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Selected AbstractsFormal support, mental disorders and personal characteristics: a 25-year follow-up study of a total cohort of older peopleHEALTH & SOCIAL CARE IN THE COMMUNITY, Issue 2 2003G. Samuelsson PhD Abstract The present study was designed to describe the pattern of long-term formal support received by people with mental disorders, and to investigate the relationship between the medical, psychological and social characteristics of the participants and types of formal support. This study is based on a cohort (n = 192) of people born in 1902 and 1903 in a community in Southern Sweden. The research participants were assessed using interviews, psychological tests and medical examinations. Information was collected about the use of primary healthcare and social services. The first assessment took place when the cohort was aged 67 years, and then on eight further occasions until they were 92. The participation rate ranged from 72% to 100%. During the observation period of 25 years, 53% of people with dementia eventually received both home help and institutional care compared to 34% of people with other psychiatric diagnoses and 12% of people with good mental health. The last group had all physical health problems and/or problems with activities of daily living. However, 35% of the dementia group, 46% with other psychiatric diagnoses and 52% of people with good mental health did not receive any formal support. Males and self-employed people were significantly less likely to use formal support. The institutionalised group reported loneliness significantly more often than the other two groups. In a logistic regression analysis, loneliness, low social class, high blood pressure and low problem-solving ability were predictors of formal support use. People with a mental disorder, including dementia, were significantly more likely to use formal support compared to people with good mental health. Social factors were the main factors predicting formal support. [source] Insulin resistance and liver injury in hepatitis C is not associated with virus-specific changes in adipocytokines,HEPATOLOGY, Issue 1 2007Ian Homer Y. Cua The role of tumor necrosis factor ,, interleukin 6, leptin, and adiponectin in the pathogenesis of hepatitis C virus (HCV)-associated insulin resistance (IR) remains controversial. We tested the hypothesis that these adipocytokines contribute to chronic HCV-associated IR and liver injury by first comparing their serum levels and homeostasis model assessment of insulin resistance (HOMA-IR) in 154 untreated, non-diabetic, HCV-infected male subjects with fibrosis stage 0-2, to that in 75 healthy volunteers matched for age, body mass index (BMI), and waist-hip ratio (WHR). We next examined whether the adipocytokine levels were associated with the extent of hepatic steatosis, portal/periportal inflammation and fibrosis in our total cohort of 240 HCV-infected male subjects. Significantly higher levels of HOMA-IR (2.12 versus 1.63, P = 0.01), TNF, (1.28 versus 0.60 pg/ml, P < 0.001) and IL6 (2.42 versus 1.15 pg/ml, P = 0.001) were noted in the HCV cohort compared with healthy controls respectively, but there were no significant differences in leptin and adiponectin concentrations. By multiple linear regression, independent predictors of HOMA-IR included the body mass index, and the serum levels of leptin (positive correlation) and adiponectin (negative correlation), but not that of TNF, and IL6. Only TNF, levels were correlated with the extent of histological injury (portal/periportal inflammation, P = 0.02). Conclusion: Whereas leptin and adiponectin contribute to IR, none of the adipocytokines accounted for the elevated IR in HCV-infected subjects. The adipocytokines were not associated with histological features of chronic HCV infection except for TNF, which correlated with portal/periportal inflammation. HCV-associated IR is most likely an adipocytokine-independent effect of the virus to modulate insulin sensitivity. (HEPATOLOGY 2007;46:66,73.) [source] Determination of the mutation spectrum of the EXT1/EXT2 genes in British Caucasian patients with multiple osteochondromas, and exclusion of six candidate genes in EXT negative cases,,HUMAN MUTATION, Issue 11 2006Lorne Lonie Abstract We describe here the spectrum and distribution of mutations in the EXT1 and EXT2 genes