| 1. |
- Andersson, Marie, 1977, et al.
(författare)
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Urological results and patient satisfaction in adolescents after surgery for proximal hypospadias in childhood
- 2020
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Ingår i: Journal of Pediatric Urology. - : Elsevier BV. - 1477-5131 .- 1873-4898. ; 16:5
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Tidskriftsartikel (refereegranskat)abstract
- Background: Proximal hypospadias repair is associated with a considerable complication risk. Long-term follow-up is required to present realistic expectations in pre-operative counseling. Objective: To investigate adolescents after childhood surgery for proximal hypospadias in a prospective cohort study describing the urological outcome, complication rates and patient satisfaction with penile appearance. Study design: 39 adolescents ≥14 years with penoscrotal to perineal hypospadias and primary urethroplasty (tubularized incised plate (TIP), preputial flap as Onlay or tubularized (Duckett)) from 1996 to 2005 at a single center were evaluated. The clinical assessment, at Md 16.5 years (14–25), included voiding history, genital examination including the Hypospadias Objective Scoring Evaluation (HOSE), uroflowmetry plus chart data from previous urinary flows and evaluation of patient satisfaction using the Penile Perception Score (PPS). Results: Twenty-nine patients with penoscrotal and 10 with scrotal/perineal hypospadias underwent surgery with TIP (N = 14), Onlay (N = 14) and Duckett (N = 11). Uroflows improved significantly compared with prepubertal maximal flows. Impaired flow rate (<10 mL/s) was found in 14% (5/36). Fifty-one percent (20/39) required reoperations, 29% (4/14) of TIP, 50% (7/14) of Onlay and 82% (9/11) of Duckett (p = 0.0062). Median penile length in adolescence was 8.7 cm (4.0–11.0). Forty-four percent (12/27) of patients were dissatisfied with penile length. Patients were ‘satisfied’ or ‘very satisfied’ with meatal position and shape despite HOSE for meatal position being 11% (4/38) distal, 76% (29/38) proximal glanular and 13% (5/38) coronal. TIP patients had more curvature at puberty than Duckett (p = 0.0062). Patients that had a decurvature procedure had shorter penile length (p = 0.019). Discussion: A high complication rate is previously described, predominantly within the first years. Our study shows 50% of reoperations were performed after >3 years, illustrating the need for long-term follow-up. Patient satisfaction with a deviant meatal position is rarely reported [1,2]. Our results support a conservative approach to an asymptomatic retracted meatus. Limitations of this descriptive study are the non-comparable groups and the retrospective data for correlation, impeding evaluation of prognostic outcome-factors. The shorter penile length found in patients after plication, and increased curvature after TIP, is therefore merely descriptive. However, the findings are in line with earlier publications suggesting limited use of TIP, and plication (recommending ventral lengthening instead) to avoid penile shortening and curvature in these cases [3–5]. Conclusions: The urological long-term outcome after proximal hypospadias repair is good, although late reoperations are common. In adolescence, patients were dissatisfied with the short penile length but satisfied with meatal position, indicating that in proximal hypospadias, preserving penile length and correcting curvature are prioritized over a distal meatus. [Table presented] © 2020 The Author(s)
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| 2. |
- Holmdahl, Gundela, 1956, et al.
(författare)
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Health-Related Quality of Life in Patients with the Bladder Exstrophy-Epispadias Complex and Relationship to Incontinence and Sexual Factors: A Review of the Recent Literature.
- 2020
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Ingår i: European journal of pediatric surgery : official journal of Austrian Association of Pediatric Surgery ... [et al] = Zeitschrift fur Kinderchirurgie. - : Georg Thieme Verlag KG. - 1439-359X. ; 30:3, s. 251-260
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Forskningsöversikt (refereegranskat)abstract
- The bladder exstrophy-epispadia complex (BEEC) includes malformations with midline closing defects of the lower abdomen and external genitalia. Long-term consequences with urinary incontinence and sexual dysfunction, in spite of multiple surgical interventions, are common and expected to affect the patient's health-related quality of life (HRQOL). The extent and the predictive factors are, however, not known. New patient-reported outcome research is emerging, but valid and reliable condition-specific HRQOL are still missing. The aim of this review is to summarize and discuss the latest published reports (2015-2019) on HRQOL in patients with the BEEC and its relationship to incontinence and sexual factors.
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| 3. |
- Nordenström, Josefin, et al.
(författare)
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Changes in differential renal function after pyeloplasty in infants and children.
