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1.
JBMR Plus ; 8(8): ziae069, 2024 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-38957400

RESUMO

This retrospective study investigates the prevalence of atypical femoral fractures (AFFs) among patients admitted with hip and shaft fractures at a tertiary referral center in Beirut, Lebanon. We analyzed electronic medical records and radiology studies of patients aged above 40 admitted with hip and shaft fractures between January 2006 and December 2019. Fractures were confirmed by ICD9 or ICD10 codes. All cases were reviewed by radiologists, and AFFs were identified according to the 2013 revised ASBMR criteria. We identified 1366 hip and shaft fracture patients, of which 14 female patients had 19 AFFs. This represents a prevalence of 1.0% among all hip and shaft fractures patients and 1.7% among all female hip and shaft fracture patients. Bilateral AFFs were found in 5 of the 14 patients. Patients with AFF tended to be younger, with a mean age of 74.3 (±8.6) yr compared to 78.0 (±10.6) for patients with non-AFF fractures. A total of 36% of AFF patients had a prior history of non-traumatic fracture at first admission. A high percentage of patients with AFFs reported intake of proton pump inhibitors (42.9%) and glucocorticoids (21.4%). Bisphosphonate exposure was noted in 64.3% of AFF patients. None of the AFF patients were active smokers or consumed alcohol regularly. BMD assessments were available for 7 AFF patients, indicating osteoporosis in 4 and osteopenia in 3 cases. Hip axis length measurements showed no significant difference between AFF patients (N = 7) and sex and age-matched controls (N = 21). The study underlines the prevalence and characteristics of AFFs in Lebanon, which is consistent with the numbers reported in the literature (0.32%-5%). A larger prospective study that includes hospitals across the nation is needed to gain a more comprehensive view of the prevalence of AFFs in the Lebanese population.

2.
Skeletal Radiol ; 53(3): 419-436, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-37589755

RESUMO

At some institutions, musculoskeletal and general radiologists rather than neuroradiologists are responsible for reading magnetic resonance imaging (MRI) of the spine. However, neurological findings, especially intrathecal ones, can be challenging. Intrathecal neurological findings in the spine can be classified by location (epidural, intradural extramedullary, and intramedullary) or etiology (tumor, infection, inflammatory, congenital). In this paper, we provide a succinct review of the intrathecal neurological findings that can be seen on MRI of the spine, primarily by location and secondarily by etiology, in order that this may serve as a helpful guide for musculoskeletal and general radiologists when encountering intrathecal neurological pathologies.


Assuntos
Imageamento por Ressonância Magnética , Coluna Vertebral , Humanos , Coluna Vertebral/diagnóstico por imagem , Coluna Vertebral/patologia , Imageamento por Ressonância Magnética/métodos
3.
Emerg Radiol ; 30(6): 699-709, 2023 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-37851147

RESUMO

PURPOSE: (1) Describe imaging utilization and findings within two weeks of the 2020 Beirut blast according to the mechanism of injury, (2) determine the appropriate imaging modality per organ/system, and (3) describe changes in the workflow of a radiology department to deal with massive crises. MATERIALS AND METHODS: Two hundred sixty patients presented to the largest emergency department in Beirut and underwent imaging within 2 weeks of the blast. In this retrospective study, patients were divided into early (1) and late (2) imaging groups. Patients' demographic, outcome, type and time of imaging studies, body parts imaged, and mechanism and types of injuries were documented. RESULTS: Two hundred five patients in group 1 underwent 502 and 55 patients in group 2 underwent 145 imaging studies. Tertiary blast injuries from direct impact and falling objects were the most common type of injuries followed by secondary (shrapnel) injuries. Both types of injuries affected mostly the head and neck and upper extremities. Plain radiographs were adequate for the extremities and CT for the head and neck. A regularly updated and practiced emergency plan is essential to mobilize staff and equipment and efficiently deliver radiology services during crises. CONCLUSION: Because the powerful Beirut blast occurred at the port located in the periphery of the city, most injuries seen on imaging were of the upper extremities and head and neck caused by the severe blast wind or penetrating shrapnel and resulted from people using their arms to protect their heads and bodies from direct impact and falling objects.


