keyword
https://read.qxmd.com/read/38605407/clinical-spectrum-over-12-year-follow-up-and-experience-of-sglt2-inhibitors-treatment-on-patients-with-glycogen-storage-disease-type-ib-a-single-center-retrospective-study
#1
JOURNAL ARTICLE
Yong-Xian Shao, Cui-Li Liang, Ya-Ying Su, Yun-Ting Lin, Zhi-Kun Lu, Rui-Zhu Lin, Zhi-Zi Zhou, Chun-Hua Zeng, Chun-Yan Tao, Zong-Cai Liu, Wen Zhang, Li Liu
BACKGROUND: Glycogen storage disease type Ib (GSD Ib) is a rare disorder characterized by impaired glucose homeostasis caused by mutations in the SLC37A4 gene. It is a severe inherited metabolic disease associated with hypoglycemia, hyperlipidemia, lactic acidosis, hepatomegaly, and neutropenia. Traditional treatment consists of feeding raw cornstarch which can help to adjust energy metabolism but has no positive effect on neutropenia, which is fatal for these patients. Recently, the pathophysiologic mechanism of the neutrophil dysfunction and neutropenia in GSD Ib has been found, and the treatment with the SGLT2 inhibitor empaglifozin is now well established...
April 11, 2024: Orphanet Journal of Rare Diseases
https://read.qxmd.com/read/38599724/pediatric-hyperlipidemia
#2
REVIEW
Ankit Garg, S Radhakrishnan
The leading cause of mortality worldwide is atherosclerotic cardiovascular disease. Atherosclerotic plaques are well known to originate early in the childhood. Identifying hyperlipidemia in early childhood creates an opportunity to prevent major cardiovascular events in adults. Children with identified risk factors are at an increased risk of developing cardiovascular incidents in later life. This article emphasizes the diagnosis and management of pediatric hyperlipidemia with reference to the recent guidelines...
March 2024: Indian Heart Journal
https://read.qxmd.com/read/38561799/circulating-ceramide-levels-and-ratios-in-emirati-youth-under-18-years-associations-with-cardiometabolic-risk-factors
#3
JOURNAL ARTICLE
Youssef M Shalaby, Bashar Al-Zohily, Anjana Raj, Javed Yasin, Sania Al Hamad, Charalambos Antoniades, Nadia Akawi, Elhadi H Aburawi
BACKGROUND: Circulating ceramide (Cer) drives various pathological processes associated with cardiovascular diseases, liver illness, and diabetes mellitus. Although recognized as predictors of cardiometabolic diseases (CMD) in research and clinical settings, their potential for predicting CMD risk in individuals under 18 remains unexplored. OBJECTIVES: This study was designed to utilize Liquid Chromatography-Mass Spectrometry (LC-MS/MS) methodology to determine the biological reference ranges for Cer in plasma samples of Emirati children and develop a risk assessment score (CERT-1) based on Cer concentrations...
April 1, 2024: Lipids in Health and Disease
https://read.qxmd.com/read/38544995/analysis-of-the-effect-of-changes-in-serum-osteopontin-levels-on-patients-with-acute-cerebral-infarction
#4
JOURNAL ARTICLE
Ying Zhang, Jia-Rong Wang, Er-Na Zhang, Zhi-Jiang Zhao
OBJECTIVE: To investigate the correlation of serum osteopontin levels with disease severity and prognosis in patients with acute cerebral infarction. METHODS: This retrospective analysis included forty patients with acute cerebral infarction (ACI) admitted to the Department of Neurology of Baoding Children's Hospital from May, 2019 to May, 2022 within 24 hours of onset were selected as the observation group, while 40 healthy subjects in our hospital during the same period were selected as the control group...
2024: Pakistan Journal of Medical Sciences Quarterly
https://read.qxmd.com/read/38509578/gene-variants-and-clinical-characteristics-of-children-with-sitosterolemia
#5
JOURNAL ARTICLE
Rui Gu, Hui Wang, Chun-Lin Wang, Mei Lu, Miao Miao, Meng-Na Huang, Yi Chen, Yang-Li Dai, Ming-Qiang Zhu, Qiong Zhou, Chao-Chun Zou
OBJECTIVE: To enhance the detection, management and monitoring of Chinese children afflicted with sitosterolemia by examining the physical characteristics and genetic makeup of pediatric patients. METHODS: In this group, 26 children were diagnosed with sitosterolemia, 24 of whom underwent genetic analysis. Patient family medical history, physical symptoms, tests for liver function, lipid levels, standard blood tests, phytosterol levels, cardiac/carotid artery ultrasounds, fundus examinations, and treatment were collected...
