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Hypospadias

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https://www.readbyqxmd.com/read/28225973/do-you-know-this-syndrome-leopard-syndrome
#1
Flávio Heleno da Silva Queiroz Cançado, Luis Candido Pinto da Silva, Paulo Franco Taitson, Ana Carolina Dias Viana de Andrade, Matheus Melo Pithon, Dauro Douglas Oliveira
Hypertrophic cardiomyopathy is known as Leopard syndrome, which is a mnemonic rule for multiple lentigines (L), electrocardiographic conduction abnormalities (E), ocular hypertelorism (O), pulmonary stenosis (P), abnormalities of genitalia (A), retardation of growth (R), and deafness (D). We report the case of a 12-year-old patient with some of the abovementioned characteristics: hypertelorism, macroglossia, lentigines, hypospadias, cryptorchidism, subaortic stenosis, growth retardation, and hearing impairment...
January 2017: Anais Brasileiros de Dermatologia
https://www.readbyqxmd.com/read/28222406/leydig-cell-clustering-and-reinke-crystal-distribution-in-relation-to-hormonal-function-in-adult-patients-with-testicular-dysgenesis-syndrome-tds-including-cryptorchidism
#2
Rikke R Soerensen, Trine H Johannsen, Niels E Skakkebaek, Ewa Rajpert-De Meyts
OBJECTIVE: Testicular dysgenesis syndrome (TDS) comprises testicular germ cell cancer, cryptorchidism and some cases of male infertility and hypospadias, which can be linked to impairment of intrauterine gonadal development. Among histological signs of TDS, large Leydig cell (LC) clusters (micronodules) are frequently present. This study aimed to investigate possible associations of LC micronodules with the presence of Reinke crystals and hormonal function of LCs, the latter primarily reflected by serum concentrations of luteinising hormone (LH) and testosterone, in patients with TDS...
October 2016: Hormones: International Journal of Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28216923/direct-visual-internal-urethrotomy-is-it-a-durable-treatment-option
#3
Dilip Kumar Pal, Sanjay Kumar, Bastab Ghosh
OBJECTIVE: To evaluate the long-term success rate of direct vision internal urethrotomy as a treatment for anterior urethral strictures. MATERIALS AND METHODS: We retrospectively analyzed the results for patients who underwent internal urethrotomy from January 2009 to January 2014 for anterior urethral strictures. Patients were followed till January 2016. Patients with complicated urethral strictures with a history of previous urethroplasty, hypospadias repair, or previous radiation were excluded from the study, as anticipated low success rate of direct visual internal urethrotomy (DVIU) in these patients...
January 2017: Urology Annals
https://www.readbyqxmd.com/read/28215832/variation-in-the-clinical-and-genetic-evaluation-of-undervirilized-boys-with-bifid-scrotum-and-hypospadias
#4
J M Swartz, R Ciarlo, E Denhoff, A Abrha, D A Diamond, J N Hirschhorn, Y-M Chan
BACKGROUND: Bifid scrotum and hypospadias can be signs of undervirilization, yet boys presenting with these findings often do not undergo genetic evaluation. In some cases, identifying an underlying genetic diagnosis can help to optimize clinical care and improve guidance given to patients and families. OBJECTIVES: The aim of this study was to characterize current practice for genetic evaluation of patients with bifid scrotum, and to identify approaches with a good diagnostic yield...
January 30, 2017: Journal of Pediatric Urology
https://www.readbyqxmd.com/read/28213855/correction-of-residual-ventral-penile-curvature-after-division-of-the-urethral-plate-in-the-first-stage-of-a-2-stage-proximal-hypospadias-repair
#5
REVIEW
Bruce J Schlomer
PURPOSE OF REVIEW: The first stage of a 2-stage proximal hypospadias repair involves division of the urethral plate and correction of any residual ventral penile curvature (VPC). Options to correct residual VPC include dorsal corporal shortening or ventral corporal lengthening techniques. This review discusses these options and suggests an approach to management. RECENT FINDINGS: Recent reports of 2-stage proximal hypospadias repairs indicate low rates of recurrent VPC with either dorsal corporal shortening or ventral corporal lengthening...
