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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, cataracts, osteoporosis and fractures, obesity, impaired growth, and infertility. Long-term complications of SSNS are likely to be under-recognized. There remain many gaps in our knowledge of long-term outcomes of childhood NS, and further study is indicated. PMID:27252935

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

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    N. Teeninga (Nynke)

    2013-01-01

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

  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. 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.

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

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

    2016-09-01

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

  6. Congenital nephrotic syndrome

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

  7. Complications of nephrotic syndrome

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    Se Jin Park; Jae Il Shin

    2011-01-01

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

  8. 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.

  9. 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.

  10. Complications of nephrotic syndrome.

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    Park, Se Jin; Shin, Jae Il

    2011-08-01

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

  11. 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.

  12. Radiation nephritis causing nephrotic syndrome

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

    1983-12-01

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

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

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    Hirayama, Tomoya; Takahashi, Fumihiko; Kikuchi, Kenjiro

    2004-10-01

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

  14. Long-term 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

  15. 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.

  16. [Nephrotic syndrome associated with ampulloma].

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

    1989-06-01

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

  17. The Genetics of Nephrotic Syndrome.

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    Rheault, Michelle N; Gbadegesin, Rasheed A

    2016-03-01

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

  18. Extrarenal complications of the nephrotic syndrome.

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    Harris, R C; Ismail, N

    1994-04-01

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

  19. Childhood Nephrotic Syndrome

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

  20. Childhood Nephrotic Syndrome

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

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

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    Al-Eisa AA

    2016-08-01

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

  2. 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.

  3. 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.

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

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

    2016-01-01

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

  5. 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.

  6. 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.

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

    Science.gov (United States)

    Al-Eisa, Amal A; Haider, Mohammad Z

    2016-01-01

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

  8. Rheumatoid disease presenting as a nephrotic syndrome.

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

    1988-01-01

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

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

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

    1998-01-01

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

  10. Renal artery embolization in severe nephrotic syndrome.

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

    2016-07-01

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

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

    Science.gov (United States)

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

    2009-01-01

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

  12. 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).

  13. Treatment of anemia of nephrotic syndrome with recombinant erythropoietin

    NARCIS (Netherlands)

    Gansevoort, RT; Vaziri, ND; deJong, PE

    1996-01-01

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

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

    Science.gov (United States)

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

    2016-01-01

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

  15. Serum lipid profile abnormalities among patients with nephrotic syndrome

    Directory of Open Access Journals (Sweden)

    Adu E.M

    2013-01-01

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

  16. Restless Legs Syndrome in Pediatric Patients With Nephrotic Syndrome

    Directory of Open Access Journals (Sweden)

    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.

  17. [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.

  18. 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.

  19. 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.

  20. 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

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

    Science.gov (United States)

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

    2014-11-01

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

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

    OpenAIRE

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

    2016-01-01

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

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

    OpenAIRE

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

    2014-01-01

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

  4. Histopathological types in adult nephrotic syndrome

    Directory of Open Access Journals (Sweden)

    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.

  5. [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.

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

    Science.gov (United States)

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

    2016-07-01

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

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

    OpenAIRE

    Putta; Yamini Devi

    2015-01-01

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

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

    OpenAIRE

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

    2012-01-01

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

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

    OpenAIRE

    Schnaper, H W; Aune, T M

    1987-01-01

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

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

    Science.gov (United States)

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

    2008-02-01

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

  11. Molecular and Genetic Basis of Inherited Nephrotic Syndrome

    Directory of Open Access Journals (Sweden)

    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.

  12. Nephrotic Syndrome in Children: From Bench to Treatment

    Directory of Open Access Journals (Sweden)

    J.-C. Davin

    2011-01-01

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

  13. [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.

  14. [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.

  15. Defining nephrotic syndrome from an integrative genomics perspective

    OpenAIRE

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

    2014-01-01

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

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

    OpenAIRE

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

    2015-01-01

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

  17. 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

  18. 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.

  19. 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.

  20. 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.

  1. 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.

  2. 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.

  3. A PROSPECTIVE STUDY OF NEPHROTIC SYNDROME IN CHILDREN

    Directory of Open Access Journals (Sweden)

    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.

  4. Chronic graft versus host disease and nephrotic syndrome

    Directory of Open Access Journals (Sweden)

    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.

  5. Chronic graft versus host disease and nephrotic syndrome.

    Science.gov (United States)

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

    2014-09-01

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

  6. History of Nephrotic Syndrome and Evolution of its Treatment

    Science.gov (United States)

    Pal, Abhijeet; Kaskel, Frederick

    2016-01-01

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

  7. 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.

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

    Science.gov (United States)

    Pal, Abhijeet; Kaskel, Frederick

    2016-01-01

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

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

    Science.gov (United States)

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

    1987-01-10

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

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

    Science.gov (United States)

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

    2015-12-01

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

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

    Science.gov (United States)

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

    2016-08-01

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

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

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    Putta

    2015-03-01

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

  13. 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.

  14. 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.

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

    OpenAIRE

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

    2000-01-01

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

  16. Treatment of steroid resistant nephrotic syndrome in children

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    Kari Jameela

    2010-01-01

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

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

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    Kurnia Dwi Astuti

    2016-07-01

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

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

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    Kurnia Dwi Astuti, Mohammad Heru Muryawan, Omega Mellyana

    2015-01-01

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

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

    OpenAIRE

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

    2009-01-01

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

  20. 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 .

  1. 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.

  2. 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.

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

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    Rajendra

    2014-09-01

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

  4. 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.

  5. 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

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

    Science.gov (United States)

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

    2014-10-22

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

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

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    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.

  8. 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.

  9. 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

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

    Science.gov (United States)

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

    2016-01-01

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

  11. Vincristine treatment in steroid-dependent nephrotic syndrome.

    Science.gov (United States)

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

    2005-10-01

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

  12. Nephroprotective effect of heparanase in experimental nephrotic syndrome.

    Directory of Open Access Journals (Sweden)

    Suheir Assady

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

  13. 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.

  14. 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

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

    Institute of Scientific and Technical Information of China (English)

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

    2015-01-01

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

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

    Science.gov (United States)

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

    1989-01-01

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

  17. 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 .

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

    DEFF Research Database (Denmark)

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

    2015-01-01

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

  19. 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...

  20. 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.

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

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    Semerdjian Ronald J

    2003-08-01

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

  3. 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.

  4. 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

  5. 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

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

    Science.gov (United States)

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

    2013-01-01

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

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

    OpenAIRE

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

    2013-01-01

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

  8. 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.

  9. 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.

  10. 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.

  11. 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.

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

    OpenAIRE

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

    2013-01-01

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

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

    OpenAIRE

    Jyoti Dwivedi; Purnima Dey Sarkar

    2009-01-01

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

  14. 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.

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

    Science.gov (United States)

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

    1990-11-01

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

  16. 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.

  17. 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.

  18. 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.

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

    Science.gov (United States)

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

    2015-01-01

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

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

    Science.gov (United States)

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

    2016-01-01

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

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

    Science.gov (United States)

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

    2016-01-01

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

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

    OpenAIRE

    Putaala, Heli

    2001-01-01

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

  3. 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.

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

    OpenAIRE

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

    2012-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Saime Paydas

    2015-01-01

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

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

    International Nuclear Information System (INIS)

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

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

    Science.gov (United States)

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

    2015-06-01

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

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

    Science.gov (United States)

    Klosterman, Emily S; Pressler, Barrak M

    2011-08-01

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

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

    Science.gov (United States)

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

    2010-10-01

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

  11. 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.

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

    Directory of Open Access Journals (Sweden)

    Zeinab A. El-Sayed

    2014-01-01

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

  13. 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...

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

    Directory of Open Access Journals (Sweden)

    Beata Bieniaś

    2012-04-01

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

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

    Directory of Open Access Journals (Sweden)

    Parsa Yousefichaijan

    2015-01-01

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

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

    DEFF Research Database (Denmark)

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

    2015-01-01

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

  18. 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

  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. 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.

  1. 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.

  2. 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

  3. 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

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

    Science.gov (United States)

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

    2016-09-01

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

  5. 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.

  6. 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.

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

    Science.gov (United States)

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

    1981-02-01

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

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

    Directory of Open Access Journals (Sweden)

    Margaret Duffy

    2015-10-01

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

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

    Directory of Open Access Journals (Sweden)

    Pio Liberatore

    2012-06-01

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

  10. 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

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

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    Mahesh Kumar Kempanahalli Basappa

    2015-04-01

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

  12. 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.

  13. Nephrotic syndrome

    Science.gov (United States)

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

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

    Directory of Open Access Journals (Sweden)

    Jyoti Dwivedi*

    2009-09-01

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

  15. [Frey syndrome in childhood].

    Science.gov (United States)

    Clarós, P; González-Enseñat, M A; Arimany, J; Vincente, M A; Clarós, A

    1993-01-01

    Frey's syndrome is distinguished by the appearing of erythema, sensation of hotness, sometimes pain, and transpiration discharge in the preauricular and temporal area when ingestion stars. We present an eleven month old child with this pathology and we review the etiology and clinic manifestations of this syndrome. PMID:8129975

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

    OpenAIRE

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

    2013-01-01

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

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

    Institute of Scientific and Technical Information of China (English)

    张丽丽

    2015-01-01

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

  18. Sweet Syndrome in childhood.

    Science.gov (United States)

    Santos, Talita Batalha Pires dos; Sales, Barbara Cristina Gouveia; Sigres, Marianne; Rosman, Fernando; Cerqueira, Ana Maria Mosca de

    2015-01-01

    Sweet syndrome or acute febrile neutrophilic dermatosis is a recurrent and rare skin disease caused by the release of cytokines, with diverse possible etiologic causes. It presents clinically with polymorphic skin lesions, fever, arthralgia, and peripheral leukocytosis. In general, it is associated with infections, malignancy and drugs. It usually regresses spontaneously and treatment is primarily to control the basic disease. The authors report the case of a child of 1 year and 11 months who developed Sweet syndrome. PMID:26375229

  19. Chronic Fatigue Syndrome (CFS): Childhood Adversity

    Science.gov (United States)

    ... childhood maltreatment. In particular, for women, emotional and sexual abuse during childhood was associated with a greater risk ... risk for chronic fatigue syndrome: results from a population-based study. Arch Gen Psychiatry. 2006 Nov;63( ...

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

    Directory of Open Access Journals (Sweden)

    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.

  1. Sweet Syndrome in childhood*

    OpenAIRE

    dos Santos, Talita Batalha Pires; Sales, Barbara Cristina Gouveia; Sigres, Marianne; Rosman, Fernando; de Cerqueira, Ana Maria Mosca

    2015-01-01

    Sweet syndrome or acute febrile neutrophilic dermatosis is a recurrent and rare skin disease caused by the release of cytokines, with diverse possible etiologic causes. It presents clinically with polymorphic skin lesions, fever, arthralgia, and peripheral leukocytosis. In general, it is associated with infections, malignancy and drugs. It usually regresses spontaneously and treatment is primarily to control the basic disease. The authors report the case of a child of 1 year and 11 months who...

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

    Science.gov (United States)

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

    1977-01-01

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

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

    Directory of Open Access Journals (Sweden)

    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

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

    Science.gov (United States)

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

    2000-10-01

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

  5. 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

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

    Science.gov (United States)

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

    2006-05-01

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

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

    Directory of Open Access Journals (Sweden)

    Ayi Dilla Septarini

    2012-01-01

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

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

    Science.gov (United States)

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

    2016-07-01

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

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

    NARCIS (Netherlands)

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

    2008-01-01

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

  10. [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.

  11. 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

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

    Institute of Scientific and Technical Information of China (English)

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

    2011-01-01

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

  13. 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.

  14. 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.

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

    International Nuclear Information System (INIS)

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

  16. 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

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

    OpenAIRE

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

    2011-01-01

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

  18. 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.

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

    OpenAIRE

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

    2014-01-01

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

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

    Science.gov (United States)

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

    2016-01-01

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

  1. 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

  2. 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

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

    Science.gov (United States)

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

    2016-05-20

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

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

    International Nuclear Information System (INIS)

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

  5. Turner syndrome in childhood and adolescence

    OpenAIRE

    McCarthy, Kateri; Bondy, Carolyn A

    2008-01-01

    This article reviews current patterns of ascertainment, clinical characteristics and quality of care for girls with Turner syndrome, based on a cohort of 100 girls (aged 7–17 years) prospectively evaluated at the National Institute of Child Health since 2001. Approximately 25% were diagnosed prenatally or at birth owing to webbed neck and other features typical of fetal lymphedema, few were diagnosed during early childhood, with the majority undiagnosed until age 9 years or older. Major clini...

  6. 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...... aberrations and that low IER3 expression was associated with a worse outcome. Therefore, we investigated the frequency and prognostic impact of IER3 expression in childhood MDS. Methods: IER3 mRNA expression was determined by quantitative real-time PCR in 58 childhood MDS patients of which 17 carried a.......01–73.3% relative to GAPDH expression) and 3.3% in normal bone marrow (range 0.81–85.5% relative to GAPDH expression) (p=0.05). A more than 4-fold decrease in IER3 expression below the mean of healthy controls was found in 74% (43/58) of the childhood MDS patients. There was no difference in IER3 mRNA expression...

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

    Science.gov (United States)

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

    2016-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Arnold H Zea

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

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

    Directory of Open Access Journals (Sweden)

    Vaya Dasitania

    2014-05-01

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

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

    Science.gov (United States)

    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.

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

    Science.gov (United States)

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

    1997-01-01

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

  12. The metabolic syndrome and body composition in childhood cancer survivors

    OpenAIRE

    Jae Hoon Chung; Ki Woong Sung; Keon hee Yoo; Soo Hyun Lee; Sung-Yoon Cho; Se-Hwa Kim; Sung Won Park; Su Jin Kim; Young Bae Sohn; Hong Hoe Koo; Dong-Kyu Jin

    2011-01-01

    Purpose : Long-term survivors of childhood cancer appear to have an increased risk for the metabolic syndrome, subsequent type 2 diabetes and cardiovascular disease in adulthood compared to healthy children. The purpose of this study was to investigate the frequency of the metabolic syndrome and associated factors in childhood cancer survivors at a single center in Korea. Methods : We performed a retrospective review of medical records of 98 childhood cancer survivors who were diagnosed and c...

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

    Science.gov (United States)

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

    2016-07-01

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

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

    Institute of Scientific and Technical Information of China (English)

    杨红霞; 刘化侠; 李蕾; 万学英; 崔文香

    2012-01-01

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

  15. IRRITABLE BOWEL SYNDROME: Relationships with Abuse in Childhood

    OpenAIRE

    Sansone, Randy A.; Sansone, Lori A.

    2015-01-01

    Irritable bowel syndrome is allegedly the most common gastrointestinal diagnosis in the United States. The etiology of this syndrome appears to entail the interaction of both genes and the environment. One potential environmental contributory factor to irritable bowel syndrome is abuse in childhood. Of the various forms of abuses previously examined, sexual abuse in childhood appears to be the most patent contributor. However, both emotional and physical abuses may also contribute to irritabl...

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

    International Nuclear Information System (INIS)

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

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

    Directory of Open Access Journals (Sweden)

    Carsten Sauer Mikkelsen

    2010-01-01

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

  18. Childhood Sexual Abuse and Psychosomatic Symptoms in Irritable Bowel Syndrome

    Science.gov (United States)

    Ross, Colin A.

    2005-01-01

    Irritable bowel syndrome is characterized by chronic gastrointestinal symptoms without a demonstrable physical cause. In a subgroup of patients, irritable bowel syndrome may be part of a cluster of psychosomatic symptoms related to childhood sexual abuse. To investigate this possibility, the Dissociative Disorders Interview Schedule (DDIS), the…

  19. IRRITABLE BOWEL SYNDROME: Relationships with Abuse in Childhood.

    Science.gov (United States)

    Sansone, Randy A; Sansone, Lori A

    2015-01-01

    Irritable bowel syndrome is allegedly the most common gastrointestinal diagnosis in the United States. The etiology of this syndrome appears to entail the interaction of both genes and the environment. One potential environmental contributory factor to irritable bowel syndrome is abuse in childhood. Of the various forms of abuses previously examined, sexual abuse in childhood appears to be the most patent contributor. However, both emotional and physical abuses may also contribute to irritable bowel syndrome, although less distinctly. Studies examining a combined childhood-abuse variable (i.e., sexual, emotional, and/or physical abuses) in relationship to irritable bowel syndrome also indicate inconsistent results. Given the presence of childhood abuse as a potential factor in the development of irritable bowel syndrome, a number of pathophysiological events are postulated to explain this relationship, including alterations in norepinephrine and serotonin levels as well as dysregulation of the hypothalamic-pituitary-adrenal axis. Only future research will clarify the specific abuse elements (i.e., further clarification of the individual types of abuse, duration of abuse, roles of the perpetrator/victim) and the pathophysiological changes that culminate in irritable bowel syndrome. PMID:26155376

  20. 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...

  1. 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

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

    Institute of Scientific and Technical Information of China (English)

    田鲁; 胡亚琳; 操轩

    2015-01-01

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

  3. 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

  4. 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.

  5. Severe childhood malaria syndromes defined by plasma proteome profiles.

    Directory of Open Access Journals (Sweden)

    Florence Burté

    Full Text Available BACKGROUND: Cerebral malaria (CM and severe malarial anemia (SMA are the most serious life-threatening clinical syndromes of Plasmodium falciparum infection in childhood. Therefore it is important to understand the pathology underlying the development of CM and SMA, as opposed to uncomplicated malaria (UM. Different host responses to infection are likely to be reflected in plasma proteome-patterns that associate with clinical status and therefore provide indicators of the pathogenesis of these syndromes. METHODS AND FINDINGS: Plasma and comprehensive clinical data for discovery and validation cohorts were obtained as part of a prospective case-control study of severe childhood malaria at the main tertiary hospital of the city of Ibadan, an urban and densely populated holoendemic malaria area in Nigeria. A total of 946 children participated in this study. Plasma was subjected to high-throughput proteomic profiling. Statistical pattern-recognition methods were used to find proteome-patterns that defined disease groups. Plasma proteome-patterns accurately distinguished children with CM and with SMA from those with UM, and from healthy or severely ill malaria-negative children. CONCLUSIONS: We report that an accurate definition of the major childhood malaria syndromes can be achieved using plasma proteome-patterns. Our proteomic data can be exploited to understand the pathogenesis of the different childhood severe malaria syndromes.

