Coronary artery disease is a global problem with high mortality rates and significant residual sequelae that affect long-term quality of life. Myocardial infarction (MI) in neonates is a recognized, uncommon entity, but the incidence and broad spectrum of the disease is unknown and likely underestimated due to limited reporting which in the majority is confined to acute ischemic events. The challenges involve clinical diagnosis which masquerades in the early phase as non-specific symptoms and signs that are commonly found in a host of neonatal disorders. Precise diagnostic criteria for neonatal MI are lacking, and management is driven by clinical presentation and hemodynamic stabilization rather than an attempt to rapidly establish the root cause of the condition. We conducted a review of the published reports of neonatal MI from 2000 to 2014, to establish an approach to the diagnosis and management based on the existing evidence. The overall evidence from 32 scientific articles stemmed from case reports and case series which were graded as low-to-very low quality. Neonatal MI resembles childhood and adult MI with features that involve characteristic ECG changes, raised biomarkers, and diagnostic imaging, but with lack of robust, standardized criteria to facilitate prompt diagnosis and timely intervention. The mortality rate of neonatal MI ranges from 40 to 50% based on inclusion criteria, but the short-term data reflect normal quality of life in survivors. An algorithm for the diagnosis and management of neonatal MI may optimize outcomes, but at the present time is based on limited evidence. Well-designed clinical studies focusing on the definition, diagnosis, and management of neonatal MI, backed by international consensus guidelines, are needed to alter the prognosis of this serious condition.
Deferasirox is an oral iron chelator used to treat patients with transfusion-related iron overload. We report, from two institutions, two children with Diamond-Blackfan anemia who developed Fanconi syndrome secondary to deferasirox administration, along with a review of the literature. The current recommendation for the laboratory monitoring of patients receiving deferasirox does not include serum electrolytes or urine analysis. Thus, despite routine clinic visits and bloodwork, these two patients presented with life-threatening electrolyte abnormalities requiring hospitalization. Hence, we propose the inclusion of serum electrolytes and urine analysis as part of routine monitoring to facilitate the early diagnosis of Fanconi syndrome in the context of high doses of deferasirox therapy.
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