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Sample records for change nephrotic syndrome

  1. Nephrotic Syndrome

    Science.gov (United States)

    ... use of certain legal and illegal drugs, or morbid obesity can lead to nephrotic syndrome. Symptoms Some kids ... KidsHealth® is for educational purposes only. For specific medical advice, diagnoses, and treatment, consult your doctor. © 1995- The Nemours Foundation. All ...

  2. Complications of nephrotic syndrome

    OpenAIRE

    Se Jin Park; Jae Il Shin

    2011-01-01

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

  3. 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.

  4. Behavioral Changes in Egyptian Children With Nephrotic Syndrome

    OpenAIRE

    Emad Emil Ghobrial; Sameh Samir Fahmey; Maha Emad Eldin Ahmed; Osama Botrous

    2013-01-01

    Introduction. Chronic illnesses, including nephrotic syndrome (NS), are associated with psychosocial stress. Our study aimed to assess psychological problems in children with NS. Materials and Methods. Sixty children with NS were assessed at the Children Hospital, in Cairo for behavioral changes. They responded to the Arabic version of the Strength and Difficulties Questionnaire. The results were compared between those with steroid-sensitive NS (SSNS), steroid-dependent NS (SDNS), and steroid...

  5. Complications of nephrotic syndrome

    Directory of Open Access Journals (Sweden)

    Se Jin Park

    2011-08-01

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

  6. Complications of nephrotic syndrome.

    Science.gov (United States)

    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

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

    OpenAIRE

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

    2009-01-01

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

  8. Nephrotic Syndrome in Adults

    Science.gov (United States)

    ... glomeruli. Nephrotic syndrome can also be caused by systemic diseases, which are diseases that affect many parts of the body, such as diabetes or lupus. Systemic diseases that affect the kidneys are called secondary causes ...

  9. Congenital nephrotic syndrome

    Science.gov (United States)

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

  10. [Nephrotic syndrome associated with ampulloma].

    Science.gov (United States)

    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

  11. Congenital nephrotic syndrome

    Directory of Open Access Journals (Sweden)

    Claudia Fanni

    2014-06-01

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

  12. Radiation nephritis causing nephrotic syndrome

    Energy Technology Data Exchange (ETDEWEB)

    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].

    Science.gov (United States)

    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. 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.

  15. The Genetics of Nephrotic Syndrome.

    Science.gov (United States)

    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

  16. Nephrotic syndrome and hepatitis in early syphilis.

    OpenAIRE

    Tang, A. L.; Thin, R N; Croft, D N

    1989-01-01

    A 54 year old man presented with features of acute hepatitis and the nephrotic syndrome. A diagnosis of active syphilis was only made by chance after extensive investigation. Syphilis should be considered in the differential diagnosis of both acute hepatitis and the nephrotic syndrome occurring separately as well as together.

  17. Steroid-responsive and nephrotic syndrome and allergy: clinical studies.

    OpenAIRE

    Meadow, S R; Sarsfield, J K

    1981-01-01

    Eighty-four children with steroid-responsive nephrotic syndrome who had been shown to have, or were believed to have, minimal change histology were investigated to study the relationship between steroid-responsive nephrotic syndrome and allergy. They were found to have a greater incidence of the standard atopic disorders--asthma, eczema, recurrent urticaria, and hay fever. Their 1st-degree relatives had an increased incidence of these atopic disorders too. A nasal discharge was a frequent pre...

  18. [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

  19. Congenital nephrotic syndrome.

    Science.gov (United States)

    Begolli, Mirije; Begolli, Ilir; Gojani, Xhenane; Arenliu-Qosaj, Fatime; Berisha, Merita

    2011-01-01

    The aim of this case is to present a case of a two month old female with congenital nephritic syndrome, which is very rare. On admission, the baby showed marked edema and distended abdomen. She was diagnosed and treated with daily albumin infusions, antibiotics, diuretic, gamma globulin replacement, ACEI and NSAIDs. Parents were informed about the nature of the disease, prognosis, and advised for further medical care in a more advanced kidney transplantation centre. This was the first treatment of this condition in the Pediatric Clinic in Kosovo and it presented a challenge for us. PMID:22299306

  20. Steroid-responsive and nephrotic syndrome and allergy: clinical studies.

    Science.gov (United States)

    Meadow, S R; Sarsfield, J K

    1981-07-01

    Eighty-four children with steroid-responsive nephrotic syndrome who had been shown to have, or were believed to have, minimal change histology were investigated to study the relationship between steroid-responsive nephrotic syndrome and allergy. They were found to have a greater incidence of the standard atopic disorders--asthma, eczema, recurrent urticaria, and hay fever. Their 1st-degree relatives had an increased incidence of these atopic disorders too. A nasal discharge was a frequent precursor or an accompaniment of nephrotic syndrome, but an overt atrophic disorder at the same time was rare. Such disorders, related to relapse, occurred in only 5 children; in none was it a consistent or recurrent happening at the time of each relapse. No example of pollen hypersensitivity nephrotic syndrome was found, and no particular allergen could be identified with certainty as responsible for a child's nephrotic syndrome. No association was found between the time of relapse and the season of the year, or the season in which the child was born. Children with nephrotic syndrome had a greater incidence of positive skin tests to common antigens, the comparative frequency of positive reactions to different antigens being similar to that found in children with asthma, although the total frequency was about half that of children with asthma. Despite the increased incidence of clinical features of atopy, measures to reduce the frequency of relapse of nephrotic syndrome by allergen avoidance, the use of sodium cromoglycate, and the use of a new oral antiallergic drug were unsuccessful. PMID:6791592

  1. 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)

  2. Congenital nephrotic syndrome. Gallium-67 imaging

    Energy Technology Data Exchange (ETDEWEB)

    Trepashko, D.W.; Gelfand, M.J.; Pan, C.C.

    1988-11-01

    Congenital nephrotic syndrome is a rare disorder. Heavy proteinuria, hypoalbuminemia, and edema occur during the first 3 months of life. Initial cases were reported from Finland and sporadic cases have occurred elsewhere. Finnish cases demonstrated an autosomal recessive inheritance pattern; currently, Finnish and non-Finnish types are recognized. The clinical course consists of failure to thrive, frequent infections, declining renal function, and early death by age 4 years from sepsis or uremia. Recently renal transplantation has improved the prognosis of patients with this disease. An abnormal Ga-67 scan in a case of congenital nephrotic syndrome is presented.

  3. Nephrotic syndrome-induced thromboembolism in adults

    Science.gov (United States)

    Al-Azzawi, Hasan F.; Obi, Onyekachi C.; Safi, Javeryah; Song, Mingchen

    2016-01-01

    Nephrotic syndrome (NS) is a well-defined syndrome characterized by the presence of nephrotic range of proteinuria, hypoalbuminemia, and hyperlipidemia. Although venous thromboembolism (VTE) is a well-reported complication associated with NS, the incidence, prevalence, risk factors, treatment options, and preventative strategies are not well-established. Thromboembolic phenomena in nephrotic patients are postulated to be a result of the urinary loss of antithrombotic factors by affected kidneys and increased production of prothrombotic factors by the liver. Most cases of VTE associated with NS reported in the literature have a known diagnosis of NS. We report a case of a young female presenting with dyspnea and a pulmonary embolism. She was found to have NS and right renal vein thrombosis. We review the available literature to highlight the best approach for clinicians treating VTE in patients with NS.

  4. 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...

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

    Directory of Open Access Journals (Sweden)

    Yonghui Xia

    2013-05-01

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

  6. Rheumatoid disease presenting as a nephrotic syndrome.

    OpenAIRE

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

    1988-01-01

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

  7. Two cases of nephrotic syndrome with different etiologies

    Directory of Open Access Journals (Sweden)

    Sanjay K Mandal

    2013-01-01

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

  8. Role of reactive oxygen species in pathogenesis of nephrotic syndrome

    OpenAIRE

    Ghodake, Santoshi R.; Suryakar, A. N.; Ankush, R. D.; Shaikh, K.; Katta, A. V.

    2010-01-01

    Nephrotic syndrome is the common chronic disorder characterized by alteration of permeability of the glomerular capillary wall, resulting in its inability to restrict the urinary loss of proteins. Nephrotic syndrome is characterized by heavy proteinuria, hypoalbuminemia, hyperlipidemia associated with peripheral edema. The molecular basis of glomerular permselectivity remains largely unknown. In recent years it has been proposed that Nephrotic syndrome is a consequence of an imbalance between...

  9. Renal artery embolization in severe nephrotic syndrome.

    Science.gov (United States)

    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

  10. [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

  11. Management of idiopathic nephrotic syndrome in childhood

    Directory of Open Access Journals (Sweden)

    Peco-Antić Amira

    2004-01-01

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

  12. Histopathological types in adult nephrotic syndrome

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    Md. Ghulam Yusuf

    2016-01-01

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

  13. Treatment of anemia of nephrotic syndrome with recombinant erythropoietin

    NARCIS (Netherlands)

    Gansevoort, RT; Vaziri, ND; deJong, PE

    1996-01-01

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

  14. Disorders of lipid metabolism in nephrotic syndrome: mechanisms and consequences.

    Science.gov (United States)

    Vaziri, Nosratola D

    2016-07-01

    Nephrotic syndrome results in hyperlipidemia and profound alterations in lipid and lipoprotein metabolism. Serum cholesterol, triglycerides, apolipoprotein B (apoB)-containing lipoproteins (very low-density lipoprotein [VLDL], immediate-density lipoprotein [IDL], and low-density lipoprotein [LDL]), lipoprotein(a) (Lp[a]), and the total cholesterol/high-density lipoprotein (HDL) cholesterol ratio are increased in nephrotic syndrome. This is accompanied by significant changes in the composition of various lipoproteins including their cholesterol-to-triglyceride, free cholesterol-to-cholesterol ester, and phospholipid-to-protein ratios. These abnormalities are mediated by changes in the expression and activities of the key proteins involved in the biosynthesis, transport, remodeling, and catabolism of lipids and lipoproteins including apoproteins A, B, C, and E; 3-hydroxy-3-methylglutaryl-coenzyme A reductase; fatty acid synthase; LDL receptor; lecithin cholesteryl ester acyltransferase; acyl coenzyme A cholesterol acyltransferase; HDL docking receptor (scavenger receptor class B, type 1 [SR-B1]); HDL endocytic receptor; lipoprotein lipase; and hepatic lipase, among others. The disorders of lipid and lipoprotein metabolism in nephrotic syndrome contribute to the development and progression of cardiovascular and kidney disease. In addition, by limiting delivery of lipid fuel to the muscles for generation of energy and to the adipose tissues for storage of energy, changes in lipid metabolism contribute to the reduction of body mass and impaired exercise capacity. This article provides an overview of the mechanisms, consequences, and treatment of lipid disorders in nephrotic syndrome. PMID:27165836

  15. Histopathological diagnosis and outcome of paediatric nephrotic syndrome

    International Nuclear Information System (INIS)

    Objective: To determine the histological picture and outcome of treatment in cases of childhood nephrotic syndrome who needed renal biopsy. Subjects and Methods: Children suffering from nephrotic syndrome who had atypical features at presentation were initially or late non-responders; frequent relapsers on > 1 mg kg/day and were steroid dependent or frequently relapsed on < 1 mg kg/day but developed steroid toxicity were included. Renal biopsy was performed in these patients. Treatment was administered according to the histopathology reports. Prednisolone 60 mg /m/sup 2//day followed after response by 40 mg /m/sup /2 on alternate days (AD) which was later tapered off. In minimal change nephrotic syndrome (MCNS) with frequent relapses cyclophosphamide, cyclosporine and levimisole were used. For steroid resistant focal segmental glomerulosclerosis (FSGS) intravenous pulses of methylprednisolone and cyclosporine were also given. These patients were followed to see the response of the therapy. Results: The commonest diagnosis was focal segmental glomerulosclerosis (FSGS) (42%) followed by minimal change disease (MCNS) (22%), membranoproliferative or mesangiocapillary glomerulonephritis (MPGN) (14%) and Mesangioproliferative glomerulonephritis (Mes PGN) (12%). There were 6% cases of membranous nephropathy and 4% of diffuse proliferative glomerulonephritis. On presentation, 40% had hematuria, 20% were found to be hypertensive, 12% patients had renal insufficiency and in 40% C3 level was low. Majority of the patients with MPGN and FSGS had atypical features whereas none of the patients with membranous nephropathy had any of these features. Thirty percent cases each of FSGS and MCNS were responders. Among non-responders there were 4 cases of FSGS and one of MPGN. Conclusion: FSGS was the commonest histology in cases of childhood nephrotic syndrome that needed renal biopsy. Highest frequency of atypical features was seen in MPGN and FSGS. (author)

  16. A whitened face woman with nephrotic syndrome.

    Science.gov (United States)

    Soo, Yannie Oi-Yan; Chow, Kai-Ming; Lam, Christopher Wai-Kei; Lai, Fernand Mac-Moune; Szeto, Cheuk-Chun; Chan, Michael Ho-Ming; Li, Philip Kam-Tao

    2003-01-01

    Skin whitening cream from developing countries is a recognized source of chronic mercury poisoning. The authors report on a 34-year-old Indonesian domestic helper who presented with nephrotic syndrome secondary to membranous nephropathy. It was subsequently found that she used a skin whitening cream regularly that was found to contain a mercury level of almost 2,000 times above the allowable limit. Her blood and urinary mercury levels were both grossly elevated. Her symptoms improved after she stopped using the cream. However, she returned to her home country before chelating therapy could be arranged. Because mercury-containing skin products are still widely available in developing countries, the use of these products should be considered a possible cause of membranous nephropathy in immigrants from those countries. PMID:12500245

  17. 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...

  18. A histopathological outlook on nephrotic syndrome: A pediatric perspective.

    Science.gov (United States)

    Arif, M K; Arif, M; Amjad, N

    2016-01-01

    The developing world is observing changing histopathological patterns of idiopathic nephrotic syndrome (INS). However, the true burden of non-minimal change disease (non-MCD) presenting as INS remains unestimated owing to a paucity of data on renal biopsies. Data were collected from January 2006 to June 2014 on 75 children up to 16 years of age who underwent renal biopsies for INS. Mean age at biopsy was 11.2 ± 3.7 years. The male to female ratio was 1.5:1. A total of 25 (33.3%) children were steroid sensitive, 36 (48%) were steroid resistant, 10 (13.3%) were steroid dependent and 4 (5.3%) came with relapse of nephrotic syndrome (NS). Focal segmental glomerulosclerosis (FSGS) was the most common histopathological subtype observed in 35 (46.8%) children followed by membranous glomerulonephritis (MGN) in 11 (14.7%), membranoproliferative glomerulonephritis (MPGN) and mesangioproliferative glomerulonephritis (MSGN) in 4 (5.3%) each and IgA nephropathy in one (1.3%). MCD was the histological lesion in 19 (25.3%) children. The histopathology established FSGS as the main underlying cause of steroid resistant NS. The study highlights the emergence of non-MCD as the common cause of INS in the pediatric population and signifies the importance of renal biopsies in children with INS. PMID:27194833

  19. 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.

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

    Directory of Open Access Journals (Sweden)

    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.

  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. PMID:20061709

  2. Splenic hypofunction in the nephrotic syndrome of childhood

    Energy Technology Data Exchange (ETDEWEB)

    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.

  3. Long-term Outcomes of Childhood Onset Nephrotic Syndrome

    Science.gov (United States)

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

    2016-01-01

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

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

    Directory of Open Access Journals (Sweden)

    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.

  5. Design of the Nephrotic Syndrome Study Network (NEPTUNE) to evaluate primary glomerular nephropathy by a multi-disciplinary approach

    OpenAIRE

    Gadegbeku, Crystal A.; Gipson, Debbie S.; Holzman, Larry; Ojo, Akinlolu O.; Song, Peter; Barisoni, Laura; Sampson, Matthew G.; Kopp, Jeffrey; Lemley, Kevin V.; Nelson, Peter; Lienczewski, Chrysta; Adler, Sharon; Appel, Gerald; Cattran, Daniel; Choi, Michael

    2013-01-01

    The Nephrotic Syndrome Study Network (NEPTUNE) is a North American multi-center collaborative consortium established to develop a translational research infrastructure for Nephrotic Syndrome. This includes a longitudinal observational cohort study, a pilot and ancillary studies program, a training program, and a patient contact registry. NEPTUNE will enroll 450 adults and children with minimal change disease, focal segmental glomerulosclerosis and membranous nephropathy for detailed clinical,...

  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. Profound nephrotic syndrome in a patient with ovarian teratoma

    Directory of Open Access Journals (Sweden)

    Abdallah Jeroudi

    2013-01-01

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

  8. 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...

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

    NARCIS (Netherlands)

    N. Teeninga (Nynke)

    2013-01-01

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

  10. Nephrotic Sydrome Developing in Severe Ovarian Hyperstimulation Syndrome

    Directory of Open Access Journals (Sweden)

    Funda Gungor Ugurlucan

    2014-01-01

    Full Text Available We report a case that developed nephrotic syndrome during hospitalization for severe ovarian hyperstimulation syndrome without history of acute renal failure. During hospitalization, she developed persistent ascites and respiratory distress. The 24 hours urine protein analysis revealed significant proteinuria and renal biopsy showed global and segmental sclerosis in glomeruli, mesangial arteritis, proliferations in visceral epithelial cells (IgA nephropathy. To the best of our knowledge, such a complication will be presented for the first time in the literature.

  11. 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...

  12. 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.

  13. 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 ...

  14. Nephrotic syndrome: a rare cause of acute coronary syndrome in a child

    International Nuclear Information System (INIS)

    Patients with nephrotic syndrome are at risk of developing thrombosis in both veins and arteries. Various manifestations in different organs have been reported. Thrombi in heart seen, associated with multi organ thrombosis have been reported on autopsy earlier, but only once in a living patient with nephrotic syndrome. Here, we report a 13 years old boy with steroid-resistant nephrotic syndrome, who developed an asymptomatic but potentially hazardous large intracardiac thrombus. The child developed nephrotic syndrome at the age of 9 years and had multiple recurrences. At the age of 13 years, he developed myocardial infarction (MI) due to embolism from a large intracardiac thrombus. Later on, he was treated with heparin and warfarin anticoagulation. (author)

  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. 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

  18. Prediction of Risk Factors of Frequent Relapse Idiopathic Nephrotic Syndrome.

    Science.gov (United States)

    Jahan, I; Hanif, M; Ali, M A; Hoque, M M

    2015-10-01

    This case control study was aimed to identify the predictive risk factors for frequent relapse idiopathic nephrotic syndrome (INS) and conducted in Sir Salimullah Medical College & Mitford Hospital, Dhaka and at Renal and Dialysis Unit of Dhaka Shishu Hospital and Bangladesh Institute of Child Health (BICH), Dhaka, from January 2006 to December 2006. We examined retrospectively the clinical course of fifty cases of frequent relapse nephrotic syndrome (FRNS) as cases and fifty cases of infrequent relapse nephrotic syndrome (IRNS) as control who met the predefined enrollment criteria, followed for at least one year after initial onset of disease. After enrollment following parameters were studied as predictors of frequent relapse: i) Socio-demographic variables: age, sex, socio-economic condition, number of living room ii) Disease related variables i.e. age of onset, duration of illness, frequency of relapse within the 1st year, regimen of initial steroid therapy, total cumulative dose of steroid for remission, day of remission after starting steroid, association with atopy and infection, concomitant upper respiratory illness iii) Biochemical and pathological variables (at the time of initial attack) i.e. Serum albumin, serum cholesterol, blood urea, 24 hours urinary protein, serum creatinine, complete blood count, urine RBC, urine pus cell, urine culture. The test statistics used to analyses the data were descriptive statistics, Chi-square probability test, Student's t-test and Binary logistic regression analysis for Odds ratio. Both univariate and multivariate logistic regression analysis revealed that age of onset (P<0.001, OR=0.9, 95% CI=0.85-0.95), poor socioeconomic status (P<0.034, OR=0.5.8, 95% CI=1.14-29.5) and low serum albumin level at the time of initial presentation (P<0.022, OR=0.8, 95% CI=0.65-0.97) were independent predictors of frequent relapse nephrotic syndrome. In conclusion, we demonstrated that age at onset, poor socioeconomic condition and low

  19. Histopathological patterns in paediatric idiopathic steroid resistant nephrotic syndrome

    International Nuclear Information System (INIS)

    Background: Steroid-resistant nephrotic syndrome (SRNS) is a common problem but difficult to treat for pediatric nephrologists. Due to paucity of studies done in few centres in southern Pakistan regarding the histopathological aspects in paediatric patients with SRNS, this study was conducted to determine the histopathological spectrum in children with SRNS at our centre. Method: This descriptive study has been conducted at the Nephrology department, The Children's Hospital Lahore from February 2014 to January 2015. Based upon history, physical examination and laboratory results, all patients diagnosed as idiopathic SRNS were included in the study and renal biopsy was done to determine the underlying pathology. Histopathology reports were retrieved and data analysis done using SPSS-20.0. Results: There were a total of 96 patients, 64 (66.7 percentage) males and 32 (33.3 percentage) females. The age range was from 0.80 to 15 years with mean age of presentation being 6.34+3.75 years. The most common histo-pathological pattern was mesangio-proliferative Glomerulonephritis found in 79 (82.3 percentage) cases followed by Focal segmental glomerulosclerosis (FSGS) in 9 (9.4 percentage) patients while Minimal change disease (MCD) was seen in 5 (5.2 percentage) subjects. Conclusion: Mesangioproliferative glomerulonephritis is the most common histological pattern seen in children presenting with idiopathic SRNS at our centre followed by FSGS and MCD. (author)

  20. 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.

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

    Science.gov (United States)

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

    2015-11-01

    Meningitis and associated intracranial bleeding have been rarely reported in patients with steroid-resistant nephrotic syndrome. We present such a case with raised intracranial tension in a 13-year-old child and discuss the management issues. Prompt recognition and appropriate treatment of these complications can be life saving in a child with nephrotic syndrome. PMID:26586071

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

    OpenAIRE

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

    2015-01-01

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

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

    Science.gov (United States)

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

    2016-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Pallavi Periwal

    2016-01-01

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

  5. 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.

  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

    Directory of Open Access Journals (Sweden)

    Abhijeet ePal

    2016-05-01

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

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

    Directory of Open Access Journals (Sweden)

    Dr. Anil Kumar*

    2013-12-01

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

  12. HDL abnormalities in nephrotic syndrome and chronic kidney disease.

    Science.gov (United States)

    Vaziri, Nosratola D

    2016-01-01

    Normal HDL activity confers cardiovascular and overall protection by mediating reverse cholesterol transport and through its potent anti-inflammatory, antioxidant, and antithrombotic functions. Serum lipid profile, as well as various aspects of HDL metabolism, structure, and function can be profoundly altered in patients with nephrotic range proteinuria or chronic kidney disease (CKD). These abnormalities can, in turn, contribute to the progression of cardiovascular complications and various other comorbidities, such as foam cell formation, atherosclerosis, and/or glomerulosclerosis, in affected patients. The presence and severity of proteinuria and renal insufficiency, as well as dietary and drug regimens, pre-existing genetic disorders of lipid metabolism, and renal replacement therapies (including haemodialysis, peritoneal dialysis, and renal transplantation) determine the natural history of lipid disorders in patients with kidney disease. Despite the adverse effects associated with dysregulated reverse cholesterol transport and advances in our understanding of the underlying mechanisms, safe and effective therapeutic interventions are currently lacking. This Review provides an overview of HDL metabolism under normal conditions, and discusses the features, mechanisms, and consequences of HDL abnormalities in patients with nephrotic syndrome or advanced CKD. PMID:26568191

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

    Directory of Open Access Journals (Sweden)

    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.

  14. Utility of gallium imaging of the kidneys in diagnosing primary amyloid nephrotic syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Gertz, M.A.; Brown, M.L.; Hauser, M.F.; Kyle, R.A. (Mayo Clinic, Rochester, MN (USA))

    1990-03-01

    We undertook a study to determine the value of gallium imaging of the kidneys in patients who had primary amyloidosis that was manifest clinically by nephrotic syndrome. We studied 28 patients with gallium-67 (67Ga) citrate scans performed 48 hr after injection. Intense (3+ to 4+) uptake was noted in both kidneys in 25 of 28 patients. Renal amyloidosis should be considered in the differential diagnosis when diffuse bilateral renal uptake of (67Ga)citrate is seen in the setting of nephrotic syndrome. Gallium uptake did not differentiate amyloid nephrotic syndrome from other causes of nephrotic syndrome. Renal gallium uptake showed a weak correlation with 24-hr urine protein excretion (p = 0.06).

  15. 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.

  16. Assessment of Behavior Abnormalities of Corticosteroids in Children with Nephrotic Syndrome

    OpenAIRE

    Usama Mahmoud Youssef; Mohamed Mohamed Abdelsalam; Ali Mohamed Abozeid; Doaa Mohammed Youssef

    2013-01-01

    Introduction. The objective of this work was to define the frequency and severity of steroid related behavioral side effects in children with steroid sensitive idiopathic nephrotic syndrome (SSNS) during Treatment for relapse. Methods. 30 pediatric patients with steroid sensitive nephrotic syndrome were studied; known as SSNS at complete remission or low dose of Prednisolone and have relapse on follow up. All children in this study were subjected to full history taking, thorough clinical exam...

  17. OCCURRENCE OF URINARY TRACT INFECTION IN CHILDREN WITH NEPHROTIC SYNDROME IN A TERTIARY CARE HOSPITAL

    OpenAIRE

    Vishwanath; Jaidev Mangalore; Mario Joseph

    2015-01-01

    BACKGROUND Nephrotic Syndrome (NS) is among the most common chronic kidney diseases occurring in childhood. Defense mechanisms are impaired in patients with NS. Immuno-compromised status of NS can result in increased susceptibility to various infections, which could be potentially serious and life-threatening without prompt diagnosis and treatment. The occurrence of Urinary Tract Infection (UTI) is about 30% in children with nephrotic syndrome; however, it often goes undiagnos...

  18. 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...

  19. Candida spp. and gingivitis in children with nephrotic syndrome or type 1 diabetes

    OpenAIRE

    Olczak-Kowalczyk, Dorota; Pyrżak, Beata; Dąbkowska, Maria; Pańczyk-Tomaszewska, Małgorzata; Miszkurka, Grażyna; Rogozińska, Izabela; Swoboda-Kopeć, Ewa; Gozdowski, Dariusz; Kalińska, Angelika; Piróg, Anna; Mizerska-Wasiak, Małgorzata; Roszkowska-Blaim, Maria

    2015-01-01

    Background Diabetes and Nephrotic syndrome (NS) promote plaque-related gingivitis and yeast-like fungal infections. The study assesses the impact of Candida spp. and general disease- or treatment-related factors on plaque-related gingivitis severity in children and adolescents with Nephrotic syndrome /diabetes. Methods Body mass index (BMI), BMI standard deviation score, and oral cavity (Plaque Index – PLI, Gingival Index – GI, mucosa status, presence and Candida enzymatic activity) were asse...

  20. Superior sagittal sinus thrombosis: a rare complication in a child with nephrotic syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Pirogovsky, A.; Adi, M.; Barzilai, N. [Dept. of Radiology, Kaplan Medical Center, Rehovot (Israel); Dagan, A.; Sinai, L.; Sthoeger, D. [Div. of Paediatrics, Kaplan Medical Center, Rehovot (Israel); Tabachnik, E. [Div. of Paediatrics, Kaplan Medical Center, Rehovot (Israel); Paediatric ICU, Kaplan Hospital, Rehovot (Israel)

    2001-10-01

    A 2-year-old boy with new-onset nephrotic syndrome developed recurrent vomiting, apathy and papilloedema. Superior sagittal sinus thrombosis was diagnosed on cranial CT and MRI. He gradually recovered after treatment with heparin, fresh frozen plasma and warfarin with complete resolution of the thrombosis after 1 month. Superior sagittal sinus thrombosis is an extremely rare complication of nephrotic syndrome in children. Early diagnosis is essential for institution of anticoagulation therapy and a successful outcome. (orig.)

  1. 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.

  2. 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

  3. 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 .

  4. 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.; Svenningsen, Per; Friis, Ulla Glenert; Jespersen, Bente; Rittig, Søren

    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......-503] μg/mmol in nephrotic urine versus 9.5 (95 % CI 8-12) μg/mmol at remission (p ...

  5. Clinical significance of measurement of plasma leptin and serum IL-6, IL-18 levels after treatment in patients with children nephrotic syndrome

    International Nuclear Information System (INIS)

    Objective: To explore the clinical significance of changes of plasma leptin and serum IL-6, IL-18 levels after treatment in patients with children nephrotic syndrome. Methods: Plasma leptin (with RIA) serum IL-6, IL-18 (with ELISA) levels were measured in 31 patients with children nephrotic syndrome both before and after treatment as well as in 30 controls. Results: Before treatment,the plasma leptin and serum IL-6, IL-18 levels were significantly higher than those in controls(P <0.01). After treatment for 3 months, the levels in patients though dropped markedly remained significantly higher than those in controls (P<0.05). Plasma leptin levels were positively correlated with IL-6, IL-18 levels (r=0.6138, 0.5784, P<0.01). Conclusion: Changes of plasma leptin and serum IL-6, IL-18 levels after treatment might be of prognostic importance in patients with children nephrotic syndrome. (authors)

  6. 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

  7. 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.

  8. 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

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

    Science.gov (United States)

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

    2016-09-01

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

  10. Nephrotic syndrome with a nephritic component associated with toxoplasmosis in an immunocompetent young man.

    Directory of Open Access Journals (Sweden)

    Tania González Velásquez

    2012-09-01

    Full Text Available Normal 0 21 false false false ES-CO X-NONE X-NONE  Introduction: Although the association of infection by toxoplasmosis with the development of nephrotic syndrome is uncommon, cases of this association have nevertheless been reported in the literature for more than two decades, not only for congenital toxoplasmosis, but also in acquired cases, and occasionally in immunocompetent patients. Development: A case is presented of an immunocompetent patient aged 15 with clinical and laboratory indications of nephrotic/nephritic syndrome, in whom serological tests showed toxoplasma infection. Conclusion: The presentation of nephrotic syndrome in ages where it is not commonly seen, leads to clinical suspicion of secondary causes. Active search for possible causes should include common tropical infections. 

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

    Science.gov (United States)

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

    2016-05-01

    Neuromyelitis optica (NMO) associated with nephrotic syndrome and autoimmune-related pancytopenia has not been reported previously. We report herein a young woman who initially presented with bilateral blurring of vision and numbness in her hands. MRI disclosed multiple white matter lesions and a long cervical spinal cord lesion extending to the medulla oblongata. Serum aquaporin-4 antibody was positive and the patient was diagnosed with NMO. While in the hospital, she presented with hypoproteinemia and heavy proteinuria, meeting the diagnostic criteria of nephrotic syndrome. After high-dose methylprednisolone treatment, her vision improved significantly and urine protein quantity decreased. However, the patient subsequently developed severe pancytopenia with a positive Coombs' test. Thrombocytopenia finally led to uncontrollable gastrointestinal bleeding as the direct cause of the patient's death. This case illustrates the extremely rare condition of concurrence of NMO, nephrotic syndrome, and autoimmune pancytopenia in one patient, which suggests the involvement of organs beyond the central nervous system in NMO spectrum disorders. PMID:27237748

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

    OpenAIRE

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

    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...

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

    Science.gov (United States)

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

    2016-01-01

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

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

    Institute of Scientific and Technical Information of China (English)

    张丽丽

    2015-01-01

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

  15. Effects of steroids therapy and ACTH therapy on the central nervous system evaluated by CT. Nephrotic syndrome and infantile spasms

    Energy Technology Data Exchange (ETDEWEB)

    Xano, E.; Yamashita, F.; Aoki, N.i; Matsuishi, T. (Kurume Univ., Fukuoka (Japan). School of Medicine); Yamamoto, M.

    1981-05-01

    CT scanning revealed cerebral atrophy of various degrees in 12 of 15 cases of nephrotic syndrome in which ACTH therapy was given and also in all the 8 cases of infantile spasms in which ACTH therapy was given. CT findings of cerebral atrophy were analysed by computor and expressed in terms of cerebrospinal fluid (CSF)-space/intracranial space ratio (%). The ratio was 6.6 +- 3.9% in nephrotic syndrome, 16.4 +- 7.8% in infantile spasms, and 1.6 +- 0.8% in control, showing statistically significant differences among them. Abnormal CT findings of 12 cases of nephrotic syndrome returned to normal after withdrawal of steroids in 9 cases (75%). CT findings in 8 cases of infantile spasms also improved in all the cases after withdrawal of ACTH therapy. The results suggested that cerebral atrophy on CT scan is a reversible change. However, the effect of steroids and ACTH on the central nervous system is not sufficiently known yet. We should be very careful about long-term administration of great amount of steroids or ACTH to infants and children.

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

    Directory of Open Access Journals (Sweden)

    Madani A

    2002-07-01

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

  17. 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

  18. 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

  19. Nephroprotective effect of heparanase in experimental nephrotic syndrome.

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    Suheir Assady

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

  20. 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.

  1. Use of high-dose prednisolone to overcome rifampicin-induced corticosteroid non-responsiveness in childhood nephrotic syndrome

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    H Barman

    2016-01-01

    Full Text Available Inducing remission in nephrotic children on anti-tubercular therapy is difficult due to the increased metabolism of prednisolone induced by rifampicin. We report a child with nephrotic syndrome treated successfully with an increased dose of steroids without discontinuing anti-tubercular therapy.

  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. 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

  4. 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.

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

    Directory of Open Access Journals (Sweden)

    Victor Gueutin

    2013-01-01

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

  6. Use of Cyclosporine Therapy in Steroid Resistant Nephrotic Syndrome (SRNS): A Review

    Science.gov (United States)

    Shah, Syed Raza; Altaf, Areeba; Arshad, Mohammad Hussham; Mari, Anum; Noorani, Sahir; Saeed, Eraj; Mevawalla, Areesh Amir; Haq, Zaiyn Ul; Faquih, Muhammad Ehsan

    2016-01-01

    A chronic, progressive disorder Steroid Resistant Nephrotic Syndrome (SRNS) accounts for 10-20% of all children with Nephrotic Syndrome. It is a heterogeneous disorder comprised of persistent edema, proteinuria, hypoalbuminemia and hyperlipidemia. Treatment for steroid-resistant nephrotic syndrome (SRNS) is challenging and children who suffer from SRNS require aggressive treatment to achieve remission. Calcineurin inhibitors have been used more in an empirical manner than on the basis of clear rationale. It was in 1984 when cyclosporine was first considered for the treatment of steroid resistant nephrotic syndrome. Cyclosporin is a calcineurin inhibitor that suppresses immune response by downregulating the transcription of various cytokine genes. Till now many studies have been conducted to determine dosages, duration of therapy, side effects and advantages of cyclosporine. Treatment of SRNS remains a difficult challenge in pediatric nephrology. Treatment should be individualized according to the underlying histopathology, and clinical and environmental conditions of the children. There is an urgent need to distinguish as soon as possible those patients who may benefit from prolonged immunosuppressive treatment from those who will not benefit from such treatment and who will just suffer from its major side effects. The emerging evidence that the majority of genetic forms of SRNS should receive symptomatic treatment only, should also be clinically tested and studies baring its significance should be evaluated in the future. PMID:26573045

  7. 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 the...

  8. 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; 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...

  9. 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.

  10. 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...

  11. 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.

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

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    Niloofar Hajizadeh

    2010-06-01

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

  13. 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 ...

  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. 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...

  16. Development of a Nephrotic Syndrome in a Patient with Gastrointestinal Stromal Tumor during a Long-Time Treatment with Sunitinib

    OpenAIRE

    PALLOTTI, MARIA CATERINA; Pantaleo, Maria Abbondanza; Nannini, Margherita; Centofanti, Francesca; Fabbrizio, Benedetta; Montanari, Mara; Baraldi, Olga; Saponara, Maristella; Lolli, Cristian; Mandrioli, Anna; Biasco, Guido; Prandini, Rita

    2012-01-01

    A patient with advanced gastrointestinal stromal tumor (GIST) receiving second-line treatment with sunitinib developed edema, increase of the serum creatinine, weight gain, nephrotic syndrome with proteinuria of 12 g/24 h, dyslipidemia, hypoalbuminemia and also presented with hypertension. A kidney biopsy showed an immunocomplex glomerulonephritis. Steroid treatment was started, but the clinical conditions and laboratory values did not improve. So in the hypothesis that the nephrotic syndrome...

  17. Congenital nephrotic syndrome of the Finnish type maps to the long arm of chromosome 19

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    Kestilae, M.; Maennikkoe, M.; Tryggvason, K.; Savolainen, E.R. (Univ. of Oulu (Finland)); Holmberg, C.; Peltonen, L. (Univ. of Helsinki (Finland)); Gyapay, G.; Weissenbach, J.

    1994-05-01

    Congenital nephrotic syndrome of the Finnish (CNF) is an autosomal recessive disease that is characterized by massive proteinuria and nephrotic syndrome at birth. CNF represents a unique, apparently specific dysfunction of the renal basement membranes, and the estimated incidence of CNF in the isolated population of Finland is 1 in 8,000 newborns. The basic defect is unknown, and no specific biochemical defect or chromosomal aberrations have been described. Here the authors report the assignment of the CNF locus to 19[sub q]12-q13.1 on the basis of linkage analysis in 17 Finnish families. Multipoint analyses and observed recombination events place the CNF locus between multiallelic markers D19S416 and D19S224, and the significant linkage disequilibrium observed suggests that the CNF gene lies in the immediate vicinity of the markers D19S224 and D19S220. 16 refs., 4 figs., 4 tabs.

  18. 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

  19. 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.

  20. 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

  1. 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...

