scholarly journals Variables Associated with Glomerular Hyperfiltration in Fabry Disease Patients

2020 ◽  
Vol 12 (3) ◽  
Author(s):  
Fernando Perretta ◽  
Norberto Antongiovanni ◽  
Sebastian Jaurretche
Keyword(s):  
Nervous System ◽  
Fabry Disease ◽  
Peripheral Nervous System ◽  
Cardiac Arrhythmia ◽  
Glomerular Hyperfiltration ◽  
Gastrointestinal Involvement ◽  
Renin Angiotensin Aldosterone System ◽  
Renin Angiotensin ◽  
System P ◽  
Cornea Verticillata

Background: Fabry disease is a genetic disorder caused by the deficiency of the lysosomal α-galactosidase A enzyme. This failure generates the storage of globotriaosylceramide in different cells with a progressive multi-organ involvement. Objectives: To report the prevalence of glomerular hyperfiltration in Fabry disease patients and the association with clinical variables. Methods: Adult patients (≥ 18 years) at the moment of FD diagnosis were evaluated. The variables studied were: central and peripheral nervous system compromise, presence of arterial hypertension, cardiac arrhythmia, left ventricular hypertrophy, albuminuria/proteinuria, cornea verticillata, gastrointestinal involvement, treatment with inhibitors of the renin-angiotensin-aldosterone system, deafness, and presence of angiokeratomas. Results: Forty-eight adults with Fabry disease (35.9 ± 11.7 years), 28 women (58.3%), and 20 men (41.7%) were analyzed. Nine (18.8%) patients with glomerular hyperfiltration, including six females and three males (mean age: 28.8 years), were detected. A significant association between and central nervous system (P = 0.021) and peripheral nervous system (P = 0.001) compromise, cardiac arrhythmia (P = 0.001), cornea verticillata (P = 0.009), and gastrointestinal involvement (P = 0.009) was observed. However, no association was found between glomerular hyperfiltration and proteinuria or treatment with inhibitors of the renin-angiotensin-aldosterone system. Conclusions: This research showed a higher prevalence of glomerular hyperfiltration in the younger group and a significant association between glomerular hyperfiltration and some typical manifestations of classic Fabry patients. Although more studies are needed, it is concluded that other mechanisms than glomerular hyperfiltration, like injury by glycosphingolipids deposit into the filtration barrier, might influence the protein loss in Fabry nephropathy.

scholarly journals MicroRNAs Regulating Renin–Angiotensin–Aldosterone System, Sympathetic Nervous System and Left Ventricular Hypertrophy in Systemic Arterial Hypertension

Biomolecules ◽  
2021 ◽  
Vol 11 (12) ◽  
pp. 1771
Author(s):  
Alex Cleber Improta-Caria ◽  
Marcela Gordilho Aras ◽  
Luca Nascimento ◽  
Ricardo Augusto Leoni De Sousa ◽  
Roque Aras-Júnior ◽  
...  
Keyword(s):  
Nervous System ◽  
Sympathetic Nervous System ◽  
Left Ventricular Hypertrophy ◽  
Signaling Pathways ◽  
Ventricular Hypertrophy ◽  
Left Ventricular ◽  
Renin Angiotensin Aldosterone System ◽  
Renin Angiotensin ◽  
Gene And Protein Expression ◽  
Sympathetic Nervous

MicroRNAs are small non-coding RNAs that regulate gene and protein expression. MicroRNAs also regulate several cellular processes such as proliferation, differentiation, cell cycle, apoptosis, among others. In this context, they play important roles in the human body and in the pathogenesis of diseases such as cancer, diabetes, obesity and hypertension. In hypertension, microRNAs act on the renin–angiotensin–aldosterone system, sympathetic nervous system and left ventricular hypertrophy, however the signaling pathways that interact in these processes and are regulated by microRNAs inducing hypertension and the worsening of the disease still need to be elucidated. Thus, the aim of this review is to analyze the pattern of expression of microRNAs in these processes and the possible associated signaling pathways.

