4G allele of the PAI-1 gene appears to be associated with PNS, especially in MN and IgAN patients. These findings suggest that specific targeting may be required for the treatment of PNS patients with the 4G/4G genotype.

  4. Sonographic Diagnosis in a Rare Aetiology of Neonatal Scrotal Swellings: A Case Report of Congenital Nephrotic Syndrome

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    Grover, Shabnam Bhandari; Kumar, Nishith; Grover, Hemal; Taneja, Dinesh Kumar; Katyan, Amit

    2016-01-01

    Summary Background Common etiologies of scrotal swelling in neonates include hydrocoele, inguinal hernia and testicular torsion; less common is epididymo-orchitis. Congenital nephrotic syndrome (CNS), a rare entity, is known to present as progressive renal failure and its leading presentation with scrotal involvement has not been reported. Material/Methods We report a rare case of CNS with primary clinical presentation as scrotal cellulitis and epididymo-orchitis. In this neonate, scrotal and abdominal ultrasound examination was performed and the laboratory data were obtained. Results Sonography revealed bilaterally enlarged echogenic kidneys, testis and epididymis with echogenic peritoneal fluid tracking into both scrotal sacs. Laboratory data revealed proteinuria and severe depletion of serum IgG. Culture of the peritoneal fluid showed gram-negative organisms. A final diagnosis of CNS, complicated with peritonitis tracking into the scrotal sacs was arrived at. Conclusions CNS may have a rare presentation with distracting symptoms of scrotal cellulitis and epididymo-orchitis, as seen in our patient. However, diligent use of abdomino-scrotal sonography, supported by relevant laboratory data can clinch the accurate diagnosis. PMID:27757175

  5. Benefits of once-daily administration of cyclosporine a for children with steroid-dependent, relapsing nephrotic syndrome.

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    Suzuki, Koichi; Oki, Eishin; Tsuruga, Kazushi; Aizawa-Yashiro, Tomomi; Ito, Etsuro; Tanaka, Hiroshi

    2010-03-01

    Cyclosporine A (CsA) is an effective steroid-sparing agent for patients with steroid-dependent, relapsing nephrotic syndrome (SDRNS). The efficacy and safety of single-daily dose administration (SDD protocol) of CsA in selected patients with SDRNS has been reported. However, the efficacy of initial CsA treatment for children with SDRNS using the SDD protocol remains to be elucidated. The SDD protocol might be associated with lower clinical toxicity, compared to the conventional twice-daily dose administration (TDD protocol). Here we evaluated the efficacy and safety of the SDD protocol versus the TDD protocol in patients with SDRNS. The data from 19 patients (9.9 +/- 4.2 years old) were retrospectively collected and analyzed. Ten patients treated according to the SDD protocol for a mean of 27 months (SDD group), while 9 patients treated with the TDD protocol for a mean of 35 months (TDD group) as an initial CsA treatment. Although the mean daily CsA dose was significantly lower in the SDD group (1.5 +/- 0.4 mg/kg/day vs. 3.7 +/- 0.7 mg/kg/day, P SDD group showed nephrotoxicity. Despite a small number of patients, this study may support that the SDD protocol is at least as effective as the conventional TDD protocol, and is more cost-effective for selected children with SDRNS.

  6. [Ultrastructure of glomerular podocyts in the incipient phase of minimal change nephrotic syndrome with thin basement membrane disease].

    Science.gov (United States)

    Ogawa, Ryo; Miyoshi, Ken-ichi; Nagao, Tomoaki; Jotoku, Masanori; Irita, Jun; Okura, Takafumi; Higaki, Jitsuo

    2012-01-01

    An 80-year-old woman was referred to the Division of Nephrology at Ehime University Hospital because of leg edema in December 2010. She had been treated with 300 mg of tocopherol for scleroderma since 2007 and treated with 9 mg of prednisolone (PSL) for autoimmune hearing loss since 2010. Due to the occurrence of mild hematuria (5-9/HPF), proteinuria (0.9 g/day) and an increased serum creatinine level (1.31 mg/dL), a renal biopsy was performed. Light microscopy (LM) showed minor abnormality in the glomeruli, and immunohistology showed the absence of deposits of immunoglobulins and complements. Electron microscopy (EM) showed a thin glomerular basement membrane with a limited level of podocyte abnormalities. Due to the findings of intimal thickening of interlobular arteries and subcapsular accumulation of global sclerosis on LM, she was diagnosed with nephrosclerosis and thin basement membrane disease. Four weeks later, her leg edema had increased considerably and urinary protein had increased to 12.4 g/day. The second biopsy showed similar findings in LM and IF as the first biopsy, but EM revealed diffuse foot process effacement. She was diagnosed with minimal change nephrotic syndrome (MCNS) and treated with methylprednisolone pulse therapy followed by 40 mg of oral PSL. Her urinary protein had completely disappeared 6 weeks later. Complete remission with PSL treatment indicates that urinary protein at first renal biopsy was due to MCNS. Our case exhibited podocyte features in the incipient phase of human MCNS.

  7. Significance of technetium-99m human serum albumin diethylenetriamine pentaacetic acid scintigraphy in patients with nephrotic syndrome.

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    Tsuyoshi Takashima

    Full Text Available It is thought that a large amount of albumin leaking from the glomerulus in nephrotic syndrome (NS is reabsorbed at the proximal tubule and catabolized. Therefore, it is possible the final quantity of urinary protein does not always reflect the amount of leakage of protein from the glomerulus. We experienced two cases without nephrotic range proteinuria thought to involve hypoproteinemia due to the same pathophysiology as NS. On these patients, we performed protein leakage scintigraphy with technetium-99m human serum albumin diethylenetriamine pentaacetic acid (99mTc-HSAD to exclude a diagnosis of protein-losing gastroenteropathy and observed diffuse positive accumulation in the kidneys with more intense uptake in the kidney than the liver on the anterior view 24 hours after 99mTc-HSAD administration. In healthy adults intravenously given 99mTc-HSAD, the same dynamics are observed as in albumin metabolism, and the organ radioactivity of the liver and kidneys after 24 hours is equal. Therefore, we thought it was possible that the renal uptake 24 hours after 99mTc-HSAD administration was a characteristic finding of NS. In order to confirm it, the subjects were divided into two groups: the NS group (n = 10 and the non-NS group (n = 7. We defined more intense uptake in the kidney than the liver on the anterior view 24 hours after 99mTc-HSAD administration as Dense Kidney (+. Furthermore, we designed regions of interest in the right and left kidneys and liver on anterior and posterior images, then calculated the kidney-liver ratio. Nine of the ten patients had Dense Kidney (+ in the NS group, compared to none in the non-NS group. And the kidney-liver ratio was significantly higher in the NS group than in the non-NS group on each view in the bilateral kidneys. In conclusion, our results suggest that the renal uptake 24 hours after 99mTc-HSAD administration is a characteristic finding of NS.

  8. Epithelial sodium channel (ENaC) subunit mRNA and protein expression in rats with puromycin aminonucleoside-induced nephrotic syndrome.

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    Audigé, A; Yu, Z R; Frey, B M; Uehlinger, D E; Frey, F J; Vogt, B

    2003-04-01

    In experimental nephrotic syndrome, urinary sodium excretion is decreased during the early phase of the disease. The molecular mechanism(s) leading to salt retention has not been completely elucidated. The rate-limiting constituent of collecting duct sodium transport is the epithelial sodium channel (ENaC). We examined the abundance of ENaC subunit mRNAs and proteins in puromycin aminonucleoside (PAN)-induced nephrotic syndrome. The time courses of urinary sodium excretion, plasma aldosterone concentration and proteinuria were studied in male Sprague-Dawley rats treated with a single dose of either PAN or vehicle. The relative amounts of alphaENaC, betaENaC and gammaENaC mRNAs were determined in kidneys from these rats by real-time quantitative TaqMan PCR, and the amounts of proteins by Western blot. The kinetics of urinary sodium excretion and the appearance of proteinuria were comparable with those reported previously. Sodium retention occurred on days 2, 3 and 6 after PAN injection. A significant up-regulation of alphaENaC and betaENaC mRNA abundance on days 1 and 2 preceded sodium retention on days 2 and 3. Conversely, down-regulation of alphaENaC, betaENaC and gammaENaC mRNA expression on day 3 occurred in the presence of high aldosterone concentrations, and was followed by a return of sodium excretion to control values. The amounts of alphaENaC, betaENaC and gammaENaC proteins were not increased during PAN-induced sodium retention. In conclusion, ENaC mRNA expression, especially alphaENaC, is increased in the very early phase of the experimental model of PAN-induced nephrotic syndrome in rats, but appears to escape from the regulation by aldosterone after day 3.

  9. Proteomics Approach Identifies Factors Associated With the Response to Low-Density Lipoprotein Apheresis Therapy in Patients With Steroid-Resistant Nephrotic Syndrome.

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    Kuribayashi-Okuma, Emiko; Shibata, Shigeru; Arai, Shigeyuki; Ota, Tatsuru; Watanabe, Sumiyo; Hisaki, Harumi; Okazaki, Tomoki; Toda, Tosifusa; Uchida, Shunya

    2016-04-01

    Low-density lipoprotein apheresis (LDL-A) has been shown to reduce proteinuria in a subgroup of nephrotic syndrome patients refractory to immunosuppressive therapy. Factors influencing the efficacy of LDL-A in nephrotic syndrome are completely unknown. Using a proteomics approach, we aimed to identify biological markers that predict the response to LDL-A in patients with steroid-resistant nephrotic syndrome (SRNS). Identification of plasma proteins bound to the dextran-sulfate column at the first session of LDL-A was determined by mass spectrometry. To investigate biological factors associated with the response to LDL-A, we compared profiles of column-bound proteins between responders (defined by more than 50% reduction of proteinuria after the treatment) and non-responders by 2-dimensional gel electrophoresis (2-DE) coupled to mass spectrometry in seven patients with SRNS. Evaluation of proteins adsorbed to LDL-A column in patients with SRNS revealed the identity of 62 proteins, which included apolipoproteins, complement components, and serum amyloid P-component (SAP). Comparative analysis of the column-bound proteins between responders and non-responders by 2-DE demonstrated that apolipoprotein E (APOE) and SAP levels were increased in non-responders as compared with responders. These results were confirmed by western blotting. Moreover, serum levels of APOE and SAP were significantly higher in the non-responder group than in the responder group by ELISA. Our data provide comprehensive analysis of proteins adsorbed by LDL-A in SRNS, and demonstrate that the serum levels of APOE and SAP may be used to predict the response to LDL-A in these patients.

  10. Levamisole in steroid-sensitive nephrotic syndrome: usefulness in adult patients and laboratory insights into mechanisms of action via direct action on the kidney podocyte.

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    Jiang, Lulu; Dasgupta, Ishita; Hurcombe, Jenny A; Colyer, Heather F; Mathieson, Peter W; Welsh, Gavin I

    2015-06-01

    Minimal change nephropathy (MCN) is the third most common cause of primary nephrotic syndrome in adults. Most patients with MCN respond to corticosteroid therapy, but relapse is common. In children, steroid-dependent patients are often given alternative agents to spare the use of steroids and to avoid the cumulative steroid toxicity. In this respect, levamisole has shown promise due to its ability to effectively maintain remission in children with steroid-sensitive or steroid-dependent nephrotic syndrome. Despite clinical effectiveness, there is a complete lack of molecular evidence to explain its mode of action and there are no published reports on the use of this compound in adult patients. We studied the effectiveness of levamisole in a small cohort of adult patients and also tested the hypothesis that levamisole's mode of action is attributable to its direct effects on podocytes. In the clinic, we demonstrate that in our adult patients, cohort levamisole is generally well tolerated and clinically useful. Using conditionally immortalized human podocytes, we show that levamisole is able to induce expression of glucocorticoid receptor (GR) and to activate GR signalling. Furthermore, levamisole is able to protect against podocyte injury in a puromycin aminonucleoside (PAN)-treated cell model. In this model the effects of levamisole are blocked by the GR antagonist mifepristone (RU486), suggesting that GR signalling is a critical target of levamisole's action. These results indicate that levamisole is effective in nephrotic syndrome in adults, as well as in children, and point to molecular mechanisms for this drug's actions in podocyte diseases.

  11. A case of Plummer-Vinson syndrome in childhood.

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    Anthony, R; Sood, S; Strachan, D R; Fenwick, J D

    1999-10-01

    The Plummer-Vinson syndrome is characterized by an association of dysphagia, iron-deficiency anemia, and esophageal webs. The authors report the case of a 6 year old with Plummer-Vinson syndrome. Plummer-Vinson syndrome usually occurs in adults, rarely in adolescents, however, there have been no previous reports in the English-language literature of the syndrome occurring in childhood.

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

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    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

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

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    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.

  14. Cytoplasmic Localization of WT1 and Decrease of miRNA-16-1 in Nephrotic Syndrome

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    Rangel-Ochoa, Gloria Azucena; Rodríguez-Padilla, Cristina

    2017-01-01

    Nephrotic syndrome (NS) is a glomerular disease that is defined by the leakage of protein into the urine and is associated with hypoalbuminemia, hyperlipidemia, and edema. Steroid-resistant NS (SRNS) patients do not respond to treatment with corticosteroids and show decreased Wilms tumor 1 (WT1) expression in podocytes. Downregulation of WT1 has been shown to be affected by certain microRNAs (miRNAs). Twenty-one patients with idiopathic NS (68.75% were SSNS and 31.25% SRNS) and 10 healthy controls were enrolled in the study. Podocyte number and WT1 location were determined by immunofluorescence, and the serum levels of miR-15a, miR-16-1, and miR-193a were quantified by RT-qPCR. Low expression and delocalization of WT1 protein from the nucleus to the cytoplasm were found in kidney biopsies of patients with SRNS and both nuclear and cytoplasmic localization were found in steroid-sensitive NS (SSNS) patients. In sera from NS patients, low expression levels of miR-15a and miR-16-1 were found compared with healthy controls, but only the miR-16-1 expression levels showed statistically significant decrease (p = 0.019). The miR-193a expression levels only slightly increased in NS patients. We concluded that low expression and delocalization from the WT1 protein in NS patients contribute to loss of podocytes while modulation from WT1 protein is not associated with the miRNAs analyzed in sera from the patients. PMID:28299339

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

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    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.

  16. Chronic Fatigue Syndrome (CFS): Childhood Adversity

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    ... reports of childhood abuse and neglect were investigated. Childhood trauma, defined as abuse, neglect, or loss, is a ... the lifespan. In various animal and human studies childhood trauma has been associated with low resting cortisol levels, ...

  17. Neurocognitive impairment in childhood chronic fatigue syndrome

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    Kei eMizuno

    2013-04-01

    Full Text Available Neurocognitive impairment is a feature of childhood chronic fatigue syndrome (CCFS. Several studies have demonstrated reduced attention control in CCFS patients in switching and divided attention tasks. In students, the extent of deterioration in task performance depends on the level of fatigue. Poor performance in switching and divided attention is common in both fatigued students and CCFS patients. Additionally, attentional functions show dramatic development from childhood to adolescence, suggesting that abnormal development of switching and divided attention may be induced by chronic fatigue. The brain structures associated with attentional control are situated in the frontal and parietal cortices, which are the last to mature, suggesting that severe fatigue in CCFS patients and students may inhibit normal structural and functional development in these regions. A combination of treatment with cognitive behavioral therapy and antidepressant medication is effective to improve attentional control processing in CCFS patients. Studies identifying the features of neurocognitive impairment in CCFS have improved our current understanding of the neurophysiological mechanisms of CCFS.

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

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    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.

  19. A novel approach to investigation of the pathogenesis of active minimal-change nephrotic syndrome using subtracted cDNA library screening.

    Science.gov (United States)

    Sahali, Djillali; Pawlak, André; Valanciuté, Asta; Grimbert, Philippe; Lang, Philippe; Remy, Philippe; Bensman, Albert; Guellaën, Georges

    2002-05-01

    Clinical and experimental observations suggest that minimal-change nephrotic syndrome (MCNS) results from T cell dysfunction, via unknown mechanisms. For the identification of genes that are potentially involved in MCNS, a subtractive cDNA library was constructed from cDNA from T cell-enriched peripheral blood mononuclear cells obtained from the same patient during relapse versus remission ("relapse minus remission"). This library was screened by differential hybridization with forward ("relapse minus remission") and reverse ("remission minus relapse") subtractive cDNAs probes, as well as unsubtracted probes from relapse and remission, and irrelevant nephrotic syndrome (membranous nephropathy). A total of 84 transcripts were isolated, of which 12 matched proteins of unknown function and 30 were unknown clones. Among the 42 known transcripts, at least 18 are closely involved in the T cell receptor-mediated complex signaling cascade, including genes encoding components of the T cell receptor and proteins associated with the cytoskeletal scaffold, as well as transcription factors. In particular, it was demonstrated that the expression levels of Fyb/Slap, L-plastin, and grancalcin were increased during relapse, suggesting that the integration of proximal signaling after T cell engagement involves the preferential recruitment of these cytoskeleton-associated proteins in MCNS. Because very low levels of interleukin-12 receptor beta2 mRNA were detected in relapse samples, the interleukin-12 signaling pathway might be defective, suggesting that, in MCNS, T cell activation evolves toward a T helper 2 phenotype. Therefore, the combination of subtractive cloning and differential screening constitutes an efficient approach to the identification of genes that are likely to be involved in the pathophysiologic processes of MCNS.

  20. Papulonodular mucinosis, Guillain-Barré syndrome and nephrotic syndrome in a patient with systemic lupus erythematosus: a case report

    OpenAIRE

    Su, Xiaole; Qiao, Xi; Li, Jing; Gao, Lifang; Wang, Chen; WANG Lihua

    2017-01-01

    Background Awareness of the spectrum of clinical manifestations of systemic lupus erythematosus (SLE), especially uncommon changes, is essential for diagnosis and effective management of patients. Case presentation A 26-year-old Chinese man with SLE initially manifested cutaneous papulonodular mucinosis and developed acute Guillain-Barré syndrome and class V lupus nephritis 2 years later. His cutaneous nodules had not been idententified for 2 years and were resected by surgical procedures twi...

  1. Congenital nephrotic syndrome

    Science.gov (United States)

    ... by: Charles Silberberg, DO, private practice specializing in nephrology, affiliated with New York Medical College, Division of Nephrology, Valhalla, NY. Review provided by VeriMed Healthcare Network. ...

  2. Extending initial prednisolone treatment in a randomized control trial from 3 to 6 months did not significantly influence the course of illness in children with steroid-sensitive nephrotic syndrome.

    Science.gov (United States)

    Sinha, Aditi; Saha, Abhijeet; Kumar, Manish; Sharma, Sonia; Afzal, Kamran; Mehta, Amarjeet; Kalaivani, Mani; Hari, Pankaj; Bagga, Arvind

    2015-01-01

    While studies show that prolonged initial prednisone therapy reduces the frequency of relapses in nephrotic syndrome, they lack power and have risk of bias. In order to examine the effect of prolonged therapy on frequency of relapses, we conducted a blinded, 1:1 randomized, placebo-controlled trial in 5 academic hospitals in India on 181 patients, 1-12 years old, with a first episode of steroid-sensitive nephrotic syndrome. Following 12 weeks of standard therapy, in random order, 92 patients received tapering prednisolone while 89 received matching-placebo on alternate days for the next 12 weeks. On intention-to-treat analyses, primary outcome of number of relapses at 1 year was 1.26 in the 6-month group and 1.54 in the 3-month group (difference -0.28; 95% confidence interval (CI) -0.75, 0.19). Relative relapse rate for 6- vs. 3-month therapy, adjusted for gender, age, and time to initial remission, was 0.70 (95% CI 0.47-1.10). Similar proportions of patients had sustained remission, frequent relapses, and adverse effects due to steroids. Adjusted hazard ratios for first relapse and frequent relapses with prolonged therapy were 0.57 (95% CI, 0.36-1.07) and 1.01 (95% CI, 0.61-1.67), respectively. Thus, extending initial prednisolone treatment from 3 to 6 months does not influence the course of illness in children with nephrotic syndrome. These findings have implications for guiding the duration of therapy of nephrotic syndrome.

  3. Childhood acne in a boy with XYY syndrome.

    Science.gov (United States)

    Kasparis, Christos; Loffeld, Annette

    2014-01-06

    A 3-year-old boy was referred to the dermatology department with a 12-month history of facial erythema associated with a papular-pustular facial eruption consistent with childhood acne. He had been diagnosed with XYY syndrome identified during genetic analysis for cardiac anomalies at birth. XYY syndrome is an aneuploidy of the sex chromosomes which affects 1 in 1000 male births. It is often asymptomatic and identified incidentally following genetic analysis for other conditions. The syndrome can be associated with an increased risk of learning difficulties and delayed language skills. Early diagnosis could alert physicians to the possibility of subtle developmental and learning abnormalities and result in prompt management. Our case highlights the fact that the presence of childhood acne could aid in the early detection of XYY syndrome.

  4. Childhood acne in a boy with XYY syndrome

    Science.gov (United States)

    Kasparis, Christos; Loffeld, Annette

    2014-01-01

    A 3-year-old boy was referred to the dermatology department with a 12-month history of facial erythema associated with a papular-pustular facial eruption consistent with childhood acne. He had been diagnosed with XYY syndrome identified during genetic analysis for cardiac anomalies at birth. XYY syndrome is an aneuploidy of the sex chromosomes which affects 1 in 1000 male births. It is often asymptomatic and identified incidentally following genetic analysis for other conditions. The syndrome can be associated with an increased risk of learning difficulties and delayed language skills. Early diagnosis could alert physicians to the possibility of subtle developmental and learning abnormalities and result in prompt management. Our case highlights the fact that the presence of childhood acne could aid in the early detection of XYY syndrome. PMID:24395875

  5. 老年肾病综合征101例临床和病理分析%Clinical and pathological analysis of 101 elderly patients with nephrotic syndrome

    Institute of Scientific and Technical Information of China (English)

    陈伟军; 陆轶君; 张晓丽; 叶志斌

    2015-01-01

    目的 了解老年肾病综合征的临床与病理分布特点. 方法 回顾性分析我院101例老年肾病综合征的临床资料,包括临床表现、病理类型、治疗效果、并发症和预后等. 结果 101例老年肾病综合征患者中,原发性和继发性肾病综合征分别为79例和22例.79例原发性肾病综合征患者中,44例有肾脏病理资料,前4位分别是膜性肾病、IgA肾病、膜增生性肾小球肾炎和局灶节段性肾小球硬化症.22例继发性肾病综合征中,糖尿病肾病17例,其次是狼疮性肾炎和显微镜下血管炎.联合应用中等剂量激素和免疫抑制剂对病理诊断明确的原发性肾病综合征患者的治疗总有效率为84.1%.原发性和继发性老年肾病综合征的主要并发症均为感染、急性肾损伤和心血管疾病. 结论 老年肾病综合征中原发性所占比例较高,原发性肾病综合征肾小球疾病病理类型以膜性肾病、IgA肾病、膜增生性肾小球肾炎较为多见,继发性肾小球疾病中以糖尿病肾病最多见.对于病理诊断明确的患者,联合应用中等剂量激素和免疫抑制剂有一定的治疗作用.%Objective To study the clinical and pathological features of nephrotic syndrome in the elderly.Methods The clinical data of 101 elderly patients with nephrotic syndrome were retrospectively analyzed,including clinical manifestations,pathological type,therapeutic effect,complications and prognosis.Results Among the 101 cases of elderly patients with nephrotic syndrome,79 cases were primary nephrotic syndrome and 22 cases were secondary nephrotic syndrome.Of the 79 cases with primary syndrome,44 cases had renal pathological data.The most common types were membranous nephropathy,IgA nephropathy,mesangial proliferative glomerulonephritis and focal segmental glomerular sclerosis.The most common types of renal pathology of the 22 cases with secondary nephrotic syndrome were diabetic nephropathy (17 cases),followed by

  6. 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...

  7. 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

  8. Genetic syndromes associated with overgrowth in childhood.

    Science.gov (United States)

    Ko, Jung Min

    2013-09-01

    Overgrowth syndromes comprise a diverse group of conditions with unique clinical, behavioral and molecular genetic features. While considerable overlap in presentation sometimes exists, advances in identification of the precise etiology of specific overgrowth disorders continue to improve clinicians' ability to make an accurate diagnosis. Among them, this paper introduces two classic genetic overgrowth syndromes: Sotos syndrome and Beckwith-Wiedemann syndrome. Historically, the diagnosis was based entirely on clinical findings. However, it is now understood that Sotos syndrome is caused by a variety of molecular genetic alterations resulting in haploinsufficiency of the NSD1 gene at chromosome 5q35 and that Beckwith-Wiedemann syndrome is caused by heterogeneous abnormalities in the imprinting of a number of growth regulatory genes within chromosome 11p15 in the majority of cases. Interestingly, the 11p15 imprinting region is also associated with Russell-Silver syndrome which is a typical growth retardation syndrome. Opposite epigenetic alterations in 11p15 result in opposite clinical features shown in Beckwith-Wiedemann syndrome and Russell-Silver syndrome. Although the exact functions of the causing genes have not yet been completely understood, these overgrowth syndromes can be good models to clarify the complex basis of human growth and help to develop better-directed therapies in the future.

  9. 老年肾病综合征的临床特点及病理类型分析%Nephrotic syndrome in the elderly:clinical and pathological analyses

    Institute of Scientific and Technical Information of China (English)

    刘小聪; 邵思南; 张燕林

    2015-01-01

    目的:探讨老年肾病综合征的临床表现和病理特点。方法收集60岁以上老年肾病综合征患者41例,对其临床表现及肾活检病理进行分析。结果41例患者中,原发性肾小球疾病28例(68.29%),病理类型以膜性肾病最常见,其次为局灶节段性肾小球硬化、微小病变性肾病;继发性肾病综合征13例(31.71%)。除肾病综合征表现外,伴血尿25例(60.98%),高血压24例(58.54%),肾功能异常20例(48.78%)。病程中出现感染11例(26.83%)。肾活检后无严重并发症。结论老年肾病综合征最常见的病因为原发性肾小球疾病,病理类型以膜性肾病最常见。主要临床表现为浮肿、血尿、高血压、肾功能减退,并发症主要为感染。行肾活检对老年患者是安全的。%Objective To analyze the clinical manifestations and pathologic characters of nephrotic syndrome in elderly patients.Methods We collected 41 nephrotic syndrome patients who were more than 60 years old in our hospital from January 2009 to January 2014 and analyzed their clinical manifestations and pathologic characters.Re-sults All of the 41 cases with nephrotic syndrome were diagnosed by renal biopsy, with 28 cases in primary glomeru-lar disease(68.29%), in which membranous nephropathy was the most common pathological type, followed by focal segmental glomerular sclerosis and minimal change disease, and with 13 cases in secondary nephrotic syndrome (31.71%).Except for nephrotic syndrome, hematuria was found in 25 cases(60.98%), hypertension in 24 cases (58.54%), renal dysfunction in 20 cases(48.78%).Infection was the major complication(26.83%).No serious complications were found after the renal biopsy.Conclusion The most common disease in the elderly patients with nephrotic syndrome is primary glomerular disease, in which membranous nephropathy is the most common pathological type.Major clinical manifestations are edema

  10. Obesity and Metabolic Syndrome Among Adult Survivors of Childhood Leukemia.

    Science.gov (United States)

    Gibson, Todd M; Ehrhardt, Matthew J; Ness, Kirsten K

    2016-04-01

    Treatment-related obesity and the metabolic syndrome in adult survivors of childhood acute lymphoblastic leukemia (ALL) are risk factors for cardiovascular disease. Both conditions often begin during therapy. Preventive measures, including dietary counseling and tailored exercise, should be initiated early in the course of survivorship, with referral to specialists to optimize success. However, among adults who develop obesity or the metabolic syndrome and who do not respond to lifestyle therapy, medical intervention may be indicated to manage underlying pathology, such as growth hormone deficiency, or to mitigate risk factors of cardiovascular disease. Because no specific clinical trials have been done in this population to treat metabolic syndrome or its components, clinicians who follow adult survivors of childhood ALL should use the existing American Heart Association/National Heart Lung and Blood Institute Scientific Statement to guide their approach.

  11. 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

  12. 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.

  13. Nephrotic syndrome with fever and widespread subcutaneous necrosis%肾病综合征伴高热、皮下广泛坏死

    Institute of Scientific and Technical Information of China (English)

    全军肾脏病研究所学术委员会

    2012-01-01

    A 23-year-old man developed severe necrotizing cellulitis on both anterior and posterior of his trunk after kneading. The patient had been treated with corticosteroids over twenty months for nephrotic syndrome. Skin biopsy of the wound area revealed cutaneous vasculitis and chronic inflammation with yeast-like organisms. The Periodic Acid Schiff staining supported that the structures were consistent with Cryptococcosis. A Cryptococcosus neoformans infection was confirmed by culture.%青年男性,临床表现肾病综合征、血清肌酐轻度升高,肾活检确诊为IgA肾病,肾小管损伤严重;长期接受大剂量糖皮质激素联合免疫抑制剂治疗.近期泡澡、搓澡后出现大面积皮肤红肿热痛,高热;血清隐球菌抗原阳性,皮肤病理检查符合隐球菌感染,皮肤破损脓液及皮肤坏死组织培养确诊新生隐球菌感染.

  14. Two epileptic syndromes, one brain: childhood absence epilepsy and benign childhood epilepsy with centrotemporal spikes.

    Science.gov (United States)

    Cerminara, Caterina; Coniglio, Antonella; El-Malhany, Nadia; Casarelli, Livia; Curatolo, Paolo

    2012-01-01

    Childhood absence epilepsy (CAE) and benign childhood epilepsy with centrotemporal spikes (BCECTS), or benign rolandic epilepsy (BRE), are the most common forms of childhood epilepsy. CAE and BCECTS are well-known and clearly defined syndromes; although they are strongly dissimilar in terms of their pathophysiology, these functional epileptic disturbances share many features such as similar age at onset, overall good prognosis, and inheritance factors. Few reports are available on the concomitance of CAE and BCECTS in the same patients or the later occurrence of generalized epilepsy in patients with a history of partial epilepsy. In most cases described in the literature, absence seizures always started after the onset of benign focal epilepsy but the contrary has never occurred yet. We describe two patients affected by idiopathic generalized epileptic syndrome with typical absences, who experienced BCECTS after remission of seizures and normalization of EEG recordings. While the coexistence of different seizure types within an epileptic syndrome is not uncommon, the occurrence of childhood absence and BCECTS in the same child appears to be extremely rare, and this extraordinary event supports the hypothesis that CAE and BCECTS are two distinct epileptic conditions. However, recent interesting observations in animal models suggest that BCECTS and CAE could be pathophysiologically related and that genetic links could play a large role.

  15. 多靶点治疗激素抵抗型肾病综合征的临床研究%Clinical trial on the effect of multi-target therapy on steroid-resistant nephrotic syndrome

    Institute of Scientific and Technical Information of China (English)

    王健英; 赵军; 王臻

    2009-01-01

    Objective To investigate the clinical effect of cyclosporine(CsA) combined with leflunomide and steroid in the treatment of steroid-resistant nephrotic syndrome. Methods The 14 patients with steroid-resistant nephrotic syndrome treated with leflunomide ,CsA and prednisone.Clinical data were observed in the 2nd,4th,6th,8th,and 12th week. Results After receiving multi-target therapy ,the proteinuria was decreased significantly(P<0.01),Serum albumin was increased significantly(P< 0.01), Complete relieving rate was 64.3% ,and total effective rate was 86.7%.Conclusion Multi-target therapy with CsA,leflunomide and steroid was an effective induction therapy with mild adverse effects for steroid-resistant nephrotic syndrome.%目的 研究多种免疫抑制剂联合治疗激素抵抗型肾病综合征的临床疗效.方法 14例患者均接受小剂量环孢素A(CsA)和来氟米特联合中等剂量的强的松治疗,在治疗后第2、4、6、8、12周动态监测各项临床指标.结果 24 h尿蛋白定量较治疗前明显下降,血浆白蛋白在治疗后明显升高,完全缓解率为64.3%,总有效率为86.7%.结论 多靶点治疗激素抵抗型肾病综合征有显著疗效,并且不良反映少,安全性较高.

