Granulomatosis with Polyangiitis with Myocarditis and Ventricular Tachycardia.

Granulomatosis with polyangiitis (GPA), previously known as Wegener's granulomatosis, is a pulmonary-renal syndrome affecting small and medium sized blood vessels. The disease has a prevalence in studies ranging from 3 to 15.7 cases per 100,000, with a noted increasing incidence and prevalence in more recent studies. Pulmonary manifestations include hemorrhage, lung cavitary lesions, and pulmonary fibrosis. Within the kidney, GPA is known to cause rapidly progressive pauci-immune crescentic glomerulonephritis. Rare and severe cardiovascular manifestations include pericarditis, arrhythmias, myocarditis, and aortic valve disease. Our patient is a 43-year-old female with typical pulmonary and renal lesions from GPA and also acute myocarditis, multiple episodes of ventricular tachycardia, and a severe reactive thrombocytosis.

The utility and accuracy of four equations in predicting sodium levels in dysnatremic patients.

BACKGROUND: Improper correction of hyponatremia can cause severe complications, including osmotic demyelination syndrome (ODS). The Adrogué-Madias equation (AM), the Barsoum-Levine (BL) equation, the Electrolyte Free Water Clearance (EFWC) equation and the Nguyen-Kurtz (NK) equation are four derived equations based on the empirically derived Edelman equation for predicting sodium at a later time (Na2) from a known starting sodium (Na1), fluid/electrolyte composition and input and output volumes. METHODS: Our retrospective study included 43 data points from 31 mostly hyponatremic patients. We calculated Na2 based on five sets of rules that were progressively more precisely calculated. Sets A-D included all 31 patients and 43 data points and set E was based on 15 patients and 27 data points. RESULTS: The root mean square error was calculated and found to be between 4.79 and 6.37 mmol/L (mEq/L) for all sets. Bland-Altman analysis showed high variability and discrepancies between the predicted and actual Na2. CONCLUSIONS: Like similar studies in hypernatremic patients, the data suggest that hyponatremic modeling equations are not reliably accurate in predicting Na2 from Na1 and available clinical data regarding sodium, potassium and fluid balance over longer time frames (12-30 h). Our study was retrospective and was done in an inpatient setting and thus was subject to limitations and laboratory measurement variability, but showed that all four equations are not able to reliably predict Na2 from Na1 and inputs across a 12-30 h period.

Multiple sites of calciphylaxis in a patient with chronic renal failure.

Calciphylaxis has seldom been reported in patients with acute renal failure or in pre-dialysis patients. It also has been reported at lower calcium phosphorous products and in patients with adynamic bone disease. We report a pre-hemodialysis (HD) patient with acute renal failure and biopsy-proven calciphylaxis involving multiple cutaneous sites with calcification of the perineal area resulting in dry gangrene of the penis that necessitated a partial penectomy. The patient had elevated serum calcium, phosphorous and parathyroid hormone level of 612 pg/mL. The same patient suffered subsequently from a calcium embolus that occluded his left ophthalmic artery and resulted in left eye blindness. Calciphylaxis is a devastating phenomenon and physicians should have a high clinical suspicion for it in HD patients as well as in patients with late stages of chronic kidney disease.

Wegener's granulomatosis mimicking inflammatory bowel disease and presenting with chronic enteritis.

Wegener's granulomatosis, also known as anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis, is a small vessel vasculitis with primarily pulmonary, renal, and sinus disease manifestations. The prevalence of Wegener's granulomatosis is three cases per 100,000 patients. Cardiovascular, neurologic, cutaneous, and joint manifestations have been reported in many case reports and case series. Gastrointestinal manifestations are less noted in Wegener's granulomatosis, although they have been previously reported in the form of intestinal perforation and intestinal ischemia. Additionally, there are characteristic findings of vasculitis that are noted with active Wegener's granulomatosis of the small bowel. We report a case of an elderly patient who presented with weight loss, diarrhea, and hematochezia. His symptoms were chronic and had lasted for more than 1 year before diagnosis. Inflammatory bowel disease or chronic enteritis due to Salmonella arizonae because of reptile exposure originally were suspected as etiologies of his presentation. The findings of proteinuria, renal failure, and pauci-immune glomerulonephritis on renal biopsy, in conjunction with an elevated c-ANCA titer, confirmed the diagnosis of Wegener's granulomatosis with associated intestinal vasculitis. This case demonstrates an atypical presentation of chronic duodenitis and jejunitis secondary to Wegener's granulomatosis, which mimicked inflammatory bowel disease.

