Multiple primary dermatofibrosarcoma protuberans tumors in a single patient with chromosomal microarray analysis: A case report and review.

Dermatofibrosarcoma protuberans (DFSP) is a cutaneous sarcoma with a high propensity for local invasion and recurrence. Although it is a rare event, the occurrence of multiple tumors in a single patient raises a diagnostic dilemma, as metastatic disease should be differentiated from multiple primary malignant events. In more than 90% of DFSP, a pathogenic t(17;22) translocation leads to the expression of COL1A1::PDGFB fusion transcripts. Karyotype analysis, fluorescence in situ hybridization, and RT-PCR can be useful ancillary studies in detecting this characteristic rearrangement, and sequencing of the fusion transcript can be used to support a clonal origin in metastatic and multifocal disease. However, previous reports have demonstrated variable sensitivity of these assays, in part due to the high sequence variability of the COL1A1::PDGFB fusion. Here, we report a patient who developed two distinct DFSP tumors over the course of 7 years. Chromosomal microarray analysis identified distinctive genomic alterations in the two tumors, supporting the occurrence of multiple primary malignant events.

Pleomorphic Dermal Sarcoma.

Pleomorphic dermal sarcoma (PDS) is a rare cutaneous/subcutaneous neoplasm of purported mesenchymal differentiation that exists along a clinicopathologic spectrum with atypical fibroxanthoma (AFX). While PDS and AFX share histopathologic and immunohistochemical features, PDS exhibits deeper tissue invasion and has a higher rate of metastasis and local recurrence than AFX. Given its aggressive clinical course, early recognition and clinical management of PDS are essential for optimizing patient outcomes. This review aims to provide a brief overview of the clinicopathologic and molecular features, prognosis, and treatment of PDS.

Myelodysplasia Cutis.

CONTEXT.­: Myelodysplasia cutis is an emerging concept in cutaneous neoplasia. Many of these cases were previously included under the umbrella of histiocytoid Sweet syndrome. However, with the advent of next-generation sequencing, cutaneous involvement by myelodysplastic syndrome is being increasingly recognized. OBJECTIVE.­: To review histiocytoid Sweet syndrome and myelodysplasia cutis and discuss our current understanding of these entities. Additionally, to discuss how next-generation sequencing can be applied in the evaluation of cutaneous infiltrates of immature histiocytoid cells. DATA SOURCES.­: The English-language literature from 2005 to 2023 on the topic of histiocytoid Sweet syndrome and myelodysplasia cutis was reviewed. CONCLUSIONS.­: Biopsy specimens showing infiltrates of histiocytoid, immature myeloid cells may represent cutaneous involvement by myelodysplastic syndrome. Close clinical correlation is recommended in these cases. Recent studies suggest that next-generation sequencing is useful in separating myelodysplasia cutis from true histiocytoid Sweet syndrome. This distinction has important implications for patients.

[Digital artery pseudoaneurysm. Case report and systematic review of the literature]. / Pseudoaneurisma de arteria digital. Reporte de un caso y revisión sistemática de la bibliografía.

INTRODUCTION: aneurysms are focal and permanent dilations of an artery; in pseudoaneurysms, the normal layers of the blood vessel are replaced by fibrous tissue. Due to their low incidence, as well as the diagnostic and therapeutic challenge they represent; our objective is to present the clinical case of a pseudoaneurysm of a digital artery of the hand and to carry out a systematic review of this pathology. MATERIAL AND METHODS: literature search in Medline, using the terms "digital artery" and "aneurysm." Studies of vascular dilation pathology affecting the hand and fingers were incorporated. Studies with pathology of proximal involvement of the hand were excluded. CASE PRESENTATION: a 79-year-old female patient who, after a sharp force trauma to the fifth finger of the left hand, develops a rapidly growing necrotic tumor. She had ultrasound and angiography that suggested hematoma. Surgical management was decided, during which it was observed that the tumor involved ulnar collateral digital artery of the fifth finger. The lesion and the arterial segment involved were resected. Post-surgical course without complications. The histopathological diagnosis of pseudoaneurysm of the lesion was confirmed. DISCUSSION: traumatic etiology is the most frequent cause of digital aneurysms. Risk factors for pseudoaneurysms include sharp force trauma and alterations of the coagulation pathways, as in the case presented. CONCLUSION: the pseudoaneurysm of a digital artery is a rare pathology with great variability of therapeutic management. Surgical resection of the lesion with vascular flow reconstruction is the recommended treatment.

