ZEIN MOHAMED SAMMOUR
Projetos de Pesquisa
Unidades Organizacionais
Instituto Central, Hospital das Clínicas, Faculdade de Medicina
7 resultados
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- Lower urinary tract symptoms in children and adolescents with Williams-Beuren syndrome(2017) SAMMOUR, Z. M.; BESSA JR., J. de; HISANO, M.; BRUSCHINI, H.; KIM, C. A.; SROUGI, M.; GOMES, C. M.Introduction Williams-Beuren syndrome (WBS) is a genetic condition caused by a microscopic deletion in the chromosome band 7q11.23. Individuals with WBS may present with congenital cardiovascular defects, neurodevelopmental disturbances and structural abnormalities of the urinary tract. Lower urinary tract symptoms (LUTS) seem to be frequent in this population, but studies on this topic are scarce and based on small case series. Objective To systematically evaluate the prevalence of lower urinary tract symptoms (LUTS) and the acquisition of bladder control in a large population with WBS. Study design A cross-sectional study evaluating 87 consecutive patients with WBS; there were 41 girls and 46 boys. Genetic studies confirmed WBS in all patients. Subjects were clinically evaluated with: a history of LUTS obtained from the parents and child, a structured questionnaire of LUTS, a 3-day urinary frequency-volume chart, a quality of life question regarding LUTS, and physical examination. A history regarding the acquisition of bladder control was directly evaluated from the parents. Results Mean age of patients was 9.0 +/- 4.2 years, ranging from 3 to 19 years. Based on the symptoms questionnaire and the frequency-volume chart, 70 patients (80.5%) were symptomatic. The most common symptom was urgency, affecting 61 (70.1%) patients, followed by increased urinary frequency in 60 (68.9%) patients, and urge-incontinence in 53 (60.9%), as shown in Summary Fig. More than half of the children reported nocturnal enuresis, including 61% of the girls and 52% of the boys. Twenty-three patients (25.6%) had a history of urinary tract infections. The mean age for acquisition of dryness during the day was 4.4 +/- 1.9 years. Parents of 61 patients (70.1%) acknowledged that LUTS had a significant impact on the quality of life of their children. Discussion A high prevalence of LUTS was confirmed with a significant negative impact on quality of life in a large population of children and adolescents with WBS. It was shown for the first time that the achievement of daytime bladder control is delayed in children with WBS. Although LUTS are not recognized as one of the leading features of the syndrome, it is believed that it should be considered as a significant characteristic of the clinical diagnosis of WBS. Conclusions LUTS are highly prevalent in children and adolescents with WBS and have a significant negative impact on patient's quality of life.
- Congenital genitourinary abnormalities in children with Williams-Beuren syndrome(2014) SAMMOUR, Zein M.; GOMES, Cristiano M.; BESSA JR., Jose de; PINHEIRO, Marcello S.; KIM, Chong A. E.; HISANO, Marcelo; BRUSCHINI, Homero; SROUGI, MiguelObjective: Williams-Beuren syndrome (WBS) is an autosomal dominant disorder caused by a gene deletion on chromosome 7q11.23. Patients with WBS usually show a group of features such as developmental delay, cardiovascular anomalies, mental retardation, and characteristic facial appearance. Abdominal wall defects, external genitalia anomalies, and structural abnormalities of the urinary tract have been scarcely evaluated and were the focus of our study. Materials and methods: We prospectively evaluated 41 boys and 38 girls with WBS, with a mean age of 8.8 +/- 4.1 (range 3-19 years). All patients were examined for the evaluation of inguinal and umbilical hernias and genital anomalies. All patients were offered a radiological evaluation, including urinary tract ultrasound, voiding cystourethrogram, and dimercaptosuccinic acid renal scintigraphy (DMSA scan). Results: Of the 41 boys, 30 (73.1%) had abnormalities on physical examination, including bilateral undescended testis in 13 (31.7%), retractile testis in four (9.7%), hypospadias in four (9.7%), and unilateral cryptorchidism in three (7.3%) patients. Of the 38 female subjects, 17 (44.7%) had at least one abnormality, including umbilical hernia in 11 (28.9%), unilateral inguinal hernia in four (10.5%), and bilateral inguinal hernia in three (7.8%) patients. Uroradiological abnormalities were found in 41 patients (51.9%). On sonography, six (7.6%) patients had unilateral hydronephrosis, three (3.8%) had a duplicated collecting system, and two (2.5%) had kidney stones. On DMSA, performed in 36 patients, four (11.1%) had unilateral renal scarring and two (5.5%) had bilateral renal scarring. Cystourethrography was obtained from 56 patients, of whom 27 (48.2%) had bladder diverticulum, 18 (32.1%) had bladder wall trabeculation, and three (5.3%) had vesicoureteral reflux. We found no association of urological abnormalities with cardiovascular defects. Conclusions: Patients with WBS have a high prevalence of abdominal wall, external genitalia, and urological abnormalities, emphasizing the importance of proper physical examination and radiological investigation in this population.
