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1.
Pediatr Surg Int ; 40(1): 166, 2024 Jul 02.
Artigo em Inglês | MEDLINE | ID: mdl-38954216

RESUMO

PURPOSE: To report our experience with laparoscopic repair of anterior congenital diaphragmatic hernia (CDH) using extracorporeal subcutaneous knot tying and to define recurrence risk factors. METHODS: This retrospective unicentric study included children who underwent laparoscopic repair of anterior CDH without patch, using extracorporeal knot tying of sutures passed through the full thickness of the abdominal wall (2013-2020). A systematic review of the literature with meta-analysis was performed using the MEDLINE database since 2000. RESULTS: Eight children were included (12 months [1-183]; 10.6 kg [3.6-65]). Among the two patients with Down syndrome, one with previous cardiac surgery had a recurrence at 17 months postoperatively. In our systematic review (26 articles), among the 156 patients included, 10 had a recurrence (none with patch). Recurrence was statistically more frequent in patients with Down syndrome (19.4%) than without (2.5%) (p < 0.0001), and when absorbable sutures were used (50%) instead of non-absorbable sutures (5.3%) (p < 0.0001). CONCLUSION: Laparoscopic repair of anterior CDH without patch was a safe and efficient surgical approach in our patients. The use of a non-absorbable prosthetic patch should be specifically discussed in anterior CDH associated with Down syndrome and/or in case of previous cardiac surgery to perform a diaphragmatic tension-free closure.


Assuntos
Hérnias Diafragmáticas Congênitas , Herniorrafia , Laparoscopia , Recidiva , Humanos , Hérnias Diafragmáticas Congênitas/cirurgia , Hérnias Diafragmáticas Congênitas/complicações , Laparoscopia/métodos , Estudos Retrospectivos , Lactente , Herniorrafia/métodos , Masculino , Feminino , Pré-Escolar , Criança , Técnicas de Sutura , Recém-Nascido , Adolescente , Síndrome de Down/complicações , Fatores de Risco
2.
World J Urol ; 41(10): 2767-2774, 2023 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-37642679

RESUMO

PURPOSE: To present our initial experience with periurethral adjustable continence therapy (ACT™) for urinary incontinence due to intrinsic sphincter deficiency (ISD) in children. METHODS: This is an approved prospective non-randomized pilot study (NCT03351634) aiming to treat children born with spinal dysraphism (SD) or exstrophy epispadias complex (EEC) with ACT™. Endpoints were patient-reported changes in daily pad count, 24-h Pad test and complications. RESULTS: Since April 2018, 13 children (six girls, seven boys) were implanted at the median age of 12 years (5-16). The etiology of incontinence was neurogenic ISD (7/13, 54%) and EEC (6/13, 46%). After ACT™ implantation, continence (no pad or 1 security pad/day) was achieved in 9(69%) patients (5/7 SD, 4/6 EEC). Additionally, two (15%) patients had a significant improvement (decreasing Pad test from 1049 to 310 g at 3 months). One patient (7%) had no improvement. Results were stable at 21 months (6-43) of follow-up. Mean final balloon volume was 2.89 ml (± 0.85) with a median of 3 fillings to obtain continence. We had four revisions due to cutaneous port erosion (n = 3) and balloon migration (n = 1) and two definitive explantations. PinQ score was significantly improved (47 vs 40.5 with balloon, p = ns). Neither degradation of the upper urinary tract nor cystomanometric changes have been observed at 6 and 12 months postoperatively. CONCLUSION: Urinary incontinence due to ISD owing to EEC or SD can be successfully treated with ACT™ periurethral balloons. Given the minimal invasiveness of this therapy, it might be a first-line option treatment in children with complex stress urinary incontinence.


Assuntos
Doenças Uretrais , Incontinência Urinária por Estresse , Incontinência Urinária , Masculino , Feminino , Humanos , Criança , Pré-Escolar , Adolescente , Projetos Piloto , Estudos Prospectivos , Incontinência Urinária/etiologia , Incontinência Urinária/terapia , Incontinência Urinária por Estresse/cirurgia , Próteses e Implantes , Resultado do Tratamento
3.
Prenat Diagn ; 42(4): 502-511, 2022 04.
Artigo em Inglês | MEDLINE | ID: mdl-35226372

RESUMO

OBJECTIVE: To assess the contribution and impact of fetal magnetic resonance imaging (MRI) in managing fetal gastroschisis. METHODS: We conducted an observational retrospective study of gastroschisis patients at three fetal medicine centers from 2008 to 2019. The primary endpoint was the number of cases in which the MRI provided relevant information related to gastroschisis. RESULTS: A total of 189 patients were included, and our study group included 38 patients who underwent MRI. For the eight patients with suspected gastroschisis, MRI confirmed the diagnosis. In six cases, it provided additional relevant information (spiral turn, intestine ischemia, and bowel size discrepancy). For the 17 patients with ultrasound signs of additional gastrointestinal anomalies, MRI detected one case of unidentified complex gastroschisis on sonography. For the 13 patients undergoing routine MRI, no significant information was obtained. One termination of pregnancy and one fetoscopy were performed a few days after the MRI results. There was no subsequent follow-up or additional bowel complications to support management. CONCLUSION: Although MRI did not change the management of pregnancies complicated by fetal gastroschisis, patients presenting with fetal gastroschisis with intraabdominal bowel dilatation could benefit from MRI to allow for more precise prenatal counseling to predict postnatal intestinal complications before birth.


