An atypical Hallermann-Streiff syndrome. Focus on dental care and differential diagnosis.

The Hallermann-Streiff syndrome (HSS) is a rare congenital disorder characterized by dyscephaly, birdlike facies, hypoplastic mandible, congenital cataracts, microphthalmia, hypotrichosis, skin atrophy, proportionate short stature, and dental anomalies. A case of a 29-year-old man with atypical HSS with neither cataracts, hair and skin alterations, nor short stature is reported, with special consideration to oral findings and dental management. Dental extractions, scaling, restorations, and endodontics were performed under local anesthesia. Later, orthodontic rehabilitation with fixed brackets was carried out. Finally, a removable partial denture for the maxillary arch was designed using transparent acrylic, and this also served as a retention splint. Young patients with HSS and other similar syndromes must be involved in personalized oral health prevention programs as early as possible. Despite numerous systemic anomalies, some of these patients may undergo conventional dental procedures under local anesthesia in the dental office.     

Geographical patterns of childhood cancer incidence in Europe, 1988-1997. Report from the Automated Childhood Cancer Information System project

Data on more than 50,000 registrations in the Automated Childhood Cancer Information System (ACCIS) database were used to present an overview of regional patterns in childhood cancer incidence in Europe during 1988-1997, and to present additional detail on selected carcinomas whose occurrence in childhood is seldom described because of their rarity. Total age-standardised incidence was 138.5 per million for Europe overall, and varied between regions from 131.1 per million in the British Isles to 160.1 per million in Northern Europe. Incidence varied significantly between regions for nearly all diagnostic groups. The greatest range of regional incidence rates was for central nervous system (CNS) tumours, from 27.0 per million in the West to 43.8 per million in the North. Differences in registration practice for non-malignant tumours account for some of this variation. There was a marked excess of carcinoma in Eastern Europe, which was wholly attributable to the high incidence of thyroid carcinoma in Belarus, though there was also evidence of inter-regional variation attributable to differences in registration practice. The geographical heterogeneity of incidence rates for other diagnostic groups seems more likely to reflect variations in underlying risk.     

Ambient fine particles modify heart rate variability in young healthy adults

Particulate air pollution has been related with cardiopulmonary morbidity and mortality. Recent studies have shown that an increase in particulate matter (PM)(2.5) ambient concentrations was associated with a decrease in heart rate variability (HRV) in the elderly with cardiovascular conditions, which could increase the risk of death. In order to assess if this association could also be observed in young adults, we studied 40 young healthy residents of the Mexico City Metropolitan Area (MCMA) who underwent 13 h Holter electrocardiographic and PM(2.5) personal monitoring. HRV was evaluated in time domain: the standard deviation of normal RR intervals (SDNN) and the percentage of differences between adjacent normal RR intervals larger than 50 ms (pNN50). In multivariate analysis with mixed effects models, a significant negative association of pNN50 with PM(2.5) accumulative exposure was found. An increase in 30 microg/m(3) of the average PM(2.5) personal exposure in the previous 2 h decreased the pNN50 in 0.08% (P=0.01). This observation revealed an acute effect related to environmental exposure to PM(2.5) with regard to HRV in normal youngsters. The long-term health consequences of this association in young healthy adults remain to be clarified.     

Fish consumption and breast cancer risk. The European Prospective Investigation into Cancer and Nutrition (EPIC)

There is current interest in fish consumption and marine omega-3 (n-3) fatty acids and breast cancer risk. Some in vitro and animal studies have suggested an inhibitory effect of marine n-3 fatty acids on breast cancer growth, but the results from epidemiological studies that have examined the association between fish consumption and breast cancer risk in humans are inconsistent. We examined fish consumption and breast cancer risk in 310,671 women aged between 25 and 70 yr at recruitment into the European Prospective Investigation Into Cancer and Nutrition (EPIC). The participants completed a dietary questionnaire between 1992-98 and were followed up for incidence of breast cancer for a median of 6.4 yr. Hazard ratio for breast cancer by intake of total and lean and fatty fish were estimated, stratified by study centre and adjusted for established breast cancer risk factors. During follow-up, 4,776 invasive incident breast cancers were reported. No significant associations between intake of total fish and breast cancer risk were observed, hazard ratio (HR) 1.01 (95% confidence interval [CI] 0.99-1.02; p = 0.28 per 10 g fish/day). When examining lean and fatty fish separately, we found a positive significant association only in the highest quintile for fatty fish (HR 1.13, 95% CI 1.01-1.26), but test for trend was not significant (p = 0.10). No associations with breast cancer risk were observed when the study participants were subdivided by menopausal status. Although the period of follow-up is relatively short, the results provide no evidence for an association between fish intake and breast cancer risk.     