  2. OCCURRENCE OF URINARY TRACT INFECTION IN CHILDREN WITH NEPHROTIC SYNDROME IN A TERTIARY CARE HOSPITAL

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    Vishwanath

    2015-12-01

    Full Text Available BACKGROUND Nephrotic Syndrome (NS is among the most common chronic kidney diseases occurring in childhood. Defense mechanisms are impaired in patients with NS. Immuno-compromised status of NS can result in increased susceptibility to various infections, which could be potentially serious and life-threatening without prompt diagnosis and treatment. The occurrence of Urinary Tract Infection (UTI is about 30% in children with nephrotic syndrome; however, it often goes undiagnosed. The present study is a prospective and a descriptive study designed to estimate the occurrence of urinary tract infection in children diagnosed to have NS. METHODS This is a prospective and descriptive study, which was conducted over a period of one year and included patients diagnosed to have NS who attended to the Paediatric Outpatient Department or were admitted at Father Muller Medical College Hospital over a study period of one year. Clean catch midstream urine samples were obtained from all the patients. Urine examination including routine analysis, microscopy, culture and sensitivity was done as per hospital standard policies. RESULTS This study included 30 children, out of which nine (30% were diagnosed with culture positive UTI (6 males and 3 females. The most common organism isolated from the urine culture was Escherichia coli, followed by Proteus, Enterococcus, Pseudomonas and Klebsiella, which was statistically significant (Binomial test p=0.043. Among the 9 cases who had culture positive UTI 3 were newly diagnosed NS and 6 were relapse NS. There was no significant difference between the two groups with regard to sex and age distribution. CONCLUSION This study concludes that urinary tract infections are an important but often under diagnosed infection in children with nephrotic syndrome. All children with nephrotic syndrome newly diagnosed or relapse should be screened for the presence of UTI.

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

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    Saime Paydas

    2015-01-01

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

  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. 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)

  6. 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

  7. 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

  8. 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...

  9. Complete Remission of Nephrotic Syndrome Without Resolution of Amyloid Deposit After Anti-Tumor Necrosis Factor α Therapy in a Patient With Ankylosing Spondylitis.

    Science.gov (United States)

    Lee, Yu Ho; Kim, Eun Young; Jeong, Da Wun; Kim, Yang-Gyun; Lee, Sang-Ho; Song, Ran; Yang, Hyung In; Lim, Sung Jig; Moon, Ju-Young; Lee, Sang-Hoon

    2016-03-01

    In secondary amyloid A amyloidosis resulting from rheumatologic diseases, tumor necrosis factor α blockers have been reported to be effective in the treatment of both arthritis and amyloidosis. However, there have been few reports concerning the alterations of renal tissue histology before and after long-term tumor necrosis factor α blockers therapy in secondary renal amyloidosis. We report the histological change after tumor necrosis factor α blocker therapy in patient with amyloid A amyloidosis and nephrotic syndrome secondary to underlying ankylosing spondylitis. The patient achieved complete remission of nephrotic syndrome after 17 months of etanercept treatment. We performed the second kidney biopsy after 40 months, and there was little change in the degree of amyloid deposition in the mesangial area and capillary loops compared with the first biopsy. The interstitial inflammation and foot process effacement, however, were fully recovered. PMID:26906302

  10. [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

  11. 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.

  12. 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

  13. 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

  14. Plasma homocysteine and B vitamins levels in Nigerian children with nephrotic syndrome

    Science.gov (United States)

    Orimadegun, Bose Etaniamhe; Orimadegun, Adebola Emmanuel; Ademola, Adebowale Dele; Agbedana, Emmanuel Oluyemi

    2014-01-01

    Introduction Available data on plasma homocysteine level in patients with nephrotic syndrome (NS) are controversial with increased, decreased and unchanged values reported. Therefore, plasma homocysteine and serum B vitamins in Nigerian children with NS were assessed in this study Methods Fasting blood samples were analysed for plasma homocysteine, serum folate and B vitamins in 42 children with NS and 42 age and sex-matched healthy controls in this case control study. Data were compared between NS and control using t test and Chi square. Relationships were tested with regression analysis with p set at 0.05. Results Prevalence of hyperhomocysteinaemia, low folate and cyanocobalamin in NS was 57.1%, 14.3% and 9.5% respectively. The mean homocysteine level was significantly higher in NS than control (11.3±2.6µmol/L versus 5.5±2.3µmol/L). Also, NS had lower folate and cyanocobalamin than control: 9.1±3.9ng/mL versus 11.2±3.1ng/dL and 268.5±95.7pg/mL versus 316±117.2pg/mL respectively. Weak but significant correlation between homocysteine and serum albumin (r = 0.347), folate (r = -0.607) and vitamin B12 (r = -0.185) were found in the NS group. Significant relationship was also found between homocysteine and vitamin B12 (ß = -0.64, 95% CI = -1.20, -0.08) after controlling for folate and vitamin B6 levels. Conclusion Clinically important hyperhomocysteinaemia and low B vitamins occur in Nigerian children with nephrotic syndrome. This data suggest that potential usefulness of folate and vitamin B supplementation for reducing high homocysteine levels in nephrotic syndrome need to be further investigated PMID:25404967

  15. Omega-3 fatty acid supplementation in primary nephrotic syndrome: effects on plasma lipids and coagulopathy.

    Science.gov (United States)

    Hall, A V; Parbtani, A; Clark, W F; Spanner, E; Huff, M W; Philbrick, D J; Holub, B J

    1992-12-01

    The effect of fish oil dietary supplementation on the dyslipidemia and coagulopathy of seven patients with nephrotic syndrome and hypoalbuminemia due to primary kidney disease was studied. Plasma lipids, platelet aggregation studies, simplate bleeding time, and fibrinogen levels were determined before and after 6 wk of treatment with fish oil (15 g/day of MaxEPA; 2.7 g of eicosapentenoic acid (EPA) and 1.8 g of docosahexenoic acid. Urea kinetics were determined from urine-urea concentration, urinary proteina, and urine volume. A 3-day dietary intake record was obtained from each patient before and after 6 wk of fish oil supplementation. There was no significant dietary change in protein, fat, or carbohydrate intake over the time period of the study. At study end, total triglycerides decreased from 2.98 +/- 1.31 to 2.18 +/- 1.14 mmol/L (P = 0.002), and very low-density lipoprotein-triglycerides decreased from 2.35 +/- 1.34 to 1.28 +/- 1.07 mmol/L (P = 0.01). Low-density lipoprotein (LDL) cholesterol increased from 5.18 +/- 1.74 to 7.35 +/- 2.83 mmol/L (P = 0.005). No significant changes occurred in bleeding time, platelet count, hematocrit, red blood cell flexibility, or whole blood viscosity. Platelet aggregation responses to collagen and arachidonic acid were consistently reduced after treatment, but there was no change in platelet response to ADP. The platelet membrane phospolipids showed a significantly increased incorporation of EPA after the fish oil diet (P = 0.03).(ABSTRACT TRUNCATED AT 250 WORDS) PMID:1477328

  16. The therapeutic potential of synthetic human atrial natriuretic peptide in nephrotic syndrome: a randomized controlled trial

    Directory of Open Access Journals (Sweden)

    Kanzaki M

    2012-06-01

    Full Text Available Motoko Kanzaki,1 Jun Wada,1 Yoko Kikumoto,1 Shigeru Akagi,1 Kazushi Nakao,3 Hitoshi Sugiyama,2 Hirofumi Makino11Department of Medicine and Clinical Science, 2Department of Chronic Kidney Disease and Peritoneal Dialysis, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan; 3Internal Medicine, Hiroshima City Hospital, Hiroshima, JapanBackground: In nephrotic syndrome, the combination of furosemide and albumin infusion is a standard regimen to treat systemic edema. The efficacy of synthetic human atrial natriuretic peptide (hANP for nephrotic syndrome to ameliorate the systemic edema and retain renal functions has not been fully demonstrated.Trial design: We conducted a prospective, randomized, controlled, open-label clinical trial. Patients were randomly assigned by a stratified biased coin design.Methods: A total of 12 patients with nephrotic syndrome between the ages of 20 to 79 years were enrolled and randomly assigned to either the conventional (CON group treated with furosemide and albumin, and hANP group, in which carperitide was administered in addition to the conventional therapies. The primary end points were: (1 the differences in serum creatinine levels, and (2 the reduction of total dosage of furosemide and albumin by the treatments of hANP. Secondary end points were body weight, systolic blood pressure, heart rate, serum protein, albumin, and urinary protein excretion.Results: A total of 13 patients were enrolled, and one patient was excluded due to severe pneumonia. In both hANP (n = 7 and CON (n = 5 groups, body weight was reduced after 2-week treatments. Serum creatinine levels at follow-up significantly increased compared with baseline. The increase in serum creatinine levels (Δ serum creatinine was smaller in the hANP group compared with the CON group (P = 0.31. The serum uric acid, serum urea nitrogen, and urinary protein excretion were reduced in the hANP group, and increased

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

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    Zeinab A. El-Sayed

    2014-01-01

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

  18. Renal vein thrombosis in nephrotic syndrome--a prospective study and review.

    OpenAIRE

    Chugh, K S; Malik, N.; Uberoi, H. S.; Gupta, V K; Aggarwal, M.L.; Singhal, P. C.; Suri, S.; Jain, S.K.

    1981-01-01

    The incidence of renal vein thrombosis (RVT) and other thrombo-embolic phenomena was evaluated in 44 unselected patients with nephrotic syndrome. Renal vein thrombosis was demonstrated by selective renal venography in 10 patients and at post-mortem in one. Extension of the thrombus from the renal veins into the inferior vena cava was seen in 3 patients. Evidence of thrombo-embolism elsewhere in the body was seen in the form of thrombophlebitis in the lower extremities in 4 patients (9.1%), pu...

  19. 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.

  20. Severe ocular hypertension secondary to systemic corticosteroid treatment in a child with nephrotic syndrome

    Directory of Open Access Journals (Sweden)

    Brito PN

    2012-10-01

    Full Text Available Pedro Nuno Brito,1 Sérgio Estrela Silva,1 José Silva Cotta,1 Fernando Falcão-Reis1,21Ophthalmology Department, Hospital S João, Porto, Portugal; 2Faculty of Medicine of Porto, University of Porto, Porto, PortugalPurpose: To report a case of severe, acute ocular hypertension in a 6-year-old child, 7 days after initiating treatment with oral prednisolone, due to nephrotic syndrome.Methods: A 6-year-old female Caucasian child was diagnosed with nephrotic syndrome and treated with oral prednisolone (60 mg/day. Seven days later the child initiated complaints of headache, vomiting, ocular pain, and photophobia. Ophthalmologic examination revealed a severely increased intraocular pressure (IOP of 52 mmHg in the right eye and 56 mmHg in the left eye. Anterior segment morphology was evaluated with ultrasound biomicroscopy. Optic disc status was evaluated by disc photography, kinetic perimetry, and optical coherence tomography.Results: Treatment was initiated with latanoprost, brimonidine, and the fixed association of timolol and dorzolamide. At each follow-up examination, progressively better control of IOP was obtained. Simultaneous with corticosteroid dosage decrease we were able to reduce antiglaucomatous medication while maintaining IOP under control. Ultrasound biomicroscopy revealed an open angle with normal anterior segment echographic findings. Perimetric evaluation revealed normal visual fields in both eyes. Four months after presentation, steroid treatment had been completed and IOP was 10 mmHg in both eyes without any antiglaucomatous medication. Optical coherence tomography revealed normal retinal nerve fiber layer thickness in all peripapillary sectors.Conclusions: Systemic steroid treatment can cause a severe, acute increase in IOP in children. Children undergoing steroid treatment should have routine ophthalmologic examinations during treatment duration. Prompt antiglaucomatous treatment prevents retinal nerve fiber layer damage and

  1. 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

  2. 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.

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

    DEFF Research Database (Denmark)

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

    2015-01-01

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

  4. The study of lipid profile, LP (a and electrolytes with oxidative stress, total protein and albumin in nephrotic syndrome

    Directory of Open Access Journals (Sweden)

    Jyoti Dwivedi

    2014-02-01

    Full Text Available Nephrotic syndrome is characterized by heavy proteinuria, hypoalbuminemia, hyperlipidemia associated with peripheral edema. Recent observation revealed that serum albumin plays an important role in the host defense mechanism as it is one of the important antioxidants. Oxidative damage by free radicals has been implicated in kidney injury, especially in nephrotic syndrome (NS. Therefore, this study was carried out to investigate oxidant and lipoprotein (a status with protein and electrolytes in nephrotic syndrome patients. The blood samples were analyzed for quantitation of malondialdehyde as index of lipid peroxide, total antioxidant capacity, lipid profile, lipoprotein (a, electrolytes, total protein and albumin. Significantly increased levels of serum lipid peroxide, lipoprotein (a LDL, VLDL, Tcholand decreased levels of serum total antioxidant capacity and total protein and albumin were noticed in the patients with nephrotic syndrome as compared to control subjects. Electrolytes are variable Na was increased and potassium was decreased. However, significant positive correlation in lipid peroxide with lipoprotein (a,and total protein and albumin with total antioxidant capacity were observed. [Int J Res Med Sci 2014; 2(1.000: 62-66

  5. The treatment of relapsing primary nephrotic syndrome in children

    Institute of Scientific and Technical Information of China (English)

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

    2005-01-01

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

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

    Science.gov (United States)

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

    2016-09-01

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

  7. 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.

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

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    Pio Liberatore

    2012-06-01

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

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

    Directory of Open Access Journals (Sweden)

    Margaret Duffy

    2015-10-01

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

  10. 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. Mapping of the locus for congenital nephrotic syndrome of the Finnish type (CNF) on chromosome 19

    Energy Technology Data Exchange (ETDEWEB)

    Kestilae, M.; Maennikkoe, M.; Tryggvason, K. [Univ. of Oulu (Finland)] [and others

    1994-09-01

    Congenital nephrotic syndrome of the Finnish type (CNF) is an autosomal recessive disease which forms a distinct entity among congenital nephrotic syndromes. It is characterized by massive proteinuria starting already in utero, large placenta and manifestation of nephrosis soon after birth. The incidence in Finland is about 1 in 8000 newborns, and the disease has been reported occasionally in other countries, particularly in Minnesota, USA. The gene defect in CNF is unknown, but the gene product is likely to be important for kidney development of glomerular filtration. We have used a random mapping approach in 17 Finnish CNF families resulting in the localization of the gene to chromosome 19q12-q13.1. Based on observed recombination events, the CNF locus is flanked by markers D19S191 and D19S224 corresponding to a region under 1 Mb in physical length. Cosmid contigs have been isolated from this region and at least two new polymorphic CA-repeat markers (MKMM1, MKMM2) have been identified from those clones. Statistically highly significant linkage disequilibrium can be observed with markers MKMM1, D19S224 and D19S220, the allelic association being about 65%. The most common haplotype, which was combined from these markers, is found in 60% of chromosomes carrying the CNF mutation. This work has enabled DNA-based diagnosis of CNF, and recently linkage and linkage disequilibrium analyses were used in prenatal diagnostics in a family with one affected child and two healthy siblings. DNA isolated from chorion villus biopsy was analyzed using markers D19S191, MKMM1, D19S224 and D19S220, and the fetus was shown to have the same genotype as the affected child.

  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. 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 ...

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

    Directory of Open Access Journals (Sweden)

    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

  17. Anesthetic management of a child with nephrotic syndrome undergoing open heart surgery: Report of a rare case

    Directory of Open Access Journals (Sweden)

    Vishnu Datt

    2012-01-01

    Full Text Available The congenital nephrotic syndrome (NS in infancy and childhood is an important entity but combination with acyanotic congenital heart disease is uncommon. Anesthesia in such cases is challenging because of associated problems like hypo-protienemia, anti-thrombin III deficiency, edema, hyperlipidemia, coagulopathy, cardiomyopathy, immunodeficiency, increased lung water etc. We describe anesthetic management of a patient with childhood NS and sinus venosus atrial septal defect (ASD undergoing open heart surgery. We also suggest guidelines for safe conduct of anesthesia and CPB in such patients.

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

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    Ayi Dilla Septarini

    2012-01-01

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

  19. 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

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

    Energy Technology Data Exchange (ETDEWEB)

    Eddy, A.A.; McCulloch, L.; Liu, E.; Adams, J. (Hospital for Sick Children, Toronto, ON (Canada))

    1991-05-01

    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.

  1. 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

  2. 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

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

    Directory of Open Access Journals (Sweden)

    Matthew G Sampson

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

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

    Directory of Open Access Journals (Sweden)

    Witold Szaflarski

    2012-01-01

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

  5. 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

  6. 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...

  7. 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

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

    Directory of Open Access Journals (Sweden)

    Al-Eisa AA

    2016-08-01

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

  9. Assay of urinary protein-bound sialic acid can differentiate steroidsensitive nephrotic syndrome from steroid-resistant cases

    Directory of Open Access Journals (Sweden)

    Niranjan Gopal

    2016-01-01

    Full Text Available The protein selectivity index as measured from the ratio of urinary immunoglobulin to albumin failed to differentiate between steroid-sensitive (SS and steroid-resistant (SR cases of nephrotic syndrome (NS. Sialic acid contributes negative charges to many plasma proteins. The negative charge is a determinant of protein excretion rate. The prognostic significance of assay of urinary excretion of protein-bound sialic acid in NS has not been evaluated. Hence, the present study was designed to evaluate whether measurement of urinary protein bound sialic acid (UPBSA can be used as a marker to differentiate SS from SR cases of NS. The urine samples of 70 (47 SS and 23 SR pediatric NS children were assayed for UPBSA by Aminoff′s method. The levels were compared and the receiver-operator curve was drawn to determine the optimum cutoff point to differentiate among the groups before starting the therapy. The excretion of UPBSA in SR cases of NS was significantly higher than that of SS cases (P<0.05. The optimum cutoff limit for UPBSA was 2.71 μg/mg of proteins with 75% sensitivity and 75.5% specificity for differentiating SS cases from SR cases (area under the plasma- concentration time curve = 0.814, P = 0.009. We conclude that UPBSA can differentiate SR cases from SS cases of NS in pediatric patients and may help in predicting the response to steroid therapy.

  10. Fine mapping and haplotype analysis of the locus for congenital nephrotic syndrome on chromosome 19q13.1

    Energy Technology Data Exchange (ETDEWEB)

    Maennikkoe, M.; Kestilae, M.; Tryggvason, K. [Univ. of Oulu (Finland)] [and others

    1995-12-01

    We have recently localized the gene for congenital nephrotic syndrome of the Finnish type (CNF) to chromosome 19q12-13.1. On the basis of observed recombination events, the gene was localized between markers D19S416/D19S425/D19S213/D19S208/D19S191 and D19S224. Here we have extended the mapping efforts, on the basis of a detailed physical map of the region. By means of three new polymorphic markers - D19S608, D19S609, and D19S610 - developed in this study, the critical candidate region could be further restricted. Significant linkage disequilibrium was observed with marker D19S610, D19S608, D19S224, and D19S220, the strongest allelic association being 84% with marker D19S610 at 19q13.1. This suggests that the CNF gene locus lies in close proximity to marker D19S610. Combination of the informative markers revealed four main haplotype categories. Different geographic distribution was observed between these haplotype groups when they were placed on the map of Finland according to the birthplaces of grandparents. 38 refs., 2 figs., 4 tabs.

  11. Nephrotic syndrome: an under-recognised immune-mediated complication of non-myeloablative allogeneic haematopoietic cell transplantation.

    Science.gov (United States)

    Srinivasan, R; Balow, J E; Sabnis, S; Lundqvist, A; Igarashi, T; Takahashi, Y; Austin, H; Tisdale, J; Barrett, J; Geller, N; Childs, R

    2005-10-01

    Nephrotic syndrome (NS) is an extremely rare complication of myeloablative allogeneic haematopoietic cell transplantation (HCT) that usually occurs in association with chronic graft-versus-host disease (C-GVHD). We observed an unexpectedly high incidence of NS in a cohort of 163 consecutive patients undergoing non-myeloablative HCT from a related human leucocyte antigen-compatible donor. Seven patients developed NS at a median 318 d post-transplant (range 119-1203 d; cumulative incidence 6.1%). The median age at onset of NS was 46 years (range 33-59 years); three of the seven patients had no evidence of C-GVHD while four had accompanying limited C-GVHD. At diagnosis, median proteinuria was 16.5 g/24 h (range 3-24 g/24 h). Renal biopsy was performed in four cases and revealed membranous nephropathy. NS was not always associated with other symptoms of C-GVHD, and in contrast to previous reports, usually did not improve with the re-initiation of aggressive immunosuppression, resulting in progressive renal failure necessitating dialysis in three of seven cases. Membranous nephropathy resulting in NS is a previously unrecognised and clinically significant complication of non-myeloablative HCT. PMID:16173966

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

    Directory of Open Access Journals (Sweden)

    Tsuyoshi Takashima

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

  13. Serum and urinary lipoproteins in the human nephrotic syndrome: evidence for renal catabolism of lipoproteins

    Energy Technology Data Exchange (ETDEWEB)

    Shore, V.G.; Forte, T.; Licht, H.; Lewis, S.B.

    1982-03-01

    The urinary excretion of lipoproteins and the possibility of catabolic alterations on glomerular filtration were investigated in four nephrotic subjects difering in etiology, serum lipoprotein profile, and 24 hr urinary output of protein and lipids. The apolipoproteins and lipoproteins of urine were compared with those of serum with respect to distribution profile, physical properties, and composition. As expected from molecular sieving effects during glomerular filtration, the urinary HDL were more abundant than the lower density lipoproteins even when the plasma LDL was elevated markedly. Intact apolipoproteins were not found in the concentrated urinary fraction isolated by ultrafiltration between the limits of 10/sup 4/ and 5 x 10/sup 4/ daltons. On the basis of immunoreactivity, gel electrophoresis, and amino acid composition, apolipoproteins B and AI are the major and minor proteins, respectively, of urinary LDL, and apo B is the major protein of the urinary IDL and VLDL. Apolipoproteins AI, AII, CI, CIII, and possibly AIV were isolated from the urinary HDL. As much as 20% of the protein moiety of the urinary HDL appeared to be large apolipoprotien fragments with molecular weights and isoelectric points similar to those of apo CII and apo CIII. The lower density classes of urinary lipoproteins also appeared to have lost apo E and apo C's and to have undergone partial proteolysis.

  14. Plasmin in nephrotic urine activates the epithelial sodium channel

    DEFF Research Database (Denmark)

    Svenningsen, Per; Bistrup, Claus; Friis, Ulla G; Bertog, Marko; Haerteis, Silke; Krueger, Bettina; Stubbe, Jane; Jensen, Ole Nørregaard; Thiesson, Helle C; Uhrenholt, Torben R; Jespersen, Bente; Jensen, Boye L; Korbmacher, Christoph; Skøtt, Ole

    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...... 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...

  15. Urinary Plasmin Inhibits TRPV5 in Nephrotic-Range Proteinuria

    OpenAIRE

    Tudpor, Kukiat; Laínez, Sergio; Kwakernaak, Arjan J.; Kovalevskaya, Nadezda V.; Verkaart, Sjoerd; van Genesen, Siebe; van der Kemp, AnneMiete; Navis, Gerjan; Bindels, René J. M.; Hoenderop, Joost G. J.

    2012-01-01

    Urinary proteins that leak through the abnormal glomerulus in nephrotic syndrome may affect tubular transport by interacting with membrane transporters on the luminal side of tubular epithelial cells. Patients with nephrotic syndrome can develop nephrocalcinosis, which animal models suggest may develop from impaired transcellular Ca2+ reabsorption via TRPV5 in the distal convoluted tubule (DCT). In nephrotic-range proteinuria, filtered plasminogen reaches the luminal side of DCT, where it is ...

  16. 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

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

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    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.

  18. Murine membranous nephropathy: immunization with α3(IV) collagen fragment induces subepithelial immune complexes and FcγR-independent nephrotic syndrome.

    Science.gov (United States)

    Zhang, Jun-Jun; Malekpour, Mahdi; Luo, Wentian; Ge, Linna; Olaru, Florina; Wang, Xu-Ping; Bah, Maimouna; Sado, Yoshikazu; Heidet, Laurence; Kleinau, Sandra; Fogo, Agnes B; Borza, Dorin-Bogdan

    2012-04-01

    Membranous nephropathy (MN) is a leading cause of nephrotic syndrome in adults and a significant cause of end-stage renal disease, yet current therapies are nonspecific, toxic, and often ineffective. The development of novel targeted therapies requires a detailed understanding of the pathogenic mechanisms, but progress is hampered by the lack of a robust mouse model of disease. We report that DBA/1 mice as well as congenic FcγRIII(-/-) and FcRγ(-/-) mice immunized with a fragment of α3(IV) collagen developed massive albuminuria and nephrotic syndrome, because of subepithelial deposits of mouse IgG and C3 with corresponding basement membrane reaction and podocyte foot process effacement. The clinical presentation and histopathologic findings were characteristic of MN. Although immunized mice produced genuine anti-α3NC1 autoantibodies that bound to kidney and lung basement membranes, neither crescentic glomerulonephritis nor alveolitis ensued, likely because of the predominance of mouse IgG1 over IgG2a and IgG2b autoantibodies. The ablation of activating IgG Fc receptors did not ameliorate injury, implicating subepithelial deposition of immune complexes and consequent complement activation as a major effector pathway. We have thus established an active model of murine MN. This model, leveraged by the availability of genetically engineered mice and mouse-specific reagents, will be instrumental in studying the pathogenesis of MN and evaluating the efficacy of novel experimental therapies. PMID:22371398

  19. 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

  20. 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.

  1. Dual energy CT pulmonary angiography for evaluation of pulmonary embolism in pediatric patients with nephrotic syndrome

    International Nuclear Information System (INIS)

    Objective: The purpose of this study was to evaluate the value of dual energy CT pulmonary angiography (DE-CTPA) in detection of pulmonary embolism in the children with nephritic syndrome. Materials and Methods: Fifty-two patients aged ≤18 years were included into this study from April 2010 to June 2011. Two radiologists reviewed and recorded the presence of perfusion defects or filling defects at dual energy CT perfusion images and CTPA images. CT enhancement values of embolic and nonembolic regions were measured with automatic and manual methods. Results: Of 52 patients, 11 (21.2%, 11/52) patients had pulmonary embolism. Of 11 patients with pulmonary embolism, 9 patients had lobar (n=3), segmental (n=5), and subsegmental (n=1) filling defects, while two patients were negative at the initial CTPA from average weighted images but positive at dual energy CT perfusion images. Eight patients had lobar (n=3), segmental (n=5) perfusion defects while 3 patients had no typical perfusion defects at dual energy CT perfusion images. Automatic and manual measurements showed the CT enhancement value of embolic regions measured with automatic method [(37.8±15.6) HU vs. (49.5±14.1) HU, t=-2.663, P=0.014] and CT enhancement value measured with annual method [(19.1±11.2) HU vs. (49.6±12.7) HU, t=-8.841, P<0.001] were lower than those of nonembolic regions. Conclusions: The incidence of pulmonary embolism was 21.2% in pediatric patients with nephritic syndrome in this study. Dual energy CTPA can show the perfusion defects resulting from pulmonary embolism, having a potential to improve the detection of pulmonary embolism in pediatric population. (authors)

  2. Cerebral sinovenous thrombosis in a nephrotic child

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    Rodrigues Marcelo Masruha

    2003-01-01

    Full Text Available Nephrotic syndrome in infancy and childhood is known to be associated with a hypercoagulable state and thromboembolic complications, but cerebral sinovenous thrombosis (CST is a very rare and serious one, with only a few isolated reports in the literature. A case is presented of a 9-year-old boy with nephrotic syndrome that acutely developed signs and symptoms of intracranial hypertension syndrome. CST was diagnosed on cranial CT and MRI and he gradually recovered after treatment with anticoagulants. The diagnosis of CST should be considered in any patient with nephrotic syndrome who develops neurologic symptoms. The discussion of this case, coupled with a review of the literature, emphasizes that early diagnosis is essential for institution of anticoagulation therapy and a successful outcome. This report also illustrates the difficulties that may be encountered in managing such a patient.

  3. 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

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

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    Carsten Sauer Mikkelsen

    2010-01-01

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

  5. 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

  6. 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

  7. Childhood Nephrotic Syndrome

    Science.gov (United States)

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

  8. Childhood Nephrotic Syndrome

    Science.gov (United States)

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

  9. 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

  10. 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...

  11. 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

  12. 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.

  13. Analysis of the genes responsible for steroid-resistant nephrotic syndrome and/or focal segmental glomerulosclerosis in Japanese patients by whole-exome sequencing analysis.

    Science.gov (United States)

    Ogino, Daisuke; Hashimoto, Taeko; Hattori, Motoshi; Sugawara, Noriko; Akioka, Yuko; Tamiya, Gen; Makino, Satoshi; Toyota, Kentaro; Mitsui, Tetsuo; Hayasaka, Kiyoshi

    2016-02-01

    Steroid-resistant nephrotic syndrome (SRNS) represents glomerular disease resulting from a number of different etiologies leading to focal segmental glomerulosclerosis (FSGS). Recently, many genes causing SRNS/FSGS have been identified. These genes encode the proteins associated with the formation and/or maintenance of glomerular filtration barrier. Next-generation sequencing is used to analyze large numbers of genes at lower costs. To identify the genetic background of Japanese patients, we studied 26 disease-causing genes using whole-exome sequencing analysis in 24 patients with SRNS and/or FSGS from 22 different Japanese families. We finally found eight causative gene mutations, four recessive and four dominant gene mutations, including three novel mutations, in six patients from five different families, and one novel predisposing mutation in two patients from two different families. Causative gene mutations have only been identified in ~20% of families and further analysis is necessary to identify the unknown disease-causing gene. Identification of the disease-causing gene would support clinical practices, including the diagnosis, understanding of pathogenesis and treatment. PMID:26467726

  14. 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.

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

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    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.

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

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    Claudio Bazzi

    2013-01-01

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

  17. 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.

  18. 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

  19. 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。

  20. 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.

  1. 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.

  2. 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

  3. White matter change on CT associated with superior vena cava syndrome: a case report

    International Nuclear Information System (INIS)

    An 11-year-old Japanese girl with nephrotic syndrome developed superior vena cava syndrome associated with hypercoagulability and an indwelling catheter. Cranial CT revealed diffuse low-density lesions in paraventricular white matter. Thrombectomy brought prompt relief of symptoms and correction of CT abnormalities. (orig.)

  4. White matter change on CT associated with superior vena cava syndrome: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Fukuda, M. (Department of Nephrology, Metropolitan Kiyose Children' s Hospital, Tokyo (Japan)); Nagai, T. (Department of Neurology, Metropolitan Kiyose Children' s Hospital, Tokyo (Japan)); Kamiyama, Y. (Department of Nephrology, Metropolitan Kiyose Children' s Hospital, Tokyo (Japan)); Kawamura, K. (Department of Nephrology, Metropolitan Kiyose Children' s Hospital, Tokyo (Japan)); Kawahara, K. (Department of Nephrology, Metropolitan Kiyose Children' s Hospital, Tokyo (Japan)); Honda, M. (Department of Nephrology, Metropolitan Kiyose Children' s Hospital, Tokyo (Japan))

    1993-03-01

    An 11-year-old Japanese girl with nephrotic syndrome developed superior vena cava syndrome associated with hypercoagulability and an indwelling catheter. Cranial CT revealed diffuse low-density lesions in paraventricular white matter. Thrombectomy brought prompt relief of symptoms and correction of CT abnormalities. (orig.)

  5. Genetic Aspects of Nephrotic Syndrome

    DEFF Research Database (Denmark)

    Joshi, Shivani

    a modifier. Nonetheless, such a combination might have variable penetrance and hence different age of onset. Thus, genetic testing in SRNS is important as it might give clues to the cause and save exposure of immunosuppressive medications in future patients with same genotype. Families comprising...... steroid dependence or become frequent relapsers. Repeated courses of corticosteroid treatment often cause significant associated morbidity. Familial occurrence of SSNS is rare and suggests a potential genetic origin. However, very little data on molecular genetics of familial SSNS is available in...... literature and no causal genes have yet been identified. Genetic aspects of NS bear important implications in therapeutic decisions and genetic counselling in SRNS patients and family members. During the present Ph.D. project we have studied the influence of genetic factors in patients with SRNS and familial...

  6. 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.

  7. 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

  8. 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.

  9. 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.

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

    Directory of Open Access Journals (Sweden)

    Madhan Krishan

    2009-01-01

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

  11. 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.

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

    Institute of Scientific and Technical Information of China (English)

    张汶娟; 刘素云; 任献青

    2013-01-01

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

  13. 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

  14. Differentiation of reversible ischemia from end-stage renal failure in nephrotic children with /sup 131/I-hippurate dynamic scintigraphy

    Energy Technology Data Exchange (ETDEWEB)

    Hattner, R.S.; Maltz, H.E.; Holliday, M.A.

    1977-05-01

    In renal failure associated with the nephrotic syndrome, therapeutic strategy is highly dependent upon the cause of the renal failure. Dynamic hippurate scintigraphy was studied in five pediatric patients. Four had nephrotic syndrome, and of these, three had acute renal failure. The fifth patient had end-stage renal failure. Specific alteration in renal hippurate kinetics offers a noninvasive assessment of renal failure in this clinical setting.

  15. 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 .

  16. Pulmonary embolism in nephrotic syndrome with thrombophilia

    OpenAIRE

    Pereira, J; F. LEITE; Pio, D.; Morais, L; Costa, T; Faria, M.; Barbot, J.; Mota, C.

    2011-01-01

    Introdução: As crianças com síndrome nefrótico (SN) têm um risco aumentado de complicações tromboembólicas. Caso clínico: Adolescente de 12 anos, sexo masculino, com síndrome nefrótico corticorresistente, medicado com prednisolona, ciclosporina, omeprazol, enalapril e ácido acetilsalicílico. Internado por quadro clínico de anasarca e oligúria com boa resposta clínica inicial à perfusão diária de albumina. Ao quarto dia de internamento iniciou febre, toracalgia, expectoração hem...

  17. 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

  18. 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

  19. 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

  20. 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

  1. 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

  2. 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.

  3. 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

  4. 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。

  5. 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.

  6. 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 .

  7. Serum erythropoietin level in anemic and non-anemic nephrotic children with normal kidney functions

    International Nuclear Information System (INIS)

    Nephrotic syndrome (NS) is associated with a significant alteration in protein metabolism. While lowering the concentration of certain proteins, the disease often raises the level of certain other proteins. The current study aimed to investigate the serum erythropoietin (EPO) levels in children with NS either anemic or non-anemic and to compare them to children with iron deficiency anemia (IDA) and healthy controls with normal hemoglobin level (NHB). Sixteen nephrotic children with anemia (NS-A) and 15 nephrotic children with normal hemoglobin level (NS-NHB) were examined and compared with 10 children with iron deficiency anemia (IDA) and 10 healthy controls (NHB). Circulating serum EPO levels, blood indices and iron status were measured in nephrotic patients with anemia (NS-A) and compared to those nephrotic patients with normal HE (NS-NHB). Most NS-A children were steroid resistant. The NS-A children showed greater EPO levels than those without anemia (21.01 ±4.02 mlU/ml versus 9.18 ± 0.79 mlU/ml; P < 0.001) but their response to treatment of anemia was inappropriately low when compared to IDA (EPO 96.9 ±4.9 mlU/ml) despite similar HB concentration. A significant positive correlation was observed between serum EPO and serum albumin in NS-A (r = 0.84, P < 0.001) and in NS-NHB group (r = 0.89, P < 0.001). Moreover, a significant positive correlation was observed between serum EPO and HB in the nephrotic groups indicating a blunted EPO response to anemia in NS-A (r 0.63, P < 0.05) and in NS-NHB group (r = 0.80, P < 0.001). In conclusion, anemia is a common feature of NS and is present even before the worsening of kidney function. Depletion of the iron stores due to loss of iron and transferrin in urine due to massive proteinurea may contribute to the development of anemia, but it was found that iron replacement was ineffective alone

  8. Metabolic Syndrome in African Americans: Views on Making Lifestyle Changes

    OpenAIRE

    Kirkendoll, Kenya D.; Clark, Patricia C.; Grossniklaus, Daurice A.; Igho-Pemu, Priscilla; Mullis, Rebecca M.; Dunbar, Sandra B.

    2010-01-01

    This study explores African American adults’ understanding of metabolic syndrome (MetS) and their motivations for making lifestyle changes. African Americans have a greater risk for components of MetS, such as hypertension.

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

    DEFF Research Database (Denmark)

    Sabroe, T.P.; Sabers, A.

