Revision Thoracic Syringo-Subarachnoid Shunt for Recurrent Syrinx With Syringobulbia: Technique and Surgical Video.

Syringomyelia and syringobulbia continue to remain a diagnosis without widely accepted treatment paradigms. Furthermore, the currently available treatment options can be complicated by delayed symptom recurrence and the need for revision surgery. Revision intradural surgery is challenging, and currently, there is a paucity of literature describing safe techniques for revision syringotomy and shunt placement. In this technical report, we present a surgical video describing the technique of revision syringo-subarachnoid shunt placement in a 61-year-old female with a history of multiple intradural surgeries who presented with progressively symptomatic ascending syringobulbia.

Adults with possible food protein-induced enterocolitis syndrome with crustacean ingestion.

BACKGROUND: Food protein-induced enterocolitis (FPIES), an entity previously thought to only affect children, has been increasingly described in adults. In this study, we report a Canadian cohort of 19 adolescents and adults with recurrent non-immunoglobulin E (IgE)-mediated gastrointestinal symptoms after crustacean ingestion, consistent with FPIES. METHODS: We conducted a retrospective chart review of patients in an outpatient allergy clinic from January 2005 to May 2020. Electronic records were searched using keywords for crustaceans and for symptoms consistent with FPIES. We included patients with gastrointestinal symptoms specifically to crustaceans on more than one occasion, who were 14 years or older at the time of index reaction. Exclusion criteria included symptoms suggestive of an IgE-mediated anaphylactic reaction or a likely alternative diagnosis. We identified 19 patients for our cohort who met the criteria. RESULTS: Our cohort was 68.4% female (13) and 32.6% (6) male. The average age at first reaction to crustaceans was 34 years old with a range of 14-68 years (median = 28 years; IQR = 32 years). Time from ingestion to beginning of symptoms ranged from 3 min to 6.5 h, with an average of 2.8 h (median = 2 h; IQR = 3.25 h). Duration of reaction ranged from less than a minute to over 48 h, with a mean of 9.4 h (median = 4 h; IQR = 7.75 h). Patients had 4.8 reactions on average; however, number of reactions ranged from 2 to 12.5 (median = 3, IQR = 3). All patients identified a "trigger" food in the crustacean group, and 12 subjects identified additional reactions to other seafood. CONCLUSIONS: This case series will better characterize and advance our understanding of this disease entity in adults. There are key differences in the presentation of FPIES in adults compared to children, namely female predominance, difference in solid food trigger, and unpredictable time course. Future studies are needed to examine the pathophysiology and natural history of adult FPIES. Specific guidelines should be developed for the diagnosis and management in adults. TRIAL REGISTRATION: retrospectively registered.

Point-of-care ultrasonography in the allergy and immunology clinic.

OBJECTIVE: To summarize evidence supporting the use of point-of-care ultrasonography as a clinical tool for allergists and immunologists. DATA SOURCES: Cochrane Library, Medline, EMBASE, and Scopus databases were searched for articles published before December 18, 2018. STUDY SELECTIONS: We included any retrospective or prospective study that evaluated ultrasonography in allergy and immunology and epinephrine autoinjector (EAI) needle length. RESULTS: The standard EAI needle length may be inadequate for intramuscular delivery of epinephrine, particularly for women, at risk of anaphylaxis. In patients who weigh less than 15 kg, the lengths of commercially available EAIs may be too long, risking inadvertent intraosseous injection and resultant complications. Ultrasonography can be routinely used in the allergy clinic to guide needle length and angle for subcutaneous allergen immunotherapy injections to minimize systemic adverse effects. CONCLUSION: Point-of-care ultrasonography can be a useful tool to enhance patient care and safety in an allergy clinic. Ideally, all patients prescribed EAIs should have ultrasonographic measurement of the skin to muscle distance and skin to bone distance to assist in identifying patients at risk of subcutaneous or intraosseous injection in anaphylaxis and those at risk of intramuscular injection during subcutaneous allergen immunotherapy injections.

Influence of Education on Disease Activity and Damage in Systemic Lupus Erythematosus: Data From the 1000 Canadian Faces of Lupus.

OBJECTIVE: To determine whether socioeconomic status assessed by education is associated with disease activity and the risk of organ damage in systemic lupus erythematosus (SLE). METHODS: Data from the 1000 Canadian Faces of Lupus, a multicenter database of adult SLE patients, was used to compare education as either low (did not complete high school) or high (completed high school or further) for disease activity and damage. Education was also studied as a continuous variable. The relationships between education and SLE outcomes (any organ damage defined as a Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index [SDI] score ≥1, serious organ damage [SDI score ≥3], and end-stage renal disease) were evaluated using logistic regression analyses adjusted for age, sex, race/ethnicity, and disease duration. RESULTS: A total of 562 SLE patients met inclusion criteria (mean age 47 years, 91% female, and mean disease duration of 10 years); 81% had high education. The low education group was twice as likely to be work disabled (30%; P < 0.0001); they had higher disease activity and reduced renal function. Linear regression analysis revealed that low education was significantly associated with higher disease activity at enrollment into the 1000 Canadian Faces of Lupus database, after adjustment for age (at entry and at diagnosis), race/ethnicity, and sex (B 1.255 + 0.507 [SE], ß = 0.115, P = 0.014). In our adjusted logistic regression models we were unable to demonstrate significant associations between education and SLE damage. Results did not change when varying the education variable. CONCLUSION: In this cohort, low education was associated cross-sectionally with higher disease activity and work disability, but not damage.