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Purpose We evaluated the clinical course of patients prenatally diagnosed and enrolled early with congenital solitary functioning kidney, and identified the risk factors for renal injury. Materials and Methods We retrospectively evaluated 322 patients with congenital solitary functioning kidney according to the inclusion criteria of 1) prenatal diagnosis of solitary kidney; 2) first evaluation at 1 to 3 months of life with confirmation of congenital solitary functioning kidney, and evaluation of possible associated congenital anomalies of the kidney and urinary tract by abdominal ultrasound, renal scintigraphy and cystography; and 3) absence of any condition potentially affecting renal function in the neonatal period as well as absence of renal injury at enrollment (1 to 3 months of life) confirmed by a normal estimated glomerular filtration rate, lack of proteinuria and hypertension. Followup of 306 patients was evaluated. Results Median followup was 7.2 years (range 1 to 23) and 1 or more signs of renal injury were found in 12 of 306 patients (3.9%). Considering the entire population the cumulative proportion of patients free from renal injury at 17 years old was 93.7%, vs 81.3% and 95.9% for subjects with and those without congenital anomalies of the kidney and urinary tract of congenital solitary functioning kidney (p <0.001), respectively. Of congenital anomalies of the kidney and urinary tract, congenital solitary functioning kidney resulted in significant risk factors for renal injury (HR 8.75, 95% CI 2.77–27.65). Conclusions In an evaluation of a large cohort of patients enrolled early with congenital solitary functioning kidney with a prenatal diagnosis, excluding those with neonatal onset of renal damage, the prevalence of renal damage was 3.9%. Among congenital anomalies of the kidney and urinary tract, congenital solitary functioning kidney represented the major risk factor. [ABSTRACT FROM AUTHOR]

Summary Background Most children with symptoms of urolithiasis and urinary solute excretion abnormalities leading to stone formation have no calculi revealed by ultrasound or X-ray plain film (“occult urolithiasis”). This covers a large group of children presenting with common symptoms such as abdominal pain, hematuria, and dysuria, often faced by general practitioners and pediatricians. However, half or more of children with urolithiasis could present with abdominal/flank pain without specific urinary symptoms. Study design We review the current evidence about prevalence, clinical presentation, and radiological detection of overt and “occult” urolithiasis in children, aiming to give readers the instruments to suspect and diagnose urolithiasis while avoiding cost-ineffective and undue diagnostic procedures. Conclusions It is important to investigate for urolithiasis first by ultrasound and, in specific cases, by urinary metabolic and different imaging studies in the following groups: 1) in children with non-glomerular hematuria or/and dysuria not presenting inflammation of external genitalia; 2) in children with acute/sub-acute or infrequent recurrent abdominal pain and family history of urolithiasis in first or second degree relatives or being at higher risk of developing stones although hematuria and dysuria are lacking; 3) in children under 8 years old, even though pain is central or diffuse to the whole abdomen; and 4) in children presenting risk factors or conditions predisposing to urolithiasis. Finally, it seems reasonable to repeat ultrasound 1–2 years later also in children with “occult” urolithiasis and high risk of developing stones to detect any (re-)appearance of calculi. [ABSTRACT FROM AUTHOR]

Abstract: Objective: To estimate dietary protein intake in children with idiopathic hypercalciuria (HC) and/or hyperuricosuria (HU). Patients and methods: We compared the 24-h urinary excretion of urea, as a reflection of protein intake, in four age- and sex-matched groups, each comprising 56 consecutive children: (1) HC, (2) HU, (3) HC+HU and (4) control. Results: Urinary urea excretion was significantly higher in HC, HU and HC+HU than in controls. HC and HU children had similar urea excretion. HC+HU children had urinary urea significantly higher than HC and HU, but urinary calcium similar to HC and urinary uric acid excretion similar to HU subjects. Urinary calcium was significantly (R 2 =0.21) correlated with urea excretion in HC children only, whereas urinary uric acid was significantly (R 2 =0.21) correlated with urinary urea in HU children only. No significant correlation between urinary urea and calcium or uric acid excretion was found in HC+HU patients although they had the highest urinary urea. A significant (p =0.004) interaction between urinary urea and sodium in increasing urinary calcium excretion resulted only in the HC group. Conclusion: The association of dietary protein excess with HC and/or HU is conditioned by an individual (genetic?) predisposition and may be produced by different mechanisms. [Copyright &y& Elsevier]

