1,720,991 research outputs found
Hypertrophic osteoarthropathy mimicking a reactive arthritis: A case report and review of the literature
Full text linkBACKGROUND:
Hypertrophic osteoarthropathy (HOA) is a syndrome characterized by abnormal proliferation of skin and periosteal tissues of the extremities. It can be a rare hereditary disease (pachydermoperiostosis) or can be secondary to various diseases, though mostly lung malignancies. Here, we report an unusual clinical presentation of HOA.
CASE PRESENTATION:
A 77-year-old man presented with fever, diarrhea, and an oligoarthritis involving the left knee and the ankles. Since left knee synovial fluid aspiration revealed an aseptic synovitis and Clostridium Difficile toxin was detectable in stool samples, a reactive arthritis secondary to a Clostridium Difficile induced colitis was initially suspected. However, the presence of a worsened digital clubbing and the lack of a good clinical response to steroid therapy led us to perform a radionuclide bone scanning, which revealed HOA. This turned out to be associated with a lepidic predominant lung adenocarcinoma, which was clinically and radiologically difficult to distinguish from a relapse of pneumonia.
CONCLUSION:
Consistent with the literature, HOA tends to have a variable clinical presentation, mimicking that of various rheumatic diseases. This clinical case shows that HOA can present as a presumptive acute reactive arthritis, and it highlights the importance of patient’s follow-up in the differential diagnosis of inflammatory arthritis, especially when a worsened digital clubbing is present
Specific Therapy in Transthyretin Amyloid Cardiomyopathy: Future Perspectives Beyond Tafamidis
No full textTransthyretin amyloid cardiomyopathy (ATTR-CM) is a relatively prevalent cause of morbidity and mortality. Over the recent years, development of disease-modifying treatments has enabled stabilization of the circulating transthyretin tetramer and suppression of its hepatic production, resulting in a remarkable improvement in survival of patients with ATTR-CM. Second-generation drugs for silencing are currently under investigation in randomized clinical trials. In vivo gene editing of transthyretin has been achieving unanticipated suppression of hepatic production in ATTR-CM. Trials of antibodies inducing the active removal of transthyretin amyloid deposits in the heart are ongoing, and evidence has gathered for exceptional spontaneous regression of ATTR-CM
Light-chain cardiac amyloidosis: a case report of extraordinary sustained pathological response to cyclophosphamide, bortezomib, and dexamethasone combined therapy
Full text linkBACKGROUND: Heart involvement represents the most ominous prognostic factor in light-chain amyloidosis (AL), often foreclosing curative therapies such as high-dose chemotherapy followed by autologous stem cell transplantation (ASCT). Heart transplantation (HTx) may be considered before ASCT in rigorously selected cases of advanced AL cardiac amyloidosis (CA). In ASCT-ineligible patients, chemotherapy with cyclophosphamide, bortezomib, and dexamethasone combined (CyBorD) regimen, even at low-dose, is feasible and effective in obtaining hematological and organ response. CASE SUMMARY: A previously healthy 50-year-old woman presented with severely symptomatic new-onset heart with preserved ejection fraction, significant cardiac hypertrophy, and an ‘apical sparing’ pattern. Bone marrow and abdominal fat biopsy revealed AL amyloidosis due to a smouldering micromolecular λ-type myeloma with severe cardiac involvement, and the patient was judged a good candidate to HTx followed by ASCT. Despite fragile conditions, she tolerated a full course of low-dose combination therapy with bortezomib and was withdrawn from HTx list because of unexpected persistent complete hematologic response and major cardiac improvement. Disease remission was achieved in the long term (>3 years). DISCUSSION: We report a case of exceptional persistent hematologic and cardiac response after CyBorD therapy in a patient with advanced AL-CA who left the transplantation lists (both HTx and ASCT). In ASCT-ineligible patients, chemotherapy with CyBorD regimen, even at low-dose, can lead to durable remission of the disease with excellent cardiac response
Evaluation of a breast cancer nomogram for predicting the likelihood of additional nodal metastases in patients with a positive sentinel node biopsy
No full textAIM:Completion axillary lymph node dissection (CALND) performed as a standard procedure after a positive sentinel node biopsy (SLNB) in breast cancer patients results, in almost 40-70% of cases, in no additional positive nodes. A nomogram has been developed at Memorial Sloan Kettering Cancer Center (MSKCC) to predict the likelihood of nonsentinel node metastases (NSLNM) after a positive SLNB. Aim of study was to assess the accuracy of MSKCC nomogram in our community breast cancer population.