  16. 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.

  17. 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.

  18. 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。

  19. The Usefulness of Determining Neutrophil Gelatinase-Associated Lipocalin Concentration Excreted in the Urine in the Evaluation of Cyclosporine A Nephrotoxicity in Children with Nephrotic Syndrome

    Directory of Open Access Journals (Sweden)

    Ewa Gacka

    2016-01-01

    Full Text Available Introduction. The use of cyclosporine (CsA in the treatment of nephrotic syndrome (NS contributed to a significant reduction in the amount of corticosteroids used in therapy and its cumulative side effects. One of the major drawbacks of CsA therapy is its nephrotoxicity. Prolonged CsA treatment protocols require sensitive, easily available, and simple to measure biomarkers of nephrotoxicity. NGAL is an antibacterial peptide, excreted by cells of renal tubules in response to their toxic or inflammatory damage. Aim of the Study. The aim of this study was to assess the suitability of the NGAL concentration in the urine as a potential biomarker of the CsA nephrotoxicity. Material and Methods. The study was performed on a group of 31 children with NS treated with CsA. The control group consisted of 23 children diagnosed with monosyptomatic enuresis. The relationship between NGAL excreted in urine and the time of CsA treatment, concentration of CsA in blood serum, and other biochemical parameters was assessed. Results. The study showed a statistically significant positive correlation between urine NGAL concentration and serum triglycerides concentration and no correlation between C0 CsA concentration and other observed parameters of NS. The duration of treatment had a statistically significant influence on the NGAL to creatinine ratio. Conclusions. NGAL cannot be used alone as a simple CsA nephrotoxicity marker during NS therapy. Statistically significant correlation between NGAL urine concentration and the time of CsA therapy indicates potential benefits of using this biomarker in the monitoring of nephrotoxicity in case of prolonged CsA therapy.

  20. 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.

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

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    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.

  2. 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.

  3. 汞中毒致大鼠肾病综合征模型的建立%The model of nephrotic syndrome induced by mercury intoxication in rats

    Institute of Scientific and Technical Information of China (English)

    陈子安; 隋昕; 聂志勇; 李万华; 邱泽武; 王永安

    2015-01-01

    Objective The present study aims to establish a model of nephrotic syndrome induced by mercury intoxication, in order to provide stable and scientific platform for clinical relevant treatment.Methods Twenty-four male BN rats were randomly divided into four groups, including three model groups( n=18) and a control group( n=6) .The rats in the three model groups were given respectively mercuric chloride solution by subcutaneous injection of 0.5, 1.0 and 2.0 mg/kg and the control group was given a subcutaneous injection of saline, all the rats were given once every other day. By monitored the urine protein, blood biochemical indicators, mercury pontent, pathological section after injected for 7, 14, 21, 28, 35 days, we identified 1.0 mg/kg was the right dose to establish the model. Another 24 rats were randomly divided into 3 groups, according to the above method the including 18 rats were all injected in 1 mg/kg mercury chloride, the other 6 rats were for the control group.On day 15, 22 and 36 the rats were sacrificed and rat kidneys were sampled to monitor the mercury content in order to observe the accumulation of mercury in the kidney.Results Fourteen days after mercury injection, high proteinuria, low albumin, hyperlipidemia and edema occurred in three model groups, and obvious pathological change of kidney damage was also shown in three model groups.Mercury content in blood and urine showed there was obvious dose -effect relationship.Compared with the control group, the related symptoms were the most prominent and stable with 1mg/kg dose group.There was a typical pathological types with nephrotic syndrome under light microscope with 1 mg/kg dose group and mercury accumulation in kidney tissues after stopped the injection.Conclusion It may be a reliable model of nephrotic syndrome induced by mercury intoxication in BN rats, which are injected subcutaneously with 1 mg/kg mercuric chloride solution once every other day for two weeks.%目的:建立汞中毒致

  4. Complex karyotype newly defined: The strongest prognostic factor in advanced childhood myelodysplastic syndrome

    NARCIS (Netherlands)

    G. Göhring (Gudrun); K. Michalova (Kyra); H.B. Beverloo (Berna); D. Betts (David); J. Harbott (Jochen); O.A. Haas (Oskar); G. Kerndrup (Gitte); L. Sainati (Laura); E. Bergstraesser (Eva); H. Hasle (Henrik); J. Stary (Jan); M. Trebo (Monica); M.M. van den Heuvel-Eibrink (Marry); M. Zecca (Marco); E.R. van Wering (Elisabeth); A. Fischer (Alexandra); P. Noellke (Peter); B. Strahm (Brigitte); F. Locatelli (Franco); C.M. Niemeyer (Charlotte); B. Schlegelberger (Brigitte)

    2010-01-01

    textabstractTo identify cytogenetic risk factors predicting outcome in children with advanced myelodysplastic syndrome, overall survival of 192 children prospectively enrolled in European Working Group of Myelodysplastic Syndrome in Childhood studies was evaluated with regard to karyotypic complexit

  5. SAPHO syndrome in childhood. A case report.

    Science.gov (United States)

    Vargas Pérez, Manuel; Sevilla Pérez, Belén

    2016-12-16

    The acronym of SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis and osteitis) combines a cluster of cutaneous and musculoskeletal manifestations, such as hyperostosis of bones of the anterior chest wall associated with acne fulminans and hidradenitis suppurativa. There are no validated diagnostic criteria in children. Nonsteroidal anti-inflammatory drugs are not always sufficient, and the use of corticosteroids, disease-modifying agents, tumor necrosis factor-α inhibitors or bisphosphonates may be necessary. We present the case of a child with polyarticular involvement, osteoarthritis of the sternoclavicular joint with severe inflammatory disorders and acne conglobata, with an excellent response to intravenous pamidronate.

  6. 糖皮质激素对原发性肾病综合征患者TNFα的影响%Effect of corticosteroids on TNFα level in patients with primary nephrotic syndrome

    Institute of Scientific and Technical Information of China (English)

    刘晓渭; 李冬梅; 李元勋; 赵正辉; 徐月清

    2001-01-01

    目的观察原发性肾病综合征(PNS)患者应用糖皮质激素(以下简称激素)前后血、尿TNFα变化. 方法检测了40例PNS患者激素治疗前及用激素后1,2,4,8 wk血、尿TNFα变化,并与20例正常人血、尿TNFα进行比较. 结果 PNS患者40例治疗前血、尿TNFα明显增高(血正常(68±19)(kU·L-1),PNS (461±362)(kU·L-1),尿正常0/kU·L-1 PNS (202±147)(kU·L-1)(P0.05). CONCLUSION TNFα is involved in invasion and disease progression of primary nephrotic syndrome. The effect and mechanisms of corticosteroids are correlated with the inhibition in the production of TNFα. Plasma and urine levels of TNFα can serve as an index of the effect of corticosteroids on patients with primary nephrotic syndrome.

  7. The clinical significance of gene polymorphism in children's primary nephrotic syndrome%基因多态性在儿童原发性肾病综合征中的临床意义

    Institute of Scientific and Technical Information of China (English)

    陈娟

    2010-01-01

    儿童原发性肾病综合征(PNS)的发病机制目前尚未完全明确.近年来研究显示,某些基因如血管紧张素转换酶基因、载脂蛋白E基因、血小板活化因子水解酶基因、人类白细胞抗原基因、糖皮质激素受体基因、细胞因子基因等的多态性与PNS的易患性、病理进展、激素疗效、疾病复发及预后明显相关,该文就与PNS有关的基因多态性的研究进展作一综述.%The pathogenesis of children's primary nephrotic syndrome has not been completely clear at present.In the past few years,The polymorphism of these genes,such as angiotensin-converting enzyme gene,apolipoprotein E gene,platelet-activating factor acetylhydrolase gene,human histocompatibility leukocyte antigen gene,glucocorticoid receptor gene and cytokine gene,ect have been discovered that were significantly correlated with susceptibility,pathological progress,steroid response,disease recurrence and prognosis of primary nephrotic syndrome.This article reviewed the research progress of PNS and gene polymorphism.

  8. 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.

  9. Unusual cause of childhood anemia: Imerslund grasbeck syndrome

    Directory of Open Access Journals (Sweden)

    Kishan Prasad Hosapatna Laxminarayana

    2011-01-01

    Full Text Available Imerslund Grasbeck syndrome (IGS is a rare autosomal recessive childhood disorder characterized by selective Vitamin (vit B 12 malabsorption with asymptomatic proteinuria without any structural renal pathology. The patients stay healthy for decades with life-long parenteral vit B12. We report a case of young female who presented with pancytopenia and proteinuria, evaluated in local hospitals as chronic hemolytic anemia (autoimmune cause, finally diagnosed as IGS on complete evaluation. She was treated with injectable vit B12 (1000 μg cyanocobalalmin and showed drastic recovery. IGS should be considered in patients with megaloblastic anemia not responding to oral vit B12 and associated proteinuria.

  10. [A case of advanced esophageal carcinoma with nephrotic syndrome completely responding to chemotherapy of docetaxel, nedaplatin and 5-fluorouracil].

    Science.gov (United States)

    Matsutani, Takeshi; Uchida, Eiji; Yoshida, Hiroshi; Suzuki, Seiji; Maruyama, Hiroshi; Yokoyama, Tadashi; Matsushita, Akira; Hirakata, Atsushi; Kawamoto, Masao; Arai, Hiroki; Umakoshi, Michinobu; Wakabayashi, Hideyuki; Sasajima, Koji

    2011-03-01

    A 78-year-old male was admitted to our hospital because of dysphagia. He had been diagnosed as nephritic syndrome at 30 years of age and had been treated with prednisolone 10 mg/day. Blood examination revealed renal dysfunction; BUN 25 mg/dL, Cr 1. 9 mg/dL, and glomerular filtration rate(GFR)47. 4 mL/min. Endoscopy showed a type 2 tumor at the middle thoracic esophagus, and the biopsy specimen revealed moderately differentiated squamous cell carcinoma pathologically. Computed tomography (CT) of the chest and abdomen showed no metastases at distant regions and lymph nodes. Clinical staging was Stage II (cT2cN0cM0). Because of old age and renal function, we chose chemotherapy using docetaxel, nedaplatin and 5-fluorouracil. The adverse event was grade 2 in leucopenia and grade 1 in inappetence, but the renal function did not progress. Repeated endoscopic examinations after chemotherapy revealed that the esophageal cancer was significantly reduced in size, and no cancer cells were pathologically detected by endoscopic biopsy, resulting in a complete response(CR). This chemotherapy of docetaxel, nedaplatin and 5-fluorouracil might be effective and tolerable for patients with renal dys- function due to nephritic syndrome.

  11. 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

  12. 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.

  13. 饮食营养干预在肾病综合征患者护理中的疗效评估%Evaluation on the Efficacy of Diet Intervention in Nursing Care of Patients With Nephrotic Syndrome

    Institute of Scientific and Technical Information of China (English)

    陈秀芹

    2015-01-01

    目的:分析肾病综合征患者接受饮食营养干预护理的效果。方法2012~2014年我院接收了60例肾病综合征患者,将这些患者分成对照组和实验组,均有30例,对照组采取常规药物治疗,实验组增加营养干预,对两组的临床护理情况进行比较分析。结果实验组患者接受营养干预后,其尿蛋白水平要比对照组低,两组的该项结果比较存在统计学差异,P<0.05。结论肾病综合征患者接受常规治疗和营养干预能够对患者的尿蛋白、血清蛋白等指标进行改善,提升治疗效果,对患者的身体免疫能力增强起到了很大的提升。%Objective To study the efficacy on patients with nephrotic syndrome under diet nutrition intervention. Methods From 2012 to 2014 in our hospital received 60 cases of patients with nephrotic syndrome,these patients were divided into the control group and the experimental group,in al 30 cases, the control group with conventional drug treatment,the experimental group with nutrition intervention,comparing and analysis of clinical nursing care between two groups. Results After the experimental group received nutritional intervention,level of the urine protein was lower than the control group, there were statistical difference,P<0.05. Conclusion Conventional therapy and nutrition intervention received by patients with nephrotic syndrome can improve their urinary protein,serum protein in patients with other indicators,improve the therapeutic effect,body's immune ability of patients.

  14. 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.

  15. Observation on Efficacy of Astragalus Injection in Treating Diabetic Nephrotic Edema

    Institute of Scientific and Technical Information of China (English)

    胡仲仪; 刘迟; 陈以平; 沈玲妹; 金亚明

    2002-01-01

    @@ At the mid-late stage of diabetic nephropathy massive proteinuria, hypoproteinemia and anasarca would develop which is nephrotic syndrome. Diuretics are often insensitive and treatment is very difficult. From January 1995 to December 1998, the authors adopted intravenous dripping infusion of Astragalus injection (AI) for patients with nephrotic syndrome due to diabetic nephropathy (DN). The control group was treated by Salvia injection (SI). The result was that the group treated by AI showed obvious diuretic and detumescent effect.

  16. 原发性肾病综合征合并甲状腺功能异常患者临床资料分析%Analysis of Clinical Data in Primary Nephrotic Syndrome Accompanied with Thyroid Dysfunction

    Institute of Scientific and Technical Information of China (English)

    申万琴; 吴歌; 赵占正; 靳云凤; 赵志红

    2011-01-01

    分析原发性肾病综合征合并甲状腺功能异常患者的临床特征,探讨其临床意叉.对37例原发性肾病综合征合并甲状腺功能异常患者的临床资料进行回顾性分析,治疗前检测甲状腺过氧化物酶抗体(Anti-TPO)、甲状腺球蛋白抗体(Anti-TG),并分别于治疗前后检测血清游离T3(FT3)、血清游离甲状腺激素(FT4)、促甲状腺激素(TSH)、及24小时尿蛋白量、血浆白蛋白,总胆固醇、甘油三酯.37例原发性肾病综合征合并甲状腺功能异常患者中,单纯FT3降低19例,占51.4%,单纯FT4降低7例,占18.9%,FT3降低及TSH升高伴Anti-TPO轻度升高2例,占5.4%;所分析患者中均无甲状腺功能减退的临床表现;血清FT3水平降低与24小时尿蛋白量呈负相关,与血浆白蛋白呈正相关,与总胆固醇、甘油三酯无明显相关性.原发性肾病综合征患者可出现FT3、FT4、TSH异常,且随病情改善而改善;甲状腺功能检测可能有助于判断原发性肾病综合征患者病情及预后.%To investigate the clinical significance, climcal features of patients with primary nephrotic syndrome accompanied with thyroid dysfunction were analysed. Thirty seven patients with primary nephrotic syndrome accompanied with thyroid dysfunction were selected in this retrospective study. Levels of FT3 , FT4 ,TSH, 24h proteinuria quantitative, serum albumin, total cholesterol, triglyceride were detected before and after treatment,while Anti-TPO and Anti-TG were detected before treatment. Decreased level of FT3 was detected in 19 cases(51.4%), decreased level of FT4 was detected in 7 cases (18.9%) and decreased level of FT3 accompanied with increased levels of TSH and Anti-TPO occured in 2 cases( 5.4 % ). None of the patients presented clinical manifestations of hypothyroidism. Serum level of FT3 was positively correlated with serum albumin but negatively correlated with urinary protein. No correlation was found between serum thyroid hormone

  17. Epidemiological characteristics of childhood migraine syndrome in Serbia

    Institute of Scientific and Technical Information of China (English)

    Marija Kne(z)evi(c)-Pogan(c)ev; Neboj(s)a Jovi(c); Vesna Iveti(c); Danka Filipovi(c); Dragan Katani(c)

    2011-01-01

    This study investigated childhood migraine, examining the clinical characteristics of different childhood migraine types, predisposing factors, and possible medical treatments in a sample of children in Serbia. This was the first epidemiological study in Serbia using the operational diagnostic criteria of the International Headache Society. The present study examined 30 636 children between 1988 and 2006, including 15 434 (50.38%) males and 15 202 (49.62%) females, in nine towns in Vojvodina, a northern province in Serbia. Migraine was reported in 2 644 (8.63%) children aged 3 to 7 years, consisting of 1 189 (8.0%) males and 1 455 (9.6%) females. Migraine with aura accounted for 25.55%, migraine without aura for 67.21% and other migraine syndromes for 7.23% of reported migraine sufferers. The mean age of first migraine attack across groups was 5 years and 1.8 months. Migraine with aura was associated with an earlier onset than migraine without aura.Migraine was more frequent among younger groups of children who were breastfed for a shorter period of time. Child healthcare in Vojvodina has a high level of specialization, with more than half of the children reporting migraine diagnosed and treated by neuropediatricians. Socioeconomic status,duration of breastfeeding, birth order, and age of entering full-day nursery care were found to be important factors for childhood migraine. These findings suggest that healthcare providers should educate parents about these predisposing factors, so they can be identified and avoided early.

  18. Tourette syndrome and other tic disorders of childhood.

    Science.gov (United States)

    Tamara, Pringsheim

    2013-01-01

    Tourettte syndrome (TS) is a common, childhood onset neuropsychiatric disorder consisting of multiple motor and one or more vocal tics which persist for more than 1 year. Comorbid psychiatric diagnoses are frequent in this patient population, including attention-deficit/hyperactivity disorder (ADHD) and obsessive-compulsive disorder (OCD). Tics can be simple or complex, and have a tendency to change over time. Tics are preceded by a premonitory sensation, wax and wane in frequency, and are often exacerbated by stress or excitement. Tic severity usually peaks in childhood, and improves in early adulthood. TS is a highly heritable disorder with a polygenic inheritance. The fundamental pathophysiology of TS is not known, although existing evidence suggests that it involves dysfunction of the basal ganglia and frontal cortical circuits, as well as dopaminergic neurotransmission. Treatment of TS involves consideration of symptom severity and comorbidity. In general, comorbid ADHD and OCD lead to greater disability in these patients, and therefore are the initial treatment priority. As treatment for tics does not alter the natural history of the disorder, it is only recommended if the tics are causing disability. Effective treatments to suppress tics include α-adrenergic agonists and antipsychotic medications.

  19. 浅析腹水浓缩回输治疗肾病综合征顽固性腹水的临床观察探讨%Analysis of Ascites Reinfusion Treatment of Nephrotic Syndrome Refractory Aascites

    Institute of Scientific and Technical Information of China (English)

    陈涛; 马涛

    2014-01-01

    目的:浅析腹水浓缩回输治疗肾病综合征顽固性腹水的临床观察探讨。方法2000年1月~2004年5月我科利用血液透析机进行腹水浓缩后再回输,治疗肾病综合征顽固性腹水患者32例。结果所有病员在治疗后腹水逐渐减退,尿量增加,双下肢水肿减轻、精神好转,食欲增加,体力增强,未出现发热、心慌及其他症状。讨论本方法由于回输自身蛋白,临床症状改善快,住院时间短,不受住院地点限制,费用降低,能将有限的资金用于更多的治疗上。%Objective To analyze the ascites concentration into the clinical observation to explore for the treatment of nephrotic syndrome with refractory ascites. Methods From January 2000 to May 2004 I col i ascites concentration in blood dialysis machine to go back to lose, after treatment of nephrotic syndrome and 32 patients with refractory ascites. Results Al stents in the treatment of ascites after gradual y decreases, increase urine output, double leg edema relieve, spirit is bet er, increase appetite, physical strength enhancement, the symptoms of fever, flustered and other not.Conclusion This method due to the lost its protein, improve the clinical symptoms rapidly, short hospitalization time, not in place to restrict, reduce the cost, can the limited funds for more treatment.

  20. Meta-analysis of the Efficacy of Bailing Capsules on Primary Nephrotic Syndrome%百令胶囊治疗肾病综合征的Meta分析

    Institute of Scientific and Technical Information of China (English)

    章建军; 谢升阳

    2013-01-01

    目的 对现有的关于百令胶囊对于肾病综合征成人患者的治疗作用的文献进行Meta分析,为临床实践提供依据.方法 通过Pubmed、中国生物医学文献数据库、中国期刊全文数据库等数据库检索,选取相关文献.提取效应尺度指标相关数据进行定量Meta分析或定性描述.结果 共纳入5篇文献.Meta分析结果显示百令胶囊能够降低肾病综合征患者的24h尿蛋白[SMD=-2.35(-3.72,-0.97),P<0.01],提高血清白蛋白水平[SMD=0.94(0.37,1.52),P=0.01],但对于血肌酐[SMD=-0.50(-1.25,0.26),P=0.20]及血尿素氮[SMD=-0.10(-0.36,0.17),P=0.48]的水平没有明显的改善作用.此外,其中有两个研究报道了百令胶囊能够降低血总胆固醇、甘油三酯及低密度脂蛋白.结论 百令胶囊的辅助治疗能够进一步减少肾病综合征患者的蛋白尿,提高血浆白蛋白水平,且可能能够降低患者血脂.但这些结论需要进一步临床试验证实.%OBJECTIVE To perform Meta-analysis of published articles on the efficacy of Bailing capsules for adult patients with primary nephrotic syndrome(PNS) and provide evidence for clinical practice.METHODS We searched Pubmed,CBM,CNKI and other databases to identify eligible articles and extract effect measure-related data to perform Meta-analysis.And qualitative description was also conducted for those unable to be combined.RESULTS Five records were selected.The results of Meta-analysis showed that Bailing capsules could reduce proteinuria [SMD=-2.35(-3.72,-0.97),P<0.01] and improve serum albumin [SMD=0.94 (0.37,1.52),P=0.01] in PNS patients.However,Bailing capsules adjuvant therapy had no effect on serum creatinine [SMD=-0.50(-1.25,0.26),P=0.20] and urea nitrogen level [SMD=-0.10(-0.36,0.17),P=0.48].Additionally,two studies reported that Bailing capsules could lower serum cholesterol,triglyceride and low density lipoprotein.CONCLUSION Addition of Bailing capsules could decrease proteinuria and enhance serum

  1. Clinical Research on Treatment of Hyperkinetic Syndrome of Childhood by Electroacupuncture plus Acupoint Application

    Institute of Scientific and Technical Information of China (English)

    WU Yao-chi; KUAI Le

    2003-01-01

    Objective To observe the clinical therapeu tic effect of hyperkinetic syndrome of childhood treated by electroacupuncture plus acupoint application. Method Sixty-five cases with hyperkinetic syndrome of childhood were treated by electroacupuncture plus acupoint application (electroacupuncture group); 53 cases were treated by acupuncture (acupuncture group) and 53 cases were treated by Ritalin (west drug group). The above three groups were compared with each other in therapeutic effect. Results The effective rate of treating hyperkinetic syndrome of childhood by electroacupuncture plus acupoint application was 87.7%; the effective rate in west drug group was 86.8% and in acupuncture group was 77.4%. A comparison among the three groups showed there was no significant difference in clinical ther apeutic effect ( P > 0.05 ). Conclusion Electroacupuncture plus acupoint application was an effective therapy of hyperkinetic syndrome of childhood.

  2. 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

  3. Childhood sexual trauma in women with interstitial cystitis/bladder pain syndrome

    DEFF Research Database (Denmark)

    Nickel, J Curtis; Tripp, Dean A; Pontari, Michel

    2011-01-01

    The impact of early lifetime trauma on symptom severity and quality of life of patients with interstitial cystitis/bladder pain syndrome (IC/BPS) has not been fully elucidated. We wanted to determine and compare the prevalence and impact of childhood traumatic events, with an emphasis on childhood...

  4. 卡托普利联合低分子肝素治疗肾病综合征的临床疗效分析%Analysis of nephrotic syndrome treatment by captopril combined with low molecular heparin

    Institute of Scientific and Technical Information of China (English)

    黄恬

    2014-01-01

    Objective To observe the treatment effect of captopril combined low molecular heparin in nephrot-ic syndrome .Methods Sixty and eight patients with nephrotic syndrome were collected from Chongqing cancer hos-pital ,which were randomly divided into control group and observation group .Conventional treatment were used in the control group ,captopril combined with low molecular heparin were added in the observation group .cholesterol , triglyceride ,urine protein ,whole blood viscosity ,hematocrit ,fibrinogen ,hematocrit of pigeon before and after treat-ment were compared .Results Total effective rate of observation group was 92 .1% ,which was significantly higher than the control group′s 83 .3% ,the difference was statistically significant (P<0 .05) .The observation group′s cho-lesterol ,triglyceride ,urine protein ,whole blood viscosity ,hematocrit ,fibrinogen ,hematocrit of pigeon were significant lower than those of the control group (P<0 .05) .Conclusion On the basis of conventional treatment ,captopril and low molecular heparin could significantly improve curative effect ,renal function and high coagulation state on pa-tients with nephrotic syndrome .%目的:观察卡托普利联合低分子肝素治疗肾病综合征的临床效果。方法选择本院收治的68例肾病综合征患者为研究对象。对照组30例患者,给予常规治疗28 d;观察组38例患者,在常规治疗的基础上给予卡托普利联合低分子肝素治疗28 d。观察并比较两组患者治疗前后胆固醇、三酰甘油、肌酐、清蛋白、尿蛋白、全血高切黏度、全血低切黏度、血浆黏度、血浆纤维蛋白原、血细胞比容等指标变化情况。结果观察组治疗总有效率达92.1%,明显高于对照组的83.3%,比较差异有统计学意义( P<0.05)。治疗28 d后,观察组胆固醇、三酰甘油、尿蛋白、全血黏度、血浆黏度、血浆纤维蛋白原、血细胞比容等指标明显低于对照

  5. Genetic Aspects of Nephrotic Syndrome

    DEFF Research Database (Denmark)

    Joshi, Shivani

    SSNS. In Study IV we performed a literature study on published disease causing variants in SRNS and based upon available evidence we developed a practical diagnostic algorithm for genetic evaluation of patients with SRNS. Several gene variants are involved in the pathogenesis of SRNS and genetic...... steroid dependence or become frequent relapsers. Repeated courses of corticosteroid treatment often cause significant associated morbidity. Familial occurrence of SSNS is rare and suggests a potential genetic origin. However, very little data on molecular genetics of familial SSNS is available...... in literature and no causal genes have yet been identified. Genetic aspects of NS bear important implications in therapeutic decisions and genetic counselling in SRNS patients and family members. During the present Ph.D. project we have studied the influence of genetic factors in patients with SRNS and familial...

  6. CHILDHOOD GUILLIAN-BARRE SYNDROME IN THE IRAN’S EAST AZERBAIJAN PROVINCE: 2001-2005

    Directory of Open Access Journals (Sweden)

    M. Barzegar

    2009-01-01

    Full Text Available  ObjectiveThis study aims at determining the epidemiologic, presenting symptoms, clinical course and electrophysiologic features of childhood Guillain-Barre Syndrome (GBS in the East Azarbaijan province over a period of five years.Materials & Methods All the patients, agedResultsOne hundred and twelve subjects were enrolled during this period. The average annual incidence rate was 2.21 per 100000 population of children agedConclusion The axonal type of GBS is a relatively common form of childhood GBS occurring in East Azerbaijan.Keywords: Guillian-Barre syndrome , Childhood, Epidemiology, Electrodiagnosis, Clinical features, East Azarbaijan

  7. 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.

