Ischemic myopathy revealing systemic calciphylaxis.

INTRODUCTION: Patients with renal failure who are being treated with dialysis frequently develop neuromuscular manifestations. Renal failure-associated calciphylaxis, also termed calcific uremic arteriolopathy (CUA), is a life-threatening condition usually observed in patients with end-stage renal disease on chronic dialysis or after renal transplantation. METHODS: We describe a hemodialyzed patient who presented with rapidly progressive unexplained systemic vasculopathy, muscle atrophy, and proximal weakness, that unexpectedly proved to be caused by calciphylaxis. RESULTS: Quadriceps muscle biopsy disclosed diffuse vascular calcific deposits on medium- and small-sized vessels, characteristic of CUA. Other changes included ischemic myopathy, focal intracellular calcium accumulation within myofibers, and calcium deposits in endomysial capillaries associated with marked complement activation and C5b9 formation. CONCLUSION: There are only a few descriptions of muscle involvement in the context of CUA, a condition with a prognosis that depends on early diagnosis and treatment. This report underscores the usefulness of muscle biopsy in the diagnosis of systemic calciphylaxis. Muscle Nerve 56: 529-533, 2017.

Low Rates of Blood Transfusion in Elective Resections of Neurofibromas in a Cohort Study: Neurofibroma Length as a Predictor of Transfusion Requirement.

BACKGROUND: Neurofibromas in neurofibromatosis type 1 induce aesthetic and functional morbidity. Perioperative bleeding has been reported as an obstacle to neurofibroma resections. The authors studied the requirement for blood transfusion during surgical treatment of neurofibromatosis type 1. METHODS: Six hundred twenty-two procedures performed on 390 neurofibromatosis type 1 patients at the national referral center from 1995 to 2011 were analyzed in two chronologic sets of patients: set 1 (February of 1995 to September of 2007), in which only one surgeon operated; and set 2 (October of 2007 to January of 2011), in which two additional surgeons were involved. Malignant peripheral nerve sheath tumors, reconstructive procedures, and spontaneous hemorrhages were excluded from the analysis. Age, sex, preoperative hemoglobin concentration, location, length, estimated volume and histologic features of the largest neurofibroma (cumulative values for multiple neurofibromas), and procedure duration were studied as potential predictors of blood transfusion that were measured in terms of units of packed red blood cells. RESULTS: Seventy reconstructive procedures, two cases of spontaneous hemorrhage, and 32 malignant peripheral nerve sheath tumor resections were excluded. Among 516 procedures (318 and 198 in sets 1 and 2, respectively), 17 (2.7 percent) required blood transfusions. The requirement for transfusion was associated with neurofibroma length in both sets, with an optimal cutoff value of 13 cm in both sets. CONCLUSIONS: Contrary to the literature, the requirement for blood transfusion was found to be low (2.7 percent of the cases) during elective resection of neurofibromas in neurofibromatosis type 1. Elective resections of benign neurofibromas less than 13 cm in length were not associated with a requirement for blood transfusion. CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, III.