February 2017: Current Urology Reports
https://www.readbyqxmd.com/read/28209183/variants-in-congenital-hypogonadotrophic-hypogonadism-genes-identified-in-an-indonesian-cohort-of-46-xy-under-virilised-boys
#6
Katie L Ayers, Aurore Bouty, Gorjana Robevska, Jocelyn A van den Bergen, Achmad Zulfa Juniarto, Nurin Aisyiyah Listyasari, Andrew H Sinclair, Sultana M H Faradz
BACKGROUND: Congenital hypogonadotrophic hypogonadism (CHH) and Kallmann syndrome (KS) are caused by disruption to the hypothalamic-pituitary-gonadal (H-P-G) axis. In particular, reduced production, secretion or action of gonadotrophin-releasing hormone (GnRH) is often responsible. Various genes, many of which play a role in the development and function of the GnRH neurons, have been implicated in these disorders. Clinically, CHH and KS are heterogeneous; however, in 46,XY patients, they can be characterised by under-virilisation phenotypes such as cryptorchidism and micropenis or delayed puberty...
February 16, 2017: Human Genomics
https://www.readbyqxmd.com/read/28199199/association-of-mamld1-single-nucleotide-polymorphisms%C3%A2-with-hypospadias-in-chinese-han-population
#7
Yidong Liu, Weijing Ye, Ming Wu, Yiran Huang
Hypospadias is one of the most common congenital malformations among children. Both gene mutations and environmental factors are thought to be involved in the development of hypospadias. The mastermind-like domain-containing 1 gene (MAMLD1, formerly CXorf6) is a new candidate gene and its mutation has been shown in some cases of hypospadias. Here, by direct sequencing of PCR products, we assessed and found mutations that occur in 220 sporadic cases of hypospadias. The mutations p.N589S (c.1766A>G) was found at a significantly higher rate among patients with hypospadias...
March 1, 2017: Frontiers in Bioscience (Landmark Edition)
https://www.readbyqxmd.com/read/28197032/double-breasting-spongioplasty-in-tubularized-tubularized-incise-plate-urethroplasty-a-new-technique
#8
Amilal Bhat, Mahakshit Bhat, Rajeev Kumar, Akshita Bhat
INTRODUCTION: The main disadvantage of currently described techniques of spongioplasty is superimposition of 3 suture lines (neourethra, spongioplasty, and skin closure) which is likely to increase the chances of a fistula. We describe and evaluate the results of a double breasting spongioplasty in urethroplasty. METHODS: A prospective study of 60 primary hypospadias was undertaken by double breasting spongioplasty from August 2012 to March 2014. Mobilization of the urethral plate and the spongiosum is done by creating a plane just proximal to the meatus...
January 2017: Indian Journal of Urology: IJU: Journal of the Urological Society of India
https://www.readbyqxmd.com/read/28163817/a-simple-dressing-for-hypospadias-surgery-in-children
#9
Roberto Méndez-Gallart, María García-Palacios, Pablo Rodríguez-Barca, Elina Estévez-Martínez, Ana Lema Carril, Adolfo Bautista-Casasnovas
One of the most controversial aspects of hypospadias surgery is the election of an appropriate wound dressing. In fact, there may be as many different types of dressing as there are types of surgical repair. Here, we describe a new, simple method for hypospadias dressing in children that minimizes painful removal.
January 2017: Canadian Urological Association Journal, Journal de L'Association des Urologues du Canada
https://www.readbyqxmd.com/read/28163807/efficacy-of-using-zaontz-urethral-stent-in-hypospadias-repair-by-the-face-legs-activity-cry-consolability-flacc-scale-a-prospective-study
#10
Serkan Ozcan, Murat Bagcioglu, Tolga Karakan, Mehmet Akif Diri, Arif Demirbas
INTRODUCTION: The developments in hypospadias surgical techniques and materials are intended to improve surgery outcomes and patient comfort. The aim of this study is to determine the effect of the Zaontz urethral stent (ZUS) (Cook Medical) on patient comfort and surgical success rates in children undergoing hypospadias surgery. METHODS: A feeding tube was used to repair 46 cases of primary distal hypospadias, and ZUS (6F, 8F, and 10F in diameter) was used to repair to 31 cases of primary distal hypospadias between December 2009 and June 2011 in our clinic...
