A patient with profuse cutaneous pigmented lesions, subcutaneous myxoid neurofibromata and atrial myxoma is described. This is the second report of this syndrome, whose importance lies in the possibility of avoiding the potentially lethal consequences of a treatable cardiac tumour by recognition of distinctive cutaneous manifestations.
Primary bone lymphoma (PBL) is a rare disease. There has been paucity of studies addressing its therapy and prognosis. We retrospectively examined PBL cases seen at the University of Miami from June 2000 to August 2007 to describe our single institution experience and review the literature. Fifty-three patients were identified with a median age of 52 (18-87) and a median follow-up of 40 months (0-106). The most common histologic type was diffuse large B-cell lymphoma (83%). Forty-one patients (77%) presented with localized disease and 48 were treated at our institution. Forty-six patients underwent chemotherapy (40 patients) and/or radiation (36 patients). Forty-four patients achieved a complete response and all patients were alive at last follow-up. The progression-free survival (PFS) was 83% at 4 years. No difference in PFS was observed between patients treated with chemotherapy or combined chemotherapy plus radiation. There was a trend toward improvement in PFS (p = 0.062) of patients with DLBCL treated with rituximab plus chemotherapy. Our single institutional experience demonstrates that the outcome of patients with PBL is excellent. Although the current data support the use of combined modality treatment for localized PBL, randomized controlled trials are needed especially now, when rituximab is routinely added to chemotherapy regimens.
Obesity and end‐stage renal disease (ESRD) are on the increase worldwide. Kidney transplantation is the treatment of choice for ESRD. However, obesity is considered a contraindication for transplantation. We investigated the effect of BMI on mortality in transplanted and patients remaining on the waiting list in the United Kingdom. We analyzed the UK Renal Registry (RR) and the National Health Service Blood and Transplant (NHSBT) Organ Donation and Transplantation data for patients listed from January 1, 2004 to December 31, 2010, with follow‐up until December 31, 2011. Seventeen thousand six hundred eighty‐one patients were listed during the study period, with BMI recorded for 13 526 (77%). One‐ and five‐year patient survival was significantly better in all BMI bands (<18.5, 18.5–<25, 25–<30, 30–<35, 35–<40, and 40+kg/m2) in the transplant group when compared to those who remained on the waiting list (p < 0.0001). The analyses were repeated excluding live donor transplants and the results were essentially the same. On analyses of patient survival with BMI as a continuous variable or using 5 kg weight bands, there was no cut‐off observed in the higher BMI patients where there would be no benefit to transplantation. For transplanted patients (N = 8088), there was no difference in patient or graft survival between the defined BMI bands. Thus, irrespective of BMI, patient survival is improved if transplanted.
Kidney disease is a recognised risk factor for poor COVID-19 outcomes. Up to 30 June 2020, the UK Renal Registry (UKRR) collected data for 2,385 in-centre haemodialysis (ICHD) patients with COVID-19 in England and Wales. Overall unadjusted survival at 1 week after date of positive COVID-19 test was 87.5% (95% CI 86.1–88.8%); mortality increased with age, treatment vintage and there was borderline evidence of Asian ethnicity (HR 1.16, 95% CI 0.94–1.44) being associated with higher mortality. Compared to the general population, the relative risk of mortality for ICHD patients with COVID-19 was 45.4 and highest in younger adults. This retrospective cohort study based on UKRR data supports efforts to protect this vulnerable patient group.
PRACTICE What you need to know• An emergency care plan allows clinicians to discuss and record patient preferences in advance, not only regarding cardiopulmonary resuscitation, but all aspects of care and treatment in an emergency• An emergency care plan provides recommendations for care and treatment for future scenarios when people might not have the capacity to communicate their preferences• Tailor the emergency care plan to consider the most likely individual situations, such as a sudden acute illness, deterioration in a long-term condition, or sudden cardiac or respiratory arrest Box 1: Sources of evidenceThis article draws on an evidence synthesis, 4 two systematic reviews, 5 6 and consensus discussions in a group of stakeholders covering a broad range of care settings and specialties. These stakeholders included nurses, patient representatives, ambulance clinicians, and doctors, who together have developed the ReSPECT process in the UK. 7 The principles described are applicable more generally to discussions about advance planning for future emergencies.Box 2: Some triggers for discussing emergency care plans• Requests by the people themselves• Recognition of long term or complex medical needs-discussions can be started in hospital clinics and wards as well as in general practice• Diagnosis of a life limiting condition-recognition that end-of-life care will be needed• Admission to hospital, especially with an acute illness of any kind• Admission to a care home or nursing home• Identified risk of acute deterioration, cardiac arrest, or death Box 3: What to do when people lack capacity for making decisions about emergency care plans• Discussion with family or other representatives should follow a similar sequence to discussion with a patient (see within "How to have a better conversation")• Try to establish the choices that the person would have made if they had capacity; find out if they have an advance care plan or advance statement recording their preferences, or a legally binding instruction such as an Advance Decision to Refuse Treatment (England & Wales)• Help the family to understand their role: to advise the healthcare team what the patient would have been likely to decide, had he or she had capacity, and what they believe to be in the patient's best interests, but not to make clinical decisions• The senior responsible clinician has ultimate responsibility to make decisions in the person's best interests or for their overall benefit• It is common for relatives to feel that they are being asked to make decisions: take care to avoid this. Explain clearly and exactly how you are asking relatives to help you make the right decision for the patient, and remember to reinforce the same message afterwards, because at these stressful times families often forget or misinterpret what has been said to themIn the UK, the exception to this is if someone has been appointed as legal proxy with powers to make decisions about life sustaining treatment: that person's decisions must be viewed as binding, as long as they...
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