MATERIAL OF STUDY:
From a retrospective database of 276 breast cancer patients we evaluated 62 consecutive cases who underwent CALND after a positive SLNB. Patient and tumor characteristics were collected and the nomogram was used to calculate the probability of NSLNM. The accuracy of MSKCC nomogram was tested by the Receiver Operating Characteristic (ROC) curve. The Area Under the Curve (AUC), sensitivity and specificity were calculated for a 10% cut-off value.
RESULTS:
Presence of macrometastases (p=0.03) and its extranodal extension (p=0.013) in sentinel node were associated with NSLNM, while other tumor and patient characteristics were not. The accuracy of MSKCC nomogram as measured by AUC was 0.67. The nomogram showed 95% sensitivity and 14% specificity. We revised the nomogram by incorporating the presence of extranodal extension and we obtained a new test with improved specificity (84%).
DISCUSSION:
The modified predictive model is a useful tool in predicting the likelihood of NSLNM in our cohort of patients and may help decision regarding the need of completion axillary lymph node dissectio
Cellulosimicrobium cellulans aortic prosthetic valve endocarditis
No full textIntroduction: Invasive infections due to Cellulosimicrobium spp. (a Gram-positive coryneform) are extremely rare. Only a few cases of bloodstream infections and endocarditis have been described, as bacteraemia due to coryneforms is usually discarded as blood culture contamination.Case presentation: A 66-year-old female, with a history of aortic valve replacement, presented with fever, left leg purpura and acute kidney injury. Multiple repeated blood cultures were positive for Cellulosimicrobium cellulans , and targeted therapy was started. At first, endocarditis was excluded by echocardiograms, and the acute nephritis was interpreted as an atypical presentation of Henoch-Shonlein purpura. High-dose prednisone was started, and after 10 weeks the patient presented again with fever, mental confusion and acute left arm ischaemia. A subsequent echocardiogram and radiolabelled leukocyte scintigraphic evaluation revealed aortic prosthetic valve endocarditis with periprosthetic abscess and arterial brachial thrombosis. The patient deceased, and the autoptic examination confirmed an aortic valve periprosthetic abscess and revealed multiple arterial thromboses and septic embolisms in the kidneys, brain, spleen and myocardium.Conclusion: Isolation of coryneform bacteria on blood culture should not always be discarded as blood culture contamination. In the case of endocarditis due to Cellulosimicrobium spp., the removal of any prosthetic material, along with prolonged in vitro active antimicrobial therapy, should be pursued in order to reduce persistence or relapses of infection
Clinicopathologic factors predicting involvement of nonsentinel axillary lymphnodes in breast cancer patients: is axillary dissection always indicated?
No full textAIM: The aim of this study was to determine factors that predict non-sentinel axillary lymph nodes (NSLNs) metastases in breast cancer patients with positive sentinel node biopsy (SLNB). MATERIAL OF STUDY: We reviewed the records of a consecutive series of 176 breast cancer patients who underwent SLNB at our institution. From the database we analysed those cases with one or more positive sentinel lymph nodes (SLNs) in order to determine factors predicting NSLN metastases.
RESULTS: From a series of 176 consecutive patients, we evaluated 41 cases (23.3%) with positive SLNB. Subsequent completion axillary lymph node dissection (CALND) revealed NSLN metastases in 15 cases (36.6%). The significant variables predictive of NSLN involvement were the presence of macrometastases with extranodal extension (p = 0.048), the presence of more than one positive SLN (p = 0.08) and a ratio between positive SLN and SLNs globally dissected higher than 0.5 (p = 0.05).
DISCUSSION: CALND is the gold standard for patients with positive SLNB, but results, in almost 40-70% of cases, in no additional positive nodes and its therapeutic benefit remains controversial. Clinicopathologic factors predictive of NSLN metastases may be useful in identifying a subset of patients with lower risk of further axillary involvement.
