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1.
Pulm Circ ; 14(3): e12402, 2024 Jul.
Article in English | MEDLINE | ID: mdl-38962181

ABSTRACT

Children with pulmonary hypertension (PH) often demonstrate limited exercise capacity. Data support exercise as an effective nonpharmacologic intervention among adults with PH. However, data on exercise training in children and adolescents are limited, and characteristics of the optimal exercise program in pediatric PH have not been identified. Exercise programs may have multiple targets, including muscle deficits which are associated with exercise limitations in both adult and pediatric PH. Wearable accelerometer sensors measure physical activity volume and intensity in the naturalistic setting and can facilitate near continuous data transfer and bidirectional communication between patients and the study team when paired with informatics tools during exercise interventions. To address the knowledge gaps in exercise training in pediatric PH, we designed a prospective, single arm, nonrandomized pilot study to determine feasibility and preliminary estimates of efficacy of a 16-week home exercise intervention, targeting lower extremity muscle mass and enriched by wearable mobile health technology. The exercIse Training in pulmONary hypertEnsion (iTONE) trial includes (1) semistructured exercise prescriptions tailored to the participant's baseline level of activity and access to resources; (2) interval goal setting fostering self-efficacy; (3) real time monitoring of activity via wearable devices; (4) a digital platform enabling communication and feedback between participant and study team; (5) multiple avenues to assess participant safety. This pilot intervention will provide information on the digital infrastructure needed to conduct home-based exercise interventions in PH and will generate important preliminary data on the effect of exercise interventions in youth with chronic cardiorespiratory conditions to power larger studies in the future.

2.
Muscle Nerve ; 69(5): 613-619, 2024 May.
Article in English | MEDLINE | ID: mdl-38515223

ABSTRACT

INTRODUCTION/AIMS: Traditional exercise is often difficult for individuals with Friedreich ataxia (FRDA), and evidence is limited regarding how to measure exercise performance in this population. We evaluated the feasibility, reliability, and natural history of adaptive cardiopulmonary exercise test (CPET) performance in children and adults with FRDA. METHODS: Participants underwent CPET on either an arm cycle ergometer (ACE) or recumbent leg cycle ergometer (RLCE) at up to four visits (baseline, 2 weeks, 4 weeks, and 1 year). Maximum work, oxygen consumption (peak VO2), oxygen (O2) pulse, and anaerobic threshold (AT) were measured in those who reached maximal volition. Test-retest reliability was assessed with intraclass coefficients, and longitudinal change was assessed using regression analysis. RESULTS: In our cohort (N = 23), median age was 18 years (interquartile range [IQR], 14-23), median age of FRDA onset was 8 years (IQR 6-13), median Friedreich Ataxia Rating Scale score was 58 (IQR 54-62), and GAA repeat length on the shorter FXN allele (GAA1) was 766 (IQR, 650-900). Twenty-one (91%) completed a maximal CPET (n = 8, ACE and n = 13, RLCE). Age, sex, and GAA1 repeat length were each associated with peak VO2. Preliminary estimates demonstrated reasonable agreement between visits 2 and 3 for peak work by both ACE and RLCE, and for peak VO2, O2 pulse, and AT by RLCE. We did not detect significant performance changes over 1 year. DISCUSSION: Adaptive CPET is feasible in FRDA, a relevant clinical trial outcome for interventions that impact exercise performance and will increase access to participation as well as generalizability of findings.


Subject(s)
Exercise Test , Friedreich Ataxia , Adult , Child , Humans , Adolescent , Friedreich Ataxia/diagnosis , Reproducibility of Results , Oxygen Consumption , Respiratory Function Tests
3.
Cureus ; 15(9): e44805, 2023 Sep.
Article in English | MEDLINE | ID: mdl-37809113

