Shakeel Shaheera, Mubarak Muhammed, I Kazi Javed, Jafry Nazrul, Ahmed Ejaz
Histopathology Department, Sindh Institute of Urology and Transplantation, Karachi, Pakistan.
Nephrology Department, Sindh Institute of Urology and Transplantation, Karachi, Pakistan.
J Nephropathol. 2013 Jan;2(1):28-35. doi: 10.5812/nephropathol.8959. Epub 2013 Jan 1.
There is no information on the frequency and clinicopathological presentation of the variants of primary focal segmental glomerulosclerosis (FSGS) in adults presenting with idiopathic nephrotic syndrome (INS) in Pakistan.
The aim of this study was to determine the frequencies of different histologic variants of primary FSGS with INS at our center and to compare our findings with those published in literature.
All consecutive adults (≥18 years) with INS, and diagnosis of FSGS on renal biopsies, were included. Their clinicopathological features at the time of presentation were retrieved and compared among the variants.
There were 120 (65.2%) males and 64 (34.8%) females. The mean age was 30.62±12.02 years. The mean 24-hr urinary protein excretion was 4.69±2.36 grams. Microscopic hematuria was found in 30 (16.3%) patients. The mean serum creatinine was 1.58±0.87 mg/dL. At presentation, 128 (69.6%) patients were normotensive, while 56 (30.4%) exhibited hypertension. FSGS, not otherwise specified (NOS) was the predominant variant, comprising 76.6% of all; collapsing variant comprised 12%, tip variant, 9.8%, perihilar, 1.1%, and cellular, 0.5%. The mean number of glomeruli involved by segmental scarring was 3.41±2.87 and there was significant difference among the variants (p= 0.001). Arteriolopathy was found in 23.4 % cases and fibrointimal thickening of arteries in 18.5%. Tubular atrophy and interstitial fibrosis (IF/TA) was noted in 93% of cases. There was no significant difference in vasculopathy and IF/TA among the variants.
Collapsing variant was the second most common variant following NOS and these findings are different from other regional studies.
在巴基斯坦，尚无关于原发性局灶节段性肾小球硬化（FSGS）变异型在特发性肾病综合征（INS）成人患者中的发病率及临床病理表现的相关信息。
本研究旨在确定我院原发性FSGS合并INS的不同组织学变异型的发病率，并将我们的研究结果与文献报道进行比较。
纳入所有连续的成人（≥18岁）INS患者，且肾活检诊断为FSGS。检索他们就诊时的临床病理特征，并在各变异型之间进行比较。
男性120例（65.2%），女性64例（34.8%）。平均年龄为30.62±12.02岁。24小时尿蛋白排泄量平均为4.69±2.36克。30例（16.3%）患者出现镜下血尿。血清肌酐平均为1.58±0.87mg/dL。就诊时，128例（69.6%）患者血压正常，56例（30.4%）患者有高血压。未另行指定的FSGS（NOS）是主要变异型，占所有病例的76.6%；塌陷型变异占12%，顶端型变异占9.8%，肾门周围型占1.1%，细胞型占0.5%。节段性瘢痕累及的肾小球平均数量为3.41±2.87个，各变异型之间存在显著差异（p=0.001）。23.4%的病例发现有小动脉病变，18.5%的病例有动脉纤维内膜增厚。93%的病例有肾小管萎缩和间质纤维化（IF/TA）。各变异型之间在血管病变和IF/TA方面无显著差异。
塌陷型变异是仅次于NOS的第二常见变异型，这些发现与其他地区的研究不同。