    2008-01-01

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

  10. Purple urine bag syndrome- changing hue!

    Directory of Open Access Journals (Sweden)

    Sadhna Sharma

    2013-02-01

    Full Text Available Purple Urine Bag Syndrome (PUBS is a unique disease entity characterised by purple discoloration of urine secondary to recurrent urinary tract infections with indigo and indirubin producing bacteria and is predominantly seen in constipated, chronically debilitated and catheterised women with alkaline urine. This syndrome indicates underlying recurrent urinary tract infections (UTIs associated with higher incidence of mortality and morbidity than urinary tract infection alone without this occurrence. This article is about an elderly hypothyroid woman with PUBS and reviews the need to be aware of this entity. [Int J Res Med Sci 2013; 1(1.000: 31-32

  11. Propofol Infusion Syndrome Heralded by ECG Changes

    NARCIS (Netherlands)

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

    2012-01-01

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

  12. Crescimento e composição corporal em crianças e adolescentes com síndrome nefrótica córtico-dependente Growth and body composition in children and adolescents with steroid-dependent nephrotic syndrome

    Directory of Open Access Journals (Sweden)

    Iza de Castro Oliveira

    2009-12-01

    Full Text Available OBJETIVO: Avaliar crescimento e composição corporal em crianças e adolescentes com Síndrome Nefrótica Córtico-Dependente (SNCD. MATERIAL E MÉTODOS: Foram incluídos todos os pacientes de 5 a 18 anos, em acompanhamento por pelo menos dois anos, com diagnóstico de SNCD. Foram coletados dados referentes a: tempo de tratamento, idade de início de tratamento, valores consecutivos do colesterol, albuminemia, proteinemia total, dose de uso de corticoide e peso, estatura e idade da primeira consulta. As avaliações antropométricas dobra cutânea triciptal e subescapular, índice de massa corpórea, circunferência muscular do braço, circunferência da cintura e z-escore de estatura/idade foram realizadas durante as consultas de rotina e realizadas somente quando se considerou a criança sem edema clinicamente visível. Estatística não paramétrica com p OBJECTIVE: To assess growth and body composition in children and adolescents with steroid-dependent nephrotic syndrome (SDNS. METHODS: All children aged 5 to 18 years diagnosed with SDNS and followed up at the Pediatric Nephrology Unit for at least two years were studied. The following data were collected from the medical records: duration of treatment; age at treatment onset; total dose of steroids (mg/ kg and mg/kg/d; weight and height at the first visit; and the albuminemia, total proteinemia, and total cholesterol values. The anthropometric measures (triceps and subscapular skinfold thickness, body mass index, arm muscle circumference, waist circumference, and height/age z-score were routinely determined when patients had no clinical edema. Nonparametric statistics was used with significance level of p < 0.05. RESULTS: The study comprised 18 patients (11 males, aged 6 to 16 years (mean age, 12.22 ± 2.98, with a mean treatment duration of 6.75 ± 3.75 years. The initial height/ age z-score values were significantly greater than the final ones (-0.69 ± 0.80 and -2.07 ± 1

  13. 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

  14. Dentofacial and Cranial Changes in Down Syndrome

    OpenAIRE

    Shukla, Deepika; Bablani, Deepika; Chowdhry, Aman; Thapar, Raveena; Gupta, Puneet; Mishra, Shashwat

    2014-01-01

    Objectives This study aimed to determine the prevalence of certain oral characteristics usually associated with Down syndrome and to determine the oral health status of these patients. Methods The cross-sectional study was conducted among patients attending a special education program at Faculty of Dentistry, Jamia Millia Islamia, Delhi, India. The study design consisted of closed-ended questions on demographic characteristics (age, sex, and education and income of parents), dietary habits, a...

  15. 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

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

    OpenAIRE

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

    2014-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Guoqiang Xie

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

  18. Cardiovascular Changes in Animal Models of Metabolic Syndrome

    Directory of Open Access Journals (Sweden)

    Alexandre M. Lehnen

    2013-01-01

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

  19. 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

  20. 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.

  1. Changing face of irritable bowel syndrome

    Institute of Scientific and Technical Information of China (English)

    Eamonn MM Quigley

    2006-01-01

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

  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. 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)

  4. 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

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

    Directory of Open Access Journals (Sweden)

    Tian-Biao Zhou

    2011-01-01

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

  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. 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.

  8. 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,

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

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    Svintsitskyy A.

    2014-06-01

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

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

    Science.gov (United States)

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

    2014-01-01

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

  11. Gray and white matter structural changes in corticobasal syndrome.

    Science.gov (United States)

    Upadhyay, Neeraj; Suppa, Antonio; Piattella, Maria Cristina; Di Stasio, Flavio; Petsas, Nikolaos; Colonnese, Claudio; Colosimo, Carlo; Berardelli, Alfredo; Pantano, Patrizia

    2016-01-01

    We investigated gray matter and white matter (WM) changes in corticobasal syndrome (CBS). T1-weighted and diffusion tensor images (3T-magnet) were obtained in 11 patients and 11 healthy subjects (HS). Magnetic resonance imaging data were analyzed using FreeSurfer and Tracts Constrained by Underlying Anatomy to evaluate cortical thickness (CTh), surface area, and subcortical volumes as well as diffusion tensor image parameters along the major WM tracts. Compared with HS, the whole patient group showed decreased CTh in the prefrontal cortex, precentral gyrus, supplementary motor area, insula, and temporal pole bilaterally. When we divided patients into 2 subgroups (left: L-CBS, right: R-CBS) on the basis of the clinically more affected upper limb, the most prominent decrease in CTh occurred in the hemisphere contralateral to the more affected side. The whole patient group also had volume loss in the putamen, hippocampus, and accumbens bilaterally, in the corpus callosum and right amygdala. Finally, we found diffusion changes in several WM tracts with axial diffusivity being altered more than radial diffusivity. The upper limb motor severity negatively correlated with the contralateral CTh in the precentral and/or postcentral gyri and contralateral volumes of putamen and accumbens. The CTh asymmetry in postcentral and/or paracentral gyri also negatively correlated with disease duration. Cortical thinning, volume loss, and fiber tract degeneration in specific brain regions are important pathophysiological abnormalities in CBS. PMID:26545629

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

    Science.gov (United States)

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

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

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

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    Yoon-Hee Cha

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

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

    International Nuclear Information System (INIS)

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

  15. Learning without Representational Change: Development of Numerical Estimation in Individuals with Williams Syndrome

    Science.gov (United States)

    Opfer, John E.; Martens, Marilee A.

    2012-01-01

    Experience engenders learning, but not all learning involves representational change. In this paper, we provide a dramatic case study of the distinction between learning and representational change. Specifically, we examined long- and short-term changes in representations of numeric magnitudes by asking individuals with Williams syndrome (WS) and…

  16. Uptake of indium-111 labelled platelets by normal, nephrotic and transplanted kidneys

    Energy Technology Data Exchange (ETDEWEB)

    Desir, G.; Lange, R.; Smith, E.; Bia, M.; Flye, M.; Kashgarian, M.; Canganelli, A.; Ezekowitz

    1984-01-01

    To determine the role of platelets in the genesis of renal transplant (T) rejection, the authors studied 3 groups of adult patients. Group I, n=8, had normal renal function (Cr=1 +- 0.1 mg%, Mean +- SD). Group II, n=9, had nephrotic syndrome (Cr=2.4 +- 1). Group III, n=7, consisted of 5 cadaveric (C) and 2 living related donor (LRD) T. In Group II, 1 patient had received a T 4 years prior to study. Group I and II received 448 +- 101 ..mu..Ci and Group III 236 +- 51 ..mu..Ci of Indium-111. In Groups I and II the first image was obtained 18 +- 6 hrs after injection. In Group II the first was obtained 6 +- 2 hr after injection and 1-3 times/day thereafter for a maximum of 7 days. Renal biopsies were obtained in all patients in Group III during imaging (n=5) or within 2 - 5 days of the last image. One patient was studied twice. In Group III, 5 patients received prednisone and azothiaprine and 2 prednisone and cyclosporine. Platelet uptake index (PUI) was calculated as the ratio of uptake over the T against a reference area. Rejection was diagnosed by biopsy. In groups I and II platelet uptake was seen only in the T patient. In Group III the PUI was 1.54 +- .13 in the rejecting T (n=5), 1.42 +- .2 in the non-rejecting T (n=3), 1.62 in a LRD non-rejecting T and 1.31 (n=2) in C non-rejecting T. In the four patients studied within 5 days of T the PUI was elevated at 1.47 +- .1. The authors conclude that: 1) platelets do not accumulate in normal or nephrotic native kidneys, 2) significant uptake occurs in the first week after C and LRD whether or not rejection is present, and 3) uptake in non-rejecting kidneys cannot be ascribed to perfusion induced endothelial injury since it was present in LRD transplants.

  17. Uptake of indium-111 labelled platelets by normal, nephrotic and transplanted kidneys

    International Nuclear Information System (INIS)

    To determine the role of platelets in the genesis of renal transplant (T) rejection, the authors studied 3 groups of adult patients. Group I, n=8, had normal renal function (Cr=1 +- 0.1 mg%, Mean +- SD). Group II, n=9, had nephrotic syndrome (Cr=2.4 +- 1). Group III, n=7, consisted of 5 cadaveric (C) and 2 living related donor (LRD) T. In Group II, 1 patient had received a T 4 years prior to study. Group I and II received 448 +- 101 μCi and Group III 236 +- 51 μCi of Indium-111. In Groups I and II the first image was obtained 18 +- 6 hrs after injection. In Group II the first was obtained 6 +- 2 hr after injection and 1-3 times/day thereafter for a maximum of 7 days. Renal biopsies were obtained in all patients in Group III during imaging (n=5) or within 2 - 5 days of the last image. One patient was studied twice. In Group III, 5 patients received prednisone and azothiaprine and 2 prednisone and cyclosporine. Platelet uptake index (PUI) was calculated as the ratio of uptake over the T against a reference area. Rejection was diagnosed by biopsy. In groups I and II platelet uptake was seen only in the T patient. In Group III the PUI was 1.54 +- .13 in the rejecting T (n=5), 1.42 +- .2 in the non-rejecting T (n=3), 1.62 in a LRD non-rejecting T and 1.31 (n=2) in C non-rejecting T. In the four patients studied within 5 days of T the PUI was elevated at 1.47 +- .1. The authors conclude that: 1) platelets do not accumulate in normal or nephrotic native kidneys, 2) significant uptake occurs in the first week after C and LRD whether or not rejection is present, and 3) uptake in non-rejecting kidneys cannot be ascribed to perfusion induced endothelial injury since it was present in LRD transplants

  18. Changing the Perspective on Early Development of Rett Syndrome

    Science.gov (United States)

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

    2013-01-01

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

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

    International Nuclear Information System (INIS)

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

  20. Visualization of podocyte substructure with structured illumination microscopy (SIM): a new approach to nephrotic disease.

    Science.gov (United States)

    Pullman, James M; Nylk, Jonathan; Campbell, Elaine C; Gunn-Moore, Frank J; Prystowsky, Michael B; Dholakia, Kishan

    2016-02-01

    A detailed microscopic analysis of renal podocyte substructure is essential to understand and diagnose nephrotic kidney disease. Currently only time consuming electron microscopy (EM) can resolve this substructure. We used structured illumination microscopy (SIM) to examine frozen sections of renal biopsies stained with an immunofluorescence marker for podocin, a protein localized to the perimeter of the podocyte foot processes and compared them with EM in both normal and nephrotic disease biopsies. SIM images of normal glomeruli revealed curvilinear patterns of podocin densely covering capillary walls similar to podocyte foot processes seen by EM. Podocin staining of all nephrotic disease biopsies were significantly different than normal, corresponding to and better visualizing effaced foot processes seen by EM. The findings support the first potential use of SIM in the diagnosis of nephrotic disease. PMID:26977341

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

    OpenAIRE

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

    2011-01-01

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

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

    OpenAIRE

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

    2010-01-01

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

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

    OpenAIRE

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

    1988-01-01

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

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

    OpenAIRE

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

    2014-01-01

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

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

    Science.gov (United States)

    McLaughlin, Katrina; Jones, Aled

    2011-01-01

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

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

    Science.gov (United States)

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

    2015-01-01

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

  7. Ischemic Changes in a Case of Unilateral Pseudoexfoliation Syndrome

    Directory of Open Access Journals (Sweden)

    Tolga Kocaturk

    2016-05-01

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

  8. Physiological Changes and Clinical Implications of Syndromic Craniosynostosis

    Science.gov (United States)

    Matsusaka, Yasuhiro; Kunihiro, Noritsugu; Imai, Keisuke

    2016-01-01

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

  9. Nephrotic-range proteinuria in an eight-year-old traveler with severe dengue: Case report and review of the literature.

    Science.gov (United States)

    Hebbal, Pooja; Darwich, Yamil; Fong, Jane; Hagmann, Stefan H F; Purswani, Murli U

    2016-01-01

    We report a case of an eight-year-old male, native of the Dominican Republic, who visited the U.S. and was admitted to a pediatric intensive care unit with severe dengue. He needed aggressive fluid management for dengue shock syndrome and developed proteinuria on the sixth day of his illness, shortly after his nadir thrombocytopenia. His proteinuria peaked on the eight day, and reduced to trace levels by the tenth day of his illness, coinciding with normalization of his platelet count. His highest random urine protein/creatinine ratio was in the nephrotic range, at 3.9 g/g. Dengue fever can cause a wide spectrum of acute kidney injury (AKI), ranging in incidence from 0.9 to 36%. Review of the literature shows that nephrotic-range proteinuria is an uncommon complication of AKI caused by dengue, reported thus far only in Southeast Asia. Immune-mediated mechanisms may explain the observed association between dengue-induced thrombocytopenia and severe proteinuria, in this case, and previously reported cases. Dengue virus infection is the commonest mosquito-borne disease in the world with substantial morbidity and mortality. Well-designed prospective studies are needed to further characterize the extent and mechanisms of AKI in populations living in countries with ongoing transmission, as well as in those with travel-associated disease. PMID:26874782

  10. 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...

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

    Science.gov (United States)

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

    2016-07-01

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

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

    OpenAIRE

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

    2007-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Piccoli Giorgina

    2012-02-01

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

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

    Directory of Open Access Journals (Sweden)

    Katherine J. Coffey

    2015-10-01

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

  15. Participant attributions for global change ratings in unexplained chronic fatigue and chronic fatigue syndrome.

    Science.gov (United States)

    Friedberg, Fred; Coronel, Janna; Seva, Viktoria; Adamowicz, Jenna L; Napoli, Anthony

    2016-05-01

    The purpose of this mixed methods study was to identify participants' attributions for their global impression of change ratings in a behavioral intervention for unexplained chronic fatigue and chronic fatigue syndrome. At 3-month follow-up, participants (N = 67) were asked "Why do you think you are (improved, unchanged, worse)?" Improved patients pointed to specific behavioral changes, unchanged patients referred to a lack of change in lifestyle, and worsened patients invoked stress and/or specific life events. Identifying patient perceptions of behaviors associated with patient global impression of change-rated improvement and non-improvement may assist in developing more effective management strategies in clinical care. PMID:24913009

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

    Science.gov (United States)

    Walton, J

    1997-08-22

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

  17. Morphological changes of carotid bodies in acute respiratory distress syndrome: a morphometric study in humans

    OpenAIRE

    E.N.G. Vinhaes; Dolhnikoff, M; Saldiva, P. H. N.

    2002-01-01

    Carotid bodies are chemoreceptors sensitive to a fall of partial oxygen pressure in blood (hypoxia). The morphological alterations of these organs in patients with chronic obstructive pulmonary disease (COPD) and in people living at high altitude are well known. However, it is not known whether the histological profile of human carotid bodies is changed in acute clinical conditions such as acute respiratory distress syndrome (ARDS). The objective of the present study was to perform a quantita...

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

    OpenAIRE

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

    2015-01-01

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

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

    Science.gov (United States)

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

    2016-08-01

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

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

    Directory of Open Access Journals (Sweden)

    Victoria O. Sánchez

    2014-07-01

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

  1. Neurologic deterioration with progressive CT changes in a child with Kearns-Shy syndrome

    International Nuclear Information System (INIS)

    A case of the rare juvenile form of Kearns-Shy syndrome with progressive external ophthalmoplegia and lid ptosis, carditis, skeletal muscle weakness, seizures, mental subnormality, short stature, EEG abnormality and deafness is presented. Electromyography revealed a myopathic pattern. Histochemical studies on quadriceps biopsy specimens showed atrophy of type II fibers and ''ragged-red fibers.'' On electron microscopy these muscle cells were seen to contain an increased amount of glycogen particles and abnormal mitochondria were increased in number and size. It is of interest that abrupt deterioration of neurological findings such as seizures, mental subnormality, speech disturbance and deafness was present in our case. Computed tomographic scanning showed progressive changes of cerebral atrophy, low density of cerebral white matter and basal ganglia calcification, which were well associated with the clinical deterioration. A review of the literature also indicated that some patients with this syndrome showed abrupt neurological deterioration in childhood. Involvement of the central nervous system in this syndrome has to be considered as the cause of sudden deterioration and death in childhood. (author)

  2. Neurologic deterioration with progressive CT changes in a child with Kearns-Shy syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Yoda, Satoru; Kitahara, Fuminori; Akabane, Taro (Shinshu Univ., Matsumoto, Nagano (Japan). Faculty of Medicine); Terauchi, Akiko

    1984-01-01

    A case of the rare juvenile form of Kearns-Shy syndrome with progressive external ophthalmoplegia and lid ptosis, carditis, skeletal muscle weakness, seizures, mental subnormality, short stature, EEG abnormality and deafness is presented. Electromyography revealed a myopathic pattern. Histochemical studies on quadriceps biopsy specimens showed atrophy of type II fibers and ''ragged-red fibers.'' On electron microscopy these muscle cells were seen to contain an increased amount of glycogen particles and abnormal mitochondria were increased in number and size. It is of interest that abrupt deterioration of neurological findings such as seizures, mental subnormality, speech disturbance and deafness was present in our case. Computed tomographic scanning showed progressive changes of cerebral atrophy, low density of cerebral white matter and basal ganglia calcification, which were well associated with the clinical deterioration. A review of the literature also indicated that some patients with this syndrome showed abrupt neurological deterioration in childhood. Involvement of the central nervous system in this syndrome has to be considered as the cause of sudden deterioration and death in childhood.

  3. Kindler syndrome

    Directory of Open Access Journals (Sweden)

    Kaviarasan P

    2005-01-01

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

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

    OpenAIRE

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

    2001-01-01

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

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

    International Nuclear Information System (INIS)

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

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

    Directory of Open Access Journals (Sweden)

    Barbara Grzechocińska

    2011-06-01

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

  7. Efecto del micofenolato mofetil (MMF en el síndrome nefrótico idiopático (SNI en niños, resistente a otros tratamientos Micophenolate mofetil (MMF in the idiopathic nephrotic syndrome (SNI resistantance to different treatments

    Directory of Open Access Journals (Sweden)

    José William Cornejo Ochoa

    2006-01-01

    Full Text Available El intento para disminuir los efectos adversos del uso prolongado y de las grandes dosis de esteroides en el tratamiento del síndrome nefrótico idiopático (SNI corticodependiente o corticoresistente, justifica el uso de inmunosupresores, muchos de los cuales también causan efectos adversos además de producir respuestas variables. El objetivo de este trabajo fue la evaluación retrospectiva de la evolución clínica y de los parámetros de laboratorio en 11 pacientes con SNI (siete corticoresistentes y cuatro corticodependientes, luego de seis meses de tratamiento con MMF. El diagnóstico histopatológico fue glomeruloesclerosis focal y segmentarias (GEFS en ocho pacientes, glomerulonefritis membranosa (GNM en dos y glomerulonefritis membranoproliferativa tipo II (GNMP en uno. El promedio de edad de inicio del SNI fue de 7,8 años (rango 2-14, y el de la edad al inicio del MMF fue de 9,9 años (rango 2,5-16. El MMF se administró en una dosis promedio de 406 mg/m2/12h (rango 186,5-600. Se encontró respuesta parcial al MMF en 4 pacientes y completa en 1; igualmente se observó tendencia a mejor control de la hipertensión arterial y resolución de edemas, manteniendo una función renal estable. The attempt to diminish the adverse effects of the prolonged use and the great doses of steroids in the treatment of the idiopathic nephritic syndrome (SNI with dependency and/or resistance to steroids, justifies the use of immunosuppressive drug, many of these with variable answers and also adverse effects. The objective of this work was to evaluate the clinical evolution and of the laboratory parameters in the patients with SNI to the 6 months of treatment with Mycophenolate mofetil. The trial included 11 patients with SNI, 7 with resistance and 4 with dependency to steroids, the histopathology diagnosis were: 8 focal and segmental glomeruloesclerosis (GEFS, 2 membranous glomerulonephritis (GNM, 1 membranoproliferative type II glomerulonephritis

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

    DEFF Research Database (Denmark)

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

    2009-01-01

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

  9. 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.

  10. 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...

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

    International Nuclear Information System (INIS)

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

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

    Directory of Open Access Journals (Sweden)

    Paula Wesendonck Caponi

    2013-07-01

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

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

    Energy Technology Data Exchange (ETDEWEB)

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

    2013-10-15

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

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

    Science.gov (United States)

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

    2014-01-01

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

  15. Bi-level CPAP does not change central blood flow in preterm infants with respiratory distress syndrome

    OpenAIRE

    Aquilano, Giulia; Galletti, Silvia; Aceti, Arianna; Vitali, Francesca; Faldella, Giacomo

    2014-01-01

    Background Current literature provides limited data on the hemodynamic changes that may occur during bi-level continuous positive airway pressure (CPAP) support in preterm infants. However, the application of a positive end-expiratory pressure may be transmitted to the heart and the great vessels resulting in changes of central blood flow. Objective To assess changes in central blood flow in infants with respiratory distress syndrome (RDS) during bi-level CPAP support. Design A prospective st...

  16. Anterior chamber depth and refractive change in late postoperative capsular bag distension syndrome: a retrospective analysis.

    Directory of Open Access Journals (Sweden)

    Min Kyu Yang

    Full Text Available To assess the characteristic findings and effects of laser capsulotomy in patients with late postoperative capsular bag distension syndrome (CBDS.Twenty patients diagnosed with late postoperative CBDS between July 2010 and August 2013 were retrospectively reviewed. Before and 1 week after capsulotomy, changes in the anterior chamber depth (ACD were assessed using ultrasound biomicroscopy. Changes in the refractive status and uncorrected visual acuity (UCVA were also measured 1 week and 1 month after capsulotomy. For patients who received bilateral cataract surgery, preoperative ACD and axial length measured by IOLMaster were compared between the two eyes.Twenty-two eyes from 20 patients who had undergone laser capsulotomy showed a mean UCVA improvement of 0.27 ± 0.24 logMAR (range, 0.00-0.90. ACD was increased by an average of +0.04 mm (95% confidence interval, +0.01 to +0.06 mm, p = 0.034, equivalent to predicted refractive change of +0.10 D. The discrepancy between actual (+1.33 D and predicted refractive change after capsulotomy suggests that refractive change may not be generated from IOL displacement in late postoperative CBDS. Preoperative ACD was deeper in the eye with late postoperative CBDS in all bilaterally pseudophakic patients (mean, 3.68 mm vs. 3.44 mm in the fellow eye, p = 0.068.Late postoperative CBDS showed refractive changes that were resolved successfully after laser capsulotomy. The convex lens effects of opalescent material in the distended capsular bag may play a major role in myopic shift. A larger preoperative ACD is possibly associated with the development of late postoperative CBDS.

  17. Kidney biopsy

    Science.gov (United States)

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

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

    Science.gov (United States)

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

    2016-04-01

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

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

    Directory of Open Access Journals (Sweden)

    B Vijaya Gowri

    2015-01-01

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

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

    Institute of Scientific and Technical Information of China (English)

    张雪哲; 王武; 卢延; 黄振国; 洪闻; 尚燕宁; 任安

    2003-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Kiani Asiabar A.

    2007-09-01

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

  2. 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...

  3. 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

  4. 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...

  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

    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 the...... Schwartz formula, and only patients with normal values were included. RESULTS: A total of 14 children were examined, 13 (93%) had 25(OH)D deficiency including 12 (86%) with moderate or severe vitamin D deficiency. The plasma 25(OH)D was positively associated with plasma albumin (p = 0.031) and negatively...

  6. Treatment of progressive renal failure and nephrotic syndrome with azathioprine and prednisolone.

    OpenAIRE

    Baker, L R; Tucker, B; Macdougall, I C; Oommen, R

    1997-01-01

    Four patients with idiopathic membranous glomerulonephritis, heavy proteinuria and progressive renal failure received azathioprine and prednisolone. Renal function improved in all four and proteinuria declined sharply in three. We suggest that treatment with azathioprine and prednisolone may be of benefit in this form of idiopathic membranous glomerulonephritis.

  7. Type-1 diabetes mellitus, nephrotic syndrome, and vur with ace gene polymorphism

    International Nuclear Information System (INIS)

    Objectives: To determine sensitivity, specificity and other operating characteristics of bedside three-point compression ultrasonography performed in emergency department by emergency physicians in comparison with duplex ultrasonography. Methods: The cross-sectional study at Rasoul-e-Akram Hospital in Tehran, Iran, prospectively evaluated 81 suspected patients of lower extremity deep vein thrombosis between March 2006 and March 2007. A trained second-year resident and one attending physician of emergency medicine evaluated the veins of all the patients with through compression ultrasonography. Then, a second-year resident of radiology assessed the patients with duplex ultrasonography. Finally, data were compared and quantitative and categorical variables were worked out along with other statistical analysis through SPSS version 16. Results: The mean age of the patients was 47.2 +- 18.6 years. When cases who lost the compressibility of at least one of their femoral or popliteal veins were considered to be positive, there were 80.2% diagnosed by compression ultrasonography and 79% by the duplex variety. Sensitivity, specificity and accuracy of the former in comparison with the latter were 85.9%, 41.2% and 84.6% respectively. Conclusion: Compression ultrasonography has relatively an acceptable sensitivity and accuracy level, but has low specificity in the diagnosis of deep vein thrombosis in the hands of Iranian emergency physicians. It is better to implement duplex ultrasonography whenever accessible. Otherwise, compression ultrasonography results should be compared with the results of duplex ultrasonography as soon as possible. (author)

  8. A fulminant case of Guillain-Barré syndrome: topographic and fibre size related analysis of demyelinating changes.

    OpenAIRE

    Kanda, T; Hayashi, H.; H. Tanabe; Tsubaki, T; Oda, M.

    1989-01-01

    In a necropsy case of early fulminant Guillain-Barré syndrome, demyelinating changes were observed throughout the peripheral nervous system, most conspicuous in the spinal nerve roots. The central/peripheral nervous system transition regions and most proximal zones of the roots escaped damage. In some root areas there were widespread early changes in myelin sheaths in the absence of significant infiltrates of inflammatory cells. In the fibre size analytical study, small myelinated fibres were...

  9. Pseudomyopathic Changes in Needle Electromyography in Lambert-Eaton Myasthenic Syndrome

    Directory of Open Access Journals (Sweden)

    Teppei Komatsu

    2013-01-01

    Full Text Available Lambert-Eaton myasthenic syndrome (LEMS is a rare presynaptic disorder of the neuromuscular junction in association with cancer and subsequently in cases in which no neoplasm has been detected (O’Neill et al., 1988. The diagnosis of LEMS is based on the combination of fluctuating muscle weakness, diminished or absent reflexes, and a more than 60% increment of compound muscle action potential (CMAP amplitude after brief exercise or 50 Hz stimulation for 1 s in a repetitive nerve stimulation (RNS test (Oh et al., 2005. On the other hand, needle electromyography (EMG findings related to LEMS have not been well described. Here, we report a case of LEMS, which showed apparent myopathic changes in needle EMG findings. Furthermore, we retrospectively examined the needle EMG findings in 8 patients with LEMS. In six of the 8 patients, the EMG findings showed myopathy-like findings. Although the findings of needle EMG indicated myopathic changes at a glance, the motor unit potential (MUP returned to normal after a sustained strong muscle contraction. We propose the name “pseudomyopathic changes” for this phenomenon.

  10. Myopathological features in MELAS syndrome without significant changes in muscle biopsy pathology

    Institute of Scientific and Technical Information of China (English)

    Pu Fang; Chengsi Wu; Meihong Zhou; Renshi Xu; Xianhua Liu; Caixia Wei

    2010-01-01

    Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) are common types of mitochondrial encephalomyopathy. The involved muscular pathology is characterized by typical changes of mitochondrial abnormalities. Gene screening has been the gold diagnostic standard for MELAS diagnosis. This study presents three primary MELAS patients, with an age of onset from 13 to 18 years, including one patient with seizure, and two with headache and vomiting.All patients had a family history of disease, with matermal inheritance. Cerebral magnetic resonance imaging revealed abnormally high signals in T2-weighted images: temporal lobe in three cases,occipital lobe in two cases, and parietal lobe in one case. Migrating stroke-like lesions were confirmed in one patient. Muscle biopsy revealed several strongly succinate dehydrogenase-reactive vessels scattered in muscle sections of three patients, but ragged-red fibers and cytochrome c oxidase-negative/dense (COX-/+) fibers were not observed. Mitochondrial DNA A3243G mutation was identified in all three cases. MELAS syndrome has obvious clinical heterogeneity, and muscle weakness was not prominent in some of the cases. Muscle pathological changes did not accompany ragged-red fibers or COX-/+ fibers, but succinate dehydrogenasereactive vessels are important for MELAS diagnosis.

  11. Neurochemical–structural changes evaluation of brain in patients with obstructive sleep apnea syndrome

    International Nuclear Information System (INIS)

    Purpose: To evaluate neurochemical and structural changes in the patients with newly diagnosed obstructive sleep apnea syndrome (OSAS) by MR spectroscopy (MRS), T2 relaxometry, and diffusion weighted imaging (DWI). Material and methods: Following the acquisition of routine cranial MR, MRS, T2 relaxometry, and DWI images; spectroscopic metabolite ratios and DWI–T2 relaxometry findings of the thalami, hippocampi, frontal white matter (FWM) and frontal cortex of 24 OSAS patients and 9 controls were statistically compared. The relationship between two groups was evaluated with Mann–Whitney test. Results: Spectroscopic measurements in the frontal cortex and frontal white matter of the OSAS patients revealed significantly lower NAA/Cr ratios than those of the control group (P = 0.004 and P = 0.006, respectively). The measurements in the frontal white matter of the OSAS patients exhibited significantly lower NAA/Cho ratios compared with those of the control group (P = 0.005). Thalamic Cho/Cr ratios of the patient group were significantly higher than those of the control group (P = 0.002). In terms of the ADC–T2 relaxometry values, there was no significant relationship between the patient and the control groups (P > 0.05). Conclusion: MRS is a useful and non-invasive modality in showing neurochemical changes in various regions of the brain but our data does not show any change on diffusion weighting or T2 quantification in the OSAS group. DWI and T2 relaxometry appear to be not effective techniques to evaluate the brain structural changes of the patients with newly diagnosed OSAS.

  12. Widespread cerebellar transcriptome changes in Ts65Dn Down syndrome mouse model after lifelong running.

    Science.gov (United States)

    Walus, Marius; Kida, Elizabeth; Rabe, Ausma; Albertini, Giorgio; Golabek, Adam A

    2016-01-01

    Our previous study showed an improvement in locomotor deficits after voluntary lifelong running in Ts65Dn mice, an animal model for Down syndrome (DS). In the present study, we employed mouse microarrays printed with 55,681 probes in an attempt to identify molecular changes in the cerebellar transcriptome that might contribute to the observed behavioral benefits of voluntary long-term running in Ts65Dn mice. Euploid mice were processed in parallel for comparative purposes in some analyses. We found that running significantly changed the expression of 4,315 genes in the cerebellum of Ts65Dn mice, over five times more than in euploid animals, up-regulating 1,991 and down-regulating 2,324 genes. Functional analysis of these genes revealed a significant enrichment of 92 terms in the biological process category, including regulation of biosynthesis and metabolism, protein modification, phosphate metabolism, synaptic transmission, development, regulation of cell death/apoptosis, protein transport, development, neurogenesis and neuron differentiation. The KEGG pathway database identified 18 pathways that are up-regulated and two that are down-regulated by running that were associated with learning, memory, cell signaling, proteolysis, regeneration, cell cycle, proliferation, growth, migration, and survival. Of six mRNA protein products we tested by immunoblotting, four showed significant running-associated changes in their levels, the most prominent in glutaminergic receptor metabotropic 1, and two showed changes that were close to significant. Thus, unexpectedly, our data point to the high molecular plasticity of Ts65Dn mouse cerebellum, which translated into humans with DS, suggests that the motor deficits of individuals with DS could markedly benefit from prolonged exercise. PMID:26304719

  13. Fetal Alcohol Syndrome

    Science.gov (United States)

    ... Condiciones Chinese Conditions Fetal Alcohol Syndrome Read in Chinese What is Fetal Alcohol Syndrome (FAS)? Fetal Alcohol Syndrome (FAS) describes changes in a baby born to a mother whose pregnancy was complicated by alcohol consumption. A broader term ...

  14. Posterior leukoencephalopathy syndrome in poststretococcal acute glomerulonephritis

    International Nuclear Information System (INIS)

    Reversible posterior leukoencephalopathy (LEPR) is a clinical entity that affects radiation usually the white matter of the cerebral hemispheres. It is frequently associated with acute arterial hypertension and immunosuppressive therapy, among other causes. The clinical presentation is varied, with headache, nausea, vomiting, impaired consciousness and abnormal behavior, seizures and visual disturbances, symptoms that often regress. Computed tomography (CT) and magnetic resonance imaging (MRI) images show white matter edema predominantly in posterior regions of the brain. We present a 10 year old boy with leprosy in the course of a nephrotic syndrome secondary to acute diffuse glomerunefritis (GNDA) poststreptococcal. (author)

  15. [A study on protein metabolism in nephrotic patients treated with Chinese herbs].

    Science.gov (United States)

    Li, L; Yu, H; Pan, J

    1995-10-01

    It was found in our previous studies that two Chinese herbs Astragali and Angelica (A&A) together with high protein diet could ameliorate the lowering of serum albumin level and increase the synthesis rate of protein as shown by 15N-glicine tracer priming protein turnover study in nephrotic rats. Further experiment was designed to investigate the role of A&A and high protein intake in protein dynamic study and nitrogen balance in nephrotic patients. The level of serum total protein (STP), serum albumin (SA), urinary protein loss (UP), serum cholesterol (Cho) and index number of protein turnover and nitrogen balance in 7 patients were measured before and after treatment of 30 days with A&A. The results showed that after treatment the patients had significantly increased STA and SA (44.3 +/- 5.60 vs 49.7 +/- 6.80 P < 0.01; 22.6 +/- 0.42 vs. 29.4 +/- 7.40 P < 0.05), decreased UP and Cho (6.54 +/- 1.83 vs 4.63 +/- 1.33 P < 0.05; 9.69 +/- 2.31 vs. 7.82 +/- 1.95 P < 0.05) and increased net rates of total protein synthesis (1.06 +/- 0.03 vs 1.27 +/- 0.12 P < 0.05). It is concluded that A&A together with high protein intake could improve the disorder of protein metabolism and increase the level of serum protein by improving the net rate of protein synthesis in nephrotic patients. PMID:8731827

  16. Cell biology and genetics of minimal change disease [version 1; referees: 2 approved

    Directory of Open Access Journals (Sweden)

    Moin A. Saleem

    2016-03-01

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

  17. 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.

  18. Clinical significance of determination of changes of plasma ET-1 and CGRP contents in elderly males with metabolic syndrome

    International Nuclear Information System (INIS)

    Objective: To investigate the significance of changes of plasma endothelin-1 (ET-1) and calcitonin gene related peptide (CGRP) contents in elderly males with metabolic syndrome. Methods: Plasma ET-1 and CGRP contents were measured with RIA in 65 elderly males with hypertension and 65 elderly males with diabetes. The blood lipid and sugar contents were measured simultaneously. 35 controls entered this study. Results: The plasma ET-1 contents in elderly males with simple hypertension, diabetes and metabolic syndrome were all significantly higher than those in controls (P<0.01, P<0.05, P<0.05). Levels in hypertensives were significantly higher than those in diabetics (P<0.05). The plasma CGRP levels in the elderly males with hypertension and with metabolic syndrome were all significantly lower than those in controls (P<0.05, P<0.05). The CGRP levels in these subjects were significantly negatively correlated with the ET-1 levels (r= -0.75, P<0.01; r=-0.53, P<0.01). Conclusion: Changes of plasma ET-1 and CGRP levels in elderly males with metabolic syndrome were clinically significant, especially in the pathogenesis of hypertension. (authors)

  19. Interactive 3D visualization of structural changes in the brain of a person with corticobasal syndrome

    Directory of Open Access Journals (Sweden)

    Claudia Hänel

    2014-05-01

    Full Text Available The visualization of the progression of brain tissue loss, which occurs in neurodegenerative diseases like corticobasal syndrome (CBS, is an important prerequisite to understand the course and the causes of this neurodegenerative disorder. Common workflows for visual analysis are often based on single 2D sections since in 3D visualizations more internally situated structures may be occluded by structures near the surface. The reduction of dimensions from 3D to 2D allows for an holistic view onto internal and external structures, but results in a loss of spatial information. Here, we present an application with two 3D visualization designs to resolve these challenges. First, in addition to the volume changes, the semi-transparent anatomy is displayed with an anatomical section and cortical areas for spatial orientation. Second, the principle of importance-driven volume rendering is adapted to give an unrestricted line-of-sight to relevant structures by means of a frustum-like cutout. To strengthen the benefits of the 3D visualization, we decided to provide the application next to standard desktop environments in immersive virtual environments with stereoscopic viewing as well. This improves the depth perception in general and in particular for the second design. Thus, the application presented in this work allows for aneasily comprehensible visual analysis of the extent of brain degeneration and the corresponding affected regions.

  20. Cerebral glucose metabolism change in patients with complex regional pain syndrome. A PET study

    International Nuclear Information System (INIS)

    The aim of this study was to examine abnormalities of the central nervous system in patients with chronic pain who were diagnosed with complex regional pain syndrome (CRPS). Brain activity was assessed using 18F-fluorodeoxyglucose positron emission tomography. The data collected from 18 patients were compared with data obtained from 13 normal age-matched controls. Our results showed that glucose metabolism was bilaterally increased in the secondary somatosensory cortex, mid-anterior cingulated cortex (ACC) or posterior cingulated cortex (PCC) (or both), parietal cortex, posterior parietal cortex (PPC), and cerebellum as well as in the right posterior insula and right thalamus in our patients. In contrast, glucose metabolism was reduced contralaterally in the dorsal prefrontal cortex and primary motor cortex. Glucose metabolism was bilaterally elevated in the mid-ACC/PCC and the PPC, which correlated with pain duration. These data suggested that glucose metabolism in the brains of patients with CRPS changes dramatically at each location. In particular, glucose metabolism was increased in the areas concerned with somatosensory perception, possibly due to continuous painful stimulation. (author)

  1. Redox Imbalance and Morphological Changes in Skin Fibroblasts in Typical Rett Syndrome

    Directory of Open Access Journals (Sweden)

    Cinzia Signorini

    2014-01-01

    Full Text Available Evidence of oxidative stress has been reported in the blood of patients with Rett syndrome (RTT, a neurodevelopmental disorder mainly caused by mutations in the gene encoding the Methyl-CpG-binding protein 2. Little is known regarding the redox status in RTT cellular systems and its relationship with the morphological phenotype. In RTT patients (n = 16 we investigated four different oxidative stress markers, F2-Isoprostanes (F2-IsoPs, F4-Neuroprostanes (F4-NeuroPs, nonprotein bound iron (NPBI, and (4-HNE PAs, and glutathione in one of the most accessible cells, that is, skin fibroblasts, and searched for possible changes in cellular/intracellular structure and qualitative modifications of synthesized collagen. Significantly increased F4-NeuroPs (12-folds, F2-IsoPs (7.5-folds NPBI (2.3-folds, 4-HNE PAs (1.48-folds, and GSSG (1.44-folds were detected, with significantly decreased GSH (−43.6% and GSH/GSSG ratio (−3.05 folds. A marked dilation of the rough endoplasmic reticulum cisternae, associated with several cytoplasmic multilamellar bodies, was detectable in RTT fibroblasts. Colocalization of collagen I and collagen III, as well as the percentage of type I collagen as derived by semiquantitative immunofluorescence staining analyses, appears to be significantly reduced in RTT cells. Our findings indicate the presence of a redox imbalance and previously unrecognized morphological skin fibroblast abnormalities in RTT patients.