- CONGENITAL GENITOURINARY ABNORMALITIES IN CHILDREN WITH WILLIAMS-BEUREN SYNDROME(2012) SAMMOUR, Zein; GOMES, Cristiano; BESSA JR., Jose; PINHEIRO, Marcello; KIM, Chong; SACOMANI, Carlos; BRUSCHINI, Homero; SROUGI, Miguel
- Neurological status predicts response to alpha-blockers in men with voiding dysfunction and Parkinson's disease(2014) GOMES, Cristiano M.; SAMMOUR, Zein M.; BESSA JUNIOR, Jose de; BARBOSA, Egberto R.; LOPES, Roberto I.; SALLEM, Flavio S.; TRIGO-ROCHA, Flavio E.; BRUSCHINI, Homero; NITTI, Victor W.; SROUGI, MiguelOBJECTIVES: To evaluate predictors of the response to doxazosin, a selective alpha-adrenoceptor antagonist, when used for the treatment of lower urinary tract symptoms in men with Parkinson's disease. METHODS: In a prospective study, 33 consecutive men (mean age 59.2 +/- 7.0 years) with Parkinson's disease and lower urinary tract symptoms were evaluated. Neurological dysfunction was assessed with the Unified Parkinson's Disease Rating Scale. Urological assessment was performed at baseline and after 12 weeks of treatment with 4 mg/day of extended-release doxazosin, including symptom evaluation with the International Continence Society male short-form questionnaire, an assessment of the impact of lower urinary tract symptoms on quality of life and urodynamics. Clinical and urodynamic predictors of response were specifically evaluated. RESULTS: Compared with the score at baseline, the total International Continence Society male short-form score was reduced after doxazosin administration, from 17.4 +/- 7.5 to 11.1 +/- 6.9 (p<0.001). The impact of lower urinary tract symptoms on quality of life was also significantly reduced, from 1.8 +/- 1.1 to 1.0 +/- 1.0 (p<0.001) and the maximum urinary flow varied from 9.3 +/- 4.4 to 11.2 +/- 4.6 ml/s (p = 0.025). The severity of neurological impairment was the only predictor of the clinical response. Additionally, patients with a Unified Parkinson's Disease Rating Scale score lower than 70 had a significantly higher chance of clinical improvement with doxazosin treatment than those with higher Unified Parkinson's Disease Rating Scale scores did (RR = 3.10, 95% CI = [1.15 to 5.37], p = 0.011). CONCLUSIONS: Doxazosin resulted in the improvement of lower urinary tract symptoms and the maximum flow rate and was well tolerated in men with Parkinson's disease. The response to treatment is dependent on the severity of neurological disability.
