FRANCISCO TIBOR DENES
Projetos de Pesquisa
Unidades Organizacionais
Instituto Central, Hospital das Clínicas, Faculdade de Medicina
LIM/55 - Laboratório de Urologia, Hospital das Clínicas, Faculdade de Medicina - Líder
LIM/55 - Laboratório de Urologia, Hospital das Clínicas, Faculdade de Medicina - Líder
14 resultados
Resultados de Busca
Agora exibindo 1 - 10 de 14
- 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, MiguelIntroduction: 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.
- Retrospective Analysis of Prognostic Factors in Pediatric Patients with Adrenocortical Tumor from Unique Tertiary Center with Long-Term Follow-Up(2022) BACHEGA, Fernanda S.; V, Caio Suartz; ALMEIDA, Madson Q.; BRONDANI, Vania B.; CHARCHAR, Helaine L. S.; LACOMBE, Amanda M. F.; MARTINS-FILHO, Sebastiao N.; SOARES, Ibere C.; ZERBINI, Maria Claudia N.; DENES, Francisco T.; MENDONCA, Berenice; I, Roberto Lopes; LATRONICO, Ana Claudia; V, Maria Candida B. FragosoPediatric adrenocortical tumors (PACTs) represent rare causes of malignancies. However, the south/southeast regions of Brazil are known to have a high incidence of PACTs because of the founder effect associated with a germline pathogenic variant of tumor suppressor gene TP53. We aimed to retrospectively analyze the types of variables among hormone production, radiological imaging, tumor staging, histological and genetic features that were associated with the occurrence of malignancy in 95 patients (71% females) with PACTs from a unique center. The worst prognosis was associated with those aged > 3 years (p < 0.05), high serum levels of 11-desoxicortisol (p < 0.001), tumor weight >= 200 g (p < 0.001), tumor size >= 5 cm (p < 0.05), Weiss score >= 5 (p < 0.05), Wieneke index >= 3 (p < 0.001) and Ki67 >= 15% (p < 0.05). Furthermore, patients with MacFarlane stage IV had an overall survival rate almost two times shorter than patients with other stages (p < 0.001). Additionally, the subtractions of BUB1B-PINK1 (<6.95) expression (p < 0.05) and IGF-IR overexpression (p = 0.0001) were associated with malignant behavior. These results helped identify patients who are likely to have an aggressive course; further multicenter prospective studies are required to confirm our results. In conclusion, PACTs with these patterns of prognostic factors could be treated using an adjuvant approach that may improve the overall survival in such patients.
- High Prevalence of Alterations in DNA Mismatch Repair Genes of Lynch Syndrome in Pediatric Patients with Adrenocortical Tumors Carrying a Germline Mutation on TP53(2020) BRONDANI, Vania Balderrama; MONTENEGRO, Luciana; LACOMBE, Amanda Meneses Ferreira; MAGALHAES, Breno Marchiori; NISHI, Mirian Yumie; FUNARI, Mariana Ferreira de Assis; NARCIZO, Amanda de Moraes; CARDOSO, Lais Cavalca; SIQUEIRA, Sheila Aparecida Coelho; ZERBINI, Maria Claudia Nogueira; DENES, Francisco Tibor; LATRONICO, Ana Claudia; MENDONCA, Berenice Bilharinho; ALMEIDA, Madson Queiroz; LERARIO, Antonio Marcondes; SOARES, Ibere Cauduro; FRAGOSO, Maria Candida Barisson VillaresAdrenocortical cancer is a rare malignant neoplasm associated with a dismal prognosis. Identification of the molecular pathways involved in adrenal tumorigenesis is essential for a better understanding of the disease mechanism and improvement of its treatment. The aim of this study is to define the prevalence of alterations in DNA mismatch repair (MMR) genes in Lynch syndrome among pediatric patients with adrenocortical neoplasia from southern Brazil, where the prevalence of a specific TP53 germline mutation (p.Arg337His) is quite high. Thirty-six pediatric patients were retrospectively evaluated. Immunohistochemistry (IHC) for theMMR enzymes MLH1, MSH2, MSH6, and PMS2, as well as next-generation sequencing (NGS) were performed. For IHC, 36 pediatric tumors were tested. In all of them, the expression of all evaluated MMR proteins was well-preserved. For NGS, 35 patients with pediatric tumor were tested. Three patients (8.57%) with the TP53 p.Arg337His germline mutation presented pathogenic and likely pathogenic variants in the MMR genes (two in MLH1 and one in MSH6). The prevalence of alteredMMR genes among pediatric patients was elevated (8.57%) and higher than in colorectal and endometrial cancer cohorts. Pediatric patients with adrenocortical tumors should, thus, be strongly considered as at genetic risk for Lynch syndrome.
