Novel minimally invasive abdominoplasty for selected cases of prune belly syndrome: Step-by-step technique description and clinical indications.

BACKGROUND: Prune belly syndrome (PBS) is characterized by the triad of abdominal flaccidity, bilateral undescended testicles and genitourinary tract anomalies. A variable spectrum of abdominal wall laxity is observed in PBS. We present the first case of a novel technique using a minimally invasive abdominoplasty to specifically address patients with localized abdominal wall weakness in PBS. CASE PRESENTATION: A two-years-old child with PBS presented with recurrent febrile urinary tract infections. Ultrasonography demonstrated a dysplastic right kidney associated with significant ipsilateral ureterohydronephrosis. Voiding urethrocystogram did not show vesicoureteral reflux and DMSA scan depicted a non-functioning right kidney. During laparoscopic right nephroureterectomy and first stage Fowler-Stephens bilateral orchiopexies, a significant right-sided lateral abdominal wall bulging was observed. A minimally invasive laparoscopic abdominoplasty was performed with a one-way running suture using an unabsorbable 2.0 prolene approximating the edges of the musculofascial defect. While undergoing the second-stage Fowler-Stephens orchiopexy, no bulging was observed. CONCLUSION: A minimally invasive abdominoplasty to improve abdominal wall lateral bulging in PBS was feasible and presented good cosmetic result. We anticipate that this technique can be applied for children with PBS with primary lateral abdominal wall bulging, employing one or more suture lines depending on the fascial defect size.

Bilateral external iliac artery pseudoaneurysms causing urinary obstruction and acute renal failure.

BACKGROUND: We report an exceedingly rare case of bilateral external iliac artery pseudoaneurysms causing urinary obstruction and acute renal failure. CASE PRESENTATION: A young man presented with acute severe bilateral testicular pain radiating to the back. Clinical and radiological workup showed bilateral external iliac artery pseudoaneurysms, which caused bilateral ureterohydronephrosis due to urinary obstruction with subsequent renal failure. Management included immediate bilateral external iliac artery endovascular repair and bilateral ureterolysis using a retroperitoneal approach, with resolution of the obstruction and successful endovascular treatment of both pseudoaneurysms. The only identifiable risk factor for cardiovascular disease was cocaine addiction. CONCLUSIONS: This case highlights an unusual and severe clinical presentation of bilateral EIA pseudoaneurysms causing bilateral ureterohydronephrosis and subsequent renal failure. Awareness of this condition may help avoid misdiagnosis and delayed management, which is of utmost importance for a favorable outcome.

Evaluating TIMP-2 and IGFBP-7 as a predictive tool for kidney injury in ureteropelvic junction obstruction

ABSTRACT A major challenge in the management of ureteropelvic junction obstruction (UPJO) is the selection of patients who would benefit from surgical treatment. Tissue inhibitor metalloproteinase-2 (TIMP-2) and insulin-like growth factor-binding protein 7 (IGFBP7) indicate renal cell stress and are associated with cell cycle arrest. The [TIMP-2] [IGFBP7] ratio (Nephrocheck®) has been recently applied in patients in intensive care units patients to predict the development of acute kidney injury. In this study, we evaluated the performance of these biomarkers performance to distinguishing obstructive hydronephrosis (HN) from non-obstructive HN. Materials and Methods: Consecutive patients with UPJO were enrolled in this study. Urinary [TIMP-2] [IGFBP7] and clinical characteristics (hydronephrosis grade, differential renal function, and drainage half-time) were measured in the following groups: 26 children with obstructive HN at initial diagnosis (group 1A) and after six months of dismembered pyeloplasty (group 1B); 22 children with non-obstructive HN (group 2), and 26 children without any urinary tract condition, as the control group (group 3). Results: Comparing the initial samples, [TIMP-2] [IGFBP7] had higher levels in the HN groups and lower levels in the control group; however, no difference was observed between the HN groups (obstructive vs. non-obstructive). After six months of follow-up, patients who underwent dismembered pyeloplasty showed stability in the urinary concentration of [TIMP-2] [IGFBP7]. All patients with [TIMP-2] [IGFBP7] higher than 1.0 (ng/mL)2/1000 had diffuse cortical atrophy on ultrasonography. Conclusions: We showed that urinary levels of urinary [TIMP-2] [IGFBP7] are higher in children with HN than controls. Nephrocheck® is not reliable in predicting the need for surgical intervention for pediatric patients with UPJO.