- 2020
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Ingår i: Journal of pediatric urology. - : Elsevier BV. - 1873-4898 .- 1477-5131. ; 16:3
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Tidskriftsartikel (refereegranskat)abstract
- Ureteropelvic junction obstruction (UPJO) is one of the most common causes of hydronephrosis in pediatric populations. Many need surgical intervention. The aim of surgery is preserving renal function and reducing symptoms such as urinary tract infections and pain.The objectives were to evaluate differential renal function (DRF) in infants and children after surgery for UPJO and to identify factors predicting postoperative improvement. The difference in outcomes between patients with antenatal hydronephrosis and those diagnosed later was evaluated.A total of 85 children (63 boys and 22 girls) aged 0-16 years, treated for UPJO with dismembered pyeloplasty, were followed up as per a structured protocol including ultrasounds and renal scans (MAG-3) pre-operatively and three and 18 months postoperatively. Five children with bilateral or single kidney UPJO were excluded. Patient records were retrospectively reviewed, and the patients were grouped as per prenatal (group 1, n=23) or postnatal (group 2, n=57) diagnosis. Univariable and multivariable logistic regression analyses searching for factors predicting >5% postoperative improvement in DRF on the obstructed side were performed. Factors included in analyses were age at diagnosis and surgery, sex, type of presentation, cause of obstruction, estimated glomerular filtration rate, pre-operative DRF, anteroposterior diameter (APD), APD/renal parenchymal thickness, and grade of hydronephrosis as per the Onen alternative grading system (grade 1-4).Pre-operative DRF on the obstructed side was a mean of 42% (standard deviation, 12), with no difference between the groups. The median age at surgery was 0.9 (0.2-10) and 8.1 (0.6-16) years in groups 1 and 2, respectively (P<0.001). The majority had unchanged DRF 18 months postoperatively, 19 (27%) patients improved by >5%, and one deteriorated. The proportion of patients with improved DRF was higher in group 1 (n=10; 45%, P=0.026). Anteroposterior diameter, APD/parenchymal thickness, pre-operative DRF, and antenatal diagnosis were predictors in the univariable analyses, and high APD (odds ratio [OR] = 1.1, P=0.0023), antenatal diagnosis (OR = 0.23, P=0.048), and low pre-operative DRF (OR = 0.90, P=0.0045) built the best model of independent factors predicting improvement in DRF in multivariable analyses (Summary Figure).The limitation of the study is that it is retrospective, but it has the advantage of auniform follow-up protocol, including patients from a five-year period, with few lost to follow-up. The results can be of interest in evaluating factors of importance for predicting recovery of function in obstructive uropathies in children.The majority of children had preserved or improved function after surgery for UPJO. Those with an antenatal diagnosis displayed a greater ability to catch up in DRF, and high APD, antenatal diagnosis, and low pre-operative DRF were independent predictive factors of an improvement in renal function after pyeloplasty.
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| 4. |
- Rieke, Johanna Magdalena, et al.
(författare)
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SLC20A1Is Involved in Urinary Tract and Urorectal Development
- 2020
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Ingår i: Frontiers in Cell and Developmental Biology. - : FRONTIERS MEDIA SA. - 2296-634X. ; 8
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Tidskriftsartikel (refereegranskat)abstract
- Previous studies in developingXenopusand zebrafish reported that the phosphate transporterslc20a1ais expressed in pronephric kidneys. The recent identification ofSLC20A1as a monoallelic candidate gene for cloacal exstrophy further suggests its involvement in the urinary tract and urorectal development. However, little is known of the functional role ofSLC20A1in urinary tract development. Here, we investigated this using morpholino oligonucleotide knockdown of the zebrafish orthologslc20a1a. This caused kidney cysts and malformations of the cloaca. Moreover, in morphants we demonstrated dysfunctional voiding and hindgut opening defects mimicking imperforate anus in human cloacal exstrophy. Furthermore, we performed immunohistochemistry of an unaffected 6-week-old human embryo and detectedSLC20A1in the urinary tract and the abdominal midline, structures implicated in the pathogenesis of cloacal exstrophy. Additionally, we resequencedSLC20A1in 690 individuals with bladder exstrophy-epispadias complex (BEEC) including 84 individuals with cloacal exstrophy. We identified two additional monoallelicde novovariants. One was identified in a case-parent trio with classic bladder exstrophy, and one additional novelde novovariant was detected in an affected mother who transmitted this variant to her affected son. To study the potential cellular impact ofSLC20A1variants, we expressed them in HEK293 cells. Here, phosphate transport was not compromised, suggesting that it is not a disease mechanism. However, there was a tendency for lower levels of cleaved caspase-3, perhaps implicating apoptosis pathways in the disease. Our results suggestSLC20A1is involved in urinary tract and urorectal development and implicateSLC20A1as a disease-gene for BEEC.
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