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Calcinosis Cutis
Dermatology Online Journal UC Davis Calcinosis cutis: A rare feature of adult dermatomyositis Inês Machado Moreira Lobo, Susana Machado, Marta Teixeira, Manuela Selores Dermatology Online Journal 14 (1): 10 Department of Dermatology, Hospital Geral de Santo António, Porto, Portugal. [email protected] Abstract Dermatomyositis is an idiopathic inflammatory myopathy with characteristic cutaneous manifestations. We describe a case of a 55- year-old woman with dermatomyositis who presented with dystrophic calcinosis resistant to medical treatment. Dermatomyositis is an idiopathic inflammatory myopathy with characteristic cutaneous manifestations, including heliotrope rash, Gottron papules, periungual telangiectasias, photodistributed erythema, poikiloderma, and alopecia. Although heliotrope rash and Gottron papules are specific cutaneous features, calcinosis of the skin or muscles is unusual in adults with dermatomyositis. However, it may occur in up to 40 percent of children or adolescents [1]. Calcinosis cutis is the deposition of insoluble calcium salts in the skin. Calcinosis cutis may be divided into four categories according to the pathogenesis as follows: dystrophic, metastatic, idiopathic, and iatrogenic. In connective tissue diseases, calcinosis is mostly of the dystrophic type and it seems to be a localized process rather than an imbalance of calcium homeostasis. Calcium deposits may be intracutaneous, subcutaneous, fascial, or intramuscular. Clinical synopsis A 55-year-old woman was referred for evaluation because of multiple, firm nodules of the lateral hips since 1994. At that time, dermatomyositis was diagnosed based on cutaneous, muscular and pulmonary involvement. The nodules, gradually enlarging since 1999, have begun to cause incapacitation pain and many exude a yellowish material suggestive of calcium. She denied an inciting traumatic event. -
MDCT of Interatrial Septum
Diagnostic and Interventional Imaging (2015) 96, 891—899 PICTORIAL REVIEW /Cardiovascular imaging MDCT of interatrial septum ∗ D. Yasunaga , M. Hamon Service de radiologie, pôle d’imagerie, CHU de Caen, avenue de la Côte-de-Nacre, 14033 Caen Cedex 9, France KEYWORDS Abstract ECG-gated cardiac multidetector row computed tomography (MDCT) allows precise Cardiac CT; analysis of the interatrial septum (IAS). This pictorial review provides a detailed description of Interatrial septum; the normal anatomy, variants and abnormalities of the IAS such as patent foramen ovale, con- Patent foramen genital abnormalities such as atrial septal defects as well as tumors and tumoral-like processes ovale; that develop on the IAS. Secundum ASD © 2015 Published by Elsevier Masson SAS on behalf of the Éditions françaises de radiologie. Introduction Major technical advances in computed tomography (CT) in recent years have made it pos- sible to use multidetector row CT (MDCT) in the field of cardiac imaging. Besides coronary arteries, ECG-gated cardiac MDCT provides high-resolution images of all cardiac structures. It is therefore important for radiologists to understand and be able to analyze the normal anatomical structures, variants and diseases of these different structures. This article provides an analysis of the interatrial septum (IAS) based on a pictorial review. After a short embryological and anatomical description, we will illustrate the nor- mal anatomy and variants of the IAS, anomalies such as patent foramen ovale (PFO), congenital diseases such as atrial septal defects (ASD) as well as tumors and tumoral-like processes that develop on the IAS. Abbreviations: ASA, atrial septal aneurysm; ASD, atrial septal defect; ECG, electrocardiogram; IAS, interatrial septum; IVC, inferior vena cava; IVS, interventricular septum; LV, left ventricle; M, myxoma; PFO, patent foramen ovale; RSPV, right superior pulmonary vein; RV, right ventricle; SVC, superior vena cava; MIP, maximal intensity projection; TEE, transesophageal echocardiography; TV, tricuspid valve. -
Soft Tissue Calcification and Ossification