  6. 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.

  7. Underlying molecular and cellular mechanisms in childhood irritable bowel syndrome.

    Science.gov (United States)

    Chumpitazi, Bruno P; Shulman, Robert J

    2016-12-01

    Irritable bowel syndrome (IBS) affects a large number of children throughout the world. The symptom expression of IBS is heterogeneous, and several factors which may be interrelated within the IBS biopsychosocial model play a role. These factors include visceral hyperalgesia, intestinal permeability, gut microbiota, psychosocial distress, gut inflammation, bile acids, food intolerance, colonic bacterial fermentation, and genetics. The molecular and cellular mechanisms of these factors are being actively investigated. In this mini-review, we present updates of these mechanisms and, where possible, relate the findings to childhood IBS. Mechanistic elucidation may lead to the identification of biomarkers as well as personalized childhood IBS therapies. PMID:26883355

  8. The metabolic syndrome and body composition in childhood cancer survivors

    Directory of Open Access Journals (Sweden)

    Jae Hoon Chung

    2011-06-01

    Full Text Available Purpose : Long-term survivors of childhood cancer appear to have an increased risk for the metabolic syndrome, subsequent type 2 diabetes and cardiovascular disease in adulthood compared to healthy children. The purpose of this study was to investigate the frequency of the metabolic syndrome and associated factors in childhood cancer survivors at a single center in Korea. Methods : We performed a retrospective review of medical records of 98 childhood cancer survivors who were diagnosed and completed anticancer treatment at Samsung Medical Center, Seoul, Korea between Jan. 1996 and Dec. 2007. Parameters of metabolic syndrome were evaluated between Jan. 2008 and Dec. 2009. Clinical and biochemical findings including body fat percentage were analyzed. Results : A total of 19 (19.4% patients had the metabolic syndrome. The median body fat percentage was 31.5%. The body mass index and waist circumference were positively correlated with the cranial irradiation dose (r=0.38, P&lt;0.001 and r=0.44, P&lt;0.00, respectively. Sixty-one (62.2% patients had at least one abnormal lipid value. The triglyceride showed significant positive correlation with the body fat percentage (r=0.26, P=0.03. The high density lipoprotein cholesterol showed significant negative correlation with the percent body fat (r=- 0.26, P=0.03. Conclusion : Childhood cancer survivors should have thorough metabolic evaluation including measurement of body fat percentage even if they are not obese. A better understanding of the determinants of the metabolic syndrome during adolescence might provide preventive interventions for improving health outcomes in adulthood.

  9. 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.

  10. 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.

  11. 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.

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

    Institute of Scientific and Technical Information of China (English)

    龙京花

    2015-01-01

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

  13. 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。

  14. 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.

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

    Directory of Open Access Journals (Sweden)

    Claudio Bazzi

    2013-01-01

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

  16. 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.

  17. Childhood obesity and the metabolic syndrome in developing countries.

    Science.gov (United States)

    Gupta, Nidhi; Shah, Priyali; Nayyar, Sugandha; Misra, Anoop

    2013-03-01

    Rapidly changing dietary practices accompanied by an increasingly sedentary lifestyle predispose to nutrition-related non-communicable diseases, including childhood obesity. Over the last 5 y, reports from several developing countries indicate prevalence rates of obesity (inclusive of overweight) >15 % in children and adolescents aged 5-19 y; Mexico 41.8 %, Brazil 22.1 %, India 22.0 % and Argentina 19.3 %. Moreover, secular trends also indicate an alarming increase in obesity in developing countries; in Brazil from 4.1 % to 13.9 % between 1974 and 1997; in China from 6.4 % to 7.7 % between 1991 and 1997; and in India from 4.9 % to 6.6 % between 2003-04 to 2005-06. Other contributory factors to childhood obesity include: high socio-economic status, residence in metropolitan cities and female gender. Childhood obesity tracks into adulthood, thus increasing the risk for conditions like the metabolic syndrome, type 2 diabetes mellitus (T2DM), polycystic ovarian syndrome, hypertension, dyslipidemia and coronary artery disease later in life. Interestingly, prevalence of the metabolic syndrome was 35.2 % among overweight Chinese adolescents. Presence of central obesity (high waist-to-hip circumference ratio) along with hypertriglyceridemia and family history of T2DM increase the odds of T2DM by 112.1 in young Asian Indians (educational programs for children should be immediately initiated in developing countries, following the successful model program in India (project 'MARG'). PMID:23334584

  18. Practitioner Review: Chronic Fatigue Syndrome in Childhood

    Science.gov (United States)

    Garralda, M. Elena; Chalder, Trudie

    2005-01-01

    RBackground: Chronic fatigue syndrome (CFS) is being increasingly recognized in children and adolescents. Yet comparatively little attention has been given in the literature to management. Methods: Description of the main features of the disorder, precipitating and maintaining factors and diagnostic assessment. Outline of different views on the…

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

    NARCIS (Netherlands)

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

    1998-01-01

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

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

    Institute of Scientific and Technical Information of China (English)

    马文明

    2015-01-01

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

  1. 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.

  2. 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

  3. 178例儿童肾病综合征免疫抑制剂用药分析%Analysis of Immunosuppressive Agents Application in 178 Cases with Pediatric Nephrotic Syndrome

    Institute of Scientific and Technical Information of China (English)

    高茗; 张征; 何旭

    2012-01-01

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

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

    Directory of Open Access Journals (Sweden)

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

    2003-10-01

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

  5. 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.

  6. Childhood opsoclonus-myoclonus syndrome: diagnosis and treatment.

    Science.gov (United States)

    Blaes, Franz; Dharmalingam, Backialakshmi

    2016-06-01

    Opsoclonus-myoclonus syndrome (OMS) is a rare and primarily immune-mediated disease in children and adults. The main symptoms include opsoclonus, myoclonus and ataxia. In children, the symptoms also include irritability, and, over a long-term course, learning and behavioural disturbances. OMS can be idiopathic, parainfectious or occur as a paraneoplastic (tumour-associated) syndrome. Paraneoplastic OMS in children is almost exclusively associated with neuroblastoma, whereas in adults, small cell lung cancer and breast cancer are the main underlying tumours. An autoimmune pathophysiology is suspected because childhood OMS patients have functionally active autoantibodies, proinflammatory changes in the cytokine network and immunotherapy responses. Children appear to respond regularly to immunosuppressive treatment. However, although the neurological symptoms show a good response, most children continue to show neuropsychological disturbances. PMID:27095464

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

    Directory of Open Access Journals (Sweden)

    Giovanni Maria Poggi

    2011-08-01

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

  8. 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.

  9. 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

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

    Institute of Scientific and Technical Information of China (English)

    骆成珠; 银青梅; 周月琼; 周琼花

    2014-01-01

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

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

    Institute of Scientific and Technical Information of China (English)

    刘红平; 雷方; 姜苗苗

    2015-01-01

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

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

    Institute of Scientific and Technical Information of China (English)

    刘春妍

    2011-01-01

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

  13. 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.

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

    Institute of Scientific and Technical Information of China (English)

    王亚芬; 孙东; 柳彩侠; 冯锦红; 崔爱东

    2011-01-01

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

  15. Genetic Aspects of Nephrotic Syndrome

    DEFF Research Database (Denmark)

    Joshi, Shivani

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

  16. 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

  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. Hyperinsulinemia and waist circumference in childhood metabolic syndrome

    International Nuclear Information System (INIS)

    To determine the characteristics of obese children presenting at a tertiary care hospital and the frequency of metabolic syndrome (MS) in them using two paediatric definitions. A total of 262 obese children aged 4-16 years, with BMI greater than 95 percentile were included. Children having obesity due to syndromes, medications causing weight gain, chronic illness and developmental disability were excluded. Blood pressure, waist circumference, fasting triglycerides, HDL, insulin and glucose levels were obtained. Obesity was defined as BMI > 95 percentile for age and gender according to the UK growth reference charts. The prevalence of metabolic syndrome was estimated using to the De Ferrantis and Lambert definitions. The frequency of MS varied between 16% and 52% depending on whether insulin levels were included in the definition. There was a significant positive correlation(r) when the metabolic parameters were correlated with waist circumference and insulin levels, except HDL which was negatively correlated. All the metabolic parameters like waist circumference, triglycerides, high density lipoprotein cholesterol and systolic blood pressure increased considerably across the insulin quartile (p < 0.05). The most noteworthy anthropometric and metabolic abnormality were the waist circumference (46.5%) and insulin levels (58%) respectively. There was a marked difference in the frequency of metabolic syndrome according to the definition used. The waist circumference and hyperinsulinemia are significant correlates of MS in obese children. There is a need for establishing normal insulin ranges according to age, gender and pubertal status. The clinical examination and investigations ought to include waist circumference and insulin levels together as a part of the definition of MS, for early detection and intervention of childhood obesity. (author)

  19. 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.

  20. 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.

  1. 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...

  2. 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.

  3. 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

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

    Institute of Scientific and Technical Information of China (English)

    周维燕; 潘斌斌; 杜新

    2012-01-01

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

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

    Institute of Scientific and Technical Information of China (English)

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

    2011-01-01

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

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

    Institute of Scientific and Technical Information of China (English)

    吕林华; 李智英; 刘晓红

    2016-01-01

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

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

    Institute of Scientific and Technical Information of China (English)

    周巧玲; 成小苗; 欧阳春; 解勤之; 陈立平; 胡杨清

    2002-01-01

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

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

    Institute of Scientific and Technical Information of China (English)

    元君辉; 王启林

    2014-01-01

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

  9. 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

  10. Childhood Physical Abuse Is Associated with Incident Metabolic Syndrome in Mid-Life Women

    Science.gov (United States)

    Midei, Aimee J.; Matthews, Karen A.; Chang, Yue-Fang; Bromberger, Joyce T.

    2013-01-01

    Objective Previous research has suggested that childhood emotional abuse, physical abuse, and sexual abuse are associated with an increased risk for ischemic heart disease. Our objective was to examine whether childhood abuse predicted incident metabolic syndrome, a precursor to heart disease, in mid-life women. Methods Participants were 342 (114 Black, 228 White) women from the Pittsburgh site of the Study of Women’s Health Across the Nation (SWAN). SWAN included a baseline assessment of premenopausal or early perimenopausal women in midlife (mean age = 45.7), and women were evaluated for presence of the metabolic syndrome over 7 annual follow-up visits. Women were classified as having metabolic syndrome if they met 3 of the following criteria: waist circumference > 88 cm, triglycerides ≥ 150 mg/dl, HDL < 50 mg/dl, SBP ≥ 130 or DBP ≥ 85 mmHg or on blood pressure medication, and fasting glucose ≥ 110 mg/dl or diabetic. The Childhood Trauma Questionnaire is a standardized measure that retrospectively assesses three domains of abuse in childhood and adolescence: emotional, physical, and sexual abuse. Results Approximately 34% of the participants reported a history of abuse. Cox model survival analysis showed that physical abuse was associated with incident metabolic syndrome over the course of seven years (HR = 2.12, p = .02), adjusted for ethnicity, age at baseline, and time-dependent menopausal status. Sexual abuse and emotional abuse were unrelated to the metabolic syndrome. Conclusion This is the first study to show that a history of childhood abuse, specifically physical abuse, is related to the development of metabolic syndrome in mid-life women. PMID:22775234

  11. 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

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

    Institute of Scientific and Technical Information of China (English)

    都娟; 黄建萍; 王硕; 赵晓艳; 肖丽丽

    2014-01-01

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

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

    Institute of Scientific and Technical Information of China (English)

    张汶娟; 刘素云; 任献青

    2013-01-01

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

  14. 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.

  15. 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

  16. 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

  17. 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…

  18. Molecular determinants of juvenile myelomonosytic leukemia and childhood myelodysplastic syndrome

    NARCIS (Netherlands)

    A.C.H. de Vries (Andrica)

    2012-01-01

    textabstractIn the general population the probability of developing cancer before the age of 18 years is around 1 in 400. In the Netherlands, approximately 600 new children each year are diagnosed with cancer (Figure 1). The most common types of childhood cancer are leukemias and the distribution of

  19. 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

  20. 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).

  1. 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

  2. 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.

  3. 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

  4. Childhood Sjögren syndrome presenting as acute brainstem encephalitis.

    Science.gov (United States)

    Matsui, Yoriko; Takenouchi, Toshiki; Narabayashi, Atsushi; Ohara, Kentaro; Nakahara, Tadaki; Takahashi, Takao

    2016-01-01

    Sjögren syndrome is an autoimmune disease characterized by dry mouth and eyes, known as sicca symptoms. The exact spectrum of neurological involvement, especially of the central nervous system, in childhood Sjögren syndrome has not been well defined. We report a girl who presented with acute febrile brainstem encephalitis. In retrospect, she had exhibited a preceding history of recurrent conjunctivitis and strong halitosis that could be considered as sicca symptoms. The histopathology results of a minor salivary biopsy, the presence of anti-SSA/Ro antibody, and keratoconjunctivitis confirmed the diagnosis of Sjögren syndrome. Commonly observed features in previously reported patients with childhood Sjögren syndrome and central nervous system complications have included fever at the time of neurologic presentation, cerebrospinal fluid pleocytosis, abnormal neuroimaging, and positivity for several specific antibodies. In children presenting with unknown acute febrile encephalopathy, Sjögren syndrome should be included in the differential diagnosis, especially when sicca symptoms are present. PMID:26006751

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

    OpenAIRE

    M. Barzegar; S. Davari Farid; Dastgiri, S.; Malekian, A; V. Toopchizadeh

    2008-01-01

     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, aged< 15 years, referred/admitted to Tabriz Children Hospital with GBS between January 2001 and December 2005 were investigated.ResultsOne hundred and twelve subjects were enrolled during this period. The average annual inciden...

  6. 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…

  7. 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.

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

    Institute of Scientific and Technical Information of China (English)

    贺建军

    2014-01-01

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

  9. 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。

  10. 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...

  11. 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

  12. 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.

  13. 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.

  14. 1例难治性肾病综合征患儿免疫抑制药物选择的药学实践%Pharmaceutical Practice in Selection of Immunosuppressive Drugs for One Case of Refractory Nephrotic Syndrome

    Institute of Scientific and Technical Information of China (English)

    孙会静; 陈文颖

    2015-01-01

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

  15. 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

  16. Congenital myasthenic syndromes in childhood: diagnostic and management challenges.

    Science.gov (United States)

    Kinali, M; Beeson, D; Pitt, M C; Jungbluth, H; Simonds, A K; Aloysius, A; Cockerill, H; Davis, T; Palace, J; Manzur, A Y; Jimenez-Mallebrera, C; Sewry, C; Muntoni, F; Robb, S A

    2008-09-15

    The Congenital Myasthenic Syndromes (CMS), a group of heterogeneous genetic disorders of neuromuscular transmission, are often misdiagnosed as congenital muscular dystrophy (CMD) or myopathies and present particular management problems. We present our experience of 46 children with CMS, referred to us between 1992-2007 with provisional diagnoses of congenital myopathy (22/46), CMS or limb-girdle myasthenia (9/46), central hypotonia or neurometabolic disease (5/46), myasthenia gravis (4/46), limb-girdle or congenital muscular dystrophy (4/46) and SMA (2/46). Diagnosis was often considerably delayed (up to 18y4 m), despite the early symptoms in most cases. Diagnostic clues in the neonates were feeding difficulties (29/46), hypotonia with or without limb weakness (21/46), ptosis (19/46), respiratory insufficiency (12/46), contractures (4/46) and stridor (6/46). Twenty-five children had delayed motor milestones. Fatigability developed in 43 and a variable degree of ptosis was eventually present in 40. Over the period of the study, the mainstay of EMG diagnosis evolved from repetitive nerve stimulation to stimulation single fibre EMG. The patients were studied by several different operators. 66 EMGs were performed in 40 children, 29 showed a neuromuscular junction abnormality, 7 were myopathic, 2 had possible neurogenic changes and 28 were normal or inconclusive. A repetitive CMAP was detected in only one of seven children with a COLQ mutation and neither of the two children with Slow Channel Syndrome mutations. Mutations have been identified so far in 32/46 children: 10 RAPSN, 7 COLQ, 6 CHRNE, 7 DOK7, 1 CHRNA1 and 1 CHAT. 24 of 25 muscle biopsies showed myopathic changes with fibre size variation; 14 had type-1 fibre predominance. Three cases showed small type-1 fibres resembling fibre type disproportion, and four showed core-like lesions. No specific myopathic features were associated with any of the genes. Twenty children responded to Pyridostigmine treatment alone

  17. 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.

  18. 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. PMID:26844222

  19. [Emotional and autonomous presentations of metabolic syndrome in childhood].

    Science.gov (United States)

    Naugol'nykh, Iu V; Sukhikh, E V; Mudrova, O A; Smirnova, E N

    2012-01-01

    We examined 46 children and adolescents, aged from 7 to 16 years, with metabolic syndrome (MS) and 20 healthy volunteers. Diagnosis was established by the presence of abdominal obesity. Total cholesterol, low density proteins, atherogenity index, blood insulin and glucose with the determination of insulin resistance index were measured. A special table suggested by A.M. Vein was used for assessment of autonomous tonus and reactivity, Kerdo index and Danini-Ashner reflex were calculated. Compensatory abilities in children with MS were determined by the results of variation cardiointervalography with the calculation of main indicators. The study of autonomous provision of activity was carried out using the experimental modeling of activity: mental, emotional. Emotions and personality were assessed by Shmishek-Leonhard questionnaire. The neurological examination did not reveal focal symptoms. The disintegration of the autonomous system activity manifested itself by the activation of ergotropic link accompanied by the changes in autonomous reactivity and formation of inadequate provision of activity. The results of psychological examination revealed the ecstatic type of accentuation that indicated high emotionality and psychological lability of subjects. PMID:22677659

  20. 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。

  1. 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.

  2. The Relationship between Metabolic Syndrome and Childhood Maternal Education Level, Job Status Findings from the Korean National Health and Nutrition Examination, 2007-2009

    OpenAIRE

    Choi, Bo-Yoon; Lee, Duk-Chul; Chun, Eun-Hye; Lee, Jee-Yon

    2014-01-01

    Background Childhood socioeconomic status (SES) is known to affect cardio-metabolic disease risk. However, the relationship between childhood SES and metabolic syndrome (MetS) remains uncertain. Therefore, we investigated the relationship between childhood SES, as measured by maternal education and occupational status and adult-onset MetS in the Korean population. Methods We examined the association between childhood SES, as measured by maternal education level and occupational status during ...