  2. Association of global weather changes with acute coronary syndromes: gaining insights from clinical trials data

    Science.gov (United States)

    Bakal, Jeffrey A.; Ezekowitz, Justin A.; Westerhout, Cynthia M.; Boersma, Eric; Armstrong, Paul W.

    2013-05-01

    The aim of this study was to develop a method for the identification of global weather parameters and patient characteristics associated with a type of heart attack in which there is a sudden partial blockage of a coronary artery. This type of heart attack does not demonstrate an elevation of the ST segment on an electrocardiogram and is defined as a non-ST elevation acute coronary syndrome (NSTE-ACS). Data from the Global Summary of the Day database was linked with the enrollment and baseline data for a phase III international clinical trial in NSTE-ACS in four 48-h time periods covering the week prior to the clinical event that prompted enrollment in the study. Meteorological events were determined by standardizing the weather data from enrollment dates against an empirical distribution from the month prior. These meteorological events were then linked to the patients' geographic region, demographics and comorbidities to identify potential susceptible populations. After standardization, changes in temperature and humidity demonstrated an association with the enrollment event. Additionally there appeared to be an association with gender, region and a history of stroke. This methodology may provide a useful global insight into assessing the biometeorologic component of diseases from international data.

  3. Histopathologic changes of hypothalamus and pituitary in a rat model of polycystic ovary syndrome

    Institute of Scientific and Technical Information of China (English)

    Jiang Yan; Meng Fan-yu; Hu Zhen-hua; Liu Fang

    2010-01-01

    Objective: To investigate the histopathologic changes of hypothalamus and pituitary in a rat model of polycystic ovary syndrome (PCOS).Methods: Rat model of PCOS was established in 6 immature female SD rats and another 6 immature rats treated with placebo were as control. The tissues of hypothalamus and pituitary were obtained and observed by light microscope and transmission electron microscope.Results: Light microscopy revealed little difference in morphology of neurons in arcuate nucleus or basophilic cells in pituitary between PCOS rats and normal rats. Electron microscopic examination showed that, compared with those in normal rats, GnRH neurons in PCOS rats were larger and fuller, with Golgi complex and mitochondria increased. The mitochondria were small, round and swelling. More high-density secretory granules and bright vesicles were observed in the cytoplasm. The Golgi complex near nucleus in pituitary gonadotropin cell in PCOS rats was fractured and expanded, and there were increased number mitochondria and different sizes of the higher electron density secretory granules in the cytoplasm. Conclusion: The morphological alterations in hypothalamus and pituitary could play a very important role in the development of PCOS.

  4. Looking for sufficient change: Evaluation of counsellor training for STI syndromic management in India.

    Science.gov (United States)

    Vaz, Melita; Kadyan, Nisha; Chalil, Sumitha; Prasad, Turlapati L N; Singh, Aman Kumar

    2016-10-01

    In India, public health care of Sexually Transmitted Infections is delivered through Designated STI/RTI Clinics (DSRCs) using syndromic management. This paper describes efforts, over three years, to improve in-service training for counsellors positioned at DSRCs-using a data approach. The programme managers realised, through rigorous monitoring of initial induction training reports that, while knowledge and attitudes of most trainees had improved as evident from t-tests, at least one-quarter scored worse on post-training assessments (n=859). Therefore, they undertook a survey using a competency approach to diagnose what critical competencies are influenced through training: counselling skills, risk reduction suggestions, labelling male and female anatomy, record-keeping and STI patient education (n=132). Survey results demonstrated that trainees failed to pass a two-thirds cutoff score in most competencies. These findings led the programme managers to modify training and implement tighter quality measures. In the second round of training - refresher training - outcomes on competency assessments before and after training showed more acceptable performance (n=833). The paper describes how programme managers, after an acceptance of such initial short-comings, developed customized assessments when literature provided limited guidance and how they worked to achieve change that was acceptable for programme needs. PMID:27372031

  5. Infection and T lymphocyte subpopulations: changes associated with bacteremia and the acquired immunodeficiency syndrome.

    Science.gov (United States)

    Fishman, J A; Martell, K M; Rubin, R H

    1983-01-01

    Patients with bacteremia, bacterial endocarditis, or acquired immunodeficiency syndrome (AIDS) were prospectively studied using monoclonal antibody reagents to assess alterations in T-lymphocyte subpopulations. Patients with endocarditis had significantly higher ratios of T-helper (OKT4+) to T-suppressor-cytotoxic (OKT8+) cells than did patients with bacteremia alone. Staphylococcus aureus endocarditis patients had a mean ratio of 8.49 (range 4.73-22.36) while S aureus bacteremia had a mean ratio of 2.75 (range 2.15 to 3.21). Similar results were found with Staphylococcus epidermidis endocarditis (mean 1.62) and bacteremia (mean 1.23). Klebsiella pneumoniae endocarditis (5.10) and sepsis (4.32), and E coli bacteremia (2.15). Nine male patients with AIDS had markedly depressed ratios (mean 0.25, range 0.04 to 0.67) while eight male homosexuals with unexplained lymphadenopathy ("pre-AIDS") had normal or increased ratios. Bacteremic infections are associated with an increased OKT4+/OKT8+ ratio with the degree of increase dependent upon virulence, location, and duration of infection. The immunomodulating effects of infection are manifested in changes in T-cell subsets, and these measurements can be useful in clinical management. PMID:6094086

  6. Changes of Indocyanine Green and Fluorescein Angiography in Multiple Evanescent White-dot Syndrome: A Case Report

    Institute of Scientific and Technical Information of China (English)

    Changxian Yi; Guilin Zhao; Jiexiong Ou; Hong Yan

    2003-01-01

    Purpose: To study the clinicopathological change on "Multiple Evanescent White-dot Syndrome (MEWDS)"through fundus angiogrphy analyses.Methods:Examing the case with fluorescein angiography (FFA) and indocyanine green angiography(ICGA), visual field and following up for 15 months.Results :There exist multiple white dots in the fundus photo and angiography. The changes on ICGA maintain longer than fundus photo and FFA. Papille may be edematous and macula could present with special hypofluorescent flecks.Conclusions:MEWDS has specific angiographic features. Its pathological changes involve deep retinal pigment epithelium(RPE) as well as choroidal capillaries. The prognosis of the visual acuity for MEWDS is very good.

  7. Changes in Enteric Neurons of Small Intestine in a Rat Model of Irritable Bowel Syndrome with Diarrhea

    OpenAIRE

    Li, Shan; Fei, Guijun; Fang, Xiucai; Yang, Xilin; Sun, Xiaohong; Qian, Jiaming; Wood, Jackie D.; Ke, Meiyun

    2016-01-01

    Background/Aims Physical and/or emotional stresses are important factors in the exacerbation of symptoms in irritable bowel syndrome (IBS). Several lines of evidence support that a major impact of stress on the gastrointestinal tract occurs via the enteric nervous system. We aimed to evaluate histological changes in the submucosal plexus (SMP) and myenteric plexus (MP) of the distal ileum in concert with the intestinal motor function in a rat model of IBS with diarrhea. Methods The rat model ...

  8. From armchair to wheelchair: How patients with a locked-in syndrome integrate bodily changes in experienced identity.

    OpenAIRE

    Nizzi, M. C.; Demertzi, Athina; Gosseries, Olivia; Bruno, Marie-Aurélie; Jouen, F.; Laureys, Steven

    2011-01-01

    Different sort of people are interested in personal identity. Philosophers frequently ask what it takes to remain oneself. Caregivers imagine their patients' experience. But both philosophers and caregivers think from the armchair: they can only make assumptions about what it would be like to wake up with massive bodily changes. Patients with a locked-in syndrome (LIS) suffer a full body paralysis without cognitive impairment. They can tell us what it is like. Forty-four chronic LIS patients ...

  9. Changes in androgens and insulin sensitivity indexes throughout pregnancy in women with polycystic ovary syndrome (PCOS): relationships with adverse outcomes

    OpenAIRE

    Falbo Angela; Rocca Morena; Russo Tiziana; D'Ettore Antonietta; Tolino Achille; Zullo Fulvio; Orio Francesco; Palomba Stefano

    2010-01-01

    Abstract Background Given the high rate of pregnancy and perinatal complications recently observed in patients with polycystic ovary syndrome (PCOS) and the lack of data on the serum variations in androgens and insulin sensitivity indexes in pregnant women with PCOS, the current study was aimed to assess these changes and their potential effect on pregnancy outcomes in a population of women with PCOS. Methods Forty-five pregnant patients with ovulatory PCOS (PCOS group) and other 42 healthy p...

  10. 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...

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

    OpenAIRE

    Katherine J. Coffey; Adam C. Knight; Benjamin Wax

    2015-01-01

    The purpose of this study was to examine the effects of equine assisted therapy on selected gait parameters in a person with Down syndrome. One female participant with Down syndrome completed two therapeutic horseback riding programs, each consisting of six riding sessions. Specific gait characteristics were analyzed with a trend analysis of the data by examining the means of the different variables. The trend analysis revealed a difference in stride length as well as hip and knee angle. Thes...

  12. Change of International Restless Legs Syndrome Study Group Rating Scale subscales with treatment and placebo: a pilot study

    Directory of Open Access Journals (Sweden)

    Mitchell UH

    2014-02-01

    Full Text Available Ulrike H Mitchell,1 Sterling C Hilton2 1Brigham Young University, Department of Exercise Sciences, 2Department of Educational Leadership and Foundations, Provo, UT, USA Background: In 2003, the 10-question International Restless Legs Syndrome Study Group Rating Scale (IRLS was developed as a means of assessing the severity of restless legs syndrome. Two subscales were identified: symptom severity (SS 1 and symptom impact (SS 2. Only one study has investigated the subscales' responsiveness to a 12-week treatment with ropinirole. This current study was undertaken to assess the impact of a 4-week, non-pharmaceutical treatment on the two subscales and to explore whether or not both subscales were impacted by the observed placebo effect. Methods: The pooled data from questionnaires of 58 patients (41 from both treatment groups and 17 from the sham treatment control group, who participated in two clinical studies, were reviewed. Their change in score over a 4-week trial was computed. The average change in both subscales in both groups was computed and t-tests were performed. Results: In the treatment group, the average scores of both subscales changed significantly from baseline to week 4 (P<0.005 for both. Compared to the control, SS 1 changed (P<0.001, but not SS 2 (P=0.18. In the sham treatment group, the scores for SS 1 changed significantly (P=0.002, but not for SS 2 (P=0.2. Conclusion: This study corroborated findings from an earlier study in which both subscales changed with a 12-week drug treatment. It also showed that the observed placebo effect is attributed to a small but significant change in symptom severity, but not symptom impact. Keywords: restless legs syndrome, RLS severity scale, IRLS subscales, symptom impact, symptom severity

  13. Age-dependent change in behavioral feature in Rubinstein-Taybi syndrome.

    Science.gov (United States)

    Yagihashi, Tatsuhiko; Kosaki, Kenjiro; Okamoto, Nobuhiko; Mizuno, Seiji; Kurosawa, Kenji; Takahashi, Takao; Sato, Yuji; Kosaki, Rika

    2012-06-01

    Rubinstein-Taybi syndrome (RTS) is characterized by developmental delay, postnatal growth retardation, typical facial appearance, and broad thumbs and big toes. The behavioral phenotype of children with RTS has been described as friendly and having good social contacts; however, a short attention span and hyperactivity are sometimes present. Little attention has been paid to the behavioral aspects of adults with RTS. We conducted an observational study focusing on behavioral problems in adolescents and adults with RTS compared with children with RTS. A total of 63 patients with RTS and their caretakers answered self-administered questionnaires regarding behavioral features including the Child Behavior Checklist (CBCL). High total CBCL scores were observed, and the mean score was beyond the clinical cut-off point. After stratification into two groups according to age, the older group (≥14 years) displayed statistically significant higher scores for Anxious/Depression (P = 0.002) and Aggressive Behavior (P = 0.036) than the younger group (≤13 years). In analyses of single items, statistically significant differences between the younger group and the older group were found for 'Nervous, high-strung, or tense' (31.3% vs 67.7%, P = 0.004) and 'Too fearful or anxious' (37.5% vs 64.5%, P = 0.032). Here, we showed that the specific behavioral phenotypes of RTS change during adolescence, with anxiety, mood instability, and aggressive behavior emerging as patients age. A clear need exists to follow-up patients with RTS to catch the eventual emergence of psychiatric problems with age. If necessary, pharmacological treatment should be considered. PMID:22639993

  14. NADPH oxidase 4 attenuates cerebral artery changes during the progression of Marfan syndrome.

    Science.gov (United States)

    Onetti, Yara; Meirelles, Thayna; Dantas, Ana P; Schröder, Katrin; Vila, Elisabet; Egea, Gustavo; Jiménez-Altayó, Francesc

    2016-05-01

    Marfan syndrome (MFS) is a connective tissue disorder that is often associated with the fibrillin-1 (Fbn1) gene mutation and characterized by cardiovascular alterations, predominantly ascending aortic aneurysms. Although neurovascular complications are uncommon in MFS, the improvement in Marfan patients' life expectancy is revealing other secondary alterations, potentially including neurovascular disorders. However, little is known about small-vessel pathophysiology in MFS. MFS is associated with hyperactivated transforming growth factor (TGF)-β signaling, which among numerous other downstream effectors, induces the NADPH oxidase 4 (Nox4) isoform of NADPH oxidase, a strong enzymatic source of H2O2 We hypothesized that MFS induces middle cerebral artery (MCA) alterations and that Nox4 contributes to them. MCA properties from 3-, 6-, or 9-mo-old Marfan (Fbn1(C1039G/+)) mice were compared with those from age/sex-matched wild-type littermates. At 6 mo, Marfan compared with wild-type mice developed higher MCA wall/lumen (wild-type: 0.081 ± 0.004; Marfan: 0.093 ± 0.002; 60 mmHg; P Marfan mice with Nox4 deficiency (Nox4(-/-)). Strikingly, Nox4 deletion in Marfan mice aggravated MCA wall thickening (cross-sectional area; Marfan: 6,660 ± 363 μm(2); Marfan Nox4(-/-): 8,795 ± 824 μm(2); 60 mmHg; P < 0.05), accompanied by decreased TGF-β expression and increased collagen deposition and Nox1 expression. These findings provide the first evidence that Nox4 mitigates cerebral artery structural changes in a murine model of MFS. PMID:26945079

  15. Morphological changes of carotid bodies in acute respiratory distress syndrome: a morphometric study in humans

    Directory of Open Access Journals (Sweden)

    Vinhaes E.N.G.

    2002-01-01

    Full Text Available Carotid bodies are chemoreceptors sensitive to a fall of partial oxygen pressure in blood (hypoxia. The morphological alterations of these organs in patients with chronic obstructive pulmonary disease (COPD and in people living at high altitude are well known. However, it is not known whether the histological profile of human carotid bodies is changed in acute clinical conditions such as acute respiratory distress syndrome (ARDS. The objective of the present study was to perform a quantitative analysis of the histology of carotid bodies collected from patients who died of ARDS. A morphometric study of carotid bodies collected during routine autopsies was carried out on three groups: patients that died of non-respiratory diseases (controls, N = 8, patients that presented COPD and died of its complications or associated diseases (N = 7, and patients that died of ARDS (N = 7. Morphometric measurements of the volume fraction of clusters of chief cells were performed in five fields on each slide at 40X magnification. The numerical proportion of the four main histological cell types (light, dark, progenitor and sustentacular cells was determined analyzing 10 fields on each slide at 400X magnification. The proportion of dark cells was 0.22 in ARDS patients, 0.12 in controls (P<0.001, and 0.08 in the COPD group. The proportion of light cells was 0.33 (ARDS, 0.44 (controls (P<0.001, and 0.36 (COPD. These findings suggest that chronic and acute hypoxia have different effects on the histology of glomic tissue.

  16. Changes in lung parenchyma after acute respiratory distress syndrome (ARDS): assessment with high-resolution computed tomography

    International Nuclear Information System (INIS)

    The aim of this study was to evaluate the appearance, extent, and distribution of parenchymal changes in the lung after acute respiratory distress syndrome (ARDS) as a function of disease severity and therapeutic procedures. High-resolution computed tomography (HRCT), clinical examination, and lung function tests were performed in 15 patients, 6-10 months after ARDS. The appearance and extent of parenchymal changes were compared with the severity of ARDS, as well as with clinical and therapeutic data. Lung parenchymal changes resembling those found in the presence of pulmonary fibrosis were observed in 13 of 15 patients (87%). The changes were significantly more frequent and more pronounced in the ventral than in the dorsal portions of the lung (p<0.01). A significant correlation was observed between the extent of lung alterations and the severity of ARDS (p<0.01), and the duration in which patients had received mechanical ventilation either with a peak inspiratory pressure greater than 30 mmHg (p<0.05), or with more than 70% oxygen (p<0.01). Acute respiratory distress syndrome frequently is followed by fibrotic changes in lung parenchyma. The predominantly ventral distribution of these changes indicates that they may be caused by the ventilation regimen and the oxygen therapy rather than by the ARDS. (orig.)

  17. Sensory changes,C-and A-fiber function,and shoulder-hand syndrome in hemiplegic patients after stroke

    Institute of Scientific and Technical Information of China (English)

    Yi Yuan; Xiaohong Zi; Xian Huang

    2008-01-01

    BACKGROUND:Clinical diagnosis of various neurological disorders involving the sensory nerves depends primarily on subjective description.which cannot be quantitatively evaluated,and is also less reproducible and specific.Quantitative sensory testing mcthods can overcome these shortcomings and is currently used to identify the function of the C-and A-fibers.OBJECTIVE:To apply the quantitative sensory testing method for analyzing changes in temperature sensation,cryalgesia,thermalgesia,and vibration sense on the skin surface of hemiplegic patients with post-stroke shoulder-hand syndrome,and to analyze the relationship between these changes and shoulder-hand syndrome.DESIGN,TIME AND SETTING:A non-randomized,concurrent,control study was performed at the Clinic and Inpatient Department of the Third Xiangya Hospital,Central South University,between June 2000 and April 2001.PARTICIPANTS:Thirty post-stroke,hemiplegic patients were divided into shoulder-hand syndrome and control groups,according to whether patients exhibited shoulder-hand syndrome,with 15 patients in each group.METHODS:A TSA2001 quantitative sensory testing device(Medoc,Israel)was used for quantitative sensory testing.All sensory testing employed limits,testing temperature sense on the palm thenar eminence and vibration sense on the thumb metacarpal.Cold threshold was≤28℃.warmth threshold was≥36℃,cold-evoked pain threshold was≤5℃.heat-evoked pain threshold was≥51℃,vibration threshold was≥5 μ m/s;if a patient met one of these items,he/she was considered to be hypoanesthesia.MAIN OUTCOME MEASURES:Cold,warm,cold-evoked pain,heat-evoked pain and vibration threshold changes on skin from the paralyzed upper extremity was measured in the shoulder-hand syndrome and control groups.RESULTS:Incidence of sensory disability in the shoulder-hand syndrome group increased more significantly than in the control group(P<0.05),with the primary manifestations being decreased cold threshold(P<0.05)and

  18. CLINICO-PATHOLOGY AND ULTRASTRUCTURAL ETUDY OF NEPHROPATHY CHANGES DUE TO LUPUS ERYTHEMATOSIS DISSEMINATUS

    Directory of Open Access Journals (Sweden)

    A. Modjtabai

    1969-01-01

    Full Text Available Nephrotic syndrome _ Nephrotic syndrome is found in 30% of all of the cases of systematic LE. Nephropathy Changes Due to Lupus Erythematosus 21 (15,16 in peripheral blood may confirm definitively lupus cryhcrornatosus. Additional serological evidence such as hyper g-gJobu":nemia, and 19_s-g_ globulin may then positively confirm the (17. Supporting evidence of 11 specific type of glomerular substantiates prior clinical and paracIinical evidence: The foregoing ultrasnucturat chnracteristtcs are then seen to represent an additional check of routine diagnostic procedures In addition observed permits an opportunity to• correlate• min~te changes with known biochemical changes previosly A. Modjtabai et at Diagnostically the presence of kidney disease and the presence of L.E. Urine albumin _ The appearance of urine albumin to the extent of 8-10 mgm.mil is a sign of kidney damage. This may be accompanied by acute kidney inadequacy, cyrtitis with fever and pain. There may be a varying globulin excretion which is taken by some as a precursor of ensuing damage. This point, of course, has been debated. When clinical and paraclinical signs indicate LE. definitive conclu, sions may be. reached utilizing electron microscopy. The disease occurs more frequently in women than in men and especially among young people. More than one person in a family may bevaffectcd leading to the assumption that the trait is inherited. The hereditary nature of the disease, however, has not been definitely estab., lishcd. Basically the disease appears to original as an abnormal imrnu., nological reaction to external or internal causes or perhaps even auto.; immunological. Causative or aggravating factors may be long exposure to sunlight, and ultraviolet rays which may free lysozyrnes or other proteolytic enzymes which attack the ground substance of the cell membranes of the endothelium. Drugs such as penicillin, sulfonamide and hydralyzin have caused allergic reactions

  19. Activity-Dependent Changes in MAPK Activation in the Angelman Syndrome Mouse Model

    Science.gov (United States)

    Filonova, Irina; Trotter, Justin H.; Banko, Jessica L.; Weeber, Edwin J.

    2014-01-01

    Angelman Syndrome (AS) is a devastating neurological disorder caused by disruption of the maternal "UBE3A" gene. Ube3a protein is identified as an E3 ubiquitin ligase that shows neuron-specific imprinting. Despite extensive research evaluating the localization and basal expression profiles of Ube3a in mouse models, the molecular…

  20. Structural Changes in the Somatosensory System Correlate with Tic Severity in Gilles de la Tourette Syndrome

    Science.gov (United States)

    Thomalla, Gotz; Siebner, Hartwig R.; Jonas, Melanie; Baumer, Tobias; Biermann-Ruben, Katja; Hummel, Friedhelm; Gerloff, Christian; Muller-Vahl, Kirsten; Schnitzler, Alfons; Orth, Michael; Munchau, Alexander

    2009-01-01

    Gilles de la Tourette syndrome (GTS) is a neuropsychiatric disorder characterized by multiple motor and vocal tics. Previous structural MRI studies have identified regional abnormalities in grey matter, especially in the basal ganglia. These findings are consistent with the assumption of alterations in cortico-striato-thalamo-cortical circuits and…

  1. Pilot observational study on haemodynamic changes after surfactant administration in preterm newborns with respiratory distress syndrome

    OpenAIRE

    Vitali, Francesca; Galletti, Silvia; Aceti, Arianna; Aquilano, Giulia; Fabi, Marianna; Balducci, Anna; Faldella, Giacomo

    2014-01-01

    Background Surfactant treatment reduces respiratory morbidity and mortality in preterm infants. Data on its haemodynamic consequences are conflicting. The aim was to characterise the haemodynamic effects of surfactant treatment on cardiac function in preterm newborns with respiratory distress syndrome (RDS). Methods Preterm infants (gestational age

  2. Temporal changes in serum creatine kinase concentration and degree of muscle rigidity in 24 patients with neuroleptic malignant syndrome

    Directory of Open Access Journals (Sweden)

    Nisijima K

    2013-06-01

    Full Text Available Koichi Nisijima, Katutoshi ShiodaDepartment of Psychiatry, Jichi Medical University, Tochigi, JapanAbstract: Neuroleptic malignant syndrome (NMS is a dangerous adverse response to antipsychotic drugs. It is characterized by the four major clinical symptoms of hyperthermia, severe muscle rigidity, autonomic dysfunction, and altered mental state. Serum creatine kinase (CK elevation occurs in over 90% of NMS cases. In the present study, the detailed temporal changes in serum CK and degree of muscle rigidity, and the relationship between CK concentration and degree of muscle rigidity over the time course from fever onset, were evaluated in 24 affected patients. The results showed that serum CK peaked on day 2 after onset of fever and returned to within normal limits at day 12. Mild muscle rigidity was observed before the onset of fever in 17 of 24 cases (71%. Muscle rigidity was gradually exacerbated and worsened until day 4 after onset of fever. These findings confirm physicians' empirical understanding of serum CK concentrations and muscle rigidity in NMS based on data accumulated from numerous patients with the syndrome, and they indicate that serum CK may contribute to the early detection of NMS.Keywords: neuroleptic malignant syndrome, creatine kinase, muscle rigidity

  3. Marfan Syndrome

    Science.gov (United States)

    ... caved-in look. He also wore glasses for myopia (say: my-OH-pee-uh), or nearsightedness, which ... syndrome, this "glue" is weaker than normal. This causes changes in many systems of the body, but ...

  4. Neuropeptide deficient mice have attenuated nociceptive, vascular, and inflammatory changes in a tibia fracture model of complex regional pain syndrome

    OpenAIRE

    Guo Tian-Zhi; Wei Tzuping; Shi Xiaoyou; Li Wen-Wu; Hou Saiyun; Wang Liping; Tsujikawa Kazutake; Rice Kenner C; Cheng Kejun; Clark David J; Kingery Wade S

    2012-01-01

    Abstract Background Distal limb fracture in man can induce a complex regional pain syndrome (CRPS) with pain, warmth, edema, and cutaneous inflammation. In the present study substance P (SP, Tac1−/−) and CGRP receptor (RAMP1−/−) deficient mice were used to investigate the contribution of neuropeptide signaling to CRPS-like changes in a tibia fracture mouse model. Wildtype, Tac1−/−, and RAMP1−/− mice underwent tibia fracture and casting for 3 weeks, then the cast was removed and hindpaw mechan...

  5. Metformin Lowers Serum Cobalamin without Changing Other Markers of Cobalamin Status: A Study on Women with Polycystic Ovary Syndrome

    OpenAIRE

    Ebba Nexo; Lauszus, Finn F.; Bor, Mustafa V.; Birgitta Trolle; Eva Greibe

    2013-01-01

    Treatment with the anti-diabetic drug metformin is followed by a decline in plasma cobalamin, but it is unsettled whether this denotes an impaired cobalamin status. This study has explored changes in the markers of cobalamin status in women with Polycystic Ovary Syndrome treated with metformin (1.5-2.5 g per day) (n = 29) or placebo (n = 23) for six months. Serum samples were collected before and after two, four, and six months of treatment. We found serum cobalamin to decline and reach signi...

  6. Analysis of clinic and osteal change in Turner syndrome (report of 15 cases)

    International Nuclear Information System (INIS)

    Objective: To research the clinical and osteal radiology imaging features in Turner's syndrome. Methods: Analyzed the clinical and osteal radiology imaging features of 15 Turner's syndrome. Results: 15 patients were Karyotyped into 3 groups I, 45, X, n=8; II, 46, XX, n=5; III, 46, XX/45, X, n = 2. Radiology imaging features: osteo-porosis, n=14; metacarpal sign, n=8; phahanx, n=9; cubitus valgus, n=10; vertebra steodystrophia, n=5; brachy-dactylya, n=9; basilar impression malformation, n=11. Conclusion: The terminal heights of Turner' s patients were marked lower than normal growth, hormone was deficiency, learning ability declined and the sex gland was hypoplastic. The typical imaging feature do main reference value to diagnosis. (authors)

  7. Interactive 3D visualization of structural changes in the brain of a person with corticobasal syndrome

    OpenAIRE

    Claudia Hänel; Peter Pieperhoff; Katrin Amunts

    2014-01-01

    The visualization of the progression of brain tissue loss in neurodegenerative diseases like corticobasal syndrome (CBS) can provide not only information about the localization and distribution of the volume loss, but also helps to understand the course and the causes of this neurodegenerative disorder. The visualization of such medical imaging data is often based on 2D sections, because they show both internal and external structures in one image. Spatial information, however, is lost. 3D vi...

  8. Reproductive and biochemical changes in obese and non obese polycystic ovary syndrome women

    OpenAIRE

    Manal Ibrahim Mahmoud; Fawzia Habeeb; Khaled Kasim

    2015-01-01

    Background: Reproductive, clinical and laboratory implication varies in polycystic ovary syndrome (PCOS) according to body weight. Objective: To compare reproductive, clinical and laboratory data between obese and non obese women with PCOS. Methods: A cohort of 180 women with PCOS who attended outpatient clinic of Taibah University from January to September 2012 was included. Studied women were classified according to body mass index (BMI) into overweight/obese (BMI >25 kg/m2) and norma...

  9. The effect of changing attitudes to Down's syndrome in the management of complete atrioventricular septal defects

    OpenAIRE

    Amark, K.; Sunnegardh, J

    1999-01-01

    OBJECTIVES—To describe the evaluation, decision making, and care of children with a complete atrioventricular septal defect (CAVSD).
STUDY DESIGN—Retrospective study of 136 consecutive cases from 1970 to 1996.
RESULTS—A total of 115 (85%) children had Down's syndrome. Denial of surgery without obvious medical reasons was more common in the early years, as was parental refusal of offered surgery and institutional care of the children. Improved results in later years encour...

  10. Change in physical mobility over 10 years in post-polio syndrome.

    Science.gov (United States)

    Bickerstaffe, A; Beelen, A; Nollet, F

    2015-03-01

    Post-polio syndrome is characterised by progressive muscle weakness and other symptoms which can limit physical mobility. We assessed the rate of decline in mobility over 10 years in relation to strength decline; and investigated potential predictors for the rate of decline of walking capacity, a measure of mobility, in 48 patients with post-polio syndrome and proven quadriceps dysfunction at baseline. Average walking capacity and self-reported physical mobility declined over 10 years, by 6 and 14%, respectively. Concomitantly people lost an average of 15% of isometric quadriceps strength. Significantly more people used walking aids offering greater support at follow-up. Notably, there was much individual variation, with 18% of participants losing a substantial amount of walking capacity (27% decline) and concomitant self-reported physical mobility (38% decline). Loss of quadriceps strength only explained a small proportion of the variance of the decline in walking capacity (R = 11%) and the rate of decline could not be predicted from baseline values for strength, walking capacity, self-reported physical mobility or basic demographics. The individual variability, yet lack of predictive factors, underscores the need for personally tailored care based on actual functional decline in patients with post-polio syndrome. PMID:25613808

  11. Bone malformations in Proteus syndrome: an analysis of bone structural changes and their evolution during growth

    International Nuclear Information System (INIS)

    The radiographic follow-up of a patient with Proteus syndrome is presented. Review of radiographs obtained at 3 years 10 months, 10 years, and 17 years 8 months indicated that the rate of growth in length of the oversized tubular bones of the hands was similar to that of the normal bones of the same hand. This observation supports the view that the primary lesion occurs in the early embryonic period, when the limb bud mesenchyme cells condense and cartilage differentiates producing oversized cartilage anlages, rather than being a defect of bone cell-mediated apposition and modelling processes of bone. Additional radiographs of the pelvis and spine were obtained at age 4 years 10 months and head CT at 8 years 10 months. This pathogenetic mechanism fits well with the hypothesis of somatic mosaicism, which is at present the most credible explanation for the aetiology of Proteus syndrome. Other skeletal malformations recognized as typical of the syndrome can be interpreted as secondary adaptations to the altered mechanical conditions induced by overgrowth of bones. (orig.)

  12. Radiological assessment of skull base changes in children with syndromic craniosynostosis: role of ''minor'' sutures

    Energy Technology Data Exchange (ETDEWEB)

    Calandrelli, Rosalinda; D' Apolito, Gabriella; Gaudino, Simona; Stefanetti, Mariangela; Colosimo, Cesare [Universita Cattolica Sacro Cuore, Institute of Radiology, Rome (Italy); Massimi, Luca; Di Rocco, Concezio [Universita Cattolica Sacro Cuore, Institute of Neurosurgery, Rome (Italy)

    2014-10-15

    This study aims to identify the premature synostosis of ''major'' and ''minor'' sutures of the four ''sutural arches'' of the skull and to perform a morphometric analysis in children with syndromic craniosynostosis in order to evaluate changes in the skull base linked with premature suture synostosis. We reviewed multiplanar high-resolution CT images, implemented with 3D reconstructions, from 18 patients with complex syndromic craniosynostosis and compared them with 18 age-matched healthy subjects. We assessed the calvarial sutures and their extension to the skull base, and then we correlated specific types of synostosis with the size, shape and symmetry of the cranial fossae. We found a marked asymmetry of the skull base growth in all patients. The synostotic involvement around the coronal ring caused a reduction in the growth of the anterior and middle fossae. The size of the posterior cranial fossa was related not only to ''major'' but also to ''minor'' suture synostosis of the lambdoid and parieto-squamosal arches. Changes in the skull base and craniofacial axis symmetry are due to structural and functional relationships between ''major'' and ''minor'' skull sutures, suggesting a structural and functional relationship between the neurocranium and basicranium. The early recognition of prematurely closed skull base sutures may help clinicians and neurosurgeons to establish correct therapeutic approaches. (orig.)

  13. Clinical significance of changes of plasma ADM, ET-1 and GMP-140 levels in patients with acute coronary syndrome (ACS)

    International Nuclear Information System (INIS)

    Objective: To investigate the clinical significance of changes of plasma levels of adrenomedullin (ADM), ET-1 and α-granule membrane protein (GMP-140) levels in the pathogenesis of acute coronary syndrome (ACS). Methods: Plasma ADM, ET-1 and GMP-140 levels were measured with RIA in (1) 31 cases of ACS with unstable angina pectoris (UAP) or non-ST segment elevation myocardial infarction (NSTEMI), (2) 32 cases of ACS with ST segment elevation myocardial infarction (STEMI), (3) 31 patients with stable angina pectoris (SAP) and, (4) 33 controls. Results: The plasma ADM, ET-1 and GMP-140 levels in both patient groups with UAP/NSTEMI and STEMI were significantly higher than those in controls (P0.05). However, the plasma levels of ADM, ET-1 and GMP-140 in the three groups of patients (2 groups of ACS and 1 group of SAP) were significantly different from each other (P< 0.01). Plasma levels of ADM, ET-1 and GMP-140 were mutually positively correlated. Conclusion: Changes of plasma levels of ADM, ET-1 and GMP-140 might reflect the severity of acute coronary syndrome and might serve as a marker for instability of the atheromatous plaques. (authors)

  14. Neprhrotic syndrome in a patient with rheumatoid arthritis treated with adalimumab: a case report

    Directory of Open Access Journals (Sweden)

    M.G. Portuesi

    2011-09-01

    Full Text Available The pathogenetic role of TNFα in inflammatory diseases has been known for a long time and has modified the therapeutic approach towards this pathology. All over the word, about 400.000 patients have been treated with biological anti-TNFα drugs. Particular attention has been taken for the safety of their use. Let us describe a case of 60 year old man affected by rheumatoid arthritis who has developed nephrotic syndrome with histological pattern typical of systemic lupus erythematosus-like syndrome, without autoantibodies.

  15. From armchair to wheelchair: how patients with a locked-in syndrome integrate bodily changes in experienced identity.

    Science.gov (United States)

    Nizzi, Marie-Christine; Demertzi, Athena; Gosseries, Olivia; Bruno, Marie-Aurélie; Jouen, François; Laureys, Steven

    2012-03-01

    Different sort of people are interested in personal identity. Philosophers frequently ask what it takes to remain oneself. Caregivers imagine their patients' experience. But both philosophers and caregivers think from the armchair: they can only make assumptions about what it would be like to wake up with massive bodily changes. Patients with a locked-in syndrome (LIS) suffer a full body paralysis without cognitive impairment. They can tell us what it is like. Forty-four chronic LIS patients and 20 age-matched healthy medical professionals answered a 15-items questionnaire targeting: (A) global evaluation of identity, (B) body representation and (C) experienced meaning in life. In patients, self-reported identity was correlated with B and C. Patients differed with controls in C. These results suggest that the paralyzed body remains a strong component of patients' experienced identity, that patients can adjust to objectives changes perceived as meaningful and that caregivers fail in predicting patients' experience. PMID:22100276

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

    Institute of Scientific and Technical Information of China (English)

    1991-01-01

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

  17. Age-Related Changes of Adaptive and Neuropsychological Features in Persons with Down Syndrome

    OpenAIRE

    Ghezzo, Alessandro; Salvioli, Stefano; Solimando, Maria Caterina; Palmieri, Alice; Chiostergi, Chiara; Scurti, Maria; Lomartire, Laura; Bedetti, Federica; Cocchi, Guido; Follo, Daniela; Pipitone, Emanuela; Rovatti, Paolo; Zamberletti, Jessica; Gomiero, Tiziano; Castellani, Gastone

    2014-01-01

    Down Syndrome (DS) is characterised by premature aging and an accelerated decline of cognitive functions in the vast majority of cases. As the life expectancy of DS persons is rapidly increasing, this decline is becoming a dramatic health problem. The aim of this study was to thoroughly evaluate a group of 67 non-demented persons with DS of different ages (11 to 66 years), from a neuropsychological, neuropsychiatric and psychomotor point of view in order to evaluate in a cross-sectional study...

