Your browser doesn't support javascript.
loading
Mostrar: 20 | 50 | 100
Resultados 1 - 20 de 27
Filtrar
1.
Pediatr Emerg Care ; 2024 May 15.
Artículo en Inglés | MEDLINE | ID: mdl-38743405

RESUMEN

BACKGROUND: Isolated fallopian tube torsion (IFTT) is very rare gynecological emergency in pediatric population. Our objective is to assess treatment options and discuss outcome of a cohort of IFTT with a focus on the association between IFTT and hydrosalpinx (HSX). METHODS: A retrospective review was conducted. Pediatric patients with IFTT operated in the same center were included. RESULTS: Seventeen girls (aged: 11-16 years) were managed for acute abdominal pain between 2008 and 2018, with intraoperative diagnosis of IFTT. All patients underwent laparoscopic exploration, with laparoscopically fallopian tube detorsion in all patients. Based on the association of IFTT with HSX after fallopian tube detorsion, patients were divided into 2 groups: group 1 (IFTT without HSX; 12 girls) and group 2 (IFTT with HSX; 5 girls). During the same surgery, complementary surgical procedures were done. In group 1: salpingectomies (4), partial salpingectomies (2) and cystectomies (6) were done. In group 2: salpingectomy (1), salpingotomy (1), and cyst ablation (1). The treatment was called conservative when the tube was preserved.Follow-up was uneventful in group 1. In group 2, for all patients with initial fallopian tube preservation, further surgical procedures were necessary (1-4 surgeries/patient), and, finally, another 3 patients required salpingectomy. CONCLUSIONS: Conservative treatment with tube preservation of IFTT without HSX appeared to be beneficial compared to those with HSX, with no recurrence of torsion or symptoms during the follow-up. However, the same conservative treatment was not sufficiently effective for IFTT with HSX and required further procedures due to recurrence of torsion. LEVEL OF EVIDENCE: IV.

2.
Pediatr Radiol ; 53(9): 1911-1918, 2023 08.
Artículo en Inglés | MEDLINE | ID: mdl-37171639

RESUMEN

BACKGROUND: One of the main limitations of 99mtechnetium-dimercaptosuccinic acid (DMSA) scan is the long acquisition time. OBJECTIVE: To evaluate the feasibility of short DMSA scan acquisition times using a cadmium-zinc-telluride-based single-photon emission computed tomography (SPECT) system in children. MATERIALS AND METHODS: The data of 27 children (median age: 4 years; 16 girls) who underwent DMSA SPECT were retrospectively analyzed. Both planar and SPECT DMSA were performed. SPECT images were analyzed using coronal-simulated planar two-dimensional images. A reduction in SPECT acquisition time was simulated to provide 4 series (SPECT-15 min, SPECT-10 min, SPECT-5 min and SPECT-2.5 min). A direct comparison of the planar and SPECT series was performed, including semi-quantification reproducibility, image quality (mean quality score on a scale of 0 to 2) and inter- and intra-observer reproducibility of the scintigraphic patterns. RESULTS: The overall image quality score (± standard deviation) was 1.3 (± 0.6) for the planar data set, 1.6 (± 0.5) for the SPECT-15 min data set, 1.4 (± 0.5) for the SPECT-10 min data set, 1.0 (± 0.5) for the SPECT-5 min data set and 0.6 (± 0.6) for the SPECT-2.5 min data set. Median Kappa coefficients for inter-observer agreement between planar and SPECT images were greater than 0.83 for all series and all readers except one reader for the SPECT-2.5 min series (median Kappa coefficient = 0.77). CONCLUSION: Shortening SPECT acquisitions to 5 min is feasible with minimal impact on images in terms of quality and reproducibility.


Asunto(s)
Ácido Dimercaptosuccínico de Tecnecio Tc 99m , Tomografía Computarizada de Emisión de Fotón Único , Niño , Femenino , Humanos , Preescolar , Reproducibilidad de los Resultados , Estudios Retrospectivos , Tomografía Computarizada de Emisión de Fotón Único/métodos
3.
J Pediatr Urol ; 19(3): 347-348, 2023 06.
Artículo en Inglés | MEDLINE | ID: mdl-36872172

RESUMEN

PURPOSE: To present one way of performing onlay preputial flap for hypospadias. PATIENTS AND METHODS: This procedure was performed following the methodology used in one hypospadias expert center to correct hypospadias in boys who are not elective for Koff procedure and in whom Koyanagi procedure is not needed. Operative details were described, and post-operative management were given as example. RESULTS: Long-term results of this technique showed a 10% complication rate (dehiscence, strictures or urethral fistulas) 2 years after surgery. CONCLUSIONS: This video is a step by step description of the onlay preputial flap technique giving the general methodology and also the details resulting from years of practice in one hypospadias expert center.


