Safety of live attenuated influenza vaccine (LAIV) in children and adults with asthma: a systematic literature review and narrative synthesis.

INTRODUCTION: Asthma is one of the most common chronic respiratory conditions worldwide and can be exacerbated by influenza. Findings from early trials demonstrated a higher risk of medically significant wheezing in otherwise healthy young children (aged 6 - 23 months) following administration of the Ann Arbor-backbone live attenuated influenza vaccine (LAIV-AA). In more recent years, several additional studies have investigated the safety of LAIV-AA in older children (2 - 17 years of age) and adults with asthma or prior wheezing, but these findings have not yet been systematically evaluated. AREAS COVERED: We conducted a systematic literature review to assess and synthesize the evidence from all available studies on the safety of LAIV-AA in people aged 2 - 49 years with a diagnosis of asthma or recurrent wheezing. EXPERT OPINION: Fourteen studies over 20 years, involving a total of 1.2 million participants, provided evidence that LAIV-AA was well tolerated with no safety concerns in individuals aged 2 - 49 years with a diagnosis of asthma or recurrent wheezing. These data can help inform guidelines for use of LAIV-AA in children and adults with a history of asthma or recurrent wheezing.

Pulmonary epithelioid hemangioendothelioma in a patient with Crohn's disease.

Pulmonary epithelioid hemangioendothelioma (PEH) is a rare neoplasm, largely unresponsive to chemotherapeutic medications, and with varied prognosis. Imaging on computerized tomography may demonstrate perivascular nodules, but diagnosis is ultimately made on biopsy with immunohistochemical analysis. Here we describe a case of PEH in a 14-year-old male with Crohn's disease, which, to our knowledge, has not previously been described in the literature.

Novel CFTR variants identified during the first 3 years of cystic fibrosis newborn screening in California.

California uses a unique method to screen newborns for cystic fibrosis (CF) that includes gene scanning and DNA sequencing after only one California-40 cystic fibrosis transmembrane conductance regulator (CFTR) panel mutation has been identified in hypertrypsinogenemic specimens. Newborns found by sequencing to have one or more additional mutations or variants (including novel variants) in the CFTR gene are systematically followed, allowing for prospective assessment of the pathogenic potential of these variants. During the first 3 years of screening, 55 novel variants were identified. Six of these novel variants were discovered in five screen-negative participants and three were identified in multiple unrelated participants. Ten novel variants (c.2554_2555insT, p.F1107L, c.-152G>C, p.L323P, p.L32M, c.2883_2886dupGTCA, c.2349_2350insT, p.K114del, c.-602A>T, and c.2822delT) were associated with a CF phenotype (42% of participants were diagnosed at 4 to 25 months of age), whereas 26 were associated with CFTR-related metabolic syndrome to date. Associations with the remaining novel variants were confounded by the presence of other diseases or other mutations in cis or by inadequate follow-up. These findings have implications for how CF newborn screening and follow-up is conducted and will help guide which genotypes should, and which should not, be considered screen positive for CF in California and elsewhere.

Understanding the relationship among pharmacoadherence measures, asthma control test scores, and office-based spirometry.

BACKGROUND: This study used accepted asthma metrics and pharmaceutical claim data to develop a new asthma metric as a possible surrogate for spirometry and to better assess asthma control. OBJECTIVE: To develop and validate a new asthma metric called the Rescue Index (RI) based on ß(2)-agonist dispensings and to test the feasibility of a multivariate model to construct a global asthma metric called the Asthma Control Index (ACI). METHODS: This prospective, observational, multicenter cohort study was conducted at the Naval Medical Center San Diego (NMCSD) and at the Phoenix VA Health Care System (PVAHCS). Pharmacy claim data were correlated with the ACT, Medication Adherence Report Scale for Asthma (MARS-A), and spirometry using univariate and multivariate analyses. RESULTS: A total of 263 individuals enrolled in the study, and 95 (PVAHCS) and 156 (NMCSD) individuals completed the study. In the PVAHCS, the ACT correlated with the asthma medication ratio (AMR) (ρ = 0.37, P < .001) and inversely correlated with the RI (ρ = -0.33, P = .001), the RI inversely correlated with forced expiratory volume in 1 second (FEV(1)) (ρ = -0.22, P = .03) and the FEV(1) to forced vital capacity ratio (ρ = 0.22, P = .03). In the NMCSD population, the ACT correlated with the MARS-A (ρ = 0.23, P = .006), FEV(1) did not correlate with ACT (ρ = 0.09, P = .25) or MARS-A (ρ = 0.16, P = .047) but directly correlated with the RI (ρ = 0.19, P = .03). The AMR was strongly inversely correlated with RI in both populations (-0.74, P < .001 in the PVAHCS group and -0.78, P < .001 in the NMCSD group). When multivariate models were applied to the NMCSD and PVAHCS groups, the combination of RI and MARS-A was the best predictor of spirometry. CONCLUSION: The RI shows promise as a new asthma metric because it correlated with AMR in both cohorts. However, because RI correlated with adult spirometry and ACT only, further validation studies are needed before RI may be included in an ACI metric.

Congenital cystic adenomatoid malformation and bronchogenic cyst in a 4-month-old infant.

Congenital cystic disease of the lung and mediastinum encompasses a continuum of entities, and a histological overlap of many of these anomalies is acknowledged. Moreover, it is possible for different lesions to coexist in the same patient. Careful evaluation prior to surgical resection will alert the surgeon to the possible presence of multiple lesions in one patient.

Vocal cord abnormalities in Williams syndrome: a further manifestation of elastin deficiency.

Williams syndrome (WS) is due to a deletion in the WS critical region at 7q11.23 which includes the elastin gene (ELN). One of the most characteristic features of this disorder is a harsh, brassy, or hoarse voice but the etiology of the vocal characteristics are unknown. We report two patients with WS who had bilateral vocal cord abnormalities, bringing to four the number of children with WS in whom such defects have been documented. We suggest that vocal cord abnormalities may be a far more common feature of WS than has been previously suspected, and that mild vocal cord dysfunction caused by abnormal vocal cord elastin may be the cause of the hoarse voice in this condition.

Chronic inspiratory stridor secondary to a retained penetrating radiolucent esophageal foreign body.

Although foreign body ingestions are common in infants and young children, penetration of the esophagus is a relatively rare event. Timely diagnosis is impeded by the absence of classical symptoms and by the ingestion of radiolucent foreign bodies. The authors present a 17-month-old girl with a 6-month history of inspiratory stridor. An extensive workup found a penetrating radiolucent foreign body at the thoracic inlet.