Presence of Anti-Glomerular Basement Membrane Antibodies and Myeloperoxidase Anti-Neutrophilic Cytoplasmic Antibodies in a Case of Rapidly Progressive Glomerulonephritis.

A 69-year-old male had initially presented with low-grade proteinuria, microhematuria, and a positive myeloperoxidase anti-neutrophilic antibody (ANCA). He subsequently developed deterioration of kidney function and developed uremic symptoms. Creatinine was 486.2 µmol/L (5.5 mg/dL). Anti-MPO was positive (titer >8 U, normal <0.4). He was clinically diagnosed with rapidly proliferative glomerulonephritis most likely due to ANCA vasculitis. He received three doses of pulse methylprednisolone therapy. Kidney biopsy showed pauci-immune glomerulonephritis. Immunofluorescence was positive for faint linear IgG staining of glomerular basement membrane (GBM). Anti-GBM antibody was positive 2.1 U (normal <1). He was started on high-dose oral steroids; monthly intravenous cyclophosphamide and plasmapheresis were also initiated. His symptoms improved and creatinine is 247.5 µmol/L (2.8 mg/dL). His repeat anti-GBM antibody was negative. This is a rare case of rapidly progressive glomerulonephritis due to dual MPO-ANCA antibodies and anti-GBM antibodies (DAV).

A review of the nonpressor and nonantidiuretic actions of the hormone vasopressin.

The pressor and antidiuretic actions of arginine vasopressin (AVP) have been well documented. This review focuses on the less widely appreciated actions of AVP which also have important physiologic functions and when better understood may provide important insights into common disease states. These actions include effects on pain perception and bone structure as well as important relationships to the varied components of metabolic syndrome. These include effects on blood glucose, lipid levels, and blood pressure. AVP may also play a role in the progression of chronic kidney disease and effect physiologic changes relating to aging, abnormal social behavior, and cognitive function. Important cellular responses including cell proliferation, inflammation, and control of infection and their relationship to AVP are described. Finally, the effects of AVP on hemostasis and the hypothalamic-pituitary-adrenal axis are noted. The goal of this summary of the various actions of AVP is to direct attention to the potential benefits of research in these underemphasized areas of importance.

Neurofibromatosis type 1-associated hypertension secondary to coarctation of the thoracic aorta.

Neurofibromatosis type 1 (NF-1), also known as von Recklinghausen's disease, is an autosomal dominant genetic disorder. NF-I vasculopathy has been used to describe various vascular malformations associated with NF-1. Secondary hypertension related to NF-1 vasculopathy has been reported because of renal artery stenosis, coarctation of the abdominal aorta and other vascular lesions; however, coarctation of the thoracic aorta has seldom been reported. We report the first case, to our knowledge, of isolated coarctation of thoracic aorta in a pregnant female with NF-1. Healthcare providers caring for patients with NF-1 should be aware of associated vascular complications.