ESPU Meeting on Saturday 20, April 2024, 08:20 - 09:00
08:20 - 08:23
S22-1 (OP)
Sharmila Devi RAMNARINE SANCHEZ 1, Naima SMEULDERS 1, Mohammad BADER 2, Karin TUSCHL 3, Wesley HAYES 4 and Alexander CHO 1
1) Great Ormond Street Hospital, Paediatric Urology, London, UNITED KINGDOM - 2) John Radcliffe Hospital, Paediatric Urology, Oxford, UNITED KINGDOM - 3) Great Ormond Street Hospital, Metabolic Medicine, London, UNITED KINGDOM - 4) Great Ormond Street Hospital, Nephrology, London, UNITED KINGDOM
PURPOSE
Menkes disease (MD) is a rare X-linked disorder of copper-absorption and transport with an incidence of 1:300,000 live-births in Europe. Recognised urological abnormalities include bladder diverticula, neuropathic bladder and VUR. This study explores any association between urolithiasis and MD.
MATERIAL AND METHODS
With institutional approval, all MD-patients on the prospective metabolic medicine database (2011-2022) were reviewed for urinary tract anomalies and urolithiasis. Data is presented as the median and range.
RESULTS
Six boys were diagnosed with MD at median 4.5months (1-17months). UTIs commenced from age 1month-5years (median 27months). Bladder-diverticula were demonstrated in all from 1-3years (median 1.5years), despite previous normal imaging of the bladder in 2. Only 1 had unilateral VUR, managed conservatively. No renal scarring was evident on ultrasound, or DMSA performed in 3. Due to large post-void residuals and recurrent UTI, five required assisted-bladder drainage (3-CIC, 2-Suprapubic catheter).
Urolithiasis was identified in 3(50%), two following symptoms (haematuria-1, pain secondary to obstruction-1) but 1 was an incidental finding on follow-up imaging (impacted PUJ-stone). Stone locations were bilateral upper tract stones in all, with additional layering debris observed within bladder diverticula. Two required emergency intervention for obstruction. Two were eventually managed palliatively after multi-disciplinary discussion. Urinary metabolic assessment showed hypercalciuria-1 and hyperoxaluria-2.
CONCLUSIONS
Urological abnormalities developed in all MD patients. Half also acquired urolithiasis. In order to prevent emergency presentations with complications of renal stones, early and regular ultrasound-screening with urological involvement in a multi-disciplinary team is essential for this complex and rare cohort of patients.
08:23 - 08:25
S22-2 (CP)
Alfredo BERRETTINI 1, Michele GNECH 2, Dario Guido MINOLI 3, Erika Adalgisa DE MARCO 4, Irene PARABOSCHI 5, Gianantonio MANZONI 4, Francesca MITZMAN 4, Giovanni MONTINI 4 and Francesca TARONI 6
1) Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy., Department of Paediatric Urology, Milan, ITALY - 2) Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Department of Paediatric Urology, Milan, ITALY - 3) Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Department of Paediatric Urology,, Milano, ITALY - 4) Fondazione IRCCS Cà Granda Ospedale Maggiore Policlinico, Department of Paediatric Urology, Milan, ITALY - 5) Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico,, Department of Paediatric Urology, Milan, ITALY - 6) Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico,, Pediatric Nephrology, Dialysis and Transplant Unit, Milan, ITALY
PURPOSE
Primary hyperoxaluria type 1 (PH1) is a rare disease with autosomal recessive transmission, characterized by increased renal excretion of calcium oxalate resulting in chronic renal failure secondary to recurrent urolithiasis and nephrocalcinosis, and accumulation of oxalate in various organs and tissues.Since 2020, an innovative pharmacological approach, Lumasiran, has been added to the therapeutic armamentarium. The efficacy has been demonstrated by international clinical trials but there is still limited experience with the use in very young patients. We describe the effect of Lumasiran initiated very early in a patient with prenatally diagnosed PH1.
CASE MANAGEMENT
A female received a prenatal diagnosis of PH1 based on family history. Her brother had the onset of disease at 2 months of age and underwent liver transplantation from a deceased donor at 13 months of age, and kidney transplantation at 8 years of age. She was born via spontaneous labor at 36 weeks + 4 days of gestation. The newborn was immediately started on therapy with Lumasiran for compassionate use administered on the tenth day of life. MS presented nephrocalcinosis at 3 months of age, and then two kidney stones at 9 months of age. At 12 months of age, MS shows normal urinary oxalate values and plasma oxalate values under the value of oversaturation. Renal function appears to be normal and the last ultrasound scan shows improvement in nephrocalcinosis and persistence of the two left kidney stones, unchanged from the previous examination. MS presently shows no signs of systemic oxalosis.
CONCLUSIONS
Early use of Lumasiran in young patients who do not yet show signs of the disease represents a therapeutic challenge for the pediatric nephrologist to date. In fact, while the therapy is shown to be excellently tolerated, some issues need to be clarified such as the ability of the drug to act on the hepatocyte of the newborn, and the most appropriate dosage to be used in this category of patients.
