Evaluation of T-Cell receptor diversity in pediatric patients with minimal change nephrotic syndrome.

Aims: To further elucidate the clinical relevance of T-cell abnormalities in minimal change nephrotic syndrome (MCNS), and to predict the consequences of MCNS, we studied T-cell receptor (TCR) diversity by analyzing CDR3 size distribution and the frequency of Vβ repertoire usage.

Methods: Participants comprised 36 pediatric patients with MCNS. 18 were frequent relapsers (FRs) and/or steroid-dependent (SD) and 18 were non-frequent relapsers (NFRs).

Isolated congenital megacystis without intestinal obstruction: a mild variant of chronic intestinal pseudoobstruction syndrome?

Megacystis is frequently involved with chronic intestinal pseudoobstruction syndrome; however, isolated megacystis without intestinal obstruction is extremely rare. We present the case of a female patient with isolated congenital megacystis without severe intestinal obstruction. In this case, barium enema did not reveal any significant findings; however, histologic evaluation of her rectum showed hypoganglionosis of the submucous and myenteric plexuses.