Abstract 19687: Morphological and Clinical Characteristics of Late and Young Age Onset Apical Hypertrophic Cardiomyopathy: Insight from a 13-Year Registry of Hypertrophic Cardomyopathy

Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Hyemoon Chung ◽  
Geu-Ru Hong ◽  
Jungwoo Son ◽  
In-Jeong Cho ◽  
Chi Young Shim ◽  
...  

Background: Apical hypertrophic cardiomyopathy (ApHCM) might have different morphological and clinical features according to the age of disease onset, probably result from the distinct mechanism of pathogenesis. The aim of this study was to evaluated the morphological and clinical differences according to the age of disease onset in patients with ApHCM. Methods: This was a retrospective, observational study of 56 ApHCM patients (31 males, mean 65±9 years). Among 426 patients with diagnosed ApHCM from 2000 until 2013, we selected 56 patients who met the inclusion criteria: [[Unable to Display Character: ⑴]] patiens who diagnosed with ApHCM before 60 years old and current age >60 years old, (group A, early onset, n=16); and [[Unable to Display Character: ⑵]] who diagnosed with ApHCM after 60 years old who had no evidence of apical hypertrophy confirmed by previous echocardiography (group B, late onset, n=40). Morphological LV feature was assessed by transthoracic echocardiography, which were divided into pure and mixed type. Furthermore, hemodynamic parameters and clinical events were also evaluated. Results: There were no differences in the presence of hypertension, LV systolic function and left atrial volume index between group A and B. However, early onset group A had more increased maximal wall thickness of LV apex (17.08±2.02 vs 15.43±1.68mm, p=0.005), and more incidence of pure type (100% (16/16) vs 56.8% (21/40), p=0.001) compare than late onset group B. Group B had older age (65±5 vs 72±4 years, p=0.001), increased LV end-diastolic dimension (51.25±2.32 vs 48.58±3.37mm, p=0.006), increased E/E’ (11.24±2.94 vs 14.60±6.37, p=0.049) with decreased E’ (5.61±1.34 vs 4.66±1.38cm/s, p=0.024) respectively.There were no significant differences in the risk of cardiovascular complication such as atrial fibrillation and stoke between group A and B. Conclusions: There was obvious different clinical characteristics according to the age of disease onset. Early onset ApHCM has typical morphological feature and relatively preserved LV diastolic function, compared with late onset ApHCM. Our result supports that late onset ApHCM was related to decreased LV diastolic function and increased LV wall thickness, which reflects the potential myocardial remodeling with aging.

2019 ◽  
Vol 173 (3) ◽  
pp. 224 ◽  
Author(s):  
Srinivas Acharya Nanduri ◽  
Susan Petit ◽  
Chad Smelser ◽  
Mirasol Apostol ◽  
Nisha B. Alden ◽  
...  

BMJ Open ◽  
2019 ◽  
Vol 9 (8) ◽  
pp. e025528
Author(s):  
Yi Zhang ◽  
Yong Yang ◽  
Leixi Xue ◽  
Jian Wen ◽  
Lin Bo ◽  
...  

ObjectiveTo investigate the clinical characteristics of patients with early-onset hyperuricaemia (HUC).MethodsA retrospective study using data from the Second Affiliated Hospital of Soochow University was conducted. 623 patients with HUC were divided into early-onset group and late-onset group. Another 201 healthy subjects ≤40 years old were regarded as control group. The data of physical measurements and biochemistry test were collected. Clinical data of early-onset group were compared with late-onset group and control group by analysis of variance (ANOVA) and χ2test. Principal component analysis (PCA) was applied. Logistic regression was used to identify the clinical factors correlated with patients with early-onset HUC.ResultsThe patients of early-onset group had different body mass index (BMI), serum albumin, alanine transaminase (ALT), aspartate aminotransferase (AST), alkaline phosphatase (AKP), gamma-glutamyltransferase (GGT), creatinine (Cr), triglyceride (TG), total cholesterol (TC), low density lipoprotein (LDL), TG/high density lipoprotein (HDL) ratio, HDL and percentage of males, hypertension (HBP) as well as fatty liver compared with healthy people in the control group. Early-onset group patients had different albumin, ALT, fasting blood glucose, Cr, percentage of males and HBP compared with late-onset group patients. PCA identified four significant patterns including PC1 (labelled ‘TG and HDL’), PC2 (labelled ‘fatty liver and liver enzymes’), PC3 (labelled ‘TC and LDL’) and PC4 (labelled ‘AKP’). The results of univariate and multivariate logistic regression analysis showed that BMI, HBP and albumin were correlative factors for early onset of HUC when the patients with early-onset and late-onset HUC were involved, while gender, BMI, PC1, PC2 and PC4 were correlative factors for early-onset HUC when the early-onset and control groups were involved.ConclusionThis study described a group of patients with early-onset HUC with distinct clinical features. Gender, BMI, ‘TG and HDL’, ‘fatty liver and liver enzymes’ and ‘AKP’ have higher values than HBP, type 2 diabetes mellitus and ‘TC and LDL’ in patients under 40 years old with early-onset HUC.


