Clinicopathologic Characterization of Early Onset Endoscopically Unresectable Adenomas (EOEUAs)

2019 ◽  
Vol 152 (Supplement_1) ◽  
pp. S55-S55
Author(s):  
Rayan Saade ◽  
Zhiyan Fu ◽  
Hwajeong Lee
Keyword(s):  
Risk Factors ◽  
Alcohol Consumption ◽  
Early Onset ◽  
Age Group ◽  
Exact Test ◽  
Pathologic Features ◽  
Increased Risk ◽  
Patients With Diabetes ◽  
Pathology Reports

Abstract Objectives Early onset colorectal carcinoma (CRC), defined as CRC detected in patients ≤50 years old, are frequently located in the distal colorectum and are associated with risk factors that are common to adenoma. We examined the risk factors, pathologic features, and clinical implications associated with EOEUA. Methods A retrospective cohort of 154 patients with EOEUA was retrieved. Clinical data were obtained from electronic medical records. A minimum of 12 months follow-up was considered valid follow-up data. Archived pathology slides were evaluated for the presence of high-grade dysplasia (HGD) and Paneth cells (PCs) in adenomatous crypts. Additional pathologic data were retrieved from pathology reports. The patients were stratified into age >50 vs ≤50 years. Fisher’s exact test and Student t test were performed when indicated (P < .05 was considered statistically significant). Results In the ≤50-year group, EOEUAs were common in the rectosigmoid colon compared to >50-year group (41.2% vs 16.8%; P = .025). The association with diabetes, obesity, and alcohol consumption was similar in the two groups, whereas the incidences of hypertension, hyperlipidemia, and smoking were significantly lower in the ≤50-year group. No significant differences in gender distribution, adenoma size, HGD, PC presence, and future adenoma burden were observed between the two groups. Conclusion The association with established risk factors of adenoma to include diabetes, obesity, and alcohol consumption in EOEUA is comparable to that of older patients. In contrast, hypertension, hyperlipidemia, and smoking do not appear to be associated with an increased risk of endoscopically unresectable adenoma in this age group. Similar to early onset CRC, EOEUAs are common in the rectosigmoid, supporting the premise that flexible sigmoidoscopy may be a viable screening alternative in this age group, especially for patients with diabetes, obesity, or alcohol history.

scholarly journals Population Attributable Fraction of Early Age of Onset of Alcohol Use in Alcohol Abuse and Dependence: A 3-Year Follow-Up Study in University Students

2020 ◽  
Vol 17 (6) ◽  
pp. 2159 ◽  
Author(s):  
Francisco Caamano-Isorna ◽  
Amy Adkins ◽  
Fazil Aliev ◽  
Lucía Moure-Rodríguez ◽  
Danielle M. Dick
Keyword(s):  
Risk Factors ◽  
Sexual Orientation ◽  
Alcohol Use ◽  
Alcohol Abuse ◽  
Early Onset ◽  
Age Of Onset ◽  
Full Time ◽  
Increased Risk ◽  
Alcohol Abuse And Dependence

Background: we aimed to determine the risk factors and associated population attributable fractions (PAFs) for the age of onset of alcohol use and also to identify protective factors. Methods: we analyzed follow-up data collected between autumn 2011 and spring 2016 (n = 5170) from the first two cohorts (2011, 2012) of the Spit for ScienceTM project. The dependent variables were alcohol abuse and dependence, and the independent variables were age of drinking onset, residence, ethnicity, religiosity, sexual orientation and work status. We determined the odds ratios (OR) using multilevel logistic regression for repeated measures in SPSSv.20. Results: the early onset of alcohol use was associated with an increased risk of alcohol abuse and dependence among females (OR = 14.98; OR = 11.83) and males (OR = 7.41; OR = 6.24). The PAFs for the early onset of alcohol use in alcohol abuse and dependence were respectively 80.9% and 71.7% in females and 71.0% and 63.5% in males. Among females, being white (OR = 1.58; OR = 1.51), living off-campus (OR = 1.73; OR = 2.76) and working full-time (OR = 1.69; OR = 1.78) were also risk factors. Strong religious beliefs were found to protect males from alcohol abuse (OR = 0.58), while same-gender sexual orientation increased the risk among females (OR = 2.09). Conclusion: delaying the age of onset by one year would reduce alcohol abuse among young adults.