  8. The analysis of clinical features and outcomes of children with late steroid resistant primary nephrotic syndrome%儿童迟发激素耐药型肾病综合征的临床和转归分析

    Institute of Scientific and Technical Information of China (English)

    杨青; 陈敏广; 叶晓华; 林洪洲

    2015-01-01

    目的分析儿童迟发激素耐药型肾病综合征的临床特点和转归。方法回顾性分析本院2008年1月至2013年12月收治的迟发激素耐药型肾病综合征患儿的临床病理特点及治疗转归。结果同期收治的激素初治敏感型肾病综合征患儿513例,其中迟发激素耐药型肾病综合征21例,占4.1%。21例中男11例,女10例,单纯型肾病16例,肾炎型肾病5例。在出现迟发性激素耐药前,14例表现为频复发或激素依赖,7例为非频复发或无复发,从激素敏感至发生激素迟发耐药的时间为(206.10±212.64)天。与激素敏感组比较,初次激素治疗尿蛋白阴转时间更长,为(14.05±7.71)天比(10.26±5.66)天(P =0.003),女性更多见(11/10比380/112,P =0.018),与肾病复发组比较,首次复发距肾病初次缓解时间更短,为(34.95±39.44)天比(122.10±168.07)天(P=0.019)。12例接受肾活检,病理显示微小病变(MCD)7例,局灶节段性肾小球硬化(FSGS)4例,C1q肾病1例。85.7%(18/21例)应用环孢素和环磷酰胺达到完全缓解,1例FSGS激素联合吗替麦考酚酯治疗达部分缓解,仅1例C1q肾病多药治疗无效,3年后因终末期肾脏病(ESRD)死亡。结论本组儿童迟发耐药性肾病综合征并非罕见,初次激素治疗尿蛋白阴转时间、首次复发距肾病初次缓解时间、性别或可预测迟发耐药的发生,病理改变以MCD和FSGS为主,对激素联合免疫抑制剂治疗大多数反应良好,预后良好,但需注意少部分病例持续耐药,预后不良。%ObjectiveTo analyze the clinical features and outcomes of children with late steroid-resistant nephrotic syndrome.MethodRetrospective analyze the clinical and pathological characteristics and treatment outcomes of children with late steroid-resistant nephrotic syndrome in our hospital from January 2008 to December 2013.Results There were 513 cases with steroid

  9. Efficacy of leflunomide in treatment of refractory nephrotic syndrome with concurrent infections%来氟米特治疗难治性肾病综合征并发感染的疗效研究

    Institute of Scientific and Technical Information of China (English)

    吴金土

    2012-01-01

    目的 观察来氟米特治疗难治性肾病综合征并发感染的临床疗效,为临床治疗提供依据.方法 选取医院2010年9月-2011年3月56例难治性肾病综合征患者,利用来氟米特进行治疗,观察其临床疗效.结果 56例患者经回访均完全缓解,缓解率为100.0%;其中29例占51.8%连续4年未复发,16例占28.6%连续3年未复发,11例占19.6%连续两年未复发;使用激素治疗共56例,持续缓解时间(5.5±0.7)个月;使用激素+环磷酰胺治疗共56例,持续缓解时间(9.5±0.8)个月;使用激素+吗替麦考酚酯治疗共33例,持续缓解时间(8.0±0.7)个月;使用激素+雷公藤治疗共25例,持续缓解时间(7.5±0.6)个月;使用激素+来氟米特治疗共56例,持续缓解时间(38.5±12.0)个月;使用激素+来氟米特治疗持续缓解时间明显长于其他药物治疗者.结论 来氟米特能够有效减少肾病综合征复发次数,减低感染率,延长难治性肾病综合征的持续缓解时间.%OBJECTIVE To observe the clinical efficacy of leflunomide in treatment of refractory nephrotic syndrome,so as to provide bases for clinical treatment- METHODS A total of 56 patients with refractory nephrotic syndrome from Sep 2010 to Mar 2011 were selected,all the subjects were treated with leflunomide, the clinical efficacy was observed. RESULTS Totally 56 patients were complete remission after a return visit with the remission rate of 100.0%, among which 29 patients (51. 8%) patients were without recurrence for 4 years, 16 patients (28. 6%) patients were without recurrence for 3 years, 11 patients (19. 6%) patients were without recurrence for 2 consecutive years; a total of 56 cases were treated with hormone with the sustained remission time (5. 5±0. 7) months; 56 cases were treated with hormone therapy plus cyclophosphamide with the sustained remission time of (9. 5±0. 8) months) 33 cases were treated with hormone plus mycophenolate mofetil with the sustained

  10. 雷公藤多苷联合泼尼松治疗老年肾病综合征的疗效观察%Effect of tripterygium wilfordii together with prednisone in treatment of elderly primary nephrotic syndrome

    Institute of Scientific and Technical Information of China (English)

    许勇芝; 黄志清; 唐蓉

    2009-01-01

    目的 观察雷公藤多苷联合中小剂量泼尼松治疗老年原发性肾病综合征的疗效.方法 78例老年原发性肾病综合征患者随机分为治疗组(n=42)和对照组(n=36),治疗组接受雷公藤多苷联合中小剂量泼尼松治疗,对照组接受大剂量泼尼松治疗.治疗期间定期监测24 h尿蛋白、血清白蛋白、肾功能、血脂等指标,观察疗效及复发率.结果 治疗6个月后治疗组完全缓解24例,部分缓解12例,无效6例,对照组分别为13例、10例、13例.治疗组总缓解率为85.7%,明显高于对照组的66.7%(P<0.05);治疗组复发率为14.3%,明显低于对照组的36.1%(P<0.05).结论 雷公藤多苷联合泼尼松治疗老年原发性肾病综合征疗效优于单纯应用泼尼松.%Objective To investigate the therapeutic effect of tripterygium wilfordii and prednisone in old patients with primary nephrotic syndrome. Methods 78 elderly primary nephrotic syndrome patients were randomly divided into two groups. 42 patients in treated-group were treated with tripterygium wilfordii and prednisone while 36 patients in control-group were treated with prednisone only. The curative effect,24 hours urinary protein, serum albumin,plasma lipid and renal function were detemined after six months. The responses of the patients were classified as complete remission(CR) ,partial remission(PR) and NO-response. Results After six months treatment, there were 24 patients got to CR,12 patients to PR,and 6 patients remained refractory to treatment group. While there were 13 patients got to CR,10 to PR,and 13 as refractory in the control group. The general effective rate in the treatment group was 85.7% ,which was markedly higher than that of the control group,which is 66. 67% (P<0. 05). The recurrence rate in the treatment group and control group were 14. 3% and 36. 1% respectively (P<0. 05 ). Conclusion The curative effect of tripterygium wilfordii and prednison on elderly primary nephrotic syndrome

  11. Observe the Curative Effect of Refractory Nephrotic Syndrome of Children in Treatment With Chinese Medicine%中药配合治疗小儿难治性肾病综合征的疗效观察

    Institute of Scientific and Technical Information of China (English)

    王晓华

    2015-01-01

    Objective To investigate the clinical efficacy of refractory nephrotic syndromewith chinese medicine treatment of children. Methods 56 patients with refractory nephrotic syndrome were randomly divided into study group and normal group,there were 28 cases. The conventional group received cytotoxic drugs shock therapy,studygroup in the conventional group based on the combined treatment of traditional chinese medicine,after 6 months of treatment,and adverse reaction were compared between the two groups of clinical efficacy.ResultsThe two groups after treatment,urine protein, cholesterol level were improved,but the study group improved amplitude better than routine group,and the total efficiency rate was higher than the conventional group,the significant difference has statistics significance(P<0.05); the study group the incidence of adverse reaction is lower than that of routine group,significant difference has statistics significance(P<0.05).Conclusion For the treatment of children with refractory nephrotic syndrome in conventional shock treatment on the basis of,the use of chinese medicine treatment can obviously improve the symptoms in children, improve the clinical efficacy,with low incidence of adverse reactions.%目的:探讨中药配合治疗小儿难治性肾病综合征的临床疗效。方法将我院收治的56例难治性肾病综合征患儿随机分为研究组和常规组,均为28例。常规组给予细胞毒类药物冲击治疗,研究组在常规组基础上联合中药治疗,治疗6个月后,对比两组临床疗效和不良反应情况。结果通过治疗后,两组胆固醇、尿蛋白定量水平均得到改善,但研究组改善幅度优于常规组,且总有效率也明显高于常规组,差异显著有统计意义(P<0.05);研究组不良反应发生率低于常规组,差异有统计意义(P<0.05)。结论对于小儿难治性肾病综合征的治疗在常规冲击治疗基础上,加用中药治疗可明显改

  12. Auriculotemporal (Frey) syndrome in late childhood: an unusual variant presenting as gustatory flushing mimicking food allergy.

    Science.gov (United States)

    Kaddu, S; Smolle, J; Komericki, P; Kerl, H

    2000-01-01

    Auriculotemporal or Frey syndrome is characterized mainly by recurrent episodes of facial gustatory flushing and/or sweating, limited to the cutaneous distribution of the auriculotemporal nerve. Although relatively common in adults following injury to the auriculotemporal nerve or parotid disease, the condition has rarely been reported in children. Moreover, in childhood, auriculotemporal syndrome has been described mainly in infancy and early childhood as a sequel of perinatal birth trauma resulting from assisted forceps delivery. We report a 13-year-old girl with a 2-month history of recurrent, painless, preauricular gustatory flushing without sweating, initially suspected to be a food allergy. Detailed inquiry revealed a history of a bicycle accident with mandibular condyle fracture 7 years prior to the onset of symptoms. Our patient demonstrates an unusual presentation of auriculotemporal syndrome in late childhood as gustatory flushing mimicking food allergy. Awareness of this variant is essential for prompt recognition, thus avoiding unnecessary laboratory tests, especially as this condition usually resolves spontaneously.

  13. Nephrotic presentation in hydatid cyst disease with predominant tubulointerstital disease

    Directory of Open Access Journals (Sweden)

    Feroz Aziz

    2009-06-01

    Full Text Available Feroz Aziz1, Tanmay Pandya1, Himanshu V Patel1, Paladugu Ramakrishna1, Kamal R Goplani1, Manoj Gumber1, Aruna V Vanikar2,  Kamal Kanodia2, Pankaj R Shah1, Hargovind L Trivedi11Department of Nephrology and Transplantation Medicine; 2Department of Pathology, Lab Medicine, Transfusion Services and Immunohematology, G.R. Doshi and K.M. Mehta Institute of Kidney Diseases and Research Centre (IKDRC, Ahmedabad, Gujarat, IndiaAbstract: Renal involvement, which can rarely occur in echinococcosis, more commonly manifests as hydatid cyst of the kidney. Scattered case reports of nephrotic syndrome secondary to hydatid cyst in the liver or lung have been reported for over two decades. The glomerular picture varied from minimal change lesion to mesangiocapillary glomerulonephritis. We report a case of predominantly tubulointerstitial nephritis with mesangioproliferative glomerulonephritis in a patient with hepatic hydatid cyst which responded to cyst resection alone. Keywords: echinococcosis, hydatid cyst, kidney, nephrotic syndrome, tubulointerstitial nephritis

  14. Cushing’s syndrome in childhood: update on genetics, treatment, and outcomes

    Science.gov (United States)

    Lodish, Maya

    2015-01-01

    Purpose of review To provide an update on the genes associated with Cushing’s syndrome in children, as well as to familiarize the clinician with recent treatment guidelines and outcome data for children with Cushing’s syndrome. Recent findings The list of genes associated with Cushing’s syndrome continues to grow. In addition, treatment for childhood Cushing’s syndrome is evolving. As long-term follow-up data on children becomes available, clinicians need to be aware of the issues that require attention. Summary Knowledge of the specific genetic causes of Cushing’s syndrome has potential implications for treatment, surveillance, and counseling. Advances in surgical technique, radiation modalities, and medical therapies offer the potential for additional treatment options in Cushing’s syndrome. Early identification and management of post-treatment morbidities in children treated for Cushing’s syndrome is crucial in order to optimize care. PMID:25517021

  15. 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.

  16. 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

  17. CD2相关蛋白在肾病综合征中的表达及意义%Expression of CD2AP on podocytes with different pathological types of nephrotic syndrome

    Institute of Scientific and Technical Information of China (English)

    史秀岩; 张春; 付应峰; 朱忠华; 朱红艳; 易丽霞; 付玲

    2012-01-01

    目的 观察不同病理类型的原发性肾病综合征(nephrotic syndrome,NS)患者肾小球足细胞中CD2相关蛋白(CD2AP)的表达,探讨其与足细胞损伤的关系.方法 选取原发性NS患者54例,10例同期肾肿瘤切除患者正常肾组织作为对照.肾活检后常规染色观察肾脏组织病理改变,肾组织行免疫荧光法CD2AP和肾小球上皮细胞蛋白-1(GLEPP1)双重标记,对肾小球CD2AP的表达进行定位;分别用real time PCR和免疫组化SP法检测组织中CD2AP的表达,采用real time PCR检测nephrin的表达,透射电镜观察足细胞的结构变化,并定量测量足突密度.结果 (1)NS患者肾小球中CD2AP的表达及nephrin的表达下调,足细胞足突不同程度融合,足突密度降低.(2)病理表现为微小病变性肾病(minimal change disease,MCD)、局灶性节段性肾小球硬化(focal segmental glomerulosclerosis,FSGS)和膜性肾病(membranous nephropathy,MN)的NS患者CD2AP表达及nephrin表达较对照组明显降低,且CD2AP与nephrin表达呈正相关,病理表现为MCD和FSGS的NS患者CD2AP表达与足突密度呈正相关.结论 本研究首次发现原发性NS患者肾小球足细胞中CD2AP的表达降低,且在MCD和FSGS中与足细胞病变程度相关,提示CD2AP低表达在足细胞病变为主的肾小球疾病中发挥重要作用.CD2AP有利于诊断足细胞病变的早期检测,对CD2AP表达减低进行早期干预可能有助于延缓疾病进展.%Purpose To investigate the CD2AP expression of the renal podocytes in patients with nephrotic syndrome, and to study the association between expression of CD2AP and podocyte injury. Methods 54 patients with nephrotic syndrome ( NS ) were studied, and 10 patients with renal tumor undergone nephrectomy were enrolled as control. Renal pathological change after renal biopsy was bserved. CD2AP and GLEPP1 by immunofluorescence double staining were performed. The renal CD2AP was detected by immunohis-tochemistry and quantitive real

  18. 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.

  19. Autonomic dysfunction in childhood Guillain-Barré syndrome.

    Science.gov (United States)

    Dimario, Francis J; Edwards, Carrie

    2012-05-01

    This investigation correlated incidence and degree of autonomic dysfunction with the degree of motor impairment in children hospitalized with Guillain-Barré syndrome. Motor weakness varies, as does the effect on autonomic function including heart rate, vasomotor stability, sweating, continence, and blood pressure. After Institutional Review Board approval, hospitalized patients with Guillain-Barré syndrome syndrome.

  20. Childhood sexual trauma in women with interstitial cystitis/bladder pain syndrome

    DEFF Research Database (Denmark)

    Nickel, J Curtis; Tripp, Dean A; Pontari, Michel

    2011-01-01

    The impact of early lifetime trauma on symptom severity and quality of life of patients with interstitial cystitis/bladder pain syndrome (IC/BPS) has not been fully elucidated. We wanted to determine and compare the prevalence and impact of childhood traumatic events, with an emphasis on childhoo...

  1. Childhood maltreatment and the response to cognitive behavior therapy for chronic fatigue syndrome.

    NARCIS (Netherlands)

    Heins, M.J.; Knoop, H.; Lobbestael, J.; Bleijenberg, G.

    2011-01-01

    Objective: To examine the relationship between a history of childhood maltreatment and the treatment response to cognitive behavior therapy for chronic fatigue syndrome (CFS). Methods: A cohort study in a tertiary care clinic with a referred sample of 216 adult patients meeting the Centers for Disea

  2. 微小病变性肾病综合征激素抵抗的多因素分析%Multiple factors analysis of steroid resistance of minimal change nephrotic syndrome

    Institute of Scientific and Technical Information of China (English)

    王明军; 张金荣; 徐婉梅; 黎伟; 张影华; 张元元; 何建静; 廖蕴华

    2015-01-01

    Aim:To evaluate risk factors of steroid-resistant minimal change nephrotic syndrome. Methods:retrospectively analysed 68 patients who were confirmed as minimal-change nephritic syndrome by renal biopsies,and divided as group steroid-resistant group(n =30)and non steroid-resistant group(n=38).A univariate analysis was performed firstly.Then the significant ones were performed a multivari-ate analysis by using binary Logistic regression model.Lastly,the sensitivity,the specificity,and the best threshold value of the indexes,which as indicating the steroid-resistant minimal change disease,was testi-fied by drawing a receiver operating characteristic curve(ROC).Results:(1)combined infection(P =0.014),red blood cell count(P =0.005 ),total cholesterol (P =0.015 ),24 h urinary protein (P = 0.000),serum albumin(P =0.011),high urine uric acid(P =0.045),C4 decrease(P =0.028)were all the risk factors of steroid-resistant minimal change disease(P 3.5 /HP,24 h u-rinary protein >5.57 g/d total cholesterol >9.49 mmol /L were the best threshold value indicating ster-oid-resistant minimal change disease.Conclusion:(1)infection,hematuria,24 h urinary protein quanti-tative is independent risk factors of Steroid-resistant minimal change nephrotic syndrome.(2)when Oc-cur hematuria,24 h urinary proteind≥5.57 g/d,total cholesterol≥9.49 mmol /L,,Should be alert to the occurrence of Steroid-resistant minimal change nephrotic syndrome.%目的:探讨微小病变性肾病综合征激素抵抗的相关危险因素.方法:回顾性调查68例经肾组织活检确诊为微小病变性肾小球肾炎患者临床资料,实验室检查结果及随访情况,将患者分为激素抵抗组(30例)及非抵抗组(38例),采用 SPSS17.0软件,进行单因素分析,对有意义的单因素采用二分类 Logistic 回归进行多因素分析,绘制受试者工作特性曲线(ROC)评价检验指标在预测微小病变性肾病综合征激素抵抗时的敏感度,特异

  3. 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

  4. 新生儿弥漫性系膜硬化型先天性肾病综合征一例报告并文献复习%To report of a case of congenital nephrotic syndrome of diffuse mesangial sclerosis and review literature

    Institute of Scientific and Technical Information of China (English)

    关则想; 徐玲玲; 莫樱; 蒋小云

    2012-01-01

    Objective: To report a case of congenital nephrotic syndrome whose renal pathology was diffuse mesangial sclerosis ( DMS) , in order to improve the recognition of the disease. Methods; To collect the clinical manifestations characteristic imaging of a case with congenital nephrotic syndrome of DMS, and to review and summarize the etiology, clinical manifestations, pathological features and prognosis of DMS. Results: She had early onset of clinical features of nephrotic syndrome, soon become renal insufficiency, progress to end - stage renal disease, and her renal biopsy pathology was diffuse mesangial sclerosis. She died two months later. Conclusions ; DMS manifested congenital nephrotic syndrome and often comprised renal insufficiency earlier, then progressing to renal failure. Renal biopsy is way to diagnose DMS. The disease has a poor prognosis, with no special treatment at present.%目的 报道一例新生儿弥漫性系膜硬化型先天性肾病综合征,以提高对该病的认识.方法 收集一例于2010年10月在中山大学附属第一医院儿科住院确诊为先天性肾病综合征弥漫性系膜硬化型的患儿临床资料,并复习文献,总结弥漫性系膜硬化型先天性肾病综合征的病因、临床表现、病理特点及预后.结果 患儿生后即有水肿、大量蛋白尿等肾病综合征表现,并很快出现肾功能不全,进展至肾功能衰竭,肾活检病理符合弥漫性系膜硬化型,2个多月后死亡.结论 先天性肾病综合征弥漫性系膜硬化型的临床表现为出生时或幼儿期内出现肾病综合征的特征,起病时可已有肾功能不全,进行性肾功能减退,确诊须肾穿病理,本病预后差,目前无特殊治疗.

  5. 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.

  6. 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

  7. Adults with Asperger Syndrome: A Childhood Disorder Grows Up

    Science.gov (United States)

    Wilkinson, Lee A.

    2007-01-01

    Asperger syndrome is a chronic developmental disorder characterized by problems in social relatedness, empathic communication and understanding, and circumscribed interests. The inclusion of Asperger's Disorder (Asperger syndrome) in the "Diagnostic and Statistical Manual of Mental Disorders" (DSM-IV; American Psychiatric Association, 1994), has…

  8. The clinical study on evidence - based nursing in patients with nephrotic syndrome%循证护理在肾病综合征护理中的临床应用

    Institute of Scientific and Technical Information of China (English)

    胡晓萍; 王亚华; 熊迎春

    2015-01-01

    目的:探讨循证护理在肾病综合征护理中的作用。方法:将入组患者随机分为两组,对照组采用传统护理方法进行护理干预。实验组依据循证护理干预法,制订出一套系统有效的护理措施。结果:循证护理能够改善患者的生活习惯,提高护理满意度(P <0.05)。结论:循证护理在肾病综合征中具有积极的护理作用,可以改善患者生活习惯,提高护理满意度。%Objective:To investigate the effect of evidence - based nursing care in thesyndrome of kidney. Methods:The patients were randomly divided into two groups,the control group used the traditional nursing care intervention. The experimen-tal group on the basis of evidence - based nursing intervention,to develop a set of system and effective nursing measures. Re-sults:the evidence based nursing can improve the patients living habits,improve nursing satisfaction(P < 0. 05). Conclusion:Evidence - based nursing haspositive effect on nephrotic syndrome nursing,improve the living habits of patients,improve nurs-ing satisfaction.

  9. Analysis of risk factors in primary nephrotic syndrome patients with urinary tract infection%原发性肾病综合征患者尿路感染危险因素分析

    Institute of Scientific and Technical Information of China (English)

    郝向勤; 郝亚宁

    2014-01-01

    ObjectiveTo investigate the risk factors in primary nephrotic syndrome (PNS) patients with urinary tract infections (UTI) and provide clinical reference for the prevention of urinary tract infection. MethodFrom February 2007 to October 2013, the clinical data of 570 cases with PNS combined with 154 cases of UTI were retrospectively analyzed. Through the comparative analysis of two groups and the statistical analysis of related factors (including gender, age, complications, hormone dosage and immunosuppressor) in 154 cases of PNS combined with UTI, in order to ifnd the relations between various factors and primary nephrotic syndrome of urinary tract infections.Result154 cases were urinary tract infection in 570 cases with PNS, and the incidence of infection was 27.0%; compared with patients with no UTI complication, the content of the urine protein, serum protein, lipoprotein (a) [Lp(a)], IgG and low density lipoprotein (LDL) in patients with PNS combined with UTI had signiifcant changes (P0.05). The results of single factor showed that the incidence of urinary tract infection was not related with age. In different stages of age, the incidence of children was greater than seniors and young adults. Two groups had signiifcant difference (P<0.05). Patients can improve the incidence of UTI (P<0.05) by the use of immunosuppressor and high doses of hormones and other complications.ConclusionLow serum proteinemia, low IgG emesis emia, high proteinuria, high LDL and (or) Lp(a) may indicate the incidence of primary nephrotic syndrome combined with urinary tract infections. Age, complications, with or without the use of immunosuppressor and concentration of hormone may be risk factors of causing urinary tract infection.%目的:探讨原发性肾病综合征(PNS)患者并发尿路感染(UTI)的危险因素,为诊断和治疗PNS并发UTI提供临床参考依据。方法对2007年2月至2013年10月本院收治的570例PNS患者中并发UTI的154例患者(试验

  10. 灯盏细辛联合低分子肝素治疗肾病综合征的临床疗效观察%Clinical Effect of Herba Erigerontis Breviscapi Combined with Low Molecular Heparin on Nephrotic Syndrome

    Institute of Scientific and Technical Information of China (English)

    廖家贤; 韦秋敏; 卢荣羡; 韦东艳

    2012-01-01

    Objective To explore the clinical effect of Herba Erigerontis Breviscapi combined with low molecular heparin on nephrotic syndrome. Methods We performed a retrospective analysis on the clinical data of 78 patients with primary nephrotic syndrome patients in our hospital from January 2009 to December 2010. The patients were randomly divided into two groups: treatment group and control group, with 39 cases in each group. Patients in the control group were given conventional treatment. Patients in the treatment group were given Herba Erigerontis Breviscapi combined with low molecular heparin besides conventional treatment. The treatment period lasted for about two weeks. The levels of urine protein, serum triglyceride (TG) , total cholesterol (Choi) , serum albumin (ALB) and activation of blood coagulation enzyme time (APTT) , fibrinogen (FIB) of patients in two groups were tested and the clinical effect was compared between two groups. Results The curative effect of the treatment group was better than the control group, with the effective rate of 92.3% and 71.8% (P <0.05). Compared with the control group, the treatment group, there was a signinicant increase of 24 h urine protein, and a significant increase of serum albumin, serum triglyceride and total cholesterol in patients in treatment group. The active site blood coagulation time was significantly longer and the fibrinogen (FIB) was reduced in treatment group than control group. Conclusion Herba Erigerontis Breviscapi combined with low molecular heparin can improve the levels of urinary albumin, decrease the blood lipids levels, improve the high coagulative state and improve the serum albumin levels of primary nephrotic syndrome patients, so has good therapeutic effect.%目的 探讨灯盏细辛联合低分子肝素治疗肾病综合征的临床疗效.方法 回顾性分析2009年1月至2010年12月在河池市中医院治疗的78例原发性肾病综合征患者的临床资料,随机分为2组,对照组39

  11. 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

  12. The prospective association between childhood cognitive ability and somatic symptoms and syndromes in adulthood : the 1958 British birth cohort

    NARCIS (Netherlands)

    Kingma, Eva M.; Rosmalen, Judith G. M.; White, Peter D.; Stansfeld, Stephen A.; Clark, Charlotte

    2013-01-01

    BACKGROUND: Cognitive ability is negatively associated with functional somatic symptoms (FSS) in childhood. Lower childhood cognitive ability might also predict FSS and functional somatic syndromes in adulthood. However, it is unknown whether this association would be modified by subjective and obje

  13. 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

  14. 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).

  15. [Multi-organ failure as first clinical sign of macrophage activation syndrome in childhood Still's disease].

    Science.gov (United States)

    López-Sánchez, M; Rubio-López, I; Obeso-González, T; Teja-Barbero, J L; Santidrián-Miguel, J P; Peiro-Callizo, E

    2010-10-01

    Macrophage activation syndrome is a form of secondary haemophagocytic lymphohistiocytosis seen in the context of rheumatic diseases. It is seen most frequently in association with systemic onset juvenile arthritis or childhood Still's disease. Hemophagocytosis is part of a sepsis-like clinical syndrome caused by hypercytokinemia due to a highly stimulated but ineffective immune response. Coagulopathy and hemorrhages, decreased white cell count, elevated levels of aspartate aminotransferase, fever, rash, hepatosplenomegaly and central nervous system dysfunction are some of diagnostic criteria of macrophage activation syndrome, but it is very difficult to diagnose due to the lack of specific clinical signs. We report a 8-year-old child who was admitted to the ICU with lethargy, fever, acute respiratory failure, coagulopathy, metabolic acidosis and multiorgan failure. Septic shock was suspected, but he was diagnosed with macrophage activation syndrome and treated with corticosteroids and intravenous immunoglobulin and later discharged from the ICU.

  16. 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.

  17. Exploration of the relationship between the influence of prednison on cell apoptosis of the peripheralblood mononuclear cell in children with primary nephrotic syndrome%激素治疗与小儿肾病综合征外周血单个核细胞凋亡的关系

    Institute of Scientific and Technical Information of China (English)

    刘振翔; 杜华; 黄欣秋; 周微雅

    2003-01-01

    AIM: To explore the influence of prednisone on cell apoptosis of the peripheral blood mononuclear cell (PBMC) in children with primary nephrotic syndrome (PNS).METHODS: According to the results of prednison after treatment,we divided 50 cases into two groups. 37 cases were steroid-responsive and 13 were not responsive. Taking blood from the two groups before and after prednison treatment (in the 2nd,4th,6th and 8th weeks), we calculated the apoptosis rate of PBMC. RESULTS: There was no significant differences in the apoptosis rate.of PBMC between the two groups and the normal control before treatment. The apoptosis rate of PBMC increased obviously after the patients were treated in the 2nd, 4th, 6th weeks,and the highest was in the second week (P<0.05,P<0.01),in comparison with that before the treatment. During the eighth week, the apoptosis rate of PBMC was obviously higher in the steroid-unresponsive than that of steroid-response (P<0.05). CONCLUSIONS: The cell apoptosis may be involved in the regulations during the process of treatment. Some disorders of cell apoptosis may be responsible for the steroid-unresponsiveness in children with nephrotic syndrome.

  18. The metabolic syndrome in survivors of childhood acute lymphoblastic leukemia in Isfahan, Iran

    Science.gov (United States)

    Reisi, Nahid; Azhir, Afshin; Hashemipour, Mahin; Raeissi, Pouran; Amini, Abasgholi; Moafi, Alireza

    2009-01-01

    BACKGROUND: To determine the prevalence of metabolic syndrome in survivors of childhood leukemia in Isfahan, Iran. METHODS: During a 4-year period (2003 to 2007), 55 children (33 male and 22 female) diagnosed with ALL at Unit of Hematology/ Oncology, Department of Pediatrics, Isfahan University of Medical Science, were enrolled in this cross-sectional study. Metabolic syndrome was defined using the modified version of Adult Treatment Panel (ATP III) crite-ria. Insulin resistance was defined based on the homeostasis model assessment index (HOMA-IR). RESULTS: The mean age of participates was 10.4 years (range 6-19 years) and the mean interval since completion of chemotherapy was 35 months. Twenty percent (11/55) of survivors (10 male, 1 female) met criteria for diagnosis of metabolic syndrome. Obesity was observed in one forth of patients and nearly 3/4 of obese patients had metabolic syndrome. High serum insulin levels were found in 16% of participants and in 63% of obese survivors. The mean insulin levels in survivors with metabolic syndrome was three-times more than those without (28.3 mu/l vs. 9.57 mu/l, p = 0.004). Insulin resistance was detected in 72.7% of survivors with metabolic syndrome and it was positively correlated with serum triglycerides (0.543, p ≤ 0.001), systolic and diastolic BP (0.348, p = 0.01 and 0.368, p = 006 respectively), insulin levels (0.914, p < 0.001) and blood sugar (0.398, p = 003). CONCLUSIONS: The prevalence of metabolic syndrome in survivors of childhood leukemia in Iran is higher than developed countries. Nearly all of the obese patients had metabolic syndrome. Weight control and regular physical exercise are recommended to the survivors. PMID:21772869

  19. 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

  20. Epidemiology of childhood leukemia in the presence and absence of Down syndrome.

    Science.gov (United States)

    Mezei, Gabor; Sudan, Madhuri; Izraeli, Shai; Kheifets, Leeka

    2014-10-01

    Down syndrome (DS) is a common congenital anomaly, and children with DS have a substantially higher risk of leukemia. Although understanding of genetic and epigenetic changes of childhood leukemia has improved, the causes of childhood leukemia and the potential role of environmental exposures in leukemogenesis remain largely unknown. Although many epidemiologic studies have examined a variety of environmental exposures, ionizing radiation remains the only generally accepted environmental risk factor for childhood leukemia. Among suspected risk factors, infections, exposure to pesticides, and extremely low frequency magnetic fields are notable. While there are well-defined differences between leukemia in children with and without DS, studies of risk factors for leukemia among DS children are generally consistent with trends seen among non-DS (NDS) children. We provide background on DS epidemiology and review the similarities and differences in biological and epidemiologic features of leukemia in children with and without DS. We propose that both acute lymphoblastic and acute myeloblastic leukemia among DS children can serve as an informative model for development of childhood leukemia. Further, the high rates of leukemia among DS children make it possible to study this disease using a cohort approach, a powerful method that is unfeasible in the general population due to the rarity of childhood leukemia.

  1. Behaviour Problems and Adults with Down Syndrome: Childhood Risk Factors

    Science.gov (United States)

    McCarthy, J.

    2008-01-01

    Background: Studies of people with intellectual disability suggest that several individual characteristics and environmental factors are associated with behaviour disorder. To date there are few studies looking at risk factors within specific syndromes and the relationship between early risk markers and later behaviour disorder. The key aim of the…

  2. Down Syndrome Temperament: The Stereotype at Middle Childhood and Adolescence.

    Science.gov (United States)

    Gunn, Pat; Cuskelly, Monica

    1991-01-01

    Behavioral ratings by mothers and teachers of 94 children with Down's Syndrome (between 8 and 14 years of age) indicated general support for the amiable personality stereotype, but ratings of low persistence were associated with maternal impressions of difficulty. There was little agreement between mothers and teachers regarding individual child…

  3. Childhood allergic bronchopulmonary aspergillosis presenting as a middle lobe syndrome

    OpenAIRE

    Shah, Ashok; Gera, Kamal; Panjabi, Chandramani

    2016-01-01

    Allergic bronchopulmonary aspergillosis (ABPA) is infrequently documented in children with asthma. Although collapse is not uncommon, middle lobe syndrome (MLS) as a presentation of ABPA is rather a rarity. A 9-year-old female child with asthma presented with increase in intensity of symptoms along with a right midzone patchy consolidation on a chest radiograph. In addition, an ill-defined opacity abutting the right cardiac border with loss of cardiac silhouette was noted. A right lateral vie...

  4. Attention in Williams Syndrome and Down's Syndrome: Performance on the New Early Childhood Attention Battery

    Science.gov (United States)

    Breckenridge, Kate; Braddick, Oliver; Anker, Shirley; Woodhouse, Margaret; Atkinson, Janette

    2013-01-01

    Attentional problems are commonly reported as a feature of the behavioural profile in both Williams syndrome (WS) and Down's syndrome (DS). Recent studies have begun to investigate these impairments empirically, acknowledging the need for an approach that considers cross-syndrome comparisons and developmental changes across the different component…

  5. 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。

  6. 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

  7. Age and geader in pathological typing of 1116 children with nephrotic syndrome%儿童肾病综合征病理类型与年龄、性别的关系

    Institute of Scientific and Technical Information of China (English)

    任献国; 刘光陵; 夏正坤; 高远赋; 樊忠民; 何旭; 茅松

    2011-01-01

    目的 探讨肾病综合征患儿的肾脏病理和性别、年龄分期的关系.方法 对1 116例经肾穿刺活检明确肾脏病理的原发性肾病综合征患儿临床资料进行回顾性分析.结果 1 116例患儿中男817例,女299例,男女比例2.73:1;平均年龄(7.3 ± 3.3)岁,其中婴幼儿期90例,学龄前期294例,学龄期409例,青春期323例.肾脏病理轻微病变(MCNS)222例,占19.9%;系膜增生性肾小球肾炎(MsPGN)726例,占65.1%;膜增生性肾小球肾炎(MPGN)55例,占4.9%;膜性肾小球肾炎(MN)27例,占2.4%;局灶节段硬化性肾小球肾炎(FSGS)86例,占7.7%.肾脏病理类型在4个年龄分期的分布差异有统计学意义,MCNS患儿以学龄前期最多,MsPGN患儿以学龄期最多,MPGN和MN患儿以青春期最多,FSGS的患儿以学龄期最多.MCNS患儿的男女比例为5.3:1,MsPGN为2.4:1,MPGN为0.96:1,MN为3.5:1,FSGS为2.7:1,肾脏病理类型在性别上的分布差异有统计学意义(P < 0.05).结论 对于无法开展肾活检的医院或有肾穿刺禁忌证的患儿,可根据年龄和性别结合临床检验初步推断肾脏病理变化的轻重,进一步指导治疗、判断预后.%Objective Renal pathological types of 1116 children suffering from primary nephrotic syndrome from March 1992 to March 2010 in Nanjing General Hospital of Nanjing Military Command were reviewed. Relationship between renal pathology and age or gender in these children was analyzed. Methods Renopuncture was performed to 1 116 children with primary nephrotic syndrome to identify pathological types. According to the age, those children were divided into four groups: infancy, pre-school age, school age and adolescence. Different distribution of renal pathological types among age stage and gender were analyzed. Results Of total 1 116 patients, 817 were boys and 299 were girls (M:F=2.73:1). The mean age was (7.3±3.3) yrs. Ninety cases were infants, 294 cases were pre-school age,409 cases were school age, 323 cases were

  8. [Parsonage-Turner syndrome in childhood and adolescence: case report].

