The experience of caring for older people with dementia in a rural area: using services

This paper reports findings from the carer component of the Gwynedd Dementia Study. It is based on carer interviews, using quantitative and qualitative data. It confirms earlier findings that carers for people with dementia are typically female and older than other carers, although it notes that caring husbands are amongst the oldest carers. The problems that are most common are those that cause psychological stress to carers. Carers were found to receive both help and appreciation from their families and to perceive neighbours to be helpful if needed. In Gwynedd, as elsewhere, levels of formal service inputs are low, but most of the carers appeared to receive the services they needed. Problems are primarily associated with crisis support and long-term care is accepted reluctantly. It is suggested that community care dementia specialists could play a supporting role for carers.     

Cree leukoencephalopathy and CACH/VWM disease are allelic at the EIF2B5 locus

Cree leukoencephalopathy is a rapidly fatal infantile autosomal recessive leukodystrophy of unknown cause observed in the native North American Cree and Chippewayan indigenous population. We found in the brain of affected individuals the typical foamy cells with the oligodendroglial phenotype described in central hypomyelination syndrome/vanishing white matter, a syndrome related to mutations in the genes encoding the five subunits of the eucaryotic translation initiation factor eIF2B. In three patients of two Cree families, we found a homozygous missense mutation resulting in a histidine substitution at arginine 195 of epsilon-eIF2B.     

MR imaging and proton MR spectroscopy in adult Krabbe disease

We present the MR imaging findings in four patients (two pairs of siblings from two unrelated families) with adult Krabbe disease. In the first family, clinical presentation mimicked familial spastic paraplegia. Their MR images showed selective, increased signal intensity on T2-weighted sequences along the corticospinal tracts, most prominently in the proband and barely detectable in her brother. Proton MR spectroscopy showed increased choline and myo-inositol in the affected white matter. In the second family, the clinical presentation differed in that the signs of pyramidal tract involvement were asymmetrical, with concomitant asymmetry on MR images in one. In adults, Krabbe disease may present on MR imaging with selective pyramidal fiber involvement.     

HIV prevalence and risk behaviors among men who have sex with men and inject drugs in San Francisco

The dual risks of male-to-male sex and drug injection have put men who have sex with men and inject drugs (MSM-IDU) at the forefront of the HIV epidemic, with the highest rates of infection among any risk group in the United States. This study analyzes data collected from 357 MSM-IDU in San Francisco between 1998 and 2002 to examine how risk behaviors differ by HIV serostatus and self-identified sexual orientation and to assess medical and social service utilization among HIV-positive MSM-IDU. Twenty-eight percent of the sample tested HIV antibody positive. There was little difference in risk behaviors between HIV-negative and HIV-positive MSM-IDU. Thirty percent of HIV-positive MSM-IDU reported distributive syringe sharing, compared to 40% of HIV negatives. Among MSM-IDU who reported anal intercourse in past 6 months, 70% of positives and 66% of HIV negatives reported unprotected anal intercourse. HIV status varied greatly by self-identified sexual orientation: 46% among gay, 24% among bisexual, and 14% among heterosexual MSM-IDU. Heterosexual MSM-IDU were more likely than other MSM-IDU to be homeless and to trade sex for money or drugs. Gay MSM-IDU were more likely to have anal intercourse. Bisexual MSM-IDU were as likely as heterosexual MSM-IDU to have sex with women and as likely as gay-identified MSM-IDU to have anal intercourse. Among MSM-IDU who were HIV positive, 15% were currently on antiretroviral therapy and 18% were currently in drug treatment, and 87% reported using a syringe exchange program in the past 6 months. These findings have implications for the development of HIV interventions that target the diverse MSM-IDU population.     

The role of parenteral nutrition

Patients with acute leukemia who undergo hematopoietic stem cell transplantation (HSCT) are susceptible to malnutrition caused by several factors including intensive cytotoxic therapy. This paper discusses the significance of malnutrition in these patients and provides an overview of nutrition therapy by the oral, enteral, and parenteral routes. The goal is to investigate whether the use of parenteral nutrition (PN) produces improved clinical outcomes in patients with acute leukemia and to identify criteria for the selection of patients most likely to benefit from this therapy. Although PN may be appropriate for patients suffering from complications such as graft-versus-host disease (GVHD) and mucositis, the data available at this time do not support PN as first-line therapy for all recipients of HSCT.     

