CT esophagogram in the emergency setting: typical findings and suggested workflow.

PURPOSE: Esophageal perforation (EP) can be a diagnostic challenge. Computed tomography (CT) and CT esophagography (CTE) are often used to rule out EP in the emergency setting with promising diagnostic performance, but the standard of care remains fluoroscopic esophagography (FE). We assess the diagnostic performance of CT and CTE when interpreted by expert and generalist radiologists and created an imaging workflow guide. METHODS: Retrospective study of patients presenting with suspected EP. Two expert radiologists independently reviewed blinded CT/CTE studies, recorded CT findings, and assigned an esophageal injury grade. We also collected initial (general radiologist) CT findings and interpretation and FE diagnoses. We assessed inter-reader reliability and diagnostic performance. RESULTS: EP was diagnosed in 46/139 (33%) encounters. The most common CT/CTE findings in EP were esophageal wall thickening (46/46, 100%), pneumomediastinum (42/46, 91%), and mediastinal stranding (39/46, 85%). CT and CTE sensitivity for detecting EP was 89% and 89% for expert radiologists, respectively, and 79% and 82% for general radiologists, compared with 46% for FE. Inter-reader agreement for detecting EP by CT and CTE was kappa 0.35 and 0.42 (both p < .001) between expert and generalist radiologists. We present radiographic images for key CT/CTE findings and a suggested workflow for the evaluation of possible EP. CONCLUSION: CT and CTE are more sensitive than FE for EP in the emergency setting. Due to the rarity of EP and current wide variability in imaging interpretation, an imaging workflow and injury grading system based on esophageal and mediastinal CT findings are offered to help guide management.

Transient Idiopathic Polyhydramnios: Maternal and Perinatal Outcomes: Maternal and Perinatal Outcomes.

OBJECTIVES: To compare maternal and perinatal outcomes in pregnancies with transient and persistent idiopathic polyhydramnios to those with normal amniotic fluid volume. METHODS: This retrospective cohort study included subjects delivering a singleton pregnancy between January 1, 2015, and June 30, 2020, with sonography ≥26 weeks' gestation demonstrating transient or persistent idiopathic polyhydramnios (amniotic fluid index [AFI] ≥24.0 cm) or normal AFI (referent group). The primary maternal outcome was a composite of spontaneous preterm delivery <37 weeks, cesarean delivery for malpresentation, abnormal labor progress, or nonreassuring fetal status, operative vaginal delivery, hemorrhage requiring transfusion, and umbilical cord prolapse. The primary perinatal outcome was a composite of birthweight >4500 g, fetal or neonatal death, 5-minute Apgar score <7, and neonatal intensive care unit admission for >24 hours. RESULTS: Patients with transient polyhydramnios (n = 259) exhibited maternal outcomes similar to those of the referent group (n = 435) but had significantly increased odds for the primary perinatal outcome (odds ratio [OR] 1.70, 95% confidence interval [CI] 1.15-2.53; P = .008) and for birthweight ≥4500 g (OR 8.70, 95% CI 1.89-40.0; P = .005). Persistent polyhydramnios (n = 176) was significantly associated with both the primary maternal (OR 1.93, 95% CI 1.27-2.95, P = .002) and primary perinatal outcome (OR 2.15, 95% CI 1.40-3.30; P < .001), and individually with cesarean delivery for abnormal labor (OR 3.22, 95% CI 1.55-6.68; P = .002) and birthweight ≥4500 g (OR 8.97, 95% CI 1.84-43.6; P = .007). CONCLUSIONS: Transient idiopathic polyhydramnios does not impact maternal outcomes but is associated with increased odds of newborn birthweight >4500 g. Persistent polyhydramnios is associated with both adverse maternal and perinatal outcomes.

Second-Trimester Ultrasound-Measured Umbilical Cord Insertion-to-Placental Edge Distance: Determining an Outcome-Based Threshold for Identifying Marginal Cord Insertions.