Assuntos
Traumatismos por Explosões , Traumatismo Múltiplo , Humanos , Estudos Retrospectivos , Tomografia Computadorizada por Raios X/métodos , Traumatismos por Explosões/diagnóstico por imagem , Pescoço
4.
J Vasc Bras ; 21: e20210213, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-36544985

RESUMO

The prevalence of coronary artery anomalies has been increasing due to the increasing usage of coronary angiography. There is a paucity of literature concerning management of viral-induced myocarditis in patients with anomalous coronary artery. We present a very unusual case of a 44-year-old man with anomalous origin of the left circumflex artery from the proximal ostium of the right coronary artery who was admitted for COVID-19-induced myocarditis. He presented with signs of heart failure and coronary angiography revealed the left circumflex artery with a separate ostium originating from the proximal right coronary artery. He was treated medically with Bisoprolol, Perindopril Arginine, Rivaroxaban, and Furosemide. His condition improved rapidly and he resumed regular life within 1 month. Coexistence of cardiac disease such as viral-induced myocarditis with an underlying anomalous origin of the coronary artery is challenging to spot and can lead to worse outcomes in case of misdiagnosis and inaccurate management.


A prevalência de anomalias da artéria coronária vem aumentando devido ao uso crescente da angiografia coronariana. Há uma escassez de literatura sobre o manejo da miocardite induzida por vírus no contexto de artéria coronária anômala. Apresentamos um caso incomum de um homem de 44 anos com origem anômala da artéria circunflexa esquerda do óstio proximal da artéria coronária direita admitido por miocardite induzida por COVID-19. O paciente apresentava sinais de insuficiência cardíaca, e a cineangiocoronariografia revelou artéria circunflexa esquerda de óstio separado originando-se da artéria coronária direita proximal. Ele foi tratado clinicamente com bisoprolol, perindopril arginina, rivaroxabana e furosemida. Sua condição melhorou rapidamente, e o paciente recuperou uma vida normal em 1 mês. A coexistência de doença cardíaca, como miocardite induzida por vírus com uma origem anômala subjacente da artéria coronária, é difícil de detectar e pode levar a resultados piores em caso de diagnóstico incorreto e manejo impreciso.

5.
Radiol Case Rep ; 17(4): 1293-1299, 2022 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-35242255

RESUMO

Snapping of the biceps femoris tendon is a rare cause of lateral knee pain often due to multiple factors including fibular head deformity and thickening of the anterior band of the biceps femoris tendon inserting on the tibia. Understanding the complex and poorly understood anatomy of the biceps femoris tendon is crucial and essential for proper diagnosis of its snapping. In this report, we describe the rare entity of bilateral biceps femoris tendon snapping in a young man using a multimodality diagnostic approach.

6.
J Pediatr Orthop B ; 31(2): e258-e263, 2022 Mar 01.
Artigo em Inglês | MEDLINE | ID: mdl-34101677

RESUMO

Expandable endoprostheses provide a limb salvage option for skeletally immature patients with bone sarcoma of the lower extremities. Initial reports of the Repiphysis prosthesis were encouraging; however, medium-term follow-up revealed high complication rates. We report on the long-term follow-up of a cohort of patients treated with the Repiphysis prosthesis. Eleven patients were included in the study. Data collected included sex, age at surgery, duration of follow-up, site of disease, histologic diagnosis, number of lengthening sessions, amount lengthened, postoperative complications, endoprosthetic failure, mode of endoprosthetic failure, duration from index surgery to failure and to revision, type of revision surgery and final limb-length discrepancy. The average duration of follow-up from the time of surgery was 180 months (range, 144-215 months). Fifteen Repiphysis implants were used in 11 patients. All implants failed with an average time from surgery to failure of 36 months (range, 3-72 months). Twenty-four complications were observed: one wound dehiscence, two deep infections, 18 mechanical failures, implant collapse with destruction of proximal tibia epiphysis in two and one periprosthetic proximal femur fracture with dislodgement of the stem. Despite being an option for limb salvage, the Repiphysis prosthesis has a high rate of mechanical failure and need for revision, similar to other expandable implants. The authors, therefore, recommend full disclosure of the potential short- and long-term complications and need for revision, as well as alternative treatment options if their use is considered. Level of evidence: IV (Therapeutic).