2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Kai-Qian Kam ◽  
Koh Cheng Thoon ◽  
Wen Sim Nancy Tee ◽  
Michelle Lay Teng Ang ◽  
Natalie Woon Hui Tan ◽  
...  

Abstract Background The current group B streptococcal (GBS) preventive measures had reduced invasive GBS early onset disease (EOD) incidences worldwide, but the late onset disease (LOD) incidences had remained unchanged. Administration of a safe and effective GBS vaccine in addition to the current strategies were thought to be the next steps in reducing the incidences of invasive GBS infection especially LOD. In this study, we aimed to examine the causative GBS serotypes in invasive GBS disease, determine the incidences of EOD and LOD, and compare the risk factors between EOD and LOD. Methods A retrospective study of infants ≤ 90-day-old over an 8-year period (2010–2017). The incidences of EOD and LOD were obtained by using patients with EOD and LOD who were born in our institution as the numerator and the live births in our institution per year of the study period as the denominator. Available GBS isolates were serotyped by the National Public Health Laboratory using capsular serotyping methods. The risk factors of EOD and LOD were compared. Results A total of 71 infants were identified; 16 (22.5%) and 55 (77.5%) of them had EOD and LOD, respectively. Serotype III (n = 42, 71.2%) was the most common serotype amongst the 59 isolates available for serotyping. Serotypes Ia, Ib, II, III, and V accounted for 98.3% (n = 58) of the invasive GBS diseases. The overall incidence was 0.42 per 1000 live births. The mean incidences of EOD and LOD were 0.13 per 1000 live births and 0.29 per 1000 live births, respectively. On multivariate analysis, risk factors for LOD as compared to EOD were: Chinese ethnicity (OR 27.1, 95% CI 3.0–243.1, p = 0.003) and negative/unknown maternal GBS status (OR 20.0, 95% CI 2.0–250.0, p = 0.012). Prematurity and intrapartum risk factors (peripartum maternal pyrexia, prolonged rupture of membrane) of EOD were not associated with LOD. Conclusions The LOD incidence had remained higher than EOD incidence in our cohort. A GBS vaccine that covers the major causative serotypes found in our cohort can potentially reduce the overall GBS disease burden in the country.


2017 ◽  
Vol 76 (2) ◽  
pp. 74-78
Author(s):  
Takeaki Tamura ◽  
Wakako Ishii ◽  
Sonoko Kubota ◽  
Kaori Kimura ◽  
Emiko Momoki ◽  
...  

2016 ◽  
Vol 15 (3) ◽  
pp. 127-134
Author(s):  
Romana Homorodean ◽  
◽  
Lacramioara Perju-Dumbrava ◽  
◽  

Background. The early onset forms of Multiple Sclerosis (Paediatric MS, P-MS) have a low frequency, occurring in 2% to 10% of all MS cases. Age at diagnosis is a key feature for disease progression and prognosis in MS patients. Objective. To evaluate the clinical features and course of MS in patients having the disease onset before the age of 18, according to age at diagnosis. Methods. Patients with MS onset before age of 18 identified from Basse-Normandie MS regional registry, were included in the study. Patients were divided in two groups according to the age at MS diagnosis: the first group (group A) of patients in whom MS diagnosis was set before age of 18 and the second group (group B) including patients older than 18 years at MS diagnosis. Data related to clinical features and disease course were evaluated and compared between the two groups and also with some clinical data of adult MS patients included in the same regional database. Results. A total of 41 patients with P-MS were included in the study, represented by 20 patients in group A and 21 patients in group B. Patients from group A had a more rapid progression to mild handicap (EDSS 3.0) than patients from group B (time to reach EDSS 3.0 of 6.34 vs 14.36 years; p=0.02) and they reached this level of disability at a younger age (21.17 vs 29.79 years; p=0.002). The duration of the first remission was significantly shorter in group A (1.44 vs 5.14 years; p<0.001) and the evolution to secondary-progressive (SP) form was faster when compared to group B (14.97 vs 27.57 years; p=0.01). Patients from group A had a greater risk to reach EDSS 3.0 or EDSS 6.0 as well as a greater probability to reach SP form (p<0.001) than patients from group B. Progression index (PI) of the entire paediatric group was significantly lower than mean PI in the adult group (0.29 vs 0.45; p=0.003) but not significantly different between the two groups (0.37 in group A vs 0.21 in group B). Conclusion. P-MS forms show some clinical peculiarities, especially those with the age of diagnosis less than 18 years. They seem to have a more severe clinical MS course and a faster progression of disability.


2021 ◽  
Author(s):  
Akihiko Shimizu ◽  
Mariko Shimizu ◽  
Shigeru Nomura ◽  
Yoshiyuki Yamada

Author(s):  
R. Matorras ◽  
A. García-Perea ◽  
F. Omeñaca ◽  
M. Diez-Enciso ◽  
R. Madero ◽  
...  

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