Familial Risk of Type I diabetes in European Children

Summary The characteristics of familial Type I (insulin-dependent) diabetes mellitus – that is Type I diabetes in a first degree relative were investigated for children diagnosed before the age of 15 years using data from an international network of population-based registries (the Eurodiab Ace network) and from a case-control study (Eurodiab Ace Substudy 2) conducted by eight of the network's centres. Ecological analysis across the 18 centres showed a positive association between the population incidence rate of Type I diabetes and the prevalence of Type I diabetes in fathers of affected children (Spearman's rank correlation coefficient r _s = 0.70, p < 0.001). A similar association was observed with the prevalence in sibling (r _s = 0.71, p < 0.001), but the association with prevalence in mothers was weaker and not significant. Pooling results from all centres showed that a greater proportion of fathers (3.4 %) of affected children had Type I diabetes than mothers (1.8 %) giving a risk ratio of 1.8 (95 % CI 1.4 to 2.5). Affected girls were more likely to have a father with Type I diabetes than affected boys (odds ratio 1.56, 95 % CI 1.07 to 2.27), but there was no evidence of a similar finding for mothers or siblings. Children with disease onset in the 0–4 year age-range were more likely to have an affected father than were children who were older at onset, and similar although weaker associations were seen in mothers and siblings. This suggests that familial Type I diabetes patients have a younger age at onset than non-familial patients. In conclusion, a positive association between the prevalence of familial Type I diabetes and the population Type I diabetes incidence rate was shown and the characteristics of familial Type I diabetes (younger age at onset and preferential transmission of disease from tather to child and particularly from father to daughter) were described. [Diabetologia (1998) 41: 1151–1156] Received: 16 February 1998 and in revised form: 4 May 1998     

Abdominal Wall Recurrences After Colorectal Resection for Cancer

PURPOSE: The purpose of the present study was to evaluate prospectively the abdominal wall recurrence rate after laparoscopic resection for colorectal cancer, to analyze the impact of the learning curve on abdominal wall recurrence, and to assess the outcome of those patients. METHODS: The Italian Registry of Laparoscopic Colorectal Surgery database was analyzed to obtain data on cancer patients with abdominal wall recurrence, concomitant local or distant metastases, and interval between initial surgery and diagnosis of trocar site or minilaparotomy recurrences. The records of the initial procedures and the technique of specimen removal were reviewed. RESULTS: From January 1992 to July 2000, 2,583 patients (1,753 cases of carcinomas and 830 cases of benign diseases) were recorded. The malignant lesions were located on the right colon in 19 percent, the left colon in 48.8 percent, and rectum in 32.2 percent. Sixteen patients with histologic evidence of colorectal adenocarcinoma recurrences at the abdominal wall were observed (0.9 percent). Ten patients presented an advanced stage (III for 7 patients and IV for 3 patients). Eleven cases occurred during the learning curve period (the first 50 consecutive cases). The median survival time after abdominal wall recurrence diagnosis was 16 (range, 12–60) months. By July 2000 only two patients were alive. CONCLUSIONS: The results of the Italian prospective Registry of Laparoscopic Colorectal Surgery confirm that the incidence of abdominal wall recurrences is similar to that reported in open studies (<1 percent). Most abdominal wall recurrences occurred in the learning curve period, suggesting that surgical experience may play a role in the development of this outcome. The prognosis of these patients is very poor.     

A new CERAD total score with equally weighted z-scores and additional executive and non-amnestic „CERAD-Plus“ tests enhances cognitive diagnosis in patients with Parkinson's disease: Evidence from the LANDSCAPE study

IntroductionThe Consortium to Establish a Registry for Alzheimer's Disease (CERAD) is a renowned cognitive test battery, which has been extended in its German version to the CERAD-Plus including tests of executive functions and processing speed. The most commonly used total score (TS) is based on the restricted CERAD version and reflects the sum of selected raw-values (Chandler et al., 2005). The CERAD-Plus extensions might be of particular diagnostic utility for cognitive assessments in Parkinson's Disease (PD), as executive functions and processing speed belong to the most vulnerable domains in PD.ObjectiveThe aim was to develop a CERAD-TS based on the extended CERAD-Plus' age-, gender-, and education-corrected z-scores and to evaluate its diagnostic accuracy compared to the established CERAD-Chandler-TS.MethodsBaseline data of n = 679 patients with PD (69% male, n = 277 PD without cognitive impairment, n = 307 PD-MCI, n = 95 PD-D) from the multicenter, prospective DEMPARK/LANDSCAPE study were analyzed. ROC-analyses were conducted for four different TS that were either based on the original CERAD or CERAD-Plus, on raw-values or z-scores, and equally-weighted or based on factor scores. AUC-comparisons were conducted to determine the best yet most parsimonious TS.ResultsThe newly designed CERAD-Plus-TS based on equally-weighted z-scores outperformed both the CERAD-Chandler-TS and cognitive screening instruments when differentiating between individuals with PD of varying cognitive impairment (0.78 ≤ AUC ≤ 0.98).ConclusionResults suggest a high relevance of non-amnestic subscales for the cognitive assessment in PD populations. The proposed CERAD-Plus-TS needs further validation. The extensions might offer diagnostic potential for non-PD populations as well.     

