Recent Clinic Visit Research Articles

The impact of tricuspid regurgitation on the timing of pulmonary valve replacement in adult patients after Tetralogy of Fallot repair

Abstract Background Tricuspid regurgitation (TR) in patients with repaired tetralogy of Fallot (rTOF) is an important finding with a wide spectrum of etiologies. TR represents an important risk factor in adult patients with rTOF, influencing clinical outcomes and the requirement for additional surgical interventions. In this study, we reviewed the long-term outcomes of TR in ACHD patients with rTOF as well as its impact on the timing of surgical pulmonary valve replacement (PVR). Methods This was a retrospective analysis of a single institutional cohort which included 149 ACHD patients with a history of rTOF (transannular patch only) with CMR data, complete follow-up, and a recent (< 1 year) outpatient clinic visit. Results Overall follow-up time was 25.5 years (95% CI 24.5,26.4). Between 2002 and 2021, 93 patients required isolated PVR at a mean time from TOF repair of 16.6 years (95% CI 15.5,17.7) and at a mean age of 17.9 years (95% CI 16.8,19.0). In the whole cohort, significant TR was associated with a two-fold increase in the likelihood of requiring PVR (log-rank p = 0-.0023, HR 2.02; 1.09-4-47). Moderate to severe TR was more frequent in those who underwent VSD closure through a right atriotomy (17 versus 0, p = .0002) and affected time to PVR (13.9 versus 16.3 years, p = .0071), with equal preoperative RVEDVi at CMR. After PVR, RVEDVi normalized in all patients, regardless of the preoperative amount of TR (median 115.3 ml/m2 versus 112.3 ml/m2, p = .7). At a median time of 6.5 years from PVR, 13 patients (13/93, 14%) underwent redo PVR. Conclusions The preoperative severity of tricuspid regurgitation (TR) may have a substantial role on the likelihood of patients needing PVR at a younger age. This, in turn, could result in a reduced duration of freedom from valve failure. Replacing the pulmonary valve too early can effectively increase the lifetime number of procedures, preserving the function of the tricuspid valve at the time of TOF repair is of paramount importance.

Responsive Neurostimulation in Pediatric and Young Adult Patients With Drug-Resistant Focal, Multifocal, and Generalized Epilepsy: A Single-Center Experience

BackgroundResponsive neurostimulation (RNS) is used off-label in pediatric patients with drug-resistant epilepsy (DRE). Our study aims to assess the safety and efficacy of RNS in pediatric and young adult patients with focal, multifocal, and generalized DRE. MethodsAll patients who underwent RNS implantation at Primary Children's Hospital in Salt Lake City, UT, between December 2017 and 2022. ResultsA total of 47 patients were retrospectively identified, of which 32 patients were included in the final analysis. Patients ranged in age from five to 21 years (pediatric n = 22, young adult n = 10) at the time of RNS implantation with focal (20 [63%]), multifocal (8 [25%]), and generalized (4 [12%]) DRE. Operative complications (3 [9%]) and negative side effects (6 [19%]) were minor. At the time of most recent clinic visit (mean 18.6 months, S.D. 13.9), 19 of 32 patients (59%) were responders with ≥50% reduction in seizure frequency (pediatric n = 14, young adult n = 5). The rate of responders increased with prolonged activation of RNS stimulation, reaching 71% (five of seven patients) after 24 months. Antiseizure medication was reduced in five (16%) patients, and seizure rescue medication usage was reduced in 10 (31%) patients. Quality of life improved in 15 (47%) patients. ConclusionsRNS implantation resulted in a sustained reduction in seizure frequency with minimal side effects in a majority of patients. Taken together, our data suggest that RNS is an effective and safe treatment option for focal, multifocal, and potentially generalized DRE in the pediatric and young adult population.

