Humans ; Fabry Disease/therapy* ; Hypertrophy, Left Ventricular/diagnosis* ; Diagnosis, Differential

Humans ; Fabry Disease/therapy* ; Hypertrophy, Left Ventricular/diagnosis* ; Diagnosis, Differential

Heart failure with preserved ejection fraction (HFpEF) is a type of heart failure characterized by left ventricular diastolic dysfunction with preserved ejection fraction. With the aging of the population and the increasing prevalence of metabolic diseases, such as hypertension, obesity and diabetes, the prevalence of HFpEF is increasing. Compared with heart failure with reduced ejection fraction (HFrEF), conventional anti-heart failure drugs failed to reduce the mortality in HFpEF due to the complex pathophysiological mechanism and multiple comorbidities of HFpEF. It is known that the main changes of cardiac structure of in HFpEF are cardiac hypertrophy, myocardial fibrosis and left ventricular hypertrophy, and HFpEF is commonly associated with obesity, diabetes, hypertension, renal dysfunction and other diseases, but how these comorbidities cause structural and functional damage to the heart is not completely clear. Recent studies have shown that immune inflammatory response plays a vital role in the progression of HFpEF. This review focuses on the latest research progress in the role of inflammation in the process of HFpEF and the potential application of anti-inflammatory therapy in HFpEF, hoping to provide new research ideas and theoretical basis for the clinical prevention and treatment in HFpEF.
Humans ; Heart Failure ; Stroke Volume/physiology* ; Hypertrophy, Left Ventricular/metabolism* ; Ventricular Dysfunction, Left/metabolism* ; Inflammation/complications* ; Obesity ; Hypertension

Muscle fibres are multinuclear cells, and the cytoplasmic territory where a single myonucleus controls transcriptional activity is called the myonuclear domain (MND). MND size shows flexibility during muscle hypertrophy. The MND ceiling hypothesis states that hypertrophy results in the expansion of MND size to an upper limit or MND ceiling, beyond which additional myonuclei via activation of satellite cells are required to support further growth. However, the debate about the MND ceiling hypothesis is far from settled, and various studies show conflicting results about the existence or otherwise of MND ceiling in hypertrophy. The aim of this review is to summarise the literature about the MND ceiling in various settings of hypertrophy and discuss the possible factors contributing to a discrepancy in the literature. We conclude by describing the physiological and clinical significance of the MND ceiling limit in the muscle adaptation process in various physiological and pathological conditions.
Humans ; Muscle Fibers, Skeletal/physiology* ; Hypertrophy/pathology* ; Muscle, Skeletal

Objective: To review the clinical data of 7 patients with Danon disease and analyze their clinical characteristics. Methods: The medical records of 7 patients with Danon disease, who were hospitalized in Peking Union Medical College Hospital of Chinese Academy of Medical Sciences from April 2008 to July 2021, were reviewed and summarized, of which 6 cases were diagnosed as Danon disease by lysosomal-associated membrane protein-2 (LAMP-2) gene mutation detection and 1 case was diagnosed by clinicopathological features. Clinical manifestations, biochemical indexes, electrocardiogram, echocardiography, skeletal muscle and myocardial biopsy and gene detection results were analyzed, and patients received clinical follow-up after discharge. Results: Six patients were male and average age was (15.4±3.5) years and the average follow-up time was (27.7±17.0) months. The main clinical manifestations were myocardial hypertrophy (6/7), decreased myodynamia (2/7) and poor academic performance (3/7). Electrocardiogram features included pre-excitation syndrome (6/7) and left ventricular hypertrophy (7/7). Echocardiography examination evidenced myocardial hypertrophy (6/7), and left ventricular dilatation and systolic dysfunction during the disease course (1/7). The results of skeletal muscle biopsy in 6 patients were consistent with autophagy vacuolar myopathy. Subendocardial myocardial biopsy was performed in 3 patients, and a large amount of glycogen deposition with autophagosome formation was found in cardiomyocytes. LAMP-2 gene was detected in 6 patients, and missense mutations were found in all these patients. During the follow-up period, implantable cardioverter defibrillator implantation was performed in 1 patient because of high atrioventricular block 4 years after diagnosis, and there was no death or hospitalization for cardiovascular events in the other patients. Conclusion: The main clinical manifestations of Danon disease are cardiomyopathy, myopathy and mental retardation. Pre-excitation syndrome is a common electrocardiographic manifestation. Autophagy vacuoles can be seen in skeletal muscle and myocardial pathological biopsies. LAMP-2 gene mutation analysis is helpful in the diagnose of this disease.
Adolescent ; Child ; Female ; Humans ; Male ; Cardiomyopathies/etiology* ; Glycogen Storage Disease Type IIb/complications* ; Hypertrophy, Left Ventricular/etiology* ; Lysosomal-Associated Membrane Protein 2/genetics* ; Pre-Excitation Syndromes/genetics*