08:25 - 08:28
S22-3 (OP)
Bashir AHMED 1, Sadaf ABA UMAR 2, Sajid SULTAN 2 and Adib-Ul-Hassan RIZVI 2
1) Sindh Institute of Urology & transplantation, Philips G Rensley Department of Paediatric Urology, Karachi, PAKISTAN - 2) Sindh Institute of Urology & transplantation, Philip G Ransley Department of Paediatric Urology, Karachi, PAKISTAN
PURPOSE
To compare the safety, efficacy, and outcome (stone clearance, number of ESWL sessions, and complications) between different age groups and locations of renal stone by ESWL.
MATERIAL AND METHODS
Records of 562 children (603 renal units)with single-renal-stones in different locations(pelvis, upper, middle, and lower calyces) managed by ESWL (StorzModulith®SLX-F2) between January2014 to December2020 were reviewed. Renal units were divided into two age groups: group-I,0-5 years(n=329), and group-II,>5-14years(n=274). Clinical records were reviewed for age, gender, stone-laterality, location, size, number of ESWL-sessions, stone-clearance, and complications. Statistical analysis was done on SPSSv.20.0. ANOVA independent t-test and Chi-square test were used for statistical analysis. p value<0.05 was considered significant.
RESULTS
M:F 2:1
|
Location stone |
Stone N=603 Stone size (cm) |
Renal Unit(M/F:2:1) N=603 |
Stone clearance |
||||
|
Group I (<5yrs) (Mean age2.9+/-1.4yrs) n=329 |
Group II (>5-14yrs) (Mean age8.7+/-2.3yrs) n=274 |
P=value |
Group I <5yrs n=329 |
Group II >5-14yrs n=274 |
P=value |
||
|
Upper Calyx |
Stone no |
13 |
15 |
|
92.3% |
86.7% |
0.55 |
|
Stone size |
1.03+/-0.25 |
0.98+/-0.27 |
0.6 |
||||
|
Mid Calyx |
Stone no |
52 |
51 |
|
92.3% |
82.4% |
0.14 |
|
Stone size |
0.89+/-0.22 |
0.91+/-0.28 |
0.64 |
||||
|
Lower Calyx |
Stone no |
100 |
129 |
|
81% |
85.3% |
0.24 |
|
Stone size |
0.87+/-0.26 |
0.87+/-0.24 |
0.81 |
||||
|
Pelvis |
Stone no |
164 |
79 |
|
87% |
86.1% |
0.47 |
|
Stone size |
1.03+/-0.24 |
1.1+/-0.26 |
0.02 |
||||
|
Overall |
Stone no |
329 |
274 |
|
86.3% |
83.9% |
0.24 |
|
Stone size |
0.96+/-0.26 |
0.96+/-0.28 |
0.87 |
||||
|
Stone clearance with CIRF |
94.8% |
93.1% |
0.39 |
||||
|
ESWL Sessions. |
1.19+/-0.39 |
1.24+/-0.42 |
0.11 |
||||
The overall stone-size, as well as the stone-size with respect to stone-locations, is comparable in both groups. Overall complication (Clavian-Dindo-I)108(17.8%), and (Clavian-Dindo-III)32(5.3%), with no significant difference in the two age groups.
CONCLUSIONS
The study showed that both groups had excellent stone-clearance with acceptable complication rates. The age of children did not effect the stone-clearance viz-a-viz stone-size and stone-location
08:40 - 08:43
S22-4 (OP)
Yesica QUIROZ MADARRIAGA 1, Victoria JAHRREISS 2, Stephen GRIFFIN 2, Erika LLORENS 1, Rocio JIMENEZ 1, Bhaskar SOMANI 2 and Anna BUJONS 1
1) Fundacio Puigvert, Paediatric Urology, Barcelona, SPAIN - 2) University Hospital Southampton NHS Trust, Urology, Southampton, UNITED KINGDOM
PURPOSE
Technological advances have led to miniaturization of instruments especially in the form of single use scopes. These smaller single use scopes seem to be advantageous especially in cases with difficult access to the renal pelvis due to challenging anatomy and might therefore be a valuable asset in the endoscopic stone treatment in children. We have conducted a multicenter study to analyze the outcomes of pediatric patients treated with the Pusen 7.5 Fr single use scopes at our institutions.
MATERIAL AND METHODS
The study was conducted at two large European tertiary endourology centers that specialize in pediatric kidney stone management. We included pediatric patients with urinary stones treated with the small Pusen 7.5 Fr single use ureteroscope. Patient data and outcomes were prospectively collected, and analysis was performed regarding patient demographics, stone parameters, as well as stone free rate (SFR), operating time, and complications.