in the largest reported British Caucasian multiple osteochondromas (MO) population. Furthermore, we report for the first time the screening of the EXT1 and EXT2 promoters, 5,UTRs, and 3,UTRs, and exclude six potential MO candidate genes in individuals without a detectable mutation within the coding region of EXT1 and EXT2. The coding exons of EXT1 and EXT2 were screened in 72 unrelated probands affected with MO. Forty-six different mutations were identified in 56 probands, of which 29 were novel. Mutation in the EXT1 and EXT2 genes each accounted for 50% of the mutations identified. Of the 72 probands, 42 were of British Caucasian descent, which when added to the 41 British Caucasian families previously reported from our total cohort, gave a total of 83 families. This cohort's proportional frequency for EXT1/EXT2 mutation was 53%/47%. We also validated the technique of high-resolution melting analysis in a blind study using 27 unique EXT1 or EXT2 mutations. This technique was found to be sensitive with a detection rate of 100% regarding heterozygote detection for EXT mutation scanning. Furthermore, this technique has a very high throughput and is very cost-effective. © 2006 Wiley-Liss, Inc. [source] Impaired Fasting Glucose Is Associated with Increased Arterial Stiffness in Elderly People without Diabetes Mellitus: The Rotterdam StudyJOURNAL OF AMERICAN GERIATRICS SOCIETY, Issue 3 2006Nicole M. Van Popele MD OBJECTIVES: To study the association between impaired fasting glucose (IFG) and arterial stiffness in older adults. DESIGN: Cross-sectional population-based study. SETTING: The Rotterdam Study, a Dutch population-based cohort study. PARTICIPANTS: Two thousand nine hundred eighty-seven subjects aged 60 and older. MEASUREMENTS: Arterial stiffness assessed by measuring common carotid arterial distensibility and glucose status classified into three categories: normal fasting glucose (NFG) (fasting glucose <6.1 mmol/L), IFG (fasting glucose 6.1,6.9 mmol/L), and diabetes mellitus (DM). RESULTS: In the total cohort, common carotid distensibility decreased with increasing impairment of glucose metabolism. Subjects younger than 75 with IFG were comparable with subjects with NFG with respect to arterial stiffness. Subjects aged 75 and older with IFG had stiffer arteries than subjects with NFG, reaching the same arterial stiffness as subjects with DM. For subjects younger than 75, mean difference in distensibility coefficient between subjects with NFG and with IFG was 0.1 (95% confidence interval (CI)=,0.04,0.05, P=.88) and between subjects with NFG and with DM was 1.2 (95% CI=0.7,1.7, P<.001). For subjects aged 75 and older, the mean difference between these groups was 0.7 (95% CI=0.2,1.2, P=.007) and 0.8 (0.3,1.4; P=.002), respectively. In the total cohort, fasting glucose was strongly associated with carotid distensibility (,-coefficient=,0.29, P<.001). CONCLUSION: IFG is related to arterial stiffness in elderly subjects. An advanced stage of arterial stiffness, comparable with that of subjects with DM, is only reached at the age of 75. [source] Cognitive profile in a large french cohort of adults with Prader,Willi syndrome: differences between genotypesJOURNAL OF INTELLECTUAL DISABILITY RESEARCH, Issue 3 2010P. Copet Abstract Background Prader,Willi syndrome (PWS) is a rare genetic disorder characterised by developmental abnormalities leading to somatic and psychological symptoms. These include dysmorphic features, impaired growth and sexual maturation, hyperphagia, intellectual delay, learning disabilities and maladaptive behaviours. PWS is caused by a lack of expression of maternally imprinted genes situated in the 15q11-13 chromosome region. The origin is a ,de novo' deletion in the paternal chromosome in 70% of the cases and a maternal uniparental disomy in 25%. The two main genotypes show differences, notably regarding cognitive and behavioural features, but the mechanisms are not clear. This study assessed cognitive impairment in a cohort of adults with genetically confirmed PWS, analysed their profiles of cognitive strengths and weaknesses, and compared the profiles in terms of genotype. Methods Ninety-nine male and female adults participated, all inpatients on a specialised unit for the multidisciplinary care of PWS. The Wechsler Adult Intelligence Scale (WAIS-III) was administered to all patients in identical conditions by