Objective Thrombocytopenia-associated multiple organ failure can lead to high mortality in critically ill children, possibly related to consequences of thrombotic microangiopathy. Plasma exchange therapy may improve thrombotic microangiopathy. The purpose of this observational cohort study is to describe whether there is an association between use of plasma exchange therapy and outcome in the Turkish thrombocytopenia-associated multiple organ failure network. Setting-Interventions We performed a retrospective cohort analysis in patients with thrombocytopenia-associated multiple organ failure at three different PICUs comparing those who received plasma exchange (+) plus standard therapies with those who did not receive plasma exchange (−) and only received standard therapies. Results Among 42 of the enrolled patients with thrombocytopenia- associated multiple organ failure, all had a primary or secondary sepsis diagnosis. Fifteen received plasma exchange therapy (PE [+] group) and 27 received standard medical treatment without plasma exchange (PE [−] group). The mean age was 17.69 months (8.24–54.22) in the PE (+) group and 13.46 months (6.47–20.55) in the PE (−) group. Age (p = 0.232), gender (p = 0.206), thrombocyte count (p = 0.09), Organ Failure Index score (p = 0.111), and pediatric logistic organ dysfunction score (p = 0.177) at admission were not statistically different between groups. The overall 28-day mortality was higher in the PE (−) group (70.37%) compared with the PE (+) group (26.67%) (univariate p = 0.006; multivariate controlling for pediatric logistic organ dysfunction, Organ Failure Index, Pediatric Risk of Mortality scores, and neurological failure p = 0.048). Length of stay was increased in the PE (+) group (p = 0.004). Conclusions The positive association found between use of plasma exchange therapy and improved survival supports the potential of this therapy in Turkish children with thrombocytopeniaassociated multiple organ failure. The positive, although less so, associated treatment effect observed after controlling for illness severity provides further rationale for performing a randomized controlled trial in the pediatric Turkish thrombocytopenia-associated multiple organ failure network. Sample size calculations call for a 100-patient trial with a pre hoc interim analysis after enrollment of 50 patients with thrombocytopenia-associated multiple organ failure.

No abstract

Goldenhar's syndrome is characterized by a combination of dermal epibulbar cysts, auricular appendices, malformation of ears, and vertebral anomalies. The average incidence ratio of Goldenhar's syndrome is estimated to be 1/5600, and the frequency of cardiovascular malformations of this syndrome is 5-58 per cent. Although the etiology of this disease is not fully understood, autosomal recessive or dominant inheritance is possible. The disease occurs as sporadic cases. Male:female ratio for the Goldenhar's syndrome is 2:1. A 72-day-old Goldenhar's syndrome case is reported who presented with multiple congenital anomalies.

Autonomic dysfunction existed in the children with BHS, but no correlation was found between BHS and deficiencies of iron, vitamin D, or vitamin B12.

No abstract