Atypical hemolytic uremic syndrome in human immunodeficiency virus-1-infected children

We describe the clinical and pathological findings of the hemolytic uremic syndrome (HUS) in two children with human immunodeficiency virus (HIV) infection. Both patients presented with microangiopathic hemolytic anemia, thrombocytopenia, and subsequently developed renal failure. The diagnosis of HUS was confirmed by renal histopathology in both patients. None of these children presented with bloody diarrhea, evidence of circulating antibody response to Escherichia coli O157 lipopolysaccharide, or other known risk factors for HUS, except for the presence of HIV infection. Each patient was treated with intravenous plasma infusion and renal replacement therapy. Their clinical course was characterized by non-oliguria and lack of significant hypertension throughout the acute phase of the disease. Despite these favorable clinical parameters, both patients developed end-stage renal failure. The etiology of this atypical HUS characterized by poor renal survival remains unknown and the role of HIV infection in its pathogenesis, although possible, is unclear. Received March 5, 1996; received in revised form and accepted October 15, 1996     

Quantitative measurement of lead in paint by XRF analysis without manual substrate correction

X-ray fluorescence analysis has been used for measurement of lead in paint for more than a decade. The early systems provided a nondestructive alternative technology to laboratory-based technologies, but were somewhat time consuming and often led to inconclusive results. The procedure required manual substrate correction, multiple measurements, operator's discretion in validating a measurement due to interfering elements and laboratory analysis of inconclusive samples. A new instrument, the RMD LPA-1 system, has been developed based on X-ray fluorescence technology that addresses all of the drawbacks to the older systems. This new system uses a carefully designed and controlled geometry and modern microprocessor technology to automatically provide a rapid quantitative measurement of lead in paint with a 95% confidence level. The improved precision and accuracy achieved with this system are due to geometric enhancements and a mathematical approach which incorporates corrections for both random and systematic errors such as matrix effects and Compton scatter. This technology has been incorporated in a hand-held X-ray fluorescence lead paint analyzer system. A key design philosophy for this system was to maintain a very narrow, task-specific focus, the system was not designed to be an all purpose XRF analyzer, rather it is optimized to meet regulatory requirements of lead paint testing in the most efficient manner. The development of the LPA-1 system is an example of what can be accomplished by listening to the needs and desires of the users, rethinking the design of an existing technique and incorporating modern microprocessor technology.     

Spontaneous regression of extramedullary plasmacytoma--a case report

A case of extramedullary plasmacytoma in the soft tissues of the posterior chest wall of an 80-year old man is reported herein. Immunofluorescence study showed that the tumor cells produced IgG lambda. An M-component was also detected in the patient's serum by paper electrophoresis. Two months following the open biopsy done to establish diagnosis, the tumor underwent spontaneous regression and the M-component in the serum also disappeared. This is the first case report of spontaneous regression of an extramedullary plasmacytoma and the probable reasons for this spontaneous regression are discussed herein.     

Role of the dopaminergic system in depression.

A hypothesis implicating dopamine in depression was proposed over 15 years ago (Randrup et al 1975). The identification of multiple new subtypes of dopamine receptors and evolving views regarding the function of the dopamine systems in the brain require a reexamination of this hypothesis. Results from studies in depression, Parkinson's disease, and animal models of depression suggest a deficiency of dopamine in depression. Dopamine precursors, dopamine agonists, and dopamine reuptake inhibitors show therapeutic efficacy in depression. Electroconvulsive therapy (ECT) and standard pharmacological antidepressants enhance dopamine function. Studies using receptor-specific drugs in clinical trials and neuroimaging studies are needed to further clarify the role of dopamine in depression.     

Gangrene of Meckel's diverticulum.

Gangrene of Meckel's diverticulum is uncommon and its pre-operative diagnosis is difficult. We report three cases with different presentations--simulating acute appendicitis, intestinal obstruction, and strangulation of the bowel.     

Whole-heart cine MRI using real-time respiratory self-gating.

Two-dimensional (2D) breath-hold cine MRI is used to assess cardiac anatomy and function. However, this technique requires cooperation from the patient, and in some cases the scan planning is complicated. Isotropic nonangulated three-dimensional (3D) cardiac MR can overcome some of these problems because it requires minimal planning and can be reformatted in any plane. However, current methods, even those that use undersampling techniques, involve breath-holding for periods that are too long for many patients. Free-breathing respiratory gating sequences represent a possible solution for realizing 3D cine imaging. A real-time respiratory self-gating technique for whole-heart cine MRI is presented. The technique enables assessment of cardiac anatomy and function with minimum planning or patient cooperation. Nonangulated isotropic 3D data were acquired from five healthy volunteers and then reformatted into 2D clinical views. The respiratory self-gating technique is shown to improve image quality in free-breathing scanning. In addition, ventricular volumetric data obtained using the 3D approach were comparable to those acquired with the conventional multislice 2D approach.     

Renal and vestibular toxicity due to inhaled tobramycin in a lung transplant recipient.

Chronic rejection is the major hurdle to long-term survival after lung transplantation. Endobronchial infection with Pseudomonas aeruginosa is common in patients with chronic rejection and this may further contribute to deterioration of the allograft. Inhaled tobramycin is commonly used to treat P aeruginosa airways infection in patients with cystic fibrosis. The safety of inhaled tobramycin in transplant recipients, however, has not been established. We describe the first report of a lung transplant recipient who developed renal failure and vestibular injury after receiving inhaled tobramycin. We review the literature regarding the safety of inhaled tobramycin and discuss potential mechanisms that may promote systemic toxicity in transplant recipients.     

Adult Wilms' tumor: report of a case and review of the literature

A 48 year old male presented with a one and a half year history of a progressively increasing, asymptomatic lump in the left hypochondrium and no history of hematuria. His general physical examination was unremarkable, and an abdominal examination revealed a large, firm, irregular and tender mass in the left hypochondrium extending into the lumbar region. Chest X-ray was normal. An intravenous urogram revealed a normally functioning right kidney with non-visualization of the left kidney. CT-scan of the abdomen revealed a large, mixed attenuating mass replacing the left kidney. At laparotomy, a large, fleshy, well-encapsulated tumor was found in the left kidney with no surrounding infiltration and a left radical nephrectomy was performed. Microscopic examination revealed a poorly differentiated tumor comprised of small round cells with focal areas of abortive embryonal tubular and glomerular differentiation suggestive of Wilms' tumor. The patient was advised chemotherapy and radiotherapy but he absconded and was lost to follow-up.