درمان گلومرولواسکلروز فوکال و سگمنتال اولیه

نوع مقاله : مقاله پژوهشی

نویسندگان

1 مرکز تحقیقات نارسایی مزمن کلیه، گروه نفرولوژی کودکان، دانشکدة پزشکی، دانشگاه علوم پزشکی جندی-شاپور اهواز، ایران.

2 مرکز تحقیقات نارسایی مزمن کلیه، گروه نفرولوژی، دانشگاه علوم پزشکی جندی-شاپور اهواز، ایران.

چکیده

گلومرولواسکلروز فوکال و سگمنتال  (FSGS)یکی از علل شایع سندرم نفروتیک در افراد بالغ و اطفال می­باشد. این بیماری ممکن است بدون علت مشخص (اولیه یا ایدیوپاتیک) و یا ثانویه به آسیب قبلی گلومرولی (ثانویه) ایجاد گردد. در درمان این بیماری افتراق نوع اولیه از نوع  ثانویه مهم است، چرا که درمان با تضعیف­کننده­های سیستم ایمنی در بیشتر بیماران با نوع اولیه و نه نوع ثانویه اندیکاسیون دارد. بیماران مبتلا به FSGS اولیه درمان نشده که با پروتئینوری در حد نفروتیک (بیش از 5/3 گرم در روز) و هیپوآلبومینمی مراجعه کرده­اند به طور تیپیک به سمت بیماری مرحلۀ پایانی کلیه پیشرفت می­نمایند. به نظر می­رسد که FSGS  اولیه در 80-40 درصد موارد به پردنیزولون با دوز 1mg/kg/day (حداکثر دوز 80-60 میلی­گرم در روز) پاسخ می­دهد. البته معمولاً برای ایجاد رمیسیون در این بیماران، دورۀ طولانی درمان با استروئید برای حداقل 16- 12 هفته لازم است. به طور شایع در بیماران عود کننده و یا مقاوم به استروئید، از سیکلوسپورین با دوز تقریبی 4mg/kg/day -2  که در دو دوز منقسم داده می­شود به همراه با دوز پایین پردنیزولون (حداکثر 15 میلی­گرم در روز) استفاده می­شود. به علاوه از سایر داروها مانند سیکلوفسفاماید و مایکوفنولیت مفتیل نیز در بیماران مقاوم به استروئید و یا عود کننده با درجات متفاوتی از پاسخ استفاده شده است.

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