Asunto(s)
Hipospadias , Masculino , Humanos , Lactante , Hipospadias/cirugía , Uretra/cirugía , Colgajos Quirúrgicos , Constricción Patológica/cirugía , Periodo Posoperatorio , Procedimientos Quirúrgicos Urológicos Masculinos/métodos
4.
Sci Rep ; 12(1): 13406, 2022 08 04.
Artículo en Inglés | MEDLINE | ID: mdl-35927301

RESUMEN

Renal pelvis dilatation (RPD) is diagnosed in utero on prenatal ultrasonography (US) and can resolve spontaneously. However, isolated RPD can also reflect ureteropelvic junction obstruction (UPJO), which requires surgical treatment to prevent progressive renal deterioration. The diagnosis of UPJO can only be confirmed after birth with repeat US and renal isotope studies. 1H Nuclear Magnetic Resonance spectroscopy (NMR) was performed on urine of newborns with prenatally diagnosed unilateral RPD and healthy controls to identify specific urinary biomarkers for UPJO. The original combination of EigenMS normalization and sparse partial-least-squares discriminant analysis improved selectivity and sensitivity. In total, 140 urine samples from newborns were processed and 100 metabolites were identified. Correlation network identified discriminant metabolites in lower concentrations in UPJO patients. Two main metabolic pathways appeared to be impaired in patients with UPJO i.e. amino acid and betaine metabolism. In this prospective study, metabolic profiling of urine samples by NMR clearly distinguishes patients who required surgery for UPJO from patients with transient dilatations and controls. This study will pave the way for the use of metabolomics for the diagnosis of prenatal hydronephrosis in clinical routine.


Asunto(s)
Hidronefrosis , Enfermedades Renales , Obstrucción Ureteral , Dilatación , Femenino , Humanos , Hidronefrosis/diagnóstico por imagen , Hidronefrosis/cirugía , Recién Nacido , Enfermedades Renales/patología , Pelvis Renal/patología , Embarazo , Diagnóstico Prenatal , Estudios Prospectivos , Espectroscopía de Protones por Resonancia Magnética , Tomografía Computarizada por Rayos X , Obstrucción Ureteral/diagnóstico por imagen
5.
Pediatr Transplant ; 26(6): e14329, 2022 09.
Artículo en Inglés | MEDLINE | ID: mdl-35655369

RESUMEN

BACKGROUND: The anti-CD20 rituximab is often used in the treatment of children with steroid-resistant nephrotic syndrome or EBV-induced post-transplant lymphoproliferative disorder. This single-center series reports the use of rituximab as induction therapy in pediatric kidney transplantation. METHODS: Four children who received rituximab as induction therapy for kidney transplantation since 2016 were retrospectively analyzed. Clinical and laboratory data were extracted from medical records. RESULTS: The patients (2 boys and 2 girls) were aged from 6.1 to 11.9 years and were treated with rituximab on the day of the transplantation procedure; all the transplants came from deceased donors. In all patients, rituximab was used because of positive EBV viral loads before kidney transplantation. Viral loads remained undetectable for the first 6 months after the transplantation procedure and remained below the 4.5 log threshold thereafter. After a median follow-up of 2.3 years, none of the patients displayed rejection or de novo donor-specific antibodies; the glomerular filtration rate remained above 70 ml/min/1.73 m2 . No post-transplant lymphoproliferative disorder was observed. CONCLUSION: The results suggest that rituximab can be used as induction therapy to prevent EBV replication and its complications in case of positive viral load prior to kidney transplantation.