scholarly journals Characteristics and Outcomes of Therapy Related Myeloid Neoplasms in Patients with Multiple Myeloma Following Autologous Stem Cell Transplantation

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 4560-4560 ◽  
Author(s):  
Kalyan Nadiminti ◽  
M Hasib Sidiqi ◽  
Kapil Meleveedu ◽  
Hassan B. Alkhateeb ◽  
Aref Al-Kali ◽  
...  
Keyword(s):  
Risk Factors ◽  
Research Funding ◽  
Survival Advantage ◽  
High Dose ◽  
Term Survival ◽  
Myeloid Neoplasms ◽  
Pathologic Features ◽  
Increased Risk

Introduction: Patients who develop therapy-related myeloid neoplasms (t-MN) have dismal outcomes. Previous studies reported the incidence and risk factors associated with t-MN development. Lenalidomide, in the setting of oral, but not intravenous, melphalan is associated with a higher risk of t-MN (Palumbo et. al, Lancet Oncology, 2014). We carried out this study to evaluate the clinical and pathologic features of t-MN, therapies employed, and factors that predict long-term survival after diagnosis. Patients and methods: We identified patients who received the first ASCT 1998-2016 at our institution. t-MN was defined per the WHO 2016 classification. Median overall survival (OS) was calculated from the time of t-MN diagnosis to last follow-up or death. Statistical analyses were performed using SAS (JMP v14.1) or GraphPad Prism (v7). Results: Out of 2115 patients that underwent at least one ASCT, 53 (2.5%) developed t-MN. Thirty-five of 53 (66%) patients who developed t-MN had received lenalidomide. Among 2062 patients that did not develop t-MN, 916 (44.4%) patients received lenalidomide. Lenalidomide exposure was associated with development of t-MN (χ2 with Yate's correction 8.9, p=0.003). Ten patients were excluded from further analyses due to lack of follow up. Clinical characteristics are shown in Table 1a (N=43). Median age at t-MN diagnosis was 70 years (range 44-79). Median time from ASCT to t-MN was 5 years (range 1-15). After a median follow-up of 70 months (95% CI, 38-134), the median OS was 12 months (95% CI, 9-17, Figure). Primary causes of death were t-MN (71%), MM (12%), both (6%), and other including infection, GVHD, and unknown (12%). Seven (16%) had t-AML and 36(84%) had t-MDS. Three (42%) of 7 patients with t-AML had pure erythroid phenotype. At the time of last follow-up, 9 (21%) were alive. Seven (17%) underwent two ASCT, 16 (36%) received more than 2 years cumulative dose of lenalidomide. Median number of cycles of alkylator therapy including high-dose melphalan (HDM) used for ASCT was 2 (range 1-6). On univariate analysis, factors predicting OS from t-MN diagnosis were ≥ 2 alkylator vs. < 2 cycles (11 vs. 27 months, p=0.02), ≥10% vs. <10% blast at the time of t-MN diagnosis (5.5 vs. 17 months, p=0.01), and the presence of complex karyotype (CK) vs. not (11 vs. 17 months, p=0.03). There was no difference in survival among t-MDS patients who transformed to t-AML vs. those who did not, those who received 1 vs. 2 ASCT, and those with lenalidomide duration ≥ 2 vs. <2 years. On multivariate analysis, the number of alkylator therapies and CK, but not % blasts predicted OS from t-MN diagnosis (Figure). Eight (19%) patients proceeded to receive an autologous (n=4) or allogeneic (n=4) transplant for t-MN (Table 1b). There was no survival advantage for patients who underwent SCT for t-MN, compared to those who did not (17 vs. 10 months, p=0.3). Similarly, there was no survival advantage for patients who received allo SCT compared to auto SCT (21 vs. 16 months, p=0.7). Survival for t-MDS and t-AML was also not different (15 vs. 6 months, p=0.2). There is only one long-term survivor in the transplant group (s/p allogeneic SCT). Four of seven (57%) patients died of progressive t-MN, 1 died of progressive MM, progressive MM and t-MN, and TRM each. Conclusions: Lenalidomide, in the setting of HDM, is associated with an increased risk of t-MN. Increased exposure to alkylators, a higher tumor burden, and the presence of CK predict poor survival in t-MN. As HDM/ASCT followed by lenalidomide maintenance remains the standard of care for eligible patients, minimizing exposure to other alkylator regimens may be prudent. While considered the standard, only a minority undergo transplant; and post-transplant outcomes remain poor. Studies to identify prognostic genetic and epigenetic risk factors are ongoing. Disclosures Al-Kali: Astex Pharmaceuticals, Inc.: Research Funding. Kumar:Celgene: Consultancy, Research Funding; Janssen: Consultancy, Research Funding; Takeda: Research Funding. Patnaik:Stem Line Pharmaceuticals.: Membership on an entity's Board of Directors or advisory committees.

