Ocular phenotype and therapeutic interventions in keratitis-ichthyosis-deafness (KID) syndrome.

Background: To report ocular manifestations, clinical course, and therapeutic management of patients with molecular genetically confirmed keratitis-ichthyosis-deafness syndrome.

Methods: Four patients, aged 19 to 46, with keratitis-ichthyosis-deafness syndrome from across the UK were recruited for a general and ocular examination and GJB2 (Cx26) mutational analysis. The ocular examination included best-corrected visual acuity, slit-lamp bio-microscopy, and ocular surface assessment.

Perioperative Risk Assessment in Children With Congenital Heart Disease Undergoing Noncardiac Procedures.

Objectives: To risk-stratify children with congenital heart disease undergoing noncardiac surgery or diagnostic procedures for perioperative cardiopulmonary complications using the authors' established institutional guidelines.

Design: A retrospective cohort study.

Setting: The study was conducted in an academic, tertiary-care children's hospital.

Autonomic Nervous System Monitoring: Cardiac Magnetic Resonance Imaging Data as a Surrogate for Autonomic Data in Children.

Perioperative autonomic nervous system (ANS) measurements are evolving toward increasing import and utility. We present a three-year-old male with Down Syndrome who underwent ambulatory autonomic monitoring during surgery followed by cardiac magnetic resonance (CMR) imaging. Autonomic data from both environments are compared to age-related norms.

Down syndrome and the autonomic nervous system, an educational review for the anesthesiologist.

Approximately one in every 700 babies in the United States is born with Down syndrome, or 0.14%. Children with Down syndrome have cognitive impairment and congenital malformations necessitating frequent occurrences of general anesthesia and surgery.