Soft Tissue Calcification and Ossification Soft-tissue Calcification Metastatic Calcification =deposit of calcium salts in previously normal tissue (1) as a result of elevation of Ca x P product above 60-70 (2) with normal Ca x P product after renal transplant Location:lung (alveolar septa, bronchial wall, vessel wall), kidney, gastric mucosa, heart, peripheral vessels Cause: (a)Skeletal deossification 1.1° HPT 2.Ectopic HPT production (lung / kidney tumor) 3.Renal osteodystrophy + 2° HPT 4.Hypoparathyroidism (b)Massive bone destruction 1.Widespread bone metastases 2.Plasma cell myeloma 3.Leukemia Dystrophic Calcification (c)Increased intestinal absorption =in presence of normal serum Ca + P levels secondary to local electrolyte / enzyme alterations in areas of tissue injury 1.Hypervitaminosis D Cause: 2.Milk-alkali syndrome (a)Metabolic disorder without hypercalcemia 3.Excess ingestion / IV administration of calcium salts 1.Renal osteodystrophy with 2° HPT 4.Prolonged immobilization 2.Hypoparathyroidism 5.Sarcoidosis 3.Pseudohypoparathyroidism (d)Idiopathic hypercalcemia 4.Pseudopseudohypoparathyroidism 5.Gout 6.Pseudogout = chondrocalcinosis 7.Ochronosis = alkaptonuria 8.Diabetes mellitus (b) Connective tissue disorder 1.Scleroderma 2.Dermatomyositis 3.Systemic lupus erythematosus (c)Trauma 1.Neuropathic calcifications 2.Frostbite 3.Myositis ossificans progressiva 4.Calcific tendinitis / bursitis (d)Infestation 1.Cysticercosis Generalized Calcinosis 2.Dracunculosis (guinea worm) (a)Collagen vascular disorders 3.Loiasis 1.Scleroderma -
Eye Neoplasm
Eye neoplasm Origin and location Eye cancers can be primary (starts within the eye) and metastatic cancer (spread to the eye from another organ). The two most common cancers that spread to the eye from another organ are breast cancer and lung cancer. Other less common sites of origin include the prostate, kidney, thyroid, skin, colon and blood or bone marrow. Types Tumors in the eye and orbit can be benign like dermoid cysts, or malignant like rhabdomyosarcoma and retinoblastoma. Signs and symptoms • Melanomas (choroidal, ciliary body and uveal) - In the early stages there may be no symptoms (the person does not know there is a tumor until an ophthalmologist or optometrist looks into the eye with an ophthalmoscope during a routine test). As the tumor grows, symptoms can be blurred vision, decreased vision, double vision, eventual vision loss and if they continue to grow the tumor can break past the retina causing retinal detachment. Sometimes the tumor can be visible through the pupil. • Nevus - Are benign, freckle in the eye. These should be checked out and regular checks on the eye done to ensure it hasn't turned into a melanoma. • Iris and conjuctival tumors (melanomas) - Presents as a dark spot. Any spot which continues to grow on the iris and the conjunctiva should be checked out. • Retinoblastoma - Strabismus (crossed eyes), a whitish or yellowish glow through the pupil, decreasing/loss of vision, sometimes the eye may be red and painful. Retinoblastoma can occur in one or both eyes. This tumor occurs in babies and young children. It is called RB for short. -
Section XI Extraskeletal (Ectopic) Calcification and Ossification
Section XI Extraskeletal (Ectopic) Calcification and Ossification Michael P. Whyte Division of Bone and Mineral Diseases, Washington University School of Medicine at Barnes-Jewish Hospital and Center for Metabolic Bone Disease and Molecular Research, Shriners Hospitals for Children, St. Louis, Missouri INTRODUCTION somewhat higher value because they have greater serum phos- phate concentrations compared with adults. However, this is A significant number and variety of disorders cause extraskel- not well established.(5) etal deposition of calcium and phosphate (Table 1). In some, The material that comprises metastatic calcification may be mineral is precipitated as amorphous calcium phosphate or as amorphous calcium phosphate initially, but hydroxyapatite is crystals of hydroxyapatite; in others, osseous tissue is formed. deposited soon after.(2) The anatomic pattern of deposition The pathogenesis of ectopic mineralization is generally attrib- varies somewhat between hypercalcemia and hyperphos- uted to one of three mechanisms (Table 1). First, a supranormal phatemia, but occurs irrespective of the specific underlying “calcium-phosphate solubility product” in extracellular fluid condition or mechanism for the disturbed mineral homeostasis. can cause metastatic calcification. Second, mineral may be Additionally, there is a predilection for certain tissues. deposited as dystrophic calcification into metabolically im- Hypercalcemia is typically associated with mineral deposits paired or dead tissue despite normal serum levels of calcium in the kidneys, lungs, and fundus of the stomach. In these and phosphate. Third, ectopic ossification (or true bone forma- “acid-secreting” organs, a local alkaline milieu may account tion) occurs in a few disorders for which the pathogenesis is for the calcium deposition. In addition, the media of large becoming increasingly understood. -
