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1.
J Urol ; 212(1): 165-174, 2024 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-38700226

RESUMO

PURPOSE: Urodynamic testing (UDS) is an important tool in the management of pediatric lower urinary tract conditions. There have been notable efforts to standardize pediatric UDS nomenclature and technique, but no formal guidelines exist on essential elements to include in a clinical report. We sought to identify ideal structure and elements of a pediatric UDS assessment based on expert consensus. MATERIALS AND METHODS: Pediatric urologists regularly performing UDS were queried using a Delphi process. Participants were invited representing varied geographic, experience, and societal involvement. Participants underwent 3 rounds of questionnaires between November 2022 and August 2023 focusing on report organization, elements, definitions, and automated electronic health record clinical decision support. Professional billing requirements were also considered. Consensus was defined as 80% agreeing either in favor of or against a topic. Elements without consensus were discussed in subsequent rounds. RESULTS: A diverse sample of 30 providers, representing 27 institutions across 21 US states; Washington, District of Columbia; and Canada completed the study. Participants reported interpreting an average number of 5 UDS reports per week (range 1-22). The finalized consensus report identifies 93 elements that should be included in a pediatric UDS report based on applicable study conditions and findings. CONCLUSIONS: This consensus report details the key elements and structure agreed upon by an expert panel of pediatric urologists. Further standardization of documentation should aid collaboration and research for patients undergoing UDS. Based on this information, development of a standardized UDS report template using electronic health record implementation principles is underway, which will be openly available for pediatric urologists.


Assuntos
Consenso , Técnica Delphi , Urodinâmica , Humanos , Criança , Urologia/normas , Pediatria/normas , Masculino , Inquéritos e Questionários
2.
Pediatr Surg Int ; 40(1): 179, 2024 Jul 06.
Artigo em Inglês | MEDLINE | ID: mdl-38971909

RESUMO

INTRODUCTION: Women born with anorectal malformation (ARM) or Hirschsprung disease (HD) may have impaired urologic function resulting in sequelae in adulthood. This study assessed and compared self-reported urinary outcomes in adult females born with ARM or HD to a reference population. METHODS: This was an IRB approved, cross-sectional study of female-born patients with ARM or HD, who completed surveys between November 2021 and August 2022. Female patients between the ages of 18 and 80 years were included. Lower Urinary Tract Symptom Questionnaires were administered through REDCap and the responses were compared to a reference population using Chi-squared or Fisher's exact tests. RESULTS: Sixty-six born female patients answered the questionnaires, two of them identified as non-binary. The response rate was 76%. Median age was 31.6 years. The majority were born with cloaca (56.3%), followed by other type of ARMs (28.1%), complex malformation (9.4%), and HD (6.3%). A history of bladder reconstruction was present for 26.6%. Catheterization through a channel or native urethra was present in 18.8%. Two had ureterostomies and were excluded from the analysis. Seven had chronic kidney disease or end-stage renal disease, three with a history of kidney transplantation. Patients with cloaca had significantly higher rates of urinary incontinence, urinary tract infection, and social problems due to impaired urological functioning, when compared to an age-matched reference population (Table 3). CONCLUSION: This study emphasizes the need for a multi-disciplinary team that includes urology and nephrology following patients with ARM long term, especially within the subgroup of cloaca. LEVEL OF EVIDENCE: III.


Assuntos
Malformações Anorretais , Doença de Hirschsprung , Humanos , Feminino , Doença de Hirschsprung/cirurgia , Malformações Anorretais/cirurgia , Malformações Anorretais/complicações , Estudos Transversais , Adulto , Adulto Jovem , Adolescente , Pessoa de Meia-Idade , Idoso , Inquéritos e Questionários , Idoso de 80 Anos ou mais , Sintomas do Trato Urinário Inferior
3.
Pediatr Surg Int ; 38(12): 1693-1699, 2022 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-36151340

RESUMO

PURPOSE: The long-term impact of anorectal malformations (ARM) and Hirschsprung disease (HD) on sexual function is well recognized but understudied. This study evaluated self-reported sexual and fertility outcomes in adult males with ARM and HD. METHODS: This was an IRB approved, prospective study of males in the Adult Colorectal Research Registry who completed surveys between October 2019 and March 2022. Electronic surveys were administered after consenting to being contacted for research. Patients completed the International Index of Erectile Function (IIEF) questionnaire and provided information on fertility outcomes. RESULTS: Sixty-five patients completed outcome questionnaires: 11 (16.9%) had HD and 54 (83.1%) had an ARM. Nineteen patients reported some degree of erectile dysfunction per IIEF criteria, a greater proportion of whom have ARM (p = 0.046). Twenty (30.7%) have reported having children; there were no differences in rates between HD and ARM patients. Most patients had not attempted to conceive, but eight patients, all of whom have ARM, have pursued fertility investigation or treatments. CONCLUSION: More patients with ARM reported some degree of erectile dysfunction compared to those with HD. Additionally, some have required fertility treatments. Further investigation is warranted to ensure true low rates of sexual and fertility dysfunction in patients with HD.


