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INTRODUCTION: Bilateral ureteral reimplantation at the time of the complete primary repair of bladder exstrophy (BUR-CPRE) has been proposed and has demonstrated favorable outcomes in the past. However, the potential benefits, including prevention of vesicoureteral reflux (VUR) and renal scarring must be tempered with any risks of reimplantation, persistent VUR, and the potential for overtreatment. We aimed to determine the impact of BUR-CPRE on reflux rates, renogram findings and bladder capacity. METHODS: An IRB approved registry of children treated for bladder exstrophy epispadias complex (BEEC) during a long-term international collaboration hosted in a region with high prevalence of BEEC was queried. Children undergoing primary CPRE for bladder exstrophy (BE) were identified. Surgical procedure and outcome measures nuclear medicine dimercaptosuccinic acid (DMSA) scintigraphy scans, voiding cystourethrogram (VCUG), and urodynamic study (UDS) were assessed for presence and degree of VUR, renogram abnormalities, and bladder capacity. RESULTS: A total cohort of 147 patients with BEEC was queried; 52 children (37 males, 71%) underwent primary CPRE for BE between 2009 and 2019 at median age of 1.1 years (IQR 0.6-1.9 years) with median follow up 4.4 years (IQR 2.4-6.4 years). BUR-CPRE was performed in 22/52 (42%). After BUR-CPRE, children were less likely to have VUR (any VUR present in 9 of 20 with imaging (45%) compared to 23 of 26 with imaging (82%) in the CPRE alone group (p = 0.007)). VUR in the BUR-CPRE group tended to be unilateral and lower grade in comparison to the CPRE alone group. DMSA abnormalities were less common in the BUR-CPRE group (4/19 (21%) vs.12/27 (44%)), although the difference did not reach statistical significance (p = 0.1). At 4 years follow-up, the BUR-CPRE group had a larger bladder capacity (p = 0.016). DISCUSSION: After BUR-CPRE, children had a lower rate of VUR, and when present, VUR was more often unilateral and lower grade compared to the CPRE alone group. Fewer numbers of children in the BUR-CPRE group depicted DMSA abnormalities. No children developed obstruction after BUR-CPRE and none have undergone repeat reimplantation. We documented a larger bladder capacity at the time of maximum follow-up available (4 years)-but further data are needed to confirm this observation. CONCLUSION: BUR-CPRE decreases the incidence and severity of VUR after CPRE, but the clinical significance of this remains unclear. We are encouraged by these initial results, but since BUR-CPRE does not uniformly eliminate VUR, we continue to proceed carefully in the well selected patient.
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Extrofia Vesical , Refluxo Vesicoureteral , Extrofia Vesical/diagnóstico por imagem , Extrofia Vesical/cirurgia , Criança , Humanos , Lactente , Masculino , Reimplante , Estudos Retrospectivos , Refluxo Vesicoureteral/diagnóstico por imagem , Refluxo Vesicoureteral/cirurgiaRESUMO
INTRODUCTION: Obesity has been thought to increase the risk of complications and need for additional resources with surgery, but only a limited amount of evidence is available in the pediatric population. OBJECTIVE: The objectives were to describe the weight status of pediatric and adolescent patients undergoing a continent urinary tract reconstruction and to determine the association between obesity and 30-day postoperative morbidity. STUDY DESIGN: A retrospective cohort study was performed for pediatric and adolescent patients aged up to 20 years who underwent a continent urinary tract reconstruction between January 2010 and November 2016. Weight status was stratified by the body mass index (BMI) for age z-scores as follows: underweight (<5th percentile), normal (5th to <85th percentiles), overweight (85th to <95th percentiles), and obese (≥95th percentile). Primary outcomes included the duration of intensive care and hospitalization as well as re-admissions and complications within 30 days. RESULTS: A total of 182 continent reconstructions were identified during the study period. Demographic and peri-operative data are provided in the Table. Weight status was not associated with any primary outcomes on univariate or multivariate analysis. There was also no association in an analysis between the non-overweight or non-obese group (BMI for age z-score <85th) and overweight or obese group (BMI for age z-score ≥85th percentiles); a subgroup analysis between patients with and without myelomeningocele; or a subgroup analysis for wound, infectious, or high-grade (Clavien-Dindo grades III or higher) complications. DISCUSSION: Obesity has been consistently associated with an increased risk of surgical site infections and wound complications after a wide variety of surgeries in adults. The results from the present study conflict with those of the few available studies in the pediatric population. The high-risk nature of the present cohort may have mitigated any effect of obesity on 30-day postoperative morbidity. The limitations of the present study include its retrospective design at a single center and the potential misclassification of weight status with the BMI. CONCLUSIONS: Almost 30% of pediatric and adolescent patients undergoing a continent urinary tract reconstruction were overweight or obese. Obesity as determined by the BMI was not associated with 30-day postoperative morbidity.