7298 Hyperventilation Associated Hypophosphatemia, Hypocalcemia, Hypokalemia And Paralysis

Abstract Disclosure: S. Afreen: Research Investigator; Self; Tandem Diabetes Care, Yes. N. Afreen: None. Introduction: Hyperventilation is over-breathing in excess of the metabolic needs of the body, which can result in respiratory alkalosis and a wide range of electrolyte abnormalities. Case: A 38 year old male with a past history of hypertension, OSA and cholecystectomy reported having episodes of palpitations, tachypnea with respiratory rate as high as 80 breaths/ minute, associated with facial numbness and tingling, tetany, progressing to paralysis of his extremities. These episodes started 7 years ago but gradually became more frequent. He denied anxiety or stressors prior to these episodes. The episodes almost always result in emergency room visits. During one of these episodes he was found to have the following labs: ionized calcium-1 mmol/l, serum phosphorus- 0.8 mg/dl, serum potassium- 2.7 mmol/l, 1.25(OH)2D- 44pg/ml, alkaline phosphatase- 60 IU/l, glucose- 94 mg/dl and GFR- 90 ml/min/1.73m2. VBG during this episode: pH - 7.59, PCO2- 23 mm Hg, and bicarbonate - 22 mmol/l. Of note, the electrolytes are normal in between the episodes. Thyroid labs are normal. Off of phosphate supplementation for 3 days, in between episodes, his serum phos- 4.2mg/dl, cr - 0.84 mg/dl. Second morning urine phos - 84 mg/dl and urine cr - 206.81 mg/dl. Tubular phosphate reabsorption was 91.9%. He had negative Holter, nuclear stress testing and MRI of the brain. No mutations were identified on genetic analysis of CACNA15, KCNJ2, RYR1, SCN4A. During his recent endocrine clinic visit, on physical exam: height- 5 feet 11 inches, weight- 366 lbs weight, BMI- 51.1 kg/m2, BP- 129/89 mm Hg, heart rate- 81 bpm and respiratory rate- 18 breaths/minute. He did have one palpable 1.5 cm left thyroid nodule. Otherwise, the exam was unremarkable. The patient was referred to pulmonology to be evaluated for potential diaphragmatic and lung causes of his tachypnea. He was also referred to psychiatry for evaluation of underlying anxiety. Since these episodes of hyperventilation associated electrolyte abnormalities lead to paralysis, he was started on calcitriol 0.25mcg daily along with 1250 mg potassium phosphate daily. Discussion: Hyperventilation can lead to a respiratory alkalosis and corresponding increase in intracellular pH. High intracellular pH can lead to increased phosphofructokinase activity and glycolysis, resulting in increased intracellular phosphate shift. Acute respiratory alkalosis can also result in ionized calcium binding to albumin. Moreover, acute respiratory alkalosis results in reduction in hydrogen ion in the intracellular fluid with extracellular potassium ion shift into cells leading to hypokalemia.These electrolyte abnormalities can cause myopathies, cardiac arrhythmias, respiratory failure, paralysis, seizures, coma and death. Physicians should be aware of the possibility of electrolyte imbalance due to hyperventilation to prevent the development of life threatening complications. Presentation: 6/1/2024

Implementing a letter template to expedite specialty medication appeal letter submission

BackgroundPrior authorizations (PAs) for biologic medications, used to treat inflammatory bowel disease (IBD), are often denied by pharmacy benefits managers and can require a complex appeal process for patients to gain access to medication. ObjectiveThis quality improvement project evaluated the impact of implementing a standardized appeal letter template and customizable clinical rationale letter content on specialty pharmacist workflow and workload in an integrated hospital health system specialty pharmacy (HSSP) IBD clinic. Practice descriptionThis initiative was conducted in an IBD outpatient clinic at a tertiary academic medical center with an integrated HSSP whose specialty pharmacists work collaboratively with providers to manage specialty medications. Practice innovationA letter template was created in the electronic health record (EHR) for pharmacists to use when submitting appeal letters. The template automatically populates patient results from recent labs, imaging, and clinic visit notes as part of the appeal documentation. Clinical rationale letter content was developed for the most common appeal reasons using EHR functionality that allows the creation of standardized notes that can be shared among team members and customized at time of use. Evaluation methodAn analysis of 2 months of data preimplementation/postimplementation was conducted using descriptive statistics to report the number of appeals submitted, time from PA denial to appeal submission, and appeal approval rate. A pharmacist postimplementation satisfaction score was also collected. ResultsThe number of appeals submitted preimplementation (n = 73) and postimplementation (n = 73) was the same. Postimplementation, 89% of appeals were submitted within 3 days of PA denial compared to 29% preimplementation. PA approval rate was high (93%) preimplementation and postimplementation. Overall pharmacist satisfaction was 9.7 out of 10. ConclusionImplementation of an appeal letter template and standardized clinical rationale letter content in the EHR led to decreased time to appeal submission and high specialty pharmacist satisfaction.

Subclinical atherosclerosis burden in carotid and femoral territories in HIV subjects: relationships with HIV and non-HIV related factors

BackgroundCardiovascular disease is a major cause of morbidity in an aging HIV population. However, risk estimation with the most frequent equations usually classifies HIV patients as having a low or moderate risk. Several studies have described a very high prevalence of subclinical atherosclerosis in a middle-aged, non-HIV population. There is insufficient body of knowledge to understand if this is the case in people living with HIV (PLWH). We aim to calculate the proportion of patients with subclinical atherosclerosis in a single site cohort of HIV-infected subjects.MethodsWe have analyzed chronically HIV infected adults (≥ 18 years) who were on active follow-up in an HIV unit specialized in the care of cardiovascular health. The most recent clinical visit and vascular ultrasonography were used to assess the objectives of our research. Our primary objective was to describe the proportion of participants with subclinical atherosclerosis (focal protrusion into the lumen > 0.5 mm or > 50% of the surrounding IMT or a diffuse thickness > 1.5 mm) in a single site cohort of PLWH. Carotid and iliofemoral territories were evaluated. As a secondary objective we have run a multivariate analysis to determine which HIV and non-HIV factors might be related with the presence of atherosclerotic plaques.FindingsWe included a total of 463 participants between November 2017 to October 2019. Subjects were predominantly male (84.2%) with a mean age of 48.8 years (SD 10.7). Hypercholesterolemia (36%) was the most prevalent comorbidity followed by Hypertension (18%) and Hypertriglyceridemia (16%). Mean duration of HIV infection is 12.3 years. Overall, participants had been receiving cART for a median of 9.5 years. Subclinical atherosclerosis was found in 197 subjects (42.5%; CI 95% [38.0–47.2]). The disease was found more frequently in the femoral arteries (37.8%) than in the carotid vascular bed (18.6%). Despite some HIV factors correlated with the presence of plaques in a univariate analysis (e.g., time with HIV-1 RNA > 50 copies/mL or time from HIV diagnosis), the only two explanatory factors that remained associated with the presence of atherosclerotic plaques in the multivariate analysis were smoking (OR 5.47, 95% CI 3.36 – 8.90) and age (OR 1.13, 95%CI 1.10 – 1.16).InterpretationWe have found a very high prevalence of subclinical atherosclerosis among our cohort of PLWH. Despite having analyzed several HIV factors, age and smoking have been found to be the only factors associated with the development of atherosclerotic plaques.