  18. Clinical significance of changes of serum hs-CRP, IL-6 and TNF-α levels after treatment in patients with polycystic ovary syndrome

    International Nuclear Information System (INIS)

    Objective: To explore the clinical significance of changes of serum hs-CRP, IL-6 and TNF-α levels in patients with polycystic ovary syndrome. Methods: Serum hs-CRP (with immuno turbidity method), IL-6, TNF-α (with RIA) levels were determined in 31 patients with polycystic ovary syndrome both before and after six, month's treatment as well as 35 controls. Results: Before treatment, the serum hs-CRP, IL-6 and TNF-α levels in the 31 patients with polycystic ovary syndrome were significantly higher than those in controls (P0.05). Serum hs-CRP levels were positive correlate with serum IL-6, TNF-α levels (r=0.6014, 0.5982, P<0.01). Conclusion: Serum hs-CRP, IL-6 and TNF-α levels were correlated to the development of polycystic ovary syndrome (PCOS). (authors)

  19. 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

  20. The Effect of Changing Serum 25-Hydroxyvitamin D Concentrations on Metabolic Syndrome: A Longitudinal Analysis of Participants of a Preventive Health Program

    Directory of Open Access Journals (Sweden)

    Truong-Minh Pham

    2015-08-01

    Full Text Available Several studies have shown that a poor vitamin D status may increase the risk of developing metabolic syndrome, which leaves the question whether improving one’s vitamin D status may reduce the risk for the syndrome. Here we investigate the effect of temporal changes in serum 25-hydroxyvitamin D (25(OHD concentrations on metabolic syndrome among Canadians enrolled in a preventive health program that promotes vitamin D supplementation. We accessed and analyzed data of 6682 volunteer participants with repeated observations on serum 25(OHD concentrations and metabolic syndrome. We applied logistic regression to quantify the independent contribution of baseline serum 25(OHD and temporal increases in serum 25(OHD to the development of metabolic syndrome. In the first year in the program, participants, on average, increased their serum 25(OHD concentrations by 37 nmol/L. We observed a statistical significant inverse relationship of increases in serum 25(OHD with risk for metabolic syndrome. Relative to those without improvements, those who improved their serum 25(OHD concentrations with less 25 nmol/L, 25 to 50 nmol/L, 50 to 75 nmol/L, and more 75 nmol/L had respectively 0.76, 0.64, 0.59, 0.56 times the risk for metabolic syndrome at follow up. These estimates were independent of the effect of baseline serum 25(OHD concentrations on metabolic syndrome. Improvement of vitamin D status may help reduce the public health burden of metabolic syndrome, and potential subsequent health conditions including type 2 diabetes and cardiovascular disease.

  1. Kindler syndrome

    OpenAIRE

    Kaviarasan P; Prasad P; Shradda; Viswanathan P

    2005-01-01

    Kindler syndrome is a rare autosomal recessive disorder associated with skin fragility. It is characterized by blistering in infancy, photosensitivity and progressive poikiloderma. The syndrome involves the skin and mucous membrane with radiological changes. The genetic defect has been identified on the short arm of chromosome 20. This report describes an 18-year-old patient with classical features like blistering and photosensitivity in childhood and the subsequent development of poikiloderm...

  2. Turner Syndrome

    OpenAIRE

    Ramachandran Sudarshan; G Sree Vijayabala; KS Prem Kumar

    2012-01-01

    Turner syndrome is a genetic disorder that affects mostly females. Affected females have characteristic features such as short stature, premature ovarian failure, and several other features. Oral manifestations of this condition are not much discussed in the literature. But reported literature includes teeth, palate, periodontal and salivary changes. So the aim of this review is to illustrate the general manifestations, and especially the oral manifestations of Turner syndrome and evaluate th...

  3. Ferulic Acid Alleviates Changes in a Rat Model of Metabolic Syndrome Induced by High-Carbohydrate, High-Fat Diet

    Directory of Open Access Journals (Sweden)

    Ketmanee Senaphan

    2015-08-01

    Full Text Available Metabolic syndrome is a cluster of metabolic abnormalities characterized by obesity, insulin resistance, hypertension and dyslipidemia. Ferulic acid (FA is the major phenolic compound found in rice oil and various fruits and vegetables. In this study, we examined the beneficial effects of FA in minimizing insulin resistance, vascular dysfunction and remodeling in a rat model of high-carbohydrate, high-fat diet-induced metabolic changes, which is regarded as an analogue of metabolic syndrome (MS in man. Male Sprague-Dawley rats were fed a high carbohydrate, high fat (HCHF diet and 15% fructose in drinking water for 16 weeks, where control rats were fed with standard chow diet and tap water. FA (30 or 60 mg/kg was orally administered to the HCHF and control rats during the last six weeks of the study. We observed that FA significantly improved insulin sensitivity and lipid profiles, and reduced elevated blood pressure, compared to untreated controls (p < 0.05. Moreover, FA also improved vascular function and prevented vascular remodeling of mesenteric arteries. The effects of FA in HCHF-induced MS may be realized through suppression of oxidative stress by down-regulation of p47phox, increased nitric oxide (NO bioavailability with up-regulation of endothelial nitric oxide synthase (eNOS and suppression of tumor necrosis factor-α (TNF-α. Our results suggest that supplementation of FA may have health benefits by minimizing the cardiovascular complications of MS and alleviating its symptoms.

  4. Qualitative changes of ocular surface in the patients with dry eye syndrome after Systein Ultra systemic using

    Directory of Open Access Journals (Sweden)

    T. I. Poltanova

    2014-07-01

    Full Text Available Purpose: To evaluate clinical efficiency of systemic usage of artificial tears drops Systein Ultra in the patients with different forms of dry eye syndrome.Methods: 42 patients (84 eyes at the age of 21‑84 with different pathogenic forms of ocular xerosis and degrees of it’s severity were investigated. Systein Ultra eye drops in instillations were prescribed to all patients 3 times a day. Complex examination of being investigated was performed before the drug administration, in 7 days and then in 2 weeks.Results: Positive dynamics after the treatment was reavealed on average in 4‑7 days. There was decrease or absolute reduction of subjective and objective signs in the majority of cases. In the severest cases of the disease all investigated parameters were not changed.Conclusion: The positive clinical result after artificial tears drops Systein Ultra using, accompanied by eye comfort without significant side effects, allows us to recommend Systein Ultra to the wide application in therapy of different pathogenic forms dry eye syndrome.

  5. Dynamic Changes in Serum Estradiol and Progesterone levels in Patients of Premenstrual Syndrome with Adverse Flow of Liver-qi

    Institute of Scientific and Technical Information of China (English)

    Qiao Mingqi; Zhang Huiyun; Yu Yanhong; Ci Yuzhen; Xu Xujie; Ye Qing; Chen Yuzhen; Wang Xinzhong

    2008-01-01

    Objective:To probe into the influence of changes of ovarian hormones on the pathogenesis of the specific sub-type premenstrual syndrome(PMS)and reveal partial microcosmic mechanisms of adverse flow of liver-qi.Methods:Estradiol(E2)and progesterone(P)levels in serum were determined at different phases of menstrual cycle by radioimmunoassay.Results:In the group of PMS with adverse flow of liver-qi.the secretive peak value Of E2 and P at the follicular phase significantly decreased,and the secretive peak value at the luteal phase did not come into being.Conclusions:Low E2 and P secretive peak at the follicular phase and absence of secretive peak at the luteal phase is one of the microcosmic mechanisms of PMS with adverse flow of liver-qi.One of the pathophysiologic mechanisms of specific sub-type PMS is probably the continuous low level of E2and P.

  6. Post-traumatic fat embolism syndrome--a 10 year retrospective study in Chang Gung Memorial Hospital.

    Science.gov (United States)

    Hsu, D T; Chao, E K; Shih, C H

    1990-06-20

    Forty patients with post-traumatic fat embolism syndrome (FES) from January 1977 through December 1986 were retrospectively analyzed. Diagnosis was made according to the criteria modified by Guard. All 40 patients had at least two major criteria, namely change in consciousness and hypoxia. Twenty patients (50%) presented with full-blown clinical features. Prompt respiratory support with oxygen mask or nasal prongs was the first line of treatment. Forty per cent of patients responded well, whereas the others had to be advanced to endotracheal intubation and mechanical ventilation with positive end expiratory pressure. Steroids were given to 80% of patients and better results were achieved than in the group not treated with steroids. The mortality rate was low (2.5%) and only four patients suffered prolonged cerebral sequelae. The clinical course and prognosis cannot be predicted from the severity of the fracture. PMID:2224610

  7. [Heptopulmonary syndrome].

    Science.gov (United States)

    Cuadrado, Antonio; Díaz, Ainhoa; Iruzubieta, Paula; Salcines, José Ramón; Crespo, Javier

    2015-01-01

    Hepatopulmonary syndrome is characterized by the presence of liver disease, pulmonary vascular dilatations, and arterial hypoxemia. It is usually associated with cirrhosis of any origin, but has been described in other liver diseases, both acute and chronic, and not always associated with portal hypertension. The gold standard method to detect pulmonary vascular dilations is contrast enhancement echocardiography with saline and is essential for the diagnosis of hepatopulmonary syndrome. These dilatations reflect changes in the pulmonary microvasculature (vasodilatation, intravascular monocyte accumulation, and angiogenesis) and induce a ventilation/perfusion mismatch, or even true intrapulmonary shunts, which eventually trigger hypoxemia. This syndrome worsens patients' prognosis and impairs their quality of life and may lead to the need for liver transplantation, which is the only effective and definitive treatment. In this article, we review the etiological, pathophysiological, clinical and therapeutic features of this syndrome. PMID:25840463

  8. Alpha-thalassemia X-linked intellectual disability syndrome identified by whole exome sequencing in two boys with white matter changes and developmental retardation.

    Science.gov (United States)

    Lee, Jin Sook; Lee, Sangmoon; Lim, Byung Chan; Kim, Ki Joong; Hwang, Yong Seung; Choi, Murim; Chae, Jong-Hee

    2015-09-15

    Alpha-thalassemia X-linked intellectual disability (ATRX) syndrome is a genetic syndrome caused by mutation of the ATRX gene associated with chromatin remodeling. Recently, a wide spectrum of brain MRI abnormalities and clinical manifestations has been recognized. We describe two male patients with genetically confirmed ATRX syndrome, both presented with developmental delay and white matter changes without typical clinical characteristics of ATRX. Whole-exome sequencing revealed the presence of ATRX mutations: a novel c.6472A>G mutation in Case 1 and a previously reported c.6532C>T mutation in Case 2. These two cases expanded the genetic and clinical spectrum of ATRX syndrome, including brain MRI abnormalities. Our results suggest that male patients with developmental delay and widespread white matter changes, even without distinctive facial dysmorphism and hematologic abnormalities, should be suspected as ATRX syndrome. We support the clinical utility of whole-exome sequencing, particularly in ultra-rare neurological diseases with nonspecific developmental disabilities and atypical presentation. PMID:25936994

  9. Assessment of Metformin as an Additional Treatment to Therapeutic Lifestyle Changes in Pediatric Patients with Metabolic Syndrome

    Directory of Open Access Journals (Sweden)

    Rebecca M. Raub

    2012-01-01

    Full Text Available Objective. To assess the effectiveness of metformin and therapeutic lifestyle changes (TLCs in a clinical setting, compared to TLC alone in adolescents with metabolic syndrome (MS. Methodology. This study was a retrospective trial consisting of 60 patients, aged 8–18 years, who were treated for MS at an outpatient clinic. Two groups were formed: the metformin group (M group and the control group (C group. The M group had been given metformin along with TLC, and the C group had been given TLC alone. Several outcome measures were obtained; the main outcome measure was measuring the change in percentile and z-score of weight and BMI. Results. There were no significant differences between the two groups at the conclusion of the study, except for height percentile (P=0.02 and z-score (P=0.03. Both groups showed promising significant intragroup decreases in weight z-score but BMI percentile and z-score were only significantly decreased in the M group. Conclusion. Metformin at an average dose of 1033 mg, when added to TLC, did not show any clinically important efficacy compared to TLC alone in a pediatric population with MS. However, both groups made significant changes in a positive direction, which may be solely due to TLC.

  10. [Antioxidative status changes in golden syrian hamsters with experimental metabolic syndrome].

    Science.gov (United States)

    Zahaĭko, A L; Voronina, L M; Kaliman, P A; Strel'chenko, K V

    2008-01-01

    Some indices of the antioxidant status (content of the alpha-tocopherol, reduced glutathione and ascorbic acid, activity of the glutathione reductase and aryl-esterase) and lipid peroxidation processes in the liver, blood serum, and some blood serum lipoprotein fractions of the Golden Syrian hamsters of different sex and age status under high-caloric diet were investigated. It has been shown that the hypercaloric diet leads to a decreaseng of reduced glutathione content and increase of the level of lipid peroxidation products in the liver of experimental animals. The ascorbic acids content in male liver is decreased and in female liver is increased. In the blood serum under hypercaloric nutrition the accumulation of lipid peroxidation products and alpha-tocopherol content a decrease in ApoB-lipoproteins and HDL is observed. Simultaneously the ascorbic acid content is increased in the blood serum of all experimental animals. Activation of free-radical oxidation both in the liver, and blood serum is more significant in males compared with females. The data obtained allow to suppose that atherosclerotic complications of metabolic syndrome development may be connected to the lipoprotein oxidant status infringement. PMID:18959034

  11. Bone mineral density and changes in bone metabolism in patients with obstructive sleep apnea syndrome.

    Science.gov (United States)

    Terzi, Rabia; Yılmaz, Zahide

    2016-07-01

    The aim of this study was to evaluate the differences between patients with obstructive sleep apnea syndrome (OSAS) and phenotypically similar subjects without OSAS in terms of bone mineral density (BMD) and bone turnover markers. The study was conducted on 30 males diagnosed with OSAS and 20 healthy males. All subjects underwent polysomnographic testing. Calcium, phosphorus parathyroid hormone, thyroid stimulating hormone, bone-specific alkaline phosphatase, 25-hydroxyvitamin D3, osteocalcin, and beta-CrossLaps (β-CTx) were measured. BMD in the lumbar spine (L1-L4) and femoral neck was measured by dual energy X-ray absorptiometry. There was no statistically significant difference between the two groups in terms of demographic data with the exception of bone mass index and waist circumference. (p < 0.05). Analyses showed significantly lower BMD measurements in the femoral neck and T-scores in the femoral neck in patients diagnosed with OSAS. Serum β-CTx levels were found to be statistically significantly higher in the OSAS group (p = 0.017). In multivariate assessments performed for apnea/hypopnea index values, mean saturation O2 levels were found to be significantly associated with osteocalcin levels and neck BMD. OSAS patients might represent a risk group with respect to loss of BMD and bone resorption. It is important to evaluate bone loss in these patients. Further studies should be carried out on larger study populations to evaluate the effects of chronic hypoxia on BMD in detail. PMID:26204846

  12. Age-related changes of adaptive and neuropsychological features in persons with Down Syndrome.

    Directory of Open Access Journals (Sweden)

    Alessandro Ghezzo

    Full Text Available Down Syndrome (DS is characterised by premature aging and an accelerated decline of cognitive functions in the vast majority of cases. As the life expectancy of DS persons is rapidly increasing, this decline is becoming a dramatic health problem. The aim of this study was to thoroughly evaluate a group of 67 non-demented persons with DS of different ages (11 to 66 years, from a neuropsychological, neuropsychiatric and psychomotor point of view in order to evaluate in a cross-sectional study the age-related adaptive and neuropsychological features, and to possibly identify early signs predictive of cognitive decline. The main finding of this study is that both neuropsychological functions and adaptive skills are lower in adult DS persons over 40 years old, compared to younger ones. In particular, language and short memory skills, frontal lobe functions, visuo-spatial abilities and adaptive behaviour appear to be the more affected domains. A growing deficit in verbal comprehension, along with social isolation, loss of interest and greater fatigue in daily tasks, are the main features found in older, non demented DS persons evaluated in our study. It is proposed that these signs can be alarm bells for incipient dementia, and that neuro-cognitive rehabilitation and psycho-pharmacological interventions must start as soon as the fourth decade (or even earlier in DS persons, i.e. at an age where interventions can have the greatest efficacy.

  13. Age-related changes of adaptive and neuropsychological features in persons with Down Syndrome.

    Science.gov (United States)

    Ghezzo, Alessandro; Salvioli, Stefano; Solimando, Maria Caterina; Palmieri, Alice; Chiostergi, Chiara; Scurti, Maria; Lomartire, Laura; Bedetti, Federica; Cocchi, Guido; Follo, Daniela; Pipitone, Emanuela; Rovatti, Paolo; Zamberletti, Jessica; Gomiero, Tiziano; Castellani, Gastone; Franceschi, Claudio

    2014-01-01

    Down Syndrome (DS) is characterised by premature aging and an accelerated decline of cognitive functions in the vast majority of cases. As the life expectancy of DS persons is rapidly increasing, this decline is becoming a dramatic health problem. The aim of this study was to thoroughly evaluate a group of 67 non-demented persons with DS of different ages (11 to 66 years), from a neuropsychological, neuropsychiatric and psychomotor point of view in order to evaluate in a cross-sectional study the age-related adaptive and neuropsychological features, and to possibly identify early signs predictive of cognitive decline. The main finding of this study is that both neuropsychological functions and adaptive skills are lower in adult DS persons over 40 years old, compared to younger ones. In particular, language and short memory skills, frontal lobe functions, visuo-spatial abilities and adaptive behaviour appear to be the more affected domains. A growing deficit in verbal comprehension, along with social isolation, loss of interest and greater fatigue in daily tasks, are the main features found in older, non demented DS persons evaluated in our study. It is proposed that these signs can be alarm bells for incipient dementia, and that neuro-cognitive rehabilitation and psycho-pharmacological interventions must start as soon as the fourth decade (or even earlier) in DS persons, i.e. at an age where interventions can have the greatest efficacy. PMID:25419980

  14. Lithium-induced minimal change disease and acute kidney injury

    Directory of Open Access Journals (Sweden)

    Parul Tandon

    2015-01-01

    Full Text Available Context: Lithium carbonate is a psychiatric medication commonly used in the treatment of bipolar disorder. It has been implicated in inducing nephrogenic diabetes inspidus, chronic tubulointerstitial nephropathy, and acute tubular necrosis. We describe a case of lithium-induced minimal change disease (MCD and acute kidney injury (AKI. Case Report: A 32-year-old female with a medical history of bipolar disorder treated with chronic lithium therapy presented with anasarca, fatigue, and tremors. Work-up revealed supra-therapeutic lithium levels, hypoalbuminemia, and significant proteinuria. The patient was treated conservatively with fluids and discontinuation of lithium therapy. Subsequently, she developed significant AKI and persistent proteinuria. She underwent a renal biopsy that demonstrated effacement of podocyte foot processes consistent with lithium-induced MCD. This was treated with corticosteroids, which decreased the proteinuria and resolved all the patient′s symptoms. Conclusion: Lithium-induced MCD is a rare disease that affects patients of all ages. It is often associated with therapeutic lithium and is typically resolved with discontinuation of lithium. In some cases, concurrent AKI may result due to vascular obstruction from hyperalbuminuria and associated renal interstitial edema. Corticosteroids may be needed to reduce the proteinuria and prevent progression to chronic kidney disease. As such, patients on lithium therapy may benefit from monitoring of glomerular function via urinalysis to prevent the onset of nephrotic syndrome.

  15. Ultrastructural Changes of Airway in Murine Models of Allergy and Diet-Induced Metabolic Syndrome

    OpenAIRE

    Leishangthem, Geeta Devi; Mabalirajan, Ulaganathan; Singh, Vijay Pal; Agrawal, Anurag; Ghosh, Balaram; Dinda, Amit Kumar

    2013-01-01

    Studying ultrastructural changes could reveal novel pathophysiology of obese-asthmatic condition as existing concepts in asthma pathogenesis are based on the histological changes of the diseased airway. While asthma is defined in functional terms, the potential of electron microscopy (EM) in providing cellular and subcellular detail is underutilized. With this view, we have performed transmission EM in the lungs from allergic mice that show key features of asthma and high-fat- or high-fructos...

  16. The Change of Interleukin-6 and Tumor Necrosis Factor in Patients with Obstructive Sleep Apnea Syndrome

    Institute of Scientific and Technical Information of China (English)

    2000-01-01

    The levels of lipopolysaccharide (LPS)-induced interleukin-6 (IL-6) and tumor necrosis factor-α (TNF-α) expression in culture of peripheral blood mononuclear cells (PBMC) and the plasma levels of IL-6 and TNF-α in the patients with obstructive sleep apnea syndrome (OSAS) were measured and the relationship between OSAS and IL-6 or TNF-α expression studied. Both IL-6 and TNF-α were detected by using ELISA in 22 patients with OSAS and 16 normal controls. The levels of LPS-induced IL-6 (787.82±151.97 pg/ml) and TNF-α (4165.45±1501.43 pg/ml) expression in the supernatant of the culture of PBMC and plasma level of IL-6 (50.67±4.70 pg/ml) and TNF-α (299.09±43.57 pg/ml) in the patients with OSAS were significantly higher than those in the normal controls (in the supernatant of the culture of PBMC: 562.69±197.54 pg/ml and 1596.25±403.08 pg/ml respectively; in the plasma: 12.69±2.75 pg/ml and 101.88±21.27 pg/ml respectively). There were significantly positive correlation between the levels of IL-6 and TNF-α and the percentage of time of apnea and hyponea, as well as the percentage of time spending at SaO2 below 90 % in the total sleep time. It was concluded that LPS-induced IL-6 and TNF-α levels as well as plasma IL-6 and TNF-α levels in the patients with OSAS were up-regulated, which may be associated with the pathogenesis of OSAS.

  17. How Is Metabolic Syndrome Treated?

    Science.gov (United States)

    ... page from the NHLBI on Twitter. How Is Metabolic Syndrome Treated? Heart-healthy lifestyle changes are the first line of treatment for metabolic syndrome. If heart-healthy lifestyle changes aren’t enough, ...

  18. Acute mental status change as the presenting feature of adrenal insufficiency in a patient with autoimmune polyglandular syndrome type II and stroke

    OpenAIRE

    Watson, Sara; Raj, Shekar; Eugster, Erica; Sanchez, Juan

    2014-01-01

    Primary adrenal insufficiency (AI) in children usually presents with non-specific symptoms such as fatigue, nausea, vomiting, and anorexia. Here, we report an unusual case of a 15 year old girl who presented with acute mental status change and was ultimately diagnosed with AI due to autoimmune polyglandular syndrome type II (APS2). Central nervous system imaging revealed a cerebral infarction. To our knowledge, the constellation of APS2, stroke and acute mental status change has not been prev...

  19. Corneal changes in ectrodactyly-ectodermal dysplasia-cleft lip and palate syndrome: case series and literature review.

    Science.gov (United States)

    Felipe, Anthony F; Abazari, Azin; Hammersmith, Kristin M; Rapuano, Christopher J; Nagra, Parveen K; Peiro, Baltasar Moratal

    2012-10-01

    The aim of this study is to describe the corneal changes in three unrelated patients with ectrodactyly-ectodermal dysplasia-cleft lip and palate (EEC) syndrome and review the literature on the possible etiology and clinical presentation of similar cases. Case 1 is an 18-year-old female with cleft lip and palate, syndactyly, and bilateral corneal pannus superiorly and inferiorly. She was initially diagnosed and treated as herpes simplex virus keratitis. Case 2 is a 3-year-old female born with cleft lip and palate, absent radial digits in both hands, and bilateral lacrimal stenosis. She developed progressive stromal scarring and neovascularization in both eyes. Her cornea perforated after developing infectious ulceration. Case 3 is a 49-year-old male with cleft palate, claw-hand deformities, absent meibomian glands and lacrimal duct, right ankyloblepharon, and a superior wedge-shaped opacity in the left cornea. The clinical findings demonstrated the different spectrum of keratopathy seen in patients with EEC. All patients were treated medically and without any surgical intervention. Limbal stem cell deficiency (LSCD) is presumed to be the cause in all three cases. Corneal changes in EEC can have variable presentation. LSCD seems to be the etiology of such keratopathy. Recurrent infection from lacrimal drainage obstruction and tear film instability are other risk factors for disease severity and progression. PMID:22618129

  20. Functional changes of vein endothelial cells of newborns of women with severe pregnancy-induced hypertension syndrome

    International Nuclear Information System (INIS)

    Objective: To investigate the functional changes of vein endothelial cells (VECs) of the newborns of the women with severe pregnancy-induced hypertension syndrome (PIH). Methods: The newborn VECs from 16 cases of severe PIH women and 17 cases of normal pregnancy women were cultured by pancreatin digestion, supernatant fluid was collected after 48 h, the levels of 6-keto-pGF1α (terminal product of metabolism from ET-1, PGI2), NO2- and VWF (terminal product of metabolism from NO) were detected. Results: The level of ET-1 in VECs of severe PIH group was increased, but compared with the control group, there was no significant difference (P>0.05). The levels of NO2- and 6-keto-pGF1α were decreased, compared with control group, there was significant difference (P<0.01), the concentration of VWF factor increased remarkably (P<0.05). Conclusion: The injure and functional change of VECs are present in newborns of severe PIH women

  1. Returning to work after suffering from burnout syndrome: Perceived changes in personality, views, values, and behaviors connected with work

    Directory of Open Access Journals (Sweden)

    Boštjančič Eva

    2014-01-01

    Full Text Available To date only a few studies have focused on returning to work after suffering from burnout syndrome. Participants were asked about their perceived work effectiveness, changes in their personal values, and obstacles and support factors that they encountered when they returned to work. Among the 27 individuals of various professions included in the study, 18 achieved an average or a high score on the Maslach Burnout Inventory, which was used to conduct a semi-structured interview. The answers were later processed by analyzing the content. The results showed that burned-out individuals only slowly return to work after recovery. When they return to work, they encounter changes in personality, personal values, and work effectiveness, and they only receive partial support from the environment. The results draw attention to insufficient detection of the disease by medical staff and employers in Slovenia. Recovering from burnout is a long-term process, which depends most on individuals themselves. At the same time, they can receive the necessary support from their family and coworkers, especially in terms of understanding them and partially adapting their responsibilities at work when they return. This study draws attention to a number of factors that can influence an individual’s process of returning to work and can be used as a basis for developing systematic rehabilitation programs.

  2. Volumetric Brain Morphometry Changes in patients with Obstructive Sleep Apnea Syndrome : effects of CPAP treatment and literature review.

    Directory of Open Access Journals (Sweden)

    Nelly T Huynh

    2014-04-01

    Full Text Available Introduction: Obstructive sleep apnea syndrome (OSAS is a frequent breathing disorder occurring during sleep that is characterized by recurrent hypoxic episodes and sleep fragmentation. It remains unclear whether OSAS leads to structural brain changes, and if so, in which brain regions. Brain region-specific gray and white matter volume (GMV and WMV changes can be measured with voxel-based morphometry (VBM. The aims of this study were to use VBM to analyze GMV and WMV in untreated OSAS patients compared to healthy controls (HC; examine the impact of OSAS-related variables (nocturnal hypoxemia duration and sleep fragmentation index on GMV and WMV; and assess the effects of therapeutic versus sham continuous positive airway pressure (CPAP. We discuss our results in light of previous findings and provide a comprehensive literature review. Methods: Twenty-seven treatment-naïve male patients with moderate to severe OSAS and seven healthy age- and education-matched control subjects (HC were recruited. After a baseline fMRI scan, patients randomly received either active (therapeutic, n=14 or sham (subtherapeutic, n=13 nasal CPAP treatment for 2 months. Results: Significant negative correlations were observed between nocturnal hypoxemia duration and GMV in bilateral lateral temporal regions. No differences in GMV or WMV were found between OSAS patients and HC, and no differences between CPAP versus sham CPAP treatment effects in OSAS patients. Conclusion: It appears that considering VBM GMV changes there is little difference between OSAS patients and HC. The largest VBM study to date indicates structural changes in the lateral aspect of the temporal lobe, which also showed a significant negative correlation with nocturnal hypoxemia duration in our study. This finding suggests an association between the effect of nocturnal hypoxemia and decreased GMV in OSAS patients.

  3. 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 ...

  4. Thyroid Malignancy Association with Cortical and Subcortical Brain SPECT Changes In Patients Presenting with a Myalgic Encephalomyelitis / Chronic Fatigue Syndrome

    International Nuclear Information System (INIS)

    Thyroid malignancy in ME/CFS patients greatly exceeds the normal incidence of thyroid malignancy in any known subgroup. The thyroid malignancy incidence in the ME/CFS group may exceed 6,000 / 100,000. As part of their investigation, Myalgic Encephalomyelitis / Chronic Fatigue Syndrome (ME/CFS) patients should be examined by thyroid ultrasound for evidence of thyroid pathology and malignancy. Thyroid pathology may be missed in this group of patients if investigation relies only upon serum testing for TSH, FT3, FT4, microsomal and thyroglobulin antibodies, which are usually normal. Thyroid uptake scans tend also to be normal and may also miss malignant lesions. A newly recognized syndrome may exist in ME/CFS patients characterized by: (a) thyroid malignancy, (b) persistent abnormal cortical and subcortical SPECT brain scans (NeuroSPECT), (c) failure of thyroidectomy surgery and hormone replacement to correct the fatigue syndrome, and (d) an unusual high incidence of cervical vertebrae osteoarthritic changes. ME/CFS patients with treated non-malignant thyroid disease and abnormal NeuroSPECT scans may also fail to improve despite adequate thyroid hormone replacement. A brief summary of the differences between ME and CFS is discussed. Lee, Hur and Ahn [1] stated that thyroid malignancy is said to be an infrequent occurrence found in 0.5 to 3 patients per 100,000 in the general population. They noted that in a subgroup of patients booked for mammography, a thyroid ultrasound was also performed. In this group, they found thyroid malignancy frequency was as high as 3 per 100,000. It is not known if their subgroup was at a higher risk for malignancy. Mittelstaedt [2] in the Globe and Mail states that thyroid malignancy was 15 per 100,000. In the past 100 patients whom I have investigated for (ME/CFS)[3], with or without associated Fibromyalgia Syndrome (FS), I have found that 6% of these patients had thyroid malignancy. In each of these patients the diagnosis was made by

  5. 儿童肾病综合征及泼尼松治疗对血清IGF-1及IGFBPs的影响%Change of insulin like growth factor-1(IGF-1) and IGF binding proteins during glucocorticoid treatment in children with nephrotic syndrome

    Institute of Scientific and Technical Information of China (English)

    董峰; 周湘; 庞宁; 魏珉

    2001-01-01

    目的了解儿童肾病综合征(NS)及糖皮质激素(GC)治疗对血清胰岛素样生长因子-1(IGF-1)及其结合蛋白(IGFBPs)的影响.方法采用免疫放射法对36名NS患儿,即未治疗的活动期组(ANS,12例),泼尼松治疗中活动期组(GNS,12例)和缓解期组(RE,12例),进行了血清IGF-1及其IGFBPs水平测定,并以同年龄组正常儿童作对照(NC,10例).结果 (1)与正常对照比较,NS活动期血清IGF-1和IGFBP-3明显降低(P<0.01),而IGFBP-1和IGFBP-2均明显升高(P<0.01);(2)NS缓解期血清IGF-1和IGFBP-3明显高于活动期 (P<0.01),而IGFBP-1和IGFBP-2明显低于活动期(P<0.01);(3)经激素治疗的活动期组血清IGF-1和IGFBP-3均明显高于未治疗活动期组 (P<0.01),IGFBP-1和IGFBP-2水平也明显低于ANS组(P<0.01);(4)NS活动期血清IGFBP-3与ALB成正相关(r=0.76,P<0.01),血清IGF-1与IGFBP-3成正相关,而与IGFBP-1、IGFBP-2成负相关.在缓解期及激素治疗中的活动期血清IGF-1及IGFBPs与ALB及CHO没有相关关系.结论肾病综合征及糖皮质激素治疗对NS儿童血清IGF-1和IGFBPs有明显影响,此影响可能与NS儿童生长障碍有关.

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

    DEFF Research Database (Denmark)

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

    2014-01-01

    . Methods We analysed additional data from a randomised controlled trial comparing completers of cognitive behavioural group therapy (46 patients) to an enhanced usual care group (66 patients). Proposed mediators (illness perceptions) and primary (physical health) and secondary (somatic symptoms and illness...... (primary analysis), and (2) whether changes in illness perceptions during the whole trial period were associated with improved outcome (secondary analysis). Results Improvements in illness perceptions during treatment partially mediated the effect of cognitive behavioural therapy on physical health one...... year after treatment (sum of indirect effects 1.556, BCa 95% CI (0.006; 3.620)). Improving perceived control was particularly important. Changes in illness perceptions from baseline to 16 months after randomisation were associated with clinically meaningful improvements in physical health, somatic...

  7. Changes in the carbohydrate-energy metabolism with radiation-induced intestine syndrome

    International Nuclear Information System (INIS)

    A local exposure of the rat abdomen in a dose of 3.6 cC/kg decreases the oxygen uptake, oxidation of glucose and fatty acids, glucose tolerance and insulin resistance, and also causes a trend toward lactic acidosis. These changes in the carbohydrate-energy metabolism are normalized with the administration of insulin and dichloracetate, and they may be interpreted as consequences of a shock provoked by a massive predominant injury to the intestine

  8. Changes in Yearly Birth Prevalence Rates of Children with Down Syndrome in the Period 1986-2007 in the Netherlands

    Science.gov (United States)

    de Graaf, G.; Haveman, M.; Hochstenbach, R.; Engelen, J.; Gerssen-Schoorl, K.; Poddighe, P.; Smeets, D.; van Hove, G.

    2011-01-01

    Background: The Netherlands are lacking reliable national empirical data in relation to the development of birth prevalence of Down syndrome. Our study aims at assessing valid national live birth prevalence rates for the period 1986-2007. Method: On the basis of the annual child/adult ratio of Down syndrome diagnoses in five out of the eight Dutch…

  9. Metabolic Syndrome

    Science.gov (United States)

    ... If you already have metabolic syndrome, making these healthy lifestyle choices can help reduce your risk of heart disease and other health problems. If lifestyle changes alone can’t control your ... to help. Maintain a healthy weight Your doctor can measure your body mass ...

  10. Cyclosporin-A induced Posterior Reversible Encephalopathy Syndrome

    Directory of Open Access Journals (Sweden)

    Saeed Bassam

    2008-01-01

    Full Text Available Posterior reversible encephalopathy syndrome (PRES is a recently proposed clinico-neuroradiological entity observed in a variety of clinical settings such as cyclosporin A (CsA neurotoxicity. We report a 3.5-year-old Syrian boy in whom steroid-resistant focal segmental glomerulosclerosis (FSGS was recently diagnosed. The patient remitted his nephrotic syndrome after 10 days of CsA administration. However, he shortly developed altered mental status, visual impairment, focal neurological deficits and seizures. We discontinued CsA that resulted in complete reversal of the patient′s encephalopathical condition over a period of 4 months. We conclude that PRES should be suspected in immunosuppresed patients with kidney disease if they have a sudden episode of neurological symptoms.

  11. Baseline brain activity changes in patients with clinically isolated syndrome revealed by resting-state functional MRI

    International Nuclear Information System (INIS)

    Background A clinically isolated syndrome (CIS) is the first manifestation of multiple sclerosis (MS). Previous task-related functional MRI studies demonstrate functional reorganization in patients with CIS. Purpose To assess baseline brain activity changes in patients with CIS by using the technique of regional amplitude of low frequency fluctuation (ALFF) as an index in resting-state fMRI. Material and Methods Resting-state fMRIs data acquired from 37 patients with CIS and 37 age- and sex-matched normal controls were compared to investigate ALFF differences. The relationships between ALFF in regions with significant group differences and the EDSS (Expanded Disability Status Scale), disease duration, and T2 lesion volume (T2LV) were further explored. Results Patients with CIS had significantly decreased ALFF in the right anterior cingulate cortex, right caudate, right lingual gyrus, and right cuneus (P < 0.05 corrected for multiple comparisons using Monte Carlo simulation) compared to normal controls, while no significantly increased ALFF were observed in CIS. No significant correlation was found between the EDSS, disease duration, T2LV, and ALFF in regions with significant group differences. Conclusion In patients with CIS, resting-state fMRI demonstrates decreased activity in several brain regions. These results are in contrast to patients with established MS, in whom ALFF demonstrates several regions of increased activity. It is possible that this shift from decreased activity in CIS to increased activity in MS could reflect the dynamics of cortical reorganization

  12. Saliva amylase as a measure of sympathetic change elicited by autogenic training in patients with functional somatic syndromes.

