Corrigendum: Case report: Selexipag in pediatric pulmonary hypertension: initiation, transition, and titration.

[This corrects the article DOI: 10.3389/fped.2023.1050508.].

Hereditary pulmonary arterial hypertension burden in pediatrics: A single referral center experience.

Introduction: Hereditary pulmonary arterial hypertension (HPAH) is a rare yet serious type of pulmonary arterial hypertension (PAH). The burden in the pediatric population remains high yet underreported. The objective of this study is to describe the distribution of mutations found on targeted PAH panel testing at a large pediatric referral center. Methods: Children with PAH panel administered by the John Welsh Cardiovascular Diagnostic Laboratory at Texas Children's Hospital and Baylor College of Medicine in Houston, Texas between October 2012 to August 2021 were included into this study. Medical records were retrospectively reviewed for clinical correlation. Results: Sixty-six children with PAH underwent PAH genetic testing. Among those, 9 (14%) children were found to have pathogenic mutations, 16 (24%) children with variant of unknown significance and 41 (62%) children with polymorphism (classified as likely benign and benign). BMPR2 mutation was the most common pathogenic mutation, seen in 6 of the 9 children with detected mutations. Hemodynamic studies showed higher pulmonary vascular resistance among those with pathogenic mutations than those without (17.4 vs. 4.6 Wood units). All children with pathogenic mutations had severe PAH requiring triple therapy. There were tendencies for higher lung transplantation rate but lower mortality among those with pathogenic mutations. Conclusions: Abnormalities on genetic testing are not uncommon among children with PAH, although majority are of unclear significance. However, children with pathogenic mutations tended to present with more severe PAH requiring aggressive medical and surgical therapies. Genetic testing should be routinely considered due to consequences for treatment and prognostic implications. Larger scale population studies and registries are warranted to characterize the burden of HPAH in the pediatric population specifically.

Case Report: Selexipag in pediatric pulmonary hypertension: Initiation, transition, and titration.

Selexipag, a selective prostacyclin receptor agonist, is approved for treating pulmonary arterial hypertension in WHO Group 1 adult patients. Compared to parenteral prostacyclin formulations, selexipag offers a significant improvement in patient's and caregiver's quality of life because of its oral formulation, frequency of administration, and mechanism of action. Although experience in the pediatric population is limited to case reports in older adolescent patients and selexipag is not approved for use in the pediatric pulmonary hypertension population, many pediatric centers are expanding the use of this therapy to this population. We report our institution's experience in the use of selexipag to treat pulmonary hypertension in children under 10 years of age, between 10 and 30 kg. Seven patients were initiated on selexipag therapy including de novo initiation and transition from intravenous treprostinil to oral selexipag. All patients were on stable background therapy with phosphodiesterase-5 inhibitor and endothelin receptor antagonist therapies at baseline. All patients reached their planned goal selexipag dose during admission without the need for changes to the titration schedule and without hemodynamic deterioration. In our experience, oral selexipag is safe and well-tolerated in young pediatric patients with pulmonary hypertension. Based on our favorable experience, we developed an institution-specific selexipag process algorithm for continued successful use in the pediatric population.

The role of a multidisciplinary team in a pediatric pulmonary hypertension center.

The multidisciplinary team in a pediatric pulmonary hypertension (PH) center can improve the delivery of care to the PH patient by helping them address the different challenges that correlate to a PH diagnosis. Currently, there are a limited number of accredited pediatric PH centers nationwide, and many healthcare facilities have little experience managing patients with this complex and rare disease. Patients with PH may see providers from multiple medical specialties, inherit a high-cost burden from their PH medications, and have little community backing due to unfamiliarity of the disease. The multidisciplinary team can embrace these challenges. Through the delineation of tasks and roles within the composition of the team, patients can experience the support, resources, and care they need. The composition of the team can vary from center to center, but it may include an attending physician, advanced practice provider, nurse, dietitian, physiologists, respiratory therapists, social workers, research coordinators, and subspecialty collaboration including cardiology, pulmonology, genetics, psychology, and palliative care. When composing a multidisciplinary team, consider the heterogeneity of the patient population being served. Look at the resources available and overall community familiarity with PH. It is important to know the center's limits and refer to an expert PH center as necessary. The goal for every patient with pulmonary hypertension is to maximize their quality of life and outcomes, and the use of the multidisciplinary team is one approach to reaching this goal.

Biomechanical analysis of rotator cuff repairs with extracellular matrix graft augmentation.

Despite advances in surgical techniques, 20% to 90% of rotator cuff (RTC) repairs fail. They tend to fail at the suture-tendon junction due to tension at the repair and gap formation prior to healing. This study evaluated the gap formation and ultimate tensile failure loads of a RTC repair with a decellularized human dermal allograft. Augmentation of a RTC repair with an extracellular matrix graft decreased gap formation and increased load to failure in a human RTC repair model.

Incidence of heterotopic ossification in direct anterior total hip arthroplasty: a retrospective radiographic review.