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

    Institute of Scientific and Technical Information of China (English)

    全军肾脏病研究所学术委员会; 许书添; 谢红浪; 刘志红

    2012-01-01

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

  4. 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

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

    Directory of Open Access Journals (Sweden)

    Atehortúa Baena, Paola Shyrley

    2013-04-01

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

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

    OpenAIRE

    Schnaper, H W; Aune, T M

    1985-01-01

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

  7. 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.

  8. 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.

  9. 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

  10. Metabolic Syndrome in Childhood: Rare Case of Alstrom Syndrome with Blindness.

    Science.gov (United States)

    Ahmad, Afzal; D'Souza, Benedicta; Yadav, Charu; Agarwal, Ashish; Kumar, Anand; Nandini, M; D'Souza, Vivian; Poornima, A M; Kamath, Nutan

    2016-10-01

    Alstrom's syndrome (AS) is a rare autosomal recessive ciliopathic condition affecting 1:10,00,000 children. It's a single gene disorder of ALMS1 on chromosome 2 with multisystem involvement with cone-rod retinal dystrophy causing juvenile blindness, obesity, insulin resistance, type 2 Diabetes mellitus, hypogonadism and sensorineural hearing loss. Till now only 800 patients with this disorder has been identified so far. In this report, we describe the case of a 9-year old male boy from south India. He had been initially referred for polyphagia, polyuria, polydipsia, generalized weakness from 1 weeks. On examination he was demonstrated features suggestive of AS, including blindness, obesity, type 2 diabetes, altered lipid profile, hypogonadism, acanthosis nigricans, seborrheic dermatitis, right ear discharge and episodes of respiratory tract infections. So, diagnosis of AS is critical as it can easily be overlooked because of the many features associated with metabolic syndrome starting at age 7, a relatively early age. PMID:27605748

  11. 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

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

    Institute of Scientific and Technical Information of China (English)

    樊剑锋; 刘小荣; 孟群; 张桂菊; 周楠; 陈植; 蒋也平

    2015-01-01

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

  13. Reversible posterior leukoencephalopathy syndrome in childhood: report of nine cases and review of the literature.

    Science.gov (United States)

    Gümüş, Hakan; Per, Hüseyin; Kumandaş, Sefer; Yikilmaz, Ali

    2010-04-01

    Reversible posterior leukoencephalopathy syndrome (RPLS) is recently described disorder with typical radiological findings in the posterior regions of the cerebral hemisphere and cerebellum. Its clinical symptoms include headache, decreased alertness, mental abnormalities, such as confusion, diminished spontaneity of speech, and changed behavior ranging from drowsiness to stupor, seizures, vomiting and abnormalities of visual perception like cortical blindness. RPLS is caused by various heterogeneous factors, the commonest being hypertension, followed by non-hypertensive causes such as eclampsia, renal diseases and immunosuppressive therapy. We presented nine patients with RPLS who had primary diagnoses such as acute post-streptococcal glomerulonephritis, idiopathic hypertension, the performing of intravenous immunoglobulin for infection with crescentic glomerulonephritis, erythrocyte transfusion for severe iron deficiency, L: -asparaginase treatment for acute lymphoblastic leukemia and performing of granulocyte-colony stimulating factor for ulcerative colitis due to neutropenia. Early recognition of RPLS as complication during different diseases and therapy in childhood may facilitate precise diagnosis and appropriate treatment. PMID:19809787

  14. [Markers of metabolic syndrome and peptides regulating metabolism in survivors of childhood acute lymphoblastic leukemia].

    Science.gov (United States)

    Skoczeń, Szymon; Tomasik, Przemysław; Balwierz, Walentyna; Surmiak, Marcin; Sztefko, Krystyna; Galicka-Latała, Danuta

    2011-01-01

    Along with the growing epidemic of overweight the risk of atherosclerosis, cardiovascular disease morbidity and mortality are increasing markedly. Metabolic syndrome (MS) is a condition clustering together several risk factors of those complications such as visceral obesity, glucose intolerance, arterial hypertension and dislipidemia. The risk of obesity in acute lymphoblastic leukemia (ALL) survivors is higher than in general population. We aimed to assess (1) the relationships between chosen adipokines and neuropeptides, chemotherapy, CRT, and body fatness and (2) evaluate adipokines and neuropeptides concentrations as a new markers of MS in children. We conducted cross-sectional evaluation of 82 ALL survivors (median age: 13.2 years; range: 4,8-26,2; median time from treatment: 3.2 years), including fasting laboratory testing: peptides (leptin, GLP-1, orexin, PYY, apelin), total cholesterol and its fractions, triglycerides; anthropometric measurements (weight, height), systolic and diastolic blood pressure. We estimated percentiles of body mass index and percentiles of blood pressure. Between 82 survivors overweight and diastolic hypertension was diagnosed in 31% of patients (35% in CRT group) and 15% respectively. At least one abnormality in lipids concentrations was found in 43%. Girls were more affected than boys. Statistically significant increased in leptin and apelin concentrations and decreased in soluble leptin receptor concentrations in the overweight group were observed compared to the non overweight subjects. Significant increase in orexin levels in females who had received CRT compared to those who had not received CRT was found. CRT is the main risk factor of elevated of body mass among survivors of childhood leukemia. Dyslipidemia and hypertension, along with increased adiposity indicate higher risk of MS development. Girls are more affected than boys. Leptin, orexin and apelin seem to be good markers of increased adiposity especially after CRT

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

    Directory of Open Access Journals (Sweden)

    Patrícia Zambi Meirelles

    2008-06-01

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

  16. 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.

  17. 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

  18. Long-term Impact of Childhood Adiposity on Adult Metabolic Syndrome Is Modified by Insulin Resistance: The Bogalusa Heart Study

    OpenAIRE

    Zhang, Huijie; Zhang,Tao; Li, Shengxu; LI, YING; Hussain, Azad; Fernandez, Camilo; Harville, Emily; Bazzano, Lydia A.; He, Jiang; Chen, Wei

    2015-01-01

    Childhood adiposity and insulin resistance are well-known risk factors for adult metabolic syndrome (MetS). This study aims to examine whether the association between childhood adiposity and adult MetS is modified by insulin resistance. The cohort consisted of 1,593 black and white subjects, aged 19–50 years at follow-up, who were examined 19 years apart on average as children and adults for MetS variables. The prevalence of adult MetS was compared between the insulin-sensitive obesity and in...

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

    Institute of Scientific and Technical Information of China (English)

    刘蕊

    2015-01-01

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

  20. 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

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

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    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.

  2. 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

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

    Institute of Scientific and Technical Information of China (English)

    梁慧开; 周建华; 仇丽茹; 程黎明; 鲍春; 李辉军

    2014-01-01

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

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Institute of Scientific and Technical Information of China (English)

    李彩霞

    2015-01-01

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

  6. 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

  7. 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。

  8. 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…

  9. 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

  10. 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.

  11. 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.

  12. 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. PMID:22510527

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

    OpenAIRE

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

    2012-01-01

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

  14. 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…

  15. The Clinical and Neurophysiological Features of Epileptic Syndromes Associated with Benign Focal Epileptiform Discharges of Childhood

    Directory of Open Access Journals (Sweden)

    N. A. Ermolaenko

    2014-01-01

    Full Text Available Benign focal epileptiform discharge of childhood (BFEDC is an age-dependent pattern determined in electroencephalograms (EEGs, which is associated with idiopathic benign focal epilepsy (BFE. Studies of BFE revealed symptomatic phenocopies in patients with cerebral struc- tural abnormalities in such conditions as infantile cerebral palsy and malformations. Some arguments against the «benign» nature of BFEDC are presented, since BFEDC may impair various cognitive functions and behavior (e.g., cause epileptic encephalophathies. Objective. To determine the clinical and neurophysiological features of epileptic syndromes associated with prolonged epileptiform activity during sleep in children and adolescents, as well as approaches to rational therapy. Patients and Methods. A total of 1862 children aged 2–18 admitted to the specialized Department of Psychoneurology of the Voronezh Regional Children Clinical Hospital No 1 in 2004–2007, who had epileptic seizures and non-epileptic neurological disorders, were exam- ined. The children underwent assessment of the neurological status, neuropsychological assessment, and video-EEG monitoring. The spike- wave index (SWI was calculated and magnetic resonance imaging (MRI of the brain was performed to register the epileptiform activity during sleep. Results and Discussion. It was demonstrated that when ISW of BFEDC patterns is >30%, evolution into epileptic encephalopathy was observed in 66% of patients (including epilepsy with electrical status epilepticus in sleep in 49% of patients and cognitive epileptiform disinte- gration in 17% of patients. The results prove the justification of prescribing antiepileptic drugs to patients with SWI ≥30% even if they have no epileptic seizures. Duo-therapy with valproate and ethosuximide or levetiracetam is most the effective therapy. Further prospective studies for children with BFEDC will give new insight into this area. 

  16. 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

  17. 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

  18. 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

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

    Institute of Scientific and Technical Information of China (English)

    黄静蓉

    2015-01-01

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

  20. 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

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

    Institute of Scientific and Technical Information of China (English)

    付平; 许国章; 黄颂敏; 余庭龙

    2001-01-01

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

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

    International Nuclear Information System (INIS)

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

  3. 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

  4. 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

  5. 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

  6. 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.

  7. 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.

  8. 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.

  9. Dysaesthesias and dysautonomia: a self-limited syndrome of painful dysaesthesias and autonomic dysfunction in childhood.

    OpenAIRE

    Nass, R.; Chutorian, A

    1982-01-01

    Three children with an acute self-limited syndrome characterised by painful dysaethesias, hypertension, and autonomic dysfunction, in the absence of motor and reflex abnormalities, are presented. They appear to have had a variant of acute polyneuritis involving sensory and autonomic systems. The pathophysiology of hypertension in the Guillain-Barré syndrome and of acute pandysautonomia is discussed. Excessive adrenergic function is considered as a cause of the pain component of the syndrome.

  10. 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。

  11. 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...

  12. 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…

  13. 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,

  14. 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

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

    Institute of Scientific and Technical Information of China (English)

    李玉柳; 王华

    2012-01-01

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

  16. 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

  17. 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.

  18. Northern epilepsy syndrome: an inherited childhood onset epilepsy with associated mental deterioration.

    OpenAIRE

    Hirvasniemi, A; Lang, H; Lehesjoki, A E; Leisti, J

    1994-01-01

    A new autosomal recessively inherited disease of the central nervous system involving childhood epilepsy and mental deterioration is described. Twenty three patients (11 males and 12 females) belonging to 11 families from northern Finland have been identified. A common ancestor has been found for nine families. The mean age of onset of epilepsy was 6.7 years (range 5-10 years) and the epilepsy was characterised by generalised tonic-clonic seizures increasing in frequency up to puberty. One th...

  19. The Clinical and Neurophysiological Features of Epileptic Syndromes Associated with Benign Focal Epileptiform Discharges of Childhood

    OpenAIRE

    N. A. Ermolaenko; I A Buchneva; Zakharova, E. I.

    2014-01-01

    Benign focal epileptiform discharge of childhood (BFEDC) is an age-dependent pattern determined in electroencephalograms (EEGs), which is associated with idiopathic benign focal epilepsy (BFE). Studies of BFE revealed symptomatic phenocopies in patients with cerebral struc- tural abnormalities in such conditions as infantile cerebral palsy and malformations. Some arguments against the «benign» nature of BFEDC are presented, since BFEDC may impair various cognitive functions and behavior (e.g....

  20. 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......, cyclophosphamide and melphalan (Bu/Cy/Mel) (23), an alternative busulfan based regimen (6), a radiation based regimen (5) or others (3). Results: After a median follow up of 4.1 (0.5 – 9.4) years, 14 patients are alive in first complete remission (CR). Seventeen patients developed relapse after a median time...

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

    Directory of Open Access Journals (Sweden)

    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.

  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...... centers or their blood supply. The presence of three common point mutations seen in families with thrombophilia (1691G-->A in the coagulation factor V gene, 677C-->T in the methylenetetrahydrofolate reductase gene, and the 20210G-->A mutation in the prothrombin gene) could increase the risk for thrombosis...... 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. 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.

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

    Directory of Open Access Journals (Sweden)

    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.

  5. Is there an increased risk of metabolic syndrome among childhood acute lymphoblastic leukemia survivors? A developing country experience.

    Science.gov (United States)

    Mohapatra, Sonali; Bansal, Deepak; Bhalla, A K; Verma Attri, Savita; Sachdeva, Naresh; Trehan, Amita; Marwaha, R K

    2016-03-01

    Data on metabolic syndrome (MS) in survivors of childhood acute lymphoblastic leukemia (ALL) from developing countries are lacking. The purpose of this single-center, uncontrolled, observational study was to assess the frequency of MS in our survivors. The survivors of ALL ≤15 years at diagnosis, who had completed therapy ≥2 years earlier, were enrolled. Anthropometric measurements (weight, height, waist circumference), biochemistry (glucose, insulin, triglycerides, high-density lipoprotein [HDL], thyroid function tests, C-reactive protein [CRP], magnesium), measurement of blood pressure, and Tanner staging were performed. MS was defined by International Diabetes Federation (IDF) and the National Cholesterol Education Program Third Adult Treatment Panel guidelines (NCEP ATP III) criteria, modified by Cook et al. (Arch Pediatr Adolesc Med. 2003;157:821-827) and Ford et al. (Diabetes Care. 2005;28:878-881). The median age of 76 survivors was 11.9 years (interquartile range [IQR]: 9.6-13.5). Twenty-four (32%) survivors were obese or overweight. The prevalence of insulin resistance (17%), hypertension (7%), hypertriglyceridemia (20%), and low HDL (37%) was comparable to the prevalence in children/adolescents in historical population-based studies from India. The prevalence of MS ranged from 1.3% to 5.2%, as per different defining criteria. Cranial radiotherapy, age at diagnosis, sex, or socioeconomic status were not risk factors for MS. The prevalence of MS in survivors of childhood ALL, at a median duration of 3 years from completion of chemotherapy, was comparable to the reference population. The prevalence of being obese or overweight was, however, greater than historical controls. PMID:26984439

  6. 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…

  7. 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.

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

    Directory of Open Access Journals (Sweden)

    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

  9. 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

  10. 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

  11. 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.

  12. Childhood Onset of Scheie Syndrome, the Attenuated Form of Mucopolysaccharidosis I

    OpenAIRE

    Thomas, Janet A.; Beck, Michael; Clarke, Joe T. R.; Cox, Gerald Frank

    2010-01-01

    Scheie syndrome is the most attenuated and rarest form of mucopolysaccharidosis type I (MPS I), an inherited lysosomal storage disorder. Only small patient series have previously been reported. Using natural history data from the uniquely large population of 78 Scheie patients enrolled in the MPS I Registry, we characterized the onset and prevalence of clinical manifestations and explored reasons for delayed diagnosis of the disease. Median patient age was 17.5 years; 46% of the patients were...

  13. [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.

  14. 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

  15. [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.

  16. 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.

  17. 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. PMID:26833960

  18. 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.

  19. 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

  20. 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

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

    Institute of Scientific and Technical Information of China (English)

    董峰; 周湘; 庞宁; 魏珉

    2002-01-01

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

  2. 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

  3. 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

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

    Institute of Scientific and Technical Information of China (English)

    康国贵; 周江瑾; 张琦; 陈惠琴; 康友群

    2011-01-01

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

  5. 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

  6. 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.

  7. Methylation of BDNF in women with bulimic eating syndromes: associations with childhood abuse and borderline personality disorder.

    Science.gov (United States)

    Thaler, Lea; Gauvin, Lise; Joober, Ridha; Groleau, Patricia; de Guzman, Rosherrie; Ambalavanan, Amirthagowri; Israel, Mimi; Wilson, Samantha; Steiger, Howard

    2014-10-01

    DNA methylation allows for the environmental regulation of gene expression and is believed to link environmental stressors to such mental-illness phenotypes as eating disorders. Numerous studies have shown an association between bulimia nervosa (BN) and variations in brain-derived neurotrophic factor (BDNF). BDNF has also been linked to borderline personality disorder (BPD) and to such traits as reward dependence. We examined the extent to which BDNF methylation corresponded to bulimic or normal-eater status, and also to the presence of comorbid borderline personality disorder (BPD) and childhood abuse. Our sample consisted of 64 women with BN and 32 normal-eater (NE) control women. Participants were assessed for eating-disorder symptoms, comorbid psychopathology, and childhood trauma, and then they were required to provide blood samples for methylation analyses. We observed a significant site×group (BN vs. NE) interaction indicating that women with BN showed increases in methylation at specific regions of the BDNF promoter. Furthermore, examining effects of childhood abuse and BPD, we observed significant site×group interactions such that groups composed of individuals with childhood abuse or BPD had particularly high levels of methylation at selected CpG sites. Our findings suggest that BN, especially when co-occurring with childhood abuse or BPD, is associated with a propensity towards elevated methylation at specific BDNF promoter region sites. These findings imply that hypermethylation of the BDNF gene may be related to eating disorder status, developmental stress exposure, and comorbid psychopathology. PMID:24801751

  8. What Are the Types and Phases of Rett Syndrome?

    Science.gov (United States)

    ... and Publications What are the types & phases of Rett syndrome? Skip sharing on social media links Share this: ... Rett Syndrome (Hanefeld Variant) 6 Late-Childhood Rett Syndrome Forme Fruste Rett Syndrome Preserved-Speech Variant of Rett Syndrome (Zappella ...

  9. 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

  10. A Case of Childhood Vitrectomy Performed for Dense Vitreous Hemorrhage Secondary to Leukemia Therapy and Tumor Lysis Syndrome

    OpenAIRE

    Kudo, Takashi; Suzuki, Yukihiko; Metoki, Tomomi; Nakazawa, Mitsuru

    2015-01-01

    Purpose To report a case of vitrectomy performed in a child with dense massive vitreous hemorrhage due to secondary acute myelogenous leukemia (AML) and tumor lysis syndrome. Case A 4-year-old boy with clear-cell renal cell carcinoma was successfully treated with chemotherapy in 2011. However, in May 2012, he developed secondary AML. Although he was treated with combined chemotherapy and radiation, tumor lysis syndrome occurred with renal and heart failure complications. After an ultrasound e...

  11. 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…

  12. 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. PMID:25562712

  13. 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.

  14. 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…

  15. 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。

  16. 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.

