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1.
Pediatr Radiol ; 53(9): 1911-1918, 2023 08.
Artigo em Inglês | MEDLINE | ID: mdl-37171639

RESUMO

BACKGROUND: One of the main limitations of 99mtechnetium-dimercaptosuccinic acid (DMSA) scan is the long acquisition time. OBJECTIVE: To evaluate the feasibility of short DMSA scan acquisition times using a cadmium-zinc-telluride-based single-photon emission computed tomography (SPECT) system in children. MATERIALS AND METHODS: The data of 27 children (median age: 4 years; 16 girls) who underwent DMSA SPECT were retrospectively analyzed. Both planar and SPECT DMSA were performed. SPECT images were analyzed using coronal-simulated planar two-dimensional images. A reduction in SPECT acquisition time was simulated to provide 4 series (SPECT-15 min, SPECT-10 min, SPECT-5 min and SPECT-2.5 min). A direct comparison of the planar and SPECT series was performed, including semi-quantification reproducibility, image quality (mean quality score on a scale of 0 to 2) and inter- and intra-observer reproducibility of the scintigraphic patterns. RESULTS: The overall image quality score (± standard deviation) was 1.3 (± 0.6) for the planar data set, 1.6 (± 0.5) for the SPECT-15 min data set, 1.4 (± 0.5) for the SPECT-10 min data set, 1.0 (± 0.5) for the SPECT-5 min data set and 0.6 (± 0.6) for the SPECT-2.5 min data set. Median Kappa coefficients for inter-observer agreement between planar and SPECT images were greater than 0.83 for all series and all readers except one reader for the SPECT-2.5 min series (median Kappa coefficient = 0.77). CONCLUSION: Shortening SPECT acquisitions to 5 min is feasible with minimal impact on images in terms of quality and reproducibility.


Assuntos
Ácido Dimercaptossuccínico Tecnécio Tc 99m , Tomografia Computadorizada de Emissão de Fóton Único , Criança , Feminino , Humanos , Pré-Escolar , Reprodutibilidade dos Testes , Estudos Retrospectivos , Tomografia Computadorizada de Emissão de Fóton Único/métodos
2.
J Paediatr Child Health ; 58(9): 1508-1519, 2022 09.
Artigo em Inglês | MEDLINE | ID: mdl-35791898

RESUMO

In hypospadias surgery, pre-operative hormonal therapy (PHT) is primarily used to increase penile dimensions and the vascularity of tissues available for reconstruction, but its use is non-uniform in clinical practice, with no consensus on application or utility. This review aims to summarise: (i) the penile tissue response to hormone therapy, (ii) its impact on hypospadias surgery outcomes, and (iii) the endocrinological considerations and sequelae. PHT is more often indicated for complex cases such as proximal hypospadias, hypospadias with microphallus and hypospadias reoperations. While PHT has clear effects on penile morphometry, and more recent controlled trials suggest improved surgical outcomes, the lack of consistent outcome definitions and generally inadequate follow-up periods continue to consign many of the potential long-term effects of PHT to the unknown. There is currently insufficient robust evidence to allow a clinical guideline to be constructed. The need for a well-powered multi-centre prospective randomised trial to address this question is evident but awaits a unified consensus on issues surrounding the understanding of aetiology, classification of hypospadias morphology, definition of important prognostic variables and uniform application of outcome measures. The effects of PHT may be utilised to improve outcomes in cases of proximal and severe hypospadias, which under the current paradigm represent a significant surgical challenge.


Assuntos
Hipospadia , Doenças do Pênis , Humanos , Hipospadia/cirurgia , Masculino , Estudos Multicêntricos como Assunto , Pênis , Estudos Prospectivos , Ensaios Clínicos Controlados Aleatórios como Assunto , Resultado do Tratamento
3.
J Sex Med ; 15(3): 314-323, 2018 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-29502980

