Kari Jameela A, Halawani Manal, Mokhtar Ghadeer, Jalalah Sawsan M, Anshasi Wasim
Department of Pediatrics, King Abdul Aziz University Hospital, Jeddah, Saudi Arabia.
Saudi J Kidney Dis Transpl. 2009 Sep;20(5):854-7.
Steroid resistant nephrotic syndrome (SRNS) remains a challenge facing pediatric nephrologists. The underlying histopathology usually affects the course of the disease and the response to treatment. We studied the pattern of histopathology in children with SRNS who presented to the King Abdul Aziz University Hospital (KAUH), Jeddah, Saudi Arabia. The records of all children with primary SRNS, who were seen between 2002 and 2007 were reviewed. Only patients who had undergone a renal biopsy were included in the study. The histopathology slides were reviewed by two renal pathologists independently. Patients with congenital nephrotic syndrome, lupus or sickle cell disease, were excluded from the study. Thirty-six children fulfilled the inclusion criteria, and included 25 girls and 11 boys with female to male ratio of 2.3:1. Fifty percent of the children (n=18) were Saudi and the remaining 50% were from various other racial backgrounds (9 Asians, 4 Arabs, 2 Africans and 3 from the Far East). Their mean age at presentation was 4.3 +/- 3.0 years (range 1-12 years). The mean serum albumin at presentation was 15.6 +/- 7.1 g/L and all of them had 4+ proteinuria on urinalysis. Five children had elevated serum creatinine at presentation while the mean serum creatinine was 50.4 +/- 45.6 micromol/L. Three children had low serum complement levels at presentation and none were positive for hepatitis B surface antigen or antinuclear antibody (ANA). The renal histopathology was compatible with focal and segmental glomerulosclerosis (FSGS) in 39% (n=14), IgM nephro-pathy in 28% (n=10), mesengioproliferative glomerulonephritis (MesPGN) in 17% (n=6), mini-mal change disease (MCD) and C1q nephropathy (C1qNP) in 8% each (n=3 + 3) and IgA nephro-pathy in 3% (n=1). Our retrospective review shows that FSGS was the commonest underlying histopathology in children who presented with SRNS followed by IgM nephropathy and other variants of MCD such as MesPGN. C1qNP was the underlying cause in some children.
类固醇抵抗性肾病综合征（SRNS）仍是儿科肾病学家面临的一项挑战。潜在的组织病理学通常会影响疾病进程及对治疗的反应。我们研究了沙特阿拉伯吉达阿卜杜勒阿齐兹国王大学医院（KAUH）收治的SRNS患儿的组织病理学模式。回顾了2002年至2007年间所有原发性SRNS患儿的病历。该研究仅纳入了接受过肾活检的患者。组织病理学切片由两名肾脏病理学家独立复查。先天性肾病综合征、狼疮或镰状细胞病患者被排除在研究之外。36名儿童符合纳入标准，其中包括25名女孩和11名男孩，男女比例为2.3:1。50%的儿童（n = 18）为沙特人，其余50%来自其他不同种族背景（9名亚洲人、4名阿拉伯人、2名非洲人以及3名来自远东地区）。他们就诊时的平均年龄为4.3±3.0岁（范围1 - 12岁）。就诊时的平均血清白蛋白为每升15.6±7.1克，且所有患儿尿液分析均显示有4+蛋白尿。5名儿童就诊时血清肌酐升高，而平均血清肌酐为50.4±45.6微摩尔/升。3名儿童就诊时血清补体水平较低，且均无乙肝表面抗原或抗核抗体（ANA）阳性。肾脏组织病理学显示，39%（n = 14）符合局灶节段性肾小球硬化（FSGS），28%（n = 10）符合IgM肾病，17%（n = 6）符合系膜增生性肾小球肾炎（MesPGN），微小病变病（MCD）和C1q肾病（C1qNP）各占8%（n = 3 + 3），IgA肾病占3%（n = 1）。我们的回顾性研究表明，FSGS是SRNS患儿最常见的潜在组织病理学类型，其次是IgM肾病以及MCD的其他变体如MesPGN。C1qNP是部分患儿的潜在病因。
