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     

Percentile regression analysis of correlated antibody responses

Antibody responses following vaccination usually are analysed by comparing geometric mean concentrations across levels of relevant covariates and by comparing the proportions of vaccinees responding. In the regression setting, the analyses are done on log-transformed concentrations, estimating geometric mean responses conditional on a vector of covariates. More detailed analyses examining the relationship of covariates to different parts of the response distribution may be performed through the application of asymmetric least squares estimation of regression percentiles. We present a method for accounting for correlation in percentile regression analyses of longitudinal antibody response data. We illustrate the procedures with measles antibody response data from Haitian children who participated in a randomized trial of high titre vaccines. The strongest dose and strain effects were seen in the low end of the antibody concentration distributions.     

Dislocation of the second through fifth carpometacarpal joints

We report a case of dislocation of the second through fifth carpometacarpal joints and review the mechanism of injury and radiographic findings in such injuries.     

Preliminary Evaluation of Sexual Problems in Combat Veterans with PTSD

This study evaluated the potential relationship between posttraumatic stress disorder (PTSD) and sexual problems. The Golombok Rust Inventory of Sexual Satisfaction was mailed to combat veterans currently in treatment at an outpatient PTSD clinic. Completed questionnaires were received from 90 patients. Results indicated that over 80% of subjects were experiencing clinically relevant sexual difficulties. Impotence and premature ejaculation were the most frequently reported problems that have corresponding psychological diagnoses. The rates of sexual problems for this sample of veterans with PTSD was similar to those reported in other studies and exceeded rates of similar problems found in samples from community samples. These data suggest mat PTSD may be a risk factor for sexual problems.     

Liver transplantation for erythropoietic protoporphyria liver disease.

In erythropoietic protoporphyria (EPP), there is excessive production of protoporphyrin, primarily in the bone marrow, resulting in increased biliary excretion of this heme precursor. Some patients will develop progressive liver disease that may ultimately require liver transplantation. However, excessive production of protoporphyrin by the bone marrow continues after transplantation, which may cause recurrent disease in the allograft. This study was performed to define post-transplant survival, the risk of recurrent disease, and specific management issues in patients transplanted for EPP liver disease. The patients studied consisted of twelve males and eight females, with an average age of 31 (range, 13-56) years at the time of transplantation. The estimated maximum MELD score prior to transplant was 21 (range, 15-29). Unique complications in the perioperative period were light induced tissue damage in four patients and neuropathy in six, requiring prolonged mechanical ventilation in four. Patient and graft survival rates were 85% at 1 year, 69% at 5 years, and 47% at 10 years. Recurrent EPP liver disease occurred in 11 of 17 patients (65%) who survived more than 2 months. Three patients were retransplanted at 1.8, 12.6, and 14.5 years after the initial transplant for recurrent EPP liver disease. In conclusion, the 5-year patient survival rate in patients transplanted for EPP liver disease is good, but the recurrence of EPP liver disease appears to diminish long term graft and patient survival.     

Development of a human cell line by selection and drug-metabolizing gene transfection with increased capacity to activate promutagens

We have isolated a human lymphoblastoid cell line with higher levels of native cytochrome P450IA1 activity and by DNA transfection introduced human cDNAs for a putative cytochrome P450IIA2 and epoxide hydrolase (E.C. 3.3.2.3). The resultant cell line, designated MCL-1, was substantially more sensitive to the mutagenicity of dimethylnitrosamine and benzo[a]pyrene than the AHH-1 cell line and was found to have increased metabolism of benzo[a]pyrene to dihydrodiols. The increase in native cytochrome P450IA1 activity was achieved by mutation and selection based on resistance to the phototoxicity of benzo[ghi]perylene. One resistant clone, designated L3, was used for subsequent studies. Two complete cDNAs, one encoding a putative cytochrome P450IIA2 and the other a microsomal epoxide hydrolase, were isolated from a human liver cDNA library. After introduction of the cDNAs into an expression vector and transfection into AHH-1 cells, gene expression was detected at the level of enzyme activity (epoxide hydrolase) or by increased sensitivity to dimethylnitrosamine cytotoxicity/mutagenicity (putative P450IIA2). A vector containing both cDNAs was then constructed and transfected into L3 cells to produce MCL-1 cells. The potential usefulness of drug-metabolizing gene transfection and of the MCL-1 cell line, in particular, for genetic toxicity testing is discussed.     

Substance Misuse Among Women Attending Family Planning Clinics in a Rural Area in Britain: prevalence and associated problems

Objectives: Little research has been carried out focusing on women who misuse substances, although existing evidence suggests that they represent a distinctive group. The aims of the current study are to determine prevalence of tobacco, alcohol and other drug use in women attending family planning services in a rural area and to investigate preferred sources of help-seeking. Methods: A prospective study using a self-completed questionnaire assessing use of tobacco, alcohol, other drugs, associated problems and sources of help-seeking. The sample included women attending family planning clinics in a rural area of southern England. Results: Fifty-three percent were current smokers,thirty-five percent misused alcohol and fourteen and a half percent had used other drugs in the last year. Those using each of the substances were significantly more likely to be 21 years or under. Not all those using substances indicated problems and sources of help varied with the presence of problems. Conclusions: Women attending family planing clinics are a self-selected group with a high level of substance use compared to the general population. This suggests that they may have a lifestyle incorporating more risky behaviours, including unsafe sexual behaviour. Once problems have occurred due to substance use, help needs to be more accessible.     

Rational approaches to the neurobiologic study of youth at risk for bipolar disorder and suicide

Objectives:  The aims of this paper are to provide an overview of neuroimaging findings specific to bipolar disorder and suicide, and to consider rational approaches to the design of future in vivo studies in youth at risk.
Methods:  Neuroimaging and related neurobiological literature pertaining to bipolar disorder and suicide in adult and pediatric samples was reviewed in a non-quantitative manner.
Results:  Specific structural and functional brain findings in bipolar disorder are described, where possible in the context of relevant current neurobiological theories of etiology. Diagnostic and prognostic implications are discussed.
Conclusions:  The simultaneous use of complementary neurobiological approaches may be a powerful way of identifying and validating factors reliably associated with bipolar disorder and suicide. A profile of neurobiological markers with which to screen for bipolar disorder and suicide risk may provide for earlier and more accurate diagnosis, perhaps even in the pre- or subsyndromal stages in high-risk youth.