Evaluating TIMP-2 and IGFBP-7 as a predictive tool for kidney injury in ureteropelvic junction obstruction.

A major challenge in the management of ureteropelvic junction obstruction (UPJO) is the selection of patients who would benefit from surgical treatment. Tissue inhibitor metalloproteinase-2 (TIMP-2) and insulin-like growth factor-binding protein 7 (IGFBP7) indicate renal cell stress and are associated with cell cycle arrest. The [TIMP-2] [IGFBP7] ratio (Nephrocheck®) has been recently applied in patients in intensive care units patients to predict the development of acute kidney injury. In this study, we evaluated the performance of these biomarkers performance to distinguishing obstructive hydronephrosis (HN) from non-obstructive HN. MATERIALS AND METHODS: Consecutive patients with UPJO were enrolled in this study. Urinary [TIMP-2] [IGFBP7] and clinical characteristics (hydronephrosis grade, differential renal function, and drainage half-time) were measured in the following groups: 26 children with obstructive HN at initial diagnosis (group 1A) and after six months of dismembered pyeloplasty (group 1B); 22 children with non-obstructive HN (group 2), and 26 children without any urinary tract condition, as the control group (group 3). RESULTS: Comparing the initial samples, [TIMP-2] [IGFBP7] had higher levels in the HN groups and lower levels in the control group; however, no difference was observed between the HN groups (obstructive vs. non-obstructive). After six months of follow-up, patients who underwent dismembered pyeloplasty showed stability in the urinary concentration of [TIMP-2] [IGFBP7]. All patients with [TIMP-2] [IGFBP7] higher than 1.0 (ng/mL)2/1000 had diffuse cortical atrophy on ultrasonography. CONCLUSIONS: We showed that urinary levels of urinary [TIMP-2] [IGFBP7] are higher in children with HN than controls. Nephrocheck® is not reliable in predicting the need for surgical intervention for pediatric patients with UPJO.

Use of contrast-enhanced ultrasound in pediatrics.

Although contrast-enhanced ultrasound has been shown to provide considerable benefits, particularly in pediatric patients, it is still used relatively rarely in Brazil. It has proven to be a safe technique, and adverse effects are rare. In this review, we address the technique and main applications of contrast-enhanced ultrasound in the pediatric population, including the evaluation of focal liver lesions, abdominal trauma, kidney grafts, liver grafts, bowel loops, and vesicoureteral reflux. It is important for pediatric radiologists to be acquainted with this promising tool, understanding its applications and limitations.

A ultrassonografia com contraste de microbolhas é um método ainda pouco utilizado no Brasil, mas que tem grandes vantagens, ainda mais na faixa pediátrica, como ausência de radiação e dispensa de sedação/anestesia. O contraste de microbolhas tem se mostrado bastante seguro e com raríssimas reações adversas graves. Apresentaremos nesta revisão a técnica para o uso desse contraste na ultrassonografia, bem como as principais aplicações na faixa pediátrica, como avaliação das lesões hepáticas focais, do trauma abdominal, do enxerto hepático e renal nos transplantes, das alças intestinais e da pesquisa de refluxo vesicoureteral. É importante que radiologistas que exerçam funções em centros pediátricos conheçam essa ferramenta promissora.