RESUMO

BACKGROUND: The bladder exstrophy-epispadias complex is a rare congenital malformation associated with severe dysfunction of the genital and urinary tracts and requiring a staged surgical reconstruction. AIM: The primary aims of this study were to report the sexuality, infertility, and urinary incontinence outcomes in a cohort of men born with bladder exstrophy-epispadias complex. The secondary aim was to highlight some predictive factors of infertility in this population. METHODS: We conducted a descriptive, cross-sectional study of men diagnosed with classic presentations of bladder exstrophy or epispadias. OUTCOMES: Patients were asked to complete 4 validated questionnaires: the International Index of Erectile Function (IIEF)-5, the Erection Hardness Score (EHS), the Self-Esteem and Relationship, and the International Consultation Incontinence modular Questionnaire-Short Form. Fertility potential was assessed with semen analysis and a non-validated questionnaire. RESULTS: 38 Patients 18-64 years old (M [mean] = 32.2) completed the questionnaires. The average IIEF-5 score was 18.1/25 (ranging from 3-25; SE = 7.62), with results indicating that 55% of the sample had normal erectile function. Results also showed higher scores for patients with normal spermatozoa concentration (M = 22.75, SE = 1.89, P = .08) than for those with oligospermia (M = 17.30, SE = 8.53, P = .08). Results on the IIEF-5 also indicated higher scores for patients who conceived children without assisted reproductive technologies (ART) (M = 22.83, SE = 2.317, P = .02) than for patients without children (M = 15.76, SE = 8.342, P = .02). The average EHS was 3.43/4 (ranging from 1-4, SE = 0.9). EHS was higher for patients who had reconstruction than for patients who had cystectomy (M = 3.88, SE = 1.07 and 2.78, SE = 1.09, P = .02). The average total Self-Esteem and Relationship score was 67.04/100 (ranging from 10.71-96.43, SE = 22.11). The average total International Consultation Incontinence modular Questionnaire-Short Form score was 4.97/21 (ranging from 0-18, SE = 5.44), higher score indicating more urinary incontinence. Among the patients surveyed, 31.6% were parents at the time of study and 50% of them benefited from ART. With regards to the 14 semen analyses performed, only 7.1% produced normal results and 44.7% indicated that ejaculation was weak and dribbling. CLINICAL TRANSLATION: Erectile function appears to be decreased and psychological aspects of sexuality indicate low self-esteem about sexual relationship. Although ethical problems could not allow prospective spermograms, our cohort is large enough to provide significant data. CONCLUSIONS: Early sperm storage for future ART, sexual medicine management, and complementary genital reconstruction in adulthood constitute potential treatment options for this population. Reynaud N, Courtois F, Mouriquand P, et al. Male Sexuality, Fertility, and Urinary Continence in Bladder Exstrophy-Epispadias Complex. J Sex Med 2017;15:314-323.


Assuntos
Extrofia Vesical/complicações , Epispadia/complicações , Comportamento Sexual , Sexualidade/fisiologia , Incontinência Urinária/epidemiologia , Adolescente , Adulto , Estudos Transversais , Ejaculação/fisiologia , Fertilidade/fisiologia , Humanos , Masculino , Pessoa de Meia-Idade , Ereção Peniana/fisiologia , Autoimagem , Análise do Sêmen , Inquéritos e Questionários , Adulto Jovem
4.
Pediatr Radiol ; 48(2): 291-303, 2018 02.
Artigo em Inglês | MEDLINE | ID: mdl-29138893

RESUMO

To promote the standardization of nephro-uroradiological terms used in children, the European Society of Paediatric Radiology uroradiology taskforce wrote a detailed glossary. This work has been subsequently submitted to European experts in pediatric urology and nephrology for discussion and acceptance to improve the quality of radiological reports and communication between different clinicians involved in pediatric urology and nephrology.


Assuntos
Pediatria/normas , Radiologia/normas , Terminologia como Assunto , Doenças Urológicas/diagnóstico por imagem , Urologia/normas , Criança , Europa (Continente) , Humanos
5.
J Proteome Res ; 16(10): 3732-3740, 2017 10 06.
Artigo em Inglês | MEDLINE | ID: mdl-28791867

RESUMO

Improving the management of neonatal diseases and prevention of chronic diseases in adulthood requires a better comprehension of the complex maturational processes associated with newborns' development. Urine-based metabolomic studies play a promising role in the fields of pediatrics and neonatology, relying on simple and noninvasive collection procedures while integrating a variety of factors such as genotype, nutritional state, lifestyle, and diseases. Here, we investigate the influence of age, weight, height, and gender on the urine metabolome during the first 4 months of life. Untargeted analysis of urine was carried out by 1H-Nuclear Magnetic Resonance (NMR) spectroscopy for 90 newborns under 4 months of age, and free of metabolic, nephrologic, or urologic diseases. Supervised multivariate statistical analysis of the metabolic profiles revealed metabolites significantly associated with age, weight, and height, respectively. The tremendous growth occurring during the neonatal period is associated with specific modifications of newborns' metabolism. Conversely, gender appears to have no impact on the urine metabolome during early infancy. These results allow a deeper understanding of newborns' metabolic maturation and underline potential confounding factors in newborns' metabolomics studies. We emphasize the need to systematically and precisely report children age, height, and weight that impact urine metabolic profiles of infants.