Asunto(s)
Infecciones por Virus de Epstein-Barr , Trasplante de Riñón , Trastornos Linfoproliferativos , Niño , Femenino , Humanos , Quimioterapia de Inducción/efectos adversos , Trasplante de Riñón/efectos adversos , Trastornos Linfoproliferativos/tratamiento farmacológico , Trastornos Linfoproliferativos/etiología , Masculino , Estudios Retrospectivos , Rituximab/uso terapéutico
6.
Eur J Pediatr ; 180(12): 3555-3563, 2021 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-34165592

RESUMEN

As the epidemiology of urolithiasis is constantly evolving, analyzing the composition of stones is crucial to better understand the determinants of lithogenesis. The aim of this study was to describe the composition of stones of pediatric patients in a tertiary center. Clinical and metabolic data from all pediatric patients with at least one stone that was analyzed by Fourier transformed infrared spectroscopy (FTIR) in the Hospices Civils de Lyon between 2013 and 2017 were retrospectively collected. A total of 111 patients (sex ratio 1.4:1) were included; their median ([IQR]) age was 7.5 (3.1-10.5) years. The main component of stones was calcium oxalate (weddellite for 34 (31%) stones, whewellite 23 (21%)), calcium phosphate (carbapatite 32 (29%), brushite 6 (5%), amorphous calcium phosphate 3 (3%)), struvite 5 (5%), cystine 4 (4%), uric acid 2 (2%), and ammonium acid urate 2 (2%). A total of 20 (18%) stones were pure and 24 (22%) were infectious. Carbapatite stones were the most frequent in patients < 2 years and calcium oxalate stones in patients > 2 years old. Metabolic abnormalities (most frequently hypercalciuria) were found in 50% of tested patients and in 54% of patients with infectious stones. Congenital anomalies of the kidney and/or urinary tract (CAKUT) or neurogenic bladder were present in 9/24 (38%) patients with infectious stones and 12/16 (76%) patients with bladder stones.Conclusion: This study confirms that calcium oxalate stones are the most frequent among pediatric patients, which could reflect the nutritional habits of predisposed patients. In contrast, infectious stones are less frequent and occur mostly in association with anatomic or metabolic favoring factors.


Asunto(s)
Cálculos Urinarios , Urolitiasis , Niño , Preescolar , Cistina , Humanos , Estudios Retrospectivos , Centros de Atención Terciaria , Cálculos Urinarios/epidemiología
7.
Acta Paediatr ; 110(3): 781-788, 2021 03.
Artículo en Inglés | MEDLINE | ID: mdl-33373057

RESUMEN

AIM: Our aim was to look at the technical, ethical and global issues related to neonatal dialysis. METHODS: We performed a PubMed research on manuscripts published from March 2010 to March 2020 and retrospectively reviewed all neonates who received dialysis in our French paediatric and neonatal intensive care units from April 2009 to March 2019. RESULTS: Dialysis is performed on neonates with pre-existing renal diseases, acute kidney injuries or inborn errors of metabolism. It is required in 0.5%-1% of neonates admitted to the neonatal intensive care units. Peritoneal dialysis and extracorporeal blood purification are both feasible, with more complications, but the results are close to those obtained in older infants, at least in children without multi-organ dysfunction. Novel haemodialysis machines are being evaluated. Ethical issues are a major concern. Multidisciplinary teams should consider associated comorbidities, risks of permanent end-stage renal disease and provide parents with full and neutral information. These should drive decisions about whether dialysis is in child's best interests. CONCLUSION: Neonatal dialysis is technically feasible, but ethically challenging, and short-term and long-term data remain limited. Prospective studies and dialysis registries would improve global management and quality of life of these patients at risk of chronic kidney disease.


Asunto(s)
Fallo Renal Crónico , Diálisis Peritoneal , Anciano , Niño , Humanos , Lactante , Recién Nacido , Estudios Prospectivos , Calidad de Vida , Diálisis Renal , Estudios Retrospectivos
8.
Nephrol Ther ; 16(7): 424-430, 2020 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-33177015