Assuntos
Malformações Anorretais , Doença de Hirschsprung , Adulto , Humanos , Masculino , Malformações Anorretais/complicações , Malformações Anorretais/fisiopatologia , Disfunção Erétil/epidemiologia , Doença de Hirschsprung/complicações , Doença de Hirschsprung/fisiopatologia , Estudos Prospectivos , Inquéritos e Questionários , Fertilidade/fisiologia
4.
Pediatr Surg Int ; 38(12): 1709-1716, 2022 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-36151341

RESUMO

PURPOSE: Long-term urinary outcomes for patients born with Hirschsprung disease (HD) and anorectal malformations (ARM) may impact their health and wellbeing into adulthood. This study describes self-reported long-term urinary outcomes in males with HD and ARM. METHODS: This was a prospective study of male patients in the Adult Colorectal Research Registry who completed surveys on urinary function between October 2019 and March 2022. Self-reported health and functional outcomes were summarized, and differences based on type of condition were compared. RESULTS: Sixty-seven patients completed the questionnaire (response rate: 59.1%), of which 17.9% (12) had HD and 82.1% (55) had an ARM. Rates of urinary incontinence and stress urinary incontinence were 16.4% (11) and 4.5% (3), respectively. On sub-analysis of patients with ARM, patients with sacral ratio (SR) of 0.4-0.69 reported higher UTI rates compared to those with SR ≥ 0.7 (57.9 vs 25.8%, p = 0.023). Renal failure rates were highest among patients with recto-bladder neck fistulas (66.0%, p = 0.012). CONCLUSION: Patients with HD and ARM report a variety of urological sequelae in adulthood. Outcomes appear to be more common in patients with ARM and may be impacted by both anatomy and sacral ratios. Transitional care to monitor and manage renal and urological function is imperative.


Assuntos
Malformações Anorretais , Neoplasias Colorretais , Doença de Hirschsprung , Fístula da Bexiga Urinária , Adulto , Humanos , Masculino , Estudos Prospectivos , Malformações Anorretais/complicações , Doença de Hirschsprung/complicações , Medidas de Resultados Relatados pelo Paciente , Fístula da Bexiga Urinária/complicações , Neoplasias Colorretais/complicações
5.
Pediatr Blood Cancer ; 68(10): e29226, 2021 10.
Artigo em Inglês | MEDLINE | ID: mdl-34245214

RESUMO

BACKGROUND: Two chemotherapeutic agents used widely in pediatric oncology are vincristine (VCR) and doxorubicin (DOX), which may cause neuropathy and myopathy, respectively. The study hypothesis is that neurotoxic effects of VCR and/or myotoxic effects of DOX affect bladder physiology and manifest clinically as lower urinary tract dysfunction (LUTD). PROCEDURE: Based on a priori power analysis, 161 children divided evenly by gender were recruited. Children aged 5-10 years completed the dysfunctional voiding scoring system (DVSS) survey. The study cohort comprised cancer survivors treated with VCR and/or DOX. Healthy controls were recruited from well-child clinic visits. Exclusion criteria included pelvic-based malignancy, pelvic irradiation, pre-existing LUTD, neurologic abnormalities, and treatment with cyclophosphamide/ifosfamide. DVSS scores and presence of LUTD, defined as DVSS scores above gender-specific thresholds (males ≥9, females ≥6), were compared across cohorts. RESULTS: Median DVSS scores were higher in the study cohort (6 vs. 4, p = .003). Moreover, children in the study cohort were more likely to exceed threshold scores for LUTD (38.8% vs. 21%, p = .014; OR 1.8). Subanalysis by gender revealed female cancer survivors are more likely to report LUTD than controls (57.5% vs. 30%, p = .013, OR 1.9). This did not hold true for males (20% vs. 12.2%, p = .339). CONCLUSIONS: Childhood cancer survivors who received VCR and/or DOX reported higher rates of LUTD than controls. Female cancer survivors appear more likely to suffer from LUTD than males. Further study with a positive control cohort of cancer survivors who received non-VCR, non-DOX chemotherapy is underway to elucidate the contribution of a cancer diagnosis to LUTD.