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Índice de Massa Corporal , Obesidade/complicações , Procedimentos de Cirurgia Plástica/métodos , Complicações Pós-Operatórias/epidemiologia , Incontinência Urinária/cirurgia , Sistema Urinário/cirurgia , Procedimentos Cirúrgicos Urológicos/métodos , Adolescente , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Masculino , Morbidade/tendências , Estudos Retrospectivos , Fatores de Risco , Estados Unidos/epidemiologia , Incontinência Urinária/complicações , Incontinência Urinária/fisiopatologia , Urodinâmica/fisiologia , Adulto JovemRESUMO
STUDY QUESTION: Is it feasible to disseminate testicular tissue cryopreservation with a standardized protocol through a coordinated network of centers and provide centralized processing/freezing for centers that do not have those capabilities? SUMMARY ANSWER: Centralized processing and freezing of testicular tissue from multiple sites is feasible and accelerates recruitment, providing the statistical power to make inferences that may inform fertility preservation practice. WHAT IS KNOWN ALREADY: Several centers in the USA and abroad are preserving testicular biopsies for patients who cannot preserve sperm in anticipation that cell- or tissue-based therapies can be used in the future to generate sperm and offspring. STUDY DESIGN, SIZE, DURATION: Testicular tissue samples from 189 patients were cryopreserved between January 2011 and November 2018. Medical diagnosis, previous chemotherapy exposure, tissue weight, and presence of germ cells were recorded. PARTICIPANTS/MATERIALS, SETTING, METHODS: Human testicular tissue samples were obtained from patients undergoing treatments likely to cause infertility. Twenty five percent of the patient's tissue was donated to research and 75% was stored for patient's future use. The tissue was weighed, and research tissue was fixed for histological analysis with Periodic acid-Schiff hematoxylin staining and/or immunofluorescence staining for DEAD-box helicase 4, and/or undifferentiated embryonic cell transcription factor 1. MAIN RESULTS AND THE ROLE OF CHANCE: The average age of fertility preservation patients was 7.9 (SD = 5) years and ranged from 5 months to 34 years. The average amount of tissue collected was 411.3 (SD = 837.3) mg and ranged from 14.4 mg-6880.2 mg. Malignancies (n = 118) were the most common indication for testicular tissue freezing, followed by blood disorders (n = 45) and other conditions (n = 26). Thirty nine percent (n = 74) of patients had initiated their chemotherapy prior to undergoing testicular biopsy. Of the 189 patients recruited to date, 137 have been analyzed for the presence of germ cells and germ cells were confirmed in 132. LIMITATIONS, REASONS FOR CAUTION: This is a descriptive study of testicular tissues obtained from patients who were at risk of infertility. The function of spermatogonia in those biopsies could not be tested by transplantation due limited sample size. WIDER IMPLICATIONS OF THE FINDINGS: Patients and/or guardians are willing to pursue an experimental fertility preservation procedure when no alternatives are available. Our coordinated network of centers found that many patients request fertility preservation after initiating gonadotoxic therapies. This study demonstrates that undifferentiated stem and progenitor spermatogonia may be recovered from the testicular tissues of patients who are in the early stages of their treatment and have not yet received an ablative dose of therapy. The function of those spermatogonia was not tested. STUDY FUNDING/COMPETING INTEREST(S): Support for the research was from the Eunice Kennedy Shriver National Institute for Child Health and Human Development grants HD061289 and HD092084, the Scaife Foundation, the Richard King Mellon Foundation, the Departments of Ob/Gyn & Reproductive Sciences and Urology of the University of Pittsburgh Medical Center, United States-Israel Binational Science Foundation (BSF), and the Kahn Foundation. The authors declare that they do not have competing financial interests.
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Criopreservação , Preservação da Fertilidade/métodos , Infertilidade Masculina/terapia , Testículo , Adolescente , Adulto , Fatores Etários , Antineoplásicos/efeitos adversos , Biópsia , Criança , Pré-Escolar , Preservação da Fertilidade/normas , Doenças Hematológicas/complicações , Doenças Hematológicas/terapia , Humanos , Infertilidade Masculina/etiologia , Masculino , Neoplasias/complicações , Neoplasias/terapia , Radioterapia/efeitos adversos , Contagem de Espermatozoides , Recuperação Espermática , Espermatogônias/fisiologia , Adulto JovemRESUMO
INTRODUCTION: A nutritional assessment is a critical but often neglected aspect of a preoperative evaluation. Malnutrition is clearly associated with worse surgical outcomes in adults undergoing major abdominal surgery, whereas a paucity of evidence is available in the pediatric population. OBJECTIVE: The objectives were to describe the preoperative nutritional status of pediatric and adolescent patients undergoing continent urinary tract reconstruction and to determine the association among malnutrition, use of total parenteral nutrition (TPN), and surgical outcomes. STUDY DESIGN: A retrospective cohort study was performed for patients aged up to 20 years who underwent continent urinary tract reconstruction between January 2012 and November 2016. Malnutrition was classified with body mass index and height for age z-scores on admission as well as change in z-scores and weight over the 3-6 months before surgery. Primary outcomes included the duration of intensive care and hospitalization as well as readmissions and complications within 30 days. RESULTS: A total of 123 patients who underwent 130 continent urinary tract reconstructions were identified during the study period. Demographic and perioperative data are provided in the Table. Anthropometric and biochemical measurements as well as the early initiation of TPN (≤2 days) were not associated with any primary outcomes. In a subgroup analysis of patients with a bowel anastomosis, the early initiation of TPN was an independent predictor for duration of hospitalization (P < 0.0001) and 30-day complications (odds ratio 9.51, P = 0.005) after adjusting for other statistically significant and clinically relevant variables. DISCUSSION: The few available studies on surgical nutrition have primarily focused on infants and young children undergoing cardiac surgery and provided no consensus on a preoperative nutritional assessment. The findings on TPN from the present study favorably compare with a growing body of evidence in adult and pediatric critically ill and surgical patients. The limitations of the present study include its retrospective design at a single institution, potential misclassification of nutritional status, and selection bias from the initiation of TPN at the discretion of the primary surgeon. CONCLUSIONS: Malnutrition was identified in greater than 20% of pediatric and adolescent patients undergoing continent urinary tract reconstruction. Anthropometric and biochemical parameters were not associated with surgical outcomes, although the early initiation of TPN did not offer any benefit for nutritional support. In a subset of patients with a bowel anastomosis, TPN was associated with worse surgical outcomes, including a longer duration of hospitalization and development of 30-day complications.