scholarly journals Nasal symptoms increase the risk of snoring and snoring increases the risk of nasal symptoms. A longitudinal population study

2021 ◽  
Author(s):  
Maria Värendh ◽  
Christer Janson ◽  
Caroline Bengtsson ◽  
Johan Hellgren ◽  
Mathias Holm ◽  
...  
Keyword(s):  
Risk Factors ◽  
Respiratory Health ◽  
Smoking Status ◽  
Northern Europe ◽  
Nasal Breathing ◽  
Nasal Symptoms ◽  
Increased Risk ◽  
Study Population ◽  
Independent Risk Factors

Abstract Purpose Humans have a preference for nasal breathing during sleep. This 10-year prospective study aimed to determine if nasal symptoms can predict snoring and also if snoring can predict development of nasal symptoms. The hypothesis proposed is that nasal symptoms affect the risk of snoring 10 years later, whereas snoring does not increase the risk of developing nasal symptoms. Methods In the cohort study, Respiratory Health in Northern Europe (RHINE), a random population from Denmark, Estonia, Iceland, Norway, and Sweden, born between 1945 and 1973, was investigated by postal questionnaires in 1999–2001 (RHINE II, baseline) and in 2010–2012 (RHINE III, follow-up). The study population consisted of the participants who had answered questions on nasal symptoms such as nasal obstruction, discharge, and sneezing, and also snoring both at baseline and at follow-up (n = 10,112). Results Nasal symptoms were frequent, reported by 48% of the entire population at baseline, with snoring reported by 24%. Nasal symptoms at baseline increased the risk of snoring at follow-up (adj. OR 1.38; 95% CI 1.22–1.58) after adjusting for age, sex, BMI change between baseline and follow-up, and smoking status. Snoring at baseline was associated with an increased risk of developing nasal symptoms at follow-up (adj. OR 1.22; 95% CI 1.02–1.47). Conclusion Nasal symptoms are independent risk factors for development of snoring 10 years later, and surprisingly, snoring is a risk factor for the development of nasal symptoms.

scholarly journals Cumulative incidence and risk factors for radiation induced leukoencephalopathy in high grade glioma long term survivors

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Robert Terziev ◽  
Dimitri Psimaras ◽  
Yannick Marie ◽  
Loic Feuvret ◽  
Giulia Berzero ◽  
...  
Keyword(s):  
Risk Factors ◽  
Cumulative Incidence ◽  
High Grade Glioma ◽  
High Grade ◽  
Nucleotide Polymorphisms ◽  
Increased Risk ◽  
Radiation Induced ◽  
Long Term Survivors