ABSTRACT

Angioimmunoblastic T-cell lymphoma (AITL) is a subtype of peripheral T-cell lymphoma with a nonspecific clinical presentation. Cutaneous manifestations of AITL are variable and include morbilliform eruptions, urticaria, papulonodules, and erythroderma. We present the case of a 74-year-old male with a medical history of AITL presenting with diffuse erythematous macules and papules coalescing into patches and plaques on the trunk and bilateral upper extremities. Histopathology demonstrated a mild perivascular lymphocytic infiltrate in the dermis. By immunohistochemistry, the lymphocytic infiltrate was strongly positive for programmed cell death protein 1 (PD-1) (CD279) as well as cluster of differentiation 3 (CD3), CD5, and (focally) B-cell lymphoma-6 (BCL-6). Many cells within the infiltrate were positive for Epstein-Barr virus (EBV) by in situ hybridization. Additionally, a bone marrow biopsy demonstrated an atypical lymphoid infiltrate with T-cell predominance, many EBV-positive cells, and clonal T-cell receptor (TCR) beta gene rearrangement. Based on these histopathological findings, a diagnosis of recurrent AITL with cutaneous involvement was made. This case is a rare example of skin findings presenting as a first sign of recurrent AITL.

4.
Med Sci Educ ; 33(5): 1155-1163, 2023 Oct.
Article in English | MEDLINE | ID: mdl-37886297

ABSTRACT

Introduction: Medical trainees do not have many opportunities to develop communication skills with patients. We established the voluntary "My Life, My Story" (MLMS) program at the Clement J. Zablocki VAMC in Milwaukee, WI, to determine if this pilot narrative medicine program enhanced trainee interpersonal skills and improved patient-centered care. Methods: Trainees at the Medical College of Wisconsin conducted in-person or virtual interviews of Veterans receiving care at the Milwaukee VAMC about their meaningful life experiences. Post-interview, trainees wrote a short first-person narrative in the Veteran's voice, which, after the Veteran's approval, was added to the electronic medical record and made available to the patient's care team. Trainees, Veterans, and health professionals completed post-interview surveys, from which we conducted descriptive statistics and qualitatively analyzed the text-based feedback. Results: Between 2020 and 2021, 24 medical trainees participated in our pilot implementation of the MLMS program, conducting a total of 32 interviews. All trainees reported a meaningful personal impact and found the pilot to be "valuable" and "rewarding." Both trainees and health professionals believed that the MLMS program improved "rapport building" with Veterans. Nearly all Veterans (n = 25, 93%) believed that their medical care team would be able to provide better care after reading their life story. Conclusions: Narrative medicine initiatives like the MLMS program may enable value-added education for trainees. Future research will allow us to better understand and maximize specific educational gains, while further enhancing patient care. Supplementary Information: The online version contains supplementary material available at 10.1007/s40670-023-01854-4.

5.
Cutis ; 112(1): E4-E6, 2023 07.
Article in English | MEDLINE | ID: mdl-37611295
6.
Pediatr Cardiol ; 44(8): 1691-1701, 2023 Dec.
Article in English | MEDLINE | ID: mdl-37382636

ABSTRACT

The Pediatric Heart Network's Fontan Udenafil Exercise Longitudinal (FUEL) Trial (Mezzion Pharma Co. Ltd., NCT02741115) demonstrated improvements in some measures of exercise capacity and in the myocardial performance index following 6 months of treatment with udenafil (87.5 mg twice daily). In this post hoc analysis, we evaluate whether subgroups within the population experienced a differential effect on exercise performance in response to treatment. The effect of udenafil on exercise was evaluated within subgroups defined by baseline characteristics, including peak oxygen consumption (VO2), serum brain-type natriuretic peptide level, weight, race, gender, and ventricular morphology. Differences among subgroups were evaluated using ANCOVA modeling with fixed factors for treatment arm and subgroup and the interaction between treatment arm and subgroup. Within-subgroup analyses demonstrated trends toward quantitative improvements in peak VO2, work rate at the ventilatory anaerobic threshold (VAT), VO2 at VAT, and ventilatory efficiency (VE/VCO2) for those randomized to udenafil compared to placebo in nearly all subgroups. There was no identified differential response to udenafil based on baseline peak VO2, baseline BNP level, weight, race and ethnicity, gender, or ventricular morphology, although participants in the lowest tertile of baseline peak VO2 trended toward larger improvements. The absence of a differential response across subgroups in response to treatment with udenafil suggests that the treatment benefit may not be restricted to specific sub-populations. Further work is warranted to confirm the potential benefit of udenafil and to evaluate the long-term tolerability and safety of treatment and to determine the impact of udenafil on the development of other morbidities related to the Fontan circulation.Trial Registration NCT0274115.