    Science.gov (United States)

    Kiba, Tadashi; Kanbara, Kenji; Ban, Ikumi; Kato, Fumie; Kawashima, Sadanobu; Saka, Yukie; Yamamoto, Kazumi; Nishiyama, Junji; Mizuno, Yasuyuki; Abe, Tetsuya; Fukunaga, Mikihiko

    2015-12-01

    The aim of this study was to discuss the effect of autogenic training (AT) on patients with functional somatic syndrome (FSS) using salivary amylase, the skin temperature of the finger, subjective severity of symptoms, and psychological characteristics as measures. We assessed 20 patients with FSS and 23 healthy controls before and after AT. Baseline levels of salivary amylase prior to an AT session were significantly higher in the FSS group than in the control group. However, this difference was not significant after AT. The skin temperature of the finger increased after AT in both the FSS and control groups. AT contributed to the improvement of somatic symptoms in patients with FSS. Our results regarding psychological characteristics suggest that mood disturbances are deeply involved in the pathology of FSS. Individuals with FSS exhibited elevated levels of sympathetic activity compared with healthy controls. Our data indicates that AT eased dysregulation of the autonomic nervous system in patients with FSS. Thus, salivary amylase may be a useful index of change induced by AT in patients with FSS. PMID:26219656

  13. Baseline brain activity changes in patients with clinically isolated syndrome revealed by resting-state functional MRI

    Energy Technology Data Exchange (ETDEWEB)

    Liu, Yaou; Duan, Yunyun; Liang, Peipeng; Jia, Xiuqin; Yu, Chunshui [Dept. of Radiology, Xuanwu Hospital, Capital Medical Univ., Beijing (China); Ye, Jing [Dept. of Neurology, Xuanwu Hospital, Capital Medical Univ., Beijing (China); Butzkueven, Helmut [Dept. of Medicine, Univ. of Melbourne, Melbourne (Australia); Dong, Huiqing [Dept. of Neurology, Xuanwu Hospital, Capital Medical Univ., Beijing (China); Li, Kuncheng [Dept. of Radiology, Xuanwu Hospital, Capital Medical Univ., Beijing (China); Beijing Key Laboratory of MRI and Brain Informatics, Beijing (China)], E-mail: likuncheng1955@yahoo.com.cn

    2012-11-15

    Background A clinically isolated syndrome (CIS) is the first manifestation of multiple sclerosis (MS). Previous task-related functional MRI studies demonstrate functional reorganization in patients with CIS. Purpose To assess baseline brain activity changes in patients with CIS by using the technique of regional amplitude of low frequency fluctuation (ALFF) as an index in resting-state fMRI. Material and Methods Resting-state fMRIs data acquired from 37 patients with CIS and 37 age- and sex-matched normal controls were compared to investigate ALFF differences. The relationships between ALFF in regions with significant group differences and the EDSS (Expanded Disability Status Scale), disease duration, and T2 lesion volume (T2LV) were further explored. Results Patients with CIS had significantly decreased ALFF in the right anterior cingulate cortex, right caudate, right lingual gyrus, and right cuneus (P < 0.05 corrected for multiple comparisons using Monte Carlo simulation) compared to normal controls, while no significantly increased ALFF were observed in CIS. No significant correlation was found between the EDSS, disease duration, T2LV, and ALFF in regions with significant group differences. Conclusion In patients with CIS, resting-state fMRI demonstrates decreased activity in several brain regions. These results are in contrast to patients with established MS, in whom ALFF demonstrates several regions of increased activity. It is possible that this shift from decreased activity in CIS to increased activity in MS could reflect the dynamics of cortical reorganization.

  14. Metformin Lowers Serum Cobalamin without Changing Other Markers of Cobalamin Status: A Study on Women with Polycystic Ovary Syndrome

    Directory of Open Access Journals (Sweden)

    Ebba Nexo

    2013-07-01

    Full Text Available Treatment with the anti-diabetic drug metformin is followed by a decline in plasma cobalamin, but it is unsettled whether this denotes an impaired cobalamin status. This study has explored changes in the markers of cobalamin status in women with Polycystic Ovary Syndrome treated with metformin (1.5–2.5 g per day (n = 29 or placebo (n = 23 for six months. Serum samples were collected before and after two, four, and six months of treatment. We found serum cobalamin to decline and reach significant lower levels after six months of treatment (p = 0.003. Despite the decline in serum cobalamin, we observed no reductions in the physiological active part of cobalamin bound to transcobalamin (holotranscobalamin, or increase in the metabolic marker of cobalamin status, methylmalonic acid. Instead, the non-functional part of circulating cobalamin bound to haptocorrin declined (p = 0.0009. Our results have two implications: The data questions whether metformin treatment induces an impaired cobalamin status in PCOS patients, and further suggests that serum cobalamin is a futile marker for judging cobalamin status in metformin-treated patients.

  15. Changes in erectile organ structure and function in a rat model of chronic prostatitis/chronic pelvic pain syndrome.

    Science.gov (United States)

    Wang, X-J; Xia, L-L; Xu, T-Y; Zhang, X-H; Zhu, Z-W; Zhang, M-G; Liu, Y; Xu, C; Zhong, S; Shen, Z-J

    2016-04-01

    There is a growing recognition of the association between chronic prostatitis/chronic pelvic pain syndrome (CP/CPPS) and erectile dysfunction (ED); however, most of the reports are based on questionnaires which cannot distinguish between organic and functional ED. The purpose of this study was to determine the exact relationship between CP/CPPS and ED, and to investigate the changes in erectile organ structure and function in a rat model of CP/CPPS. We established a rat model of experimental autoimmune prostatitis (EAP), which is a valid model for CP/CPPS. Erectile function in EAP and normal rats was comparable after cavernous nerve electrostimulation. The serum testosterone and oestradiol levels, ultrastructure of the corpus cavernosum and expression of endothelial nitric oxide synthase and neuronal nitric oxide synthase in the two groups were similar; however, there was a decrease in smooth muscle-to-collagen ratio and alpha-smooth muscle actin expression and an increase in transforming growth factor-beta 1 expression was observed in EAP rats. Thus, organic ED may not exist in EAP rats. We speculate that ED complained by patients with CP/CPPS may be psychological, which could be caused by impairment in the quality of life; however, further studies are needed to fully understand the potential mechanisms underlying the penile fibrosis in EAP rats. PMID:25990367

  16. Volume changes of whole brain gray matter in pediatric patients with Tourette syndrome: evidence from voxel-based morphometry

    International Nuclear Information System (INIS)

    Objectives: To identify the related abnormalities of gray matter in pediatric patients with Tourette syndrome (TS) by using the optimized voxel-based morphometry (VBM). Methods: Three dimensional T1WI was acquired in 31 TS children (28 boys, 3 girts, mean age 8 years, range 4-15 years) and 50 age- and sex-matched controls on a 1.5 Tesla Philips scanner. Images were pre-processed and analyzed using a version of VBM 2 in SPM 2. The whole brain gray matter volume was compared between the study and control group by using t-test. Multivariate linear regression analysis was used for analyzing the correlation between the change of grey matter volume within each brain region (mm3) and YGTSS score and course of disease of TS patients. Statistical analyses were performed by using SPSS 13.0. Results: Using VBM, significant increases in gray matter volumes in left superior parietal lobule, right cerebellar hemisphere and left parahippocampal gyrus were detected in TS patients, and the volume changes were 4059, 2126 and 84 mm3 (t=3.93, 3.71, 3.58, P<0.05) respectively. Compared to the control group, decreased grey matter volumes were found in medulla and left pons, and the volume changes were 213 and 117 mm3 (t=3.53, 3.48, P<0.05)respectively. Tic severity was not correlated with any volume changes of gray matter in brain (P>0.05, a small volume correction, KE ≥ 10 voxel). Tic course was negatively correlated with the gray matter volume of left parahippocampal gyrus (Beta =-0.391, P=0.039). Conclusions: Using VBM technique, the gray matter abnormalities can be revealed in TS patients without obvious lesions on conventional MR imaging. The increasing volume of temporal and parietal lobes and cerebellar may be an adaptive anatomical change in response to experiential demand. The gray matter volume of the parahippocampal gyrus may be used as one potential objective index for evaluating the prognosis of TS. (authors)

  17. Rapunzel syndrome

    International Nuclear Information System (INIS)

    An 18-year-old single female patient, presented with non specific gastrointestinal symptoms of anorexia, abdominal pain, and change in bowel habit. Clinically she was anemic, cachectic, and depressed. Abdominal examination revealed mobile epigastric mass. The scalp alopecia and endoscopy coupled by computed tomography scan, confirmed the diagnoses of trichobezoar, but it was not diagnosed as Rapunzel syndrome except after laparotomy, gastrotomy, and enterotomy. There are less than 16 cases of Rapunzel syndrome described worldwide, and this is the first case to be described in the middle east. (author)

  18. Joubert syndrome

    International Nuclear Information System (INIS)

    Joubert syndrome is a rare malformation of the posterior fossa, mainly affecting the cerebellar vermis, which generally appears as a dysplastic lesion. Other structures of the cervico medullary junction may be involved, with accompanying brainstem hypoplasia according to neuroimaging studies. The diagnosis is usually reached during, childhood, based on a constellation of changes in the child's neurological development that are supported by the results of imaging studied. Respiratory problems are the most common signs in newborns,leading to the suspicion of the presence of this syndrome. (Author) 11 refs

  19. Turner Syndrome

    Directory of Open Access Journals (Sweden)

    Ramachandran Sudarshan

    2012-08-01

    Full Text Available Turner syndrome is a genetic disorder that affects mostly females. Affected females have characteristic features such as short stature, premature ovarian failure, and several other features. Oral manifestations of this condition are not much discussed in the literature. But reported literature includes teeth, palate, periodontal and salivary changes. So the aim of this review is to illustrate the general manifestations, and especially the oral manifestations of Turner syndrome and evaluate their possible management. [Archives Medical Review Journal 2012; 21(4.000: 246-252

  20. Developmental changes in mental rotation ability and visual perspective-taking in children and adults with Williams Syndrome

    Directory of Open Access Journals (Sweden)

    Masahiro Hirai

    2013-12-01

    Full Text Available Williams syndrome (WS is a genetic disorder caused by the partial deletion of chromosome 7. Individuals with WS have atypical cognitive abilities, such as hypersociability and compromised visuospatial cognition, although the mechanisms underlying these deficits, as well as the relationship between them, remain unclear. Here, we assessed performance in mental rotation (MR and level 2 visual perspective taking (VPT2 tasks in individuals with and without WS. Individuals with WS obtained lower scores in the VPT2 task than in the MR task. These individuals also performed poorly on both the MR and VPT2 tasks compared with members of a control group. For the individuals in the control group, performance scores improved during development for both tasks, while the scores of those in the WS group improved only in the MR task, and not the VPT2 task. Therefore, we conducted a second experiment to explore the specific cognitive challenges faced by people with WS in the VPT2 task. In addition to asking participants to change their physical location (self-motion, we also asked them to adopt a third-person perspective by imagining that they had moved to a specified location (self-motion imagery. This enabled us to assess their ability to simulate the movement of their own bodies. The performance in the control group improved in both the self-motion and self-motion imagery tasks and both performances were correlated with verbal mental age. However, we did not find any developmental changes in performance for either task in the WS group. Performance scores for the self-motion imagery task in the WS group were low, similar to the scores observed for the VPT2 in this population. These results suggest that MR and VPT2 tasks involve different processes, and that these processes develop differently in people with WS. Moreover, difficulty completing VPT2 tasks may be partly because of an inability of people with WS to accurately simulate mental body motion.

  1. Developmental changes in mental rotation ability and visual perspective-taking in children and adults with Williams syndrome

    Science.gov (United States)

    Hirai, Masahiro; Muramatsu, Yukako; Mizuno, Seiji; Kurahashi, Naoko; Kurahashi, Hirokazu; Nakamura, Miho

    2013-01-01

    Williams syndrome (WS) is a genetic disorder caused by the partial deletion of chromosome 7. Individuals with WS have atypical cognitive abilities, such as hypersociability and compromised visuospatial cognition, although the mechanisms underlying these deficits, as well as the relationship between them, remain unclear. Here, we assessed performance in mental rotation (MR) and level 2 visual perspective taking (VPT2) tasks in individuals with and without WS. Individuals with WS obtained lower scores in the VPT2 task than in the MR task. These individuals also performed poorly on both the MR and VPT2 tasks compared with members of a control group. For the individuals in the control group, performance scores improved during development for both tasks, while the scores of those in the WS group improved only in the MR task, and not the VPT2 task. Therefore, we conducted a second experiment to explore the specific cognitive challenges faced by people with WS in the VPT2 task. In addition to asking participants to change their physical location (self-motion), we also asked them to adopt a third-person perspective by imagining that they had moved to a specified location (self-motion imagery). This enabled us to assess their ability to simulate the movement of their own bodies. The performance in the control group improved in both the self-motion and self-motion imagery tasks and both performances were correlated with verbal mental age. However, we did not find any developmental changes in performance for either task in the WS group. Performance scores for the self-motion imagery task in the WS group were low, similar to the scores observed for the VPT2 in this population. These results suggest that MR and VPT2 tasks involve different processes, and that these processes develop differently in people with WS. Moreover, difficulty completing VPT2 tasks may be partly because of an inability of people with WS to accurately simulate mental body motion. PMID:24376412

  2. Genetics Home Reference: cyclic vomiting syndrome

    Science.gov (United States)

    ... males and females, but fathers do not pass traits associated with changes in mtDNA to their children. ... Syndrome Cyclic Vomiting Syndrome Association International Foundation for Functional Gastrointestinal Disorders: Cyclic Vomiting Syndrome in Adults International ...

  3. [Refeeding syndrome].

    Science.gov (United States)

    Ševela, Stanislav; Novák, František; Kazda, Antonín; Brodská, Helena

    2016-01-01

    Despite being known more than 60 years, refeeding syndrome (RS) still bears many uncertainties. For example, its definition is not clear and definite, and the attitude to it varies from the complete neglect to over-prevention.The term "refeeding syndrome" refers to electrolyte and metabolic changes occurring in malnourished patients after the readministration of nutrition. These changes concern especially to phosphates and ions. Potassium, magnesium, naturism and fluids balance are involved. The changes lead to cell energetic metabolism and electric potential disturbances, with related clinical symptoms.Fully developed refeeding syndrome is quite rare; nevertheless it can be fatal for the patient. However, even its development can lead to many complications increasing the patient's morbidity and the length of stay in the hospital. Yet the refeeding syndrome is more or less predictable and if kept in mind also preventable.The aim of this article is to get the reader to know more about this metabolic phenomenon and possible attitudes towards it. PMID:27088791

  4. Serotonin Syndrome

    Directory of Open Access Journals (Sweden)

    Harold Muñoz Cortés

    2004-08-01

    Full Text Available The serotonin syndrome is a clinical condition associated with serotonin agonists, prescribed to treat some psychiatric and non psychiatric diseases like affective, anxiety and pain disorders. Is due to an excessive stimulation of central and peripheral serotonin receptors that leads to mental, autonomic and neuromuscular changes. Usually the disorder resolves within the first 24 hours after the medications are discontinued, however some patients progress to a multiple organ failure and die. This paper is a theoretical review of the fundamental aspects of the serotonin syndrome, beginning with a brief review of the anatomic and physiologic features of serotonin system, to continue to examine the most relevant historic, diagnosis, clinical and treatment aspects of the syndrome.

  5. A prospective study of the psychobehavioral factors responsible for a change from non-patient irritable bowel syndrome to IBS patient status

    OpenAIRE

    Nomura Shinobu; Fujii Yasushi

    2008-01-01

    Abstract Background To investigate non-patient irritable bowel syndrome (IBS) change to IBS and to determine factors predictive of the onset of IBS, individual biological factors, psychological factors, behavioral factors, and environmental factors were examined. Methods The subjects were 105 non-patient IBS (male = 59, female = 46, average age:21.49 ± 2.37), including 68 of the diarrhea-predominant type and 37 of the constipation-predominant type selected from 1,409 university and technical ...

  6. The effect of dietary changes on distinct components of the metabolic syndrome in a young Sri Lankan population at high risk of CVD.

    Science.gov (United States)

    Guess, Nicola; Wijesuriya, Mahen; Vasantharajah, Laksha; Gulliford, Martin; Viberti, Giancarlo; Gnudi, Luigi; Karalliedde, Janaka

    2016-08-01

    South Asian populations are predisposed to early onset of the metabolic syndrome. Lifestyle intervention programmes have demonstrated a reduction in the metabolic syndrome and CVD risk; however, the most effective components of the multi-faceted lifestyle interventions are unknown. We studied 2637 Sri Lankan males (n 1237) and females (n 1380), with a mean BMI of 23·9 (sd 4·2) kg/m2, aged 22·5 (sd 10·0) years, who had participated in a 5-year lifestyle-modification programme to examine the effect of dietary changes on distinct components of the metabolic syndrome. The dietary intervention comprised advice to replace polished starches with unpolished starches, high-fat meat and dairy products with low-fat products and high-sugar beverages and snacks with low-sugar varieties. For the purposes of this analysis, data from the control and intensive lifestyle groups were combined. Anthropometric and biochemical data were recorded, and a FFQ was completed annually. Multiple regression was used to determine the effect of the dietary changes on distinct components of the metabolic syndrome. The ratio unpolished:polished rice was inversely related to change in fasting glucose (β=-0·084, P=0·007) and TAG (β=-0·084, P=0·005) and positively associated with change in HDL-cholesterol (β=0·066, P=0·031) at the 5-year follow-up after controlling for relevant confounders. Red meat intake was positively associated with fasting glucose concentrations (β=0·05, P=0·017), whereas low-fat (β=-0·046, P=0·018) but not high-fat dairy products (β=0·003, P=0·853) was inversely related to glucose tolerance at the follow-up visit. Replacement of polished with unpolished rice may be a particularly effective dietary advice in this and similar populations. PMID:27358019

  7. Morphological changes of intestinal mucosa in patients with different clinical variants of irritable bowel syndrome using tetracyclic antidepressants and selective serotonin reuptake inhibitor

    OpenAIRE

    Nagieva S.; Svintsitskyy A.; Kuryk O.; Korendovych I.

    2015-01-01

    Objective. To assess histological changes of colonic mucosa in patients with clinically different types of irritable bowel syndrome (IBS) before and after the treatment with tetracyclic antidepressant and selective serotonin reuptake inhibitor. Methods. Adult patients (over 18 years) with confirmed diagnosis of IBS were examined. Biopsy specimens were taken from colon during colonoscopy for the next histological examination. One expert gastrointestinal pathologist assessed all tissue samples....

  8. Relation of EEG alpha background to cognitive fuction, brain atrophy, and cerebral metabolism in Down's syndrome. Age-specific changes

    International Nuclear Information System (INIS)

    We studied 19 young adults (19 to 37 years old) and 9 older patients (42 to 66 years old) with Down's syndrome (DS) and a control group of 13 healthy adults (22 to 38 years old) to investigate the relation of electroencephalographic (EEG) alpha background to cognitive function and cerebral metabolism. Four of the older patients with DS had a history of mental deterioration, disorientation, and memory loss and were demented. Patients and control subjects had EEGs, psychometric testing, quantitative computed tomography, and positron emission tomography with fludeoxyglucose F 18. A blinded reader classified the EEGs into two groups--those with normal alpha background or those with abnormal background. All the control subjects, the 13 young adult patients with DS, and the 5 older patients with DS had normal EEG backgrounds. In comparison with the age-matched patients with DS with normal alpha background, older patients with DS with decreased alpha background had dementia, fewer visuospatial skills, decreased attention span, larger third ventricles, and a global decrease in cerebral glucose utilization with parietal hypometabolism. In the young patients with DS, the EEG background did not correlate with psychometric or positron emission tomographic findings, but the third ventricles were significantly larger in those with abnormal EEG background. The young patients with DS, with or without normal EEG background, had positron emission tomographic findings similar to those of the control subjects. The mechanism underlying the abnormal EEG background may be the neuropathologic changes of Alzheimer's disease in older patients with DS and may be cerebral immaturity in younger patients with DS

  9. Country, sex, EDSS change and therapy choice independently predict treatment discontinuation in multiple sclerosis and clinically isolated syndrome.

    Directory of Open Access Journals (Sweden)

    Claire Meyniel

    Full Text Available OBJECTIVES: We conducted a prospective study, MSBASIS, to assess factors leading to first treatment discontinuation in patients with a clinically isolated syndrome (CIS and early relapsing-remitting multiple sclerosis (RRMS. METHODS: The MSBASIS Study, conducted by MSBase Study Group members, enrols patients seen from CIS onset, reporting baseline demographics, cerebral magnetic resonance imaging (MRI features and Expanded Disability Status Scale (EDSS scores. Follow-up visits report relapses, EDSS scores, and the start and end dates of MS-specific therapies. We performed a multivariable survival analysis to determine factors within this dataset that predict first treatment discontinuation. RESULTS: A total of 2314 CIS patients from 44 centres were followed for a median of 2.7 years, during which time 1247 commenced immunomodulatory drug (IMD treatment. Ninety percent initiated IMD after a diagnosis of MS was confirmed, and 10% while still in CIS status. Over 40% of these patients stopped their first IMD during the observation period. Females were more likely to cease medication than males (HR 1.36, p = 0.003. Patients treated in Australia were twice as likely to cease their first IMD than patients treated in Spain (HR 1.98, p = 0.001. Increasing EDSS was associated with higher rate of IMD cessation (HR 1.21 per EDSS unit, p<0.001, and intramuscular interferon-β-1a (HR 1.38, p = 0.028 and subcutaneous interferon-β-1a (HR 1.45, p = 0.012 had higher rates of discontinuation than glatiramer acetate, although this varied widely in different countries. Onset cerebral MRI features, age, time to treatment initiation or relapse on treatment were not associated with IMD cessation. CONCLUSION: In this multivariable survival analysis, female sex, country of residence, EDSS change and IMD choice independently predicted time to first IMD cessation.

  10. Clinico-demographic profile, sexual dysfunction and readiness to change in male alcohol dependence syndrome inpatients in a tertiary hospital.

    Science.gov (United States)

    Pandey, A K; Sapkota, N; Tambi, A; Shyangwa, P M

    2012-03-01

    Persons with prolonged and heavy alcohol use generally suffer from alcohol dependence syndrome (ADS) and develop physical, sexual as well as psychiatric co-morbidity. Successful recovery to normalcy depends on multiple factors including patient's motivation. To study clinico-demographic profile, reasons for initiating alcohol use, sexual and psychiatric disorders and eagerness for treatment and quitting alcohol in ADS inpatients. Fifty consecutive ADS inpatients with matching controls were enrolled. Clinico-demographic profile, factors for initiating alcohol use, psychiatric and sexual co-morbidity and want for treatment and being abstinent was studied applying relevant scales. All subjects were males with a mean age of 37.5 years, 80% were married, majority were Hindu (88%) and from nuclear families (56%). Fifty two percent had an education level of Graduation or more and 68% of patients reported peer pressure to be the initiating factor for alcohol use. Seventy six percent had psychiatric co-morbidity including personality Problems and other Psychiatric disorders 19(38%), delirium tremens 14 (28.00%) and Mood disorders 12(24%).Depression being most common mood disorder (14%). Nicotine was the most common other substance of use 32 (64%). Sixty eight percent of the patient reported one or another sexual dysfunction. 68% of ADS inpatients acknowledged of having problems related to their drinking, expressed desire for change and were eager to avail treatment and to remain abstinent. ADS patients commonly suffer from psychiatric co-morbidity and sexual dysfunctions. They also wish to have effective treatment and to quit alcohol. PMID:23441492

  11. Antiphospholipid Syndrome

    Science.gov (United States)

    ... Awards Enhancing Diversity Find People About NINDS NINDS Antiphospholipid Syndrome Information Page Synonym(s): Hughes Syndrome Table of Contents ( ... research is being done? Clinical Trials What is Antiphospholipid Syndrome? Antiphospholipid syndrome (APS) is an autoimmune disorder caused ...

  12. 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

  13. Changes in small intestinal chromogranin A-immunoreactive cell densities in patients with irritable bowel syndrome after receiving dietary guidance.

    Science.gov (United States)

    Mazzawi, Tarek; El-Salhy, Magdy

    2016-05-01

    Chromogranin A (CgA) is a common marker for enteroendocrine cells in the gut, and CgA-immunoreactive cell densities are abnormal in patients with irritable bowel syndrome (IBS). The majority of patients with IBS report that their symptoms develop after consuming certain foodstuffs. In the present study, we investigated the effects of dietary guidance on the total enteroendocrine cell densities in the small intestine, as detected by CgA. A total of 14 patients with IBS underwent a gastroscopy with duodenal biopsies and 11 of them also underwent a colonoscopy, with biopsy samples obtained from the ileum. Fourteen control subjects were also included. Each patient received 3 sessions of dietary guidance. Gastroscopies and colonoscopies were performed on both the controls and patients with IBS (at baseline and at 3-9 months after receiving guidance). Biopsy samples obtained from the duodenum and ileum were immunostained for CgA using the avidin-biotin complex (ABC) method and were quantified using computerized image analysis. The density of CgA-immunoreactive cells in the duodenum (mean ± SEM values) in the control subjects was 235.9 ± 31.9 cells/mm2; in the patients with IBS, the density was 36.9 ± 9.8 and 103.7 ± 16.9 cells/mm2 before and after they received dietary guidance, respectively (P=0.007). The density of CgA-immunoreactive cells in the ileum in the control subjects was 47.4 ± 8.3 cells/mm2; in the patients with IBS, the density was 48.4 ± 8.1 and 17.9 ± 4.4 cells/mm2, before and after they received dietary guidance, respectively (P=0.0006). These data indicate that changes in CgA-immunoreactive cell densities in patients with IBS after receiving dietary guidance may reflect a change in the densities of the small intestinal enteroendocrine cells, which may contribute to an improvement in the IBS symptoms. PMID:26987104

  14. Clinical significance of changes of plasma leptin and serum T, E2, FSH, LH, PRL levels in patients with polycystic ovary syndrome (PCOS)

    International Nuclear Information System (INIS)

    Objective: To explore the clinical significance of changes of plasma leptin and serum T, E2, FSH, LH, PRL levels in patients with polycystic ovary syndrome(PCOS). Methods: Plasma leptin (with RIA), serum T, E2, FSH, LH, PRL (with ECLIA) levels were detected in 31 patients with polycystic ovary syndrome(PCOS) as well as in 35 controls. Results: The plasma leptin and serum T, LH, PRL levels in the patients with PCOS were significantly higher than those in controls (P2, FSH levels were not significantly different from those in controls (P>0.05). Plasma leptin levels were positively correlated with those of serum T, LH, PRL (r=0.5784, 0.5411, 0.6082, P<0.01). Conclusion: Development of PCOS was closely related to the plasma leptin and serum T, LH, PRL levels. (authors)

  15. Schistosomal glomerulopathy and changes in the distribution of histological patterns of glomerular diseases in Bahia, Brazil

    Directory of Open Access Journals (Sweden)

    Washington Luis Conrado dos-Santos

    2011-11-01

    Full Text Available Distinct patterns of glomerular lesions, including membranoproliferative glomerulonephritis and focal segmental glomerulosclerosis, are associated with infection by Schistosoma mansoni or Schistosoma japonicum. Evidence suggests that immune complex deposition is the main mechanism underlying the different forms of schistosomal glomerulonephritis and that immune complex deposition may be intensified by portal hypertension. The relationship between focal segmental glomerulosclerosis and schistosomiasis remains poorly understood. A clinicopathologic classification of schistosomal glomerulopathies was proposed in 1992 by the African Association of Nephrology. In Brazil, mass treatment with oral medications has led to a decrease in the occurrence of schistosomal glomerulopathy. In a survey of renal biopsies performed in Salvador, Brazil, from 2003-2009, only 24 (4% patients were identified as positive for S. mansoni infection. Among these patients, only one had the hepatosplenic form of the disease. Focal segmental glomerulosclerosis was found in seven patients and membranoproliferative glomerulonephritis was found in four patients. Although retrospective studies on the prevalence of renal diseases based on kidney biopsies may be influenced by many patient selection biases, a change in the distribution of glomerulopathies associated with nephrotic syndrome was observed along with a decline in the occurrence of severe forms of schistosomiasis.

  16. Minimal Change Disease and IgA Deposition: Separate Entities or Common Pathophysiology?

    Directory of Open Access Journals (Sweden)

    Brandon S. Oberweis

    2013-01-01

    Full Text Available Introduction. Minimal Change Disease (MCD is the most common cause of nephrotic syndrome in children, while IgA nephropathy is the most common cause of glomerulonephritis worldwide. MCD is responsive to glucocorticoids, while the role of steroids in IgA nephropathy remains unclear. We describe a case of two distinct clinical and pathological findings, raising the question of whether MCD and IgA nephropathy are separate entities or if there is a common pathophysiology. Case Report. A 19-year old man with no medical history presented to the Emergency Department with a 20-day history of anasarca and frothy urine, BUN 68 mg/dL, Cr 2.3 mg/dL, urinalysis 3+ RBCs, 3+ protein, and urine protein : creatinine ratio 6.4. Renal biopsy revealed hypertrophic podocytes on light microscopy, podocyte foot process effacement on electron microscopy, and immunofluorescent mesangial staining for IgA. The patient was started on prednisone and exhibited dramatic improvement. Discussion. MCD typically has an overwhelming improvement with glucocorticoids, while the resolution of IgA nephropathy is rare. Our patient presented with MCD with the uncharacteristic finding of hematuria. Given the improvement with glucocorticoids, we raise the question of whether there is a shared pathophysiologic component of these two distinct clinical diseases that represents a clinical variant.

  17. Robinow Syndrome

    Directory of Open Access Journals (Sweden)

    Gökhan Gökalp

    2010-05-01

    Full Text Available Introduction: Robinow syndrome is characterized by dwarfism demonstrating short-limbed extremities, vertebral malsegmentation/malformation (hemivertebra, costal dysplasia, genital hypoplasia, and fetal facial appearance (wide and prominent forehead, hypertelorism, small and wide nose, molar hypoplasia, and retrognathia. It is a rare genetic disease which may present with either mild autosomal dominant form or severe recessive form. Vertebral and costal abnormalities are common diagnostic signs that may be severe. The disease presents with kyphoscoliosis and chest abnormalities along with thoracic vertebral fusion and hemivertebral appearance. Ribs may demonstrate fusion. Based on those involvements, the disease can be categorized as spondylothoracic, spondylocostal, ischiovertebral dysplasia, and cervicofaciothoracic syndrome.Diagnosis is established by the help of clinical characteristics. Radiography might contribute to the diagnosis by revealing changes in the skeletal system. Case Report: A three-year-old male patient presented with operated left undescendent testis and buried penis. On physical examination, he also had a dysmorphic face characterized by macrocephaly, hypertelorism, prominent eyes, a flattened nasal bridge, triangular-fish mouth, gingival hypertrophy and left hand clinodactyly. Radiographic examination documented mesomelic shortening of the radius-ulna, malsegmentation of the thoracal spine and the ribs fusion.Conclusion: Robinow syndrome is a rare syndrome which can be diagnosed by typical facial appearance and radiologic findings. (Journal of Current Pediatrics 2010; 8: 44-7

  18. Mechanisms of renal NaCl retention in proteinuric disease

    DEFF Research Database (Denmark)

    Svenningsen, Per; Friis, Ulla G; Versland, Jostein B;

    2013-01-01

    In diseases with proteinuria, for example nephrotic syndrome and pre-eclampsia, there often are suppression of plasma renin-angiotensin-aldosterone system components, expansion of extracellular volume and avid renal sodium retention. Mechanisms of sodium retention in proteinuria are reviewed. In...... animal models of nephrotic syndrome, the amiloride-sensitive epithelial sodium channel ENaC is activated while more proximal renal Na(+) transporters are down-regulated. With suppressed plasma aldosterone concentration and little change in ENaC abundance in nephrotic syndrome, the alternative modality of...

  19. Change of teicoplanin loading dose requirement for incremental increases of systemic inflammatory response syndrome score in the setting of sepsis.

    Science.gov (United States)

    Nakano, Takafumi; Nakamura, Yoshihiko; Takata, Tohru; Irie, Keiichi; Sano, Kazunori; Imakyure, Osamu; Mishima, Kenichi; Futagami, Koujiro

    2016-08-01

    Background Target trough concentrations are recommended for teicoplanin (TEIC) to minimize its adverse effects and to maximize efficacy in sepsis caused by grampositive cocci, including methicillin-resistant Staphylococcus aureus infection. However, optimal doses to attain proper trough values in patients with sepsis have not yet been well established for TEIC. Objective This study investigated whether the systemic inflammatory response syndrome (SIRS) score could predict the pharmacokinetics of TEIC in patients with sepsis. Setting This study was conducted at Fukuoka University Hospital in Japan. Methods We retrospectively reviewed the records of patients using TEIC between April 2012 and March 2015. SIRS positive was defined as infection with a SIRS score ≥2. Estimates of pharmacokinetic parameters were calculated using a Bayesian method. Creatinine clearance rates were estimated by the Cockcroft-Gault formula (eCcr). Main outcome measure Change of TEIC loading dose requirement for incremental increases of SIRS score. Results In total, 133 patients were enrolled: 50 non-SIRS patients and 83 patients with SIRS. The TEIC plasma trough concentration was significantly lower in SIRS than non-SIRS patients (15.7 ± 7.1 vs. 20.1 ± 8.6 μg/mL; P < 0.01), although there was no significant difference in the loading dose administered. Moreover, SIRS scores were increasingly predictive of eCcr and TEIC clearance in a stepwise manner. To achieve the target trough concentration (15-30 μg/mL), the optimal doses required in non-SIRS versus SIRS patients were 12-24 versus 18-30 mg/kg/day, respectively, during the first 48 h. Conclusions These findings suggest that the pharmacokinetics of TEIC are altered in SIRS patients, who required higher doses than non-SIRS patients to achieve the target trough concentration. We suggest that the SIRS score can become a new modality to determine the initial TEIC loading dose. PMID:27125378

  20. Contribution of regional cerebral blood flow changes on the evaluation of neuropsychological impairment in patients with Tourette syndrome

    International Nuclear Information System (INIS)

    Aims: The literature to date has suggested that intellectual ability is normally distributed in Tourette syndrome (TS). However, patients with TS have significant discrepancies between their verbal and non verbal abilities. Specific cognitive deficits in TS include visuomotor integration problems, impaired fine motor skill, and executive dysfunction. The presence of a learning disability or cognitive deficit may lead to a greater obstacle for patients with TS than the tic disorder itself. It would be important that patients with TS will be evaluated to having neuropsychological difficulties as soon as possible. The aim of this study was to evaluate the correlation between the clinical severity and neuropsychological impairment (especially memory deficit) of patients with TS and rCBF abnormalities. Methods: The assessment included neurological, psychiatrical examination (Yale Scale [YS], staging of the severity of disease), CT/MRI, and a specific test of explicit and implicit memory (Probabilistic Classification Learning test, PCL). 99mTc-HMPAO-SPECT studies were carried out with a standard technique for each patient. The data were analyzed visually and by a special ROIs program. RCBF data were correlated to the results of the PCL test and the YS. Results: The SPECT measurements showed significant (p40 in the temporal medial and lateral regions, and also in the cerebellum, compared to the patients with YS<40. Also, an increase of rCBF was observed in the left striatal, temporal medial and lateral regions, compared to the contralateral homologous regions (p<0.05). The YS (disease severity) demonstrated significant (p<0.05) and strong correlation with the rCBF in the right temporal region (r= -0.83) and in the thalamus (r=-0.69). The performance in the explicit memory test showed significant (p<0.05) and strong correlation with the perfusion in both temporopolar (r=0.9) and left temporomedial region (r=0.92). Conclusion: RCBF SPECT proved to be concordant with disease

  1. Perioperative Complications Prognosis Based on Local Neurocirculatory and Trophic Changes in the Anterior Eye Segment in Pseudoexfoliation Syndrome (Report II

    Directory of Open Access Journals (Sweden)

    A.V. Petrayevsky

    2009-03-01

    Full Text Available ABSTRACT Purpose To develop clinical method of prognosis of cataract surgery possible complications in pseudoexfoliation syndrome (PES patients convenient for routine practice of the ophthalmosurgeon. Material and methods We examined 111 patients (111 eyes between the ages of 58 and 76, who were operated for cataract. Of those, 66 patients (66 eyes had cataract combined with PES and 45 patients (45 eyes had cataract without PES signs. We used the following examination methods: visometry, eye anterior segment biomicroscopy with estimation of severity of iris trophic changes and pseudoexfoliation signs according to special classification schemes, computer tonography, ultrasonic biometry, ophthalmometry, vasotonometry in anterior ciliary arteries (ACA, and pupil cycle time (PCT calculation. The patients with cataract were operated by extracapsular extraction using corneal access in 60% of cases, and by mechanical phacofragmentation using scleral access in 40% of cases. All patients were implanted posterior chamber intraocular lenses (IOL. In the patients with cataract and PES we additionally used intracapsular extraction with implantation IOL RSP3 in 15% of cases. Results In operated patients, we recorded such complications of cataract sur gery as lens posterior capsule rupture, zonula fibers rupture, fibrinoidtype reactions, and postoperative ocular hypertension. The rate of complications was higher in PES patients (37,9% and lower in patients without PES (17,8%. An average PCT was 1840±82 ms in PES against 1186±35 ms without PES. ACA blood pressure in the PES-patients was 44,1±1,0 mm Hg against 53,1±0,98 mm Hg in the patients without PES. The rate of iris trophic changes was significantly higher in PES-patients than in the patients without PES. The rate of complications depended on frequency and severity of iris trophic changes and intensity of PES. Most of complicated cases were observed in the PES-patients with III–IV grade of PES

  2. Does Cardiac Rehabilitation After an Acute Cardiac Syndrome Lead to Changes in Physical Activity Habits? Systematic Review

    NARCIS (Netherlands)

    ter Hoeve, Nienke; Huisstede, Bionka M. A.; Stam, Henk J.; van Domburg, Ron T.; Sunamura, Madoka; van den Berg-Emons, Rita J. G.

    2015-01-01

    Background. Optimal physical activity levels have health benefits for patients with acute coronary syndrome (ACS) and are an important goal of cardiac rehabilitation (CR). Purpose. The purpose of this study was to systematically review literature regarding short-term effects (= 6 months after comple

  3. Language Development in Infants and Toddlers with Fragile X Syndrome: Change over Time and the Role of Attention

    Science.gov (United States)

    Kover, Sara T.; McCary, Lindsay M.; Ingram, Alexandra M.; Hatton, Deborah D.; Roberts, Jane E.