Septal reduction therapies, which include septal myectomy and alcohol septal ablation and so on, are the current treatment strategies for patients with obstructive hypertrophic cardiomyopathy and drug-refractory symptoms. With the deepening of theoretical understanding and the rapid development of interventional therapies, some researchers have tried to perform transcatheter mitral valve edge-to-edge repair to treat high-risk patients with hypertrophic cardiomyopathy, including obstructive and non-obstructive. The reported results are relatively satisfactory, but many urgent problems need to be solved, such as the lack of data on animal experiments and large cohort studies, and the unknown medium- and long-term outcomes. However, transcatheter mitral valve edge-to-edge repair brings new ideas for the diagnosis and treatment of patients with hypertrophic cardiomyopathy. On one hand, it can be used as a monotherapy, on the other hand, it can be combined with novel molecular targeted drug therapy or emerging minimally invasive surgical procedures targeting hypertrophic ventricular septum, which deserves our further attention and exploratory research.
Humans ; Treatment Outcome ; Cardiomyopathy, Hypertrophic/surgery* ; Mitral Valve/surgery* ; Ventricular Septum/surgery* ; Hypertrophy

Humans ; Child ; Adenoids/surgery* ; Palatine Tonsil/surgery* ; Adenoidectomy ; Hypertrophy/surgery* ; Tonsillectomy

Objective:To explore the effects of mouth opening breathing for different reasons on children's maxillofacial development. Methods:One hundred and fifty-one children were selected as the research objects of this experiment. They were divided into 49 cases of adenoid hypertrophy group（group A）, 52 cases of tonsillar hypertrophy group（group B） and 50 cases of adenoid with tonsillar hypertrophy group（Group C）. Healthy children in the same period were selected as the control group, a total of 45 cases. The reflex nasopharyngeal measurement parameters, facial development indexes and cephalometric parameters of group A, group B, group C and control group were analyzed, and the incidence of Angle ClassⅡand Angle Class Ⅲ in group A, group B and group C were studied. Results:Compared with the control group, the reflex nasopharyngeal measurement parameters in group A, group B and group C was significantly different（P<0.05）, and the cephalometric parameters changed with variation in groups（P<0.05）. The incidence of Angle Class Ⅱ facial pattern in group A and group C was higher, but the incidence of Angle Class Ⅲ facial pattern in group B and group C was higher（P<0.05）. Conclusion:Adenoid hypertrophy leads to mandibular retraction; tonsil hypertrophy leads to anterior mandibular arch; adenoid hypertrophy and tonsil hypertrophy are easy to lead to clockwise rotation of the mandible. In clinical practice, to avoid children's uncoordinated maxillofacial development, we should correct the maxillofacial situation of children as soon as possible.
Child ; Humans ; Maxillofacial Development ; Malocclusion, Angle Class III/complications* ; Nasopharynx ; Adenoids ; Palatine Tonsil ; Mouth Breathing/etiology* ; Hypertrophy/complications* ; Mouth

Objective:To discuss the application of virtual endoscopy in the diagnosis of adenoid hypertrophy and the morphologic classification of adenoid. Methods:The clinical data of 97 children with adenoid hypertrophy admitted to Department of Otorhinolaryngology Head and Neck Surgery, Shenzhen University General Hospital from July 2022 to December 2022 were collected. The virtual endoscopic reconstruction of the nasopharynx was performed by cone beam computed tomography. The results of virtual endoscopic adenoid size measurement were compared with the results of nasopharyngeal CT median sagittal position and nasopharyngeal endoscopy. Virtual endoscopic classification of adenoid based on the size of the adenoids and their relationship with the torus tubarius. Results:The t-test results of the size of adenoids measured by virtual endoscopy and nasopharyngeal CT were t=1.699 and P=0.093, and the results of intra-group correlation coefficient（ICC） analysis were ICC=0.921 and P<0.01. The proportion of adenoids measured by virtual endoscopy and nasopharyngeal CT was highly consistent. The t-test results of the size of adenoids measured virtual endoscopy and nasopharyngeal endoscopy were t=1.543 and P=0.15, and the results of intra-group correlation coefficient（ICC） analysis were ICC=0.900 and P<0.01. The proportion of adenoids measured by virtual endoscopy and nasopharyngeal endoscopy was highly consistent. Among the 97 children, the morphological classification results of adenoids were 48 cases of overall hypertrophy type, 47 cases of central bulge type, and 2 cases of flat thickening type. Conclusion:The diagnosis of adenoid hypertrophy by virtual endoscopy has high accuracy, which not only avoids the invasive operation of traditional nasopharyngeal endoscopy, but also can observe the adenoid condition and its relationship with the torus tubarius from multiple angles. And, the morphological classification of adenoids using virtual endoscopy has guiding significance for perioperative preparation.
Child ; Humans ; Adenoids/surgery* ; Nasopharynx/diagnostic imaging* ; Adenoidectomy ; Endoscopy/methods* ; Hypertrophy/surgery*