Assuntos
Neoplasias Ósseas , Neoplasias Femorais , Sarcoma , Neoplasias Ósseas/diagnóstico por imagem , Neoplasias Ósseas/cirurgia , Criança , Neoplasias Femorais/cirurgia , Seguimentos , Humanos , Salvamento de Membro , Extremidade Inferior , Desenho de Prótese , Falha de Prótese , Implantação de Prótese , Estudos Retrospectivos , Sarcoma/diagnóstico por imagem , Sarcoma/cirurgia , Resultado do Tratamento
7.
J. vasc. bras ; 21: e20210213, 2022. graf
Artigo em Inglês | LILACS-Express | LILACS | ID: biblio-1422028

RESUMO

Abstract The prevalence of coronary artery anomalies has been increasing due to the increasing usage of coronary angiography. There is a paucity of literature concerning management of viral-induced myocarditis in patients with anomalous coronary artery. We present a very unusual case of a 44-year-old man with anomalous origin of the left circumflex artery from the proximal ostium of the right coronary artery who was admitted for COVID-19-induced myocarditis. He presented with signs of heart failure and coronary angiography revealed the left circumflex artery with a separate ostium originating from the proximal right coronary artery. He was treated medically with Bisoprolol, Perindopril Arginine, Rivaroxaban, and Furosemide. His condition improved rapidly and he resumed regular life within 1 month. Coexistence of cardiac disease such as viral-induced myocarditis with an underlying anomalous origin of the coronary artery is challenging to spot and can lead to worse outcomes in case of misdiagnosis and inaccurate management.


Resumo A prevalência de anomalias da artéria coronária vem aumentando devido ao uso crescente da angiografia coronariana. Há uma escassez de literatura sobre o manejo da miocardite induzida por vírus no contexto de artéria coronária anômala. Apresentamos um caso incomum de um homem de 44 anos com origem anômala da artéria circunflexa esquerda do óstio proximal da artéria coronária direita admitido por miocardite induzida por COVID-19. O paciente apresentava sinais de insuficiência cardíaca, e a cineangiocoronariografia revelou artéria circunflexa esquerda de óstio separado originando-se da artéria coronária direita proximal. Ele foi tratado clinicamente com bisoprolol, perindopril arginina, rivaroxabana e furosemida. Sua condição melhorou rapidamente, e o paciente recuperou uma vida normal em 1 mês. A coexistência de doença cardíaca, como miocardite induzida por vírus com uma origem anômala subjacente da artéria coronária, é difícil de detectar e pode levar a resultados piores em caso de diagnóstico incorreto e manejo impreciso.

8.
Oncol Lett ; 21(5): 354, 2021 May.
Artigo em Inglês | MEDLINE | ID: mdl-33747211

RESUMO

Extraskeletal Ewing sarcoma (EES) is a relatively uncommon primary tumor of the soft tissues, which accounts for 20-30% of all reported cases of ES. Being uncommon, all members of the ES family tumors are treated following the same general protocol of sarcoma tumors. The present review summarizes the diagnosis, management and prognosis of EES, focusing on the differences between the subtypes of ESS. The clinical features and imaging of EES are also discussed. Magnetic resonance imaging is the modality of choice for diagnostic imaging and local staging, while core-needle biopsy with pathological testing is used to obtain a definitive diagnosis. Although several oncology groups endorse that ES family of tumors should be treated with similar algorithm and protocols, some studies have demonstrated that surgery and radiotherapy may be used as a form of local control. However, further studies are required to conclude the optimum treatment option for EES.

