Genitourinary Tract Abnormalities Research Articles

Alpha-Thalassemia X-Linked Intellectual Disability Syndrome in a Boy with a Strong Family History of Smith–Lemli–Opitz's Syndrome

AbstractAlpha-thalassemia X-linked intellectual disability (ATRX) syndrome is a rare disorder characterized by facial dysmorphism, genitourinary tract abnormalities, alpha-thalassemia, and cognitive impairment. Patients present in early infancy with hypotonia, microcephaly, failure to thrive, and delayed milestones. We report a boy with strong family history of the Smith–Lemli–Opitz's syndrome (SLOS) phenotype. All affected family members displayed facial features and presentation identical to those of our patient. None had hypocholesterolemia. All affected family members were boys and have died. Molecular genetic testing in our patient revealed a likely pathogenic mutation in the X-linked recessive ATRX gene. We conclude that SLOS and ATRX syndrome are uncommon disorders sharing similar manifestations. Awareness of such similarity should prompt physicians to explore the possibility of ATRX syndrome in relatives of patients with unproven SLOS.

The correlation with abnormal fetal outcome and a high level of amniotic fluid alpha-fetoprotein in mid-trimester

ObjectiveTo evaluate the correlation of high levels [>2.0 multiples of median (MoM)] of amniotic fluid alpha-fetoprotein (AFAFP) in midtrimester with abnormal fetal outcome. Materials and methodsWe retrospectively studied 6245 pregnant women with singleton pregnancy who had undergone amniocentesis between 15 and 27 weeks’ gestation at Mackay Memorial Hospital between January 2014 and June 2020. Fifty-five cases had high AFAFP levels (>2.0 MoM). We investigated the abnormal fetal outcomes. ResultsAmong the fifty-five cases with high AFAFP levels (>2.0 MoM), thirty (54.5%) had fetal chromosomal abnormalities, major structural abnormalities, and/or adverse obstetric events. Eight cases (14.5%) had chromosomal abnormalities including trisomy 21 (3 cases), trisomy 18 (3 cases), mosaic trisomy 18 (1 cases), and mosaic ring 13 (1 case). Seventeen cases (30.9%) had major structural abnormalities including abdominal wall defect (6 cases) and central nervous system (5 cases), gastrointestinal tract (3 cases), cardiovascular (2 cases), and genitourinary tract (2 cases) abnormalities. Fifteen cases (27%) had adverse obstetric events, including preterm delivery (5 cases), intrauterine fetal demise (4 cases), small for gestational age (4 cases), preeclampsia (4 cases), gestational diabetes mellitus (2 cases), gestational hypertension (1 case), preterm prelabor rupture of membrane (1 case), prolonged labor (1 case), and preterm uterine contraction (1 case). ConclusionA high AFAFP level (>2.0 MoM) in midtrimester can be associated with abnormal fetal outcome, including chromosomal abnormalities, major structural abnormalities, and adverse obstetric events. Women with a prenatal diagnosis of high AFAFP levels (>2.0 MoM) should be alerted of the possibility of abnormal fetal outcomes, and further detailed genetic studies and serial sonographic examinations are recommended.

Does prophylactic antibiotics post pediatric pyeloplasty reduce the incidence of febrile UTIs?