January 2017: Canadian Urological Association Journal, Journal de L'Association des Urologues du Canada
https://www.readbyqxmd.com/read/28161405/neo-yoke-repair-for-severe-hypospadias-a-simple-modification-for-better-outcome
#11
Hamed M Seleim, Hani Morsi, Mohamed M Elbarbary
BACKGROUND: Although staged repair for reconstructing severe hypospadias is more popular, various one-stage repairs have been attempted. Koyanagi repair (parameatal-based and fully extended circumferential foreskin flap urethroplasty) has enabled correction of severe hypospadias in one stage. However, its un-acceptably high incidence of complications has initiated a series of technical modifications, including the "yoke" repair. OBJECTIVES: To retrospectively analyze the outcome of a proposed modification of the originally described yoke repair, for patients with severe hypospadias...
January 14, 2017: Journal of Pediatric Urology
https://www.readbyqxmd.com/read/28160395/a-novel-disorder-of-sex-development-characterized-by-progressive-regression-of-testicular-function-and-cystic-leukoencephalopathy
#12
Massimiliano Rossi, Alexandre Vasiljevic, Audrey Labalme, Frédérique Dijoud, Delphine Mallet-Motak, Carmen Adina Petcu, Renaud Touraine, Christine Vianey-Saban, Laurent Guibaud, Patrick Edery, Damien Sanlaville, Yves Morel
We report a novel syndromic disorder of sex development observed in three male siblings, presenting with the association of micropenis without hypospadias, cryptorchidism, very low level of antimüllerian hormone in the neonatal period, and no persistent müllerian duct structures, suggesting a progressive regression of testicular function. The patients described here showed a striking neurological involvement including bilateral periventricular cysts observed in the anterior part of the frontal horns prenatally and increasing in size and number over time, associated with infra and supratentorial parenchymal atrophy, dilated ventricular system, corpus callosum hypoplasia, severe intellectual disability, and epilepsy...
February 4, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28152494/transverse-testicular-ectopia-with-scrotal-hypospadias-but-without-inguinal-hernia-case-report-of-a-rare-association
#13
Pradeep Kajal, Kamal Nain Rattan, Namita Bhutani, Vijender Sangwan
Transverse testicular ectopia is an extremely rare anomaly, in which both the testis migrate towards the same hemiscrotum through the same inguinal canal. It is usually associated with other abnormalities such as persistent Mullerian duct syndrome, true hermaphroditism, inguinal hernia, pseudohermaphroditism, and scrotal anomalies; the association with inguinal hernia being the commonest. We, here, report a case of transverse testicular ectopia in a 12 years old boy having the ectopic testis in contralateral hemiscrotum without any inguinal hernia but having scrotal hypospadias with severe chordee...
January 19, 2017: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/28131500/resurfacing-the-penis-of-complex-hypospadias-repair-hypospadias-cripples
#14
Mina M Fam, Moneer K Hanna
PURPOSE: After the creation of a neourethra in a "hypospadias cripple," resurfacing the penis with healthy skin is a significant challenge because local tissue is often scarred and unusable. We reviewed our experience with various strategies to resurface the penis of hypospadias cripples. MATERIALS AND METHODS: We retrospectively reviewed the records of 215 patients referred after multiple unsuccessful hypospadias repairs from 1981 to 2014. In 130 of 215 patients we performed resurfacing using local penile flaps using various techniques, including Byars flaps, Z-plasty or double Z-plasty, or a dorsal relaxing incision...