9.
J Magn Reson Imaging ; 54(3): 904-909, 2021 09.
Artigo em Inglês | MEDLINE | ID: mdl-33644967

RESUMO

BACKGROUND: Prior imaging studies characterizing lumbar arachnoiditis have been based on small sample numbers and have reported inconsistent results. PURPOSE: To review the different imaging patterns of lumbosacral arachnoiditis, their significance, and clinical implications. STUDY TYPE: Retrospective. POPULATION: A total of 96 patients (43 women; average age 61.3 years) with imaging findings of arachnoiditis (postsurgical: N = 49; degenerative: N = 29; vertebral fracture: N = 6; epidural and subdural hemorrhage: N = 3, infectious: N= 1; other: N = 8) from January 2009 to April 2018. FIELD STRENGTH/SEQUENCE: Sagittal and axial T2-weighted Turbo Spin Echo at 1.5 T and 3 T. ASSESSMENT: Chart review was performed to assess the cause of arachnoiditis, and imaging was reviewed by two musculoskeletal and three neurology radiologists, blinded to the clinical data and to each other's imaging interpretation. Previous classification included a three-group system based on the appearance of the nerve roots on T2-weighted images. A fourth group was added in our review as "nonspecified" and was proposed for indeterminate imaging findings that did not fall into the classical groups. The presence/absence of synechiae/fibrous bands that distort the nerve roots and of spinal canal stenosis was also assessed. STATISTICAL TESTS: The kappa score was used to assess agreement between readers for both classification type and presence/absence of synechiae. RESULTS: Postsurgical (51%) and degenerative changes (30%) were the most common etiologies. About 7%-55% of arachnoiditis were classified as group 4. There was very poor classification agreement between readers (kappa score 0.051). There was also poor interreader agreement for determining the presence of synechiae (kappa 0.18) with, however, strong interreader agreement for the presence of synechia obtained between the most experienced readers (kappa 0.89). DATA CONCLUSION: This study demonstrated the lack of consensus and clarity in the classification system of lumbar arachnoiditis. The presence of synechia has high interreader agreement only among most experienced readers and promises to be a useful tool in assessing arachnoiditis. EVIDENCE LEVEL: 3 TECHNICAL EFFICACY: Stage 2.


Assuntos
Aracnoidite , Aracnoidite/diagnóstico por imagem , Feminino , Humanos , Vértebras Lombares , Imageamento por Ressonância Magnética , Pessoa de Meia-Idade , Reprodutibilidade dos Testes , Estudos Retrospectivos
10.
Pediatr Blood Cancer ; 68(1): e28784, 2021 01.
Artigo em Inglês | MEDLINE | ID: mdl-33128443

RESUMO

BACKGROUND: Patients with sickle cell disease are at higher risk of infections with encapsulated bacteria due to immature immune responses and functional asplenia. We aimed to study our patient population for the emergence of gram-negative organisms other than Salmonella as the cause of osteomyelitis and document a vast decrease in Streptococcus pneumoniae bacteremia rates. METHODS: We conducted a retrospective chart review of 158 patients with sickle cell disease registered at our hospital. Over a period of 13 years, every patient presenting to the emergency department (ED) with fever had their medical record reviewed for blood cultures, wound cultures, and magnetic resonance imaging results for osteomyelitis. RESULTS: The number of patients presenting to the ED with fever was 105, with 581 febrile episodes and 893 blood cultures. Among those, no culture grew Streptococcus pneumoniae, 14 grew coagulase-negative staphylococci (1.5%), one grew Salmonella enterica Paratyphi B, and three grew Salmonella enterica group C (in the same patient). The total number of osteomyelitis episodes in patients with sickle cell disease presenting with fever and documented by imaging was nine (1.5%). In patients with osteomyelitis, organisms were isolated in four patients (44%), including Enterobacter cloacae, Bacteroides, Pseudomonas aeruginosa, and Salmonella enterica group C. CONCLUSIONS: Immunization against Streptococcus pneumoniae and the use of prophylactic penicillin has virtually eliminated pneumococcal bacteremia among our patients. We observed the emergence of gram-negative organisms other than Salmonella as the cause of osteomyelitis in patients with sickle cell disease.