    Science.gov (United States)

    Pérez-de la Cruza, Sagrario

    2012-10-01

    We present the case of a 17-year-old male whose diagnosis is Parsonage-Turner syndrome relapsing in the right arm. In his medical record, he was diagnosed as having amyotrophic neuralgia of the upper limb in three previous occasions. The diagnosis was similar in all episodes, although the affected upper limb was alternating. He was treated in the Rehabilitation Services of two hospitals. At physical examination, in every relapse, he showed acute pain in both, shoulder and arm, and loss of strength in the shoulder blade and the affected upper limb. The aim of his treatment combines the healing of the pain and, together with physiotherapy, fighting against muscular atrophy. The patient evolved favourably in each of the episodes. Nowadays, he does not show any symptom, and he has been discharged from the rehabilitation service.

  9. Angelman syndrome: Mutations influence features in early childhood.

    Science.gov (United States)

    Tan, Wen-Hann; Bacino, Carlos A; Skinner, Steven A; Anselm, Irina; Barbieri-Welge, Rene; Bauer-Carlin, Astrid; Beaudet, Arthur L; Bichell, Terry Jo; Gentile, Jennifer K; Glaze, Daniel G; Horowitz, Lucia T; Kothare, Sanjeev V; Lee, Hye-Seung; Nespeca, Mark P; Peters, Sarika U; Sahoo, Trilochan; Sarco, Dean; Waisbren, Susan E; Bird, Lynne M

    2011-01-01

    Angelman syndrome (AS) is a neurodevelopmental disorder caused by a lack of expression of the maternal copy of UBE3A. Although the "classic" features of AS are well described, few large-scale studies have delineated the clinical features in AS. We present baseline data from 92 children with a molecular diagnosis of AS between 5 and 60 months old who are enrolled in the National Institutes of Health Rare Diseases Clinical Research Network Angelman Syndrome Natural History Study from January 2006 to March 2008. Seventy-four percent of participants had deletions, 14% had either uniparental disomy (UPD) or imprinting defects, and 12% had UBE3A mutations. Participants with UPD/imprinting defects were heavier (P = 0.0002), while those with deletions were lighter, than the general population (P < 0.0001). Twenty out of 92 participants were underweight, all of whom had deletions or UBE3A mutations. Eight out of 92 participants (6/13 (46%) with UPD/imprinting defects and 2/11 (18%) with UBE3A mutations) were obese. Seventy-four out of 92 participants (80%) had absolute or relative microcephaly. No participant was macrocephalic. The most common behavioral findings were mouthing behavior (95%), short attention span (92%), ataxic or broad-based gait (88%), history of sleep difficulties (80%), and fascination with water (75%). Frequent, easily provoked laughter was observed in 60%. Clinical seizures were reported in 65% of participants but all electroencephalograms (EEGs) were abnormal. We conclude that the most characteristic feature of AS is the neurobehavioral phenotype, but specific EEG findings are highly sensitive for AS. Obesity is common among those with UPD/imprinting defects.

  10. Stress and obesity/metabolic syndrome in childhood and adolescence.

    Science.gov (United States)

    Pervanidou, Panagiota; Chrousos, George P

    2011-09-01

    Chronic distress contributes to the development of obesity and comorbid states. Stress is the disturbance of the complex dynamic equilibrium that all organisms must maintain, and is associated with activation of the Stress system comprising of the hypothalamic-pituitary-adrenal axis and the arousal/sympathetic nervous systems. The stress system functions in a baseline circadian fashion and interacts with other systems of the organism to regulate a variety of behavioral, endocrine, metabolic, immune and cardiovascular functions. The experience of perceived or real uncontrollable intense and/or chronic stress (distress) may lead to several psychopathologic conditions, including anxiety, depressive and psychosomatic disorders, substance abuse, obesity and the metabolic syndrome, and osteoporosis, as well as impaired reproductive and immune functions. Developing children and adolescents are particularly vulnerable to the effects of chronic stress. Both behavioral and biological pathways are involved in the connection between chronic stress and obesity in adults and children. Emotional "comfort" eating, lack of sleep, impulsive behaviours and selection of specific foods often characterize stressed individuals. In addition to specific behaviours, dysregulation of the stress system through increased secretion of cortisol and catecholamines, especially in the evening hours, and in concert with concurrently elevated insulin concentrations, leads to development of central obesity, insulin resistance and the metabolic syndrome. In children, chronic alterations in cortisol secretion may have additional effects on cognitive and emotional development, timing of puberty and final stature. Obese children and adolescents are frequently entangled in a vicious cycle between distress, impairing self-image and distorted self-image, maintaining and worsening distress.

  11. The metabolic syndrome in survivors of childhood acute lymphoblastic leukemia in Isfahan, Iran

    Directory of Open Access Journals (Sweden)

    Nahid Reisi

    2009-04-01

    Full Text Available

    • BACKGROUND: To determine the prevalence of metabolic syndrome in survivors of childhood leukemia in Isfahan, Iran.
    • METHODS: During a 4-year period (2003 to 2007, 55 children (33 male and 22 female diagnosed with ALL at Unit of Hematology/ Oncology, Department of Pediatrics, Isfahan University of Medical Science, were enrolled in this crosssectional study. Metabolic syndrome was defined using the modified version of Adult Treatment Panel (ATP III criteria. Insulin resistance was defined based on the homeostasis model assessment index (HOMA-IR.
    • RESULTS: The mean age of participates was 10.4 years (range 6-19 years and the mean interval since completion of chemotherapy was 35 months. Twenty percent (11/55 of survivors (10 male, 1 female met criteria for diagnosis of metabolic syndrome. Obesity was observed in one forth of patients and nearly 3/4 of obese patients had metabolic syndrome. High serum insulin levels were found in 16% of participants and in 63% of obese survivors. The mean insulin levels in survivors with metabolic syndrome was three-times more than those without (28.3 mu/l vs. 9.57 mu/l, p = 0.004. Insulin resistance was detected in 72.7% of survivors with metabolic syndrome and it was  ositively correlated with serum triglycerides (0.543, p < 0.001, systolic and diastolic BP (0.348, p = 0.01 and 0.368, p = 006 respectively, insulin levels (0.914, p < 0.001 and blood sugar (0.398, p = 003.
    • CONCLUSIONS: The prevalence of metabolic syndrome in survivors of childhood leukemia in Iran is higher than developed countries. Nearly all of the obese patients had metabolic syndrome. Weight control and regular physical exercise are recommended to the survivors.
    • KEYWORDS: Acute lymphoblastic leukemia, metabolic syndrome, obesity, children.

  12. Effect observation of application of low molecular heparin in the treatment of children with primary nephrotic syndrome and hypercoagulative state%应用低分子肝素治疗儿童原发性肾病综合征高凝状态的效果观察

    Institute of Scientific and Technical Information of China (English)

    曲春霞

    2015-01-01

    Objective:To explore the treatment effect of low molecular heparin in the treatment of children with primary nephrotic syndrome and hypercoagulative state.Methods:38 patients with primary nephrotic syndrome and hypercoagulative state were selected.They were randomly divided into the control group and the treatment group with 19 cases in each group.The control group was treated with oral dipyridamole,and the treatment group was given subcutaneous injection of low molecular weight heparin on the basis of the control group,then we compared the effect of two groups.Results:At 1 week and 2 weeks after treatment, compared with before treatment,the plasma levels of FB and DD of the two groups were significantly decreased(P<0.05);complete remission time of urinary protein in the treatment group was significantly lower than that of the control group(P<0.05).Conclusion:The treatment effect of low molecular heparin in the treatment of children with primary nephrotic syndrome and hypercoagulative state was significant.%目的:探讨低分子肝素对儿童原发性肾病综合征并发高凝状态时的治疗效果。方法:收治合并高凝状态的原发性肾病综合征患儿38例,随机分为对照组和治疗组,各19例。对照组口服双嘧达莫,治疗组在对照组基础上加皮下注射低分子肝素钙,比较两组效果。结果:治疗1周及2周时两组血浆FB和DD均较治疗前有明显下降(P<0.05),治疗组患儿尿蛋白完全缓解时间明显低于对照组(P<0.05)。结论:低分子肝素对儿童原发性肾病综合征并发高凝状态时的治疗效果显著。

  13. Clinical Pharmacists'Practice in Pharmaceutical Care for One Nephrotic Syndrome Patient with Gastrointestinal Infection%临床药师参与1例肾病综合征并发胃肠道感染患者的药学监护

    Institute of Scientific and Technical Information of China (English)

    唐景财; 阎敏

    2016-01-01

    OBJECTIVE:To explore the role of clinical pharmacists in clinical rational drug use for nephrotic syndrome patients . METHODS: To introduce the treatment process of one nephrotic syndrome patient with gastrointestinal infection participated by the clinical pharmacists .RESULTS & CONCLUSIONS: Proper drugs were selected through the discussion of clinical pharmacists and clinicians , the medication scheme was adjusted according to the patient's conditions and clinical efficacy of drugs , and necessary pharmaceutical care was provided for the patient . Meanwhile , the patient's urinary protein decreased without any complications like thrombus , and the gastrointestinal tract infection had been cured .Therefore , clinical pharmacists‘ participation in the formulation of the related treatment regimen can improve patient's compliance and clinical treatment efficacy and also promote medical quality .The clinical pharmacists play an important role in the rational drug use for nephrotic syndrome patients .%目的:探讨临床药师在肾病综合征患者合理用药中的作用。方法:介绍临床药师参与1例肾病综合征并发胃肠道感染患者的治疗过程。结果与结论:经临床药师与医师讨论并选择合适的药物,根据患者病情变化及药物疗效及时调整用药方案,可为患者提供必要的药学服务。结果,该患者尿蛋白减少,未出现血栓等并发症,胃肠道感染治愈。由此表明,临床药师参与患者有关治疗方案的制订,可提高患者的用药依从性和临床治疗效果,医疗质量提升,临床药师在肾病综合征患者合理用药中发挥了重要作用。

  14. Treatment of primary nephrotic syndrome with glucocorticoid and alfacalcidol%泼尼松联合阿法骨化醇治疗原发性肾病综合征的临床观察

    Institute of Scientific and Technical Information of China (English)

    徐鹏; 张骅; 陈星华

    2011-01-01

    目的 观察泼尼松联合阿法骨化醇治疗原发性肾病综合征的临床疗效.方法 选择2008年1月-2009年10月住院及门诊治疗的原发性肾病综合征患者56例,随机分为2组:C组28例常规泼尼松治疗,T组28例常规泼尼松联合阿法骨化醇(0.5 μg/d)治疗,比较2组治疗前后临床症状的改善,血清钙、磷、甲状旁腺激素(PTH)、25-(OH)2D3以及1,25-(OH)2D3水平以及尿蛋白定量变化.结果 治疗3、6及12个月后,T组缓解率均明显高于C组(P<0.05).治疗6个月,C组患者24 h尿蛋白下降(P<0.05),血钙、血磷、血25-(OH)2D3及1,25-(OH)2D3、PTH水平稍有改善,但差异无统计学意义(P﹥0.05);T组血钙、25-(OH)2D3及1,25-(OH)2D3水平上升(P<0.05),24 h尿蛋白、PTH水平下降(P<0.05),且与C组治疗后相比,差异具有统计学意义(P<0.05),而血磷水平无明显变化(P>0.05).结论 泼尼松联合阿法骨化醇治疗原发性肾病综合征疗效明显.%Objective To investigate the effect of prednisone combined with alfacalcidol in patients with primary nephrotic syndrome( PNS ).Methods 56 hospitalized patients of PNS were chosen from January, 2008 to October, 2009.They were randomly allocated into two groups: 28 patients ( Group C ) were treated with regular prednisone, and other 28 patients ( Group T ) were treated with regular prednisone combined with alfacalcidol ( 0.5 μg/d ).The level of serum calcium, phosphonium, parathyroid hormone ( PTH ), 25-( OH )2 D3, 1,25-( OH )2 D3 and proteinuria were compared before and after the therapy in each group.Results 3 months, 6 months and 12 months after treatment, the effect in T group was better than C group( P < 0.05 ).The levels of calcium,25-( OH )2 D3 and 1,25-( OH )2 D3 were decreased( all P < 0.05 ), the level of PTH was increased in Group C and Group T.After prednisone treatment, the above parameters did not change obviously in Group C.But with the treatment of prednisone combined with alfacalcidol, the levels

  15. Childhood allergic bronchopulmonary aspergillosis presenting as a middle lobe syndrome.

    Science.gov (United States)

    Shah, Ashok; Gera, Kamal; Panjabi, Chandramani

    2016-01-01

    Allergic bronchopulmonary aspergillosis (ABPA) is infrequently documented in children with asthma. Although collapse is not uncommon, middle lobe syndrome (MLS) as a presentation of ABPA is rather a rarity. A 9-year-old female child with asthma presented with increase in intensity of symptoms along with a right midzone patchy consolidation on a chest radiograph. In addition, an ill-defined opacity abutting the right cardiac border with loss of cardiac silhouette was noted. A right lateral view confirmed a MLS, which was further corroborated by high resolution computed tomography. Central bronchiectasis was also observed, which prompted a work-up for ABPA. The child met 7/8 major diagnostic criteria for ABPA. She was then initiated on oral prednisolone that resulted in a marked clinical improvement within a fortnight. Radiological clearance occurred at 3 months with inflation of the middle lobe. ABPA presenting with MLS in a child is yet to be reported. A high index of suspicion is required to establish the diagnosis of ABPA in a child presenting with MLS. This would obviate the invasive investigations usually done to ascertain the cause of MLS.

  16. Genetic susceptibility to obesity and metabolic syndrome in childhood

    Directory of Open Access Journals (Sweden)

    Concepción M. Aguilera

    2013-09-01

    Full Text Available Obesity is one of the major public health problems worldwide. It is a chronic, complex, and multifactorial-origin disease characterised by body fat excess mainly due to an imbalance between dietary intake and energy expenditure. One of the major complications of obesity is metabolic syndrome, which comprises anthropometrical, clinical, and metabolic dysfunctions that predispose the affected individual to the development of type 2 diabetes mellitus and cardiovascular diseases. It is hypothesised that the variability in the susceptibility to obesity-mediated metabolic complications involves both environmental and genetic factors. Whereas advances in the knowledge of the variations in the human genome have led to the identification of susceptibility genes that contribute to obesity and related disorders, relatively few studies have specifically focused on the interactions between obesity and genetic polymorphisms and the development of metabolic complications. Despite these limited efforts, an increasing amount of evidence suggests that the effects of some gene variants on metabolic traits are modified by or present only in the setting of obesity. Furthermore, some of these loci may have larger effects on metabolic phenotypes in the presence of certain dietary or lifestyle factors. In the present manuscript, we reviewed the genes and their variants that have been evidenced to play a role in obesity-associated metabolic complications through genetic association studies, including candidate gene and genome-wide association approaches in adults and children.

  17. Genetic susceptibility to obesity and metabolic syndrome in childhood.

    Science.gov (United States)

    Aguilera, Concepción M; Olza, Josune; Gil, Angel

    2013-09-01

    Obesity is one of the major public health problems worldwide. It is a chronic, complex, and multifactorial origin disease characterised by body fat excess mainly due to an imbalance between dietary intake and energy expenditure. One of the major complications of obesity is metabolic syndrome, which comprises anthropometrical, clinical, and metabolic dysfunctions that predispose the affected individual to the development of type 2 diabetes mellitus and cardiovascular diseases. It is hypothesised that the variability in the susceptibility to obesity-mediated metabolic complications involves both environmental and genetic factors. Whereas advances in the knowledge of the variations in the human genome have led to the identification of susceptibility genes that contribute to obesity and related disorders, relatively few studies have specifically focused on the interactions between obesity and genetic polymorphisms and the development of metabolic complications. Despite these limited efforts, an increasing amount of evidence suggests that the effects of some gene variants on metabolic traits are modified by or present only in the setting of obesity. Furthermore, some of these loci may have larger effects on metabolic phenotypes in the presence of certain dietary or lifestyle factors. In the present manuscript, we reviewed the genes and their variants that have been evidenced to play a role in obesity-associated metabolic complications through genetic association studies, including candidate gene and genome-wide association approaches in adults and children.

  18. Neurologic complications of cerebral angiography in childhood moyamoya syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Robertson, R.L.; Chavali, R.V.; Robson, C.D.; Barnes, P.D.; Burrows, P.E. [Department of Radiology, Children`s Hospital Medical Center, Boston, MA (United States); Eldredge, E.A. [Department of Anesthesia, Children`s Hospital Medical Center and Harvard Medical School, Boston, MA (United States); Scott, R.M. [Department of Neurosurgery, Children`s Hospital Medical Center and Harvard Medical School, Boston, MA (United States)

    1998-11-01

    Purpose. To determine the incidence of neurologic complications of cerebral angiography in children with moyamoya syndrome (MMS) as compared to children without MMS. Materials and methods. One-hundred-ninety consecutive cerebral angiograms obtained in 152 children were evaluated. Sixty of these angiograms were obtained in 40 children with MMS. Patients underwent neurologic evaluation prior to and after the procedure. For this study, a neurologic complication was defined as any new focal neurologic deficit or alteration in mental status occurring during the procedure or within the ensuing 24 hours. Results. There were 2 neurologic complications within 24 hours of angiography, one in the MMS group and one in the non-MMS group. One patient with MMS became mute following angiography. The symptom resolved within 12 hours. One patient without MMS being examined postoperatively for residual arteriovenous malformation developed intracranial hemorrhage requiring reexploration 12 hours after the angiogram. Using a two-tail Fisher`s exact test, there was no significant statistical difference in the ischemic (P = 0.3) or hemorrhagic (P = 1.0) complication rates between the group of patients with MMS and the non-MMS groups. Conclusion. The risk of a neurologic complication from cerebral angiography in children with MMS is low and not statistically different from the risk in children with other cerebrovascular disorders. (orig.) With 8 tabs., 37 refs.

  19. 儿童原发性肾病综合征并发尿路感染的临床分析%Primary Nephrotic Syndrome Complicated by Urinary Tract Infection in Children

    Institute of Scientific and Technical Information of China (English)

    陈文波; 许文峰; 苏永智

    2014-01-01

    目的:研究小儿原发性肾病综合征(primary nephrotic syndrome,PNS)并发尿路感染(urinary tract infec-tion,UTI)的临床特征,分析其危险因素和对抗生素的敏感情况,为儿童 PNS 并发 UTI 的诊断和治疗提供参考依据。方法收集62例 PNS 并发 UTI 患儿(UTI 组)的临床资料,分析其实验数据特点,并与同期60例符合 PNS诊断标准的非 UTI 患儿(非 UTI 组)进行对比。结果62例并发 UTI 的 PNS 患儿中,有症状者14例(占22.5%),无症状性菌尿者48例(占77.5%)。UTI 组患儿24 h 尿蛋白定量、血肌酐(Scr)等指标水平均显著高于非 UTI 组(P <0.01),而血浆清蛋白(Alb)、免疫球蛋白 IgG(IgG)等指标水平均显著低于非 UTI 组(均 P <0.01);UTI 组患儿中激素联合免疫抑制剂治疗患儿28例(45.1%),显著高于非 UTI 组[13例(21.6%)](P <0.05)。致病菌以革兰阴性杆菌为主(75.00%),大肠杆菌为优势菌群,其他依次为肺炎克雷伯杆菌、变形杆菌等,氨苄青霉素、哌拉西林等抗生素已被大多数病原菌耐受。结论PNS 合并 UTI 临床过程隐匿,大量蛋白尿、低 IgG 血症、低 Alb 水平、肾功能不全、使用免疫抑制剂是导致 PNS 易发生 UTI 的因素。应对并发 UTI 的易感因素进行早期干预,治疗时应根据病原学和药敏结果用药。%Objective To study the clinical features,risk factors and sensitivity to antibiotics of primary nephrotic syndrome(PNS)complicated by urinary tract infection(UTI)in children,and to provide reference basis for the diagnosis and treatment of PNS complicated by UTI.Methods Clinical data of 62 children with PNS and UTI(UTI group)were collected to analyze the labora-tory features,and were compared with data of 60 non-UTI children who met diagnostic criteria for PNS(non-UTI group).Results Among the 62 children with PNS and UTI,14 (22

  20. A CLINICAL STUDY TO EVALUATE THE ROLE OF AKSHITARPANA, SHIRODHARA AND AN AYURVEDIC COMPOUND IN CHILDHOOD COMPUTER VISION SYNDROME

    Directory of Open Access Journals (Sweden)

    Singh Omendra Pal

    2011-03-01

    Full Text Available Computer vision syndrome is one among the lifestyle disorders in children. About 88% of people who use computers everyday suffer from this problem and children are no exception. Computer Vision Syndrome (CVS is the complex of eye and vision problems related to near works which are experienced during the use of Video Display Terminals (TV and computers. Therefore, considering these prospects a randomized double blind placebo control study was conducted among 40 clinically diagnosed children (5-15 years age group of computer Vision Syndrome to evaluate the role of akshitarpana, shirodhara and an ayurvedic compound in childhood computer vision syndrome.

  1. 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

  2. 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。

  3. Molecular study of idiopathic nephrotic syndrome

    OpenAIRE

    Bullich Vilanova, Gemma

    2016-01-01

    Aquesta tesi és una contribució al coneixement de les bases moleculars de la síndrome nefròtica idiopàtica concretament, la nefropatia membranosa idiopàtica i la síndrome nefròtica córtico-resistent (SNCR) o glomeruloesclerosis segmentària i focal (GESF). La primera part d'aquesta tesi presenta l'associació de determinades variants genètiques tan amb el risc de desenvolupar nefropatia membranosa idiopàtica com amb el curs clínic de la malaltia, en població espanyola. Els nostres resultats mos...

  4. Epidemiology of childhood Guillain-Barré syndrome as a cause of acute flaccid paralysis in Honduras: 1989-1999.

    Science.gov (United States)

    Molinero, Marco R; Varon, Daniel; Holden, Kenton R; Sladky, John T; Molina, Ida B; Cleaves, Francisco

    2003-11-01

    The objective of this study was to investigate the incidence of acute flaccid paralysis in the pediatric population of Honduras over an 11-year period, determine what percentage of acute flaccid paralysis was Guillain-Barré syndrome, and identify the epidemiologic features of Guillain-Barré syndrome. There were 546 childhood cases of acute flaccid paralysis seen between January 1989 and December 1999 at the Hospital Escuela Materno-Infantil in Tegucigalpa, Honduras. Of these cases with acute flaccid paralysis, 394 (72.2%) were diagnosed with Guillain-Barré syndrome. Our incidence of Guillain-Barré syndrome in the Honduran pediatric population (1.37/100,000 per year) is higher than that shown in other studies. There was a significantly higher incidence of Guillain-Barré syndrome in younger children (ages 1-4 years), a significant preponderance of cases from rural areas, and a mild predominance in boys but a typical clinical presentation. The Honduran pediatric Guillain-Barré syndrome population had an increased mortality rate. Guillain-Barré syndrome has become the leading cause of childhood paralysis in Honduras. A better understanding of the population at highest risk and opportunities for earlier intervention with more effective therapeutic modalities may permit reducing the mortality among Honduran children who develop Guillain-Barré syndrome.

  5. 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

  6. Gitelman syndrome manifesting in early childhood and leading to delayed puberty: a case report

    Directory of Open Access Journals (Sweden)

    Raza Farhan

    2012-10-01

    Full Text Available Abstract Introduction Gitelman syndrome is an inherited autosomal recessive renal salt-wasting disorder. It presents with variable clinical symptoms including muscle weakness and fatigue, and the diagnosis is based on metabolic alkalosis, hypokalemia, hypomagnesemia and hypocalciuria. It is usually diagnosed incidentally in early adulthood. There are rare cases of Gitelman syndrome presenting in early childhood; however, to the best of our knowledge it has not previously been associated with delayed puberty. Case presentation A 17-year-old South Asian man with recurrent episodes of generalized muscle weakness, fatigue and cramps from the age of two years was admitted for further workup. Before the age of 12 years, the episodes had been mild, but they then got progressively worse. Other symptoms include polyuria, polydipsia, nocturia, paresthesia and occasional watery diarrhea. He also had a history of short stature, poor weight gain and delayed developmental landmarks. His family history was unremarkable except for the consanguineous marriage of his parents. An examination revealed a thin and lean man with blood pressure of 95/60mmHg. His height and weight were below the third percentile and his sexual development was at Tanner Stage II. Laboratory work revealed serum sodium of 124mmol/L, potassium 2.4mmol/L, calcium 6.5mmol/L and magnesium of 1.2mg/dL. His testosterone level was low (0.85ng/mL, normal for his age 2.67 to 10.12ng/mL with normal levels of luteinizing hormone and follicle-stimulating hormone. The sex hormone findings were attributed to delayed puberty. A 24-hour urinary analysis revealed decreased excretion of calcium (25.9mg/24 hours. Based on the findings of hypokalemic metabolic alkalosis without hypertension, severe hypomagnesemia and hypocalciuria, a diagnosis of Gitelman syndrome was made. Treatment was started with oral supplementation of potassium, magnesium and calcium along with spironolactone and liberal salt intake

  7. Complex karyotype newly defined: the strongest prognostic factor in advanced childhood myelodysplastic syndrome.

    Science.gov (United States)

    Göhring, Gudrun; Michalova, Kyra; Beverloo, H Berna; Betts, David; Harbott, Jochen; Haas, Oskar A; Kerndrup, Gitte; Sainati, Laura; Bergstraesser, Eva; Hasle, Henrik; Stary, Jan; Trebo, Monika; van den Heuvel-Eibrink, Marry M; Zecca, Marco; van Wering, Elisabeth R; Fischer, Alexandra; Noellke, Peter; Strahm, Brigitte; Locatelli, Franco; Niemeyer, Charlotte M; Schlegelberger, Brigitte

    2010-11-11

    To identify cytogenetic risk factors predicting outcome in children with advanced myelodysplastic syndrome, overall survival of 192 children prospectively enrolled in European Working Group of Myelodysplastic Syndrome in Childhood studies was evaluated with regard to karyotypic complexity. Structurally complex constitutes a new definition of complex karyotype characterized by more than or equal to 3 chromosomal aberrations, including at least one structural aberration. Five-year overall survival in patients with more than or equal to 3 clonal aberrations, which were not structurally complex, did not differ from that observed in patients with normal karyotype. Cox regression analysis revealed the presence of a monosomal and structurally complex karyotype to be strongly associated with poor prognosis (hazard ratio = 4.6, P < .01). Notably, a structurally complex karyotype without a monosomy was associated with a very short 2-year overall survival probability of only 14% (hazard ratio = 14.5; P < .01). The presence of a structurally complex karyotype was the strongest independent prognostic marker predicting poor outcome in children with advanced myelodysplastic syndrome.

  8. Analysis of frequent relapses related factors of primary nephrotic syndrome in children%儿童原发性肾病综合征频反复相关因素分析

    Institute of Scientific and Technical Information of China (English)

    范树颖; 张碧丽; 王文红; 张暄

    2011-01-01

    目的 通过对原发性肾病综合征(PNS)患儿临床资料统计分析,探讨小儿PNS频反复的影响因素.方法 收集2007年1月至2010年2月于我科诊断为PNS的患儿住院及门诊随访病历,对获得的完整临床资料进行分析.结果 245例激素敏感型肾病综合征患儿中,非频反复202例(82.4%),频反复43例(17.6%).单因素分析结果中,首次反复距初治缓解时间<3个月、起病时血总蛋白、胆固醇水平、血清IgE水平在频反复及非频反复两组间差异有统计学意义(P<0.05).多因素非条件logistic回归分析中,首次反复距初治缓解时间<3个月及血清IgE水平升高(>358IU/ml)为频反复的危险因素(P<0.05).引起反复的诱因中,以上呼吸道感染最多见,共194次(65.1%),有诱因的反复发生在1~3月份最多,占30.4%.结论 频反复在正规激素治疗的PNS中所占比例达17.6%,存在冬春季复发高峰的季节性特点.起病时血总蛋白<40g/L、血胆固醇水平>10mmol/L可作为预测PNS易出现频反复的指标.感染是肾病综合征反复的主要诱因,不规则治疗、特应性体质导致反复亦应引起重视.%Objective To explore the influencing factors of frequent relapse primary nephritic syndrome (PNS) in children according to the statistical analysis of their clinical data and laboratory examinations. Method We collected in-hospital and out-patient medical records of children who were diagnosed PNS from Jan. 2007 to Feb. 2010 and whole clinical data were analyzed. Results In 245 cases of children with steroid sensitine nephrotic syndrome, non-frequent replapse (NFR) accounted for 82.4% and frequent relapse (FR) accounted for 17.6%. In univariate analysis, period of first relapse between initial treatment remission within 3 months , level of serum total protein and cholesterol and IgE at onset had statistical significance in FR children and NFR children, which were significant predictors of FR. The logistic

  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. 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

  12. 80例原发性肾病综合征合并甲状腺功能减退的临床和病理分析%Clinical and Pathological Analysis for 80 Cases of Primary Nephrotic Syndrome Complicating with Hypothyroidism

    Institute of Scientific and Technical Information of China (English)

    陈晓风; 李晶; 谷小丽; 王攀

    2016-01-01

    Objective: To discuss the clinical significance of 80 patients’clinical characteristics and pathological types who have primary nephrotic syndrome complicating with hypothyroidism.Method:80 patients of primary nephrotic syndrome complicating with hypothyroidism in our hospital were selected,they all received kidney biopsy and thyroid function test(TT3,TT4,FT3,FT4 and TSH),plasma albumin,24 hours urine protein quantitative and so on,and then the pathological types and clinical characteristics were analyzed.Result:The pathological types were membranous glomerulonephritis (MGN) of 43 cases, minimal change disease (MCD) of 29 cases, mesangial proliferative glomerulonephritis (MSPGN) of 4 cases,focal segmental glomerulosclerosis (FSGS) of 2 cases, and membranoproliferative glomerulonephritis (MPGN) of 2 cases. For patients with primary nephrotic syndrome,their serum albumin had positive correlation with serum TT3, FT3,TT4,FT4, and had negative correlation with serum TSH;24 hours urine protein quantitative had negative correlation with serum TT3,FT3,TT4,FT4,and had no obvious correlation with serum TSH.Conclusion:For patients of primary nephrotic syndrome complicating with hypothyroidism,the pathological types are membranous glomerulonephritis and minimal change disease as the common.The level of thyroxin and albumin decrease have a close correlation.So, the test for thyroxin has an important reference value to judge the lesion degree,prognosis and pathological types for patients with primary nephrotic syndrome complicating with hypothyroidism.%目的:对80例原发性肾病综合征合并甲状腺功能减退患者的临床特点和病理类型进行分析,探讨其临床意义。方法:本院住院的80例原发性肾病综合征合并甲状腺功能减退患者均进行肾脏病理穿刺、检测甲状腺功能(TT3、TT4、FT3、FT4及TSH)、血浆白蛋白、24 h尿蛋白定量等,并对其病理类型及临床特点进行分析。结果:肾脏病理

  13. A case of childhood stiff-person syndrome with striatal lesions: a possible entity distinct from the classical adult form.