Assuntos
Gastrosquise , Feminino , Gastrosquise/diagnóstico por imagem , Humanos , Imageamento por Ressonância Magnética , Gravidez , Prognóstico , Estudos Retrospectivos , Ultrassonografia Pré-Natal/métodos
4.
Acta Paediatr ; 111(4): 845-849, 2022 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-34923666

RESUMO

AIM: Management of teenagers with primary spontaneous pneumothorax (PSP) is not consensual. We report our experience over an 11-year period. METHODS: For each patient under 20 years hospitalised with PSP from 2008 to 2018, demographic data, smoking habits, clinical presentation, hospitalisation unit, radiological management and its results, therapeutic management (observation, needle aspiration, chest tube drainage and surgery), complications, length of stay, given advice at discharge and recurrence were collected. RESULTS: Seventy patients were included in different paediatric or adult surgery or pulmonology wards (82.9% boys; 16.8 ± 1.7 years; one severe presentation; 18/58 smokers). Chest CT-scan (n = 42/70, 60%) revealed blebs/bullae in 18/39 examinations (46.2%). Treatment consisted of observation (14/70, 20%), needle aspiration (2/70, 2.9%), chest tube (53/70, 75.7%) and video-assisted thoracoscopy surgery (27/70, 38.6%). Half patients with interventional procedure presented complications. A median of 10 chest X-rays was noted during a median stay of 8 days. Advice concerning sport practice, flying, smoking, etc., was variably delivered. PSP recurrence concerned 35/70 patients (50%) without identified predictive factors. CONCLUSION: Compared to recent recommendations of a more conservative approach, chest CT-scan and interventional strategy are overused in our teenagers with PSP. Observation, more or less needle aspiration, should be clearly the first-line treatments.


Assuntos
Pneumotórax , Adolescente , Adulto , Tubos Torácicos , Criança , Drenagem/métodos , Feminino , Humanos , Masculino , Pneumotórax/cirurgia , Recidiva , Estudos Retrospectivos , Cirurgia Torácica Vídeoassistida/métodos , Resultado do Tratamento
5.
BJU Int ; 124(5): 876-882, 2019 11.
Artigo em Inglês | MEDLINE | ID: mdl-30776193

RESUMO

OBJECTIVES: To report the clinical spectrum of genital defects diagnosed before birth, identify predictive factors for severe phenotypes at birth, and determine the rate of associated malformations. PATIENTS AND METHODS: A retrospective study (2008-2017) of 4580 fetuses, identified prenatally with abnormalities evaluated by our Reference Center for Fetal Medicine, included cases with fetal sonographic findings of abnormal genitalia or uncertainty of fetal sex determination. Familial, prenatal and postnatal data were collected via a standardised questionnaire. RESULTS: In all, 61 fetuses were included. The positive predictive value (PPV) of the prenatal diagnosis of genital defects was 90.1%. Most cases were 46,XY-undervirilized boys, 42 cases (68.8%), which included 29 with mid-penile or posterior hypospadias, nine with anterior hypospadias, and epispadias, micropenis, scrotal transposition, and buried penis (one each). In all, 46,XX-virilized girls were identified in seven cases (11.5%), which included four with congenital adrenal hyperplasia, two with isolated clitoromegaly, and one with ovotestis. Other defects included prune belly syndrome and persistent cloaca (six cases). Early detection during the second trimester (58.1% vs 18.8%, P = 0.03), intra-uterine growth restriction (IUGR) (45.2% vs 9.1%, P = 0.06), and curvature of the penis (38.7% vs 0%, P = 0.02), were more frequently related to severe defects in male newborns. Associated malformations (14 cases, 22.9%) and genetic defects (six) were frequent in undervirilized boys. CONCLUSION: Prenatal imaging of genital defects leads to a wide range of phenotypes at birth. Its PPV is high and extra-urinary malformations are frequent. Early diagnosis during the second trimester, associated IUGR, and curvature of the genital tubercle, should raise suspicion of a severe phenotype and may justify delivery near a multidisciplinary disorders/differences of sex development team.