Assuntos
Anemia Falciforme/epidemiologia , Bactérias Gram-Negativas/patogenicidade , Infecções por Bactérias Gram-Negativas/complicações , Hospitalização/estatística & dados numéricos , Osteomielite/epidemiologia , Adolescente , Anemia Falciforme/patologia , Anemia Falciforme/virologia , Criança , Pré-Escolar , Feminino , Seguimentos , Infecções por Bactérias Gram-Negativas/virologia , Humanos , Incidência , Lactente , Recém-Nascido , Líbano/epidemiologia , Masculino , Osteomielite/patologia , Osteomielite/virologia , Prognóstico , Estudos Retrospectivos
12.
Eur J Radiol ; 124: 108855, 2020 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-32018075

RESUMO

PURPOSE: To assess the value of using Apparent Diffusion Coefficient (ADC) mapping in Diffusion Weighted Imaging (DWI) when monitoring treatment response in pediatric Osteogenic and Ewing sarcomas and to correlate with level of necrosis on post-surgical excision pathology. METHOD: This retrospective study includes 7 Osteosarcoma and 8 Ewing sarcoma patients. Pre-treatment and post-treatment focal MRIs were evaluated for ADC values, tumor volumes and variability of both measurements. We also compared the measurement between Ewing and Osteosarcoma groups, as well as between good (=/>90 % necrosis post-excision) and poor (<90 % necrosis post-excision) responders. RESULTS: All measurements except Maximum ADC (p = 0.20) showed a statistically significant difference when comparing them before and after treatment. When we segregated our population according to pathologic complete response, there was no difference in ADC measurements, volumetric measurements or either variability between good (8 Patients) and poor responders (7 Patients). When comparing the before-after changes in our measurement between the Ewing sarcoma and Osteosarcoma cases, there was no significant difference in the change between pre and post treatment (Δ) Mean or Maximum ADC, or in Δtumor-volume when measured on STIR or SPIR T1 post-contrast sequences. Only the ΔMinimum-ADC showed a statistically significant difference (p < 0.02) in this group. CONCLUSIONS: ADC can potentially reflect cellular changes associated with chemotherapy use, reflecting a response to treatment. However, quantitative use of those parameters to dictate a change in management, treatment regimen or chemotherapy dose in order to target a good response (>/ = 90 % necrosis post-excision) needs further investigation.


Assuntos
Neoplasias Ósseas/diagnóstico por imagem , Imagem de Difusão por Ressonância Magnética/métodos , Osteossarcoma/diagnóstico por imagem , Sarcoma de Ewing/diagnóstico por imagem , Adolescente , Neoplasias Ósseas/tratamento farmacológico , Criança , Feminino , Humanos , Masculino , Osteossarcoma/tratamento farmacológico , Estudos Retrospectivos , Sarcoma de Ewing/tratamento farmacológico , Resultado do Tratamento
13.
Artigo em Inglês | MEDLINE | ID: mdl-30704897

RESUMO

Epithelioid osteoblastoma is a clinically aggressive subtype of osteoblastoma that favors the mandible and the maxilla. Its histologic features lie on a spectrum between conventional osteoblastoma and low-grade osteosarcoma, thus making it difficult at times to confirm the diagnosis. It is known to have a high risk of recurrence after surgical resection, but it is a benign entity and does not have the propensity to metastasize. To our knowledge, there are no published reports on findings of epithelioid osteoblastoma on positron emission tomography/computed tomography (PET/CT). We report a case of a 25-year-old male patient with a diagnosis of epithelioid osteoblastoma of the mandible. The lesion exhibited significantly increased fluorodeoxyglucose uptake on PET/CT with a maximum standardized uptake value of 5.5. PET/CT is not specific in differentiating between malignant and benign bone lesions but may be necessary to rule out distant lesions when a confirmed diagnosis of epithelioid osteoblastoma cannot be obtained through histologic examination.


Assuntos
Neoplasias Mandibulares/diagnóstico por imagem , Osteoblastoma/diagnóstico por imagem , Adulto , Diagnóstico Diferencial , Fluordesoxiglucose F18 , Humanos , Masculino , Mandíbula , Recidiva Local de Neoplasia , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada
14.
CEN Case Rep ; 7(2): 316-319, 2018 11.
Artigo em Inglês | MEDLINE | ID: mdl-29978297

RESUMO

Renal cell carcinoma (RCC) is a tumor that usually metastasizes to lung, liver, bone and brain, but rarely to skeletal muscles. We report a case of an elderly man with a history of bilateral metachronous RCC for which he underwent curative bilateral nephrectomies and renal transplantation, was in remission, and presented with a large solitary skeletal muscle metastasis from the initial RCC, 3 years later.


