Vinit Nicolas, Khoury Antoine, Lopez Pauline, Heidet Laurence, Botto Nathalie, Traxer Olivier, Boyer Olivia, Blanc Thomas, Lottmann Henri B
Department of Pediatric Surgery and Urology, Necker-Enfants Malades Hospital, APHP, Paris, France.
Department of Pediatric Nephrology, Reference Center for Inherited Renal Disease (MARHEA), Necker-Enfants Malades Hospital, APHP, Paris, France.
Front Pediatr. 2021 Nov 16;9:763317. doi: 10.3389/fped.2021.763317. eCollection 2021.
Cystinuria is a genetic disorder characterized by a defective reabsorption of cystine and dibasic amino acids leading to development of urinary tract calculi from childhood onward. Cystine lithiasis is known to be resistant to fragmentation. The aim was to evaluate our long-term experience with extracorporeal shockwave lithotripsy (ESWL) used as first-line urological treatment to treat cystine stones in children. We retrospectively reviewed the charts of all children who underwent ESWL for cystine stone. We assessed the 3-month stone-free rate, according to age, younger (group 1) or older (group 2) than 2 years old. Between 2003 and 2016, 15 patients with a median (IQR) age at first treatment of 48 (15-108) months underwent ESWL in monotherapy. Median age was, respectively, 15 and 108 months in each group. The median (IQR) stone burden was 2,620 (1,202-8,265) mm in group I and 4,588 (2,039-5,427) mm in group II ( = 0.96). Eleven patients had bilateral calculi. ESWL was repeated on average 2.4 times, with a maximum of 4 for patients of group I, and 4.8 times, with a maximum of 9 for group II ( > 0.05). ESWL in monotherapy was significantly more efficient to reach stone-free status for children under 2 years of age: 83% vs. 6.2% ( = 0.040). The median (IQR) follow-up of the study was 69 (42-111) months. ESWL appears as a valid urological option for the treatment of cystine stones, in young children. Even if cystine stones are known to be resistant to fragmentation, we report 83% of stone-free status at 3 months with ESWL used in monotherapy in children under 2 years old with cystinuria. In older children, the success rate is too low to recommend ESWL as a first line approach.
胱氨酸尿症是一种遗传性疾病,其特征是胱氨酸和二碱基氨基酸的重吸收存在缺陷,导致从儿童期起就会出现尿路结石。已知胱氨酸结石难以破碎。本研究旨在评估我们将体外冲击波碎石术(ESWL)作为儿童胱氨酸结石一线泌尿外科治疗方法的长期经验。我们回顾性分析了所有接受ESWL治疗胱氨酸结石儿童的病历。我们根据年龄(2岁以下为较年幼组,即第1组;2岁及以上为较年长组,即第2组)评估了3个月时的结石清除率。2003年至2016年期间,15例首次治疗时中位(四分位间距)年龄为48(15 - 108)个月的患者接受了ESWL单一疗法治疗。每组的中位年龄分别为15个月和108个月。第1组结石负荷的中位(四分位间距)为2620(1202 - 8265)mm,第2组为4588(2039 - 5427)mm(P = 0.96)。11例患者有双侧结石。ESWL平均重复2.4次,第1组患者最多重复4次,第2组为4.8次,最多重复9次(P>0.05)。ESWL单一疗法对于2岁以下儿童达到结石清除状态的效率显著更高:83% 对比6.2%(P = 0.040)。本研究的中位(四分位间距)随访时间为69(42 - 111)个月。对于幼儿胱氨酸结石的治疗,ESWL似乎是一种有效的泌尿外科治疗选择。即使已知胱氨酸结石难以破碎,但我们报告在2岁以下患有胱氨酸尿症的儿童中,采用ESWL单一疗法在3个月时结石清除率达83%。在年龄较大的儿童中,成功率过低,不建议将ESWL作为一线治疗方法。