RESUMEN

BACKGROUND: Little data are available for infants who started renal replacement therapy before 6 months of age. Because of extra-renal comorbidities and uncertain outcomes, whether renal replacement therapy in neonates is justified remains debatable. METHODS: We performed a retrospective analysis of all patients who began chronic peritoneal dialysis below 6 months between 2007 and 2017 in two tertiary centres. Results are presented as median (min;max). RESULTS: Seventeen patients (10 boys) were included (8 prenatal diagnoses, 6 premies), with the following diagnoses: congenital anomalies of kidney and urinary tract (n=9), oxalosis (n=5), congenital nephrotic syndrome (n=2) and renal vein thrombosis (n=1). Five patients had associated comorbidities. At peritoneal dialysis initiation, age was 2.6 (0.1;5.9) months, height-standard deviation score (SDS) -1.3 (-5.7;1.6) and weight-SDS -1.4 (-3.6;0.6). Peritoneal dialysis duration was 12 (2;32) months, and at peritoneal dialysis discontinuation height-SDS was -1.0 (-4.3;0.7) weight-SDS -0.7 (-3.2;0.2), parathyroid hormone 123 (44;1540) ng/L, and hemoglobin 110 (73;174) g/L. During the first 6 months of peritoneal dialysis, the median time of hospitalisation stay was 69 (15;182) days. Ten patients presented a total of 27 peritonitis episodes. Reasons for peritoneal dialysis discontinuation were switch to hemodialysis (n=6), transplantation (n=6), recovery of renal function (n=2) and death (n=1). After a follow-up of 4.3 (1.7;10.3) years, 12 patients were transplanted, 2 patients were still on peritoneal dialysis, 2 patients were dialysis free with severe chronic kidney disease and 1 patient had died. Seven patients displayed neurodevelopmental delay, of whom five needed special schooling. CONCLUSION: We confirm that most infants starting peritoneal dialysis before 6 months of age will be successfully transplanted and will have a favourable growth outcome. Their quality of life will be impacted by recurrent hospitalisations and neurodevelopmental delay is frequent.


Asunto(s)
Diálisis Peritoneal , Femenino , Estudios de Seguimiento , Humanos , Hiperoxaluria/terapia , Lactante , Recién Nacido , Riñón/anomalías , Trasplante de Riñón/estadística & datos numéricos , Masculino , Síndrome Nefrótico/congénito , Síndrome Nefrótico/terapia , Trastornos del Neurodesarrollo/etiología , Insuficiencia Renal Crónica/etiología , Venas Renales , Estudios Retrospectivos , Trombosis de la Vena/terapia
9.
J Pediatr Urol ; 16(6): 831.e1-831.e7, 2020 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-32980262

RESUMEN

BACKGROUND: Vaginal lesions are rare and of various types in children. Clinical presentation is generally undifferenciated. Histological examination is fundamental to ascertain the nature of the lesion. Regarding tumoral lesions, histological subtypes encountered are radically different from those seen in adults, dominated by stromal benign lesions. OBJECTIVE: The aim of this retrospective study was to describe characteristics and pathological aspects of pediatric vaginal lesions, diagnosed in a single pediatric experienced center. STUDY DESIGN: A database analysis was performed on all vaginal samples of patients under 18 years old received in a pediatric-specialized pathology laboratory of an academic hospital, over a 26-year period. RESULTS: Among 36 vaginal tissue samples reported, a total of 15 tumoral or pseudotumoral processes was recorded. Primitive malignant tumors included embryonal rhabdomyosarcoma (n = 3) and germ-cell tumors, yolk-sac type (n = 2). Benign tumoral or pseudotumoral processes included inflammatory stromal polyps (n = 8), epidermic cyst (n = 1), and benign Müllerian papilloma (n = 1). DISCUSSION: Over 15 primitive vaginal tumors, 1/3 was malignant with embryonal rhabdomyosarcoma being the most common. The remaining 2/3 specimens were benign, with stromal inflammatory lesions being the most commonly observed. Fibro-epithelial polyp is a debated entity, which covers a wide histological spectrum, with varying inflammation and stromal cellularity, raising sometimes the question of the differential diagnosis with rhabdomyosarcoma. Stromal cells morphology along with their immunohistochemical profile suggest their reactive myofibroblastic nature. Pseudotumoral inflammatory lesions display very similar histological findings with these entities. A common pathogenesis beginning with an inflammatory process, potentially accelerated by chronic traumatic factors, could be discussed. CONCLUSION: We confirmed the rarity and the diversity of vaginal lesions in children. Vaginoscopy and biopsy sample should be systematic, given the non-specific presentation of tumoral processes. Myogenin immunostain must be systematic in case of vaginal polypoid mass, in order to rule out malignancy.


Asunto(s)
Neoplasias de Células Germinales y Embrionarias , Rabdomiosarcoma , Neoplasias Vaginales , Adolescente , Adulto , Niño , Femenino , Humanos , Estudios Retrospectivos , Neoplasias Vaginales/diagnóstico , Neoplasias Vaginales/epidemiología
10.
Childs Nerv Syst ; 35(6): 957-963, 2019 06.
Artículo en Inglés | MEDLINE | ID: mdl-30915531