Assuntos
Carcinoma de Células Renais/patologia , Neoplasias Renais/patologia , Perna (Membro)/patologia , Músculo Esquelético/patologia , Idoso , Carcinoma de Células Renais/diagnóstico por imagem , Carcinoma de Células Renais/secundário , Carcinoma de Células Renais/cirurgia , Imagem de Difusão por Ressonância Magnética/métodos , Humanos , Neoplasias Renais/secundário , Transplante de Rim/métodos , Perna (Membro)/diagnóstico por imagem , Perna (Membro)/cirurgia , Masculino , Músculo Esquelético/cirurgia , Metástase Neoplásica , Nefrectomia/métodos , Ultrassonografia/métodos
15.
Cardiovasc Res ; 114(12): 1605-1616, 2018 10 01.
Artigo em Inglês | MEDLINE | ID: mdl-29800268

RESUMO

Aims: During pregnancy, there is a significant increase in heart rate (HR) potentially associated with an increased risk of arrhythmias or exacerbation of pre-existing cardiac conditions endangering both mother and foetus. Calcium homeostasis plays an important role in regulating automaticity of the sinoatrial node (SAN); however, its contribution to the accelerated HR during pregnancy remains unknown. Methods and results: Using murine SAN cells, we showed that pregnancy increased L-type Ca2+ current (ICaL) and CaV1.3 mRNA expression, whereas T-type Ca2+ current (ICaT) and its underlying channel were unchanged. Analysis of SAN intra-cellular Ca2+ oscillations showed that the rate of spontaneous Ca2+ transients was significantly higher in pregnant mice along with a higher mRNA expression of ryanodine receptor. Assessment of supra-ventricular arrhythmias using programmed electrical stimulation protocols on anaesthetized mice revealed higher susceptibility in pregnancy. Of note, the modifications associated with pregnancy were reversible following delivery. Furthermore, chronic administration of 17ß-estradiol (E2) to nodal-like human-induced pluripotent stem cell-derived cardiomyocytes (N-hiPSC-CM), control mice, oestrogen-receptor-ß knockout (ERKOß) but not ERKOα mice, accelerated cardiac automaticity, recapitulating the pregnancy phenotype in both mouse and human SAN cell models. Conclusion: Together, these results indicate that pregnancy considerably alters intra-cellular Ca2+ homeostasis sustaining faster HR during pregnancy. Importantly, these changes were dependent on an oestrogen receptor α (ERα) mechanism that resulted in increased ICaL and spontaneous Ca2+ release from the sarcoplasmic reticulum, highlighting a novel role for oestrogen in regulating HR.


Assuntos
Arritmias Cardíacas/metabolismo , Relógios Biológicos , Sinalização do Cálcio , Cálcio/metabolismo , Frequência Cardíaca , Complicações Cardiovasculares na Gravidez/metabolismo , Nó Sinoatrial/metabolismo , Animais , Arritmias Cardíacas/genética , Arritmias Cardíacas/fisiopatologia , Arritmias Cardíacas/prevenção & controle , Relógios Biológicos/efeitos dos fármacos , Canais de Cálcio Tipo L/genética , Canais de Cálcio Tipo L/metabolismo , Sinalização do Cálcio/efeitos dos fármacos , Linhagem Celular , Estradiol/farmacologia , Receptor alfa de Estrogênio/genética , Receptor alfa de Estrogênio/metabolismo , Receptor beta de Estrogênio/genética , Receptor beta de Estrogênio/metabolismo , Feminino , Frequência Cardíaca/efeitos dos fármacos , Homeostase , Humanos , Células-Tronco Pluripotentes Induzidas/metabolismo , Potenciais da Membrana , Camundongos Endogâmicos C57BL , Camundongos Knockout , Miócitos Cardíacos/metabolismo , Gravidez , Complicações Cardiovasculares na Gravidez/genética , Complicações Cardiovasculares na Gravidez/fisiopatologia , Complicações Cardiovasculares na Gravidez/prevenção & controle , Canal de Liberação de Cálcio do Receptor de Rianodina/genética , Canal de Liberação de Cálcio do Receptor de Rianodina/metabolismo , Nó Sinoatrial/efeitos dos fármacos , Fatores de Tempo
16.
Sci Rep ; 8(1): 952, 2018 01 17.
Artigo em Inglês | MEDLINE | ID: mdl-29343862