Nervous system

2013 ◽  
pp. 140-145
Author(s):  
Andrew Graham ◽  
Clare Galton
Keyword(s):  
Nervous System ◽  
Peripheral Nervous System ◽  
Lupus Erythematosus ◽  
Transverse Myelitis ◽  
Neurological Complications ◽  
Neurological Involvement ◽  
Disease Modifying Therapy ◽  
Entrapment Neuropathies ◽  
System P ◽  
Tunnel Syndrome

Rheumatological conditions may be complicated by a variety of both central and peripheral nervous system disorders. Common complications such as entrapment neuropathies are familiar to rheumatologists but accurate diagnosis of less common neurological disorders may be challenging; careful clinical reasoning is essential, supplemented where necessary by imaging, neurophysiology, and other special investigations including cerebrospinal fluid examination. Complications vary according to the nature of the background condition. In rheumatoid arthritis, neurological involvement is typically related to the mechanical consequences of advancing disease; most commonly, entrapment neuropathies such as carpal tunnel syndrome and cervical myelopathy due to atlantoaxial subluxation. By contrast, neurological involvement in systemic lupus erythematosus (SLE) tends to occur earlier in the disease course, with a much wider range of manifestations. The management of stroke or seizures in SLE is not necessarily any different from that in the general population, unless complicated by the antiphospholipid syndrome. However, less common neurological syndromes may demand more specific investigation and treatment. For example, longitudinally extensive transverse myelitis and recurrent optic neuritis (neuromyelitis optica, or Devic’s disease) is frequently associated with antibodies to aquaporin-4, and is highly likely to relapse unless treated vigorously with humoral immunosuppression. Nervous system involvement in vasculitis is common. Finally, not all neurological disorder in rheumatological disease is necessarily due to the underlying condition; neurological complications of disease-modifying therapy are increasingly recognized, in particular central and peripheral nervous system demyelination associated with TNF-α‎‎ inhibitors.

scholarly journals Early diagnosis of peripheral nervous system involvement in Fabry disease and treatment of neuropathic pain: the report of an expert panel

BMC Neurology ◽  
2011 ◽  
Vol 11 (1) ◽  
Author(s):  
Alessandro P Burlina ◽  
Katherine B Sims ◽  
Juan M Politei ◽  
Gary J Bennett ◽  
Ralf Baron ◽  
...  
Keyword(s):  
Nervous System ◽  
Neuropathic Pain ◽  
Early Diagnosis ◽  
Fabry Disease ◽  
Peripheral Nervous System ◽  
Expert Panel ◽  
System Involvement

Inhibition of the Renin-Angiotensin-Aldosterone System by l-Dopa with and without Inhibition of Extracerebral Dopa Decarboxylase in Man

1981 ◽  
Vol 61 (2) ◽  
pp. 187-190 ◽  
Author(s):  
C. Barbieri ◽  
R. Caldara ◽  
C. Ferrari ◽  
Rosa Maria Crossignani ◽  
M. Recchia
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Plasma Renin ◽  
Normal Subjects ◽  
Plasma Aldosterone ◽  
Renin Angiotensin Aldosterone System ◽  
Plasma Aldosterone Concentration ◽  
Renin Angiotensin ◽  
Drugs Analysis ◽  
Catecholamine Levels

1. The present study was undertaken to investigate the possibility that central nervous system mono-aminergic pathways may play a role in the control of the renin-angiotensin-aldosterone system in man. 2. Eight normal subjects received in a randomized order placebo, l-dopa (500 mg, orally) and l-dopa (100 mg, orally) plus carbidopa (35 mg, orally) after pretreatment with carbidopa (50 mg every 6 h for four doses). 3. l-Dopa administration elicited a significant fall in plasma renin activity (PRA) (P < 0.01 at 120, 150 and 180 min) and in plasma aldosterone levels (P < 0.05 at 90, 120, 150 and 180 min); L-dopa plus carbidopa induced a decrease in PRA (P < 0.05 at 120 and 150 min, P < 0.01 at 180 min) and in plasma aldosterone concentration (P < 0.05 at 30 and 60 min, P < 0.01 at 90 and 120 min), in comparison with placebo administration; between-drugs analysis revealed no difference in the decreases in PRA and plasma aldosterone levels induced by the two regimens. 4. Since l-dopa, as well as l-dopa plus carbidopa, has been shown to augment catecholamine levels in the brain of various animal species, the present data suggest that in man PRA and plasma aldosterone concentration might be inhibited by increased central nervous system catecholamine levels.