PurposeThe use of postoperative prophylactic antibiotics in pediatric upper urinary tract reconstruction remains controversial. In this study, we examined whether low dose antibiotics administered following pediatric pyeloplasty reduce the incidence of febrile urinary tract infections at our institution. As a secondary outcome, in those patients with infection, additional analysis was performed to better quantify which patient population benefits the most from low dose prophylactic antibiotics.MethodsInstitutional review board approval (IRB) was obtained. All methods were carried out in accordance with relevant guidelines and regulations. A retrospective study was performed in patients who underwent pyeloplasty (2011–2017) at our institution. Surgical approach (laparoscopic versus robotic assisted versus open, with or without internal JJ ureteral stent) were based on surgeon preference. Patients of 8 fellowship trained pediatric urologists were included in the study period. Patients with prior history of urologic interventions or other congenital genitourinary tract abnormalities were excluded. Demographics (age, gender, ethnicity, insurance status), prior history of culture proven urinary tract infection, surgical details (administration of perioperative antibiotics), and postoperative outcomes including; 1) re-admission 30 days post-surgery, 2) any urine cultures collected due to suspected urinary tract infection.ResultsA total of 209 patients (149 boys, 60 girls) met our inclusion criteria with 55/209 (26%) receiving postoperative prophylactic antibiotics. The average age was 6 years (range: 2 months-18 years). Indwelling ureteral stent was used in 176 (84%) patients. Eleven patients (5%) had a culture-proven urinary tract infection within 30-days postoperatively. No significant differences were seen in postoperative complications or incidence of urinary tract infection when comparing surgical approaches, +/- ureteral stent, or the use of antibiotics. Secondary analysis noted statistically significant increase in post-operative urinary tract infection in younger children (2.8 v. 6.2 years, p = 0.02), those patients who had a positive preoperative urine culture (8/11, p = 0.01) and those with public health insurance (p = 0.038).ConclusionThe incidence of postoperative urinary tract infection following pyeloplasty in our cohort was relatively low. There was a higher incidence of urinary tract infection in patients less than 3 years old. The use of antibiotics in patients post pyeloplasty did not appear to affect the incidence of post-operative urinary tract infection, however, they may have a role in children who have not yet potty trained and in patients with positive preoperative urine culture.

Sleep Disorders, Comorbidities, Actions, Lower Urinary Tract Dysfunction, and Medications (“Sleep C.A.L.M.”) in the Evaluation and Management of Nocturia: A Simple Approach to a Complex Diagnosis

(Abstracted from Neurourol Urodyn 2023;42:562–572) The number of times urine is passed during the main sleep period, or nocturia, is a condition based on a fundamentally mismatched relationship between functional bladder storage capacity and nocturnal urine production, modulated by sleep patterns. Although nocturia may be caused by genitourinary tract abnormalities, other factors such as medical diseases, medications, sleep disorders, or modifiable behavioral factors may exert consequences between sleep and urine storage/production.

Clinical characteristics and immunological status of patients with 22q11.2 deletion syndrome in Northern Thailand.

22q11.2 deletion syndrome is one of the most prevalent microdeletion syndromes in humans. The syndrome is characterized by extensive phenotypic variability. to investigate the clinical characteristics, immunological features, and intellectual status of 22q11.2 deletion syndrome patients at Chiang Mai University Hospital, Thailand. Patients who had a confirmed diagnosis of 22q11.2 deletion syndrome by fluorescent in situ hybridization (FISH) were enrolled. Data collated and evaluated included that pertaining to history, physical examination, laboratory testing including T-cell, immunoglobulin, calcium, thyroid and parathyroid levels in the blood, cardiac and urological imaging, and intellectual status. 34 patients diagnosed with 22q11.2 deletion syndrome; 18 (53%) were female. The median age of patients was 18.5 months (IQR; 1.5-35.8). Ninety-one percent of patients had characteristic facial features; 94% had a congenital heart defect with tetralogy of Fallot being the most frequent (72%); 88% had hypocalcemia, and 35% had genitourinary tract abnormalities. Recurrence of 22q11.2 deletion syndrome in the family was detected in 18% of cases. Twenty-eight patients (82%) were found to have a low number or percentage of T-cells. Five patients (15%) had low immunoglobulin levels. Intellectual disability (IQ/DQ scores < 70) were found in 20 out of 25 patients who were evaluated (80%), whereas the other five (20%) performed at a level of borderline intellectual function. Tetralogy of Fallot, hypocalcemia, immunologic defect, and cognitive impairment were common in our 22q11.2 deletion syndrome study group. We recommend that all affected patients have a multi-system evaluation by a comprehensive care team.

