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A 7 years old girl diagnosed as case of juvenile idiopathic arthritis since the age of 1.5 years, generalized onset with systemic presentation is complicated by secondary nephrotic syndrome. Renal biopsy showing amyloidosis. Patient has severe disease course being complicated by sever flare ups of disease symptoms and body swelling. Since onset of disease patient got NSAIDs, methotrexate, steroids, anti TNF inhibitors with no or partial response. After diagnosis with secondary nephrotic syndrome due to secondary amyloidosis patient is started on intravenous tocilizumab. There is improvement of patient symptoms.

Idiopathic multicentric Castleman disease (iMCD) is a systemic and polyclonal lymphoproliferative disease involving multiple organs, including the kidneys, due to the overproduction of interleukin-6 (IL-6). Recently, several reports have suggested that excessive IL-6 actions in iMCD could have a causal relationship with the development of diverse histopathological renal manifestations that cause nephrotic syndrome. However, the treatment for such cases remains unclear. We report a series of three cases of nephrotic syndrome due to iMCD that helps to delineate the importance of early and continuous therapy with the anti-interleukin-6 receptor antibody tocilizumab. First, treatment was suspended for infectious control, and the patient presented with nephrotic syndrome due to diffuse mesangial and endocapillary hypercellularity without immune deposits complicating acute kidney injury. Second, iMCD was treated with prednisolone alone. The patient suddenly developed nephrotic syndrome due to immune-complex glomerulonephritis, not otherwise specified, complicated with acute kidney injury. In the third case, nephrotic syndrome secondary to membranous glomerulonephritis was diagnosed, with a skin rash and IgE antibodies to tocilizumab, and was therefore treated with prednisolone alone. In contrast to the first two cases, the third progressed to end-stage renal disease on hemodialysis. Taken together, this series suggests that clinicians should maintain clinical vigilance for iMCD as a possible underlying component of nephrotic syndrome, since iMCD presents with a variety of renal pathologies. Prompt initiation and continuous administration of tocilizumab are likely key determinants of renal outcomes in such cases. In particular, when tocilizumab is suspended due to infection or in the perioperative period, consideration of its expeditious resumption should be made, taking into account both the withdrawal period and systemic conditions.

Abstract Background Nephrotic syndrome (NS) is one of the most frequent occurring chronic kidney diseases in childhood, despite its rarely occurrence in the general population. Detailed information about clinical data of NS (e.g. average length of stay, complications) as well as of secondary nephrotic syndrome (SNS) is not well known. Methods A nationwide ESPED follow-up study presenting the clinical course and management of children with NS in Germany. Results In course of 2 years, 347 children developed the first onset of NS, hereof 326 patients (93.9%) had a primary NS, and 19 patients had a SNS (missing data in 2 cases), the majority due to a Henoch-Schönlein Purpura. Patients with steroid-resistant NS (SRNS) stayed significantly longer in hospital than children with steroid-sensitive NS (25.2 vs. 13.3 d, p

Background: Nephrotic syndrome (NS) is one of the most frequent occurring chronic kidney diseases in childhood, despite its rarely occurrence in the general population. Detailed information about clinical data of NS (e.g. average length of stay, complications) as well as of secondary nephrotic syndrome (SNS) is not well known.Methods: A nationwide ESPED follow-up study presenting the clinical course and management of children with NS in Germany.Results: In course of 2 years, 347 children developed the first onset of NS, hereof 326 patients (93.9%) had a primary NS, and 19 patients had a SNS (missing data in 2 cases), the majority due to a Henoch-Schönlein Purpura. Patients with steroid-resistant NS (SRNS) stayed significantly longer in hospital than children with steroid-sensitive NS (25.2 vs. 13.3 d, p <  0.001). Patients with bacterial/viral infections stayed longer in hospital (24.9 d/19.5d) than children without an infection (14.2 d/14.9 d; p <  0.001; p = 0.016). Additionally, children with urinary tract infections (UTI) (p < 0,001), arterial hypertension (AH) (p < 0.001) and acute renal failure (ARF) (p < 0,001) stayed significantly longer in hospital. Patients with SRNS had frequent complications (p = 0.004), such as bacterial infections (p = 0.013), AH (p < 0.001), UTI (p < 0.001) and ARF (p = 0.007). Children with a focal segmental glomerulosclerosis (FSGS) had significantly more complications (p = 0.04); specifically bacterial infections (p = 0.01), UTI (p = 0.003) and AH (p < 0,001). Steroid-resistance was more common in patients with UTI (p < 0.001) and in patients with ARF (p = 0.007). Furthermore, steroid-resistance (p < 0.001) and FSGS (p < 0.001) were more common in patients with AH.Conclusions: This nationwide, largest German study presents results on the clinical course of children with NS considering a diverse range of complications that can occur with NS. The establishment of a region-wide and international pediatric NS register would be useful to conduct further diagnostic and therapy studies with the aim to reduce the complication rate and to improve the prognosis of NS, and to compare the data with international cohorts. [ABSTRACT FROM AUTHOR]