INTRODUCCIÓN: los aneurismas son dilataciones vasculares localizadas y permanentes de una arteria; en los pseudoaneurismas, las capas normales del vaso sanguíneo son reemplazadas por tejido fibroso. Debido a su baja incidencia, así como el desafío diagnóstico y terapéutico que representan; nuestro objetivo es presentar el caso clínico de un pseudoaneurisma de una arteria digital de la mano y realizar una revisión sistemática sobre dicha patología. MATERIAL Y MÉTODOS: búsqueda bibliográfica en Medline, utilizando los términos "arteria digital" y "aneurisma". Se incorporaron estudios de patología de dilatación vascular que afecte la mano y los dedos. Se excluyeron trabajos con patología de afección proximal de la mano. PRESENTACIÓN DE CASO: paciente femenino de 79 años de edad, que posterior a herida cortante de quinto dedo de mano izquierda, desarrolla tumoración necrótica de rápido crecimiento. Contaba con ecografía y angiografía que sugerían hematoma. Se decidió manejo quirúrgico, durante el cual se observó que la tumoración involucraba arteria digital colateral cubital del quinto dedo. Se resecó lesión y segmento arterial involucrado. Cursó postquirúrgico sin complicaciones. Se confirmó el diagnóstico histopatológico de pseudoaneurisma de la lesión. DISCUSIÓN: la etiología traumática es la causa más frecuente de los aneurismas digitales. Los factores de riesgo para los pseudoaneurismas incluyen lesiones penetrantes y alteraciones de la cascada de coagulación, como en el caso presentado. CONCLUSIÓN: el pseudoaneurisma de una arteria digital es una patología rara y con gran variabilidad de manejo terapéutico. La resección quirúrgica de la lesión con la reconstrucción del flujo vascular, es el tratamiento recomendado.

Late consolidation of the scaphoid. Case report and literature review.

Scaphoid fractures are a common wrist injury accounting for 2-7% of all adult fractures. Nonunion is described in 5-12% of cases leading to osteoarthritis. Several classifications have been developed focused on this pathology and its complication. We present a case of a 28 years old male patient with a scaphoid fracture and nonunion who spontaneously consolidates without treatment. We performed a literature review to recognize this pathology, its common evolution and possible treatment options.

Las fracturas de escafoides son una lesión frecuente de la muñeca y representan de 2-7% de todas las fracturas en adultos. La no unión se describe en 5-12% de los casos y conduce a la osteoartritis. Se han desarrollado varias clasificaciones centradas en esta patología y su complicación. Presentamos el caso de un paciente varón de 28 años con fractura y no unión de escafoides que consolida espontáneamente sin tratamiento. Realizamos una revisión bibliográfica para reconocer esta patología, su evolución habitual y las posibles opciones de tratamiento.

La utilización de un implante anatómico comparado a una LCP recta disminuye su extracción en la osteosíntesis percutánea posterior del húmero / The use of an anatomical implant compared to a straight LCP decreases extraction in posterior humeral MIPO