March 20, 2024: Lipids in Health and Disease
https://read.qxmd.com/read/38392258/apolipoprotein-b-and-lipid-profile-in-italian-children-and-adolescents
#6
JOURNAL ARTICLE
Francesco Martino, Tarcisio Niglio, Eliana Martino, Vincenzo Paravati, Luisa de Sanctis, Ornella Guardamagna
RATIONALE: The prevention of cardiovascular (CV) disease is mandatory from childhood onwards. Among biochemical markers related to the clinical cardiovascular outcome, LDL cholesterol (LDL-C), non-HDL-C and apolipoprotein B (ApoB) are recognized as main target parameters. Emphasis on ApoB concentrations is growing, as representative of any class of atherogenic lipoprotein. This consideration allows checking of subjects under 18 years of age when the CV risk occurs. The aim of this study is to evaluate ApoB levels in a sample of Italian hyperlipidemic children and adolescents, and their siblings, to test any relationship with their lipid profile...
January 29, 2024: Journal of Cardiovascular Development and Disease
https://read.qxmd.com/read/38388929/establishing-non-fasting-reference-values-for-plasma-lipids-levels-based-on-age-sex-and-puberty-stage-in-a-french-canadian-pediatric-population
#7
JOURNAL ARTICLE
Sophie Bouhour, Rosalie Plantefève, Virginie Gillet, Armita Abolghasemi, Fatima Zahra Bouchouirab, Andrea A Baccarelli, Larissa Takser, Artuela Çaku
BACKGROUND: Dyslipidemias, including familial hypercholesterolemia (FH), are a significant risk factor for cardiovascular diseases. FH is a genetic disorder resulting in elevated levels of low-density lipoprotein cholesterol (LDL-C) and an increased probability of early cardiovascular disorders. Heterozygous familial hypercholesterolemia (HeFH) is the most common form, affecting approximately 1 in 250 individuals worldwide, with a higher prevalence among the French-Canadian population...
February 22, 2024: Lipids in Health and Disease
https://read.qxmd.com/read/38369194/evaluation-of-cardiovascular-risk-factors-in-children-aged-6-16-years-and-their-evolution-at-early-adulthood-in-a-10-year-follow-up-study
#8
JOURNAL ARTICLE
Eleni Ntretsiou, Dimitris Benas, Paraskevi Trivilou, George Pavlidis, Ignatios Ikonomidis, Achilleas Attilakos, Efstathios Iliodromitis, Helen Triantafyllidi
AIM: Obesity and arterial hypertension (AH) in children represent well recognized risk factors for cardiovascular (CV) events during adult life. We investigated any changes regarding several CV risk (CVR) factors of children after a 10-year follow-up period. METHODS: A cohort of 143 healthy children, elementary/high-school students, 6-16 years old was initially evaluated in 2010-2011 regarding CVR factors [obesity, blood pressure (BP), aortic stiffness (PWV), lipid profile] plus food habits/sports activity...
February 16, 2024: Hellenic Journal of Cardiology: HJC
https://read.qxmd.com/read/38183071/repeated-implants-failure-in-young-patient-with-idiopathic-nephrotic-syndrome-a-case-report-with-brief-review-of-the-literature
#9
JOURNAL ARTICLE
Lamees R Alssum
BACKGROUND: Nephrotic syndrome is a chronic disorder characterized by heavy proteinuria, hypoalbuminemia, hyperlipidemia, and edema. Idiopathic minimal-change disease is the most common form encountered in children. Corticosteroids are the cornerstone for the treatment of idiopathic nephrotic syndrome (INS), with different regimens depending on the response to therapy and frequency of relapses. This case report presents complications after implant treatment in patient with INS. CASE PRESENTATION: 20 years old female patient presented for implant consultation...
January 5, 2024: BMC Oral Health
https://read.qxmd.com/read/38133765/mechanisms-and-risk-factors-of-metabolic-syndrome-in-children-and-adolescents
#10
REVIEW
Valentina Codazzi, Giulio Frontino, Luca Galimberti, Andrea Giustina, Alessandra Petrelli
Metabolic syndrome (MetS) is a complex disorder characterized by abdominal obesity, elevated blood pressure, hyperlipidemia, and elevated fasting blood glucose levels. The diagnostic criteria for MetS in adults are well-established, but there is currently no consensus on the definition in children and adolescents. The etiology of MetS is believed to involve a complex interplay between genetic predisposition and environmental factors. While genetic predisposition explains only a small part of MetS pathogenesis, modifiable environmental risk factors play a significant role...