RESUMEN

BACKGROUND: Postnatal closure of a myelomeningocele remains the standard of care in many countries. The prenatal closure has given hope for decreasing the damage to the neural placode and has challenged classic management. However, this technique presents potential sources of complications. Patients with MMC with an anatomical level of L4 and below have a better functional prognosis than higher level malformations. Are they still candidates for prenatal surgery? OBJECTIVE: To evaluate outcome of MMC with an anatomical level of L4 and below and discuss, with support of the literature, the indications to perform prenatal closure in this particular group of patients. MATERIALS AND METHODS: Twenty-nine children were included in this observational study. The level of the vertebral malformation was sacral in 12 cases (41.4%) or lumbar (level ≤ L4) in 17 cases (58.6%). All the patients was operated postnatally for closure of the MMC with microsurgical technique as soon as possible after clinical evaluation (range 0-97 days). RESULTS: Only 11 out of 29 patients (37.9%) needed of a CSF diversion. A Chiari II malformation was present before MMC closure in 17 patients (58.6%) and only in 5 (17%) after. Twenty-six patients (89.7%) were able to walk. Seven (23%) and 16 (55%) of our patients have a normal bladder and bowel control, respectively. All school-aged children attend school. CONCLUSIONS: The functional outcome for low-level MMC is good when managed with modern microneurosurgical techniques with a low risk for the patient and the mother. Therefore, we do not suggest prenatal surgery for subgroup of infant with MM.


Asunto(s)
Meningomielocele/patología , Meningomielocele/cirugía , Femenino , Estudios de Seguimiento , Humanos , Lactante , Recién Nacido , Masculino , Procedimientos Neuroquirúrgicos/métodos , Resultado del Tratamiento
11.
Pediatr Transplant ; 22(2)2018 03.
Artículo en Inglés | MEDLINE | ID: mdl-29341372

RESUMEN

RTx remains challenging in children under 3 years of age. This single-center study reviewed the medical records of children <3 years transplanted since 1987 (N = 32, Group 1). They were matched for donor type and RTx period with children aged 3-13 years (N = 32, Group 2) and 13-18 years (N = 32, Group 3). There were no between-group significant differences regarding distributions of gender, primary renal disease, proportion of dialysis before RTx, and growth (SDS). Compared to Groups 2 and 3, Group 1 had more peritoneal dialyses (P < .001), more EBV mismatches (P = .04), and longer warm ischemia times (P < .001). The risk of graft loss was not significantly different among age groups (hazard ratio, 2.4 in Group 2 and 2.0 in Group 3 vs Group 1; P = .2). Death occurred in four patients (3 in Group 1 and 1 in Group 2) and graft loss occurred in 28 patients, mainly due to chronic allograft nephropathy. In recipients <3 years of age, the outcomes of RTx are close to those obtained in older pediatric age groups. Thus, young patients may be transplanted in experienced multidisciplinary teams without additional risks provided that particular attention is paid to donor selection and prevention/early diagnosis of comorbidities and complications.


Asunto(s)
Rechazo de Injerto/etiología , Supervivencia de Injerto , Trasplante de Riñón , Complicaciones Posoperatorias/etiología , Adolescente , Factores de Edad , Niño , Preescolar , Femenino , Rechazo de Injerto/epidemiología , Humanos , Lactante , Recién Nacido , Estimación de Kaplan-Meier , Trasplante de Riñón/mortalidad , Modelos Lineales , Estudios Longitudinales , Masculino , Evaluación de Resultado en la Atención de Salud , Complicaciones Posoperatorias/epidemiología , Modelos de Riesgos Proporcionales , Calidad de Vida , Estudios Retrospectivos , Medición de Riesgo , Factores de Riesgo
12.
J Proteome Res ; 16(10): 3732-3740, 2017 10 06.
Artículo en Inglés | MEDLINE | ID: mdl-28791867

RESUMEN

Improving the management of neonatal diseases and prevention of chronic diseases in adulthood requires a better comprehension of the complex maturational processes associated with newborns' development. Urine-based metabolomic studies play a promising role in the fields of pediatrics and neonatology, relying on simple and noninvasive collection procedures while integrating a variety of factors such as genotype, nutritional state, lifestyle, and diseases. Here, we investigate the influence of age, weight, height, and gender on the urine metabolome during the first 4 months of life. Untargeted analysis of urine was carried out by 1H-Nuclear Magnetic Resonance (NMR) spectroscopy for 90 newborns under 4 months of age, and free of metabolic, nephrologic, or urologic diseases. Supervised multivariate statistical analysis of the metabolic profiles revealed metabolites significantly associated with age, weight, and height, respectively. The tremendous growth occurring during the neonatal period is associated with specific modifications of newborns' metabolism. Conversely, gender appears to have no impact on the urine metabolome during early infancy. These results allow a deeper understanding of newborns' metabolic maturation and underline potential confounding factors in newborns' metabolomics studies. We emphasize the need to systematically and precisely report children age, height, and weight that impact urine metabolic profiles of infants.