  17. 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)

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

    International Nuclear Information System (INIS)

    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)

  19. A Case of Childhood Vitrectomy Performed for Dense Vitreous Hemorrhage Secondary to Leukemia Therapy and Tumor Lysis Syndrome

    Directory of Open Access Journals (Sweden)

    Takashi Kudo

    2015-01-01

    Full Text Available Purpose: To report a case of vitrectomy performed in a child with dense massive vitreous hemorrhage due to secondary acute myelogenous leukemia (AML and tumor lysis syndrome. Case: A 4-year-old boy with clear-cell renal cell carcinoma was successfully treated with chemotherapy in 2011. However, in May 2012, he developed secondary AML. Although he was treated with combined chemotherapy and radiation, tumor lysis syndrome occurred with renal and heart failure complications. After an ultrasound examination by pediatricians found bilateral subretinal protrusions, he was referred to our clinic. Fundus examinations confirmed that the protrusions were bilateral subretinal or choroidal hemorrhages. A few weeks later, dense vitreous hemorrhages occurred bilaterally, and he completely lost vision in both eyes. Electroretinograms were extinguished in both eyes. After improvement of his general condition, we performed a 25-gauge vitrectomy combined with lens extraction in his left eye in December 2012. After removal of the vitreous hemorrhage, we found the subretinal hemorrhage had already been absorbed, leaving a mottled fundus color. However, the optic disc was not pale. Nine months after the surgery, his best-corrected visual acuity finally improved to 0.1. Conclusion: We successfully treated a case of severe vitreous hemorrhage secondary to leukemia therapy and tumor lysis syndrome using 25-gauge vitrectomy. This procedure may be safe and effective to perform, even in children with complications.

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

    OpenAIRE

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

    2006-01-01

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

  1. 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.

  2. 儿童布-力口综合征三例%Childhood Budd-Chiari syndrome in 3 cases

    Institute of Scientific and Technical Information of China (English)

    傅宏娜; 刘风林

    2008-01-01

    @@ 布-加综合征(Budd-Chiari syndrome,BCS)是一种可以危及生命,临床少见的疾病.在远东或亚洲地区(日本、中国和东南亚地区)发病率较高,发病年龄多集中在20~40岁[1].儿童BCS的报道在国内外少见,而2岁以下的BCS更是罕见.

  3. De novo WDR45 mutation in a patient showing clinically Rett syndrome with childhood iron deposition in brain.

    Science.gov (United States)

    Ohba, Chihiro; Nabatame, Shin; Iijima, Yoshitaka; Nishiyama, Kiyomi; Tsurusaki, Yoshinori; Nakashima, Mitsuko; Miyake, Noriko; Tanaka, Fumiaki; Ozono, Keiichi; Saitsu, Hirotomo; Matsumoto, Naomichi

    2014-05-01

    Rett syndrome (RTT) is a neurodevelopmental disorder mostly caused by MECP2 mutations. We identified a de novo WDR45 mutation, which caused a subtype of neurodegeneration with brain iron accumulation, in a patient showing clinically typical RTT. The mutation (c.830+1G>A) led to aberrant splicing in lymphoblastoid cells. Sequential brain magnetic resonance imaging demonstrated that iron deposition in the globus pallidus and the substantia nigra was observed as early as at 11 years of age. Because the patient showed four of the main RTT diagnostic criteria, WDR45 should be investigated in patients with RTT without MECP2 mutations. PMID:24621584

  4. Single dose rasburicase in the management of tumor lysis syndrome in childhood acute lymphoblastic leukemia: A case series.

    Science.gov (United States)

    Latha, S M; Krishnaprasadh, D; Murugapriya, P; Scott, J X

    2015-01-01

    Tumor lysis syndrome (TLS) occurs in malignancies with high proliferative potential and tumor burden, such as lymphomas and leukemias. TLS syndrome is an oncologic emergency, requiring prompt intervention. The metabolic derangements cause acute kidney failure and may lead to cardiac arrhythmias, seizures, and death. With the advent of rasburicase, a recombinant urate oxidase, there has been a decline in the TLS-mediated renal failure and the need for dialysis. The recommended regimen and doses pose a heavy financial burden for patients in developing countries like India. With data and studies proving a similar efficacy for the reduced dose and lesser number of rasburicase, we report here a case series of seven children with acute leukemias, whose TLS was managed by a single dose of rasburicase. A retrospective analysis of case records of seven children with acute lymphoblastic leukemia and TLS, admitted to our Pediatric Oncology Unit of our Hospital between the period 2011 and 2013, was done. All our patients responded to a single dose, indicating that in appropriately monitored patients, single dose followed by as-needed dosing can be cost-saving. PMID:25838646

  5. [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

  6. 儿童Jeavons综合征4例报告%Four cases report of Jeavons syndrome in childhood

    Institute of Scientific and Technical Information of China (English)

    徐哲峰; 刘占利; 黄先玫; 蒋春明; 陈丽琼

    2014-01-01

    目的:探讨儿童Jeavons综合征的临床、脑电图特征及药物治疗效果。方法回顾性分析4例儿童Jeavons综合征患儿的临床、脑电图特征及治疗效果等临床资料。结果4例患儿中女性3例、男性1例,起病年龄1~6岁。临床表现为反复特征性眼睑肌阵挛,伴或不伴失神发作。视频脑电图(VEEG)监测发作期脑电图,2例为全导对称同步3~6 Hz棘慢波或多棘慢波阵发,以闭眼诱发明显;2例为全导3.0~3.5 Hzδ节律发放。采用丙戊酸治疗2例、左乙拉西坦1例、丙戊酸联合左乙拉西坦1例。结果发作控制1例,发作减轻2例,发作仍频繁1例。结论 Jeavons综合征是一种特发性全面性癫综合征,发作主要为眼睑肌阵挛伴或不伴失神。VEEG对明确诊断有重要价值,丙戊酸钠或左乙拉西坦对控制发作有效。%Objective To explore the clinical manifestations, electroencephalographic characteristics and therapeutic effect of drugs in children with Jeavons syndrome. Methods The clinical and electroencephalographic characteristics and thera-peutic effect of drugs were analyzed in 4 children with Jeavons syndrome. Results Among the four children there were 3 female and 1 male. The age at the onset of the disease was from 1 to 6 years. The typical clinical manifestations of this disease were brief, fast and repeated eyelid myoclonia (EM) with or without absence seizure. The typical electroencephalography (EEG) in two patients showed 3-6 Hz generalized spike and waves and polyspikes burst, and the eye closure and intermittent photic stimu-lation helped to induce discharges and clinical events. The typictal EEG in the other two patients showed 3.0-3.5 Hz generalizedδslow wave rhythm burst. The drugs of choice for treatment was sodium valproate monotherapy in two cases, levetiracetam in one case, sodium valproate combined with levetiracetam in one case. During the follow-up, seizures were controlled in one case

  7. Cranial nerve palsies in childhood

    OpenAIRE

    Lyons, C J; Godoy, F; ALQahtani, E

    2015-01-01

    We review ocular motor cranial nerve palsies in childhood and highlight many of the features that differentiate these from their occurrence in adulthood. The clinical characteristics of cranial nerve palsies in childhood are affected by the child's impressive ability to repair and regenerate after injury. Thus, aberrant regeneration is very common after congenital III palsy; Duane syndrome, the result of early repair after congenital VI palsy, is invariably associated with retraction of the g...

  8. Childhood Stress

    Science.gov (United States)

    ... 5 Things to Know About Zika & Pregnancy Childhood Stress KidsHealth > For Parents > Childhood Stress Print A A ... and feel stress to some degree. Sources of Stress Stress is a function of the demands placed ...

  9. Childhood Obesity

    OpenAIRE

    Wilkinson, Justine; Howard, Simon

    2014-01-01

    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. 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。

  11. Childhood Cancer

    Science.gov (United States)

    ... Story" 5 Things to Know About Zika & Pregnancy Childhood Cancer KidsHealth > For Parents > Childhood Cancer Print A A A Text Size What's ... in children, but can happen. The most common childhood cancers are leukemia , lymphoma , and brain cancer . As ...

  12. Usher Syndrome

    Science.gov (United States)

    ... of their hearing within the first year of life. Progressive vision loss caused by retinitis pigmentosa becomes occurs in childhood. ... type III have progressive hearing loss and vision loss beginning in the first few decades of life. Unlike the other forms of Usher syndrome, infants ...

  13. 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.

  14. 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.

  15. 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); Childhood Acute Megakaryocytic Leukemia (M7); 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; Chronic Myelomonocytic Leukemia; de Novo Myelodysplastic Syndromes; Refractory Anemia; Refractory Anemia With Excess Blasts; Refractory Anemia With Excess Blasts in Transformation; Refractory Anemia With Ringed Sideroblasts; Secondary Myelodysplastic Syndromes; Untreated Childhood Acute Myeloid Leukemia and Other Myeloid Malignancies

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

    Institute of Scientific and Technical Information of China (English)

    DONG Fei-xia; HE Li-qun

    2009-01-01

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

  17. Childhood Obesity

    OpenAIRE

    Aydın, Ahmet; Koca, Fahrettin; Fıçıcıoğlu, Can; Çam, Halit; Mıkla, Şerare

    1995-01-01

    Management of childhood obesity and its early and late complications are among the most difficult problems confronted by pediatricians and practitioners The purpose of this review is to provide information for the evaluation and treatment of childhood obesity Key nbsp;words: nbsp;Child Obesity Etiology Management Complications

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

    OpenAIRE

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

    1989-01-01

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

  19. Hypertriglyceridemia Thalassemia Syndrome: A Report of 4 Cases

    OpenAIRE

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

    2014-01-01

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

  20. Adult onset Leigh syndrome

    Directory of Open Access Journals (Sweden)

    Pandit Lekha

    2007-01-01

    Full Text Available Leigh syndrome is a rare progressive mitochondrial disorder of oxidative metabolism. Though it has been reported in infancy and childhood, it is rarely described in adults. The authors describe a patient who had clinical and magnetic resonance imaging features diagnostic of Leigh syndrome, with supportive biochemical and muscle histochemistry evidence.

  1. Childhood Leukemia

    Science.gov (United States)

    Leukemia is cancer of the white blood cells. It is the most common type of childhood cancer. ... blood cells help your body fight infection. In leukemia, the bone marrow produces abnormal white blood cells. ...

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

    Science.gov (United States)

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

    2011-01-01

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

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

    OpenAIRE

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

    2015-01-01

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

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

    OpenAIRE

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

    2012-01-01

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

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

    DEFF Research Database (Denmark)

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

    2015-01-01

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

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

    NARCIS (Netherlands)

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

    2003-01-01

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

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

    Science.gov (United States)

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

    2016-01-01

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

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

    NARCIS (Netherlands)

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

    2010-01-01

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

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

    Science.gov (United States)

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

    2016-05-01

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

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

    DEFF Research Database (Denmark)

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

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

  11. Clinical Features of Turner Syndrome

    Science.gov (United States)

    ... growth is attenuated in utero, and statural growth lags during childhood and adolescence, resulting in adult heights ... easily treated with thyroid hormone supplements. Cognitive Function/Educational Issues In general, individuals with Turner syndrome have ...

  12. Genetics Home Reference: Menkes syndrome

    Science.gov (United States)

    ... In rare cases, symptoms begin later in childhood. Occipital horn syndrome (sometimes called X-linked cutis laxa ) ... bone at the base of the skull (the occipital bone), coarse hair, and loose skin and joints. ...

  13. Membranous nephropathy and lupus-like syndrome after hematopoietic cell transplantation: a case report

    Directory of Open Access Journals (Sweden)

    Stylianou Kostas

    2010-09-01

    Full Text Available Abstract Introduction The kidney is increasingly recognised as a target organ of chronic graft-versus-host disease after hematopoietic cell transplantation in the context of the development of the nephrotic syndrome. Chronic graft-versus-host disease is associated with autoimmune phenomena similar, but not identical, to those observed in various rheumatologic disorders, implicating autoimmunity as an important component of the disease. Case presentation We report the case of a 57-year-old Caucasian man who developed the nephrotic syndrome due to membranous nephropathy in association with recurrent chronic graft-versus-host disease, along with a lupus-like syndrome manifested with pancytopenia, hair loss, positive anti-DNA antibodies and sub-epithelial and mesangial immune deposits. To the best of our knowledge, this is the first case reported in the literature. The nephrotic syndrome subsided soon after he was treated with a short course of cyclosporin with steroids. Unfortunately he died seven months later due to a relapse of leukemia. Conclusions Our case report confirms the notion that chronic graft-versus-host disease is characterized by the appearance of autoimmune phenomena similar, but not identical, to those seen in autoimmune diseases. The decision for more immunosuppression has to be weighed against the need for preservation of the graft versus leukemia phenomenon.

  14. Childhood obesity

    DEFF Research Database (Denmark)

    Heitmann, Berit L; Koplan, Jeffrey; Lissner, Lauren

    2009-01-01

    Despite progress toward assuring the health of today's young population, the 21(st) century began with an epidemic of childhood obesity. There is general agreement that the situation must be addressed by means of primary prevention, but relatively little is known about how to intervene effectively....... The evidence behind the assumption that childhood obesity can be prevented was discussed critically in this roundtable symposium. Overall, there was general agreement that action is needed and that the worldwide epidemic itself is sufficient evidence for action. As the poet, writer, and scholar Wittner Bynner...

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

    OpenAIRE

    Pham, C; V. Utz; A. Marcotty; A. Zeft; P. Rychwalski

    2014-01-01

    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 diagnos...

  16. Childhood Obesity

    Science.gov (United States)

    Yuca, Sevil Ari, Ed.

    2012-01-01

    This book aims to provide readers with a general as well as an advanced overview of the key trends in childhood obesity. Obesity is an illness that occurs due to a combination of genetic, environmental, psychosocial, metabolic and hormonal factors. The prevalence of obesity has shown a great rise both in adults and children in the last 30 years.…

  17. Childhood Obesity

    Centers for Disease Control (CDC) Podcasts

    2013-08-06

    In this podcast, Dr. Tom Frieden, CDC Director, discusses the decrease in childhood obesity rates and what strategies have been proven to work to help our children grow up and thrive.  Created: 8/6/2013 by National Center for Injury Prevention and Control.   Date Released: 3/6/2014.

  18. Childhood obesity.

    Science.gov (United States)

    Strauss, R

    1999-01-01

    Approximately 10% of children are obese. Twin and adoption studies demonstrate a large genetic component to obesity, especially in adults. However, the increasing prevalence of obesity over the last 20 years can only be explained by environmental factors. In most obese individuals, no measurable differences in metabolism can be detected. Few children engage in regular physical activity. Obese children and adults uniformly underreport the amount of food they eat. Obesity is particularly related to increased consumption of high-fat foods. BMI is a quick and easy way to screen for childhood obesity. Treating childhood obesity relies on positive family support and lifestyle changes involving the whole family. Food preferences are influenced early by parental eating habits, and when developed in childhood, they tend to remain fairly constant into adulthood. Children learn to be active or inactive from their parents. In addition, physical activity (or more commonly, physical inactivity) habits that are established in childhood tend to persist into adulthood. Weight loss is usually followed by changes in appetite and metabolism, predisposing individuals to regain their weight. However, when the right family dynamics exist--a motivated child with supportive parents--long-term success is possible.

  19. The use of the Berlin definition for acute respiratory distress syndrome during infancy and early childhood : multicenter evaluation and expert consensus

    NARCIS (Netherlands)

    De Luca, Daniele; Piastra, Marco; Chidini, Giovanna; Tissieres, Pierre; Calderini, Edoardo; Essouri, Sandrine; Medina Villanueva, Alberto; Vivanco Allende, Ana; Pons-Odena, Marti; Perez-Baena, Luis; Hermon, Michael; Tridente, Ascanio; Conti, Giorgio; Antonelli, Massimo; Kneyber, Martin

    2013-01-01

    A new acute respiratory distress syndrome (ARDS) definition has been recently issued: the so-called Berlin definition (BD) has some characteristics that could make it suitable for pediatrics. The European Society for Pediatric Neonatal Intensive Care (ESPNIC) Respiratory Section started a project to

  20. Membranous nephropathy and lupus-like syndrome after hematopoietic cell transplantation: a case report

    OpenAIRE

    Stylianou Kostas; Stratakis Stavros; Mavroeidi Vasiliki; Petrakis Ioannis; Xydakis Dimitris; Vardaki Eleftheria; Stratigis Spyros; Perakis Kostas; Katsarou Theodora; Kanellou Peggy; Xylouri Irene; Petraki Constantina; Alexandrakis Michael; Daphnis Eugene

    2010-01-01

    Abstract Introduction The kidney is increasingly recognised as a target organ of chronic graft-versus-host disease after hematopoietic cell transplantation in the context of the development of the nephrotic syndrome. Chronic graft-versus-host disease is associated with autoimmune phenomena similar, but not identical, to those observed in various rheumatologic disorders, implicating autoimmunity as an important component of the disease. Case presentation We report the case of a 57-year-old Cau...

  1. Childhood vitiligo

    Directory of Open Access Journals (Sweden)

    Aparna Palit

    2012-01-01

    Full Text Available Childhood vitiligo is often encountered in dermatological practice. When present in infancy or early childhood, various nevoid and hereditary disorders are to be differentiated. In many cases, familial aggregation of the disease is seen and other autoimmune disorders may be associated. Segmental presentation is more common, and limited body surface area involvement is usual in this age group. Children with vitiligo often suffer from anxiety and depression because of their unusual appearance. Management of vitiligo in children is difficult as therapeutic options are restricted when compared to that in adult patients. Selection of treatment should be careful in these patients with the aim to achieve best results with minimal side effects as well as relieving patients′ and parents′ anxiety.

  2. Childhood psoriasis

    OpenAIRE

    Dogra Sunil; Kaur Inderjeet

    2010-01-01

    Psoriasis is a common dermatosis in children with about one third of all patients having onset of disease in the first or second decade of life. A chronic disfiguring skin disease, such as psoriasis, in childhood is likely to have profound emotional and psychological effects, and hence requires special attention. Psoriasis in children has been reported to differ from that among adults being more frequently pruritic; plaque lesions are relatively thinner, softer, and less scaly; face and flexu...