Transcranial magnetic stimulation and magnetoencephalography are feasible alternatives to invasive methods in optimizing responsive neurostimulation device placement

Responsive neurostimulation (RNS) is a treatment option for patients with refractory epilepsy when surgical resection is not possible due to overlap of the irritative zone and eloquent cortex. Presurgical evaluations for RNS placement typically rely on invasive methods. This study investigated the potential of transcranial magnetic stimulation (TMS) and magnetoencephalography (MEG) to provide key presurgical information non-invasively. We hypothesized that these non-invasive methods may assist in optimizing RNS placement by providing useful information for seizure localization by MEG and eloquent cortex mapping by TMS. A retrospective chart review identified nine patients who underwent RNS placement (mean age = 20.4 years [SD = 5.6], two-thirds were female). Characterization of the irritative zone using MEG was successful in eight of nine patients. Non-invasive mapping of relevant eloquent cortex was attempted in all patients. TMS was successful in eight of nine patients, and MEG was successful in two of six patients. Importantly, patients mapped with non-invasive modalities experienced an average seizure reduction of 77 % at their most recent clinic visit, compared to 75 % seizure reduction in those with invasive evaluations, indicating appropriate RNS placement. These data demonstrate that TMS and MEG can provide key information for RNS and may be feasible alternatives to invasive methods for assisting in decision making regarding RNS placement. Non-invasive methods for determining RNS placement have a high rate of success when data from multiple non-invasive modalities converge and can inform more accurate placement of intracranial electrodes prior to RNS placement or mitigate their need.

Bone Transport for Large Segmental Tibial Defects Using Taylor Spatial Frame versus the Ilizarov Circular Fixator.

Bone transport has become the gold standard for treating large segmental tibial bone defects. The technique for application the Ilizarov circular fixator (ICF) has a long learning curve and is associated with many complications. There are few clinical studies on bone transport via the Taylor spatial frame (TSF). The main purpose of this study was to compare the radiological and clinical and outcomes of bone transport by using the TSF and the ICF. There were 62 patients included in this retrospective study from June 2011 to June 2021 and distributed to two groups according to the fixation method: a TSF group consisting of 30 patients and an ICF group consisting of 32 patients. Demographic information, surgical duration, external fixation times, external fixation index, final radiographic results, complications, and clinical outcomes were recorded and examined. The clinical outcomes were assessed using the ASAMI criteria during the most recent clinical visit. Then, statistical analysis such as independent-samples t tests or chi-Square test was performed. The mean surgical duration in the TSF group was 93.8 ± 7.3 min, which was shorter than that in the ICF group (109.8 ± 1.4 min) (p < 0.05). Compared to the ICF group (10.2 ± 2.0 months), the TSF group (9.7 ± 1.8 months) had a shorter average external fixation time (p > 0.05). The external fixation index was 1.4 ± 0.2 m/cm and 1.5 ± 0.1 m/cm in the two groups. Moreover, there was no significant difference between the two groups. At the last follow-up visit, the medial proximal tibial angle (MPTA) and posterior proximal tibial angle (PPTA) in the TSF group were 88.1 ± 12.1° and 80.9 ± 1.3°, respectively. The MPTA and PPTA in the ICF group were 84.4 ± 2.4° and 76.2 ± 1.9°, respectively. There were statistically significant differences between the two groups (all p < 0.05). The complication rate was 50% in the TSF group and 75% in the ICF group. Moreover, the ASAMI score between the two groups was no statistically significant difference (p > 0.05). No statistically significant difference was found in clinical outcomes between the use of Taylor spatial frame and Ilizarov circular fixator for treating large segmental tibial bone defects. However, TSF is a shorter and simpler procedure that causes fewer complications and improves limb alignment.

Impact of a Hospital-Based Food Pharmacy Program on Health Outcomes of Vulnerable Patients.