OBJECTIVES: We sought to study potential diagnostic criteria for marginal placental cord insertions as determined by associations with obstetric outcomes. METHODS: This single-center retrospective cohort investigation included singleton gestations delivering from January 1, 2012, to December 31, 2016, and having a standard or detailed fetal ultrasound examination from 18 weeks to 21 weeks 6 days. Cord insertion-to-placental edge distances were used to create a referent comparison group and 3 patient groups: greater than 3.0 cm (comparison); greater than 2.0 to 3.0 cm (group 1); greater than 1.0 to 2.0 cm (group 2); and 1.0 cm or less (group 3). The primary outcome consisted of any one of spontaneous delivery before 37 weeks, fetal growth restriction, oligohydramnios, placental abruption, or intrauterine fetal demise. RESULTS: The numbers of participants and mean distances ± SDs for the comparison group and groups 1 to 3, respectively, were 628 and 4.68 ± 1.17 cm, 106 and 2.52 ± 0.26 cm, 131 and 1.60 ± 0.29 cm, and 77 and 0.36 ± 0.37 cm, respectively. The primary outcome was significantly associated only with cord insertion-to-placental edge distances of 1.0 cm or less (adjusted odds ratio, 3.05; 95% confidence interval, 1.73-5.38). CONCLUSIONS: Marginal cord insertions may be diagnosed when the cord insertion-to-placental edge distance is 1.0 cm or less.

Phenylephrine infusion impact on surgical site infections after lower extremity bypass surgery.

OBJECTIVE: Lower extremity bypass (LEB) operations have high rates of surgical site infections (SSI). Phenylephrine is a commonly used vasoconstrictor which may reduce skin blood flow and increase the likelihood of SSI in these patients. We studied the potential effect of phenylephrine infusion during LEB surgery on SSI. METHODS: LEB cases and their demographic data were identified through the Vascular Quality Initiative registry. SSI in this population was identified using the hospital epidemiology surveillance database. Phenylephrine use in this population was identified through chart review. RESULTS: We identified 699 patients who underwent LEB; 82 (11.7%) developed an SSI, and 244 of 698 (35.0%) were treated with phenylephrine infusion. In bivariate analysis, higher body mass index (28.8 kg/m2 vs 27.3 kg/m2; P = .034), diabetes (14.6% vs 9.4%; P = .035), hypertension (12.6% vs 4.7%; P = .038), groin incision (13.2 vs 5.4%; P = .013) and longer procedure times (17.1% for >220 minutes and 8.9% for ≤220 minutes; P = .003) were associated with higher rates of SSI. Whereas phenylephrine infusion exhibited a trend toward a higher rate (14.8% vs 9.9%; P = .057). In the logistic regression model, diabetes (odds ratio [OR], 1.8; 95% confidence interval [CI], 1.0-3.2; P = .032), total procedure time (OR, 1.85; 95% CI, 1.1-3.1; P = .026) and vertical groin incision (OR, 2.6; 95% CI, 1.1-6.5; P = .035) were independent predictors of increased SSI rates, whereas body mass index (OR, 1.04; 95% CI, 0.99-1.08; P = .09), hypertension (OR, 2.5; 95% CI, 0.6-10.9; P = .22), and phenylephrine infusion (OR, 1.08; 95% CI, 0.63-1.85; P = .78) were not independent predictors of increased SSI rates. CONCLUSIONS: Phenylephrine infusion did not increase the risk of SSI in patients who underwent LEB.

Perinatal Outcomes Associated With Isolated Velamentous Cord Insertion in Singleton and Twin Pregnancies.