    Science.gov (United States)

    Sanefuji, Masafumi; Torisu, Hiroyuki; Kira, Ryutaro; Yamashita, Hiroshi; Ejima, Kazuna; Shigeto, Hiroshi; Takada, Yui; Yoshida, Keiko; Hara, Toshiro

    2013-06-01

    Parainfectious or autoimmune striatal lesions have been repeatedly described in children. We report a 7-year-old girl with painful muscle spasms, leading to the diagnosis of childhood stiff-person syndrome (SPS). Striatal lesions were demonstrated by diffusion-weighted magnetic resonance imaging (MRI) and single-photon emission computed tomography but not by conventional MRI. Autoantibodies against glutamic acid decarboxylase (GAD) were absent. Steroid pulse therapy and high-dose intravenous immunoglobulin resolved all the symptoms with slight sequelae. Childhood SPS may be characterized by absent anti-GAD antibodies and a transient benign clinical course, and it may have a pathomechanism distinct from that in adult SPS.

  14. 小剂量多巴胺联合酚妥拉明治疗原发性肾病综合征中重度水肿%Treatment of primary nephrotic syndrome in children with severe edema by phentolamine associated with low-dose dopamine

    Institute of Scientific and Technical Information of China (English)

    银宏伟

    2015-01-01

    Objective To investigate the clinical effects of application of low-dose dopamine combined with phentolamine on primary nephritic syndrome in children with severe edema.Methods One hundred cases of children with primary nephrotic syndrome were randomly divided into observation group and control group,with 50 cases in each group,low-dose dopamine combined phentolamine treatment were used in the observation group; furosemide was used in the control group.The urine volume,edema subsided,fade time and incidence of adverse reactions of the two groups were compared.Results Urine of children in the observation group was significantly higher than that in the control group,edema subsided significantly shorter than that in the control group,the incidence of adverse events was significantly lower than that in the control group,the differences between the two groups were statistically significant (P < 0.05).Conclusions Treatment of primary nephrotic syndrome in children with severe edema by phentolamine associated with low-dose dopamine,can increase urine volume,rapidly swelled and with low side effect,the clinical results are good,should be widely applied.%目的 探讨小剂量多巴胺联合酚妥拉明对原发性肾病综合征中重度水肿的临床疗效.方法 将100例原发性肾病综合征患者随机分为观察组和对照组,每组50例,观察组采用小剂量多巴胺与酚妥拉明联合治疗,对照组采用呋塞米治疗,观察对比两组患者尿量、水肿消退程度、消退时间及不良反应发生率.结果 观察组尿量显著多于对照组,患儿水肿的消退时间显著短于对照组,不良反应发生率显著低于对照组,两组比较差异有统计学意义(P<0.05).结论 采用小剂量多巴胺联合酚妥拉明治疗原发性肾病综合征患儿中重度水肿可增加尿量,迅速消肿且不良反应小,临床效果良好,值得临床推广应用.

  15. 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

  16. 肾病综合征的疾病构成:单中心临床研究%The spectrum of nephrotic syndrome from a single-center clinical research

    Institute of Scientific and Technical Information of China (English)

    史晓虎; 李文歌; 杨冠男; 邹古明; 芦建华

    2012-01-01

    目的:探讨肾病综合征(NS)的疾病构成,为临床诊治提供数据.方法:调研中日友好医院肾内科2009年1月~2010年12月住院患者中全部NS患者的临床资料.结果:2年中在住院NS患者共282例,其中男158例,女性124例;患者年龄14~80岁,平均42.5±17.4岁;青年109例,中年119例,老年54例.282例NS患者中,伴高血压、糖尿病、慢性肾功能衰竭、急性衰竭者分别为87例、21例、31例、14例.其中184例进行了肾穿刺活检,青年患者(86例)肾脏病理前3位疾病是:局灶节段性肾小球硬化症(FSGS)24例、微小病变性肾病(MCD)21例、IgA肾病(IgAN)21例;中年患者(81例)肾脏病理前4位疾病分别是:原发性膜性肾病(MN)25例、FSGS 13例、继发性膜性肾病(SMN)12例、MCD 10例;老年患者(17例)肾脏病理前2位是:MN 9例、FSGS3例.结论:NS患者以中青年为主,常合并高血压、糖尿病、肾功能衰竭.青年患者肾脏病理类型以FSGS、MCD和IgAN肾病为主;中年患者以MN、FSGS和SMN为主;老年患者以MN和FSGS为主.%Objective:To investigate the constitute of nephrotic syndrome(NS)to provide data for clinical diag-nosis and treatment.Methods: A total of 282 cases in China-Japan Friendship Hospital from Jan.2009 to Dec. 2010 were enrolled in this prospective study .Their clinical and pathological datas were prospectively analyzed. Results:During this period,there were 282 cases of NS,accounts for 17.2% in total hospitalization.There were 158 males and 124 females,half of them were from cities,The average age of NS patients was 42.5±17.4 years old-Among them, 109 cases were youth(=35 yr),119 cases were middle-aged(36~59yr)and 54 cases the elder (=60yr),Eighty seven of patients with malignant hypertension and 21 cases were diagnosed with diabetes, 31 and 14 cases with chronic renal failure and acute renal failure, respectively .There were 184 cases accepted renal pathological biopsy.There were 86 cases in the youth group

  17. Transcranial Direct Current Stimulation for Treatment of Childhood Pharmacoresistant Lennox-Gastaut Syndrome; A Pilot Study

    Directory of Open Access Journals (Sweden)

    Narong eAuvichayapat

    2016-05-01

    Full Text Available Background: Lennox-Gastaut syndrome (LGS is a severe childhood epileptic syndrome with high pharmacoresistance. The treatment outcomes are still unsatisfied. Our previous study of cathodal transcranial direct current stimulation (tDCS in children with focal epilepsy showed significant reduction in epileptiform discharges. We hypothesized that cathodal tDCS when applied over the primary motor cortex (M1 combined with pharmacologic treatment will be more effective for reducing seizure frequency in patients with LGS than pharmacologic treatment alone. Material and Method: Study participants were randomized to receive either (1 pharmacologic treatment with 5-consecutive days of 2 mA cathodal tDCS over M1 for 20 min or (2 pharmacologic treatment plus sham tDCS. Measures of seizure frequency and epileptic discharges were performed before treatment and again immediately post-treatment and 1-, 2-, 3-, and 4-week follow-up. Result: Twenty two patients with LGS were enrolled. Participants assigned to the active tDCS condition reported significantly more pre- to post-treatment reductions in seizure frequency and epileptic discharges that were sustained for 3 weeks after treatment.Conclusion: Five consecutive days of cathodal tDCS over M1 combined with pharmacologic treatment appears to reduce seizure frequency and epileptic discharges. Further studies of the potential mechanisms of tDCS in the LGS are warranted.Trial Registration: ClinicalTrials.gov, NCT02731300 (https://register.clinicaltrials.gov.

  18. Minimal-change disease as a paraneoplastic syndrome in a patient with ovarian carcinoma.

    Science.gov (United States)

    González-Fontal, Guido R; Restrepo, Juan G; Henao-Martínez, Andrés F

    2011-12-01

    Minimal-change disease (MCD) is an exceptional paraneoplastic presentation. We are describing the case of an ovarian paraneoplastic nephrotic syndrome. The kidney biopsy was consistent with MCD. Steroids and immunosuppressive therapy were given with no change in the nephrotic-range proteinuria. A complete resolution of the nephrotic syndrome was soon observed with improvement of her clinical condition after five cycles of chemotherapy with paclitaxel and carboplatin and tumor-debulking surgery. Ovarian carcinoma paraneoplastic nephrotic syndrome secondary to MCD is an extremely rare event, which is important to recognize since it is responsive to the standard chemotherapy.

  19. 常规剂量甲泼尼龙、泼尼松序贯疗法在原发性肾病综合征中的疗效分析%Analysis of therapeutic effect of sequential therapy in primary nephrotic syndrome with regular dose methylprednisolone and prednisone

    Institute of Scientific and Technical Information of China (English)

    李晓勇; 杜娜; 张杰; 王玉路; 李方晓; 李斌

    2013-01-01

    Objective To analyze the efficacy of regular dose methylprednisolone , prednisone of sequential therapy in primary nephrotic syndrome .Methods 80 patients with primary nephrotic syndrome were divided into two groups according to pathological type , glucocorticoids group ( A) , and another group ( B) with glucocorticoid and cytotoxic drugs . Then each group was randomly divided into two groups , namely , the application of methylprednisolone group ( treatment group) and oral prednisone group ( control group) , and made a curative effect analysis .Results There was no statistical difference in the remission rate of the two groups between regular dose methylprednisolone , prednisone sequential therapy and regular prednisone therapy (P > 0 .05 ) , but the effect of the 24 - hour urinary protein excretion , serum albumin , cholesterol , edema and urinary protein clearance time in treatment group was superior to those in the control group , There was no significant statistical difference (P < 0 .05 ) .Conclusion The treatment of the regular dose of methylprednisolone , prednisone sequential therapy can more quickly relieve the patient's symptom than regular prednisone application in primary nephrotic syndrome .%目的:分析常规剂量甲泼尼龙、泼尼松序贯疗法在原发性肾病综合征中的疗效。方法80例原发性肾病综合征患者依病理类型分为2组,A 组应用糖皮质激素,B 组为应用糖皮质激素加细胞毒类药物。再将上2组各随机分为2组,即甲泼尼龙组(治疗组)、口服泼尼松组(对照组),进行疗效分析。结果常规剂量甲泼尼龙、泼尼松序贯疗法较常规泼尼松疗法在病情缓解率方面2组差异无统计学意义(P >0.05),但在24 h 尿蛋白定量、血浆白蛋白、胆固醇、水肿消退时间、尿蛋白转阴时间等方面均优于对照组,差异有统计学意义(P <0.05)。结论常规剂量甲泼尼龙、泼尼松序贯疗法在原发

  20. 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

  1. 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.

  2. 甲泼尼龙与泼尼松治疗肾病综合征合并乙型肝炎的疗效及安全性比较%The comparison of the efficacy and safety of methylprednisolone and prednisone in the treatment of nephrotic syndrome complicated with hepatitis B

    Institute of Scientific and Technical Information of China (English)

    王敬

    2012-01-01

    目的 比较甲泼尼龙与泼尼松治疗肾病综合征合并乙型肝炎的临床疗效及安全性.方法 选取肾病综合征合并乙型肝炎患者66例,随机分为对照组和观察组,每组33例.对照组于清晨顿服泼尼松,观察组顿服甲泼尼龙.比较两组患者的临床疗效及肝损害不良反应的发生情况.结果 治疗后两组患者的各指标均比治疗前明显改善(均P<0.05);观察组的总有效率(93.94%)高于对照组(84.85%)(x2=1.44,P>0.05);观察组HBV-DNA转阴率显著高于对照组(x2=4.69,P<0.05);观察组患者肝损害作用显著低于对照组(t =5.62,5.13,P<0.05).结论 甲泼尼龙与泼尼松均能有较治疗肾病综合征合并乙型肝炎,但甲泼尼龙的效果优于泼尼松,对患者肝损害作用较轻.%Objective To compare the clinical efficacy and side effects of liver damage of the methylprednisolone (methylprednisolone tablets ) and prednisone treating nephrotic syndrome compicated with hepatitis B.Methods 66 patients with nephrotic syndrome complicated with hepatitis B were randomly divided into control group and observation group,and each group had 33 cases.The control group was given prednisone in the morning,while the observation group was received methylprednisolone.The clinical efficacy and side effects of liver damage were compared between the two groups.Results After the treatment,the biochemical index significantly improved compared with the pre-treatment( all P < 0.05 ) ;The total effective rate of observation group was 93.94% higher than 84.85% of the control group( x2 =1.44,P > 0.05 ).However,there was no significant difference between the two groups( P >0.05) ;The observation group was significantly higher than the control group in negative rate of HBV-DNA (x2 =4.69,P < 0.05 ) ;The side effects of liver damage in the observation group were lower than the control group (t =5.62,5.13,P < 0.05 ).Conclusion Methylprednisolone and prednisone for

  3. Clinical Observation of Sequential Therapy with Dexamethasone and Prednisone in Treating Adult Primary Nephrotic Syndrome%地塞米松泼尼松序贯疗法治疗成人原发性肾病综合征临床观察

    Institute of Scientific and Technical Information of China (English)

    张万超; 蒲月英; 王程; 左松; 欧阳治

    2013-01-01

    Objective To explore the effect of sequential therapy using dexamethasone and prednisone in treating a-dult primary nephrotic syndrome. Methods 64 patients suffered with primary nephrotic syndrome were randomly divided into 3 groups, which were treated respectively by sequential therapy with dexamethasone and prednisone (observational group, 22 cases), standard treatment with prednisone administrated orally (control group A, 21 cases), and sequential therapy with methylprednisolone and prednisone (control group B, 21 cases). Regression time of edema, disappearing time of urine protein, relieving time of the symptoms, therapeutic effect, as well as incidence of adverse effects were recorded and compared between the different groups. Results Regression time of edema and disappearing time of urine protein of observational group were significantly less than those of control group A (P0. 05). Therapeutic effects between the 3 groups exhibited no statistical differences (P>0. 05). As for relieving time of the symptoms, it was statistically shorter for observational group as compared with control group A (P0, 05), And no differences in incidence of adverse effects were found among the three groups. Conclusion As compared with standard prednisone treatment in treating primary nephrotic syndrome, sequential therapy with dexamethasone and prednisone can induce relieving the symptoms promptly, shortening regression time of edema as well as disappearing time of urine protein. At the same time, such a treatment method exhibited less water sodium retention effects and is much cheaper than sequential therapy with methylprednisolone and prednisone, thus is worthy of spreading its use in primary hospitals.%目的 探讨地塞米松、泼尼松序贯疗法治疗成人原发性肾病综合征的疗效.方法 64例原发性肾病综合征患者随机分成地塞米松、泼尼松序贯疗法组(观察组)22例、口服泼尼松标准疗法组(对照组A)21例和甲泼尼龙、泼尼

  4. Efficacy and impact on urinary protein of mycophenolate mofetil on primary nephrotic syndrome%霉酚酸酯治疗原发性肾病综合征的临床疗效及对尿蛋白的影响

    Institute of Scientific and Technical Information of China (English)

    崔玉波

    2014-01-01

    目的 研究霉酚酸酯(MMF)治疗原发性肾病综合征的疗效及对尿蛋白的影响.方法 选取原发性肾病综合征患者136例,采用随机数字表法分为MMF组和对照组各68例,对照组行常规治疗,MMF组加用霉酚酸酯治疗.两组均连续治疗6个月后进行疗效评价.结果 MMF组总缓解率(82.8%)显著高于对照组(60.6%)(x2=10.86,P<0.05);两组BUN、血肌酐(Scr)、24h尿蛋白定量均有不同程度降低,MMF组降低更为显著,各项差异具有统计学意义(t =2.50、4.59、11.12,均P<0.05).两组血清白蛋白均上升,但MMF组升高更明显,差异具有统计学意义(t =3.09,P<0.05).结论 霉酚酸酯对原发性肾病综合征及尿蛋白具有疗效显著、副反应小、安全可靠等优点,值得临床推广使用.%Objective To study the effect of mycophenolate mofetil(MMF) on primary nephrotic syndrome and the impact on urinary protein.Methods According to the digital table,136 cases of nephrotic syndrome were randomly divided into MMF group and control group,68 cases in the control group were dealed with conventional treatment,MMF group was treated with MMF.After 6 months,the treatment effects of both groups were evaluated.Results The overall response rate of MMF group was 82.8%,higher than that of control group 60.6% with statistically significant difference (x2 =10.86,P < 0.05),;Blood urea nitrogen(BUN),serum creatinine(Scr),24 h urinary protein of two groups had varying degree of reduction,the indexes of MMF group decreased even more significant (t =2.50,4.59,11.12,all P < 0.05).Serum albumin of the two groups had risen,but MMF group increased more significantly (t =3.09,P < 0.05).Conclusion The advantages of MMF are significant effect,reduction in side effects,safe and reliable on nephrotic syndrome and urinary protein,which is worthy of clinical use.

  5. Childhood Obesity: Common Misconceptions

    Science.gov (United States)

    ... of childhood obesity. Yes, hypothyroidism (a deficit in thyroid secretion) and other rarer and more severe genetic and metabolic disorders (eg, Prader-Willi syndrome, Turner syndrome, Cushing syndrome) ...

  6. 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

    Directory of Open Access Journals (Sweden)

    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

  7. 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

  8. Klinefelter syndrome: clinical and auxological features of 14 patients diagnosed in childhood

    Directory of Open Access Journals (Sweden)

    M.F. Messina

    2013-08-01

    Full Text Available Klinefelter syndrome (KS is the most frequent chromosomal aneuploidy with a prevalence of 1: 500 men but it often remains a largely undiagnosed condition and only 10% of cases are identified in childhood and adolescence. We report the anamnestic, clinical and auxological findings of 14 KS patients diagnosed in paediatric age. 3/14 patients (21% with KS were diagnosed in prenatal age by amniocentesis, 1 patient was diagnosed at birth due to genital ambiguity and the remaining 10/14 (71.4% were diagnosed at a chronological age younger than 15 years old for a clinical picture characterized by a peculiar cognitive and behavioral pattern or genital anomalies and abnormalities of pubertal development. The classical karyotype 47 XXY was present in 10/14 subjects (72%, a mosaic form (46 XY/47 XXY was present in 2/14 (14% and a complex aneuploidy (48 XXYY and 48 XXXY was present in the remaining 2/14 (14% patients. All KS patients diagnosed in childhood and adolescence (10/14 =71.4 % showed a stature taller than the respective target height and also the predicted final height (calculated from a chronological age older than 7 years old and the reached final height were significantly taller than target height. Conclusion: according to our retrospective data we can assert that KS in paediatric age is characterized by a stature taller than target height, often associated with a characteristic cognitive and behavioral pattern while the typical clinical signs and symptoms of KS are lacking and manifest only in late adolescence or adulthood.

  9. CHILDHOOD GUILLIAN-BARRE SYNDROME IN THE IRAN’S EAST AZERBAIJAN PROVINCE: 2001-2005

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    M. Barzegar

    2008-10-01

    Full Text Available Objective This study aims at determining the epidemiologic, presenting symptoms, clinical course and electrophysiologic features of childhood Guillain-Barre Syndrome(GBS in the East Azarbaijan province over a period of five years. Materials & Methods All the patients, aged< 15 years, referred/admitted to Tabriz Children Hospital with GBS between January 2001 and December 2005 were investigated.Results One hundred and twelve subjects were enrolled during this period. The average annual incidence rate was 2.21 per 100000 population of children aged<15years(CI 95%: 1.8-2.6; the highest proportion of 29% was observed in winter (P=0.10; mean age (SD of subjects was 5.1 (3.3 years, while 61.6% were ≤5years. The male/female ratio was 1.3. Antecedent events were identified in 80(71.4% patients. The most common manifestation was limb weakness; at the peak of the disease course, only 8 (7.1% patients were able to walk without assistance. Cranial nerve involvement was found in 37.5%. Fifteen percent of patients had autonomic dysfunction, and 32.2% complained of neuropathic pain. Artificial ventilation was needed in 10.7% of patients, and three patients (2.7% died due to cardiac arrest. Electrophysiological examination showed the demyelinating type of the disease in 54.5%; axonal type in 35.7% and 9.8% as unclassified or normal pattern. Short time to reach nadir (P=0.008, cranial nerve involvement (P=0.000, autonomic involvement (P=0.001, and axonal pattern on electro diagnosis (P=0.043 were found as risk factors for respiratory failure. In follow-up, at the end of one year, 95% of patients could walk without aid. ConclusionThe axonal type of GBS is a relatively common form of childhood GBS occurring in East Azerbaijan.

  10. IQ in Childhood and the Metabolic Syndrome in Middle Age: Extended Follow-Up of the 1946 British Birth Cohort Study

    Science.gov (United States)

    Richards, Marcus; Black, Stephanie; Mishra, Gita; Gale, Catharine R.; Deary, Ian J.; Batty, David G.

    2009-01-01

    IQ in early adulthood has been inversely associated with risk of the metabolic syndrome in midlife. We tested this association in the British 1946 birth cohort, which assessed IQ at age eight years and ascertained the metabolic syndrome at age 53 years based on modified (non-fasting blood) ATPIII criteria. Childhood IQ was inversely associated…

  11. 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。

  12. Changes of renal tissue TNF-α,Plasma TNF-α and urine TNF-α in patients with primary nephrotic syndrome%原发性肾病综合征患者肾组织、血和尿TNF-α变化及其意义

    Institute of Scientific and Technical Information of China (English)

    刘晓渭; 朱妙珍; 李冬梅; 王汉民; 陈威; 刘水芹; 朱军

    2001-01-01

    目的 探讨TNF-α在原发性肾病综合征(PNS)发病中所起的作用。方法 检测40例PNS患者和10名正常人血、尿、肾组织TNF-α含量。40例PNS患者接受糖皮质激素常规治疗。分别于治疗后1、2、4、8周复查血、尿TNF-α水平,并与正常人血、尿TNF-α水平进行比较。结果 40例PNS患者肾组织局部、血、尿TNF-α均较正常对照组明显增高(P0.05). The plasma and urine TNF-α had positive correlation with BUN, Scr, Pro(24 hour's urinary protein volume) and negative correlation with Ccr.Conclusion TNF-α participates in with occurrence and development of primary nephrotic syndrome. The effect mechanisms of corticosteroids can treat PNS, being related to inhibition in production of TNF-α. Plasma and uria levels of TNF-α can be served as an clinical index of estimating occurrence and activity of PNS.

  13. 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

    目的 通过槐杞黄颗粒联合糖皮质激素治疗儿童原发性肾病综合征(PNS)与单纯糖皮质激素治疗儿童原发性肾病综合征的对比研究,探讨槐杞黄颗粒对肾病综合征患儿的疗效及其免疫调节作用,观察其不良反应.方法 将40例原发性肾病综合征患儿随机分为2组,治疗组20例采用槐杞黄联合糖皮质激素治疗,对照组20例单独服用糖皮质激素治疗,观察两组患儿水肿消退、蛋白尿转阴时间;观察期内感染例数;两组患儿治疗前后体液免疫:IgA、IgM、IgG,细胞免疫:CD3、CD4、CD8、CD4/CD8的变化情况;槐杞黄颗粒的不良反应;观察周期:连续观察12周.结果 治疗组尿蛋白转阴、水肿消退时间明显小于对照组(P<0.05),感染发生率小于对照组(P<0.05);治疗组患儿体液免疫及细胞免疫较对照组明显改善,IgC、CD3、CD4/CD8均增高(P<0.01);治疗组治疗后IgA、IgM有一定增高趋势,但与对照组比较,其差异无统计学意义(P>0.05);2例患儿出现恶心、呕吐、腹泻,未见皮疹、肝肾功能异常等不良反应.结论 槐杞黄颗粒辅助治疗肾病综合征患儿,可以缩短水肿消退、蛋白尿转阴的时间,可以增强肾脏病患儿的免疫调节功能、减少感染发生的机会,进而增强激素疗效改善预后,同时在治疗过程中未出现明显的不良反应,临床证实是安全有效的辅助治疗药物.%Objective To investigate the effects of Huaiqihuang granule on treatment, immune regulation and its side effect of pediatric primary nephrotic syndrome by the contrast research of Huaiqihuang granule puls glucocorticoid and glucocorti-coid only. Methods 40 children with primary nephrotic syndrome were randomly tow groups. The treatment group(20 children) were teated with Huaiqihuang granule puls glucocorticoid, the contrast group (20 children) had glucocorticoid only. The follow were obseved; the time of edema regression and prourine

  14. Childhood apraxia of speech without intellectual deficit in a patient with cri du chat syndrome.

    Science.gov (United States)

    Marignier, Stéphanie; Lesca, Gaetan; Marguin, Jessica; Bussy, Gérald; Sanlaville, Damien; des Portes, Vincent

    2012-06-01

    We report an 11-year-old girl for whom the diagnosis of cri du chat syndrome (CdCS) was made during a genetic investigation of childhood apraxia of speech. The patient presented with the classic chromosome 5 short arm deletion found in CdCS. The microdeletion, characterised using aCGH (array Comparative Genomic Hybridisation), was 12.85 Mb, overlapping the 5p15.2 and 5p15.3 critical regions. CdCS is typically associated with severe mental retardation while this patient had normal intellectual performance, confirmed by normal results from categorisation tasks. This mild phenotype was assessed using a comprehensive cognitive battery. Language evaluation showed normal receptive vocabulary scores, in contrast with obvious oro-facial dyspraxia. Disabled fine motor skills were confirmed as well as weak visuo-spatial reasoning abilities. In conclusion, fine cognitive assessment may be worthwhile for patients with CdCS since good intellectual functioning may be masked by severe speech and gestural dyspraxia, thus requiring specific teaching and rehabilitation strategies.

  15. Altered microstructure within social-cognitive brain networks during childhood in Williams syndrome.

    Science.gov (United States)

    Haas, Brian W; Barnea-Goraly, Naama; Sheau, Kristen E; Yamagata, Bun; Ullas, Shruti; Reiss, Allan L

    2014-10-01

    Williams syndrome (WS) is a neurodevelopmental condition caused by a hemizygous deletion of ∼26-28 genes on chromosome 7q11.23. WS is associated with a distinctive pattern of social cognition. Accordingly, neuroimaging studies show that WS is associated with structural alterations of key brain regions involved in social cognition during adulthood. However, very little is currently known regarding the neuroanatomical structure of social cognitive brain networks during childhood in WS. This study used diffusion tensor imaging to investigate the structural integrity of a specific set of white matter pathways (inferior fronto-occipital fasciculus [IFOF] and uncinate fasciculus [UF]) and associated brain regions [fusiform gyrus (FG), amygdala, hippocampus, medial orbitofrontal gyrus (MOG)] known to be involved in social cognition in children with WS and a typically developing (TD) control group. Children with WS exhibited higher fractional anisotropy (FA) and axial diffusivity values and lower radial diffusivity and apparent diffusion coefficient (ADC) values within the IFOF and UF, higher FA values within the FG, amygdala, and hippocampus and lower ADC values within the FG and MOG compared to controls. These findings provide evidence that the WS genetic deletion affects the development of key white matter pathways and brain regions important for social cognition.

  16. Less efficient and costly processes of frontal cortex in childhood chronic fatigue syndrome

    Directory of Open Access Journals (Sweden)

    Kei Mizuno

    2015-01-01

    Full Text Available The ability to divide one's attention deteriorates in patients with childhood chronic fatigue syndrome (CCFS. We conducted a study using a dual verbal task to assess allocation of attentional resources to two simultaneous activities (picking out vowels and reading for story comprehension and functional magnetic resonance imaging. Patients exhibited a much larger area of activation, recruiting additional frontal areas. The right middle frontal gyrus (MFG, which is included in the dorsolateral prefrontal cortex, of CCFS patients was specifically activated in both the single and dual tasks; this activation level was positively correlated with motivation scores for the tasks and accuracy of story comprehension. In addition, in patients, the dorsal anterior cingulate gyrus (dACC and left MFG were activated only in the dual task, and activation levels of the dACC and left MFG were positively associated with the motivation and fatigue scores, respectively. Patients with CCFS exhibited a wider area of activated frontal regions related to attentional resources in order to increase their poorer task performance with massive mental effort. This is likely to be less efficient and costly in terms of energy requirements. It seems to be related to the pathophysiology of patients with CCFS and to cause a vicious cycle of further increases in fatigue.

  17. 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

  18. Preeclampsia: is it because of the asymptomatic, unrecognized renal scars caused by urinary tract infections in childhood that become symptomatic with pregnancy?

    Science.gov (United States)

    Ozlü, Tülay; Alçelik, Aytekin; Calişkan, Billur; Dönmez, Melahat Emine

    2012-11-01

    Preeclampsia is an important disease of pregnancy whose exact etiology is still unknown despite continuing developments in medicine. Although most commonly it is believed to be caused by a defective placentation, in this paper, we hypothesize that the primary underlying problem in the development of preeclampsia can be in kidneys in a greater proportion of cases than it is believed today. The increased intravascular volume and the increased work load of kidneys together with the resulting glomerular hypertrophy may precipitate nephrotic syndrome, which in this case is called "preeclampsia" in a previously affected kidney. Urinary tract infections in childhood leaving silent, unrecognized small scars in the kidneys may be the underlying renal cause which disrupts its silence with an increased work load of kidneys prominently occurring after the midtrimester. The histopathologic finding in kidneys with renal scars after childhood urinary tract infections and in preeclampsia is focal segmental glomerulosclerosis in the majority of cases and this similarity strengthens our hypothesis.

  19. 霉酚酸酯和环磷酰胺治疗儿童肾病综合征型紫癜性肾炎的临床研究%Clinical research of mycophenolate mofetil and cyclophosphamide in the treatment of Children Henoch-Schonlein pur-pura nephritis accompanied by nephrotic syndrome

    Institute of Scientific and Technical Information of China (English)

    苗静; 周俊霖; 彭晓杰; 郑卫民; 傅睿; 黄玉辉

    2015-01-01

    Objective To compare the therapeutic effects and side effects of mycophenolate mofetil (MMF) and cyclophos-phamide (CTX) in the treatment of Children Henoch-Schonlein purpura nephritis (HSPN) accompanied by nephrotic syndrome. Methods Thirty-one children who suffered from HSPN with nephrotic-range proteinuria and hematuria or with only nephrotic-range proteinuria were given intravenous administration of pulse methylprednisolone firstly ,who did not respond to the treatment were enrolled. These patients were divided into two groups. Nineteen patients were treated with oral intake of MMF (MMF group) 20-25mg·kg-1·d-1 for 6months,then gradually withdrawn at the 12th month. Other twelve patients were treated with intravenous pulse administration of cyclophosphamide (CTX group, 10 mg·kg-1·d-1,2 days) once every two weeks,6-8 course of treatments totally. Clinical assessment,24-hour urinary protein,creatinine clearance (CCr),liver function and side effects were performed at the 3rd,6th and 12th month. Results The complete remission rates in the 6th,12th month after MMF and CTX therapy were 42. 1%and 41.7%,84.2%and 75.0%(P>0.05),respectively. The total remission rates were 94.7%and 91.7%(P>0.05);There was no significant difference in the efficiency rate between MMF and CTX. The side effects of MMF were much weak.The side effects of CTX included the decreased white blood cells and vomitting. Conclusion There is no significant difference in the efficiency rate between MMF and CTX for treating Children Henoch-Schonlein purpura nephritis (HSPN) accompanied by nephrotic syndrome. Side effects of MMF were obviously fewer than CTX.%目的 比较霉酚酸酯(MMF)和环磷酰胺(CTX)治疗儿童肾病综合征型紫癜性肾炎(HSPN)的疗效及其不良反应.方法 有过敏性紫癜病史,临床表现为肾病水平的蛋白尿和肉眼血尿或大量镜下血尿的患儿31例,患儿先给予甲泼尼龙冲击一疗程后分为MMF组19例和CTX组12例. MMF

  20. Low putamen activity associated with poor reward sensitivity in childhood chronic fatigue syndrome

    Directory of Open Access Journals (Sweden)

    Kei Mizuno, Ph.D.