Subject(s)
Oxygen Consumption , Sulfonamides , Humans , Child , Sulfonamides/therapeutic use , Exercise , Pyrimidines/therapeutic use , Exercise Test , Exercise Tolerance
7.
Cureus ; 15(4): e37457, 2023 Apr.
Article in English | MEDLINE | ID: mdl-37187636

ABSTRACT

Renal cell carcinoma (RCC) is a common cancer type in the United States, and at the time of diagnosis, many patients already have metastatic disease. RCC typically metastasizes to the lungs, liver, and bones, with few cases manifesting cutaneous metastasis. Most incidences of RCC metastases reported in the literature have been on the face and scalp. We discuss a case of a 64-year-old male patient who presented with a history of RCC and a purpuric nodule on his lateral thigh. Histopathological examination revealed vacuolated cytoplasm with areas of cytoplasmic clearing; the cells stained positively for cytokeratin AE1/AE3, CAM5.2, and PAX8. Cutaneous metastatic RCC was subsequently diagnosed. Cutaneous manifestations of RCC, particularly to the thigh, remain a rare presentation of metastatic RCC.

8.
J Drugs Dermatol ; 22(5): 451-456, 2023 May 01.
Article in English | MEDLINE | ID: mdl-37133469

ABSTRACT

Understanding the metastatic potential of a skin cancer is essential to effective management. Gene expression profiling (GEP) is an innovative technology that has allowed for a better understanding of tumor biology in various skin cancers. Current methods focus on identifying and quantifying ribonucleic acid (RNA) transcripts in tissue samples. Using reverse transcriptase-polymerase chain reaction, specific RNA transcripts are reverted into deoxyribonucleic acid (DNA) for quantification. The addition of RNA-seq has further enhanced our knowledge of genomes not only by measuring known sequences, but also by identifying novel genes in various skin cancers. GEP requires only a small amount of RNA and has a high level of reproducibility. Using this technology, several GEPs for skin cancers have been developed to augment diagnosis and prognosis of skin cancer. This article reviews the process of gene expression profiling and the current GEPs that are available or under investigation for skin cancer. J Drugs Dermatol. 2023;22(5): doi:10.36849/JDD.7017.


Subject(s)
Gene Expression Profiling , Skin Neoplasms , Humans , RNA, Messenger/analysis , RNA, Messenger/genetics , RNA, Messenger/metabolism , Reproducibility of Results , Gene Expression Profiling/methods , RNA/analysis , Skin Neoplasms/diagnosis , Skin Neoplasms/genetics
10.
Plast Reconstr Surg ; 151(3): 388e-397e, 2023 03 01.
Article in English | MEDLINE | ID: mdl-36730490

ABSTRACT

BACKGROUND: "Prominent ear" remains one of the most common referrals to pediatric plastic surgery. The perceived deformity has been corrected using a multitude of techniques for over a century, and significant variation in practice still exists. Recent studies suggesting that cartilage-scoring techniques are associated with more major complications than suture techniques may have led to an adverse international perception of the technique. Thus, waning use of anterior scoring prominent ear correction appears to be occurring. For appropriate cases, the authors have used anterior scoring for over 20 years, with high patient satisfaction and low complication rates. They present a review of all cases and outcomes from 2005 to 2015. The authors believe this is the largest case series of anterior scoring otoplasty published to date. METHODS: All pediatric cases undergoing prominent ear correction from 2005 to 2015 were included in this retrospective case note analysis and follow-up study. Patient demographics, operative details including early and late complications, and postoperative results were analyzed. METHODS: Over a 10-year period, 1199 otoplasties were performed (1134 bilateral, 65 unilateral), for a total of 2333 ear corrections. A total of 1575 ears were corrected using the anterior scoring technique. The remaining cases underwent correction by means of suture only, cartilage reduction, or combination techniques. There was a significantly lower all-cause reoperation rate for anterior scoring compared to suture-only techniques ( P = 0.0039; significant at P < 0.025). There were no reported cases of cartilage necrosis. CONCLUSIONS: This study demonstrates that in appropriately selected patients, anterior scoring otoplasty is a low-morbidity procedure. In the authors' institution, when compared to suture techniques, it was associated with a lower rate of complications and reoperation rate than suture-only techniques. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.