Asunto(s)
Recien Nacido Prematuro/orina , Metaboloma/genética , Metabolómica , Proteínas/genética , Niño , Femenino , Edad Gestacional , Humanos , Lactante , Recién Nacido , Recien Nacido Prematuro/crecimiento & desarrollo , Espectroscopía de Resonancia Magnética , Masculino , Análisis Multivariante , Proteínas/metabolismo
13.
J Urol ; 197(4): 1144-1149, 2017 04.
Artículo en Inglés | MEDLINE | ID: mdl-27916713

RESUMEN

PURPOSE: We compared the prognostic value of anteroposterior intrasinus diameter of the renal pelvis, urinary tract dilatation and the Society for Fetal Urology grading system in children with prenatally diagnosed unilateral urinary tract dilatation. MATERIALS AND METHODS: All newborns with prenatally diagnosed unilateral urinary tract dilatation, normal bladder and anteroposterior intrasinus diameter 10 mm or greater on the first postnatal ultrasonography were prospectively enrolled from January 2011 to February 2015. Indications for surgery were recurrent febrile urinary tract infections and/or decrease of relative renal function more than 10% on serial isotope studies and/or increasing anteroposterior intrasinus diameter greater than 20% on serial ultrasounds. Sensitivity, specificity and ROC curves were calculated to evaluate the accuracy of anteroposterior intrasinus diameter, urinary tract dilatation and Society for Fetal Urology grading system in determining which children would need surgery within 24 months. RESULTS: A total of 57 males and 13 females were included. Of the patients 33 required surgery at a median age of 5 months (IQR 3.8 to 6.4). Urinary tract dilatation remained stable in 14 cases and decreased in 23 with a median followup of 42 months (IQR 25 to 67). Anteroposterior intrasinus diameter, urinary tract dilatation and Society for Fetal Urology scores were all correlated with the need for surgery. Anteroposterior intrasinus diameter with a threshold of 20 mm had the best prognostic value, with a sensitivity of 81.8% and a specificity of 91.7%. CONCLUSIONS: Our study confirms that the prognostic value was comparable between anteroposterior intrasinus diameter of the renal pelvis, urinary tract dilatation and Society for Fetal Urology grading system in newborns with prenatally diagnosed unilateral urinary tract dilatation. Anteroposterior intrasinus diameter and abnormal parenchymal thickness are the most important ultrasound criteria to identify children at risk for requiring surgery.


Asunto(s)
Hidronefrosis/diagnóstico por imagen , Pelvis Renal/diagnóstico por imagen , Dilatación Patológica/diagnóstico por imagen , Femenino , Humanos , Lactante , Masculino , Pronóstico , Estudios Retrospectivos , Ultrasonografía , Ultrasonografía Prenatal , Sistema Urinario/diagnóstico por imagen , Sistema Urinario/patología
14.
J Pediatr Urol ; 12(6): 347-351, 2016 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-27751834

RESUMEN

OBJECTIVE: The aim was to describe the clinical presentation and the surgical management of penile epispadias associated with a buried penis in five children. PATIENTS AND METHODS: This is a 5-year retrospective review of patients presenting with a buried penis, a congenital defect of the penile skin shaft associated with an unretractable foreskin for whom a penile epispadias was found at the time of surgery. All had undergone surgery combining a Cantwell-Ransley procedure and refashioning of the penile skin following the authors' technique. RESULTS: Three children had a glanular epispadias and two had a midshaft epispadias. Four had a satisfactory outcome, and one required a complementary urethroplasty for glanular dehiscence. CONCLUSION: Buried penis and epispadias are usually isolated congenital anomalies, although they can be associated. It is therefore recommended to warn parents about the possibility of underlying penile anomaly in children with buried penises and unretractable foreskin. Careful palpation of the dorsum of the glans through the foreskin looking for a dorsal cleft could indicate an associated epispadiac urethra. Surgical correction of both anomalies can be done at the same time. Parents of boys with buried penises should be warned that underlying penile anomaly may exist.