Assuntos
Recém-Nascido Prematuro/urina , Metaboloma/genética , Metabolômica , Proteínas/genética , Criança , Feminino , Idade Gestacional , Humanos , Lactente , Recém-Nascido , Recém-Nascido Prematuro/crescimento & desenvolvimento , Espectroscopia de Ressonância Magnética , Masculino , Análise Multivariada , Proteínas/metabolismo
6.
J Urol ; 197(4): 1144-1149, 2017 04.
Artigo em Inglês | MEDLINE | ID: mdl-27916713

RESUMO

PURPOSE: We compared the prognostic value of anteroposterior intrasinus diameter of the renal pelvis, urinary tract dilatation and the Society for Fetal Urology grading system in children with prenatally diagnosed unilateral urinary tract dilatation. MATERIALS AND METHODS: All newborns with prenatally diagnosed unilateral urinary tract dilatation, normal bladder and anteroposterior intrasinus diameter 10 mm or greater on the first postnatal ultrasonography were prospectively enrolled from January 2011 to February 2015. Indications for surgery were recurrent febrile urinary tract infections and/or decrease of relative renal function more than 10% on serial isotope studies and/or increasing anteroposterior intrasinus diameter greater than 20% on serial ultrasounds. Sensitivity, specificity and ROC curves were calculated to evaluate the accuracy of anteroposterior intrasinus diameter, urinary tract dilatation and Society for Fetal Urology grading system in determining which children would need surgery within 24 months. RESULTS: A total of 57 males and 13 females were included. Of the patients 33 required surgery at a median age of 5 months (IQR 3.8 to 6.4). Urinary tract dilatation remained stable in 14 cases and decreased in 23 with a median followup of 42 months (IQR 25 to 67). Anteroposterior intrasinus diameter, urinary tract dilatation and Society for Fetal Urology scores were all correlated with the need for surgery. Anteroposterior intrasinus diameter with a threshold of 20 mm had the best prognostic value, with a sensitivity of 81.8% and a specificity of 91.7%. CONCLUSIONS: Our study confirms that the prognostic value was comparable between anteroposterior intrasinus diameter of the renal pelvis, urinary tract dilatation and Society for Fetal Urology grading system in newborns with prenatally diagnosed unilateral urinary tract dilatation. Anteroposterior intrasinus diameter and abnormal parenchymal thickness are the most important ultrasound criteria to identify children at risk for requiring surgery.


Assuntos
Hidronefrose/diagnóstico por imagem , Pelve Renal/diagnóstico por imagem , Dilatação Patológica/diagnóstico por imagem , Feminino , Humanos , Lactente , Masculino , Prognóstico , Estudos Retrospectivos , Ultrassonografia , Ultrassonografia Pré-Natal , Sistema Urinário/diagnóstico por imagem , Sistema Urinário/patologia
7.
Mod Pathol ; 29(11): 1399-1414, 2016 11.
Artigo em Inglês | MEDLINE | ID: mdl-27469328

RESUMO

Disorders of sex development are defined as congenital conditions with discordance between the phenotype, the genotype, the karyotype, and the hormonal profile. The disorders of sex development consensus classification established in 2005 are mainly based on chromosomal and biological data. However, histological anomalies are not considered. The aims of this study were to define the specific pathological features of gonads in various groups of disorders of sex development in order to clarify the nosology of histological findings and to evaluate the tumor risk in case of a conservative approach. One hundred and seventy-five samples from 86 patients with disorders of sex development were analyzed following a strict histological reading protocol. The term 'gonadal dysgenesis' for the histological analysis was found confusing and therefore excluded. The concept of 'dysplasia' was subsequently introduced in order to describe the architectural disorganization of the gonad (various degrees of irregular seminiferous tubules, thin albuginea, fibrous interstitium). Five histological types were identified: normal gonad, hypoplastic testis, dysplastic testis, streak gonad, and ovotestis. The analysis showed an association between undifferentiated gonadal tissue, a potential precursor of gonadoblastoma, and dysplasia. Dysplasia and undifferentiated gonadal tissue were only encountered in cases of genetic or chromosomal abnormality ('dysgenesis' groups in the disorders of sex development consensus classification). 'Dysgenetic testes', related to an embryonic malformation of the gonad, have variable histological presentations, from normal to streak. Conversely, gonads associated with hormonal deficiencies always display a normal architecture. A loss of expression of AMH and α-inhibin was identified in dysplastic areas. Foci of abnormal expression of the CD117 and OCT4 immature germ cells markers in dysplasia and undifferentiated gonadal tissue were associated with an increased risk of neoplasia. This morphological analysis aims at clarifying the histological classification and gives an indication of tumor risk of gonads in disorders of sex development.