Subject(s)
Ear, External , Patient Satisfaction , Humans , Child , Follow-Up Studies , Retrospective Studies , Ear, External/surgery , Suture Techniques , Ear Cartilage/surgery , Treatment Outcome
11.
Cleft Palate Craniofac J ; 60(7): 823-832, 2023 Jul.
Article in English | MEDLINE | ID: mdl-35234518

ABSTRACT

OBJECTIVE: To evaluate the results of a single stage composite cleft septorhinoplasty procedure ("The Gujrat Technique") to correct the exaggerated cleft nose deformity after completion of nasal growth in an adult patient cohort. METHODS: Adult patients with a residual unilateral cleft nasal deformity were deemed eligible for the proposed "Gujrat Technique". Over a 10-year period (2007-2017), 96 adult patients underwent this composite cleft septorhinoplasty as a single stage operation. Post-operative nasal symmetry evaluation was undertaken using the validated computer program 'SymNose'. Functional outcome and patient satisfaction were assessed using Nasal Obstruction Symptom Evaluation scale and Rhinoplasty Outcome Evaluation (ROE) questionnaires respectively. Various statistical analysis methods were used to validate the obtained results. RESULTS: Due to poor compliance with follow-up, post-operative assessments were undertaken in only 32 patients. The single group study design using the non-parametric matching pairs Wilcoxon Sign test (p < 0.001) showed overall good to excellent functional and aesthetic outcomes and higher scores of the digital SymNose grading system. There was a significant improvement in ROE scores (from 26.4 ± 2.9 to 85.9 ± 4.7, p < 0.001). There were no major complications or revisions needed in our series. CONCLUSION: The individual components of "The Gujrat Technique" are not novel but their combination in this adult unilateral cleft rhinoplasty cohort has demonstrated a high patient satisfaction with its aesthetic appeal and functional versatility. In the background of limited resources and unpredictable patient follow up, the simplicity, reproducibility and cost effectiveness of this technique make it a practical reconstructive option.


Subject(s)
Cleft Lip , Nose Diseases , Respiratory System Abnormalities , Rhinoplasty , Adult , Humans , Rhinoplasty/methods , Cleft Lip/surgery , Cleft Lip/complications , Reproducibility of Results , Treatment Outcome , Esthetics, Dental , Nose/surgery , Nose Diseases/surgery
12.
Phys Sportsmed ; 51(3): 260-268, 2023 06.
Article in English | MEDLINE | ID: mdl-35191360

ABSTRACT

BACKGROUND: Lower limb injuries constitute a problem in the sport of football. Our aim is to explore patterns and trends of lower limb injuries in an English professional football club. METHODS: This is a descriptive epidemiological study. Reports provided by the club's physical therapy team were screened for injuries among professional football players sustained over four seasons, from 2015/2016 to 2018/2019. Data included setting of injury (in-match or training), anatomical location of injury, type of injury, number of days off and month of injury. RESULTS: A total of 296 lower limb injuries were recorded in our study, with a rate of 11.14 per 1000 football hours. Injury rate during games was 51.38 per 1000 football hours, significantly greater than that during training at 3.81 per 1000 football hours (p = 0.021). The thigh was significantly the most commonly injured location at 4.67 per 1000 football hours (p < 0.001). Grade I tears were the most common injury type at 1.73 per 1000 football hours, significantly greater than grade III tears (p = 0.027), contusions (p = 0.043), fractures (p = 0.02), and lacerations (p = 0.019). Injury rates were found to be greatest during preseason and declined as season progressed. On average, an injury sidelined the affected footballer for a total of 20 days. CONCLUSION: Lower limb injuries were more common during matches than training. The thigh is the most common injury location, and grade I muscle tear was the most common type of injury. Injury rates were higher early on in the season. Potential prevention strategies include spacing out competition, adopting training and exercise regimens that cater for recovery, and increasing research regarding injury mechanisms.


