The heart as an endocrine organ

The discovery of atrial natruietic peptide firmly established that the heart was not just a pump and provided the long-sought-after link between the heart and the kidney in the control of soduim balance. Pharmacological targeting of the natruiretic peptide system is now leading to novel advances in the treatment of hypertension and of heart failure - two of the most common causes of human disability and death.     

The heart as an endocrine organ

Modern data on atrial natriuretic factor (ANF) are presented. Synthesis of this factor, its storage and release from cardiac atria are described. The role of ANF in the body fluid volume regulation and blood pressure homeostasis is discussed. ANF is regarded as a circulating hormone.     

The dysplastic naevus syndrome and endocrine disease.

Members of two different families were found to have the dysplastic naevus syndrome and coexistent endocrine abnormalities. The dysplastic naevus syndrome is probably inherited as an autosomal dominant trait and has been associated with other primary malignancies. This is the first time that it has been described in association with endocrine abnormalities.     

Coeliac disease in endocrine diseases of autoimmune origin

Abstract Coeliac disease (CD, sometimes called gluten-sensitive enteropathy or nontropical sprue) is an inflammatory disorder of the small intestine of autoimmune origin. It occurs in genetically predisposed people and is induced by a gluten protein, which is a component of wheat. The prevalence of histologically confirmed CD is estimated in screening studies of adults in the United States and Europe to be between 0.2% and 1.0%. The results of previous studies have indicated that the prevalence of CD is increased in patients with other autoimmune disorders such as: autoimmune thyroid diseases, type 1 diabetes mellitus, and Addison's disease. A coincidence of the above diseases constitutes autoimmune polyglandular syndrome (APS). The high prevalence of CD in APS is probably due to the common genetic predisposition to the coexistent autoimmune diseases. The majority of adult patients have the atypical or silent type of the disease. This is the main reason why CD so often goes undiagnosed or the diagnosis is delayed. CD, if undiagnosed and untreated, is associated with many medical disorders including haematological (anaemia), metabolical (osteopenia/osteoporosis), obstetric-gynaecological (infertility, spontaneous abortions, late puberty, early menopause), neurological (migraine, ataxia, epilepsy) as well as with an increased risk of malignancy, especially: enteropathy-associated T-cell lymphoma, small intestine adenocarcinoma, and oesophageal and oropharyngeal carcinomas. Early introduction of a gluten-free diet and lifelong adherence to this treatment decreases the risk of these complications.     

The heart and control of renal excretion: neural and endocrine mechanisms.

There has been a great deal of research concerning the heart being an important regulator of renal fluid and electrolyte excretion. This cardiac-renal connection involves two different types of major mechanisms, both of which are covered in this review. The first of these to be discovered was neural reflex regulation. This type of control is due to the fact that the heart possesses nerve receptors whose activity is altered by changes in the degree of cardiac stretch that occur as a result of changes in blood volume. These receptors affect various humoral, neural, and perhaps hemodynamic mechanisms that modify renal excretion. A second, more recently discovered type of regulation is based on the concept that the heart is also an endocrine gland. Similar to neural receptor activity, cardiac hormone secretion is also linked to the degree of cardiac stretch or filling. These cardiac peptides have been shown to have a variety of physiologic effects, most of which directly or indirectly affect renal excretion. Both of the above cardiorenal control mechanisms, one neural and one humoral, may be important not only in maintaining normal fluid-electrolyte balance but may also have pathophysiologic relevance.     

The changing pattern of ischemic heart disease

Male and female death rates from all the major forms of cardiovascular disease were approximately equal until about 1920. Since that time the male:female ratio in fatal ischemic heart disease (IHD) has risen dramatically, but some closely related diseases such as cerebrovascular disease and uncomplicated angina pectoris have maintained sex ratios close to unity. It is difficult to reconcile this divergent trend in the sex ratio of IHD with a simple stenotic-thrombotic view of myocardial infarction (MI) and it is suggested that the modern epidemic of MI in men may be the result of a disorder of muscle metabolism (“vulnerable myocardium”) superimposed on a relatively stable background of stenotic-thrombotic arterial disease. The proposed mechanism would also help to explain the selective action of some modern “coronary risk factors” (such as cigarette smoking and physical inactivity) which increase the risk of MI but have little or no effect on the risk of developing cerebrovascular disease or uncomplicated angina pectoris.     

The surgical treatment of acquired heart disease

Success in the surgical treatment of certain forms of congenital cardiac malformation has led to a renewed interest in the treatment of acquired heart disease. Similarly, technical advances and a better understanding of cardiac physiology under operative conditions have enabled surgeons to broaden the field and improve the possibilities of cardiac operations.     

The therapeutic potential of stem cells in heart disease

Abstract.  Coronary heart disease and chronic heart failure are common and have an increasing frequency. Although interventional and conventional drug therapy may delay ventricular remodelling, there is no basic therapeutic regime available for preventing or even reversing this process. Chronic coronary artery disease and heart failure impairs quality of life and are associated with subsequent worsening of the cardiac pump function. Numerous studies within the past few years have been demonstrated, that the intracoronary stem cell therapy has to be considered as a safe therapeutic procedure in heart disease, when destroyed and/or compromised heart muscle must be regenerated. This kind of cell therapy with autologous bone marrow cells is completely justified ethically, except for the small numbers of patients with direct or indirect bone marrow disease (e.g. myeloma, leukaemic infiltration) in whom there would be lesions of mononuclear cells. Several preclinical as well as clinical trials have shown that transplantation of autologous bone marrow cells or precursor cells improved cardiac function after myocardial infarction and in chronic coronary heart disease. The age of infarction seems to be irrelevant to regenerative potency of stem cells, since stem cells therapy in old infarctions (many years old) is almost equally effective in comparison to previous infarcts. Further indications are non-ischemic cardiomyopathy (dilative cardiomyopathy) and heart failure due to hypertensive heart disease.     

Anderson-Fabry disease in heart failure

Anderson-Fabry disease is an X-linked lysosomal storage disorder caused by mutations in the GLA gene that result in deficiency of the enzyme alpha-galactosidase A. The worldwide incidence of Fabry’s disease is reported to be in the range of 1 in 40,000–117,000, although this value may be a significant underestimate given under recognition of symptoms and delayed or missed diagnosis. Deficiency in alpha-galactosidase A causes an accumulation of neutral glycosphingolipids such as globotriaosylceramide (Gb3) in lysosomes within various tissues including the vascular endothelium, kidneys, heart, eyes, skin and nervous system. Gb3 accumulation induces pathology via the release of pro-inflammatory cytokines, growth-promoting factors and by oxidative stress, resulting in myocardial extracellular matrix remodelling, left ventricular hypertrophy (LVH), vascular dysfunction and interstitial fibrosis. Cardiac involvement manifesting as ventricular hypertrophy, systolic and diastolic dysfunction, valvular abnormalities and conduction tissue disease is common in AFD and is associated with considerable cardiovascular morbidity and mortality from heart failure, sudden cardiac death and stroke-related death.