Assuntos
Doenças dos Genitais Masculinos , Ultrassonografia Pré-Natal , Feminino , Feto/diagnóstico por imagem , Doenças dos Genitais Masculinos/congênito , Doenças dos Genitais Masculinos/diagnóstico por imagem , Doenças dos Genitais Masculinos/patologia , Humanos , Masculino , Pênis/anormalidades , Pênis/diagnóstico por imagem , Pênis/patologia , Gravidez , Estudos Retrospectivos
6.
J Pediatr Gastroenterol Nutr ; 68(4): 527-532, 2019 04.
Artigo em Inglês | MEDLINE | ID: mdl-30444834

RESUMO

OBJECTIVES: In Hirschsprung disease (HD), despite successful surgical treatment, 50% of children experience long-term functional gastrointestinal problems, particularly chronic functional obstructive symptoms. We report our experience regarding clinical effects of neurostimulation-guided anal intrasphincteric botulinum toxin (BT) injections on postoperative obstructive symptoms attributed to a nonrelaxing anal sphincter complex in HD patients. METHODS: In this monocenter cohort study, 15 HD patients with postoperative functional intestinal obstructive symptoms received neurostimulation-guided anal intrasphincteric BT injections. Short-, medium-, and long-term effects were evaluated. The Bristol stool form scale was used to assess stool consistency, and the Jorge-Wexner (JW) score to assess fecal continence. RESULTS: The median age at first injection was 4 years. In the short-term, a significant improvement in stool consistency was noted in 12 of 14 patients (P = 0.0001) and JW score decreased for 14 of 15 patients (P = 0.001). In the medium-term, JW score significantly decreased for all patients (P = 0.0001), with an improvement of 50% or more for 10 patients (66.7%). In the long term, 83.3% of patients had normal stool consistency and JW score was <3 for all. Recurrent enterocolitis decreased from 86.7% to 8.3%. A complete resolution of all symptoms without further medication was observed in 66.7% of patients in the long term. CONCLUSIONS: Intrasphincteric BT injection was a safe, effective, and durable option for the management of postoperative functional intestinal obstructive symptoms in HD. The use of neurostimulator guidance for specific delivery of BT to muscular fibers of nonrelaxing anal sphincter complex takes into consideration the variability of patient's anatomy secondary to curative surgery.


Assuntos
Canal Anal/fisiopatologia , Toxinas Botulínicas Tipo A/uso terapêutico , Doença de Hirschsprung , Obstrução Intestinal/tratamento farmacológico , Toxinas Botulínicas Tipo A/administração & dosagem , Criança , Pré-Escolar , Estudos de Coortes , Feminino , Humanos , Lactente , Injeções Intralesionais , Masculino , Complicações Pós-Operatórias/tratamento farmacológico , Resultado do Tratamento
7.
J Urol ; 200(4): 890-894, 2018 10.
Artigo em Inglês | MEDLINE | ID: mdl-29723568

RESUMO

PURPOSE: While familial forms of complex disorders/differences of sex development have been widely reported, data regarding isolated hypospadias are sparse and a family history is thought to be less frequent. We aimed to determine the frequency of hypospadias in families of boys with hypospadias, to establish whether these familial forms exhibit a particular phenotype and to evaluate the prevalence of genetic defects of the main candidate genes. MATERIALS AND METHODS: A total of 395 boys with hypospadias were prospectively screened for a family history with a standardized questionnaire, extensive clinical description, family tree and sequencing of AR, SF1, SRD5A2 and MAMLD1. RESULTS: Family history of hypospadias was more frequent than expected (88 patients, 22.3%). In 17 instances (19.3%) familial hypospadias cases were multiple. Familial hypospadias was related to the paternal side in 59.1% of cases, consisting of the father himself (30.7%) as well as paternal uncles and cousins. Premature birth, assisted reproductive techniques, other congenital abnormalities and growth retardation were not more frequent in familial hypospadias than in sporadic cases. The severity of phenotype was similar in both groups. The results of genetic analysis combined with previous data on androgen receptor sequencing revealed that familial cases more frequently tend to demonstrate genetic defects than sporadic cases (5.68% vs 1.63%, p = 0.048). CONCLUSIONS: Familial forms of hypospadias are far more frequent than previously reported. Even minor and isolated forms justify a full clinical investigation of the family history. Detecting these hereditary forms may help to determine the underlying genetic defects, and may improve followup and counseling of these patients.


