Thyrotoxicosis

Paz-Filho GJ, Graf H. · SEMPR - Serviço de Endocrinologia e Metabologia da Universidade Federal do Paraná,Curitiba, Brazil. · Expert Opin Biol Ther. · Pubmed #18847307 No free full text.

Abstract: BACKGROUND: Recombinant human thyroid stimulating hormone (rhTSH) is a valuable tool in the management of thyroid diseases. It is useful for radioiodine ablation in patients with differentiated thyroid carcinoma (DTC), for their follow-up, and for treatment of selected patients with metastatic or recurrent DTC. More recently, it has been suggested that rhTSH is useful for treatment of multinodular goiter and amiodarone-induced thyrotoxicosis, as well as for diagnosis of congenital hypothyroidism. OBJECTIVE: To provide an outline of literature regarding the uses of rhTSH in thyroid diseases. METHODS: We performed a literature search for relevant articles in the PubMed database. CONCLUSION: rhTSH has important roles in management of thyroid diseases, and some are still controversial. For patients with DTC, it avoids the need for thyroid hormone withdrawal, without being detrimental to short-term outcomes. Further studies are warranted to assess its effects on long-term outcomes.

Piga M, Serra A, Boi F, Tanda ML, Martino E, Mariotti S. · Department of Nuclear Medicine and Endocrinology, University Policlinic, University of Cagliari, Cagliari, Italy. · Minerva Endocrinol. · Pubmed #18846027 No free full text.

Abstract: Amiodarone (AM), a potent class III anti-arrhythmic drug, is an iodine-rich compound with a structural resemblance to thyroid hormones triiodothyronine (T3) and thyroxine (T4). At the commonly employed doses, AM causes iodine overload up to 50-100 times the optimal daily intake, which may be responsible of a spectrum of effects on thyroid function often counterbalancing its heart benefits. Although most patients on chronic AM treatment remain euthyroid, a consistent proportion may develop thyrotoxicosis (AM-induced thyrotoxicosis, AIT) or hypothyroidism. AIT is more prevalent in iodine-deficient areas and is currently subdivided in two different clinico-pathological forms (AIT I and AIT II). AIT I develops in subjects with underlying thyroid disease, and is caused by an exacerbation by iodine load of thyroid autonomous function; AIT II occurs in patients with no underlying thyroid disease and is probably consequent to a drug-induced destructive thyroiditis. Mixed or indeterminate forms of AIT encompassing several features of both AIT I and AIT II may be also observed. The differential diagnosis between AIT I and AIT II (which is important for the choice of the appropriate therapy) is currently made on radioiodine uptake (RAIU), which may be high, normal or low but detectable in AIT I, while is consistently very low or undetectable in AIT II and on colour-flow Doppler sonography (CFDS) showing normal or increased vascularity in AIT I and absent vascularity in AIT II. Quite recently, studies carried out in our Units at the University of Cagliari (Italy) showed that sestaMIBI thyroid scintigraphy may represent the best single test to differentiate AIT I (showing increased MIBI retention) from AIT II (displaying no significant uptake). Treatment of AIT is dependent from its etiology. AIT usually responds to combined thionamides and potassium perchlorate (KClO4) therapy, AIT II generally responds to glucocorticoids, while indeterminate forms may require both therapeutic approaches. In patients with AIT I definitive treatment of hyperthyroidism by administration of (131)I, initially not feasible for the low RAIU and/or the risk of thyrotoxicosis exacerbation, is advised after normalization of iodine overload. To control severe AIT additional treatment with lithium carbonate, the use of short course of iopanoic acid and plasmapheresis have been also proposed. In cases resistant to medical treatment and/or in patients with severe cardiac diseases who cannot interrupt AM or require quick AM reintroduction, total thyroidectomy (possibly carried out by minimally invasive video-assisted technique) may be proposed after rapid correction of thyrotoxicosis with combination of thionamides, KClO4, corticosteroids and a short course of iopanoic acid.

Dahl P, Danzi S, Klein I. · No affiliation provided · Curr Heart Fail Rep. · Pubmed #18752767 No free full text.