T PATHOLOGIC CALCIFICATION Deposition of Calcium Salts In
t PATHOLOGIC CALCIFICATION Deposition of calcium salts in tissues other than osteoid or enamel is called pathologic or heterotopic calcification. Two distinct types of pathologic calcification are recognised: Dystrophic calcification, which is characterised by deposition of calcium salts in dead or degenerated tissues with normal calcium metabolism and normal serum calcium levels. Dystrophic calcification may occur due to 2 types of causes: ● Calcification in dead tissue ● Calcification of degenerated tissue Metastatic calcification, on the other hand, occurs in apparently normal tissues and is associated with deranged calcium metabolism and hypercalcaemia. Since metastatic calcification occurs in normal tissues due to hypercalcaemia, its causes would include one of the following two conditions: ● Excessive mobilisation of calcium from the bone. ● Excessive absorption of calcium from the gut. Pathogenesis of Dystrophic Calcification The process of dystrophic calcification has been likened to the formation of normal hydroxyapatite in the bone involving 2 phases: ● Initiation and propagation: Initiation is the phase in which precipitates of calcium phosphate begin to accumulate intracellularly in the mitochondria, or extracellularly in membrane-bound vesicles. Propagation is the phase in which minerals deposited in the initiation phase are propagated to form mineral crystals. Pathogenesis of metastatic calcification Metastatic calcification occurs due to excessive binding of inorganic phosphate ions with calcium ions, which are elevated due to underlying metabolic derangement. This leads to formation of precipitates of calcium phosphate at the preferential sites. Metastatic calcification is reversible upon correction of underlying metabolic disorder. GANGRENE Gangrene is a form of necrosis of tissue with superadded putrefaction. The type of necrosis is usually coagulative due to ischaemia (e.g. -
Cardiovascular System Note: the Cardiovascular System Develops Early (Week 3), Enabling the Embryo to Grow Beyond the Short
Lymphatics: Lymph vessel formation is similar to blood angiogenesis. Lymphatics begin as lymph sacs in three regions: jugular (near brachiocephalic veins); cranial abdominal (future cysterna chyla); and iliac region. Lym- phatic vessels (ducts) form as outgrowths of the sacs. mesenchyme Lymph nodes are produced by localized mesoder- sinusoid lymph duct lumen mal invaginations that partition the vessel lumen into sinu- soids. The mesoderm develops a reticular framework within which mesodermal lymphocytes accumulate. The spleen and hemal nodes (in ruminants) invagination develop similar to the way lymph nodes develop. Lymph Node Formation Prior to birth, fetal circulation is designed for an in utero aqueous environment where the pla- centa oxygenates fetal blood. Suddenly, at birth... Three In-Utero Adjustments ductus Stretching and constriction of arteriosus umbilical arteries shifts fetal blood flow aortic arch from the placenta to the fetus. Reduced pulmonary trunk L atrium venous return through the (left) umbili- foramen ovale R cal vein and ductus venosus allows the atrium latter to gradually close (over a period caudal vena cava of days). Bradykinin released by expand- ductus venosus ing lungs and increased oxygen concen- tration in blood triggers constriction of aorta the ductus arteriosus which, over two liver months, is gradually converted to a fibrous structure, the ligamentum arte- umbilical v. riosum. portal v. The increased blood flow to the lungs and then to the left atrium equalizes pres- sure in the two atria, resulting in closure umbilical aa. of the foramen ovale that eventually grows permanent. 29 The cardiogenic area, the place where the embryonic heart originates, is located . -
Endothelial Dysfunction May Link Interatrial Septal Abnormalities and MTHFR-Inherited Defects to Cryptogenic Stroke Predisposition