Integrating Patient Reported Outcomes With Clinical Cancer Registry Data: A Feasibility Study of the Electronic Patient-Reported Outcomes From Cancer Survivors (ePOCS) System

Background

Routine measurement of Patient Reported Outcomes (PROs) linked with clinical data across the patient pathway is increasingly important for informing future care planning. The innovative electronic Patient-reported Outcomes from Cancer Survivors (ePOCS) system was developed to integrate PROs, collected online at specified post-diagnostic time-points, with clinical and treatment data in cancer registries.

Objective

This study tested the technical and clinical feasibility of ePOCS by running the system with a sample of potentially curable breast, colorectal, and prostate cancer patients in their first 15 months post diagnosis.

Methods

Patients completed questionnaires comprising multiple Patient Reported Outcome Measures (PROMs) via ePOCS within 6 months (T1), and at 9 (T2) and 15 (T3) months, post diagnosis. Feasibility outcomes included system informatics performance, patient recruitment, retention, representativeness and questionnaire completion (response rate), patient feedback, and administration burden involved in running the system.

Results

ePOCS ran efficiently with few technical problems. Patient participation was 55.21% (636/1152) overall, although varied by approach mode, and was considerably higher among patients approached face-to-face (61.4%, 490/798) than by telephone (48.8%, 21/43) or letter (41.0%, 125/305). Older and less affluent patients were less likely to join (both P<.001). Most non-consenters (71.1%, 234/329) cited information technology reasons (ie, difficulty using a computer). Questionnaires were fully or partially completed by 85.1% (541/636) of invited participants at T1 (80 questions total), 70.0% (442/631) at T2 (102-108 questions), and 66.3% (414/624) at T3 (148-154 questions), and fully completed at all three time-points by 57.6% (344/597) of participants. Reminders (mainly via email) effectively prompted responses. The PROs were successfully linked with cancer registry data for 100% of patients (N=636). Participant feedback was encouraging and positive, with most patients reporting that they found ePOCS easy to use and that, if asked, they would continue using the system long-term (86.2%, 361/419). ePOCS was not administratively burdensome to run day-to-day, and patient-initiated inquiries averaged just 11 inquiries per month.

Conclusions

The informatics underlying the ePOCS system demonstrated successful proof-of-concept – the system successfully linked PROs with registry data for 100% of the patients. The majority of patients were keen to engage. Participation rates are likely to improve as the Internet becomes more universally adopted. ePOCS can help overcome the challenges of routinely collecting PROs and linking with clinical data, which is integral for treatment and supportive care planning and for targeting service provision.     

Prospective assessment after pediatric cardiac ablation: demographics, medical profiles, and initial outcomes

INTRODUCTION: A multicenter prospective study was designed and implemented to assess the short- and longer-term results and risks associated with radiofrequency (RF) ablation in children. METHODS AND RESULTS: Patients recruited for the study were aged 0 to 16 years with supraventricular tachycardia due to accessory pathways or AV nodal reentrant tachycardia (AVNRT), excluding patients with nontrivial congenital heart disease. A national registry also was established, and contributing centers were encouraged to enroll all pediatric patients, aged 0 to 21 years, undergoing ablation at their center. This report summarizes acute results of these procedures. For analysis, subjects were divided into three groups: the prospective cohort (n = 481), cohort-eligible registry participants (n = 504), and not cohort eligible registry participants (n = 1,776). Prospectively enrolled cohort patients were similar to cohort-eligible patients in terms of demographic and other patient characteristics. Overall success rates for RF ablation were high (95.7%), with higher success rates for left-sided and particularly left free-wall pathways (97.8%) than right free-wall pathways (90.8%). Complications of both electrophysiologic study and RF ablation were infrequent (4.2% and 4.0%, respectively), and there were no deaths. AV block was uncommon overall (1.2%) and was limited to ablation in AVNRT (2.1%) and septal accessory pathways (3.0%). CONCLUSION: Despite the multicenter and prospective design, the study demonstrates high success rates and low complication rates, which are comparable to prior single-center retrospective studies. These results may serve as the current best benchmark for expected results in the pediatric population, aged 0 to 16 years, both in terms of acute success rates and the occurrence of complications.