Thromboembolic Complications in Children with Nephrotic Syndrome

Hoyer, Peter F.; Gonda, S.; Barthels, Monika; Krohn, H. P.; Brodehl, J.

doi:10.1111/j.1651-2227.1986.tb10294.x

Cited by 143 publications

(112 citation statements)

References 18 publications

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“…An increased hepatic protein synthesis is not sufficient to compensate for the renal loss of the proteins which have molecular weight below 70000 daltons. However, it leads to a marked increase in the plasma proteins which have molecular weights of more than 100000 daltons, which are not filtered in selective proteinuria [8]. An anti-thrombin III deficiency [7] and various anomalies of the platelet function, as well as the release of different products (Adenosine di phosphate (ADP), thrombin, collagen and arachidonic acid (AA) )by the platelets have been linked to an increased thromboembolic phenomenon [9][10][11][12].…”

Section: Discussionmentioning

confidence: 99%

Platelet Functions and Coagulation Changes in Indian Children with Nephrotic Syndrome

Mittal

Aggarwal²,

Saluja³

et al. 2013

JCDR

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Introduction: Only little is known on the effect of the platelet function in the paediatric nephrotic syndrome. The earlier studies which had been done on hypercoagulability have mainly featured the secondary forms of the nephrotic syndrome and the data on the minimal change type of disease is limited.We therefore, made an effort to study the platelet functions and the coagulation profile in children with the nephrotic syndrome,to find the relationship between the steroid response and the coagulation profile, and to look for the correlation between thromboembolism and the hypercoagulable states.Methodology: Twenty nine children with the steroid responsive nephrotic syndromewere studied to see the platelet aggregation and the coagulation parameters and their response to the steroid therapy. Doppler studies were done for the renal vein and the inferior vena cava (IVC) thrombus.Results: It was seen that an increased aggregability of the platelets occurred with Adenosine diphosphate (ADP) and collagen (out of the four agonists, ADP, Collagen, Ristocetin and Arachidonic acid) which were used as agonists for the assay.We also observed that the Partial thromboplastin time (PTT) had become prolonged and a significant decline in the high values of the procoagulant proteins (Protein C and Protein S) was seen after the steroid therapy and when the children went into remission. These findings were suggestive of a reversibility of the changes in the steroid responsive nephrotic syndrome with the steroid therapy.One child was found to have thrombosis of the inferior vena cava (IVC) on Doppler studies, which resolved with treatment subsequently.Conclusions: An increased platelet aggregability contributes to the hypercoagulable states, that may increase the incidence of thrombosis in such patients. Although the incidence of such complications is very low, in a given child with the hypercoagulable states, Doppler may be used to look for any evidence of a latent thrombus and, an early intervention could be instituted. A change in the coagulation parameters points to the reversibility of the changes which are produced in a diseased state.

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Section: Discussionmentioning

confidence: 99%

Platelet Functions and Coagulation Changes in Indian Children with Nephrotic Syndrome

Mittal

Aggarwal²,

Saluja³

et al. 2013

JCDR

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“…Pulmonary embolic episodes are silent [63]. Many pulmonary embolisms in children with NS should be suspected if they present with pulmonary or cardiovascular symptoms and can be confirmed by angiography or radioisotope scanning [64].…”

Section: (Ii) Thromboembolismmentioning

confidence: 99%

Steroid Sensitive Nephrotic Syndrome in Children

Bhimma¹

2014

J Nephrol Ther

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Nephrotic Syndrome (NS) is one of the most frequent glomerular diseases seen in children. Children who go into complete remissin following treatment with corticosteroids are classified as having "steroid sensitive" NS. In developed countries over 80% of children with idiopathic NS have steroid sensitive disease although response to steroids is somewhat tempered in developing countries, especially in black children, the majority of whom have steroid resistant disease.The exact pathogenesis of this condition remains elusive. Podocyte injury and proteinuria are the two main issues in the pathogenesis. Recent studies suggest alterations in both innate and adaptive immune responses. There is release of cytokines by T-cells as well as a strong contribution of B-cell immunity. Genetic studies have reported human leucocyte antigen (HLA) class II antigens DR and DQ associations linked to steroid sensitive NS and in small case studies, single gene mutations e.g. PLCE1 although to date no homozygous mutations in NPHS1 or NPHS2 and WT-1 genes have been reported.Most children with steroid sensitive NS have multiple relapses and a significant percentage also develop steroid dependent NS. These children receive multiples courses of steroids and are at high risk of developing steroid toxicity. Patient with frequent relapses who develop steroid dependency thus require alternative treatment. Steroids sparing agents used include levamisole, cyclophosphamide, mycophenolate mofetil (MMF), calcineurin inhibitors (cyclosporine and tacrolimus), rituximab and vincristine. The steroid-sparing effects of these agents have greatly reduced the adverse effects seen with long-term use of steroids.Despite the wide arsenal of agents available, therapy needs to be individualised to achieve optimal care of each child. More randomised controlled trials are required to evaluate the therapeutic and economic efficacy of this agent, define criteria for selection of patients for use of particular agents and to determine the safety profile of these drugs in children.This article reviews the epidemiology, pathogenesis, clinical presentation, diagnosis, complications, management and long term outcome of children with steroid sensitive NS.