Assuntos
Sobreviventes de Câncer , Doxorrubicina , Sintomas do Trato Urinário Inferior , Neoplasias , Vincristina , Criança , Doxorrubicina/efeitos adversos , Feminino , Humanos , Masculino , Neoplasias/tratamento farmacológico , Estudos Prospectivos , Bexiga Urinária , Vincristina/efeitos adversos
6.
Pediatr Surg Int ; 37(4): 491-494, 2021 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-33433664

RESUMO

Vaginoplasty with colon is a common technique for vaginal replacement in patients with cloaca. Malignancy in the neovagina is a rare outcome and typically presents decades after reconstruction. We present a case of an adolescent female with history of cloaca, ulcerative colitis, and high-grade dysplasia of the sigmoid neovagina.


Assuntos
Colite Ulcerativa , Vagina/cirurgia , Adolescente , Adulto , Cloaca , Colo Sigmoide , Constrição Patológica/complicações , Feminino , Procedimentos Cirúrgicos em Ginecologia/métodos , Humanos , Pessoa de Meia-Idade
7.
Pediatr Surg Int ; 37(1): 151-159, 2021 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-33161476

RESUMO

PURPOSE: Enhanced recovery after surgery (ERAS) is a perioperative management strategy to hasten postoperative recovery. We examined the effects of a pilot implementation of ERAS for pediatric patients on anesthetic outcomes. METHODS: We performed a prospective case-control study utilizing an ERAS protocol in patients aged < 18 years undergoing urologic reconstruction that included a bowel anastomosis. Protocol elements included: multimodal analgesia, opioid minimization, and routine nausea/vomiting prophylaxis. ERAS patients were propensity-matched with historical controls. Outcomes of interest included maximum PACU pain score, time to first opioid, opioid-free days, and need for opioids on day of discharge. RESULTS: A total of 13 ERAS patients and 26 historical controls were included, with median ages 9.9 years (IQR 9.1-11) and 10.4 years (IQR 8.0-12.4), respectively. ERAS increased the percentage of patients who did not receive any intraoperative or postoperative opioids (0% vs 15%, p = 0.046 for both) and reduced maximum PACU pain score (3 vs 0, p < 0.001). The use of postoperative supplemental oxygen was decreased in the ERAS group (85% vs 38%, p = 0.013). CONCLUSIONS: The implementation of an ERAS protocol appears to decrease postoperative pain, opioid usage, and positively impact other anesthetic outcomes in children undergoing urologic reconstructive surgery utilizing a bowel anastomosis.


Assuntos
Analgesia/métodos , Analgésicos Opioides/administração & dosagem , Recuperação Pós-Cirúrgica Melhorada , Dor Pós-Operatória/tratamento farmacológico , Procedimentos de Cirurgia Plástica/métodos , Náusea e Vômito Pós-Operatórios/prevenção & controle , Procedimentos Cirúrgicos Urológicos/métodos , Anastomose Cirúrgica/métodos , Estudos de Casos e Controles , Criança , Feminino , Humanos , Intestinos/cirurgia , Tempo de Internação/estatística & dados numéricos , Masculino , Estudos Prospectivos
8.
World J Urol ; 38(10): 2531-2536, 2020 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31781896

RESUMO

PURPOSE: To estimate how many boys with UDT must undergo orchiopexy to prevent one case of TC, one death from TC and one exposure to TC treatment beyond radical orchiectomy as compared to being treated at an older age. METHODS: This retrospective study utilized data from a 2007 Swedish study of males who underwent orchiopexy for UDT (Pettersson et al.). TC incidence for boys undergoing orchiopexy for UDT was assessed based on the age at orchiopexy (0-6 years, 7-9 years, 10-12 years, 13-15 years). The incidence of TC in each age cohort was calculated and used to determine the number needed to treat (NNT) for each age group using assumptions based on published TC outcomes. RESULTS: For an index patient ≤ 6 years, 372 boys need to undergo orchiopexy to prevent a single case of TC, 1488 boys to prevent exposure to TC therapy beyond radical orchiectomy, and 5315 boys to prevent a single TC-related death compared to treatment at an older age. CONCLUSION: While there is evidence supporting benefits of early orchiopexy, the NNT to affect TC outcomes is very high. Even those with delayed orchiopexies have low risk for TC poor outcomes. This information can be used when counseling patients and families faced with UDT about the risks related to TC, especially with comorbidities.


