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The physiology of the endocrine system.

Understanding the basic physiology of endocrinology and metabolism is important for the safe conduct of anesthesia of patients with endocrine disease. Full endocrine assessment and considered interpretation of the results is essential before anesthesia and surgery. In this chapter, we have covered the background physiology and the basic investigations that may be required to diagnose and direct treatment in order to optimize perioperative care.

Anesthesia↗

Bone scanning in metabolic and endocrine bone disease.

99mTc diphosphonate bone scanning has proved to be a sensitive, though nonspecific, way of detecting a wide variety of metabolic bone disorders. Classic scan appearances of generalized increased tracer uptake throughout the skeleton are found in many types of metabolic bone disease, and whole-body retention measurements can be used to detect early forms of disease. Focal disease identified on bone scanning such as pseudofractures and brown tumors are clearly identified on the 99mTc diphosphonate bone scan, with isotope scan changes preceding radiological changes by up to 18 months. The use of whole-body retention measurements will further increase sensitivity of detection in patients with metabolic bone disease.

Bone Diseases↗

Endocrine disorders affecting the cardiovascular system.

Endocrine disorders can affect the cardiovascular system in many ways. Patients admitted to an intensive care unit with primary cardiovascular diagnoses may have underlying endocrine dysfunction that can cause or adversely influence cardiovascular problems. Early diagnosis and treatment of endocrine disease can often prevent or reverse cardiovascular problems and contribute to more acceptable patient outcomes.

Cardiovascular Diseases↗

[Addisonian crisis provoked by levothyroxine substitution therapy].

A women aged 36 with a positive family anamnesis for autoimmune endocrine diseases and a history of thyroid diseases, developed major complaints of general malaise, orthostatic hypotension and loss of appetite after the start of a treatment with levothyroxin because of (sub)clinical hypothyroidism. She was found to suffer from primary adrenocortical insufficiency masked by excessive use of liquorice and a lowered metabolism, but which via the suppletion with thyroid hormone had led to an addisonian crisis.

Addison Disease↗

A short history of pediatric endocrinology in North America.

Pediatric endocrinology evolved as a subspecialty from the era of biochemical and metabolic clinical investigation led by John Howland, Edwards Park, and James Gamble at Johns Hopkins; Allan Butler at Boston University and Harvard University; Daniel Darrow at Yale University; and Irving McQuarrie at the University of Rochester and the University of Minnesota during the early 20th century. The father of the new subspecialty was Lawson Wilkins, a private pediatric practitioner in Baltimore, Maryland, who was invited by Dr. Edwards Park to establish an endocrine clinic at the Harriet Lane Home at Johns Hopkins in 1935. Dr. Wilkins managed his practice and the clinic until 1946, when, at the age of 52, he accepted a full-time position at the University. Dr. Nathan Talbot was invited to develop a pediatric endocrine clinic at Massachusetts General Hospital by Allan Butler in 1942. These units and their associated subspecialty training programs during the 1950s and 1960s provided the large majority of the second-generation pediatric endocrinologists who went on to establish endocrine subspecialty programs in university medical centers in North America as well as Europe and South America. Diabetes as a clinical pediatric discipline evolved in parallel from the early clinics of Elliott Joslin and Priscilla White in Boston, M.C. Hardin and Robert Jackson at the University of Iowa, George Guest at the University of Cincinnati Children's Hospital, and Alex Hartman at the St. Louis Children's Hospital. The Lawson Wilkins Pediatric Endocrine Society was founded in 1971, and the Council on Diabetes and Youth was established within the American Diabetes Association in 1980. Medical and economic factors led to increasing integration of pediatric diabetes and general endocrine care and training, and diabetes care now is a major activity within the subspecialty of pediatric endocrinology. The growth of pediatric endocrinology in North America has paralleled the growth of academic medicine during the past half-century. In 2002, there were 72 training programs in North America: 65 in the United States and seven in Canada. The endocrinology sub-board of the American Board of Pediatrics was established in 1978 to certify training and competence in endocrinology, including diabetes. By 2002, the board had certified 927 pediatric endocrinologists. Pediatric endocrine subspecialists during the past half-century have contributed major advances in our understanding of the ontogeny of endocrine systems and the diagnosis and treatment of fetal-perinatal endocrine disorders; newborn screening for endocrine and metabolic disorders; the physiology and therapies for disorders of sexual differentiation and pubertal maturation; the development of anthropometric standards for childhood growth and development; the characterization and physiology of hormone systems, including receptors and hormone actions; the molecular genetics of a number of congenital endocrine disorders and heritable endocrine diseases; development of pediatric endocrine diagnostics and reference standards; the pathophysiology and management of autoimmune endocrine disease; and development of a growing armamentarium of therapeutic agents for treatment of endocrine and metabolic diseases.