Use of contrast-enhanced ultrasound in pediatrics / Utilização da ultrassonografia com contraste de microbolhas na pediatria

Abstract Although contrast-enhanced ultrasound has been shown to provide considerable benefits, particularly in pediatric patients, it is still used relatively rarely in Brazil. It has proven to be a safe technique, and adverse effects are rare. In this review, we address the technique and main applications of contrast-enhanced ultrasound in the pediatric population, including the evaluation of focal liver lesions, abdominal trauma, kidney grafts, liver grafts, bowel loops, and vesicoureteral reflux. It is important for pediatric radiologists to be acquainted with this promising tool, understanding its applications and limitations.

Resumo A ultrassonografia com contraste de microbolhas é um método ainda pouco utilizado no Brasil, mas que tem grandes vantagens, ainda mais na faixa pediátrica, como ausência de radiação e dispensa de sedação/anestesia. O contraste de microbolhas tem se mostrado bastante seguro e com raríssimas reações adversas graves. Apresentaremos nesta revisão a técnica para o uso desse contraste na ultrassonografia, bem como as principais aplicações na faixa pediátrica, como avaliação das lesões hepáticas focais, do trauma abdominal, do enxerto hepático e renal nos transplantes, das alças intestinais e da pesquisa de refluxo vesicoureteral. É importante que radiologistas que exerçam funções em centros pediátricos conheçam essa ferramenta promissora.

Can extended upper pole ureterectomy prevent ureteral stump syndrome after proximal approach for duplex kidneys?

ABSTRACT Introduction: Symptomatic duplex kidneys usually present with recurrent urinary tract infection due to ureteral obstruction (megaureter, ureterocele or ectopic ureter) and/or vesicoureteral reflux. Upper-pole nephrectomy is a widely accepted procedure to correct symptomatic duplex systems with poor functioning moieties, also known as upper or proximal approach. The distal ureteral stump syndrome (DUSS) can be a late complication of this approach. There is no consensus upon the length of ureteral dissection and the better approach to symptomatic disease in duplex systems, so we aim to identify if extended ureteral dissection can prevent DUSS in top-down approach. Materials and Methods: Forty-four consecutive patients with symptomatic duplex system were retrospectively classified into two groups: those with limited ureteral excision after heminephrectomy (HN) (group-1) and those with extended ureterectomy after HN (group-2). Patients were followed-up for at least 36 months regarding outcomes of distal ureteral stump. Results: Overall complication was 20%. A total of 8 patients required unplanned further surgery in Group-1 (30%) whereas only 1 patient required unplanned surgery in group 2 (6%) (p=0.07). Subgroup analysis showed that Group-1 presented more DUSS requiring surgery during follow-up than group-2 (p=0.04). Factors possibly affecting complications incidence (such as ureterocele or ectopic ureter) did not differ between groups (p=0.72 and p=0.78). Conclusion: Upper pole nephrectomy should be performed with extended distal ureteral dissection to prevent ureteral stump complications.

Patients with encrusted ureteral stents can be treated by a single session combined endourological approach

ABSTRACT Purpose: To describe our experience in the management of retained encrusted ureteral stents using a single session combined endourological approach. Materials and Methods: Patients with retained encrusted ureteral stents who had been submitted to a single session combined endourological approach from June 2010 to June 2018 were prospectively evaluated. Patients were divided according to the Forgotten-Encrusted-Calcified (FECal) classification. The stone burden, surgical intervention, number of interventions until stone free status, operation time, hospital stay, complications, stone analysis, and stone-free rate were compared between groups. ANOVA was used to compare numerical variables, and the Mann-Whitney or Chi-square test to compare categorical variables between groups. Results: We evaluated 50 patients with a mean follow-up of 2.9±1.4 years (mean±SD). The groups were comparable in terms of age, sex, laterality, BMI, comorbidities, ASA, reason for stent passage, and indwelling time. The stone burden was higher for grades IV and V (p=0.027). Percutaneous nephrolithotomy was the most common procedure (p=0.004) for grades IV and V. The number of procedures until the patients were stone-free was 1.92±1.40, and the hospital stay (4.2±2.5 days), complications (22%), and stone analysis (66% calcium oxalate) were similar between groups. The stone-free rate was lower in grades III to V (60%, 54.5%, and 50%). Conclusions: The endoscopic combined approach in the supine position is a safe and feasible technique that allows removal of retained and encrusted stents in a single procedure. The FECal classification seems to be useful for surgical planning.