biomolecules Article Endothelial Dysfunction May Link Interatrial Septal Abnormalities and MTHFR-Inherited Defects to Cryptogenic Stroke Predisposition 1, 2, 1 1 Luca Sgarra y, Alessandro Santo Bortone y, Maria Assunta Potenza , Carmela Nacci , Maria Antonietta De Salvia 1, Tommaso Acquaviva 2, Emanuela De Cillis 2, Marco Matteo Ciccone 2, Massimo Grimaldi 3 and Monica Montagnani 1,* 1 Department of Biomedical Sciences and Human Oncology—Section of Pharmacology, Medical School, University of Bari “Aldo Moro”, 70124 Bari, Italy; [email protected] (L.S.); [email protected] (M.A.P.); [email protected] (C.N.); [email protected] (M.A.D.S.) 2 Department of Emergency and Organ Transplantation—Section of Cardiovascular Diseases, Medical School, University of Bari “Aldo Moro”, 70124 Bari, Italy; [email protected] (A.S.B.); [email protected] (T.A.); [email protected] (E.D.C.); [email protected] (M.M.C.) 3 General Hospital “F. Miulli” Acquaviva delle Fonti, 70021 Bari, Italy; fi[email protected] * Correspondence: [email protected] These authors contributed equally to this work. y Received: 28 March 2020; Accepted: 2 June 2020; Published: 4 June 2020 Abstract: We explored the significance of the L-Arginine/asymmetric dimethylarginine (L-Arg/ADMA) ratio as a biomarker of endothelial dysfunction in stroke patients. To this aim, we evaluated the correlation, in terms of severity, between the degree of endothelial dysfunction (by L-Arg/ADMA ratio), the methylene tetrahydrofolate reductase (MTHFR) genotype, and the interatrial septum (IAS) phenotype in subject with a history of stroke. Methods and Results: L-Arg, ADMA, and MTHFR genotypes were evaluated; the IAS phenotype was assessed by transesophageal echocardiography. -
PATHOPHYSIOLOGY UNIT-1 .Basic Principles of Cell Injury And
B.PHARMACY2nd SEMESTER SUBJECT: PATHOPHYSIOLOGY UNIT-1 .Basic Principles of Cell Injury and Adaptation Cell Injury: Introduction • Cell injury is defined as a variety of stresses a cell encounters as a result of changes in its internal and external environment. • The cellular response to stress may vary and depends upon the following: – The type of cell and tissue involved. – Extent and type of cell injury. ETIOLOGY OF CELL INJURY: 1. Genetic causes • Developmental defects: Errors in morphogenesis • Cytogenetic (Karyotypic) defects: chromosomal abnormalities • Single-gene defects: Mendelian disorders • Multifactorial inheritance disorders. 2. Acquired causes • Hypoxia and ischaemia • Physical agents • Chemical agents and drugs • Microbial agents • Immunologic agents • Nutritional derangements • Aging • Psychogenic diseases • Iatrogenic factors • Idiopathic diseases. 2.1. Oxygen deprivation: HYPOXIA Ischemia (loss of blood supply). Inadequate oxygenation (cardio respiratory failure). Loss of oxygen carrying capacity of the blood (anemia or CO poisoning). 2.2. PHYSICAL AGENTS: Trauma Heat Cold Radiation Electric shock 2.3. CHEMICAL AGENTS AND DRUGS: Endogenous products: urea, glucose Exogenous agents Therapeutic drugs: hormones Nontherapeutic agents: lead or alcohol. 2.4. INFECTIOUS AGENTS: Viruses Rickettsiae Bacteria Fungi Parasites 2.5. Abnormal immunological reactions: The immune process is normally protective but in certain circumstances the reaction may become deranged. Hypersensitivity to various substances can lead to anaphylaxis or to more localized lesions such as asthma. In other circumstances the immune process may act against the body cells – autoimmunity. 2.6. Nutritional imbalances: Protein-calorie deficiencies are the most examples of nutrition deficiencies. Vitamins deficiency. Excess in nutrition are important causes of morbidity and mortality. Excess calories and diet rich in animal fat are now strongly implicated in the development of atherosclerosis. -
The Interventricular Septum by E
Thorax: first published as 10.1136/thx.12.4.304 on 1 December 1957. Downloaded from Thorax (1957), 12, 304. THE INTERVENTRICULAR SEPTUM BY E. W. T. MORRIS From the Anatomy Department, St. Thomas's Hospital Medical School, Londoni (RECEIVED FOR PUBLICATION JULY 26, 1957) It is difficult to find in the literature a clear and between the tips of its two horns where the concise account of the development and form of boundary is formed by the fused atrioventricular the interventricular septum. Moreover, some of cushion (A, in Fig. 4). This septum does not lie the accounts in the clinical literature are at vari- in one plane and the main part of its free border ance with that generally accepted by embryo- forms a spiral (Figs. 4 and 5). logists. For