Abstract: The most recognizable features of hyperthyroidism are those that result from the effects of triiodothyronine (T3) on the heart and cardiovascular system: decreased systemic vascular resistance and increased resting heart rate, left ventricular contractility, blood volume, and cardiac output. Although these measures of cardiac performance are enhanced in hyperthyroidism, the finding of clinical cardiac failure can be somewhat paradoxical. About 6% of thyrotoxic individuals develop symptoms of heart failure, but less than 1% develop dilated -cardiomyopathy with impaired left ventricular systolic function. Heart failure resulting from thyrotoxicosis is due to a tachycardia-mediated mechanism leading to an increased level of cytosolic calcium during diastole with reduced ventricular contractility and diastolic dysfunction, often with tricuspid regurgitation. Pulmonary artery hypertension in thyrotoxicosis is gaining awareness as a cause of isolated right-sided heart failure. In both cases, older individuals are more likely to be affected. Treatment needs to be directed at management of the acute cardiovascular complications, control of the heart rate, and thyroid-specific therapy to restore a euthyroid state that will lead to resolution of the signs and symptoms of heart failure.

Butte N, Böttiger BW, Teschendorf P. · Klinik für Anästhesiologie und Operative Intensivmedizin, Klinikum der Universität zu Köln, Köln, Germany. · Anaesthesist. · Pubmed #18704341 No free full text.

Abstract: Cardiac arrhythmias are a common problem in the perioperative period. The incidence found in the current literature varies depending on the population studied and the definition of arrhythmia used. Overall supraventricular arrhythmias, namely atrial fibrillation, are the most common form. Because of its broad spectrum amiodarone is often used to suppress supraventricular and ventricular arrhythmias. It is believed to be safe for treating patients with severe cardiac disease and it has less proarrhyhmogenic potential than many other antiarrhythmic drugs. However, the use of amiodarone is limited by its cardiac and non-cardiac adverse effects, such as life-threatening bradycardia, pulmonary fibrosis or thyrotoxicosis. According to the guidelines of the American Heart Association, amiodarone can be used to treat atrial fibrillation. Because spontaneous conversion rates in the perioperative setting are high and the advantage of a rhythm control strategy over rate control is questionable, a rate control strategy using less toxic drugs like beta blockers or calcium channel blockers should be preferred in hemodynamically stable patients. The current guidelines of the European Resuscitation Council (ERC) recommend amiodarone to treat hemodynamically stable ventricular tachycardia and in this setting ajmaline is also highly effective. Amiodarone should be administered to patients with cardiac arrest if ventricular tachycardia or ventricular fibrillation persists after three attempts at defibrillation. Dronedarone is a derivate of amiodarone with a similar mechanism of action but with less non-cardiac side effects and is currently being tested in clinical trials. The use of the atrial-specific potassium channel blockers AZD7009 and vernakalant are also being investigated. Furthermore, the role of statins, ACE inhibitors and angiotensin receptor blockers in the prevention of atrial fibrillation has to be evaluated.

Duvic M, Talpur R. · The University of Texas MD Anderson Cancer Center, Division of Internal Medicine, Department of Dermatology, 1515 Holcombe Blvd, Unit 1452, Houston, TX 77030, USA. · Future Oncol. · Pubmed #18684057 No free full text.

Abstract: Denileukin diftitox (Ontak) is a novel recombinant fusion protein consisting of peptide sequences for the enzymatically active and membrane translocation domain of diphtheria toxin linked to human IL-2. Denileukin diftitox specifically binds to IL-2 receptors on the cell membrane, is internalized via receptor-mediated endocytosis and inhibits protein synthesis by ADP ribosylation of elongation factor 2, resulting in cell death. This article focuses on the clinical trial that led to the US FDA approval of the drug for cutaneous T-cell lymphoma in 1999, and other investigational studies for hematologic malignancies, recurrent and refractory chronic lymphocytic leukemia, non-Hodgkin B-cell lymphoma, graft-versus-host disease and autoimmune disease, demonstrating the activity and adverse effects of the drug.