    2016-01-01

    Full Text Available Motivational signals influence a wide variety of cognitive processes and components of behavioral performance. Cognitive dysfunction in patients with childhood chronic fatigue syndrome (CCFS may be closely associated with a low motivation to learn induced by impaired neural reward processing. However, the extent to which reward processing is impaired in CCFS patients is unclear. The aim of the present functional magnetic resonance imaging (fMRI study was to determine whether brain activity in regions related to reward sensitivity is impaired in CCFS patients. fMRI data were collected from 13 CCFS patients (mean age, 13.6 ± 1.0 years and 13 healthy children and adolescents (HCA (mean age, 13.7 ± 1.3 years performing a monetary reward task. Neural activity in high- and low-monetary-reward conditions was compared between CCFS and HCA groups. Severity of fatigue and the reward obtained from learning in daily life were evaluated by questionnaires. Activity of the putamen was lower in the CCFS group than in the HCA group in the low-reward condition, but not in the high-reward condition. Activity of the putamen in the low-reward condition in CCFS patients was negatively and positively correlated with severity of fatigue and the reward from learning in daily life, respectively. We previously revealed that motivation to learn was correlated with striatal activity, particularly the neural activity in the putamen. This suggests that in CCFS patients low putamen activity, associated with altered dopaminergic function, decreases reward sensitivity and lowers motivation to learn.

  1. Low putamen activity associated with poor reward sensitivity in childhood chronic fatigue syndrome.

    Science.gov (United States)

    Mizuno, Kei; Kawatani, Junko; Tajima, Kanako; Sasaki, Akihiro T; Yoneda, Tetsuya; Komi, Masanori; Hirai, Toshinori; Tomoda, Akemi; Joudoi, Takako; Watanabe, Yasuyoshi

    2016-01-01

    Motivational signals influence a wide variety of cognitive processes and components of behavioral performance. Cognitive dysfunction in patients with childhood chronic fatigue syndrome (CCFS) may be closely associated with a low motivation to learn induced by impaired neural reward processing. However, the extent to which reward processing is impaired in CCFS patients is unclear. The aim of the present functional magnetic resonance imaging (fMRI) study was to determine whether brain activity in regions related to reward sensitivity is impaired in CCFS patients. fMRI data were collected from 13 CCFS patients (mean age, 13.6 ± 1.0 years) and 13 healthy children and adolescents (HCA) (mean age, 13.7 ± 1.3 years) performing a monetary reward task. Neural activity in high- and low-monetary-reward conditions was compared between CCFS and HCA groups. Severity of fatigue and the reward obtained from learning in daily life were evaluated by questionnaires. Activity of the putamen was lower in the CCFS group than in the HCA group in the low-reward condition, but not in the high-reward condition. Activity of the putamen in the low-reward condition in CCFS patients was negatively and positively correlated with severity of fatigue and the reward from learning in daily life, respectively. We previously revealed that motivation to learn was correlated with striatal activity, particularly the neural activity in the putamen. This suggests that in CCFS patients low putamen activity, associated with altered dopaminergic function, decreases reward sensitivity and lowers motivation to learn.

  2. Thimerosal-Preserved Hepatitis B Vaccine and Hyperkinetic Syndrome of Childhood

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    David A. Geier

    2016-03-01

    Full Text Available (1 Background: Hyperkinetic syndrome of childhood (HKSoC is an International Classification of Diseases, Ninth Revision, Clinical Modification (ICD-9 category in which the majority of the children are also diagnosed under the Diagnostic and Statistical Manual of Mental Disorders, 4th Edition, Text Revision (DSM-IV-TR, where the umbrella term is “Attention-Deficit and Disruptive Behavior Disorders”. The diagnostic criteria for HKSoC are developmentally inappropriate inattention, hyperactivity, and impulsivity. Some studies have implicated mercury (Hg exposure as a risk factor. (2 Methods: This hypothesis testing study; using the Vaccine Safety Datalink; assessed the toxicological effects of bolus exposure to organic-Hg from Thimerosal-containing vaccines (TCVs by examining the relationship between Thimerosal-preserved hepatitis B vaccines (TM-HepB given at varying levels and at specific intervals in the first six months after birth and the risk of a child being diagnosed with HKSoC. (3 Results: Children diagnosed with HKSoC were significantly more likely to be exposed to increased organic-Hg from TM-HepB doses given within the first month (odds ratio = 1.45; 95% confidence interval (CI = 1.30–1.62; within the first two months (odds ratio = 1.43; 95% CI = 1.28–1.59; and within the first six months (odds ratio = 4.51; 95% CI = 3.04–6.71 than controls. (4 Conclusion: The results indicate that increasing organic-Hg exposure from TCVs heightens the risk of a HKSoC diagnosis.

  3. Attention in Williams Syndrome and Down's Syndrome: Performance on the New Early Childhood Attention Battery

    Science.gov (United States)

    Breckenridge, Kate; Braddick, Oliver; Anker, Shirley; Woodhouse, Margaret; Atkinson, Janette

    2013-01-01

    Attentional problems are commonly reported as a feature of the behavioural profile in both Williams syndrome (WS) and Down's syndrome (DS). Recent studies have begun to investigate these impairments empirically, acknowledging the need for an approach that considers cross-syndrome comparisons and developmental changes across the different…

  4. The CAST (Childhood Asperger Syndrome Test): preliminary development of a UK screen for mainstream primary-school-age children.

    Science.gov (United States)

    Scott, Fiona J; Baron-Cohen, Simon; Bolton, Patrick; Brayne, Carol

    2002-03-01

    The article describes a pilot and follow-up study of the preliminary development of a new tool to screen for Asperger syndrome (AS) and related social and communication conditions (the Childhood Asperger Syndrome Test, CAST) in children aged 4-11 years, in a non-clinical setting. In the pilot study, parents of 13 children with AS and of 37 typically developing children completed the CAST. There were significant differences between the AS and typical sample means. The pilot was used to establish preliminary cut-off scores for the CAST. In the main study, parents of 1150 primary-school-age children were sent the CAST, and 174 took part in the full data analysis. Results suggest that compared with other tools currently available, the CAST may be useful for identifying children at risk for AS and related conditions, in a mainstream non-clinical sample. Further research is ongoing.

  5. 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

  6. 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

  7. 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

  8. 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

  9. Efficacy and safety analysis of different doses of low molecular weight heparin combined with hormone in refractory nephrotic syndrome%不同剂量低分子肝素联合激素治疗难治性肾病综合征的疗效及安全性

    Institute of Scientific and Technical Information of China (English)

    刘海燕; 王亚莉

    2016-01-01

    Objective To discuss the efficacy and safety analysis of different doses of low molecular weight heparin combined with hormone in the treatment of refractory nephrotic syndrome.Methods 60 cases of patients with refractory nephrotic syndrome in our hospital from January 2014 to January 2016 were selected,According to the random number table,60 patients were divided into high,medium and low dose of low molecular weight heparin group and control group,control group were treated with hormonal therapy,the high,medium and low dose of low molecular weight heparin group were injected low-molecular-weight heparin calcium on the basis of the control group,twice daily,once daily and every other day,respectively.Results After treatment,the clinical total effective rate of the high,medium and low dose group and control group patients was 14(93.3%),12(80.0%),10(66.7%) and 9(60.0%)respectively,the total efficiency of the high dose group was significantly higher than that of the control group(P<0.05),while there was no significant difference among control,medium and low dose group.The 24 h urine protein quantitation,ALB and Cr in high dose group improved better than those in medium and low dose group(P<0.05).The TG,TC and LDL in high dose group improved better than those in control group(P<0.05). The prothrombin time(PT)and activated partial thromboplastin time(APTT)in high and medium dose group were longer than those in control group(P<0.05),while fibrinogen(FIB)was lower(P<0.05).The PT in low dose group was longer than control group(P<0.05),while there was no significant difference in other coagulation indicators compared with control group.The APTT in high dose group was longer than low dose group(P<0.05).There was no obvious adverse reactions among each dose group.Conclusion The clinical curative effect in different doses of low molecular weight heparin combined with hormone in the treatment of refractory nephrotic syndrome is better,which could be significantly improved in

  10. Immunosuppressive treatment for nephrotic idiopathic membranous nephropathy: a meta-analysis based on Chinese adults.

    Directory of Open Access Journals (Sweden)

    Guoqiang Xie

    Full Text Available BACKGROUND: Idiopathic membranous nephropathy (IMN is the most common pathological type for nephrotic syndrome in adults in western countries and China. The benefits and harms of immunosuppressive treatment in IMN remain controversial. OBJECTIVES: To assess the efficacy and safety of different immunosuppressive agents in the treatment of nephrotic syndrome caused by IMN. METHODS: PubMed, EMBASE, Cochrane Library and wanfang, weipu, qinghuatongfang, were searched for relevant studies published before December 2011. Reference lists of nephrology textbooks, review articles were checked. A meta-analysis of randomized controlled trials (RCTs meeting the criteria was performed using Review Manager. MAIN RESULTS: 17 studies were included, involving 696 patients. Calcineurin inhibitors had a better effect when compared to alkylating agents, on complete remission (RR 1.61, 95% CI 1.13, to 2.30 P = 0.008, partial or complete remission (effective (CR/PR, RR 1.29, 95% CI 1.09 to 1.52 P = 0.003, and fewer side effects. Among calcineurin inhibitors, tacrolimus (TAC was shown statistical significance in inducing more remissions. When compared to cyclophosphamide (CTX, leflunomide (LET showed no beneficial effect, mycophenolate mofetil (MMF showed significant beneficial on effectiveness (CR/PR, RR: 1.41, 95% CI 1.16 to 1.72 P = 0.0006 but not significant on complete remission (CR, RR: 1.38, 95% CI 0.89 to 2.13 P = 0.15. CONCLUSIONS: This analysis based on Chinese adults and short duration RCTs suggested calcineurin inhibitors, especially TAC, were more effective in proteinuria reduction in IMN with acceptable side effects. Long duration RCTs were needed to confirm the long-term effects of those agents in nephrotic IMN.

  11. Minimal change disease

    Science.gov (United States)

    ... get worse You develop new symptoms, including side effects from the medicines used to treat the disorder Alternative Names Minimal change nephrotic syndrome; Nil disease; Lipoid nephrosis; Idiopathic nephrotic syndrome of childhood Images ...

  12. Long-term effects of oxandrolone treatment in childhood on neurocognition, quality of life and social-emotional functioning in young adults with Turner syndrome

    NARCIS (Netherlands)

    Freriks, K.; Verhaak, C.M.; Sas, T.C.; Menke, L.A.; Wit, J.M.; Otten, B.J.; Keizer-Schrama, S.M.; Smeets, D.F.C.M.; Netea-Maier, R.T.; Hermus, A.R.M.M.; Kessels, R.P.C.; Timmers, H.J.L.M.

    2015-01-01

    Turner syndrome (TS) is the result of (partial) absence of one X-chromosome. Besides short stature, gonadal dysgenesis and other physical aspects, TS women have typical psychological features. Since psychological effects of androgen exposure in childhood probably are long-lasting, we explored long-t

  13. Long-term effects of oxandrolone treatment in childhood on neurocognition, wellbeing and social-emotional functioning in young adults with Turner syndrome

    NARCIS (Netherlands)

    Freriks, K.; Verhaak, C.M.; Sas, T.C.J.; Menke, L.A.; Wit, J.M.; Otten, B.J.; Muinck Keizer-Schrama, S.M.P.F. de; Smeets, D.F.C.M.; Netea-Maier, R.T.; Hermus, A.R.M.M.; Kessels, R.P.C.; Timmers, H.J.L.M.

    2015-01-01

    Turner syndrome (TS) is the result of (partial) absence of one X-chromosome. Besides short stature, gonadal dysgenesis and other physical aspects, TS women have typical psychological features. Since psychological effects of androgen exposure in childhood probably are long-lasting, we explored long-t

  14. Superior oblique tendon (Brown’s syndrome as the presenting finding in childhood onset HLA-B27-related enthesitis and juvenile idiopathic oligoarticular arthritis

    Directory of Open Access Journals (Sweden)

    C. Pham

    2014-11-01

    Full Text Available We report two patients who presented with Brown’s syndrome. The first is a 7-year-old boy who at the time of his diagnosis was also found to have enthesitis and HLA-B27 positivity. The second patient was diagnosed with bilateral Brown’s syndrome at 13 months of age. At age 7 she developed a persistent oligoarticular arthritis and unilateral anterior iritis consistent with the oligoarticular Juvenile Idiopatic Arthritis (JIA phenotype. These cases highlight ophthalmologic findings and diagnostic considerations with respect to Brown’s syndrome and associated childhood onset rheumatologic disease.

  15. Imprinted anomalies in fetal and childhood growth disorders: the model of Russell-Silver and Beckwith-Wiedemann syndromes.

    Science.gov (United States)

    Netchine, Irène; Rossignol, Sylvie; Azzi, Salah; Brioude, Fréderic; Le Bouc, Yves

    2012-01-01

    Fetal growth is a complex process. Its restriction is associated with morbidity and long term metabolic consequences. Imprinted genes have a critical role in mammalian fetal growth. The human chromosome 11p15 encompasses two imprinted domains regulated by their own differentially methylated region (DMR), also called Imprinted Control Region (ICR1 at the H19/IGF-2 domain, paternally methylated), and ICR2 at the KCNQ1/CDKN1C domain (maternally methylated). Loss of imprinting at these two domains is implicated in two growth disorders clinically opposite. A loss of DNA methylation (LOM) at ICR1 is identified in over 50% of patients with Russell-Silver syndrome (RSS), characterized by intrauterine and postnatal growth retardation, spared cranial growth, frequent body asymmetry and severe feeding difficulties. Inversely, a gain of methylation at ICR1 is found in 10% of patients with Beckwith-Wiedemann syndrome (BWS), an overgrowth syndrome with an enhanced childhood tumor risk. We have identified over 150 RSS patients with 11p15 LOM allowing long-term follow-up studies and proposal of clinical guidelines. We also found that ∼10% of RSS patients and ∼25% of BWS patients have multilocus LOM at imprinted regions other than ICR1 or ICR2 11p15, respectively. Recent studies have identified cis-acting regulatory elements and trans-acting factors involved in the regulation of 11p15 imprinting, establishing new potential mechanisms of RSS and BWS.

  16. 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

  17. Childhood obesity.

    Science.gov (United States)

    Seth, Anju; Sharma, Rajni

    2013-04-01

    Childhood obesity is an issue of serious medical and social concern. In developing countries including India, it is a phenomenon seen in higher socioeconomic strata due to the adoption of a western lifestyle. Consumption of high calorie food, lack of physical activity and increased screen time are major risk factors for childhood obesity apart from other genetic, prenatal factors and socio-cultural practices. Obese children and adolescents are at increased risk of medical and psychological complications. Insulin resistance is commonly present especially in those with central obesity and manifests as dyslipidemia, type 2 diabetes mellitus, impaired glucose tolerance, hypertension, polycystic ovarian syndrome and metabolic syndrome. Obese children and adolescents often present to general physicians for management. The latter play a key role in prevention and treatment of obesity as it involves lifestyle modification of the entire family. This article aims at discussing the approach to diagnosis and work-up, treatment and preventive strategies for childhood obesity from a general physician's perspective.

  18. 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.

  19. 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.

  20. Acquired Demyelinating Syndromes: Focus on Neuromyelitis Optica and childhood-onset Multiple Sclerosis

    NARCIS (Netherlands)

    E.D. van Pelt - Gravesteijn (Daniëlle)

    2016-01-01

    markdownabstractAcquired demyelinating syndromes (ADS) cover a broad spectrum of central nervous system (CNS) inflammatory demyelinating syndromes, of which multiple sclerosis (MS) is the most common subtype. This thesis focuses on two relatively rare clinical subtypes of ADS: neuromyelitis optica s

  1. Examination of Work Environment Factors Relating to Burnout Syndrome of Early Childhood Educators in Greece

    Science.gov (United States)

    Rentzou, Konstantina

    2012-01-01

    Early childhood education is a profession which requires the professional staff to spend considerable time in intense involvement with other people. The pressure from the demands this profession has can create a sense of physical and emotional exhaustion that often leads to burnout. Thus, previous research has linked perceptions of the work…

  2. Sudden infant death syndrome, childhood thrombosis, and presence of genetic risk factors for thrombosis

    DEFF Research Database (Denmark)

    Larsen, TB; Nørgaard-Pedersen, B; Lundemose, JB

    2000-01-01

    . The vulnerability of the infant brain stem to ischemia has been suggested to be a conceivable cause of sudden infant death syndrome. This is compatible with a hypothesis that genetic risk factors for cerebral thrombosis could cause microinfarction in the brain stem during the first month of life, affecting vital......Sudden infant death syndrome or "cot death" has until the late eighties been a significant cause of death in children between the ages of 1 month and 1 year. Approximately two per 1000 children born alive dies of sudden infant death syndrome each year in Western Europe, North America, and Australia...... in the child. This prompted us to investigate these genetic markers of thromboembolic disease in 121 cases of sudden infant death syndrome and in relevant controls, in the expectation of a more frequent occurrence of these markers if thrombosis is an etiological factor in sudden infant death syndrome...

  3. Sudden infant death syndrome, childhood thrombosis, and presence of genetic risk factors for thrombosis

    DEFF Research Database (Denmark)

    Larsen, T B; Nørgaard-Pedersen, B; Banner, Jytte

    2000-01-01

    in the child. This prompted us to investigate these genetic markers of thromboembolic disease in 121 cases of sudden infant death syndrome and in relevant controls, in the expectation of a more frequent occurrence of these markers if thrombosis is an etiological factor in sudden infant death syndrome......Sudden infant death syndrome or "cot death" has until the late eighties been a significant cause of death in children between the ages of 1 month and 1 year. Approximately two per 1000 children born alive dies of sudden infant death syndrome each year in Western Europe, North America, and Australia....... The vulnerability of the infant brain stem to ischemia has been suggested to be a conceivable cause of sudden infant death syndrome. This is compatible with a hypothesis that genetic risk factors for cerebral thrombosis could cause microinfarction in the brain stem during the first month of life, affecting vital...

  4. 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。

  5. Childhood Tourette's syndrome and the Thematic Apperception Test: is there a recognizable pattern?

    Science.gov (United States)

    Wodrich, D L; Thull, L M

    1997-10-01

    26 children, one-half of whom were diagnosed with Tourette's syndrome, were administered the Thematic Apperception Test. A pilot investigation suggested that children with Tourette's syndrome produced high rates of responses in four categories: references to physical aggression, supernatural power, character names, and specific quantities. When compared with other children treated for emotional and behavioral problems at the same facility and matched on demographic variables and IQ, children with Tourette's syndrome were not more likely to produce Thematic Apperception Test responses in any of the four categories. This study shows that a recognizable pattern on projective testing was not easily established among these 13 children with Tourette's syndrome and further highlights the importance of using mental health comparison groups when investigating clinical disorders.

  6. Mutations in NPHS2 in familial steroid-resistant nephrotic syndrome in Southern Chinese Hart ethnic group%中国南方汉族人家族性激素耐药型肾病综合征家系NPHS2基因突变

    Institute of Scientific and Technical Information of China (English)

    付荣; 陈新民; 王庆华; 陈胜平; 余自华; 叶礼燕; 任榕娜; 黄隽; 王承峰

    2008-01-01

    目的 分析中国南方汉族人家族性激素耐药型肾病综合征(SRNS)家系NPHS2基因突变及其特点.方法 研究对象为A、B、C 3个南方汉族人SRNS家系先证者及其姐和父母,50例尿检正常的南方汉族成年人作为对照人群.取所有研究对象外周静脉血3 ml,提取基因组DNA,PCR扩增NPHS2全部8个外显子及其周围的部分内含子和启动子全长序列,对PCR产物直接进行DNA序列测定.结果 对3个南方汉族人SRNS家系先证者NPHS2全部8个外显子及其周围的部分内含子进行突变分析,未发现NPHS2突变,仅在外显子8上检测到1个NPHS2基因多态性(954T>C).在3个家系的先证者及其姐和父母的NPHS2启动子上检测到6个变异:-1715A>G、-1709G>A、-1000A>T、-670C>T、-116C>T和-51G>T.其中5个变异(-1709G>A、-1000A>T、-670C>T、-116C>T和-51G>T)在100条正常染色体中也有检出,它们在SRNS患者中的等位基因频率分别与在对照人群中的等位基因频率比较差异均无统计学意义(P>0.05);另1个变异(-1715A>G)在家系C的先证者及其母亲(尿检正常)中检出,为杂合变异,而在100条正常染色体中未发现.-1000A>T为新发现的NPHS2基因多态性,-1715A>G为新发现的NPHS2变异.结论 NPHS2基因突变不是本研究3个南方汉族人家族性SRNS家系的主要致病原因.%Objective Mutations in NPHS2 mapped to 1q25-q31 and encoding podecin,which is exclusively expressed in glomerular podocytes,are responsible for autosomal recessive familial steroidresistant nephrotic syndrome(SRNS)with minor glomerular abnormalities or focal segmental glomendosclerosis.Different groups from European and North American countries have screened NPHS2 mutations in familial SRNS with recessive inheritance,documenting a mutation detection rate of 45%-55% in families.This study aimed to examine mutations in the NPHS2 gene in Southern Chinese Han ethnic group patients with familial SRNS

  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. 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

  10. 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.

  11. Epidemiology of childhood Guillan-Barre syndrome in the north west of Iran

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    Karegarmaher Mohammad H

    2007-08-01

    Full Text Available Abstract Background and aims This study was carried out to investigate the incidence, annual time trend and some epidemiological and clinical features of Guillain-Barre syndrome in children in the north west of Iran. Materials and methods In this population-based cross sectional research, epidemiological and clinical features of 143 cases with Guillain-Barre syndrome between 2001 and 2006 were studied. The setting of the study was Tabriz Children Medical Centre, the major University-Hospital located in Tabriz city of the East Azarbaijan province covering whole region. Data collected included age, gender, chronological information, preceding events, functional grade of motor deficit. Results The mean age (standard deviation of subjects was 5.4 (3.6 years. The male/female ratio was 1.3. The average annual incidence rate was 2.27 per 100 000 population of 15 years children (CI95%: 1.9–2.6. The majority of cases occurred in March, July and November and the highest proportion of the syndrome was observed in winter (29 percent, P > 0.10. Conclusion The results indicated that an unexpected high incidence of Guillain-Barre syndrome has occurred in 2003 in the region. We concluded that a monitoring and surveillance system for Guillain-Barre syndrome is essential to set up in this region.

  12. Study on traditional Chinese and Western medicine treatment of primary nephrotic syndrome%原发性肾病综合征的中西医结合治疗研究

    Institute of Scientific and Technical Information of China (English)

    李信雨

    2012-01-01

    原发性肾病综合征(PNS)以严重水肿、大量蛋白尿、低蛋白血症、高脂血症为临床特点,常合并血液高凝状态甚至可形成血栓,且易合并感染.治疗以肾上腺皮质激素、细胞毒药物为基础,其疗效与年龄、病理类型相关.中西医结合治疗PNS在提高疗效,减少复发以及降低激素等不良反应方面显示了巨大作用.中西医结合治疗原发性肾病综合征,不仅大大提高了疗效,减少了疾病的复发,而且明显减少了西药的毒副作用,在临床中有着良好的治疗前景.%The primary nephralic syndrome (PNS) has the clinical features of severe edema, a large number of urine protein, hypopro-teinemia, hyperlipidemia, often associated with hypercoagulable states of blood and even thrombosis, and easy to co-infection. The treatment of PNS based on adrenal corticosleroids, cytotoxic drugs, and its curative effect was correlated with age and pathological type. It showed a great role in reducing recurrence and the adverse effects of hormones treated PNS with traditional Chinese medicine and Western medicine. It could not only increase efficacy, reduce the recurrence of the disease, but also significantly reduce the side effects of Western medicine when PNS was treated with traditional Chinese medicine and Western medicine. It would have a good therapeutic prospect in the clinic.

  13. SPASTIC FORM OF CEREBRAL PALSY, EPILEPSY WITH BENIGN EPILEPTIFORM DISCHARGE OF CHILDHOOD ON ELECTROENCEPHALOGRAM, AND IATROGENIC STEVENS–JOHNSON SYNDROME (CASE DESCRIPTION

    Directory of Open Access Journals (Sweden)

    A. S. Kotov

    2016-01-01

    Full Text Available The article describes the phenomenon of dual pathology – a combination of structural changes in the brain and benign epileptiform discharge of childhood on electroencephalogram. The uniqueness lies in the observation that the child, since birth suffering from spastic form of cerebral palsy and severe epilepsy, demonstrated the development of Stevens–Johnson syndrome due to intolerance of one of the antiepileptic drugs. Therapeutic approaches to overcome a whole range of violations are discussed in the article.

  14. A New Guidelines For Using Erythromycin In Management Of Childhood Cyclic Vomiting Syndrome

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    Mahmood Haghighat

    2016-11-01

    Full Text Available Cyclic vomiting syndrome (CVS is a functional disorder without any determined cause, though some motility disorders are considered in stream of this syndrome. There is no clear treatment for CVS and all treatments are borrowed from treatment of other similar diseases such as migraine. Clinical practice guideline instructs the pediatricians and pediatric gastroenterologists for treatment of CVS in both inpatient and outpatient settings. Since there is no practical guideline for the empirical management of CVS, this guideline was prepared for framing the treatments in a scienti­c and simple way for this disorder.

  15. Childhood Ataxia with Cerebral Hypomyelination (CACH syndrome: A study of three siblings

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    Vaidya Sachin

    2004-07-01

    Full Text Available We report a family of three siblings with Childhood Ataxia with Cerebral Hypomyelination. All the siblings presented with early onset cerebellar ataxia beginning around five years of age with mild mental retardation. MRI showed diffuse white matter signal changes in all three patients with cerebellar atrophy while the spectroscopy was abnormal only in the eldest who was the most severely affected. The cases are reported for their rarity as well as for an opportunity of observing this uncommon disease in its stages of evolution in three siblings.

  16. Combination Chemotherapy in Treating Young Patients With Down Syndrome and Acute Myeloid Leukemia or Myelodysplastic Syndromes

    Science.gov (United States)

    2017-02-07

    Childhood Acute Basophilic Leukemia; Childhood Acute Eosinophilic Leukemia; Childhood Acute Erythroleukemia (M6); Childhood Acute Megakaryocytic Leukemia (M7); Childhood Acute Minimally Differentiated Myeloid Leukemia (M0); Childhood Acute Monoblastic Leukemia (M5a); Childhood Acute Monocytic Leukemia (M5b); Childhood Acute Myeloblastic Leukemia With Maturation (M2); Childhood Acute Myeloblastic Leukemia Without Maturation (M1); Childhood Acute Myelomonocytic Leukemia (M4); Childhood Myelodysplastic Syndromes; de Novo Myelodysplastic Syndromes; Secondary Acute Myeloid Leukemia; Secondary Myelodysplastic Syndromes; Untreated Childhood Acute Myeloid Leukemia and Other Myeloid Malignancies

  17. Glucose transporter-1 (GLUT1) deficiency syndrome: diagnosis and treatment in late childhood.

    NARCIS (Netherlands)

    Gramer, G.; Wolf, N.I.; Vater, D.; Bast, T.; Santer, R.; Kamsteeg, E.J.; Wevers, R.A.; Ebinger, F.

    2012-01-01

    BACKGROUND: Typical cases of glucose transporter-1 deficiency syndrome (GLUT1-DS) present with early-onset epilepsy. We report symptoms, diagnostic results, and effects of therapy in two patients diagnosed with GLUT1-DS at the age of 10 and 15 years, respectively. PATIENTS: Patient 1: After four cer

  18. Quality of life in adults with childhood-onset of Complex Regional Pain Syndrome type I

    NARCIS (Netherlands)

    Tan, Edward C T H; van de Sandt-Renkema, Nienke; Krabbe, Paul F M; Aronson, Daniel C; Severijnen, René S V M

    2009-01-01

    INTRODUCTION: The clinical presentation of Complex Regional Pain Syndrome type I (CRPS I) in children differs compared to the presentation in adults. Reported results of treatment of CRPS I in children are usually more favourable and seem better than the reported treatment of adults with CRPS I. We

  19. Benign occipital epilepsy of childhood: Panayiotopoulos syndrome in a 3 year old child

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    Menon Narayanankutty Sunilkumar , Vadakut Krishnan Parvathy

    2014-11-01

    Full Text Available Panayiotopoulos syndrome (PS is a relatively frequent and benign epileptic syndrome seen in children in the age group of 3-6 years and is characterised by predominantly autonomic symptoms and/or simple motor focal seizures followed or not by impairment of consciousness. Although multifocal spikes with high amplitude sharp-slow wave complexes at various locations can be present in the EEG, interictal electroencephalogram (EEG in children with this particular type of epilepsy characteristically shows occipital spikes. This syndrome has known to be a masquerader and can imitate gastroenteritis, encephalitis, syncope, migraine, sleep disorders or metabolic diseases. In the absence of thorough knowledge of types of benign epilepsy syndromes and their various clinical presentations, epilepsy such as PS can be easily missed. The peculiar aspects of this type of epilepsy in children should be known not only by paediatricians but also by general doctors because a correct diagnosis would avoid aggressive interventions and concerns on account of its benign outcome. In this case study, we report a case of PS in a 3 year old child.

  20. Family Health and Characteristics in Chronic Fatigue Syndrome, Juvenile Rheumatoid Arthritis, and Emotional Disorders of Childhood.

    Science.gov (United States)

    Rangel, Luiza; Garralda, M. Elena; Jeffs, Jim; Rose, Gillian

    2005-01-01

    Objective: To compare family health and characteristics in children with chronic fatigue syndrome (CFS), in juvenile rheumatoid arthritis (JRA), and emotional disorders. Method: Parents of 28 children and adolescents aged 11 to 18 years with CFS, 30 with JRA, and 27 with emotional disorders (i.e., anxiety and/or depressive disorders) were…

  1. Ontogeny of polycystic ovary syndrome and insulin resistance in utero and early childhood

    Science.gov (United States)

    Polycystic ovary syndrome (PCOS) is a prevalent hyperandrogenic infertility and cardiometabolic disorder that increases a woman's lifetime risk of type 2 diabetes mellitus. It is heritable and intensely familial. Progress toward a cure has been delayed by absence of an etiology. Evidence is mounting...

