Vantris vs. deflux for treatment of paediatric vesicoureteral reflux: Efficacy and obstruction risk.

INTRODUCTION: The aim was to compare the efficacy of polyacrylate polyalcohol copolymer (PPC) injections and dextranomer/hyaluronic acid (Dx/Ha) injections for the endoscopic treatment of vesicoureteral reflux in children. MATERIAL: This retrospective cohort study included 189 young patients who had endoscopic treatment for vesicoureteral reflux from January 2012 to December 2019 in our center. Among them, 101 had PCC injections and 88 had Dx/Ha injections. Indications for treatment were vesicoureteral reflux with breakthrough urinary tract infection or vesicoureteral reflux with renal scarring on dimercaptosuccinic acid (DMSA) renal scan. Endoscopic injection was performed under the ureteral meatus. Early complications, recurrence of febrile urinary tract infection and vesicoureteral reflux after endoscopic injection, ureteral obstruction and reintervention were evaluated and compared between groups. RESULTS: Endoscopic treatment was successful in 90.1% of patients who had PPC injection and in 82% of patients who had Dx/Ha injection. Four patients presented a chronic ureteral obstruction after PPC injection, one with a complete loss of function of the dilated kidney. One patient in the Dx/Ha group presented a postoperative ureteral dilatation after 2 injections. CONCLUSION: Despite a similar success rate after PPC and Dx/Ha injections for endoscopic treatment of VUR, there may be a greater risk of postoperative ureteral obstruction after PPC injections. The benefit of using PPC to prevent febrile UTI and renal scarring in children with low-grade VUR does not seem to outweigh the risk of chronic ureteral obstruction.

Precocious initiation of spermatogenesis in a 19-month-old boy with Hurler syndrome.

Mucopolysaccharidosis type IH (MPS IH) is a rare autosomal recessive lysosomal storage disorder. Haematopoietic stem cell transplantation (HSCT) has been proposed for the treatment of MPS IH patients and offers the possibility to grow into their adulthood. Precocious puberty has been described in few MPS patients. We report, to the best of our knowledge and for the first time, the initiation of the first waves of spermatogenesis fortuitously observed in seminiferous tubules of a pre-pubertal 19-month-old boy, affected by MPS IH and who did not present any clinical signs of precocious puberty. This patient benefited from testicular tissue cryopreservation before HSCT. Seminiferous tubule size, germ cell differentiation and Sertoli cell expression of androgen receptor and anti-müllerian hormone corresponded to the pattern observed in a pubertal boy. The Hurler syndrome may be responsible for the precocious initiation of spermatogenesis. A specific follow-up during childhood may be useful to confirm if such abnormal testis development is common in young boys with MPS IH and if it may lead to precocious onset of puberty in survivors despite HSCT. Furthermore, we have observed that Sertoli cell maturation (up-regulation of AR expression, down-regulation of AMH expression) occurred before the clinical signs of puberty and before the increase of testosterone plasmatic level.

La Mucopolysaccharidose de type IH (MPS IH) est une maladie rare lysosomale de transmission récessive autosomique. L'allogreffe de cellules souches hématopoïétiques (CSH) a été proposée pour le traitement des patients atteints de MPS IH et offre la possibilité d'une survie de ces patients jusqu'à l'âge adulte. La puberté précoce a été décrite chez quelques patients MPS. Nous rapportons, à notre connaissance et pour la première fois, l'initiation de la première vague de spermatogenèse observée fortuitement dans les tubules séminifères d'un garçon de 19 mois, atteint de MPS IH et sans signes cliniques de puberté précoce. Ce patient a bénéficié d'une cryoconservation de tissu testiculaire avant la greffe de CHS. Le diamètre des tubes séminifères, la différenciation des cellules germinales et l'expression du récepteur aux androgènes et de l'hormone antimüllérienne dans les cellules de Sertoli présentent les caractéristiques retrouvées dans le testicule d'un garçon pubère. Le syndrome de Hurler peut être responsable de l'initiation précoce de la spermatogenèse. Un suivi spécifique pendant l'enfance peut être utile pour confirmer si cette maturation prématurée du testicule est fréquente chez les jeunes garçons atteints de MPS IH et si elle peut conduire à une puberté précoce chez les patients malgré la greffe de CSH. En outre, notre observation démontre que la maturation des cellules de Sertoli se produit avant les signes cliniques de puberté et avant l'augmentation de la concentration plasmatique de testostérone.

[Cryopreservation of testicular tissue in children]. / Cryopréservation du tissu testiculaire chez l'enfant.

The toxicity of cancer therapies can affect all organs and tissues. Some treatments damage spermatogonial stem cells (SSCs), with a risk of infertility. Storage and reimplantation of frozen testicular tissue is a recent approach tofertilitypreservationfor young boys. However, thawed frozen prepubertal testicular tissue must undergo a maturation process to restore sperm production. This process, currently being studied in animal models, can be achieved by in vivo transplantation of SSCs into seminiferous tubules or by testicular grafting, possibly following in vitro maturation.

Quality of life in adults with bladder exstrophy-epispadias complex.

PURPOSE: We evaluated quality of life in adults with bladder exstrophy-epispadias complex to improve patient and parent counseling. MATERIALS AND METHODS: We evaluated quality of life in a multicenter study using the SF-36® Medical Outcome Study questionnaire and a local questionnaire. A total of 47 patients born in 1957 to 1990 were followed due to bladder exstrophy-epispadias complex at the 4 participating hospitals. RESULTS: Nine women and 16 men were included in analysis, including 15 who underwent staged reconstruction for bladder exstrophy-epispadias complex and 10 who underwent primary or secondary urinary diversion. Nine patients were married and 2 were in a long-term partnership. A total of 22 patients achieved a high school degree with examination success and 17 worked full time. Six women and 12 men achieved penetrative intercourse. A total of 13 children were born to 3 women and 7 men. Quality of life scores in our patients were less than the norm based scores on 2 of the 8 health concepts, including limitations in physical activity due to health problems and general health perception. Results were statistically different among patients depending on dryness, voiding and urinary reconstruction/diversion. Patient scores did not differ in regard to gender, number of interventions, sexual life, cosmesis or renal function. CONCLUSIONS: Patient scores were less than norm based scores on only 2 health concepts and patients were generally well integrated into society. Urinary diversion was usually well tolerated. It remains a therapeutic option when incontinence interferes with social life or renal function is compromised.

Ovarian tissue thawing: a comparison of two conditions.

Two different protocols to thaw cryopreserved human ovarian cortex have been evaluated using a histological analysis. The slower one, based on a progressive dilution of cryoprotectants, seems to maintain an optimal follicle morphology.