  3. Childhood pancreatitis.

    Science.gov (United States)

    Uretsky, G; Goldschmiedt, M; James, K

    1999-05-01

    Acute pancreatitis is a rare finding in childhood but probably more common than is generally realized. This condition should be considered in the evaluation of children with vomiting and abdominal pain, because it can cause significant morbidity and mortality. Clinical suspicion is required to make the diagnosis, especially when the serum amylase concentration is normal. Recurrent pancreatitis may be familial as a result of inherited biochemical or anatomic abnormalities. Patients with hereditary pancreatitis are at high risk for pancreatic cancer.

  4. Age-related consequences of childhood obesity.

    Science.gov (United States)

    Kelsey, Megan M; Zaepfel, Alysia; Bjornstad, Petter; Nadeau, Kristen J

    2014-01-01

    The severity and frequency of childhood obesity has increased significantly over the past three to four decades. The health effects of increased body mass index as a child may significantly impact obese youth as they age. However, many of the long-term outcomes of childhood obesity have yet to be studied. This article examines the currently available longitudinal data evaluating the effects of childhood obesity on adult outcomes. Consequences of obesity include an increased risk of developing the metabolic syndrome, cardiovascular disease, type 2 diabetes and its associated retinal and renal complications, nonalcoholic fatty liver disease, obstructive sleep apnea, polycystic ovarian syndrome, infertility, asthma, orthopedic complications, psychiatric disease, and increased rates of cancer, among others. These disorders can start as early as childhood, and such early onset increases the likelihood of early morbidity and mortality. Being obese as a child also increases the likelihood of being obese as an adult, and obesity in adulthood also leads to obesity-related complications. This review outlines the evidence for childhood obesity as a predictor of adult obesity and obesity-related disorders, thereby emphasizing the importance of early intervention to prevent the onset of obesity in childhood. PMID:24434909

  5. Children with Usher syndrome

    DEFF Research Database (Denmark)

    Dammeyer, Jesper Herup

    2012-01-01

    Background: Mental and behavioral disorders among adults with Usher syndrome have been discussed and reported in some case studies but no research has been reported on children with Usher syndrome. Methods: This article investigates the prevalence and characteristics of mental and behavioral...... disorders among 26 children, 3-17 years of age, with Usher syndrome. Results: Six of the 26 children were diagnosed with a mental or behavioral disorder (1 with schizophrenia and mild mental retardation, 1 with atypical autism and severe mental retardation, 1 with atypical autism and mild mental retardation......, 1 with mild mental retardation, and 2 with conduct disorder). Another 3 children had had a mental or behavioral disorder previously in their childhood. Conclusion: Even though vision impairment first manifests in late childhood, some children with Usher syndrome seem to develop mental and behavioral...

  6. Childhood Traumatic Grief

    Science.gov (United States)

    ... Educators Resources for Kids and Teens Childhood Traumatic Grief What is Childhood Traumatic Grief? Children grieve in their own way following the ... child may have a condition called Childhood Traumatic Grief (CTG). Thinking about the person who died—even ...

  7. Childhood Cancer Statistics

    Science.gov (United States)

    ... Shop With CureSearch Blog Donate Now Select Page Childhood Cancer Statistics Home > Understanding Children’s Cancer > Childhood Cancer Statistics Childhood Cancer Statistics – Graphs and Infographics Number of Diagnoses ...

  8. Childhood Short Stature

    Directory of Open Access Journals (Sweden)

    J.Ray

    2012-07-01

    Full Text Available Childhood short stature comprises Varity of endocrinal, systemic, Skeletal & genetic disorders of pediatrics and is not just confined for endocrinal disorder only. A systemic approach often reduces the need for test which is often expensive &unnecessary. Use growth chart & asses bone age during evaluation. Short & heavy child are generally due to Endocrine causes, Short & thin are due to systemic disease, Short with normal velocity are may be due to Constitutional delay in growth &puberty or Familial short stature, differentiation can be done by Bone Age. In Girls Turner syndrome has to be kept in mind. Purpose of evaluation to find out the child who does not need treatment, who cannot be treated & the child who can be benefited from treatment.

  9. Adiponectin in childhood.

    Science.gov (United States)

    Jeffery, Alison N; Murphy, Michael J; Metcalf, Brad S; Hosking, Joanne; Voss, Linda D; English, Patrick; Sattar, Naveed; Wilkin, Terence J

    2008-01-01

    Adiponectin, a hormone produced and secreted by adipocytes, is present in circulation in high circulating concentrations, suggesting an important physiological role. An indirect regulator of glucose metabolism, adiponectin increases insulin sensitivity, improves glucose tolerance and inhibits inflammation. Plasma adiponectin relates inversely to adiposity and, importantly, reflects the sequelae of accumulation of excess adiposity. The role of adiponectin in adults has been explored in detail. Studies in children are now available and, given the increasing rates of childhood obesity, it is important to establish the role of adiponectin in mediating insulin resistance and cardiovascular disease in this age group. This paper reviews the regulation of adiponectin, its effect on body mass, glucose metabolism and cardiovascular risk in infants, children and adolescents. It demonstrates clear links between adiponectin and features of the metabolic syndrome in obese children and adolescents. However, adiponectin's role as a predictor of metabolic dysfunction in healthy, normal-weight youngsters is less clear. PMID:19086185

  10. Neurocysticercosis presenting as Millard Gubler syndrome

    Directory of Open Access Journals (Sweden)

    Rajniti Prasad

    2012-01-01

    Full Text Available Neurocysticercosis is a common childhood neurological illness in India. A variety of presentations have been reported in the literature, including weber syndrome. Neurocysticercosis, manifesting as Millard Gubler syndrome, have not been reported in literature. Therefore, we report a child presented to us with Millard Gubler syndrome due to pontomedullary neurocysticercosis and was treated successfully.

  11. Combined dyslipidemia in childhood.

    Science.gov (United States)

    Kavey, Rae-Ellen W

    2015-01-01

    Combined dyslipidemia (CD) is now the predominant dyslipidemic pattern in childhood, characterized by moderate-to-severe elevation in triglycerides and non-high-density lipoprotein cholesterol (non-HDL-C), minimal elevation in low-density lipoprotein cholesterol (LDL-C), and reduced HDL-C. Nuclear magnetic resonance spectroscopy shows that the CD pattern is represented at the lipid subpopulation level as an increase in small, dense LDL and in overall LDL particle number plus a reduction in total HDL-C and large HDL particles, a highly atherogenic pattern. In youth, CD occurs almost exclusively with obesity and is highly prevalent, seen in more than 40% of obese adolescents. CD in childhood predicts pathologic evidence of atherosclerosis and vascular dysfunction in adolescence and young adulthood, and early clinical cardiovascular events in adult life. There is a tight connection between CD, visceral adiposity, insulin resistance, nonalcoholic fatty liver disease, and the metabolic syndrome, suggesting an integrated pathophysiological response to excessive weight gain. Weight loss, changes in dietary composition, and increases in physical activity have all been shown to improve CD significantly in children and adolescents in short-term studies. Most importantly, even small amounts of weight loss are associated with significant decreases in triglyceride levels and increases in HDL-C levels with improvement in lipid subpopulations. Diet change focused on limitation of simple carbohydrate intake with specific elimination of all sugar-sweetened beverages is very effective. Evidence-based recommendations for initiating diet and activity change are provided. Rarely, drug therapy is needed, and the evidence for drug treatment of CD in childhood is reviewed. PMID:26343211

  12. Cranial nerve palsies in childhood.

    Science.gov (United States)

    Lyons, C J; Godoy, F; ALQahtani, E

    2015-02-01

    We review ocular motor cranial nerve palsies in childhood and highlight many of the features that differentiate these from their occurrence in adulthood. The clinical characteristics of cranial nerve palsies in childhood are affected by the child's impressive ability to repair and regenerate after injury. Thus, aberrant regeneration is very common after congenital III palsy; Duane syndrome, the result of early repair after congenital VI palsy, is invariably associated with retraction of the globe in adduction related to the innervation of the lateral rectus by the III nerve causing co-contraction in adduction. Clinical features that may be of concern in adulthood may not be relevant in childhood; whereas the presence of mydriasis in III palsy suggests a compressive aetiology in adults, this is not the case in children. However, the frequency of associated CNS abnormalities in III palsy and the risk of tumour in VI palsy can be indications for early neuroimaging depending on presenting features elicited through a careful history and clinical examination. The latter should include the neighbouring cranial nerves. We discuss the impact of our evolving knowledge of congenital cranial dysinnervation syndromes on this field. PMID:25572578

  13. Reiter’s Syndrome in Childhood. A Case Report Síndrome de Reiter en la infancia. Reporte de un caso

    Directory of Open Access Journals (Sweden)

    Alexander Torres Molina

    2011-08-01

    Full Text Available Reiter’s Syndrome is a condition typical of genetically predisposed individuals. They tend to develop this syndrome after a urogenital or gastrointestinal infection. These infections are the triggering factor of complex immunological reactions responsible for the cutaneous, mucous, ocular and articular sintomatology. The most common clinical presentation is not frequent in children, but in men between 20 and 40 years old. The case of an eight years old, male patient, with clinical manifestations compatible with Reiter’s Syndrome is presented. The study and clinical description of the patient were performed. Seronegative oligoartcular arthritis of the right ankle, heel pain and anthesitis of Achilles tendon, keratoconjunctivitis and aseptic urethritis with circinate balanitis were detected. Antiflamatory treatment was initiated with favorable evolution.El Síndrome de Reiter es una afección que aparece en sujetos genéticamente predispuestos luego de una infección urogenital o gastrointestinal. Estas infecciones son el desencadenante de reacciones inmunológicas complejas responsables de la sintomatología cutánea, mucosa, ocular y articular. La forma clínica típica es poco frecuente en la infancia siendo más frecuente en hombres de entre 20 y 40 años de edad. Se presenta el caso de un paciente de sexo masculino de ocho años de edad, con manifestaciones clínicas compatibles con el diagnóstico de Síndrome de Reiter. Se realizó estudio y descripción clínica del hábito externo, se detectaron como principales rasgos distintivos: artritis oligoarticular seronegativa del tobillo derecho, talalgia y entesitis del tendón de Aquiles, queratoconjuntivitis y uretritis aséptica con balanitis circiniada. Se impuso tratamiento anti-inflamatorio con evolución favorable.

  14. Polycystic Ovary Syndrome in Adolescence

    OpenAIRE

    Buggs, Colleen; Rosenfield, Robert L.

    2005-01-01

    Polycystic ovary syndrome (PCOS) is a syndrome of variable combinations of menstrual irregularity, hirsutism or acne, and obesity. It can be diagnosed in adolescence and has early childhood antecedents. PCOS is the single most common endocrine cause of anovulatory infertility and a major risk factor for the metabolic syndrome and, in turn, development of type 2 diabetes mellitus (T2DM) in women. Thus, it appears that PCOS increases a woman’s risk of developing cardiovascular disease. Therefor...

  15. Gorlin syndrome: A case report

    Directory of Open Access Journals (Sweden)

    Patil K

    2005-01-01

    Full Text Available Gorlin syndrome is an autosomal dominant inherited condition that exhibits high penetrance and variable expressivity. It is characterized mainly by Basal cell carcinomas, Odontogenic keratocysts and skeletal anomalies. However, medical literature documents both common and lesser known manifestations of the disorder involving the skin, central nervous system, skeletal system etc. Diagnosis of the syndrome in childhood is basically through oral abnormalities. A case of Gorlin syndrome has been reported here, with review of literature.

  16. Childhood psoriasis

    Directory of Open Access Journals (Sweden)

    Dogra Sunil

    2010-01-01

    Full Text Available Psoriasis is a common dermatosis in children with about one third of all patients having onset of disease in the first or second decade of life. A chronic disfiguring skin disease, such as psoriasis, in childhood is likely to have profound emotional and psychological effects, and hence requires special attention. Psoriasis in children has been reported to differ from that among adults being more frequently pruritic; plaque lesions are relatively thinner, softer, and less scaly; face and flexural involvement is common and guttate type is the characteristic presentation. Whether onset in childhood predicts a more severe form of psoriasis is a matter of controversy, it may cause significant morbidity particularly if it keeps relapsing. Most children have mild form of psoriasis which can be generally treated effectively with topical agents such as emollients, coal tar, corticosteroids, dithranol, calcipotriol etc. according to age and the sites affected. Narrow band UVB is the preferred form of phototherapy in children for moderate to severe disease or in patients not responding to topical therapy alone. Systemic therapies are reserved for more severe and extensive cases that cannot be controlled with topical treatment and/or phototherapy such as severe plaque type, unstable forms like erythrodermic and generalized pustular psoriasis and psoriatic arthritis. There are no controlled trials of systemic therapies in this age group, most experience being with retinoids and methotrexate with favorable results. Cyclosporine can be used as a short-term intermittent crisis management drug. There is an early promising experience with the use of biologics (etanercept and infliximab in childhood psoriasis. Systemic treatments as well as phototherapy have limited use in children due to cumulative dose effects of drugs, low acceptance, and risk of gonadal toxicity. More evidence-based data is needed about the effectiveness and long-term safety of topical

  17. [A woman with beta-propeller protein-associated neurodegeneration identified by the WDR45 mutation presenting as Rett-like syndrome in childhood].

    Science.gov (United States)

    Morisada, Naoya; Tsuneishi, Syuichi; Taguchi, Kazuhiro; Yagi, Ryuzaburo; Nishiyama, Masahiro; Toyoshima, Daisaku; Nakagawa, Taku; Takeshima, Yasuhiro; Takada, Satoshi; Iijima, Kazumoto

    2016-05-01

    Beta-propeller protein-associated neurodegeneration (BPAN) is one of the neurodegenerative disorders characterized by iron deposition in the brain and is the only known disease in humans to be caused by an aberration in autophagocytosis. Here, we present the case of a 42-year-old woman with BPAN identified by the WDR45 mutation. From early childhood, she was recognized as having global developmental delay, and she frequently sucked her hand, which was considered to be a stereotypical movement. She had a febrile convulsion at 6 months of age but there was no history of epilepsy. The delay in language development was more severe than the delay in motor development; she was able to dress herself, walk unaided, and follow simple instructions until adolescence. After the age of 20, her movement ability rapidly declined. By the time she was 42 years old, she was bedridden and unable to communicate. Brain magnetic resonance imaging (MRI) at 21 years revealed no abnormality except non-specific cerebral atrophy. However, MRI at 39 years revealed abnormalities in the globus pallidus and substantia nigra, with neurodegeneration and iron accumulation in the brain. Genetic analysis for WDR45 revealed that she had a splice site mutation (NM_007075.3: c.830 + 2 T > C) which was previously reported, and a diagnosis of BPAN was confirmed. For specific therapies to be developed for BPAN in the future, it is necessary to establish early diagnosis, including genetic analysis. PMID:27349085

  18. Barraquer-Simons syndrome

    DEFF Research Database (Denmark)

    Heidemann, Lene Nyhøj; Thomsen, Jørn Bo; Sørensen, Jens Ahm

    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...

  19. Childhood psoriasis.

    Science.gov (United States)

    Farber, E M; Nall, L

    1999-11-01

    Psoriasis is a common skin disease in infants, children, and adolescents. A review of the clinical, epidemiologic, genetic, and therapeutic aspects of childhood psoriasis is presented. Population studies indicate that the first signs of psoriatic lesions occur in the pediatric age group, birth to 18 years of age, and that both genetic and environmental factors interact to precipitate the development of psoriasis. Koebner reactions are the result of external or internal triggering factors, such as physical injury to the skin, low humidity, and certain drugs. The most frequently observed variant to psoriasis is the plaque type, followed by guttate psoriasis, and juvenile psoriatic arthritis. Pustular psoriasis and erythrodermic psoriasis are rare forms of the disease, but are seen in children from infancy to adolescence. The scalp is the most frequently affected site of involvement in pediatric psoriasis, followed by the appearance of lesions on the extensor surfaces of the extremities, trunk, and nails. Although not common in adult psoriasis, the face and ears are often involved. Topical medications such as corticosteroids, calcipotriol, coal tar preparations, anthralin formulations, and ultraviolet B are recommended in monotherapy or in combination therapy, whereas psoralen plus ultraviolet A, methotrexate, and retinoids should only be administered in crisis situations. The treatment objectives in childhood psoriasis are to preserve skin surfaces, to afford physical relief from the disease, and to employ treatments that do not endanger the health or future development of the child.

  20. Beckwith-Wiedemann syndrome: dental management.

    LENUS (Irish Health Repository)

    Garvey, M T

    1997-06-01

    Beckwith-Wiedemann syndrome (BWS) comprises multiple congenital anomalies with a risk of childhood tumours. Macroglossia is the most common manifestation. Two cases are presented to illustrate the importance of early referral and the role of preventive dentistry.

  1. An Evaluation of Acylated Ghrelin and Obestatin Levels in Childhood Obesity and Their Association with Insulin Resistance, Metabolic Syndrome, and Oxidative Stress

    Science.gov (United States)

    Razzaghy-Azar, Maryam; Nourbakhsh, Mitra; Pourmoteabed, Abdolreza; Nourbakhsh, Mona; Ilbeigi, Davod; Khosravi, Mohsen

    2016-01-01

    Background: Ghrelin is a 28-amino acid peptide with an orexigenic property, which is predominantly produced by the stomach. Acylated ghrelin is the active form of this hormone. Obestatin is a 23-amino acid peptide which is produced by post-translational modification of a protein precursor that also produces ghrelin. Obestatin has the opposite effect of ghrelin on food intake. The aim of this study was to evaluate acylated ghrelin and obestatin levels and their ratio in obese and normal-weight children and adolescents, and their association with metabolic syndrome (MetS) parameters. Methods: Serum acyl-ghrelin, obestatin, leptin, insulin, fasting plasma glucose (FPG), lipid profile, and malondialdehyde (MDA) were evaluated in 73 children and adolescents (42 obese and 31 control). Insulin resistance was calculated by a homeostasis model assessment of insulin resistance (HOMA-IR). MetS was determined according to IDF criteria. Results: Acyl-ghrelin levels were significantly lower in obese subjects compared to the control group and lower in obese children with MetS compared to obese subjects without MetS. Obestatin was significantly higher in obese subjects compared to that of the control, but it did not differ significantly among those with or without MetS. Acyl-ghrelin to obestatin ratio was significantly lower in obese subjects compared to that in normal subjects. Acyl-ghrelin showed significant negative and obestatin showed significant positive correlations with body mass index (BMI), BMI Z-score, leptin, insulin, and HOMA-IR. Acyl-ghrelin had a significant negative correlation with MDA as an index of oxidative stress. Conclusion: Ghrelin is decreased and obestatin is elevated in obesity. Both of these hormones are associated with insulin resistance, and ghrelin is associated with oxidative stress. The balance between ghrelin and obestatin seems to be disturbed in obesity. PMID:27348010

  2. Extending the mutation spectrum for Galloway-Mowat syndrome to include homozygous missense mutations in the WDR73 gene.

