-
Internal Medicine (Tokyo, Japan) Aug 2023A 16-year-old Japanese girl developed a fever, thrombocytopenia, and renal dysfunction. Treatment was started with steroids, but cervical lymphadenopathy and ascites...
A 16-year-old Japanese girl developed a fever, thrombocytopenia, and renal dysfunction. Treatment was started with steroids, but cervical lymphadenopathy and ascites developed. A lymph node biopsy indicated TAFRO syndrome. The patient's renal function deteriorated, and dialysis was started. Refractory hypertension and subsequent encephalopathy developed. Treatment was started with an anti-IL-6 receptor antibody and an anti-CD20 monoclonal antibody. A kidney biopsy showed malignant nephrosclerosis-like microangiopathy and glomerular collapse due to narrowing of the small arteries. The majority of TAFRO syndrome cases are adult-onset, with glomerular microangiopathy. To our knowledge, this is the first report of adolescent-onset TAFRO syndrome presenting with malignant nephrosclerosis-like lesions associated with hypertension.
Topics: Adult; Female; Humans; Adolescent; Nephrosclerosis; Castleman Disease; Kidney Glomerulus; Kidney Diseases; Hypertension; Edema
PubMed: 36517029
DOI: 10.2169/internalmedicine.0529-22 -
Pediatric and Developmental Pathology :... 2023Pathologic characterization of pulmonary complications following hematopoietic stem cell transplantation (HSCT) is limited. We describe lung findings in pediatric...
BACKGROUND
Pathologic characterization of pulmonary complications following hematopoietic stem cell transplantation (HSCT) is limited. We describe lung findings in pediatric patients who died following HSCT and attempt to identify potential clinical associations.
METHODS
Pathology databases at Texas Children's Hospital and the Children's Hospital of Philadelphia were queried (2013-2018 CHOP and 2017-2018 TCH). Electronic medical records and slides were reviewed.
RESULTS
Among 29 patients, 19 received HSCT for hematologic malignancy, 8 for non-malignant hematologic disorders, and 2 for metastatic solid tumors. Twenty-five patients (86%) showed 1 or more patterns of acute and organizing lung injury. Sixty-two percent had microvascular sclerosis, with venous involvement noted in most cases and not correlating with clinical history of pulmonary hypertension, clinical transplant-associated thrombotic microangiopathy, irradiation, or graft-versus-host disease. Features suggestive of graft-versus-host-disease were uncommon: 6 patients had lymphocytic bronchiolitis, and only 2 patients had evidence of bronchiolitis obliterans (both clinically unexpected), both with a mismatched unrelated donor transplant.
CONCLUSIONS
Acute and subacute alveolar injury (diffuse alveolar damage or organizing pneumonia) is common in pediatric patients who died following HSCT and is difficult to assign to a specific etiology. Microvascular sclerosis was frequent and did not correlate with a single distinct clinical feature.
PubMed: 37165556
DOI: 10.1177/10935266231170101 -
Surgical Endoscopy Dec 2023For patients with cancer or injury of the esophagus, esophagectomy with reconstruction using the stomach (gastric pull-up, GPU) or colon (colonic interposition, CI) can...
INTRODUCTION
For patients with cancer or injury of the esophagus, esophagectomy with reconstruction using the stomach (gastric pull-up, GPU) or colon (colonic interposition, CI) can restore function but is associated with high morbidity. We sought to describe the differences in outcomes between the two replacement organs using a national database.
METHODS
From ACS-NSQIP, patients who underwent GPU or CI between 2006 and 2020 were identified. Univariate analyses were performed on length of stay, complications, reoperation, readmission, and mortality. Variables with P ≤ 0.2 were included in the multivariate regression. Primary outcomes were 30-day reoperation, readmission, and mortality. Data were assessed using Chi-squared tests and logistic regression.
RESULTS
There were 12,545 GPU and 502 CI patients. GPU patients were older with higher BMI, and more likely to be male (80.3% versus 70.3%, P < 0.0001) and white (77.8% versus 69.1%, P < 0.0001). More GPU patients had independent functional status and underlying bleeding disorders, but fewer other preoperative comorbidities than CI patients. On univariate analysis, CI patients had longer hospital stays (13 versus 10 days, P < 0.0001); more reoperations (23.9% versus 14.5%, P < 0.0001); a lower rate of discharge to home (70.9% versus 82.1%, P < 0.0001); and a higher mortality rate (6.2% versus 2.9%, P < 0.0001). On multivariate analysis, CI was associated with increased risk of reoperation but not with readmission or mortality. Reoperation was associated with CI, smoking, chronic wound, hypertension, higher ASA class, contaminated or dirty wound class, and longer operative time. Readmission was associated with female gender, hypertension, and longer operative time. Mortality was associated with age, metastatic cancer, preoperative sepsis, preoperative renal failure, malignant esophageal disease, higher ASA class, incomplete closure, and longer operative time.