the same psychologist. Eighty-five patients were able to cope with the test situation. Their scores were analysed with non-parametric statistical tools. The correlations with sex, age and body mass index were explored. Two genotype groups were compared: deletion (n = 57) and non-deletion (n = 27). Results The distribution of intelligence quotients in the total cohort was non-normal, with the following values (medians): Full Scale Intelligence Quotient (FSIQ): 52.0 (Q1:46.0; Q3:60.0), Verbal Intellectual Quotient (VIQ): 53.0 (Q1:48; Q3:62) and Performance Intellectual Quotient (PIQ): 52.5 (Q1:48; Q3:61). No correlation was found with sex, age or body mass index. Comparison between groups showed no significant difference in FSIQ or VIQ. PIQ scores were significantly better in the deletion group. The total cohort and the deletion group showed the VIQ = PIQ profile, whereas VIQ > PIQ was observed in the non-deletion group. The subtest scores in the two groups showed significant differences, with the deletion group scoring better in three subtests: object assembly, picture arrangement and digit symbol coding. Some relative strengths and weaknesses concerned the total cohort, but others concerned only one genotype. Discussion We documented a global impairment in the intellectual abilities of a large sample of French PWS patients. The scores were slightly lower than those reported in most other studies. Our data confirmed the previously published differences in the cognitive profiles of the two main PWS genotypes and offer new evidence to support this hypothesis. These results could guide future neuropsychological studies to determine the cognitive processing in PWS. This knowledge is essential to improve our understanding of gene-brain-behaviour relationships and to open new perspectives on therapeutic and educational programmes. [source] Role of long-term lamivudine treatment of hepatitis B virus recurrence after liver transplantationJOURNAL OF MEDICAL VIROLOGY, Issue 11 2008Hyun Young Woo Abstract In this study, the long-term (>3 years) efficacy of combination therapy for hepatitis B virus (HBV) recurrence and the associated factors were investigated. One hundred and sixty-five consecutive HBsAg-positive patients (92 with liver cirrhosis, 73 with hepatocellular carcinoma; HCC) who underwent liver transplantation were assessed with a median follow-up time of 40 months. One hundred and twenty-one patients (121/165, 73.3%) were treated with lamivudine before transplantation for a mean of 8.4 months (range 0.1,72 months). The post-transplantation treatment protocol consisted of a high dose intravenous hepatitis B immunoglobulin (HBIg) followed by a low dose intramuscular HBIg and lamivudine combination therapy. Seven (4.2%, 7/165) recipients experienced HBV recurrence at a median time of 19 months (range 5,36 months) following transplantation. Six of seven cases of HBV recurrence were treated with lamivudine before transplantation for a median period of 15 months (range 0.6,30 months). Eighteen (24.6%, 18/73) patients had HCC recurrences after transplantation. Of the four patients with both HCC and HBV recurrence, three experienced HBV recurrence after recurrence of HCC. The clinical factor associated with HBV recurrence in the total cohort (n,=,165) was the duration of antiviral treatment (over 6 months) before transplantation (P,=,0.004). In the HCC group, HCC recurrence after transplantation (P,=,0.002), tumor burden before transplantation (P,=,0.005), and postoperative adjuvant chemotherapy (P,=,0.002), were additional factors for HBV recurrence. Combination therapy of HBIg and antiviral drugs was effective over 3 years regardless of the pretransplantation viral load. However, the possible recurrence of HBV needs to be monitored cautiously in patients treated with long-term (over 6 months) lamivudine. J. Med. Virol. 