Assuntos
Predisposição Genética para Doença/epidemiologia , Hipospadia/epidemiologia , Hipospadia/genética , Linhagem , 3-Oxo-5-alfa-Esteroide 4-Desidrogenase/genética , Pré-Escolar , Seguimentos , Humanos , Incidência , Lactente , Masculino , Programas de Rastreamento/métodos , Estudos Prospectivos , Receptores Androgênicos/genética
8.
Surg Radiol Anat ; 40(8): 963-965, 2018 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-29785678

RESUMO

Ectopic pancreas also known as heterotopic pancreas (HP) is a rare congenital anomaly, mainly found as incidental finding during autopsy or abdominal exploration for an other condition. Incidence rate is probably underestimated as patients are mostly asymptomatic; otherwise, it is capable of producing symptoms depending on its location, size, often appearing in the 4th-to-6th decades. Complications such as inflammation, obstruction, bleeding, and malignancy degeneration must be considered. Pediatrics cases are very rare, generally concerning HP within Meckel's diverticulum, manifesting by gastrointestinal bleeding and intussusception. We report a rare case of jejunum bleeding, due to an isolated HP in a 15-year-old adolescent. Endoscopic and computed tomographic scan were normal, in particular did not found Meckel's diverticulum. Diagnosis and treatment have been apprehended performing a laparoscopic exploration. It is a singular location for HP, predominantly found in upper gastrointestinal tractus. So far, there have been no case reports of jejunal bleeding from ectopic pancreas without Meckel's diverticulum in children.


Assuntos
Coristoma/diagnóstico , Hemorragia Gastrointestinal/etiologia , Doenças do Jejuno/diagnóstico , Pâncreas , Adolescente , Coristoma/complicações , Coristoma/patologia , Coristoma/cirurgia , Colonoscopia , Diagnóstico Diferencial , Hemorragia Gastrointestinal/diagnóstico , Humanos , Doenças do Jejuno/complicações , Doenças do Jejuno/patologia , Doenças do Jejuno/cirurgia , Laparoscopia , Masculino , Divertículo Ileal/diagnóstico , Tomografia Computadorizada por Raios X
9.
Prenat Diagn ; 37(7): 666-672, 2017 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-28453880

RESUMO

OBJECTIVE: The objective of this study was to evaluate the accuracy of fetal diffusion-weighted magnetic resonance imaging with apparent diffusion coefficient (ADC) determination to predict postnatal renal function (nadir creatinine at 1 year and eGFR) of men with posterior urethral valves (PUV). METHODS: Between 2003 and 2014, 11 MRI were performed on fetuses (between 28 and 32 weeks) in whom second trimester sonography suggested severe bilateral urinary tract anomalies, suspected of PUV. RESULTS: The ADC of the 11 fetuses ranged from 1.3 to 2.86 mm2  s-1 (median = 1.79 mm2  s-1 , normal range for fetal kidney: 1.1-1.8). Two pregnancies with ADC > 2.6 mm2  s-1 were interrupted; the autopsy confirmed PUV and Potter syndrome. For the remaining nine babies, the follow-up was 5.4 years (0.8-10). Four children with abnormal ADC (1.8-2.3) had chronic kidney disease. The remaining five cases with normal nadir creatinine and eGFR had normal ADC. One case with unilateral elevated ADC had a poor ipsilateral renal function on dimercaptosuccinic acid scan. CONCLUSION: Here, it seems that diffusion-weighted magnetic resonance imaging with ADC determination could be useful in accurately evaluating fetal kidneys in PUV and predicting renal function. It may be an additional, non-invasive method when biologic and sonographic findings are inconclusive, especially in the case of oligohydramnios. Further studies are needed to confirm our data. © 2017 John Wiley & Sons, Ltd.


Assuntos
Imagem de Difusão por Ressonância Magnética/métodos , Uretra/anormalidades , Anormalidades Urogenitais/diagnóstico por imagem , Feminino , Humanos , Recém-Nascido , Masculino , Gravidez , Ultrassonografia Pré-Natal , Uretra/diagnóstico por imagem
10.
J Pediatr Urol ; 19(3): 248.e1-248.e6, 2023 06.
Artigo em Inglês | MEDLINE | ID: mdl-36746718

RESUMO

INTRODUCTION: To compare the efficacy, side effects, and cost-effectiveness between a single-use digital flexible ureteroscope and a reusable flexible ureteroscope in the treatment of paediatric renal stones. METHODS: This analytic, case-control, monocentric study included all patients undergoing flexible ureterosopies for stone treatment. Between April 2016 and February 2019, a reusable (Flex-XC®, Karl Storz) flexible ureteroscope was used (control group), whereas a single-use (Uscope®, PUSEN Medical©) flexible ureteroscope was used in all procedures from March 2019 to April 2021. Clinical and procedural outcomes, operative times, complication rates, hospital stay, and costs per procedure were evaluated. RESULTS: Forty-three cases using a reusable flexible ureteroscope and thirty-nine using a single-use flexible ureteroscope were included in the study. Demographic patient characteristics, stone burden, location and composition, preoperative presence of a double-J stent, procedural outcomes, mean length of postoperative hospital stay, and complications (4.6% versus 5%, p = 0.81) were comparable between the two groups. Median operative duration for stone removal was 93 min (20-170) with reusable versus 81 min (55-107) with the single-use scope (p = 0.18). Scope failure occurred four times with the reusable scope and in no case with the single-use. The total cost per procedure associated with the use of single-use scopes (798 Euros) was lower than a reusable scope (1483.23 Euros). DISCUSSION: Single-use flexible ureteroscopes were created to bypass the problems incurred when reusable scopes were damaged and therefore not available for use in surgical procedures. Single-use flexible ureteroscopes are always immediately available and ready to be used, even in urgent cases, as they typically do not require maintenance or sterilization. Compared with their reusable counterparts, single-use flexible ureteroscopes have similar digital performance (270°), image quality and we found no difference in the success and complication rates. Cost analysis of a reusable flexible ureteroscope must consider the purchase price, maintenance and repair costs, and decontamination costs (including handling, detergent, bacterial culture, transportation, and storage costs). In contrast, only purchase price is included in cost analysis for single-use flexible ureteroscopes. Our study suggests that single-use flexible ureteroscopes may be associated with lower costs per procedure than their reusable counterparts. CONCLUSION: Single-use flexible ureteroscopes are an interesting alternative to their reusable counterparts, particularly in terms of material resource management. Cost analyses conducted using a low volume of cases representative of a paediatric urology division favour the use of single-use ureteroscopes.