Animals↗

Diseases associated with calcium pyrophosphate deposition disease.

Although many metabolic and endocrine diseases have been reported to predispose to calcium pyrophosphate dihydrate crystal deposition, the validity of many of these associations remains unclear. A critical review of the literature relating to these associations, with illustrative cases and data derived from the authors' own experience, is presented. It is concluded that there is good evidence to associate hypophosphatasia, hypomagnesemia, and hyperparathyroidism with chondrocalcinosis and acute attacks of "pseudogout." Meta-analysis also suggests a small but significant association between hypothyroidism and chondrocalcinosis. Hemochromatosis stands alone in clearly associating not only with chondrocalcinosis but also with structural change and chronic arthropathy. The biochemical mechanisms that may produce these various associations are discussed. Recommendations are made concerning appropriate screening for metabolic and endocrine disease in patients with chondrocalcinosis.

Acromegaly↗

Familial syndrome of progressive cone dystrophy, degenerative liver disease, and endocrine dysfunction. III. Genetic studies.

A syndrome of progressive cone dystrophy, endocrine dysfunctions and degenerative liver diseases has been observed in seven patients, six of whom belonged to one extensive kindred. Genetic analyses revealed a segregation ratio indicating autosomal recessive inheritance of the syndrome, and the kindred from which six of the seven patients originated was heavily inbred. Thus, the results of the segregation analyses as well as of the inbreeding analyses provide evidence that this previously unrecognized disorder is inherited as an autosomal recessive trait. Genetic marker analyses were conducted with respect to 22 marker systems, and linkage information was obtained with respect to 15 of them. No strong suggestion of linkage emerged from the analyses, but very close linkage could be excluded for several of genetic marker systems. Pedigree analysis was helpful in establishing the spectrum of clinical manifestations belonging to the syndrome proper. The data presently available suggest that elevated levels of creatine phosphokinase, which were found in all patients, may be useful in tracing heterozygotes for this disorder. This possibility will be further examined.

Abnormalities, Multiple↗

[Endocrine and metabolic diseases].

Multiple gene mutation inducing common metabolic diseases(diabetes mellitus and hyperlipidemia) and single gene mutation inducing congenital metabolic diseases or inherited endocrine disease are the main targets of DNA diagnosis. We review our current understanding of DNA diagnosis of representative inherited endocrine and metabolic diseases.

Endocrine System Diseases↗

Hormonal determinants of apolipoprotein B,E receptor expression in human liver. Positive association of receptor expression with plasma estrone concentration in middle-aged/elderly women.