  2. Detection of metabolic syndrome features among childhood cancer survivors: A target to prevent disease

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    Adriana Aparecida Siviero-Miachon

    2008-08-01

    Full Text Available Adriana Aparecida Siviero-Miachon1, Angela Maria Spinola-Castro1, Gil Guerra-Junior21Division of Pediatric Endocrinology, Department of Pediatrics, Federal University of Sao Paulo – UNIFESP/EPM, Brazil; 2Division of Pediatric Endocrinology, Department of Pediatrics, State University of Campinas – FCM/UNICAMP, BrazilAbstract: Along with the growing epidemic of obesity, the risk of atherosclerosis, cardiovascular disease morbidity, and mortality are increasing markedly. Several risk factors for cardiovascular disease, such as visceral obesity, glucose intolerance, arterial hypertension, and dyslipidemia commonly cluster together as a condition currently known as metabolic syndrome. Thus far, insulin resistance, and endothelial dysfunction are the primary events of the metabolic syndrome. Several groups have recommended clinical criteria for the diagnosis of metabolic syndrome in adults. Nonetheless, in what concerns children and adolescents, there are no unified definitions, and modified adult criteria have been suggested by many authors, despite major problems. Some pediatric disease states are at risk for premature cardiovascular disease, with clinical coronary events occurring very early in adult life. Survivors of specific pediatric cancer groups, particularly acute lymphocytic leukemia, central nervous system tumors, sarcomas, lymphomas, testicular cancer, and following bone marrow transplantation, may develop metabolic syndrome traits due to: hormonal deficiencies (growth hormone deficiency, thyroid dysfunction, and gonadal failure, drug or radiotherapy damage, endothelial impairment, physical inactivity, adipose tissue dysfunction, and/or drug-induced magnesium deficiency. In conclusion, some primary and secondary prevention remarks are proposed in order to reduce premature cardiovascular disease risk in this particular group of patients.Keywords: metabolic syndrome X, cardiovascular diseases, insulin resistance, obesity, growth hormone

  3. An overlap syndrome involving autoimmune hepatitis and systemic lupus erythematosus in childhood

    Institute of Scientific and Technical Information of China (English)

    Yusuf Usta; Figen Gurakan; Zuhal Akcoren; Seza Ozen

    2007-01-01

    We report a 12 years old female patient with an overlap syndrome involving autoimmune hepatitis (ALM) and systemic lupus erythematosus (SLE). The patient presented with jaundice, hepatosplenomegaly, malAlse, polyarthralgia, arthritis and butterfly rash on the face. Laboratory tests revealed severe liver dysfunction, Coombs positive hemolytic anemia and a positive ANA/ anti-dsDNA test. Renal biopsy showed class IIA kidney disease, while liver biopsy showed chronic hepatitis with severe inflammatory activity. The patient satisfied the international criteria for both SLE and ALM. Clinical symptoms and laboratory findings of SLE improved with high dose treatment with corticosteroids and azathioprine, however, remission of the liver disease could not be achieved. Repeat biopsy of the liver after three years of therapy revealed ongoing chronic hepatitis with high level of inflammatory activity. The present case indicates that children with liver dysfunction and SLE should be investigated for ALM. There is much diagnostic and therapeutic dilemma in patients with ALH-SLE overlap syndrome.

  4. Cyclosporin A acute encephalopathy and seizure syndrome in childhood: clinical features and risk of seizure recurrence.

    Science.gov (United States)

    Gleeson, J G; duPlessis, A J; Barnes, P D; Riviello, J J

    1998-07-01

    Cyclosporin A is associated with an acute encephalopathy including seizures and alterations in mental status, herein referred to as cyclosporin A acute encephalopathy and seizure syndrome. The clinical history, electroencephalogram (EEG), and neuroimaging findings in 19 children with cyclosporin A acute encephalopathy and seizure syndrome over a 10-year period were reviewed in order to delineate clinical characteristics, imaging features, and to determine the risk of seizure recurrence in this population. All 19 had motor seizures associated with other features of cortical and subcortical dysfunction. The acute mean cyclosporin A level was 342 microg/L, but was within the "therapeutic" range in five cases. Brain imaging by computed tomography (CT) or magnetic resonance imaging (MRI) in the acute or subacute phase revealed lesions characteristic of cyclosporin A toxicity in 14 cases. Acute EEG abnormalities were present in all and included epileptiform discharges or focal slowing. Patients were followed for a median of 49 months (1-9 years). Follow-up imaging (n = 10) showed lesion resolution or improvement in the majority while EEG (n = 10) had normalized in only three. Seizures recurred in six patients and only in those with persistent EEG or imaging abnormalities. No patient had a second episode of cyclosporin A associated neurotoxicity or seizure. It appears that a significant risk of seizure recurrence exists following cyclosporin A acute encephalopathy and seizure syndrome and primarily in those children with persistent EEG or imaging abnormalities.

  5. 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

  6. 吗替麦考酚酯联合低剂量激素治疗HBsAg阳性的成人微小病变性肾病综合征%Mycophenolate mofetil combined with low dose prednisone in the treatment for adults with minimal change nephrotic syndrome and concomitant HBsAg positive

    Institute of Scientific and Technical Information of China (English)

    李夏玉; 田炯; 陈江华; 李恒; 何强; 林维勤; 倪勤; 贺学林; 吴建永

    2009-01-01

    Objective To assess the safety and efficacy of mycophenolate mofetil (MMF) combined with low dose corticosteroid in the treatment of adults with minimal change nephrotic syndrome and concomitant HBsAg positive (MCNS-HBsAg). Methods Thirty adults with MCNS-HBsAg were enrolled in this prospective study and were assigned to two groups. The MMF group (n=14) received low dose of prednisone combined with MMF (MMF 1.0 to 2.0 g/d patients of Pred group versus 35.7% patients of MMF group. 43.8% patients of Pred group versus 21.4% patients of MMF group received lamivudine therapy. Elevation of alanine aminotransferase(ALT) ocurred in 50% patients of Pred group and 28.6% patients of MMF group. The complete remission (CR) rate after 24 weeks treatment was 11/14 in Pred group versus 10/12 in MMF group. 6/11 patients of the Pred group and 4/10 patients of the MMF group who achieved CR experienced relapses during follow-up. Conclusions Use of MMF combined with low dose prednisone is as effective as conventional prednisone regimen in treating adults with MCNS-HBsAg. The MMF protocol seems to be superior in HBV reactivation to conventional prednisone protocol.%目的 探讨吗替麦考酚酯(MMF)联合低剂量糖皮质激素方案治疗HBsAg阳性的成人微小病变性.肾病综合征的疗效及安全性.方法 前瞻性地选择HBsAg阳性、HBeAg阴性及血清HBV-DNA<1000拷贝/ml的成人微小病变性肾病综合征患者30例,分成激素组(16例)及MMF组(14例).激素组接受常规激素治疗方案(泼尼松片,1 mg·kg-1·d-1);MMF组接受低剂量激素(泼尼松片,0.5 mg·kg-1·d-1)联合MMF 1.0~2.0 g/d.结果 激素组和MMF组乙肝病毒激活发生比例分别为62.5%及35.7%,其中接受拉米呋定治疗分别为43.8%及21.4%;谷丙转氨酶升高发生比例分别为50.0%及28.6%.激素组及MMF组的完全缓解比例分别为11/14和10/12,两组复发比例分别为6/11和4/10.结论 与常规激素治疗方案比较,MMF联合低剂量糖皮质激素

  7. 彩色多普勒血流检测对原发性肾病综合征并肾间质损害的评估价值%THE VALUE OF COLOR DOPPLER FLOW MEASUREMENT IN THE ASSESSMENT OF PATIENTS WITH PRIMARY NEPHROTIC SYNDROME COMPLICATING RENAL INTERSTITIAL DAMAGE

    Institute of Scientific and Technical Information of China (English)

    于宁; 禹静; 杨雪

    2012-01-01

    Objective To investigate the value of color Doppler flow measurement in evaluation of primary nephrotic syndrome (PNS) complicating renal interstitial impairment. Methods Fifty-one PNS patients, according to the degree of impairment of renal interstitium. Were divided into mild-moderate-and severe-damage groups, and 25 healthy individuals served as controls. The blood stream parameters of segmental renal artery (SRA) and interlobar artery (IRA) were tested in all subjects. Re-sults In moderate-damage group, end-diastolic velocity (Vmin) of IRA stepped down, and the resistance index (RI) elevated, the differences were significant versus that in the control and mild-damage groups (F=5. 70-15.10,q=4. 49 - 5. 67,P<0.05)j the differences between severe-damage group and other groups were significant in terms of decreased peak velocity in systole and the end-diastolic velocity, and increased pulsed index (q=2. 88 - 7. 64.P<0. 05). Conclusion The extent of renal interstitial impairment is an important factor that influences the therapeutic efficacy and prognosis of PNS. Color Doppler renal blood flow measurement can reflect the degree of impairment of renal interstitium, which is conducive to judgement of the condition of the patients and their prognosis.%目的 了解肾脏彩色多普勒血流检测对原发性肾病综合征并肾间质损害的评估价值.方法 将51例原发性肾病综合征病人根据肾间质损害程度分为轻度损害组、中度损害组、重度损害组,25例健康对照者作为对照组;检测所有受检者肾脏段动脉( SRA)、叶间动脉(IRA)血流参数.结果 中度损害组肾IRA舒张末期最低血流速度(Vmin)减慢、阻力指数(RI)增高,与对照组及轻度损害组比较差异有显著性(F=5.70~15.10,q=4.49~5.67,P<0.05);重度损害组与其他组比较肾脏各级动脉最大血血流速度度、最小血血流速度度明显减慢,RI明显增高,差异有显著性(q=2.88~7.64,P<0.05).结论 肾间质损

  8. [Electrodiagnostic criteria for childhood Guillain-Barre syndrome. Eight years' experience].

    Science.gov (United States)

    Lopez-Esteban, Pilar; Gallego, Isabel; Gil-Ferrer, Victoria

    2013-03-01

    INTRODUCTION. The Guillan-Barre syndrome is the most frequent case of acute flacid paralysis in children. The diagnostic criteria differ according to the demyelinating or axonal variant and the prevalence by geographical area. The electro-myographic study permits identifying variants, evaluating the prognosis and predicting the evolution, is in addition an objective tool for the monitoring. AIM. To describe the electromyographic characteristics of the Guillain-Barre syndrome evaluated in hospital and its classification by physiopathological pattern. PATIENTS AND METHODS. All the cases diagnosed between 2005 and 2012 are included. Studies of motor and sensitive nervous conduction and F waves in 14 girls and 11 boys between 1 and 13 years of age. RESULTS. 19 cases of acute inflammatory demyelinating polyneuropathy (AIDP) and five of acute motor axonal neuropathy (AMAN) were diagnosed. The electromyogram was performed between 1 and 30 days after the beginning of symptoms. In AIDP cases, multifocal demyelination, four of them with the preserved sural and 13 with alteration and absence of F wave were objectified. In the cases of AMAN, four had low amplitude potential and in one of them they were not evoked. CONCLUSIONS. The demyelinating form of the illness is the most frequent although the high number of AMAN cases stands out, probably related to the population object of study. The evolution was favorable in three cases of motor axonal neuropathy and in 15 accute demyelinating polyneuropathy. In four cases the symptoms became chronic; three of them with persistent demyelination a similar occurrence in other studies with children.

  9. Acute Childhood Cardiorenal Syndrome and Impact of Cardiovascular Morbidity on Survival

    Directory of Open Access Journals (Sweden)

    Wasiu A. Olowu

    2011-01-01

    Full Text Available Cardiorenal syndrome (CRS clinical types, prevalence, aetiology, and acute cardiovascular morbidity impact on the outcome of acute kidney function perturbation were determined. Forty-seven of 101 (46.53% patients with perturbed kidney function had CRS. Types 3 and 5 CRS were found in 10 and 37 patients, respectively. Type 3 CRS was due to acute glomerulonephritis (AGN; =7, captopril (=1, frusemide (=1, and hypovolaemia (=1. Malaria-associated haemoglobinuria (=20, septicaemia (=11, lupus nephritis (=3, tumour lysis syndrome (=2, and acute lymphoblastic leukaemia (=1 caused Type 5 CRS. The cumulative mortality in hypertensive CRS was similar to nonhypertensive CRS (51.4% versus 40.9%; =.119. Mortality in CRS and non-CRS was similar (45.7% versus 24.5%; =.053. Type 5 survived better than type 3 CRS (66.7% versus 12.5%; =.001. Risk factors for mortality were Type 3 CRS (=.001, AGN-associated CRS (=.023, dialysis requiring CRS (=.008, and heart failure due to causes other than anaemia (=.003. All-cause-mortality was 34.2%. Preventive measures aimed at the preventable CRS aetiologies might be critical to reducing its prevalence.

  10. 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

  11. [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.

  12. Expanding the phenotypic profile of Kleefstra syndrome: A female with low-average intelligence and childhood apraxia of speech.

    Science.gov (United States)

    Samango-Sprouse, Carole; Lawson, Patrick; Sprouse, Courtney; Stapleton, Emily; Sadeghin, Teresa; Gropman, Andrea

    2016-05-01

    Kleefstra syndrome (KS) is a rare neurogenetic disorder most commonly caused by deletion in the 9q34.3 chromosomal region and is associated with intellectual disabilities, severe speech delay, and motor planning deficits. To our knowledge, this is the first patient (PQ, a 6-year-old female) with a 9q34.3 deletion who has near normal intelligence, and developmental dyspraxia with childhood apraxia of speech (CAS). At 6, the Wechsler Preschool and Primary Intelligence testing (WPPSI-III) revealed a Verbal IQ of 81 and Performance IQ of 79. The Beery Buktenica Test of Visual Motor Integration, 5th Edition (VMI) indicated severe visual motor deficits: VMI = 51; Visual Perception = 48; Motor Coordination < 45. On the Receptive One Word Picture Vocabulary Test-R (ROWPVT-R), she had standard scores of 96 and 99 in contrast to an Expressive One Word Picture Vocabulary-R (EOWPVT-R) standard scores of 73 and 82, revealing a discrepancy in vocabulary domains on both evaluations. Preschool Language Scale-4 (PLS-4) on PQ's first evaluation reveals a significant difference between auditory comprehension and expressive communication with standard scores of 78 and 57, respectively, further supporting the presence of CAS. This patient's near normal intelligence expands the phenotypic profile as well as the prognosis associated with KS. The identification of CAS in this patient provides a novel explanation for the previously reported speech delay and expressive language disorder. Further research is warranted on the impact of CAS on intelligence and behavioral outcome in KS. Therapeutic and prognostic implications are discussed.

  13. Predictors of Relapse in Adult-Onset Nephrotic Minimal Change Disease.

    Science.gov (United States)

    Lee, Hajeong; Yoo, Kyung Don; Oh, Yun Kyu; Kim, Dong Ki; Oh, Kook-Hwan; Joo, Kwon Wook; Kim, Yon Su; Ahn, Curie; Han, Jin Suk; Lim, Chun Soo

    2016-03-01

    Minimal change disease (MCD) is a well-known benign primary glomerulonephritis because of its distinct rare tendency to progress to end-stage renal disease. However, factors associated with relapse in adults are not well known. We aimed to identify predictors of relapse in adult-onset MCD patients.A retrospective cohort of 195 patients with adult-onset primary MCD with nephritic syndrome and disease onset between 1979 and 2013 was followed up for >12 months. The number of relapses was counted and predictors of relapse were analyzed.A total of 195 patients were included. Median age at diagnosis was 38 years (IQR, 23-53 years) and 113 (57.9%) were men. During 81 months (IQR, 44-153 months) of follow-up, 92% of patients achieved remission after initial treatment. However, only 60 (32.8%) did not experience a relapse and 11 patients failed to remit. Among the remaining 124 patients, 65 experienced a relapse once or twice and 59 experienced a relapse more than twice. Younger onset age, increased severity of nephrotic features such as lower serum albumin levels and higher cholesterol level were associated with relapse. Interestingly, the grade of mesangial proliferation was lower in patients who experienced a relapse. Initial combined treatment with corticosteroids (CS) and cyclophosphamide reduced the number of relapses. In addition, patients with shorter treatment duration tended to experience relapse more often. Multivariate analysis showed that younger onset age, combined mesangial proliferation, initial treatment regimen, and treatment duration were independent risk factors for relapse. Progression to end-stage renal disease was developed in only a patient.In conclusion, more than two-thirds of adult-onset nephrotic MCD patients experienced relapse, although their renal progression was rare. Younger onset age, CS without cyclophosphamide treatment, and shorter treatment duration were independent risk factors for relapse in adult-onset MCD patients.

  14. Involvement of the fractalkine pathway in the pathogenesis of childhood hemolytic uremic syndrome.

    Science.gov (United States)

    Ramos, María Victoria; Fernández, Gabriela C; Patey, Natasha; Schierloh, Pablo; Exeni, Ramón; Grimoldi, Irene; Vallejo, Graciela; Elías-Costa, Christian; Del Carmen Sasiain, Maria; Trachtman, Howard; Combadière, Christophe; Proulx, François; Palermo, Marina S

    2007-03-15

    Thrombotic microangiopathy and acute renal failure are cardinal features of postdiarrheal hemolytic uremic syndrome (HUS). These conditions are related to endothelial and epithelial cell damage induced by Shiga toxin (Stx) through the interaction with its globotriaosyl ceramide receptor. However, inflammatory processes contribute to the pathogenesis of HUS by sensitizing cells to Stx fractalkine (FKN), a CX(3)C transmembrane chemokine expressed on epithelial and endothelial cells upon activation, is involved in the selective migration and adhesion of specific leukocyte subsets to tissues. Here, we demonstrated a selective depletion of circulating mononuclear leukocytes expressing the receptor for FKN (CX(3)CR1) in patients with HUS. We found a unique phenotype in children with HUS distinct from that seen in healthy, uremic, or infected controls, in which monocytes lost CX(3)CR1, down-modulated CD62L, and increased CD16. In addition, the CD56(dim) natural killer (NK) subpopulation was decreased, leading to an altered peripheral CD56(dim)/CD56(bright) ratio from 10.0 to 4.5. It is noteworthy that a negative correlation existed between the percentage of circulating CX(3)CR1(+) leukocytes and the severity of renal failure. Finally, CX(3)CR1(+) leukocytes were observed in renal biopsies from patients with HUS. We suggest that the interaction of CX(3)CR1(+) cells with FKN present on activated endothelial cells may contribute to renal injury in HUS.

  15. Role of rufinamide in the management of Lennox-Gastaut syndrome (childhood epileptic encephalopathy).

    Science.gov (United States)

    Kluger, Gerhard; Bauer, Bettina

    2007-02-01

    Rufinamide, a triazole derivative that is structurally distinct from currently marketed antiepileptic drugs (AEDs), is in development for the adjunctive treatment of Lennox-Gastaut syndrome (LGS) in children and adults. Rufinamide is well absorbed after oral administration, demonstrates low protein binding, and is metabolized by enzymatic hydrolysis without involvement of cytochrome P450 enzymes, conferring a low drug interaction potential. In a randomized, double-blind trial involving 138 adult and pediatric patients with LGS, compared with placebo, rufinamide 45 mg/kg/day resulted in significantly superior reductions in drop attacks (median change -42.5% vs +1.4% with placebo) and total seizures (-32.1% vs -11.7% with placebo), accompanied by significantly higher responder rates. These results are comparable with findings reported for other AEDs in randomized, controlled clinical trials in patients with LGS. Rufinamide produced statistically significant seizure reduction which was maintained during long-term therapy and accompanied by good tolerability. The most frequently reported adverse events from a pooled safety database evaluating short- and long-term therapy were headache (22.9% and 29.5%), dizziness (15.5% and 22.5%) and fatigue (13.6% and 17.7%). Rufinamide therefore presents a favorable efficacy and tolerability profile and is a promising candidate for the adjunctive therapy of LGS.

  16. Long-term Impact of Temporal Sequence from Childhood Obesity to Hyperinsulinemia on Adult Metabolic Syndrome and Diabetes: The Bogalusa Heart Study

    Science.gov (United States)

    Zhang, Tao; Zhang, Huijie; Li, Ying; Li, Shengxu; Fernandez, Camilo; Bazzano, Lydia; He, Jiang; Xue, Fuzhong; Chen, Wei

    2017-01-01

    This study aims to delineate the temporal relations between body mass index (BMI) and insulin in childhood and their impact on adult metabolic syndrome (MetS) and type 2 diabetes mellitus (T2DM).The longitudinal cohort consisted of 609 whites and 339 blacks who had BMI and fasting insulin measured twice in childhood (mean age = 10.5 years at baseline and 15.9 years at follow-up). Incident MetS and T2DM were identified in adulthood (mean age = 30.5 years). Cross-lagged panel and mediation analysis models were used. After adjusting for age, race, gender, and follow-up years, the cross-lagged path coefficient of BMI → insulin (β = 0.326, p < 0.001) was significantly greater than that of insulin → BMI (β = −0.023, p = 0.207) in childhood, with p < 0.001 for the difference in βs. The path coefficient for BMI → insulin was significantly greater in MetS than in non-MetS groups (0.510 vs 0.190, p < 0.001), and greater in hyperglycemia than in normoglycemia groups (0.503 vs 0.285, p = 0.026). The mediation effect of childhood insulin on the BMI-MetS and BMI-hyperglycemia associations was estimated at 19.2% (p < 0.001) and 18.3% (p < 0.001), respectively. These findings provide evidence that higher BMI levels precede hyperinsulinemia during childhood, and this one-directional relation plays a significant role in the development of MetS and T2DM in adult life. PMID:28230104

  17. Neuroanatomical phenotype of Klinefelter syndrome in childhood: a voxel-based morphometry study.

    Science.gov (United States)

    Bryant, Daniel M; Hoeft, Fumiko; Lai, Song; Lackey, John; Roeltgen, David; Ross, Judith; Reiss, Allan L

    2011-05-04

    Klinefelter syndrome (KS) is a genetic disorder characterized by a supernumerary X chromosome. As such, KS offers a naturally occurring human model for the study of both X-chromosome gene expression and androgen on brain development. Previous neuroimaging studies have revealed neuroanatomical variations associated with KS, but have differed widely with respect to subject inclusion criteria, including mosaicism, pubertal status, and history of testosterone replacement therapy (TRT), all factors likely to influence neurodevelopment. We conducted a voxel-based morphometry study of regional gray and white matter (GM and WM, respectively) volumes in 31 KS males (mean age, 9.69 ± 1.70 years) and 36 typically developing (TD) male controls (10.99 ± 1.72 years). None of the participants with KS had received TRT, and all were prepubertal and had nonmosaic 47,XXY karyotypes. After controlling for age, males with KS showed trends (0.05 < p < 0.10) for significantly reduced total gray matter volume (TGMV) and total white matter volume (TWMV), relative to TD males. After controlling for TGMV and age, the KS group had significantly increased sensorimotor and parietal-occipital GM and significantly reduced amygdalar, hippocampal, insular, temporal, and inferior frontal GM relative to TD controls. After controlling for TWMV and age, the KS group had significantly increased left parietal WM as well as significantly reduced frontal and temporal WM. These findings are indicative of a characteristic prepubertal neuroanatomical phenotype that may be associated with cognitive-behavioral features of KS. This work offers new insight into the relationships among X-chromosome gene expression, neuroanatomy, and cognitive-behavioral functions impaired in KS, including language and attention.

  18. Clinical, electrophysiological subtypes and antiganglioside antibodies in childhood Guillain-Barré syndrome

    Directory of Open Access Journals (Sweden)

    Meena A Kannan

    2011-01-01

    Full Text Available Background: Guillain-Barré syndrome (GBS has been the most common cause of flaccid paralysis in children after the decline in the incidence of poliomyelitis. There are not any published data from the Indian subcontinent documenting electrophysiological patterns and antiganglioside antibodies in pediatric GBS. Materials and Methods: The study population included children with GBS referred for electrodiagnostic evaluation and also children with GBS admitted to our institute between August 2006 and July 2007. Nerve conduction studies were done to determine GBS subtypes and serum antiganglioside antibodies were measured using enzyme-linked immunosorbent assay (ELISA. Clinical and electrophysiological features were correlated with antiganglioside antibody results. Results: Of the 43 (male to female ratio = 2.1:1 children studied, 97.6% had motor weakness, 76.7% had cranial nerve palsies, 13.9% had autonomic disturbances and respiratory paralysis was found in 9.3% children. Antecedent illness was recorded in 69.8% children. The GBS subtype distribution was as follows: acute inflammatory demyelinating polyradiculoneuropathy (AIDP in 21 (48.8%, acute motor axonal neuropathy (AMAN in 19 (44.2%, and 3 (6.9% children were unclassified. The severity of illness was similar in both AMAN and AIDP subtypes and the recovery in both the subtypes was complete without any significant difference in the duration of recovery. Preceding diarrheal illness was more common in AMAN subtype as compared to AIDP subtype (57.9% vs. 4.7%, P = 0.007. Sensory symptoms were more common in AIDP subtype than in AMAN subtype (66.6% vs. 21%, P = 0.03}. The commonest ganglioside antibody was IgM GM2. Anti GM3 antibodies were exclusively seen in children with AMAN and IgG GD1b was significantly associated with (36.7 vs. 4%; P = 0.007 AMAN subtype. IgG GT1b was identified in 50% of patients with AIDP as compared to 22.7% in patients with AMAN. Conclusion: In this study, AMAN subtype

  19. Therapy-Related Myelodysplastic Syndrome Following Treatment for Childhood Acute Lymphoblastic Leukemia: Outcome of Patients Registered in the EWOG-MDS 98/06 Studies

    DEFF Research Database (Denmark)

    Strahm, Birgitte; Amann, Roland; De Moerloose, Barbara

    Objective: Therapy-related myelodysplastic syndrome (tMDS) following treatment of childhood acute lymphoblastic leukemia (ALL) is one of the most frequently observed secondary malignancies in survivors of childhood cancer. Allogeneic stem cell transplantation (SCT) is the only curative treatment....... This analysis was performed to asses the outcome of patients with tMDS following treatment for childhood ALL reported to the EWOG-MDS study group. Patients and Transplant Procedure: Forty-three patients (19 male/24 female) were diagnosed with tMDS between August 1989 and August 2009. The median age at diagnosis...... was 8.9 yrs (3.4–20.5). The median interval from diagnosis of ALL to the diagnosis of tMDS was 3.3 yrs (1.7–7.0). Five patients did not receive SCT and died due to progressive disease at a median of 5.6 mo after diagnosis. Thirty-eight patients were transplanted. One patient was excluded from...

  20. Síndrome mielodisplásica na infância Myelodysplastic syndromes in childhood

    Directory of Open Access Journals (Sweden)

    Luiz F. Lopes

    2006-09-01

    ,7%. Quanto à evolução clínica dos pacientes encontramos: óbito em 54,8% dos casos, remissão espontânea em 5,4% e 16,1% encontrava-se em tratamento conservador. Infecções em pacientes com SMD e naqueles que sofreram transformação leucêmica foram as causas mais freqüentes de óbito (58,8%. Propomos também neste artigo uma abordagem de diagnóstico em genética e biologia molecular voltada para os casos infantis assim como uma revisão de literatura sobre transplante de medula óssea para os casos pediátricos, além de outros aspectos que diferem da abordagem feita para pacientes adultos.The Brazilian Cooperative Study Group on Pediatric Myelodysplastic Syndromes (GCB-SMD-PED started in January 1997 with the goal of studying under 18-year-old patients with MDS or suspected MDS from all over the country. Some primary or secondary disorders are incorrectly called MDS. Because of this the GCB-SMD-PED is a referral group in the country to review and also to give diagnostic support (morphology, genetics, etc.. Some groups still use the FAB classification but two new classifications for pediatric cases have been published: one from the Sick Children's Hospital, University of Toronto, Canada the "CCC Classification" (category, cytology and cytogenetic, and the WHO pediatric classification by Hasle et al. Our proposal here is to present data from the 173 pediatric cases which were referred to the GCB-SMD-PED from 15 states (41 centers. From 1983 to 1997, 51 pediatric cases were registered as retrospective cases and from January 1998 to February 2003, 122 prospective cases were registered. From these 173 cases, 93 where confirmed as MDS. In 36.5% of them there was a transformation into acute leukemia with 82.3% as AML and 17.7% as ALL. The follow up showed that 54.8% died, 5.4% had spontaneous remission and 16.1% were in treatment with no chemotherapy (just transfusion or conservative approach. Infections were the primary cause of death (58.8%. Additionally, in this

  1. 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

  2. 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

  3. 原发性肾病综合征并发急性肾损伤患者血清及肾组织中NGAL的表达及意义%Expression of NGAL in serum and renal tissues of patients with primary nephrotic syndrome and acute kidney injury

    Institute of Scientific and Technical Information of China (English)

    刘静; 李绍梅; 薛雯; 温文龙; 杨林; 杨万霞; 王建荣

    2012-01-01

    目的:检测原发性肾病综合征(PNS)患者血清及肾组织中中性粒细胞明胶酶相关脂质运载蛋白(NGAL)的水平,探讨PNS合并急性肾损伤(AKI)时患者血清及肾组织中NGAL浓度的变化.方法:72例PNS患者根据病理结果分为:(1)PNS合并急性肾小管坏死(ATN)组 15例,其中微小病变(MCD)合并ATN 10例,系膜增生(MsPGN)合并ATN 5例;(2)PNS不合并ATN组 57例,其中MCD组 24例,膜性肾病(MN)组23例和MsPGN 组10例.15例健康体检者的血液及5例正常肾组织作为正常对照组.采用ELISA检测血清NGAL的水平;采用免疫组化法检测肾组织中NGAL的表达.结果:(1)PNS患者血清NGAL水平及肾组织中NGAL表达明显高于正常对照组(P0.05);血清中NGAL与肾组织NGAL的表达呈正相关(P<0.01).结论:血清NGAL可能作为判断原发性肾病综合征并发急性肾损伤的早期、无创、敏感指标.%AIM; To observe the changes of neutrophil gelatinase -associated lipocalin (NGAL) level in ser-um and renal tissues of the patients with primary nephrotic syndrome (PNS) and acute kidney injury (AKI). METH-ODS : Seventy - two PNS patients were selected in the study and divided into 2 groups according to the pathological results of renal biopsy. The patients in PNS + AKI group included 15 cases of PNS with acute tubular necrosis ( ATN) , in which there were 10 cases of minimal change disease ( MCD) with ATN and 5 cases of mesangial proliferative glomerulonephritis ( MsPGN) with ATN. The patients in PNS alone group included 57 cases of PNS without ATN. According to the pathologi-cal types, they were divided into MCD group (24 cases) , membranous nephropathy (MN) group (23 cases) and MsPGN group (10 cases). Serum samples from 15 healthy persons and 5 cases of normal renal tissues were used as controls. The serum levels of NGAL were detected by ELISA. The distribution and expression of NGAL in the renal tissues were observed by immunohistochemical method. RESULTS: ( 1) The serum

  4. 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

  5. 高效液相色谱法检测肾病综合征患者泼尼松血药浓度及其临床意义%HPLC Determination of Prednisone in Serum of Nephrotic Syndrome and its Clinical Significance

    Institute of Scientific and Technical Information of China (English)

    夏运成; 张旭光; 彭灿辉; 李铮; 彭佑铭

    2011-01-01

    observed in 10 ~ 500 μg/L range for prednisone, the detection limit was 5 mg/ml.The serum concentration of NS group was different from that in the control group( P < 0.05 ), there was no significant difference between the females and males groups( P >0.05 ) , the serum concentration of hormone resistant group was lower significantly compared with hormone sensitive group( P < 0.05 ).Conclusion: This is an accurate and convenient method to determine the concentration of PS in serum , it can monitor the plasma concentrations of PS in patients with long - term PS treatment and nephrotic syndrome, and help to know Individualized differences, normalize the preparation method,lt holds significant implications in the Pharmacodynamic Study.