CONCLUSIONS: In patients with early breast carcinoma and a positive SLNB, the size of SLN metastases, the presence of extranodal extension, more than one positive SLN and a nodal ratio higher than 0.5 are the factors that significantly increase the frequency of additional axillary positive lymph nodes
I dieci punti che il cardiologo deve conoscere su scintigrafia miocardica con traccianti ossei, amiloidosi e cuore
No full textL’amiloidosi cardiaca (AC), da sempre considerata una malattia rara ed incurabile, è stata oggetto, negli ultimi 10 anni, di una rivoluzione diagnostico-terapeutica. Da un lato la disponibilità di metodiche non invasive, tipicamente la scintigrafia miocardica con traccianti ossei, ha drasticamente cambiato l’epidemiologia della malattia, svelando la reale e crescente prevalenza della forma transtiretino-relata (ATTR), e modificato gli algoritmi diagnostici, riducendo il ricorso alla biopsia endomiocardica. Dall’altro, la disponibilità di nuove terapie specifiche, come il tafamidis, ne ha reso il riconoscimento precoce una necessità clinica e non uno sterile esercizio diagnostico. Da quando, nel 2016, Gillmore e collaboratori hanno proposto una nuova flow-chart diagnostica per la diagnosi differenziale fra ATTR e amiloidosi da catene leggere delle immunoglobuline (AL), attribuendo un ruolo dirimente alla scintigrafia con traccianti ossei, questa metodica è entrata nel vocabolario e nella pratica clinica del cardiologo impegnato nel campo delle cardiomiopatie. In questo articolo cercheremo di rispondere ai principali quesiti in tema di scintigrafia miocardica con marcatori ossei ed AC, offrendo al cardiologo le conoscenze utili per orientarsi in merito a richiesta, interpretazione e contestualizzazione clinica dell’esame
A case report of isolated cardiac light chain amyloidosis without clinically overt heart failure: an under-recognized presentation
Full text linkBackground: Cardiac involvement in amyloid light-chain (AL) amyloidosis usually represents a brick in the wall of a multi-system disease. The presence of cardiac deposition of free light chains (FLCs) is the main determinant of survival. Isolated cardiac AL is an uncommon scenario characterized by a challenging diagnostic and therapeutic workup.
Case summary: A 57-year-old asymptomatic man was presented for an incidental finding of myocardial necrosis at the electrocardiogram (ECG) performed for newly diagnosed arterial hypertension. Alongside signs of previous myocardial infarction, transthoracic echocardiography showed a severely increased left ventricular (LV) wall thickness not consistent with ECG voltages, segmental akinaesia with normal LV systolic function with 'apical sparing' pattern. Laboratory assessment showed an unexpectedly high level of natriuretic peptide and persistently abnormal troponin in the absence of symptoms or signs of heart failure or ongoing ischaemia. Coronary angiogram confirmed the coronary artery disease. Before revascularization, a complete diagnostic workup was carried. Serum electrophoresis detected a monoclonal gammopathy that was further investigated by serum immunofixation, revealing high lambda FLCs concentration. Fat pad, bone marrow, and salivary glands biopsies resulted negative for amyloid deposition. Finally, endomyocardial biopsy was consistent with AL amyloidosis. Urgent percutaneous revascularization was performed, and the patients was timely started on chemotherapy.
Discussion: The diagnosis of isolated cardiac AL amyloidosis is challenging and carries important therapeutic implications. As the short-term prognosis might be severely compromised, an accurate diagnostic flowchart has to be systematically pursued to obtain a precise diagnosis and address the optimal, tailored management
Clinical and diagnostic imaging of bisphosphonate-associated osteonecrosis ofthe jaws.
No full textIt is important to recognize osteonecrosis of the jaws in patients treated with bisphosphonates because an early diagnosis can make a significant difference to the outcome of the disease. The aim of this study is to describe the radiological features of bisphosphonate osteonecrosis (BON) in order to aid its prompt recognition. METHODS: A conventional radiograph, a computed tomograph (CT), a magnetic resonance image (MRI) and a 99Tc(m)-MDP 3-phase bone scan were carried out for 11 patients with BON. The main imaging findings of osteonecrosis are described. RESULTS: Conventional radiography and CT displayed osteolytic lesions with the involvement of cortical bone. MRI demonstrated the characteristic features of osteonecrosis and the oedema of soft tissues. Both CT and MRI were very useful in defining the extent of the lesions. 99Tc(m)-MDP three-phase bone scan was the most sensitive tool to detect the osteonecrosis at an early stage. CONCLUSIONS: 99Tc(m)-MDP three-phase bone scans who c
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