CONCLUSION
Colonic interposition, although a more difficult option with traditionally worse outcomes, should still be considered for patients requiring esophagectomy if the stomach cannot be used to restore continuity, as differences in outcomes appear to be due to underlying frailty of patients rather than the procedure.
Topics: Humans; Male; Female; Esophagectomy; Postoperative Complications; Esophagus; Reoperation; Neoplasms; Hypertension; Retrospective Studies; Risk Factors; Patient Readmission
PubMed: 37730851
DOI: 10.1007/s00464-023-10420-3 -
European Journal of Heart Failure Apr 2024Cardio-oncology is a rapidly growing field of cardiovascular (CV) medicine that has resulted from the continuously increasing clinical demand for specialized CV... (Review)
Review
Cardio-oncology is a rapidly growing field of cardiovascular (CV) medicine that has resulted from the continuously increasing clinical demand for specialized CV evaluation, prevention and management of patients suffering or surviving from malignant diseases. Dealing with CV disease in patients with cancer requires special knowledge beyond that included in the general core curriculum for cardiology. Therefore, the European Society of Cardiology (ESC) has developed a special core curriculum for cardio-oncology, a consensus document that defines the level of experience and knowledge required for cardiologists in this particular field. It is structured into 8 chapters, including (i) principles of cancer biology and therapy; (ii) forms and definitions of cancer therapy-related cardiovascular toxicity (CTR-CVT); (iii) risk stratification, prevention and monitoring protocols for CTR-CVT; (iv) diagnosis and management of CV disease in patients with cancer; (v) long-term survivorship programmes and cardio-oncology rehabilitation; (vi) multidisciplinary team management of special populations; (vii) organization of cardio-oncology services; (viii) research in cardio-oncology. The core curriculum aims at promoting standardization and harmonization of training and evaluation in cardio-oncology, while it further provides the ground for an ESC certification programme designed to recognize the competencies of certified specialists.
Topics: Humans; Cardiology; Curriculum; Medical Oncology; Societies, Medical; Neoplasms; Europe; Cardiovascular Diseases; Cardiotoxicity; Cardio-Oncology
PubMed: 38059343
DOI: 10.1002/ejhf.3102 -
Bulletin Du Cancer Oct 2023Pheochromocytomas and paragangliomas are rare neuroendocrine tumors, developed respectively in the adrenal medulla and in extra-adrenal locations. Their malignancy is...
Pheochromocytomas and paragangliomas are rare neuroendocrine tumors, developed respectively in the adrenal medulla and in extra-adrenal locations. Their malignancy is defined by the presence of distant metastases. Forty percent of them are inherited and can be part of different hereditary syndromes. Their management is ensured in France by the multidisciplinary expert centers of the ENDOCAN-COMETE national network "Cancers of the Adrenal gland", certified by the National Cancer Institute and discussed within multidisciplinary team meetings. The diagnostic and therapeutic work-up must be standardized, based on an expert analysis of clinical symptoms, hormonal biological secretions, genetics, morphological and specific metabolic imaging. In the context of a heterogeneous survival sometimes beyond seven to ten years, therapeutic intervention must be justified. This is multidisciplinary and relies on surgery, interventional radiology, external or internal radiotherapy and medical treatments such as sunitinib or dacarbazine and temodal chemotherapy. The personalized approach based on functional imaging fixation status and genetics is progressing despite the extreme rarity of this disease.
PubMed: 37573200
DOI: 10.1016/j.bulcan.2023.06.002 -
Frontiers in Immunology 2024Pulmonary hypertension (PH) is a malignant disease with progressive increase of pulmonary vascular pressure, which eventually leads to right heart failure. More and more... (Review)
Review
Pulmonary hypertension (PH) is a malignant disease with progressive increase of pulmonary vascular pressure, which eventually leads to right heart failure. More and more evidences show that immune cells and inflammation play an important role in the occurrence and development of PH. In the context of pulmonary vascular diseases, immune cells migrate into the walls of the pulmonary vascular system. This leads to an increase in the levels of cytokines and chemokines in both the bloodstream and the surrounding tissues of the pulmonary vessels. As a result, new approaches such as immunotherapy and anti-inflammatory treatments are being considered as potential strategies to halt or potentially reverse the progression of PH. We reviewed the potential mechanisms of immune cells, cytokines and chemokines in PH development. The potential relationship of vascular cells or bone morphogenetic protein receptor 2 (BMPR2) in immune regulation was also expounded. The clinical application and future prospect of immunotherapy were further discussed.
Topics: Humans; Hypertension, Pulmonary; Lung; Inflammation; Cytokines; Chemokines
PubMed: 38529271
DOI: 10.3389/fimmu.2024.1374506 -
BMJ Case Reports Dec 2023A woman in her 30s presented with complaints of sudden onset of defective vision in the right eye for 2 days, with history of headache for a month. On examination,...