Can extended upper pole ureterectomy prevent ureteral stump syndrome after proximal approach for duplex kidneys?

INTRODUCTION: Symptomatic duplex kidneys usually present with recurrent urinary tract infection due to ureteral obstruction (megaureter, ureterocele or ectopic ureter) and/or vesicoureteral reflux. Upper-pole nephrectomy is a widely accepted procedure to correct symptomatic duplex systems with poor functioning moieties, also known as upper or proximal approach. The distal ureteral stump syndrome (DUSS) can be a late complication of this approach. There is no consensus upon the length of ureteral dissection and the better approach to symptomatic disease in duplex systems, so we aim to identify if extended ureteral dissection can prevent DUSS in top-down approach. MATERIALS AND METHODS: Forty-four consecutive patients with symptomatic duplex system were retrospectively classified into two groups: those with limited ureteral excision after heminephrectomy (HN) (group-1) and those with extended ureterectomy after HN (group-2). Patients were followed-up for at least 36 months regarding outcomes of distal ureteral stump. RESULTS: Overall complication was 20%. A total of 8 patients required unplanned further surgery in Group-1 (30%) whereas only 1 patient required unplanned surgery in group 2 (6%) (p=0.07). Subgroup analysis showed that Group-1 presented more DUSS requiring surgery during follow-up than group-2 (p=0.04). Factors possibly affecting complications incidence (such as ureterocele or ectopic ureter) did not differ between groups (p=0.72 and p=0.78). CONCLUSION: Upper pole nephrectomy should be performed with extended distal ureteral dissection to prevent ureteral stump complications.

Urinary extracellular matrix proteins as predictors of the severity of ureteropelvic junction obstruction in children.

INTRODUCTION: Ureteropelvic junction obstruction (UPJO) particularly represents a challenge in regard to management, as not all hydronephrosis (HN) represent a kidney-damaging state. Urinary biomarkers have been proposed as noninvasive tools. Extracellular matrix (ECM) proteins are associated with tissue fibrosis in the setting of UPJO and is poorly explored. OBJECTIVE: To investigate whether urinary ECM proteins are useful to discriminate the severity of urinary obstruction on unilateral UPJO. STUDY DESIGN: Children with unilateral UPJO were prospective enrolled. Urinary (u) matrix metalloproteinases (MMP-1,-2,-9 and TIMP-1,-2) as well as clinical characteristics were measured in the following groups: 26 children with obstructive HN at initial diagnosis and after six months of dismembered pyeloplasty; 22 children with non-obstructive HN at diagnosis and after six months of observation; 26 children without any urinary tract condition, as the control group. Results were assessed statistically using for homogenous groups, a one-way analysis of variances (ANOVA) and for nonparametric groups, Mann-Whitney test or Kruskal-Wallis test was performed. ROC curves were performed. RESULTS: Baseline samples demonstrated a higher concentration of uMMP-1/Cr, uMMP-2/Cr, u-TIMP-1/Cr and u-TIMP-2/Cr in obstructive HN group; uMMP-9/Cr levels were higher in non-obstructive HN group and all studied biomarkers had lower concentrations for the control group. On follow-up, for the obstructive HN group, urinary concentration of uTIMP-1/Cr and uTIMP-2/Cr decreased, and uMMP-1/Cr, uMMP-2/Cr and uMMP-9/Cr increased when comparing preoperative to postoperative values. In the non-obstructive HN group, all proteins analyzed were stable after six months of observation. ROC curves analysis showed a promising diagnostic profile for the detection of obstructive HN for uTIMP-1/Cr (area under the curve -AUC-; of 0.692), uTIMP-2/Cr (AUC of 0.678) and for uMMP-2/Cr (AUC of 0.655). DISCUSSION: The severity of kidney obstruction could correlate with the urinary ECM proteins concentration in this study. This is concordant with prior studies demonstrating that a disruption of the balance of accumulation/degradation of the ECM proteins occur on obstructive uropathy. Limitations of our study include the older age of our patients and that these markers had no influence at all on the surgical decision. CONCLUSION: We demonstrate that obstructive HN have significantly higher uMMP-2, uTIMP-1 and uTIMP-2 concentrations. Particularly, uTIMP-2 levels were correlated to severity of obstruction and therefore, it might be a useful urinary biomarker to correctly allocate children with HN between surgical management vs follow-up. After pyeloplasty, uTIMP-1 and uTIMP-2 presented a progressive decrease postoperatively, which is also highly desirable for urinary markers.