Assuntos
Cálculos Renais , Urolitíase , Humanos , Criança , Ureteroscópios , Ureteroscopia/métodos , Desenho de Equipamento , Cálculos Renais/cirurgia
12.
J Pediatr Urol ; 18(3): 367.e1-367.e7, 2022 06.
Artigo em Inglês | MEDLINE | ID: mdl-35477665

RESUMO

INTRODUCTION: Refinements in endoscopic instrumentation, the widespread popularization of endourology and the minimal invasiveness of endoscopic approaches have led to evolving interest in expanding applications for their use and now include incision of posterior urethral valves (PUV). We aimed to report our paediatric experience of PUV incision with Holmium:YAG laser updating of the endoscopic technique, how we set parameters for the laser energy and provide some tips and tricks to increase the likelihood of completing treatment. METHODS: A monocentric, prospective, continuous series of boys with PUV were treated endoscopically using a Holmium: YAG laser (1.2 J, 20 Hz, 800 µs). Feasibility was evaluated using operative time in minutes, spontaneous normal micturition after bladder catheter removal, and the duration of bladder catheterization in days in the absence of satisfactory micturition. Peri-operative complications were recorded. A VCUG was performed at 6 weeks postoperatively to exclude residual valves. RESULTS: Since September 2018, 18 children with PUV were included. The median age at the time of endoscopic laser incision was 12 days (1 day-5 years). The median operative duration was 28 min (17-35). The urinary catheter was systematically removed on the first postoperative day. There were no intraoperative or anaesthesia-related complications. More specifically, no urethral injuries and no bleeding were recorded. No incomplete VUP incision was found on follow-up VCUG, and no endoscopic revision was necessary thus far, with a median follow-up of 44 months (6 months-60 months). DISCUSSION: The use of the Holmium: YAG laser introduces new perspectives in the treatment of PUV. Its mechanism of action is considered a photothermic effect with a vapourization effect. The laser energy released by the Holmium: YAG source has a short tissue penetration distance and is strongly absorbed in an aqueous environment and therefore limits thermal tissue damage and favours early tissue re-epithelialization, reducing the risk of urethral stricture and decreasing postoperative oedema. The use of the laser in "incision" mode is the setting that most solicits the capacities of the laser (high energy, high frequency, and long pulse). The use of laser energy has the advantage of allowing tissue vapourization while ensuring maximal haemostasis and the possibility of introducing the laser fibre through the working channels of small, 6-Fr paediatric endoscopes. CONCLUSION: In our experience, endoscopic PUV incision using the Holmium: YAG laser appears to be a safe and efficient technique.


Assuntos
Terapia a Laser , Lasers de Estado Sólido , Obstrução Uretral , Criança , Hólmio , Humanos , Terapia a Laser/métodos , Lasers de Estado Sólido/uso terapêutico , Masculino , Estudos Prospectivos , Resultado do Tratamento , Obstrução Uretral/etiologia , Obstrução Uretral/cirurgia
13.
Exp Dermatol ; 20(5): 401-7, 2011 May.
Artigo em Inglês | MEDLINE | ID: mdl-21355886

RESUMO

Touch is detected through receptors located in the skin and the activation of channels in sensory nerve fibres. Epidermal keratinocytes themselves, however, may sense mechanical stimulus and contribute to skin sensation. Here, we showed that the mechanical stimulation of human keratinocytes by hypo-osmotic shock releases adenosine triphosphate (ATP) and increases intracellular calcium. We demonstrated that the release of ATP was found to be calcium independent because emptying the intracellular calcium stores did not cause ATP release; ATP release was still observed in the absence of external calcium and it persisted on chelating cytosolic calcium. On the other hand, the released ATP activated purinergic receptors and mobilized intracellular calcium stores. The resulting depletion of stored calcium led to the activation of capacitative calcium entry. Increase in cytosolic calcium concentration was blocked by the purinergic receptor blocker suramin, phospholipase C inhibitor and apyrase, which hydrolyses ATP. Collectively, our data demonstrate that human keratinocytes are mechanically activated by hypo-osmotic shock, leading first to the release of ATP, which in turn stimulates purinergic receptors, resulting in the mobilization of intracellular calcium and capacitative calcium entry. These results emphasize the crucial role of ATP signalling in the transduction of mechanical stimuli in human keratinocytes.