December 22, 2023: Endocrine
https://read.qxmd.com/read/38096914/expression-and-role-of-pdk4-on-childhood-dyslipidemia-and-lipid-metabolism-in-hyperlipidemic-mice
#11
JOURNAL ARTICLE
Wenjuan Cai, Xin Wang, Qian Deng, Jian Gao, Yuqing Chen
Hyperlipidemia is a common metabolic disorder that can lead to cardiovascular disease. PDK4 is a key enzyme that regulates glucose and fatty acid metabolism and homeostasis. The aim of this study is to explore the correlation between PDK4 expression and dyslipidemia in obese children, and to find new therapeutic targets for hyperlipidemia in children. The expression of PDK4 in serum was detected by qRT-PCR. Receiver operating characteristic curve was used to analyze the relationship between PDK4 and dyslipidemia...
December 14, 2023: Hormone and Metabolic Research
https://read.qxmd.com/read/38016774/-analysis-of-clinical-and-genetic-variation-in-neonatal-intrahepatic-cholestasis-caused-by-citrin-deficiency
#12
JOURNAL ARTICLE
L L Ge, C F Chen, L Liu, X Zheng, X M Zhang, Y D Zhang, S Y Mei
Objective: To investigate the clinical phenotype and gene variation conditions in neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD), so as to provide a basis for genetic counseling and clinical diagnosis and treatment of the family. Methods: 11 cases of neonatal intrahepatic cholestasis who visited the Children's Hospital Affiliated to Zhengzhou University between February 2019 and March 2021 were selected as the study subjects. High-throughput sequencing technology was used to detect the gene variation condition in 11 neonatal patients and 100 normal control neonates...
October 20, 2023: Zhonghua Gan Zang Bing za Zhi, Zhonghua Ganzangbing Zazhi, Chinese Journal of Hepatology
https://read.qxmd.com/read/38009919/influence-of-post-disaster-evacuation-on-childhood-obesity-and-liver-dysfunction-the-fukushima-health-management-survey
#13
JOURNAL ARTICLE
Mitsuaki Hosoya, Hironori Nakano, Koichi Hashimoto, Tetsuya Ohira, Akira Sakai, Michio Shimabukuro, Seiji Yasumura, Hitoshi Ohto, Kenji Kamiya
BACKGROUND: After the Great East Japan Earthquake and subsequent Fukushima Daiichi Nuclear Power Plant accident in 2011, the Fukushima Prefectural Government launched a long-term health management survey for the population of Fukushima. Results of the Comprehensive Health Check (CHC) showed that some children aged 6-15 years, who resided in the evacuation area at the time of the disaster, had obesity, hyperlipidemia, liver dysfunction, and/or renal dysfunction from as early as 2011...
2023: Pediatrics International: Official Journal of the Japan Pediatric Society
https://read.qxmd.com/read/37990466/-a-case-of-interstitial-lung-and-liver-disease-caused-by-mars1-gene-mutation
#14
JOURNAL ARTICLE
Wen-Jing Peng, Yan Zhu, Lai-Shuan Wang, Wei Lu, Lin Yang, Li Zhu
The patient is a female infant, 4 months and 9 days old, who was admitted to the hospital due to recurrent fever, cough, and hepatomegaly for over a month. The patient was a healthy full-term infant with a normal birth history. At 2 months and 22 days after birth, she developed recurrent fever, cough, and respiratory distress. Chest imaging revealed diffuse bilateral lung lesions, and fiberoptic bronchoscopy showed interstitial changes in both lungs. These suggested the presence of interstitial lung disease...
November 15, 2023: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://read.qxmd.com/read/37953296/inflammatory-dilated-cardiomyopathy-associated-with-psoriasis-a-case-report
#15
JOURNAL ARTICLE
Hamidreza Riasi, Emad Asgari Jafarabadi, Hadis Enayati, Ali Fanoodi, Shiva Salehi, Ali-Reza Jamshidi, Forod Salehi, Azam Rezaee
BACKGROUND: Psoriasis is a chronic inflammatory skin disease with a genetic basis. Psoriasis is accepted as a systemic, immune-mediated disease. Hypertension, obesity, metabolic disorders including diabetes mellitus and hyperlipidemia, and psychiatric disorders are more prevalent among children with psoriasis compared to children without psoriasis. In this study, we report a case of dramatic response of inflammatory cardiomyopathy to anti-inflammatory treatment of psoriasis; which might reveal similar pathogenesis basis of these two diseases...