- Transperitoneal laparoscopic pyeloplasty in children: does upper urinary tract anomalies affect surgical outcomes?(2018) BRUNHARA, Joao Arthur; MOSCARDI, Paulo Renato Marcelo; MELLO, Marcos Figueiredo; ANDRADE, Hiury Silva; CARVALHO, Paulo Afonso de; CEZARINO, Bruno Nicolino; DENES, Francisco Tibor; LOPES, Roberto IglesiasObjective: To assess the feasibility and outcomes of laparoscopic pyeloplasty in children with complex ureteropelvic junction obstruction (UPJO) and compare to children with iso-lated UPJO without associated urinary tract abnormalities. Material and Methods: Medical records of 82 consecutive children submitted to trans-peritoneal laparoscopic pyeloplasty in a 12-year period were reviewed. Eleven cases were con-sidered complex, consisting of atypical anatomy including horseshoe kidneys in 6 patients, pelvic kidneys in 3 patients, and a duplex collecting system in 2 patients. Patients were di-vided into 2 groups: normal anatomy (group 1) and complex cases (group 2). Demographics, perioperative data, outcomes and complications were recorded and analyzed. Results: Mean age was 8.9 years (0.5-17.9) for group 1 and 5.9 years (0.5-17.2) for group 2, p=0.08. The median operative time was 200 minutes (180-230) for group 1 and 203 minutes (120-300) for group 2, p=0.15. Major complications (Clavien =3) were 4 (5.6%) in group 1 and 1 (6.3%) in group 2, p=0.52. No deaths or early postoperative complications such as: urinoma or urinary leakage or bleeding, occurred. The success rate for radiologic improvement and flank pain improvement was comparable between the two groups. Re-garding hydronephrosis, significant improvement was present in 62 patients (93.4%) of group 1 and 10 cases (90.9%) of group 2, p=0.99. The median hospital stay was 4 days (IQR 3-4) for group 1 and 4.8 days (IQR 3-6) for group 2, p=0.27. Conclusions: Transperitoneal laparoscopic pyeloplasty is feasible and effective for the management of UPJO associated with renal or urinary tract anomalies.
- Surgical Treatment after Failed Primary Correction of Urogenital Sinus in Female Patients with Virilizing Congenital Adrenal Hyperplasia: Are Good Results Possible?(2016) SIRCILI, Maria Helena Palma; BACHEGA, Tania Sartori Sanchez; MADUREIRA, Guiomar; GOMES, Larissa; MENDONCA, Berenice Bilharinho; DENES, Francisco TiborPurpose: Genital reconstruction in female patients with virilizing congenital adrenal hyperplasia (CAH) is very challenging. Our aim was to evaluate the techniques employed to treat complications after failure of primary urogenital sinus (UGS) surgery, as well as the result of these reoperations. Patients and methods: Twenty girls with virilizing CAH who were previously submitted to genitoplasty in our service and elsewhere had recurrent UGS stenosis and vaginal introitus stenosis that required surgical treatment. The main symptoms were recurrent urinary tract infection (UTI) in nine, dyspareunia in six, and hematocolpos in three (two associated with sepsis). The anatomical findings were the persistence of UGS with stenosis in 17 patients and vaginal introitus stenosis in 3. The mean age at procedure was 15.2 years, averaging 13.1 years after the first surgery. The surgical techniques employed were isolated perineal flap in 17 patients and perineal flap with partial mobilization of UGS in 3. The mean follow-up after the procedure was 4.8 years (varying from 1 to 17 years). Results: Vaginal dilations were performed after surgery in 15 patients. Good functional and anatomical results were obtained in 15 patients, with vaginal introitus amenable to dilators of 3.0 cm in diameter. Five patients with high vaginal insertion had recurrent vaginal stenosis and required a surgical revision. No patients presented menstrual obstruction or UTI after surgery. Eight of the 15 adult patients are sexually active. Conclusion: The reoperation to treat failed primary UGS treatment using Y-V flap and partial mobilization techniques associated with vaginal dilations, promoted good anatomical, and functional results with low morbidity in 75% of the patients.
