Hepatic fibrosis in fetal alcohol syndrome. Pathologic similarities to adult alcoholic liver disease

The light- and electron-microscopic changes in a biopsy of the liver obtained at age 17 mo from a child with fetal alcohol syndrome were studied. At the time of biopsy, hepatomegaly and raised serum transaminases were present as well as neurologic and growth defects and the typical facial anomalies seen in this syndrome. Light microscopy of the liver biopsy specimen revealed parenchymal fat with portal and perisinusoidal fibrosis. Ultrastructurally, perisinusoidal spaces contained deposits of intermediate-size and large collagen fibers, myofibroblasts and occasional Ito cells, and subendothelial basement membrane-like material. These changes resemble those seen in adult human and baboon alcoholic liver disease and suggest that hepatic fibrosis in fetal alcohol syndrome has a similar pathogenesis.     

Investigating the role of heparin sulfate proteoglycans in hereditary multiple exostoses (HME) tumourigenesis

Introduction Heparin sulfate (HS) has long been implicated in the bone deformity hereditary multiple exostoses (HME), and it is now clear that HME is associated with mutations in the HS biosynthetic genes EXT1 and EXT2. Interestingly, HME is also associated with an increased risk of chondro‐ and osteo‐sarcomas. Methods and results Preliminary analysis of GAG samples purified from fibroblasts of both HME and isolated non‐HME exostoses patients reveal a dramatic shift in the ratio of CS : HS, with the HME and isolated cases having a much higher proportion of CS relative to normal controls. This is true in the case of both shed and cell surface material but is far more extreme in the latter, with the HS reducing from approximately 45% in the controls to less than 10% in HME patients. Initial analysis also reveals shortened chain length within these samples; indeed they often have two populations of chains present. Simple analysis of the total disaccharide composition of these samples demonstrates no significant differences against controls. However, detailed analysis of the subpopulations of chains (as determined by chain length) within these samples as well as cartilaginous samples from exostoses patients may provide further insight into the changes that occur within the biosynthetic pathway following disrupted EXT function. We are also carrying out immunocytochemistry with a variety of HS‐specific antibodies with the aim to further investigate normal HS structure and localization. This is being carried out on human primary chondrocytes isolated from normal patients and also adult mesenchymal stem cells as they undergo differentiation into chondrocytes. HS has been identified in both these cell types, and it is hoped that the manipulation of these cells through RNAi of different enzymes of the HS biosynthetic pathway will provide a suitable model for studying what changes may occur in cellular HS structures over the initial differentiation process in the growth plate. Discussion Together, these investigations should provide a good model to allow us to determine the role of HS in chondrocyte differentiation and maturation in both normal and diseased states.     

FS13.5Occupational contact dermatitis: printer worker's viewpoints

Introduction:  Occupational contact dermatitis (OCD) is very common in the printing industry due to contact with chemicals, paper, and wet work. It can be avoided by adequate protective measures, but the effectiveness of intervention depends heavily on the employer's and employee's awareness of this health risk.
Objectives:  The study aimed to collect information on the knowledge, attitudes and beliefs of print workers about the risk of OCD and methods of prevention.
Methods:  A series of focus groups were held with print workers, health and safety officers and managers to discuss their awareness of dermal risk factors, risk behaviour at work, attitudes to health and safety and options on possible preventive measures. A number of companies were also visited to observe, overtly and covertly, the normal work practices.
Results:  OCD was not perceived to be either a major problem or a health and safety priority. There was general agreement about the processes and work practices that could cause skin problems. However, work practices varied considerably and did not always reflect this awareness. There was general concern about the type and availability of personal protective equipment, especially gloves and after‐work skin cream. The provision of an occupational health service was generally felt to be inadequate, and no company had a policy in place that specifically addressed skin care.
Conclusions:  These findings highlight the urgency to intensify health and safety education on skin care within the printing industry. Recommendations were developed for the evaluation of a series of risk reduction strategies.     