OBJECTIVES: To evaluate perinatal outcomes in singleton and twin pregnancies with pathologically confirmed velamentous cord insertion without vasa previa. METHODS: This retrospective case-control study included all nonanomalous singleton and twin pregnancies with pathologically confirmed velamentous cord insertion delivered in a single institution between January 1, 2005, and July 1, 2015, and having an ultrasound examination by maternal-fetal medicine. For each case, the next 2 consecutive deliveries matched for gestational age at delivery ± 1 week and, in twins, amnionicity and chorionicity served as controls. Primary outcomes included surgical delivery for a nonreassuring intrapartum fetal heart rate tracing, umbilical arterial cord pH of less than 7.2, 5-minute Apgar score of less than 7, birth weight below the 10th percentile, neonatal intensive care unit admission, fetal or neonatal death, and cord avulsion necessitating manual placental extraction. RESULTS: Outcomes were available for 53 singletons with 103 matched controls and 33 twin pregnancies with 65 matched controls. In singletons, velamentous cord insertion was associated with cord pH of less than 7.2 (odds ratio [OR] 3.5; 95% confidence interval [CI], 1.1-11.2; P = .039), 5-minute Apgar score of less than 7 (OR, 5.3; 95% CI, 0.99-28.1; P = .045), and cord avulsion requiring manual placental extraction (7.5% versus 0%; P = .012). Associations were suggested with increased surgical delivery for a nonreassuring intrapartum fetal heart rate tracing (OR, 2.4; 95% CI, 0.9-6.9; P = .14), birth weight below the 10th percentile (OR, 2.1; 95% CI, 0.8-5.9; P = .21), and fetal or neonatal death (3.8% versus 0%; P = .11). Velamentous cord insertions were also associated with placental abruption in singletons (7.5% versus 0%; P = .013). Among twins, velamentous cord insertion was associated with fetal or neonatal death (9.1% versus 0%; P = .036). CONCLUSIONS: Isolated confirmed velamentous cord insertion is associated with adverse perinatal outcomes in singleton and twin gestations.

Does Pain Reduction with Oral Steroids Predict Pain Reduction after a First-Time Cervical Epidural Steroid Injection in Patients with Cervical Radicular Pain? A Pilot Study.

OBJECTIVE: Oral and injected steroids are used commonly in the treatment of cervical radicular pain despite a paucity of data demonstrating their efficacy. The purpose of this study is to assess whether the response to orally administered steroids among patients with acute cervical radicular pain who develop recurrent pain is associated with their subsequent response to cervical epidural steroid injections. METHODS: Patients referred to our center were evaluated and then referred for cervical epidural steroid injections at the clinical discretion of the provider; those who met inclusion criteria were offered participation in the study. After the injection was administered, patients were contacted by telephone and asked to complete the Brief Pain Inventory Short Form at one week, one month, three months, and six months postinjection. RESULTS: Pain reduction after cervical steroid injection was not significantly different between 49 patients who reported pain reduction with a prior course of oral steroids and 22 patients who reported no pain reduction. Average pain scores decreased over six months (P < 0.001) among 72 patients treated with epidural steroid injection for cervical radicular pain. Of the 55 who provided baseline and six-month data, 14 (25.5%) reported complete relief at six months and 20 (36.4%) reported decreased pain. CONCLUSIONS: Patients can be reassured that they may experience pain reduction after a cervical epidural steroid injection even if oral steroid therapy was not effective. The majority of patients treated for cervical radicular pain with epidural steroid injection have reduced or absent pain for at least six months after treatment.

A prospective, randomized trial of liposomal bupivacaine compared to conventional bupivacaine on pain control and postoperative opioid use in adults receiving adductor canal blocks for total knee arthroplasty.

BACKGROUND: Total knee arthroplasty (TKA) is a commonly performed procedure to alleviate pain and improve functional limitations caused by end-stage joint damage. Effective management of postoperative pain following TKA is crucial to the prevention of complications and enhancement of recovery. Adductor canal blocks (ACB) with conventional bupivacaine (CB) provide adequate analgesia after TKA, but carry a risk of rebound pain following block resolution. Liposomal bupivacaine (LB) is an extended-release local anesthetic that can provide up to 72 h of pain relief. The objective of this study was to compare postoperative outcomes between ACBs using LB and CB after TKA. METHODS: This single institution, prospective, randomized, clinical trial enrolled patients scheduled for TKA. Participants were randomized to receive ACB with either LB or CB. Pain scores up to 72 h postoperatively were assessed as the primary outcome. Opioid consumption and length of stay were evaluated as secondary outcomes. RESULTS: A total of 80 patients were enrolled. Demographic and clinical characteristics were similar between the two groups. LB group showed significantly lower cumulative opioid use during the 72 h evaluated (P = 0.016). There were no differences in pain scores or length of stay between the groups. CONCLUSION: The study demonstrated that LB ACBs led to significantly lower opioid consumption in the days following TKA without affecting pain scores or length of stay. This finding has important implications for improving postoperative outcomes and reducing opioid use in TKA patients. Previous studies have reported inconsistent results regarding the benefits of LB, highlighting the need for further research. TRIAL REGISTRATION: This project was retrospectively registered with clinicaltrials.gov ( NCT05635916 ) on 2 December 2022.