Access to healthy foods, especially for those who are living with diabetes and hypertension, is crucial in managing these chronic diseases. This study evaluates the implementation of a food pharmacy and food prescription program at a safety-net hospital that serves vulnerable populations. Patients who screen as food insecure using the USDA adult food security survey receive a referral from the physician to the food pharmacy program where a dietician reviews their dietary requirements based on their chronic disease and develops a diet plan. Patients then receive fresh produce, meats and other products every 2weeks that meets their nutritional needs from the food pharmacy. Biometric data from the patient's most recent clinic visit at the time of enrollment was collected as the baseline measures including blood pressure, weight, and HbA1c (if diabetic). Additionally, biometric information was collected from the patient's medical records from regularly scheduled clinic visits at 6month intervals. A total of 266 patients were enrolled in the program during the 13-month period that was studied (121 patients with 6-month data and 68 patients with 12-month data). The statistical analysis showed a significant improvement in diastolic blood pressure at 12months and in weight at both the 6months and 12months timeframes when comparing to baseline biometrics.

Psychotropic Medication Prescription Patterns in Down Syndrome in a Large Pediatric Specialty Clinic.

Objectives: Patterns of psychotropic medication use in children and adolescents with Down syndrome (DS) are largely unknown. Clinical decisions are often made from evidence and experience from individuals with autism spectrum disorder (ASD) or intellectual disability (ID). Methods: Longitudinal data from 670 children with DS who received care in a specialty DS clinic from March 2021 to February 2024 were collected. After each clinic visit, the clinician indicated the presence or absence of co-occurring neurodevelopmental (ND) or mental health (MH) diagnoses, as well as whether the individual was prescribed a psychopharmacological treatment. We used descriptive statistics and analyzed associations between psychotropic medication use, co-occurring ND/MH conditions, and demographic data. Results: 19.1% of patients were prescribed at least one psychotropic medication at their most recent clinical visit. Alpha-agonists were the most commonly prescribed medication class (30.8%), followed by stimulants (18.9%), and antidepressants (16.7%). There was a significant difference in psychotropic medication use by age, with older children having increased odds of being prescribed a psychotropic medication. There were no differences in psychotropic medication use across sex (p = 0.10), race (p = 0.10), or household income (p = 0.16). Conclusions: We found that one-fifth of patients with DS were prescribed psychotropic medications. Nearly every individual with DS who was prescribed a psychotropic medication had a co-occurring ND/MH condition, yet these rates were lower than what have been reported in children with ID, ASD, and attention deficit/hyperactivity disorder. Further research needs to include those with DS to further understand medication efficacy and safe dosing practices to ensure optimal outcomes.

GLP-1 Receptor Agonists Promote Weight Loss Among People with HIV.

Weight gain and associated metabolic complications are increasingly prevalent among people with HIV (PWH). Glucagon-like peptide-1 receptor agonists (GLP-1RAs) are incretin-based therapies for diabetes and weight management that have been shown to result in substantial weight loss; however, studies of their effects in PWH are limited. A retrospective single-center cohort study was conducted among PWH who were taking GLP-1RAs at UC San Diego Owen Clinic between 2/1/2021 to 2/1/2023. Baseline clinical data were collected and changes in weight, body mass index (BMI), and hemoglobin A1C (A1C) before starting GLP-1RAs compared to the most recent clinic visit were calculated (with a minimum of 3 months follow-up time required). Logistic regression was performed to identify variables associated with >5% of total body weight loss. A total of 225 patients received on average 13 months of GLP-1RA therapy, with 85 (37.8%) achieving the maximum GLP-1RA dose. GLP-1RA therapy resulted, on average, in a loss of 5.4 kg, decrease in BMI by 1.8 kg/m2, and decrease in A1C by 0.6%. In the multivariable analysis, higher baseline BMI [OR 1.10 (1.03-1.16)], treatment duration of GLP-1RA therapy greater than 6 months [OR 3.12 (1.49-6.49], and use of tirzepatide [OR 5.46 (1.44-20.76)] were significantly more likely to be associated with >5% weight loss. Use of GLP-1RAs led to declines in weight, BMI, and hemoglobin A1C among PWH and offers an additional strategy to address weight gain and diabetes.

The Association of Race, Ethnicity, and Insurance Status with the Visual Acuity of Retinoblastoma Survivors in the IRIS® Registry

ABSTRACT Purpose To identify sociodemographic factors associated with the visual outcomes of retinoblastoma survivors. Methods Retrospective cohort study using a US-based clinical data registry. All individuals < 18 years of age with a history of retinoblastoma in the Intelligent Research in Sight (IRIS®) Registry (1/1/2013–12/31/2020). The primary outcome was visual acuity below the threshold for legal blindness (20/200 or worse) in at least one eye. Multivariable logistic regression was used to evaluate the association between visual outcomes and age, sex, laterality, race, ethnicity, type of insurance, and geographic location. Results This analysis included 1545 children with a history of retinoblastoma. The median length of follow-up was 4.1 years (IQR, 2.2–5.9 years) and the median age at most recent clinical visit was 12 years (IQR, 8–16 years). Retinoblastoma was unilateral in 54% of cases. Poor vision in at least one eye was identified in 78% of all children and poor vision in both eyes in 17% of those with bilateral disease. Poor visual outcomes were associated with unilateral diagnosis (OR, 1.55; 95% CI,1.13–2.12; p = .007), Black race (OR, 2.03; 95% CI, 1.19–3.47; p = .010), Hispanic ethnicity (OR, 1.65; 95% CI, 1.16–2.37; p = .006), and non-private insurance (OR, 1.47; 95% CI, 1.02–2.10; p = .037). Conclusions Poor visual outcomes appear to be more common among Black, Hispanic, and publicly insured children with a history of retinoblastoma, raising concerns regarding healthcare inequities. Primary care physicians should ensure that young children receive red reflex testing during routine visits and consider retinoblastoma in the differential diagnosis of abnormal eye exams.