Subject(s)
Athletic Injuries , Soccer , Sprains and Strains , Humans , Incidence , Lower Extremity/injuries , Soccer/injuries
13.
J Am Heart Assoc ; 11(24): e027464, 2022 12 20.
Article in English | MEDLINE | ID: mdl-36515264

ABSTRACT

Background Skeletal muscle deficits are associated with worse exercise performance in the Fontan circulation and may be improved by exercise training. We aimed to assess the change in leg lean mass (a marker of skeletal muscle), exercise performance, and functional health status after a lower extremity-focused exercise intervention in adolescents with Fontan circulation. Methods and Results Densitometry for measurement of leg lean mass, cardiopulmonary exercise test, exercise cardiac magnetic resonance, peripheral vascular testing, physical activity questionnaire, and quality of life assessment were performed at baseline and after a 24-week, hybrid center- and home-based training program. Leg lean mass Z-scores were generated, and exercise parameters were expressed as percentage expected based on reference data. The effect of training was assessed by paired t-tests and simple linear regression. Twenty participants (15.6±1.7 years, 50% male) demonstrated low baseline leg lean mass Z-scores with no significant improvement with training (-1.38±1.02 pre versus -1.31±1.06 post, P=0.33). Maximum and percent predicted work increased from 121.9±29.8 (0.66±0.12) to 131.3±35.1 (0.70±0.15) watts (P=0.02). Peak respiratory exchange ratio increased (1.19±0.02 versus 1.25±0.01, P=0.02) but percent predicted oxygen consumption was unchanged, suggesting higher anaerobic activity after training. Physical activity questionnaire score positively associated with peak work at baseline (ß=18.13 [95% CI, 0.83-35.44], R2=0.21; P=0.04) but physical activity questionnaire, quality of life scores, exercise cardiac magnetic resonance performance, and peripheral vascular function were unchanged with training. Conclusions Peak work rate and anaerobic activity increased with lower extremity-focused training in adolescents with Fontan circulation. Larger studies should test the impact of these changes on functional status and quality of life.


Subject(s)
Fontan Procedure , Heart Defects, Congenital , Humans , Male , Adolescent , Female , Quality of Life , Fontan Procedure/adverse effects , Exercise/physiology , Lower Extremity/surgery , Muscle, Skeletal , Exercise Test , Exercise Tolerance/physiology , Oxygen Consumption , Heart Defects, Congenital/surgery
14.
Cureus ; 14(9): e29777, 2022 Sep.
Article in English | MEDLINE | ID: mdl-36340545

ABSTRACT

Neonatal limb ischaemia is a rare disease entity with devastating morbidity, including compartment syndrome, tissue loss, limb loss, reduced limb growth, irreparable neuropathies and Volkmann's syndrome.  We report a case of limb revascularisation and salvage due to intrauterine brachial artery thrombosis. Published literature is limited to case reports and case series, with various treatment modalities discussed. Early recognition, prompt institution of appropriate treatment and monitoring is vital to achieve successful revascularisation and prevention of life-long morbidity. A male baby at (36+6week) gestation was born to a nulliparous mother with gestational diabetes via uncomplicated elective caesarean section. Aetiology was due to dense fibrotic circumferential constriction of the brachial vessels and plexus. Successful revascularization was achieved with a contralateral interposition reversed great saphenous vein graft.  Though extremely rare and the clinical presentation varies with the location and timing after birth, the surgeon should maintain a low threshold for suspicion of in the presence of the characteristic sequelae of ischaemia. Doppler ultrasonography can aid the diagnosis where ambiguous, and therapy should be individualised based on the clinical presentation; this case emphasises the role of surgery in limb salvage.

15.
Front Pediatr ; 10: 1025420, 2022.
Article in English | MEDLINE | ID: mdl-36275051

ABSTRACT

Background: Skeletal muscle deficits are associated with worse exercise performance in adults with pulmonary hypertension (PH) but the impact is poorly understood in pediatric PH. Objective: To study muscle deficits, physical inactivity, and performance on cardiopulmonary exercise test (CPET) and exercise cardiac magnetic resonance (eCMR) in pediatric PH. Methods: Youth 8-18 years participated in a prospective, cross-sectional study including densitometry (DXA) for measurement of leg lean mass Z-score (LLMZ), handheld dynamometer with generation of dominant and non-dominant handgrip Z-scores, Physical Activity Questionnaire (PAQ), CPET, and optional eCMR. CPET parameters were expressed relative to published reference values. CMR protocol included ventricular volumes and indexed systemic flow at rest and just after supine ergometer exercise. Relationships between LLMZ, PAQ score, and exercise performance were assessed by Pearson correlation and multiple linear regression. Results: There were 25 participants (13.7 ± 2.8 years, 56% female, 64% PH Group 1, 60% functional class I); 12 (48%) performed both CPET and eCMR. Mean LLMZ (-0.96 ± 1.14) was associated with PAQ score (r = 50, p = 0.01) and with peak oxygen consumption (VO2) (r = 0.74, p = < 0.001), VO2 at anaerobic threshold (r = 0.65, p < 0.001), and peak work rate (r = 0.64, p < 0.01). Higher handgrip Z-scores were associated with better CPET and eCMR performance. On regression analysis, LLMZ and PAQ score were positively associated with peak VO2, while handgrip Z-score and PAQ score were positively associated with peak work rate. Conclusion: Muscle mass and strength are positively associated with exercise performance in pediatric PH. Future studies should determine the effect of rehabilitation programs on muscle properties and exercise performance.

