Urinary albumin excretion and chronic kidney disease in children with vesicoureteral reflux.

BACKGROUND: Albuminuria is a potential biomarker of chronic kidney disease (CKD) in various glomerular diseases. Vesicoureteral reflux (VUR) often progresses to CKD, and study is required of use of albuminuria as a biomarker for this condition. The aim of this study was to evaluate the association between albuminuria and glomerular filtration rate (GFR) or filtration fraction (FF) in children with VUR. STUDY DESIGN: In this retrospective study, renal parameters of 141 children with VUR were investigated, using inulin clearance, FF, and albuminuria. The association between urinary albumin to creatinine ratio (ACR), GFR, and FF was analyzed in a continuous manner by calculating the ß coefficient, and also in a binary manner by calculating the OR. RESULTS: Using both continuous and binary analyses, ACR values were negatively and significantly associated to GFR values in patients with low, normal, or high FF values (Table). It was also positively and significantly associated with FF values in patients with low, normal or high GFR values (Table). No association was found between ACR and gender, VUR stages or laterality, number of urinary tract infection, presence of a single functional kidney, history of reflux surgery, or renal scars or hypertension. DISCUSSION: ACR is associated with CKD in patients with VUR. In addition, increased urinary albumin excretion cannot be completely and solely explained by decreased GFR and/or increased FF values. The two main limitations of this study are the crude assessment of renal scarring, which prevented finer analysis between albuminuria and renal scarring surface area, and that the study cohort may not be representative of the true VUR population. CONCLUSION: This study shows that albuminuria is associated with decreased renal function in patients with VUR and that it could be used to monitor renal function in this condition.

[Patients with variations of sex development : an example of interdisciplinary care]. / Patients avec variation du développement sexuel : un exemple de prise en charge interdisciplinaire.

The medical, psychological and social aspects of disorders of sex development (DSD) represent a challenge for the management of these patients. However, advances in our understanding of the etiology and genetics of this condition, novel surgical approaches and the growing influence of patient groups as well as wider recognition of ethical issues have helped improve the care of patients with a DSD. Importantly, a multidisciplinary approach involving specialists is crucial for understanding and treating such rare and complex cases. According to the recommendations of the Swiss National Ethical Commission, we shall use the term « Variation of Sex Development ¼ rather than « Disorder of Sex Development ¼ in this publication. This article addresses the care of DSD patients throughout development from the point of view of specialists in complementary fields.

La prise en charge des personnes avec une variation du développement sexuel (VDS) (disorder of sex development, DSD) est un défi tant sur le plan médical, psychologique que social. L'amélioration des connaissances étiologiques et génétiques, les nouvelles approches chirurgicales et l'influence tant des groupes de patients que de la Commission d'éthique suisse ont considérablement modifié la vision de la prise en charge de ces personnes durant ces dernières décennies. Une approche pluridisciplinaire et spécialisée est cruciale pour appréhender ces situations rares et souvent complexes. Le point de vue des différents spécialistes impliqués au long de la vie dans la prise en charge d'une VDS est abordé dans cet article.

[Inguinal hernias in girls: is caryotype necessary?]. / Pédiatrie. 1. Hernie inguinale chez la fille: un caryotype est-il nécessaire?

The incidence of inguinal hernias in children is 1-4% with predominance in boys (10 to 1). Based on the data, 0.8-2.4% of premenstrual girls presenting with inguinal hernias have a complete androgen insensitivity syndrome (CAIS). On the other hand, 80-90% of girls with CAIS present uni- or bilateral inguinal hernias. Unfortunately, this burdened pathology is very rarely looked for by pediatricians, or paediatric surgeons.

Anorectal Malformations: Finding the Pathway out of the Labyrinth.