Two-Year Outcomes After Total Ankle Replacement With a Novel Fixed-Bearing Implant By a Single Surgeon Non-Inventor

Total ankle arthroplasty continues to gain popularity amongst surgeons and patients as an alternative to arthrodesis. Historically the designs of early implants were plagued with complications and frequently abandoned. Since that time the procedure and materials have undergone significant advancements in both surgical approach as well as design and function of the available implants. In this study, 40 consecutive patients who received a semiconstrained prosthesis with a unique fixed-bearing polyethylene insert were identified. Minimum follow-up was 2 y. Demographic, social, and past medical data was retrospectively reviewed. Concomitant procedures were also recorded. Radiographic analysis included lateral ankle radiograph postoperative range of motion (ROM) with maximum dorsiflexion and maximum plantarflexion weight bearing at the most recent clinic visit. Clinical outcomes included VAS, FFI, and AOFAS scores. Lateral radiographs taken in the office at a minimum 2-y follow-up showed mean maximum dorsiflexion of 11.8 degrees and plantarflexion of 13.9 degrees. VAS, FFI, and AOFAS scores improved from 8.1, 92.9, and 44.8 to 1.4, 15.3, and 90.1 postoperatively, respectively. A total of 2.5% (n = 1) required revision surgery for poly failure, and 5.0% (n = 2) underwent local wound care in the office setting postoperatively and healed without complications. Overall survivorship was 97.5% at the minimum follow-up of 2 y. In conclusion, similar studies have reported survivorship from 90% to 100% with modern ankle implants in short to mid-term follow-up. Although this is a small sample size, our data shows a 97.5% survivorship at 2 y postoperatively with favorable patient-reported statistically significant functional outcome scores, and ankle range of motion consistent with existing literature.

Outcomes of a newly established transitional urology outpatient clinic: a real-world experience.

To evaluate the demographic and clinical characteristics of patients who visited our transitional urology (TU) outpatient clinic formed by pediatric urologists with urology background within the first year upon its establishment. Files of 130 consecutive patients who visited our TU outpatient clinic, which was established in 01 March 2021, between 01 March 2021 and 01 March 2022 were retrospectively collected. Patients were divided into two groups: those with a previous follow-up in our pediatric urology department (Group I, n: 81, 62.3%) and those who were followed up in other clinics during childhood (Group II, n: 49, 37.7%) afterwards. Demographic characteristics, complaints at admission, previous medical history, and management plans at the recent clinical visit were noted. We defined a successful and smooth transition from childhood to adult care as not being without follow-up within the first year after the age of 18years and not requiring extraordinary medical assistance (e.g., emergency room visits, hospitalization, intensive care unit admissions) from the last urological control to the TU outpatient clinic visits. The most common diagnoses were vesicoureteral reflux (n: 32, 24.6%), neuropathic bladder accompanied by spina bifida (n: 31, 23.8%), obstructive uropathy (n: 25, 19.2%), hypospadias (n: 20, 15.3%), non-neurogenic lower urinary tract dysfunction (n: 19, 14.6%), and bladder exstrophy (n: 8, 6.1%). The distribution of primary diagnosis in the two patient groups was similar. The median time from the last pediatric urology visit to the current TU clinical visit was significantly longer in Group II (12 vs. 60months, p < 0.001),consequently, the median patient age at admission was significantly higher in Group II (21 vs. 23years, p = 0.020). The rate of a successful and smooth transition was 86.4% in Group I, whereas Group II had a completely unsuccessful transition period. Upon admission to TU outpatient clinic, the requirement of a surgical intervention was lower in Group I (21% vs. 38.8%, p = 0.028).Also,the need for medical treatment changes was higher in Group II (9.9% vs. 53.1%, p < 0.001). Our findings emphasize the importance of patient referral to a TU clinic that deal with lifelong problems of congenital genitourinary diseases. Delays in receiving medical or surgical treatments during transition from childhood to adulthood may be associated with higher need for subsequent surgical interventions in this vulnerable patient population.

Angiotensin II type 1 receptor antibodies and native kidney function in pediatric liver and intestinal transplant recipients.

