atrial-natriuretic-factor and Syndrome

The natriuretic peptide system (atrial natriuretic peptide, brain natriuretic peptide, BNP, and C natriuretic peptide) is an important marker of cardiac failure. These peptides are synthesized in atrial or ventricular myocytes in response to wall tension. In several studies the correlation between high BNP levels and mortality, in patients with acute coronary syndrome and heart failure, has been demonstrated. On the other hand, plasma levels of BNP could be considered as independent predictors of mortality in patients with heart failure. BNP could be used, for instance, as an early diagnostic marker for the differential diagnosis between cardiogenic and non cardiogenic dyspnea. In the Emergency Department its use will be important in the diagnosis of thoracic pain origin since it may help in the diagnostic and therapeutic course of this patient and to define the modality of hospitalization. Moreover, it can be used as a marker of heart failure severity and as an important negative prognostic factor. Some studies have confirmed that plasma BNP reflects the degree of left ventricular dysfunction and the prognostic significance after acute myocardial infarction and chronic heart failure.

Topics: Angina, Unstable; Atrial Natriuretic Factor; Biomarkers; Diagnosis, Differential; Humans; Myocardial Infarction; Natriuretic Peptide, Brain; Prognosis; Protein Precursors; Syndrome; Ventricular Dysfunction, Left

Topics: Adrenergic beta-Antagonists; Aged; Aged, 80 and over; Angina, Unstable; Angiotensin-Converting Enzyme Inhibitors; Atrial Natriuretic Factor; Diagnosis, Differential; Electrocardiography; Female; Heart Failure; Humans; Male; Myocardial Infarction; Natriuretic Peptide, Brain; Prognosis; Randomized Controlled Trials as Topic; Sensitivity and Specificity; Syndrome; Ventricular Dysfunction, Left

B-type natriuretic peptide (BNP) and the N-terminal fragment of its prohormone (N-proBNP) are released from the heart in response to increased wall stress. Assays for these peptides are now commercially available, and measurement of BNP and N-proBNP is becoming commonplace in patients with suspected heart failure. BNP and N-proBNP facilitate diagnosis and risk stratification in patients with heart failure, and may help guide response to therapy. This review focuses on the emerging role of BNP and N-proBNP measurement in patients with acute coronary syndromes (ACS). Although experimental studies demonstrate rapid BNP release in response to cardiac ischemia, it is unlikely that BNP will be used to diagnose cardiac ischemia, because many other conditions are also associated with modest BNP elevation. In contrast, BNP holds tremendous promise as a prognostic marker in patients with ACS. Studies to date have shown consistently that higher BNP levels are associated with worse clinical outcomes, and that BNP provides unique information to clinical variables, other biomarkers, and left ventricular ejection fraction. Future studies are needed to identify the therapeutic implications of BNP elevation in patients with ACS.

Topics: Acute Disease; Angina, Unstable; Atrial Natriuretic Factor; Cardiotonic Agents; Humans; Myocardial Infarction; Myocardial Ischemia; Natriuretic Peptide, Brain; Protein Precursors; Syndrome

Essential hypertension probably results from combinations of small genetic variations that are partly normal variations and may not be appreciably harmful individually. Strategies to identify genes contributing to hypertension are discussed in this review. Gene targeting approaches, especially gene titration, have been used in these studies of hypertension. Gene titration experiments vary the expression of a chosen gene product by generating animals having different numbers of copies of the gene coding for the product. Gene titration is powerful for analyzing quantitative variations seen in common polygenic disorders, such as kidney diseases, diabetes mellitus, and atherosclerosis, as well as hypertension, because it allows tests of causation by determining the effects on a phenotype by changes in expression of the altered gene and because it matches normal quantitative variations more closely than is possible with classic transgenic mice. The use of zero-copy (gene "knockout") animals generated by gene disruption for studies of qualitative gene effects is also discussed. These various gene targeting experiments help identify genes regulating BP, promote a better understanding of the pathophysiology of the condition, and help identify potential targets for therapies.

Topics: Animals; Atrial Natriuretic Factor; Blood Pressure; Endothelin-1; Gene Targeting; Humans; Hyperaldosteronism; Hypertension; Mice; Mice, Knockout; Models, Genetic; Mutation; Nitric Oxide Synthase; Receptor, Bradykinin B2; Receptors, Bradykinin; Renin-Angiotensin System; Syndrome

Topics: Atrial Natriuretic Factor; Brain Injuries; Child; Diagnosis, Differential; Female; Fluid Therapy; Hemodynamics; Humans; Hyponatremia; Inappropriate ADH Syndrome; Infant; Male; Syndrome

Although it has been recognized for almost 70 years that there is a substantial genetic component to the pathogenesis of hypertension, only recently have systematic efforts been made to identify the responsible genetically determined mechanisms. In the case of several rare syndromes, spectacular progress has been made in identifying the underlying molecular mechanisms responsible for the clinical expression. Glucocorticoid-suppressible aldosteronism and Liddle's syndrome, each inherited as an autosomal-dominant condition, complete the list. In the case of randomly selected patients and families with essential hypertension, inheritance involves many genes and progress has been far more modest. Probably the most promising lead has involved the genes governing the structure of angiotensinogen, the substrate in the renin reaction. Linkage has been established and confirmed. At the moment, however, neither the relation of the genetic abnormality to the underlying mechanisms, nor the contribution of this abnormality to hypertension in the individual patient, has been defined. We know less about other candidate genes, with the exception of studies that rigorously ruled out a contribution. The development of the concept of the "intermediate phenotype," a physiological feature that makes it possible to identify a homogeneous subpopulation, should help to sort out many of these issues. Unfortunately, the identification and characterization of intermediate phenotypes is substantially more difficult at the moment than are the genetic studies, and so progress is likely to be slow. The field is complicated by the reporting of claims made on the basis of small patient samples. In the case of polymorphisms in the angiotensin-converting enzyme gene as a risk factor for tissue injury, for example, substantial follow-up studies have systematically failed to confirm the original report, which was based on a small patient sample. The fact that the same DNA collection is likely to be examined many times for multiple gene candidates creates a setting in which type I errors are likely, and so we are likely to see many more examples. Caveat lector. Again, the development of relevant intermediate phenotypes will make the spurious association less likely.

Topics: Angiotensinogen; Atrial Natriuretic Factor; Genes, Dominant; Humans; Hyperaldosteronism; Hypertension; Nitric Oxide Synthase; Peptidyl-Dipeptidase A; Phenotype; Polymorphism, Genetic; Racial Groups; Renin-Angiotensin System; Syndrome

The atria, strategically located at the junction of the venous and arterial circulation, contain a network of neural and humoral structures by which they sense and regulate intravascular volume. Atrial receptors, most commonly consisting of complex unencapsulated nerve endings discharging into myelinated vagal fibers, are located in the intrapericardial portions of the caval and pulmonary veins and the adjacent atrial walls. Receptor activation by atrial distension results in increased afferent vagal fiber discharge, which in turn leads to tachycardia (Bainbridge's reflex) and decreased renal sympathetic nerve activity, renal vasomotor tone, and antidiuretic hormone activity. In addition, atrial distension also releases ANF, a peptide with potent diuretic, natriuretic, and vasorelaxant actions. The combined effect of these neurohumoral changes is the production of a large hypotonic diuresis. In the clinical setting the volume-regulating role of the atria is demonstrated by the tachycardia-polyuria syndrome. Laboratory and clinical evidence points to the activation of atrial neurohumoral mechanisms in response to atrial distension as the mediators of the polyuria that often accompanies paroxysmal tachycardias. The involvement of these mechanisms in other forms of cardiac congestion and the capability to easily measure in the blood an index of atrial distension, namely ANF, provide the opportunity to elucidate the pathophysiology and hence to open new therapeutic avenues in many cardiac disorders.