16.
Pediatr Cardiol ; 43(5): 1029-1036, 2022 Jun.
Article in English | MEDLINE | ID: mdl-35137274

ABSTRACT

Children with advanced heart failure may require ventricular assist devices (VAD) while awaiting heart transplantation. Currently, no data exist regarding the safety of exercise rehabilitation (ER) in children on VAD support. The purpose of this study was to determine the safety and feasibility of ER in children on VAD support awaiting heart transplantation. Eligible patients underwent VAD placement between 1998 and 2019; both inpatient and outpatient participants were included. After VAD implantation and when ambulatory, patients were enrolled in ER. Exercise sessions were scheduled three times a week and consisted of aerobic and musculoskeletal conditioning. A total of 29 patients (59% male, mean age 14 ± 3.2 years) were included with a median VAD duration of 120 ± 109 days. Cardiac diagnoses included cardiomyopathy (81%) and congenital heart disease (19%). VAD type included pulsatile (59%) and continuous-flow devices (41%). Eight hundred and sixty-four (85%) ER sessions were successfully completed and began at a mean of 49 days (range 19-108) after VAD implant. No adverse events, including episodes of hypotension, significant complex arrhythmia, or VAD malfunction occurred during exercise testing or ER, and no sessions were discontinued prematurely. Pediatric patients on VAD support can safely participate in ER with relatively high compliance, and sessions can be implemented early after VAD implantation. Given the safety profile, ER in pediatric VAD recipients, which is a modifiable pre-transplant risk factor that may improve functional capacity, warrants further study as a potential modality to improve post-transplant outcomes.


Subject(s)
Heart Failure , Heart Transplantation , Heart-Assist Devices , Adolescent , Child , Feasibility Studies , Female , Heart Failure/etiology , Heart Failure/surgery , Heart-Assist Devices/adverse effects , Humans , Male , Retrospective Studies , Treatment Outcome
17.
J Med Biol Eng ; 42(1): 1-10, 2022.
Article in English | MEDLINE | ID: mdl-35095378

ABSTRACT

Purpose: Human respiratory aerosols may have important implications for transmission of pathogens. The study of aerosol production during vigorous breathing activities such as exercise is limited. In particular, data on aerosol production during cardiopulmonary exercise testing (CPET) are lacking. Methods: In this pilot project, we used a high-powered, pulsed Nd:YAG laser to illuminate a region of interest in front of two healthy adult subjects during CPET. Subjects exercised to the point of respiratory compensation. Images were captured with a high-speed, high-resolution camera to determine net exhaled particle (NEP) counts at different phases of CPET, including resting breathing, submaximal exercise, peak exercise, and active recovery. Experiments were performed with the room ventilation activated. Results: Net exhaled particle counts remained relatively constant until late/peak exercise when they decreased prior to rebounding into recovery. NEP counts at resting breathing were higher than those reported using other methods of measurement. Exhaled particles were in the submicron size range. Conclusion: Our method of aerosol particle quantification enables measurement of significant quantities of ultrafine particles and dynamic assessment of aerosol production during CPET. The unique pattern of aerosol production observed during submaximal and peak exercise suggests that extension of results from resting breathing to CPET may not be appropriate.