Angiotensin II type-1 receptor antibody (AT1R-Ab) has been associated with vascular injury and kidney dysfunction in pediatric kidney transplant recipients. The role of AT1R-Ab in the development of chronic kidney disease in pediatric liver and intestinal transplant recipients has not been explored. Twenty-five pediatric intestinal transplant recipients and 79 pediatric liver transplant recipients had AT1R-Ab levels measured at varying time points in the post-transplant period. Estimated glomerular filtration rate (eGFR) was determined using creatinine based CKiD U25 equation and measured at time of AT1R-Ab measurement, at 1year post-AT1R-Ab measurement, at 5years post-AT1R-Ab measurement, and at the most recent routine clinic visit. The prevalence of hypertension and antihypertensive medication use were also evaluated. Younger age at time of AT1R-Ab measurement was associated with AT1R-Ab positivity in liver transplant recipients. There was no association between AT1R-Ab status and change in eGFR, prevalence of hypertension, or use of antihypertensive medications at the described time points. AT1R-Ab positivity was not associated with a decline in eGFR or hypertension in pediatric liver and intestinal transplant recipients. Further studies are needed using other markers of kidney function, such as cystatin C, to validate this finding. A higher resolution version of the Graphical abstract is available as Supplementary information.

Obesity Risk of Pediatric Central Nervous System Tumor Survivors: A Cross-Sectional Study.

Adult survivors of pediatric central nervous system (CNS) tumors are at the highest risk for morbidity and late mortality among all childhood cancers due to a high burden of chronic conditions, and environmental and lifestyle factors. This study aims to epidemiologically characterize young adult survivors of pediatric CNS tumors using body mass index (BMI) to assess risk factors for obesity. Using a cross-sectional design, young adults (18-39 years) previously treated for pediatric CNS tumors and followed in a survivorship clinic during 2016-2021 were examined. Demographic, BMI, and diagnosis information were extracted from medical records of the most recent clinic visit. Data were assessed using a two-sample t-test, Fisher's exact test, and multivariable logistical regression. 198 survivors (53% female, 84.3% White) with a BMI status of underweight (4.0%), healthy weight (40.9%), overweight (26.8%), obesity (20.2%), and severe obesity (8.1%) were examined. Male sex (OR, 2.414; 95% CI, 1.321 to 4.414), older age at follow-up (OR, 1.103; 95% CI, 1.037 to 1.173), and craniopharyngioma diagnosis (OR, 5.764; 95% CI, 1.197 to 27.751) were identified as significant (p < 0.05) obesity-related (≥25.0 kg/m2) risk factors. The majority of patients were overweight or obese. As such, universal screening efforts with more precise determinants of body composition than BMI, risk stratification, and targeted lifestyle interventions are warranted during survivorship care.

Lichen Sclerosus Throughout Childhood and Adolescence: Not Only a Premenarchal Disease

ABSTRACT Lichen sclerosus (LS) is a chronic dermatologic condition most commonly seen in prepubertal girls or postmenopausal women that causes inflammation and epidermal thinning in the anogenital region. Lichen sclerosus is suspected to have a genetic component due to a strong association with family history and is associated with various autoimmune diseases. Treatment involves medium- and high-potency topical corticosteroids, as well as calcineurin inhibitors; however, the optimal treatment regimen is unknown. LS follows a chronic and recurring pattern that causes significant discomfort and impaired quality of life over time; however, little data exist on the disease course in adolescent patients. This retrospective cohort study aimed to characterize and compare symptoms and physical examination findings of a cohort of pediatric, adolescent, and young adult females with vulvar LS. Patients 21 years or younger presenting to the University of Michigan between January 2006 and September 2020 with vulvar LS were included. Demographic data, personal and family history of autoimmune disease, specialty of provider, age of onset, age at menarche, presenting symptoms, and physical examination findings were abstracted from the chart. Patients were grouped for analysis based on symptom onset, first clinical visit, and the most recent clinical visit. Group 1 was patients who were premenarchal at their first clinic visit, and group 1a included those within group 1 who were postmenarchal at the most recent clinical visit. Group 2 included patients who were postmenarchal at the first clinic visit, and group 2a were those within group 2 who reported first onset of symptoms postmenarche. A total of 196 patients met inclusion criteria, with 141 in group 1, 26 in group 1a, 55 in group 2, and 36 in group 2a. The average age in group 1 was 7.02 years and in group 2 was 19.08 years. Patients in group 2 were more likely to have a personal history of autoimmune disease compared with group 1 (P = 0.04). Of the 26 patients in group 1a, 10 (38.5%; 95% CI, 19.8–57.2) continued to have symptoms of LS after undergoing menarche. Compared with patients with symptom onset postmenarche (group 2a), the premenarchal cohort (group 1) was significantly more likely to present with vulvar itching (70.2% vs 52.8%), vulvar bleeding (26.2% vs 5.6%), and bowel symptoms (16.3% vs 0%). The premenarchal cohort (group 1) was significantly more likely than the postmenarchal cohort (group 2a) to have subepithelial hemorrhages (24.8% vs 5.6%), whereas the postmenarchal group (group 2a) was significantly more likely to have architectural changes of the vulva including clitoral adhesions (25.0% vs 4.3%) and loss of labia minora (47.2% vs 2.1%). Of the 55 postmenarchal patients (group 2), 39 had at least 1 architectural change of the vulva, and among the 36 with first onset of symptoms postmenarche (group 2a), 20 (55.6%) had at least 1 vulvar skin change. This study of the largest cohort to date of young patients with vulvar LS demonstrates approximately 40% of patients with LS that went through puberty continued to have symptoms, and that symptoms and examination findings were different and often more severe among those who developed symptoms postmenarche versus premenarche.