Assuntos
Criptorquidismo/cirurgia , Orquidopexia , Neoplasias Testiculares/prevenção & controle , Adolescente , Fatores Etários , Criança , Pré-Escolar , Humanos , Incidência , Lactente , Masculino , Estudos Retrospectivos , Neoplasias Testiculares/epidemiologia , Resultado do Tratamento , Adulto Jovem
9.
Am J Physiol Renal Physiol ; 317(1): F197-F206, 2019 07 01.
Artigo em Inglês | MEDLINE | ID: mdl-31066574

RESUMO

Cytotoxic chemotherapy is the foundation for the treatment of the wide variety of childhood malignancies; however, these therapies are known to have a variety of deleterious side effects. One common chemotherapy used in children, doxorubicin (DOX), is well known to cause cardiotoxicity and cardiomyopathy. Recent studies have revealed that DOX impairs skeletal and smooth muscle function and contributes to fatigue and abnormal intestinal motility in patients. In this study, we tested the hypothesis that systemic DOX administration also affects detrusor smooth muscle (DSM) function in the urinary bladder, especially when administered at a young age. The effects on the DSM and bladder function were assessed in BALB/cJ mice that received six weekly intravenous injections of DOX (3 mg·kg-1·wk-1) or saline for the control group. Systemic DOX administration resulted in DSM hypertrophy, increased voiding frequency, and a significant attenuation of DSM contractility, followed by a slower relaxation compared with the control group. Gene expression analyses revealed that unlike DOX-induced cardiotoxicity, the bladders from DOX-administered animals showed no changes in oxidative stress markers; instead, downregulation of large-conductance Ca2+-activated K+ channels and altered expression of myosin light-chain kinase coincided with reduced myosin light-chain phosphorylation. These results indicate that in vivo DOX exposure caused DSM dysfunction by dysregulation of molecules involved in the detrusor contractile-relaxation mechanisms. Collectively, our findings suggest that survivors of childhood cancer treated with DOX may be at increased risk of bladder dysfunction and benefit from followup surveillance of bladder function.


Assuntos
Antibióticos Antineoplásicos/toxicidade , Doxorrubicina/toxicidade , Sintomas do Trato Urinário Inferior/induzido quimicamente , Contração Muscular/efeitos dos fármacos , Relaxamento Muscular/efeitos dos fármacos , Cadeias Leves de Miosina/metabolismo , Miosinas de Músculo Liso/metabolismo , Doenças da Bexiga Urinária/induzido quimicamente , Bexiga Urinária/efeitos dos fármacos , Urodinâmica/efeitos dos fármacos , Fatores Etários , Animais , Feminino , Hipertrofia , Subunidades alfa do Canal de Potássio Ativado por Cálcio de Condutância Alta/metabolismo , Subunidades beta do Canal de Potássio Ativado por Cálcio de Condutância Alta/metabolismo , Sintomas do Trato Urinário Inferior/metabolismo , Sintomas do Trato Urinário Inferior/patologia , Sintomas do Trato Urinário Inferior/fisiopatologia , Masculino , Camundongos Endogâmicos BALB C , Quinase de Cadeia Leve de Miosina/metabolismo , Fosforilação , Transdução de Sinais , Fatores de Tempo , Bexiga Urinária/metabolismo , Bexiga Urinária/patologia , Bexiga Urinária/fisiopatologia , Doenças da Bexiga Urinária/metabolismo , Doenças da Bexiga Urinária/patologia , Doenças da Bexiga Urinária/fisiopatologia
11.
Pediatr Surg Int ; 35(2): 233-237, 2019 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-30392127

RESUMO

PURPOSE: Traditionally, the care of children and adults has been arbitrarily separated into pediatric and adult medicine and surgery. Despite progress in pediatric surgical techniques, patients born with congenital anomalies still suffer from significant functional sequelae, which persist into adulthood. We aim to describe some of the most common problems experienced by adult patients with congenital colorectal malformations. METHODS: Following IRB approval, we performed a retrospective database review of all adult patients who were treated by our group from 1983 until 2017. RESULTS: We identified 88 cases. 51 patients had ARM, 18 cloacas, 9 presacral masses, 3 HD, 2 spina bifida and 5 with other diagnoses (3 vaginal anomalies, 1 cloacal exstrophy, 1 obstructed seminal vesical). The specific problems addressed were: complications from previous operations (41), rectal prolapse (25), fecal incontinence (11), gynecologic concerns (12), urologic concerns (6), and recurrent recto urogenital fistula (3). We performed 83 surgical interventions, including 13 rectal prolapse repair, 13 continent appendicostomies, 44 PSARP or redo PSARP, 11 resections of presacral masses, 11 vaginoplasties, 2 examinations under anesthesia, and 2 Mitrofanoff procedures. Five patients were treated medically (bowel management program, obstetric, urologic evaluation). CONCLUSION: There is a growing need to better prepare adult providers to assume the care of patients born with congenital colorectal disease as these patients transition to adulthood. A collaboration between specialized pediatric referral centers with adult colorectal surgeons, urologists and gynecologists is a potential pathway for the adequate transition of care.