Topics: Animals; Atrial Natriuretic Factor; Blood Volume; Heart Atria; Humans; Polyuria; Receptors, Atrial Natriuretic Factor; Receptors, Cell Surface; Syndrome; Tachycardia

While recent guidelines for the treatment of acute heart failure syndromes (AHFS) recommend pharmacotherapy with vasodilators in patients without excessively low blood pressure (BP), few reports have compared the relative efficiency of vasodilators on hemodynamics in AHFS patients. The present study aimed to assess the differences in hemodynamic responses between intravenous carperitide and nicorandil in patients with AHFS. Thirty-eight consecutive patients were assigned to receive 48-h continuous infusion of carperitide (n = 19; 0.0125-0.05 μg/kg/min) or nicorandil (n = 19; 0.05-0.2 mg/kg/h). Hemodynamic parameters were estimated at baseline, and 2, 24, and 48 h after drug administration using echocardiography. After 48 h of infusion, systolic BP was significantly more decreased in the carperitide group compared with that in the nicorandil group (22.1 ± 20.0 % vs 5.3 ± 10.4 %, P = 0.003). While both carperitide and nicorandil significantly improved hemodynamic parameters, improvement of estimated pulmonary capillary wedge pressure was greater in the carperitide group (38.2 ± 14.5 % vs 26.5 ± 18.3 %, P = 0.036), and improvement of estimated cardiac output was superior in the nicorandil group (52.1 ± 33.5 % vs 11.4 ± 36.9 %, P = 0.001). Urine output for 48 h was greater in the carperitide group, but not to a statistically significant degree (4203 ± 1542 vs 3627 ± 1074 ml, P = 0.189). Carperitide and nicorandil were differentially effective in improving hemodynamics in AHFS patients. This knowledge may enable physicians in emergency wards to treat and manage patients with AHFS more effectively and safely.

Topics: Aged; Aged, 80 and over; Atrial Natriuretic Factor; Chi-Square Distribution; Drug Administration Schedule; Female; Heart Failure; Hemodynamics; Humans; Infusions, Intravenous; Japan; Male; Middle Aged; Nicorandil; Syndrome; Time Factors; Treatment Outcome; Vasodilator Agents

A self controlled clinical trial was carried out to study the acute and chronic effects of ventricular pacing (VVI) on the atrial natriuretic factor (ANF). Eleven people were selected from a pool of 20 DDD paced patients. Pacemakers were programmed to the VVI mode for 1 month and their effectiveness tested by ECG at rest and after an effort test. AnF was measured by radioimmunoassay at baseline, after 15 minutes, and again 1 month after programming. The reliability of the radioimmunoassay was confirmed using the coefficients of variation between (12.5%) and within assay (9.7%). Data analysis was done using Wilcoxon's test. Our results showed that the onset of VVI pacing led to a sudden sharp rise in ANF in all patients (P < 0.0001). During VVI pacing, three patients were dropped from the study (2 were withdrawn because of symptoms and 1 voluntarily withdrew). After 1 month of VVI pacing, a significant increase of ANF above the baseline was observed (P < 0.05). The results showed that ventricular pacing led to an immediate rise in ANF and, that with long-term VVI pacing, there was an increase in ANF levels as well. The role of these findings in the pathophysiology of the pacemaker syndrome calls for further research.

Topics: Adult; Aged; Atrial Natriuretic Factor; Cardiac Pacing, Artificial; Electrocardiography; Exercise Test; Female; Follow-Up Studies; Humans; Longitudinal Studies; Male; Middle Aged; Pacemaker, Artificial; Patient Dropouts; Physical Exertion; Reproducibility of Results; Rest; Syndrome; Ventricular Function

To demonstrate the presence of endothelin-1 (ET-1), atrial natriuretic peptide (ANP) in peritoneal fluid of women and their effects on pathogenesis of pelvic venous congestion syndrome after sterilization (PVCSS).. This randomized controlled study determined the concentrations of ET-1 and ANP in both peritoneal fluid and plasma, counts of macrophage in peritoneal fluid and volumes of peritoneal fluid in 21 cases of PVCSS. 12 normal women after sterilization and 11 normal women as control in early follicular phase by radioimmunoassay.. concentrations of ET-1, ratio of ET-1/ANP and counts of macrophage in peritoneal fluid with PVCSS were lower than those in control (P < 0.005, P < 0.001, P < 0.001) and all had significant negative correlation with scores quantifying the severity of PVCSS (P < 0.05), but volumes of peritoneal fluid in PVCSS were larger than that in control (P < 0.001); counts of macrophage in peritoneal fluid had significant positive correlation with the concentrations of ET-1 of peritoneal fluid in all the three groups (P < 0.05); plasma concentrations of ET-1 and ANP didn't show any significant differences among the three groups (P > 0.05).. ET-1 was present in peritoneal fluid of normal women. Lower concentrations of ET-1 and (or) lower ratio of ET-1/ANP in peritoneal fluid contributed to the pathogenesis of PVCSS.

Topics: Adult; Ascitic Fluid; Atrial Natriuretic Factor; Endothelin-1; Female; Humans; Pelvic Pain; Pelvis; Sterilization, Tubal; Syndrome

Hypertension occurred in 50% obstructive sleep apnea-hypopnea syndrome (OSAHS) patients meanwhile OSAHS occurred in 30% hypertension patients. The present study aimed to explore the molecular mechanism of GATA2-EDN1-AGT induced hypertension in the development of obstructive sleep apnea-hypopnea syndrome. OSAHS patients (56 cases: 36 cases of male, 20 cases of female, 42~60 years old) were divided into two groups (case group: patients with hypertension monitored by 24 h ambulatory blood pressure and polysomnography; control group: patients without hypertension). Wistar rats were used to establish the OSAHS model (narrow pharyngeal cavity). PaO2 and PaCO2 of patients and rats were measured by an automatic blood gas analyzer. The profile of total protein in the OSAHS group and normal group was evaluated. Protein-protein-interaction (PPI) was carried out to show all matter proteins related. The levels of EDN-1, AGTII and atrial natriuretic peptide (ANP) in blood samples of patients and rats were analyzed by enzyme-linked immunosorbent assay (ELISA). The expression of GATA2, EDN1, endothelin-converting enzyme 1 (ECE-1) and AGTⅡ was measured. The results showed that SaO2 and AHI were positively associated with systolic pressure (P<0.05) in OSAHS patients. There was no correlation among other indexes (P>0.05). It was also observed that GATA2 had a strong relationship with AGTⅡ and EDN1. The results of ELISA presented that the levels of EDN1, AGTⅡ and ANP in the OSAHS group of human and animal models were significantly increased (P<0.05). The results of immunochemistry showed that the expression of GATA2 and AGTⅡ in the vascular of OSAHS group was upregulated manifestly (P<0.05). It was concluded that OSAHS can induce AHI, which increases hypertension via the GATA2-EDN1-AGT Ⅱ axis.