Antecedentes y objetivo: El abordaje posterior percutáneo del húmero se ha descrito utilizando una placa LCP de 4,5mm. A pesar de que las placas rectas han demostrado buenos resultados, estas no han sido diseñadas para adaptarse a la metáfisis del húmero distal. El objetivo de este estudio es probar la hipótesis nula de que no existen diferencias en la extracción de la osteosíntesis después de una MIPO posterior utilizando una placa recta comparado a una placa anatómica. Materiales y métodos: Se incluyeron retrospectivamente en 2 instituciones pacientes mayores de 18 años que habían sufrido una fractura diafisaria de húmero distal tratados mediante técnica percutánea posterior con una placa bloqueada y con un seguimiento mínimo de 12 meses. Los pacientes se dividieron en: grupo 1 (placa recta LCP de 4,5mm) y grupo 2 (placa de forma anatómica de 3,5mm). Durante el postoperatorio se reportó la evaluación clínica y radiológica, así como la necesidad de retirar el implante debido al dolor. Resultados: Sesenta y siete pacientes cumplieron los criterios de inclusión. Veintisiete pacientes en el grupo 1 y 40 en el grupo 2. No se perdió ningún paciente durante el seguimiento. Dentro del grupo 1, el 18% (IC 95%: 6-38%) de los pacientes requirieron extracción del implante, mientras que en el grupo 2 esta incidencia fue del 0% (IC 95%: 0-9%) (p 0,009). No hubo diferencias estadísticas entre las medidas de resultado informadas por los pacientes; todas las fracturas consolidaron. Conclusión: Los resultados de nuestro estudio demostrarían que el uso de placas LCP rectas de 4,5mm comparado a las placas anatómicas LCP de 3,5mm en MIPO posterior de húmero genera mayores molestias y, por lo tanto, conllevan un incremento en el riesgo de extracción del implante de un 18%.(AU)

Purpose: Posterior MIPO approach in the humerus has been described by using a 4.5mm LCP plate. Although straight plates have shown good results, they have not been designed to adapt to the distal humeral metaphysis. The goal of the study was to test the null hypothesis that there is no difference in hardware removal after posterior MIPO with either a straight or a pre-contoured plate. Methods: Patients older than 18 years, who had suffered mid-distal humeral shaft fracture, were treated by a posterior MIPO technique with a locking plate and had a minimum of 12-month follow-up were retrospectively included. Patients were separated into: group 1 (LCP 4.5mm straight plate); and group 2 (3.5mm anatomically shaped plate). Clinical and radiological evaluation were performed in the postoperative period. Patient-reported outcomes and the need of hardware removal because of pain were assessed. Results: Sixty-seven patients fulfilled the inclusion criteria. Twenty-seven patients in group 1 and 40 in group 2. No patient was lost to follow-up. There were no statistical differences between in patient reported outcomes measures. All the fractures healed. Within group 1, 18% (95%CI: 6-38%) of the patients required implant removal while in group 2 this incidence was 0% (95%CI: 0-9%) (P 0.009). Conclusion: These results suggest that the use of a 4.5mm LCP compared to an anatomical 3.5mm LCP in posterior MIPO of the humerus generates greater discomfort and therefore leads to a 18% increase in the risk of implant removal.(AU)

[Translated article] The use of an anatomical implant compared to a straight LCP decreases extraction in posterior humeral MIPO / La utilización de un implante anatómico comparado a una LCP recta disminuye su extracción en la osteosíntesis percutánea posterior del húmero

Purpose: Posterior MIPO approach in the humerus has been described by using a 4.5mm LCP plate. Although straight plates have shown good results, they have not been designed to adapt to the distal humeral metaphysis. The goal of the study was to test the null hypothesis that there is no difference in hardware removal after posterior MIPO with either a straight or a pre-contoured plate. Methods: Patients older than 18 years, who had suffered mid-distal humeral shaft fracture, were treated by a posterior MIPO technique with a locking plate and had a minimum of 12-month follow-up were retrospectively included. Patients were separated into: group 1 (LCP 4.5mm straight plate); and group 2 (3.5mm anatomically shaped plate). Clinical and radiological evaluations were performed in the postoperative period. Patient-reported outcomes and the need of hardware removal because of pain were assessed. Results: Sixty-seven patients fulfilled the inclusion criteria. Twenty-seven patients in group 1 and 40 in group 2. No patient was lost to follow-up. There were no statistical differences between in patient reported outcomes measures. All the fractures healed. Within group 1, 18% (95%CI: 6–38%) of the patients required implant removal while in group 2 this incidence was 0% (95%CI: 0–9%) (P 0.009). Conclusion: These results suggest that the use of a 4.5mm LCP compared to an anatomical 3.5mm LCP in posterior MIPO of the humerus generates greater discomfort and therefore leads to a 18% increase in the risk of implant removal.(AU)