AbstractThe incidence and risk factors associated with radiation-induced leukoencephalopathy (RIL) in long-term survivors of high-grade glioma (HGG) are still poorly investigated. We performed a retrospective research in our institutional database for patients with supratentorial HGG treated with focal radiotherapy, having a progression-free overall survival > 30 months and available germline DNA. We reviewed MRI scans for signs of leukoencephalopathy on T2/FLAIR sequences, and medical records for information on cerebrovascular risk factors and neurological symptoms. We investigated a panel of candidate single nucleotide polymorphisms (SNPs) to assess genetic risk. Eighty-one HGG patients (18 grade IV and 63 grade III, 50M/31F) were included in the study. The median age at the time of radiotherapy was 48 years old (range 18–69). The median follow-up after the completion of radiotherapy was 79 months. A total of 44 patients (44/81, 54.3%) developed RIL during follow-up. Twenty-nine of the 44 patients developed consistent symptoms such as subcortical dementia (n = 28), gait disturbances (n = 12), and urinary incontinence (n = 9). The cumulative incidence of RIL was 21% at 12 months, 42% at 36 months, and 48% at 60 months. Age > 60 years, smoking, and the germline SNP rs2120825 (PPARg locus) were associated with an increased risk of RIL. Our study identified potential risk factors for the development of RIL (age, smoking, and the germline SNP rs2120825) and established the rationale for testing PPARg agonists in the prevention and management of late-delayed radiation-induced neurotoxicity.

10 Years' Experience in Balanitis Xerotica Obliterans: A Single-Institution Study

2018 ◽  
Vol 29 (03) ◽  
pp. 302-306 ◽  
Author(s):  
Akhilesh Pradhan ◽  
Radhika Patel ◽  
A. Said ◽  
Manasvi Upadhyaya
Keyword(s):  
Average Duration ◽  
Age Group ◽  
Histological Findings ◽  
Single Institution ◽  
Exact Test ◽  
Balanitis Xerotica Obliterans ◽  
Tertiary Teaching ◽  
Tertiary Teaching Hospital ◽  
High Degree

Introduction Balanitis xerotica obliterans (BXO) is uncommon in children. Diagnosis of the condition is almost always clinical and supported by histology. Our aim was to evaluate the outcomes of children undergoing circumcision for BXO and explore the correlation between surgical and histological findings. Materials and Methods A 10-year retrospective review (2007–2017) of all children, aged 16 and less, undergoing circumcision at a tertiary teaching hospital was conducted. Statistical analysis was performed using Fisher's exact test. Results BXO occurred in 91/1025 (8.9%) children. The highest incidence of BXO was in the 5 to 10 age group (13.3%; p < 0.0001). The commonest symptom was foreskin scarring (62.6%). Intraoperatively, involvement of foreskin alone was seen in 26.4%, foreskin and meatus in 47.2%, and foreskin, meatus, and glans in 26.4%. Preoperatively, 24.2% of patients were prescribed steroid cream. Histologically, all patients showed microscopic changes confirming BXO. A total of 87.9% of patients only had a circumcision, and 11% required a meatal procedure along with the circumcision. Postoperatively, 19.8% of patients required a further procedure after an average duration of 5.8 months (range: 2–12 months); the majority of whom (83.3%) were prescribed postoperative steroid cream. Conclusion Clinical correlation by surgeons has a high degree of accuracy (>90%). Meatal involvement is more common than previously reported. The use of pre- or postoperative steroids does not obviate the need for further surgical procedures. Patients can have recurrent symptoms 1 year following surgery, and prolonged follow-up is necessary.

scholarly journals Stroke in hemodialysis patients randomized to different intravenous iron strategies: a prespecified analysis from the PIVOTAL trial

