FRANCISCO TIBOR DENES

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Instituto Central, Hospital das Clínicas, Faculdade de Medicina
LIM/55 - Laboratório de Urologia, Hospital das Clínicas, Faculdade de Medicina - Líder

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  • article 75 Citação(ões) na Scopus
    46,XY disorder of sex development (DSD) due to 17 beta-hydroxysteroid dehydrogenase type 3 deficiency
    (2017) MENDONCA, Berenice B.; GOMES, Nathalia Lisboa; COSTA, Elaine M. F.; INACIO, Marlene; MARTIN, Regina M.; NISHI, Mirian Y.; CARVALHO, Filomena Marino; TIBOR, Francisco Denes; DOMENICE, Sorahia
    17 beta-hydroxysteroid dehydrogenase 3 deficiency consists of a defect in the last phase of steroidogenesis, in which androstenedione is converted into testosterone and estrone into estradiol. External genitalia range from female-like to atypical genitalia and most affected males are raised as females. Virilization in subjects with 17 beta-HSD3 deficiency occurs at the time of puberty and several of them change to male social sex. In male social sex patients, testes can be safely maintained, as long as they are positioned inside the scrotum The phenotype of 46,XY DSD due to 17 beta-HSD3 deficiency is extremely variable and clinically indistinguishable from other causes of 46,XY DSD such as partial androgen insensitivity syndrome and 5 alpha-reductase 2 deficiency. Laboratory diagnosis is based on a low testosterone/androstenedione ratio due to high serum levels of androstenedione and low levels of testosterone. The disorder is caused by a homozygous or compound heterozygous mutations in the HSD17B3 gene that encodes the 17 beta-HSD3 isoenzyme leading to an impairment of the conversion of 17-keto into 17-hydroxysteroids. Molecular genetic testing confirms the diagnosis and provides the orientation for genetic counseling. Our proposal in this article is to review-the previously reported cases of 17 beta-HSD3 deficiency adding our own cases. (C) 2016 Published by Elsevier Ltd.
  • article 11 Citação(ões) na Scopus
    Urethral duplication type influences on the complications rate and number of surgical procedures
    (2017) LOPES, Roberto Iglesias; GIRON, Amilcar Martins; MELLO, Marcos Figueiredo; BARBOSA NETO, Cristovao Machado; SANTOS, Joana dos; MOSCARDI, Paulo Renato Marcelo; SROUGI, Victor; DENES, Francisco Tibor; SROUGI, Miguel
    Introduction: Urethral duplication is rare. Characterized by the presence of two urethral channels. This anomaly presents a great variety of clinical findings that depend on the type of duplication that often is associated with other anomalies. Material and Methods: We report thirteen boys with urethral duplication managed in our institution between 1988-02015. Clinical findings, associated anomalies, treatment of urethral duplication and our results are described. Patients were classified according to Effmann classification. Results: Mean patient's age was 38.3 +/- 34.7 months (3-0136 months). Mean follow-up was 7.7 +/- 3.4 years (3y8m-014y2m). Type II A2 was the most common pattern (8/13 patients, 61.5%), followed by type IA (3/13 patients, 23%) and IIA1 (2/13 patients, 15.3%). The most frequent clinical manifestations were urinary tract infections (UTI) observed in 11/13 patients (84.6%) and anal urinary leakage, found in 7/13 patients (53.8%). Associated anomalies were found in 9/13 patients (69.2%). Required surgeries were 3.53 +/- 2.84 procedures per patient. Considering groups: Type IIA2 4.25 +/- 3.28, type IIA1 4 +/- 1.41 and type IA 1.33 +/- 0.57 needed procedures per patient. Complications rate were 0% for type IA, 50% for type IIA1 and 75% for type IIA2. Conclusions: Patients with incomplete duplication (type I A or I B) can totally be asymptomatic, with no need of surgical correction. Type IIA2 is the most complex form of duplication to correct and multiple procedures might be required because of the very hypoplastic orthotopic dorsal urethral tissue. Surgical treatment should be individualized and parents should be advised on complications and need of multiple surgeries according to urethral duplication type.
