Common urological problems in children: inguinoscrotal pathologies.

Urological problems in children are often encountered in general clinical practice. This review forms the second paper of our series on common urological problems in children about inguinoscrotal pathologies. We aimed to provide concise information for doctors who are unfamiliar with this topic.

Common urological problems in children: prepuce, phimosis, and buried penis.

Parents often bring their children to the family doctor because of urological problems. Many general practitioners have received little training in this specialty. In this review, we aimed to provide a concise and informative review of common urological problems in children. This review will focus on the prepuce.

Correction: "Childhood intussusception: 17-year experience at a tertiary referral centre in Hong Kong.

[This corrects the article DOI: 10.12809/hkmj144456].

Hirschsprung disease, associated syndromes and genetics: a review.

Hirschsprung disease (HSCR, aganglionic megacolon) represents the main genetic cause of functional intestinal obstruction with an incidence of 1/5000 live births. This developmental disorder is a neurocristopathy and is characterised by the absence of the enteric ganglia along a variable length of the intestine. In the last decades, the development of surgical approaches has importantly decreased mortality and morbidity which allowed the emergence of familial cases. Isolated HSCR appears to be a non-Mendelian malformation with low, sex-dependent penetrance, and variable expression according to the length of the aganglionic segment. While all Mendelian modes of inheritance have been described in syndromic HSCR, isolated HSCR stands as a model for genetic disorders with complex patterns of inheritance. The tyrosine kinase receptor RET is the major gene with both rare coding sequence mutations and/or a frequent variant located in an enhancer element predisposing to the disease. Hitherto, 10 genes and five loci have been found to be involved in HSCR development.

Prokineticin signaling is required for the maintenance of a de novo population of c-KIT⁺ cells to sustain neuroblastoma progression.

High cellular heterogeneity within neuroblastomas (NBs) may account for the non-uniform response to treatment. c-KIT(+) cells are frequently detected in NB, but how they influence NB behavior still remains elusive. Here, we used NB tumor-initiating cells to reconstitute NB development and demonstrated that c-KIT(+) cells are de novo generated and dynamically maintained within the tumors to sustain tumor progression. c-KIT(+) NB cells express higher levels of neural crest and stem cell markers (SLUG, SOX2 and NANOG) and are endowed with high clonogenic capacity, differentiation plasticity and are refractory to drugs. With serial transplantation assays, we found that c-KIT expression is not required for tumor formation, but c-KIT(+) cells are more aggressive and can induce tumors ninefold more efficiently than c-KIT(-/low) cells. Intriguingly, c-KIT(+) cells exhibited a long-term in vivo self-renewal capacity to sustain the formation of secondary and tertiary tumors in mice. In addition, we showed that Prokineticin signaling and mitogen-activated protein kinase pathways are crucial for the maintenance of c-KIT(+) cells in tumor to promote NB progression. Our results highlight the importance of this de novo population of NB cells in sustainable growth of NB and reveal specific signaling pathways that may provide targets leading to more effective NB therapies.

Continuous-wave fiber optical parametric oscillator.

We report continuous-wave operation of singly resonant fiber optical parametric oscillators. In a cavity formed by 100 m of highly nonlinear fiber and two fiber Bragg gratings, the pump power threshold was 240 mW; the output wavelength could be tuned over 80 nm by tuning of the pump. We also obtained an internal conversion efficiency of 30%, compared with the maximum theoretical value of 50%, by use of a 1-km-long cavity.