The relationships of the expression of hepatic low-density lipoprotein (LDL) receptors (apo B,E receptors) to several plasma hormone concentrations were examined in 15 fasted women aged 37-75 years (mean, 57 years), who were undergoing laparotomy for non-neoplastic disease. No subject had clinical or biochemical evidence of familial hypercholesterolemia, renal disease, hepatic disease, or endocrine disease. Hepatic apo B,E receptor expression was quantified in vitro as the EDTA-suppressible binding of 125I-labeled human LDL (15 micrograms protein/ml) by liver homogenate at 37 degrees C; values were 23-75 ng LDL protein/mg cell protein (mean, 47 ng/mg). Receptor expression was strongly correlated with plasma estrone concentration (rs = +0.70, P = 0.035), but was unrelated to the concentrations of testosterone, thyroxine, free triiodothyronine, cortisol, sex hormone-binding globulin (SHBG) or cortisol-binding globulin. Insulin and estradiol concentrations were mostly very low. The correlation of receptor expression with plasma total estrone concentration reflected associations with both the albumin-bound (rs = +0.78, P = 0.014) and unbound (rs = +0.80, P = 0.009) fractions, but not with the SHBG-bound fraction (rs = -0.22, P = 0.574), of this hormone. As the non-SHBG-bound fractions of gonadal steroids are considered to be the biologically active components, these results are consistent with experimental evidence that the synthesis of apo B,E receptors in hepatocytes is stimulated by estrogens, and suggest that circulating estrone may be the major hormonal determinant of receptor expression in fasted middle-aged/elderly women.

Adult↗

[Importance of routine examinations in patients with obstructive sleep apnea syndrome].

BACKGROUND AND OBJECTIVE: In patients with obstructive sleep apnea syndrome (OSAS) there is an increased comorbidity of internal diseases such as hypertension, coronary heart disease, chronic obstructive pulmonary disease and endocrine diseases. We analyzed prospectively frequency and consequences of pathological results in routine examinations of internal medicine in sleep laboratory. METHODS: 250 patients with OSAS underwent routine bodyplethysmography, blood- gas analysis, electrocardiogram and laboratory studies after anamnesis and clinical examination. Prior to this we indicated, whether we based on history and physical examination deemed any of these examinations necessary. Frequency and kind of pathological results as well as the consequences were analyzed. RESULTS: 129 results of bodyplethysmography and blood-gas analysis were pathological (51.6 % of all investigations), most frequently hypoxemia (22 %) and obstructive pattern (16.4 %). Further steps were necessary in 19 patients (7.6 %); the indication was seen before in 13 patients, therefore 6 results with consequences (2.4 %) were not expected. Laboratory studies were abnormal in 133 patients (53.2 % of all investigations), most of them hyperglycaemia (26.8 %) and elevated liver enzymes (20.4 %). 29 results (11.6 %) had consequences, of which 16 (6.4 %) were not expected. 82 electrocardiograms were pathological (32.8 % of all investigations), presenting most frequently as coronary heart disease (21.2 %). Further steps were necessary in 5 patients (2 %), while that was not expected in 3 patients (1.2 %). CONCLUSIONS: Routine examinations of internal medicine in patients with OSAS frequently present pathological results, which seldom have further consequences. As most of the important results can be predicted by history and clinical investigation, pathological by chance diagnoses are rare. With the exception of blood sugar tests routine examinations for patients with OSAS should be considered critically.

Blood Gas Analysis↗

Are Gulf War veterans suffering war-related illnesses? Federal and civilian hospitalizations examined, June 1991 to December 1994.

A previous epidemiologic study demonstrated no unexplained increase in risk for postwar hospitalization among Gulf War veterans who had remained on active duty. The authors sought to expand this study to include Reserve and separated military personnel. They examined hospitalization data from the Department of Defense, the Department of Veterans Affairs (VA), and the California Office of Statewide Health Planning and Development hospital systems for the years 1991-1994. Since denominator data were not available, the authors compared the proportional morbidity ratios (PMRs) of hospitalization discharge diagnoses (both large categories and specific diagnoses) between Gulf War veterans and other veterans of the same era. There were no indications that Gulf War veterans were suffering increased PMRs for infectious diseases; neoplasms; endocrine diseases; blood diseases; skin conditions; or diseases of the nervous system, circulatory system, or musculoskeletal system. However, these veterans did experience proportionally more hospitalizations for various specific diagnoses, namely, fractures and bone and soft-tissue injuries (Department of Defense and California Office of Statewide Health Planning and Development), various diseases of the respiratory (including asthma) and digestive systems (VA), and diverse symptom diagnoses (VA). While these findings may be influenced by chance or by a number of potential confounders, including health registry participation, they merit further examination using other study designs.

Adolescent↗