Tanda ML, Bogazzi F, Martino E, Bartalena L. · Division of Endocrinology, Department of Clinical Medicine, University of Insubria, Ospedale di Circolo, Viale Borri, 57, 21100 Varese, Italy. · Eur J Endocrinol. · Pubmed #18667589 links to  free full text

This publication has no abstract.

Hardin SR, Steele JR. · School of Nursing, University of North Carolina at Charlotte, Charlotte, NC 28223, USA. · J Gerontol Nurs. · Pubmed #18649821 No free full text.

Abstract: Atrial fibrillation is the most common arrhythmia among older adults. Valvular heart disease, dilated cardiomyopathy, aortic stenosis, hypertension, coronary artery disease, pericarditis, thyrotoxicosis, pulmonary disease, cardiac surgery, alcohol excess, and alcohol withdrawal are associated with atrial fibrillation. Nurses caring for older adults need to understand the condition's pathophysiology, signs and symptoms, diagnostic data and treatment protocols, and adherence issues to prevent the formation of emboli in chronic atrial fibrillation and to understand treatment of this common arrhythmia. This article presents an individual example of an elderly man exhibiting a new onset of atrial fibrillation and the interventions required to manage the associated complications. Atrial fibrillation places patients at risk for stroke from a thromboembolism; thus, pharmacological and nonpharmocological care strategies for managing patients with atrial fibrillation are discussed.

Aslam M, Inayat M. · Department of Pediatrics and Division of Newborn Medicine, Harvard Neonatal-Perinatal Fellowship Program, Children's Hospital Boston, Boston, MA, USA. · South Med J. · Pubmed #18622337 No free full text.

Abstract: Fetal and neonatal thyrotoxicosis from maternal Graves disease is a very rare entity. Fetal symptoms result from the transplacental passage of thyroid-stimulating immunoglobulins, which persist in the neonate resulting in neonatal symptoms. We present a case of fetal and neonatal thyrotoxicosis from maternal Graves disease. Fetal symptoms were controlled with maternal administration of antithyroid drugs leading to fetal thyroid inhibition. We present this case with a brief review of the literature.

Bassett JH, Williams GR. · Molecular Endocrinology Group, Division of Medicine and MRC Clinical Sciences Centre, Imperial College London, Hammersmith Hospital, London, W12 0NN, UK. · Bone. · Pubmed #18585995 No free full text.

Abstract: Studies in genetically modified mice have highlighted the importance of the hypothalamic-pituitary-thyroid (HPT) axis during skeletal development and the maintenance of adult bone. Recently, the conventional view that skeletal responses to abnormal thyroid status result solely from altered T3 action in bone has been complicated by studies proposing TSH as a negative regulator of bone turnover. Although skeletal consequences of thyrotoxicosis may result from thyroid hormone excess or TSH deficiency, the two alternatives are not necessarily mutually exclusive and cannot easily be differentiated because the HPT axis maintains them in a physiological reciprocal relationship. By contrast, situations in which this inverse relationship is disrupted have the potential to resolve the roles of T3 and TSH in the skeleton. We discuss these situations and the relative importance of T3 and TSH in skeletal homeostasis.

Watkins MG, Dejkhamron P, Huo J, Vazquez DM, Menon RK. · Department of Pediatrics and Communicable Diseases, C.S. Mott Children's Hospital, University of Michigan, Ann Arbor, Michigan 48109-5718, USA. · Endocr Pract. · Pubmed #18558604 No free full text.

