Legg-Calvé-Perthes disease and the risk of injuries requiring hospitalization

Background and purpose Previous studies have suggested that Legg-Calvé-Perthes disease (LCPD) is associated with repetitive trauma, coagulation problems and anatomical abnormalities of the blood supply to the femoral head. The hypothesis that repetitive trauma can affect the blood supply of the femoral head, leading to LCPD, is supported by an animal model. For evidence of an increased risk of repetitive trauma, we investigated whether patients with LCPD have a higher risk for severe injuries requiring hospitalization. Patients and methods We identified 2579 patients with LCPD in Sweden during the period 1964-2005. 13,748 individuals without LCPD were randomly selected from the Swedish general population, matched by year of birth, sex and region (control group). Cox proportional hazard regression estimated the risks. Results Compared to the control group, patients with LCPD had a modestly raised hazard ratio (HR) of 1.2 (95% CI 1.1-1.3) for injury requiring hospitalization. The risks were slightly higher for soft tissue injuries (HR = 1.3, 95% CI:1.1-1.4) than for fractures (HR = 1.1, 95% CI: 1.0-1.3) and more pronounced among females. Compared to the control group, the higher risk for injury only applied to the lower extremities (HR = 1.2, 95% CI: 1.0-1.4) in patients with LCPD. Interpretation Patients with LCPD are vulnerable to injuries which could be interpreted as a marker of hyperactive behavior. It could also implicate that anatomical changes in the bone formation or blood supply of the femoral head - increasing its sensibility for trauma - contribute to the etiology of LCPD. © Copyright: ©Nordic Orthopaedic Federation

Maternal smoking during pregnancy and appetite control in offspring

Aims: Intrauterine exposure to tobacco smoke products has been associated with long-term neurobehavioral effects. Modified appetite control might explain the recently observed association between maternal smoking during pregnancy and obesity in offspring. Methods: Some 10,557 British adults aged 42 years born between 3-9 March 1958 were followed up in a birth cohort study (NCDS). The main outcome measure was self-reported poor appetite at age 42 years and main exposure was maternal smoking during pregnancy. Results: The proportion of offspring with poor appetite increased with maternal smoking during pregnancy: nonsmoking 4.5%; (4.0% - 5.0%), medium smoking 5.6%; (4.5 % - 6.8 %), variable smoking 6.8 %; (4.9 % - 9.1 %) and heavy smoking 7.7 %; (6.3 % - 9.4 %). The unadjusted odds ratios for maternal smoking during pregnancy (ever/never) and poor appetite is 1.49 (1.25 - 1.77) and after adjustment for BMI at 42 years and other potential confounding factors it is 1.22 (1.07 - 1.48). Conclusions: Offspring of mothers who smoked during pregnancy were more likely to report a poor appetite independent of a number of potential confounding factors. Although not in the expected direction, the results suggest maternal smoking during pregnancy may influence appetite perception through a developmental influence or through confounding by social factors

Maternal smoking during pregnancy, other prenatal and perinatal factors, and the risk of Legg-Calvé-Perthes disease

OBJECTIVE. The causes of Legg-Calvé-Perthes disease are largely unknown, but this pediatric disease seems to result from interruption of the blood supply to the proximal femur and is considered a vascular disease. Because maternal smoking during pregnancy influences fetal development and is associated with cardiovascular diseases in offspring, we hypothesized that this exposure is a risk for Legg-Calvé- Perthes disease and also investigated other markers of impaired fetal development and early-life exposures. MATERIALS AND METHODS. The Swedish Inpatient Register identified 852 individuals with a diagnosis of Legg-Calvé-Perthes disease from 1983 to 2005, individually matched by year of birth, age, sex, and region of residence with 4432 randomly selected control subjects. Linkage with the Swedish Medical Birth Register provided information on prenatal factors, including maternal smoking. Conditional logistic regression examined associations of maternal smoking during pregnancy and the other measures with the risk of Legg-Calvé-Perthes disease in offspring, adjusted for socioeconomic index and other potential confounding factors. RESULTS. Maternal smoking during pregnancy was associated with an increased Legg-Calvé-Perthes disease risk, and heavy smoking was associated with a risk increase of almost 100%. Very low birth weight and cesarean section were independently associated with ̃240% and 36% increases in the risk of Legg-Calvé-Perthes disease, respectively. CONCLUSION. Maternal smoking during pregnancy and other factors indicated by impaired fetal development may be associated with an increased risk of Legg-Calvé- Perthes disease