A PROSPECTIVE OBSERVATIONAL STUDY ON PREVALENCE AND TREATMENT OF URINARY TRACT INFECTIONS IN A TERTIARY CARE TEACHING HOSPITAL IN TELANGANA STATE

Objective: To study the Prevalence of urinary tract infections and related risk factors and to study the Management of urinary tract infections. Methods: This was a prospective observational study conducted in General Medicine Department, Osmania General Hospital (OGH), a tertiary care teaching hospital in Telangana State, where patients presenting or highly suspicious of having UTIs were included in the study. Pregnant women, Children, Patients who are not willing to participate were excluded from the study. Data was collected in predesigned Data collection forms. Results: Over a period of 6 Mo a total of 75 UTI cases were observed in General Medicine Department of OGH, which included 33 Males, and 42 Females. High prevalence of UTI were observed in Females in the middle age group i.e. 31-45 y, whereas in Males, in senior adult group i.e.,&gt;60 y. The age group 18-30 y have shown the dominance of Uncomplicated UTIs(19%) and Primary UTIs (17%) and the age group&gt;60 y have shown the dominance of Complicated UTIs (25%) and Recurrent UTIs(23%). The most common causative organism was found to be E. coli (69.3%). It was confirmed that the most common risk factor was found to be Diabetes Mellitus, followed by Hypertension and Chronic Kidney Disease. The most common antibiotic prescribed for UTI was Piperacillin+Tazobactum and for fungal UTIs, an antifungal drug i.e., Fluconazole was prescribed. Conclusion: E. coli are the major cause of UTIs among patients It is discovered that UTI is common among females. Hospitalisation, married individuals, Diabetes mellitus, genitourinary tract abnormalities, congestive cardiac failure, hypertension, prostatitis and female gender are the most important risk factors of UTIs. Appropriate measures may help to reduce UTIs due to these associated factors.

Is Empirical antibiotic treatment in UTI justified in resource constraint settings in India

UTI, one of the most common infections noted in children, is reported with incidence of 2% in boys and 8% in girls of less than 8year. Absence of specific signs of UTI in younger children and increased risk for complications make it almost mandatory to test for UTI in very young children with fever. Fairly common genitourinary tract abnormalities in young children makes it even more important to screen for UTI as it can lead to chronic kidney injury later. Emperic antibiotic treatment in suspected UTI in children is rampant in our community; equally high is the possibility of antibiotic resistance, which is not studied due to cost limitations. Hence the need for identifying the change in microbial flora causing UTI and their changes in resistance pattern at community level, making it an important tool for preventing and addressing the growth issues, complications like CKD, etc. The study aims at addressing this infelt need of the community. Analysis of culture-positive UTI cases referred as suspected UTI from the community for evaluation from April 2017-March 2018 were done. Demographic data, organisms, their susceptibility to common antibiotics prescribed in our community settings were analysed. UTI was common in boys between 1-5years. The common organism isolated was E.coli and Enterococcus species, followed by Klebsiella in girls and Proteus in males. Nearly 40% of GNB isolates were ESBL producing organisms with E.coli being highest. 70% of E.coli were resistant to commonly used 1stline antibiotics-Ampicillin and cephalexin. 50% of other isolates were resistant to common antibiotics. This may lead to failure of treatment in UTI, leading to long-term complications. Hence empirical antibiotic therapy is not advised even in resource constraint community settings.

Ultrasound in Hospitalized Children With First Febrile UTI: What Exactly Are We Looking For?

Detection of genitourinary abnormalities and vesicoureteral reflux (VUR) in young children who present with a febrile urinary tract infection (UTI) is important because severe VUR may predispose to renal scar formation, which may lead to hypertension and impaired kidney function. In this issue of Hospital Pediatrics , Wallace et al1 attempt to identify clinical factors that could be used to predict which hospitalized children 0 to 24 months of age with first febrile UTI are likely to have an abnormal renal bladder ultrasound (RBUS). The authors were not able to determine any clinical factors (other than known genitourinary tract abnormalities) that could be used to guide selective RBUS screening. The authors conclude that selective screening cannot be applied and recommend screening all young children at the time of first febrile UTI. Screening for genitourinary abnormalities technically precedes UTI in most children because severe genitourinary abnormalities and clinically significant hydronephrosis may be noted on a prenatal ultrasound. One study of 4586 children revealed that a prenatal ultrasound identified severe genitourinary tract abnormalities requiring immediate intervention with a negative predictive value of 99.9%.2 In a prescreened population, we would not expect an ultrasound at the time of first UTI to detect prenatally missed posterior urethral valves or high-grade obstruction. The population in this study was a prescreened population in which all patients had a normal prenatal ultrasound, and all patients with …

The use of fetal MRI for renal and urogenital tract anomalies.