this reason and in view of the recent (2) While the muscular part is forming. changes technical advances in the surgery of the heart, it are taking place in the relative positions of the seems opportune to describe the development and bulbus cordis and the ventricles. Earlier the heart anatomy of the interventricular septum and to tube is flexed at the bulboventricular junction so correlate this knowledge as far as possible with that the bulbus cordis comes to lie ventrally and the sites of interventricular septal defects. to the right of the ventricle (Fig. 2). Their con- At an early stage the heart consists of the sinus tiguous walls form a septum-the bulboven- venosus, the common atrium, the common ven- tricular septum-around the lower free border tricle, and the bulbus cordis, serially arranged in of which the two cavities communicate (see Figs.copyright. -
Cardiovascular System Note: the Cardiovascular System Develops Early (Week-3), Enabling the Embryo to Grow Beyond the Short
Cardiovascular System Note: The cardiovascular system develops early (week-3), enabling the embryo to grow beyond the short distances over which diffusion is efficient for transferring 2O , CO2, and cellular nutrients & wastes. Heart: Beginning as a simple tube, the heart undergoes differential growth into a four chambered struc- ture, while it is pumping blood throughout the embryo and into extra-embryonic membranes. Angiogenesis begins with blood island formation in splanchnic mesoderm of the yolk sac and allantois. Vessel formation occurs when island vesicles coalesce, sprout buds, and fuse to form vascular channels. Hematopoiesis (blood cell formation) occurs in the liver and spleen and later in the bone marrow. The transition from fetal to adult circulation involves new vessel formation, vessel merger, and degeneration of early vessels. Formation of a Tubular Heart: The first evidence of heart develop- amnionic cavity ment is bilateral vessel formation within ectoderm the cardiogenic plate (splanchnic meso- embryo derm situated anterior to the embryo). The cardiogenic plate moves ven- tral to the pharynx as the head process cardiogenic yolk sac endoderm mesoderm grows upward and outward. plate Bilateral endocardial tubes meet at the midline & fuse into a single endo- embryo cardial tube, the future heart. Splanchnic mesoderm surround- ing the tube forms cardiac muscle cells heart capable of pumping blood. yolk sac Primitive Heart Regions: Differential growth of the endocardial tube establishes five primitive heart regions: 1] Truncus arteriosus — the output region of the heart. It will develop into the ascending aorta and pulmonary trunk. truncus 2] Bulbus cordis — a bulb-shaped region des- arteriosus tined to become right ventricle. -
Atrial-Myxomas.Pdf
Atrial myxomas Atrial myxomas are the most common primary heart tumors. They are benign intracavitary cardiac neoplasms and accounting for one-third to one-half of cases at postmortem and for about three quarter of tumors treated surgically (Braunwald 2001). Because of nonspecific symptoms, early diagnosis may be a challenge. Left atrial myxoma may or may not produce characteristic findings on auscultation. Most atrial myxomas, whether left or right, arise from the atrial septum, usually from the region of the limbus of fossa ovalis. About 10% have other sites of origin, particularly posterior wall, anterior wall and the appendages (in order of frequency) (McAllister 1979). (Two- dimensional echocardiography is the diagnostic procedure of choice. Most atrial myxomas are benign and can be removed by surgical resection. Pathophysiology Myxomas account for 40-50% of primary cardiac tumors. Approximately 90% are solitary and pedunculated, and 75-85% occur in the left atrial cavity. Up to 25% of cases are found in the right atrium. Most cases are sporadic. Approximately 10% are familial and are transmitted in an autosomal dominant mode. Multiple tumors occur in approximately 50% of familial cases and are more frequently located in the ventricle (13% vs 2% in sporadic cases). Myxomas are polypoid, round, or oval. They are gelatinous with a smooth or lobulated surface and usually are white, yellowish, or brown such as of the figures 1 and 2 Fig. 1 Pathology specimens of multiple small myxomas resected from the right atrial surface. Fig. 2 Pathology specimen of myxoma from the vicinity of the interatrial groove, after near-total resection.