The Joint Outcome Study (JOS), a randomized controlled trial, demonstrated that children with severe hemophilia A (HA) initiating prophylactic factor VIII (FVIII) prior to age 2.5 years had reduced joint damage at age 6 years compared with those treated with episodic FVIII for bleeding. The Joint Outcome Continuation Study (JOS-C) evaluated early vs delayed prophylaxis effects on long-term joint health, following JOS participants to age 18 years in an observational, partially retrospective study. Index joint magnetic resonance imaging (MRI) scores of osteochondral (OC) damage (primary outcome), joint physical examination scores, and annualized rates of joint/other bleeding episodes (secondary outcomes) were collected. Thirty-seven of 65 JOS participants enrolled in JOS-C, including 15 randomized to prophylaxis at mean age 1.3 years (“early prophylaxis”); 18 initially randomized to episodic treatment, starting “delayed prophylaxis” at mean age 7.5 years; and 4 with high-titer inhibitors. At JOS-C exit, MRI OC damage was found in 77% of those on delayed and 35% of those on early prophylaxis for an odds ratio of OC damage, in the delayed vs early prophylaxis group, of 6.3 (95% confidence interval, 1.3, 29.9; P = .02). Annualized bleeding rates were higher with delayed prophylaxis (mean plus or minus standard deviation, 10.6 ± 6.6 vs 3.5 ± 2.1; P < .001), including when only comparing time periods on prophylaxis (6.2 ± 5.3 vs 3.3 ± 1.9; P < .05). In severe HA, early initiation of prophylaxis provided continued protection against joint damage throughout childhood compared with delayed initiation, but early prophylaxis was not sufficient to fully prevent damage. This trial was registered at www.clinicaltrials.gov as #NCT01000844.
Intraoperative pathological findings at the time of open surgical treatment were universally recognized on MRI but not on CT scans. MRI should be considered for evaluation of the hip following closed reduction for the treatment of a posterior dislocation in children and adolescents as it reliably allows assessment of intra-articular pathology without the risk of radiation exposure.
• Large study describing the time frames of fracture healing in young infants. • Features of fracture healing develop in a logical progression. • Evidence provided for determining fractures are consistent with a proposed time frame. • It is of critical importance to have sound evidence for the dating of fractures.
The most common vascular tumors of infancy are hemangiomas. These are further classified as infantile or congenital. Infantile hemangiomas are not present at birth, go on to proliferate and then involute, whereas congenital hemangiomas are mature at birth. Congenital hemangiomas are further characterized as rapidly involuting (RICH) or noninvoluting (NICH). Rapidly involuting congenital hemangiomas (RICH) are more common with the majority involuting completely by 12 months of age. Noninvoluting congenital hemangiomas (NICH) never involute, demonstrate proportional growth and require eventual excision. We report a unique case of an intracranial rapidly involuting congenital hemangioma. Pre- and postnatal imaging, as well as clinical follow-up, demonstrate the rapid regression of both the intracranial and cutaneous portions of this lesion during the first year of life.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.