Acquired Non-histaminergic Angioedema With C1q Autoantibody and Urticaria: A Case Report.

Acquired angioedema (AAE) is a rare disease with life-threatening complications. This pathology has classically been associated with medication use and B cell lymphoproliferative disorders. In this report, we describe a 61-year-old man with a six-year history of angioedema, unrelated to any known triggers or malignancy. Extensive workup has led to a diagnosis of idiopathic nonhistaminergic AAE with normal C1 inhibitor. The patient is currently being treated with lanadelumab, which has resolved the patient's symptoms. This case provides insight into the onset, exploration, treatment, and outcomes of an extremely rare disease process.

Cognitive profile of children with neurofibromatosis and reading disabilities.

A large percentage of children with Neurofibromatosis Type 1(NF-1) have learning disabilities, often in the realm of reading. Previous studies have indicated that children with NF-1 show a neuropsychological profile similar to idiopathic reading disabilities (IRD); however, studies typically have not subdivided children with NF-1 into those who do and do not have RD (NF+RD and NFnoRD, respectively). The current study examined the cognitive profile of children with NF-1 with and without RD and compared them to children with IRD as well as to typically developing readers (Controls). Findings showed that children with NF+RD performed similarly to children with IRD on phonological, rapid naming, and reading comprehension measures; however, children with NF+RD displayed pronounced visual-spatial deficits as compared to IRD and Control groups. In addition, when comparing the NF-1 groups to each other as well as to the control and IRD groups, the current study reported that there were no oral language differences; lack of findings in the realm of oral language was attributed to the fact that groups were equated on IQ. Overall, findings suggest that a more refined classification of children with NF-1 may be helpful for tailoring academic interventions.

Effects of fluency, oral language, and executive function on reading comprehension performance.

Reading disability (RD) typically consists of deficits in word reading accuracy and/or reading comprehension. While it is well known that word reading accuracy deficits lead to comprehension deficits (general reading disability, GRD), less is understood about neuropsychological profiles of children who exhibit adequate word reading accuracy but nevertheless develop specific reading comprehension deficits (S-RCD). Establishing the underlying neuropsychological processes associated with different RD types is essential for ultimately understanding core neurobiological bases of reading comprehension. To this end, the present study investigated isolated and contextual word fluency, oral language, and executive function on reading comprehension performance in 56 9- to 14-year-old children [21 typically developing (TD), 18 GRD, and 17 S-RCD]. Results indicated that TD and S-RCD participants read isolated words at a faster rate than participants with GRD; however, both RD groups had contextual word fluency and oral language weaknesses. Additionally, S-RCD participants showed prominent weaknesses in executive function. Implications for understanding the neuropsychological bases for reading comprehension are discussed.

The contribution of executive skills to reading comprehension.

Although word recognition deficits (WRD) are a known cause of reading comprehension deficits (RCD), other contributions to RCD, including executive function (EF), have not been fully explored. We examined the contribution of EF (working memory and planning), along with attention, decoding, fluency, and vocabulary to reading comprehension in 60 children (including 16 WRD and 10 RCD), ages 9-15 years. After controlling for commonly accepted contributors to reading comprehension (i.e., attention, decoding skills, fluency, and vocabulary), EF continued to make a significant contribution to reading comprehension but not to word recognition skills. These findings highlight the need for consideration of the role of EF in RCD.

Cognitive profile of neurofibromatosis type 1.

General consensus has yet to be reached with regard to the exact cognitive profile of children with neurofibromatosis type 1 (NF1). The current overview seeks to provide a more comprehensive review of the literature by examining studies that have specifically compared individuals with NF1 to siblings, controls, and/or norms. We also examined results of studies that compared individuals with NF1 with various manifestations to each other. Consistent with previous reviews, no clear cognitive profile emerged; however, greater insight into patterns was obtained. Additionally, future directions for research on NF1 were suggested.