Uncomplicated Streptococcal Bacteremia: The Era of Oral Antibiotic Step-down Therapy?

BACKGROUND: The purpose of this study was to compare the clinical outcomes of adults with uncomplicated streptococcal bacteremia who received either oral (PO) step-down or continued intravenous (IV) therapy. METHODS: This was a retrospective, single-center, cohort study, including adults admitted with Streptococcal bloodstream infection between January 1, 2013, and December 31, 2020. Only patients with uncomplicated Streptococcal bloodstream infections were included. Patients who transitioned to PO therapy within 5 days from bacteremia onset were compared to patients receiving continued IV therapy. The primary outcome was clinical failure, defined by either 90-day hospital readmission or mortality. Secondary outcomes included hospital length of stay (LOS) and antibiotic-related adverse events (AAEs). RESULTS: Of the 264 patients included, 42% were transitioned to PO therapy. Group B Streptococcus (22.7%) was the most common isolate. The most common sources of infection were skin and soft tissue (35%) and pulmonary (25%). Intensive care unit (ICU) stay was more common in the continued IV therapy group (22.2%) than in the PO step-down group (5.4%). The frequency of clinical failure was similar in the IV and PO groups (24.2% vs. 18.0%, P=0.23). The IV group had longer hospital LOS (median, [interquartile range (IQR)]) compared with the PO group (7 [5-13.5] vs. 4 [3-5] days, P<0.001). The incidence of AAEs was similar in the IV and PO groups (1.3% vs. 1.8%, P=0.74). CONCLUSION: Oral antibiotic step-down therapy may be appropriate for the treatment of uncomplicated Streptococcal bacteremia, with consideration of factors such as patient comorbidities, type of infection, source control and clinical progress.

Buprenorphine Continuation During Critical Illness Associated With Decreased Inpatient Opioid Use in Individuals Maintained on Buprenorphine for Opioid Use Disorder in a Retrospective Study.

The number of patients maintained on buprenorphine is steadily increasing. To date, no study has reported buprenorphine management practices for these patients during critical illness, nor its relationship with supplemental full-agonist opioid administration during their hospital stay. In this single-center retrospective study, we have explored the incidence of buprenorphine continuation during critical illness among patients receiving buprenorphine for the treatment of opioid use disorder. Additionally, we investigated the relationship between nonbuprenorphine opioid exposure and buprenorphine administration during the intensive care unit (ICU) and post-ICU phases of care. Our study included adults maintained on buprenorphine for opioid use disorder admitted to the ICU between December 1, 2014, and May 31, 2019. Nonbuprenorphine, full agonist opioid doses were converted to fentanyl equivalents (FEs). Fifty-one (44%) patients received buprenorphine during the ICU phase of care, with an average dose of 8 (8-12) mg/day. During the post-ICU phase of care, 68 (62%) received buprenorphine, with an average dose of 10 (7-14) mg/day. Lack of mechanical ventilation and acetaminophen use were also associated with buprenorphine use. Full agonist opioid use was more frequent on days when buprenorphine was not given (odds ratio [OR], 6.2 [95% CI, 2.3-16.4]; P < .001). Additionally, the average cumulative dose of opioids given on nonbuprenorphine administration days was significantly higher both in the ICU (OR, 1803 [95% CI, 1271-2553] vs OR, 327 [95% CI, 152-708] FEs/day; P < 0.001) and after ICU discharge (OR, 1476 [95% CI, 962-2265] vs OR, 238 [95% CI, 150-377] FEs/day; P < .001). Given these findings, buprenorphine continuation during critical illness should be considered, as it is associated with significantly decreased full agonist opioid use.

A prospective, randomized trial of the effect of buprenorphine continuation versus dose reduction on pain control and post-operative opioid use.