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“…Vascular occlusions (pulmonary thromboembolism and arterial or venous thrombosis) have been observed in both adults and children. 1,3 The reported incidence is lower in children despite more marked coagulation abnormalities. 2 However, the possibility of clinically silent thrombosis in children cannot be ruled out.…”

Section: Discussionmentioning

confidence: 99%

“…2 However, the possibility of clinically silent thrombosis in children cannot be ruled out. 1 These conditions result, most probably, from the conjunction of hypoalbuminemia and hyperlipemia with several changes in blood coagulation, fibrinolysis, and platelet functions. 5,51 Marked lipid abnormalities, such as elevation of lipoproteins containing apo B, ie, VLDL, LDL, and Lp(a), have been previously reported 52 and were confirmed by results of the present studies.…”

Section: Discussionmentioning

confidence: 99%

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Effect of Individual Plasma Lipoprotein(a) Variations In Vivo on Its Competition With Plasminogen for Fibrin and Cell Binding

Soulat

Loyau

Baudouin

et al. 2000

ATVB

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Abstract-Simultaneous natural changes in lipoprotein(a) [Lp(a)] and plasminogen occur in the nephrotic syndrome and offer a unique opportunity to investigate their effects on plasminogen activation under conditions fashioned in vivo. Plasminogen, Lp(a), and apolipoprotein(a) in plasma were characterized, and their competitive binding to carboxyterminal lysine residues of fibrin and cell membrane proteins was determined in nephrotic children during a flare-up of the disease (61 cases) and after 6 weeks (33 cases) and 6 months (42 cases) of remission. Low plasminogen concentrations (median 1.34 mol/L, range 0.39 to 1.96 mol/L) and high Lp(a) levels (median 0.27 g/L, range 0.07 to 2.57 g/L) were detected at flare-up. These changes were associated with an increased Lp(a) binding ratio onto fibrin (3.13Ϯ0.48) and cells (1.53Ϯ0.24) compared with binding ratios of control children (1.31Ϯ0.19 and 1.05Ϯ0.07, respectively) with normal plasminogen and low Lp(a) (median 0.071 g/L). After 6 weeks and 6 months of remission, the values for net decrease in Lp(a) binding to fibrin were 1.7Ϯ0.22 (after 6 weeks) and 1.88Ϯ0.38 (after 6 months) and were correlated with low Lp(a) concentrations (median 0.2 g/L, range 0.07 to 0.8 g/L; and median 0.12 g/L, range 0.07 to 1.34 g/L) and inversely associated with increased plasminogen levels (median 1.82 mol/L, range 1.4 to 2.1 mol/L; and median 1.58 mol/L, range 1.1 to 2.1 mol/L). These studies provide the first quantitative evidence that binding of Lp(a) to lysine residues of fibrin and cell surfaces is directly related to circulating levels of both plasminogen and Lp(a) and that these glycoproteins may interact as competitive ligands for these biological surfaces in vivo. This mechanism may be of relevance to the atherothrombotic role of Lp(a), particularly in nephrotic patients.

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Thromboembolic Complications in Children with Nephrotic Syndrome

Cited by 143 publications

References 18 publications

Platelet Functions and Coagulation Changes in Indian Children with Nephrotic Syndrome

Platelet Functions and Coagulation Changes in Indian Children with Nephrotic Syndrome

Steroid Sensitive Nephrotic Syndrome in Children

Effect of Individual Plasma Lipoprotein(a) Variations In Vivo on Its Competition With Plasminogen for Fibrin and Cell Binding

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