RESUMO

Heart failure (HF) is associated with high mortality and affects men and women differently. The underlying mechanisms for these sex-related differences remain largely unexplored. Accordingly, using mice with cardiac-specific overexpression of the angiotensin II (ANGII) type 1 receptor (AT1R), we explored male-female differences in the manifestations of hypertrophy and HF. AT1R mice of both sexes feature electrical and Ca2+ handling alterations, systolic dysfunction, hypertrophy and develop HF. However, females had much higher mortality (21.0%) rate than males (5.5%). In females, AT1R stimulation leads to more pronounced eccentric hypertrophy (larger increase in LV mass/body weight ratio [+31%], in cell length [+27%], in LV internal end-diastolic [LVIDd, +34%] and systolic [LVIDs, +67%] diameter) and dilation (larger decrease in LV posterior wall thickness, +17%) than males. In addition, in female AT1R mice the cytosolic Ca2+ extrusion mechanisms were more severely compromised and were associated with a specific increased in Ca2+ sparks (by 187%) and evidence of SR Ca2+ leak. Altogether, these results suggest that female AT1R mice have more severe eccentric hypertrophy, dysfunction and compromised Ca2+ dynamics. These findings indicate that females are more susceptible to the adverse effects of AT1R stimulation than males favouring the development of HF and increased mortality.


Assuntos
Angiotensina II/metabolismo , Cardiomiopatia Dilatada/metabolismo , Cardiomiopatia Dilatada/mortalidade , Animais , Cálcio/metabolismo , Cardiomegalia/metabolismo , Diástole/fisiologia , Feminino , Coração/fisiologia , Insuficiência Cardíaca/metabolismo , Masculino , Camundongos , Camundongos Endogâmicos C57BL , Receptor Tipo 1 de Angiotensina/metabolismo , Retículo Sarcoplasmático/metabolismo , Função Ventricular Esquerda/fisiologia
17.
J Radiol Case Rep ; 12(8): 17-24, 2018 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-30651917

RESUMO

Bone metastasis to the hands and feet, known as acrometastasis, is a very rare finding and tends to be associated with extensive metastasis. We herein report the case of a 14-year-old girl known to have a history of successfully treated Ewing's sarcoma arising from the ribs, who presented with a pathologically proven isolated metastatic lesion to the talus 7 years after achieving clinical and radiologic remission. We describe the imaging findings on MRI, CT scan and PET-CT. To our knowledge, talar metastasis from Ewing's sarcoma has been previously reported only twice in the English literature. Noteworthy is the fact that one of the previously reported lesions was considered a skip metastasis, and the other was under-described in terms of primary and secondary tumor location and time to metastasis. In addition, the overall imaging findings were rather suggestive of a benign lesion, particularly on CT scan.


Assuntos
Neoplasias Ósseas/patologia , Neoplasias Ósseas/secundário , Costelas/patologia , Sarcoma de Ewing/patologia , Tálus/patologia , Adolescente , Neoplasias Ósseas/diagnóstico por imagem , Diagnóstico Diferencial , Feminino , Humanos , Imageamento por Ressonância Magnética , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada , Prognóstico , Sarcoma de Ewing/diagnóstico por imagem , Tálus/diagnóstico por imagem , Fatores de Tempo , Tomografia Computadorizada por Raios X
18.
Pediatr Blood Cancer ; 65(1)2018 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-28727257

RESUMO

Frequent surveillance imaging is routine practice for pediatric patients after cancer therapy. This retrospective study evaluated the follow-up of 301 children with extracranial tumors diagnosed between 2002 and 2012, at a tertiary pediatric cancer center in Beirut, Lebanon. Recurrence occurred in 15% of patients, at a median of 12 months after end of primary therapy. Outcome was not different comparing patients with recurrence detected via imaging surveillance versus clinically. False positive findings in 55 patients led to further interventions. These results raise important questions regarding benefit of current surveillance practices as standard care, especially in countries with limited resources.