Antecedentes y objetivo: El abordaje posterior percutáneo del húmero se ha descrito utilizando una placa LCP de 4,5mm. A pesar de que las placas rectas han demostrado buenos resultados, estas no han sido diseñadas para adaptarse a la metáfisis del húmero distal. El objetivo de este estudio es probar la hipótesis nula de que no existen diferencias en la extracción de la osteosíntesis después de una MIPO posterior utilizando una placa recta comparado a una placa anatómica. Materiales y métodos: Se incluyó retrospectivamente en 2 instituciones a pacientes mayores de 18 años que habían sufrido una fractura diafisaria de húmero distal tratados mediante técnica percutánea posterior con una placa bloqueada y con un seguimiento mínimo de 12 meses. Los pacientes se dividieron en: grupo 1 (placa recta LCP de 4,5mm) y grupo 2 (placa de forma anatómica de 3,5mm). Durante el postoperatorio se reportó la evaluación clínica y radiológica, así como la necesidad de retirar el implante debido al dolor. Resultados: Sesenta y siete pacientes cumplieron los criterios de inclusión. Veintisiete pacientes en el grupo 1 y 40 en el grupo 2. No se perdió ningún paciente durante el seguimiento. Dentro del grupo 1, el 18% (IC del 95%: 6-38%) de los pacientes requirieron extracción del implante, mientras que en el grupo 2 esta incidencia fue del 0% (IC del 95%: 0-9%) (p = 0,009). No hubo diferencias estadísticas entre las medidas de resultado informadas por los pacientes; todas las fracturas consolidaron. Conclusión: Los resultados de nuestro estudio demostrarían que el uso de placas LCP rectas de 4,5mm comparado a las placas anatómicas LCP de 3,5mm en MIPO posterior de húmero genera mayores molestias y, por lo tanto, conllevan un incremento en el riesgo de extracción del implante de un 18%.(AU)

Cutaneous malakoplakia arising on the ankle of a patient with pyoderma gangrenosum.

Malakoplakia is a rare chronic inflammatory condition that most commonly involves the urogenital tract. Cutaneous malakoplakia is extremely rare and many patients diagnosed with skin involvement are immunosuppressed. While the clinical presentation of cutaneous malakoplakia is variable, the histopathologic features are quite distinct and include sheets of closely packed dermal histiocytes with foamy-appearing cytoplasm and Michaelis-Gutmann bodies that are positive with certain immunohistochemical stains. While the exact pathogenesis of malakoplakia is unknown, it has been associated with certain bacterial infections. Treatment generally involves a combination of surgery and antimicrobial agents and/or modulation of immunosuppressant therapy if appropriate. Herein, the authors report a unique case of cutaneous malakoplakia arising in a patient on chronic immunosuppressive therapy for the management of pyoderma gangrenosum.

Hypertrophic Lichen Planus: An Up-to-Date Review and Differential Diagnosis.

CONTEXT.­: Hypertrophic lichen planus (HLP) is a variant of lichen planus that can be difficult to diagnose based on histopathologic features alone. Thus, patient clinical history and clinicopathologic correlation are essential considerations to make the correct diagnosis. OBJECTIVE.­: To discuss the clinical and histologic presentation of HLP and provide a thorough review of commonly encountered mimickers in the differential diagnosis. DATA SOURCES.­: Data were derived from a literature review, personal clinical and research experiences, and a review of cases in the archives of a tertiary care referral center. CONCLUSIONS.­: In general, HLP involves the lower extremities and is characterized by thickened, scaly nodules and plaques that are often pruritic and chronic in nature. HLP affects both males and females and is most common in adults 50 to 75 years of age. Unlike conventional lichen planus, HLP tends to have eosinophils and classically displays a lymphocytic infiltrate most concentrated around the tips of rete ridges. The differential diagnosis for HLP is broad and encompasses numerous entities in many different categories, including premalignant and malignant neoplasms, reactive squamoproliferative tumors, benign epidermal neoplasms, connective tissue disease, autoimmune bullous disease, infection, and drug-related reactions. Therefore, a high index of suspicion must be maintained to avoid a misdiagnosis and potential inappropriate treatments.