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Estado Nutricional , Nutrição Parenteral Total/estatística & dados numéricos , Doenças Urológicas/cirurgia , Procedimentos Cirúrgicos Urológicos/métodos , Adolescente , Criança , Estudos de Coortes , Feminino , Humanos , Masculino , Desnutrição/complicações , Desnutrição/diagnóstico , Cuidados Pré-Operatórios , Estudos Retrospectivos , Doenças Urológicas/complicaçõesRESUMO
INTRODUCTION: Patients with anorectal malformations (ARMs) have a high incidence of genitourinary anomalies. Those with a recto-bladder neck fistula may represent a high-risk group, but their long-term urologic outcomes are poorly described. OBJECTIVE: To evaluate the clinical and urodynamic outcomes in a large cohort of patients with an ARM subtype of recto-bladder neck fistula. MATERIALS AND METHODS: A retrospective cohort study was performed of patients who had been treated for a recto-bladder neck fistula at the present institution since 2007. The primary outcomes were the ability to achieve urinary continence after 4 years of age, and development of a mildly decreased glomerular filtration rate (GFR) or worse (<89 ml/min/1.73 m2). Continence was defined as the ability to store urine for 3-4 h during the day and 8 h overnight without leakage. RESULTS: Demographic and clinical data are provided in the Summary Table. The most recent urodynamic findings included the presence of detrusor overactivity in 30 (75%) patients, median leak point pressure of 56.0 cmH2O (range, 14-140), median functional cystometric capacity at 40 cmH2O of 125.5% age-expected capacity (range, 36-473%), and median maximum cystometric capacity of 131.0% age-expected capacity (range, 44-473%). A mildly decreased GFR or worse developed in 13 (24%) patients. Of the 52 (78%) patients who were followed by pediatric urology at the present institution with a median follow-up of 30.9 months (range, 0.0-86.8), 35 (67%) were at least 4 years of age and could be assessed for continence. Continence was achieved in five (14%) patients voiding spontaneously and 15 (43%) performing CIC. Recurrent urinary tract infections (UTI) (OR 0.70, P = 0.006) were an independent predictor of incontinence, while urethral anomalies (OR 1.40, P = 0.03) were an independent predictor of chronic kidney disease (CKD) on multiple logistic regression analysis. DISCUSSION: The findings favorably compared with other studies, but were more robust due to the size of the cohort and breadth of urologic evaluation. Limitations included the retrospective design at a single institution. Incomplete clinical data and misclassification of continence may have lead to bias. CONCLUSIONS: This large cohort of patients with an ARM subtype of recto-bladder neck fistula had a high incidence of genitourinary anomalies. They were rarely able to achieve continence with spontaneous voiding alone and were at risk of developing CKD, both of which were likely multifocal in origin. Long-term urologic follow-up is warranted for patients with a recto-bladder neck fistula.
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Malformações Anorretais/fisiopatologia , Malformações Anorretais/cirurgia , Fístula Retal/fisiopatologia , Fístula Retal/cirurgia , Fístula da Bexiga Urinária/fisiopatologia , Fístula da Bexiga Urinária/cirurgia , Malformações Anorretais/complicações , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Fístula Retal/complicações , Estudos Retrospectivos , Fatores de Risco , Resultado do Tratamento , Fístula da Bexiga Urinária/complicações , Incontinência Urinária/etiologia , Incontinência Urinária/prevenção & controle , UrodinâmicaRESUMO
INTRODUCTION: Stomal stenosis is the most common complication after the creation of a continent catheterizable channel (CCC), but is challenging to treat. OBJECTIVE: To describe early experience with triamcinolone injection for the treatment of stomal stenosis. MATERIALS AND METHODS: A retrospective review was performed of patients who had undergone a triamcinolone injection (40 mg/ml) for the treatment of stomal stenosis at the present institution. The primary outcome of success was defined as a patient-reported or caregiver-reported return to ease with catheterization, and avoidance of stomal revision or indwelling catheter. The cost of care with various techniques for the treatment of stomal stenosis was also assessed by representing the cost as a percentage of charges for a re-operative Monti ileovesicostomy. RESULTS: A total of 22 injections were performed in 18 CCCs of 17 patients. Demographic and clinical data are provided in the Summary Table. Thirteen CCCs (72%) were successfully treated with a single injection. Three patients (10%) required a total of five repeat injections at a median of 5.1 months between injections (IQR, 4.6-12.4). One patient required a stomal revision at 34.9 months after the initial injection, while one patient also required a Chait cecostomy catheter. The median length of time for ease with catheterization was 11.6 months (IQR, 3.5-18.0) after the initial injection, and 10.4 months (IQR, 4.5-16.0) after any injection. No adverse effects or complications occurred from the injection. There were no variables associated with failure after the initial or any injection on univariate analysis. Represented as a percentage of charges for a reoperative Monti ileovesicostomy, the cost of care was 11.3% for a stomal revision, 5.8% for triamcinolone injection in the operating room, and 0.3% as an office-based procedure. DISCUSSION: The success rate for triamcinolone injection favorably compared with other options for the treatment of stomal stenosis. It surpassed the reported success rate of stomal dilation and approached that of stomal revision. Other studies have similarly observed a minimal risk of adverse effects and complications from both topical and intralesional corticosteroids. The limitations of the present study included its retrospective design at a single institution. A larger cohort of patients with a longer follow-up is necessary to determine its long-term durability. CONCLUSIONS: Triamcinolone injection was an effective and well-tolerated option for the treatment of stomal stenosis in any cutaneous stoma, thereby avoiding the need for a more invasive and costly stomal revision.
