RETINAL AND CHOROIDAL VASCULAR OCCLUSION FOLLOWING AQUEOUS MISDIRECTION SYNDROME IN A PATIENT WITH SICKLE CELL TRAIT.

PURPOSE: To report a patient with retinal and choroidal vascular occlusion as a presenting sign of sickle cell trait following the development of aqueous misdirection syndrome. METHODS: Retrospective chart review. RESULTS: A patient treated for bilateral chronic angle-closure glaucoma with sequential EX-PRESS glaucoma filtration device surgery developed sequential bilateral aqueous misdirection syndrome. The left eye developed retinal arterial and localized choroidal vascular occlusions subsequent to an acute elevation in intraocular pressure and possibly the use of oral acetazolamide. The patient was subsequently found to have sickle cell trait. The right eye developed aqueous misdirection with acute elevation of intraocular pressure as well, but the patient was not treated with oral acetazolamide and did not develop vascular occlusion. CONCLUSION: Retinal and choroidal vascular occlusions can be the presenting sign of a patient with sickle cell trait. Sickle cell screening may be beneficial in African American or Middle Eastern patients after an acute rise in intraocular pressure, particularly before initiation of treatment with oral carbonic anhydrase inhibitors.

Sodium hypochlorite-induced acute kidney injury.

Sodium hypochlorite (bleach) is commonly used as an irrigant during dental procedures as well as a topical antiseptic agent. Although it is generally safe when applied topically, reports of accidental injection of sodium hypochlorite into tissue have been reported. Local necrosis, pain and nerve damage have been described as a result of exposure, but sodium hypo-chlorite has never been implicated as a cause of an acute kidney injury (AKI). In this report, we describe the first case of accidental sodium hypochlorite injection into the infraorbital tissue during a dental procedure that precipitated the AKI. We speculate that oxidative species induced by sodium hypochlorite caused AKI secondary to the renal tubular injury, causing mild acute tubular necrosis.

Profound nephrotic syndrome in a patient with ovarian teratoma.

The nephrotic syndrome (NS) has been associated with a variety of malignancies in a number of reports in the literature, but has been reported in only nine cases associated with ovarian neoplasms. Membranous nephropathy is the most common glomerular pathology causing the NS in patients with solid tumors. There has been only one report of an ovarian neoplasm associated with minimal change disease (MCD). We describe the case of a 36-year-old woman who presented with the NS secondary to biopsy-proven MCD, likely secondary to mature ovarian teratoma. Treatment by tumor removal and prednisone led to remission of the NS. To the best of our knowledge, this is the first report of an ovarian teratoma and the second report of an ovarian neoplasm associated with MCD.

Posterior reversible encephalopathy syndrome in a patient with lupus nephritis.

Posterior reversible encephalopathy syndrome (PRES) is characterized by acute onset of headache, nausea, focal neurological deficits or seizures along with radiological findings of white matter defects in the parietal and occipital lobes. Causes of PRES include uremia, hypertensive encephalopathy, eclampsia and immunosuppressive medications. Usually, the treatment of choice involves correcting the underlying abnormality. We describe an unusual case of recurrent PRES caused by uremia during a lupus flare in a patient with biopsy-proven Class IV Lupus Nephritis (LN) with vasculitis. PRES in systemic lupus erythematosis (SLE) is a rare clinical phenomenon and, when reported, it is associated with hypertensive encephalopathy. Our patient did not have hypertensive crisis, but had uremic encephalopathy. The patient's PRES-related symptoms resolved after initiation of hemodialysis. The temporal correlation of the correction of the uremia and the resolution of the symptoms of PRES show the etiology to be uremic encephalopathy, making this the first reported case of uremia-induced PRES in Class IV LN with vasculitis.

Rituximab in the treatment of refractory lupus nephritis with vasculitis.