Purpose: We determined the long-term evolution of renal damage associated with vesicoureteral reflux. Materials and Methods: We retrospectively selected 74 consecutive children with unilateral primary vesicoureteral reflux, ipsilateral renal differential uptake less than 45% at dimercapto-succinic acid scintigraphy performed 4 to 6 months after urinary tract infection (60 patients) or shortly after diagnosis of vesicoureteral reflux investigated for prenatal hydronephrosis (14), and normal ultrasound and scintigraphic imaging of the contralateral nonrefluxing kidney. Average patient age at diagnosis was 3 years. The outcome was assessed via dimercapto-succinic acid scan at 5 to 24 years (mean 8.9). Results: In 65 patients (88%) variations of less than 5% in differential uptake were recorded. Three patients (4%) showed an increase of greater than 5% in differential uptake of the refluxing kidney. Six patients (8%) demonstrated a decrease of greater than 5%, of whom 3 had 1 and 3 had no febrile urinary tract infection during followup. A total of 18 patients had a differential uptake of 35% to 45% at the first visit, of whom 3 exhibited a decrease of 5.2% to 27% in differential uptake and had no history of febrile urinary tract infection. Conclusions: In most cases differential uptake of the unilaterally refluxing affected kidney remains stable from early childhood to puberty despite the increase in body mass, which necessitates increasing renal work. In some patients a significant decrease in differential uptake may be observed even in the absence of recurrent febrile urinary tract infections. A mild decrease in differential uptake (35% to 45%) at diagnosis does not exclude the possibility of a subsequent significant decrease, even in the absence of febrile urinary tract infection. [Copyright &y& Elsevier]

Abstract: Objective: In adults, it is well known that high protein intake may increase the risk of nephrolithiasis through increased urinary calcium excretion and other mechanisms. We aimed to assess the role of protein intake on urinary calcium excretion in children with idiopathic hypercalciuria (HC). Patients and methods: The 24-h urinary urea excretion as a reflection of protein intake was evaluated in 65 children with HC and in 76 normocalciuric control children. All of them were on a free diet and did not receive any specific drug. Results: Urinary urea excretion was 18.9±6.6 (SD)mmol/kg/day in the HC group and 14.2±4.9 in controls (P <0.0001). Urinary urea excretion significantly decreased with age, as well as with body weight and height gain in both groups. Calcium excretion significantly increased with increasing sodium and urea excretion in the HC but not the control group. There was a significant (P =0.005) interaction between urinary sodium and urea excretion in the rising calciuria of the HC group. Multiple regression analysis showed that, in the HC group, variations in urinary urea explained 11.4% of the entire variability of urinary calcium excretion, whereas adding the urinary sodium effect increased this relation only to 16%. Conclusion: Children with HC have a higher dietary protein intake than normocalciuric children. The decrease in urea excretion with increasing age and body mass may reflect the relatively higher protein intake of young growing individuals. Salt and protein have a cumulative effect on rising calcium excretion in HC children, but the role of protein intake is outstanding. [Copyright &y& Elsevier]

Abstract: Recurrent abdominal pains (RAPs) represent a common problem in children sometimes leading to unnecessary and invasive procedures. The rates of appendectomy were evaluated consecutively in 180 children with idiopathic hypercalciuria (HC) and/or hyperuricosuria (HU) and RAPs, and in 270 control subjects. Of the HC/HU patients 10% and of controls 1.5% underwent appendectomy (p &#60;0.0001). In 15 out of 18 HC/HU patients appendectomized, RAPs persisted with the same frequency and severity after the operation; 10 out of 18 had dysuria and/or gross hematuria 2 days–12 months after appendectomy. At our first visit, urinalysis was negative for hematuria in 13 out of 18 patients and in eight out of the 10 with a previous history of dysuria or gross hematuria. Pain recurrences resolved or improved in nine out of the 11 patients followed at least 1 year after general or specific therapy for HC/HU. The inconstant association of dysuria and hematuria with RAPs in children with HC/HU may lead to the urological origin of pain being overlooked, and may explain the high rate of appendectomy among these children. The possibility of HC/HU therefore should be taken into account in children with RAPs even when dysuria and hematuria are not present. [Copyright &y& Elsevier]