Kidney360 ◽  
2021 ◽  
pp. 10.34067/KID.0004272021
Author(s):  
Patrick B. Mark ◽  
Pardeep S. Jhund ◽  
Matthew R. Walters ◽  
Mark C. Petrie ◽  
Albert Power ◽  
...  
Keyword(s):  
Risk Factors ◽  
Stroke Risk ◽  
Controlled Trial ◽  
Intravenous Iron ◽  
Hemodialysis Patients ◽  
High Dose ◽  
Pivotal Trial ◽  
Increased Risk ◽  
Iv Iron

Background: People with kidney failure treated with hemodialysis (HD) are at increased risk of stroke compared to similarly aged people with normal kidney function. One concern is that treatment of renal anemia might increase stroke risk. We studied risk factors for stroke in a prespecified secondary analysis of a randomized controlled trial of intravenous iron treatment strategies in HD. Methods: We analyzed data from the Proactive IV IrOn Therapy in HaemodiALysis Patients (PIVOTAL) trial focusing on variables associated with risk of stroke. The trial randomized 2,141 adults, who had started hemodialysis <12 months earlier and who were receiving an erythropoiesis-stimulating agent (ESA), to high-dose IV iron administered proactively or low-dose IV iron administered reactively in a 1:1 ratio. Possible stroke events were independently adjudicated. We performed analyses to identify variables associated with stroke during follow-up and assessed survival following stroke. Results: During a median 2.1 years follow-up, 69 (3.2%) patients experienced a first post randomization stroke. 57 (82.6%) were ischemic strokes and 12 (17.4%) hemorrhagic strokes. There were 34 post randomization strokes in the proactive arm and 35 in the reactive arm (hazard ratio (95% confidence interval): 0.90 (0.56, 1.44), p=0.66). In multivariable models, female gender, diabetes, history of prior stroke at baseline, higher baseline systolic blood pressure, lower serum albumin and higher C-reactive protein were independently associated with stroke events during follow up. Hemoglobin, total iron or ESA dose were not associated with risk of stroke. 58% of patients with a stroke event died during follow-up, compared to 23% without a stroke. Conclusions: In hemodialysis patients, stroke risk is broadly associated with risk factors previously described to increase cardiovascular risk in this population. Proactive intravenous iron does not increase stroke risk.

scholarly journals MDCT evaluation of coronary artery calcification in Nepalese population undergoing CT thorax examination for non-cardiac pathology

2014 ◽  
Vol 6 (4) ◽  
pp. 40-44
Author(s):  
Mukesh Kumar Gupta ◽  
Raj Kumar Rauniyar ◽  
Naveen Kumar Pandey ◽  
Deepak Kumar Yadav
Keyword(s):  
Diabetes Mellitus ◽  
Risk Factors ◽  
Cross Sectional Study ◽  
Age Group ◽  
Medical Sciences ◽  
Cross Sectional ◽  
Cardiac Pathology ◽  
Patients With Diabetes ◽  
Ct Evaluation ◽  
Atherosclerotic Risk Factors

Background:Coronary artery calcification (CAC) is frequently encountered as incidental findings during CT evaluation of thorax; however, little is known about its magnitude and association with atherosclerotic risk factors in Nepalese population. The purpose of this study was to evaluate the frequency of incidental CAC in patients undergoing standard thoracic CT examination for non-cardiac pathology and to correlate it with risk factors for coronary atherosclerosis.Methods: A hospital based prospective cross-sectional study was conducted on 216 patients over 8 month duration. The coronary arteries were evaluated for calcification on 16-slice MDCT and the frequency of CAC was correlated with atherosclerotic risk factors viz. age, sex, diabetes mellitus, hypertension, smoking and obesity.Results: Incidental CAC was seen in 72(33.3%) of total 216 patients. The rates of CAC below 40, 40-60 and above 60 years age group were 0%, 31.6% and 43.9% respectively. The frequency of CAC in male and female were 35% and 31.2% for all ages, 43.6% and 20% for 40-60 years group, and 35.2% and 61.1% for above 60 years age group respectively. The frequency of CAC was higher in patients with diabetes mellitus (47.5%), hypertension (42.3%), smoking (43%) and obesity (38.9%).Conclusion: Incidental CAC was seen in 33.3% of the patients and it had significant association with advancing age, diabetes mellitus, hypertension and smoking. Rate of CAC was significantly higher in male for 40-60 years group but the frequency drastically increased in female for above 60 years group. DOI: http://dx.doi.org/10.3126/ajms.v6i4.11205 Asian Journal of Medical Sciences Vol.6(4) 2015 40-44