  6. 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

  7. Spectrum and drug sensitivity of pathogenic bacteria in children with nephrotic syndrome complicated by urinary tract infection: an analysis of 97 cases%97例肾病综合征合并尿路感染患儿的病原菌分布及药敏分析

    Institute of Scientific and Technical Information of China (English)

    宋少娜; 张碧丽; 王文红; 张瑄

    2012-01-01

    目的 探讨肾病综合征(NS)合并尿路感染(UTI)患儿的菌群分布及其药物敏感性特点,指导临床合理应用抗生素.方法 对2011年1~12月住院的97例NS合并UTI患儿(初发53例,复发44例)的病原菌分布特点及其药敏情况进行回顾性分析.结果 NS患儿合并UTI的发生率为36.5%,其中NS复发患儿UTI的发生率高于初发者(44.0% vs 31.9%,P<0.05),临床表现以无症状菌尿为主.病原菌分析示肠球菌比例最高(50.5%),其中屎肠球菌29.4%,粪肠球菌21.1%.其次为革兰阴性菌大肠埃希菌(15.6%)、肺炎克雷伯菌(14.7%).肠球菌对呋喃妥因、万古霉素及利奈唑胺的敏感性较高,对四环素、莫西沙星耐药率高;屎肠球菌和粪肠球菌多重耐药菌株的检出率分别为72%和17% (P <0.05).大肠埃希菌、肺炎克雷伯菌对丁胺卡那霉素、亚胺培南、哌拉西林/他唑巴坦敏感性较高.产超广谱β-内酰胺酶( ESBLs)革兰阴性菌的检出率为25%,产ESBLs革兰阴性菌对亚胺培南、丁胺卡那霉素及哌拉西林/他唑巴坦的敏感性为100%,对氨苄西林、头孢唑林及头孢曲松几乎无敏感性.结论 复发NS患儿较初发者更易发生UTI;肠球菌已成为NS患儿合并UTI的主要致病菌,且耐药现象严重,其中屎肠球菌多呈多重耐药.%Objective To investigate the spectrum and drug sensitivity of pathogenic bacteria in children with nephrotic syndrome (NS ) complicated by urinary tract infection (UTI). Methods A retrospective analysis was performed on the spectrum and drug sensitivity of pathogenic bacteria in 97 children with NS complicated by UTI, who hospitalized from January to December, 2011. Results The incidence of UTI in children with NS was 36. 5%. It was significantly more common in children with recurrent NS than in those with primary NS (44. 0% vs 31.9% ; P < 0. 05 ) . These cases mainly presented with asymptomatic bacteriuria. Enterococcus was the most

  8. A retrospective analysis of CTX or MMF in the treatment of children refractory nephrotic syndrome%环磷酰胺与霉酚酸酯治疗小儿难治性肾病综合征47例回顾性分析

    Institute of Scientific and Technical Information of China (English)

    孟莲花

    2014-01-01

    Objective To compare the clinical effects of CTX and MMF on children with refractory nephrotic syndrome (RNS). Methods The clinical data of children with RNS admitted in the 159th central hospital of Jinan military region from January 2004 to January 2009 were collected. According to different medications,the patients were divided into the CTX group and MMF group. The remission rate,urine protein clearance time,renal function changes,recurrence within 3 years and adverse reactions were compared between the two groups. Results The study ultimately included 47 cases,with 21 cases in CTX group and 26 cases in MMF group. There were no significant differences in the gender,age,disease duration between the two groups. The complete remission rate in the MMF group and CTX group at the end of treatment was 28. 57% and 42. 31% respectively, but the difference was not significant(χ2 =0. 949,P=0. 330). And the overall remission of the two groups has no significant difference(Z= -0. 538,P=0. 590). After treatment,the indicators of kidney function improved compared with those before treatment(P﹤0. 05). But the levels of 24 h urine protein,Alb,Scr of the MMF group were superior to those of the CTX group (P﹤0. 05). The urine protein clearance time was(23. 9 ± 12. 36)days in the MMF group,and(73. 67 ± 40. 33)days in the CTX group,the difference was significant( P=0. 002). During the 3 years after the treatment,8 patients in CTX group relapsed, and 6 patients in MMF group relapsed,the difference was not significant(χ2 = 1. 253,P=0. 263). During the treatment,both groups showed adverse reactions,and the main adverse reactions were anorexia,nausea and vomiting,but there were no serious adverse reactions. Conclusion In the small sample clinical study,MMF is superior to CTX in the urine protein clearance time, improving the renal function of children with RNS,but both of MMF and CTX can alleviate the disease,and the complete remis-sion rates,recurrence rates and incidences of

  9. 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.

  10. Kindler syndrome

    Directory of Open Access Journals (Sweden)

    Kaviarasan P

    2005-01-01

    Full Text Available Kindler syndrome is a rare autosomal recessive disorder associated with skin fragility. It is characterized by blistering in infancy, photosensitivity and progressive poikiloderma. The syndrome involves the skin and mucous membrane with radiological changes. The genetic defect has been identified on the short arm of chromosome 20. This report describes an 18-year-old patient with classical features like blistering and photosensitivity in childhood and the subsequent development of poikiloderma. The differential diagnosis of Kindler syndrome includes diseases like Bloom syndrome, Cockayne syndrome, dyskeratosis congenita, epidermolysis bullosa, Rothmund-Thomson syndrome and xeroderma pigmentosum. Our patient had classical cutaneous features of Kindler syndrome with phimosis as a complication.

  11. 糖皮质激素诱导胰岛素样生长因子1降低对原发性肾病综合征患者骨代谢的影响%Impact of the reduction of insulin-like growth factor 1 induced by glucocorticoid therapy on bone metabolism in primary nephrotic syndrome patients

    Institute of Scientific and Technical Information of China (English)

    王玲; 袁伟杰; 谷立杰; 尚明花

    2011-01-01

    ) therapy and to explore the effect of its change on bone metabolism in primary nephrotic syndrome (PNS) patients.Methods A total of 39 PNS patients with mean age of (36.73±12.15) years received GC therapy were selected from January 2008 to August 2009 in our hospital.Serum IGF-1,albumin,calcium,phosphorus,parathormone (PTH),25hydroxy vitamin D3,bone gla protein (BGP),degradation products of C-terminal telopeptides of type I collagen (CTx),24-hour urinary protein excretion and the ratio of urinary calcium to creatinine were measured at five time points-before GC therapy,4 weeks,8 weeks,12 weeks and 24 weeks after the use of GC.BMD was also detected at the same time points.Correlations among indexes were analyzed by Pearson.Results Thirty-six PNS patients fulfilled the follow-up and had complete clinical data,while other 3 patients lost.After GC treatment,serum calcium and 25hydroxy vitamin D3 were significantly increased in a time-dependent manner and were negatively correlated with 24-hour urinary protein excretion (r=-0.749,r=-0.831,P<0.05,respectively).Serum BGP and IGF-1 were decreased after GC therapy in a time-dependent manner while CTx was significantly increased until week 12 after treatment (P<0.05).Compared with pre-treatment,BMD of various parts had no significant difference at week 4; BMD of lumbar spine (L1-L4) was significantly decreased until week 8 (P<0.05); BMD of femoral neck and femoral shaft was significantly decreased at week 24 (P<0.05).IGF-1 was positively correlated with BGP and BMD (r=0.896,r=0.495,P<0.05) and negatively correlated with serum CTx (r=-0.697,P<0.05 ).Conclusions Serum IGF-1 level decreases in a time-dependent manner after GC treatment,which is correlated to BGP,CTx and BMD.Glucocorticoid treatment affects bone metabolism through IGF1 pathway possably in patients with PNS.IGF-1 may be used as a new bone biochemical marker of glucocoritcoid - induced osteoporosis.

  12. 槐杞黄颗粒对原发性肾病综合征患儿免疫状态调节作用研究%The immune and regulatory function of haiqihuang particle in the patients with primary nephrotic syndrome

    Institute of Scientific and Technical Information of China (English)

    戴路露; 吴玉斌; 吴惠颖

    2012-01-01

    目的 观察槐杞黄颗粒辅助治疗原发性肾病综合征(PNS)患儿对淋巴细胞亚群、免疫球蛋白及感染次数的影响.方法 2008-01-01—2009-10-31中国医科大学附属盛京医院小儿肾脏风湿免疫科将住院的101例PNS患儿随机分为A组(62例)和B组(39例),选取正常体检儿童22名为正常对照组.A组采用糖皮质激素联合槐杞黄颗粒治疗,B组单独服用激素.分别于治疗前和治疗3个月、6个月检测患儿的淋巴细胞亚群、免疫球蛋白及记录感染次数、肾病复发次数和药物副反应.结果 (1)A、B组治疗前,CD8+高于正常对照组(P<0.05),CD4+、CD47CD8+和NK细胞均明显降低于正常对照组(P<0.05),IgA与IgG均低于正常对照组(P<0.05).(2)治疗3个月,A组(39例)与B组(30例)之间CD8+细胞差异无统计学意义,两组均明显高于正常对照组,差异有统计学意义(P<0.01);B组CD4+及CD4+/CD8+显著低于A组和正常对照组(P<0.05);A组与B组NK细胞均高于治疗前,B组低于同期A组(P<0.05);IgG明显升高但组间差异无统计学意义.(3)治疗6个月,A组(23例)、B组(9例)间各指标均差异无统计学意义,均低于正常对照组(P<0.01).(4)感染次数,A组发生上呼吸道感染6例次,肺炎2例次,肾病综合征复发4例次.B组发生上呼吸道感染10例次,肺炎3例次,泌尿系感染2例次,肾病综合征复发6例次.1例患儿服槐杞黄颗粒后出现较严重腹泻(排除感染性腹泻).结论 PNS患儿治疗前细胞、体液免疫功能降低、紊乱;槐杞黄颗粒辅助治疗过程中,可能通过提高NK细胞和T辅助淋巴细胞活性,减少感染并有减少肾病综合征复发的趋势,且副反应轻微.%Objective Observe the effect of Huaiqihuang particles on the lymphocyte populations, immunoglobins and the times of infection of patients with primary nephrotic syndrome(PNS). Methods Totally 101 patients first diagnosed with PNS were randomly divided into group A (62 cases) and

  13. Use of the Childhood Autism Rating Scale (CARS) for Children with High Functioning Autism or Asperger Syndrome

    Science.gov (United States)

    Mayes, Susan Dickerson; Calhoun, Susan L.; Murray, Michael J.; Morrow, Jill D.; Yurich, Kirsten K. L.; Cothren, Shiyoko; Purichia, Heather; Mahr, Fauzia; Bouder, James N.; Petersen, Christopher

    2012-01-01

    The authors of the "Childhood Autism Rating Scale" (CARS) state in the manual that the best cutoff score for distinguishing low functioning autism (LFA) from intellectual disability is 30 for children and 28 for adolescents and adults. This study determined that a cutoff score of 25.5 was most accurate in differentiating between high functioning…

  14. 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

  15. 肾病综合征患儿尿微量白蛋白及肾功能系列指标检测的临床价值%Clinical value of microalbuminuria and blood-renal function indexes in children with nephrotic syndrome

    Institute of Scientific and Technical Information of China (English)

    段文冰; 刘义庆; 宋晓斐; 王子娥; 陈兰兰; 赵万辉; 张培莉; 张炳昌

    2016-01-01

    临床治疗尿MA转为正常时,联合肾功能系列指标检查对临床判断有一定指导意义.%Objective To investigate the clinical value of microalbuminuria and blood-renal function indexes in children with nephrotic syndrome (NS).Methods One hundred and fourteen children with NS from January to December 2014 in Shandong Provincial Hospital Affiliated to Shandong University were enrolled as observation group which was divided into normal microalbuminuria group(52 cases) and abnormal microalbuminuria group (62 cases) according to the detection after treatment.Thirty healthy children with similar ages were selected as control group.Urine protein,microalbuminuria,urine immunoglobulin(Ig) G and blood-renal function indexes [albumin (ALB),blood urea nitrogen (BUN),serum creatinine (SCr),cystatin C (CysC),β2-microglobulin (β2-MG),retinol binding protein(RBP),complement C1q] were analyzed.Results ①In observation group,urine protein positive rate was 31.6% (36/114) and microalbuminuria positive rate was 54.4% (62/114);the difference was statistically significant(P < 0.05).②In normal microalbuminuria group,β2-MG,BUN,RBP were significantly lower than those in control group;urine IgG,BUN/SCr and C1q were significantly higher than those in control group[(1.48 ± 0.24) mg/L vs (1.84 ± 0.47) mg/L,(4.4 ± 1.5) mmol/L vs (4.6 ± 0.8) mmol/L,(29 ± 10)mg/L vs (33 ±7)mg/L,(4.4 ±3.3) mg/L vs (4.0 ±0.6)rmg/L,(122 ±65) vs (60 ± 10),(180 ± 22) mg/L vs (176 ± 13) mg/L] (P < 0.01 or P < 0.05);ALB,SCr,CysC showed no significant differences between groups(P >0.05).In abnormal microalbuminuria group,urine IgG,BUN,BUN/SCr,CysC,RBP and C1q were significantly higher than those in control group;ALB was significantly lower than those in control group [(28.7 ± 44.3) mg/L vs (4.0 ± 0.6) mg/L,(5.0 ± 1.9) mmol/L vs (4.6 ± 0.8) mmol/L,(129 ± 43) vs (60 ± 10),(0.98 ±0.24) mg/L vs (0.81 ± 0.14) mg/L,(35 ± 12) mg/L vs (33 ± 7) mg/L,(184 ± 25) mg/L vs (176

  16. Childhood Depressive Disorders

    DEFF Research Database (Denmark)

    Wesselhöft, Rikke Thaarup

    2016-01-01

    This case report describes a female patient diagnosed with Barraquer-Simons syndrome, a rare form of acquired partial lipodystrophy characterised by symmetrical loss of adipose tissue from face, neck, upper extremities and the trunk with onset in early childhood. Initial symptoms were seen...

  17. Long-term effects of oxandrolone treatment in childhood on neurocognition, quality of life and social-emotional functioning in young adults with Turner syndrome.

    Science.gov (United States)

    Freriks, K; Verhaak, C M; Sas, T C J; Menke, L A; Wit, J M; Otten, B J; de Muinck Keizer-Schrama, S M P F; Smeets, D F C M; Netea-Maier, R T; Hermus, A R M M; Kessels, R P C; Timmers, H J L M

    2015-03-01

    Turner syndrome (TS) is the result of (partial) absence of one X-chromosome. Besides short stature, gonadal dysgenesis and other physical aspects, TS women have typical psychological features. Since psychological effects of androgen exposure in childhood probably are long-lasting, we explored long-term psychological functioning after oxandrolone (Ox) therapy during childhood in adults with TS in terms of neurocognition, quality of life and social-emotional functioning. During the initial study, girls were treated with growth hormone (GH) combined with placebo (Pl), Ox 0.03 mg/kg/day, or Ox 0.06 mg/kg/day from the age of eight, and estrogen from the age of twelve. Sixty-eight women participated in the current double-blinded follow-up study (mean age 24.0 years, mean time since stopping GH/Ox 8.7 years). We found no effects on neurocognition. Concerning quality of life women treated with Ox had higher anxiety levels (STAI 37.4 ± 8.4 vs 31.8 ± 5.0, p=0.002) and higher scores on the depression subscale of the SCL-90-R (25.7 ± 10.7 vs 20.5 ± 4.7, p=0.01). Regarding social-emotional functioning, emotion perception for fearful faces was lower in the Ox-treated patients, without effect on interpersonal behavior. Our exploratory study is the first to suggest that androgen treatment in adolescence possibly has long-term effects on adult quality of life and social-emotional functioning. However, differences are small and clinical implications of our results seem limited. Therefore we would not recommend against the use of Ox in light of psychological consequences.

  18. An Extra X or Y Chromosome: Contrasting the Cognitive and Motor Phenotypes in Childhood in Boys with 47,XYY Syndrome or 47,XXY Klinefelter Syndrome

    Science.gov (United States)

    Ross, Judith L.; Zeger, Martha P. D.; Kushner, Harvey; Zinn, Andrew R.; Roeltgen, David P.

    2009-01-01

    Objective: The goal of this study was to contrast the cognitive phenotypes in boys with 47,XYY (XYY) karyotype and boys with 47,XXY karyotype [Klinefelter syndrome, (KS)], who share an extra copy of the X-Y pseudoautosomal region but differ in their dosage of strictly sex-linked genes. Methods: Neuropsychological evaluation of general cognitive…

  19. Fine-Motor Skill Deficits in Childhood Predict Adulthood Tic Severity and Global Psychosocial Functioning in Tourette's Syndrome

    Science.gov (United States)

    Bloch, Michael H.; Sukhodolsky, Denis G.; Leckman, James F.; Schultz, Robert T.

    2006-01-01

    Background: Most children with Tourette's syndrome (TS) experience a significant decline in tic symptoms during adolescence. Currently no clinical measures have been identified that can predict whose tic symptoms will persist into adulthood. Patients with TS have deficits on neuropsychological tests involving fine-motor coordination and…

  20. Psychological and behavioural aspects of patients with Turner syndrome from childhood to adulthood: a review of the clinical literature.

    Science.gov (United States)

    Christopoulos, P; Deligeoroglou, E; Laggari, V; Christogiorgos, S; Creatsas, G

    2008-03-01

    Turner syndrome (TS) is a chromosomal abnormality, which occurs in approximately one of every 2500 female births. Short stature, infertility, additional physical abnormalities, skeletal and medical problems may be present. Genetic, hormonal, and medical problems associated with TS are likely to affect psychosexual development of female adolescent patients, and thus influence their psychological functioning, behavior patterns, social interactions and learning ability. Although TS constitutes a chronic medical condition, with possible physical, social and psychological complications in a woman's life, hormonal and estrogen replacement therapy and assisted reproduction, are treatments that can be helpful for TS patients and improve their quality of life. Authors report on a review of the research literature clinical aspects of the syndrome as well as the beneficial effect of hormonal therapy in such patients.

  1. Neuro-behavioral profile and brain imaging study of the 22q13.3 deletion syndrome in childhood

    Energy Technology Data Exchange (ETDEWEB)

    Philippe, A.; Malan, V.; De Blois, M.C.; Colleaux, L.; Munnich, A. [Hop Necker Enfants Malad, Assistance Publ Hop Paris, Natl Inst Hlth and Med Res, Paris (France); Philippe, A.; De Blois, M.C.; Colleaux, L.; Munnich, A. [HopNecker Enfants Malad, Assistance Publ Hop Paris, Dept Genet, Paris (France); Boddaert, N. [Natl Inst Hlth and Med Res, Mixed Unit Res 0205, Orsay (France); Vaivre-Douret, L.; Robel, L.; Golse, B. [Hop Necker Enfants Malad, Assistance Publ Hop Paris, Dept Psychiat, Paris (France); Vaivre-Douret, L. [Univ Paris 10, Mixed Unit Res S0669, Univ Paris 05, Univ Paris 11, Paris 10 (France); Vaivre-Douret, L. [Assistance Publ Hop Paris, Dept Obstet et Gynaecol, Paris (France); Danon-Boileau, L. [Natl Ctr Sci Res, Mixed Unit Res 7114, Paris (France); Heron, D. [Hop La Pitie Salpetriere, Assistance Publ HopParis, Dept Genet, Paris (France)

    2008-07-01

    The 22q13.3 deletion syndrome (Online Mendelian Inheritance in Man No. 606232) is a neuro-developmental disorder that includes hypotonia, severely impaired development of speech and language, autistic-like behavior, and minor dysmorphic features. Although the number of reported cases is increasing, the 22q13.3 deletion remains under-diagnosed because of failure in recognizing the clinical phenotype and detecting the 22qter deletion by routine chromosome analyses. Our goal is to contribute to the description of the neuro-behavioral phenotype and brain abnormalities of this micro-deletional syndrome. We assessed neuro-motor, sensory, language, communication, and social development and performed cerebral MRI and study of regional cerebral blood flow measured by positron emission tomography in 8 children carrying the 22q13.3 deletion. Despite variability in expression and severity, the children shared a common developmental profile characterized by hypotonia, sleep disorders, and poor response to their environment in early infancy; expressive language deficit contrasting with emergence of social reciprocity from ages similar to 3 to 5 years; sensory processing dysfunction; and neuro-motor disorders. Brain MRI findings were normal or showed a thin or morphologically atypical corpus callosum. Positron emission tomography study detected a localized dysfunction of the left temporal polar lobe and amygdala hypoperfusion. The developmental course of the 22q13.3 deletion syndrome belongs to pervasive developmental disorders but is distinct from autism. An improved description of the natural history of this syndrome should help in recognizing this largely under-diagnosed condition. (authors)

  2. 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。

  3. 儿童期受虐与肠易激综合征关系的临床研究%A Clinical Study of Childhood Abuse on Irritable Bowel Syndrome

    Institute of Scientific and Technical Information of China (English)

    刘羽; 王彦彦; 纪菊英; 潘园园; 孙剑

    2016-01-01

    目的:探讨儿童期受虐在肠易激综合征(IBS)发生及患者精神心理状态、生活质量中所起的作用。方法:72例 IBS 患者(患者组)评定儿童期虐待问卷(CTQ),评估儿童期受虐情况,并与92例正常健康人(对照组)进行对照。 IBS 患者同时还评定抑郁自评量表(SDS)、焦虑自评量表(SAS)、肠易激综合征患者生活质量表(IBS -QOL)。结果:①患者组 CTQ 评分显著高于对照组,差异有统计学意义(t =3.590,P <0.001);患者组儿童期受虐发生率为44.4%,高于对照组的27.2%,差异有统计学意义(χ2=5.313,P <0.05);②与无儿童期受虐的 IBS 患者(40例)比较,有儿童期受虐的 IBS 患者(32例)SAS 评分显著较高,差异有统计学意义(t =2.099,P <0.05);③有儿童期受虐的 IBS 患者 QOL 评分显著高于无儿童期受虐的 IBS 患者,差异有统计学意义(t =‐2.223,P <0.05)。结论:儿童期受虐可能是 IBS 发生的社会心理学因素之一,并可能对 IBS 患者的心理状况、生活质量产生不良的影响。%Objective :To explore the clinical correlativity of childhood abuse and irritable bowel syndrome cause , psychological symptoms and quality of life .Methods :A clinical childhood abuse survey was conducted with childhood trauma questionnaire(CTQ)in 72 cases of patients with irritable bowel syndrome (case group)and 92 cases of health (control group) .All patients with irritable bowel syndrome were also assessed by self - rating depression scale (SDS) ,self - rating anxiety scale(SAS) ,and the irritable bowel syndrome quality of life questionnaire(IBS - QOL) . Results :Compared to controls ,patients with irritable bowel syndrome had higher scores of CTQ (t = 3 .590 ,P <0 .001) ,and higher prevalence of childhood abuse (27 .2% vs 44 .4% ,χ2 = 5 .313 ,P < 0 .05) .Irritable bowel syndrome patients with

  4. General Information about Mycosis Fungoides and the Sezary Syndrome

    Science.gov (United States)

    ... Childhood Hodgkin Lymphoma Treatment Childhood NHL Treatment Research Mycosis Fungoides and the Sézary Syndrome Treatment (PDQ®)–Patient Version General Information About Mycosis Fungoides and the Sézary Syndrome Go to Health ...

  5. Stages of Mycosis Fungoides and the Sezary Syndrome

    Science.gov (United States)

    ... Childhood Hodgkin Lymphoma Treatment Childhood NHL Treatment Research Mycosis Fungoides and the Sézary Syndrome Treatment (PDQ®)–Patient Version General Information About Mycosis Fungoides and the Sézary Syndrome Go to Health ...

  6. Treatment Options by Stage (Mycosis Fungoides and the Sezary Syndrome)

    Science.gov (United States)

    ... Childhood Hodgkin Lymphoma Treatment Childhood NHL Treatment Research Mycosis Fungoides and the Sézary Syndrome Treatment (PDQ®)–Patient Version General Information About Mycosis Fungoides and the Sézary Syndrome Go to Health ...

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

    Institute of Scientific and Technical Information of China (English)

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

    2009-01-01

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

  8. Childhood Schizophrenia

    Science.gov (United States)

    Childhood schizophrenia Overview By Mayo Clinic Staff Childhood schizophrenia is an uncommon but severe mental disorder in which children interpret reality abnormally. Schizophrenia involves a range of problems with thinking (cognitive), ...

  9. Childhood obesity

    OpenAIRE

    Wilkinson, Justine; Howard, Simon

    2006-01-01

    Childhood obesity has important consequences for health and wellbeing both during childhood and also in later adult life. The rising prevalence of childhood obesity poses a major public health challenge in both developed and developing countries by increasing the burden of chronic non-communicable diseases. Despite the urgent need for effective preventative strategies, there remains disagreement over its definition due to a lack of evidence on the optimal cut-offs linking childhood BMI to dis...

  10. Harlequin syndrome in childhood: case report Síndrome de Arlequim na infância: relato de caso

    Directory of Open Access Journals (Sweden)

    Juliano de Avelar Breunig

    2012-12-01

    Full Text Available Harlequin syndrome happens in only one side of the face. In the affected half, the face does not sweat or flush even with simulation. Arms and trunk could also be affected. This condition is induced by heat, exercise and emotional factors. The article reports a case of a nine-year-old female with a 3-year history of unilateral flushing and sweating after exercise; a brief literature review is included. Despite the rarity of this syndrome, dermatologists should recognize this condition and refer these patients to ophthalmological and neurological examination.A Síndrome de Arlequim ocorre em apenas um lado da face. No metade afetada, a face não produz suor ou flushing, mesmo estimulada. Braços e tórax raramente podem ser afetados. Esta condição é geralmente induzida por calor, exercícios e fatores emocionais. O artigo relata um caso de uma menina de 9 anos de idade com uma história de 3 anos de flushing e sudorese unilaterais no rosto após exercícios e inclui uma revisão da literatura. Apesar da raridade desta síndrome, dermatologistas devem reconhecer esta condição e encaminhar estes pacientes a um exame oftalmológico e neurológico.

  11. [Age-dependent visceral medicine: paediatric visceral medicine - visceral medical paediatrics - considerations on the short bowel syndrome in childhood].

    Science.gov (United States)

    Krause, H; Heiduk, M; Wachowiak, R; Till, H

    2013-08-01

    There are several reasons for the possible development of a short bowel syndrome, which, however, occurs only rarely. The main causes consist of extended intestinal resections in cases of congenital anomalies (e.g., gastroschisis, intestinal atresia or dysplasia) or ischaemic lesions due to a volvulus. In addition, an intestinal stoma at a more upper segment of the GI tract can result in the functional manifestation of a short bowel syndrome. The differentiation between temporary and persisting types is essential for initiation of an adequate treatment. Loss or exclusion of organic resorption area at the inner surface of the (small) intestine can be associated with numerous pathological consequences requiring treatment. As a principle consideration from the paediatric point of view, the potential of intestinal adaptation needs to be assessed. Basic conservative treatment options are parenteral and enteral nutrition regimens, in particular, to prevent complications (such as D-lactate acidosis). The main surgical approaches are the procedures called LILT (longitudinal intestinal lengthening and tailoring) according to Bianchi and STEP (serial transverse enteroplasty). The technique to create intestinal segments of antiperistalsis has been abandoned. Because of the encouraging results of intestinal transplantation, this novel treatment option has gained greater attention over the past few years and is now also an option for paediatric patients. The limiting factor and thus major complication is the central venous catheter for long-term treatment. Catheter-related complications are still the main reason for a considerable mortality in these children.

  12. Improved survival in patients obtaining remission of nephrotic range albuminuria in diabetic nephropathy

    DEFF Research Database (Denmark)

    Hovind, Peter; Tarnow, Lise; Rossing, Peter

    2004-01-01

    BACKGROUND: The level of albuminuria is related to progression of diabetic nephropathy, and patients with nephrotic range albuminuria have advanced renal structural changes and the fastest decline in glomerular filtration rate (GFR). We have previously demonstrated that the rate of decline in GFR...... risk of reaching the end point, 1.42 (1.08 to 1.87), P= 0.01. CONCLUSION: Our prospective study suggests that remission of nephrotic range albuminuria in type 1 diabetic patients, induced by aggressive antihypertensive treatment with and without ACE inhibitors, is associated with a slower progression...... in diabetic nephropathy and a substantially improved survival....

  13. [Non-autistic pervasive developmental disorders: Rett syndrome, disintegrative disorder and pervasive developmental disorder not otherwise specified

    NARCIS (Netherlands)

    Mercadante, M.T.; Gaag, R.J. van der; Schwartzman, J.S.

    2006-01-01

    The category "Pervasive Developmental Disorders" includes autistic disorder, Asperger's syndrome, Rett's syndrome, childhood disintegrative disorder, and a residual category, named pervasive developmental disorder not otherwise specified. In this review, Rett's syndrome and childhood disintegrative

  14. 小儿急性偏瘫综合征%Study on acute hemiplegia syndrome in childhood

    Institute of Scientific and Technical Information of China (English)

    王纪文

    2004-01-01

    小儿急性偏瘫综合征(acute hemiplegia syndrome in childhood.AHS)早在1897年即由Sigmund Freud详细描述,直到1960年以后才有较多报道。小儿急性偏瘫是由多种原因引起的以急性一侧肢体瘫痪为主要表现的临床综合征,目前该诊断名词倾向于只用于未找到特异原闪的急性偏瘫,其他则可用更为明确的诊断如:脑动脉血栓形成等代替,本文仍延用以往文献称谓,泛指上述情况。美国有学者调查15岁以下儿童中,脑卒中引起的急性偏瘫为2.48/万。国内尚缺乏流行病学方面的调查。其发病机制主要是脑血流灌注不足,导致局部脑组织缺血、坏死累及锥体束的功能。

  15. The Study on the Relationship between Serum Vascular Endothelial Growth Factor and Proteinuria in Adriamycin-induced Nephrotic Rats

    Institute of Scientific and Technical Information of China (English)

    ZHU; Zhonghua(

    2001-01-01

    [1]Carmeliet P Collen D.Molecular analysis of blood vessel formation and disease.Am J Physiol 1997 273 :H2091[2]Gleade J M Ebert B L Firth J D.Regulation of angiogenic growth factor expression by hypoxia transition metals and chelating agents.Am J Physiol 1995 268:C1362[3]Uchida K Uchida S.Glomerular endothelial cells in culture express and secrete vascular endothelial growth factor.AmJPhysiol 1994 266:F240[4]Simon M Grone H J Johren O.Expression of vascular endotheial growth factor and its receptors in human renal ontogenetis and adult kidney.Am J Physiol 1995 268:F81[5]Bailey E Bottomley M J.Vascular endothelial growth factor mRNA expression in minimal change membranous and diabetic nephrothy demonstrated by non-isotopic in situ hybridization.J Clin Pathol 1999 52:735[6]Nicholas J A Webb N J Waston C J.Circulation vascular endotheial growth factor is not increased during relapses of steroid-sensitive nephrotic syndrome.Kidney Int 1999 55:1063[7]Shulman K Rosen S Tognazzi K.Expression of VPF is altered in many glomerulous disease.J Am Soc Nephrol 1996 7:661

  16. 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.

  17. 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.

  18. Troyer Syndrome

    Science.gov (United States)

    ... atrophy of the hand muscles, developmental delays, fluctuating emotions, and short stature. Onset is typically in early childhood, and symptoms gradually worsen over time. Troyer syndrome is an autosomal recessive disorder (meaning that both parents must carry and pass on the defective gene ...

  19. Combination Chemotherapy With or Without Bone Marrow Transplantation in Treating Children With Acute Myelogenous Leukemia or Myelodysplastic Syndrome

    Science.gov (United States)

    2013-01-15

    Childhood Acute Erythroleukemia (M6); C