Dysfunction of the B lymphocyte, an important component of adaptive immunity, is thought to be important in the pathogenesis of lupus nephritis (LN). There are several novel strategies emerging including B-cell depletion by the monoclonal antibodies to B-cell markers, rituximab. We describe an unusual clinical response of a 22-year-old Hispanic woman with class IV LN with vasculitis while on dialysis to cyclophosphamide (CY) and adjunct rituximab. The patient had a history of class III/V LN and was treated with nine months of CY and maintenance therapy with mycophenolate mofetil (MMF) for three years. While on MMF, the patient developed class IV LN with vasculitis leading to end-stage renal disease (ESRD). While the patient was on peritoneal dialysis, the patient was treated with two doses of rituximab and six doses of intravenous CY. The patient responded to this regimen and recovered kidney function within four months. The kidney function remained stable nine months after discontinuing peritoneal dialysis.

Crosstalk between medulloblastoma cells and endothelium triggers a strong chemotactic signal recruiting T lymphocytes to the tumor microenvironment.

Cancer cells can live and grow if they succeed in creating a favorable niche that often includes elements from the immune system. While T lymphocytes play an important role in the host response to tumor growth, the mechanism of their trafficking to the tumor remains poorly understood. We show here that T lymphocytes consistently infiltrate the primary brain cancer, medulloblastoma. We demonstrate, both in vitro and in vivo, that these T lymphocytes are attracted to tumor deposits only after the tumor cells have interacted with tumor vascular endothelium. Macrophage Migration Inhibitory Factor (MIF)" is the key chemokine molecule secreted by tumor cells which induces the tumor vascular endothelial cells to secrete the potent T lymphocyte attractant "Regulated upon Activation, Normal T-cell Expressed, and Secreted (RANTES)." This in turn creates a chemotactic gradient for RANTES-receptor bearing T lymphocytes. Manipulation of this pathway could have important therapeutic implications.

Regression of serous macular detachment due to peripheral exudative hemorrhagic chorioretinopathy following intravitreal bevacizumab.

PURPOSE: To describe a case of exudative macular detachment secondary to peripheral exudative chorioretinopathy with a favorable anatomic and visual outcome following treatment with intravitreal bevacizumab. METHODS: The medical records of a patient with peripheral exudative chorioretinopathy were reviewed. RESULTS: Macular reattachment was achieved with a corresponding visual acuity improvement that was maintained over an uncomplicated 17-month clinical course. CONCLUSIONS: Bevacizumab may be an effective treatment option for exudative macular detachment secondary to peripheral exudative hemorrhagic chorioretinopathy.

What is my goal? Expected weight loss and comorbidity outcomes among bariatric surgery patients.

BACKGROUND: A number of reasons lead patients to choose to undergo weight loss procedures. Previous studies have demonstrated that patients have unrealistic weight loss goals. However, there is a general paucity of information on a patient's expectations in regards to comorbidity improvement and resolution. The purpose of this study is to examine the impact a patient's comorbid conditions have on the motivation to proceed with bariatric surgery. Furthermore, we examined the patient's expectations regarding postoperative weight loss and comorbidity improvement. METHODS: Forty-five subjects completed a modified Goals and Relative Weights Questionnaire assessment 1 week prior to their anticipated bariatric surgery. The first portion addressed a patient's personal weight loss goals and factors that influence their procedure selection. The second part assessed the expectations and evaluations of a variety of specifically determined weight loss outcomes. RESULTS: A total of 45 patients completed the survey (laparoscopic adjustable gastric band (LAGB) 23/45; Roux-en-Y gastric bypass (RNYGB) 22/45). The mean goal percentage of excess weight loss was 85.0% (21-130%). This translated to 80.2% (21-127%) in the LAGB group and 90.5% (37-130%) in the RNYGB group. Of 13 possible reasons, "a desire for change in medical comorbidities" was deemed as most important in choosing a goal weight. Comorbidities with the highest dissatisfaction level included urinary incontinence and hypertension. All patients expected some degree of comorbidity improvement at 1 year postsurgery, with 26% (6/23) expecting a resolution of their diabetes, 36% (10/28) expecting a resolution of hypertension, and 40% (10/25) expecting a resolution of obstructive sleep apnea. CONCLUSION: A dissatisfaction with obesity-related comorbidities, most notably hypertension and urinary incontinence, was a motivating factor in choosing to undergo bariatric surgery. Patients expected improvement in their comorbid illnesses; however, they still possessed unrealistic weight loss expectations for their intended weight loss procedure.