80:1891,1899, 2008. © 2008 Wiley-Liss, Inc. [source] The Occurrence of Dental Pain and Extractions over a 3-Year Period in a Cohort of Children Aged 3-6 YearsJOURNAL OF PUBLIC HEALTH DENTISTRY, Issue 2 2008M. Tickle PhD Abstract Objectives: To describe the occurrence of dental pain and extractions in young children in relation to the caries and restoration history of their primary molar teeth. Methods: A prospective cohort study of 739 children aged 2.8 to 6.2 years attending 50 dental practices in the North West of England followed for 3 years. Incidence rates for pain and extraction in primary molar teeth were calculated for children with and without dental caries. Tooth years at risk of extraction or pain were calculated for each primary molar according to whether they were caries-free, carious and unrestored, or restored. Results: A total of 119 (16.1 percent) children had caries at recruitment and 157 developed caries during follow-up. Each year approximately one in five children with caries, but only one in 100, who was caries-free, presented with dental pain. In the whole population, each year, approximately one in 40 children had a primary molar tooth extracted but in children with caries it was one in 10. In the total cohort, incidence of pain was higher in unrestored carious teeth than restored, but incidence of extraction was higher in restored than in unrestored teeth. Conclusion: The majority of children attending general dental practice remained caries-free and did not experience pain or extraction over 3 years. Children with caries had a substantial risk of developing pain or having an extraction. The study was unable to demonstrate that restoring carious primary molar teeth prevents pain and extraction. [source] Smoke exposure interacts with ADAM33 polymorphisms in the development of lung function and hyperresponsivenessALLERGY, Issue 6 2009N. E. Reijmerink Introduction:,ADAM33 is the first identified asthma gene by positional cloning, especially asthma combined with bronchial hyperresponsiveness (BHR). Moreover, ADAM33 is associated with early-life lung function and decline of forced expiratory volume in 1 s (FEV1) in the general population. In utero and postnatal cigarette smoke exposure (CSE) are associated with reduced lung function, and development of BHR and asthma. We hypothesized that this may occur via interaction with ADAM33. Aim:, To replicate the role of ADAM33 in childhood lung function and development of BHR and asthma. Furthermore, we investigated gene,environment interaction of ADAM33 with in utero and postnatal CSE in the Dutch PIAMA cohort. Methods:, Six ADAM33 single-nucleotide polymorphisms (SNPs) were genotyped. Rint was measured at age 4 and 8 years, FEV1 and BHR at age 8 years; asthma was based on questionnaire data at age 8. Results:, In the total cohort, the rs511898 A, rs528557 C, and rs2280090 A alleles increased the risk to develop asthma (+BHR). There existed interaction between in utero but not postnatal CSE and the rs528557 and rs3918396 SNPs with respect to development of BHR, the rs3918396 SNP with Rint at age 8 and the rs528557 SNP with FEV1% predicted. Conclusions:, We confirm associations between ADAM33 and the development of asthma (+BHR). This is the first study suggesting that interaction of in utero CSE with ADAM33 results in reduced lung function and the development of BHR, which needs further confirmation. [source] Assessment of glycemic control by continuous glucose monitoring system in 50 children with type 1 diabetes starting on insulin pump therapyPEDIATRIC DIABETES, Issue 3 2004Dorothee Deiss Abstract:, Objective:, To report experience with a continuous glucose monitoring system (CGMS) and to identify factors influencing glycemic control in a large cohort of children and adolescents with type 1 diabetes and change to insulin pump therapy via continuous subcutaneous insulin infusion (CSII). Research design and methods:, In 50 patients [21 boys, 29 girls; median age 12.6 yr (range: 1.3,16.4 yr); diabetes duration 5.0 yr (0.2,13.3)], hemoglobin A1c (HbA1c) and ambulatory CGMS were performed before and 6 wk after starting CSII. Average glucose concentration per 24 h, during day and night time as well as number of excursions, duration, and area under the curve (AUC) of glucose values above 180 mg/dL and below 60 mg/dL were calculated from CGMS data. Simultaneously, metabolic control was documented by standardized self-monitoring of blood glucose (SMBG). Results:, In the total cohort, HbA1c improved from 8.1 ± 1.2% at baseline to 7.7 ± 0.9% after 6 wk of CSII (p < 0.001). This effect was more distinct in boys (8.0 ± 1.4 vs. 7.5 ± 1.1%, p = 0.007) than in girls (8.1 ± 1.1 vs. 7.8 ± 0.7%, p = 0.039) as well as in patients with poor glycemic control (HbA1c > 8.0%) at baseline (8.9 ± 0.6 vs. 8.1 ± 0.8%, p < 0.001) and in those older than 12 yr (8.2 ± 1.2 vs. 7.7 ± 1.0%, p < 0.001). At 6 wk of