Symptoms Of Nephrotic Syndrome Research Articles

#2698 Regression of amyloid deposits after treatment with tocilizumab

Abstract Background and Aims AA amyloidosis is a multisystem disease characterized by the deposition of serum amyloid A protein, which is secondary to chronic inflammation. Tocilizumab (an interleukin-6 inhibitor monoclonal antibody) is effective in suppressing inflammation, normalizing serum amyloid A protein levels and inducing remission in patients with amyloidosis. Recently, tocilizumab treatment has been associated with the disappearance of amyloid deposits. Results We report a case of AA amyloidosis secondary to chronic arthritis in a 61-year-old woman referred to our hospital in 2011 due to oligoarthritis of the knees and elevation of acute-phase reactants. Corticosteroids and methotrexate were prescribed, without clinical response. Two years later, the patient presented with foamy urine, nocturia, sweating and dizziness. An elevated C-reactive protein (CRP), erythrocyte sedimentation rate and nephrotic-range proteinuria were found. Rectal and oral mucosa biopsies were performed and amyloid AA deposits were detected in both (Fig. 1A, B). Magnetic resonance imaging (MRI) of the right knee showed arthropathy due to amyloid deposition (Fig. 2). Intravenous monthly tocilizumab was prescribed with rapid improvement of CRP, proteinuria (Fig. 3) and nephrotic syndrome symptoms. Arthritis also improved significantly. Two years later, a new biopsy of the rectal mucosa did not show amyloid deposits (Fig. 1C, D) and right knee MRI was normal, without evidence of amyloid synovitis (Fig. 4). Eight years later, the patient continues treatment with tocilizumab and remains clinically stable, with no evidence of recurrence. Literature review: The efficacy of tocilizumab has been described mainly in patients with rheumatoid arthritis, but also in AA amyloidosis secondary to systemic juvenile idiopathic arthritis, familial Mediterranean fever, psoriatic arthritis, ankylosing spondylitis, Still's disease, Muckle-Wells syndrome, Behçet syndrome, polyarteritis nodosa and Takayasu arteritis. Some of the studies report long-term effectiveness of up to 9 years. In 11 case reports of patients with AA amyloidosis treated with tocilizumab, a follow-up biopsy was performed and a reduction or disappearance of the deposits was also observed. Lane et al. analyzed pre- and on-treatment serial scintigraphy scans in 20 patients with AA amyloidosis, showing that AA amyloid deposits either regressed or did not increase whilst on tocilizumab. We have not identified any study demonstrating the disappearance of amyloid arthropathy in MR images. Conclusion Tocilizumab treatment controls chronic inflammatory disease and improves symptoms of AA amyloidosis. Long-term treatment with tocilizumab may remove amyloid deposits from tissues, leading to a definitive cure of this disease.

Adult primary glomerular diseases due to podocytopathies: a single center experience on patient characteristics, treatment and outcomes

Purpose: This study aims to evaluate the demographic, clinical, and pathologic characteristics and response to immunosuppressive therapy, particularly corticosteroids, in adult patients with primary focal segmental glomerulosclerosis (FSGS) and minimal change disease (MCD), which are classified as podocytopathies. 
 Materials and Methods: Between January 1998 and December 2014, this study included 44 patients (27 with primary FSGS and 17 with MCD) aged older than 18 years with a histopathologic diagnosis, symptoms of nephrotic syndrome, and a minimum follow-up of six months. Patients were divided into two groups according to the treatment they received and three groups according to their response to treatment. Patients diagnosed with primary FSGS and MCD were evaluated based on clinical, demographic, and laboratory findings, as well as response to treatment, and a comparison was conducted between the two groups.
 Results: 59.1% of the patients were male with a mean age of 44.8±17.7 years. At the time of diagnosis, there were no statistically significant differences in clinical and demographic characteristics between MCD and primary FSGS patients. However, in patients with MCD, the mean creatinine clearance (118.0±46.7 ml/min) was higher and the rate of microscopic hematuria (11.8%) was lower at the time of diagnosis. There was an increased need for alternative immunosuppressive treatments besides corticosteroids in patients with primary FSGS to achieve partial or complete remission. At both the third and sixth-month follow-ups, MCD patients achieved a higher rate of complete remission (proteinuria