Assuntos
Malformações Anorretais/epidemiologia , Anormalidades Congênitas/epidemiologia , Transição para Assistência do Adulto , Adolescente , Adulto , Malformações Anorretais/terapia , Colorado/epidemiologia , Anormalidades Congênitas/terapia , Bases de Dados Factuais , Procedimentos Cirúrgicos do Sistema Digestório , Incontinência Fecal/etiologia , Feminino , Doenças dos Genitais Femininos/etiologia , Procedimentos Cirúrgicos em Ginecologia , Humanos , Masculino , Pessoa de Meia-Idade , Complicações Pós-Operatórias/terapia , Prolapso Retal/etiologia , Estudos Retrospectivos , Doenças Urológicas/etiologia , Adulto Jovem
12.
Pediatr Surg Int ; 35(9): 985-987, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31256297

RESUMO

AIM OF THE STUDY: Misdiagnosing a cloaca as a disorder of sex development may lead to inappropriate testing, treatment, and negative emotional consequences to families. We were impressed by the fact that a significant number of patients suffering from a cloaca were referred to us with the diagnosis of a "disorder of sex development" previously referred as "ambiguous genitalia" or "intersex". On re-evaluation, none of them truly had a disorder of sex differentiation. This prompted us to conduct the following retrospective review to try to find the cause of the misdiagnosis and the way to prevent it. METHODS: A retrospective review of our colorectal database was performed to identify the total number of patients with cloacas and the number initially diagnosed as "ambiguous genitalia, intersex"/disorder of sex development. The external appearance of their genitalia and unnecessary testing or treatment received were recorded. MAIN RESULTS: A total of 605 patients with cloacas were identified. Of these, 77 (12.7%) were referred to us with the diagnosis of "ambiguous genitalia" and 13 of them (17%) went on to receive an intervention that was not indicated: karyotyping (10), steroids (3), and ovarian biopsy (1). The karyotype result in all patients was XX. The misdiagnosis was triggered by the external appearance of the perineum, simulating a case of virilization with a hypertrophic clitoris, but was simply prominent labial skin. Careful examination of the perineal structure allowed us to determine that it consisted of folded skin with no evidence of corpora. CONCLUSION: Patients born with a cloaca are at risk for mismanagement from being erroneously labeled as disorders of sex development. The diagnosis of a cloacal anomaly is a clinical one. The practitioner must distinguish between phallus-like clitoral hypertrophy and a normal clitoris with prominent labial skin.


Assuntos
Cloaca/anormalidades , Erros de Diagnóstico/prevenção & controle , Transtornos do Desenvolvimento Sexual , Bases de Dados Factuais , Feminino , Genitália/anormalidades , Humanos , Períneo/anormalidades , Estudos Retrospectivos
13.
Pediatr Surg Int ; 35(9): 1005-1008, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31278478

RESUMO

PURPOSE: The literature about ectopic ureters in anorectal malformations is limited. Repair of an anorectal malformation may require dissection near the normal or abnormal insertion of the ureters. Knowledge of the presence and location of ectopic ureters may prevent intraoperative injury. We aim to describe the incidence and location of ectopic ureters in patients with anorectal malformations and to characterize associated renal anomalies. METHODS: This is an IRB-approved retrospective study of patients with anorectal malformations and ectopic ureters identified in our colorectal database. RESULTS: Of 2283 patients with anorectal malformation, 79 (3.5%) had ectopic ureter(s). Of those, 29% had bilateral ectopic ureters. Nearly all (87%) of bilateral ectopic ureters occurred in females. Ectopic ureters most commonly inserted into the bladder neck (33%), vagina (15%), or urethra (13%). Renal dysfunction was noted in a high proportion of patients. The majority (59%) of ectopic ureters were associated with dysfunction of the ipsilateral kidney. Interestingly, 29% of patients with unilateral ureteral ectopia had an abnormal contralateral kidney. Only 22 patients (28%) had two normal kidneys, and 5 (6%) had documented renal failure with 2 (2.5%) requiring renal transplant. This compares to a transplant rate of 0.6% among anorectal malformation patients without ectopic ureter. CONCLUSIONS: The incidence of ectopic ureter is 3.5% among anorectal malformation patients. Cloaca and recto-bladder neck fistula are the types of anorectal malformation with higher incidence. Ureteral ectopia seems to confer an increased risk of renal failure. Identifying ectopic ureters is important for surgical planning and monitoring renal function vigilantly.