March 2017: Journal of Urology
https://www.readbyqxmd.com/read/28118624/durham-smith-vest-over-pant-technique-simple-procedure-for-a-complex-problem-post-hypospadias-repair-fistula
#15
Venkat A Gite, Saurabh R Patil, Sachin M Bote, Mohd Ayub Karam Nabi Siddiqui, Jayant V Nikose, Anitha J Kandi
OBJECTIVE: Urethrocutaneous fistula, which occurs after hypospadias surgery, is often a baffling problem and its treatment is challenging. The study aimed to evaluate the results of the simple procedure (Durham Smith vest-over-pant technique) for this complex problem (post-hypospadias repair fistula). METHODS: During the period from 2011 to 2015, 20 patients with post-hypospadias repair fistulas underwent Durham Smith repair. Common age group was between 5 and 12 years...
January 25, 2017: Urologia Internationalis
https://www.readbyqxmd.com/read/28111210/commentary-to-does-maternal-exposure-during-pregnancy-to-higher-ambient-temperature-increase-the-risk-of-hypospadias
#16
Nicolas Fernandez, Ignacio Zarante
No abstract text is available yet for this article.
January 12, 2017: Journal of Pediatric Urology
https://www.readbyqxmd.com/read/28111208/outcome-of-urethroplasty-after-parenteral-testosterone-in-children-with-distal-hypospadias
#17
P Menon, K L N Rao, A Handu, L Balan, N Kakkar
INTRODUCTION: Pre-operative testosterone use in hypospadias surgery is known to increase penile dimensions and vascularity, which should facilitate tension-less formation of the urethral tube and tissue healing. However, androgens can have a negative effect on wound healing. There are very few randomized studies on postoperative results after androgen use, and this study attempted to understand the utility of pre-operative testosterone in distal hypospadias. OBJECTIVE: To study the effect of parenteral testosterone in children undergoing single stage urethroplasty for distal hypospadias, especially the occurrence of urethrocutaneous fistula and wound dehiscence...
January 11, 2017: Journal of Pediatric Urology
https://www.readbyqxmd.com/read/28108798/augmented-perineal-urethrostomy-using-a-dorsal-buccal-mucosal-graft-bi-institutional-study
#18
Jessica DeLong, Kurt McCammon, Leandro Capiel, Augustín Rovegno, Jeremy B Tonkin, Gerald Jordan, Ramón Virasoro
PURPOSE: To present our technique and outcomes for perineal urethrostomy augmented with a dorsal onlay buccal mucosa graft (BMG). Results from initial series and collaboration from an international center are included. METHODS: A retrospective chart review of all adult patients who underwent urethral reconstruction with perineal urethrostomy utilizing a buccal mucosal graft between January 1, 2002 and January 1, 2013 was performed. All surgeries were performed by three surgeons using the same technique (GHJ, KAM, and RV)...
January 20, 2017: World Journal of Urology
https://www.readbyqxmd.com/read/28089292/assessing-quality-of-life-of-patients-with-hypospadias-a-systematic-review-of-validated-patient-reported-outcome-instruments
#19
REVIEW
K J Sullivan, Z Hunter, V Andrioli, L Guerra, M Leonard, A Klassen, M A Keays
BACKGROUND: Patient-reported outcomes have the potential to provide invaluable information for evaluation of hypospadias patients, aid in decision-making, performance assessment, and improvement in quality of care. To appropriately measure patient-relevant outcomes, well-developed and validated patient-reported outcome (PRO) instruments are essential. OBJECTIVE: To identify and evaluate existing PRO instruments designed to measure quality of life and/or satisfaction of individuals with hypospadias that have been developed and validated in a hypospadias population...
December 5, 2016: Journal of Pediatric Urology
https://www.readbyqxmd.com/read/28089291/repair-of-stenosed-neourethra-using-the-proximal-neourethral-diverticulum-a-technique-in-redo-hypospadias-surgery
#20
Cenita J Sam, Sudipta Sen, Pavai A Arunachalam
Neourethral diverticulum secondary to distal neourethral stenosis is not uncommon after proximal hypospadias repair. We describe a technique using the neo urethral diverticular roof and overlying skin as a composite flap to reconstruct the urethral stenosis distal to the diverticulum. The composite flap is advanced distally for reconstruction of the stenosed urethra either as a tube or as an onlay. Urethra at the diverticular bed is tubularized to reduce its caliber. This technique has yielded excellent results in three children...
December 21, 2016: Journal of Pediatric Urology
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