Adherence to lipid-lowering therapy and reaching treatment goals in youth seen in a preventive cardiology clinic.

The efficacy of lipid-lowering therapy in reducing cardiovascular disease in adults is well-established. Unfortunately, it is also well-established that adults have inadequate adherence to lipid-lowering therapy, which is associated with increased costs and mortality. However, the adherence patterns of youth prescribed lipid-lowering therapy is not well-described. We analyzed data that was prospectively collected from patients <27 years-old who were referred to a large regional preventive cardiology clinic from 2010 to 2017. Adherence to lipid-lowering therapy was self-reported at the patient's most recent clinic visit and categorized as either adequate adherence (≥80%) or inadequate adherence (<80%). We compared adherence rates by demographic factors, class of lipid-lowering therapy, length of time on lipid-lowering therapy, family history, lipid parameters, and laboratory measures of adverse effects. In our cohort, we had 318 patients prescribed a lipid-lowering medication over a seven-year period. Of those, 235 (75%) had adequate adherence. Those with adequate adherence had an improved LDL-C (123mg/dL [standard deviation (SD) 32.3] vs. 167mg/dL [SD 50.4], p<0.05), total cholesterol (198mg/dL [49.5] vs. 239mg/dL [SD 53.2]), and non-HDL-C (148mg/dL [SD 38.7] vs. 193mg/dL [SD 43.9]). In addition, patients with adequate adherence were more likely to reach goal LDL-C of <130mg/dL than those with inadequate adherence (130 vs. 25, p<0.01). The relationship between LDL-C and adherence remained statistically significant after controlling for age, gender, and the length of time on therapy (β=-0.66, p<0.01). Adherence level did not differ by gender, class of lipid-lowering therapy, length of time on lipid-lowering therapy, or presence of a family history of an atherosclerotic event. The findings were similar when we only analyzed those prescribed a statin. Self-reported adherence to lipid-lowering therapy in youth is excellent and was associated with achieving goal LDL-C goals. Obtaining adherence data from patients may help more patients reach LDL-C goals.

Abstract P1-12-05: The Impact of Adjuvant Chemotherapy, Including Taxanes, on Breast Cancer-Related Lymphedema Risk

Abstract BACKGROUND: Adjuvant chemotherapy, particularly taxane-based chemotherapies, which are used to treat high-risk breast cancers, has been shown to be efficacious in prolonging overall survival. However, the impact of such therapies on risk of breast cancer-related lymphedema (BCRL) is yet to be fully understood. Available studies have contradictory findings as to whether or not taxanes increase BCRL risk. Unfortunately, methodological flaws, such as not incorporating a preoperative baseline arm volume measurement or utilizing flawed measurement techniques and diagnostic criteria, result in misdiagnosis of the primary outcome of BCRL and it is therefore difficult to draw conclusions from these studies. As such, a more comprehensive understanding of the potential risks associated with these treatment modalities can improve quality of care as patients are better prepared to face a potentially devastating sequela of breast cancer treatment. PURPOSE: The purpose of this study is to determine if chemotherapy administered in the adjuvant setting is an independent risk factor of BCRL, as well as to assess any specific risks of taxane-based chemotherapy on BCRL. METHODS: From 2005 to 2021, 2049 patients treated for breast cancer were enrolled in a prospective BCRL screening trial and screened from preoperative baseline through last follow-up. Screening included objective arm volume measurements via perometry. Chemotherapy data and clinicopathological and demographic characteristics were collected directly from the electronic medical record. Patients who had not received neoadjuvant chemotherapy were eligible for the current study; they were classified based on whether they had received taxane-based chemotherapy, non-taxane chemotherapy only, or no chemotherapy. Relative volume change (RVC) increase ≥10% from preoperative baseline &amp;gt;3 months postoperatively was used to define BCRL. Patients were censored at cancer recurrence or most recent clinic visit. We fit a Cox regression model to estimate adjusted hazard ratios (aHR) for BCRL. The model was adjusted for baseline BMI, axillary lymph node dissection (ALND), regional lymph node radiation (RLNR), age at diagnosis, and smoking history. RESULTS: The eligible cohort included 1759 patients, including 564 (32%) who received taxane-based chemotherapy and 104 (6%) who received non-taxane chemotherapy only. The median follow-up time for the entire cohort was 58 months. 141/1759 (8%) of patients developed BCRL. The median time to develop BCRL was 21 months post-operatively. Compared to no chemotherapy, the aHR associated with taxane-based chemotherapy was 1.04 (95% CI 0.37, 1.8; p = 0.62). Compared to no chemotherapy, the aHR for non-taxane chemotherapy was 0.82 (95% CI 0.67, 1.6; p = 0.86). There was no significant difference between taxane and non-taxane therapies in terms of BCRL risk (p = 0.55). ALND, RLNR, and high BMI remained independent risk factors for BCRL, consistent with the published literature. CONCLUSION: The receipt of adjuvant chemotherapy and specifically adjuvant taxane-based chemotherapy were not associated with increased risk of BCRL in this cohort of 1759 patients at risk of and prospectively screened for BCRL. Citation Format: Amanda W. Jung, Brooke Juhel, Louisa H. Smith, Cheryl L. Brunelle, Elizabeth K. Hausman, Loryn K. Bucci, George E. Naoum, Alphonse G. Taghian. The Impact of Adjuvant Chemotherapy, Including Taxanes, on Breast Cancer-Related Lymphedema Risk [abstract]. In: Proceedings of the 2022 San Antonio Breast Cancer Symposium; 2022 Dec 6-10; San Antonio, TX. Philadelphia (PA): AACR; Cancer Res 2023;83(5 Suppl):Abstract nr P1-12-05.