Bladder and Bowel Dysfunction Network: Improving the Management of Pediatric Bladder and Bowel Dysfunction.

Lower urinary tract symptoms with constipation characterize bladder and bowel dysfunction (BBD). Due to high referral volumes to hospital pediatric urology clinics and time-consuming appointments, wait times are prolonged. Initial management consists of behavioral modification strategies that could be accomplished by community pediatricians. We aimed to create a network of community pediatricians trained in BBD (BBDN) management and assess its impact on care. METHODS: We distributed a survey to pediatricians, and those interested attended training consisting of lectures and clinical shadowing. Patients referred to a hospital pediatric urology clinic were triaged to the BBDN and completed the dysfunctional voiding symptom score and satisfaction surveys at baseline and follow-up. The Bristol stool chart was used to assess constipation. Results were compared between BBDN and hospital clinic patients. RESULTS: Surveyed pediatricians (n = 100) most commonly managed BBD with PEG3350 and dietary changes and were less likely to recommend bladder retraining strategies. Baseline characteristics were similar in BBDN (n = 100) and hospital clinic patients (n = 23). Both groups had similar improvements in dysfunctional voiding symptom score from baseline to follow-up (10.1 ± 4.2 to 5.6 ± 3.3, P = 0.01, versus 10.1 ± 4.2 to 7.8 ± 4.5, P = 0.02). BBDN patients waited less time for their follow-up visit with 56 (28-70) days versus 94.5 (85-109) days for hospital clinic patients (P < 0.001). Both groups demonstrated high familial satisfaction. CONCLUSIONS: Community pediatricians may require more knowledge of management strategies for BBD. Our pilot study demonstrates that implementing a BBDN is feasible, results in shorter wait times, and similar improvement in symptoms and patient satisfaction than a hospital pediatric urology clinic.

Laparoscopic hidden incision endoscopic surgery (hides) nephrectomy VS. Traditional laparoscopic nephrectomy: Non-inferior surgical outcomes and better cosmetic results.