Fetal anomalies are detected in approximately 2% of all fetuses and, among these, genitourinary tract abnormalities account for 30% to 50% of all structural anomalies present at birth. Although ultrasound remains the first line diagnostic modality, fetal MRI provides important additional structural and functional information, especially with the development of faster sequences and the use of functional sequences. The added value of MRI-based imaging is three-fold: (a) improvement of diagnostic accuracy by adequate morphological examination, (b) detection of additional anomalies, and (c) in addition, MRI has the potential to provide information regarding renal function. In this review, we describe the role of fetal MRI in the anatomical evaluation of renal and urogenital tract anomalies, and we also touch upon the contribution of functional MRI to the diagnostic workup of these conditions.

A CASE OF URINOTHORAX

SESSION TITLE: Wednesday Fellows Case Report Posters SESSION TYPE: Fellow Case Report Posters PRESENTED ON: 10/23/2019 09:45 AM - 10:45 AM INTRODUCTION: A urinothorax is a rare cause of pleural effusion which can occur in urinary obstruction or from trauma to the genitourinary system. We present a case of urinothorax in a patient with bilateral hydronephrosis. CASE PRESENTATION: A 70 year old female with metastatic cholangiocarcinoma presented with abdominal pain, anuria, and shortness of breath for three days. She was found to have an acute kidney injury with bilateral hydronephrosis and left sided forniceal rupture. Physical examination also revealed decreased breath sounds over the left hemithorax. Chest imaging revealed complete opacification of the left hemithorax with mediastinal shift to the right. She was admitted to the intensive care unit and a diagnostic and therapeutic thoracentesis was performed with removal of 1.9L of straw colored transudative fluid. Pleural fluid creatinine was 12.6. The pleural fluid creatinine to serum creatinine ratio was >1. Bilateral percutaneous nephrostomy tubes were placed and renal function and urine output improved. Chest imaging done two months later did not show any re-accumulation of pleural effusion. DISCUSSION: A urinothorax is an uncommon complication of obstructive uropathy and genitourinary tract abnormalities that can occur in malignancy or traumatic injury. It is typically a unilateral, straw-colored effusion with a pH lower than 7.40 and transudative in nature by Light’s criteria. A urinothorax has two possible mechanisms. Urine can collect in the retroperitoneal space if an obstruction is present and the fluid enters the peritoneal cavity and thereafter leaks through transdiaphragmatic holes into the pleural space. Urine may also travel through lymphatics into the pleural cavity. A high degree of clinical suspicion is needed to make the diagnosis. In patients with concomitant urinary tract infections, especially with ammonia producing organisms, the effusion may be exudative as well as having a pH higher than 7.40, thereby confusing the clinical picture. A pleural fluid creatinine to serum creatinine ratio of greater than 1 clinches the diagnosis of a urinothorax. Further confirmatory workup can be done with a renal scintigraphy study to reveal extravasation of urine from the genitourinary system into the pleural space. Treatment of a urinothorax is aimed at the management of the underlying genitourinary condition. CONCLUSIONS: A urinothorax should be included in the differential diagnosis in a patient with obstructive uropathy or recent surgical genitourinary interventions presenting with a pleural effusion. The condition is completely reversible after treatment of the underlying cause. Reference #1: Handa A, Agarwal A, Aggarwal AN. Urinothorax: an unusual cause of pleural effusion. Singapore Med J 2007; 48(11):e289–e292 Reference #2: Chandra A, Pathak A, Kapur A, Russia N, Bhasin N. Urinothorax: A rare cause of severe respiratory distress. Indian J Crit Care Med. 2014 May; 18(5): 320–322. Reference #3: Garcia-Pachon E, Romero S. Urinothorax: a new approach. Curr Opin Pulm Med. 2006; 12: 259-263 DISCLOSURES: No relevant relationships by Sujith Cherian, source=Web Response No relevant relationships by Rosa Estrada-Y-Martin, source=Web Response No relevant relationships by Bibi Aneesah Jaumally, source=Web Response

Prevalence of Bacterial Urinary Tract Infections and Associated Factors among Patients Attending Hospitals in Bushenyi District, Uganda.

