Concomitant congenital CMV infection and inherited liver diseases.

Inherited liver diseases may present in infancy as cholestatic jaundice progressing to severe hepatic dysfunction. Congenital cytomegalovirus (cCMV) infection may initially involve the liver, yet in otherwise healthy hosts rarely leads to long-term hepatic disease. We report a series of three patients, diagnosed with hereditary liver diseases: progressive familial intrahepatic cholestasis (PFIC) type IV, alpha 1 anti-trypsin deficiency (A1ATD) and Alagille syndrome (ALGS), who were also diagnosed with cCMV infection. All patients were treated with valgancilovir for symptomatic cCMV infection (6-12 months), followed by suppressive dosing in the 2 patients with PFIC and A1ATD. Following 15-24 months of follow-up - the patients with PFIC and A1ATD developed severe liver failure, and the third had ongoing cholestatic disease with stable synthetic function. We propose a significant contribution of cCMV infection to the course of the inherited primary disease, possibly leading to further compromise of the liver. We recommend screening patients with inherited liver disease for cCMV, and considering anti-viral treatment with valganciclovir to delay hepatic disease progression.

Varicella vaccine strain infection in a non-immunocompromised patient. A case report and review of literature.

Varicella live attenuated vaccine led to a significant reduction in morbidity and mortality from varicella zoster disease. Vaccine adverse effects are mostly mild. Immunosuppression is the main risk factor for severe varicella. Risk factors for disease following vaccination are less studied. We report a 12-month-old infant with no T-cell immunodeficiency who developed severe varicella infection by vaccine strain.

Highly Selective Eating in Autism Spectrum Disorder Leading to Scurvy: A Series of Three Patients.

BACKGROUND: Some children with autism spectrum disorder (ASD) have highly specific food selectivity and therefore are prone to nutritional deficiencies of different kinds. PATIENTS: We document three children with ASD who presented with refusal to walk and gingivitis who underwent comprehensive evaluations before establishing the diagnosis of vitamin C deficiency (scurvy). The symptoms resolved after treatment with vitamin C. CONCLUSIONS: Prevention of nutritional deficiencies in children with ASD is essential, and providing multivitamin supplementation whenever high food selectivity is noted may prevent significant morbidity.

Single dose oral midazolam for minor emergency department procedures in children: a retrospective cohort study.

BACKGROUND: In the pediatric emergency department, patients are commonly treated with a single dose of oral midazolam for minor procedures. We sought to evaluate the effect of this treatment on procedure completion rates. METHODS: We conducted a single-center retrospective cohort study of all patients who were treated with pre-procedure oral midazolam between January 2011 and June 2016. The primary outcome was the procedure completion rate. RESULTS: During the study period, 1,504 patients were treated with oral midazolam as per department protocol; 1,467 received midazolam and 37 declined midazolam. Oral midazolam was used in 14 different types of emergency department procedures. The procedure completion rates in the treatment and non-treatment groups were 1,402/1,467 (95.6%) and 24/37 (64.8%), respectively (difference 30.7%; 95% confidence interval [CI] 17.3%-46.8%); p<0.0001. Treatment group patients had procedure completion rates of 25/33 (75.8%), 165/188 (87.8%%), 1,154/1,187 (97.2%), and 58/59 (98.3%), in the less than 0.3 mg/kg group, 0.3 to less than 0.5 mg/kg group, 0.5 to less than 0.7 mg/kg group, and 0.7 to less than 0.9 mg/kg group, respectively. Multivariate regression did not demonstrate an association between sex, ethnicity, dosage of 0.5 mg/kg or greater, type of procedure, and failure to complete procedure. Severe adverse events were not recorded. A dose of less than 0.3 mg/kg was significantly associated with an increased risk of failure to complete a procedure (adjusted odds ratio 8.34, 95% CI 3.32-20.9; p<0.0001). CONCLUSION: The findings suggest that oral midazolam in a single dose of 0.5 mg/kg or greater is associated with successful completion of minor pediatric procedures.