Assuntos
Transtornos do Desenvolvimento Sexual/classificação , Transtornos do Desenvolvimento Sexual/patologia , Gônadas/patologia , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Recém-Nascido , Masculino
8.
BJU Int ; 118(6): 980-986, 2016 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-27322857

RESUMO

OBJECTIVE: To document the long-term outcomes of paediatric augmentation gastrocystoplasty (AGC) in terms of preservation of renal function and maintenance of dryness, and to analyse the rate of complications. PATIENTS AND METHODS: The medical records of children who had undergone AGC between 1992 and 2000 (minimum time interval of 15 years) were reviewed retrospectively. The following data were collected: age at surgery, the cause of bladder dysfunction, functioning of the AGC, any complications, and the long-term outcome of the patients. All of the patients were re-contacted by telephone. RESULTS: A total of 11 AGCs were carried out between 1992 and 2000, at a median (range) age of 11 (6.5-14) years. The diagnosis of patients undergoing AGC included myelomeningocele (four), bladder exstrophy (four), posterior urethral valves (one), irradiated bladder (one), and Prune Belly syndrome (one). The median [interquartile range (IQR)] follow-up was 17 (15-19.5) years. Renal function was preserved or improved in seven of the 11 patients and nine patients were dry after AGC. Seven of the 11 patients reported symptoms linked to haematuria-dysuria syndrome, which was resistant to treatment in one case and requiring excision of the gastric patch. Three of the 11 patients developed a tumour on the gastric graft after a median (range) delay of 20 (11-22) years after the initial procedure. All had gastric adenocarcinoma of which two were metastatic at the time of diagnosis requiring pelvectomy with pelvic lymph node dissection and adjuvant chemotherapy. Seven of the 11 patients underwent excision of the gastric patch after a median (IQR) time of 11 (8.5-20.5) years. CONCLUSIONS: Our long-term data confirmed that most patients undergoing AGC had preservation of their renal function and were continent. However, long-term, AGC was associated with a significant risk of malignant transformation and a high rate of surgical re-intervention involving removal of the gastric patch. These results question the use of this technique for bladder augmentation, irrespective of the indication. We highlight the importance of strict endoscopic follow-up of all patients already having undergone an AGC and the need to inform and educated patients about tumour-related symptoms.


Assuntos
Estômago/transplante , Doenças da Bexiga Urinária/cirurgia , Bexiga Urinária/cirurgia , Adolescente , Fatores Etários , Criança , Feminino , Humanos , Masculino , Estudos Retrospectivos , Resultado do Tratamento , Procedimentos Cirúrgicos Urológicos/métodos , Adulto Jovem
9.
J Thromb Thrombolysis ; 38(2): 218-22, 2014.
Artigo em Inglês | MEDLINE | ID: mdl-24338249

RESUMO

Portal vein thrombosis (PVT) is a rare but potentially life-threatening complication of laparoscopic splenectomy (LS) and can lead to bowel ischemia or portal hypertension. In childhood, this complication is reported in 5-10 % of the cases whereas it can be up to 50 % in adult population. Our aim was to evaluate PVT incidence after LS and associated risks factors. A retrospective chart review identified 37 children who underwent elective LS from 2005 to 2013. The main indications were spherocytosis or sickle cell disease. Median age and weight were respectively 7.4 years and 25.1 kg. Thromboembolic prophylaxis was not routinely given. Duration of surgery was 129 min and hospital length of stay 4 days. Doppler ultrasound scan (USS) was performed post-operatively in 26 cases. Post-operative course was uneventful in all but one patient. She was a 17 year-old girl previously operated for an ovarian tumor with hyperandrogenism. Histopathology revealed a splenic lymphoma. At day 4, a systematic USS showed a PVT extending in the portal branches. Therapeutic low molecular weight heparin was used and then transitioned to fluindione for 3 months. Follow-up USS performed at 1 and 4 months demonstrated complete resolution of the PVT. PVT after pediatric LS is a rare event in our series. Clinician should be cautious in oncologic cases and if very large spleen or if thrombocythemia >650.10(9)/L is present. If detected early, PVT can be treated efficiently. We therefore recommend a systematic USS during the first postoperative week.


Assuntos
Laparoscopia/efeitos adversos , Veia Porta , Esplenectomia/efeitos adversos , Trombose/etiologia , Adolescente , Adulto , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Masculino , Complicações Pós-Operatórias , Estudos Retrospectivos , Trombose/diagnóstico por imagem , Trombose/terapia , Fatores de Tempo , Ultrassonografia
10.
Pediatr Hematol Oncol ; 30(4): 288-90, 2013 May.
Artigo em Inglês | MEDLINE | ID: mdl-23444873

RESUMO

Laparoscopy in adrenocortical carcinoma is controversial due to high risk of local recurrence in case of per-operative spillage. We wanted to report the case of a successful laparoscopic transperitoneal adrenalectomy performed in a 3-year-old girl presenting a 5.5-cm adrenocortical carcinoma.