Asunto(s)
Anomalías Múltiples/cirugía , Epispadias/complicaciones , Pene/anomalías , Preescolar , Epispadias/patología , Humanos , Lactante , Recién Nacido , Masculino , Estudios Retrospectivos , Procedimientos Quirúrgicos Urológicos Masculinos/métodos
15.
Pediatr Nephrol ; 31(9): 1517-29, 2016 09.
Artículo en Inglés | MEDLINE | ID: mdl-27060059

RESUMEN

BACKGROUND: Experience in combined liver-kidney transplantation (CLKT) in children is limited. METHODS: We conducted a retrospective study of all pediatric CLKTs performed at our medical institution between 1992 and 2013. RESULTS: We identified 18 pediatric patients (9 girls) who had undergone CLKT at our institution during the study period. The median age [range] and body weight [range] of this patient group was 3.6 [1.0-18.6] years and 13 [10-40] kg, respectively; 11 patients weighed <15 kg at the time of CLKT. Indications for CLKT were primary hyperoxaluria (PH1; n = 14), association of hepatic fibrosis and end-stage renal disease (n = 3) and methylmalonic acidemia (n = 1). In the early postoperative period, eight patients required dialysis. Median stay in the pediatric intensive care unit was 10 [6-29] days. One patient died from cardiovascular disease 10 years after CLKT. There were no liver graft losses despite six acute liver rejection episodes, whereas four kidney grafts were lost. At last follow-up (6 [0.5-21] years) for patients with a functioning renal graft, the glomerular filtration rate was 71 [26-146] mL/min/1.73 m(2). In PH1 patients, urine oxalate normalized in six patients within 3 years after CLKT, but three patients still presented with elevated oxaluria at 1, 2 and 3 years after CLKT. CONCLUSIONS: Pediatric CLKT provides encouraging results in the long term, even in the youngest patients.


Asunto(s)
Trasplante de Riñón , Trasplante de Hígado , Adolescente , Niño , Preescolar , Femenino , Humanos , Lactante , Hígado , Masculino , Diálisis Renal , Estudios Retrospectivos , Resultado del Tratamiento
16.
Curr Opin Organ Transplant ; 20(5): 543-9, 2015 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-26270957

RESUMEN

PURPOSE OF REVIEW: The experience of combined liver-kidney transplantation (CLKT) is limited in pediatric populations. This strategy is, however, required in specific diseases such as metabolic diseases (namely primary hyperoxaluria type one and methylmalonic acidemia), autosomal recessive polycystic kidney disease, miscellaneous ciliopathies and atypical hemolytic uremic syndrome. RECENT FINDINGS: Different series and registry studies have confirmed the feasibility of pediatric CLKT with encouraging results in the long term, even in the youngest and smallest patients, provided that highly trained multidisciplinary teams are involved in this global management. As such, the long-term outcomes after CLKT are currently comparable to that of isolated liver or kidney transplantations, even though the immediate postoperative period remains challenging. SUMMARY: Some questions remain nevertheless unanswered, such as the respective place of combined versus sequential liver-kidney transplantation, especially in primary hyperoxaluria and autosomal recessive polycystic kidney disease. The aim of this review was therefore to provide a 2015 update on pediatric CLKT. In the future, international collaborative studies and registries may help to improve our knowledge of this rare and still highly challenging technique.


Asunto(s)
Enfermedades Renales/cirugía , Trasplante de Riñón , Hepatopatías/cirugía , Trasplante de Hígado , Niño , Humanos , Hiperoxaluria Primaria/cirugía , Enfermedades Renales/complicaciones , Trasplante de Riñón/métodos , Hepatopatías/complicaciones , Trasplante de Hígado/métodos , Sistema de Registros
17.
Curr Opin Organ Transplant ; 18(5): 555-62, 2013 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-23995376

RESUMEN

PURPOSE OF REVIEW: During childhood, growth retardation, decreased final height and renal osteodystrophy are common complications of chronic kidney disease (CKD). These problems remain present in patients undergoing renal transplantation, even though steroid-sparing strategies are more widely used. In this context, achieving normal height and growth in children after transplantation is a crucial issue for both quality of life and self-esteem. The aim of this review is to provide an overview of pathophysiology of CKD-mineral bone disorder (MBD) in children undergoing renal transplantation and to propose keypoints for its daily management. RECENT FINDINGS: In adults, calcimimetics are effective for posttransplant hyperparathyroidism, but data are missing in the pediatric population. Fibroblast growth factor 23 levels are associated with increased risk of rejection, but the underlying mechanisms remain unclear. A recent meta-analysis also demonstrated the effectiveness of rhGH therapy in short transplanted children. SUMMARY: In 2013, the daily clinical management of CKD-MBD in transplanted children should still focus on simple objectives: to optimize renal function, to develop and promote steroid-sparing strategies, to provide optimal nutritional support to maximize final height and avoid bone deformations, to equilibrate calcium/phosphate metabolism so as to provide acceptable bone quality and cardiovascular status, to correct all metabolic and clinical abnormalities that can worsen both bone and growth (mainly metabolic acidosis, anemia and malnutrition), promote good lifestyle habits (adequate calcium intake, regular physical activity, no sodas consumption, no tobacco exposure) and eventually to correct native vitamin D deficiency (target of 25-vitamin D >75 nmol/l).