- Antibiotic Prophylaxis for Prevention of Febrile Urinary Tract Infections in Children with Vesicoureteral Reflux: A Meta-Analysis of Randomized, Controlled Trials Comparing Dilated to Nondilated Vesicoureteral Reflux(2015) BESSA JR., Jose de; MRAD, Flavia Cristina de Carvalho; MENDES, Evilin Feitosa; BESSA, Marcia Carvalho; PASCHOALIN, Victor Pereira; TIRABOSCHI, Ricardo Brianezi; SAMMOUR, Zein Mohamed; GOMES, Cristiano Mendes; BRAGA, Luis H.; NETTO, Jose Murillo BastosPurpose: The followup and treatment of children with vesicoureteral reflux has been debated for many years. Antibiotic prophylaxis has a role for preventing urinary tract infection in these children. Recent studies and guidelines suggested that prophylaxis has little or no role in preventing urinary tract infection in those children, especially those with low grades (I and II) of reflux. Materials and Methods: We analyzed all published randomized, controlled trials comparing antibiotic prophylaxis vs no prophylaxis or placebo in children with vesicoureteral reflux. The children were divided into those with nondilated (grades I and II) and dilated (grades III and IV) vesicoureteral reflux. After data were analyzed the RIVUR study was published and, therefore, it was added to the analyzed data. Results: After analyzing the first published studies we found that antibiotic prophylaxis would be beneficial only in children with high grade vesicoureteral reflux. With the addition of the data in the RIVUR study these results changed. The new pooled data support antibiotic prophylaxis in all children with vesicoureteral reflux. Conclusions: Vesicoureteral reflux management is still controversial. In contrast to recently published studies and guidelines, this meta-analysis supports antibiotic prophylaxis in all children with vesicoureteral reflux regardless of reflux grade. More studies are needed to support this finding.
bookPart Cistite intersticial(2013) GOMES, Cristiano Mendes; SAMMOUR, Zein Mohamed- The Effects of Oxybutynin on Urinary Symptoms in Children with Williams-Beuren Syndrome(2012) SAMMOUR, Zein M.; GOMES, Cristiano M.; BESSA JR., Jose de; PINHEIRO, Marcello S.; KIM, Chong A.; HONJO, Rachel S.; TRIGO-ROCHA, Flavio E.; BRUSCHINI, Homero; SROUGI, MiguelPurpose: Williams-Beuren syndrome is a genomic disorder caused by a hemizygous contiguous gene deletion on chromosome 7q11.23. Lower urinary tract symptoms are common in children with Williams-Beuren syndrome. However, there are few data on the management of voiding symptoms in this population. We report our experience using oxybutynin to treat urinary symptoms in children with Williams-Beuren syndrome. Materials and Methods: We prospectively analyzed 42 patients with Williams-Beuren syndrome and significant lower urinary tract symptoms due to detrusor overactivity diagnosed on urodynamics in a 12-week, open-label study. Urological assessment included symptomatic evaluation, the impact of lower urinary tract symptoms on quality of life, frequency-volume chart, urodynamics and urinary tract sonography. After 12 weeks of treatment with 0.6 mg/kg oxybutynin per day given in 3 daily doses, patients were assessed for treatment efficacy and side effects. Results: A total of 17 girls and 19 boys completed medical therapy and were assessed at 12 weeks. Mean +/- SD patient age was 9.2 +/- 4.3 years (range 3 to 18). The most common urinary complaint was urgency, which occurred in 31 patients (86.1%), followed by urge incontinence, which was seen in 29 (80.5%). Compared to baseline, urinary symptoms were substantially improved. The negative impact of storage symptoms on quality of life was significantly decreased from a mean +/- SD of 3.3 +/- 1.7 to 0.5 +/- 0.9 (p <0.001). Mean +/- SD maximum urinary flow improved from 14.2 +/- 15.0 to 20.5 +/- 6.4 ml per second (p <0.001). Conclusions: A total of 12 weeks of therapy with 0.6 mg/kg oxybutynin daily resulted in improvement of lower urinary tract symptoms, quality of life and maximum flow rate in most patients with Williams-Beuren syndrome.