    Science.gov (United States)

    Rosti, Rasim O; Dikoglu, Esra; Zaki, Maha S; Abdel-Salam, Ghada; Makhseed, Nawal; Sese, Jordan C; Musaev, Damir; Rosti, Basak; Harbert, Mary J; Jones, Marilyn C; Vaux, Keith K; Gleeson, Joseph G

    2016-04-01

    Galloway-Mowat syndrome is a rare autosomal-recessive disorder classically described as the combination of microcephaly and nephrotic syndrome. Recently, homozygous truncating mutations in WDR73 (WD repeat domain 73) were described in two of 31 unrelated families with Galloway-Mowat syndrome which was followed by a report of two sibs in an Egyptian consanguineous family. In this report, seven affecteds from four families showing biallelic missense mutations in WDR73 were identified by exome sequencing and confirmed to follow a recessive model of inheritance. Three-dimensional modeling predicted conformational alterations as a result of the mutation, supporting pathogenicity. An additional 13 families with microcephaly and renal phenotype were negative for WDR73 mutations. Missense mutations in the WDR73 gene are reported for the first time in Galloway-Mowat syndrome. A detailed phenotypic comparison of all reported WDR73-linked Galloway-Mowat syndrome patients with WDR73 negative patients showed that WDR73 mutations are limited to those with classical Galloway-Mowat syndrome features, in addition to cerebellar atrophy, thin corpus callosum, brain stem hypoplasia, occasional coarse face, late-onset and mostly slow progressive nephrotic syndrome, and frequent epilepsy. PMID:27001912

  3. Childhood medulloblastoma.

    Science.gov (United States)

    Massimino, Maura; Biassoni, Veronica; Gandola, Lorenza; Garrè, Maria Luisa; Gatta, Gemma; Giangaspero, Felice; Poggi, Geraldina; Rutkowski, Stefan

    2016-09-01

    Medulloblastoma accounts for 15-20% of childhood nervous system tumours. The risk of dying was reduced by 30% in the last twenty years. Patients are divided in risk strata according to post-surgical disease, dissemination, histology and some molecular features such as WNT subgroup and MYC status. Sixty to 70% of patients older than 3 years are assigned to the average-risk group. High-risk patients include those with disseminated and/or residual disease, large cell and/or anaplastic histotypes, MYC genes amplification. Current and currently planned clinical trials will: (1) evaluate the feasibility of reducing both the dose of craniospinal irradiation and the volume of the posterior fossa radiotherapy (RT) for those patients at low biologic risk, commonly identified as those having a medulloblastoma of the WNT subgroup; (2) determine whether intensification of chemotherapy (CT) or irradiation can improve outcome in patients with high-risk disease; (3) find target therapies allowing tailored therapies especially for relapsing patients and those with higher biological risk. PMID:27375228

  4. Application of nuclear medicine to heart diseases in childhood

    International Nuclear Information System (INIS)

    Various procedures to obtain radioisotopic images of the cardiovascular system were described, and representative cases in childhood were presented. Apparatuses, radiopharmaceuticals such as sup(99m)Tc pertechnatate, 201Thallium, and 133Xenon gas, and their dosage in childhood were briefly reviewed. Several cases of persistent fetal circulation, common A-V valve, tetralogy of Fallot, pulmonary atresia, etc. were presented with figures with special emphasis on acute febrile mucocutaneous lymph node syndrome and 201Thallium myocardial imaging. (Kondo, M.)

  5. Childhood Overweight and Obesity

    Science.gov (United States)

    ... Childhood Obesity Facts The prevalence of obesity among low-income children aged 2 through 4 years, by state ... Obesity now affects 1 in 6 children and adolescents in the United States. Childhood Obesity Facts How ...

  6. Childhood Brain Tumors

    Science.gov (United States)

    Brain tumors are abnormal growths inside the skull. They are among the most common types of childhood ... still be serious. Malignant tumors are cancerous. Childhood brain and spinal cord tumors can cause headaches and ...

  7. Rhabdomyosarcoma in patients with constitutional mismatch-repair-deficiency syndrome

    OpenAIRE

    Kratz, Christian P; Holter, Spring; Etzler, Julia; Lauten, Melchior; Pollett, Aaron; Charlotte M. Niemeyer; Gallinger, Steven; Wimmer, Katharina

    2009-01-01

    Abstract Background: Biallelic germline mutations in the mismatch repair genes MLH1, MSH2, MSH6 or PMS2 cause a recessive childhood cancer syndrome characterized by early-onset malignancies and signs reminiscent of neurofibromatosis type 1 (NF1). Alluding to the underlying genetic defect we refer to this syndrome as constitutional mismatch repair-deficiency (CMMR-D) syndrome. The tumor spectrum of CMMR-D syndrome includes hematological neoplasias, brain tumors and Lynch syndrome as...

  8. 儿童阻塞性睡眠呼吸暂停低通气综合征麻醉方式的选择%Choose the method of anesthetization for childhood obstructive sleep apnea hypopnea syndrome

    Institute of Scientific and Technical Information of China (English)

    段宗瑾; 朱明

    2010-01-01

    目的 探讨手术治疗儿童阻塞性睡眠呼吸暂停低通气综合征(OSAHS)的麻醉方式.方法 选择98例扁桃体伴腺样体肥大患儿,随机分为局部浸润麻醉组(局麻组)和全身静脉麻醉组(全麻组).局麻组行扁桃体挤切、腺样体刮除;全麻组行扁桃体剥离术,小儿鼻内镜直视下腺样体刮除.对两组出血量、手术时间、进食时间、术后残体、住院费用及手术疗效进行比较.结果 全麻组术中出血多,手术时间长,住院费用高;而局麻组术后残体多,个别患儿不配合需改用全麻.但两种麻醉方式下,术后睡眠打鼾、张口呼吸、憋气等症状明显改善,手术疗效几乎相同.结论 两种麻醉方式各有优缺点,但局麻下手术治疗儿童OSAHS与全麻一样安全有效,应针对不同情况选择.%Objective To discuss how to choose the method of anesthetization for childhood obstructive sleep apnea hypopnea syndrome(OSAHS).Methods 98 children with tonsillar and adenoidal hypertrophy were seleted,and were randomly divided into two groups.Tonsillectomy and adenoidectomy were performed for the group with local infiltration anesthesia,while Tonsil dissection and endoscopic-assisted adenoidectomy through nose were for the group with intravenous general anesthesia.Then blood loss,operation time,intake time,operation damage,psychological trau-ma,hospitalization fares and operative efficacy were compared.Results The group with general anesthesia lost more blood,underwent longer operations with higher cost;the group with local anesthesia had more operation damage,grea-ter psyhologieal trauma and sometimes general anesthesia was necessary for the children who didn't cooperate.But these two methods of anesthetization almost had the same operative efficacy,and the symptoms such as snoring,oral breathing and suffocating were significantly improved after surgery.Condusion Every method had own advantages and disadvantages.Local anesthesia was as effective as

  9. Schizophrenia patients with a history of childhood trauma have a pro-inflammatory phenotype

    OpenAIRE

    Dennison, Una; McKernan, Declan P.; Cryan, John F; Dinan, Timothy G.

    2012-01-01

    Background. Increasing evidence indicates that childhood trauma is a risk factor for schizophrenia and patients with this syndrome have a pro-inflammatory phenotype. We tested the hypothesis that the pro-inflammatory phenotype in schizophrenia is associated with childhood trauma and that patients without a history of such trauma have a similar immune profile to healthy controls. Method. We recruited 40 schizophrenia patients and 40 controls, all of whom completed the Childhood Trauma ...

  10. Pathogenesis of neurocognitive and neuropsychiatric manifestations in childhood-onset lupus: An overview

    OpenAIRE

    Tanya Baqai; Isenberg, David A; Yiannis Ioannou

    2013-01-01

    This review explores current understanding of neuropsychiatric systemic lupus erythe-matosus (NPSLE) of childhood onset, in particular neurocognitive impairment. As yet, fewer studies have focused on childhood onset NPSLE compared to adult onset NPSLE and diagnosis still involves the 1999 American College of Rheumatology case definitions of neuropsychiatric syndromes, which were developed for adults. Although a validated core set of neuropsychometric tests exist for childhood onset NPSLE, the...

  11. Mitral valve disease in Marfan's syndrome.

    OpenAIRE

    Marlow, N; Gregg, J. E.; Qureshi, S A

    1987-01-01

    Cardiovascular disease in Marfan's syndrome presenting in childhood affects the mitral valve more often than the aortic valve or the aorta, as in adults. Early evaluation of the cardiovascular system is necessary for any child in whom Marfan's syndrome is suspected.

  12. Childhood proptosis

    International Nuclear Information System (INIS)

    Proptosis in children is a hallmark of orbital diseases which can present a diagnostic challenge requiring thoughtful investigation. The aim of this review is to provide the reader an overview of the subject of childhood proptosis with an emphasis on the systematic and practical approach for the work-up of proptosis in children. Use of proper imaging studies is essential for the correct diagnosis. Computed tomography is a good screening test for any space occupying lesion of the orbit. Proptosis describes eye prominence due to space occupying orbital lesions. Congenital lesions usually present in the first decade of life. Acquired orbital lesions such as lymphangiomas, orbital varix, rhabdomyosarcoma and neural tumors may present at the end of the first decade of life. Metastatic tumors to the orbit, adenocarcinoma of lacrimal gland and rapidly growing masses may present with proptosis associated with pain. Visual loss can be the presenting symptoms in the patients with optic nerve (ON) gliomas, orbital meningiomas and posteriorly located tumors. Cystic lesions of the orbit may be congenital or acquired, dermoid cysts being the most common congenital orbital lesions. Some of the vascular lesions of the orbit include capillary hemangiomas, lymphangiomas, orbital varix, and arteriovenous malformations. Inflammatory process of the orbit in children include cellulitis and pseudotumor. Neural tumors such as neurofibromas, ON gilomas and meningiomas are less common causes of proptosis in children. Rhabdomyosarcoma is the most common primary orbital malignancy in children which can present with acute proptosis and is one of the few life-threatening diseases seen initially by an ophthalmologist. Secondary orbital tumors invade the orbit from adjacent sinuses, cranium or extended from the eye itself. The most common distant metastases in children include neuroblastoma and Ewing's sarcoma. Although many orbital processes can be diagnosed based on history, clinical

  13. 46,XX Male Syndrome

    Directory of Open Access Journals (Sweden)

    Bekir Uçan

    2013-06-01

    Full Text Available 46, XX male syndrome – testicular disorder of sexual differentiation (DSD is a rare condition characterized by a spectrum of clinical presentations, ranging from ambiguous to normal male genitalia. These cases are diagnosed more easily in childhood. In adults, the diagnosis can be difficult due to the current normal gender development. Here, we report hormonal, molecular and cytogenetic results in an adult male patient with primary hypogonadism who was diagnosed with 46, XX male syndrome in our clinic. Turk Jem 2013; 17: 46-8

  14. Treatment of Tourette Syndrome

    OpenAIRE

    Kurlan, Roger M.

    2013-01-01

    Tourette’s syndrome (TS) consists of chronic motor and phonic tics and characteristically begins in childhood. The tics can be disabling and commonly associated behavioral comorbities such as attention deficit hyperactivity disorder (ADHD) and obsessive-compulsive disorder (OCD), can also cause problems in daily functioning. The underlying etiology and neurobiology of TS remain unknown although genetic factors appear to be important, cortical control of basal ganglia motor function appears to...

  15. Contributors to Adult Sibling Relationships and Intention to Care of Siblings of Individuals with Down Syndrome

    Science.gov (United States)

    Cuskelly, Monica

    2016-01-01

    The contribution of childhood sibling relationships to adult sibling relationships and intention to provide care was investigated in a sample in which one member of each dyad had Down syndrome. Thirty-nine adult siblings of an adult with Down syndrome who had participated in a study of sibling relationships in childhood/adolescence provided data…

  16. Beals Syndrome

    Science.gov (United States)

    ... Boards & Staff Annual Report & Financials Contact Us Donate Marfan & Related Disorders What is Marfan Syndrome? What are ... the syndrome. How does Beals syndrome compare with Marfan syndrome? People with Beals syndrome have many of ...

  17. Chromosomal rearrangements in Tourette syndrome

    DEFF Research Database (Denmark)

    Bertelsen, Birgitte; Debes, Nanette Mol; Hjermind, Lena E;

    2013-01-01

    Tourette syndrome (TS) is a childhood-onset complex neurobiological disorder characterized by a combination of persistent motor and vocal tics and frequent presence of other neuropsychiatric comorbidities. TS shares the fate of other complex disorders, where the genetic etiology is largely unknown...

  18. Sotos syndrome

    Directory of Open Access Journals (Sweden)

    Cormier-Daire Valérie

    2007-09-01

    Full Text Available Abstract Sotos syndrome is an overgrowth condition characterized by cardinal features including excessive growth during childhood, macrocephaly, distinctive facial gestalt and various degrees of learning difficulty, and associated with variable minor features. The exact prevalence remains unknown but hundreds of cases have been reported. The diagnosis is usually suspected after birth because of excessive height and occipitofrontal circumference (OFC, advanced bone age, neonatal complications including hypotonia and feeding difficulties, and facial gestalt. Other inconstant clinical abnormalities include scoliosis, cardiac and genitourinary anomalies, seizures and brisk deep tendon reflexes. Variable delays in cognitive and motor development are also observed. The syndrome may also be associated with an increased risk of tumors. Mutations and deletions of the NSD1 gene (located at chromosome 5q35 and coding for a histone methyltransferase implicated in transcriptional regulation are responsible for more than 75% of cases. FISH analysis, MLPA or multiplex quantitative PCR allow the detection of total/partial NSD1 deletions, and direct sequencing allows detection of NSD1 mutations. The large majority of NSD1 abnormalities occur de novo and there are very few familial cases. Although most cases are sporadic, several reports of autosomal dominant inheritance have been described. Germline mosaicism has never been reported and the recurrence risk for normal parents is very low (

  19. Childhood trauma and childhood urbanicity in relation to psychotic disorder

    NARCIS (Netherlands)

    Frissen, Aleida; Lieverse, Ritsaert; Drukker, Marjan; van Winkel, Ruud; Delespaul, Philippe; Cahn, W

    2015-01-01

    BACKGROUND: Urban upbringing and childhood trauma are both associated with psychotic disorders. However, the association between childhood urbanicity and childhood trauma in psychosis is poorly understood. The urban environment could occasion a background of social adversity against which any effect

  20. Metabolic Syndrome in Childhood Cancer Survivors

    OpenAIRE

    Waas, Marjolein

    2012-01-01

    textabstractOver 200,000 children under the age of fifteen are diagnosed with cancer worldwide every year. Cancer is the second most common cause of death among children between the ages of 1 and 14 years in developed countries, surpassed only by accidents.Nearly one third of the cancers diagnosed in children are leukemias (particularly acute lymphoblastic leukemia (ALL)), followed by cancer of the brain or central nervous system (21%), soft tissue sarcomas (including neuroblastoma (7%) and r...

  1. Depressive symptoms and childhood sleep apnea syndrome

    Directory of Open Access Journals (Sweden)

    Carotenuto M

    2012-08-01

    Full Text Available Marco Carotenuto,1 Maria Esposito,1 Lucia Parisi,2 Beatrice Gallai,3 Rosa Marotta,4 Antonio Pascotto,1 Michele Roccella21Sleep Clinic for Developmental Age, Clinic of Child and Adolescent Neuropsychiatry, Second University of Naples, Naples, Italy; 2Child Neuropsychiatry, Department of Psychology, University of Palermo, Palermo, Italy; 3Unit of Child and Adolescent Neuropsychiatry, University of Perugia, Perugia, 4Department of Psychiatry, "Magna Graecia" University of Catanzaro, Catanzaro, ItalyBackground: The relationship between sleep and mood regulation is well known, and some reports suggest a key role of sleep-related breathing disorders (SRBD in the development of the symptomatology of depression, even if no conclusive data are actually found in the clinical literature. The aim of this study was to assess the relationship between SRBD and depressive symptoms in a population of school-aged children.Methods: The study population comprised 94 children affected by SRBD and 107 healthy children. To identify the severity of SRBD, an overnight respiratory evaluation was performed. All subjects filled out the Italian version of the Children Depression Inventory (CDI to screen for the presence of depressive symptoms.Results: The group with SRBD showed higher CDI scores than the group without SRBD, with a positive correlation found between CDI scores, apnea-hypopnea index, and oxygen desaturation index values. Logistic regression showed that an apnea-hypopnea index ≥ 3 and an oxygen desaturation index ≥ 1 could be risk factors for development of depressive symptoms. According to receiver-operating characteristic curve analysis, the cutoff point for the apnea-hypopnea index that could cause a pathological CDI score (≥19 was >5.66, and the cutoff point for the oxygen desaturation index was >4.2. The limitations of this study are that our data are derived from one single psychometric test and not from a complete psychiatric evaluation, and our subjects came from a small group in southern Italy.Conclusion: Our results suggest the importance of mood assessment in children affected by SRBD.Keywords: depression, sleep-related breathing disorders, cardiorespiratory monitoring, children

  2. CHILDHOOD HEADACHE SYNDROMES(PART II

    Directory of Open Access Journals (Sweden)

    Mohammad GHOFRANI

    2010-10-01

    Full Text Available ObjectiveHeadache is one of the most common reason that children are referred to the Pediatric Neurology Services. It is said that ten percent of children aged 5 to 15 years have migraine.Subsequently, it is essential for clinician to have a through, comprehensive and systematic approach to the evaluation and management of the child or adolescent who complains of headache.This writing aims to explore the symptoms of headache, its epidemiology, classification, appropriate evaluation, differential diagnosis and management.Headaches are divided into primary and secondary categories. Migraine and tension type headaches are prototype of primary headaches without underlying pathology. On the other hand, the type of headache which stems from organic diseases such as: brain tumor, increased intracranial pressure, systemic disease, drug toxicity, ear-nose and throat problems are considered secondary.On the whole, the majority of children with primary headache have two patterns of headache. One is a chronic low-grade and the other is an intermittent disabling headache. The cause of the former is either caffeine or analgesic abuse, and the latter is predominantly migraine.Traditionally, if a child presents himself with chief complain of headache, care taker physician begins with history taking followed by thorough physical and neurological examinations.In the majority of the cases, this initial process leads to a diagnosis or indicate the need for further testing.Once the diagnosis is made, a management program can be put into place.Keywords:Headaches, Child, Children, Migraine

  3. Depressive symptoms and childhood sleep apnea syndrome

    OpenAIRE

    Carotenuto M; Esposito M; Parisi L; Gallai B; Marotta R; Pascotto A; Roccella M

    2012-01-01

    Marco Carotenuto,1 Maria Esposito,1 Lucia Parisi,2 Beatrice Gallai,3 Rosa Marotta,4 Antonio Pascotto,1 Michele Roccella21Sleep Clinic for Developmental Age, Clinic of Child and Adolescent Neuropsychiatry, Second University of Naples, Naples, Italy; 2Child Neuropsychiatry, Department of Psychology, University of Palermo, Palermo, Italy; 3Unit of Child and Adolescent Neuropsychiatry, University of Perugia, Perugia, 4Department of Psychiatry, "Magna Graecia" University of Catan...