INTRODUCTION: The benefits of laparoscopic total nephrectomy in pediatric patients are well established. Traditional Ports placement (TPP) still follows Clayman's classic description: except for the umbilical scar, the other laparoscopic scars are exposed in the abdomen. Advances in robotic surgery permitted the development of HidES (hidden incision endoscopic surgery) technique, to obtain a better final cosmetic aspect with the scars located intraumbilically and in the hypogastric region, in an area easily hidden by underwear. As robotic surgery is related to higher costs and lacks availability, a pure laparoscopic HidES technique was developed. OBJECTIVES: 1.Evaluate safety and efficacy of pure laparoscopic HidES.2. Compare HidES with TPP nephrectomy series to assess non-inferiority and cosmetic outcomes. STUDY DESIGN: Twenty-one pediatric patients with symptomatic poor functioning kidneys (DMSA<10%) underwent HidES nephrectomy. Their intra and post-operative outcomes were recorded prospectively. HidES group was compared to an equivalent group of thirty-two patients who underwent TPP nephrectomy. RESULTS: There were no conversions to open surgery in the TPP or HidES groups. There was a significant difference in operative time between HidES (53.4min) and TPP (109.4 min), with p = 0.004 and the mean bleeding volume was 65.5 ml. There was no significant difference in bleeding between HidES (71 ml) and TPP (120 ml) (p = 0.06), no intraoperative complications and no complications above Clavien-Dindo II during the 6-week follow-up. Satisfaction reached 100% in HidES group, whereas in TPP satisfaction was 63% (p = 0.004). DISCUSSION: HidES benefit over TPP is to conceal visible scars above underwear, improving cosmetical outcome. A prospective HidES group was compared to a retrospective TPP database due to decreasing number of nephrectomies being performed, which is a limitation of our study. HidES surgeries were performed by pediatric urologists (associate staff) while TPP group surgeries were performed by PGY-5 urological residents assisted by the associate staff, which is a clear limitation and can explain shorter operative times for HidES. During HidES surgeries an improved visualization of the operative field by the inferior trocar positioning was noted, which helps the posterior dissection of renal pedicle. Previous published study comparing robotic TPP and HidES pyeloplasty showed equal results for both groups with cosmetic advantages. Objective satisfaction was assessed with statistically advantage in favor of HidES procedure, consonant with other evidence that supports the benefit of scar location in patient and parental satisfaction. CONCLUSION: HidES nephrectomy proved to be safe, feasible and not inferior to the traditional nephrectomy in experienced hands, with better cosmesis.

Patients with encrusted ureteral stents can be treated by a single session combined endourological approach.

PURPOSE: To describe our experience in the management of retained encrusted ureteral stents using a single session combined endourological approach. MATERIALS AND METHODS: Patients with retained encrusted ureteral stents who had been submitted to a single session combined endourological approach from June 2010 to June 2018 were prospectively evaluated. Patients were divided according to the Forgotten-Encrusted-Calcified (FECal) classification. The stone burden, surgical intervention, number of interventions until stone free status, operation time, hospital stay, complications, stone analysis, and stone-free rate were compared between groups. ANOVA was used to compare numerical variables, and the Mann-Whitney or Chi-square test to compare categorical variables between groups. RESULTS: We evaluated 50 patients with a mean follow-up of 2.9±1.4 years (mean±SD). The groups were comparable in terms of age, sex, laterality, BMI, comorbidities, ASA, reason for stent passage, and indwelling time. The stone burden was higher for grades IV and V (p=0.027). Percutaneous nephrolithotomy was the most common procedure (p=0.004) for grades IV and V. The number of procedures until the patients were stone-free was 1.92±1.40, and the hospital stay (4.2±2.5 days), complications (22%), and stone analysis (66% calcium oxalate) were similar between groups. The stone-free rate was lower in grades III to V (60%, 54.5%, and 50%). CONCLUSIONS: The endoscopic combined approach in the supine position is a safe and feasible technique that allows removal of retained and encrusted stents in a single procedure. The FECal classification seems to be useful for surgical planning.

Management of functioning pediatric adrenal tumors.