We report the case of a 7-year-old unimmunized boy who presented with generalized anasarca for the first time, along with nephrotic-range proteinuria, hypoalbuminemia, microscopic hematuria and hypertension. Special investigations revealed ELISA test to be positive for hepatitis B surface antigen (HBsAg) and hepatitis B envelope antigen (HBeAg); hepatitis B viral DNA load (HBV DNA) level (real-time polymerase chain reaction) was 54 360 903 IU/ml. For hepatitis B virus (HBV)-related glomerulopathy, he was started on enalapril and lasilactone, and percutaneous renal biopsy was performed, which revealed membranous nephropathy (MN). A diagnosis of MN secondary to HBV infection contracted via horizontal transmission was made. The patient was started on peginterferon alfa-2b (50 μg/week) for 24 weeks. He failed to attain remission and seroconversion after interferon (IFN) therapy. Then, oral therapy with entecavir was started, and he attained remission as well as seroconversion after 3 months of therapy. He maintained his seroconversion status at his 6-month and the recent 12-month (quantitative HBV DNA level was 373 IU/ml) follow-up visit. Entecavir seems a promising drug for HBV-related glomerulopathy, especially in IFN-resistant cases. [ABSTRACT FROM AUTHOR]

Abstract: Steroid resistant nephrotic syndrome (SRNS) continues to be a challenge for pediatric nephrologists the world over. Secondary causes of nephrotic syndrome need to be searched for in all cases of steroid resistance. Hepatitis B virus (HBV) is associated with several types of glomerulonephritis, most commonly being membranous nephropathy (MN) in children. It is an important cause of secondary nephrotic syndrome in countries with high prevalence of chronic hepatitis B virus (HBV) infection. We present a case of SRNS in a 5-yr-old boy who had received 3 weeks of daily steroids before referral to our hospital. At presentation the child had urinary tract infection (UTI) which was adequately treated. The child had persistence of proteinuria, even after completing 4 weeks of daily steroids in adequate dose. Secondary causes of nephrotic syndrome were looked for which revealed presence of chronic HBV infection in the patient with a very high viral load. Kidney biopsy was characteristic of MN with predominant IgG, & minor IgM, and C3 deposits in subepithelial region. The child responded to treatment with Lamivudine with reduction in edema and proteinuria. [Copyright &y& Elsevier]

Introduction and Materials and methods: We encountered a case in which nephrotic syndrome developed after radiation therapy for squamous cell carcinoma of the lung, an epithelial tumor; the patient's quality of life decreased markedly and treatment was extremely difficult, as reported here. Case and Discussion: In the current case, diagnosis of a malignant tumor came first; a paraneoplastic syndrome, nephrotic syndrome was found about a month later. Paraneoplastic syndromes are a group of conditions that occur with various malignant tumors; many neoplastic syndromes are reported with lung cancer in particular. Noted among these are Lambert-Eaton myasthenic syndrome, hypertrophic osteoarthropathy, and the syndrome of inappropriate secretion of antidiuretic hormone, but with paraneoplastic nephrotic syndrome symptoms due to nephrotic syndrome are also noted in addition to symptoms due to the malignant tumor, so QOL is markedly impaired. The patient's PS ultimately worsened, and nephrotic syndrome was treated with an oral prescription of corticosteroids. However, membranous nephropathy, which often complicates carcinomas, is generally resistant to corticosteroids and adverse effects due to corticosteroids can easily become more severe in the elderly, so to the extent possible a diagnosis should be reached early on with a kidney biopsy and then the condition should be treated. [ABSTRACT FROM AUTHOR]

Inflammatory myofibroblastic tumor (IMT) of the lung is a benign, non-metastasizing tumor with the possibility of local infiltration, recurrence or persistent local growth. This kind of tumor arises due to an unregulated growth of inflammatory cells. To our knowledge, IMT associated with nephrotic syndrome has not yet been recognized. Therefore, we present the case of a 14-year-old girl with lung IMT associated with secondary nephrotic syndrome (NS), which was cured after tumor removal. [ABSTRACT FROM AUTHOR]