Topics: Angiotensinogen; Animals; Atrial Natriuretic Factor; Blood Pressure Monitoring, Ambulatory; Endothelin-1; Female; GATA2 Transcription Factor; Hypertension; Male; Rats; Rats, Wistar; Sleep Apnea, Obstructive; Syndrome

Cytokine release syndrome (CRS) is a life-threatening complication of several new immunotherapies used to treat cancers and autoimmune diseases

Topics: alpha-Methyltyrosine; Animals; Atrial Natriuretic Factor; Catecholamines; CD3 Complex; Cytokines; Epinephrine; Female; Humans; Immunotherapy, Adoptive; In Vitro Techniques; Kaplan-Meier Estimate; Male; Mice; Mice, Inbred BALB C; Mice, Inbred C57BL; Myeloid Cells; Norepinephrine; Receptors, Antigen, T-Cell; Syndrome; T-Lymphocytes

Timely diagnosis, early introduction of appropriate treatment, accurate risk stratification, and optimal titration of therapy are all key to the management of acute and chronic heart failure (HF). Plasma concentrations of the cardiac natriuretic peptides (NPs) are valuable aids in each of these elements of care. However, most data are derived from cohorts with undifferentiated HF or HF with reduced ejection fraction (HFREF), and the performance and best application of NPs in HF with preserved ejection fraction (HFPEF) is less certain. This review outlines the evidence for use of NPs in the evaluation and management of HFPEF.

Topics: Acute Disease; Aged; Atrial Natriuretic Factor; Biomarkers; Cardiac Output, Low; Case-Control Studies; Chronic Disease; Female; Heart Failure; Humans; Male; Middle Aged; Prognosis; Reference Values; Sensitivity and Specificity; Stroke Volume; Syndrome

The role of biomarkers is rapidly emerging as an important tool in the management of the cardiorenal syndromes (CRS). Natriuretic peptides (NPs), due to their low cost and rapid and accurate ability to provide additional information not surmised from clinical evaluation, are the standard bearer for the newer biomarkers. Although the NP-guided therapy has been shown to improve patient outcomes, this has yet to be demonstrated for the novel renal biomarkers. Most of the renal biomarkers studies in CRS have been performed in the setting of cardiac surgery. It will be critical to validate these new biomarkers in multicenter and prospective studies encompassing a broad spectrum of patients. Work with NPs has also shown that novel biomakers are not to be used as 'stand-alone' tests; rather they are best used as adjuncts to everything else the health care provider brings to the table. It is likely that panels of multiple biomarkers will be needed for optimal evaluation, risk stratification, timely treatment initiation and follow-up of patients with CRS.

Topics: Atrial Natriuretic Factor; Biomarkers; Blood Pressure; Gelatinases; Glomerular Filtration Rate; Heart Failure; Humans; Hypertension; Lipocalins; Natriuretic Peptides; Neutrophils; Prognosis; Renal Insufficiency; Syndrome

The cardiorenal syndromes (CRS) are composed of five recently defined syndromes which represent common clinical scenarios in which both the heart and the kidney are involved in a bidirectional injury process leading to dysfunction of both organs. Common to each subtype are multiple complex pathogenic factors, a precipitous decline in function and a progressive course. Most pathways that lead to CRS involve acute injury to organs which manifest evidence of chronic disease, suggesting reduced ability to sustain damage, maintain vital functions, and facilitate recovery. Prevention of CRS is an ideal clinical goal, because once initiated, CRS cannot be readily aborted, are not completely reversible, and are associated with serious consequences including hospitalization, complicated procedures, need for renal replacement therapy, and death. Principles of prevention include identification and amelioration of precipitating factors, optimal management of both chronic heart and kidney diseases, and future use of multimodality therapies for end-organ protection at the time of systemic injury. This paper will review the core concepts of prevention of CRS with practical applications to be considered in today's practice.

Topics: Anemia; Atrial Natriuretic Factor; Cardiotonic Agents; Dopamine; Heart Failure; Humans; Inflammation; Infusions, Intravenous; Kidney Diseases; Kidney Failure, Chronic; Myocardial Ischemia; Randomized Controlled Trials as Topic; Renal Circulation; Sleep Apnea, Obstructive; Sodium; Sodium Potassium Chloride Symporter Inhibitors; Syndrome

A 49-year-old man had sudden chest pain and paralysis of the lower right limb. An acute aortic dissection was diagnosed in a computed tomography scan and the patient underwent an emergency operation. After the operation, myonephropathic metabolic syndrome developed, and human atrial natriuretic peptide was administered for 11 days until the volume of daily urine output reached at least 10,000 mL, which would facilitate limb salvage and the preservation of life without hemodialysis. This report documents that postoperative myonephropathic metabolic syndrome improved due to the strong diuretic action of human atrial natriuretic peptide without hemodialysis.

Topics: Acute Disease; Aortic Aneurysm, Thoracic; Aortic Dissection; Atrial Natriuretic Factor; Blood Vessel Prosthesis Implantation; Dose-Response Relationship, Drug; Follow-Up Studies; Humans; Infusions, Intravenous; Male; Metabolic Diseases; Middle Aged; Postoperative Complications; Radiography; Rhabdomyolysis; Syndrome

Mutations in SALL1 lead to the dominant multiorgan congenital anomalies that define Townes-Brocks syndrome (TBS). The majority of these mutations result in premature termination codons that would be predicted to trigger nonsense-mediated decay (NMD) of mutant mRNA and cause haploinsufficiency. Our previous studies using a gene targeted mouse model (Sall1-DeltaZn) suggested that TBS phenotypes are due to expression of a truncated mutant protein, not haploinsufficiency. In this report, we strengthen this hypothesis by showing that expression of the mutant protein alone in transgenic mice is sufficient to cause limb phenotypes that are characteristic of TBS patients. We prove that the same pathogenetic mechanism elucidated in mice is occurring in humans by demonstrating that truncated SALL1 protein is expressed in cells derived from a TBS patient. TBS mutant protein is capable of dominant negative activity that results in ectopic activation of two downstream genes, Nppa and Shox2, in the developing heart and limb. We propose a model for the pathogenesis of TBS in which truncated Sall1 protein causes derepression of Sall-responsive target genes.