AIM: The aim of this study is to present our experience in the management of hormonally active adrenal tumors in children. MATERIAL AND METHODS: We did a retrospective chart review of all children with hormonally active adrenal tumors evaluated at the endocrinology clinic and operated at our institution between 1983 and 2019. RESULTS: There were 75 patients included in the study, 58 with adrenocortical tumors (ACTs) and 17 with pheochromocytomas (PCCs). Within the group of patients with ACTs, there were 41 females and 17 males. The mean age was 58.3 (SD: 87.9; range: 9-211) months. The clinical manifestation of the tumor's hormonal activity was virilization in 37 cases, Cushing syndrome in 5, and mixed in 16. A positive family history was present in 11 patients (18.9%). The mean tumor size was 48.2 (SD: 22.4; range: 7-120) mm. The pathological diagnosis was adenoma in 42 cases, carcinoma in 15 cases, and macronodular hyperplasia in 1. Median follow-up was 192 (range: 50-290) months. Tumor recurrence occurred in 6 patients (10.3%), and there were three disease-related deaths (5%). Within the group of patients with PCCs, there were 11 males and 6 females. The mean age was 146.7 (SD: 71.2; range: 60-216) months. A positive family history was present in 7 patients (41.2%). The mean tumor size was 36.6 (SD: 16.7; range: 7-120) mm. The pheochromocytoma was classified as benign in 15 cases and as malignant in 2. During a median follow-up of 180 (range: 127-300) months, recurrence was observed in 6 cases (35.3%) and disease-related death in 1 case (5.9%). CONCLUSIONS: Proper diagnosis and management at our referral center were associated with a high cure rate, even in cases of malignant tumors. Familial surveillance is highly recommended. LEVEL OF EVIDENCE: Level IV.

Prune perineum surgical correction - Treatment of a rare syndrome.

INTRODUCTION: Prune Perineum Syndrome (PPS) is rare, with only three cases described in the literature. Treatment requires abdominal and pelvic surgeries. Our goal is to provide a video with steps of the surgical corrections of this syndrome. MATERIALS AND METHODS: We present a case of an 8 months-old boy with PPS. At our first evaluation, he had already been submitted to a colostomy with mucous fistula and a vesicostomy. RESULTS: By the time of this publication, he had been submitted to six surgical procedures. First, osteotomy, followed by colostomy, cystoscopic evaluation, cystoplasty, perineal and abdominal correction with a dual mesh, bilateral orchidopexy, treatment of the vesicocolonic fistula, sigmoidectomy and appendicectomy. He is 6 years old, walks with no assistance, has satisfactory abdominal tonus which allows him to void with Valsalva Maneuver. Clean intermittent catheterization is also performed. He had no UTI since his first surgery. Although submitted to several procedures, treatment is not yet concluded. A future urethral dilation together with a genitoplasty might improve his quality of life. CONCLUSION: PPS is a very rare condition, with scarce literature concerning its treatment. Multiple procedures might be necessary to correct malformations and improve patient's quality of life.

Gonadal Function and Reproductive System Anatomy in Postpubertal Prune-Belly Syndrome Patients.

OBJECTIVE: To gain insight into the causes of infertility in Prune Belly Syndrome (PBS) by evaluating reproductive system anatomy and gonadal function in a cohort of postpubertal PBS patients. METHODS: We contacted all PBS patients 14 years old or older treated and followed at our institution. Age at orchiopexy, type of orchiopexy (with or without ligation of gonadal vessels), testicular volumes and positions were evaluated. Pelvic magnetic resonance imaging (to assess prostate size, seminal vesicles, and vas) and hormonal profile were ordered. Sperm analysis and analysis of urine after masturbation were performed after informed consent. RESULTS: Fifteen patients were included in this study. Mean age was 19.2 years. Mean age at orchiopexy was 18 months. Fourteen patients (93.3%) had normal and orthotopic testes. Mean testicular volume was 6.9 cc. Eight patients collected semen, 5 of them (62.5%) had spermatozoa in the specimen and motile sperm was found in 4 (50%). Mean hormone levels were LH: 5.3 mg/dL, FSH: 6.9 mg/dL, testosterone 531 mg/dL. Magnetic resonance imaging revealed hypoplastic prostates in 66.6% and unilateral seminal vesicle absence in 66.6%. No vasal abnormality was noted. CONCLUSION: Patients with PBS may have normal sexual hormonal levels. Motile spermatozoa were found in half of the patients. Our study highlights a high prevalence of prostate and seminal vesicle abnormalities that may represent an important cause for their infertility.

Recent advances in pediatric bladder malignancies.