Objective: To assess the clinical features and relative factors of left ventricular hypertrophy (LVH) in children with primary hypertension. Methods: In this retrospective cohort study, 430 children diagnosed with primary hypertension in Children's Hospital, Capital Institute of Pediatrics from January 2019 to September 2022 were enrolled. Their clinical data was analyzed and LVH was assessed by echocardiography. According to left ventricular geometry, these children were assigned to the LVH group and normal geometry group. General conditions, laboratory indicators and ambulatory blood pressure parameters between two groups were compared by independent sample t-test or Mann-Whitney U test. Spearman correlation coefficient was used to analyze the correlation between LVH and clinical indicators including blood pressure, biochemical and metabolic indicators. The independent risk factors of LVH were analyzed by multivariable logistic regression. The receiver operating characteristic (ROC) curve was used to explore the value of risk factors in the diagnosis of LVH. Results: Among the 430 children with primary hypertension, 342 (79.5%) were males and 88 (20.5%) females. Their age was (12.6±2.3) years, and 123 children (28.6%) of them had LVH. Body mass index (BMI) ((30.0±5.2) vs. (26.2±4.3) kg/m²), ratio of stage 2 hypertension (75.6% (93/123) vs. 59.6% (183/307)), 24-hour systolic blood pressure (24 h SBP)((131±10) vs. (128±10) mmHg,1 mmHg=0.133 kPa), daytime systolic blood pressure (SBP) ((135±11) vs. (131±11) mmHg), nighttime SBP ((128±11) vs. (123±10) mmHg), cholesterol level ((4.0±0.7) vs. (3.9±0.7) mmol/L), serum uric acid level ((447±81) vs. (426±91) μmol/L) and incidence of hyperinsulinemia (69.9% (86/123) vs.59.0% (181/307)) were significantly elevated in the LVH group compared with those in the normal geometry group (all P<0.05). There were more patients with a disease course over 5 years in the LVH group than in the normal geometry group, with a statistically significant difference (χ²=8.90,P=0.031). Spearman correlation analysis showed that BMI, 24 h SBP, daytime SBP, nighttime SBP, triglyceride, uric acid, and serum sodium level were positively correlated with LVMI (r=0.43, 0.20, 0.18, 0.18, 0.18, 0.16, and 0.12, all P<0.05). BMI, hyperinsulinemia, and cholesterol level were positively correlated with relative wall thickness (RWT) (r=0.22, 0.12, and 0.16, all P<0.05). The multivariate logistic regression analysis showed that BMI (OR=1.17, 95%CI 1.10-1.25) and 24 h SBP (OR=1.04, 95%CI 1.01-1.08) were the independent risk factors for LVH (both P<0.05). The area under the receiver operator characteristic curve, combined with BMI and 24 h SBP, was 0.72 (95%CI 0.67-0.77, P<0.05), with a sensitivity and specificity of 71.5% and 64.8%, respectively. Conclusions: BMI and 24 h SBP are the independent risk factors for LVH in children with primary hypertension, and the combination of BMI and 24 h SBP has an acceptable diagnostic value for LVH. Early monitoring of these indexes is necessary to predict preclinical cardiac damage.
Male ; Female ; Humans ; Child ; Adolescent ; Hypertension/diagnosis* ; Hypertrophy, Left Ventricular/etiology* ; Uric Acid ; Blood Pressure Monitoring, Ambulatory ; Retrospective Studies ; Blood Pressure/physiology* ; Risk Factors ; Essential Hypertension ; Hyperinsulinism ; Cholesterol