    2015-01-01

    Fragile X syndrome (FXS) is associated with significant language and communication delays, as well as problems with attention. This study investigated early language abilities in infants and toddlers with FXS (n = 13) and considered visual attention as a predictor of those skills. We found that language abilities increased over the study period of…

  4. Urodynamic changes in a female case of obstructive sleep apnea syndrome with enuresis: 7 years' follow-up

    Institute of Scientific and Technical Information of China (English)

    CAO Xia; HU Ke; CHEN Xue-qin; XIANYU Yun-yan; Lü Sheng-qi; LI Qing-quan

    2010-01-01

    @@ Obstructive sleep apnea syndrome (OSAS) is characterized by repetitive upper airway occlusion resulting in apnea lasting 10 seconds or more. Clinical manifestations include snoring, daytime somnolence,intellectual deficiency, sexual impotence, and nocturnal polyuria. Enuresis associated with OSAS is suggested to be more common in children than in adults.1

  5. Country, Sex, EDSS Change and Therapy Choice Independently Predict Treatment Discontinuation in Multiple Sclerosis and Clinically Isolated Syndrome

    NARCIS (Netherlands)

    Meyniel, Claire; Spelman, Timothy; Jokubaitis, Vilija G.; Trojano, Maria; Izquierdo, Guillermo; Grand'Maison, Francois; Oreja-Guevara, Celia; Boz, Cavit; Lugaresi, Alessandra; Girard, Marc; Grammond, Pierre; Iuliano, Gerardo; Fiol, Marcela; Antonio Cabrera-Gomez, Jose; Fernandez-Bolanos, Ricardo; Giuliani, Giorgio; Lechner-Scott, Jeannette; Cristiano, Edgardo; Herbert, Joseph; Petkovska-Boskova, Tatjana; Bergamaschi, Roberto; van Pesch, Vincent; Moore, Fraser; Vella, Norbert; Slee, Mark; Santiago, Vetere; Barnett, Michael; Havrdova, Eva; Young, Carolyn; Sirbu, Carmen-Adella; Tanner, Mary; Rutherford, Michelle; Butzkueven, Helmut

    2012-01-01

    Objectives: We conducted a prospective study, MSBASIS, to assess factors leading to first treatment discontinuation in patients with a clinically isolated syndrome (CIS) and early relapsing-remitting multiple sclerosis (RRMS). Methods: The MSBASIS Study, conducted by MSBase Study Group members, enro

  6. DSM-5 Changes and the Prevalence of Parent-Reported Autism Spectrum Symptoms in Fragile X Syndrome

    Science.gov (United States)

    Wheeler, Anne C.; Mussey, Joanna; Villagomez, Adrienne; Bishop, Ellen; Raspa, Melissa; Edwards, Anne; Bodfish, James; Bann, Carla; Bailey, Donald B.

    2015-01-01

    We used survey methodology to assess parent-reported autism symptomology in 758 individuals (639 males; 119 females) with fragile X syndrome (FXS). Caregivers reported whether their child with FXS had been diagnosed with an autism spectrum disorder (ASD) and endorsed symptoms based on a list of observable behaviors related to ASD diagnoses.…

  7. Progressive cerebellar degenerative changes in the severe mental retardation syndrome caused by duplication of MECP2 and adjacent loci on Xq28.

    LENUS (Irish Health Repository)

    Reardon, William

    2010-08-01

    Localised duplications, involving the MECP2 locus, at Xq28 have been associated with a syndrome comprising X-linked mental retardation, hypotonia and recurrent infections in males. We now present neuroradiological evidence that progressive cerebellar degenerative changes may also be a consistent feature of this syndrome, emerging in the second decade of life. We report seven affected males, from three different families who, in addition to the previously described clinical findings, have a reduction in the volume of the white matter and mild dilatation of the lateral ventricles. Three of the older patients show a consistent cerebellar degenerative phenotype. Furthermore, we describe the first female affected with the disorder. The female was mildly affected and shows X-inactivation in the ratio of 70:30, demonstrating that X-inactivation cannot be exclusively relied upon to spare the female carriers from symptoms. In conclusion, there is a radiological phenotype associated with Xq28 duplication which clearly demonstrates progressive degenerative cerebellar disease as part of the syndrome.

  8. POLYCYSTIC OVARY SYNDROME

    OpenAIRE

    Akula Annapurna

    2013-01-01

    Polycystic ovary syndrome is a condition in which a woman has an imbalance of female sex hormones. This may lead to menstrual cycle changes, cysts in the ovaries, trouble getting pregnant, and other health changes. In PCOS, mature eggs are not released from the ovaries. Instead, they can form very small cysts in the ovary. These changes can contribute to infertility. Common symptoms of PCOS include Menstrual disorders, Infertility, High levels of testosterone and Metabolic syndrome. Obesity, ...

  9. Syndrome in question*

    Science.gov (United States)

    Peruzzo, Juliano; Nazar, Fernanda Luca; Tubone, Mariana Quirino; Escobar, Gabriela Fortes; Cestari, Tania Ferreira

    2015-01-01

    Waardenburg syndrome is an inherited disease characterized by sensorineural hearing loss, pigmentation changes and minor facial malformations. It has four clinical variants. We report the case of a girl who, like her mother, was affected by this syndrome. The diagnosis was made after detection and treatment of deafness. PMID:26375234

  10. Syndrome in Question.

    Science.gov (United States)

    Peruzzo, Juliano; Nazar, Fernanda Luca; Tubone, Mariana Quirino; Escobar, Gabriela Fortes; Cestari, Tania Ferreira

    2015-01-01

    Waardenburg syndrome is an inherited disease characterized by sensorineural hearing loss, pigmentation changes and minor facial malformations. It has four clinical variants. We report the case of a girl who, like her mother, was affected by this syndrome. The diagnosis was made after detection and treatment of deafness. PMID:26375234

  11. Syndrome in question*

    OpenAIRE

    Peruzzo, Juliano; Nazar, Fernanda Luca; Tubone, Mariana Quirino; Escobar, Gabriela Fortes; Cestari, Tania Ferreira

    2015-01-01

    Waardenburg syndrome is an inherited disease characterized by sensorineural hearing loss, pigmentation changes and minor facial malformations. It has four clinical variants. We report the case of a girl who, like her mother, was affected by this syndrome. The diagnosis was made after detection and treatment of deafness.

  12. Rothmund - Thomson Syndrome

    Directory of Open Access Journals (Sweden)

    Sharma N. L

    2003-01-01

    Full Text Available Rothmund-Thomson syndrome is a rare geno-photodermatosis of children. Poikilodermatous cutaneous changes, growth retardation, juvenile cataract and high incidence of malignancy are its classical features. A Thomson type of Rothmund-Thomson syndrome with characteristic poikiloderma congenitale, growth retardation, absence of juvenile cataract and parental non-consanguinity is described in an 8 year old Indian girl.

  13. Metabolic syndrome X as a clinical outcome of hormonal changes on the liquidators of the Chernobyl accident consequences (the problem analysis)

    International Nuclear Information System (INIS)

    An analysis of hormonal changes in the liquidators of the Chernobyl accident consequences, which promote the metabolic syndrome X development, was carried out on the base of clinical and experimental data. The system insulin-glucose, the mechanisms of insulinresistance and gyperinslinemia forming, the pathogenetical role of hyperinsulinemia interaction with others hormomes, some aspects of hormone - receptor interaction, an interconnection of hyperinsulinemia and vascular pathology, peculiarities of radiobiological stress as a non-classic adaptive reaction, which frequently resulting with a pathology, were discussed in the article

  14. Changes in the epidemiological pattern of sudden infant death syndrome in southeast Norway, 1984-1998: implications for future prevention and research

    OpenAIRE

    Arnestad, M; Andersen, M.; Vege, A; Rognum, T

    2001-01-01

    AIM—To look for changes in risk factors for sudden infant death syndrome (SIDS) after decrease and stabilisation of the SIDS rate.
METHODS—Questionnaires were distributed to parents of 174 SIDS infants, dying between 1984 and 1998, and 375 age and sex matched controls in southeast Norway.
RESULTS—The proportion of infants sleeping prone has decreased, along with the decrease in SIDS rate for the region during the periods studied, but over half of the SIDS victims are stil...

  15. Changing patterns of Kaposi's sarcoma in Danish acquired immunodeficiency syndrome patients with complete follow-up. The Danish Study Group for HIV Infection (DASHI)

    DEFF Research Database (Denmark)

    Lundgren, Jens Dilling; Melbye, M; Pedersen, C;

    1995-01-01

    The objective was to study changes in the occurrence of human immunodeficiency virus type 1-related Kaposi's sarcoma and the association with degree of immunodeficiency over time. Danish patients with acquired immunodeficiency syndrome (AIDS) diagnosed between 1979 and 1990 (n = 687) were followed...... developing Kaposi's sarcoma after the AIDS diagnosis. Age, antiretroviral therapy, and primary Pneumocystis carinii pneumonia prophylaxis failed to influence the development of Kaposi's sarcoma. Thus, the occurrence of Kaposi's sarcoma remained constant over time but developed later in the course of AIDS and...

  16. Changes in dead space/tidal volume ratio and pulmonary mechanics after surfactant replacement therapy in respiratory distress syndrome of the newborn infants.

    OpenAIRE

    Chung, E. H.; Ko, S. Y.; Kim, I Y; Chang, Y. S.; Park, W. S.

    2001-01-01

    This study was performed to elucidate the mechanism of improved oxygenation after surfactant replacement therapy in respiratory distress syndrome (RDS) of the newborn infants. In 26 newborns with RDS, end tidal-CO2 tension (PetCO2), arterial blood gas analysis and pulmonary function tests were measured at baseline, 30 min, 2 hr and 6 hr after surfactant administration. The changes in dead space/tidal volume ratio (VD/VT ratio=(PaCO2-PetCO2)/PaCO2), oxygenation index and arterial-alveolar part...

  17. Functional and metabolic changes in the brain in neuropathic pain syndrome against the background of chronic epidural electrostimulation of the spinal cord.

    Science.gov (United States)

    Sufianov, A A; Shapkin, A G; Sufianova, G Z; Elishev, V G; Barashin, D A; Berdichevskii, V B; Churkin, S V

    2014-08-01

    Changes in functional and metabolic activities of the brain were evaluated by EEG and positron-emission/computer tomography with 18F-fluorodeoxyglucose in patients with neuropathic pain syndrome previous to and 3 months after implantation of a system for chronic epidural spinal cord stimulation. In most cases, the use of a nerve stimulator was followed by alleviation of neuropathic pain and partial normalization of functional and metabolic activities of brain structures responsible for pain perception, emotiogenic, behavioral, and autonomic responses. PMID:25113605

  18. Histopathological changes in exocrine glands of murine transplantation chimeras. II: Sjögren's syndrome-like exocrinopathy in mice without lupus nephritis. A model of primary Sjögren's syndrome

    DEFF Research Database (Denmark)

    Ussing, Anne Phaff; Prause, J.U.; Sørensen, Inger;

    1992-01-01

    Autoimmune disease, primary Sjögren's syndrome, transplantation chimeras, experimental model, exocrinopathy, inbred mouse strains......Autoimmune disease, primary Sjögren's syndrome, transplantation chimeras, experimental model, exocrinopathy, inbred mouse strains...

  19. Changes in small intestinal chromogranin A-immunoreactive cell densities in patients with irritable bowel syndrome after receiving dietary guidance

    OpenAIRE

    Mazzawi, Tarek; El-Salhy, Magdy

    2016-01-01

    Chromogranin A (CgA) is a common marker for enteroendocrine cells in the gut, and CgA-immunoreactive cell densities are abnormal in patients with irritable bowel syndrome (IBS). The majority of patients with IBS report that their symptoms develop after consuming certain foodstuffs. In the present study, we investigated the effects of dietary guidance on the total enteroendocrine cell densities in the small intestine, as detected by CgA. A total of 14 patients with IBS underwent a gastroscopy ...

  20. Developmental changes in mental rotation ability and visual perspective-taking in children and adults with Williams syndrome

    OpenAIRE

    Hirai, Masahiro; Muramatsu, Yukako; Mizuno, Seiji; Kurahashi, Naoko; Kurahashi, Hirokazu; Nakamura, Miho

    2013-01-01

    Williams syndrome (WS) is a genetic disorder caused by the partial deletion of chromosome 7. Individuals with WS have atypical cognitive abilities, such as hypersociability and compromised visuospatial cognition, although the mechanisms underlying these deficits, as well as the relationship between them, remain unclear. Here, we assessed performance in mental rotation (MR) and level 2 visual perspective taking (VPT2) tasks in individuals with and without WS. Individuals with WS obtained lower...

  1. Joubert Syndrome

    Science.gov (United States)

    ... Awards Enhancing Diversity Find People About NINDS NINDS Joubert Syndrome Information Page Table of Contents (click to ... Organizations Related NINDS Publications and Information What is Joubert Syndrome? Joubert syndrome is a rare brain malformation ...

  2. Marfan Syndrome

    Science.gov (United States)

    Marfan syndrome is a disorder that affects connective tissue. Connective tissues are proteins that support skin, bones, ... fibrillin. A problem with the fibrillin gene causes Marfan syndrome. Marfan syndrome can be mild to severe, ...

  3. Membranous nephropathy in the cat: a clinical and pathological study.

    Science.gov (United States)

    Nash, A S; Wright, N G; Spencer, A J; Thompson, H; Fisher, E W

    1979-07-28

    A series of 13 cases of feline membranous nephropathy is presented. Two groups were distinguished clinically; eight cats had the nephrotic syndrome and five others were in renal failure but not nephrotic. The definitive diagnosis was based on histological, immunofluorescence and ultrastructural examinations of renal tissue obtained at renal biopsy or necropsy. Glomerular lesions were classified according to the degree of glomerular change into three distinct groups; mild, moderately severe and advanced. A relationship was established between the mild and moderately severe groups and cats with the nephrotic syndrome, and the advanced group and cats in renal failure. Diuretic therapy was satisfactory in initial control of oedema in the nephrotic cases. Monitoring of previously nephrotic cats for up to three years indicated that the disease is progressive, although in some cases it is sufficiently slow for a cat to live a relatively normal life without continuing treatment. The prognosis for cats presented in renal failure is hopeless. PMID:552741

  4. Factitious proteinuria - the most dominant feature in a young female patient with Munchausen syndrome.

    Science.gov (United States)

    Lela, Ivana Vuković; Karanović, Sandra; Matišić, Danica; Kuzmanić, Duško; C Orić, Marijana; Šimić, Mirjana; Jelaković, Bojan

    2013-02-01

    Munchausen syndrome is a factitious disorder with predominantly physical signs and symptoms, resulting from the patient's high motivation for assuming a sick role, without any external incentives or boundaries. We report the case of a young female patient with factitious proteinuria in the nephrotic range and a fairly eventful medical history. After performing many expensive and unnecessary investigations and procedures,the real origin of the proteinuria was determined;it was found to be caused by the patient carefully adding calibrated egg albumin to her urine samples. This discovery roused suspicions about multiple, non-corroborated conditions from her history (e.g., multiple miscarriages, breast cancer, and thyroid disorders).The diversity of diseases presented by a single Munchausen patient tends to be bizarre,and thus is a challenge for health care providers to diagnose the condition. Teamwork is therefore of the utmost necessity to diagnose Munchausen syndrome. PMID:23364208

  5. Targeted exome sequencing integrated with clinicopathological information reveals novel and rare mutations in atypical, suspected and unknown cases of Alport syndrome or proteinuria.

    Directory of Open Access Journals (Sweden)

    Rajshekhar Chatterjee

    Full Text Available We applied customized targeted next-generation exome sequencing (NGS to determine if mutations in genes associated with renal malformations, Alport syndrome (AS or nephrotic syndrome are a potential cause of renal abnormalities in patients with equivocal or atypical presentation. We first sequenced 4,041 exons representing 292 kidney disease genes in a Caucasian woman with a history of congenital vesicoureteral reflux (VUR, recurrent urinary tract infections and hydronephrosis who presented with nephrotic range proteinuria at the age of 45. Her biopsy was remarkable for focal segmental glomerulosclerosis (FSGS, a potential complication of longstanding VUR. She had no family history of renal disease. Her proteinuria improved initially, however, several years later she presented with worsening proteinuria and microhematuria. NGS analysis revealed two deleterious COL4A3 mutations, one novel and the other previously reported in AS, and a novel deleterious SALL2 mutation, a gene linked to renal malformations. Pedigree analysis confirmed that COL4A3 mutations were nonallelic and compound heterozygous. The genomic results in conjunction with subsequent abnormal electron microscopy, Collagen IV minor chain immunohistochemistry and progressive sensorineural hearing loss confirmed AS. We then modified our NGS approach to enable more efficient discovery of variants associated with AS or a subset of FSGS by multiplexing targeted exome sequencing of 19 genes associated with AS or FSGS in 14 patients. Using this approach, we found novel or known COL4A3 or COL4A5 mutations in a subset of patients with clinically diagnosed or suspected AS, APOL1 variants associated with FSGS in African Americans and novel mutations in genes associated with nephrotic syndrome. These studies demonstrate the successful application of targeted capture-based exome sequencing to simultaneously evaluate genetic variations in many genes in patients with complex renal phenotypes and

  6. WILLIAMS SYNDROME PREDISPOSES TO VASCULAR STIFFNESS MODIFIED BY ANTI-HYPERTENSIVE USE AND COPY NUMBER CHANGES IN NCF1

    Science.gov (United States)

    Kozel, Beth A.; Danback, Joshua; Waxler, Jessica; Knutsen, Russell H.; Fuentes, Lisa de las; Reusz, Gyorgy S.; Kis, Eva; Bhatt, Ami; Pober, Barbara R

    2014-01-01

    Williams syndrome, is caused by the deletion of 26-28 genes, including elastin, on human chromosome 7. Elastin insufficiency leads to the cardiovascular hallmarks of this condition, namely focal stenosis and hypertension. Extrapolation from the Eln+/− mouse suggests that affected persons may also have stiff vasculature, a risk factor for stroke, myocardial infarction and cardiac death. NCF1, one of the variably deleted Williams genes, is a component of the NAD(P)H oxidase complex and is involved in the generation of oxidative stress, making it an interesting candidate modifier for vascular stiffness. Using a case-control design, vascular stiffness was evaluated by pulse wave velocity in 77 Williams cases and matched controls. Cases had stiffer conducting vessels than controls (p<0.001), with increased stiffness observed in even the youngest Williams children. Pulse wave velocity increased with age at comparable rates in cases and controls and, although the degree of vascular stiffness varied, it was seen in both hypertensive and normotensive Williams participants. Use of anti-hypertension medication and extension of the Williams deletion to include NCF1 were associated with protection from vascular stiffness. These findings demonstrate that vascular stiffness is a primary vascular phenotype in Williams syndrome and that treatment with anti-hypertensives and/or agents inhibiting oxidative stress may be important in managing patients with this condition, potentially even those who are not overtly hypertensive. PMID:24126171

  7. Dynamic changes of pulmonary lesions on CT in patients with severe acute respiratory syndrome treated by non-invasive positive pressure ventilation

    International Nuclear Information System (INIS)

    Objective: To study the dynamic changes of pulmonary lesions of severe acute respiratory syndrome (SARS) treated by non-invasive positive pressure ventilation (NIPPV) on CT. Methods: Serial chest CT images in 18 patients with SARS were retrospectively analyzed before and after NIPPV (4-12 days), and a long-term follow-up (3-36 months) examination by CT was carried out. And 27 SARS cases without NIPPV were enrolled as the control group. Results: (1) There were dynamic changes in 15 cases of 18 cases: partial multilobar consolidations of bilateral lungs changed into ground-glass opacities in 7 of 18 cases, a circular pulmonary emphysema around bronchial vascular strains of the lower lobes was seen in 5 of 18, subpleural pulmonary emphysema was seen in 8 of 18, pulmonary barotraumas were showed in 2 of 18. Residual of subpleural curvilinear shadow related to NIPPV was found in 2 eases on the follow-up scan. (2) Above changes on CT were not found in 27 cases without NIPPV. Conclusion: There were changes of pulmonary lesions of SARS on CT related to NIPPV treatment, It is beneficial to assess the CT images in the NIPPV treatment of the related pneumonia in SARS. (authors)

  8. Electroacupuncture at Tianshu(ST 25)for diarrhea-predominant irritable bowel syndrome using positron emission tomography Changes in visceral sensation center

    Institute of Scientific and Technical Information of China (English)

    Huirong Liu; Enhua Zhou; Huangan Wu; Li Qi; Xiaolong Wang; Yihui Guan; Chuantao Zuo; Linying Tan; Lingsong Yuan; Xiaopeng Ma; Xiaomei Wang

    2010-01-01

    Previous studies have demonstrated that electroacupuncture therapy is effective in the treatment of irritable bowel syndrome.However,the precise mechanism of this therapy is unknown.The present study served to investigate the effects of electroacupuncture therapy on treatment of patients with diarrhea-predominant irritable bowel syndrome(IBS).We compared brain activation maps based on the changes of cerebral glucose metabolism obtained by 18-fluorodeoxyglucose positron emission tomography scanning under three conditions: resting,rectal balloon distension and rectal balloon distension plus electroacupuncture.Under the resting condition,compared with healthy controls,IBS patients displayed an increasing regional cerebral metabolic rate of glucose over a wide range:bilateral superior temporal gyrus,right middle occipital gyrus,superior frontal gyrus and bilateral middle frontal gyrus.However,there was no significant activity in the visceral pain center.Compared with the resting condition,under the rectal balloon distension condition,patients with IBS had a greater regional cerebral metabolic rate of glucose in the prefrontal cortex,left anterior cingulate cortex,postcentral gyrus,precentral gyrus and temporal gyrus.Under the rectal balloon distension plus electroacupuncture condition,stimulation by electroacupuncture at Tianshu(ST 25)manifested a decreased regional cerebral metabolic rate of glucose in the left cingulate gyrus,right insula,right caudate nucleus,fusiform gyrus and hippocampal gyrus.Electroacupuncture therapy relieved abdominal pain,distension or discomfort by decreasing glucose metabolism in the brain.

  9. Hyperacute cognitive stroke syndromes.

    Science.gov (United States)

    Ferro, J M

    2001-10-01

    Cognitive syndromes are common clinical manifestations of hyperacute stroke and may be the single or dominant presenting features. They are related to acute dysfunction of complex integrated distributed functional networks serving different cognitive domains. The most common cortical syndromes include nonfluent or fluent aphasia, neglect, collor agnosia, pure alexia and Balint's syndrome. Disturbances of declarative memory are common following posterior cerebral artery and thalamic strokes. Abulia can follow thalamic, caudate and capsular lesions. Intraventricular and subarachnoid haemorrhages can cause preeminent neuropsychological changes. Disorientation is present in about 40% of acute stroke patients and delirium complicates the course of 25% of acute strokes. Some hyperacute cognitive stroke syndromes are useful indicators of later disability. Cognitive syndromes may pose special difficulties to neurology residents, unless formal teaching in neuropsychology and psychiatry is included in their training programs. PMID:11697519

  10. Changes in left ventricular systolic function in patients with chronic heart failure with preserved ejection fraction and cardiorenal anemic syndrome

    Directory of Open Access Journals (Sweden)

    Vasylenko V.A.

    2015-09-01

    Full Text Available The feature of chronic heart failure (CHF in elderly people is increasing incidence of heart failure with preserved left ventricular ejection fraction (LVEF which is associated with age. Such patients account for almost half of the total number of patients with heart failure. Cardiorenal syndrome (CRS is associated with an increased risk of mortality in patients with CHF. The impact of CRS on the structural and functional condition of the heart in these patients is studied insufficiently. The study involved 103 patients with CHF II-IV NYHA with preserved LVEF (>45% and CRS (hemoglobin <120 g/l and

  11. Disease: H00626 [KEGG MEDICUS

    Lifescience Database Archive (English)

    Full Text Available H00626 Nephrotic syndrome and focal segmental glomerulosclerosis Congenital nephrotic...256300 600995 256370 610725 PMID:21691316 (gene, description) Gubler MC Nephrotic syndrome: Genetic testing in steroid-resistant neph...rotic syndrome. Nat Rev Nephrol 7:430-1 (2011) PMID:20969579 (gene) Benoit G, Machu

  12. Effects of a healthy Nordic diet on gene expression changes in peripheral blood mononuclear cells in response to an oral glucose tolerance test in subjects with metabolic syndrome

    DEFF Research Database (Denmark)

    Leder, Lena; Kolehmainen, Marjukka; Narverud, Ingunn;

    2016-01-01

    BACKGROUND: Diet has a great impact on the risk of developing features of metabolic syndrome (MetS), type 2 diabetes mellitus (T2DM), and cardiovascular diseases (CVD). We evaluated whether a long-term healthy Nordic diet (ND) can modify the expression of inflammation and lipid metabolism......-related genes in peripheral blood mononuclear cells (PBMCs) during a 2-h oral glucose tolerance test (OGTT) in individuals with MetS. METHODS: A Nordic multicenter randomized dietary study included subjects (n = 213) with MetS, randomized to a ND group or a control diet (CD) group applying an isocaloric study...... the mRNA gene expression analysis was measured by quantitative real-time polymerase chain reaction (qPCR). We analyzed the mRNA expression changes of 44 genes before and after a 2hOGTT at the beginning and the end of the intervention. RESULTS: The healthy ND significantly down-regulated the expression...

  13. Longitudinal Magnetic Resonance Imaging (MRI) Analysis of the Developmental Changes of Tourette Syndrome Reveal Reduced Diffusion in the Cortico-Striato-Thalamo-Cortical Pathways

    DEFF Research Database (Denmark)

    Debes, Nanette; Jeppesen, Signe; Raghava, Jayachandra Mitta;

    2015-01-01

    There is evidence that cortico-striato-thalamo-cortical pathways are involved in Tourette syndrome. We performed a longitudinal imaging study in 22 patients and 21 healthy controls in order to examine the development of tics and its correlation with magnetic resonance imaging (MRI) findings....... Patients were divided in a group with persisting and a group with remission of tics. We found a decrease in volume of left putamen in controls, but not in patients. We found changes in mean diffusivity between patients and controls in right caudate nucleus, thalamus, and frontal lobe. In contrast to...... controls, parallel and perpendicular diffusivity decreased in patients and were most pronounced in the patients with persisting tics compared to those with remission. The findings suggest that the development of the brain in patients with remission resembles the normal development more than in patients...

  14. Transcriptional changes in response to X chromosome dosage in the mouse: implications for X inactivation and the molecular basis of Turner Syndrome

    Directory of Open Access Journals (Sweden)

    Sargent Carole A

    2010-02-01

    Full Text Available Abstract Background X monosomic mice (39,XO have a remarkably mild phenotype when compared to women with Turner syndrome (45,XO. The generally accepted hypothesis to explain this discrepancy is that the number of genes on the mouse X chromosome which escape X inactivation, and thus are expressed at higher levels in females, is very small. However this hypothesis has never been tested and only a small number of genes have been assayed for their X-inactivation status in the mouse. We performed a global expression analysis in four somatic tissues (brain, liver, kidney and muscle of adult 40,XX and 39,XO mice using the Illumina Mouse WG-6 v1_1 Expression BeadChip and an extensive validation by quantitative real time PCR, in order to identify which genes are expressed from both X chromosomes. Results We identified several genes on the X chromosome which are overexpressed in XX females, including those previously reported as escaping X inactivation, as well as new candidates. However, the results obtained by microarray and qPCR were not fully concordant, illustrating the difficulty in ascertaining modest fold changes, such as those expected for genes escaping X inactivation. Remarkably, considerable variation was observed between tissues, suggesting that inactivation patterns may be tissue-dependent. Our analysis also exposed several autosomal genes involved in mitochondrial metabolism and in protein translation which are differentially expressed between XX and XO mice, revealing secondary transcriptional changes to the alteration in X chromosome dosage. Conclusions Our results support the prediction that the mouse inactive X chromosome is largely silent, while providing a list of the genes potentially escaping X inactivation in rodents. Although the lower expression of X-linked genes in XO mice may not be relevant in the particular tissues/systems which are affected in human X chromosome monosomy, genes deregulated in XO mice are good candidates for

  15. What Causes Prader-Willi Syndrome?

    Science.gov (United States)

    ... Research Information Clinical Trials Resources and Publications What causes Prader-Willi syndrome (PWS)? Skip sharing on social ... from parent to child. The genetic changes that cause Prader-Willi syndrome occur in a portion of ...

  16. Genetics Home Reference: Wolf-Hirschhorn syndrome

    Science.gov (United States)

    ... 4 . This chromosomal change is sometimes written as 4p-. The size of the deletion varies among affected ... Gene Review: Wolf-Hirschhorn Syndrome Genetic Testing Registry: 4p partial monosomy syndrome MedlinePlus Encyclopedia: Epilepsy These resources ...

  17. Hepatorenal syndrome

    Institute of Scientific and Technical Information of China (English)

    Jan Lata

    2012-01-01

    Hepatorenal syndrome (HRS) is defined as a functional renal failure in patients with liver disease with portal hypertension and it constitutes the climax of systemic circulatory changes associated with portal hypertension.This term refers to a precisely specified syndrome featuring in particular morphologically intact kidneys,where regulatory mechanisms have minimised glomerular filtration and maximised tubular resorption and urine concentration,which ultimately results in uraemia.The syndrome occurs almost exclusively in patients with ascites.Type 1 HRS develops as a consequence of a severe reduction of effective circulating volume due to both an extreme splanchnic arterial vasodilatation and a reduction of cardiac output.Type 2 HRS is characterised by a stable or slowly progressive renal failure so that its main clinical consequence is not acute renal failure,but refractory ascites,and its impact on prognosis is less negative.Liver transplantation is the most appropriate therapeutic method,nevertheless,only a few patients can receive it.The most suitable "bridge treatments" or treatment for patients ineligible for a liver transplant include terlipressin plus albumin.Terlipressin is at an initial dose of 0.5-1 mg every 4 h by intravenous bolus to 3 mg every 4 h in cases when there is no response.Renal function recovery can be achieved in less than 50% of patients and a considerable decrease in renal function may reoccur even in patients who have been responding to therapy over the short term.Transjugular intrahepatic portosystemic shunt plays only a marginal role in the treatment of HRS.

  18. The cerebro-morphological fingerprint of a progeroid syndrome: white matter changes correlate with neurological symptoms in xeroderma pigmentosum.

    Directory of Open Access Journals (Sweden)

    Jan Kassubek

    Full Text Available BACKGROUND: Xeroderma pigmentosum (XP is a rare autosomal recessive progeroid syndrome. It has recently been shown that the underlying DNA repair defect plays a central role in the aging process. In addition to skin symptoms, various premature neurological abnormalities have been reported. METHODOLOGY/PRINCIPAL FINDINGS: We present the clinical neurological phenotype in 14 XP patients (seven subtypes, in seven of these patients together with conventional and multiparametric advanced MRI data to assess the macrostructural and microstructural cerebral morphology in comparison to controls, including volumetric measurements, MR spectroscopy ((1H MRS, and diffusion tensor imaging (DTI. Clinical hallmarks were spinocerebellar ataxia, pyramidal tract signs, and mild cognitive deficits. DTI demonstrated significantly reduced WM directionality in all regions investigated, i.e. the thalamus, the corticospinal tracts and the dorsal corpus callosum. Single patients showed a marked relative hippocampal volume reduction, but the patients were not different from controls in the volumetric measurements of hippocampal and whole brain volumes at group level. However, (1H MRS demonstrated that the hippocampal formation was metabolically altered. CONCLUSIONS: The most prominent feature was the white matter affectation, as assessed by DTI, with volume and directionality reductions of the fiber projections involving both the craniocaudal fibers and the interhemispheric connections. These findings, although heterogeneous among the study sample, could be correlated with the clinico-neurological symptoms. The imaging findings support the position that myelin structures degrade prematurely in the brain of XP patients.

  19. A study of the changes of Th17 cells and IL-23 in patients with Guillain-Barré syndrome

    Directory of Open Access Journals (Sweden)

    Gai-ping HE

    2014-06-01

    Full Text Available Guillain-Barré syndrome (GBS is an acute autoimmune-mediated inflammatory neuropathy. In this paper, the percentage of Th17 in peripheral blood lymphocyte and the level of IL-23 in serum and cerebrospinal fluid (CSF of patients with GBS were detected. Twenty-five patients with GBS and 20 controls were analyzed. The percentage of Th17 cells in patients with GBS was (4.86 ± 0.60%, which was higher than that in controls [(2.71 ± 0.54%; t = 2.616, P = 0.012]. Serum IL-23 increased in patients with GBS [(111.80 ± 13.84 pg/ml vs (72.55 ± 8.19 pg/ml; t = 2.300, P = 0.026]. The level of IL-23 in serum and CSF from patients with GBS before therapy [(110.50 ± 15.66 and (102.30 ± 7.52 pg/ml] declined to normal after therapy [(74.13 ± 6.18 and (72.92 ± 12.09 pg/ml], and the difference was statistically significant (t = 2.557, P = 0.022; t = 1.422, P = 0.046. Th17 cells and IL-23 increased in patients with GBS, indicating that they may involve in the pathogenesis of GBS. doi: 10.3969/j.issn.1672-6731.2014.06.012

  20. Cognitive rehabilitation in a child with Joubert Syndrome: Developmental trends and adaptive changes in a single case report.

    Science.gov (United States)

    Gagliardi, Chiara; Brenna, Viola; Romaniello, Romina; Arrigoni, Filippo; Tavano, Alessandro; Romani, Marta; Valente, Enza Maria; Borgatti, Renato

    2015-12-01

    We report the clinical and rehabilitative follow up of M, a female child carrying a compound heterozygous pathogenic mutations in the TCTN1 gene and affected by Joubert Syndrome (JS). JS is a congenital cerebellar ataxia characterized by "the molar tooth sign" on axial MRI, a pathognomonic neuroradiological malformation involving the cerebellum and brainstem. JS presents with high phenotypic/cognitive variability, and little is known about cognitive rehabilitation programs. We describe the therapeutic settings, intensive rehabilitation targets and outcome indexes in M's cognitive development. Using a single case evidence-based approach, we attempt to distinguish the effectiveness of the intervention from the overall developmental trend. We assume that an adequate amount of focused, goal directed treatment in a relative short period of time can be at least as effective as one provided in longer time, and much less interfering with the child's everyday life. We conclude by discussing specific issues in cognitive development and rehabilitation in JS and, more broadly, in cerebellar malformations. PMID:26489806

  1. Changes in the folding landscape of the WW domain provide a molecular mechanism for an inherited genetic syndrome

    Science.gov (United States)

    Pucheta-Martinez, Encarna; D’Amelio, Nicola; Lelli, Moreno; Martinez-Torrecuadrada, Jorge L.; Sudol, Marius; Saladino, Giorgio; Gervasio, Francesco Luigi

    2016-07-01

    WW domains are small domains present in many human proteins with a wide array of functions and acting through the recognition of proline-rich sequences. The WW domain belonging to polyglutamine tract-binding protein 1 (PQBP1) is of particular interest due to its direct involvement in several X chromosome-linked intellectual disabilities, including Golabi-Ito-Hall (GIH) syndrome, where a single point mutation (Y65C) correlates with the development of the disease. The mutant cannot bind to its natural ligand WBP11, which regulates mRNA processing. In this work we use high-field high-resolution NMR and enhanced sampling molecular dynamics simulations to gain insight into the molecular causes the disease. We find that the wild type protein is partially unfolded exchanging among multiple beta-strand-like conformations in solution. The Y65C mutation further destabilizes the residual fold and primes the protein for the formation of a disulphide bridge, which could be at the origin of the loss of function.

  2. Changing spectrum of Zimmerman-Laband syndrome: a six years follow up case report of a family

    Directory of Open Access Journals (Sweden)

    Suryakanta Narendra

    2015-11-01

    Full Text Available The Zimmermann-Laband syndrome (ZLS is a rare genetic disorder inherited as an autosomal dominant fashion, with clinical characters like, gingival fibromatosis, bulbous soft nose, thick floppy ears, nail dysplasia, joint hyperextensibility, hepatosplenomegaly, skeletal anomalies and occasional mental retardation. Idiopathic gingival enlargement is a hereditary condition; it can be expressed as autosomal dominant inheritance. Here association of Idiopathic gingival enlargement has been reported in a family, with an autosomal dominant inheritance diagnosed to be ZLS. Other clinical features associated are, hypertrichosis, bulbous soft nose, thick floppy ears, nail dysplasia, joint hyper extensibility, bimaxillary protrusion and enlarged palm and fingers with flat feet. The unusual clinical presentations of massive gingival fibromatosis, unusual length of upper limbs and bimaxillary protrusion supported the variable spectrum of phenotype expression of the ZLS. The Biopsy report confirmed the diagnosis of gingival fibromatosis. Gingivectomy was carried out in all four quadrants for exposing the natural teeth and to bring back the original contour of the gingiva. But there was recurrence of the enlargement found to be associated during 6 years follow up. [Int J Res Med Sci 2015; 3(11.000: 3402-3405

  3. A Controlled Study on the Psychological Characteristics of Nephritic Syndrome%肾病综合征患儿的个性、行为及社会适应能力对照研究

    Institute of Scientific and Technical Information of China (English)

    戴文献; 王广吉; 齐娅贤

    2003-01-01

    Objective: To understand the psychological characteristics of nephrotic syndrome. Methods: The personality charac-teristics, intelligence and social adaptive behavior(SAB) of 67 moderate to severe nephrotie syndrome aged 7 - 14 years were mea-sured by means of EPQ, CBCL, CRT and SAB scales. Results: The EPQ - N score of nephritic syndrome group was higher than that of control group( P 0.05). The nephritic group got a significantly lower social/self direction factor score than the control group.Conclusion:The results showed that nephritic syndrome possessed personality characteristics of neurotieism and obvious behavior problems;and their social adaptive behavior, particularly social/self- direction factor, was significantly lower than that of normal children.

  4. Cushing's Syndrome

    Science.gov (United States)

    ... Cushing's syndrome, also called hypercortisolism , is a rare endocrine disorder caused by chronic exposure of the body's tissues ... removing the tumor while minimizing the chance of endocrine deficiency or long-term ... for Cushing's Syndrome Clinical Trials ...

