Kaiserová E, Furková K, Slugen I, Páleníková O
Detská klinika NsP ak. L. Dérera, Bratislava.
Cesk Pediatr. 1991 Mar;46(3):129-38.
The authors evaluated the course and prognosis of the disease in 109 children with minor glomerular abnormalities manifested clinically in 45.9% as nephrotic syndrome (NS), in 33% as nephritic syndrome (GN), in 11.9% as isolated haematuria (IH) and in 9.2% as Schönlein-Henoch's purpura (PSH). In NS 78% of the children had before biopsy of the kidneys frequent relapses, 22% were resistant to cortisonoids. After biopsy all children were given cortisonoids, 94% immunosuppressive treatment with cytostatics and some of the children additional treatment. The number of resistant cases declined to 10% and the mean number of relapses from four to one in 12 months. Children under five years had more relapses (P less than 0.05) but also more complete remissions (P less than 0.001) than older children. Relapses occurred up to 10.2 years after the onset of the disease (mean = 4 years). With advancing age and duration of the disease their number declined after treatment. An adverse symptom was resistance to cortisonoids and immunosuppressive treatment, major haematuria and persisting hypertension but not immunological activity (elevated level of immune complexes, reduced C3, positive immunohistochemical finding in renal tissue). The morphological finding which at the onset was slightly beyond the range of minor abnormalities had a poorer prognosis when associated with greater clinical activity. The group developed 88% complete remissions and 6% CHRI. After 22 years the probability of survival in complete continual remission is 66%, the probability in CHRI is 10% (with morphological progression). In nephritic syndrome the children were given after biopsy prednisone in 80.6% and cytostatics in 44.4%. In PSH this treatment was given to 100% and 60% of the children, in IH to 61.5% and 7.7%. On evaluation in nephritic syndrome complete remission was recorded in 47.2%, after 0.4-10.5 years since the onset of the disease; 30.6% did not improve and in 2.8% CHRI developed. In PSH remission developed in 60% after 0.8-6.9 years, no improvement was recorded in 20%, incl. 10% where CHRI developed after a resistant course of NS. In IH 84.6% of the patients did not improve, but in none the renal function deteriorated. The course was in all instances milder than in NS, most frequently only with microscopic haematuria and/or slight proteinuria, respectively minor immunological activity. In the entire group of minor glomerular abnormalities complete remission was achieved in two-thirds of the children, in one quarter the disease did not improve, incl. 4.6% where CHRI developed, always associated with progression of morphological changes.(ABSTRACT TRUNCATED AT 400 WORDS)
作者评估了109例患有轻度肾小球异常的儿童的病情发展及预后情况。临床上，45.9%表现为肾病综合征（NS），33%表现为肾炎综合征（GN），11.9%表现为孤立性血尿（IH），9.2%表现为过敏性紫癜（PSH）。在NS组中，78%的儿童在肾脏活检前频繁复发，22%对皮质类固醇耐药。活检后，所有儿童均接受皮质类固醇治疗，94%接受细胞毒性药物免疫抑制治疗，部分儿童还接受了其他治疗。耐药病例数降至10%，12个月内平均复发次数从4次降至1次。5岁以下儿童的复发次数更多（P<0.05），但完全缓解的次数也比大龄儿童更多（P<0.001）。疾病发作后长达10.2年仍有复发（平均4年）。随着年龄增长和病程延长，治疗后复发次数减少。不良症状包括对皮质类固醇和免疫抑制治疗耐药、大量血尿和持续性高血压，但不包括免疫活性（免疫复合物水平升高、C3降低、肾组织免疫组化结果阳性）。发病时形态学表现略超出轻度异常范围的，若伴有更严重的临床活动，预后较差。该组88%实现完全缓解，6%发生慢性肾功能不全（CHRI）。22年后，持续完全缓解的生存概率为66%，CHRI的生存概率为10%（伴有形态学进展）。在肾炎综合征组中，80.6%的儿童活检后接受泼尼松治疗，44.4%接受细胞毒性药物治疗。在PSH组中，100%的儿童接受了这种治疗，60%接受了细胞毒性药物治疗；在IH组中，61.5%和7.7%的儿童接受了相应治疗。评估显示，肾炎综合征组在疾病发作后0.4 - 10.5年，47.2%实现完全缓解；30.6%无改善，2.8%发生CHRI。PSH组在0.8 - 6.9年后60%实现缓解，20%无改善，其中10%在经历耐药性NS病程后发生CHRI。IH组中84.6%的患者无改善，但肾功能均未恶化。所有病例的病程均比NS组轻，最常见的情况分别只是镜下血尿和/或轻度蛋白尿，以及轻微免疫活性。在整个轻度肾小球异常组中，三分之二的儿童实现完全缓解，四分之一的儿童病情无改善，其中4.6%发生CHRI，且均伴有形态学改变的进展。（摘要截断于400字）