Abstract: OBJECTIVE: To report the case of a neonate presenting with nonautoimmune thyrotoxicosis and failure to thrive in whom an activating TSHR mutation was suspected. METHODS: We describe the clinical and laboratory findings in a neonate who presented with nonautoimmune thyrotoxicosis and failure to thrive, including results of DNA analysis of TSHR, which encodes the thyroid-stimulating hormone receptor (TSHR). Relevant literature is also reviewed. RESULTS: The proband was born spontaneously at 35 weeks' gestation, and his early neonatal period was remarkable for meconium aspiration, pneumothorax, hepatomegaly with associated elevated transaminases, and direct hyperbilirubinemia. On days 9 and 11 of life, thyroid function studies revealed hyperthyroidism, which remained persistent on day 26 of life. On day 44 of life, the infant was admitted to the hospital. The mother reported he had an increased activity level, disturbed sleep, jitteriness, and exaggerated startle response. Weight was at the third percentile. After additional workup, Lugol's iodine solution, propanolol, and propylthiouracil were prescribed, which led to improvement in thyroid function. No TSHR antibodies were detected in the mother's or patient's sera. Analysis of the patient's DNA revealed a heterozygous T-to-C substitution at amino acid 568 in exon 10 (Ile568Thr), which predicts an isoleucine to threonine conversion in the second extracellular loop of TSHR. The mutation was not identified in the parents' DNA. CONCLUSIONS: A mutation causing constitutive activation of TSHR was confirmed in this patient, a finding that has implications for genetic counseling and consideration of total thyroidectomy or long-term thionamide therapy followed by radioiodide ablation as treatment options. Although rare, TSHR mutations should be considered in an infant presenting with thyrotoxicosis in absence of demonstrable TSHR antibodies in serum.

Krysiak R, Marek B, Okopień B. · Klinika Chorób Wewnetrznych i Farmakologii Klinicznej Katedry Farmakologii, Slaski Uniwersytet Medyczny, Katowice, Poland. · Endokrynol Pol. · Pubmed #18465693 No free full text.

Abstract: Postpartum thyroiditis is a form of autoimmune thyroiditis developing during the first 12 months postpartum as a consequence of the immunologic flare following the immune suppression of pregnancy. This disease, found in 5-10% of women in a general population and even more frequently in patients suffering from other autoimmune disorders, may re-occur in about 70% of women after a subsequent pregnancy. Postpartum thyroiditis is strongly associated with antithyroid peroxidase antibodies. Patients may present with symptoms of either thyrotoxicosis or hypothyroidism which may be transient or, in some (20-30%) cases of hypothyroidism, permanent in nature. A thyrotoxic phase of postpartum thyroiditis is usually brief and often unnoticed before a more long-lasting hypothyroid phase occurs. The diagnosis of postpartum thyroiditis is based on the observation of abnormal thyroid function tests in a postpartum antithyroid peroxidase- positive woman. In this paper, we discuss the etiopathogenesis, clinical picture, diagnosis, prognosis and treatment of postpartum thyroiditis and provide the reader with some practical guidance concerning dealing with a patient suffering from this disorder.

Porterfield JR, Thompson GB, Farley DR, Grant CS, Richards ML. · Department of Surgery, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA. · World J Surg. · Pubmed #18357484 No free full text.

Abstract: BACKGROUND: Toxic multinodular goiter (Plummer's disease) has posed challenges to surgeons, endocrinologists, and radiation oncologists since its description in 1913. A literature review with evidenced-based methodology has not yet been reported. METHODS: A systematic review of the English literature from 1950 to 2007 and report of Mayo Clinic experience since 1950 was undertaken to establish evidence-based recommendations for management. RESULTS: Surgery and radioactive iodine (RI) are both supported by level IV evidence in the treatment of solitary toxic nodules and toxic multinodular goiter, and treatment is determined by symptoms and co-morbidities. No evidence suggests a difference in treatment outcome based on pretreatment clinical or subclinical hyperthyroidism. Level IV evidence supports thyroidectomy over RI for large goiters. When compressive symptoms are present, level IV evidence supports thyroidectomy for maximal symptom relief in patients at moderate risk. Occult malignancies are found in 2-3% of thyroidectomy specimens for Plummer's disease. Despite technical reports of RI dose considerations, there are no prospective studies validating a dose formula. Ethanol ablation of toxic nodules in patients unfit for surgery is supported by level III evidence. Level V data suggest a cost benefit favoring surgery. CONCLUSIONS: Treatment of Plummer's disease with antithyroid medications, ethanol ablation, RI ablation, or surgery must balance the goals of therapy, durability of cure, relief of symptoms, risk of malignancy, and risk of complications. Between 1950 and 2006, 948 (70%) of 1,356 patients with Plummer's disease have been treated surgically at Mayo Clinic.