Bombesin receptor subtypes in human cancers: detection with the universal radioligand (125)I-[D-TYR(6), beta-ALA(11), PHE(13), NLE(14)] bombesin(6-14).

PURPOSE: Bombesin and bombesin receptors have been shown to play a role in cancer. Whereas the gastrin-releasing peptide (GRP) receptor is a bombesin receptor subtype frequently expressed by tumors, the other three subtypes, the neuromedin B (NMB), BB3, and BB4 receptors, have been poorly investigated in human tissues. EXPERIMENTAL DESIGN: We investigated 161 human tumors for their bombesin receptor subtype expression using in vitro receptor autoradiography with the universal bombesin radioligand (125)I-[D-Tyr(6), beta-Ala(11), Phe(13), Nle(14)]bombesin(6-14) in displacement experiments with unlabeled GRP, bombesin, NMB, and [D-Tyr(6), beta-Ala(11), Phe(13), Nle(14)]bombesin(6-14). The distinct rank order of potencies of these analogues for each receptor subtype allows us to identify the predominant subtype expressed by each tumor. RESULTS: Twelve of 12 prostate cancers, 41 of 57 breast cancers, and 5 of 5 gastrinomas expressed predominantly GRP receptors; 11 of 24 intestinal, 1 of 26 bronchial, and 1 of 1 thymic carcinoids had preferentially NMB receptors; 9 of 26 bronchial carcinoids, 1 large cell neuroendocrine lung carcinoma, and 4 of 9 small cell lung carcinomas had preferentially BB3 receptors, whereas 3 of 9 small cell lung carcinomas had GRP receptors. Renal cell carcinomas had GRP receptors in 6 of 16 cases and BB3 receptors in 4 of 16 cases. Finally, 2 of 10 Ewing sarcomas had BB3 receptors. In situ hybridization detected BB3 receptor mRNA in neuroendocrine tumors expressing the BB3 protein. CONCLUSIONS: This is the first study detecting the proteins of BB3, NMB, and GRP receptors in a group of human tumors using differential binding techniques. Particularly relevant is the BB3 expression in lung carcinoids and other neuroendocrine lung tumors, whereas gastrointestinal carcinoids preferably express NMB receptors. These tumors may be targets for diagnostic and radiotherapeutic applications of subtype-selective bombesin analogues.     

Effects of dietary cholesterol restriction in a feline model of Niemann-Pick type C disease

A feline model of Niemann–Pick disease type C (NPC) was employed to evaluate the effect of dietary cholesterol restriction on progression of disease. Two NPC-affected treated cats were fed a cholesterol-restricted diet beginning at 8 weeks of age; the cats remained on the diet for 150 and 270 days respectively. The study goal was to lower the amount of low density lipoprotein (LDL) available to cells, hypothetically reducing subsequent lysosomal accumulation of unesterified cholesterol and other lipids. Neurological progression of disease was not altered and dietary cholesterol restriction did not significantly decrease storage in NPC-affected treated cats. One NPC-affected treated cat had decreased serum alkaline phosphatase activity (ALP) and decreased serum cholesterol concentration. Liver lipid concentrations of unesterified cholesterol, cholesterol ester and phospholipids in NPC-affected treated cats were similar to those seen in NPC-affected untreated cats. Ganglioside concentrations in the NPC-affected treated cats and NPC-affected untreated cats were similar. Histological findings in liver sections from NPC-affected treated cats showed a diffuse uniform microvacuolar pattern within hepatocytes and Kupffer cells, in contrast to a heterogeneous macro/microvacuolar pattern and prominent nodular fibrosis in NPC-affected untreated cats. Similar differences in vacuolar patterns were seen in splenic macrophages. Although some hepatic parameters were modified, dietary cholesterol restriction did not appear to alter disease progression in NPC-affected kittens.