INTRODUCTION: An increasing number of individuals are taking buprenorphine for management of opioid use disorder (OUD). Pain control can be challenging when these patients develop acute pain requiring supplemental analgesia. Buprenorphine's pharmacokinetic profile can render supplemental opioid-based analgesia ineffective. There is limited guidance on the optimal management of buprenorphine when acute pain is anticipated. Although there is growing acceptance that the risk of OUD relapse with buprenorphine discontinuation overshadows the risks of increased opioid utilization and difficult pain control with buprenorphine continuation, perioperative courses comparing buprenorphine dose reduction and full dose buprenorphine continuation have yet to be investigated. Here, we describe the protocol for our randomized controlled, prospective trial investigating the effect of buprenorphine continuation compared to buprenorphine dose reduction on pain control, post-operative opioid use, and OUD symptom management in patients on buprenorphine scheduled for elective surgery. METHODS AND ANALYSIS: This is a single institution, randomized trial that aims to enroll 80 adults using 12 mg buprenorphine or greater for treatment of OUD, scheduled for elective surgery. Participants will be randomly assigned to receive 8mg of buprenorphine on the day of surgery onwards until postsurgical pain subsides or to have their buprenorphine formulation continued at full dose perioperatively. Primary outcome will be a clinically significant difference in pain scores 24 hours following surgery. Secondary outcomes will be opioid consumption at 24, 48, and 72 hours postoperatively, opioid dispensing up to 30 days following surgery, changes in mood and withdrawal symptoms, opioid cravings, relapse of opioid misuse, and continued use of buprenorphine treatment postoperatively.

The antinuclear antibody assay: developing criteria for reflexive anti-dsDNA antibody testing in a laboratory setting.

BACKGROUND: We examined the relationship between antinuclear antibody (ANA) data and the presence of anti-double stranded DNA antibodies (anti-dsDNA). METHODS: De-identified demographic, ANA and anti-dsDNA data were available for 30,196 individuals aged ≥ 20 years, whose sera were submitted sequentially to our laboratory. When multiple sera were received for the same subject, data from the earliest sample were used. Anti-dsDNA frequency was stratified by ANA titer and pattern, sample referral source, and by the patient's age, gender, and diagnosis. RESULTS: For sera with ANA titer ≥ 256 and an accompanying diagnosis of systemic lupus erythematosus, anti-dsDNA frequency was 53.7%, 35.3%, and 37.5% for homogeneous, speckled, or multiple ANA patterns, respectively. Among remaining sera with ANA titer ≥ 256, anti-dsDNA frequency was highest for the homogeneous pattern (15.9%). Anti-dsDNA frequency was three-fold higher among sera submitted by rheumatologists compared with other providers. However, its relative distribution by ANA pattern and titer was similar between these groups. Patient age and gender had no significant effect on anti-dsDNA frequency after ANA data were taken into account. CONCLUSIONS: ANA pattern and titer, together with the diagnosis submitted with the serum sample, can be used to guide decisions for reflexive anti-dsDNA testing in a clinical laboratory setting.

The relationship between antinuclear antibody data and antibodies against extractable nuclear antigens in a large laboratory cohort.

BACKGROUND: Anti-extractable nuclear antigen antibodies (anti-ENA) have diagnostic significance in systemic rheumatic disease (SRD). METHODS: Anti-ENA were tested in 1685/30,196 sera that were submitted sequentially for antinuclear antibody (ANA) testing. Frequency was stratified by ANA titer and pattern, by referral source, by submitted diagnosis and by patient age and sex. RESULTS: Anti-ENA frequency increased with ANA titer (7.3% at < 32%-43.3% at ≥ 1024). Anti-histone (11.6%) and anti-SSA/SSB (13.9%) were the most frequent finding with a homo\xadgeneous pattern; anti-SSA/SSB (39.7%) and anti-RNP/anti-Sm (37.7%) were the most frequent finding with a speckled pattern. Sera with speckled, multiple and homogenous ANA patterns accounted for 92.6% of positive anti-ENA findings. At ANA titer ≥ 256, 29.2% of these sera were tested for anti-ENA, of which 41.2% were positive; frequency was higher with an accompanying diagnosis of SRD (53.5% vs. 36.5%, p < 0.004 by χ2-test) but not with referral by rheumatologists (43.5% vs. 35.9%) and did not differ by patient age or sex. CONCLUSIONS: Reflexive anti-ENA testing may be helpful among sera with ANA titer ≥ 256 and homogeneous, speckled or multiple patterns, irrespective of referral source or accompanying diagnosis. Further work is needed to evaluate the clinical impact of this protocol.