Karger S, Führer D. · Klinik für Endokrinologie, Diabetologie und Nephrologie, Universitätsklinikum Leipzig, Leipzig, Germany. · Dtsch Med Wochenschr. · Pubmed #18302101 No free full text.

Abstract: Thyroid storm or thyrotoxic crisis is a rare but life-threatening condition requiring immediate treatment, preferably in an intensive care unit. Its incidence is about 1-2% among patients with overt hyperthyroidism. A thyrotoxic crisis occurs predominantly in the elderly and is three to five times more common in women than in men. The overall mortality is 10-20%. Even though the pathogenesis is still not fully understood, an increased sensitivity to catecholamines appears to be an important mechanism, and a number of endogenous and exogenous stress factors that can provoke the onset of a thyrotoxic storm have been identified. The diagnosis of a thyrotoxic crisis is made entirely on the clinical findings. Most importantly, there is no difference in thyroid hormone levels between patients with "uncomplicated" thyrotoxicosis and those undergoing a thyroid storm. Any delay in therapy, e.g. by awaiting additional laboratory results, must be strictly avoided, because the mortality rate may rise to 75%. Thus early thyroidectomy should be considered as the treatment of choice, if medical treatment fails to result in clinical improvement. Medical treatment is based on three principles: 1) counteracting the peripheral effects of thyroid hormones; 2) inhibition of thyroid hormone synthesis; and 3) treatment of systemic complications. These measures should bring about clinical improvement within 12-24 hours. If death occurs it is most likely to be cardiopulmonary failure, particularly in the elderly.

Mikosch P. · 2. Medizinische Abteilung, Landeskrankenhaus Klagenfurt, St. Veiterstrasse 47, Klagenfurt, Austria. · Wien Med Wochenschr. · Pubmed #18286247 No free full text.

Abstract: Amiodarone is a frequently used antiarrhythmic drug with a high antiarrhythmic potency. However, beside its antiarrhythmic effects Amiodarone also reveals a variety of adverse effects and drug-related complications. The affected organs include the eyes, skin, lungs, nervous system, liver, gastrointestinal tract and the thyroid. The thyroid is one of the most frequently affected organs by Amiodarone. An altered hormone equilibrium always occurs and has to be distinguished from Amiodarone induced hyperthyroidism and hypothyroidism. The differentiation of these states frequently causes problems and may even be a diagnostic and therapeutic challenge in certain cases. The article gives an overview on the interactions between Amiodarone and the thyroid, the diagnostic and therapeutic options and management strategies of patient on Amiodarone therapy in the view of thyroid function.

Silva JE, Bianco SD. · Baystate Medical Center, Tufts University Medical School, Springfield, Massachusetts 01199, USA. · Thyroid. · Pubmed #18279016 No free full text.

Abstract: The sympathoadrenal system, including the sympathetic nervous system and the adrenal medulla, interacts with thyroid hormone (TH) at various levels. Both systems are evolutionary old and regulate independent functions, playing probably independent roles in poikilothermic species. With the advent of homeothermy, TH acquired a new role, which is to stimulate thermogenic mechanisms and synergize with the sympathoadrenal system to produce heat and maintain body temperature. An important part of this new function is mediated through coordinated and, most of the time, synergistic interactions with the sympathoadrenal system. Catecholamines can in turn activate TH in a tissue-specific manner, most notably in brown adipose tissue. Such interactions are of great adaptive value in cold adaptation and in states needing high-energy output. Conversely, in states of emergency where energy demand should be reduced, such as disease and starvation, both systems are turned down. In pathological states, where one of the systems is fixed at a high or a low level, coordination is lost with disruption of the physiology and development of symptoms. Exaggerated responses to catecholamines dominate the manifestations of thyrotoxicosis, while hypothyroidism is characterized by a narrowing of adaptive responses (e.g., thermogenic, cardiovascular, and lipolytic). Finally, emerging results suggest the possibility that disrupted interactions between the two systems contribute to explain metabolic variability, for example, fuel efficiency, energy expenditure, and lipolytic responses.