- Malignant paraganglioma in children treated with embolization prior to surgical excision(2016) MIRANDA, Eduardo de Paula; LOPES, Roberto Iglesias; PADOVANI, Guilherme Philomeno; MOSCARDI, Paulo Renato Marcelo; NISHIMURA, Fernanda Gardini Maciel; MENDONCA, Berenice Bilharinho de; CARNEVALE, Francisco Cesar; CRISTOFANI, Lilian Maria; DUARTE, Ricardo Jordao; SROUGI, Miguel; DENES, Francisco TiborBackground: Paragangliomas (PGL) are rare tumors derived from neural crest cells, whose origins may vary along the chain of the sympathetic nervous system. Such tumors are often characterized by secretion of catecholamines, but sometimes they are biochemically inactive, which makes diagnosis often challenging. Malignant paraganglioma is defined by the presence of this tumor at sites where chromaffin cells are usually not found or by local invasion of the primary tumor. Recurrence, either regional or metastatic, usually occurs within 5 years of the initial complete resection but long-term recurrence is also described. Malignancy is often linked to a SDHB mutation. Preoperative embolization has been applied in the surgical management of PGLs with the objective to decrease intra-operative blood loss and surgery length without complications. Case Presentation: We report two cases of patients with abdominal or pelvic malignant PGLs who have been treated surgically at our center after preoperative embolization. Surgery was a very challenging procedure with multiple surgical teams involved and embolization did not prevent major blood loss and intraoperative complications. Patients required adjuvant treatment with either chemotherapy or radiotherapy. Conclusions: Many studies in the adult population have established recommendations for the diagnosis and therapeutic management of PGL, but few studies concern the pediatric population. Because malignant PGL is more important in the pediatric population, screening and early diagnosis of PGL is advisable in children with genetic predisposing. Surgical resection is the mainstay of treatment, but a multimodal approach is often required due to the complexity of cases. The role of preoperative embolization is not established and in our experience it has provided little benefit and major complications.
- MMP9 overexpression is associated with good surgical outcome in children with UPJO: Preliminary results(2016) REIS, Sabrina Thalita; LEITE, Katia R. M.; VIANA, Nayara Izabel; LOPES, Roberto Iglesias; MOURA, Caio Martins; IVANOVIC, Renato F.; MACHADO, Marcos; DENES, Francisco Tibor; GIRON, Amilcar; NAHAS, William Carlos; SROUGI, Miguel; PASSEROTTI, Carlo C.Background: Ureteropelvic junction obstruction (UPJO) diagnosed prenatally occurs in 1: 150 -1: 1200 pregnancies. Although many studies investigating the molecular changes of this obstructed segment have been performed, the underlying mechanisms are still unclear. The role of extracellular matrix (ECM) components remains controversial, and the investigations in the field of ECM changes, might help the better understanding of the pathogenesis of this common condition. The aim of the present study was to investigate for the first time in the literature whether MMP9 and its specific inhibitors, TIMP1 and RECK, are expressed in a reproducible, specific pattern in UPJ. Methods: UPJO specimens were obtained from 16 children at the time of dismembered pyeloplasty due to intrinsic UPJ stenosis. Expression levels of the three genes (MMP9, TIMP1 and RECK) were analyzed by quantitative real-time polymerase chain reaction (qRT-PCR). Then correlated the expression levels of the genes according to grade study population that was divided in 2 categories according to Society of Fetal Urology classification, grade 3 (moderate) and 4 (severe). For DTPA we subdivided the childrens in 2 groups, obstructive (T 1/2 more than 20 min) and partial obstructive (T 1/2 between 10 and 20 min) and success in a surgery was defined as decrease in T 1/ 2 to less than 20 min, absence of symptoms, improving renal function and decreasing dilatation on successive exams. Results: MMP9 was underexpressed and TIMP1 and RECK were overexpressed in children with obstructive DTPA but the differences were not statistically significant. Overexpression of MMP9 was higher among patients with severe grade of UPJ compared to those with moderate grade. Surprisingly expression levels of MMP-9 was three times higher in children who were successfully treated by surgery (n = 10) (p = 0.072), so those who were followed for at least 1 year after surgery and remained with improvement in renal function and decreasing dilation on intravenous urogram and TIMP-1 was underexpressed in 100 % of this cases (p = 0.00). Conclusions: We showed an increase in expression of MMP9 and a decrease in expression of TIMP1 in children who improving renal function and decreasing dilation after surgery. We believe that the higher expression of MMP9 in these cases can reflect an increase in degradation and remodeling process that could be used as a marker for surgical outcome.