Assuntos
Malformações Anorretais/cirurgia , Ureter/anormalidades , Pré-Escolar , Bases de Dados Factuais , Feminino , Humanos , Rim/anormalidades , Masculino , Estudos Retrospectivos
14.
Int Braz J Urol ; 45(1): 145-149, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30620155

RESUMO

OBJECTIVE: In the majority of published series, children undergoing open pyeloplasty are admitted for at least one night. We hypothesized that it would be possible in the majority of infants to perform open pyeloplasty as an outpatient procedure. MATERIALS AND METHODS: All patients who underwent open pyeloplasty by a single surgeon between 2008 and 2016 were retrospectively reviewed. Demographic data (age at surgery, gender, pre- and postoperative imaging studies, laterality, type of local anesthesia), operative time, duration of hospital stay, need for narcotic analgesics, complications, readmission within 1-month after surgery and need for additional procedures were abstracted. RESULTS: A total of 18 infants underwent open pyeloplasty by single surgeon. Mean age at time of surgery was 19 months (range 3-23 months). There were 8 girls and 10 boys. In addition to general anesthesia, all of the patients received regional anesthesia (caudal block 8, epidural block 8, subcutaneous nerve block 2). Median operative time was 135 minutes (range 81-166). Median hospital stay was 1 day (range 1 to 2). Two patients required iv narcotics for pain management. None of the patients required parenteral administration of other medications during the short hospitalization. No patients required any additional procedures or hospital readmissions within 1 month from surgery. CONCLUSIONS: In appropriately selected patients, outpatient pyeloplasty appears to be feasible with an oral postoperative analgesia plan to be administered at home.


Assuntos
Obstrução Ureteral/cirurgia , Procedimentos Cirúrgicos Urológicos/métodos , Feminino , Humanos , Lactente , Masculino , Pacientes Ambulatoriais , Cuidados Pós-Operatórios , Estudos Retrospectivos
15.
Am J Physiol Renal Physiol ; 315(5): F1320-F1328, 2018 11 01.
Artigo em Inglês | MEDLINE | ID: mdl-30089034

RESUMO

Lower urinary tract dysfunction (LUTD) is a common problem in children and constitutes up to 40% of pediatric urology clinic visits. Improved diagnosis and interventions have been leading to better outcomes in many patients, whereas some children are left untreated or do not respond to the treatment successfully. In addition, many of these patients are lost by the pediatric urologists during their teenage years, and the outcome in later life largely remains unidentified. Studies suggest childhood LUTD is associated with subsequent adult urinary tract symptoms. However, whether and how early life LUTD attributes to urinary symptoms in those patients later in life remains to be elucidated. In the current study, we investigated the effects of early life voiding perturbation on bladder function using a neonatal maternal separation (NMS) protocol in mice. The NMS group displayed a delayed development of voluntary voiding behavior, a significant reduction of functional bladder capacity, and bladder overactivity compared with control mice later in life. In vitro evaluation of detrusor smooth muscle and molecular study showed a decrease in muscarinic contribution alongside an increase in purinergic contribution in detrusor contractility in NMS mice compared with control group. These results suggest that early life bladder dysfunction interfered with the normal maturation of the voluntary micturition control and facilitated LUTD in a later stage, which is at least partly attributed to an alteration of muscarinic and purinergic signaling in the urinary bladder.


Assuntos
Fibras Colinérgicas/metabolismo , Sintomas do Trato Urinário Inferior/metabolismo , Receptores Muscarínicos/metabolismo , Receptores Purinérgicos P2X1/metabolismo , Bexiga Urinária Hiperativa/metabolismo , Bexiga Urinária/inervação , Micção , Urodinâmica , Fatores Etários , Animais , Animais Recém-Nascidos , Ansiedade de Separação/complicações , Ansiedade de Separação/psicologia , Modelos Animais de Doenças , Feminino , Sintomas do Trato Urinário Inferior/fisiopatologia , Sintomas do Trato Urinário Inferior/psicologia , Masculino , Privação Materna , Camundongos Endogâmicos C57BL , Reflexo , Bexiga Urinária Hiperativa/fisiopatologia , Bexiga Urinária Hiperativa/psicologia
16.
World J Urol ; 36(12): 2051-2058, 2018 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-29855697

RESUMO

PURPOSE: Surgical repair for proximal hypospadias has been associated with long-term success rates of 32-68%. In a prior study, outcomes for proximal hypospadias in patients with a diagnosis of disorders of sex development (DSD) were no different than those of patients without DSD. The objective of our study is to report our experience with proximal hypospadias repair in patients with and without DSD. METHODS: We retrospectively reviewed patients who underwent repair of proximal hypospadias between 2005 and 2016. Data collected included patient and disease characteristics, operative details, complications, and follow-up. The primary outcome was unplanned reoperation. RESULTS: Sixty seven patients were identified; 30 (44.8%) with DSD and 37 (55.2%) without DSD. Median follow-up was 28.3 months (IQR 18.9-45.7). 41 patients (61.2%) underwent at least one unplanned reoperation, median time to unplanned reoperation 10.3 months. More patients with DSD needed an unplanned reoperation (80 vs. 45.9%, p = 0.024). During the first 12 months after initial repair, there was no difference in unplanned reoperation rates (40 vs. 32.4%, p = 0.611), but there was a difference in the first 24 months post-operatively (76.7 vs. 43.2%, p = 0.007). On multivariate logistic regression, older age at initial repair (OR 1.144) and two stage repair (OR 7.644) were positively associated with unplanned reoperation in the first 2 years after repair. CONCLUSIONS: Proximal hypospadias repair is associated with an overall 61.2% reoperation rate regardless of associated DSD diagnosis. Patients with DSD are more likely to undergo an unplanned reoperation in the first 2 years after repair.