Urothelial pediatric neoplasms are relatively rare. Papillary urothelial neoplasms of low malignant potential (PUNLMPs) and rhabdomyosarcoma (RMS) are the most common bladder malignancies in the pediatric population. Clinical presentation encompasses macroscopic hematuria or lower urinary tract symptoms (or both) or is detected incidentally at imaging. Tumors arising from the bladder can originate from any of its four histological layers (urothelium, lamina propria, detrusor, and adventitia) and are divided into tumors that have an epithelial origin (arising from the urothelium) and those that have a non-epithelial origin (mesenchymal neoplasms). RMS is the most common malignant tumor of the urinary bladder in children younger than 10 years. Deriving from the embryonic mesenchymal cell, the histopathologic subtypes of RMS are embryonal RMS (>90%) and alveolar histology (<10%). Pre-treatment imaging should be carried out by computed tomography (CT) or at present is more likely with magnetic resonance imaging of the pelvis. Chest CT and bone scintigraphy are used to screen for metastases. In selected cases, a positron emission tomography scan may be recommended to evaluate suspicious lesions. The current prognostic classification considers age, histologic subtype, tumor site, size, and extent (nodal or distant metastases). Staging is based on pre-operative findings, group is based on intra-operative findings and pathology, and risk stratification is derived from both stage and group data. Pre-operative chemotherapy is the most common first-line intervention for bladder/prostate RMS, before surgery or radiation therapy. Collaborative groups such as the Soft Tissue Sarcoma Committee of the Children's Oncology Group and the European Pediatric Soft Tissue Sarcoma Study Group endorse this therapy. PUNLMPs are generally solitary, small (1-2 cm), non-invasive lesions that do not metastasize. Therapy is usually limited to a transurethral resection of the bladder tumor. About 35% are recurrent and around 10% of them increase in size if they are not treated.

Influence of low free thyroxine on progression of chronic kidney disease.

BACKGROUND: Hypothyroidism is highly prevalent in patients with chronic kidney disease (CKD) and has been associated with poorer clinical outcomes, including faster decline of kidney function. However, there is no consensus whether low free thyroxin (LFT) affects the rate of estimated glomerular filtration rate (eGFR) decline and how the presence of proteinuria influences the progression of renal dysfunction in hypothyroidism. METHODS: We assessed thyroid status, proteinuria, and progression of eGFR by Modification of Diet in Renal Disease equation and CKD-EPI equation in a cohort of CKD patients followed in general nephrology clinics. We estimated the association of LFT levels, and the degree of proteinuria on progression of eGFR. We adjusted for other covariables: age, gender, body mass index, diabetes, hypertension, HbA1c, uric acid, cholesterol, and triglycerides levels.. RESULTS: One thousand six hundred ten patients (64 ± 15 years, 46.8% men, 25.3% diabetic) were included. At beggnining of follow up eGFR was between 45 and 60, 30-45 and 15-30 ml/min/1.73m2 in 479 (29.8%), 551(34.2%), and 580(36.0%) patients, respectively. LFT levels were available at initial evaluation in 288(17.9%) patients and 735(48.5%) had assessment of proteinuria (19.6% with LFT vs. 15.4% without LFT, p = 0.032). Median follow-up time was of 21 months, and 1223(76%) had at least 1 year of follow up. Overall, eGFR decline per month was - 0.05(- 0.26, 0.23) ml/min/1.73m2, reaching 1.7(1.3, 2.4) ml/min/1.73m2 by the end of study period. Similar results were obtained using CKD-EPI. Multivariable mixed linear analysis showed that proteinuria and age were independently associated with eGFR decline, with no effect of LFT, and no interaction between proteinuria and LFT. In patients without proteinuria, there was an improvement of eGFR despite the presence of LFT. CONCLUSIONS: We confirmed a faster rate of eGFR declined in patients with proteinuria. However, despite the pathophysiological rational that hypothyroidism can lead to increased rate of CKD progression, we failed to demonstrate an association between LFT and rate of CKD progression. We conclude that the benefit of hypothyroidism treatment in CKD patients needs to be evaluate in prospective studies.