Nephrological Outcome and Associated Congenital Anomalies in Pediatric Patients with Horseshoe Kidney
试验速览
- 阶段
- 不适用
- 状态
- 招募中
- 入组人数
- 500
- 试验地点
- 1
- 主要终点
- Chronic kidney disease
研究概览
简要总结
A retrospective, observational multicentre trial on the nephrological outcome and associated congenital anomalies in children with Horseshoe Kidney.
详细描述
Congenital abnormalities of the kidney and urinary tract (CAKUT) represent the main cause of CKD in children. Among them, the horseshoe kidney (HSK) represents one of the most frequent and is characterised by the presence of two distinct, functioning kidneys positioned on each side of the spine, fused together at one of the poles. The incidence of this condition is approximately 1 case every 400-600 new births with a prevalence in the male sex with a ratio of 2:1. Even though these children aregenerally asymptomatic and the diagnosis is often incidental , some may develop symptoms due to complications, such as infections, nephrolithiasis and urinary tract obstruction. More rarely risks of neoplastic degeneration and renal damage are described following trauma to the abdomen and lumbosacral spine. This condition is also associated in up to a third of cases with other abnormalities: the most frequent affect the urinary tract and genitals; however, abnormalities affecting other organs or systems as well as well-defined syndromic pictures. Due to its frequent asymptomatic nature, horseshoe kidney is historically considered a condition with a good prognosis and rarely as a risk factor capable of reducing survival or predisposing the kidney to long-term damage.
The primary objective of the study is:
To assess the 'nephrological outcome' understood as 'prevalence of patients who developed' and 'survival time free from": chronic renal failure, proteinuria, hypertension nephrolithiasis, UTI, renal neoplasms and renal trauma.
The secondary objectives of the study are:
- Prevalence of congenital and associated genito-urinary and systemic abnormalities;
- Description of renal anatomical features;
- Assessment of the usefulness of second level radiological investigations (VCUG, static renal scintigraphy, ddynamic renal scintigraphy).
研究设计
- 研究类型
- Observational
- 观察模型
- Cohort
- 时间视角
- Retrospective
入排标准
- 年龄范围
- — 至 18 Years（Child, Adult）
- 性别
- All
- 接受健康志愿者
- 否
入选标准
- •Patients with confirmed ultrasound and/or scintigraphic diagnosis of horseshoe kidney;
- •Patients with an age at first assessment of between 0 and 18 years;
- •Obtaining written Informed consent.
排除标准
- •Patients with a definitive diagnosis of another form of CAKUT during the diagnostic pathway.
结局指标
主要结局
Chronic kidney disease
时间窗: at baseline
Defined as a reduction in eGFR \<90ml/min/1.73m2, calculated using the Schwartz formula(18) corrected for age: * δ= 0.45 for children \<1 yr * δ= 0.55 for children of both sexes from 2 to 12 yrs and for female children aged 13 to 18 yrs * δ= 0.70 for children aged 13 to 18 yrs The different stages of chronic kidney have been further defined according to eGFR: * Stage I: \>90ml/min/1.73m2 * Stage II: between 89 and 60ml/min/1.73m2 * Stage III: between 59 and 30ml/min/1.73m2 * Stage IV: between 30 and 15ml/min/1.73m2 * Stadio V: lower than15ml/min/1.73m2 or patient in dialysis
Proteinuria
时间窗: at baseline
Defined as the urinary protein:creatinine ratio (mg/mg) \>0.5 in chilrden up to 2 yrs of age and \>0.2 in chilrden older than 2 yrs
Hypertension
时间窗: at baseline
Arterial blood pressure ≥95th percentile for sex, age and height, on at least three different readings
次要结局
- Urinary tract infections(at baseline)
- Nephrolithiasis(at baseline)
- Renal neoplasms(at baseline)
- Renal anatomical features(at baseline)
- Renal trauma(at baseline)