Topics: Abnormalities, Multiple; Animals; Atrial Natriuretic Factor; Codon, Nonsense; Heart Defects, Congenital; Humans; Limb Deformities, Congenital; Mice; Mice, Transgenic; Mutation; Natriuretic Peptide, C-Type; Phenotype; Protein Precursors; RNA Stability; Syndrome; Transcription Factors; Transcriptional Activation

Symptoms of hyponatremia and diuresis due to cerebral salt wasting syndrome (CSWS) are often observed after aneurysmal subarachnoid hemorrhage (SAH). Inadequately treated CSWS is known to work as a trigger of symptomatic vasospasm in SAH patients. Therefore, it is indispensable to detect and treat CSWS as early as possible in ICU. A 36-year-old man with SAH was admitted to our ICU. His urine volume increased excessively 3 days after ICU admission, and it reached a peak (39,250 ml x day(-1)) on the 6th day in ICU. Since infusion volume was controlled with regard to daily urinary output, hyponatremia was not noticeable and excessive urine volume stood out conspicuously. Though vasopressin and desmopressin were administered, the symptoms of natriuresis and hyponatremia were aggravated, associated with hyper secretion of natriuretic peptides (ANP 160 pg x dl(-1), BNP 172 pg x dl(-1)). Recent studies revealed that hyponatremia and hypovolemia following SAH might be caused by exaggerated secretion of natriuretic peptides. Experimental studies showed that the administration of vasopressin and desmopressin cause excessive secretion of natriuretic peptides under the circumstance of volume expansion in rats. We infer that the administration of vasopressin and desmopressin to our patient deterionated natriuresis in CSWS as in the previous experimental findings.

Topics: Adult; Animals; Atrial Natriuretic Factor; Brain Diseases; Contraindications; Humans; Hyponatremia; Hypovolemia; Male; Natriuresis; Rats; Subarachnoid Hemorrhage; Syndrome; Urination Disorders; Vasopressins

Severe idiopathic tricuspid regurgitation (TR) occurs in the aged, but the mechanism of TR is unclear and there is little information on atrial abnormalities associated with this condition. This study retrospectively analyzed patients with severe functional TR presenting with common clinical features suggesting a distinct syndrome.. Eleven patients with severe functional TR were identified by reviewing the records of 16,235 consecutive patients. All patients had undergone clinical evaluation including echocardiography, electrocardiography and laboratory data.. The median age of patients with severe functional TR was 78 years. All had a long-standing history of atrial fibrillation (median duration, 23 years). Clinical features are characterized by severe functional TR due to annular dilation, markedly dilated right atrium, episodes of right-sided heart failure, absent or diminished fibrillation waves on electrocardiogram, bradycardia probably due to partial atrial standstill, and decreased atrial natriuretic peptide secretion. During long-term follow up, right atrial size progressively increased in association with worsening TR.. Severe functional TR occurs with long-standing atrial fibrillation and causes right-sided heart failure. The TR is caused by tricuspid valve systolic coaptation loss due to tricuspid annular dilation associated with atrial dilation. This condition is associated with atrial abnormalities, such as atrial standstill and impaired atrial natriuretic peptide secretion. We propose that atrial remodeling associated with atrial fibrillation is central to the occurrence of the syndrome.

Topics: Aged; Aged, 80 and over; Atrial Fibrillation; Atrial Natriuretic Factor; Female; Follow-Up Studies; Heart Atria; Heart Failure; Humans; Male; Retrospective Studies; Severity of Illness Index; Syndrome; Tricuspid Valve Insufficiency

Topics: 3T3 Cells; Abnormalities, Multiple; Active Transport, Cell Nucleus; Animals; Atrial Natriuretic Factor; Base Sequence; Cell Line; Cell Nucleus; Codon, Nonsense; DNA; DNA Mutational Analysis; Family Health; Female; Heart Defects, Congenital; Humans; Limb Deformities, Congenital; Luciferases; Male; Mice; Molecular Sequence Data; Mutation; Pedigree; Promoter Regions, Genetic; Protein Binding; Recombinant Fusion Proteins; Sequence Deletion; Syndrome; T-Box Domain Proteins

Topics: Acute Disease; Atrial Natriuretic Factor; Biomarkers; C-Reactive Protein; Coronary Disease; Creatinine; Glycated Hemoglobin; Humans; Inflammation; Kidney Diseases; Natriuretic Peptide, Brain; Predictive Value of Tests; Prognosis; Risk Assessment; Risk Factors; Syndrome

Topics: Aged; Angina, Unstable; Atrial Natriuretic Factor; Case-Control Studies; Female; Humans; Male; Myocardial Infarction; Natriuresis; Natriuretic Peptide, Brain; Peptide Fragments; Prognosis; Protein Precursors; Randomized Controlled Trials as Topic; Syndrome

Most patients (about 85%) seen in the ED to rule out an acute coronary event do not have acute coronary disease. In addition, the presenting ECG findings have been nondiagnostic in 50% of patients with acute MI. Our current knowledge of atherosclerosis as being a chronic low-grade inflammatory process triggered the search for reliable serum markers that have improved the diagnostic accuracy management and prognosis of this prevalent disease. Newer and potential inflammatory markers currently under investigation deserve watching in future reports. These among others include those markers produced by the arterial wall itself, that is, cell adhesion molecules (CAM), inter-cellular adhesion molecules (ICAM), and vascular adhesion molecules (VCAM). The expression of CAM is a marker of dysfunctional endothelial cells. It is likely that more cardiac markers will be reported in the future. Time will tell.

Topics: Acute Disease; Atrial Natriuretic Factor; Biomarkers; Coronary Disease; Female; Humans; Male; Natriuretic Peptide, Brain; Pregnancy-Associated Plasma Protein-A; Syndrome

In patients with acute coronary syndromes (ACS), troponin I (TnI), C-reactive protein (CRP), and B-type natriuretic peptide (BNP) each predict adverse cardiac events. Little is known, however, about the utility of these biomarkers in combination.. Baseline measurements of TnI, CRP, and BNP were performed in 450 patients in OPUS-TIMI 16. Elevations in TnI, CRP, and BNP each were independent predictors of the composite of death, myocardial infarction (MI), or congestive heart failure (CHF). When patients were categorized on the basis of the number of elevated biomarkers at presentation, there was a near doubling of the mortality risk for each additional biomarker that was elevated (P=0.01). Similar relationships existed for the endpoints of MI, CHF, and the composite, both at 30 days and through 10 months. In a validation cohort of 1635 patients in TACTICS-TIMI 18, the number of elevated biomarkers remained a significant predictor of the composite endpoint after adjustment for known clinical predictors: patients with one, two, and three elevated biomarkers had a 2.1- (P=0.006), 3.1- (P<0.001), and 3.7- (P=0.001) fold increase in the risk of death, MI, or CHF by 6 months.. Troponin, CRP, and BNP each provide unique prognostic information in patients with ACS. A simple multimarker strategy that categorizes patients based on the number of elevated biomarkers at presentation allows risk stratification over a broad range of short- and long-term major cardiac events.