The dietary intake of homeless males sleeping rough in Central London

Data concerning the food and beverage intake (including alcohol) of 79 males, aged 19–50 years, sleeping rough in Central London, who did not appear to be under the influence of drink, drugs or suffering from mental illness were collected by prompted 24-h recall and food-frequency questionnaire (FFQ). The source of food was noted and the subjects were asked about food availability and food needs. The group had intakes significantly lower than Dietary Reference Values (DRV) for the UK for energy, carbohydrate, non-starch polysaccharides (NSP), folate, zinc and magnesium. Significantly higher values were seen for protein, fat, vitamin B₁₂, calcium and iron. Alcohol intake was higher than that assumed to approximate current intakes of the UK population. Mean macronutrient intakes for the group were similar to means for British adult men (OPCS, 1990), only protein and NSP were significantly lower. The FFQ showed very low consumption/week of fruit, vegetables, salad, fruit juice and wholewheat/brown products. Organized charities provided the major source of food energy for the group as a whole. Self-supplied sources provided the major source of total energy. It is suggested that the group may benefit from an increase in dietary fibre and energy, with an increase in the proportion of energy supplied by carbohydrate and a reduction in energy from fat and alcohol. A varied supply of food and hot meals during weekends as well as during the week may benefit the group both aesthetically and nutritionally. It may be advisable to increase the supply of wholemeal/brown bread, fruits, vegetables and salad foods without decreasing the energy content of the food available.     

Health-related quality of life and pressure ulceration assessment in patients treated in the community

Little is known of the impact of pressure ulceration on adult patients' health-related quality of life. The purpose of this study was to determine the impact pressure ulceration has on pressure ulcer patients cared for in the community. A case control study design was used by drawing a random sample from patients receiving community nursing care, stratified by the presence of pressure ulceration. In all, 75 patients with pressure ulcers were compared with 100 controls without ulcers using the four-point ulcer grading scale described by United Kingdom consensus guidelines. Patients were interviewed using the Short Form-36 (SF-36) questionnaire and activities of daily living assessed using the modified Barthel scale. Patients with pressure ulcers had significantly poorer physical function (mean difference (d) = 37.6, 95% CI 28.6-46.6, p < 0.001) and social functioning (d = 33.9, 95 % CI 24.0-43.9, p < 0.001) than published age- and sex-matched normative data from the United Kingdom. The difference between cases and controls was much smaller in these domains, with neither approaching statistical significance. After adjustment for age and gender, scores for bodily pain were poorer in patients with no ulceration (d = -10.5, 95% CI - 20.6 to - 0.4, p = 0.042) indicating greater pain in these patients compared with the cases with ulceration. Activities of daily living determined by the modified Barthel scale showed reduced self-care (d = -7.6, 95% CI -12.5 to - 2.7, p = 0.010) and mobility (d = -9.2, 95% CI -14.6 to - 3.8, p = 0.001) in patients with pressure ulceration. The overall ability to perform these activities was also significantly poorer in this group (d = -16.3, 95% CI -27.3 to -5.3, p = 0.004). While patients with pressure ulceration experience some deficits in their health-related quality of life compared with a normal population, these differences are similar to those experienced by other patients receiving community nursing care.     

Three novel PROC gene lesions causing protein C deficiency

Hallam PJ, Mannucci P, Tripodi A, Bevan D, Laursen B, Tengborn L, Wacey A, Cooper DN. Three novel PROC gene lesions causing protein C deficiency. Clin Genet 1998: 54: 231–233. 0 Munksgaard, 1998
Missense mutations. three of them novel (Am210→Val, Asn248→ Ile, Ah355→Val), were found in the protein c ( PROC ) genes of 7 patients with inherited protein C deficiency associated with venous thrombosis. Comparison with the phenotypic effects of mutations in the analogous residues of factor IX causing hdernophilia B and the use of molecular modelling has provided explanations as to how these lesions might alter either the structure, function or secretion of the protein C molecules encoded.