  5. Turner Syndrome

    Science.gov (United States)

    Turner syndrome is a genetic disorder that affects a girl's development. The cause is a missing or incomplete ... t work properly. Other physical features typical of Turner syndrome are Short, "webbed" neck with folds of skin ...

  6. Metabolic Syndrome

    Science.gov (United States)

    Metabolic syndrome is a group of conditions that put you at risk for heart disease and diabetes. These ... doctors agree on the definition or cause of metabolic syndrome. The cause might be insulin resistance. Insulin is ...

  7. Asperger syndrome

    Science.gov (United States)

    Asperger syndrome is often considered a high functioning form of autism. It can lead to difficulty interacting socially, repeat behaviors, and clumsiness. Asperger syndrome is a part of the larger developmental disorder ...

  8. Pseudoaminopterin syndrome.

    Science.gov (United States)

    Kraoua, Lilia; Capri, Yline; Perrin, Laurence; Benmansour, Abdelmajjid; Verloes, Alain

    2012-09-01

    Pseudoaminopterin syndrome or aminopterin syndrome-like sine aminopterin (ASSA syndrome--OMIM 600325] is a rare autosomal recessive syndrome defined by characteristic dysmorphic features, skeletal defects, limb anomalies, cryptorchidism, and growth retardation. The syndrome owes its name to the fact that patients resemble the children exposed to aminopterin or to methotrexate, two dihydrofolate reductase inhibitors used for chemotherapy, or as an abortificient in early pregnancy. Ten patients have been described with pseudoaminopterin syndrome. Their phenotype is variable, and differs from the phenotype resulting from folic acid deprivation, leading to the notion that the pathogenesis may be more complex than simple vitamin deficiency. We report on an Algerian patient with pseudoaminopterin syndrome, review the previously reported cases and confirm that pseudoaminopterin syndrome does not result from a detectable contiguous gene imbalance as high resolution CGH array was normal in this child. PMID:22811276

  9. Usher Syndrome

    Science.gov (United States)

    Usher syndrome is an inherited disease that causes serious hearing loss and retinitis pigmentosa, an eye disorder ... hearing and vision. There are three types of Usher syndrome: People with type I are deaf from ...

  10. Turner Syndrome

    Science.gov (United States)

    Turner syndrome is a genetic disorder that affects a girl's development. The cause is a missing or ... t work properly. Other physical features typical of Turner syndrome are Short, "webbed" neck with folds of ...

  11. Proteus Syndrome

    Science.gov (United States)

    ... Gift Stock Gift Sunshine Society Contact Privacy Policy Proteus Syndrome Definition Common Signs Diagnostic Criteria (I have ... NIH to go with this criteria) Glossary Videos Proteus Syndrome is a condition which involves atypical growth ...

  12. LEOPARD syndrome is not linked to the Marfan syndrome and the Watson syndrome loci

    Energy Technology Data Exchange (ETDEWEB)

    Rass-Rothchild, A.: Abeliovitch, D.; Kornstein, A. [Tel Aviv Univ. (Israel)]|[Hebrew Univ., Jerusalem (Israel)

    1994-09-01

    The acronym LEOPARD stands for a syndromic association of Lentigines, Eletrocardiographic changes, Ocular hypertelorism, Pulmonic stenosis, Abnormal genitalia, Retardation of growth and sensorineural Deafness. Inheritance is autosomal dominant with high penetrance and variable expressivity. In 1990 Torok et al. reported on the association of LEOPARD and Marfan syndrome. In addition a clinical similarity (cardiac and cutaneous involvement) exists with the Watson syndrome (neurofibromatosis and pulmonic stenosis) which is linked to the marker D17S33 on chromosome 17. We studied possible linkage of LEOPARD syndrome to the Marfan syndrome locus on chromosome 15 (D15S1, MF13, and (TAAAA)n repeats) and to the NF-1 locus on chromosome 17 in a family with 9 cases of LEOPARD syndrome. Close linkage between LEOPARD syndrome and both the Marfan locus on chromosome 15 and the NF-1 locus on chromosome 17 was excluded (lod score <-2.0 through {theta} = 0.1).

  13. Learning about Marfan Syndrome

    Science.gov (United States)

    ... genetic terms used on this page Learning About Marfan Syndrome What is Marfan syndrome? What are the ... Syndrome Additional Resources for Marfan Syndrome What is Marfan syndrome? Marfan syndrome is one of the most ...

  14. Changes of CD4+CD25+Regulatory T Cells in Patients with Acute Coronary Syndrome and the Effects of Atorvastatin

    Institute of Scientific and Technical Information of China (English)

    HU Zhenping; LI Dazhu; HU Yingfeng; YANG Keping

    2007-01-01

    The function of CD4+CD25+regulatory T lymphocytes (Treg) in patients with acute coronary syndrome (ACS) and the effects of atorvastatin were investigated. Forty-eight patients with ACS were randomly divided into two groups: group C receiving conventional therapy (n=24), and group C+A receiving conventional therapy+atorvastatin (10 mg/day, n=24). T lymphocytes from ACS patients (before and 2 weeks after the treatment) or 18 healthy subjects were separated and the flow cytometry was used to measure the percentage of Treg. The inhibitory ability of Treg on effector T cells was determined by mixed lymphocyte reaction (MLR). ELISA was used to measure the serum levels of cytokines (IL-10, TGF-β1 and IFN-γ) before and after treatment. The results showed that as compared with normal control group, Treg percentage was decreased significantly (P<0.01), the in- hibitory ability of Treg on the T lymphocytes proliferation was reduced (P<0.01), IFN-γ, levels were increased and IL-10 and TGF-β1 levels were lowered in ACS patients. After treatment with atorvas- tatin, Treg percentage and the inhibitory ability of Treg on T lymphocytes proliferation were signifi- cantly increased in ACS patients. Serum IFN-γ, was decreased significantly, while IL-10 and TGF-β1 were elevated significantly as compared with the non-atorvastatin group. The number of Treg was positively correlated with serum TGF-β1, but negatively with serum IFN-γ and CRP. It was concluded that ACS was associated with decreased number and defected function of Treg, which may play an important role in initiating immune-inflammatory response in ACS. The inhibitory ef- fects of atorvastatin on inflammation in ACS may be due to its beneficial effects on Treg and restora- tion of immune homeostasis.

  15. Mathematical model describing erythrocyte sedimentation rate. Implications for blood viscosity changes in traumatic shock and crush syndrome

    Directory of Open Access Journals (Sweden)

    Shevchuk Nikolai A

    2005-04-01

    Full Text Available Abstract Background The erythrocyte sedimentation rate (ESR is a simple and inexpensive laboratory test, which is widespread in clinical practice, for assessing the inflammatory or acute response. This work addresses the theoretical and experimental investigation of sedimentation a single and multiple particles in homogeneous and heterogeneous (multiphase medium, as it relates to their internal structure (aggregation of solid or deformed particles. Methods The equation system has been solved numerically. To choose finite analogs of derivatives we used the schemes of directional differences. Results (1 Our model takes into account the influence of the vessel wall on group aggregation of particles in tubes as well as the effects of rotation of particles, the constraint coefficient, and viscosity of a mixture as a function of the volume fraction. (2 This model can describe ESR as a function of the velocity of adhesion of erythrocytes; (3 Determination of the ESR is best conducted at certain time intervals, i.e. in a series of periods not exceeding 5 minutes each; (4 Differential diagnosis of various diseases by means of ESR should be performed using the aforementioned timed measurement of ESR; (5 An increase in blood viscosity during trauma results from an increase in rouleaux formation and the time-course method of ESR will be useful in patients with trauma, in particular, with traumatic shock and crush syndrome. Conclusion The mathematical model created in this study used the most fundamental differential equations that have ever been derived to estimate ESR. It may further our understanding of its complex mechanism.

  16. Alagille syndrome.

    OpenAIRE

    Krantz, I D; Piccoli, D A; Spinner, N B

    1997-01-01

    Alagille syndrome (OMIM 118450) is an autosomal dominant disorder associated with abnormalities of the liver, heart, eye, skeleton, and a characteristic facial appearance. Also referred to as the Alagille-Watson syndrome, syndromic bile duct paucity, and arteriohepatic dysplasia, it is a significant cause of neonatal jaundice and cholestasis in older children. In the fully expressed syndrome, affected subjects have intrahepatic bile duct paucity and cholestasis, in conjunction with cardiac ma...

  17. Cushing Syndrome

    Science.gov (United States)

    ... links Share this: Page Content What is Cushing’s syndrome? Cushing’s syndrome is a condition that occurs when the body’s ... medication or as a result of a tumor, Cushing’s syndrome can develop. Many factors influence whether this happens, ...

  18. Dumping Syndrome

    Science.gov (United States)

    ... Disease Organizations​​ (PDF, 341 KB)​​​​​ Alternate Language URL Dumping Syndrome Page Content On this page: What is ... Nutrition Points to Remember Clinical Trials What is dumping syndrome? Dumping syndrome occurs when food, especially sugar, ...

  19. [Psychological changes in intensive care patients with acute Guillain-Barré syndrome--psychoanalytic aspects of loss of communication and adjustment].

    Science.gov (United States)

    Weiss, H

    1991-04-01

    Ten patients who had been hospitalized with acute Guillain-Barré-syndrome (GBS) were monitored during their course of treatment and were asked in short intervals through semi-structured interviews how they experienced their illness. States of anxiety were especially evident at the initial phase of the disease, during the dissemination and maximum intensity of paralysis. In contrast, depressive symptoms were primarily noticeable during the phase of remission. As a rule, the degree of anxiety correlated in intensity and duration with the degree of severity of the neurological deficit. Five patients experienced a temporary derealization, among those, three patients showed productive-psychotic symptoms (optical and acoustical hallucinations, delusional reactions). Frequently, dreams were reported, which were associated with elementary experiences of anxiety and in part took on an overwhelming realistic character. Finally, the psychic changes are interpreted in context with the extreme condition of the disease which does not only signify a situation of forced dependence and regression for the patient but also results in--through loss of mobility and communication (cranial nerve dysfunction, artificial respiration)--a fundamental change in the perception of reality. PMID:2055586

  20. Two cases of cervical disc disease with intramedullary pathological changes, which are responsible for their neurological syndromes, on delayed CT myelography

    International Nuclear Information System (INIS)

    We report two cases of cervical disc disease with myelopathy classified as of motor system syndrome type showing small contrast accumulation within the spinal cord on delayed CT myelography. In our two cases, high density spots on delayed CT myelography were bilaterally localized within the spinal cord, and believed represent pathological changes of the spinal cord, such as collection of microcavities or cystic necrosis. In case 1, the high density areas seemed to be localized in the anterior horn and corticospinal tract, and in case 2, they seemed to be localized in the corticospinal tract. The patient in case 1 produced signs and symptoms resembling motor neurone disease and lesion could not be differentiated from the latter. Delayed CT myelography showed that the cause of the upper limb amyotrophy was attributed to an anterior horn disorder and that of pyramidal tract sign to a corticospinal tract disorder. Therefore, we could differentiate the lesion from motor neurone disease on delayed CT myelography in case 1. In conclusion, we emphasize that delayed CT myelography can demonstrate the intramedullary pathological changes in the cervical disc disease and is useful in distinguishing between cervical disc disease simulating motor neurone disease and the latter. (author)

  1. Clinical significance of determination of changes of serum TNF-α levels, peripheral B lymphocyte count and T lymphocyte subsets distribution pattern in patients with pregnancy induced hypertension syndrome

    International Nuclear Information System (INIS)

    Objective: To explore the changes of serum TNF-α levels, peripheral B cell count and T subsets distribution pattern in patients with pregnancy induced hypertension syndrome. Methods: Serum TNF-α levels (with RIA), peripheral B cell count as well as T subsets (with monoclonal technique) were examined in 34 patients with pregnancy induced hypertension syndrome and 35 controls. Results: The serum TNF-α levels and B lymphocytes count were significantly higher than those in controls (P3, CD4, CD4/CD8 ratio were significantly lower than those in controls (P<0.01). Conclusion: Pregnancy induced hY- pertension syndrome is a kind of autoimmune diseases with abnormal immunoregulation. (authors)

  2. Metabolic syndrome predicts vascular changes in whole body magnetic resonance imaging in patients with long standing diabetes mellitus

    Directory of Open Access Journals (Sweden)

    Findeisen Hannes M

    2010-08-01

    Full Text Available Abstract Background Although diabetic patients have an increased rate of cardio-vascular events, there is considerable heterogeneity with respect to cardiovascular risk, requiring new approaches to individual cardiovascular risk factor assessment. In this study we used whole body-MR-angiography (WB-MRA to assess the degree of atherosclerosis in patients with long-standing diabetes and to determine the association between metabolic syndrome (MetS and atherosclerotic burden. Methods Long standing (≥10 years type 1 and type 2 diabetic patients (n = 59; 31 males; 63.3 ± 1.7 years were examined by WB-MRA. Based on the findings in each vessel, we developed an overall score representing the patient's vascular atherosclerotic burden (MRI-score. The score's association with components of the MetS was assessed. Results The median MRI-score was 1.18 [range: 1.00-2.41] and MetS was present in 58% of the cohort (type 2 diabetics: 73%; type 1 diabetics: 26%. Age (p = 0.0002, HDL-cholesterol (p = 0.016, hypertension (p = 0.0008, nephropathy (p = 0.0093, CHD (p = 0.001 and MetS (p = 0.0011 were significantly associated with the score. Adjusted for age and sex, the score was significantly (p = 0.02 higher in diabetics with MetS (1.450 [1.328-1.572] compared to those without MetS (1.108 [0.966-1.50]. The number of MetS components was associated with a linear increase in the MRI-score (increase in score: 0.09/MetS component; r2 = 0.24, p = 0.038. Finally, using an established risk algorithm, we found a significant association between MRI-score and 10-year risk for CHD, fatal CHD and stroke. Conclusion In this high-risk diabetic population, WB-MRA revealed large heterogeneity in the degree of systemic atherosclerosis. Presence and number of traits of the MetS are associated with the extent of atherosclerotic burden. These results support the perspective that diabetic patients are a heterogeneous population with increased but varying prevalence of atherosclerosis

  3. Dynamic Changes, Cut-Off Points, Sensitivity, and Specificity of Laboratory Data to Differentiate Macrophage Activation Syndrome from Active Disease

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    Raheleh Assari

    2015-01-01

    Full Text Available Purpose. To compare the laboratory data and changes in these data between patients with MAS and patients with flare-up of the autoimmune diseases. Methods. In a prospective study, the static laboratory data and dynamic changes in the selected data in 17 consecutive patients with MAS and 53 patients with active disease of SJIA, PJIA, Kawasaki disease, and SLE were compared. The ROC curve analysis was used to evaluate cut-off points, sensitivity, and specificity of the static and dynamic laboratory data to differentiate between MAS and active disease. Results. In the MAS group, the mean CRP3, ALT, AST, total bilirubin, ferritin, LDH, PT, PTT, and INR were significantly higher and the mean WBC2, PMN2, Lymph2, Hgb1, 2, 3, ESR2, serum albumin, and sodium were significantly lower than in control group. Some of the important cut-off points were PLT2 38.5, ALT > 38, WBC 5277 ng/mL. Conclusion. The dynamic changes in some laboratory data, especially PLT, can differentiate between MAS and active disease. The changes in WBC, PMN, and ESR and the levels of the liver enzymes may also be helpful in the early differentiation. Very high levels of ferritin may also help the diagnosis along with other clinical and laboratory signs.

  4. Metabolic syndrome in patients with systemic lupus erythematosus: association with traditional risk factors for coronary heart disease and lupus characteristics.

    Science.gov (United States)

    Telles, Rw; Lanna, Ccd; Ferreira, Ga; Ribeiro, Al

    2010-06-01

    The objective of this study was to determine the frequency of Metabolic Syndrome (MetS) in patients with SLE and to analyze the association of MetS with traditional risk factors for CHD and lupus characteristics. In this cross-sectional study the frequency of MetS was determined according to the National Cholesterol Education Program Adult Treatment Panel III in patients with SLE. The association of MetS with the traditional risk factors for CHD not included in the syndrome definition, and with lupus characteristics was examined. The mean age (sd) of the 162 females patients was 38.8(11.2) years. The frequency of MetS was 32.1%. Abdominal obesity and hypertension were the two most common components of the syndrome (86.5% each) followed by low levels of HDL-cholesterol (84.6%), hypertriglyceridemia (69.2%) and hyperglycemia (15.4%). MetS was significantly associated with older age, family history of CHD, obesity, postmenopausal status, LDL-c > or =100mg/dl, and higher Framingham risk score. Lupus characteristics associated with MetS were history of nephrotic proteinuria during follow-up and current cyclophosphamide use, higher modified SLEDAI-2k, higher damage index score (SLICC/ACR), and older age at lupus diagnosis. In the logistic regression analysis, obesity, LDL-c > or =100mg/dl, older age at lupus diagnosis, higher damage index and nephrotic proteinuria were independently associated with MetS. We conclude that MetS diagnosis was frequent in patients with lupus. The syndrome was associated not only with traditional risk factors for CHD, confirming the clustering of those risk factors, but also with lupus characteristics. Some of those factors, especially LDL-c > or =100mg/dl and age at lupus diagnosis, have been associated with atherosclerosis in lupus patients. Lupus (2010) 19, 803-809. PMID:20118159

  5. Aging male syndrome

    Directory of Open Access Journals (Sweden)

    Valer Donca

    2012-12-01

    Full Text Available Aging Male Syndrome is a medical condition through which men could pass between the ages of 35 and 65, when testosterone levelsin their body decline considerably. Androgen deficiency in the aging male has become a topic of increasing interest and debate throughout theworld. In contrast to female menopause, the process of aging in the male genital system is slow and highly variable between individuals. Thecharacteristic symptoms of Aging Male Syndrome include weakness, depression, fatigue and changes in body hair and skin, decreased sexualdesire, decreased lean body mass accompanied by increased visceral fat, decreased bone mineral density. Aging Male Syndrome is usually diagnosedby testing the blood for testosterone levels. The usual treatment method for Aging Male Syndrome includes testosterone injections,testosterone patches, testosterone gels and oral preparations.

  6. High-resolution MRI with a microscopy coil for carpal tunnel syndrome patients. Morphological changes in the carpal canal during finger motion

    International Nuclear Information System (INIS)

    Thickening of the flexor tendon synovium has been considered as the cause of idiopathic carpal tunnel syndrome; however, this pathogenesis has not been clarified. We investigated the dynamic morphological changes in the carpal canal during finger motion by using high-resolution MRI with a microscopy coil. We examined 11 wrists of 10 patients. They were diagnosed with idiopathic carpal tunnel syndrome based on clinical symptoms and electrophysiological examination, and they finally underwent carpal canal release surgery. The patients were all females and the mean age was 59.1 years. As a control group, 9 wrists of 9 asymptomatic individuals were also examined. The control group consisted of 6 males and 3 females, and the mean age was 33.4 years. A 1.5T MR system (Philips) and a 47-mm microscopy surface coil were used. Axial sections of 2.0 mm thickness with a spacing of 0.2 mm were obtained by T1-weighted and T2-weighted fast spin echo and T2-weighted fast field echo. The field of view (F.O.V.) was 50 mm. We evaluated the cross-sectional area of the flexor tendons that included their surrounding flexor tendon synovium and the median nerve in both the finger-extended and finger-flexed positions. The structures in the carpal canal were clearly revealed on the MR images. The cross-sectional area of the flexor tendons, including the flexor tendon synovium, in patients was significantly enlarged in the finger-flexed position as compared with the finger-extended position. In the finger-flexed position, the median nerve was compressed in the radiovolar direction because of the enlarged flexor tendon synovium around the flexor digitorum profundus (FDP) tendons, which covered the entire ulnar side of the central carpal canal. In the control group, these changes were negligible. Therefore, we suggest that repeated mechanical stress of the flexor tendons with the thickening of the flexor tendon synovium can result in median nerve neuropathy. (author)

  7. The short-to-mid-term blood gas changes after TIPS with the shunting established at different sites of portal vein for the treatment of hepatopulmonary syndrome

    International Nuclear Information System (INIS)

    Objective: To discuss the short-to-mid-term blood gas changes in patients with hepatopulmonary syndrome (HPS) after receiving TIPS with the shunting established at different sites of portal vein. Methods: During the period from July 2008 to May 2013, transjugular intrahepatic portosystemic shunting (TIPS) was employed in treating clinically - proved HPS at the hospital. A total of 81 cases had complete material, and their clinical data were retrospectively analyzed. Of the 81 cases, shunting was established at the main branch of portal vein in 30 (group A), at the left branch of portal vein in 24 (Group B) and at the right branch of portal vein in 27 (Group C). After TIPS all the patients were followed up for 3- 12 months. With the patients laying in horizontal position and receiving no oxygen inhalation, A-aPO2, PaO2 and SO2 levels were determined both before and after TIPS. The results were analyzed and compared between each other among the three groups by using paired t-test. Results: Half months after TIPS, A-aPO2, PaO2 and SO2 levels of group A and group B showed a significant improvement when compared with those determined before TIPS (P<0.05), while no significant differences in A-aPO2, PaO2 and SO2 levels were found in group C after TIPS (P>0.05). Three months after TIPS, A-aPO2, PaO2 and SO2 levels of group A and group B still showed an obvious improvement when compared with those determined before TIPS (P<0.05), while in group C only the A-aPO2 level showed a significant improvement when compared with that determined before TIPS (P<0.05). Twelve months after TIPS, A-aPO2, PaO2 and SO2 levels of group B still showed a significant improvement when compared with those determined before TIPS (P<0.05), while those of group A and group C showed no significant changes when compared with those determined before TIPS (P>0.05). Conclusion: In performing TIPS for the treatment of hepatopulmonary syndrome, to establish the shunting at the main branch of portal vein

  8. Morphological changes of intestinal mucosa in patients with different clinical variants of irritable bowel syndrome using tetracyclic antidepressants and selective serotonin reuptake inhibitor

    Directory of Open Access Journals (Sweden)

    Nagieva S.

    2015-12-01

    Full Text Available Objective. To assess histological changes of colonic mucosa in patients with clinically different types of irritable bowel syndrome (IBS before and after the treatment with tetracyclic antidepressant and selective serotonin reuptake inhibitor. Methods. Adult patients (over 18 years with confirmed diagnosis of IBS were examined. Biopsy specimens were taken from colon during colonoscopy for the next histological examination. One expert gastrointestinal pathologist assessed all tissue samples. We patent semi quantitative assessment of the severity of cell infiltration of colonic mucosa, which could be assessed as inflammatory (neutrophils, immune (lymphocytes, plasma cells, macrophages, or allergic (eosinophils response (0 to 3 degrees. All patients received treatment due to the clinical variant of IBS: 1 IBS-constipation – mirtazapinum 15 mg/night+lactulose 30ml/morning (+30ml/night if needed; 2 IBS-diarrhea – escitalopram 5mg/night+rifaximine 600mg/twice a day; 3 IBS-unspecified – mirtazapinum 15 mg/escitalopram 5mg/ night; 4 IBS-mixed – mirtazapinum 15 mg, lactulose 30ml/morning (+30ml/night if needed / escitalopram 5mg/night+rifaximine 600mg/twice a day. Results. 107 patients were examined, 36 of them had constipation (I group, 35 – diarrhea (II group, 22- unspecified variant (III group and 12 patients had mixed variant of IBS (IV group due to Rome III criteria (2006. 1st degree of lymphocyte infiltration was detected in 100% IBS-constipation patients and in 58,3% IBS-mixed variant (p0.05. No cases of 2nd or 3rd degree of colonic mucosa infiltration were found. Conclusion. After the treatment with tetracyclic antidepressant and selective serotonin reuptake inhibitor we found that the degree of inflammation of colonic mucosa was reduced or disappeared, due to the zero degree of infiltration according to our patented classification. Citation: Nagieva S, Svintsitskyy A, Kuryk O, Korendovych I. [Morphological changes of intestinal mucosa

  9. [Changes in prescription patterns to patients with bipolar syndromes. Increased use of lamotrigine and decreased use of lithium].

    Science.gov (United States)

    Karanti, Alina; Kardell, Mathias; Lundberg, Ulrika; Landén, Mikael

    Lithium is a first line option in the maintenance treatment of bipolar disorder, but several alternative treatment regimens have been introduced in recent years, among them treatment with antiepileptic compounds and atypical antipsychotic drugs. Little is known about if and how this has changed the prescription patterns of mood stabilizers. We analysed trends in prescription of mood stabilisers in Sweden using the national quality register for bipolar disorder (BipoläR), the Prescribed Drug Register, and the Patient Register during the years 2007-2011. We found that lithium use decreased while lamotrigine use increased in bipolar patients. These changes could not be ex-plained by differences in bipolar subtypes; lithium use decreased in both bipolar type I and type II, and the use of lamotrigine increased in bipolar type II. Lithium use was more common in men, whereas lamotrigine use was more common in women. The prescription of other mood stabilisers did not change during these years.  PMID:25514669

  10. Diagnosis of Irritable Bowel Syndrome

    Science.gov (United States)

    ... your anus. The doctor will fill your large intestine with barium . You may be asked to change positions several times during the test. ​​​​​ ​February 23, 2015​​​​ Previous: Symptoms and Causes of Irritable Bowel Syndrome Next: Treatment for Irritable Bowel Syndrome Digestive Disease ...

  11. X-ray diagnosis of predisposition to pulmonary nephrotic edema in patients with glomerulonephritis

    International Nuclear Information System (INIS)

    To develop criteria for determining predisposition to pulmonary edema in patients with glomerulonephritis, clinical, laboratory and X-ray examinations were made in 697 patients with glomerulonephritis at different stages of its development. X-ray examination included chest tele X-ray and its densitometric analysis. Twenty two patients underwent computerized tomography with gistographic analysis. In 106 patients, X-ray findings were compared with the volume of circulating blood, cardiac and stroke indices. CHanges in the lungs and pleural cavities were found in 22,7%, pulmonary edema was revealed in 15,7% of the patients. The prognostically unfavourable criteria for the development of pulmonary edema were found to be Stage 2 pulmonary venous hypertension with hypervolemia and peripheral edemas

  12. The developmental trajectory of disruptive behavior in Down syndrome, fragile X syndrome, Prader-Willi syndrome and Williams syndrome.

    Science.gov (United States)

    Rice, Lauren J; Gray, Kylie M; Howlin, Patricia; Taffe, John; Tonge, Bruce J; Einfeld, Stewart L

    2015-06-01

    The aim of this study was to investigate the developmental trajectories of verbal aggression, physical aggression, and temper tantrums in four genetic syndrome groups. Participants were part of the Australian Child to Adult Development Study (ACAD), which collected information from a cohort of individuals with an intellectual disability at five time points over 18 years. Data were examined from a total of 248 people with one of the four following syndromes: Down syndrome, Fragile X syndrome, Prader-Willi syndrome, or Williams syndrome. Changes in behaviors were measured using validated items from the Developmental Behavior Checklist (DBC). The results indicate that, while verbal aggression shows no evidence of diminishing with age, physical aggression, and temper tantrums decline with age before 19 years for people with Down syndrome, Fragile X syndrome, and William syndrome; and after 19 years for people with Prader-Willi syndrome. These findings offer a somewhat more optimistic outlook for people with an intellectual disability than has previously been suggested. Research is needed to investigate the mechanisms predisposing people with PWS to persistence of temper tantrums and physical aggression into adulthood. PMID:25983069

  13. Complex regional pain syndrome.

    Science.gov (United States)

    Bruehl, Stephen

    2015-01-01

    Complex regional pain syndrome is a chronic pain condition characterized by autonomic and inflammatory features. It occurs acutely in about 7% of patients who have limb fractures, limb surgery, or other injuries. Many cases resolve within the first year, with a smaller subset progressing to the chronic form. This transition is often paralleled by a change from "warm complex regional pain syndrome," with inflammatory characteristics dominant, to "cold complex regional pain syndrome" in which autonomic features dominate. Multiple peripheral and central mechanisms seem to be involved, the relative contributions of which may differ between individuals and over time. Possible contributors include peripheral and central sensitization, autonomic changes and sympatho-afferent coupling, inflammatory and immune alterations, brain changes, and genetic and psychological factors. The syndrome is diagnosed purely on the basis of clinical signs and symptoms. Effective management of the chronic form of the syndrome is often challenging. Few high quality randomized controlled trials are available to support the efficacy of the most commonly used interventions. Reviews of available randomized trials suggest that physical and occupational therapy (including graded motor imagery and mirror therapy), bisphosphonates, calcitonin, subanesthetic intravenous ketamine, free radical scavengers, oral corticosteroids, and spinal cord stimulation may be effective treatments. Multidisciplinary clinical care, which centers around functionally focused therapies is recommended. Other interventions are used to facilitate engagement in functional therapies and to improve quality of life. PMID:26224572

  14. Proliferative glomerulonephritis and primary antiphospholipid syndrome

    International Nuclear Information System (INIS)

    Little is known regarding the association of primary antiphospholipid syndrome (APLS) and proliferative glomerulonephiritis (GN). We describe a biopsy-documented case with primary APLS and proliferative (GN) with no evidence of thrombotic microangiopathy (TMA), and in the absence of other manifestations of systematic lupus erythematosus (SLE). She presented initially with left popliteal deep venous thrombosis and nephrotic syndrome. Her first pregnancy at the age of 26 years resulted in the intra-uterine fetal death at term. Two subsequent pregnancies ended up with miscarriages at 3 and 4 months of gestation. Urinalysis revealed glomerular red blood cells of 1.0000.000/ml and granular cast; proteinuria of 13.4grams/24 hours, which was non-selective; hemoglobin 12 gm/dl, normal white blood cell and platelets; serum albumin 2.6gm/dl; anti-nuclear antibody (ANA) and anti DNA were negative and complement levels normal. Lupus anticoagulant was positive leading to a diagnosis of primary APLS. The biopsy findings were consistent with membranoproliferative GN. She continued to have steroid-resistant proteinuria, but stable renal function after a 12-year follow up period. She had 2 pregnancies during this period and was delivered at term using caesarian section. She received heparin during the pregnancies. Later she developed hypertension easily controlled by atenolol. This case provides evidence that primary APLS can be associated with proliferative GN due to immune deposits and not only TMA as previously reported, and in the complete absence of SLE. Performing more renal biopsies in this group of patients may disclose a greater prevalence of proleferative GN and may help in devising a rationale for treatment. (author)

  15. Spatial cognitive deficits in an animal model of Wernicke-Korsakoff syndrome are related to changes in thalamic VDAC protein concentrations.

    Science.gov (United States)

    Bueno, K O; de Souza Resende, L; Ribeiro, A F; Dos Santos, D M; Gonçalves, E C; Vigil, F A B; de Oliveira Silva, I F; Ferreira, L F; de Castro Pimenta, A M; Ribeiro, A M

    2015-05-21

    Proteomic profiles of the thalamus and the correlation between the rats' performance on a spatial learning task and differential protein expression were assessed in the thiamine deficiency (TD) rat model of Wernicke-Korsakoff syndrome. Two-dimensional gel-electrophoresis detected 320 spots and a significant increase or decrease in seven proteins. Four proteins were correlated to rat behavioral performance in the Morris Water Maze. One of the four proteins was identified by mass spectrometry as Voltage-Dependent Anion Channels (VDACs). The association of VDAC is evident in trials in which the rats' performance was worst, in which the VDAC protein was reduced, as confirmed by Western blot. No difference was observed on the mRNA of Vdac genes, indicating that the decreased VDAC expression may be related to a post-transcriptional process. The results show that TD neurodegeneration involves changes in thalamic proteins and suggest that VDAC protein activity might play an important role in an initial stage of the spatial learning process. PMID:25766938

  16. Changes of plasma IL-6 and TNF-α levels after CPAP treatment in patients with obstructive sleep apnea syndrome (OSAS)

    International Nuclear Information System (INIS)

    Objective: To investigate the changes of plasma IL-6 and TNF-α levels after continuous positive airway pressure (CPAP) therapy in patients with obstructive sleep apnea syndrome (OSAS). Methods: Plasma IL-6 and TNF-α levels were measured with RIA in 60 patients with OSAS both before and after CPAS therapy as well as in 30 controls. Results: Before CPAP therapy, the plasma IL-6 and TNF-α levels in patients with OSAS were significantly higher than those in controls (25.92 ± 4.48pg/ ml and 11.27 ± 2.60pg/ml vs 13.21 ± 1.97pg/ml and 5.83±0.99pg/mi, P2 level (r=-0.495, 0.483, P<0.05). After treatment with CPAP for three months, the plasma IL-6 and TNF-α levels were significantly decreased (15.37±1.78pg/ml and 6.79±0.87pg/ml, vs pre-treatment levels, P<0.05, P<0.01). Conclusion: CPAP therapy could effectively decrease the plasma IL-6 and TNF-α levels in patients with OSAS. (authors)

  17. The Key Proteins of Dopaminergic Neurotransmission of Human Peripheral Blood Lymphocytes: Changed mRNA Level in Alcohol Dependence Syndrome.

    Science.gov (United States)

    Taraskina, A E; Grunina, M N; Zabotina, A M; Nasyrova, R F; Ivanov, M V; Krupitsky, E M; Schwartzman, A L

    2015-12-01

    The expression of dopamine receptor (DRD), Nurr1 transcription factor (NR4A2), and α-sinucleine (SNCA) genes in peripheral blood lymphocytes is evaluated. The results indicate that alcohol dependence is associated with high expression of SNCA and DRD4 (signifi cantly higher than in the control group) and is not associated with changes in the work of NR4A2 and DRD3 genes. The levels of DRD3 and DRD4 mRNA form a positive linear correlation (p≤0.05). The expression of SNCA and DRD4 genes can serve as an important peripheral marker of alcohol dependence development, which is essential for antipsychotic therapy. PMID:26621272

  18. Urinary peptide profiling to differentiate between minimal change disease and focal segmental glomerulosclerosis.

    Directory of Open Access Journals (Sweden)

    Vanessa Pérez

    Full Text Available BACKGROUND: Minimal change disease (MCD and primary focal segmental glomerulosclerosis (FSGS are the main causes of primary idiopathic nephrotic syndrome in children and adults, with diagnosis being essential for the appropriate choice of therapy and requiring renal biopsy. However, the presence of only normal glomeruli on renal biopsy of FSGS patients may lead to the misclassification of these patients as having MCD. The aim of this study was to (i compare the peptide profile of MCD and FSGS patients with that of a group of healthy subjects, (ii generate and validate a class prediction model to classify MCD and FSGS patients and (ii identify candidate biomarkers of these glomerular entities by analysis of the urinary peptidome. METHODS: The urinary peptide profile was analyzed by magnetic bead-based technology combined with MALDI-TOF mass spectrometry in 44 patients diagnosed of MCD (n = 22 and FSGS (n = 22. The resulting spectra were compiled and analyzed using ClinProTools software. RESULTS: A class prediction model was developed to differentiate MCD and FSGS patients. The validation of this model correctly classified 81.8% (9/11 of MCD patients and 72.7% (8/11 of FSGS patients. Moreover, the signal with m/z 1913.60, identified as a fragment of uromodulin, and the signal with m/z 2392.54, identified as a fragment of alpha-1-antitrypsin, showed higher and lower peak areas, respectively, in FSGS patients compared with MCD patients. CONCLUSIONS: The simple, non-invasive technique described in the present study may be a useful tool to help clinicians by confirming diagnoses achieved by renal biopsy, thereby reducing misdiagnoses and avoiding the implementation of inappropriate therapies.

  19. Metastatic Basal Cell Carcinoma Accompanying Gorlin Syndrome

    OpenAIRE

    Yeliz Bilir; Erkan Gokce; Banu Ozturk; Faik Alev Deresoy; Ruken Yuksekkaya; Emel Yaman

    2014-01-01

    Gorlin-Goltz syndrome or basal cell nevus syndrome is an autosomal dominant syndrome characterized by skeletal anomalies, numerous cysts observed in the jaw, and multiple basal cell carcinoma of the skin, which may be accompanied by falx cerebri calcification. Basal cell carcinoma is the most commonly skin tumor with slow clinical course and low metastatic potential. Its concomitance with Gorlin syndrome, resulting from a mutation in a tumor suppressor gene, may substantially change morbidity...

  20. Changes in sensitivity of reward and motor behavior to dopaminergic, glutamatergic, and cholinergic drugs in a mouse model of fragile X syndrome.

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    Eric W Fish

    Full Text Available Fragile X syndrome (FXS is a leading cause of intellectual disability. FXS is caused by loss of function of the FMR1 gene, and mice in which Fmr1 has been inactivated have been used extensively as a preclinical model for FXS. We investigated the behavioral pharmacology of drugs acting through dopaminergic, glutamatergic, and cholinergic systems in fragile X (Fmr1 (-/Y mice with intracranial self-stimulation (ICSS and locomotor activity measurements. We also measured brain expression of tyrosine hydroxylase (TH, the rate-limiting enzyme in dopamine biosynthesis. Fmr1 (-/Y mice were more sensitive than wild type mice to the rewarding effects of cocaine, but less sensitive to its locomotor stimulating effects. Anhedonic but not motor depressant effects of the atypical neuroleptic, aripiprazole, were reduced in Fmr1 (-/Y mice. The mGluR5-selective antagonist, 6-methyl-2-(phenylethynylpyridine (MPEP, was more rewarding and the preferential M1 antagonist, trihexyphenidyl, was less rewarding in Fmr1 (-/Y than wild type mice. Motor stimulation by MPEP was unchanged, but stimulation by trihexyphenidyl was markedly increased, in Fmr1 (-/Y mice. Numbers of midbrain TH+ neurons in the ventral tegmental area were unchanged, but were lower in the substantia nigra of Fmr1 (-/Y mice, although no changes in TH levels were found in their forebrain targets. The data are discussed in the context of known changes in the synaptic physiology and pharmacology of limbic motor systems in the Fmr1 (-/Y mouse model. Preclinical findings suggest that drugs acting through multiple neurotransmitter systems may be necessary to fully address abnormal behaviors in individuals with FXS.