[Translated article] The use of an anatomical implant compared to a straight LCP decreases extraction in posterior humeral MIPO.

PURPOSE: Posterior MIPO approach in the humerus has been described by using a 4.5mm LCP plate. Although straight plates have shown good results, they have not been designed to adapt to the distal humeral metaphysis. The goal of the study was to test the null hypothesis that there is no difference in hardware removal after posterior MIPO with either a straight or a pre-contoured plate. METHODS: Patients older than 18 years, who had suffered mid-distal humeral shaft fracture, were treated by a posterior MIPO technique with a locking plate and had a minimum of 12-month follow-up were retrospectively included. Patients were separated into: group 1 (LCP 4.5mm straight plate); and group 2 (3.5mm anatomically shaped plate). Clinical and radiological evaluations were performed in the postoperative period. Patient-reported outcomes and the need of hardware removal because of pain were assessed. RESULTS: Sixty-seven patients fulfilled the inclusion criteria. Twenty-seven patients in group 1 and 40 in group 2. No patient was lost to follow-up. There were no statistical differences between in patient reported outcomes measures. All the fractures healed. Within group 1, 18% (95%CI: 6-38%) of the patients required implant removal while in group 2 this incidence was 0% (95%CI: 0-9%) (P 0.009). CONCLUSION: These results suggest that the use of a 4.5mm LCP compared to an anatomical 3.5mm LCP in posterior MIPO of the humerus generates greater discomfort and therefore leads to a 18% increase in the risk of implant removal.

Cutaneous vascular tumors: an updated review.

Cutaneous vascular tumors constitute a heterogeneous group of entities that share overlapping morphologic and immunohistochemical features, which can be diagnostically challenging for pathologists and dermatopathologists. Our understanding and knowledge of vascular neoplasms have improved over time, resulting in both a refinement of their classification by the International Society for the Study of Vascular Anomalies (ISSVA) and an improvement in the accurate diagnosis and clinical management of vascular neoplasms. This review article aims to summarize the updated clinical, histopathological, and immunohistochemical characteristics of cutaneous vascular tumors, as well as to highlight their associated genetic mutations. Such entities include infantile hemangioma, congenital hemangioma, tufted angioma, spindle cell hemangioma, epithelioid hemangioma, pyogenic granuloma, Kaposiform hemangioendothelioma, retiform hemangioendothelioma, pseudomyogenic hemangioendothelioma, Kaposi sarcoma, angiosarcoma, and epithelioid hemangioendothelioma.

The use of an anatomical implant compared to a straight LCP decreases extraction in posterior humeral MIPO. / La utilización de un implante anatómico comparado a una LCP recta disminuye su extracción en la osteosíntesis percutánea posterior del húmero.

PURPOSE: Posterior MIPO approach in the humerus has been described by using a 4.5mm LCP plate. Although straight plates have shown good results, they have not been designed to adapt to the distal humeral metaphysis. The goal of the study was to test the null hypothesis that there is no difference in hardware removal after posterior MIPO with either a straight or a pre-contoured plate. METHODS: Patients older than 18 years, who had suffered mid-distal humeral shaft fracture, were treated by a posterior MIPO technique with a locking plate and had a minimum of 12-month follow-up were retrospectively included. Patients were separated into: group 1 (LCP 4.5mm straight plate); and group 2 (3.5mm anatomically shaped plate). Clinical and radiological evaluation were performed in the postoperative period. Patient-reported outcomes and the need of hardware removal because of pain were assessed. RESULTS: Sixty-seven patients fulfilled the inclusion criteria. Twenty-seven patients in group 1 and 40 in group 2. No patient was lost to follow-up. There were no statistical differences between in patient reported outcomes measures. All the fractures healed. Within group 1, 18% (95%CI: 6-38%) of the patients required implant removal while in group 2 this incidence was 0% (95%CI: 0-9%) (P 0.009). CONCLUSION: These results suggest that the use of a 4.5mm LCP compared to an anatomical 3.5mm LCP in posterior MIPO of the humerus generates greater discomfort and therefore leads to a 18% increase in the risk of implant removal.