Chawla M, Bal CS. · All India Institute of Medical Sciences, New Delhi, India. · Thyroid. · Pubmed #18225976 No free full text.

This publication has no abstract.

Kyriazopoulou V, Michalaki M, Georgopoulos N, Vagenakis AG. · Division of Endocrinology and Metabolism, Department of Internal Medicine, University Hospital, Patras 26500, Greece. · Expert Opin Pharmacother. · Pubmed #18220492 No free full text.

Abstract: Disorders of thyroid function are common among women of childbearing age. It is known that thyroid physiology normally alters during gestation. Clinicians, often, overlook thyroid dysfunction in pregnant women because of nonspecific symptoms and the hypermetabolic state of normal pregnancy. After all, the clinician must consider thyroid dysfunction as a possibility in pregnant women and differentiate normal physiological changes from thyroid disease. Abnormalities of maternal thyroid function can affect the fetus directly or indirectly. The fetal thyroid begins to produce thyroid hormones after the first trimester, so the critical thyroid hormones for fetal brain development must be supplied by the mother. It is of interest how sensitive the fetus is to decreases in circulating maternal thyroid hormones and in maternal thyrotoxicosis. Over recent years there has been an expansion of knowledge regarding thyroid disease in pregnancy and subsequently controversies about management of thyroid disease in pregnancy. This review focuses on maternal overt and subclinical hypothyroidism in pregnancy, suggesting guidelines for appropriate treatment for this disease due to its frequency, especially the subclinical manifestation, and the controversy that exists. It also reviews the complications in mother and fetus when hypothyroidism is untreated. On the other hand, with respect to other differing opinions, some recommendations should be given by the authors about supplying thyroxin to pregnant women with thyroid disease other than hypothyroidism.

Mittra ES, Niederkohr RD, Rodriguez C, El-Maghraby T, McDougall IR. · Division of Nuclear Medicine and Molecular Imaging Program at Stanford, Department of Radiology, Stanford University Hospital and Clinics, Stanford, California 94305-5281, USA. · J Nucl Med. · Pubmed #18199610 links to  free full text

Abstract: Apart from the common causes of thyrotoxicosis, such as Graves' disease and functioning nodular goiters, there are more than 20 less common causes of elevated free thyroid hormones that produce the symptoms and signs of thyrotoxicosis. This review describes these rarer conditions and includes 14 illustrative patients. Thyrotropin and free thyroxine should be measured and, when the latter is normal, the free triiodothyronine level should be obtained. Measurement of the uptake of (123)I is recommended for most patients.

Mina A, Favaloro EJ, Koutts J. · Institute of Clinical Pathology and Medical Research (ICPMR), Westmead Hospital, Westmead, Australia. · Semin Thromb Hemost. · Pubmed #18175285 No free full text.

Abstract: We review the association between disorders of endocrine function and hemostasis. The content of more than 570 review articles were appraised to provide the core of 81 key articles referenced in this chapter. The search method used MEDLINE and EMBASE electronic databases and the key words e NDOCRINE DYSFUNCTION, DIABETES, GRAVES' DISEASE, HYPOTHYROIDISM, HYPERTHYROIDISM, THYROTOXICOSIS, VON WILLEBRAND DISEASE, VON WILLEBRAND FACTOR, BLEEDING DISORDERS, PLATELETS DYSFUNCTION, HEMOSTASIS DYSFUNCTION, and REVIEW. Abnormalities of hemostasis, platelets, and endothelium and the presence of microparticles, abnormal expression of adhesion molecules, and elevated von Willebrand factor are all associated with cardiovascular disease and are also features of various endocrine disorders, including diabetes and its complications, insulin resistance, polycystic ovary syndrome, and various thyroid disorders. Related causes and associated factors, including obesity, alcohol, hyperlipidemia, omega fatty acids, vitamin D, serotonin, insulin-like growth factors, angiotensin-converting enzyme, and C-reactive protein, are also discussed in this review.

Kuefner MA, Heinrich M, Bautz W, Uder M. · Radiologisches Institut, Universität Erlangen-Nürnberg, Maximiliansplatz 1, D-91054 Erlangen, Germany. · Rontgenpraxis. · Pubmed #19294866 No free full text.