Penerapan Manajemen Cairan Pada Asuhan Keperawatan Anak Dengan Sindrom Nefrotik

Introduction: Nephrotic syndrome is a collection of symptoms of renal impairment with edema and proteinuria being the main signs. Cases of nephrotic syndrome commonly occur in children. The prevalence of nephrotic syndrome cases in children is 80-90% with no known cause. Nursing problems that arise in this case are related to fluid balance disorders such as excess fluid volume. Fluid management is important in overcoming edema: To analyze nursing care with fluid management measures against edema in children with nephrotic syndrome. Methods: Case study with short nursing care in children. Samples used 1 patient with nephrotic syndrome with fluid management nursing interventions for 3x24 hours in the form of monitoring fluid intake as needed, 24-hour fluid balance, and collaborative diuretic administration. Results: In nursing care for nephrotic syndrome in children with excess fluid volume problems can be resolved with fluid management. Conclusion: Fluid management management effectively reduces symptoms of edema and overcomes hypervolemia in nephrotic syndrome. Recommendations to nurses to conduct careful monitoring of signs and symptoms of nephrotic syndrome, including edema, weight loss, blood pressure, and urine. Intensive monitoring can help early detection of changes in condition.

A case of dasatinib-induced focal segmental glomerulosclerosis in a patient with Philadelphia chromosome positive chronic myeloid leukemia

Focal segmental glomerulosclerosis is a common glomerular histological lesion, which is usually characterised by non-nephrotic range proteinuria or nephrotic syndrome. It may be idiopathic or occurs secondarily to drugs, diabetes, obesity or HIV nephropathy and other infections. Dasatinib, a tyrosine kinase inhibitor that has been used for the treatment of Philadelphia chromosome-positive chronic myeloid leukemia, has a few renal adverse effects. Exceptional cases with non-nephrotic range proteinuria have been reported in relation with dasatinib. In this case, we report a patient with symptoms of nephrotic syndrome and nephrotic range proteinuria, who was diagnosed as focal segmental glomerulosclerosis by kidney biopsy after treated with dasatinib.

Hodgkin disease through symptoms of paraneoplastic nephrotic syndrome by the case of a 13-year old boy – case study

Idiopathic nephrotic based on medical history, physical examination supplemented by ultrasound check. It does not often happen that a medical sign characteristic of nephrotic syndrome with generalized enlargement of lymph nodes (lymphadenopathy), liver and spleen may indicate an occurrence of lymphoma. A paraneoplastic syndrome is the state of clinical symptoms related to tumors, not being a close implication of a local neoplastic and metastatic infiltration. The objective of this article is drawing attention to the possibility of concealment of Hodgkin disease through symptoms of nephrotic syndrome by the case of a 13-year old boy.

A typical 22q11.2 deletion syndrome and pseudohypoparathyroidism

Rationale:It is rare to find 22q11.2 deletion syndrome with pseudohypoparathyroidism in children. Furthermore, the phenotypic spectrum of this disorder varies widely.Patient concerns:A patient was diagnosed with pseudohypoparathyroidism at age 14 years because of convulsions, hypocalcemia, hyperphosphatemia, normal parathyroid hormone levels, and basal ganglia calcifications. Thereafter, the child presented with symptoms of nephrotic syndrome; subsequently, he was diagnosed with nephrotic syndrome at the local hospital.Diagnosis:At our hospital, multiplex ligation-dependent probe amplification confirmed that the patient had 22q11.2 deletion syndrome.Interventions:The patient continued to be treated with calcium supplements.Outcomes:Seizure activity and proteinuria ceased.Lessons:Signs of this syndrome include delayed speech development due to velofacial dysfunction, recurrent croup attacks during early childhood due to latent hypocalcemia, and mild dysmorphic features. The findings of this patient indicated that 22q11.2 deletion syndrome may include a wide spectrum of clinical findings and that this diagnosis needs to be considered for all patients presenting with hypocalcemia, regardless of age.