Spectrum of sodium hypochlorite toxicity in man-also a concern for nephrologists.

Sodium hypochlorite (NaOCl) is the active ingredient in household bleach and is a very common chemical. It has been used in medical and commercial situations dating back to the 18th century for its disinfectant properties, including topical use in medicine as an antiseptic. For this indication, NaOCl is a proven and safe chemical. However, exposure of NaOCl beyond topical use, whether it is intentional or accidental, is associated with significant risks due to its strong oxidizing properties. Potentially damaging scenarios include ingestion, inhalation, deposition into tissue or injection into the bloodstream. All of these scenarios can lead to significant morbidity and even mortality. In this review, we examine the toxicity associated with NaOCl exposure and analyze potential mechanisms of injury, placing special emphasis on the potential for renal toxicity. Due to the extreme ease of access to household bleach products and its use in medicine, it is important for the clinician to understand the potential damage that can occur in NaOCl exposures so that complications can be prevented before they arise.

Persistent photopsia following course of oral voriconazole.

SUMMARY STATEMENT: A 56-year-old woman presented with photopsia and color changes in the left eye that had been present since starting voriconazole treatment 5 months earlier. The patient was seen in ophthalmologic follow-up 7 and 20 months after discontinuation of voriconazole with persistent visual complaints, including photopsia and "additional sources of light" in her left eye. PURPOSE: To describe a case of persistent photopsia following course of oral voriconazole. METHODS: The medical records of a patient with voriconazole treatment and persistent photopsia were reviewed. RESULTS: Persistent photopsia and visual complaints were noted 20 months after discontinuation of voriconazole. CONCLUSIONS: In light of increasing use of voriconazole to combat fungal infections, voriconazole-related photopsia may persist in certain patients.

Pulmonary alveolar proteinosis in a kidney transplant: a rare complication of sirolimus.

Pulmonary alveolar proteinosis (PAP) has been associated with the immunosuppressant sirolimus in transplant patients. PAP is a progressive lung disease characterized by the accumulation of surfactant-like material in the lungs leading to decreased pulmonary function with shortness of breath and cough as common symptoms. We report a rare case of sirolimus-associated PAP in a kidney transplant recipient with a history of end-stage renal disease secondary to haemolytic uraemic syndrome (HUS) and review of the literature. Discontinuation of sirolimus and initiation of tacrolimus led to resolution of PAP without recurrence of HUS.

Medical management of primary hyperparathyroidism with concomitant multiple myeloma.

Primary hyperparathyroidism (PHPT) and multiple myeloma (MM) are frequently observed in the adult population and can each independently lead to hypercalcemia. Despite the frequency of hypercalcemia secondary to PHPT and MM, these two conditions only rarely concurrently present in patients. We describe the management of PHPT in the setting of poorly differentiated MM in a patient presenting with hypercalcemia and pancytopenia. The patient was deemed at increased risk for surgical removal of the parathyroid gland and refused surgical intervention, so we chronically managed her PHPT and hypercalcemia with Cinacalcet and bisphosphonates. All of the similar cases in the literature are reviewed in this report along with medical management of PHPT. We believe that we describe the first medically managed PHPT in the setting of MM.

Osteonecrosis secondary to antiphospholipid syndrome: a case report, review of the literature, and treatment strategy.

