Quantitative analysis of the natural history of prolidase deficiency: description of 17 families and systematic review of published cases.

Purpose: Prolidase deficiency is a rare inborn error of metabolism causing ulcers and other skin disorders, splenomegaly, developmental delay, and recurrent infections. Most of the literature is constituted of isolated case reports. We aim to provide a quantitative description of the natural history of the condition by describing 19 affected individuals and reviewing the literature.

[Values of cerebrospinal fluid cytochemistry in pediatric patients with ventriculoperitoneal shunt].

Background: The ventriculo-peritoneal shunt (VPS) is the treatment for hydrocephalus, the cerebrospinal fluid (CSF) is evaluated for the management of its complications; however, information on the values of the cytochemistry in this population is insufficient.

Aim: To describe the characteristics of the CSF cytochemistry of children in VPS management.

Methods: Descriptive observational study, developed in Bogotá (Colombia), from 2008 to 2016.