Topics: Acute Disease; Atrial Natriuretic Factor; Biomarkers; C-Reactive Protein; Cohort Studies; Coronary Disease; Heart Failure; Humans; Multivariate Analysis; Myocardial Infarction; Natriuretic Peptide, Brain; Prognosis; Risk Assessment; Syndrome; Troponin I

The cardiac homeobox protein Nkx2-5 is essential in cardiac development, and mutations in Csx (which encodes Nkx2-5) cause various congenital heart diseases. Using the yeast two-hybrid system with Nkx2-5 as the 'bait', we isolated the T-box-containing transcription factor Tbx5; mutations in TBX5 cause heart and limb malformations in Holt-Oram syndrome (HOS). Co-transfection of Nkx2-5 and Tbx5 into COS-7 cells showed that they also associate with each other in mammalian cells. Glutathione S-transferase (GST) 'pull-down' assays indicated that the N-terminal domain and N-terminal part of the T-box of Tbx5 and the homeodomain of Nkx2-5 were necessary for their interaction. Tbx5 and Nkx2-5 directly bound to the promoter of the gene for cardiac-specific natriuretic peptide precursor type A (Nppa) in tandem, and both transcription factors showed synergistic activation. Deletion analysis showed that both the N-terminal domain and T-box of Tbx5 were important for this transactivation. A G80R mutation of Tbx5, which causes substantial cardiac defects with minor skeletal abnormalities in HOS, did not activate Nppa or show synergistic activation, whereas R237Q, which causes upper-limb malformations without cardiac abnormalities, activated the Nppa promoter to a similar extent to that of wildtype Tbx5. P19CL6 cell lines overexpressing wildtype Tbx5 started to beat earlier and expressed cardiac-specific genes more abundantly than did parental P19CL6 cells, whereas cell lines expressing the G80R mutant did not differentiate into beating cardiomyocytes. These results indicate that two different types of cardiac transcription factors synergistically induce cardiac development.

Topics: Atrial Natriuretic Factor; Binding Sites; Cell Differentiation; Cell Line; Genes, Reporter; Heart Defects, Congenital; Homeobox Protein Nkx-2.5; Homeodomain Proteins; Humans; Limb Deformities, Congenital; Mutation; Myocardial Contraction; Myocardium; Natriuretic Peptide, C-Type; Promoter Regions, Genetic; Protein Binding; Protein Precursors; Syndrome; T-Box Domain Proteins; Transcription Factors; Transcriptional Activation; Xenopus Proteins

Holt-Oram syndrome is caused by mutations in TBX5, a member of the T-box gene family. In order to identify DNA sequences to which the TBX5 protein binds, we have performed an in vitro binding site selection assay. We have identified an 8 bp core sequence that is part of the Brachyury consensus-binding site. We show that TBX5 binds to the full palindromic Brachyury binding site and to the half-palindrome, whereas Brachyury does not bind to the TBX5 site. Amino acids 1-237 of TBX5 are required for DNA binding. Analysis of the effects of specific substitution mutations that arise in Holt-Oram patients indicates that G80R and R237Q eliminate binding to the target site. DNA database analysis reveals that target sites are present in the upstream regions of several cardiac-expressed genes including cardiac alpha actin, atrial natriuretic factor, cardiac myosin heavy chain alpha, cardiac myosin heavy chain beta, myosin light chain 1A, myosin light chain 1V and Nkx2.5. Cell transfection studies demonstrate that TBX5 activates the transcription of an atrial natriuretic factor reporter construct and this effect is significantly reduced by deletion of the TBX5 binding site.

Topics: Abnormalities, Multiple; Animals; Atrial Natriuretic Factor; Base Sequence; Binding Sites; Binding, Competitive; Cell Line; COS Cells; Fetal Proteins; Gene Expression Regulation; Genes, Reporter; Heart Defects, Congenital; Heart Ventricles; In Situ Hybridization; Limb Deformities, Congenital; Mice; Molecular Sequence Data; Mutation; Mutation, Missense; Myocardium; Oligonucleotides; Plasmids; Promoter Regions, Genetic; Sequence Homology, Nucleic Acid; Syndrome; T-Box Domain Proteins

Heterozygous Tbx5(del/+) mice were generated to study the mechanisms by which TBX5 haploinsufficiency causes cardiac and forelimb abnormalities seen in Holt-Oram syndrome. Tbx5 deficiency in homozygous mice (Tbx5(del/del)) decreased expression of multiple genes and caused severe hypoplasia of posterior domains in the developing heart. Surprisingly, Tbx5 haploinsufficiency also markedly decreased atrial natriuretic factor (ANF) and connexin 40 (cx40) transcription, implicating these as Tbx5 target genes and providing a mechanism by which 50% reduction of T-box transcription factors cause disease. Direct and cooperative transactivation of the ANF and cx40 promoters by Tbx5 and the homeodomain transcription factor Nkx2-5 was also demonstrated. These studies provide one potential explanation for Holt-Oram syndrome conduction system defects, suggest mechanisms for intrafamilial phenotypic variability, and account for related cardiac malformations caused by other transcription factor mutations.

Topics: Abnormalities, Multiple; Aging; Animals; Atrial Natriuretic Factor; Base Sequence; Binding Sites; Bone Development; Cell Differentiation; Connexins; Disease Models, Animal; Electrocardiography; Embryonic and Fetal Development; Forelimb; Gap Junction alpha-5 Protein; Heart; Heart Defects, Congenital; Heterozygote; Homozygote; Humans; Mice; Mice, Knockout; Molecular Sequence Data; Myocardium; Promoter Regions, Genetic; Rats; Sequence Alignment; Sequence Homology, Nucleic Acid; Sheep; Syndrome; T-Box Domain Proteins

Left atrial systolic function and the plasma of atrial natriuretic factor (ANF) and cyclic guanosine monophosphate (cGMP) were investigated as possible markers for the development of pacemaker syndrome during VVI pacing. Patients who developed pacemaker syndrome during VVI pacing had a significant decrease in left atrial emptying fraction and a substantial increase in ANF and cGMP plasma levels.

Topics: Aged; Atrial Natriuretic Factor; Cardiac Pacing, Artificial; Echocardiography; Guanosine Monophosphate; Heart Atria; Humans; Middle Aged; Syndrome

A state of normokalemic renal sodium wasting associated with an apparently inappropriate secretion of atrial natriuretic peptide (ANP) has not been previously recognized. We here report an 11-year-old boy who presented with a chronic "salt-losing" nephropathy manifested by normonatremic or mildly hyponatremic extracellular fluid volume depletion, hypodipsia, absence of salt appetite, normokalemic metabolic alkalosis, hyper-reninemic hyperaldosteronism, hypertrophy of the juxtaglomerular apparatus, and highly conserved capacities for concentrating diluting the urine. Plasma ANP values were paradoxically elevated (between 10 and 47 fmol/ml), despite the coexistence of intravascular volume depletion and increased plasma levels of renin and aldosterone. Although the patient had some clinical similarities to Bartter's syndrome, fractional sodium chloride (NaCl) reabsorption during hypotonic saline diuresis was normal and no clinical amelioration was observed while on indomethacin therapy. Neither a tumor nor cardiac or cerebral abnormalities, which could be responsible for the increased ANP secretion, were detected. These clinical, biochemical, and histological features have not been previously described together and may represent a new clinical syndrome. The pathophysiology of this entity remains unknown, but an attractive, although unproven, hypothesis is that the renal defect in NaCl reabsorption in this patient could be related to an inappropriate and unregulated secretion of ANP.