CSII, the values of glucose average per 24 h, AUC and time above 180 mg/dL, particularly during the day, improved. HbA1c was correlated with AUC above 180 mg/dL (r = 0.742, p < 0.001) and CGMS average glucose per 24 h (r = 0.628, p = 0.002), but to a lesser extent with SMBG values (r = 0.418, p = 0.054). Conclusion:, With the change to CSII, HbA1c improved significantly after 6 wk of therapy. CGMS usage provided additional information about glycemic control in these patients. [source] Life with continuous subcutaneous insulin infusion (CSII) therapy: child and parental perspectives and predictors of metabolic controlPEDIATRIC DIABETES, Issue 2 2001Aristides k Maniatis Abstract: Objective:, The purpose of this study was twofold (i): to evaluate metabolic control in patients receiving CSII therapy in a routine pediatric diabetes clinic by describing reasons for initiating therapy and daily management issues, including needle fear; and (ii) to assess the change in parental involvement and anxiety once their child initiated CSII therapy. Research design and methods: The study included 52 subjects (aged 7.6,23.6 yr) from a general pediatric diabetes clinic. Management issues were defined as diet, exercise, home blood glucose monitoring (HBGM) frequency, and self/staff assessment of needle fear. Characteristics were analyzed both according to a 0.5% change in HbA1c status (decreased vs. stable vs. increased) compared with pre-CSII therapy, and final HbA1c achieved (, 8.1 vs. > 8.1%). Results: The primary recommendation source for CSII use was most often the physician/diabetes team (48.1%), followed by a combination of the former with a personal referral source (32.7%). The most common reason (71.2%) for CSII initiation was a combination of wanting to achieve better metabolic control, dislike of insulin injections, and/or increased flexibility in daily living. Over one-quarter (26.9%) of subjects were identified as being needle-fearful, and this characteristic was predictive of final metabolic control (3/25 subjects ,,8.1% vs. 11/27 subjects >,8.1%, p =,0.03). On CSII therapy, dietary carbohydrate consistency was highly variable, and most subjects (65.3%) exclusively used an insulin to carbohydrate ratio for insulin bolus dosage calculation. The most common adjustment strategy (63.5%) for exercise was a combination of decreasing the insulin basal rate, disconnecting the pump, and/or eating extra carbohydrates. For the total cohort, the frequency of HBGM significantly increased on CSII therapy (4.31,4.85 tests/day, p =,0.02). Females did not have a significant change in HBGM frequency, while the youngest subjects had the highest HBGM frequency. Parental involvement and anxiety primarily stayed the same or decreased, regardless of the child's age (, 18 vs. > 18 yr) or metabolic control. Conclusions:, Analyses of the various characteristics identified only needle fearfulness as being predictive of poor metabolic control. Interestingly, poor control with CSII therapy did not result in a significant increase in parental involvement and/or anxiety. [source] Mortality in a Swedish rubber tire manufacturing plant: Occupational risks or an "unhealthy worker" effect?AMERICAN JOURNAL OF INDUSTRIAL MEDICINE, Issue 8 2006Gun Wingren PhD Abstract Background In this cohort study a classification of 12 work categories, with specified common exposures, is used to evaluate the mortality among workers in a Swedish rubber tire plant. Methods Mortality among the cohort members is compared with expected values from national rates. Standardized mortality ratios (SMR) were calculated for the total cohort, for sub-cohorts and with the inclusion of a latency requirement. Results Male workers had increased risks for total mortality, circulatory death, respiratory death, mental disorders, epilepsy, injuries/poisoning, and for alcohol-related death. Many risks were noted among workers exposed to dust, fumes, or vapor. No trend in risk with increasing duration of employment was seen. Discussion Some established risks for rubber workers could be related to some specified work categories. The highest risks were noted among workers having a very short duration of employment indicating a possible "unhealthy worker effect" associated with life-style factors or to tasks offered to unskilled workers. Am. J. Ind. Med. 2006. © 2006 Wiley-Liss, Inc. [source] Cancer mortality