RESULTS
In this pilot study, 26 patients were included with a median age of 12 years (7.0 - 16.0) and a male to female ratio of 14:12. The mean cumulative stone size was 15.15 mm (SD± 11.1) and multiple stones were present in 9 (34.6%) patients. 12 (46.2%) patients had a preoperatively inserted stent in place. During the procedure a ureteral access sheath (UAS) was used in 23 (88.5%) cases, being smaller in all cases, with respect to the UAS previously used in the institution. The median operative time was 47 minutes (IQR: 40.0 - 63.8). 24 (92.3%) patients were stone free, while no intra or postoperative complications were observed.
CONCLUSIONS
Our study demonstrates that the use of the small 7.5 single use ureteroscope is safe and efficient for the treatment of urinary stones in pediatric patients with high stone-free rates and no complications noted in our series. To confirm and validate our findings further studies with larger cohorts are warranted.
08:43 - 08:46
S22-5 (OP)
Shayel BERCOVICH 1, Hen HENDEL 2, Yossi VENTURA 2, Paz LOTAN 2, Roy MORAG 3, David BEN-MEIR 3 and David LIFSHITZ 2
1) Schneider Children's Medical Center, Urology, Tel Aviv, ISRAEL - 2) RABIN MEDICAL CENTER, Urology, Petah Tikva, ISRAEL - 3) Schneider children's hospital, Urology, Petah Tikva, ISRAEL
PURPOSE
The prevalence of children presenting with renal colic is significantly lower than adults. The presentation as well as the chances of spontaneous expulsion and the rate of intervention may differ between the age groups. The aim of the current study was to assess such potential differences.
MATERIAL AND METHODS
We retrospectively assessed data of a consecutive group of children and adults presenting to the emergency department and diagnosed with a ureteral stone. Clinical characteristics, radiological exams, and the rate of intervention spontaneous stone expulsion were compared between the adult and children cohorts.
RESULTS
106 children were compared to 99 adults who visited the ER for symptomatic ureteral stones. The median stone size was similar between the children and adult group (5 vs. 4.5 mm [IQR 3-6 mm], respectively). Flank pain as the presenting symptom was less common in children compared to adults (67% vs. 88% [X2 p<0.001]), while moderate or severe hydronephrosis was observed more frequently in children compared to adults (35% vs.17% [X2 p<0.001]). In patients treated conservatively the rate of spontaneous stone expulsion was similar between the groups (72% and 73% in the children and adult population, respectively [X2 P=0.8]). However, the rate of immediate intervention was higher in the children group (36% vs 11%, respectively [X2 p<0.001]).
CONCLUSIONS
The current study highlights differences between children and adults presenting to the ER with a ureteral stone. Diagnosis in children requires a higher index of suspicion as presentation may be less typical as evident by the lower rate of flank pain and a higher rate of hyderonephrosis that may suggest delayed diagnosis. Children are more likely to undergo immediate intervention probably due to difficulties in pain management. However when allowed expectant therapy, the rate of spontaneous stone expulsion is similar between the groups.
08:46 - 08:49
S22-6 (OP)
Mohamed EDWAN 1, Mohamed HUSSINY 1, Ahmed ABDELHALIM 1, Hassan ABOL-ENEIN 1, Ahmed ELASSMY 1, Abdalla ABDELHAMID 2, Mohamed ABO EL GHAR 2, Mohamed E. DAWABA 1, Tamer HELMY 1 and Ashraf T. HAFEZ 1
1) Urology and Nephrology center - Mansoura University, Urology department, Mansoura, EGYPT - 2) Urology and Nephrology center - Mansoura University, Radiology department, Mansoura, EGYPT
PURPOSE
Shear Wave Elastography (SWE) is a noninvasive technique that gives a numerical value to show the stiffness. After a minimally invasive procedures such as PCNL and ESWL, the tissues become stiffer. Our aim is to evaluate the value of ultrasound elastography in detection of renal scars in such group of patients.
MATERIAL AND METHODS
We included 120 renal units (49 which had ESWL and 71 which had PCNL) at our center between 2014-2021. The elasticity at the intervention site was evaluated by SWE and compared to the normal contralateral kidney which was carried out by a single radiologist. Also, evaluation of the renal function using DMSA scan was done. All patients were ≤18 years old. Exclusion criteria included patients with other congenital anomalies of the urinary system e.g (UPJO), patients with augmentation ileocystoplasty and patients who underwent bilateral stone intervention.
RESULTS
Renal scaring was detected in 19 renal units by DMSA scan (6 which had ESWL(12.24%) and 13 which had PCNL(18.3%)). Out of these 19 patients, 14 (73.68%) patients showed increased renal stiffness which is statistically significant. The sensitivity and specificity of the elastography was 73% and 87% respectively. There was insignificant difference between SWE in the treated units compared to the control units (2.94±1.14 m/s compared to 2.30 ±0.62 m/s in ESWL group (P =0.15) and 2.6±1.2 m/s compared to 2.30 ±0.62 m/s in PCNL group (P=0.2).
CONCLUSIONS
Shear wave elastography can be a valuable tool in detection of renal scar tissue post ESWL or PCNL with a high sensitivity and specificity. It is cost effective and may replace the risky radiation exposure from radioisotope studies.