November 13, 2023: Journal of Medical Case Reports
https://read.qxmd.com/read/37915902/the-long-term-benefits-of-early-intensive-therapy-in-chronic-diseases-the-legacy-effect
#16
REVIEW
Carmine Zoccali, Francesca Mallamaci, Giovanni Tripepi, Edouard L Fu, Vianda S Stel, Friedo W Dekker, Kitty J Jager
The 'legacy effect' refers to the long-term benefits of intensive therapy that are observed long after the end of clinical trials and trial interventions in chronic diseases such as diabetes, hyperlipidaemia and hypertension. It emphasizes the importance of intensive treatment to prevent long-term complications and mortality. In chronic kidney disease (CKD), the legacy effect is evident in various studies. Long-term nephroprotection in diabetes is well documented in major studies in the early stages of diabetes, such as Diabetes Control and Complications Trial-Epidemiology of Diabetes Interventions and Complications (DCCT-EDIC), UK Prospective Diabetes Study (UKPDS) and Intensified Multifactorial Intervention in Patients with Type 2 Diabetes and Microalbuminuria (STENO-2)...
November 2023: Clinical Kidney Journal
https://read.qxmd.com/read/37869045/a-study-of-dyslipidemia-and-its-clinical-implications-in-childhood-nephrotic-syndrome
#17
JOURNAL ARTICLE
Pritikar Dowerah, Arpita Gogoi, Caroline D Shira, Bikash Sarkar, Sarada Mazumdar
BACKGROUND: Nephrotic syndrome in children is characterized by dyslipidemia, which is an indirect risk factor for cardiovascular diseases, progressive glomerulosclerosis, and related complications. The objective is to determine the range of lipid profile abnormalities in relation to onset, relapse, and remission, as well as to determine if there is any relationship between dyslipidemia and the frequency of relapses. METHODS: One hundred and two diagnosed cases of nephrotic syndrome in the age group of less than 12 years were included, out of which 64 patients belonged to the first episode of nephrotic syndrome and 38 patients were relapse cases...
October 2023: Curēus
https://read.qxmd.com/read/37831337/adiponectin-rs1501299-and-chemerin-rs17173608-gene-polymorphism-in-children-with-type-1-diabetes-mellitus-relation-with-macroangiopathy-and-peripheral-artery-disease
#18
JOURNAL ARTICLE
N Y Salah, S S Madkour, K S Ahmed, D A Abdelhakam, F A Abdullah, R A E H Mahmoud
AIM: Although macrovascular complications represent the leading cause of mortality in type 1 diabetes mellitus (T1DM), the prevalence of subtle macrovascular affection including peripheral artery disease (PAD) among children with T1DM and its genetic predictors remains to be unraveled. Increasing evidence suggests a link between adiponectin rs1501299 and chemerin rs17173608 gene polymorphism and atherogenesis, and insulin resistance. Hence, this study assess the prevalence of these variants among children with T1DM in comparison to healthy controls and their association with macrovascular complications, namely PAD and hyperlipidemia...
October 13, 2023: Journal of Endocrinological Investigation
https://read.qxmd.com/read/37776083/hyperlipidemia-in-children-and-adolescents-with-acute-lymphoblastic-leukemia-a-systematic-review-and-meta-analysis
#19
REVIEW
Renate Dagsdottir Laumann, Louise Lindkvist Pedersen, Liv Andrés-Jensen, Christian Mølgaard, Kjeld Schmiegelow, Thomas Leth Frandsen, Bodil Als-Nielsen
BACKGROUND: The established association between acute lymphoblastic leukemia (ALL) and hyperlipidemia has, in some studies, been linked to toxicities such as pancreatitis, thrombosis, and osteonecrosis. However, a systematic review investigating the incidence, management, and clinical implications of hyperlipidemia during childhood ALL treatment is lacking. OBJECTIVES: Systematically assess the incidence of hyperlipidemia during ALL treatment, explore associations with risk factors and severe toxicities (osteonecrosis, thrombosis, and pancreatitis), and review prevalent management strategies...
December 2023: Pediatric Blood & Cancer
https://read.qxmd.com/read/37720341/assessing-adherence-to-clinical-practice-guidelines-among-primary-care-physicians-in-bahrain-a-cross-sectional-study
#20
JOURNAL ARTICLE
Dhabya Al Sada, Yusra Irshad Husain, Zainab Al Misbah
OBJECTIVES: To assess the adherence to clinical practice guidelines (CPGs) and explore the barriers to their implementation among primary care physicians (PCPs) in Bahrain. METHODS: A cross-sectional study was conducted using an online survey among physicians working in 20 randomly selected public sector primary health centers in Bahrain. Private-sector physicians and family medicine residents were excluded. Outcome measures were assessing the extent of adherence to CPGs, describing the results in association with work roles, gender, level of formal training, and years of working experience, and investigating the barriers to adhering to CPGs...
July 2023: Oman Medical Journal
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