Assuntos
Neoplasias do Córtex Suprarrenal/cirurgia , Carcinoma Adrenocortical/cirurgia , Procedimentos Cirúrgicos Minimamente Invasivos/métodos , Neoplasias do Córtex Suprarrenal/mortalidade , Adrenalectomia , Carcinoma Adrenocortical/mortalidade , Pré-Escolar , Feminino , Humanos , Laparoscopia
11.
J Pediatr Urol ; 19(3): 347-348, 2023 06.
Artigo em Inglês | MEDLINE | ID: mdl-36872172

RESUMO

PURPOSE: To present one way of performing onlay preputial flap for hypospadias. PATIENTS AND METHODS: This procedure was performed following the methodology used in one hypospadias expert center to correct hypospadias in boys who are not elective for Koff procedure and in whom Koyanagi procedure is not needed. Operative details were described, and post-operative management were given as example. RESULTS: Long-term results of this technique showed a 10% complication rate (dehiscence, strictures or urethral fistulas) 2 years after surgery. CONCLUSIONS: This video is a step by step description of the onlay preputial flap technique giving the general methodology and also the details resulting from years of practice in one hypospadias expert center.


Assuntos
Hipospadia , Masculino , Humanos , Lactente , Hipospadia/cirurgia , Uretra/cirurgia , Retalhos Cirúrgicos , Constrição Patológica/cirurgia , Período Pós-Operatório , Procedimentos Cirúrgicos Urológicos Masculinos/métodos
12.
J Clin Endocrinol Metab ; 108(5): e129-e138, 2023 04 13.
Artigo em Inglês | MEDLINE | ID: mdl-36402139

RESUMO

CONTEXT: Determination of steroid levels in the amniotic fluid gives some insight on fetal adrenal and gonadal functions. OBJECTIVE: Our objectives were to establish reference ranges of 12 steroid levels throughout pregnancy and to compare them with steroid levels from pregnancies with fetuses presenting with 21-hydroxylase deficiency (21OHD). METHODS: Liquid chromatography coupled with tandem mass spectrometry (LC-MS/MS) was applied to 145 "control" amniotic fluid samples from gynecology activity (12 + 6 to 32 + 4 gestational weeks, GW). The following steroids were analyzed according to gestational age and compared to 23 amniotic fluid samples from fetuses with classic 21OHD confirmed by molecular studies: delta-4-androstenedione (D4), dehydroepiandrosterone (DHEA), 17-hydroxyprogesterone (17OHP), 11-deoxycortisol (11OH), 21-deoxycortisol (21OH), corticosterone, deoxycorticosterone (DOC), testosterone, pregnenolone, 17-hydroxypregnenolone (17Pregn), cortisol, and cortisone. Chromosomal sex was determined by karyotype and gestational age by biometric measurements. RESULTS: Analysis of control samples showed a statistically significant difference for D4 and testosterone levels according to fetal sex. Cortisol, corticosterone, and DOC had lower concentrations before 20 GW than after 20 GW, whereas 17Pregn and pregnenolone had higher concentrations before 20 GW. This allowed us to establish age- and sex-dependent reference values. We observed higher 21OH, 17Pregn, D4, and testosterone levels in females with 21OHD than female controls. The ratios 17OHP/17Pregn, D4/DHEA, and 11OH/17OHP appeared discriminant for the diagnosis of 21OHD. CONCLUSION: Our study provides information on fetal steroidogenesis and suggests reference values for 12 steroids during pregnancy. This allows a prenatal diagnosis of 21OHD within 24 hours and might be useful in the diagnosis of other variations of sex development.


Assuntos
Corticosterona , Hidrocortisona , Gravidez , Humanos , Feminino , Hidrocortisona/análise , Valores de Referência , Líquido Amniótico/química , Cromatografia Líquida/métodos , Espectrometria de Massas em Tandem , Esteroides/análise , 17-alfa-Hidroxiprogesterona/análise , Testosterona/análise , Pregnenolona , Desidroepiandrosterona
13.
Endocr Connect ; 12(3)2023 Mar 01.
Artigo em Inglês | MEDLINE | ID: mdl-36606580