Assuntos
Trifosfato de Adenosina/metabolismo , Queratinócitos/fisiologia , Mecanotransdução Celular/fisiologia , Adenosina Trifosfatases/antagonistas & inibidores , Trifosfato de Adenosina/análogos & derivados , Trifosfato de Adenosina/farmacologia , Canais de Cálcio/metabolismo , Sinalização do Cálcio/fisiologia , Células Cultivadas , Quelantes/farmacologia , Ácido Egtázico/análogos & derivados , Ácido Egtázico/farmacologia , Gadolínio/farmacologia , Humanos , Canais Iônicos/antagonistas & inibidores , Canais Iônicos/metabolismo , Queratinócitos/efeitos dos fármacos , Mecanotransdução Celular/efeitos dos fármacos , Pressão Osmótica/fisiologia , Estimulação Física , Antagonistas do Receptor Purinérgico P2Y/farmacologia , Receptores Purinérgicos P2Y/metabolismo , Tapsigargina/farmacologia , Canais de Potencial de Receptor Transitório/agonistas , Canais de Potencial de Receptor Transitório/metabolismo , Fosfolipases Tipo C/antagonistas & inibidores , Fosfolipases Tipo C/metabolismo
14.
Ann Thorac Surg ; 112(5): 1553-1558, 2021 11.
Artigo em Inglês | MEDLINE | ID: mdl-32599038

RESUMO

BACKGROUND: The natural evolution of bronchogenic cysts (BCs) is unpredictable. Although most surgeons agree that symptomatic BCs should be resected, questions remain regarding the optimal management of asymptomatic mediastinal cysts. We present a case series of BCs to compare patients who underwent preventive operation with those who underwent surgical procedure after symptom onset. METHODS: This 15-year multicenter retrospective study included 114 patients (32 children and 82 adults). Data on clinical history, pathology, mean hospital stay, intraoperative and postoperative complications, and associated intraoperative procedures were analyzed separately for symptomatic and asymptomatic patients. RESULTS: A total of 53 asymptomatic patients (46.5%) were compared with 61 symptomatic patients (53.5%). There were significantly more adults in the symptomatic group than in the asymptomatic group (48 vs 34 patients, P < .05). A thoracoscopic approach was used in 88 patients (77%), with 7 conversions to thoracotomy (9%), all in symptomatic patients. There were significantly more additional procedures (20% vs 4%, P = .01) and more intraoperative complications (20% vs 4%, P = .01) in symptomatic patients, but postoperative complications between symptomatic and asymptomatic patients were similar. The postoperative length of stay was significantly longer in symptomatic patients (5.71 days vs 4 days, P < .001). Pathologic examination found significantly more inflammatory reactions in symptomatic patients. CONCLUSION: Early surgical management of BCs may be recommended to prevent symptomatic complications, which are unpredictable and whose management is more complicated in advanced BCs. Surgery can be performed with a thoracoscopic approach, which is easier and safer when the cyst is small and uncomplicated.


Assuntos
Doenças Assintomáticas , Cisto Broncogênico/cirurgia , Adolescente , Adulto , Idoso , Cisto Broncogênico/diagnóstico , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Complicações Intraoperatórias/epidemiologia , Masculino , Pessoa de Meia-Idade , Complicações Pós-Operatórias/epidemiologia , Estudos Retrospectivos , Resultado do Tratamento , Adulto Jovem
15.
J Pediatr Surg ; 55(8): 1481-1487, 2020 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-32063371

RESUMO

PURPOSE: To determine the quality of life and neuropsychological development of school-aged children with Hirschsprung's disease. METHODS: In this observational monocentric study, a multidisciplinary team prospectively assessed quality of life, neuropsychometric development and bowel functional outcomes. This study was registered on ClinicalTrial.gov (NCT03406741). Kidscreen and VSP-A questionnaires assessed the quality of life and were compared to the reference population (Eurostat database). Intelligence, attention and executive functions, perceptual organization and memory were evaluated using the Wechsler Children's Intelligence Scale, the NEuroPSYchological assessment, and the Rey figure test. Bowel functional outcomes were obtained using the Krickenbeck score. RESULTS: Fifteen patients were included, with a mean age of 10.25 years. The children's Kidscreen-assessed quality of life index was higher than the reference population (p = 0.01). The Full-Scale Intelligent Quotient was dissociated in 64% of children. The Perceptional Reasoning Index and the Processing Speed Index were observed at lower levels. There were no disturbances in executive functions. A satisfactory bowel functional outcome was noted in 46.7% of children. CONCLUSION: Children with Hirschsprung's disease have been shown to have subtle decreased performances in some areas of intelligence. Performing a neuropsychological assessment upon entering elementary school could help to detect these specific learning disabilities. LEVELS OF EVIDENCE: Level II, prognosis study.


