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Table of contents

SRNS usually progresses to end-stage renal failure. Unfortunately, there is no curative treatment for majority of patients.

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Majority of the SRNS patients have the histologic picture of focal segmental glomerulosclerosis. Interestingly, the risk of recurrence in the kidney graft in patients with hereditary SRNS is lower than in those who do not have genetic background.


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The etiology and pathogenesis of SRSN has remained enigma for decades. The discovery of 39 dominant or recessive SRNS genes enabled better understanding of the function of the glomerular podocytes and slit membrane. The diagnostic panel included 21 genes with a recessive mode of inheritance and 6 genes with a dominant mode of inheritance. Single-gene cause was detected in The identification of causative single-gene mutations may have important therapeutic consequences in some cases.

In these patients the treatment with coenzyme Q10 may be indicated. Also, patients with recessive mutations in PLCE1 may respond fully to the treatment with steroids or cyclosporine A. The detection of causative mutations may also be very important for familial genetic counseling and for prenatal diagnosis. Pediatr Transplant ; Pediatr Nephrol.

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Wada T Nangaku M. A circulating permeability factor in focal segmental glomerulosclerosis: the hunt continues. Clin Kidney J. Circulating urokinase receptor as a cause of focal segmental glomerulosclerosis. Nat Med.

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Genetic testing in steroid-resistant nephrotic syndrome: when and how? Nephrol Dial Transplant. Nephrotic syndrome in newborn and young infants. Ann Paediatr Fenn. Positionally cloned gene for a novel glomerular protein-nephrin is mutated in congenital nephrotic syndrome. Mol Cell. Jalanko H.

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Congenital nephrotic syndrome. NPHS2 encoding the glomerular podocin is mutated in autosomal recessive steroid-resistant nephrotic syndrome. Nat Genet. Steroid-resistant nephrotic syndrome with mutations in NPHS2 podocin : report from a three-generation family.


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Mutationdependent recessive inheritance of NPHS2-associated steroid-resistant nephrotic syndrome. Nephrotic Syndrome Study Group. Rapid detection of monogenic causes of childhood-onset steroidresistant nephrotic syndrome. Clin J Am Soc Nephrol. A single-gene cause in Genetic Variation in AhR Gene.

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Immunodepression and Immunosuppression. Chronic Immune Response Hypothesis. Microbial Immunosuppression.

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Cellular Therapies for Immunosuppression. Low Immunogenic Potential. Induction Therapy in Renal Transplant Recipients. Radiotherapy and Immunity A Mini Review. The Role of Cyclosporine A in the Treatment. Clinical Immunosuppression in Solid Organ. EHealth 2 0 Developments in Treatment and Research.

Immunosuppression in Helminth Infection.