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Anti-Inflamatórios/administração & dosagem , Cistostomia/efeitos adversos , Estomas Cirúrgicos/patologia , Triancinolona/administração & dosagem , Cateterismo Urinário/efeitos adversos , Criança , Constrição Patológica , Feminino , Humanos , Injeções , Masculino , Estudos Retrospectivos , Cateterismo Urinário/instrumentaçãoRESUMO
INTRODUCTION: Botulinum toxin injection (BTI) has been advocated as a second line therapy in management of neuropathic bladder in pediatric population for refractory patients to conventional medical management such as anticholinergics. The purpose was to review the safety and efficacy of BTI in children with neuropathic bladder refractory to conservative non-surgical measures. We hypothesized that BTI would be an effective alternative to bladder augmentation in certain patients but not all. METHODS: We retrospectively identified 22 patients with neuropathic bladder due to any condition who underwent urologic BTI at our hospital since 2010. Multiple clinicopathologic variables were examined including the following: demographics, use of anticholinergics, the presence of anticholinergic refractoriness or intolerance, dosage of BTI, urodynamic variables, and continence status. RESULTS: The mean patient age at time of BTI was 10 years with a follow up of 12 months. Indications for BTI were anti-cholinergic refractory (AR) urodynamic parameters and/or incontinence and anticholinergic intolerance (AI). Nearly all patients received 300 Units at BTI into detrusor. No complications occurred from BTI. Overall 54% had improved continence after the initial BTI whereas 45% had achieved complete continence between catheterizations. Cystometric capacity increased by 46% and maximum detrusor pressure decreased by 43% following initial BTI (See Table). 75% of AI patients were continent between CIC after BTI compared to 50% of AR patients (P = 0.002). The observed mean duration of clinical improvement after initial BTI was 4.6 months and four patients underwent repeat BTI. Pre BTI % of age expected bladder capacity Post BTI % of age expected bladder capacity % Improvement in Urodynamic Parameter P value Cystometric Capacity (mL) 227 60 331 87 46 0.008 Maximum Detrusor Pressure (cm H2O) 63 44 43 0.002 Compliance (mL/cm H2O) 4.3 8.8 104 0.001. DISCUSSION: Our results are comparable to existing literature with respect to urodynamic parameters. The observed differences may be due to heterogenous patient population of various etiologies of neuropathic bladder and no uniform criteria to proceed with bladder augmentation. The AR patients in our cohort may have had a higher degree of bladder fibrosis which BTI would be less likely to impact and explain the differences in clinical response between AR and AI patients. CONCLUSIONS: BTI is a safe and effective treatment option for pediatric patients with neuropathic bladder refractory to standard therapy. The degree of continence observed after BTI in our series was higher for AI rather than AR patients.
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Toxinas Botulínicas Tipo A/administração & dosagem , Bexiga Urinaria Neurogênica/tratamento farmacológico , Urodinâmica/fisiologia , Administração Intravesical , Adolescente , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Masculino , Fármacos Neuromusculares/administração & dosagem , Estudos Retrospectivos , Fatores de Tempo , Resultado do Tratamento , Bexiga Urinaria Neurogênica/fisiopatologia , Adulto JovemRESUMO
OBJECTIVE: To present our experience with infant pyeloplasty, comparing outcomes between robotic-assisted laparoscopic pyeloplasty (RALP) and open pyeloplasty (OP). MATERIALS AND METHODS: A retrospective review was performed of all children <1 year of age who underwent unilateral dismembered pyeloplasty at a single pediatric institution since January 2007. Patients with standard laparoscopic pyeloplasty were excluded. Patient demographics, intraoperative details, narcotic usage, and complications were reviewed. RESULTS: A total of 70 infants (51 boys and 19 girls) were identified, with nine RALP and 61 OP performed. Median age was 9.2 months (range, 3.7-11.9 months) for RALP and 4.1 months (range, 1.0-11.6 months) for OP (p = 0.005). Median weight was 8 kg (range, 5.8-10.9 kg) for RALP and 7 kg (range, 4-14 kg) for OP (p = 0.163). Median operative time was 115 min (range, 95-205 min) for RALP and 166 min (range, 79-300 min) for OP (p = 0.028). Median hospital stay was 1 day (range, 1-2 days) for RALP and 3 days (range, 1-7 days) for OP (p < 0.001). Median postoperative narcotic use of morphine equivalent was <0.01 mg/kg/day (range, 0-0.1 mg/kg/day) for RALP and 0.05 mg/kg/day (range, 0-2.2 mg/kg/day) for OP (p < 0.001). Median follow-up was 10 months (range, 7.2-17.8 months) for RALP and 43.6 months (3.4-73.8 months) for OP (p < 0.001). The success rate was 100% for RALP and 98% for OP. CONCLUSIONS: Infant RALP was observed to be feasible and efficacious with shorter operative time, hospital stay, and narcotic utilization than OP.