Topics: Aldosterone; Atrial Natriuretic Factor; Blood Volume; Child; Cyclooxygenase Inhibitors; Electrocardiography; Humans; Indomethacin; Kidney; Kidney Diseases; Male; Sodium; Sodium, Dietary; Syndrome; Water-Electrolyte Imbalance

The pathophysiology of preeclampsia has not been fully clarified. A variety of factors have been implicated with this disease including vasoactive peptides and hormones during the last 20 years. Inadequate generation of atrial natriuretic peptide (ANP) has been one of the mechanisms discussed as to possibly contribute to the development of hypertension. In human pregnancy multiple studies of ANP-plasma-concentration in normal or hypertensive pregnancies showed conflicting results. The complexity of the clinical findings of hypertension in pregnancy makes it very difficult to carry out comparative clinical and biochemical studies in humans. In an animal experience genetic as environmental influences could be excluded. Therefore, the present study shows an experimental preeclampsia-like syndrome in the rat by reduction of the utero-placental flow. We observed a significant increase of plasma ANP in pregnant rats with experimentally induced hypertension. Furthermore, our results suggest that the ventricles could be an important source of ANP gene expression.

Topics: Animals; Atrial Natriuretic Factor; Female; Heart Atria; Heart Ventricles; Myocardium; Pre-Eclampsia; Pregnancy; Rats; Rats, Wistar; RNA, Messenger; Syndrome

Hyponatremia and malignant hypertension are rare manifestations of Wilms' tumor. Hyponatremia associated with malignant hypertension of any cause is not explained. We present a patient with hyponatremia, malignant hypertension, Wilms' tumor, and an elevated atrial natriuretic factor (ANF). We believe the elevated ANF caused the hyponatremia.

Topics: Atrial Natriuretic Factor; Female; Humans; Hypertension, Malignant; Infant; Renin; Sodium; Syndrome; Wilms Tumor

The hormonal regulation of sodium and volume homeostasis was investigated in three patients (two related) with the syndrome of familial hyperkalemic acidosis and hypertension with normal glomerular filtration rate. Recumbent plasma renin activity was low during normal sodium intake (135 mmol daily), and the response to upright posture or to low sodium diet (10 mmol daily) was blunted. Recumbent plasma aldosterone levels were normal in two patients and high in one, and the standing values were elevated in one; responses to upright posture were brisk on low sodium diet. Angiotensin II infusion induced a marked increase in plasma aldosterone. Plasma atrial natriuretic peptide was at the upper limit of normal during normal sodium intake, decreased during diuretic therapy, and increased during sodium chloride infusion in one patient. Basal urinary prostaglandin E2, prostaglandin F2 alpha, and 6-ketoprostaglandin F1 alpha excretion rates were decreased, and thromboxane B2 was increased. Total blood and plasma volumes were subnormal, whereas extracellular fluid volume and exchangeable sodium values were close to or above (in one patient) the mean normal values. Chronic treatment with hydrochlorothiazide in two patients corrected the hyperkalemic acidosis and hypertension, but on its discontinuation (in one patient) all biochemical abnormalities promptly reappeared.

Topics: Adult; Aldosterone; Angiotensin II; Atrial Natriuretic Factor; Family; Female; Humans; Hydrochlorothiazide; Hyperkalemia; Hypertension; Male; Pedigree; Syndrome

A 32-year-old man was diagnosed as having pseudo-Bartter syndrome due to surreptitious habitual vomiting and to maldigestion related to decayed teeth. His chief complaints were muscle pain and weakness. In this case, metabolic alkalosis, hypokalemia, hypochloremia, increased plasma renin activity and aldosterone levels were noticed with marked decreases in urinary chloride excretion. Creatinine clearance (GFR) and renal plasma flow (RPF) were also decreased. Blood pressure was normal, but the pressor response to angiotensin II was attenuated. Before treatment with 0.9% saline infusion, plasma vasopressin (AVP) was not suppressed sufficiently by lowering the plasma osmolality (Posm) with an oral water load (WL), but it normally responded to a rise in Posm due to hypertonic saline infusion. Moreover, plasma AVP was normally suppressed by WL after the replenishment of saline. Plasma atrial natriuretic peptide (ANP) was low before WL, but increased normally in response to WL. However, inconsistent with the normal response in this case, decreases in plasma AVP failed to dilute urinary osmolality and to increase urine flow, irrespective of the levels of plasma ANP. These results indicate that chronic inanition due to surreptitious vomiting causes impaired renal diluting ability through decreases in GFR and RPF, irrespective of the levels of plasma AVP and ANP.

Topics: Adult; Arginine Vasopressin; Atrial Natriuretic Factor; Bartter Syndrome; Diuresis; Glomerular Filtration Rate; Humans; Male; Muscular Diseases; Syndrome; Vomiting; Water

The syndrome of hypertension and hyperkalemia, hyperchloremic acidosis with normal glomerular filtration rate (Gordon's syndrome) is characterised by volume expansion, suppressed renin and reduced mineralocorticoid-induced renal clearance of potassium. The clinical and biochemical defects are aggravated by high salt diet and corrected by low salt diet, leading to the hypothesis of excessive sodium reabsorption in the nephron proximal to where aldosterone acts. In this study, we used lithium clearance as a marker of proximal sodium reabsorption in three patients with Gordon's syndrome, in order to further localise the site in the nephron of defective sodium handling. Fractional excretion of lithium was decreased, and absolute and fractional proximal reabsorption of sodium was increased compared to normal controls. In addition, absolute distal reabsorption of sodium was decreased, consistent with decreased mineralocorticoid activity. Fractional excretion of potassium was markedly decreased and did not rise with increased distal delivery of sodium during saline infusion. However, after severe dietary sodium restriction had elevated plasma aldosterone (lowering plasma potassium levels to normal), fractional excretion of potassium was raised by saline infusion. Reduced lithium clearance in patients with Gordon's syndrome supports the hypothesis of increased proximal sodium reabsorption in this condition.

Topics: Absorption; Adult; Aldosterone; Atrial Natriuretic Factor; Female; Glomerular Filtration Rate; Humans; Hyperkalemia; Hypertension; Kidney Tubules, Proximal; Lithium; Male; Metabolic Clearance Rate; Potassium; Renin; Sodium; Syndrome

Topics: Adult; Anemia, Hemolytic; Atrial Natriuretic Factor; Blood Pressure; Female; Humans; Liver Function Tests; Pre-Eclampsia; Pregnancy; Prospective Studies; Syndrome; Thrombocytopenia; Water-Electrolyte Balance

