A 10-year-old boy with Trisomy 21 presented to the emergency department with a history of rash on his legs, easy bruising and bleeding, and walking with a limp. One month prior to presentation, he had developed a non-pruritic, non-painful, petechial rash on his lower legs ( Figure 1). Eczema was suspected and he was treated with a topical steroid cream, but there was no improvement in the rash. Subsequently, he developed easy bruising and bleeding gums when brushing his teeth. The day prior to presentation, he began limping and then refused to walk. His parents also reported a several-month history of decreased appetite and poor weight gain. They deny any fever, night sweats, or recent illnesses.His medical history was also significant for autism, chronic constipation, and very restricted diet for the past 3 years. Over this time, his diet had been limited to peanut butter and jelly sandwiches, soy bacon, pasta, waffles, wheat porridge, cheese crackers, vanilla pudding, toaster pastries, chocolate sandwich cookies, and pretzels. He refused to eat all other foods. He was not taking any supplemental medications, vitamins, or alternative therapies. PHYSICAL EXAMINATIONOn presentation, he was noted to be a thin-appearing child who had fine, kinky hair. He was 24.5 kilograms (15 th percentile on growth curve for Trisomy 21). He had gingival swelling and bleeding around the base of several teeth, but otherwise good dentition without caries. There was blood in the left external auditory canal obscuring the left tympanic membrane; the right canal and right tympanic membrane were normal. Heart, lung, and abdominal examinations were unremarkable. His left knee was swollen without effusion or tenderness and had full range of motion; the right knee was normal. His skin was remarkable for perifollicular petechiae over bilateral lower extremities, face, and trunk, with scattered bruises over his legs. HOSPITAL COURSEHe was admitted to the hospital for further evaluation. Complete blood count, serum chemistries, liver function panel, and coagulation panel (prothrombin time, activated partial thromboplastin time, and international normalized ratio) were normal. Plain radiographs of the lower extremities were unremarkable. Additional laboratory testing confirmed the diagnosis.Editor's note: Each month, this department features a discussion of an unusual diagnosis. A description and images are presented, followed by the diagnosis and an explanation of how the diagnosis was determined. As always, your comments are welcome via email at pedann@Healio.com.
The success of a quality improvement (QI) project depends not only on the implementation of the initiative but also on the sustainability of the changes. 1 While there is large body of literature showing the successful implementation of QI initiatives in hospitals across the nation, reports showing sustainability in follow-up periods are often not available. 1 We implemented a QI initiative to increase the rate of nasogastric (NG) hydration in children with bronchiolitis from January to April 2016. 2 The rationale for the initiative was that NG hydration, compared with intravenous (IV) hydration, can provide better nutrition since breast milk or formula can be given via NG tube. Decreased nutritional intake early in the hospital course has been associated with a longer length of stay in infants with bronchiolitis. 3,4 Increased protein energy intake in critically ill infants with bronchiolitis promotes anabolism, an important goal of nutritional support of sick children. 5 Our initiative was successful in increasing rates of NG hydration in eligible children from 0% to 58% in 6 months. 2 We sought to determine if this change could be sustained without further interventions.
Severe respiratory syncytial virus (RSV) bronchiolitis in early life is a significant risk factor for future recurrent wheeze (RW) and asthma. The goal of the Azithromycin to Prevent Wheezing following severe RSV bronchiolitis II (APW-RSV II) clinical trial is to evaluate if azithromycin treatment in infants hospitalized with RSV bronchiolitis reduces the occurrence of RW during the preschool years. The APW-RSV II clinical trial is a double-blind, placebo-controlled, parallel-group, randomized trial, including otherwise healthy participants, ages 30 days-18 months, who are hospitalized due to RSV bronchiolitis. The study includes an active randomized treatment phase with azithromycin or placebo for 2 weeks, and an observational phase of 18–48 months. Two hundred participants were enrolled during three consecutive RSV seasons beginning in the fall of 2016 and were randomized to receive oral azithromycin 10 mg/kg/day for 7 days followed by 5 mg/kg/day for an additional 7 days, or matched placebo. The study hypothesis is that in infants hospitalized with RSV bronchiolitis, the addition of azithromycin therapy to routine bronchiolitis care would reduce the likelihood of developing post-RSV recurrent wheeze (≥3 episodes). The primary clinical outcome is the occurrence of a third episode of wheezing, which is evaluated every other month by phone questionnaires and during yearly in-person visits. A secondary objective of the APW-RSV II clinical trial is to examine how azithromycin therapy changes the upper airway microbiome composition, and to determine if these changes are related to the occurrence of post-RSV RW. Microbiome composition is characterized in nasal wash samples obtained before and after the study treatments. This clinical trial may identify the first effective intervention applied during severe RSV bronchiolitis to reduce the risk of post-RSV RW and ultimately asthma.
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