Urinary tract infections (UTIs) are one of the major causes of morbidity and comorbidities in patients with underlying conditions, and it accounts for the majority of the reasons for hospital visit globally. Sound knowledge of factors associated with UTI may allow timely intervention that can easily bring the disease under control. This study was designed to determine the prevalence of UTI by isolating and characterizing the different bacterial etiological agents and to evaluate the factors associated with UTI. In this cross-sectional study, a total of 267, clean catch midstream urine (MSU) samples were collected aseptically and analyzed using standard microbiology methods. Data for the factors associated with UTI were obtained by use of questionnaires and standard laboratory tests for selected underlying conditions. The study revealed 86/267 (32.2%) UTI prevalence among patients attending hospitals in Bushenyi District, Uganda. Escherichia coli was the most prevalent bacterial uropathogen with 36/86 (41.9%) followed by Staphylococcus aureus 27/86 (31.4%), Klebsiella pneumoniae 10/86 (11.6%), Klebsiella oxytoca 6/86 (7.0%), Proteus mirabilis 3/86 (3.5%), Enterococcus faecalis 3/86 (3.5%), and Proteus vulgaris 1/86 (1.2%). This study has demonstrated that age ≤19 years, female gender, married individuals, genitourinary tract abnormalities, diabetes, hospitalization, indwelling catheter <6 days, and indwelling catheter >6 days had statistically significant relationships (p < 0.05) with UTI. Screening for UTI in hospitalized patients, female gender, married individuals, genitourinary tract abnormalities, indwelling catheter, and diabetics should be adopted.

Fraser Syndrome: A Case Report of a Very Rare Syndrome

Fraser syndrome is a rare autosomal recessive multiple malformation syndrome. It is characterized by cryptophthalmos, cutaneus syndactyly, malformation of the larynx and genitourinary tract, craniofacial dysmorphisms, mental retardation, and musculoskeletal abnormalities. The diagnosis of this syndrome can be made on the basis of physical examination and occasionally with prenatal ultrasound imaging. Various mutations of FRAS1, FREM2 and GRIP1 genes are known to play a role in the etiology of the disorder. In this article we want to introduce a case of a female newborn, who presented with cryptophthalmos, syndactyly of both feet and hands, ambiguous genitalia, anal fistule, umbilical hernia, unilateral renal agenesis, and craniofacial dysmorphism. In this case report, we aimed to present and discuss our patient’s clinical findings, genetic basis of Fraser syndrome and follow-up cases from the literature.

Anomalies of the genitourinary tract in children with 22q11.2 deletion syndrome.

The 22q11.2 deletion syndrome (22q11.2DS) involves multiple organ systems with variable phenotypic expression. Genitourinary tract abnormalities have been noted to be present in up to 30-40% of patients. At our institution, an internationally recognized, comprehensive, and multidisciplinary 22q11.2DS care center has been providing care to these children. We sought to report on the incidence of genitourinary tract anomalies in this large cohort and, therefore, retrospectively reviewed all patients who underwent a complete evaluation from 1992 to March 2017. We identified all children with any genital or urinary tract anomaly. For all children with a diagnosis of hydronephrosis, the underlying etiology was determined, when possible. Overall, 1,073 of 1,267 children with 22q11.2DS underwent renal evaluations at our institution. Hundered Sixty-Two (15.1%) children had structural abnormalities of their kidneys/urinary tracts. The majority of children with hydronephrosis (63%) had isolated upper tract dilation without any additional diagnoses. Boys were significantly more likely to be diagnosed with a genital abnormality than girls (7.7 vs. 0.5%, p < 0.001). Of the 649 boys in the entire cohort, 24 (3.7%) had cryptorchidism and 24 (3.7%) had hypospadias, which was noted to be mild in all except one boy. Overall, findings of hydronephrosis, unilateral renal agenesis, and multicystic dysplastic kidney occur at higher rates than expected in the general population. Given these findings, in addition to routine physical examination, we believe that all patients with 22q11.2DS warrant screening RBUS at time of diagnosis.

