Horseshoe Kidney
Conditions
Keywords
Horseshoe Kidney, Congenital anomalies, Children, CAKUT
Brief summary
A retrospective, observational multicentre trial on the nephrological outcome and associated congenital anomalies in children with Horseshoe Kidney.
Detailed description
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: 1. Prevalence of congenital and associated genito-urinary and systemic abnormalities; 2. Description of renal anatomical features; 3. Assessment of the usefulness of second level radiological investigations (VCUG, static renal scintigraphy, ddynamic renal scintigraphy).
Interventions
None listed
Sponsors
IRCCS Azienda Ospedaliero-Universitaria di Bologna
Study design
Observational model
COHORT
Time perspective
RETROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
No minimum to 18 Years
Healthy volunteers
No
Inclusion criteria
* 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.
Exclusion criteria
* Patients with a definitive diagnosis of another form of CAKUT during the diagnostic pathway.
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Chronic kidney disease | 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 | 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 | at baseline | Arterial blood pressure ≥95th percentile for sex, age and height, on at least three different readings |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Renal anatomical features | at baseline | Renal length measured as bipolar diameter with renal hypoplasia defined as the presence of kidneys with DBP \<5° centile reference for age and height |
| Urinary tract infections | at baseline | Confirmed by urine analysis and urine culture in a compatible clinical picture |
| Renal trauma | at baseline | Presence of instrumentally documented renal lesions (ultrasound and/or CT scan) following a traumatic episode |
| Nephrolithiasis | at baseline | Documented finding by imaging techniques (ultrasound and/or CT) of formations compatible with lithiasis formation |
| Renal neoplasms | at baseline | Diagnosis confirmed by imaging and histology of the neoplasm of renal origin (especially Wilms' tumour) |
Countries
Italy
Contacts
Primary ContactClaudio La Scola, MD, PhD
Outcome results
None listed