Mesenchymal tumours with melanocytic expression: a potential pitfall in the differential diagnosis of malignant melanoma.

Mesenchymal tumours with melanocytic expression can pose a diagnostic challenge because they frequently demonstrate both morphological and immunohistochemical overlap with other cutaneous melanocytic neoplasms. Therefore, they present potential pathological pitfalls that may lead to a misdiagnosis of malignant melanoma. Mesenchymal neoplasms that closely mimic melanoma include malignant melanotic nerve sheath tumour (melanotic schwannoma), epithelioid schwannoma, malignant peripheral nerve sheath, cutaneous syncytial myoepithelioma, clear cell sarcoma of soft tissue, and perivascular epithelioid cell tumour. Awareness of these melanoma mimics is necessary for establishing the correct diagnosis so that the appropriate clinical management can be rendered to the patient. This in-depth review highlights key diagnostic features and molecular genetics and also discusses the differential diagnosis and treatment of mesenchymal tumours that exhibit melanocytic expression.

Delusions of parasitosis and delusions of parasitosis by proxy in a mother and her children.

Delusions of parasitosis by proxy is an uncommon entity wherein an individual projects a delusional belief onto another person who lacks capacity to hold the same belief. We report a case of delusions of parasitosis by proxy in a mother who believed that she was infested with scabies and projected her fixed, delusional belief of scabies infestation onto her children. She subjected her children to numerous home remedies and medical treatments as well as removing them from school in an effort to cure them of the supposed infestation. Child maltreatment can be a concern in such cases with a low threshold for involving child protective services if harm to the children is suspected.

Nevus Sebaceus Arising Within a Scalp Whorl of a Healthy Male Neonate.

Nevus sebaceus (NS) and scalp whorl are both benign congenital findings that have not previously been reported to occur simultaneously. In most cases, the isolated finding of a single, classic-appearing NS or a single hair whorl can be followed clinically with observation. However, the number of lesions, distribution, and size of NS along with atypical placement of a scalp hair whorl can indicate an underlying syndrome or even underlying cranial abnormalities. We present a unique case of NS arising within a hair whorl on the vertex scalp of an otherwise healthy male neonate. After ultrasound showed no vascular malformations or proliferations and no cranial extension at the site, the lesion was later treated with surgical excision at six months old per the parents' preference, thus allowing for histologic confirmation of NS. Additionally, we discuss herein the diagnostic implications, recommendations for work-up, and treatment options of NS.

Asymptomatic Plantar Nodules in a Toddler.

Precalcaneal congenital fibrolipomatous hamartoma (PCFH) is a rare benign skin lesion that typically presents at birth, or within the first several years of life, as single or multiple asymptomatic skin-colored papules or nodules on the plantar heels. We present a classic case of PCFH in a 3-year-old child. This uncommon entity has no reported malignant features or malignant transformations. We demonstrate how this diagnosis can be made clinically without subjecting pediatric patients to potentially painful, traumatizing, costly skin biopsies and unnecessary imaging.

A review of simulation experiment techniques used to analyze wildfire effects on water quality and supply.