Síndrome Prune Belly: sobrevida de un paciente con insuficiencia renal

El síndrome de Prune Belly es un trastorno congénito infrecuente, caracterizado por alteración de la musculatura abdominal (hipoplasia), criptorquidia bilateral y anomalías del tracto genitourinario; esta última característica es determinante para la amplia variedad clínica con que se presenta este trastorno en la etapa neonatal y, además, está directamente asociada con el pronóstico de la enfermedad, dado que este depende del grado de compromiso de la función renal al momento del diagnóstico. En este reporte, se presenta el caso de un neonato con diagnóstico prenatal de este síndrome, con manejo multidisciplinario durante el periodo neonatal . Se recomienda el abordaje diagnóstico-terapéutico temprano y oportuno que disminuya el riesgo de complicaciones y permita prolongar la sobrevida en este tipo de pacientes.

A female patient with urethral calculus presenting with bladder distention: A case report

Urethral calculi are extremely rare, especially in female patients, and usually associated with genitourinary tract abnormalities such as urethral stricture or diverticulum. Primary calculus of the urethra is very rare and usually associated with congenital diverticulum. Secondary urethral calculus, often originate from the kidney or bladder. However, primary calculus of the urethra is very rare and is usually associated with congenital diverticulum. Diagnosis of urethral calculus is difficult due to location, and failure of the diagnosis may cause urethral injury, incontinence or renal insufficiency. Herein, we report the computed tomography, clinical and surgical findings of a urethral calculus in a female patient without any urogenital anomaly.

A Challenging Form of Non-autoimmune Insulin-Dependent Diabetes in a Wolfram Syndrome Patient with a Novel Sequence Variant.

Wolfram syndrome type 1 is a rare, autosomal recessive, neurodegenerative disorder that is diagnosed when insulin-dependent diabetes of non-auto-immune origin and optic atrophy are concomitantly present. Wolfram syndrome is also designated by DIDMOAD that stands for its most frequent manifestations: diabetes insipidus, diabetes mellitus, optic atrophy and deafness. With disease progression, patients also commonly develop severe neurological and genito-urinary tract abnormalities. When compared to the general type 1 diabetic population, patients with Wolfram Syndrome have been reported to have a form of diabetes that is more easily controlled and with less microvascular complications, such as diabetic retinopathy.We report a case of Wolfram syndrome in a 16-year-old male patient who presented with progressive optic atrophy and severe diabetes with very challenging glycemic control despite intensive therapy since diagnosis at the age of 6. Despite inadequate metabolic control he did not develop any diabetic microvascular complications during the 10-year follow-up period. To further investigate potential causes for this metabolic idiosyncrasy, we performed genetic analyses that revealed a novel combination of homozygous sequence variants that are likely the cause of the syndrome in this family. The identified genotype included a novel sequence variant in the Wolfram syndrome type 1 gene along with a previously described one, which had initially been associated with isolated low frequency sensorineural hearing loss (LFSNHL). Interestingly, our patient did not show any abnormal findings with audiometry testing.

Congenital pouch colon in girls: Genitourinary abnormalities and their management.

