Prevalence of Undiagnosed Acute Hepatic Porphyria in Cyclic Vomiting Syndrome and Overlap in Clinical Symptoms.

BACKGROUND AND AIMS: Acute hepatic porphyria (AHP) presents with nausea and vomiting and can mimic cyclic vomiting syndrome (CVS). The prevalence of AHP in CVS and overlap in clinical symptomatology is not known. We thus sought to determine the prevalence of pathogenic variants for AHP and characterize symptom overlap between CVS and AHP. METHODS: We conducted a cross-sectional study of 234 CVS patients using Rome criteria. Patients were eligible for AHP genetic testing if they had recurrent episodes of severe, diffuse abdominal pain with ≥ 2 of the following-peripheral nervous system (muscle weakness/aching, numbness, tingling), central nervous system (confusion, anxiety, seizures, hallucinations), autonomic nervous system (hyponatremia, tachycardia, hypertension, constipation) symptoms, red/brownish urine, or blistering skin lesions on sun-exposed areas. A family history of AHP or elevated urinary porphobilinogen (PBG)/aminolaevulinic acid (ALA) were also criteria for genetic testing and was performed using a 4-gene panel. RESULTS: Mean age was 38.7 ± 14.5 years, 180 (76.9%) were female and 200 (85.5%) were Caucasian. During a CVS attack, 173 (92%) reported abdominal pain, 166 (87.2%) had peripheral nervous system, 164 (86.8%) had central nervous system and 173 (92) % had autonomic symptoms. Ninety-one eligible patients completed genetic testing. None were positive for AHP but two had variants of uncertain significance (VUS) in the HMBS gene. CONCLUSIONS: There is a high prevalence of non-gastrointestinal symptoms in CVS, like AHP, which is important for clinicians to recognize. AHP was not detected in this study and larger studies are warranted to ascertain its prevalence.

Black patients with multiple myeloma have better survival than white patients when treated equally: a matched cohort study.

We assessed differences in survival between non-Hispanic black (NHB) and non-Hispanic white (NHW) patients with multiple myeloma (MM), and the sequential effects of patient characteristics, and diagnosis and treatment-related factors on the survival disparity using data from 3319 NHB and 20,831 NHW MM patients in the SEER-Medicare (1999-2017) database. Four sets of 3319 NHWs were matched sequentially to the same set of 3319 NHBs, based on demographics (age, sex, year of diagnosis, marital status, and SEER site), socioeconomic status (SES, demographics plus SES), presentation factors (SES variables plus comorbidity), and treatment factors (presentation variables plus antimyeloma therapies). We found NHBs were less likely to receive treatment than NHWs even among patients matched for demographics, SES, and comorbidities. The absolute difference in 5-year survival between NHBs and NHWs was not significant in the demographics match (0.6%; P = 0.30) and remained non-significant after matching for SES (1.4%, P = 0.17). When matching for presentation, NHBs had significantly longer 5-year survival than NHWs (absolute difference = 3.8%, P = 0.003). Additional matching on treatment-related factors further enlarged the racial difference in 5-year survival to 4.6% (P < 0.001). Our findings reinforce the importance of equitable access to effective treatment modalities to further improve the survival of NHB patients with MM.

Guideline-concordant treatment predicts survival: a National Cancer Database validation study of novel composite locoregional and systemic treatment scores among women with early stage breast cancer.

BACKGROUND: The aim of this large nationwide study was to validate two novel composite treatment scores that address guideline-concordant locoregional and systemic breast cancer care. We examined the relationship between these two scores and their association with survival. METHODS: Women with Stage I-III unilateral breast cancer were identified within the National Cancer Database. For each woman, a locoregional and a systemic treatment score (0, 1, 2) was assigned based on receipt of guideline-concordant care. Multivariable Cox regression models evaluated the association between the scores and survival. RESULTS: 623,756 women were treated at 1,221 different American College of Surgeons Commission on Cancer (CoC) facilities. Overall, 86% had a locoregional treatment score of 2 (most guideline-concordant), 75% had a systemic treatment score of 2, and 72% had both scores of 2. Median follow-up was 4.5 years. Compared to women with a locoregional treatment score of 2, those with a score of 1 or 0 had a 1.7-fold and 2.0-fold adjusted greater risk of death. Compared to women with a systemic treatment score of 2, those with a score of 1 or 0 had a 1.5-fold and 2.1-fold adjusted greater risk of death. Risk-adjusted 5-year overall survival was 91.6% when both scores were 2 compared to 73.4% when both scores were 0. CONCLUSIONS: In this large national study of CoC facilities, two composite scores capturing guideline-concordant breast cancer care had independent and combined robust effects on survival. These clinically constructed novel scores are promising tools for health services research and quality-of-care studies.

