InsomniaI nsomnia (trouble falling asleep or staying asleep) is a common problem. It can cause daytime tiredness and sleepiness and difficulty paying attention at school or being alert at work. Insomnia affects people of all ages. Insomnia can be transient (short-term) and related to a specific condition like stress or travel, or it can be a chronic (long-term) problem that persists for many months. The June 28, 2006, issue of JAMA includes an article about treatment of chronic insomnia in older adults. This Patient Page is based on one previously published in the May 21, 2003, issue of JAMA.
Background
Donor organ acceptance practices vary among pediatric heart transplant professionals. We sought to understand what is known about the interactions between the “high‐risk” recipient and the “marginal donor,” and how donor risk scores can impact this discussion.
Methods
A systematic review of published literature on pediatric HTx was undertaken with the assistance of a medical librarian. Two authors independently assessed search results, and papers were reviewed for inclusion.
Results
We found that there are a large number of individual factors, and clusters of factors, that have been used to label individual recipients “high‐risk” and individual donors “marginal.” The terms “high‐risk recipient” and “marginal donor” have been used broadly in the literature making it virtually impossible to make comparisons between publications. In general, the data support that patients who could be easily agreed to be “sicker recipients” are at more risk compared to those who are clearly “healthier,” albeit still “sick enough” to need transplantation. Given this variability in the literature, we were unable to define how being a “high‐risk” recipient interplays with accepting a “marginal donor.” Existing risk scores are described, but none were felt to adequately predict outcomes from factors available at the time of offer acceptance.
Conclusions
We could not determine what makes a donor “marginal,” a recipient “high‐risk,” or how these factors interplay within the specific recipient‐donor pair to determine outcomes. Until there are better risk scores predicting outcomes at the time of organ acceptance, programs should continue to evaluate each organ and recipient individually.
There is limited information regarding ideal dosage of alglucoside alfa in patients with Infantile Onset Pompe Disease (IOPD). The U.S. Food and Drug Administration approved alglucoside alfa at dosing of 20 mg/kg every other week, but there are small studies and case reports suggesting that dosing higher than this leads to improved ventilator free survival and development without adverse events. We review the clinical course and short term clinical outcomes one year following late diagnosis of IOPD in a 3 month old who presented severely affected and was treated with 40 mg/kg twice a week for 21 infusions until six months of age then transitioned to 40 mg/kg/week. The patient responded well to 40 mg/kg twice a week treatment without adverse reactions and significant clinical improvement.
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