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Accidental overdose of zoledronic acid in a pediatric patient with osteogenesis imperfecta
No full textPrelude to the perfect storm: the many triggers of secondary hemophagocytic lymphohistiocytosis.
No full textHemophagocytic lymphohistiocytosis (HLH) is an increasingly recognized disorder of immune hyperactivity that often leads to multiorgan failure and death. In adults, HLH is usually triggered by infection, malignancy, or an autoimmune/autoinflammatory disorder that precipitates a destructive cytokine storm. Treatment aims to deescalate the hyperimmunity by treating the triggers while interfering with the immune pathways that cause the morbidity. We summarize what is known about this devastating disorder while adding insights gleaned from our review of the literature coupled with our own experiences. For example, we recognized that 1) more than one trigger may coexist in the same patient, 2) there is a predilection for HLH in lymphoma patients, particularly in the presence of Epstein-Barr virus infection, 3) aspartate transaminase elevation exceeds that of alanine transaminase, and 4) encephalopathy is underrecognized. These and other observations may assist the readers in identifying HLH, distinguishing its clinical presentation from that of its triggers, and thoroughly assessing all possible triggers rather than anchoring on the first diagnosed
Strategies to Promote and Cultivate Student Interest in the Midst of Institutional Change
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Dacryocystorhinostomy outcomes for congenital nasolacrimal duct obstruction associated with craniofacial abnormalities.
No full textPURPOSE: To compare the clinical characteristics and outcomes of pediatric patients with and without craniofacial abnormalities who underwent dacryocystorhinostomy (DCR) for recalcitrant congenital nasolacrimal duct obstruction (CNLDO).
METHODS: This multicenter retrospective cohort study included all patients \u3c 17 years of age who underwent DCR for CNLDO over a 20-year period, from 2003 to 2023. Outcome measures included residual epiphora following surgery and need for additional surgery including DCR revision.
RESULTS: A total of 40 patients (55 eyes) underwent DCR, of whom 28 (51%) had associated craniofacial abnormalities. Mean age at surgery was 6.3 ± 4.0 years, and 30 (55%) were female. There were no significant differences in age at surgery, sex, or surgical approach between isolated CNLDO and CNLDO associated with craniofacial abnormalities, but the craniofacial cohort was significantly more likely to require multispecialty surgical care (43% vs 7% [P \u3c 0.05]) and have proximal lacrimal system pathology (OR = 7.92, P = 0.001). In the craniofacial cohort, 46% of eyes had residual symptoms following surgery compared with 26% in the isolated group (OR = 3.3, P = 0.06), and patients with craniofacial abnormalities who underwent multispecialty surgery were significantly more likely to have complete symptom resolution (OR = 8.33, P = 0.017). In the craniofacial group, there were 6 revisions; in the isolated group, 2.
CONCLUSIONS: In our patient cohort, CNLDO associated with craniofacial abnormalities had a higher likelihood of persistent epiphora following DCR than isolated CNLDO, but multidisciplinary surgical care was associated with improved outcomes. Surgeons should be prepared to address proximal lacrimal pathology at the time of DCR in children with craniofacial abnormalities