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Hidronefrose/cirurgia , Pelve Renal/cirurgia , Robótica , Procedimentos Cirúrgicos Urológicos/métodos , Analgésicos Opioides/uso terapêutico , Estudos de Coortes , Feminino , Seguimentos , Humanos , Hidronefrose/diagnóstico por imagem , Lactente , Pelve Renal/diagnóstico por imagem , Pelve Renal/fisiopatologia , Tempo de Internação , Masculino , Procedimentos Cirúrgicos Minimamente Invasivos/métodos , Duração da Cirurgia , Dor Pós-Operatória/tratamento farmacológico , Dor Pós-Operatória/fisiopatologia , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/fisiopatologia , Estudos Retrospectivos , Medição de Risco , Fatores de Tempo , Resultado do Tratamento , UltrassonografiaAssuntos
Isquemia Encefálica/genética , Interleucina-10/genética , Polimorfismo de Nucleotídeo Único , Acidente Vascular Cerebral/genética , Fator de Necrose Tumoral alfa/genética , Adulto , Idoso , Feminino , Estudos de Associação Genética , Predisposição Genética para Doença , Humanos , Índia , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Regiões Promotoras GenéticasRESUMO
BACKGROUND & OBJECTIVE: Automobile exhaust consists of many toxic components and is considered to be a major health concern in urban areas. Traffic policemen are occupationally exposed to vehicular exhaust during the traffic control. Hence, the present study was aimed to evaluate genotoxic effects of vehicular exhaust in traffic policemen in Hyderabad, south India. METHODS: Analysis of chromosomal aberrations was carried out in 136 traffic policemen, including 78 non smokers and 58 smokers who were exposed to vehicular exhaust for a period of 1-28 yr. For comparison, 115 healthy males including 69 non smokers and 46 smokers of the same age group and socio-economic status (who were not exposed to any chemical or radiation at their workplace) were studied. RESULTS: A significant increase (P<0.05) was observed in the mean frequency of chromosomal aberrations in non smoker and smoker traffic policemen (6.48 and 8.96 respectively) when compared to their respective control groups (3.35 and 4.30). According to the age a significant increase in the frequency of chromosomal aberrations was observed both in control and exposed groups (P<0.05). As the duration of exposure increased in traffic policemen, there was a corresponding increase in the frequency of chromosomal aberrations. INTERPRETATION & CONCLUSION: Cytogenetic damage was more pronounced in smokers when compared to non smokers. Age and duration of exposure also appear to play a vital role in causing cytogenetic damage. Thus the present study suggests that the induction of cytogenetic damage might be due to the cumulative effect of smoking, age and duration of exposure to vehicular exhaust.
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Poluentes Ocupacionais do Ar/toxicidade , Emissões de Veículos/toxicidade , Adulto , Estudos de Casos e Controles , Aberrações Cromossômicas , Análise Citogenética , Humanos , Índia , Masculino , Pessoa de Meia-Idade , Mutagênicos/toxicidade , Exposição Ocupacional , Polícia , Adulto JovemRESUMO
A screening program was carried out for amino acid disorders in children with mental handicaps from the state of Andhra Pradesh (India) during the last two decades. Forty-one (0.9%) cases were detected with amino acid disorders among 4500 children surveyed. We reported amino acid disorders of rare occurrence such as dicarboxylic aminoaciduria, hydroxykynureninuria, persistent hypertyrosinemia, hydroxyprolinemia, hypervalinemia, etc. A new metabolic defect threoninemia was also detected. We have observed a preponderance of males with amino acid disorders. Parental consanguinity was present in 54% of cases with amino acid disorders.