  4. Childhood Headache Syndromes(Part I

    Directory of Open Access Journals (Sweden)

    Mohammad GHOFRANI

    2010-07-01

    Full Text Available Headache is one of the most common reason that children are referred to the Pediatric Neurology Services. It is said that ten percent of children aged 5 to 15 years have migraine. Subsequently, it is essential for clinician to have a through, comprehensive and systematic approach to the evaluation and management of the child or adolescent who complain of headache.This writing aims to explore the symptoms of headache, its epidemiology, classification, appropriate evaluation, differential diagnosis and management.Headaches are divided into primary and secondary categories. Migraine and tension type headaches are prototype of primary headaches without underlying pathology. On the other hand, the type of headache which stems from organic diseases such as: brain tumor, increased intracranial pressure, systemic disease, drug toxicity or Ear Nose  and Throat problems are considered secondary. On the whole, the majority of children with primary headache have two patterns of headache. One is a chronic low-grade and the other is an intermittent disabling headache. The cause of the former is either caffeine or analgesic abuse, and the latter is predominantly migraine. Traditionally, if a child presents himself with chief complain of headache, care taker physician begins with history taking followed by thorough physical and neurological examinations. In the majority of the cases, this initial process leads to a diagnosis or indicate the need for further testing. Once the diagnosis is made, a management program can be put into place.Key words: Headaches, Child,  Children, Migraine. 

  5. Childhood Headache Syndromes(Part I

    Directory of Open Access Journals (Sweden)

    Mohammad Ghofrani

    2010-06-01

    Full Text Available AbstractHeadache is one of the most common reason that children are referred to the Pediatric Neurology Services. It is said that ten percent of children aged 5 to 15 years have migraine. Subsequently, it is essential for clinician to have a through, comprehensive and systematic approach to the evaluation and management of the child or adolescent who complain of headache.This writing aims to explore the symptoms of headache, its epidemiology, classification, appropriate evaluation, differential diagnosis and management.Headaches are divided into primary and secondary categories. Migraine and tension type headaches are prototype of primary headaches without underlying pathology. On the other hand, the type of headache which stems from organic diseases such as: brain tumor, increased intracranial pressure, systemic disease, drug toxicity or Ear Nose and Throat problems are considered secondary. On the whole, the majority of children with primary headache have two patterns of headache. One is a chronic low-grade and the other is an intermittent disabling headache. The cause of the former is either caffeine or analgesic abuse, and the latter is predominantly migraine. Traditionally, if a child presents himself with chief complain of headache, care taker physician begins with history taking followed by thorough physical and neurological examinations. In the majority of the cases, this initial process leads to a diagnosis or indicate the need for further testing. Once the diagnosis is made, a management program can be put into place.Headaches, Child, Children, Migraine.

  6. CHILDHOOD HEADACHE SYNDROMES(PART II

    Directory of Open Access Journals (Sweden)

    Mohammad GHOFRANI

    2010-09-01

    Full Text Available ObjectiveHeadache is one of the most common reason that children are referred to the Pediatric Neurology Services. It is said that ten percent of children aged 5 to 15 years have migraine.Subsequently, it is essential for clinician to have a through, comprehensive and systematic approach to the evaluation and management of the child or adolescent who complains of headache.This writing aims to explore the symptoms of headache, its epidemiology, classification, appropriate evaluation, differential diagnosis and management.Headaches are divided into primary and secondary categories. Migraine and tension type headaches are prototype of primary headaches without underlying pathology. On the other hand, the type of headache which stems from organic diseases such as: brain tumor, increased intracranial pressure, systemic disease, drug toxicity, ear-nose and throat problems are considered secondary.On the whole, the majority of children with primary headache have two patterns of headache. One is a chronic low-grade and the other is an intermittent disabling headache. The cause of the former is either caffeine or analgesic abuse, and the latter is predominantly migraine.Traditionally, if a child presents himself with chief complain of headache, care taker physician begins with history taking followed by thorough physical and neurological examinations.In the majority of the cases, this initial process leads to a diagnosis or indicate the need for further testing.Once the diagnosis is made, a management program can be put into place.

  7. Disorders of childhood growth and development: childhood obesity.

    Science.gov (United States)

    Mendez, Robert; Grissom, Maureen

    2013-07-01

    The incidence of childhood obesity in the United States is estimated at 17%, or 12 million children ages 2 to 19 years. Obesity is a multifactorial condition with syndromic and nonsyndromic variants. Genetic, social, ethnic, endocrinologic, and behavioral issues are all potential etiologic factors. Preventive efforts should begin with monitoring from birth and include breastfeeding until age 6 months, avoiding juices, and promoting fruit and vegetable consumption and adequate exercise. Childhood obesity is diagnosed based on body mass index; a child is considered overweight at the 85th to 95th percentiles and obese at or above the 95th percentile. After obesity is diagnosed, testing should include blood pressure levels, fasting lipid profile, diabetes screening, and liver function tests. The physician should obtain a detailed history of the physical activity level and food intake and assess possible complications of obesity, including depression and hypertension, annually. Lifestyle interventions with family involvement are the mainstay of management, with pharmacotherapy or bariatric surgery considered for adolescents only if intensive lifestyle modifications have failed and in the presence of comorbidities. Intervention by multiple disciplines (ie, medicine, nutrition, psychology) is recommended, and family physicians are encouraged to become more involved in encouraging physical activity and improved nutrition for children.

  8. Childhood as a value

    OpenAIRE

    EWELINA PIECUCH

    2011-01-01

    The article encompasses the problems of childhood and its influence on the rest of one's life. I have concentrated on this crucial and specific time in life. It is demonstrated by biology, medicine, psychology, and psychoanalysis that human habits are formed in childhood. Health, hygiene and aesthetic behaviour determine one's further fate and influence life in its entirety. It is that phase of human life that determines the rest of it. In childhood children manifest their cogn...

  9. Clinical and Experimental Study on Treatment of Childhood Hyperkinetic Syndrome with Yizhidan%益智丹治疗儿童多动症的临床与实验研究

    Institute of Scientific and Technical Information of China (English)

    陈永辉; 黄斌; 赵霞

    2001-01-01

    Objective:To observe the clinical effect of Yizhidan (YZD) intreating childhood hyperkinetic syndrome (CHS) and its mechanism. Methods: Random single blind method was used in clinical study to observe the clinical effect of YZD and compared with that of Ritalin. The dysmnesia model animal was used to observe the effect of YZD on learning memory function and cerebral monoamine neurotransmitter content. Results: The markedly effective rate and the total effective rate of YZD were 45.56% and 87.78%, which were similar to those of Ritalin (53.33% and 86.67%). The Conners index lowered in both groups, showing insignificant difference (P>0.05). Experimental observation showed that YZD could significantly improve the soft neurologic signs and abnormal encephalogram with less side-effects in comparing with Ritalin (P<0.05). Moreover, YZD could obviously improve the learning memory disturbance induced by scopolamine, sodium nitrite and ethanol, and significantly increased the contents of DA in cerebral tissue, as compared with that of the control group, the difference was significant (P<0.01). It also showed effect on DOPAC, NE, HVA, 5-HT and 5-HIAA of mice in certain degree. Conclusion: YZD is an effective drug in treating CHS, its mechanism might be probably related to the improvement of the memory and the enhancement of DA receptor in cerebral tissue.%目的:观察益智丹治疗儿童多动症(CHS)的临床疗效及其作用机制。方法:临床研究采用单盲法随机观察益智丹与利他林的临床疗效;实验研究制作记忆障碍模型,观察益智丹对小鼠学习记忆功能和对小鼠脑组织单胺类神经递质含量的影响。结果:益智丹治疗CHS的显效率、总有效率分别为45.56%、87.78%,与利他林(53.33%、86.67%)相当;Conners指数均明显降低,两组间疗效及多动指数比较差异均无显著性(P>0.05)。益智丹还能显著改善患儿软神经征及异常脑电图,副反

  10. Childhood Cancer Survivor Study: An Overview

    Science.gov (United States)

    ... Cancers of Childhood Treatment Childhood Cancer Genomics Research Childhood Cancer Survivor Study: An Overview In 2016, it ... Late Effects of Treatment for Childhood Cancer .) The Childhood Cancer Survivor Study ( CCSS ), funded by the National ...

  11. Asperger syndrome related suicidal behavior: two case studies.

    Science.gov (United States)

    Kocourkova, Jana; Dudova, Iva; Koutek, Jiri

    2013-01-01

    Asperger syndrome hinders adaptation to developmental challenges during childhood and adolescence, particularly with regard to interpersonal relationships. Individuals with Asperger syndrome display lack of empathy and limited ability to understand social and emotional exchanges with other people. Individuals with Asperger syndrome are significantly exposed to the risk of suicidal behavior, especially during adolescence. The authors describe cases of suicidal behavior in two adolescent boys with Asperger syndrome. PMID:24294002

  12. Asperger syndrome related suicidal behavior: two case studies.

    Science.gov (United States)

    Kocourkova, Jana; Dudova, Iva; Koutek, Jiri

    2013-01-01

    Asperger syndrome hinders adaptation to developmental challenges during childhood and adolescence, particularly with regard to interpersonal relationships. Individuals with Asperger syndrome display lack of empathy and limited ability to understand social and emotional exchanges with other people. Individuals with Asperger syndrome are significantly exposed to the risk of suicidal behavior, especially during adolescence. The authors describe cases of suicidal behavior in two adolescent boys with Asperger syndrome.

  13. Hyponatremic-Hypertensive Syndrome in an 18-Month-Old Male Child

    Directory of Open Access Journals (Sweden)

    Dilek Yilmaz

    2016-04-01

    Full Text Available An eighteen-month-old boy presented with polyuria, polydipsia, hypertension, severe hyponatremia, metabolic alkalosis and nephrotic-range proteinuria. Hypertension was drug resistant. Renal artery angiogram revealed right renal artery occlusion. Nonfunctional right kidney was also detected on the nuclear renal scan. As percutaneous transluminal renal artery angioplasty was not appropriate for our patient, nephrectomy was performed. Right nephrectomy resulted in the resolution of all the symptoms. Rarity of hyponatremic hypertensive syndrome in children may project its under-recognition. We aimed to increase awareness of early diagnosis of HHS among polyuric, hypertensive and hyponatremic children in order to prevent renal damage and life-threatening complications.

  14. Late endocrine effects of childhood cancer.

    Science.gov (United States)

    Rose, Susan R; Horne, Vincent E; Howell, Jonathan; Lawson, Sarah A; Rutter, Meilan M; Trotman, Gylynthia E; Corathers, Sarah D

    2016-06-01

    The cure rate for paediatric malignancies is increasing, and most patients who have cancer during childhood survive and enter adulthood. Surveillance for late endocrine effects after childhood cancer is required to ensure early diagnosis and treatment and to optimize physical, cognitive and psychosocial health. The degree of risk of endocrine deficiency is related to the child's sex and their age at the time the tumour is diagnosed, as well as to tumour location and characteristics and the therapies used (surgery, chemotherapy or radiation therapy). Potential endocrine problems can include growth hormone deficiency, hypothyroidism (primary or central), adrenocorticotropin deficiency, hyperprolactinaemia, precocious puberty, hypogonadism (primary or central), altered fertility and/or sexual function, low BMD, the metabolic syndrome and hypothalamic obesity. Optimal endocrine care for survivors of childhood cancer should be delivered in a multidisciplinary setting, providing continuity from acute cancer treatment to long-term follow-up of late endocrine effects throughout the lifespan. Endocrine therapies are important to improve long-term quality of life for survivors of childhood cancer. PMID:27032982

  15. Dissociative experiences as mediators between childhood trauma and auditory hallucinations.

    Science.gov (United States)

    Perona-Garcelán, Salvador; Carrascoso-López, Francisco; García-Montes, José M; Ductor-Recuerda, María Jesús; López Jiménez, Ana Ma; Vallina-Fernández, Oscar; Pérez-Álvarez, Marino; Gómez-Gómez, María Teresa

    2012-06-01

    The purpose of this work was to study the relationship between reported traumatic experiences in childhood and positive psychotic symptoms. We hypothesized that dissociative experiences were potential mediators between childhood trauma and hallucinations, but not delusions. The sample comprised 71 patients diagnosed with psychoses. They were assessed with the Dissociative Experiences Scale (DES-II; Carlson & Putnam, 1993), a questionnaire on trauma (TQ; Davidson, Hughes, & Blazer, 1990), and the Positive and Negative Syndrome Scale (PANSS; Kay, Opler, & Lindenmayer, 1988) delusions and hallucinations items. The results showed that childhood trauma was positively associated with the dissociation scale scores (r = .40) and also the hallucination (r = .36) and delusions scale scores (r = .32). Furthermore, it was demonstrated that the dissociation variable was a potential mediator between childhood trauma and hallucinations, but not between childhood trauma and delusions. Of the 3 DES-II factors, only depersonalization showed a mediating relationship between childhood trauma and hallucinations. The main conclusion is that the impact of childhood trauma on hallucinations may not simply be direct, but mediated by dissociative experiences, especially depersonalization. Clinical implications are also briefly discussed. PMID:22589015

  16. Childhood Obesity. ERIC Digest.

    Science.gov (United States)

    Summerfield, Liane M.

    In this discussion of childhood obesity, the medical and psychological problems associated with the condition are noted. Childhood obesity most likely results from an interaction of nutritional, psychological, familial, and physiological factors. Three factors--the family, low-energy expenditure, and heredity--are briefly examined. Early…

  17. Efficacy of olanzapine versus methylphenidate treatment for childhood hyperkinetic syndrome%奥氮平与哌醋甲酯治疗儿童多动症效果对照

    Institute of Scientific and Technical Information of China (English)

    郝学儒; 崔文波

    2005-01-01

    BACKGROUND: Attention deficit and hyperkinetic disorder (ADHD) is frequently treated with psychostimulant medications, which had been shown to improve both cognitive and behavioral outcomes for most chil dren. OBJECTIVE: To compare the efficacy and adverse effects of olanzapine vs methylphenidate treatment in childhood hyperkinetic syndrome.DESIGN: Clinical comparative study. SETTING: Department of Psychiatry, Beijing Huilongguan Hospital; De partment of Mental Psychiatry, Shangdong Mouping People's Hospital. PARTICIPANT S: Sixty children with hyperkinetic syndrome from the clinics of the Department of Psychiatry,Beijing Huilongguan Hospital and the Department of Mental Psychiatry of Shandong Mouping People's Hos pital between March 2002 and April 2004 were enrolled into this prospec tive study after obtaining the consents from their guardians.The patients were randomly divided into 2 group: the olanzapine group (n=30) and the methylphenidate group (n=30). METHODS: In the olanzapine group olanzapine 2.5-7.5 mg per day was given as a single dose in every evening.In the methylphenidate group methylphenidate 5-15 mg per day was given in divided dose in every morning and evening.The dosage ofolanzapine and methylphenidate were adjusted according to age, body mass and patient's clinical condi tions. And the period of treatment for either drug was 12 weeks. Conners teachers scale was used before and at the end of the 12 weektreatment (①hyperkinetic index consisted of 10 items②hyperkinetic behavior factor consisted of 7 items. Each item was rated “none=0, a little=1, quite a lot=2, and very much=3 ). Side effect scale was used to evaluate the hy perkinetic symptom and adverse reactions which consisted of behavior toxicity, abnormal laboratory tests , symptoms of the nervous system, au tonomic nervous system, cardiovascular system and skin etc with. 0=none; 1 =suspicious or very mild, 2=mild, 3 =moderate, 4=severe. The highest score was 22,and the lowest was 0 with a

  18. Auriculotemporal Syndrome (Frey Syndrome).

    Science.gov (United States)

    Motz, Kevin M; Kim, Young J

    2016-04-01

    Frey syndrome is a common sequela of parotidectomy, and although it is not frequently manifested clinically, it can cause significant morbidity for those affected. Frey syndrome results from synkinetic autonomic reinnervation by transected postganglionic parasympathetic nerve fiber within the parotid gland to the overlying sweat glands of the skin. Many surgical techniques have been proposed to prevent the development of Frey syndrome. For those who develop clinical symptoms of Frey syndrome, objective testing can be performed with a Minor starch-iodine test. Some of the current methods to prevent and treat symptomatic Frey syndrome are reviewed. PMID:26902982

  19. Improved final height in girls with Turner's syndrome treated with growth hormone and oxandrolone

    OpenAIRE

    Karlberg, J; Nilsson, KO; AlbertssonWikland, K; Alm, J.; Aronson, S.; Gustafsson, J; Hagenäs, L; Häger, A; Ivarsson, SA; Kriström, B; Marcus, C; Moell, C; Ritzén, M; Tuvemo, T; Wattsgård, C

    1996-01-01

    The spontaneous growth process in Turner's syndrome is characterized by a progressive decline in height velocity during childhood and no pubertal growth spurt. Therefore, therapy aimed at improving height during childhood as well as increasing final height is desirable for most girls with Turner's syndrome. Forty-five girls with Turner's syndrome, 9-16 yr of age (mean age, 12.2 yr), were allocated to three study groups. Group 1 (n = 13) was initially treated with oxandrolone alone; after 1 yr...