Safety of ECT in patients receiving an oral anticoagulant.

INTRODUCTION: This study assessed the use, tolerability, and safety of anticoagulation via direct oral anticoagulants or warfarin in medical and psychiatric inpatients receiving ECT. METHODS: This retrospective cohort study included 32 patients who received ECT while on either a direct oral anticoagulant (9) or warfarin (23) and spanned 247 encounters at Maine Medical Center between December 2012 and December 2018. Data are presented descriptively and analyzed using SPSS version 25 and Microsoft Excel version 2016. RESULTS: Among the 247 ECT patient encounters, there were few major adverse effects of ECT in this medically complex population. These adverse effects included headache during 4 encounters (1.6%), respiratory distress during 2 encounters (0.8%) and a cardiovascular event during 1 encounter (0.4%). One patient (3.1%) who was receiving concurrent rivaroxaban and venlafaxine experienced gastrointestinal bleeding that was determined to be unrelated to ECT. One patient on fluoxetine and warfarin experienced hemoptysis thought to be secondary to epistaxis. No other major bleeding or clotting event occurred during an ECT session nor for the duration of the hospitalization. DISCUSSION: Direct oral anticoagulants and warfarin appear safe in the treatment of patients with atrial fibrillation or acute venous thromboembolism who are receiving concomitant ECT. Prospective studies are needed to confirm these findings.

Efficacy of Sex Steroid Therapy Without Progestin or GnRH Agonist for Gonadal Suppression in Adult Transgender Patients.

CONTEXT: Testosterone (T) or estradiol (E2) are administered to suppress gonadal function in female-to-male (FTM) and male-to-female (MTF) transgender patients. How often sex steroids cause adequate suppression without GnRH agonist (GnRHa) or progestin therapy has not been reported. OBJECTIVES: (1) To determine how often T and E2 therapy alone can effectively suppress gonadal function in MTF and FTM transgender patients, and (2) to determine the frequency and range of serum E2 levels above the normal male range in FTM patients receiving T therapy. DESIGN: Retrospective cohort study. SETTING: Outpatient reproductive endocrinology clinic at an academic medical center. PATIENTS: A total of 65 FTM and 33 MTF patients were included who were > 18 years of age and not receiving progestin or GnRHa therapy. INTERVENTION: Female-to-male patients were receiving T through injections or gel. Male-to-female patients were receiving oral or subcutaneous E2. MAIN OUTCOME MEASUREMENTS: In FTM patients the indicator of ovary suppression was amenorrhea. In MTF patients, the indicator of testes suppression was T levels <50 ng/dL. RESULTS: Median serum total T level for FTM patients was 712 ng/dL (range, 370-1164 ng/dL). On T therapy alone, 90.8% of patients achieved amenorrhea and 49.2% of patients had serum E2 levels above the normal range for women. For MTF patients, the median serum E2 level was 129.2 pg/mL (range, 75-197 pg/mL). On E2 therapy alone, 84.8% of MTF patients had adequate suppression of testicular function. CONCLUSIONS: Testosterone and E2 therapy are usually effective without progestin or GnRHa therapy to suppress gonadal function in transgender patients. Progestin and/or GnRHa therapy should only be initiated in those patients who do not have adequate gonadal suppression on optimized doses of T or E2 alone.

Urinary stone disease prevalence and associations in cystic fibrosis.