among European asphalt workers: An international epidemiological study.AMERICAN JOURNAL OF INDUSTRIAL MEDICINE, Issue 1 2003Abstract Background Inhalation of bitumen fumes is potentially carcinogenic to humans. Methods We conducted a study of 29,820 male workers exposed to bitumen in road paving, asphalt mixing and roofing, 32,245 ground and building construction workers unexposed to bitumen, and 17,757 workers not classifiable as bitumen workers, from Denmark, Finland, France, Germany, Israel, the Netherlands, Norway, and Sweden, with mortality follow-up during 1953,2000. We calculated standardized mortality ratios (SMRs) and 95% confidence intervals (CIs) based on national mortality rates. Poisson regression analyses compared mortality of bitumen workers to that of building or ground construction workers. Results The overall mortality was below expectation in the total cohort (SMR 0.92, 95% CI 0.90,0.94) and in each group of workers. The SMR of lung cancer was higher among bitumen workers (1.17, 95% CI 1.04,1.30) than among workers in ground and building construction (SMR 1.01, 95% CI 0.89,1.15). In the internal comparison, the relative risk (RR) of lung cancer mortality among bitumen workers was 1.09 (95% CI 0.89,1.34). The results of cancer of the head and neck were similar to those of lung cancer, based on a smaller number of deaths. There was no suggestion of an association between employment in bitumen jobs and other cancers. Conclusions European workers employed in road paving, asphalt mixing and other jobs entailing exposure to bitumen fume might have experienced a small increase in lung cancer mortality risk, compared to workers in ground and building construction. However, exposure assessment was limited and confounding from exposure to carcinogens in other industries, tobacco smoking, and other lifestyle factors cannot be ruled out. Am. J. Ind. Med. 43:18,27, 2003. © 2003 Wiley-Liss, Inc. [source] Cardiac Allograft Remodeling After Heart Transplantation Is Associated with Increased Graft Vasculopathy and MortalityAMERICAN JOURNAL OF TRANSPLANTATION, Issue 1 2009E. Raichlin The aim of this study was to assess the patterns, predictors and outcomes of left ventricular remodeling after heart transplantation (HTX). Routine echocardiographic studies were performed and analyzed at 1 week, 1 year and 3,5 years after HTX in 134 recipients. At each study point the total cohort was divided into three subgroups based on determination of left ventricle mass and relative wall thickness: (1) NG,normal geometry (2) CR,concentric remodeling and (3) CH,concentric hypertrophy. Abnormal left ventricular geometry was found as early as 1 week after HTX in 85% of patients. Explosive mode of donor brain death was the most significant determinant of CH (OR 2.9, p = 0.01) at 1 week. CH at 1 week (OR 2.72, p = 0.01), increased body mass index (OR 1.1, p = 0.01) and cytomegalovirus viremia (OR , 4.06, p = 0.02) were predictors of CH at 1 year. CH of the cardiac allograft at 1 year was associated with increased mortality as compared to NG (RR 1.87, p = 0.03). CR (RR 1.73, p = 0.027) and CH (RR 2.04, p = 0.008) of the cardiac allograft at 1 year is associated with increased subsequent graft arteriosclerosis as compared to NG. [source] Prevalence, risk factors, and outcome of uveitis in juvenile idiopathic arthritis: A long-term followup studyARTHRITIS & RHEUMATISM, Issue 2 2007R. K. Saurenmann Objective To assess the prevalence, risk factors, and long-term outcome of uveitis in patients with juvenile idiopathic arthritis (JIA). Methods An inception cohort of all 1,081 patients diagnosed as having JIA at a single tertiary care center was established. A questionnaire and followup telephone calls were used to confirm the diagnosis of uveitis. Ophthalmologists' records of patients with uveitis were collected. Kaplan-Meier and Cox regression analyses were used to assess risk factors for developing uveitis and for complications of uveitis. Results After a mean followup time of 6.9 years, 142 of 1,081 patients (13.1%) had developed uveitis. Risk factors were young age at diagnosis, female sex, antinuclear antibody positivity, and the subtype of JIA. The relative contribution of these risk factors was different for the different subtypes of JIA. Until the end of the study, uveitis complications had developed in 53 of 142 patients with uveitis (37.3%; 