scholarly journals Community prevalence of SARS-CoV-2 in England: Results from the ONS Coronavirus Infection Survey Pilot

2020 ◽  
Author(s):  
Koen B Pouwels ◽  
Thomas House ◽  
Julie V Robotham ◽  
Paul Birrell ◽  
Andrew B Gelman ◽  
...  
Keyword(s):  
Risk Factors ◽  
Credible Interval ◽  
Control Measures ◽  
Cross Sectional ◽  
Direct Patient Contact ◽  
Increased Risk ◽  
Potential Risk Factors ◽  
Coronavirus Infection ◽  
Over Time

Objective: To estimate the percentage of individuals infected with severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) over time in the community in England and to quantify risk factors. Design: Repeated cross-sectional surveys of population-representative households with longitudinal follow-up if consent given. Setting: England. Participants: 34,992 Individuals aged 2 years and over from 16,722 private residential households. Data were collected in a pilot phase of the survey between 26 April and 28 June 2020. Main outcome measures: Percentage of individuals in the community testing positive for SARS-CoV-2 RNA using throat and nose swabs. Individuals were asked about any symptoms and potential risk factors. Results: The percentage of people in private-residential households testing positive for SARS-CoV-2 reduced from 0.32% (95% credible interval (CrI) 0.19% to 0.52%) on 26 April to 0.08% (95% CrI 0.05% to 0.12%) on 28 June, although the prevalence stabilised near the end of the pilot. Factors associated with an increased risk of testing positive included having a job with direct patient contact (relative exposure (RE) 4.06, 95% CrI 2.42 to 6.77)), working outside the home (RE 2.49, 95% CrI 1.39 to 4.45), and having had contact with a hospital (RE 2.20, 95% CrI 1.09 to 4.16 for having been to a hospital individually and RE 1.95, 95% CrI 0.81 to 4.09 for a household member having been to a hospital). In 133 visits where individuals tested positive, 82 (61%, 95% CrI 53% to 69%) reported no symptoms, stably over time. Conclusion: The percentage of SARS-CoV-2 positive individuals declined between 26 April and 28 June 2020. Positive tests commonly occurred without symptoms being reported. Working outside your home was an important risk factor, indicating that continued monitoring for SARS-CoV-2 in the community will be essential for early detection of increases in infections following return to work and other relaxations of control measures.

scholarly journals Perioperative changes of response to antiplatelet medication in vascular surgery patients

PLoS ONE ◽  
2020 ◽  
Vol 15 (12) ◽  
pp. e0244330
Author(s):  
Thomas Hummel ◽  
Saskia Hannah Meves ◽  
Andreas Breuer-Kaiser ◽  
Jan-Ole Düsterwald ◽  
Dominic Mühlberger ◽  
...  
Keyword(s):  
Risk Factors ◽  
Platelet Count ◽  
Vascular Surgery ◽  
Reactive Protein ◽  
Antiplatelet Medication ◽  
Impedance Aggregometry ◽  
Increased Risk ◽  
Potential Risk Factors ◽  
Artery Disease