A Case Report of Nephrotic Syndrome While Undergoing Quinine Therapy

We summarize the case of an 81-year-old Caucasian female who presented to her family physician with signs and symptoms of nephrotic syndrome following a brief exposure to quinine. Prior to that visit, she was clinically well with no chronic medical ailments and met with her family physician for annual physical assessments. She had taken 11 tablets of quinine for nocturnal leg cramps over the course of 28 days before starting to notice mild peripheral edema, which subsequently progressed, leading to a family physician review. Her initial serum albumin level was 12 g/L, and a 24-hour urine protein output was quantified at 8.14 g/day; she was diagnosed as having nephrotic syndrome. A kidney biopsy confirmed the diagnosis of minimal change disease (MCD). Quinine therapy was stopped, and she was initiated on a tapering regime of prednisone with concurrent cyclosporine therapy. Within a fortnight of starting therapy, she went into remission and her immunosuppressive medications were rapidly tapered and discontinued. This paper reports an association between the use of quinine and subsequent MCD. This case report proposes that the use of quinine has an association with, and may be causal for, the development of minimal change disease. As this is yet an unreported adverse effect, this paper seeks to increase the knowledge of the varied and numerous effects of quinine.

Latest research progress on acute nephrotic syndrome

Etiology of nephrotic syndrome is somewhat complex in nature. It may range from primary to secondary forms. Nephrotic syndrome patients often need immunosuppressive treatment although it has some side effects and may lead to renal disease which may be acute or severe. This review deals with herbal treatment and other recent approaches for treating symptoms of nephrotic syndrome.

Dalbergioidin Ameliorates Doxorubicin-Induced Renal Fibrosis by Suppressing the TGF-β Signal Pathway.

We investigated the effect of Dalbergioidin (DAL), a well-known natural product extracted from Uraria crinita, on doxorubicin- (DXR-) induced renal fibrosis in mice. The mice were pretreated for 7 days with DAL followed by a single injection of DXR (10 mg/kg) via the tail vein. Renal function was analyzed 5 weeks after DXR treatment. DXR caused nephrotoxicity. The symptoms of nephrotic syndrome were greatly improved after DAL treatment. The indices of renal fibrosis, the phosphorylation of Smad3, and the expression of alpha-smooth muscle actin (α-SMA), fibronectin, collagen III (Col III), E-cadherin, TGF-β, and Smad7 in response to DXR were all similarly modified by DAL. The present findings suggest that DAL improved the markers for kidney damage investigated in this model of DXR-induced experimental nephrotoxicity.

P-205: Nephrotic syndrome revealing Hodgkin’s disease

Objectifs The association between paraneoplasic nephrotic syndrome and Hodgkin’s disease is rare estimated to be about 0.5 to 1% (0.4% as minimal change disease and 0.1% as amyloid). Methodes We report the case of an Hodgkin’s disease revealed by an isolated nephrotic syndrome. Resultats A 34-year-old man presented with the clinical symptoms of nephrotic syndrome, Physical examination was notable for lower extremity edema. Though his hematologic evaluation was consistent with anemia (white blood cells = 11300/mm 3 ; hemoglobin, 7,9g/dL; platelets, 482000/mm 3 ), there was no evidence of hemolysis but a biologic inflammatory syndrome. Nephrotic range proteinuria (3,4g in 24 hours), acute renal failure (creatinine 160umol/L), and hypoalbuminemia (23g/L) were also present. Kidney biopsy revealed endocapillary proliferative glomerulone-phritis associated with vasculitis. A treatment with prednisone was started with favorable response. Two months after the diagnostic of nephrotic syndrome was made, the patient presented prolonged fever and weight loss. The physical examination showed cervical lymphadenopathy, which lymph node biopsy revealed Hodgkin lymphoma of the nodular sclerosing type. The patient was treated for Hodgkin disease stage IVB with ABVD regimen, and was in complete remission of both Hodgkin’s disease and nephrotic syndrome. Conclusion Causes of nephrotic syndrome in Hodgkin’s disease can include renal vein thrombosis, amyloidosis or paraneoplastic syndrome. This case attracts attention that a rare cause of nephrotic syndrome can be Hodgkin’s disease.