The aim of the study was to investigate plasma concentrations of atrial natriuretic peptide, aldosterone, and renin during experimentally induced acute central venous congestion.. Two experimental calf models were used: (1) right heart failure due to pulmonary artery obstruction; (2) inferior vena cava syndrome produced by inferior vena caval obstruction. Hormonal responses and haemodynamic variables were measured over 6 h.. Experiments were performed on three female "Schwarzbund" calves, age 3 months, weight 92 +/- 8 kg.. In the pulmonary artery obstructed group there was an increase of plasma aldosterone from 6.5(SEM 1.6) to 22.1(3.2) ng.dl-1 (p less than 0.05), of renin from 0.7(0.1) to 2.5(0.3) Goldblatt units x 10(-4).ml-1 (p less than 0.05), and of atrial natriuretic peptide from 22.1(4.5) to 141.4(27.8) pmol.litre-1 (p less than 0.05). During inferior vena caval obstruction, aldosterone increased from 2.4(0.4) to 20.9(2.0) ng.dl-1 (p less than 0.05), and renin increased from 0.4(0.05) to 2.0(0.20) Goldblatt units x 10(-4).ml-1 (p less than 0.05). In this experiment, atrial natriuretic peptide remained unchanged. Cardiac output decreased in both groups. There was significant fluid and electrolyte retention during both experiments, with urine volume decreasing from 87.7(11.6) to 35.0(1.2) ml-h-1 in experiment (1), and from 185(14) to 95.7(8.6) ml.h-1 in experiment (2).. The study suggests (1) that in an experimental acute state of reduced cardiac output due to pulmonary artery stenosis with constantly increased right heart pressures, raised endogenous atrial natriuretic peptide failed to induce diuresis and natriuresis; (2) that in acute right heart failure, renin and aldosterone secretion could not be suppressed by raised atrial natriuretic peptide concentrations; and (3) atrial natriuretic peptide secretion seemed to be exhausted after 6 h continuous atrial distension.

Topics: Acute Disease; Aldosterone; Animals; Atrial Natriuretic Factor; Blood Pressure; Cardiac Output; Cardiac Output, Low; Cattle; Central Venous Pressure; Constriction, Pathologic; Diuresis; Electrolytes; Female; Heart Atria; Pulmonary Artery; Renin; Syndrome; Vena Cava, Inferior

An increase in central blood volume in microgravity may result in increased plasma levels of atrial natriuretic factor (ANF). Since elevations in plasma ANF are found in clinical syndromes associated with edema, and since space motion sickness induced by microgravity is associated with an increase in central blood volume and facial edema, we determined whether ANF increases capillary permeability to plasma protein. Conscious, bilaterally nephrectomized male rats were infused with either saline, ANF + saline, or hexamethonium + saline over 2 h following bolus injections of 125I-albumin and 14C-dextran of similar molecular size. Blood pressure was monitored and serial determinations of hematocrits were made. Animals infused with 1.0 micrograms.kg-1.min-1 ANF had significantly higher hematocrits than animals infused with saline vehicle. Infusion of ANF increased the extravasation of 125I-albumin, but not 14C-dextran from the intravascular compartment. ANF also induced a depressor response in rats, but the change in blood pressure did not account for changes in capillary permeability to albumin; similar depressor responses induced by hexamethonium were not accompanied by increased extravasation of albumin from the intravascular compartment. ANF may decrease plasma volume by increasing permeability to albumin, and this effect of ANF may account for some of the signs and symptoms of space motion sickness.

Topics: Adaptation, Physiological; Animals; Atrial Natriuretic Factor; Capillary Permeability; Edema; Male; Nephrectomy; Rats; Rats, Inbred Strains; Serum Albumin, Radio-Iodinated; Space Flight; Syndrome

Seven patients (6 men and 1 woman, mean age 39.1 +/- SD 6.9 years) with psychosis, intermittent hyponatremia, and polydipsia (PIP syndrome) underwent serial determinations of serum sodium (SOD), plasma atrial natriuretic peptide (ANP), and urinary osmolality (UOSM) at 7 AM and 4 PM. There was a diurnal increase in ANP (7 AM 17.9 +/- 5.1 pg/ml and 4 PM 27.7 +/- 9.0 pg/ml, p = 0.02), a diurnal decrease in serum sodium (7 AM 141.1 +/- 1.7 mEq/l, 4 PM 129.9 +/- 3.2 mEq/l, p less than 0.0001) and no diurnal change in UOSM. The diurnal increase in ANP in the the PIP syndrome contrasts to the diurnal decrease in ANP reported in normal subjects. Our data, while preliminary, suggest that patients with the PIP syndrome have increased intravascular volume leading to ANP secretion, natriuresis, and hyponatremia.

Topics: Adult; Atrial Natriuretic Factor; Circadian Rhythm; Female; Humans; Hyponatremia; Male; Middle Aged; Osmolar Concentration; Psychotic Disorders; Sodium; Syndrome; Thirst; Urine

Gordon's syndrome was diagnosed in a 19-year-old woman who had hypertension, hyperkalemia and hyperchloremic acidosis. In family screening, hyperkalemia and hyperchloremic acidosis were also found in the patient's mother and brother. The proband and her brother were studied and showed normal glomerular function with normal renal sodium conservation and urine acidification mechanisms. The levels of plasma aldosterone were normal in both subjects. The renin activity was low in the proband but normal in the brother. Both the basal and the volume-stimulated plasma concentration of atrial natriuretic peptide was low in the two patients. As compared with controls, the kaliuretic response to infusion of sodium chloride was not decreased in the patients. Hydrochlorothiazide promptly corrected the acidosis and the hyperkalemia as well as normalized the raised blood pressure of the proband. We suggest that a deficiency of atrial natriuretic peptide rather than an unusual avidity for sodium chloride reabsorption by the renal tubules plays a significant pathogenetic role in Gordon's syndrome.

Topics: Acid-Base Equilibrium; Adolescent; Adult; Atrial Natriuretic Factor; Female; Glomerular Filtration Rate; Humans; Hyperkalemia; Hypertension; Male; Renin-Angiotensin System; Sodium Chloride; Syndrome

We present the case of a 14-year-old boy who had secondary hyperkalemic periodic paralysis caused by Gordon's syndrome. This syndrome consists of hypertension, tubular acidosis, and hyperkalemia with normal glomerular filtration rate. The pathophysiological mechanism is still unknown. Pathophysiological studies suggest that in this disorder the kidney lacks sensitivity to atrial natriuretic peptide. After treatment with hydrochlorothiazide, serum potassium and plasma aldosterone values, plasma renin activity, and blood pressure became normal and the attacks of periodic paralysis disappeared.

Topics: Adolescent; Atrial Natriuretic Factor; Humans; Hydrochlorothiazide; Hyperkalemia; Hypertension; Male; Paralysis; Syndrome

The case of a 7-year-old boy with the normotensive form of "chloride-shunt" syndrome is described. An unusual feature was the clinical presentation with lithiasis, caused by marked hypercalciuria of renal origin. The present studies were carried out to investigate the nature of the renal tubular defect. Indices for proximal and distal sodium chloride reabsorption were increased during hypotonic saline diuresis. Baseline sodium chloride excretion was low but increased above the range of control values after acute furosemide administration. Baseline potassium excretion was low, was not modified by the infusion of sodium chloride and increased significantly during infusions of sodium sulphate or sodium bicarbonate. Calcium excretion remained unchanged during sodium chloride, sodium sulphate or sodium bicarbonate infusions, but increased after furosemide administration. Nasal insufflation of 1-desamino-8-D-arginine-vasopressin induced both an increase in potassium excretion and a decrease in calcium and magnesium excretion. Plasma atrial natriuretic peptide was increased and was not significantly modified by infusion of hypertonic saline or acute administration of furosemide. These findings indicate that the primary renal abnormality appears to be an enhanced tubular reabsorption of sodium chloride, apparently present in the proximal tubule and the ascending loop of Henle. The associated presence of hypercalciuria also suggests a transport defect in the distal tubule. Decreased potassium excretion probably depends on a voltage-shunting defect in the cortical collecting tubule, which can be reversed by increasing the delivery of non-reabsorbable anions or by enhancing the conductance of the luminal membrane.(ABSTRACT TRUNCATED AT 250 WORDS)