RESUMO

Objectives: To examine the changes in diagnostic practices and clinical management of patients with 5α-reductase type 2 (SRD5A2) or 17ß-hydroxysteroid dehydrogenase type 3 (HSD17B3) deficiency since molecular diagnoses became available. Methods: Clinical, laboratory, and therapeutic data were retrieved from the medical records of 52 patients with a molecular diagnosis of SRD5A2 (n = 31) or HSD17B3 (n = 21) deficiency. Temporal trends regarding age at assessment and initial sex assignment over 1994-2020 were qualitatively analyzed. Age at molecular diagnosis was compared between two subgroups of patients according to their year of birth. Results: Fifty-eight percent (n = 30) patients were diagnosed during the perinatal period, 33% (n = 17) during infancy, and 9% (n = 5) during adolescence or adulthood. Over the studied period, the patients' age at initial assessment and diagnosis frankly decreased. The median (range) age at diagnostic confirmation was 10.5 (0-53.2) years for patients born before 2007 and 0.4 (0-9.3) years for those born in 2007 or later (P = 0.029). Genetic testing identified 27 different variants for the SRD5A2 gene (30% novel, n = 8) and 18 for the HSD17B3 gene (44% novel, n = 8). Before 2002, most patients were initially assigned as females (95%, n = 19), but this proportion dropped for those born later (44%, n = 14; P < 0.001). The influence of initial genital appearance on these decisions seemingly decreased in the most recent years. Therapeutic interventions differed according to the sex of rearing. Ten percent (n = 2) patients requested female-to-male reassignment during adulthood. Conclusion: This study showed, over the past two decades, a clear trend toward earlier diagnosis and assignment of affected newborns as males.

14.
J Urol ; 188(3): 717-23, 2012 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-22818132

RESUMO

PURPOSE: We describe the outcomes of undescended testes and sex development disorders in adolescence and young adulthood. We reviewed the requirements for the long-term care of children born with these and other major congenital anomalies of the genitourinary system. MATERIALS AND METHODS: The current English language literature was retrieved with a PubMed® search for articles on these subjects. Only articles covering outcomes at ages past puberty were included in analysis. The material was supplemented from the database of the clinic for adults with sex development disorders at University College London Hospitals. RESULTS: An undescended testis has impaired spermatogenesis. In men in whom a unilateral undescended testis was corrected before puberty the incidence of paternity is normal at around 90% of those who attempt it. The equivalent rate for those with bilateral undescended testes is about 65%. If surgery for bilateral undescended testes is delayed until after puberty, fertility is unlikely. The risk of testicular neoplasms is overestimated and the relative risk is between 2.5 and 8. Children born with a sex development disorder receive multidisciplinary treatment throughout childhood and require the same care as adults. Males who are under virilized likely have a micropenis (greater than 2 SD below the mean stretched length) but they may have normal sexual function. Fertility depends on the underlying condition. Virilized females, who most commonly have congenital adrenal hyperplasia, currently present to adult clinics with an inadequate vagina after infantile surgery. Reconstruction is required to allow intercourse. CONCLUSIONS: The care of adults born with abnormalities of the genitalia is complex. Early management may define upbringing in childhood but requirements for sexuality and fertility in adult life are different. Multidisciplinary care is essential and a case can be made to establish a subspecialty of urology to coordinate it.


Assuntos
Criptorquidismo/terapia , Transtornos do Desenvolvimento Sexual/terapia , Transição para Assistência do Adulto , Adolescente , Criança , Feminino , Humanos , Masculino , Resultado do Tratamento , Adulto Jovem
15.
Curr Opin Urol ; 21(6): 465-9, 2011 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-21826002

RESUMO

PURPOSE OF REVIEW: To list the main questions and dilemmas raised by hypospadias management and its long-term evaluation. RECENT FINDINGS: Criteria of evaluation of hypospadias have changed in terms of anatomical description and biological screening. A better understanding of the causative and epidemiological aspects of the development anomalies of the genital tubercle has led to a more adjusted biological and surgical approach. The place of preoperative biological screening, preoperative hormonal stimulation and the choice of urethroplasty are the three main fields of discussion between hypospadiologists. Evaluation of outcome is the critical point as there are no current consensual protocols, and long-term results are often lacking as well as psychological evaluation. SUMMARY: Efforts are made to coordinate the management and evaluation of disorders of sex development (DSD) patients and more specifically hypospadias patients. National, European (EuroDSD) and international (consensus conferences) aim at finding common tracks to improve the quality of treatment and follow-up.