4.9% of the total cohort). Only 16 of 175 involved eyes (9.1%) in 14 of 108 patients (13%; 1.3% of the total cohort) for whom ophthalmology reports were available had best corrected visual acuity less than 20/40 (mean followup time for uveitis of 6.3 years). Abnormal vision was associated with synechiae or cataract. Conclusion Risk factors for developing uveitis were different among subtypes of JIA. The long-term outcome of JIA-associated uveitis in our cohort was excellent despite the high rate of complications. [source] Early onset, severe fetal growth restriction with absent or reversed end-diastolic flow velocity waveform in the umbilical artery: Perinatal and long-term outcomesAUSTRALIAN AND NEW ZEALAND JOURNAL OF OBSTETRICS AND GYNAECOLOGY, Issue 1 2009Scott G. PETERSEN Objective: To assess perinatal and long-term outcomes for pregnancies complicated by early onset, severe fetal growth restriction with absent or reverse end-diastolic flow velocity waveform (AREDF) in the umbilical artery. Methods: A retrospective cohort study of 36 singleton pregnancies with AREDF when the estimated fetal weight (EFW) is less than 501 g at presentation. Results: At presentation, the median gestational age and EFW were 24 (18,29) weeks and 364 (167,496) g, respectively. The median interval between presentation and live birth or diagnosis of intrauterine fetal death (IUFD) was 13 (0,60) days. Delivery was for IUFD in 19 cases (53%), fetal indications in 13 cases (36%) and maternal indications in four cases (11%). Caesarean section (CS) was performed for the 17 live births of which 10 (59%) were by classical CS. Of the total cohort, five infants survived to hospital discharge giving an overall perinatal survival rate of 14%. All survivors had short-term morbidity. The cognitive function in four children was assessed as normal at two years of age. One survivor had developmental delay. None of the surviving children had any evidence of cerebral palsy. Conclusion: The overall perinatal survival rate for pregnancies complicated by early onset, severe growth restriction with an EFW of < 501 g and AREDF is low. When delivery occurs for fetal indications, the majority of these women require classical CS. Short-term neonatal morbidity is high though none of the survivors had cerebral palsy. [source] Growth hormone (GH) replacement in hypopituitary adults with GH deficiency evaluated by a utility-weighted quality of life index: a precursor to cost,utility analysisCLINICAL ENDOCRINOLOGY, Issue 1 2008Maria Ko, towska-Häggström Summary Objectives To examine quality of life (QoL) measured by a utility-weighted index in GH-deficient adults on GH replacement and analyse the impact of demographic and clinical characteristics on changes in utilities during treatment. Design Utilities for items in the QoL-Assessment of Growth Hormone Deficiency in Adults (QoL-AGHDAutility) were estimated based on data obtained from the general population in England and Wales (E&W). These estimates were used to calculate QoL changes in GH-treated patients and compare these with normative population values. Patients A total of 894 KIMS patients (53% women) from E&W were followed for 1 to 6 years. Measurements QoL-AGHDAutility at baseline and at the last reported visit, total QoL-AGHDAutility gain and QoL-AGHDAutility gain per year of follow-up. Results QoL-AGHDAutility in patients before GH treatment differed from the expected population values [0·67 (SD 0·174) vs. 0·85 (SD 0·038), P < 0·0001], constituting a mean deficit of ,0·19 (SD 0·168). There was a difference in the mean QoL-AGHDAutility deficit for men [,0·16 (SD 0·170)] and women [,0·21 (SD 0·162)] (P < 0·001). The main improvement occurred during the first year of treatment [reduction of a deficit to ,0·07 (SD 0·163) (P < 0·001) in the total cohort]; however, patients' utilities remained lower than those recorded for the general population during subsequent follow-up (P < 0·001). Despite an observed impact of age, primary aetiology, disease onset and comorbidities on QoL-AGHDAutility, all patients showed a similar beneficial response to treatment. Conclusions QoL-AGHDAutility efficiently monitors treatment effects in patients with GHD. The study confirmed the QoL-AGHDAutility deficit before treatment and a similar QoL-AGHDAutility gain observed after commencement of GH replacement in all patients. [source] | |||||||||||||