Assuntos
Neoplasias de Cabeça e Pescoço/diagnóstico por imagem , Neoplasias de Cabeça e Pescoço/terapia , Adolescente , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Lactente , Líbano , Masculino , Estudos Retrospectivos , Centros de Atenção Terciária
19.
Circ Cardiovasc Genet ; 10(5)2017 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-29021306

RESUMO

BACKGROUND: Long-QT syndrome is a potentially fatal condition for which 30% of patients are without a genetically confirmed diagnosis. Rapid identification of causal mutations is thus a priority to avoid at-risk situations that can lead to fatal cardiac events. Massively parallel sequencing technologies are useful for the identification of sequence variants; however, electrophysiological testing of newly identified variants is crucial to demonstrate causality. Long-QT syndrome could, therefore, benefit from having a standardized platform for functional characterization of candidate variants in the physiological context of human cardiomyocytes. METHODS AND RESULTS: Using a variant in Kir2.1 (Gly52Val) revealed by whole-exome sequencing in a patient presenting with symptoms of long-QT syndrome as a proof of principle, we demonstrated that commercially available human induced pluripotent stem cell-derived cardiomyocytes are a powerful model for screening variants involved in genetic cardiac diseases. Immunohistochemistry experiments and whole-cell current recordings in human embryonic kidney cells expressing the wild-type or the mutant Kir2.1 demonstrated that Kir2.1-52V alters channel cellular trafficking and fails to form a functional channel. Using human induced pluripotent stem cell-derived cardiomyocytes, we not only confirmed these results but also further demonstrated that Kir2.1-52V is associated with a dramatic prolongation of action potential duration with evidence of arrhythmic activity, parameters which could not have been studied using human embryonic kidney cells. CONCLUSIONS: Our study confirms the pathogenicity of Kir2.1-52V in 1 patient with long-QT syndrome and also supports the use of isogenic human induced pluripotent stem cell-derived cardiomyocytes as a physiologically relevant model for the screening of variants of unknown function.


Assuntos
Células-Tronco Pluripotentes Induzidas/metabolismo , Síndrome do QT Longo , Modelos Biológicos , Mutação de Sentido Incorreto , Miócitos Cardíacos/metabolismo , Canais de Potássio Corretores do Fluxo de Internalização , Adulto , Substituição de Aminoácidos , Feminino , Células HEK293 , Humanos , Células-Tronco Pluripotentes Induzidas/patologia , Síndrome do QT Longo/genética , Síndrome do QT Longo/metabolismo , Síndrome do QT Longo/patologia , Miócitos Cardíacos/patologia , Canais de Potássio Corretores do Fluxo de Internalização/genética , Canais de Potássio Corretores do Fluxo de Internalização/metabolismo
20.
J Glob Oncol ; 3(1): 23-30, 2017 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-28717738

RESUMO

BACKGROUND: Children with malignant bone tumors have average 5-year survival rates of 60% to 70% with current multimodality therapy. Local control modalities aimed at preserving function greatly influence the quality of life of long-term survivors. In developing countries, the limited availability of multidisciplinary care and limited expertise in specialized surgery and pediatric radiation therapy, as well as financial cost, all form barriers to achieving optimal outcomes in this population. METHODS: We describe the establishment of a collaborative pediatric bone tumor program among a group of pediatric oncologists in Lebanon and Syria. This program provides access to specialized local control at a tertiary children's cancer center to pediatric patients with newly diagnosed bone tumors at participating sites. Central review of pathology, staging, and treatment planning is performed in a multidisciplinary tumor board setting. Patients receive chemotherapy at their respective centers on a unified treatment plan. Surgery and/or radiation therapy are performed centrally by specialized staff at the children's cancer center. Cost barriers were resolved through a program development initiative led by St Jude Children's Research Hospital. Once program feasibility was achieved, the Children's Cancer Center of Lebanon Foundation, via fundraising efforts, provided continuation of program-directed funding. RESULTS: Findings over a 3-year period showed the feasibility of this project, with timely local control and protocol adherence at eight collaborating centers. We report success in providing standard-of-care multidisciplinary therapy to this patient population with complex needs and financially challenging surgical procedures. CONCLUSION: This initiative can serve as a model, noting that facilitating access to specialized multidisciplinary care, resolution of financial barriers, and close administrative coordination all greatly contributed to the success of the program.

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