Steroid-resistant nephrotic syndrome.
Steroid-resistant nephrotic syndrome.
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DOI：
10.1038/ki.2008.297
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发表时间：
2008-11
影响因子：
19.6
通讯作者：
A. Kitamura;H. Tsukaguchi;K. Maruyama;A. Shono;K. Iijima;S. Kagami;T. Doi
中科院分区：
文献类型：
--
作者：
A. Kitamura;H. Tsukaguchi;K. Maruyama;A. Shono;K. Iijima;S. Kagami;T. Doi
一名5岁的女性被转诊为激素抵抗性肾病综合征（SRNS）的评价。3岁时，她的家人担心蛋白尿（3+）和血尿（2+），并将她带到医院进行评估。无前驱感染、发热或皮疹。未发现肾脏疾病家族史，其父母和哥哥的尿分析结果均为阴性（图1a）。初次访视时，实验室检查显示低蛋白血症（血清白蛋白3.1 g/100 ml）和高脂血症（总胆固醇282 mg/100 ml），而肾功能正常（血清肌酐0.2 mg/100 ml，估计肾小球滤过率135 ml/min/1.73 m2，图1b、c和表1）。到医院就诊时，患者表现正常。体格检查无异常，生长正常;身高为98.2 cm（+ 0.8 sd，68百分位数），体重为13.0 kg（-0.5 sd，38百分位数）。没有畸形特征、水肿、关节肿胀或听力损失。包括心血管、神经系统和眼科检查在内的全身检查无异常。肾脏超音波显示肾脏大小正常，对称，无肾积水或囊肿。包括计算机断层扫描在内的泌尿系统检查正常。
A 5-year-old female was referred for evaluation of steroid-resistant nephrotic syndrome (SRNS). At 3 years of age, her family was concerned about the proteinuria (3+) and hematuria (2+) and brought her to the hospital for evaluation. There was no antecedent infection, fever, or rash. No family history of renal disease was noted, and her parents and her elder brother were negative for urinalysis (Figure 1a). At the initial visit, laboratory studies revealed hypoproteinemia (serum albumin 3.1 g/100 ml) and hyperlipidemia (total cholesterol 282 mg/100 ml), whereas renal function was normal (serum creatinine 0.2 mg/100 ml, and an estimated glomerular filtration rate 135 ml/min/1.73 m 2, Figure 1b, c and Table 1). On presentation to the hospital, the patient appeared normal. Physical examination was unremarkable with normal growth; the height was 98.2 cm (+ 0.8 sd, 68 percentile) and weight was 13.0 kg (− 0.5 sd, 38 percentile). There was no dysmorphic features, edema, joint swelling, or hearing loss. The systemic workup including cardiovascular, neurological, and ophthalmological examination was unremarkable. Renal sonogram demonstrated normal-sized, symmetric kidneys without hydronephrosis or cysts. Urological studies including computed tomography were normal.