Cancer risk among patients with multiple sclerosis and their parents

Background: We investigated cancer risk among patients with multiple sclerosis (MS) and whether variation by age at MS diagnosis helps to elucidate mechanisms underlying the previously reported reduced cancer risk. We also studied cancer risk among parents to ascertain if MS susceptibility genes may confer protection against cancer in relatives. Methods: Cox proportional hazards regression, adjusted for age, sex, area, and socioeconomic index, estimated cancer risk among 20,276 patients with MS and 203,951 individuals without MS, using Swedish general population register data. Similar analyses were conducted among 11,284 fathers and 12,006 mothers of patients with MS, compared with 123,158 fathers and 129,409 mothers of controls. Results: With an average of 35 years of follow-up, there was a decreased overall cancer risk among patients with MS (hazard ratio = 0.91, 0.87-0.95). Increased risks were observed for brain tumors (1.44, 1.21-1.72) and urinary organ cancer (1.27, 1.05-1.53). Parents of patients with MS did not have a notably increased or decreased overall cancer risk. Conclusions: The reduction in cancer risk in patients with multiple sclerosis (MS) may result from behavioral change, treatment, or we speculate that some immunologic characteristics of MS disease activity improve antitumor surveillance. The lack of association among parents indicatesthat a simple inherited characteristic is unlikely to explain the reduced cancer risk among patients with MS. MS is associated with increased risk for some cancers, such as of urinary organs and brain tumors (although surveillance bias may be responsible). copyright © by AAN Enterprises, Inc

Cystic fibrosis gene mutations and gastrointestinal diseases

Background: This study examined if CF mutation heterozygosity is associated with diseases of gastrointestinal epithelial barrier function. Design and methods: Swedish registers identified 865 patients with a diagnosis of CF between 1968 and 2003 and matched with 8101 individuals without CF. Gastrointestinal disease risk was examined among 1534 biological parents and 1396 siblings of CF patients, compared with 15,526 parents and 15,542 siblings of individuals without CF. Results: First-degree relatives of CF patients were not at lower risk of the gastrointestinal diseases, in contrast with a raised risk among CF patients. Conclusion: Heterozygosity for CF gene mutations does not protect against gastrointestinal diseases where impaired barrier function may be relevant. © 2010 European Cystic Fibrosis Society

Cancer risk among patients with cystic fibrosis and their first-degree relatives

Patients with cystic fibrosis (CF) are at increased risk of some cancers. Little is known about the cancer risks among carriers heterozygous for the CF mutation and it is hypothesized this may be associated with reduced cancer risk. Using Swedish general population-based registers, we identified 884 patients with CF from 1968 to 2003 and 3,033 of their first-degree relatives The subjects were followed from birth of index persons or 1958, whichever came later, until death, emigration or 2003, whichever came first. Cancer risks were compared with the general Swedish population using standardized incidence ratios (SIR) with 95% confidence intervals (CI). Patients, followed for an average of 21 years, were at a higher overall risk of cancer. Some 26 cancer diagnoses, after excluding multiple diagnoses of nonmelanoma skin cancer in one man, produced an overall SIR of 3.2 (95%CI 2.1-4.6).We found statistically significantly increased risks for kidney, thyroid, endocrine, lymphoma and nonmelanoma skin cancer. There was no modification of cancer risk among parents and siblings, with an average of 21 years of follow-up. This study did not identify a heterozygote advantage for CF gene mutations in relation to cancer risk. © 2009 UICC

Bilateral Vallecular Cysts as a Cause of Dysphagia: Case Report and Literature Review

Cysts of the vallecula are rare, accounting for 10.5% to 20.1% of all laryngeal cysts. Vallecular cysts may present with diverse symptoms affecting the voice, airway, and swallowing. We describe the evaluation and treatment of a 70-year-old woman who presented with dysphagia caused by large bilateral vallecular cysts