Assuntos
Desenvolvimento Infantil/fisiologia , Doença de Hirschsprung , Testes Neuropsicológicos , Qualidade de Vida , Criança , Doença de Hirschsprung/fisiopatologia , Doença de Hirschsprung/psicologia , Humanos , Inquéritos e Questionários
16.
J Laparoendosc Adv Surg Tech A ; 19 Suppl 1: S245-7, 2009 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-19215209

RESUMO

PURPOSE: In this paper, we discuss the value of the laparoscopic ipsilateral ureteroureterostomy for duplication anomalies of the urinary tract in children. MATERIALS AND METHODS: A laparoscopic retroperitoneal ureteroureterostomy was undertaken in 2 children with 3-mm instruments and a 30-degree 5-mm optical trocar. Cystoscopy and stent placement in the recipient ureter was performed at the beginning of each procedure. The end-to-side anastomosis was performed with running 6:0 sutures. The type of procedure, position of operating devices, postoperative stenting, hospital stay, and outcome were recorded; each child had a control ultrasound at 6 months of follow-up. RESULTS: The two operations were performed on girls. Patients were 8 and 18 months of age, respectively. Diagnosis was, in each case, obstructed upper pole ureter, without reflux and with a functioning upper pole. Operative time was 120 minutes. There were no intra- and no postoperative complications. Patients were discharged home after 4 and 7 days. Ultrasound at 6 months after the operations showed an important decrease of the ureterohydronephrosis in one case and a complete disappearance of any dilatation in the second case. Parenchyma of the upper poles were considered as normal. CONCLUSION: Initial results of the minimally invasive ipsilateral ureteroureterostomy were excellent, with a minimal requirement of analgesia and perfect cosmetic results. Long-term follow-up, concentrating on the absence of urinary infection and of destruction of the upper poles, is mandatory.


Assuntos
Laparoscopia , Ureter/anormalidades , Ureter/cirurgia , Ureterostomia/métodos , Feminino , Humanos , Lactente , Procedimentos Cirúrgicos Urológicos/métodos
17.
Paediatr Anaesth ; 18(6): 532-8, 2008 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-18363623

RESUMO

BACKGROUND: Few data report remifentanil use in the neonatal population. We described here our experience with remifentanil-sevoflurane balanced anesthesia in neonates and children less than 2 years who underwent general anesthesia for abdominal surgery. METHODS: We retrospectively studied the pattern of remifentanil infusion associated with sevoflurane inhalation in preterm neonates (PTN; n = 18) (born before 37 weeks of gestation and <45 weeks of postmenstrual age), full-term neonates (FTN; n = 21) (born after 37 weeks of gestation and less than 29 days old) and older children up to 2 years (CUT; n = 24). We recorded heart rate (HR), mean arterial pressure (MAP), mean remifentanil dose and sevoflurane concentration before incision and at 5, 10, 20, 30, 45, 60, 90, and 105 min after incision. RESULTS: We observed that remifentanil doses used during surgery were lower in PTN than in both FTN and CUT and lower in FTN than in CUT. This was because of a progressive decrease in remifentanil dose during anesthesia in PTN and FTN. Conversely, remifentanil doses increased in CUT during anesthesia. Sevoflurane concentrations were higher in CUT group than in PTN and FTN groups. MAP and HR did not vary in the three groups during anesthesia. CONCLUSIONS: Remifentanil-sevoflurane anesthesia can be used for general anesthesia in neonates. We observed that anesthetists used lower doses of remifantanil and lower concentrations of sevoflurane in neonates compared with the older children.


Assuntos
Analgésicos Opioides , Anestesia Geral , Anestésicos Inalatórios , Éteres Metílicos , Piperidinas , Abdome/cirurgia , Analgésicos Opioides/administração & dosagem , Analgésicos Opioides/metabolismo , Análise de Variância , Anestésicos Inalatórios/administração & dosagem , Anestésicos Inalatórios/metabolismo , Pressão Sanguínea/efeitos dos fármacos , Relação Dose-Resposta a Droga , Feminino , Frequência Cardíaca/efeitos dos fármacos , Humanos , Lactente , Recém-Nascido , Recém-Nascido Prematuro , Intubação Intratraqueal , Masculino , Éteres Metílicos/administração & dosagem , Éteres Metílicos/metabolismo , Piperidinas/administração & dosagem , Piperidinas/metabolismo , Remifentanil , Estudos Retrospectivos , Sevoflurano , Fatores de Tempo
18.
Surg Laparosc Endosc Percutan Tech ; 18(1): 89-91, 2008 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-18287994

RESUMO

The management of common bile duct stones in neonates has not been clearly fully standardized, although percutaneous image-guided washing of the bile duct is generally adopted as the first-line treatment. We report the case of a 10-week-old infant with a choledocholithiasis in whom anterograde sphincter balloon dilation was achieved by combining image-guided access and flexible gastroduodenoscopy. This mini-invasive strategy may be an alternative to surgery in cases of impacted choledocholithiasis resistant to bile duct washing.