Aims:To discuss the assessment and management of genitourinary (GU) tract abnormalities in 21 girls with Types I-III congenital pouch colon (CPC), studied over a period of 10 years.Materials and Methods:Assessment included clinical and radiological assessment, examination under anesthesia (EUA), endoscopy of the lower GU tract, and evaluation of the surgical findings, operative procedures for the GU anomalies, and the results of management.Results:Initial examination of the external genitalia showed a “clover-leaf” appearance (n = 6) and a single perineal opening (n = 6). In 9 patients, the openings of the urethra and double vagina were seen, of which a vestibular fistula was seen in 5 and an anterior perineal fistula in 1. Seventeen patients (81%) had urinary incontinence (UI) - partial in 10, and complete in 7. Renal function tests, X-ray sacrum, and abdominal US were normal in all patients. Micturating cystourethrogram (n = 9) showed a wide, bladder neck incompetence (BNI) with reduced bladder capacity in seven patients. EUA and endoscopy revealed a septate vagina in all patients and the urethral opening at a “high” position (n = 14) or at a relatively normal or “low” position (n = 7). In 8 patients, the intervaginal septum was thick and fleshy. Endoscopy showed a short, wide urethra, an open incompetent bladder neck, poorly developed trigone, and reduced bladder capacity in the patients with UI. The fistula from the colonic pouch opened in the proximal urethra (n = 4), high in the vestibule (n = 3), low in the vestibule (n = 8), perineum just posterior to the vestibule (n = 1), and undetermined (n = 5). Vaginoscopy (n = 8) showed normal cervices in all and cervical mucus in 4 patients. The subtypes of CPC were Type I CPC (n = 4), Type II CPC (n = 16), and Type III CPC (n = 1). All 21 patients had uterus didelphys. In four patients with UI, during tubular colorraphy, a segment of the colonic pouch was preserved for later bladder augmentation if required. A Young-Dees bladder-neck repair (BNR) was performed in four older girls for treatment of UI, with marked improvement in urinary continence in two girls, some improvement in one girl with complete urinary incontinence, and minimal improvement in one child. Division of the intervaginal septum was performed in three girls.Conclusions:GU abnormalities in girls with CPC need to be assessed and managed by a tailored protocol. UI is frequent, and its correction may require BNR. A segment of the colonic pouch can be preserved for possible future bladder augmentation. All girls have a septate vagina, often widely separated, and uterus didelphys. Gynecologic assessment and monitoring is required throughout adult life. Considering the wide opening of the vestibule, surgical management of the urogenital component by division of the intervaginal septum and if required, the vagino-fistula septum on each side results in a normal or a hypospadiac urethral opening and an adequate vaginal inlet.

Group d salmonella urinary tract infection in an immunocompetent male.

A 62-year-old male with past medical history of benign prostatic hyperplasia presented to the emergency department with complaints of decreased urinary flow, inability to fully empty his bladder, and gross hematuria. Physical examination was unremarkable. Urinalysis revealed large amount of blood and more than 700 white blood cells suggesting a urinary tract infection. Urine culture grew group D Salmonella greater than 100,000 colony-forming units per mL. He was prescribed 6 weeks of trimethoprim/sulfamethoxazole and had resolution of symptoms. Retrospectively, he reported a 3-day history of watery diarrhea about a week prior to onset of urinary symptoms that was presumed to be the hematogenous source in this case. Urinary tract infection from nontyphoidal Salmonella (NTS) is rare and is usually associated with immunosuppression, chronic diseases, such as diabetes or structural abnormalities of the genitourinary tract. Genitourinary tract abnormalities previously reported in the literature that predispose to nontyphoidal Salmonella urinary tract infection include nephrolithiasis, chronic pyelonephritis, retrovesicular fistula, urethrorectal fistula, hydrocele, and post-TURP. We present an exceedingly uncommon case of 62-year-old male with group D Salmonella urinary tract infection predisposed by his history of benign prostatic hyperplasia.

Sonographic Finding of Persistent Renal Pelvic Wall Thickening in Children

Renal pelvic wall thickening (RPWT) is a sonographic finding, which is associated with urinary tract infection (UTI) and other genitourinary tract abnormalities. We aimed to determine the prevalence of RPWT and whether persistent RPWT related to vesicoureteral reflux (VUR). We retrospectively reviewed sonographic findings of RPWT in children and adolescents referred for renal ultrasound study from January 2010 to December 2011. A total of 502 patients showing RPWT were included, 372 of whom received follow-up sonograms. Among them, 86 children underwent both follow-up sonograms and voiding cystourethrography studies. The association between persistent RPWT and VUR was analyzed. A total of 602 sonograms with RPWT were identified, accounting for a prevalence of 11.4%. Follow-up sonograms, revealing that these patients had recovered from RPWT, was found in 93.7% (459/490) of renal units and in 92.7% (345/372) of the patients. Children with persistent RPWT had a strong association with VUR occurrence (p=0.018) and high VUR grading (p=0.006) compared to those without persistent RPWT. RPWT is a common finding in children and adolescents. Persistent RPWT is associated with VUR, especially with high grade VUR. Complementary uroradiological studies should be performed for children and adolescents with persistent RPWT.

Accessory Scrotum with Bifid Scrotum and Hypospadias

Congenital abnormalities of the scrotum are extremely rare, and sometimes associated with other genitourinary tract abnormalities. In this report we presented a male neonate with multiple perineal anomalies including accessory scrotum, bifid scrotum, and hypospadias.