Evaluation of treatment simplification strategies in patients living with HIV with multi-drug resistance.

Patients living with HIV (PLWH) with multi-drug resistance (MDR) and prior episodes of virologic failure have few therapeutic options remaining. These patients are often prescribed 'salvage' antiretroviral therapy (ART) regimens with high pill burdens, leading to potential decreased medication adherence and increased side effects and drug-drug interactions. In this retrospective, observational cohort study, we included adult patients with a diagnosis of HIV-1 who received care at our institution's Ryan White Clinic and who received 'salvage' ART, defined as three of more antiretroviral agents from at least three different HIV drug classes. Patients were grouped into two cohorts, simplified ART cohort and non-simplified ART cohort, based on whether their ART regimen was reduced by at least one tablet daily. The primary outcome was the percentage of patients who had their viral load suppressed (HIV-1 RNA <50 copies/ml) at their most recent clinic visit. Secondary outcomes were virologic failure (HIV-1 RNA ⩾200 copies/ml), percentage of time patients were virologically suppressed over the past 2 years, and the emergence of new treatment-resistant mutations. There were 50 patients included in the final analysis, 28 in the simplified ART cohort and 22 in the non-simplified ART cohort. The percentage of patients who had their HIV-1 viral load suppressed at their most recent clinic visit was n = 24 (86%) in the simplified ART cohort and n = 16 (73%) in the non-simplified ART cohort (p = 0.302). There were no statistically significant differences between the two cohorts in terms of the secondary outcomes. Our study found that simplification of ART regimens based on HIV genotype in PLWH with a history of MDR and prior virologic failures, regardless of the presence of HIV-1 viremia at the time of simplification, resulted in similar rates of virologic suppression and virologic failure as non-simplified ART regimens.

DISP-16. NUTRITIONAL SCREENING OF PEDIATRIC CENTRAL NERVOUS SYSTEM TUMOR SURVIVORS REVEALS HIGH PREVALENCE OF MALNOURISHMENT WITH SOCIODEMOGRAPHIC AND CLINICAL RISK FACTORS

Abstract Adult survivors of pediatric central nervous system (CNS) tumors are at the highest risk for morbidity and late mortality among all childhood cancers, partially due to chronic health conditions intersected with environmental and lifestyle factors. We aimed to study the prevalence of and risk factors for malnourishment (underweight, overweight, obese) amongst adult survivors of pediatric CNS tumors. We conducted a cross-sectional study assessing young adults (18-39 years) previously treated for pediatric CNS tumors who were followed in our survivorship clinic during 2016-2021. Sociodemographic, clinical, and treatment information was extracted from medical records with BMI status, per CDC guidelines, obtained from the most recent clinic visit. Data was assessed using one-way ANOVA, fishers exact test, and multivariable logistical regression. We identified 198 survivors (53% female, 84.3% white) categorized as underweight (4.0%), normal weight (40.9%), overweight (26.8%), obese (20.2%), and severely obese (8.1%). Patients were median age 8 years [4-12] at diagnosis and 24 years [20-28] at follow-up. Most common primary diagnosis and tumor location was low grade glioma (50.5%) and posterior fossa (39.4%) with 90.4% receiving surgery, 51.5% chemotherapy, and 52.5% radiotherapy. Male sex (OR 2.376, p = 0.006), hypothalamic/optic pathway tumor location (OR 2.362, P = 0.031), and radiation therapy (OR 2.844, p = 0.005) were associated with increased overweight/obesity risk, while chemotherapy (OR 0.363, p = 0.006) was associated with decreased risk. Male sex (OR 0.511, p = 0.007) and white ‘race’ (OR 0.166, p = 0.022) were associated with decreased underweight risk. The majority of young adult survivors seen in our clinic were not healthy weight by BMI, and the distribution of BMI groups appears comparable to contemporary studies of the general young adult U.S. population. The prevalence of malnourishment amongst PNO survivors warrants universal screening efforts with more accurate determinants of body composition than BMI, risk stratification, as well as targeted interventions.