Background: In our clinical practice we observed that -differently from patients with defined glomerulopathies- children with persistent isolated microscopic hematuria (PImH) usually shows at dipstick on first compared with second morning voiding lower degree of hematuria. We hypothesized that comparison of hematuria degree between first and second morning voiding (dipstick comparison test) could help in selection of patients needing of specific investigations. In this study, we aimed preliminarily validating our hypothesis.Methods: To define the risk of active glomerulopathies we used patients with defined glomerulopathies as cases. We prospectively enrolled 103 children during a one-year period. Sixty-nine of them had PImH, 16 microscopic/macroscopic hematuria (mMH), and 18 defined glomerulopathies. Inclusion criteria were: 1) persistent microscopic hematuria (≥1+ at dipstick) with or without defined glomerulopathy and/or previous episodes of macroscopic hematuria; 2) for the patients without defined glomerulopathy, having made regular biannual follow-up visits for at least 2 years in our department; 3) normal renal function and blood pressure at enrollment visit. We compared hematuria degree between first and second morning voiding. Possible findings were absence (NH), less degree (LH), similar degree (SH), and higher degree (HH) of hematuria in the first compared with second voiding.Results: At dipstick comparison test, 27.2% of patients presented NH, 44.7% LH, 28.1% SH, and none HH. NH showed specificity and PPV of 100% and significant area under receiver operating characteristic curve (AUROC = 0.66; 95%CI:0.55-0.78; p = 0.03) for absence of defined glomerulopathies. LH showed better sensitivity (76.9% Vs 33%) and NPV (78.9% Vs 24%) and lower specificity (88.2% Vs 100%) and PPV (87.0% Vs 100%) than NH for absence of defined glomerulopathies. Moreover, the LH showed a non-significant AUROC curve for presence of defined glomerulopathies. SH showed specificity of 80%, NPV of 91.9% and significant AUROC curve for presence of defined glomerulopathies (0.67; 95%CI:0.55-0.80; p = 0.009). The ROC curve analysis examining the delta of hematuria degree comparing first with second morning urine voiding showed a significant AUROC curve (AUROC = 0.78; 95%CI,0.68-0.89; p = 0.0002) with delta ≤ 1 showing the best sensitivity (80%) and specificity (66.7%) for defined glomerulopathies. None of the patients with defined glomerulopathies presented NH. Having SH presented relative risk for defined glomerulopathy of 5.1 (95%CI:1.9-13.6; p = 0.0007). Conversely, taken together NH and LH the RR was 0.2 (95%CI 0.1-0.5; p = 0.0007).Interpretations: The dipstick on the first urine voiding underestimates the hematuria in about 70% of patients. The dipstick comparison test might easily screen patients with PImH and mMH needing of further investigations and treatment. [ABSTRACT FROM AUTHOR]

Bladder ultrasound is becoming pivotal in the management and treatment of lower urinary tract dysfunction. There is a paucity of data regarding intra-observer and interobserver reliability of bladder ultrasound, especially in children. A previous study assessed interobserver agreement for both the postvoid residual volume and measurement of the bladder dimensions in adults showing excellent agreement. To examine the interobserver and intra-observer reliability of bladder wall thickness, bladder urinary and postvoid residual urinary volume using ultrasound evaluated by paediatricians having different training levels. Four sonographers, 3 pediatric trainees and one experienced pediatric urologist measured the full bladder volume, the voiding residual volume, and the bladder wall thickness. Each sonographer made 3 measurements of each parameter. We assessed the interobserver and intra-observer variability by using intraclass correlations (ICCs). ICCs were calculated and tested with a significance level of 5%. The interrater ICC was calculated from the mean of the three measurements of each variable (full bladder volume, postvoid residual, bladder wall thickness). ICC ≥0.75 was considered excellent. Bland-Altman plots were also used to assess the interobserver agreement. Sixty children were recruited (7.3 ± 1.1 years). The interobserver ICCs for bladder volume and voiding residual volume were 0.91 (confidence interval 0.85–0.95) for both. The interobserver ICCs for the bladder wall thickness was satisfactory 0.43, with a minimum detectable difference of 2 mm. The observed values for intra-observer analysis showed an excellent (ICC ≥ 0.90) agreement between the three measurements performed by each one of the sonographers. Bland-Altman plots showed that the bias (mean difference) was −0.35 and the limits of agreement were −3.43 and 2.73 for the bladder wall thickness measurements (Figure). The interobserver reliability for both the postvoid residual volume and full bladder volume were excellent even in operators with different levels of training, pointing out the role of bladder ultrasound in the routine clinical practice. Weaker agreement was found for the bladder wall thickness measurement. The intra-observer agreement was excellent for all the measurements. These results showed a good reliability of urinary bladder ultrasound in children aged 7.3 ± 1.1 years as far as bladder volume measurement is concerned. Given the variability of bladder wall thickness, a standardized methodology is desirable to increase its reliability. [ABSTRACT FROM AUTHOR]

