Kessler O J, Ziv N, Livne P M, Merlob P
Department of Urology, Schneider Children's Medical Center of Israel, Tel Aviv, Israel.
Pediatrics. 1998 Dec;102(6):E73. doi: 10.1542/peds.102.6.e73.
To document the involution rate and long-term results of management of multicystic dysplastic kidney.
Data were collected retrospectively for all 23 infants (16 boys) with multicystic dysplastic kidney who were treated at our center over the last 19 years (1977-1995). The diagnosis was based on prenatal ultrasound in 18 patients and on palpable abdominal mass in 5, and confirmed in all patients by postnatal ultrasound and radioisotope scan. Voiding cystography was performed in 18 patients to exclude vesicoureteral reflux. Mean follow-up was 46 months (range, 3 months to 5 years) and included serum creatinine measurements and renal ultrasonography.
Two groups of patients were identified. Ten (43.6%) with other urologic abnormalities (group A) and 13 patients without other urologic abnormalities (group B). Vesicoureteral reflux was observed in 4 patients. Nephrectomy was performed in 4 patients, all from group B. The other 19 patients were treated conservatively. Complete involution was observed in 8 patients in group A and 6 in group B after a mean follow-up period of 9.2 and 10 months, respectively. Two patients, 1 from each group, later underwent nephrectomy not because of no involution but because of an increase in the size of the kidney involved.
Patients with multicystic renal dysplasia have significant associated urologic malformations, and the natural history of the disease is unpredictable. All patients require appropriate investigation of the urinary tract and long-term follow-up. The most outstanding finding of the study is the much higher involution rate of multicystic renal dysplasia and the rate of associated urologic abnormalities than that reported in the literature. Surgery remains an option for the patients in the absence of no involution. multicystic dysplastic kidney, surgery, conservative.
记录多囊性发育不良肾的退化率及长期治疗结果。
回顾性收集过去19年（1977 - 1995年）在本中心接受治疗的23例多囊性发育不良肾婴儿（16例男孩）的数据。18例患者的诊断基于产前超声，5例基于可触及的腹部肿块，所有患者均经产后超声和放射性核素扫描确诊。18例患者进行了排尿性膀胱尿道造影以排除膀胱输尿管反流。平均随访46个月（范围3个月至5年），包括血清肌酐测量和肾脏超声检查。
确定了两组患者。10例（43.6%）伴有其他泌尿系统异常（A组），13例无其他泌尿系统异常（B组）。4例患者观察到膀胱输尿管反流。4例患者接受了肾切除术，均来自B组。其他19例患者接受保守治疗。A组8例患者和B组6例患者分别在平均随访9.2个月和10个月后观察到完全退化。两组各有1例患者后来接受了肾切除术，并非因为未退化，而是因为患侧肾脏增大。
多囊性肾发育不良患者伴有明显的相关泌尿系统畸形，疾病的自然病程不可预测。所有患者都需要对尿路进行适当检查并进行长期随访。该研究最突出的发现是多囊性肾发育不良的退化率及相关泌尿系统异常的发生率比文献报道的要高得多。在未出现退化的情况下，手术仍是患者的一种选择。多囊性发育不良肾、手术、保守治疗。