Development of minimal-change glomerular disease and Hashimoto's thyroiditis during the treatment of sarcoidosis.

Minimal-change glomerular disease, sarcoidosis and autoimmune thyroid disease rarely occur in the same patient. We herein report a patient in which minimal-change glomerular disease and Hashimoto's thyroiditis developed during the treatment of sarcoidosis with steroids. A 66-year-old female was admitted to our hospital with symptoms of nephrotic syndrome. Nine months before admission, she was diagnosed as having ocular and pulmonary sarcoidosis, for which prednisolone at an initial dose of 40mg/day was started. When the dose of prednisolone was tapered to 20mg/day, she noticed swelling of the lower extremities. Examination confirmed the simultaneous occurrence of minimal-change glomerular disease and Hashimoto's thyroiditis, which were diagnosed based on kidney histology, ultrasonography of the thyroid gland and positive antithyroglobulin antibodies. We used intravenous methylprednisolone pulse therapy followed by 40mg/day oral prednisolone. The patient achieved complete remission of nephrotic syndrome and steroids were tapered without relapse.

Nitro-oleic acid protects against adriamycin-induced nephropathy in mice

Adriamycin (ADR) administration in susceptible rodents such as the BALB/c mouse strain produces injury to the glomerulus mimicking human focal glomerular sclerosis. The goal of the present study was to use this model to investigate antiproteinuric action of nitro-oleic acid (OA-NO2), a nitric oxide-derived endogenous lipid product, which has exhibited multiple attractive signaling properties particularly in the kidney. BALB/c mice were pretreated for 2 days with OA-NO2 at 5 mg·kg(-1)·day(-1) via an osmotic minipump, followed by a single injection of vehicle or adriamycin (10 mg/kg) via the tail vein. Albuminuria and renal function were analyzed at 1 wk post-ADR treatment. ADR mice developed prominent albuminuria, hypoalbuminemia, hyperlipidemia, and severe ascites. In contrast, the symptoms of nephrotic syndrome were greatly improved by OA-NO2 treatment. In parallel, plasma creatinine and plasma urea nitrogen were elevated in the ADR group, and the severity was less in the ADR+OA-NO2 group. OA-NO2 attenuates ADR-induced glomerulosclerosis, podocyte loss, and tubulointerstitial fibrosis. Indices of oxidative stress, including plasma and urinary thiobarbituric acid-reactive substances and renal expression of NAD(P)H oxidase p47(phox) and gp91(phox), and inflammation, including renal expression of TNF-α, IL-1β, and MCP-1 in response to ADR, were all similarly suppressed. Together, these findings suggest that OA-NO2 exerts renoprotective action against ADR nephropathy likely via its anti-inflammatory and antioxidant properties.

Castleman disease with preceding symptoms of nephrotic syndrome

Castleman disease with preceding symptoms of nephrotic syndrome

Hodgkin's disease revealed by a nephrotic syndrome: a case report with literature review

The association between paraneoplasic nephrotic syndrome and Hodgkin's disease is rare. We report a case of Hodgkin's lymphoma in a young female patient with nephrotic syndrome. A 40-year-old woman presented with the clinical symptoms of nephrotic syndrome, kidney biopsy revealed minimal change glomerulonephritis. A treatment with prednisone was started but the response was partial. Twenty-four months after the diagnostic of nephrotic syndrome was made, she presented prolonged fever and weight loss, the physical examination showed cervical lymphadenopathy, which lymph node biopsy revealed Hodgkin lymphoma of the nodular sclerosing type. The patient was treated for Hodgkin disease stage IVB with ABVD regimen, and was in complete remission of both Hodgkin's disease and nephrotic syndrome.