Assuntos
Cateterismo , Coledocolitíase/terapia , Esfinterotomia Endoscópica , Colangiopancreatografia Retrógrada Endoscópica/instrumentação , Colangiopancreatografia Retrógrada Endoscópica/métodos , Colecistectomia Laparoscópica , Humanos , Lactente , Masculino
19.
J Invest Dermatol ; 138(4): 946-956, 2018 04.
Artigo em Inglês | MEDLINE | ID: mdl-29138055

RESUMO

In the skin, Merkel cells connect with keratinocytes and Aß nerve fibers to form a touch receptor that functions as a slow adapting mechanoreceptor (slow adapting type 1). In human and mouse Merkel cells, we observed an increased concentration of intracellular Ca2+ ions in response to cold temperature and transient receptor potential melastatine 8 (TRPM8) ion channel agonists. A reduction in the response to cooling and TRPM8 agonists occurred after the addition of TRPM8 antagonists, as well as in TRPM8 knockout mice. Cold temperature and TRPM8 agonists also induced a current that was inhibited by a TRPM8 antagonist. Our results indicate that Merkel cells sense cooling through TRPM8 channels. We hypothesized that cooling modulates the slow adapting type 1 receptor response. Cooling mouse skin to 22°C reduced the slow adapting type 1 receptor discharge frequency. Interestingly, we observed no such reduction in TRPM8 knockout mice. Similarly, in human skin, a temperature of 22°C applied to the slow adapting type 1 receptive field reduced the spiking discharge. Altogether, our results indicate that Merkel cells are polymodal sensory cells that respond to mild cold stimuli through the activation of TRPM8 channels. Thermal activation of Merkel cells, and possibly other TRPM8-expressing non-neuronal cells, such as keratinocytes, potentially adapts the discharge of slow adapting type 1 receptors during cooling.


Assuntos
Regulação da Expressão Gênica , Células de Merkel/metabolismo , RNA Mensageiro/genética , Canais de Cátion TRPM/genética , Animais , Células Cultivadas , Temperatura Baixa , Humanos , Queratinócitos/citologia , Queratinócitos/metabolismo , Mecanorreceptores/metabolismo , Células de Merkel/citologia , Camundongos , Camundongos Knockout , Modelos Animais , Canais de Cátion TRPM/biossíntese
20.
Prog Urol ; 17(5): 987-91, 2007 Sep.
Artigo em Francês | MEDLINE | ID: mdl-17969803

RESUMO

INTRODUCTION: The objective of this study was to analyse the postoperative results of conservative management of functional ectopic ureter on duplicated collecting system by low uretero-ureterostomy in children. MATERIALS AND METHOD: The medical records of children with duplicated collecting system and a functional ectopic ureter admitted to our department between 2001 and 2005 were reviewed. A postnatal radiological assessment comprising urinary tract ultrasound, retrograde cystography, intravenous urography and DMSA renal scintigraphy was performed. In each patient, the operative technique consisted of low uretero-ureterostomy with end-to-side uretero-ureteric anastomosis. RESULTS: This series comprised 3 girls and 2 boys. The ectopic ureter was located on the left side in all patients. Postnatal ultrasound confirmed the presence of dilatation of the upper collecting system and the retrovesical ureter estimated at 10 +/- 2 mm in 3 cases; in one case, dilatation of the upper collecting system was estimated at 25 mm and the retrovesical ureter was estimated at 16 +/- 2 mm; in the last patient, dilatation of the upper collecting system above the retrovesical ureter was estimated at 6.5 mm. Mean renal function of the upper pole (DMSA) was 23 +/- 7%. The mean age at the time of the operation was 8 months. The immediate postoperative course was uneventful in all patients. Mean follow-up was 38 months (range: 18 months to 4.5 years). All children were asymptomatic and a reduction of collecting system and ureteric dilatation was confirmed on follow-up ultrasound at 10 months in all patients. CONCLUSION: Low uretero-ureteric anastomosis is an alternative to high collecting system anastomosis or ureteric reimplantation in the presence of a functional ectopic ureter on duplicated collecting system in children.


Assuntos
Ureter , Ureterostomia/métodos , Anastomose Cirúrgica , Criança , Coristoma/diagnóstico por imagem , Feminino , Humanos , Masculino , Pielonefrite/etiologia , Estudos Retrospectivos , Ultrassonografia
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