Cystic fibrosis (CF) may predispose patients to urinary stone disease (USD), but reported prevalence of USD in patients with CF in previous small studies is variable. To date, analysis of risk factors for USD within the CF population has been limited. We studied 29,396 patients in the Cystic Fibrosis Foundation Patient Registry to calculate age and sex-stratified prevalence of USD. For adult patients, we examined age and multivariable-adjusted cross-sectional associations between demographic and clinical factors, CFTR mutation class, and prevalent USD. Prevalence of USD was 0.4% (95% CI 0.3-0.5%) under age 18 years, 3.1% (2.7-3.6%) at 18-24 years, 6.4% (5.8-7.1%) at 25-34 years, 7.5% (6.5-8.5%) at 35-44 years, and 6.7% (5.8-7.8%) at 45 years and older. Prevalence for women was higher than men at younger (< 45 years) but not older ages (P value for interaction < 0.0005). Multivariable odds of prevalent USD were significantly increased for severe CFTR mutations, OR 1.53 (1.14-2.06), diabetes, OR 1.24 (1.03-1.50), hypertension, OR 1.58 (1.29-1.93), and chronic macrolide therapy, OR 1.27 (1.07-1.52). BMI was not associated with USD. USD prevalence in CF is similar to that in the general population. With the exception of BMI, known risk factors for USD in the general population also appear to be important for patients with CF. We identified several novel associations in CF patients, including greater prevalence of USD in individuals with severe CFTR mutations and among young women.

Methadone bioavailability and dose conversion implications with intravenous and enteral administration: A scoping review.

PURPOSE: Despite its availability for more than 70 years, many details concerning methadone remain contentious, such as the dosing equivalents for intravenous and enteral administration. A scoping review was performed to evaluate whether existing literature on methadone bioavailability in human subjects support the current recommendation that an equivalent enteral dose is twice the intravenous dose. METHODS: A librarian-assisted search of the PubMed and EMBASE databases identified all English-language articles with the terms methadone and bioavailability and/or conversion in the title or abstract published from inception though December 2019. A manual search of references was also performed to identify any additional articles. Studies were included in a scoping review if they were published in English and evaluated methadone bioavailability in human subjects. RESULTS: Among 65 publications initially identified, 6 studies involving a total of 50 patients were included in the review. Bioavailability data for healthy volunteers and patients with opioid use disorder, metastatic cancer, chronic pain from malignant or nonmalignant disease were available for analysis. The pooled mean (95% confidence interval) bioavailability (F) was 85.4% (75.2%-95.6%), with heterogeneity (I2) of 0. In the 4 studies that provided individual patient-level data, F was >50% in 40 of 42 patient measurements (95.2%) and ≥75% in 33 of 42 patient measurements (78.6%). CONCLUSION: Available evidence suggests the bioavailability of methadone is generally more than 75%, there is limited evidence for the currently recommended 1:2 ratio (intravenous:enteral), and a more appropriate dosing ratio may be 1:1.3. This scoping review underscores the need for further research to establish an effective and safe ratio when converting between intravenous and enteral dosing formulations of methadone.

Prevalence of steroid sulfatase deficiency in California according to race and ethnicity.

OBJECTIVE: Estimate steroid sulfatase deficiency (STSD) prevalence among California's racial/ethnic groups using data from a previous study focused on prenatal detection of Smith-Lemli-Opitz syndrome (SLOS). SLOS and STSD both have low maternal serum unconjugated estriol (uE3) levels. METHODS: Prevalence was estimated using three steps: listing clinically identified cases; modeling STSD frequency at three uE3 intervals using diagnostic urine steroid measurements; applying this model to determine frequency in pregnancies not providing urine. RESULTS: Overall, 2151 of 777 088 pregnancies (0.28%) were screen positive; 1379 of these were explained and excluded. Fifty-four cases were diagnosed clinically among 707 remaining pregnancies with a male fetus. Urine steroid testing identified 74 additional STSD cases: 66 (89.2%) at uE3 values < 0.15 MoM, 8 (10.8%) at 0.15-0.20 MoM, and 0 (0%) at > 0.20 MoM. Modeling estimated 107.5 STSD cases among 370 pregnancies without urine samples. In males, STSD prevalence was highest among non-Hispanic Whites (1:1230) compared to Hispanics (1:1620) and Asians (1:1790), but differences were not significant. No STSD pregnancies were found among 65 screen positive Black women. CONCLUSION: The overall prevalence estimate of 1:1500 males is consistent with published estimates and is reasonable for counseling, except among Black pregnancies where no reliable estimate could be made.