This review addresses the critical knowledge gap of techniques simulating combustion and heating characteristics present in natural wildfires and their use in assessing postfire impacts on water quality and quantity. Our assessment includes both laboratory and plot-scale techniques with burn and rainfall simulation components. Studies included focus on advancing understanding of changes in chemical and physical properties of soil, as well as subsequent runoff changes. Advantages of simulation experiments include: overcoming logistical challenges of collecting in situ wildfire data, reducing the high spatial variability observed in natural settings (i.e., the heterogeneity of burn intensity and the underlying vegetation and soil properties), and controlling the magnitude of key drivers of wildfire impacts. In sum, simulation experiments allow for more direct attribution of water quality and quantity responses to specific drivers than experiments conducted in situ. Drawbacks of simulation techniques include the limitation of observing only local-scale processes, the potential misrepresentation of natural settings (i.e., lack of spatial variability in vegetation, soil structure, burn intensity, etc.), uncertainty introduced through experimental error, and subsequent challenges in upscaling results to larger scales relevant for water management. This review focuses primarily on simulation techniques, with the goal of providing a foundation of knowledge for the design of future simulation experiments.

Patient perspectives following initiation of elexacaftor-tezacaftor-ivacaftor in people with cystic fibrosis and advanced lung disease.

BACKGOUND: Elexacaftor-tezacaftor-ivacaftor partially restores cystic fibrosis transmembrane conductance regulator function, and has been shown to induce significant clinical improvement in patients with at least one Phe508del allele. Yet little data exist on patient perspectives following elexacaftor-tezacaftor-ivacaftor initiation. METHODS: A mixed methods study was conducted using an online 13-item questionnaire (including 9 closed questions and 4 open questions), submitted from July 10th to August 21th 2020 to French patients aged 12 years and older with advanced CF who were treated with elexacaftor-tezacaftor-ivacaftor. Their responses were summarized as numbers (%), and free-text items were analysed using a grounded theory approach. RESULTS: Of 245 patients who started elexacaftor-tezacaftor-ivacaftor in France, 101 (41%) participated. Median [IQR] age was 35 [28-41] years and duration of elexacaftor-tezacaftor-ivacaftor treatment was 4.3 [3.0-5.6] months. Patients generally reported a rapid impact on respiratory symptoms, sleep quality, general well-being and physical self-esteem, and a reduction in overall treatment burden. The majority of patients contrasted treatment burden, symptom severity, depression and a closed future marked by death or transplantation before elexacaftor-tezacaftor-ivacaftor, to renewed and unexpected physical strength, leading to greater self-confidence, autonomy and long-term planning, after treatment initiation. A small number of patients expressed concerns, mainly regarding changes in body representation and/or the fear of becoming dependent on the treatment. CONCLUSION: After initiation of elexacaftor-tezacaftor-ivacaftor, CF patients with advanced disease reported rapid and positive physical, psychological and social effects, which translated into improved quality of life and the formulation of new life goals.

Galeazzi fracture-dislocations: Long-term prognosis of the distal radioulnar joint.

The aim of the present study was to evaluate long-term functional and radiographic outcome in the distal radioulnar joint (DRUJ) for Galeazzi fracture-dislocation after anatomic reduction and rigid fixation of the radius. Fourteen patients, with an average age of 38 years, presenting with Galeazzi fracture-dislocation treated by open reduction and internal fixation (ORIF) of the radius and closed reduction of the DRUJ were retrospectively evaluated, with a minimum follow-up of 6 years. At final evaluation (mean: 8 years), the DRUJ was objectively and subjectively evaluated for range of motion (RoM), grip strength, ballottement test, pain on axial loading, function on visual analog scale (VAS) and DASH score. Radiographs and dynamic CT scans were performed to screen for DRUJ instability and/or osteoarthritis. Six of the patients had a positive comparative ballottement test, but none reported pain during the maneuver. No significant differences in RoM were found between the injured and uninjured wrist. Mean grip strength in the injured wrist was 77% of the contralateral value. Mean pain on VAS was 0.6. Mean global function on VAS was 9. Mean DASH score was 3. Dynamic CT showed no clear subluxation in any of the patients, and none showed severe articular changes. Our findings suggest that long-term clinical and radiological prognosis for the DRUJ in Galeazzi lesions is favorable when adequate closed reduction of the ulna is achieved after anatomical ORIF of the radius. LEVEL OF EVIDENCE: IV. Therapeutic case series.