  • article 21 Citação(ões) na Scopus
    Videolaparoscopic radical nephrectomy after chemotherapy in the treatment of Wilms' tumor: Long-term results of a pioneer group
    (2017) DUARTE, Ricardo Jordao; CRISTOFANI, Lilian Maria; ODONE FILHO, Vicente; SROUGI, Miguel; DENES, Francisco Tibor
    Introduction A high cure rate for Wilms' tumor has been achieved using a multidisciplinary approach. The natural step forward is to offer the benefits of a minimally invasive technique for surgery, which is an obligatory part of treatment. Nevertheless, some authors resist using videolaparoscopic radical nephrectomy (VRN) because of concerns about reducing the cure index. Methods The present study included children with unilateral Wilms' tumor treated from December 2003 to December 2015 with neoadjuvant chemotherapy followed by VRN. Patients were selected based on the size of their tumors compared with the contralateral kidney, and on their stature. Results VRN was performed in 24 patients of age range 10-93 months, with an average of 38.04 +/- 23.37 months. The tumoral kidney's largest diameter after chemotherapy averaged 10% of a patient's height. There was no tumor rupture or spillage and no patient presented intra or immediate postoperative complications, except for prolonged ileum in two patients. One patient required intraoperative transfusion because of preoperative anemia. Another developed a late herniation in the umbilical port that required surgical correction. After an average of 6.65 years of follow-up, two patients presented relapse: one with a stage IV disease had relapse in the lung and another with a stage III, involving the liver, had local relapse because of an unwanted delay in the adjuvant treatment. Conclusion VRN can be considered a feasible alternative to open surgery in selected cases of children with Wilms' tumor. The present experience shows that besides the benefits of minimally invasive procedures and better cosmetic results, there is no evidence of increased tumor rupture or spillage, peritoneal or port site metastasis, and the long-term oncological results are the same as open procedures.
  • article 11 Citação(ões) na Scopus
  • article 22 Citação(ões) na Scopus
    Reoperative Laparoscopic Ureteropelvic Junction Obstruction Repair in Children: Safety and Efficacy of the Technique
    (2017) MOSCARDI, Paulo Renato Marcelo; BARBOSA, Joao Arthur Brunhara Alves; ANDRADE, Hiury Silva; MELLO, Marcos Figueiredo; CEZARINO, Bruno Nicolino; OLIVEIRA, Lorena Marcalo; SROUGI, Miguel; DENES, Francisco Tibor; LOPES, Roberto Iglesias
    Purpose: Failure after pyeloplasty for ureteropelvic junction obstruction in children may occur in up to 10% of cases. Therapeutic options include Double-J (R) stent placement, endoscopic treatment and reoperation. Laparoscopic and robotic reoperative modalities seem safe and efficacious, although pediatric series are limited in the literature. We report the largest known series of reoperative laparoscopic ureteropelvic junction obstruction repair in children and compare this approach to primary laparoscopic pyeloplasty. Materials and Methods: We reviewed all children undergoing laparoscopic pyeloplasty at a single institution from 2004 to 2015. Reoperative laparoscopic ureteropelvic junction obstruction repair was compared to primary pyeloplasty. Groups were analyzed regarding demographics, operative time, complications, length of hospital stay and success, defined by improvement of symptoms, ultrasound and renogram. Results: We identified 11 cases of reoperation (8 redo pyeloplasties and 3 ureterocalycostomies) and 71 primary pyeloplasties. Groups were not different in age, gender or weight. Median followup was 37 months. Median time between primary pyeloplasty and reoperation was 34 months. Median operative time was 205 minutes for the reoperative group and 200 for primary pyeloplasty (p = 0.98). Length of stay was longer in the reoperative group (p = 0.049), although no major complications were recorded in this group. All reoperative cases and 96% of primary pyeloplasty cases remained asymptomatic following surgery (p = 0.99). Postoperative improvement was similar for both groups on ultrasound (90% for reoperation vs 92% for primary pyeloplasty, p = 0.99) and renogram (80% vs 88%, p = 0.6). Conclusions: Laparoscopy seems to be safe and effective for management of failed pyeloplasty in children. Based on our data, reoperation is as safe and effective as primary pyeloplasty.
  • conferenceObject
    GONADAL FUNCTION AND REPRODUCTIVE SYSTEM ANATOMY IN POST PUBERAL PRUNE BELLY SYNDROME PATIENTS
    (2017) TIBOR-DENES, Francisco; TAVARES, Alessandro; COCUZZA, Marcello; TISEU, Bruno; MACHADO, Marcos Gianetti; GIRON, Amilcar Martins; SROUGI, Miguel