A woman in her 30s presented with complaints of sudden onset of defective vision in the right eye for 2 days, with history of headache for a month. On examination, best corrected visual acuity was 20/40 in the right eye and 20/20 in the left eye. Anterior segment examination was normal. Fundus examination of both the eyes showed generalised arteriolar attenuation with diffuse, hyperaemic disc oedema and serous retinal detachment at macula in the right eye. Her blood pressure (BP) was 230/140 mm Hg. Other systemic evaluation was unremarkable. In the review visit, patient's BP reduced to 140/100 mm Hg, and visual acuity in the right eye improved to 20/20. Fundus in the right eye showed resolving disc oedema with macular star formation, and the left eye had developed soft exudates. This seemed to confirm the diagnosis of the disc oedema being caused by hypertension and a highly asymmetrical presentation of hypertensive retinopathy.
Topics: Female; Humans; Hypertension, Malignant; Retinal Detachment; Fundus Oculi; Macula Lutea; Edema
PubMed: 38061857
DOI: 10.1136/bcr-2023-255723 -
Identifying therapeutic targets for cancer among 2074 circulating proteins and risk of nine cancers.Nature Communications Apr 2024Circulating proteins can reveal key pathways to cancer and identify therapeutic targets for cancer prevention. We investigate 2,074 circulating proteins and risk of nine...
Circulating proteins can reveal key pathways to cancer and identify therapeutic targets for cancer prevention. We investigate 2,074 circulating proteins and risk of nine common cancers (bladder, breast, endometrium, head and neck, lung, ovary, pancreas, kidney, and malignant non-melanoma) using cis protein Mendelian randomisation and colocalization. We conduct additional analyses to identify adverse side-effects of altering risk proteins and map cancer risk proteins to drug targets. Here we find 40 proteins associated with common cancers, such as PLAUR and risk of breast cancer [odds ratio per standard deviation increment: 2.27, 1.88-2.74], and with high-mortality cancers, such as CTRB1 and pancreatic cancer [0.79, 0.73-0.85]. We also identify potential adverse effects of protein-altering interventions to reduce cancer risk, such as hypertension. Additionally, we report 18 proteins associated with cancer risk that map to existing drugs and 15 that are not currently under clinical investigation. In sum, we identify protein-cancer links that improve our understanding of cancer aetiology. We also demonstrate that the wider consequence of any protein-altering intervention on well-being and morbidity is required to interpret any utility of proteins as potential future targets for therapeutic prevention.
Topics: Humans; Neoplasms; Female; Risk Factors; Mendelian Randomization Analysis; Biomarkers, Tumor; Male; Blood Proteins
PubMed: 38684708
DOI: 10.1038/s41467-024-46834-3 -
Kidney International May 2024Thrombotic microangiopathies (TMA) are usually associated with hematological features (RH-TMA). The epidemiology of TMA limited to kidneys (RL-TMA) is unclear Therefore,...
Thrombotic microangiopathies (TMA) are usually associated with hematological features (RH-TMA). The epidemiology of TMA limited to kidneys (RL-TMA) is unclear Therefore, patients with TMA and native kidney biopsies were identified during 2009-2022 in 20 French hospitals and results evaluated. RL-TMA was present in 341/757 (45%) patients and associated with lower creatinine levels (median 184 vs 346 μmol/L) than RH-TMA. RL-TMA resulted from virtually all identified causes, more frequently from anti-VEGF treatment and hematological malignancies but less frequently from shigatoxin-associated hemolytic uremic syndrome (HUS), systemic sclerosis, gemcitabine and bacterial infection, and even less frequently when three or more causes/triggers were combined (RL-TMA: 5%; RH-TMA: 12%). RL-TMA was associated with significantly lower major cardiovascular events (10% vs 20%), kidney replacement therapy (23% vs 43%) and death (12% vs 20%) than RH-TMA during follow-up (median 28 months). Atypical HUS (aHUS) was found in 326 patients (RL-TMA: 43%, RH-TMA: 44%). Among the 69 patients with proven complement-mediated aHUS, eculizumab (anti-C5 therapy) was used in 43 (62%) (RL-TMA: 35%; RH-TMA: 71%). Among the 257 other patients with aHUS, including 51% with RL-TMA, eculizumab was used in 29 but with unclear effects of this treatment. Thus, RL-TMA represents a very high proportion of patients with TMA and results from virtually all known causes of TMA and includes 25% of patients with complement-mediated aHUS. Adverse outcomes of RL-TMA are lower compared to RH-TMA but remain significant. Anti-C5 therapy was rarely used in RL-TMA, even in proven complement-mediated aHUS, and its effects remain to be assessed.
Topics: Adult; Humans; Kidney; Thrombotic Microangiopathies; Atypical Hemolytic Uremic Syndrome; Complement System Proteins; Kidney Function Tests
PubMed: 38431217
DOI: 10.1016/j.kint.2024.02.014