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     

Malignant schwannoma with melanocytic and neuroepithelial differentiation in an infant with congenital giant melanocytic nevus: A complex neurocristopathy

We describe an infant girl, born with a pigmented giant nevus, who developed a malignant schwannoma in the retroperitoneum at 16 months of age. At birth the nevus covered over 50% of her body and histologically was a compound nevus with extension into the deep dermis surrounding dermal appendages. The malignant schwannoma was biphasic with areas composed of spindle and round cells. Ultrastructurally, the majority of the tumor cells exhibited a Schwann cell phenotype, but neuroepithelial and melanocytic cells were identified as well. We believe that this constellation of findings represents a form of neurocristopathy. Neurocristopathy, as defined by Bolande (Hum Pathol 5:409–429, 1974), is a disease that results from aberrations in the migration, growth, or cytodifferentiation of neural crest tissues. These diseases may be simple (a singular pathologic process, usually localized) or complex (multiple neuroectodermal lesions). We report this case because the occurrence of retroperitoneal malignant schwannoma arising in a 16-month-old infant born with a pigmented giant nevus is unique, and may represent a previously undescribed form of a complex neurocristopathy.     

Mortality from asthma in children and young adults in Louisiana.

BACKGROUND: Asthma mortality rates have been increasing despite our improved understanding of the pathophysiology of asthma and advanced methods of treatment. Although there are many national studies of asthma mortality rates, few studies have concentrated on subnational regions such as individual states. OBJECTIVES: We sought to determine the trends in asthma mortality in children and young adults during the last 15 years in Louisiana. METHODS: Asthma mortality data were acquired from the Louisiana State Center for Health Statistics for the years 1983 through 1997. All asthma deaths coded International Classification of Disease, Ninth Revision 493 were included in the data set along with year, sex, and rate characteristics. State population estimates obtained from the United States Bureau of Census for three 5-year periods (1983 to 1987, 1988 to 1992, and 1993 to 1997) were used to calculate crude and adjusted mortality rates. These time periods were compared with one another and with the period 1983 to 1987, which was used as a baseline. RESULTS: For all residents, the mortality rate increased from 0.41 deaths per 100,000 persons during the 1983 to 1987 time period to 0.49 and 0.63 deaths per 100,000 persons during 1988 to 1992 and 1993 to 1997 time periods, respectively. Mortality rates have increased in both the white and non-white populations over the 15-year study period, with the most pronounced increases among the non-white population. CONCLUSION: Asthma mortality rates in Louisiana have increased, particularly among the non-white population, over the last 15 years, although the results are not statistically significant. When compared with other states, asthma mortality in Louisiana is lower than in the northeast and central north states.     

The impact of the herpetic eye disease studies on the management of herpes simplex virus ocular infections

Herpes simplex virus (HSV) is a leading cause of chronic infectious ocular disease in the United States. The morbidity from recurrent herpetic episodes is high, and the resultant corneal scarring may require penetrating keratoplasty for visual rehabilitation. Effective treatments for acute episodes of HSV have been verified by early Herpetic Eye Disease Study (HEDS) trials. The recent HEDS trial on the efficacy of oral acyclovir as prophylaxis against recurrent stromal keratitis represents the first report of a treatment likely to reduce long-term scarring from herpetic disease. This article reviews all the HEDS trials and the implications of their findings for the management of patients with ocular HSV.     

Reductions in clinical inflammation and oral neutrophils with improving oral hygiene

Clinical Oral Investigations - This study compared the effects of oral hygiene with a toothpaste formulated with zinc (test) to a fluoride dentifrice (control) for effects on oral polymorphonuclear...     

Unruptured sinus of valsalva aneurysm presenting as acute coronary syndrome

An unusual presentation of sinus of Valsalva aneurysm causing right ventricular outflow tract obstruction and presenting as acute coronary syndrome is reported. A 38-year-old lady presented with ischemic chest pain, probably due to embolization from an unruptured sinus of Valsalva aneurysm.