Abstract: Iodinated contrast media are widely used in computed tomography and angiography. Adverse reactions such as contrast-medium induced nephropathy (CIN), anaphylactoid reactions and iodine-induced thyrotoxicosis are associated with intravasal administration of contrast agents. Iodinated contrast agents are generally considered to be safe, but in rare cases they can cause severe life threatening situations. In this review we present an overview about the incidence, pathways, and risk factors of adverse reactions. Simple schemes including hydration protocols for prevention of CIN, medication for prophylaxis of iodine-induced thyrotoxicosis with thyreostatics and anaphylactoid reactions with histamine antagonists and corticosteroids are suggested.

Serrao R, Zirwas M, English JC. · Department of Dermatology, University of Pittsburgh, Pittsburgh, Pennsylvania, USA. · Am J Clin Dermatol. · Pubmed #18039017 No free full text.

Abstract: Palmar erythema (PE), an often overlooked physical finding, is due to several physiologic or systemic pathologic states. PE can exist as a primary physiologic finding or as a secondary marker of systemic pathology. Primary or physiologic PE can be due to heredity, occurs in at least 30% of pregnant women as a result of associated alterations in the function of the skin and its microvasculature, or may be a diagnosis of exclusion (i.e. idiopathic PE). Secondary PE from systemic pathology encompasses a wide range of disease states. Twenty-three percent of patients with liver cirrhosis, from varying causes, can manifest PE as a result of abnormal serum estradiol levels. Patients with a rare neonatal liver disease such as Wilson disease and hereditary hemochromatosis may exhibit PE along with the other systemic manifestations of the genodermatoses. PE has been reported to occur in >60% of patients with rheumatoid arthritis and is associated with a favorable prognosis. Up to 18% of patients with thyrotoxicosis and 4.1% of patients with diabetes mellitus can have PE. This cutaneous manifestation of diabetes occurs more often than the more classic diseases such as necrobiosis lipoidica diabeticorum (0.6%). PE can be seen in early gestational syphilis and among patients with human T-lymphotrophic virus-1-associated myelopathy. Drug-induced PE with hepatic damage has been documented with use of amiodarone, gemfibrozil, and cholestyramine, while topiramate and albuterol (salbutamol) have been reported to cause PE in the setting of normal liver function. Fifteen percent of patients with both metastatic and primary brain neoplasms may have PE. Increased levels of angiogenic factors and estrogens from solid tumors have been postulated as the cause of PE in such cases. Erythema ab igne can mimic PE, and patients with atopic diathesis are more likely to have PE than matched control subjects. Smoking and chronic mercury poisoning are environmental causes of PE.No treatment of primary PE is indicated. If medication is the cause of PE, the drug responsible should be discontinued if possible. Identification of PE related to underlying disorders should be followed by treatment of the underlying condition.In light of the numerous etiologies of PE, this article reviews the current literature and provides a framework to help guide the clinician in determining the cause of PE in patients presenting with this finding.

Wild EJ, Tabrizi SJ. · Department of Neurodegenerative Disease, UCL Institute of Neurology, London/National Hospital for Neurology and Neurosurgery, Queen Square, London, UK. · Pract Neurol. · Pubmed #18024776 No free full text.

Abstract: Chorea is a hyperkinetic movement disorder characterised by excessive spontaneous movements that are irregularly timed, randomly distributed and abrupt. In this article, the authors discuss the causes of chorea, particularly Huntington's disease and the genetic syndromes that may resemble it, including HDL1-3, inherited prion disease, spinocerebellar ataxias 1, 3 and 17, neuroacanthocytosis, dentatorubro-pallidoluysian atrophy (DRPLA), brain iron accumulation disorders, Wilson's disease, benign hereditary chorea, Friedreich's ataxia and mitochondrial disease. Acquired causes of chorea include vascular disease, post-infective autoimmune central nervous system disorders (PANDAS), drugs, systemic lupus erythematosus, antiphospholipid syndrome, thyrotoxicosis, AIDS, chorea gravidarum, and polycythaemia rubra vera. The authors suggest an approach to the clinical assessment of chorea, the value of investigations, including genetic tests (for which they offer a structured framework highlighting the importance of prior counselling), and finally briefly discuss symptomatic drug treatment of chorea.