scholarly journals Role of the Renin-Angiotensin-Aldosterone System in Various Disease Processes: An Overview

2021 ◽  
Author(s):  
Volkan Gelen ◽  
Abdulsamed Kükürt ◽  
Emin Şengül
Keyword(s):  
Blood Pressure ◽  
Nervous System ◽  
Electrolyte Balance ◽  
Physiological System ◽  
Renin Angiotensin Aldosterone System ◽  
Renin Angiotensin ◽  
Working Principle ◽  
Kidney Liver ◽  
Regulation Of Blood Pressure

The renin-angiotensin-aldosterone system is a physiological system that plays an important role in the regulation of blood pressure and body water-electrolyte balance, in which the kidney, liver and lungs play a role in its activation. This system comes into play in various diseases such as the cardiovascular, renal, pulmonary and nervous system where blood pressure and fluid-electrolyte balance may change. The purpose of this study, which is presented in line with this information, is to explain the working principle of this system, how this system is activated, how it comes into play in the mentioned diseases, and what kind of results occur.

Nervous system

2013 ◽  
Author(s):  
Andrew Graham ◽  
Clare Galton
Keyword(s):  
Nervous System ◽  
Peripheral Nervous System ◽  
Lupus Erythematosus ◽  
Transverse Myelitis ◽  
Neurological Complications ◽  
Neurological Involvement ◽  
Disease Modifying Therapy ◽  
Entrapment Neuropathies ◽  
System P ◽  
System Disorder

Rheumatological conditions may be complicated by a variety of both central and peripheral nervous system disorder. Common complications such as entrapment neuropathies are familiar to rheumatologists but accurate diagnosis of less common neurological disorders may be challenging; careful clinical reasoning is essential, supplemented where necessary by imaging, neurophysiology, and other special investigations including cerebrospinal fluid examination. Complications vary according to the nature of background condition. In rheumatoid arthritis, neurological involvement is typically related to the mechanical consequences of advancing disease; the commonest complications are carpal tunnel syndrome and cervical myelopathy due to atlantoaxial subluxation. By contrast, neurological involvement in systemic lupus erythematosus (SLE) tends to occur earlier in the disease course, with a much wider range of manifestations. The management of stroke or seizures in SLE is not necessarily any different from that in the general population, unless complicated by the antiphospholipid syndrome. However, less common neurological syndromes may demand more specific investigation and treatment. For example, longitudinally extensive transverse myelitis and recurrent optic neuritis (neuromyelitis optica, or Devic's disease) is frequently associated with antibodies to aquaporin-4, and is highly likely to relapse unless treated vigorously with humoral immunosuppression. Nervous system involvement in vasculitis is common. Finally, not all neurological disorder in rheumatological disease is necessarily due to the underlying condition; neurological complications of disease-modifying therapy are increasingly recognized, in particular central and peripheral nervous system demyelination associated with TNF-α‎ inhibitors.

Peripheral Nervous SystemAutonomic Nervous System

2013 ◽  
Author(s):  
Adam Fisch
Keyword(s):  
Nervous System ◽  
Autonomic Nervous System ◽  
Sympathetic Nervous System ◽  
Peripheral Nervous System ◽  
Parasympathetic Nervous System ◽  
Urinary System ◽  
Autonomic Nervous ◽  
System P ◽  
Sympathetic Nervous

Chapter 6 discusses how to draw the peripheral nervous system, specifically the autonomic nervous system, including autonomic fiber arrangements, the parasympathetic nervous system, the sympathetic nervous system, the urinary system, and the cardiac reflex.

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