Introduction Reduced antiplatelet activity of aspirin (ALR) or clopidogrel (CLR) is associated with an increased risk of thromboembolic events. The reported prevalence data for low-responders vary widely and there have been few investigations in vascular surgery patients even though they are at high risk for thromb-embolic complications. The aim of this prospective observational monocentric study was to elucidate possible changes in ALR or CLR after common vascular procedures. Methods Activity of aspirin and clopidogrel was measured by impedance aggregometry using a multiple electrode aggregometer (Multiplate®). Possible risk factors for ALR or CLR were identified by demographical, clinical data and laboratory parameters. In addition, a follow-up aggregometry was performed after completion of the vascular procedure to identify changes in antiplatelet response. Results A total of 176 patients taking antiplatelet medications aspirin and/or clopidogrel with peripheral artery disease (PAD) and/or carotid stenosis (CS) were included in the study. The prevalence of ALR was 13.1% and the prevalence of CLR was 32% in the aggregometry before vascular treatment. Potential risk factors identified in the aspirin group were concomitant insulin medication (p = 0.0006) and elevated C-reactive protein (CRP) (p = 0.0021). The overall ALR increased significantly postoperatively to 27.5% (p = 0.0006); however, there was no significant change in CLR that was detected. In a subgroup analysis elevation of the platelet count was associated with a post-procedure increase of ALR incidence. Conclusion The incidence of ALR in vascular surgery patients increases after vascular procedures. An elevated platelet count was detected as a risk factor. Further studies are necessary to analyse this potential influence on patency rates of vascular reconstructions.

Abstract 1158: Sudden Death in Children with Cardiomyopathy: Long Term Follow-Up from a National Population-Based Study of Childhood Cardiomyopathy

Circulation ◽  
2008 ◽  
Vol 118 (suppl_18) ◽  
Author(s):  
Tara Bharucha ◽  
Andrew M Davis ◽  
Christian Turner ◽  
Robert Justo ◽  
Terry Robertson ◽  
...  
Keyword(s):  
Risk Factors ◽  
Primary Prevention ◽  
Sudden Death ◽  
Systolic Dysfunction ◽  
Population Based ◽  
Z Score ◽  
Icd Implantation ◽  
Population Based Study ◽  
Increased Risk

Introduction Better data regarding the incidence and risk factors for sudden cardiac death (SCD) in children with cardiomyopathy (CM) is critical in defining appropriate primary prevention strategies. Methods The National Australian Childhood Cardiomyopathy Study is a prospective cohort study, including all children in Australia with primary CM diagnosed at 0 – 10 years of age, between 1987–1997. SCD was defined as sudden and unexpected death in children who were not hospitalized and not in congestive heart failure at the time of death. Nine subjects with sudden death as presenting symptom were excluded. Indexed echocardiographic measurements at latest follow-up were compared between subjects with SCD and survivors. Results Study criteria were met by 291 children. Mean duration of follow-up was 9.2 years. The incidence of sudden death relative to each CM type, for all cases and as a proportion of deaths, is shown in the Table : Incidence of SCD by CM type. SCD incidence was significantly associated with CM type, for all cases ( p = 0.006) and when only those subjects who died were considered ( p = 0.005), with LVNC and RCM having up to 4 times the risk of other CM types. Children with familial DCM had a significantly higher rate of SCD than subjects with non-familial CM (12% vs 3%; p = 0.028), however, familial CM was not a risk factor in other CM types. DCM SCD subjects had larger LVEDd Z score than survivors (median 5.53 vs 1.16; p <0.0001) and lower FS Z score (median −9.23 vs −0.51; p = 0.0025). HCM SCD subjects had thicker LVPW dimension Z scores than survivors (median 4.63 vs 1.18; p = 0.007). Twelve subjects (2 DCM, 8 HCM and 2 LVNC) underwent ICD implantation (8/12 for primary prevention). Conclusions: This population based study defines new risk factors for sudden death in children with CM. RCM is well known to have a high incidence of SCD. In addition, children with LVNC and those with DCM who have severe dilatation, systolic dysfunction or familial DCM are at increased risk of sudden death.

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