Assuntos
Hipospadia/cirurgia , Procedimentos Cirúrgicos Urológicos Masculinos , Humanos , Hipospadia/diagnóstico , Hipospadia/epidemiologia , Masculino , Fatores de Tempo , Resultado do Tratamento , Procedimentos Cirúrgicos Urológicos Masculinos/efeitos adversos
16.
PLoS One ; 16(4): e0249800, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-33857192

RESUMO

Hypospadias (H) is a common birth defect affecting the male urinary tract. It has been suggested that exposure to endocrine disrupting chemicals might increase the risk of H by altering urethral development. However, whether H risk is increased in places heavily exposed to agricultural pesticides, such as vineyards, remains debated and difficult to ascertain. The objective of the work is to test the possible association of H with residential proximity to vineyards. Residential address at birth of 8,766 H cases born 1980-2011 was taken from 17 specialized surgery centers. The geographical distribution of vineyards was obtained from the European Land Parcel Identification System (LPIS) and the distance of address to the nearest vineyard was computed. A first estimate of the variation of H relative risk with distance to vineyards was obtained using as controls 13,105 cryptorchidism (C) cases operated during the same period in the same centers. A separate estimate was obtained from a case-control study using "virtual controls" (VC) defined as points of the map sampled to match the demographic distribution of births within the recruitment territories of the study centers. Non-exposed patients were defined as those with a residence between 5,000 and 10,000 m from the closest vineyard. The residential distance to vineyard was smaller for H than for C cases (p<10-4). We found 42/8766 H cases (0.48%) and 50/13,105 C cases (0.38%) born to mothers living within 20 m of a vineyard. The odds ratios for H were 2.48 (CI: 1.0 to 5.1) and 2.4 (CI: 1.3 to 4.4), vs C or vs VC, respectively, when pregnant mothers lived 10-20 m from a vineyard. In conclusion, our study supports that children born to mothers living close to a vineyard have a two-fold increased risk of H. For environmental research, the use of VC provides an alternative to classical case control technique.


Assuntos
Agricultura/métodos , Disruptores Endócrinos/efeitos adversos , Fazendas/estatística & dados numéricos , Hipospadia/epidemiologia , Exposição Materna/efeitos adversos , Praguicidas/efeitos adversos , Efeitos Tardios da Exposição Pré-Natal/induzido quimicamente , Estudos de Casos e Controles , Exposição Ambiental , Feminino , França/epidemiologia , Humanos , Hipospadia/etiologia , Hipospadia/patologia , Masculino , Gravidez , Fatores de Risco
17.
Arch Esp Urol ; 63(7): 495-504, 2010 Sep.
Artigo em Inglês, Espanhol | MEDLINE | ID: mdl-20876944

RESUMO

Disorders of Sex Development (DSD) remain a fascinating challenge for the paediatricians, endocrinologists, biologists, psychiatrists, geneticists, radiologists, surgeons and for the whole society. This article aims at highlighting the current controversies and questions met with genital reconstruction in children born with abnormal genitalia. The main current techniques of masculinization and feminization are reviewed with their progress and their problems. The tools of decision used to assign a gender in some newborns with complex DSD are discussed showing that at the dawn of the third millenium, one still does not know why a boy is a boy, and a girl is a girl.


Assuntos
Transtornos do Desenvolvimento Sexual/cirurgia , Feminino , Humanos , Masculino , Procedimentos Cirúrgicos Urológicos/métodos
18.
J Clin Endocrinol Metab ; 105(7)2020 07 01.
Artigo em Inglês | MEDLINE | ID: mdl-32386308

RESUMO

PURPOSE: Urethral fistula and dehiscence are common after hypospadias surgery. Preoperative androgens have been considered to reduce these complications although this consideration is not evidence-based. Dermatologists have reported the benefits of topical estrogens on skin healing. We investigated whether the preoperative use of topical promestriene could reduce healing complications in hypospadias surgery. Our primary objective was to demonstrate a reduction of healing complications with promestriene vs placebo. Impact on reoperations and other complications, clinical tolerance, bone growth, and biological systemic effects of the treatment were also considered. METHODS: We conducted a prospective, randomized, placebo-controlled, double-blind, parallel group trial between 2011 and 2015 in 4 French centers. One-stage transverse preputial island flap urethroplasty (onlay urethroplasty) was selected for severe hypospadias. Promestriene or placebo was applied on the penis for 2 months prior to surgery. The primary outcome was the presence of postoperative urethral fistula or dehiscence in the first year postsurgery. For safety reasons, hormonal and anatomical screenings were performed. RESULTS: Out of 241 patients who received surgery, 122 patients were randomized to receive placebo, and 119 patients received promestriene. The primary outcome was unavailable for 11 patients. Healing complications were assessed at 16.4% (19/116) in the placebo vs 14.9% (17/114) in the promestriene arm, and the odds ratio adjusted on center was 0.93 (95% confidence interval 0.45-1.94), P = 0.86. CONCLUSIONS AND RELEVANCE: Although we observed an overall lower risk of complications compared to previous publications, postsurgery complications were not different between promestriene and placebo, because of a lack of power of the study or the inefficacy of promestriene.