Assuntos
Transtornos do Desenvolvimento Sexual/epidemiologia , Hipospadia/cirurgia , Procedimentos de Cirurgia Plástica/estatística & dados numéricos , Reoperação/estatística & dados numéricos , Procedimentos Cirúrgicos Urológicos Masculinos/estatística & dados numéricos , Fatores Etários , Estudos de Casos e Controles , Criptorquidismo/epidemiologia , Humanos , Hipospadia/epidemiologia , Lactente , Modelos Logísticos , Masculino , Análise Multivariada , Estudos Retrospectivos , Fatores de Risco
17.
Am J Physiol Renal Physiol ; 313(5): F1149-F1160, 2017 Nov 01.
Artigo em Inglês | MEDLINE | ID: mdl-28768664

RESUMO

Posterior urethral valves are the most common cause of partial bladder outlet obstruction (PBOO) in the pediatric population. Pathological changes in the bladder developed during PBOO are responsible for long-lasting voiding dysfunction in this population despite early surgical interventions. Increasing evidence showed PBOO induces an upregulation of hypoxia-inducible factors (HIFs) and their transcriptional target genes, and they play a role in pathophysiological changes in the obstructed bladders. We hypothesized that blocking HIF pathways can prevent PBOO-induced bladder dysfunction. PBOO was surgically created by ligation of the bladder neck in male C57BL/6J mice for 2 wk. PBOO mice received intraperitoneal injection of either saline or 17-DMAG (alvespimycin, 3 mg/kg) every 48 h starting from day 1 postsurgery. Sham-operated animals received injection of saline on the same schedule as PBOO mice and served as controls. The bladders were harvested after 2 wk, and basal activity and evoked contractility of the detrusor smooth muscle (DSM) were evaluated in vitro. Bladder function was assessed in vivo by void spot assay and cystometry in conscious, unrestrained mice. Results indicated the 17-DMAG treatment preserved DSM contractility and partially prevented the development of detrusor over activity in obstructed bladders. In addition, PBOO caused a significant increase in the frequency of micturition, which was significantly reduced by 17-DMAG treatment. The 17-DMAG treatment improved urodynamic parameters, including increases in the bladder pressure at micturition and nonvoid contractions observed in PBOO mice. These results demonstrate that treatment with 17-DMAG, a HIF inhibitor, significantly alleviated PBOO-induced bladder pathology in vivo.


Assuntos
Benzoquinonas/farmacologia , Lactamas Macrocíclicas/farmacologia , Contração Muscular/efeitos dos fármacos , Músculo Liso/efeitos dos fármacos , Obstrução do Colo da Bexiga Urinária/prevenção & controle , Bexiga Urinária/efeitos dos fármacos , Animais , Modelos Animais de Doenças , Masculino , Camundongos Endogâmicos C57BL , Músculo Liso/metabolismo , Obstrução do Colo da Bexiga Urinária/tratamento farmacológico , Micção/fisiologia , Urodinâmica/efeitos dos fármacos
18.
J Urol ; 195(4 Pt 2): 1250-6, 2016 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-26926557