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Erros Inatos do Metabolismo dos Aminoácidos/epidemiologia , Erros Inatos do Metabolismo dos Aminoácidos/genética , Deficiência Intelectual/complicações , Adolescente , Criança , Consanguinidade , Feminino , Frequência do Gene , Humanos , Índia/epidemiologia , Masculino , Programas de Rastreamento , Linhagem , Fatores SexuaisRESUMO
OBJECTIVES: To study the epidemiological factors in children with syndromic hearing loss. STUDY DESIGN: Interview based prospective study. SETTINGS: Govt. ENT Hospital, AYJNIHH, NIMH-SEC, and Schools for the Deaf-in Hyderabad and SecunderabadPatients: Children aged below 14 years, with hearing loss, their parents/guardians. INTERVENTION(S): The study revealed type and degree of hearing impairment. In high risk groups genetic counseling was offered. RESULTS: Epidemiological studies were carried out in 743 children below 14 years with hearing impairment and 138 (18.57%) were found to have syndromic deafness. Majority of the children with hearing loss have an association of ocular abnormality (22.46%, n=31) followed by skeletal anomalies 14.49% (n=20) and dental anomalies (10.86%). We observed 24 cases (3.21%) with genetically well recognized syndromes. CONCLUSION: Data is generated on epidemiological and etiology of Hearing Impairment. Hearing Impairment is due to both environmental and genetic causes. Environmental factors in 17 (13.77%), genetically inherited 21 cases (15.22%) and the cause is not known in the remaining cases. Such a data is required in order to offer genetic counseling to reduce the genetic burden.
RESUMO
The semen quality of 57 workers from a welding plant in South India and 57 controls was monitored. Blood nickel and chromium concentrations were determined by ICP-MS. Analysis of semen samples was performed in accordance with World Health Organization criteria. The blood level of nickel and chromium for the 28 exposed workers was 123.3 +/- 35.2 and 131.0 +/- 52.6 microg/l, resepctively, which was significantly higher than the 16.7 +/- 5.8 and 17.4 +/- 8.9 microg/l for the control group (n=27). Sperm concentrations of exposed workers were 14.5 +/- 24.0 millions/ml and those of the control group were 62.8 +/- 43.7 millions/ml. Rapid linear sperm motility was decreased in exposed workers compared to controls. There was a significant positive correlation between the percentage of tail defects and blood nickel concentration in exposed workers. The sperm concentration showed a negative correlation with blood chromium content in workers. More abnormal characteristics were found in the semen of exposed workers. Semen abnormalities correlated with the number of years of exposure to welding fumes containing nickel and chromium.
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Poluentes Ocupacionais do Ar/toxicidade , Cromo/efeitos adversos , Níquel/efeitos adversos , Exposição Ocupacional/efeitos adversos , Sêmen/metabolismo , Soldagem , Adulto , Poluentes Ocupacionais do Ar/sangue , Sobrevivência Celular/efeitos dos fármacos , Cromo/sangue , Cromo/metabolismo , Monitoramento Ambiental , Nível de Saúde , Humanos , Índia , Masculino , Espectrometria de Massas , Níquel/sangue , Níquel/metabolismo , Análise de Regressão , Sêmen/química , Sêmen/efeitos dos fármacos , Motilidade dos Espermatozoides/efeitos dos fármacos , Fatores de TempoRESUMO
INTRODUCTION: Patients with chronic obstructive pulmonary disease (COPD) are more likely to develop pulmonary morbidity following major abdominal surgery. The purpose of this study was to examine the utility of epidural analgesia in patients with COPD who underwent elective transperitoneal abdominal aortic aneurysm (AAA) repair. METHODS: During a 7-year period, all patients diagnosed with COPD undergoing elective AAA repair (n=425) from three hospitals were reviewed. Inclusion criteria were an FEV(1)/FVC ratio <75% and/or a PaCO(2)>45 mmHg. Clinical outcomes were compared between those who received epidural analgesia (epidural group) and those who did not (control group). Primary endpoints measured were duration of intubation, ICU stay, hospital days, and pulmonary complications. RESULTS: Strict inclusion criteria were met by 131 patients, which included 86 patients in the epidural group and 45 patients in the control group. When comparing the epidural vs. control group, the mean AAA size was 6.3+/-0.9 cm vs. 6.0+/-1.5 cm (NS), FEV(1) was 57.2+/-24.7% vs. 49.0+/-10.3% (NS), and the mean FEV(1)/FVC ratio was 52.0+/-11.4% vs. 50.6+/-6.7% (NS), respectively. The epidural group had a significantly lower incidence of post-operative ventilator dependency and ICU stay (p<0.05), as well as a decreased trend in pulmonary complications when compared to the control group. The overall hospital stay remained similar between the two groups. The relative risk of developing a pulmonary complication in the absence of epidural analgesia was 2.3. CONCLUSIONS: Perioperative epidural analgesia is beneficial in patients with COPD undergoing AAA repair by reducing both the post-operative ventilator duration and ICU stay. Epidural analgesia should be considered in all COPD patients undergoing elective transperitoneal AAA repair.