Osteonecrosis is commonly present in patients with antiphospholipid syndrome (APS) and systemic lupus erythematosus (SLE). Treatment of this condition remains extremely controversial. We present a treatment strategy of avascular necrosis of the knee in a patient with catastrophic antiphospholipid syndrome with a history of SLE and APS. Aggressive treatment with 12 rounds of plasmapheresis, intravenous immunoglobulin, rituximab, and cyclophosphamide led to the patient's recovery with no recurrence of symptoms during 16 months of follow up. In this report, we further discuss the pathogenesis of osteonecrosis and current understanding of the treatment of this disease.

IRS1 regulation by Wnt/beta-catenin signaling and varied contribution of IRS1 to the neoplastic phenotype.

Dysregulation of beta-catenin levels and localization and constitutive activation of beta-catenin/TCF (T cell factor)-regulated gene expression occur in many cancers, including the majority of colorectal carcinomas and a subset of ovarian endometrioid adenocarcinomas. Based on the results of microarray-based gene expression profiling we found the insulin receptor substrate 1 (IRS1) gene as one of the most highly up-regulated genes upon ectopic expression of a mutant, constitutively active form of beta-catenin in the rat kidney epithelial cell line RK3E. We demonstrate expression of IRS1 can be directly activated by beta-catenin, likely in part via beta-catenin/TCF binding to TCF consensus binding elements located in the first intron and downstream of the IRS1 transcriptional start site. Consistent with the proposal that beta-catenin is an important regulator of IRS1 expression in vivo, we observed that IRS1 is highly expressed in many cancers with constitutive stabilization of beta-catenin, such as colorectal carcinomas and ovarian endometrioid adenocarcinomas. Using a short hairpin RNA approach to abrogate IRS1 expression and function, we found that IRS1 function is required for efficient de novo neoplastic transformation by beta-catenin in RK3E cells. Our findings add to the growing body of data implicating IRS1 as a critical signaling component in cancer development and progression.

Bilateral leiomyosarcoma of the kidney with family history of kidney cancer.

Sarcomas make up 1%-2% of all malignant renal tumors in adults, and the incidence increases with advancing age. Renal sarcomas are less common, but more lethal than sarcomas of any other genitourinary site. The common clinical presentation of renal sarcomas in adults include a palpable mass, abdominal or flank pain, and hematuria, similar to those seen with large, rapidly growing renal cell carcinomas. Usually, radical nephrectomy remains the treatment of choice for these tumors, which exhibit an aggressive biological behavior and an unfavorable prognosis. We describe an unusual case of bilateral renal leiomyosarcoma in a 61-year-old white male. The patient also had an uncle who had bilateral kidney cancer. In addition, our patient presented with a pulmonary embolism, which is different from the classic presentation of leiomyosarcoma. The patient did not undergo surgery, as the tumor had also invaded the surrounding vasculature and was felt to be unresectable by the consulting surgeons. He was treated with gemcitabine and docetaxel chemotherapy, with stabilization of disease.

Catastrophic antiphospholipid syndrome presenting as ischemic pancreatitis in systemic lupus erythematosus.

CONTEXT: Antiphospholipid syndrome is often associated with systemic lupus erythematosus. Both syndromes have different clinical manifestations based on organ involvement. Antiphospholipid syndrome commonly causes spontaneous abortions, cerebral vascular occlusion, and deep venous thrombosis. Catastrophic antiphospholipid syndrome occurs when three or more organ systems are affected by thromboses in less than a week. CASE REPORT: We report a unique case of a young woman with a history of systemic lupus erythematosus and antiphospholipid syndrome who presented with recurrent ischemic pancreatitis. Pancreatitis was refractory to anticoagulation and low dose steroids. Secondary to recurrence of pancreatitis and other organ involvement, she was treated as a presumed case of catastrophic antiphospholipid syndrome. Aggressive treatment with plasmapheresis, corticosteroids, cyclophosphamide, and anticoagulation eventually led to her recovery. CONCLUSION: Awareness of this rare, rapidly fatal medical condition prompts vital, early intervention to improve patients' survival. This case report aims to add to the limited therapeutic data available as well as suggest a possible approach to treating this rare syndrome with very high morbidity and mortality.