- Pediatric genitourinary oncology(2013) DENES, Francisco Tibor; DUARTE, Ricardo Jordao; CRISTOFANI, Lilian Maria; LOPES, Roberto IglesiasTumors of the kidney, bladder, prostate, testis, and adrenal represent a large part of the adult urologic practice, but are relatively infrequent in children. The natural history and management of these tumors in the pediatric age is different from that of the adults. As result of the successful work of several clinical trial groups in recent decades, there has been a significant improvement in their cure rates. The aim of this article is to review their most significant clinical aspects, as well as to present an update in their management.
- Quality of life in young adult patients treated for bladder exstrophy(2016) CRUZ, Jose Arnaldo Shiomi da; MATTOS, Bruno de; SROUGI, Miguel; NGUYEN, Hiep; BONAN, Rafael; DENES, Francisco; GIRON, Amilcar; PASSEROTTI, CarloIntroduction Bladder exstrophy (BE) is a rare condition that requires complex surgical corrections to achieve the goals of bladder functionality, normal sexual function, continence, and finally cosmesis. The purpose of this study was to identify clinical parameters that predict better quality of life (QOL) scores using a validated questionnaire (SF-36) with young adults after completing surgical reconstruction. Material and methods Forty-three young adults (mean age 22.35 years, 29 men and 14 women) treated for BE were evaluated using the Short Form 36 general health questionnaire (SF-36). Clinical assessment involved evaluation of the actual condition regarding continence, sexual function, genital satisfaction and overall cosmesis. Results Both genders presented similar QOL scores (p = 0.36). The QOL was not age-related (p = 0.63). Neither genders did not present any differences in the number of procedures (p = 0.27). Although no significant gender difference was found, clinical impairments -such as urinary fistula, incontinence, penile length and infertility - were associated with worse QOL scores and were male-related (p<0.01). The most common complaint after complete surgical repair was about penile length (26/29 patients, 89.6%). Conclusions Age and gender were not predictors of better QOL scores. Any clinical impairment, such as urinary leakage due to incontinence or fistula, penile length and infertility, tended to significantly decrease the overall QOL in male patients with BE. The male genitalia seems to be the most troublesome aspect post-adolescence in treated male patients with exstrophy-epispadias. It has an important impact on the overall QOL, mainly having a social affect on those patients.
- Prune-Belly Syndrome(2023) DéNES, F. T.; CALDAMONE, A. A.Prune-belly syndrome has three main features: urinary tract dilation, cryptorchidism, and abdominal wall flaccidity, with potential associated pulmonary, cardiac, gastrointestinal, and musculoskeletal abnormalities. It has three categories of presentation according to the severity of the malformations. Category 1 patients have the most severe nephro-urological abnormalities, with associated pulmonary hypoplasia, and rarely survive the neonatal period. Category III patients have scant uro-nephrological abnormalities and only require orchidopexy and potentially abdominoplasty. Up to 30% of patients with category II syndrome, despite initial normal renal function, develop chronic renal failure during childhood or adolescence due to urinary tract obstruction and infection. In order to prevent late renal insufficiency in this group, continuous urinary tract monitoring and antibiotic prophylaxis are necessary in these patients. Upper urinary tract reconstruction is indicated when there is concern of progressive functional or anatomical deterioration due to obstruction or high-grade vesicoureteral reflux (VUR), with or without recurrent upper tract infections. Abdominoplasty improves body posture, as well as bladder emptying, cough, and defecation in some patient series. Orchidopexy in one or two stages is required in all patients of categories II and III, being best performed early in life. Comprehensive surgery that includes urinary tract reconstruction, orchidopexy, and abdominoplasty is feasible and has good results in properly selected patients. With individualization of care and improvements in the surgical management, the overall outlook for survival and quality of life of these patients has improved considerably. © Springer-Verlag GmbH Germany, part of Springer Nature 2023.