Heterotopic ossification (HO) is a complication following total hip arthroplasty (THA) with traditional approaches. The direct anterior approach (DAA) has become a popular approach for THA; however, no study has evaluated HO formation following DAA THA. We examined the incidence of HO in a consecutive series of THA using the DAA in two separate hospitals. Standard preoperative radiographs were examined to determine the type of degenerative arthritis, and follow-up radiographs of at least 6 months after surgery were evaluated for the presence and classification of HO. The overall incidence of HO after DAA THA in this study was 98/236, or 41.5%, which falls within the reported range from recent studies involving more traditional approaches to the hip.

Masked hypodiploidy in anaplastic meningiomas by duplication of the original clone found in atypical meningiomas: illustration of the evolution of genetic alterations.

Meningiomas are common, usually benign neoplasms of the central nervous system. Atypical and anaplastic meningiomas can be aggressive, show more rapid growth, and a greater propensity to recur following resection. General consensus believes that genetic abnormalities leading to anaplastic transformation are present at initial tumor presentation; however, this has not been demonstrated by array-comparative genome hybridization. We confirm the hypothesis by showing the evolution of genetic alterations in the transformation of an atypical meningioma to an anaplastic meningioma. Additionally, we provide potential genes responsible for malignant transformation of meningiomas, which, with further research, may provide diagnostic and therapeutic implications.

Comprehensive approach to the evaluation of groin pain.

Groin pain is often related to hip pathology. As a result, groin pain is a clinical complaint encountered by orthopaedic surgeons. Approximately one in four persons will develop symptomatic hip arthritis before age 85 years. Groin injuries account for approximately 1 in 20 athletic injuries, and groin pain accounts for 1 in 10 patient visits to sports medicine centers. Many athletes with chronic groin pain have multiple coexisting pathologies spanning several disciplines. In treating these patients, the orthopaedic surgeon must consider both musculoskeletal groin disorders and nonorthopaedic conditions that can present as groin pain. A comprehensive history and physical examination can guide the evaluation of groin pain.

Successful treatment of de Quervain tenosynovitis with ultrasound-guided percutaneous needle tenotomy and platelet-rich plasma injection: a case presentation.

De Quervain tenosynovitis is a disorder of the tendons of the first dorsal compartment of the wrist that causes pain and functional disability, which may be refractory to conservative treatments. We present a case of ultrasound-guided percutaneous needle tenotomy and platelet-rich plasma injection for the successful treatment of de Quervain tenosynovitis.

Removal of trabecular metal osteonecrosis intervention implant and conversion to primary total hip arthroplasty.

Core decompression and placement of the Trabecular Metal Osteonecrosis Intervention Implant have shown to be initially successful in treating early osteonecrosis. When treatment fails, however, patients often undergo primary total hip arthroplasty (THA) requiring removal of a previously inserted trabecular metal implant. We describe a technical tip for removal of a well-ingrown trabecular metal screw. A metal-cutting trephine placed over the screw allows for removal in an efficient manner while minimizing additional dissection and bone loss during conversion to THA.

Treatment of glenohumeral sepsis with a commercially produced antibiotic-impregnated cement spacer.

BACKGROUND: We report our experience in treating infected shoulder arthroplasty and primary shoulder sepsis using a commercially produced antibiotic-impregnated cement spacer. MATERIALS AND METHODS: We treated 16 shoulders in 15 patients for infected arthroplasty or osteomyelitis of the proximal humerus with irrigation and débridement, hardware removal, or humeral head resection, or both, and placement of an interval articulating hemiarthroplasty with a commercially made gentamicin-impregnated cement spacer. RESULTS: Mean follow-up was 20.5 months after spacer placement. At the time of débridement, 12 shoulders had positive cultures; the most common organisms were methicillin-resistant Staphylococcus aureus (n = 3) and S. epidermidis (n = 3). Twelve patients underwent revision. Four refused revision and have retained antibiotic spacers. White blood cell counts returned to within normal ranges in all patients at the time of revision, the erythrocyte sedimentation rate in 5 of 12 patients, C-reactive protein in 8 of 12 patients, and interleukin-6 in 9 of 11 patients. Mean visual analog pain scale score decreased from 8.4 before spacer placement to 0.5 at the final follow-up. Active forward flexion increased from a mean of 65 degrees to 110 degrees , and active external rotation from -5 degrees to 20 degrees . Mean University of California Los Angeles (UCLA) Shoulder Rating Scale score increased from 7 to 26, Simple Shoulder Test (SST) from 1.2 to 6.6, American Shoulder and Elbow Surgeons (ASES) Standardized Shoulder Assessment Form score from 16 to 74, and Constant score from 16 to 57. There was no recurrence of infection. CONCLUSIONS: Treatment of glenohumeral sepsis with a commercially produced antibiotic-impregnated cement spacer appears to be an effective treatment modality, and serum interleukin-6 level appears to be useful in the evaluation of shoulder infection.

Total dislocation of the talus and the navicular: a case report.

Total dislocation of the talonavicular joint is rare. Detection of a major tarsal bone extrusion is simple. However, its management and treatment are not. We present a case of a total extrusion of the talus and navicular of an open dislocation and the reconstruction options that subsequently followed. In spite of a well-perfused foot, our patient decided to undergo a below the knee amputation after an infection.