The impact of a cardiopulmonary resuscitation video on reducing surrogates' anxiety: A pilot randomized controlled trial.

PURPOSE: To test the primary hypothesis that a CPR video will reduce ICU patients' surrogates' anxiety when deciding code status, as measured by the Hamilton Anxiety Rating (HAM-A) Scale, as compared to the no video group. MATERIALS AND METHODS: This is a prospective randomized control trial. Twenty-seven ICU patients' surrogates were enrolled in the study after receiving an ICU team-led code status discussion. After the enrollment, twelve surrogates were randomized to the video group and fifteen to the no video group. The primary outcome of anxiety was quantified using the HAM-A Scale. Demographic information, clinical data, and patients' provenance information (Home vs. Not Home) were collected. The patients' severity of illness was calculated using the Sequential Organ Failure Assessment (SOFA) Score. RESULTS: The HAM-A score in the video group was 5.65 points lower than in the no video group ([ß = -5.65, 95% CI -11.12 -0.18] P = 0.04). The statistically significant difference was maintained when adjusting for patients' SOFA Score and patients' provenance (P = 0.03). CONCLUSION: CPR video used to supplement ICU team-led code status discussions reduced surrogates' anxiety, as compared to no video. TRIAL REGISTRATION: ClinicalTrials.gov Identifier NCT03630965.

Hot-Water Bathing Improves Symptoms in Patients with Cyclic Vomiting Syndrome and Is Modulated by Chronic Cannabis Use.

BACKGROUND: Cyclic vomiting syndrome (CVS) is a chronic functional GI disorder; a characteristic compulsive "hot-water bathing" pattern is reported to alleviate symptoms during an acute episode. There is limited data on this bathing pattern: proposed mechanisms include core temperature increase via effects on cannabinoid type 1 receptors in the brain, skin transient receptor potential vanilloid 1 receptor stimulation, and blood flow shift from viscera to skin. AIMS: We thus sought to characterize the hot-water bathing pattern in patients with CVS and identify differences between heavy cannabis users in comparison to occasional and non-users. METHODS: We conducted a cross-sectional study of 111 patients with CVS at a single tertiary referral center. Questionnaires regarding clinical characteristics, hot-water bathing, and cannabis use were administered. Patients were classified based on cannabis usage into regular cannabis users (≥ 4 times/week), and occasional + non-users (< 4 times/week and no current use). RESULTS: A total of 81 (73%) respondents reported the hot-water bathing behavior during an episode. The majority (> 80%) noted a marked improvement in nausea, vomiting, abdominal pain and symptoms associated with panic. Regular cannabis users were more likely to use "very-hot" water (50% vs. 16%, p = 0.01) and time to relief of symptoms was longer (> 10 min) in this group, compared to the rest of the cohort. CONCLUSIONS: Hot-water bathing relieves both GI and symptoms related to panic in most patients which appear to be modulated by chronic cannabis use. These findings can help inform future physiologic studies in CVS pathogenesis.

AKI in Hospitalized Patients with COVID-19 and Seasonal Influenza: A Comparative Analysis.

Background: Coronavirus disease 2019 (COVID-19) is often compared with seasonal influenza and the two diseases have similarities, including the risk of systemic manifestations such as AKI. The aim of this study was to perform a comparative analysis of the prevalence, risk factors, and outcomes of AKI in patients who were hospitalized with COVID-19 and influenza. Methods: Retrospective cohort study of patients who were hospitalized with COVID-19 (n=325) or seasonal influenza (n=433). AKI was defined by Kidney Disease: Improving Global Outcomes (KDIGO) criteria. Baseline characteristics and hospitalization data were collected, and multivariable analysis was performed to determine the independent predictors for AKI. Results: AKI occurred in 33% of COVID-19 hospitalizations (COV-AKI) and 33% of influenza hospitalizations (FLU-AKI). After adjusting for age, sex, and comorbidity count, the risk of stage 3 AKI was significantly higher in COV-AKI (OR, 3.46; 95% CI, 1.63 to 7.37). Pre-existing CKD was associated with a six- to seven-fold increased likelihood for FLU-AKI and COV-AKI. Mechanical ventilation was associated with a higher likelihood of developing AKI in the COVID-19 cohort (OR, 5.85; 95% CI, 2.30 to 15.63). Black race, after adjustment for comorbidities, was an independent risk for COV-AKI. Conclusions: Pre-existing CKD was a major risk factor for AKI in both cohorts. Black race (independent of comorbidities) and mechanical ventilation were associated with a higher risk of developing COV-AKI, which is characterized by a higher burden of stage 3 AKI and overall poorer prognosis.