Serum C-peptide levels and breast cancer risk: results from the European Prospective Investigation into Cancer and Nutrition (EPIC)

It has been hypothesized that chronic hyperinsulinemia, a major metabolic consequence of physical inactivity and excess weight, might increase breast cancer risk by direct effects on breast tissue or indirectly by increasing bioavailable levels of testosterone and estradiol. Within the European Prospective Investigation into Cancer and Nutrition (EPIC), we measured serum levels of C-peptide--a marker for pancreatic insulin secretion--in a total of 1,141 incident cases of breast cancer and 2,204 matched control subjects. Additional measurements were made of serum sex hormone binding globulin (SHBG) and sex steroids. Conditional logistic regression models were used to estimate breast cancer risk for different levels of C-peptide. C-peptide was inversely correlated with SHBG and hence directly correlated with free testosterone among both pre and postmenopausal women. C-peptide and free estradiol also correlated positively, but only among postmenopausal women. Elevated serum C-peptide levels were associated with a nonsignificant reduced risk of breast cancer diagnosed up to the age of 50 years [odds ratio (OR)=0.70, (95% confidence interval (CI), 0.39-1.24); ptrend=0.05]. By contrast, higher levels of C-peptide were associated with an increase of breast cancer risk among women above 60 years of age, however only among those women who had provided a blood sample under nonfasting conditions [OR=2.03, (95% CI, 1.20-3.43); ptrend=0.01]. Our results do not support the hypothesis that chronic hyperinsulinemia generally increases breast cancer risk, independently of age. Nevertheless, among older, postmenopausal women, hyperinsulinemia might contribute to increasing breast cancer risk.     

Polymorphisms of genes coding for insulin-like growth factor 1 and its major binding proteins, circulating levels of IGF-I and IGFBP-3 and breast cancer risk: results from the EPIC study

Insulin-like growth factor I (IGF-I) stimulates cell proliferation and can enhance the development of tumours in different organs. Epidemiological studies have shown that an elevated level of circulating IGF-I is associated with increased risk of breast cancer, as well as of other cancers. Most of circulating IGF-I is bound to an acid-labile subunit and to one of six insulin-like growth factor binding proteins (IGFBPs), among which the most important are IGFBP-3 and IGFBP-1. Polymorphisms of the IGF1 gene and of genes encoding for the major IGF-I carriers may predict circulating levels of IGF-I and have an impact on cancer risk. We tested this hypothesis with a case-control study of 807 breast cancer patients and 1588 matched control subjects, nested within the European Prospective Investigation into Cancer and Nutrition. We genotyped 23 common single nucleotide polymorphisms in IGF1, IGFBP1, IGFBP3 and IGFALS, and measured serum levels of IGF-I and IGFBP-3 in samples of cases and controls. We found a weak but significant association of polymorphisms at the 5' end of the IGF1 gene with breast cancer risk, particularly among women younger than 55 years, and a strong association of polymorphisms located in the 5' end of IGFBP3 with circulating levels of IGFBP-3, which confirms previous findings. Common genetic variation in these candidate genes does not play a major role in altering breast cancer risk in Caucasians.     

Prevalence of depression and associated medical and psychosocial factors in elderly hospitalized patients with heart failure in Spain

INTRODUCTION AND OBJECTIVES: This study provides an estimate of the prevalence of depression, and identifies associated medical and psychosocial factors, in elderly hospitalized patients with heart failure (HF) in Spain. METHODS: The study included 433 patients aged 65 years or more who underwent emergency admission at four Spanish hospitals between January 2000 and June 2001 and who had a primary or secondary diagnosis of HF. Depression was defined as the presence of three or more symptoms on the 10-item Geriatric Depression Scale. RESULTS: In total, 210 (48.5%) study participants presented with depression: 71 men (37.6%) and 139 women (57.0%). Depression was more common in patients with the following characteristics: NYHA functional class III-IV (adjusted odds ratio or aOR=2.00, 95% confidence interval or 95% CI, 1.23-3.24), poor score on the physical domain of the quality-of-life assessment (aOR=3.14; 95% CI, 1.98-4.99), being dependent for one or two basic activities of daily living (BADLs) (aOR=2.52; 95% CI, 1.41-4.51), being dependent for > or =3 BADLs (aOR=2.47; 95% CI, 1.20-5.07), being limited in at least one instrumental activity of daily living (aOR=2.20: 95% CI, 1.28-3.79), previous hospitalization for HF (aOR=1.71; 95% CI, 1.93-5.45), spending more than 2 hours/day alone at home (aOR=3.24; 95% CI, 1.93-5.45), and being dissatisfied with their primary care physician (aOR=1.90; 95% CI, 1.14-3.17). CONCLUSIONS: Depression is very common in elderly hospitalized patients with HF and is associated with several medical and psychosocial factors. The high prevalence of depression, the poorer prognosis for HF in patients with depressive symptoms, and the existence of simple diagnostic tools and effective treatment argue in favor of systematic screening for depression in these patients.