Intravenous cyclophosphamide induces remission in children with difficult to treat steroid resistant nephrotic syndrome from minimal change disease

Abstract Background Steroid resistant nephrotic syndrome (SRNS), while uncommon in children, is associated with significant morbidity. Calcineurin inhibitors (CNIs) remain the first line recommended therapy for children with non-genetic forms of SRNS, but some children fail to respond to them. Intra...

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Autores principales:	Maha Haddad, Arundhati Kale, Lavjay Butani
Formato:	article
Lenguaje:	EN
Publicado:	BMC 2021
Materias:	Steroid resistant nephrotic syndrome Cyclophosphamide Pediatric Remission Diseases of the genitourinary system. Urology RC870-923
Acceso en línea:	https://doaj.org/article/1b3f346ab94a4d5e8e72d40d8b102586
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spelling	oai:doaj.org-article:1b3f346ab94a4d5e8e72d40d8b1025862021-12-05T12:26:01ZIntravenous cyclophosphamide induces remission in children with difficult to treat steroid resistant nephrotic syndrome from minimal change disease10.1186/s12882-021-02605-61471-2369https://doaj.org/article/1b3f346ab94a4d5e8e72d40d8b1025862021-11-01T00:00:00Zhttps://doi.org/10.1186/s12882-021-02605-6https://doaj.org/toc/1471-2369Abstract Background Steroid resistant nephrotic syndrome (SRNS), while uncommon in children, is associated with significant morbidity. Calcineurin inhibitors (CNIs) remain the first line recommended therapy for children with non-genetic forms of SRNS, but some children fail to respond to them. Intravenous (IV) cyclophosphamide (CTX) has been shown to be effective in Asian-Indian children with difficult to treat SRNS (SRNS-DTT). Our study evaluated the outcome of IV CTX treatment in North American children with SRNS-DTT. Methods Retrospective review of the medical records of children with SRNS-DTT treated with IV CTX from January 2000 to July 2019 at our center. Data abstracted included demographics, histopathology on renal biopsy, prior and concomitant use of other immunosuppressive agents and serial clinical/laboratory data. Primary outcome measure was attainment of complete remission (CR). Results Eight children with SRNS-DTT received monthly doses (median 6; range 4–6) of IV CTX. Four (50%) went into CR, 1 achieved partial remission and 3 did not respond. Three of the 4 responders had minimal change disease (MCD). Excluding the 1 child who responded after the 4th infusion, the median time to CR was 6.5 (range 0.5–8) months after completion of IV CTX infusions. Three remain in CR at a median of 8.5 years (range: 3.7–10.5 years) after completion of CTX; one child relapsed and became steroid-dependent. No infections or life-threatening complications related to IV CTX were observed. Conclusions IV CXT can induce long term remission in North-American children with MCD who have SRNS-DTT.Maha HaddadArundhati KaleLavjay ButaniBMCarticleSteroid resistant nephrotic syndromeCyclophosphamidePediatricRemissionDiseases of the genitourinary system. UrologyRC870-923ENBMC Nephrology, Vol 22, Iss 1, Pp 1-6 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	Steroid resistant nephrotic syndrome Cyclophosphamide Pediatric Remission Diseases of the genitourinary system. Urology RC870-923
spellingShingle	Steroid resistant nephrotic syndrome Cyclophosphamide Pediatric Remission Diseases of the genitourinary system. Urology RC870-923 Maha Haddad Arundhati Kale Lavjay Butani Intravenous cyclophosphamide induces remission in children with difficult to treat steroid resistant nephrotic syndrome from minimal change disease
description	Abstract Background Steroid resistant nephrotic syndrome (SRNS), while uncommon in children, is associated with significant morbidity. Calcineurin inhibitors (CNIs) remain the first line recommended therapy for children with non-genetic forms of SRNS, but some children fail to respond to them. Intravenous (IV) cyclophosphamide (CTX) has been shown to be effective in Asian-Indian children with difficult to treat SRNS (SRNS-DTT). Our study evaluated the outcome of IV CTX treatment in North American children with SRNS-DTT. Methods Retrospective review of the medical records of children with SRNS-DTT treated with IV CTX from January 2000 to July 2019 at our center. Data abstracted included demographics, histopathology on renal biopsy, prior and concomitant use of other immunosuppressive agents and serial clinical/laboratory data. Primary outcome measure was attainment of complete remission (CR). Results Eight children with SRNS-DTT received monthly doses (median 6; range 4–6) of IV CTX. Four (50%) went into CR, 1 achieved partial remission and 3 did not respond. Three of the 4 responders had minimal change disease (MCD). Excluding the 1 child who responded after the 4th infusion, the median time to CR was 6.5 (range 0.5–8) months after completion of IV CTX infusions. Three remain in CR at a median of 8.5 years (range: 3.7–10.5 years) after completion of CTX; one child relapsed and became steroid-dependent. No infections or life-threatening complications related to IV CTX were observed. Conclusions IV CXT can induce long term remission in North-American children with MCD who have SRNS-DTT.
format	article
author	Maha Haddad Arundhati Kale Lavjay Butani
author_facet	Maha Haddad Arundhati Kale Lavjay Butani
author_sort	Maha Haddad
title	Intravenous cyclophosphamide induces remission in children with difficult to treat steroid resistant nephrotic syndrome from minimal change disease
title_short	Intravenous cyclophosphamide induces remission in children with difficult to treat steroid resistant nephrotic syndrome from minimal change disease
title_full	Intravenous cyclophosphamide induces remission in children with difficult to treat steroid resistant nephrotic syndrome from minimal change disease
title_fullStr	Intravenous cyclophosphamide induces remission in children with difficult to treat steroid resistant nephrotic syndrome from minimal change disease
title_full_unstemmed	Intravenous cyclophosphamide induces remission in children with difficult to treat steroid resistant nephrotic syndrome from minimal change disease
title_sort	intravenous cyclophosphamide induces remission in children with difficult to treat steroid resistant nephrotic syndrome from minimal change disease
publisher	BMC
publishDate	2021
url	https://doaj.org/article/1b3f346ab94a4d5e8e72d40d8b102586
work_keys_str_mv	AT mahahaddad intravenouscyclophosphamideinducesremissioninchildrenwithdifficulttotreatsteroidresistantnephroticsyndromefromminimalchangedisease AT arundhatikale intravenouscyclophosphamideinducesremissioninchildrenwithdifficulttotreatsteroidresistantnephroticsyndromefromminimalchangedisease AT lavjaybutani intravenouscyclophosphamideinducesremissioninchildrenwithdifficulttotreatsteroidresistantnephroticsyndromefromminimalchangedisease
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Intravenous cyclophosphamide induces remission in children with difficult to treat steroid resistant nephrotic syndrome from minimal change disease

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