Summary Introduction Since standardization of the ICCS terminology, only two small case series of children with extraordinary daytime only urinary frequency (EDOUF) have been published. The aims of the present study were i) to describe a large cohort of children affected by EDOUF, to evaluate its rate among the main micturition pediatric disturbances, and to determine if there is different EDOUF onset among seasons; ii) to investigate possible associations with urodynamic abnormalities by non-invasive techniques; iii) to evaluate whether postponing micturition exercise (PME) can objectively verify the anamnestic data hinting at the EDOUF diagnosis; and iv) to determine the effect of postponing micturition at home. Material and methods We reviewed the records of all patients with EDOUF, nocturnal enuresis, and/or overactive bladder firstly examined from March 2012 to February 2016. We evaluated post-void residual and bladder wall thickness by urinary ultrasound and uroflowmetry and recorded the season in which the EDOUF started. Through the PME, the EDOUF diagnosis was confirmed if patients were able to postpone micturition reaching at least 80% of the expected bladder capacity without showing urinary incontinence. At home, we recommended postponing micturition for a maximum of 3 h if EDOUF affected the normal daily activities of both children and parents. We set a telephone interview for 3 months later. Results The clinical characteristics of the EDOUF population are shown in the Table. The EDOUF rate was 12.1%. The rate of EDOUF onset was significantly lower during the summer than in other seasons ( p = 0.02) and the OR for onset of EDOUF in the summer – compared with the other seasons – was 0.37 (95% CI 0.18–0.74; p = 0.005). Eighty-five (80.2%) patients reported an intermittent trend of the EDOUF with variable periods of improvement and worsening. All the EDOUF patients had normal uroflowmetry, 1/106 had post-micturition bladder wall thickness >5 mm and one post-void residual. At the PME, 106 out of 106 (100%) patients with EDOUF were able to reach at least 80% of the EBC without showing urinary incontinence or urgency incontinence. After 3 months, in 98.1% of the patients the symptoms had disappeared or improved. Discussion and conclusions Childhood EDOUF is rather common and is generally associated with normal non-invasive urodynamic patterns. The PME allows verification of anamnestic data of EDOUF. The sole recommendation to postpone micturition for a maximum of 3 h or until the micturition postponement became stressful could be considered as a possible approach. Table Clinical characteristics of the 106 patients with EDOUF. Age at EDOUF diagnosis, yr 6.8 ± 2.3 Female sex, no. (%) 53 (50) SBP, SDS 0.31 ± 0.7 DBP, SDS 0.17 ± 0.8 Age at continence, yr 2.3 ± 0.5 Nocturia, no. (%) 1 (0.9) Constipation, no. (%) 19 (17.4) Urgency, no. (%) 0 (0) Incontinence, no. (%) 0 (0) EBC >80% at PME, no. (%) 106 (100) Post-micturition bladder wall thickness >5 mm, no. (%) 1 (0.9) Presence of post-void residual, no. (%) 1 (0.9) a Maximum flow, mL/s 19.34 ± 10.2 Normal uroflowmetry, no. (%) 106 (100) Normal urinalysis, no. (%) 106 (100) Previous UTIs, % 2 (1.9) b Daily micturitions, no. 20.1 ± 7.2 Mean daily voided volumes (% of the EBC) 28.7 ± 9.2 Data are given as means ± SDS, unless stated otherwise. DBP, diastolic blood pressure; EBC, expected bladder capacity; EDOUF, extraordinary daytime only urinary frequency; PME, postponing micturition exercise; SBP, systolic blood pressure; SDS, standard deviation score; UTI, urinary tract infection. a Only one patient presented a post-void residual of 30 mL. b All the UTIs were not febrile. [ABSTRACT FROM AUTHOR]