RESUMO

PURPOSE: Posterior urethral valves are the most common cause of partial bladder outlet obstruction in the pediatric population. However, to our knowledge the etiology and the detailed mechanisms underlying pathological changes in the bladder following partial bladder outlet obstruction remain to be elucidated. Recent findings suggest that hypoxia and associated up-regulation of HIFs (hypoxia-inducible factors) have a key role in partial bladder outlet obstruction induced pathology in the bladder. We examined the effects of pharmacological inhibition of HIF pathways by 17-DMAG (17-(dimethylaminoethylamino)-17-demethoxygeldanamycin) in pathophysiological phenotypes after partial bladder outlet obstruction. MATERIALS AND METHODS: Partial bladder outlet obstruction was surgically created in male C57BL/6J mice. The animals received oral administration of 17-DMAG or vehicle daily starting from the initiation of obstruction up to 5 days. Sham operated mice served as controls. Bladders were harvested from each group 2, 4 and 7 days postoperatively, and analyzed for histological and biochemical changes. Bladder function was assessed by in vitro muscle contractility recordings. RESULTS: Partial bladder outlet obstruction caused a significant increase in the bladder mass accompanying enhanced collagen deposition in the bladder wall while 17-DMAG treatment suppressed those increases. Treatment with 17-DMAG attenuated the degree of up-regulation of HIFs and their target genes involving the development of tissue fibrosis in obstructed bladders. Treatment with 17-DMAG improved the decreased responses of obstructed bladder strips to electrical field stimulation and KCl. CONCLUSIONS: In vivo 17-DMAG treatment decreased partial bladder outlet obstruction induced pathophysiological changes in the bladder. HIF pathway inhibition has a potential clinical implication for the development of novel pharmacological therapies to treat bladder pathology associated with partial bladder outlet obstruction.


Assuntos
Fatores de Transcrição Hélice-Alça-Hélice Básicos/antagonistas & inibidores , Benzoquinonas/farmacologia , Benzoquinonas/uso terapêutico , Lactamas Macrocíclicas/farmacologia , Lactamas Macrocíclicas/uso terapêutico , Obstrução do Colo da Bexiga Urinária/prevenção & controle , Bexiga Urinária/efeitos dos fármacos , Animais , Modelos Animais de Doenças , Hipertrofia/prevenção & controle , Masculino , Camundongos , Camundongos Endogâmicos C57BL , Bexiga Urinária/patologia , Bexiga Urinária/fisiopatologia , Obstrução do Colo da Bexiga Urinária/patologia , Obstrução do Colo da Bexiga Urinária/fisiopatologia
19.
Pediatr Surg Int ; 31(3): 287-90, 2015 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-25475503

RESUMO

PURPOSE: To review our experience with infants undergoing distal hypospadias repair without a postoperative stent to determine if an unacceptable complication rate might justify its use. METHODS: Children <1 year of age who underwent distal hypospadias repair by a single surgeon were identified through a prospectively maintained database. The use of a postoperative urethral stent was recorded for each case. Demographics, meatus position, operative technique and complications were also recorded. Patients older than 1 year or with hypospadias proximal to a subcoronal position were excluded. RESULTS: Eighty-nine patients without a stent were identified in addition to 21 patients who had a stent for a minimum of 3 days. At 3 months follow-up, 4/89 (4.49 %) patients in the stentless group required circumcision revision. 1 patient experienced urinary retention. 1/21 (4.76 %) patients with a postoperative stent required reoperation for meatal stenosis. CONCLUSIONS: The complication rate for infants undergoing distal hypospadias repair is low, does not appear to be significantly increased by forgoing a postoperative urethral stent. Avoiding a stent likely reduces a variety of associated adverse events and needs for short-term follow-up.


Assuntos
Hipospadia/cirurgia , Complicações Pós-Operatórias/epidemiologia , Stents , Uretra/cirurgia , Seguimentos , Humanos , Lactente , Masculino , Estudos Prospectivos , Reoperação/estatística & dados numéricos
20.
Pediatr Surg Int ; 31(9): 871-7, 2015 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-26143412

RESUMO

OBJECTIVES: Our objectives were to: (1) delineate factors associated with surgery at <1 year and (2) determine if early intervention was associated with increased risk of readmission. METHODS: We conducted a retrospective review of children age 0-18 years with a diagnosis of UPJ obstruction who underwent pyeloplasty from 1/1/1999 to 9/1/2009 using the PHIS database. Data collected included patient factors (race, gender, insurance type, APR-DRG severity of illness) and hospital factors (annual case volume, census region, academic status). Outcomes assessed were: age at surgery and hospital readmission within 1 year of surgery. Data were analyzed using logistic regression and Cox PH for multivariate analyses. RESULTS: 4499 children met study criteria. Minority race (OR 1.55), male gender (OR 1.49), public insurance (OR 1.37), high severity of illness (OR 3.60), Southern region (OR 1.44), and low hospital volume (OR 1.37) were significant predictors of early surgery (p < 0.05). Only early surgery (HR 2.42; 95% CI 1.67-3.49 2.42) was associated with increased risk of readmission. CONCLUSIONS: In children with UPJ obstruction, age at surgery is associated with patient demographic and hospital factors. Early surgery is associated with higher rates of readmission, suggesting that variations in age at surgery may be associated with significant differences in outcomes.


Assuntos
Obstrução Ureteral/cirurgia , Procedimentos Cirúrgicos Urológicos/métodos , Adolescente , Fatores Etários , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Estudos Retrospectivos , Fatores de Risco , Resultado do Tratamento
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