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Multiple acyl CoA dehydrogenase deficiency/glutaric aciduria type II ethylmalonic-adipic aciduria
Published in William L. Nyhan, Georg F. Hoffmann, Aida I. Al-Aqeel, Bruce A. Barshop, Atlas of Inherited Metabolic Diseases, 2020
William L. Nyhan, Georg F. Hoffmann, Aida I. Al-Aqeel, Bruce A. Barshop
A few patients developed a progressive extrapyramidal movement disorder [58]. Uziel and colleagues described a boy with gradually progressive spastic ataxia and leukodystrophy without ever having experienced episodic metabolic crises [59]. One adult patient presented for several years with cyclic vomiting and was initially diagnosed with cyclic vomiting syndrome [60]. A depressive state and intermittent nausea were the first symptoms of an adolescent patient with late-onset riboflavin-responsive MADD [61]. Brain magnetic resonance imaging of this patient showed disseminated high-intensity areas in the periventricular white matter and in the splenium of the corpus callosum on T2-weighted images and fluid-attenuated inversion-recovery images before starting the treatment. In summary, late-onset MADD is characterized by a progressive myopathy of varying degrees and time course, risk of acute deteriorations and metabolic decompensation, and, occasionally, additional neurologic symptoms.
Cholinergic Agonists
Published in Sahab Uddin, Rashid Mamunur, Advances in Neuropharmacology, 2020
Rupali Patil, Aman Upaganlawar
Muscarinic agonists act on blood vessels and show generalized vasodilation due to availability of M3 receptors on the endothelial lining of the vessel. They show fall in BP though they do not have a parasympathetic supply. Stimulation of M3 receptors releases NO by the action of nitric oxide synthase on l-arginine. NO causes accumulation of cyclic guanosine monophosphate (cGMP) responsible for smooth muscle relaxation (Sneddon and Graham, 1992).
Platelet Disorders Douglas Triplett
Published in Genesio Murano, Rodger L. Bick, Basic Concepts of Hemostasis and Thrombosis, 2019
Although cyclic thrombocytopenia is an unusual clinical state, it falls within the group of ineffective thrombopoiesis.217 In this disorder, there is alternating thrombocytosis and thrombocytopenia at regular intervals.
Cyclic thrombocytopenia associated with estradiol: a case report
Published in Hematology, 2023
Ningbo Pang, Yingwei Li, Kangxi Zhou, Chunliang Liu, Rong Yan, Chenglin Sun, Weiling Xiao, Changgeng Ruan, Zhimin Zhai, Kesheng Dai
Cyclic thrombocytopenia (CTP) is a rare blood disorder characterized by periodic fluctuations in platelet counts [1–3]. Each cycle usually lasts three to five weeks [2,3]. The median nadir and peak platelet counts in this reported cases were around 10 × 109/L and 330 × 109/L [2,3]. Therefore, periods of life-threatening thrombocytopenia or rebound increased the risk of thrombosis found in some CTP patients [1–4]. Due to the symptom of low platelet counts, CTP is often misdiagnosed as idiopathic thrombocytopenic purpura (ITP). However, distinct from ITP, CTP generally responds poorly to most treatments used successfully in ITP, such as corticosteroids, splenectomy, and intravenous immunoglobulin [1,2,5]. The characteristic of CTP is a heterogeneous disorder. Although autoimmune platelet destruction [6], megakaryocytic hypoplasia or aplasia [7], infectious [8], and hormonal [9–11] etiology were thought to be potential mechanisms, the pathogenesis of CTP is still unclear [1,2]. Interestingly, CTP was more commonly found in females [2], a predominance disproportionately in favor of pre-menopausal women, and these fluctuations of platelets are in phase with the menstrual cycle [2,9,12–14], suggesting the correlation of hormonal cycle may be associated with fluctuations in platelet counts.
Low patient engagement is associated with reduced health-related quality of life in adults with cyclic vomiting syndrome
Published in Scandinavian Journal of Gastroenterology, 2022
Geoffrey Dang-Vu, Lisa Rein, Aniko Szabo, Thangam Venkatesan
Cyclic Vomiting Syndrome (CVS) is a chronic disorder of gut-brain interaction marked by recurrent episodes of severe nausea and vomiting in between periods of otherwise normal health [1]. This stereotypical pattern of vomiting is often triggered by psychological or physiological stress [2]. CVS is also associated with multiple comorbid chronic conditions such as anxiety, depression, autonomic dysfunction and migraine [3]. As a result, patients generally have the poor health-related quality of life (HRQOL) and increased health care utilization [4]. Treatment of CVS consists of prophylactic therapy to prevent episodes, abortive therapy at the onset of symptoms, and addressing psychosocial comorbidity to help with chronic disease management which is pivotal to achieving better outcomes in these patients [5]. This requires a high degree of engagement by all stakeholders including healthcare providers and patients. However, to date, there is no data on patient engagement in CVS and how this impacts overall HRQOL in these patients.
Cyclic guanosine monophosphate and 10-year change in left ventricular mass: the Multi-Ethnic Study of Atherosclerosis (MESA)
Published in Biomarkers, 2021
Vinita Subramanya, Di Zhao, Pamela Ouyang, Wendy Ying, Dhananjay Vaidya, Chiadi E. Ndumele, Joao A. Lima, Eliseo Guallar, Ron C. Hoogeveen, Sanjiv J. Shah, Susan R. Heckbert, David A. Kass, Wendy S. Post, Erin D. Michos
Heart failure with preserved ejection fraction (HFpEF) accounts for half of all HF cases and is more prevalent in older women.Cyclic guanosine monophosphate (cGMP) is a second messenger that is involved in cardiac remodelling. - Much of what is known about cGMP and its effects on the heart and vasculature comes from largely preclinical studies.The aim of this study was the to assess the association of plasma cGMP with left ventricular remodelling and to evaluate for sex differences in these associations, in a multi-ethnic population of older men and post- menopausal women.We found that cGMP levels were associated with an increase in left ventricular mass over 10 years, perhaps reflecting compensatory changes.Further studies are needed to understand the mechanistic roles of cGMP in cardiac remodelling and associated sex differences.