Anorectal malformations (ARMs) are a complex group of congenital anomalies involving the distal anus and rectum, as well as the urinary and genital tracts in a significant number of cases. Most ARMs result from abnormal development of the urorectal septum in early fetal life. In most cases, the anus is not perforated and the distal enteric component ends blindly (atresia) or as a fistula into the urinary tract, genital tract, or perineum. ARMs are also present in a great number of syndromes and associations of congenital anomalies. The classification of ARMs is mainly based on the position of the rectal pouch relative to the puborectal sling, the presence or absence of fistulas, and the types and locations of the fistulas. All of this information is crucial in determining the most appropriate surgical approach for each case. Imaging studies play a key role in evaluation and classification of ARMs. In neonates, clinical and radiologic examinations in the first 3 days of life help determine the type of ARM and the need for early colostomy. In older children, preoperative pelvic magnetic resonance imaging is the most efficient diagnostic method for evaluating the size, morphology, and grade of development of the sphincteric musculature.

Urodynamic score in children with lipomyelomeningocele: a prospective study.

PURPOSE: We assessed the correlation between urodynamic score and presence of symptoms in children with lipomyelomeningocele, and the usefulness of this measure in monitoring these children. MATERIALS AND METHODS: We prospectively evaluated 29 patients 1 month to 13 years old (mean 1.9 years) with lipomyelomeningocele from 1995 to 2010. A 4-parameter urodynamic score ranging from 0 to 17 was assigned to each patient at diagnosis and followup. Indications for surgery were presence of symptoms and signs or abnormal urodynamic score. Children were divided into 2 groups, symptomatic and asymptomatic. The latter group was further divided into operated and conservatively managed cases. Average followup was 6.7 years (range 2 to 16). RESULTS: The symptomatic group contained 11 children (38%) operated on at a mean age of 2.9 years. Mean followup was 6.7 years (range 2 to 12). All patients presented with an abnormal urodynamic score. Postoperative urodynamic score improved in 6 patients (55%), remained abnormal in 4 (36%) and deteriorated in 1 (9%). The asymptomatic group contained 18 patients, of whom 7 (39%) were operated on at a mean age of 1.4 years. Mean followup was 10 years (range 3 to 16). Postoperative score improved in 6 patients (86%) and remained unchanged in 1 (14%). A total of 11 cases (61%) were managed conservatively, with a mean followup 4.3 years (range 2 to 7). Of these patients 3 (27%) presented with late urodynamic score deterioration at 3, 5 and 6 years of followup while remaining asymptomatic. CONCLUSIONS: Urodynamic score is a useful tool in monitoring children with lipomyelomeningocele because it has a good correlation with the presence of symptoms.

A lower fistula rate in hypospadias surgery.

OBJECTIVE: To determine whether specific steps taken after a critical quality control of our results in hypospadias surgery lead to a decrease in fistula rate. PATIENTS AND METHODS: Retrospective review of prospectively collected data. Between 1994 and 2001, our series of 85 tubularized plate urethroplasties (modified Duplay or Duplay-Snodgrass procedure) had a fistula rate of 25.9%. In 2001, we modified our approach by systematically padding the urethral suture with a layer of vascularized subcutaneous preputial tissue, as described by Snodgrass. Scrotal hypospadias were excluded. Surgical outcome was assessed at 1 and 12 months. In both groups, all repairs were performed by or under direct supervision of the senior author (BJM). RESULTS: After 2001, 57 hypospadias repairs were performed in 57 patients aged 8 months to 14 years (median 1.4 years). Fistula occurred in two cases, one of which closed spontaneously within 6 months. Our fistula rate had dropped to 3.5%, with a minimum follow up of 12 months. CONCLUSION: Covering the urethral suture with a padding flap of vascularized preputial tissue helps avoid fistula formation. Technique modification after critical appraisal of our own series led to a much better outcome in this demanding surgery.

Nephrogenic adenoma of the urethra: an unusual cause of hematuria in the child.

The authors describe a 9-year-old boy who had an accident with his bicycle. He presented with hematuria a few weeks later, and cystoscopy results showed a polypod lesion near the veru montanum. The lesion was resected, and histologic examination showed a nephrogenic adenoma (NA), which recurred 6 years later with hematuria. NA is a rare lesion in a child's urethra and can be a source of hematuria.