18.
JACC Adv ; 1(4): 100107, 2022 Oct.
Article in English | MEDLINE | ID: mdl-38939702

ABSTRACT

Background: Exercise stress testing (EST) in pediatric hypertrophic cardiomyopathy (HCM) patients has not well described in a large heterogenous cohort. Objectives: The objective of the study was to determine the clinical utility of EST in pediatric HCM. Methods: This was a retrospective single-center analysis of HCM patients younger than 21 years who had EST between January 1, 2000, and January 1, 2019. Clinical, demographic characteristics, and EST data were analyzed, using the last EST during the study or prior to the event in subjects with a primary outcome. The primary composite endpoint included cardiac death, transplant, or arrhythmia requiring implantable cardioverter-defibrillator placement. Outcome analysis was performed using Cox proportional hazard modeling. Results: The study cohort included 140 patients, 52% with a recognized genetic variant. There were 2 tests aborted due to safety concerns (ST-segment changes, ventricular ectopy). The median age at first EST was 13.6 years. Ninety percent of patients were tested using cycle ergometry, and 44% were on a beta-blocker. The median peak oxygen consumption was 37.1 mL/kg/min (IQR: 12.5 mL/kg/min) or 81.2% predicted, the mean anaerobic threshold was 21.8 Ml (IQR: 8.3 mL), and the median peak power was 2.6 ± 1.1 W/kg or 73.7% predicted. Ectopy during EST was seen in 44% of patients, and 8% had an abnormal blood pressure response to exercise. The endpoint was reached in 12 patients. The presence of any degree of ectopy was a predictor of the composite endpoint (hazard ratio: 5.8; 95% CI: 1.3-26.7). Conclusions: EST is clinically useful in select pediatric patients with HCM. Ectopy on EST is a risk factor for cardiac death, cardiac transplant, and arrhythmias requiring implantable cardioverter-defibrillator.

19.
J Craniofac Surg ; 32(3): 952-955, 2021 May 01.
Article in English | MEDLINE | ID: mdl-34779595

ABSTRACT

ABSTRACT: Throat packs are used in a wide range of surgical and dental procedures. Indications for use include; airway stabilization, reducing the risk of aspiration, and postoperative nausea and vomiting. Despite these perceived benefits, a retained throat pack can be fatal. This has been highlighted since the 1970s but as of yet no effective fail-safe mechanisms exist to prevent the potential tragic consequences of a retained throat pack.A multifaceted questionnaire was distributed at the annual Craniofacial Society of Great Britain and Ireland 2017 to identify current views and practices amongst cleft surgeons. The questionnaire contained seventeen questions related to throat pack use. The responses to the questions were collated and discussed in the context of the current literature.Twenty-four cleft surgeons completed the questionnaire; 67% always use a throat pack, with 84% not securing the throat pack in any way. Almost half (48%) had encountered a serious adverse incident secondary to the use of a throat pack.This survey and review of the literature highlight ongoing discrepancies and a lack of standardization on a national basis. Adverse incidents and fatalities are still occurring. Overwhelming evidence now confirms the lack of benefit regarding postoperative nausea and vomiting and the authors urge both surgeons and anesthetists to strongly consider the need for a throat pack in routine elective procedures.


Subject(s)
Pharynx , Postoperative Nausea and Vomiting , Humans , Ireland , Neck , Pharynx/surgery , Postoperative Nausea and Vomiting/prevention & control , United Kingdom
20.
Shoulder Elbow ; 13(3): 329-333, 2021 Jun.
Article in English | MEDLINE | ID: mdl-34659474

ABSTRACT

AIMS: To review patients treated with a functional latissimus dorsi flap for congenital and acquired elbow flexion deficits. METHODS: Retrospective review of functional latissimus dorsi flaps performed in one regional unit. Patient notes were reviewed to determine aetiology, pre-op deficits and function, surgical technique, complications and outcomes. RESULTS: A total of six functional latissimus dorsi transfers were performed on four patients. Two patients had bilateral latissimus dorsi transfers for congenital defects. The remaining two procedures were for traumatic defects. Post-operatively both children had excellent outcomes with full range of active movement allowing them to perform key activities of daily living. SURGICAL TECHNIQUE: Epimysium of latissimus dorsi folded to form a pseudo-tendon, tunnelled subcutaneously and either attached to a remnant of biceps tendon or secured to the radius. Congenital patients achieved better outcomes; pre-operatively, there was no active elbow flexion in all four elbows but 90-100 of passive flexion. COMPLICATIONS: One latissimus dorsi dehiscence which required revision surgery. Two donor-site seromas. CONCLUSIONS: Functional latissimus dorsi transfer has been shown to achieve excellent elbow flexion in patients with congenital absence of biceps and brachialis muscles. Outcomes in older patients with traumatic injuries have been less successful in achieving a full range of active flexion.

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