Assuntos
Estradiol/análogos & derivados , Fístula/prevenção & controle , Hipospadia/cirurgia , Complicações Pós-Operatórias/tratamento farmacológico , Procedimentos Cirúrgicos Urológicos Masculinos/efeitos adversos , Administração Tópica , Método Duplo-Cego , Estradiol/administração & dosagem , Fístula/etiologia , Humanos , Lactente , Masculino , Cuidados Pré-Operatórios , Estudos Prospectivos , Procedimentos de Cirurgia Plástica/efeitos adversos , Deiscência da Ferida Operatória/etiologia , Deiscência da Ferida Operatória/prevenção & controle , Resultado do Tratamento , Doenças Uretrais/etiologia , Doenças Uretrais/prevenção & controle
19.
BJU Int ; 103(10): 1418-21, 2009 May.
Artigo em Inglês | MEDLINE | ID: mdl-19154455

RESUMO

OBJECTIVE: To assess the long-term outcome and cosmetic results of forearm free-flap phalloplasty for correcting micropenis associated with bladder exstrophy in men. PATIENTS AND METHODS: From 1992 to 2000, six men with bladder exstrophy and unsatisfactory penile appearance underwent phalloplasty at our institution. The mean (range) follow-up was 113 (62-153) months. The surgical procedure consisted of a modified radial free-flap phalloplasty and prosthesis implantation. One-stage urethroplasty was electively performed according to the patient's voiding pattern. Early and late complications were recorded and quality of life after phalloplasty was assessed. RESULTS: The mean (range) age was 21.2 (17-26) years and flap survival was 100%. Two incontinent patients underwent immediate perineal urethrostomy. One sepsis occurred, requiring the removal of the penile prosthesis and a cutaneous continent urinary diversion; this patient was the only one to complain about the outcome. Two anastomotic urethral strictures were conservatively treated by endoscopic urethrotomy and only one patient underwent subsequent urethral self-dilatations. Five patients were very satisfied with the cosmesis of the neophallus and three patients reported having regular sexual intercourse. CONCLUSIONS: Although short, this series indicates the low incidence of early complications and the excellent long-term satisfaction with forearm free-flap phalloplasty in bladder exstrophy. Phalloplasty has potential indications when adult exstrophy patients are dissatisfied with the cosmetic appearance of their external genitalia or have unsatisfactory sexual intercourse.


Assuntos
Extrofia Vesical/complicações , Pênis/cirurgia , Procedimentos de Cirurgia Plástica/métodos , Transplante de Pele/métodos , Retalhos Cirúrgicos , Adulto , Extrofia Vesical/cirurgia , Coito , Antebraço , Humanos , Masculino , Satisfação do Paciente , Prótese de Pênis , Pênis/anormalidades , Resultado do Tratamento , Adulto Jovem
20.
Paediatr Anaesth ; 19(4): 313-9, 2009 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-19335344

RESUMO

Allergic or immediate hypersensitivity reactions to latex have been reported in children with increasing frequency in the past. The reported prevalence varies greatly depending upon the population studied and the methods used to detect sensitization. Children's subpopulations at particular risk include: atopics, individuals with spina bifida, children undergoing surgical procedure during the neonatal period and individuals who required frequent surgical instrumentations. Latex allergy is also an important medical issue, particularly for healthcare personnel. Sensitization mainly occurs by wound or mucosal contact with latex devices during surgery or by inhalation of airborne allergens released from powdered latex gloves. Regarding diagnosis, the medical history, skin prick test and search for specific serum IgE are crucial but cost effective. The development of a guide listing latex-containing drugs is essential for the primary prevention of allergic reactions. Immunotherapy or specific premedication seems not effective in preventing the risk of anaphylaxis during the perioperative course. The most effective strategy to decrease the incidence of latex sensitization is complete avoidance. This strategy is efficient in patients and also in health care workers and has been applied since 2002 in our pediatric surgical hospital. One of major problem with the latex-free gloves was that surgeons find them considerably more difficult to work with. But today, manufacturers made considerable effort and free-latex gloves with an equal tactile sensation than the latex-gloves are now available. The extra cost of free latex gloves is well counterbalanced as allergen test, long stay hospital for allergic reaction, and worker's compensation are no longer needed. Since the introduction of this program in our institution, no allergic reaction to latex has been reported in 25000 anesthetized children or with the health care workers.


Assuntos
Hipersensibilidade ao Látex/imunologia , Hipersensibilidade ao Látex/prevenção & controle , Criança , Humanos , Látex/imunologia , Hipersensibilidade ao Látex/epidemiologia , Salas Cirúrgicas , Assistência Perioperatória , Medição de Risco
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