Topics: Atrial Natriuretic Factor; Calcium; Chlorides; Humans; Hydrochlorothiazide; Infant; Kidney; Kidney Diseases; Kidney Tubules; Male; Potassium; Sodium Chloride; Syndrome

Basic examination of Mr S., 45 years of age, short in stature and overweight (1.60 m, 76 kg), was carried out because of the mild hypertension (mean AP 125 mm Hg) from which he had suffered for 20 years. The results were as follows: (1) variable hyperkalemia: plasma potassium values were 5.3 to 6.9 mmol/l; (2) normal renal function: serum creatinine 91.5 mumol/l, clearance of inulin 136.6 ml/mn; (3) proximal tubular acidosis: plasma bicarbonate and chloride values were 18.4 and 109 mmol/l, respectively; urinary pH was 7.1 with negative H+ ions urinary excretion (-33 mumol/mn); when plasma bicarbonate level was raised to 26 mmol/l by acute loading, fractional excretion of bicarbonate increased to 19,5 p. 100 while plasma potassium value decreased to 4.2 mmol/l; (4) low PRA (0.29 ng/ml/h) and normal plasma aldosterone concentration (63 pg/ml) with a normal intake of sodium and in a recumbent position. Plasma atrial natriuretic factor (ANF) level was normal: 14 fmol/ml. Intravenous infusion of ANF for 2 h (1 microgram/mn) induced the expected increases in urinary flow rate, and sodium and potassium excretions (+226, +307 and +171 p. 100, respectively). Intravenous infusion of isotonic saline (2 l in 2 h) and oral administration of fludrocortisone acetate for 4 weeks (400 micrograms per day) resulted in a normal decrease in PRA and plasma aldosterone concentration, a normal rise in plasma ANF level (22 and 42 fmol/ml) while slightly increasing AP without improving bicarbonaturia and acidosis.(ABSTRACT TRUNCATED AT 250 WORDS)

Topics: Acidosis, Renal Tubular; Aldosterone; Atrial Natriuretic Factor; Humans; Hyperkalemia; Hypertension; Kidney; Male; Middle Aged; Pseudohypoaldosteronism; Renal Tubular Transport, Inborn Errors; Renin; Syndrome

A family with the syndrome of hypertension and hyperkalaemia affecting six members in two generations is reported from Australia, where the first two sporadic cases were described. All family members had hyperkalaemia, hyperchloraemia and normal creatinine clearance. Only one affected adult and no affected children were hypertensive, possibly because of habitual low-salt diets. Plasma potassium fell significantly during fludrocortisone acetate administration, and urine potassium increased during saline infusion, consistent with renal tubular responsiveness to mineralocorticoid. Low plasma renin activity and pressor hyper-responsiveness to angiotensin II suggested sodium volume overload, but atrial natriuretic factor (ANF) was normal or only slightly elevated when compared with clearly elevated levels in primary aldosteronism. Plasma ANF was unresponsive to the usually reliable stimulus of angiotensin infusion in the two brothers affected and to saline infusion in one of them. These findings are consistent with a renal tubular avidity for sodium, leading to volume expansion, suppression of renin, and, depending on dietary sodium intake, hypertension. A role for dysregulation of ANF in the pathophysiology is possible.

Topics: Aldosterone; Angiotensin II; Atrial Natriuretic Factor; Australia; Fludrocortisone; Humans; Hyperkalemia; Hypertension; Pedigree; Renin; Syndrome

Plasma concentrations of immunoreactive human atrial natriuretic peptide (human ANP) were sequentially determined in 12 infants with respiratory distress syndrome (RDS) or meconium aspiration syndrome (MAS) during various phases of diuresis to elucidate the role of human ANP in the occurrence of spontaneous diuresis in the newborn. Plasma immunoreactive ANP concentrations during the diuretic as well as the maximum diuretic phase were significantly (p less than 0.001) higher than during the prediuretic phase. A gradual decrease occurred during the post diuretic phase, returning to prediuretic values after one week of life. Significant natriuresis, increased glomerular filtration rate, mild hyponatremia, and decreased blood pressure were observed in the diuretic phase in all the cases studied. These results suggest that hypersecretion of human ANP may play an important part in initiating spontaneous diuresis in sick neonates.

Topics: Atrial Natriuretic Factor; Diuresis; Female; Humans; Infant, Newborn; Kidney; Male; Meconium; Pneumonia, Aspiration; Respiratory Distress Syndrome, Newborn; Syndrome; Urine

A 14-year-old boy with the syndrome of hypertension and hyperkalaemia with normal glomerular filtration rate (Gordon's syndrome) is described. The patient's clinical symptoms consisted of periodic paralysis, slight metabolic acidosis of the proximal type and hypercalciuria. Prostaglandin excretion was normal. Infusion of atrial natriuretic peptide had no effect on electrolyte excretion or glomerular function although a normal increase in cyclic guanosine monophosphate was demonstrated in plasma and urine. This lack of sensitivity to atrial natriuretic peptide offers a new pathophysiological concept in this syndrome. Treatment with hydrochlorothiazide was successful in this case.

Topics: Adolescent; Atrial Natriuretic Factor; Glomerular Filtration Rate; Humans; Hyperkalemia; Hypertension; Kidney Glomerulus; Male; Paralysis; Syndrome

Topics: Adenoma; Adrenal Gland Neoplasms; Atrial Natriuretic Factor; Bartter Syndrome; Humans; Hyperaldosteronism; Plasma Volume; Radioimmunoassay; Syndrome

Plasma levels of ANP were measured in normal subjects and in three conditions associated with disturbed volume homeostasis. Levels of ANP were appropriately raised in seven patients with primary aldosteronism, and fell to normal following removal of an aldosterone-producing adenoma in six and dexamethasone treatment in one patient with glucocorticoid-suppressible hyperaldosteronism. The level of ANP in one patient with Gordon's syndrome (a condition associated with plasma volume expansion) was lower than in the patients with primary aldosteronism, both before and after saline infusion. This is consistent with reduced ANP responsiveness in this condition. responsiveness in this condition. Levels of ANP were inappropriately elevated in three patients with Bartter's syndrome (a condition with plasma volume contraction) and rose further during saline infusion. This is consistent with primary hypersecretion of ANP.

Topics: Adult; Atrial Natriuretic Factor; Bartter Syndrome; Humans; Hyperaldosteronism; Hyperkalemia; Hypertension; Male; Middle Aged; Plasma Volume; Syndrome

Topics: 2,4-Dichlorophenoxyacetic Acid; Atrial Natriuretic Factor; Central Nervous System Stimulants; Convulsive Therapy; Electroconvulsive Therapy; Electroshock; Syndrome