Assuntos
Analgesia Epidural , Aorta Abdominal/cirurgia , Aneurisma da Aorta Abdominal/cirurgia , Doença Pulmonar Obstrutiva Crônica/cirurgia , Idoso , Aneurisma da Aorta Abdominal/complicações , Aneurisma da Aorta Abdominal/fisiopatologia , Estudos de Casos e Controles , Cuidados Críticos , Volume Expiratório Forçado , Humanos , Tempo de Internação , Pulmão/fisiopatologia , Seleção de Pacientes , Pneumonia/complicações , Doença Pulmonar Obstrutiva Crônica/complicações , Doença Pulmonar Obstrutiva Crônica/fisiopatologia , Insuficiência Respiratória/complicações , Estudos Retrospectivos , Resultado do Tratamento , Capacidade VitalRESUMO
Tobacco dust mainly contains nitrosamines, which are readily absorbed by the body tissues like skin, respiratory epithelium, and mucous membrane of mouth, nose and intestines. Exposure to tobacco dust is known to affect the respiratory tracts in humans. In the present study, cytogenetic effects of exposure to tobacco dust are evaluated in 154 male tobacco factory workers and 138 age and sex matched controls by analysing chromosomal aberrations in their peripheral blood lymphocytes. The workers were in the age group of 20-55 years and were employed in the tobacco processing factory for 1-32 years. Heparinised blood samples were collected from workers and control subjects and lymphocyte cultures were carried out by using standard technique. Slides were prepared and 150 metaphases were screened for each sample for various structural and numerical types of abnormalities. A statistically significant increase was observed in the frequencies of chromosomal aberrations in non-smoking and smoking exposed groups when compared to the respective controls. An increase in the frequencies of chromosomal aberrations was also observed with increase in years of service in the exposed subjects.
Assuntos
Aberrações Cromossômicas , Nicotiana/toxicidade , Exposição Ocupacional , Adulto , Poeira , Humanos , Linfócitos/efeitos dos fármacos , Masculino , Pessoa de Meia-Idade , Fumar/efeitos adversosRESUMO
Cement industry is considered as a major pollution problem on account of dust and particulate matter emitted at various steps of cement manufacture. Cement dust consists of many toxic constituents. The workers who are employed in cement industries are exposed to cement dust for long periods. Therefore, it is mandatory to evaluate the mutagenic effects of occupational exposure to cement dust in such workers. In the present study, we analyzed the samples of 124 male workers including 59 smokers and 65 non-smokers who were employed in cement industry for a period of 1-17 years. For comparison, 106 controls (including 47 smokers and 59 non-smokers) of the same age group and socio-economic status were also studied. Controls had no exposure to cement dust or any known physical or chemical agent. A significant increase in the incidence of chromosomal aberrations was observed in the exposed group when compared to the control group. The results were analyzed separately for non-smokers and smokers. The chromosomal damage was more pronounced in the smokers when compared with the non-smokers both in control and exposed groups. A significant increase in the frequency of chromosomal aberrations was also observed with increase in age in both control and exposed subjects.
Assuntos
Aberrações Cromossômicas , Materiais de Construção , Poeira/efeitos adversos , Exposição Ocupacional , Adulto , Fatores Etários , Compostos de Alumínio/toxicidade , Células Cultivadas , Humanos , Linfócitos/ultraestrutura , Masculino , Pessoa de Meia-Idade , Silicatos/toxicidade , Fumar/genéticaRESUMO
PURPOSE: We previously reported on the short-term (4 weeks) morphometric analysis of a large bladder acellular matrix allograft used as a bladder bioprosthesis (average size 24 cm.2). We demonstrated cellular repopulation through the entire thickness of the graft. We now present the long-term (12 weeks) morphometric results of graft regenerated porcine bladders using segments measuring an average of 40 cm.2. MATERIALS AND METHODS: Bladders harvested from pigs were subjected to detergent and enzymatic extractions to render them acellular. Partial cystectomy was performed in 21 pigs and the defect was repaired with a bladder acellular matrix allograft (average size 40.52 cm.2). Of the animals 8 were sacrificed at 1, 2 and 4 weeks and 13 were sacrificed at 8 and 12 weeks. To evaluate cellular repopulation and matrix reorganization the native bladder and graft were analyzed using standard histological and immunofluorescent techniques. To evaluate for calcium deposits in the grafts a radiological evaluation of the graft was performed after explantation. RESULTS: All animals survived the surgical procedure and there were no significant urinary leaks. No stones were noted in any of the bladders. At 1 week there was a diffuse infiltration with acute inflammatory cells. At 2 weeks the luminal surface of the graft was lined with a single layer of urothelium, and there was stromal infiltration with unorganized smooth muscle cells and angiogenesis. At 4 weeks the urothelium was multilayered with organizing groups of smooth muscle cells and angiogenesis. At 8 and 12 weeks there was repopulation throughout the bladder acellular matrix allograft implant with all native cellular components participating. CONCLUSIONS: We present evidence that large patch bladder acellular matrix allograft implantation is technically feasible and may prove to be a viable surgical alternative to bladder augmentation with intestinal segments. Its advantages may include the potential for complete and functional regeneration of a bladder substitute.