Mycobacterium avium-intracellulare otomastoiditis in a young AIDS patient: case report and review of the literature.

Mycobacterium avium-intracellulare (MAI) complex is a common opportunistic infection that generally occurs in patients with a CD4 cell count less than 75. Current recommendations for prophylaxis include using a macrolide once a week, while treatment usually requires a multidrug regimen. Disseminated MAI infections often occur in patients who are not compliant with prophylaxis or their highly active antiretroviral therapy (HAART). Many manifestations of MAI infection are well documented in human immunodeficiency virus (HIV) patients, including pulmonary and cutaneous manifestations, but other unusual manifestations such as pericarditis, pleurisy, peritonitis, brain abscess, otitis media, and mastoiditis are sporadically reported in the infectious diseases literature. This case report is of a 22-year-old female who contracted HIV at a young age and who was subsequently noncompliant with HAART, MAI prophylaxis, and prior treatment for disseminated MAI infection. Unsurprisingly, the patient developed recurrent disseminated MAI infection. The patient's presentation was atypical, as she developed severe otomastoiditis and posterior reversible encephalopathy syndrome. The posterior reversible encephalopathy syndrome was thought to be due to the disseminated MAI infection or to immune reconstitution inflammatory syndrome. The infection was confirmed to be secondary to MAI by culture of the mastoid bone. Microbiological analysis of the MAI strain cultured showed resistance to several first-line antibiotics used for prophylaxis against and treatment of MAI. This was likely due to the patient's chronic noncompliance. Otomastoiditis secondary to MAI is predominantly a pediatric disease and a rare entity in general. It has been reported in three case reports and one case series in pediatric patients, and now in this case report of an adult patient with HIV [corrected]. Improved clinician education in the diagnosis, treatment, and, most important, prevention of MAI and other opportunistic infections is needed. Greater HIV screening, appropriate HAART medication administration, and availability of infectious disease specialists is needed in at-risk populations to help prevent such serious infections. Patient education and greater access to care should serve to prevent medication nonadherence and to enhance affordability of HAART and prophylactic antibiotics.

Three Presentations of Takayasu's Arteritis in Hispanic Patients.

Takayasu's arteritis (TA) is a medium and large vessel vasculitis, defined as a nonspecific aortitis that usually involves the aorta and its branches Kobayashi and Numano (2002). Its etiology remains unclear, and its complications are diverse and severe, including stenosis of the thoracic and abdominal aorta, aortic valve damage and regurgitation, and stenosis of the branches of the aorta. Carotid stenosis, coronary artery aneurysms, and renal artery stenosis resulting in renovascular hypertension are also reported sequellae of TA Kobayashi and Numano (2002). The disease was first described in Japan, but has also been diagnosed in India and Mexico Johnston (2002). Its incidence in the United States has been quoted as 2.6 patients per 1,000,000 people/year Johnston (2002). In Japan, its incidence is 3.6 patients per 1,000,000 patients/year and prevalence is 7.85 patients per 100,000 per year Morita et al. (1996). The natural history of this disease, which is commonly present in Asian populations, has only recently been studied in Hispanic patients despite the notable incidence and prevalence of TA in Mexican, South American, and Indian populations (Johnston 2002, Gamarra et al. 2010 ). We present three cases of Hispanic patients who presented with TA at Olive-View-UCLA Medical Center (OVMC). We review their clinical and radiographic presentations. Finally, we review the literature to compare the clinical features of our three patients with data regarding the presentation of TA in more traditional Asian populations.