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Purpose: Solitary functioning kidney (SFK) may be associated to hypertrophy, hypertension and chronic kidney disease. We evaluated blood pressure (BP) of children with congenital SFK comparing agenesis to multicystic dysplastic kidney (MCDK) and correlated BP profiles with renal dimensions of affected and contralateral kidney.Methods: We compared 40 patients with MCDK, grouped for either treatment options (A: conservative vs B: nephrectomy) or involution time (A1: before 4 years-of-age vs A2: persistence-of-MCDK), to 10 unilateral agenesis (C). Patients were evaluated with ultrasound, scintigraphy, office-ambulatory BP monitoring.Results: Compensatory hypertrophy was demonstrated in most of the subjects, without differences between subgroups, with an increase over time (p < 0.001). A1-C showed an overall percentage of hypertrophy significantly higher than A2-B (83%-88% vs 70%-73%, respectively; p = 0.03); moreover, cumulative risk to develop hypertension in A1-C is significantly higher compared to A2-B in office and ambulatory BP monitoring (p = 0.03). Insufficient dipping in systolic and/or diastolic BP was found in 82% children, without differences between subtypes.Conclusions: Patients with a small/absent dysplastic kidney have an increased risk to develop hypertrophy and hypertension compared to patients with a large residual, regardless of nephrectomy. ABPM revealed absent dipping in most patients with SFK, warning further investigations in apparently not symptomatic patients. [ABSTRACT FROM AUTHOR]

Background: Abnormalities involving the skin coverage of the penis are difficult to define, but they can significantly alter penile appearance, and be a cause of parental concern. Data sources: The present review was based on a nonsystematic search of the English language medical literature using a combination of key words including "penile skin anomalies" and the specific names of the different conditions. Results: Conditions were addressed in the following order, those mainly affecting the prepuce (phimosis, balanitis xerotica obliterans, balanitis, paraphimosis), those which alter penile configuration (inconspicuous penis and penile torsion), and lastly focal lesions (cysts, nevi and vascular lesions). Most of these anomalies are congenital, have no or minimal influence on urinary function, and can be detected on clinical examination. Spontaneous improvement is possible. In the majority of cases undergoing surgery, the potential psychological implications of genital malformation on patient development are the main reason for treatment, and the age generally recommended for surgery is after 12 months of age. Conclusion: This review provides the pediatrician with a handy tool to identify the most common penile skin anomalies, counsel parents adequately, make sensible and evidence based choices for management, and recognize complications or untoward outcomes in patients undergoing surgery. [ABSTRACT FROM AUTHOR]

Purpose: The incidence of GER, related symptoms and complications in patients treated for congenital diaphragmatic hernia (CDH) are poorly defined. The aim was to evaluate incidence and development of GER in children treated for CDH in a short- and long-term follow-up period, identifying potential risk factors of morbidity. Methods: Thirty-six patients were evaluated with pH-MII at a median age of 6 months (T1) and re-evaluated with pH-MII and endoscopy at a median age of 5 years (T2). Results: The incidence of reflux was 83 % in T1 and 61 % in T2; the incidence of symptoms was 62 % in T1 and 38 % in T2. In both groups the reflux was mainly non-acidic. Patch, intrathoracic stomach and esophageal dysmotility were risk factors for GER. Conclusions: The incidence of GER and symptoms decrease over the time but it was higher than in the literature, probably because it is mainly non-acidic and evaluable only with MII. The esophageal dysmotility was found to be the main risk factor. An high incidence of reflux and esophagitis was found also in asymptomatic patients, and so a close follow-up is recommended in all patients even if it is asymptomatic. [ABSTRACT FROM AUTHOR]

Purpose: The aim of study was to evaluate if pelvic ultrasound can be useful in managing children with chronic idiopathic constipation.Methods: A total of 270 children with idiopathic chronic constipation were enrolled in the study. At baseline and at monthly checkups children were evaluated by clinical score and pelvic ultrasound (US). Patients have been divided in 2 groups, based on pelvic US results: group A with a rectal diameter >3 cm, group B with a rectal diameter <3 cm or rectum not visualized. Both groups were subsequently randomly divided in two subgroups (A1, A2, B1, B2) on the basis of the prescribed treatment (disimpaction for the first week and daily laxative or only daily laxative).Results: After 1 month of therapy all clinical features improved in group A1 and at pelvic US, rectal size reduced and became not visualized; group A2 showed poor clinical response and transverse diameter of rectum did not modify significantly; B1 and B2 groups showed significant improvement only after 2-3 months.Conclusions: Rectal disimpaction is necessary only in presence of MR. Pelvic US is a useful to diagnose MR and to set up the most appropriate treatment protocol for different chronic constipation cases. [ABSTRACT FROM AUTHOR]

Purpose: To assess the influence of the method for stent placement, the duration of stenting, and the presence of bladder drainage on the complication rate of open pyeloplasty.Patients and Methods: Complications were, retrospectively, compared in 228 consecutive open pyeloplasties performed at institution A using a trans-pyelostomic 6-Fr splint/stent for 5 days and no bladder drainage, and 150 consecutive open pyeloplasties performed at institution B using a trans-nephrostomic 6-Fr splint/stent for 9 days plus bladder drainage.Results: Median age at surgery was comparable between groups. The overall complication rate was 13% and was comparable at the two institutions, but for the presence of perioperative bleeding that was more common when the stent was placed trans-nephrostomically (institution B) and the rate of stent dislodgements, which was lower at institution A, perhaps due to some technical details aiming to prevent any inadvertent traction on the stent. Additional procedures, such as double J internal stent insertion, were required in <2% of cases.Conclusions: A 5-day period of stenting after open pyeloplasty is generally enough. Trans-nephrostomic and trans-pyelostomic stent placement is equally effective. However, the former can be associated with a slightly higher bleeding rate. Details during stent placement are keys to avoid postoperative dislodgement and malfunctioning. Systematic bladder drainage seems unnecessary. [ABSTRACT FROM AUTHOR]

Prenatal exposure to antiepileptic drugs (AEDs) increases the risk of major congenital malformations (MCM) in the fetus. AED-related abnormalities include heart and neural tube defects, cleft palate, and urogenital abnormalities. Among the various congenital anomalies of the kidney and urinary tract (CAKUT), multicystic dysplastic kidney (MCDK) disease is one of the most severe expressions. Although prenatal ultrasound (US) examination has increased the prenatal diagnosis of MCDK, the pathogenesis is still unclear. We report on four cases of MCDK in infants of epileptic women treated with AEDs during pregnancy. From October 2003 to June 2006, we observed four infants with unilateral MCDK born to epileptic women. Three patients were considered to have typical features of multicystic dysplastic kidney, and one infant was operated because of a cystic pelvic mass in the absence of a kidney in the left flank. The macroscopic appearance of this mass showed an ectopic multicystic kidney confirmed by histological findings. All patients have been studied by US scans, voiding cystourethrogram (VCUG), and radionuclide screening isotope imaging. The prenatal exposure to AEDs increases the risk of major congenital malformations from the background risk of 1–2% to 4–9%. AEDs may determine a defect in apoptosis regulation that could lead to abnormal nephrogenesis, causing MCDK. Carbamazepine (CBZ) and phenobarbital (PHB) during pregnancy should be used at the lowest dosage compatible with maternal disease. The reduction, or even suspension, of drug dosage should be achieved from the periconceptional period to the first 8 weeks of gestation to avoid any interference with organogenesis. [ABSTRACT FROM AUTHOR]