Congedo V, Celi FS. · Clinical Endocrinology Branch, National Institute of Diabetes & Digestive & Kidney Disease, National Institutes of Health Bethesda, Maryland 20892, USA. · Pediatr Endocrinol Rev. · Pubmed #17982391 No free full text.

Abstract: Involvement of the thyroid gland in the McCune-Albright Syndrome (MAS) is a common and sometimes overlooked feature of this disorder. The characteristics of the thyroid in MAS include isolated or combinations of generalized inhomogeneity, cystic and/or nodular features coupled with functional abnormalities. While most often the pathology is diffuse, it is rarely associated with compressive symptoms and signs. The functional abnormalities, which are seldom observed in the absence of underlying echographic structural changes, are characterized by autonomous function, frequently with a shifted T3/T4 ratio, suggesting an increase in intra-thyroidal conversion of the pro-hormone T4 into the active metabolite T3. While MAS-associated thyrotoxicosis is not always symptomatic, it is often linked with increased morbidity, especially in the presence of underlying conditions requiring surgical intervention. Although thyroid cancer has been described in two cases of MAS, the prevalence of malignancy does not appear to be high. The therapeutic options in MAS involvement of the thyroid gland include thionamides, 131Iodine and surgery.

Kwon KT, Tsai VW. · Department of Emergency Medicine, School of Medicine, University of California-Irvine, 101 The City Drive South, Orange, CA 92868, USA. · Emerg Med Clin North Am. · Pubmed #17950135 No free full text.

Abstract: Metabolic diseases can vary as much in clinical presentation as they can in classification, and neonates and infants frequently present with symptoms similar to those seen with other emergencies. Vomiting, alterations in neurologic status, and feeding difficulties are the most prominent features of metabolic emergencies. This article discusses the recognition and management of specific disorders, including diabetic ketoacidosis, congenital adrenal hyperplasia, inborn errors of metabolism, and thyrotoxicosis. Also highlighted are specific laboratory entities, including hypoglycemia, hyponatremia, and metabolic acidosis.

Dionigi G, Dionigi R, Bartalena L, Tanda ML, Piantanida E, Castano P, Annoni M, Boni L, Rovera F, Bacuzzi A, Vanoli P, Sessa F. · Centro di Ricerche in Endocrinochirurgia, Dipartimento di Scienze Chirurgiche , Università degli Studi dell'Insubria, Varese. · Minerva Chir. · Pubmed #17947947 No free full text.

Abstract: Thyroid surgery, one of the most common interventions in endocrine surgery, is practiced by many specialists who perform this procedure exclusively. It accounts for the bulk of work even in reference centers that treat rare endocrine tumors (e.g. adrenal and gastrointestinal tract cancer). Better results are obtained by experienced and skilled operators. Surgeons who correctly perform thyroid surgery can achieve excellent outcomes even in other areas of endocrine surgery. So it is surprising that not more is being done to teach the procedure, which has always been considered something of an art, perhaps because surgical treatment of rare endocrine tumors is more stimulating to teach than routine surgical procedures. Nonetheless, teaching correct surgical technique is essential for reducing and avoiding postoperative complications caused by inadequate experience and knowledge. Numerous studies have reported that the incidence of complications is high and that the rate is growing: 5% involve permanent injury to the recurrent laryngeal nerve after intervention for a benign tumor, despite repeated reports that the incidence could be reduced to near zero or at least to 1%. Alarmingly high is the 20% incidence of persistent hypoparathyroidism after total thyroidectomy. Here, too, accurate technique could reduce this rate to 1%. An important point is that permanent laryngeal nerve injury and persistent hypoparathyroidism are both sources of considerable discomfort for patients. One of the chief objectives of modern endocrine surgery is, therefore, to reduce the complications rate to acceptable levels by establishing adequate, uniform teaching protocols and universal guidelines that would help improve the practice of surgery.