Nephrotic Syndrome in Diabetic Kidney Disease: An Evaluation and Update of the Definition

Nephrotic syndrome is defined as urine total protein excretion greater than 3.5 g/d or total protein-creatinine ratio greater than 3.5 g/g, low serum albumin level, high serum cholesterol level, and peripheral edema. These threshold levels have not been rigorously evaluated in patients with diabetic kidney disease or by using urine albumin excretion, the preferred measure of proteinuria in patients with diabetes. Diagnostic test study. Adults with type 2 diabetes, hypertension, and urine total protein level greater than 0.9 g/d enrolled in the Irbesartan in Diabetic Nephropathy Trial. Baseline measures of proteinuria (total protein and albumin excretion and protein-creatinine and albumin-creatinine ratios). Linear regression to relate measures. Other signs and symptoms of nephrotic syndrome at baseline (serum albumin < 3.5 g/dL, serum total cholesterol > 260 mg/dL or use of a statin, and edema or use of a loop diuretic); progression of chronic kidney disease during follow-up (doubling of baseline serum creatinine level or requirement for dialysis or kidney transplantation). Logistic regression to relate index and reference tests. In 1,608 participants, total urine protein level of 3.5 g/d was equivalent to urine albumin level of 2.2 g/d (95% confidence interval, 1.4 to 3.5). Of 1,467 participants, 641 (44%) had urine total protein level of 3.5 g/d or greater at baseline, 132 (9%) had other signs and symptoms of nephrotic syndrome at baseline, and 385 (26%) had progression of kidney disease during a mean follow-up of 2.6 years. Areas under the receiver operating curves for measures of proteinuria were 0.80 to 0.83 for other signs and symptoms of nephrotic syndrome and 0.72 to 0.74 for kidney disease progression. Threshold levels for nephrotic-range proteinuria and albuminuria were close to the points of maximal accuracy for both outcomes. Study population limits generalizability; inability to adjust for several variables known to affect serum albumin levels; lack of spot urine samples. The historical definition of nephrotic-range proteinuria appears reasonable in patients with diabetic kidney disease. Equivalent thresholds for nephrotic-range albuminuria and albumin-creatinine ratio are 2.2 g/d and 2.2 g/g, respectively.

Nephrotic syndrome preceding Hodgkin's lymphoma by 13 months

This report documents the occurrence of a minimal change nephrotic syndrome in a patient antedating the recognition of Hodgkin's lymphoma. An 18-year-old woman presented with the clinical symptoms of nephrotic syndrome and was started on prednisolone with partial response. 13 months after the diagnosis was made, she presented with weight loss and right upper abdominal pain for two months. Kidney biopsy revealed minimal change glomerulonephritis. She subsequently developed cervical lymphadenopathy, which on excision biopsy report, turned out to be Hodgkin's disease, nodular sclerosing type. Hodgkin's lymphoma clinical stage IIIB was diagnosed. A review of literature yielded altogether three cases in which the onset of nephrotic syndrome preceded the diagnosis of Hodgkin's lymphoma by more than 12 months.

Two Paraneoplastic Syndromes in a Patient with Ovarian Cancer: Nephrotic Syndrome and Paraneoplastic Cerebellar Degeneration

Background. A paraneoplastic syndrome—a rare, indirect system disturbance located distant from a neoplasm—has been reported in association with ovarian cancer.Case. Two paraneoplastic syndromes—nephrotic syndrome and paraneoplastic cerebellar degeneration (PCD)—developed in a patient who had no symptoms from ovarian cancer. She presented with the nephrotic syndrome. Evaluation for metastasis, including computed tomography (CT) scan and ultrasonography, showed a slightly enlarged right ovary and a 2-cm lower paraaortic lymph node on the right side. A 5-cm ovarian mass and multiple paraaortic lymph nodes were excised, and six cycles of carboplatin and paclitaxel were given. Nephrotic syndrome symptoms had greatly subsided at 6 weeks after surgery. At 7 months after surgery, symptoms of PCD developed yet CT, CA-125 levels, and physical examination showed no evidence of ovarian cancer.Conclusion. To our knowledge, development of two paraneoplastic syndromes in one patient with ovarian cancer has not been reported. Nephrotic syndrome resolved with treatment of the neoplasm whereas PCD did not.