  20. Total Marrow and Lymphoid Irradiation and Chemotherapy Before Donor Transplant in Treating Patients With Myelodysplastic Syndrome or Acute Leukemia

    Science.gov (United States)

    2016-08-10

    Adult Acute Lymphoblastic Leukemia in Complete Remission; Acute Myeloid Leukemia in Remission; Previously Treated Myelodysplastic Syndrome; Recurrent Adult Acute Lymphoblastic Leukemia; Recurrent Adult Acute Myeloid Leukemia; Recurrent Childhood Acute Lymphoblastic Leukemia; Recurrent Childhood Acute Myeloid Leukemia; Childhood Acute Lymphoblastic Leukemia in Complete Remission

  1. The therapeutic effect of monocyte chemoattractant protein-1 delivered by an electrospun scaffold for hyperglycemia and nephrotic disorders

    Directory of Open Access Journals (Sweden)

    Yong C

    2014-02-01

    Full Text Available Cai Yong,2,* Zhengxin Wang,1,* Xing Zhang,3 Xiaomin Shi,1 Zhijia Ni,1 Hong Fu,1 Guoshan Ding,1 Zhiren Fu,1 Hao Yin1,3 1Department of Surgery, Organ Transplant Center, Shanghai Changzheng Hospital, Second Military Medical University, Shanghai, People's Republic of China; 2Department of Transplantation, First Affiliated Hospital of Wenzhou Medical College, Wenzhou, People's Republic of China; 3Department of Surgery, University of Chicago, Chicago, IL, USA *These authors contributed equally to this article Abstract: Here, we investigated in diabetic mice the therapeutic effect of monocyte chemoattractant protein-1 (MCP-1, locally delivered by an electrospun scaffold, on transplanted islets. This therapeutic scheme is expected to exert a synergistic effect to ameliorate hyperglycemia and its associated nephrotic disorders. The cumulative amount of MCP-1 released from the scaffold in vitro within a 3-week window was 267.77±32.18 ng, without a compromise in bioactivity. After 8 weeks following the transplantation, the islet population stimulated by MCP-1 was 35.14%±7.23% larger than the non-stimulated islet population. Moreover, MCP-1 increased concentrations of blood insulin and C-peptide 2 by 49.83%±5.29% and 43.49%±9.21%, respectively. Consequently, the blood glucose concentration in the MCP-1 group was significantly lower than that in the control group at week 2 post-surgery. MCP-1 also enhanced the tolerance of sudden oral glucose challenge. The rapid decrease of blood creatinine, urine creatinine, and blood urea nitrogen suggested that the recovery of renal functions compromised by hyperglycemia could also be attributed to MCP-1. Our study shed new light on a synergistic strategy to alleviate hyperglycemia and nephrotic disorders in diabetic patients. Keywords: MCP-1, electrospinning, islet transplantation, diabetes

  2. Childhood trauma and childhood urbanicity in relation to psychotic disorder

    OpenAIRE

    Frissen, Aleida; Lieverse, Ritsaert; Drukker, Marjan; van Winkel, Ruud; Delespaul, Philippe; [...

    2015-01-01

    Background Urban upbringing and childhood trauma are both associated with psychotic disorders. However, the association between childhood urbanicity and childhood trauma in psychosis is poorly understood. The urban environment could occasion a background of social adversity against which any effect of childhood trauma increases. Also, any impact of the urban environment on likelihood of exposure to childhood trauma could be stronger in children who later develop psychotic disorder. The aim of...

  3. Childhood trauma and childhood urbanicity in relation to psychotic disorder

    OpenAIRE

    Frissen, Aleida; Lieverse, Ritsaert; Drukker, Marjan; van Winkel, Ruud; Delespaul, Philippe; Cahn, W.

    2015-01-01

    BACKGROUND: Urban upbringing and childhood trauma are both associated with psychotic disorders. However, the association between childhood urbanicity and childhood trauma in psychosis is poorly understood. The urban environment could occasion a background of social adversity against which any effect of childhood trauma increases. Also, any impact of the urban environment on likelihood of exposure to childhood trauma could be stronger in children who later develop psychotic disorder. The aim o...

  4. Treosulfan, Fludarabine Phosphate, and Total-Body Irradiation Before Donor Stem Cell Transplant in Treating Patients With High-Risk Acute Myeloid Leukemia, Myelodysplastic Syndrome, Acute Lymphoblastic Leukemia

    Science.gov (United States)

    2013-10-29

    Accelerated Phase Chronic Myelogenous Leukemia; Adult Acute Lymphoblastic Leukemia in Remission; Adult Acute Myeloid Leukemia in Remission; Adult Acute Myeloid Leukemia With 11q23 (MLL) Abnormalities; Adult Acute Myeloid Leukemia With Del(5q); Adult Acute Myeloid Leukemia With Inv(16)(p13;q22); Adult Acute Myeloid Leukemia With t(15;17)(q22;q12); Adult Acute Myeloid Leukemia With t(16;16)(p13;q22); Adult Acute Myeloid Leukemia With t(8;21)(q22;q22); Blastic Phase Chronic Myelogenous Leukemia; Childhood Acute Lymphoblastic Leukemia in Remission; Childhood Acute Myeloid Leukemia in Remission; Childhood Chronic Myelogenous Leukemia; Childhood Myelodysplastic Syndromes; Chronic Myelomonocytic Leukemia; de Novo Myelodysplastic Syndromes; Previously Treated Myelodysplastic Syndromes; Recurrent Adult Acute Lymphoblastic Leukemia; Recurrent Adult Acute Myeloid Leukemia; Recurrent Childhood Acute Lymphoblastic Leukemia; Recurrent Childhood Acute Myeloid Leukemia; Secondary Myelodysplastic Syndromes; Untreated Adult Acute Lymphoblastic Leukemia; Untreated Childhood Acute Lymphoblastic Leukemia

  5. Noonan syndrome

    Directory of Open Access Journals (Sweden)

    van der Burgt Ineke

    2007-01-01

    Full Text Available Abstract Noonan Syndrome (NS is characterised by short stature, typical facial dysmorphology and congenital heart defects. The incidence of NS is estimated to be between 1:1000 and 1:2500 live births. The main facial features of NS are hypertelorism with down-slanting palpebral fissures, ptosis and low-set posteriorly rotated ears with a thickened helix. The cardiovascular defects most commonly associated with this condition are pulmonary stenosis and hypertrophic cardiomyopathy. Other associated features are webbed neck, chest deformity, mild intellectual deficit, cryptorchidism, poor feeding in infancy, bleeding tendency and lymphatic dysplasias. The syndrome is transmitted as an autosomal dominant trait. In approximately 50% of cases, the disease is caused by missense mutations in the PTPN11 gene on chromosome 12, resulting in a gain of function of the non-receptor protein tyrosine phosphatase SHP-2 protein. Recently, mutations in the KRAS gene have been identified in a small proportion of patients with NS. A DNA test for mutation analysis can be carried out on blood, chorionic villi and amniotic fluid samples. NS should be considered in all foetuses with polyhydramnion, pleural effusions, oedema and increased nuchal fluid with a normal karyotype. With special care and counselling, the majority of children with NS will grow up and function normally in the adult world. Management should address feeding problems in early childhood, evaluation of cardiac function and assessment of growth and motor development. Physiotherapy and/or speech therapy should be offered if indicated. A complete eye examination and hearing evaluation should be performed during the first few years of schooling. Preoperative coagulation studies are indicated. Signs and symptoms lessen with age and most adults with NS do not require special medical care.

  6. Reducing Childhood Obesity

    Science.gov (United States)

    ... Bar Home Current Issue Past Issues Reducing Childhood Obesity Past Issues / Summer 2007 Table of Contents For ... Ga. were the first three We Can! cities. Obesity Research: A New Approach The percentage of children ...

  7. Childhood vitiligo: Treatment paradigms

    Directory of Open Access Journals (Sweden)

    Amrinder Jit Kanwar

    2012-01-01

    Full Text Available Childhood vitiligo differs from the adults by showing a higher incidence in females, segmental vitiligo being more common and less frequent association with other systemic autoimmune and endocrine disorders.Childhood vitiligo is often associated with a marked psychosocial and long lasting effect on the self-esteem of the affected children and their parents, hence an adequate treatment is very essential. Treatment of vitiligo is indeed a tough challenge for the dermatologists′ more so in the background of childhood vitiligo. Although multiple therapeutic modalities are available in the therapeutic armamentarium, not all can be used in children. This brief report updates regarding various therapies available in the treatment of childhood vitiligo.

  8. Cardiovascular Conditions of Childhood

    Science.gov (United States)

    ... This childhood illness can result in long-term heart complications. Learn the symptoms, diagnosis and treatment for Kawasaki disease. Rheumatic Fever This inflammatory infection can occur after strep ...

  9. Childhood Immunization Schedule

    Science.gov (United States)

    ... Why Immunize? Vaccines: The Basics Instant Childhood Immunization Schedule Recommend on Facebook Tweet Share Compartir Get the ... See Disclaimer for additional details. Based on Immunization Schedule for Children 0 through 6 Years of age ...

  10. Early Childhood Caries

    OpenAIRE

    Kawashita, Yumiko; Kitamura, Masayasu; Saito, Toshiyuki

    2011-01-01

    Dental caries is one of the most common childhood diseases, and people continue to be susceptible to it throughout their lives. Although dental caries can be arrested and potentially even reversed in its early stages, it is often not self-limiting and progresses without proper care until the tooth is destroyed. Early childhood caries (ECC) is often complicated by inappropriate feeding practices and heavy infection with mutans streptococci. Such children should be targeted with a professional ...

  11. Stress and childhood epilepsy

    OpenAIRE

    Campen, J.S. van

    2015-01-01

    Epilepsy is one of the most common chronic diseases in childhood, characterized by the enduring predisposition to generate epileptic seizures. Children with epilepsy and their parents often report seizures precipitated by stress. In order to increase our understanding of the pathophysiological mechanisms underlying the effects of stress on seizures in childhood epilepsy, we performed a variety of studies, which are described in this thesis. In part I we evaluate the extent of stress sensitivi...

  12. Childhood Ovarian Malignancy

    OpenAIRE

    Mahadik, Kalpana; Ghorpade, Kanchanmala

    2014-01-01

    Objective of this article is to appraise diagnostic aspects and treatment modalities in childhood ovarian tumor in background of available evidence. Literature search on Pubmed revealed various aspects of epidemiology, histopathological diagnosis, and treatment of pediatric ovarian tumor. 85 % of childhood tumors are germ cell tumors. The varied histopathological picture in germ cell tumors poses a diagnostic and therapeutic challenge. Immunohistochemistry and newer genetic markers like SALL4...

  13. Early childhood aggression

    OpenAIRE

    Alink, Lenneke Rosalie Agnes

    2006-01-01

    In this thesis the development, stability, and correlates of early childhood aggression were investigated. The normative development was examined in a general population sample using questionnaires completed by the parents of 12-, 24-, and 36-month-old children and again one year later. Results showed an early childhood aggression curve, with increasing rates of aggression in the second year of life and decreasing rates in the fourth year. One-year stabilities were moderate for 12-month-olds ...

  14. Pesticides and childhood cancers.

    OpenAIRE

    Daniels, J L; Olshan, A.F.; Savitz, D A

    1997-01-01

    To evaluate the possible association between pesticides and the risk of childhood cancers, epidemiologic studies published between 1970 and 1996 were critically reviewed. Thirty-one studies investigated whether occupational or residential exposure to pesticides by either parents or children was related to increased risk of childhood cancer. In general, the reported relative risk estimates were modest. Risk estimates appeared to be stronger when pesticide exposure was measured in more detail. ...

  15. [Bilateral tibial compartment syndrome with crush symptoms after cannabis abuse].

    Science.gov (United States)

    Huber, F X; Eckstein, H H; Allenberg, J R

    1997-08-01

    Acute compartmental syndrome (CS) is a surgical emergency. Different conditions in which high non-physiological pressure appears within a closed fascial space reduce the necessary blood perfusion. CS is caused by trauma, burns, bleeding in patients with coagulopathies arterial injuries, nephrotic syndrome or unusual physical exercise with secondary compartmental swelling. When decompression occurs too late, permanent loss of function and limb contracture may result. In the following paper we report on a case of four-compartmental syndrome in the lower legs of a patient with drug intoxication. After cannabis consumption, the patient fell asleep sitting cross-legged. During our first examination several hours later, the signs of compartmental syndrome with spontaneous pain, turgid swelling and paresis were present. Bilateral skin incision technique was used to gain entrance into the four compartments in both lower legs. Immediately after the operation, the patient showed crush syndrome with high serum creatine kinase activity 140.501 U/l and acute renal failure caused by rhabdomyolysis. Within 2 weeks of haemofiltration and dialysis, a full recovery to a normal serum creatinine level of 0.7 mg/dl was achieved. After emergency treatment and rehabilitation, the patient showed neither vascular nor neural defects. PMID:9377997

  16. Leopard syndrome

    Directory of Open Access Journals (Sweden)

    Dallapiccola Bruno

    2008-05-01

    Full Text Available Abstract LEOPARD syndrome (LS, OMIM 151100 is a rare multiple congenital anomalies condition, mainly characterized by skin, facial and cardiac anomalies. LEOPARD is an acronym for the major features of this disorder, including multiple Lentigines, ECG conduction abnormalities, Ocular hypertelorism, Pulmonic stenosis, Abnormal genitalia, Retardation of growth, and sensorineural Deafness. About 200 patients have been reported worldwide but the real incidence of LS has not been assessed. Facial dysmorphism includes ocular hypertelorism, palpebral ptosis and low-set ears. Stature is usually below the 25th centile. Cardiac defects, in particular hypertrophic cardiomyopathy mostly involving the left ventricle, and ECG anomalies are common. The lentigines may be congenital, although more frequently manifest by the age of 4–5 years and increase throughout puberty. Additional common features are café-au-lait spots (CLS, chest anomalies, cryptorchidism, delayed puberty, hypotonia, mild developmental delay, sensorineural deafness and learning difficulties. In about 85% of the cases, a heterozygous missense mutation is detected in exons 7, 12 or 13 of the PTPN11 gene. Recently, missense mutations in the RAF1 gene have been found in two out of six PTPN11-negative LS patients. Mutation analysis can be carried out on blood, chorionic villi and amniotic fluid samples. LS is largely overlapping Noonan syndrome and, during childhood, Neurofibromatosis type 1-Noonan syndrome. Diagnostic clues of LS are multiple lentigines and CLS, hypertrophic cardiomyopathy and deafness. Mutation-based differential diagnosis in patients with borderline clinical manifestations is warranted. LS is an autosomal dominant condition, with full penetrance and variable expressivity. If one parent is affected, a 50% recurrence risk is appropriate. LS should be suspected in foetuses with severe cardiac hypertrophy and prenatal DNA test may be performed. Clinical management should

  17. Childhood obesity: pathophysiology and treatment.

    Science.gov (United States)

    Klish, W J

    1995-02-01

    Childhood obesity is among the most difficult problems which pediatricians treat. It is frequently ignored by the pediatrician or viewed as a form of social deviancy, and blame for treatment failure placed on the patients or their families. The definition of obesity is difficult. Using total body electrical conductivity (TOBEC) technology, total body fat ranges between 12% and 30% of total body weight in normal children and adolescents. This is influenced not only by age, but also by physical fitness. Anthropometry is the easiest way to define obesity. Children whose weight exceeds 120% of that expected for their height are considered overweight. Skinfold thickness and body mass index are indices of obesity that are more difficult to apply to the child. Childhood obesity is associated with obese parents, a higher socioeconomic status, increased parental education, small family size and a sedentary lifestyle. Genetics also clearly plays a role. Studies have demonstrated that obese and non-obese individuals have similar energy intakes implying that obesity results from very small imbalances of energy intake and expenditure. An excess intake of only 418 kJ per day can result in about 4.5 kg of excess weight gain per year. Small differences in basal metabolic rate or the thermic effects of food may also account for the difference in energy balance between the obese and non-obese. In the Prader Willi Syndrome, there appears to be a link between appetite and body fatness. When placed on growth hormone, lean body mass increases, body fat decreases, sometimes to normal, and appetite becomes more normal.(ABSTRACT TRUNCATED AT 250 WORDS)

  18. Childhood Adrenocortical Tumours: a Review

    Directory of Open Access Journals (Sweden)

    Marques-Pereira Rosana

    2006-05-01

    Full Text Available Abstract Childhood adrenocortical tumour (ACT is not a common disease, but in southern Brazil the prevalence is 15 times higher than in other parts of the world. One hundred and thirty-seven patients have been identified and followed by our group over the past four decades. Affected children are predominantly girls, with a female-to-male ratio of 3.5:1 in patients below 4 years of age. Virilization alone (51.6% or mixed with Cushing's syndrome (42.0% was the predominant clinical picture observed in these patients. Tumours are unilateral, affecting both glands equally. TP53 R337H germline mutations underlie most childhood ACTs in southern Brazil. Epidemiological data from our casuistic studies revealed that this mutation has ~10% penetrance for ACT. Surgery is the definitive treatment, and a complete resection should always be attempted. Although adjuvant chemotherapy has shown some encouraging results, its influence on overall outcome is small. The survival rate is directly correlated to tumour size; patients with small, completely excised tumours have survival rates close to 90%, whereas in those patients with inoperable tumours and/or metastatic disease it is less than 10%. In the group of patients with large, excisable tumours, half of them have an intermediate outcome. Recent molecular biology techniques and genomic approaches may help us to better understand the pathogenesis of ACT, the risk of developing a tumour when TP53 R337H is present, and to predict its outcome. An ongoing pilot study consisting of close monitoring of healthy carriers of the TP53 R337H mutation - siblings and first-degree relatives of known affected cases - aims at the early detection of ACTs and an improvement of the cure rate.

  19. Rett and ICF syndromes: methylation moves into medicine

    OpenAIRE

    Kumar, Arun

    2000-01-01

    Two human genetic disorders, Rett and ICF syndromes, have recently been shown to be caused by mutations in genes encoding proteins involved in gene silencing through DNA methylation. Rett (RTT) syndrome is a progressive childhood neurodevelopmental disorder that affects females exclusively with an incidence of 1 in 10,000–15,000 female births.

  20. Kindler′s syndrome: A rare case report

    Directory of Open Access Journals (Sweden)

    Neelam Suman

    2014-01-01

    Full Text Available Kindler syndrome is a rare hereditary disorder, associated with skin fragility. 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 a 16-year-old patient with classical features like blistering and photosensitivity in childhood and the subsequent development of poikiloderma.