Asunto(s)
Huesos/metabolismo , Trasplante de Riñón , Animales , Desarrollo Óseo , Humanos , Enfermedades Renales/etiología , Trasplante de Riñón/efectos adversos , Calidad de Vida
18.
Soins Pediatr Pueric ; (272): 23-4, 2013.
Artículo en Francés | MEDLINE | ID: mdl-23789174

RESUMEN

Vesicoureteral reflux is the most frequent uropathy in children and can have serious consequences if left untreated, including impaired kidney function.As urinary infection is the main element leading to its diagnosis, it is important not to ignore such an infection and to act quickly to treat it and prevent any recurrence.


Asunto(s)
Reflujo Vesicoureteral/diagnóstico , Reflujo Vesicoureteral/terapia , Niño , Endoscopía , Humanos , Procedimientos Quirúrgicos Urológicos
19.
Transpl Int ; 26(2): 154-61, 2013 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-23227963

RESUMEN

Data on long-term outcomes after pediatric renal transplantation (Tx) are still limited. We report on a 20-year single-center experience. Medical charts of all consecutive pediatric Tx performed between 1987 and 2007 were reviewed. Data of patients who had been transferred to adult units were extracted from the French databases of renal replacement therapies. Outcomes were assessed using Kaplan-Meier and Cox models. Two hundred forty Tx were performed in 219 children (24.1% pre-emptive and 17.5% living related donor Tx). Median age at Tx was 11.1 years and median follow-up was 10.4 years. Patient survival was 94%, 92%, and 91% at 5, 10, and 15 years post-Tx, respectively. Overall, transplant survival was 92%, 82%, 72%, and 59% at 1, 5, 10, and 15 years post-Tx, respectively. The expected death-censored graft half-life was 20 years. Sixteen patients developed malignancies during follow-up. Median height at 18 years of age was 166 cm in boys and 152 cm in girls with 68% of patients being in the normal range. The proportion of socially disadvantaged young people was higher than in general population. Excellent long-term outcomes can be achieved in pediatric renal Tx, but specific problems such as malignancies, growth, and social outcome remain challenging.


Asunto(s)
Trasplante de Riñón/estadística & datos numéricos , Insuficiencia Renal/terapia , Adolescente , Niño , Preescolar , Femenino , Supervivencia de Injerto , Humanos , Inmunosupresores/uso terapéutico , Trasplante de Riñón/efectos adversos , Trasplante de Riñón/mortalidad , Donadores Vivos , Masculino , Modelos Estadísticos , Modelos de Riesgos Proporcionales , Estudios Retrospectivos , Resultado del Tratamiento
20.
Nephrol Ther ; 7(7): 604-7, 2011 Dec.
Artículo en Francés | MEDLINE | ID: mdl-22118790

RESUMEN

Graft survival is worse in recipient aged less than 5 years due to the greater risk of vascular thrombosis. Thrombosis may be prevented by the choice of the donor, method of surgery, perioperative hemodynamic optimisation and preventive anti-coagulation. Normal growth is a major objective of the management of transplanted children. The mean final height increased during the 20 last years to be between -1.63 and -0.92 SDS depending on age and period of the transplantation.


Asunto(s)
Estatura , Supervivencia de Injerto , Trasplante de Riñón , Trombosis/prevención & control , Adolescente , Distribución por Edad , Algoritmos , Niño , Preescolar , Francia/epidemiología , Rechazo de Injerto/prevención & control , Humanos , Lactante , Recién Nacido , Fallo Renal Crónico/cirugía , Trasplante de Riñón/efectos adversos , Trasplante de Riñón/mortalidad , Factores de Riesgo , Trombosis/etiología
SELECCIÓN DE REFERENCIAS
DETALLE DE LA BÚSQUEDA
...