Assuntos
Bexiga Urinária/cirurgia , Procedimentos Cirúrgicos Urológicos , Animais , Estudos de Viabilidade , Feminino , Imuno-Histoquímica , Masculino , Procedimentos de Cirurgia Plástica , Suínos , Transplante Homólogo , Bexiga Urinária/patologia , Urotélio/patologiaRESUMO
PURPOSE: We compared sutured pyeloplasty to 2 newer techniques of tissue anastomosis, including laser soldered pyeloplasty using a diode laser with 50% albumin solder mixed with indocyanine green and fibrin glue. MATERIALS AND METHODS: We performed 53 pyeloplasties in 50 pigs using suture, laser or fibrin glue. In the immediate group anastomotic leak pressure was measured immediately postoperatively, and then animals were euthanized. At 1, 3 and 4 weeks postoperatively a pressure flow study at 10 cc per minute in cm. H2O was performed, and tissue was sent for histological and collagen content analysis. RESULTS: In the immediate studies laser soldering achieved a significantly higher mean anastomotic leak pressure (50.5 +/- 15.1 cm. H2O) than sutured (17.3 +/- 5.4) or fibrin glued (3.5 +/- 1.5) repairs. In the 1, 2 and 4-week studies animals in the sutured pyeloplasty group had no complications, and all pressure flow studies except 1 were normal. However, in the laser soldered groups we observed 8 urinomas in 19 animals, and most occurred during the first part of our study. This complication was prevented by stopping urine flow at the anastomotic site at laser irradiation and by improving application of the solder. Of the 11 animals in which pressure flow studies were performed only 2 were obstructed. Of the 7 chronic fibrin glue group 4 animals had urinomas and 2 had unobstructed pressure flow studies. Histological studies and immunohistochemical staining for collagen showed no differences in collagen distribution among the 3 procedures. CONCLUSIONS: Laser soldering and fibrin glue pyeloplasties are not superior in the long-term compared to sutured pyeloplasty. Fibrin glue in our animal model had the highest failure rate. Further improvements in the technical aspect of laser tissue welding need to be made to benefit from its theoretical advantages in minimally invasive surgery.
Assuntos
Adesivo Tecidual de Fibrina/uso terapêutico , Rim/cirurgia , Terapia a Laser , Técnicas de Sutura , Adesivos Teciduais/uso terapêutico , Anastomose Cirúrgica/métodos , Animais , Modelos Animais de Doenças , Estudos de Viabilidade , SuínosRESUMO
PURPOSE: We evaluated the usefulness of postoperative imaging studies after extravesical ureteroneocystostomy and identified patient subsets in which these studies are necessary. MATERIALS AND METHODS: Records of 438 patients who had undergone extravesical ureteroneocystostomy from 1991 to 1997 were reviewed retrospectively. Study inclusion criterion was primary reflux with at least 1 year of postoperative followup. Grades I to III reflux were defined as low, and grades IV and V were defined as high. All patients were on prophylactic antibiotics. Evaluation included ultrasound and a voiding cystourethrogram 3 months postoperatively, and if reflux persisted the studies were repeated at 12 months. RESULTS: A total of 438 patients (723 renal units) underwent extravesical ureteral reimplantation. At 3 months the procedure was successful in 93.2% of ureters (91.3% patients), and at 12 months the success rate increased to 97.9% of ureters (95.4% patients). There were 49 renal units in 38 cases that were refluxing at 3 months, 11 of which were contralateral. At 12 months reflux resolved spontaneously in 20 of 38 ipsilateral and 8 of 11 contralateral ureters. Of the remaining 18 ipsilateral units reflux was high grade preoperatively in 12 and persisted postoperatively in 4. There was a statistically significant difference in the success rate at 1 year between high (94%) versus low (99%) grade reflux (p = 0.007). Age and preoperative bladder function did not significantly affect the success rates but males had a lower success rate. Hydronephrosis was noted in 7.2% of ureters at 6 weeks and in only 0.005% at 12 months. CONCLUSIONS: Extravesical reimplantation is successful in treating vesicoureteral reflux. Postoperative voiding cystourethrogram should be reserved for high grade reflux. Limiting these studies will help reduce patient discomfort and the cost of treatment.
Assuntos
Reimplante , Ureter/cirurgia , Refluxo Vesicoureteral/cirurgia , Criança , Estudos de Avaliação como Assunto , Feminino , Humanos , Masculino , Período Pós-Operatório , Resultado do Tratamento , MicçãoRESUMO
OBJECTIVES: To evaluate the use of a large-segment (> 24 cm2) bladder substitution with porcine bladder acellular matrix allograft (BAMA) in a large animal model. Materials and methods Bladders were harvested from pigs at the time of necropsy and subjected to detergent and enzymatic extractions to render them acellular. The BAMA produced had the surgical handling and suture-retaining properties of normal bladder tissue. Six pigs had BAMA segments implanted under general anaesthesia, through a low midline abdominal incision and after partial cystectomy. The defect was repaired with a BAMA patch (mean size 43.88 cm2, range 12-72), with no urinary diversion. Two animals each were then killed at 9, 16 and 30 days and the bladders explanted. The native bladder and BAMA patch were analysed morphometrically to evaluate cellular re-population and matrix re-organization. RESULTS: All animals survived surgery; there were no urinary leaks and no stones detected in any of the bladders. At 9 days there was a diffuse infiltration with acute inflammatory cells, but no areas of necrosis. There were isolated areas of smooth muscle cell (SMC) infiltration of the BAMA. At 16 days the luminal surface was lined with a single layer of urothelium, there was stromal infiltration with disorganized SMC and angiogenesis, with mature vessels in the BAMA patch. At 30 days the urothelium was multilayered with organizing groups of SMCs and angiogenesis. The highest cell density was at the periphery of the repopulated BAMA patch, decreasing towards the centre. CONCLUSIONS: The implantation of large patches of BAMA is technically feasible and may prove to be a viable surgical alternative to bladder augmentation with intestinal segments. The advantages of BAMA include the potential for complete and functional regeneration of a bladder substitute. This model provides a tool with which to obtain a better understanding of the cellular and molecular aspects of matrix re-population.